Browsing by Department "Department of Paediatrics and Child Health"

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    Open Access
    A 15-year retrospective review of urodynamic studies in Children at Red Cross War Memorial Childrens Hospital (RCWMCH), Cape Town, South Africa
    (2022) Mosalakatane, Thembisile Dintle; Coetzee, Ashton; Wright, Anne; Raad, Jeanette; Lazarus, John; Nourse, Peter; Howlett, Justin; McCulloch, Mignon
    Background: Despite the undeniable diagnostic benefits of urodynamic studies (UDS), their adoption into clinical practice in Africa has been slow. This study aimed to review the use of invasive UDS in children at a tertiary paediatric hospital in South Africa. Methods: A retrospective analysis of 1108 UDS was conducted. Patient demographic characteristics, primary diagnosis, indication and urodynamic outcomes were reviewed. Presence of urodynamic high-risk features were documented, and a comparison was made between the first study and follow-up study. Results: This study revealed increasing trends in the use of UDS from 2015. Referrals were from Urology (37.7%), Spinal defects clinic (34.4%), Nephrology (20.8%) and other departments (7.0%). The most common reason for referral was review of medical treatment (36.5%). Spinal dysraphism (58.3%) accounted for the majority of conditions seen. Majority (59.1%) of the patients were receiving more than one type of bladder treatment at the time of their first study, with clean intermittent catheterisation (46.5%) being the most common form of bladder management. 97.5% of studies were performed using transurethral bladder catheterization. Urodynamic diagnosis was neurogenic in 74.0%, anatomical (12.2%), functional (8.8%) and normal (5.0%). There was statistically significant improvement in bladder compliance, detrusor leak point pressure and detrusor sphincter dyssynergia between the first study and a subsequent study following therapeutic intervention. Conclusion: The unique ability of UDS to demonstrate changes in detrusor pressures, which is a common reason for therapy failure, makes UDS an invaluable tool in the diagnosis and management of children with lower urinary tract dysfunction.
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    Open Access
    A comparison of oral feeding and total parenteral nutrition in infants of very low birth mass
    (1975) Higgs, Stephen Charles
    This work was car'red out in the Neonatal Intensive Care Unit at Groote Schuur Hospital, Cape Town, from July, 1972, to December, 1973, under the guidance of Dr. A.F. Malan. This was a newly opened Unit for Coloured and African Neonates requiring special care, well-equipped with trained staff and good monitoring apparatus. For the first time it was possible to provide facilities for patient care in line with those available abroad. The problem of feeding preterm infants has interested many authors (Abramowicz, 1966; Babson, 1971; Gamsu, 1972). The ideal quantity, content of nutrients, calorific value and mode of administration of feeds remains, at best, approximate. With the advent of Total Parenteral Nutrition (TPN) and the availability of relatively safe solutions of fats and amino-acids for intravenous use, interest has been stimulated in the optimal nutrient and caloric requirements of the growing infant.
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    Open Access
    A comparison of the accuracy of various methods of postnatal gestational age estimation; including Ballard score, foot length, vascularity of the anterior lens, last menstrual period and also a clinician's non-structured assessment
    (2020) Stevenson, Alexander Graham; Tooke, Lloyd; Joolay, Yaseen
    Rationale Gestational age is a strong determinant of neonatal mortality and morbidity. Early obstetric ultrasound is the clinical reference standard, but is not widely available in many developing countries. There is a well recognised need to identify reliable and simple methods of postnatal gestational age estimation. Methods A prospectively designed methods comparison study in a tertiary referral hospital in a developing country. Early ultrasound (<20 weeks) was the clinical reference standard. Methods evaluated included anthropometric measurements (including foot-length), vascularity of the anterior lens, the New Ballard Score and Last Menstrual Period. Clinicians' non-structured global impression “End of Bed” Assessment was also evaluated. Results 106 babies were included in the study. Median age at birth was 34 weeks (IQR 29-36). Ballard Score and “End of Bed” Assessment had a mean bias of -0.14 and 0.06 weeks respectively but wide 95% limits of agreement. The physical component of the Ballard score, the total Ballard score and Foot-length's ability to discriminate between term and preterm infants gave an AUROC of 0.97, 0.96 and 0.95 respectively. Discussion Although “End of Bed” Assessment and Ballard score had small mean biases, the wide confidence intervals render the methods irrelevant in clinical practice. Foot-length was particularly poor in Small for Gestational Age infants. None of the methods studied were superior to a non-structured clinician's informal “End of Bed” Assessment. Conclusion None of the methods studied met the a priori definition of clinical usefulness. Improving access to early ultrasound remains a priority. Instead of focusing on chronological accuracy, future research should compare the ability of early ultrasound and Ballard score to predict morbidity and mortality.
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    Open Access
    A description of premature and ex-premature infants admitted to a Paediatric Intensive Care Unit in the first six months of life
    (2018) Mathew, Grace; Rossouw, Beyra
    Background: Prematurity is a major risk factor for morbidity and mortality in children. Rehospitalisation with paediatric intensive care unit (PICU) admission constitutes significant morbidity. There is a paucity of literature regarding rehospitalisations of premature infants in South Africa. Objective: To describe the outcomes, clinical course and characteristics of premature infants admitted to a South African PICU, and to identify any predictors of mortality. Methods: This prospective observational study analysed unplanned PICU admissions of premature and ex-premature infants in the first six months of life, over a six-month period. The primary and secondary outcomes were mortality and length of PICU stay, respectively. Data were analysed using standard descriptive and inferential statistics. Results: 29 infants (65% male; median (IQR) birth weight (BW) and gestational age (GA) 1715 (1130 - 2340) g and 32 (29 - 34) weeks respectively) in 33 admissions were included. Five (17.2%) infants died in PICU. Apnoea (39.4%), respiratory failure (24.2%) and shock (24.2%) were the commonest reasons for PICU admission, secondary to pneumonia (33.3%), sepsis (27.3%) and meningitis (12.1%). 72.4% of infants were mechanically ventilated and 48.3% received blood transfusions. Higher revised Paediatric Risk of Mortality (PIM2) score (p = 0.03), inotrope use (p < 0.0001), longer duration of mechanical ventilation (p = 0.03), and cardiac arrest in PICU (p < 0.0001) were associated with mortality on univariate analysis with no independent predictors of mortality. Conclusion: Infections leading to apnoea, respiratory failure and shock are common indications for PICU readmission in premature infants. Mechanical ventilation and blood transfusion were frequently required.
