Spectrum of illness in pediatric common variable immunodeficiency.

BACKGROUND: Common variable immunodeficiency (CVID) may present at any age but usually presents during adulthood. OBJECTIVE: To study the presentation and associated medical conditions found in pediatric patients with CVID. METHODS: A medical record review of patients diagnosed as having CVID before the age of 18 years was performed at a tertiary care immunology clinic from 1992 to 2005. Inclusion criteria consisted of presentation with recurrent infections and decrease in 2 of 3 immunoglobulin isotypes (IgG, IgA, IgM) 2 SDs below the age-specific range, with a poor or absent response to immunization. There had to be no other identifiable predisposing cause of the immunodeficiency. RESULTS: A total of 12 patients were identified. The mean age at presentation was 8 years. All patients had low IgG levels with poor functional antibody responses. The most common presenting infections were sinusitis (75%), otitis media (67%), and pneumonia (58%). Bronchiectasis was seen in 3 children. One patient presented with chronic diarrhea due to Giardia. Two patients presented with failure to thrive. Asthma was seen in 10 patients (83%) but was usually diagnosed after the initial presentation. Autoimmune disorders were seen, including 1 patient with idiopathic thrombocytopenia and 2 with neutropenia. Other disorders encountered were growth hormone deficiency, hypothyroidism, end-stage renal disease, and sarcoma. CONCLUSIONS: CVID is a difficult diagnosis in the pediatric population because of an unpredictable presentation. Autoimmune disease, growth hormone deficiency, renal disease, and cancer were noted in our population. A high incidence of asthma also may be associated with pediatric CVID.

Modern prevalence of insect sensitization in rural asthma and allergic rhinitis patients.

Inhalant allergy to insects other than cockroaches and dust mites was described in the 1950s, but little attention has been paid to these findings. Sensitization rates for many allergens have increased since then. In this rural population, we describe the current prevalence of sensitization to commonly found insects. We evaluated 200 patients (38 adults and 162 children), for immunoglobulin E (IgE)-mediated sensitization to the caddis fly, mayfly, moth, carpenter ant, and housefly using the prick technique and commercially available whole-body extracts. All had symptoms consistent with asthma and/or allergic rhinitis. They also were tested to indoor and outdoor aeroantigens. Of the 200 patients, 60 patients (30%) tested positive to at least one of the five insects. The most prevalent positive skin test was to the mayfly (37/60), followed by the housefly (36/60), caddis fly (27/60), moth (14/60), and ant (10/60). Of the 60 patients positive for insects, 35 patients (58%) also tested positive to at least one species of mite, 36 patients (60%) tested positive to the cockroach. Twelve of the 60 patients (20%) with positive insect testing did not react to either cockroaches or mites. In our patients, the immunoglobulin E-mediated sensitization rate to insects excluding cockroaches is 30%. Many patients also were sensitized to mites and/or cockroaches. These insects are all present in the indoor/outdoor environment of this rural population. Cross-reactivity with mites and cockroaches (insect panallergy) may partially explain our results. The presence of skin test sensitivity in the absence of cockroach or mite allergy suggests possible true insect sensitization.

Mouse allergy among asthmatic children from rural Appalachia.

BACKGROUND: Mice are a common finding in the indoor environment of many homes. In a recent study, 18% of children with asthma from an inner-city environment were reported to be allergic to mouse allergen. OBJECTIVE: To determine the frequency of skin test reactivity among asthmatic children in a rural environment. METHODS: We consecutively evaluated 209 (82 female, 127 male) children between the ages of 5 months and 19 years with asthma for mouse allergy. A careful environmental history was obtained on all children. Children older than 3 years of age were skin tested to mouse allergen and other indoor/outdoor inhalant allergens. Children younger than 3 years were skin tested to mouse and indoor allergens. RESULTS: Thirty-three percent of parents reported seeing mice in their homes. Overall, 25 of 209 (12%) children with asthma were skin test-positive for mouse. For children 3 years or younger, 6 of 52 were skin test-positive for mouse (12%). There was no correlation among socioeconomic status, skin test reactivity, and the presence of mice in the home. Children with multiple skin test reactions were more likely to be reactive to mouse (P < 0.01). Mice seen in the home did not correlate with positive mouse skin tests. CONCLUSIONS: The frequency of skin test reactivity to mouse allergen in asthmatic children from rural areas appears slightly less than that in children from inner-city environments. However, a frequency of 12% suggests that skin testing for this allergen provides useful information for environmental control measures in the home.

Ten-year experience using a plastic, disposable curette for the diagnosis of primary ciliary dyskinesia.

BACKGROUND: Primary ciliary dyskinesia (PCD) results in impaired mucociliary clearance. Patients with this disorder develop chronic sinopulmonary disease with recurrent sinusitis, otitis media, nasal polyposis, pneumonia, and, ultimately, bronchiectasis. Other associated findings of dysfunctional ciliary activity include situs inversus, dextrocardia, and infertility. OBJECTIVE: To describe our 10-year experience using a small, plastic, disposable curette to perform a screening procedure for cilia function and to collect samples for electron microscopy. METHODS: In the past 10 years, we screened infants and children with severe chronic sinusitis and other chronic recurrent upper respiratory tract problems for PCD by using a plastic, disposable curette to collect tissue samples from the nasal mucosa. Samples were placed in sterile saline and examined under light microscopy for the presence of cilia. Failure to note ciliary movement prompted another examination 1 month later. If no functional cilia were noted at the follow-up examination, a specimen was obtained and sent for electron microscopy. RESULTS: We identified 7 patients with PCD; 2 had situs inversus totalis. Average age at diagnosis was 3 years. The most common symptom at presentation was frequent upper respiratory tract infections with severe otitis media (7 patients) and sinusitis (5 patients). Recurrent pneumonia was present in 6 patients. Dynein arm deficiency was the most common electron microscopic diagnosis. CONCLUSIONS: Evaluating children for PCD by using a plastic, disposable curette is a relatively simple procedure that could be used by allergists in practice. Primary ciliary dyskinesia occurs frequently enough that physicians should consider it as part of the differential diagnosis in evaluating children with recurrent, severe sinopulmonary infections.