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 |
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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. |
doi_str_mv | 10.1203/00006450-198605000-00012 |
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L ; CLARK, M. M ; CODY, C. S ; STANLEY, C. A ; BAKER, L ; DOUGLAS, S. D</creator><creatorcontrib>KOVEN, N. L ; CLARK, M. M ; CODY, C. S ; STANLEY, C. A ; BAKER, L ; DOUGLAS, S. D</creatorcontrib><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.</description><identifier>ISSN: 0031-3998</identifier><identifier>EISSN: 1530-0447</identifier><identifier>DOI: 10.1203/00006450-198605000-00012</identifier><identifier>PMID: 3459131</identifier><identifier>CODEN: PEREBL</identifier><language>eng</language><publisher>Hagerstown, MD: Lippincott Williams & Wilkins</publisher><subject>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</subject><ispartof>Pediatric research, 1986-05, Vol.20 (5), p.438-442</ispartof><rights>1987 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c418t-98670fb1853545003f9d65d23fbd41ee4c41dbfb80c5957db0791543c5231b873</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=8101433$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=8106607$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/3459131$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><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. D</creatorcontrib><title>Impaired chemotaxis and neutrophil (polymorphonuclear leukocyte) function in glycogenosis type IB</title><title>Pediatric research</title><addtitle>Pediatr Res</addtitle><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.</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. L</creator><creator>CLARK, M. M</creator><creator>CODY, C. S</creator><creator>STANLEY, C. A</creator><creator>BAKER, L</creator><creator>DOUGLAS, S. D</creator><general>Lippincott Williams & Wilkins</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>19860501</creationdate><title>Impaired chemotaxis and neutrophil (polymorphonuclear leukocyte) function in glycogenosis type IB</title><author>KOVEN, N. L ; CLARK, M. M ; CODY, C. S ; STANLEY, C. A ; BAKER, L ; DOUGLAS, S. D</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c418t-98670fb1853545003f9d65d23fbd41ee4c41dbfb80c5957db0791543c5231b873</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1986</creationdate><topic>Adolescent</topic><topic>Applied sciences</topic><topic>Biological and medical sciences</topic><topic>Blood Bactericidal Activity</topic><topic>Carbohydrates (enzymatic deficiencies). 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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D</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Impaired chemotaxis and neutrophil (polymorphonuclear leukocyte) function in glycogenosis type IB</atitle><jtitle>Pediatric research</jtitle><addtitle>Pediatr Res</addtitle><date>1986-05-01</date><risdate>1986</risdate><volume>20</volume><issue>5</issue><spage>438</spage><epage>442</epage><pages>438-442</pages><issn>0031-3998</issn><eissn>1530-0447</eissn><coden>PEREBL</coden><abstract>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.</abstract><cop>Hagerstown, MD</cop><pub>Lippincott Williams & Wilkins</pub><pmid>3459131</pmid><doi>10.1203/00006450-198605000-00012</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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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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