Objective There are no objective methods for evaluating the severity of vasospasms in patients with refractory coronary spastic angina (R-CSA) under adequate medical therapy. We examined whether spasm provocation tests performed under adequate medication are useful for evaluating the severity of disease in R-CSA patients on emergency admission.
Methods and Results We performed spasm provocation tests before and after the administration of medical therapy in eight R-CSA patients, including one ventricular fibrillation survivor (VF-S) and seven patients with unstable angina (UAP) on emergency readmission. We also performed these tests only after medical therapy on urgent admission in four R-CSA patients, including two patients with UAP, one patient with VF-S and one patient with acute coronary syndrome. All 12 R-CSA patients had been medicated with ≥2 vasodilator drugs. Positive coronary spasms were defined as >99% transient narrowing. The coronary artery spasms disappeared in three patients under medication, and mitigation of vasospasticity was observed in three patients. In these six cases we continued the same medications. Meanwhile in two patients, we recommended a consultation for psychosomatic medicine. In contrast, the remaining six R-CSA patients exhibited higher levels of vasospasticity, irrespective of the administration of aggressive medical therapy, in which the doses of vasoactive drugs were increased in order to suppress coronary artery spasms.
Conclusion In some R-CSA patients on emergency admission, performing spasm provocation tests under medical therapy is useful for determining the subsequent treatment strategy. Therefore, this test may become a new tool in the treatment of R-CSA.

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Objective The treatment for asymptomatic primary hyperparathyroidism (PHPT) remains controversial. In 2008, the Third International Workshop on the Management of Asymptomatic PHPT proposed a set of guidelines for the management of asymptomatic PHPT. We therefore evaluated the application of the Workshop recommendations in Japanese patients with asymptomatic PHPT.
Methods We analyzed each parameter of the guidelines in 87 Japanese patients with asymptomatic PHPT who underwent methoxy-isobutyl-isonitrile (MIBI) scintigraphy.
Results Fewer than 10% of the patients were less than 50 years of age. The bone mineral density T-score was below -2.5 SD in 20 women (46.5% of all women analyzed) and in none of the men. The eGFR was <60 mL/min/1.73 m² in 17 patients. A majority of patients (43) satisfied only one parameter, while 25 patients satisfied none of the parameters. Furthermore, the MIBI-positive and surgically treated patients tended to satisfy a larger number of the parameters. The Workshop criteria and levels of PTH, daily Ca excretion, serum ALP and 1,25(OH)2 Vitamin D were further analyzed in relation to the findings of MIBI scans, and almost all of the parameters were found to be significantly correlated with the results of the MIBI studies.
Conclusion Our results suggest the need to reassess the Workshop guidelines for the treatment of hyperparathyroidism in Japanese patients. It is desirable that MIBI scintigraphy be performed whenever possible, as this modality is anticipated to play an important role in determining whether or not surgery is appropriate.

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Toxic megacolon is an infrequent but life-threatening complication that occurs most commonly in patients with severe ulcerative colitis. Intravenous steroids are often recommended for patients with toxic megacolon secondary to ulcerative colitis. However, steroid dependency may mask the presence of intra-abdominal sepsis and is associated with refractoriness, during which cytomegalovirus reactivation may occur. In this report, we present two rare cases of megacolon accompanying pancolonic severe ulcerative colitis that were successfully treated with oral tacrolimus, including one steroid-naïve patient. In cases of ulcerative colitis with megacolon, treatment with oral tacrolimus is recommended, thereby avoiding steroid dependency and improving the long-term prognosis.

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Reactivation of hepatitis B virus (HBV) has recently been reported as a fatal complication in patients undergoing cytotoxic chemotherapy. We herein describe a case of reactivation in a 76-year-old man who had undergone pelvic exenteration for colorectal cancer (CRC). He was treated with a modified FOLFOX6 chemotherapy regimen after the operation. Thirteen months later, his laboratory data showed severe liver dysfunction. His hepatitis B surface antigen (HBsAg) test was positive, and his HBV-DNA level was elevated. We diagnosed the patient with HBV reactivation as his HBsAg test was negative before starting chemotherapy. His liver dysfunction improved after administration of entecavir. This is the first report describing HBV reactivation following chemotherapy for an HBsAg-negative CRC patient.

