Progressive muscular dystrophy. Functional improvement after a renal allograft.

From his first years a child showed signs of a primary and rapidly developing muscular dystrophy. The diagnosis was established by an increased serum CK level and by electromyography and muscle biopsies. Afterwards this child developed a severe renal deficiency which needed binephrectomy and the graft of a normal kidney. During the few months just after the graft, the disability increased and the patient could not stand upright by himself. Later on, he gradually became able to walk on his own and without bracing. He could climb stairs and stand up from the floor. The CK activity returned to normal. At present, 4 years after the graft (the patient is 16 years), the improvement of his functional abilities is constant, although the CK activity has increased again. In this article we give evidence that this patient suffers from a primary muscular dystrophy. We discuss the type of dystrophy concerned. We believe that it is the graft of a normal kidney which was responsible for the improvement observed, and not the physiotherapy or the drugs administered after the graft.

[Precocious puberty connected with a hamartoma. Treatment with medroxyprogesterone. 2 cases]. / Puberté précoce liée à un hamartome. Traitement par la médroxyprogestérone. Deux observations

PIP: 2 cases of precocious puberty, a boy diagnosed at age 22 months and a girl at 40 months, were treated with farlutal (medroxyprogesterone) orally. The boy had excessive appetite and penile development at 16 months. Upon examination at 22 months, his bone age was 10 years, and his height and weight were above the third standard deviation. Fractional gas encephalography showed a hamartoma 10 mm in diameter. 3 tablets of farlutal daily were prescribed from 4 years of age to 7 1/2 years. The girl, a twin, had breast development and pubic hair at 3 years, and was examined at 3 years 4 months when she was over the second standard deviation in height and weight, although her twin was below the mean. She also had a hamartoma visible by fractional encephalography. She menstruated at 3 years 11 months and began taking farlutal 20 mg/day, which was increased to 40 mg/day from 4 years to 8 years of age. Pubertal development was arrested by medroxyprogesterone in both children, but bone age was not affected.^ieng