Background. Literature suggests an association between in-utero SARS-CoV-2 exposure and necrotising enterocolitis (NEC). Objective. To determine whether admitted infants exposed to SARS-CoV-2 in utero had a higher NEC incidence than unexposed infants; and to describe the overall incidence of NEC during the COVID-19 pandemic to explore potential associations. Methods. The evaluation period included 1-year pre-COVID-19 (April 2019 - March 2020) and a 2-year COVID-19 period (April 2020 - March 2022). An electronic database was used to identify infants and to collect routine data. Additional databases were used to identify mothers with COVID-19 and SARS-CoV-2-exposed neonates. Results. All infants exposed to SARS-CoV-2 in utero tested negative for COVID-19 before admission, indicating no vertical transmissions. Among 32 SARS-CoV-2-exposed infants, one developed NEC (incidence 3.1%), compared with 2.5% in unexposed infants (p=0.834). NEC incidence was similar across periods (1.8% v. 2.5%; p=0.147). The median day of life of NEC diagnosis was 12 days for both periods. The sex distribution of infants with NEC was similar during both periods. Conclusion. While this study did not demonstrate a statistically significant increase in NEC incidence during the COVID-19 pandemic, a potential association cannot be excluded, owing to the potential pathophysiological mechanisms that support this hypothesis.
Background. Neonatal healthcare is a key area in reducing global child mortality. Unwell neonates are usually managed in the neonatal intensive care unit (NICU), but may also be admitted to paediatric intensive care unit (PICU). Objectives. To describe the profile of neonates admitted to a South African PICU and to identify risk factors associated with mortality. Methods. This was a prospective observational study of patients with a post-menstrual age of <44 weeks admitted to the PICU between November 2018 and October 2019. Associations with mortality were evaluated with univariate and multivariate logistic regression analyses. Results. A cohort of 266 neonates were included, accounting for 18.4% of PICU admissions. Median birth weight was 2 210 g, with an interquartile range (IQR) of 1 397 - 2 995 g. Chronological and post-menstrual age (IQR) at admission was 11 (2 - 28) days and 38 (35 - 40) weeks, respectively. The largest referral source was tertiary NICUs. Surgical admissions accounted for most patients. Congenital abnormalities occurred in 50.4% of the cohort. Neonatal mortality at ICU discharge was 10.9% compared with 3.8% in older patients (odds ratio=3.08, 95% confidence interval 1.89 - 5.02; p<0.001). The most common condition associated with mortality was congenital abnormalities, followed by necrotising enterocolitis and infections. Logistic regression analysis showed that the only variables independently associated with death/palliation were oscillatory ventilation and feeds received. Conclusion. Findings on the patient profile of this cohort may help policy-makers and unit managers improve neonatal care, with directions for further research.
Background. Hypertension significantly contributes to the global burden of cardiovascular disease, especially in low- and middle- income countries, including sub-Saharan Africa. Objective. To identify existing interventions targeting hypertension prevention among youth aged 13 - 25 years and evaluate the incorporation of precision prevention strategies. In doing so, this review seeks to guide future efforts in reducing hypertension among young populations in SSA. Methods. We conducted a scoping review aimed to identify interventions for hypertension risk reduction in SSA and map research in this area. Searches across academic databases and grey literature identified 3 301 articles. After removing duplicates, 3 089 articles were screened, with data extracted using a custom template on Covidence and analysed descriptively using narrative synthesis. Results. The review identified 6 interventions targeting hypertension prevention among youth in SSA. While some interventions reported efficacy in improving adherence to dietary guidelines and physical activity, others showed mixed results, particularly regarding substance use. Interventions varied in design, setting, and duration, with a focus on internal (psychological/knowledge-based) and external (health behaviour) conditions. None of the interventions utilised precision prevention methodologies. Conclusion. This review identified limited but diverse youth-focused interventions for NCD prevention in SSA, none specifically targeting hypertension or using precision prevention (PP). Despite structural and sociocultural barriers, tailored PP strategies, youth involvement, and theory-based frameworks show promise for enhancing engagement, sustainability, and impact in hypertension prevention for high- risk youth in SSA.
