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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes. Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include Cholangitis, hepatomegaly and signs of portal hypertension.
The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation.
Chronic liver diseases like chronic hepatitis, chronic alcohol abuse or chronic toxic liver disease may cause
- liver failure and hepatorenal syndrome
- fibrosis and cirrhosis of liver
Cirrhosis may also occur in primary biliary cirrhosis. Rarely, cirrhosis is congenital.
Necrotizing granulomas can develop in patients with rheumatoid arthritis, typically manifesting as bumps in the soft tissues around the joints (so-called rheumatoid nodules) or in the lungs.
Crohn's disease is an inflammatory condition of uncertain cause characterized by severe inflammation in the wall of the intestines and other parts of the abdomen. Within the inflammation in the gut wall, granulomas are often found and are a clue to the diagnosis.
This may cause fatty liver, hepatitis, fibrosis and sclerosis leading to cirrhosis and finally liver failure.
Localized granuloma annulare has a tendency towards spontaneous resolution. Localized lesions have been treated with potent topical corticosteroids.
Annular elastolytic giant-cell granuloma (also known as "Giant cell elastophagocytosis," "Meischer's granuloma," "Miescher's granuloma of the face") is a cutaneous condition characterized histologically by a dermal infiltrate of macrophages.
This large, atypical haemangioma of the liver may present with abdominal pain or fullness due to haemorrhage, thrombosis or mass effect. It may also lead to left ventricular volume overload and heart failure due to the increase in cardiac output which it causes. Further complications are Kasabach-Merritt syndrome, a form of consumptive coagulopathy due to thrombocytopaenia, and rupture.
Cholestasis is a condition where bile cannot flow from the liver to the duodenum. The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system that can occur from a gallstone or malignancy, and metabolic types of cholestasis which are disturbances in bile formation that can occur because of genetic defects or acquired as a side effect of many medications.
Liver haemangiomas are typically hyperechoic on ultrasound though may occasionally be hypoechoic; ultrasound is not diagnostic. Computed tomography (CT), magnetic resonance imaging (MRI) or single-photon emission computed tomography (SPECT) using autologous labelled Red Blood Cells (RBC) with Tc-99m is diagnostic. Biopsy is avoided due to the risk of haemorrhage.
Hepatic haemangiomas can occur as part of a clinical syndrome, for example Klippel-Trenaunay-Weber syndrome, Osler–Weber–Rendu syndrome and Von Hippel-Lindau syndrome.
Signs and symptoms depend largely upon the primary lesions giving rise to the condition. In addition to the heart or lung symptoms, there will be a sense of fullness and tenderness in the right hypochondriac region. Gastrointestinal catarrh is usually present, and vomiting of blood may occur. There is usually more or less jaundice. Owing to portal obstruction, ascites occurs, followed later by generalised oedema. The stools are light or clay-colored, and the urine is colored by bile. On palpation, the liver is found enlarged and tender, sometimes extending several inches below the costal margin of the ribs.
Under a microscope, the individual hepatocytes will have a brownish-green stippled appearance within the cytoplasm, representing bile that cannot get out of the cell. Canalicular bile plugs between individual hepatocytes or within bile ducts may also be seen, representing bile that has been excreted from the hepatocytes but cannot go any further due to the obstruction. When these plugs occur within the bile duct, sufficient pressure (caused by bile accumulation) can cause them to rupture, spilling bile into the surrounding tissue, causing hepatic necrosis. These areas are known as bile lakes, and are typically seen only with extra-hepatic obstruction.
Cats with eosinophilic granuloma complex (EGC) may have one or more of four patterns of skin disease.
The most frequent form is "eosinophilic plaque". This is a rash comprising raised red to salmon-colored and flat-topped, moist bumps scattered on the skin surface. The most common location is on the ventral abdomen and inner thigh.
Another form of EGC is the "lip ulcer". This is a painless, shallow ulcer with raised and thickened edges that forms on the upper lip adjacent to the upper canine tooth. It is often found on both sides of the upper lips.
The third form of the EGC is the "collagenolytic granuloma". This is a firm swelling that may be ulcerated. The lesions may form on the skin, especially of the face, in the mouth, or on the feet, or may form linear flat-topped raised hairless lesions on the back of the hind legs, also called "linear granuloma".
The least common form of EGC is "atypical eosinophilic dermatitis". It is unique in that it is caused by mosquito bite allergy and the lesions form on the parts of the body with the least hair affording easy access to feeding mosquitoes. This includes the bridge of the nose, the outer tips of the ears and the skin around the pads of the feet. The lesions are red bumps, shallow ulcers and crusts.
