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Specific Antibody Deficiency In A Patient With Jacobsen Syndrome And Paris-Trousseau Syndrome
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Specific Antibody Deficiency In A Patient With Jacobsen Syndrome And Paris-Trousseau Syndrome J. C. Rabbat1,2, J. N. Moy1,2; 1Rush University Medical Center, Chicago, IL, 2Stroger Hospital of Cook County, Chicago, IL. RATIONALE: Jacobsen syndrome is a rare 11q terminal deletion disorder characterized by short stature, cognitive deficits, congenital heart disease, thrombocytopenia (Paris-Trousseau syndrome), genitourinary anomalies, psychiatric disorders, ophthalmologic disorders, and recurrent infections. To date, 3 immunodefiencies have been reported among these patients, namely IgM and IgA deficiency, IgM deficiency with T-cell quantitative and qualitative deficiency, and common variable immunodeficiency. We report a patient with Jacobsen syndrome and Paris Trousseau syndrome who was diagnosed with specific antibody deficiency. METHODS: A 10 year-old male with Jacobsen syndrome and a history of failure to thrive presented for chronic sinusitis refractory to adenoidectomy and otitis media refractory to serial tympanostomy tubes. He was given antibiotics as frequently as every 6 weeks and was on prophylactic nasal antibiotics. No invasive or opportunistic infections were diagnosed. RESULTS: In vitro IgE tests to aeroallergens were negative. Serum IgG and IgA were normal; IgM was extremely low. IgG subclasses were normal. Specific antibody testing showed non-protective titers to all 7 serotypes of S. pneumoniae in Prevnar, despite having received the full series of Prevnar as an infant. Four weeks after a booster vaccine with Prevnar, he responded to only 3 serotypes. After another 6 weeks he lost immunity to an additional serotype. CONCLUSIONS: We report the first case of specific antibody deficiency in a patient with Jacobsen syndrome. Our findings suggest that specific antibody deficiency may be a cause for these patients’ recurrent upper respiratory infections.
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Use of MODIS Satellite Images and an Atmospheric Dust Transport Model To Evaluate Juniperus Spp. Pollen Phenology and Dispersal to Support Public Health Alerts J. C. Luvall1, W. Sprigg2, E. Levetin3, A. Huete4, S. Nickovic2, G. Pejanovic2, P. Van de Water5, O. Myers6, A. Budge6, T. Crimmins7, H. Krapfl8, A. Zelicoff9; 1NASA Marshall Space Flight Center, huntsville, AL, 2University of Arizona, Tucson, AZ, 3University of Tulsa, Tulsa, OK, 4University of Arizona, tucson, AZ, 5California State University, Fresno, Fresno, CA, 6University of New Mexico, Albuquerque, NM, 7University of Arizona National Phenology Network, Tucson, AZ, 8New Mexico Department of Health, Albuquerque, NM, 9Ares Corporation, Albuquerque, NM. RATIONALE: Juniperus spp. pollen is a significant aeroallergen and has been observed to be transported 200-600 km from the source. Local observations of Juniperus spp. phenology may not be consistent with the timing and source of pollen collected by pollen sampling instruments. METHODS: The Dust REgional Atmospheric Model (DREAM)is a verified model for atmospheric dust transport modeling using MODIS data products to identify source regions and quantities of dust. The use of satellite data products for studying plant phenology is well documented and is able to identify Juniperus spp. community pollen phenology. We successfully modified the DREAM model to incorporate pollen transport (PREAM) and used MODIS satellite images to identify Juniper forest types during pollen formation and release. RESULTS: MODIS phenology profiles, as measured by the standard vegetation index (VI) product show a fairly strong seasonal signature in the EVI(Enhanced Vegetation Index) measure of vegetation activity through periods of snow, understory green-up and brown-down, and peak periods of vegetation activity. There was also quite strong interannual seasonal variability that are useful in analysis of the pre-conditions for timing and magnitudes of pollen release. The DREAM model was successfully modified to incorporate pollen transport (PREAM). Modeled transport of Juniperus spp. pollen closely matched the peak pollen concentrations in March 2006 for Albuquerque and Los Alamos. CONCLUSIONS: We believe that advanced pollen forecasts will lead to better preparedness for asthma and allergy sufferers, hospital admissions personnel, and workforce managers, pharmaceutical marketing and
distribution functions, and contribute to a reduction in false positive respiratory diagnoses.
