Pregnancies in glycogen storage disease type Ia.
OBJECTIVE
Reports on pregnancies in women with glycogen storage disease type Ia (GSD-Ia) are scarce. Because of improved life expectancy, pregnancy is becoming an important issue. We describe 15 pregnancies by focusing on dietary treatment, biochemical parameters, and GSD-Ia complications.


METHODS
Carbohydrate requirements (milligrams per kilogram per minute), triglyceride and uric acid levels, liver ultrasonography, and creatinine clearance were investigated before, during, and after pregnancy. Data from the newborn infants were obtained from the records.


RESULTS
In the first trimester, a significant increase in carbohydrate requirements was observed (P = .007). Most patients had acceptable triglyceride and uric acid levels during pregnancy. No increase in size or number of adenomas was seen. In 3 of 4 patients, a decrease in glomerular filtration rate was observed after pregnancy. In 3 pregnancies, lactic acidosis developed during delivery with severe multiorgan failure in 1. All but 1 of the children are healthy and show good psychomotor development.


CONCLUSIONS
Successful pregnancies are possible in patients with GSD-Ia, although specific GSD-Ia-related risks are present.
Martens
Daniëlle H J
DH
Department of Pediatrics, Beatrix Children's Hospital, University Medical Center Groningen, the Netherlands. d.h.j.martensj@bkk.umcg.nl
Rake
Jan Peter
JP
Schwarz
Martin
M
Ullrich
Kurt
K
Weinstein
David A
DA
Merkel
Martin
M
Sauer
Pieter J J
PJ
Smit
G Peter A
GP
D000328:Adult; D004040:Dietary Carbohydrates / Q000627:therapeutic use*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000178:diet therapy*; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011248:Pregnancy Complications; D011256:Pregnancy Outcome; D016896:Treatment Outcome; D014280:Triglycerides / Q000097:blood; D014527:Uric Acid / Q000097:blood
D004040:Dietary Carbohydrates; D014280:Triglycerides; D014527:Uric Acid