Alagille Syndrome: A case report of a rare oral and multisystem manifestation

Alagille syndrome is an inherited multisystem disorder of autosomal dominant transmission. Its prevalence is estimated at 1 per 70,000 to 100,000 live births. We report the case of a young patient suffering from Alagille syndrome who consulted the center of diagnosis and dental treatment of Rabat - MOROCCO (CCTD). The general manifestations are facial dysmorphia, hepatic, cardiac, and ocular disorders. Hepatic cholestasis causes oral repercussions such as a yellow oral mucosa, hypomineralization of the teeth, and a high tendency to dental caries. The management of such a patient requires the knowledge of the general health of the patient, therefore collaboration with the attending physicians, the establishment of rigorous oral hygiene, personalized prophylaxis with a consequent contribution of fluorine.

Prevalence of individual brain and eye defects potentially related to Zika virus in pregnancy in 22 U.S. states and territories, January 2016 - June 2017

During the Centers for Disease Control and Prevention’s Zika Virus Response, birth defects surveillance programs adapted to monitor birth defects potentially related to Zika virus (ZIKV) infection during pregnancy. Pregnancy outcomes occurring during January 2016-June 2017 in 22 U.S. states and territories were used to estimate the prevalence of those brain and eye defects potentially related to ZIKV. Jurisdictions were divided into three groups: areas with widespread ZIKV transmission, areas with limited local ZIKV transmission, and areas without local ZIKV transmission. Prevalence estimates for selected brain and eye defects and microcephaly per 10,000 live births were estimated. Prevalence ratios (PRs) and 95% confidence intervals (CIs) were estimated using Poisson regression for areas with widespread and limited ZIKV transmission compared to areas without local ZIKV transmission. Defects with significantly higher prevalence in areas of widespread transmission were pooled, and PRs were calculated by quarter, comparing subsequent quarters to the first quarter (January – March 2016). Nine defects had significantly higher prevalence in areas of widespread transmission. The highest PRs were seen in intracranial calcifications (PR=12.6, 95% CI [7.4, 21.3]), chorioretinal abnormalities (12.5 [7.1, 22.3]), brainstem abnormalities (9.3, [4.7, 18.4]), and cerebral/cortical atrophy (6.7, [4.2, 10.8]). The PR of the nine pooled defects was significantly higher in three quarters in areas with widespread transmission. The largest difference in prevalence was observed for defects consistently reported in infants with congenital ZIKV infection. Birth defects surveillance programs could consider monitoring a subset of birth defects potentially related to ZIKV in pregnancy.

Trends in the Incidence of New‐Onset Hypertensive Disorders of Pregnancy Among Rural and Urban Areas in the United States, 2007 to 2019

Background Hypertensive disorders of pregnancy are growing public health problems that contribute to maternal morbidity, mortality, and future risk of cardiovascular disease. Given established rural‐urban differences in maternal cardiovascular health, we described contemporary trends in new‐onset hypertensive disorders of pregnancy in the United States. Methods and Results We conducted a serial, cross‐sectional analysis of 51 685 525 live births to individuals aged 15 to 44 years from 2007 to 2019 using the Centers for Disease Control and Prevention Natality Database. We included gestational hypertension and preeclampsia/eclampsia in individuals without chronic hypertension and calculated the age‐adjusted incidence (95% CI) per 1000 live births overall and by urbanization status (rural or urban). We used Joinpoint software to identify inflection points and calculate rate of change. We quantified rate ratios to compare the relative incidence in rural compared with urban areas. Incidence (95% CI) of new‐onset hypertensive disorders of pregnancy increased from 2007 to 2019 in both rural (48.6 [48.0–49.2] to 83.9 [83.1–84.7]) and urban (37.0 [36.8–37.2] to 77.2 [76.8–77.6]) areas. The rate of annual increase in new‐onset hypertensive disorders of pregnancy was more rapid after 2014 with greater acceleration in urban compared with rural areas. Rate ratios (95% CI) comparing incidence of new‐onset hypertensive disorders of pregnancy in rural and urban areas decreased from 1.31 (1.30–1.33) in 2007 to 1.09 (1.08–1.10) in 2019. Conclusions Incidence of new‐onset hypertensive disorders of pregnancy doubled from 2007 to 2019 with persistent rural‐urban differences highlighting the need for targeted interventions to improve the health of pregnant individuals and their offspring.

