Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome

We report a patient with idiopathic portal hypertension (IPH) associated with systemic sclerosis (SSc) and Sjögrens syndrome. A 72-year-old Japanese woman was admitted to our hospital because of Raynauds phenomenon, sclerodactyly, and dyspnea. The patient had splenomegaly, esophageal varices in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Immunological studies revealed positive anti-nuclear antibodies and high titers of anti-Scl-70, anti-SS-A, anti-centromere, and anti-mitochondrial M2 antibodies. Histological examinations of the liver biopsy specimen revealed stenosis and loss of small portal veins without findings of primary biliary cirrhosis. The patient was diagnosed as having IPH associated with SSc and Sjögrens syndrome. These observations suggest an immunological role in the pathogenesis of IPH.     

Tumor necrosis factor suppression and microcirculatory disturbance amelioration in ischemia/reperfusion injury of rat liver after ischemic preconditioning

BACKGROUND: Brief periods of hepatic ischemia produce immediate tolerance for subsequent prolonged ischemia. Although the beneficial effect of this ischemic preconditioning is recognized, the mechanism itself remains poorly understood. METHODS: Male Wistar rats were divided into two groups: a control group that was subjected to 30 min of ischemia + following reperfusion, and an ischemic preconditioning group that was subjected to 5 min of ischemia + 5 min of reperfusion + 30 min of ischemia + following reperfusion. By using this model, hepatic damage, microcirculatory disturbances, and tumor necrosis factor-alpha protein production and mRNA expression were analyzed during the course of reperfusion in both groups. For the hepatic damage evaluations, hepatic enzyme levels, histology, apoptosis analysis, and intravital microfluorography for dead cells were examined. For the microcirculatory disturbance analysis, an adhesion molecule and intravital microfluorography for endothelial-adherent leukocytes were examined. RESULTS: In the ischemic preconditioning group, ischemia/reperfusion injuries (shown by hepatic enzymes elevation, histological degeneration, and increases in the number of apoptotic cells and microfluorographic dead cells) were markedly reduced. Moreover, microcirculatory disturbances represented by intercellular adhesion molecule-1 expression and leukocyte adhesion on the endothelium were ameliorated. Tumor necrosis factor-alpha protein production and mRNA expression were also suppressed in the ischemic preconditioning group. CONCLUSION: The suppression of tumor necrosis factor-alpha and the subsequent amelioration of microcirculatory disturbances were observed, suggesting that the mechanism underlying the protective effect of ischemic preconditioning in hepatic ischemia/reperfusion injuries may involve tumor necrosis factor-alpha and microcirculatory regulation.     

Histological analysis of the papilla after endoscopic papillary balloon dilation

BACKGROUND/AIMS: Endoscopic papillary balloon dilation is an effective procedure in the management of bile duct stones and is believed to preserve the papillary function. The papillary architecture is also considered to be preserved. However, little is known about the effects of balloon dilation on papillary structure. The present study was conducted to elucidate these effects. METHODOLOGY: Since May 1994, endoscopic papillary balloon dilation was performed in 467 patients (407 patients for removal of bile duct stone, 57 for insertion of stent and 3 for baby cholangioscope). Of those, histological examinations were undertaken in 10 patients. The indications of endoscopic papillary balloon dilation were bile duct stone removal in 2 patients, stent insertion in 8. The specimens were obtained 2 to 63 weeks after endoscopic papillary balloon dilation during surgical operation in 6 patients and autopsy in 4 patients. Serial sections through the papilla were stained by hematoxylin and eosin and Masson's trichrome. The specimens were also obtained from 4 patients without biliary disease as control. RESULTS: No smooth muscle disruption nor architectural distortion was observed except for one patient who had mild disruption of smooth muscle caused by a biliary drainage tube. However, mild to moderate inflammation and fibrosis were seen in 9 patients and in 8, respectively. CONCLUSIONS: The papillary architecture is not affected by endoscopic papillary balloon dilation. This may imply that endoscopic papillary balloon dilation preserves papillary function.     

