<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 Reinkes edema and explain in detail? [/INST] Reinkes edema is the swelling of the vocal cords due to fluid (edema) collected within the Reinkes space. First identified by the German anatomist Friedrich B. Reinke in 1895, the Reinkes space is a gelatinous layer of the vocal cord located underneath the outer cells of the vocal cord. When a person speaks, the Reinkes space vibrates to allow for sound to be produced (phonation). The Reinkes space is sometimes referred to as the superficial lamina propria.Reinkes edema is characterized by the "sac-like" appearance of the fluid-filled vocal cords.  The swelling of the vocal folds causes the voice to become deep and hoarse.  Therefore, the major symptom of Reinkes edema is a hoarseness similar to laryngitis.  The major cause associated with Reinkes edema is smoking.  In fact, 97% of patients diagnosed with Reinkes edema are habitual smokers.  Other identified risk factors include overuse of the vocal cords, gastroesophageal reflux, and hypothyroidism.  The disease is more often cited in women than in men, because lower voice changes are more noticeable in women.The first cases of Reinkes edema were recorded in 1891 by M. Hajek, followed by F. Reinke in 1895.  In his investigations, Reinke injected a stained glue into the superficial lamina propria (Reinkes space) to mimic edema. Reinkes edema is considered to be a benign (non-cancercous) polyp (protrusion) that represents 10% of all benign laryngeal pathologies. Treatment of Reinkes edema starts with the elimination of associated risk factors, such as smoking, gastric reflux, and hypothyroidism.  Advanced cases may undergo phonosurgery to remove the fluid from the vocal cords.

Signs and symptoms
List of common symptoms:

"sac-like" appearance of the vocal folds
Hoarseness and deepening of the voice
Trouble speaking (Dysphonia)
Reduced vocal range with diminished upper limits
Stretching of the mucosa (Distension)
Shortness of breath (Dyspnoea)Reinkes edema is characterized by a "sac-like" appearance of the vocal folds.  The edema is a white translucent fluid that causes a bulging (distension) of the vocal cord. The most common clinical symptom associated with Reinkes edema is an abnormally low pitched voice with hoarseness. The low pitch voice is a direct result of increased fluid in the Reinkes space, which vibrates at a lower frequency than normal (females <130 Hz;  males <110 Hz).  Hoarseness is a common problem of many laryngeal diseases, such as laryngitis. It is described as a harsh and breathy tone of voice. Hoarseness is often seen alongside dysphonia, a condition in which the individual has difficulty speaking.The swelling of the vocal cords and the lowering of the voice are warning signs that an individual has Reinkes edema.  At the microscopic level, an examination of the vocal cords in patients with Reinkes edema will show lowered levels of collagen, elastin, and extracellular matrix proteins. These characteristics can be used to diagnose Reinkes edema.  Reinkes edema is considered a benign polyp that may become precancerous if smoking is involved.  An indicator of cancer is the development of leukoplakia, which manifests as white patches on the vocal folds.Smoking, gastric reflux, and hypothyroidism are all risk factors for Reinkes edema.  The symptoms of Reinkes edema are considered to be chronic symptoms because they develop gradually over time and depend on how long the individual is exposed to the risk factor.  In the case of smoking, as long as the individual continues the habit of smoking, the Reinkes edema will continue to progress.  This is true for other risk factors as well, such as untreated gastric reflux and overuse of the voice, which is common to professions such as singers and radio announcers.

Causes
Smoking is the number one cause of Reinkes edema.  Other factors include gastroesophageal reflux, hypothyroidism and chronic overuse of the voice. Smoking and reflux are the only risk factors that may lead to cancer. Additionally, the combination of several risk factors increase the likelihood of an individual developing Reinkes edema. For example, an individual who smokes and also has gastric reflux would have an increased susceptibility for developing Reinkes edema over time.Reinkes edema is commonly diagnosed in middle-aged females with a history of smoking (aged 50 years or older).  Because males have lower pitched voices than females, males are less likely to observe a significant changes in the voice, and are therefore less likely to seek treatment.  Females also report more physical discomfort due to Reinkes edema.  The risk of Reinkes edema increases with age and also with prolonged exposure to smoking.  Additionally, individuals in professions that require constant use of the voice, such as singers, teachers, and radio hosts, may be at an increased risk for developing the disease.Because the disease is heavily linked to smoking, there is no established way to screen for Reinkes edema. Similarly, the only way to prevent Reinkes edema is to avoid smoking. By adopting a non-smoking lifestyle after being diagnosed with Reinkes edema, it is possible to stop the diseases progression, although it is not possible to reverse it. Therefore, it is critical to maintain a non-smoking lifestyle even after surgery, because the fluid can re-emerge. In fact, in many cases surgeons will not perform surgery without the guarantee that the individual will stop smoking.

Mechanism
The vocal cords consist of five layers of cells:

Squamous epithelium
Superficial lamina propria (Reinkes space)
Intermediate lamina propria
Deep lamina propria
Vocalis muscleIn order for humans to produce sound for speech, the vocal folds must readily vibrate.  The two layers of the vocal cords that vibrate are the Reinkes space and the overlying epithelium.  In fact, these layers move freely over the more rigid intermediate and deep lamina proprias. Accumulation of fluid within the Reinkes space alters the elasticity of the vocal cord, making it less stiff and more gelatinous.  This slows the vocal cord vibration, which results in a deepened and hoarse voice.  Because men normally have a lower voice than women, the change is more noticeable in women.Edema usually occurs on both vocal cords. This is known as bilateral Reinkes edema. The pathophysiology or mechanism of Reinkes edema is not well known, however, chemicals contained within cigarette smoke are associated with an increased vascular permeability of blood vessels, which results in fluid leaking into the Reinkes space.  Normally, the vocal cords are surrounded by neatly aligned blood vessels, however, these blood vessels can become disarranged and fragile in Reinkes edema. In addition, cigarette smoke can create reactive oxygen species that alter the environment of the vocal cords.  Tissue analysis of Reinkes edema shows decreased amounts of the proteins fibronectin, elastin, collagens I and III, and extracellular matrix proteins.  This leads to an overall decreased stiffness of the tissue layer, which vibrates more slowly and produces a deeper sounding voice.The progression of Reinkes edema is gradual and is directly related to the duration of exposure to risk factors, such as smoking and gastric reflux. Disease progression is divided into two types: "pale" and "livid". The “pale” type of Reinkes edema is defined by a glazed appearance of the vocal cords with a clear (colorless) fluid underneath.  This represents the early stage of the disease. The advanced “livid” type of Reinkes edema is identified by an increased amount of fluid, accompanied by a color change from colorless to yellow-grey.  The swelling of the vocal folds cause ballooned-like appearance, known as a polyp. The polyps of Reinkes edema are usually benign, however, there may be a risk of cancer if the patient is a smoker.  Additionally, if the edema becomes too severe, patients may experience difficulty breathing due to blockage of the airway.

Diagnosis
Reinkes edema is often diagnosed by an Ear, Nose & Throat (ENT) specialist or an Otolaryngologist by examination of the vocal cords. First, the doctor will review the patients medical history and symptoms, such as hoarseness, dysphonia, and reduced vocal range. There is no familial or hereditary link to Reinkes edema. Because Reinkes edema is linked heavily to smoking, the doctor will need to know if the patient is a habitual smoker. Once the patients history is reviewed, the vocal cords will be visualized using laryngoscopy, a technique in which a tube with a camera (endoscope) is passed through the nose and down the larynx.  Laryngoscopes can be rigid or flexible.  Flexible laryngoscopes, such as fiber laryngoscopes, allow the patient to produce sound as the tube is placed, and therefore allows the doctor to visualize movement of the vocal cord. The use of rigid laryngoscopes generally requires general anaesthesia due to the discomfort involved in distracting the soft tissues of the mouth and pharynx.
Based on the results of the laryngoscopy, Reinkes edema can be classified using a standardized system set in place by Yonekawa. This system characterizes the disease based disease severity.Yonekawa Classification:
Grade I – Lesions contact the anterior third of the vocal fold
Grade II – Lesions contact the anterior two-thirds of the vocal fold
Grade III – Lesions contact the entirety of the vocal foldIf further evaluation is needed, stroboscopy is used to examine mucosal waves of the vocal cords.  Mucosal waves describe the waves produced by vibration of the vocal cords during speech.  Stroboscopes produce flashes of light that are timed to the patients vocal frequency.  Every time the light is flashed, it will create a still frame image of the vocal cords at that particular moment in time.  These are combined to produce an image of the wave. In the case of Reinkes edema, structural changes to the vocal cords will result in abnormal wave patterns.

