Pregnancies in glycogen storage disease type Ia.
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Pregnancies in glycogen storage disease type Ia. / Martens, Daniëlle H J; Rake, Jan Peter; Schwarz, Martin; Ullrich, Kurt; Weinstein, David A; Merkel, Martin; Sauer, Pieter J J; Smit, G Peter A.
in: AM J OBSTET GYNECOL, Jahrgang 198, Nr. 6, 6, 2008, S. 1-7.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Pregnancies in glycogen storage disease type Ia.
AU - Martens, Daniëlle H J
AU - Rake, Jan Peter
AU - Schwarz, Martin
AU - Ullrich, Kurt
AU - Weinstein, David A
AU - Merkel, Martin
AU - Sauer, Pieter J J
AU - Smit, G Peter A
PY - 2008
Y1 - 2008
N2 - 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. STUDY DESIGN: 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. CONCLUSION: Successful pregnancies are possible in patients with GSD-Ia, although specific GSD-Ia-related risks are present.
AB - 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. STUDY DESIGN: 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. CONCLUSION: Successful pregnancies are possible in patients with GSD-Ia, although specific GSD-Ia-related risks are present.
M3 - SCORING: Zeitschriftenaufsatz
VL - 198
SP - 1
EP - 7
JO - AM J OBSTET GYNECOL
JF - AM J OBSTET GYNECOL
SN - 0002-9378
IS - 6
M1 - 6
ER -