Inborn Errors of Immunity and Efforts to Diagnose Affected Children in the Absence of Training and Specialty Practice in Clinical Immunology in Ethiopia: a Brief Report.

Four in five children with inborn errors of immunity globally remain undiagnosed. These figures are disproportionally high in low-income countries like Ethiopia. Apart from the inclusion of basic overviews of these disorders in to postgraduate pediatric curricula, little effort has been placed in to establishing clinical immunology training programs. This report summarizes the existing epidemiology of inborn errors of immunity in Ethiopia, unique presentations in Ethiopian children, challenges faced in diagnosing them, and efforts to improve their management.

Assessment of level of asthma control and related factors in children attending pediatric respiratory clinics in Addis Ababa, Ethiopia.

BACKGROUND: Asthma is a common airways disease with significant morbidity and mortality in all ages. Studies of pediatric asthma control and its determinants yielded variable results across settings. However, there is paucity of data on asthma control and its factors in Ethiopian children. We aimed to assess the level of asthma control and the related factors in children attending pediatric respiratory clinics at three tertiary hospitals in Addis Ababa. METHODS: We conducted a cross-sectional study from March 1 to August 30, 2020 using standardized questionnaires and review of patient's charts. Data was analyzed using SPSS software for window version 26. RESULTS: A total of 105 children (56.2% male) were included in the study. The mean age (± SD) and age at Asthma diagnosis (± SD) were 6 (± 3.3) and 4 (± 2.8) respectively. Uncontrolled asthma was present in 33 (31%) of children. Comorbidities (Atopic dermatitis and allergic Rhinitis (AOR = 4.56; 95% CI 1.1-18.70; P = 0.035), poor adherence to controller medications (AOR = 3.23; 95% CI 1.20-10.20; P = 0.045), inappropriate inhaler technique (AOR = 3.48; 95% CI 1.18-10.3; P = 0.024), and lack of specialized care (AOR = 4.72; 95% CI 1.13-19.80; P = 0.034) were significantly associated with suboptimal asthma control. CONCLUSION: One-third of children attending pediatric respiratory clinics in Addis Ababa had uncontrolled Asthma. Treatment of comorbidities, training of appropriate inhaler techniques, optimal adherence to controllers, and proper organization of clinics should be emphasized to improve asthma control among children.

Respiratory syncytial virus in severe lower respiratory infections in previously healthy young Ethiopian infants.

BACKGROUND: Respiratory Syncytial Virus (RSV) is the commonest cause of acute lower respiratory infections (ALRI) in infants. However, the burden of RSV is unknown in Ethiopia. We aimed to determine the prevalence, seasonality and predictors of RSV infection in young infants with ALRI for the first time in Ethiopia. METHODS: We performed RSV immuno-chromatographic assay from nasopharyngeal swabs of infants, 29 days to 6 months of age. We included the first 10 eligible infants in each month from June 2018 to May 2019 admitted in a tertiary pediatric center. Clinical, laboratory and imaging data were also collected, and chi-square test and regression were used to assess associated factors with RSV infection. RESULTS: Among a total of 117 study children, 65% were male and mean age was 3 months. Bronchiolitis was the commonest diagnosis (49%). RSV was isolated from 26 subjects (22.2%) of all ALRI, 37% of bronchiolitis and 11% of pneumonia patients. Although RSV infection occurred year round, highest rate extended from June to November. No clinical or laboratory parameter predicted RSV infection and only rainy season (Adjusted Odds Ratio (AOR) 10.46 [95%. C.I. 1.95, 56.18]) was independent predictor of RSV infection. CONCLUSIONS: RSV was isolated in a fifth of young infants with severe ALRI, mostly in the rainy season. Diagnosis of RSV infection in our setting require specific tests as no clinical parameter predicted RSV infection. Since RSV caused less than a quarter of ALRI in our setting, the other causes should be looked for in future studies.

Knowledge and attitudes about genetic counseling in patients at a major hospital in Addis Ababa, Ethiopia.

