Predictors of low bone density and fracture risk in Loeys-Dietz syndrome.

PURPOSE: Loeys-Dietz syndrome (LDS) is a connective tissue disorder affecting multiple organ systems, including bone. METHODS: We defined the bone phenotype and clinical predictors of low bone density and fracture risk in 77 patients with LDS type 1 to type 5. RESULTS: Patients with LDS had dual-energy x-ray absorptiometry (DXA) Z-scores significantly < 0, and 50% of children and 9% of adults had Z-scores < -2. Sixty percent of patients had ≥1 fracture, and 24% of patients with spinal x-rays scans showed spinal compression fractures. Lower body mass index, asthma, male sex and eosinophilic gastrointestinal disease were correlated with lower DXA Z-scores. The count of 5 LDS-associated skeletal features (scoliosis, pes planus, arachnodactyly, spondylolisthesis, and camptodactyly) in patients with LDS was correlated with DXA Z-score. Adults with ≥1 skeletal features had DXA Z-scores significantly < 0, and children with >2 features had DXA Z-score significantly < -2. Bone turnover markers suggest accelerated bone resorption. Data from 5 patients treated with bisphosphonates suggest a beneficial effect. CONCLUSION: All LDS types are associated with reduced bone density and increased risk of fracture, which may be due to increased bone resorption. Clinical features can predict a subgroup of patients at highest risk of low bone density and fracture risk.

IgE testing can predict food allergy status in patients with moderate to severe atopic dermatitis.

BACKGROUND: Diagnosing food allergy in patients with atopic dermatitis (AD) is complicated by their high rate of asymptomatic sensitization to foods, which can lead to misdiagnosis and unnecessary food avoidance. OBJECTIVE: We sought to determine whether food-specific (sIgE) or component immunoglobulin (Ig) E levels could predict allergic status in patients with moderate to severe AD and elevated total IgE. METHODS: Seventy-eight children (median age, 10.7 years) with moderate to severe AD were assessed for a history of clinical reactivity to milk, egg, peanut, wheat, and soy. The IgE levels for each food and its components were determined by ImmunoCAP. The level and pattern of IgE reactivity to each food and its components, and their ratio to total IgE, were compared between subjects who were allergic and tolerant to each food. RESULTS: Ninety-one percent of subjects were sensitized, and 51% reported allergic reactivity to at least 1 of the 5 most common food allergens. Allergy to milk, egg, and peanut were most common, and IgE levels to each of these foods were significantly higher in the allergic group. Component IgEs most associated with milk, egg, and peanut allergy were Bos d8, Gal d1, and Ara h2, respectively. The ratio of sIgE to total IgE offered no advantage to sIgE alone in predicting allergy. CONCLUSION: Specific IgE levels and the pattern of IgE reactivity to food components can distinguish AD subjects allergic vs tolerant to the major food allergens and may therefore be helpful in guiding the clinical management of these patients.