A rare case of polyarthritis panniculitis and pancreatitis.

Extra pancreatic manifestations of pancreatitis are rare, with a prevalence of 2-3%. One such rare manifestation is the triad of joint pain (polyarthritis), tender skin lesions (panniculitis), and pancreatic inflammation (pancreatitis), known as PPP. The pathogenesis of this phenomenon is not fully understood but is believed to involve lipolysis by pancreatic enzymes at lipid-rich skin and joint sites. PPP primarily affects middle-aged males with a history of alcohol use disorder. Diagnosis can be challenging due to the absence of typical abdominal symptoms. Delayed diagnosis may significantly worsen outcomes. Supportive therapy is the mainstay, but resolution requires addressing the underlying pancreatic abnormality. We present a case of a patient with a history of alcohol use disorder and recurrent acute pancreatitis who developed joint pain and skin rash. Extensive work-up ruled out other causes, and imaging and biopsy confirmed the diagnosis of PPP. Symptomatic management and treatment of the underlying pancreatic abnormality led to complete resolution of symptoms. Our case serves to raise awareness of this rare but potentially fatal syndrome.

A pilot randomized controlled trial of an online intervention for Hodgkin lymphoma survivors to increase knowledge about late effects and recommended screening.

BACKGROUND: Hodgkin lymphoma (HL) survivors who received chest radiotherapy are at risk for breast cancer and cardiovascular disease, but screening adherence is low. We assessed the acceptability/feasibility of a web-based educational intervention and its impact on knowledge of health risks and screening. METHODS: HL survivors were randomized to either an interactive online educational intervention or handouts only. Surveys were completed at baseline and 3 months post-intervention. We described the acceptability/feasibility of the intervention and compared knowledge between groups. RESULTS: Fifty-two HL survivors participated; 27 in the intervention group and 25 in the control group. Eighteen (66%) intervention participants completed the intervention and reported high acceptability (89-100%). At baseline, adherence to breast cancer screening was low across all participants. Post-intervention, those in the intervention group more often than controls correctly identified breast cancer and echocardiogram screening guidelines (35% vs. 28%, P = 0.02 and 82% vs. 52%, P = 0.04) and reported knowing how to address potential complications from cancer treatments (87% vs. 64%, P = 0.03). We detected no increase in screening behavior post-intervention. CONCLUSION: Online education modules for high-risk HL survivors are an acceptable method to improve knowledge of health risks and screening guidelines. Future interventions should focus on improving screening uptake in this population. IMPLICATIONS FOR CANCER SURVIVORS: Web-based learning can be useful in increasing cancer survivor knowledge of their unique risks and screening recommendations but does not necessarily change patient behavior. Involvement in a cancer survivorship program can help assess individual barriers and monitor uptake of screening.

Modified triple pelvic osteotomy for residual acetabular dysplasia through double incisions: Technical note and review of short-term results.

PURPOSE: To assess validity, safety, and efficacy of the modified triple pelvic osteotomy (TPO) approach for correction of residual acetabular dysplasia. METHODS: This is a retrospective case series conducted on 15 hips in 15 patients from 2019 to 2023 with residual acetabular dysplasia treated by modified TPO as described by Tonnis with two modifications. The first modification is using a single medial incision for pubic and ischial cuts (the Vladimirov modification). The second modification is having the ischial cut closer to the acetabulum (Li modification) allowing free movement of the acetabular fragment for better femoral head coverage. The mean age at the time of surgery was 11.85 years, (range 8-23). Cases presenting were 10 males (66.7%) and 5 females (33.3%). The mean follow-up period was 36.533 months (24-60 months). RESULTS: Our study revealed significant clinical and radiological improvement. The CE angle improved from a mean value of 10° (range 2-17) pre-operatively to 32.785° (range 18°-40°) post-operatively. The AI improved from a mean value of 32° pre-operatively to a mean value of 13.89° post-operatively. HHS increased from a preoperative mean value of 74.80° to a post-operative mean value of 90.67°. Also, there was a significant improvement in ROM (abduction and internal rotation). LLD improved from a mean value of 2.60 cm preoperatively to a mean value of 0.37 cm postoperatively. Delayed union was found in 3 cases. No cases of osteonecrosis or neurovascular complication were encountered in our study. CONCLUSION: The modified TPO technique using dual incisions can be considered safe and effective, providing adequate coverage of the femoral head in acetabular dysplasia with less surgical time, satisfactory functional outcomes, and minimal complications. LEVEL OF EVIDENCE: IV.

