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1.
BMJ Case Rep ; 13(12)2020 Dec 22.
Artigo em Inglês | MEDLINE | ID: mdl-33370971

RESUMO

A 12-year-old patient of thalassaemia major developed autoimmune cytopaenia after undergoing haematopoietic stem cell transplantation. She was started on cyclosporine (CsA) in view of poor response to steroids. She developed CsA toxicity manifesting as gum hyperplasia with multiple episodes of gum bleed. During endotracheal intubation for an elective splenectomy, she developed significant bleeding from gums requiring massive transfusion. Postoperatively the gum bleed persisted even after embolisation of facial artery and multiple transfusions. The catastrophic sequelae include transfusion-related lung injury, acute circulatory failure with subsequent cardiac arrest and death. Gum hyperplasia is a commonly reported toxic effect of CsA. Lethal presentations of this toxicity with such severity are limited in the medical literature. Evaluation of the patient's medical and laboratory records, along with a review of literature, was very helpful in understanding more about the toxicity of CsA.


Assuntos
Ciclosporina/efeitos adversos , Hemorragia Gengival/diagnóstico , Hiperplasia Gengival/induzido quimicamente , Parada Cardíaca/etiologia , Imunossupressores/efeitos adversos , Lesão Pulmonar Aguda Relacionada à Transfusão/etiologia , Transfusão de Sangue , Criança , Evolução Fatal , Feminino , Hemorragia Gengival/etiologia , Hemorragia Gengival/terapia , Hiperplasia Gengival/complicações , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Intubação Intratraqueal/efeitos adversos , Pancitopenia/tratamento farmacológico , Pancitopenia/imunologia , Índice de Gravidade de Doença , Choque , Talassemia/terapia
2.
Arch Osteoporos ; 15(1): 148, 2020 09 21.
Artigo em Inglês | MEDLINE | ID: mdl-32955681

RESUMO

Despite the significant improvements in the management of thalassemia, there are growing concerns regarding their long-term complications. We showed that low bone mass is one of the most prevalent complications among these patients. Insufficient physical activity and hypogonadism are the main possible associated factors followed by DM and insufficient sun exposure. PURPOSE: Despite the significant improvements in the management of transfusion-dependent thalassemia (TDT), there are growing concerns regarding their long-term complications. METHODS: This cross-sectional study included 615 TDT patients who were registered and followed in a comprehensive thalassemia clinic in Southern Iran. We measured serum biochemical tests and bone mineral density in all patients. We recorded physical activity and sun exposure subjectively, and an endocrinologist visited and examined all the patients. A group of age- and gender-matched healthy volunteers participated in the study as the control group. RESULTS: The mean age of the studied population was 28.4 ± 7.7 years, and 55.8% were female. The prevalence of vitamin D deficiency was 45.6% and 54.4% in TDT patients and the control group. A portion of TDT patients suffered from different endocrinopathies, which included hypogonadism (49.8%), diabetes mellitus (17.2%), hypoparathyroidism (14.6%), and hypothyroidism (6.3%). The prevalence of low bone mass in patients with TDT was 48.3 and 74.6% in the femoral and lumbar bones, respectively. Low physical activity, insufficient sun exposure, diabetes mellitus, and hypogonadism were associated with low bone mass. CONCLUSION: Low bone mass is highly prevalent among TDT patients in Southern Iran. Insufficient physical activity and hypogonadism are the main possible associated factors, followed by DM and insufficient sun exposure.


