Non-infectious Lung Disease in Patients with Adenosine Deaminase Deficient Severe Combined Immunodeficiency
Adenosine deaminase deficiency is a disorder of purine metabolism manifesting severe combined immunodeficiency (ADA-SCID) and systemic abnormalities. Increased levels of the substrate deoxyadenosine triphosphate (dATP) lead to immunodeficiency and are associated in a murine model with pulmonary insu...
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| Published in: | Journal of clinical immunology Vol. 32; no. 3; pp. 449 - 453 |
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| Main Authors: | , , , , , |
| Format: | Journal Article |
| Language: | English |
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| Abstract | Adenosine deaminase deficiency is a disorder of purine metabolism manifesting severe combined immunodeficiency (ADA-SCID) and systemic abnormalities. Increased levels of the substrate deoxyadenosine triphosphate (dATP) lead to immunodeficiency and are associated in a murine model with pulmonary insufficiency. We compared a cohort of patients with ADA-SCID and X-linked SCID and found that despite similar radiological and respiratory findings, positive microbiology is significantly less frequent in ADA-SCID patients (
p
< 0.0005), suggesting a metabolic pathogenesis for the lung disease. Clinicians should be aware of this possibility and correct metabolic abnormalities either through enzyme replacement or haematopoietic stem cell transplant, in addition to treating infectious complications. |
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| AbstractList | Adenosine deaminase deficiency is a disorder of purine metabolism manifesting severe combined immunodeficiency (ADA-SCID) and systemic abnormalities. Increased levels of the substrate deoxyadenosine triphosphate (dATP) lead to immunodeficiency and are associated in a murine model with pulmonary insufficiency. We compared a cohort of patients with ADA-SCID and X-linked SCID and found that despite similar radiological and respiratory findings, positive microbiology is significantly less frequent in ADA-SCID patients (p<0.0005), suggesting a metabolic pathogenesis for the lung disease. Clinicians should be aware of this possibility and correct metabolic abnormalities either through enzyme replacement or haematopoietic stem cell transplant, in addition to treating infectious complications.[PUBLICATION ABSTRACT] Adenosine deaminase deficiency is a disorder of purine metabolism manifesting severe combined immunodeficiency (ADA-SCID) and systemic abnormalities. Increased levels of the substrate deoxyadenosine triphosphate (dATP) lead to immunodeficiency and are associated in a murine model with pulmonary insufficiency. We compared a cohort of patients with ADA-SCID and X-linked SCID and found that despite similar radiological and respiratory findings, positive microbiology is significantly less frequent in ADA-SCID patients (p < 0.0005), suggesting a metabolic pathogenesis for the lung disease. Clinicians should be aware of this possibility and correct metabolic abnormalities either through enzyme replacement or haematopoietic stem cell transplant, in addition to treating infectious complications. Adenosine deaminase deficiency is a disorder of purine metabolism manifesting severe combined immunodeficiency (ADA-SCID) and systemic abnormalities. Increased levels of the substrate deoxyadenosine triphosphate (dATP) lead to immunodeficiency and are associated in a murine model with pulmonary insufficiency. We compared a cohort of patients with ADA-SCID and X-linked SCID and found that despite similar radiological and respiratory findings, positive microbiology is significantly less frequent in ADA-SCID patients ( p < 0.0005), suggesting a metabolic pathogenesis for the lung disease. Clinicians should be aware of this possibility and correct metabolic abnormalities either through enzyme replacement or haematopoietic stem cell transplant, in addition to treating infectious complications. |
| Author | Xu-Bayford, J. Gaspar, H. B. Owens, C. Algar, V. E. Booth, C. Fairbanks, L. |
