Background: Children with sickle cell anemia (SCA) in Sub-Saharan Africa are at high risk of sick... more Background: Children with sickle cell anemia (SCA) in Sub-Saharan Africa are at high risk of sickle cerebrovascular injury (SCVI). Hydroxyurea, a commonly used disease-modifying therapy, may prevent or decrease SCVI for reduced incident stroke, stroke risk and potentially cognitive dysfunction. We aim to test the impact of daily hydroxyurea therapy on these outcomes in Ugandan children with SCA. We hypothesize that hydroxyurea therapy over 36 months will prevent, stabilize or improve these complications of SCA. Methods: The BRAIN SAFE II study is an open-label, single-arm trial of daily hydroxyurea for 270 children with SCA (HbSS) in Uganda, ages 3-9 years. Following baseline assessments, participants began hydroxyurea therapy and clinically followed per local guidelines. Standard hydroxyurea dose is escalated to maximum tolerated dose (MTD). SCVI is assessed by cerebral arterial velocity using Doppler ultrasound, with cognitive function determined by formal neurocognitive testing (primary outcomes). Structural SCVI is assessed by magnetic resonance imaging (MRI) and angiography (MRA) in a sub-sample of 90 participants ages >5 years, along with biomarkers of anemia, inflammation and malnutrition (secondary outcomes). At trial midpoint (18 months) and completion (36 months), primary outcomes will be compared to participants' baseline to determine hydroxyurea impact and relationships to secondary outcomes. This open-label, single-arm trial will examine the impact of hydroxyurea on preventing or ameliorating SCA SCVI in children, assessed by reducing incident stroke, stroke risk and neurocognitive dysfunction. Trial results will provide important insight into the role of hydroxyurea therapy on critical manifestations of SCVI in children with SCA.
In their study of the burden, risk factors, and magnitude of neurological involvement in acute fa... more In their study of the burden, risk factors, and magnitude of neurological involvement in acute falciparum malaria in Kenyan children, Dr Idro and colleagues 1 did not consider the use of salicylates in their patient histories. Salicylates are widely available in many African countries and are commonly used for initial management of symptoms that malaria shares with other diseases. 2 Since salicylate toxicity in African children has been associated with Reye syndrome and has been noted to have many clinical features in common with cerebral falciparum malaria (acidosis, coma, seizure, and hypoglycemia), 3 assessing the salicylate plasma concentrations in all children with suspected cerebral malaria and a history of salicylate use is needed to avoid a misdiagnosis.
medRxiv (Cold Spring Harbor Laboratory), Jul 1, 2023
Background: Most of neonatal deaths occur among low birth weight infants. However, in resource-li... more Background: Most of neonatal deaths occur among low birth weight infants. However, in resource-limited settings, these infants are commonly discharged early which further exposes them to mortality. This study aimed at determining the proportion and predictors of mortality among low birth weight neonates that are discharged from Mulago National Referral Hospital in Uganda. Methods: This was a prospective cohort study of 220 low birth weight neonates discharged from a Special Care Baby Unit at Mulago National Referral Hospital. These were followed up to 28 completed days of life, or death, whichever occurred first. Proportions were used to express mortality. To determine the predictors of mortality, Cox hazards regression was performed. .