Assuntos
Transfusão de Sangue , Densidade Óssea , Talassemia/terapia , Adulto , Colecistectomia , Estudos Transversais , Feminino , Humanos , Irã (Geográfico)/epidemiologia , Masculino , Prevalência , Esplenectomia , Talassemia/epidemiologia , Resultado do Tratamento , Deficiência de Vitamina D/epidemiologia , Adulto Jovem
3.
Ann Hematol ; 99(10): 2289-2294, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32737633

RESUMO

Iron overload-induced cardiomyopathy is the leading cause of death in patients with transfusion-dependent thalassemia (TDT). The mortality is extremely high in these patients with severe cardiac complications, and how to rescue them remains a challenge. It is reasonable to use combined chelation with deferiprone (L1) and deferoxamine (DFO) because of their shuttle and synergistic effects on iron chelation. Here, seven consecutive patients with TDT who had severe cardiac complications between 2002 and 2019 and received combined chelation therapy with oral high-dose L1 (100 mg/kg/day) and continuous 24-h DFO infusion (50 mg/kg/day) in our hospital were reported. Survival for eight consecutive patients receiving DFO monotherapy for their severe cardiac complications between 1984 and 2001 was compared. We found that combined chelation therapy with high-dose L1 and DFO was efficient to improve survival and cardiac function in patients with TDT presenting severe cardiac complications. Reversal of arrhythmia to sinus rhythm was noted in all patients. Their 1-month follow-up left ventricular ejection fraction increased significantly (P < 0.001). There were no deaths, and all patients were discharged from hospital with good quality of life. In contrast, all the eight patients receiving DFO monotherapy died (P < 0.001). Accordingly, combined chelation therapy with high-dose L1 and DFO should be considered in patients with TDT presenting cardiac complications.


Assuntos
Arritmias Cardíacas/tratamento farmacológico , Terapia por Quelação/métodos , Deferiprona/uso terapêutico , Desferroxamina/uso terapêutico , Insuficiência Cardíaca/tratamento farmacológico , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Talassemia/terapia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/fisiopatologia , Transfusão de Sangue , Deferiprona/administração & dosagem , Desferroxamina/administração & dosagem , Avaliação de Medicamentos , Quimioterapia Combinada , Feminino , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Quelantes de Ferro/administração & dosagem , Sobrecarga de Ferro/etiologia , Masculino , Qualidade de Vida , Estudos Retrospectivos , Talassemia/complicações , Reação Transfusional , Resultado do Tratamento , Função Ventricular Esquerda
4.
Ann Hematol ; 99(9): 1967-1977, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32621178

RESUMO

Thalassemia is characterized by a defect in the synthesis of one or more of the globin subunits of hemoglobin. This defect results in imbalance in the α/ß-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and iron overload. With advances in diagnosis, treatment, and transfusion support, the prognosis of patients with thalassemia has improved over the past few decades. An increasing number of patients with thalassemia is living with long-term complications, including cardiomyopathy, chronic liver disease, endocrinopathy, and infections. In this paper, we review common complications that bring the patient with thalassemia to urgent or emergent medical attention. We also discuss the aspects of emergency care that are most relevant while caring for the patient with thalassemia in the emergency department.


Assuntos
Serviços Médicos de Emergência/tendências , Serviço Hospitalar de Emergência/tendências , Doenças Raras/diagnóstico por imagem , Doenças Raras/terapia , Talassemia/diagnóstico por imagem , Talassemia/terapia , Betacoronavirus , Transfusão de Sangue/métodos , Transfusão de Sangue/tendências , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/epidemiologia , Cardiomiopatias/terapia , Infecções por Coronavirus/diagnóstico por imagem , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/terapia , Diagnóstico Diferencial , Serviços Médicos de Emergência/métodos , Humanos , Hepatopatias/diagnóstico por imagem , Hepatopatias/epidemiologia , Hepatopatias/terapia , Pandemias , Pneumonia Viral/diagnóstico por imagem , Pneumonia Viral/epidemiologia , Pneumonia Viral/terapia , Doenças Raras/epidemiologia , Talassemia/epidemiologia
5.
Pediatr Blood Cancer ; 67(9): e28483, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32568454

