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Pediatric Blood & Cancer Jun 2021Cardiac disease is the primary cause of death in sickle cell disease (SCD). Cardiac abnormalities begin in childhood and progress throughout life. Right and left...
Cardiac disease is the primary cause of death in sickle cell disease (SCD). Cardiac abnormalities begin in childhood and progress throughout life. Right and left ventricular (RV, LV) myocardial strain are early markers of systolic dysfunction but are not well investigated among individuals with SCD. The objectives of this review were to (1) identify all published studies that have evaluated ventricular myocardial strain, (2) summarize their values, and (3) compare findings with those obtained from controls. From search results of four electronic databases-Medline, Embase, Scopus, and Web of Science-42 potential articles were identified, of which 18 articles and 17 studies met eligibility criteria for inclusion. The evaluated studies demonstrate that RV and LV myocardial strain are generally abnormal in individuals with SCD compared with controls, despite having normal ejection/shortening fraction. Myocardial strain has been inconsistently evaluated in this population and should be considered any time an echocardiogram is performed.
Topics: Adult; Anemia, Sickle Cell; Cardiomyopathies; Child; Echocardiography; Heart Ventricles; Humans; Infant; Middle Aged; Stroke Volume; Ventricular Dysfunction, Left; Ventricular Dysfunction, Right
PubMed: 33742492
DOI: 10.1002/pbc.28973 -
Orphanet Journal of Rare Diseases Mar 2021Hydroxyurea and blood transfusion therapies remain the main therapeutic strategies for Sickle cell disease. Preliminary data suggest substantial variation and... (Review)
Review
BACKGROUND
Hydroxyurea and blood transfusion therapies remain the main therapeutic strategies for Sickle cell disease. Preliminary data suggest substantial variation and inconsistencies in practice of these two therapeutic modalities in South Asia. In this systematic review we searched Medline, Cochrane library and Scopus for articles on usage of hydroxyurea and blood transfusion therapies for sickle cell disease in South Asia published in English between October 2005 and October 2020.
RESULTS
We selected 41 papers: 33 from India, 3 from Sri Lanka, 2 each from Pakistan and Bangladesh and one from Nepal. Only 14 prospective trials focused on hydroxyurea therapy from which majority (n = 10; 71.4%) adopted fixed low dose (10 mg/kg/day) regimen. With hydroxyurea therapy, 12 and 9 studies reported significant reductions in vaso-occlusive crises and transfusion requirement respectively. Severe anaemia (haemoglobin level < 6-7 g/dl) was the commonest indicator (n = 8) for transfusion therapy followed by vaso-occlusive crisis.
CONCLUSIONS
Published data on the hydroxyurea and transfusion therapies in South Asia are limited and heterogeneous. A clear gap of knowledge exists about the nature of the sickle cell disease in the Indian subcontinent particularly from countries outside India necessitating further evidence-based assessments and interventions.
Topics: Anemia, Sickle Cell; Bangladesh; Blood Transfusion; Humans; Hydroxyurea; India; Neglected Diseases; Pakistan; Prospective Studies
PubMed: 33757549
DOI: 10.1186/s13023-021-01781-w -
Frontiers in Medicine 2022Infections in the male genitourinary system with bacterial and viral agents may play a significant role in male infertility. These agents usually infect the urethra,...
OBJECTIVES
Infections in the male genitourinary system with bacterial and viral agents may play a significant role in male infertility. These agents usually infect the urethra, seminal vesicles, prostate, epididymis, vas deferens, and testes retrograde through the reproductive system. A meta-analysis review study was performed to evaluate the presence of bacterial and viral agents in the semen of infertile men and its correlation with infertility.
METHODS
Relevant cross-sectional and/or case-control studies were found by an online review of national and international databases (Web of Science, PubMed, Scopus, Science Direct, and Google scholar), and suitable studies were selected. A checklist determined the qualities of all studies. Heterogeneity assay among the primary studies was evaluated by Cochran's test and I index (significance level 50%). A statistical analysis was conducted using the Comprehensive Stata ver. 14 package (StataCorp, College Station, TX, United States).
