Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Volume 58 , Issue 16
Showing 1-30 articles out of 30 articles from the selected issue
ORIGINAL ARTICLES
  • Tomoyuki Kitagawa, Yasumi Katayama, Ikuhiro Kobori, Yo Fujimoto, Masay ...
    2019 Volume 58 Issue 16 Pages 2277-2282
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Colonic diverticular bleeding often recurs, and readmissions are common. The aim of this study was to identify predictors of colonic diverticular recurrent bleeding and readmission within 90 days.

    Methods Subjects comprised 144 patients diagnosed with colonic diverticular bleeding who received inpatient hospital care between January 2012 and June 2017. A retrospective comparative study was carried out regarding the clinical characteristics during the hospital stay by dividing the cases into 2 groups: patients with recurrent bleeding requiring readmission within 90 days (n=17) and patients without recurrent bleeding (n=127).

    Results A univariate analysis showed that recurrent bleeding and readmission were significantly more frequent among cases with hypovolemic shock on admission (p=0.009), blood transfusion during hospitalization (p=0.029), and hyperlipidemia (p=0.020) than among others. Shock on admission (odds ratio, 5.118; 95% confidence interval, 1.168-22.426, p=0.030) remained a significant predictor on a multivariate analysis.

    Conclusion Shock may predict recurrent colonic diverticular bleeding and readmission within 90 days. Careful and adequate endoscopic hemostasis is recommended for patients showing shock on admission.

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  • Masaya Iwamuro, Haruo Urata, Takehiro Tanaka, Seiji Kawano, Yoshiro Ka ...
    2019 Volume 58 Issue 16 Pages 2283-2289
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective This study aimed to investigate the endoscopic features of lanthanum-associated duodenal lesions and the prevalence of duodenal involvement among patients with pathologically proven lanthanum deposition in the gastrointestinal tract.

    Methods We retrospectively reviewed 24 patients with pathologically proven lanthanum deposition in the gastrointestinal tract. Patients were subdivided into three groups: Group A, patients with pathologically-proven lanthanum deposition in the duodenum; Group B, patients without lanthanum deposition in the duodenum; and Group C, patients without a biopsy of the duodenum.

    Results A biopsy examination of the duodenum was performed in 19 patients, and lanthanum deposition was detected in 17 patients (17/19, 89.5%). In group A (n=17), whitish duodenal villi were detected in 15 patients during esophagogastroduodenoscopy (15/17, 88.2%). While the other two patients showed no whitish villi, a biopsy of the duodenal mucosa revealed lanthanum deposition. The deposition of a white substance showing a clear margin was visible within multiple villi under magnified observation in some patients of group A. Group B patients (n=2) also showed whitish villi. However, the whitish color was faint in one case and sparse in the other case.

    Conclusion Lanthanum deposits in the duodenum may resemble white villi. However, in some cases, these deposits may be unrecognizable during esophagogastroduodenoscopy due to the subtle degree of deposition. Endoscopists should biopsy the duodenum as well as the stomach, regardless of the presence or absence of white villi, for an accurate determination of lanthanum deposition in the gastrointestinal tract.

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  • Atsushi Jogo, Norifumi Nishida, Akira Yamamoto, Ken Kageyama, Mariko N ...
    2019 Volume 58 Issue 16 Pages 2291-2297
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Balloon-occluded retrograde transvenous obliteration (B-RTO) for gastric varices (GV) is associated with drawbacks including a postoperative increase in portal pressure and the risk of subsequent worsening of esophageal varices (EV). Selective B-RTO that embolizes only the varices may have the potential to minimize such risks. The aim of this study is to retrospectively compare the postoperative course of patients after selective B-RTO (Group S) and conventional B-RTO (Group B).

    Methods One hundred four patients treated from January 2007 to April 2012 were classified into Groups S (n=5) and B (n=99). In the univariate analysis, the volume of 5% ethanolamine oleate iopamiodol (EOI) administered at baseline and the GV blood flow on endoscopic ultrasound after B-RTO were considered as covariates. The rates of GV recurrence and EV aggravation was also compared between Groups B and S.

