Objective- We aimed to describe the use of blood products following cardiac surgery, as well as the outcomes and factors associated with post-operative blood product use Design- Prospective, single centre observational study Setting- Paediatric intensive care unit (PICU) in Cape Town, South Africa Patients- One hundred and twenty-six children <18 years old admitted to the PICU following cardiac surgery between July 2017 and January 2018 Interventions- None Measurements and Main Results- The data was prospectively obtained from blood bank charts, intraoperative and PICU observation charts. Demographic data, intraoperative details and post-operative blood product use were extracted from patient records and entered in a standardised case record form. Fifty three percent of children received blood products following cardiac surgery. The blood products transfused included cryoprecipitate (30.9%), packed red cells (22.2%), albumin (18.3%), fresh frozen plasma FFP (15.9%) and platelet concentrate (15.1%). Low haemoglobin level was commonest indication (86%) for red cell use. Bleeding was the commonest indication for FFP (70%) and cryoprecipitate (67%) use. Thrombocytopenia was the commonest indication (84%) for platelet use while hypotension episodes were predominant (95%) in those who received albumin. The standardized mortality ratio was 3.1 vs 0, respectively, among transfused versus non-transfused patients (p<0.0001). The median (IQR) duration of PICU stay was 5 (3-11) vs 2 (2-5) days, respectively in those transfused versus non-transfused (p<0.0001). The median (IQR) ventilation duration was 47(22-132) hours vs 20 (6-27) hours, respectively among the transfused versus non-transfused (p=<0.0001). The factors associated with blood-product use post cardiac surgery include previous cardiac surgery, younger age, lower weights, and prolonged coagulation parameters (p=<0.05). Conclusion- There is high usage of blood products among children post cardiac surgery. The children transfused had a longer ICU stay, ventilation duration, and higher standardized mortality ratio compared to the non-transfused.

Includes abstract. Includes bibliographical references.

Introduction: Congenital and acquired heart diseases are highly prevalent in developing countries despite limited specialised care. Namibia established a paediatric cardiac service in 2009 with significant human resource and infrastructural constraints. Therefore, patients are referred for cardiac interventions to South Africa. Objectives: To describe the diagnoses, clinical characteristics, interventions, post-operative morbidity and mortality and follow-up of patients referred for care. Methods: Demographics, diagnoses, interventions, intra- and postoperative morbidity and mortality as well as longitudinal follow-up data of all patients referred to South Africa were recorded and analysed. Results: The total cohort constituted 193 patients of which 179 (93%) had congenital and 7% acquired heart disease. The majority of patients (78.8%) travelled more than 400 km to Windhoek prior to transfer. There were 28 percutaneous interventions. Palliative and definitive surgery was performed in 27 and 129 patients respectively. Eighty (80/156, 51.3%) patients had postoperative complications, of which 15 (9.6%) were a direct complication of surgery. Surgical mortality was 8/156 (5.1%, 95% confidence interval 1.2.2-9.8), with a 30- day mortality of 3.2%. Prolonged ICU stay was associated with a 5% increased risk of death (Hazard Ratio 1.05, 95% confidence interval: 1.02-1.08, p=0.001). Follow-up was complete in 151 (78%) patients over seven years. Conclusions: Despite the challenges associated with a cardiac programme referring patients for intervention to a neighbouring country and the adverse characteristics of multiple lesions and complexity associated with late presentation, we report good surgical and interventional outcomes. Our goal remains to develop a comprehensive sustainable cardiac service in Namibia.

Background: The paucibacillary nature of paediatric pulmonary tuberculosis (PTB) makes microbiological diagnosis difficult and limits the usefulness of microbiology for assessing treatment efficacy. Clinical response to treatment has thus been used by clinicians to monitor disease activity, as well as by researchers in clinical case definitions of intrathoracic TB to differentiate those with unconfirmed PTB from those with other lower respiratory tract infections (LRTIs). There is, however, limited data on the expected pattern and timing of resolution of symptoms and signs, and whether this does indeed differ between those with PTB and those without. Objectives: To longitudinally investigate clinical response to TB treatment in children treated for PTB, to compare this to the clinical course of children with other LRTIs, and to identify factors associated with persistence of symptoms and signs. Methods: This study is a secondary analysis of data collected prospectively in a TB diagnostic study from 1 February 2009 to 31 December 2018. We enrolled children ≤15 years with features suggestive of PTB. Study participants were categorized into 3 groups according to NIH consensus definitions; confirmed PTB, unconfirmed PTB and unlikely PTB. Children were followed at 1 and 3 months after enrolment. Those with confirmed or unconfirmed TB were also followed at 6 months. At enrolment and follow-up symptoms of PTB were recorded using a standardized questionnaire and physical examination was done including anthropometry and respiratory parameters. Data were analysed using STATA version 16.1. The effect of potential predictors of persistence of symptoms and signs was explored with univariable and multivariable logistic regression modelling. Results: Two thousand and nineteen children were included in this analysis, 427 (21%) with confirmed PTB, 810 (40%) with unconfirmed PTB, and 782 (39%) with unlikely PTB. Symptoms resolved rapidly in the vast majority of participants. At 1 month, 9.2% (129/1402) of all participants who had a cough and 11.1% (111/999) of those with loss of appetite at baseline reported no improvement in these symptoms. At 3 months this declined to 2.0% (24/1222) and 2.6% (23/886) respectively, with no differences between the groups. Clinical signs persisted in a greater proportion of participants. At 3 months, tachypnoea persisted in 56.7% (410/723) of participants. Abnormal auscultatory findings (including wheeze, crackles, reduced breath sounds or abnormal breath sounds) similarly persisted in almost a third of participants, with greater proportion in the confirmed group (37.1%) than unconfirmed (23.0%) and unlikely (26.2%) groups (p=0.002). Children living with HIV and those with abnormal baseline chest radiographs had greater odds of persistence of signs or symptoms (including cough, loss of appetite, abnormal auscultatory findings, or no weight improvement if underweight at baseline). No features of clinical response differentiated those with PTB from those without. Conclusion: Symptoms resolved rapidly in the majority of children investigated for PTB whilst clinical signs took longer to resolve. The timing and pattern of resolution of symptoms and signs cannot differentiate those with PTB from those without – and is thus not a suitable parameter for confirming disease classification in paediatric TB research.

