Kei Inai

Publications (2)7.34 Total impact

• Source

  Available from: Mitsunori Nishiyama

  Article: Outcomes of childhood pulmonary arterial hypertension in BMPR2 and ALK1 mutation carriers.

  Ayako Chida, Masaki Shintani, Hisato Yagi, Maya Fujiwara, Yasuko Kojima, Hiroki Sato, Shinichiro Imamura, Masato Yokozawa, Norio Onodera, Hitoshi Horigome, [......], Tatsuya Kawano, Yuichi Nomura, Hong Gu, Takahiro Ishiwata, Yoshiyuki Furutani, Kei Inai, Tsutomu Saji, Rumiko Matsuoka, Shigeaki Nonoyama, Toshio Nakanishi
  [show abstract] [hide abstract]
  ABSTRACT: Mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene and the activin receptor-like kinase 1 (ALK1) gene have been reported in heritable pulmonary arterial hypertension (HPAH) and idiopathic pulmonary arterial hypertension (IPAH). However, the relation between clinical characteristics and each gene mutation in IPAH and HPAH is still unclear, especially in childhood. The aim of this study was to determine, in a retrospective study, the influence and clinical outcomes of gene mutations in childhood IPAH and HPAH. Fifty-four patients with IPAH or HPAH whose onset of disease was at <16 years of age were included. Functional characteristics, hemodynamic parameters, and clinical outcomes were compared in BMPR2 and ALK1 mutation carriers and noncarriers. Overall 5-year survival for all patients was 76%. Eighteen BMPR2 mutation carriers and 7 ALK1 mutation carriers were detected in the 54 patients with childhood IPAH or HPAH. Five-year survival was lower in BMPR2 mutation carriers than mutation noncarriers (55% vs 90%, hazard ratio 12.54, p = 0.0003). ALK1 mutation carriers also had a tendency to have worse outcome than mutation noncarriers (5-year survival rate 64%, hazard ratio 5.14, p = 0.1205). In conclusion, patients with childhood IPAH or HPAH with BMPR2 mutation have the poorest clinical outcomes. ALK1 mutation carriers tended to have worse outcomes than mutation noncarriers. It is important to consider aggressive treatment for BMPR2 or ALK1 mutation carriers.
  The American journal of cardiology 05/2012; 110(4):586-93. · 3.58 Impact Factor
• Article: Missense Mutations of the (BMPR1B) ((ALK6)) Gene in Childhood Idiopathic Pulmonary Arterial Hypertension.

  Ayako Chida, Masaki Shintani, Tomotaka Nakayama, Yoshiyuki Furutani, Emiko Hayama, Kei Inai, Tsutomu Saji, Shigeaki Nonoyama, Toshio Nakanishi
  [show abstract] [hide abstract]
  ABSTRACT: (Circ J 2012; 76: 1501-1508).
  Circulation Journal 01/2012; 76(7):1804. · 3.77 Impact Factor