Kounis syndrome is hypersensitivity coronary disorder induced by various types of environmental exposures, drugs, conditions and stents. Allergic, hypersensitivity, anaphylactic and anaphylactoid reactions are associated with this syndrome. The disorder manifests as coronary spasms, acute myocardial infarction and stent thrombosis and affects the cerebral and mesenteric as well as coronary arteries. Importantly, its manifestations are broad and its etiology is continuously increasing. Recently, a variety of unusual etiologies have been reported including Anisakis simplex, scombroid syndrome, the use of Gelofusin or ultrasound contrast agents, kiwifruit, fly bites, and bee stings. Furthermore, losartan and the paradox of corticosteroid allergy have been implicated as possible causes. Although not rare, Kounis syndrome is infrequently diagnosed. Therefore, awareness of its etiology, manifestations and pathophysiology is important for providing the proper diagnosis and treatment and determining prognosis.
Objective Aldosterone plays an important role in the pathogenesis of atherosclerosis; however, the significance of mineralocorticoid receptor blockade for atherosclerosis has not been fully elucidated. In this study, the effect of add-on eplerenone on the degree of arterial stiffness was examined in patients with uncontrolled hypertension. Methods Forty-seven uncontrolled hypertensive patients who had previously been treated with anti-hypertensive drugs were examined retrospectively. Thirty-two patients received add-on therapy consisting of eplerenone (Group E) and 15 patients received add-on therapy with a Calcium channel blocker (CCB) or an increased dose of CCB (Group C) in addition to their baseline medications. Both the systolic blood pressure (SBP) and diastolic blood pressure (DBP) values were significantly decreased at two and 12 months in Group C. In contrast, neither the SBP nor DBP values were significantly changed at two months and eventually decreased at 12 months in Group E. The degree of arterial stiffness, as evaluated according to the cardio-ankle vascular index (CAVI), did not improve at either two or 12 months in Group C, whereas the CAVI values improved as early as at two months and the improvement was sustained at 12 months in Group E. The extent of change in the CAVI was not associated with the level of changes in the SBP or DBP values in Group E. Conclusion Treatment with eplerenone added to the patient's baseline medications improves the degree of arterial stiffness as early as at two months after the beginning of treatment, independent of the blood pressure-lowering actions of these drugs in patients with uncontrolled hypertension.
Objective Insulin degludec (IDeg), a new long-acting basal insulin, and FlexTouch, a new injection device, recently became available in Japan. The efficacy and usefulness of IDeg and FlexTouch, compared with insulin glargine or detemir, were assessed in patients with type 2 diabetes mellitus. Methods We performed an open-label longitudinal trial in 20 patients. After informed consent was obtained, all subjects recorded their self-monitoring data of the blood glucose (BG) level; thereafter, basal insulin was replaced by an IDeg-prefilled FlexTouch with the same dose and duration of time (2 weeks). After using FlexTouch, the patients were provided a device-specific questionnaire. Results The patients were divided into two groups according to the dose of basal insulin (≥10 U and <10 U). Although the mean fasting BG levels were unchanged, the mean BG levels before basal insulin injection and its standard deviation were significantly reduced after switching to IDeg in the patients receiving a higher dose of basal insulin (mean BG before basal insulin injection: 164 to 144 mg/dL, p=0.002; mean standard deviation: 32 to 22, p=0.031); however, this difference was not observed in the patients receiving a lower dose. The patients with a shorter duration of diabetes and a single injection of insulin preferred FlexTouch compared with conventional insulin devices. Conclusion Replacing basal insulin with IDeg is useful for the stable and accurate control of blood glucose levels in type 2 diabetes for those receiving a higher dose of basal insulin. Furthermore, the patients with a shorter duration of diabetes and a single insulin injection preferred FlexTouch.
