Objective We aimed to investigate the association between the ABO blood groups and the risk of coronary artery disease (CAD) and myocardial infartion (MI) in a young Taiwanese population. Methods We retrospectively recruited 277 consecutive subjects (men younger than 45 years and women younger than 55 years) who underwent coronary angiography (136 with documented CAD and 129 without CAD) at our center, between 2005 and 2008. Their ABO blood groups were determined using standard agglutination techniques. Results Patients with CAD showed a significantly different blood group distribution (O, 30.1%; A, 39.7%; B, 26.5%; AB, 3.7%) than that shown by the controls (O, 42.6%; A, 24.0%; B, 27.1%; AB, 6.2%; p=0.032). Patients with blood group A had a greater risk of CAD and MI than those with non-A blood groups (OR=2.08, 95% CI=1.23-3.54; OR=2.21, 95% CI=1.19-4.09, respectively). After adjustment for common cardiovascular risk factors such as age, gender, hypertension, cigarette smoking, diabetes mellitus, body mass index, family history of CAD, and lipid profiles; blood group A remained significantly associated with an increased risk of CAD and MI (OR=2.61, 95% CI 1.11-6.14, p=0.028; OR=3.53, 95% CI=1.21-10.29, p=0.021, respectively). Conclusion Our findings suggest that blood group A is an independent risk factor for CAD and MI in young people in Taiwan.
Objective The aim of this study was to establish a causal relationship between excess body weight and the onset of diabetes in a retrospective cohort study. Methods This 10-year observational cohort study investigated 969 men and 585 women (23 to 80 years of age), who underwent voluntary complete medical check-ups and an annual 75-g oral glucose tolerance test (75 g-OGTT). Participants with fasting plasma glucose ≥126 mg/dL, 2-h glucose level in a 75 g-OGTT ≥200 mg/dL and/or received medical treatment for type 2 diabetes during the previous year were considered as new-onset diabetics. We assessed the independent contribution of increased BMI to the risk of developing type 2 diabetes with Cox proportional hazard model. Result During the follow-up period, we diagnosed 86 men and 49 women with new-onset type 2 diabetes. In the Cox proportional hazards model, the risk of diabetes mellitus increased with increasing BMI, even after adjusting for age, sex, blood pressure, metabolic profiles, and insulin resistance. In the final model, setting BMI less than 25 as a reference group, the Hazard ratios for diabetes mellitus was 3.12 for those with a BMI of 25-27.4 and it was increased to 3.80 for participants with a BMI of 27.5 or higher. Conclusion Overweight/obesity (high BMI) is an independent and dose-dependent risk factor for type 2 diabetes in overweight Japanese patients. Our results confirmed the usefulness of BMI as a classic parameter, and the importance of lifestyle modification and better management among people with overweight/obesity for prevention of type 2 diabetes mellitus.
Objective We investigated the present state of, and trends in, hemodialysis therapy in Wakayama, with the aim of identifying present and future problems. Methods We compared the number of patients on maintenance hemodialysis, patients newly commencing hemodialysis each year, and proportion of diseases prompting the initiation of hemodialysis, between Wakayama and all Japan from 2002 to 2009, using the CD-ROM, "An overview of dialysis treatment in Japan," published by the Japanese Society for Dialysis Therapy. Results The number of patients on maintenance hemodialysis per head of population was higher in Wakayama than in all Japan throughout the study period. The number of patients newly commencing hemodialysis per head of population was higher in Wakayama than in all Japan from 2002 to 2004, but no significant difference was seen after 2005. The proportion of patients with chronic glomerulonephritis as the causative disease for hemodialysis initiation was higher in Wakayama than in all Japan. However, nephrosclerosis was less common as the causative condition in Wakayama than in all Japan. The proportions of the different causative diseases were similar in all patients on maintenance hemodialysis in Wakayama as in the newly initiated patients. Accordingly, some patients diagnosed with chronic glomerulonephritis might actually have nephrosclerosis, or treatment may be inadequate. Conclusion In order to reduce the number of patients requiring maintenance hemodailysis, it is important to accurately differentiate between chronic glomerulonephritis and nephrosclerosis, and also to treat patients with either disease appropriately.
