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Biomedical subjects

J Valayer

Publications and source records attributed to J Valayer.

At least 37 records · Page 2Linked to original sources
PubMed1994

Liver transplantation for end-stage liver disease associated with alpha-1-antitrypsin deficiency in children: pretransplant natural history, timing and results of transplantation.

Alpha-1-antitrypsin deficiency is an inborn metabolism error which can cause emphysema and liver disease. As regards the pathophysiology of liver disease, this deficiency is poorly understood, and it is also not known why only a small proportion of Pi ZZ individuals progress towards cirrhosis and liver failure. Since there is no specific therapy for end-stage liver disease associated with alpha-1-antitrypsin deficiency, patients are considered candidates for liver transplantation. In this paper, the natural history of 16 children who underwent liver transplantation is reviewed. Fourteen patients had neonatal cholestasis as a first symptom of the disease and hepatosplenomegaly was present in all children by the age of 12 months. In 11 children, jaundice recurred, always with liver function deterioration. Two patients had a histological paucity of interlobular bile ducts and required early transplantation due to rapid progression of liver failure. At the time of pretransplant assessment, all the patients in this study had portal hypertension and seven of them had experienced at least one episode of gastrointestinal bleeding. One child had moderate intrapulmonary shunts with hypoxemia, but the others had normal spirometry and blood gases. There was no other extrahepatic complication of alpha-1-antitrypsin deficiency. Eighteen orthotopic liver transplantations were performed in 16 patients. One patient died 8 days after retransplantation due to graft necrosis. Fifteen patients (94%) were alive after a median follow-up of 22 months with an excellent quality of life, normal serum alpha-1-antitrypsin levels and without evidence of liver disease recurrence or pulmonary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1994

[The role of brachytherapy in the therapeutic strategy of vesico-prostatic rhabdomyosarcoma in children].

Because of its ballistic and physical characteristics, brachytherapy (BT) represents the optimal irradiation treatment in pediatric tumors, especially in prostate or bladder rhabdomyosarcoma. In a limited series of 15 children treated with BT (including three patients treated with salvage BT), 8 out of 9 patients surviving with NED benefited from conservative treatment among 12 patients treated with BT as first-line treatment. Sequelae were minimal consisting of one grade I cystitis and one asymptomatic vesical and ureteral reflux. BT allows administration of high doses to limited volumes sparing normal tissues. BT--whenever possible--represents the treatment of choice compared with external radiotherapy. Indications of BT depend on tumor accessibility and size (usually < or = 4 cm).

Adolescent↗
PubMed1994

[Vascular complications of liver transplantation: surgical treatment].

Vascular complications constitute a major cause of morbidity and mortality after liver transplantation. They are dominated by arterial complications, the most frequent being hepatic artery thrombosis. Venous complications essentially consist of portal vein thrombosis. The preventive treatment of vascular complications is based on a better understanding of the risk factors. Close cooperation between surgeon, and radiologist is essential for effective surgical correction, which requires a rapid diagnosis and is designed to save not only the patient's life, but also, whenever possible, the liver transplant.

Aneurysm, False↗
PubMed1993

[Transplantation of reduced-size livers in children].

Nowadays, liver reduction techniques make it possible to use livers obtained from adults or adolescents for implantation in children. These techniques have been evaluated by analysis of 100 liver transplantations performed between January 1988 and October 1991 in 85 children. Forty-six full-size grafts implanted in 38 children (group 1) were compared with 54 reduced-size grafts implanted in 47 children (group 2). The overall actuarial survival at 4 years was 86 percent. There was no statistical significant difference between the two groups as regards the rates of death (8 versus 19 percent), reoperation (54 versus 64 percent), retransplantation (15 versus 16 percent), hepatic artery thrombosis (13 versus 15 percent) and graft survival (82 versus 70 percent) respectively. Haemorrhage was significantly more frequent in group 1 than in group 2 (P = 0.04), irrespective of whether transplantation was performed urgently or electively. Using reduced-size livers considerably increases the number of liver grafts available to children. Apart from a greater risk of haemorrhage, the results obtained with reduced-size livers were identical with those obtained with full-size livers.

Adolescent↗
PubMed1993

Pediatric liver transplantation for primary hepatocellular carcinoma associated with hepatitis virus infection.

