Cumulative risk of developing a new symptom in patients with primary biliary cholangitis and its impact on prognosis.

Background and Aim: Symptoms of primary biliary cholangitis (PBC) frequently impair one's quality of life (QOL). Nonetheless, with improved treatment, the prognosis of PBC also improves. QOL plays an important role in patients with PBC. In this study, we aimed to reevaluate the transition of new symptom development in PBC and its predictive factors. Methods: This retrospective multicenter study enrolled 382 patients with PBC for symptom analysis. The impact of a newly developed symptom on PBC prognosis was investigated by Kaplan-Meier analysis with propensity score matching and logistic progression analysis. Results: The cumulative risk of developing a new symptom after 10 and 20 years of follow-up was 7.6 and 28.2%, and specifically that of pruritus, which was the most common symptom, was 6.7 and 23.3%, respectively. In Cox hazard risk analysis, serum Alb level (hazard ratio [HR], 1.097; 95% confidence interval [CI], 1.033-1.165; P = 0.002), the serum D-Bil level (HR, 6.262; 95% CI, 2.522-15.553, P < 0.001), and Paris II criteria (HR, 0.435; 95% CI, 0.183-1.036; P = 0.037) were significant independent predictors of a new symptom. Kaplan-Meier analysis showed that the overall survival and liver-related death were not significant between patients with and without a new symptom. Conclusion: The cumulative risk of new symptom development is roughly 30% 20 years after diagnosis and could be predicted by factors including serum albumin levels, serum D-Bil level, and Paris II criteria.

Longitudinal increase in albumin-bilirubin score is associated with non-malignancy-related mortality and quality of life in patients with liver cirrhosis.

Due to the developments in the treatment for hepatitis, it is possible to prevent the progression of liver fibrosis and improve patients' prognosis even if it has already led to liver cirrhosis (LC). Consequently, a two-step study was conducted. To begin with, a retrospective study was conducted to identify the potential predictors of non-malignancy-related mortality from LC. Then, we prospectively analyzed the validity of these parameters as well as their association with patients' quality of life. In the retrospective study, 89 cases were included, and the multivariate Cox regression analysis indicated that age (P = 0.012), model for end-stage liver disease (MELD) score (P = 0.012), and annual rate of change of the albumin-bilirubin (ALBI) score (P < 0.001) were significantly associated with LC prognosis. In the prospective study, 70 patients were included, and the patients were divided into cirrhosis progression and non-progression groups. The univariate logistic regression analysis indicated the serum procollagen type III N-terminal peptide level (P = 0.040) and MELD score (P = 0.010) were significantly associated with the annual rate of change of the ALBI score. Furthermore, the mean Chronic Liver Disease Questionnaire score worsened from 5.3 to 4.9 in the cirrhosis progression group (P = 0.034). In conclusion, a longitudinal increase in the ALBI score is closely associated with non-malignancy-related mortality and quality of life.

Jejunal varices after choledochojejunostomy treated with laparotomic transcatheter variceal embolization.

Portal hypertension induces collateral shunt formation between the portal and systemic circulation, decompressing the elevated portal pressure. Ectopic varices outside of the gastroesophageal region, such as jejunal varices, are rare conditions. This report describes the successful embolization of ruptured jejunal varices resulting from an extrahepatic portal obstruction. A 62-year-old man was admitted to our hospital with recurrent massive gastrointestinal bleeding. Fourteen months earlier, he had undergone a choledochojejunostomy and pancreatic cystojejunostomy for bile duct stenosis with an enlarged pancreatic pseudocyst due to severe chronic pancreatitis. Contrast-enhanced computed tomography showed jejunal intramural dilated vessels close to the choledochojejunal anastomosis, but extravasation was not observed. Due to the lack of a rapid definitive diagnosis, the patient required massive blood transfusions. Hemorrhagic scintigraphy using 99mTc-HSAD finally identified the site of the hemorrhage. Angiography and double-balloon endoscopy revealed the anastomotic jejunal varices to be the result of an extrahepatic portal obstruction. Laparotomic transcatheter variceal embolization with microcoils was successful in halting the refractory gastrointestinal bleeding. This surgery preserved hepatopetal portal venous flow by another route, and no complications were observed. At present, 4 years post-surgery, there has been no recurrence of gastrointestinal hemorrhage. The development of jejunal varices is often associated with postoperative adhesions. Some patients with a history of hepatico- or choledochojejunostomy may experience portal hypertension resulting from extrahepatic portal obstruction, leading to the formation of jejunal varices as hepatopetal portal collaterals. The choice of therapy in each patient should be based on the individual hemodynamics of the ectopic varices.

