Regional consensus opinion for the management of Beta thalassemia major in the Arabian Gulf area

Qari, Mohamad; Wali, Yasser; Albagshi, Muneer; Alshahrani, Mohammad; Alzahrani, Azzah; Al-Hijji, Ibrahim; Almomen, Abdulkareem; Al‐Jefri, Abdullah; Saeed, Hussain H. Al; Alaqel, Shaker; Rustumani, Ahmad Al; Mahour, Khoutir; Mousa, Shaker A.

doi:10.1186/1750-1172-8-143

Cited by 20 publications

(18 citation statements)

References 87 publications

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“… Avoiding adverse reactions of blood transfusion therapy with appropriate red cell antigen typing, cross matching, and screening for new antibody production. 7 …”

Section: Transfusion Protocolmentioning

confidence: 99%

Reducing the iron burden and improving survival in transfusion-dependent thalassemia patients: current perspectives

Bayanzay¹,

Alzoebie²

2016

JBM

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Hypertransfusion regimens for thalassemic patients revolutionized the management of severe thalassemia; transforming a disease which previously led to early infant death into a chronic condition. The devastating effect of the accrued iron from chronic blood transfusions necessitates a more finely tuned approach to limit the complications of the disease, as well as its treatment. A comprehensive approach including carefully tailored transfusion protocol, continuous monitoring and assessment of total body iron levels, and iron chelation are currently the mainstay in treating iron overload. There are also indications for ancillary treatments, such as splenectomy and fetal hemoglobin induction. The main cause of death in iron overload continues to be related to cardiac complications. However, since the widespread use of iron chelation started in the 1970s, there has been a general improvement in survival in these patients.

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“… Avoiding adverse reactions of blood transfusion therapy with appropriate red cell antigen typing, cross matching, and screening for new antibody production. 7 …”

Section: Transfusion Protocolmentioning

confidence: 99%

Reducing the iron burden and improving survival in transfusion-dependent thalassemia patients: current perspectives

Bayanzay¹,

Alzoebie²

2016

JBM

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“…28 The observed difference might be due to a difference in study participants. In the USA, the study was conducted on elderly patients with a mean age of 82 ± 9 which may be the cause of the higher incidence, but our study participant had median age 31 years old and reported with a few elder age groups (16,4.2%) whereas, in Nigeria, all participants were pregnant women (86% of the subjects were multigravid). Moreover, most of the transfusions administered were women who had an incomplete abortion and placenta previa with fetal-maternal hemorrhage which might be associated with ATR.…”

Section: Discussionmentioning

confidence: 92%

“…Blood component transfusion is an essential part of modern health care which can save lives and improve health. 1,2 The main goal of blood transfusion is to treat chronic anemia and ineffective erythropoietin, 3,4 coagulopathy, life-threatening bleeding diathesis. It is also used for the treatment of von Willebrand's disease, Hemophilia A, Factor XIII deficiency and hypofibrinogenemia, especially when recombinant products are not available.…”

Section: Introductionmentioning

confidence: 99%

<p>Proportion of Acute Transfusion Reaction and Associated Factors Among Adult Transfused Patients at Felege Hiwot Compressive Referral Hospital, Bahir Dar, Northwest Ethiopia: A Cross-Sectional Study</p>

Gelaw¹,

Woldu²,

Melku³

2020

JBM

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Introduction: Acute transfusion reactions are adverse events occurring within 24 hrs of transfusion and cause simple-to-severe complications. They may vary with the blood component transfused and recipient factors. In Ethiopia, there is a limited evidence about the incidence and associated factors of transfusion reactions. Objective: To determine the proportion of acute transfusion reactions and associated factors among adult transfused patients. Methods: A total of 384 study participants were included in the study. Structured questionnaires were used for socio-demographic and past medical history data collection. Vital signs were measured as a baseline for every study participants and monitored and followed for 24 hrs. Laboratory tests like complete blood count, direct anti-human globulin test and urine hemoglobin were done as a baseline for suspected patients. Cross-match, blood grouping, and blood culture for patients and donors sample were also done for suspected patients. Descriptive statistics, bivariable and multivariable binary logistic regression were analyzed using SPSS version 20. P-value of <0.05 in the multivariable model was considered as statistically significant. Results: Acute transfusion reactions were observed in 5.2% of patients. Of total cases of acute transfusion reaction, the majority developed allergic reactions (65%) and febrile non-hemolytic transfusion reaction (30%). It was significantly associated with transfusion history (AOR=3.4; 95% CI: 1.2-9.7), abortion history (AOR=5.0; 95% CI: 1.5-16.4), longer blood storage time (AOR=5.1; 95% CI: 1.7-15.2) and receiving three or more unit of blood (AOR= 4.1; 95% CI: 1.5-11.2). Conclusion: Acute transfusion reactions were observed in 5.2% of patients (allergic reactions (65%), febrile non-hemolytic transfusion reaction (30%) and alloimmunization (5%)). Patients with a history of transfusion, abortion, transfused with blood stored ≥14 days and multi-transfused patients should be closely monitored.

