1/89. Ferumoxide-enhanced MRI of sideronecrosis superimposed on genetic hemochromatosis.Genetic hemochromatosis is an autosomal recessive disorder characterized by excessive iron absorption from the gut, resulting in increased total body iron stores, multisystem organ dysfunction, and an increased risk of hepatocellular carcinoma. The magnetic susceptibility effects of excess hepatocellular iron generally cause diffuse hepatic signal loss on T2- or T2*-weighted MR images. Although hepatic iron deposition is usually diffuse, focal areas of iron sparing can occur, and, when present, superimposed neoplasm is a consideration. We describe a patient with cirrhosis, hemochromatosis, and multiple small benign relatively hyperintense iron-poor foci consisting of piecemeal sideronecrosis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/89. 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) |
3/89. Cirrhotic ascites, ovarian carcinoma, and CA-125.We describe two postmenopausal women with ascites and elevated CA-125 level, a serologic marker used to detect ovarian cancer. Both patients had unrecognized liver disease but underwent surgical exploration for suspected ovarian disease, which subsequently revealed benign pelvic organs. Elevated serum CA-125 levels have been reported in many patients with ascites due to liver disease and cirrhosis. Thus, the presence of both ascites and an elevated CA-125 level mandates a thorough elevation for liver disease as well as for a possibility of ovarian carcinoma. These cases outline the common finding and provide insight into the management of patients with ascites and elevated CA-125 values.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/89. Markedly elevated CA125 in hepatic cirrhosis: two case illustrations and review of the literature.CA125 is the most widely used tumor marker presently available for use in patients with epithelial ovarian cancer. Although elevated in a high percentage of patients with ovarian cancer, serum CA125 levels have also been detected in patients with numerous benign and malignant nongynecologic disorders, including various diseases of the liver. Despite this well-publicized fact, it has become apparent that the association between CA125 elevation, particularly the degree of elevation, and liver disease may not be as widely recognized as one would suspect. When marked CA125 elevations occur, diagnostic confusion is common. We describe two cases illustrative of this point. Both cases involve middle-aged women who presented with massive ascites and due to markedly elevated serum CA125 levels underwent exploratory laparotomy with hysterectomy and/or bilateral salpingo-oopherectomy before their referral to our center. Because preservation of a woman's reproductive organs is a significant concern, it is imperative that both primary care physicians and specialists are aware of such associations and the proper use of tumor markers.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/89. Cirrhosis in a child with hypothalamic syndrome and central precocious puberty treated with cyproterone acetate.Before the advent of gonadotropin-releasing-hormone analogues, cyproterone acetate (CPA) had been widely prescribed for the treatment of precocious puberty. Although it is usually well tolerated, liver toxicity has been recognized as a complication of its long-term use. We report the occurrence of cirrhosis in a 10-year-old boy with hypothalamic syndrome and precocious puberty who was treated with CPA for over 50 months. Despite discontinuation of the medication, the liver disease progressed. The patient died of sepsis and multiorgan failure at the age of 14 years. This is the first paediatric report of substantial liver damage and liver toxicity progressing to cirrhosis associated with CPA treatment. CONCLUSION: Prolonged cyproterone acetate treatment may induce cirrhosis. Monitoring of liver function both during treatment and for several months after discontinuation of therapy is recommended.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/89. Successful liver transplantation using an 87 year-old donor.The increasing number of patients listed for liver transplantation and the persistent low number of potential donors have led, in recent years, to the expansion of organ availability, as is the case of liver procurement from elderly donors. So far, results reported in the literature are controversial and only a few reports described satisfactory outcomes of transplantation using donors older than 60 years. In the case presented herein, we report the successful elective liver transplantation of a 65 year-old patient, who received a graft from an 87 year-old donor, whose well-preserved hemodynamic conditions, normal hepatic function, absence of parenchymal alterations, as well as short ischemia time, were the key points for this good outcome. Even very old donors can be suitable for liver transplantation. A careful evaluation of all the possible donor-related risk factors, mostly the presence of steatotic changes at liver biopsy, is always mandatory.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/89. 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) |
8/89. Various extrahepatic manifestations caused by hepatitis c virus infection.It has been reported that hepatitis c virus (HCV) causes not only liver disease but also disorders of other organs and tissues. Previously, many HCV-related extrahepatic manifestations have been reported. In this study, we report 2 patients in whom tongue cancer was detected during the treatment of HCV-related liver disease. In one patient, tongue cancer was detected during the treatment of HCV-related liver cirrhosis, and articular rheumatism developed thereafter. The duration of HCV-related liver disease was 10 years. In the other patient, tongue cancer was detected during the treatment of HCV-related hepatocellular carcinoma. This patient had a past history of thyroid disease. The duration of HCV-related liver disease was 6 years. In these patients, the possibility that several conditions incidentally and concurrently developed cannot be denied. However, the conditions described above may be regarded as HCV-related extra-hepatic manifestations. In patients with HCV infection, it is important to examine conditions in organs other than the liver. Careful follow-up is needed.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
9/89. Sequential observation of liver cell regeneration after massive hepatic necrosis in auxiliary partial orthotopic liver transplantation.The morphogenesis of hepatocytes after massive hepatic necrosis to recovery through liver cell regeneration has not been fully understood. Sequential biopsies were performed on the native liver of a 22-year-old man who underwent auxiliary partial orthotopic liver transplantation 1 month after fulminant hepatitis. Auxiliary partial orthotopic liver transplantation was successful, and the biopsy samples permitted us to examine the regenerating process of hepatocytes after massive necrosis. At the time of auxiliary partial orthotopic liver transplantation (postoperative day 0), 95% of hepatocytes were lost and a few ductules were found in the portal areas. The ductules stained with cytokeratin 19. At postoperative day 7, the ductules began to increase in size and number and became dilated over a period of 1 month, when individual hepatocytes with clear cytoplasm appeared from the ductules. As the differentiation of hepatocytes increased, the expression of cytokeratin 19 was found to decrease. From 2 to 3 months, all of the ductules were transformed into hepatocytes, and they began to form round cell clusters. From 3 to 6 months, the round cell clusters became organized into trabecula with fibrosis. From 6 to 12 months, a lobular architecture was established, and by 14 months, the necrotic liver was fully recovered to normal. This study by examination of sequential biopsies demonstrates the progression of the regenerating process from total hepatic necrosis to complete recovery.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/89. Severe type II gaucher disease with ichthyosis, arthrogryposis and neuronal apoptosis: molecular and pathological analyses.Severe infantile gaucher disease associated with ichthyosis and neonatal death is a rare subgroup of Type II gaucher disease. This group of infants has little, if any, detectable beta-glucocerebrosidase activity, and prior genetic analyses have been limited in detecting the mutations responsible for this phenotype. We document an Hispanic infant succumbing with arthrogryposis and collodion membrane covering the skin who had no detectable beta-glucocerebrosidase activity in tissue samples and who was homozygous for a rare recombinant allele, RecNciI. Microscopic evaluation demonstrated accumulation of Gaucher cells in visceral organs and extensive loss of neurons in the anterior horns, brainstem, and cortex of the nervous system. The apoptosis of neuronal cells from the anterior horns and brainstem are a reasonable explanation for the arthrogryposis and neonatal death, respectively.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
| | Next -> |