Renal manifestations of tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous disorder involving the skin, brain, kidney, heart and other organs. Renal manifestations are mainly angiomyolipomas, renal cysts and cancer. We report three female patients, mean aged of 31 years (range: 22-37), with a family history of TSC in one case. Cutaneous and nervous symptoms were found in all patients. Clinical complaints were mainly abdominal pain (3 cases), hematuria (2 cases), right flank palpable mass (1 case). Angiomyolipomas (AML) were diagnosed by ultrasonography in all patients, associated to renal cysts in one case. A patient underwent total right nephrectomy without any event during the follow-up. In a second patient, a selective arterial embolization of AML was indicated. Total left nephrectomy was performed due to the persistence of hematuria and the increased flank mass. Pathological examination of the kidney revealed a clear cell carcinoma. The third patient with small AML, associated with renal cysts, required careful monitoring. Renal manifestations in TSC are frequent and serious, they are the second leading cause of death after nervous lesions. Clinical and morphological aspects are variable and different therapeutic indications must be discussed.

[Calcium needs in hemodialyzed-parathyroidectomized patients]. / Les besoins en calcium chez les hémodialysés parathyroïdectomisés.

Parathyroidectomy changes the homeostasis of calcium balance in patients under dialysis for kidney failure. The aim of this work is to value calcium needs in 20 hemodialysed patients who underwent parathyroidectomy, in the department of nephrology of UHC Ibn Rochd of Casablanca from January 1994 to June 1999. These patients, 12 women (60%) and 8 men (40%), aged between 14 and 70 years (mean=46.10+/-13.62 years). Hungry bone syndrome was noted in 8 patients and postoperative hypocalcemia in 15 (75%). Mean minimal serum calcium was 196+/-0.21 mmol/l, with clinical signs in 6 patients. Mean calcium supplement the first postoperative week was 18.1+/-0,54 g/day in the 8 patients with hungry bone syndrome and 14.28+/-0,86 g/day in the 12 remaining patients. Between 6 and 18 months postoperatively, required calcium supplementation was 4.5 to 12 g/day in patients with hungry bone syndrome compared with 3 to 6g/day at the remaining patients. Mean serum calcium remained stable between 2.16 mmol/l to the 3(rd) month and 2.48 mmol/l to the 36(th) month. Postoperative hypocalcemia remains a major concern after parathyroidectomy requiring massive substitution with calcium and active vitamin D metabolite under close supervision to spare these patients from hypercalcemia resulting from parathyroid dysfunction.