1/68. liver transplantation for endstage hepatitis c cirrhosis in a patient with primary hypogammaglobulinaemia.liver transplantation was performed in a patient with primary hypogammaglobulinaemia, chronic hepatitis c and hepatic failure. The immediate posttransplant period was uncomplicated. Owing to a stricture of the choledochojejunostomy the patient was reoperated with construction of a hepaticojejunostomy 11 months posttransplant. The patient remained hepatitis c virus (HCV) rna-positive, with high and increasing levels of HCV. Liver biopsies demonstrated the recurrence of HCV. 14 months after the transplantation the patient developed severe diarrhoea caused by cryptosporidium parvum. The infection did not respond to available therapeutic measures. He deteriorated with development of liver failure and died 18 months after the transplantation. The present case report illustrates the difficulties associated with organ transplantation in patients with primary hypogammaglobulinaemia.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/68. Erythropoietic protoporphyria with fatal liver failure.A 33-year-old woman with a history of photosensitivity, persistent abdominal pain, and liver dysfunction was admitted to our department because of abdominal pain and progression of liver dysfunction. On admission, levels of protoporphyrin and coproporphyrin within erythrocytes were markedly increased. Autofluorescent erythrocytes were also detected, leading to a diagnosis of erythropoietic protoporphyria. A liver biopsy specimen revealed cirrhosis with dark brown granules filling hepatocytes, bile canaliculi, and bile ductules. Transfusion of washed erythrocytes, hemodialysis, and administration of cholestyramine and beta-carotene transiently improved levels of porphyrins and liver function. The patient died of rupture of esophageal varices followed by multiple organ failure. However, the treatments were believed to have extended survival.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
3/68. Remarkable case of early Aspergillus endophthalmitis.BACKGROUND: This case demonstrates the early stage of Aspergillus endophthalmitis and is the second ultrastructural study of endogenous Aspergillus endophthalmitis. It is the first description of phagocytosis of Aspergillus fungi by retinal pigment epithelium (RPE). methods: A case report and detailed light- and electron microscopic findings are presented. RESULTS: Histopathological examination of serial sections of the affected right eye displayed a spread of aspergillus fumigatus fungi along two separate paths: via the retinal and choroidal vessels. The retinal and choroidal lesions were not contiguous. The organisms penetrated blood vessel walls, Bruch's membrane and the internal limiting membrane, but not the RPE layer. A curious accumulation of the Aspergillus fungi was present on the internal aspect of Bruch's membrane, where the RPE acted as a barrier and the subretinal space was not invaded. phagocytosis of fungi by the RPE was observed. No inflammatory cells were present between Bruch's membrane and the RPE. CONCLUSIONS: This report describes a remarkable barrier function, possible local immunosuppression and phagocytosis by the RPE cells in a case of early Aspergillus endophthalmitis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/68. Successful treatment of a patient suffering from severe acute potassium dichromate poisoning with liver transplantation.BACKGROUND: Oral ingestion of potassium dichromate produces a complex spectrum of complications. It has an extremely poor prognosis and usually leads to rapid death. methods: We report the case of a 16-year-old male patient who was admitted to hospital after oral ingestion of potassium dichromate with suicidal intention. RESULTS: The patient's condition deteriorated, and he became comatose within 5 days in spite of immediate attempts at detoxification. Because of irreversible liver failure, which occurred within 2 days after admission, and because of cerebral edema, the decision to perform a liver transplantation was made. On day 6 after admission, a compatible donor liver was transplanted. The course of liver transplantation and the patient's subsequent recovery were uneventful. CONCLUSION: The rationale for the delayed transplantation was to avoid damage of the new organ because of high serum chromium levels. Despite severe organ damage, the chromium content of the liver was increased. To the authors' knowledge, this is the first case report of acute toxic liver failure, caused by potassium dichromate poisoning, treated successfully by means of liver transplantation.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
5/68. End-stage liver disease as the only consequence of a mitochondrial respiratory chain deficiency: no contra-indication for liver transplantation.The prerequisite for liver transplantation as a therapeutic option for inherited metabolic diseases should be that the enzyme defect, being responsible for the major clinical (hepatic and/or extra-hepatic) abnormalities, is localised in the liver. Furthermore, no adequate dietary or pharmacological treatment should be available or such treatment should have an unacceptable influence on the quality of life. We report an infant, who developed end-stage liver disease with persistent lactic acidaemia in his first months of life. Analysis of the mitochondrial respiratory chain in liver tissue revealed a combined partial complex I and IV deficiency. No extra-hepatic involvement could be demonstrated by careful screening for multiple organ involvement, including analysis of the mitochondrial respiratory chain in muscle tissue and cultured skin fibroblasts. The boy received a reduced size liver graft at the age of 8 months. He recovered successfully. Almost 5 years after transplantation he is in good clinical condition. No clinical or biochemical signs of any organ dysfunction have been demonstrated. The considerations on which basis it was decided that there was no contra-indication to perform liver transplantation in this patient are discussed. CONCLUSION: The possibility of a mitochondrial respiratory chain deficiency should be considered in liver disease of unknown origin prior to liver transplantation. liver transplantation is a therapeutic option in mitochondrial respiratory chain deficiency-based end-stage liver disease provided that extra-hepatic involvement is carefully excluded.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
