Clinical analysis and outcome of interstitial lung disease complicated with juvenile dermatomyositis and juvenile polymyositis.

Objectives: The aim of this study was to determine the clinical phenotype and outcome of interstitial lung disease (ILD) complicated with juvenile dermatomyositis (JDM) or juvenile polymyositis (JPM).

Methods: This was a single-center retrospective study. From 1984 to 2015, we retrospectively reviewed 29 patients who were diagnosed with JDM/JPM, among whom eight cases were ILD and 21 were non-ILD.

Extensively hydrolyzed formula (MA-mi) induced exacerbation of food protein-induced enterocolitis syndrome (FPIES) in a male infant.

Background: Food protein-induced enterocolitis syndrome (FPIES) is a severe, cell-mediated food allergy in which digestive symptoms such as severe vomiting and diarrhea are induced by cow's milk and/or soy protein in infants. Generally, a food-specific IgE is not detected, and FPIES may be caused by inadvertent exposure to allergenic foods.

Case Summary: The patient in our case was a male infant in whom vomiting had been induced by ingestion of a cow's milk-based formula and bloody diarrhea had been caused by ingestion of breast milk during the neonatal period.

Juvenile dermatomyositis: clinical characteristics and the relatively high risk of interstitial lung disease.

To clarify the clinical features of juvenile dermatomyositis (JDM) in Japanese cases, we retrospectively evaluated the characteristics of 16 children with JDM that were treated at Saitama Children's Medical Center between 1985 and 2004. The age at disease onset ranged from 3.5 to 14.

[Thalidomide therapy for infantile-onset Crohn's disease].

A 6-month-old boy was diagnosed as having Crohn's disease (CD) by the endoscopic examination. Primary immunodeficiency syndrome was initially suspected due to a refractory infection that occurred just after birth and a family history that his older brother died at the age of 3 months of septicemia associated with perirectal abscess. Thalidomide was used because conventional medical treatment by steroids and immunosuppressives was ineffective.

[A case of Williams syndrome with p47-phox-deficient chronic granulomatous disease].

A 2-month-old boy with a characteristic elfin face was diagnosed as having Williams syndrome by means of specific fluorescence in situ hybridization (FISH) analysis for a chromosomal microdeletion located in 7q11.23. He was suspected to have immunodeficiency because of a persistent enlargement of axillary lymphnodes after immunization with Bacille Calmette-Guerin (BCG) vaccine since 7 month-old of age.