CD56/NCAM-Positive Langerhans Cell Sarcoma: A Clinicopathologic Study of 4 Cases

This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive Langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100beta, and langerin, the presence of which suggests derivation from Langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.     

Burden of disease in bone and joint diseases

In order to evaluate the burden of disease in bone and joint diseases, DALY (Disability-adjusted Life Year) values were calculated by using existing medical database. DALY value of rheumatoid arthritis was calculated to 76,038, and the value of arthritis was calculated to 86,783. The sum of value of DALY of these diseases is about half of diabetes mellitus and very large.     

Utility of T-wave alternans during night time as a predictor for ventricular fibrillation in patients with Brugada syndrome

The prognostic value of T-wave alternans (TWA) during the night time in patients with Brugada syndrome (Br-S) remains unknown. We assessed TWA for risk stratification using 24-h multichannel Holter electrocardiogram (24-M-ECG) in Br-S. We enrolled 129 patients with Br-S [grouped according to histories of ventricular fibrillation (VF), n = 16; syncope, n = 10; or no symptoms (asymptomatic), n = 103] and 11 controls. Precordial electrodes were attached to the third (3L-V1, 3L-V2) and fourth (4L-V1, 4L-V2 and 4L-V5) intercostal spaces. We measured the values of maximum TWA (max-TWA) during the night time (12 a.m.–6 a.m.) and the day time (12 p.m.–6 p.m.) and calculated parameters of heart rate variability. Compared to the asymptomatic and control groups, the VF and syncope groups showed significantly greater 3L-V2 max-TWA during the night time. The cutoff value for the 3L-V2 max-TWA during the night time was determined as 20 µV (sensitivity 94 % and specificity 48 %; p = 0.01). Multivariate analysis revealed that 3L-V2 max-TWA during the night time ≥20 µV and previous VF episodes were independent predictors of future VF episodes. During a mean follow-up period of 68 ± 37 months, 16 patients experienced VF episodes. The incidence of VF episodes was the highest during the night time (p < 0.001). The 3L-V2 max-TWA during the night time may be a useful predictor for VF episodes in patients with Br-S.     

Beta-blocker therapy induces ventricular resynchronization in dilated cardiomyopathy with narrow QRS complex.

OBJECTIVES: We sought to evaluate the effects of beta-blocker therapy on regional and global myocardial mechanics in addition to ventricular synchrony in patients with heart failure and normal QRS by using tissue Doppler and strain echocardiography. BACKGROUND: It is unknown whether beta-blocker therapy can influence mechanical synchrony. METHODS: Conventional and strain echocardiography were performed in 15 healthy age-matched volunteers and in 25 patients with idiopathic dilated cardiomyopathy (IDC). Of these, 15 IDC patients on standard heart failure therapy were studied prior to and at 1 and 6 months after initiation of carvedilol therapy and compared to the controls. RESULTS: There was significant mechanical dyssynchrony in IDC compared with control patients. Patients placed on carvedilol demonstrated a significant decrease in the inferoseptal to lateral wall delay in peak strain (normalized to the R-R interval) between baseline and 1 month and between baseline and 6 months. Similarly, global time to peak segmental strain (455 +/- 51 ms vs. 423 +/- 59 ms, respectively, p = 0.02, and 455 +/- 51 ms vs. 415 +/- 50 ms, respectively, p = 0.01) and the coefficient of variation of the time to peak segmental strain decreased (17 +/- 4% vs. 15 +/- 5%, respectively, p = 0.02, and 17 +/- 4% vs. 14 +/- 5%, respectively, p = 0.03), from baseline to 1 month and between baseline and 6 months, respectively. Global strain significantly increased from baseline to 1 month (-8.2 +/- 1.8 to -10.4 +/- 3.9, respectively, p = 0.01) and between baseline and 6 months (-8.2 +/- 1.8% to -12.0 +/- 3.2%, respectively, p = 0.008). Improvements in left ventricular ejection fraction and reverse remodeling were coincident with reductions in mechanical dyssynchrony. CONCLUSIONS: The use of carvedilol improves contractile function and dyssynchrony in heart failure patients with normal QRS.