Acute kidney injury is more common in hospitalised children with sickle cell anaemia in Africa.

AIM: To document the prevalence, severity, hospital outcome and factors associated with acute kidney injury (AKI) in hospitalised children with sickle cell anaemia (SCA). METHODS: In this prospective observational study involving children aged 0.5-17 years with SCA requiring hospitalisation, we used serum creatinine level at 0 and 48 h of hospitalisation to determine the presence of AKI. RESULTS: The study involved 155 children with SCA aged 0.5-17 years with a median (interquartile range) age of 7.8 (4.3-11.0) years. Acute kidney injury occurred in 27 (17.4%) children with 33.3% reaching stage 3. Hepatomegaly (81.5% vs. 55.4%; p = 0.015), splenomegaly (33.3% vs. 10.9%; p = 0.003), dipstick proteinuria (22.2% vs. 5.4%; p = 0.004), and hematuria (29.6% vs. 3.1%; p = <0.001) were more common in those with AKI. In contrast, children with AKI had lower haematocrit (16.9% vs. 22.2%; p = <0.001) and serum bicarbonate (16.7 vs. 19.1 mmoL/L; p = 0.010) compared with those without AKI. Those with AKI had longer hospital stay (median [interquartile range]: 7 [4-12] days vs. 4 [3-6] days; p = 0.008). CONCLUSION: AKI is common among hospitalised children with AKI and is associated with longer hospital stay.

Estimating vaccine coverage in conflict settings using geospatial methods: a case study in Borno state, Nigeria.

Reliable estimates of subnational vaccination coverage are critical to track progress towards global immunisation targets and ensure equitable health outcomes for all children. However, conflict can limit the reliability of coverage estimates from traditional household-based surveys due to an inability to sample in unsafe and insecure areas and increased uncertainty in underlying population estimates. In these situations, model-based geostatistical (MBG) approaches offer alternative coverage estimates for administrative units affected by conflict. We estimated first- and third-dose diphtheria-tetanus-pertussis vaccine coverage in Borno state, Nigeria, using a spatiotemporal MBG modelling approach, then compared these to estimates from recent conflict-affected, household-based surveys. We compared sampling cluster locations from recent household-based surveys to geolocated data on conflict locations and modelled spatial coverage estimates, while also investigating the importance of reliable population estimates when assessing coverage in conflict settings. These results demonstrate that geospatially-modelled coverage estimates can be a valuable additional tool to understand coverage in locations where conflict prevents representative sampling.

Hiding in plain sight: genetics of childhood steroid-resistant nephrotic syndrome in Sub-Saharan Africa.

Steroid-resistant nephrotic syndrome (SRNS) is the most severe form of childhood nephrotic syndrome with an increased risk of progression to chronic kidney disease stage 5. Research endeavors to date have identified more than 80 genes that are associated with SRNS. Most of these genes regulate the structure and function of the podocyte, the visceral epithelial cells of the glomerulus. Although individuals of African ancestry have the highest prevalence of SRNS, especially those from Sub-Saharan Africa (SSA), with rates as high as 30-40% of all cases of nephrotic syndrome, studies focusing on the characterization and understanding of the genetic basis of SRNS in the region are negligible compared with Europe and North America. Therefore, it remains unclear if some of the variants in SRNS genes that are deemed pathogenic for SRNS are truly disease causing, and if the leading causes of monogenic nephrotic syndrome in other populations are the same for children in SSA with SRNS. Other implications of this lack of genetic data for SRNS in the region include the exclusion of children from the region from clinical trials aimed at identifying potential novel therapeutic agents for this severe form of nephrotic syndrome. This review underlines a need for concerted efforts to advance the genetic basis of SRNS in children in SSA. Such endeavors will complement global efforts at understanding the genetic basis of nephrotic syndrome.

Assessment of the utility of a screening tool for COVID-19 diagnosis in an accident and emergency department in Lagos, Nigeria: A pilot study.

