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Antenatal manifestations of mitochondrial disorders.

Authors :
Tavares MV
Santos MJ
Domingues AP
Pratas J
Mendes C
Simões M
Moura P
Diogo L
Grazina M
Source :
Journal of inherited metabolic disease [J Inherit Metab Dis] 2013 Sep; Vol. 36 (5), pp. 805-11. Date of Electronic Publication: 2013 Jan 30.
Publication Year :
2013

Abstract

Mitochondrial respiratory chain diseases are a heterogeneous group of pathologies caused by genetic alterations affecting mitochondrial energy production. Theoretically, this deficiency may lead to any symptoms, in any organ or tissue, at any age even before birth. The aim of our study was to identify the frequency and characterize antenatal manifestations identifying possible associations between mitochondrial disease and more specific and earlier manifestation. We retrospectively review the files of 44 paediatric subjects with genetic and biochemical alterations of respiratory chain identified in the first decade of life and compare data with a control group (n = 88). Our results show that maternal age was similar in both groups. The female gender was predominant in patients group. Gestational age at delivery was similar in both groups. Concerning birth weight, it was significantly lower (p = 0.001) in patients (2899.9 ± 538.3 vs. 3246.6 ± 460.2 g). Fifteen pregnancies of the patients group were considered abnormal. Our findings show that intrauterine growth restriction was the most frequent antenatal feature observed. Neonatal morbidity was significantly higher (fivefold) in patients (p < 0.001). The clinical findings are independent of the molecular defect type. Our results are preliminary and more studies are needed, in order to learn more about mitochondrial physiology and activity in embryological development for the assessment of mitochondrial disease progress in fetal life. However, the present work is a significant contribution, given the scarcity of information in this field.

Details

Language :
English
ISSN :
1573-2665
Volume :
36
Issue :
5
Database :
MEDLINE
Journal :
Journal of inherited metabolic disease
Publication Type :
Academic Journal
Accession number :
23361304
Full Text :
https://doi.org/10.1007/s10545-012-9567-x