Objective In acetaminophen-induced acute liver failure (ALF), the hepatic coma grade worsens and mortality rates increase, as the number of systemic inflammatory response syndrome components fulfilled (SIRS score) increases. This study aimed to investigate the impact of SIRS score on clinical features of non-acetaminophen-related ALF.
Methods Ninety-nine patients with non-acetaminophen-related ALF with hepatic coma who did not undergo liver transplantation were investigated. Each patient was given a SIRS score of 0, 1, 2, 3 or 4 at the time of diagnosis.
Results At the diagnosis of ALF with hepatic coma, with the increase of SIRS score, hepatic coma grade and prothrombin activity were deteriorated. After the diagnosis of ALF with hepatic coma, 25 patients (25%) developed acute respiratory distress syndrome (ARDS), 31 patients (31%) developed disseminated intravascular coagulation (DIC), and 21 patients (22%) developed acute renal failure (ARF). Thirty-eight patients (38%) developed MOF. With the increase of SIRS score, frequencies of the development of ARDS, DIC and MOF increased. ARF was more frequently developed in patients with a SIRS score of 2 or higher. Overall, 36 patients (36%) survived. Overall survival rate was 66% in 29 patients with a score of 0, 43% in 21 patients with a score of 1, 17% in 29 patients with a score of 2 and 15% in 20 patients with a score of 3 or 4.
Conclusion SIRS score will be useful for predicting not only the overall survival but also the development of complications such as ARDS, DIC and MOF in non-acetaminophen-related ALF with hepatic coma.

View full abstract

Background It has been reported that the use of benzodiazepines (BDZs) is associated with an increased risk of coronary artery disease (CAD). However, it is still unclear whether or not non-BDZs use has the same adverse effect on the cardiovascular system.
Methods We conducted a hospital-based case-control study with 864 CAD patients and 1,008 controls to explore the association between non-BDZs use and CAD risk in Chinese Han population.
Results Non-BDZs use was found to be associated with a decreased risk of CAD (adjusted odds ratio, OR=0.73; 95% confidence interval, CI=0.54-0.98). Adjusted ORs were 0.91 (95% CI=0.63-1.32) for using non-BDZs 0 to 5 years, 0.64 (95% CI=0.38-0.97) for 5 to 10 years, and 0.42 (95% CI=0.18-0.85) for >10 years. The risk of CAD tended to decrease with an increase in duration of non-BDZs use (p<0.001 for trend).
Conclusion Our study demonstrates that non-BDZs use is associated with a decreased risk of CAD. The potential cardiovascular protective mechanisms of non-BDZs should be investigated further.

View full abstract

Background Epicardial adipose tissue (EAT), localized beneath the visceral pericardium, is a metabolically active endocrine and paracrine organ with possible interactions within the heart. Coronary artery ectasia (CAE) is a clinical entity characterized with localized or diffuse dilatation, of the coronary arteries, with a diameter of greater than 1.5 times that of adjacent segments. Although the etiopathogenesis is not clearly understood, some studies have revealed that CAE may be a form of atherosclerosis that has greater inflammatory properties than atherosclerosis. The goal of this study was to investigate whether EAT and the level of C-reactive protein (CRP) are increased in patients with isolated CAE compared to normal subjects.
Methods Thirty-three patients with isolated CAE (mean age: 57±9 years) and 32 age- and gender-matched control participants with NCA, but without CAE (mean age: 56±10 years), were included in the study. The relationship between EAT thickness, CRP levels and the presence of CAE was investigated.
Results Epicardial adipose tissue thickness was significantly higher in CAE group compared to NCA group (7.2±3.2 vs. 4.7±2.1 mm, p<0.001). Body mass index (BMI, p=0.013), CRP (p=0.047), and the percentage of isolated CAE (p=0.012) were significantly higher in patients with an increased EAT thickness. While CRP correlated with increased EAT, it was not related to CAE. However, CRP levels were higher in patients with diffuse coronary ectatic involvement than the focal lesions (0.58±0.32 vs. 0.31±0.11 mg/dL, p=0.046). When we performed multiple logistic regression analysis, only increased EAT thickness was related to CAE independent of CRP and BMI (OR: 1.442, 95%CI: 1.066-1.951, p=0.018).
Conclusion This is the first study, displaying a significantly higher EAT-thickness in patients with isolated CAE. We believe that further studies are needed to clarify the role of adipose tissue in patients with isolated CAE.

