scholarly journals Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana

2017 ◽  
Vol 17 (1) ◽  
Author(s):  
Patrick Adu ◽  
Essel K. M. Bashirudeen ◽  
Florence Haruna ◽  
Edward Morkporkpor Adela ◽  
Richard K. D. Ephraim
2020 ◽  
Author(s):  
Fatima Zahra Alaoui ◽  
Achraf LAGHMICH ◽  
Naima GHAILANI NOUROUTI ◽  
Amina BARAKAT ◽  
Mohcine BENNANI MECHITA

Abstract Background: Hemoglobinopathies are the most frequent widely spread genetic disorders. In Morocco, epidemiological and clinical data are scarce. The present study aims to determine the spectrum and geographic distribution of hemoglobinopathies in Larache province, North of Morocco.Methods: A retrospective cross-sectional study was conducted from January 2015 to December 2018 at the provincial hospital of Larache city. All patients’ records having a hemoglobinopathy were analyzed. Background data (age, gender, and origin) of each case were analyzed. Results: Our study showed an overall frequency of hemoglobinopathies of 3.6%. Sickle cell trait was registered to be the most common disorder in our studied population (42.3%), followed by β-thalassemia Trait (20.2%), sickle cell disease (19.8%), major β-thalassemia (9.8%) and sickle/β-thalassemia (8%). The average age of subjects with hemoglobin disorders was 17.5 years. The majority of the patients (66.5%) were less than 20 years old. A disparity in the diseases geographic distribution was observed, 74.6% of patients came from rural areas. Conclusions: The present study is the first of its kind offering comprehensive data on hemoglobinopathies pattern in Northern Morocco. Our work may lay the foundation for screening programs for better prevention and coverage.


Author(s):  
Varsha Kose ◽  
Saurabh Kose

Background: Pregnancy in sickle cell women has numerous obstetrical, nonobstetrical and fetal complications. Our objective was to study the pregnancy outcome in women with sickle cell disease/trait.Methods: A descriptive cross sectional study in 2 years study period was conducted. 57 women with sickle cell disease/trait were identified. They were attending the antenatal clinic and were admitted in obstetric ward and followed till 7 days after delivery.Results: Out of total 57 women 49 women (85.96%) were sickle cell trait (AS pattern) and 8 women (14.03%) were sickle cell disease (SS pattern). In women with sickle cell trait Pregnancy induced hypertension was the most common complication. Anaemia and hypothyroidism was the common associated medical problem. 5 babies need NICU admission, 6 babies PBU admission and 33 babies given to mother side just after birth. In women with sickle cell disease severe anaemia, preeclampsia, oligohydramnios and intrauterine growth restriction, lower segment caesarean section for preterm baby was the most common complication. 1 baby required NICU admission, 5 babies require PBU admission and only 2 babies given to mother just after delivery. There were no maternal mortality and neonatal mortality.Conclusions: Maternal morbidity and neonatal morbidity is more in sickle cell disease women. They require early diagnosis, premarital and preconceptional counselling, good multidisciplinary obstetrics and neonatal care and early referral to higher centre.


Author(s):  
Chintan Gamit ◽  
MohamedAnas M. Patni ◽  
Sukesha Gamit

Background: India has the largest concentration of tribal populations globally. A high prevalence of sickle cell anemia is seen in tribal communities of South Gujarat. The aims and objectives were to study socio- demographic profile of the patients having positive sickle cell status.Methods: It was a cross-sectional study, where Bardoli taluka was selected purposively. All patients of sickle cell anemia registered at all PHCs of Bardoli Taluka during period between June 2010 and May 2011 & aged between 18 to 30 years were taken in study.Results: This study included total 276 cases of which 264 (96%) had sickle cell trait, while 12 (4%) had sickle cell disease. Majority of patients were from Umrakh and Vanskui PHC. More than 90% of patients belonged to ST caste and more than 40% among them were from Rathod sub caste.Conclusions: More than 90% of patients were laborers and majority of them belonged to low socio economic class. Mean age at diagnosis of sickle cell anemia ranged from 21 to 25 years. 


2016 ◽  
Vol 4 (3) ◽  
pp. e195-e200 ◽  
Author(s):  
Grace Ndeezi ◽  
Charles Kiyaga ◽  
Arielle G Hernandez ◽  
Deogratias Munube ◽  
Thad A Howard ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Patrick Adu ◽  
David Larbi Simpong ◽  
Godfred Takyi ◽  
Richard K. D. Ephraim

Background.Blood transfusion is a therapeutic procedure usually undertaken in patients with severe anaemia. In Ghana, severe anaemia is mostly due to malaria caused by severePlasmodium falciparuminfection, road traffic accidents, and haemoglobinopathy-induced acute haemolysis.Method.This cross-sectional study evaluated coinheritance of sickle cell haemoglobin variant and G6PD enzymopathy among individuals that donated blood at the Holy Trinity Hospital, Berekum, in the Brong-Ahafo Region, Ghana. Demographic data and other pertinent information were captured using questionnaire. Sickle cell haemoglobin variants were determined using cellulose acetate electrophoresis (pH 8.6). Qualitative G6PD status and quantitative G6PD enzyme activity were determined using methaemoglobin reduction and Trinity Biotech G6PD test kit, respectively.Results.Prevalence of sickle cell trait (SCT) and G6PD enzymopathy coinheritance was 7%. In addition, 19.5% of the donors had 10%–60% of normal G6PD enzyme activity suggesting that these donor units are prone to stressor-induced acute haemolysis when given to recipients. Mild G6PD activity (p=0.03, OR: 2.410 (CI: 1.049–5.534)), commercial (p=0.020, OR: 5.609 (CI: 1.309–24.035)), and voluntary (p=0.034, OR: 2.404 (CI: 1.071–5.397)) donors were significantly associated with SCT.Conclusion.Screening for red cell pathologies must be incorporated into existing protocols for populations with high incidence of haemoglobinopathies to protect high-risk recipients.


Author(s):  
Vijay Shah ◽  
Akash Patel ◽  
Praful Bambharoliya ◽  
Jigisha Patadia

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.


Author(s):  
Seham Fathy Khedr ◽  
Mohamed Hosny El Bradaey ◽  
Hala Mohamed Nagy ◽  
Mohamed Ramadan El-Shanshory ◽  
Eslam Elhawary

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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