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We herein report three cases of alcoholic cirrhosis complicated by deep bleeding. In two of the three cases, intramuscular or retroperitoneal hematomas developed spontaneously. In contrast, in the remaining case, an intramuscular hematoma developed after trauma. In the former two patients, the intramuscular hematomas recurred at other sites during hospitalization. All three patients received conservative therapy, and one patient with a retroperitoneal hematoma underwent transcatheter arterial embolization. All of the patients eventually died of liver failure. The occurrence of severe alcoholic liver disease with deep bleeding has recently been reported with increasing frequency, and clinicians should bear this condition in mind as a life-threatening complication of alcoholic liver disease.

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A 53-year-old woman with systemic lupus erythematosus and antiphospholipid syndrome presented with central nervous system (CNS) lupus and vegetation of the mitral and aortic valves. Her CNS lupus was relieved with methylprednisolone pulse therapy; however, her mitral regurgitation worsened, and she developed acute decompensated heart failure. The mitral and aortic valves were replaced with mechanical heart valves. Microscopic examination of the excised valves showed no bacterial invasion, and Libman-Sacks (LS) endocarditis of both valves was confirmed. This was a case of LS endocarditis with clear vegetation that spread over the mitral and aortic valves.

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A 41-year-old man was admitted with acute heart failure and shock status. An echocardiogram showed a mobile tumor obstructing blood flow through the mitral valve with a stalk on the posterior left atrium (LA) wall. Emergent open-heart surgery was performed to resect the tumor (77×36×30 mm). Histological examination confirmed that it was an undifferentiated pleomorphic sarcoma. Unfortunately, positron emission tomography performed five months after surgery demonstrated a local recurrence around the right pulmonary vein and LA. We performed proton beam radiotherapy to treat the local recurrence, and it was effective. The patient has survived more than one year since the first life-threatening heart failure episode.

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Osteoporosis and bone fractures are commonly seen in patients with Cushing's syndrome (CS). Fractures usually occur in the vertebrae and ribs whereas pubic fractures are less common. Similar to obvious hypercortisolemia, subclinical hypercortisolemia can increase the risk of fractures. However, in subclinical cases, bone fractures are very rarely seen as the presenting symptom. We herein report the case of a 62-year-old postmenopausal woman who was presented with a pubic fracture. During the evaluation of the fracture, thoracoabdominal magnetic resonance imaging of the patient demonstrated an adrenal mass. Although the patient did not show any signs of overt hypercortisolism, an endocrinologic evaluation revealed hypercortisolism due to an adrenal tumor. Adrenalectomy was performed, which resulted in a cure of the disease. During the orthopedic follow-up, the patient's pubic area pain gradually improved, and the pubic fracture healed without any accompanying new bone fractures. One year after the surgery, a remarkable improvement was detected in the patient's bone density in spite of the lack of administration of any medications for osteoporosis. Subclinical CS can present as a pubic fracture, and awareness of this relationship can help physicians to diagnose the disease.

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It is well known that neurofibromatosis type 1 (NF1) is uncommonly associated with pheochromocytoma development and also, to a larger extent, with gastrointestinal stromal tumors (GISTs). We herein document three cases with the rare condition of NF1 coexisting with GIST and pheochromocytoma, while one of them also has a composite tumor and another has papillary thyroid carcinoma.

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Hypothalamic hyperphagia and obesity are characterized by a lack of satiety and an abnormally high appetite that is difficult to control. We herein report the cases of two patients with hypothalamic hyperphagia and obesity with MRI-detectable hypothalamic lesions. These patients suffered from diabetes mellitus associated with an abnormal eating behavior and weight gain. Liraglutide was successfully used to treat their diabetes mellitus and suppress their abnormal appetites. Glucagon-like peptide-1 analogues, including liraglutide, are promising treatment options in patients with hypothalamic hyperphagia and obesity, as these agents enhance the hypothalamic input of the satiety signal, which is lacking in such patients.