Treatment
The first step in treating Reinkes edema is to eliminate or control those risk factors that are causing the disease.  This includes the cessation of smoking, the control of gastric reflux using antacids and/or Proton Pump Inhibitors (PPIs), and the discontinuation of activities that cause vocal distress. Those experiencing a hoarseness of the voice may choose to undergo voice therapy to improve the voices quality and range.  Most cases of Reinkes edema are caused by the long term usage of cigarettes. In this case, it is important to make lifestyle changes to stop smoking.  While this will not resolve or improve the edema, the cessation of smoking will halt the diseases progression.If the elimination of risk factors is not sufficient to improve the patients symptoms, surgery may be required.  The most common type of surgery performed today for Reinkes edema is called surgical microlaryngoscopy. Most procedures follow the microflap technique set in place by Hirano. During surgery, an incision is made into the vocal cord using either microscissors or a CO2 laser.  A flap of mucosa is lifted and the affected tissue is removed using suction or a microdebrider.  The flap is then re-draped and trimmed to the appropriate size.Most cases of Reinkes Edema are bilateral - affecting both vocal cords - rather than unilateral.  In the case of bilateral edema, the surgeon must choose whether to operate each side of the vocal cord in two separate surgeries or to operate both sides in a single surgery.  The complication associated with removing tissue from both sides in a single surgery is that the raw, cut ends of the vocal cords may form an anterior glottis web, in which the two sides grow together in a continuous sheet.  Other complications of surgery include tissue scarring due to damage to the vocal ligament during the incision and vocal cord stiffening due to over-suctioning of the superficial lamina propria (Reinkes space).While surgical microlaryngoscopy has its associated risks, if left untreated, Reinkes edema can lead to a variety of long-term complications.  Besides dysphonia (impaired speech), the most serious of these complications is airway obstruction due to severe inflammation of the vocal cords. The risk of complications has decreased drastically with the creation of new tools, such as the CO2 laser for surgical microlaryngoscopy.  Before the Hirano microflap method was developed in 1895, vocal stripping was the most common procedure used to correct Reinkes Edema.  Vocal stripping was often performed without magnification and with a monocular laryngoscope, instead of a binocular scope.  This led to major complications such as vocal ligament scarring.
Women are more likely than men to undergo surgery due to a greater change in vocal pitch and quality. Surgery is capable of restoring the voice, with the condition that smoking is not resumed after surgery.  Post-operative voice therapy is also advised to restore the voices strength. Reinkes edema is not a fatal pathology unless the tissue becomes precancerous.

Research
Recent studies have examined the role of specific cell types in Reinkes edema, including the role of vocal cord fibroblasts.  In normal tissue, these spindle-shaped CD34+ fibroblasts produce extracellular matrix proteins such as collagen and elastin.  Recent findings have shown a morphological change in fibroblasts extracted from the tissue of Reinkes edema to a more dendritic-like shape with several protrusions.  Large populations of these altered CD34+ fibroblasts have been found surrounding the areas of edema.  They lack normal expression of several cluster of differentiation (CD) proteins and express additional proteins that are not expressed in normal vocal cord fibroblasts.  Furthermore,  cigarette smoke was discovered to increase COX-2 and PGE2 expression in fibroblasts, which could indicate the role of cigarette smoke in Reinkes edema.While smoking is a clear risk factor to Reinkes edema, other risk factors are being identified to explain Reinkes edema in nonsmokers. Research has suggested the role of bacterial colonies in non-neoplastic lesions such as Reinkes edema.  Using pyrosequencing, strains of S. pseudopneumoniae were found as the dominant bacterial strain across most non-neoplastic lesions.  Of all the sequences analyzed, streptococcus represented 72.9% of bacteria found within these lesions </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 Uremic pruritus 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 Meigss 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 Urocanic aciduria 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 central nervous system lymphoma and explain in detail? [/INST] Primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system (DLBCL-CNS), is a primary intracranial tumor appearing mostly in patients with severe immunodeficiency (typically patients with AIDS). It is a subtype and one of the most aggressive of the diffuse large B-cell lymphomas. PCNSLs represent around 20% of all cases of lymphomas in HIV infections. (Other types are Burkitts lymphomas and immunoblastic lymphomas). Primary CNS lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and those immunosuppressed), and does not have a predilection for any particular age group. Mean CD4+ count at time of diagnosis is ~50/uL.  In immunocompromised patients, prognosis is usually poor.  In immunocompetent patients (that is, patients who do not have AIDS or some other immunodeficiency), there is rarely an association with EBV infection or other DNA viruses. In the immunocompetent population, PCNSLs typically appear in older patients in their 50s and 60s.  Importantly, the incidence of PCNSL in the immunocompetent population has been reported to have increased more than 10-fold from 2.5 cases to 30 cases per 10 million population.  The cause for the increase in incidence of this disease in the immunocompetent population is unknown.

Signs and symptoms
A primary CNS lymphoma usually presents with seizure, headache, cranial nerve findings, altered mental status, or other focal neurological deficits typical of a mass effect.  Systemic symptoms may include fever, night sweats, or weight loss. Other symptoms include

diplopia
dysphagia
vertigo
monocular vision loss
progressive dementia or stupor in patients with a nonfocal neurologic exam and minimal abnormalities on MRI (more common in AIDS patients)
facial hypoesthesia

Diagnosis
The current standard for diagnosis typically includes positive CSF cytology, vitreous biopsy, or brain/leptomeningeal biopsy. Histopathological confirmation is essential for definitive diagnosis.MRI or contrast enhanced CT classically shows multiple ring-enhancing lesions in the deep white matter. The major differential diagnosis (based on imaging) is cerebral toxoplasmosis, which is also prevalent in AIDS patients and also presents with a ring-enhanced lesion, although toxoplasmosis generally presents with more lesions and the contrast enhancement is typically more pronounced.  Imaging techniques cannot distinguish the two conditions with certainty, and cannot exclude other diagnoses.  Thus, patients undergo a brain biopsy or vitreous biopsy, if there is intraocular involvement.

Classification
Most PCNSLs are diffuse large B cell non-Hodgkin lymphomas.

Treatment
Surgical resection is usually ineffective because of the depth of the tumour. Treatment with irradiation and corticosteroids often only produces a partial response and tumour recurs in more than 90% of patients. Median survival is 10 to 18 months in immunocompetent patients, and less in those with AIDS. The addition of IV methotrexate and folinic acid (leucovorin)  may extend survival to a median of 3.5 years. If radiation is added to methotrexate, median survival time may increase beyond 4 years.  However, radiation is not recommended in conjunction with methotrexate because of an increased risk of leukoencephalopathy and dementia in patients older than 60.  In AIDS patients, perhaps the most important factor with respect to treatment is the use of highly active anti-retroviral therapy (HAART), which affects the CD4+ lymphocyte population and the level of immunosuppression. The optimal treatment plan for patients with PCNSL has not been determined. Combination chemotherapy and radiotherapy at least doubles survival time, but causes dementia and leukoencephalopathy in at least 50% of patients who undergo it. The most studied chemotherapeutic agent in PCNSL is methotrexate (a folate analogue that interferes with DNA repair). Methotrexate therapy in patients with PCNSL typically requires hospitalization for close monitoring and intravenous fluids. Leucovorin is often given for the duration of the therapy </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 Stomatitis nicotina and explain in detail? [/INST] Stomatitis nicotina is a diffuse white patch on the hard palate, usually caused by tobacco smoking, usually pipe or cigar smoking. It is painless, and it is caused by a response of the palatal oral mucosa to chronic heat. A more pronounced appearance can occur with reverse smoking, sometimes distinguished from stomatitis nicotina by the term reverse smokers stomatitis. While stomatitis nicotina that is caused by heat is not a premalignant condition (i.e. it does not carry an increased risk of transformation to oral cancer), the condition that is caused by reverse smoking is premalignant.