Holoprosencephaly (HPE) is a rare congenital brain malformation caused by incomplete forebrain cleavage during embryogenesis. Alobar HPE, the most severe form, is characterised by a single cerebral hemisphere and absent midline structures, accompanied by severe facial anomalies. We present the case of a neonate with alobar HPE, diagnosed postnatally, displaying facial dysmorphism, seizures and global hypertonia. Genetic testing revealed no chromosomal or significant genetic anomalies. Neuroimaging confirmed characteristic findings, including a mono ventricle and fused thalami. This report highlights the diagnostic challenges, multifactorial aetiology and the need for multidisciplinary care in managing this complex condition, emphasising its poor prognosis and associated complications.
We report a case of a 5-year-old boy with seizures and regression of speech. He had a normal and uneventful birth history and a normal developmental course before the age of 5 years. He presented for admission to the hospital at the age of 5 years, with unprovoked tonic- clonic convulsions, which were followed by marked regression in speech. An electroencephalogram (EEG) showed brain electrical patterns of abnormal electrical discharges in the temporoparietal area of language control, in keeping with Landau-Kleffner syndrome (LKS). Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) were normal. He was diagnosed with LKS and treatment with sodium valproate, risperidone and prednisone was initiated. Convulsions were controlled and he was discharged for follow-up as an outpatient. Children with LKS may present with uncommon electroclinical presentation, hence more awareness and consideration of the disease is needed in the evaluation of children with language abnormalities and/or convulsive disorders in the 3- to 9-years age group. Further research is required to clearly describe its prevalence, clinical heterogenicity and more successful treatment modalities.
Background. Sepsis is a leading cause of morbidity and mortality in children, particularly in resource-restricted settings. Early recognition and management are crucial to improving patient outcomes. Despite fairly extensive clinical exposure, paediatric registrars often manage these cases without direct supervision or time for critical reflection. Simulation-based learning experiences (SBLEs) offer a platform for mastering emergency care without risking patient safety. Objective. To develop, implement and evaluate an SBLE for paediatric registrars to enhance their confidence and learning in managing paediatric sepsis, incorporating the recently published Phoenix Sepsis Criteria and current evidence-based guidelines. Methods. An SBLE was developed according to the ADDIE instructional model. The learning experience included a prebriefing phase, a scenario execution using a SimBaby® manikin and a structured debriefing. Paediatric registrars evaluated the SBLE using the modified Simulation Effectiveness Tool (SET-M), which assessed self-reported learning and confidence across the prebriefing, simulated scenario and debriefing phases. Results. Seven registrars participated in the SBLE. The SET-M demonstrated high scores across all domains, with high median scores obtained for prebriefing (6/6), learning (17/18), confidence (16/18) and debriefing (15/15). Conclusion. This SBLE enhanced both confidence and learning related to managing paediatric sepsis as reported by participants. The simulation also provided a structured platform to introduce the Phoenix Sepsis Criteria and discuss context-appropriate care in resource-limited settings. The value of simulation in paediatric emergency training is demonstrated.
Congenital lymphoedema is a rare disorder presenting in the newborn period and infancy. It is characterised by impaired lymphatic drainage due to lymphatic vessel abnormalities, which presents at birth or within two years thereafter. We report on a 6-month-old infant with congenital lymphoedema presenting with non-pitting unilateral lower-limb swelling and various cutaneous markings. This case underscores the importance of imaging techniques such as lymphangiography and the challenging aspects of management owing to limited interventional options.
Background. The continuous rise in the prevalence of type 1 diabetes mellitus (T1DM) places pressure on countries to increase the resources needed to manage patients adequately. Studies have documented an association between poor glycaemic control and stunting, which poses a significant health concern. Minimal data are available on the prevalence of stunting in T1DM in South Africa. Objective. To establish the prevalence of stunting in a population of paediatric T1DM patients and determine contributing factors. Methods. A descriptive, cross-sectional study was conducted at the Paediatric Diabetes Clinic of the Steve Biko Academic Hospital, Pretoria. Data were collected from patient files, a questionnaire and the National Health Laboratory Service database. Stunting was defined as height-for-age Z-score (HAZ)<–2. Results. Of the 169 recruited patients, 115 were included (56.5% female). The prevalence of stunting was 10.4%. The median haemoglobin A1c (HbA1c) was 11.8%. Stunting was significantly associated with poor glycaemic control (p=0.008), older age (p=0.039), presence of comorbidities (p=0.026), underweight (p=<0.001) and food insecurity (p=0.021). Genetic factors were also associated with stunting, specifically lower paternal height (p=0.006) and decreased mid-parental height Z-score (p=0.035). Conclusion. Stunting in children and adolescents with T1DM was associated with poor glycaemic control, nutritional and socioeconomic factors, comorbidities, older age and genetic factors. The results point to a multifactorial contribution to impaired growth. To promote growth in children with T1DM, a multidisciplinary approach is essential, with a focus on optimising glycaemic control, addressing nutritional status and food insecurity, managing comorbidities and monitoring growth against genetic potential regularly.
