Inflammatory consequences of inherited disorders affecting neutrophil function
نویسندگان
چکیده
منابع مشابه
Inherited traits affecting platelet function.
Inherited platelet disorders constitute a large group of diseases involving a wide range of genetic defects that can lead to bleeding symptoms of varying severity. They are associated with defects in surface membrane glycoproteins resulting in e.g. Bernard Soulier Syndrome and Glanzmann Thrombasthenia causing defects in platelet adhesion and aggregation, respectively, as well as in receptors fo...
متن کاملDisorders of neutrophil function.
Major bacterial infections are most commonly associated with agranulocytosis or an abnormality of immunoglobulins or complement. Occasionally, repeated infections cannot be attributed to these relatively common causes. In such cases, a quantitative abnormality in neutrophil function should be sought. Complete evaluation of neutrophil function, including: chemotaxis, adhesion, aggregation, phago...
متن کاملDiagnosis of inherited metabolic disorders affecting the nervous system.
Knowledge of the molecular causes for genetic diseases that affect the nervous system is rapidly expanding. Especially striking has been the finding in several autosomal dominant neurodegenerative disorders that unstable expansions of trinucleotide repeats are responsible for the genetic disorder and that the length of the repeat can be correlated with the age of onset and the severity of sympt...
متن کاملInherited and Acquired Disorders of Platelet Function
Platelet function defects are caused by rare congenital or, more frequently, by acquired disorders. They may lead to bleeding or thrombotic tendencies despite of normal platelet counts. The corresponding symptoms are often quite heterogeneous. A disorder of platelet function is suspected on the basis of case and family history, physical examination and platelet function tests. The so far primar...
متن کاملInherited Neutrophil Disorders: Molecular Basis and New Therapies.
Recent advances in our understanding of the molecular basis of inherited neutrophil disorders and complementary studies in transgenic mouse models have provided new insights into the normal mechanisms regulating myelopoiesis and the functional responses of mature neutrophils. Neutrophil specific granule deficiency is a rare disorder of neutrophil function characterized by a lack of neutrophil s...
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ژورنال
عنوان ژورنال: Blood
سال: 2019
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood-2018-11-844563