Haemophagocytic lymphohistiocytosis following culture-proven pneumococcal infective endocarditis of the tricuspid valve

We report a case of a 2-year-old boy presenting with persistent fever and splenomegaly who fulfilled the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH) according to the Histiocyte Society (2004). The persistence of a cardiac murmur despite multiple transfusions, and therapy which rendered him afebrile, led us to do an echocardiogram as part of surveillance for sepsis. This revealed tricuspid vegetation and a small ventricular septal defect. Blood culture and postoperative histology of the anterior leaflet of the tricuspid valve confirmed Streptococcus pneumoniae infection. The patient was successfully treated with intravenous antibiotic therapy for 6 weeks and dexamethasone for 8 weeks and remains well and in remission (from HLH) a year later with residual tricuspid regurgitation awaiting tricuspid valve replacement

[123I]-metaiodobenzylguanidine imaging findings and outcome in patients with metastatic neuroblastoma

Background: 123I-Metaiodobenzylguanidine ([123I]mIBG) is the agent of choice to assess for presence of metastases and therapy response in patients with neuroblastoma. Aim: To assess [123I]mIBG scan results and outcome in patients with stage 4 neuroblastoma at our institution. Setting: Red Cross War Memorial Children’s Hospital. Methods: A retrospective review of baseline and follow-up [123I]mIBG scans of patients who presented between January 2001 and May 2015. The clinical follow up extended until October 2019. The association between the baseline and post-induction Curie score (CS) and overall survival (OS) were assessed. Results: Thirty-four patients with stage 4 disease were included. Twenty-two (65%) patients died. The median age at diagnosis for survivors was 15.5 months vs 39 months for those who died (Kruskal Wallis c2 = 4.63, p = 0.03). Neither the baseline CS nor the post-induction CS predict the outcome or duration of survival. The median OS for a baseline CS ≤ 12 and CS 12 was 19 and 26 months, p = 0.13. The median OS for a post-induction CS 2 and CS ≤ 2 was 28 and 26 months, p = 0.66. Conclusion: In this study, baseline, post-induction and reduction in CS did not predict OS in stage 4 neuroblastoma. Factors such as small patient numbers, less intensive treatment regimes, and possible poorly dedifferentiated disease have been identified for this finding. Contribution: In contrast to international studies the Curie score did not predict treatment outcome in the South African setting where the vast majority of patients are treated with OPEC/OJEC OPEC/OJEC (vincristine [O], cisplatin [P], etoposide [E], cyclophosphamide [C] and carboplatin [J]) chemotherapy

A cost comparison of rasburicase versus dialysis in the management of children with acute leukaemia and lymphoma at a South African centre

Background: Tumour lysis syndrome is a common complication of haematological malignancies and has historically been managed with hyperhydration, urine alkalinisation and allopurinol with renal dialysis reserved for patients in acute renal failure. Rasburicase has been shown to drastically reduce the need for dialysis; however, its use is limited in developing countries owing to its cost and availability. Aim: This retrospective analysis aimed to compare the cost to state per patient of rasburicase compared to dialysis in the management and prevention of tumour lysis syndrome in paediatric patients presenting with haematological malignancies admitted to Red Cross War Memorial Children’s Hospital (RCWMCH). Setting: Red Cross War Memorial Children’s Hospital. Methods: Patients from two consecutive 35 month periods, before and after the availability of rasburicase at RCWMCH, were grouped according to treatment modality, and the cumulative costs of hospitalisation, dialysis and drug administration were compared. Results: The groups were comparable in mean age and gender. The mean total length of hospital stay was 10.04 days shorter for the rasburicase group than the dialysis group with the average cost per patient in the rasburicase group being R40 989.64 lower than the dialysis group. Conclusion: The use of rasburicase results in a significant per patient cost saving when compared to dialysis, which often requires intensive care admission, and results in extended hospitalisation. The study supports the continued use of rasburicase as an essential adjunct in the management and prevention of tumour lysis syndrome, reaffirming its use as a cost-effective and efficient drug

Building a psychosocial and spiritual care service for children with cancer and their families

Background: Comprehensive, coordinated psychosocial, supportive and spiritual care is an essential component of the holistic care of childhood cancer sufferers and their families. Aim: The authors detail the development and value of a multidisciplinary psychosocial care team as an essential adjunct to care of childhood cancer sufferers. Methods: A historic preamble details a period during which psychosocial and supportive care was the sole province of the paediatric oncologists and social workers and describes that the process of creating a multidisciplinary psychosocial and spiritual care team has enhanced medical care. Results: Each member of the psychosocial group describes their care philosophy and their role in the clinical setting. We also describe the critical role of the meeting as a teaching vehicle for oncology fellows. Conclusion: This reproducible partnership between public and private sector practitioners, designed in a resource-constrained setting, affords a diverse and highly skilled group of professionals the opportunity to meet the medical, psychological, social and spiritual needs of patients and families as they transition through the care journey

