Acquired Hepatocerebral Degeneration: A Case Series of a Rare Condition.

BACKGROUND: Acquired hepatocerebral degeneration (AHD) is a neurologic complication of severe chronic liver disease (CLD) with portosystemic shunts. The proposed etiology is manganese accumulation in the brain tissue, especially in the basal ganglia. Combination of clinical manifestation, mostly extrapyramidal movement disorders, and hyperintensities on T1-weighted brain magnetic resonance imaging (MRI) is diagnostic. Although liver transplantation controversial, it is suggested for AHD. OBJECTIVES: To depict clinical and neuroimaging characteristics and response to treatments in patients diagnosed with AHD at Sheba Medical Center. METHODS: Review of patients with AHD diagnosis at the Liver Diseases Center at Sheba Medical Center between 2012 and 2017, data of clinical and neuroimaging, follow-up, and response to treatments, including liver transplantation were recorded. RESULTS: Five patients with diagnosis of AHD were identified, median age at diagnosis 55 years (range 45-64 years). Four patients had cirrhosis at the time of AHD diagnosis. The main risk factor for AHD was the presence of portosystemic shunts. The most prevalent clinical manifestations were movement disorders, specifically a combination of extrapyramidal and cerebellar signs including instability, rigidity, tremor, bradykinesia, and cognitive impairment. Brain MRI revealed hyperintensities on T1-weighted images in the basal ganglia in all patients. Administration of antiparkinsonian drugs showed clinical improvement, whereas liver transplantation performed in two patients was not associated to neurological improvement. CONCLUSIONS: AHD is related to portosystemic shunts. The combination of Parkinsonism and cerebellar signs and MRI pallidal lesions should alert physicians to the diagnosis. The role of liver transplantation in AHD is still controversial.

The possible association of proton pump inhibitor use with acute cholangitis in patients with choledocholithiasis: a multi-center study.

BACKGROUND: Gallstone disease is common worldwide and can lead to severe complications, including cholangitis; thus, it is important to identify modifiable risk factors for cholangitis. Proton pump inhibitors (PPIs) are commonly prescribed to treat gastroenterological disorders. We aimed to explore whether PPI use is associated with an increased risk of acute cholangitis in patients with gallstone disease. METHODS: This retrospective multicenter study included all patients arriving to the hospital over a 10-year period with various presentations of choledocholithiasis. We compared active PPI use in two groups: those with cholangitis (group A) vs. without cholangitis (group B). RESULTS: Overall, 811 patients were included, 161 in group A and 650 in group B. The average age ± standard deviation (SD) in groups A and B was 74.5 ± 20.6 vs. 61.6 ± 20.9 years, respectively. PPI use in group A was higher vs. group B (42.9% vs. 29.1%, p = 0.001). On univariate analysis, male gender (OR 1.47, 95% confidence interval (CI) 1.04-2.08), age (OR 1.04, 95% CI 1.03-1.05), ischemic heart disease (IHD) (OR 1.68, 95% CI 1.07-2.64), hyperlipidemia (OR 1.59, 95% CI 1.11-2.29), hypertension (OR 1.81, 95% CI 1.28-2.57) and PPI use (OR 1.83, 95% CI 1.28-2.61), all were associated with acute cholangitis. On multivariate analysis, only PPI use kept its association after adjustment for age (OR 1.64, 95% CI 1.2-3.7). CONCLUSIONS: Active PPI use was associated with a higher rate of cholangitis among patients with choledocholithiasis. We advocate considering this risk before prescribing PPIs to patients with gallstones. TRIAL REGISTRATION NUMBER: NHR-0263-20 received on 14/01/2021 date 'retrospectively registered'.

Nonattendance to gastroenterologist follow-up after discharge is associated with a thirty-days re-admission in patients with inflammatory bowel disease: a multicenter study.

BACKGROUND: Inflammatory bowel disease (IBD) is a set of chronic inflammatory diseases associated with significant morbidity and high hospitalization rate. IBD patients are particularly prone to rehospitalization resulting in high medical cost and morbidity. The aim of this study was to assess laboratory and clinical predictors of readmission in patients who were hospitalized with IBD flare. METHODS: A multicenter, retrospective, cross-sectional analysis included IBD patients who were admitted with disease exacerbation from January 1, 2019 to January 1, 2020 in three Israeli university hospitals (Nazareth Hospital, Galilee Medical Center and Hadassah Medical Organization). RESULTS: Overall, a total of 176 hospitalizations for IBD flares were included. Seventeen patients were readmitted within 30 days after discharge (group A), as compared to 159 patients who were not (group B). The average age was 35.3±19.2 years in group A vs. 38.6±16 years in group B. Eight (47.1%) and 9 (52.9%) patients had Crohn's disease (CD) and ulcerative colitis (UC) in group A as compared to 102 (64.2%) and 57 (35.9%) in group B, respectively. On univariate analysis, only the attendance to gastroenterology clinic follow-up after discharge from hospitalization due to IBD flare was significantly protective factor to with 30-days readmission (OR=0.37, 95% CI: 0.13-1, P=0.05). There were no associations with the other assessed clinical and laboratory parameters and importantly IBD type (OR=1.99, 95% CI: 0.74-5.34, P=0.17). Notably, there was no effect of the day of discharge white blood counts, albumin and C reactive protein (CRP) values on readmission rates (odds ratio [OR]=1.07, 95% CI: 0.96-1.20, P=0.19, OR=0.86, 95% CI: 0.39-1.91, P=0.71 and OR=0.99, 95% CI: 0.97-1.01, P=0.59), respectively. CONCLUSIONS: Attendance to out-patient gastroenterologist follow-up is the only significant protective parameter to 30-days readmission in patients with IBD. This finding highlights the vital need of adequate gastroenterological follow-up of these patients after hospital discharge. Further studies are warranted to precisely define timing and role of outpatient follow-up in reducing IBD readmissions.

