Decreased numbers of chemotactic factor receptors in chronic neutropenia with defective chemotaxis: Spontaneous recovery from the neutrophil abnormalities during early childhood

Childhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8‐month‐old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N‐formyl‐methionyl‐leucyl‐phenylalanine (3H‐FMLP). The patient&...

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Veröffentlicht in:Am. J. Hematol.; (United States) 1987-05, Vol.25 (1), p.67-75
Hauptverfasser: Yasui, Kozo, Yamazaki, Munehiro, Miyagawa, Yukiaki, Komiyama, Atsushi, Akabane, Taro
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container_title Am. J. Hematol.; (United States)
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creator Yasui, Kozo
Yamazaki, Munehiro
Miyagawa, Yukiaki
Komiyama, Atsushi
Akabane, Taro
description Childhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8‐month‐old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N‐formyl‐methionyl‐leucyl‐phenylalanine (3H‐FMLP). The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (< 3 SD) as compared with those of control cells of 52,000 ± 6,000 (mean ± SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. These results demonstrate a transient decrease of neutrophil chemotactic factor receptors as one of the pathophysiological bases of a transient defect of neutrophil chemotaxis in this disorder.
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School of Medicine, Matsumoto, Japan</creatorcontrib><title>Decreased numbers of chemotactic factor receptors in chronic neutropenia with defective chemotaxis: Spontaneous recovery from the neutrophil abnormalities during early childhood</title><title>Am. J. Hematol.; (United States)</title><addtitle>Am J Hematol</addtitle><description>Childhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8‐month‐old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N‐formyl‐methionyl‐leucyl‐phenylalanine (3H‐FMLP). The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (&lt; 3 SD) as compared with those of control cells of 52,000 ± 6,000 (mean ± SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. 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The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (&lt; 3 SD) as compared with those of control cells of 52,000 ± 6,000 (mean ± SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. These results demonstrate a transient decrease of neutrophil chemotactic factor receptors as one of the pathophysiological bases of a transient defect of neutrophil chemotaxis in this disorder.</abstract><cop>New York</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>3578264</pmid><doi>10.1002/ajh.2830250107</doi><tpages>9</tpages></addata></record>
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subjects 550901 - Pathology- Tracer Techniques
AGE GROUPS
Agranulocytosis - metabolism
AMINO ACIDS
BASIC BIOLOGICAL SCIENCES
Biological and medical sciences
BIOLOGICAL MATERIALS
BIOLOGICAL RECOVERY
BLOOD
BLOOD CELLS
BODY FLUIDS
CARBOXYLIC ACIDS
Cell Movement
CHILDREN
Chronic Disease
DISEASES
Female
formyl‐methionyl‐leucyl‐phenylalanine (FMLP)
Hematologic and hematopoietic diseases
HEMIC DISEASES
Humans
immunodeficiency syndrome
Infant
ISOTOPE APPLICATIONS
LABELLED COMPOUNDS
LEUKOCYTES
MATERIALS
Medical sciences
MEMBRANE PROTEINS
N-Formylmethionine Leucyl-Phenylalanine - metabolism
Neutropenia - metabolism
neutrophil mobility
NEUTROPHILS
Neutrophils - cytology
ORGANIC ACIDS
ORGANIC COMPOUNDS
Other diseases. Hematologic involvement in other diseases
PATHOGENESIS
PATIENTS
phagocyte
PHENYLALANINE
PROTEINS
RECEPTORS
Receptors, Formyl Peptide
Receptors, Immunologic - analysis
RECOVERY
TRACER TECHNIQUES
Tritium
TRITIUM COMPOUNDS
title Decreased numbers of chemotactic factor receptors in chronic neutropenia with defective chemotaxis: Spontaneous recovery from the neutrophil abnormalities during early childhood
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