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J Pediatr. 2008 Feb;152(2):276-80. doi: 10.1016/j.jpeds.2007.06.039. Epub 2007 Oct 22.

Cause-specific risks of childhood death in inherited epidermolysis bullosa.

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  • 1National Epidermolysis Bullosa Registry, Nashville, TN, USA.

Abstract

OBJECTIVE:

To determine the cause-specific risks of death in children with epidermolysis bullosa (EB).

STUDY DESIGN:

Data were collected throughout the continental United States between 1986 and 2002 by the National EB Registry. The study design is cross-sectional (n = 3280), containing within it a nested randomly sampled longitudinal subcohort (n = 450).

RESULTS:

The risk of death during infancy and childhood was greatest in junctional EB (JEB), with cumulative and conditional risks of 40% to 44.7% by age 1 in both JEB subtypes, rising to 61.8% in children with JEB, Herlitz subtype and 48.2% in those with JEB, non-Herlitz subtype (JEB-nH) by age 15. In decreasing order, sepsis, failure to thrive, and respiratory failure were the major causes of death in children with JEB, plateauing by age 2 to 6. A small minority of children with epidermolysis bullosa simplex, Dowling-Meara subtype was at risk for death by age 1 (cumulative risk, 2.8%), with sepsis and respiratory failure accounting for cumulative risks of 1.9% and 0.9%. Only a minority of children with recessive dystrophic epidermolysis bullosa, Hallopeau-Siemens subtype was at risk of death (cumulative risk = 8% by age 15). Renal failure also rarely accounted for death in children with JEB-nH.

CONCLUSIONS:

Infants and children with inherited EB, particularly those with JEB, are at significant risk of death as a result of disease complications.

[PubMed - indexed for MEDLINE]
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