Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children
Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting—and postprandial 2 h—...
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creator | Nagasaka, Hironori Yorifuji, Tohru Bandsma, Robert H. J. Takatani, Tomozumi Asano, Hisaki Mochizuki, Hiroshi Takuwa, Mayuko Tsukahara, Hirokazu Inui, Ayano Tsunoda, Tomoyuki Komatsu, Haruki Hiejima, Eitaro Fujisawa, Tomoo Hirano, Ken-ichi Miida, Takashi Ohtake, Akira Taguchi, Tadao Miwa, Ichitomo |
description | Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting—and postprandial 2 h—plasma mannose and other blood carbohydrates and lipids for seven GSD Ia children receiving dietary interventions using cornstarch and six healthy age-matched children. Next, one-day successive intra-individual parameter changes were examined for six affected and two control children. Although there were no significant differences in fasting—and postprandial 2 h—glucose and insulin levels, the mannose level of the affected group was invariably much higher than that of the control group (
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doi_str_mv | 10.1007/s10545-012-9514-x |
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fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1273516522</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1908350653</sourcerecordid><originalsourceid>FETCH-LOGICAL-c5165-775a5470b1de2ad1e471631fbd0cd002624dad85fee8dac3db8b218c170f9a193</originalsourceid><addsrcrecordid>eNqFkU1v1DAQhiMEokvhB3BBlhASl4DHiePkiEqBRUUcgLPl2JOsq8ReMknb_fd4leVDSIiTLc_zzoz1ZNlT4K-Ac_WagMtS5hxE3kgo87t72QakKnJRVfJ-tuFQQl43Up5lj4iuOedNLeXD7EyIRkEj1Sa7-bLQbHxAx3a-37H9YGg0bDQhREI2IBEjDORnf4NsjqwbFjsvZvahZ-0Qo2N9ejmyPqTrwcYeA6M5TqZH5jyhSbX5sEe2Nczu_OAmDI-zB50ZCJ-czvPs27vLrxcf8qvP77cXb65yK6GSuVLSyFLxFhwK4wBLBVUBXeu4dZyLSpTOuFp2iLUztnBt3QqoLSjeNQaa4jx7ufbdT_H7gjTr0ZPFYTAB40IahCqOk4RI6PO_0Ou4TCFtp6HhdSF5JYtEwUrZKRJN2On95EczHTRwfZSiVyk6SdFHKfouZZ6dOi_tiO5X4qeFBLw4AYasGbrJBOvpN6cqLlVdJ06t3K0f8PD_yfrj9tPbVJEpKdYkpVDocfrjc__c-wdru7a8</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1908350653</pqid></control><display><type>article</type><title>Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><source>SpringerLink Journals</source><creator>Nagasaka, Hironori ; Yorifuji, Tohru ; Bandsma, Robert H. J. ; Takatani, Tomozumi ; Asano, Hisaki ; Mochizuki, Hiroshi ; Takuwa, Mayuko ; Tsukahara, Hirokazu ; Inui, Ayano ; Tsunoda, Tomoyuki ; Komatsu, Haruki ; Hiejima, Eitaro ; Fujisawa, Tomoo ; Hirano, Ken-ichi ; Miida, Takashi ; Ohtake, Akira ; Taguchi, Tadao ; Miwa, Ichitomo</creator><creatorcontrib>Nagasaka, Hironori ; Yorifuji, Tohru ; Bandsma, Robert H. J. ; Takatani, Tomozumi ; Asano, Hisaki ; Mochizuki, Hiroshi ; Takuwa, Mayuko ; Tsukahara, Hirokazu ; Inui, Ayano ; Tsunoda, Tomoyuki ; Komatsu, Haruki ; Hiejima, Eitaro ; Fujisawa, Tomoo ; Hirano, Ken-ichi ; Miida, Takashi ; Ohtake, Akira ; Taguchi, Tadao ; Miwa, Ichitomo</creatorcontrib><description>Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting—and postprandial 2 h—plasma mannose and other blood carbohydrates and lipids for seven GSD Ia children receiving dietary interventions using cornstarch and six healthy age-matched children. Next, one-day successive intra-individual parameter changes were examined for six affected and two control children. Although there were no significant differences in fasting—and postprandial 2 h—glucose and insulin levels, the mannose level of the affected group was invariably much higher than that of the control group (
p
< 0.001): the fasting level of the affected group was about two-fold that of the control group; the postprandial-2 h level remained almost unchanged in the affected group, although it was one-half of the fasting level in the control group. Inter-individual analyses revealed that the GSD Ia group mannose level was significantly and positively correlated with lactate and triglycerides levels at both time points (