Community Acquired Bacterial Meningitis (CABM) is a leading cause of morbidity and mortality in A... more Community Acquired Bacterial Meningitis (CABM) is a leading cause of morbidity and mortality in Africa, affecting over 2.8 million people annually. The African continent bears the largest burden of CABM with six out of the ten countries having the highest incidence and mortality. This systematic review set out to determine the leading causes of CABM in Africa, the distribution of etiologies across regions, and the extent of antimicrobial resistance of identified microbial agents. The databases PubMed and Embase were queried with key search words "bacterial meningitis in Africa", "community acquired bacterial meningitis" or "meningitis in Africa" published between 1990 and 2019. Using the PRISMA guidelines for systematic reviews, the search retrieved 112,972 articles. We included studies reporting primary patient data with confirmed CABM. Results: Sixty-four studies were analyzed which were conducted in 23 African countries between 1990 and 2019. Cumulatively, we analyzed 118,716 suspected cases of CABM, with confirmed CABM in 34,593 (29%) cases. Streptococcal pneumoniae (Spn) was found to be the most prevalent cause of disease at 12.4% (CI 11.1%-13.6%), Neisseria meningitidis (Nmn) at 8.1% (CI 7.1%-9.2%) and Hemophilus influenza (Hib) at 4.0%(CI 3.6%-4.3)%. The etiologies varied by region, with Nmn being most prevalent in West and South Africa regions and Spn in the rest of the continent. Salmonella prevalence was 0.35%(0.22%-0.47%) representing 16% of the total cases in East Africa but less than 1% in other regions. Of the 64 studies reviewed, 29 (45%) reported on antimicrobial resistance. Resistance was found to be highest in ampicillin 2.7% (CI 2.1%-3.3%) and gentamycin 2.75%(2.09-3.40). We found a gross paucity of information on CABM in Africa despite baring the highest burden of disease. Spn and Nmn are still the leading causes of disease, and etiology varies markedly with region. Antimicrobial resistance of ampicillin and gentamycin are widespread despite being the commonest drugs used in the treatment of CABM in Africa.
Children recovering from severe malarial anaemia (SMA) remain at high risk of readmission and dea... more Children recovering from severe malarial anaemia (SMA) remain at high risk of readmission and death after discharge from hospital. However, a recent trial found that post-discharge malaria chemoprevention (PDMC) with dihydroartemisinin-piperaquine reduces this risk. We developed a mathematical model describing the daily incidence of uncomplicated and severe malaria requiring readmission among 0-5-year old children after hospitalised SMA. We fitted the model to a multicentre clinical PDMC trial using Bayesian methods and modelled the potential impact of PDMC across malaria-endemic African countries. In the 20 highest-burden countries, we estimate that only 2-5 children need to be given PDMC to prevent one hospitalised malaria episode, and less than 100 to prevent one death. If all hospitalised SMA cases access PDMC in moderate-to-high transmission areas, 38,600 (range 16,900-88,400) malaria-associated readmissions could be prevented annually, depending on access to hospital care. We estimate that recurrent SMA post-discharge constitutes 19% of all SMA episodes in moderate-to-high transmission settings. Severe malarial anaemia (SMA) still contributes substantially to childhood mortality and morbidity in malaria-endemic countries in Africa. P. falciparum malaria causes anaemia by triggering severe haemolysis of erythrocytes and suppression of erythropoiesis 1. In highly endemic areas, around one-third of all hospitalised children may be severely anaemic (Hb<5 g/dL) 2,3 and severe anaemia may contribute to around half of all malaria-attributed deaths 4. While malaria transmission has declined in many countries in the past two decades, highly endemic conditions persist in parts of sub-Saharan Africa. 70% of global malaria deaths occurred in just ten countries in Africa in 2020 5. These countries are now the focus of the WHO High Burden to High Impact (HBHI) initiative, aimed at accelerating progress in malaria control in the hardest-hit areas.
Background: Benign familial neonatal epilepsy (BFNE), a form of autosomal dominant epilepsy of in... more Background: Benign familial neonatal epilepsy (BFNE), a form of autosomal dominant epilepsy of infancy, is caused by gene mutations in the KCNQ2 or KCNQ3 which code the voltage dependent potassium channels K v 7.2 or K v 7.3 expressing in neurons. Objective: We analyzed KCNQ2 and KCNQ3 gene in two Japanese families with BFNE, and studied the mutant KCNQ2/KCNQ3 potassium channel property to elucidate the mechanism of BFNE. Patients and methods: We investigated KCNQ2 and KCNQ3 gene sequence of two families with BFNE. Whole-cell potassium currents were recorded from HEK293 cells transfected with the plasmid vector encoding either wild type or mutant KCNQ2/KCNQ3 gene. Current-voltage relationships were determined by using test pulses between −120 mV and 40 mV from the holding potential of −100 mV. Results: The sequence analysis of KCNQ2 and KCNQ3 gene identified R581X mutation in KCNQ2 and S337Y mutation in KCNQ3. Wild type KCNQ2 and KCNQ3 channels showed normal potassium currents, however, these mutant channels showed no significant potassium currents. Conclusion: We identified the novel mutations R581X of KCNQ2 and S337Y of KCNQ3 gene in Japanese families with BFNE. These mutations cause a deficiency of the potassium conductance and impair the repolarization of neurons. Thus it may cause abnormal prolongation of the neuronal excitation, which finally induces epilepsy.
