[Cowden's disease: a new paediatric observation]. / La maladie de Cowden: une nouvelle observation pédiatrique.

We report on a paediatric observation of Cowden's disease in a 6-year-old child. Familial steroid-resistant nephrotic syndrome was associated to papulous and papillomatous lesions of gingiva and oral mucosa, multiple hamartoma of the back and of upper limbs, facial dysmorphism and follicular thyroid cancer. Thyroid cancer evolved favorably after surgical treatment, radioactive iodine and L-thyroxin supplementation. Nephrotic syndrome evolved to chronic renal insufficiency after 11 years. The early diagnosis of Cowden's disease, or multiple hamartoma syndrome, allows a careful monitoring of the patients who are facing the risk of cancer transformation, which is the principal complication of the condition.

[Gastrointestinal polyposis in Bourneville's tuberous sclerosis: an involvement often overlooked]. / La polypose digestive de la sclérose tubéreuse de Bourneville: une atteinte souvent méconnue.

Ten patients (5 male, 5 female, mean age: 29.4 years) with tuberous sclerosis were investigated for gastrointestinal polyps. Three had adenomatous colonic polyps, one had a single duodenal hamartomatous polyp and the fifth a hyperplastic gastric polyp. This high prevalence (50%) should put the gastro-intestinal investigation as a useful test in the diagnosis of tuberous sclerosis, particularly in the incomplete varieties of disease. Conversely, tuberous sclerosis should be considered in the differential diagnosis of digestive polyposis.