Signs and symptoms
The palate may appear gray or white and contain many papules or nodules that are slightly elevated with red dots in their center. These red dots represent the ducts of minor salivary glands which have become inflamed by heat. The condition is painless. If a denture is normally worn while smoking, then the mucosa underneath the denture appears unaffected by the condition. In severe cases, the mucosa may show fissuring and develop a "dried lake bed" appearance. Other changes associated with tobacco use may be evident such as brown or black extrinsic staining of teeth from tar and other components of tobacco smoke.

Causes
The cause of nicotine stomatitis is thought to be chemical or thermally induced keratosis. The chemicals in tobacco may act as irritants in this condition. Chronic heat exposure is also responsible. Pipe smoking produces more heat on the palate than any other forms of smoking. Long-term drinking of very hot beverages can also cause a similar condition. The severity of the changes correlates with the frequency of the habit. The prevalence depends on a societys use of consuming hot beverages and of smoking in its various forms.
A similar, but more pronounced palatal keratosis occurs with reverse smoking. This is where the lit end of the cigar or cigarette is held in the mouth, another form of smoking associated with high levels of heat in the mouth. This form of the condition is sometimes termed "reverse smokers keratosis", and is a premalignant lesion. That is, the condition is associated with an increased risk of malignant transformation to oral squamous cell carcinoma (a type of oral cancer). Some sources do not distinguish between reverse smokers keratosis and smokers palate that is caused by heat. As such, these sources tend to state that stomatitis nicotina is a premalignant condition. Some reports show that there is an increased risk of tonsillar cancer, lung cancer and tumors of the posterior oral cavity in people who develop stomatitis nicotina.

Diagnosis
The diagnosis is normally made based upon the clinical appearance and history. Tissue biopsy is not usually indicated unless there are areas of ulceration or localized erythroplakia (red patches). The differential diagnosis is with other causes of white lesions (see leukoplakia for a more complete discussion). Specific conditions which can produce a similar appearance include Dariers disease, discoid lupus erythematosus, oral candidiasis, and oral lichen planus.If a biopsy is taken, the histopathologic appearance is one of hyperkeratosis and acanthosis. There may be squamous metaplasia of excretory ducts, which results in the visible papules if the ducts become hyperplastic. Neutrophils may fill some ducts. It is characterized as a "fissured" or "dried mud" appearance from excess keratin production by cells. Dysplasia is rarely seen.

Treatment
When the appearance is caused by heat, the lesion is usually completely reversible within a few weeks if the smoking habit is stopped </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 Steatopygia 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 Keratoderma blennorrhagicum 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 Gerodermia osteodysplastica 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 Unilateral nevoid telangiectasia 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 Hypertensive urgency and explain in detail? [/INST] A hypertensive urgency is a clinical situation in which blood pressure is very high (e.g., 220/125 mmHg) with minimal or no symptoms, and no signs or symptoms indicating acute organ damage. This contrasts with a hypertensive emergency where severe blood pressure is accompanied by evidence of progressive organ or system damage.

Definition
Hypertensive urgency is defined as severely high blood pressure with no evidence of end organ damage. The term "malignant hypertension" was also included under this category with grade III/IV hypertensive retinopathy </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 Lower motor neuron lesion 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 Hemoglobin C and explain in detail? [/INST] Hemoglobin C (abbreviated as HbC) is an abnormal hemoglobin in which glutamic acid residue at the 6th position of the β-globin chain is replaced with a lysine residue due to a point mutation in the HBB gene. People with one copy of the gene for hemoglobin C do not experience symptoms, but can pass the abnormal gene on to their children. Those with two copies of the gene are said to have hemoglobin C disease and can experience mild anemia. It is possible for a person to have both the gene for hemoglobin S (the form associated with sickle cell anemia) and the gene for hemoglobin C; this state is called hemoglobin SC disease, and is generally more severe than hemoglobin C disease, but milder than sickle cell anemia.HbC was discovered by Harvey Itano and James V. Neel in 1950 in two African-American families. It has since been established that it is most common among people in West Africa. It confers survival benefits as individuals with HbC are naturally resistant to malaria caused by Plasmodium falciparum,  albeit incompletely.

Symptoms and signs
People with one copy of the gene for hemoglobin C (termed heterozygous) do not experience significant symptoms, but can pass the abnormal gene onto their children; this condition is called hemoglobin C trait. When two hemoglobin C genes are present (termed homozygous), the individual is said to have hemoglobin C disease, and may develop mild anemia, as red blood cells containing hemoglobin C have a decreased lifespan. The anemia in hemoglobin C disease is classified as hemolytic, because it is caused by the destruction of red blood cells. An enlarged spleen, and sometimes jaundice, may also occur. Some persons with this disease may develop gallstones that require treatment. Continued hemolysis may produce pigmented gallstones, an unusual type of gallstone composed of the dark-colored contents of red blood cells.

Red blood cell abnormalities
The red blood cells of people with hemoglobin C disease are usually abnormally small (microcytic) with a high mean corpuscular hemoglobin concentration (MCHC). The high MCHC is caused by a decreased concentration of water inside the cells. Target cells, microspherocytes, and HbC crystals can be seen on microscopic examination of blood smears from homozygous patients.

Combinations with other conditions
HbC can combine with other abnormal hemoglobins and cause serious hemoglobinopathies. Individuals with sickle cell–hemoglobin C (HbSC), have inherited the gene for sickle cell disease (HbS) from one parent and the gene for hemoglobin C disease (HbC) from the other parent.  Since HbC does not polymerize as readily as HbS, there is less sickling in most cases. There are fewer acute vaso-occlusive events and therefore in some cases fewer sickle cell crises. The peripheral smear demonstrates mostly target cells, occasional hemoglobin C crystals, and only a few sickle cells.  However, persons with hemoglobin SC disease (HbSC) have more significant retinopathy, ischemic necrosis of bone, and priapism than those with pure SS disease.There are also a few cases of HbC in combination with HbO, HbD and beta thalassemia.

Genetics
Hemoglobin C is produced when a point mutation in the HBB gene causes amino acid substitution of glutamic acid to lysine at the 6th position of the β-globin chain of the hemoglobin. The mutation can be homozygous, occurring on both the chromosomes (alleles), or heterozygous, affecting only one allele. Under heterozygous condition, people are said to have hemoglobin C trait, or as hemoglobin C carriers, and they have one gene for HbC with either one HbA gene or HbS gene. Their red blood cells contain both hemoglobin C and either normal hemoglobin A or hemoglobin S. Hemoglobin C mutation is an autosomal recessive disorder that results from the biparental inheritance of the allele that encodes for hemoglobin C. If both parents are carriers of hemoglobin C, there is a chance of having a child with hemoglobin C disease. Assuming both parents are carriers, there is a 25% chance of having a child with hemoglobin C disease, a 50% chance of having a child who is a carrier of hemoglobin C, and a 25% chance of having a child who is neither a carrier nor affected by hemoglobin C disease.This mutated form reduces the normal plasticity of host erythrocytes causing a hemoglobinopathy. In those who are heterozygous for the mutation, about 28–44% of total hemoglobin (Hb) is HbC, and no anemia develops. In homozygotes, nearly all Hb is in the HbC form, resulting in mild hemolytic anemia, jaundice and enlargement of spleen.

