Search

The negative influence of professional hierarchies between health cadres can silence nurse-midwives and junior medical staff,6 and may even demotivate personnel from participating in MPDSR. Maternal and perinatal death surveillance and response (MPDSR) is a health systems process entailing the continuous cycle of identification, notification and review of maternal and perinatal deaths (Surveillance), followed by actions to improve service delivery and quality of care (Response).1 Before the coronavirus disease 2019 (COVID-19) pandemic, there were an estimated 4.6 million maternal and neonatal deaths and stillbirths each year.2 During the pandemic, maternal and perinatal health outcomes have worsened, especially in low- and middle-income countries,3 highlighting the urgent need to galvanise MPDSR to end preventable mortality and strengthen health systems. Overcoming the blame culture that currently impedes MPDSR implementation requires action at all levels of the health system. 1 Therefore, dual national prioritisation on the value of systems learning and quality improvement that MPDSR encompasses needs to be matched with political priority for health system investment to implement I response i , deliver improved health outcomes and reduce the number of preventable deaths. [Extracted from the article]

Background: Angelman Syndrome (AS) is a rare neurodevelopment disorder resulting from deficient expression or function of the maternally inherited allele of UBE3A gene. The aim of the study is to attempt at providing a detailed definition of neurodevelopmental profile in AS, with particular regard to motor, cognitive, communicative, behavioural and neurovisual, features by using standardized instruments. Method: A total of ten subjects aged from 5 to 11 years (4 males and 6 females) with molecular confirmed diagnosis of AS (7 15q11.2-q13 deletion and 3 UBE3A mutation) were enrolled in our study. All of them underwent an assessment protocol including neurological and neurovisual examination and the evaluation of motor (Gross Motor Function Measure Scale), cognitive (Griffiths Mental Development Scale and Uzgiris-Hunt Scale); adaptive (Vineland Adaptive Behavioural Scale); communication (MacArthur-Bates Communicative Development Inventory and video-recordings children's verbal expression), behavioural aspects (IPDDAG Scale) and neurovisual aspects. Results: All children presented motor function involvement. A severe cognitive impairment was detected with different profiles according to the test applied. In all cases, communicative disability (phonemic inventory, word/ gesture comprehension and production) and symptoms of inattention disorder were revealed. Neurovisual impairment was characterized by refractive errors, fundus oculi anomalies, strabismus and/or oculomotor dysfunction. Conclusion: AS presents a complex neurodevelopmental profile in which several aspects play a negative role in global development leading to a severe functional impairment. Intellectual disability is not the only component because neurovisual functions and behavioural disorders may worsen the global function and are needed of specific rehabilitation programs. [ABSTRACT FROM AUTHOR]