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, HbSβ0thalassemia, HbSβ+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.

Germinal matrix-intraventricular hemorrhage (GM-IVH) is a common intracranial complication in preterm infants, especially those born before 32 weeks of gestation and very-low-birth-weight infants. Hemorrhage originates in the fragile capillary network of the subependymal germinal matrix of the developing brain and may disrupt the ependymal lining and progress into the lateral cerebral ventricle. GM-IVH is associated with increased mortality and abnormal neurodevelopmental outcomes such as posthemorrhagic hydrocephalus, cerebral palsy, epilepsy, severe cognitive impairment, and visual and hearing impairment. Most affected neonates are asymptomatic, and thus, diagnosis is usually made using real-time transfontanellar ultrasound. The present review provides a synopsis of the pathogenesis, grading, incidence, risk factors, and diagnosis of GM-IVH in preterm neonates. We explore brief literature related to outcomes, management interventions, and pharmacological and nonpharmacological prevention strategies for GM-IVH and posthemorrhagic hydrocephalus.

Background HIV-exposed children are vulnerable to undernutrition. Understanding the prevalence and factors with undernutrition remains essential for developing effective strategies to reduce the impact of malnutrition among this vulnerable population. Methods This was a cross sectional, descriptive and analytical study to determine the prevalence, feeding practices, and factors associated with undernutrition among HIV-exposed uninfected children aged 6 to 18 months in Bushenyi District. The study involved 245 mother-child pairs attending four highly active antiretroviral treatment (HAART) clinics. Data was collected from April to June 2021. Questionnaire interviews were used to obtain factors associated with undernutrition. A digital weighing scale, stadiometer and mid-upper-arm circumference (MUAC) tape were used for anthropometry. Undernutrition was defined by either a length-for-age or a weight-for-length or a weight-for-age below − 2 SD based on the 2006 World Health Organization growth standards. Data were entered and analysed using IBM SPSS 27.0 statistics for windows. The prevalence of undernutrition and indicators for feeding practices were determined as proportion, whereas binary logistic regression was used to determine factors associated with undernutrition. A p-value < 0.05 was considered for statistical significance at multivariable level. Results Of the 245 participants, 48(19.6%) had undernutrition. The prevalence of stunting, wasting and underweight was 11%, 5.3% and 6.5% respectively. Most study participants 176(71.8%) were initiated on breastfeeding during their first hour of life, and 185(75.5%) were exclusively breastfed for 6 months. However, 93(38%) of them did not receive an acceptable meal frequency and 188 (76.7%) did not have an acceptable dietary diversity score during the last 24 hours. Only 15(6.1%) continued breastfeeding after 1 year of age. The majority of mothers 162(66.1%) were advised on feeding practices. Low birthweight [p = 0,03; aOR 2.94(1.23–7.03)], history of illness in the past two weeks [p = 0,02; aOR 2.35(1.13–4.91)], maternal viral load of ≥ 1000 copies/µL [p = 0,03; aOR 3.12(1.10–8.89)], maternal undernutrition [p = 0,003; aOR 4.05(1.61–10.20)], and mixed feeding [p = 0,04; aOR 1.35(0.06–2.73)] were factors significantly associated with undernutrition. Conclusion The prevalence of undernutrition among HIV-exposed uninfected children was relatively high in this study. It demonstrates the need for routine nutritional assessment of HIV-exposed uninfected children.