Intra- and inter-observer agreement in the classification of dyssynergic defecation on high-resolution anorectal manometry using water-perfusion system.

BACKGROUND AND AIMS: Dyssynergic defecation (DD) classified on anorectal manometry is based on variations in anal sphincter pressures and rectal pressure generated during bearing down. There is a paucity of data on intra- and inter-observer agreement in the classification of DD on high-resolution anorectal manometry (HRAM) using a water-perfusion system. METHODS: This cross-sectional observational study was conducted between June 2014 and May 2016 on 70 adult patients with DD. Observer 1 did the baseline analysis and reanalyzed the readings at least 6 months later, blinded to the initial analysis results (intra-observer variability). Four other blinded observers (5-25 years in anorectal manometry and minimum 5 years of experience in HRAM) then individually analyzed the manometry data (inter-observer variability). RESULTS: Intra-observer agreement was excellent (kappa 0.862) for classifying DD. Agreement between the six observations (i.e. two from observer 1 and four from independent observers) was also good (kappa 0.632). The highest agreement was for type 4 DD (kappa 0.738), followed by type 1 (0.680), type 2 (0.664), and type 3 (0.537). Cronbach's alpha value was 0.93, suggesting excellent internal consistency. CONCLUSION: The diagnosis of the types of DD on anorectal manometry using a water-perfusion system has excellent intra-observer and good inter-observer agreement .

Normal Values of High-resolution Anorectal Manometry of Healthy Indians.

Background/Aims: High-resolution anorectal manometry (HRAM) measures anal sphincter function and anorectal co-ordination. This study aims to provide normal data for HRAM and evaluate the effect of gender, age, and body mass index (BMI) on anorectal functions in healthy Indian subjects. Methods: HRAM was performed on 93 healthy volunteers using a 20-channel, water-perfused catheter. We evaluated anorectal pressures, rectal sensation, and balloon expulsion time. Measurements were recorded during rest, squeeze, and simulated defecation (push). Results: Median anal resting pressure (88 mmHg vs 94 mmHg, P = NS), anal squeeze pressure (165 mmHg vs 147 mmHg, P = NS) were not significantly different between males and females. Rectal pressure (70 mmHg vs 54 mmHg, P = 0.024) and anal pressure (82 mmHg vs 63 mmHg, P = 0.008) during simulated evacuation without rectal distention, were higher in males. The threshold for the first sensation was lower in females (40 mL vs 30 mL, P = 0.021) but desire to defecate (105 mL vs 90 mL, P = NS) and maximum tolerable volume (160 mL vs 140 mL, P = NS) were not significantly different in males and females. Anal residual pressure (median mmHg 83 vs 71 mmHg, P = 0.025) was higher in subjects < 40 years of age. Maximum anal squeeze pressure (185 mmHg vs 165 mmHg, P = 0.024) and maximum rectal pressure (75 mmHg vs 62 mmHg, P = 0.032) during push higher in BMI < 23 kg/m2. Conclusions: The present study provides normal data for the Indian population that can be used for comparison and further work. Age, gender, and BMI affect anorectal parameters in HRAM and should be considered while reporting.

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.

Budd-Chiari syndrome has different presentations and disease severity during adolescence.

There are limited data on clinical profile of adolescent patients with Budd-Chiari syndrome (BCS). We studied clinical, radiological, thrombophilia profile and treatment outcomes in adolescent patients with BCS. METHODS: Forty-three consecutive patients of BCS with onset of symptoms during adolescence (10-19 years) were enrolled in the study. 129 randomly selected adult patients with BCS and 36 children with BCS formed the two control groups. The clinical history, physical examination, laboratory tests, thrombophilic disorders, radiological features and treatment outcomes of adolescents were compared to adults and children. RESULTS: In adolescents, ascites (25/43 vs. 110/129, p = 0.0004) and thrombophilic disorders (16/43 vs. 93/129 p < 0.0001) were less frequent than adults. More adolescents (14/43) presented with hepatomegaly alone without ascites than adults (9/129, p < 0.001) or children (1/36, p = 0.005). Adolescents had lower Clichy scores [3.75 (1.2)] than adults [4.72 (1.3), p < 0.0001) or children [4.43 (1.7), p = 0.041]. JAK-2 V617F mutation was the most common thrombophilic disorder in adolescents (5/43) and more common than children (0/36, p = 0.043). Response to therapy was better in adolescents (74.4%) than children (52.8%, p = 0.038), but similar to adults (63.56%, p = 0.13). CONCLUSION: During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.