Background Sub-Saharan Africa bears the highest burden of epilepsy worldwide. A proportion is presumed to be genetic, but this aetiology is buried under the burden of infections and perinatal insults, in a setting of limited awareness and few options for testing. Children with developmental and epileptic encephalopathies (DEEs), are most severely affected by this diagnostic gap, as the rate of actionable findings is highest in DEE-associated genes. This research study investigated the genetic architecture of epilepsy in South African (SA) children clinically diagnosed with DEE, highlighting the clinical utility of informative genetic findings and relevance to precision medicine for DEEs in a resource-constrained setting. Methods A group of 234 genetically naïve SA children with drug-resistant epilepsy and a diagnosis or suspicion of DEE, were recruited between 2016 and 2019. All probands were genetically tested using a DEE gene panel of 71 genes. Of the panel-negative probands, 78 were tested with chromosomal microarray and 20 proband/parent trios underwent exome sequencing. Statistical comparison of electroclinical features in children with and without candidate variants was performed to identify characteristics most likely predictive of a positive genetic finding. Results Pathogenic/likely pathogenic (P/LP) variants were identified in 41/234(17.5%) * probands. Of these, 29/234(12.4%) * were sequence variants in epilepsy-associated genes and 12/234(5.1%) * were genomic copy number variants (CNVs). Sixteen variants of uncertain significance (VUS) were detected in 12 patients. Of the 41 children with P/LP variants, 26/234(11%) had variants supporting precision therapy. Multivariate regression modelling highlighted neonatal or infantile-onset seizures with movement abnormalities and attention difficulties as predictive of a positive genetic finding. This, coupled with an emphasis on precision medicine outcomes, was used to propose the pragmatic “Think-Genetics” decision tree for early recognition of a possible genetic aetiology, pragmatic testing, and multidisciplinary consultation. Conclusion The findings presented here emphasise the relevance of an early genetic diagnosis in DEEs and highlight the importance of access to genetic testing. The “Think-Genetics” strategy was designed for early recognition, appropriate interim management, and genetic testing for DEEs in resource constrained settings. The outcomes of this study emphasise the pressing need for augmentation of the local genetic laboratory services, to incorporate gene panels and exome sequencing. *These percentages were rounded off to whole numbers in the published articles included in this thesis (i.e., rounded off to 18%, 12% and 5%, respectively).

M.Sc. (Med. (Paediatric Cardiology)), Faculty of Health Sciences, University of the Witwatersrand, 2008 / Left ventricular (LV) noncompaction is characterized by the presence of an extensive trabecular myocardial layer within the luminal aspect of the compact myocardium of the ventricular wall. The trabeculae are both excessive in number and more prominent than normal. Noncompaction may occur in isolation usually with clinical features of dilated cardiomyopathy, or it may be associated with congenital or acquired heart diseases. Echocardiography is the reference standard for diagnosis, where a ratio of thickness of trabecular-to-compact myocardium (compaction ratio) of >2 is a major diagnostic criterion. Noncompaction is usually considered to result from persistence of the highly trabeculated myocardium found in early cardiogenesis of the human embryo. If persistence of excess trabeculae is the only determinant of the compaction ratio it would be expected that it would remain a consistent measurement in postnatal life. However, temporal changes in the degree of noncompaction in individual case reports have raised the question as to whether the compaction ratio might be sensitive to haemodynamic or other factors. In the present dissertation, I assessed echocardiographically whether the compaction ratio is associated with increases in indices of LV volume preload in 100 children or adolescents with ventricular septal defects (VSD), and 36 with chronic rheumatic heart disease (RHD). Compared to 79 normal controls (compaction ratio=1.4±0.07), patients with VSDs (compaction ratio=2.0±0.2, p<0.0001) and RHD (compaction ratio = 2.0±0.3, p< 0.0001) had a marked increase in the compaction ratio. A compaction ratio>2 was found in 42% of patients with VSDs and 47% with RHD. In VSDs, independent of age and gender, the compaction ratio was positively associated with LV mass index (LVMI) (partial r=0.44, p<0.0001), VSD size (partial r=0.4, p<0.0001), LV end diastolic diameter indexed (LVEDD) (partial r=0.24, p= 0.01), and the presence of additional shunts (partial r=0.21, p=0.02). In RHD, independent of age and gender, the compaction ratio was positively associated with LVEDD (partial r=0.62, p=0.0001), and LVMI (partial r=0.48, p=0.005), and negatively with LV ejection fraction (partial r=0.31, p=0.03). The strong association of indices of LV volume load and the compaction ratio would suggest that haemodynamic influences are contributing to the compaction ratio both in congenital and acquired cardiac disease in childhood. Thus an increased compaction ratio may be the consequence of an increased volume preload, and therefore may not necessarily occur only as a result of persistence of embryonic patterns.

