Challenges and lessons learnt in the management of an HIV-exposed neonate with gastroschisis in a resource-limited setting: case report.

Introduction and importance: The incidence of congenital abdominal wall defects is increasing, but few cases have been reported in the African population. Case presentation: The authors report a case of gastroschisis in a term neonate who was delivered through spontaneous vaginal delivery (SVD) in a remote health facility before transfer to a tertiary hospital in Uganda. Although there was no environmental exposure to teratogens, the major risk factor of Gastroschisis, the neonate was low birth weight, HIV-exposed, and the mother had not received folic acid supplementation during the first trimester, known risk factors of gastroschisis. Physical examination revealed intrauterine growth restriction in addition to the findings of the abdominal wall defect. Clinical discussion: There were many missed opportunities in the management of this case which was marred by delayed essential care of the newborn, delayed surgical repair, and transfer to the tertiary surgical centre. At the tertiary surgical centre, a modified silo technique with delayed secondary closure was used to repair the defect, but the neonate still met its death before completing day 7 of life. Conclusion: This case of gastroschisis shows how the diagnosis and management of neonates born with major congenital structural abnormalities in resource-limited settings is still desirable due to lack of sophisticated medical care services to assist in early detection during pregnancy and early surgical intervention at birth to prevent associated mortality. The authors discuss the lessons learnt and provide recommendations for improvement in the care of neonates born with abdominal wall defects and other congenital birth defects.

Prevalence and Factors Associated with Iron Deficiency Anaemia among Children Aged 6-23 Months in Southwestern Uganda.

Iron deficiency anaemia is still a global public health concern with the highest burden among children 6 to 23 months due to their rapid growth spurt exceeding breastmilk supply. Therefore, nutritional supply is a key source of iron to attain the required nutrients for better growth and development. This was a cross-sectional descriptive study done at Ishaka Adventist Hospital (IAH) and Kampala International University Teaching Hospital (KIUTH) from April to July 2022. Participants were consecutively enrolled in the study. Structured questionnaires, 24-hour dietary recall, and clinical assessment were used to obtain data. Data analysis was done using the statistical package for social scientists (SPSS) V22.0. Bivariable and multivariable analyses were done using logistic regression for associations with significance set at P value < 0.05. A total of 364 participants were enrolled, with the majority being males (198, 54.4%) and born at term (333, 91.5%). The modal age was 12-17 months [163(44.8%)] with a mean age of 14.1 months (SD 5.32). The overall prevalence of IDA was 151/364 (41.5%). The factors associated with IDA included male sex (aOR 1.61), current episode of diarrhoea (aOR 1.71), poor meal frequency (aOR 1.78), no vegetable consumption (aOR 2.47), and consuming fruits once (aOR 1.97) in 7 days preceding the study. The study finds a high prevalence of IDA among infants 6-23 months with at least four in 10 being affected. Screening for IDA should be recommended in male children with current diarrhoea, poor intake of fruits and vegetables, and poor meal frequency. The Mentzer index is an equally good alternative screening test for IDA.

One year overall survival of wilms tumor cases and its predictors, among children diagnosed at a teaching hospital in South Western Uganda: a retrospective cohort study.

BACKGROUND: Wilms tumor (WT) is the second most common solid tumor in Africa with both low overall survival (OS) and event-free survival (EFS) rates. However, no known factors are predicting this poor overall survival. OBJECTIVE: The study was to determine the one-year overall survival of WT cases and its predictors among children diagnosed in the pediatric oncology and surgical units of Mbarara regional referral hospital (MRRH), western Uganda. METHODOLOGY: Children's treatment charts and files diagnosed and managed for WT were retrospectively followed up for the period between January 2017 to January 2021. Charts of children with histologically confirmed diagnoses were reviewed for demographics, clinical and histological characteristics, as well as treatment modalities. RESULTS: One-year overall survival was found to be 59.3% (95% CI: 40.7-73.3), with tumor size greater than 15 cm (p 0.021) and unfavorable WT type (p 0.012) being the predominant predictors. CONCLUSION: Overall survival (OS) of WT at MRRH was found to be 59.3%, and predictive factors noted were unfavorable histology and tumor size greater than 115 cm.

Sickle Cell Disease in Children and Adolescents: A Review of the Historical, Clinical, and Public Health Perspective of Sub-Saharan Africa and Beyond.