Utility of apical four-chamber longitudinal strain in the assessment of childhood cancer survivors: A multicenter study.

BACKGROUND: A previous multicenter study showed that longitudinal changes in standard cardiac functional parameters were associated with the development of cardiomyopathy in childhood cancer survivors (CCS). Evaluation of the relationship between global longitudinal strain (GLS) changes and cardiomyopathy risk was limited, largely due to lack of quality apical 2- and 3-chamber views in addition to 4-chamber view. We sought to determine whether apical 4-chamber longitudinal strain (A4LS) alone can serve as a suitable surrogate for GLS in this population. METHODS: A4LS and GLS were measured in echocardiograms with acceptable apical 2-, 3-, and 4-chamber views. Correlation was evaluated using Pearson and Spearman coefficients, and agreement was evaluated with Bland-Altman plots. The ability of A4LS to identify normal and abnormal values compared to GLS as the reference was evaluated. RESULTS: Among a total of 632 reviewed echocardiograms, we identified 130 echocardiograms from 56 patients with adequate views (38% female; mean age at cancer diagnosis 8.3 years; mean follow-up 9.4 years). Correlation coefficients between A4LS and GLS were .89 (Pearson) and .85 (Spearman), with Bland-Altman plot of GLS-A4LS showing a mean difference of -.71 ± 1.8. Compared with GLS as the gold standard, A4LS had a sensitivity of 86% (95% CI 79%-93%) and specificity of 82% (69%-95%) when using normal range cutoffs and 90% (82%-97%) and 70% (58%-81%) when using ±2 standard deviations. CONCLUSION: A4LS performs well when compared with GLS in this population. Given the more recent adoption of apical 2- and 3-chamber views in most pediatric echocardiography laboratories, A4LS is a reasonable stand-alone measurement in retrospective analyses of older study cohorts and echocardiogram biorepositories.

Liver transplantation for refractory hepatic hydrothorax is associated with a greater survival benefit compared to other complications of cirrhosis.

Hepatic hydrothorax (HH) is a significant complication of cirrhosis associated with increased mortality. Liver transplantation (LT) remains the best treatment modality. We aim to assess predictors of mortality and the survival benefit of LT in patients with HH. A prospectively maintained cohort of adult patients with cirrhosis, being evaluated for LT at our institution, was retrospectively reviewed from 2015 to 2020. The primary outcome was death or LT. Cox proportional hazard regression identified associations between covariates and death. We calculated the years saved due to LT by comparing patients who were on the waiting list with patients who received an LT. This was done by calculating the area under the Kaplan-Meier curve. Censoring occurred at the time of the last follow-up or death. Patients with refractory HH had the lowest median survival of only 0.26 years. Within the HH group, having a refractory HH group was significantly associated with an increased risk of mortality (HR 1.73; 95% CI 1.06-2.81; p -value 0.03). Refractory HH was also significantly associated with mortality when evaluated in the entire cohort and after adjusting for other covariates (HR 1.48, 95% CI 1.03-2.11; p -value 0.03). Patients with refractory HH had the highest 1-year survival benefit with LT (0.48 y), followed by patients with non-refractory HH (0.28 y), then patients with other complications of cirrhosis (0.19 y). In this large study evaluating the prognostic impact of HH on patients with cirrhosis, refractory HH was an independent predictor of mortality. LT provides an additional survival benefit to patients with HH compared with those without HH.

Adequate versus deep response to ursodeoxycholic acid in primary biliary cholangitis: To what extent and under what conditions is normal alkaline phosphatase level associated with complication-free survival gain?

