SATISFACTION AND ADHERENCE TO SICKLE CELL DISEASE TREATMENT IN BRAZIL: CROSS-SECTIONAL ANALYSIS CONSIDERING PATIENTS AND HEALTHCARE PROVIDER PERSPECTIVES

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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Cross-Sectional analysis of neurocognitive function, retinopathy, and retinal thinning by Spectral-Domain optical coherence tomography in sickle cell patients

Middle East African Journal of Ophthalmology ◽

10.4103/0974-9233.150632 ◽

2016 ◽

Vol 23 (1) ◽

pp. 79 ◽

Cited By ~ 4

Author(s):

HeatherE Moss ◽

EricaZ Oltra ◽

ClementC Chow ◽

Thomas Wubben ◽

JenniferI Lim ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Sickle Cell ◽

Neurocognitive Function ◽

Spectral Domain ◽

Optical Coherence ◽

Cross Sectional ◽

Cross Sectional Analysis ◽

Retinal Thinning ◽

Sectional Analysis

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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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