Assessment Of Quality Of Care In Children With Thalassemia Major

摘要

conditions.Inthe12GyTBIgroup,thediseasefreesurvivalafter2yearspostHSCT was 56.7 %. In the 2 Gy single fraction TBI group, 50% children havesurvived and graft rejection was seen in 30%. Out of these, comprehensivelate toxicity assessment was done in 25 survivors. The male: female ratiowas1.6:1.Themedianageattransplantationwas7years(range3yearsto19years). The median follow up was 3 years post HSCT (range 1 year to 9years). Sixteen children underwent transplantation for relapsed / high riskmalignancies and 8 children for benign conditions like Fanconi anemia (4),primary immune deficiency (4) and severe aplastic anemia(1). Eight chil-dren received single dose 2 Gy TBI and 16 children received 12Gy TBI in 8fractions.Growthfailureintheformofstuntingwasobservedin37%(9/24),outof which 3childrenhad Fanconianemia. Gonadal failurewas seen in 4%(1/24), who had low sex hormone level and was treated with hormonereplacement. Hypothyroidism was seen in 25% of children (6/24). Cardiacfunction and ophthalmological evaluation was within normal limits in allchildren. Spirometrycould be performed in 20 children and 35% (7/20) hadrestrictive pulmonary function (severe restriction in 1, moderate in 2 andmild in 4 patients). Renal and liver function was normal in all childrenexcept one who had transaminitis. Audiometry revealed sensorineuralhearing loss in one child (who was later diagnosed to have vestibularschwanomma)andbilateralconductivelossin1child.Secondaryneoplasmwas seen in 1 patient who had vestibular schwanomma. Neuropsycholog-ical assessment of the 17 patients evaluated revealed problems of anxietyand depression in 30% of children (5/17).