[Celiac disease:a case report detailing clinical and pathological improvement with a gluten-free diet].

Celiac disease has a morbidity of 0.2 to 1% in Europe and the United States (US), but appears to be extremely rare in Japan with only a few case reports. This report details a Japanese woman with celiac disease diagnosed by histopathological findings and the improvement of her clinical symptoms on a gluten-free diet. The woman was in her 60s and presented with diarrhea, abdominal pain, and vomiting which were repeatedly exacerbated over several weeks. Therefore, she was referred to our hospital for a detailed examination and treatment. Upper gastrointestinal endoscopy revealed a crude granular mucosa with an erythematous color in the duodenum. No special findings were noted on colonoscopy or capsular endoscopy. Histopathological findings of the duodenum noted villous atrophy, crypt hyperplasia, and lymphocytic infiltration within the surface epithelium, which were suspicious for celiac disease. A gluten-free diet was started which resulted in improved clinical symptoms. Repeat endoscopic imaging and histopathological findings after initiation of the gluten-free diet demonstrated improved small bowel villous atrophy. In this case, appropriate testing had ruled out inflammatory bowel disease, amyloidosis, infectious enteritis, parasitic disease, and allergies. Although anti-gliadin antibody (AGA) and anti-tissue transglutaminase antibody (ATTGA) were serologically negative, and HLA typing was HLA-DQ6, the patient was ultimately diagnosed as having celiac disease based on the characteristic pathological findings and clinical course. In many cases of celiac disease reported in Japan, serum antibodies such as AGA and ATTGA have not been detected, and HLA testing has been negative for DQ2 or DQ8. Therefore, it is possible that celiac disease in Japan might have different genetic and immunological characteristics than the disease in the US and Europe. In the future, additional cases with histology and molecular biological analysis are necessary to test this hypothesis.

Prognostic significance of serum tyrosine concentration in patients with primary biliary cholangitis under ursodeoxycholic acid therapy.

AIM: Chronic liver insufficiency is often associated with alteration in amino acid metabolism. We evaluated the prognostic value of changes in serum amino acid concentrations in patients with primary biliary cholangitis. METHODS: A total of 75 primary biliary cholangitis patients who started urusodeoxycholic acid therapy were retrospectively enrolled. Baseline serum concentrations of branched-chain amino acids and tyrosine, and branched-chain amino acid-to-tyrosine ratio were determined. The hazard ratios of factors associated with liver-related events were analyzed by Cox proportional hazard analysis. RESULTS: Of the 75 patients enrolled, 12 showed a decrease in serum branched-chain amino acid levels, and 15 showed an increase in serum tyrosine levels. The branched-chain amino acid-to-tyrosine ratio decreased in 16 patients. During a median 5.6-year follow up, liver-related events occurred in 11 patients. Multivariate analysis showed that high serum tyrosine levels at baseline and high alkaline phosphatase levels 48 weeks after starting urusodeoxycholic acid therapy were independent risk factors for event occurrence. From the receiver operator characteristics curve analysis, serum tyrosine concentration >110 µmol/L was identified as a cut-off value with an adjusted hazard ratio of 20.9 (95% confidence interval 4.3-101.5, P < 0.001). Kaplan-Meier analysis showed that the 5-year cumulative incidences of event occurrence in patients with high and low serum tyrosine concentration were 56.5% and 5.5%, respectively (P < 0.001). The 10-year survival probabilities also showed significant differences between patients with high and low serum tyrosine concentration (44.9% vs. 92.0%, P < 0.001). CONCLUSION: Elevation of serum tyrosine concentration indicates a high risk of liver-related events in primary biliary cholangitis patients receiving urusodeoxycholic acid therapy.

Elevated serum procalcitonin levels and their association with the prognosis of patients with liver cirrhosis.

OBJECTIVES: Bacterial infection is a major complication in patients with liver cirrhosis. Procalcitonin is an early diagnostic marker of bacterial infection. This study aimed to investigate the association between the serum procalcitonin levels and the prognosis of patients with liver cirrhosis. METHODS: We retrospectively analyzed the serum procalcitonin levels in 236 hospitalized patients with liver cirrhosis. The impact of the serum procalcitonin level on their prognoses was evaluated using multivariate Cox proportional hazards analyses and the Kaplan-Meier method. RESULTS: The serum procalcitonin level was higher (≥0.05 ng/mL) in 151 (64%) patients, and it was significantly higher in the patients with Child-Turcotte-Pugh class C than in those with Child-Turcotte-Pugh classes A/B. Patients with refractory ascites, hepatic encephalopathy, gastrointestinal bleeding, and bacterial infections had elevated serum procalcitonin levels. The multivariate analyses showed a serum procalcitonin level ≥0.05 ng/mL was an independent prognostic factor for liver cirrhosis (hazard ratio = 1.64; 95% confidence interval = 1.07-2.53; P = 0.024). During a median follow-up interval of 2.1 years, the three-year cumulative survival rates for the patients with normal and elevated serum procalcitonin levels were 72.9 and 56.0%, respectively (P < 0.001). The subgroup analyses that stratified the patients according to age, the Child-Turcotte-Pugh classification, and the presence of liver cancer showed the serum procalcitonin level was significantly associated with their prognoses. CONCLUSIONS: The patients with liver cirrhosis had higher serum procalcitonin levels, regardless of local bacterial infections, and higher procalcitonin levels were associated with poor prognoses.

Eosinophilic Gastroenteritis in an Ulcerative Colitis Patient During Treatment with Tumor Necrosis Factor-alpha Antagonist.

A 45-year-old man with steroid-dependent ulcerative pancolitis was hospitalized with frequent diarrhea, abdominal pain and distension 3 months after induction of golimumab, a tumor necrosis factor-alpha antagonist. Computed tomography showed wall thickening from the stomach to the colon and massive ascites. Peripheral blood test revealed eosinophilia. A large number of eosinophils were observed in the ascites fluid. Although esophagogastroduodenoscopy showed no abnormal findings and colonoscopy showed ulcerative colitis with a Mayo endoscopic subscore of 1, eosinophil infiltration was histologically observed. Based on these findings, we diagnosed him with eosinophilic gastroenteritis and started prednisolone. Consequently, his eosinophil counts and abdominal symptoms dramatically improved.