p
< 0.01). In each control, mannose levels fluctuated greatly, maintaining strong and significant negative correlations with glucose and insulin levels (
p
< 0.001). Correlations were lower or nonexistent in GSD Ia children. In individuals with high lactate and triglycerides levels, strikingly high mannose levels never changed against glucose and insulin fluctuations. Plasma mannose is less sensitive to blood glucose and insulin in GSD Ia children. Its basal level and the fluctuation pattern differ by their metabolic activity.</description><identifier>ISSN: 0141-8955</identifier><identifier>EISSN: 1573-2665</identifier><identifier>DOI: 10.1007/s10545-012-9514-x</identifier><identifier>PMID: 22971957</identifier><identifier>CODEN: JIMDDP</identifier><language>eng</language><publisher>Dordrecht: Springer Netherlands</publisher><subject>Adolescent ; Biochemistry ; Biological and medical sciences ; Blood glucose ; Blood Glucose - metabolism ; Carbohydrates ; Carbohydrates (enzymatic deficiencies). Glycogenosis ; Child ; Children ; Errors of metabolism ; Fasting ; Fasting - blood ; Female ; General aspects ; Glucose ; Glucose - metabolism ; Glucose-6-phosphate ; Glucose-6-Phosphate - metabolism ; Glycogen ; Glycogen Storage Disease Type I - blood ; Glycogen Storage Disease Type I - metabolism ; Human Genetics ; Humans ; Insulin ; Internal Medicine ; Laboratory testing ; Lactic acid ; Lactic Acid - blood ; Lactic Acid - metabolism ; Lipids ; Lipids - blood ; Liver - metabolism ; Male ; Mannose ; Mannose - blood ; Medical genetics ; Medical sciences ; Medicine ; Medicine & Public Health ; Metabolic Diseases ; Original Article ; Pediatrics ; Plasma ; Postprandial Period - physiology ; Triglycerides ; Triglycerides - blood ; Triglycerides - metabolism</subject><ispartof>Journal of inherited metabolic disease, 2013-01, Vol.36 (1), p.75-81</ispartof><rights>SSIEM and Springer 2012</rights><rights>2013 SSIEM</rights><rights>2014 INIST-CNRS</rights><rights>Journal of Inherited Metabolic Disease is a copyright of Springer, 2013.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5165-775a5470b1de2ad1e471631fbd0cd002624dad85fee8dac3db8b218c170f9a193</citedby><cites>FETCH-LOGICAL-c5165-775a5470b1de2ad1e471631fbd0cd002624dad85fee8dac3db8b218c170f9a193</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s10545-012-9514-x$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s10545-012-9514-x$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,41464,42533,45550,45551,51294</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=27605788$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22971957$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Nagasaka, Hironori</creatorcontrib><creatorcontrib>Yorifuji, Tohru</creatorcontrib><creatorcontrib>Bandsma, Robert H. J.</creatorcontrib><creatorcontrib>Takatani, Tomozumi</creatorcontrib><creatorcontrib>Asano, Hisaki</creatorcontrib><creatorcontrib>Mochizuki, Hiroshi</creatorcontrib><creatorcontrib>Takuwa, Mayuko</creatorcontrib><creatorcontrib>Tsukahara, Hirokazu</creatorcontrib><creatorcontrib>Inui, Ayano</creatorcontrib><creatorcontrib>Tsunoda, Tomoyuki</creatorcontrib><creatorcontrib>Komatsu, Haruki</creatorcontrib><creatorcontrib>Hiejima, Eitaro</creatorcontrib><creatorcontrib>Fujisawa, Tomoo</creatorcontrib><creatorcontrib>Hirano, Ken-ichi</creatorcontrib><creatorcontrib>Miida, Takashi</creatorcontrib><creatorcontrib>Ohtake, Akira</creatorcontrib><creatorcontrib>Taguchi, Tadao</creatorcontrib><creatorcontrib>Miwa, Ichitomo</creatorcontrib><title>Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children</title><title>Journal of inherited metabolic disease</title><addtitle>J Inherit Metab Dis</addtitle><addtitle>J Inherit Metab Dis</addtitle><description>Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting—and postprandial 2 h—plasma mannose and other blood carbohydrates and lipids for seven GSD Ia children receiving dietary interventions using cornstarch and six healthy age-matched children. Next, one-day successive intra-individual parameter changes were examined for six affected and two control children. Although there were no significant differences in fasting—and postprandial 2 h—glucose and insulin levels, the mannose level of the affected group was invariably much higher than that of the control group (