Bloodstream infections in oncology patients at Red Cross War Memorial Children’s Hospital, Cape Town, from 2012 to 2014

Objectives: This study was performed to investigate the epidemiology of bloodstream infection (BSI) in oncology patients at Red Cross War Memorial Children’s Hospital (RCWMCH), Cape Town, with focus placed on the most common causes, complications, and antimicrobial susceptibilities in BSI. Methods: A retrospective cross-sectional study was conducted in the Haematology–Oncology Unit of RCWMCH. All positive blood cultures from RCWMCH oncology patients obtained in 2012 to 2014 were retrieved to identify cases of BSI. Results: Three hundred and forty-three positive cultures were identified, for 150 BSI episodes among 89 patients; 49.1% of the culture isolates were Gram-positive bacteria, 41.6% were Gram-negative bacteria, and 9.3% were fungal. Coagulase-negative Staphylococcus and viridans group Streptococcus were the most common Gram-positive isolates. Escherichia coli and Klebsiella species were the most common Gram-negative isolates. The majority of BSI episodes occurred in patients with haematological malignancies (74%), in the presence of severe neutropenia (76.4%), and were associated with chemotherapy (88%). Complications occurred in 14% of BSI. Fungal infections had the highest prevalence of complications (21.4%). Three children died during BSI, giving a case-fatality rate of 2%. Conclusions: BSI in these patients was caused mainly by Gram-positive bacteria and was associated with a low case-fatality rate. These results are consistent with worldwide experience of BSI in paediatric oncology. Keywords: Bloodstream infection, Childhood cancer, Bacteraemi

Does somatosensory discrimination activate different brain areas in children with unilateral cerebral palsy compared to typically developing children? An fMRI study

Aside from motor impairment, many children with unilateral cerebral palsy (CP) experience altered tactile, proprioceptive, and kinesthetic awareness. Sensory deficits are addressed in rehabilitation programs, which include somatosensory discrimination exercises. In contrast to adult stroke patients, data on brain activation, occurring during somatosensory discrimination exercises, are lacking in CP children. Therefore, this study investigated brain activation with functional magnetic resonance imaging (fMRI) during passively guided somatosensory discrimination exercises in 18 typically developing children (TD) (age, M = 14 +/- 1.92 years; 11 girls) and 16 CP children (age, M = 15 +/- 2.54 years; 8 girls). The demographic variables between both groups were not statistically different. An fMRI compatible robot guided the right index finger and performed pairs of unfamiliar geometric shapes in the air, which were judged on their equality. The control condition comprised discrimination of music fragments. Both groups exhibited significant activation (FDR, p < .05) in frontoparietal, temporal, cerebellar areas, and insula, similar to studies in adults. The frontal areas encompassed ventral premotor areas, left postcentral gyrus, and precentral gyrus; additional supplementary motor area (SMAproper) activation in TD; as well as dorsal premotor, and parietal operculum recruitment in CP. On uncorrected level, p < .001, TD children revealed more left frontal lobe, and right cerebellum activation, compared to CP children. Conversely, CP children activated the left dorsal cingulate gyrus to a greater extent than TD children. These data provide incentives to investigate the effect of somatosensory discrimination during rehabilitation in CP, on clinical outcome and brain plasticity.publisher: Elsevier articletitle: Does somatosensory discrimination activate different brain areas in children with unilateral cerebral palsy compared to typically developing children? An fMRI study journaltitle: Research in Developmental Disabilities articlelink: http://dx.doi.org/10.1016/j.ridd.2013.02.017 content_type: article copyright: Copyright © 2013 Elsevier Ltd. All rights reserved.status: publishe

How does brain activation differ in children with unilateral cerebral palsy compared to typically developing children, during active and passive movements, and tactile stimulation? An fMRI study

The aim of the functional magnetic resonance imaging (fMRI) study was to investigate brain activation associated with active and passive movements, and tactile stimulation in 17 children with right-sided unilateral cerebral palsy (CP), compared to 19 typically developing children (TD). The active movements consisted of repetitive opening and closing of the hand. For passive movements, an MRI-compatible robot moved the finger up and down. Tactile stimulation was provided by manually stroking the dorsal surface of the hand with a sponge cotton cloth. In both groups, contralateral primary sensorimotor cortex activation (SM1) was seen for all tasks, as well as additional contralateral primary somatosensory cortex (S1) activation for passive movements. Ipsilateral cerebellar activity was observed in TD children during all tasks, but only during active movements in CP children. Of interest was additional ipsilateral SM1 recruitment in CP during active movements as well as ipsilateral S1 activation during passive movements and tactile stimulation. Another interesting new finding was the contralateral cerebellum activation in both groups during different tasks, also in cerebellar areas not primarily linked to the sensorimotor network. Active movements elicited significantly more brain activation in CP compared to TD children. In both groups, active movements displayed significantly more brain activation compared to passive movements and tactile stimulation.status: publishe