The natural history of the disease varies greatly among individual patients with primary biliary cirrhosis (PBC). Some patients live long without any symptoms while other patients present jaundice and develop hepatic failure in early phases of the disease. Previous studies showed that the natural course of PBC is altered by the use of ursodeoxy cholic acid (UDCA). In this review we discuss variation in the natural course of the disease and it's alteration by UDCA, and risk factors that predict disease progression. Based on clinical observations, there are three types of clinical evolution in PBC: 1) minimal to slow progression over several years; 2) rapid progression to jaundice and hepatic failure, and 3) progression to portal hypertension without developing deep jaundice. Notably, based on our analyses accelerated progression to jaundice and liver failure are reflected by a sustained serologic presence of anti-gp210 antibodies whereas patients with portal hypertension in the absence of jaundice have anti-centromere autoantibodies. These observations highlight the clinical importance of antinuclear antibody analysis in patients with PBC.
Background Cytaphresis (CAP) is an effective modality in the treatment of active ulcerative colitis (UC), but the time lag before a notable clinical response on scheduled therapy frequently causes a significant delay in the modification of treatment. We previously reported that the clinical response after CAP was predicted by early application of transabdominal ultrasound (TAUS), but the predictability of long-term outcome after CAP still remains uncertain. Methods Patients: Twenty-six patients with active UC who received CAP were followed for 1 year. In addition to CAP they received pharmaceutical regimens, such as corticosteroid, 5-aminosalicylic acid, and immunomodulator, as indicated clinically. The mean UC-DAI score was 9.7 before CAP, and 3.2 at 1 year after CAP. Prognostic factor: Total colonic wall thickness was measured by TAUS at 2 to 3 weeks after the initiation of the treatment, and decrement from baseline was calculated. Early ultrasonographic response (EUR) was defined as a decrement statistically. UC-DAI score of 2 or less at 1 year was defined as sustained clinical remission. Score of 6 or more was defined as clinical relapse. Results EUR was defined as a decrement in wall thickness by at least 2.5 mm from the baseline. EUR was noted in 11 patients, and the remaining 15 did not attain EUR. Outcome measures: In the UC-DAI score measured at 1 year after initiation of treatment 90.9% of patients with EUR, whereas 40.0% with non-EUR (p<0.05) showed sustained clinical remission. Regarding relapse, within 1 year 9.1% of patients with EUR relapsed whereas 46.7% with non-EUR (p<0.05) relapsed. Conclusion Early application of TAUS may predict the long-term clinical outcome after CAP in patients with active UC.
Objective We aimed to determine the prevalences of important genetic causes of thromboembolism for the first time in the western Black Sea Region of Turkey. Patients and Methods One hundred and eighty-eight patients diagnosed early with thrombophilia were included in the study. The samples were genotyped using real-time LightCycler. Results Of the 188 patients, 179 (95.2%) had one or more mutations. The frequencies of Factor V (FV) Leiden (FVL, G1691A), FV H1299R (A1299G), Factor II (FII G20210A), methylenetetrahydrofolate reductase (MTHFR) C677T and A1298C were 11.7%, 5.6%, 2.5%, 30.4% and 39.1%, respectively. FV1691A was commonly represented in deep venous thrombosis (34.2%). The highest frequencies of FV1299G and FII20210A were in the vascular headache and deep venous thrombosis groups (10% and 10.5%, respectively). MTHFR677T was common in the pulmonary embolism (37%). MTHFR1298C frequency was 55.9% in recurrent abortus. Within-group comparisons yielded significant differences in the distributions of the FVL and FV H1299R mutations (p=0.002 and p=0.039, respectively). Conclusion There were significant positive associations between venous thromboembolism and FVL and FV H1299R. FVL mutation in DVT may be an important predisposing factor that needs to be tested routinely in this population.
Background Glycated albumin (GA), which is an alternative glycemic marker, is influenced by factors associated with albumin turnover, and it is not clear whether proteinuria influences GA values in diabetic patients with chronic kidney disease (CKD). Methods We enrolled 94 diabetic patients with CKD stages 3 to 5. GA, glycated hemoglobin, and urinary protein excretion (UP) levels were consecutively obtained in each patient. The correlations between GA and UP and those between changes in GA and UP were examined. Results There was a significant correlation between GA and UP in all cases (r=-0.46, p<0.0001), however no significant correlation was found in cases with UP of 0-3.49 g/day (r=0.01). GA values in cases with UP ≥3.5 g/day were significantly lower than those in cases with UP <3.5 g/day [UP ≥3.5 g/day and serum albumin (Alb) ≤3 g/dL; 12.0 ± 1.3%, UP ≥3.5 g/day and Alb >3 g/dL; 17.8 ± 4.3%, 0≥ UP <3.5 g/day; 21.2 ± 4.2%], while no significant difference in HbA1c or glucose levels was found. In cases with a minimum of UP ≥0.5 g/day, no significant correlation was found between the difference in GA and the difference in UP at the point of maximum UP and minimum UP (r=0.04). Conclusion Nephrotic-range proteinuria decreases GA values independent of the glycemic state, while non-nephrotic range proteinuria has no significant influence on GA values in diabetic CKD patients.
