New-onset Ascites Research Articles

Ascites development is associated with worse outcome in patients after kidney transplantation.

We provide detailed analysis and outcomes in patients post-kidney transplant (KT) developing ascites, which has never been categorically reported. Ascites was identified by ICD9/10 codes and detailed chart review in patients post-KT from 01/2004-06/2019. The incidence of patient death and graft loss were determined per 100-person-years, and the incidence rate ratio was obtained. Of 3329 patients receiving KT, 83 (2.5%) patients had new-onset ascites, of whom 58% were male, 21% blacks, and 29% whites. Seventy-five percentage were on hemodialysis. Patients were maintained primarily on tacrolimus and mycophenolate for immunosuppression. Only 14% of patients with ascites had the appropriate diagnostic workup. There was a trend toward an increased mortality in patients with ascites (incidence rate ratio, IRR [95% CI]: 1.8 [0.92, 3.19], p=.06), and a significantly higher incidence of graft loss (IRR: 5.62 [3.97, 7.76], p<.001), compared with non-ascites patients. When classified by ascites severity, determined by imaging, moderate/severe ascites patients had the worst clinical outcomes, with a mortality of 32% and graft failure in 57%, compared with 9% and 10%, respectively, in those without ascites. In this large cohort employing stepwise analysis of ascites post-KT, worse outcomes were noted, dictating the need for optimized management to improve clinical outcomes.

Development and validation a simple model for identify malignant ascites.

The differential diagnosis of benign ascites and malignant ascites is incredibly challenging for clinicians. This research aimed to develop a user-friendly predictive model to discriminate malignant ascites from non-malignant ascites through easy-to-obtain clinical parameters. All patients with new-onset ascites fluid were recruited from January 2014 to December 2018. The medical records of 317 patients with ascites for various reasons in Renmin Hospital of Wuhan University were collected and reviewed retrospectively. Thirty-six parameters were included and selected using univariate logistic regression, multivariate logistic regression, and receiver operating characteristic (ROC) curve analyses to establish a mathematical model for differential diagnosis, and its diagnostic performance was validated in the other groups. Age, cholesterol, hypersensitivity C-reactive protein (hs-CRP) in serum, ascitic fluid adenosine deaminase (AF ADA), ascitic fluid lactate dehydrogenase (AF LDH) involvement in a 5-marker model. With a cut-off level of 0.83, the sensitivity, specificity, accuracy, and area under the ROC of the model for identifying malignant ascites in the development dataset were 84.7%, 88.8%, 87.6%, and 0.874 (95% confidence interval [CI], 0.822-0.926), respectively, and 80.9%, 82.6%, 81.5%, and 0.863 (95% CI,0.817-0.913) in the validation dataset, respectively. The diagnostic model has a similar high diagnostic performance in both the development and validation datasets. The mathematical diagnostic model based on the five markers is a user-friendly method to differentiate malignant ascites from benign ascites with high efficiency.

Difficult-to-treat ascitic fluid infection is a predictor of transplant-free survival in childhood decompensated chronic liver disease.

To study the clinico-bacteriological profile of ascitic fluid infection (AFI) and its impact on outcome in childhood chronic liver disease (CLD). It was a retrospective study on pediatric CLD patientsrequiring an ascitic tap. Logistic regression was performed to study the predictive factors for AFI. Two hundredand fifty-two (30.9%) of 814 children with CLD underwentascitic tap on suspicion of AFI of whom 79 (31.3%) had AFI, culture negative neutrocytic ascites being the commonest. Younger age (p = 0.002), male gender (p = 0.007), new onset/rapid increase in ascites (p = 0.032), fever (p = 0.012), and blood total leukocyte count (TLC)(p = 0.001) were found to be independently associated with AFI. Twenty-three children had positive ascitic fluid culture: 15 Gram negative; 11 (52.3%) were multidrug resistant organism. Hepatic encephalopathy (HE) (p = 0.001), Model for End-stage Liver Disease/Pediatric End-stage Liver Disease (MELD/PELD) (p < 0.0005), and difficult-to-treat AFI (p = 0.007) were found to be independently associated with death and or LT. Children with ascites should undergoa diagnostic paracentesis in presence of fever, increasing or new-onset ascites, and/or increased TLC. Death or liver transplant are more likely due to advanced liver disease (high PELD /HE) and in those with difficult-to-treat AFI.

