***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
    ***DOCUMENTATION*** 
    ***EXAMPLES*** 
    Input: In the phase I/II pediatric hydroxyurea  safety trial ( HUG-KIDS ) , school-aged children with sickle cell anemia receiving hydroxyurea at the maximally tolerated dose ( MTD ) had variable increases in the percentage of fetal hemoglobin ( % HbF ) . To identify predictors of the HbF response to hydroxyurea therapy , baseline clinical and laboratory values ( age , sex , hemoglobin concentration , % HbF , reticulocytes , white blood cell [ WBC ] , platelets , and serum chemistries ) , as well as treatment variables ( number of toxicities , noncompliance , MTD dose , and MTD blood counts ) were analyzed in 53 HUG-KIDS children who achieved MTD . Baseline % HbF values ( P = .001 ) , baseline hemoglobin concentration ( P = .01 ) , MTD dose ( P = .02 ) , and compliance ( P = .02 ) were significantly associated with a higher % HbF at MTD ; in contrast , age , sex , number of toxicities , and other baseline hematologic parameters were not . After adjusting for variations in baseline % HbF , the baseline reticulocyte count ( P = .05 ) and baseline WBC count ( P = .05 ) were also significantly associated with a higher % HbF at MTD . Hydroxyurea-induced increases in the hemoglobin concentration and mean corpuscular volume ( both higher absolute values at MTD and larger positive changes from baseline values ) , as well as hydroxyurea-induced decreases in reticulocytes and WBC count , were significantly associated with a higher % HbF at MTD . These data suggest that selected baseline laboratory parameters , a higher MTD dose with attention to compliance , and greater therapy-related changes in blood counts may predict the HbF response to hydroxyurea therapy for children with sickle cell anemia . The HbF response to hydroxyurea is variable and complex , however , and even children with low baseline % HbF values can develop substantial increases in % HbF at MTD OBJECTIVE To prospect ively assess compliance with antibiotic prophylaxis among children with sickle cell anemia . METHODS A total of 108 children ( aged 3 months to 4(1/2 ) years , 45 % male ) were recruited from the Hematology Center in Belo Horizonte , Brazil , and followed up for 15 months . Data on compliance were obtained from three interviews with the primary caregivers , from the children 's medical records and from assay of antibacterial activity in urine sample s of 81 children . Antibiotics were available free of charge . RESULTS Penicillin was used in 106 cases ( majority by oral route ) ; erythromycin in 2 cases . Urine sample s tested positive for the antibiotic in 56 % of the cases ; 48 % of the caregivers assured during interviews that all doses had been administered to children ; 89 % of medical files recorded no compliance failures . Considering a child compliant if none or just one of these methods detected missing doses , the rate of compliance was 67 % . The agreement between methods for assigning a child as compliant or not was low . No significant association of compliance rate with gender , nutritional status , per capita income of the family , caregivers ' schooling , or number of family members was demonstrated . CONCLUSIONS Compliance rate with  prophylactic antibiotic therapy  was low when assessed by interviews and urine tests and overestimated by analysis of medical records . Compliance should preferably be assessed by several methods due to the complex character of its measurement . The results of the present study suggest the need for a comprehensive educational program involving healthcare professionals , families and children with sickle cell anemia OBJECTIVE To assess factors related to compliance with penicillin prophylaxis among patients with sickle cell disease . DESIGN Prospect i ve case series . SETTING Urban pediatric medical center where universal penicillin prophylaxis is recommended for all patients with any sickle cell hemoglobinopathy independent of age . PARTICIPANTS Eligible patients with sickle cell hemoglobinopathies were enrolled in either the emergency department or the sickle cell clinic . MAIN OUTCOME MEASURES Compliance was assessed by  structured interview  and by urine assay with an established method ( Micrococcus luteus with disk diffusion ) that detects excreted penicillin up to 15 hours after each dose administration . RESULTS Of the 159 patients actively followed up at the