[Heat stroke and topiramate]. / Coup de chaleur et topiramate.

Heatstroke, which is a major disorder related to environmental hyperthermia, is a rare event in children. The risk is increased with predisposing medical conditions and specific medications. We report the case of a 10-year-old epileptic patient, who received topiramate. Topiramate causes hypohydrosis and hyperthermia. We suggest that topiramate treatment may be a risk factor for heatstroke.

[Medical aspects of commercial air travel]. / Aspects médicaux des voyages aériens commerciaux.

A high proportion of commercial plane passengers are children: two million fly every year on Air-France airlines. In this field, the physician can be called upon to authorize or prohibit travelling. He could take measures or offer his services at the request of the cabin staff. The authors review flight physiology and stress, their potential effects on healthy children or those with medical conditions, and their fitness to fly.

[Dietary water intoxication: a preventable pathology]. / Erreur diététique et intoxication par l'eau: une pathologie évitable.

CASE REPORT: The authors report the case of an infant presenting with status epilepticus, apneas and hypothermia, related to hyponatremia. The cause was water intoxication, secondary to feeding a dilute formula and free water. The parents were notoriously challenged by the care of their children. Such disorder could have been prevented. CONCLUSION: Dietary history may be key to the diagnosis of hyponatremia in children. Water intoxication of nutritional origin can be prevented.

Apparent segregation of null alleles ascribed to deletions of the ornithine transcarbamylase gene in congenital hyperammonaemia.

Ornithine transcarbamylase (OTC) deficiency is an X-linked disorder of the urea cycle mapped to chromosome Xp21.1. Here, we show that apparent segregation of null alleles at the OTC locus and flanking polymorphic loci mimicked false maternity or false paternity in three affected families. Based on these observations, we suggest giving consideration to gene deletion when dealing with segregation of null alleles in OTC deficiency.

[Congenital cyst of the choledochus manifested by hemobilia]. / Kyste congénital du cholédoque révélé par une hémobilie.

A 18-months-old boy, without antecedent nor abdominal trauma, revealed a congenital choledochal cyst by massive hemobilia. This way of revelation is extremely rare and to our knowledge has not been previously reported. The difficulties of diagnostic and surgical management are discussed.

[Recklinghausen's disease and pseudohermaphrodism]. / Maladie de Recklinghausen et pseudo-ambiguïté sexuelle.

A clitoral hypertrophy in a girl presenting with Recklinghausen's disease should suggest the possibility of a local neurofibromatosis without any endocrine etiology. Thirteen cases collected in the literature and a new one reported here, give the opportunity to discuss such a possibility. Better knowledge of this unusual localization should, in each case, allow to discuss the extent of hormonal investigations needed.

Inhibitory action of serum from a Laron dwarf on normal cellular function.

Glucose uptake and O2 consumption of confluent glial cells grown in culture were measured in the presence of serum-free buffer and compared with those measured in the presence of serum from a normal volunteer, from an hGH-deficient dwarf and from a Laron dwarf. Cellular glucose uptake and respiration in the absence or presence of insulin or hGH are inhibited by Laron serum.

Sleep related growth hormone and prolactin secretion in children during constant rate enteral nutrition.

The aim of this study was to investigate the nocturnal secretion of growth hormone and prolactin in a particular model where nutrients are delivered continuously. Six children with severe intestinal diseases undergoing total constant rate enteral nutrition for 1.5 to 8 months have been studied; all children had a normal nutritional status at the time of the recording. Sleep patterns were studied by the usual polygraphic methods from 10 p.m. to 8 a.m. Blood samples were taken every 20 min through an indwelling catheter for growth hormone and prolactin plasma level determination. Several growth hormone peaks were observed with a peak always secreted in connection with stage III-IV of the first cycle. This early peak was significantly higher than the following ones. Nocturnal patterns of prolactin secretion showed individual differences characterized by a series of episodic releases which consisted of a few long rises (4 patients) and several small fluctuations; no correlation was found with the sleep patterns; no increase in the level throughout the night was observed. Loss of the rhythmicity of alimentation does not alter the secretion of growth hormone during sleep.

[Effect of constant rate enteral nutrition on gastrin secretion in children (author's transl)]]. / Effet de la nutrition entérale à débit constant sur la sécrétion de gastrine chez l'enfant.

The aim of this work was to study gastrin secretion in children receiving constant rate enteral nutrition (CREN) then avoiding intermittent stimulation induced by meals. Induced hypergastrinemia was performed in 12 children during CREN and compared with that in 8 children with intermittent oral feeding as a control group. Before stimulation by protein load there was no significant difference between the 2 groups; 20 minutes after stimulation, there was no change in the serum gastrin concentration in the CREN group, but a dramatic increase in the control group. The hypothetic role of gastrin and/or intestinal factors is advanced to explain the inhibition of gastrin secretory response during CREN. A progressive substitution from continuous to discontinuous intakes is suggested to avoid weaning complications of this nutritional technique.

[Stupor due to sodium valproate. Immediate arousal after intravenous diazepam (author's transl)]. / Etat stuporeux prolongé au cours d'une monothérapie au valproate de sodium immédiatement réversible après injection in intraveineuse de diazepam.

A 7 year-old boy had had partial complex seizures from the age 6 years. Two days after commencing a treatment by VPA in a daily dose of 55 mg/kg body weight, disturbances of consciousness became obvious. He was admitted to hospital two days later. The E.E.G. on admission showed bilateral delta activity. The plasma levels of VPA was 140 mg/l. Diazepam, 7 mg, was given intravenously. Four minutes later, the clinical state and the E.E.G. returned to normal. This observation supports the hypothesis that VPA may have a paradoxical epileptogenic effect in some types of epilepsy.