Abstract
A thirty-two year old woman with primary renal tubular acidosis, severe nephrocalcinosis and reduced glomerular function had bilateral, symmetric pathologic fractures of the femoral heads secondary to atypical osteitis fibrosa generalisata. There was no evidence of concomitant osteomalacia. The clinical, roentgenographic and pathologic features indicated that the hyperparathyroidism was secondary to the renal tubular acidosis. The course of the skeletal disease was monitored by serial metabolic balance measurements, radiocalcium turnover studies, x-ray examinations and bone biopsies. Almost complete reversal of the metabolic bone disease was achieved after subtotal parathyroidectomy and calcium lactate and vitamin D 2 therapy. Nevertheless, glomerular insufficiency progressed and caused death after a total course of between four and ten years. An awareness of atypical cases of renal tubular acidosis can facilitate early diagnosis and effective management, and improve the prognosis in this unusual condition.
Published Version
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