BACKGROUND AND AIMS: Normal alkaline phosphatase (ALP) levels in ursodeoxycholic acid (UDCA)-treated patients with primary biliary cholangitis (PBC) are associated with better long-term outcome. However, second-line therapies are currently recommended only when ALP levels remain above 1.5 times the upper limit of normal (×ULN) after 12-month UDCA. We assessed whether, in patients considered good responders to UDCA, normal ALP levels were associated with significant survival gains. APPROACH AND RESULTS: We performed a retrospective cohort study of 1047 patients with PBC who attained an adequate response to UDCA according to Paris-2 criteria. Time to liver-related complications, liver transplantation, or death was assessed using adjusted restricted mean survival time (RMST) analysis. The overall incidence rate of events was 17.0 (95% CI: 13.7-21.1) per 1000 out of 4763.2 patient-years. On the whole population, normal serum ALP values (but not normal gamma-glutamyl transpeptidase (GGT), alanine aminotransferase (ALT), or aspartate aminotransferase (AST); or total bilirubin < 0.6 ×ULN) were associated with a significant absolute complication-free survival gain at 10 years (mean 7.6 months, 95% CI: 2.7 - 12.6 mo.; p = 0.003). In subgroup analysis, this association was significant in patients with a liver stiffness measurement ≥ 10 kPa and/or age ≤ 62 years, with a 10-year absolute complication-free survival gain of 52.8 months (95% CI: 45.7-59.9, p < 0.001) when these 2 conditions were met. CONCLUSIONS: PBC patients with an adequate response to UDCA and persistent ALP elevation between 1.1 and 1.5 ×ULN, particularly those with advanced fibrosis and/or who are sufficiently young, remain at risk of poor outcome. Further therapeutic efforts should be considered for these patients.

Nosocomial spontaneous bacterial peritonitis is associated with high mortality - a systematic review and meta-analysis.

INTRODUCTION: It is unclear if Nosocomial Spontaneous Bacteria Peritonitis (NSBP) is associated with higher mortality compared with community acquired spontaneous bacterial peritonitis. METHODS: Database search from inception to May 2022 was conducted. The databases included MEDLINE, EMBASE, Cochrane registry of Controlled Trials, Cochrane Database of Systematic Reviews, and Scopus. Inclusion criteria were as follows: adult patients, age >18 years, with a diagnosis of NSBP. Pooled estimates of mortality were calculated following the restricted maximum likelihood method. The mortality rate between NSBP and CA-SBP was reported as odds ratio (OR) and 95% confidence interval (CI). Data synthesis was obtained using random effects meta-analysis. Heterogeneity was reported as I2. RESULTS: A total of 482 unique titles were screened. Twenty-two articles were included. A total of 2,145 patients with NSBP were included. Patients were followed for a median of 90 days. The pooled mortality rate of NSBP was 52.51% (95% CI 42.77-62.06%; I2 83.72%). Seven studies compared the mortality outcome of patients with NSBP and CA-SBP. NSBP was significantly associated with a higher rate of mortality (OR 2.78, 95% CI 1.87-4.11; I2 36.00%). CONCLUSION: NSBP was associated with higher mortality rate compared to CA-SBP, which could be due to a higher rate of resistance organisms.

Non-Puerperal Chronic Inversion of Uterus Due to Big Fibroid Uterus in a Post-Menopausal Woman.

Uterine inversion occurs in puerperal and non-puerperal conditions; non-puerperal uterine inversion (NPUI) may run acute and chronic clinical course. Most on the NPUI are chronic variety while a few are acute variety. NPUI occurs if there is long standing big sub-mucosal fibroid and it is very rare to present in acute setting. Here we report a case of acutely presented NPUI. A 58-year-old widow of lower socioeconomic status presenting to the emergency center of Chittagong medical college Hospital with complaints of sudden protrusion of a big mass through introitus in an attempt of passing out hard stool during defecation on the day of admission with a history of per vaginal watery discharge for a long time and severe anemia. Anemia was corrected and a broad-spectrum antibiotic was given prior to operative management. Under general anesthesia vaginal myomectomy followed by vaginal hysterectomy was performed in the same sitting. Pathological examination revealed a fibroid uterus. Postoperatively patient recovered without any residual problem. Infection should be suspected and treated with appropriate broad-spectrum antibiotics before planning surgery. Vaginal route restoration of NPUI is very difficult but possible with careful attempt. During a vaginal hysterectomy, care to locate and salvage the bladder and distal urinary collecting system is warranted. So, a high index of suspicion is the key to limit morbidity and approach for proper management of such rare clinical condition.

Acute idiopathic pancreatitis is associated with more aggressive disease course in Crohn's disease but not in ulcerative colitis.

