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Persistent Indoor Air Pollution Levels in the Homes of New York City Children over 4 to 5 years
AB94 Abstracts
351
SUNDAY
Increasing IgM Associated with Severe Autoimmunity and Lymphoproliferation in Common Variable Immunodeficiency C. S. Patel, D. F. LaRosa; University of Pennsylvania School of Medicine, Philadelphia, PA. RATIONALE: Data from the European Common Variable Immunodeficiency (CVID) Disorders registry reveals IgM at presentation correlates with polyclonal lymphoproliferation and lymphoid malignancy. Here we report two patients with CVID complicated by lymphoproliferation and autoimmunity that demonstrate marked, steroid-responsive increases in IgM paralleling disease activity. METHODS: Clinical data over 8-years follow-up was reviewed. As the basis for CVID diagnosis, patients at presentation were severely deficient in IgG and IgA and unresponsive to pneumovax. RESULTS: Both patients are women. The first presented at age 30 with sino-pulmonary infections, thrombocytopenia, and IgM of 104 mg/dl. Two years later, she developed symptomatic granulomatous-lymphocytic interstitial lung disease (GLILD) and IgM increased to 1,040 mg/dl. Her symptoms improved with steroids and IgM decreased to 300-range. Subsequently, flaring of GLILD was associated with IgM elevations, peaking at 1,600 mg/dl, and improvement in symptoms with immunosuppression associated with IgM decreases, with nadir of 235 mg/dl. This patient also suffered two posterior circulation strokes with CNS angiitis suspected from angiography. The second patient presented at age 41 with a history of sinus infections, GLILD, thrombocytopenia, and IgM of 97 mg/dl. Similarly, flaring of GLILD was associated with IgM elevations, peaking at 1,240 mg/dl, and improvement associated with IgM decreases. After 8 years of follow-up, this patient developed severe, persistent autoimmune neutropenia and has been maintained on immunosuppression and G-CSF. In both, there has been no evidence of a paraprotein or clonal lymphocyte population in blood or tissue. CONCLUSION: In the CVID autoimmune/lymphoproliferative clinical phenotype, increasing IgM may be a sign of worsening disease.
Novel L670P Mutation In The Stat3 Gene Discovered In A 44 Year Old Male With Job's Syndrome G. D. Marshall, S. A. Bozeman; University of Mississippi, Jackson, MS. RATIONALE: Hyper-immunoglobulin E (IgE) syndromes are rare primary immune deficiencies that are characterized by an elevated IgE, dermatitis, and recurrent skin and lung infections. There are 2 forms; the dominant form, also associated with skeletal, connective tissue, cardiac, and brain abnormalities, is diagnosed by a mutation in the STAT3 gene but no genetic associations for the recessive form have been established. Although the diagnosis of Job’s syndrome is usually made in childhood, we report a 44 year old newly diagnosed with Job’s syndrome by demonstrating the above characteristics and a novel L670P missense mutation in the STAT3 gene. METHODS: Medical records were extensively reviewed for this patient and the L670P mutation in the STAT3 gene was discovered by PCR-amplification for analysis of exons 12-16 and exons 20-21 of the STAT3 gene and their flanking splice sites by GeneDx. RESULTS: The patient presented with a multiyear history of recurrent skin abscesses (cultures positive for Methicillin-resistant Staphylococcus aureus), long standing eczema, septic arthritis, and pulmonary aspergillosis. Laboratory studies showed persistently elevated IgE levels (3940-4580 IU/ml) and eosinophilia with several values over 1, 000 K/mL. Chest computed tomography revealed patchy ground glass opacities, cystic bronchiectatic changes, and multiple thick walled cysts. Given the clinical presentation, he was referred for further immune workup where peripheral blood was sent for gene analysis. CONCLUSIONS: We believe this case is significant in the fact that this man was not diagnosed with Job’s syndrome until adulthood and patient was confirmed to be heterozygous for a novel L670P mutation in the STAT3 gene.