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We herein present a rare case of acquired methemoglobinemia associated with alkaptonuria. Alkaptonuria is a congenital error of metabolism caused by the deficiency of homogentisic acid oxidase, which subsequently results in the accumulation of homogentisic acid (HGA) in body tissues. As renal dysfunction progresses, the level of HGA excretion in the urine decreases and the blood concentration of HGA increases. HGA oxidizes oxyhemoglobin to methemoglobin, which can induce multiple organ failure accompanied by tissue hypoxia, intravascular hemolysis and metabolic acidosis. The mortality of this disease is high when alkaptonuria is associated with the presence of methemoglobinemia; therefore, treatment should be carefully planned in such cases.

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Collagenofibrotic glomerulopathy is a rare glomerular disease characterized by the massive deposition of type III collagen in mesangial and subendothelial spaces. We observed markedly increased serum hyaluronan levels in patients with collagenofibrotic glomerulopathy; levels in three patients were more than 1,000-times greater than the normal upper limit. However, one kidney transplant patient had normal serum hyaluronan levels. We found that serum levels and activities of the enzyme hyaluronidase were normal, and hyaluronan was not markedly deposited in the mesangial or subendothelial spaces. Our findings suggest that serum hyaluronan levels may be a specific diagnostic marker of collagenofibrotic glomerulopathy, and kidney transplantation may alleviate marked increases in serum hyaluronan.

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A 61-year-old man presented with lower extremity paralysis and severe hypokalemia. His thyroid function test showed thyrotoxicosis. Despite attempts to correct his hypokalemia, he developed pulseless polymorphic ventricular tachycardia two hours later. He was successfully resuscitated after defibrillation. We performed continuous venovenous hemodiafiltration for 10 days due to acute kidney injury and rhabdomyolysis. We observed life-threatening polymorphic ventricular tachycardia requiring urgent defibrillation, as well as rhabdomyolysis requiring dialysis during the transient thyrotoxic phase of painless thyroiditis. Pay attention to the possibility of the development of life-threatening ventricular tachycardia associated with hypokalemia in the setting of thyroiditis and thyrotoxic paralysis.

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A 77-year-old woman was admitted because of bilateral hand numbness and dyspnea on exertion. Her serum IgG was increased, and a bone marrow aspiration analysis supported a diagnosis of multiple myeloma. Additionally, computed tomography scans of the chest showed bilateral ground glass attenuations, linear opacities, and consolidations. Transbronchial lung biopsy revealed Congo Red-positive amorphous eosinophilic deposits. She was therefore diagnosed with diffuse parenchymal pulmonary amyloidosis accompanied by multiple myeloma. Following combination chemotherapy including bortezomib, her serum monoclonal protein levels were normalized, and pulmonary function and oxygenation improved.

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Bevacizumab was initially contraindicated in patients with brain metastases, but several reports have shown the efficacy and safety of bevacizumab for brain metastases. We herein report the case of a patient with pulmonary pleomorphic carcinoma for which bevacizumab plus weekly paclitaxel following whole-brain radiotherapy (WBRT) was effectively and safely administered for critical and refractory brain metastases. Although the 50-year-old male patient received WBRT with anti-edema therapies for progressive brain metastases, his clinical symptoms deteriorated rapidly. After the completion of WBRT, we administered bevacizumab plus weekly paclitaxel, and his neurological symptoms improved dramatically. Brain magnetic resonance imaging demonstrated a marked response by the brain metastases and improved brain edema. This case suggested both synergism between WBRT and bevacizumab, and an anti-edema effect of bevacizumab. Bevacizumab may be therefore a potent therapeutic option for patients with refractory brain metastases.

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Esophagomediastinal fistula secondary to mediastinal tuberculosis (TB) lymphadenitis is a rare and unusual complication. A 32-year-old woman visited our clinic because of chest pain. Computed tomography (CT) demonstrated an esophagomediastinal fistula with subcarinal lymphadenopathy and no remarkable parenchymal lung lesions. The esophagomediastinal fistula was confirmed by esophagoscopy; however, the patient's bronchoscopy findings were unremarkable. The endobronchial ultrasound-guided lymph node aspiration did not confirm a diagnosis of TB. Finally, the patient was diagnosed via a lymph node biopsy. A drug-sensitivity test revealed the presence of a multidrug-resistant pathogen. To the best of our knowledge, this is the first case of esophagomediastinal fistula secondary to multidrug-resistant (MDR-) TB mediastinal lymphadenitis.

