Morbidity characteristics of patients with congenital insensitivity to pain with anhidrosis (CIPA).

Abstract

BACKGROUND:

Congenital insensitivity to pain with anhidrosis (CIPA) is a congenital autonomic sensory neuropathy. In southern Israel, there are many patients with this disease. We here tried to characterize the different infections acquired by children with CIPA.

METHODS:

We collected all the available data about CIPA patients in southern Israel in the year 1991-2005, including the lesion types, area in the body where the infection occurs, and the treatment given.

RESULTS:

The current study included 30 children with CIPA, out of 44 known CIPA patients in southern Israel (68.2%). A total of 382 different episodes of infections, fever, orthopedic lesions, and jaw and mouth lesions led our patients to our outpatient clinic or resulted in hospitalization.

CONCLUSION:

We found that children with CIPA mainly have infections of the skin and skeleton, and that the most frequent pathogen is Staphylococcus aureus. We also found that a fair amount of these pathogens are resistant to conventional treatment regimens.

• Authors: Yariv Fruchtman, Zvi H Perry, Jacov Levy
• Journal: Journal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) Vol. 26 Issue 3-4 Pg. 325-32 ( 2013) ISSN: 0334-018X [Print] Germany
• PMID: 23744299 (Publication Type: Journal Article)

Topics

• Adolescent
• Arthritis, Infectious (epidemiology)
• Bacterial Infections (epidemiology)
• Child
• Child, Preschool
• Female
• Fever (epidemiology)
• Hereditary Sensory and Autonomic Neuropathies (epidemiology)
• Humans
• Infant
• Infant, Newborn
• Israel (epidemiology)
• Male
• Morbidity
• Self-Injurious Behavior (epidemiology)
• Skin Ulcer (epidemiology)
• Staphylococcal Infections (epidemiology)
• Stomatognathic Diseases (epidemiology)
• Wound Healing