RESUMO

BACKGROUND: The role of splenectomy prior to hematopoietic stem cell transplantation (HSCT) is controversial. Only few studies compared the outcomes of splenectomized and nonsplenectomized children with transfusion-dependent thalassemia (TDT) undergoing allogeneic HSCTs. METHODS: A retrospective analysis was undertaken on a transplantation cohort of TDT patients; August 1987-December 2014 to compare transplant outcomes between splenectomized and nonsplenectomized groups. RESULTS: Ninety-six transplants in 86 TDT patients were analyzed. Sixteen patients were splenectomized before HSCTs. The splenectomized patients were significantly older (8.0 ± 1.9 vs 4.7 ± 0.6 years; P = 0.001), had larger livers and spleens (P = 0.001), and had a significantly shorter neutrophil engraftment time (absolute neutrophil count > 500/mm3 ; 15.0 ± 2.3 vs 19.2 ± 1.3 days; P = 0.004). Graft rejection occurred in 13.8% of the nonsplenectomized group, but not among the splenectomized patients. Though the splenectomized group's mortality rate was higher (25.0% vs 8.8%), this was not statistically significant (P = 0.491). The main causes of death in both groups were severe infections. The five-year overall survival (OS) rate was better for the nonsplenectomized group (91.78% vs 75.00%; P = 0.06). CONCLUSIONS: Although splenectomies prior to HSCT for the TDT patients in our cohort were associated with faster neutrophil engraftments and lower rejection rates, they did not produce significantly better OS or affect the mortality. As the splenectomies did not provide any distinct advantages, this procedure should not be routinely performed as a pre-HSCT regimen for TDT patients with splenomegaly. Better pre-HSCT preparation for TDT patients, including early and adequate blood transfusions to avoid splenomegaly, is recommended.


Assuntos
Rejeição de Enxerto/mortalidade , Transplante de Células-Tronco Hematopoéticas , Sistema de Registros , Esplenectomia , Talassemia/mortalidade , Talassemia/terapia , Adolescente , Aloenxertos , Transfusão de Sangue , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos Retrospectivos , Taxa de Sobrevida
7.
Lab Med ; 51(2): 143-150, 2020 Mar 10.
Artigo em Inglês | MEDLINE | ID: mdl-32155272

RESUMO

BACKGROUND: Cardiomyopathic manifestations induced by continuous blood transfusion are the leading cause of death among patients with thalassemia major (TM). Despite introduction of chelation therapy, heart failure after cardiomyopathic manifestations is still a major threat to patients. METHODS: We performed a search of relevant English-language literature, retrieving publications from the PubMed database and the Google Scholar search engine (2005-2018). We used "thalassemia major", "cardiomyopathy", "iron overload", "cardiac magnetic resonance T2" "chelation therapy", and "iron burden" as keywords. RESULTS: The results of the studies we found suggest that cardiac hepcidin is a major regulator of iron homeostasis in cardiac tissue. Unlike previous assumptions, the heart appears to have a limited regeneration capability, originating from a small population of hypoxic cardiomyocytes. CONCLUSIONS: Oxygen levels determine cardiomyocyte gene-expression patterns. Upregulation of cardiac hepcidin in hypoxia preserves cardiomyocytes from forming out of reactive oxygen species catalyzed by free cellular iron in cardiomyocytes. Using the limited regeneration capacity of cardiac cells and gaining further understanding of the cellular aspects of cardiomyopathic manifestations may help health care professionals to develop new therapeutic strategies.


Assuntos
Cardiomiopatias/diagnóstico , Cardiomiopatias/terapia , Terapia por Quelação/métodos , Testes Diagnósticos de Rotina/métodos , Gerenciamento Clínico , Sobrecarga de Ferro/complicações , Talassemia/complicações , Cardiomiopatias/patologia , Cardiomiopatias/fisiopatologia , Humanos , Talassemia/terapia
8.
Indian J Pediatr ; 87(1): 75-81, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31620986