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We herein describe the case of a 74-year-old man who experienced pulmonary consolidation and chest pain following administration of dabigatran, a novel oral anticoagulant. The consolidation settled spontaneously in another lung area, a condition sometimes referred to as "wandering pneumonia." Although we did not find specific pathological evidence of interstitial lung disease on transbronchial lung biopsy, a lung opacity spontaneously disappeared following discontinuance of dabigatran, and there was no recurrence. There are no other reports of dabigatran-induced lung injury, except alveolar hemorrhage and eosinophilic pneumonia. We should consider that any novel drug could cause various types of pulmonary injuries.

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A 35-year-old man with a six-month history of progressive exertional dyspnea was referred to our institution. He had been diagnosed with Mycobacterium intracellulare pulmonary infection with lung cavitation two years earlier, and was being followed up without any medications. After being referred to our hospital, he underwent computed tomographic pulmonary angiography, which indicated a pulmonary thrombus and lung cavitation. Furthermore, right heart catheterization confirmed pulmonary hypertension, and we made a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). Following successful pulmonary endarterectomy, the patient's symptoms and hemodynamics were significantly improved, with the disappearance of lung cavitation. It is important to suspect CTEPH in patients with unaccountable infectious lung cavities.

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A 63-year-old woman was diagnosed with advanced lung adenocarcinoma complicated by Trousseau's syndrome characterized by non-bacterial thrombotic endocarditis, asymptomatic brain infarction, deep venous thrombosis, and low-grade disseminated intravascular coagulation (DIC). The patient's DIC rapidly became widespread, and multiple micropulmonary embolisms led to severe respiratory failure. She received a blood transfusion and anticoagulant treatment with heparin and recombinant human soluble thrombomodulin, which modestly ameliorated her symptoms, and additional chemotherapy led to tumor shrinkage with concomitant resolution of Trousseau's syndrome. Although there are no established medical approaches for managing Trousseau's syndrome, intensive anticoagulant treatment may be effective for improving the patients' general condition in order for them to be able to undergo subsequent combination chemotherapy.

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We herein describe the case of a 77-year-old woman with acquired factor X deficiency that was likely caused by atypical amyloidosis. The patient developed severe gastrointestinal bleeding as a result of a significant decrease of factor X activity. Neither proteinuria nor diarrhea was observed as an initial manifestation. Although a bone marrow examination revealed direct fast scarlet-positive extracellular deposits, they did not exhibit red-to-green dichroism under polarized light. Immunofluorescence microscopy showed that the fibrillar proteins were positive for CD138 but negative for β₂-microglobulin or amyloid A antibodies. These atypical pathological features of immunoglobulin light chain-amyloidosis in this patient might be related to its unique clinical presentation.

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A 63-year-old woman presented to our hospital with fever, purpura and pain in both legs and died 4 days after admission. Her blood smear and skin biopsy showed cylinder-like bodies (20×120 μm). She was diagnosed to have monoclonal gammopathy (IgG, lambda type). An autopsy revealed cylinder-like bodies in the vasculature of various organs. We noted a proliferation of atypical plasma cells in her bone marrow, suggesting pre-existing myeloma. Crystalglobulinemia is a rare manifestation of hypergammaglobulinemia that can cause multiple embolisms of the small vessels, and this resulted in the patient's fulminant course. The identification of cylinder-like bodies in the peripheral blood may help in reaching a diagnosis in such cases.

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Posterior reversible encephalopathy syndrome (PRES) is a rare and potentially harmful complication in patients receiving targeted chemotherapy. We herein present a case of PRES that occurred after 33 days of high-dose bortezomib treatment and propose both overwhelmed cerebral autoregulation and toxin-mediated blood-brain barrier damage as the underlying mechanisms based on the imaging findings. A favorable recovery was observed two days after achieving stringent hypertension control and withholding bortezomib. Follow-up images obtained two weeks later unveiled a dramatic remission, with faint areas of subcortical hyperintensity in the bilateral parieto-occipital regions. With explicit clinical and imaging features, this case highlights the need for early recognition of PRES during the clinical course of bortezomib therapy.

