G156 Sickle Cell Disease in Malawian Children is Associated with Restrictive Spirometry: A Cross Sectional Survey

AbstractBackgroundSickle Cell Disease (SCD) affects two in 100 Cameroonian new-borns, with 50-90% of affected children dying before their fifth birthday. Despite this burden, there is no national SCD programme in Cameroon. This study aimed to assess parents’ and physicians’ knowledge of SCD, their satisfaction with the quality of care and their recommendations to improve the treatment of SCD in Cameroon.MethodsA multi-centre cross-sectional survey was conducted in English and French, using structured questionnaires distributed in electronic format to physicians throughout Cameroon. Paper-based questionnaires were also administered to parents in the West and North West regions of Cameroon. Data were entered into Microsoft Excel and analysed using the SPSS statistical software.ResultsFifty-four parents and 205 physicians were recruited. We found that 72.2% of parents had good knowledge of SCD, 72.2% of parents were satisfied with the quality of care. Attending a sickle cell clinic (AOR 22, 95% CI 17.70-250) was significantly associated with having good knowledge. Just 14.2% of physicians had good knowledge and 23.3% of physicians were satisfied with the available management standards of SCD. Seeing more than five patients per month (AOR 3.17, 95% CI 1.23-8.20) was significantly associated with having good knowledge. Sickle cell clinics, national guidelines and subsidised treatment were the top three measures proposed by physicians and parents to improve the management of SCD.ConclusionKnowledge of SCD and satisfaction with care were poor among Cameroonian physicians. There is a need for a national programme and a comprehensive system of care for SCD in Cameroon.

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Influence of haematological profile on the exercise capacity of children with sickle cell disease in Kano

Bayero Journal of Pure and Applied Sciences ◽

10.4314/bajopas.v13i2.10 ◽

2021 ◽

Vol 13 (2) ◽

pp. 79-83

Author(s):

O. Adeyinka ◽

U.M. Badaru ◽

J.M. Nuhu ◽

R.Y. Ahmad ◽

B. Bello ◽

...

Keyword(s):

Sickle Cell Disease ◽

Exercise Capacity ◽

Sickle Cell ◽

Negative Influence ◽

Full Blood Count ◽

Cell Disease ◽

Cross Sectional Survey ◽

Cross Sectional ◽

Haematological Profile ◽

Minute Walk

The Exercise Capacity (ExC) of children with sickle cell disease (SCD) may be influenced negatively by both haematological and environmental factors.This study aimed to assessthe influence of haematological profile on the ExC of children with SCD in Kano and to ascertain the safety of conducting 6 minute walk test (6MWT) on those children. In the cross-sectional survey, 162 children were recruited from Murtala Mohammed SpecialistHospital, Kano. Each of them walked to-and-fro for 6 minutes on a 10 meter marked level floor at their own walking pace in order to determine their actual 6 minute walk distance (6MWD). The actual 6MWD was compared with a predicted one in order to determine their ExC. Full blood count was used to evaluate haematological profiles. The data wereanalysed with Pearson product moment correlation and unpaired t test, at a level of significance of p<0.05 using SPSS version 20. Results showed that seventy (70) males (43.2%) and ninety two (92) females (56.8%) with mean age of 10.7±3.27 years took part in the study. The actual 6MWD was 366.20 m ± 59.88m (95%CI=356.91m - 375.49m) which was 59.17% of the predicted one. ExCcorrelated with each of White blood cell count (WBC)(r= - 0.22; p=0.005), Sex (r= - 0.27; p=0.001) and age (r=0.19; p=0.013). None of the participants experienced exercise-induced vaso-occlusive crisis during or immediately after the 6MWT.It was concluded that infection (signified by increased WBC count) and female gender have negative influence on ExC. 6MWT is safe to be performed by children with SCD.

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Men with sickle cell disease experience greater sexual dysfunction when compared with men without sickle cell disease

Blood Advances ◽

10.1182/bloodadvances.2020002062 ◽

2020 ◽

Vol 4 (14) ◽

pp. 3277-3283

Author(s):

Ibrahim M. Idris ◽

Akib Abba ◽

Jamil A. Galadanci ◽

Sharfuddeen A. Mashi ◽

Nafiu Hussaini ◽

...