Resistance to malaria
Individuals with HbC have reduced risk of P. falciparum malaria infection. HbC has been described as being more advantageous than HbS because, even in homozygous individuals, it is usually non-fatal. However, in contrast to HbS, it does not prevent malaria due to P. vivax, and is less effective in resistance to falciparum malaria in heterozygous conditions. Homozygous HbC is more resistant to heterozygous condition, or to thalassemias. But HbC mutation  does not prevent the infection. P. falciparum do not survive in red blood cells with homozygous hemoglobins, but can survive in the presence of heterozygous hemoglobins. HbC reduced the binding force (cytoadherence) of P. falciparum by reducing the activity of PfEMP1. Evidences indicate that HbC reduced the level of  PfEMP1, which is required for effective binding and invasion of RBC by the malarial parasite. It has been predicted that with the trend of HbC mutation and falciparum prevalence, HbC would replace HbS in central West Africa in the future.

Diagnosis
Physical examination may show an enlarged spleen. Tests that may be done include: complete blood count (CBC), hemoglobin electrophoresis, and peripheral blood smear.

Prevention
Genetic counseling may be appropriate for high-risk couples who wish to have a baby.

Treatment
Usually no treatment is needed. Folic acid supplementation may help produce normal red blood cells and improve the symptoms of anemia

Prognosis
Overall, hemoglobin C disease is one of the more benign hemoglobinopathies. Mild-to-moderate reduction in RBC lifespan may accompany from mild hemolytic anemia.  Individuals with hemoglobin C disease have sporadic episodes of musculoskeletal (joint) pain. People with hemoglobin C disease can expect to lead a normal life.

Epidemiology
Hemoglobin C is found most abundantly in areas of West Africa, such as Nigeria, where Yorubas live. Hemoglobin C gene is found in 2-3% of African-Americans while 8% of African-Americans have hemoglobin S (Sickle) gene. Thus Hemoglobin SC disease is significantly more common than Hemoglobin CC disease. The trait also affects people whose ancestors came from Italy, Greece, Latin America, and the Caribbean region. However, it is possible for a person of any race or nationality to have hemoglobin C trait. In terms of geographic distribution, the hemoglobin C allele is found at the highest frequencies in West Africa, where it has been associated with protection against malaria. Hemoglobin C disease is present at birth, though some cases may not be diagnosed until adulthood. Both males and females are affected equally.

History
Studying the molecular basis of sickle cell disease, Linus Pauling and Harvey Itano at the California Institute of Technology discovered in 1949 that the disease was due to abnormal hemoglobin called HBS. In 1950, Itano and James V </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 Axis (anatomy) and explain in detail? [/INST] In anatomy,  the axis (from Latin axis, "axle") or epistropheus is the second cervical vertebra (C2) of the spine, immediately inferior to the atlas, upon which the head rests.
The axis defining feature is its strong odontoid process (bony protrusion) known as the dens, which rises dorsally from the rest of the bone.

Structure
The body is deeper in front or in the back and is prolonged downward anteriorly to overlap the upper and front part of the third vertebra.
It presents a median longitudinal ridge in front, separating two lateral depressions for the attachment of the longus colli muscles.

Dens
The dens, also called the odontoid process or the peg, is the most pronounced projecting feature of the axis.  The dens exhibits a slight constriction where it joins the main body of the vertebra. The condition where the dens is separated from the body of the axis is called os odontoideum and may cause nerve and circulation compression syndrome. On its anterior surface is an oval or nearly circular facet for articulation with that on the anterior arch of the atlas. On the back of the neck, and frequently extending on to its lateral surfaces, is a shallow groove for the transverse atlantal ligament which retains the process in position. The apex is pointed and gives attachment to the apical odontoid ligament. Below the apex, the process is somewhat enlarged and presents on either side a rough impression for the attachment of the alar ligament; these ligaments connect the process to the occipital bone.
The internal structure of the odontoid process is more compact than that of the body. The odontoid peg is the ascension of the atlas fused to the ascension of the axis. The peg has an articular facet at its front and forms part of a joint with the anterior arch of the atlas. It is a non-weight bearing joint. The alar ligaments, together with the apical ligaments, are attached from the sloping upper edge of the odontoid peg to the margins of the foramen magnum. The inner ligaments limit rotation of the head and are very strong. The weak apical ligament lies in front of the upper longitudinal bone of the cruciform ligament and joins the apex of the deltoid peg to the anterior margin of the foramen magnum. It is the fibrous remnant of the notochord.

Other features
The pedicles are broad and strong, especially in the front, where they coalesce with the sides of the body and the root of the odontoid process </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 Black heel and palm 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 Transplant glomerulopathy 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 Pseudobulbar palsy 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 Periorbital dermatitis 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 Langer–Giedion syndrome and explain in detail? [/INST] Langer–Giedion syndrome (LGS) is a very uncommon autosomal dominant genetic disorder caused by a deletion of a small section of material on chromosome 8. It is named after the two doctors who undertook the main research into the condition in the 1960s. Diagnosis is usually made at birth or in early childhood.

Signs and symptoms
The features associated with this condition include: mild to moderate learning difficulties, short stature, unique facial features, small head and skeletal abnormalities including bony growths projecting from the surfaces of bones.

Craniofacial
Individuals with Langer–Giedion syndrome may display characteristic craniofacial abnormalities. These include a long prominent philtrum, a thin upper lip,  wide spaced eyes, a bulbous nasal tip, a broad nasal bridge, wide nostrils, micrognathia, retrognathia, deep set eyes and large ears. The head itself is often  unusually small in comparison to that of unaffected individuals of the same age and sex. Dental abnormalities, such as supernumerary central incisors and the absence of some teeth, may occur

Muscoskeletal
Langer–Giedion syndrome causes cone shaped epiphyses of the phalanges of the hands and short fingers and toes. The fifth fingers are sometimes  bent.  Skeletal abnormalities not affecting the hands and feet may also occur </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 Eruptive vellus hair cyst and explain in detail? [/INST] Eruptive vellus hair cysts (or EVHC) are small lesions that occur most often in the chest wall, abdomen and extremities, often with a crusted surface.: 680  EVHC may occur randomly, or it can be inherited as an autosomal dominant trait. The condition affects males and females equally, and sporadic cases usually appear at 4–18 years of age. The cysts appear similar clinically to steatocystoma multiplex, as well as acneiform eruptions and milia. Therapeutic techniques that are safe and effective are rare, with incision and drainage being the primary form of treatment when sporadic regression does not occur </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 Obstructive shock and explain in detail? [/INST] Obstructive shock is one of the four types of shock, caused by a physical obstruction in the flow of blood. Obstruction can occur at the level of the great vessels or the heart itself. Causes include pulmonary embolism, cardiac tamponade, and tension pneumothorax. These are all life-threatening. Symptoms may include shortness of breath, weakness, or altered mental status. Low blood pressure and tachycardia are often seen in shock. Other symptoms depend on the underlying cause.The physiology of obstructive shock is similar to cardiogenic shock. In both types, the hearts output of blood (cardiac output) is decreased. This causes a back-up of blood into the veins entering the right atrium. Jugular venous distension can be observed in the neck. This finding can be seen in obstructive and cardiogenic shock. With the decrease cardiac output, blood flow to vital tissues is decreased. Poor perfusion to organs leads to shock. Due to these similarities, some sources place obstructive shock under the category of cardiogenic shock.However, it is important to distinguish between the two types, because treatment is different. In cardiogenic shock, the problem is in the function of the heart itself. In obstructive shock, the underlying problem is not the pump. Rather, the input into the heart (venous return) is decreased or the pressure against which the heart is pumping (afterload) is higher than normal. Treating the underlying cause can reverse the shock. For example, tension pneumothorax needs rapid needle decompression. This decreases the pressure in the chest. Blood flow to and from the heart is restored, and shock resolves.

