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Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study

The porphyrias comprise a heterogeneous group of rare, primarily hereditary, metabolic diseases caused by a partial deficiency in one of the eight enzymes involved in the heme biosynthesis. Our aim was to assess whether acute or cutaneous porphyria has been associated with excess risks of adverse pr...

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Autores principales: Tollånes, Mette Christophersen, Aarsand, Aasne Karine, Sandberg, Sverre
Formato: Texto
Lenguaje:English
Publicado: Springer Netherlands 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3026662/
https://www.ncbi.nlm.nih.gov/pubmed/20978938
http://dx.doi.org/10.1007/s10545-010-9231-2
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author Tollånes, Mette Christophersen
Aarsand, Aasne Karine
Sandberg, Sverre
author_facet Tollånes, Mette Christophersen
Aarsand, Aasne Karine
Sandberg, Sverre
author_sort Tollånes, Mette Christophersen
collection PubMed
description The porphyrias comprise a heterogeneous group of rare, primarily hereditary, metabolic diseases caused by a partial deficiency in one of the eight enzymes involved in the heme biosynthesis. Our aim was to assess whether acute or cutaneous porphyria has been associated with excess risks of adverse pregnancy outcomes. A population-based cohort study was designed by record linkage between the Norwegian Porphyria Register, covering 70% of all known porphyria patients in Norway, and the Medical Birth Registry of Norway, based on all births in Norway during 1967–2006. The risks of the adverse pregnancy outcomes preeclampsia, delivery by caesarean section, low birth weight, premature delivery, small for gestational age (SGA), perinatal death, and congenital malformations were compared between porphyric mothers and the rest of the population. The 200 mothers with porphyria had 398 singletons during the study period, whereas the 1,100,391 mothers without porphyria had 2,275,317 singletons. First-time mothers with active acute porphyria had an excess risk of perinatal death [adjusted odds ratio (OR) 4.9, 95% confidence interval (CI) 1.5–16.0], as did mothers with the hereditable form of porphyria cutanea tarda (PCT) (3.0, 1.2–7.7). Sporadic PCT was associated with an excess risk of SGA [adjusted relative risk (RR) 2.0, 1.2–3.4], and for first-time mothers, low birth weight (adjusted OR 3.4, 1.2–10.0) and premature delivery (3.5, 1.2–10.5) in addition. The findings suggest women with porphyria should be monitored closely during pregnancy.
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spelling pubmed-30266622011-02-22 Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study Tollånes, Mette Christophersen Aarsand, Aasne Karine Sandberg, Sverre J Inherit Metab Dis Original Article The porphyrias comprise a heterogeneous group of rare, primarily hereditary, metabolic diseases caused by a partial deficiency in one of the eight enzymes involved in the heme biosynthesis. Our aim was to assess whether acute or cutaneous porphyria has been associated with excess risks of adverse pregnancy outcomes. A population-based cohort study was designed by record linkage between the Norwegian Porphyria Register, covering 70% of all known porphyria patients in Norway, and the Medical Birth Registry of Norway, based on all births in Norway during 1967–2006. The risks of the adverse pregnancy outcomes preeclampsia, delivery by caesarean section, low birth weight, premature delivery, small for gestational age (SGA), perinatal death, and congenital malformations were compared between porphyric mothers and the rest of the population. The 200 mothers with porphyria had 398 singletons during the study period, whereas the 1,100,391 mothers without porphyria had 2,275,317 singletons. First-time mothers with active acute porphyria had an excess risk of perinatal death [adjusted odds ratio (OR) 4.9, 95% confidence interval (CI) 1.5–16.0], as did mothers with the hereditable form of porphyria cutanea tarda (PCT) (3.0, 1.2–7.7). Sporadic PCT was associated with an excess risk of SGA [adjusted relative risk (RR) 2.0, 1.2–3.4], and for first-time mothers, low birth weight (adjusted OR 3.4, 1.2–10.0) and premature delivery (3.5, 1.2–10.5) in addition. The findings suggest women with porphyria should be monitored closely during pregnancy. Springer Netherlands 2010-10-27 2011 /pmc/articles/PMC3026662/ /pubmed/20978938 http://dx.doi.org/10.1007/s10545-010-9231-2 Text en © The Author(s) 2010 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.
spellingShingle Original Article
Tollånes, Mette Christophersen
Aarsand, Aasne Karine
Sandberg, Sverre
Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
title Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
title_full Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
title_fullStr Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
title_full_unstemmed Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
title_short Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
title_sort excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3026662/
https://www.ncbi.nlm.nih.gov/pubmed/20978938
http://dx.doi.org/10.1007/s10545-010-9231-2
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