[Bardet - Biedl syndrome in the child. A study of 11 cases]. / Le syndrome de Bardet - Biedl chez l'enfant. Etude de 11 observations.

BACKGROUND: The syndrome of Bardet-Biedl is definite clinically by the association of obesity, polydactyly, pigmentary retinopathy, hypogonadism and backwardness. AIM: To study the epidemiologic, clinical, biological, genetic, therapeutic and evolutionary characteristic of our patients. METHODS: We carried out a retrospective study concerning 11 hospitalized children and/or follow-ups with the service of pediatry of the CHU Hédi Chaker of Sfax for syndrome of Bardet-Biedl during a period of 21 years (1987-2007). RESULTS: The obesity was constant among all patients, polydactyly was found in 9 cases, the fall of night vision in 7 cases. The hypogonadism was constant among all our boys. The bottom of eye was practised among 9 patients, it showed a pigmentary aspect of retinopathy among 8 patients. The electroretinogram was done in 10 patients, it showed a pigmentary retinopathy in all the cases. The radiological exploration of the urinary tract made it possible to identify morphological anomalies in 3 cases. The genetic study concerned the families of one of our patients and it allowed the identification of a new gene BBS8 at one of the families. Treatment was only symptomatic. After 6 years an average retreat, we noted an aggravation of obesity (9cas) and visual deficit (7cas). Only one patient evolved to the chronic renal insufficiency. CONCLUSION: The syndrome of Bardet-Biedl is a hereditary disease characterized by a genetic heterogeneity. The diversity of the systemic attacks defining this syndrome is a source of several handicaps: blindness, backwardness and obesity. The forecast is conditioned by the renal attack of or the interest of an early tracking and genetic council.

[Protective effect of breast feeding in childhood obesity]. / Place de l'allaitement maternel dans la prevention de l'obesite infantile.

THE AIM: To evaluate the frequency of obesity in children scolarised in Zaghouan, and to show the protective effect of breast feeding. METHODS: A retrospective study, included 162 children (aged 2 to 6 years), from 3 kindergartens situated in Zaghouan, during 2006. A questionnaire was drawn up, filled out by parents, concerning the breast feeding, the duration, the family antecedents of obesity, and the nutritional practices. Obesity is defined by an Body Mass Index (BMI) > or =97 centile referring to curve of the centiles of BMI for the age (Rolland Cachera). RESULTS: 86% of children were breast fed. 85 children (52%) were nursed by breast exclusively 6 months or over (A group). 54 (34%) are exclusively breast-fed lower than 6 months (B group). 23 (14%) were never breast fed (group C). An overweight was found in 20 children (12%). This overweight is noted respectively in 8%, 9% and 8/23 in the groups A, B and C. Our results showed that there is a statistically significant relation between the BMI and the breast feeding (P < 10 -8). Breast feeding has probably a protective effect against obesity of the child (Odds Ratio = 0.17). CONCLUSION: Promotion of the breast-feeding and information on its methods and its control are a public health priority, it protects the child and his mother from certain diseases and prevents from childhood obesity. OMS recommends an exclusive breast feeding until the 6 months age, and to continue if possible until the 2 years.

[Clinical features and treatment in Cushing's disease. 18 cases]. / Aspects cliniques et thérapeutiques de la maladie de Cushing. A propos de 18 cas.

We report a retrospective study about 18 Cushing's diseases in the department of endocrinology at Charles Nicolle hospital in 24 years. The mean age was 33.7 years with a male-female ratio of 4/14. Diagnosis was established on adrenocorticotropin hormone analysis and pituitary imaging. Transsphenoidal adenomectomy was released in 8 cases of Cushing's disease, it was successful in 5 patients (62.5%). Bilateral adrenalectomy was performed in three cases.

[Case report: bilateral Cushing's syndrome]. / Syndrome de Cushing bipolaire: a propos d'un cas.

The authors report a case of a 49-year-old woman presenting a Cushing's syndrome (January 1997). The Magnetic Resonance Image of the pituitary gland revealed a microadenoma without extension in the cavernous sinus and a partial empty sella. The computed tomography scan showed a discreet bilateral adrenal hyperplasia with a left nodule (23 mm in diameter) a second nodule was noted. These data suggested the eventuality of maconodular adrenocortical hyperplasia in long-standing Cushing's disease. We discuss the implications of this finding for diagnosis, treatment and follow-up.