Presence of Ascites at Presentation is Associated With Absence of Long-Term Response Amongst Patients With Budd-Chiari Syndrome When Treated With Medical Therapy Alone: A Single Centre Real-Life Experience.

Background: There is lack of data on long-term outcomes of patients with Budd-Chairi Syndrome (BCS) treated with medical therapy including anticoagulation alone. Methods: Consecutive patients (N = 138, mean [standard deviation, SD] age 29.3 [12.9] years; 66 men) with BCS, treated with medical therapy alone including anticoagulation, with minimum follow-up of 12 months were included. Initial response was classified as complete (CR), partial (PR) or nonresponse (NR) and on follow-up as loss of response (LoR) or maintenance of response (MoR). The association of baseline, clinical and biochemical parameters with different responses was evaluated. Results: Seventy-six patients (55.1%) had CR, 26 (18.8%) had PR and 36 (26.1%) had NR. None with PR or NR had CR later. At a median follow-up of 40 (range 12-174) months, LoR was more common in PR group than in CR group (12 [46.2%] vs 18 [23.7%], P = 0.03). LoR was associated with presence of ascites (odds ratio [OR] 1.5; 95% confidence interval [CI] 0.06-0.71), gastrointestinal bleed (OR 1.33; 95% CI 0.09-0.82) or jaundice (OR 1.01; 95% CI 0.11-0.97) at baseline and duration of follow-up (OR 0.018; 95% CI 1.006-1.030). Mortality was higher in NR (28 [77.8%]) compared with CR (15 [19.7%], P = 0.001) and PR (8 [30.8%], P = 0.001). On binary logistic regression analysis, presence of ascites at baseline was associated with LoR (OR 0.303 [0.098-0.931]). Conclusion: Patients with initial CR have better survival than nonresponders. One-third had LoR on follow-up. The presence of ascites at baseline is associated with LoR.

Patients With Budd-Chiari Syndrome Have Variable Coagulation Status on Thromboelastography at Diagnosis.

BACKGROUND & AIMS: Budd-Chiari Syndrome (BCS) is considered a thrombophilic state, and most patients with BCS have thrombophilic disorder. Liver dysfunction-related coagulopathy makes coagulation function unpredictable in BCS. Thromboelastography (TEG) assesses the dynamics, strength, and stability of clot formation. We conducted a pilot study using TEG to evaluate coagulation status in patients with BCS. METHODS: Fifty-one patients with newly diagnosed BCS (age 32.3 [10.7] years; 23 men) underwent TEG (TEG®5000 Hemostasis Analyzer®, USA), and its components were analyzed and correlated with clinical profile and thrombophilic disorders. Patients who had received anticoagulation, antiplatelet drugs, or radiological intervention were excluded. RESULTS: Twenty-nine patients had normal TEG, 11 had procoagulant TEG, and 11 had hypocoagulant TEG. Among patients with hypocoagulant TEG, Coagulation Index (CI) was < -3 in 11 patients, R was >8 min in 6 patients, K was >3 min in 9 patients, alpha <55 in 9 patients, and MA <51 in 7 patients; among those with hypercoagulant TEG, CI was >3 in 3 patients, R < 2 min in 2 patients, K <1 min in 2 patients, alpha >78 in none, and MA >69 mm in 7 patients. TEG findings were similar in patients with and without thrombophilic disorder. The mean platelet count (1.75, 2.22, and 1.79 × 105/mm3; P = 0.13) and international normalized ratio (1.27, 1.34, and 1.28, P = 0.69) were similar in those with procoagulant, normal, and hypocoagulant TEG. Two patients in Rotterdam class-III had abnormal LY30. Other clinical parameters did not correlate with TEG findings. CONCLUSION: Patients with BCS are heterogeneous with respect to coagulation status, with one-fifth of patients are hypocoagulant on TEG. Patients with advanced disease may have accelerated fibrinolysis.

Fecograph: A graphical representation of daily stool forms to subtype irritable bowel syndrome in office practice.

