Demographic data of patients with β-thalassemia major recorded in the electronic system in the north of Iran, 2016

Abstract

Background: Major thalassemia prevention project in Iran began in 1997. Mazandaran Province in northern Iran took effective steps in preventing the birth of patients with thalassemia major in the country. We report on the demographic status of patients with thalassemia major registered in the electronic system of Mazandaran Province in Iran.

Methods: The web-based application of the Mazandaran Thalassemia Registry (THRegistry) was designed based on the Net Framework platform in VB.Net and the 2014Sql Server database. The information source included clinical records in 14 thalassemic wards of the Mazandaran province hospitals. Epidemiological data of patients and date of transfusion, blood group, educational status, employment, marital status and having children were recorded.

Results: The study population was 1,725 patients including 889 (51.5%) women and 836 (48.5%) men with a mean age of 30±9.6 years. A total of 188 patients born have been identified after the launch of the country's disease prevention plan in 1997. Prenatal diagnosis (PND) services were not used in 99 cases, PND result was mistaken in 6 other cases and in the remaining 5 cases PND was performed. A total of 1,314 patients (76.2%) were blood transfusion dependent thalassemia (TDT). Mean haemoglobin level in male and female patients was 9.4±6.3 and 9.01±5.1/100 g/dl, respectively. The mean age of onset of transfusion was 4±6.3 years. A total of 382 cases (22.1%) of married patients had a child of their own.

Conclusions: The system is a good resource to design descriptive and cohort studies, survival studies, therapeutic planning, and observation of the success of the major thalassemia prevention project.