No description available.

No description available.

A research report submitted to the faculty of health sciences, University of Witwatersrand, Johannesburg, partial fulfillment of the requirements for the degree of master of science in occupational therapy Johannesburg, 2017 / MT2017

ABSTRACT Emphasis on humanistic values and personal experience in nursing has led to the popularisation of holistic nursing approach to nursing care. Although holistic nursing care as a construct is widely discussed in nursing literature. Contextual clinical application has been difficult, in the absence of guiding conceptual framework and guidelines that directs nursing practice. In this study, the purpose was to examine the meaning of holistic nursing care and develop a framework for holistic nursing care, which can be utilised in nurse education settings and in clinical nursing practice in the context of paediatric nursing in academic hospitals. To achieve this aim, qualitative methodological perspectives were employed based on careful selection of the population, sampling, collection and analysis of data and trustworthiness. To enable the accomplishment of the purpose, the study objectives were formulated into two phases. Phase one objective enabled the identification of the characteristics of the concept holistic nursing care through concept analysis and by obtaining the emic viewpoints of the paediatric nurses working in academic hospitals. A philosophical inquiry was employed using Rodgers’ evolutionary method of concept analysis. To elucidate the concept holistic nursing care a qualitative, interpretive, explorative and contextual research design was employed. Holistic nursing care was interpreted as whole care fostering person-centred and family-centred care. The results confirm the current discourse in nursing literature with respect to “person-centred”, “family-centred care” as opposed to “patient-centred care”. The emphasis is on recognition of the need to transform current linguistic ontology from “patient care” towards the provision of “whole-person” care. Participants’ interpreted v holistic nursing care as whole care directed towards a unique and complex human being. The dynamic, which is the driving force for the achievement of whole care, is established through enabling goal-directed nurse-family relationships. One of the key finding is the prominence of spirituality and the inclusion of spirituality in different aspects of child nursing. The dependency of individual nurses to spiritual sources for personal strength and support was recognised and acknowledged. Knowledge of disease, person and “know how” are necessary for the acomplishment of ethically, safe person-centred whole care. Attributes of holistic nursing care yielded two dimensions; whole person and mind-body-spirit dimension. The decriptors of whole person include physical, mental, emotional, spirit and spitual being. Spirituality is the predominant antecedent. Holistic nursing care is initiated by the recognition of the individual, in need of health care, as a spiritual being with mind-body-spirit dimension. Spirituaity is an ever-present force pervading all human experience. Complimentary alternative medicine (CAM) was identified as a surrogate term. The connection of CAM with holistic nursing care is the focus of therapetic interventions that are directed to the mind-body-spirit domain. The emphasis is on health rather than curing. Preventative therapeutic interventions are desingned to meet the needs of the whole-person. Caution is advocated in the use of CAM therapies in child nursing, as CAM efficacy has not been sufficiently investigated in child health care. The conceptual framework is presented as unique contribution to nursing. The framework may be introduced at undergraduate teaching of child and family nursing care and in specialists’ paediatric nurse education. Recognition of the human being as a whole person with mind-body-spirit dimension is not restricted to a child or family care. Therefore, the vi framework is presented as a fundamental structure that can be used generally to all intervention activities in relation to human–human interactions. Its use may be broadened to any therapeutic environments. The framework may be tested in adult nursing in variety of settings in health care. There is a potential to expand and transfer certain elements of the framework to other discipline beyond nursing: in doctor-patient relationships, manager-employee relationships, and person-to-person interactions. Perhaps the South African Nursing Council, as the regulating body responsible for developing the educational framework of nursing education in this country may adopt this framework in line with their philosophy of nursing to articulate with their intended goal of providing holistic nursing care for the people of this nation. Adoption of the framework may require a shift from the current “patient-centred care” towards “person-centred care”.