p
< 0.001): the fasting level of the affected group was about two-fold that of the control group; the postprandial-2 h level remained almost unchanged in the affected group, although it was one-half of the fasting level in the control group. Inter-individual analyses revealed that the GSD Ia group mannose level was significantly and positively correlated with lactate and triglycerides levels at both time points (
p
< 0.01). In each control, mannose levels fluctuated greatly, maintaining strong and significant negative correlations with glucose and insulin levels (
p
< 0.001). Correlations were lower or nonexistent in GSD Ia children. In individuals with high lactate and triglycerides levels, strikingly high mannose levels never changed against glucose and insulin fluctuations. Plasma mannose is less sensitive to blood glucose and insulin in GSD Ia children. Its basal level and the fluctuation pattern differ by their metabolic activity.</description><subject>Adolescent</subject><subject>Biochemistry</subject><subject>Biological and medical sciences</subject><subject>Blood glucose</subject><subject>Blood Glucose - metabolism</subject><subject>Carbohydrates</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Child</subject><subject>Children</subject><subject>Errors of metabolism</subject><subject>Fasting</subject><subject>Fasting - blood</subject><subject>Female</subject><subject>General aspects</subject><subject>Glucose</subject><subject>Glucose - metabolism</subject><subject>Glucose-6-phosphate</subject><subject>Glucose-6-Phosphate - metabolism</subject><subject>Glycogen</subject><subject>Glycogen Storage Disease Type I - blood</subject><subject>Glycogen Storage Disease Type I - metabolism</subject><subject>Human Genetics</subject><subject>Humans</subject><subject>Insulin</subject><subject>Internal Medicine</subject><subject>Laboratory testing</subject><subject>Lactic acid</subject><subject>Lactic Acid - blood</subject><subject>Lactic Acid - metabolism</subject><subject>Lipids</subject><subject>Lipids - blood</subject><subject>Liver - metabolism</subject><subject>Male</subject><subject>Mannose</subject><subject>Mannose - blood</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Metabolic Diseases</subject><subject>Original Article</subject><subject>Pediatrics</subject><subject>Plasma</subject><subject>Postprandial Period - physiology</subject><subject>Triglycerides</subject><subject>Triglycerides - blood</subject><subject>Triglycerides - metabolism</subject><issn>0141-8955</issn><issn>1573-2665</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNqFkU1v1DAQhiMEokvhB3BBlhASl4DHiePkiEqBRUUcgLPl2JOsq8ReMknb_fd4leVDSIiTLc_zzoz1ZNlT4K-Ac_WagMtS5hxE3kgo87t72QakKnJRVfJ-tuFQQl43Up5lj4iuOedNLeXD7EyIRkEj1Sa7-bLQbHxAx3a-37H9YGg0bDQhREI2IBEjDORnf4NsjqwbFjsvZvahZ-0Qo2N9ejmyPqTrwcYeA6M5TqZH5jyhSbX5sEe2Nczu_OAmDI-zB50ZCJ-czvPs27vLrxcf8qvP77cXb65yK6GSuVLSyFLxFhwK4wBLBVUBXeu4dZyLSpTOuFp2iLUztnBt3QqoLSjeNQaa4jx7ufbdT_H7gjTr0ZPFYTAB40IahCqOk4RI6PO_0Ou4TCFtp6HhdSF5JYtEwUrZKRJN2On95EczHTRwfZSiVyk6SdFHKfouZZ6dOi_tiO5X4qeFBLw4AYasGbrJBOvpN6cqLlVdJ06t3K0f8PD_yfrj9tPbVJEpKdYkpVDocfrjc__c-wdru7a8</recordid><startdate>201301</startdate><enddate>201301</enddate><creator>Nagasaka, Hironori</creator><creator>Yorifuji, Tohru</creator><creator>Bandsma, Robert H. J.