The use of reverse transcription-polymerase chain reaction (RT-PCR) is the gold standard laboratory test for diagnosing SARS-CoV-2 infection. However, it has the disadvantage of a long turnaround time and cost. The Nigeria Centre for Disease Control (NCDC) formulated a case definition for COVID-19. We sought to determine the utility of a 14-item, point-weighted clinical screening questionnaire adapted from the NCDC case definition in identifying patients more likely to have the disease. This was to aid prompt clinical decision-making. Methods: We retrospectively reviewed the data of 113 non-surgical patients presenting to the Accident and Emergency Department (A and E) of Lagos University Teaching Hospital, Lagos, Nigeria. Patients were stratified based on screening scores into low (0-2), moderate (3-5) and high (6) pre-test categories. Patients with low and high scores ≥6 were admitted to the A and E and the COVID-19 holding ward, respectively, while the moderate group had chest computed tomography scans to aid further decision-making, pending the outcome of their RT-PCR results. The validity of the triage score as compared to the RT-PCR test result was calculated and the kappa score of agreement was utilised to evaluate the concordance between two triage scores. The optimum cut-off score was also obtained based on the maximal Younden's index. Results: The frequencies of low, moderate and high pre-test scores were 34 (30%), 43 (38.1%) and 36 (31.9%), respectively. Overall, 38.1% (43/113) were RT-PCR positive. RT-PCR was positive in 26.5% (9/34) with low screening scores, 55.8% (24/43) with moderate scores and 27.8% (10/36) with high scores. The sensitivity and specificity of a high score of 6 were 25% and 92.86%, while the lower score of 3 had sensitivity and specificity of 62.5% and 58.6%, respectively. Conclusion: The screening tool showed a high specificity in its initial design, which suggests that anyone with a low score using this tool has a high probability of testing negative. We recommend a cut-off score of 4 (score A) or 6 (score B) of the current screening tool be used to increase the chances of identifying persons with COVID-19 for RT-PCR testing.

Paediatric Nephrology in Africa.

PURPOSE OF REVIEW: We highlight the unique facets of paediatric nephrology in Africa in terms of the spectrum of kidney diseases, available diagnostic and treatment modalities, kidney healthcare financing options, paediatric nephrology manpower and the contribution of geography and demographics. RECENT FINDINGS: Paediatric acute kidney injury in Africa is now commonly due to sepsis rather than gastroenteritis. Steroid-sensitive form of nephrotic syndrome is far more common than was two decades ago. SUMMARY: The hot arid climate in North Africa and the tropical climate in most of sub-Saharan Africa, and the high rate of consanguinity, sickle cell disease and HIV drive the spectrum of paediatric kidney diseases in the continent. Kidney diseases are often precipitated by infectious triggers associated with poor living conditions and little access to medical care thus resulting in late presentation and often end-stage kidney disease. Although accessibility to kidney care has improved in the continent due to training opportunities provided by international professional organisations, most children still face significant barriers to kidney care because they live in rural areas, governments spend the least on healthcare and the continent has the least density of healthcare practitioners and nephrology trainees.

A comparative study of perceived burden in parent caregivers of adolescents with epilepsy in a resource-restricted setting: Investigating the explanatory factors of perceived burden.

OBJECTIVE: Parent caregivers often play vital roles in the care of adolescents with epilepsy (AWE) in resource-restricted settings; however, little is known about the burden borne by these parents. This study investigated the burden perceived by parents of AWE and described the explanatory factors. METHODS: An equal number (n = 121) of age- and gender-matched parent caregivers of AWE (cases) and parents of adolescents with sickle cell disease (comparison group) were interviewed with the Parent Illness Intrusiveness Rating Scale to assess disruptions in their relationships and lifestyle. Parents of AWE were assessed for psychological distress with the 12-item General Health Questionnaire, and AWE were interviewed with the Hospital Depression-Anxiety Scale. RESULTS: The majority of the cases and the comparison group were mothers (76%), with mean (SD) ages of 44.11 (SD = 6.92) versus 43.59 (SD = 6.39) years, respectively. The prevalence rate of psychological distress in cases was 38%, and depressive-anxiety symptom was prevalent in 39.7% of AWE. The level of perceived burden was significant in all parent caregivers, albeit higher in cases relative to the comparison group across multiple domains, including relationship/personal development, intimacy, instrumental and global. A high level of burden in parents of AWE was predicted by a poor family financial and material support to the adolescents, increased contact hours with adolescents, psychological distress in the parent caregivers, and anxiety-depressive symptoms in AWE after controlling for cofounders. CONCLUSION: The study findings underscore the need for psychosocial support to bolster resilience and adaptive coping styles in parents of AWE, particularly in resource-restricted settings. A culturally sensitive interdisciplinary blueprint of locally viable actions model for psychosocial support for parent caregivers of AWE is strongly suggested. Future studies are indicated to shed more light on the modifiable risks of perceived burden, and the effectiveness of psychosocial interventions in parents of AWE.