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    A descriptive analysis of children and adolescents with Graves disease attending the paediatric endocrinology services of the Red Cross War Memorial Children's Hospital and Groote Schuur Hospital over 20 years
    (2021) Mendes, Jacqueline; Carrihill, Michelle
    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.
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    A descriptive retrospective audit of the obstetric conditions which occur in mothers of babies with neonatal encephalopathy at Mowbray Maternity Hospital in 2016
    (2019) Dietrich, Liesl Bertha Kay; Fawcus, Susan; Linley, Lucy
    Introduction: Neonatal encephalopathy (NE) is an important condition which may result in mortality or severe and permanent morbidity placing much strain on busy under-resourced health care services, parents and families, and the greater community. There is much debate on its aetiology; whether it is caused by antepartum conditions or intrapartum obstetric complications (known as sentinel events); and the relative contribution of intrapartum hypoxia. Unlike perinatal mortality, NE rates are not routinely audited by maternity facilities. At Mowbray Maternity Hospital, a formal audit was conducted in 2008, which measured the NE rate, focussed on obstetric factors associated with NE and identified avoidable factors in the care provided. It was thought to be of clinical value to repeat this audit to identify whether there were any trends in rates and the pattern of obstetric factors. Aims and objectives: The aim was to describe the obstetric factors occurring in patients who delivered neonates at MMH, diagnosed with NE. Specifically, it was planned to determine the NE rate, to describe obstetric factors occurring in these patients and to assess the avoidable factors related to the patients, health system and clinical management. Methodology: This was a retrospective descriptive study which included patients whose neonates were diagnosed with NE and were born at MMH in 2016. The diagnosis of NE was made according to the MMH NE protocol where NE is defined as a voltage suppression in amplitude-integrated electroencephalography (aEEG) or seizures; or clinical seizures or dystonic movements; or moderate to severe clinical signs of NE as defined by Shankaran and a level of consciousness which is decreased with abnormal tone. The neonates’ names were retrieved from a NE register in the neonatal unit and the corresponding mothers’ folders retrieved. Data on relevant obstetric and clinical management factors were collected from the folders using a data collection tool developed in the Western Cape and all cardiotocographic tracings were assessed by the researcher. Ethics approval was granted by the University of Cape Town Human Research Ethics Committee (UCT HREC) prior to the commencement of the study. STATA 14 was used for the analysis. Results: In 2016, 53 neonates with NE were identified out of 9,702 live births (LB) at MMH. The NE rate was 5.5 per 1000 LB. Of the 53 neonates, 48 maternal patient files were retrieved and analysed. There were 58% who had been referred to MMH from the midwife obstetric units (MOUs), and 42% fully managed at MMH. All patients were booked for 14 antenatal care, the mean age was 27.5 years and 50% were nulliparous. The mean gestational age at delivery was 39 weeks. The majority (87.5%) experienced labour, spontaneous in 72.9% and induced in 14.6%. Antenatal complications occurred in 77.1%, the most frequent being prolonged pregnancy (25%) hypertensive disorders (18.8%), antepartum haemorrhage (8.3%) and prelabour rupture of membranes (8.3%). Obstetric problems in labour included prolonged second stage of labour (25% of patients who had a second stage of labour); multiple vaginal examinations (28.6%) and prolonged first stage of labour (17.9%). Fetal monitoring at the MOUs was done according to protocol in 70% of patients in the latent phase but only 12.5% of those in the active phase of labour. At MMH, all patients in labour had Cardiotocograph (CTG) monitoring with 90.6% of CTGs being pathological and 6.3% suspicious, as assessed by the researcher. Meconium stained liquor occurred in 40.5% of patients. The mode of delivery was normal vertex, (27.1%), Caesarean sections (58.3%) and assisted vaginal delivery (14.6%). Most CS (71.4%) were done for pathological CTGs. Sentinel events occurred in 15 (31.3%) patients; approximately two-thirds occurring intrapartum and one- third antenatal. Sentinel events included shoulder dystocia (10.4%), prolonged second stage of labour (10.4%), abruptio placenta (6.3%), cord prolapse (2.1%) and eclampsia (2.1%). Of the 37 (68.7%) without a sentinel event, 75.8% had a pathological CTG. Considering avoidable factors, there was an ambulance delay in 42.9%, and a delay in accessing theatre for 53.6% of patients requiring a CS. Poor quality CTG tracing and monitoring occurred in 20.8% of patients; and for 34.4%, the researcher identified an abnormal CTG but it was not detected by the attendant health care workers. Discussion and conclusion: The NE rate for MMH is 5.5 per 1000 LBs, this is higher than the 3.7 found in the previous 2008 MMH study, despite a higher CS rate. Possible reasons for the increase include changes in case ascertainment, increased workload with same staff component, or a shift from perinatal hypoxic mortality to morbidity, notably NE. This NE rate compares with other lower resource settings and the previous MMH audit, as does the high proportion of intrapartum obstetric sentinel events. This is in contrast to findings from high resource settings. Areas for service improvement include regular and ongoing intrapartum care training, including fetal heart monitoring, for medical and nursing staff; and addressing the health system issues identified.
