Effect of Trans-Resveratrol on Sickle Cell Disease during Pregnancy: Implications for Maternal and Fetal Health | Chapter 9 | An Overview of Disease and Health Research Vol. 5

 

The application of natural products has long been used in the treatment of illness and disease, many of which formed the industrial inspiration to develop from pharmacognosy into active pharmaceuticals. Resveratrol is a common phytochemical found in abundance in nutritious and wholesome foods and thus has likely been part of the human diet for many centuries. As such, resveratrol may contribute to the success of the French Paradox, where the inclusion of red wines is a familiar dietary addition and is associated with reduced incidence of cardiovascular disease. The primary metabolic action of resveratrol and related phenolic compounds likely rests in its potential antioxidant activity, being able to quench reactive free radicals (ROS) and other inflammatory activities in peripheral tissues. Resveratrol has been suggested as an adjunct in the clinical management of numerous metabolic disorders, including diabetes, obesity, hypertension, pre-eclampsia, and sickle cell disease, among others associated with chronic inflammation. Thus, the purpose of this review is to examine the safety and efficacy of resveratrol in pregnancy. This review also discusses the biochemical, pharmacologic and potential toxicologic aspects of trans-resveratrol administration as an adjunct in the treatment of hemoglobinopathies, including Sickle cell disease (SCD), during pregnancy. SCD represents a serious complication of normal pregnancy with few successful treatments, as the change in amino acid charge alters the protein’s hydrophobic structure and function. When present, it can result in the abnormal polymerisation to form deoxy-HbS and sickling, particularly during physiologic processes that incur high oxygen demand, including later stages of gestation and parturition.  The results indicate that resveratrol is likely a safe and effective adjunct in treating the hypoxia and chronic inflammation of pregnancy, in addition to its applications in various hemoglobinopathies, including sickle cell disease, by inducing increased production of sickle-resistant fetal haemoglobin (HbF) in addition to reducing the magnitude of chronic inflammation, while enhancing oxygen delivery to peripheral tissues via actions of fetal haemoglobin. Thus, RSV may also be able to partially correct the effects linked to the globin chain imbalance in SCD patients, while at the same time facilitating oxygen transport to myoglobin in peripheral tissues due to a more favourable oxygen-delivering capacity than is observed in adult haemoglobin.

 

 

Author(s) Details

Orien L Tulp
University of Science Arts and Technology, Montserrat, Colleges of Medicine and Graduate Studies, Spain, The Einstein Medical Institute, North Palm Beach, Florida, USA, East West College of Natural Medicine, Sarasota, FL, USA and Universidad de Sevilla, Seville, Spain.

 

Frantz Sainvil
University of Science Arts and Technology, Montserrat, Colleges of Medicine and Graduate Studies, Spain, The Einstein Medical Institute, North Palm Beach, Florida, USA and Broward College, Davie, FL, USA.

 

Uzoamaka Nwokorie
University of Science Arts and Technology, Montserrat, Colleges of Medicine and Graduate Studies, Spain.

 

George P Einstein
University of Science Arts and Technology, Montserrat, Colleges of Medicine and Graduate Studies, Spain and The Einstein Medical Institute, North Palm Beach, Florida, USA

 

Please see the book here :- https://doi.org/10.9734/bpi/aodhr/v5/6062

 

Assessing the Risk Factors for Metabolic Syndrome in Adult Patients with Sickle Cell Anemia in Jazan Region, Kingdom of Saudi Arabia | Chapter 7 | Disease and Health: Research Developments Vol. 8

Hematological anemia is a characteristic of the hereditary condition known as sickle cell disease (SCD). SCD is considered the world's most prevalent and widespread hemoglobinopathy. According to several studies, adults with sickle cell disease may exhibit metabolic syndrome symptoms such as hyperglycemia, hypertension, and dyslipidemia. This study's objective was intended to assess the steady-state risk variables for metabolic syndrome in adults with sickle cell anemia in the Jazan region. A case-control research with 35 adult sickle cell disease patients and 35 healthy controls was carried out. The study was conducted in the Jazan area in the period of 2021 to 2022. The population was Saudi patients with sickle cell anemia as a test group and healthy volunteers as a control test. The National Cholesterol Education Program - Adult Treatment Panel III (NCEP-ATPIII) was used to diagnose metabolic syndrome after the data were examined to determine fasting plasma glucose, lipid profile, and uric acid. Mean, standard deviation (SD), and p-value were used to express the data. In order to establish statistical significance, a value of 0.05 was employed. Fasting triglyceride levels were comparable between patient and control groups (P-value = 0.54), high-density lipoprotein cholesterol (HDL-C) was significantly low in the case group compared to the control group (P-value = 0.03) and the uric acid level was significantly different in patient and control groups (P-value = 0.55). There was significant decrease in HDL-C and FPG among SCD patients compared to the control group while no significant difference in fasting triglyceride and uric acid between patients and control groups. Focusing on the development of metabolic syndrome in SCA patients may provide new insights for therapeutic strategies with the aim of reducing mortality and morbidity.

