[Recovery of Cushing syndrome revealing McCune-Albright syndrome]. / Guérison d'un syndrome de Cushing révélateur d'un syndrome de McCune-Albright.

Cushing syndrome (CS) is a rare feature of McCune-Albright syndrome. Treatments consist of bilateral adrenalectomy followed by lifelong glucocorticoid and mineralocorticoid treatment. However, cases of spontaneous remission of CS have been reported in the literature. We report a case of McCune-Albright syndrome with CS treated with metyrapone for 30 months with prolonged remission after a 12-year follow-up. Adrenalectomy may be avoided in some cases of CS caused by McCune-Albright syndrome. Metyrapone could be a good alternative to surgical treatment.

[Severe corrosive oesophagitis : are high doses of methyl prednisolone efficient to prevent oesophageal caustic stricture in children?]. / Les oesophagites caustiques severes. Les doses elevees de solumedrol sont-elles efficaces pour prevenir les stenoses caustiques ?

BACKGROUND: Corrosive oesophagitis stricture is the long term complication of severe corrosive oesophagitis. The aim of our study was to evaluate the effect of a high doses of steroids on incidence and quality of oesophageal stricture. PATIENTS AND METHODS: We reviewed the case histories of 28 children seen at children hospital from 31 December 1991 to 31 december 2001. These children has second and third degree oesophageal burns and they were treated by systemic Methylprednisolone (1000mg/1,73/m2 SC). RESULTS: the frequency of stricture was 12/26 (46%). Ten children required A mean of 6,7 (5,74) dilatation range (1 - 17). One patient required an oesocoloplasty. The frequency of stricture in the group treated early before the 24th hour and after the 24th hours was (9/21) (47,4%) versus (3/7) (42,9%). This difference was not significant (P = 1). The frequency of stricture in the group treated less than 21 days and more than 21 days was 6/17 (40%) versus 6/9 (66,7%). This difference was not significant (P = 0,400). High doses of methyl prednisolone seems to decrease the risk of oesophageal stricture. We found no difference between the children treated before the 24th hours and those treated after the 24 hours and those treated less than 21 days and those more than 21 days.

[High doses of steroids in the management of caustic esophageal burns in children]. / Corticothérapie à forte dose dans le traitement des oesophagites caustiques sévères chez l'enfant.

UNLABELLED: Esophageal burns are frequent in some countries. Esophageal stricture is a severe complication after caustic ingestion. Its frequency is estimated to 5% and reaches 47% in severe esophagitis. AIM OF THE STUDY: To study the influence of management of severe esophageal burns with high doses of steroids in the occurrence of esophageal stricture through the experience of an endoscopic unit. PATIENTS AND METHODS: Twenty-six children with a mean age of 3.5 years (15 months-8 years) with a second b- (N =22) or a third-degree (N =4) esophageal burns due to accidental ingestion of a caustic substance were included between 1993 and 1999. Corrosive substances ingested were sodium hydroxide (N =17), bleach (N =4), alkali (N =3), others (N =2). Upper GI endoscopy was performed in 17 children within the 24 hours of caustic ingestion and within the first 48 hours in all cases. All patients received methylprednisolone (1 g/1.73 m2/day) plus cimetidine and ampicillin plus early oral feeding resumption. RESULTS: Three children (2 grade II et one grade I) were not controlled on day 30 of the protocol and have then been excluded. The remaining 23 children were divided in two groups depending on the upper GI endoscopic results: group I of 12 patients completely or partially healed and group II of 11 patients who developed an esophageal stricture. No difference was observed between the two groups with regard to the delay between caustic ingestion and the start of treatment and the number of shots of methylprednisolone. Second b- degree esophagitis complicated with an esophageal stricture underwent a median of five esophageal dilations (1-12). After a median follow-up of three years, four of them have a normal esophagus. All children with a third-degree esophagitis developed an esophageal stricture. One had a surgical replacement of the esophagus with a segment of colon with good outcome. The three others underwent a median of seven esophageal dilations (5-10). One of them has a normal esophagus after a follow-up of five years while the two others were lost to follow-up. CONCLUSION: High dose of corticosteroids seems to improve second b-degree esophagitis prognosis and may prevent from esophageal stricture.

[Therapeutic management and clinical course of severe caustic oesophageal burns in children treated with methyl-prednisolone. Experience at a digestive endoscopic unit]. / Analyse des modalites therapeutiques et evolutives des oesophagites caustiques severes chez l'enfant traites par les bolus de methyl prednisolone. Experience d'une unite d'endoscopie digestive.

We reviewed the case histories of 28 children seen at children hospital from 31 December 1991 to 31 December 2001. These children has second and third degree oesophageal burns and they were treated by systemic Methylprednisolone (1000mg/1, 73/m2 SC). We divided the 26 children in four groups according to the time we began the steroids (before or beyond the 24th hours of the accident and according the number of steroids's bolus (less or more than 21 bolus). We analysed the number and the treatment of stricture in each group. High doses of methyl prednisolone seem to decrease the risk of oesophageal stricture. We found no difference between the children treated before the 24th hours and those treated after the 24 hours and those treated with less than 21 bolus and those with more than 21 bolus.

[Heterotopic gray matter. Report of four pediatric cases]. / Hétérotopies de la substance grise. A propos de quatre observations pédiatriques.

Severe infant epilepsy is included within difficult etiologic diagnosis. Gray matter heterotopias are an uncommon cause. The authors report four observations of gray matter heteropias concerning three-, six-, seven- and nine-year-old girls, presenting no particular antecedents. No consanguinity was noted. The first occurrence of epilepsy ranged from the age of nine months to the age of four years. A mild mental retardation was found in three cases, and mental regression in one case. A status epilepticus was noted in three children. Magnetic resonance imaging scans showed subependymal heterotopias in one case and diffuse cortical heterotopias in three cases associated to a partial agenesis of corpus calloseum in one case and pachygyria in two cases.