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    A descriptive study of the community-based follow-up and outcomes of very low birth weight babies discharged from a regional hospital
    (2022) Gondwana de Wit, Thandi Maya; Hendricks, Michael
    Background Neonatal mortality continues to be a significant global health concern, especially in low/middle income countries. In South Africa, neonatal deaths contribute to 32% of the under-five mortality rate, with 48% of these deaths attributed to prematurity. One of the components aimed at reducing neonatal deaths in the Western Cape, is the Home and Community-Based Services (HCBS) for very low birth weight (VLBW) babies. This intervention could reduce neonatal deaths by 25%. This study aimed to describe a VLBW baby cohort discharged from a regional hospital in Cape Town over a year; the HCBS referral process; their follow-up; their outcomes and caregivers' perceptions of the service. Methods This was a retrospective descriptive mixed methods study. Quantitative data from an accredited database were used to describe the VLBW cohort. Meetings with stakeholders and referral form analyses were used to assess the referral pathway and follow-up. Telephonic interviews were held with VLBW babies' caregivers to obtain further quantitative and qualitative data about the HCBS programme. Results During 2018, 169 VLBW babies were included in the population with a mean (SD) gestational age of 30 (±2.21) weeks and median (IQR) birthweight of 1210g (1045-1390g). At delivery, 84.6% had respiratory distress with 60% requiring continuous positive airway pressure; 64% had presumed and 15.3% had suspected or proven nosocomial sepsis. Caregiver characteristics included unbooked pregnancies (10%), primigravida deliveries (15%), smoking (11%), maternal alcohol use (9%), teenage pregnancy (5%), drug addiction (3%) and babies born before hospital arrival (4%) with 14% being referred to a social worker. Folder review showed plans for HCBS referral in only 49 (43.4%) of the cohort, however only 20 (17.7%) referral forms were received by HCBS. Learning about the VLBW HCBS programme identified several challenges relating to the referral process from both the hospital and HCBS side. Overall, the caregivers interviewed had positive perceptions of the HCBS. Those not visited by HCBS felt they would have benefitted from a visit. Conclusion The burden of this medically and socially vulnerable VLBW cohort, who are at high risk of neonatal mortality and morbidity, remains large at this regional hospital - constituting nearly 15% of all their neonatal discharges. Despite the identified challenges, the caregivers' interviewed remained positive about the HCBS. HCBS can play an essential role in providing education, counselling and support following hospital discharge. However, for the HCBS to be fully effective, further promotion, strengthening and monitoring of the referral system is required.
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    A descriptive study of vancomycin usage at Red Cross War Memorial Children's Hospital, Cape Town
    (2023) Greybe, Leonore; Nuttall, James; Eley Brian
    Background: Antimicrobial stewardship principles guide the clinical use of vancomycin, but paediatric vancomycin prescribing practices have not been evaluated in South Africa. Objectives: To document the use, prescribing practices and monitoring of intravenous vancomycin and the spectrum of bacteria isolated on microbiological culture in children treated with intravenous vancomycin during a 12-month period at Red Cross War Memorial Children's Hospital (RCWMCH). Methods: A retrospective audit of intravenous vancomycin use in children admitted to RCWMCH during 2019. Results: All 158 vancomycin prescription episodes for 143 children were included. Overall usage of intravenous vancomycin was 63 days of therapy/1000 patient days (IQR 38–72). The median starting dose was 15 mg/kg/dose (IQR 14 ̶ 15) and median daily dose was 45 mg/kg/day (IQR 43–60). Vancomycin was prescribed as empiric (127/158, 80%) and directed (31/158, 20%) treatment. The median duration of treatment for the directed group was longer than the empiric group (p=0.001). Only 65/98 (66%) episodes where vancomycin treatment exceeded three days had vancomycin serum trough concentrations performed, and only 16/65 (25%) of these samples were obtained before the fourth dose. Prolonged antibiotic treatment of 14 days or more was not associated with gram positive bacteria on culture (OR 1.02, 95% CI 0.17 ̶ 4.2). Conclusion: Prolonged empiric treatment and inappropriate vancomycin monitoring were problems associated with vancomycin prescriptions. Contribution: Our study identified multiple opportunities for improved vancomycin prescribing and monitoring. Further research and implementation of improved prescribing practices could contribute to the preservation of vancomycin as an effective antibiotic.