 

Author (s) Details

 

Saif Elden B. Abdalla
Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jazan, Saudi Arabia.

 

Muntaser Mohammed Fadoul Alhassen
Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jazan, Saudi Arabia.

 

Mohammed Shane Alam
Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jazan, Saudi Arabia.

 

Sarra Kamal Mustafa
Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jazan, Saudi Arabia.

 

Mohamed Mubark Almaki
Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jazan, Saudi Arabia.

 

Mohamed H. Abdelrahman
Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jazan, Saudi Arabia.

 

 

Please see the book here:- https://doi.org/10.9734/bpi/dhrd/v8/4820

Understanding the Pathoanatomy of Arthritic Hip for Improvising the Technique in Primary Uncemented Total Hip Arthroplasty | Chapter 7 | Advancement and New Understanding in Medical Science Vol. 10

 Background: Hip osteoarthritis is a condition that leads to significant morbidity and decreases the quality of patient’s life. Total Hip Arthroplasty (THA) is preferred by most of orthopaedic surgeons to relieve pain, provide motion while maintaining stability. In this study we aim to discuss the difficulties encountered in reference to patho-anatomy during THA in various conditions along with their solutions.

 

Materials and Methods: In our study, we had observed 100 hip joints of secondary osteoarthritis in 67 patients of with different type of underlying pathology like avascular necrosis, sickle cell disease, rheumatoid arthritis, ankylosing spondylitis. All the patients were evaluated using modified harris hip score. The findings were evaluated using SPSS software (Ilionois, Chicago) with p value less than 0.05 considered as significant.

 

Observation and Results: We had operated 100 hip joints (n=100) having arthritic changes among 67 patients (33 patients with bilateral hip affection, 34 patients with unilateral hip affection) at our tertiary care hospital from June 2015 till December 2019. The mean Harris Hip Score at the final follow up was 88 among them 98 hip joints had excellent and 2 hip joints had good to fair results. During our study we majorly encountered three types of collapse – Anterosuperior collapse (56%), Concentric collapse (25%) and hip arthritis due to inflammatory conditions (19%). We had found that according to duration and types of collapse different soft tissues were contracted and a step-wise release of those structures is necessary for proper intra-operation reduction and post-operative better range of motion. The steps for global release as and when required in different patients include adductor tenotomy, release of external rotator muscles, release of anterior fibers of  gluteus medius, release of tensor fascia lata and tendinous insertion of gluteus maximus along with release of posterior capsule, release of anterior capsule followed by release of iliopsoas tendinous insertion on lesser trochanter.

 

Conclusion: The chronicity of the disease will lead to anatomical changes of the surrounding soft tissue and that will affect the surgical dissection. We had described surgical problems encountered during the operation and their solutions for each type of pathoanatomy. This will help orthopedic surgeons to anticipate and prepare well in advance while performing total hip arthroplasty.

Author(s) Details:

Paresh Golwala,
SBKS& MIRC, Sumandeep Vidyapeeth (Deemed to be University), India.

Parth N. Rathi,
MM Hospital & Hariom Trust, Adipur, Bhuj Kutch 370205, Gujarat, India.

Prassana Shah,
Department of Orthopedics, Dhiraj Hospital, Smt. BK Shah Medical Institute & Research Centre, Sumandeep Vidyapeeth (Deemed to be University), Waghodia, Vadodara 391760, Gujarat, India.

Please see the link here: https://stm.bookpi.org/ANUMS-V10/article/view/13927

Epidemiological, Diagnostic and Evolutionary Characteristics of Malaria in Children with Sickle Cell Disease | Chapter 7 | Recent Updates in Disease and Health Research Vol. 1

Background: Malaria is a major public health problem in our countries where its distribution is similar to sickle cell disease. According to the latest world report on malaria, there are 249 million cases in 2022 with 608,000 deaths.

Children under 5 years old represent approximately 78% of all malaria deaths in WHO Africa Region where we find 94% of all malaria cases and 95% of deaths [16=1].