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Associations between Traffic-related Exposures and Incidence of Wheeze among Children Age 5-7 in NYC S. Hsu1, K. H. Jung1, K. Moors1, K. Bernabe1, P. L. Kinney2, S. N. Chillrud3, M. S. Perzanowski2, R. M. Whyatt2, L. A. Hoepner2, S. Wang2, B. Yan3, J. M. Ross3, F. Perera2, R. L. Miller1,2; 1Columbia University Department of Medicine, New York City, NY, 2Columbia University Mailman School of Public Health, Columbia Center for Children’s Environmental Health, New York City, NY, 3Lamont-Dohery Earth Observatory, Palisades, NY. RATIONALE: Exposure to traffic-related pollutants has been associated with asthma exacerbations in children. However, evidence linking traffic exposure, particularly diesel, to the development of asthma among young children is limited. We hypothesized that black carbon (BC), absorption coefficient (Abs), and PM2.5 concentrations measured from residences are associated with incident wheeze among children age 5-7 in NYC. METHODS: Two-week residential monitoring of BC via multi-wavelength, Abs via reflectance, and PM2.5 were conducted in 346 children ages 5/6 as part of the longitudinal Columbia Center for Children’s Environmental Health (CCCEH) cohort. Respiratory outcomes were assessed annually by administering International Study of Asthma and Allergies in Childhood questionnaire. Incident wheeze at age 6 or 7 was defined as report of wheeze (according to ‘‘ever’’ or ‘‘last 12-months’’ questions) among children without wheeze at age 5. Logistic regression adjusting for sex, ethnicity, smoking, maternal asthma and cold/flu season were applied to estimate the effects of pollution on incident wheeze. RESULTS: Twenty children (10%) were classified as incident wheeze cases. Exposure to residential BC, Abs, and PM2.5 was associated with excess risks of incident wheeze. The odds ratios (ORs) between incident wheeze, derived from ‘‘ever wheeze’’, and an interquartile range increase in BC, Abs, and PM2.5 were 1.46 (95% CI: 1.00 - 2.13), 2.13 (95% CI: 1.20 - 3.80), and 1.38 (95% CI: 1.01 - 1.88), respectively. Positive but nonsignificant ORs were found when using ‘‘wheeze in the last 12-months’’ to define incident wheeze. CONCLUSIONS: Exposure to traffic-generated pollutants may be associated with incident wheeze among young urban children.
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Juniperus asheii (Mountain Cedar) Pollen Utilized As An Antigen In The Biogenics Chamber: Comparison Of Natural And Controlled Exposures D. A. Ramirez, R. L. Jacobs, C. P. Andrews; Biogenics Research Chamber, San Antonio, TX. RATIONALE: Juniperus asheii pollen allergy is a robust model commonly used in natural studies for pharmacologic efficacy. This study was performed to compare the symptoms elicited by Mountain cedar pollen in the natural season with symptoms in a controlled environment outside of the season. METHODS: Eighteen subjects sensitized to Juniperus asheii were monitored for symptoms during the 2009-2010 season. Symptomatic subjects were then exposed six months later in a controlled environment. Pollen levels of 11,500 grains/m3 were utilized during 2 hour chamber exposures on consecutive days. Total nasal symptom scores (TNSS) and total ocular symptom scores (TOSS) were compared in the natural season to the chamber exposure. TNSS scores of 7 or greater and TOSS scores of 5 or greater were considered meaningful. RESULTS: Up to four priming exposures were required to reach meaningful TNSS symptoms in 61% of subjects. Meaningful TOSS scores were seen in 78%. Chamber runs utilized pollen counts higher than the mean of natural exposure (3,500/m3.) These counts were 50% of maximum pollen counts during the recording interval of the season. CONCLUSIONS: In 2 hr priming chamber runs, Juniperus asheii pollen elicited meaningful symptoms in subjects shown to be symptomatic during the natural season. With consecutive chamber exposures, subjects can be selected for pharmacologic studies utilizing Juniperus asheii pollen in a controlled environment.
