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.

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 .