View full abstract

Objective Women with gestational diabetes mellitus (GDM) treated with insulin are more likely to develop type 2 diabetes after pregnancy compared to mild GDM cases treated with medical nutrition treatment (MNT) alone. We aimed to compare levels of subclinical atherosclerosis markers in women with previous GDM treated with insulin and MNT alone.
Methods Eighty-one women with previous GDM (45 treated with insulin, 36 treated with MNT) and 35 age-matched lean controls were included. Fasting glucose, insulin and lipids, circulating fibrinogen, CRP, PAI-1 and IL-6 levels were assayed. Carotid intima media thickness (IMT) was measured.
Results Women with previous GDM treated with insulin in pregnancy had significantly higher fasting glucose, plasma PAI-1 levels and carotid IMT compared to women treated with MNT alone. In multiple regression analysis, insulin need in pregnancy was associated with increased carotid IMT and plasma PAI-1 levels (corrected for age, BMI, postpartum duration, fasting glucose and lipids; model r²=0.132; beta=0.297, p=0.014 for carotid IMT; model r²=0.198; beta=0.345, p=0.003 for PAI-1).
Conclusion Women with previous GDM treated with insulin in pregnancy had a worse cardiovascular risk profile compared to mild GDM patients. An intensive preventive approach for cardiovascular disorders is particularly essential for this subgroup of women.

View full abstract

Objective We conducted a cohort study to identify the risk factors for vitamin D deficiency in predialyzed patients with chronic kidney disease (CKD).
Methods An observational study of 135 outpatients with stage 3-5 CKD was undertaken. Clinical and biochemical parameters were analyzed in terms of nutritional status, inflammation, and mineral metabolism in relation to serum levels of 25-hydroxyvitamin D [25(OH)D]. Levels of 25(OH)D lower than 15 ng/mL were considered to be deficient.
Results The 25(OH)D-deficient group had a higher body mass index (24.1±4.2 kg/m² vs. 22.5±4.0 kg/m², p=0.0322), and had more diabetic patients (27.9% vs. 3.6%, p=0.0003). The multivariate analysis revealed that body mass index (odds ratio=2.758; 95% CI, 1.048-7.721; p=0.0398), the presence of diabetes (odds ratio=7.792; 95% CI, 1.808-55.439; p=0.0043), lower hemoglobin concentration (odds ratio=0.297; 95% CI, 0.099-8.732; p=0.821), higher serum levels of non-HDL cholesterol (odds ratio=3.570; 95% CI, 1.449-9.442; p=0.0053) and triglyceride (odds ratio=2.447; 95% CI, 0.779-1.776; p=0.0258) were the factors associated with low 25(OH)D levels.
Conclusion Vitamin D deficiency was common among the predialysis CKD patients, and the factors identified as being associated with vitamin D deficiency were diabetes and obesity.

View full abstract

Objective Several recent studies report that, after allogeneic hematopoietic cell transplantation (allo-HCT), eosinophilia is a favorable factor for transplant outcomes. However, whether the degree of eosinophilia influences transplant outcomes is yet to be established.
Methods We studied 144 patients with hematological malignancy who received allo-HCT at our institution. The stem cell sources were bone marrow in 84 patients, peripheral blood stem cells in 32 patients, and cord blood in 28 patients. One hundred and twelve patients underwent myeloablative conditioning and 49 patients had high-risk disease. We performed semi-landmark analysis to examine the influence of eosinophilia.
Results Eosinophilia developed at a median of 47 days after transplantation in 63 patients (44%). The patients with eosinophilia showed significantly better overall survival (OS) and a lower relapse rate at three years, compared to those without eosinophilia (66% vs 55%, p=0.04 and 30% vs 50%, p=0.002). On analysis following division into groups with mild (500-1,500×10⁶/L) and hyper- (>1,500×10⁶/L) eosinophilia, three-year OS and relapse rates were 68% and 65% (p=0.92), and 31% and 28% (p=0.90), respectively. On multivariate analysis, eosinophilia was significantly associated with lower relapse rates [HR: 0.5 (95% CI: 0.3-0.9), p=0.01] and the same trend was preserved in the analysis of the mild and hyper-eosinophilic groups.
Conclusion The results suggest that eosinophilia after allo-HCT was associated with better OS and a lower relapse rate, regardless of the levels. The mechanism of this effect is still unclear, and requires study of the pathophysiological process to clarify the relationship between the higher levels of eosinophilia after allo-HCT and organ infiltration.