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    A profile of children with traumatic brain injury admitted to the Red Cross War Memorial Children?s Hospital Paediatric Intensive Care Unit in Cape Town, South Africa, between 2015 and 2019
    (2023) Du Plooy, Elri; Salie, Mogamat; Figaji Anthony
    A Profile of Children with Traumatic Brain Injury Admitted to the Red Cross War Memorial Children's Hospital Paediatric Intensive Care Unit in Cape Town, South Africa, between 2015 and 2019 Background: Paediatric traumatic brain injury (TBI) is a public health problem with high morbidity and mortality. Objectives: We aim to highlight risk factors and describe associated morbidity and mortality of children admitted to our Paediatric Intensive Care Unit (PICU) at Red Cross War Memorial Children's Hospital in Cape Town. Methods: We retrospectively documented the hospitalization of all children with TBI admitted into our PICU between 2015 and 2019. Results: Of 272 children identified, 232 were enrolled: 190 (81.9%) had severe TBI (Glasgow Coma Scale [GCS] ≤8), 32 (13.8%) moderate TBI (GCS 9-12) and 10 (4.3%) mild TBI (GCS ≥13). Median age was 6.5 (IQR 3.5-9) years; 144 (62.1%) were male. Motor vehicle accidents accounted for 77% (179) of injuries. Two hundred (86.2%) children were invasively ventilated for a median of 3.5 (IQR 1-7) days; 26 children (13%, n=200) had a failed extubation and 16/200 (8%) required tracheostomies. Ninety-eight children (42.2%) had intracranial pressure monitoring. Almost 30% (67/232) required vasopressor support. Approximately a third (83/232) developed trauma-related seizures; 25 children (10.8%) required a Thiopentone infusion and 9 children (3.9%) a decompressive craniectomy. Common complications were post-extubation stridor (29/200 [14.5%]), hemiparesis (20/232 [8.6%]) and diabetes insipidus (15/232 [6.5%]). Median PICU stay was 3 (IQR 1-8.3) days, and hospitalization 11 (IQR 5-20) days. Eighty- three (35.8%) children were transferred for further rehabilitation; 24 (10.3%) died. Conclusion: Despite marked improvement since the 1990's, children admitted to PICU with TBI had considerable morbidity and mortality. Enhanced primary preventative strategies, especially for motor vehicle accidents, are imperative to prevent TBI in children.
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    A prospective study to assess the value of liquid chromatography-tandem mass spectrometry in the management of paediatric poisoning at Red Cross War Memorial Children's Hospital, Cape Town, South Africa
    (2020) Washaya, Norbertta Nzwisisayi; Buys, Heloise; Muloiwa Rudzani
    Background: Paediatric poisoning is a common presentation to emergency departments worldwide. There is a paucity of data on the role of liquid chromatography-tandem mass spectrometry (LC-MS/MS), in the management of paediatric poisoning in low-and middle-income countries (LMICs). In high-income countries, most studies are retrospective, and few include children. Objective: The study describes the prevalence of liquid chromatography-tandem mass spectrometry confirmed paediatric poisoning at Red Cross War Memorial Children's Hospital, Cape Town, South Africa. Methods Children admitted with suspected poisoning between 1 January 2017 and 31 December 2017, were recruited. All patients had a urine and/or blood sample sent for LC-MS/MS toxicology. Data collected included demographic data, clinical features, investigations, management, outcome and social interventions. Results 152 children, with median age of 39 (IQR 25 -61) months were enrolled of which 128 (84%) were poisoning cases. Of the 128 poisoning cases, 88 (69%) presented with a history of ingesting a known substance, 16(12%) an unknown substance and 24(19%) were cases of occult poisoning. LC-MS/MS was able to identify a substance in 92% of the cases of occult poisoning. In those who had presented with a seemingly known substance, LC-MS/MS found a different substance in 15 cases. LC-MS/MS was also able to detect multiple drugs in 40 patients. Of the poisoning cases, six (5%) cases were attempted homicide cases and 5 (4%) cases were attempted suicide cases. No children died. Individualized social interventions were instituted in poisoning cases. Emergency placement safety reasons was required in 6 children. Conclusion: When the limitations are known, LC-MS/MS is useful in identifying cases of occult poisoning; identifying patients who have ingested multiple substances and/or an unknown substance and when targeted towards child protection. As LC-MS/MS is an expensive test, it should be used judiciously in LMICs.
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    A randomized controlled trial of intermittent compared with daily cotrimoxazole preventive therapy in HIV-infected children
    (2010) Jele, N; Mulligan, Christine; Le, Roux Stanzi; , ennings
    Objective: Cotrimoxazole preventive therapy (CPT) reduces morbidity and mortality in HIV-infected children. The WHO recommends prolonged daily CPT for HIV-infected infants and children. In adults, intermittent CPT has been associated with less adverse events than daily, with increased tolerability and equal efficacy. We investigated the efficacy and tolerability of intermittent CPT compared with daily CPT in HIV-infected children over a 5-year period. Design: A prospective randomized controlled study. Methods: HIV-infected children aged at least 8 weeks were randomized to thrice weekly or daily CPT. Outcome measures were mortality, bacterial infections, hospitalizations and adverse events. Results: Three hundred and twenty-four children (median age 23 months) were followed for 672 child-years; 165 (51%) were randomized to intermittent CPT. Most children (287, 89%) were Centers for Disease Control and Prevention clinical category B or C; 207 (64%) received HAART during the study. Mortality (53 deaths, 16%) was similar in the intermittent CPT compared with the daily CPT group {24 (14%) vs. 29 (18%), hazard ratio 0.75 [95% confidence interval (CI) 0.44–1.29]}. The predominant causes of death in both groups were sepsis (17, 32%), pneumonia (13, 25%) or diarrhoea (8, 15%). Intermittent CPT was associated with more bacteraemias [incidence rate ratio 2.36 (95% CI 1.21–4.86)]. Children receiving intermittent CPT also spent more days in hospital [incidence rate ratio 1.15 (95% CI 1.04–1.28)]. The rate of serious adverse events was similar between groups [incidence rate ratio 1.07 (95% CI 0.58–2.02)]. Conclusion: Intermittent CPT was associated with more invasive bacterial disease than daily CPT, but survival was similar. Both regimens were well tolerated. On balance, daily CPT remains preferable to intermittent therapy for HIV-infected children.
