Ronald M. Ferdman

Prolonged Morbidity Due to Delays in the Diagnosis and Treatment of Obstructive Sleep Apnea in Children

The inclusion of a query concerning the presence of snoring in a questionnaire used by the Allergy Service of Childrens Hospital Los Angeles (CHLA) uncovered a significant number of patients who were experiencing prolonged and discomforting symptoms owing to previously undiagnosed obstructive sleep apnea (OSA) caused by adenotonsillar hypertrophy. Of 352 patients who were discharged with a diagnosis of OSA and tonsillectomy and/or adenoidectomy at CHLA in 1996-1997, a retrospective study of the first 45 randomly selected patients who agreed to participate in a telephone interview was performed. Analysis revealed that all patients experienced severe and discomforting symptoms with all describing severe or moderate snoring. Other symptoms included chronic mouth breathing (84%), frequent otitis media (64%), sinusitis (56%), sore throat (51%), choking (47%), and daytime drowsiness (42%). Other symptoms included poor school performnce, enuresis, poor appetite and/orxweight gain, dysphagia, and vomiting. Symptoms began at a mean age of approximately 2 years (”birth“-9 years), and the mean period of time between the development of significant symptoms and OSA was 3.3 years (6 months-1 3 years). Delay between onset of significant symptoms and surgery was>1 year in 82% of the patients,>2 years in 51% of the patients,>4 years in 31% of the patients, and>6 years in 13% of the patients. Forty percent of patients were self-referred to an otolaryngologist for treatment despite their primary care physician being aware of the symptoms. These results indicate that patient with OSA experienced prolonged morbidity and delays in treatment, which is probably widespread. Physican, parent, and third-party factors were found to have contributed to the delays in treatment.

Association of Staphylococcal Superantigen-Specific Immunoglobulin E with Mild and Moderate Atopic Dermatitis

OBJECTIVE To examine the frequency of allergic sensitization to staphylococcal superantigens in young children with mild to moderate atopic dermatitis (AD). STUDY DESIGN AD severity was assessed with objective Scoring AD. Serum IgE to staphylococcal enterotoxin (SE) A, SEB, SEC, SED, and toxic shock syndrome toxin-1 were measured with ImmunoCAP. Comparisons between mild AD and moderate AD were performed by using logistic regressions. RESULTS The prevalence of allergic sensitization to staphylococcal superantigens in patients with mild and moderate AD was 38% and 63%, respectively. Allergic sensitization to staphylococcal superantigens, particularly SEA and SED, was found to be associated with moderate AD, compared with mild AD. CONCLUSIONS Our results suggest that allergic sensitization to staphylococcal superantigens is common even in young children with mild to moderate AD, and such sensitization may contribute to the disease severity of these patients.

Late-onset of IgE sensitization to microbial allergens in young children with atopic dermatitis.

Background  A significant proportion of young children with atopic dermatitis (AD) is sensitized to microbial allergens, which play a potential role in the pathogenesis of AD inflammation.

Rodent allergen in Los Angeles inner city homes of children with asthma

Recent studies have examined the presence of mouse allergen in inner city children with asthma. Researchers have found high levels of rodent allergen in homes sampled in the northeast and midwest United States, but there has been considerable variation between cities, and there have been few studies conducted in western states. We evaluated the frequency of rodent sightings and detectable mouse allergen and the housing conditions associated with these outcomes in inner city homes in Los Angeles. Two hundred and two families of school children, ages 6–16 living in inner city neighborhoods, participated in the study. Families were predominantly Latino (94%), and Spanish speaking (92%). At study entry, parents completed a home assessment questionnaire, and staff conducted a home evaluation and collected kitchen dust, which was analyzed for the presence of mouse allergen. Fifty-one percent of homes had detectable allergen in kitchen dust. All 33 families who reported the presence of rodents had detectable allergen in the home and were also more likely to have increased levels of allergen compared to those who did not report rodents. Unwashed dishes or food crumbs, lack of a working vacuum, and a caretaker report of a smoker in the home were all significantly associated with a greater risk of rodent sightings or detectable allergen (P < 0.05). Detached homes were significantly more likely to have detectable allergen. The prevalence of allergen is common enough that it may have public health implications for asthmatic children, and detectable allergen was not routinely identified based on rodent sightings. Many of the predictors of rodent allergen are amenable to low-cost interventions that can be integrated with other measures to reduce exposure to indoor allergens.