The relationship between sickle cell disease and malaria is the subject of much controversy. However, there is a lack of data on our services. Our objective was to study the epidemiological, diagnostic and evolutionary characteristics of malaria in children with sickle cell disease followed in a specialized setting.

Patients and Methods: We conducted a retrospective, descriptive, and analytical study of children with sickle cell disease (SCD) who presented with malaria and were followed at the Ambulatory Care Unit for Sickle Cell Children and Adolescents (USAD) at the Albert Royer National Children’s Hospital in Dakar, Senegal, West Africa from January 1st, 2017, to December 31st, 2019. We included all the following pediatric patients, less than 16 years, with sickle cell disease who presented at least one episode of malaria, confirmed by a positive thick drop, during these 3 years. We did not include patients with incomplete records or those older than 16 years. The clinical and biological signs, and the follow-up was collected and analyzed with Excel package 2019.

Results: Of 3773 patients followed for sickle cell disease, 21 had presented malaria. The frequency was 0.5% or 7 cases/year. However, we exploited the data of 14 of them. The sex ratio was 6 boys for a girl and the mean age at admission was 7.3 years. The highest number of malaria cases was observed in 2018 and the peak frequency was observed in November with 8 cases (57.1%). Fever was the most frequent symptom, observed in 10 patients (71.4%). All patients were SS homozygous, with a mean baseline hemoglobin level of 7.5g/dl. All patients had a positive thick blood smear and Plasmodium falciparum was the only species found in the blood smear, with a mean parasite density of 1693 parasites/ml of blood. All patients had anemia, with a mean hemoglobin level of 7.74 g/dl. Twelve patients (85.7%) were hospitalized and had all received inject- able artesunate followed by oral Artemisinin Combination Therapy (ACT). Long-lasting insecticidal nets were used in 9 patients (69.2%). The evolution was favorable in all patients, and any death was reported.

Conclusion: The patients who presented the association of malaria and sickle cell disease were all SS homozygotes. However, malaria must be considered as serious in this chronic anemic setting. That’s why it is important to improve prophylaxis. Therefore, it is important to make different strategies to reduce the global burden.

Author(s) Details:

Indou Deme/Ly,
Cheikh Anta Diop University, Dakar, Senegal.

Cheikh Binetou Fall,
Cheikh Anta Diop University, Dakar, Senegal.

Awa Kane,
Cheikh Anta Diop University, Dakar, Senegal.

Ibrahima Diop,
Cheikh Anta Diop University, Dakar, Senegal.

Aminata Mbaye,
Cheikh Anta Diop University, Dakar, Senegal.

Yaaye Joor Dieng,
Cheikh Anta Diop University, Dakar, Senegal.

Denika Estelle Liapoui,
Cheikh Anta Diop University, Dakar, Senegal.

Idrissa Demba Ba,
Cheikh Anta Diop University, Dakar, Senegal.

Abou Ba,
Cheikh Anta Diop University, Dakar, Senegal.

Aliou Thiongane,
Cheikh Anta Diop University, Dakar, Senegal.

Papa Moctar Faye,
Cheikh Anta Diop University, Dakar, Senegal.

Amadou Lamine Fall,
Cheikh Anta Diop University, Dakar, Senegal.

Ibrahima Diagne,
Gaston Berger University, Saint-Louis, Senegal.

Ousmane Ndiaye,
Cheikh Anta Diop University, Dakar, Senegal.

Please see the link here: https://stm.bookpi.org/RUDHR-V1/article/view/13215

A Review on Prevalence and Risk Factors of Gallstone Disease in Uganda | Chapter 7 | Advancement and New Understanding in Medical Science Vol. 4

Introduction: Globally, the burden and complications from gallstone disease are a major public health issue. It contributes significantly to health care costs and is a common cause of surgical intervention. Most sickle cell disease (SCD) patients have radiolucent stones, probably of pigment type, containing little or no calcium. While most patients with gallstones are asymptomatic, only 20% after 10 years become symptomatic. The prevalence of gallstone disease varies among different populations. The purpose of this review is to understand the profile of gallstone disease patients in Uganda which may facilitate better treatment options and may reduce the complications of gallstone disease.