SATURDAY
Abstracts AB19
J ALLERGY CLIN IMMUNOL VOLUME 127, NUMBER 2
Specific Antibody Deficiency In A Patient With Jacobsen Syndrome And Paris-Trousseau Syndrome J. C. Rabbat1,2, J. N. Moy1,2; 1Rush University Medical Center, Chicago, IL, 2Stroger Hospital of Cook County, Chicago, IL. RATIONALE: Jacobsen syndrome is a rare 11q terminal deletion disorder characterized by short stature, cognitive deficits, congenital heart disease, thrombocytopenia (Paris-Trousseau syndrome), genitourinary anomalies, psychiatric disorders, ophthalmologic disorders, and recurrent infections. To date, 3 immunodefiencies have been reported among these patients, namely IgM and IgA deficiency, IgM deficiency with T-cell quantitative and qualitative deficiency, and common variable immunodeficiency. We report a patient with Jacobsen syndrome and Paris Trousseau syndrome who was diagnosed with specific antibody deficiency. METHODS: A 10 year-old male with Jacobsen syndrome and a history of failure to thrive presented for chronic sinusitis refractory to adenoidectomy and otitis media refractory to serial tympanostomy tubes. He was given antibiotics as frequently as every 6 weeks and was on prophylactic nasal antibiotics. No invasive or opportunistic infections were diagnosed. RESULTS: In vitro IgE tests to aeroallergens were negative. Serum IgG and IgA were normal; IgM was extremely low. IgG subclasses were normal. Specific antibody testing showed non-protective titers to all 7 serotypes of S. pneumoniae in Prevnar, despite having received the full series of Prevnar as an infant. Four weeks after a booster vaccine with Prevnar, he responded to only 3 serotypes. After another 6 weeks he lost immunity to an additional serotype. CONCLUSIONS: We report the first case of specific antibody deficiency in a patient with Jacobsen syndrome. Our findings suggest that specific antibody deficiency may be a cause for these patients’ recurrent upper respiratory infections.
59
Use of MODIS Satellite Images and an Atmospheric Dust Transport Model To Evaluate Juniperus Spp. Pollen Phenology and Dispersal to Support Public Health Alerts J. C. Luvall1, W. Sprigg2, E. Levetin3, A. Huete4, S. Nickovic2, G. Pejanovic2, P. Van de Water5, O. Myers6, A. Budge6, T. Crimmins7, H. Krapfl8, A. Zelicoff9; 1NASA Marshall Space Flight Center, huntsville, AL, 2University of Arizona, Tucson, AZ, 3University of Tulsa, Tulsa, OK, 4University of Arizona, tucson, AZ, 5California State University, Fresno, Fresno, CA, 6University of New Mexico, Albuquerque, NM, 7University of Arizona National Phenology Network, Tucson, AZ, 8New Mexico Department of Health, Albuquerque, NM, 9Ares Corporation, Albuquerque, NM. RATIONALE: Juniperus spp. pollen is a significant aeroallergen and has been observed to be transported 200-600 km from the source. Local observations of Juniperus spp. phenology may not be consistent with the timing and source of pollen collected by pollen sampling instruments. METHODS: The Dust REgional Atmospheric Model (DREAM)is a verified model for atmospheric dust transport modeling using MODIS data products to identify source regions and quantities of dust. The use of satellite data products for studying plant phenology is well documented and is able to identify Juniperus spp. community pollen phenology. We successfully modified the DREAM model to incorporate pollen transport (PREAM) and used MODIS satellite images to identify Juniper forest types during pollen formation and release. RESULTS: MODIS phenology profiles, as measured by the standard vegetation index (VI) product show a fairly strong seasonal signature in the EVI(Enhanced Vegetation Index) measure of vegetation activity through periods of snow, understory green-up and brown-down, and peak periods of vegetation activity. There was also quite strong interannual seasonal variability that are useful in analysis of the pre-conditions for timing and magnitudes of pollen release. The DREAM model was successfully modified to incorporate pollen transport (PREAM). Modeled transport of Juniperus spp. pollen closely matched the peak pollen concentrations in March 2006 for Albuquerque and Los Alamos. CONCLUSIONS: We believe that advanced pollen forecasts will lead to better preparedness for asthma and allergy sufferers, hospital admissions personnel, and workforce managers, pharmaceutical marketing and
distribution functions, and contribute to a reduction in false positive respiratory diagnoses.
