Defective neutrophil and monocyte functions in glycogen storage disease type Ib: A literature review
العنوان: | Defective neutrophil and monocyte functions in glycogen storage disease type Ib: A literature review |
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المؤلفون: | Nils U. Bosshard, R. Gitzelmann |
المصدر: | European Journal of Pediatrics. 152:33-38 |
بيانات النشر: | Springer Science and Business Media LLC, 1993. |
سنة النشر: | 1993 |
مصطلحات موضوعية: | medicine.medical_specialty, Neutropenia, Phagocyte, Neutrophils, business.industry, Monocyte, Motility, Glycogen Storage Disease Type I, Lithium, Phagocytic dysfunction, Monocytes, Transport protein, medicine.anatomical_structure, Endocrinology, In vivo, Internal medicine, Pediatrics, Perinatology and Child Health, Immunology, Glycogen Storage Disease Type Ib, Glucose-6-Phosphatase, Humans, Medicine, Respiratory system, business, Respiratory Burst |
الوصف: | A summary review of leukocyte function in 42 published cases of glycogen storage disease Ib is presented. Polymorphonuclear and monocyte dysfunctions were evidenced in the majority of cases, whereas lymphocytes appeared to be unaffected. Phagocyte dysfunctions comprised in vivo mobilization and motility, in vitro random and directed migration, and one or several component functions of the "metabolic" ("respiratory") burst. On the basis of the available data it is impossible to know whether a primary functional deficit of the glucose 6-phosphate transport protein of the microsomal glucose 6-phosphatase system, as demonstrated in liver, also exists in these phagocytic cells and is responsible for this dysfunction. |
تدمد: | 1432-1076 0943-9676 |
URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2ae2e82f3455a14eb3b20354c753f78b https://doi.org/10.1007/bf02072085 |
حقوق: | CLOSED |
رقم الأكسشن: | edsair.doi.dedup.....2ae2e82f3455a14eb3b20354c753f78b |
قاعدة البيانات: | OpenAIRE |
تدمد: | 14321076 09439676 |
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