Objective Postsplenectomy sepsis (PSS) and overwhelming postsplenectomy infection (OPSI) following splenectomy or the development of hyposplenism are associated with a high mortality rate. The presence of Howell-Jolly bodies (HJBs) in peripheral erythrocytes is attracting attention as a parameter of hyposplenism. To date, whether HJBs appear following partial splenic embolization (PSE) has not been investigated. Therefore, we examined the prevalence of HJBs in patients who have undergone PSE.
Methods Whether HJBs were present in 95 patients who underwent PSE between November 2007 and August 2012 was assessed.
Results No serious complications occurred due to PSE; however, 17 of the 95 patients (17.89%) exhibited HJBs during the follow-up. The residual spleen volume and splenic infarction rate did not differ significantly compared to those observed in the HJB-negative group.
Conclusion With the recent increase in the use of autoanalyzers, the opportunities to perform microscopic examinations have been decreasing. Therefore, the presence of HJBs, which can only be confirmed visually, may be overlooked, and the clinical significance of these bodies tends to be disregarded. However, the presence of HJBs is associated with a risk of PSS and OPSI due to hyposplenism. Because HJBs are common in the peripheral erythrocytes of patients who have undergone PSE, irrespective of the residual spleen volume or splenic infarction rate, the presence or absence of HJBs should be assessed visually. In HJB-positive patients, preventing serious infections, for example, by administering the pneumococcal vaccine, is important.

View full abstract

Objective This study was undertaken to determine the relationship between the red cell distribution width (RDW) and the one year outcomes in Chinese patients with stable angina pectoris.
Methods A total of 1,442 patients with stable angina pectoris on admission were divided into four groups according to quartiles of the baseline RDW. The relationships between the RDW and one-year cardiac mortality as well as the incidence of acute coronary syndrome (ACS) were assessed.
Results Higher RDW values were associated with an increased one-year cardiac mortality (quartile 1: 0.51%; quartile 2: 0.56%; quartile 3: 0.86%; quartile 4: 2.27%; p<0.001) and one-year ACS (quartile 1: 1.55%; quartile 2: 1.96%; quartile 3: 2.89%; quartile 4: 3.70%; p<0.001). A logistic regression analysis revealed that the RDW independently predicted cardiac mortality (OR: 1.544, 95% CI: 1.058-3.216, p<0.001) and ACS (OR: 1.861, 95% CI: 1.226-3.487, p<0.001) during a one-year follow-up in patients with stable angina pectoris.
Conclusion The present study indicates that an elevated RDW value is associated with an increased risk of one-year adverse outcomes in patients with stable angina pectoris.

View full abstract

Objective The visceral fat area (VFA) was measured, and the relationships between the VFA and the body mass index (BMI), waist circumference, blood pressure, and indices of lipid and sugar metabolism were evaluated.
Methods The subjects included 607 consecutive patients who underwent VFA examinations using computed tomography (CT) scans. In addition to the routine examination parameters, the levels of adiponectin and homeostasis model assessment as an index of insulin resistance (HOMA-IR) were measured in all subjects, and the levels of malondialdehyde-modified low-density lipoprotein (MDA-LDL), remnant-like particles (RLP), lipoprotein (a) (Lp(a)), apolipoprotein (Apo) AI, ApoB and ApoE were measured in 270 subjects.
Results In both men and women, the VFA showed significant positive correlations with the age, BMI, waist circumference, subcutaneous fat area, visceral fat area/subcutaneous fat area (v/s) ratio, systolic blood pressure, diastolic blood pressure, the fasting blood sugar (FBS), the hemoglobin A1c (HbA1c), high-density lipoprotein cholesterol (HDLC), triglyceride (TG), uric acid, HOMA-IR and ApoB and the ApoB/LDLC ratio and significant negative correlations with the levels of HDLC and adiponectin. The levels of the total cholesterol (TC), low-density lipoprotein cholesterol (LDLC), non-HDLC, MDA-LDL and Lp(a) and the ApoB/ApoAI ratio were not correlated with the VFA in either men or women. The RLP exhibited a significant positive correlation with the VFA in women.
Conclusion The VFA exhibited high positive correlations with the waist circumference, blood pressure and TG level and a negative correlation with the HDLC level, regardless of gender, supporting the validity of the present diagnostic method for evaluating metabolic syndrome (MS). Although the LDLC level is not included in the diagnostic criteria for MS, the positive correlations between the VFA and the ApoB level and ApoB/LDLC ratio observed in both men and women indicate qualitative abnormalities of lipoproteins, such as an increase in the amount of small dense LDL. Measuring the levels of apolipoproteins in addition to lipoproteins during health screening is therefore useful for evaluating of atherogenicity.