Background: Studies on health related quality of life (HRQOL) of children with disabilities in lo... more Background: Studies on health related quality of life (HRQOL) of children with disabilities in low income countries are limited. Objective: To inform interventions for children with spina bifida in low income countries, HRQOL of children with spina bifida and siblings, predictors, relationships between HRQOL and parental stress in Uganda were examined. Methods: Demographic, impairment, daily, social functioning data, and HRQOL using the KIDSCREEN-10 were collected from 39 children, 33 siblings, and 39 parents from a cohort of families of children with spina bifida. T-tests, correlations, analysis of variance and regression analysis were used to compare means between children with spina bifida and their siblings, understand relationships between variables, and identify predictors of HRQOL. Results: Children with spina bifida (N ¼ 39) had lower HRQOL compared to their siblings (N ¼ 33) (t ¼ À3.868, p < .001 parental; t ¼ À3.248, p ¼ .002 child ratings). Parents (N ¼ 39) indicated higher parental stress for their child with spina bifida (t ¼ 2.143, p ¼ 0.036). HRQOL child outcomes were predicted by the presence of hydrocephalus (b ¼ À.295, p ¼ 0.013) for children with spina bifida, and daily functioning levels (b ¼ .336, p ¼ 0.038), and parental support (b ¼ .357, p ¼ 0.041) for siblings specifically. Parent rated HRQOL outcomes were predicted by parental distress (b ¼ À.337, p ¼ 0.008), incontinence (b ¼ .423, p ¼ 0.002), and daily functioning levels (b ¼ .325, p ¼ 0.016) for children with spina bifida. Conclusions: To improve HRQOL investment in neurosurgical care, community based rehabilitation, incontinence management, and parental support are required. A combination of child friendly semistructured and creative research methods are recommended to study HRQOL.
A195 d'origine occidentale où ces croyances ne sont pas acceptées. Dans d'autres pays, la stigmat... more A195 d'origine occidentale où ces croyances ne sont pas acceptées. Dans d'autres pays, la stigmatisation des troubles psychiatriques seront un frein pour l'utilisation des échelles sous forme de questionnaire ou d'interview. La construction ou l'utilisation d'outils de dépistage ou de diagnostic des troubles du comportement en zones tropicales doivent se baser sur la connaissance de la maladie dans l'environnement social, culturel et traditionnel du patient. L'influence de ces facteurs et la perception de ces troubles par la population doivent être intégrées lors de la mise en place de ces outils le plus souvent validés dans les pays occidentaux. En absence de respect de ces conditions d'utilisation, ces outils ne sont pas adaptés aux zones tropicales.
The World Health Organization estimates that the proportionate share of the total global burden o... more The World Health Organization estimates that the proportionate share of the total global burden of disease resulting from neurologic and psychiatric disorders will rise to 14.7 % by 2020. Although these disorders comprise only 1.4 % of all deaths, they account for a remarkable 28 % of all years of life lived with a disability. In Africa, brain injury acquired during childhood underlies the majority of the neurologic disorders. There are however very few comprehensive studies of these on the continent. Available studies are mostly of specific etiologies and often conducted in localized areas. This chapter provides an overview of traumatic and acquired non-traumatic brain injury in children in sub-Saharan Africa. It highlights the burden, causes, manifestations and management challenges and provides some perspectives for the future. The chapter will deal with only acquired brain injury in children after birth in sub-Saharan Africa. Genetic disorders and disorders due to early adverse foetal exposures are not included here.
Background Children with cerebral palsy (CP) frequently experience chronic pain. The burden and s... more Background Children with cerebral palsy (CP) frequently experience chronic pain. The burden and severity of such pain is often underestimated in relation to their other impairments. Recognition and awareness of this chronic pain among children with CP constitute the cornerstone for caretakers and clinicians to improve the quality of life of those children. This study aimed to determine the prevalence of chronic pain among children with CP, and the factors associated. Methods A cross-sectional study of children with CP, aged 2–12 years, attending the CP rehabilitation clinic and Pediatric Neurology Clinic at Mulago Hospital, Uganda from November 2017 to May 2018. A detailed history and clinical examination were performed and the co-morbidities were determined. CP was classified using the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System, Communication Function Classification System (CFCS), and the Eating and Drinking Ability Classification Syste...
