Ryan White Center for Pediatric Infectious Disease and Global Health, Department of Pediatrics, Indiana University School of Medicine, 1044 W. Walnut St, R4 402D, Indianapolis, IN, 46202, USA. chjohn@iu.edu.
From Centre Hospitalier Monkole, Kinshasa, Democratic Republic of Congo (L.T.); the Department of Medicine, University Health Network and Mt. Sinai Hospital, and the University of Toronto, Toronto (G.T.); the Kenya Medical Research Institute (KEMRI)-Wellcome Trust Research Program, Kilifi, Kenya (T.N.W.); the Department of Medicine, Imperial College London, London (T.N.W.); Hospital Pediátrico David Bernardino, Luanda, Angola (B.S.); Mbale Clinical Research Institute and Mbale Regional Referral and Teaching Hospital-Busitema University, Mbale, Uganda (P.O.-O.); the Division of Hematology, Department of Pediatrics, Cincinnati Children's Hospital (A.L., S.E.S., T.S.L., P.T.M., R.E.W.), University of Cincinnati College of Medicine (A.L., P.T.M., R.E.W.), and the Global Health Center, Cincinnati Children's Hospital Medical Center (S.E.S., P.T.M., R.E.W.), Cincinnati; and Cohen Children's Medical Center, New Hyde Park, and the Zucker School of Medicine at Hofstra/Northwell, Hempstead - both in New York (B.A.).
Ryan White Center for Pediatric Infectious Disease and Global Health, Department of Pediatrics, Indiana University School of Medicine, 1044 W. Walnut St, R4 402D, Indianapolis, IN, 46202, USA.
Ryan White Center for Pediatric Infectious Disease and Global Health, Department of Pediatrics, Indiana University School of Medicine, 1044 W. Walnut St, R4 402D, Indianapolis, IN, 46202, USA.
From Centre Hospitalier Monkole, Kinshasa, Democratic Republic of Congo (L.T.); the Department of Medicine, University Health Network and Mt. Sinai Hospital, and the University of Toronto, Toronto (G.T.); the Kenya Medical Research Institute (KEMRI)-Wellcome Trust Research Program, Kilifi, Kenya (T.N.W.); the Department of Medicine, Imperial College London, London (T.N.W.); Hospital Pediátrico David Bernardino, Luanda, Angola (B.S.); Mbale Clinical Research Institute and Mbale Regional Referral and Teaching Hospital-Busitema University, Mbale, Uganda (P.O.-O.); the Division of Hematology, Department of Pediatrics, Cincinnati Children's Hospital (A.L., S.E.S., T.S.L., P.T.M., R.E.W.), University of Cincinnati College of Medicine (A.L., P.T.M., R.E.W.), and the Global Health Center, Cincinnati Children's Hospital Medical Center (S.E.S., P.T.M., R.E.W.), Cincinnati; and Cohen Children's Medical Center, New Hyde Park, and the Zucker School of Medicine at Hofstra/Northwell, Hempstead - both in New York (B.A.).
Proteinuria is a manifestation of sickle cell anemia (SCA)-related renal disease and is a risk factor of renal impairment. Angiotensin-converting enzyme (ACE) inhibitors have benefits, but their role ...
Screening for obstructive sleep apnea (OSA) is recommended by current guidelines in children with sickle cell anemia (SCA), but no specific approach is described. The Pediatric Sleep Questionnaire (PS...
A prospective study, in children 4 to 18 years old with SCA. Subjects were assessed for snoring and PSQ administered at the same visit. All children with snoring were then referred for polysomnography...
A total of 106 subjects were screened. Habitual snoring prevalence was 51/106 (48.1%). In the snoring group, OSA was detected in 83.9% (apnea-hypopnea index [AHI] ≥1.0/h) and 22.6% (AHI ≥5.0/h), respe...
PSQ is a poor screening tool for detection of OSA in those children with SCA who snore. Physician assessment for snoring could however be an initial approach before polysomnography....
