Assessment of proportional hazard rate of endocrine disease in major beta-thalassemia in Shiraz 2005-2006

Background: Thalassemia is the most common hemolytic anemia in Iran and around the world
that needs regular blood transfusion. Transfusion leads to iron overload in the body. Absence or
irregular chelation therapy in these patients leads to various complications such as cardiac
diseases, endocrine disorders and infectious diseases like HIV and hepatitis. The purpose of this
study was to evaluate the hazard rate of endocrine disease and related factors using COX model.
Materials and Methods: This cross-sectional study was carried out on eight-hundred and six
thalassemic (406 male and 400 female) patients referring to Cooley’s ward of Dastgheib hospital in
Shiraz who were studied using a structured questionnaire through enumeration method. Age of
occurrence, demographic information, age of onset and type of transfusion, and age of onset and
method of chelation therapy were specified. Specialist physicians diagnosed the complications.
Data were analyzed using descriptive and analytical (Proportional hazard rate and its confidence
interval using COX model) statistical.
Results: The range of the subject’s age was between 1- 43 years and mean age ± SD was
15.34±6.82 years. The prevalence rate of hypogonadism, hypothyroidism, hyperparathyroidism and
diabetes mellitus was 14.5%, 2.4%, 6.9% and 7.5% respectively and the mean age of onset of
hypogonadism, hypothyroidism, hypoparathyroidism and diabetes mellitus was 18.42±3.6,
15.83±3.5, 16.98 ± 4.5 and 17.19 ± 4.8 respectively. There was a significant association between
proportional hazard rate of all complications and age of chelation therapy onset (P<0/05).
Proportional hazard rate of hypogonadism also was related to the sex and educational level of the
patient’s mothers (P<0/05). Proportional hazard rate of hyperparathyroidism also was related to
sex and occupational status of the patient’s mothers.
Conclusions: Early onset of blood transfusion with late onset of chelating increases the
occurrence probability and early onset of complications. To prevent these complications, it is
recommended that onset of blood transfusion be according to the onset of chelating. Educational
level of parents and educating parents and patients is very important. Progression of complications
can be prevented by regular survey and early diagnosis of complications and can lead to high
efficacy of patients.