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Placental steroid sulphatase deficiency: an approach to antenatal care and delivery.

ABSTRACT: Placental steroid sulphatase deficiency (SSD) is an X-linked inborn error of metabolism. Congenital X-linked ichthyosis (XLI) is a genetic disorder of keratinisation caused by steroid sulphatase (STS) deficiency, which results in a scaling skin condition in male infants shortly after birth. It may be associated with failed induction of labor and prolonged labor leading to cesarean delivery due to 'cervical dystocia'. We present two cases of congenital ichthyosis. Thorough counselling of women with a previously affected pregnancy during the antenatal period should include discussion about mode of delivery and a critical review of the complexities of prenatal diagnosis in this condition. We propose a clinical management pathway to offer women with a previous pregnancy affected by this rare condition. SIMILAR CASES PUBLISHED: Less than 50 cases reported.

SUBMITTER: Dreyer FE

PROVIDER: S-EPMC6302985 | biostudies-other | 2018 Nov-Dec

REPOSITORIES: biostudies-other

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Placental steroid sulphatase deficiency: an approach to antenatal care and delivery.

Dreyer Frieda-Elsje FE Abdulrahman Ganiy Opeyemi GO Waring Gareth G Hinshaw Kim K

Annals of Saudi medicine 20181101 6

Placental steroid sulphatase deficiency (SSD) is an X-linked inborn error of metabolism. Congenital X-linked ichthyosis (XLI) is a genetic disorder of keratinisation caused by steroid sulphatase (STS) deficiency, which results in a scaling skin condition in male infants shortly after birth. It may be associated with failed induction of labor and prolonged labor leading to cesarean delivery due to 'cervical dystocia'. We present two cases of congenital ichthyosis. Thorough counselling of women wi ...[more]

PMID: 30531180

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Project description:Antenatal care reduces maternal and perinatal mortality and morbidity through the detection and treatment of some conditions, but its coverage is less than optimal within certain populations. Supply kits for maternal health were designed to overcome barriers present when providing care during pregnancy and childbirth particularly to women from underserved population.We conducted a mixed-methods systematic review on the use of supply kits. This manuscript presents the findings from qualitative studies that reported barriers, facilitators, and user's recommendation in the adoption and implementation of any type of kit designed to be used during pregnancy or childbirth.This review included eight studies, and seven were implemented in developing countries. Most studies assessed the implementation of clean delivery kits to be used during labour and delivery, and contributed to gain insights into factors that may hinder or foster the use of kits.Clean delivery kits were conceived to cope with barriers related mainly to access. The most important barrier identified were those related to the socio-cultural and the lack of knowledge dimension such as who held the decision-making authority in the household, as well as popular beliefs behind the idea that birth preparation could bring bad luck, may prevent clients from adhering to their use. In addition, financial constraints and limited understanding of the instructions of use were accessibility barriers found. On the other hand, once used, clean delivery kits for maternal health were accepted by women and health workers. Convenience, hygienic components, and avoidance of delays in receiving care were viewed as satisfactory features.Supply kits are mostly affordable and easily deployable. Increasing awareness among the population about the offered kits and providing information on their benefits emerges as a critical step to foster use in settings where kits are available. Implementation of this strategy requires low complexity resources and could make the use of kits an accepted alternative to increase the use of evidence-based interventions and thus improve quality of care during pregnancy, childbirth and neonatal period mainly at the community level in low income countries and remote areas with low access.

Project description:ObjectivesUptake of maternal health services remains suboptimal in Ethiopia. Significant proportions of antenatal care attendees give birth at home. This study was conducted to identify the predictors of non-institutional delivery among women who received antenatal care in the Southern Nations Nationalities and Peoples Region, Ethiopia.DesignA community-based cross-sectional survey was conducted among women who delivered in the year preceding the survey and who had at least one antenatal visit. Multistage cluster sampling was deployed to select 2390 women from all administrative zones of the region. A mixed-effects multivariable logistic regression analysis was performed to assess the predictors of non-institutional delivery; adjusted ORs (AOR) with 95% CIs are reported.ResultsThe proportion of non-institutional deliveries among participants was 62.2% (95% CI 60.2% to 64.2%). Previous experience of short and simple labour (46.9%) and uncomplicated home birth (42.9%), night-time labour (29.7%), absence of pregnancy-related problem (18.8%) and perceived providers poor reception of women (17.8%) were the main reasons to have non-institutional delivery. Attending secondary school and above (AOR=0.51; 95% CI 0.30 to 0.85), being a government employee (AOR=0.27; 95% CI 0.10 to 0.78) and woman's autonomy in healthcare utilisation decision making (AOR=0.51; 95% CI 0.33 to 0.79) were among the independent predictors negatively associated with non-institutional delivery. On the other hand, unplanned pregnancy (AOR=1.67; 95% CI 1.16 to 2.42), not experiencing any health problem during pregnancy (AOR=8.1; 95% CI 3.12 to 24.62), not perceiving the risks associated with home delivery (AOR=6.64; 95% CI 4.35 to 10.14) were the independent predictors positively associated with non-institutional delivery.ConclusionsThere is a missed opportunity among women attending antenatal care in southern Ethiopia. Further health system innovations that help to bridge the gap between antenatal care attendance and institutional delivery are highly recommended.

Project description:INTRODUCTION: Despite the international emphasis in the last few years on the need to address the unmet health needs of pregnant women and children, progress in reducing maternal mortality has been slow. This is particularly worrying in sub-Saharan Africa where over 162,000 women still die each year during pregnancy and childbirth, most of them because of the lack of access to skilled delivery attendance and emergency care. With a maternal mortality ratio of 673 per 100,000 live births and 19,000 maternal deaths annually, Ethiopia is a major contributor to the worldwide death toll of mothers. While some studies have looked at different risk factors for antenatal care (ANC) and delivery service utilisation in the country, information coming from community-based studies related to the Health Extension Programme (HEP) in rural areas is limited. This study aims to determine the prevalence of maternal health care utilisation and explore its determinants among rural women aged 15-49 years in Tigray, Ethiopia. METHODS: The study was a community-based cross-sectional survey using a structured questionnaire. A cluster sampling technique was used to select women who had given birth at least once in the five years prior to the survey period. Univariable and multivariable logistic regression analyses were carried out to elicit the impact of each factor on ANC and institutional delivery service utilisation. RESULTS: The response rate was 99% (n=1113). The mean age of the participants was 30.4 years. The proportion of women who received ANC for their recent births was 54%; only 46 (4.1%) of women gave birth at a health facility. Factors associated with ANC utilisation were marital status, education, proximity of health facility to the village, and husband's occupation, while use of institutional delivery was mainly associated with parity, education, having received ANC advice, a history of difficult/prolonged labour, and husbands' occupation. CONCLUSIONS: A relatively acceptable utilisation of ANC services but extremely low institutional delivery was observed. Classical socio-demographic factors were associated with both ANC and institutional delivery attendance. ANC advice can contribute to increase institutional delivery use. Different aspects of HEP need to be strengthened to improve maternal health in Tigray.

Dataset Information

Placental steroid sulphatase deficiency: an approach to antenatal care and delivery.

Publications

Placental steroid sulphatase deficiency: an approach to antenatal care and delivery.

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