It is well established that splenic dysfunction occurs in early childhood in sickle cell anemia (SCA), although the determinants and consequences of splenic injury are not fully understood. In this st...
Children with sickle cell anemia (SCA) are at increased risk of acute kidney injury (AKI) that may lead to death or chronic kidney disease. This study evaluated AKI prevalence and risk factors in chil...
We prospectively enrolled 185 children from 2 - 18 years of age with SCA (Hemoglobin SS) hospitalized with a VOC at a tertiary hospital in Uganda. Kidney function was assessed on admission, 24-48 h of...
Using KDIGO, 90/185 (48.7%) children had AKI with 61/185 (33.0%) AKI cases present on admission, and 29/124 (23.4%) cases of incident AKI. Overall, 23 children with AKI had a 1.5-fold increase in crea...
These results demonstrate that AKI is a common complication in children with SCA admitted with VOC. The sKDIGO definition of AKI in children with SCA was a better predictor of clinical outcomes in chi...
Fetal hemoglobin (HbF) is a modifier of the clinical and hematologic phenotype of sickle cell anemia (SCA). Three quantitative trait loci (QTL) modulate HbF expression. The neurocognitive effects of v...
The prospective longitudinal cohort study, Sickle Cell Clinical Research and Intervention Program, was used as a discovery cohort (n = 166). The genotypes for 11 SNPs were extracted through whole geno...
HbF was positively associated with IQ (minimum raw p = 0·0018) at pFDR<0·05. HbF mediated the relationship between two BCL11A SNPs, rs1427407 and rs7606173, HBS1L-MYB: rs9494142, and PGS...
As the major modulator of the severity of SCA, HbF also influences neurocognition, which is done through mediation of its QTL. These findings have implications for early identification of neurocogniti...
National guidelines recommend that children with sickle cell anemia receive annual transcranial Doppler (TCD) screening to assess stroke risk. Our objectives were to estimate the rate of TCD screening...
Cross-sectional....
Using the 2009-2017 IBM MarketScan Commercial Database, we identified children aged 2 to 15 years who met a validated claims-based definition of sickle cell anemia. We calculated the proportion of chi...
The 2519 children in the analysis accounted for 7197 person-years of enrollment; 14% of person-years were from HDHP enrollees. During 2009-2017, the proportion of children receiving TCD screening rang...
Among privately insured children with sickle cell anemia, fewer than half received annual TCD screening. Out-of-pocket spending exceeded $100 for 27% of TCD screens. Although HDHP enrollment was not a...
Children with sickle cell disease (SCD) who began hydroyxurea before age five years scored no differently on a measure of cognitive funciton than age, sex, and race-matched unaffected peers....
Sickle cell anemia (SCA) is a monogenic hemoglobinopathy associated with severe acute and chronic complications, with the highest incidence worldwide in Sub-Saharan Africa. The wide variability in cli...
A cross-sectional study was conducted in a hospital dedicated to SCA management in Kinshasa. Clinical history of patients was recorded, a complete physical examination performed. The diagnosis was con...
The study group consisted of 166 genetically confirmed SCA patients. The SCA severity was mild in 28.9%, moderate in 64.5% and severe in 6.6%. The disease severity score increased with patient's age (...
In this selected, hospital-based populations of adults with SCA, severe disease was rare, which may be due to survival bias. However, two thirds had moderate severity of the disease, mostly with a low...
To analyze the temporal trend of mortality from sickle cell anemia in Brazil, by region, in the period 1997-2017....
epidemiological study, with an ecological design, with a temporal trend, carried out with data from the Mortality Information System. For descriptive analysis, absolute and relative frequencies were u...
6,813 deaths from sickle cell anemia were registered. Brown individuals (50.87%) were more frequent, with a predominance of males (50.4%), aged between 25 and 34 years and a higher incidence of deaths...
sickle cell anemia showed increasing mortality in the 21 years analyzed and alerts health professionals and managers....