Chronic liver failure usually occurs in the context of cirrhosis, itself potentially the result of many possible causes, such as excessive alcohol intake, hepatitis B or C, autoimmune, hereditary and metabolic causes (such as iron or copper overload, steatohepatitis or non-alcoholic fatty liver disease).
Pulmonary hyalinizing granuloma is a lesional pattern of pulmonary inflammatory pseudotumor.
Angiolymphoid hyperplasia with eosinophilia (also known as: "Epithelioid hemangioma," "Histiocytoid hemangioma," "Inflammatory angiomatous nodule," "Intravenous atypical vascular proliferation," "Papular angioplasia," "Inflammatory arteriovenous hemangioma," and "Pseudopyogenic granuloma") usually presents with pink to red-brown, dome-shaped, dermal papules or nodules of the head or neck, especially about the ears and on the scalp.
It, or a similar lesion, has been suggested as a feature of IgG4-related skin disease, which is the name used for skin manifestations of IgG4-related disease.
About 25–50% of hepatic adenomas cause pain in the right upper quadrant or epigastric region of the abdomen. Since hepatic adenomas can be large (8–15 cm), patients may notice a palpable mass. However, hepatic adenomas are usually asymptomatic, and may be discovered incidentally on imaging ordered for some unrelated reason. Large hepatic adenomas have a tendency to rupture and bleed massively inside the abdomen. If not treated, there is a 30% risk of bleeding. Bleeding may lead to hypotension, tachycardia, and sweating (diaphoresis).
Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.
Patch-type granuloma annulare (also known as "Macular granuloma annulare") is a skin condition of unknown cause, more commonly affecting women between 30 and 70 years of age, characterized by flat or slightly palpable erythematous or red-brown skin lesions.
Aside from the visible rash, granuloma annulare is usually asymptomatic. Sometimes the rash may burn or itch. People with granuloma annulare usually notice a ring of small, firm bumps (papules) over the backs of the forearms, hands or feet, often centered on joints or knuckles. The bumps are caused by the clustering of T cells below the skin. These papules start as very small, pimple looking bumps, which spread over time from that size to dime, quarter, half-dollar size and beyond. Occasionally, multiple rings may join into one. Rarely, granuloma annulare may appear as a firm nodule under the skin of the arms or legs. It also occurs on the sides and circumferential at the waist and without therapy can continue to be present for many years. Outbreaks continue to develop at the edges of the aging rings.
Eosinophilic granuloma is a form of Langerhans cell histiocytosis. It is a condition of both human and veterinary pathology. Colloquially, the reaction pattern is sometimes referred to as a rodent ulcer.
Feline eosinophilic granuloma complex is synonymous with "feline eosinophilic skin diseases". This is considered to be a cutaneous reaction pattern that can be the manifestation of a number of underlying infections, allergies or ectoparasite infestations. It can also be idiopathic, that is have no known underlying trigger. The eosinophilic reaction is common in feline inflammatory disease and the eosinophilic granuloma can be a hereditary reaction pattern in some lines of domestic cats.
Acute liver failure is defined as "the rapid development of hepatocellular dysfunction, specifically coagulopathy and mental status changes (encephalopathy) in a patient without known prior liver disease".
The disease process is associated with the development of a coagulopathy of liver aetiology, and clinically apparent altered level of consciousness due to hepatic encephalopathy. Several important measures are immediately necessary when the patient presents for medical attention. The diagnosis of acute liver failure is based on physical exam, laboratory findings, patient history, and past medical history to establish mental status changes, coagulopathy, rapidity of onset, and absence of known prior liver disease respectively.
The exact definition of "rapid" is somewhat questionable, and different sub-divisions exist which are based on the time from onset of first hepatic symptoms to onset of encephalopathy. One scheme defines "acute hepatic failure" as the development of encephalopathy within 26 weeks of the onset of any hepatic symptoms. This is sub-divided into "fulminant hepatic failure", which requires onset of encephalopathy within 8 weeks, and "subfulminant", which describes onset of encephalopathy after 8 weeks but before 26 weeks. Another scheme defines "hyperacute" as onset within 7 days, "acute" as onset between 7 and 28 days, and "subacute" as onset between 28 days and 24 weeks.
Supperative granuloma are chronic inflammatory cells at periphery with central abscess formation composed of histiocytes and multinucleated giant cells with central collections of neutrophils.
A fibrin ring granuloma, also known as doughnut granuloma, is a histopathological finding that is characteristic of Q fever. On hematoxylin-eosin staining, the fibrin ring granuloma consists of a central lipid vacuole (usually washed-out during fixing and staining, leaving only an empty hole) surrounded by a dense red fibrin ring and epithelioid macrophages. Fibrin ring granulomas may also be seen in Hodgkin's disease and infectious mononucleosis.