RESULTS
Seventy-two studies were included in this meta-analysis. Publication bias was compared with Egger's test, and the impact of each research on overall estimate was evaluated by sensitivity analysis. In 56 studies, the rate of bacterial infections in the semen of infertile men was 12% [95% confidence interval (CI): 10-13]. Also, in 26 case-control studies, the association of infertility in men with bacterial infections was evaluated. The results show that the odds ratio of infertility in men exposed to bacterial infections is 3.31 times higher than that in non-infected men (95% CI: 2.60-4.23). Besides, in 9 studies that examined the prevalence of human papillomavirus (HPV), herpes simplex virus 1 (HSV1), herpes simplex virus 2 (HSV2), and herpes simplex virus 1-2 (HSV1-2) in infertile men, the frequency of these viruses was 15% (95% CI: 9-21). In 6 case-control studies, the association between human cytomegalovirus (HCMV), Cytomegalovirus (CMV), and HPV and male infertility was evaluated. The chance of male infertility due to exposure to these viruses was 2.24 times higher than those without exposure to these viruses (CI 95%: 1.9-4.52). The results show that the chance of infertility in men exposed to bacteria was significantly higher than that in the uninfected population.
CONCLUSION
This meta-analysis showed that viral and bacterial infections are a risk factor and could impair male fertility potential. Moreover, our study supports the hypothesis that bacterial and viral infections of the genital tract correlate positively with impairment of sperm quality in the male population.
PubMed: 35602502
DOI: 10.3389/fmed.2022.835254 -
The Cochrane Database of Systematic... Aug 2019Thalassaemia is a quantitative abnormality of haemoglobin caused by mutations in genes controlling production of alpha or beta globins. Abnormally unpaired globin chains...
BACKGROUND
Thalassaemia is a quantitative abnormality of haemoglobin caused by mutations in genes controlling production of alpha or beta globins. Abnormally unpaired globin chains cause haemolytic anaemia by causing membrane damage and cell death within organ systems and destruction of erythroid precursors in the bone marrow. The life-long management of the general health effects of thalassaemia in affected individuals is a highly challenging issue in and of itself; and failure to deal with dental and orthodontic complications in people with thalassaemia exacerbates the public health, financial and personal burden posed by the condition. There exists a lack of evidence-based guidelines for care-seekers and providers to best deal with such dental and orthodontic complications in thalassaemia, which this review seeks to address.
OBJECTIVES
The main objective of this review was to assess different methods to treat dental and orthodontic complications in people with thalassaemia.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We searched the reference lists of relevant articles and reviews.Date of last search: 01 August 2019.We also searched nine online databases (PubMed, Google Scholar, ClinicalTrials.gov, WHO International Clinical Trials Registry Platform, Literature in the Health Sciences in Latin America and the Caribbean database, African Index Medicus, Index Medicus for South East Asia Region, Index Medicus for the Eastern Mediterranean Region, Indexing of Indian Medical Journals). We searched the reference lists of relevant articles and reviews and contacted haematologists, experts in fields of dentistry, organizations, pharmaceutical companies and researchers working in this field.Date of last search: 22 July 2019.
SELECTION CRITERIA
We searched for published or unpublished randomised controlled trials for treatment of dental and orthodontic complications in individuals diagnosed with thalassaemia, irrespective of phenotype, severity, age, gender and ethnic origin.
DATA COLLECTION AND ANALYSIS
Two review authors independently screened 35,202 titles from search results. We identified four unique randomised controlled trials, of which one seemed potentially relevant. Based on closer inspection, the trial was found not to be eligible for inclusion.
MAIN RESULTS
We did not find any relevant trials for inclusion in the review.
AUTHORS' CONCLUSIONS
We were unable to draw any conclusions due to the lack of available data and trials. This review highlights the need for conducting and appropriate reporting, of high-quality randomised controlled trials investigating the effectiveness of various treatment modalities for dental and orthodontic complications in people with thalassaemia.
Topics: Humans; Malocclusion; Randomized Controlled Trials as Topic; Thalassemia; Tooth Diseases
PubMed: 31425614
DOI: 10.1002/14651858.CD012969.pub2 -
Pediatric Blood & Cancer Jul 2021Vaso-occlusive episodes (VOEs) are a common complication of sickle cell disease (SCD) and a significant cause of morbidity. Managing VOE pain can be difficult and... (Review)
Review
Vaso-occlusive episodes (VOEs) are a common complication of sickle cell disease (SCD) and a significant cause of morbidity. Managing VOE pain can be difficult and complex. Ketamine, an N-methyl-D-aspartate (NMDA) receptor antagonist, has been used to manage VOE pain. This systematic literature review synthesizes research published from 2010 to 2020 on the use of ketamine infusion to decrease VOE pain. The review demonstrates that ketamine, a safe and effective treatment for VOE pain, could be considered more widely. However, the significant variability among published clinical studies with regard to dosing, timing of initiation, duration of infusion, and timing of discontinuation highlights the need for standardized ketamine infusion protocols for the management of VOE pain. We conclude with a brief discussion of key components of a potential standardized protocol supported by the literature reviewed as well as areas for future investigation.
