Rationale: The paucity of access to electroencephalograms (EEGs) in sub-Saharan Africa results in a high patient load attending the few centres with neurophysiology units. Sleep state for EEGs performed on children improves yield and reduces artefact. Melatonin induces “natural sleep” without the risk of airway compromise. This study evaluated the effectiveness of oral melatonin in attainment of useful electroencephalograms in South African children. Methods: Consecutive children booked for routine EEG who were either unable to cooperate or were referred for sleep EEG received oral melatonin (3mg < 15kg; 6mg > 15kg) (September 2013-March 2014). Comparison was made to a retrospective control group who received the previous sleep protocol agent, chloral hydrate. Outcome measures were the proportion of children who achieved sleep, useful EEG study data, sleep latency and duration, presence and level of artifacts and presence of recorded EEG study abnormalities. Results: 173 children were recruited, 88 (51%) male, median age 4 years 9 months (interquartile range of 2 years 2 months – 7 years 6 months). 87% of the children achieved stage 2 sleep and were deemed to have successfully entered sleep state. The median sleep latency was 44.5 minutes and the duration of sleep was 25 minutes (range 18.5 – 29 minutes). Children showed no signs of post-sedation irritability or persistent drowsiness. They were awoken and were immediately able to go home. In the melatonin group there were no adverse events, and no child needed their study deferred due to inter-current illnesses. All children administered melatonin cooperated and permitted a successful EEG recording with useful records even if sleep was not achieved. Sedation with melatonin was less successful (74% compared to 88%) in children with developmental and behavioural problems (χ 2 = 6.18, P= 0.046), they also had higher rate of artifacts (χ 2 = 5.83, P=0.05). 33.5% of the study group children (n=58) had abnormal EEG studies. These outcomes were comparable to a historical cohort of age equivalent children who were sedated with chloral hydrate (45.5%) (χ 2 = 1.22, P= 1.27). 79% that received melatonin compared with 86% of those that were sedated with chloral hydrate had artifacts (χ 2 = 0.63, P= 0.42) Conclusion: Melatonin is effective and safe in inducing sleep for EEG recording in our setting.

Includes bibliographical references. / Objective: To investigate the difference in expression of CD146 immunohistochemical staining on intermediate trophoblast in early and late onset pre-eclampsia placentas as well as comparing this expression to gestational age matched control placentas. Study Design: Retrospective case series of 100 placentas: 25 early onset and 25 late onset pre-eclampsia placentas as well as 25 early and 25 late gestational age matched control placentas. Placentas were obtained from patients delivering in Tygerberg Hospital. Methods: Placentas were routinely fixed and processed. 2 sections of one preselected block from each case was cut and stained with CD146 and MNF116 immunohistochemical stain. The expression of the staining would be performed by means of analytical and image analysing software. Results: The study failed to demonstrate differences in CD146 expression by the intermediate trophoblast in the pre-eclampsia and control placentas. The analytical approach was deemed to be subjective and the image analysing software had too much background staining and inaccurate identification of the intermediate trophoblast in order to produce reproducible consistent results. Conclusion: Dual staining, using immunofluorescent staining of CD146 and MNF116 on smaller biopsies of the decidua are thought to be able to produce much better material for image analyses software.