Objective We conducted a retrospective study assessing the relationship between comorbidity, using the Charlson Comorbidity Index (CCI), and the prognoses of acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) patients. Methods We analyzed the data of 47 patients with ALI and ARDS who were admitted to our center between April 2004 and July 2009. The patients were classified into 2 groups (survival and non-survival) 3 months after diagnosis, and demographic and clinical characteristics were analyzed. We also evaluated the ROC curve and Akaike's information criterion (AIC) to determine the most appropriate cut-off level for the CCI at 3 months survival. The survival rate was estimated based on the AIC results. Results The mean age was 71.0 years; 25 (53%) of the patients died within 3 months of the diagnosis. Although age, etiology of ALI and ARDS, and APACHE II score did not differ between the two groups, smoking history, CCI, SOFA score, and steroid use were higher in the non-survival group than in the survival group. Age was not significantly correlated with CCI; however, CCI had weak, but statistically significant correlations with the APACHE II and SOFA scores (r=0.387, p<0.01 and r=0.288, p<0.05, respectively). AIC analysis revealed that a score of 4 on the CCI was the most appropriate cut off level for 3 months survival. The 3-month survival rate was lower in patients with a CCI≥4 than in those with a CCI<4 (9.5% vs. 55.5%, p<0.05). Discussion This study showed that the prognosis of ALI and ARDS was affected more by comorbidity than by age, and that the CCI was useful for assessing patient comorbidities in ALI and ARDS. We have to consider that patients with a CCI score of 4 or more are at risk of developing multi-organ failure and have a poor prognosis.
Patients undergoing continuous ambulatory peritoneal dialysis (CAPD) are at an increased risk of peritonitis following colonoscopy with or without polypectomy. Guidelines for peritoneal dialysis patients recommend administration of prophylactic antibiotics and drainage of the abdomen before colonoscopy. In this report, we describe a 53-year-old woman on CAPD who underwent colonoscopy with polypectomy and developed peritonitis within 24 hours. She presented with severe abdominal pain, typical rebounding tenderness, and turbid dialysate containing increased white blood cells with a predominance of neutrophils. A culture of the patient's peritoneal fluid grew polymicrobial species including Escherichia coli, Klebsiella pneumoniae, and Enterococcus faecalis. She was treated with intraperitoneal and intravenous administration of combination antibiotics, and she fully recovered within 3 weeks. We suggest that nephrologists and endoscopists should be familiar with the risks and follow the guidelines to prevent such complications in CAPD patients. If peritonitis occurs, medical therapy with antibiotics should be considered before surgical intervention for catheter salvage.
A 54-year-old man was referred to a local hospital, located about 90 km from our hospital, with cardiogenic shock due to left main coronary artery infarction (LMCA-MI). Percutaneous coronary intervention (PCI) was performed under intra-aortic balloon pumping (IABP) support, but resulted in insufficient reperfusion and his condition worsened. The helicopter emergency medical service (HEMS) rapidly transported the patient to our hospital. After percutaneous cardio-pulmonary support system (PCPS) insertion, PCI could establish the coronary flow. A series of intensive therapies saved the patient. The cooperation of medical and emergency service system following revascularization and intensive care saved the patient with LMCA-MI accompanied by cardiogenic shock.
This report describes an obese 39-year-old man who experienced ST-segment elevation myocardial infarction with total thrombotic occlusion of the right coronary artery. Culprit vessel flow was improved by aspiration. Data suggested that myocardial infarction had resulted from paradoxical embolus via a patent foramen ovale triggered by the Mueller maneuver, which had induced negative intrathoracic pressure following an acute increase of right-heart volume in the context of obesity and sleep-disordered breathing (SDB). Obesity is increasing among younger populations and it represents a risk for SDB and thrombosis. Thus, this mechanism should be included within the differential diagnosis for myocardial infarction in young patients.