Previous work at St. Paul's Hospital Millennium Medical College (SPHMMC) in Addis Ababa, Ethiopia, demonstrated a need for genetic counseling (GC) services, with 4% of pediatric, neonatal intensive care, and prenatal patients identified as having indications for genetic evaluation (Quinonez et al, 2019). The aim of this study was to investigate SPHMMC patients' familiarity with, knowledge of, and attitudes toward GC services. Surveys were adapted from previous work in North America populations (Riesgraf et al, 2015 and Gemmell et al, 2017) and administered to 102 patients, and results were compared to North American populations using Student's t test. 30% of respondents reported at least some familiarity with GC, primarily via the media or healthcare providers. Patients had generally positive attitudes toward GC, reporting they would trust information provided by a genetic counselor and that GC is in line with their values. Knowledge of GC showed similar trends overall when compared to results from North American populations. Our work indicates limited exposure to GC in this population, but generally positive feelings toward GC. Patients' attitudes toward GC were comparable to rural North American populations surveyed using the same tool on most items; however, cultural differences including views on abortions and directiveness of healthcare providers could account for discrepancies and are important considerations when implementing genetic services globally.

Retropharyngeal Tuberculous Abscess: A Rare Cause of Upper Airway Obstruction and Obstructive Sleep Apnea in Children: A Case Report.

Retropharyngeal tuberculous abscess (RPTBA) is a rare manifestation of tuberculosis (TB) even in high TB burden areas. It rarely manifests as a cause of upper airway obstruction and obstructive sleep apnea (OSA) in children with few case reports in the literature. We report a 22 months old toddler who presented with upper airway obstruction and OSA and was diagnosed with RPTBA. The child recovered completely and growing normally after intra-oral aspiration and 6 months of anti-tuberculosis treatment.

Patients' perception of quality of nursing care; a tertiary center experience from Ethiopia.

BACKGROUND: Nursing care closely influences patients' satisfaction with the overall quality of care, and the importance of measuring patient satisfaction with nursing care cannot be emphasized enough. Data are however scarce regarding patients' perception of quality of nursing care in Ethiopia. We performed this study to assess patient's perception of the quality of nursing care in a tertiary center in Ethiopia. METHODS: Data were collected prospectively using Quality of Nursing Care Questionnaires-patient of Safford & Schlotfeldt. A total of 340 patients were included using systematic random sampling and data were analyzed using SPSS for windows version- 20. RESULT: The nursing care performance was highest for nurse-physician relation (mean = 3.95) and low for education and home care preparation and physical care (mean score of 2.79 and 2.89 respectively). The emotional care and nurse administration mean score were 3.5 and 3.83 respectively. The overall nursing quality was neither satisfying nor dissatisfying (mean of 3.39). While only 36% of the respondents were satisfied with the nursing care, patient education has the strongest (AOR of 7.4) association with satisfaction. CONCLUSION: Patients perceived low quality of physical care, education and preparation for home care but better nurse-physician relation and nursing administration. However the overall quality measure was neither satisfying nor dissatisfying. This calls for an action from the health care administrators, educators and other stakeholders to improve the patient perception of quality nursing care.

Effectiveness of bubble continuous positive airway pressure for treatment of children aged 1-59 months with severe pneumonia and hypoxaemia in Ethiopia: a pragmatic cluster-randomised controlled trial.

