دورية أكاديمية
[Adults with hereditary fructose intolerance: risks of fructose infusion].
| العنوان: | [Adults with hereditary fructose intolerance: risks of fructose infusion]. |
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| عنوان ترانسليتريتد: | Erwachsene mit hereditärer Fructoseintoleranz: Gefährdung durch Fructoseinfusion. |
| المؤلفون: | Steegmanns I; Neurologische Klinik, Heinrich-Lanz-Krankenhauses Mannheim., Rittmann M, Bayerl JR, Gitzelmann R |
| المصدر: | Deutsche medizinische Wochenschrift (1946) [Dtsch Med Wochenschr] 1990 Apr 06; Vol. 115 (14), pp. 539-41. |
| نوع المنشور: | Case Reports; English Abstract; Journal Article |
| اللغة: | German |
| بيانات الدورية: | Publisher: G. Thieme Country of Publication: Germany NLM ID: 0006723 Publication Model: Print Cited Medium: Print ISSN: 0012-0472 (Print) Linking ISSN: 00120472 NLM ISO Abbreviation: Dtsch Med Wochenschr Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Stuttgart [etc.] : G. Thieme, 1946- |
| مواضيع طبية MeSH: | Fructose/*adverse effects , Fructose Intolerance/*diagnosis , Fructose Metabolism, Inborn Errors/*diagnosis, Adult ; Female ; Fructose/administration & dosage ; Fructose Intolerance/genetics ; Fructose-Bisphosphate Aldolase/analysis ; Hepatomegaly ; Humans ; Infusions, Intravenous ; Liver/enzymology ; Pedigree ; Splenomegaly ; Ultrasonography ; gamma-Glutamyltransferase/blood |
| مستخلص: | After her first grand mal seizure a 30-year-old woman was given a fructose infusion by an emergency doctor. On admission to hospital she complained of severe nausea. Ultrasonography revealed hepatosplenomegaly and the gamma-GT concentration was raised to 25 U/l. As hyperinsulinism was suspected an oral glucose tolerance test was suggested, but refused by the patient. She reported marked aversion to all sweet foods. Examination of an endoscopically obtained liver biopsy revealed clear reduction in fructoaldolase activity in liver tissue, i.e. the diagnosis of hereditary fructose intolerance. Three of the patient's siblings were also affected. The widespread use of infusion solutions containing sorbitol and fructose has twice proved acutely hazardous in this patient and is generally life-threatening for persons with an inborn error of metabolism whose pathologic status often remains undiagnosed to an adult age. |
| المشرفين على المادة: | 30237-26-4 (Fructose) EC 2.3.2.2 (gamma-Glutamyltransferase) EC 4.1.2.13 (Fructose-Bisphosphate Aldolase) |
| تواريخ الأحداث: | Date Created: 19900406 Date Completed: 19900511 Latest Revision: 20061115 |
| رمز التحديث: | 20221213 |
| DOI: | 10.1055/s-2008-1065044 |
| PMID: | 1969793 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 0012-0472 |
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| DOI: | 10.1055/s-2008-1065044 |