Journal of the International Child Neurology Association, 2019
Emerging genetic therapies for rare disorders at high cost, cannot realistically address the glob... more Emerging genetic therapies for rare disorders at high cost, cannot realistically address the global burden of disease. Stakeholders must develop new pathways to ensure safe, fair and sustainable provision of such therapies.
Introduction: Tetralogy of Fallot is one of the conotruncal abnormalities and is the most common ... more Introduction: Tetralogy of Fallot is one of the conotruncal abnormalities and is the most common cyanotic heart disease worldwide. Conotruncal abnormalities have been closely associated with 22.q.11 deletion syndrome. Children with 22.q.11 deletion syndrome often have thymic hypoplasia, hypocalcaemia and have an increased susceptibility to viral, fungal and bacterial infections due to poor T-cell production. Autoimmune vasculitis has not been well documented among individuals with Tetralogy of Fallot (TOF) though a few reports exist in association with 22.q11.2 deletion syndrome. Case summary: We report a case of an 11year old child post TOF repair who presented to the Uganda Heart Institute with persistent high grade fevers, abdominal pain, mucosal bleeds and hypocalcaemia, hypokalemia, hypomagnesaemia, thrombocytopenia and elevated acute phase reactants-leukocytosis (17.9x103), neutrophil count 16x103(89%), elevated C-reactive protein (80mg/l) and elevated immunological tests (C-ANCA, 18U/ml). She improved on intravenous methyl prednisolone. Conclusion: Children with Tetralogy of Fallot can still manifest with complications such as autoimmune disorders and severe infections given the association with 22q.11 deletion syndrome. Screening for autoimmune abnormalities and electrolytes especially calcium improves on their outcome. We were not able to perform the FISH analysis (for Fluorescent in Situ Hybridization) to confirm the diagnosis of 22q.11.2 deletion syndrome in KT.
Background: Children with sickle cell anemia (SCA) in Sub-Saharan Africa are at high risk of sick... more Background: Children with sickle cell anemia (SCA) in Sub-Saharan Africa are at high risk of sickle cerebrovascular injury (SCVI). Hydroxyurea, a commonly used disease-modifying therapy, may prevent or decrease SCVI for reduced incident stroke, stroke risk and potentially cognitive dysfunction. We aim to test the impact of daily hydroxyurea therapy on these outcomes in Ugandan children with SCA. We hypothesize that hydroxyurea therapy over 36 months will prevent, stabilize or improve these complications of SCA. Methods: The BRAIN SAFE II study is an open-label, single-arm trial of daily hydroxyurea for 270 children with SCA (HbSS) in Uganda, ages 3-9 years. Following baseline assessments, participants began hydroxyurea therapy and clinically followed per local guidelines. Standard hydroxyurea dose is escalated to maximum tolerated dose (MTD). SCVI is assessed by cerebral arterial velocity using Doppler ultrasound, with cognitive function determined by formal neurocognitive testing (primary outcomes). Structural SCVI is assessed by magnetic resonance imaging (MRI) and angiography (MRA) in a sub-sample of 90 participants ages >5 years, along with biomarkers of anemia, inflammation and malnutrition (secondary outcomes). At trial midpoint (18 months) and completion (36 months), primary outcomes will be compared to participants' baseline to determine hydroxyurea impact and relationships to secondary outcomes. This open-label, single-arm trial will examine the impact of hydroxyurea on preventing or ameliorating SCA SCVI in children, assessed by reducing incident stroke, stroke risk and neurocognitive dysfunction. Trial results will provide important insight into the role of hydroxyurea therapy on critical manifestations of SCVI in children with SCA.