S2518 A Unique Case of Radio-Embolization-Induced Liver Disease (REILD)

INTRODUCTION: Trans Arterial Radio-Embolization (TARE) is a form of selective radiation therapy used for the treatment of primary and metastatic hepatic cellular cancer (HCC) that involves delivering yttrium 90 (Y90) microspheres intra-arterially to target the tumor cells. Postradioembolization syndrome, hepatic dysfunction with deranged liver enzymes, vascular injury, etc. are the reported complications after TARE. Radio-Embolization Induced Liver Disease (REILD) characterized by ascites, weight gain, and elevated liver enzymes in the absence of tumor progression has been noticed with the incidence of 0–5% within 2 weeks to 4 months after TARE. Herein we report a unique case of REILD in a patient with abdominal distension 1 month after therapy treated with oral prednisone. CASE DESCRIPTION/METHODS: 75-year old female with a history of obesity, NAFLD presented with abdominal distension for last 1 month. She was diagnosed with HCC 3 months ago by histopathology of the right hepatic lobe lesion that was found in MRI of the abdomen (Figure#1). She had 2 sessions of TARE with Y90 one month prior. Laboratory results showed an increase in liver enzymes as shown in Table 1. CT scan of the abdomen showed slight increase in the size of right hepatic lobe lesion measuring 6 cm with the resolution of left lobe lesion (Figure 2). US of the abdomen revealed the same mass and new-onset moderate ascites. Ascitic fluid findings were consistent with portal hypertension. MRCP of the abdomen and US duplex hepatic and portal vessels were negative for biliary obstruction and veno-occlusion respectively. A provisional diagnosis of REILD was made, and oral prednisone was started. Her liver enzymes trended down over the course of five days suggestive of REILD. However, hospital course complicated by hepatorenal syndrome requiring dialysis. DISCUSSION: REILD is an uncommon side effect after RE. The absorbed dose in non-tumor parenchyma and reduced functional reserve (cirrhosis / previous treatments) are the key factors in predicting the development of liver injury. Treatment is mainly supportive, including avoidance of hepatotoxic agents and reduction of excessive extracellular volume. Medications like ursodeoxycholic acid and methylprednisolone combined together has showed to improve liver enzymes. However, no definitive treatment guidelines are formulated. It is warranted to formulate guidelines for identifying high-risk population and their treatment as RE is gaining more popularity with only increasing cases of REILD.Figure 1.: MRI of the abdomen without contrast at the time of diagnosis demonstrating right hepatic lobe mass measuring 5.6 × 5.7 × 5.0 cm.Figure 2.: CT of the abdomen and pelvis showing slight interval increase in size of right hepatic lobe mass measuring 6 cm and moderate ascites.Table 1.: Trend of Liver enzymes and creatinine during hospitalization

S2656 New Diagnosis of Autoimmune Hepatitis in a Patient With Remote History of SVR From DAA-Based Hepatitis C Treatment

INTRODUCTION: Autoimmune hepatitis (AIH) is characterized by increased aminotransferases, often in the presence of autoantibodies, and biopsy evidence of interface hepatitis. The cause of AIH is not known but it is likely due to a “triggering event” which leads to a T-cell mediated response directed against liver autoantigens. Hepatitis C virus (HCV) and its treatment with direct antiviral agents (DAA) have both has been suspected as risk factors for the development of AIH. We describe a patient with remote history of DAA-cured HCV who later developed decompensated cirrhosis secondary to AIH requiring liver transplantation. This case raises the question as to whether immunologic changes associated with HCV viral clearance are the cause of AIH in some cases. CASE DESCRIPTION/METHODS: A 67-year-old male was referred to our clinic for abnormal liver function tests. He was diagnosed with HCV cirrhosis, treated with 12 weeks of sofosbuvir/ledipasvir and achieved sustained virologic response (SVR). He was well for nearly five years until he developed new-onset ascites and worsening MELD-Na. Acute viral hepatitis serologies for hepatitis A/B/E were negative and HCV RNA levels were undetectable. CT imaging was negative for HCC or thrombus and he denied alcohol use. Smooth muscle IgG antibody was strongly positive and IgG was elevated at 3g/dL; ANA and LKM antibodies were negative. He was on not on any medications traditionally associated with AIH. His liver disease rapidly progressed and he was transplanted without receiving steroids. He is now well post-transplant. Explant biopsy demonstrated interface activity and lobular hepatitis with lymphoplasmacytic inflammation and plasma cells cluster (Figures 1 and 2). DISCUSSION: HCV has been linked to the development of AIH, likely through a process of molecular mimicry whereby the immune system reacts to hepatic autoantigens. While rare, this association has only been documented during active HCV infection or during treatment with DAAs. Our patient had successfully completed treatment for HCV with DAA therapy five years before onset of AIH. We present this case not only to highlight an atypical timeline for development of HCV-induced AIH but to emphasize that providers need to maintain a high index of suspicion for AIH in patients with prior history of HCV treated with DAAs who experience subsequent liver injury.Figure 1.: Low magnification (10x) view of liver section obtained from liver explant, demonstrating interface activity and lobular hepatitis with lymphoplasmacytic inflammation.Figure 2.: Higher magnification (40x) view of the liver section demonstrating plasma cells cluster.

