Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 4-week-old male infant was admitted to the hospital with acute gastrointestinal bleeding and marked coagulopathy secondary to vitamin K malabsorption in the presence of cholestasis. Physical examination revealed hepatomegaly and cutaneous haemangiomas. Ultrasonography, CT, and MRI demonstrated a multifocal vascular process and allowed the diagnosis of infantile hepatic haemangioendothelioma to be made without the use of more invasive diagnostic procedures. To avoid high-output congestive heart failure, the patient was treated with oral corticosteroids. After 5 months, rapid involution of the vascular malformations ensued. At age 2 years, a magnetic resonance scan confirmed complete resolution of the hepatic haemangioendothelioma.
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PMID:Imaging diagnosis and follow-up of infantile hepatic haemangioendothelioma: a case report. 815 13

Giant hepatic hemangiomas are rare entities. They can be symptomatic with pain and increased erythrocyte sedimentation rate. Cholestasis has already been reported. We describe a case of biliary dilatation secondary to a hemorrhagic giant hepatic hemangioma documented on CT and MRI.
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PMID:[Giant hepatic hemangioma causing dilatation of the intrahepatic biliary ducts]. 923 42

Cholestasis may present as extrahepatic (obstructive jaundice), as purely intrahepatic (hepatocellular), as part of a cholestatic hepatitis or as a result of intrahepatic obstruction. Anamnestic clues and clinico-chemical analyses (bilirubin, gamma-GT and alkaline phosphatase) together with sonography and finally ERCP usually will furnish the diagnosis. However, with technical advances computed tomography and MRI cholangiography will soon emerge as often useful techniques in hepatobiliary imaging.
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PMID:[Cholestasis: diagnosis]. 985 63

A 6 year-old boy with autoimmune hepatitis accompanied with cirrhosis was reported. He was admitted to our hospital because of abdominal distention, high fever, and diarrhea. Laboratory examination revealed abnormalities in hepatic function, cholestasis, anemia, thrombocytopenia, hypoalbuminemia, hypocomplementemia, and low concentration of coagulation factors. Abdominal MRI, and asialoglycoprotein receptor-mediated liver scintigraphy strongly indicated liver cirrhosis. Viral hepatitis, Wilson's disease, and antitrypsin deficiency were excluded serologically. Instead, hypergammaglobulinemia, and positive antinuclear antibody suggested autoimmune hepatitis, and the survey of anti-mitochondrial antibody, anti-smooth muscle antibody, and anti-LKM-1 antibody was negative, indicating type I autoimmune hepatitis. Finally, the histology of liver biopsy specimen indicating the destruction of hepatic lobular architecture, dense mononuclear cell infiltrates, and severe fibrosis confirmed the diagnosis. He was treated firstly with methylprednisolone pulses, and then prednisolone p.o. + azathioprine p.o. All of the abnormal laboratory parameters improved to normal levels, indicating that the immunosuppressive therapy will be effective for the severe AIH with cirrhosis.
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PMID:[An infant of autoimmune hepatitis (type I) with cirrhosis]. 1053 82

The aim of this study was to describe the clinico-pathological characteristics of malignant biliary papillomatosis and the diagnostic and therapeutic approach adopted in this case, which extended from the left hepatic biliary duct to the bifurcation of the 2nd and 3rd segments. The patient was studied with ultrasonography, endoscopic retrograde cholangiopancreatography (ERCP), CT scan and MRI cholangiography, which showed the morphological characteristics and the extent of the disease in the biliary tract. The malignancy of the disease was confirmed by preoperative cytological analysis of bile collected via a nasobiliary probe. The surgical treatment, preceded by intraoperative ultrasonography, consisted in a left hepatectomy, sparing the 1st segment, and a right intrahepatic jejunostomy. The final pathological analysis revealed a malignant biliary papillomatosis. Preoperative ERCP clearly documented the exact distribution of the biliary tract lesions and allowed both treatment of the cholestasis and diagnosis of the nature of the lesion through cytological analysis of the bile. Surgical treatment must be carried out and guided not only by the preoperative diagnosis, but also by perioperative ultrasonography. Finally, owing to the rarity of such cases and consequently our very limited knowledge of the disease, an intensive follow-up is recommended.
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PMID:[Malignant biliary papillomatosis: a case report]. 1083 46

