[Physical activity and exercise training for patients with cystic fibrosis]. / Activité physique et réentraînement à l'effort du patient atteint de mucoviscidose.

In France patients with cystic fibrosis benefit from a multidisciplinary follow-up in Cystic Fibrosis Centres. In this follow-up, despite the numerous therapeutic benefits of exercise in this disease, little emphasis is placed on the promotion of physical activity. The aim of this article is to improve this aspect of management, giving advice from a working group of experts, based on the medical literature and clinical experience. These proposals include quantification of physical activity, evaluation of exercise, training and rehabilitation programs and finally, modification of behaviour to include physical activity in the overall cystic fibrosis treatment strategy. It is intended to set up multicentre studies to evaluate the impact of these proposals.

[Elastosis perforans serpiginosa with vitamin A deficiency in a child with trisomy 21]. / Elastome perforant serpigineux avec hypovitaminose A chez une enfant ayant une trisomie 21.

INTRODUCTION: Elastosis perforans serpiginosa frequently occurs in trisomy 21. Usually no cause is found. We report a case in which vitamin deficiency may have contributed to the development of skin lesions. CASE REPORT: A 11-year-old girl with trisomy 21 developed papulokeratosic eruptions with a linear serpiginous distribution, predominantly involving the lower limbs. On ultrastructure examination, numerous elastic fibers penetrated the epiderma and the baseline membrane had disappeared. The patient also had a totally patent atrioventricular canal with hypoplasia of the left ventricle and cardiac liver. Serum vitamin A level was low (0.56 mumol/l, normal > 1.55). Acitretine was prescribed at the dose of 0.5 mg/kg/day but had to be stopped 2 months later due to elevated liver enzyme levels despite a clear clinical improvement. DISCUSSION: This association between elastosis perforans serpiginosa and vitamin A deficiency, observed here in a child with trisomy 21, has never been reported by others. Vitamin A deficiency might aggravate the skin lesions. In our case, there was probably a relationship between the vitamin A deficiency and the cardiogenic liver disease. The keratoregulatory effect of vitamin A on elastic tissue is less well known. Treatment with retinoids provided clinical improvement but had to be stopped due to hepatotoxicity. Parenteral vitamin A would be an interesting alternative but the risk of side effects would theoretically be greater than with oral retinoids.

[Healthy subjects with malignant cells in their sputum. Apropos of 60 cases]. / Les cracheurs sains de cellules malignes. A propos de 60 cas.

Sixty cases of occult carcinoma of the lung are described. A distinction is made between "revealed cases" (23 cases) in which the carcinoma revealed itself within 5 months after the first cytological findings, and true occult carcinomas (37 cases) in which lesions developed within 5 months to 8 years after the first positive cytology. Mean survival in the "revealed cases" was 13 months. It was 51 months for true occult lung carcinomas and 68.5 months for occult carcinomas treated by chemotherapy starting at the time of discovery of malignant cells in the sputum. The method of choice is selective bronchial aspirates and brushings after repeated sputum cytology which allows for localization of the lesion and early surgery.