Pediatric brain abscess - etiology, management challenges and outcome in Lagos Nigeria.

BACKGROUND: Brain abscess in children is a neurosurgical emergency with potentially catastrophic outcome despite the advances made in neuroimaging techniques and antibiotic therapy. Symptoms are nonspecific and may vary with the child's age, location, size, numbers and stage of abscess, and the primary source of infection. Treatment is usually with broad-spectrum antibiotics in combination and surgical evacuation in most cases or antibiotics alone in selected cases with clear-cut indications. This study was to document clinical characteristics, etiological factors, and spectrum of bacteriologic agents responsible for pediatric brain abscess in an African city, the challenges and management outcome over the study period. METHODS: This was a retrospective study over an 11-year period involving 89 children who presented with brain abscess. Information of interest was extracted from the medical records of each participant. The results from data analysis were presented in charts and tables. RESULTS: Eighty-nine children aged 0.85-15.7 years (median age of 6.4 years) met the inclusion criteria. The male-to-female ratio was 1.8:1. Headache (80%), fever (78%), and hemiparesis (78%) were the most common symptoms. Brain imaging deployed was CT scan in 56 (63%), MRI in 9 (10%), and transfontanel ultrasound scan in 24 (27%) children. Seventy-one (80%) children had antibiotics with surgical evacuation while 18 (20%) children received only antibiotics. In 19 (27%) children, the culture of the abscess was negative. In 53 (75%) children, Gram-positive aerobic organisms were isolated. A total of 75 patients (84%) had a favorable outcome. CONCLUSION: Pediatric brain abscess still poses significant public health challenge, especially in resource-limited regions. Successful management of brain abscess requires high index of suspicion for early diagnosis, referral, and intervention.

Trends in the epidemiology of childhood nephrotic syndrome in Africa: A systematic review.

Background: Childhood nephrotic syndrome, if left untreated, leads to progressive kidney disease or death. We quantified the prevalence of steroid-sensitive nephrotic syndrome, steroid-resistant nephrotic syndrome, and histological types as the epidemiology of nephrotic syndrome in Africa remains unknown, yet impacts outcomes. Methods: We searched MEDLINE, Embase, African Journals Online, and WHO Global Health Library for articles in any language reporting on childhood nephrotic syndrome in Africa from January 1, 1946 to July 1, 2020. Primary outcomes included steroid response, biopsy defined minimal change disease, and focal segmental glomerulosclerosis (FSGS) by both pooled and individual proportions across regions and overall. Findings: There were 81 papers from 17 countries included. Majority of 8131 children were steroid-sensitive (64% [95% CI: 63-66%]) and the remaining were steroid-resistant (34% [95% CI: 33-35%]). Of children biopsied, pathological findings were 38% [95% CI: 36-40%] minimal change, 24% [95% CI: 22-25%] FSGS, and 38% [95% CI: 36-40%] secondary causes of nephrotic syndrome. Interpretation: Few African countries reported on the prevalence of childhood nephrotic syndrome. Steroid-sensitive disease is more common than steroid-resistant disease although prevalence of steroid-resistant nephrotic syndrome is higher than reported globally. Pathology findings suggest minimal change and secondary causes are common. Scarcity of data in Africa prevents appropriate healthcare resource allocation to diagnose and treat this treatable childhood kidney disease to prevent poor health outcomes. Funding: Funding was provided by the Canadian Institute for Health Research (CIHR) and the National Institute of Health (NIH) for the H3 Africa Kidney Disease Research Network. This research was undertaken, in part, from the Canada Research Chairs program.