In their study of the burden, risk factors, and magnitude of neurological involvement in acute fa... more In their study of the burden, risk factors, and magnitude of neurological involvement in acute falciparum malaria in Kenyan children, Dr Idro and colleagues 1 did not consider the use of salicylates in their patient histories. Salicylates are widely available in many African countries and are commonly used for initial management of symptoms that malaria shares with other diseases. 2 Since salicylate toxicity in African children has been associated with Reye syndrome and has been noted to have many clinical features in common with cerebral falciparum malaria (acidosis, coma, seizure, and hypoglycemia), 3 assessing the salicylate plasma concentrations in all children with suspected cerebral malaria and a history of salicylate use is needed to avoid a misdiagnosis.
medRxiv (Cold Spring Harbor Laboratory), Jul 1, 2023
Background: Most of neonatal deaths occur among low birth weight infants. However, in resource-li... more Background: Most of neonatal deaths occur among low birth weight infants. However, in resource-limited settings, these infants are commonly discharged early which further exposes them to mortality. This study aimed at determining the proportion and predictors of mortality among low birth weight neonates that are discharged from Mulago National Referral Hospital in Uganda. Methods: This was a prospective cohort study of 220 low birth weight neonates discharged from a Special Care Baby Unit at Mulago National Referral Hospital. These were followed up to 28 completed days of life, or death, whichever occurred first. Proportions were used to express mortality. To determine the predictors of mortality, Cox hazards regression was performed. .
Community Acquired Bacterial Meningitis (CABM) is a leading cause of morbidity and mortality in A... more Community Acquired Bacterial Meningitis (CABM) is a leading cause of morbidity and mortality in Africa, affecting over 2.8 million people annually. The African continent bears the largest burden of CABM with six out of the ten countries having the highest incidence and mortality. This systematic review set out to determine the leading causes of CABM in Africa, the distribution of etiologies across regions, and the extent of antimicrobial resistance of identified microbial agents. The databases PubMed and Embase were queried with key search words "bacterial meningitis in Africa", "community acquired bacterial meningitis" or "meningitis in Africa" published between 1990 and 2019. Using the PRISMA guidelines for systematic reviews, the search retrieved 112,972 articles. We included studies reporting primary patient data with confirmed CABM. Results: Sixty-four studies were analyzed which were conducted in 23 African countries between 1990 and 2019. Cumulatively, we analyzed 118,716 suspected cases of CABM, with confirmed CABM in 34,593 (29%) cases. Streptococcal pneumoniae (Spn) was found to be the most prevalent cause of disease at 12.4% (CI 11.1%-13.6%), Neisseria meningitidis (Nmn) at 8.1% (CI 7.1%-9.2%) and Hemophilus influenza (Hib) at 4.0%(CI 3.6%-4.3)%. The etiologies varied by region, with Nmn being most prevalent in West and South Africa regions and Spn in the rest of the continent. Salmonella prevalence was 0.35%(0.22%-0.47%) representing 16% of the total cases in East Africa but less than 1% in other regions. Of the 64 studies reviewed, 29 (45%) reported on antimicrobial resistance. Resistance was found to be highest in ampicillin 2.7% (CI 2.1%-3.3%) and gentamycin 2.75%(2.09-3.40). We found a gross paucity of information on CABM in Africa despite baring the highest burden of disease. Spn and Nmn are still the leading causes of disease, and etiology varies markedly with region. Antimicrobial resistance of ampicillin and gentamycin are widespread despite being the commonest drugs used in the treatment of CABM in Africa.
Children recovering from severe malarial anaemia (SMA) remain at high risk of readmission and dea... more Children recovering from severe malarial anaemia (SMA) remain at high risk of readmission and death after discharge from hospital. However, a recent trial found that post-discharge malaria chemoprevention (PDMC) with dihydroartemisinin-piperaquine reduces this risk. We developed a mathematical model describing the daily incidence of uncomplicated and severe malaria requiring readmission among 0-5-year old children after hospitalised SMA. We fitted the model to a multicentre clinical PDMC trial using Bayesian methods and modelled the potential impact of PDMC across malaria-endemic African countries. In the 20 highest-burden countries, we estimate that only 2-5 children need to be given PDMC to prevent one hospitalised malaria episode, and less than 100 to prevent one death. If all hospitalised SMA cases access PDMC in moderate-to-high transmission areas, 38,600 (range 16,900-88,400) malaria-associated readmissions could be prevented annually, depending on access to hospital care. We estimate that recurrent SMA post-discharge constitutes 19% of all SMA episodes in moderate-to-high transmission settings. Severe malarial anaemia (SMA) still contributes substantially to childhood mortality and morbidity in malaria-endemic countries in Africa. P. falciparum malaria causes anaemia by triggering severe haemolysis of erythrocytes and suppression of erythropoiesis 1. In highly endemic areas, around one-third of all hospitalised children may be severely anaemic (Hb<5 g/dL) 2,3 and severe anaemia may contribute to around half of all malaria-attributed deaths 4. While malaria transmission has declined in many countries in the past two decades, highly endemic conditions persist in parts of sub-Saharan Africa. 70% of global malaria deaths occurred in just ten countries in Africa in 2020 5. These countries are now the focus of the WHO High Burden to High Impact (HBHI) initiative, aimed at accelerating progress in malaria control in the hardest-hit areas.