RESUMO

Beta thalassemia major (TM) is the most frequent form of transfusion-dependent inherited anemia in India. The thalassemia syndromes exhibit enormous variability in their genetic basis and phenotypic expression. The authors recommend that the diagnosis of TM or non-transfusion-dependent thalassemia (NTDT) should not be based on a one-time assessment. Many patients have a chronic anemia that is not severe enough to justify regular transfusions, but the clinical course can evolve with age. Continued observation may reveal that some patients who are considered NTDT will benefit from transfusions later in life. Clinical decision making can be influenced by the perceived difficulty in access to a safe blood supply and the cost of therapy. Here, authors present selected case scenarios that address common issues in the management of TM or NTDT. The recommendations are based on published evidence where available or on the authors' shared experience. Among the topics under discussion are deciding when to start regular transfusions, the role of hydroxyurea in TM, the procedure for blood administration, the use of deferasirox for chelation and monitoring of side effects, the role of splenectomy, and the prospects for gene therapy. In order to achieve an optimal outcome with blood transfusions and chelation therapy over the lifetime, it is essential to adhere to the current guidelines for the management of thalassemia.


Assuntos
Talassemia/terapia , Transfusão de Sangue/métodos , Terapia por Quelação/métodos , Humanos , Hidroxiureia/uso terapêutico , Índia , Esplenectomia/métodos , Talassemia beta/terapia
9.
Transfusion ; 60(1): 16-25, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31758587

RESUMO

BACKGROUND: Children with transfusion dependent anemia, such as sickle cell disease (SCD) and thalassemia, are at an increased risk for developing red blood cell (RBC) alloantibodies due to their lifelong need for transfusion therapy. With the advent of genotyping, extended RBC antigen typing can be incorporated into chronic transfusion therapy programs (CTTPs) to improve patient care and provide antigen matched blood for this population of patients. STUDY DESIGN AND METHODS: The hospital, blood center (BC), and hematology clinic caring for children requiring long-term transfusion support developed a CTTP. Genotyping was performed at entry to determine patient RBC antigen type. Limited versus extended antigen matching of transfusions was provided based on known RBC antibodies. RESULTS: Fifty patients with the following disorders were enrolled: 20 with SCD, 23 with thalassemia, and 7 with other disorders. At enrollment, nine (18%) had RBC alloantibodies, including six (30%) of patients with SCD and three (13%) with thalassemia. Two children developed antibodies after enrollment; one warm autoantibody following limited "CEK" matched RBCs and one patient with a hemizygous variant RHD allele developed anti-D. Six (30%) patients with SCD had variant RHCE alleles; two had homozygous variant alleles and four had a variant present along with a wild type allele. CONCLUSION: We demonstrate how a CTTP can be developed in a community hospital through collaboration with the blood supplier, hospital, and clinical care team. A model of incorporating RBC genotyping informs risk for alloimmunization and allows consideration of transfusion strategy for providing prophylactic antigen matched blood.


Assuntos
Alelos , Anemia Falciforme , Transfusão de Eritrócitos , Eritrócitos/metabolismo , Técnicas de Genotipagem , Sistema do Grupo Sanguíneo Rh-Hr , Talassemia , Adolescente , Anemia Falciforme/sangue , Anemia Falciforme/genética , Anemia Falciforme/terapia , Criança , Pré-Escolar , Feminino , Genótipo , Humanos , Isoanticorpos/sangue , Masculino , Sistema do Grupo Sanguíneo Rh-Hr/sangue , Sistema do Grupo Sanguíneo Rh-Hr/genética , Talassemia/sangue , Talassemia/genética , Talassemia/terapia
10.
Ann Ist Super Sanita ; 55(4): 351-356, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31850862

RESUMO

INTRODUCTION: Blood transfusion is a lifesaving procedure for patients affected by hematological diseases or hemorrhage risk. AIM: This retrospective study was aimed to evaluate clinical safety of pediatric transfusions by comparing the frequency of adverse events caused by apheretic blood components vs whole blood. METHODS: From 2011 to 2015, 214 patients (blood malignancy patients, n = 144 and thalassemic patients, n = 70) received 12 531 units of blood components. The adverse acute reactions occurred during patient hospitalization were reported to the Hemovigilance system and assessed by fitting a logistic mixed-effect model. RESULTS: A total of 33 (0.3%) adverse acute events occurred. Odds ratio (OR) of adverse events from apheresis vs whole blood transfusion adjusted by patient classification was not statistically significant (OR [95% CI], 0.75 [0.23-2.47]). CONCLUSION: Our findings showed no significant differences in the prevalence of adverse acute events between blood component collected by apheresis vs whole blood in our study center.