Changing epidemiology of nephrotic syndrome in Nigerian children: A cross-sectional study.

BACKGROUND: Recent reports from small studies in West Africa suggest that Black children may have high rate of steroid sensitivity nephrotic syndrome (SSNS) contrary to long held knowledge. Herein, we determined the proportion of children with idiopathic nephrotic syndrome (INS) who achieved complete remission with steroid therapy and identified factors associated with complete remission. METHODS: We reviewed the medical records of 241 children with INS in two centres in Lagos from 2010 to 2019. We extracted demographic data, clinical features, laboratory values at the time of diagnosis, and receipt and response to steroids and other immunosuppressants. RESULTS: The median (interquartile range) age at diagnosis of INS was 5.1 (3.0-8.7) years and boys were 60.2% of the study population. Children with SSNS made up 85.9% (n = 207) of the study cohort. Among those aged 0-5 years, 92.6%were SSNS compared with 69.2% in those aged 11-17 years at the time of diagnosis. In addition, the proportion of children with SSNS increased from 73.8% between year 2010 and 2012 to 88.4% afterwards. Also, children with SSNS had lower serum creatinine (0.44 vs 0.70; p<0.001) and higher estimated glomerular filtration rate (101 vs 74.3 ml/min/1.73 m2; p = 0.008) at the time of diagnosis than those with steroid resistant nephrotic syndrome (SRNS). CONCLUSION: Among Black children in Lagos, the proportion with SSNS is comparable to proportions described in children of Asian and European descent. Furthermore, children with SSNS had lower serum creatinine and higher glomerular filtration rate than those with SRNS.

Patient- and parent proxy-reported outcome measures for life participation in children with chronic kidney disease: a systematic review.

BACKGROUND: The burden of chronic kidney disease (CKD) and its treatment may severely limit the ability of children with CKD to do daily tasks and participate in family, school, sporting and recreational activities. Life participation is critically important to affected children and their families; however, the appropriateness and validity of available measures used to assess this outcome are uncertain. The aim of this study was to identify the characteristics, content and psychometric properties of existing measures for life participation used in children with CKD. METHODS: We searched MEDLINE, Embase, PsychINFO, Cumulative Index to Nursing and Allied Health Literature and the Cochrane Kidney and Transplant register to August 2019 for all studies that used a measure to report life participation in children with CKD. For each measure, we extracted and analyzed the characteristics, dimensions of life participation and psychometric properties. RESULTS: From 128 studies, we identified 63 different measures used to assess life participation in children with CKD. Twenty-five (40%) of the measures were patient reported, 7 (11%) were parent proxy reported and 31 (49%) had both self and parent proxy reports available. Twenty-two were used in one study only. The Pediatric Quality of Life Inventory version 4.0 generic module was used most frequently in 62 (48%) studies. Seven (11%) were designed to assess ability to participate in life, with 56 (89%) designed to assess other constructs (e.g. quality of life) with a subscale or selected questions on life participation. Across all measures, the three most frequent activities specified were social activities with friends and/or family, leisure activities and self-care activities. Validation data in the pediatric CKD population were available for only 19 (30%) measures. CONCLUSIONS: Life participation is inconsistently measured in children with CKD and the measures used vary in their characteristics, content and validity. Validation data supporting these measures in this population are often incomplete and are sparse. A meaningful and validated measure for life participation in children with CKD is needed.

Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome.

To understand the genetics of steroid-sensitive nephrotic syndrome (SSNS), we conducted a genome-wide association study in 987 childhood SSNS patients and 3,206 healthy controls with Japanese ancestry. Beyond known associations in the HLA-DR/DQ region, common variants in NPHS1-KIRREL2 (rs56117924, P=4.94E-20, odds ratio (OR) =1.90) and TNFSF15 (rs6478109, P=2.54E-8, OR=0.72) regions achieved genome-wide significance and were replicated in Korean, South Asian and African populations. Trans-ethnic meta-analyses including Japanese, Korean, South Asian, African, European, Hispanic and Maghrebian populations confirmed the significant associations of variants in NPHS1-KIRREL2 (Pmeta=6.71E-28, OR=1.88) and TNFSF15 (Pmeta=5.40E-11, OR=1.33) loci. Analysis of the NPHS1 risk alleles with glomerular NPHS1 mRNA expression from the same person revealed allele specific expression with significantly lower expression of the transcript derived from the risk haplotype (Wilcox test p=9.3E-4). Because rare pathogenic variants in NPHS1 cause congenital nephrotic syndrome of the Finnish type (CNSF), the present study provides further evidence that variation along the allele frequency spectrum in the same gene can cause or contribute to both a rare monogenic disease (CNSF) and a more complex, polygenic disease (SSNS).

