Fertility and pregnancy in women affected by glycogen storage disease type I, results of a multicenter Italian study.

Journal of Inherited Metabolic Disease
Annalisa SechiMaja Di Rocco

Abstract

Life expectancy of patients with glycogen storage disease (GSD) type I has improved considerably, opening new problems correlated with adult age. In females polycystic ovaries (PCOs) has been described as frequently associated with the disease, however successful pregnancies have been reported. Whether or not GSD I is associated with impaired reproductive function is still unclear. Data about female patients with GSD Ia and Ib, who were 16 years or older, were obtained from clinical records and interviews. A total of 32 women with GSD I (25 GSD Ia, 7 GSD Ib), median age 26 years (range 16-55), were included. 9/32 patients had delayed menarche, 17/32 had irregular cycles, 8/22 had documented polycystic ovaries. Five successful spontaneous pregnancies in four patients with GSD Ia and two in a woman with GSD Ib were reported. The latter had development and enlargement of hepatic adenomas during pregnancies. Despite the high prevalence of irregular menstruation cycles and polycystic ovaries, fertility seems not to be impaired in women with GSD I. During pregnancy monitoring for adenoma development is mandatory.

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Citations

Jun 7, 2013·Journal of Diabetes and Metabolic Disorders·Patrícia Margarida Serra CarvalhoJosé Manuel Nascimento Costa
Jan 31, 2014·The Journal of Perinatal & Neonatal Nursing·Iris A FerrecchiaDavid A Weinstein
Oct 31, 2014·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Priya S KishnaniMichael S Watson
Jun 20, 2015·Best Practice & Research. Clinical Obstetrics & Gynaecology·Elaine Murphy
Mar 24, 2018·Journal of Pediatric Gastroenterology and Nutrition·Pietro VajroHenkjan J Verkade
May 24, 2015·Current Opinion in Clinical Nutrition and Metabolic Care·Patricie Burda, Michel Hochuli
Nov 2, 2019·Digestive Diseases and Sciences·Christina LingDenis McCarthy
Nov 10, 2013·Obstetrics and Gynecology·Stephanie L AustinPriya S Kishnani
Jul 26, 2018·Journal of Inherited Metabolic Disease·Elizabeth D BrooksDwight D Koeberl
Sep 11, 2018·Reviews in Endocrine & Metabolic Disorders·Gisela Wilcox
May 15, 2021·HPB : the Official Journal of the International Hepato Pancreato Biliary Association·Martijn P D HaringVincent E de Meijer

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