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“…A study by reported progressive renal failure to be one the main complications in βTM children. 20,21 Mishra 22,23 The use of iron chelation also affect functioning of glomeruli as DFO (Deferoxamine) given intravenously can also cause renal toxicity when given in high dose. However, DFO has decreased the rate of mortality by decreasing the cardiac and other complications.…”

Section: Resultsmentioning

confidence: 99%

Cystatin C an early marker of Glomerular dysfunction in thalassemia major.

Saghir

Riaz²,

Hasan

et al. 2020

TPMJ

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Objectives: The objective of the present study is to investigate the role of cystatin C as an early marker of glomerular dysfunction in thalassemia major. Study Design: Cross sectional comparative study. Setting: Department of Biochemistry Post Graduate Medical institute with the Thalassemia Center in Sir Ganga Ram Hospital Lahore. Period: July 2017 and July 2018. Material & Methods: This study examined 90 male children all between the ages of 5-11, using non probability sampling techniques. The children were grouped as 21 healthy male children as control group I and 69 diagnosed male thalassemia major children further subdivided on the basis of serum ferritin level as group II, serum ferritin level <2500ng/ml, group III, serum ferritin level 2500-5000ng/ml and group IV serum ferritin level >5000ng/ml respectively. Individuals with hereditary renal diseases, on steroid therapy, or other co-morbid renal diseases were excluded from the study. Complete blood analysis, serum ferritin, creatinine and cystatin C were measured by Micro lab 300 and solid phase enzyme linked immune sorbent assay (ELISA) respectively. The results were compared by using SPSS version 20. Results: Group I: n=21 healthy children with ferritin between normal range 105.33 ± 30.03, serum creatinine 0.41 ± 0.05, serum cystatin C 0.57 ± 0.14. eGFR-creatinine 133.38±7.63, eGFR-cystatin C 122.9±17.63. Group II: n=20 (β-TM with ferritin <2500ng/ml). Mean ferritin was 1997.5±300.68 ng/ml (P<0.001), creatinine was 0.43± 0.05, serum cystatin C 0.66 ± 0.05 (P<0.05). eGFR-creatinine 121.45±4.89 P<0.05, eGFR-cystatin C 105.15±6.49 P<0.001. Group III: n= 25 (β-TM with ferritin 2500-5000 ng/ml). Mean ferritin level was 3850.0± 718.18 ng /ml (P<0.001), creatinine was 0.5±.07, cystatin C 0.96±0.13 (P<0.001). eGFR-creatinine 103.29±8.26(P<0.001), eGFR-cystatin C 75.75±10.67 (P<0.001). Group IV: n=24 (β-TM with ferritin >5000 ng/ml). Mean ferritin level was 6311.67±1060.61 ng/ml P value (P<0.001), creatinine was 0.57 ± 0.07, cystatin C 1.11 ± 0.09 (P<0.001). eGFR-creatinine 94.42±8.69 (P<0.001), eGFR cystatin C 64.67±4.23(P<0.001). Conclusion: A highly significant positive relation was found between serum ferritin-cystatin C as compared to creatinine among the study groups II, III and IV and significant inverse relationship between cystatin C and eGFR that concluded cystatin C as an early marker of glomerular dysfunction than creatinine in thalassaemia major children.

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Regional consensus opinion for the management of Beta thalassemia major in the Arabian Gulf area

Cited by 20 publications

References 87 publications

Reducing the iron burden and improving survival in transfusion-dependent thalassemia patients: current perspectives

Reducing the iron burden and improving survival in transfusion-dependent thalassemia patients: current perspectives

<p>Proportion of Acute Transfusion Reaction and Associated Factors Among Adult Transfused Patients at Felege Hiwot Compressive Referral Hospital, Bahir Dar, Northwest Ethiopia: A Cross-Sectional Study</p>

Cystatin C an early marker of Glomerular dysfunction in thalassemia major.

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