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2.
Pediatrics ; 147(1)2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-33328337

RESUMEN

Pediatric sepsis is a major public health problem. Published treatment guidelines and several initiatives have increased adherence with guideline recommendations and have improved patient outcomes, but the gains are modest, and persistent gaps remain. The Children's Hospital Association Improving Pediatric Sepsis Outcomes (IPSO) collaborative seeks to improve sepsis outcomes in pediatric emergency departments, ICUs, general care units, and hematology/oncology units. We developed a multicenter quality improvement learning collaborative of US children's hospitals. We reviewed treatment guidelines and literature through 2 in-person meetings and multiple conference calls. We defined and analyzed baseline sepsis-attributable mortality and hospital-onset sepsis and developed a key driver diagram (KDD) on the basis of treatment guidelines, available evidence, and expert opinion. Fifty-six hospital-based teams are participating in IPSO; 100% of teams are engaged in educational and information-sharing activities. A baseline, sepsis-attributable mortality of 3.1% was determined, and the incidence of hospital-onset sepsis was 1.3 cases per 1000 hospital admissions. A KDD was developed with the aim of reducing both the sepsis-attributable mortality and the incidence of hospital-onset sepsis in children by 25% from baseline by December 2020. To accomplish these aims, the KDD primary drivers focus on improving the following: treatment of infection; recognition, diagnosis, and treatment of sepsis; de-escalation of unnecessary care; engagement of patients and families; and methods to optimize performance. IPSO aims to improve sepsis outcomes through collaborative learning and reliable implementation of evidence-based interventions.


Asunto(s)
Educación Continua , Evaluación de Procesos y Resultados en Atención de Salud , Mejoramiento de la Calidad , Sepsis/terapia , Niño , Adhesión a Directriz , Hospitales Pediátricos , Humanos , Guías de Práctica Clínica como Asunto , Estados Unidos
3.
Genes Chromosomes Cancer ; 58(8): 551-557, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-30706625

RESUMEN

The t(7;21)(p22;q22) resulting in RUNX1-USP42 fusion, is a rare but recurrent cytogenetic abnormality associated with acute myeloid leukemia (AML) and myelodysplastic syndromes. The prognostic significance of this translocation has not been well established due to the limited number of patients. Herein, we report three pediatric AML patients with t(7;21)(p22;q22). All three patients presented with pancytopenia or leukopenia at diagnosis, accompanied by abnormal immunophenotypic expression of CD7 and CD56 on leukemic blasts. One patient had t(7;21)(p22;q22) as the sole abnormality, whereas the other two patients had additional numerical and structural aberrations including loss of 5q material. Fluorescence in situ hybridization analysis on interphase cells or sequential examination of metaphases showed the RUNX1 rearrangement and confirmed translocation 7;21. Genomic SNP microarray analysis, performed on DNA extracted from the bone marrow from the patient with isolated t(7;21)(p22;q22), showed a 32.2 Mb copy neutral loss of heterozygosity (cnLOH) within the short arm of chromosome 11. After 2-4 cycles of chemotherapy, all three patients underwent allogeneic hematopoietic stem cell transplantation (HSCT). One patient died due to complications related to viral reactivation and graft-versus-host disease. The other two patients achieved complete remission after HSCT. Our data displayed the accompanying cytogenetic abnormalities including del(5q) and cnLOH of 11p, the frequent pathological features shared with other reported cases, and clinical outcome in pediatric AML patients with t(7;21)(p22;q22). The heterogeneity in AML harboring similar cytogenetic alterations may be attributed to additional uncovered genetic lesions.


Asunto(s)
Cromosomas Humanos Par 21 , Cromosomas Humanos Par 7 , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/genética , Translocación Genética , Adolescente , Factores de Edad , Biomarcadores , Biomarcadores de Tumor , Niño , Femenino , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Pruebas Genéticas , Humanos , Inmunofenotipificación , Masculino , Fenotipo
4.
J La State Med Soc ; 160(3): 138-41, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18655650

RESUMEN

We present a child with a cerebellar medulloblastoma, diagnosed at age three, treated with near total surgical resection, radiotherapy, and chemotherapy, that recurred 13 years after the initial diagnosis. This late recurrence exceeds the typical 10-year survival statistics that are in common use, and exceeds the Collins rule. Continued follow-up of these children is justified to increase the likelihood of detecting these late recurrences early and to learn more about these late recurrences.


Asunto(s)
Neoplasias Cerebelosas/diagnóstico , Meduloblastoma/diagnóstico , Recurrencia Local de Neoplasia/diagnóstico , Adolescente , Neoplasias Cerebelosas/fisiopatología , Neoplasias Cerebelosas/cirugía , Humanos , Masculino , Meduloblastoma/fisiopatología , Meduloblastoma/cirugía , Factores de Tiempo
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