scholarly journals Evaluation of Microchip-Based Point-Of-Care Device “Gazelle” for Diagnosis of Sickle Cell Disease in India

2021 ◽  
Vol 8 ◽  
Author(s):  
Shweta Shrivas ◽  
Madhav Patel ◽  
Rajat Kumar ◽  
Anil Gwal ◽  
Ramswaroop Uikey ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Cellulose Acetate ◽  
Sickle Cell ◽  
High Accuracy ◽  
Diagnostic Methods ◽  
Diagnostic Tools ◽  
Cell Disease ◽  
Cellulose Acetate Electrophoresis ◽  
Indian States ◽  
Trained Manpower

Sickle cell disease is a major public health problem in India. Lack of rapid and reliable diagnostic methods result in many avoidable deaths in affected population. Current diagnostic tools are laboratory based, expensive and need trained manpower. Here, we evaluated the performance of a microchip-based cellulose acetate electrophoresis test, “Gazelle” in the tribal-dominated Indian states of Chhattisgarh and Madhya Pradesh. A total of 1,050 patients were screened by sickle cell solubility, hemoglobin (cellulose acetate) electrophoresis, high-performance liquid chromatography (HPLC) and Gazelle. Of the total 1,027 test results obtained, 960 tests were “Valid” (93.5%) and included in the analysis. Gazelle identified all patients with disease (HbSS and Thalassemia Major) with 100% accuracy. Gazelle demonstrated 100% sensitivity when comparing sickle cell disease (SCD) vs. sickle cell trait and SCD vs. normal. Specificity was 98.9% and 99.5% when comparing SCD vs. trait and trait vs. normal, respectively. Specificity was 99.8% when comparing SCD vs. normal and sensitivity was 99.3% when comparing trait vs. normal. Overall, Gazelle yielded a high accuracy (99.0%) compared to reference standard tests (hemoglobin electrophoresis and HPLC). Gazelle is a low-cost, rapid diagnostic test with high accuracy for detecting SCD both quantitatively and qualitatively. Gazelle can be a potential screening tool for the rapid diagnosis in resource limited settings and developing countries with high burden of hemoglobin disorders.

scholarly journals Prevalence of Sickle Cell Disease and Other Haemoglobin Variants in Calabar, Cross River State, Nigeria

2019 ◽  
pp. 1-6
Author(s):  
Akaba Kingsley ◽  
Ofem Enang ◽  
Ofonime Essien ◽  
Annette Legogie ◽  
Omini Cletus ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Genetic Disorder ◽  
Globin Gene ◽  
Specific Gene ◽  
Cell Disease ◽  
Cellulose Acetate Electrophoresis ◽  
Genetic Changes ◽  
Female Ratio ◽  
Cross River State

Background: Sickle cell disease (SCD) is the commonest genetic disorder worldwide with a global prevalence of 20-25 million. About 12-15 million affected persons are in Sub-Sahara Africa with Nigeria bearing the highest burden of people living with sickle cell disease. SCD is a disease characterized as an autosomal, recessive, heterogeneous, and a monogenetic disorder caused by an A-to-T point mutation in the β-globin gene responsible for the production of abnormal hemoglobin S (HbS), which polymerizes in the deoxygenated state and results in the sickling of erythrocytes.  Haemoglobin variants are mutant forms of haemoglobin in a population usually occurring as a result of genetic changes in specific genes, or globins that causes change on alterations in the amino acid. They could affect the structure, behavior, the production rate and the stability of the specific gene. Well-known haemoglobin variants such as sick-cell anaemia are responsible for diseases and are considered haemoglobinopathies. Other variants cause no detectable pathology and are thus considered as non-pathological variants. Aim: The study is aimed at evaluating the burden of sickle cell disease and other haemoglobin variants in Calabar, South-South Nigeria. Methods: This is a retrospective study done at the haematology laboratory of University of Calabar Teaching Hospital, Calabar. Cellulose acetate electrophoresis at alkaline pH was used for the evaluation of haemoglobinopathies. The data were entered into Microsoft Excel 2016 spreadsheet and analysed with the IBM SPSS Version 22. Data were summarized into percentage of different phenotypes. Results: Results of the total 3648 haemoglobin electrophoresis recorded, 1368 (37.50%) were male while the remaining 2280 (62.5%) females given a male to female ratio of 1:1.7. Five haemoglobin phenotypes were identified as HbAA, HbAS, HbAC, HbSC and HbSS. The overall average values of their prevalence were HbAA 64.78%, HbAS 32.62%, HbSS 2.14%, HbAC 0.33%, HbSC 0.14%. Thus, the prevalence of SCD (Prevalence of HbSS+HbSC) was 2.28%. The highest proportion of SCD was observed in 2011 with least in 2016 and 2017 respectively. Conclusion: The prevalence of SCD and other haemoglobin variants in Calabar is similar to that of the national prevalence rate. There is need for continuous enlightenment and premarital counselling on the pattern of inheritance of SCD most especially with the increased burden of sickle traits in the environment has reported in this study.