BACKGROUND: The safety and efficacy of bubble continuous positive airway pressure (bCPAP) for treatment of childhood severe pneumonia outside tertiary care hospitals is uncertain. We did a cluster-randomised effectiveness trial of locally made bCPAP compared with WHO-recommended low-flow oxygen therapy in children with severe pneumonia and hypoxaemia in general hospitals in Ethiopia. METHODS: This open, cluster-randomised trial was done in 12 general (secondary) hospitals in Ethiopia. We randomly assigned six hospitals to bCPAP as first-line respiratory support for children aged 1-59 months who presented with severe pneumonia and hypoxaemia and six hospitals to standard low-flow oxygen therapy. Cluster (hospital) randomisation was stratified by availability of mechanical ventilation. All children received treatment in paediatric wards (in a dedicated corner in front of a nursing station) with a similar level of facilities (equipment for oxygen therapy and medications) and staffing (overall, one nurse per six patients and one general practitioner per 18 patients) in all hospitals. All children received additional care according to WHO guidelines, supervised by paediatricians and general practitioners. The primary outcome was treatment failure (defined as any of the following: peripheral oxygen saturation <85% at any time after at least 1 h of intervention plus signs of respiratory distress; indication for mechanical ventilation; death during hospital stay or within 72 h of leaving hospital against medical advice; or leaving hospital against medical advice during intervention). The analysis included all children enrolled in the trial. We performed both unadjusted and adjusted analyses of the primary outcome, with the latter adjusted for the stratification variable and for the design effect of cluster randomisation, as well as selected potentially confounding variables, including age. We calculated effectiveness as the relative risk (RR) of the outcomes in the bCPAP group versus low-flow oxygen group. This trial is registered with ClinicalTrial.gov, NCT03870243, and is completed. FINDINGS: From June 8, 2021, to July 27, 2022, 1240 children were enrolled (620 in hospitals allocated to bCPAP and 620 in hospitals allocated to low-flow oxygen). Cluster sizes ranged from 103 to 104 children. Five (0·8%) of 620 children in the bCPAP group had treatment failure compared with 21 (3·4%) of 620 children in the low-flow oxygen group (unadjusted RR 0·24, 95% CI 0·09-0·63, p=0·0015; adjusted RR 0·24, 0·07-0·87, p=0·030). Six children died during hospital stay, all of whom were in the low-flow oxygen group (p=0·031). No serious adverse events were attributable to bCPAP. INTERPRETATION: In Ethiopian general hospitals, introduction of locally made bCPAP, supervised by general practitioners and paediatricians, was associated with reduced risk of treatment failure and in-hospital mortality in children with severe pneumonia and hypoxaemia compared with use of standard low-flow oxygen therapy. Implementation research is required in higher mortality settings to consolidate our findings. FUNDING: SIDA Sweden and Grand Challenges Ethiopia.

Unilateral pulmonary vein atresia presenting with recurrent hemoptysis and bronchial varices in an Ethiopian adolescent: a case report.

BACKGROUND: Congenital unilateral pulmonary vein atresia is a rare anomaly resulting from failure of the pulmonary vein to incorporate in the left atrium. It is a very rare cause of recurrent respiratory infections and hemoptysis requiring a high index of suspicion for proper diagnosis and management in early childhood. CASE PRESENTATION: We report a 13-year old Anuac (Ethiopia, Region of Gambela) male adolescent with a delayed diagnosis of isolated atresia of the left pulmonary veins despite early childhood presentation with recurrent chest infections, hemoptysis and exercise intolerance. Contrast enhanced CT of thorax with reconstructed planes confirmed the diagnosis. He underwent pneumonectomy for severe and recurrent symptoms and did well on subsequent follow ups after 6 months of pneumonectomy. CONCLUSION: Although a rare anomaly, congenital unilateral pulmonary vein atresia should be considered in the differential diagnosis of a child presenting with recurrent chest infections, exercise intolerance and hemoptysis to facilitate early appropriate diagnosis and treatment.

Re-Expansion Pulmonary Edema in Children - A Rare Complication After Pneumothorax Drainage: A Case Report.

Background: Re-expansion Pulmonary Edema (RPE) is a non-cardiogenic form of pulmonary edema which occurs following rapid lung expansion after drainage of significant pneumothorax or pleural effusion, and rarely following resection of obstructive mediastinal mass. RPE is a rare but potentially fatal phenomenon with only few case reports in the pediatric literature. Methods: We are reporting a case of RPE in a 5-year-old girl following drainage of pneumothorax who succumbed to worsening hypoxemia despite therapy with mechanical ventilation and other supportive care. Conclusion: RPE should be anticipated, and early preventive, diagnostic and therapeutic measures should be instituted in high-risk patients who require significant pleural fluid or air drainage.

Clinical Characteristics and Outcome of Pediatric COVID-19 Patients in Ethiopia During the Early COVID-19 Pandemic: A Prospective Cohort Study.

