[Familial esophageal leiomyomatosis associated with Alport's syndrome in a 9-year-old boy]. / Léiomyomatose oesophagienne familiale associée à un syndrome d'Alport chez un garçon de 9 ans.

The authors describe a family in which the mother and one son are affected by oesophageal leiomyomatosis and nephritis with haematuria. The mother also presents hypertrophy of vulva and clitoris, and her son has perceptive deafness and congenital cataract. In the medical literature only 15 cases of oesophageal leiomyomatosis in children and adolescents could be found. The association with Alport's syndrome was first described by Torres and Guarner in 1983.

[Laurence-Moon-Bardet-Biedl syndrome, chronic diffuse tubulo-interstitial nephritis and liver involvement]. / Syndrome de Laurence-Moon-Bardet-Biedl, néphrite tubulo-interstitielle chronique diffuse et atteinte hépatique.

The authors report the case of a 6-year-old girl with Laurence-Moon-Bardet-Biedl syndrome and chronic tubulo-interstitial nephritis with glomerulosclerosis requiring chronic peritoneal dialysis, then haemodialysis. One year later, minimal hepatic involvement occurs, leading to portal hypertension. The liver biopsy reveals periportal fibrosis with many small bile ducts.

[Accidental poisoning by volatile hydrocarbons in children (apropos of 57 cases)]. / Intoxication accidentelle par les hydrocarbures volatils chez l'enfant (a propos de 57 observations.

Most of the accidentally intoxicated children who are admitted into hospital have swallowed volatile hydrocarbons. The ingested products are either aliphatic hydrocarbons, whether white spirit or petrol, or aromatic hydrocarbons. We have collected informations upon 57 children admitted into the pediatric department of The American Memorial Hospital in Reims, from 1973 to 1982. The children's average age is 24 months and they have usually swallowed very small amounts of the products. Early clinical manifestations involve mainly respiratory and digestive systems, while the most frequent manifestation is high fever. The early roentgenologic manifestations have a predominantly alveolar pattern which is to be found in the lower lobes. The appearance of pneumatoceles is the typical roentgenologic disturbance. The recovery is usually complete. Hydrocarbons directly reach the lungs down the respiratory tract-even without the children's swallowing down the wrong tract-owing to the physical characteristics of those products. That accounts for any gastric lavage being both unnecessary and dangerous.

[Choroid plexus papilloma (author's transl)]. / Les papillomes des plexus choroïdes.

The authors report a new case of choroid plexus papilloma. Hydrocephalus produced by this tumour has special features. Diagnosis is made by CAT scan. Despite surgery and the begnignity of such tumours, prognosis is generally poor. In the reported case two radiological pictures over a 5 year period did not any increasing of the size of the tumour.

[Localized form of xanthogranulomatous pyelonephritis in a child. Case report]. / Forme localisée de la pyélonéphrite xanthogranulomateuse chez l'enfant. A propos d'un cas.

The authors report the case of a 4 1/2-year-old boy with a localized xanthogranulomatous pyelonephritis. Review of the literature shows that this rare condition is often misdiagnosed before surgery, a preoperative diagnosis of Wilms' tumor being proposed instead of the exact one which is of benign prognosis. The pathogenesis is unknown.

[Renal lesions in congenital syphilis. A case report]. / L'atteinte rénale de la syphilis congénitale. A propos d'une observation.

The authors describe a case of congenital syphilitic nephropathy in an infant. The review of the reports in the literature shows many similarities of this congenital disease with the syphilitic nephrotic syndrome in the adult. The prognosis is favorable provided the diagnosis is made sufficiently early. Histological studies utilizing light-, electron- and immunofluorescence-microscopy gave results consistent with extramembranous glomerulonephritis with possible evolution towards endo- and extracapillary proliferative glomerulonephritis. This is one of the rare renal diseases with a known antigen.

[Visceral Kaposi sarcoma associated with a thyroid medullary carcinoma in a child in prolonged remission of lymphoblastic leukaemia (author's transl)]. / Sarcome de Kaposi viscéral associé à un carcinome médullaire thyroïdien chez une enfant en très longue rémission de leucémie lymphoblastique.

This report concerns a 12-years-old child, in remission for 8 years from lymphoblastic leukaemia. The course was rapidly fatal. The authors compare this case with those published in the literature of Kaposi sarcoma occuring in transplant patients and discuss the possible mechanisms, in particular the role of immunodepression.