An 8-year-old boy suffered from an unconsciousness attack and torsade de pointes arrhythmia during sleep or at rest. His electrocardiogram showed prolonged QT intervals, but the T wave morphology was atypical for type 1, 2 or 3 congenital long-QT syndrome (LQTS). Intravenous epinephrine slightly prolonged the QT interval whereas mexiletine infusion shortened the QT interval. Although these clinical characteristics might suggest type 3 LQTS, a genetic analysis identified the G604S-KCNH2 mutation (type 2 LQTS). Because mismatches between the genotype and phenotype of LQTS are possible, genetic analysis of LQTS is important to identify the most appropriate therapeutic option and risk stratification.
We encountered a 39-year-old man with documented ventricular fibrillation (VF). His ECGs showed intermittent Wolff-Parkinson-White (WPW) syndrome pattern. During electrophysiological study, no ventricular preexcitation was observed. An accessory pathway located at the posterior mitral annulus was identified, and successfully eliminated by radiofrequency catheter ablation. VF was not induced. His ECGs in the absence of delta waves demonstrated early repolarization in the inferior leads. This case raises the possibility that patients with manifest WPW syndrome may have an arrhythmogenic substrate associated with early repolarization, and the characteristic J waves can be masked by the presence of ventricular preexcitation.
Intracardiac thrombosis is an exceptional complication of Behçet's disease. The management of this involvement is difficult due to the risk of recurrence. We present the case of a young man admitted to our hospital for intermittent fever. The microbiologic investigations did not show any causative germ. We discovered a right ventricle thrombus on echocardiography. We confirmed the diagnosis of pulmonary embolism on CT angiogram. The patient developed oral and genital ulcerations which were consistent with Behçet's syndrome. The thrombus had disappeared after treatment with anticoagulant, corticosteroid and immunosuppressors. Intracardiac thrombosis can reveal Behçet's disease. An exhaustive examination and close monitoring should be performed in order to reveal pathognomonic signs as soon as possible and to promptly start the appropriate treatment.
We herein report a case of severe postsurgical hypocalcemia associated with primary hyperparathyroidism (pHPT), Graves' disease (GD) and acromegaly (AC). A 54-year-old woman was referred to our clinic for treatment of pHPT and GD. She also had active AC and was clinically diagnosed as multiple endocrine neoplasm type 1 because of pHPT and AC. Two enlarged parathyroid glands were detected by preoperative examinations. We performed total parathyroidectomy and thyroidectomy. After the operation, she showed severe hypocalcemia induced by postsurgical hypoparathyroidism and hungry bone syndrome. This is a rare case of postsurgical severe hypocalcemia associated with pHPT, GD and AC.
A 57-year-old woman who had been treated with interferon β-1a (IFNβ-1a) for multiple sclerosis was diagnosed with diabetic ketosis. Her fasting serum C-peptide (F-CPR) was 1.9 ng/mL and her daily urinary C-peptide (U-CPR) was 24.1 μg/day. Her anti-glutamic acid decarboxylase (GAD) antibody was 3.5 U/mL. Seven months later, she was hospitalized with body weight loss and a high level of hemoglobin A1c [11.1% (JDS)]. Her F-CPR and U-CPR were very low (0.1 ng/mL and 8.35 μg/day, respectively), and anti-GAD antibody became distinctly positive (12.4 U/mL). She had HLA-DRB1*04:05, A24, and B54. For these reasons, IFNβ-1a administration was considered a possible cause of type 1 diabetes mellitus in this case.
