2024
Preservation of Pulmonary Function Following Hematopoietic Cell Transplant for Sickle Cell Disease: A STAR Study
Horan F, Bendiak G, Abraham A, Liu K, Gillespie S, Chellapandian D, Shah R, Bhatia M, Chaudhury S, Eckrich M, Jaroscak J, Kasow K, Krajewski J, Ngwube A, Horan J, Krishnamurti L, Shenoy S, Guilcher G, Stenger E. Preservation of Pulmonary Function Following Hematopoietic Cell Transplant for Sickle Cell Disease: A STAR Study. Transplantation And Cellular Therapy 2024, 30: s45-s46. DOI: 10.1016/j.jtct.2023.12.079.Peer-Reviewed Original ResearchHematopoietic cell transplantationPulmonary function testsPost-HCTYears post-HCTSickle cell diseaseAmerican Thoracic SocietyCell transplantationPulmonary dysfunctionPulmonary functionCurative hematopoietic cell transplantationPreservation of pulmonary functionCell diseaseGroup of SCD patientsPulmonary function test dataPost-HCT patientsProgressive pulmonary dysfunctionLung volume valuesSevere clinical phenotypeMyeloablative conditioningMedian followHLA matchingMedian ageNo significant differencePFT resultsPre-HCT
2023
Circulating Mitochondrial DNA Is Associated With High Levels of Fatigue in Two Independent Sarcoidosis Cohorts
Fiorini V, Hu B, Sun Y, Yu S, McGovern J, Gandhi S, Woo S, Turcotte-Foster S, Pivarnik T, Khan Z, Adams T, Herzog E, Kaminski N, Gulati M, Ryu C. Circulating Mitochondrial DNA Is Associated With High Levels of Fatigue in Two Independent Sarcoidosis Cohorts. CHEST Journal 2023, 165: 1174-1185. PMID: 37977267, PMCID: PMC11110677, DOI: 10.1016/j.chest.2023.11.020.Peer-Reviewed Original ResearchPatient-related outcome measuresToll-like receptor 9Fatigue Assessment ScalePlasma mtDNA concentrationsTLR9 activationSarcoidosis patientsMtDNA concentrationsMulti-organ sarcoidosisCommon chief complaintInnate immune activationNovel therapeutic strategiesDomains of fatigueSevere clinical phenotypePsychobiologic mechanismsSarcoidosis cohortScadding stageCorticosteroid useCytokine levelsExtrapulmonary diseaseProspective cohortFAS scoresPulmonary fibrosisChief complaintImmune activationPatient populationRegimen Intensity and Age Affect Transplant-Related Outcomes after Matched Related Donor Hematopoietic Cell Transplantation for Sickle Cell Disease: A STAR Registry Study
John T, Chellapandian D, Shah R, Gillespie S, Liu K, Xiang Y, Bhatia M, Chaudhury S, Eckrich M, Guilcher G, Jaroscak J, Kasow K, Krajewski J, Ngwube A, Olson T, Rangarajan H, Horan J, Krishnamurti L, Shenoy S, Abraham A, Stenger E. Regimen Intensity and Age Affect Transplant-Related Outcomes after Matched Related Donor Hematopoietic Cell Transplantation for Sickle Cell Disease: A STAR Registry Study. Blood 2023, 142: 4904. DOI: 10.1182/blood-2023-182532.Peer-Reviewed Original ResearchHematopoietic cell transplantationRejection-free survivalAnti-thymocyte globulinSickle cell diseaseRelated donor hematopoietic cell transplantationDonor hematopoietic cell transplantationBu/CyShorter hospital stayCumulative doseSevere GVHDHospital stayOverall survivalCell transplantationCell diseaseDay 5Grade III/IVBu/cyclophosphamideBu/FluUse of alemtuzumabKaplan-Meier methodCurrent clinical trialsRisk of rejectionSmall cohort sizeSevere clinical phenotypeConditioning chemotherapyDonor Hemoglobin Genotype Does Not Impact Outcomes Following Matched Related Donor Hematopoietic Cell Transplantation for Sickle Cell Disease: A STAR Study