scholarly journals Smartphone-based Sickle Cell Disease Detection and Monitoring for Point-of-Care Settings

2020 ◽  
Author(s):  
Shazia Ilyas ◽  
Mazhar Sher ◽  
E Du ◽  
Waseem Asghar
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Early Stage ◽  
Point Of Care ◽  
Unmet Need ◽  
Optical Microscope ◽  
Diagnostic Methods ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Resource Limited

AbstractSickle cell disease (SCD) is a worldwide hematological disorder causing painful episodes, anemia, organ damage, stroke, and even deaths. It is more common in sub-Saharan Africa and other resource-limited countries. Conventional laboratory-based diagnostic methods for SCD are time-consuming, complex, and cannot be performed at point-of-care (POC) and home settings. Optical microscope-based classification and counting demands a significant amount of time, extensive setup, and cost along with the skilled human labor to distinguish the normal red blood cells (RBCs) from sickled cells. There is an unmet need to develop a POC and home-based test to diagnose and monitor SCD and reduce mortality in resource-limited settings. An early-stage and timely diagnosis of SCD can help in the effective management of the disease. In this article, we utilized a smartphone-based image acquisition method for capturing RBC images from the SCD patients in normoxia and hypoxia conditions. A computer algorithm is developed to differentiate RBCs from the patient’s blood before and after cell sickling. Using the developed smartphone-based technique, we obtained similar percentage of sickle cells in blood samples as analyzed by conventional method (standard microscope). The developed method of testing demonstrates the potential utility of the smartphone-based test for reducing the overall cost of screening and management for SCD, thus increasing the practicality of smartphone-based screening technique for SCD in low-resource settings. Our setup does not require any special storage requirements and is particularly useful in assessing the severity of the SCD. This is the characteristic advantage of our technique as compared to other hemoglobin-based POC diagnostic techniques.

scholarly journals Sickle cell disease in anaemic children in a Sierra Leonean district hospital: a case series

2019 ◽  
Vol 2019 (7) ◽  
Author(s):  
Maria B Italia ◽  
Sandy Kirolos
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
District Hospital ◽  
Case Series ◽  
Severe Anaemia ◽  
Sub Saharan Africa ◽  
Diagnostic Tools ◽  
Cell Disease ◽  
Sierra Leonean ◽  
Sub Saharan

Abstract Sickle cell disease (SCD) is the most common inherited haemoglobinopathy wordwide, with the highest prevalence in sub-Saharan Africa. Due to the lack of national strategies and scarcity of diagnostic tools in resource-limited settings, the disease may be significantly underdiagnosed. We carried out a 6-month retrospective review of paediatric admissions in a district hospital in northern Sierra Leone. Our aim was to identify patients with severe anaemia, defined as Hb < 7 g/dl, and further analyse the records of those tested for SCD. Of the 273 patients identified, only 24.5% had had an Emmel test, among which 34.3% were positive. Furthermore, only 17% of patients with a positive Emmel test were discharged on prophylactic antibiotics. Our study shows that increased awareness of SCD symptoms is required in high-burden areas without established screening programmes. In addition, the creation or strengthening of follow-up programmes for SCD patients is essential for disease control.

scholarly journals Establishing Sickle Cell Diagnostics and Characterizing a Pediatric Sickle Cell Disease Cohort in Malawi

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2070-2070
Author(s):  
Jonathan Brett Heimlich ◽  
Godwin Chipoka ◽  
Portia Kamthunzi ◽  
Yuri D. Fedoriw ◽  
Nigel S. Key ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Joint Pain ◽  
Clinical Care ◽  
Diagnostic Methods ◽  
Sub Saharan Africa ◽  
Age Group ◽  
Cell Disease ◽  
Sub Saharan