PURPOSE: Patients with inflammatory bowel disease (IBD), whether Crohn's disease (CD) or ulcerative colitis (UC), have an increased risk of acute pancreatitis. The prognostic value of diagnosing acute idiopathic pancreatitis in patients with IBD is not well understood. METHODS: A retrospective review of 56 patients with IBD and acute pancreatitis was conducted in a tertiary center from 2011 to 2020. Aggressive disease course was defined as (i)biologic change, (ii)biologic dose escalation, or (iii)IBD-related surgeries occurring within 1 year of acute pancreatitis diagnosis. Logistic regression modelling identified associations between covariates and an aggressive disease course. RESULTS: Baseline characteristics between idiopathic pancreatitis and other causes of acute pancreatitis, in both CD and UC cohorts, were similar. Idiopathic pancreatitis was significantly associated with an aggressive disease course in CD (P = 0.04). No confounding factors were associated with an aggressive disease course in CD. Idiopathic pancreatitis, however, was not associated with an aggressive disease course in UC (P = 0.35). CONCLUSION: The diagnosis of acute idiopathic pancreatitis may provide a prognostic indicator of a more severe disease course in CD. No such association appears to exist with UC. To the best of our knowledge, this is the first study that identifies an association and possible prognostic value between idiopathic pancreatitis and a more severe disease course in CD. More studies with a larger sample size are needed to validate these findings, further define idiopathic pancreatitis as an extraintestinal manifestation of IBD and elucidate a clinical strategy to optimize care in patients with aggressive CD and idiopathic pancreatitis.

Type of donor liver transplant does not affect pregnancy outcomes-a systematic review and meta-analysis.

Liver transplant (LT) has become increasingly common among reproductive-aged women. The effect of the type of liver donor, either a living donor LT (LDLT) or a deceased donor LT, on pregnancy outcomes is unknown. As such, we aim to review the available literature and assess obstetric, pregnancy, or delivery outcomes in LDLT. We conducted a comprehensive literature review of MEDLINE, EMBASE, Cochrane, and Scopus databases. Random-effect meta-regression assessed the association between the percentage of women who underwent LDLT (independent variable) and the proportion of outcomes. Meta-regression results were expressed as a regression coefficient, which transforms the proportion of outcomes of interest associated with a 1% increase in the percentage of LDLT patients. A value of 0 denotes no relationship between the outcomes and LDLT. A total of 6 articles (438 patients) were included, with a total of 806 pregnancies. Eighty-eight (20.09%) patients underwent LDLT. None of the studies segregated the data based on the type of donor LT. The median time from LT to pregnancy was 4.86 (4.62-5.03) years. Twelve (1.5%) stillbirths were reported. LDLT was statistically significantly associated with a higher rate of stillbirths (coefficient 0.002, p < 0.001; I 2 0%). The type of donor LT was not associated with an increased risk of other obstetric, pregnancy, or delivery complications. This is the first meta-analysis to evaluate the effect of the type of donor LT on pregnancy outcomes. This study highlights the lack of robust literature addressing this important topic. The results suggest that pregnancy outcomes after LDLT and deceased donor LT are comparable. Despite LDLT being statistically significantly associated with a higher rate of stillbirths, the association is weak and is unlikely to be clinically significant.

Transition practices for survivors of childhood cancer: a report from the Children's Oncology Group.

PURPOSE: Pediatric healthcare systems must support childhood cancer survivors to optimize their transition to adult care. This study aimed to assess the state of healthcare transition services provided by Children's Oncology Group (COG) institutions. METHODS: A 190-question online survey was distributed to 209 COG institutions to assess survivor services, including transition practices, barriers, and implementation of services aligned with the six core elements of Health Care Transition 2.0 from the US Center for Health Care Transition Improvement. RESULTS: Representatives from 137 COG sites reported on institutional transition practices. Two-thirds (66.4%) of site discharge survivors to another institution for cancer-related follow-up care in adulthood. Transfer to primary care (33.6%) was a commonly reported model of care for young adult-aged survivors. Site transfer at ≤ 18 years (8.0%), ≤ 21 years (13.1%), ≤ 25 years (7.3%), ≥ 26 years (12.4%), or when survivors are "ready" (25.5%). Few institutions reported offering services aligned with the structured transition process from the six core elements (Median = 1, Mean = 1.56, SD = 1.54, range: 0-5). The most prevalent barriers to transitioning survivors to adult care were perceived lack of late-effects knowledge among clinicians (39.6%) and perceived lack of survivor desire to transfer care (31.9%). CONCLUSIONS: Most COG institutions transfer adult-aged survivors of childhood cancer elsewhere for survivor care, yet few programs report delivering recognized standards for quality healthcare transition programming to support survivors. IMPLICATIONS FOR CANCER SURVIVORS: Development of best practices for survivor transition is needed to help promote increased early detection and treatment of late effects among adult survivors of childhood cancer.

