Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease

Chonat, Satheesh; Quinn, Charles T.

doi:10.1007/978-1-4939-7299-9_3

Cited by 30 publications

(30 citation statements)

References 87 publications

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“…Using CHROM agar media have equal or greater sensitivity of detection, superior detection of mixed cultures as a result of better visualization of different species, and enable shorter times to report results. The life expectancy of patients with thalassemia has improved dramatically over the past 50 years owing Improved survival has mainly been achieved by early diagnosis of the disease and proper treatment through optimized antifungal therapy [2,19].…”

Section: Resultsmentioning

confidence: 99%

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Antifungal Susceptibility of Oral Candida Species Isolated from Patients with Thalassemia Major

Kasem

Shokry

Mohamed

et al. 2017

JBMOA

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Background: Invasive fungal infections are the primary cause of morbidity and mortality in patients with thalassemias. This high mortality rate stems, in part, from the prevalence of resistance to antifungal agents. Candida species remain the most common cause of invasive fungal infection in patients with thalassemia. Nevertheless, other Candida species become increasingly spreading and need to be identified to a species level.Objectives: The objectives of the study were to estimate the prevalence of Candida infection among Thalassemia major patients compared to healthy controls and to identify the resistance patterns among the recovered species.Methods: A total of 60 oral samples were collected from two groups: Group I: 40 oral swabs were collected from patients clinically diagnosed with thalassemia major from the out-patient clinics of Pediatric department in Ain Shams University Hospital. Group II: 20 oral swabs were collected from healthy subjects matched in age and sex with the patients' group. The swabs were cultured onto both CHROM and Sabaroud's agar media. The recovered colonies on Sabaroud's agar were identified using Gram stain and Germ tube test while the colonies on CHROM agar were self-identified through color produced according to manufacturer instructions. For all identified colonies API 20 C AUX was used as a confirmatory test. Antifungal Susceptibility testing using Fluconazole (25μg).Voriconazole (1μg).Nystatin (100 units) was used to identify the resistance strains.Results: C. albicans, were isolated from 69.2% of cases while 30.8% were non C. albicans. (23% C. tropicalis and 7.8% C. krusei). Susceptibility testing for all identified colonies revealed that (13.8%) of all isolates were resistant to fluoconazole, (17.3%) to voriconazole and (89.7%) to nystatin. Conclusion:The use of CHROM agar media offers multiple advantages without increasing costs in comparison to other procedures. CHROM agar media have equal or greater sensitivity of detection, superior detection of mixed cultures as a result of better visualization of different species, and enable shorter times to report results. Antifungal susceptibility tests are now required to optimize antifungal treatment to avoid emergence of new resistant strains.

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Section: Resultsmentioning

confidence: 99%

“…However, infection-related deaths in developing and developed countries still occur [2]. Invasive fungal infections are the major cause of infections in patients with thalassemias.…”

Section: Introductionmentioning

confidence: 99%

Antifungal Susceptibility of Oral Candida Species Isolated from Patients with Thalassemia Major

Kasem

Shokry

Mohamed

et al. 2017

JBMOA

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“…However, although HbAS stands as a protective condition for malaria infection, carriers can suffer from exertional rhabdomyolysis and other non-fatal exercise complications, progressing to end-stage renal disease in chronic kidney disease and elevated risk of venous thromboembolism and arterial events [7]. The homozygous state (HbSS) represents the most serious phenotype of sickle cell disease [8][9][10].…”

Section: Sickle Cell Diseasementioning

confidence: 99%

“…Beta-thalassemia usually results from point mutations in globin genes located on the short arm of chromosome 11 [8,10,13]. The clinical severity is also a function of the number and type of genetic defects, ranging from less severe forms such as beta thalassemia trait which is asymptomatic and characterized by mild microcytic, hypochromic anaemia, to more severe forms like beta thalassemia major in which patients suffer from severe anaemia from infancy.…”

Section: Thalassemia Majormentioning

confidence: 99%

“…The clinical severity is also a function of the number and type of genetic defects, ranging from less severe forms such as beta thalassemia trait which is asymptomatic and characterized by mild microcytic, hypochromic anaemia, to more severe forms like beta thalassemia major in which patients suffer from severe anaemia from infancy. Between these two extremes lies beta thalassemia intermedia with a variable phenotype between the two aforementioned [10,13].…”

Section: Thalassemia Majormentioning

confidence: 99%

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Present and Future Strategies with Curative Intent for Hereditary Hemoglobinopathies

Oliveira¹,

Saraiva²,

Costa³

et al. 2019

obm transplant

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Nowadays, hematopoietic stem cell transplantation (HSCT) is a common procedure in hematology units within reference centers, mainly for the treatment of hematological malignancies such as multiple myeloma, lymphoma, and acute leukemia. Nevertheless, HSCT has much wider applications, namely in autoimmune diseases, congenital metabolic defects, and hemoglobinopathies. Thalassemia major and sickle cell disease make up the most frequent hereditary hemoglobinopathies worldwide. Despite advances on the prevention and treatment of complications related to these diseases, the only curative approach available resides in allogeneic HSCT. The main challenges of this treatment remain focused on the toxicity of pre-transplant conditioning regimens and short-term transplant related complications like graft-versus-host disease, infections, and disease recurrence. Thus, it is crucial to establish a balance between the risk vs benefit of HSCT for each patient and follow the available guidelines for both diseases. Recently, gene therapy has become a real alternative to allogeneic HSCT. Recent advances in molecular biology methods have provided more accurate and reliable gene editing techniques such as the CRISP/CAS9 system. The long-term outcome of gene manipulation procedures remains uncertain, especially in

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Development of Healthcare Transition Policy and Concepts

Betz

2021

Transitioning Care From Pediatric to Adult Pulmonology

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Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease

Cited by 30 publications

References 87 publications

Antifungal Susceptibility of Oral Candida Species Isolated from Patients with Thalassemia Major

Antifungal Susceptibility of Oral Candida Species Isolated from Patients with Thalassemia Major

Present and Future Strategies with Curative Intent for Hereditary Hemoglobinopathies

Development of Healthcare Transition Policy and Concepts

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