1/622. Recurrent fetal polycystic kidneys associated with glutaric aciduria type II.A woman had two pregnancies terminated in the 20th and 21st weeks of gestation after ultrasonographic detection of enlarged hyperechoic kidneys in both fetuses. The combination of polycystic kidneys and steatotic liver found at autopsy suggested glutaric aciduria type II (GA II), which was confirmed by biochemical investigation. GA II or multiple acyl-coa dehydrogenase deficiency is an autosomal recessively inherited defect of mitochondrial energy metabolism, which usually results in neonatal death. When pregnancy is terminated because of enlarged hyperechoic kidneys in the fetus, autopsy is crucial for establishing the correct diagnosis. The combination of polycystic kidneys and steatotic liver should bring GA II to mind, and prompt appropriate biochemical investigations so that genetic counselling and first trimester diagnosis can be offered in future pregnancies.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/622. Segment IV liver cyst with biliary communication following laparoscopic deroofing.Simple cysts of the liver rarely have a biliary communication. We record the development of a biliary communication following laparoscopic deroofing of a segment IV simple cyst of liver and document its successful sclerosis with tetracycline.- - - - - - - - - - ranking = 3keywords = liver (Clic here for more details about this article) |
3/622. 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 = 0.5keywords = liver (Clic here for more details about this article) |
4/622. Autologous bone marrow transplantation for recurrent malignant lymphoma after liver transplantation.BACKGROUND: Cancer chemotherapy in chronic carriers of hepatitis b virus is known to promote viral replication, and, when immunosuppressive treatment is stopped, the return of immune competence can be followed by a fulminant hepatitis. liver transplantation may be required and has been successfully performed for this condition. However, malignancy recurrence after transplantation has not been reported yet. methods AND RESULTS: We here report the case of an asymptomatic hepatitis B surface antigen carrier who developed a malignant lymphoma, which was treated by chemotherapy. After cessation of chemotherapy, he developed a fulminant hepatitis, requiring liver transplantation. Three years later, he developed a recurrent malignant lymphoma, which was treated successfully by autologous bone marrow transplantation. In order to prevent viral replication, lamivudine and intermittent administration of fresh-frozen plasma highly concentrated in anti-HBs immunoglobulin was initiated before the bone marrow transplantation. The patient remains well 12 and 56 months after autologous bone marrow and liver transplantation, respectively. CONCLUSIONS: This experience suggests that all hepatitis B surface antigen-positive patients for whom chemotherapy is indicated would benefit from prophylactic antiviral hepatitis b virus therapy. Furthermore, successful autologous bone marrow transplantation is possible after liver transplantation.- - - - - - - - - - ranking = 3.5keywords = liver (Clic here for more details about this article) |
5/622. Two separate episodes of hemophagocytic syndrome at a two-year interval in an apparently immunocompetent male.We describe two separate episodes of hemophagocytic syndrome (HPS) at an interval of two years in a seemingly immunocompetent male. This case suggests the possible existence of an inherent predisposition to HPS, in which otherwise negligible self-limited viral infection may trigger HPS. Laboratory data for a 16-year-old boy admitted with persistent high grade fever and severe thrombocytopenia disclosed coagulation abnormality, liver damage, and hypercytokinemia. A bone marrow aspiration revealed a proliferation of histiocytes with fresh hemophagocytosis. We diagnosed that he was suffering from HPS. Responding to steroid pulse therapy, he recovered completely and was discharged. After two years of healthy life, he became febrile again and was readmitted. The fever was refractory to antibiotics and was associated with a sudden drop in platelet count. Laboratory data and the bone marrow picture were consistent with those of HPS. He was again successfully treated with steroid. After the second episode, he has been healthy for more than two years.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
6/622. cholestasis and liver cell damage due to hypersensitivity to erythromycin stearate--recurrence following therapy with erythromycin succinate.erythromycin is a frequently used antibiotic in patients with atypical respiratory infection and/or an allergy to penicillin. We report the case of a young woman who developed severe cholestasis and jaundice following treatment with erythromycin stearate. Two years later her general practitioner prescribed erythromycin succinate for pharyngitis. She experienced a severe second episode of jaundice and malaise. Different esters of erythromycin have been introduced to reduce side effects such as allergic reactions to erythromycin. The findings in our patient underline the fact that hypersensitivity is caused by the erythromycin molecule, independent from the type of esterification. Because of these side effects newer makrolides should be given preference over erythromycin.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