Simultaneous combined balloon-occluded retrograde transvenous obliteration and partial splenic embolization for gastric fundal varices.

BACKGROUND: We previously reported the techniques and usefulness of simultaneous combined balloon-occluded retrograde transvenous obliteration (B-RTO) and partial splenic embolization (PSE), based on the hypothesis that concomitant PSE can diminish the increase in portal venous pressure after B-RTO. OBJECTIVE: After experiencing more cases and performing longer-term follow-up, we re-evaluated the efficacy of simultaneous combined B-RTO and PSE for gastric fundal varices (GVs). METHODS: We performed B-RTO in 36 consecutive patients treated for GVs from 2005 to 2013. Twenty-three patients underwent simultaneous combined B-RTO and PSE (Group 1) and 13 underwent B-RTO monotherapy (Group 2). The outcomes were retrospectively evaluated. RESULTS: There were no significant differences in baseline characteristics between the two groups except that the splenic volumes were larger in Group 1 than 2. B-RTO was technically successful in 21 of 23 patients (91.3%) in Group 1 and in 12 of 13 patients (92.3%) in Group 2. In all patients with ruptured GVs (six in Group 1 and five in Group 2), complete hemostasis was obtained by B-RTO. Exacerbation of esophageal varices was significantly less frequent in Group 1 than 2 (p = 0.0017). CONCLUSION: Concomitant PSE with B-RTO may contribute to prevention of the exacerbation of esophageal varices after B-RTO.

[A case of malignant peritoneal mesothelioma successfully treated with systemic chemothrapy].

A 64-year-old man with a 2-month history of abdominal distension was admitted for transient cerebral ischemic attack. A CT scan revealed massive ascites. Laparoscopy showed multiple whitish nodules on the visceral peritoneum and the omentum. Peritoneal biopsy revealed tumor cells consistent with malignant peritoneal mesothelioma (MPeM). Pemetrexed in combination with cisplatin was administered because it has been reported to be active in patients with MPeM. However his disease progressed. As second-line therapy paclitaxel was tried which yielded a complete response (CR). Eighteen months later he developed abdominal pain of the right upper region where a CT scan showed a mass with surrounding inflammation. As third-line therapy, gemcitabine was administered and again resulted in a CR. He is alive at 3 years from first presenting. Searches for case studies published in medical journals on MPeM were carried out, and 59 cases were analyzed in comparison with this case.

[A case of gastric cancer with abdominal wall invasion treated by weekly low-dose paclitaxel therapy].

Here we report a case of gastric cancer with diffuse abdominal wall invasion treated with weekly low-dose paclitaxel therapy. A 62-year-old male visited our hospital because of abdominal distention, prepubic tumor,and testicular hydrocele. Computed tomography revealed diffuse swelling of the abdominal wall and hydronephrosis of the right kidney. Upper gastrointestinal endoscopy demonstrated type 3' advanced gastric cancer. Pathological diagnosis of both gastric tumor and abdominal wall biopsy specimens was poorly-differentiated adenocarcinoma containing signet ring cell carcinoma. Low-dose paclitaxel (90 mg/body) was given once a week for 3 weeks. Abdominal wall swelling like cuirass disappeared after 2 courses of low-dose paclitaxel therapy. Nine repeated courses of this regimen have been given until now; the relapse of the abdominal wall invasion has not become apparent, and primary gastric lesion has been a stable disease. Diffuse abdominal wall invasion of gastric cancer like cuirass without ascites is a rare condition, and low-dose paclitaxel was very effective for this condition.