Assuntos
Transfusão de Sangue/estatística & dados numéricos , Reação Transfusional/epidemiologia , Adolescente , Remoção de Componentes Sanguíneos , Transfusão de Componentes Sanguíneos/efeitos adversos , Transfusão de Componentes Sanguíneos/estatística & dados numéricos , Segurança do Sangue , Transfusão de Sangue/métodos , Criança , Feminino , Neoplasias Hematológicas/terapia , Humanos , Modelos Logísticos , Masculino , Razão de Chances , Prevalência , Distribuição Aleatória , Estudos Retrospectivos , Talassemia/terapia , Adulto Jovem
11.
Med J Malaysia ; 74(5): 365-371, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31649210

RESUMO

INTRODUCTION: Thalassemias are the most common human monogenic disorders in the world. Regular blood transfusion and increased intestinal absorption of iron among thalassemia patients will lead to iron overload, which will not only markedly decrease their life expectancy but also pose a heavy burden to the healthcare system. The objective of this study was to evaluate the level of knowledge, attitude and practice towards dietary iron among thalassemia patients and their caregivers. METHODS: An analytical cross-sectional study using purposive sampling method was conducted at eight thalassemia societies in Peninsular Malaysia. 260 respondents comprised of patients and caregivers were assessed with two separate sets of questionnaires. RESULTS: Knowledge on dietary iron among the respondents was unsatisfactory, despite them having good knowledge on thalassemia disorder. Female patients were found to have better dietary knowledge, attitude and practice compared to males. The percentage of caregivers with good attitude and good practice were significantly higher compared to adult patients. Caregivers with children on iron chelators were noted to have better dietary attitude and practice. Thalassemia knowledge and children on vitamins were found to be the predictors of dietary knowledge among the patients and caregivers respectively. CONCLUSION: The level of knowledge on dietary iron among the patients and caregivers was unsatisfactory in spite of their attitude and practice towards dietary iron were good. Effective delivery of dietary information to the patients and caregivers is essential to enable them to choose a healthy diet for their condition.


Assuntos
Cuidadores/normas , Conhecimentos, Atitudes e Prática em Saúde , Ferro na Dieta/farmacologia , Talassemia/terapia , Adolescente , Adulto , Idoso , Estudos Transversais , Suplementos Nutricionais , Feminino , Humanos , Incidência , Malásia/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Inquéritos e Questionários , Talassemia/epidemiologia , Adulto Jovem
13.
Acta Biomed ; 90(3): 225-237, 2019 09 06.
Artigo em Inglês | MEDLINE | ID: mdl-31580308