View full abstract

Objective Recently, pulsed high-dose dexamethasone (HD-Dexa) therapy was proposed as a possible alteration for the classical prednisolone (PSL) therapy for primary immune thrombocytopenia (ITP) patients, however it remains to be confirmed which of these remedies is superior. So the objective of this study is to compare the efficacy and the sustainability of these options.
Methods The first-line therapy at our institute for untreated adult ITP cases was accordingly changed as follows, and we retrospectively evaluated the outcomes: 1) daily administration of 0.5-1 mg/kg PSL for 2-4 weeks and subsequently stepwise reduction, 2) one course of HD-Dexa (40 mg/day for four consecutive days, 1xHD-Dexa), 3) three courses of the same dose of HD-Dexa (3xHD-Dexa) repeated biweekly. This study was approved by the ethical committee of the University of Tokyo.
Results Twenty-five patients were enrolled consecutively. A good initial response was attained through all the regimens. Meanwhile, time to next treatment for lack of response or relapse was significantly longer in the PSL group than in the other groups (log-rank test, PSL vs. 1xHD-Dexa p<0.001, PSL vs. 3xHD-Dexa p=0.0053, respectively). Additionally, PSL regimen conferred a significantly longer duration time of response (PSL vs. 1xHD-Dexa p=0.0024, PSL vs. 3xHD-Dexa p=0.028, respectively) and CR (PSL vs. 1xHD-Dexa p=0.012, PSL vs. 3xHD-Dexa p=0.0090, respectively). No patient discontinued the treatment due to side effects in this study.
Conclusion PSL regimen was considered to be superior to pulsed HD-Dexa regimens in the sustainability of response.

View full abstract

Objective Anhedonia is one of the main non-motor symptoms in Parkinson's disease (PD); it is assessed using the Snaith-Hamilton pleasure scale (SHAPS). To assess anhedonia in the Japanese population, we prepared a Japanese language version of SHAPS (SHAPS-J), and evaluated its validity and reliability in 8 neurological centers. Seventy subjects (48 patients with PD and 22 healthy subjects) were enrolled in this study.
Methods The validity of the test was assessed by the correlation between SHAPS-J and the apathy scale, based on the fact that anhedonia is considered a symptom of apathy syndrome. Test-retest reliability and internal consistency were assessed by Cohen's kappa and Cronbach's alpha coefficients, respectively.
Results In the evaluation of validity, the total scores obtained on SHAPS-J during the test and retest significantly correlated with scores on Item 4 in Part 1 of the unified Parkinson's disease rating scale (p<0.0008 and p<0.0036, respectively). Cohen's kappa coefficient was >0.3 on all items (p<0.0005 on all items). Cronbach's alpha coefficient was 0.90 at the baseline and 0.88 at the retest.
Conclusion These results indicate that SHAPS-J has good validity, test-retest reliability, and internal consistency, thus establishing an available measure of anhedonia in Japanese.

View full abstract

Objective While much attention is given to the fifth vital sign, the utility of the 4 classic vital signs (blood pressure, respiratory rate, body temperature, and heart rate) has been neglected. The aim of this study was to assess a possible association between vital signs and the Sequential Organ Failure Assessment (SOFA) score in patients with sepsis.
Methods We performed a prospective, observational study of 206 patients with sepsis. Blood pressure, respiratory rate, body temperature, and heart rate were measured on arrival at the hospital. The SOFA score was also determined on the day of admission.
Results Bivariate correlation analysis showed that all of the vital signs were correlated with the SOFA score. Multiple regression analysis indicated that decreased values of systolic blood pressure (multivariate regression coefficient [Coef] = -0.030, 95% confidence interval [CI] = -0.046 to -0.013) and diastolic blood pressure (Coef = -0.045, 95% CI = -0.070 to -0.019), increased respiratory rate (Coef = 0.176, 95% CI = 0.112 to 0.240), and increased shock index (Coef = 4.232, 95% CI = 2.401 to 6.062) significantly influenced the SOFA score.
Conclusion Increased respiratory rate and shock index were significantly correlated with disease severity in patients with sepsis. Evaluation of these signs may therefore improve early identification of severely ill patients at triage, allowing more aggressive and timely interventions to improve the prognosis of these patients.