352
J ALLERGY CLIN IMMUNOL FEBRUARY 2011
353
Association Between Asthma-related Emergency Department Visits, Asthma-related Hospital Admissions, And Airborne Pollen And Mold Concentrations In The Bronx, 2001-2008 S. P. Jariwala, J. Toh, A. Geevarghese, S. Reddy, D. Rosenstreich; Albert Einstein/Montefiore Medical Center, Bronx, NY. OBJECTIVE: To examine the relationship between asthma-related emergency department visits (AREDV), asthma-related hospitalizations (ARH), and the concentrations of several pollen types and mold in a high asthma prevalence area, the New York City borough of the Bronx. METHODS: The numbers of daily adult and pediatric AREDV and ARH from 2001-2008 were obtained from two Bronx hospitals (MontefioreMoses and Montefiore-Weiler). AREDV and ARH data were acquired through the Clinical Looking Glass data analysis software, which allowed a retrospective search of asthma-related patients by ICD-9 code. Daily counts for tree, grass, and weed pollen and mold spores from 3/2001 to 10/2008 were obtained from the Armonk counting station located in close proximity to the Bronx. All data were statistically analyzed and graphed as daily values. RESULTS: From 2001-2008, there were a total of 42,065 AREDV and 10,132 ARH at the two hospitals. AREDV showed three distinct peaks of increased visits - in January, May, and November. For most of the years studied, the spring peak was most prominent and consistently overlapped with high tree pollen levels. There were no distinct peaks of ARH nor identifiable associations with AREDV numbers. Weed peaks were observed in August-September, but did not consistently correlate with AREDVor ARH numbers. Neither grass nor mold spores demonstrated consistent peaks or associations with AREDV or ARH. CONCLUSIONS: There is a large, spring increase in AREDV in the Bronx that closely correlates with high tree pollen counts. Weed, grass pollen, and mold spore peaks are likely not strongly associated with AREDV peaks or ARH in this region.
354
Persistent Indoor Air Pollution Levels in the Homes of New York City Children over 4 to 5 years J. M. Camacho1, S. Hsu1, K. H. Jung1, K. M. Moors1, K. J. Bernabe1, P. L. Kinney2, S. N. Chillrud3, R. Whyatt2, L. A. Hoepner2, J. M. Ross3, F. Perera2, R. L. Miller1,2; 1Division of Pulmonary, Allergy and Critical Care of Medicine, Department of Medicine, College of Physicians and Surgeons, Columbia University, PH8E, 630 W. 168 St. New York, New York 10032, New York, NY, 2Mailman School of Public Health, Department of Environmental Health Sciences, Columbia University, 60 Haven Ave., B-1 New York, New York 10032, New York, NY, 3Lamont-Doherty Earth Observatory, Columbia University, 61 Rt, 9W Palisades, New York 10964, New York, NY. RATIONALE: Exposure to traffic-related air pollution is associated with adverse health effects. Due to continued government programs to reduce emissions through retrofitting or replacing diesel engines with cleaner technologies, we hypothesized that exposure of children to traffic-related pollution in Northern Manhattan and South Bronx homes may have decreased over time. METHODS: Under the auspices of the Columbia Center for Children’s Environmental Health (CCCEH), residential exposure to particulate matter (PM2.5) and black carbon (BC) over two weeks were measured for children (n562) who did not move, at ages 5-6 and again at ages 9-10 years from 2005 to 2010. Data collected six months apart at each age were averaged. RESULTS: Average levels of PM2.5 did not decrease (18.29 1/-9.57 standard deviation (SD) at age 5-6 versus 18.31 1/- 15.95 SD ug/m3 at age 9-10 years). Average levels of the calculated absorbance coefficient (BC indicator) also did not decrease (0.93 1/- 0.29 SD at age 5-6 versus 0.92 1/- 0.30 SD at age 9-10 years m-1* 10-5). Results remained the same after controlling for borough, second hand smoke exposure, heat season, ethnicity. CONCLUSIONS: On a cohort level, exposure to PM2.5 and BC persist for urban children at ages 5-6 and 9-10 years. Future studies will look at the effects of exposure to these pollutants on respiratory symptoms among the cohort children. Current results suggest that children continue to be exposed to traffic-related air pollution in NYC and additional public policy measures may be needed to reduce exposure.
