<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Hip fracture and explain in detail? [/INST] A hip fracture is a break that occurs in the upper part of the femur (thigh bone). Symptoms may include pain around the hip, particularly with movement, and shortening of the leg. Usually the person cannot walk.They most often occur as a result of a fall. (Femoral head fractures are a rare kind of hip fracture that may also be the result of a fall but are more commonly caused by more violent incidents such as traffic accidents.) Risk factors include osteoporosis, taking many medications, alcohol use, and metastatic cancer. Diagnosis is generally by X-rays. Magnetic resonance imaging, a CT scan, or a bone scan may occasionally be required to make the diagnosis.Pain management may involve opioids or a nerve block. If the persons health allows, surgery is generally recommended within two days. Options for surgery may include a total hip replacement or stabilizing the fracture with screws. Treatment to prevent blood clots following surgery is recommended.About 15% of women break their hip at some point in life; women are more often affected than men. Hip fractures become more common with age. The risk of death in the year following a fracture is about 20% in older people.

Signs and symptoms
The classic clinical presentation of a hip fracture is an elderly patient who sustained a low-energy fall and now has groin pain and is unable to bear weight. Pain may be referred to the supracondylar knee. On examination, the affected extremity is often shortened and externally rotated compared to the unaffected leg.

Complications
Nonunion, failure of the fracture to heal, is common in fractures of the neck of the femur, but much more rare with other types of hip fracture. Avascular necrosis of the femoral head occurs frequently (20%) in intracapsular hip fractures, because the blood supply is interrupted.Malunion, healing of the fracture in a distorted position, is very common.  The thigh muscles tend to pull on the bone fragments, causing them to overlap and reunite incorrectly. Shortening, varus deformity, valgus deformity, and rotational malunion all occur often because the fracture may be unstable and collapse before it heals.  This may not be as much of a concern in patients with limited independence and mobility.Hip fractures rarely result in neurological or vascular injury.

Medical
Many people are unwell before breaking a hip; it is common for the break to have been caused by a fall due to some illness, especially in the elderly.  Nevertheless, the stress of the injury, and a likely surgery, increases the risk of medical illness including heart attack, stroke, and chest infection.Hip fracture patients are at considerable risk for thromboemoblism, blood clots that dislodge and travel in the bloodstream. Deep venous thrombosis (DVT) is when the blood in the leg veins clots and causes pain and swelling. This is very common after hip fracture as the circulation is stagnant and the blood is hypercoagulable as a response to injury. DVT can occur without causing symptoms.  A pulmonary embolism (PE) occurs when clotted blood from a DVT comes loose from the leg veins and passes up to the lungs. Circulation to parts of the lungs is cut off which can be very dangerous. Fatal PE may have an incidence of 2% after hip fracture and may contribute to illness and mortality in other cases.Mental confusion is extremely common following a hip fracture. It usually clears completely, but the disorienting experience of pain, immobility, loss of independence, moving to a strange place, surgery, and drugs combine to cause delirium or accentuate pre-existing dementia.
Urinary tract infection (UTI) can occur. Patients are immobilized and in bed for many days; they are frequently catheterised, commonly causing infection.
Prolonged immobilization and difficulty moving make it hard to avoid pressure sores on the sacrum and heels of patients with hip fractures. Whenever possible, early mobilization is advocated; otherwise, alternating pressure mattresses should be used.

Risk factors
Hip fracture following a fall is likely to be a pathological fracture. The most common causes of weakness in bone are:
Osteoporosis.
Other metabolic bone diseases such as Pagets disease, osteomalacia, osteopetrosis and osteogenesis imperfecta. Stress fractures may occur in the hip region with metabolic bone disease.
Elevated levels of homocysteine, a toxic natural amino acid.
Benign or malignant primary bone tumors are rare causes of hip fractures.
Metastatic cancer deposits in the proximal femur may weaken the bone and cause a pathological hip fracture.
Infection in the bone is a rare cause of hip fracture.
Tobacco smoking (associated with osteoporosis).

Mechanism
Functional anatomy
The hip joint is a ball-and-socket joint. The femur connects at the acetabulum of the pelvis and projects laterally before angling medially and inferiorly to form the knee. Although this joint has three degrees of freedom, it is still stable due to the interaction of ligaments and cartilage. The labrum lines the circumference of the acetabulum to provide stability and shock absorption. Articular cartilage covers the concave area of acetabulum, providing more stability and shock absorption.  Surrounding the entire joint itself is a capsule secured by the tendon of the psoas muscle and three ligaments. The iliofemoral, or Y, ligament is located anteriorly and serves to prevent hip hyperextension. The pubofemoral ligament is located anteriorly just underneath the iliofemoral ligament and serves primarily to resist abduction, extension, and some external rotation. Finally the ischiofemoral ligament on the posterior side of the capsule resists extension, adduction, and internal rotation. When considering the biomechanics of hip fractures, it is important to examine the mechanical loads the hip experiences during low energy falls.

Biomechanics
The hip joint is unique in that it experiences combined mechanical loads. An axial load along the shaft of the femur results in compressive stress. Bending load at the neck of the femur causes tensile stress along the upper part of the neck and compressive stress along the lower part of the neck. While osteoarthritis and osteoporosis are associated with bone fracture as we age, these diseases are not the cause of the fracture alone. Low energy falls from standing are responsible for the majority of fractures in the elderly, but fall direction is also a key factor. Elderly tend to fall to the side as instead of forward, and the lateral hip and strikes the ground first. During a sideways fall, the chances of hip fracture see a 15-fold and 12-fold increase in elderly males and females, respectively.

Neurological factors
Elderly individuals are also predisposed to hip fractures due to many factors that can compromise proprioception and balance, including medications, vertigo, stroke, and peripheral neuropathy.

Diagnosis
Physical examination
Displaced fractures of the trochanter or femoral neck will classically cause external rotation and shortening of the leg when the patient is laying supine.

Imaging
Typically, radiographs are taken of the hip from the front (AP view), and side (lateral view). Frog leg views are to be avoided, as they may cause severe pain and further displace the fracture. In situations where a hip fracture is suspected but not obvious on x-ray, an MRI is the next test of choice. If an MRI is not available or the patient can not be placed into the scanner a CT may be used as a substitute. MRI sensitivity for radiographically occult fracture is greater than CT. Bone scan is another useful alternative however substantial drawbacks include decreased sensitivity, early false negative results and decreased conspicuity of findings due to age-related metabolic changes in the elderly.A case demonstrating a possible order of imaging in initially subtle findings:

As the patients most often require an operation, full pre-operative general investigation is required. This would normally include blood tests, ECG and chest x-ray.

Types
X-rays of the affected hip usually make the diagnosis obvious; AP (anteroposterior) and lateral views should be obtained.
Trochanteric fractures are subdivided into either intertrochanteric (between the greater and lesser trochanter) or pertrochanteric (through the trochanters) by the Müller AO Classification of fractures. Practically, the difference between these types is minor. The terms are often used synonymously. An isolated trochanteric fracture involves one of the trochanters without going through the anatomical axis of the femur, and may occur in young individuals due to forceful muscle contraction. Yet, an isolated trochanteric fracture may not be regarded as a true hip fracture because it is not cross-sectional.

Prevention
The majority of hip fractures are the result of a fall, particularly in the elderly.  Therefore, identifying why the fall occurred, and implementing treatments or changes, is key to reducing the occurrence of hip fractures.  Multiple contributing factors are often identified. These can include environmental factors and medical factors (such as postural hypotension or co-existing disabilities from disease such as Stroke or Parkinsons disease which cause visual and/or balance impairments).  A recent study has identified a high incidence of undiagnosed cervical spondylotic myelopathy (CSM) amongst patients with a hip fracture. This is relatively unrecognised consequent of CSM.Additionally, there is some evidence to systems designed to offer protection in the case of a fall. Hip protectors, for example appear to decrease the number of hip fractures among the elderly, but they are often not used.

Management
Most hip fractures are treated surgically by implanting a prosthesis. Surgical treatment outweighs the risks of nonsurgical treatment which requires extensive bedrest. Prolonged immobilization increases risk of thromboembolism, pneumonia, deconditioning, and decubitus ulcers. Regardless, the surgery is a major stress, particularly in the elderly. Pain is also significant, and can also result in immobilization, so patients are encouraged to become mobile as soon as possible, often with the assistance of physical therapy. Skeletal traction pending surgery is not supported by the evidence. Regional nerve blocks are useful for pain management in hip fractures. Peripheral nerve blocks may reduce pain on movement and acute confusional state, may improve time to first mobilisation, and may reduce the risk of postoperative lower respiratory tract infection. Surgery can be performed under general anaesthesia or with neuraxial techniques – choice is based on surgical and patient factors, as outcomes such as mortality and post-procedure complications including pneumonia, MI, stroke or confusion, are not affected by anaesthetic technique.Red blood cell transfusion is common for people undergoing hip fracture surgery due to the blood loss sustained during surgery and from the injury. The benefits of giving blood when the hemoglobin is less than 10 g/dL versus less than 8 g/dL are not clear. Waiting until the hemoglobin was less than 8 g/dL or the person had symptoms may increase the risk of heart problems.If operative treatment is refused or the risks of surgery are considered to be too high the main emphasis of treatment is on pain relief. Skeletal traction may be considered for long-term treatment. Aggressive chest physiotherapy is needed to reduce the risk of pneumonia and skilled rehabilitation and nursing to avoid pressure sores and DVT/pulmonary embolism Most people will be bedbound for several months. Non-operative treatment is now limited to only the most medically unstable or demented patients or those who are nonambulatory at baseline with minimal pain during transfers.Surgery on the same day or day following the break is estimated to reduce postoperative mortality in people who are medically stable.

