Dinesh Raj

Childhood Obesity and Risk of Allergy or Asthma

The simultaneous increment in the prevalence of obesity and allergic diseases suggests a possible link between them. This review focuses on the consequences of obesity on allergic diseases, especially asthma in children and adolescents, and evaluates the available evidence on the possible mechanisms. Obesity is related more strongly to nonatopic than atopic asthma, suggesting non-eosinophilic inflammation and Th1 polarization. Among other allergic diseases, the association is more consistent with eczema compared to allergic rhinitis/rhinoconjunctivitis. The mechanisms of asthma in obese individuals could involve mechanical effects of obesity on lung function, adipokines-mediated inflammation, shared factors (diet, genetics, sedentary lifestyle) and comorbidities.

Prevalence and risk factors for allergic bronchopulmonary aspergillosis in Indian children with cystic fibrosis

ObjectivesAllergic bronchopulmonary aspergillosis (ABPA) is a common complication in patients with cystic fibrosis. This crosssectional study was planned to determine the prevalence and risk factors for ABPA in Indian children with cystic fibrosis.MethodsClinical evaluation, spirometry, chest radiograph, sputum, total IgE, specific IgE for Aspergillus fumigatus, IgG precipitins and skin prick tests were done in 33 CF patients.ResultsPrevalence of allergic bronchopulmonary aspergillosis was 18.2% (95% CI 6.9%–35.4%): allergic bronchopulmonary aspergillosis was higher in patients with low cystic fibrosis score, age >12 years, atopy, and eosinophilia.ConclusionPrevalence of ABPA is higher in Indian children with cystic fibrosis.

Pulmonary alveolar proteinosis secondary to Pneumocystis jiroveci infection in an infant with common variable immunodeficiency.

The authors report an infant with common variable immunodeficiency (CVID) with Pneumocystis pneumonia who developed secondary pulmonary alveolar proteinosis (PAP). This is the youngest infant reported to develop PAP secondary to Pneumocystis infection in an immunocompromised state. He was effectively managed with anti-microbials, frequent lung toilet, optimized mechanical ventilation, and supportive care.

Fractional exhaled nitric oxide for identification of uncontrolled asthma in children

ObjectivesTo determine the utility of Fractional Exhaled Nitric Oxide (FENO) in the identification of uncontrolled asthma in children on therapy, and to identify its cut-off value for determining asthma control.Methods207 children (age 5–15 y) with physician-diagnosed asthma on therapy with at least 12 months follow up were enrolled. Spirometry and FENO measurements were performed. Asthma control was assessed as per GINA guidelines. Sensitivity and specificity of various cut-off values of FENO (15 ppb, 20 ppb, 25 ppb, 30 ppb) for identification of status of control of asthma were calculated.Results156 (75%) children had uncontrolled or partly controlled asthma and 51 children were assessed to have controlled asthma. Median (IQR) FENO in children with controlled and uncontrolled asthma was 16 (11–23) ppb and 13 (11–25) ppb, respectively (P=0.26). No FENO cut-off had a reasonable combination of sensitivity and specificity to discriminate between controlled and uncontrolled asthma.ConclusionFENO, in itself, does not have good discriminatory value in assessment of controlled and uncontrolled asthma in children on asthma therapy.

Fractional exhaled nitric oxide in children with acute exacerbation of asthma

ObjectiveTo determine whether fractional exhaled nitric oxide (FENO) has a utility as a diagnostic or predictive maker in acute exacerbations of asthma in children.DesignAnalysis of data collected in a pediatric asthma cohort.SettingPediatric Chest Clinic of a tertiary care hospitalMethodsA cohort of children with asthma was followed up every 3 months in addition to any acute exacerbation visits. Pulmonary function tests (PFT) and FENO were obtained at all visits. We compared the FENO values during acute exacerbations with those at baseline and those during the follow up.Results243 asthmatic children were enrolled from August 2009 to December 2011 [mean (SD) follow up — 434 (227) days]. FENO during acute exacerbations was not different from FENO during follow up; however, FENO was significantly higher than personal best FENO during follow up (P < 0.0001). FENO during acute exacerbation did not correlate with the severity of acute exacerbation (P=0.29). The receiver operating characteristics curve for FENO as a marker for acute exacerbation had an area under the curve of 0.59. Cut-off of 20 ppb had a poor sensitivity (44%) and specificity (68.7%) for acute exacerbation.ConclusionsFENO levels during acute exacerbation increase from their personal best levels. However, no particular cut off could be identified that could help in either diagnosing acute exacerbation or predicting its severity.

Aeroallergen sensitization in childhood asthmatics in Northern India

AbstractTo determine the prevalence of sensitization to common aeroallergens in asthmatic children and study the differences in characteristics of atopics and non atopics.DesignAnalysis of data from a prospective cohort study.SettingPediatric Chest Clinic of tertiary care center in Northern IndiaPatientsAsthmatic children from 5–18 year of age.Main outcome measuresPrevalence of sensitization to common aeroallergens.ResultsSkin prick testing (SPT) was performed on 180 children above 5 years of age, with a mean (SD) age of 111.4 (34.2) months. 100 children (55.6%) were sensitized to at least one aeroallergen, suggesting atopy; 68 (37.8%) were sensitized to more than one allergen. 36.7% children were sensitized to housefly antigen; 31.1% to rice grain dust, 18.3% to cockroach, and 7.8% to house dust mite antigens. Atopic children had significantly higher median FENO during follow up than nonatopic children (17.5 ppb vs 13 ppb, P=0.002). There was a positive correlation between age and the number of allergens that an individual was sensitized to (r= 0.21; P=0.0049).ConclusionsMore than half of asthmatic children in our cohort had sensitization to one or more aeroallergens suggesting atopy; sensitization was most commonly seen to housefly antigen and rice grain dust. Atopic children had significantly higher FENO measurements during follow up as compared to non-atopic children.