Assessment of Women’s Experience of Care during Childbirth for Quality Improvement at Level 5 Health Facilities in Bungoma County, Kenya

High maternal and newborn mortality is a pressing problem in developing countries. Poor treatment during childbirth contributes directly and indirectly to this problem. Many women experience disrespectful and abusive treatment during childbirth worldwide which violates their rights. In Kenya, 20% of women report having experienced some form of disrespect and abuse (D&A). Bungoma County is among the 15 counties with the worst maternal and newborn health statistics in Kenya. The maternal mortality rate is 382 per 100,000 live births and newborn deaths 32 per 1,000 live births, while skilled birth attendance is 41.4%. This study was motivated by the poor maternal and newborn indicators, rising incidences of D&A, limited formal research on respectful maternity care. The study aimed at assessing women’s experience of care during childbirth at Level 5 health facilities in Bungoma County. The specific objectives were to determine the women’s experiences of care during childbirth, to determine factors contributing to disrespect and abuse during childbirth and to identify strategies for addressing issues affecting respectful maternity care for promoting quality of maternal and newborn care. A cross-sectional descriptive study design was used. It involved 360 mothers. Analysis of quantitative data was done using SPSS. Descriptive statistics were presented in graphs, tables, frequencies and percentages. Qualitative data was analysed thematically. The prevalence of D&A was 42.2%, younger age and lower education aggravated D&A. Autonomy, privacy and confidentiality, and absence of birth companionship were major aspects of D&A. Health workforce shortage, inadequate supervision, space and beds, poor provider-patient relationships were factors leading to D&A. It was concluded that there is a need for increased incorporation of Respectful Maternity Care (RMC) in routine care, deploy more staff, avail equipment and supplies, and enhance support supervision. The study information intends to assist stakeholders in prioritising policy actions for improving the quality of maternal and newborn health outcomes and indicators.

Determinants of fetal macrosomia among live births in southern Ethiopia: a matched case-control study

The full text of this preprint has been withdrawn by the authors due to author disagreement with the posting of the preprint. Therefore, the authors do not wish this work to be cited as a reference. Questions should be directed to the corresponding author.

Natural history and epidemiology of cleft lip and palate: a registry-based study in Iran (2000-19)

The aim of this study was to provide the natural history and epidemiology of cleft lip and cleft palate in the northwest region of Iran between 2000 and 2019. Since 2000, infants born with birth defects have been registered in the Tabriz Registry of Congenital Anomalies (TRoCA). For this study, the information and data were collected using the TRoCA registry system. Prevalence of cleft lip and cleft palate was 1.48 (95% CI 1.34; 1.62) per 1000 live births over the past two decades in the region. The occurrence of cleft lip and cleft palate was more common in males than females. The fetal death ratio was 5 percent of live born children. The proportion of infants with cleft lip and cleft palate surviving to the second week was 54 percent. The results may have a role in planning and evaluating the strategies for primary prevention of cleft lip and cleft palate, particularly in high-risk populations.