Intracapsular fractures
For low-grade fractures (Garden types 1 and 2), standard treatment is fixation of the fracture in situ with screws or a sliding screw/plate device. This treatment can also be offered for displaced fractures after the fracture has been reduced.Fractures managed by closed reduction can possibly be treated by percutaneously inserted screws.In elderly patients with displaced or intracapsular fractures many surgeons prefer to undertake a hemiarthroplasty, replacing the broken part of the bone with a metal implant. However, in elderly people who are medically well and still active, a total hip replacement may be indicated. Independently mobile older adults with hip fractures may benefit from a total hip replacement instead of hemiarthroplasty.Traction is contraindicated in femoral neck fractures due to it affecting blood flow to the head of the femur.The latest evidence suggests that there may be little or no difference between screws and fixed angle plates as internal fixation implants for intracapsular hip fractures in older adults. The findings are based on low quality evidence that cant firmly conclude major difference in hip function, quality of life, and additional surgery.

Trochanteric fracture
A trochanteric fracture, below the neck of the femur, has a good chance of healing.
Closed reduction may not be satisfactory and open reduction then becomes necessary. The use of open reduction has been reported as 8-13% among pertrochanteric fractures, and 52% among intertrochanteric fractures. Both intertrochanteric and pertrochanteric fractures may be treated by a dynamic hip screw and plate, or an intramedullary rod.The fracture typically takes 3–6 months to heal. As it is only common in elderly, removal of the dynamic hip screw is usually not recommended to avoid unnecessary risk of second operation and the increased risk of re-fracture after implant removal. The most common cause for hip fractures in the elderly is osteoporosis; if this is the case, treatment of the osteoporosis can well reduce the risk of further fracture. Only young patients tend to consider having it removed; the implant may function as a stress riser, increasing the risk of a break if another accident occurs.

Subtrochanteric fractures
Subtrochanteric fractures may be treated with an intramedullary nail or a screw-plate construction and may require traction pre-operatively, though this practice is uncommon. It is unclear if any specific type of nail results in different outcomes than any other type of nail.A lateral incision over the trochanter is made and a cerclage wire is placed around the fracture for reduction. Once reduction has been achieved a guide canal for the nail is made through the proximal cortex and medullary. The nail is inserted through the canal and is fixated proximally and distally with screws. X-rays are obtained to ensure proper reduction and placement of the nail and screws are achieved.

Rehabilitation
Rehabilitation has been proven to increase daily functional status. Forty percent of individuals with hip fractures are also diagnosed with dementia or mild cognitive impairment which often results in poorer post-surgical outcomes.  In such cases enhanced rehabilitation and care models have been shown to have limited positive effects in reducing delirium and hospital length of stay.   It is unclear if the use of anabolic steroids effects recovery.There is not enough evidence to ascertain what are the best strategies to promote walking after hip fracture surgery. On the other hand, there is moderate-certaintly evidence that rehabilitation after hip fracture surgery, when delivered by a multidisciplinary team and supervised by an appropriate medical specialist, results in fewer cases of poor outcome, like death and deterioration in residential status.

Nutrition supplementation
Oral supplements with non-protein energy, protein, vitamins and minerals started before or early after surgery may prevent complications during the first year after hip fracture in aged adults; without seemingly effects on mortality.

Surgical complications
Deep or superficial wound infection has an approximate incidence of 2%. It is a serious problem as superficial infection may lead to deep infection. This may cause infection of the healing bone and contamination of the implants. It is difficult to eliminate infection in the presence of metal foreign bodies such as implants. Bacteria inside the implants are inaccessible to the bodys defence system and to antibiotics. The management is to attempt to suppress the infection with drainage and antibiotics until the bone is healed. Then the implant should be removed, following which the infection may clear up.
Implant failure may occur; the metal screws and plate can break, back out, or cut out superiorly and enter the joint. This occurs either through inaccurate implant placement or if the fixation does not hold in weak and brittle bone. In the event of failure, the surgery may be redone or changed to a total hip replacement. Mal-positioning: The fracture can be fixed and subsequently heal in an incorrect position; especially rotation. This may not be a severe problem or may require subsequent osteotomy surgery for correction.

Prognosis
Hip fractures are very dangerous episodes, especially for elderly and frail patients. The risk of dying from the stress of the surgery and the injury in the first thirty days is about 10%. At one year after fracture, this may reach 30%. If the condition is untreated the pain and immobility imposed on the patient increase that risk. Problems such as pressure sores and chest infections are all increased by immobility. The prognosis of untreated hip fractures is very poor.

Post operation
Among those affected over the age of 65, 40% are transferred directly to long-term care facilities, long-term rehabilitation facilities, or nursing homes; most of those affected require some sort of living assistance from family or home-care providers.  50% permanently require walkers, canes, or crutches for mobility; all require some sort of mobility assistance throughout the healing process. Most of the recovery of walking ability and activities of daily living occurs within 6 months of the fracture. After the fracture about half of older people recover their pre-fracture level of mobility and ability to perform instrumental activities of daily living, while 40–70% regain their level of independence for basic activities of daily living.Among those affected over the age of 50, approximately 25% die within the next year due to complications such as blood clots (deep venous thrombosis, pulmonary embolism), infections, and pneumonia.Patients with hip fractures are at high risk for future fractures including hip, wrist, shoulder, and spine. After treatment of the acute fracture, the risk of future fractures should be addressed. Currently, only 1 in 4 patients after a hip fracture receives treatment and work up for osteoporosis, the underlying cause of most of the fractures. Current treatment standards include the starting of a bisphosphonate to reduce future fracture risk by up to 50%.

Epidemiology
Hip fractures are seen globally and are a serious concern at the individual and population level. By 2050, it is estimated that there will be six million cases of hip fractures worldwide. One study published in 2001 found that in the US alone, 310,000 individuals were hospitalized due to hip fractures, which can account for 30% of Americans who were hospitalized that year. Another study found that in 2011, femur neck fractures were among the most expensive conditions seen in US hospitals, with an aggregated cost of nearly $4.9 billion for 316,000 inpatient hospitalizations. Rates of hip fractures are declining in the United States, possibly due to increased use of bisphosphonates and risk management. Falling, poor vision, weight, and height are all seen as risk factors. Falling is one of the most common risk factors for hip fractures. Approximately 90% of hip fractures are attributed to falls from standing height.Given the high morbidity and mortality associated with hip fractures and the cost to the health system, in England and Wales, the National Hip Fracture Database is a mandatory nationwide audit of care and treatment of all hip fractures.

Population
All populations experience hip fractures but numbers vary with race, gender, and age. Women have three times as many hip fractures as men. In a lifetime, men have an estimated 6% risk whereas postmenopausal women have an estimated 14% risk of having a hip fracture </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Acute cutaneous lupus erythematosus and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Primary myelofibrosis and explain in detail? [/INST] Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a type of myeloproliferative neoplasm, a group of cancers in which there is growth of abnormal cells in the bone marrow. This is most often associated with a somatic mutation in the JAK2, CALR, or MPL gene markers. In PMF, the healthy marrow is replaced by scar tissue (fibrosis), resulting in a lack of production of normal blood cells. Symptoms include anemia, increased infection and an enlarged spleen (splenomegaly).
In 2016, prefibrotic primary myelofibrosis was formally classified as a distinct condition that progresses to overt PMF in many patients, the primary diagnostic difference being the grade of fibrosis.