Background. Research supports routine monitoring of blood loss, early bundled treatment of postpartum haemorrhage (PPH), and suction uterine tamponade for refractory PPH. Objectives. To implement recent research evidence and World Health Organization guidance on the prevention and management of PPH. Methods. We co-designed, with input from midwifery and medical staff, and conducted a quality-of-care improvement project at Princess Marina Hospital, Gaborone, Botswana. We conducted training and placed posters in the labour ward promoting evidence-based third-stage care with a novel checklist mnemonic, BOND (Baby skin-to-skin, Oxytocic, iNitiate blood loss monitoring, Delay cord clamping); use of the reusable MaternaWell Tray for blood loss monitoring after birth; early implementation of the MOTIVE bundle for PPH (uterine Massage, Oxytocin, Tranexamic acid, IntraVenous fluids, Examination, and, if needed, Escalation); and early use of suction tube uterine tamponade with the improvised Levin tube for refractory PPH. We provided in-service mentorship. Observations. Unstructured observations indicate good uptake of the treatment interventions. Routine blood loss monitoring has been difficult to institutionalise. Conclusion. The mnemonic-driven checklist approach is promising. Creative solutions are needed to further advance behaviour change. Training materials are available on the Obstetric Skills Library (YouTube).
Background. Conventional models of cardiotocograph (CTG) interpretation training have focused on adherence to interpretation guidelines. There is a need for training in interpreting CTGs that emphasises integrating the complete clinical picture to inform clinical decision-making and improve patient safety. Objectives. To develop and validate a CTG interpretation training programme for midwives in South Africa to address the existing inconsistencies in interpretation. Methods. This study was underpinned by the Analysis, Development, Design, Implementation and Evaluation (ADDIE) theoretical framework. The study followed an explanatory sequential mixed-methods design with the aim of exploring and describing midwives' level of knowledge of CTG interpretation and their learning needs. The learning needs identified informed the development of a training programme. A panel of nine experts participated in a virtual nominal group technique to validate the training programme and prioritise the learning units through anonymous ranking. Results. CTG interpretation training made up of the following learning units was developed and validated with the assistance of maternal and child health experts: Introduction to CTG interpretation; Technical aspects of CTG monitoring; Factors that affect the fetal heart rate during labour; Diagnosis; Clinician-woman relationship; Effective and timely communication of accurate information; Formulation of a comprehensive management plan after CTG interpretation; Using appropriate guidelines to inform decision-making; and Medicolegal hazards. Conclusion. This CTG interpretation programme differs from other conventional training programmes in that it focuses on the multifaceted nature of CTG interpretation and does not confine the training to CTG interpretation guidelines.