Abstract Sickle cell disease (SCD) is highly prevalent in sub-Saharan Africa; however, there are relatively few studies describing the clinical profile for children with laboratory-confirmed SCD. Prior to December 2014, neither neonatal screening nor standardized methods for SCD diagnosis were routinely available in Malawi, as hemoglobin electrophoresis and alternative diagnostic methods were absent. We describe implementation of hemoglobin electrophoresis for children with clinically suspected SCD at Kamuzu Central Hospital, one of two national teaching hospitals in Malawi. Children with clinically suspected SCD were recruited January - May 2015 and underwent comprehensive clinical and laboratory characterization. 137 total patients were recruited and 117 were confirmed to have HbSS disease. Among children who were being cared for as SCD prior to enrollment, 86% had HbSS suggesting generally accurate clinical diagnosis by local providers. Baseline clinical parameters and self-reported SCD complications for the study population are displayed in Table 1. Of those with confirmed SCD, median age was 7.3 years (IQR 2.7-10.4) with 53% males. Prior malaria was reported by 39% of patients, and was higher in the 0-5 age group compared with the over 5 age group (46% vs. 31%, p=0.03). The most commonly reported SCD complications were anemia (72%), joint pain (56%), jaundice (52%), and acute pain episodes (50%). Children with confirmed SCD had median hemoglobin of 7.3 g/dL (IQR 6.9-7.9), total bilirubin of 1.7 mg/dL (IQR 1.1-2.6) and lactate dehydrogenase of 658 IU/L (IQR 527-773). Urinalysis demonstrated 26% of patients with blood and 7% with proteinuria by dipstick. As of May 2015, more than 250 samples for enrolled children as well as routine clinical care had been batch-processed weekly with an average turn-around time of 36 hours for results. Three Malawian laboratory technicians were trained to perform hemoglobin electrophoresis, all of whom have been performing the test independently since April 2015. Our findings highlight a need for wider implementation of resource-appropriate diagnostics as an essential foundation for care and research. Children had substantial clinical and laboratory evidence of SCD-related morbidity. Earlier diagnosis can improve care for this population by facilitating earlier therapeutic interventions, as well as providing a basis for research to better understand SCD-related morbidity in sub-Saharan Africa. These efforts can ultimately inform management strategies to improve outcomes and increase life expectancy among children with SCD in Malawi. Table 1. All (n=117) Male (n= 62) Female (n=55) p value Age years, median (IQR) 7.3 (2.7-10.4) 5.3 (2.3-9.4) 8.9 (4.2-11.9) 0.004 Height cm, median (IQR, n) 115 (88-131, 60) 111 (89-128, 36) 119.5 (93-140, 24) 0.21 Weight kg, median (IQR, n) 19 (13-27, 108) 16.5 (12-23.6, 58) 21 (14-30, 50) 0.01 Blood Pressure Systolic mmHg, median (IQR, n) 103 (98-110, 83) 101 (94-108, 43) 103 (99-110, 40) 0.37 Blood Pressure Diastolic mmHg, median (IQR, n) 60 (55-65, 83) 58 (53-65, 43) 61 (56-68, 40) 0.13 Heart Rate BPM, median (IQR, n) 104 (91-118, 114) 105 (94-123, 61) 104 (88-112, 53) 0.15 O2 Saturation %, median (IQR, n) 93 (88-97, 108) 91 (85-96, 59) 95 (91-98, 49) 0.004 % Hypoxemic (SPO2 < 90%), n (%) 36 (30.7) 26 (41.9) 10 (18.2) 0.005 Body Temperature Celsius, median (IQR, n) 37 (36.7-37.4, 91) 37 (36.7-37, 46) 37 (36.4-37.2, 45) 0.22 Positive History of: Malaria, n (%) 45 (38.5) 22 23 0.34 0-5 years, n (%) 25 (46.3) - - 0.03 6-18 years, n (%) 20 (31.7) - - Pneumonia, n (%) 29 (24.8) 10 (16.1) 19 (34.5) 0.02 HIV, n (%) 0 0 0 - Anemia, n (%) 84 (71.8) 49 (79.0) 35 (63.6) 0.06 Pallor, n (%) 16 (13.7) 7 (11.3) 9 (16.4) 0.43 Jaundice, n (%) 61 (52.1) 33 (53.2) 28 (50.9) 0.82 Received Blood Transfusion, n (%) 87 (74.4) 47 (75.8) 40 (72.7) 0.47 Days since last transfusion, median (IQR) 316 (133-1144) 240 (111-410) 577 (180-1784) 0.03 Pain episodes, n (%) 58 (49.6) 27 (43.5) 31 (56.4) 0.16 Joint pain, n (%) 66 (56.4) 33 (53.2) 33 (60.0) 0.34 Dactylitis, n (%) 41 (35.0) 19 (30.6) 22 (40.0) 0.29 Leg ulcers, n (%) 5 (4.3) 5 (8.1) 0 0.03 Stroke, n (%) 10 (8.5) 5 (8.1) 5 (9.1) 0.84 Nocturnal Enuresis, n (%) 24 (20.5) 12 (19.4) 12 (21.8) 0.74 Disclosures No relevant conflicts of interest to declare.

