ABSTRACT
INTRODUCTION AND OBJECTIVES: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. MATERIALS AND METHODS: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. RESULTS: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22-72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0-20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). CONCLUSIONS: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required.
Subject(s)
Liver Cirrhosis , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Cholangitis , Esophageal and Gastric Varices/diagnosis , Esophageal and Gastric Varices/etiology , Esophageal and Gastric Varices/therapy , Gastrointestinal Hemorrhage/etiology , Kidney Diseases, Cystic/complications , Liver Cirrhosis/diagnosis , Liver Cirrhosis/etiology , Liver Cirrhosis/therapy , Retrospective StudiesABSTRACT
BACKGROUND: Reliable devices that quantitatively monitor the level of neuromuscular blockade after neuromuscular blocking agents' administration are crucial. Electromyography and acceleromyography are two monitoring modalities commonly used in clinical practice. The primary outcome of this study is to compare the onset of neuromuscular blockade, defined as a Train-Of-Four Count (TOFC) equal to 0, as measured by an electromyography-based device (TetraGraph) and an acceleromyography-based device (TOFscan). The secondary outcome was to compare intubating conditions when one of these two devices reached a TOFC equal to 0. METHODS: One hundred adult patients scheduled for elective surgery requiring neuromuscular blockade were enrolled. Prior to induction of anesthesia, TetraGraph electrodes were placed over the forearm of patients' dominant/non-dominant hand based on randomization and TOFscan electrodes placed on the contralateral forearm. Intraoperative neuromuscular blocking agent dose was standardized to 0.5 mg.kg-1 of rocuronium. After baseline values were obtained, objective measurements were recorded every 20 seconds and intubation was performed using video laryngoscopy once either device displayed a TOFC = 0. The anesthesia provider was then surveyed about intubating conditions. RESULTS: Baseline TetraGraph train-of-four ratios were higher than those obtained with TOFscan (Median: 1.02 [0.88, 1.20] vs. 1.00 [0.64, 1.01], respectively, p < 0.001). The time to reach a TOFC = 0 was significantly longer when measured with TetraGraph compared to TOFscan (Median: 160 [40, 900] vs. 120 [60, 300] seconds, respectively, p < 0.001). There was no significant difference in intubating conditions when either device was used to determine the timing of endotracheal intubation. CONCLUSIONS: The onset of neuromuscular blockade was longer when measured with TetraGraph than TOFscan, and a train-of-four count of zero in either device was a useful indicator for adequate intubating conditions. CLINICAL TRIAL NUMBER AND REGISTRY: URL NCT05120999, https://clinicaltrials.gov/ct2/show/NCT05120999.
Subject(s)
Anesthetics , Neuromuscular Blockade , Neuromuscular Blocking Agents , Neuromuscular Nondepolarizing Agents , Adult , Humans , Androstanols , Neuromuscular Monitoring , Prospective Studies , RocuroniumABSTRACT
Abstract Background Reliable devices that quantitatively monitor the level of neuromuscular blockade after neuromuscular blocking agents' administration are crucial. Electromyography and acceleromyography are two monitoring modalities commonly used in clinical practice. The primary outcome of this study is to compare the onset of neuromuscular blockade, defined as a Train-Of-Four Count (TOFC) equal to 0, as measured by an electromyography-based device (TetraGraph) and an acceleromyography-based device (TOFscan). The secondary outcome was to compare intubating conditions when one of these two devices reached a TOFC equal to 0. Methods One hundred adult patients scheduled for elective surgery requiring neuromuscular blockade were enrolled. Prior to induction of anesthesia, TetraGraph electrodes were placed over the forearm of patients' dominant/non-dominant hand based on randomization and TOFscan electrodes placed on the contralateral forearm. Intraoperative neuromuscular blocking agent dose was standardized to 0.5 mg.kg−1 of rocuronium. After baseline values were obtained, objective measurements were recorded every 20 seconds and intubation was performed using video laryngoscopy once either device displayed a TOFC = 0. The anesthesia provider was then surveyed about intubating conditions. Results Baseline TetraGraph train-of-four ratios were higher than those obtained with TOFscan (Median: 1.02 [0.88, 1.20] vs. 1.00 [0.64, 1.01], respectively, p < 0.001). The time to reach a TOFC = 0 was significantly longer when measured with TetraGraph compared to TOFscan (Median: 160 [40, 900] vs. 120 [60, 300] seconds, respectively, p < 0.001). There was no significant difference in intubating conditions when either device was used to determine the timing of endotracheal intubation. Conclusions The onset of neuromuscular blockade was longer when measured with TetraGraph than TOFscan, and a train-of-four count of zero in either device was a useful indicator for adequate intubating conditions. Clinical trial number and registry URL NCT05120999, https://clinicaltrials.gov/ct2/show/NCT05120999.
