Objective Coronary spasm as well as atherosclerosis plays an important role in the pathogenesis of coronary heart disease. However, the relationship between coronary spasm and atherosclerosis is not well known. The purpose of the present study was to examine the differences and interactions between risk factors for coronary spasm and atherosclerosis and thereby explore the pathogenesis of coronary spasm. Methods The study subjects consisted of 938 patients with chest discomfort (522 men and 416 women, mean age 65.2±11.0) who underwent intracoronary-acetylcholine provocation tests for coronary spasm. Coronary risk factors, including age, gender, body mass index, blood pressure, high-sensitivity C-reactive protein (hsCRP), white blood cells, glucose, lipid profiles, and other laboratory chemistries were examined. Results Four hundred and ninety-six patients (315 men and 181 women, mean age: 65.1±11.4) were diagnosed with coronary spastic angina (CSA), while the remaining 442 patients (207 men and 235 women, mean age: 65.3±10.7) were diagnosed with non-CSA. A multiple logistic regression analysis revealed men, smoking, hsCRP, and low diastolic blood pressure (DBP) to be predictors (p=0.001, p=0.009, p=0.034, and p=0.041, respectively) for CSA, while age, diabetes mellitus, low high-density lipoprotein-cholesterol, systolic blood pressure (SBP), uric acid and male gender were found to be predictors (p<0.001, p<0.001, p<0.001, p=0.002, p=0.006 and p=0.029, respectively) for atherosclerosis. Conclusion Predictors for coronary spasm were smoking, hsCRP and low DBP, whereas those for atherosclerosis were age, diabetes mellitus, high SBP, and uric acid in that order. These findings suggest that the pathogenesis of coronary spasm differs from that of atherosclerosis.
A 78-year-old Japanese woman presented with anemia. Oral double-balloon endoscopy (DBE) detected multiple ulcerative lesions covered with coagula extending up to approximately 20 cm from the mid-jejunum. Based on the histopathological findings, the patient's condition was diagnosed as mucosa-associated lymphoid tissue (MALT) lymphoma of the small intestine. During the second DBE examination, a small intestinal perforation occurred in one of the ulcerative lesions, and an emergency segmental small intestinal resection was performed. The present case suggests that in MALT lymphoma, intestinal wall fragility may lead to perforation even though the lesion may appear to be a superficial ulcer on endoscopy.
In December 2011, an 84-year-old Japanese man was admitted with severe cholecystitis. We treated the cholecystitis using antibiotics, and his symptoms improved. In March 2012, he was readmitted after experiencing a sudden bloating sensation and vomiting. We determined that a gastric wall abscess had protruded into the antrum. The gastric wall was adjacent to the gallbladder, and the gastric abscess was related to a known case of cholecystitis. Upper gastrointestinal endoscopy indicated that the abscess was under the mucous membrane of the gastric antrum. Pus, exuding from the abscess, was aspirated with an endoscope.
A 65-year-old man presented with upper abdominal pain and was suspected of having choledocholithiasis. Endoscopic extraction of the stone was performed; however, a round filling defect in the common bile duct (CBD) persisted. Subsequent cholangioscopy showed the presence of a papillary tumor in the CBD, and the lesion was removed via the Whipple procedure. Microscopically, the papillary lesion was composed of pancreatic tissue in the submucosal layer of the CBD and therefore diagnosed as reflective of heterotopic pancreas. This is only the seventh case of heterotopic pancreas in the CBD reported in the English literature.
A 57-year-old man was referred and admitted to our hospital for treatment of a symptomatic pancreatic mass. Pancreatic arteriovenous malformation (AVM) was diagnosed based on the findings of contrast-enhanced computed tomography (CT) and angiography, and transcatheter arterial embolization (TAE) with N-butyl-2-cyanoacrylate (NBCA) was performed without complications. The patient's symptoms subsequently improved after TAE, and resolution of the pancreatic AVM was detected on contrast-enhanced CT performed six months after the embolization procedure. This case indicates that TAE with NBCA is a safe and effective treatment for pancreatic AVM.
