Helping rural women in Pakistan to prevent postpartum hemorrhage: A quasi experimental study

BACKGROUND: According to the Pakistan Demographic and Health Survey from 2006–2007, the maternal mortality ratio in rural areas is 319 per 100,000 live births. Postpartum hemorrhage is the leading cause of maternal deaths in Pakistan. The objectives of the study were to document the feasibility of distribution of misoprostol tablets by community-based providers mainly traditional birth attendants and acceptability and use of misoprostol by women who gave birth at home. METHODS: A quasi-experimental design, comprising intervention and comparison areas, was used to document the acceptability of providing misoprostol tablets to pregnant women to prevent postpartum hemorrhage in the rural community setting in Pakistan. Data were collected using structured questionnaires administered to women before and after delivery at home and their birth attendants. RESULTS: Out of 770 women who delivered at home, 678 (88%) ingested misoprostol tablets and 647 (84%) ingested the tablets after the birth of the neonate but prior to the delivery of the placenta. The remaining women took misoprostol tablets after delivery of the placenta. Side effects were experienced by 40% of women and were transitory in nature. Among women who delivered at home, 80% said that they would use misoprostol tablets in the future and 74% were willing to purchase them in the future. CONCLUSIONS: Self-administration of misoprostol in the home setting is feasible. Community-based providers, such as traditional birth attendants and community midwives with proper training and counseling, play an important role in reducing postpartum hemorrhage. Proper counseling and information exchange are helpful for introducing new practices in resource-constrained rural communities. Until such a time that skilled birth attendance is made more universally available in the rural setting, alternative strategies, such as training and using the services of traditional birth attendants to provide safe pregnancy care, must be considered

NEXMIF encephalopathy: an X-linked disorder with male and female phenotypic patterns

Purpose: Pathogenic variants in the X-linked gene NEXMIF (previously KIAA2022) are associated with intellectual disability (ID), autism spectrum disorder, and epilepsy. We aimed to delineate the female and male phenotypic spectrum of NEXMIF encephalopathy. / Methods: Through an international collaboration, we analyzed the phenotypes and genotypes of 87 patients with NEXMIF encephalopathy. / Results: Sixty-three females and 24 males (46 new patients) with NEXMIF encephalopathy were studied, with 30 novel variants. Phenotypic features included developmental delay/ID in 86/87 (99%), seizures in 71/86 (83%) and multiple comorbidities. Generalized seizures predominated including myoclonic seizures and absence seizures (both 46/70, 66%), absence with eyelid myoclonia (17/70, 24%), and atonic seizures (30/70, 43%). Males had more severe developmental impairment; females had epilepsy more frequently, and varied from unaffected to severely affected. All NEXMIF pathogenic variants led to a premature stop codon or were deleterious structural variants. Most arose de novo, although X-linked segregation occurred for both sexes. Somatic mosaicism occurred in two males and a family with suspected parental mosaicism. / Conclusion: NEXMIF encephalopathy is an X-linked, generalized developmental and epileptic encephalopathy characterized by myoclonic–atonic epilepsy overlapping with eyelid myoclonia with absence. Some patients have developmental encephalopathy without epilepsy. Males have more severe developmental impairment. NEXMIF encephalopathy arises due to loss-of-function variants

Asthma knowledge and approach among Pakistani family physicians and the impact of an educational programme.

OBJECTIVES: To assess asthma knowledge and approach among Pakistani family physicians and the impact of an educational programme. DESIGN: A total of 89 family physicians attending a continuing medical education programme were evaluated using a 17-item questionnaire before and after a lecture and seminar-based educational programme. Three scores—total score (TSc), knowledge subscore (KSc) and approach subscore (ASc)—were calculated before and after the course. RESULTS: A total of 82 (92%) family physicians completed the questionnaire. A minimum TSc of 9 was achieved by 57% physicians before the course; this increased to 78% after the course. Physicians with 10 years since graduation achieved a higher baseline TSc than those who had graduated 20 years previously. The latter group showed a larger post-course improvement. Analysis of individual questions disclosed that 79% of family physicians were unable to assess the empirical risk of asthma in an unborn child, 61% were unable to correctly classify/treat Stage 2 asthma and a similar number peak expiratory flow rate (PEFR) (l/min) based acute asthma exacerbation. Reassuringly, 97% correctly identified clinical signs and 87% life-threatening features of asthma. CONCLUSION: Of all family physicians who responded to the questionnaire, 57% achieved acceptable baseline standards. Deficiencies regarding areas such as genetic counselling and disease severity assessment were observed. Strengths in identifying clinical signs and life-threatening features were evident. Continuing medical education resulted in a significant improvement in the post-course assessment, regardless of the years since graduation. KEY WORDS: asthma; family physicians; knowledge; approach; continuing medical education