Hepatic hydrothorax is not associated with increased complications or poor survival after liver transplantation.

BACKGROUND: Hepatic hydrothorax (HH) is associated with a poor prognosis. Liver transplant (LT) is the best treatment modality. We aim to assess post-LT morbidity and mortality in patients with cirrhosis and HH. RESEARCH DESIGN AND METHODS: Adult patients with cirrhosis, who underwent LT at our institution from 2015 to 2020, were retrospectively reviewed. Baseline data was obtained at the time of LT. Patients were followed from baseline until the last follow-up or death. Censoring occurred at the time of the last follow-up or death, whichever occurred earlier. Cumulative incidence of outcomes was determined by the Kaplan-Meier method. Short-term post-operative complications were compared between both groups as well. RESULTS: 428 patients had a LT, of which 72 (16.8%) had HH. Most of the baseline characteristics were similar between patients with and without HH; however, patients in the HH group had a higher proportion of pre-operative history of ascites and hepatic encephalopathy. Pre-operative HH was not significantly associated with post-LT mortality (Hazard ratio 1.12, 95% confidence interval 0.54-2.32; P-value 0.76). Patients had similar short-term post-operative complications between both groups. CONCLUSIONS: LT is an excellent therapeutic option for patients with cirrhosis and HH, with excellent long-term survival without increased morbidity.

Growth Hormone Deficiency in Childhood Intracranial Germ Cell Tumor Survivors.

Background and Aims: Intracranial germ cell tumor (iGCT) survivors have multiple risk factors for growth hormone (GH) deficiency, a commonly reported late effect in childhood cancer survivors. The objective of this study is to examine the prevalence of GH deficiency among childhood iGCT survivors. Methods: Participants were previously enrolled in the Germ Cell Tumor Epidemiology Study (GaMETES), a case parent triad study conducted using the Children's Oncology Group registry protocols, including 216 cases with iGCTs. Data on late effects and outcomes are available for 129 iGCT cases who consented for a follow-up study including a self-administered questionnaire and medical record retrieval. GH deficiency was identified via self-report and validated through medical record review. Chi-squared and Fisher's exact tests were used to examine cases with GH deficiency predating iGCT detection. Logistic regression was used to identify predictors of GH deficiency as a late effect. Results: Of 129 iGCT cases who participated in the late effects study, 45% had GH deficiency; 18% had GH deficiency predating the iGCT and 27% developed GH deficiency within a median of 19 months after diagnosis. Younger age at diagnosis, suprasellar location, and higher radiation doses were associated with GH deficiency as a late effect. Conclusions: GH deficiency is highly prevalent as an early clinical sign for iGCT and frequently arises as an early late effect after treatment. Additional investigation is needed to address earlier detection and treatment for this highly prevalent late effect in iGCT survivors.

HPV vaccination coverage for pediatric, adolescent and young adult patients receiving care in a childhood cancer survivor program.

Pediatric, adolescent and young adult patients undergoing cancer treatment and/or hematopoietic stem cell transplant are at increased risk for developing a secondary human papillomavirus (HPV)-associated malignancy. The objective of this study was to determine HPV vaccination coverage among individuals participating in a childhood cancer survivor program (CCSP). A retrospective cohort study was conducted among CCSP patients age 11-26 years attending a CCSP visit between 2014 and 2019. Survivors were age-, sex-, and race-matched 1:2 with controls without cancer. Data were abstracted from the electronic health record and state-based vaccination registry. Analysis was limited to Minnesota residents to minimize missing vaccination data. Survivorship care plans (SCPs) were reviewed for vaccine recommendations. 592 patients were included in the analyses (200 CCSP patients; 392 controls). By study design, mean age (18.4 years), race (72 % white), and sex (49 % female) were similar in the two groups. Among CCSP patients 22 % resided in a rural area compared to 3.8 % of controls. Vaccination coverage among CCSP patients was not statistically significantly different from controls [60.0 % vs 66.3 %, OR = 0.82, 95 % CI: (0.55, 1.23), p = 0.35]. Completion of 3 doses was not different between groups even though 3 doses is recommended for all CCSP patients regardless of age at initiation (28.5 % vs 30.1 %, p = 0.09). Only 8.0 % of SCPs recommended HPV vaccination. Although patients participating in a CCSP did not have significantly different HPV vaccination coverage compared to controls, HPV vaccination initiation and 3-dose series completion are still suboptimal in a patient population at high-risk of a secondary HPV-associated cancer.