Signs and symptoms
The primary feature of primary myelofibrosis is bone marrow fibrosis, but it is often accompanied by:

Abdominal fullness related to an enlarged spleen (splenomegaly).
Bone pain
Bruising and easy bleeding due to inadequate numbers of platelets
Cachexia (loss of appetite, weight loss, and fatigue)
Enlargement of both the liver and spleen
Fatigue
Gout and high uric acid levels
Increased susceptibility to infection, such as pneumonia
Pallor and shortness of breath due to anemia
In rarer cases, a raised red blood cell volume
Cutaneous myelofibrosis is a rare skin condition characterized by dermal and subcutaneous nodules.: 746

Causes
The underlying cause of PMF is unknown (idiopathic disease). There is an association between mutations to the JAK2, CALR, or MPL gene and myelofibrosis. Approximately 90% of those with myelofibrosis have one of these mutations and 10% carry none of these mutations. These mutations are not specific to myelofibrosis, and are linked to other myeloproliferative neoplasms, specifically polycythemia vera and essential thrombocythemia.The V617F mutation to the JAK2 protein is found in approximately half of individuals with primary myelofibrosis. The V617F mutation is a change of valine to phenylalanine at the 617 position. Janus kinases (JAKs) are non-receptor tyrosine kinases essential for the activation of signaling that is mediated by cytokine receptors lacking catalytic activity. These include receptors for erythropoietin, thrombopoietin, most interleukins and interferon. JAK2 mutations are significant because JAK2 plays a role in controlling production of blood cells from hematopoietic stem cells. The V617F mutation appears to make hematopoietic cells more sensitive to growth factors that need JAK2 for signal transduction, which include erythropoietin and thrombopoietin.The MPL gene codes for a protein that acts as a receptor for thrombopoietin. A mutation in that gene, known as a W515 mutation, leads to the production of an abnormal thrombopoietin receptor protein, which results in the overproduction of abnormal megakaryocytes. The abnormal megakaryocytes stimulate other cells, the fibroblasts, to produce collagen in the bone marrow, by secreting PDGF and TGF-β1.

Mechanism
Myelofibrosis is a clonal neoplastic disorder of hematopoiesis, the formation of blood cellular components. It is one of the myeloproliferative disorders, diseases of the bone marrow in which excess cells are produced at some stage. Production of cytokines such as fibroblast growth factor by the abnormal hematopoietic cell clone (particularly by megakaryocytes) leads to replacement of the hematopoietic tissue of the bone marrow by connective tissue via collagen fibrosis. The decrease in hematopoietic tissue impairs the patients ability to generate new blood cells, resulting in progressive pancytopenia, a shortage of all blood cell types. However, the proliferation of fibroblasts and deposition of collagen is a secondary phenomenon, and the fibroblasts themselves are not part of the abnormal cell clone.In primary myelofibrosis, progressive scarring, or fibrosis, of the bone marrow occurs, for the reasons outlined above. The result is extramedullary hematopoiesis, i.e. blood cell formation occurring in sites other than the bone marrow, as the hemopoietic cells are forced to migrate to other areas, particularly the liver and spleen. This causes an enlargement of these organs. In the liver, the abnormal size is called hepatomegaly. Enlargement of the spleen is called splenomegaly, which also contributes to causing pancytopenia, particularly thrombocytopenia and anemia. Another complication of extramedullary hematopoiesis is poikilocytosis, or the presence of abnormally shaped red blood cells.Myelofibrosis can be a late complication of other myeloproliferative disorders, such as polycythemia vera, and less commonly, essential thrombocythemia. In these cases, myelofibrosis occurs as a result of somatic evolution of the abnormal hematopoietic stem cell clone that caused the original disorder. In some cases, the development of myelofibrosis following these disorders may be accelerated by the oral chemotherapy drug hydroxyurea.

Sites of hematopoiesis
The principal site of extramedullary hematopoiesis in myelofibrosis is the spleen, which is usually markedly enlarged, sometimes weighing as much as 4000 g. As a result of massive enlargement of the spleen, multiple subcapsular infarcts often occur in the spleen, meaning that due to interrupted oxygen supply to the spleen partial or complete tissue death happens. On the cellular level, the spleen contains red blood cell precursors, granulocyte precursors and megakaryocytes, with the megakaryocytes prominent in their number and in their bizarre shapes. Megakaryocytes are believed to be involved in causing the secondary fibrosis seen in this condition, as discussed under "Mechanism" above. Sometimes unusual activity of the red blood cells, white blood cells, or platelets is seen. The liver is often moderately enlarged, with foci of extramedullary hematopoiesis. Microscopically, lymph nodes also contain foci of hematopoiesis, but these are insufficient to cause enlargement.There are also reports of hematopoiesis taking place in the lungs. These cases are associated with hypertension in the pulmonary arteries.The bone marrow in a typical case is hypercellular and diffusely fibrotic. Both early and late in disease, megakaryocytes are often prominent and are usually dysplastic.

Diagnosis
Epidemiologically, the disorder usually develops slowly and is mainly observed in people over the age of 50.Diagnosis is made on the basis of bone marrow biopsy. Fibrosis grade 2 or 3 defines overt PMF whereas grade 0 or 1 defines prefibrotic primary myelofibrosis.A physical exam of the abdomen may reveal enlargement of the spleen, the liver, or both.Blood tests are also used in diagnosis. Primary myelofibrosis can begin with a blood picture similar to that found in polycythemia vera or chronic myeloid leukemia. Most people with myelofibrosis have moderate to severe anemia. Eventually thrombocytopenia, a decrease of blood platelets develops. When viewed through a microscope, a blood smear will appear markedly abnormal, with presentation of pancytopenia, which is a reduction in the number of all blood cell types: red blood cells, white blood cells, and platelets. Red blood cells may show abnormalities including bizarre shapes, such as teardrop-shaped cells, and nucleated red blood cell precursors may appear in the blood smear (leukoerythroblastic reaction). Normally, mature red blood cells in adults do not have a cell nucleus, and the presence of nucleated red blood cells suggests that immature cells are being released into the bloodstream in response to a very high demand for the bone marrow to produce new red blood cells. Immature white cells and platelets (large megakaryocytes) are also seen in blood samples, and basophil counts are increased. When late in the disease progression an attempt is made to take a sample of bone marrow by aspiration, it may result in a dry tap, meaning that where the needle can normally suck out a sample of semi-liquid bone marrow, it produces no sample because the marrow has been replaced with collagen fibers </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Craniodiaphyseal dysplasia and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is X-linked endothelial corneal dystrophy and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Sinusoidal hemangioma and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Sick building syndrome and explain in detail? [/INST] Sick building syndrome (SBS) is a condition in which people in a building develop symptoms of illness or become infected with chronic disease from the building in which they work or reside. The outbreaks may or may not be a direct result of inadequate cleaning or inappropriate cleaning methods. SBS has also been used to describe staff concerns in post-war buildings with misplanned building aerodynamics, defects in the construction materials or assembly process and-or inadequate maintenance. Certain symptoms tend to increase in severity with the time people spend in the building; often improving over time or even disappearing when people are away from the building. SBS is also used interchangeably with "building-related symptoms", which orients the name of the condition around patients symptoms rather than a "sick" building. A 1984 World Health Organization (WHO) report suggested up to 30% of new and remodeled buildings worldwide may be subject of complaints related to poor indoor air quality.
Other causes have been attributed to contaminants produced by outgassing of some types of building materials, volatile organic compounds (VOC), improper exhaust ventilation of ozone (byproduct of some office machinery), light industrial chemicals used within, or lack of adequate fresh-air intake/air filtration (see minimum efficiency reporting value).The main identifying observation is an increased incidence of complaints of symptoms such as headache, eye, nose, and throat irritation, fatigue, and dizziness and nausea. In fact the 1989 Oxford English Dictionary defines SBS in that way. The World Health Organization created a 484-page tome on indoor air quality back in 1984 when SBS was attributed only to non-organic causes, and suggested that the book might form a basis for legislation or litigation.Case studies can contribute to a better understanding and management of sick building syndrome. Sick building causes are frequently pinned down to flaws in the heating, ventilation, and air conditioning (HVAC) systems. However, there have been inconsistent findings on whether air conditioning systems result in SBS or not.More recently, sick building was confirmed as a vector for the transmission of SARS in 2003. Norovirus has also been linked with buildings because of "the small inoculum required to produce infection (<100 viral particles), prolonged viral shedding, and its ability to survive in the environment." Many norovirus outbreaks have been traced back to cruise ships with food service in unsanitary conditions, or filthy personal hygiene around the toilet.

Signs and symptoms
Human exposure to aerosols has been documented to give rise to a variety of adverse health effects. Building occupants complain of symptoms such as sensory irritation of the eyes, nose, or throat; neurotoxic or general health problems; skin irritation; nonspecific hypersensitivity reactions; infectious diseases; and odor and taste sensations. Exposure to poor lighting conditions has led to general malaise.Extrinsic allergic alveolitis has been associated with the presence of fungi and bacteria in the moist air of residential houses and commercial offices. A study in 2017 correlated several inflammatory diseases of the respiration tract with objective evidence of damp-caused damage in homes.The WHO has classified the reported symptoms into broad categories, including: mucous membrane irritation (eye, nose, and throat irritation), neurotoxic effects (headaches, fatigue, and irritability), asthma and asthma-like symptoms (chest tightness and wheezing), skin dryness and irritation, gastrointestinal complaints and more.Several sick occupants may report individual symptoms which do not appear to be connected. The key to discovery is the increased incidence of illnesses in general with onset or exacerbation within a fairly close time frame – usually within a period of weeks. In most cases, SBS symptoms will be relieved soon after the occupants leave the particular room or zone. However, there can be lingering effects of various neurotoxins, which may not clear up when the occupant leaves the building. In some cases – particularly in sensitive individuals – there can be long-term health effects.

