Inderpal Randhawa

Clinical efficacy of omalizumab in an elderly veteran population with severe asthma.

Severe asthma in elderly patients is underdiagnosed, difficult to treat, and often accompanied by atopy. This study was designed to compare clinical outcomes of omalizumab therapy in an elderly veteran population with severe allergic asthma. A retrospective, observational data analysis was performed over 2 years. Cohort outcome measures 1 year before omalizumab therapy were compared with 1 year of active treatment. Statistical analysis included two sample t-tests. The total number of patients enrolled was 17 with median age of 60 years. Omalizumab therapy was associated with a significant reduction in acute asthma exacerbations requiring prednisone treatment (p < 0.01), a significant improvement in forced expiratory volume in 1 second of 0.28 L (p < 0.01), and significantly higher Asthma Control Test (ACT) scores at 3 (p = 0.043), 6 (p = 0.039), and 12 months of therapy (p < 0.01). Two of five patients on daily prednisone for >6 months were able to discontinue systemic steroid use within 3 months of omalizumab treatment. Our study suggests elderly patients with severe atopic asthma show a significant positive clinical response to omalizumab.

Clinical control in the dual diagnosis of obstructive sleep apnea syndrome and rhinitis: a prospective analysis.

Background Obstructive sleep apnea syndrome (OSAS) and allergic rhinitis (AR) are common coexisting disorders. Upper airway, specifically al resistance, is thought to increase during exacerbations of AR and nonallergic rhinitis (NAR), as well as in OSAS. The study objective was to determine if a correlation exists between clinical control of rhinitis and OSAS. Methods This prospective study followed 43 patients with concurrent OSAS and AR or NAR. OSAS was diagnosed by polysomnography, and AR or NAR was diagnosed by history, skin testing, serum-specific IgE, and total IgE levels. Measurements of control of OSAS included the Epworth Sleepiness Scale (ESS) survey and compliance with continuous positive airway pressure (CPAP) device. Measurements of rhinitis control included Assessment of Nasal Symptom Severity and Assessment of Nonnasal Symptom Severity (NSS refers to both) and Global Assessment of Nasal and Nonnasal Symptom Severity surveys (GSS). Higher NSS scores correlate with more rhinitis symptoms, whereas higher GSS scores correlate with less symptoms. Results All patients completed the study. There was a positive correlation between ESS and NSS scores (p < 0.001), inverse correlation between ESS and GSS scores (p < 0.001), inverse correlation between CPAP compliance and NSS scores (p < 0.001), and positive correlation between CPAP compliance and GSS scores (p < 0.001). There was no statistically significant difference between the AR, NAR, and AR/NAR groups. Conclusion Our study showed a statistically significant positive correlation between clinical control of rhinitis symptoms and clinical control of OSAS. This study emphasizes the importance of achieving concurrent optimal control of both OSAS and AR/NAR.

Diagnosis of cystic fibrosis in the kindred of an infant with CFTR-related metabolic syndrome: Importance of follow-up that includes monitoring sweat chloride concentrations over time

Newly implemented newborn screening (NBS) programs in California have resulted in a large subset of patients in whom at least two cystic fibrosis transmembrane conductance regulator (CFTR) mutations are identified, but subsequent sweat chloride analysis reveals normal or indeterminate values. These patients are diagnosed with CFTR‐Related Metabolic Syndrome (CRMS). However, the natural progression and management of these patients are not clearly understood and frequently after the age of 1‐year these patients are lost to follow‐up with Cystic Fibrosis (CF) Centers. We present the first case of an infant who was referred to Miller Childrens Hospital for a NBS positive for CF and subsequent discovery of identical mutations in six of his seven older brothers. Several siblings had positive sweat chloride results on repeat testing after the age of 3 years. We suggest the need for continued follow‐up of CRMS in a CF center with diagnostic evaluation including repeat sweat chloride testing, beyond the currently recommended period. Pediatr Pulmonol. 2014; 49:E103–E108.