Hereditary xanthinuria is an extremely rare purine metabolism disorder caused by a genetic abnormality in xanthine dehydrogenase. A new case of hereditary xanthinuria type 1 accompanied by bilateral renal calculi was encountered. We performed an allopurinol loading test and diagnosed classical type 1 xanthinuria. Through genetic diagnosis, we identified a mutation site in the xanthine dehydrogenase gene. Genetic analysis revealed a homozygous deletion of cytosine 2,567 in the xanthine dehydrogenase gene, and as a result, a stop codon was formed at position 928. Renal failure caused by the deposition of xanthine crystals is a known complication because xanthine is poorly soluble in water. With high fluid intake and low purine diet, no significant increase in calculi has been observed in this patient for 2 years.
A large number of renal biopsy studies have shown the concurrent presence of non-diabetic renal disease in diabetics. This report describes one such diabetic female patient with nephrotic syndrome due to minimal change glomerular disease who was successfully treated with prednisolone. Despite the remission of her nephrotic syndrome, she had gradual development of malignant ascites, which was finally interpreted to be linked to primary peritoneal carcinoma. It is necessary to bear in mind that malignancies may not only be the underlying etiology for paraneoplastic glomerular injuries, but also can be an independent pathogenic process, regardless of their nephrotic status during the overall management of the patients with ascites.
Azathioprine hypersensitivity is a clinical syndrome which may manifest from isolated fever and rash to multi-organ failure. This rare condition is usually self-limiting following the discontinuation of azathioprine. Therefore, it is important to maintain a high index of clinical suspicion for hypersensitivity reactions with azathioprine therapy. We report a case of azathioprine hypersensitivity in a 69-year-old woman who developed cardiogenic shock and Sweet's syndrome following the initiation of azathioprine for her underlying autoantibodies to neutrophil cytoplasmic antigens (ANCA) associated microscopic polyangiitis.
Sarcoidosis is a systemic granulomatous disease of unknown etiology with characteristic pulmonary lesions, which are often distributed in the upper lung fields. We describe a unique case of sarcoidosis with lower lung field-dominant reticular shadows. Three years after the diagnosis of sarcoidosis based on histologic findings of the mediastinal lymph nodes and transbronchial lung biopsy specimens, the patient developed acute respiratory failure and died. The autopsy showed usual interstitial pneumonia (UIP), with honeycombing and superimposed diffuse alveolar damage of the lungs. The findings suggest that the patient had both sarcoidosis and UIP, and that the UIP later progressed to acute exacerbation.
Anticoagulant therapy is the mainstay in the management of venous thromboembolism. Nevertheless, the situation is entirely different in the patients with submassive or massive pulmonary embolism (PE) and cardiac arrest, and the diagnosis and therapy strategy for such conditions are lacking. This patient, who presented with a cardiac arrest event after varicose vein surgery, was diagnosed as acute pulmonary embolism. She survived after administration of 50 mg recombinant tissue plasminogen activator (rt-PA) for over half an hour, along with continued anticoagulant therapy. Unfortunately, gastrointestinal and cerebral hemorrhaging occurred during the process.
Riluzole has recently been proven as the first effective drug for the treatment of amyotrophic lateral sclerosis (ALS). We report two rare cases of lung injury caused by riluzole therapy in patients with ALS. Chest radiographs showed bilateral lower lobe, dorsal-dominant ground glass opacity, and/or consolidation. A drug lymphocyte stimulation test (DLST) of peripheral blood or bronchoalveolar lavage cells was positive for riluzole. Histopathological examination of lung biopsy specimens revealed lung injury without fungoid granuloma, vasculitis, or diffuse alveolar damage. To the best of our knowledge, this is the first report of riluzole-induced lung injury with positive DLST results.
Non-small cell lung cancer was metastasized at the septal side of right atrium in 59-year-old woman who had undergone surgery for lung cancer 11 years ago. The cardiac metastasis was found by whole-body 18F-fluoro-deoxy-glucose positron emission tomography/computed tomography (FDG-PET/CT), and cytologically confirmed by myocardial aspiration biopsy with right heart catheterization. The patient was treated with 4 cycles of carboplatin/pemetrexed followed by maintenance therapy with pemetrexed. The metastatic cardiac tumor shrank, and the atrioventricular (AV) block in ECG was improved. In this case, FDG-PET and chemotherapy were valuable for diagnosis and treatment of cardiac metastasis from non-small cell lung cancer.