Signs and Symptoms
As in all types of shock, low blood pressure is a key finding in patients with obstructive shock. In response to low blood pressure, heart rate increases. Shortness of breath, tachypnea, and hypoxia may be present. Because of poor blood flow to the tissues, patients may have cold extremities. Less blood to the kidneys and brain can cause decreased urine output and altered mental status, respectively.Other signs may be seen depending on the underlying cause. For example, jugular venous distension is a significant finding in evaluating shock. This occurs in cardiogenic and obstructive shock. This is not observed in the other two types of shock, hypovolemic and distributive. Some particular clinical findings are described below.
A classic finding of cardiac tamponade is Becks triad. The triad includes hypotension, jugular vein distension, and muffled heart sounds. Kussmauls sign and pulsus paradoxus may also be seen. Low-voltage QRS complexes and electrical alternans are signs on EKG. However, EKG may not show these findings and most often shows tachycardia.Tension pneumothorax would have decreased breath sounds on the affected side. Tracheal deviation may also be present, shifted away from the affected side. Thus, a lung exam is important. Other findings may include decreased chest mobility and air underneath the skin (subcutaneous emphysema).Pulmonary embolism similarly presents with shortness of breath and hypoxia. Chest pain worse with inspiration is frequently seen. Chest pain can also be similar to a heart attack. This is due to the right ventricular stress and ischemia that can occur in PE. Other symptoms are syncope and hemoptysis. DVT is a common cause. Thus, symptoms including leg pain, redness, and swelling can be present. The likelihood of pulmonary embolism can be evaluated through various criteria. The Wells score is often calculated. It gives points based on these symptoms and patient risk factors.

Causes
Causes include any obstruction of blood flow to and from the heart. There are multiple, including pulmonary embolism, cardiac tamponade, and tension pneumothorax. Other causes include abdominal compartment syndrome, severe aortic valve stenosis, and disorders of the aorta. Constrictive pericarditis is a rare cause. Masses can grow to press on major blood vessels causing shock.

Tension pneumothorax
A pneumothorax occurs when air collects in the pleural space around the lungs. Normally, this space has negative pressure to allow the lung to fill. Pressure increases as more air enters this space. The lung collapses, impairing normal breathing. Surrounding structures may also shift. When severe enough to cause these shifts and hypotension, it is called a tension pneumothorax. This is life-threatening. The veins supplying the heart are compressed. Thus, venous return is decreased. With the heart unable to fill, cardiac output drops. Hypotension and shock ensue. If not rapidly treated, it can lead to cardiac arrest and death.

Pulmonary embolism
A pulmonary embolism (PE) is an obstruction of the pulmonary arteries. Deaths from PE have been estimated at ~100,000 per year in the United States. However, this may be higher in recent years. Most often, the obstruction is a blood clot that traveled from elsewhere in the body. Most commonly, this is from a deep vein thrombosis (DVT) in the legs or pelvis. Risk factors are conditions that increase the risk of clotting. This includes genetic (factor V Leiden) and acquired conditions (cancer). Trauma, surgery, and prolonged bed-rest are common risks. Covid-19 is a recent risk factor.This obstruction increases the pulmonary vascular resistance. If large enough, the clot increases the load on the right side of the heart. The right ventricle must work harder to pump blood to the lungs. With back-up of blood, the right ventricle can begin to dilate. Right heart failure can ensue, leading to shock and death.A PE is considered "massive" when it causes hypotension or shock. A submassive PE causes right heart dysfunction without hypotension.

Cardiac tamponade
A pericardial effusion is fluid in the pericardial sac. When large enough, the pressure compresses the heart. This causes shock by preventing the heart from filling with blood. This is called cardiac tamponade. The chambers of the heart can collapse from this pressure. The right heart has thinner walls and collapses more easily. With less venous return, cardiac output decreases. The lack of blood flow to vital organs can cause death.Various conditions can cause a pericardial effusion. Inflammation of the pericardium is called pericarditis. This is caused by infection, renal failure or autoimmune disease. Trauma can cause blood to fill the pericardium. Cancer can also cause effusions.
Whether an effusion causes tamponade depends on the amount of fluid and how long it took to accumulate. When fluid collects slowly, the pericardium can stretch. Thus, a chronic effusion can be as large as 1 liter. Acute effusions can cause tamponade when small because the tissue does not have time to stretch.

Diagnosis
Rapid evaluation of shock is essential given its life-threatening nature. Diagnosis requires a thorough history, physical exam, and additional tests. One must also consider the possibility of multiple types of shock being present. For example, a trauma patient may be hypovolemic from blood loss. This patient could also have tension pneumothorax due to trauma to the chest.Vital signs in obstructive shock may show hypotension, tachycardia, and/or hypoxia. A physical exam include be thorough, including jugular vein exam, cardiac and lung exams, and assessing skin tone and temperature. Response to fluids may aid in diagnosis. Labs including a metabolic panel can assess electrolytes and kidney and liver function. Lactic acid rises due to poor tissue perfusion. This may even be an initial sign of shock and rise before blood pressure decreases. Lactic acid should lower with appropriate treatment of shock. EKG should also be performed. Tachycardia is often present, but other specific findings may be present based on the underlying cause.At the bedside, point-of-care echocardiography should be used. This is non-invasive and can help diagnose the four types of shock. Echocardiography can look for ventricular dysfunction, effusions, or valve dysfunction. Measurement of the vena cava during the breathing cycle can help assess volume status. A point-of-care echocardiogram can also assess for causes of obstructive shock. The vena cava would be dilated due to the obstruction. In pulmonary embolism, the right ventricle will be dilated. Other findings include paradoxical septal motion or clots in the right heart or pulmonary artery. Echocardiography can assess for pericardial effusion. In tamponade, collapse of the right atrium and ventricle would be seen due to pressure in the pericardial sac.A chest X-ray can rapidly identify a pneumothorax, seen as absence of lung markings. Ultrasound can show the lack of lung sliding. However, imaging should not delay treatment. CT angiography is the standard of diagnosis of pulmonary embolism. Clots appear in the vasculature as filling defects.

Treatment
In any type of shock, rapid treatment is essential. Delays in therapy increase the risk of mortality. This is often done as diagnostic assessment is still occurring. Resuscitation addresses the ABCs - airway, breathing, and circulation. Supplemental oxygen is given, and intubation is performed if indicated. Intravenous fluids can increase blood pressure and maintain blood flow to organs.However, fluids should be given with caution. Too much fluid can cause overload and pulmonary edema. In some cases, fluids may be beneficial. Fluids can improve venous return. For example, tamponade prevents normal cardiac filling due to pressure compressing the heart. In this case, giving fluids can improve right heart filling. However, in other causes of obstructive shock, too much fluid can worsen cardiac output. Thus, fluid therapy should be monitored closely.After these stabilizing measures, further treatment depends on the cause. Treatment of the underlying condition can quickly resolve the shock. For tension pneumothorax, needle decompression should be done immediately. A chest tube is also inserted. Cardiac tamponade is treated through needle or surgical decompression. Needle pericardiocentesis can be done at the bedside. This is often the preferred therapy. A catheter may be placed for continued drainage </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 Cleidocranial dysostosis and explain in detail? [/INST] Cleidocranial dysostosis (CCD), also called cleidocranial dysplasia, is a birth defect that mostly affects the bones and teeth. The collarbones are typically either poorly developed or absent, which allows the shoulders to be brought close together. The front of the skull often does not close until later, and those affected are often shorter than average. Other symptoms may include a prominent forehead, wide set eyes, abnormal teeth, and a flat nose. Symptoms vary among people; however, intelligence is typically unaffected.The condition is either inherited from a persons parents or occurs as a new mutation. It is inherited in an autosomal dominant manner. It is due to a defect in the RUNX2 gene which is involved in bone formation. Diagnosis is suspected based on symptoms and X-rays with confirmation by genetic testing. Other conditions that can produce similar symptoms include mandibuloacral dysplasia, pyknodysostosis, osteogenesis imperfecta, and Hajdu-Cheney syndrome.Treatment includes supportive measures such as a device to protect the skull and dental care. Surgery may be performed to fix certain bone abnormalities. Life expectancy is generally normal.It affects about one per million people. Males and females are equally commonly affected. Modern descriptions of the condition date to at least 1896. The term is from cleido meaning collarbone, cranial from the Greek κρανιὀς meaning skull, and dysostosis meaning formation of abnormal bone.