Introduction: Most previous pediatric COVID-19 studies reported milder disease in children. However, there are limited pediatric data from low-income settings. We aimed to assess the characteristics and outcomes of pediatric COVID-19 in Ethiopia. Setting: St. Paul's COVID-19 treatment center; a tertiary COVID-19 center. Pediatric care was provided in a dedicated ward but with a common ICU. Methods: St. Paul's Hospital COVID-19 cohort (SPC-19) included inpatient COVID-19 RT-PCR confirmed cases from August 2020 to January 2021. Data were extracted from case report forms attached to patient charts and completed by the clinicians. Data were uploaded into the Redcap database and exported to SPSS 20 for analysis. Binary logistic regression and chi-square test were used in the analysis. Results: Seventy-nine patients 0-19 years were included from the SPC-19 cohort over 6 months. Pediatric admissions accounted for 11% of cases in the cohort. The mean age (SD) was 6.9 (±6.36) years and 40 (50.6%) were female. The disease was asymptomatic or mild in 57 (72.2%), moderate in 15 (19%), and severe or critical in 7 (8.8%). The commonest presentations in symptomatic children were prostration (26.6%) followed by vomiting (12.7%), fever and cough (11.4% each), and dyspnea (10%). About 53 (67%) children had multimorbidity, and 14 (17.7%) children died. All deaths were in children with comorbidities with tuberculosis and malignancy being associated with 43% of deaths. Nearly 5% of children reported long-COVID symptoms highlighting the need for prolonged follow-up in those children. Conclusion: Despite lower admissions and severity, high mortality and morbidity was documented in our pediatric cohort. The presence of comorbidity and inadequate care organization likely contributed to high mortality. COVID-19 centers of low-income settings should emphasize optimizing the care of children with COVID-19 and multimorbidity, and vaccination should be considered in those children to prevent high morbidity and mortality.

Mendelian Susceptibility to Mycobacterial Diseases (MSMD) in a 13-Year-Old Ethiopian Girl with Autosomal Dominant Interferon Gamma Receptor 1(IFN-γ R1) Defect: A Clinical Diagnostic and Treatment Challenge.

Background: Mendelian susceptibility to mycobacterial diseases (MSMD) is an inborn error of immunity categorized as defects in intrinsic and innate immunity. MSMD is characterized by vulnerability to less virulent mycobacteria, such as Bacillus Calmette-Guérin (BCG) vaccine strains, as well as environmental mycobacteria (EM). The definitive diagnosis is made by genetic analysis. Treatments constitute antimycobacterial, interferon-gamma, surgery, and hematopoietic stem cell transplantation (HSCT), which is the only known curative treatment. The mortality rate ranges from 40% to 80% depending on the severity of the mutation. Case: A 13-year-old female patient had multiple hospital visits since the age of 6 months. The most striking diagnosis was repeated mycobacterial infections. She had tuberculosis affecting lymph nodes, skin and soft tissue, bone and joints, the lungs, and epidural and paraspinal regions. She has taken all the childhood vaccines, including BCG. She has been treated four times with first-line and once with second-line antituberculosis drugs. Currently, she is on treatment for nontuberculous mycobacteria and is receiving interferon-gamma. Genetic studies showed autosomal dominant Mendelian susceptibility to mycobacterial disease due to IFNG-R1 defect. Conclusion: To the authors' knowledge, this is the first case report of Mendelian susceptibility to mycobacterial diseases secondary to interferon gamma receptor 1(IFNG-R1) defect in Ethiopia. Although it has been immensely challenging, our multidisciplinary team has learned a lot from the clinical presentation, diagnosis, and management of this child.

Perception and experience of clinicians and caregivers in treating childhood severe pneumonia and hypoxemia using bubble continuous positive airway pressure in Ethiopian tertiary and general hospitals.