    Results In Group S, the volume of 5% EOI was significantly lower (Group S vs. Group B: 14.6±5.5 vs. 28.5±16.4 mL; p=0.0012) and the rate of EV aggravation was lower in comparison to Group B (p=0.045). However, in Group S, the rate of complete eradication of GV blood flow was significantly lower (Group S vs. Group B: 0% vs. 89.9%; p<0.001) and the rate of re-treatment for GV was higher in comparison to Group B (Group S vs. Group B: 60% vs. 1.0%; p<0.001).

    Conclusion Selective B-RTO for GV could minimize the risk of a worsening of EV or reduce the amount of sclerosants; however, the rate of recurrence was high in comparison to conventional B-RTO.

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  • Jun Urushikubo, Shunichi Yanai, Shotaro Nakamura, Keisuke Kawasaki, Ri ...
    2019 Volume 58 Issue 16 Pages 2299-2304
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Indigo naturalis (IN) is a traditional Chinese medicine that has recently been reported to be effective for ulcerative colitis (UC). The aim of this study was to evaluate the efficacy and safety of IN.

    Methods We performed a retrospective observational study for 14 patients with UC treated with IN from October 2015 to December 2016.

    Results After 8 weeks of oral administration of IN, the partial Mayo score decreased from 4 (2-5) to 1.5 (0-4) [median, interquartile range (IQR), p=0.015]. Among 10 active UC patients, 5 (50%) showed a clinical response, and 4 (40%) achieved clinical remission. Serial changes of endoscopic activity were evaluated in nine patients using the Mayo endoscopic subscore (MES), Rachmilewitz endoscopic index (REI), and UC endoscopy index of severity (UCEIS). The MES decreased from 2 (2-3) to 1 (1-2) [median (IQR), p=0.005], the REI decreased from 7 (5.5-11) to 3 (1-7) [median (IQR), p=0.008], and the UCEIS decreased from 3 (3-4.5) to 1 (0.5-3.5) [median (IQR), p=0.039]. One patient developed acute right-sided colitis with wall thickening and edematous change, and the remaining 13 showed no adverse events.

    Conclusion We conclude that IN is effective for patients with UC as a therapy for inducing remission.

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  • Yu Nishida, Shuhei Hosomi, Hirokazu Yamagami, Naoko Sugita, Shigehiro ...
    2019 Volume 58 Issue 16 Pages 2305-2313
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective The need for and efficacy of immunomodulators for maintaining remission after tacrolimus therapy have not been sufficiently defined. This study evaluated the efficacy of immunomodulators for maintaining remission in patients with ulcerative colitis after tacrolimus therapy.

    Methods Patients with active ulcerative colitis who started oral tacrolimus between January 2009 and September 2017 and were responsive were retrospectively evaluated. Long-term outcomes were compared using Cox proportional hazard regression with inverse probability of treatment weighting.

    Results Among the 63 patients in the study, 45 received immunomodulators. During the follow-up, 30 patients (47.6%) experienced a relapse. The relapse-free survival rate was significantly worse in the group that did not receive immunomodulators than in those that did (p=0.01, log-rank test); the 2-year relapse-free rates were 22.5% and 63.6% in the non-immunomodulator and immunomodulator groups, respectively. A multivariate analysis showed immunomodulator treatment to be an independent protective factor for clinical relapse (adjusted hazard ratio: 0.35, 95% confidence interval: 0.16-0.78, p=0.01). A Cox regression analysis using inverse probability of treatment weighting also showed that immunomodulator maintenance therapy was correlated with a longer relapse-free survival (hazard ratio: 0.31, 95% confidence interval: 0.15-0.64, p<0.01), A similar response was also observed in non-steroid-dependent patients (hazard ratio: 0.36, 95% confidence interval: 0.14-0.99, p=0.047). No serious adverse events occurred due to tacrolimus or immunomodulator, and immunomodulator use did not increase the incidence of adverse events caused by tacrolimus.

    Conclusion Our data suggest that the use of immunomodulators to maintain remission after tacrolimus therapy is beneficial for patients with ulcerative colitis.

