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Browsing by Author "Onishi, Toshihiro"
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Item Magnetic resonance imaging enhancement of spinal nerve roots in a boy with X-linked adrenoleukodystrophy before diagnosis of chronic inflammatory demyelinating polyneuropathy(Elsevier, 2023-11-18) Miller, Derryl; Walsh, Laurence; Smith, Lisa; Supakul, Nucharin; Ho, Chang; Onishi, Toshihiro; Neurology, School of MedicineWe present a boy with X-linked adrenoleukodystrophy (X-ALD) who was found to have lumbar nerve root enhancement on a screening MRI of the spine. The MRI was performed for lower extremity predominant symptoms. Several weeks after this MRI, he developed leg pain and was averse to walking long distances. He was diagnosed with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) with electromyography, nerve conduction studies, and serial imaging. His case is consistent with CIDP in association with X-ALD based on improvement with intravenous immunoglobulin (IVIG) with continued contrast enhancement and lower extremity symptoms 8 weeks after his initial scans. Contrast enhancement of nerve roots has not been previously described in X-ALD. Nerve root enhancement has been seen in other leukodystrophies such as globoid cell leukodystrophy and metachromatic leukodystrophy. This case also demonstrates comorbid X-ALD with CIDP and highlights possible mechanisms from the literature for this association. We also review the broad differential of cauda equina nerve root enhancement.Item Outcomes of Pediatric Patients with Therapy-Related Myeloid Neoplasms(Springer Nature, 2021) Sharma, Akshay; Huang, Sujuan; Li, Ying; Brooke, Russell J.; Ahmed, Ibrahim; Allewelt, Heather B.; Amrolia, Persis; Bertaina, Alice; Bhatt, Neel S.; Bierings, Marc B.; Bies, Joshua; Brisset, Claire; Brondon, Jennifer E.; Dahlberg, Ann; Dalle, Jean-Hugues; Eissa, Hesham; Fahd, Mony; Gassas, Adam; Gloude, Nicholas J.; Goebel, W. Scott; Goeckerman, Erika S.; Harris, Katherine; Ho, Richard; Hudspeth, Michelle P.; Huo, Jeffrey S.; Jacobsohn, David; Kasow, Kimberly A.; Katsanis, Emmanuel; Kaviany, Saara; Keating, Amy K.; Kernan, Nancy A.; Ktena, Yiouli P.; Lauhan, Colette R.; López-Hernandez, Gerardo; Martin, Paul L.; Myers, Kasiani C.; Naik, Swati; Olaya-Vargas, Alberto; Onishi, Toshihiro; Radhi, Mohamed; Ramachandran, Shanti; Ramos, Kristie; Rangarajan, Hemalatha G.; Roehrs, Philip A.; Sampson, Megan E.; Shaw, Peter J.; Skiles, Jodi L.; Somers, Katherine; Symons, Heather J.; de Tersant, Marie; Uber, Allison N.; Versluys, Birgitta; Cheng, Cheng; Triplett, Brandon M.; Pediatrics, School of MedicineLong-term outcomes after allogeneic hematopoietic cell transplantation (HCT) for therapy-related myeloid neoplasms (tMNs) are dismal. There are few multicenter studies defining prognostic factors in pediatric patients with tMNs. We have accumulated the largest cohort of pediatric patients who have undergone HCT for a tMN to perform a multivariate analysis defining factors predictive of long-term survival. Sixty-eight percent of the 401 patients underwent HCT using a myeloablative conditioning (MAC) regimen, but there were no statistically significant differences in the overall survival (OS), event-free survival (EFS), or cumulative incidence of relapse and non-relapse mortality based on the conditioning intensity. Among the recipients of MAC regimens, 38.4% of deaths were from treatment-related causes, especially acute graft versus host disease (GVHD) and end-organ failure, as compared to only 20.9% of deaths in the reduced-intensity conditioning (RIC) cohort. Exposure to total body irradiation (TBI) during conditioning and experiencing grade III/IV acute GVHD was associated with worse OS. In addition, a diagnosis of therapy-related myelodysplastic syndrome and having a structurally complex karyotype at tMN diagnosis were associated with worse EFS. Reduced-toxicity (but not reduced-intensity) regimens might help to decrease relapse while limiting mortality associated with TBI-based HCT conditioning in pediatric patients with tMNs.