Includes bibliographical references. / The assessment of pain and anxiety in South Africa is complicated by language barriers, cultural differences, socio-economic difficulties and delayed cognitive development. The high number of paediatric burn injuries (annually 2000) treated at the Red Cross War Memorial Children's Hospital, the need to accurately assess pain and drug efficacy and the current lack of specifically designed methods to do so, led to the development of the Burn Observational Pain and Anxiety Scale (BOPAS). This scale is believed to be the first of its kind and was designed to measure both pain and anxiety in burned children. The aim of this study was: - To develop an observational pain and anxiety scale that can overcome ianguage barriers by excluding patient involvement in the assessment process. - To develop a scale that can differentiate between pain and anxiety during wound care procedures. - To develop a method that facilitates the translation of nominai information into numerical data. - To develop a scale that can evaluate drug and dose efficacy. A total of 105 chiidren, (M = 65, F = 40) aged 2-12 (average age 6.8 years), admitted for minor to moderate burn injuries to the Burns Unit of the Red Cross War Memorial Children's Hospital, were included in the sample. Five different consecutive studies varying between explorative and quasi-experimental were conducted to determine different levels of validity and reliability.

Background and objective: To describe the clinical-radiological-pathological characteristics and treatment outcomes of childhood exogenous lipoid pneumonia (ELP) and elucidate oil administration practices. Methods: A retrospective study of children with histologically-confirmed ELP at Red Cross Children’s Hospital, South Africa. Caregivers were interviewed to understand oil administration practices. Results: Twelve children of Zimbabwean heritage aged 2.1-10.8 months were identified between 2012 and 2017. Repeated oral administration of plant-based oil for cultural reasons was reported by 10/11 caregivers. Cough (12/12), tachypnea (11/12), hypoxia (9/12) and diffuse alveolar infiltrates on chest radiography (12/12) were common at presentation. Chest computed tomography revealed ground glass opacification with lower zone predominance (9/9) and interlobular septal thickening (8/9). All bronchoalveolar lavage specimens appeared cloudy/milky, with abundant lipid laden macrophages and extracellular lipid on Oil-Red-O staining and documented polymicrobial (6/12) and Mycobacterium abscessus (2/12) co-infection. Antibiotics, systemic corticosteroids and therapeutic partial lung lavage were interventions in all, 8 and 5 patients respectively. Median time to clinical resolution was 1.1 months IQR (0.5-8.0) with radiological resolution only in 2/12 cases. Conclusions: Paediatric ELP resembles pulmonary alveolar proteinosis. Health workers should explicitly probe for a history of oil administration in children with non-resolving pneumonia and consider the diagnosis of ELP in settings where this is a common practice.

Includes bibliographical references.

Introduction: Dialysis is a temporary renal replacement therapy (RRT) to keep the child healthy and alive when in end stage kidney failure (ESKF) while being worked up for kidney transplant, the preferred treatment. Chronic peritoneal dialysis (PD) is the preferred first choice of dialysis modality in many centers because of its advantages over hemodialysis (HD). In recent years, there have been advances to improve the performance and survival of PD as a modality for renal replacement. Despite these improvements, complications still arise, sometimes warranting a switch to HD. We sought to investigate the extent to which children at Red Cross War Memorial Children's Hospital (RCWMCH) drop-out from chronic PD and describe some of the reasons for this drop-out. Objectives: To describe the rate of drop-out of children with ESKD from chronic PD, the timing and factors associated with this drop-out at RCWMCH. Methods: This was a retrospective descriptive study, carried out in the renal ward, E2, of RCWMCH in Cape Town. Eligible participants were identified from the renal transplant waiting lists over the study period. Patient folders were retrieved following ethical approval, for extraction of relevant data. Outcome measures: Proportion dropping-out during the study period (permanent switch to HD or death from PD related complications), factors associated with dropout and time from initiation of chronic PD to drop-out. Utility of the study: Findings from this study will help in designing strategies to improve chronic PD patient outcomes, prolongation of PD technique survival and reducing the costs of chronic dialysis at RCWMCH. Results: A total of 111 children were listed for transplantation between January 2009 and December 2018, 67 were treated with PD. Complete data was available for 52 of the 67 children who received PD. Overall, 17/52 (32.7%) dropped-out during the study period. Most (>50%) of them dropped-out within the first 1-2 years of being on PD. The only significant associated factor was one or more episodes of peritonitis. Recommendation: There is a need to step up measures to prevent peritonitis in chronic PD patients so as to prolong stay on PD until a kidney transplant is available, as well as improve kidney transplantation rates. Dissemination of results: Results were presented at the Department of Pediatrics and Child Health Research Day 2019 and at the world congress of nephrology international conference 2021. Results will be submitted for publication in a peer reviewed journal.