Discussion: In Uganda, the prevalence of gallstone disease is high in patients presenting to hospital. The risk factors for gallstone disease include a previous history of biliary symptoms and the use of hormonal contraceptives. SCD in Sub-Saharan Africa is also a major health problem and a risk factor for gallstone disease. Acute abdominal pain in SCD patients was thought to be due to sickle cell crises until the advent of ultrasonography. Differentiating between acute cholecystitis and sickle cell crises may be difficult. An increased production of unconjugated bilirubin from a sickle cell crisis may precipitate acute cholecystitis. While in SCD patients with acute cholecystitis, a haemolytic crisis may occur.

Conclusions: While the prevalence of gallstone disease in Uganda from hospital-based studies is high the majority of patients are symptomatic at presentation. In the general population, the prevalence of gallstones may be determined using abdominal ultrasound as a screening tool. Any SCD patient who has an abdominal crisis should have an abdominal ultrasound to prevent missing any episodes of acute cholecystitis.

Author(s) Details:

Richard Wismayer,
Department of Surgery, Faculty of Health Sciences, Habib Medical School, IUIU University, Kampala, Uganda and Department of Surgery, Masaka Regional Referral Hospital, Masaka, Uganda.

Please see the link here: https://stm.bookpi.org/ANUMS-V4/article/view/13177

Behavior Therapy in Curing Depression for Adolescents with Sickle Cell Disease | Chapter 2 | Advanced Concepts in Medicine and Medical Research Vol. 10

Cavity has been identified all at once of the commonest insane-health problems in SCD, accompanying prevalence rates as extreme as 27% - 46% reported [1]. While psychological cures have been establish to be beneficial in gentle to moderate depression, their use as non-pharmacological orders amongst adolescents in substitute-Saharan Africa, specifically Nigeria, is still at its infancy. This member determines the effect of behavioural incitement therapy on depression in juveniles living with Cut Cell Disease accompanying out-patient clinic at the Lagos State Academy Teaching Hospital, Lagos, Nigeria. The study was conducted in Lagos State Academy Teaching Hospital, Lagos, Nigeria, 'tween November 2017 to February 2018 (4 months) among adolescents endure SCD and depression accompanying out-patient clinic. The participants were a sample of agreeing adolescents (12 - 17 age) with SCD who had temperate to moderate depression. We forbade those with severe cavity on anti-sedative medications. The participants were detached into two groups, the treatment group and wait list (control) group. A randomized control trial (RCT) was transported among participants filling a place treatment (30) against control (30) group. A manualized behavioral therapy programme developed was brought to the treatment group. The dossier was analysed using the Statistical Whole for Social Sciences (SPSS) form 23. Pair T-test was used to compare the two groups across constant variables, pre- and post-intervention. Study of Covariance (ANCOVA) was used to deter- mine treatment belongings controlling for standard scores. After the inter- vention, the mean despair score reduced from 22.13 ± 3.08 to 13.02 ± 4.56 while Paediatric feature of life score increase from 62.57 ± 17.85 to 67.90 ± 7.99 in the treatment group (BDI, p < 0.001; PedsQL, p = 0.045). Nevertheless, the pre- and post-intervention mean concavity scores in the control group showed insignificant decline from 22.23 ± 3.24 to 21.60 ± 2.75 and mean PedsQL from pre-intervention scores of 59.67 ± 12.60 to 56.73 ± 8.94 post attack (BDI, p = 0.388; PedsQL, p = 0.242). From multivariate analysis (ANCOVA), only the Quality of Growth scores ceased to show some significant effect of the intervention.Finally, the effect of behavioral incitement therapy (BAT) in juveniles with SCD and gentle to moderate depression using a randomized reserved trial has been grown. The results reflected that BAT happened in significant betterings in self-reported rating of depressing symptoms (effect intensity 2.1) and depression literacy suggesting productiveness of BAT in relieveing depressive symptoms in this place cohort, still the QoL scores initially appeared to have had a beneficial change but ceased expected so after controlling for likely confounders.

Author(s) Details:

Tolulope Taiwo,
Lagos State University Teaching Hospital, Lagos State University, Lagos, Nigeria and Crisis Resolution and Home Treatment Team (CRHTT), Birmingham Womens and Children NHS Trust, UK.

Olayinka Atilola,
Department of Behavioral Medicine, Lagos State University College of Medicine, Lagos, Nigeria.

Cornelius Ani,
Division of Psychiatry, Imperial College London, Surrey and Borders Partnership NHS Foundation Trust, UK.

Bola A. Ola,
Department of Behavioral Medicine, Lagos State University College of Medicine, Lagos, Nigeria and Acute Mental Health Unit, Toowoomba Base Hospital, Queensland, Australia.

