Chondroosseous dysplasia in severe combined immunodeficiency due to adenosine deaminase deficiency (chondroosseous dysplasia in ADA deficiency scid)
العنوان: | Chondroosseous dysplasia in severe combined immunodeficiency due to adenosine deaminase deficiency (chondroosseous dysplasia in ADA deficiency scid) |
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المؤلفون: | J Lobell, P Borns, V S Chakravarti, S D Douglas |
المصدر: | Pediatric Radiology. 21:447-448 |
بيانات النشر: | Springer Science and Business Media LLC, 1991. |
سنة النشر: | 1991 |
مصطلحات موضوعية: | Male, Enzyme deficiency, Adenosine Deaminase, Ribs, Adenosine deaminase, Immunopathology, medicine, Humans, Radiology, Nuclear Medicine and imaging, Bone Diseases, Developmental, Severe combined immunodeficiency, biology, business.industry, Infant, Enzyme replacement therapy, medicine.disease, Adenosine deaminase deficiency, Radiography, medicine.anatomical_structure, Dysplasia, Epiphysis, Pediatrics, Perinatology and Child Health, Immunology, biology.protein, Severe Combined Immunodeficiency, business |
الوصف: | Adenosine deaminase (ADA) deficiency may manifest as severe combined immunodeficiency (SCID) in early infancy. Some of these children develop radiologic changes which may be in part related to effects of this enzyme deficiency on the bony epiphysis. We describe the radiologic changes in a neonate with ADA deficiency and their resolution with polyethylene glycol conjugated adenosine deaminase (PEG-ADA, ADAGEN: Enzon, Inc., South Plainfield, NJ) enzyme replacement therapy. |
تدمد: | 1432-1998 0301-0449 |
DOI: | 10.1007/bf02026688 |
URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f68750c1b3c9223f039ac26a82c4634c https://doi.org/10.1007/bf02026688 |
Rights: | CLOSED |
رقم الانضمام: | edsair.doi.dedup.....f68750c1b3c9223f039ac26a82c4634c |
قاعدة البيانات: | OpenAIRE |
تدمد: | 14321998 03010449 |
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DOI: | 10.1007/bf02026688 |