RESUMEN
65 cases of thalassaemia major were selected out of 300 cases of anaemia [21.66%] admitted to Paediatric Unit A in Women and Children Hospital, Abbottabad since June 1995, based on their family history, clinical data, laboratory investigations and X-ray analyses. Most of them were between 1-5 years of age. No difference was noted between the two sexes. Parental consanguinity was present in 49.23% of cases and non-consanguinity in 24.61%, whereas data was not available in 26.15%, of cases. All the patients had moderate to severe anaemia and failure to thrive as their presenting symptoms. Other predominant associated features were splenomegaly [90%], typical facies [60%], skull changes on X-rays [60%], hepatomegaly [45%], jaundice [10%] and repeated infections such as gastroenteritis, pneumonia, etc. Some of them, particularly older children having received multiple blood transfusions, presented with complications such as viral hepatitis, congestive cardiac failure [4.61%] and diabetic ketoacidosis [1.53%]. A variety of problems were encountered in diagnosis and management of these cases and are listed with suggested improvements in patient care