sickle cell center , 123 ( 77.3 % ) eligible patients were enrolled . Reported compliance by structured interview  ( > or = 1 dose of penicillin V potassium within 15 hours of enrollment ) was 83 of 123 patients ( 67.5 % ; 95 % confidence interval , 59.2%-75.8 % ) , whereas measured compliance as determined by urine assay was 53 of 123 patients ( 43.1 % ; 95 % confidence interval , 31.3%-51.7 % ) . Measured compliance was significantly greater in patients younger than 5 years than in those older than 5 years ( 25/41 [ 61 % ] vs 28/82 [ 34 % ] , respectively ; P = .004 ) , and was significantly greater in patients with private insurance than in those with public insurance ( 17/28 [ 61 % ] vs 33/90 [ 37 % ] , respectively ; P = .02 ) . Measured compliance was not significantly associated with sex , site of recruitment , hemoglobinopathy , or chief complaint in the emergency department . CONCLUSIONS Measured compliance was poor , and patients and /or their families frequently misrepresented their compliance when interviewed . These data suggest that efforts are necessary to improve overall compliance , and they identify groups at greatest risk for noncompliance Forty children homozygotes for sickle-cell disease ( SCD ) aged 2 - 5 years were enrolled in a study to assess the efficacy of pneumococcal vaccine combined with penicillin prophylaxis in preventing pneumococcal infection . The vaccine was given initially and then every 2 years as a booster . In addition to pneumococcal vaccine , 24 children were prescribed penicillin-V orally twice daily while the remaining 16 were given benzathine penicillin intramuscularly every 4 weeks in the clinic . The study period lasted for 4 years during which the clinic attendance rate of both groups was 90 % and 92 % respectively . The compliance with oral penicillin intake was checked by r and om urine testing for penicillin . The test was positive in 128 out of 320 urine sample s ( 40 % ) . The parenteral penicillin was given at every clinic visit and the rate of compliance was 92 % . Twelve episodes of bacterial sepsis were documented : 10 of them were caused by Gram-negative organisms . The pneumococcus caused a fatal episode of septicaemia and meningitis in a child who did not comply with penicillin prophylaxis . In a retrospective search , 15 episodes of bacterial sepsis have been documented in 22 children receiving no prophylaxis for 2 years . Eight of those episodes were caused by the pneumococcus . The combination of pneumococcal vaccine with penicillin prophylaxis was effective in preventing pneumococcal sepsis in children with SCD followed up for 153 patients years AIMS To evaluate compliance with  prophylactic penicillin therapy in sickle cell disease ( SCD ) in children . METHODS Forty-five children aged 37 + /- 19 ( 9 - 84 ) months [ mean + /- s.d ; range ] with homozygous SCD were recruited . After a baseline period of 2 months the patients were r and omized to either the intervention or non-intervention group . The intervention consisted of a slide show explaining the pathogenesis of sickle cell disease and its complications ; weekly phone calls by the clinic social worker ; and a calendar . Compliance was again evaluated after the 2 month intervention period and after a further 2 month monitoring period without intervention .  Compliance was monitored using the Medication Event Monitoring System .  At the end of the 6 months , parents in both groups completed a question naire the aims of which were to determine knowledge and underst and ing of sickle cell disease and previous experience with infection . Patient admissions to the hospital during the study were recorded . RESULTS Compliance during the 2 month baseline assessment was 66.0 + /- 32.5 (1.3 - 98.2)% and 69.3 + 25.4 (19.8 - 96.5)% in the intervention ( n = 13 ) and non-intervention ( n = 10 ) groups respectively ( P = 0.79 ) . During the next 2 months , compliance in the intervention group ( n = 11 ) was 79.0 + /- 31.4 (11.0 - 100.0)% and in the non-intervention group ( n = 9 ) was 66.0 + /- 20.2 (42.2 - 96.8)% ( P = 0.297 ) . In the final 2 month monitoring period compliance was 82.0 + /- 34.7 (3.8 - 100.0)% and 65.8 + /- 25.3 (25.0 - 98.2)% in the intervention ( n = 7 ) and the non-intervention ( n = 6 ) groups respectively ( P = 0.366 ) . No statistically significant differences were found when comparing compliance between the groups . CONCLUSIONS Compliance with  prophylactic