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    A retrospective description of primary immunodeficiency diseases at Red Cross War Memorial Children's Hospital, Cape Town, South Africa, 1975 – 2017
    (2020) Moodley, Sashmi; Eley, Brian
    Background. The primary immunodeficiency diseases (PIDs) constitute a diverse and everexpanding group of inborn errors affecting a wide range of immune functions. They are not well documented in Sub-Saharan Africa. An important barrier to care is limited awareness of PIDs and their management among health care professionals. This fascinating spectrum of diseases is rapidly expanding worldwide, and not as rare as we think. Genetic characterization and newborn screening for primary Immunodeficiency diseases (PIDs) may be the gold standard in the first world setting but are neither practical nor feasible for our doctors. Yet, other low and middle income countries in the world have also established reasonable services and created registries for children with PIDs, including other African countries. Objective. To describe the spectrum of PIDs at a tertiary paediatric hospital. Methods. A retrospective descriptive study of PIDs diagnosed at Red Cross War Memorial Children's Hospital, Cape Town, South Africa between 1975 and 2017 was undertaken. Results. 252 children with PIDs were identified, spanning 8 of the 9 categories listed in the 2017 classification of the International Union of Immunological Societies. Predominantly antibody deficiencies, combined immunodeficiencies with associated syndromic features, and immunodeficiencies affecting cellular and humoral immunity accounted for 79% of all PIDs. The mean age (standard deviation) at diagnosis was 46 (50) months and the male to female ratio was 1.5:1. A history of parental consanguinity was present in 3 children (1.2%). Recurrent infection was the most prevalent presenting phenotype, manifesting in 70.2% of the patients. Genetic or chromosomal confirmation was obtained in 42/252 (16.7%) of the children. Common interventions used to prevent infection were antimicrobial prophylaxis and immunoglobulin replacement therapy, administered to 37.7% and 36.9% of the patients respectively. Six of seven children who underwent haematopoietic stem cell transplantation (HSCT) had successful outcomes. The 7th patient died 2 months post-HSCT from overwhelming infection. Although we could not account for the children lost to follow up during the study period, 53 (21.0%) deaths were confirmed. Conclusions. Several challenges exist in the recognition and treatment of children with PIDs in our setting. These include limited access to genetic diagnostics and HSCT. Sub-optimal treatment options contribute to the overall mortality of PIDs in South Africa. Greater awareness among clinicians treating children and more laboratory diagnostic capacity are needed to increase the recognition PIDs among children in South Africa. The treatment options that are available in South Africa are unevenly distributed. Hence, treatment capacity should be expanded throughout the country, especially advanced interventions such as HSCT. Ongoing reporting of registries such as ours and increased community awareness should strengthen the lobby for greater investment in rare diseases such as the PIDs.
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    A retrospective description of primary immunodeficiency diseases at Red Cross War Memorial Children's Hospital, Cape Town, South Africa, 1975 – 2017
    (2020) Moodley, Sashmi; Eley, Brian
    Background. The primary immunodeficiency diseases (PIDs) constitute a diverse and everexpanding group of inborn errors affecting a wide range of immune functions. They are not well documented in Sub-Saharan Africa. An important barrier to care is limited awareness of PIDs and their management among health care professionals. This fascinating spectrum of diseases is rapidly expanding worldwide, and not as rare as we think. Genetic characterization and newborn screening for primary Immunodeficiency diseases (PIDs) may be the gold standard in the first world setting but are neither practical nor feasible for our doctors. Yet, other low and middle income countries in the world have also established reasonable services and created registries for children with PIDs, including other African countries. Objective. To describe the spectrum of PIDs at a tertiary paediatric hospital. Methods. A retrospective descriptive study of PIDs diagnosed at Red Cross War Memorial Children's Hospital, Cape Town, South Africa between 1975 and 2017 was undertaken. Results. 252 children with PIDs were identified, spanning 8 of the 9 categories listed in the 2017 classification of the International Union of Immunological Societies. Predominantly antibody deficiencies, combined immunodeficiencies with associated syndromic features, and immunodeficiencies affecting cellular and humoral immunity accounted for 79% of all PIDs. The mean age (standard deviation) at diagnosis was 46 (50) months and the male to female ratio was 1.5:1. A history of parental consanguinity was present in 3 children (1.2%). Recurrent infection was the most prevalent presenting phenotype, manifesting in 70.2% of the patients. Genetic or chromosomal confirmation was obtained in 42/252 (16.7%) of the children. Common interventions used to prevent infection were antimicrobial prophylaxis and immunoglobulin replacement therapy, administered to 37.7% and 36.9% of the patients respectively. Six of seven children who underwent haematopoietic stem cell transplantation (HSCT) had successful outcomes. The 7th patient died 2 months post-HSCT from overwhelming infection. Although we could not account for the children lost to follow up during the study period, 53 (21.0%) deaths were confirmed. Conclusions. Several challenges exist in the recognition and treatment of children with PIDs in our setting. These include limited access to genetic diagnostics and HSCT. Sub-optimal treatment options contribute to the overall mortality of PIDs in South Africa. Greater awareness among clinicians treating children and more laboratory diagnostic capacity are needed to increase the recognition PIDs among children in South Africa. The treatment options that are available in South Africa are unevenly distributed. Hence, treatment capacity should be expanded throughout the country, especially advanced interventions such as HSCT. Ongoing reporting of registries such as ours and increased community awareness should strengthen the lobby for greater investment in rare diseases such as the PIDs.