Significant Burden and Psychological Distress Among Caregivers of Children With Nephrotic Syndrome: A Cross-Sectional Study.

BACKGROUND: Childhood nephrotic syndrome (NS) follows a chronic course in most children. However, little is known about the psychosocial burden of NS on the caregivers despite evidence that caregiver burden or impairment in their well-being may alter the outcome of chronic childhood illnesses. OBJECTIVES: To determine the frequency and predictors of significant caregiver burden and psychological distress among caregivers of children with NS. DESIGN: A cross-sectional study. SETTING: Two pediatric nephrology clinics in Lagos, Nigeria. PATIENTS: We included primary caregivers of children with idiopathic NS for at least 6 months. MEASUREMENTS: The primary outcomes were psychological distress and significant caregiver burden among caregivers. METHODS: We interviewed caregivers using the 12-item General Health Questionnaire (GHQ-12) and the 6-item Zarit Burden Interview (ZBI-6). The GHQ-12 scores ≥ 3 and ZBI-6 scores ≥ 6 indicated psychological distress and significant caregiver burden, respectively. RESULTS: The caregivers were mostly mothers (77.9%) and married (92.4%), whereas the children (n = 172) were mainly male (65.1%). Most of the children (n = 152; 88.4%) had steroid-sensitive NS including 24 (14%) children with frequent relapses or steroid dependence and 20 (11.6%) with steroid-resistant NS. Of the 172 caregivers, 53 (30.8%) and 30 (17.4%) reported psychological distress and significant burden, respectively. Caregivers of children in relapse had adjusted an odds ratio (aOR) with 95% confidence interval (CI) of 2.45 (1.05-5.67) and 3.30 (1.22-8.92) of psychological distress and significant caregiver burden, respectively. Furthermore, caregivers of male children and those who needed help paying for health care had an aOR of 4.61 (1.34-15.68) and 3.06 (1.06-8.87) of significant caregiver burden, respectively. LIMITATIONS: The study was limited by its cross-sectional design and the use of generic rather than disease-specific instruments. CONCLUSION: One in every 6 caregivers of children with idiopathic NS reported significant caregiver burden, and it was associated with psychological distress. Our findings underscore the need for psychosocial support for caregivers of children with NS, especially those with identifiable vulnerability.