Clinical significance of respiratory isolates for Mycobacterium abscessus complex from pediatric patients

Mycobacterium abscessus complex is the most virulent of rapidly growing mycobacteria causing invasive lung disease. To better delineate clinical pediatric experience and outcomes with M. abscessus complex, we retrospectively gathered 5‐year data on M. abscessus complex infection and outcomes in a large, hospital‐based pediatric pulmonary center. Patients were selected from the database of the microbiology department at Miller Childrens Hospital in Long Beach, CA. Patients had at least one positive pulmonary isolate for M. abscessus complex from February 2006 to May 2011. Treatment modality data were collected and successful therapy of disease was determined as clearance of M. abscessus complex infection after antibiotics proven by culture negative respiratory isolate within at least 12 months of therapy initiation. Two cystic fibrosis patients with M. abscessus complex were identified, one with failed therapy and the other with stable pulmonary status despite persistent isolation. One primary ciliary dyskinesia patient had successful clearance of M. abscessus complex, however is now growing M. avium intracellulare. A patient with no prior medical history was successfully treated with antimycobacterial therapy. Eleven patients with neuromuscular disorders had tracheal aspirates positive for M. abscessus complex. None were treated due to stable lung status and all but two had spontaneous clearance of the mycobacteria. The two remaining persist with sporadic isolation of M. abscessus complex without clinical significance. We concluded that patients with tracheostomy associated M. abscessus complex infections do not appear to require treatment and often have spontaneous resolution. Cystic fibrosis or primary ciliary dyskinesia patients may have clinical disease warranting treatment, but current antimycobacterial therapy has not proven to be completely successful. As M. abscessus complex gains prevalence, standardized guidelines for diagnosis and therapy are needed in the pediatric population. Multicenter cohort analysis is necessary to achieve such guidelines. Pediatr Pulmonol. 2013; 48:470–480.

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

AbstractPurposeThe pulmonary complications of sickle cell disease (SCD) are a leading cause of morbidity and mortality (MacLean et al. Am J Respir Crit Care Med 178:1055–1059, 2008; Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; National Heart, Lung, and Blood Institute, 2009). Despite this recognition, predictive markers of lung dysfunction progression remain elusive (Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; Platt et al. N Engl J Med 330:1639–1644, 1994; Caboot et al. Curr Opin Pediatr 20:279–287, 2008; Field et al. Am J Hematol 83:574–576, 2008; Shirlo et al. Peadiatr Respir Review 12:78–82, 2011). This study was designed describe the longitudinal progression and identify specific markers that influence bronchial disease in SCD.MethodsA retrospective, chart review of 89 patients with SCD was conducted. All patients underwent spirometry in conjunction with body plethysmography as part of routine care. Eleven lung function variables were assessed, five of which were selected to establish patterns of normal, obstructive, restrictive, or mixed obstructive-restrictive physiology (Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; Field et al. Am J Hematol 83:574–576, 2008).ResultsIn the unadjusted model, forced expiratory volume in one second (FEV1)% of predicted trended downward with age, while total lung capacity (TLC)% of predicted showed a bimodal distribution and carbon monoxide diffusion capacity corrected for hemoglobin (DLCOcor)% of predicted remained stable. Adjusting for acute chest syndrome (ACS) episodes, medication status, and growth velocity (GV), the final model demonstrated that the downward trend between FEV1% of predicted with age was further influenced by the latter two factors.ConclusionsInitial decline in FEV1% of predicted is associated with worsening pulmonary dysfunction over time. Independent of ACS episodes, the factors most influential on the progression of FEV1% predicted include the introduction of medications as well as the promotion of adequate prepubertal growth. Efforts to ensure normal prepubertal GV and treatment with bronchodilators, such as short-acting beta2 agonists and inhaled corticosteroids (ICS), should be considered at an early age to delay progression of pulmonary dysfunction.

Wiskott-Aldrich Syndrome: Description of a New Gene Mutation With Normal Platelet Volume.

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency characterized by an increased incidence of autoimmunity, malignancy, microthrombocytes with thrombocytopenia, eczema, and recurrent infections. In this case report, we present a novel mutation, hemizygous for c.1125_1129delTGGAC mutation in the WAS gene, and a unique clinical presentation. Our patient was initially diagnosed with a milk protein allergy after presenting with a lower gastrointestinal bleed, leukopenia, and thrombocytopenia with normal platelet volume. However, signs of vasculitis and detection of microthrombocytes required additional testing and consideration of WAS. This case report illustrates the importance of retaining a high index of clinical suspicion despite normal platelet volume, as well as adding to the growing number of known mutations associated with WAS.

Pediatric fiberoptic bronchoscopy as adjunctive therapy in acute asthma with respiratory failure.

Status asthmaticus respiratory failure is associated with thickened mucus secretions necessitating aggressive pulmonary clearance. The role of bronchoscopy in pediatric mechanically ventilated asthmatic patients has not been published.