Post-operative analgesia currently relies on multimodal therapy including epidural analgesia, intravenous morphine and/or paracetamol (Perfalgan ®) infusion. Local wound infusion has been effectively utilized in adults with promising results but has not been prospectively tested in children undergoing different abdominal operations. The aim of this study was to compare continuous local anesthetic wound infusion to the current standard of care in post-operative pain control in children. Methods: We conducted a prospective randomized, pain assessor blinded trial comparing Bupivacaine wound infusion {Continuous Local Anaesthetic Wound Infusion - CLAWI) in addition to intravenous paracetamol (Perfalgan®) and morphine for rescue analgesia. This was compared to: (a) epidural bupivacaine plus intravenous morphine and Perfalgan® [EPI] for children undergoing open abdominal surgery and (b) intravenous morphine and Perfalgan® infusion alone [standard post-operative analgesia - SAPA] in children undergoing Lanz incision laparotomy for complicated appendicitis. Patients aged between 3 months and 12 years undergoing laparotomy or open appendectomy were randomly selected for local anesthetic wound infusion (CLAWI) versus EPI or CLAWI versus (SAPA) respectively. Exclusion criteria were neurological impairment, post-operative ventilation and history of adverse reaction to bupivacaine. Consent from the guardian, assent from patients above the age of 7 years and ethics approval from the University of Cape Town Human Ethics Research Committee was obtained. The wound infusion catheter ('lnfiltralLong', PANJUNK®) was placed sub-fascially after suture of the peritoneum and 0.2 % bupivacaine 2mls/kg infused on anesthetic reversal followed by 0.2ml/kg/hour thereafter for 48 hours. Pain assessments were performed for each patient at regular intervals by a single assessor who had training in pediatric pain management and who was blinded to the group allocation. The duration of surgery, length of incision, perioperative antibiotics, wound class risk of surgical site infection, time to return to full feeds, drug reactions; hospital stay, surgical site infection and wound catheter and epidural catheter complications were recorded for each patient. Primary outcome measure was total morphine used in the appendectomy-SAPA vs appendectomy-CLAW! group and rescue morphine requirements in the laparotomy-EPI vs laparotomy-CLAWI group. The secondary outcomes were pain control as measured using the FLACC scale, time to full feeds, mobilization and requirement for urinary catheter.

Background: Klebsiella pneumoniae (KP) is a significant paediatric bloodstream pathogen in children. There is little data about KP in South African children. The focus for the present study was to address this knowledge gap. Methods: This study addressed a retrospective case notes review on bloodstream Klebsiella pneumoniae infections at a children's hospital in Cape Town, South Africa 2006-2011 using conventional descriptive and comparative statistical methods. Results: Of 410 hospitalised children with laboratory confirmed KP bloodstream infection (KPBSI), 339 (83%) were presumed extended-spectrum β-lactamase (ESBL) producing isolates. The median age (interquartile range, IQR) was 5.0 (2-16) months, 212 (51.7%) were male, 82 (20%) were HIV-infected, and 241 (58.8%) were moderately or severely underweight. The infection was nosocomial or healthcare-associated in 389 (95%) children and community-acquired in 21 (5%) children. Significant risk factors for the acquisition of ESBL-KP bloodstream infection included cephalosporin exposure in the preceding 12 months prior to the KPBSI p=<0.0001: aRR 1.25 (95% CI: 1.15-1.36); and those who had intravenous infusions for more than 3 days prior to the KPBSI, p=0.004: aRR 1.18 (95% CI: 1.05-1.31).

Pneumonia due to polymicrobial infection is known to increase the severity and risk of fatality among young children. A retrospective study was undertaken on Sudden Unexpected Infant Death cases occurring, between 1 May and 30 September 2009, which were admitted to a medico-legal mortuary servicing the Cape Town western metropole. Published studies have shown the risk factors for lower respiratory tract infection to include lack of breast feeding, prenatal and environmental tobacco smoke exposure, prematurity, immunosuppression, underlying medical conditions and overcrowding. The present study was aimed at determining which of the known epidemiological factors were associated with SUDI death types admitted to this mortuary and to describe the associated histopathology. In addition, in the knowledge that drugs, specifically Methamphetamine are widely used on the Cape Flats from where almost all this mortuary's SUDI cases are derived, this study has attempted to find out whether or not the usage of drugs by the caregiver at the time of infant death was another independent risk factor in SUDI deaths.