Background. The caesarean section (CS) rate at Queen Nandi Regional Hospital (QNRH) in northern KwaZulu-Natal Province, South frica (SA), has remained above the national average for a public hospital in SA. In view of the short-and long-term complications of CS, effective strategies are required to reduce unnecessary CS. Objectives. To identify the indications for CS and the factors that contributed to the high CS rate at QNRH, with the aim of implementing interventions to reduce unnecessary and avoidable CS without compromising mother and baby outcomes. Methods. We conducted a 3-month retrospective audit (Q1 2024) of 100 randomly selected CS files from the labour ward birth register and from the postnatal ward. The data collected included patient demographics, referring facilities, CS category, indication for CS, Robson Ten Group classification, timing of CS, and maternal and perinatal outcomes. The CS rate for Q1 2024 was compared with Q2 2024 and Q3 2024 with and without local clinic deliveries. Referrals from the local clinics for CS in Q1 2024 were compared with institutional audits from 2020 and 2023. Tables and figures were used for analysis. Institutional ethics permission was obtained for the clinical audit and publication of the data. Results. During the study period, there were 2 077 deliveries in total and 1 366 CSs, giving a CS rate of 66%. Among the 100 randomly selected files analysed, there were 1 set of twins, 5 stillbirths (3 due to abruptio placentae grade 3b), 96 live births, and no maternal deaths. Of the patients, 44% were aged >20-30 years, 41% were in their first pregnancy, 70% were referred from our local clinics, and 48% were low risk; 26% had a body mass index >40 kg/m(2), 15% were hypertensive, and 25% were HIV positive. In 47% of cases the indication for CS was fetal distress/non-reassuring cardiotocograph, followed by failed induction of labour (13%), failure to progress (6%), and breech presentation and abruptio placentae (5% each). More than 80% were urgent CSs, with more of these done during the night than during the day (37% v. 28%). A further 22% of the urgent CS were done during changeover time. Of the infants, 72% were born at a gestational age of 34-40 weeks and 80% had a birthweight of >2 500-4 000 g. All liveborn infants had a normal Apgar score. There were no maternal adverse outcomes. As expected, and owing to the short time frame for repeat in-depth analysis, there was no significant impact of CS trends when comparing Q1 2024 with Q2 and Q3 2024 for both institutional-based and population-based CS rates. Referrals from the local clinics contributed 70% towards our CS rate in Q1 2024 and remained consistently similar to 80% on average, when compared with institutional audits from 2020 (80%) and 2023 (85%). Conclusion. While measures were already in place to reduce rates of unnecessary CS at QNRH, the findings from our audit show that current strategies to reduce the CS rate will be unlikely to reach the target of 28% for public sector facilities, owing to our referral pattern and the drainage areas covered by this hospital. Despite the small sample size in this audit, evidence points towards the need for a district hospital in this subdistrict to help reduce our facility CS rate.
Background. This study presents a conceptual framework for respectful, woman-centred maternity care, integrating insights from the literature, systems theory, and critical theory. The framework incorporates healthcare institution and provider factors, ensuring dignity, respect and high-quality care throughout the maternity experience. Objectives. To outline the development process of this framework, which serves as a foundation for promoting respectful maternity care (RMC) in South Africa's public healthcare settings. Methods. A theory-generative methodology was applied, using both inductive and deductive strategies to derive key conclusions, ensuring a comprehensive approach to theory construction. Results. The framework identifies and describes essential concepts that foster synergy in promoting RMC. By integrating diverse knowledge sources, it establishes a strong theoretical foundation, linking its principles to RMC and broader quality healthcare standards. Conclusion. The conceptual framework provides a structured approach for enhancing maternity care through a woman-centred perspective. It underscores the value of combining multiple viewpoints to inform maternity care strategies, aiming to improve the overall maternal experience and healthcare outcomes.
Background. Intraventricular haemorrhage (IVH) is a common complication among very low-birthweight infants (VLBWIs), affecting similar to 20-30% of VLBWIs. Most published reports on the incidence of IVH in South Africa (SA) come from public sector hospitals, with few studies having been conducted in private sector hospitals. Objectives. To determine the incidence of IVH and factors associated with moderate to severe IVH in VLBWIs admitted to private sector hospitals. Methods. This was a retrospective study of VLBWIs admitted to neonatal intensive care units in three private hospitals in Johannesburg, SA, from 2018 to 2022. The study was a secondary analysis of data provided by the Vermont Oxford Network. VLBWIs admitted in three hospitals that gave permission for their data to be used were analysed. Incidence was determined based on all VLBWIs who had cranial ultrasound scans performed during admission. Comparisons were performed between VLBWIs with normal findings or mild IVH and those with moderate to severe IVH. Results. A total of 186 VLBWIs were admitted to the three private hospitals during the study period, of whom 135 (72.6%) had cranial ultrasound scans performed. Of these VLBWIs, 34 had some degree of IVH and 18 had moderate to severe IVH, giving an incidence of 25.2% for any IVH and 13.3% for moderate to severe IVH. The odds of having moderate to severe IVH were lower if birth was via caesarean section (CS) v. vaginal (adjusted odds ratio (aOR) 0.09; 95% confidence interval (CI) 0.02-0.45; p=0.004) and gestational age was >= 28 weeks (aOR 0.08; 95% CI 0.01-0.86; p=0.037), while odds were higher for neonates born to mothers who received antenatal magnesium sulphate (aOR 8.60; 95% CI 1.64-44.87; p=0.011). Conclusion. The incidence of IVH in VLBWIs admitted to certain private hospitals was comparable to the incidence reported in high-income countries. The odds of VLBWIs having moderate to severe IVH were lower when infants were born by CS or were of higher gestational age, but higher if the mother received antenatal magnesium sulphate. Although routine CS cannot be recommended for delivery of VLBWIs based on the study findings, as we did not review the indications for CS, some VLBWIs may benefit from being delivered by CS. The unexpected finding of magnesium sulphate being associated with high odds of moderate to severe IVH needs to be studied further.
