Delayed separation of the umbilical cord in two siblings with Interleukin-1 receptor-associated kinase 4 deficiency: rapid screening by flow cytometer.

Takada, Hidetoshi; Yoshikawa, Hideto; Imaizumi, Masue; Kitamura, Taro; Takeyama, Junji; Kumaki, Satoru; Nomura, Akihiko; Hara, Toshiro · J Pediatr · 2006

case_report · Level V

Where this comes from

Abstract

We describe 2 siblings who had interleukin-1 receptor-associated kinase 4 deficiency with a novel mutation in exon 2. They had delayed separation of the umbilical cord. The flow cytometric analysis of monocytic intracellular tumor necrosis factor-alpha production in response to lipopolysaccharide may be a useful method to screen for the disease.

Medical subject headings