Diagnostic Value of Ascitic Fluid Total Protein in Differential Diagnosis of Ascites: A Prospective Multicenter Study

Background: Diagnostic performance of ascitic fluid total protein (AFTP) concentration remained unsettled. Our aim was to determine diagnostic value of AFTP in differential diagnosis of ascites. Methods: 704 consecutive patients with new-onset ascites were prospectively enrolled in this study. Findings: In the training cohort, diagnostic performance of quantitative AFTP assay was superior to that of Rivalta test in differential diagnosis of ascites. At the pre-determined cut-off value of 25 g/L, quantitative AFTP assay was more useful in the differentiation of non-portal hypertensive ascites from portal hypertensive ascites compared with the exudate-transudate classification, area under curve (AUC) of receiver operating characteristic (ROC) curve was 0.958. Quantitative AFTP assay was superior to serum-ascites albumin gradient (SAAG) in the detection of non-portal hypertensive ascites, especially malignant ascites and tuberculous peritonitis. In mixed ascites, AFTP was useful in identifying peritoneal lesions. Interpretation: Assay of AFTP represents a beneficial adjunct to SAAG in non-portal hypertensive ascites and mixed ascites, thus it should be determined in diagnostic workup of the patients with ascites. Funding: This work was partially supported by National Natural Science Foundation of China and Clinical Research Physician Program of Tongji Medical College, Huazhong University of Science and Technology. Declaration of Interest: None. Ethical Approval: Our study was conducted in accordance with the principle of Declaration of Helsinki, and also approved by Ethics committee of Tongji Medical College, HUST. This study was registered with the Chinese Clinical Trial Registry Platform (http://www.chictr.org.cn/) as ChiCTR-DCD-15006907.

Sterile cerebrospinal fluid ascites presenting as high SAAG ascites: a case report

BackgroundCerebrospinal fluid ascites is a rare complication of ventriculoperitoneal shunting and is the result of infection and subsequent peritonitis in the majority of cases. Sterile cerebrospinal fluid ascites in which no known infectious etiology is identified, is even more unusual.Case PresentationA 26-year-old female with Loeys-Dietz syndrome and congenital hydrocephalus treated with a ventriculoperitoneal shunt, was evaluated after developing new-onset ascites of unclear etiology after abdominal surgery for repair of an aortic aneurysm requiring multiple therapeutic paracenteses. Her serum ascites albumin gradient (SAAG) was greater than 1.1, suggestive of a portal hypertensive etiology. Gram stain as well as multiple cultures of her ascites fluid were both negative. Extensive investigation including hepatic venous portal gradient measurement and liver biopsy revealed no evidence of hepatic disease or portal hypertension. She was ultimately found to have sterile cerebrospinal fluid ascites which was treated successfully with a peritoneovenous shunt.ConclusionSterile cerebrospinal fluid ascites is a rare clinical entity that has only been reported approximately 50 times in the medical literature. In this report, we also highlight it as a rare cause of high SAAG ascites. Moreover, we describe the use of a peritoneovenous shunt as a novel therapeutic option in the management of this condition.

Ascitic fluid total protein, a useful marker in non-portal hypertensive ascites.

Diagnostic performance of ascitic fluid total protein (AFTP) concentration remained unsettled. Our aim was to determine diagnostic value of AFTP in differential diagnosis of causes of ascites. Seven hundred four consecutive patients with new-onset ascites were prospectively enrolled in this study. In the training cohort, diagnostic performance of quantitative AFTP assay was superior to that of Rivalta test in differential diagnosis of ascites. At the predetermined cut-off value of 25g/L, quantitative AFTP assay was more useful in the differentiation of non-portal hypertensive ascites from portal hypertensive ascites compared with the exudate-transudate classification, area under curve of receiver operating characteristic curve was 0.958. Quantitative AFTP assay was superior to serum-ascites albumin gradient in the detection of non-portal hypertensive ascites, especially malignant ascites and tuberculous peritonitis. In mixed ascites, AFTP was useful in identifying peritoneal lesions. Ascitic fluid total protein is a useful marker in non-portal hypertensive ascites; thus, it should be determined in diagnostic work-up of the patients with ascites.