Background: Tuberculosis remains a major challenge in transplantation particularly in endemic countries. The incidence, clinical manifestations, and optimal investigations for TB specifically in the paediatric post-transplant population have not yet been adequately studied. This study aims to describe the incidence, clinical presentation and outcomes of tuberculosis in paediatric kidney transplant recipients and to assess the impact of Isoniazid prophylaxis. Methods: Single-centre retrospective descriptive analysis of children who received kidney transplants from 1995-2019. The cohort was stratified according to receipt of isoniazid prophylaxis which began in 2005. Results: 212 children received a kidney transplant during the study period. Median age at transplantation was 11.2 years (IQR: 2.2 - 17.9) and 56% were males. Tuberculosis was diagnosed in 20 (9%) children, with almost two thirds (n=12) occurring within the first year post-transplant. The main presenting symptoms included fever (n=13/20), weight loss (n=12/20) and cough (n=10/20). Tuberculin skin test was positive in four of 20 children. Coinfection with Ebstein Barr virus, Cytomegalovirus or Staphylococcus was found in five children. Due to interactions an up to three fold increase in calcineurin inhibitor dose was required to maintain therapeutic blood levels. Isoniazid prophylaxis was protective against development of tuberculosis (p=0.04). ) and was associated with fewer infections within the first year of transplantation although this was not statistically significant (p = 0.3). Gender, age and type of allograft were not significant risk factors for developing tuberculosis. All the tuberculosis infections were successfully treated. There was graft and patient survival of 100%. Conclusion: Kidney transplant recipients have a high risk of tuberculosis. Diagnosis remains a challenge. Frequent and meticulous monitoring of immunosuppression drug levels during treatment of TB is required to avoid loss of patient or graft. Isoniazid prophylaxis protects against development of TB in this population. (HREC 463/2020)

A research report submitted to the Faculty of health Sciences, University of the Witwatersrand, Johannesburg, in particular fulfillment of requirements for the degree of Masters in Medicine (MMed) Johannesburg, / MT2017

Background The surgical infant requires care in specialized neonatal units. Very low birth weight (VLBW) infants are a group particularly vulnerable to the risks and outcomes associated with preterm birth. There is an increased number of abdominal emergencies seen, attributed to improved survival in this birthweight category. Objectives To describe the short-term survival to discharge in VLBW infants following abdominal surgery at a South African public tertiary hospital and to examine the utility of common scoring systems for prognostication. Methods A retrospective study of VLBW infants with abdominal surgery was conducted in patients admitted to the neonatal unit at Groote Schuur Hospital between 2012 and 2016. CRIB and SNAPPE scores were calculated for patients where sufficient data was available. Results Fifty-two patients were included. The mean gestational age (GA) and birthweight (BW) were 29.5 weeks (SD 2.1) and 1102g (SD 197.8) respectively. Necrotizing enterocolitis was the most common (50%) surgical emergency. The leading postoperative complication was sepsis (37%). Fourty-two (81%) infants survived to discharge, the mean age at presentation 21 days (SD 21.1) with a mean hospital stay of 74 days in survivors vs 52 days in the non-survivors (p=0.06). There was no statistically significant difference in SNAPPE scores between survivors and non-survivors. Conclusion Abdominal emergencies have a high mortality and adds to the overall length of stay in VLBW infants. Neonatal scoring systems have proven to be useful adjuncts in predicting neonatal mortality, further study is warranted in infants who deteriorate due to surgical abdominal complications.