BACKGROUND AND AIM: Interpreting stool form diaries for subtyping patients with the irritable bowel syndrome (IBS) is cumbersome; a picture showing a trend would be easier to interpret. METHODS: Fifty-one consecutive adults with IBS (median age 35.5 years; 47 men), diagnosed using the Rome III criteria, were given a picture of the Bristol stool form scale (BSFS) and asked to record their stool frequency and form for 7 days. The numbers were plotted by a technician as dots on a chart. On the y axis, BSFS category 4 was marked as 0, harder stools as +1 to +3, and softer stools as -1 to -3; each bowel movement was represented on the x axis. A line graph was plotted by connecting the dots. Each "fecograph" was then given for visual interpretation to three gastroenterologists (observers). When most readings appeared to be 0, +1, or -1, it was to be reported as normal; most above +1 as IBS-constipation (IBS-C); most below -1 as IBS-diarrhea (IBS-D); and readings crossing 1 on either side as IBS-mixed (IBS-M). If no clear trend was noted, it was IBS-unclassified (IBS-U). Each observer reported all graphs in different orders twice, at 1-month intervals; thus, 306 reports were available. Interclass correlation coefficient (ICC) was calculated. RESULTS: Eighteen patients had IBS-C, 13 IBS-D, 4 IBS-M, and 16 IBS-U. The 51 fecographs were reported in mean 20 min 36 s. ICC for intra- and interobserver reliability was 0.62 (0.50-0.73). CONCLUSION: The fecograph is a reliable and easy-to-use tool to subtype patients with IBS.

Findings from a large Asian chronic hepatitis C real-life study.

There is a paucity of information on chronic hepatitis C (CHC) patients treated with direct antiviral agents (DAAs) in Asia. We invited Asia-Pacific physicians to collate databases of patients enrolled for CHC treatment, recording baseline clinical, virologic and biochemical characteristics, sustained virologic response at week 12 (SVR12) and virologic failure. SVR12 outcome was based on intention to treat (ITT). Multivariate analysis was used to assess independent risk factors for SVR12 using SPSS version 20. A total of 2171 patients from India (n = 977), Myanmar (n = 552), Pakistan (n = 406), Thailand (n = 139), Singapore (n = 72) and Malaysia (n = 25) were collected. At baseline, mean age was 49 years, 50.2% were males, and 41.8% had cirrhosis. Overall, SVR12 was 89.5% and by genotype (GT) based on ITT and treatment completion, respectively, was 91% and 92% for GT1, 100% and 100% for GT2, 91% and 97% for GT3, 64% and 95% for GT4, 87% and 87% for GT6 and 79% and 91% for GT untested. Patients with cirrhosis had SVR12 of 85% vs 93% for noncirrhosis (P < 0.001) (RR 2.1, 95% CI 1.4-3.1, P = 0.0002). Patients with GT1 and GT3 treated with sofosbuvir/ribavirin (SR) had 88% and 89% SVR12, respectively, but those GT6 treated with sofosbuvir/ledipasvir (SL) had only 77.6% SVR12. Multivariate analysis showed absence of cirrhosis was associated with higher SVR12 (OR 2.0, 95% CI 1.3-3.1, P = 0.002). In conclusion, patients with GT1 and GT3 with/without cirrhosis had surprisingly high efficacy using SR, suggesting that Asians may respond better to some DAAs. However, poor GT6 response to SL suggests this regimen is suboptimal for this genotype.

Pregnancy outcomes in women with Budd Chiari Syndrome before onset of symptoms and after treatment.

BACKGROUND & AIMS: Pregnancy is known to have poor outcomes in women with Budd Chiari syndrome. There are no data on fertility and pregnancy outcomes prior to onset of symptoms or the effect of therapy on these parameters. We therefore evaluated reproductive profile of women with Budd Chiari Syndrome before the onset of symptoms and after therapy. PATIENTS AND METHODS: Eighty women with Budd Chiari Syndrome (29 years [20-45]) were enrolled over 8 years. Baseline demographic characteristics, disease severity, thrombophilic disorders and treatment were reviewed. Their obstetric history before symptoms and after therapy was noted and compared. RESULTS: Sixty women conceived at least once (150 pregnancies) before symptom onset and 20 had primary infertility. Eighty-one pregnancies resulted in live births and remaining 69 pregnancies had adverse pregnancy outcomes. Post-intervention, 28 women (15 with primary infertility) attempted conception. Thirteen patients conceived 15 times. More women had live births after successful therapy as compared to presymptomatic period (0/28 vs 5/28 P = .000, Odds ratio5.6; 95% CI: 2.16-14.5). In women with primary infertility, conception (0/15 vs 3/15 P = .007, Odds ratio 5, 95% CI: 1.44-17.27) and proportion of live births (0/15 vs 2/15 P = .002, Odds ratio 7.5, 95% CI: 1.71-32.79) was higher as compared to presymptomatic period. CONCLUSION: Primary infertility is common and pregnancy outcomes are poor before the onset of symptoms in women with Budd-Chiari syndrome. Effective therapy of Budd-Chiari syndrome may improve fertility and pregnancy outcomes.