Cause
ASHRAE has recognized that polluted urban air, designated within the United States Environmental Protection Agency (EPA)s air quality ratings as unacceptable, requires the installation of treatment such as filtration for which the HVAC practitioners generally apply carbon-impregnated filters and their likes. Different toxins will aggravate the human body in different ways. Some people are more allergic to mold, while others are highly sensitive to dust. Inadequate ventilation will exaggerate small problems (such as deteriorating fiberglass insulation or cooking fumes) into a much more serious indoor air quality problem. Common products such as paint, insulation, rigid foam, Particle Board, plywood, duct liners, exhaust fumes and other chemical contaminants from indoor or outdoor sources, and biological contaminants can be trapped inside by the HVAC AC system. As this air is recycled using fan coils the overall oxygenation ratio drops and becomes harmful. When combined with other stress factors such as traffic noise, poor lighting, inhabitants of buildings located in a polluted urban area can quickly become ill as their immune system is overwhelmed. Certain VOCs, considered toxic chemical contaminants to humans, are used as adhesives in all common building construction products. These aromatic carbon rings / VOCs can cause acute and chronic health effects on the occupants of a building, including cancer, paralysis, lung failure, and others. Bacterial spores, fungal spores, mold spores, pollen, and viruses are types of biological contaminants and can all cause allergic reactions or illness described as SBS. In addition, pollution from outdoors, such as motor vehicle exhaust, can infiltrate into poorly designed buildings and contribute to poor indoor air quality, high ppm of CO and CO2. Adult SBS symptoms were associated with a history of allergic rhinitis, eczema and asthma.A 2015 study concerning the association of SBS and indoor air pollutants in office buildings in Iran found that, as carbon dioxide levels increase in a building, symptoms like nausea, headaches, nasal irritation, dyspnea, and throat dryness have also been shown to increase. Certain work conditions have been found to be correlated with specific symptoms. For example, higher light intensity was significantly related to skin dryness, eye pain, and malaise. Higher temperature has also been found to correlate with symptoms such as sneezing, skin redness, itchy eyes and headache, while lower relative humidity has been associated with sneezing, skin redness, and pain of the eyes.In 1973, in response to the oil crisis and conservation concerns, ASHRAE Standards 62-73 and 62-81 reduced required ventilation from 10 cubic feet per minute (4.7 L/s) per person to 5 cubic feet per minute (2.4 L/s) per person, but this was found to be a contributing factor to sick building syndrome. As of the 2016 revision, ASHRAE ventilation standards call for 5 to 10 cubic feet per minute of ventilation per occupant (depending on the occupancy type) in addition to ventilation based on the zone floor area delivered to the breathing zone.

Psychological factors
One study looked at commercial buildings and their employees, comparing some environmental factors suspected of inducing SBS to a self-reported survey of the occupants, finding that the measured psycho-social circumstances appeared more influential than the tested environmental factors. The authors of that study pointed to the self-reported nature of the observations as a limitation of the research.Research has shown that SBS shares several symptoms common in other conditions thought to be at least partially caused by psychosomatic tendencies. The umbrella term "autoimmune/inflammatory syndrome induced by adjuvants" has been suggested. Other members of the suggested group include silicosis, macrophagic myofascitis, Gulf War syndrome, and post-vaccination phenomena.

Workplace
Greater effects were found with features of the psycho-social work environment including high job demands and low support. The report concluded that the physical environment of office buildings appears to be less important than features of the psycho-social work environment in explaining differences in the prevalence of symptoms. However, there is still a relationship between sick building syndrome and symptoms of workers regardless of workplace stress.Excessive work stress or dissatisfaction, poor interpersonal relationships and poor communication are often seen to be associated with SBS, recent studies show that a combination of environmental sensitivity and stress can greatly contribute to sick building syndrome.Specific work-related stressors are related with specific SBS symptoms. Workload and work conflict are significantly associated with general symptoms (headache, abnormal tiredness, sensation of cold or nausea). While crowded workspaces and low work satisfaction are associated with upper respiratory symptoms. Work productivity has been associated with ventilation rates, a contributing factor to SBS, and theres a significant increase in production as ventilation rates increase, by 1.7% for every two-fold increase of ventilation rate. Printer effluent, released into the office air as ultra-fine particles (UFPs) as toner is burned during the printing process, may lead to certain SBS symptoms. Printer effluent may contain a variety of toxins to which a subset of office workers are sensitive, triggering SBS symptoms.Specific careers are also associated with specific SBS symptoms. Transport, communication, healthcare, and social workers have highest prevalence of general symptoms. Skin symptoms such as eczema, itching, and rashes on hands and face are associated with technical work. Forestry, agriculture, and sales workers have the lowest rates of sick building syndrome symptoms.From the assessment done by Fisk and Mudarri, 21% of asthma cases in the United States were caused by wet environments with mold that exist in all indoor environments, such as schools, office buildings, houses and apartments. Also found the exposure to the mold increases the chances of respiratory issues by 30 to 50 percent. Additionally, studies showing that health effects with dampness and mold in indoor environments found that increased risk of adverse health effects occurs with dampness or visible mold environments.Milton et al. determined the cost of sick leave specific for one business was an estimated $480 per employee, and about five days of sick leave per year could be attributed to low ventilation rates. When comparing low ventilation rate areas of the building to higher ventilation rate areas, the relative risk of short-term sick leave was 1.53 times greater in the low ventilation areas.

Home
Sick building syndrome can also occur due to factors of the home. Laminate flooring can cause more exposure to chemicals and more resulting SBS symptoms compared to stone, tile, and cement flooring. Recent redecorating and new furnishings within the last year were also found to be associated with increased symptoms, along with dampness and related factors, having pets, and the presence of cockroaches. The presence of mosquitoes was also a factor related to more symptoms, though it is unclear whether it was due to the presence of mosquitoes or the use of repellents.

Mold
Some studies have found that Sick building syndrome may be associated with indoor mould or mycotoxin contamination. However, the attribution of sick building syndrome to mould is controversial and supported by little evidence.
 Reducing the dampness in all indoor environment will prevent all the respiratory issues caused by dampness and mold in indoor environment.

Indoor temperature
Indoor temperature under 18 °C (64 °F) has been shown to be associated with increased respiratory and cardiovascular diseases, increased blood levels, and increased hospitalization.

Diagnosis
While sick building syndrome (SBS) encompasses a multitude of non-specific symptoms, building-related illness (BRI) comprises specific, diagnosable symptoms caused by certain agents (chemicals, bacteria, fungi, etc.). These can typically be identified, measured, and quantified. There are usually 4 causal agents in BRI; 1.) Immunologic, 2.) Infectious, 3.) toxic, and 4.) irritant. For instance, Legionnaires disease, usually caused by Legionella pneumophila, involves a specific organism which could be ascertained through clinical findings as the source of contamination within a building.

Prevention
Regular inspections to indicate for presence of mold or other toxins
Adequate maintenance of all building mechanical systems
Toxin-absorbing plants, such as sansevieria
Roof shingle non-pressure cleaning for removal of algae, mold, and Gloeocapsa magma
Using ozone to eliminate the many sources, such as VOCs, molds, mildews, bacteria, viruses, and even odors. However, numerous studies identify high-ozone shock treatment as ineffective despite commercial popularity and popular belief.
Replacement of water-stained ceiling tiles and carpeting
Only using paints, adhesives, solvents, and pesticides in well-ventilated areas or only using these pollutant sources during periods of non-occupancy
Increasing the number of air exchanges; the American Society of Heating, Refrigeration and Air-Conditioning Engineers recommend a minimum of 8.4 air exchanges per 24-hour period
Increased ventilation rates that are above the minimum guidelines
Proper and frequent maintenance of HVAC systems
UV-C light in the HVAC plenum
Installation of HVAC air cleaning systems or devices to remove VOCs and bioeffluents (people odors)
Central vacuums that completely remove all particles from the house including the ultrafine particles (UFPs) which are less than 0.1 μm
Regular vacuuming with a HEPA filter vacuum cleaner to collect and retain 99.97% of particles down to and including 0.3 micrometers
Placing bedding in sunshine, which is related to a study done in a high-humidity area where damp bedding was common and associated with SBS
Lighting in the workplace should be designed to give individuals control, and be natural when possible
Relocating office printers outside the air conditioning boundary, perhaps to another building
Replacing current office printers with lower emission rate printers
Identification and removal of products containing harmful ingredients