Background Hyperthyroidism occurs in about 1 per 5000 children and adolescents. Graves disease (GD) is the most common cause of hyperthyroidism in children and adolescents. The treatments that are currently available for children with GD include Carbimazole (CMZ), radioactive iodine (RAI) therapy and surgery. The paucity of GD data in children from the sub-Saharan African region, challenge the physician in identifying the best suited diagnostic and treatment strategies for the patient population in their setting. Objective The aim was to describe the population group attending the Paediatric and Adolescent Endocrinology Services (PAES) at Red Cross War Memorial Children's Hospital (RCCH) and Groote Schuur Hospital (GSH) in Cape Town, Western Cape. This study hoped to contribute information to the body of evidence concerning GD in the paediatric population of South Africa. Methods This was a retrospective folder review of all children and adolescents diagnosed with GD, attending the PAES in the previous two decades. Their demographic profile, clinical and laboratory findings and the treatment modalities utilised were described. All patients diagnosed with GD between the ages of 1 and 20 years old were included. Data were described as proportions and percentages. The measures of central tendency were described by median, and inter-quartile range (IQR). Results Thirty-one patients were included in the study. Twenty-six patients were female. The median age at presentation was 10.1 years (IQR: 8.9; 11.7). All patients were initially treated medically with Carbimazole (CMZ). Two patients experienced mild adverse reactions attributed to CMZ. Twelve (39%) patients went into remission after a single course of CMZ, after a median of 16.3 months(IQR: 8.6; 35.1). At the study's conclusion, nine (29%) patients were in remission, nine (29%) remained on CMZ therapy, ten (32%) underwent RAI and three (10%) relapsed before GD remission was achieved. One-quarter of the patients (n=8) were known with Down syndrome (DS). These patients presented at a significantly younger age than those without DS. Conclusion The children and adolescents diagnosed with GD managed in the PAES were similar in sex distribution, slightly younger in age and tolerated CMZ better than reported in literature. This study demonstrated the importance of considering prolonging CMZ therapy in patients not yet in remission and as a viable retreatment option in patients that relapse.

Background: Epilepsy is largely a clinical diagnosis based on the patient's history. Neurological examination, interictal electroencephalogram and neuroimaging can all be normal in patients with epilepsy. Making the correct diagnosis in patients with suspected epilepsy is often difficult. Studies show that some 30% of patients are incorrectly diagnosed as suffering from epilepsy. Further, the diagnosis and treatment of epilepsy is often delayed. In the majority of patients, the diagnosis of epilepsy depends on the description of the attack by a witness but the inter-observer interpretations of verbal descriptions of events can be diverse and challenging. The use of mobile phone video recordings has gained worldwide prominence in recent years, including resource limited settings. This study explores the utility of mobile phone video recordings of paroxysmal events as adjuncts in the diagnosis of epilepsy, particularly in resource poor settings where there may exist significant diagnostic delays and misdiagnoses with far reaching implications on resource allocation and poor clinical outcomes. The aim of the study is to assess the accuracy of witness and referring clinician descriptions and to determine the impact, ideally enabling earlier diagnosis and management, of the use of home video recordings i.e the accuracy and timeliness of epilepsy diagnosis in paediatrics with the aim of improving practice in our setting. The existing literature is explored to guide optimal outcomes and ethical approaches to the tool which can be adapted for the paediatric setting. Objective: This study aims at outlining the utility of home videos in the diagnosis of paediatric epilepsy, and specifically describing how home videos influence the accuracy and timeliness of epilepsy diagnosis in clinical practice. Study design: This study has several arms, firstly a literature review to establish the current body of knowledge and established common practice for this intervention. The literature review critiques if there is an accepted and normed model for the use of this tool or existing evidence to support its use beyond anecdotal practice. Further, to define the apparent advantages and potential disadvantages of the intervention and to identify and develop subsequent study questions for a future prospective arm of the study. This will include questions relating to optimal recommended models for the intervention and safe ethical practice. To further assist with this consolidation of data a convenience sampling of videos randomly sent through to the neurology department over the previous year were retrospectively reviewed to assess the nature and route of the referral, the information included, the quality of the video, any ethical or safety concerns and any subsequent change in practice. Results: Eight studies met the inclusion criteria. Methodologies varied, only three included videos of children and quality of the videos was formally scored in two. Overall the studies supported that home videos of good quality had a role in assisting in the differentiation of epilepsy from non-epileptic events, this outcome increased in the setting that history was provided and with the level of experience of the clinician. Studies did not focus on the ethics or risks of home videos and for some of the studies only a small proportion of the epilepsy client load were able to provide video material. Outcome: Data relating to the role of home video of events in children is overall lacking. There are no official guidelines recommended to families for the acquisition of good quality videos in the management and assessment of seizures. Further, the ethical implications of transfer of sensitive recorded material has not been adequately addressed for this group.

