Keywords : thalassemia major


Growth Retardation in β-Thalassemia Major

Shaima Khidar Abbass; Mohammed Shaker Al-Awady; Qais Abdullah Ali Al-Salehe

Iraqi Postgraduate Medical Journal, 2016, Volume 15, Issue 2, Pages 267-273

ABSTRACT:
BACKGROUND:
Thalassemia is an inherited autosomal recessive blood disease caused by genetic defects result in reduced rate of synthesis of one of the globin chains that make up hemoglobin. The combination of transfusion and chelating therapy has dramatically extended the life expectancy of thalassemic patients.
OBJECTIVE:
The objective of this study is to determine the factors associated with short stature in thalassemic patients and main endocrine complications.
PATIENTS AND METHODS:
A case-control study was performed prospectively in Ibn-Al Balady hospital in (Al- Sader city-Baghdad- Rasafa) for the period extended from the 1st of January to 31st of May (2013). Data were collected from 181 thalassemic patients, their ages were 10 to 20 years old being attended the hospital for regular follow up and blood transfusion. The control in this research work was one of the patients' relatives with the same age after thalassemia was ruled out in the control. Data collected in this study included: age, sex, height and weight were assessed by the National Center of Health statistics (NCHS) growth curves , history of splenectomy, times of blood transfusion , hepatitis infection, type of chelating agent. Serum ferritin, hormones level, fasting and random blood sugar and serum calcium were estimated.
RESULT:
It was founded that 79% of the β-thalassemic patients had short stature (their height equal or less than5rd percentile) with significant relation with high serum ferritin (P-value=0.006). Delayed puberty was the commonest endocrine complication in thalassemic patients (83.9%).
CONCLUSION:
High serum ferritin is associated with growth retardation and many endocrine complications . Aggressive iron-chelating therapy and regular measurement of hormones concentration are necessary for thalassemic patients mainly during puberty to avoid growth retardation.
KEY WORDS: β-thalassemia major, short stature, endocrine complications, growth retardation.

Growth Retardation in β-Thalassemia Major

Qais Abdullah Ali Al-Salehe; Mohammed Shaker Al-Awady; Shaima Khidar Abbass

Iraqi Postgraduate Medical Journal, 2015, Volume 14, Issue 2, Pages 267-273

ABSTRACT:
BACKGROUND:
Thalassemia is an inherited autosomal recessive blood disease caused by genetic defects result in reduced rate of synthesis of one of the globin chains that make up hemoglobin. The combination of transfusion and chelating therapy has dramatically extended the life expectancy of thalassemic patients.
OBJECTIVE:
The objective of this study is to determine the factors associated with short stature in thalassemic patients and main endocrine complications.
PATIENTS AND METHODS:
A case-control study was performed prospectively in Ibn-Al Balady hospital in (Al- Sader city-Baghdad- Rasafa) for the period extended from the 1st of January to 31st of May (2013). Data were collected from 181 thalassemic patients, their ages were 10 to 20 years old being attended the hospital for regular follow up and blood transfusion. The control in this research work was one of the patients' relatives with the same age after thalassemia was ruled out in the control. Data collected in this study included: age, sex, height and weight were assessed by the National Center of Health statistics (NCHS) growth curves , history of splenectomy, times of blood transfusion , hepatitis infection, type of chelating agent. Serum ferritin, hormones level, fasting and random blood sugar and serum calcium were estimated.
RESULT:
It was founded that 79% of the β-thalassemic patients had short stature (their height equal or less than5rd percentile) with significant relation with high serum ferritin (P-value=0.006). Delayed puberty was the commonest endocrine complication in thalassemic patients (83.9%).
CONCLUSION:
High serum ferritin is associated with growth retardation and many endocrine complications . Aggressive iron-chelating therapy and regular measurement of hormones concentration are necessary for thalassemic patients mainly during puberty to avoid growth retardation.

Glucose Tolerance Test in Beta-Thalassemia Major in Al-Sader City

Qais Abdullah Ali Al-Salahe; Mohammed Shaker Al-Awady; Abas Abdalsaheb Al-Gharbawy

Iraqi Postgraduate Medical Journal, 2014, Volume 13, Issue 4, Pages 550-559

ABSTRACT:
BACKGROUND:
β-thalassemia are inherited defects in the synthesis of B-globin portion of hemoglobin. The combination of transfusion and chelating therapy has dramatically extended the life expectancy of thalassemic patients.
OBJECTIVE:
This study is to determine the prevalence of diabetes mellitus and association of some factors with impaired glucose tolerance test in transfusion dependent β- thalassemia major.
PATIENTS AND METHODS:
A case-control study done on patients attending blood disease center in Ibn-Al-Balady hospital from 1st of July to 31st of October 2011. Data collected from 287 thalassemic patients (being attended the hospital for regular follow up and blood transfusion) and their relatives which included age, sex, height, weight, history of splenectomy, family history of DM, hepatitis B or C infection, serum ferritin level and an oral glucose tolerance test.
RESULTS:
It was found that 9.7% of β- thalassemic patients had impaired glucose tolerance test and 1.1% were diagnosed with diabetes, 32.7% were splenectomized, 38.3% had viral hepatitis infection, mean serum ferritin in those older than 20 years was 5923±1033µg/l and 16.8% had family history of diabetes.
CONCLUSION:
Increased serum ferritin concentration, splenectomy and viral hepatitis infection are associated with abnormal glucose tolerance in patients with blood transfused b-thalassemia. Aggressive iron-chelating therapy and prevention and treatment of viral hepatitis infection were warranted.