Management
SBS, as a non specific blanket term, does not have any specific cause or cure. Any known cure would be associated with the specific eventual disease that was cause by exposure to known contaminants. In all cases, alleviation consists of removing the affected person from the building associated. BRI, on the other hand, utilizes treatment appropriate for the contaminant identified within the building (e.g., antibiotics for Legionnaires disease).In most cases, simply improving the indoor air quality (IAQ) of a particular building will attenuate, or even eliminate, the continued exposure to toxins. For the individual, the recovery may be a process involved with targeting the acute symptoms of a specific illness, as in the case of mold toxins. Treating various building-related illnesses is vital to the overall understanding of SBS. Careful analysis by certified building professionals and Medical Doctors can help to identify the exact cause of the BRI, and help to illustrate a causal path to infection. With this knowledge one can, theoretically, remediate a building of contaminants and rebuild the structure with new materials. Office BRI may more likely than not be explained by three events: "Wide range in the threshold of response in any population (susceptibility), a spectrum of response to any given agent, or variability in exposure within large office buildings." Isolating any one of the three aspects of office BRI can be a great challenge, which is why those who find themselves with BRI should take three steps, history, examinations, and interventions. History describes the action of continually monitoring and recording the health of workers experiencing BRI, as well as obtaining records of previous building alterations or related activity. Examinations go hand in hand with monitoring employee health. This step is done by physically examining the entire workspace and evaluating possible threats to health status among employees. Interventions follow accordingly based on the results of the Examination and History report.

Epidemiology
Some studies have found that women have higher reports of SBS symptoms than men. It is not entirely clear, however, if this is due to biological, social, or occupational factors.
A 2001 study published in the Journal Indoor Air, gathered 1464 office-working participants to increase the scientific understanding of gender differences under the Sick Building Syndrome phenomenon. Using questionnaires, ergonomic investigations, building evaluations, as well as physical, biological, and chemical variables, the investigators obtained results that compare with past studies of SBS and gender. The study team found that across most test variables, prevalence rates were different in most areas, but there was also a deep stratification of working conditions between genders as well. For example, mens workplaces tend to be significantly larger and have all-around better job characteristics. Secondly, there was a noticeable difference in reporting rates, specifically that women have higher rates of reporting roughly 20% higher than men. This information was similar to that found in previous studies, thus indicating a potential difference in willingness to report.There might be a gender difference in reporting rates of sick building syndrome, because women tend to report more symptoms than men do. Along with this, some studies have found that women have a more responsive immune system and are more prone to mucosal dryness and facial erythema. Also, women are alleged by some to be more exposed to indoor environmental factors because they have a greater tendency to have clerical jobs, wherein they are exposed to unique office equipment and materials (example: blueprint machines, toner-based printers), whereas men often have jobs based outside of offices.

History
In the late 1970s, it was noted that nonspecific symptoms were reported by tenants in newly constructed homes, offices, and nurseries. In media it was called "office illness". The term "sick building syndrome" was coined by the WHO in 1986, when they also estimated that 10–30% of newly built office buildings in the West had indoor air problems. Early Danish and British studies reported symptoms.
Poor indoor environments attracted attention. The Swedish allergy study (SOU 1989:76) designated "sick building" as a cause of the allergy epidemic as was feared. In the 1990s, therefore, extensive research into "sick building" was carried out. Various physical and chemical factors in the buildings were examined on a broad front.
The problem was highlighted increasingly in media and was described as a "ticking time bomb". Many studies were performed in individual buildings.
In the 1990s "sick buildings" were contrasted against "healthy buildings". The chemical contents of building materials were highlighted. Many building material manufacturers were actively working to gain control of the chemical content and to replace criticized additives. The ventilation industry advocated above all more well-functioning ventilation. Others perceived ecological construction, natural materials, and simple techniques as a solution.
At the end of the 1990s came an increased distrust of the concept of "sick building". A dissertation at the Karolinska Institutet in Stockholm 1999 questioned the methodology of previous research, and a Danish study from 2005 showed these flaws experimentally. It was suggested that sick building syndrome was not really a coherent syndrome and was not a disease to be individually diagnosed, but a collection of as many as to dozen semi related diseases. In 2006 the Swedish National Board of Health and Welfare recommended in the medical journal Läkartidningen that "sick building syndrome" should not be used as a clinical diagnosis. Thereafter, it has become increasingly less common to use terms such as "sick buildings" and "sick building syndrome" in research. However, the concept remains alive in popular culture and is used to designate the set of symptoms related to poor home or work environment engineering. "Sick building" is therefore an expression used especially in the context of workplace health.
Sick building syndrome made a rapid journey from media to courtroom where professional engineers and architects became named defendants and were represented by their respective professional practice insurers. Proceedings invariably relied on expert witnesses, medical and technical experts along with building managers, contractors and manufacturers of finishes and furnishings, testifying as to cause and effect. Most of these actions resulted in sealed settlement agreements, none of these being dramatic. The insurers needed a defense based upon Standards of Professional Practice to meet a court decision that declared that in a modern, essentially sealed building, the HVAC systems must produce breathing air for suitable human consumption. ASHRAE (American Society of Heating, Refrigeration and Air Conditioning Engineers, currently with over 50,000 international members) undertook the task of codifying its indoor air quality (IAQ) standard.
ASHRAE empirical research determined that "acceptability" was a function of outdoor (fresh air) ventilation rate and used carbon dioxide as an accurate measurement of occupant presence and activity. Building odors and contaminants would be suitably controlled by this dilution methodology. ASHRAE codified a level of 1,000 ppm of carbon dioxide and specified the use of widely available sense-and-control equipment to assure compliance. The 1989 issue of ASHRAE 62.1-1989 published the whys and wherefores and overrode the 1981 requirements that were aimed at a ventilation level of 5,000 ppm of carbon dioxide, (the OAHA workplace limit), federally set to minimize HVAC system energy consumption. This apparently ended the SBS epidemic.
Over time, building materials changed with respect to emissions potential. Smoking vanished and dramatic improvements in ambient air quality, coupled with code compliant ventilation and maintenance, per ASHRAE standards have all contributed to the acceptability of the indoor air environment.

See also
Aerotoxic syndrome
Havana syndrome
Healthy building
Multiple chemical sensitivity
NASA Clean Air Study
Nosocomial infection
Somatization disorder
Fan death

References
Further reading
Martín-Gil J., Yanguas M. C., San José J </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Dysexecutive syndrome and explain in detail? [/INST] Dysexecutive syndrome (DES) consists of a group of symptoms, usually resulting from brain damage, that fall into cognitive, behavioural and emotional categories and tend to occur together. The term was introduced by Alan Baddeley to describe a common pattern of dysfunction in executive functions, such as planning, abstract thinking, flexibility and behavioural control. It is thought to be Baddeleys hypothesized working memory system and the central executive that are the hypothetical systems impaired in DES. The syndrome was once known as frontal lobe syndrome; however dysexecutive syndrome is preferred because it emphasizes the functional pattern of deficits (the symptoms) over the location of the syndrome in the frontal lobe, which is often not the only area affected.
Classification code in ICD-10 - F07

Symptoms and signs
Symptoms of DES fall into three broad categories: cognitive, emotional and behavioural. Many of the symptoms can be seen as a direct result of impairment to the central executive component of working memory, which is responsible for attentional control and inhibition. Although many of the symptoms regularly co-occur, it is common to encounter patients who have several, but not all symptoms. The accumulated effects of the symptoms have a large impact on daily life.

Cognitive symptoms
Cognitive symptoms refer to a persons ability to process thoughts. Cognition primarily refers to memory, the ability to learn new information, speech, and reading comprehension. Deficits within this area cause many problems with everyday life decisions.One of the main difficulties for an individual with DES is planning and reasoning. Impaired planning and reasoning affect the individuals ability to realistically assess and manage the problems of everyday living. New problems and situations may be especially poorly handled because of the inability to transfer previous knowledge to the new event. An individual that has DES may have a short attention span due to impairment in attentional control. This may alter the individuals ability to focus, and as such have difficulty with reading and following a storyline or conversation. For instance, they can easily lose track of conversations which can make it difficult to hold a meaningful conversation and may result in avoiding social interactions.Individuals with DES will have very poor working memory and short term memory due to executive dysfunction. The dysfunction can range from mild and subtle to severe and obvious. There is a tremendous variability in the manifestations of executive dysfunction with strong influences often apparent from the affected persons personality, life experiences and intellect. Individuals with DES may experience confabulation, which is the spontaneous reporting of events that never happened. This can affect their autobiographical memory. It is thought that patients may not be able to assess the accuracy of memory retrieval and therefore elaborate on implausible memories.Individuals with dementia, delirium or other severe psychiatric illnesses combined with DES often have disturbed sleep patterns. Some will not recognize that it is night-time and may become upset when someone tries to correct them.

