{"doi":"10.1177/08830738221081141","title":"Time to Transplant in X-Linked Adrenoleukodystrophy","abstract":"<jats:sec>\n                    <jats:title>Objectives</jats:title>\n                    <jats:p>Cerebral X-linked adrenoleukodystrophy (cALD) is an inflammatory demyelination of the brain that can lead to death unless treated by hematopoietic stem cell transplantation. Survival and improved outcomes for cerebral adrenoleukodystrophy are associated with hematopoietic stem cell transplantation at earliest evidence of disease on magnetic resonance imaging (MRI). Our goal was to determine average duration between diagnosis of cALD and hematopoietic stem cell transplantation.</jats:p>\n                  </jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods</jats:title>\n                    <jats:p>This was a retrospective review of data of patients aged 18 years or younger, using a nationwide administrative health care database (Pediatric Health Information System), with an International Classification of Diseases, Tenth Revision ( ICD-10) diagnosis of adrenoleukodystrophy. Time range was October 1, 2015, through June 30, 2021. We determined time to hematopoietic stem cell transplantation by duration between index brain MRI and a code for hematopoietic stem cell transplantation.</jats:p>\n                  </jats:sec>\n                  <jats:sec>\n                    <jats:title>Results</jats:title>\n                    <jats:p>We identified 27 patients with cerebral adrenoleukodystrophy. Total charges for the cohort was $53 million. Time to transplant averaged 97 days. For Hispanic patients, time to transplant was 117 days, compared with 80 days for White, non-Hispanic patients. Comparison of different hospitals showed significant variability in time to hematopoietic stem cell transplantation.</jats:p>\n                  </jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion</jats:title>\n                    <jats:p>We found that time to hematopoietic stem cell transplantation was &gt;3 months for patients with cerebral adrenoleukodystrophy in the hospitals we evaluated. We noted differences in average time by race/ethnicity and by hospital. Our findings suggest opportunity to reduce time to transplant in cerebral adrenoleukodystrophy.</jats:p>\n                  </jats:sec>","journal":"Journal of Child Neurology","year":2022,"id":34672,"datarank":0.46921347382066747,"base_score":2.4849066497880004,"endowment":2.4849066497880004,"self_citation_contribution":0.37273599746820013,"citation_network_contribution":0.09647747635246733,"self_endowment_contribution":0.37273599746820013,"citer_contribution":0.09647747635246733,"corpus_percentile":null,"corpus_rank":null,"citation_count":11,"citer_count":9,"citers_with_citation_signal":3,"citers_with_endowment":3,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":179211,"name":"Jacob Wilkes","orcid":null,"position":1,"is_corresponding":false},{"id":179210,"name":"Joshua L. Bonkowsky","orcid":"0000-0001-8775-147X","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"base_score":2.4849066497880004,"endowment":2.4849066497880004,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"35238239","pmcid":"PMC9086106","openalex_id":"https://openalex.org/W4214875083","authors":[],"funders":[{"funder_name":"National Institutes of Health","grant_id":"U54 NS115052","title":null},{"funder_name":"National Institutes of Health","grant_id":"3U54NS115052-03S1","title":"Utility of advanced MRI and wearable technology to identify sensory motor outcome measures in leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL)"}],"total_grants":2,"fwci":0.7796,"citation_percentile":0.68183519,"influential_citations":0,"citation_trend":[{"year":2023,"count":3},{"year":2024,"count":3},{"year":2025,"count":3},{"year":2026,"count":2}],"oa_status":"green","license":"https://journals.sagepub.com/page/policies/text-and-data-mining-license","oa_locations":[{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/9086106","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9086106","host_type":"GREEN"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/9086106","host_type":"repository"},{"url":"https://journals.sagepub.com/doi/pdf/10.1177/08830738221081141","host_type":"publisher"},{"url":"https://journals.sagepub.com/doi/full-xml/10.1177/08830738221081141","host_type":"publisher"},{"url":"https://doi.org/10.1177/08830738221081141","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/35238239","host_type":"repository"}],"fields_of_study":["Peroxisome Proliferator-Activated Receptors","Metabolism and Genetic Disorders","Pituitary Gland Disorders and Treatments","Medicine","03 medical and health sciences","0302 clinical medicine","Adrenoleukodystrophy","Child","Hematopoietic Stem Cell Transplantation","Humans","Magnetic Resonance Imaging","Retrospective Studies"],"mesh_terms":["Adrenoleukodystrophy","Child","Humans","Magnetic Resonance Imaging","Retrospective Studies","Hematopoietic Stem Cell Transplantation"],"keywords":["Adrenoleukodystrophy","Hematopoietic stem cell transplantation","Transplantation","Medicine","Retrospective cohort study","Stem cell","Hematopoietic stem cell","Internal medicine","Haematopoiesis","Pediatrics","Biology","Genetics","Brain","Children","Pediatric","epidemiology","Leukodystrophy","Humans","Child","Magnetic Resonance Imaging","Retrospective Studies"],"sdg_mappings":[{"sdg_number":3,"sdg_label":"3. 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