Literature DB >> 21834969

Serum ferritin levels, socio-demographic factors and desferrioxamine therapy in multi-transfused thalassemia major patients at a government tertiary care hospital of Karachi, Pakistan.

Haris Riaz1, Talha Riaz, Muhammad Ubaid Khan, Sina Aziz, Faizan Ullah, Anis Rehman, Qandeel Zafar, Abdul Nafey Kazi.   

Abstract

BACKGROUND: Beta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan. Recurrent transfusions lead to iron-overload manifested by increased serum Ferritin levels, for which chelation therapy is required.
FINDINGS: The study was conducted in the Pediatric Emergency unit of Civil Hospital Karachi after ethical approval by the Institutional Review Board of Dow University of Health Sciences. Seventy nine cases of beta thalassemia major were included after a written consent. The care takers were interviewed for the socio-demographic variables and the use of Desferrioxamine therapy, after which a blood sample was drawn to assess the serum Ferritin level. SPSS 15.0 was employed for data entry and analysis.Of the seventy-nine patients included in the study, 46 (58.2%) were males while 33 (41.8%) were females. The mean age was 10.8 (± 4.5) years with the dominant age group (46.2%) being 10 to 14 years. In 62 (78.8%) cases, the care taker education was below the tenth grade. The mean serum Ferritin level in our study were 4236.5 ng/ml and showed a directly proportional relationship with age. Desferrioxamine was used by patients in 46 (58.2%) cases with monthly house hold income significant factor to the use of therapy.
CONCLUSIONS: The mean serum Ferritin levels are approximately ten times higher than the normal recommended levels for normal individuals, with two-fifths of the patients not receiving iron chelation therapy at all. Use of iron chelation therapy and titrating the dose according to the need can significantly lower the iron load reducing the risk of iron-overload related complications leading to a better quality of life and improving survival in Pakistani beta thalassemia major patients.Conflicts of Interest: None.

Entities:  

Year:  2011        PMID: 21834969      PMCID: PMC3162919          DOI: 10.1186/1756-0500-4-287

Source DB:  PubMed          Journal:  BMC Res Notes        ISSN: 1756-0500


  22 in total

Review 1.  Iron overload, public health, and genetics: evaluating the evidence for hemochromatosis screening.

Authors:  M E Cogswell; S M McDonnell; M J Khoury; A L Franks; W Burke; G Brittenham
Journal:  Ann Intern Med       Date:  1998-12-01       Impact factor: 25.391

Review 2.  Optimizing iron chelation strategies in beta-thalassaemia major.

Authors:  John B Porter
Journal:  Blood Rev       Date:  2009-12       Impact factor: 8.250

Review 3.  Management of iron overload in the pediatric patient.

Authors:  A Cohen
Journal:  Hematol Oncol Clin North Am       Date:  1987-09       Impact factor: 3.722

4.  Excess alcohol greatly increases the prevalence of cirrhosis in hereditary hemochromatosis.

Authors:  Linda M Fletcher; Jeannette L Dixon; David M Purdie; Lawrie W Powell; Darrell H G Crawford
Journal:  Gastroenterology       Date:  2002-02       Impact factor: 22.682

5.  Antenatal diagnosis of thalassaemia major.

Authors:  D V Fairweather; B Modell; V Berdoukas; B P Alter; D G Nathan; D Loukopoulos; W Wood; J B Clegg; D J Weatherall
Journal:  Br Med J       Date:  1978-02-11

6.  Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine.

Authors:  Caterina Borgna-Pignatti; Simone Rugolotto; Piero De Stefano; Huaqing Zhao; Maria Domenica Cappellini; Giovanni Carlo Del Vecchio; Maria Antonietta Romeo; Gian Luca Forni; Maria Rita Gamberini; Roberta Ghilardi; Antonio Piga; Avital Cnaan
Journal:  Haematologica       Date:  2004-10       Impact factor: 9.941

7.  Screening for hemochromatosis by measuring ferritin levels: a more effective approach.

Authors:  Jill Waalen; Vincent J Felitti; Terri Gelbart; Ernest Beutler
Journal:  Blood       Date:  2007-11-19       Impact factor: 22.113

8.  Survival in medically treated patients with homozygous beta-thalassemia.

Authors:  N F Olivieri; D G Nathan; J H MacMillan; A S Wayne; P P Liu; A McGee; M Martin; G Koren; A R Cohen
Journal:  N Engl J Med       Date:  1994-09-01       Impact factor: 91.245

9.  Noninvasive prediction of cirrhosis in C282Y-linked hemochromatosis.

Authors:  Melanie Beaton; Dominique Guyader; Yves Deugnier; Romain Moirand; Subrata Chakrabarti; Paul Adams
Journal:  Hepatology       Date:  2002-09       Impact factor: 17.425

Review 10.  A risk-benefit assessment of iron-chelation therapy.

Authors:  J B Porter
Journal:  Drug Saf       Date:  1997-12       Impact factor: 5.228

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  7 in total

1.  Iron overload in Beta thalassaemia major and intermedia patients.

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3.  Quality of Life (QoL) and the Factors Affecting it in Transfusion-dependent Thalassemic Children.

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Journal:  Indian J Pediatr       Date:  2018-05-12       Impact factor: 1.967

4.  Sociodemographic determinants associated with parental knowledge of screening services for thalassemia major in Lahore.

Authors:  Iram Manzoor; Rubeena Zakar
Journal:  Pak J Med Sci       Date:  2019 Mar-Apr       Impact factor: 1.088

5.  Epidemiology and risk factors of transfusion transmitted infections in thalassemia major: a multicenter study in Pakistan.

Authors:  Humaira Yasmeen; Shahida Hasnain
Journal:  Hematol Transfus Cell Ther       Date:  2019-06-28

6.  Body iron status of children and adolescents with transfusion dependent β-thalassaemia: trends of serum ferritin and associations of optimal body iron control.

Authors:  Tharindi Suriapperuma; Ravindu Peiris; Chamila Mettananda; Anuja Premawardhena; Sachith Mettananda
Journal:  BMC Res Notes       Date:  2018-08-02

7.  Prevalence of Transfusion Transmitted Infections and the Quality of Life in β-thalassemia Major Patients.

Authors:  May Al-Moshary; Eman Al-Mussaed; Adnan Khan
Journal:  Cureus       Date:  2019-11-12
  7 in total

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