Type III glycogenosis with deposition of urate and amyloid
A case of a 44-year-old man with hepatic form of glycogenosis was presented. The patient had abdominal distension and muscular weakness. The glucose tolerance test showed a diabetic pattern, though he had hypoglycemia in fasting state. The fructose tolerance test showed an ability of conversion from...
Saved in:
Published in | Acta pathologica japonica Vol. 30; no. 4; p. 599 |
---|---|
Main Authors | , , , |
Format | Journal Article |
Language | English |
Published |
Australia
01.07.1980
|
Subjects | |
Online Access | Get more information |
Cover
Loading…
Abstract | A case of a 44-year-old man with hepatic form of glycogenosis was presented. The patient had abdominal distension and muscular weakness. The glucose tolerance test showed a diabetic pattern, though he had hypoglycemia in fasting state. The fructose tolerance test showed an ability of conversion from fructose to glucose. The double glucagon test showed no rise of blood glucose in fasting state but a rise 2 hours after meal. These symptoms and laboratory data supported the clinical diagnosis of type III glycogenosis. At autopsy, glycogen was markedly deposited in the liver, and slightly in the kidneys and heart. The glycogen pooled in the hepatic cells histochemically showed a normal reaction to several glycogen stainings. Electron microscopy by using Thiéry's method revealed that the pooled glycogen particles were clearly arranged as rosettes measuring 1,000A in largest diameter composed of clustered monoparticulates. There were marked hyalinization of the islets of Langerhans containing amyloid. As to its pathogenesis, this change can be interpreted as a morphological expression of the hypofunction of beta-cells ascribed to long-standing hypoglycemia. |
---|---|
AbstractList | A case of a 44-year-old man with hepatic form of glycogenosis was presented. The patient had abdominal distension and muscular weakness. The glucose tolerance test showed a diabetic pattern, though he had hypoglycemia in fasting state. The fructose tolerance test showed an ability of conversion from fructose to glucose. The double glucagon test showed no rise of blood glucose in fasting state but a rise 2 hours after meal. These symptoms and laboratory data supported the clinical diagnosis of type III glycogenosis. At autopsy, glycogen was markedly deposited in the liver, and slightly in the kidneys and heart. The glycogen pooled in the hepatic cells histochemically showed a normal reaction to several glycogen stainings. Electron microscopy by using Thiéry's method revealed that the pooled glycogen particles were clearly arranged as rosettes measuring 1,000A in largest diameter composed of clustered monoparticulates. There were marked hyalinization of the islets of Langerhans containing amyloid. As to its pathogenesis, this change can be interpreted as a morphological expression of the hypofunction of beta-cells ascribed to long-standing hypoglycemia. |
Author | Yotsumoto, S Shirasawa, K Nagahara, S Kawaguchi, Y |
Author_xml | – sequence: 1 givenname: Y surname: Kawaguchi fullname: Kawaguchi, Y – sequence: 2 givenname: K surname: Shirasawa fullname: Shirasawa, K – sequence: 3 givenname: S surname: Yotsumoto fullname: Yotsumoto, S – sequence: 4 givenname: S surname: Nagahara fullname: Nagahara, S |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/6998254$$D View this record in MEDLINE/PubMed |
BookMark | eNotj0tLw0AURmdRqbX2JwiD-8Q7dx5J3EnxESi4qesyM7lTU5JMSFI0_96CPZuPs_ng3LFFFzti7FFAKi48nVKhFCQixywVRQ7p5EBIrdPfBVsBgEiMkXjLNuN4uigYNDqTS7Y0RZGjViv2vJ974mVZ8mMz-3ikLo71yH_q6ZtX1F9kqmPHY-DnwU7EbVdx285NrKt7dhNsM9Lmumv29fa6334ku8_3cvuyS7yCrEi88QjorDAhgNLkVaGlQy8taUlIFKwg640hpyoKwpGTiig3ubJISuKaPfz_9mfXUnXoh7q1w3y4NuAfIw9Mkw |
CitedBy_id | crossref_primary_10_1016_S1169_8330_03_00077_2 crossref_primary_10_1016_S1297_319X_03_00020_4 |
ContentType | Journal Article |
