[Pseudohypoaldosteronisme type I: a rare cause of failure to thrive]. / Une cause rare de stagnation pondérale à la naissance : le pseudohypoaldostéronisme de type I.

We report on a boy, born on term, presenting with a weight loss and a persistent failure to thrive after 10 days despite a normal behavior under bottle-feeding. The clinical examination was normal and biological assessment revealed hyponatremia with hyponatriuria, normal kaliemia and elevated aldosterone values, leading to type I pseudohypoaldosteronism diagnosis. Treatment with salt supplementation allowed growth improvement. The diagnosis was confirmed by the identification of a mutation in the mineralocorticoid receptor. This change was also found in several family members.

[Verocytotoxin positive haemolytic uremic syndrome and HIV-associated nephropathy]. / Syndrome hémolytique et urémique typique et néphropathie à VIH.

UNLABELLED: Haemolytic uremic syndrome (HUS) and HIV-associated nephropathy (HIVAN) are common renal diseases in the course of HIV-infected patients. CASE REPORT: We report the case of a 13-month-old Caucasian boy hospitalised for a verocytotoxin positive HUS associated with HIV infection. After the acute phase of HUS the creatinine level returned to normal values. Because of progressive renal failure with severe overload hypertension and glomerular proteinuria despite antiretroviral therapy and angiotensine converting enzyme inhibitor, the child required peritoneal dialysis 12 months later. Clinical and biological course together with pathological findings were consistent with both typical HUS and HIVAN. CONCLUSION: This is the first paediatric case of typical HUS revealing a HIVAN. The association of HUS and HIVAN may explain the progression to end-stage renal failure despite antiretroviral therapy associated with angiotensine converting enzyme inhibitor and a good control of HIV replication. HIVAN is rare in children and may occur in the early phase of HIV infection even not only in black patients.

[Normal values in children of bone mineral content measured by dual photon absorptiometry]. / Valeurs normales chez l'enfant du contenu minéral osseux mesuré par absorptiométrie biphotonique.

We analyzed the results of dual photon absorptiometry measurements in 43 normal children. Results were correlated with age, body weight, and stature. Reference stature-specific values for bone mineral content in children are proposed. Furthermore, our results show that mineralization continues beyond the end of statural growth.

[Growth and bone maturity during congenital hypothyroidism screened in the neonatal period. Apropos of 82 cases]. / Etude de la croissance et de la maturation osseuse au cours de l'hypothyroïdie congénitale dépistée en période néonatale. A propos de 82 observations.

Systematic neonatal screening of congenital hypothyroidism (CH) allowed to detect 82 children (62 girls and 20 boys) including 73% with ectopic thyroid, 18% with athyreosis and 9% with eutopic thyroid. They were followed for 1 to 7 years according to the same protocol. Skeletal maturity was studied according to 2 methods: TW2 and Sempé's numerical. Height growth was compared to the standards for French children. T4 values were normal at all ages, TSH values were slightly higher than standard. Concerning the skeletal maturity the delay observed in boys lessened from age 6 years (TW2 method: less than 25th percentile until 3 years, = 25th from 3 to 5 years, between P25 and P50 by 7 years of age). This delay was less important in children who, at the time of diagnosis (day 15) had an epiphyseal surface greater than or equal to 5 mm2. In girls, it was always above the 25th percentile. The expression of the delay was greater with Sempé's method than with the TW2 method. Thus, early substitutive treatment of CH allows a normal height growth. The skeletal maturity is somewhat abnormal in boys. This could be explained by the high percentage (66.6%) of boys with a small epiphyseal surface during the neonatal period and by a slight under-treatment.

[Abnormalities of intestinal permeability of 51Cr-EDTA in mucoviscidosis]. / Anomalie de la perméabilité intestinale à l'EDTA-51Cr dans la mucoviscidose.

Intestinal permeability to 51Cr-EDTA was studied in 20 children with cystic fibrosis (CF) and a mean age of 10.7 years, in 7 control adults and in 11 control children. 51Cr-EDTA urinary excretion (mean +/- SD) expressed as a percentage of the orally administered activity was: control children: 1.59 +/- 0.55%, control adults: 1.47 +/- 0.62%, CF patients: 10.7 +/- 8.6%. The difference between CF patients and control children on one hand, between CF patients and control adults on the other hand, was statistically significant (p less than 0.001). Only 3 CF patients had values within the limits of those observed in controls. A statistically significant correlation (p less than 0.01) was observed between the 51Cr-EDTA urinary excretion and steatorrhea. No correlation was found between 51Cr-EDTA urinary excretion and the following parameters: age, sex, weight, height, Shwachman score, liver cirrhosis, oral administration of a mucolytic agent. The eventual, especially nutritional, consequences of this increase of the intestinal permeability in the course of CF remain to be clarified.