Growth characteristics of children with ectodermal dysplasia syndromes.

OBJECTIVE: Clinical observations suggested that growth abnormalities may be present in children with ectodermal dysplasia (ED) syndromes. This study characterizes the longitudinal pattern of growth in a cohort of children with the ED syndromes. We hypothesized that (1) linear and ponderal growth abnormalities are present in children with ED from infancy through adolescence, and (2) linear and ponderal growth abnormalities differ among the clinical variants of these disorders. METHODS: We studied 138 children who had ED and were registered with the National Foundation for Ectodermal Dysplasias, 74% of whom had clinical features consistent with the hypohidrotic EDs (HEDs). Height (or length) and weight measurements were obtained by standardized techniques and from review of available medical records. We converted these measurements to weight-for-height (children younger than 5 years and <103 cm in length) or BMI (children > or =2 years old). Height, weight, weight-for-height, and BMI were converted to age- and gender-specific z scores. We applied linear regression, 1-sample t tests, and analysis of variance to detect linear and ponderal growth abnormalities in children with ED compared with a reference population. RESULTS: Mean weight-for-age, weight-for-height, and BMI-for-age z scores but not height-for-age z score, were significantly lower in children with the ED syndromes than in the reference population. Mean weight-for-age and weight-for-height z scores but not BMI-for-age or height-for-age z scores increased significantly with increasing age. The mean height-for-age z score of children with the ED syndromes other than the HEDs was significantly lower than that of children with the HEDs. CONCLUSIONS: Growth abnormalities, measured as weight deficits, were present at an early age in children with the ED syndromes and persisted through adolescence. Height deficits were seen only in children with ED syndromes other than HEDs. Clinicians should evaluate carefully children with ED syndromes for growth abnormalities.

Rice-induced enterocolitis in an infant: TH1/TH2 cellular hypersensitivity and absent IgE reactivity.

BACKGROUND: Although food allergy is common in children, rice allergy is unusual in Western cultures. OBJECTIVE: To report a case of T-cell-mediated rice intolerance in an 11-month-old girl. METHODS: To evaluate the intolerance to rice in this patient, a graded rice food challenge was performed. To examine the immunologic reactivity to rice, in vitro lymphoproliferative responses and cytokine synthesis of rice-stimulated peripheral blood lymphocytes (PBLs) was performed. Subsequently, skin patch testing to rice and other foods was performed. RESULTS: Allergy skin prick test results were negative for rice and positive for egg, milk, and soy. Specific IgE antibodies to rice, egg, peanut, wheat, walnut, codfish, milk, soybean, corn, shrimp, scallops, and clams were undetectable. Results of a single-blind rice food challenge were positive, manifested by emesis that persisted for more than an hour and required intravenous hydration. In vitro lymphoproliferation by the patient's PBLs to rice stimulation was positive. In addition, cytokine synthesis of interferon-gamma, interleukin 10 (IL-10), tumor necrosis factor a, and IL-5 by the patient's rice-stimulated PBLs was elevated, indicating a TH1/TH2 cell response to rice. Endoscopy revealed normal esophageal, gastric, and duodenal mucosa; a biopsy specimen revealed mild esophagitis. Duodenal explant T cells were initially established by stimulation with rice and IL-2. After a 2-day rest, the lymphocytes were restimulated with rice for 7 days and revealed increased interferon-gamma and IL-5 synthesis. Twenty billion colony forming units of Lactobacillus GG were added to the patient's diet twice daily. After 6 weeks, rice rechallenge resulted in emesis within 1 hour. Results of patch testing were positive to rice, wheat, and barley but negative to soy, which the patient tolerated on food challenge. CONCLUSIONS: Although this patient did not demonstrate IgE antibody to rice, TH1/TH2 cell-mediated responses to rice were detected, and the patient experienced significant morbidity. Patch testing for gastrointestinal food allergies may be useful when the food specific IgE antibody is negative. Probiotic therapy in this patient did not ameliorate her sensitivity to rice, and food elimination remains the only reliable treatment for TH1/TH2-mediated food hypersensitivity.