title: Pregnancy and its management in women with GSD type III - a single centre experience.
abstract: We present a review of our experience and pregnancy outcome in patients with GSD III managed by our centre. Between 1997 and 2010 there were 15 pregnancies in seven women with GSD III. Four women had GSD IIIb (nine pregnancies) and three GSD IIIa (six pregnancies). There was a successful outcome in all 15 pregnancies with delivery of 15 liveborn infants. Four infants were of low birthweight (<2nd centile) but all have developed normally apart from one with behavioural/psychiatric problems. Three women had pre-existing cardiomyopathy prior to pregnancy. One of these women had deterioration of her cardiomyopathy during pregnancy and again in the post-partum period. Women with GSD III do not seem to have any issues with fertility. Overall the outcome of pregnancy for both mother and child is good. Care needs to be taken to avoid maternal hypoglycemia which may be associated with intrauterine growth restriction and low birth weight. Cardiac function should be monitored carefully particularly in those with pre-existing cardiomyopathy.
authors.affiliation: Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Internal Mailbox 92 Queen Square, London, WC1N 3BG, UK.
authors.forename: Radha
authors.initials: R
authors.lastname: Ramachandran
authors.forename: Yehani
authors.initials: Y
authors.lastname: Wedatilake
authors.forename: Caroline
authors.initials: C
authors.lastname: Coats
authors.forename: Fiona
authors.initials: F
authors.lastname: Walker
authors.forename: Perry
authors.initials: P
authors.lastname: Elliott
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Lee
authors.forename: Robin H
authors.initials: RH
authors.lastname: Lachmann
authors.forename: Elaine
authors.initials: E
authors.lastname: Murphy
mesh_terms: D000328:Adult; D019468:Disease Management; D005260:Female; D006010:Glycogen Storage Disease Type III / Q000150:complications* / Q000628:therapy*; D006801:Humans; D008875:Middle Aged; D011247:Pregnancy; D011248:Pregnancy Complications / Q000628:therapy*; D011256:Pregnancy Outcome; D012189:Retrospective Studies; D055815:Young Adult