Pulmonary blastoma is rare and its prognosis very poor. A 6-year-old boy was referred to our hospital with chest pain. Computed tomography demonstrated that the left pleural cavity was filled with a tumor. Cardiac echocardiography demonstrated that the tumor had invaded through the pulmonary vein into the left atrium and that the tumor extended into the left ventricle. Part of the tumor was adhered to the anterior leaflet of the mitral valve. To increase operative radicality, an autotransplantation technique was performed concomitantly with resection of the original lesion. Through a median sternotomy, a moderate hypothermic cardiopulmonary bypass was established to obtain cardiac arrest. First, longitudinal incision of right-sided of the left atrium was made. The tumor invaded into the left atrium through the left superior pulmonary orifice. The ascending aorta, the main pulmonary artery, and both caval veins were transected. The left atrium was incised along the pulmonary venous orifices. The heart was completely removed from the mediastinum and transferred to another table. Resection of the intracardiac metastatic lesion and mitral valve replacement was accomplished. During this time, thoracic surgeons performed a left pneunectomy. The left atrial wall around the left pulmonary venous orifices was resected in combination with the left lung. After the deficit of the left atrial wall was repaired with a Gore-Tex patch, the heart was replaced and we reconstructed the great arteries and caval veins. The autotransplantation technique is a useful procedure for combined lesions of the heart and lung.

We describe Takotsubo syndrome, which developed after elective mitral valve repair and tricuspid annuloplasty in a 76-year-old woman. A preoperative echocardiogram confirmed severe mitral regurgitation due to posterior leaflet prolapse, moderate tricuspid regurgitation, and normal left ventricular function. Mitral valve repair and tricuspid annuloplasty were performed. After uneventful weaning off cardiopulmonary bypass, intraoperative transesophageal echocardiography revealed adequate mitral leaflet function and normal left ventricular contractions. After being transferred to the intensive care unit, the patient's hemodynamic parameters progressively deteriorated. Transthoracic echocardiography showed akinesis and ballooning of the apex and hyperkinesis of the base, and the ejection fraction was 20% on postoperative day 1. The serum aminotransferase and CPK-MB levels increased on postoperative day 2. The left ventricular function did not improve despite supportive therapy with vasopressors. She developed cardiogenic cerebral infarction due to obstruction of the right middle cerebral artery on postoperative day 8. Endovascular thrombectomy was performed within 2 h of the onset of cerebral infarction. Thereafter, the patient gradually recovered and was discharged without any sequelae on postoperative day 31. The ejection fraction was 65% with normal left ventricular motion at discharge. An electrocardiogram revealed a deep negative T wave in II, III, aVF, and V3-V6. After 2 months, the electrocardiogram findings were normalized. Coronary lesions were not observed on pre- or postoperative coronary angiographies; therefore, we diagnosed Takotsubo cardiomyopathy after mitral valve repair. Takotsubo cardiomyopathy should be considered as a possible complication of cardiac surgery, especially after mitral valve surgery.

BACKGROUND/AIMS: Noninvasive objective monitoring is advantageous for optimizing treatment strategies in patients inflammatory bowel disease (IBD). Fecal calprotectin (FCP) is superior to traditional biomarkers in terms of assessing the activity in patients with IBD. However, there are the differences among several FCP assays in the dynamics of FCP. In this prospective multicenter trial, we investigated the usefulness of FCP measurements in adult Japanese patients with IBD by reliable enzyme immunoassay using a monoclonal antibody. METHODS: We assessed the relationship between FCP levels and disease or endoscopic activity in patients with ulcerative colitis (UC, n=64) or Crohn’s disease (CD, n=46) compared with healthy controls (HCs, n=64). RESULTS: FCP levels in UC patients strongly correlated with the Disease Activity Index (rs =0.676, P < 0.0001) and Mayo endoscopic subscore (MES; rs =0.677, P < 0.0001). FCP levels were significantly higher even in patients with inactive UC or CD compared with HCs (P=0.0068, P < 0.0001). The optimal cutoff value between MES 1 and 2 exhibited higher sensitivity (94.1%). FCP levels were significantly higher in active UC patients than in inactive patients (P < 0.001), except those with proctitis. The Crohn’s Disease Activity Index tended to correlate with the FCP level (rs =0.283, P=0.0565). CONCLUSIONS: Our testing method using a monoclonal antibody for FCP was well-validated and differentiated IBD patients from HCs. FCP may be a useful biomarker for objective assessment of disease activity in adult Japanese IBD patients, especially those with UC.
Adult* ; Antibodies, Monoclonal ; Asian Continental Ancestry Group* ; Biomarkers ; Colitis, Ulcerative ; Crohn Disease ; Humans ; Immunoenzyme Techniques ; Inflammatory Bowel Diseases* ; Leukocyte L1 Antigen Complex* ; Methods ; Multicenter Studies as Topic ; Proctitis ; Prospective Studies*

