Antenatal and Intrapartum care of a pregnant woman with glycogen storage disease type 1a

Lewis, Rachael; Scrutton, Mark; Lee, Philip; Standen, Graham R.; Murphy, Deirdre J.

doi:10.1016/j.ejogrb.2004.04.010

Cited by 12 publications

(8 citation statements)

References 5 publications

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“…[9][10][11][12][13][14] Reports concerning the dietary management during pregnancy and the effect of pregnancy on adenomas and renal function in GSD Ia patients are not available at all. In this study, we describe the course and management of 15 pregnancies in 11 GSD Ia patients, with special focus on dietary treatment, metabolic control and the course of adenomas and renal function during pregnancy.…”

Section: Introductionmentioning

confidence: 99%

Pregnancies in glycogen storage disease type Ia

Martens

Rake

Schwarz

et al. 2008

American Journal of Obstetrics and Gynecology

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Objective-Reports on pregnancies in women with GSD-Ia are scarce. Due to improved life expectancy, pregnancy is becoming an important issue. We describe 15 pregnancies focusing on dietary treatment, biochemical parameters and GSD-Ia complications.Study Design-Carbohydrate requirements (mg/kg/min), triglyceride and uric acid levels, liver ultrasonography and creatinine clearance were investigated before, during and after pregnancy. Data of the newborns were obtained from the records.Results-In the first trimester, a significant increase in carbohydrate requirements was observed (p=0,007). Most patients had acceptable triglyceride and uric acid levels during pregnancy. No increase in size/number of adenomas was seen. In 3/4 patients, a decrease in GFR was observed after pregnancy. In three pregnancies, lactic acidosis developed during delivery with severe multiorgan failure in one. All but one of the children are healthy and show good psychomotor development. Conclusion-Successful pregnancies are possible in GSD-Ia patients, although specific GSD-Ia related risks are present.

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Section: Introductionmentioning

confidence: 99%

Pregnancies in glycogen storage disease type Ia

Martens

Rake

Schwarz

et al. 2008

American Journal of Obstetrics and Gynecology

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“…As described in previous reports,13, 14 if the patient is compliant with diet, nasogastric and nocturnal feeding are the best ways to achieve a successful pregnancy. In other cases, as described in this article, insufficient dietary compliance may result in severe complications for both mother and child, and liver transplantation might be the right therapeutic option 10, 15…”

Section: Discussionmentioning

confidence: 85%

“…It has been reported that the fertility of GSD Ia patients is poor as a result of the high incidence of polycystic ovaries and the effects of chronic disease 10. Metabolic abnormalities can compromise fetal outcome, and even though hypoglycemic episodes can be asymptomatic and undetected (as in the case of a child of diabetic mother), it can lead to fetal death 10, 13, 14. Other potential complications are deteriorating hypertension and the development of preeclampsia 15.…”

Section: Discussionmentioning

confidence: 99%

Combined liver-kidney transplantation in glycogen storage disease Ia: A case beyond the guidelines

et al. 2007

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Glycogen storage disease type Ia (GSD Ia) is a rare metabolic disorder due to hepatic glucose-6-phosphatase deficiency. Although great progress has been made in managing affected patients, severe hypoglycemia, lactic acidosis, hyperlipidemia, hepatic cytolysis, and impaired kidney function are frequent. Liver transplantation is the only radical treatment, for which the main indications are hepatic adenomatosis, hepatocellular carcinoma, or severe hepatic dysfunction. We present the case of a patient with end-stage renal disease without focal hepatic lesions and with moderate hepatic metabolic control, and we explain how combined liver-kidney transplantation (LKT) made it possible to correct the metabolic defects responsible for the impaired glucose homeostasis, liberalize the diet, and give birth to a healthy child after an uneventful pregnancy. Patients with end-stage renal disease that resulted from GSD Ia should be considered for LKT even in the absence of hepatic lesions with the aim of improving their quality of life. Liver Transpl 13:762-764, 2007.

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“…In 2008 Martens DH et al described 11 GSD Ia patients who have had pregnancies, three out of seven women, whose cases were investigated, presented with PCOs, but only one of them required folliclestimulating hormone therapy to conceive (Martens et al 2008). Previously, Johnson MP et al reported a triplet gestation induced by clomifene in a patient with GSD Ia, no other cases of recourse to ovarian stimulation to achieve pregnancy has been signalled in the other four women with GSD Ia, and the three women with GSD Ib who had the other pregnancies reported, until now, in the literature (Johnson et al 1990;Faber et al 1976;Ryan et al 1994;Lee et al 2004;Lewis et al 2004;Dagli et al 2010). In our population, only one woman was unable to conceive after more than 12 months of trying, but it must be noted that she also suffered from other endometrial problems not GSD related; on the other hand, all the other patients who wished to become pregnant conceived spontaneously within 12 months, and unplanned pregnancies were also reported.…”

Section: Discussionmentioning

confidence: 99%