Antidepressants in chronic idiopathic urticaria.

Chronic idiopathic urticaria (CIU) is a common disease estimated to affect 0.1% of the population and can be very difficult to treat. Many psychotropic medications have been reported to be successful in treating refractory CIU. The purpose of this article was to discuss the pathophysiology of chronic urticaria and provide practicing allergists and dermatologists alternative treatment options in the management of refractory CIU, especially in those who have concurrent psychiatric comorbidity. A review was performed of pertinent literature pertaining to the pathophysiology of CIU and the many psychotropic medications reportedly successful in disease management. Although more research is needed, this article serves to broaden the mind of the physician treating CIU.

The persistent thrombus: complications, diagnosis, and novel treatment intervention.

Objective: To review the findings and discuss the implications of the topic of pharmacomechanical thrombolysis in pediatric patients with persistent thrombus. Design: A pediatric case presentation with a brief literature review on treatment of venous thrombosis and pharmacomechanical thrombolysis. Interventions: None. Main Results: Thrombotic events refractory to standard medical and surgical care remain a life-threatening clinical challenge in the pediatric population. Research on persistent deep venous thrombosis and treatment modalities is limited. We present a pediatric patient with a history of malignant osteosarcoma who was diagnosed with deep venous thrombosis. Despite appropriate anticoagulation therapy, the thrombus remained persistent. Pharmacomechanical thrombolysis was utilized and proved to be an effective method in providing diagnosis and treatment. Conclusion: Pharmacomechanical thrombolysis is a valuable and effective method in providing diagnosis and treatment of persistent thrombus.

Properdin deficiency–associated bronchiectasis

The complement system plays a critical role in host innate immureveal lipid laden alveolar macrophages, making aspiration unnity against microorganisms. Properdin, a basic glycoprotein of 442 amino acids, is a key component of the alternative complement pathway.1,2 Properdin binds to C3 convertase, resulting in amplification and stabilization of the C3bBb complex and thus allowing for alternative pathway activation.3 Properdindeficiency is characterized byNeisseriameningococcal infections that present in the second decade of life.4 A prior report also described a case of recurrent otitis media and pneumonia due to nonmeningococcal disease in children.5 Properdin deficiency associated with chronic infections has not been reported in adults. We report a case of a 44-year-old white womanwhowas evaluated for recurrent sinopulmonary infections and bronchiectasis. The patient presented with a history significant for recurrent sinopulmonary infections as a child. Four to 5 years before presentation, she was noted to have severe pneumonias associated with sepsis, requiring prolonged and recurrent hospitalizations. She had multiple positive sputum culture results for Pseudomonas, Aspergillus, Fusarium species, and Stenotrophomonas. She required recurrent antibiotic and antifungal treatments. Two to 3 years before presentation, she developed bronchiectasis, with significant decreases in pulmonary function, and became oxygen dependent. At the time of presentation, the patient’s pulmonary function test revealed the following: forced vital capacity, 2.78 (74%); forced expiratory volume in 1 second,1.38 (47%); ratio of forced expiratory volume in 1 second to forced vital capacity, 50; forced expiratory flow between 25% and 75%, 0.62 L (19%). She was empirically being treated with long-term posaconazole for Aspergillus and aerosolized tobramycin for Pseudomonas. Her remaining medical history was significant for gastroesophageal reflux disease and hypothyroidism. There was no known family history of immunodeficiency and recurrent infections. She did not smoke and had no known secondhand smoking exposure. She did not have history of dextrocardia or sinus diseases. Physical examination revealed diffuse wheezing on both expiration and inspiration and crackles at the bilateral lung bases. At the time of presentation, our patient had already been evaluated at an outside hospital with a normal cystic fibrosis genetic panel, sweat chloride test, complete blood cell count, basic metabolic studies, and a1-antitrypsin measurement. Her initial workup included high-resolution computed tomography of the chest, which revealed mild bronchiectasis noted in lingula and bilateral lower lung lobes, ground glass opacities, and tree in bud nodules in the left upper lung lobe and scatter micronodules in the lower lung lobes (Fig 1). Bronchoscopy findings were unremarkable. On the basis of distribution of her bronchiectasis, clinical concern was for either aspiration or possible immunodeficiency. Nuclear medicine aspiration scan result was negative, and bronchoscopy did not