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Progressive familial intrahepatic cholestasis (PFIC) is a group of familial cholestatic conditions caused by defects in biliary epithelial transporters. The clinical presentation usually occurs first in childhood with progressive cholestasis. This usually leads to failure to thrive, cirrhosis, and the need for liver transplantation. [citation ...
Polycystic liver disease (PLD) usually describes the presence of multiple cysts scattered throughout normal liver tissue. [1] PLD is commonly seen in association with autosomal-dominant polycystic kidney disease , with a prevalence of 1 in 400 to 1000, and accounts for 8–10% of all cases of end-stage renal disease . [ 2 ]
Primary familial brain calcification [1] (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, [1] is a rare, [2] genetically dominant or recessive, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.
The first symptoms typically include fever, intermittent abdominal pain, and an enlarged liver.Occasionally, yellow discoloration of the skin occurs. [4] Caroli disease usually occurs in the presence of other diseases, such as autosomal recessive polycystic kidney disease, cholangitis, gallstones, biliary abscess, sepsis, liver cirrhosis, kidney failure, and cholangiocarcinoma (7% affected). [2]
This is a shortened version of the third chapter of the ICD-9: Endocrine, Nutritional and Metabolic Diseases, and Immunity Disorders.It covers ICD codes 240 to 279.The full chapter can be found on pages 145 to 165 of Volume 1, which contains all (sub)categories of the ICD-9.
This leads to jaundice and an enlarged liver. If the obstruction is not relieved, permanent damage may occur to the liver - scarring and cirrhosis - with the signs of portal hypertension (obstruction to the flow of blood through the liver) and ascites (fluid accumulation in the abdomen). There is an increased risk of cancer in the wall of the cyst.
Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone, but calcium can be deposited abnormally in soft tissue, [1] [2] causing it to harden. Calcifications may be classified on whether there is mineral balance or not, and the location of the calcification. [3]
Simple liver cysts are seen most commonly in women and kids. [2] In terms of pathophysiology, they are formed in response to developmental events and in response to trauma and inflammation. [ 2 ] In addition, liver cysts can be seen with polycystic kidney disease and echinococcosis infection ( hydatid disease ).