Reduction in Lifetime Fertility Through MNCS in Rural Bangladesh

This study assesses the effect of a customised Maternal Neonatal and Child Survival (MNCS) intervention in the rural areas of Bangladesh. This study attempts to estimate the lifetime fertility rate and the proportion of live births ≥3, and the age-specific lifetime fertility patterns among the women of reproductive age. This quasi-experimental study used impact evaluation data from the MNCS intervention in 2013 and compared these with the baseline data collected in 2008. We used a multi-stage, cluster random sampling technique to include 6,000 and 4,800 women in 2008 and 2013, respectively. The respondents were either mothers who had alive/deceased infants or the mothers whose pregnancy was terminated or who had living children of 12–59 months without pregnancy outcomes in the preceding year of the surveys. Based on the mean difference of live births from baseline to endline year for each intervention union, and then we compared these two areas (intervention and control unions). Overall lifetime fertility rate declined significantly in high-performing intervention unions (from 2.6 to 2.2/woman, p < .001) or in control unions (from 2.4 to 2.2/woman; p < .001). The degree of reduction of fertility increased significantly with age, and such a change was most prominent in the case of women ≥35 years old. Multivariate analyses suggest that the likelihood of having live births ≥3 reduced significantly in high-performed intervention compared to control unions. In conclusion, the probability of reducing lifetime fertility over time increases with a higher level of access, degree and duration of the customised intervention.

Congenital anomalies in Santa Catarina: case distribution and trends in 2010–2018

Objective: To evaluate the distribution of cases of congenital anomalies in the state of Santa Catarina by health macro-region, to determine the frequency according to maternal and neonatal variables, to estimate the related mortality, and the trends in the period 2010–2018. Methods: An ecological time-series study with secondary data on congenital anomalies and the sociodemographic and health variables of mothers and newborns living in Santa Catarina, from 2010 to 2018. For temporal trend analysis, generalized linear regression was performed using the Prais-Winsten method with robust variance. Results: The average prevalence of congenital anomalies in the period was 8.9 cases per 1,000 live births, being 9.4 cases by 1,000 live births in 2010 and, in 2018, 8.2/1,000. The trend remained stable in the analyzed period. The major malformations were musculoskeletal, hip, and foot malformations, with a proportion ≥30%. There was a higher prevalence of congenital anomalies in low birthweight, preterm, male livebirths with Apgar≤7, born by cesarean section, mothers of older age (≥40 years), and less educated (less than eight years of study). Infant mortality due to congenital malformations was 2.6 deaths/1,000 live births, representing about 25.8% of the total infant deaths in the period. Conclusions: The frequency of congenital anomalies and the mortality with anomalies was stable in the studied period in Santa Catarina. The presence of anomalies was associated with low birth weight, prematurity, and low Apgar score. The highest proportion of congenital anomalies was in the musculoskeletal system.

Early neonatal sepsis: prevalence, complications and outcomes in newborns with 35 weeks of gestational age or more

ABSTRACT Objective: To analyze the incidence, complications, and hospital discharge status in newborns with ≥35 weeks of gestational age with early neonatal sepsis. Methods: This is a cross-sectional, retrospective study. Cases of early-onset sepsis registered from January 2016 to December 2019 in neonates with gestational age of 35 weeks or more were reviewed in a level III neonatal unit. The diagnoses were performed based on the criteria by the Brazilian Health Regulatory Agency (Anvisa), and the episodes were classified according to microbiological classification and site of infection. The following complications were evaluated: shock, coagulation disorders, and sequelae of the central nervous system. The conditions at hospital discharge were also assessed. The collected data were analyzed with the descriptive analysis. Results: In the period, early neonatal sepsis occurred in 46 newborns, corresponding to 1.8% of all newborns admitted to the neonatal unit, with a prevalence of 4/1,000 live births. Culture confirmed sepsis ocurred in three patients (0.3/1,000 live births), with the following agents: S. pneumoniae, S. epidermidis and S. agalactiae. As to site of infection, there were 35 cases of primary bloodstream infection, seven cases of pneumonia and four cases of meningitis. Most patients (78.3%) had at least one risk factor for sepsis, and all were symptomatic at admission. There were no deaths. Complications occurred in 28.2% of the cases, especially shock (10 cases – 21.7%). Conclusions: The prevalence of proven early neonatal sepsis was low. Despite the common occurrence of complications, there were no deaths.