Pregnancy in Complement-Mediated Thrombotic Microangiopathy: Maternal and Neonatal Outcomes

Haninger-Vacariu, Natalja; Gleiß, Andreas; Gaggl, Martina; Aigner, Christof; Kain, Renate; Prohászka, Zoltán; Szilágyi, Ágnes; Csuka, Dorottya; Böhmig, Georg A.; Sunder‐Plaßmann, Raute; Sunder‐Plassmann, Gere; Schmidt, Alice

doi:10.1016/j.xkme.2023.100669

Cited by 2 publications

(1 citation statement)

References 24 publications

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“…By way of contrast, the preterm birth rate in this analysis was highest in southern Asia (13.2%) [ 15 ]. Thus, prematurity in Fabry disease is even more frequent as compared to less developed countries and compares more or less to other hereditary disease such as complement mediated thrombotic microangiopathy [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Pregnancy outcomes of Fabry disease in Austria (PROFABIA)-a retrospective cohort-study

Haninger-Vacariu,

Anastopoulos,

Aigner

et al. 2024

Orphanet J Rare Dis

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Background Pregnancy and delivery outcomes in women with Fabry disease are not well described. Methods Retrospective cohort-study of women with Fabry disease in Austria using a specific questionnaire and the Austrian Mother–Child Health Passport. Results Out of a total of 44 enrolled women (median age at study entry 44 years, p25: 30, p75: 51), 86.4% showed signs and symptoms of Fabry disease with an increase in pain burden during pregnancy, primarily in women with moderate pain before pregnancy. Thirty-two of 44 women with Fabry disease reported a total of 70 pregnancies (median age at first pregnancy 24 years, p25: 21, p75: 31), 61 (87.1%) of which resulted in 64 live births including 3 sets of twins, six miscarriages (8.6%) in five women, and three induced abortions (4.3%) in two women. Risk factors for poor maternal and foetal outcomes during pregnancy, overrepresented in our cohort as compared to the general population, were hypertension (n = 10, 16.4%), proteinuria (n = 17, 27.9%) and smoking (n = 24, 39.3%). Preeclampsia was reported in 7 pregnancies (11.5%). Fifty-one (79.7%) children were born at term and 13 (20.3%) were preterm (including one neonatal death), with a median gestational age of 39 weeks (p25: 38, p75: 40) and delivery by C-section in 15 pregnancies (24.6%). Thirteen (20.3%) children presented with low birth weight and 18 (28.1%) were small for their gestational age. In comparison to global and national data-sets, preeclampsia, prematurity, low birth weight, being small for their gestational age as well as inpatient stay were significantly more common in patients with Fabry disease. Conclusions Our cohort-study in women with Fabry disease shows an increase of pain burden during pregnancies and clearly points to an increased risk for preeclampsia, prematurity, and neonates small for gestational age. With a substantial number of high-risk pregnancies, neonatal outcomes are somewhat worse in Fabry disease than in the general public. Thus, we provide valuable data enabling informed decision-making in pregnancy counselling for Fabry disease.

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Section: Discussionmentioning

confidence: 99%