351
SUNDAY
Increasing IgM Associated with Severe Autoimmunity and Lymphoproliferation in Common Variable Immunodeficiency C. S. Patel, D. F. LaRosa; University of Pennsylvania School of Medicine, Philadelphia, PA. RATIONALE: Data from the European Common Variable Immunodeficiency (CVID) Disorders registry reveals IgM at presentation correlates with polyclonal lymphoproliferation and lymphoid malignancy. Here we report two patients with CVID complicated by lymphoproliferation and autoimmunity that demonstrate marked, steroid-responsive increases in IgM paralleling disease activity. METHODS: Clinical data over 8-years follow-up was reviewed. As the basis for CVID diagnosis, patients at presentation were severely deficient in IgG and IgA and unresponsive to pneumovax. RESULTS: Both patients are women. The first presented at age 30 with sino-pulmonary infections, thrombocytopenia, and IgM of 104 mg/dl. Two years later, she developed symptomatic granulomatous-lymphocytic interstitial lung disease (GLILD) and IgM increased to 1,040 mg/dl. Her symptoms improved with steroids and IgM decreased to 300-range. Subsequently, flaring of GLILD was associated with IgM elevations, peaking at 1,600 mg/dl, and improvement in symptoms with immunosuppression associated with IgM decreases, with nadir of 235 mg/dl. This patient also suffered two posterior circulation strokes with CNS angiitis suspected from angiography. The second patient presented at age 41 with a history of sinus infections, GLILD, thrombocytopenia, and IgM of 97 mg/dl. Similarly, flaring of GLILD was associated with IgM elevations, peaking at 1,240 mg/dl, and improvement associated with IgM decreases. After 8 years of follow-up, this patient developed severe, persistent autoimmune neutropenia and has been maintained on immunosuppression and G-CSF. In both, there has been no evidence of a paraprotein or clonal lymphocyte population in blood or tissue. CONCLUSION: In the CVID autoimmune/lymphoproliferative clinical phenotype, increasing IgM may be a sign of worsening disease.
Novel L670P Mutation In The Stat3 Gene Discovered In A 44 Year Old Male With Job's Syndrome G. D. Marshall, S. A. Bozeman; University of Mississippi, Jackson, MS. RATIONALE: Hyper-immunoglobulin E (IgE) syndromes are rare primary immune deficiencies that are characterized by an elevated IgE, dermatitis, and recurrent skin and lung infections. There are 2 forms; the dominant form, also associated with skeletal, connective tissue, cardiac, and brain abnormalities, is diagnosed by a mutation in the STAT3 gene but no genetic associations for the recessive form have been established. Although the diagnosis of Job’s syndrome is usually made in childhood, we report a 44 year old newly diagnosed with Job’s syndrome by demonstrating the above characteristics and a novel L670P missense mutation in the STAT3 gene. METHODS: Medical records were extensively reviewed for this patient and the L670P mutation in the STAT3 gene was discovered by PCR-amplification for analysis of exons 12-16 and exons 20-21 of the STAT3 gene and their flanking splice sites by GeneDx. RESULTS: The patient presented with a multiyear history of recurrent skin abscesses (cultures positive for Methicillin-resistant Staphylococcus aureus), long standing eczema, septic arthritis, and pulmonary aspergillosis. Laboratory studies showed persistently elevated IgE levels (3940-4580 IU/ml) and eosinophilia with several values over 1, 000 K/mL. Chest computed tomography revealed patchy ground glass opacities, cystic bronchiectatic changes, and multiple thick walled cysts. Given the clinical presentation, he was referred for further immune workup where peripheral blood was sent for gene analysis. CONCLUSIONS: We believe this case is significant in the fact that this man was not diagnosed with Job’s syndrome until adulthood and patient was confirmed to be heterozygous for a novel L670P mutation in the STAT3 gene.