</creator><creator>Takatani, Tomozumi</creator><creator>Asano, Hisaki</creator><creator>Mochizuki, Hiroshi</creator><creator>Takuwa, Mayuko</creator><creator>Tsukahara, Hirokazu</creator><creator>Inui, Ayano</creator><creator>Tsunoda, Tomoyuki</creator><creator>Komatsu, Haruki</creator><creator>Hiejima, Eitaro</creator><creator>Fujisawa, Tomoo</creator><creator>Hirano, Ken-ichi</creator><creator>Miida, Takashi</creator><creator>Ohtake, Akira</creator><creator>Taguchi, Tadao</creator><creator>Miwa, Ichitomo</creator><general>Springer Netherlands</general><general>Springer</general><general>Blackwell Publishing Ltd</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>3V.</scope><scope>7QP</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PHGZM</scope><scope>PHGZT</scope><scope>PJZUB</scope><scope>PKEHL</scope><scope>PPXIY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>201301</creationdate><title>Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children</title><author>Nagasaka, Hironori ; Yorifuji, Tohru ; Bandsma, Robert H. J. ; Takatani, Tomozumi ; Asano, Hisaki ; Mochizuki, Hiroshi ; Takuwa, Mayuko ; Tsukahara, Hirokazu ; Inui, Ayano ; Tsunoda, Tomoyuki ; Komatsu, Haruki ; Hiejima, Eitaro ; Fujisawa, Tomoo ; Hirano, Ken-ichi ; Miida, Takashi ; Ohtake, Akira ; Taguchi, Tadao ; Miwa, Ichitomo</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5165-775a5470b1de2ad1e471631fbd0cd002624dad85fee8dac3db8b218c170f9a193</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adolescent</topic><topic>Biochemistry</topic><topic>Biological and medical sciences</topic><topic>Blood glucose</topic><topic>Blood Glucose - metabolism</topic><topic>Carbohydrates</topic><topic>Carbohydrates (enzymatic deficiencies). Glycogenosis</topic><topic>Child</topic><topic>Children</topic><topic>Errors of metabolism</topic><topic>Fasting</topic><topic>Fasting - blood</topic><topic>Female</topic><topic>General aspects</topic><topic>Glucose</topic><topic>Glucose - metabolism</topic><topic>Glucose-6-phosphate</topic><topic>Glucose-6-Phosphate - metabolism</topic><topic>Glycogen</topic><topic>Glycogen Storage Disease Type I - blood</topic><topic>Glycogen Storage Disease Type I - metabolism</topic><topic>Human Genetics</topic><topic>Humans</topic><topic>Insulin</topic><topic>Internal Medicine</topic><topic>Laboratory testing</topic><topic>Lactic acid</topic><topic>Lactic Acid - blood</topic><topic>Lactic Acid - metabolism</topic><topic>Lipids</topic><topic>Lipids - blood</topic><topic>Liver - metabolism</topic><topic>Male</topic><topic>Mannose</topic><topic>Mannose - blood</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Metabolic Diseases</topic><topic>Original Article</topic><topic>Pediatrics</topic><topic>Plasma</topic><topic>Postprandial Period - physiology</topic><topic>Triglycerides</topic><topic>Triglycerides - blood</topic><topic>Triglycerides - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Nagasaka, Hironori</creatorcontrib><creatorcontrib>Yorifuji, Tohru</creatorcontrib><creatorcontrib>Bandsma, Robert H. J.</creatorcontrib><creatorcontrib>Takatani, Tomozumi</creatorcontrib><creatorcontrib>Asano, Hisaki</creatorcontrib><creatorcontrib>Mochizuki, Hiroshi</creatorcontrib><creatorcontrib>Takuwa, Mayuko</creatorcontrib><creatorcontrib>Tsukahara, Hirokazu</creatorcontrib><creatorcontrib>Inui, Ayano</creatorcontrib><creatorcontrib>Tsunoda, Tomoyuki</creatorcontrib><creatorcontrib>Komatsu, Haruki</creatorcontrib><creatorcontrib>Hiejima, Eitaro</creatorcontrib><creatorcontrib>Fujisawa, Tomoo</creatorcontrib><creatorcontrib>Hirano, Ken-ichi</creatorcontrib><creatorcontrib>Miida, Takashi</creatorcontrib><creatorcontrib>Ohtake, Akira</creatorcontrib><creatorcontrib>Taguchi, Tadao</creatorcontrib><creatorcontrib>Miwa, Ichitomo</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest Central (New)</collection><collection>ProQuest One Academic (New)</collection><collection>ProQuest Health & Medical Research Collection</collection><collection>ProQuest One Academic Middle East (New)</collection><collection>ProQuest One Health & Nursing</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of inherited metabolic disease</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Nagasaka, Hironori</au><au>Yorifuji, Tohru</au><au>Bandsma, Robert H. J.