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Biomedical subjects

J Valayer

Publications and source records attributed to J Valayer.

At least 19 recordsLinked to original sources
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↗
PubMed1990

[Liver transplantation in children. New experience in a Pediatric Surgery unit. Evaluation of the first 18 months' experience].

The authors report an eighteen month experience with orthotopic liver transplantation (OLT) in children in the Pediatric Department of Hospital Bicêtre in Paris, from January 1988 until July 1989. Thirty-eight OLTs including 4 retransplant operations, were performed in 34 children, aged from 7 months to 13 years, 20 of them under the age of 3 years. Biliary atresia was the indication for 22 children. Twenty-eight donors were children. A reduced-size liver was used in 17 cases. The technique for OLT was based on the description by TE Starzl. Surgical complications led to reoperation in 15 cases, mostly in relation to hepatic artery thrombosis (HAT), which occurred in 7 cases: small donor liver was considered to be one of the causative factors. In all but one case of HAT a retransplant was considered; to date it is has been performed in 3 of these children. Thirty-one children have survived, with a mean follow-up of 8 months, all are at home, except for one child at the time of this report. Liver function tests are normal for 22, and moderately altered for 5. For the four remaining children, retransplant is planned for 3 cases and one child has just been retransplanted. The authors emphasise the fact that OLT in small children requires a specialised pediatric environment, particularly as regards intensive care and nursing.

Adolescent↗
PubMed1989

[Rapid liver perfusion in multiple organ procurement in children. For liver transplantation].

We report our experience of the rapid liver flush technique in multiple organ harvesting from donor children. Between January 1988 and May 1989, out of 33 liver transplantations in 30 children, 24 were performed using livers obtained from children. The donors' mean age was 75 months. In every case several organs were removed (24 kidneys, 9 heart-lung blocks, 9 hearts, 1 pancreas). The mean duration of the procedure was 153 minutes. Supernumerary hepatic vessels (6 left and 2 right hepatic arteries) were left intact. Twenty-two hepatic grafts were classified as functional. Two grafts classified as non-functional were responsible for 2 deaths in this series. The rapid liver flush technique seems to be satisfactory in donor children when the following characteristics are taken into account: tendency to haemodynamic instability, small caliber of the vessels and fragility of tissues.

Adolescent↗
PubMed1989

H-type shunt with an autologous venous graft for treatment of portal hypertension in children.

From 1981 to 1987, 86 children aged 16 months to 16 years underwent a portosystemic shunt procedure using an autologous venous graft (internal jugular vein in 80 cases). Fifty-five mesocaval, 20 splenorenal, 4 portacaval, and 7 makeshift shunts were constructed. The indication for shunting was an extrahepatic portal obstruction in 59 cases, intrahepatic portal obstruction in 23 cases (including 6 cases of congenital hepatic fibrosis), and Budd-Chiari syndrome in 4 cases. One patient of the latter group died early from intractable ascites with a nonfunctioning shunt, and a second child died 2 months after operation from unknown reasons with a patent shunt. With a follow-up over 1 year for 58 of the 84 survivors, 78 successes and 6 failures were recorded according to the clinical outcome and the findings of ultrasonic and endoscopic examinations. Three of the six children with a failed shunt have been submitted to a second successful H-type shunt operation. No case of encephalopathy was recorded in this series. Thus, with an approximate success rate of 95%, the H-type shunt with a venous graft should be recommended for treatment of portal hypertension of extrahepatic origin, especially in young children.

Adolescent↗
PubMed1988

Budd-Chiari syndrome in children: report of 22 cases.

Clinical, radiologic, and histologic features in 22 children with Budd-Chiari syndrome are reported. Three children had acute refractory ascites; all the others had hepatomegaly, which was detected either fortuitously or because of abdominal pain or distention. Results of liver function tests were normal or only moderately abnormal. In most cases a combination of ultrasonography and needle liver biopsy pointed to the diagnosis of Budd-Chiari syndrome, which was confirmed by angiography. Eighteen children underwent surgery involving various techniques, depending on the degree of patency of the inferior vena cava. Five children died postoperatively. Histologic studies of the liver, carried out in 12 of the surviving children, showed disappearance or regression of centrilobular hemorrhagic infiltration. Half of the surviving surgical patients are now free of complications after a follow-up of 7 months to 7 years; the others have either secondary thrombosis of the inferior vena cava or stenosis of the shunt or have experienced late pulmonary complications. Our results suggest that (1) Budd-Chiari syndrome must be considered a possible diagnosis in children with firm hepatomegaly and normal or near normal liver function, (2) surgery provides good results in many instances, and (3) the possibility of late complications requires careful follow-up.