Please see the link here: https://stm.bookpi.org/ACMMR-V10/article/view/12816

Using of PCR Technique for Prenatal Diagnosis of Sickle Cell Disease | Chapter 12 | Research Developments in Medicine and Medical Science Vol. 10

 In present study, fetal diagnostic ease for couples carrying sickle container gene was settled with the aim for fear that birth of affected blastula and to calculate the nervousness of ARMS-PCR by comparing results accompanying baby’s Hemoglobin pattern on follow up HPLC. Sickle cell ailment (SCD) is common in Central India and causes important morbidity and mortality. There is a shortage of prenatal demonstrative facilities close by physically to the SCD population. This is a pilot study in our domain with the aim of establishing a fetal diagnostic facility in Central India for couples accomplishing the sickle container gene in consideration of help them make an cognizant decision about a foetus stirred with SCD, in addition to calculating the sensitivity of the polymerase vicious circle (PCR) technique in our arrangement with follow-up souped up liquid chromatography (HPLC) of the baby's blood sample. Follow-up HPLC was accomplished to detect baby’s Hb pattern. Prenatal disease of sickle cell blood deficiency was offered in total 37 cases exhausted which individual (2.7%) fetal sample was inadequate. Total 26 (70.27%) fetuses had AS Hb genotype, 3 (8.11%) had AA Hb genotype and 3 (8.11%) had SS Hb genotype while surplus 4 (10.81%) were given AA/AS Hb genotype. All couples accompanying SS fetuses opted for MTP. Follow up HPLC was acted in 24 cases, out of which 18 (75%) were compared and 6 (25%) were mismatched. In present study sympathy of ARMS-PCR was 75%. ARMS-PCR is a simple method to be established originally for providing rapid fetal diagnosis to the couples with famous sickle container mutation. The subtlety of ARMS-PCR can be increased by utilizing suitable methods to detect motherly cell DNA on society can so be reduced by sensitising couples accompanying the sickle container gene through proper historical counselling and providing the alternative of Medical Termination of Pregnancy for affected foetuses. More couples will benefit from now on if we raise awareness about the safety and efficiency of modern fetal diagnostic techniques.

Author(s) Details:

Praneeta J. Singh,
Department of Pathology, Indira Gandhi Government Medical College, Nagpur, India.

A. C. Shrivastava,
Department of Pathology, Indira Gandhi Government Medical College, Nagpur, India.

A. V. Shrikhande,
Department of Pathology, Indira Gandhi Government Medical College, Nagpur, India.

Please see the link here: https://stm.bookpi.org/RDMMS-V10/article/view/10683

β-like Globin Gene Cluster Haplotypes in Sickle Cell Disease Patients in Odisha, India | Chapter 13 | Perspective of Recent Advances in Medical Research Vol. 11

 Though Sickle container disease (SCD) is innately characterised by a single point mutation, skilled are various modulators that influence the phenotype of this disease accompanying varying grades of clinical asperity. The most important phenotype modulators are HbF concentration, union of α-thalassemia, haplotype. SCA is associated with five "classic" haplotypes, each with different terrestrial distributions across different domains of the world. It has happened suggested that these haplotypes also influence HbF aggregation. Lowest HbF concentration is in the direction of Benin and Bantu haplotype and highest in Indian-Arab (AI) haplotype. Eight polymorphic sites inside the β-globin gene cluster (Xmnl Gγ, Hind-III Gγ, Hind-III Aγ, HincIl  ψβ, HincII 3’ ψβ, RsaI 5’β, AvaII β and HinfI 3’β) were resolved by Restriction Fragment Length Polymorphism (RFLP). We observed the closeness of Arab-Indian haplotype in all our samples.  We have also establish that 75.86% showed homozygous Arab-Indian culture followed by 24.14% heterozygous Arab-Indian population.

Author(s) Details:

Pulak Pritam,
AIPH University, Bhubaneswar, Odisha-751002, India and School of Applied Sciences, Kalinga institute of Industrial Technology, KIIT University, India.

Bimal Prasad Jit,
All India Institute of Medical Science, Ansari Nagar, New Delhi-110029, India.

Padmalaya Das,
AIPH University, Bhubaneswar, Odisha-751002, India.