Background: Benign familial neonatal epilepsy (BFNE), a form of autosomal dominant epilepsy of in... more Background: Benign familial neonatal epilepsy (BFNE), a form of autosomal dominant epilepsy of infancy, is caused by gene mutations in the KCNQ2 or KCNQ3 which code the voltage dependent potassium channels K v 7.2 or K v 7.3 expressing in neurons. Objective: We analyzed KCNQ2 and KCNQ3 gene in two Japanese families with BFNE, and studied the mutant KCNQ2/KCNQ3 potassium channel property to elucidate the mechanism of BFNE. Patients and methods: We investigated KCNQ2 and KCNQ3 gene sequence of two families with BFNE. Whole-cell potassium currents were recorded from HEK293 cells transfected with the plasmid vector encoding either wild type or mutant KCNQ2/KCNQ3 gene. Current-voltage relationships were determined by using test pulses between −120 mV and 40 mV from the holding potential of −100 mV. Results: The sequence analysis of KCNQ2 and KCNQ3 gene identified R581X mutation in KCNQ2 and S337Y mutation in KCNQ3. Wild type KCNQ2 and KCNQ3 channels showed normal potassium currents, however, these mutant channels showed no significant potassium currents. Conclusion: We identified the novel mutations R581X of KCNQ2 and S337Y of KCNQ3 gene in Japanese families with BFNE. These mutations cause a deficiency of the potassium conductance and impair the repolarization of neurons. Thus it may cause abnormal prolongation of the neuronal excitation, which finally induces epilepsy.
Background: Studies on health related quality of life (HRQOL) of children with disabilities in lo... more Background: Studies on health related quality of life (HRQOL) of children with disabilities in low income countries are limited. Objective: To inform interventions for children with spina bifida in low income countries, HRQOL of children with spina bifida and siblings, predictors, relationships between HRQOL and parental stress in Uganda were examined. Methods: Demographic, impairment, daily, social functioning data, and HRQOL using the KIDSCREEN-10 were collected from 39 children, 33 siblings, and 39 parents from a cohort of families of children with spina bifida. T-tests, correlations, analysis of variance and regression analysis were used to compare means between children with spina bifida and their siblings, understand relationships between variables, and identify predictors of HRQOL. Results: Children with spina bifida (N ¼ 39) had lower HRQOL compared to their siblings (N ¼ 33) (t ¼ À3.868, p < .001 parental; t ¼ À3.248, p ¼ .002 child ratings). Parents (N ¼ 39) indicated higher parental stress for their child with spina bifida (t ¼ 2.143, p ¼ 0.036). HRQOL child outcomes were predicted by the presence of hydrocephalus (b ¼ À.295, p ¼ 0.013) for children with spina bifida, and daily functioning levels (b ¼ .336, p ¼ 0.038), and parental support (b ¼ .357, p ¼ 0.041) for siblings specifically. Parent rated HRQOL outcomes were predicted by parental distress (b ¼ À.337, p ¼ 0.008), incontinence (b ¼ .423, p ¼ 0.002), and daily functioning levels (b ¼ .325, p ¼ 0.016) for children with spina bifida. Conclusions: To improve HRQOL investment in neurosurgical care, community based rehabilitation, incontinence management, and parental support are required. A combination of child friendly semistructured and creative research methods are recommended to study HRQOL.