Includes abstract. / Includes bibliographical references (leaves 56-60). / To audit the crod blood thryoid screening programme in the Peninsula Maternal and Neonatal Services (PMNS) in the 5 year period from 01/01/2000 to 31/12/2004, focusing on coverage, recall rate and success, number of cases detected, incidence of congenital hypothryoidism in this population; and cost efficiency of the programme. All babies born in the PMNS from 01/01/2000 to 31/12/2004 were included in the audit. The medical records of all babies recalled following an abnormal screen were examined. 140 507 babies were born in the PMNS during the audit period, while 130 389 primary Thyroid Stimulating Hormone (TSH) screens were done (92.8% coverage). 2 207 of the screened babies had abnormal results requiring review.

Background Cystic fibrosis (CF) is described more commonly in Caucasian populations in whom p.Phe508del is the most common mutation. There is a paucity of data of CF in black African children. The aim of this study was to describe and compare the presentation and outcomes of black African children with CF to those with p.Phe508del genotype. Methods A retrospective case-controlled study was conducted from January 2000 – March 2018 of children with CF attending two CF centres in South Africa. Presentation, genotype, nutrition and pulmonary function outcomes of black African children were compared to matched controls with the p.Phe508del mutation. Results Thirty-four black African children (cases) with median age of diagnosis (5.5 months, IQR 2.0- 15.0) were matched to 34 controls. Among cases, 3120+1G->A CFTR mutation was most commonly identified; homozygous n=22 (64.7%) and heterozygous=7(20.5%). Compared to controls, cases at diagnosis were more malnourished and fewer presented with neonatal bowel obstruction [cases n=2 (5.9%) vs. controls n=10 (29.4%); p = 0.03]. Nutrition and pulmonary function (FEV1 in children ≥ 6 years) outcomes and changes over time from ages 3-16 years were similar in both groups; median FEV1 z-score at age 6,10 and 14 years was -0.9 (±1.5), -1.8 (±2.0) and -1.8 (±1.9) respectively for all patients. Deaths were recorded in three cases (8.8%) and one control (2.9%) (p = 0.6). Conclusion Black African children with CF were more malnourished at diagnosis, and fewer presented with neonatal bowel obstruction. Cases and controls had comparable nutritional, pulmonary function and early mortality outcomes.

Introduction: Epilepsy is the most common neurological condition worldwide. Literature on the antiepileptic medications and biochemical markers of bone metabolism has revealed inconsistent results. Most of these studies were undertaken in Europe and America where the burden and the associated comorbidities are different to the ones in Africa. Methods: A hospital based case control study was undertaken at Red Cross War Memorial Children's Hospital where children were recruited from a dedicated Epilepsy clinic and controls were obtained from a day surgical ward. Blood and urine samples were taken for the assessment of markers of bone metabolism. Results: Seventy-five cases and 75 controls were recruited. The median age for the children with epilepsy was 9 years with a range of 1 to 17 and controls 3 years with a range of 1 to 12. Vitamin D deficiency was present in 11(16.2%) of children with epilepsy compared to 6(8.8%) in the control group. Vitamin D insufficiency was present in 30(44.1%) in children with epilepsy compared to 27(39.7%) in the control group. Children with epilepsy on enzyme inducing AEDs had lower mean Vitamin D levels (24.67±11.4 vs 30.72±7.4, p=0.08), lower mean Vitamin D2 (0.25±0.07 vs 0.4±0.17,p=0.0018),lower mean Vitamin D3 (1.61±1.06 vs 2.58±0.86,p=0.004), lower mean serum phosphate levels (1.39±0.2 vs 1.76±0.7,p=0.000) and a higher mean parathyroid hormone levels (4.47±2.33 vs 2.7±0.97, p=0.03) compared to the control group. Children with Epilepsy on enzyme inhibitors had higher mean Vitamin D2 (0.44±0.37 vs 0.25±0.07,p=0.000004) and mean Vitamin D3 (2.26±0.86 vs 1.61±1.06,p=0.028) compared to children on enzyme inducers. Dietary intake and ancestry did not influence Vitamin D levels between the cases and controls. Conclusion and Recommendations: Vitamin D deficiency is common in children with epilepsy on AEDs. Children on enzyme inducing AEDs should be investigated for vitamin D deficiency and managed accordingly.

