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Pathological Case of the Month

Russell Chesney, MD; Aaron Friedman, MD; Enid Gilbert-Barness, MD
Am J Dis Child. 1992;146(2):255-256. doi:10.1001/archpedi.1992.02160140121033.
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This 12-year-old girl had normal growth and development until age 5 years, when lethargy, nausea, vomiting, and pallor due to anemia developed. She had the features of uremia, with foul breath and facial edema. Laboratory values were as follows: serum calcium, 1.47 mmol/L; serum potassium, 5.9 mmol/L; and blood urea nitrogen, 71 mmol/L. Extensive nephrocalcinosis was evident on abdominal roentgenograms. The patient's height was 107 cm at age 5 years and failed to increase during the next 7 years (Fig 1). After 6 months of hemodialysis, the patient underwent a bilateral renal transplant. After a minor fall, a transverse fracture of the right femur at the level of the lesser trochanter (Fig 2) occurred. She was immobilized in a hip-spica cast, and her serum calcium level increased to 3.3 mmol/L. Her parathyroid hormone level was 11 mmol/L (in the normal range). A parathyroidectomy was performed in which three parathyroid glands


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