Endocrine and Growth Disorders in Children with Haemoglobin-SS

Background: Suboptimal growth and certain metabolic disorders are commonly presented by children with Sickle Cell Disease (SCD). SCD is an autosomal recessive genetic condition common in regions with intense malaria prevalence. The cycles of de-oxygenation and oxygenation of redblood cells producing repeated sickling and unsickling, leading to red cell damage is a concern. There was need to establish the cause of common triggers for Vaso-Occlusive crises which include dehydration, infections, extreme temperature and emotional stress. Recurrent painful episodes, several physical and biochemical disorders including suboptimal growth, low immunity, anemia and a variety of serious organ system complications that cause life-long disabilities and/or early death in HbSS patients was a challenge. The highest frequencies (3 to 4% of populations) of Homozygous Sickle Cell disease occur in Sub-Saharan Africa. Methodology: Twenty eight children aged 4-10 years with Hemoglobin-SS (HbSS) and Vaso-Occlusive crisis attending Children Emergency Clinic at  Ladoke Akintola University of Technology Teaching Hospital and another 30 healthy sex and age matched children with HbAA (controls) participatedin this study. Plasma levels of Growth Hormone(GH), cortisol, prolactin, Total Thyroxin(TT4), Total Triiodotyronine(TT3), Thyroid Stimulating  Hormone(TSH) and insulin were determined in all respondents using enzyme linked immunosorbent assay methods. Results: The weight, height and BMI decreased significantly (p<0.05) in HbSS children compared with the controls. Plasma levels of GH, cortisol, TT3 and TT4 increased significantly (p<0.05) in HbSS-children compared with controls. Plasma levels of prolactin, TSH and insulin did not show significant (p>0.05) changes in the HbSS children compared with the controls. There was a significant (r=0.46, p=0.04) positive correlation between cortisol and GH in the children with HbSS. A negative correlation (r=-0.45, p=0.045) existed between TT4 and weight of HbSSchildren. Conclusion: The lower levels of height, weight and BMI despite increased plasma level of GH could suggest peripheral tissue resistance and/or GH-receptor deficiency in HbSS children. Elevated cortisol levels and the positive correlation between cortisol and GH could suggest a link between metabolic stress and GH secretion in HbSS Children. Key words: Hormones, growth disorders, Haemoglobin-SS(HbSS

Adaptation of the Wound Healing Questionnaire universal-reporter outcome measure for use in global surgery trials (TALON-1 study): mixed-methods study and Rasch analysis

BackgroundThe Bluebelle Wound Healing Questionnaire (WHQ) is a universal-reporter outcome measure developed in the UK for remote detection of surgical-site infection after abdominal surgery. This study aimed to explore cross-cultural equivalence, acceptability, and content validity of the WHQ for use across low- and middle-income countries, and to make recommendations for its adaptation.MethodsThis was a mixed-methods study within a trial (SWAT) embedded in an international randomized trial, conducted according to best practice guidelines, and co-produced with community and patient partners (TALON-1). Structured interviews and focus groups were used to gather data regarding cross-cultural, cross-contextual equivalence of the individual items and scale, and conduct a translatability assessment. Translation was completed into five languages in accordance with Mapi recommendations. Next, data from a prospective cohort (SWAT) were interpreted using Rasch analysis to explore scaling and measurement properties of the WHQ. Finally, qualitative and quantitative data were triangulated using a modified, exploratory, instrumental design model.ResultsIn the qualitative phase, 10 structured interviews and six focus groups took place with a total of 47 investigators across six countries. Themes related to comprehension, response mapping, retrieval, and judgement were identified with rich cross-cultural insights. In the quantitative phase, an exploratory Rasch model was fitted to data from 537 patients (369 excluding extremes). Owing to the number of extreme (floor) values, the overall level of power was low. The single WHQ scale satisfied tests of unidimensionality, indicating validity of the ordinal total WHQ score. There was significant overall model misfit of five items (5, 9, 14, 15, 16) and local dependency in 11 item pairs. The person separation index was estimated as 0.48 suggesting weak discrimination between classes, whereas Cronbach's α was high at 0.86. Triangulation of qualitative data with the Rasch analysis supported recommendations for cross-cultural adaptation of the WHQ items 1 (redness), 3 (clear fluid), 7 (deep wound opening), 10 (pain), 11 (fever), 15 (antibiotics), 16 (debridement), 18 (drainage), and 19 (reoperation). Changes to three item response categories (1, not at all; 2, a little; 3, a lot) were adopted for symptom items 1 to 10, and two categories (0, no; 1, yes) for item 11 (fever).ConclusionThis study made recommendations for cross-cultural adaptation of the WHQ for use in global surgical research and practice, using co-produced mixed-methods data from three continents. Translations are now available for implementation into remote wound assessment pathways