Budd-Chiari syndrome refers to the clinical picture that occurs when there is partial or complete hepatic venous outflow obstruction.
Budd-Chiari syndrome is rare. A Japanese study estimated the prevalence to be in the region of 2.4 cases/million 4. In Western populations, the mo...
Glycogen storage disease (GSD) refers to a number of syndromes which are characterised by a defect in synthesis, metabolism or storage of glycogen.
There are many types of GSD:
type I: von Gierke disease
type II: Pompe disease
type III: Cori or Forbes disease
type IV: Andersen di...
HELLP syndrome is a pregnancy-related condition and is an abbreviation for:
elevated liver enzymes and
It is considered a severe and life threatening form of pre-eclampsia although it can occur without co-existing pre-eclampsia.
The estimated inciden...
Hepatopulmonary syndrome (HPS) refers to the combination of
hepatic dysfunction (cirrhosis)
hypoxaemia (alveolar-arterial O2 gradient of >15mmHg; >20mmHg in >64year old patients).
peripheral pulmonary arterial dilatation (due to right to left micro-shunts).
It is estimated to b...
Hereditary haemorrhagic telangiectasia (HHT) is also known as Osler-Weber-Rendu syndrome.
Worldwide prevalence ~1.5 per 100,000. Wide geographic variability with much higher incidence in certain regions, e.g. 1 in 200 in Dutch Antilles, 1 in 3500 in France.
Lemmel syndrome is defined as an obstructive jaundice caused by a periampullary duodenal diverticulum (of the second part of the duodenum) compressing the intrapancreatic part of the common bile duct with resultant upstream dilatation of the extra- and intrahepatic bile ducts.
Low phospholipid-associated cholelithiasis (LPAC) syndrome is one of the syndromes associated with ABCB4/MDR3 mutation. Characteristics of this syndrome include 1,2:
symptomatic cholesterol stones with early onset (<40 years)
recurrent symptoms post cholecys...
The Mirizzi syndrome refers to an uncommon phenomenon which results in extrinsic compression of an extrahepatic biliary duct from one or more calculi within the cystic duct or gallbladder. It is a functional hepatic syndrome but can often present with biliary duct dilatation and can mimic other ...
Pepper syndrome is of interest only (the term is not readily used in day-to-day practice), and refers to primary adrenal neuroblastoma with extensive liver metastases 1. In essence, it refers to stage 4S neuroblastoma (see staging of neuroblastoma).
Stauffer syndrome is paraneoplastic nephrogenic hepatomegaly. It most commonly occurs in the setting of renal cell carcinoma, and is the enlargement the liver without hepatic metastases. It is a cause of cholestasis and cholestatic jaundice.
History and etymology
It is named after Maurice H. ...
Whipple triad is the clinical presentation of pancreatic insulinomas and consists of:
fasting hypoglycemia (<50 mg/dl)
symptoms of hypoglycemia
immediate relief of symptoms after the administration of IV glucose
History and etymology
As a good piece of trivia, one would suspect that Whipple...