Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report our experience with nine adult patients (six females, three males) with Caroli's syndrome. Most of these patients were young (mean age 27.0 yr), and had presented at a mean interval of 12.1 yr after the onset of symptoms. Recurrent abdominal pain was the most common symptom, present in eight of nine patients. Mild jaundice (serum bilirubin 1.8-4.2 mg/dl) and raised serum alkaline phosphatase (18-36 King-Armstrong units) were the only other significant findings. Ultrasound proved to be a good screening test. However, the diagnosis was confirmed on cholangiography. Six of nine patients had associated extrahepatic biliary cysts (type IVa). The operative management was directed mainly at excising the extrahepatic cysts and draining the biliary tree, and was met with satisfactory results.
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PMID:Caroli's syndrome: a heterogeneous entity. 230 39

Forty cases of dilatation of the biliary tract in infants and children were diagnosed and treated at the Veterans General Hospital, Taipei and Taichung between 1980 and 1992. The male to female ratio was 1:2.6 and the age ranged from 1 day to 14 years. Major clinical presentations were abdominal pain (57.5%), jaundice (42.5%), vomiting (37.5%) and abdominal mass (30%); the classical triad of abdominal pain, jaundice and abdominal mass occurred in only three cases (7.5%). Abnormal liver function tests included elevated bilirubin (Bil) (22/37), aminotransferase (30/40), alkaline phosphatase (Alk-P) (34/37), lactic dehydrogenase (LDH) (22/34) and gamma-glutamyl transpeptidase (GGT) (13/15). The amylase in bile was detected in 16 cases with 10 cases higher than 500 Somogyi units/dL. All cases were diagnosed correctly, using real time sonography. Technetium-labeled scintiscan (Tc-99m DISIDA scan) was interpreted correctly in 88% (15/17) and abdominal computerized tomography (CT) in 10 cases (10/10). According to Todani's classification, type I cyst was the most common (35 cases, 87.5%) and the remaining cases were type IV-A (3 cases) and type V (2 cases). Operation was performed in 38 cases except for two who had type V dilatation of biliary tract (Caroli's disease). Complications included rupture of the gall bladder in two patients and common bile duct stone in one; mortality after operation occurred in one case. Surgical specimens of the liver revealed periportal fibrosis in nine instances.
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PMID:Dilatation of the biliary tract in pediatric patients. 836 78

We describe 8 young dogs with congenital dilatation of the intra- and extrahepatic bile ducts and diffuse cystic kidney disease, compatible with Caroli's disease in humans. The dogs were referred between 1980 and 2000 because of chronic disease at an age of 6 months to 3 years. These dogs included 3 Collies, 2 Frisian Stabyhouns, 2 Jack Russell Terriers, and 1 mixed-breed dog. The most common signs were vomiting (6/6), polyuria and polydipsia (4/6), and anorexia (4/6). Ascites was a common finding (4/6). Clinicopathologic abnormalities were available for 6 dogs. All had increased plasma alkaline phosphatase activity and fasting bile acids: increased alanine aminotransferase activity and urea and creatinine concentrations were present in 50% of dogs. Ultrasound examination of the liver showed severely dilated bile ducts without evidence of obstruction, and calcification in all cases but 1. Postmortem examination revealed severe dilatation of the larger intra- and extrahepatic bile ducts. The common bile duct and gall bladder were normal, and the bile system was patent. The ducts contained a clear viscid fluid often with calcified material. Microscopically, marked portal fibrosis was present, often with abnormally structured dilated bile ducts lined with columnar or cuboid epithelium and regularly small calcifications. The lesion was complicated by ascending cholangitis in 1 dog. The kidneys showed marked cortical and medullary fibrosis with a diffuse radial cystic pattern; only slight renal fibrosis was found in the oldest dog. Seven dogs were euthanized without treatment; the oldest dog was alive and well 5 months after diagnosis and was maintained on a protein-restricted diet.
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PMID:Congenital dilatation of the bile ducts (Caroli's disease) in young dogs. 1256 24