[Prader-Willi syndrome: specific management by a multidisciplinary team]. / Le syndrome de Prader Willi: intérêt d'une prise en charge pluridisciplinaire.

Prader Willi syndrome can be viewed as a physiopathological model of obesity. Such patients deserve specific management, preferably in a multidisciplinary setting. The paper reports on 6 patients followed in the paediatric endocrine service at the University of Liege.

Cardiac autonomic dysfunction in diabetic children.

OBJECTIVE: Adults with type 1 diabetes may have abnormal alterations in heart rate variability (HRV) due to cardiac autonomic neuropathy. This prospective study was performed to determine whether HRV can be used to detect subclinical autonomic neuropathy in diabetic children. RESEARCH DESIGN AND METHODS: We examined five time domain and three frequency domain HRV indices determined from 24-h Holter recordings in 73 diabetic children and adolescents aged 3-18 years (mean 12.1 years) with a mean duration of diabetes of 55 months. The measures were compared with normal ranges. Z scores were established for each parameter and were compared with classic risk factors of other diabetic complications. RESULTS: Most HRV indices were significantly depressed in children aged > or = 11 years, and the levels of HRV abnormalities were significantly correlated with long-term metabolic control (mean GHb for 4 years) in that age-group. In younger patients, HRV indices were within the normal range and were not correlated with the level of metabolic control. Illness duration and microalbuminuria but not short-term metabolic control (most recent GHb) were also independently predictive of HRV abnormalities. CONCLUSIONS: These results suggest that early puberty is a critical period for the development of diabetic cardiac autonomic dysfunction. Therefore, all type 1 diabetic patients should be screened for this complication by HRV analysis beginning at the first stage of puberty regardless of illness duration, microalbuminuria, and level of metabolic control.

[Diabetes mellitus in childhood and adolescence. 1. Specific management by a multidisciplinary team]. / Le diabète de l'enfant et de l'adolescent. 1. Prise en charge par le Service Universitaire de Pêdiatrie et la Cellule "Education-Diabète en Pédiatrie".

The management and follow up of diabetes in youth is a multidisciplinary challenge due to both short and long term objectives. Awareness of the feelings and problems faced by the families is critical. The experience of our team has started in the 1960s and is briefly described and updated in this article.

[Diabetes mellitus in childhood and adolescence: 2. Specific aspects in adolescents]. / Le diabète de l'enfant et de l'adolescent. 2. Aspects particuliers chez l'adolescent.

The management of adolescents with diabetes mellitus involves specific aspects at diagnosis and during follow-up. A type 2 diabetes should be excluded at diagnosis since this condition is increasingly observed in closed relationhip with the progression of obesity in young people. During initial education process, the parents should be involved while a specific space and time for interaction with the adolescents is required. During follow-up, all aspects of the adolescent process should be taken into account together with diabetes. This includes risk-taking or exploratory behaviours, feeling of being different, angryness and difficulties for the adolescents to consider the long-term complication risk. Special attention should be devoted to the transition towards adult care.

[Implementation of oral rehydration in gastroenteritis in infants]. / Actualisation de la réhydratation orale dans la gastro-entérite du nourrisson.

The best treatment of the infant acute gastroenteritis, when it is applied at home, is oral rehydration. The latter should be limited in duration (between 12 to 24 hours at the longest), should be performed with a specifically prepared solution, should be administered in sufficient amount and should be followed with an early realimentation.

[The clinical case of the month. Malignant whooping cough in an infant]. / Le cas clinique du mois. Coqueluche maligne du nourrisson: à propos d'un cas.

Morbidity and mortality of whooping cough remain significant among infants despite vaccination. This article presents a case of whooping cough with complications in a 3 month old infant who had already received his first dose of anti-pertussis vaccine at 2 months and 3 weeks of age.

[How I investigate...difficult cases of tuberculous meningitis]. / Comment j'explore ... certains cas difficiles de méningite tuberculeuse.

To formally document the presence of the bacillus of Koch in meninges still remains difficult and depends on many variables. We report two cases where the diagnosis proved difficult. The presentation of tuberculous meningitis can take several aspects. Mantoux reaction is frequently negative. The BK cultures, which demonstrate the diagnosis, depend on the volume of CSF and on the importance of BK dissemination. It, unfortunately, remains difficult to make the diagnosis of tuberculous meningitis even though this pathology can have extremely deleterious consequences.

[Pneumocystis carinii pneumonia in a child without immunodepression]. / Pneumonie à Pneumocystis carinii chez un enfant non immunodéprimé.

We report the case of a 5 1/2 month baby with an atypical pneumonia. The PCR revealed a Pneumocystis carinii pneumonia. The complementary approach failed to show any immunosuppression. We will discuss the aetiology, physiopathology and treatment of Pneumocystis carnii pneumonia, particularly in cases without immunosuppression. We will also recall the importance of this disease in AIDS.

[Self instruction, multiple choice questions. Daily management of diabetes in children and adolescents]. / L'autoapprentissage, questions de choix. Gestion quotidienne du diabète chez l'enfant et l'adolescent.

The practitioner has usually relatively little experience in the daily management of pediatric diabetes, a complex issue due to multiple factors and individual variability. Seventeen multiple choice questions are proposed, which have been created for an interactive teaching session (see next article). For each question, a single answer is considered to be the most or the only correct one. This answer is given after the questions together with a brief comment about some of the proposed answers. The objective for the reader is self-teaching. The questions are not intended for self-evaluation.

Action of phenobarbital given to rats together with diethylnitrosamine on the O6-ethylguanine content of liver DNA.

When rats are fed diethylnitrosamine (10 mg/kg/day), no O6-ethylguanine is found in liver DNA after 2 weeks, but a considerable amount accumulates after 4 weeks. On the other hand, a 2-week feeding of diethylnitrosamine is not sufficient to induce liver cancers, whereas a 4-week treatment leads to hepatocarcinomas in 50% of the animals. Administration of phenobarbital (75 mg/kg/day) together with diethylnitrosamine during 4 weeks prevents the formation of liver cancers. It also prevents accumulation of O6-ethylguanine in liver DNA. Phenobarbital does not change the amount of O6-ethylguanine repair activity present in liver chromatin after 2 or 4 weeks of treatment with diethylnitrosamine. It is thus concluded that, by inducing the development of the endoplasmic reticulum, phenobarbital decreases the equilibrium concentration of the ultimate carcinogen derived from this indirect alkylating agent.

Inactivation of alpha(2)-Macroglobulin by Activated Human Polymorphonuclear Leukocytes.

The proteolytic activity of trypsin releases the dye Remazol Brilliant Blue from its high molecular weight substrate, the skin powder (Hide Powder Azure, Sigma), with an increase in absorbance at 595 nm. Active alpha(2)- macroglobulin (80 mug/ml) totally inhibits the proteolytic activity of trypsin (14 mug/ml) by trapping this protease. But after a 20 min incubation of alpha(2)-macroglobulin at 37 degrees C with 2 x 10(6) human polymorphonuclear leukocytes activated by N-formyl-L-methionyl-L-leucyl-L-phenylalanine (10(-7) M) and cytochalasin B (10(-8) M), 100% of trypsin activity was recovered, indicating a total inactivation of alpha(2)-macroglobuHn. Incubation with granulocyte myeloperoxidase also inactivates alpha(2)-macroglobulin. Hypochlorous acid, a by-product of myeloperoxidase activity, at a concentration of 10(-7) M also inactivates alpha(2)-macroglobulin, which indicates that an important cause of alpha(2)-macroglobulin inactivation by activated polymorphonuclear leukocytes could be the activity of myeloperoxidase.