Signs and symptoms
Cleidocranial dysostosis is a general skeletal condition so named from the collarbone (cleido-) and cranium deformities which people with it often have.
People with the condition usually present with a painless swelling in the area of the clavicles at 2–3 years of age. Common features are:

Clavicles (collarbones) can be partly missing leaving only the medial part of the bone. In 10% of cases, they are completely missing. If the collarbones are completely missing or reduced to small vestiges, this allows hypermobility of the shoulders including ability to touch the shoulders together in front of the chest. The defect is bilateral 80% of the time. Partial collarbones may cause nerve damage symptoms and therefore have to be removed by surgery.
The mandible is prognathic due to hypoplasia of maxilla (micrognathism) and other facial bones.
A soft spot or larger soft area in the top of the head where the fontanelle failed to close, or the fontanelle closes late.
Bones and joints are underdeveloped. People are shorter and their frames are smaller than their siblings who do not have the condition.
The permanent teeth include supernumerary teeth. Unless these supernumeraries are removed they will crowd the adult teeth in what already may be an underdeveloped jaw. If so, the supernumeraries will probably need to be removed to make space for the adult teeth. Up to 13 supernumerary teeth have been observed. Teeth may also be displaced. Cementum formation may be deficient.
Failure of eruption of permanent teeth.
Bossing (bulging) of the forehead.
Open skull sutures, large fontanelles.
Hypertelorism.
Delayed ossification of bones forming symphysis pubis, producing a widened symphysis.
Coxa vara can occur, limiting abduction and causing Trendelenburg gait.
Short middle fifth phalanges, sometimes causing short and wide fingers.
Vertebral abnormalities.
On rare occasions, brachial plexus irritation can occur.
Scoliosis, spina bifida and syringomyelia have also been described.Other features are: parietal  bossing, basilar invagination (atlantoaxial impaction), persistent metopic suture, abnormal  ear structures with hearing loss, supernumerary ribs, hemivertebrae with spondylosis,  small and high scapulae, hypoplasia of illiac bones, absence of the pubic bone, short / absent fibular bones, short / absent  radial bones, hypoplastic terminal phalanges.

Genetics
CCD is usually autosomal dominant, but in some cases its cause is not known.  The main mechanism is thought to involve haploinsufficiency caused by mutations in CBFA1 (also known as Runx2), a gene located on the short arm of chromosome 6 (6p21), which encodes a transcription factor required for the differentiation of stem cells into osteoblasts. This results in delayed ossification of midline structures of the body and ensuing defects in membranous and endochondral bone formation.

Diagnosis
Different features of the dysostosis are significant </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 Delayed pressure urticaria 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 Bruck syndrome and explain in detail? [/INST] Bruck syndrome is characterized as the combination of arthrogryposis multiplex congenita and osteogenesis imperfecta. Both diseases are uncommon, but concurrence is extremely rare which makes Bruck syndrome very difficult to research. Bruck syndrome is thought to be an atypical variant of osteogenesis imperfecta most resembling type III, if not its own disease. Multiple gene mutations associated with osteogenesis imperfecta are not seen in Bruck syndrome. Many affected individuals are within the same family, and pedigree data supports that the disease is acquired through autosomal recessive inheritance. Bruck syndrome has features of congenital contractures, bone fragility, recurring bone fractures, flexion joint and limb deformities, pterygia, short body height, and progressive kyphoscoliosis. Individuals encounter restricted mobility and pulmonary function. A reduction in bone mineral content and larger hydroxyapatite crystals are also detectable Joint contractures are primarily bilateral and symmetrical, and most prone to ankles. Bruck syndrome has no effect on intelligence, vision, or hearing.

Genetics
The genetics of Bruck syndrome differs from osteogenesis imperfecta. Osteogenesis imperfecta involves autosomal dominant mutations to Col 1A2 or Col 1A2 which encode type 1 procollagen. Bruck syndrome is linked to mutations in two genes, and therefore is divided in two types. Bruck syndrome type 1 is caused by a homozygous mutation in the FKBP10 gene.  Type 2 is caused by a homozygous mutation in the PLOD2 gene.

Mechanism
Type 1  encodes FKBP65, an endoplasmic reticulum associated peptidyl-prolyl cis/trans isomerase (PPIase) that functions as a chaperone in collagen biosynthesis. Osteoblasts deficient in FKBP65 have a buildup of procollagen aggregates in the endoplasmic reticulum which reduces their ability to form bone. Furthermore, Bruck syndrome type 1 patients have under-hydroxylated  lysine residues in the collagen telopeptide and as a result show diminished hydroxylysylpyridinoline cross-links.Type 2  encodes the enzyme, lysyl hydroxylase 2, which catalyzes hydroxylation of lysine residues in collagen cross-links. PLOD2 is most expressed in active osteoblasts since collagen cross-linking is tissue-specific. Mutation in PLOD2 alters the structure of telopeptide lysyl hydroxylase and prevents fibril formation of collagen type 1. Bone analysis shows the lysine residues of telopeptides in collagen type 1 are under-hydroxylated.

Diagnosis
Diagnosis of Bruck syndrome must distinguish the association of contractures and skeletal fragility. Ultrasound is used for prenatal diagnosis. The diagnosis of a neonate bears resemblance to arthrogryposis multiplex congenital, and later in childhood to osteogenesis imperfecta.

Management
Until more molecular and clinical studies are performed there will be no way to prevent the disease. Treatments are directed towards alleviating the symptoms </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 Spindle cell rhabdomyosarcoma 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 Hereditary mucoepithelial 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 Pancreas divisum and explain in detail? [/INST] Pancreatic divisum is a congenital anomaly in the anatomy of the ducts of the pancreas in which a single pancreatic duct is not formed, but rather remains as two distinct dorsal and ventral ducts. Most individuals with pancreas divisum remain without symptoms or complications. A minority of people with pancreatic divisum may develop episodes of abdominal pain, nausea or vomiting due to acute or chronic pancreatitis. The presence of pancreas divisum is usually identified with cross sectional diagnostic imaging, such as MRI or computed tomography (CT) imaging. In some cases, endoscopic retrograde cholangiopancreatography (ERCP) is performed, revealing the diagnosis of pancreas divisum. If no symptoms or complications are present, then treatment is not necessary. However, if there is recurrent pancreatitis, then a sphincterotomy of the minor papilla may be indicated.

Causes
The human embryo begins life with two ducts in the pancreas, the ventral duct and the dorsal duct. Normally, the two ducts will fuse together to form one main pancreatic duct; this occurs in more than 90% of embryos. In approximately 10% of embryos the ventral and dorsal ducts fail to fuse together, resulting in pancreas divisum. In utero, the majority of the pancreas is drained by the dorsal duct which opens up into the minor duodenal papilla. The ventral duct drains the minority of the pancreas and opens into the major duodenal papilla. In adults however, this situation is reversed whereby 70% of the pancreas is drained by the ventral duct. Therefore in pancreas divisum, where fusion of the ducts does not occur, the major drainage of the pancreas is done by the dorsal duct which opens up into the minor papilla.

Diagnosis
The most common and accurate way of diagnosing an individual with this anomaly is by MRCP (Magnetic Resonance Cholangiopancreatography) or ERCP (Endoscopic Retrograde Cholangiopancreatography) </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 Breastfeeding difficulties and explain in detail? [/INST] Breastfeeding difficulties refers to problems that arise from breastfeeding, the feeding of an infant or young child with milk from a womans breasts.  Although babies have a sucking reflex that enables them to suck and swallow milk, and human breast milk is usually the best source of nourishment for human infants, there are circumstances under which breastfeeding can be problematic, or even in rare instances, contraindicated.
Difficulties can arise both in connection with the act of breastfeeding and with the health of the nursing infant.