CONTEXTE: Le syndrome néphrotique (SN) de l'enfant suit dans la plupart des cas une évolution chronique. On en sait toutefois peu sur le fardeau psychosocial du SN pour les aidants naturels, malgré qu'il soit prouvé qu'un tel fardeau ou qu'une atteinte à leur bien-être peut altérer l'issue des maladies chroniques de l'enfance. OBJECTIF: Établir la fréquence et les facteurs prédictifs d'un important fardeau des aidants et de la détresse psychosociale chez les personnes qui prennent soin d'un enfant atteint du SN. TYPE D'ÉTUDE: Étude transversale. CADRE: Deux cliniques de néphrologie pédiatrique de Lagos au Nigéria. PARTICIPANTS: Ont été inclus les aidants naturels d'enfants atteints du SN idiopathique depuis au moins six mois. MESURES: Les principaux résultats étaient la détresse psychologique et un important fardeau chez les aidants naturels. MÉTHODOLOGIE: Nous avons interrogé des aidants naturels à l'aide d'un questionnaire en 12 points sur l'état de santé général (GHQ-12) et de l'Inventaire du fardeau en six points (Zarit Burden Interview­ZBI-6). Un résultat égal ou supérieur à 3 au GHQ-12 indiquait la présence de détresse psychologique alors qu'un résultat égal ou supérieur à 6 au ZBI-6 signifiait un important fardeau de l'aidant. RÉSULTATS: Dans la majorité des cas (77,9 %), l'aidant naturel était la mère et celle-ci était mariée (92,4 %). Les enfants (n = 172) étaient majoritairement des garçons (65,1 %). La plupart des enfants (n = 152; 88,4 %) étaient atteints d'un SN stéroïdosensible, dont 24 (14 %) avaient des rechutes fréquentes ou une dépendance aux stéroïdes. Seuls 20 patients (11,6 %) étaient atteints d'un SN résistant aux stéroïdes. Des 172 aidants naturels inclus, 53 (30,8 %) ont rapporté vivre de la détresse psychologique et 30 (17,4 %) un important fardeau des aidants. Le rapport de cote corrigé (RCc) avec intervalle de confiance à 95 % (IC 95 %) des aidants d'enfants en rechute s'établissait à 2,45 (1,05-5,67) pour la détresse psychologique et à 3,30 (1,22-8,92) pour le fardeau des aidants. En outre, les soignants d'un garçon ou ceux qui avaient besoin d'aide pour payer les soins de santé présentaient respectivement un RCc de 4,61 (1,34-15,68) et de 3,06 (1,06-8,87) pour le fardeau des aidants. LIMITES: Les résultats sont limités par la nature transversale de l'étude et par l'emploi d'instruments de mesure générique plutôt que spécifiques à la maladie. CONCLUSION: Une personne sur six s'occupant d'un enfant atteint du SN idiopathique a rapporté un lourd fardeau des aidants associé à de la détresse psychologique. Nos résultats font ressortir le besoin de soutien psychologique pour les soignants d'enfants atteints du SN, particulièrement ceux dont la vulnérabilité est facilement repérable.

Acute Kidney Injury in Children with Severe Malaria Is Common and Associated with Adverse Hospital Outcomes.

BACKGROUND: The prevalence of acute kidney injury (AKI) in children with severe malaria in sub-Saharan African may have been underestimated. The study aimed to determine the prevalence of AKI in children with severe malaria and its association with adverse hospital outcomes. METHODS: At presentation, we measured complete blood count, serum bilirubin, and serum electrolytes, urea and creatinine in children with severe malaria. At 24 h after hospitalization, we repeated serum creatinine measurement. Urine passed in the first 24 h of hospitalization was also measured. We defined AKI and its severity using the Kidney Disease: Improving Global Outcome AKI guidelines. RESULTS: The study involved 244 children (53.3% males) with a median age of 3.5 (1.9-7.0) years. One hundred and forty-four (59%) children had AKI, and it reached maximum Stages 1, 2 and 3 in 56 (23%), 45 (18.4%) and 43 (17.6%) children, respectively. The majority (86.1%) with AKI had only elevated serum creatinine. Mortality increased with increasing severity of AKI on univariate analysis but weakened on multiple logistic regression. Mortality was also higher in those with both oliguria and elevated serum creatinine than in those with elevated serum creatinine only (50% vs. 4.8%, p < 0.001). Furthermore, children with AKI spent three days more in hospital than those without AKI (p < 0.001). CONCLUSIONS: Acute kidney injury complicates severe malaria in 6 out of every 10 children and is commonly identified using elevated serum creatinine. It is also associated with adverse hospital outcome.

Helicobacter pylori Infection in Malnourished Children in Lagos.

BACKGROUND/AIM: Helicobacter pylori infection is acquired in childhood, but there are conflicting reports on malnutrition and the infection. This study aimed to determine the prevalence of H. pylori infection among malnourished children and highlight the socioeconomic (SE) and clinical factors associated with the infection. METHODOLOGY: This was a descriptive cross-sectional study of 122 malnourished children and 120 healthy controls. Anthropometry was done for all the study participants, and the H. pylori status was determined with the use of monoclonal stool antigen test in all the participants. Logistic regression analysis was used to determine the factors that could predict the occurrence of the infection in the children. RESULTS: Seventy percent (70.8%) of the malnourished children had moderate malnutrition, whereas 29.2% were severely malnourished. The prevalence of H. pylori in the malnourished children was 22.8% compared to 32.5% in the controls (P = 0.09). The infection was most prevalent in toddlers (60.7%). The SE class was significantly related to the infection (P = 0.01) and about a fifth (21.3%) of the malnourished children who belonged to the low SE class were H. pylori positive compared to 9.2% of the controls. About 64.3% of the malnourished children with H. pylori infection had fever and 25.8% had diarrhea. Multivariate analysis showed that stunting was significantly related to the infection (P = 0.02). CONCLUSION: H. pylori infection was prevalent among the toddlers and was significantly associated with stunting in this cohort of malnourished children. Screening of children for the infection is still advocated, and infected children should be referred for appropriate treatment and follow-up. The relationship between SE class and the infection still requires further research.