scholarly journals Neurologic complications of sickle cell disease in Africa

Neurology ◽  
2017 ◽  
Vol 89 (14) ◽  
pp. 1516-1524 ◽  
Author(s):  
Jean Jacques Noubiap ◽  
Michel K. Mengnjo ◽  
Nicolas Nicastro ◽  
Joseph Kamtchum-Tatuene
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Providers ◽  
Diagnostic Tools ◽  
Screening Methods ◽  
Neurologic Complications ◽  
Care Providers ◽  
Neurocognitive Deficits ◽  
Cell Disease ◽  
Methodologic Quality

Objective:To summarize prevalence data on the neurologic complications of sickle cell disease (SCD) in Africa.Methods:We searched EMBASE, PubMed, and African Index Medicus to identify all relevant articles published from inception to May 31, 2016. Each study was reviewed for methodologic quality. A random-effects model was used to estimate the prevalence of neurologic complications of SCD across studies.Results:Thirty-one studies were included. Methodologic quality was high or moderate in 90% of studies. Stroke, conditional and abnormal cerebral blood flow, seizures, and headache were the complications most frequently reported, with overall prevalence rates of 4.2%, 10.6%, 6.1%, 4.4%, and 18.9%, respectively. Some complications, like silent brain infarcts, peripheral neuropathies, neurocognitive deficits, or moyamoya disease, have been rarely or not studied at all in the African setting. Incidence data were scarce and of poor quality.Conclusions:The burden of neurologic complications of SCD is important in Africa and most likely underestimated. A better evaluation of this burden requires larger prospective studies using standard up-to-date screening methods. Accessibility to diagnostic tools such as neuroimaging, transcranial Doppler, EEG, and neuropsychological evaluation, as well as to preventive and therapeutic interventions and trained health care providers, should be improved in routine clinical practice.

Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review

2017 ◽  
Vol 34 (10) ◽  
pp. 797-804 ◽  
Author(s):  
Keneisha Bailey ◽  
Jagila Wesley ◽  
Adebayo Adeyinka ◽  
Louisdon Pierre
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
Fat Embolism ◽  
Diagnostic Tools ◽  
Acute Chest Syndrome ◽  
Extensive Literature ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours. We postulate that FES is not well recognized in SCD and that FES scores are useful diagnostic tools in patients with SCD. We queried the electronic medical records with the diagnostic codes for SCD with acute chest syndrome (ACS), pulmonary embolism, or acute respiratory distress syndrome admitted to our hospital from 2008 to 2016 to identify patients suspected of having FES. In addition, we performed an extensive literature review to evaluate the management practice of pediatric patients with FES and SCD from 1966 to 2016. Six patients met our selection criteria from the hospital records, and 4 case reports from the literature search were also included. We applied the Gurd and Wilson criteria and the Schonfeld Fat Embolism Index to identify patients who met the criteria for FES. Nine patients fulfilled Gurd and Wilson criteria, and 9 patients who were evaluable met the Schonfeld criteria for FES. A rapidly deteriorating clinical course in a patient with SCD presenting with ACS or severe vaso-occlusive crisis should trigger a high index of suspicion for FES. Gurd and Wilson criteria or the Schonfeld Fat Embolism Index are useful diagnostic tools for FES in SCD.

scholarly journals Characterising demographics, knowledge, practices and clinical care among patients attending sickle cell disease clinics in Eastern Uganda