Medulloblastoma is the most frequently occurring childhood malignant brain tumour, affecting 1 of 5 children presenting with a brain tumour, between the ages of 0 and 9 years. The basic prognostic stratification that relies on clinical and histological findings alone has proven unsatisfactory as an outcome predictor. Distinct molecular genetic profiles have been described, with four molecular variants of medulloblastoma with specific demographic and prognostic features. These are the WNT subgroup, SHH subgroup, Group 3 and Group 4 tumours. The aim of this study was to describe the expression status of β-catenin, and MYCN, using IHC and FISH respectively, and to correlate these findings with clinico-pathological and demographic characteristics and clinical outcome. Materials and Methods This study was a nested retrospective analytical study, reviewing 54 cases of childhood medulloblastoma diagnosed between 1988 and 2014. Results Classic histology accounted for 40.7% of cases, LCA 37%, ND 16.7% and 5.6% MBEN). Based on β-catenin IHC, the WNT subgroup accounted for 16.7% of cases. This group had no mortalities or recurrences. Seven patients showed amplification of MYCN gene. The SHH group, defined by ND/MBEN histology and/or MYCN amplification, accounted for 27.7% of patients. Non-WNT/non-SHH tumours 30 patients (55.6%) showed a male predilection, and accounted for 37.5% recurrences and 50%. mortalities also falling in this group. Conclusions Nuclear β-catenin identifies WNT tumours. Nodular desmoplastic morphology is useful in identifying some, but not all cases of SHH group medulloblastomas. MYCN positive tumours also showed classical, and LCA morphology.. Patients of all the beta-catenin positive cases were free of recurrence and alive at last follow up. Patients with MYCN amplification and non-ND histology (LC/A or classic) had poorer outcomes than patients with ND histology. One patient showed both MYCN amplification and nuclear β-catenin translocation, and had good clinical outcome. This finding requires validation with other molecular techniques.

Introduction: Technecium (Tc) 99m Sucralfate scan has been shown to be a reliable and non-invasive screening modality after caustic substance ingestion, followed by oesophagoscopy under general anaesthesia to grade the extent and severity of injury. Aim: To determine a correlation between the 99mTc Sucralfate scan and the endoscopy findings in children presenting with caustic oesophageal injury. Methods: An observational analytic study of children who had both 99mTc Sucralfate scan and endoscopy after caustic substance ingestion at Red Cross War Memorial Children’s Hospital in a period between January 2009 and September 2016. The oesophageal injury was classified into low grade and high grade according to the degree of adhesion on 99mTc Sucralfate scan and modification of Zargar endoscopic grading. Approval of the study by the University of Cape Town Faculty of Health Sciences Human Research Ethics Committee was obtained, REF. 049/2017. Results: Out of a total of 197 children, 40 children were identified who had both investigations done on average 26hours post injury. Low grade adhesion on 99mTc Sucralfate scan was found in 27 children (68%), and all had low grade Zargar’s oesophageal injuries. None of these subsequently developed residual pathology. Thirteen had high grade adhesion and five of these had high grade injury on endoscopy. Three (23%) developed oesophageal strictures. Correlation of 99mTc Sucralfate and endoscopic findings reached statistical significance with a p-value of 0.0014. No morbidity was associated with either the scan or endoscopy. Conclusions: Low grade Sucralfate scan finding has potential to successfully eliminate the need for invasive endoscopy under general anaesthesia and thereby reducing procedure related morbidity, hospitalization and associated costs. However, mandatory endoscopy is required in children with high grade adhesion seen on 99mTc Sucralfate scan. This requires confirmation using a larger prospective study.