We report two cases of early primary hepatocellular carcinoma (PHC) in children, after probable maternal transmission of hepatitis B, that were treated with orthotopic liver transplantation (OLT). Both children were 8.5 years old and had elevated levels of serum alpha-feto-protein. The diagnosis of PHC was made at 8 years and confirmed histologically. Serum hepatitis B surface antigen (HBs Ag) was detected in the mothers and suggested vertical transmission. An attempt at complete liver tumor resection failed, leading to OLT. In order to prevent recurrence of the hepatitis B virus (HBV) infection, hepatitis B immunoprophylaxis was used. Two years after OLT, one child presented with recurrent HBV infection. No tumor recurrence was observed at follow-up in either of the patients. From these two cases we conclude that (1) HBV infection may play an important causal role in PHC in children, with an even shorter incubation period than that in adults; (2) close follow-up is needed for children who are HBs Ag-positive carriers; and (3) liver transplantation should be proposed early after the diagnosis of PHC, when tumor resection is not feasible.

Carcinoma, Hepatocellular↗
PubMed1993

[Surgery of liver transplantation in children].

From 1988 until 1992, 94 children with end-stage liver disease were put forward for orthotopic liver transplantation (OLT) by the surgical and anesthesic teams of the Bicetre Hospital. Due to a majority of adult donors, and to the young age of the recipients (mean = 3 years 3 months), a reduced-size graft was prepared in 64 out of the 111 transplants performed (58%). A number of children (42) had to be reoperated on due to complications: hepatic artery thrombosis, one of the most severe complications following pediatric OLT, occurred in 14 cases, and was an indication for seven out of the 17 retransplantations in this series. The eight deaths which occurred in the early postoperative period were the toll of this particularly challenging surgery.

Adolescent↗
PubMed1992

Emergency liver transplantation for fulminant liver failure in infants and children.

We report our results with orthotopic liver transplantation in children with fulminant liver failure. Thirty-five children with fulminant liver failure were evaluated for liver transplantation. The main causes of liver failure were viral hepatitis (54.2%), drug-induced liver injury (14.2%) and Wilson's disease (11.4%). Children were considered as candidates for liver transplantation only if hepatic encephalopathy was associated with a decrease in the level of factor V to below 25%. Seven children (20%) did not meet this criterion and recovered spontaneously. Six children (17.1%) had contraindications for liver transplantation and died. In three of these six children, contraindications included irreversible brain damage at the time of admission. Twenty-two children (62.8%) met the criteria for liver transplantation and were placed on the emergency transplant list. Three of them died awaiting grafts. Nineteen children underwent liver transplantation; 13 of them (68.4%) are alive without sequelae, after 6 mo to 4 yr of follow-up, at this writing. Four of the children who died after surgery had severe encephalopathy on admission that did not improve after liver transplantation. In conclusion, emergency liver transplantation appears to be an effective treatment for children with fulminant liver failure. Nevertheless, irreversible brain damage developed in 10 patients, and they died before or after surgery. We postulate that many of these deaths could have been avoided if children had been transferred to a liver transplantation facility and had undergone transplantation earlier. We emphasize that children with acute liver failure should be transferred to a center that performs liver transplantation before the development of hepatic encephalopathy.

Adolescent↗
PubMed1990

Long-term outcome after surgery for biliary atresia. Study of 40 patients surviving for more than 10 years.

To define long-term prognosis of children who underwent surgery for biliary atresia, a retrospective study was undertaken in 122 children who underwent one of the Kasaï procedures between 1968 and 1977. Forty of the 122 children (32.7%) were alive after 10 years. Firm hepatomegaly was present in 31 and splenomegaly in 29 children. Serum bilirubin or all liver function tests were normal in 21 and 11 children, respectively; survival rate decreased with the age at operation, but no significant difference was observed in the rate of children surviving with normal serum bilirubin whether they underwent surgery before age 2 months or between 2 and 3 months. Twenty-four had esophageal varices and 15 experienced gastrointestinal bleeding. Normal liver-function tests and absence of portal hypertension were observed in 11 of 122 children. These results indicate that Kasaï's procedures were helpful in a significant proportion of children with biliary atresia who underwent surgery during this period. However, 80% of children who initially underwent surgery with Kasaï's procedures should eventually undergo liver transplantation.

Adolescent↗
PubMed1990

Orthotopic liver transplantation for Byler disease.

Byler disease is a rare form of familial intrahepatic cholestasis that is fatal before puberty. This retrospective study reviewed the results of orthotopic liver transplantation in 14 children with Byler disease using 12 whole-liver grafts and 2 reduced-size grafts. One post-operative death occurred after retransplantation for arterial thrombosis. In the other patients, infectious problems and rejection episodes were the most frequent complications during the postoperative period. In the 13 patients alive, graft function, growth, and quality of life were good after an average follow-up of 17 months without any sign of disease recurrence.

Adolescent↗