A Japanese woman was treated with injectable methylprednisolone and oral prednisolone for dermatomyositis. On admission, her serum was positive for anti-hepatitis C virus (HCV) antibodies, although HCV RNA was undetectable on polymerase chain reaction. Glucocorticoid therapy improved the dermatomyositis; however, the serum alanine aminotransferase levels rapidly increased, with positive serum HCV RNA and a high viral titer. Both parameters decreased in association with prednisolone tapering, whereas dermatomyositis subsequently recurred and the administration of glucocorticoid therapy was repeated. The serum alanine aminotransferase and HCV RNA levels subsequently increased in a similar manner to that observed after the first course of therapy. Liver enzymes and the viral load should be monitored in anti-HCV-positive patients receiving immunosuppressives, even if serum HCV RNA is negative.
Home parenteral nutrition (HPN) is a well-established intervention to sustain life in malnourished patients at home. Because it is difficult for patients with anorexia nervosa (AN) to gain weight or stop purging, such patients require repeated hospitalizations. Although HPN has not been commonly used for AN patients in Japan, we utilized this approach to treat seven AN patients. We herein present the clinical course and outcome of these seven patients, the application criteria for HPN in our institution, and the potential problems associated with HPN. Despite its complications, HPN may be a useful measure to help patients with persistent AN avoid multiple hospitalizations.
Immunoglobulin (Ig)-G4-related disease is a multi-organ disease that may affect the lung. We herein describe a patient with IgG4-related lung disease (IgG4-RLD) who was radiologically and pathologically diagnosed with organizing pneumonia. He was successfully treated with a combination of prednisolone (PSL) and azathioprine (AZA), and his clinical course has been uneventful since tapering off PSL. This is a rare case of IgG4-RLD manifesting as organizing pneumonia, and, to our knowledge, this is also the first case showing the effectiveness of AZA in treating IgG4-RLD.
Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder, the most common complication of which influencing the prognosis is pulmonary fibrosis. In the present report, we describe an autopsy case of a Japanese woman with HPS. The patient was diagnosed at 50 years of age based on the presence of oculocutaneous albinism, hemorrhagic diathesis, ceroid-lipofuscin accumulation and pulmonary fibrosis. Although systemic steroids, immunosuppressants and pirfenidone were administered for pulmonary involvement, she died from respiratory failure two years later. Obtaining an early diagnosis and taking into consideration the need for lung transplantation is necessary in order to improve the prognosis of HPS. We herein report this very rare Japanese case of HPS with a review of the treatment approaches for HPS complicated with pulmonary fibrosis.
A 55-year-old woman was admitted for an evaluation of a mediastinal mass, bilateral cervical lymphadenopathy and a left breast tumor. Although pathology revealed a diagnosis of breast cancer, the cervical lymph nodes differed from the breast lesion. An anaplastic lymphoma kinase (ALK) gene analysis revealed ALK rearrangement in the cervical lymph nodes only, which were therefore diagnosed as reflective of metastasis of lung adenocarcinoma. The mediastinal tumor was also diagnosed as an ALK-positive lung adenocarcinoma based on its therapeutic response. ALK gene analyses can be used to identify primary lesions in patients with cancers of unknown primary sites.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy arising from plasmacytoid dendritic cell precursors. BPDCN typically manifests in the skin, but it can also evolve into a leukemic form or be complicated by acute myeloid leukemia, some cases with a preceding myelodysplastic syndrome (MDS). We herein report the first case of complete spontaneous regression of cutaneous BPDCN followed by acute monocytic leukemia evolving from MDS. This is also the first reported case of gastric BPDCN invasion.