Subject(s)
Humans , Adult , Neuromuscular Nondepolarizing Agents , Neuromuscular Blockade , Neuromuscular Blocking Agents , Prospective Studies , Neuromuscular Monitoring , Rocuronium , Androstanols , AnestheticsABSTRACT
INTRODUCTION AND AIMS: Serum electrolyte derangements are common in patients with decompensated cirrhosis hospitalized for hepatic encephalopathy. There are limited data describing the association between electrolyte levels and outcomes in hepatic encephalopathy. We assessed the association between initial serum electrolyte values and outcomes in patients with hepatic encephalopathy. MATERIAL AND METHODS: A total of 385 consecutive patients hospitalized with encephalopathy were included in the study. Baseline electrolyte levels (sodium, potassium, chloride, bicarbonate, calcium and phosphorus) were measured at the time of admission and assessed for association with outcomes, which included survival, admission to the intensive care unit, requirement for mechanical ventilation, and length of hospital stay. P-values ≤ 0.0083 were considered significant after adjustment for multiple testing. RESULTS: In unadjusted analysis, significant associations were identified regarding both bicarbonate and phosphorus (admission to intensive care unit), and calcium (mechanical ventilation); however these findings weakened and no longer approached statistical significance when adjusting for confounding variables. No other significant associations between serum electrolyte measurements and outcomes were observed. CONCLUSIONS: Our findings suggest that in patients hospitalized with encephalopathy, serum electrolyte measurements are not strong predictors of patient outcome.
Subject(s)
Electrolytes/blood , Hepatic Encephalopathy/blood , Liver Cirrhosis/complications , Patient Admission , Adult , Aged , Aged, 80 and over , Biomarkers/blood , Female , Hepatic Encephalopathy/diagnosis , Hepatic Encephalopathy/etiology , Hepatic Encephalopathy/therapy , Hospital Mortality , Humans , Length of Stay , Liver Cirrhosis/blood , Liver Cirrhosis/diagnosis , Liver Cirrhosis/therapy , Male , Middle Aged , Predictive Value of Tests , Respiration, Artificial , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome , Young AdultABSTRACT
Surgical site infections (SSIs) after liver transplantation (LT) are associated with an increased risk of graft loss and death. The incidence of SSIs after LT and their risk factors have been determined for first LT but not for second LT. The importance of reporting the incidence of SSIs risk-stratified by first LT versus second LT is not known. All patients undergoing second LT at a single institution between 2003 and 2011 (n = 152) were reviewed. The Kaplan-Meier method was used to estimate the cumulative SSI incidence. Relative risks (RRs) and 95% confidence intervals (CIs) from Cox proportional hazards regression models were used to evaluate associations of potential risk factors with SSIs after second LT. Thirty-one patients developed SSIs (6 superficial SSIs, 1 deep SSI, and 24 organ/space SSIs). The cumulative incidence of SSIs 30 days after LT was 20.8% (95% CI = 14%-27%), which was slightly but not significantly higher than the previously reported incidence of SSIs after first LT at our institution between 2003 and 2008 (16%, RR = 1.32, 95% CI = 0.90-1.93, P = .16). Units of transfused red blood cells [RR (doubling) = 1.38, 95% CI = 1.02-1.86, P = .04] and hepaticojejunostomy (RR = 2.22, 95% CI = 1.05-4.72, P = .04) were the only factors associated with SSIs after second LT in single-variable analysis. The associations weakened in a multivariate analysis (P = .07 and P = .07, respectively), potentially because of the correlation of red blood cell transfusions and hepaticojejunostomy (P = .08). In conclusion, the incidence of SSIs after second LT was slightly higher but not significantly different than the published incidence of SSIs (16%) after first LT at the same institution. Significant independent risk factors for SSIs after second LT were not identified. Risk stratification for retransplantation may not be necessary when the incidence of SSIs after LT is being reported.