RESUMO

BACKGROUND: More than five decades ago, thalassemia major (TDT) was fatal in the first decade of life. Survival and quality of life have improved progressively thanks to the implementation of a significant advance in diagnostic and therapeutic methods, consisting mainly of a frequent transfusion program combined with intensive chelation therapy. Improvement also includes imaging methods used to measure liver and cardiac iron overload. Improved survival has led to a growing number of adults requiring specialised care and counselling for specific life events, such as sexual maturity and acquisition of a family. AIMS OF THE STUDY: The main aim is to present the results of a survey on the marital and paternity status in a large population of adult males with TDT and NTDT living in countries with a high prevalence of thalassemia and a review of current literature using a systematic search for published studies. RESULTS: Ten out of 16 Thalassemia Centres (62.5%) of the ICET-A Network, treating a total of 966 male patients, aged above 18 years with ß- thalassemias (738 TDT and 228 NTDT), participated in the study. Of the 966 patients, 240 (24.8%) were married or lived with partners, and 726 (75.2%) unmarried. The mean age at marriage was 29.7 ± 0.3 years. Of 240 patients, 184 (76.6%) had children within the first two years of marriage (2.1 ± 0.1 years, median 2 years, range 1.8 - 2.3 years). The average number of children was 1.32 ± 0.06 (1.27 ± 0.07 in TDT patients and 1.47 ± 0.15 in NTDT patients; p: >0.05). Whatever the modality of conception, 184 patients (76.6%) had one or two children and 1 NTDT patient had 6 children. Nine (4.8%) births were twins. Of 184 patients, 150 (81.5%) had natural conception, 23 (12.5%) required induction of spermatogenesis with gonadotropins (hCG and hMG), 8 (4.3%) needed intracytoplasmic sperm injection (ICSI) and 3 adopted a child. 39 patients with TDT and NTDT asked for medical help as they were unable to father naturally: 7 TDT patients (17.9%) were azoospermic, 17 (37.7%) [13 with TDT and 4 with NTDT] had dysspermia and 15 (33.3%) [13 with TDT and 2 with NTDT] had other "general medical and non-medical conditions". CONCLUSIONS: Our study provides detailed information in a novel area where there are few contemporary data. Understanding the aspects of male reproductive health is important for physicians involved in the care of men with thalassemias to convey the message that prospects for fatherhood are potentially good due to progressive improvements in treatment regimens and supportive care.


Assuntos
Transfusão de Sangue , Estado Civil , Paternidade , Talassemia/terapia , Adulto , Comorbidade , Ferritinas/sangue , Humanos , Masculino , Talassemia/sangue
14.
BMJ Health Care Inform ; 26(1)2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31533923

RESUMO

OBJECTIVES: To investigate the impact of electronic medical record (EMR) on improvement of healthcare parameters in a thalassaemia centre located at the Fujairah Hospital, Fujairah, UAE. MATERIALS AND METHODS: A hospital-wide EMR system (Wareed) was implemented across the hospitals in the Ministry of Health and Prevention, UAE, including two major thalassaemia centres. We aim to investigate the impact of this intervention across a number of healthcare parameters over two quarters (before and after implementation of the system). RESULTS: Since preimplementation data were not available for one facility, comparisons were made between parameters in two quarters in Fujairah hospital only. After introduction of Wareed, we found an increase in number of appointments (12%) (p=0.00), decrease in the number of appointment cancellations due to non-availability of blood products (p=0.02), reduction in the time to cannulation (p=0.00), decrease in number of physician days (p=0.295) among other parameters observed. DISCUSSION: Research shows that EMR systems have a positive impact on reduction in medical expenditure, improvement of healthcare quality and overall health outcomes. thalassaemia is highly prevalent in the Middle Eastern countries and drains the medical, social and financial resources of these nations. Our study is an attempt to create an insight into the difference in healthcare parameters before and after introduction of the system. CONCLUSION: Being the first of a kind in this region, our study created favourable evidence that introduction of an EMR has an overall positive impact on the healthcare delivery system for thalassaemia care.


Assuntos
Registros Eletrônicos de Saúde/organização & administração , Segurança do Paciente/estatística & dados numéricos , Melhoria de Qualidade/organização & administração , Talassemia/terapia , Agendamento de Consultas , Cateterismo/estatística & dados numéricos , Procedimentos Clínicos/normas , Gastos em Saúde/estatística & dados numéricos , Humanos , Tempo de Internação/estatística & dados numéricos , Fatores de Tempo , Emirados Árabes Unidos , Listas de Espera
16.
Expert Rev Hematol ; 12(11): 997-1003, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31486684