Infantile supratentorial subdural empyema managed by percutaneous aspiration: an outcome study in a Nigerian city.

Subdural empyema (SDE), a common neurosurgical emergency in the developing countries, accounts for 15%-20% of localised paediatric intracranial infections. In regions where modern diagnostic tools are scarce and inaccessible, detection of SDE may be delayed with subsequent poor outcome. Percutaneous subdural aspiration in patients with open anterior fontanel may be the only surgical option in resource-poor regions of the world. This review focuses on the management outcome, including neurological outcome of these children. Clinical charts of children with SDE and treated by percutaneous subdural tap between February 2006 and August 2014 were reviewed. Demographic, clinical, radiological, bacteriological parameters and outcome data were analysed. Forty-five children with a mean age of 10.6 ± 6.2 months (range: 2-17 months) and followed up for a median duration of 16.4 months were included. The most frequent clinical features were enlarged head circumference, fever, focal neurologic deficits and altered level of consciousness. Diagnosis of SDE was confirmed using trans-fontanel ultrasound scan in 32 (71.1%) children, computerised tomography in 12 (26.7%) children and magnetic resonance imaging in one (2.2%) child. SDE was unilateral in 73.3% and bilateral in 26.7%. In 23 (51.1%) children with a positive culture, Staphylococcus aureus (n = 10), anaerobes (n = 7), Escherishia coli and Haemophilus influenza (n = 6 each) were the most common organisms. Forty-three children (95.6%) survived, 36 of which had good Glasgow outcome score. Seven children still had moderate deficits at 3 months. Treatment of SDE in young children with patent fontanel using percutaneous subdural tap has good therapeutic and neurological outcome.

Genetics of Childhood Steroid Sensitive Nephrotic Syndrome: An Update.

Advances in genome science in the last 20 years have led to the discovery of over 50 single gene causes and genetic risk loci for steroid resistant nephrotic syndrome (SRNS). Despite these advances, the genetic architecture of childhood steroid sensitive nephrotic syndrome (SSNS) remains poorly understood due in large part to the varying clinical course of SSNS over time. Recent exome and genome wide association studies from well-defined cohorts of children with SSNS identified variants in multiple MHC class II molecules such as HLA-DQA1 and HLA-DQB1 as risk factors for SSNS, thus stressing the central role of adaptive immunity in the pathogenesis of SSNS. However, evidence suggests that unknown second hit risk loci outside of the MHC locus and environmental factors also make significant contributions to disease. In this review, we examine what is currently known about the genetics of SSNS, the implications of recent findings on our understanding of pathogenesis of SSNS, and how we can utilize these results and findings from future studies to improve the management of children with nephrotic syndrome.

Genetic Testing for Steroid-Resistant-Nephrotic Syndrome in an Outbred Population.