View full abstract

Small bowel tumors are rare; most are single and located in the duodenum. When a patient presents with unreasonable abdominal pain and distension, with normal upper gastrointestinal endoscopy and colonoscopy, it is important to consider this disease. Here, we report a case of segmental and multiple adenocarcinoma of the small bowl presenting with unreasonable abdominal pain and distension in a 76-year-old woman, and provide a brief review on this subject. Our report highlights the fact that segmental and multiple small bowel tumors are very rare and the clinical characteristics are generally vague and nonspecific.

View full abstract

Celiac disease (CD) is treated by eliminating all gluten from the diet. A 49-year-old man with CD was admitted to our clinic with complaints of recurrent diarrhea and abdominal pain despite strict adherence to a gluten-free diet. The duodenum was seen to be edematous on gastroduodenoscopic examination. Histological examination of the biopsy specimen taken from the duodenum showed multiple round shaped Strongyloides stercoralis (S. stercoralis) larvae within the crypts. He was successfully treated with albendazole. This case emphasizes the importance of duodenal biopsy in CD. To our knowledge, this is the first case in the literature showing CD and S. stercoralis together.

View full abstract

We describe a well-differentiated hepatocellular carcinoma (HCC) with alcohol-related liver cirrhosis in a 69-year-old man. Ultrasonography (US) disclosed a 10 mm hypoechoic nodule in segment 4; Sonazoid contrast-enhanced US and gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) revealed no defect in either the Kupffer phase or the hepatobiliary phase. Computed tomography during hepatic arteriography (CTHA), however, revealed a hypovascular nodule, but CT during arterial portography showed no perfusion defect. Histological analysis indicated a well-differentiated HCC. Thus, our detection of well-differentiated HCC disclosed by only CTHA attested to the efficiency of this modality, suggesting that it is more sensitive than Gd-EOB-GTPA-enhanced MRI.

View full abstract

Neurocardiogenic syncope (NCS) is a common clinical entity. Most of these patients are managed medically by internists and general practitioners. Though NCS is frequently a benign disease, a malignant form of this disorder with episodes of prolonged cardioinhibition culminating in asystole is described. Here, we describe a 52-year-old woman who had such a life threatening malignant form of arrhythmia during a routine colonoscopy and review the literature of similar cases.

View full abstract

Stress-induced cardiomyopathy, a reversible left ventricular dysfunction, has been reported following anaphylaxis; this clinical circumstance seems to be linked to elevated levels of circulating catecholamines. We present a 36-year-old woman diagnosed as stress-induced cardiomyopathy following ceftriaxone-induced anaphylaxis. After anaphylactic reaction, the patient initially presented with cardiogenic shock, and subsequently developed multiple organ failure. She recovered basically by multiple organ supportive therapies including intra-aortic balloon pump and continuous veno-venous hemofiltration. This case provides a unique opportunity to observe the triggering of stress-induced cardiomyopathy, and also it provides evidence to support the role of catecholamine in the pathogenesis of this disease.

View full abstract

This case report represents the difficulties in diagnosing brucellosis, which is an enigma with unusual cardiovascular complications. A 32-year-old Caucasian man with acute chest pain was examined at Sfax Hedi Chaker's Hospital. He had a night fever, although his cardiac examination was normal. Further laboratory analyses showed an elevated C-reactive protein of 20.8 mg/dL and troponine I of 1.469 IU/L. A cardiac MRI using delayed enhancement was then performed. The T2-weighted short-axis showed a subepicardial delaying enhancement of infero-lateral and the basal walls of the left ventricle. Accordingly, a diagnosis of Brucella-related myocarditis was made.