352
J ALLERGY CLIN IMMUNOL FEBRUARY 2011
353
Association Between Asthma-related Emergency Department Visits, Asthma-related Hospital Admissions, And Airborne Pollen And Mold Concentrations In The Bronx, 2001-2008 S. P. Jariwala, J. Toh, A. Geevarghese, S. Reddy, D. Rosenstreich; Albert Einstein/Montefiore Medical Center, Bronx, NY. OBJECTIVE: To examine the relationship between asthma-related emergency department visits (AREDV), asthma-related hospitalizations (ARH), and the concentrations of several pollen types and mold in a high asthma prevalence area, the New York City borough of the Bronx. METHODS: The numbers of daily adult and pediatric AREDV and ARH from 2001-2008 were obtained from two Bronx hospitals (MontefioreMoses and Montefiore-Weiler). AREDV and ARH data were acquired through the Clinical Looking Glass data analysis software, which allowed a retrospective search of asthma-related patients by ICD-9 code. Daily counts for tree, grass, and weed pollen and mold spores from 3/2001 to 10/2008 were obtained from the Armonk counting station located in close proximity to the Bronx. All data were statistically analyzed and graphed as daily values. RESULTS: From 2001-2008, there were a total of 42,065 AREDV and 10,132 ARH at the two hospitals. AREDV showed three distinct peaks of increased visits - in January, May, and November. For most of the years studied, the spring peak was most prominent and consistently overlapped with high tree pollen levels. There were no distinct peaks of ARH nor identifiable associations with AREDV numbers. Weed peaks were observed in August-September, but did not consistently correlate with AREDVor ARH numbers. Neither grass nor mold spores demonstrated consistent peaks or associations with AREDV or ARH. CONCLUSIONS: There is a large, spring increase in AREDV in the Bronx that closely correlates with high tree pollen counts. Weed, grass pollen, and mold spore peaks are likely not strongly associated with AREDV peaks or ARH in this region.
354
Persistent Indoor Air Pollution Levels in the Homes of New York City Children over 4 to 5 years J. M. Camacho1, S. Hsu1, K. H. Jung1, K. M. Moors1, K. J. Bernabe1, P. L. Kinney2, S. N. Chillrud3, R. Whyatt2, L. A. Hoepner2, J. M. Ross3, F. Perera2, R. L. Miller1,2; 1Division of Pulmonary, Allergy and Critical Care of Medicine, Department of Medicine, College of Physicians and Surgeons, Columbia University, PH8E, 630 W. 168 St. New York, New York 10032, New York, NY, 2Mailman School of Public Health, Department of Environmental Health Sciences, Columbia University, 60 Haven Ave., B-1 New York, New York 10032, New York, NY, 3Lamont-Doherty Earth Observatory, Columbia University, 61 Rt, 9W Palisades, New York 10964, New York, NY. RATIONALE: Exposure to traffic-related air pollution is associated with adverse health effects. Due to continued government programs to reduce emissions through retrofitting or replacing diesel engines with cleaner technologies, we hypothesized that exposure of children to traffic-related pollution in Northern Manhattan and South Bronx homes may have decreased over time. METHODS: Under the auspices of the Columbia Center for Children’s Environmental Health (CCCEH), residential exposure to particulate matter (PM2.5) and black carbon (BC) over two weeks were measured for children (n562) who did not move, at ages 5-6 and again at ages 9-10 years from 2005 to 2010. Data collected six months apart at each age were averaged. RESULTS: Average levels of PM2.5 did not decrease (18.29 1/-9.57 standard deviation (SD) at age 5-6 versus 18.31 1/- 15.95 SD ug/m3 at age 9-10 years). Average levels of the calculated absorbance coefficient (BC indicator) also did not decrease (0.93 1/- 0.29 SD at age 5-6 versus 0.92 1/- 0.30 SD at age 9-10 years m-1* 10-5). Results remained the same after controlling for borough, second hand smoke exposure, heat season, ethnicity. CONCLUSIONS: On a cohort level, exposure to PM2.5 and BC persist for urban children at ages 5-6 and 9-10 years. Future studies will look at the effects of exposure to these pollutants on respiratory symptoms among the cohort children. Current results suggest that children continue to be exposed to traffic-related air pollution in NYC and additional public policy measures may be needed to reduce exposure.