The generalized fractional NU method for the diatomic molecules in the Deng-Fan model.

Abstract: A solution of the fractional N-dimensional radial Schrödinger equation (SE) with the Deng-Fan potential (DFP) is investigated by the generalized fractional Nikiforov-Uvarov (NU) method. The analytical formulas of energy eigenvalues and corresponding eigenfunctions for the DFP are generated. Furthermore, the current results are applied to several diatomic molecules (DMs) for the DFP as well as the shifted Deng-Fan potential (SDFP). For both the DFP and its shifted potential, the effect of the fractional parameter ( δ ) on the energy levels of various DMs is examined numerically and graphically. We found that the energy eigenvalues are gradually improved when the fractional parameter increases. The energy spectra of various DMs are also evaluated in three-dimensional space and higher dimensions. It is worthy to note that the energy spectrum raises as the number of dimensions increases. In addition, the dependence of the energy spectra of the DFP and its shifted potential on the reduced mass, screening parameter, equilibrium bond length and rotational and vibrational quantum numbers is illustrated. To validate our findings, the energy levels of the DFP and SDFP are estimated at the classical case ( δ = 1 ) for various DMs and found that they are entirely compatible with earlier studies. Graphical abstract: In this study, a new algorithm of the generalized fractional Nikiforov-Uvarov method is employed to obtain new solutions to the fractional N-dimensional radial Schrödinger equation with the Deng-Fan potential. In addition, the results are applied to several diatomic molecules. The impact of the fractional parameter on the energy levels of various diatomic molecules is investigated. We found that the energy of the diatomic molecule is more bounded at lower fractional parameter values than in the classical case.

Liver stiffness measurement by vibration-controlled transient elastography improves outcome prediction in primary biliary cholangitis.

BACKGROUND & AIMS: Liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) has been shown to predict outcomes of patients with primary biliary cholangitis (PBC) in small-size studies. We aimed to validate the prognostic value of LSM in a large cohort study. METHODS: We performed an international, multicentre, retrospective follow-up study of 3,985 patients with PBC seen at 23 centres in 12 countries. Eligibility criteria included at least 1 reliable LSM by VCTE and a follow-up ≥ 1 year. Independent derivation (n = 2,740) and validation (n = 568) cohorts were built. The primary endpoint was time to poor clinical outcomes defined as liver-related complications, liver transplantation, or death. Hazard ratios (HRs) with CIs were determined using a time-dependent multivariable Cox regression analysis. RESULTS: LSM was independently associated with poor clinical outcomes in the derivation (5,324 LSMs, mean follow-up 5.0 ± 3.1 years) and validation (1,470 LSMs, mean follow-up 5.0 ± 2.8 years) cohorts: adjusted HRs (95% CI) per additional kPa were 1.040 (1.026-1.054) and 1.042 (1.029-1.056), respectively (p <0.0001 for both). Adjusted C-statistics (95% CI) at baseline were 0.83 (0.79-0.87) and 0.92 (0.89-0.95), respectively. Between 5 and 30 kPa, the log-HR increased as a monotonic function of LSM. The predictive value of LSM was stable in time. LSM improved the prognostic ability of biochemical response criteria, fibrosis scores, and prognostic scores. The 8 kPa and 15 kPa cut-offs optimally separated low-, medium-, and high-risk groups. Forty percent of patients were at medium to high risk according to LSM. CONCLUSIONS: LSM by VCTE is a major, independent, validated predictor of PBC outcome. Its value as a surrogate endpoint for clinical benefit in PBC should be considered. LAY SUMMARY: Primary biliary cholangitis (PBC) is a chronic autoimmune disease, wherein the body's immune system mistakenly attacks the bile ducts. PBC progresses gradually, so surrogate markers (markers that predict clinically relevant outcomes like the need for a transplant or death long before the event occurs) are often needed to expedite the drug development and approval process. Herein, we show that liver stiffness measurement is a strong predictor of clinical outcomes and could be a useful surrogate endpoint in PBC trials.

Refractory Hepatic Hydrothorax Is an Independent Predictor of Mortality When Compared to Refractory Ascites.