Emotional symptoms
The emotional symptoms that individuals with DES experience may be quite extreme and can cause extensive problems. They may have difficulty inhibiting many types of emotions such as anger, excitement, sadness, or frustration. Due to multiple impairments of cognitive functioning, there can be much more frustration when expressing certain feelings and understanding how to interpret everyday situations. Individuals with DES may have higher levels of aggression or anger because they lack abilities that are related to behavioural control. They can also have difficulty understanding others points of view, which can lead to anger and frustration.

Behavioural symptoms
Behavioural symptoms are evident through an individuals actions. People with DES often lose their social skills because their judgments and insights into what others may be thinking are impaired. They may have trouble knowing how to behave in group situations and may not know how to follow social norms. The central executive helps control impulses; therefore when impaired, patients have poor impulse control. This can lead to higher levels of aggression and anger. DES can also cause patients to appear self-centered and stubbornUtilization behaviour is when a patient automatically uses an object in the appropriate manner, but at an inappropriate time. For example, if a pen and paper are placed in front of an individual with DES they will start to write or if there is a deck of cards they will deal them out. Patients showing this symptom will begin the behaviour in the middle of conversations or during auditory tests. Utilization behaviour is thought to occur because an action is initiated when an object is seen, but patients with DES lack the central executive control to inhibit acting it out at inappropriate times.Perseveration is also often seen in patients with DES. Perseveration is the repetition of thoughts, behaviours, or actions after they have already been completed. For instance, continually blowing out a match, after it is no longer lit is an example of perseveration behaviour. There are three types of perseveration: continuous perseveration, stuck-in-set perseveration, and recurrent perseveration. Stuck-in-set perseveration is most often seen in dysexecutive syndrome. This type of perseveration refers to when a patient cannot get out of a specific frame of mind, such as when asked to name animals they can only name one. If you ask them to then name colours, they may still give you animals. Perseveration may explain why some patients appear to have obsessive-compulsive disorder.

Comorbid disorders
DES often occurs with other disorders, which is known as comorbidity. Many studies have examined the presence of DES in patients with schizophrenia. Results of schizophrenic patients on the Behavioural Assessment of the Dysexecutive Syndrome (BADS) test (discussed below) are comparable to brain injured patients. Further, results of BADS have been shown to correlate with phases of schizophrenia. Patients in the chronic phase of the disorder have significantly lower scores than those who are acute. This is logical due to the similarities in executive disruptions that make everyday life difficult for those with  schizophrenia and symptoms that form DES.
Patients with Alzheimers disease and other forms of dementia have been shown to exhibit impairment in executive functioning as well. The effects of DES symptoms on the executive functions and working memory, such as attentiveness, planning and remembering recently learned things, are some of the earliest indicators of Alzheimers disease and dementia with Lewy bodies.
Studies have also indicated that chronic alcoholism (see Korsakoffs syndrome) can lead to a mild form of DES according to results of BADS.

Causes
The most frequent cause of the syndrome is brain damage to the frontal lobe. Brain damage leading to the dysexecutive pattern of symptoms can result from physical trauma such as a blow to the head or a stroke  or other internal trauma.
It is important to note that frontal lobe damage is not the only cause of the syndrome. It has been shown that damage, such as lesions, in other areas of the brain may indirectly affect executive functions and lead to similar symptoms (such as ventral tegmental area, basal ganglia and thalamus). There is not one specific pattern of damage that leads to DES, as multiple affected brain structures and locations have led to the symptoms. This is one reason why the term frontal lobe syndrome is not preferred.

Diagnosis
Assessment of patients with DES can be difficult because traditional tests generally focus on one specific problem for a short period of time. People with DES can do fairly well on these tests because their problems are related to integrating individual skills into everyday tasks. The lack of everyday application of traditional tests is known as low ecological validity.

Behavioural
The Behavioural Assessment of the Dysexecutive Syndrome (BADS) was designed to address the problems of traditional tests and evaluate the everyday problems arising from DES. BADS is designed around six subtests and ends with the Dysexecutive Questionnaire (DEX). These tests assess executive functioning in more complex, real-life situations, which improves their ability to predict day-to-day difficulties of DES.

The six tests are as follows:Rule Shift Cards - Assesses the subjects ability to ignore a prior rule after being given a new rule to follow.
Action Program - This test requires the use of problem solving to accomplish a new, practical task.
Key Search - This test reflects the real-life situation of needing to find something that has been lost. It assesses  the patients ability to plan how to accomplish the task and monitor their own progress.
Temporal Judgment - Patients are asked to make estimated guesses to a series of questions such as, "how fast do racehorses gallop?". It tests the ability to make sensible guesses.
Zoo Map - Tests the ability to plan while following a set of rules.
Modified Six Elements - This test assesses the subjects ability to plan, organize and monitor behaviour.The Dysexecutive Questionnaire (DEX) is a 20-item questionnaire designed to sample emotional, motivational, behavioural and cognitive changes in a subject with DES. One version is designed for the subject to complete and another version is designed for someone who is close to the individual, such as a relative or caregiver. Instructions are given to the participant to read 20 statements describing common problems of everyday life and to rate them according to their personal experience. Each item is scored on a 5-point scale according to its frequency from never (0 point) to very often (4 points).

Treatment
There is no cure for individuals with DES, but there are therapies to help them cope with their symptoms. DES can affect a number of functions in the brain and vary from person to person. Because of this variance, it is suggested that the most successful therapy would include multiple methods. Researchers suggest that a number of factors in the executive functioning need to be improved, including self-awareness, goal setting, planning, self-initiation, self-monitoring, self-inhibition, flexibility, and strategic behaviour.
One method for individuals to improve in these areas is to help them plan and carry out actions and intentions through a series of goals and sub-goals. To accomplish this, therapists teach patients a three-step model called the General Planning Approach. The first step is Information and Awareness, in which the patients are taught about their own problems and shown how this affects their lives. The patients are then taught to monitor their executive functions and begin to evaluate them.  The second stage, Goal Setting and Planning, consists of patients making specific goals, as well as devising a plan to accomplish them. For example, patients may decide they will have lunch with a friend (their goal). They are taught to write down which friend it may be, where they are going for lunch, what time they are going, how they will get there, etc. (sub-goals). They are also taught to make sure the steps go in the correct order. The final stage, named Initiation, Execution, and Regulation, requires patients to implement their goals in their everyday lives. Initiation can be taught through normal routines. The first step can cue the patient to go to the next step in their plan. Execution and regulation are put into action with reminders of how to proceed if something goes wrong in the behavioural script. This treatment method has resulted in improved daily executive functioning, however no improvements were seen on formal executive functioning tests.
Since planning is needed in many activities, different techniques have been used to improve this deficit in patients with DES. Autobiographical memories can be used to help direct future behaviour. You can draw on past experiences to know what to do in the future. For example, when you want to take a bus, you know from past experience that you have to walk to the bus stop, have the exact amount of change, put the change in the slot, and then you can go find a seat. Patients with DES seem to not be able to use this autobiographical memory as well as a normal person. Training for DES patients asks them to think of a specific time when they did an activity previously. They are then instructed to think about how they accomplished this activity. An example includes "how would you plan a holiday". Patients are taught to think of specific times they went on a holiday and then to think how they may have planned these holidays. By drawing on past experiences patients were better able to make good decisions and plans.
Cognitive Analytic Therapy (CAT) has also been used to help those with DES. Because individuals with this syndrome have trouble integrating information into their actions it is often suggested that they have programmed reminders delivered to a cell phone or pager. This helps them remember how they should behave and discontinue inappropriate actions. Another method of reminding is to have patients write a letter to themselves. They can then read the letter whenever they need to. To help patients remember how to behave, they may also create a diagram </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Nasolacrimal duct obstruction and explain in detail? [/INST] Nasolacrimal duct obstruction is the obstruction of the nasolacrimal duct and may be either congenital or acquired. Obstruction of the nasolacrimal duct leads to the excess overflow of tears called epiphora.

Sign and symptoms
Excessive tearing is the most common complaint of patients with nasolacrimal duct obstruction, followed by acute or chronic infections.  Pain at the side of the nose suggests dacryocystitis.
Nasolacrimal duct obstruction is more common with increasing age and more common in females than males.

Cause
Involutional stenosis
Involutional stenosis is probably the most common cause of nasolacrimal duct obstruction in older people. It affects women twice as frequently as men. Although the inciting event in this process is unknown, clinicopathologic study suggests that compression of the lumen of the nasolacrimal duct is caused by inflammatory infiltrates and edema. This may be the result of an unidentified infection or possibly an autoimmune disease.

Dacryolith
Dacryoliths or cast formation, within the lacrimal sac can also produce obstruction of the nasolacrimal duct.