Keyword(s):

Erectile Dysfunction ◽

Sexual Dysfunction ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Attention ◽

Cell Disease ◽

Cross Sectional Survey ◽

Cross Sectional ◽

Ischemic Priapism ◽

Stuttering Priapism

Abstract Recurrent ischemic priapism is a common complication of sickle cell disease (SCD). We assessed the burden, characteristics, and types of priapism, including sexual dysfunction, in a cohort of men with and those without SCD, to test the hypothesis that sexual dysfunction is more prevalent in men with SCD. In Kano, Nigeria, we conducted a comparative cross-sectional survey that included 500 and 250 men 18 to 40 years of age, with and without SCD, respectively. The survey used the Priapism Questionnaire and the International Index of Erectile Function for sexual function assessment. All eligible participants approached for the study gave informed consent and were enrolled. Stuttering and major priapism were defined based on the average duration of priapism experiences that lasted ≤4 and >4 hours, respectively. The prevalence of priapism was significantly higher in men with SCD than in those without it (32.6% vs 2%; P < .001). Stuttering priapism accounted for 73.6% of the priapism episodes in men with SCD. Nearly 50% of the participants with SCD-related priapism had never sought medical attention for this complication. The majority of the men with SCD-related priapism used exercise as a coping mechanism. Priapism affected the self-image of the men with SCD, causing sadness, embarrassment, and fear. The percentage of the men with SCD who had erectile dysfunction was more than twofold higher than that of those without SCD who had erectile dysfunction (P = .01). The men with SCD had a higher prevalence of priapism and sexual dysfunction than the men without SCD.

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Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

Journal of Lipids ◽

10.1155/2016/7650530 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 5

Author(s):

Richard K. D. Ephraim ◽

Patrick Adu ◽

Edem Ake ◽

Hope Agbodzakey ◽

Prince Adoba ◽

...

Keyword(s):

Blood Pressure ◽

Steady State ◽

Sickle Cell Disease ◽

Total Cholesterol ◽

Sickle Cell ◽

Density Lipoprotein ◽

Hdl Cholesterol ◽

Lipoprotein Cholesterol ◽

Cell Disease ◽

Cross Sectional

Background.Abnormal lipid homeostasis in sickle cell disease (SCD) is characterized by defects in plasma and erythrocyte lipids and may increase the risk of cardiovascular disease. This study assessed the lipid profile and non-HDL cholesterol level of SCD patients.Methods.A hospital-based cross-sectional study was conducted in 50 SCD patients, in the steady state, aged 8–28 years, attending the SCD clinic, and 50 healthy volunteers between the ages of 8–38 years. Serum lipids were determined by enzymatic methods and non-HDL cholesterol calculated by this formula: non-HDL-C = TC-HDL-C.Results.Total cholesterol (TC) (p=0.001) and high-density lipoprotein cholesterol (HDL-C) (p<0.0001) were significantly decreased in cases compared to controls. The levels of non-HDL-C, low-density lipoprotein cholesterol (LDL-C), and triglyceride (TG) were similar among the participants. The levels of decrease in TC and HDL were associated with whether a patient was SCD-SS or SCD-SC. Systolic blood pressure and diastolic blood pressure were each significantly associated with increased VLDL [SBP,p=0.01, OR: 0.74 (CI: 0.6–0.93); DBP,p=0.023, OR: 1.45 (CI: 1.05–2.0)].Conclusion.Dyslipidemia is common among participants in this study. It was more pronounced in the SCD-SS than in SCD-SC. This dyslipidemia was associated with high VLDL as well as increased SBP and DBP.

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Reduction of the Six-Minute Walk Distance in Children with Sickle Cell Disease Is Correlated with Silent Infarct: Results from a Cross-Sectional Evaluation in a Single Center in Belgium

PLoS ONE ◽

10.1371/journal.pone.0108922 ◽

2014 ◽

Vol 9 (10) ◽

pp. e108922 ◽

Cited By ~ 7

Author(s):

Laurence Dedeken ◽

Rudy Chapusette ◽

Phu Quoc Lê ◽

Catherine Heijmans ◽

Christine Devalck ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Single Center ◽

Cell Disease ◽

Walk Distance ◽

Cross Sectional ◽

Minute Walk Distance ◽

Minute Walk

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The Practices of Anaesthesiologists in the Management of Patients with Sickle Cell Disease: Empirical Evidence from Cameroon

Healthcare ◽

10.3390/healthcare9121617 ◽

2021 ◽

Vol 9 (12) ◽

pp. 1617

Author(s):

Dominique Djomo Tamchom ◽

Aristide Kuitchet ◽

Raymond Ndikontar ◽

Serge Nga Nomo ◽

Hermine Fouda ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Perioperative Management ◽

