Hypocalcemic Tetany in a 13-Year-Old Girl with Wilson's Disease.
10.5223/kjpgn.2011.14.1.86
- Author:
Chaeik RA
1
;
Sang Yong KIM
;
Hong KOH
Author Information
1. Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea. KHONG@yuhs.ac
- Publication Type:Case Report
- Keywords:
Wilson's disease;
Hypoparathyroidism;
Hypocalcemic tetany;
Vitamin D deficiency
- MeSH:
Adolescent;
Brain;
Calcium;
Copper;
Cornea;
Disease Progression;
Hepatolenticular Degeneration;
Humans;
Hypocalcemia;
Hypoparathyroidism;
Liver;
Magnesium;
Tetany;
Vitamin D;
Vitamin D Deficiency
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
2011;14(1):86-90
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Wilson's disease is an autosomal recessive disorder marked by disruptions in copper metabolism which leads to accumulation of copper in the liver, brain, cornea, and other tissues. Manifestations of this disease are more likely to be hepatic during early childhood and neurologic in adolescent. In addition, abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. Here we report a 13-year-old girl who presented with hypocalcemic tetany shortly after being diagnosed with Wilson's disease. Despite aggressive calcium, magnesium, and vitamin D replacement therapy, the hypocalcemia and hypomagnesemia did not promptly respond. Mineral levels in the blood took longer than 3 weeks to normalize. We speculate that a parathyroid insufficiency and disrupted vitamin D metabolism caused by copper accumulation and hepatic dysfunction resulted in hypocalcemic tetany.
