Pregnancy in GNE myopathy patients: a nationwide repository survey in Japan
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(2020) 15:245
RESEARCH
Open Access
Pregnancy in GNE myopathy patients: a nationwide repository survey in Japan Wakako Yoshioka1,2, Naoyuki Miyasaka3, Ryo Okubo4, Reiko Shimizu5, Yuji Takahashi6, Yuriko Oda7, Ichizo Nishino1, Harumasa Nakamura5 and Madoka Mori-Yoshimura6*
Abstract Background: GNE myopathy is an autosomal recessive adult-onset distal myopathy. While a few case reports have described the progression of GNE myopathy during pregnancy, to our knowledge, none have examined disease progression after delivery or obstetric complications. Objective: This study aimed to reveal maternal complications, newborn complications, and the impact of pregnancy on disease progression in GNE myopathy patients. Methods: We conducted a questionnaire survey on pregnancy, delivery, and newborns involving female GNE myopathy patients who are currently registered in a national registry in Japan. Results: The response rate for the questionnaire survey was 60.0% (72/120). Of the 72 respondents, 44 (61.1%) had pregnancy experience (average, 1.8 pregnancies; 53 pregnancies before onset and 28 after onset). The incidence of threatened abortion was 26.9% among post-onset pregnancies, which was higher compared to those of the general Japanese population (p = 0.03). No other maternal or infant complications were commonly observed. Over 80% were unaware of changes in disease progression during pregnancy (mean age, 32.8 ± 3.5 years) or after delivery (32.9 ± 3.8 years), while 19.0% experienced disease exacerbation within a year after delivery (30.0 ± 1.0 years). Six patients developed myopathy within a year after delivery (29.7 ± 4.6 years), while none developed myopathy during pregnancy. Conclusions: There were no serious maternal or newborn complications, and subjective progression did not differ during or after delivery in the majority of GNE myopathy patients. However, our findings suggest the importance of considering the possibility of threatened abortion and disease progression after delivery. Keywords: GNE myopathy, Pregnancy, Delivery, Threatened abortion, Disease progression
* Correspondence: [email protected] 6 Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry (NCNP), 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8502, Japan Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds
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