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Biomedical subjects

G Huault

Publications and source records attributed to G Huault.

At least 37 records · Page 2Linked to original sources

Outcome of ABO-incompatible liver transplantation in children with no specific alloantibodies at the time of transplantation.

The shortage of suitable liver donors for children has motivated the use of ABO-incompatible (ABO-I) grafts for transplantation in urgent situations. However, survival after ABO-I liver grafts has been reported at about 30% as compared with 80% in cases of ABO-identical or -compatible liver grafts. This difference has been attributed to antibody-mediated, hyperacute or chronic liver rejection, due to preformed ABO antibodies (alloantibodies). In this study, we report our results with ABO-I livers in children without alloantibodies at the time of transplantation. From January 1988 to June 1993, 143 OLT were performed in 122 children. Eight children received 8 ABO-I liver grafts. Of these, 7 patients were included in the study. All 7 were alloantibody free before OLT. Five children were spontaneously alloantibody free, while in 2 children, the plasma alloantibodies were eliminated before and after transplantation using intravenous infusion of specific blood group antigens of the donor blood group (soluble antigens). Immunosuppression consisted of a triple-drug treatment combining CsA, AZA, and steroids. The follow-up period was between 10 and 48 months. One child died from a surgical complication. Six children survived, but 1 died 10 months later from intestinal obstruction. There were no graft losses and no episodes of hyperacute or chronic rejection. The graft and patient survival rate was 71%. There was a 28% incidence of rejection, but all were mild (requiring steroid boluses only). Our results suggest that the absence of ABO alloantibodies at the time of and after transplantation can protect ABO-I liver grafts against antibody-mediated rejection, whether hyperacute or chronic, and that soluble antigens are effective in eliminating alloantibodies in children.

ABO Blood-Group System↗

[Homozygote CB deficiency revealed by recurrent Neisseria meningitidis infections in an adolescent].

BACKGROUND: Meningococcal infections associated with late complement component deficiency are rarely severe and usually occur during adolescence and adulthood. We report severe manifestations in a boy in whom the first episode appeared early. CASE REPORT: A 14 year-old gypsy boy was admitted because of a febrile meningococcal meningitis that was complicated by a rapidly extensive and necrotic purpura, obnubilation and clotting abnormalities without hemodynamic anomalies. The patient was given symptomatic therapy and a 12-day course of antibiotics that resulted in rapid and complete recovery. Medical history of this patient showed that he had been admitted at the age of 3 years for a severe febrile purpura with septic shock and clotting abnormalities followed by rapid and complete recovery after symptomatic and antibiotic therapy. No germ had been then isolated. The complement system was studied 3 weeks after the second hospitalization: total hemolytic complement activity could not be detected and C2, C3 and C4 were normal. Examination of the terminal pathway-revealed total C8 deficiency. The patient received meningococcal vaccine and was discharged on oral penicillin prophylaxis. He remained healthy during the ensuing 4 years. CONCLUSIONS: Meningococcal infections associated with late complement component deficiency are generally uncomplicated but they remain potentially severe. Early screening for this late complement component deficiency should be considered after severe clinical manifestations.

Adolescent↗

[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↗

[Pre- and postoperative intensive care after liver transplantations].

Physiological and metabolic disorders must be identified and treated prior to liver transplantation (if necessary plasmaphaeresis, hemofiltration in the case of severe hepatic failure). Postoperatively, the goal is to restore and maintain metabolic functions, to prevent any organ failure and to detect early complications. Primary liver dysfunction must be recognized as soon as possible in order to decide upon retransplantation. Hepatic artery thrombosis must be detected by doppler ultrasonography and managed surgically. Acute liver rejection must be identified and treated. Nosocomial infection should be avoided and specifically treated. The success of liver transplantation depends on the indications, patient selection, and the close cooperation between the members of the transplantation team.

Child↗

Coma associated with intense bursts of abnormal movements and long-lasting cognitive disturbances: an acute encephalopathy of obscure origin.

We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.

Acute Disease↗

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↗

[Severe poisoning with intravenous phenytoin in the newborn. Value of peritoneal dialysis].

A severe poisoning with intravenous phenytoin in a newborn was treated with peritoneal dialysis in spite of its reported poor effectiveness, the drug being 90% protein-bound. During 10 hours, 1.41 mg/hr were eliminated by biotransformation and 1.80 mg/hr were removed with peritoneal dialysis allowing to suppress quickly life threatening cardiac side effects. This efficacy may be explained by an increase in the unbound protein fraction and a diffusion speed through the peritoneum higher than in older patients.

Biotransformation↗

[Prognostic evaluation of severe head injuries in children].

In 34 children overcoming a severe head injury (coma greater than 1 d, mean: 10 d), the follow-up (mean: 25 m) has been correlated with several data. 1) The quality of life, according to a 3 grade-score, is mostly dependent upon the degree of neuropsychological sequelae. By decreasing frequency were noted disturbances of: memory, intelligence (the most significantly correlated with the quality of life), attention, rapidity of performances, behaviour, visuoconstructive activities. Most often several disturbances were associated, but without systematization. 2) Correlations between general outcome and early findings: a coma greater than 10 d, a subdural collection on the CT scan at 2-6 weeks have a pejorative meaning. Age does not appear to be relevant. 3) In a sequential study of neuropsychological functions, an early evaluation is poorly predictive, except in case of rapid normalization. Evaluation at 6 months gives a good picture of the outcome. Sectorial improvements can occur beyond the 1st year but do not appear to modify dramatically the general outcome.

Adolescent↗

[Severe thrombocytopenia induced by heparin in an infant with acute myocarditis].

A severe heparin-induced thrombocytopenia is reported in a child suffering from acute myocarditis. Thrombocytopenia (80.10(9)/l) occurred on day 5 (D5) of heparin therapy which was thus discontinued during 8 hours but reintroduced on the view of a negative platelet aggregation test using standard heparin. On D10, while thrombocytopenia persisted the presence of two intra-cardiac thrombi led to replace standard heparin by low molecular weight heparin (Fraxiparin), associated with vitamin K antagonist (phenindione). On D12, a 3rd intra-cardiac thrombus required immediate discontinuation of Fraxiparin therapy. Platelet aggregation tests performed on D14 were positive in the presence of standard heparin and of low molecular weight heparin, thus demonstrating the existence of thrombocytopenia induced by standard heparin and secondly by low molecular weight heparin. This observation led the authors to discuss the diagnosis and the therapeutic management of heparin-induced thrombocytopenia.

Acute Disease↗