View full abstract

A 73-year-old woman presented to our hospital with scleritis, cough, and hemoptysis. Chest computed tomography showed a mass lesion, multiple ground-glass opacities, and nodules. Bronchoscopy showed a skip lesion of mucosal erosion with white necrotic substance; however, a biopsy specimen of the erosion yielded no characteristic findings. Transbronchial lung biopsy showed alveolar hemorrhage and capillaritis. Proteinase-3 antineutrophil cytoplasmic antibody was elevated to 62.5 U/mL, and she was diagnosed as having Wegener's granulomatosis. Steroid therapy combined with cyclophosphamide was started; however, hemoptysis was not resolved until plasmapheresis therapy was added. Her general condition initially improved, but respiratory insufficiency worsened with fever on the 30th hospital day. The patient subsequently died on the 41st hospital day, and cytomegalovirus infection was suggested as a possible cause of death.

View full abstract

Reversible posterior leukoencephalopathy syndrome (RPLS) is uncommon neurological syndrome that is characterized by specific clinical and radiologic findings. Previous reported associations of RPLS include hypertension, eclampsia, renal impairment and drugs. Prompt diagnosis and therapy is critical to ensure resolution of the neurological disability. Some cases have been reported in association with the increased use of antineoplastic agents in cancer patients. We report the case of a 62-year-old man who was diagnosed with RPLS after receiving carboplatin and paclitaxel chemotherapy for lung cancer. This case appears to be the first recognized association of RPLS with this regimen.

View full abstract

We describe the first case of genetically diagnosed congenital dyserythropoietic anemia (CDA) type 1 in a Japanese man. The patient had hemolytic anemia since he was a child, and he developed diabetes, hypogonadism, and liver dysfunction in his thirties, presumably from systemic iron overload. When he was 48 years old a diagnosis was finally made by genetic analysis that revealed a homozygous mutation of CDAN1 gene (Pro1129Leu). His serum hepcidin-25 level was inappropriately low. We conclude that physicians should be aware of the possibility of CDA in a patient with anemia and systemic iron overload at any age.

View full abstract

We report a patient with partial oculomotor paresis due to midbrain infarction. A 69-year-old man noticed diplopia suddenly. Ptosis, and impaired adduction and supraduction were found in the right eye. The pupillary size and light reflexes were normal on both sides. Magnetic resonance imaging disclosed an acute lesion in the right inferolateral oculomotor fascicle. These clinicoradiological findings suggested that the inferolateral fascicular damage could cause palsy of the levator palpebrae, medial rectus, superior rectus and inferior oblique muscles. Physicians should pay more attention to oculomotor fascicular infarction in patients with incomplete oculomotor paresis, and spared pupil sphincter and inferior rectus muscles.

View full abstract

Venous congestive myelopathy associated with spinal dural arteriovenous fistulas (DAVFs) is a treatable disorder that can be controlled without sequelae if it is diagnosed at an early stage. It is important to consider spinal DAVFs in the differential diagnosis based on clinical history and neurologic examination. We report the unique case of a patient with DAVFs at the craniocervical junction presenting with occipital/neck pain associated with sleep.

View full abstract

Beriberi is a disease caused by thiamine deficiency resulting in peripheral neuropathy and myocardial dysfunction. Increases in vascular endothelial growth factor (VEGF) are seen in polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, called POEMS syndrome. We present herein two cases of wet beriberi accompanied by a moderate increase in VEGF level. Serum VEGF decreased after treatment in both cases. Our experience with these cases suggests that beriberi should be considered in the differential diagnosis of polyneuropathy with a moderate increase in serum VEGF, and that the serum VEGF level may be a therapeutic marker for beriberi.

View full abstract

We report a patient with restless legs syndrome (RLS) and chronic liver disease (CLD), whose RLS symptoms fluctuated in correlation with serum ammonia level. RLS in this patient seemed to be secondary to CLD because palliative medications for the liver dysfunction showed an additional effect on RLS symptoms that were partially controlled by levodopa. CLD should be born in mind as one of the factors to cause RLS symptoms; the therapeutic mechanism of RLS enhanced by palliative medications for CLD is discussed.