View full abstract

Objective Predictors of the need to initiate noninvasive ventilation (NIV) in stable COPD outpatients with acute exacerbation of chronic obstructive pulmonary disease (COPD-AE) are insufficiently defined. The objective of this study was to investigate predictors of the need to initiate NIV in stable COPD-AE outpatients.
Methods A total of 140 consecutive stable COPD outpatients who were hospitalized for COPD-AE for the first time were retrospectively examined. Demographic and clinical parameters measured in the stable state were evaluated, including data for arterial blood gases, the pulmonary function, body mass index and dyspnea scores.
Results Of the 140 patients included, NIV was used in 32 (23%) patients. In a univariate Cox proportional hazards analysis, the baseline partial pressure of arterial carbon dioxide (PaCO₂) (hazard ratio (HR), 1.14 per mmHg; 95% confidence interval (CI), 1.08-1.21) and forced expiratory volume in one second (FEV₁)% predicted (HR, 1.03 per %; 95% CI, 1.01-1.05) were identified to be significant predictors. A multivariate logistic regression analysis showed only the PaCO₂ (HR, 1.18 per mmHg; 95% CI, 1.12-1.26) to be a significant predictor.
Conclusion Our results suggest that the PaCO₂ measured in the stable state is an independent significant predictor of the need to initiate NIV in COPD-AE patients who are hospitalized for the first time.

View full abstract

Objective High-sensitivity C-reactive protein (hs-CRP) is an extremely sensitive systemic marker of inflammation and tissue damage, and increased levels of hs-CRP are strongly associated with inflammatory reactions. Microglia-mediated neuroinflammation has been hypothesized to play an important role in the pathogenesis of idiopathic Parkinson's disease (PD). However, the clinical value of the hs-CRP level in patients with PD is poorly defined. Therefore, we conducted this study to analyze the differences in the hs-CRP levels in PD patients with and without dementia.
Methods We examined 72 PD patients without dementia (PDwoD) and 45 PD patients with dementia (PDD), as well as 84 control subjects. We investigated the differences in the hs-CRP and fibrinogen levels between these three groups.
Results The mean hs-CRP and fibrinogen values were not significantly different between the PDwoD and PDD groups; however, these two groups had significantly higher mean hs-CRP and fibrinogen values than the control group.
Conclusion It is known that inflammation plays a role in the pathogenesis of PD and dementia. However, based on the results of this study, we cautiously speculate that although neuroinflammation plays a role in the development of neurodegenerative diseases, including PD and dementia, it may be unrelated to the pathogenesis of dementia in patients with PD.

View full abstract

Objective The aim of the present study was to examine the immunohistological characteristics of disseminated Mycobacterium avium infection after the initiation of antiretroviral therapy (ART) for acquired immunodeficiency syndrome (AIDS).
Methods We histologically investigated five autopsied AIDS patients with systemic M. avium infection.
Results The inflammatory cell composition in the affected tissues was assessed using immunohistochemistry. The celiac lymph nodes and intestinal canal were the most commonly involved organs in the AIDS cases. The most common histological feature was unstructured aggregation of histiocytes. Immunohistochemistry revealed depression of CD4⁺, CD8⁺ and CD57⁺ cells in the gut lamina propria and mesenteric lymph nodes.
Conclusion These findings suggest that local cell-mediated immunity is depressed in affected tissues and that the primary histological feature is poor organization of granulomas in mycobacterial lesions, despite the administration of adequate ART.

View full abstract

Mutations in SCN5A have been reported to cause several types of hereditary arrhythmias (overlap syndrome). We herein report two patients with the overlapping phenotypes of juvenile sick sinus syndrome (SSS) and Brugada syndrome (BrS). The proband was a man who was in his twenties and had been diagnosed with both SSS and ventricular tachycardia (VT). A pilsicainide challenge test revealed a coved type ST segment elevation. His teenage brother also suffered from SSS, but no VT had been documented. A pilsicainide challenge failed to produce a Brugada-type ST elevation, but there was a marked prolongation of the His-ventricle interval. Their electrocardiograms at rest did not display any Brugada-type ST elevations. We identified a novel SCN5A (F1775Lfs*15) mutation in both patients, even though there was a phenotype discrepancy.