BACKGROUND: In low and middle-income countries (LMICs), severe pneumonia with hypoxemia is the leading cause of child deaths, even with the provision of WHO-recommended antibiotic therapy, oxygen therapy and other supportive care. Previous studies found positive outcomes from the use of bubble continuous positive airway pressure (bCPAP) for treating these children compared to the standard oxygen therapy. Due to lack of data on the perceptions and experiences of hospital health care workers and caregivers of children on the feasibility and acceptability of bCPAP in treating children with severe pneumonia and hypoxemia in real-life settings, we examined these issues in tertiary and general hospitals in Ethiopia. METHODS: As part of a three-stages clinical trial, this qualitative study was conducted in two tertiary (stage I) and two general (stage II) hospitals from September 2019 to July 2020. During stages I and II, we have consecutively enrolled children with severe pneumonia and hypoxemia and put them on bCPAP to examine its feasibility and acceptability by clinicians and parents. A total of 89 children were enrolled (49 from two tertiary and 40 from two general hospitals). Then qualitative data were collected through 75 repeated in-depth interviews by social-science experts with purposively selected 30 hospital health workers and 15 parents of 12 children who received bCPAP oxygen therapy in the hospitals. Interview data were supplemented by 6 observations in the hospitals. Data were analyzed using a thematic approach. RESULTS: Identified structural and functional challenges for the introduction of bCPAP in treating childhood severe pneumonia and hypoxemia in the study hospitals include: inadequate number of pulse oximeters; unavailability of nasal prongs with age-specific size; inadequate and non-functioning oxygen flow meters, concentrator, and cylinders; disruption in power-supply; and inadequate number of staff. The opportunities in introducing bCPAP oxygen therapy included the availability of a dedicated corner for the study patients situated in front of nurse's station, required medicines and satisfactory level of clinicians' knowledge and skills for treating severe pneumonia patients. Additionally, the identified operational challenges were occasional lack of bubbling in the water-filled plastic bottle, lack of stand for holding the water-filled plastic bottle, and delayed shifting of oxygen source from an oxygen concentrator to a cylinder, particularly during electricity disruption. Participants (clinicians and parents) expressed their satisfaction as bCPAP oxygen therapy was found to be simple to handle, children had ease of breathing and recovered fast without major ill effects. CONCLUSION: Our study identified some important structural, functional, and operational challenges that need to be addressed before implementation of bCPAP oxygen therapy especially in frontline general hospitals with limited resources. In spite of these observed challenges, the clinicians and caregivers were highly satisfied with the overall performance of bCPAP oxygen therapy.

Isolated absence of right pulmonary artery in a 4-year old child: a case report.

Isolated unilateral absence of the pulmonary artery is a rare anomaly with congenital absence of the left or right pulmonary artery in the absence of other cardiac malformation, and diagnosis is usually made during adolescence. We report a 4-year old male child with isolated absence of the right pulmonary artery, who presented with recurrent chest infection and wheezing since infancy.

Antibiotic prescribing practice in the management of cough or diarrhea among children attending hospitals in Addis Ababa: a cross-sectional study.

BACKGROUND: Irrational use of antibiotics is a global problem. Failure to follow clinical guidelines is one of the main reasons for irrational use of antibiotics. Cough and/or diarrhea are the main childhood illnesses in Ethiopia, and health care providers are expected to follow the national guideline while managing these illnesses. This study tried to assess the extent of adherence to the guideline while managing cases of childhood diarrhea and/or cough. METHODS: The study was conducted in 23 hospitals in Addis Ababa. Data were collected prospectively from April to June 2016 using a structured questionnaire. A total of 1,073 children aged 2-59 months who visited the hospitals for cough or diarrhea during the study period were included in the study. Equal number of cases were allocated to each hospital and consecutive cases were included in the study until the calculated sample size was attained for each hospital. Data collectors approached cases after they were seen by the health care provider. SPSS version 20 was used to analyze the data. RESULT: Of the total number of cases, an antibiotic was prescribed for 794 (74.0%) of the children. Cotrimoxazol 209 (26.3%), amoxicillin 185 (23.3%), and cephalosporines 174 (21.9%) were the three most commonly prescribed antibiotics. Six hundred eighty-eight (86.6%) of the prescriptions were determined to be inappropriate. Of the inappropriate prescriptions, 631 (91.7%) were for prescribing antibiotics when not necessary and 57 (8.3%) were for prescribing the wrong spectrum of antibiotics. Using multivariate analysis, a child not having diarrhea was independently associated with appropriate antibiotic prescription (adjusted odds ratio =0.261, 95% confidence interval: 0.095-0.714). The prescriber being qualified as a pediatrician was an independent predictor of inappropriate antibiotic prescription (adjusted odds ratio =9.967, 95% confidence interval: 4.221-23.532). CONCLUSION: The magnitude of inappropriate antibiotic prescription while managing cough and/or diarrhea in our setting was high. It needs urgent action to prevent emergence of antibiotic-resistant microorganisms.