Background. Intraventricular haemorrhage (IVH) remains a major concern as a result of the high prevalence of preterm births globally. Objectives. To identify maternal and neonatal risk factors associated with IVH, and to assess its incidence and most frequently occurring grade in a population of very low-birthweight infants (VLBWIs) at Kalafong Provincial Tertiary Hospital, South Africa (SA). Methods. This retrospective cohort study included all infants of birthweight <1 500 g admitted between 1 January 2019 and 31 December 2020. Associations between IVH and maternal and neonatal risk factors were analysed using Pearson's chi 2 test and Fisher's exact test. Risk factors significantly associated with IVH were included in a binary logistic regression model to identify independent predictors. Risk factors significantly associated with IVH (p<0.05) corresponded to a 95% confidence level. Results. IVH was diagnosed during the first week of life in 43 out of 127 VLBWIs who met the inclusion criteria. Hypoglycaemia remained the most significant independent risk factor after adjusting for potential confounders in the multivariable logistic regression: adjusted odds ratio 4.02 (95% confidence interval (CI) 1.52-10.64). The incidence of IVH was 33.9% (95% CI 25.7-42.1). Grade I IVH occurred in 83.7% of infants, grade II in 9.3% and grades III-IV in 7.0%. Conclusion. This study found an association between hypoglycaemia and IVH, but causality could not be established owing to the nature of the study. In this tertiary hospital in SA, a third of VLBWIs had IVH, mostly graded as mild.
Background. Armed conflict of over a decade in north-eastern Nigeria has led to interrupted access to antiretroviral therapy (ART) among HIV-positive children in this region. Objective. To determine the prevalence of lipid abnormalities among HIV-positive children on ART in an area experiencing prolonged armed conflict. Methods. This descriptive cross-sectional study involved 249 children aged 2 - 15 years on ART presenting at the University of Maiduguri Teaching Hospital between April 2021 and March 2022. Sociodemographic and clinical characteristics were obtained through a questionnaire, while serum lipid levels were determined from blood samples. Results. Dyslipidaemia was found in 63.1% of the study sample. Hypertriglyceridaemia was the most prevalent abnormality (38.6%), followed by hypercholesterolaemia (32.3%), high levels of low-density lipoprotein cholesterol (24.9%) and low levels of high-density lipoprotein cholesterol (21.3%). Significant associations were found between dyslipidaemia and prolonged ART (>5 years) (p<0.001), increasing clinical disease stage (p=0.031) and use of protease inhibitors (p=0.016). All patients whose treatment had been interrupted (n=55) owing to the conflict experienced treatment failure and were switched to protease inhibitor-based regimens, although treatment interruption was not significantly associated with dyslipidaemia.