A195 d'origine occidentale où ces croyances ne sont pas acceptées. Dans d'autres pays, la stigmat... more A195 d'origine occidentale où ces croyances ne sont pas acceptées. Dans d'autres pays, la stigmatisation des troubles psychiatriques seront un frein pour l'utilisation des échelles sous forme de questionnaire ou d'interview. La construction ou l'utilisation d'outils de dépistage ou de diagnostic des troubles du comportement en zones tropicales doivent se baser sur la connaissance de la maladie dans l'environnement social, culturel et traditionnel du patient. L'influence de ces facteurs et la perception de ces troubles par la population doivent être intégrées lors de la mise en place de ces outils le plus souvent validés dans les pays occidentaux. En absence de respect de ces conditions d'utilisation, ces outils ne sont pas adaptés aux zones tropicales.
The World Health Organization estimates that the proportionate share of the total global burden o... more The World Health Organization estimates that the proportionate share of the total global burden of disease resulting from neurologic and psychiatric disorders will rise to 14.7 % by 2020. Although these disorders comprise only 1.4 % of all deaths, they account for a remarkable 28 % of all years of life lived with a disability. In Africa, brain injury acquired during childhood underlies the majority of the neurologic disorders. There are however very few comprehensive studies of these on the continent. Available studies are mostly of specific etiologies and often conducted in localized areas. This chapter provides an overview of traumatic and acquired non-traumatic brain injury in children in sub-Saharan Africa. It highlights the burden, causes, manifestations and management challenges and provides some perspectives for the future. The chapter will deal with only acquired brain injury in children after birth in sub-Saharan Africa. Genetic disorders and disorders due to early adverse foetal exposures are not included here.
Background Children with cerebral palsy (CP) frequently experience chronic pain. The burden and s... more Background Children with cerebral palsy (CP) frequently experience chronic pain. The burden and severity of such pain is often underestimated in relation to their other impairments. Recognition and awareness of this chronic pain among children with CP constitute the cornerstone for caretakers and clinicians to improve the quality of life of those children. This study aimed to determine the prevalence of chronic pain among children with CP, and the factors associated. Methods A cross-sectional study of children with CP, aged 2–12 years, attending the CP rehabilitation clinic and Pediatric Neurology Clinic at Mulago Hospital, Uganda from November 2017 to May 2018. A detailed history and clinical examination were performed and the co-morbidities were determined. CP was classified using the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System, Communication Function Classification System (CFCS), and the Eating and Drinking Ability Classification Syste...
Journal of the International Child Neurology Association, 2019
Emerging genetic therapies for rare disorders at high cost, cannot realistically address the glob... more Emerging genetic therapies for rare disorders at high cost, cannot realistically address the global burden of disease. Stakeholders must develop new pathways to ensure safe, fair and sustainable provision of such therapies.
Introduction: Tetralogy of Fallot is one of the conotruncal abnormalities and is the most common ... more Introduction: Tetralogy of Fallot is one of the conotruncal abnormalities and is the most common cyanotic heart disease worldwide. Conotruncal abnormalities have been closely associated with 22.q.11 deletion syndrome. Children with 22.q.11 deletion syndrome often have thymic hypoplasia, hypocalcaemia and have an increased susceptibility to viral, fungal and bacterial infections due to poor T-cell production. Autoimmune vasculitis has not been well documented among individuals with Tetralogy of Fallot (TOF) though a few reports exist in association with 22.q11.2 deletion syndrome. Case summary: We report a case of an 11year old child post TOF repair who presented to the Uganda Heart Institute with persistent high grade fevers, abdominal pain, mucosal bleeds and hypocalcaemia, hypokalemia, hypomagnesaemia, thrombocytopenia and elevated acute phase reactants-leukocytosis (17.9x103), neutrophil count 16x103(89%), elevated C-reactive protein (80mg/l) and elevated immunological tests (C-ANCA, 18U/ml). She improved on intravenous methyl prednisolone. Conclusion: Children with Tetralogy of Fallot can still manifest with complications such as autoimmune disorders and severe infections given the association with 22q.11 deletion syndrome. Screening for autoimmune abnormalities and electrolytes especially calcium improves on their outcome. We were not able to perform the FISH analysis (for Fluorescent in Situ Hybridization) to confirm the diagnosis of 22q.11.2 deletion syndrome in KT.
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Papers by Richard Idro