Growth and metabolic disturbances in a patient with total parenteral nutrition: A case of hypercalciuric hypercalcemia

Hypercalciuria is a common side effect during total parenteral nutrition (T PN). We report a patient with long-term TPN, who demonstrated hypercalciuri a, hypercalcemia and growth retardation. The patient is a six-year-old Japa nese girl with Hirschsprung disease (jejunal agangliosis). Jejunostomy was performed at one-month old and since then her nutrition has depended mostly on TPN. When she was 3 years old, continuous TPN was switched to cyclic TP N (on TPN for 11 hrs and off TPN for 13 hrs). The urinary calcium level has been elevated (Ca/Cre ratio, 1.0) since 3 months of age, whereas serum cal cium levels stayed within normal range for a while. The serum calcium level s started to elevate to 12 similar to 13 mg/dl when she was 3 years and 8 m onths old. She showed growth retardation (height SD score was -4.2SD when s he was 5 years and 8 months old) and deteriorated renal tubular function wi th renal glycosuria, elevated beta 2-microglobulin (beta 2-MG) and N-acetyl -beta-D-glucosaminidase. She was referred to our division for the investiga tion and treatment of growth disturbance and Ca metabolism. Her bone age wa s delayed (BA/CA 0.62) and serum IGF-I level was decreased but her GH respo nse to provocation test was normal. Bilateral nephrocalcinosis was revealed by renal echogram and CT scan. By reducing calcium content in TPN solution , the serum and urinary calcium levels could be maintained within normal ra nge and her renal function and growth velocity was improved.