Effect of tolvaptan on the prognosis of patients with hepatic ascites.

Despite accumulating evidence concerning the efficacy of tolvaptan in the treatment of body fluid retention or hyponatremia, the effect of tolvaptan on the prognosis of patients with hepatic ascites has not been fully investigated. A total of 628 patients with hepatic ascites who were treated with diuretics (furosemide, spironolactone, or tolvaptan) between 2007 and 2017 were enrolled and divided into two groups: those who received tolvaptan (original tolvaptan group, n = 278) and those who did not (original control group, n = 350). The cumulative survival rates between the groups were compared and the factors associated with survival in patients with hepatic ascites were identified using a Cox regression analysis. In addition, propensity score matching was applied in patients who started conventional diuretics for new-onset hepatic ascites after September 2013 (pre-matching tolvaptan group, n = 177; pre-matching control group, n = 63), and the cumulative survival rates were compared between the post-matching tolvaptan and control groups. The survival rate was significantly higher in the tolvaptan group than the control group (P = 0.005), and tolvaptan therapy was identified as an independent factor associated with survival (hazard ratio 0.721 for death relative to control, P < 0.001). The propensity score-matched comparison also showed a significantly higher survival rate in the tolvaptan group (n = 51) than in the control group (n = 51) (P = 0.009). This study suggests that tolvaptan might improve the prognosis of patients with hepatic ascites.

Ascitic cholesterol is superior to serum‐ascites albumin gradient in the detection of non‐portal hypertensive ascites and the diagnosis of mixed ascites

The diagnostic value of ascitic cholesterol in the differential diagnosis of ascites is controversial. To investigate the diagnostic performance of ascitic cholesterol in the differential diagnosis of ascites. Consecutive patients with new-onset ascites were enrolled prospectively. The pertinent data were collected from 629 patients with all forms of ascites. In the training cohort, determination of the ascitic cholesterol level was a highly effective method of distinguishing non-portal hypertension (NPH) from portal hypertension (PH). At the pre-determined cut-off value of 45mg/dL, the sensitivity of ascitic cholesterol was superior to the serum-ascites albumin gradient (SAAG) in identifying NPH-related ascites; the area under the receiver operating characteristic curve was 0.945. In the patients misdiagnosed based on SAAG classification, the diagnostic accuracy of ascitic cholesterol was 69%. The ascitic cholesterol level showed excellent performance in identifying peritoneal lesions in patients with mixed ascites. Ascitic cholesterol is an excellent measure for detecting NPH ascites and for identifying peritoneal lesions in mixed ascites. Thus, this simple and cost-effective measure should be determined in patients with new-onset ascites (www.chictr.org.cn; ChiCTR-DCD-15006907).

Complete Esophageal Obstruction Following Routine Esophageal Variceal Ligation

A 67-year-old man with decompensated NASH cirrhosis presented for EGD for surveillance of esophageal varices. The patient was diagnosed 2 years prior when he presented with new-onset ascites. He underwent screening EGD previously, noting large (Grade III) esophageal varices and was started on non-selective beta blocker. However, he was intolerant secondary to presyncopal symptoms. He later presented with variceal hemorrhage necessitating variceal ligation; 2 bands were placed with hemostasis at that time. After initial episode of variceal ligation he was restarted on nonselective beta blocker. He underwent subsequent endoscopy 2 months later, where another band was placed for variceal ligation. Six weeks from his last variceal ligation, he presented for repeat routine outpatient variceal surveillance to ensure complete eradication. His EGD was notable for Grade II varices in the lower third of the esophagus; two bands were successfully placed (Figure A) with compete eradication, resulting in deflation of the varices. Immediately after the procedure, the patient complained of substernal chest pain, dysphagia, excessive salivation, nausea and had emesis in the recovery area. He was admitted overnight for observation. His symptoms continued despite symptom-targeted treatment that included antiemetic, proton pump inhibitors and intravenous fluid while was kept nill-by-mouth. In particular, he continued to be intolerant to oral secretions and to complain of dysphagia. Water soluble esophagram was obtained and was notable for complete luminal obstruction (Figure B). The patient was offered endoscopy for evaluation and possible intervention but he declined and opted to continue with the conservative management described earlier with addition of scopolamine patch. He eventually tolerated liquid diet 4 days following variceal ligation; repeat esophagram noted contrast passing into the stomach (Figure C).1721_A Figure 1 No Caption available.1721_B Figure 2 No Caption available.1721_C Figure 3 No Caption available.