Objective Clopidogrel has potent antiplatelet effects, but recent interest has focused on clopidogrel resistance, in which platelet function is not inhibited despite taking the drug. This study evaluated clopidogrel resistance in ischemic stroke patients. Methods After taking oral clopidogrel 75 mg/day for ≥1 week, platelet aggregometry was performed by turbidimetry in all patients, and by a screen filtration pressure method using whole blood in 37 patients. Using turbidimetry, resistance was defined as platelet maximum aggregation rate ≥34% with aggregation-inducing agent ADP 1 μmol/L, or ≥66% with ADP 4 μmol/L. Using the screen filtration pressure method, resistance was defined as a minimum concentration of ≤3 μmol/L ADP to induce secondary aggregation of platelets. Patients This study was conducted in 72 patients (52 men, 20 women; mean age, 69 ± 8 years; range, 50-84 years) with non-cardiogenic ischemic cerebrovascular disease. Results Based on turbidimetry, the rate of clopidogrel resistance was 8.3% with ADP 1 μmol/L and 18.1% with 4 μmol/L. Based on the screen filtration pressure, the rate of clopidogrel resistance was 8.1%. The differences between turbidimetry and screen filtration pressure methods, regarding the measurement of the presence of resistance in the same patient, were observed. Conclusion Clopidogrel resistance varies greatly depending on the method of measuring platelet aggregation and the definition of resistance. Rates of 8-18% were obtained using our methods and criteria.
Objective Tsutsugamushi disease (scrub typhus) is caused by Orientia tsutsugamushi, and has been endemic in Asia and Western Pacific islands. Though sporadic case reports have described the clinical consequences of this vector-borne disease, data on the actual incidence of complications or mortality are scarce. It also remains unclear how a delay in effective treatments affects the occurrence of complications associated with this Rickettsial disease. Methods Using the Japanese Diagnosis Procedure Combination inpatient database in Japan, we identified patients with Tsutsugamushi disease between July 1 and December 31 in 2007 and 2008. We examined location of hospitals, patient's age, sex, comorbidities, complications, inhospital deaths, date of admission, date of starting therapy with tetracyclines. A logistic regression was conducted to analyze the association between delay in effective treatments and the occurrence of complications. Results A total of 210 cases were identified. Overall, 29 (13.8%) had at least one complication and two deaths were identified. Age was a significant risk factor for complications [odds ratio (OR), 1.48; 95% confidence interval (CI), 1.08-2.03; p=0.014, for a 10-year age increase]. Patients with ≥2 days delay in treatment with tetracyclines had a significantly higher risk of complications compared to those with no delay (OR, 2.71; 95% CI, 1.03-7.12; p=0.044). Conclusion Tsutsugamushi disease remains a threat to public health. Our study clearly indicates the importance of early diagnosis and immediate tetracycline treatment to prevent severe complications in Tsutsugamushi disease.
An 18-year-old woman, who had undergone cardiac allograft transplantation, developed continuous back pain two months after surgery. Abdominal computed tomography showed multiple enhanced lesions in her liver, which were not present before transplantation. One tumor bulged from the surface of the liver and compressed the stomach. Partial resection of the liver was performed and her symptoms improved. The pathological diagnosis was focal nodular hyperplasia (FNH). To our knowledge, this is the first report of multiple FNH after heart transplantation. Transplant clinicians may need to keep this possibility under consideration following heart transplantation.
Paraneoplastic nephropathy is a rare complication of malignant disease. We present a case of cervical cancer with biopsy-proven membranous nephropathy and associated nephrotic syndrome. Irradiation to the specific neoplasm site and to the metastatic paraaortic lymph node tissues lead to regression of the nephrotic syndrome without causing severe adverse events. Radiation therapy can be the first choice in the treatment of paraneoplastic nephrotic syndrome if the primary neoplasm is unresectable. Invasiveness of intervention and patient prognosis should be carefully deliberated in the management of the two diseases.
A 46-year-old man presented with fever, thrombocytopenia, and a considerable accumulation of ascites. Although prednisolone and intravenous cyclophosphamide pulse (IVCY) were effective, he experienced recurrence. Renal insufficiency and hemolytic anemia developed subsequently. Schistocytes, negative Coombs' test, and elevated lactate dehydrogenase levels indicated thrombotic microangiopathy (TMA). He recovered well after receiving plasma infusion, IVCY, and prednisolone. Renal biopsy revealed subendothelial widening, thereby confirming TMA. This is a very rare case of TMA that presented with a considerable volume of ascites.
We herein report a case of sinobronchial allergic mycosis (SAM) caused by basidiomycetous (BM) fungi (probably Phanerochaete velutina). The patient with bronchial asthma that accompanied allergic fungal sinusitis (AFS) fulfilled all 6 criteria for diagnosing SAM. In this case, the BM fungus may act as an allergen, reacting continually in both the upper and lower respiratory tract. The antifungal drug (itraconazole 50 mg/day) seemed to achieve a partial response. Basidiomycetous fungi may attract attention because of the possibility as a causative antigen in this new clinical concept of SAM.
A 75-year-old woman became ill suddenly with pneumonia two weeks after operation for colon cancer. Despite intensive support measures in the intensive care unit she died six hours after admission and 12 hours after her first symptom. Autopsy showed necrotizing pneumonia. Aeromonas hydrophila was isolated from a blood culture taken at admission and from the lung at autopsy. In patients who develop a fulminant disease of pneumonia, particularly those who have underlying medical conditions, Aeromonas hydrophila infection, though rare, should be considered.