Budd-Chiari Syndrome↗
PubMed1988

[Surgery of hepatoblastoma and hepatocarcinoma in children in the era of preoperative chemotherapy. Current progress and limitations].

Twenty seven children aged 1 to 15 years were admitted from 1981 to 1987 for the investigation and/or the surgical treatment of a primary liver malignancy (25 hepatoblastomas and 2 hepatocellular carcinomas). All were submitted to preoperative multidrug chemotherapy. One child died from circulatory failure early after the diagnosis and two children considered to be non operable died also. A macroscopically satisfactory resection could be achieved in 24/27 cases. Sixteen out of these 24 children are alive and disease free, 11 of them with a follow-up over 2 years. Two of these 16 survivors underwent a second operation for resection of a local recurrence. Four children died after a massive unresectable hepatic relapse, two after a second relapse and a one after the onset of pulmonary metastases during her chemotherapeutic postoperative course. One further child is at yet in a critical situation because of a late small secondary hepatic relapse. Two factors contributed to improvement of survival an cure rates during the past ten years: 1. Routine preoperative chemotherapy, which may help in controlling the disease and greatly facilitates surgery by shrinking of massive tumors. 2. Better surgical management and especially the total hepatic vascular exclusion, used in 13 cases of this series. Three situations still have a poor outlook: 1. Hepatocellular carcinoma 2. Hepatoblastoma with a low response to chemotherapy 3. Multifocal tumors and those involving the main hepatic vessels. The feasibility of a liver transplantation in some selected cases is discussed.

Adolescent↗
PubMed1988

[Practical aspects of surveillance after hepatic transplantation in children].

From a practical point of view, the care of a child after liver transplantation comprises 3 periods with decreasing severity: -the first one takes place in the intensive care unit: immunosuppression is started with steroids, azathioprine and cyclosporine and four major complications are sought for and treated: primary graft dysfunction, acute rejection, hepatic artery thrombosis and infection; -the second period takes place in conventional hospital settings: cyclosporine is progressively switched from IV to oral; rejection, cytomegalovirus infection and biliary complications are sought for and treated; -the third period takes place at home: the level of immunosuppression is progressively lowered under biochemical supervision; long-term complications (renal and malignancies) are detected.

Bacterial Infections↗
PubMed1988

[Budd-Chiari syndrome with thrombosis of the inferior vena cava. Treatment by mesenterico-innominate shunt].

We report two cases of patients, 12 and 28 years old, suffering from a Budd-Chiari syndrome with a thrombosed inferior vena cava, who were treated by a shunt interposed between the superior mesenteric vein and the left innominate vein. The aim of this shunt was to avoid pericardiotomy in these patients with peroperative ascites, in order to reduce the risk of pericarditis after surgery. A reinforced polytetrafluoroethylene prosthesis (Gore-Tex) was used, associated with an interposition jugular vein graft in the 12-year old child, alone in the other case. The left innominate vein was isolated through a midline sternotomy, and the prosthesis was placed in a retrosternal position. Operation and postoperative course were uneventful. All signs of ascites disappeared. The patients were free of symptoms, 8 and 4 months after surgery respectively. Shunt patency was documented by angiography and magnetic nuclear resonance.

Adult↗
PubMed1988

[Lower intestinal obstruction caused by a lactobezoar].

Two case of the so-called "milk curd obstruction" syndrome are reported. Although seldom found elsewhere than in the stomach, more distal obstruction in the ileum or the transverse colon by milk curd aggregates may mimic more classical causes of obstruction if one is not aware of the possibility of such incident related to feeding. Appropriate interviewing of the mother about regimen and medications will be of great help, in investigating the cause of the abdominal disorder, obviating the need for surgical intervention if confirmed by radiopaque enema.

Bezoars↗