Subject(s)
Liver Transplantation/adverse effects , Reoperation/adverse effects , Surgical Wound Infection/diagnosis , Surgical Wound Infection/epidemiology , Adult , Aged , Aged, 80 and over , Erythrocyte Transfusion , Female , Follow-Up Studies , Humans , Incidence , Jejunostomy , Kaplan-Meier Estimate , Liver Transplantation/methods , Male , Middle Aged , Multivariate Analysis , Proportional Hazards Models , Regression Analysis , Reoperation/methods , Retrospective Studies , Risk Factors , Treatment Outcome , Young AdultABSTRACT
OBJECTIVE: To analyze the benefit of voiding chain cystourethrography (VCC) [placing a radiographic opaque chain into the urethra and bladder and asking the patient to void under fluoroscopy] in the urodynamic evaluation of female bladder outlet obstruction (BOO). MATERIALS AND METHODS: Females with post anti-incontinence operation voiding dysfunction who underwent urodynamic evaluation augmented with VCC and later had urethrolysis were identified. Six diagnostic criteria for obstruction were applied to each patient: (1) VCC ( obstructed: chain was angulated and could not be voiding out) (2) Video urodynamic study (VUDS) (detrusor contraction combined with radiographic obstruction) (3) maximum flow (Qmax) ≤ 15 cc/sec, detrusor pressure (pDet)@ Qmax ≥ 20 cm H20 (4) Qmax ≤ 11 cc/sec, pDet@ Qmax ≥ 25 cm H20 (5) Qmax ≤ 12 cc/sec, pDet@ Qmax ≥ 25 cm H20 (6) Blaivas-Groutz (B-G) nomogram. Urethrolysis results were reviewed. Agreement in assessment of BOO criteria was assessed by estimating the proportion of pair-wise agreements along with an exact binomial 95% confidence interval (CI) and by estimating kappa along with a 95 % CI. RESULTS: Twenty-one patients were identified. Twenty of the 22 urethrolyses (91%) were clinically successful. Diagnosis of BOO was most common for VCC (86 %) and then B-G Nomogram (67 %). Agreement with the VCC was relatively poor for each of the five other methods (14% -62%) with the video urodynamic study (VUDS) being the best. Three patients with successful urethrolysis were diagnosed only by the VCC. All of kappa values regarding agreement with the VCC were low; the highest value of 0.15 was observed for VUDS. CONCLUSION: VCC may augment selection criteria for urethrolysis.
Subject(s)
Fiducial Markers , Urethra/diagnostic imaging , Urinary Bladder Neck Obstruction/diagnosis , Urinary Bladder/diagnostic imaging , Urodynamics/physiology , Adult , Aged , Aged, 80 and over , Female , Humans , Middle Aged , Radiography , Reference Values , Reproducibility of Results , Urethra/surgery , Urinary Bladder Neck Obstruction/diagnostic imaging , Urinary Incontinence/surgeryABSTRACT
Objective To analyze the benefit of voiding chain cystourethrography (VCC) [placing a radiographic opaque chain into the urethra and bladder and asking the patient to void under fluoroscopy] in the urodynamic evaluation of female bladder outlet obstruction (BOO). Materials and Methods Females with post anti-incontinence operation voiding dysfunction who underwent urodynamic evaluation augmented with VCC and later had urethrolysis were identified. Six diagnostic criteria for obstruction were applied to each patient: (1) VCC ( obstructed: chain was angulated and could not be voiding out) (2) Video urodynamic study (VUDS) (detrusor contraction combined with radiographic obstruction) (3) maximum flow (Qmax) ≤ 15 cc/sec, detrusor pressure (pDet)@ Qmax ≥ 20 cm H20 (4) Qmax ≤ 11 cc/sec, pDet@ Qmax ≥ 25 cm H20 (5) Qmax ≤ 12 cc/sec, pDet@ Qmax ≥ 25 cm H20 (6) Blaivas-Groutz (B-G) nomogram. Urethrolysis results were reviewed. Agreement in assessment of BOO criteria was assessed by estimating the proportion of pair-wise agreements along with an exact binomial 95% confidence interval (CI) and by estimating kappa along with a 95% CI. Results Twenty-one patients were identified. Twenty of the 22 urethrolyses (91%) were clinically successful. Diagnosis of BOO was most common for VCC (86%) and then B-G Nomogram (67%). Agreement with the VCC was relatively poor for each of the five other methods (14%-62%) with the video urodynamic study (VUDS) being the best. Three patients with successful urethrolysis were diagnosed only by the VCC. All of kappa values regarding agreement with the VCC were low; the highest value of 0.15 was observed for VUDS. Conclusion VCC may augment selection criteria for urethrolysis. .