Disorders of growth and puberty in children with non-tumoral hydrocephalus.

Hydrocephalus may cause disorders of growth and puberty. 31 patients (25 girls) with non-tumoral hydrocephalus were seen at 8.5 +/- 3.1 (SD) years for short stature (8 patients), overweight (8 patients), central early puberty (onset before 9 years, 21 patients), premature pubarche (1 patient) and/or delayed puberty (2 patients). Among the patients with short stature, 4 had meningomyelocele and one had untreated early puberty. Only 1/11 patients evaluated had growth hormone deficiency. Among the overweight patients, 5 had early puberty. The plasma leptin concentrations were positively correlated with the body mass index (r = 0.65, p < 0.01, n = 14). Free thyroxin, cortisol, prolactin and concomitant plasma and urinary osmolalities were normal in all cases evaluated, except one who had low free thyroxin. The 7 patients with early puberty and who were given gonadotropin releasing hormone analog for over 2 years had mean predicted adult height of -2.45 +/- 1.9 SD before treatment and -2.46 +/- 1.4 SD afterwards. Ventriculocisternostomy performed on 2 girls seen for delayed puberty was followed by breast development and menarche. In conclusion, in children with hydrocephalus, short stature is frequently due to meningomyelocele and rarely to GH deficiency. Central early puberty is the most frequent endocrine disorder.

[Terminal and pre-terminal chronic renal insufficiency in newborns in French neonatal intensive care units: survey of the French pediatric nephrologic society of resuscitation and emergency]. / Insuffisance rénale chronique terminale et préterminale du nouveau-né dans les services de réanimation français: enquête de la Société de néphrologie pédiatrique auprès du Groupe francophone de réanimation et d'urgences pédiatriques.

OBJECTIVES: The aim of this study was to describe the intensive care unit neonatologists' attitudes about a neonate with terminal or pre-terminal renal failure. METHODS: A questionnaire was sent to all French neonatal intensive care units. Physicians were asked to describe their attitude about neonatal chronic renal failure (Would you agree with dialysis and graft for these children?). Physicians were also presented with two clinical observations involving neonates with varying degrees of renal insufficiency and a complicating comorbidity, including neurological abnormality or socioeconomic circumstances. RESULTS: Responses were obtained from 92% of the university neonatal care units. The will to take care of a neonate with end-stage renal failure till the renal graft, varied greatly from a centre to another one. Three (9%) university-teams said they had a strong will to bring the baby from the neonatal period to the time of renal graft. Eleven other centres (32%) did not have any will for accompanying the baby till the renal graft. Eight centres (24%) would be rather favourable to the idea of dialysis and graft, and 12 others (35%) would be rather unfavourable. CONCLUSION: The results of this study show great differences between French neonatologists when they are faced to newborns with end stage renal failure. Ethical, medical and organisational difficulties are matters of controversy. The epidemiological impact of the perinatal discussion could be a 20% variation of all the renal grafts in children.

Mitochondrial respiratory chain deficiency revealed by hypothermia.

We observed a 17-month-old girl with profound and initially isolated episodes of hypothermia. Thereafter, she developed growth delay, repetitive corneal and bone lesions. Persistent hyperlactataemia in plasma and in CSF prompted us to investigate respiratory chain enzymes. A deficit in respiratory chain complexes III and IV was demonstrated in isolated skeletal muscle mitochondria, circulating lymphocytes and fibroblasts by spectrophotometric and polarographic studies. Moreover, UCP3 mRNA expression in muscle was decreased.