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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:BACKGROUND:Low quality and frequency of antenatal care (ANC) are associated with lower uptake of facility-based deliveries-a key intervention to reduce maternal and neonatal mortality. We implemented group ANC (G-ANC), an alternative service delivery model, in Kenya and Nigeria, to assess its impact on quality and attendance at ANC and uptake of facility-based delivery. METHODS:From October 2016‒January 2018, we conducted a facility-based, pragmatic, cluster-randomized controlled trial with 20 clusters per country. We recruited women <24 weeks gestation during their first ANC visit and enrolled women at intervention facilities who agreed to attend G-ANC in lieu of routine individual ANC. The G-ANC model consisted of five monthly 2-hour meetings with clinical assessments alongside structured gestationally specific group discussions and activities. Quality of care was defined as receipt of eight specific ANC interventions. Data were obtained through facility records and self-report during a home-based postpartum survey. Analysis was by intention to treat. FINDINGS:All women who completed follow up are included in the analysis (Nigeria: 1018/1075 enrolled women [94.7%], Kenya: 826/1013 [81.5%]). In Nigeria women in the intervention arm were more likely to have a facility-based delivery compared to those in the control arm (Nigeria: 76.7% [391/510] versus 54.1% [275/508]; aOR 2.30, CI 1.51-3.49). In both countries women in the intervention arm were more likely than those in the control arm to receive quality ANC (Nigeria: aOR 5.8, CI 1.98-17.21, p<0.001; Kenya: aOR 5.08, CI 2.31-11.16, p<0.001) and to attend at least four ANC visits (Nigeria: aOR 13.30, CI 7.69-22.99, p<0.001; Kenya: aOR 7.12, CI 3.91-12.97, p<0.001). CONCLUSIONS:G-ANC was associated with higher facility-based delivery rates in Nigeria, where those rates associated with individual ANC were low. In both Kenya and Nigeria it was associated with a higher proportion of women receiving quality ANC and higher frequency of ANC visits.

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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