When cholestatic liver disease is present, liver ultrasound should be performed to ascertain if cholestasis is extrahepatic or intrahepatic. If bile ducts appear dilated and the probability of interventional treatment is high, endoscopic retrograde cholagio-pancreatography (ERCP) or trans-hepatic cholangiography (THC) should be the next step. If the probability of interventional therapeutics is low, cholangio-MRI should be performed. Once bile duct dilation and space occupying lesions are excluded, a work up for intrahepatic cholestasis should be started. Some specific clinical situations may be helpful in the diagnostic strategy. If cholestasis occurs in the elderly, drug-induced cholestatic disease should be suspected, whereas if it occurs in young people with risk factors, cholestatic viral hepatitis is the most likely diagnosis. During the first trimester of pregnancy cholestasis may occur in hyperemesis gravidorum, and in the third trimester of gestation cholestasis of pregnancy should be suspected. A familial history of recurrent cholestasis points to benign recurrent intrahepatic cholestasis. The occurrence of intrahepatic cholestasis in a middle-aged woman is a frequent presentation of primary biliary cirrhosis, whereas primary sclerosing cholangitis should be suspected in young males with inflammatory bowel disease. The presence of vascular spider nevi, ascites, and a history of alcohol abuse should point to alcoholic hepatitis. Neonatal cholestasis syndromes include CMV, toxoplasma and rubinfections or metabolic defects such as cystic fibrosis, alpha1-antitrypsin deficiency, bile acid synthesis defects, or biliary atresia. The treatment of cholestasis should include a management of complications such as pruritus, osteopenia and correction of fat soluble vitamin deficiencies. When hepatocellular failure or portal hypertension-related complications occur, liver transplantation should be considered.
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PMID:Diagnostic and therapeutic approach to cholestatic liver disease. 1497 98

Prompted by the histomorphological aspect of ductopenia, chronic intrahepatic liver diseases are increasingly being subsumed under the term vanishing bile duct syndrome. Classification by cholestasis syndromes in adults (e.g. primary biliary cirrhosis, primary sclerosing cholangitis) and in the newborn or children (e.g. alphal antitrypsin deficiency, cystic fibrosis) makes good sense. Decisive for the diagnosis are, depending on the disease presenting, a typical constellation of laboratory results, detection of autoantibodies, imaging procedures (e.g. ERC, MRI), liver biopsy where indicated, or suspected drug-induced cholestasis. Byway of treatment, ursodeoxycholic acid, an antibiotic in cholestasis, and liver transplantation in some cases, are possible options. Supportive treatment should be aimed at extrahepatic manifestations of cholestasis (e.g. osteoporosis, vitamin deficiency, pruritus).
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PMID:[Diagnosis and treatment of intrahepatic cholestasis syndrome]. 1537 30

The modern cross-sectional radiological methods of ultrasound, computed tomography, and magnetic resonance imaging provide manifold possibilities for the diagnosis of inflammatory and tumorous diseases of the gallbladder and the bile ducts as well as stone-related diseases. The invasive methods for direct imaging of the bile ducts, such as endoscopic retrograde cholangiopancreatography, are mainly used within therapeutic concepts. According to the literature, ultrasound and magnetic resonance imaging show a sensitivity of up to 100% in the diagnosis of intra- and extrahepatic cholestasis, but concerning the diagnosis of the cause of cholestasis these methods are limited. Therefore, additional MRI sequences or computed tomography are necessary. Computed tomography is particularly efficient for the diagnosis of the biliary system and adjacent anatomical and pathological structures within the pre- and postoperative period.
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PMID:[Value of radiological methods in the diagnosis of biliary diseases]. 1624 Jan 38

Cholestasis is due to abnormal biliary secretion, from hepatic or extra hepatic causes. The diagnostic strategy of anicteric cholestasis will be discussed, defining hepatic biologic abnormalities, and the role and sequence of imaging techniques based on clinical and biological findings. Main causes will be emphasized and illustrated with different radiological techniques (US, CT and MRI).
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PMID:[Anicteric cholestasis: imaging and diagnostic strategy]. 1669 Nov 75

Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosiderosis has also been described to trigger carcinogenesis. A significant iron overload, as found in hereditary hemochromatosis (HHC), is a risk factor for HCC and may also promote the symptoms of porphyria cutanea tarda (PCT). A 68-year old male patient presented to our clinic with a suspected HCC, elevated alpha-fetoprotein but normal liver function tests. He reported a 25 year-old history of vitiligo upon exposure to sunlight. The patient underwent an extended left hemihepatectomy, and the recovery was uneventful, with the exception of a persistent hyperbilirubinemia. Perfusion problems and extrahepatic cholestasis were ruled out by CT-scan with angiography and MR-cholangiopancreatography. However, MRI showed an iron overload. Histology confirmed the HCC (pT3, pN0, G3, R0) and revealed a portal fibrosis and hemosiderosis. Based on the skin lesions we suspected a PCT that was confirmed by laboratory tests showing elevated porphyrin, uroporphyrin, coproporphyrin and porphobilinogen. Concurrently, molecular diagnostics revealed homozygosity for the C282Y mutation within the hemochromatosis HFE gene. After phlebotomy and normalization of liver function tests the patient was discharged. This is the first case ever showing the unusual combination of HCC in a fibrotic liver with HHC and PCT. This diagnosis not only warrants oncological follow-up but also symptomatic therapy to normalize iron metabolism and thereby improve liver function and alleviate the symptoms of HHC and PCT. Thus progression of fibrosis may be prevented and liver regeneration supported.
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PMID:An unhappy triad: hemochromatosis, porphyria cutanea tarda and hepatocellular carcinoma-a case report. 1746 5


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