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.

Cirrhotic cardiomyopathy is less prevalent in patients with Budd-Chiari syndrome than cirrhosis of liver.

BACKGROUND AND AIM: Cirrhotic cardiomyopathy (CCM) is associated with high mortality after transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation in patients with cirrhosis. There is no data about the prevalence or impact of CCM in Budd-Chiari syndrome (BCS). We assessed the prevalence of CCM in patients with BCS and its impact on outcome after radiological intervention. METHODS: Thirty-three consecutive patients with BCS (15 men) and 33 controls with hepatitis B-related cirrhosis (18 men, matched for Child-Pugh score) were evaluated with baseline electrocardiography (ECG), echocardiography (ECHO) and dobutamine stress ECHO, and ECG (DSE). The two groups were compared for prevalence of CCM. Patients with BCS with and without CCM were assessed for development of heart failure, duration of intensive care unit (ICU) stay, and in-hospital mortality immediately after radiological intervention. RESULTS: Fewer patients with BCS had CCM (7/21 vs. 21/33; p = 0.001, OR-0.16, CI [0.05-0.5]), diastolic dysfunction (DD) (0/33 vs. 6/33; p = 0.01, OR-0.06, CI [0.00-1.1]), and prolonged QTc interval (5/33 vs.17/33; p = 0.001, OR-0.16, CI [0.05-0.5]) despite correction for age. Patients with BCS had lower end-systolic and end-diastolic volumes of left and right ventricles. None of the 19 patients (five with CCM) with BCS undergoing radiological intervention (12 TIPS, 4 inferior vena cava, and 3 hepatic vein stenting) developed heart failure or had prolonged ICU stay. There was no in-hospital mortality. CONCLUSION: Patients with BCS have lower frequency of CCM as compared to patients with cirrhosis. CCM may not adversely affect outcomes after radiological interventions.

Ingestion of a carbonated beverage decreases lower esophageal sphincter pressure and increases frequency of transient lower esophageal sphincter relaxation in normal subjects.

Transient lower esophageal sphincter relaxation (tLESR) and decreased basal lower esophageal sphincter (LES) pressure are postulated mechanisms of gastroesophageal reflux (GER). There is conflicting evidence on the effect of carbonated drinks on lower esophageal sphincter function. This study was conducted to assess the effect of a carbonated beverage on tLESR and LES pressure. High resolution manometry tracings (16 channel water-perfused, Trace 1.2, Hebbard, Australia) were obtained in 18 healthy volunteers (6 men) for 30 min each at baseline, and after 200 mL of chilled potable water and 200 mL of chilled carbonated cola drink (Pepsi [Pepsico India Ltd]). The sequence of administration of the drinks was determined by random number method generated by a computer. The analysis of tracings was done using TRACE 1.2 software by a physician who was unaware of the sequence of administration of fluids. The mean (SD) age of the participant was 37.3 (12.9) years. The median (range) frequency of tLESr was higher after the carbonated beverage (10.5 [0-26]) as compared to baseline (0 [0-3], p = 0.005) as well as after water (1 [0-14], p = 0.010). The LES pressure decreased after ingestion of the carbonated beverage (18.5 [11-37] mmHg) compared to baseline (40.5 [25-66] mmHg, p = 0.0001) and after water (34 [15-67] mmHg, p = 0.003). Gastric pressure was not different in the three groups. Ingestion of a carbonated beverage increases tLESr and lowers LES pressure in healthy subjects.