View full abstract

A 79-year-old woman presented with unexplained hypoxia that became exacerbated by an upright posture (platypnea-orthodeoxia syndrome). A ^99mTc-macroaggregated albumin pulmonary perfusion scan revealed a right to left shunt of 25.5% in the supine position and 32.3% in the sitting position. A dynamic CT scan and a transoesophageal echocardiogram confirmed the presence of a shunt across an atrial septal defect (ASD). A percutaneous transcatheter closure of the defect significantly improved the patient's blood oxygenation levels when she was in the upright position. An ASD should therefore be included in the differential diagnosis of platypnea-orthodeoxia syndrome, regardless of the patient's age.

View full abstract

Numb chin syndrome (NCS) and painful ophthalmoplegia (PO) are neurological syndromes associated with the disturbance of certain cranial nerves and their downstream nerves. These syndromes are caused by various diseases, and, in rare cases precede the diagnosis of systemic malignant lymphoma. We herein present the case of a 59-year-old man diagnosed simultaneously with NCS and PO caused by a tumor located around the cavernous sinus and with diffuse large B-cell lymphoma that was identified via biopsy of a large retroperitoneal tumor. He was successfully treated with a standard rituximab-containing chemotherapy combined with high-dose intravenous methotrexate and intrathecal chemotherapy.

View full abstract

We herein report an unusual case of profound brain infarction of the posterior circulation due to a dolichoectatic vertebrobasilar dissecting aneurysm (DVDA) originating from atherosclerosis. On autopsy, diffuse atherosclerosis was observed with a multi-fusiform aneurysm measuring 1 to 2 cm in diameter ranging from the left vertebral artery to the basilar artery. The microscopic findings of the aneurysm revealed severe stenosis of the artery caused by intimal thickening, intimal flap formation and thrombosis, indicating the presence of a dissecting aneurysm originating from atherosclerosis. The DVDA observed in this case was considered to be slowly progressive and associated with the development of atherosclerosis. The etiology of structural destabilization in patients with DVDA involves rupture of the internal elastic lamina, which is dislodged by massive hematomas that form atheromatous lesions.

View full abstract

We herein report an autopsy case of the Marburg variant of multiple sclerosis (MS). A 29-year-old woman developed acute and progressive neurological symptoms. A diagnosis of MS was suspected based on the patient's clinical background and brain MRI findings and the lack of evidence of malignancy on a brain biopsy. Despite the administration of typical treatment for MS, a fatal outcome occurred three months after disease onset. The autopsy revealed multiple inflammatory demyelinating lesions in the central nervous system. In addition, two noteworthy histopathological features were observed compared with prototypical MS. We evaluate the pathogenic differences between the Marburg type and prototypical MS by discussing the neuropathology and cerebrospinal fluid (CSF) findings of our case.

View full abstract

We herein report the findings of 2 cases of normotensive scleroderma renal crisis (SRC) that developed soon after the commencement of a glucocorticoid therapy. We also review 8 cases of normotensive SRC reported in Japan, including our cases. The common characteristics of these 8 cases are as follows: the recent onset of systemic sclerosis, the presence of diffuse skin sclerosis, the presence of myositis and/or serositis, a high titer of antinuclear antibody and positivity for anti-Scl-70 antibody. In 7 of the 8 patients, thrombotic microangiopathy developed within one month of starting the glucocorticoid treatment. We should be careful with the use of glucocorticoids in systemic sclerosis patients exhibiting these features in order to avoid cases of normotensive SRC.

View full abstract

A 21-year-old man was admitted to our hospital with a fever, erythema, cervical lymphadenopathy and pancytopenia. A diagnosis of Sweet's syndrome (SS) with Kikuchi's disease (KD) and hemophagocytic syndrome (HPS) was made based on the results of a bone marrow aspiration along with the results from biopsy specimens of the brachial skin and a cervical lymph node. The expression of retinoic acid-inducible gene-I (RIG-I) in the skin epidermal basal layer as well as in a cervical lymph node was revealed through immunohistochemistry. He successfully entered remission through treatment with prednisolone. This findings of this case indicate that when SS with KD presents as HPS, it may suggest an association with an RIG-I-related innate immunity.

View full abstract