Endocrine Abstracts (2006) 11 P144

Prolonged hungry bone syndrome in a patient with wolfram syndrome: a case report

Okcan Basat1, Sema Ucak1, Ahsen Bas Oncul2, Selcuk Seber1, Nezake Eren3 & Yuksel Altuntas1

1Sisli Etfal Research and Training Hospital, Diabetes Endocrinology and Metabolism, Istanbul, Turkey; 2Sisli Etfal Research and Training Hospital, Infection Clinic, Istanbul, Turkey; 3Sisli Etfal Research and Training Hospital, Biochemistry, Istanbul, Turkey.

Hungry bone syndrome (HBS) is associated with recent parathyroid surgery. The Wolfram syndrome is characterised by diabetes insipidus, diabetes mellitus, optic atrophy and deafness. Other anomalies, such as renal outflow tracts and multiple neurological disorders may develop later. We present a Wolfram patient with tertiary hyperparathyroidism due to chronic renal failure associated with bilateral reterohidronephrosis who developed prolonged HBS after parathyroid adenomectomy.

A 21 year-old women with Type-1 DM, diabetic polineurophaty, chronic renal failure due to neurological vesica, bilateral hydroureteronephrosis and single parathyroid adenomectomy 3 months ago was hospitalized for glucose regulation. Calcium concentration was 7.7 mg/dl. She was taking 1 μg oral calcitriol, 4000 mg calcium daily. After five days she developed hypocalcaemia symptoms. Serum calcium level decreased to 6.1 mg/dl. Oral calcitriol dosage was elevated up to 7 μg gradually, calcium concentration decreased to 4.8 mg/dl. Continuous intravenous calcium was initiated, 1500 mg Ca2+daily. Intravenous calcium was necessary for 2 months. After operation serum alkaline phosphatase levels increased up to 1614 IU/l, but gradually decreased to 795 IU/l 2 months later. Fundoscopic examination revealed bilateral optic atrophy. Voiding urography and renal ultrasonography revealed hydroureteronephrosis. Urodynamics revealed low capacity, low compliance bladder, emptying problem. Audiometry demonstrated bilateral sensoryneural hearing loss. Diagnosis of Wolfram syndrome was made. We report a patient with chronic renal failure that caused prolonged HBS lasting for 6 months postoperatively. Current case was diagnosed with diabetes mellitus, neurogenic bladder, and bilateral hydroureteronephrosis at the age of 12. Since she did not have diabetic retinopathy in ophthalmic examinations, her chronic renal failure was taught to be associated with recurrent pyelonephritis. She developed bilateral optic atrophy. To our knowledge this is the first case with Wolfram syndrome and prolonged hungry bone syndrome following surgery for tertiary hyperparathyroidism are coexisting.

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