Hepatic microcirculatory thrombosis in acute-on-chronic hepatic failure

BackgroundAcute-on-chronic hepatic failure is an increasing number of identified distinct disorders encompassing an acute deterioration of hepatic functions in patients with chronic liver diseaseAimThe aim was to evaluate the possibility of hepatic microcirculatory thrombosis in acute-on-chronic hepatic failure and the value of plasma fibrin monomer (FM) and D-dimer in the diagnosis.Patients and methodsA total of 50 patients with chronic hepatitis C infection developing new-onset ascites, encephalopathy, and/or jaundice with raised international normalisation ratio (INR) (group 1); group 2 included 30 patients with compensated chronic hepatitis C virus infection who served as the control group. Ascetic fluid examination and culture were done for group 1, in addition to complete blood count, liver enzymes, INR, serum bilirubin and albumin, blood culture, α-fetoprotein, D-dimer, plasma FM, abdominal ultrasound, and Doppler for portal vein were done for all patients groups.ResultsGroup 1was subdivided according to the level of FM into subgroups A and B. FM showed a significant difference between group 1A and other groups; group IB showed nonsignificant elevation of the level of FM and a significant increase in D-dimer compared with the control group. A marked reduction in portal flow mean velocity in group 1A was recorded with further deterioration of portal flow direction after 2 weeks from the admission time. Three months follow-up showed significant reduction of FM, improvement of portal flow mean velocity and direction in group 1A; FM was significantly positively correlated with portal flow mean velocity.ConclusionHepatic microcirculatory thrombosis may occur in acute-on-chronic hepatic disease; determination of the FM and D-dimer level may be useful biomarkers predicting hepatic microcirculatory thrombosis.

Case of Chylous Ascites: A Diagnostic Dilemma

We present a case of an 83 year-old male who is status post orthotopic liver transplant in November 2005 for alcoholic cirrhosis, presenting to Henry Ford Hospital with increasing abdominal girth. His symptoms began 2 months prior to presentation following cardiac pacemaker placement for heart block. He was hospitalized shortly after his procedure for shortness of breath and was found to have a pericardial effusion, which was thought to be inflammatory in nature and resolved with steroids. He had been following in the transplant clinic on a regular basis without issues, and was maintained on a stable immunosuppression regimen of mycophenolate and Sirolimus for several years. As part of the work up for his new-onset ascites, a paracentesis was performed. Fluid analysis demonstrated 702 WBCs with 77% lymphocytes, serum-albumin ascites gradient (SAAG) < 1.1 g/dL, total protein < 2.5 g/dL and triglycerides of 2360 mg/dL; consistent with chylous ascites. Lipase and amylase levels were within normal limits. Fungal cultures, acid-fast bacilli stains and cytology were all negative. Echocardiogram showed normal right-sided pressures with preserved ejection fraction and stable pericardial effusion. CT abdomen was unremarkable with no evidence of cirrhosis or portal hypertension. Lymphoscintigraphy study was unremarkable. Liver biopsy was performed without evidence of cirrhosis or rejection. Cardiology was consulted to determine whether the ascites was secondary to venous/lymphatic obstruction as a complication of pacemaker placement or heart failure; this was felt unlikely given preserved cardiac function and normal positioning of pacemaker leads on x-ray. Ultimately, Sirolimus was felt to be the culprit of his chylous ascites. Sirolimus was subsequently discontinued and cyclosporine was initiated with resolution of his ascites. Chylous ascites is a rare finding. It most commonly occurs in the setting of malignancy, lymphatic/vascular obstruction or cirrhosis. Less common etiologies include mycobacterial infection, congenital, inflammatory, post-operative, traumatic and medications such as calcium channel blockers and Sirolimus. Few cases of chylous ascites secondary to Sirolimus have been reported, particularly in renal transplant patients. There are no reported cases of Sirolimus-induced chylous ascites in liver transplant patients. The diagnosis is established by abdominal paracentesis, with fluid studies demonstrating a triglyceride level >200 mg/dL. SAAG and protein levels may vary. Acid-fast bacilli stain and ADA should be performed as well to rule out tuberculous ascites in those at high risk, along with amylase, lipase and standard fluid studies. Abdominal CT should be obtained to rule out pathologic lymph nodes and masses, and lymphoscintigraphy or lymphangiography can aid in detecting leakage from lymphatics and patency of the thoracic duct. Failure to identify a clear etiology following this workup should prompt an investigation into drug etiologies. Treatment is aimed at correction of the underlying etiology (or withholding the offending agent) along with therapeutic paracenteses as needed. A high protein and low fat diet with medium chain triglycerides is recommended. Occasionally, surgery may be performed for obstructive etiologies such as malignancy. Transjugular intrahepatic shunts may be performed for cirrhotic patients. There is also limited evidence for the use of orlistat, somatostatin and octreotride in the mitigation of chylous ascites.