7/622. sarcoidosis with selective involvement of a second liver allograft: report of a case and review of the literature.A case of sarcoidosis recurrent in a patient's second liver allograft is described. There was no granulomatous disease seen in the patient's first liver allograft. After the second orthotopic liver transplantation (OLT), the patient was successfully treated for acute rejection, aspergillus infection, and cytomegalovirus viremia. Approximately 2 months after the second OLT, the patient was treated with long-term interferon-alpha for recurrent hepatitis c. Five years after the operation, he experienced liver failure secondary to recurrent hepatitis and underwent a third OLT. This is only the second reported case of sarcoidosis recurrent in the liver parenchyma of a transplanted organ and the first in which interferon-alpha might have played a role.- - - - - - - - - - ranking = 4.5keywords = liver (Clic here for more details about this article) |
8/622. Treatment of upper abdominal malignancies with organ cluster procedures.Upper abdominal exenteration for upper abdominal malignancies was carried out in 15 patients with removal of the liver, spleen, pancreas, duodendum, all or part of the stomach, proximal jejunum and ascending and transverse colon. Organ replacement was with the liver, pancreas and duodenum plus, in some cases, a short segment of jejunum. Eleven of the 15 patients survived for more than 4 months; 2 died, after 61/2 and 10 months, of recurrent tumor. Of the 9 patients who are surviving after 61/2 to 14 months, recurrent tumor is suspected in only 1 and proven in none. Four patients with sarcomas and carcinoid tumors (2 each) have had no recurrences. The other 5 survivors had duct cell cancers (3 examples), a cholangiocarcinoma (1 example), and a hepatoma (1 example). The experience so far supports further cautious trials with this drastic cancer operation.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
9/622. Levels of soluble FasL and FasL gene expression during the development of graft-versus-host disease in DLT-treated patients.Three patients with different clinical symptoms of graft-versus-host disease (GVHD) who had received donor lymphocyte transfusion (DLT) for the treatment of relapsed leukaemia after an allogeneic bone marrow transplantation (BMT) from HLA-matched sibling donors were analysed for the presence of soluble FasL (sFasL) in the sera and for the expression of the Fas ligand (FasL) gene in the peripheral blood mononuclear cells (PBMNC). Two patients who demonstrated liver damage with increased levels of serum bilirubin showed significantly increased levels of serum sFasL. The increase in the sFasL level was observed prior to the increase in the bilirubin during the clinical courses of both patients. The high dose of methyl predonisolone administered to one of these patients greatly reduced the levels of sFasL in the serum. The bilirubin levels were also reduced thereafter. The third patient (without liver damage) did not show any increase in the serum sFasL level. The expression of the FasL gene in the PBMNC of these three patients was examined. All three patients showed increased levels of the FasL gene expression during their clinical courses. However, only one patient showed a parallel alteration of FasL gene expression with sFasL in the serum. These cases provide evidence that the Fas/FasL system is closely associated with human GVHD, especially in the development of liver GVHD.- - - - - - - - - - ranking = 1.5keywords = liver (Clic here for more details about this article) |
10/622. Enterovesical fistula complicating pregnancy. A case report.BACKGROUND: Enterovesical fistula is a rare cause of recurrent urinary tract infections. This condition is unusual in young people as common etiologies include diverticular disease and cancer. When an enterovesical fistula occurs in women of childbearing age, Crohn's disease is a likely cause. To our knowledge, enterovesical fistula complicating pregnancy has not been reported before. CASE: A pregnant woman with recurrent urinary tract infections was evaluated. cystoscopy was suggestive of an enterovesical fistula, which was confirmed by charcoaluria following oral charcoal administration. The prenatal course was complicated by two episodes of hemorrhagic cystitis despite antibiotic prophylaxis. The patient had an uncomplicated term spontaneous vaginal delivery. An upper gastrointestinal series performed postpartum was suggestive of Crohn's disease and confirmed an enterovesical fistula. Surgical repair was successfully performed three months following delivery, revealing Crohn's disease. CONCLUSION: Enterovesical fistula may be an unusual cause of recurrent urinary tract infections in pregnancy. In this case, enterovesical fistula was the presenting symptom of Crohn's disease.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
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