Background: Steroid-resistant nephrotic syndrome (SRNS) is a leading cause of end-stage kidney disease in children and young adults. Despite advances in genomic science that have led to the discovery of >50 monogenic causes of SRNS, there are no clear guidelines for genetic testing in clinical practice. Methods: Using high throughput sequencing, we evaluated 492 individuals from 181 families for mutations in 40 known SRNS genes. Causative mutations were defined as missense, truncating, and obligatory splice site variants with a minor allele frequency <1% in controls. Non-synonymous variants were considered pathogenic if determined to be deleterious by at least two in silico models. We further evaluated for differences in age at disease onset, family history of SRNS or chronic kidney disease, race, sex, renal biopsy findings, and extra-renal manifestations in subgroups with and without disease causing variants. Results: We identified causative variants in 40 of 181 families (22.1%) with SRNS. Variants in INF2, COL4A3, and WT1 were the most common, accounting for over half of all causative variants. Causative variants were identified in 34 of 86 families (39.5%) with familial disease and 6 of 95 individuals (6.3%) with sporadic disease (χ2 p < 0.00001). Family history was the only significant clinical predictor of genetic SRNS. Conclusion: We identified causative mutations in almost 40% of all families with hereditary SRNS and 6% of individuals with sporadic disease, making family history the single most important clinical predictors of monogenic SRNS. We recommend genetic testing in all patients with SRNS and a positive family history, but only selective testing in those with sporadic disease.

Human immunodeficiency virus status in malnourished children seen at Lagos.

INTRODUCTION: Human immunodeficiency virus and protein energy malnutrition are still prevalent in Nigeria and the occurrence of the two conditions together confers a poor prognosis. The aim of this study was to determine the current categories of malnutrition amongst under-5 children in Lagos, document their HIV status and determine any peculiarities in the clinical features, haematological and some biochemical profile in these children. METHODS: The study was a cross-sectional study conducted at the Paediatric departments of the Lagos University Teaching Hospital and the Massey Street Children's Hospital, both in Lagos, over a 6-month period. All the subjects had anthropometry, HIV testing, full blood count and serum proteins done. The factors associated with HIV status were determined with the logistic regression analysis. RESULTS: Two hundred and fourteen (214) malnourished children ≤5 years, including 25 (11.7%) with HIV were recruited in the study. Among the study participants, 150 (70.1%) and 54 (29.9%) had moderate and severe malnutrition, respectively. Fever, cough and diarrhea were the most common symptoms in the study participants. The haematological indices were comparable in the two groups, the serum globulin levels though higher in the HIV infected group was not statistically significantly different from the non-infected group.(p = 0.66). None of the factors explored on multivariate analysis was able to predict the occurrence of the infection in this cohort. CONCLUSION: Malnourished children remain a high risk group for HIV infection and the prevalence of the infection obtained in this group of children is still unacceptably high. Discriminatory features between malnutrition and HIV remains difficult. The presence of hyperglobulinaemia on laboratory analysis in a malnourished child may heighten the suspicion of possible underlying associated HIV infection. Screening of malnourished children for HIV infection and further longitudinal studies on malnourished children with HIV is advocated.

Enuresis in children and adolescents with sickle cell anaemia is more frequent and substantially different from the general population.

BACKGROUND: No large studies have examined the prevalence of enuresis, its various forms and risk factors in children with sickle cell anaemia (SCA) in Sub-Saharan Africa using standardised definitions. We determined age and gender-specific prevalence of enuresis and compared the nature of enuresis in children with and without SCA. We also identified predictors of enuresis in children with SCA. METHODS: Caregivers of children with SCA attending a tertiary centre haematology clinic in Nigeria were interviewed using a questionnaire. In addition, a separate questionnaire was completed for every sibling aged 5-17 years whose haemoglobin genotype was known. Enuresis and its various forms were defined using the definitions of the International Children's Continence Society. RESULTS: The study involved 243 children with SCA and 243 controls matched for age and sex. The mean age of the study cohort was 9.9 (3.4). Females made up 45.7% of the cohorts. The prevalence of enuresis was 49.4% and 29.6% in children with and without SCA, respectively (p = 0.009). In both groups, the prevalence of enuresis declined with age but remained five times higher at 25% in children with SCA aged 14-17 years compared with controls. Also, children with SCA and enuresis were older, more likely to have non-monosymptomatic enuresis and wet at least three nights per week than controls. Independent predictors of enuresis in children with SCA were a family history of enuresis and young age. CONCLUSION: Children with SCA had more frequent and more severe enuresis which persisted to late adolescence than age and sex-matched controls. These features indicate a subset of enuresis that is difficult to treat in the general population. Young age and enuresis in a family member define a subset of children with SCA more likely to have enuresis. Healthcare workers need to discuss enuresis with parents of children with SCA and offer referral to continence services.