DBID | CGR CUY CVF ECM EIF NPM |
DOI | 10.1111/j.1440-1827.1980.tb01355.x |
DatabaseName | Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed |
DatabaseTitle | MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) |
DatabaseTitleList | MEDLINE |
Database_xml | – sequence: 1 dbid: NPM name: PubMed url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: EIF name: MEDLINE url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search sourceTypes: Index Database |
DeliveryMethod | no_fulltext_linktorsrc |
Discipline | Medicine |
ExternalDocumentID | 6998254 |
Genre | Journal Article Case Reports |
GroupedDBID | .55 .GJ 1OB 1OC 3O- 53G ACBWZ ACGFS ALMA_UNASSIGNED_HOLDINGS ASPBG AVWKF AZFZN BDRZF CGR CUY CVF ECM EIF F5P NPM SAMSI WXSBR X7J X7M ZGI ZJWQK |
ID | FETCH-LOGICAL-c4079-c6c202ba16ff045ec4953b2c3ae53e2eefa1eac66eb4def1beb34ee8684a2e432 |
ISSN | 0001-6632 |
IngestDate | Sat Sep 28 08:35:43 EDT 2024 |
IsPeerReviewed | true |
IsScholarly | true |
Issue | 4 |
Language | English |
LinkModel | OpenURL |
MergedId | FETCHMERGED-LOGICAL-c4079-c6c202ba16ff045ec4953b2c3ae53e2eefa1eac66eb4def1beb34ee8684a2e432 |
PMID | 6998254 |
ParticipantIDs | pubmed_primary_6998254 |
PublicationCentury | 1900 |
PublicationDate | July 1980 |
PublicationDateYYYYMMDD | 1980-07-01 |
PublicationDate_xml | – month: 07 year: 1980 text: July 1980 |
PublicationDecade | 1980 |
PublicationPlace | Australia |
PublicationPlace_xml | – name: Australia |
PublicationTitle | Acta pathologica japonica |
PublicationTitleAlternate | Acta Pathol Jpn |
PublicationYear | 1980 |
SSID | ssj0000626573 |
Score | 1.2666519 |
Snippet | A case of a 44-year-old man with hepatic form of glycogenosis was presented. The patient had abdominal distension and muscular weakness. The glucose tolerance... |
SourceID | pubmed |
SourceType | Index Database |
StartPage | 599 |
SubjectTerms | Adult Amyloid - metabolism Fructose Intolerance - diagnosis Glycogen Storage Disease - pathology Glycogen Storage Disease Type IV - metabolism Glycogen Storage Disease Type IV - pathology Gout - etiology Humans Islets of Langerhans - ultrastructure Liver Diseases - metabolism Liver Diseases - pathology Liver Glycogen - metabolism Male Uric Acid - metabolism |
Title | Type III glycogenosis with deposition of urate and amyloid |
URI | https://www.ncbi.nlm.nih.gov/pubmed/6998254 |
Volume | 30 |
hasFullText | |
inHoldings | 1 |
isFullTextHit | |
isPrint | |
link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1NT9wwELUKFYhL1UIRpR_yoTcUlEwcE7ghVMQWsSeQ4IRsZ7zaCjardisEv54Z22x2EaCWSxTZUr7ey2RmMn4jxHdVW2sBfAbe-Ew1u1VmTZVnCpxpjLMlIKcGTvr66Ez9PK_Ou1LesLpkYrfd3ZPrSl6DKo0RrrxK9j-QnR6UBmif8KUtIUzbf8OY86e9Xm9rcHXrWpZbZXmRkFpt8KEci93BvywIEXVZrylCH85159x3E8P6qg92cOuXGbNg7tRgH5sbM-CmKZ29DqKOw9_mD03NJUsv2gk9gTY0Z-rSqn0zMKwM3Q02ceVdnU-LUjvjWWTkoMwZz_RTZTibGQiWsIp9j16w0OGnMuxs87k4QV2Q6xNrN2egG18H7DRFhRDVpl-cfKSdnWYWxMJOzeavn1I58TMNukoVCOnGkihtqvR65gJXxFI67KNIJHgkp-_FuxRKyP3Iiw_iDY5WxfJJKpZYE3tMD0n0kLP0kEwP2dFDtl4Gekiih0z0-CjODn-cHhxlqVVG5igi382cdpCDNYX2npx0dFw2bMGVBqsSAdGbgj6xWqNVDfrCoi0VYq1rZQBVCeticdSOcENIcpC9p6Bc16VVpQdbKI6iwdvcYJPDJ7Ee7_pyHPVQLtPj2Hxu4rNY6fj0Rbz19PrhV_LlJvZbwOQew2BFVQ |
link.rule.ids | 786 |
linkProvider | National Library of Medicine |
openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Type+III+glycogenosis+with+deposition+of+urate+and+amyloid&rft.jtitle=Acta+pathologica+japonica&rft.au=Kawaguchi%2C+Y&rft.au=Shirasawa%2C+K&rft.au=Yotsumoto%2C+S&rft.au=Nagahara%2C+S&rft.date=1980-07-01&rft.issn=0001-6632&rft.volume=30&rft.issue=4&rft.spage=599&rft_id=info:doi/10.1111%2Fj.1440-1827.1980.tb01355.x&rft_id=info%3Apmid%2F6998254&rft_id=info%3Apmid%2F6998254&rft.externalDocID=6998254 |
thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0001-6632&client=summon |
thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0001-6632&client=summon |
thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0001-6632&client=summon |