Breastfeeding problems
While breastfeeding difficulties are not uncommon, putting the baby to the breast as soon as possible after birth helps to avoid many problems. The policy of the American Academy of Pediatrics on breastfeeding instructs to, "delay weighing, measuring, bathing, needle-sticks, and eye prophylaxis until after the first feeding is completed."  Many breastfeeding difficulties can be resolved with research based hospital procedures, properly trained nurses and hospital staff, speech pathologists and lactation consultants. Another source of information is the volunteer-based breastfeeding promotion organization, La Leche League.
A variety of factors and conditions can interfere with successful breastfeeding:

Ankyloglossia (tongue tie)
Formula feeding
Distractions or interruptions during feeds
Long separations from the mother
Tachypnea (rapid breathing) such as in transient tachypnea of the newborn, surfactant deficiency, respiratory distress syndrome or other infant medical conditions
Presence of an actual physical barrier between mother and infant
Swallowing difficulties such as with prematurity and coordination of sucking, swallowing and breathing, or gastro-intestinal tract abnormalities like tracheo-oesophageal fistula.
Pain resulting from surgical procedures like circumcision, blood tests, or vaccinations.
Latching onto the breast
Hypoplastic breasts/insufficient glandular tissue
Galactorrhea
Lactation failure
Polycystic ovarian syndrome
Diabetes
Maternal stress
Insufficient rest/support of the mother during the first 6 weeks post-partum
Early return to work due to lack of financial support/maternity leave of mother
Cleft palate
Thrush
Hypoglycemia or hyperglycemia
Hypotonia, or "low-tone" infant disorder
Hyperlactation syndrome
Overactive let-down
Premature babies can have difficulties coordinating their sucking reflex with breathing. They may need to be fed more frequently because their stomachs tend to be smaller, and they may get sleepier during feedings. Premature infants unable to take enough calories by mouth may need enteral or gavage feeding - inserting a feeding tube into the stomach to provide enough breast milk or a substitute. This is often done together with Kangaroo care (prolonged skin-to-skin contact with the mother) which makes later breastfeeding easier. For some suckling difficulties, such as may happen with cleft lip/palate, the baby can be fed with a Haberman Feeder.
Dysphoric milk ejection reflex (D-MER) is a newly recognized condition affecting lactating women that is characterized by an abrupt dysphoria, or negative emotions that occur just before milk release and continuing not more than a few minutes. Preliminary testing tells us that D-MER is treatable and preliminary research tells us that inappropriate dopamine activity at the time of the milk ejection reflex is the cause of D-MER.

Low milk supply
Primary lactation failure: occurs when the mother has a condition incompatible with full milk production, for example breast hypoplasia, breast reduction surgery, or bilateral mastectomy.
Secondary lactation failure: milk production that is low due to preventable factors, such as formula supplementation, poor milk transfer by the baby, or unrelieved breast engorgement.
Chronic low milk supply is estimated to be experienced by 10-15% of women.

Breast pain
Pain often interferes with successful breastfeeding. It is cited as the second most common cause for the abandonment of exclusive breastfeeding after perceived low milk supply.

Inverted nipples
Inverted or retracted nipples sometimes make attachment to the breast difficult. These mothers need additional support to feed their babies. Treatment is started after the birth of the baby. The nipple is manually stretched out several times a day. A pump or a plastic syringe is used to draw out the nipple and the baby is then put to the breast.

Engorgement
Breast engorgement is the sense of breast fullness experienced by most women within 36 hours of delivery. Normally, this is a painless sensation of "heaviness". Breastfeeding on demand is the primary way of preventing painful engorgement.
When the breast overfills with milk it becomes painful. Engorgement comes from not getting enough milk from the breast. It happens about 3 to 7 days after delivery and occurs more often in first time mothers. The increased blood supply, the accumulated milk and the swelling all contribute to the painful engorgement. Engorgement may affect the areola, the periphery of the breast or the entire breast, and may interfere with breastfeeding both from the pain and also from the distortion of the normal shape of the areola/nipple. This makes it harder for the baby to latch on properly for feeding. Latching may occur over only part of the areola. This can irritate the nipple more, and may lead to ineffective drainage of breast milk and more pain. Reverse pressure softening (RPS) is a technique that can soften the areola enabling deeper latching and more milk transfer; RPS involves gentle positive pressure in the direction of the chest wall from the fingertips around the areola. Engorgement may begin as a result of several factors such as nipple pain, improper feeding technique, infrequent feeding or infant-mother separation.
To prevent or treat engorgement, remove the milk from the breast, by breastfeeding, expressing or pumping. Gentle massage can help start the milk flow and so reduce the pressure. The reduced pressure softens the areola, perhaps even allowing the infant to feed. Warm water or warm compresses and expressing some milk before feeding can also help make breastfeeding more effective. Some researchers have suggested that after breastfeeding, mothers should pump and/or apply cold compresses to reduce swelling pain and vascularity even more. One published study suggested the use of "chilled cabbage leaves" applied to the breasts. Attempts to reproduce this technique met with mixed results. Nonsteroidal anti-inflammatory drugs or paracetamol (acetaminophen) may relieve the pain. A warm shower and using cold compresses to help ease the discomfort.

Nipple pain
Sore nipples (nipple pain, or thelalgia) are probably the most common complaint after the birth. They are generally reported by the second day after delivery but improve within 5 days. Pain beyond the first week, severe pain, cracking, fissures or localized swelling is not normal. The mother should see a doctor for further evaluation. Sore nipples, a common cause of pain, often come from the baby not latching on properly. Factors include too much pressure on the nipple when not enough of the areola is latched onto and an improper release of suction at the end of the feeding. Improper use of breast pumps or topical remedies can also contribute. Nipple pain can also be a sign of infection.

Candidiasis
Symptoms of candidiasis of the breast include pain, itching, burning and redness, or a shiny or white patchy appearance. The baby could have a white tongue that does not wipe clean. Candidiasis is common and may be associated with infant thrush.
Both mother and baby must be treated to get rid of this infection. First-line therapies include nystatin, ketaconazole or miconazole applied to the nipple and given by mouth to the baby. Strict cleaning of clothing and breast pumps is also required to eradicate the infection.Another non-prescription treatment of candidia is gentian violet. It usually works, and relief is rapid. It is messy, and will stain clothing. The babys lips will turn purple, but the purple will disappear after a few days.

Milk stasis
Milk stasis is when the milk ducts are blocked and cannot drain properly, usually due to swelling and insufficient breast emptying during the engorgement phase. This may affect only a part of the breast and is not associated with any infection. It can be treated by varying the babys feeding position and applying heat before feeding. If it happens more than once, further evaluation is needed. Milk stasis is an urgent matter for mothers who wish to breastfeed, as failure to remove milk from the breasts causes milk production to decrease and eventually stop.

Mastitis
Mastitis is an inflammation of the breast. It causes local pain (dolor), redness (rubor), swelling (tumor), and warmth (calor). Later stages of mastitis cause symptoms of systemic infection like fever and nausea. It mostly occurs 2–3 weeks after delivery but can happen at any time. Typically results from milk stasis with primary or secondary local, later systemic infection. Infectious organisms include Staphylococcus sp., Streptococcus sp. and E. coli. Continued breastfeeding, plenty of rest and adequate fluid supply is the best treatment for light cases.

Overactive let-down
Overactive let-down (OALD) is the forceful ejection of milk from the breast during breastfeeding. The forceful spray of milk can cause the baby to consume too much milk too quickly as well as to swallow air during the period of rapid swallowing following the let-down.

Raynauds of the nipple
Nipple pain can be caused by vasospasm of the nipple. In essence, blood does not flow properly to the nipple which causes the nipple to blanch. This can be caused by trauma to the nipple through early breastfeeding or candidal infection of the nipple. The pain is intense during the latch stage and in between breastfeeding sessions there is a throbbing pain when the nipple is blanched. The nipple can be massaged to help blood flow return to reduce pain, as well as avoiding cold. In some instances, heart medication, nifedipine, is used to help the blood flow return to the nipple.

Infant health problems
Infants with classic galactosemia cannot digest lactose and therefore cannot benefit from breast milk. Breastfeeding might harm the baby also if the mother has untreated pulmonary tuberculosis, is taking certain medications that suppress the immune system. has HIV, or uses potentially harmful substances such as cocaine, heroin, and amphetamines. Other than cases of acute poisoning, no environmental contaminant has been found to cause more harm to infants than lack of breastfeeding. Although heavy metals such as mercury are dispersed throughout the environment and are of concern to the nursing infant, the neurodevelopmental benefits of human milk tend to override the potential adverse effects of neurotoxicants.

