Clinical impact of recombinant thrombomodulin administration on disseminated intravascular coagulation due to severe acute cholangitis (Recover-AC study).

BACKGROUND AND AIM: Recombinant thrombomodulin (rhTM) is potentially effective in the treatment of disseminated intravascular coagulation (DIC). Several studies related to drugs for the treatment of acute cholangitis have shown negative results in improvement of overall survival (OS) with rhTM. The aim of this multicenter study was to evaluate the clinical effectiveness of rhTM in patients with acute cholangitis and sepsis-induced DIC who underwent biliary drainage. METHODS: A total of 284 consecutive patients, who were complicated with sepsis-induced DIC due to severe acute cholangitis, were included (rhTM group, n = 173; non-rhTM, n = 111) in this study. The primary outcome was the DIC resolution rate at 7 days after starting treatment. The 28-day survival rate was secondarily evaluated. RESULTS: DIC scores in the rhTM group improved significantly compared with the non-rhTM group on day 7 (P = .020). According to multivariate analysis, etiology of cholangitis (malignant, HR 2.28), rhTM (non-administration, HR 4.13), and DIC score (≥5, HR 2.46) were significant factors associated with failed DIC resolution on day 7. Propensity score matching created 103 matched pairs. Survival rate at day 28 was significantly higher in rhTM group (94.3%) compared with non-rhTM group (82.6%; P = .048) after propensity score matching. rhTM (non-administration, HR 2.870), DIC score (≥5, HR 2.751), and APACHE II score (≥20, HR 9.310) were significant factors associated with decreasing survival rate at day 28. CONCLUSION: In conclusion, rhTM seemed to improve patient survival, but future studies should only include patients with benign or malignant disease and should be performed according to APACHE II scores.

Correlations of Hepatic Hemodynamics, Liver Function, and Fibrosis Markers in Nonalcoholic Fatty Liver Disease: Comparison with Chronic Hepatitis Related to Hepatitis C Virus.

The progression of chronic liver disease differs by etiology. The aim of this study was to elucidate the difference in disease progression between chronic hepatitis C (CHC) and nonalcoholic fatty liver disease (NAFLD) by means of fibrosis markers, liver function, and hepatic tissue blood flow (TBF). Xenon computed tomography (Xe-CT) was performed in 139 patients with NAFLD and 152 patients with CHC (including liver cirrhosis (LC)). The cutoff values for fibrosis markers were compared between NAFLD and CHC, and correlations between hepatic TBF and liver function tests were examined at each fibrosis stage. The cutoff values for detection of the advanced fibrosis stage were lower in NAFLD than in CHC. Although portal venous TBF (PVTBF) correlated with liver function tests, PVTBF in initial LC caused by nonalcoholic steatohepatitis (NASH-LC) was significantly lower than that in hepatitis C virus (C-LC) (p = 0.014). Conversely, the liver function tests in NASH-LC were higher than those in C-LC (p < 0.05). It is important to recognize the difference between NAFLD and CHC. We concluded that changes in hepatic blood flow occurred during the earliest stage of hepatic fibrosis in patients with NAFLD; therefore, patients with NAFLD need to be followed carefully.

[Mixed adenoneuroendocrine carcinoma of the gallbladder diagnosed by endoscopic transpapillary catheterization using bile cytology assessed by the cell block method].

A 65-year-old woman was suspected of having advanced gallbladder cancer based on imaging results. This was considered inoperable because it was accompanied by possible liver metastasis. To confirm the diagnosis prior to chemotherapy, endoscopic transpapillary catheterization in the gallbladder (ETCG) was performed. The bile cytology was analyzed by the cell block method, which revealed mixed adenoneuroendocrine carcinoma. Based on the cytological results, extended cholecystectomy and partial hepatectomy were performed for the metastatic lesions. Bile cytology by ETCG with the cell block method was useful for deciding the therapeutic strategy in this patient with metastatic gallbladder cancer.

Invasive carcinoma derived from "flat type" branch duct intraductal papillary mucinous neoplasms of the pancreas: impact of classification according to the height of mural nodule on endoscopic ultrasonography.

BACKGROUND: It has been reported that many branch duct intraductal papillary mucinous neoplasms (BD-IPMN) with a mural nodule (MN) reveal adenocarcinomas. On the other hand, invasive cancer derived from BD-IPMN without MN on endoscopic ultrasound (EUS) also exists. The aim of this study was to elucidate the clinicopathological features of invasive cancer derived from BD-IPMN without MN on EUS. METHODS: Twenty-one patients pathologically diagnosed with invasive cancer derived from BD-IPMN were included in this study. RESULTS: Based on the height of MNs on EUS, the subjects could be clearly classified as 12 patients whose background BD-IPMNs had high MNs (nodule-forming type IPMN) and nine whose background BD-IPMNs showed no MNs (flat type IPMN). The background BD-IPMN of the 12 patients with nodule-forming type IPMN were non-gastric type. On the other hand, the background BD-IPMN of the nine patients with flat type IPMN was gastric type. The recurrence rate was higher (33% vs. 67%) and the 5-year survival was worse (76% vs. 33%) in flat type IPMN. CONCLUSIONS: There may be a pathway for the development of invasive cancer without the formation of an MN in BD-IPMN, and attention should be paid even to the patients with BD-IPMN which does not present an MN.

[A case of primary Sjögren's syndrome complicated by chronic progressive myositis].

The patient was a 64-year-old woman with a nearly 20-year history of sicca symptoms, having been given a diagnosis of primary Sjögren's syndrome. Three years previously, she experienced difficulty in walking up a slope and had leg malaise, which insidiously progressed to an inability to go up and down stairs. This disability brought her to our hospital, where her muscle strength was examined by manual muscle testing, and she was found to have reduced muscle strength in proximal muscles like the thigh muscles and the neck flexor muscles. Blood studies revealed elevated ESR, increased serum IgG, mildly increased myogenic enzymes, and positive results for anti-SS-A and -SS-B antibodies. MRI scans disclosed extensive muscle atrophy as well as fatty degeneration in the thigh. A biopsy of the quadriceps femoris muscle provided a diagnosis of myositis based on the finding of muscle fibers of unequal size, nuclear centralization, and inflammatory cell infiltration into muscle fibers. CD4-positive lymphocytes were the predominant inflammatory cells. We diagnosed the case as myositis in primary Sjögren's syndrome based on the clinical course and laboratory findings. She recovered well with steroid medication. It is noteworthy that myositis associated with primary Sjögren's syndrome presents with mild symptoms and unremarkable laboratory data but may run a chronic progressive course.