Topics: Anemia, Sickle Cell; Humans; Ketamine; Pain; Pain Management; Pain Measurement
PubMed: 33788404
DOI: 10.1002/pbc.28989 -
Hemoglobin Nov 2023The sickle cell disease (SCD) population has been considered particularly vulnerable to viral pandemics since the emergence of H1N1 in 2009. In this sense, the advance... (Meta-Analysis)
Meta-Analysis Review
The sickle cell disease (SCD) population has been considered particularly vulnerable to viral pandemics since the emergence of H1N1 in 2009. In this sense, the advance of the COVID-19 pandemic from 2020 has brought this group of patients to the center of concern. However, scientific knowledge about the susceptibility of patients with SCD to a severe COVID-19 pandemic is still insufficient, and efforts to establish a general profile of the disease in these patients, remain inadequate. The present study, therefore, sought to characterize the case fatality rate and severity of COVID-19 in patients with SCD throughout the world. A systematic review of Pubmed/MEDLINE, Scopus, Cochrane Library, and Virtual Health Library databases through December 2021 was then performed. Subsequently, the primary and secondary outcomes were used in the meta-analysis in RStudio® software. Seventy-two studies were included with 6,011 SCD patients confirmed to have SARS-CoV-2 infection between mid-2020 and early 2022. The mean age of patients was 27 years. During this period, 218 deaths caused by COVID-19 were reported in the studied population, corresponding to an overall case fatality rate of 3%. In addition, 10% of patients with SCD were admitted to the ICU after complications caused by COVID-19, and 4% of them required invasive ventilatory support. In conclusion, the high fatality rate, intensive care unit admission and need for mechanical ventilation due to COVID-19 in young patients with SCD indicate that this population is at high risk for severe disease progression.
Topics: Humans; Adult; COVID-19; SARS-CoV-2; Pandemics; Influenza A Virus, H1N1 Subtype; Anemia, Sickle Cell
PubMed: 37325879
DOI: 10.1080/03630269.2023.2219847 -
The Cochrane Database of Systematic... Dec 2019Sickle cell disease is the most common single gene disorder and the commonest haemoglobinopathy found with high prevalence in many populations across the world.... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Sickle cell disease is the most common single gene disorder and the commonest haemoglobinopathy found with high prevalence in many populations across the world. Management of dental complications in people with sickle cell disease requires special consideration for three main reasons. Firstly, dental and oral tissues are affected by the blood disorder resulting in several oro-facial abnormalities. Secondly, living with a haemoglobinopathy and coping with its associated serious consequences may result in individuals neglecting their oral health care. Finally, the treatment of these oral complications must be adapted to the systemic condition and special needs of these individuals, in order not to exacerbate or deteriorate their general health. Guidelines for the treatment of dental complications in this population who require special care are unclear and even unavailable in many aspects. Hence this review was undertaken to provide a basis for clinical care by investigating and analysing the existing evidence in the literature for the treatment of dental complications in people with sickle cell disease. This is an update of a previously published review.
OBJECTIVES
To assess methods of treating dental complications in people with sickle cell disease.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Review Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Date of last search: 01 August 2019. Additionally, we searched nine online databases (PubMed, Google Scholar, ClinicalTrials.gov, WHO International Clinical Trials Registry Platform, Literature in the Health Sciences in Latin America and the Caribbean database, African Index Medicus, Index Medicus for South East Asia Region, Index Medicus for the Eastern Mediterranean Region, Indexing of Indian Medical Journals). We also searched the reference lists of relevant articles and reviews and contacted haematologists, experts in fields of dentistry, organizations, pharmaceutical companies and researchers working in this field. Date of last search: 07 November 2019.
SELECTION CRITERIA
We searched for published or unpublished randomised controlled studies of treatments for dental complications in people with sickle cell disease.
DATA COLLECTION AND ANALYSIS
Two review authors intended to independently extract data and assess the risk of bias of the included studies using standard Cochrane methodologies; however, no studies were identified for inclusion in the review.
MAIN RESULTS
No randomised controlled studies were identified.
AUTHORS' CONCLUSIONS
This Cochrane Review did not identify any randomised controlled studies assessing interventions for the treatment of dental complications in people with sickle cell disease. There is an important need for randomised controlled studies in this area, so as to identify the most effective and safe method for treating dental complications in people with sickle cell disease.
