Polyethylene glycol–modified adenosine deaminase improved lung disease but not liver disease in partial adenosine deaminase deficiency

Because partial ADA deficiency might not be detected early, it is important to recognize these clinical and pathologic features, which could be the presenting manifestations of ADA deficiency and can be reversed with PEG-ADA or immunosuppression. Â At diagnosis (15 mo) Normal range or control value...

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Veröffentlicht in:	Journal of allergy and clinical immunology 2009-10, Vol.124 (4), p.848-850
Hauptverfasser:	Somech, Raz, MD, Lai, Yew Hon, MD, Grunebaum, Eyal, MD, Le Saux, Nicole, MD, Cutz, Ernest, MD, Roifman, Chaim M., MD
Format:	Artikel
Sprache:	eng
Schlagworte:	Adenosine Deaminase - deficiency Adenosine Deaminase - therapeutic use Allergy and Immunology Erythrocytes - enzymology Erythrocytes - metabolism Follow-Up Studies Humans Immune system Immunoglobulins - blood Infant, Newborn Liver Diseases - complications Liver Diseases - drug therapy Liver Diseases - enzymology Lung Diseases - complications Lung Diseases - drug therapy Lung Diseases - enzymology Lymphocytes Lymphocytes - enzymology Lymphocytes - metabolism Male Mutation Rodents Severe Combined Immunodeficiency - complications Severe Combined Immunodeficiency - drug therapy Severe Combined Immunodeficiency - enzymology T cell receptors
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Zusammenfassung:	Because partial ADA deficiency might not be detected early, it is important to recognize these clinical and pathologic features, which could be the presenting manifestations of ADA deficiency and can be reversed with PEG-ADA or immunosuppression. Â At diagnosis (15 mo) Normal range or control value Serum immunoglobulins Â Â IgG (g/L) 4.7 4.5-14.3 IgM (g/L) 0.4 0.2-1.0 IgA (g/L) 0.3 0.2-1.5 IgE (IU/mL) 1600
ISSN:	0091-6749 1097-6825
DOI:	10.1016/j.jaci.2009.07.003