Treatment Guideline ◽

Binary Logistic Regression ◽

Cross Sectional Study ◽

Cell Disease ◽

Binary Logistic Regression Model ◽

Cross Sectional ◽

Elective Surgical Procedures

Patients with sickle cell disease are more likely to undergo surgery during their lifetime, especially given the numerous complications they may develop. There is a paucity of data concerning the management of patients with sickle cell disease by anaesthesiologists, especially in Africa. This study aimed to describe the practices of anaesthesiologists in Cameroon concerning the perioperative management of patients with sickle cell disease. A cross-sectional study was carried out over four months and involved 35 out 47 anaesthesiologists working in hospitals across the country, who were invited to fill a data collection form after giving their informed consent. The data were analysed using descriptive statistics and a binary logistic regression model. Among the 35 anaesthesiologists included in the study, most (29 (82.9%)) had managed patients with sickle cell disease for both emergency and elective surgical procedures. Most of them had never asked for a haematology consultation before surgery. Most participants (26 (74.3%)) admitted to having carried out simple blood transfusions, while 4 (11.4%) carried out exchange transfusions. The haemoglobin thresholds for transfusion varied from one practitioner to another, between < 6 g/dl and < 9 g/dl. Only 6 (17.1%) anaesthesiologists had a treatment guideline for the management of patients with sickle cell disease in the hospitals where they practiced. Only 9 (25.7%) prescribed a search for irregular agglutinins. The percentage of haemoglobin S before surgery was always available for 5 (14.3%) of the participants. The coefficient (0.06) of the occurrence of a haematology consultation before surgery had a significant influence on the probability of management of post-operative complications (coefficient 0.06, 10% level of probability). This study highlights the fact that practices in the perioperative management of patients with sickle cell disease in Cameroon vary greatly from one anaesthesiologist to another. We disclosed major differences in the current recommendations, which support the fact that even in Sub-Saharan countries, guidelines applicable to the local settings should be published.

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Sexual Maturity in Adolescents Suffering from Sickle Cell Disease: A Cross-sectional Study

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2021/47287.14981 ◽

2021 ◽

Author(s):

Vijay Shah ◽

Akash Patel ◽

Praful Bambharoliya ◽

Jigisha Patadia

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Sexual Maturity ◽

Tanner Stage ◽

Cross Sectional Study ◽

Reproductive Capacity ◽

Sectional Study ◽

Cell Disease ◽

Cross Sectional ◽

The Mean

Introduction: Sickle Cell Disease (SCD) is an inherited chronic haemolytic anaemia. The diseased person suffers from various complications such as anaemia, frequent infection, fever, hand-foot syndrome, stroke, etc. Puberty changes includes the appearance of the secondary sexual characteristics, increase in height, change in body composition and development of reproductive capacity. Aim: To study the sexual maturity and effect of multiple blood transfusions in adolescents suffering from SCD. Materials and Methods: It was a cross-sectional study conducted on 35 adolescents of age group 11 to 15 years, suffering from SCD. Study was conducted over a period of six months from March 2018 to September 2018 at Department of Paediatrics. SCD was diagnosed by Haemoglobin (Hb) electrophoresis. Weight and height were measured of all the participants. For assessing the sexual maturity, Tanners staging was used. Unpaired t-test was done for data analysis. Results: The mean age of the patients was 13.03±1.7 years. There were 25 males and 10 females. The mean age of male patients between Tanner stage 2(14.63±0.52 years) and Tanner stage 3 (14.75±0.5 years) was significantly higher than the Indian data for males (11.3 and 12.8 years, respectively). The mean age of female patients between Tanner stages 2 (13.5±2.12 years) and Tanner stage 3 (14.33±1.16 years) was higher than the Indian reference data for girls (10.2 and 11.6 years respectively). Conclusion: This study concluded that adolescents with SCD were significantly shorter in height and weight than the standard reference population. Sexual maturity is delayed in adolescents with sickle cell anaemia.

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Asymmetric Dimethyl Arginine Level in Children with Sickle Cell Disease: A Cross Sectional Study

Journal of Advances in Medicine and Medical Research ◽

10.9734/jammr/2021/v33i1831059 ◽

2021 ◽

pp. 93-98

Author(s):

Seham Fathy Khedr ◽

Mohamed Hosny El Bradaey ◽

Hala Mohamed Nagy ◽

Mohamed Ramadan El-Shanshory ◽

Eslam Elhawary

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Cross Sectional Study ◽

Sectional Study ◽

Cell Disease ◽

Adma Level ◽

Cross Sectional ◽

Asymmetric Dimethyl Arginine ◽

Dimethyl Arginine

Background: Sickle cell disease (SCD) consists of a group of hemoglobinopathies in which individuals inherit hemoglobin variants derived from single point mutations. Asymmetric dimethylarginine (ADMA) contributes to limiting Nitric Oxide (NO) bioavailability in SCD. The aim of the present study was to assess the level of the Asymmetric Dimethyl Arginine in children with sickle cell. Methods: This cohort cross-sectional study was carried out on 60 children which were divided in to 3 equal groups. Group I: SCD children with sickle retinopathy. Group II: SCD children without retinopathy. Group III: healthy control children who were selected from the outpatient clinic. Results: There was a significant increase in ADMA level among participants withSCD. There was a positive significant correlation between ADMA level and family history as well as the incidence of hepatomegaly. There was no significant correlation between ADMA level and demographic and laboratory parameters except LDH. Conclusions: The level of ADMA is elevated in children with sickle cell anemia. High plasma ADMA level is a risk for hepatomegaly in children with sickle cell anemia.

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