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Biomedical subjects

A Lachaux

Publications and source records attributed to A Lachaux.

At least 37 records · Page 2Linked to original sources
PubMed2000

[Liver transplantation in infants and children. Evaluation of the first 40 cases (March 1991-March 1997)].

BACKGROUND: Liver transplantation (LT) is the treatment of end-stage liver disease in children. We report our experience with LT using grafts from living related (LRD) and cadaver donors (CD). POPULATION: From March 1991 to March 1997, 40 children and infants received a total of 42 liver grafts. A reduced-size liver was used in 28 cases. We studied pre-transplantation status, survival rate, and medical and surgical complications in these patients. RESULTS: The survival rate in our series was respectively 85 and 80% at 1 and 7 years after LT. Low weight infants required a prolonged ventilatory assistance. Five of the six deaths noticed during the first three months after LT occurred in children weighing less than 12 kg. One year after LT, no significant difference in the incidence of rejection was found, neither between low-weight children and the others, nor between patients transplanted from CD or LRD. Biliary tract stricture was the major surgical complication. CONCLUSION: This series consisted of a majority of low-weight children. The survival rate in the patients weighting less than 12 kg is lower than in the others. This may be explained by the nutritional status of these patients and early postsurgical complications. The use of grafts from living donors offers more flexibility since the operation is performed electively, but it did not seem to modify the incidence of acute rejections and surgical complications.

Adolescent↗
PubMed2000

Statistical models for predicting response to interferon-alpha and spontaneous seroconversion in children with chronic hepatitis B.

To develop prognostic models for identifying children with hepatitis B who are likely to respond to interferon-alpha (IFN-alpha) or to spontaneously seroconvert, we evaluated results of a multinational controlled trial comprising 70 children with chronic hepatitis B who received IFN-alpha and 74 children who did not receive therapy. Prognostic models were developed using SMILES (similarity of least squares), which is a data analysis network that incorporates multidimensional relationships in the clinical data of complex diseases. Commonly collected clinical data included age, gender, serum aminotransferase (aspartate aminotransferase [AST] and alanine aminotransferase [ALT]) and hepatitis B virus (HBV) DNA levels, and IFN-alpha dose. Additional data included pretreatment directional information (e.g. increases or decreases in serum aminotransferase and HBV DNA levels), liver biopsy results, race and transmission mode. Using data available prior to initiation of treatment, the SMILES models achieved prospective predictions of 89% for responders, 96% for non-responders, 100% for seroconverters and 93% for non-seroconverters. Although not predictive by themselves, the variables that had the greatest impact on predictions for IFN-alpha response were HBV DNA pretreatment direction, baseline HBV DNA, IFN-alpha dose and gender. The variables that had the greatest impact on predictions for spontaneous seroconversion were ALT pretreatment direction, baseline HBV DNA level, age and AST pretreatment direction. Therefore, these models may be useful in determining, in children with hepatitis B, the likelihood of response to IFN-alpha and spontaneous seroconversion.

Adolescent↗
PubMed2000

Effects of liver transplantation on long-chain polyunsaturated fatty acid status in infants with biliary atresia.

BACKGROUND: The long-chain polyunsaturated fatty acid (LC-PUFA) status of infants with untreated biliary atresia (BA) is known to be poor and is correlated to the severity of the liver disease. Liver transplantation (LT) markedly increases survival of patients with BA but the extent to which this reverses poor LC-PUFA status is not known. METHODS: To explore this question, the erythrocyte (red blood cell, RBC) phospholipid content of eight infants with BA who underwent LT was determined 2 months after an initial portoenterostomy, immediately before LT, and 6 and 12 months after LT. Before LT, all infants were fed a protein hydrolysate formula containing medium-chain triglycerides and essential fatty acids. Afterward, they were fed a normal diet for age. The RBC phospholipid content at each time point was compared with that of 28 age-matched control infants. RESULTS: Just before LT, median RBC phospholipid content of C20:4n-6, C20:5n-3, and C22:6n-3 was 25%, 48%, and 30% lower, respectively, than that observed in age-matched control infants. After LT, the RBC phospholipid content of most fatty acids reached normal values by 6 months. However, that of C20:4n-6 and C22:6n-3 contents remained 5% and 15% lower, respectively, than in normal control infants. Twelve months after LT, C20:4n-6 content remained lower than in normal children, but that of C22:6n-3 did not differ. The ratio of C20:3n-6/C20:4n-6, a reflection of delta-5 desaturase activity, was abnormal compared with normal children before LT (0.17 vs. 0.10, P < 0.009) but normalized by 6 months after LT (0.11 vs. 0.10, not significant). CONCLUSIONS: These data show that the abnormal LC-PUFA status of children with BA improves after LT but is not entirely reversed within a year after surgery. They suggest that the abnormal status before LT may be secondary, in part, to low delta-5 desaturase activity. The extent to which a different pre- and/or post-LT diet can prevent PUFA deficiency and/or hasten recovery of PUFA status remains to be determined.