A validated score predicting common bile duct stone in patients hospitalized with acute calculus cholecystitis: a multi-center retrospective study.

BACKGROUND: Concomitant common bile duct (CBD) stone in the setting of acute calculous cholecystitis (ACC) should be suspected once abnormal liver indices are noticed. AIM: We aimed to identify predictors of CBD stone in patients hospitalized with ACC. METHODS: We performed a retrospective multi-center, case-controlled, study from 1st of January 2016 until the 31th of December 2018. Inclusion criteria included patients with an established diagnosis of ACC based on clinical, laboratory and radiological criteria and who had an endoscopic ultrasound (EUS) for suspected CBD stone. One-hundred and twelve patients were included, of these fifty-three patients (47.3%) were diagnosed with CBD stone by EUS. RESULTS: In univariate analysis, Age (OR 1.038, P = 0.001), total bilirubin (mg/dl) (OR 1.429, P = 0.02) and CBD width (mm) by US (OR 1.314, P = 0.01) were statistically significant in predicting CBD stone and remained significant in multivariate regression analysis. We developed a diagnostic score that included these three parameters, with assignment of weights for each variable according to the coefficient estimate. A low cut-off score of 0 was associated with sensitivity of 100% for CBD stone, whereas a high cut-off score of 3 was associated with sensitivity of 10% and specificity of 96.6% with a positive predictive value of 67% (ROC of 0.7558). We validated this score with an independent cohort (ROC of 0.7416) with a sensitivity of 46.6%, a specificity of 91.5% and a PPV of 87.1%. CONCLUSION: We recommend incorporating this score as an aid for stratifying patients with ACC into low or high probability for concomitant CBD stone.

Unawareness of thromboprophylaxis is associated with low venous thromboembolism occurrence in hospitalized patients with acute inflammatory bowel disease flare.

BACKGROUND: Inflammatory bowel disease (IBD) is a set of chronic inflammatory diseases associated with significant morbidity. Generally, IBD patients have twice the risk of venous thromboembolism (VTE) compared to healthy controls. VTE can occur both, during hospital stay or after discharge. We aimed to assess the incidence among IBD patients who were hospitalized for disease exacerbation. METHODS: In a retrospective cross-sectional analysis all IBD patients who were admitted with disease exacerbation at Galilee Medical Center and Hadassah Medical Organization were included in the study. Excluding criteria was IBD with already known hypercoagulable state. RESULTS: One-hundred and sixteen patients with 176 admissions due to IBD flare were included in the study. The average age was 38.3±16.3 years. Sixty-six admissions (37.5%) occurred in patients with ulcerative colitis exacerbation and 110 in patients with Crohn's disease exacerbation (62.5%). Thirty-nine patients (22.1%) were smokers. Fifty-four patients (30.7%) and 68 patients (38.6%) were on previous (within 3 months) biological and steroid treatment, respectively. Twelve patients (6.8%) were on prophylactic subcutaneous anticoagulation (enoxaparin) throughout their hospital stay and only 3 patient (1.7%) developed in-hospital clinical VTE episode. The mean hospitalization length was 6.8±7.9 days and among patients who developed VTE episode, the length of stay was significantly higher as compared to patients without VTE episodes (36.7 vs. 6.3 days, P<0.0001). Notably, in-hospital IBD related-surgical procedure was the only risk factor for the development of VTE (Odds Ratio: 36.2; P=0.01). CONCLUSIONS: In-hospital VTE is rare among IBD patients admitted with exacerbation. Further studies are warranted to assess risk factors for in-hospital VTE development and to assess further the role of prophylactic anticoagulation among IBD patients with bloody diarrhea.

Prediction of Resistance to Intravenous Immunoglobulin in Children With Kawasaki Disease.

BACKGROUND: Approximately 10%-20% of patients with Kawasaki disease (KD) are refractory to initial intravenous immunoglobulin (IVIg) therapy, and these "nonresponders" are at higher risk of coronary artery abnormalities. Early identification of these patients, who may benefit from additional therapy, is challenging. The aim of the present study is to identify predictors for IVIg resistance. METHODS: We reviewed clinical records of 312 consecutive KD patients from 9 medical centers in Israel (development dataset) and 186 patients from additional 5 centers (validation dataset). Using multivariate analysis, we identified predictors of IVIg resistance. A third small prospective cohort of consecutive KD patients from a single medical center was used to test the accuracy of the predictors. RESULTS: Coronary artery abnormalities in the initial echocardiogram and presenting before day 5 of fever were independent predictors of IVIg nonresponse. Using either of these variables generated an area under the receiver-operating-characteristics curve of 0.7 (95% confidence interval [CI], 0.6-0.8). Sensitivity to predict nonresponse was 81% (95% CI, 67-90) and specificity was 50% (95% CI, 44-56). Similar results were found in the validation dataset and in the small prospective cohort. CONCLUSIONS: Coronary artery abnormalities in the initial echocardiogram and presenting before day 5 of fever show high sensitivity in identifying IVIg nonresponders among our KD patients.