Sickle cell disease (SCD) is an umbrella term for a group of life-long debilitating autosomal recessive disorders that are caused by a single-point mutation (Glu→Val) that results in polymerization of hemoglobin (Hb) and reversible sickle-shape deformation of erythrocytes. This leads to increased hemolysis of erythrocytes and microvascular occlusion, ischemia-reperfusion injury, and tissue infarction, ultimately causing multisystem end-organ complications. Sickle cell anemia (HbSS) is the most common and most severe genotype of SCD, followed by HbSC, HbSß 0thalassemia, HbSß+thalassemia, and rare and benign genotypes. Clinical manifestations of SCD occur early in life, are variable, and are modified by several genetic and environmental factors. Nearly 500 children with SCD continue to die prematurely every day, due to delayed diagnosis and/or lack of access to comprehensive care in sub-Saharan Africa (SSA), a trend that needs to be urgently reversed. Despite proven efficacy in developed countries, newborn screening programs are not universal in SSA. This calls for a consolidated effort to make this possible, through the use of rapid, accurate, and cheap point-of-care test kits which require minimal training. For almost two decades, hydroxyurea (hydroxycarbamide), a century-old drug, was the only disease-modifying therapy approved by the U.S. Food and Drug Administration. Recently, the list expanded to L-glutamine, crizanlizumab, and voxelotor, with several promising novel therapies in the pipeline. Despite its several limitations, hematopoietic stem cell transplant (HSCT) remains the only curative intervention for SCD. Meanwhile, recent advances in gene therapy trials offer a glimpse of hope for the near future, although its use maybe limited to developed countries for several decades.

A neonate with Klippel-Trénaunay syndrome: a case report.

BACKGROUND: Klippel-Trénaunay syndrome is a rare congenital capillary-lymphatic-venous condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft-tissue hypertrophy. It has a very low incidence of about 1:100,000. CASE PRESENTATION: We report the case of 21-day-old neonate Black African female (born in Uganda) with Klippel-Trénaunay syndrome who presented with macrodactyly and ectrodactyly on the left foot, as well as numerous port wine stains on the left thoracoabdominal region and anteroposterior left lower limb. Color Doppler ultrasound examination of the left lower limb and abdomen revealed varicose veins without signs of arteriovenous fistula. CONCLUSION: The report presents the case of a neonate with a rare congenital vascular disorder type Klippel-Trénaunay syndrome.

Compartment syndrome following intramuscular self-injection of kerosene and rodenticide: A case report.

INTRODUCTION AND IMPORTANCE: Kerosene and rodenticides are used in many households in developing countries. This case report aims to discuss the progression and management of a patient with intentional kerosene and rodenticide poisoning. To our knowledge, this is the first documented case of blended kerosene-rodenticide poisoning in medical literature. CASE PRESENTATION: This report describes a 23-year-old man who survived after intramuscular self-injection of 5 ml of kerosene mixed with a rodenticide into his left upper limb, with intent to commit suicide. He was admitted to our hospital following a convulsion and brief loss of consciousness. Compartment syndrome developed within 24 h of admission, necessitating urgent fasciotomy, repeated surgical debridement, limb elevation, wound cleaning and dressing, in addition to intravenous fluids, antibiotics, and close observation. Blood transfusion, phytomenadione (vitamin K1), tetanus toxoid, and analgesics were recommended. The patient also received physiotherapy, and was treated for depression. The limb healed completely, with contractures at the left wrist joint. CLINICAL DISCUSSION: Injected kerosene and rodenticide may result in compartment syndrome and variable local and systemic complications which require multifaceted care and a prolonged follow-up period. CONCLUSION: Seemingly minor injuries at presentation may quickly progress into considerable complications such as compartment syndrome. It is imperative that physicians comprehensively investigate patients with poisoning for multiorgan dysfunction. Anticipation of local and systemic complications of injected poisons and timely medical and surgical intervention is life-saving.

Prevalence and associated factors of pneumonia among under-fives with acute respiratory symptoms: a cross sectional study at a Teaching Hospital in Bushenyi District, Western Uganda.

Objectives: This study assessed the prevalence and associated factors of pneumonia among children under-five years presenting with acute respiratory symptoms. Methodology: This was a cross sectional study at the Pediatric Department of Kampala International University - Teaching Hospital, from the month of April to August 2019. The study included 336 children aged 2 to 59 months presenting with acute respiratory symptoms to the pediatric clinic. Pneumonia diagnosis was made according to the World Health Organization definition, modified by a chest radiograph. Structured questionnaires were used to collect data on socio-demographic, environmental and nutrition factors and multivariate logistic regression analysis using STATA version 13.0 was done to assess for the factors independently associated with pneumonia. Results: Of the 336 children with acute respiratory symptoms, eighty-six, 86 (25.6%) had pneumonia. Factors significantly associated with pneumonia included: age below 6 months (OR=3.2, 95%CI=1.17-8.51, p=0.023), rural residence (OR=5.7, 95%CI=2.97-11.05, p <0.001), not up-to-date for age immunization status (OR=2.9, 95%CI=1.05-7.98, p=0.039), severe acute malnutrition (OR=10.8, 95%CI=2.01-58.41, p=0.006), lack of exclusive breastfeeding during the first six months (OR=2.9, 95%CI=1.53-5.53, p=0.001) and exposure to cigarette smoke (OR=3.0, 95%CI=1.35-6.80, p=0.007). Conclusion: The prevalence of pneumonia in children under-five years was high. Most of the factors associated with pneumonia are modifiable; addressing these factors could reduce this prevalence.