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  1. Home
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Browsing by Author "Scott, Christiaan"

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    Open Access
    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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    Adverse impact of hospitalisation on infant breastfeeding practices: a prospective cohort study
    (2019) Alisio, Michelle; Scott, Christiaan; Kroon, Max
    Background: In South Africa, the exclusive breastfeeding prevalence at six months is low at 24% and the under-5 mortality rate remains high. Improving breastfeeding rates is the most cost-effective intervention to reduce under-5 mortality and morbidity. Data on the effect of infant hospitalisation on breastfeeding may inform facility-based interventions to protect and support exclusive and prolonged breastfeeding. Aim: To assess the impact of hospitalisation on breastfeeding and explore reasons for stopping or continuing breastfeeding. Methods: We conducted a prospective cohort study of infant feeding practices among mother-infant dyads admitted to general paediatric wards at a tertiary children’s hospital in Cape Town, South Africa. Medical, demographic and feeding practice data were collected through semi-structured interviews on admission, again during hospitalisation and a third interview was conducted telephonically post discharge. Logistic regression analysis was used to assess factors associated with different feeding practices. Results: Between January and April 2018, 119 mothers (median age 26 years, IQR 22-32; 28% HIV-positive) were interviewed at admission; 39% (46/119) breastfed exclusively (EBF) and 28 (24%) reported no breastfeeding. Most infants (median age 1.8 months, IQR 1.0-3.2; 34% preterm) were admitted for lower respiratory tract infection (59%) or diarrhoea (14%). EBF at admission was associated with younger infant age (per month increase, aOR 0.18, 95% CI 0.07-0.43); none of the children admitted for diarrhoea had been EBF. A second in-hospital interview occurred at median 4 days (IQR 2-6) after admission. The overall prevalence of any breastfeeding declined from 77% at admission to 61% in-hospital. Risk factors for in-hospital breastfeeding cessation included low birth weight (<2500g; OR 3.81, 95% CI 1.35-10.74) and feeding via either bottle/tube (OR 51.00, 95% CI 6.38-407.71). Maternal expression of breastmilk (vs no expression in-hospital) was protective against in hospital breastfeeding cessation (OR 0.07, 95% CI 0.01-0.33). Post-discharge telephonic interviews (median 5 months after discharge) were available for 92 mother-infant dyads; 21 infants were ≤ six months of age, of whom 24% (5/21) were still exclusively breastfeeding. Breastfeeding cessation at any time after admission and before post-discharge telephonic interview was associated with maternal HIV infection (OR 2.82, 95% CI 0.84-9.40), full time employment (OR 4.95, 95% CI 1.40-17.46) and preterm birth (OR 3.53, 95% CI 1.27-9.81). Conclusion: Prevalence of both any and exclusive breastfeeding was low at admission to hospital, and lack of breastfeeding strongly correlated with increased risk of an infectious morbidity diagnosis. In addition, hospitalisation substantially reduced the probability of continued breastfeeding. In-hospital breastfeeding support and facilitation of breastmilk expression while infants are unable to breastfeed should be increased. Implementation research may define effective in-hospital breastfeeding support interventions.
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    Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study
    (BioMed Central, 2016-11-11) Okong’o, Lawrence O; Esser, Monika; Wilmshurst, Jo; Scott, Christiaan
    Background: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. Methods: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa. Results: Twenty five cases were identified: 16 female and 9 male; thirteen (52 %) were of indigenous African, eleven (44 %) mixed and one (4 %) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0–9.7) and 7.9 (range 3.4–9.75) years respectively. Eleven patients had calcinosis while the mortality was 2/ 25 (8 %). Only 40 % of the patients had clinically inactive disease by PRINTO criteria (modified) at last review. There was no statistically significant difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p-value = 0.75) between patients who had clinically active and inactive disease. Conclusion: The demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. Majority of the patients remained with clinically active disease, which put them at risk of further disease complications. Long term follow up and use of appropriate treatment guidelines may be indicated in management of JDM patients for optimum treatment outcomes.
