Rie Shirayama

University of Occupational and Environmental Health Japan

Author Statistics

Papers

Citation

H-Index

i-10 index

Research Trends

Author Order

Document Type

Co-Authors

Koichi Kusuhara

University of Occupational and Environmental Health Japan

Michio Sakai

Yao Tokushukai General Hospital

Tetsuji Sato

Kitakyushu City Hospital Organization

Takuma Ito

University of Occupational and Environmental Health Japan

Koichi Oshida

University of Occupational and Environmental Health Japan

Yasuharu Nishida

Osaka National Hospital

Azusa Nagao

Ogikubo Hospital

Tetsuji Sato

Kitakyushu City Hospital Organization

Akira Shirahata

Kitakyushu City Hospital Organization

Shinji Kunishima

Gifu University of Medical Science

Cooperative Institutions

University of Occupational and Environmental Health Japan

The University of Texas MD Anderson Cancer Center

Ogikubo Hospital

The University of Tokyo

Hyogo Medical University

University Hospital Münster

Kyushu University

Tokyo Medical University

Tokyo Medical University Hospital

Children's Clinical University Hospital

Author Statistics

Papers

Citation

H-Index

i-10 index

Research Field

Catastrophic antiphospholipid syndrome with Epstein–Barr virus-associated hemophagocytosis: A clinicopathological conference

Lupus (2022)

Aya Nawata Rie Shirayama Koichi Oshida Tetsuji Sato Takuma Ito

Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome associated with multiorgan thrombosis in a short term. We present the case of a 14-year-old immunocompetent girl who developed renal, intestinal, and pulmonary infarction; thrombocytopenia; and hemolytic anemia within 1 week. She was diagnosed with thrombotic microangiopathy. Hence, plasma exchange and corticosteroid therapy were initiated, which improved thrombocytopenia. However, the patient's platelet count decreased. Her general condition gradually worsened with eventual death. An autopsy revealed multiple infarctions in the kidneys bilaterally, jejunum, ileum, and pulmonary parenchyma. Microthrombi were not detected. Massive hemophagocytosis was observed in the splenic pulp, lymph nodes, and bone marrow. Several Epstein-Barr viruses (EBVs)-encoded small ribonucleic acid (RNA)-positive lymphocytes were also found in the bone marrow. The presence of antibodies to both viral capsid antigen-immunoglobulin G and EBV nuclear antigen indicated past infection. Antiphospholipid antibody was positive after her death. The patient was finally diagnosed with CAPS and EBV-associated hemophagocytosis, possibly due to EBV reactivation. Establishing a clinical diagnosis of CAPS was relatively difficult because two different causes of thrombocytopenia, CAPS and hemophagocytosis, led to a difficulty in understanding this case's pathogenesis.

Hemophagocytosis

Catastrophic antiphospholipid syndrome

10.1177/09612033221118819

Cite

Citations (1)

Perioperative safety and haematostatic efficacy of a new bypassing agent pd-FVIIa/FX (Byclot) in haemophilia patients with high-responding type inhibitors

Blood Coagulation & Fibrinolysis (2019)

Rie Shirayama Hideyuki Takedani Yushi Chikasawa Akira Ishiguro Masataka Ishimura

: The novel agent pd-FVIIa/FX is a 1 : 10 protein weight mixture of activated factor VII (FVIIa) and factor X (FX) derived from donated blood plasma. A phase III clinical trial of pd-FVIIa/FX revealed high efficacy for bleeding episodes in haemophilia patients with inhibitors. However, up to now, only one case of this new agent being used for surgery had been reported. The objective of this study is to evaluate the perioperative haemostatic efficacy and safety of pd-FVIIa/FX in haemophilia patients with inhibitors. We retrospectively reviewed 25 operation charts from 14 haemophilia patients with high-responding inhibitors using pd-FVIIa/FX during the perioperative period. Efficacy was evaluated by attending physicians and results divided into four groups (excellent, good, fair, and poor). The operation chart was provided by nine Japanese medical institutes with expertise in haemophilia management. Out of the total of 25 surgical procedures, 44% (11/25) were classified as major surgery and the remainders were minor surgeries. In all of the surgeries but one, rFVIIa and/or APCC were administered in combination or sequential method. In all cases except one, the haemostatic efficiency rate was judged as excellent or good by treating physicians for an overall efficacy rate of 96%. No thrombotic adverse effects were reported. This study's results suggest that both combination and sequential therapy of pd-FVIIa/FX and other bypassing agents are well tolerated and effective for the control of perioperative bleeding in haemophilia patients with high-responding inhibitors.

