Pregnancies in glycogen storage disease type Ia

Danielle H. J. Martens; Jan Peter Rake; Martin Schwarz; Kurt Ullrich; David A. Weinstein; Martin Merkel; Pieter J. J. Sauer; G. Peter A. Smit

doi:10.1016/j.ajog.2007.11.050

Pregnancies in glycogen storage disease type Ia

Danielle H. J. Martens^*, Jan Peter Rake, Martin Schwarz, Kurt Ullrich, David A. Weinstein, Martin Merkel, Pieter J. J. Sauer, G. Peter A. Smit

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

27 Citations (Scopus)

Abstract

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.

Original language	English
Article number	ARTN 646.e1
Number of pages	7
Journal	American Journal of Obstetrics and Gynecology
Volume	198
Issue number	6
DOIs	https://doi.org/10.1016/j.ajog.2007.11.050
Publication status	Published - Jun-2008
Event	42nd Annual Meeting of the Society-for-the-Study-of-Inborn-Errors-of-Metabolism - , France Duration: 6-Sept-2005 → 9-Sept-2005

Keywords

complications
glucose
glycogen storage disease type Ia
pregnancy
RENAL-FUNCTION
ESGSD-I
MANAGEMENT
CREATININE

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@article{38352c366c874b8e8586786c5d219aab,

title = "Pregnancies in glycogen storage disease type Ia",

abstract = "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.",

keywords = "complications, glucose, glycogen storage disease type Ia, pregnancy, RENAL-FUNCTION, ESGSD-I, MANAGEMENT, CREATININE",

author = "Martens, {Danielle H. J.} and Rake, {Jan Peter} and Martin Schwarz and Kurt Ullrich and Weinstein, {David A.} and Martin Merkel and Sauer, {Pieter J. J.} and Smit, {G. Peter A.}",

year = "2008",

month = jun,

doi = "10.1016/j.ajog.2007.11.050",

language = "English",

volume = "198",

journal = "American Journal of Obstetrics and Gynecology",

issn = "0002-9378",

publisher = "MOSBY-ELSEVIER",

number = "6",

note = "42nd Annual Meeting of the Society-for-the-Study-of-Inborn-Errors-of-Metabolism ; Conference date: 06-09-2005 Through 09-09-2005",

}

TY - JOUR

T1 - Pregnancies in glycogen storage disease type Ia

AU - Martens, Danielle 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 - 2008/6

Y1 - 2008/6

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.

KW - complications

KW - glucose

KW - glycogen storage disease type Ia

KW - pregnancy

KW - RENAL-FUNCTION

KW - ESGSD-I

KW - MANAGEMENT

KW - CREATININE

U2 - 10.1016/j.ajog.2007.11.050

DO - 10.1016/j.ajog.2007.11.050

M3 - Article

SN - 0002-9378

VL - 198

JO - American Journal of Obstetrics and Gynecology

JF - American Journal of Obstetrics and Gynecology

IS - 6

M1 - ARTN 646.e1

T2 - 42nd Annual Meeting of the Society-for-the-Study-of-Inborn-Errors-of-Metabolism

Y2 - 6 September 2005 through 9 September 2005

ER -

Pregnancies in glycogen storage disease type Ia

Abstract

Keywords

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