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    Demographic and clinical characteristics of children with juvenile dermatomyositis in Cape Town
    (2015) Okong'o, Lawrence Owino; Scott, Christiaan
    Study rationale: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory myopathy of childhood with an incidence of 1.9-3.2 per million. The aetiology of JDM is uncertain but may result from immune dysregulation triggered by environmental factors in genetically susceptible children. The demographic and clinical characteristics of JDM may thus differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. There is need for further studies for better understanding of the epidemiology, clinical characteristics and outcome of patients with JDM from the continent. Methods: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004-2013 in Red Cross, Groote Schuur and Tygerberg hospitals in Cape Town. Data was analyzed using R version 3.1.0 (2014-04-10). Results: Twenty-five cases were identified: 16 female and 9 male. Thirteen (52%) of the cases were of indigenous African, eleven (44%) mixed and one (4%) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0-9.7) and 7.9 (range 3.4-9.75) years respectively. Muscle weakness and characteristic cutaneous manifestations occurred in all the 25 patients while 24 had elevated muscle enzymes. All the patients received corticosteroids, seventeen (73.9%) received methotrexate and four received rituximab. Eleven patients had calcinosis during the disease course [median follow up period of 50 (range 0.5-159) months]. The mortality was 2/25 (8%) while only 40% of the patients had clinically inactive disease by PRINTO criteria. There was no difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p -value = 0.75) between patients who had clinically active and inactive disease. Discussion: The demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. The median delay in diagnosis (4 months) was not longer than that reported in most other studies. However, some children had prolonged delay of up to 7 years due to misdiagnosis that denied them appropriate treatment in a timely manner. Majority (60%) of the patients also remained with clinically active disease, which put them at risk of further disease complications including calcinosis. Even though the mortality rate was low (8%) this was still more than double that reported in most recent large studies especially from the resource rich countries. Conclusions: Long-term follow up of JDM patients is advisable since majority of patients seem to have clinically active disease many years after disease onset despite treatment. Formulation and use of appropriate treatment guidelines and protocols may aid in the early diagnosis and appropriate management for optimum outcomes.
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    Juvenile idiopathic arthritis (JIA) in two tertiary centres in the Western Cape, South Africa
    (BioMed Central Ltd, 2011) Weakley, Kate; Esser, Monika; Pope, Chris; Scott, Christiaan
    Background: JIA is a disease that shows wide variations between differing populations. Due to recent international consensus on classification criteria, JIA has been widely described in many countries and population groups. There has been almost no data that describes JIA in an African, specifically Sub-Saharan African setting. Objective: To describe disease characteristics and functional disability in two tertiary centres in the Western Cape, South Africa
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    Juvenile idiopathic arthritis in two tertiary centres in the Western Cape, South Africa
    (BioMed Central Ltd, 2012) Weakley, Kate; Esser, Monika; Scott, Christiaan
    BACKGROUND:Juvenile idiopathic arthritis (JIA) is a disease that shows wide variations between differing populations. Since the recent international consensus on classification criteria, JIA has been widely described in many countries and population groups. There has been almost no data that describes JIA in an African, specifically Sub-Saharan African, setting. Therefore, the aim of this study is to describe disease characteristics, disease course, and functional disability in two tertiary centres in the Western Cape, South Africa and compare the findings to other JIA populations. METHODS: Eighty-six children were recruited during random clinic visits to rheumatology clinics at Tygerberg and Groote Schuur Hospital between April 2010 and April 2011. Children were diagnosed using International League of Associations for Rheumatology (ILAR) 2001 classification criteria. Consent was obtained and medical records examined. The Childhood Health Assessment Questionnaires (CHAQ) and visual analogue scales (VAS) for pain and general well-being were completed and all children were examined by a researcher in conjunction with a paediatric rheumatologist. HIV status as well as tuberculosis disease and treatment were investigated. RESULTS: A total of 86 children were enrolled. Eight children were excluded (2 HIV arthropathy, 1 TB arthritis, 1 SLE, 4 with insufficient data), leaving a total of 78 patients. There was an equal female to male ratio-39 males and 39 females. There were 6 systemic JIA patients (7.69%), 17 persistent oligoarthritis (21.79%), 4 extended oligoarthritis (5.12%), 11 polyarthritis rheumatoid factor (RF) positive (14.10%), 21 polyarthritis RF negative (26.9%), 1 psoriatic arthritis (1.28%), and 18 enthesitis-related arthritis (23%). The median CHAQ for the group was 0.5 (IQR 0.1-1.25), the median VAS for pain was 18 mm (IQR 4-42) and median VAS for general well-being was 25 mm (IQR 3-49). Enthesitis-related arthritis and polyarthritis disease subtypes in this South African population may be more common than seen in JIA populations described in northern Europe, India, United Kingdom, and Turkey. CONCLUSION: This Western Cape South African JIA population appears to have a different profile of JIA than what has been described elsewhere. Enthesitis-related arthritis and polyarthritis disease subtypes appear to be more prevalent. There are also significant challenges in this setting such as later presentation to pediatric rheumatologists, different disease characteristics, and variable disease courses.