Effectiveness of 10-valent pneumococcal conjugate vaccine against vaccine-type invasive pneumococcal disease in Pakistan

Objective: To assess the effectiveness of 10-valent pneumococcal conjugate vaccine (PCV10) against invasive pneumococcal disease (IPD) due to vaccine serotypes of Streptococcus pneumoniae post introduction of the vaccine into the routine immunization program in Pakistan. Methods: A matched case–control study was conducted at 16 hospitals in Sindh Province, Pakistan. Children aged <5 years (eligible to receive PCV10) who presented with radiographically confirmed pneumonia and/or meningitis were enrolled as cases. PCR for the lytA gene was conducted on blood (for radiographic pneumonia) and cerebrospinal fluid (for meningitis) samples to detect S. pneumoniae. The proportion of IPD due to vaccine serotypes (including vaccine-related serogroups) was determined through serial multiplex PCR. For each case, at least five controls were enrolled from children hospitalized at the same institution, matched for age, district, and season. Results: Of 92 IPD patients enrolled during July 2013 to March 2017, 24 (26.0%) had disease caused by vaccine serotypes. Most case (87.5% of 24) and control (66.4% of 134) children had not received any PCV10 doses. The estimated effectiveness of PCV10 against vaccine-type IPD was 72.7% (95% confidence interval (CI) −7.2% to 92.6%) with at least one dose, 78.8% (95% CI −11.9% to 96.0%) for at least two doses, and 81.9% (95% CI −55.7% to 97.9%) for all three doses of vaccine. Conclusions: The vaccine effectiveness point estimates for PCV10 were high and increased with increasing number of doses. However, vaccine effectiveness estimates did not reach statistical significance, possibly due to low power. The findings indicate the likely impact of vaccine in reducing the burden of vaccine-type IPD if vaccine uptake can be improved. Keywords: Pneumococcal conjugate vaccine, Effectiveness, Invasive pneumococcal diseas

Impact of the COVID-19 pandemic on patients with paediatric cancer in low-income, middle-income and high-income countries: a multicentre, international, observational cohort study

OBJECTIVES: Paediatric cancer is a leading cause of death for children. Children in low-income and middle-income countries (LMICs) were four times more likely to die than children in high-income countries (HICs). This study aimed to test the hypothesis that the COVID-19 pandemic had affected the delivery of healthcare services worldwide, and exacerbated the disparity in paediatric cancer outcomes between LMICs and HICs. DESIGN: A multicentre, international, collaborative cohort study. SETTING: 91 hospitals and cancer centres in 39 countries providing cancer treatment to paediatric patients between March and December 2020. PARTICIPANTS: Patients were included if they were under the age of 18 years, and newly diagnosed with or undergoing active cancer treatment for Acute lymphoblastic leukaemia, non-Hodgkin's lymphoma, Hodgkin lymphoma, Wilms' tumour, sarcoma, retinoblastoma, gliomas, medulloblastomas or neuroblastomas, in keeping with the WHO Global Initiative for Childhood Cancer. MAIN OUTCOME MEASURE: All-cause mortality at 30 days and 90 days. RESULTS: 1660 patients were recruited. 219 children had changes to their treatment due to the pandemic. Patients in LMICs were primarily affected (n=182/219, 83.1%). Relative to patients with paediatric cancer in HICs, patients with paediatric cancer in LMICs had 12.1 (95% CI 2.93 to 50.3) and 7.9 (95% CI 3.2 to 19.7) times the odds of death at 30 days and 90 days, respectively, after presentation during the COVID-19 pandemic (p<0.001). After adjusting for confounders, patients with paediatric cancer in LMICs had 15.6 (95% CI 3.7 to 65.8) times the odds of death at 30 days (p<0.001). CONCLUSIONS: The COVID-19 pandemic has affected paediatric oncology service provision. It has disproportionately affected patients in LMICs, highlighting and compounding existing disparities in healthcare systems globally that need addressing urgently. However, many patients with paediatric cancer continued to receive their normal standard of care. This speaks to the adaptability and resilience of healthcare systems and healthcare workers globally