We report the case of a 58-year-old woman who presented with acutely developed coma characterized by electroencephalographic triphasic waves (TWs) in the absence of metabolic derangement. The patient's coma and TWs were promptly resolved after the administration of glucocorticoids, and thereafter isolated ACTH deficiency was diagnosed. Isolated ACTH deficiency may present with glucocorticoid-responsive acute encephalopathy without hypoglycemia, hyponatremia, or systemic hypotension. Electroencephalographic TWs or bursts of slow waves may be a clue to the diagnosis of this rare condition in patients with coma of unknown origin.
Amyotrophic lateral sclerosis (ALS) with demyelinating polyneuropathy is a rare condition. We describe two ALS patients with demyelinating neuropathy. Immunomodulatory therapies brought slight symptomatic benefits to the patients, but the treatments could not halt the progression of ALS. Chance coincidence of the two diseases is unlikely in view of the low prevalence. ALS, mainly consisting of progressive axonal degeneration, might show temporal demyelinating features of peripheral nerves both electrophysiologically and pathologically. The pathomechanism for the demyelination in ALS remains to be elucidated.
A 63-year-old man with a past history of resection of pulmonary adenocarcinoma and COPD visited our hospital because of fever, cough and purulent sputum. Chest CT showed an infiltration shadow with multiple bullae in the right lung. There was a slight elevation of the inflammatory response. We established a definitive diagnosis by frequent isolation of Mycobacterium kyorinense on a sputum culture test of acid-fast bacilli. Clarithromycin and levofloxacin were administered after identification of M. kyorinense using a 16S rRNA gene sequence. Subsequently his symptoms improved following combined therapy with clarithromycin and levofloxacin.
A 28-year-old woman was referred to our hospital for treatment of tuberculous lymphadenitis, after presenting with fever, left cervical lymphadenopathy, and a positive interferon-gamma release assay (QuantiFERON®-TB Gold In-Tube; QFT) result. Surprisingly, biopsy specimens of the cervical lymph nodes showed necrotic lesions with prominent nuclear debris and a proliferation of histiocytes, consistent with Kikuchi-Fujimoto disease (KFD). A diagnosis was made of KFD complicated by latent tuberculosis infection (LTBI), and all symptoms had resolved completely two months post-diagnosis. KFD may be misdiagnosed as tuberculous lymphadenitis, and antibiotics unnecessarily prescribed. Careful attention should therefore be paid when diagnosing cervical lymphadenopathy.
Since the worldwide spread of the novel influenza type A virus in 2009, trivalent vaccines against H1N1 (pandemic) 09 and seasonal influenza have been used. We describe a 33-year-old woman who presented with hypoesthesia below the Th7 level fifteen days after vaccination without any preceding infection. Cerebrospinal fluid showed an increased level of myelin basic protein and positive oligoclonal IgG bands. Magnetic resonance imaging revealed disseminated lesions in the brain and thoracic cord. Steroid therapy improved her symptoms. She was diagnosed as having acute disseminated encephalomyelitis (ADEM) possibly related to the vaccination. As a potential adverse effect of the influenza vaccine, in addition to Guillain-Barré syndrome, ADEM should also be recognized.
Syncope is common in clinical practice, but the cause is often difficult to diagnose. We report a 75-year-old man who was referred to the emergency department because of syncope after an urgent sensation of urinating during jogging. He was finally diagnosed as having a giant bladder diverticulum due to prostatic hyperplasia accompanied by neurally mediated syncope. Excessive urinary retention is a possible cause of not only giant bladder diverticula but also syncope due to vagal enhancement.