Topics: Anemia, Sickle Cell; Craniofacial Abnormalities; Dental Care; Humans; Randomized Controlled Trials as Topic; Tooth Diseases
PubMed: 31841224
DOI: 10.1002/14651858.CD011633.pub3 -
Hemoglobin Jul 2022Iron chelation therapy (ICT) is essential to prevent complications of iron overload in patients with transfusion-dependent thalassemia. However, the role that adherence... (Review)
Review
Iron chelation therapy (ICT) is essential to prevent complications of iron overload in patients with transfusion-dependent thalassemia. However, the role that adherence to ICT plays in health-related outcomes is less well known. Our objectives were to identify adherence rates of ICT, and to assess methods of measurement, predictors of adherence, and adherence-related health outcomes in the literature published between 1980 and 2020. Of 543 articles, 43 met the inclusion criteria. Studies measured ICT adherence, predictors, and/or outcomes associated with adherence. Most studies were across multiple countries in Europe and North America ( = 8/43, 18.6%), recruited in clinics ( = 39/43, 90.7%), and focused on β-thalassemia (β-thal) ( = 25/43, 58.1%). Common methods of assessing ICT adherence included patient self-report ( = 24/43, 55.8%), pill count ( = 9/43, 20.9%), prescription refill history ( = 3/43, 7.0%), provider scoring ( = 3/43, 7.0%), and combinations of methods ( = 4/43, 9.3%). Studies reported adherence either in 'categories' with different levels of adherence ( = 24) or 'quantitatively' as a percentage of doses of medication taken out of those prescribed ( = 17). Adherence levels varied (median 91.7%, range 42.0-99.97%). Studies varied in sample size and methods of adherence assessment and reporting, which prohibited meta-analysis. Due to a lack of consensus on how adherence is defined, it is difficult to compare ICT adherence reporting. Further research is needed to establish guidelines for assessing adherence and identifying suboptimal adherence. Behavioral digital interventions have the potential to optimize ICT adherence and health outcomes.
Topics: Adult; Humans; beta-Thalassemia; Chelation Therapy; Iron; Iron Chelating Agents; Iron Overload; Medication Adherence; Thalassemia
PubMed: 35930250
DOI: 10.1080/03630269.2022.2072320 -
Indian Journal of Psychiatry Nov 2023Mood disorders are among the common mental disorders worldwide. Because of the persistence of cytomegalovirus (CMV) in the body and nervous system, this virus can be... (Review)
Review
Mood disorders are among the common mental disorders worldwide. Because of the persistence of cytomegalovirus (CMV) in the body and nervous system, this virus can be activated when the immune system is weakened and continues to exert its destructive effects throughout life. This study aimed to investigate the seroprevalence and association of human cytomegalovirus with mood disorders. Eligible articles were extracted using online international databases Science Direct, Medline, Web of Science, Scopus, and Google Scholar between 2000 and 2023. After quality assessment and specific inclusion and exclusion criteria, a total of eight eligible articles were included in the meta-analysis. Our finding showed that the seropositivity of CMV in mood disorders was 51.6% (95% CI; 42.8-60.4). There were statistical differences between mood disorders and control groups regarding the seropositivity of CMV 1.327% (95% CI; 13.27-10.45). The results of the publication bias using the Egger test confirmed no publication bias in each sub-group. The results of this meta-analysis study demonstrated that CMV infection might have associations with the incidence of mood disorders. Furthermore, we found that there were statistical differences between mood disorders and control groups regarding the seropositivity of CMV.
PubMed: 38249142
DOI: 10.4103/indianjpsychiatry.indianjpsychiatry_672_23 -
Developmental Medicine and Child... Feb 2022To summarize developmental delay among infants and toddlers with sickle cell disease (SCD).
AIM
To summarize developmental delay among infants and toddlers with sickle cell disease (SCD).
METHOD
This systematic review included studies that reported developmental outcomes of children with SCD between 0 months and 48 months of age and followed standards set forth by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
Ten studies were included, describing 596 unique developmental assessments. The rate of developmental delay ranged from 17.5% to 50% and increased with age. Cognition was the only domain included in all studies and the most frequently identified delay. One study reported that more severe SCD genotypes predicted worse development, while five studies reported no difference in rates of developmental delay across genotypes.
INTERPRETATION
These findings emphasize the need for standardized screening to identify children with SCD at risk of delay at a young age to facilitate appropriate referrals for therapeutic intervention. Frequent and comprehensive developmental screening is necessary among all SCD genotypes.
Topics: Anemia, Sickle Cell; Child, Preschool; Developmental Disabilities; Humans; Infant; Infant, Newborn
PubMed: 34535892
DOI: 10.1111/dmcn.15048