Cases reported "Jaundice"

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1/14. Heterotopic gastric mucosa involving the gallbladder and biliary tree.

    A case of heterotopic gastric mucosa in the common bile duct, cystic duct and gallbladder is reported in a 3-year-old girl with abdominal pain and jaundice. Abdominal US and CT showed dilatation of the biliary tree and a well-defined mass in the common bile duct that narrowed its lumen. The gallbladder was contracted in both examinations. The common bile duct and the gallbladder were resected and a choledochojejunostomy was performed. Although gastric heterotopy has been described throughout the entire length of the gastrointestinal tract, it is a very uncommon finding in the gallbladder and extremely rare in the biliary tree. A firm diagnosis of gastric heterotopia is based on the presence of fundal mucosa replete with parietal and chief cells. To our knowledge, this is the fifth reported case of heterotopic gastric tissue within the common bile duct, and the first to describe the US and CT findings. A relevant literature review and brief outline of the histological and radiological features are included in the discussion.
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2/14. Smallest Todani's type II choledochal cyst.

    A choledochal cyst is defined as an isolated or combined congenital dilation of the extra hepatic or intrahepatic biliary tree. Todani and colleagues proposed the five types of congenital choledochal cysts which have gained widespread acceptance. Type II choledochal cyst, a diverticulum of common bile duct, is rarest, and most reported cases of Type II were as large as several centimeters in size. We herein report the case of a small Type II choledochal cyst which was resected at pancreatoduodenectomy for carcinoma of the papilla of Vater. A 58-year-old Japanese male was referred to our hospital for the evaluation of jaundice. Preoperative cholangiogram via the percutaneous transhepatic biliary drainage tube revealed a complete obstruction at the narrow terminal segment. Furthermore, a small diverticular protrusion was demonstrated on the lower part of the common bile duct. The resected specimen showed a 2.2 x 1.7 x 1.2 cm carcinoma of the major papilla, and a deep, 2 mm in diameter and 5 mm in depth, depression on the posterior wall of the common bile duct. The anomalous pancreatobiliary duct was not seen. The deep depression was confirmed microscopically to penetrate the fibromuscular layer of the common bile duct and diagnosed as a Todani's Type II choledochal cyst. To our knowledge, the current case is the smallest Type II choledochal cyst which was completely resected.
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3/14. Clinical problems with developmental anomalies of the biliary tract.

    Cholestatic jaundice defined as conjugated hyperbilirubinemia is a typical feature of neonatal liver disease. biliary atresia is the most common disorder producing cholestasis during the first 2 months of life. Syndromic and non-syndromic paucity of the intralobular bile ducts and choledochal cysts can also present with cholestasis during early life. Liver dysfunction from obstruction of the biliary tree must be differentiated from numerous disorders affecting hepatocytes such as congenital infection and inborn errors of metabolism. Early recognition and a stepwise diagnostic evaluation of the cholestatic infant are essential in successfully treating many metabolic and infectious liver diseases of the infant as well as surgically relieving obstruction in patients with biliary atresia.
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4/14. Clinical vignette in antiretroviral therapy: jaundice.