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    Medium term health and quality of life outcomes in a cohort of children with MIS-C in Cape Town, South Africa
    (2025) Phoya, Frank; Webb, Kate; Scott, Christiaan
    Background: Multisystem inflammatory syndrome in children (MIS-C) is a disease that occurs after exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Its short term effects have been documented but little data exist on the longer term effects of MIS-C on the health and quality of life of patients. The objective of this study was to assess the long-term effects of MIS-C on the quality of life of children. Methods: This study was a prospective, case control, cohort study. We included 24 participants with previous MIS-C and 20 children with juvenile idiopathic arthritis (JIA) as a positive comparator group. All children were examined and completed a paediatric quality of life (PedsQL) generic inventory score. This score was used to evaluate the school functioning, social, emotional, and physical domains of the two groups. Results: All participants with previous MIS-C made a full recovery with normal physical examination after a median of 705 days after acute MIS-C. The PedsQL inventory revealed that 16.7% of the children with previous MIS-C showed a deficit in the physical domain compared to 60% of the children with JIA (p<0.001). There was a deficit in the psychosocial domain (which combines emotional, social and educational scores) in 12.5% children with previous MIS-C compared to 40% children with JIA (p=0.035) Conclusions: In 24 children with previous MIS-C, after approximately 2 years, no medical complications were reported. A small proportion felt a prolonged effect on their quality of life even after making a full recovery, which was less severe than in children with JIA. This highlights the need to continue to follow up these patients and offer more comprehensive long-term care.
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    Missed opportunities for timely diagnosis of pediatric lupus in South Africa: a qualitative study
    (BioMed Central, 2017-02-23) Lewandowski, Laura B; Watt, Melissa H; Schanberg, Laura E; Thielman, Nathan M; Scott, Christiaan
    Background: Systemic Lupus Erythematosus (SLE) is a serious multisystem autoimmune disease, which is more aggressive in children and people of African descent. In South Africa, pediatric SLE (pSLE) patients are at high risk for severe disease. Similar to pSLE worldwide, South African children and adolescents with SLE require subspecialized medical care. The aim of this study is to describe the care-seeking experiences of families and examine factors that contribute to delays in the diagnosis of pSLE. Specifically, we sought to identify factors to inform interventions that support the timely referral and diagnosis of pediatric SLE patients in South Africa. Methods: In-depth, semi-structured interviews were conducted with 22 caregivers of pSLE patients recruited from two government hospitals in Cape Town, South Africa in 2014. Interviews were audio-recorded, transcribed, and analyzed for themes related to barriers to diagnosis. Results: Six themes were identified and classified as either caregiver or health system barriers to diagnosis. Caregiver barriers included lack of knowledge regarding SLE, financial difficulties, and the social stigma of SLE. Health system barriers were lack of trained staff, a complex medical system, and misdiagnosis. Conclusion: Caregivers reported missed opportunities for diagnosing pSLE in their children. Raising public awareness may improve caregiver awareness and reduce stigma of pSLE. Improving family education at diagnosis holds potential to increase patient-physician trust and mitigate fear. Education modules for primary care providers at initial point of contact with the health care system may improve recognition of early pSLE and facilitate expedited referral to a specialist.