Immunologic and virologic effects of glucocorticoids on human immunodeficiency virus infection in children : a preliminary study

The immune dysfunction in human immunodeficiency virus (HIV) infection is complex and cannot be explained solely on the basis of numerical depletion of T lymphocytes. Inappropriate, uncontrolled activation of the immune system may be involved. In a test of this hypothesis, five HIV-infected children were prospectively treated with prednisone and selected immunologic and virologic indices were analyzed. Subjects had marked T lymphopenia (CD4+ T lymphocytes < 500 cells/ml) and antigenemia (serum p24 antigen > 30 pg/ml) and were free of opportunistic infections. There was a significant drop in serum p24 antigen concentrations from baseline (60.2 +/- 10.1% SEM; P < 0.005) 4 weeks after initiation of prednisone, which returned to baseline concentrations as the prednisone was tapered. Concomitant with this decrease, there was decreased expression of cell surface activation markers (HLA-DR, CD25 (interleukin 2 receptor) and CD26 (Ta-1)) in peripheral T lymphocytes. There was no significant change in either T lymphocyte subset numbers or mitogen and antigen-specific lymphoproliferation. A regulatory dysfunction of the immune system, allowing inappropriate activation of T lymphocytes, may be involved in the pathogenesis of HIV disease, and further studies involving selective immunosuppression in HIV disease are warranted.

Down-regulation of atopic dermatitis-associated serum chemokines by wet-wrap treatment: a pilot study.

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Correlation of a Unique "Los Angeles" Phonospirometry Technique with Peak Expiratory Flows in Children with Asthma

Background. Measurement of peak expiratory flow (PEF) is recommended as part of the assessment of patients with asthma. However, there are multiple barriers in the use of PEF, even for older pediatric patients. Objective. Phonospirometry, as measured by the Los Angeles (LA) technique, was assessed and compared with standard PEF measurements in patients with asymptomatic and symptomatic asthma. Methods. A convenience sample of patients with asthma aged 8–17 years was enrolled from visits in the Allergy/Immunology Clinic and in the Emergency Department of Children’s Hospital Los Angeles. The phonospirometry technique was demonstrated, and the length of time the patient repeated the syllable “lah” continuously with the same breath was measured. After a brief interval of time to recover, the patient performed conventional PEF measurement. Results. Using the first observation for each patient in our study, the Pearson correlation coefficient between phonospirometry and PEF was r = 0.67, p = .0016 for asymptomatic asthma patients and r = 0.77, p < .0001 for symptomatic asthma patients. Analysis of the first and last measurements of the symptomatic asthma patients who had multiple measurements revealed a Pearson correlation coefficient between phonospirometry and PEF at first measurement r = 0.69, p = .0008 and at the last measurement r = 0.76, p < .0001. Conclusions. Using the LA technique, phonospirometry was shown to have a linear correlation with PEF in pediatric patients with asymptomatic and symptomatic asthma. It is simple and easily reproducible, as well as cross-cultural. This novel technique shows promise to aid the assessment of patients with acute asthma exacerbations.

Disseminated Mycobacterium kansasii Disease in Complete DiGeorge Syndrome

PurposeComplete DiGeorge syndrome (cDGS) describes a subset of patients with DiGeorge syndrome that have thymic aplasia, and thus are at risk for severe opportunistic infections. Patients with cDGS and mycobacterial infection have not previously been described. We present this case to illustrate that patients with cDGS are at risk for nontuberculous mycobacterial infections and to discuss further antimicrobial prophylaxis prior to thymic transplantation.MethodsA 13-month old male was identified as T cell deficient by the T cell receptor excision circle (TREC) assay on newborn screening, and was subsequently confirmed to have cDGS. He presented with fever and cough, and was treated for chronic aspiration pneumonia as well as Pneumocystis jirovecii infection without significant improvement. It was only after biopsy of mediastinal lymph nodes seen on CT that the diagnosis of disseminated Mycobacterium kansasii was made. We reviewed the literature regarding atypical mycobacterial infections and prophylaxis used in other immunocompromised patients, as well as the current data regarding cDGS detection through TREC newborn screening.ResultsMultiple cases of cDGS have been diagnosed via TREC newborn screening, however this is the first patient with cDGS and disseminated mycobacterial infection to be reported in literature. Thymic transplantation is the definitive treatment of choice for cDGS. Prophylaxis with either clarithromycin or azithromycin has been shown to reduce mycobacterial infections in children with advanced human immunodeficiency virus infection.ConclusionsChildren with cDGS should receive thymic transplantion as soon as possible, but prior to this are at risk for nontuberculous mycobacterial infections. Severe, opportunistic infections may require invasive testing for diagnosis in patients with cDGS. Antimicrobial prophylaxis should be considered to prevent disseminated mycobacterial infection in these patients.