BACKGROUND: Hepatic hydrothorax (HHT) is an uncommon but significant complication of cirrhosis and portal hypertension, associated with a worse prognosis and mortality. Nearly 25% of patients with HHT will have refractory pleural effusion. It is unclear if refractory HHT has a different prognosis compared to refractory ascites. AIMS: We aim to evaluate the prognostic significance of refractory HHT when compared to refractory ascites. METHODS: Forty-seven patients who had refractory HHT in a tertiary care center were identified, and matched, retrospectively, one-to-one by age, gender and MELD-Na with 47 patients with refractory ascites. One-year mortality rate was compared between both groups. Cox proportional hazard regression was used to identify the association between different covariates and primary endpoint. RESULTS: The 1-year mortality was 51.06% in the HHT group compared to 19.15% in the refractory ascites group. The median survival for patients with refractory hepatic hydrothorax was 4.87 months while the median survival for patients with refractory ascites exceeded 1 year. The presence of HHT was statistically significant in predicting the development of 1-year mortality [Hazard Ratio (HR) 4.45, 95% Confidence Interval (CI) 2.25-8.82; P value < 0.001]. Furthermore, refractory HHT remained associated with one-year mortality after adjusting for all other covariates. In a subgroup of patients with MELD-Na ≤ 20, HHT continued to be a significant predictor of one-year mortality (HR 3.30, 95% CI 1.47-7.40; P value 0.004). CONCLUSIONS: Refractory HHT is a significant independent predictor of mortality and offers additional prognostic value.

Spontaneous bacterial empyema: a tertiary care center experience and a systematic review.

BACKGROUND: Spontaneous bacterial empyema (SBE) is an infection of a preexisting hepatic hydrothorax (HH). We aim to describe the experience in managing SBE in a liver transplant (LT) referral center and assessing the incidence and mortality rates of SBE after conducting a systematic review. METHODS: 992 patients with cirrhosis were retrospectively reviewed from 2015 to 2020. SBE was diagnosed by (i) positive microbiological culture and polymorphonuclear leukocyte count >250 cells/µL or (ii) negative microbiological culture, compatible clinical course, and polymorphonuclear count >500 cells/µL in pleural fluid. Furthermore, we conducted a comprehensive literature search of MEDLINE, EMBASE, and Google Scholar for studies evaluating SBE. RESULTS: Twelve patients (10.4%) had spontaneous bacterial empyema out of 115 patients with HH. Five patients underwent LT, 6 had died, and 1 did not get transplanted and was alive throughout the duration of follow-up. Ten studies were included in the systematic review. Pooled incidence in patients with HH was 19.03%. Only 20.69% of the patients received a LT. Pooled mortality rate was 46.45%, with only 3.45% of the patients dying post-transplant. CONCLUSION: SBE is a severe complication of cirrhosis and HH. LT may provide a survival benefit. Thus, patients should be considered for early transplant.

Secondary Prophylaxis of Gastric Variceal Bleeding: A Systematic Review and Network Meta-Analysis.

There is no clear consensus regarding the optimal approach for secondary prophylaxis of gastric variceal bleeding (GVB) in patients with cirrhosis. We conducted a systematic review and network meta-analysis (NMA) to compare the efficacy of available treatments. A comprehensive search of several databases from each database's inception to March 23, 2021, was conducted to identify relevant randomized controlled trials (RCTs). Outcomes of interest were rebleeding and mortality. Results were expressed as relative risk (RR) and 95% confidence interval (CI). We followed the Grading of Recommendations Assessment, Development, and Evaluation approach to rate the certainty of evidence. We included 9 RCTs with 647 patients who had histories of GVB and follow-ups >6 weeks. A total of 9 interventions were included in the NMA. Balloon-occluded retrograde transvenous obliteration (BRTO) was associated with a lower risk of rebleeding when compared with ß-blockers (RR, 0.04; 95% CI, 0.01-0.26; low certainty), and endoscopic injection sclerotherapy (EIS)-cyanoacrylate (CYA) (RR, 0.18; 95% CI, 0.04-0.77; low certainty). ß-blockers were associated with a higher risk of rebleeding compared with most interventions and with increased mortality compared with EIS-CYA (RR, 4.12, 95% CI, 1.50-11.36; low certainty), and EIS-CYA + nonselective ß-blockers (RR, 5.61; 95% CI, 1.91-16.43; low certainty). Analysis based on indirect comparisons suggests that BRTO may be the best intervention in preventing rebleeding, whereas ß-blocker monotherapy is likely the worst in preventing rebleeding and mortality. Head-to-head RCTs are needed to validate these results.