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    MyastheniaGravis(MG) in a patient with Juvenile Idiopathic Arthritis
    (BioMed Central Ltd, 2011) Scott, Christiaan; Kalla, Asgar
    Introduction; Myasthenia Gravis associated with Juvenile Idiopathic Arthritis has been reported in 5children with various subtypes of JIA [1,2]. Methods: We present a 17year old girl known with Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis for 4 years who developed Myasthenia Gravis while on therapy with Methotrexate, Prednisone and Ibuprofen. Results: This patient presented to the emergency room with a respiratory infection. She had been feeling weak and had noticed tongue weakness and difficulty swallowing, which had worsened significantly since the respiratory infection. On examination she was found to have clinical signs of Right Middle Lobe pneumonia and was found to be weak, especially in her proximal muscle groups. She had bilateral ptosis as well as facial weakness. She had active arthritis in multiple joints. Despite intravenous antibiotics and full supportive management she deteriorated rapidly, and within 12 hours required intubation and ventilation. The patient was found to have high ACH receptor antibodies and responded dramatically to pyridostygmine therapy, confirming the diagnosis of MG. High prednisone and azathioprine have been added to her regime. Discussion: Myasthenia Gravis is a rare association with JIA. The majority of cases appear to be associated with oligo-articular JIA. This patient presented after an acute infection and a recent worsening in her JIA symptoms.
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    Paediatric non-infectious uveitis in Cape Town, South Africa: a retrospective review of disease characteristics and outcomes on immunomodulating treatment
    (2021-04-01) Slamang, Waheba; Tinley, Christopher; Brice, Nicola; Scott, Christiaan
    Background Non-infectious uveitis is a well-reported cause of blindness in more developed countries, however data from sub-Saharan Africa is lacking. Here we aim to describe the diseases associated with paediatric non-infectious uveitis and the effect 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: median age at first visit of 74 months (IQR 49–86 months), female to male ratio of 0.9:1, predominantly of mixed ancestry (72.4%). Juvenile idiopathic arthritis associated uveitis (JIAU) (48.3%), idiopathic uveitis (41.4%), sarcoidosis (6.9%) and Behcet’s disease (3.5%) were diagnosed. Chronic anterior uveitis (72.4%) was the most frequent finding. Fifty-five percent had complications at presentation and all children with idiopathic uveitis presented with cataracts. Only 6.5% of the JIA cohort had JIAU. All JIA children had chronic anterior uveitis. There were no differences between JIA children with uveitis and those without uveitis, for sex (p = 0.68) and race (p = 0.58). Significantly, children with uveitis presented at an overall younger age (p = 0.008), had oligo-articular JIA (p = 0.01) and were antinuclear antibody positive (p < 0.001). Children with idiopathic uveitis were predominantly male (66.6%) with chronic anterior uveitis (41.7%). Nineteen children (65.5%) in the cohort had inactive disease on treatment at 12 months from diagnosis, which included 10 on topical corticosteroid therapy. At the last clinical visit 17 (58.6%) on standard initial therapy, 8 (27.6%) on tumour necrosis factor inhibitors and 2 on additional DMARDs were in remission. Five of these children still required topical corticosteroids. Surgery was performed in 41.4%, primarily in the idiopathic group. Visual acuity improved or was maintained on treatment. Conclusion Current practice seems to detect children with potentially sight-threatening disease but the high rate of complications and the low percentage of children with JIAU raises concerns of delayed healthcare intervention. Tumour necrosis factor inhibitors have improved outcomes in refractory cases in this cohort, however further studies are needed.