BACKGROUND/AIMS: Recent genome-wide analyses have provided strong evidence concerning adverse events caused by thiopurine drugs such as azathioprine (AZA) and 6-mercaptopurine. The strong associations identified between NUDT15 p.Arg139Cys and thiopurine-induced leukopenia and severe hair loss have been studied and confirmed over the last 2 years. However, other coding variants, including NUDT15 p.Val18_Val19insGlyVal, NUDT15 p.Val18Ile, and FTO p.Ala134Thr, and a noncoding variation in RUNX1 (rs2834826) remain to be examined in detail in this respect. Therefore, we investigated the correlation between these adverse events and the 5 recently identified variants mentioned above among Japanese patients with inflammatory bowel diseases (IBD). METHODS: One hundred sixty thiopurine-treated patients with IBD were enrolled. Genotyping was performed using TaqMan SNP Genotyping Assays or Sanger sequencing. RESULTS: None of the 5 variants were associated with gastrointestinal intolerance to AZA. However, NUDT15 p.Arg139Cys was significantly associated with the interval between initiation and discontinuation of AZA among patients with gastrointestinal intolerance. This variant was strongly associated with early (<8 weeks) and late (≥8 weeks) leukopenia and severe hair loss. Moreover, it correlated with the interval between initiation of thiopurine therapy and leukopenia occurrence, and average thiopurine dose. NUDT15 p.Val18_Val19insGlyVal, NUDT15 p.Val18Ile, FTO p.Ala134Thr, and RUNX1 rs2834826 exhibited no significant relationship with the adverse events examined. CONCLUSIONS: Of the 5 variants investigated, NUDT15 p.Arg139Cys had the strongest impact on thiopurine-induced leukopenia and severe hair loss; therefore, its genotyping should be prioritized over that of other variants in efforts to predict these adverse events in Japanese patients with IBD.
6-Mercaptopurine ; Asian Continental Ancestry Group* ; Azathioprine ; Clinical Coding ; Hair ; Humans ; Inflammatory Bowel Diseases* ; Leukopenia

Prevention, early diagnosis, and early treatment of skeletal-related events (SREs) are important in the treatment of potential or current cases of bone metastasis. In August 2020, our hospital established the bone metastasis team and the bone metastasis board (BMB) started actively engaging in activities aimed at improving the outcome of bone metastasis. We retrospectively examined whether a combined modality therapy started in the diagnosis of bone metastases could prevent the onset of SREs and whether it could prolong survival and improve activities of daily living. The 75 advanced cancer patients who underwent BMB at our hospital from August 1, 2020 to July 31, 2022 were divided into two groups according to when BMB performed before and after SREs for comparative analysis. Numerical Rating Scale improved, however Performance Status did not improve in both groups, and there was no difference in survival between the both groups (15.3 vs. 9.0 months, HR: 0.74, 95%; CI: 0.42–1.29, p=0.29). In conclusion, patients who suffered from SREs from the time of bone metastasis diagnosis were treated early. However, the incidence of SREs after BMB in our hospital was 22.6%, and it is necessary to actively work to prevent SREs in the future.