2020 ◽  
Vol 5 ◽  
pp. 87
Author(s):  
Peter Olupot-Olupot ◽  
Ham Wabwire ◽  
Carolyne Ndila ◽  
Ruth Adong ◽  
Linus Ochen ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Clinical Care ◽  
Positive Family History ◽  
Diagnostic Methods ◽  
Common Symptom ◽  
Cell Disease ◽  
Eastern Uganda

Background: In Uganda to date, there are neither established registries nor descriptions of facility-based sickle cell disease (SCD) patient characteristics beyond the central region. Here, we summarize data on the baseline clinical characteristics and routine care available to patients at four clinics in Eastern Uganda as a prelude to a clinical trial. Methods: Between February and August 2018, we conducted a cross-sectional survey of patients attending four SCD clinics in Mbale, Soroti, Atutur and Ngora, all in Eastern Uganda, the planned sites for an upcoming clinical trial (H-PRIME: ISRCTN15724013). Data on socio-demographic characteristics, diagnostic methods, clinic schedules, the use of prophylactic and therapeutic drugs, clinical complications and patient understanding of SCD were collected using a structured questionnaire. Results: Data were collected on 1829 patients. Their ages ranged from 0 to 64 years with a median (IQR) of 6 (3-11) years. 50.9% of participants were male. The majority (1151; 62.9%) reported a positive family history for SCD. Approximately half knew that SCD is inherited from both parents but a substantial proportion did not know how SCD is transmitted and small numbers believed that it is acquired by either transfusion or from other people. Only 118/1819 (6.5%) participants had heard about or were using hydroxyurea while 356/1794 (19.8%) reported stigmatization. Participants reported a median of three (IQR 1-4) hospital admissions during the preceding 12 months; 80.8% had been admitted at least once, while 14.2% had been admitted more than five times. Pain was the most common symptom, while 83.9% of those admitted had received at least one blood transfusion. Conclusion: The majority of patients attending SCD clinics in Eastern Uganda are children and few are currently being treated with hydroxyurea. The data collected through this facility-based survey will provide background data that will be useful in planning for the H-PRIME trial.

scholarly journals Characterising demographics, knowledge, practices and clinical care among patients attending sickle cell disease clinics in Eastern Uganda

2020 ◽  
Vol 5 ◽  
pp. 87
Author(s):  
Peter Olupot-Olupot ◽  
Ham Wabwire ◽  
Carolyne Ndila ◽  
Ruth Adong ◽  
Linus Ochen ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Clinical Care ◽  
Positive Family History ◽  
Diagnostic Methods ◽  
Common Symptom ◽  
Cell Disease ◽  
Eastern Uganda

Background: In Uganda to date, there are neither established registries nor descriptions of facility-based sickle cell disease (SCD) patient characteristics beyond the central region. Here, we summarize data on the baseline clinical characteristics and routine care available to patients at four clinics in Eastern Uganda as a prelude to a clinical trial. Methods: Between February and August 2018, we conducted a cross-sectional survey of patients attending four SCD clinics in Mbale, Soroti, Atutur and Ngora, all in Eastern Uganda, the planned sites for an upcoming clinical trial (H-PRIME: ISRCTN15724013). Data on socio-demographic characteristics, diagnostic methods, clinic schedules, the use of prophylactic and therapeutic drugs, clinical complications and patient understanding of SCD were collected using a structured questionnaire. Results: Data were collected on 1829 patients. Their ages ranged from 0 to 64 years with a median (IQR) of 6 (3-11) years. 49.1% of participants were male. The majority (1151; 62.9%) reported a positive family history for SCD. Approximately half knew that SCD is inherited from both parents but a substantial proportion did not know how SCD is transmitted and small numbers believed that it is acquired by either transfusion or from other people. Only 118/1819 (6.5%) participants had heard about or were using hydroxyurea while 356/1794 (19.8%) reported stigmatization. Participants reported a median of three (IQR 1-4) hospital admissions during the preceding 12 months; 80.8% had been admitted at least once, while 14.2% had been admitted more than five times. Pain was the most common symptom, while 83.9% of those admitted had received at least one blood transfusion. Conclusion: The majority of patients attending SCD clinics in Eastern Uganda are children and few are currently being treated with hydroxyurea. The data collected through this facility-based survey will provide background data that will be useful in planning for the H-PRIME trial.

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