Impaired chemotaxis and neutrophil (polymorphonuclear leukocyte) function in glycogenosis type IB

Polymorphonuclear leukocyte (PMN) function was investigated in two patients with glycogen storage disease type IB and neutropenia. Glycogen storage disease type IB was documented by liver biopsy and a normal amount of latent glucose-6-phosphatase activity. Patient A had stomatitis, skin infections,...

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Veröffentlicht in:Pediatric research 1986-05, Vol.20 (5), p.438-442
Hauptverfasser: KOVEN, N. L, CLARK, M. M, CODY, C. S, STANLEY, C. A, BAKER, L, DOUGLAS, S. D
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container_end_page 442
container_issue 5
container_start_page 438
container_title Pediatric research
container_volume 20
creator KOVEN, N. L
CLARK, M. M
CODY, C. S
STANLEY, C. A
BAKER, L
DOUGLAS, S. D
description Polymorphonuclear leukocyte (PMN) function was investigated in two patients with glycogen storage disease type IB and neutropenia. Glycogen storage disease type IB was documented by liver biopsy and a normal amount of latent glucose-6-phosphatase activity. Patient A had stomatitis, skin infections, and septicemia; patient B had respiratory infections, periodontitis, and oral candidiasis. Absolute neutrophil counts ranged from 114 to 2580/mm3. Diminished and delayed migration of PMN into a skin "window" occurred in B. Random and directed PMN migration under agarose toward f-Met-Leu-Phe, pepstatin A, and zymosan-activated serum were severely diminished in both patients. At 10(-7) M f-Met-Leu-Phe, mean random and directed migration were 52 and 23% (A, n = 3) and 48 and 13% (B, n = 4) of controls. These results were independent of incubation time and chemoattractant concentration. Patients' PMN had diminished quantitative nitroblue tetrazolium reduction compared to controls. B had a significant defect in PMN bactericidal activity with Escherichia coli with less than 0.2 log killing at 2 h. These results further characterize the defect in PMN migration reported by Beaudet et al. (J Pediatr 97:906, 1980). The finding of other abnormalities of PMN function suggests a metabolic defect in the neutrophil which may be related to the microsomal membrane defect in hepatocytes in glycogen storage disease type IB.
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At 10(-7) M f-Met-Leu-Phe, mean random and directed migration were 52 and 23% (A, n = 3) and 48 and 13% (B, n = 4) of controls. These results were independent of incubation time and chemoattractant concentration. Patients' PMN had diminished quantitative nitroblue tetrazolium reduction compared to controls. B had a significant defect in PMN bactericidal activity with Escherichia coli with less than 0.2 log killing at 2 h. These results further characterize the defect in PMN migration reported by Beaudet et al. (J Pediatr 97:906, 1980). 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The finding of other abnormalities of PMN function suggests a metabolic defect in the neutrophil which may be related to the microsomal membrane defect in hepatocytes in glycogen storage disease type IB.</description><subject>Adolescent</subject><subject>Applied sciences</subject><subject>Biological and medical sciences</subject><subject>Blood Bactericidal Activity</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Chemotaxis, Leukocyte</subject><subject>Child</subject><subject>Errors of metabolism</subject><subject>Exact sciences and technology</subject><subject>Female</subject><subject>Glycogen Storage Disease Type I - physiopathology</subject><subject>Granulocytes - physiology</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Neutrophils - physiology</subject><subject>Other techniques and industries</subject><issn>0031-3998</issn><issn>1530-0447</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1986</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkEtLxDAQgIMo6_r4CUIOInqoZjZJkx518bEgeNFzSdPUraZJTVqw_96o654dCMMw30yYDyEM5BIWhF6RFDnjJINC5oSnKksPFjtoDpymgjGxi-aEUMhoUch9dBDjWyIYl2yGZpTxAijMkVp1vWqDqbFem84P6rONWLkaOzMOwffr1uLz3tup86Ffezdqa1TA1ozvXk-DucDN6PTQeodbh1_tpP2rcT6mLcPUG7y6OUJ7jbLRHG_yIXq5u31ePmSPT_er5fVjphnIIUt3CNJUIDnl6TBCm6LOeb2gTVUzMIYlrK6aShLNCy7qiogCOKOaLyhUUtBDdPa7tw_-YzRxKLs2amOtcsaPsRS5FCBkkUD5C-rgYwymKfvQdipMJZDy2275Z7fc2i1_7KbRk80fY9WZeju40Zn6p5u-ilrZJiin27jFJJA8J-IfGDBK6ReQI47i</recordid><startdate>19860501</startdate><enddate>19860501</enddate><creator>KOVEN, N. 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Glycogenosis</topic><topic>Chemotaxis, Leukocyte</topic><topic>Child</topic><topic>Errors of metabolism</topic><topic>Exact sciences and technology</topic><topic>Female</topic><topic>Glycogen Storage Disease Type I - physiopathology</topic><topic>Granulocytes - physiology</topic><topic>Humans</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Neutrophils - physiology</topic><topic>Other techniques and industries</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>KOVEN, N. L</creatorcontrib><creatorcontrib>CLARK, M. M</creatorcontrib><creatorcontrib>CODY, C. S</creatorcontrib><creatorcontrib>STANLEY, C. A</creatorcontrib><creatorcontrib>BAKER, L</creatorcontrib><creatorcontrib>DOUGLAS, S. 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subjects Adolescent
Applied sciences
Biological and medical sciences
Blood Bactericidal Activity
Carbohydrates (enzymatic deficiencies). Glycogenosis
Chemotaxis, Leukocyte
Child
Errors of metabolism
Exact sciences and technology
Female
Glycogen Storage Disease Type I - physiopathology
Granulocytes - physiology
Humans
Male
Medical sciences
Metabolic diseases
Neutrophils - physiology
Other techniques and industries
title Impaired chemotaxis and neutrophil (polymorphonuclear leukocyte) function in glycogenosis type IB
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