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1.
Phys Ther Res ; 24(1): 52-68, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33981528

RESUMO

OBJECTIVE: Early mobilization and rehabilitation has become common and expectations for physical therapists working in intensive care units have increased in Japan. The objective of this study was to establish consensus-based minimum clinical practice standards for physical therapists working in intensive care units in Japan. It also aimed to make an international comparison of minimum clinical practice standards in this area. METHODS: In total, 54 experienced physical therapists gave informed consent and participated in this study. A modified Delphi method with questionnaires was used over three rounds. Participants rated 272 items as "essential/unknown/non-essential". Consensus was considered to be reached on items that over 70% of physical therapists rated as "essential" to clinical practice in the intensive care unit. RESULTS: Of the 272 items in the first round, 188 were deemed essential. In round 2, 11 of the 62 items that failed to reach consensus in round 1 were additionally deemed essential. No item was added to the "essential" consensus in round 3. In total, 199 items were therefore deemed essential as a minimum standard of clinical practice. Participants agreed that 42 items were not essential and failed to reach agreement on 31 others. Identified 199 items were different from those in the UK and Australia due to national laws, cultural and historical backgrounds. CONCLUSIONS: This is the first study to develop a consensus-based minimum clinical practice standard for physical therapists working in intensive care units in Japan.

2.
Int J Hematol ; 113(2): 290-296, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32865708

RESUMO

Germline GATA2 heterozygous mutations were identified as complex immunodeficiency and hematological syndromes characterized by cytopenia (monocytes, B-cells, NK-cells), susceptibility to mycobacterium, fungus, or Epstein-Barr virus (EBV) infection, and myelodysplastic syndrome (MDS)/acute myelogenous leukemia (AML) development. Herein, we report a patient with AML who had a fatal infection after allogeneic hematopoietic stem cell transplantation (HSCT) due to impaired immune reconstitution associated with GATA2 mutation. A 15-year-old man was diagnosed with AML with monosomy 7. His family history was negative for immunodeficiency and hematological disorders. He attained complete remission after HSCT from an HLA-identical sister. Post-HSCT examinations performed 15 months later revealed pancytopenia, especially monocytopenia and the absence of B and NK cells, resulting in the occurrence of donor-type MDS. Twenty-one months after HSCT, he developed central nervous system aspergillosis and finally died of the disease. Two months later (24 months after PBSCT), the donor was diagnosed with persistent EBV infection accompanied by MDS with multilineage dysplasia. Genetic analysis of GATA2 revealed a novel heterozygous mutation (c.1023_1026dupCGCC) in both siblings. GATA2 mutations were highly prevalent among adolescent MDS/AML patients with monosomy 7. Therefore, the screening of GATA2 mutations in relatives is necessary when performing HSCT from a relative donor.


Assuntos
Fator de Transcrição GATA2/genética , Mutação em Linhagem Germinativa , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/etiologia , Doadores de Tecidos , Biomarcadores , Biópsia , Medula Óssea/patologia , Aberrações Cromossômicas , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/terapia , Transplante Homólogo
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