Cases reported "Liver Diseases"

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1/55. Combined hepatocellular and cystadenocarcinoma presenting as a giant cyst of the liver--a case report.

    Primary cystic lesions of the liver are very rare. Most of the solid tumours are hepatocellular carcinomas (HCC) with a smaller number being cholangiocarcinomas. The association of HCC with other primary liver malignancies is also extremely rare. This case report is about a 27 year old male patient who presented with a giant cystic lesion of the left liver. A CT scan showed a cystic lesion with internal septations and a thrombus in the main portal vein. The patient underwent an extended left hepatectomy and a portal venotomy with removal of the thrombus. Coexistent hepatocellular and cystadenocarcinoma were reported on histopathological examination. The patient was put on 5-FU postoperatively. He is doing well 11 months after surgery.
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2/55. Liver disease in Navajo neuropathy.

    OBJECTIVE: To describe clinical and histologic features of liver disease in infants and children with Navajo neuropathy (NN). methods: physicians at Navajo Area Indian health Service facilities and neurologists and gastroenterologists at regional referral hospitals were surveyed for identification of patients born between 1980 and 1994 with known or suspected NN. Clinical records and liver histologic findings were reviewed. RESULTS: Liver disease was present in all children with NN. Three clinical phenotypes of NN were observed, based on age at presentation and course: infantile NN presented in 5 infants before 6 months of age with jaundice and failure to thrive and progressed to liver failure before 2 years of age; childhood NN presented in 6 children between 1 and 5 years of age with liver dysfunction, which progressed to liver failure and death within 6 months; and classical NN presented in 9 children with variable onset of liver disease but progressive neurologic deterioration. Liver histologic findings were characterized by multinucleate giant cells, macrovesicular and microvesicular steatosis, pseudo-acini, inflammation, cholestasis, and bridging fibrosis and cirrhosis. Cases of all 3 phenotypes occurred within the same kindred. CONCLUSIONS: Liver disease is an important component of NN and may be the predominant feature in infants and young children. We propose changing the name of this disease to Navajo neurohepatopathy.
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3/55. Liver granulomatosis is not an exceptional cause of hypercalcemia with hypoparathyroidism in dialysis patients.

    In 4 of our patients on chronic dialysis, we were intrigued by the association of hypercalcemia /- hyperphosphatemia and normal intact PTH, with anicteric cholestasis without cytolysis. This picture occurred in 2 patients after they resumed dialysis because of a transplant rejection and in a third one after discontinuation of corticosteroids, prescribed for an idiopathic thrombocytopenia. No patient was under calcitriol, CaCO3 therapy, and their hypercalcemia persisted on a low calcium dialyzate (1.25 mmol/l). Obvious etiologies of hypercalcemia were not found: vitamin d or A intoxication, hyperparathyroidism, aluminum intoxication, hemopathy, hiv infection. The hypothesis of a granulomatous disease was made and a liver biopsy was performed showing granulomas with giant epitheloid cells. In one case foreign material (silicon ?) was present in the macrophages. Extensive investigations for sarcoidosis, tuberculosis and mycosis were negative. In 2 cases the so-called "dialysis" granulomatosis actually occurred in transplanted patients, suggesting the role of a transplantation related factor (toxic or virus). In the last case HCV seroconversion was present. In the 4 cases, corticotherapy led to the disappearance of hypercalcemia and to an increase of PTH. Our patients had the biological pattern of low bone turnover disease (hypercalcemia and normal intact PTH) and bone biopsy performed in 2 showed osteomalacia or ABD without aluminum. The association of this pattern with cholestasis should evoke liver granulomatosis, which should be confirmed by a liver biopsy and lead to a treatment by corticosteroids. The masking effect of previous corticoid therapy for transplantation should be pointed out. In 2 cases serial monitoring of plasma calcitriol showed a relation between decreasing high normal calcitriol with prednisone and normalization of calcemia, suggesting the role of inappropriate synthesis of calcitriol by the granuloma. In conclusion, liver granulomatosis should be looked for in dialysis patients on the association of unexplained hypercalcemia and normal PTH with anicteric cholestasis, and confirmed by a liver biopsy. Although still of unknown etiology, its evolution is favourable under corticotherapy.
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4/55. prenatal diagnosis of a fetal abdominal cyst.

    Hepatic liver cysts are rare and few cases have been described in the literature. Initially, these cysts are detected by a prenatal ultrasound as abdominal, and consequently, the final diagnosis of hepatic cysts is done during the postnatal period. We present a case of a giant hepatic cyst found incidently during a routine ultrasound examination. The diagnosis and management of a fetal intraabdominal cyst is discussed.
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5/55. Giant hepatic granuloma caused by bartonella henselae.

    We report a 10-year-old girl with a 3.0- by 3.5-cm giant hepatic granuloma caused by bartonella henselae. Such a solitary and large granuloma associated with B. henselae infection has not been previously reported. We believe that B. henselae infection is a consideration in the differential diagnosis of a large hepatic mass.
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6/55. Cavernous hemangioma of the liver with giant cyst formation: degeneration by apoptosis?

    Cavernous hemangioma of the liver with cyst formation is a very rare condition. A case of cavernous hemangioma of the liver with unilocular giant cyst formation undergoing surgical removal is reported. Notably, the patient also had budd-chiari syndrome with an obstructing lesion in the inferior vena cava. The cystic degeneration of the hemangioma implied a relationship with apoptosis. This is the first reported case of budd-chiari syndrome caused by advanced cystic degeneration of hepatic cavernous hemangioma.
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7/55. Giant hepatomegaly and portal hypertension in an elderly patient with primary liver amyloidosis: an uncommon clinical occurrence.

    Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloid, characterized by marked hepatomegaly and portal hypertension without hepato-cellular failure and by a severe prognosis, without systemic involvement, is less frequent. The authors describe an unusual case of primary hepatic amyloidosis with giant hepatomegaly, intrahepatic cholestasis, portal hypertension and splenomegaly, occurred in an elderly patient.
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8/55. Liver penetration by a duodenal ulcer in a young woman.

    Liver penetration is a rare but serious complication of peptic ulcer disease. We report a case of a 33-year-old woman who took large doses of nonsteroidal antiinflammatory drugs and developed a giant duodenal ulcer that penetrated into her liver. The diagnosis was based on histologic examination of endoscopic biopsies. She was initially treated with a proton pump inhibitor, but, within 5 weeks, she developed a symptomatic postbulbar stricture that required surgical correction. We also review 11 other reported cases of endoscopically and histologically diagnosed peptic ulcer penetration into the liver.
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9/55. Treatment of kasabach-merritt syndrome by embolisation of a giant liver hemangioma.

    We report the case of a 14-month-old child with kasabach-merritt syndrome, due to a giant liver hemangioma. The therapeutic approach consisted of peripheral transcatheter embolisation of the right hepatic artery with Ivalon microspheres without the addition of thrombogenic material. This procedure brought to a sensible permanent reduction of the size of the liver hemangioma with normalisation of the previous altered coagulation parameters after 6 years of follow-up.
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10/55. A giant multiacinar macroregenerative nodule in an explanted liver.

    Macroregenerative nodules (MRN) have been detected with increased incidence in explanted livers since orthotopic liver transplantation (OLTx) has become a routine treatment for end-stage liver disease. autopsy series suggest that MRN may be more common than once thought, and several studies point to the malignant potential of these lesions. With increasing waiting times for OLTx, the propensity for these premalignant lesions to arise in cirrhotic livers has important implications for the supervision of patients on OLTx waiting lists. We present here a striking example of a MRN and review a topic that is generating considerable interest.
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