Incarcerated inguinal hernia as a complication of new-onset ascites

This case report presents a patient who developed an acute incarcerated inguinal hernia as a result of newly acquired ascites, a complication of ascites that is not well documented in...

Atraumatic chylous ascites: an unusual presentation of bladder cancer

We describe a case of bladder cancer presenting with atraumatic chylous ascites, which remains an extremely rare presentation of this condition. A previously well, elderly ex-smoker with no prior history...

Abnormal Liver Stiffness Measurement in a Patient with Waldenstrom Macroglobulinemia: Nuances in Interpreting the Findings

Background: Liver stiffness measurement (LSM) using vibration-controlled transient elastography (VCTE) provides an estimate of liver fibrosis, non-invasively, in a quick and efficient manner. Its use and interpretation of LSM is limited. In the current case, we report our findings of abnormal LSM and liver histology in a patient with Waldenstrom Macroglobulineima (WM) with evidence of portal hypertension. Case: A 84-year-old white man with history notable for WM and history of pulmonary embolism on chronic anticoagulation presented with new-onset ascites. His blood work revealed isolated elevation in alkaline phosphatase (AST 14 U/L, ALT 7 U/L, ALP 173 U/L, GGT 275 u/L and total bilirubin 0.6 mg/dL) with a platelet count was 208 k/cumm. Blood work for competing etiology was negative for viral, metabolic, and autoimmune hepatitis (negative viral hepatitides, normal iron studies, normal alpha-1-antitrypsin level, negative anti-smooth muscle antibody and negative anti-mitochondrial antibody with normal immunoglobulins (other than presence of IgM kappa monoclonal protein) ruling out primary biliary cirrhosis). Ultrasound with dopplers showed cirrhotic appearing liver, cholelithiasis, mild ascites not amenable to diagnostic paracentesis, and patent portal veins. MRCP showed normal appearing liver, mild ascites, and gallbladder sludge without biliary dilation. Evaluation with VCTE after paracentesis revealed elevated LSM at 12.2 with IQR% of 9%. Pressure measurements during transjugular liver biopsy noted normal hepatic vein pressure gradient (HVPG) of 6 mmHg (corrected sinusoidal pressure 10 mmHg (RA 1 mmHg, IVC 5 mmHg, free HV 5 mmHg, wedge 11 mmHg), consistent with pre-sinusoidal portal hypertension. Liver histology showed nodularity in the absence of significant fibrosis. Reticulin stain confirmed nodularity and showed areas of hepatic trabecular compression and others with trabecular thickening. Focal mild sinusoidal dilatation was seen. The portal tracts are largely unremarkable with patchy mild chronic inflammation and minimal ductular reaction. Congo red stain was negative as well ruling out amyloidosis. Conclusion: This case highlights the rare presentation of non-cirrhotic portal hypertension with WM and the false increase in LSM due to infiltration with M protein.

Chylothorax and Chylous Ascites - A Rare Manifestation of Signet Ring Cell Carcinoma

