[Medical follow-up, personal experiences and everyday life of young adults after liver transplantation during childhood]. / Suivi médical, vie quotidienne et vêcu de jeunes adultes après transplantation hépatique dans l'enfance.

UNLABELLED: Over the last two decades liver transplantation for children (pLT) with life-threatening acute or chronic liver diseases has yielded high long-term success rates. Long-term follow-up of pLT recipients has focused mainly on somatic complications (infections, chronic rejection, biliary problems, cancer occurrence, etc.). Other studies have examined precise aspects of everyday life, and particularly health-related quality of life. In contrast, no global surveys of everyday life, including educational and vocational issues, academic performance, personal feelings and concerns and at-risk behaviors have yet been carried out among adults who received liver grafts during childhood. We conducted a global survey of these young adults' everyday lives. POPULATION AND METHODS: The study was based on a structured questionnaire administered during phone interviews. One hundred sixteen pLT patients managed in a single pediatric liver unit since 1986 were interviewed between April 2005 and July 2006 by the same pediatrician (JPD), who was not involved in their personal medical management. Mean age at interview was 21 +/- 4 (17-33) years; mean age at pLT was 7.0 +/- 4.6 (0.5-16) years; and the mean and median follow-up periods after LT were respectively 13.9 +/- 3.9 years and 15 years. Three-quarters of the patients said they were satisfied with their quality of life and 81% were satisfied with their health status. A significant difference in the age at which LT was performed was found between patients reporting "good or very good" quality of life and patients reporting "neither good nor bad" quality of life (mean age at LT 6.2 +/- 4.1 vs 9.4 +/- 1.4 years; p = 0.0002). Two-thirds of the patients were still attending school. One-third were in age-appropriate school grades, and 31%, 23% and 13% were respectively 1, 2 and 3 years behind. Twenty-five per cent of patients were in paid employment and 12% were unemployed. Reported at-risk behaviors (tobacco and cannabis use) were not more frequent than among these patients' peers, and alcohol consumption was significantly lower (p<0.0001). Strict adherence to medications was reported by only 55% of patients. Concerns about their future health were expressed by 53% of patients. Many patients were reluctant to speak openly to their peers about their LT status. The vast majority of patients wished to discuss personal problems with a physician (quality of life, vocational problems, future health, sexuality, pregnancy), and also wanted more medical information from caregivers. A large majority of young adults transplanted during childhood have good quality of life. Educational level and academic performance are lower than among these patients' peers. This study highlights personal difficulties encountered by a noteworthy proportion of young adults transplanted during childhood. This needs to be taken into account both by pediatricians and by adult medical care providers.

[Usefulness of the child health record]. / Du bon usage du carnet de santé de l'enfant.

A good use of the child health record requires the respect of the confidentiality without compromising its informativeness. The maintenance of the confidentiality must be the subject of specific measurements in very particular cases like the infection by the HIV. The experts must be conscious that the personal child health record remains a tool for bond between the various professionals of health, in particular for an optimal follow-up of the growth and psychomotor development of the child. A specific teaching concerning the good use of the health record is wished in the course of the studies of medicine.

Features and outcome of autoimmune hepatitis type 2 presenting with isolated positivity for anti-liver cytosol antibody.

BACKGROUND AND AIMS: Autoimmune hepatitis (AIH) type 2 is identified by the presence in the serum of anti-liver/kidney microsome type 1 autoantibody. Anti-liver cytosol autoantibody has been reported in children with autoimmune liver disorders mostly in association with anti-liver/kidney microsome reactivity. However, its role as a sole marker of AIH type 2 is debated. We describe here a series of 18 children and adolescents (15 girls, 3 boys) with AIH with serum anti-liver cytosol type 1 (aLC1) as the only autoimmune marker. METHODS: A retrospective review was conducted from 3 pediatric hepatology units of all children with an autoimmune liver disease associated with aLC1 as found by immunofluorescence and/or immunodiffusion or immunoblotting. RESULTS: Age at first symptoms ranged from 11 months to 14 years; 12 children presented with acute hepatitis, 1 with progressive jaundice, and 5 were asymptomatic. Anti-liver/kidney microsome, antimitochondria, and anti-actin autoantibodies were not detected. Signs of cirrhosis were present in 11 children. Immunosuppressive treatment was effective in all except 2 children who had subfulminant hepatic failure and who required liver transplantation. Sixteen patients (14 with their native liver) currently are alive; 14 patients still are on immunosuppressive therapy after 1 to 22 years. According to the international scoring system for the diagnosis of AIH, 16 patients corresponded to a definite diagnosis and 2 corresponded to a probable diagnosis. CONCLUSIONS: The presence of aLC1 in children with acute or chronic liver disease of unknown origin strongly supports a diagnosis of AIH and is an indication for early immunosuppressive therapy.