Idiopathic T cell lymphopenia identified in New York State Newborn Screening.

Quantification of T-cell receptor excision circles (TRECs) for newborn screening for SCID has advanced the diagnosis of severe combined immune deficiency (SCID). However, it has led to the identification of infants with T cell lymphopenia without known cause. The clinical characteristics, appropriate laboratory monitoring, and outcomes of patients remain unclear. We performed a retrospective review of clinical and laboratory studies for 26 infants collected from 7 New York State referral centers from 2010 to 2016 with low TRECs (mean, 70copies/µl) and subnormal CD3 counts (mean, 1150/cubicmm). Over time absolute CD3 counts increased in 17 and decreased in 9; 22 (85%) have done well clinically regardless of absolute T cell values. Additional infants with TCL will continue to be identified in newborn screening panels. While most patients seem to do well clinically, parameters for diagnosis and monitoring have yet to be formalized, and additional information needs to be collected, causes and outcomes reported.

Growth comparison in children with and without food allergies in 2 different demographic populations.

OBJECTIVE: To examine the effects of food avoidance on the growth of children with food allergies. STUDY DESIGN: A retrospective chart review was performed for children with and without food allergies followed at 2 New York City general pediatric practices. Charts were selected based on codes from the International Classification of Diseases, 9th Revision, for well child visit, food allergy, anaphylaxis, and/or epinephrine autoinjector prescriptions. Heights and weights were obtained to calculate body mass index, height, and weight z-scores. RESULTS: Of the 9938 children seen, 439 (4.4%) were avoiding one or more foods. Of those with commercial insurance, children with food allergies were significantly shorter (mean height z-score = 0.06; P = .01) and weighed less (mean weight z-score -0.1; P = .006) than children without food allergies (mean height z-score = 0.42; mean weight z-score = 0.07). In contrast, children with food allergies and state insurance were not smaller in height or weight compared with children without food allergies. Among white subjects, there was a significant effect of food allergies on height and weight (ANOVA for height P = .012, for weight P = .0036) that was not observed for Hispanic/Latino, black, or Asian subjects. Children with allergies to milk weighed significantly less than children without milk allergies (P = .0006). CONCLUSIONS: Children with food allergies and commercial insurance have significant impairment in growth compared with those without food allergies. Additionally, children avoiding all forms of milk are shorter and weigh less than matched counterparts. Therefore, height and weight measurements should be assessed routinely in children with food allergies because there is risk for growth impairment in this population.

Phellinus tropicalis abscesses in a patient with chronic granulomatous disease.

Chronic Granulomatous Disease (CGD), caused by genetic defects in components of the phagocyte NADPH oxidase pathway, leads to recurrent life-threatening bacterial and invasive fungal infections. While a number of unique pathogens have been associated with this disease, the causative organisms may be difficult to identify. Here, we present a 24 year old male with known X-linked CGD who concurrently developed a cervical abscess and an abscess in the subcutaneous tissues of the right hip, both of which were surgically drained. Cultures failed to identify any organisms. He was treated empirically with ertapenem but the hip abscess recurred at the original site and in contiguous dependent areas in the posterior thigh and knee. A filamentous organism was observed microscopically, initially considered a contaminant, but on culture yielded a mold growth, identified as Phellinus tropicalis (synonym: Inonotus tropicalis) based on phenotypic and molecular methods. This is the third case report of human infection with P. tropicalis, all in subjects with CGD. The patient was treated with voriconazole with resolution of his symptoms.

Growth and nutritional concerns in children with food allergy.

PURPOSE OF REVIEW: To describe the potential effect that avoidance diets for food allergy may have on nutrition and growth in children. RECENT FINDINGS: We report here the findings from the previous studies suggesting impairment of growth and nutritional deficiencies because of elimination diets for food allergy. Feeding difficulties have also been reported, particularly in children with eosinophilic esophagitis that may further impact the nutrient intake. SUMMARY: Food allergies are becoming more prevalent and better recognized. Treatment options typically include strict dietary elimination of major food allergens such as milk, eggs, wheat, soy, peanut, tree nuts, fish and shellfish. Monitoring growth and guiding food allergic patients in choosing appropriate alternatives to supply necessary nutrients becomes crucial to avoid deficiencies and retardation in growth.