View full abstract

Immunoglobulin G4 (IgG4)-related disease is a distinctive mass-forming disorder with frequent systemic involvement, most commonly in the pancreas, salivary glands and lacrimal glands. A few cases of dural involvement and one case of central airway stenosis have also been described. We report here a rare case of IgG4-related disease with intracranial hypertrophic pachymeningitis and irregular tracheobronchial stenosis. We review four previously reported cases of IgG4-related pachymeningitis. We currently lack international standards for the diagnosis of extrapancreatic IgG4-related disease. Based on the findings of the present case and those reported previously, we discuss the distinctive features of IgG4-related pachymeningitis.

View full abstract

A 53-year-old man visited our hospital complaining of high fever. Chest computed tomography showed left pleural effusion and mediastinitis. He developed painful red subcutaneous nodules in his bilateral lower extremities. Thoracoscopy-assisted exploratory excision showed visceral pleura thickening; panniculitis in the periaortic area was histologically proven. The patient was treated with corticosteroid therapy which immediately reduced the fever. Subsequent imaging examinations after corticosteroid therapy showed improvement of mediastinitis and pleural effusion. This case reminds us that Weber-Christian disease (WCD) should be included in the differential diagnosis of mediastinitis although WCD is rarely associated with thoracic involvement.

View full abstract

We describe a case of 78-year-old woman with a 1-week history of fever and left hemiparesis. Head magnetic resonance imaging showed a small infarct. After admission, she showed altered consciousness and another small infarct. She finally had diagnoses of miliary tuberculosis (miliary-TB) and tuberculous meningitis (TBM). She recovered after receiving anti-tuberculous therapy (ATT) with prednisolone. However, 5 weeks later, we found another infarct. This is a rare case of TBM with recurrent infarcts in atypical lesions in spite of ATT. We suggest the possibility that the new infarct after ATT was due to a paradoxical reaction.

View full abstract

Brucellosis is an important disease in developing countries. Endocarditis is a complication of brucellosis with the highest mortality. Although the most generally accepted therapy is the combination of medication and surgery, it has been reported that antibiotic treatment only might also be adequate. We present two cases for whom antibiotic treatments were initiated, and a follow-up surgery was planned for one of them. The surgery could not be done due to death of the patient, but the other patient fully recovered with antibiotic treatment only. Optimum treatment and prognostic criteria are not well defined for brucella endocarditis, but medical therapy alone can be considered for some chosen patients.

View full abstract

An 84-year-old-man was admitted to the Department of Neurosurgery for a sudden episode of fainting. Brain computed tomography and magnetic resonance imaging demonstrated no fresh lesions. Anorexia, fever and elevation of C-reactive protein and creatine phosphokinase were observed, and the patient was transferred to the Department of Internal Medicine for further examination and treatment. High-dose steroids and antibiotics were administered, and his fever subsided. However, massive hemoptysis suddenly developed and the patient died. A thoracic aortic aneurysm that had coalesced and ruptured a left lung bronchus was detected at autopsy. Pathological examination revealed an inflammatory aortic aneurysm.

View full abstract

IgG4-related disease (IgG4RD) is a unique systemic lymphoproliferative disorder characterized by elevated serum IgG4 levels and IgG4-producing plasma cell expansion in the affected tissues, which are accompanied by fibrotic or sclerotic changes. Vascular lesions may also be a part of IgG4RD as a number of case reports have discussed inflammatory abdominal aortic aneurysms associated with IgG4RD, but coronary artery lesions seem to be rare complications of IgG4RD. A 71-year-old man suffered from multiple giant coronary aneurysms and an abdominal aortic aneurysm with concurrent pancreatic, gall bladder, bile duct, and salivary gland lesions resulting from IgG4RD. The present observations suggest that coronary aneurysms may also develop as a consequence of this disease.

View full abstract

A 34-year-old Japanese woman presented at our institution weighing 182.7 kg, 148.2 cm tall, and with a body mass index of 83.2 kg/m². She had been overweight since childhood, but no abnormality was found to explain her obesity. Treatments, including mazindol, bofu-tsusho-san, dietary restriction, and BioEnterics Intragastric Balloon, did not result in improvement of her obesity. Finally, we performed sleeve gastrectomy, and she has maintained her weight within 130-140 kg without rebounding for 2 and a half years. We followed the clinical changes before and after the operation. This case provides potentially interesting information regarding operative treatment for morbid obesity in Japanese.

View full abstract