Background. The COVID-19 pandemic's collateral damage has severely impacted global child health services. This study investigates its effects on neonatal services and outcomes at a tertiary hospital in Tshwane, South Africa. Objective. The main aim was to assess if decreased breastfeeding, coupled with increased infant formula use owing to COVID-19-related visitation disruptions, was associated with higher incidences of healthcare-associated infections (HAI) and necrotising enterocolitis (NEC) in the hospital's neonatal unit. Methods. As part of the Tshwane Maternal-Child COVID-19 study, we collected relevant data from three periods: pre-COVID-19, COVID-year-1 (COVID-Y1) and COVID-year-2 (COVID-Y2). Results. Exclusive breastfeeding at discharge decreased from 72.9% pre-COVID-19 to 47.0% during COVID-19. Preterm formula purchases rose by 37.5% in COVID-Y1 and 25.0% in COVID-Y2, and term formula purchases increased by 77.8% in COVID-Y1. HAI incidence increased in both COVID-Y1 and COVID-Y2 compared with pre-COVID-19 (11.6% v. 8.1% and 12.0% v. 8.1%). NEC incidence significantly increased in COVID-Y2 for infants >= 1 500 g (1.8% v. 0.7%). Conclusion. This study underscores the indirect consequences of COVID-19 parental visitation restrictions, leading to infringements of breastfeeding rights and coinciding with increased HAI and NEC incidences in the hospital's neonatal unit.
Background. Gastroschisis is associated with significant morbidity and mortality in neonates. Healthcare-associated infections (HAIs) worsen outcomes, with a high mortality, and timely management is crucial for survival. In South Africa, data regarding mortality and HAI management in neonates with gastroschisis are limited. Objectives. To evaluate the incidence of mortality and factors associated with mortality in neonates with gastroschisis admitted in the intensive care unit (ICU) and treated for HAI. Methods. A retrospective study was conducted from July 2017 to October 2023. Eligible infants were identified using intensive care admission registers and the neonatal database. Patient demographics, treatment modalities, and outcomes, including mortality, were analysed. The Score for Neonatal Acute Physiology II (SNAP-II) severity was calculated retrospectively. Results. A total of 66 neonates with gastroschisis and HAI that were admitted in the intensive care units were included in this study. Three neonates were managed for HAI twice, resulting in 69 HAI episodes. The incidence of culture-positive HAI was 77.9%, with a mortality of 84.8% per included neonate. Approximately half of the positive bacterial cultures with sensitivity testing were resistant to at least three classes of antimicrobial drugs. A SNAP-II score >= 30 was associated with higher mortality (p=0.038). Inotropic support was required in 76.8% of cases, correlating with higher mortality (p<0.001). Conclusion. HAI remains a major cause of mortality in neonates with gastroschisis. Higher SNAP-II scores and inotropic support were associated with poor outcome. The high incidence of multidrug-resistant pathogens highlights the need for tailored antimicrobial strategies. Further research is needed to improve survival rates in this high-risk populations.
Background. There is limited information on whether the period of highest movement restrictions during the COVID-19 pandemic impacted on antiretroviral coverage for supporting safe breastfeeding among HIV-exposed children. Objectives. To (i) measure the prevalence of breastfeeding among HIV-exposed children during peak COVID-19 lockdown (March-June 2020); and (ii) explore its association with antiretroviral coverage and other factors. Methods. A cross-sectional telephonic survey was conducted (September to November 2020), among women living with HIV (WLHIV) from rural Ehlanzeni District, South Africa (SA). Participants who were previously enrolled in a face-to-face study during 2019 and who provided consent for future studies, were invited to take part in the present survey. Data, including child feeding practices, were collected from maternal recall of the COVID-19 pandemic peak lockdown period. Descriptive statistics were calculated and factors associated with breastfeeding were analysed using log binomial regression. Results. A total of 322 WLHIV aged between 19 and 46 years were successfully interviewed. Of these, only 6.4% reported disruption in antiretroviral treatment (ART), 18 (5.6%) had children living with HIV (CLHIV), all of whom were on ART. Out of the 300 HIV-exposed uninfected children (HEUs) who were eligible for extended postnatal prophylaxis (extended PNP) (those still breastfeeding, and the mother either had a viral load (VL) >= 1 000 copies/mL or had not undergone VL testing within the past 6 months), 47.7% received it. During the COVID-19 peak lockdown, 30.1% of children were breastfed, including 28.6% (n/N=87/304) of HEUs and 62.1% of breastfeeding HEUs were on extended PNP. Log binomial regression analysis showed HEUs on extended PNP and CLHIV had increased likelihood of breastfeeding (prevalence ratio (PR) 1.8 (95% confidence interval (CI) 1.2-2.6) and PR 2.6 (95% CI 1.6-4.4), compared with eligible HEUs who were not on extended PNP. Conclusion. Good antiretroviral coverage supported safe breastfeeding practices during the COVID-19 peak lockdown in this rural setting in SA, with promising ongoing adoption of extended PNP during breastfeeding.