Objective The aim of this study was to assess the incidence of vitamin D deficiency in Japanese postmenopausal women with type 2 diabetes mellitus. Methods Serum 25-hydroxyvitamin D [25(OH)D], intact-parathyroid hormone (PTH), and various bone markers were measured. The primary outcome was the serum level of 25(OH)D. Patients This study included postmenopausal women with type 2 diabetes mellitus. Results The study patients included 170 women with a mean 25(OH)D of 20.0 ng/mL. With regard to the serum level of 25(OH)D, the patients were defined as normal (≥30 ng/mL, 8.2% of the patients) and abnormal (<30 ng/mL, 91.8% of the patients, vitamin D deficiency). The latter group was subdivided into severe deficiency (<10 ng/mL, 2.9% of the patients), deficiency (10-19 ng/mL, 47.1% of the patients), and insufficiency (20-29 ng/mL, 41.8% of the patients). There was a significant negative correlation between the serum 25(OH)D level with type I collagen cross-linked N-telopeptides (NTX) and intact-PTH, but not between 25(OH)D and the bone quality markers. There was a significant positive correlation between 25(OH)D and the radial bone mineral density, but not between 25(OH)D and the bone mineral density on the lumbar vertebrae and femur. A multivariate analysis identified NTX as the only significant determinant of 25(OH)D. The cutoff value of 25(OH)D was 18.5 ng/mL based on a Receiver Operatorating Characteristic analysis. Conclusion Our results showed an alarmingly high incidence of vitamin D deficiency in Japanese women with type 2 diabetes mellitus, with a risk of radial bone osteoporosis, particularly in those patients with a serum 25(OH)D level of <18.5 ng/mL.
A 16-year-old boy was diagnosed with Crohn's disease. Treatment with oral mesalazine was started at 3 g per day; however, he complained of high fever, a nonproductive cough, and left shoulder pain after 2 weeks. His chest radiography and chest computed tomography showed cardiomegaly and left pleural effusion, while an echocardiogram revealed pericardial effusion. Because no infection was detected by thoracentesis and the drug lymphocyte stimulation tests for mesalazine were positive, the patient was diagnosed with mesalazine-induced pleuropericarditis. After the cessation of mesalazine, the clinical symptoms and laboratory findings quickly improved.
A 47-year-old man was found to have a 3-cm epiphrenic esophageal diverticulum on an upper gastrointestinal (UGI) barium study. He developed the symptoms of heartburn approximately 12 months later. UGI endoscopy indicated non-erosive gastroesophageal reflux disease (NERD) and an epiphrenic esophageal diverticulum. A proton pump inhibitor (PPI) did not relieve the symptoms. An UGI barium study at that time showed that the epiphrenic esophageal diverticulum had enlarged to 7 cm, and esophageal manometry showed findings of achalasia and diffuse esophageal spasm (DES), thus vigorous achalasia was diagnosed. Resection of the epiphrenic esophageal diverticulum, myotomy, and fundoplication (the Heller-Dor procedure) were successfully performed and no postoperative symptoms were encountered.
An 80-year-old woman was admitted to our hospital with right hypochondralgia. Abdominal ultrasonography showed a well-circumscribed round lesion in segments 5 and 6 of the liver. Contrast-enhanced ultrasonography (CEUS) indicated that the tumor was enhanced in the arterial phase, changing to hypo-enhanced 30 seconds following contrast injection. After admission, the patient complained of epigastralgia, and CEUS subsequently demonstrated extravasation from the tumor. Although abdominal angiography was successful for achieving hemostasis, the tumor grew rapidly and the patient died. An autopsy revealed the presence of sarcomatoid hepatocellular carcinoma (HCC). The literature is lacking in details regarding CEUS findings for sarcomatoid HCC and rupture. We herein report a rare case of sarcomatoid HCC.
A 69-year-old woman was hospitalized due to progressive lethargy with hyperammonemia. Five months before the current admission, she was diagnosed with depression based on her low level of daily physical activity and thus began taking antidepressants. Abdominal computed tomography revealed a portosystemic shunt running between the left renal vein and inferior mesenteric vein. Balloon-occluded retrograde transvenous obliteration of the shunt vessel was performed, and the patient showed a remarkable clinical improvement. The possibility of a portosystemic shunt should be considered in the presence or absence of underlying liver disease and the ammonia level should be measured before diagnosing depression, as portosystemic encephalopathy may be reversible with interventional radiology treatment.
Among the etiologies of pyogenic liver abscess (PLA), bacterial spread from the biliary tract or portal flow is the major cause, while the onset of PLA due to arterial bacterial transmission is rare. We herein report two cases of PLA thought to be caused by arterial transmission from dental disease. In both cases, there was benign biliary stricture as a result of alcoholic chronic pancreatitis, although normal oral flora was detected as the causative bacteria and oral hygiene was poor in both patients. We presumed that the origin of PLA was dental disease and successfully treated the patients with percutaneous drainage, antibiotics and dental procedures.
Myocardial calcification is rare. An 88-year-old man who had previously been diagnosed with apical hypertrophic cardiomyopathy exhibited left ventricular asynergy on echocardiography before undergoing cholecystectomy. Computed tomography revealed severe calcification in the apical region of the left ventricular myocardium, although the coronary arteries were intact and the hemodynamics on right heart catheterization were normal. The cause of the left ventricular asynergy appeared to be myocardial calcification, thought to be the result of rheumatic fever based on the patient's past history. Stress echocardiography showed a latent risk for the development of heart failure due to the distensibility of the calcified left ventricular myocardium.