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    A retrospective description of primary immunodeficiency diseases at Red Cross War Memorial Children's Hospital, Cape Town, South Africa, 1975-2017
    (2023) Moodley, Sashmi; Donald, Kirsten
    Background: Even in the absence of global threat, children with disabilities were underrepresented in monitoring efforts and were less likely to have been included in response strategies during the COVID - 19 pandemic. The harmful effects of the pandemic were expected to be most damaging for those in the poorest countries. Objectives: Our study aimed to report the impact of the COVID-19 pandemic on families of children with disabling conditions in South Africa. Methods: Parents from an online parent network, initiated by Shonaquip Social Enterprise during the pandemic, were invited to participate in an online survey in October 2021. A quantitative analysis was conducted using Stata Statistical Software. Results: Sixty-eight (17%) of 400 parents in the network, completed the survey, 89% were mothers. Children aged 5-12 years represented 43% of the group. Eleven parents (16%) reported child health worsening compared to before the pandemic. Parent concerns included practicalities of infection control measures for their children and concerns about caregivers falling ill. Six (9%) children contracted COVID-19 infection and two (3%) children demised. Few families received telephonic contact from health providers. Indirect effects of the pandemic included loss of income and food shortages in more than half of the families. Twentytwo( 32%) families had opted not to send their children back to school even by the third wave of infections. Some positive experiences of lockdown reported by parents included more family time and the opportunity to reach out to other families, however 33 (49%) parents reported being highly stressed, with five (7%) revealing a need for substances to cope. Most parents (n = 50, 74%) reported feeling that care for children with disabilities was not prioritized in South Africa. Conclusion: This is one of the few studies in South Africa that explored childhood disability during the pandemic. A proportion of caregivers reported deterioration in their children's health during a time of limited access to health services. They have reported loss of family income, reduced access to educational services and challenges to mental health of caregivers. Families did not feel supported. Including the voices of parents highlight a range of challenges that need greater awareness. Post-pandemic, much change in practice is needed for families and children with disabilities.
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    A retrospective review of acute liver failure in children admitted at Red Cross War Memorial Children's Hospital
    (2021) Mlotha-Mitole, Rachel; Goddard, Elizabeth; de Lacy, Ronalda
    Acute liver failure (ALF) describes a clinical syndrome resulting from severe liver damage and extensive loss of functional parenchymal liver mass triggered by various factors. Early recognition and initiation of specific therapy may improve outcomes and reduce the need for liver transplantation, a treatment modality not universally available in resource constraint areas. There is paucity of data describing this syndrome in Sub-Saharan Africa in children. Objective This study aims to retrospectively review and determine the clinical presentation, aetiology, complications & outcome of ALF in children admitted at the Red Cross War Memorial Children's Hospital (RCWMCH). Methods All records of children from 0 to 13 years admitted at the RCWMCH over the period from January 2005 to December 2016 with ALF were retrospectively reviewed, after obtaining ethical approval. Patients with pre-existing evidence of chronic liver disease were excluded. Demographic variables as well as clinical presentation and investigations were captured, with determination of outcomes at 3 weeks and 6 weeks of diagnosis. Results Study included 24 children., 16 females (66.7%) and 8 males (33.3%). Median Age was 15 months, with interquartile range from 5 to 28 months. Diarrhoea, jaundice, respiratory distress, hepatomegaly and encephalopathy were common clinical features. Aetiology was infection in 37.5 % of cases (n=9, 2 of whom had autoimmune hepatitis comorbidity) and hepatitis A was most common infectious cause (n=4, 44%). Causes were indeterminate in 29.2%. Two patients had autoimmune hepatitis without co-morbidity; Reye syndrome 12.5% and 17% had miscellaneous causes. Transaminases were raised to thousands in viral causes of hepatitis, with a low C reactive protein. INR >4 and Total Bilirubin>210umol/L were associated with death outcome (p=0.04 and p=0.03 respectively. Conclusion Viral hepatitis A is the leading infective cause of acute liver failure in this study cohort and 29.2% of cases were indeterminable. INR >4 and Bilirubin > 210umol/l were predictors of poor outcome. Follow up study is recommended to better understand clinical spectrum and outcomes of children with acute liver failure in this low resource setting.
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    A retrospective review of all children admitted with acute severe asthma to the paediatric intensive care unit, Red Cross War Memorial Children's Hospital between 2009-2019.
    (2023) Salie, Moegamad; Salie, Mogamat
    Background and Aim Asthma is one of the commonest chronic conditions of childhood and affects children worldwide. The majority of children who experience an acute exacerbation of asthma do not require admission to a paediatric intensive care unit (PICU). There is limited data on the admission rates, treatment modalities and length of PICU stay, for children who have acute severe asthma (ASA) in a South African context. In this study, we aim to describe the patient profiles and treatment of all children admitted to the PICU with ASA. Method We conducted a retrospective audit of all children admitted with ASA to the paediatric intensive care unit at Red Cross War Memorial Children's Hospital in Cape Town, South Africa between 01 January 2009 - 31 December 2019. Results There were 14592 PICU admissions over the 11-year period, of which 180 admissions (1,2%) were for acute severe asthma. There were 96 male (53,3%) admissions and the median, interquartile range (IQR) age on admission was 67 (37 – 93) months. Nearly all the patients received nebulisations, steroids, and magnesium sulphate before PICU admission. Half of the patients were loaded with IV salbutamol (n=96; 53,3%) and about a third (n=61; 34%) received a salbutamol infusion before admission to PICU. Similar proportions received nebulisations and steroids in PICU, 34 patients (19%) received magnesium sulphate again in PICU and a total of 130 patients (72,2%) received a salbutamol infusion. Most children received non-invasive respiratory support (n=167; 90,3%), and 18 children (9,7%) required mechanical ventilation for a median (IQR) of 3 (2 – 4) days. The median PICU stay was 1 (IQR 1 – 2) days and median hospital stay was 4 (IQR 3 – 6) days. No children died. Conclusion There has been an increasing number of children admitted to PICU with ASA over the 11- year period. There has been increased utilization of non-invasive ventilation (NIV) strategies, mainly HFNC and the duration of PICU support is short.