Sinus disease
Sinus disease often occurs in conjunction with, and in other instances may contribute to the development of nasolacrimal duct obstruction. Patients should be asked about previous sinus surgery, as the nasolacrimal duct is sometimes damaged when the maxillary sinus ostium is being enlarged anteriorly.

Trauma
Naso-orbital fractures may involve the nasolacrimal duct. Early treatment by fracture reduction with stenting of the entire lacrimal drainage system should be considered. However, such injuries are often not recognized or are initially neglected as more serious injuries are managed. In such cases, late treatment of persistent epiphora usually requires dacryocystorhinostomy.

Inflammatory disease
Granulomatous disease, including sarcoidosis, granulomatosis with polyangiitis, and  midline granuloma, may also lead to nasolacrimal duct obstruction.

Lacrimal plugs
As with similar cases of canalicular obstruction, dislodged punctual and canalicular plugs can migrate to and occlude the nasolacrimal duct.

Neoplasm
Neoplasm should be considered in any patient presenting with nasolacrimal duct obstruction. In patients with atypical presentations, including younger age and male gender, further workup is appropriate. Bloody punctual discharge or lacrimal sac distension above the medial canthal tendon is also highly suggestive of neoplasm.

Congenital
Congenital nasolacrimal duct obstruction, or dacryostenosis, occurs when the lacrimal duct has failed to open at the time of birth, most often due to an imperforate membrane at the valve of Hasner. Around 6% of infants have congenital nasolacrimal duct obstruction, or dacryostenosis, usually experiencing a persistent watery eye even when not crying. If a secondary infection occurs (Dacryocystitis), purulent (yellow / green) discharge may be present.
Most cases resolve spontaneously, with antibiotics reserved only if conjunctivitis occurs. Lacrimal sac massage has been proposed as helping to open the duct, though this is not always successful. The aim of massage is to generate enough hydrostatic pressure (downward, toward the nose) to "pop" open any obstruction.  Additional massage may then be performed up toward the lacrimal punctum, in order to express any infectious material out of the nasolacrimal sac.  When discharge or crusting is present, the lids should be gently cleaned using cooled pre-boiled water or saline.
Referral to an ophthalmologist is indicated if symptoms are still present at 12 months, or sooner if significant symptoms or recurrent infections occur.  Nasolacrimal duct probing may be performed in the office setting (usually from 4 to 8 months of age) or under general anesthesia in an operating room for older patients.  The success rate of probing is higher for younger children.  A silastic tube or stent may be employed along with probing to maintain tear duct patency. A systematic review comparing immediate probing with deferred probing found that in children with unilateral nasolacrimal duct obstruction, immediate probing resulted in a higher success rate of treatment compared to deferred probing.

Diagnosis
Evaluation is in the form of a dye disappearance test followed by irrigation test. By using this sequence (with modifications) as a guide, the physician can frequently streamline diagnostic testing.

Dye disappearance test
The dye disappearance test is useful for assessing the presence or absence of adequate lacrimal outflow, especially in unilateral cases. It is more heavily relied upon in children, in whom lacrimal irrigation is impossible without deep sedation. Using a drop of sterile 2% fluorescein solution or a moistened fluorescein strip, the examiner instills fluorescein into the conjunctival fornices of each eye and then observes the tear film, preferably with the cobalt blue filter of the slit lamp </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Bronchocele and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Hypertrophic osteoarthropathy and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Epidermal nevus syndrome and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Sacroiliac joint dysfunction and explain in detail? [/INST] Sacroiliac joint dysfunction generally refers to pain in the sacroiliac joint region that is caused by abnormal motion in the sacroiliac joint, either too much motion or too little motion.

Signs and symptoms
Common symptoms include lower back pain, buttocks pain, sciatic leg pain, groin pain, hip pain (for explanation of leg, groin, and hip pain, see referred pain), urinary frequency, and "transient numbness, prickling, or tingling."  Pain can range from dull aching to sharp and stabbing and increases with physical activity.  Symptoms also worsen with prolonged or sustained positions (i.e., sitting, standing, lying).  Bending forward, stair climbing, hill climbing, and rising from a seated position can also provoke pain. Pain can increase during menstruation in women.  People with severe and disabling sacroiliac joint dysfunction can develop insomnia and depression. Sacral torsion that is untreated over a long period of time can cause severe Achilles tendinosis.

Causes
Hypermobility
Sacroiliac joint dysfunction is an outcome of either extra-articular dysfunction or from intraarticular dysfunction.
SI joint dysfunction is sometimes referred to as "sacroiliac joint instability" or "sacroiliac joint insufficiency"  due to the support the once strong and taut ligaments can no longer sustain. When the joint is hypermobile or loose, it is classified as an extra-articular dysfunction because abnormal joint movement and alignment is a consequence of weakened, injured, or sprained ligaments, while the joint itself is structurally normal and healthy. The sacroiliac joint itself often will not show degenerative changes, such as arthritis, until many years of the dysfunction being allowed to continue. Injury to the ligaments that hold the sacroiliac joints in proper support is thought to be caused by a torsion or high impact injury (such as an automobile accident) or a hard fall, resulting in the hypermobility.  As many as 58% of people diagnosed with sacroiliac joint pain had some inciting traumatic injury based on clinical examination findings.  The joint that was once stabilized by strong ligaments, now overly stretched, sprained, or torn, will move beyond its normal range. This is thought to result in the ilium and sacral surfaces "locking" in an incongruent or asymmetrical fashion (one innominate bone is tilted anteriorly; the other innominate bone is tilted posteriorly) causing pain that can be debilitating.Hormone imbalances, particularly those associated with pregnancy and the hormone relaxin, can also cause a ligamentous laxity resulting in the weakening of the sacroiliac structure.
  During pregnancy, relaxin serves as natures way of allowing the female pelvis to achieve distention of the birthing canal. Pelvic joint pain in post pregnancy women is thought to be derived from the inability of the stretched out ligaments to return to normal tautness. Women who have delivered large babies or who have had extended labors also are prone to developing chronic sacroiliac joint pain and instability.In some people, the sacroiliac joints reverse the normal concave-convex locking relationship, which can lead to rotational misalignment.  The variation in joint configuration results in some sacroiliac joints being inherently weaker or more prone to misalignment.Certain biomechanical or muscle length imbalances may ultimately predispose a person to sacroiliac dysfunction and pain. Likely, this is a result of altered gait patterns and repetitive stress to the SI joint and related structures. These conditions exist in persons with leg-length inequality, scoliosis, a history of polio, poor-quality footwear, and hip osteoarthritis.There is also a notable incidence of lumbar spinal fusion patients that present with sacroiliac pain and hypermobility, potentially due to the adjacent lumbar joints being fixed and unable to move. Clinical studies have found up to 75% of post-lumbar fusion patients develop SI joint degeneration within five years of surgery.

Hypomobility
Pathological hypomobility (too little movement) of the sacroiliac joint is an intra-articular disorder in which the joint locks due to wearing down with age or degenerative joint disease.Hypomobility of this kind can also occur with an inflammatory disease such as ankylosing spondylitis, rheumatoid arthritis, or an infection.

Pathophysiology
The sacroiliac joint is a true diarthrodial joint that joins the sacrum to the pelvis.  The sacrum connects on the right and left sides to the ilia (pelvic bones) to form the sacroiliac joints. The pelvic girdle is made up of two innominate bones (the iliac bones) and the sacrum. The innominate bones join in the front of the pelvis to form the pubic symphysis, and at back of the sacrum to form the sacroiliac (SI) joints. Each innominate bone (ilium) joins the femur (thigh bone) to form the hip joint; thus the sacroiliac joint moves with walking and movement of the torso.In this joint, hyaline cartilage on the sacral side moves against fibrocartilage on the iliac side. The sacroiliac joint contains numerous ridges and depressions that function in stability. Studies have documented that motion does occur at the joint; therefore, slightly subluxed and even locked positions can occur.Muscles and ligaments surround and attach to the SI joint in the front and back, primarily on the ilial or sacral surfaces. These can all be a source of pain and inflammation if the SI joint is dysfunctional.  The sacroiliac joint is highly dependent on its strong ligamentous structure for support and stability. The most commonly disrupted and/or torn ligaments are the iliolumbar ligament and the posterior sacroiliac ligament. The ligamentous structures offer resistance to shear and loading. The deep anterior, posterior, and interosseous ligaments resist the load of the sacrum relative to the ilium.  More superficial ligaments (e.g., the sacrotuberous ligament) react to dynamic motions (such as straight-leg raising during physical motion). The long dorsal sacroiliac ligament can become stretched in periods of increased lumbar lordosis (e.g., during pregnancy).