Correlation between impulse oscillometry and spirometry parameters in Indian patients with cystic fibrosis.

Impulse oscillometry (IOS) is an emerging tool to assess lung function in chronic respiratory diseases, more often in preschool children and patients who are unable to perform spirometry. We conducted a prospective cross-sectional study on patients with cystic fibrosis (CF). Primary objective was to evaluate correlation between IOS and spirometry parameters. Secondary objective was to evaluate the ability of IOS parameters to discriminate patients with airflow limitation at various forced expiratory volume in 1 second (FEV1) cutoffs. Patients with CF above 6 years of age, who were following up on a routine visit, were enrolled in the study. Patients underwent IOS and spirometry as per guidelines. A total of 39 patients (34 children and 5 adults) were enrolled in the study. There was a significant moderate negative correlation between spirometry parameters (FEV1, forced vital capacity, and peak expiratory flow rate) and IOS parameters, that is, impedance at 5 Hz (Z5), resistance at 5 Hz (R5), and reactance area, both between raw values and percent predicted values. Of the various IOS parameters, Z5 percent predicted had the maximum area under the curve (AUC) of 0.8152 and 0.8448 for identifying children with FEV1 <60% and <80%, respectively. R5 percent predicted had an AUC of 0.8185 for identifying children with FEV1 <40%. IOS can be used as an alternative pulmonary function test in patients with CF more so in patients who are unable to perform spirometry.

Biochemical and MRI findings of Kallmann's syndrome

Kallmanns syndrome is a neuronal migration disorder characterised by anosmia/hyposmia and hypogonadotropic hypogonadism. We present a case of a 21-year-old man who was unable to sense smell since birth and who displayed non-development of secondary sexual characteristics for the past 10 years. Blood investigations showed low basal levels of serum follicle stimulating hormone (FSH), serum luteinising hormone (LH) and serum testosterone. After a gonadotropin releasing hormone challenge test there was a slight increase in serum FSH and serum LH, and after a human chorionic gonadotropin (HCG) challenge test the patients serum testosterone level increased to 34 times that of his basal level. MRI of the brain showed absence of bilateral olfactory bulbs and sulcus with an apparently normal appearing pituitary gland, and bilateral loss of distinction between the gyrus rectus and medial orbital gyrus, thus confirming the diagnosis. The patient is on treatment with injection of HCG 2000 IU deep intramuscular twice a week and is on follow-up.

Family History of Early Infant Death Correlates with Earlier Age at Diagnosis But Not Shorter Time to Diagnosis for Severe Combined Immunodeficiency

Background Severe combined immunodeficiency (SCID) is fatal unless treated with hematopoietic stem cell transplant. Delay in diagnosis is common without newborn screening. Family history of infant death due to infection or known SCID (FH) has been associated with earlier diagnosis. Objective The aim of this study was to identify the clinical features that affect age at diagnosis (AD) and time to the diagnosis of SCID. Methods From 2005 to 2016, 147 SCID patients were referred to the Asian Primary Immunodeficiency Network. Patients with genetic diagnosis, age at presentation (AP), and AD were selected for study. Results A total of 88 different SCID gene mutations were identified in 94 patients, including 49 IL2RG mutations, 12 RAG1 mutations, 8 RAG2 mutations, 7 JAK3 mutations, 4 DCLRE1C mutations, 4 IL7R mutations, 2 RFXANK mutations, and 2 ADA mutations. A total of 29 mutations were previously unreported. Eighty-three of the 94 patients fulfilled the selection criteria. Their median AD was 4 months, and the time to diagnosis was 2 months. The commonest SCID was X-linked (n = 57). A total of 29 patients had a positive FH. Candidiasis (n = 27) and bacillus Calmette–Guérin (BCG) vaccine infection (n = 19) were the commonest infections. The median age for candidiasis and BCG infection documented were 3 months and 4 months, respectively. The median absolute lymphocyte count (ALC) was 1.05 × 109/L with over 88% patients below 3 × 109/L. Positive FH was associated with earlier AP by 1 month (p = 0.002) and diagnosis by 2 months (p = 0.008), but not shorter time to diagnosis (p = 0.494). Candidiasis was associated with later AD by 2 months (p = 0.008) and longer time to diagnosis by 0.55 months (p = 0.003). BCG infections were not associated with age or time to diagnosis. Conclusion FH was useful to aid earlier diagnosis but was overlooked by clinicians and not by parents. Similarly, typical clinical features of SCID were not recognized by clinicians to shorten the time to diagnosis. We suggest that lymphocyte subset should be performed for any infant with one or more of the following four clinical features: FH, candidiasis, BCG infections, and ALC below 3 × 109/L.

Nevus lipomatosus cutaneous superficialis resembling rudimentary male external genitalia in a neonate

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare developmental abnormality characterized by group of ectopic fat cells dispersed in collagen bundles of the papillary dermis. Herein we present a case of congeniatal solitary NLCS in the perianal region of a female neonate presented with features of pseudo hermaphrodism which was ruled out by routine investigation. Simple surgical exicision was done and histological examination was suggestive of NLCS.