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—...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Journal of inherited metabolic disease 2013-01, Vol.36 (1), p.75-81
Hauptverfasser: 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
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page 81
container_issue 1
container_start_page 75
container_title Journal of inherited metabolic disease
container_volume 36
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 ( p  
doi_str_mv 10.1007/s10545-012-9514-x
format Article
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  &lt; 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  &lt; 0.01). In each control, mannose levels fluctuated greatly, maintaining strong and significant negative correlations with glucose and insulin levels ( p  &lt; 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 &amp; 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&amp;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  &lt; 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  &lt; 0.01). In each control, mannose levels fluctuated greatly, maintaining strong and significant negative correlations with glucose and insulin levels ( p  &lt; 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 &amp; 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 &amp; 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 &amp; Calcified Tissue Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Health &amp; 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 &amp; Medical Complete (Alumni)</collection><collection>Health &amp; Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest Central (New)</collection><collection>ProQuest One Academic (New)</collection><collection>ProQuest Health &amp; Medical Research Collection</collection><collection>ProQuest One Academic Middle East (New)</collection><collection>ProQuest One Health &amp; 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  &lt; 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  &lt; 0.01). In each control, mannose levels fluctuated greatly, maintaining strong and significant negative correlations with glucose and insulin levels ( p  &lt; 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>
fulltext fulltext
identifier ISSN: 0141-8955
ispartof Journal of inherited metabolic disease, 2013-01, Vol.36 (1), p.75-81
issn 0141-8955
1573-2665
language eng
recordid cdi_proquest_miscellaneous_1273516522
source MEDLINE; Wiley Online Library Journals Frontfile Complete; SpringerLink Journals
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
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-15T02%3A24%3A33IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Sustained%20high%20plasma%20mannose%20less%20sensitive%20to%20fluctuating%20blood%20glucose%20in%20glycogen%20storage%20disease%20type%20Ia%20children&rft.jtitle=Journal%20of%20inherited%20metabolic%20disease&rft.au=Nagasaka,%20Hironori&rft.date=2013-01&rft.volume=36&rft.issue=1&rft.spage=75&rft.epage=81&rft.pages=75-81&rft.issn=0141-8955&rft.eissn=1573-2665&rft.coden=JIMDDP&rft_id=info:doi/10.1007/s10545-012-9514-x&rft_dat=%3Cproquest_cross%3E1908350653%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1908350653&rft_id=info:pmid/22971957&rfr_iscdi=true