[Autopsy case of child with severe motor and intellectual disabilities showing hyper IgE subsequent to food allergy].
We presented an autopsy case with severe motor and intellectual disabilities (SMID) who showed a severe increase in the serum level of IgE. Although the patient had rarely been exposed to food allergens, the serum level of IgE continued increasing. He developed a high titer of serum IgE against specific antigens to which he had never been exposed. There was no mutation of the STAT3 gene, and no abnormalities in immunological functions including serum levels of cytokines. Death was from a hemorrhage caused by a tracheoinnominate artery fistula. At autopsy, the submucosal area of the colon presented with an increase in the number of mast cells immunoreactive for tryptase, which could have been involved with hyper IgE. Since SMID patients tend to be sensitized by food allergens subsequent to recurrent gastroesophageal reflux, we should pay attention to complications of food allergy even when using an elemental diet.
Miwa
Naho
N
Department of Pediatrics, Tokyo Metropolitan Fuchu Medical Center for the Disabled, Fuchu, Tokyo. Naho_Miwa@member.metro.tokyo.jp
Tanuma
Naoyuki
N
Hayashi
Masaharu
M
D000293:Adolescent; D001344:Autopsy; D016122:Brachiocephalic Trunk; D003106:Colon / Q000166:cytology / Q000473:pathology; D019988:Children with Disabilities; D017809:Fatal Outcome; D005512:Food Hypersensitivity / Q000209:etiology* / Q000473:pathology*; D005764:Gastroesophageal Reflux / Q000150:complications*; D006470:Hemorrhage; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology* / Q000473:pathology*; D007073:Immunoglobulin E; D008607:Intellectual Disability / Q000150:complications*; D007413:Intestinal Mucosa / Q000166:cytology / Q000473:pathology; D008297:Male; D008407:Mast Cells / Q000473:pathology*; D012720:Severity of Illness Index; D014133:Tracheal Diseases; D016157:Vascular Fistula
D007073:Immunoglobulin E