| Author_xml | – sequence: 1 givenname: C. surname: Booth fullname: Booth, C. organization: Centre of Immunodeficiency, Molecular Immunology Unit, Institute of Child Health, Department of Clinical Immunology, Great Ormond Street Hospital National Health Service Trust – sequence: 2 givenname: V. E. surname: Algar fullname: Algar, V. E. organization: Department of Clinical Immunology, Great Ormond Street Hospital National Health Service Trust – sequence: 3 givenname: J. surname: Xu-Bayford fullname: Xu-Bayford, J. organization: Department of Clinical Immunology, Great Ormond Street Hospital National Health Service Trust – sequence: 4 givenname: L. surname: Fairbanks fullname: Fairbanks, L. organization: Purine Research Laboratory, Guy’s and St. Thomas’s NHS Trust – sequence: 5 givenname: C. surname: Owens fullname: Owens, C. organization: Department of Radiology, Great Ormond Street Hospital National Health Service Trust – sequence: 6 givenname: H. B. surname: Gaspar fullname: Gaspar, H. B. email: h.gaspar@ucl.ac.uk organization: Centre of Immunodeficiency, Molecular Immunology Unit, Institute of Child Health, Department of Clinical Immunology, Great Ormond Street Hospital National Health Service Trust |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/22350222$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1084/jem.192.2.159 10.1016/j.jpeds.2003.10.055 10.1016/j.jaci.2009.07.003 10.1056/NEJM199605233342104 10.1016/j.clim.2006.12.009 10.1056/NEJM198008143030706 10.1067/mpd.2001.115023 10.1056/NEJMoa0805817 10.1073/pnas.92.9.3673 10.1203/00006450-199333011-00008 10.1016/j.iac.2010.01.002 10.1016/S0090-1229(95)90306-2 10.1016/S0022-3476(76)80793-7 |
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| References | Cederbaum, Kaitila, Rimoin, Stiehm (CR8) 1976; 89 Hirschhorn, Paageorgiou, Kesarwala, Taft (CR6) 1980; 303 Wakamiya, Blackburn, Jurecic (CR12) 1995; 92 Gaspar (CR1) 2010; 30 Booth, Hershfield, Notarangelo (CR4) 2007; 123 Somech, Lai, Grunebaum, Le, Cutz, Roifman (CR14) 2009; 124 Hirschhorn (CR2) 1993; 33 Rogers, Lwin, Fairbanks, Gerritsen, Gaspar (CR11) 2001; 139 Hershfield (CR3) 1995; 76 Aiuti, Cattaneo, Galimberti (CR5) 2009; 360 Blackburn, Volmer, Thrasher (CR13) 2000; 192 Bollinger, Rredondo-Vega, Santisteban (CR9) 1996; 334 Ratech, Greco, Gallo (CR10) 1985; 120 Albuquerque, Gaspar (CR7) 2004; 144 19179314 - N Engl J Med. 2009 Jan 29;360(5):447-58 10899903 - J Exp Med. 2000 Jul 17;192(2):159-70 6156414 - N Engl J Med. 1980 Aug 14;303(7):377-80 4014441 - Am J Pathol. 1985 Jul;120(1):157-69 8614422 - N Engl J Med. 1996 May 23;334(21):1367-71 978320 - J Pediatr. 1976 Nov;89(5):737-42 19665771 - J Allergy Clin Immunol. 2009 Oct;124(4):848-50 7731963 - Proc Natl Acad Sci U S A. 1995 Apr 25;92 (9):3673-7 20493398 - Immunol Allergy Clin North Am. 2010 May;30(2):221-36 17300989 - Clin Immunol. 2007 May;123(2):139-47 14760277 - J Pediatr. 2004 Feb;144(2):278-80 8433873 - Pediatr Res. 1993 Jan;33(1 Suppl):S35-41 11445793 - J Pediatr. 2001 Jul;139(1):44-50 7554473 - Clin Immunol Immunopathol. 1995 Sep;76(3 Pt 2):S228-32 MR Blackburn (9658_CR13) 2000; 192 R Somech (9658_CR14) 2009; 124 M Wakamiya (9658_CR12) 1995; 92 R Hirschhorn (9658_CR6) 1980; 303 C Booth (9658_CR4) 2007; 123 SD Cederbaum (9658_CR8) 1976; 89 W Albuquerque (9658_CR7) 2004; 144 MS Hershfield (9658_CR3) 1995; 76 MH Rogers (9658_CR11) 2001; 139 HB Gaspar (9658_CR1) 2010; 30 R Hirschhorn (9658_CR2) 1993; 33 ME Bollinger (9658_CR9) 1996; 334 H Ratech (9658_CR10) 1985; 120 A Aiuti (9658_CR5) 2009; 360 |
| References_xml | – volume: 192 start-page: 159 year: 2000 end-page: 70 ident: CR13 article-title: Metabolic consequences of adenosine deaminase deficiency in mice are associated with defects in alveogenesis, pulmonary inflammation, and airway obstruction publication-title: J. Exp Med. doi: 10.1084/jem.192.2.159 contributor: fullname: Thrasher – volume: 144 start-page: 278 year: 2004 end-page: 80 ident: CR7 article-title: Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency publication-title: J Pediatr doi: 10.1016/j.jpeds.2003.10.055 contributor: fullname: Gaspar – volume: 120 start-page: 157 year: 1985 end-page: 69 ident: CR10 article-title: Pathologic findings in adenosine deaminase-deficient severe combined immunodeficiency. I. 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| SubjectTerms | Adenosine Deaminase - deficiency Adenosine Deaminase - therapeutic use Agammaglobulinemia - diagnosis Agammaglobulinemia - microbiology Agammaglobulinemia - therapy Biomedical and Life Sciences Biomedicine Bronchoalveolar Lavage Child, Preschool Enzyme Replacement Therapy Female Hematopoietic Stem Cell Transplantation Humans Immunology Infant Infant, Newborn Infectious Diseases Internal Medicine Lung Diseases - diagnosis Lung Diseases - microbiology Lung Diseases - therapy Male Medical Microbiology Severe Combined Immunodeficiency - diagnosis Severe Combined Immunodeficiency - microbiology Severe Combined Immunodeficiency - therapy Tomography, X-Ray Computed X-Linked Combined Immunodeficiency Diseases - diagnosis X-Linked Combined Immunodeficiency Diseases - microbiology X-Linked Combined Immunodeficiency Diseases - therapy |
| Title | Non-infectious Lung Disease in Patients with Adenosine Deaminase Deficient Severe Combined Immunodeficiency |
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