Kostmann syndrome

Kostmann syndrome
an autosomal recessive form of severe congenital neutropenia (gene locus: 1q21.3), characterized by early onset of recurrent pyogenic infections of the skin and lung, absence of neutrophils in the blood, absolute monocytosis and eosinophilia, and early death. Called also infantile genetic agranulocytosis and Kostmann neutropenia.

Medical dictionary. 2011.

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  • Kostmann syndrome — Classification and external resources ICD 10 D70 ICD 9 288.01 …   Wikipedia

  • Kostmann syndrome (neutropenia) — Kost·mann syndrome (neutropenia) (kostґmahn) [Rolf Kostmann, Swedish physician, 1909–1982] see under syndrome …   Medical dictionary

  • Syndrome, Kostmann — A condition with a lack of neutrophils (a type of white blood cell that is important in fighting infection). Children with the syndrome suffer frequent infections from bacteria which in the past led to death in three quarters of cases before 3… …   Medical dictionary

  • Kostmann disease — Children born with this condition lack neutrophils (a type of white blood cell that is important in fighting infection). These children suffer frequent infections from bacteria which in the past led to death in three quarters of cases before 3… …   Medical dictionary

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Kostmann neutropenia — see under syndrome …   Medical dictionary

  • Chédiak–Higashi syndrome — Classification and external resources ICD 10 E70.3 (E70.340 ILDS) ICD 9 288.2 …   Wikipedia

  • Neutrophil immunodeficiency syndrome — Classification and external resources OMIM 608203 Neutrophil immunodeficiency syndrome is a condition caused by mutations in the Rac2 gene.[1] See also …   Wikipedia

  • Kostmann&’s syndrome — Autosomal recessive disease characterized by profound neutropenia. It appears that bone marrow precursor cells fail to respond to the endogenous (normal) levels of functional G CSF though they will respond to pharmacologic doses of G CSF and the… …   Dictionary of molecular biology

  • Kostmann's syndrome — severe congenital neutropenia a hereditary (autosomal recessive) disorder characterized by severe neutropenia. This results in frequent bacterial infections, and death often occurs before the age of 6 months …   The new mediacal dictionary

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