Background. In South Africa, there is a paucity of data on the profile of hypophosphataemic rickets and response to conventional treatment like oral phosphate and alfacalcidol Objectives. To assess the clinical, biochemical and radiological profile of children with genetic hypophosphataemic rickets and their response to conventional treatment. Methods. Retrospective descriptive study of children under the age of 18 years with hypophosphataemic rickets. Height, calcium, phosphorus, alkaline phosphatase (ALP), and parathyroid hormone (PTH) levels were assessed at 3, 6, 9 and 12 months of age and annually thereafter. The Thacher radiological score at baseline and at regular intervals was determined after commencing treatment. Results. Seventy patients met the inclusion criteria. A positive family history was obtained in 32 (46%) patients. Thirty-three (47%) patients were poorly compliant with treatment. The patients were short-statured with a mean height-for-age Z-score (HAZ) of -3.4 (1.79). The mean (standard deviation) calcium, phosphate, ALP and PTH levels and median (range) Thacher score were 2.3 (0.16) mmol/L, 0.84 (0.19) mmol/L, 776.6 (531) U/L, 7.15 (4.8) pmol/L and 8 (4 - 8), respectively, at baseline. At 5-year follow-up, improvements were seen in ALP (525 (232) v. 776 (531); p<0.001) and Thacher scores (2 (1-3.5) v. 8 (4 - 8); p=0.01) with treatment, however, there were no changes in phosphate levels or HAZ. Conclusion. Conventional therapy for treatment of hypophosphataemic rickets is not associated with an improvement in HAZ despite an improvement of the Thacher score and ALP. Compliance is a major challenge for majority of patients.
Background. Patent ductus arteriosus (PDA) is a congenital heart disease (CHD) whereby the ductus arteriosus fails to close within 72 hours after birth. The aetiology of a PDA is multifactorial. Well-described neonatal associations include prematurity, low birthweight, hypoxic states, infectious states and other co-existing congenital heart defects. Maternal associations include diabetes, hypertension, lack of antenatal care (ANC), advanced maternal age (AMA) and HIV. However, such data are limited to two studies in sub-Saharan Africa. Objective. The primary outcome was determining the incidence and trend of PDA at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH) from January 2013 to December 2020. The secondary outcomes were to determine the association between neonatal and maternal variables, and the development of a PDA from January 2013 to December 2020 at CMJAH. Methods. A retrospective record analysis involving 13 265 neonates admitted to CMJAH, a tertiary hospital in South Africa, from January 2013 to December 2020 was done. Inclusion criteria included confirmed or absent PDA on echocardiography. Exclusion criteria included missing more than 15% of the neonatal and maternal variables. Results. PDA occurred in 4.6% of the population. On multivariate analysis extremely low birthweight (ELBW) and very low birthweight (VLBW) were significant associations (ELBW odds ratio (OR)=1.884, p p=0.0134; VLBW OR=2.291, p p<0.001). Other significant associations were CHD (OR=16.485, p<0.001), invasive ventilation (OR=3.062, p<0.001) and AMA (OR=1.692, p=<0.001). Maternal hypertension emerged as a protective factor (OR=0.474, p<0.001). Both sex (OR=1.125, p p=0.3188) and ANC (OR=0.755, p p=0.1027), as protective factors, were not significant. Various complications and associations such as sepsis after day 3 (OR=8.21, p<0.001) and necrotising enterocolitis (NEC) (OR=4.504, p<0.001) were strongly associated with PDA on univariate analysis. PDA was also associated with a greater length of hospital stay (OR=1.6, p<0.001). Conclusions. PDA is a multifactorial disease with incidence in this study remaining relatively low. Co-existing CHD appears to be the most associated variable with PDA. Other significant variables include lower birthweight, invasive ventilation, advanced maternal age, late-onset sepsis (after day 3 of life) and maternal hypertension. It was also found that the presence of a PDA significantly prolonged hospital stay. The study provides insight into PDA in the South African setting; it also highlights that early PDA screening and appropriate intervention may improve mortality and morbidity.
