Sickle Cell Transplantation Evaluation of Long-term and Late Effects Registry (STELLAR) to Compare Long-term Outcomes After Hematopoietic Cell Transplantation to Those in Siblings Without Sickle Cell Disease and in Nontransplanted Individuals With Sickle Cell Disease: Design and Feasibility Study.

BACKGROUND: There are sparse data on the long-term and late effects of hematopoietic cell transplantation (HCT) for sickle cell disease (SCD). OBJECTIVE: This study aims to establish an international registry of long-term outcomes post-HCT for SCD and demonstrate the feasibility of recruitment at a single site in the United States. METHODS: The Sickle Cell Transplantation Evaluation of Long-Term and Late Effects Registry (STELLAR) was designed to enroll patients with SCD ≥1 year post-HCT, their siblings without SCD, and nontransplanted controls with SCD to collect web-based participant self-reports of health status and practices by using the Bone Marrow Transplant Survivor Study (BMTSS) surveys, health-related quality of life (HRQOL) using the Patient-Reported Outcomes Measurement Information System (PROMIS) Pediatric Profile-25 or Pediatric Profile-29 survey, chronic graft-versus-host disease (cGVHD) using the symptom scale survey, daily pain using an electronic pain diary, the economic impact of HCT using the financial hardship survey, sexual function using the PROMIS Sexual Function SexFSv2.0 survey, and economic productivity using the American Time Use Survey (ATUS). We also piloted retrieval of clinical data previously submitted to the Center for International Blood and Marrow Transplant Research (CIBMTR); recorded demographics, height, weight, blood pressure, waist and hip circumferences, timed up and go (TUG) test, and handgrip test; and obtained blood for metabolic screening, gonadal function, fertility potential, and biorepository of plasma, serum, RNA, and DNA. RESULTS: Of 100 eligible post-HCT patients, we enrolled 72 (72%) participants aged 9-38 (median 17) years. We also enrolled 19 siblings aged 5-32 (median 10) years and 28 nontransplanted controls with SCD aged 4-46 (median 22) years. Of the total 119 participants, 73 (61%) completed 85 sets of surveys and 41 (35%) contributed samples to the biorepository. We completed ATUS interviews of 28 (24%) participants. We successfully piloted retrieval of data submitted to the CIBMTR and expanded recruitment to multiple sites in the United States, Canada, the United Kingdom, and Nigeria. CONCLUSIONS: It is feasible to recruit subjects and conduct study procedures for STELLAR in order to determine the long-term and late effects of HCT for SCD. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/36780.

Knowledge, attitude and premarital screening practices for sickle cell disease among young unmarried adults in an urban community in Lagos, Nigeria.

Introduction: sickle cell disease (SCD) refers to a group of inherited blood disorders that are life-long and affect many people globally. An estimate of 2.3% of the Nigerian population suffer from SCD and about 25% of adults have the sickle cell gene. Premarital screening for sickle cell gene is considered one of the methods of preventing new births of children with SCD among the young adults. The study assessed the knowledge, attitude, willingness to take premarital screening test for SCD and factors influencing knowledge among young unmarried adults in an urban community in Lagos, Nigeria. Methods: the study was cross-sectional descriptive among 300 respondents who were selected using multistage sampling technique. Data were collected using a pre-tested, interviewer-administered questionnaire and analyzed using SPSS software, version 25. Univariate and bivariate analysis were conducted with level of significance at p ≤ 0.05. Results: the mean age of respondents was 21.2± 3.5 years, and most 188 (62.7%) were males. About 139 (46.3%) and 165 (55.0%) of the respondents respectively had good knowledge and positive attitude towards SCD and premarital screening. Only 43% of the respondents knew their haemoglobin phenotype, however, majority (92.4%) were willing to have Hb phenotype test done. Knowledge of SCD and premarital screening was statistically significant with age, level of education and occupation of respondents (p<0.001). Conclusion: this study found less than half of the respondents with good knowledge, about half had positive attitude and poor premarital screening practices of SCD. Therefore, community-based health education and awareness programs on SCD and premarital screening among young adults is recommended.

Prevalence and foetomaternal effects of iron deficiency anaemia among pregnant women in Lagos, Nigeria.

Anaemia in pregnancy is a major health problem and an important cause of adverse foetomaternal outcomes in developing countries. Iron deficiency is the cause of the overwhelming majority of the cases of anaemia in pregnancy. Iron deficiency anaemia (IDA) has been linked with adverse foetal and maternal outcomes. This study investigated the prevalence of IDA and evaluated its effects on foetomaternal outcomes among parturients in Lagos, Nigeria. This was a cross-sectional study that enrolled 220 women aged 15-49 years with singleton gestation at term, between May 1, 2016, and March 31, 2017. Participants were selected by systematic sampling and baseline data were collected through interviews. Venous blood samples were obtained to measure haemoglobin and serum ferritin concentrations, and the associations between IDA (defined as anaemia and iron deficiency) and pregnancy outcomes were examined. A P-value <0.05 was considered as statistically significant. The prevalence of IDA was 12.3%. Routine antenatal iron supplementation (adjusted odds ratio 0.18, 95% confidence interval 0.07-0.46; P = 0.001) and interpregnancy interval of at least 2 years (adjusted odds ratio 0.20, 95% confidence interval 0.05-0.97; P = 0.021) have significant association with IDA. Iron deficiency anaemia was not significantly associated with adverse perinatal outcomes but there were significant associations with increased risk of blood transfusion (P = 0.001) and maternal infectious morbidities such as puerperal pyrexia (P = 0.041) and wound infection (P = 0.020). IDA is still a fairly common condition among parturients in Lagos and it's mostly associated with maternal peripartum morbidities. Adequate pregnancy spacing through the use of effective contraception and routine antenatal iron supplementations in pregnancy is a recommended preventive measure against IDA and its adverse sequelae. Future studies should adopt the use of transferrin saturation (TSAT) in compliment with serum ferritin assay as a more sensitive marker of iron deficiency.