    hiv caregivers face many challenges following initiation of ART. The development of jaundice is uncommon but worrisome. In this case, two distinct and contrasting episodes of jaundice were observed. In the first instance, isolated elevation of the indirect bilirubin without elevation of the alkaline phosphatase was noted. The normal PT and serum aminotransferase levels indicate the absence of intrinsic liver dysfunction. Elevations in the indirect bilirubin may result from either impaired uptake/conjugation or excess production. The latter, usually from acquired hemolysis, may be a complication of an occult NHL. A work-up for this AIDS-related malignancy was not initiated since the caregivers recognized jaundice as a complication of IDV, which inhibits UDP-glucuronyl transferase and produces a Gilbert's-like syndrome. physicians can expect to encounter this syndrome even more frequently with ATV. Experienced patients given RTV-boosted ATV have experienced elevations of unconjugated hyper-bilirubinemia in up to 45 percent of cases in clinical trials. However, such elevations do not reflect liver dysfunction and symptomatic jaundice requiring dosage reduction that occurred infrequently (7 to 8 percent of study patients). counseling patients about this syndrome may promote adherence and prevent self-directed interruptions of ATV that compromise efficacy. The second case of jaundice provides a more formidable diagnostic challenge. The triad of LFT abnormalities (mild elevation of aminotransferases, normal PT, and marked cholestatic jaundice) implies an acute process that is mildly toxic to hepatocytes without affecting their synthetic function. The subacute nature of the patient's cholestatic jaundice suggests either intrahepatic infiltrative disease of the liver or extrahepatic obstruction of the biliary tree, most likely due to the patient's relatively modest level of pain and lack of fever. Despite LFT abnormalities occurring 17 months after a switch in his ART, cumulative drug-related toxicities must still be considered. ritonavir can produce significant elevations in the AST/ALT, especially with pre-existing chronic liver disease as with hepatitis c virus coinfection. The NRTIs can produce hepatic steatosis, a result of mitochondrial toxicity and impaired fatty acid oxidation. However, jaundice and cholestasis are not typical of the latter syndrome. With a negative contrast CT that excludes parenchymal liver disease, investigation of the biliary tree to assess the presence of AIDS-related cholangitis was the next step. Performing a sphincterotomy or stent placement, and obtaining brushings or biopsy specimens to determine the extent of extrahepatic obstruction may help define a pathogen and be life-saving. The negative results of the ERCP justify the final diagnostic step, a liver biopsy to evaluate microscopic infiltrative disease that might not have been detected on contrast abdominal CT. Examples might include granulomatous disease (MAC), fungal etiologies (histoplasmosis), carcinomatosis (lymphoma, hepatoma, cholangiocarcinoma), and microvascular disease (bacillary angiomatosis). The failure to observe granulomatous inflammation in the liver does not exclude MAC infection, as MAC may involve other peri-aortic or mesenteric lymph nodes. This form of iris is unlikely given the abdominal CT findings, lack of systemic complaints, and extended persistence of liver aminotransferases. The nonspecific results of the liver biopsy are a common outcome in advanced AIDS patients with elevated alkaline phosphatase levels. Despite not having identified a pathogen, the biopsy establishes chronic liver disease and prompts re-evaluation and change of treatment to NFV. The subsequent normalization of the patient's aminotransferase levels suggests a prior adverse effect of LPV/r in the setting of unexplained, chronic liver disease. Most importantly, this case highlights the importance of hiv caregivers to review ART for safety when noting chronic liver dysfunction. patients need to be counseled to minimize acetaminophen use, to consume alcohol in moderation, and to avoid behavior with risk for hepatitis c. Finally, all hiv patients should receive appropriate vaccination against hepatitis a and B if serology shows lack of protective immunity.
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5/14. Dual hereditary jaundice: simultaneous occurrence of mutations causing Gilbert's and Dubin-Johnson syndrome.