Beta-adrenoreceptor blockers are essential in controlling the peripheral actions of thyroid hormones and a rapid heart rate in patients with thyroid storm, although they should be used with great caution when there is the potential for heart failure. A 67-year-old woman was diagnosed as having thyroid storm in addition to marked tachycardia with atrial fibrillation and heart failure associated with a reduced left ventricular function. The administration of an oral beta blocker, bisoprolol fumarate, induced hypotension and was not tolerable for the patient, whereas landiolol hydrochloride, a short-acting intravenous beta-adrenoreceptor blocker with high cardioselectivity and a short elimination half-life, was useful for controlling the patient's tachycardia and heart failure without causing hemodynamic deterioration.
Diaphenylsulfone (DDS: Dapsone) is used for Pneumocystis pneumonia (PCP) prophylaxis, and methemoglobinemia has rarely been reported as a side effect of DDS. We herein report two cases of DDS-related methemoglobinemia in an 81-year-old man with organizing pneumonia and an 84-year-old woman with eosinophilic pneumonia under treatment with prednisolone. Both patients initially received trimethoprim/sulfamethoxazole for PCP prophylaxis and were switched to DDS due to side effects and subsequently exhibited a clinically unexplainable decrease in SpO2. Methemoglobinemia was diagnosed based on the findings of arterial blood gas analyses. In both cases, the methemoglobinemia improved after discontinuing DDS.
We herein report an extremely rare case of pulmonary Langerhans cell histiocytosis with a solitary enlarged inguinal lymph node. A 19-year-old man presented with a non-productive cough lasting for over a five-month period and an enlarged left inguinal lymph node that had persisted for four months. A histopathological study of the lymph node specimens found Langerhans cells coupled with eosinophils. Positive immunohistochemical staining for langerin, Cluster of Differentiation 1a, S100 in the Langerhans cells confirmed the diagnosis, and a mildly impaired ventilation function in addition to multiple peripheral pulmonary cystic lesions were detected. The patient was managed with prednisone (0.5 mg/kg daily), with slow tapering over several months.
A 36-year-old man with diffuse large B-cell lymphoma presented with polyneuropathy, and the diagnostic work-up revealed the presence of IgM antibodies against gangliosides with disialosyl residues (GD1b, GD3). He was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone and received high-dose intravenous immunoglobulin for the treatment of neuropathy. After initiating the treatments, the patient's neurological impairment improved dramatically. He currently remains in complete remission without a flare-up of the polyneuropathy. Based upon our experience and other case reports of lymphoma with immune-mediated neuropathy caused by anti-disialosyl ganglioside IgM antibodies, we conclude that determining the anti-ganglioside antibody profile and beginning early treatment should be considered promptly for patients with malignant lymphoma who develop polyneuropathy.
A 55-year-old man was diagnosed with remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. Contrast-enhanced computed tomography for cancer screening showed a mass with low-density centers with an enhanced rim in the left iliopsoas muscle. We suspected an iliopsoas abscess and performed computed-tomography-guided puncture of the mass. Both Gram staining and the culture of the fluid were negative. We diagnosed the patient with RS3PE syndrome with iliopsoas bursitis and administered low-dose corticosteroids without antibiotics. The symptoms, including left hip pain, quickly disappeared following treatment. Clinicians should be aware that iliopsoas bursitis may resemble an iliopsoas abscess. As a result, it is important to make an accurate differential diagnosis.
A 16-year-old boy stayed in Tokyo near Yoyogi Park for extracurricular high school activities. After returning home, he experienced an episode of fever and visited our emergency outpatient unit. He initially exhibited symptoms of leukopenia, thrombocytopenia and concomitant rhabdomyolysis and after admission simultaneously developed a biphasic fever and systemic erythema. Based on the results of reverse transcription polymerase chain reaction testing, he was finally diagnosed with dengue fever. After an absence of 70 years, dengue fever has reemerged as a domestic infection. Awareness of this trend led to our diagnosis.
The absence of meningeal signs and symptoms is rare in patients with bacterial meningitis and may lead to a delay in diagnosis and treatment. Furthermore, the onset of bacterial meningitis associated with pneumocephalus is a rare complication of ear infections. We herein report a rare case of otogenic meningitis complicated by pneumocephalus that was initially missed due to the absence of typical meningeal signs and symptoms and later diagnosed correctly based on a thorough review of the patient's systems.