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    A Retrospective review of medical gastrointestinal endoscopy in children attending Red Cross War Memorial Children’s Hospital, Cape Town
    (2019) Eke, Christopher Bismarck; Goddard, Elizabeth; de Lacy, Ronalda; Brown, R A
    Background: Gastrointestinal endoscopy has evolved to become an important diagnostic, therapeutic as well as surveillance and follow-up modes of management in children with diverse gastrointestinal diseases. There is a paucity of data on gastrointestinal endoscopy in children in the sub- Saharan African region. The objectives of the study were to describe the socio-demographic characteristics; presenting symptoms; indications; endoscopic yield; impact of endoscopy on management; as well as its safety profile and complications. In addition algorithms for the indications of medical gastrointestinal endoscopy in children were designed using the results derived from the presenting symptoms and indications for gastrointestinal endoscopy among the patients. Methods: This was a cross sectional descriptive study. Subjects were children < 18 years attending Red Cross War Memorial Children’s Hospital (RCWMCH) who underwent medical gastrointestinal endoscopic procedures from 2007 to 2016. Study ethical approval was obtained from University of Cape Town while written permission from the RCWMCH Research and Management Committee prior to the commencement of the study. Data sheet was used in retrieving relevant patients variables from the hospital’s medical records and the Division of Paediatric Gastroenterology endoscopy and laboratory (histopathology) databases. Data was analysed using Stata 13.1. A p- value of less than 0.05 was considered statistically significant. Results: A total of 402 children were studied with 773 endoscopies performed comprising 670 oesophagogastroduodenoscopies (OGD) and 103 colonoscopies. For OGD: 179 (26.7%), 287(42.8%) and 204 (30.4%) procedures were for diagnostic, therapeutic and follow - up indications. A total of 78 (10.1%) combined OGD/colonoscopy were carried out. Out of 103 total colonoscopies performed, 67 (65.0%), 30(29.1%), and 6 (5.8%), were for diagnostic, follow - up and therapeutic indications respectively. vi Feeding difficulty 112 (25.4%) and rectal bleeding 11 (2.7%) were the main presenting symptoms for OGD and colonoscopy respectively. Main diagnostic indications for OGD, combined OGD/colonoscopy and colonoscopy alone respectively were chronic abdominal pain 51 (12.6%) and probable inflammatory bowel disease (IBD) 30 (7.5%) and IBD 30 (7.5%). Change 143 (35.6%)/ insertion 87(21.6%) of percutaneous gastrostomy were the most common therapeutic indications for OGD and polypectomy 8 (2.7%) for colonoscopy. Abnormal (positive) macroscopic findings on endoscopy were reported on 79/179(44.1%), 35/68(51.55%), and 46/67(53.7%) of OGD, combined OGD with lower scope, and colonoscopy alone respectively. Also, positive histological findings on OGD, combined OGD with colonoscopy, and colonoscopy alone were reported in 62/179(34.6%), 34/68(50.0%), and 32/67(47.8%) respectively. The overall normal endoscopic findings (both abnormal macroscopic findings on endoscopy and histological findings) were 63/179(35.3%) and 25/67(37.3%) for OGD and colonoscopy while overall diagnostic (endoscopic) yield was 116/179(64.8%) for OGD and 42/67(62.7%) for colonoscopy respectively. For OGD the main endoscopic yield reported were gastritis in 50(27.9%) and oesophageal varices 31(17.3%) while inflammatory bowel disease (Crohn’s disease 9(13.4%), ulcerative colitis 7(10.4%), juvenile polyps 9(13.4%) and intestinal tuberculosis 7(10.4%) were observed in colonoscopy respectively. A significant impact of endoscopy on the management of subjects were recorded in 298(74.1%) (p < 0.001) including diagnostic (change of medication, addition of new medication) and therapeutic (insertion/change of PEG; sclerotherapy 29 (9.8%) , band ligation of oesophageal varices 28 (9.4%), and polypectomy 8(2.7%)). The overall complication rate was 4.0% (16 patients). Conclusion: Feeding difficulty and rectal bleeding were the most common presenting symptoms for OGD and colonoscopy; with chronic abdominal pain and IBD being the most common indication for performing OGD and colonoscopy respectively. Therapeutic modalities of endoscopy performed were PEG insertion/change, polypectomy, sclerotherapy/band ligation for varices. vii Endoscopic yield was 116/179(64.8%) for OGD and 42/67(62.7%) for colonoscopy respectively a significant impact of endoscopy on the management of subjects were recorded in 298(74.1%) (p < 0.001). No mortalities were recorded following the procedures, however 16(4%) had some complications. Use of societal guidelines in selecting children with appropriate indications for gastrointestinal endoscopy will result in higher diagnostic yield and application of therapeutic modalities in children with gastrointestinal disorders resulting in significant impact on patient’s management and minimize complications.