Introduction: Chylous ascites is the accumulation of milk-like peritoneal fluid rich in triglycerides in the abdominal cavity. Chylothorax is the accumulation of this lipid-rich fluid in the thoracic cavity, and has rarely been reported in the literature as a consequence of malignancy. We present a case of a patient with signet ring cell carcinoma presenting with chylothorax and chylous ascites. Case Presentation: A 68 year old man with severe COPD and atrial fibrillation presented with shortness of breath. A chest x-ray (Figure 1A) and CT Chest (Figure 1B) revealed a right pleural effusion. Multiple thoracenteses revealed cloudy, white fluid. Infectious and cytologic studies on the fluid, as well as a whole body PET-CT were negative for malignancy.Figure 1Three months later the patient was admitted for reaccumulating hydrothorax, as well as new-onset ascites. A diagnostic paracentesis revealed milky, white chylous fluid (Figure 1C). Ascitic fluid analysis revealed Triglycerides of 500, WBC 605 (76% lymph), Amylase 13, Total Protein 3.1, Albumin 1.6 and a SAAG of 2.1. Blood work was not consistent with cirrhosis. A triple phase CT revealed normal liver size and parenchyma, with no identifiable hepatic or intra-abdominal lesions. EGD revealed a small area of mucosal nodularity in the 2nd portion of the duodenum (Figure 2A). A colonoscopy revealed mucosal thickening in the rectosigmoid colon (Figure 2B). Biopsies of both lesions were positive for poorly differentiated adenocarcinoma with signet ring cells.Figure 2The patient initiated therapy with FOLFOX regimen, however ultimately opted for hospice and expired three months later. Discussion: Chylous ascites is rare, with an incidence of approximately 1 per 20,000 admissions over the last 20 years. In the setting of malignancy, chylous fluid extravasation is thought to result from obstruction of the lymphatic channels by either direct tumor cell invasion or mass effect. Chylous ascites and chylothorax are often with lymphoma. There are only a few reported cases of chylous ascites or chylothorax from signet ring cell carcinoma. All reported cases depict this finding as a late manifestation of metastatic disease. Conclusion: Chylous ascites and chylothorax are exceedingly rare manifesations of signet ring cell carcinoma. All reported cases have been diagnosed in the setting of late stage metastatic disease. These findings should prompt further evaluation for an underlying gastrointestinal malignancy with EGD and colonoscopy.

US-Guided Paracentesis Complicated by Delayed Hemoperitoneum with Hemorrhagic Shock

Delayed hemoperitoneum is a rare complication of paracentesis, a generally safe procedure with overall complications occurring in less than one percent of cases. Vascular injuries are often addressed during or immediately following paracentesis. Regardless, life-threatening hemorrhage may arise following this well-tolerated procedure, especially in the setting of coagulopathy. We present a case of delayed hemorrhagic shock due to bleeding from a branch of the right inferior epigastric artery after ultrasound-guided paracentesis. A 67-year-old female with history of primary biliary cirrhosis and recent tibia-fibula fracture complicated by deep vein thrombosis on full-dose anticoagulation presented with abdominal distention and pain secondary to new-onset ascites. With her anticoagulation held, platelet count of 63 thousand/mcl and INR of 1.4, she underwent an ultrasound-guided paracentesis with removal of 5L of clear, yellow ascitic fluid. Afterward, anticoagulation was resumed and diuretics were initiated. The next day, she developed asymptomatic hypotension. Labs were notable for a hemoglobin drop from 11.6 mg/dL to 10.8mg/dL; INR 1.5. Despite aggressive volume resuscitation, she was transferred to the intensive care unit with multi-organ system failure. Repeat hemoglobin was 4.8mg/dL. Noncontrast computed tomography was notable for a large amount of ascites with intraperitoneal blood (Figure 1a). Urgent coil embolization of a pseudoaneurysm of the right inferior epigastric artery was performed (Figure 1b, 1c). Her course was complicated by ischemic hepatitis, acute kidney injury, disseminated intravascular coagulopathy, arrhythmia, and acute encephalopathy necessitating intubation and eventual withdrawal of life-sustaining measures.Figure 1Most hemorrhagic complications following paracentesis are due to abdominal wall trauma. Often iatrogenic and perhaps underreported, damage to the inferior epigastric artery can be fatal. Currently, there are no known guidelines to correct coagulopathies prophylactically or use vascular-flow imaging during ultrasound-guided paracentesis to prevent and identify bleeds. Given the infrequent occurrence of delayed hemoperitoneum, ultrasound-guided paracentesis with Doppler may allow for better visualization of abdominal wall vessels, especially in high-risk patients on anticoagulation.