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    Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations
    (2019-11-26) Harrison, Michael J; Zühlke, Liesl J; Lewandowski, Laura B; Scott, Christiaan
    Abstract Background Pediatric onset of systemic lupus erythematosus (SLE) is associated with major organ involvement, and African patients tend to develop more aggressive disease than patients of European descent. Although cardiovascular involvement is common in pediatric SLE, there are few published reports on the subject. This study describes the frequency and characteristics of cardiac and vascular manifestations of pediatric SLE in a multi-ethnic South African cohort. Methods Demographic, clinical, and echocardiographic data were collected from pediatric SLE patients at two centers in Cape Town, South Africa. At the time of investigation, this cohort consisted of 93 participants diagnosed with SLE according to international classification criteria prior to the age of 19. Individuals with cardiac and/or vascular involvement were identified by retrospective chart review. Cardiac manifestations were defined as presence of pericardial effusion, myocarditis, cardiomyopathy, cardiac failure, Libman-Sacks endocarditis, myocardial infarction, and arrhythmia. Vascular manifestations included deep vein thrombosis, pulmonary embolism, sinus thrombosis, stroke, critical limb ischemia, cerebral vasculitis and systemic vasculitis. Statistical analysis was performed using R (v3.4.1). Results Cardiac and vascular involvement was present in 47% of the cohort. Previous studies have reported prevalence of 5%—50%. Demographic features of those with cardiac/vascular involvement did not differ from the overall cohort. Echocardiographic data were available for 23 participants. The most common cardiac manifestations were pericardial effusion (n = 24) and cardiac failure (n = 8), while the most common vascular manifestations were cerebral vasculitis (n = 9), stroke (n = 7), and pulmonary embolism (n = 7). Cardiovascular manifestations were frequently severe; one third of pericardial effusion cases required intervention, including three cases of cardiac tamponade. Cardiac and vascular involvement conferred an increased risk of mortality (31.1% versus 10.4%). Conclusions Cardiac and vascular involvement were highly prevalent in this South African cohort. The mortality rate was high, and severe manifestations were frequent. Prospective research is needed to improve knowledge of pediatric SLE in Africa and to improve outcomes for this high-risk population.
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    Revising the WHO Essential Medicines List for paediatric rheumatology
    (2021-01-23) Scott, Christiaan; Smith, Nicola; James, Rebecca; Whitehead, Ben; Green, Rochelle; Foster, Helen E
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    Serious adverse drug reactions at two children’s hospitals in South Africa
    (2020-01-04) Mouton, Johannes P; Fortuin-de Smidt, Melony C; Jobanputra, Nicole; Mehta, Ushma; Stewart, Annemie; de Waal, Reneé; Technau, Karl-Günter; Argent, Andrew; Kroon, Max; Scott, Christiaan; Cohen, Karen
    Abstract Background The high HIV prevalence in South Africa may potentially be shaping the local adverse drug reaction (ADR) burden. We aimed to describe the prevalence and characteristics of serious ADRs at admission, and during admission, to two South African children’s hospitals. Methods We reviewed the folders of children admitted over sequential 30-day periods in 2015 to the medical wards and intensive care units of each hospital. We identified potential ADRs using a trigger tool developed for this study. A multidisciplinary team assessed ADR causality, type, seriousness, and preventability through consensus discussion. We used multivariate logistic regression to explore associations with serious ADRs. Results Among 1050 patients (median age 11 months, 56% male, 2.8% HIV-infected) with 1106 admissions we found 40 serious ADRs (3.8 per 100 drug-exposed admissions), including 9/40 (23%) preventable serious ADRs, and 8/40 (20%) fatal or near-fatal serious ADRs. Antibacterials, corticosteroids, psycholeptics, immunosuppressants, and antivirals were the most commonly implicated drug classes. Preterm neonates and children in middle childhood (6 to 11 years) were at increased risk of serious ADRs compared to infants (under 1 year) and term neonates: adjusted odds ratio (aOR) 5.97 (95% confidence interval 1.30 to 27.3) and aOR 3.63 (1.24 to 10.6) respectively. Other risk factors for serious ADRs were HIV infection (aOR 3.87 (1.14 to 13.2) versus HIV-negative) and increasing drug count (aOR 1.08 (1.04 to 1.12) per additional drug). Conclusions Serious ADR prevalence in our survey was similar to the prevalence found elsewhere. In our setting, serious ADRs were associated with HIV-infection and the antiviral drug class was one of the most commonly implicated. Similar to other sub-Saharan African studies, a large proportion of serious ADRs were fatal or near-fatal. Many serious ADRs were preventable.
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