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  • Yota Koyabu, Shichiro Abe, Masashi Sakuma, Tomoaki Kanaya, Syotaro Obi ...
    2019 Volume 58 Issue 16 Pages 2315-2322
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Although several clinical trials have shown that the mid- and long-term safety and efficacy of prasugrel are better than those of clopidogrel after percutaneous coronary intervention (PCI), there are few data regarding the short-term safety.

    Methods In this study, we retrospectively analyzed the short-term (72 hours) PCI-related bleeding complications and mid-term (12 months) efficacy in 250 consecutive coronary artery disease patients who underwent PCI and received aspirin plus prasugrel (prasugrel group; 67.7±10.0 years, 200 men).

    Patients The comparison group consisted of 250 age- and gender-matched patients who received aspirin plus clopidogrel (clopidogrel group: 67.2±11.2 years, 199 men).

    Results The incidence of a composite of PCI-related bleeding complications in the acute phase post-PCI was significantly higher in the prasugrel group than in the clopidogrel group (22.4% vs. 13.2%, p=0.007), although the incidence of non-PCI-related bleeding complications over 12 months was comparable between the 2 groups. The cumulative incidence of major cardiovascular events (MACEs) was comparable between the prasugrel and clopidogrel groups (log-rank test; p=0.561). A multivariate logistic regression analysis of the 250 prasugrel-treated patients showed that acute coronary syndrome tended to be negatively associated with the incidence of PCI-related bleeding complications (p=0.061).

    Conclusion Prasugrel and clopidogrel may have similar efficacy for preventing cardiovascular events as the post-PCI antiplatelet regimen; however, prasugrel should be used cautiously because of the risk of PCI-related bleeding complications.

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  • Fumiya Saito, Shigeru Toyoda, Takuo Arikawa, Shu Inami, Ryo Watanabe, ...
    2019 Volume 58 Issue 16 Pages 2323-2331
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Embolic events are frequent and life-threatening complications of infective endocarditis (IE). Recently, an embolic risk assessment at admission, based on the Embolic Risk (ER) French Calculator, was designed to predict the development of symptomatic emboli associated with IE. This study aimed to validate the ER French Calculator for the prediction of in-hospital events, including embolic events.

    Methods We retrospectively analyzed the clinical features of 52 consecutive patients with left-sided IE to identify possible predictors of in-hospital events within 30 days of admission.

    Results New embolic events were seen in 15 patients (29%), cardiac surgery was performed in 22 patients (42%), and 1 patient (2%) died within 30 days of admission. A composite endpoint of embolic complications, cardiac surgery, or death was observed in 28 patients (54%). The cumulative incidence of new embolic events was significantly higher in the high-risk group identified by the ER French Calculator than in the low-risk group (log-rank test; p=0.0004). The incidence of the composite endpoint was higher in the high-risk group than in the low-risk group (log-rank test; p<0.0001). A multivariate Cox proportional hazards model indicated that the high-risk designation on the ER French Calculator predicted embolic events (p=0.0410) and composite events (p=0.0371) independently of other candidate predictors.

    Conclusion The ER French Calculator may be a useful tool for predicting new in-hospital embolic events and other unfavorable in-hospital events in patients with IE.

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  • Yoshimasa Morimoto, Nobuhiro Nishii, Saori Tsukuda, Satoshi Kawada, Ma ...
    2019 Volume 58 Issue 16 Pages 2333-2340
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Remote monitoring (RM) of cardiac implantable electric devices (CIEDs) has been advocated as a healthcare standard. However, expert consensus statements suggest that all patients require annual face-to-face follow-up consultations at outpatient clinics even if RM reveals no episodes. The objective of this study was to determine the critical event rate after CIED implantation through RM.

    Methods This multicenter, retrospective, cohort study evaluated patients with pacemakers (PMs), implantable cardioverter defibrillators (ICDs), or cardiac resynchronization therapy defibrillator (CRT-Ds) and analyzed whether or not the data drawn from RM included abnormal or critical events.

    Patients A total of 1,849 CIED patients in 12 hospitals who were followed up by the RM center in Okayama University Hospital were included in this study.