Biliary Atresia↗
PubMed1999

Liver transplantation for glycogen storage disease types I, III, and IV.

UNLABELLED: Glycogen storage disease (GSD) types I, III, and IV can be associated with severe liver disease. The possible development of hepatocellular carcinoma and/or hepatic failure make these GSDs potential candidates for liver transplantation. Early diagnosis and initiation of effective dietary therapy have dramatically improved the outcome of GSD type I by reducing the incidence of liver adenoma and renal insufficiency. Nine type I and 3 type III patients have received liver transplants because of poor metabolic control, multiple liver adenomas, or progressive liver failure. Metabolic abnormalities were corrected in all GSD type I and type III patients, while catch-up growth was reported only in two patients. Whether liver transplantation results in reversal and/or prevention of renal disease remains unclear. Neutropenia persisted in both GSDIb patients post liver transplantation necessitating continuous granulocyte colony stimulating factor treatment. Thirteen GSD type IV patients were liver transplanted because of progressive liver cirrhosis and failure. All but one patient have not had neuromuscular or cardiac complications during follow-up periods for as long as 13 years. Four have died within a week and 5 years after transplantation. Caution should be taken in selecting GSD type IV candidates for liver transplantation because of the variable phenotype, which may include life-limiting extrahepatic manifestations. It remains to be evaluated, whether a genotype-phenotype correlation exists for GSD type IV, which may aid in the decision making. CONCLUSION: Liver transplantation should be considered for patients with glycogen storage disease who have developed liver malignancy or hepatic failure, and for type IV patients with the classical and progressive hepatic form.

Adolescent↗
PubMed1999

Depletion of mitochondrial DNA associated with infantile cholestasis and progressive liver fibrosis.

Few cases of infantile liver disease associated with mitochondrial DNA (mtDNA) depletion have been reported. Most of the patients died before 1 year of age of severe liver failure. We describe a new case, a 28-month-old child, presenting with cholestasis at age 2 months, complicated by progressive portal and lobular liver fibrosis. Growth and psychomotor development are undisturbed. There is no clinical evidence of either myopathy or neurological involvement. Metabolic investigation in plasma revealed an abnormal oxido-reduction status after fasting and after carbohydrate-rich meals. Light microscopy performed on liver biopsies revealed steatosis, abnormal hepatocytes with an "oncocytic" appearance and extensive fibrosis. Electron microscopic investigation showed an increased number of mitochondria with rare or enlarged cristae. Biochemical studies of liver biopsies showed that the respiratory chain activities containing mtDNA-encoded subunits were severely decreased (complexes I, III and IV). In contrast, the complex II activity was normal and the citrate synthase activity was greatly increased. Southern blotting analysis revealed that the ratio of mtDNA to nuclear DNA in liver was only 15% and 20% of the mean control value at ages 12 and 21 months, respectively. For this mtDNA depletion syndrome which is clinically expressed in the liver, a liver transplantation is discussed.

Child, Preschool↗
PubMed1999

Isolated deficient alpha6beta4 integrin expression in the gut associated with intractable diarrhea.

BACKGROUND: An infant born with pyloric atresia had development of intractable diarrhea and was found to have total epithelial detachment of gastric and small and large bowel mucosa. She had no skin abnormalities. Parental consanguinity and pyloric atresia in a sibling who died without autopsy suggest an inherited origin for this disorder. The purpose of this study was to examine defects in intestinal and skin cell adhesion. METHODS: Histologic, immunohistochemical, and ultrastructural characteristics of the skin and gut of the patient were compared with that of normal control subjects. Distribution of adhesion molecules was determined. RESULTS: Immunofluorescent analysis of the digestive mucosa showed alpha6beta4 integrin expression deficiency at the epithelial cell-lamina propria junction. Ultrastructural examination of the digestive mucosa revealed a complete epithelial detachment with a cleavage plane lying between the lamina densa and the basal pole of the enterocytes. Consistent with the absence of skin blistering, integrin alpha6beta4 was expressed at the dermal-epidermal junction. Electron micrographs of skin biopsy specimens showed the presence of normal hemidesmosomes and the absence of dermal-epidermal dysadhesion. CONCLUSION: It was postulated that this patient had protracted diarrhea related to epithelial detachment of the digestive mucosa as a consequence of a deficiency of an integrin alpha6beta4 isoform specific to the gut.