60
Associations between Traffic-related Exposures and Incidence of Wheeze among Children Age 5-7 in NYC S. Hsu1, K. H. Jung1, K. Moors1, K. Bernabe1, P. L. Kinney2, S. N. Chillrud3, M. S. Perzanowski2, R. M. Whyatt2, L. A. Hoepner2, S. Wang2, B. Yan3, J. M. Ross3, F. Perera2, R. L. Miller1,2; 1Columbia University Department of Medicine, New York City, NY, 2Columbia University Mailman School of Public Health, Columbia Center for Children’s Environmental Health, New York City, NY, 3Lamont-Dohery Earth Observatory, Palisades, NY. RATIONALE: Exposure to traffic-related pollutants has been associated with asthma exacerbations in children. However, evidence linking traffic exposure, particularly diesel, to the development of asthma among young children is limited. We hypothesized that black carbon (BC), absorption coefficient (Abs), and PM2.5 concentrations measured from residences are associated with incident wheeze among children age 5-7 in NYC. METHODS: Two-week residential monitoring of BC via multi-wavelength, Abs via reflectance, and PM2.5 were conducted in 346 children ages 5/6 as part of the longitudinal Columbia Center for Children’s Environmental Health (CCCEH) cohort. Respiratory outcomes were assessed annually by administering International Study of Asthma and Allergies in Childhood questionnaire. Incident wheeze at age 6 or 7 was defined as report of wheeze (according to ‘‘ever’’ or ‘‘last 12-months’’ questions) among children without wheeze at age 5. Logistic regression adjusting for sex, ethnicity, smoking, maternal asthma and cold/flu season were applied to estimate the effects of pollution on incident wheeze. RESULTS: Twenty children (10%) were classified as incident wheeze cases. Exposure to residential BC, Abs, and PM2.5 was associated with excess risks of incident wheeze. The odds ratios (ORs) between incident wheeze, derived from ‘‘ever wheeze’’, and an interquartile range increase in BC, Abs, and PM2.5 were 1.46 (95% CI: 1.00 - 2.13), 2.13 (95% CI: 1.20 - 3.80), and 1.38 (95% CI: 1.01 - 1.88), respectively. Positive but nonsignificant ORs were found when using ‘‘wheeze in the last 12-months’’ to define incident wheeze. CONCLUSIONS: Exposure to traffic-generated pollutants may be associated with incident wheeze among young urban children.
61
Juniperus asheii (Mountain Cedar) Pollen Utilized As An Antigen In The Biogenics Chamber: Comparison Of Natural And Controlled Exposures D. A. Ramirez, R. L. Jacobs, C. P. Andrews; Biogenics Research Chamber, San Antonio, TX. RATIONALE: Juniperus asheii pollen allergy is a robust model commonly used in natural studies for pharmacologic efficacy. This study was performed to compare the symptoms elicited by Mountain cedar pollen in the natural season with symptoms in a controlled environment outside of the season. METHODS: Eighteen subjects sensitized to Juniperus asheii were monitored for symptoms during the 2009-2010 season. Symptomatic subjects were then exposed six months later in a controlled environment. Pollen levels of 11,500 grains/m3 were utilized during 2 hour chamber exposures on consecutive days. Total nasal symptom scores (TNSS) and total ocular symptom scores (TOSS) were compared in the natural season to the chamber exposure. TNSS scores of 7 or greater and TOSS scores of 5 or greater were considered meaningful. RESULTS: Up to four priming exposures were required to reach meaningful TNSS symptoms in 61% of subjects. Meaningful TOSS scores were seen in 78%. Chamber runs utilized pollen counts higher than the mean of natural exposure (3,500/m3.) These counts were 50% of maximum pollen counts during the recording interval of the season. CONCLUSIONS: In 2 hr priming chamber runs, Juniperus asheii pollen elicited meaningful symptoms in subjects shown to be symptomatic during the natural season. With consecutive chamber exposures, subjects can be selected for pharmacologic studies utilizing Juniperus asheii pollen in a controlled environment.
SATURDAY
Abstracts AB19
J ALLERGY CLIN IMMUNOL VOLUME 127, NUMBER 2