    Results During the mean follow-up period of 774.9 days, 16,560 transmissions were analyzed, of which 11,040 (66.7%) were abnormal events and only 676 (4.1%) were critical events. The critical event rate in the PM group was significantly lower than that in the ICD or CRT-D groups (0.9% vs. 5.0% or 5.9%, p<0.001). A multivariate analysis revealed that ICD, CRT-D, and a low ejection fraction were independently associated with critical events. In patients with ICD, the independent risk factors for a critical event were old age, low ejection fraction, Brugada syndrome, dilated phase hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy.

    Conclusion Although abnormal events were observed in two-thirds of the transmitted RM data, the critical event rate was <1% in patients with a PM, which was lower in comparison to the rates in patients with ICDs or CRT-Ds. A low ejection fraction was an independent predictor of critical events.

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  • Xueyan Zhou, Chunxia Chen, Di Yin, Feng Zhao, Zejun Bao, Yun Zhao, Xi ...
    2019 Volume 58 Issue 16 Pages 2341-2347
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Previous studies have suggested that variations in the ABCC8 gene may be closely associated with T2DM susceptibility and repaglinide response. However, these results have not been entirely consistent, and there are no related studies in a Chinese population, suggesting the need for further exploration. The current study investigated the associations of the ABCC8 rs1801261 polymorphism with type 2 diabetes mellitus (T2DM) susceptibility and repaglinide therapeutic efficacy in Chinese Han T2DM patients.

    Methods A total of 234 T2DM patients and 105 healthy subjects were genotyped for ABCC8 rs1801261 polymorphism by a polymerase chain reaction-restriction fragment length polymorphism assay. A total of 70 patients with the same genotypes of CYP2C8*3 139Arg and OATP1B1 521TT were randomized to orally take 3 mg repaglinide per day (1 mg each time before meals) for 8 consecutive weeks. The pharmacodynamic parameters of repaglinide and biochemical indicators were then determined before and after repaglinide treatment.

    Results The frequency of ABCC8 rs1801261 allele was higher in T2DM patients than in the control subjects (22.6% vs.11.0%, p<0.01). After repaglinide treatment, T2DM patients carrying genotype CT showed a significantly attenuated efficacy on FPG (p<0.01) and HbA1c (p<0.01) compared with those with genotype CC.

    Conclusion These results suggested that the ABCC8 rs1801261 polymorphism might influence T2DM susceptibility and the therapeutic effect of repaglinide in Chinese Han T2DM patients. This study was registered in the Chinese Clinical Trial Register on May 14, 2013 (No. ChiCTR-CCC13003536).

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  • Keisuke Kakisaka, Aiko Sakai, Yuichi Yoshida, Akio Miyasaka, Fumiaki T ...
    2019 Volume 58 Issue 16 Pages 2349-2355
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Objective Since healthcare providers face an increased risk of hepatitis B virus (HBV) infection because of their work, vaccination plays a critical role in preventing HBV transmission. However, the duration for which acquired HBV surface antibodies (anti-HBs) persist remains unknown. To evaluate the primary immunologic response to HBV vaccination and its persistence in healthy Japanese adolescents.

    Methods In total, 690 young adults underwent HBV vaccination with a three-dose schedule. The primary response was determined by the anti-HBs titers at 1-2 months after the final dosage. Subjects with anti-HBs titers of <10, 10-100, and >100 mIU/mL were classified as "non-responders," "low-responders," and "sufficient responders," respectively. Anti-HB titers were re-measured at 1 or 2 years after vaccination.

    Results First, 95.8% and 72.8% of the subjects had anti-HBs titers of >10 and >100 mIU/mL, respectively, as a primary response. The anti-HBs titers measured at 1 and 2 years after vaccination were significantly correlated with those of the primary response (1 year: r=0.893, p<0.0001; 2 years: r=0.902, p<0.001). Most subjects with a titer of >100 mIU/mL at the primary response maintained an anti-HBs titer of >10 mIU/mL [1 year after vaccination, 208/209 (99.5%); 2 years after vaccination, 72/81 (90.1%)]. However, in subjects with a primary response of 10-100 mIU/mL the anti-HBs titer frequently declined; 17/38 (44.7%) and 9/10 (90.0%) subjects had a titer of <10 mIU/mL at 1 and 2 years, respectively.