Long Term Outcomes of Patients with Significant Biliary Obstruction Due to Portal Cavernoma Cholangiopathy and Extra-Hepatic Portal Vein Obstruction (EHPVO) With No Shuntable Veins.

AIMS: The natural history of portal cavernoma cholangiopathy (PCC) in patients with significant biliary obstruction (SBO) who cannot undergo shunt surgery, is not known. We therefore, analyzed data of patients of extra-hepatic portal venous obstruction (EHPVO) with PCC. METHODS: Prospectively recorded details of 620 (age 21.2 [11.4] years; 400 [65%] males) patients with primary EHPVO were reviewed. Outcomes (hepatic decompensation/mortality) of patients with PCC and SBO without shuntable veins were noted at follow up of 7 [4-11] years. RESULTS: Ninety-seven of 620 (15.6% [60 men]) EHPVO patients had PCC-SBO. Of these 57 did not have shuntable veins. The median duration from any index symptom to symptomatic PCC was 7 (0-24) years and from index bleed to symptomatic PCC was and 12 (5-24) years, respectively. Thirteen patients underwent endoscopic retrograde cholangiography; nine repeatedly over 7 (4-10) years. Decompensation was seen in 5 patients. Presentation other than variceal bleed was associated with hepatic decompensation (5/19 versus 0/38, P = 0.003). CONCLUSIONS: Majority of patients with PCC-SBO do not have shuntable veins, and may have good long-term outcomes. Patients presenting with variceal bleed have low chance of decompensation. Symptomatic PCC appears to be a late event in EHPVO.

Simple biochemical parameters and a novel score correlate with absence of fibrosis in patients with nonalcoholic fatty liver disease.

BACKGROUND/AIMS: Liver biopsy is the gold standard for detecting fibrosis in patients with nonalcoholic fatty liver disease (NAFLD). Due to limitations of biopsy, various combinations of serum markers have been studied to predict liver fibrosis; many of these are patented and expensive, thereby restricting their evaluation. We prospectively evaluated the correlation of commonly used serum markers with fibrosis in Indian patients with NAFLD. METHODS: Fifty-one patients (age 50.4 [SD 11.5] years) with biopsy-proven NAFLD underwent estimation of platelet count, total bilirubin, AST, ALT, serum albumin, γ-glutamyl transpeptidase (GGT), prothrombin time, serum cholesterol, triglycerides, α2-macroglobulin (A2M), apolipoprotein A1 (Apo A1), and haptoglobin. FIB-4, AST/platelet ratio index (APRI), and AST/ALT ratio were calculated and correlated with fibrosis (NAS-II score) on liver biopsy. RESULTS: Thirty-eight (74.5 %) patients had inflammation and 48 (94.1 %) had ballooning degeneration on histology; 29 had fibrosis, of whom 11 had ≥F2 fibrosis. High GGT (odds ratio [OR] 8.4 [1.85-38.10]; p = 0.007, area under the curve [AUROC] 0.65), low platelet count (OR 7.57 [1.83-31.45]; p = 0.001, AUROC 0.833), and low Apo A1 (OR 12.04 [2.98-47.3]; p = 0.0002, AUROC 0.76) were associated with advanced fibrosis on multiple logistic regression; a novel score formulated by assigning 1 point for an abnormal value for each of these parameters correlated with absence of fibrosis (p = 0.0001; OR 0.102 [95 % confidence interval (CI) CI 0.025-0.418]), with negative predictive value of 94.29 % [95 % CI 80.81 to 99.13]. CONCLUSIONS: A score using simple markers including GGT, Apo A1, and platelet count correlated with absence of liver fibrosis in patients with NAFLD.