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This month's theme
Contents
T HE J OURNAL OF
Allergy Clinical Immunology AND
VOLUME 120 NUMBER 4 d
OFFICIAL JOURNAL OF THE AMERICAN ACADEMY OF ALLERGY, ASTHMA
Y
& IMMUNOLOGY
This month’s theme: Primary immunodeficiency About the cover
The goal of researchers and health professionals in primary immunodeficiencies is to help those affected lead full, healthy lives. Our cover shows this goal realized for 5 young friends who have X-linked severe combined immunodeficiency (SCID) successfully treated by bone marrow transplantation. This issue of the Journal is devoted to the topic of primary immunodeficiencies of children and adults (see the Images in Allergy and Immunology feature on p 982), the uncovering of molecular lesions, and immunoreconstitution, cellular in the case of these 5 young boys, who are not only surviving but also obviously thriving. Several feature articles highlight the theme of primary immunodeficiency. As part of our Current Reviews series, Raffaele Badolato and Silvia Parolini (p 735) examine adaptor protein 3 complex deficiency and how flaws in a protein transport system lead to Hermansky-Pudlak syndrome type 2, whereas in a Molecular Mechanisms review article, Troy R. Torgerson and Hans D. Ochs (p 744) discuss how studies of immune dysregulation, polyendocrinopathy, enteropathy, X-linked disorder have provided knowledge about FOXP3 expression and Treg function that may, in turn, impact how other autoimmune disorders are approached. In a Clinical Pearls article, Javier Chinen and colleagues (p 795) provide an example of the importance of follow-up for patients with immunodeficiencies. Finally, Jennifer M. Puck and the SCID Newborn Screening Working Group contribute a Rostrum (p 760) that looks at how population-based screening of newborns for SCID might be implemented. For further discussion of primary immunodeficiencies, see the original articles and letters to the editor marked with the red starburst next to their titles in the Table of Contents. Cover image provided through Dr Jennifer M. Puck, University of California, San Francisco, by the families of the children pictured. The photograph was taken at a meeting of the Immune Deficiency Foundation.
This month in Beyond Our Pages A study explored the efficacy of as-needed combination inhaled steroid–long-acting b-agonist therapy for mild persistent asthma. d The existence of a novel autocrine pathway for goblet cell production in the airways was demonstrated. d Encouraging preliminary findings suggested that children with adenoidal hypertrophy that is not associated with tonsillar hypertrophy should be considered for intranasal mometasone treatment before surgery is planned. d A report described an unexpected role for TH2 cytokines in mammary gland development. d A study explored the ongoing question of whether measurement of exhaled nitric oxide levels helps in determining therapy in persistent asthma. d In an experimental animal model, chemokine sequestration by D6 was found to be a novel and effective method of tumor suppression. d Findings in another study may help explain the current greater frequency of chronic obstructive pulmonary disease–related fatalities in women than in men in the United States. Ó 2007 American Academy of Allergy, Asthma & Immunology The Journal of Allergy and Clinical Immunology (ISSN: 0091-6749) is published monthly by Elsevier Inc., 360 Park Avenue South, New York, NY 100101710. Business Office: 1600 John F. Kennedy Boulevard, Suite 1800, Philadelphia, PA 19103-2899. Editorial Office: 11830 Westline Industrial Drive, St Louis, MO 63146-3318. Customer Service Office: 6277 Sea Harbor Drive, Orlando, FL 32887-4800. Periodicals postage paid at New York, NY and additional mailing offices. POSTMASTER: Send address changes to The Journal of Allergy and Clinical Immunology, Elsevier Periodicals Customer Service, 6277 Sea Harbor Drive, Orlando, FL 32887-4800.
4A October 2007
J ALLERGY CLIN IMMUNOL
T HE J OURNAL OF
Allergy Clinical Immunology AND
VOLUME 120 NUMBER 4 d
OFFICIAL JOURNAL OF THE AMERICAN ACADEMY OF ALLERGY, ASTHMA
Y
& IMMUNOLOGY
This month’s theme: Primary immunodeficiency About the cover
The goal of researchers and health professionals in primary immunodeficiencies is to help those affected lead full, healthy lives. Our cover shows this goal realized for 5 young friends who have X-linked severe combined immunodeficiency (SCID) successfully treated by bone marrow transplantation. This issue of the Journal is devoted to the topic of primary immunodeficiencies of children and adults (see the Images in Allergy and Immunology feature on p 982), the uncovering of molecular lesions, and immunoreconstitution, cellular in the case of these 5 young boys, who are not only surviving but also obviously thriving. Several feature articles highlight the theme of primary immunodeficiency. As part of our Current Reviews series, Raffaele Badolato and Silvia Parolini (p 735) examine adaptor protein 3 complex deficiency and how flaws in a protein transport system lead to Hermansky-Pudlak syndrome type 2, whereas in a Molecular Mechanisms review article, Troy R. Torgerson and Hans D. Ochs (p 744) discuss how studies of immune dysregulation, polyendocrinopathy, enteropathy, X-linked disorder have provided knowledge about FOXP3 expression and Treg function that may, in turn, impact how other autoimmune disorders are approached. In a Clinical Pearls article, Javier Chinen and colleagues (p 795) provide an example of the importance of follow-up for patients with immunodeficiencies. Finally, Jennifer M. Puck and the SCID Newborn Screening Working Group contribute a Rostrum (p 760) that looks at how population-based screening of newborns for SCID might be implemented. For further discussion of primary immunodeficiencies, see the original articles and letters to the editor marked with the red starburst next to their titles in the Table of Contents. Cover image provided through Dr Jennifer M. Puck, University of California, San Francisco, by the families of the children pictured. The photograph was taken at a meeting of the Immune Deficiency Foundation.
This month in Beyond Our Pages A study explored the efficacy of as-needed combination inhaled steroid–long-acting b-agonist therapy for mild persistent asthma. d The existence of a novel autocrine pathway for goblet cell production in the airways was demonstrated. d Encouraging preliminary findings suggested that children with adenoidal hypertrophy that is not associated with tonsillar hypertrophy should be considered for intranasal mometasone treatment before surgery is planned. d A report described an unexpected role for TH2 cytokines in mammary gland development. d A study explored the ongoing question of whether measurement of exhaled nitric oxide levels helps in determining therapy in persistent asthma. d In an experimental animal model, chemokine sequestration by D6 was found to be a novel and effective method of tumor suppression. d Findings in another study may help explain the current greater frequency of chronic obstructive pulmonary disease–related fatalities in women than in men in the United States. Ó 2007 American Academy of Allergy, Asthma & Immunology The Journal of Allergy and Clinical Immunology (ISSN: 0091-6749) is published monthly by Elsevier Inc., 360 Park Avenue South, New York, NY 100101710. Business Office: 1600 John F. Kennedy Boulevard, Suite 1800, Philadelphia, PA 19103-2899. Editorial Office: 11830 Westline Industrial Drive, St Louis, MO 63146-3318. Customer Service Office: 6277 Sea Harbor Drive, Orlando, FL 32887-4800. Periodicals postage paid at New York, NY and additional mailing offices. POSTMASTER: Send address changes to The Journal of Allergy and Clinical Immunology, Elsevier Periodicals Customer Service, 6277 Sea Harbor Drive, Orlando, FL 32887-4800.
4A October 2007
J ALLERGY CLIN IMMUNOL