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    A Retrospective Review of Paediatric Non-Infectious Uveitisin Cape Town: Disease Characteristics and Outcomes on Immunomodulating Treatment
    (2020) Slamang, Waheba; Scott, Christiaan
    Background Uveitis is a known cause of blindness in the developed world, where non-infectious diseases dominate the spectrum of underlying aetiologies. However, data from sub-Saharan Africa is lacking. Here we aim to describe the diseases associated with non-infectious uveitis and the impact of currently available treatment in this setting. Methods A retrospective observational analysis of children with non-infectious uveitis from January 2010 to December 2017, attending the tertiary paediatric rheumatology and ophthalmology referral units in Cape Town was conducted. Statistical analysis utilising STATA13 software was performed with p < 0.05 considered significant. Results Twenty-nine children were identified with a median age at first visit of 74 months (IQR 49–86 months), female to male ratio of 0.9:1, predominantly of mixed race (72.4%). Juvenile idiopathic arthritis associated uveitis (JIAU) (48.3%) was the most frequent diagnosis. All children with JIAU had chronic anterior uveitis and 3 (21.4%) presented with uveitis before arthritis. There were no differences between children with uveitis and those with arthritis only, for gender (p = 0.68) and race (p = 0.58) but significantly, children with uveitis presented at an overall younger age (p = 0.008), with antinuclear antibody positive (p < 0.001) oligo-articular JIA (p = 0.01) and older age appeared to be protective (p = 0.01 OR1.0 CI 0.6-1.7). Children with idiopathic uveitis (41.4%) were predominantly male (66.6%), of mixed race (75%), with chronic anterior uveitis (41.7%) and presented with cataracts (100%). Less commonly, sarcoidosis (6.9%) and Behcet's disease (3.5%) were diagnosed. 55.2% had complications at presentation, predominantly cataracts (87.5%). 19 children (65.5%) had inactive disease at 12 months from diagnosis and remission as assessed at the last clinical visit, was achieved in 58.6% on standard initial therapy and in 75% of those on tumour necrosis factor inhibitors. Surgery was needed in 41.4%, primarily in the idiopathic group. Visual acuity improved or was maintained on treatment. Conclusion The spectrum and characteristics of immune mediated non-infectious uveitis are comparable to that reported in developed countries. Current practice detects children with potentially sight-threatening disease and access to tumour necrosis factor inhibitors has improved outcomes in refractory cases.
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    A review of the use of high flow nasal cannula oxygen therapy in hospitalized children at a regional hospital in the Cape Town Metro, South Africa
    (2018) Hoffman, Elizabeth; Cooke, Melissa Louise
    Background: High-flow nasal cannula oxygen (HFNC) is a non-invasive alternative to nasal continuous positive pressure oxygen (CPAP) therapy for infants and children requiring respiratory support. There is a paucity of literature to support its use in children, with no published data from sub-Saharan Africa. Objective: To describe the outcomes and adverse events of HFNC in the first year of its use in a level two (L2) general paediatric ward, compared with outcomes of a historical cohort when this intervention was unavailable. Methods: This retrospective descriptive study included children aged <13 years who received HFNC in the first 12 months after its introduction (HFNC-availability group; n=66). Demographic data, clinical characteristics, and outcomes (death, treatment failure, length of HFNC, and HFNC-related adverse events) were assessed. A comparative description of children that required transfer to level 3 (L3) for respiratory support (more than available standard low-flow oxygen) in the 12-month period prior to HFNC availability (pre-HFNC group; n=54) was performed and outcomes were compared using standard descriptive and comparative statistics. Results: The median age of the cohort was 5 months (interquartile range [IQR] 1.9– 14.6). Sixteen children (13.3%) were malnourished, 10 (8%) were HIV infected, and 30 (25%) were ex-premature infants. The most common diagnoses were pneumonia, bronchiolitis, and asthma. Asthma, anaemia, and cardiac abnormalities were the most prevalent underlying co-morbidities. Two children died in each group. All 54 children in the pre-HFNC group were transferred to L3; 38 (70.4%) needed CPAP or invasive ventilation. In the HFNC-availability period, 85 children were assessed as needing more than standard low-flow oxygen therapy: 19 were immediately transferred to L3 where 17 (89.4%) received CPAP or invasive ventilation; 66 received HFNC at L2, 16 (24.2%) of these children required transfer to L3 for CPAP or invasive ventilation. The median duration of HFNC was 46.3 h (IQR 19.5–93.5) overall, and was 12 h (IQR 4-28) and 58.5 h (IQR 39.5–106) for those who failed or were successfully managed on HFNC, respectively. No HFNCrelated serious adverse events were recorded at L2. Conclusion: HFNC is a safe, effective, feasible option for non-invasive ventilation of children with respiratory illnesses in a resource-limited L2 setting. A greater proportion of children admitted with lower respiratory tract infections required support in the HFNC-availability group, but the intervention reduced the bed- pressure on L3. Improved identification of HFNC failures and better adherence to the protocol is needed at L2.
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    A review of the use of inhaled nitric oxide in the PICU at Red Cross Children's Hospital, 2011-2015: A retrospective cohort study
    (2019) Padayachee, Sandhia; Salie, Shamiel
    Background: Inhaled Nitric Oxide (iNO) functions as a selective pulmonary vasodilator. It is an expensive treatment that is often employed as rescue therapy for refractory hypoxaemia in acute respiratory distress syndrome (ARDS) and pulmonary hypertension (PHT) following cardiac surgery. Objectives: To describe the response to treatment with iNO. Secondary observations were deaths, comorbidities of the patients treated, lengths of treatment and admission, and the cost of treatment. Methods: A retrospective descriptive study of all patients treated with iNO in the Paediatric Intensive Care Unit (PICU) at Red Cross War Memorial Children’s Hospital (RCWMCH) from 2011- 2015. Results: A total of 140 patients were treated with iNO during this time period, 82 were for PHT following cardiac surgery, 53 for ARDS and 5 for PPHN. A response to treatment was observed in 64% of the cohort as a whole, 80% of those with PPHN, 67% of those with PHT post-cardiac surgery, and 64% of those with ARDS. A longer duration of ICU and hospital admission, and higher in hospital mortality (53%) was seen in the group with ARDS, in particular those with adenoviral infection (63%), when compared to patients treated for PHT (18%) and for PPHN (20%). There is no protocol in place guiding the use of iNO in our unit, and it was found that response to treatment was not being objectively measured and documented and that practise varied between clinicians. Conclusions: Considering the cost of treatment and lack of evidence to support beneficial effects of iNO therapy, its continued use in our resource poor setting should be guided by protocol.