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We herein report the case of a 22-year-old Caucasian man with known vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, cardiac defects, renal and limb anomalies (VACTERL) association who presented with a headache and vertigo following the sudden and temporary loss of consciousness while attending a concert four days before admission to the hospital. On a physical examination, the following findings were found: a low body height, low-set ears, thoracic scoliosis and a mild holosystolic heart murmur. A neurosonological examination revealed a partial subclavian steal phenomenon. CT angiography of the neck vessels and aortic arch confirmed an anomalous right subclavian artery -known as the lusorian artery. Further studies are warranted in patients with VACTERL in order to identify possible links between the prevalence of an aberrant right subclavian artery (lusorian artery) and possible congenital subclavian steal syndrome or dysphagia lusoria. In addition, duplex ultrasound of the carotid and vertebral arteries may be performed as part of screening examinations in patients with congenital syndromes.

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A 36-year-old man with progressive hearing impairment visited our hospital complaining of a severe headache. A neurological examination revealed bilateral sensorineural hearing impairment, mild ataxia, hyperreflexia and mild cognitive dysfunction. Brain MRI demonstrated hydrocephalus and typical hypointensity rimming the brain surface on T2^*-weighted images. The patient was diagnosed as having superficial siderosis. Spinal MRI disclosed the presence of a lumbar intradural extramedullary mass. The surgically resected tumor was histologically found to be a cavernous angioma. Superficial siderosis is an important cause of hearing loss. With respect to the detection of disorders underlying this pathological condition, MRI examinations, including those of the brain and whole spinal cord, are recommended.

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Adult-onset Still's disease (AOSD) is a multisystemic inflammatory disorder, but pulmonary involvement is rare. We herein describe the case of a woman diagnosed with AOSD; treatment resolved her symptoms, but nine days later she was admitted with pyrexia and a productive cough. A chest X-ray revealed diffuse pulmonary nodules and patchy shadows. A high-resolution chest computed tomography scan confirmed diffuse infiltration in the pulmonary parenchyma, signs of alveolar nodules, distribution along the lobule center, several areas of tree-in-bud patterns, and bilateral pleural effusion. The patient was treated with high doses of corticosteroids, which rapidly reduced the size of her diffuse pulmonary nodules and dramatically improved her pleural effusion.

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Lymphomatoid granulomatosis (LyG) is a rare, B-cell derived, lymphoproliferative disorder that often presents as pulmonary nodular lesions with a histopathology of lymphatic invasion of the vascular wall. The development of LyG may be associated with reactivation of the Epstein-Barr virus under an immunosuppressive state. We herein report a case of Grade 3 LyG that developed during methotrexate therapy for rheumatoid arthritis and regressed following the withdrawal of the drug.

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Elizabethkingia meningoseptica is recovered from urine samples in very unusual cases, although the clinical significance of its detection remains unclear. Four cases of E. meningoseptica bacteriuria were analyzed, and E. meningoseptica was found to be the causative agent of healthcare-associated bacteriuria, including urinary tract infections, asymptomatic bacteriuria and transient bacteriuria, primarily in elderly patients with diabetes. Although E. meningoseptica cultured from urine was found to be resistant to most antimicrobial agents, bacteriuria caused by E. meningoseptica resolved in all four cases. However, instances of death have also been previously reported.

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A brain abscess is a life-threatening infection. There are few reports describing Prevotella bacteremia with middle cerebral artery (MCA) occlusion and brain abscess following dental extraction in the literature. We herein describe a 32-year-old healthy man who experienced headache after tooth extraction. He was not correctly diagnosed until he experienced a stroke and a blood culture revealed Prevotella denticola weeks later. This case and our detailed review of related cases highlight the importance of thorough medical history-taking and clinical evaluations. Brain abscess formation should be considered in previously healthy patients with fever, stroke, and a recent history of tooth extraction.

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