6/68. Polyacrylonitrile membrane interposition between a xenograft and a patient in fulminant liver failure: the concept of xenohemodiafiltration in clinical practice.Fulminant hepatic failure is an important cause of morbidity and mortality in intensive care units. Conventional therapies are not sufficiently effective. liver transplantation may be life saving, but a "bridge therapy" is needed until transplantation is performed. Hepatic extracorporeal xenohemodiafiltration (XHDF) is aimed at the transitory support of a patient with fulminant hepatic failure. The first clinical case of XHDF is presented. The system consisted of cross-circulation between a porcine liver and a patient with fulminant liver failure through a polyacrylonitrile membrane. The procedure lasted for 5 hours and produced hemodynamic, biochemical, and metabolic improvements. intracranial pressure decreased from 34 to 5 cm H2O, serum ammonia fell from 673 to 370 ng/dl, lactic acid from 11 to 5.3 mmol/L, and bilirubin from 7.4 to 2.5 mg/dl. Hemodynamic values were maintained stable throughout the procedure. The patient was able to undergo transplantation and remains alive 11 months later. XHDF is a clinical experimental method that can constitute an alternative clinical therapy to support patients with fulminant hepatic failure until an organ is available for transplantation.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/68. Coexistent cutaneous Aspergillus and cytomegalovirus infection in a liver transplant recipient.Cutaneous infections are a significant cause of morbidity in solid organ recipients. These infections may be complex with multiple pathogens occurring in the same lesion. We describe the unusual association of cutaneous Aspergillus and cytomegalovirus (CMV) infections in a liver transplant recipient. Cutaneous CMV infection is rare and often indicates severe systemic involvement, whereas Aspergillus is a frequent cause of opportunistic cutaneous fungal infection. Seven weeks after liver transplantation, our patient had hemorrhagic, eroded plaques develop on his arms. The results of routine histology, immunoperoxidase staining for CMV antibody, and fungal culture revealed coexistent cutaneous aspergillus flavus and CMV infections. The patient was treated with ganciclovir, amphotericin b, and topical terbinafine cream; however, 2 weeks after the development of the cutaneous lesions, the patient died.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/68. Orthotopic liver transplantation for mitochondrial respiratory chain disorders: a study of 5 children.BACKGROUND: Liver involvement in mitochondrial respiratory chain disorders (MRCD) frequently ends in liver failure and death. Because of the high risk of extrahepatic, particularly neuromuscular, manifestations of the disease, the indication of orthotopic liver transplantation (OLT) in these patients remains controversial. We report on 5 such children in whom OLT was carried out, in an attempt to help clarify the matter. patients: patients 1 and 2 presented with fulminant liver failure at ages 7 and 6 months respectively. Emergency liver transplantation was performed before etiological investigations were completed. Retrospective examination of the explanted livers showed defects in complexes I, III and IV. In patient 1, severe neurological deterioration occurred 2 months after OLT with fatal outcome 9 months later. Patient 2 is alive 22 months after OLT with moderate motor impairment. patients 3, 4 and 5 presented with progressive liver failure before 6 months of age. Surgical liver biopsies displayed a 50% defect in complex IV (patient 3), a defect in complexes I, IV (patient 4) and in complexes I, III, IV (patient 5). Because there was no clinical extrahepatic involvement on investigations, OLT was carried out in these patients. Patient 3 died of multiple organ failure soon after OLT, patients 4 and 5 are alive respectively 21 months and 12 months after OLT with normal neurological examination. CONCLUSION: OLT may be a valid therapeutic option in infants with delayed liver cell failure due to MRCD, only after performing in emergency a thorough inves tigation to exclude clinically significant extrahepatic, especially neuromuscular, involvement.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/68. Mitochondrial dna depletion, near-fatal metabolic acidosis, and liver failure in an hiv-infected child treated with combination antiretroviral therapy.A child with controlled human immunodeficiency virus infection presented with neurologic deterioration, lactic acidosis, and organic aciduria. Muscle biopsy revealed abnormal mitochondrial (mt) morphology, reduced mt enzyme activities, and mtDNA depletion. After adjustment of antiretroviral therapy to a regimen free of nucleoside analogs, marked improvement was seen in clinical status and mt abnormalities.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/68. Diffuse hepatic intravascular carcinomatous embolization resulting in fatal liver failure: a clinicopathologic study of 4 cases.The authors report four cases in which patients died of acute or fulminant hepatic failure resulting from massive intravascular metastatic carcinomatous embolization, a rarely reported manifestation of metastatic disease. neoplasms were high grade carcinomas. Tumor emboli were present within portal branches ranging 0.12-2.9 mm in diameter and were free floating or attached to the vascular wall, with or without varying degrees of superimposed organization. In one case, intravascular tumor necrosis was prominent and appeared as granular casts with superimposed dystrophic calcification and/or entrapped foamy histiocytes. There were associated geographical areas of parenchymal (4 cases) and tumor (1 case) ischemic necrosis with a multifocal and regional topographic distribution. An associated predominant pattern of intrasinusoidal tumor infiltration (with or without fibrosis) was present in 3 cases, whereas the fourth case had underlying micronodular cirrhosis, providing ancillary evidence for preexisting altered intrahepatic microcirculation. The literature on fatal hepatic failure resulting from neoplasia is reviewed with a reassessment of its pathobiological significance.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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