antibiotic therapy in children with sickle cell disease is highly variable and its evaluation is problematic Abstract Deferoxamine ( DFO ) is an iron chelator used to treat iron overload in patients receiving chronic blood transfusions , and is usually administered as overnight subcutaneous infusions . ISOSFER was a prospect i ve , observational , cross-sectional study conducted in metropolitan France that evaluated patient characteristics , quality of life ( QoL ) , compliance and patient satisfaction with DFO monotherapy . Of 70 patients with either thalassemia , sickle cell disease or myelodysplastic syndromes , 30 % were ' satisfied ' or ' very satisfied ' with DFO . Patients ' SF-36 scores were lower than those of the general French population , and lower among patients with comorbidities and those dissatisfied with treatment . Although 72 % of patients had good compliance to DFO , 57 % reported missing at least one infusion in the previous month , and 82 % of patients expressed a preference for oral therapy . These results suggest that QoL is severely compromised in patients receiving DFO , and that compliance is not optimal BACKGROUND Subject retention and adherence are essential to maintain the power and validity of the Pediatric Hydroxyurea Phase III Clinical Trial ( BABY HUG ) . We design ed a study to assess adherence with study medication administration and study visits and to evaluate  socioeconomic factors ( SES ) that may influence these measurements of adherence . These data are important for assessing impact of adherence on BABY HUG trial outcome and defining impact of SES on adherence . METHODS Each subject 's  median study medication ( MedAd ) and  mean visit adherence ( VAd )  were evaluated . We examined associations of adherence with SES of participating families . RESULTS MedAd data were available on 153 of the 191 subjects who started r and omized study medication . MedAd was 101.7 % of volume prescribed , with 88.9 % of subjects taking at least 80 % of doses . VAd data were available on 185 of the 191 subjects who started r and omized study medication .  VAd was 97.3 % , with 82.2 % of subjects having no missed visits . During dose titration , subjects had on average 12.9 % higher medication adherence than subjects who were on a stable dose and had less frequent study visits . MedAd and VAd were not significantly associated with SES . CONCLUSION Subjects in the BABY HUG trial have had excellent adherence . SES was not associated with adherence , suggesting that SES should not be used as a criterion for enrolment in clinical trials . Additional efforts are needed to maintain medication adherence , particularly when the interval between scheduled visits increases . ( Clinical Trials.gov number , NCT00006400 ) OBJECTIVE --To assess compliance with oral penicillin prophylaxis in children with sickle cell disease and identify possible reasons for poor compliance . DESIGN --Closed question naires given to parents of children with sickle cell disease and general practitioners in Brent . Urine sample s from 23 children were tested for penicillin . SETTING --Paediatric haematology clinic , Central Middlesex Hospital , and general practice s in Brent . SUBJECTS--50 children ( aged less than or equal to 16 ) attending clinic with sickle cell disease over six months ( 33 HbSS , 12 HbSC , five HbS beta thalassaemia ) . 30 general practitioners : 15 with the greatest number of patients with sickle cell disease on the Brent register ; 15 selected r and omly from family practitioner committee 's list . MAIN OUTCOME MEASURES --Reported compliance with and awareness of importance of penicillin prophylaxis . Results of urine tests for penicillin .  RESULTS --31 parents cl aim ed that their children received  penicillin  every day and 19 that they received it most days ( greater than or equal to 5 days a week ) .  Penicillin  was detected in only 10 of 23 urine sample s tested 
    Output:
    
Medication factors contributed to adherence .
For example , prophylactic antibiotic adherence was better with intramuscular than oral administration .
Barriers included fear of side effects , incorrect dosing , and forgetting .
Nonadherence was associated with more vaso-occlusive crises and hospitalizations .
The limited data available on moderate adherence to iron chelation and hydroxyurea indicates some clinical benefit .
: Moderate adherence is typical among pediatric patients with SCD .