Biomedical subjects

Philippe Ichaï

Publications and source records attributed to Philippe Ichaï.

5 recordsLinked to original sources

PubMed2006

[Wilson's disease].

Wilson's disease is an autosomal recessive disorder of copper excess. This illness results from mutations of the ATP7B gene chromosome 13. The discovery of the gene allowed a better understanding of cytosolic copper trafficking its relationship with ceruloplasmin synthesis. Symptomatic patients may present with hepatic, neurologic or psychiatric forms. Clinical and phenotypic evidences provide only presumptive arguments for this disease which can be routinely assessed by molecular analysis. This disease can be efficiently treated by chelation and zinc therapy. Liver transplantation is the therapy to patients with hepatic fulminant course, or in those with relentless progression of hepatic dysfunction in spite of medical therapy.

Hepatolenticular Degeneration↗

PubMed2005

Cadaveric full-size liver transplantation and the graft alternatives in adults: a comparative study from a single centre.

BACKGROUND/AIMS: This study aims to compare the results of living donor (LDLT), cadaveric split (SLT) and domino (DO) liver transplantation which are currently available alternatives to the conventional cadaveric full-size liver transplantation (CAD). METHODS: Immunologic, vascular and biliary complications (BC) were evaluated in 38 LDLT, 20 SLT, 17 DO and 38 CAD recipients. RESULTS: The incidence of acute rejection (AR) was similar between groups, and between blood-related and blood-unrelated patients. AR was more severe in the SLT group according to the Banff scores (P=0.03, P<0.001, P<0.001). The evolution of AR was similar between the groups, in terms of development of chronic rejection. No venous complications occurred in the overall population. The rate of arterial thrombosis (10.5, 10, 0 and 3%, respectively) was statistically similar between groups. The rate of BC (26, 40, 12, and 8%, respectively) was higher in the partial grafts than in the whole grafts (P=0.006), but was not significantly different within each group. There was an association between the severity of preservation injury and occurrence of AR (P=0.01) and arterial thrombosis (P=0.016), but not BC. One- and 2-year graft and patient survival rates were similar between groups. CONCLUSIONS: None of the graft types seemed to confer immunological advantage. BC remained problematic in the partial grafts, independently from ischemia time.

Adult↗

PubMed2005

Hepatitis C virus viral recurrence and liver mitochondrial damage after liver transplantation in HIV-HCV co-infected patients.

BACKGROUND/AIMS: As life expectancy in HIV-HCV co-infected patients improves, end stage liver disease requiring liver transplantation (LT) may become an emerging problem. We report the Paul Brousse Hospital experience of transplantation for end stage cirrhosis in HIV-HCV co-infected patients. METHODS: Seven consecutive HIV-HCV co-infected patients were transplanted between December 1999 and December 2002 for end stage liver disease due to HCV. All patients were treated by highly active antiretroviral therapy (HAART), HIV plasma viral load was <400 copies/ml and median CD4 lymphocyte count was 306 cells/mm3 (range, 103-510) before LT. At the time of evaluation (March 2003), the median follow-up was 21 months (range, 4-40). RESULTS: Two patients died, 4 and 22 months, respectively after LT. At the last biopsy, METAVIR score was staged F4 in two patients, F3 in two, and F1 in one. Microvesicular steatosis was noted in nearly all patients. The ratio of mitochondrial to nuclear DNA was low in three of four patients examined as compared with the amount of liver mtDNA found in eight HIV-negative, HCV-infected controls (P=0.01). CONCLUSIONS: A significant defect in the activity of the respiratory chain complex IV was noted in all five patients studied. Mitochondrial hepatotoxicity and severe HCV recurrence occur in HIV-HCV co-infected patients after LT.

Adult↗

PubMed2003

Liver transplantation for hepatopulmonary syndrome: a ten-year experience in Paris, France.

BACKGROUND: Although the possibility of reversing hepatopulmonary syndrome (HPS) after liver transplantation is now well established, the proportion of patients in whom reversibility is observed and the time to resolution of HPS remain uncertain. METHODS: We analyzed the outcome of all adult patients with HPS who underwent orthotopic liver transplantation in all the liver transplant centers in Paris, during a 10-year period. RESULTS: Twenty-three adult patients (median age, 47 years; range, 14-64) underwent transplantation in four institutions. Median PaO(2) was 52 (range, 32-67) mm Hg and median alveolar-arterial oxygen gradient was 66 mm Hg. When patients were breathing 100% O(2), median PaO(2) was 310 (range, 74-663) mm Hg. Median isotopic shunt ratio was 33% (range, 0-80%). The overall mortality during the study period was 30.5% (7/23). Perioperative mortality was 8.5%, whereas late mortality was 22%. None of the preoperative characteristics of HPS (isotopic shunt ratio, PaO(2) on room air or on 100% oxygen) was associated with overall postoperative mortality. Of the 21 patients surviving the perioperative period (median follow-up, 17 months; range, 0.5-72), a decrease in alveolar-arterial oxygen gradient of at least 5 mm Hg and at least 10 mm Hg was observed in 21 of 21 and in 18 of 21 patients, respectively, with great variations in the time of improvement. The threshold of 70 mm Hg was reached in 15 patients. The lower the preoperative PaO(2), the longer the time to reach this point. CONCLUSION: Our data strongly support the role of orthotopic liver transplantation in adult patients with HPS, regardless of its severity.

Adolescent↗

PubMed2002

Terlipressin in patients with cirrhosis and type 1 hepatorenal syndrome: a retrospective multicenter study.

BACKGROUND & AIMS: Type 1 hepatorenal syndrome (HRS) is a severe complication of cirrhosis associated with a short median survival time (<2 weeks). Although the administration of terlipressin improves renal function, its effect on survival is unknown. This study investigated predictive factors of survival in patients with type 1 HRS treated with terlipressin. METHODS: Ninety-nine patients with type 1 HRS treated with terlipressin in 24 centers were retrospectively studied. Terlipressin-induced improved renal function was defined as a decrease in serum creatinine value to <130 micromol/L or a decrease of at least 20% at the end of treatment. RESULTS: At inclusion, the Child-Pugh score was 11.8 +/- 1.6 (mean +/- SD). Terlipressin (3.2 +/- 1.3 mg/day) was administered for 11 +/- 12 days. Renal function improved in 58% of patients (serum creatinine decreased by 46% +/- 17% from 272 +/- 114 micromol/L). Median survival time was 21 days. Survival rate was 40% at 1 month. Multivariate analysis showed that improved renal function and Child-Pugh score < or =11 at inclusion were independent predictive factors of survival (P < 0.0001 and 0.02, respectively). Thirteen patients underwent liver transplantation (92 +/- 95 days after HRS onset), 10 of whom had received terlipressin and had had improved renal function. CONCLUSIONS: This retrospective uncontrolled study shows that in patients with type 1 HRS, terlipressin-induced improved renal function is associated with an increase in survival. Thus, a randomized trial investigating the effect of terlipressin on survival in patients with type 1 HRS should be performed.

Aged↗