    Conclusion The primary response was associated with the anti-HBs titers at 1 and 2 years after vaccination, and the anti-HBs titers of 54.2% of the low responders were not maintained for 2 years, even if they were vaccinated as healthy young adults.

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CASE REPORTS
  • Kohei Oka, Ken Inoue, Naoto Iwai, Tasuku Hara, Yutaka Inada, Toshifumi ...
    2019 Volume 58 Issue 16 Pages 2357-2362
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Inflammatory fibroid polyp (IFP) appears most often in the stomach. We herein report an extremely rare case of esophageal IFP resected using laparoscopy endoscopy cooperative surgery (LECS). A 73-year-old man with dysphagia underwent esophagogastroduodenoscopy. A 50-mm semi-pedunculated submucosal tumor was observed in the esophagogastric junction. Endoscopic ultrasonography showed a homogeneous, hypoechoic, and well-vascularized tumor in the second and third layers of the stomach, but endoscopic ultrasonography-guided fine-needle aspiration failed to establish a definite diagnosis. Since he was suffering from dysphagia, LECS was performed. The resected specimen proved that the tumor was an IFP originating from the esophagus.

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  • Ryotaro Maeda, Daisuke Naito, Atsuo Adachi, Hirokazu Shiraishi, Takash ...
    2019 Volume 58 Issue 16 Pages 2363-2366
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    A 61-year-old Japanese man with IgG4-related autoimmune pancreatitis developed a mass in the right atrium (RA) and a mass lesion surrounding the left anterior descending coronary artery. We performed an intracardiac echo catheter-guided percutaneous biopsy of the RA mass, and histologically diagnosed it as IgG4-related disease. Oral corticosteroid therapy gradually downsized the mass lesions. We encountered a very rare case with mass lesions in the cardiovascular system of the IgG4-related disease that were able to be diagnosed using an intracardiac echo-guided biopsy.

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  • Keisuke Matsuo, Takashi Ishiguro, Takatomo Najama, Yoshihiko Shimizu, ...
    2019 Volume 58 Issue 16 Pages 2367-2372
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    A 62-year-old man presented to our hospital for the further evaluation and treatment of his back pain, general fatigue, and dyspnea, which had developed 4 days after the 29th administration of nivolumab to treat his lung cancer. Based on his clinical history, elevated serum cardiac enzyme values, and cardiac magnetic resonance (CMR) imaging and myocardial biopsy findings, he was diagnosed with myocarditis induced by nivolumab. Corticosteroid therapy improved his condition, and CMR performed on hospital day 11 also showed remarkable improvement. Although nivolumab-induced myocarditis is rare, cardiologists should consider it when encountering patients treated with such a drug for malignant disease.

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  • Tamami Nakagawa, Mika Mori, Hayato Tada, Miho Ohira, Kenji Iino, Kensh ...
    2019 Volume 58 Issue 16 Pages 2373-2376
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Aorto-mitral discontinuity is considered a relatively rare complication of infective endocarditis, usually requiring invasive surgical procedures, including double valve replacement in addition to aortic root replacement/repair. In the present case report, images were produced using transthoracic echocardiograms and transesophageal echocardiograms, which may assist in the planning of surgical procedures. The images captured using real-time three-dimensional transesophageal echocardiograms revealed aorto-mitral discontinuity and a perforated mitral valve aneurysm of the anterior leaflet.

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  • Mirai Matsumura, Chikage Oshita, Yuichi Fujii, Tomohiro Ueda, Hiroki T ...
    2019 Volume 58 Issue 16 Pages 2377-2381
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    The spasm provocation test (SPT) is important for diagnosing vasospastic angina (VSA), and acetylcholine (ACh) is usually used for this test in Japan. However, some patients with VSA have negative SPT results with the use of the standard ACh regimen alone. We herein report two cases in which VSA was diagnosed by the SPT with the combined use of ACh and ergonovine (EM). VSA could not be diagnosed in either case by the SPT using ACh infusions alone. For patients with negative SPT results, cardiologists should consider performing the SPT using a combination of ACh and EM.

