Paediatric-History taking & Physical examination of a patient with Thalassemia

Paediatric-History taking & Physical examination of a patient with ThalassemiaCreated OnApril 28, 2020Last Updated OnApril 28, 2020byadmin You are here: Main Clinical Examination Paediatric-History taking & Physical examination of a patient with Thalassemia < All Topics Table of Contents History taking: Ask about identification of patient- age, sex, race, address, informant β-Thalassaemia major typically presents with anaemia at 4 – 6 months of age. Ask about age of first presentation and symptoms If β-Thalassaemia minor type, usually unremarkable symptoms If β-Thalassaemia major type, there will usually be severe hemolytic anemia, requiring blood transfusion In severe type α-Thalassemia major (Hb-Bart), it causes hydrops fetalis In HbH disease, initial presentation will be moderate haemolytic anemia and jaundice Ask how was he/she diagnosed Results of investigations should be enquired Ask about the age of first transfusion and indications Ask about transfusion history Which hospital, Frequency, reactions, complications, venous assess, usual pre-transfusion Hb and post-transfusion Hb. Routine screening and follow-up Yearly hearing, vision, infective screening status, OGTT Ask about chelation history Age of initiation, after how many transfusion, route of administration, type of chelating agent, dosage, compliance, side effects of medications, serum ferritin level. Ask about complications of thalassemia and treatment Complications of iron overload due to hemolysis and transfusion Secondary hemochromatosis Complications of chelators – impaired hearing and vision, risk of infection Anemic symptoms – easy fatigue, palpitations, shortness of breath, fainting episodes Abdominal distension and discomfort secondary to splenomegaly Growth retardation (reasons are being discussed below) Skeletal deformities due to ineffective erythropoiesis and extramedullary hematopoiesis. Ask about past medical/surgical history Splenectomy and gall bladder stone If splenectomy was done, enquire on vaccinations and antibiotic prophylaxis. Complications of splenectomy? Infection? Ask about birth history Newborn screening test Hepatosplenomegaly, jaundice and anemia at birth (common in HbH disease) Kernicterus If Hb-Bart which is the most severe variant of α-thalassemia, there will be intrauterine hydrops fetalis, severe hepatosplenomegaly and often cardiac and skeletal abnormalities (usually death in utero or shortly after birth) Ask about immunization history Routine immunization as per Malaysian Immunization Schedule Any extra vaccinations given Ask about dietary history Avoidance of iron rich products Ask about family history Get a complete family history by plotting a family tree Any thalassaemia running in the family, any other disease, consanguinity Screening for parents and siblings Negative family history does not rule...

Continue reading

Please Login/Register to read full article.