Pregnancy in Women With Glycogen Storage Disease Ia and Ib

Ferrecchia, Iris A.; Guenette, Ginny; Potocik, Elizabeth A.; Weinstein, David A.

doi:10.1097/jpn.0000000000000017

Cited by 20 publications

(18 citation statements)

References 25 publications

(18 reference statements)

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“…In the past, fertility among GSD women was also reported to be a challenge . With improvements in care, puberty and reproduction are now all normal, and over 100 children have now been born to mothers with GSD Ia …”

Section: Discussionmentioning

confidence: 99%

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Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

et al. 2017

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Prior to 1971, type Ia glycogen storage disease was marked by life-threatening hypoglycemia, lactic acidosis, severe failure to thrive, and developmental delay. With the introduction of continuous feeds in the 1970s and cornstarch in the 1980s, the prognosis improved, but complications almost universally developed. Changes in the management of type Ia glycogen storage disease have resulted in improved metabolic control, and this manuscript reviews the increasing evidence that complications can be delayed or prevented with optimal metabolic control as previously was seen in diabetes.

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Section: Discussionmentioning

confidence: 99%

“…40 With improvements in care, puberty and reproduction are now all normal, and over 100 children have now been born to mothers with GSD Ia. 43,44 6 | CONCLUSION…”

Section: Other Complicationsmentioning

confidence: 99%

Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

et al. 2017

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“…Due to the metabolic demands of the fetus and hormonal adaptations during pregnancy, women with GSD 0 are prone to metabolic derangements with hypoglycemia, hyperketonemia and hyperlactatemia. Therefore, careful monitoring is necessary throughout pregnancy, and maternal blood glucose levels need to be maintained in a healthy range for the safety and proper development of the fetus [8]. For other types of GSDs, it has been shown that good metabolic control before conception and throughout pregnancy is directly related to successful outcomes [8].…”

Section: Discussionmentioning

confidence: 99%

“…Apart from respiratory distress, the newborn of our GSD 0 patient also showed a tendency towards hypoglycemia, which is reminiscent to that seen in newborns from mothers with gestational diabetes. In healthy pregnant women hormonal changes during pregnancy with a rise in anti-insulinergic hormones, such as human placental lactogen, progesterone, and estrogen, lead to increasing insulin resistance [8].…”

Section: Discussionmentioning

confidence: 99%

Three Successful Pregnancies in a Patient With Glycogen Storage Disease Type 0

Grünert

Hannibal

Schumann

et al. 2020

Preprint

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BackgroundGlycogen storage disease type 0 (GSD 0) is a rare inborn error of metabolism due to deficiency of the enzyme glycogen synthase (EC 2.4.1.11). Patients with this disorder are unable to store glucose as glycogen in the liver. GSD 0 is therefore characterized by ketotic fasting hypoglycemia in combination with postprandial hyperglycemia and hyperlactatemia. So far, only one pregnancy has been described in a woman with GSD 0. Case presentationWe herein report a 32 year-old patient GSD 0 with three successful pregnancies. The diagnosis of GSD 0 was made in early childhood due to characteristic symptoms. The patient had two healthy children at the time of her first visit in our metabolic centre. The diet was optimised prior to her third pregnancy with a protein-rich diet including cornstarch and protein supplements. Pregnancy was confirmed at week 6 of gestation. Dietary management was difficult during pregnancy, especially in the first trimester due to severe nausea. Labour was induced at 37 weeks of gestation due to cholestasis of pregnancy, and the patient delivered a healthy baby girl. Perinatally, the mother received a high glucose infusion to stabilize blood glucose levels. The neonate also required a glucose infusion postnatally because of impaired glucose homeostasis. Similar to diabetic fetopathy, recurrent maternal hyperglycemia may result in hyperinsulinism of the child and trigger neonatal hypoglycemia.ConclusionsAll four pregnancies in women with GSD 0 described to date occurred with minor complications and resulted in healthy offspring, which underpins the good prognosis and rather benign character of this rare metabolic disease. Careful monitoring during pregnancy and delivery is, however, necessary to minimize the risk of recurrent hypoglycemia for both mother and child.

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“…Good metabolic control prior to conception and maintained throughout gestation directly correlates with successful outcome in women with GSD [137,138].…”

Section: Glycogen Storage Diseasesmentioning

confidence: 99%

AISF position paper on liver disease and pregnancy

Morisco¹,

Bruno²,

Bugianesi³

et al. 2016

Digestive and Liver Disease

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The relationship between liver disease and pregnancy is of great clinical impact. Severe liver disease in pregnancy is rare; however, pregnancy-related liver disease is the most frequent cause of liver dysfunction during pregnancy and represents a severe threat to foetal and maternal survival. A rapid differential diagnosis between liver disease related or unrelated to pregnancy is required in women who present with liver dysfunction during pregnancy. This report summarizes the recommendation of an expert panel established by the Italian Association for the Study of the Liver (AISF) on the management of liver disease during pregnancy. The article provides an overview of liver disease occurring in pregnancy, an update on the key mechanisms involved in its pathogenesis, and an assessment of the available treatment options. The report contains in three sections: (1) specific liver diseases of pregnancy; (2) liver disease occurring during pregnancy; and (3) pregnancy in patients with pre-existing chronic liver disease. Each topic is discussed considering the most relevant data available in literature; the final statements are formulated according to both scientific evidence and clinical expertise of the involved physicians, and the AISF expert panel recommendations are reported.

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Pregnancy in Women With Glycogen Storage Disease Ia and Ib

Cited by 20 publications

References 25 publications

Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

Three Successful Pregnancies in a Patient With Glycogen Storage Disease Type 0

AISF position paper on liver disease and pregnancy

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