Successful in vitro fertilization, twin pregnancy and labor in a woman with inherited propionic acidemia

Wójtowicz, Anna; Hill, Melanie; Strobel, Spencer; Gillett, Godfrey T; Kieć‐Wilk, Beata

doi:10.5603/gp.2019.0114

Cited by 2 publications

(1 citation statement)

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“…There were no metabolic problems reported for these cases [38]. In general, a protein-restricted diet and carnitine supplementation were successfully employed to manage pregnancy in PA [35,38,[51][52][53]. Complications during pregnancy included growth retardation and preeclampsia [51].…”

Section: Pa/mma and Pregnancymentioning

confidence: 96%

Organic Aciduria Disorders in Pregnancy: An Overview of Metabolic Considerations

et al. 2023

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Organic acidurias are a heterogeneous group of rare inherited metabolic disorders (IMDs) caused by a deficiency of an enzyme or a transport protein involved in the intermediary metabolic pathways. These enzymatic defects lead to an accumulation of organic acids in different tissues and their subsequent excretion in urine. Organic acidurias include maple syrup urine disease, propionic aciduria, methylmalonic aciduria, isovaleric aciduria, and glutaric aciduria type 1. Clinical features vary between different organic acid disorders and may present with severe complications. An increasing number of women with rare IMDs are reporting successful pregnancy outcomes. Normal pregnancy causes profound anatomical, biochemical and physiological changes. Significant changes in metabolism and nutritional requirements take place during different stages of pregnancy in IMDs. Foetal demands increase with the progression of pregnancy, representing a challenging biological stressor in patients with organic acidurias as well as catabolic states post-delivery. In this work, we present an overview of metabolic considerations for pregnancy in patients with organic acidurias.

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