Publication:

Study of thalassemia in pediatrics population

Background Thalassemia is an inherited disorder of AR gene caused by impaired synthesis of one or more globin chains. Study aimed to study of thalassemia in pediatrics population.

Methods This observational prospective Study done in GMC Shahdol from January 2020 to January 2023 in 60 patients. Children with thalassemia, ≥6 months age and children on regular blood transfusion with or without iron chelation therapy are included and Children with other causes of anemia like nutritional anemia, aplastic anemia, SCA were excluded.

Results The mean age was 10.2 years, 75% were males. MC clinical features were (97%) icterus followed by (90%) pallor, 80% had moderate to severe hepatomegaly. Mean Hb was 8.5±0.9 gm/dl, 67% had severe anemia, mean MCV level was 73±2 µm3, Mean MCH and MCHC level were 20±2 pg/cell and 28±2 ghb/dl. Mean ferritin level was 1281.8±219.9 ng/dl, 39% had more than 2500 ng/dl, mean frequency of blood transfusion 16±2 times a year, mean interval between transfusion 22 ±2 days, 10% had hyperglycemia. The 6.5% had impaired GTT, while 3.3% patient’s diabetic range. 18% had hypocalcemia, 1.6% had subclinical hypothyroidism and only 4% had overt hypothyroidism. Tablet deferasirox chelating agent in all thalassemia patients. The common adverse reaction was diarrhea (26.1%), abdominal pain (23%), skin rash. Hepatitis C virus infection in 76% and hepatitis B in 48%. Two patients (4%) had decreased LVEF of 35 to 40%.

Conclusions This study done to evaluate the thalassemic pediatrics patients in whole aspects, which is very important to know about this.