Weak sucking reflex
Artificial teats (nipples) or dummies (pacifiers) can suppress the sucking reflex in infants. In addition, when a baby is put to the breast for shorter periods of time, milk production decreases. The time spent sucking by the infant on the pacifier or artificial teat reduces the time on the breast.  The CDC also currently (2022) reports that early use of pacifiers can have a negative outcome on the success of breastfeeding and they suggest that it should be delayed until breastfeeding is firmly established.

Transmission of infection
Tuberculosis
It is not safe for mothers with active, untreated tuberculosis to breastfeed until they are no longer contagious. According to the American Academy of Pediatrics 2006 Redbook: Women with tuberculosis who have been treated appropriately for 2 or more weeks and who are not considered contagious may breastfeed. Women with tuberculosis disease suspected of being contagious should refrain from breastfeeding or any other close contact with the infant because of potential transmission through respiratory tract droplets (see Tuberculosis, p 678). Mycobacterium tuberculosis rarely causes mastitis or a breast abscess, but if a breast abscess caused by M. tuberculosis is present, breastfeeding should be discontinued until the mother no longer is contagious.
In areas where BCG vaccination is the standard of care, the WHO provides treatment recommendations and advises mothers to continue breastfeeding. TBC may be congenital, or perinatally acquired through airborne droplet spread.

HIV
Research published in the Lancet has highlighted a lower risk of HIV transmission with exclusive breastfeeding by HIV positive mothers (4% risk), compared to mixed feeding (10-40% risk). Research on the timing of HIV transmission in 2000 revealed that a "substantial transmission occurs early during breastfeeding," concluding that 75% of all breast milk transmission had occurred within the first 6 months during a randomized control trial in Kenya. This research is of particular importance in developing countries where infant formula is not widely available or safe to prepare. In fact, the World Health Organization recommended breastfeeding in 1987 and 1992 for seropositive and seronegative women in areas where malnutrition and infectious diseases are the major cause of infant mortality. In 1996 UNAIDS issued a recommendation that women in developing countries consider the risks and benefits of each feeding practice on an individual level; they recommended women make an informed choice about infant feeding.  In the days before the AIDS epidemic was clearly understood, some researchers pointed to the need to increase breastfeeding rates and pointed to the risks of formula feeding, citing increased rates of marasmus and diarrhea. D Jelliffe and E Jelliffe also criticized the marketing of infant formulas by US companies to resource-poor countries, something they termed "comerciogenic malnutrition." A more recent article from 1992 describes how the health of an infant can be compromised by water, which in many resource-poor countries holds the risk of environmental pathogens that are not present in breastmilk.

Transmission of drugs and toxins
Medications
The vast majority of over the counter and prescription medicines are compatible with breastfeeding, but there are some that might be passed onto the child through the milk.

Tobacco smoke
If one does continue tobacco smoking after giving birth, however, it is still more beneficial to breastfeed than to completely avoid this practice altogether. There is evidence that breastfeeding offers protection against many infectious diseases, especially diarrhea. Even in babies exposed to the harmful effects of nicotine through breast milk, the likelihood of acute respiratory illness is significantly diminished when compared to infants whose mothers smoked but were formula fed.  Regardless, the benefits of breastfeeding outweigh the risks of nicotine exposure.
The main concern about smoking and breastfeeding is that infants may have smoking-induced reductions to the milk iodine content.  Smoking can adversely affect the lactation process by decreasing milk production and altering the milk composition. Smoking reduces daily milk output by roughly 250–300 mL.  Not only will this be problematic on a daily basis for not producing enough milk, it will also cause the mother to wean her baby early.  The altered milk composition also caused infants to exhibit daily behaviors such as colic and crying which can promote early weaning, again something that is not beneficial to the infant.Also, the nicotine obtained from smoking travels through a woman into her breast milk, thus giving nicotine to her child.Heavy use of cigarettes by the mother (more than 20 per day) has been shown to reduce the mothers milk supply and cause vomiting, diarrhoea, rapid heart rate, and restlessness in breastfed infants. Sudden Infant Death Syndrome (SIDS) is more common in babies exposed to a smoky environment. Breastfeeding mothers who smoke are counseled not to do so during or immediately before feeding their child, and are encouraged to seek advice to help them reduce their nicotine intake or quit.

Other substance abuse
With respect to alcohol, the American Academy of Pediatrics states that when breastfeeding, "moderation is definitely advised" and recommends waiting for 2 hours after drinking before nursing or pumping. A 2014 review found that "even in a theoretical case of binge drinking, the children would not be subjected to clinically relevant amounts of alcohol [through breastmilk]", and would have no adverse effects on children as long as drinking is "occasional".If the mother consumes too much caffeine, it can cause irritability, sleeplessness, nervousness and increased feeding in the breastfed infant </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 Hydrops fetalis and explain in detail? [/INST] Hydrops foetalis or hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively.

Signs and symptoms
Locations can include the subcutaneous tissue on the scalp, the pleura (pleural effusion), the pericardium (pericardial effusion) and the abdomen (ascites). Edema is usually seen in the fetal subcutaneous tissue, sometimes leading to spontaneous abortion.  It is a prenatal form of heart failure, in which the heart is unable to satisfy demand (in most cases abnormally high) for blood flow.

Causes
Hydrops fetalis usually stems from fetal anemia, when the heart needs to pump a much greater volume of blood to deliver the same amount of oxygen. This anemia can have either an immune or non-immune cause. Non-immune hydrops can also be unrelated to anemia, for example if a fetal tumor or congenital cystic adenomatoid malformation increases the demand for blood flow. The increased demand for cardiac output leads to heart failure, and corresponding edema.

Immune pathophysiology
Erythroblastosis fetalis, also known as Rh disease, is the only immune cause of hydrops fetalis. Rh disease is a hemolytic disease of newborns. Pregnant mothers do not always have the same blood type as their child. During birth or throughout the pregnancy, the mother may be exposed to the infants blood. In the event of a pregnancy where the fetus has the Rh-D blood antigen and the mother does not, the mothers immune system will respond to the red blood cells as foreign and create antibodies against the Rh-D antigen on the fetal blood cells. Rh disease develops in the event of a second pregnancy where the mothers immune system launches an attack, via IgG, against the infants Rh-D positive blood cells. The immune response results in hemolysis of fetal red blood cells causing severe anemia.Hemolysis caused by the Rh incompatibility, causes extramedullary hematopoiesis in the fetal liver and bone marrow. The push to make more erythroblasts to help compensate with the hemolysis over works the liver causing hepatomegaly. The resulting liver dysfunction decreases albumin output which in turn decreases oncotic pressure. Consequentially, the decrease in pressure results in overall peripheral edema and ascites.Rh disease is currently an uncommon cause of immune-mediated hydrops fetalis. Due to preventative methods developed in the 1970s, the incidence of Rh disease has markedly declined. Rh disease can be prevented by administration of anti-D IgG (Rho(D) Immune Globulin) injections to RhD-negative mothers during pregnancy and/or within 72 hours of the delivery. However, a small percentage of pregnant mothers are still susceptible to Rh disease even after receiving anti-D IgG (Rho(D) Immune Globulin).

Non-immune pathophysiology
Severe anemia leads to hyperdynamic circulation, which means high-output cardiac failure causes the blood to circulate rapidly. The excessive pumping of blood causes the left side of the heart to fail leading to pulmonary edema. The build up of fluid in the lungs increases the pressure in the lungs leading to vasoconstriction. The coupled vasoconstriction and pulmonary hypertension causes the right side of the heart to fail which in turn, increases the venous hydrostatic pressure in the body. The summation of these effects ultimately leads to peripheral edema and ascites. All in all, the left side failure of the heart will lead to pulmonary edema whereas right side failure will lead to peripheral edema and ascites </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 Trachonychia 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 Paraumbilical hernia and explain in detail? [/INST] A paraumbilical (or umbilical) hernia is a hole in the connective tissue of the abdominal wall in the midline with close approximation to the umbilicus. If the hole is large enough there can be protrusion of the abdominal contents, including omental fat and/or bowel </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 Status marmoratus 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 Smouldering myeloma and explain in detail? [/INST]  </s>