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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e3182400e8b
Letter to the Editor

Zinc and Copper Deficiency in the Microvillus Inclusion Disease

Diamanti, Antonella; Basso, Maria S.; Candusso, Manila; Panetta, Fabio; Gambarara, Manuela

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Hepatology, Gastroenterology and Nutrition Unit, Children's Hospital “Bambino Gesù,” Rome, Italy

To the Editor:

We read with great interest the article by Halac et al (1) about the clinical management of microvillus inclusion disease (MID). They reported the main clinical complications observed over time in MID as growth failure, developmental delay, osteoporosis, and liver and kidney disease. We would like to emphasize the high prevalence in these patients of trace element deficiencies. We have studied 5 patients with MID in the last 20 years. Four patients developed trace element deficiencies, despite standard parenteral supplementation and additional zinc (2). Three of them presented with acrodermatitis enteropathica–like rash. Serum zinc level was in all of the cases under the normal range (66–110 μg/100 mL). Serum zinc level normalized after further zinc intravenous supplementation, and rash improved in all 3 patients. One patient developed macrocytic anemia and neutropenia that did not respond to combined iron and parenteral vitamin B12 supplementation. Bone marrow examination revealed findings considered suggestive of copper deficiency (3). Serum copper level was 10 μg/100 mL (normal range 75–145 μg/100 mL). After supplementation, copper level normalized and anemia and neutropenia resolved.

In our experience, patients with MID seem to be at high risk of developing zinc and copper deficiency, likely because of the loss of gastrointestinal fluids high in zinc and copper content. We recommend close monitoring of serum zinc and copper (twice yearly).

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REFERENCES

1. Halac U, Lacaille F, Joly F, et al. Microvillous inclusion disease: how to improve the prognosis of a severe congenital enterocyte disorder. J Pediatr Gastroenterol Nutr 2011; 52:460–465.

2. ESPGHAN. Iron, minerals and trace elements. J Pediatr Gastroenterol Nutr 2005;41:S39–46.

3. Kumar N. Copper deficiency myelopathy (human swayback). Mayo Clin Proc 2006; 81:1371–1384.

Copyright 2012 by ESPGHAN and NASPGHAN

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