Stenger E, John T, Chellapandian D, Shah R, Gillespie S, Xiang Y, Liu K, Bhatia M, Guilcher G, Jaroscak J, Kasow K, Krajewski J, Ngwube A, Rangarajan H, Horan J, Krishnamurti L, Shenoy S, Abraham A. Donor Hemoglobin Genotype Does Not Impact Outcomes Following Matched Related Donor Hematopoietic Cell Transplantation for Sickle Cell Disease: A STAR Study. Blood 2023, 142: 4955. DOI: 10.1182/blood-2023-188514.Peer-Reviewed Original ResearchHematopoietic cell transplantationSickle cell diseaseDonor hematopoietic cell transplantationLong-term outcomesPost-HCTOrgan dysfunctionCell transplantationCell diseaseRelated donor hematopoietic cell transplantationSignificant differencesVaso-occlusive pain crisesComparable long-term outcomesLong-term outcome dataLast platelet transfusionMarkers of hemolysisCategorical variablesSymptom-free survivalSignificant organ dysfunctionHb AASickle cell traitContinuous variablesSevere clinical phenotypeSevere disease phenotypeChronic GVHDPain crisisMesangial C3 Deposition, Complement-Associated Variant, and Disease Progression in IgA Nephropathy
Kang Y, Xu B, Shi S, Zhou X, Chen P, Liu L, Li Y, Leng Y, Lv J, Zhu L, Zhang H. Mesangial C3 Deposition, Complement-Associated Variant, and Disease Progression in IgA Nephropathy. Clinical Journal Of The American Society Of Nephrology 2023, 18: 1583-1591. PMID: 37651123, PMCID: PMC10723908, DOI: 10.2215/cjn.0000000000000290.Peer-Reviewed Original ResearchConceptsMesangial C3 depositionGenome-wide association studiesIgA nephropathyComplement factor HC3 depositionGenotype-phenotype correlationIntensity of C3 depositionAssociation studiesLong-term kidney outcomesSusceptibility variantsProgression of IgA nephropathySevere clinical phenotypeKaplan-Meier analysisDeposition of IgAIgA nephropathy cohortIgA nephropathy progressionComplement component 3Chi-square testPrimary GNClinical phenotypeKidney outcomesFactor HGenotypesChinese patientsComplement regulators
2022
Long-Term Organ Function After HCT for SCD: A Report From the Sickle Cell Transplant Advocacy and Research Alliance
Stenger E, Xiang Y, Wetzel M, Gillespie S, Chellapandian D, Shah R, Arnold S, Bhatia M, Chaudhury S, Eckrich M, Kanter J, Kasow K, Krajewski J, Nickel R, Ngwube A, Olson T, Rangarajan H, Wobma H, Guilcher G, Horan J, Krishnamurti L, Shenoy S, Abraham A. Long-Term Organ Function After HCT for SCD: A Report From the Sickle Cell Transplant Advocacy and Research Alliance. Transplantation And Cellular Therapy 2022, 29: 47.e1-47.e10. PMID: 36273784, DOI: 10.1016/j.jtct.2022.10.012.Peer-Reviewed Original ResearchConceptsHematopoietic cell transplantationSickle cell diseaseOrgan functionMultivariable analysisBrain magnetic resonance imagingCentral nervous system indicationsPost-HCT patientsRelated bone marrowSevere acute GVHDPredictors of dysfunctionLong-term survivalMagnetic resonance imagingSevere clinical phenotypeAcute GVHDChronic graftIntense conditioningHost diseaseMyeloablative conditioningNeurologic eventsOvert strokeRetrospective cohortMedian ageOrgan dysfunctionCardiac dysfunctionCell transplantation
2018
KLF1 E325K-associated Congenital Dyserythropoietic Anemia Type IV
Ravindranath Y, Johnson RM, Goyette G, Buck S, Gadgeel M, Gallagher PG. KLF1 E325K-associated Congenital Dyserythropoietic Anemia Type IV. Journal Of Pediatric Hematology/Oncology 2018, 40: e405-e409. PMID: 29300242, PMCID: PMC6092092, DOI: 10.1097/mph.0000000000001056.Peer-Reviewed Original ResearchConceptsCongenital dyserythropoietic anemia type IVClinical courseSevere clinical courseType IVSevere clinical phenotypeIV patientsFetal anemiaTransfusion dependenceFunctional abnormalitiesClinical phenotypePatientsHematologic phenotypeChildrenErythrocyte membranesPhenotypeSplenectomyAnemiaComplete sex reversalFetalisAbnormalities
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