Affected muscle groups
Many large and small muscles have relationships with the ligaments of the sacroiliac joint including the piriformis (see "piriformis syndrome", a condition often related with sacroiliac joint dysfunction), rectus femoris, gluteus maximus and minimus, erector spinae, latissimus dorsi, thoracolumbar fascia, and iliacus.  Any of these muscles can be involved or spasm with a painful and dysfunctional sacroiliac joint.  The SI joint is a pain-sensitive structure richly innervated by a combination of unmyelinated free nerve endings and the posterior primary rami of spinal segments L2-S3. The wide possibility of innervation may explain why pain originating from the joint can manifest in so many various ways, with different and unique referral patterns (see "referred pain") for individual patients. Patients with sacroiliac joint dysfunction can also develop tightness and dysfunction in the hamstring, quadriceps, iliotibial tract (see "iliotibial band syndrome") and hip flexors, including the psoas muscle. Individuals with severe and long-standing sacroiliac joint dysfunction can develop muscle deconditioning and atrophy throughout the body due to limitation of activities and exercise that bring about pain in the low back.

Diagnosis
Perhaps the biggest reason for misdiagnosis or lack of diagnosis of sacroiliac joint dysfunction is based on the inability of common radiological imaging to discern the disorder. Diagnostic testing, such as X-ray, CT scan, or MRI, do not usually reveal abnormalities; therefore, they cannot reliably be used for diagnosis of sacroiliac joint dysfunction. There is a new imaging test SPECT/CT which can sometimes detect sacroiliac joint dysfunction. There is also a lack of evidence that sacroiliac joint mobility maneuvers (Gillet, Standing flexion test, and Seated Flexion test) detect motion abnormalities. Given the inherent technical limitations of the visible and palpable signs from these sacroiliac joint mobility maneuvers another broad category of clinical signs have been described called provocative maneuvers. These maneuvers are designed to reproduce or increase pain emanating within the sacroiliac joint.A clinician (i.e., a spine surgeon, orthopedic surgeon, sports medicine doctor, athletic trainer, medical massage therapist, physical therapist, physiatrist, osteopath or chiropractor) can develop a probable diagnosis of sacroiliac joint dysfunction by using a hands on approach through palpating the painful areas and performing the  following provocative maneuvers below:
Gaenslen test  - This pain provocation test applies torsion to the joint. With one hip flexed onto the abdomen, the other leg is allowed to dangle off the edge of the table. Pressure should then be directed downward on the leg in order to achieve hip extension and stress the sacroiliac joint.
Iliac Gapping Test - Distraction can be performed to the anterior sacroiliac ligaments by applying pressure to the anterior superior iliac spine.
Iliac Compression Test - Apply compression to the joint with the patient lying on his or her side. Pressure is applied downward to the uppermost iliac crest.
FABER or Patrick test - To identify if pain may come from the sacroiliac joint during flexion, abduction, and external rotation, the clinician externally rotates the hip while the patient lies supine. Then, downward pressure is applied to the medial knee stressing both the hip and sacroiliac joint.
Thigh Thrust - This test applies anteroposterior shear stress on the SI joint. The patient lies supine with one hip flexed to 90 degrees. The examiner stands on the same side as the flexed leg. The examiner provides either a quick thrust or steadily increasing pressure through the line of the femur. The pelvis is stabilized at the sacrum or at the opposite ASIS with the hand of the examinerCautious interpretation is warranted because there are no biomechanical studies showing that the Thigh Thrust test isolates forces in the SIJ when performed at 90 degrees and due to intra-individual variation in body type, hip flexibility, general flexibility of the trunk and pelvis.
In all the tests, pain along the typical area raises suspicion for sacroiliac joint dysfunction. However no single test is very reliable in the diagnosis of sacroiliac joint dysfunction.  It is important to remember true neurogenic weakness, numbness, or loss of reflex should alert the clinician to consider nerve root pathology.The current "gold standard" for diagnosis of sacroiliac joint dysfunction emanating within the joint is sacroiliac joint injection confirmed under fluoroscopy or CT-guidance using a local anesthetic solution.  The diagnosis is confirmed when the patient reports a significant change in relief from pain and the diagnostic injection is performed on 2 separate visits.  Published studies have used at least a 75 percent change in relief of pain before a response is considered positive and the sacroiliac joint deemed the source of pain.
However, several other injection studies have compared intra-articular with extra-articular injection, and indicate that the ligament injection behind the joint is oftentimes superior to injection in the joint and seems to be a very underutilized diagnostic tool.

Misdiagnosis
In the early 1900s, dysfunction of the sacroiliac joint was a common diagnosis associated with low back and sciatic nerve pain.  However, research by Danforth and Wilson in 1925 concluded that the sacroiliac joint could not cause sciatic nerve pain because the joint does not have a canal in which the nerves can be entrapped against the joint. The biomechanical relationship between the sacroiliac joint, the piriformis muscle (see "piriformis syndrome"), and the sciatic nerve had not yet been discovered.In 1934, the work of Mixter and Barr shifted all emphasis in research and treatment from the sacroiliac to the herniated intervertebral disc, namely lumbar discs. Medical focus on herniated discs was further forwarded by the invention of the MRI in 1977. Over-diagnosis and attention on herniated discs has led to the SI joint becoming an underappreciated pain generator in an estimated 15% to 25% of patients with axial low back pain.The ligaments in the sacroiliac are among the strongest in the body and are not suspected by many clinicians to be susceptible to spraining or tearing. Skepticism of the existence of sacroiliac joint dysfunction within the medical community is furthered by the debate on how little or much the sacroiliac joint moves. A discrepancy as large as 2-17 degrees has been reported in clinical findings.

Treatment
Treatment is often dependent on the duration and severity of the pain and dysfunction. In the acute phase (first 1–2 weeks) for a mild sprain of the sacroiliac, it is typical for the patient to be prescribed rest, ice/heat, spinal manipulation, and physical therapy; anti-inflammatory medicine can also be helpful.If the pain does not resolve in the first 1–2 weeks, then the patient may benefit from a steroid and anesthetic mixture fluoroscopically injected into the joint (this also serves in confirming diagnosis), as well as manipulative or manual therapy. For the most severe and chronic forms of sacroiliac dysfunction, treatment should proceed with the support of a sacroiliac belt, injection therapy, and finally, surgery.  The anti-inflammatory effect of injection therapy is not permanent, and the injections do not offer an opportunity to stabilize an incompetent joint. Surgery is often considered a last resort, but for some patients, it is the only method of effectively stabilizing the loose joint </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Warty dyskeratoma and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Urethral hypermobility and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Acrodysostosis and explain in detail? [/INST] Acrodysostosis  is a rare congenital malformation syndrome which involves shortening of the interphalangeal joints of the hands and feet, intellectual disability in approximately 90% of affected children, and peculiar facies. Other common abnormalities include short head (as measured front to back), small broad upturned nose with flat nasal bridge, protruding jaw, increased bone age, intrauterine growth retardation, juvenile arthritis and short stature. Further abnormalities of the skin, genitals, teeth, and skeleton may occur.Other common abnormalities include short head (as measured front to back), small broad upturned nose with flat nasal bridge, protruding jaw, increased bone age, intrauterine growth retardation, juvenile arthritis and short stature </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Pemphigus herpetiformis and explain in detail? [/INST]  </s>


<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information. <</SYS>> What is Papular mucinosis and explain in detail? [/INST] Papular mucinosis (also known as scleromyxedema, "generalized lichen myxedematosus" and "sclerodermoid lichen myxedematosus") is a rare skin disease. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. Frequently, all three forms are regarded as papular mucinosis. However, some authors restrict it to only mild cases. Another form, acral persistent papular mucinosis is regarded as a separate entity.

Presentation
Papular mucinosis is chronic and may be progressive. The dermal layer of the skin breaks out into small and solid bumps, usually conical in shape and measured from 2 to 4 mm or sometimes flat-topped papules. Unlike pustules, these bumps do not contain pus. Instead they contain mucin, a waxy substance of mucus, the bodys natural and protective lubricant found in saliva and epithelial cells in lungs and the sensitive part of the nose. They usually come in clusters such as linear arrays. Less frequently, urticarial, nodular, or sometimes annular lesions may be appreciated. The dorsal aspect of the hands, face, elbows, and extensor portions of the extremities are most frequently affected. Mucosal lesions are absent. The coalescence of papules on the face, particularly on the glabella, results in longitudinal folding and gives the appearance of a leonine facies.
In scleromyxedema, symptoms can occur on larger part of the body. Redness and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced. Proximal myopathy, inflammatory polyarthritis, central nervous system symptoms, esophageal aperistalsis, and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro syndrome is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.

Diagnosis
Classification
Papular mucinosis may be divided into several types or variants:
Acral persistent papular mucinosis
Self-healing papular mucinosis
Papular mucinosis of infancy
Cutaneous mucinosis of infancy
Nodular lichen myxedematosus
Localized lichen myxedematosus
Discrete papular lichen myxedematosus

Treatment
There is no effective treatment for this condition </s>