Abdominal symptoms of hereditary angioedema and early weaning

European Journal of Clinical Nutrition (2010) 64, 10251027

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SHORT COMMUNICATION

Abdominal symptoms of hereditary angioedema and early weaning

Z Kelemen1, B Visy2, D Csuka1, I Czaller1, G Fst1 and H Farkas1

1Faculty of Medicine, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary and 2Heim Pl Pediatric Hospital, Budapest, Hungary

Hereditary angioedema (HAE), a condition caused by deficiency of C1 inhibitor that results in acute and painful swelling in locations that can include the face, neck, abdomen, extremities and genitals, is a potentially life-threatening disorder. Many factors may contribute to phenotype development. A case report prompted us to investigate the potential influence of early weaning on HAE gastrointestinal symptoms. Retrospective analysis was performed based on clinical data from 89 patients registered with our HAE center, including duration of breast-feeding, timing of cows milk introduction, age at symptom onset and localization of the attacks. We did not find any relationship between these factors. Although breastfeeding is known to confer protection against numerous diseases, it showed no efficacy against the manifestations of HAE in our patient population. European Journal of Clinical Nutrition (2010) 64, 10251027 doi:http://dx.doi.org/10.1038/ejcn.2010.134

Web End =10.1038/ejcn.2010.134 ; published online 14 July 2010

Keywords: hereditary angioedema; breast-feeding; early weaning; cows milk

Introduction

Hereditary angioedema (HAE), a condition resulting from congenital C1 inhibitor deficiency, is an autosomal dominant disorder characterized by recurrent, self-limiting episodes of edema formation in the subcutaneous tissues and/or submucosa (Agostoni et al., 2004). Three plasma cascade systems are activated in HAE, a process resulting in the release of bradykininthe vasoactive mediator responsible for the fluid extravasation that causes the swelling seen in HAE patients (Cugno et al., 2009). Edema of the larynx can cause upper airway obstruction leading to suffocation without adequate treatment (Bork et al., 2000). Submucosal edema can develop in the wall of the gastrointestinal tract and produce a range of gastrointestinal symptoms that include nausea, vomiting, colicky abdominal pain, watery post-attack diarrhea or mimic an acute abdomen (Farkas et al., 2001; Bork et al., 2006). Abdominal attack can be the first and only manifestation of the disease (Farkas et al., 2007). It is critical to recognize abdominal HAE attacks because without appropriate diagnosis these patients will