Background: The World Health Organisation estimates approximately 10% of neonatal deaths in sub-Saharan Africa and South Asia are due to congenital malformations. Neonatal mortality in the Republic of South Africa needs to be benchmarked against high income countries' (HIC) standard of care to identify means to reduce infant mortality, much of which is due to congenital anomalies amenable to surgical correction. Objectives: (1) Assess 30-day, 6-month and 12-month post-operative mortality for neonates operated for gastrointestinal and abdominal wall defects at a tertiary freestanding paediatric hospital in Western Cape, South Africa, over a 12-year period. (2) Ascertain the causes and risk factors associated with 30-day post-operative mortality. Method: A retrospective folder audit of all neonates that underwent gastrointestinal & abdominal wall surgery within the neonatal period at Red Cross War Memorial Children's Hospital (RCWMCH) during the 12-year period from 1 January 2007 to 31 December 2018. Results: The 30-day post-operative mortality rate was 73/762 (11%). Mortality was found in 9 conditions. An additional 57/762 patients (7%) died post-surgery between 30 days from surgery and 6 months of age. A further 34 patients (4%) died between 6 and 12 months of age. Mortality resulted from: sepsis (74%), palliation due to ultra-short bowel length (12%); in patients with necrotizing enterocolitis, intestinal atresia and malrotation with volvulus, ventilation associated pneumonia (10%), associated congenital cardiac lesions (3%) and intestinal failure associated liver disease (1%). Most neonates (69%) who died were prematurely born. Mean age at surgery was 10 days (median 6 days; interquartile range (IQR) 3-16) and mean age at death was 6 days (median 5 days; IQR 2-12; range 1-30). Nearly all patients who died were managed in the intensive care unit post-operatively (97%), with a median stay of 7 days (IQR 1-10) and overall hospital stay of 8 days (IQR2-12). Mortality in patients from referral hospitals more than an hour drive from RCWMCH was high (15/39, 38%). The odds ratio for death for patients with travel time over one hour from the referral hospital was 3.6 [95% confidence interval 1.8 to 7.3; z-statistic 3.6; p=0.0003]. The majority of surgical procedures in patients who died were for abdominal surgery 70/73 (96%). Surgery for necrotizing enterocolitis (NEC) had the greatest mortality (38%), followed by spontaneous intestinal perforation at (29%), gastroschisis (18%). Thirty-day mortality for oesophageal atresia, congenital diaphragmatic hernia and malrotation with volvulus was 9% each, followed by intestinal atresia at 8%, anorectal malformation (5%) and inguinal hernia (3%). No post-operative mortality was reported for Hirschsprung disease, choledochal malformation, hypertrophic pyloric stenosis, biliary atresia and omphalocele. Relook procedures were conducted for 37%, with the highest percentage of revision surgery for necrotizing enterocolitis at 42%. Abdominal compartment syndrome was noted post operatively in 15% patients. Significant modifiable risk factors for sepsis in patients who died were central lineassociated bloodstream infections (65%), respiratory tract infections (41%) and surgical complications [anastomotic breakdown (7%) and wound infection (24%)]. Conclusion: The 30-day post-operative mortality rate in this middle-income setting is similar to the overall mortality rate in HIC, despite excluding pre-operative mortality in this study. Prevention and improvement strategies for infection control are imperative to improve outcomes in surgical neonates, including optimizing timing of surgical intervention for bowel perforation or obstruction through timeous patient transfer for definite management and intensive care unit capacity optimization, central line care and post-operative infection surveillance. Liberal abdominal compartment pressure monitoring and delayed abdominal closure in selected patients may further reduce mortality. Addressing modifiable factors for morbidity and mortality in this vulnerable patient group is required for comparable outcomes to HIC.