Antigens, Surface↗
PubMed1998

Autoimmune enteropathy in infants. Pathological study of the disease in two familial cases.

In two brothers with autoimmune enteropathy there was total villous atrophy in the small intestine and marked lymphoid cell infiltration in the lamina propria of the entire digestive tract, discovered at autopsy in one of these patients. In addition, the pancreas showed diffuse interstitial infiltration by lymphocytes. The liver was enlarged, with extensive haematopoiesis and cholestasis. Similar lesions in the digestive tract were noticed in the second boy, but on immunosuppressive therapy his diarrhoea gradually disappeared. When he was 16 months of age, percutaneous biopsies showed moderately aggressive chronic hepatitis and a focal interstitial lymphoid infiltrate in the kidney. After 3 years of immunosuppressive therapy (prednisone, cyclosporin), the child ate well and total parenteral nutrition was discontinued. The intestinal lesions had regressed but fibrotic lesions of the liver persisted.

Anti-Inflammatory Agents↗
PubMed1998

Interferon alfa therapy for chronic hepatitis B in children: a multinational randomized controlled trial.

BACKGROUND & AIMS: Treatment of chronic hepatitis B with interferon alfa is not approved in children. The aim of this study was to evaluate the safety and efficacy of interferon alfa (IFN-alpha) in children with chronic hepatitis B and increased transaminase levels. METHODS: Children were given either IFN-alpha2b (6 megaunits/m2 thrice weekly for 24 weeks) or no treatment. Clearance of markers of viral replication was evaluated 24 weeks after therapy and after 48 weeks of observation in controls. RESULTS: Of 149 children enrolled, 144 were evaluable (70 treated and 74 controls). Serum hepatitis B e antigen and viral DNA became negative in 26% of treated children and 11% of controls (P < 0.05). Serum aminotransferase levels normalized and liver histology improved among responders. Hepatitis B surface antigen became undetectable in 10% of treated patients and 1% of controls. Female gender and interferon treatment were the only significant predictors of response. Ethnic origin, baseline aminotransferase level, initial DNA levels, and histology did not correlate with response. Most adverse reactions were mild or moderate, and dose was reduced in 24% of children. CONCLUSIONS: In children with chronic hepatitis B, INF-alpha promotes loss of viral replication markers and surface antigen and improves aminotransferases and histology.

Adolescent↗
PubMed1998

[Legislative and ethical aspects of drug experimental in children and adolescents].

Experimentation of drugs in children meets with ethical and legal difficulties. After studying historical development of drug trials regulation, the different ways to define the modalities of clinical trials (law, technical aspect, deontology, ethics) are described. The importance of the informed consent and its particularities when children are concerned are emphasized. Finally, it appears ethical and necessary to practice drug trials in children in order to obtain safe and efficient drugs and prescriptions in pediatrics.

Adolescent↗
PubMed1997

[Hypersensitivity to 5-aminosalicylic acid. Value of desensitization by oral route].

BACKGROUND: Treatment with sulphasalazine of patients with mild and moderate forms of Crohn's disease may result in side effects in some of them. CASE REPORT: An 11-year-old girl with Crohn's disease was given 40 mg/kd/day of sulphasalazine after achieving remission with prednisone. She developed urticaria and eosionophilia 8 days later, then extended skin edema during a second course of sulphasalazine requiring methyl prednisolone therapy. Mesalazine administration (25 mg/kg/day), one month later, resulted to watery stools, vomiting, and fever, a side effect observed again after two further mesalazine challenges of one-month interval. Eosionophilia was present and the lymphocyte stimulation test with mesalazine was positive. Successful desensitization to sulphasalazine could be obtained 9 months later permitting further safe administration of this drug up to 18 months. CONCLUSION: Hypersensitivity to sulphasalazine may be successfully overcame by oral desensitization, especially in those patients with low-grade activity of Crohn's disease.

Administration, Oral↗
PubMed1996

[Autoimmune enteropathy].

Autoimmune enteropathy was initially described in young male infants presenting as severe secretory diarrhea. The disease is characterized by an inflammatory reaction which may involve several organs (bowel, pancreas, thyroid, kidneys, liver) with the presence of various circulating antibodies. The disease may also be observed in older children and in females with usually less bowel involvement. In view of the autoimmune basis of the disease, treatment requires immunosuppressive agents in addition to parenteral nutrition.

Autoimmune Diseases↗