Haemophilia B

10.1097/mbc.0000000000000851

Cite

Citations (7)

Medical care for carriers of hemophilia —carrier testing, carrier check-up and perinatal management

Rie Shirayama Orie Ono Takuma Ito Koichi Oshida Tetsuji Sato

Carrier testing

10.11412/jspho.56.275

Cite

Citations (1)

A Pediatric Case of Acquired Hemophilia A: The Usefulness of the Activated Partial Thromboplastin Time (APTT) Cross-Mixing Test for Early Diagnosis

Journal of UOEH (2018)

Hiroshi Asai Rie Shirayama Koichi Oshida Yuko Honda Tetsuji Sato

後天性血液凝固第Ⅷ因子障害（後天性血友病A）は，凝固第Ⅷ因子（FⅧ）に対する自己抗体（インヒビター）が後天的に出現し，重篤な出血症状を来し得る疾患である．年間発症率は0.25～1人/100万人と言われていたが，疾患概念が浸透したことで成人領域では診断例が増加している．一方，小児では0.045人/100万人と極めてまれな疾患であるが，成人と同様に重篤な出血を起こすため早期診断が重要である．今回，活性化部分トロンボプラスチン時間（APTT）クロスミキシングテストの結果から後天性血友病Aを疑い，早期診断が可能であった小児例を経験した．症例は12歳女児．出血性疾患の家族歴，出血症状の既往はない．誘因のない両側大腿痛を主訴に近医整形外科を受診し，MRIで筋肉内出血を認めた．その後，鼻出血も出現したため出血性疾患を疑われ産業医科大学小児科を受診した．血液検査ではAPTTのみ延長していた．APTT延長の原因精査のために施行したAPTTクロスミキシングテストでは，即時反応より遅延反応でより明確な凝固第Ⅷ因子に対する抗凝固因子（インヒビター）パターンを認めたため，初診時よりインヒビターの存在を強く疑い注意深く経過観察を行った．確定診断後から治療を開始した．筋肉内出血は止血治療を要さず安静のみで軽快し，後天性血友病Aはプレドニゾロン内服のみで寛解した．小児期（12歳）発症の後天性血友病Aは極めてまれであるが，重篤な出血も起こすため早期に診断することが重要であり，APTTクロスミキシングテストが診断に有用であった．

Prothrombin time

Coagulation testing

Bleeding time

10.7888/juoeh.40.331

Cite

Citations (1)

HTRS2023.P2.11 Personalized prophylaxis with simoctocog alfa in adult Japanese previously treated patients with severe hemophilia A

Research and Practice in Thrombosis and Haemostasis (2023)

Yasuharu Nishida Masashi Taki Rie Shirayama Tadashi Matsushita Azusa Nagao

Background: Regular prophylaxis is recommended to prevent bleeds in patients with severe hemophilia A and ideally this should be individualized.The NuPreviq study (GENA-21) conducted in Europe showed that pharmacokinetic (PK)-guided personalized prophylaxis with simoctocog alfa (Nuwiq ® ) provides excellent protection from bleeding, with 83% of patients having zero spontaneous bleeds during 6 months.The GENA-21b study conducted in North America, Europe

10.1016/j.rpth.2023.100234

Cite

Citations (0)

Two Cases of Neonatal Cow's Milk Allergy with a Diversity of Clinical Symptoms

日本未熟児新生児学会雑誌 = Journal of Japan Society for Premature and Newborn Medicine (2013)

Rie Shirayama Shunsuke Araki Mami Eguchi Shutaro Suga Takamitsu Marutani

Source

Cite

Citations (0)

Efficacy and Safety of Personalized Prophylaxis with Simoctocog Alfa in Adult Japanese Previously Treated Patients with Severe Hemophilia a

Blood (2022)

Midori Shima Masashi Taki Rie Shirayama Tadashi Matsushita Yasuharu Nishida