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Primary ciliary dyskinesia in Japan: systematic review and meta-analysis.
Inaba, Atsushi; Furuhata, Masanori; Morimoto, Kozo; Rahman, Mahbubur; Takahashi, Osamu; Hijikata, Minako; Knowles, Michael R; Keicho, Naoto.
Afiliación
  • Inaba A; Department of Pathophysiology and Host Defense, the Research Institute of Tuberculosis, Japan Anti-tuberculosis Association, 3-1-24, Matsuyama, Kiyose, Tokyo, 204-8533, Japan.
  • Furuhata M; Department of Respiratory Medicine, Graduate School of Medicine, The University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
  • Morimoto K; Department of Pathophysiology and Host Defense, the Research Institute of Tuberculosis, Japan Anti-tuberculosis Association, 3-1-24, Matsuyama, Kiyose, Tokyo, 204-8533, Japan.
  • Rahman M; Dapartment of Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
  • Takahashi O; Department of Pathology, National Center for Global Health and Medicine, 1-21-1, Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.
  • Hijikata M; Fukujuji Hospital, Japan Anti-tuberculosis Association, Respiratory Disease Center, 3-1-24 Matsuyama, Kiyose, Tokyo, 204-0022, Japan.
  • Knowles MR; Center for Clinical Epidemiology, St. Luke's International University Graduate School of Public Health, 3-6 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
  • Keicho N; Center for Clinical Epidemiology, St. Luke's International University Graduate School of Public Health, 3-6 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
BMC Pulm Med ; 19(1): 135, 2019 Jul 25.
Article en En | MEDLINE | ID: mdl-31345208
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare genetic disorder. Although the genetic tests and new diagnostic algorithms have recently been recommended, clinical signs and electron microscope (EM) findings have historically been the mainstays of diagnosis in Asia. To characterize PCD previously reported in Japan, we conducted a systematic review and meta-analysis. METHODS: A search using MEDLINE, EMBASE, and Japana Centra Revuo Medicina (in Japanese) databases was carried out to identify articles reporting PCD, Kartagener syndrome, or immotile cilia syndrome in Japanese patients and published between 1985 and 2015. RESULTS: After excluding duplication from 334 reports, we extracted 316 patients according to the criteria. Diagnosis was most frequently made in adulthood (148 patients [46.8%] ≥ 18 years old, 24 patients [7.6%] < 1 year old, 68 patients [21.5%] 1-17 years old and 76 patients [24.1%] lacking information). Of the 230 patients (72.8%) who received EM examination, there were patients with inner dynein arm (IDA) defects (n = 55; 23.9%), outer dynein arm (ODA) defects (14; 6.1%), both ODA and IDA defects (57; 24.8%), other structural abnormalities (25; 10.9%), no abnormalities (4; 1.7%), and no detailed conclusion or description (75; 32.6%). CONCLUSION: Delayed diagnosis of this congenital disease with high frequency of IDA defects and low frequency of ODA defects appear to be historical features of PCD reported in Japan, when EM was a main diagnostic tool. This review highlights problems experienced in this field, and provides basic information to establish a modernized PCD diagnosis and management system in the future.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndrome de Kartagener / Dineínas Tipo de estudio: Diagnostic_studies / Prognostic_studies / Systematic_reviews Límite: Humans País/Región como asunto: Asia Idioma: En Revista: BMC Pulm Med Año: 2019 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndrome de Kartagener / Dineínas Tipo de estudio: Diagnostic_studies / Prognostic_studies / Systematic_reviews Límite: Humans País/Región como asunto: Asia Idioma: En Revista: BMC Pulm Med Año: 2019 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Reino Unido