Please see the link here: https://stm.bookpi.org/PRAMR-V11/article/view/9575

Nanotechnology in the Treatment of Sickle Cell Disease | Chapter 7 | Current Innovations in Medicine and Medical Science Vol. 5

 Numerous cases won't be relieved of their symptoms after surgery because the remedial efficacity of the medicine varies from case to case. The thing of this case report is to compare the remedial efficacity of two patches, ketoprofen and fentanyl, inpost-operative cases. We present a case report on the extent of pain relief compared with visual analogue scale scores( VAS score) afterpost-operative surgery other than chine surgery using ketoprofen transdermal patch and fentanyl transdermal patch on cases. The subjects were divided into two groups, manly and womanish, and the treatment was given for over to three days.

After completing the study, we concluded that the subjects who entered ketoprofen remedy had the loftiest VAS score when compared to those who entered fentanyl remedy, and complete pain relief was observed in three days. The statistical analysis revealed that it was largely significant..

Author(s) Details:

Bimal Prasad Jit,
All India Institute of Medical Science, Ansari Nagar, New Delhi - 110029, India and School of Life Sciences, Sambalpur University, Burla, Odisha - 768019, India and AIPH University, Bhubaneswar, Odisha - 751002, India.

Abhisekh Sahu,
School of Life Sciences, Sambalpur University, Burla, Odisha - 768019, India.

Rakesh Arya,
School of Life Sciences, Sambalpur University, Burla, Odisha - 768019, India.

Ashok Sharma,
All India Institute of Medical Science, Ansari Nagar, New Delhi - 110029, India..

Padmalaya Das,
AIPH University, Bhubaneswar, Odisha - 751002, India.

Please see the link here: https://stm.bookpi.org/CIMMS-V5/article/view/8465

Determination of Sickle Cell Disease Control Prospects the Emerging Necessities Due to Impactful Covid-19 Pandemic in Sub Saharan Africa | Chapter 15 | Highlights on Medicine and Medical Science Vol. 14

Background: People with sickle cell disease (SCD) seek medical help frequently since the disease is linked to a high rate of morbidity and mortality. The COVID-19 epidemic has forced a shift in health-care focus and resources away from routine demands. COVID-19 cases have infiltrated health-care facilities, physicians, and care coordinators. This has exacerbated Sub-Saharan Africa's already dismal status of SCD management (SSA). It's critical to consider strategies that have been tested and proven to lower the occurrence of SCD. The goal of this review is to propose potential solutions and approaches for reducing SCD prevalence.

Study Goal: To study the literature and focus on research, presentations, and information on SCD control.

The review's goals were to describe and debate the approaches that have been used to lower the prevalence of SCD in order to see if they may be used in SSA. A review of the literature, with a focus on studies, presentations, and information about SCD control. PubMed, PubMed Central, Google Scholar, and African Index Medicus were used as sources. Any part of SCD control by educational, counselling, awareness, prevention, testing, and providing information served as the basis for selection. These possibilities have been investigated, analysed, and presented in this article.

Author (S) Details

Mwanda O. Walter
Haematology & Blood Transfusion, University of Nairobi, Kenya.

Peter Oyiro
Department of Clinical Medicine and Therapeutics, University of Nairobi, Kenya.

View Book :- https://stm.bookpi.org/HMMS-V14/article/view/2566

Gene Therapy in Sickle Cell Disease: Advanced Study | Chapter 2 | Emerging Research in Medical Sciences Vol. 4

Sickle cell disease (SCD) results from a homozygous missense mutation in the β-globin gene that leads to polymerization of hemoglobin S. Its clinical manifestations can be critical with considerable morbidity and mortality. SCD can be treated using bone marrow transplantation, but is restricted to only those patients having an appropriately matched donor. Hence, gene therapy, involving the patient’s own cells, either by gene insertion or gene editing is a primary therapeutic option to cure SCD. However, only a few clinical trials have been performed with genetic therapy for treating SCD. In recent years, significant progress has been made in the area of gene therapy for treating monogenic hemoglobin disorders. Numerous therapies are currently in clinical trial stages or in preclinical stages. The safety and efficacy of gene therapy has been greatly improved with the initial use of γ-retrovirus vectors, followed by next-generation lentivirus vectors and latest gene editing techniques. Although the clinical interpretation of gene therapy has been successful, it involves certain limitations including complex cellular abnormalities, inadequate transgene expression and challenges in achieving effective and persistent inhibition of polymerization of hemoglobin S. This review intends to discuss gene therapy strategies specific to SCD, present state of the field and current status of the gene therapy clinical trials.

Author(s) Details

Dr. Salma AlDallal
Kuwait Ministry of Health, Amiri Hospital, Sharq, Kuwait.

View Book :-  http://bp.bookpi.org/index.php/bpi/catalog/book/167