    BACKGROUND & AIMS: Dubin-Johnson syndrome is recessively inherited, conjugated hyperbilirubinemia induced by mutations in the ABCC2/MRP2 gene encoding the canalicular transporter for conjugated bilirubin. Gilbert's syndrome is recessively inherited, unconjugated hyperbilirubinemia caused by decreased conjugation rate of bilirubin associated mostly with homozygous A(TA) 7 TAA variant of the TATAA-box in the UGT1A1 gene promoter. Our aim was to establish the molecular diagnosis in a 3-year-old male with atypical, intermittent, predominantly unconjugated, hyperbilirubinemia. methods: 99m Tc-HIDA cholescintigraphy was used for imaging the biliary tree. Expression of ABCC2/MRP2 protein in hepatocytes was investigated immunohistochemically. UGT1A1 and ABCC2/MRP2 genes were sequenced from genomic dna, and the mutations were verified by fragment analysis, sequencing the cloned exons, and restriction fragment length polymorphism. RESULTS: Cholescintigraphy revealed delayed visualization of the gallbladder. A brown granular lipopigment differing from melanin-like pigment reported in Dubin-Johnson syndrome was present in hepatocytes, but, otherwise, liver histology was normal. ABCC2/MRP2 protein was not detected on the canalicular membrane of hepatocytes, and 2 novel mutations were found in the ABCC2/MRP2 gene: a heterozygous in-frame insertion-deletion mutation 1256insCT/delAAACAGTGAACCTGATG in exon 10 inherited from the father and a heterozygous deletion 4292delCA in exon 30 inherited from the mother. In addition, the patient was homozygous for -3279T>G and A(TA) 7 TAA mutations in the UGT1A1 gene promoter. CONCLUSIONS: Our patient represents a case of digenic mixed hyperbilirubinemia-a distinct type of constitutive jaundice resulting from coinherited defects in ABCC2/MRP2 and UGT1A1 genes.
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6/14. Traumatic avulsion of the intrapancreatic common bile duct: case report.

    Injuries of the extra hepatic biliary tree following blunt trauma to the abdomen are rare. We present a case of avulsion of the intrapancreatic common bile duct. Very often the lesion is not identifiable until the signs of jaundice and biliary ascites occur. Intraoperative cholangiography is mandatory for the diagnosis, but the noninvasive magnetic resonance cholangiopancreatography could readily depict the injury of the extrahepatic bile duct preoperatively. When the diagnosis is late the corner stone of treatment is biliary diversion and definitive repair after complete resolution of sepsis with a choledochojejunostomy.
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7/14. Granular cell myoblastoma of the biliary tree.

    A granular cell myoblastoma of the common bile duct is described which caused episodes of jaundice in a young woman. Histological examination demonstrated a close association of the tumour with nerve fibres, tending to support the theory of origin from schwann cells. The lesion is benign and after excision carries an excellent prognosis.
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8/14. Embryonal rhabdomyosarcoma of the biliary tree in children: a case report.

    Embryonal rhabdomyosarcoma (ERS) in children is a very rare tumor. jaundice is the capital symptom. diagnosis can be made by echography, which shows echogenic formations without posterior conic shadows in a dilated biliary duct. The prognosis is bleak in spite of the combination of surgery, radiotherapy and chemotherapy.
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9/14. Hepatic toxicity associated with gold therapy.

    Three patients with rheumatoid arthritis developed jaundice after initiation of chrysotherapy. gold sodium thiomalate had been administered in dosages of 37.5, 60, and 110 mg in these patients before the onset of jaundice. Liver function studies indicated a cholestatic jaundice in all subjects. One patient underwent exploratory laparotomy because of progressive jaundice. Liver biopsy was done in two patients. In one patient significant bile stasis and thrombi were seen in the biliary tree. In another patient liver biopsy showed ballooning of hepatocytes with minimal cholestasis. All patients recovered spontaneously. awareness of this rare complication may prevent unnecessary diagnostic and surgical procedures in patients with rheumatoid arthritis who may develop jaundice while receiving chrysotherapy.
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10/14. Postoperative jaundice in children. The influence of halothane.

    At the Hospital for Sick Children, Great Ormond Street, during the 23-year period 1957 to 1979, 165400 anaesthetics were administered. Almost all of the patients anaesthetised during this time would have been exposed to halothane. Seventy-four patients became jaundiced for the first time in the post-operative period. halothane-associated hepatitis was excluded as the cause of the postoperative jaundice in all but two of the 74 patients. In these two patients in whom the diagnosis of halothane-associated hepatitis was possible the hepatitic illness was mild and both patients made an uneventful recovery. In this survey the risk of a patient becoming jaundiced due to halothane associated hepatitis was greater than 1 in 82000. It would seem that in children halothane can be used whenever it is warranted and can be used repeatedly.
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