Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood

Hankins, Jane S.; Estepp, Jeremie H.; Hodges, Jason R.; Villavicencio, Martha; Robison, Leslie L.; Weiss, Mitchell J.; Kang, Guolian; Schreiber, Jane E.; Porter, Jerlym S.; Kaste, Sue C.; Saving, Kay L.; Bryant, Paulette; Deyo, Jeffrey E.; Nottage, Kerri; King, Allison A.; Brandow, Amanda M.; Lebensburger, Jeffrey D.; Adesina, Oyebimpe O.; Chou, Stella T.; Zemel, Babette S.; Smeltzer, Matthew P.; Wang, Winfred C.; Gurney, James G.

doi:10.1002/pbc.27228

Cited by 61 publications

(54 citation statements)

References 82 publications

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“…Kidney biopsies in pediatric SCN will add to our understanding of its pathophysiology. Longitudinal prospective cohorts such as the Sickle Cell Clinical Research and Intervention Program will provide a wealth of clinical data to answer the pertinent questions in SCN [88].…”

Section: Resultsmentioning

confidence: 99%

Sickle Cell Nephropathy in the Pediatric Population

et al. 2018

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Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population. Summary: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiotensin converting enzyme inhibitors and hydroxyurea in treating albuminuria is addressed here as well as novel treatments that may be of benefit.

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Section: Resultsmentioning

confidence: 99%

Sickle Cell Nephropathy in the Pediatric Population

et al. 2018

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“…In this analysis, participants receiving chronic transfusion therapy had a significantly lower eGFR than participants on the HUSTLE study receiving hydroxyurea using 2 different creatinine‐based definitions of eGFR . In addition to these shorter‐term randomized intervention trials to reduce hyperfiltration, longer‐term natural history studies, including Baby HUG Follow‐up Study (NCT 00890396) and the St. Jude Clinical Research and Intervention Program (NCT 02098863), can evaluate if hydroxyurea therapy or chronic transfusion therapy initiated very early in life and continued throughout childhood will prevent the development of hyperfiltration . This information is vital to determine whether sickle cell disease modifying therapies alone or the addition of renal‐targeted therapies are necessary to normalize eGFR in children with sickle cell disease.…”

Section: Discussionmentioning

confidence: 99%

Hyperfiltration during early childhood precedes albuminuria in pediatric sickle cell nephropathy

et al. 2019

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Background In patients with diabetes mellitus, hyperfiltration precedes the development of albuminuria. Pediatric sickle cell anemia (SCA) patients have a high prevalence of hyperfiltration and albuminuria during early childhood and adolescence. We tested the hypothesis that hyperfiltration precedes the development of albuminuria in a longitudinal pediatric SCA cohort. Methods We identified 91 participants with HbSS or SB0 thalassemia 5‐21 years of age enrolled in a longitudinal sickle cell nephropathy cohort study who had a cystatin C measured during early childhood (4‐10 years of age). Early hyperfiltration was defined as a mean eGFR >180 mL/min/1.73m2 using cystatin C obtained from 4 to 10 years of age. Persistent albuminuria was defined as an albumin to creatinine ratio > 30 mg/g on two of three untimed urine specimens. Time to event analysis estimated survival curves for participants with and without hyperfiltration using Kaplan‐Meier curves and used logrank test for categorical variables to assess the association with time to development of the first episode persistent albuminuria. Results Persistent albuminuria occurred more often and at an earlier age in participants with early hyperfiltration compared to those without early hyperfiltration (log‐rank, P = .004). Participants who developed albuminuria have a significant increase in their eGFR during childhood (P = .003) as compared to participants who have not yet progressed to albuminuria (P = .26). For every 1 g/dL increase in hemoglobin, the hazard ratio for developing persistent proteinuria decreased by 0.56 (95% CI: 0.3, 1.06, P = .07). Conclusion Hyperfiltration precedes the development of persistent proteinuria in pediatric SCA patients. Intervention strategies should target lowering eGFR during early childhood.

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“…The present study provides a preliminary analysis of attention measures from the neurocognitive assessments conducted as part of a large, prospective, lifespan cohort study of SCD at St. Jude (Sickle Cell Clinical Research and Intervention Program [SCCRIP]; Hankins et al , ). See Table SI for a full list of measures included in the neurocognitive assessment.…”

Section: Sample Characteristics Based On Paediatric–adult Care Transfmentioning

confidence: 99%

Attention difficulties are associated with lower engagement in adult care amongst youth with sickle cell disease

et al. 2020

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Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood

Abstract: The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD.

Cited by 61 publications

References 82 publications

Sickle Cell Nephropathy in the Pediatric Population

Sickle Cell Nephropathy in the Pediatric Population

Hyperfiltration during early childhood precedes albuminuria in pediatric sickle cell nephropathy

Attention difficulties are associated with lower engagement in adult care amongst youth with sickle cell disease

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