RESUMO

Objectives: Iron overload might lead to bone loss in transfusion-dependent beta-thalassemia (TDT) patients. To investigate the role of iron chelation therapy (ICT) on bone mineral density (BMD) of TDT patients suffering from iron overload, the authors compared the efficacy of five different iron chelation regimens through assessing serum ferritin and BMD.Methods: In 256 consecutive TDT patients, BMD was measured by dual-energy X-ray absorptiometry in lumbar spine and femoral neck regions. Treatment outcome of five iron chelation regimens including Deferoxamine (DFO), Deferiprone (DFP), Deferasirox (DFX), and combination therapy was evaluated to compare the mean differences of serum ferritin and BMD indices pre- and post-treatment during 12-months follow-up period.Results: No significant difference was observed in DXA characteristics and serum ferritin level changes between ICT groups, but combination of DFO and DFX had the best outcome in improving bone mass through assessing each group individually.Conclusion: Combination therapy with DFX and DFO had the highest impact on reducing serum ferritin, however insignificant, and improving bone loss in both lumbar spine and femoral neck in comparison with other regimens. A randomized prospective clinical trial is advised to accurately assess the efficacy of iron chelation regimens on BMD measurements of TDT patients.


Assuntos
Absorciometria de Fóton , Transfusão de Sangue , Colo do Fêmur/efeitos dos fármacos , Quelantes de Ferro/administração & dosagem , Sobrecarga de Ferro , Vértebras Lombares/diagnóstico por imagem , Talassemia , Adulto , Feminino , Humanos , Sobrecarga de Ferro/diagnóstico por imagem , Sobrecarga de Ferro/tratamento farmacológico , Masculino , Tamanho do Órgão , Estudos Retrospectivos , Talassemia/diagnóstico por imagem , Talassemia/terapia
18.
PLoS One ; 14(8): e0220852, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31419232

RESUMO

OBJECTIVES: Our aim was to describe the numbers and distribution of patients with different types of thalassemia and to assess the standards of care in all thalassemia treatment centers throughout Sri Lanka and the success of the ongoing prevention programme. METHODS: This cross-sectional island-wide survey was conducted by two trained medical graduates, who visited each thalassemia center to collect data from every patient, using a standardized form. Data was collected through review of patient registers and clinical records. RESULTS: We collected data on 1774 patients from 23 centers. 1219 patients (68.7%) had homozygous ß-thalassemia, 360 patients (20.3%) had hemoglobin E ß-thalassemia, and 50 patients (2%) had sickle ß-thalassemia. There were unacceptably high serum ferritin levels in almost all centers. The annual number of births of patients with ß-thalassaemia varied between 45-55, with little evidence of reduction over 19 years. CONCLUSIONS: Central coordination of the treatment and ultimately prevention of thalassemia is urgently needed in Sri Lanka. Development of expert centers with designated staff with sufficient resources will improve the quality of care and is preferred to managing patients in multiple small units.


Assuntos
Talassemia/prevenção & controle , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Qualidade da Assistência à Saúde , Sri Lanka/epidemiologia , Talassemia/epidemiologia , Talassemia/terapia , Talassemia beta/epidemiologia , Talassemia beta/prevenção & controle , Talassemia beta/terapia
19.
Transfus Clin Biol ; 26(3): 147-149, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31300265

RESUMO

Thalassemia and sickle cell disease (SCD) are among the most common inherited diseases worldwide. Red blood cell transfusion is a cornerstone of their treatment, but its indications have significantly changed over the past years. New therapies are emerging in both syndromes: among them, hematopoietic stem cell transplantation is now routinely proposed, and gene therapy has shown promising preliminary results.


Assuntos
Transfusão de Eritrócitos , Hemoglobinopatias/terapia , Aloenxertos , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/terapia , Criança , Emergências , Transfusão Total , Terapia Genética , Transplante de Células-Tronco Hematopoéticas , Humanos , Hidroxiureia/uso terapêutico , Insuficiência de Múltiplos Órgãos/terapia , Talassemia/terapia
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