Liver Transplantation in Patients With Niemann-Pick Disease--Single-Center Experience.

Niemann-Pick disease (NPD) is a rare syndrome caused by abnormal intracellular sphingomyelin lipid storage in cells known as "Pick cells." NPD can start in childhood or develop insidiously, with a predilection for reticuloendothelial cells and the nervous system. NPD is a heterogeneous clinical, and biomolecular disorder which has 6 variants. There is no curable treatment for NPD. Generally, the treatment for all types of Niemann-Pick disease is to support. Type B NPD (NPD-B) is mostly characterized by hepatosplenomegaly, which can evolve to hepatic cirrhosis. In patients who progress to liver failure, liver transplantation may be improve liver function. The Transplant Service of Hospital Universitário Walter Cantídio performed its first liver transplants in patients with NPD-B with good results, demonstrating the efficacy of this procedure in selected cases.

Endemic and opportunistic infections in Brazilian solid organ transplant recipients.

OBJECTIVE: To evaluate the frequency and clinical features of endemic and other opportunistic infections in liver or kidney transplant recipients in four transplant centres in different geographical areas of Brazil. METHODS: Retrospective analysis of medical and laboratory records of four transplant centres on endemic and other opportunistic infections in liver or kidney transplant recipients. Analyses were performed with spss statistical software. RESULTS: From 2001 to 2006, 1046 kidney and 708 liver transplants were registered in all centres. The average age was 42 years. Among 82 (4.7%) cases with infections, the most frequent was tuberculosis (2.0%), followed by systemic protozoal infections (0.7%), toxoplasmosis (0.4%) and visceral leishmaniasis (0.3%). Systemic fungal infections occurred in 0.6%, of which 0.4% were cryptococcosis and 0.2% were histoplasmosis. Dengue was the only systemic viral infection and was registered in two cases (0.1%), of which one was classified as the classic form and the other as dengue haemorrhagic fever. Nocardiosis was described in one case (0.05%). The infectious agents most frequently associated with diarrhoea were Blastocystis sp., Schistosoma mansoni and Strongyloides stercoralis. CONCLUSIONS: Opportunistic Infections in transplant patients have a wide spectrum and may vary from asymptomatic to severe infections with high mortality. A better understanding of the epidemiology of endemic pathogens and clinical manifestations can contribute to the establishment of an early diagnosis as well as correct treatment aimed at decreasing morbidity and mortality.

Orthotopic liver transplantation for hepatocellular carcinoma: one center's experience in the Northeast of Brazil.

Hepatocellular carcinoma (HCC) is the fifth most common cancer in the world and the third leading cause of cancer-related death. In this study, we sought to assess the outcome of patients with HCC who underwent orthotopic liver transplantation (OLT) in a center in the northeast of Brazil. Between May 2002 and July 2008, 294 OLTs were performed at our center. In 45 patients, HCC was confirmed by histological examination of the explant. Patients were predominantly men of ages ranging from 14-67 years. Hepatitis C virus was involved in 55.4% of the cases. Alpha fetoprotein (AFP) levels were normal in 65.2% of the patients and surpassed 100 ng/mL in only 10.4%. The median waiting time on the list was 10 months. Seventeen patients (37.7%) presented a solitary nodule, 19 (42.2%) had 2 or 3 nodules, and 9 patients (20%) had more than 3 nodules. The maximal diameter of the largest tumor was <3 cm in 26 patients (57.7%) and exceeded 5 cm in 6 patients (13.3%). Ten tumors were well differentiated, 32 were moderately differentiated, and 3 were poorly differentiated. Eleven tumors showed microvascular invasion. There have been 4 tumor recurrences. There was an association between microvascular invasion and tumor recurrence with a statistically significant relative risk. In conclusion, OLT is an excellent option for patients with HCC. The recurrence rate was low (<10%). However, we believe that more prospective studies are needed about OLT beyond the Milan criteria because our study suggested that microvascular invasion may be more important than tumor size or number.

A new biliodigestive anastomosis technique after hepatic artery thrombosis as bridge to liver retransplantation: a case presentation.

Arterial complications after liver transplantation are frequent. Hepatic artery thrombosis (HAT) is usually associated with biliary complications. Herein we have reported a case of a patient who was admitted for jaundice, itch, and elevated aspartate aminotransferase and alanine aminotransferase levels at 6 weeks after liver transplantation. HAT associated with a biloma was diagnosed and an urgent operation performed requiring a new biliodigestive anastomosis technique. Fourteen months after the first transplant, the patient was retransplanted. The operation performed may be an alternative to treat biliary complications due to late HAT.

Evaluation of hepatic function after orthotopic liver transplantation: a comparative study using Belzer and Collins solutions.

Belzer and Collins are solutions used in liver transplantation. The purpose of this study was to compare liver function after utilization of two different schemes of graft preservation using both solutions. Between December 2004 and September 2005, 43 liver transplantations were performed. Twenty-three of these used 2 L of Collins solution and 2 L of Belzer solution as the preservation solution. The others used three L of Collins and 1 L of Belzer solution as the preservation solution. The cold ischemia time of both groups was similar. We analyzed liver function using the serum ALT, AST, bilirubin and international normalized ratio. On the first day after the transplantation, the level of international normalized ratio of the group of patients that used 1 L of Belzer was significantly higher than the other group (P<.05). The levels of ALT, AST, and bilirubin were not different on day 7 after transplantation. We concluded that using only 1 L of Belzer solution is as efficient with the advantage that it is less expensive.

Pro-inflammatory effects of cholera toxin: role of tumor necrosis factor alpha.

Cholera toxin has been traditionally described as the one that does not induce inflammation. It has, however, potent adjuvant and immuno-modulatory activities. Since the adjuvanticity of other compounds is linked to their capacity to induce inflammation, in the present study the pro-inflammatory activity of cholera toxin was investigated. We studied this activity in the following rat models of inflammation: paw edema and neutrophil migration into the peritoneal cavity, and evaluated cholera toxin's effect on tumor necrosis factor alpha (TNF-alpha) production by mouse macrophages. We, also, explored the effects of dexamethasone (DEXA) and of two inhibitors of TNF-alpha production, thalidomide (TAL) and pentoxifylline, on paw swelling. Cholera toxin-induced significant and dose-dependent paw edema, which peaked 48 h after toxin challenge (Cholera toxin(2.5 microg): 2.39 +/- 0.22 ml). Cholera toxin B subunit did not show edematogenic activity. DEXA, TAL and pentoxifylline significantly reduced cholera toxin-induced edema (DEXA(0.5 mg/kg): 42.6% of inhibition; TAL(45 mg/kg): 36% of inhibition; pentoxifylline (45 mg/kg): 61% of inhibition). Neither cholera toxin nor its B subunit induced neutrophil migration into peritoneal cavities. Cholera toxin stimulated the release of TNF-alpha by macrophages (cholera toxin(10 microg): 11.46 +/- 0.44 UI/ml). These data provide evidences that cholera toxin exhibits significant pro-inflammatory activity. It also indicates the role of TNF-alpha upon the pathophysiology of this event based on the inhibitory action of DEXA, TAL and pentoxifylline, and on TNF-alpha secretion induced by cholera toxin.