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  • Mami Orimo, Mitsuko Kondo, Kiyoshi Takeyama, Kazuhiro Abe, Azusa Miyos ...
    2019 Volume 58 Issue 16 Pages 2383-2386
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    A 33-year-old woman presented with a productive cough from childhood. She had suffered from repeated bacterial pneumonia. Her clinical and imaging findings revealed chronic sinusitis, bronchiectasis and situs inversus. We suspected primary ciliary dyskinesia (PCD) and performed a bronchial mucosal biopsy. The ciliary beat pattern according to high-speed video microscopy was complete loss. Electron microscopic findings of cilia showed defect of outer dynein arm (ODA). A genetic examination detected compound heterozygous mutations of DNAH5 that encode ODA components. There are few reports of genetic mutation analyses in Japanese PCD patients. We herein report a PCD patient with DNAH5 mutations and review the related literature.

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  • Akane Kunitomi, Yuta Hasegawa, Junji lmamura, Yoshiyuki Yokomaku, Taka ...
    2019 Volume 58 Issue 16 Pages 2387-2391
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: May 22, 2019
    JOURNALS OPEN ACCESS

    Acute promyelocytic leukemia (APL) in human immunodeficiency virus (HIV)-infected individuals is very rare. There is currently no consensus regarding the use of anti-cancer drugs with highly active anti-retroviral therapy (ART) in these patients due to their small number. We herein report two cases of APL with HIV-infected patients. Both cases received all-trans-retinoic acid-containing chemotherapies and achieved complete remission. ART was continued throughout the treatment course. The clinical course of these cases suggests that it is preferable to perform standard chemotherapy for APL with ART if patients have an adequate performance status.

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  • Yuji Kato, Takeshi Hayashi, Ritsushi Kato, Masaki Takao
    2019 Volume 58 Issue 16 Pages 2393-2395
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: April 17, 2019
    JOURNALS OPEN ACCESS

    Catheter ablation for atrial fibrillation creates an iatrogenic atrial septal defect by transseptal puncture, which may produce a transient right-to-left shunt. We encountered a 44-year-old man who presented with de novo migraine-like headache after cryoballoon ablation for atrial fibrillation. On reviewing the literature, we found additional cases in which migraine-like headache occurred within one week after the procedure and spontaneously within three months. We should recognize migraine-like headache as a potential complication of catheter ablation.

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  • Haitian Nan, Ryusuke Takaki, Keisuke Shimozono, Yuta Ichinose, Kishin ...
    2019 Volume 58 Issue 16 Pages 2397-2400
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: April 17, 2019
    JOURNALS OPEN ACCESS

    Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17) with mutations in the MAPT gene is a hereditary neurodegenerative tauopathy with various clinical phenotypes. We herein report the first Japanese patient with FTDP-17 caused by an IVS10+3G>A mutation in the MAPT gene, which is linked to an H1M haplotype. The present study suggests that the IVS10+3G>A mutation in the MAPT gene can have originated from a non-Caucasian population. In the disease course, myoclonus and respiratory failure can be observed. This study may expand on the clinical and genetic findings for FTDP-17 with mutations in the MAPT gene.

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  • Ryo Koda, Masafumi Tsuchida, Noriaki Iino, Masaki Murata, Kohei Inui, ...
    2019 Volume 58 Issue 16 Pages 2401-2406
    Published: August 15, 2019
    Released: August 15, 2019
    [Advance publication] Released: April 17, 2019
    JOURNALS OPEN ACCESS

    A 56-year-old man was referred to our facility after developing edema in his right leg. Enhanced computed tomography (CT) revealed a periarterial soft tissue mass surrounding the right iliac artery compressing the iliac vein. His elevated serum IgG4 level indicated IgG4-related disease (IgG4-RD). Both a percutaneous and surgical biopsy of this periarterial lesion proved challenging and were not pursued. A prostate biopsy, however, finally provided a histological diagnosis of IgG4-RD. Oral steroid therapy successfully resolved his leg edema and periarterial lesion. This case illustrates the usefulness of an alternative prostate biopsy for the histological diagnosis of IgG4-RD when approaching the primary affected lesion is difficult.

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