A Comparative Study of Serum Ferritin Levels Among Unfit and Fit Blood Donors.

BACKGROUND: Cheap methodologies are being utilized by low-resource countries to determine blood donors' fitness. Important hematological biomarkers might have to be evaluated to enhance the use of these methods. AIMS: The study evaluated the pattern of serum ferritin in 18-24 fit and unfit prospective blood donors (PDBs) and the prevalence of iron store deficiency. SETTINGS AND DESIGN: This study was a cross-sectional, comparative study which was conducted at the blood donor clinic of the Lagos University Teaching Hospital. MATERIALS AND METHODS: Blood samples were collected by venipuncture into sodium-ethylenediaminetetraacetic acid and plain bottles. The latter was centrifuged and used for ferritin determination via human ferritin enzyme-linked immunosorbent assay test kit, while the former was used for red cell indices analysis using an autoanalyzer. STATISTICAL ANALYSIS: Data were analyzed using SPSS version 20, values were presented as mean ± standard deviation, and P ≤ 0.05 was considered statistically significant. RESULTS: A total of 263 PDB were recruited into the study consisting of 210 (79%) males and 53 (21%) females, with a mean age of 32.88 ± 8.22. Only 110 (41.8%) of the participants were considered fit, while 153 (58.2%) were unfit using copper sulfate specific gravity. There was no statistically significant difference (P = 0.301) in the mean level of serum ferritin in unfit blood donors (74.5 ± 90.8 µg/L) compared to that of the fit blood donors (61.5 ± 54.5 µg/L). The prevalence of iron store depletion among blood donors in Lagos state was 11.8% (31 of 263) with a higher proportion (7.6%) occurring among unfit donors. However, low levels of serum ferritin (<15 µg/L) were significantly associated with the occurrence of anemia (hemoglobin < 12.5 g/gl) among unit donors (19%; P = 0.05). CONCLUSION: Although serum ferritin depletion appears to be higher in the unfit blood donors, the use of serum ferritin as an index for the screening and determination of PDBs' fitness requires further evaluation.

Thyroid, cortisol and growth hormone levels in adult Nigerians with metabolic syndrome.

INTRODUCTION: The similarities in presentation of cortisol excess, growth hormone deficiency, hypothyroidism and metabolic syndrome suggest that subtle abnormalities of these endocrine hormones may play a causal role in the development of metabolic syndrome. The aim of this study is to determine the levels of cortisol, thyroid and growth hormones in adult Nigerians with metabolic syndrome and determine the relationship between levels of these hormones and components of the syndrome. METHODS: This was a case control study conducted at the Lagos University Teaching Hospital, Lagos, Nigeria. Participants were fifty adult men and women with the metabolic syndrome, and fifty, age and sex matched males and females without the metabolic syndrome. Metabolic syndrome was defined based on the NCEP-ATPIII criteria. Written Informed consent was obtained from the participants. Socio demographic and clinical data were collected using a structured questionnaire. Venous blood was collected after an over-night fast. The Ethics committee of the Lagos University Teaching Hospital, Lagos, Nigeria, approved the study protocol. Comparison of continuous variables was done using the Student's t test. Correlation analysis was employed to determine the associations between variables. Statistical significance was set at P<0.05. RESULTS: Triiodotyronine (T3) was significantly decreased (p<0.001) and thyroxine (T4 ) significantly increased ( p<0.001) in metabolic syndrome compared to healthy controls. T3 correlated positively and significantly with waist circumference (p=0.004), glucose (p= 0.002), total cholesterol ( p=0.001) and LDL- cholesterol ( p<0.001 ) and negatively with body mass index ( p<0.001 )and triglyceride ( p=0.026). T4 had a negative significant correlation with waist circumference (p=0.004). Cortisol and growth hormone levels were similar in metabolic syndrome and controls. Cortisol however had a positive significant correlation with waist/hip ratio (p<0.001) while growth hormone correlated positively with HDL ( p=0.023)and negatively with diastolic blood pressure (p=0.049). CONCLUSION: Thyroid hormones T3 and T4 were associated with metabolic syndrome. The thyroid hormones, cortisol and growth hormones correlated with components of the syndrome. A therapeutic role may exit for these hormones in the management of metabolic syndrome and related disorders.