Nodular Regenerative Hyperplasia

A 38-year-old man with a history of two kidney transplants as well as genotype 3A hepatitis C virus (HCV) without cirrhosis presented with hematemesis. The patient was a nonresponder to pegylated IFN and ribavirin. A transjugular liver biopsy 2 years prior revealed stage 0 fibrosis without portal hypertension (HVPG=2 mm Hg). The patient completed a 12-week course of daclatasvir, sofosbuvir, and ribavirin, and achieved viral eradication with SVR12. Following HCV eradication, the patient developed new-onset ascites. Abdominal ultrasound revealed nodular-appearing liver and moderate ascites. Diagnostic paracentesis showed SAAG > 1.1 with low total protein (< 1.0 g/dL) and no evidence of SBP. He presented after one episode of hematemesis and was found to have a hemoglobin of 6.7 g/dL (baseline 10 g/dL). Emergent EGD revealed new medium-sized esophageal varices that were banded. Despite octreotide infusion, he developed recurrent hematemesis on hospital day 2. Repeat EGD revealed evidence of recurrent variceal bleeding. Liver biopsy demonstrated sinusoidal portal hypertension (HVPG=13 mm Hg) and nodular regenerative hyperplasia of the liver. He underwent TIPS insertion for recurrent variceal bleed with significant reduction in portal pressure gradient and resolution of bleeding. Nodular regenerative hyperplasia (NRH) is a form of noncirrhotic portal hypertension (NCPH). Histologically, NRH is distinguished from other causes of NCPH by diffuse parenchymal regenerative nodules and an absence of or minimal fibrosis in the perisinusoidal/periportal regions. The exact pathophysiology is not clearly defined; however, a generally accepted hypothesis involves hepatocyte hyperplasia in response to chronic inflammation, vascular disease, or direct endothelial injury from drugs, autoantibodies, T-cells, malignancies, or viruses. Development of NRH typically occurs within 1 to 6 years of insult, with some cases reporting development of NRH as early as 5-6 months after exposure toxic agents (i.e. azathioprine, oxaliplatin). Treatment involves avoiding and/or discontinuing offending agents and management of portal hypertension. While NRH is a rare but known complication of solid organ transplantation, it has yet to be reported following successful HCV therapy with direct-acting antiviral agents. In conclusion, we report a novel case of clinically significant NCPH secondary to NRH that developed in a kidney transplant recipient immediately following eradication of HCV.

Von Willebrand factor and procoagulant imbalance predict outcome in patients with cirrhosis and thrombocytopenia

Several lines of evidence suggest that the hemostatic disorders of cirrhosis may have a significant clinical impact. We investigated the independent predictive value of components of the hemostatic system on the occurrence of ascites, variceal bleeding (VB), and survival. One hundred and two patients with thrombocytopenia (Child-Pugh class A/B/C: 34/34/34) were enrolled. Platelet counts, factors (F) II, V, VII, and VIII, antithrombin, protein C (PC), FVIII-to-PC ratio as an index of procoagulant imbalance, von Willebrand factor antigen (vWF-Ag), and model for end-stage liver disease (MELD) were evaluated. Two multivariate analyses were performed: one excluding (model 1) and one including MELD (model 2). Higher vWF-Ag levels and FVIII-to-PC ratios were the most prominent hemostatic disorders in patients with cirrhosis. Increased levels of vWF-Ag and FVIII, and higher FVIII-to-PC ratios independently predicted the presence of ascites and varices at baseline. Independent predictors of ascites and VB during follow-up were vWF-Ag (model 1/2: p=0.001/p=0.009 and p=0.008/p=0.01, respectively) and FVIII-to-PC ratio (model 1/2: p=0.003/p=0.02 and p=0.01/p=0.03, respectively). vWF-Ag (model 1/2: p=0.007/p=0.002), FVIII-to-PC ratio (model 1/2: p=0.001/p=0.01), and MELD (p=0.02) independently predicted mortality. Patient groups with significantly higher probability of new-onset ascites, VB, and mortality were identified by certain cut-offs of vWF-Ag (213%, 466%, and 321%, respectively) and FVIII-to-PC ratio (1.99, 3.29, and 2.36, respectively). vWF-Ag and FVIII-to-PC ratio equaled MELD in mortality prediction. Advanced cirrhosis is characterized by increased thrombotic potential. vWF-Ag and FVIII-to-PC ratio independently predict new-onset ascites, VB, and mortality. Targeting hypercoagulability could improve the outcome of patients with cirrhosis. Higher von Willebrand factor antigen (vWF-Ag) levels and factor VIII-to-protein C (FVIII-to-PC) ratio are the prominent hemostatic disorders in patients with cirrhosis. vWF-Ag and FVIII-to-PC ratio independently predict new-onset ascites, variceal bleeding, and mortality in these patients.