</au><au>Takatani, Tomozumi</au><au>Asano, Hisaki</au><au>Mochizuki, Hiroshi</au><au>Takuwa, Mayuko</au><au>Tsukahara, Hirokazu</au><au>Inui, Ayano</au><au>Tsunoda, Tomoyuki</au><au>Komatsu, Haruki</au><au>Hiejima, Eitaro</au><au>Fujisawa, Tomoo</au><au>Hirano, Ken-ichi</au><au>Miida, Takashi</au><au>Ohtake, Akira</au><au>Taguchi, Tadao</au><au>Miwa, Ichitomo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children</atitle><jtitle>Journal of inherited metabolic disease</jtitle><stitle>J Inherit Metab Dis</stitle><addtitle>J Inherit Metab Dis</addtitle><date>2013-01</date><risdate>2013</risdate><volume>36</volume><issue>1</issue><spage>75</spage><epage>81</epage><pages>75-81</pages><issn>0141-8955</issn><eissn>1573-2665</eissn><coden>JIMDDP</coden><abstract>Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting—and postprandial 2 h—plasma mannose and other blood carbohydrates and lipids for seven GSD Ia children receiving dietary interventions using cornstarch and six healthy age-matched children. Next, one-day successive intra-individual parameter changes were examined for six affected and two control children. Although there were no significant differences in fasting—and postprandial 2 h—glucose and insulin levels, the mannose level of the affected group was invariably much higher than that of the control group (
p
< 0.001): the fasting level of the affected group was about two-fold that of the control group; the postprandial-2 h level remained almost unchanged in the affected group, although it was one-half of the fasting level in the control group. Inter-individual analyses revealed that the GSD Ia group mannose level was significantly and positively correlated with lactate and triglycerides levels at both time points (
p
< 0.01). In each control, mannose levels fluctuated greatly, maintaining strong and significant negative correlations with glucose and insulin levels (
p
< 0.001). Correlations were lower or nonexistent in GSD Ia children. In individuals with high lactate and triglycerides levels, strikingly high mannose levels never changed against glucose and insulin fluctuations. Plasma mannose is less sensitive to blood glucose and insulin in GSD Ia children. Its basal level and the fluctuation pattern differ by their metabolic activity.</abstract><cop>Dordrecht</cop><pub>Springer Netherlands</pub><pmid>22971957</pmid><doi>10.1007/s10545-012-9514-x</doi><tpages>7</tpages></addata></record> |
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subjects | Adolescent Biochemistry Biological and medical sciences Blood glucose Blood Glucose - metabolism Carbohydrates Carbohydrates (enzymatic deficiencies). Glycogenosis Child Children Errors of metabolism Fasting Fasting - blood Female General aspects Glucose Glucose - metabolism Glucose-6-phosphate Glucose-6-Phosphate - metabolism Glycogen Glycogen Storage Disease Type I - blood Glycogen Storage Disease Type I - metabolism Human Genetics Humans Insulin Internal Medicine Laboratory testing Lactic acid Lactic Acid - blood Lactic Acid - metabolism Lipids Lipids - blood Liver - metabolism Male Mannose Mannose - blood Medical genetics Medical sciences Medicine Medicine & Public Health Metabolic Diseases Original Article Pediatrics Plasma Postprandial Period - physiology Triglycerides Triglycerides - blood Triglycerides - metabolism |
title | Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children |
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