A 45-year-old man who had undergone splenectomy 20 years earlier for immune thrombocytopenia (ITP) presented with a fever, arthralgia and vesicular skin rash. The skin rash was typical for varicella, as confirmed on serological studies. He exhibited isolated thrombocytopenia and was diagnosed with ITP. In addition, an accessory spleen was detected. The platelet count responded to treatment with prednisolone (PSL), and the varicella subsided uneventfully following therapy with acyclovir. Furthermore, the platelet count was maintained after PSL was discontinued. This case suggests an etiological link between varicella and very late relapse of ITP after initial splenectomy.
We herein describe the case of a Japanese cerebrotendinous xanthomatosis (CTX) patient with a novel CYP27A1 gene mutation. The patient had been diagnosed with cataracts at 25 years of age and subsequently developed neurological symptoms in his forties, being referred to our hospital at 47 years of age. Upon admission, Achilles tendon xanthomas, cognitive impairment, dysphagia, dysarthria, dystonia, spasticity, muscle weakness and ataxia were observed. Brain MRI revealed abnormal signals in the dentate nuclei, periventricular white matter and pyramidal tract, and the serum cholestanol level was elevated. A CYP27A1 gene analysis identified compound heterozygosity for p.A335V, a novel mutation, and p.R405Q, a previously reported mutation. Making an early diagnosis of CTX is crucial, as the administration of chenodeoxycholic acid reverses metabolic derangement.
A 58-year-old man presented with right chest pain, anorexia, general malaise, and fever. Chest computed tomography showed a mass lesion with right middle lobe atelectasis. The bronchoscopy showed polypoid lesions with a smooth surface in each right middle lobe bronchial lumen. The histopathology revealed the dense accumulation of histiocyte-like cells with foamy cytoplasm under the bronchial epithelium along with yeast-like fungi stained positively with both Alcian blue and Grocott's stains. Cryptococcus neoformans was cultured from the bronchial washings. We diagnosed the patient with pulmonary cryptococcosis with endobronchial lesions. The fluconazole treatment was changed to liposomal amphotericin-B and flucytosine after the diagnosis of cryptococcal meningitis. The minimum inhibitory concentration of the fungi suggested resistance to fluconazole and flucytosine. The lesion regressed after these treatments.
We herein present the case of a 64-year-old immunocompetent man with a diagnosis of pulmonary cryptococcosis who presented with cavitary nodules, one of which contained a fungus ball, on chest CT. The coincidence of cavitary cryptococcosis and an Aspergillus fungus ball was histologically confirmed on a thoracoscopic lung biopsy. Encapsulated round-to-oval yeasts (Cryptococcus) were observed throughout the entire specimen, including the cavity, cavity wall and lung parenchyma. In contrast, filamentous fungi (Aspergillus) were noted within the cavity only. The probable mechanism of this rare manifestation is that the Cryptococcus formed cavities, after which an Aspergillus fungus ball developed within one cavity.
We herein report a rare case of intraspinal ectopic endometrial tissue associated with tethered cord syndrome. The patient underwent MR imaging of the lumbar spine and CT spinal angiography. Asymptomatic dysraphism was also detected, including spinal bifida, low-lying conus medullaris, spinal meningocele and a lumbosacral lipoma. Venous reflux obstruction was also suggested. The patient underwent L2-S1 laminectomy and microdecompression of the lesion. The histological and immunohistochemical features were characteristic of ectopic endometrial tissue. Since the surgery, no neurological signs have been noted, either during or outside the patient's menstrual periods. The two-month follow-up MRI scans showed a regression of the lesion.
Caffeine is an easily available drug which is generally regarded as being safe. However, it can lead to a lethal outcome in severe cases of caffeine intoxication. We herein report a case of a 32-year-old man who ingested approximately 15.6 g of caffeine in a suicide attempt. He suffered from sustained ventricular tachycardia despite conservative treatment. Therefore, we performed a combined therapy of hemoperfusion and hemodialysis which resulted in rapid improvement of the patient's ventricular tachycardia as well as a reduction of the patient's plasma caffeine level. To the best of our knowledge, this combination has not been previously reported to treat caffeine intoxication.