Comparison of inpatient outcomes in patients with Hepatitis B, Hepatitis C, and Hepatitis B and C co-infection with Cirrhosis.

BACKGROUND: Hepatitis B (HBV) and Hepatitis C (HCV) are among the most common causes of cirrhosis in the USA, with high mortality and morbidity but comparative outcomes were not well studied. METHODS: We retrospectively analyzed cirrhosis patients with HBV, HCV, and HBV/HCV coinfection from 2016 to 2019 in National Inpatient Sample (NIS) database. Our primary outcome was the length of stay (LOS), mean hospital charge and mortality. RESULTS: Our study included 701464 cirrhosis patients with HCV (89.7%), HBV (6.8%), and coinfection (3.5%) (P < 0.001). Male gender and white race were more common in all three cohorts (p < 0.001). The mean age for HBV, HCV, and coinfection was 55.59, 58.69, and 58.27 years. The mean LOS for HBV, HCV, and coinfection were 6.59 ± 0.1, 6.02 ± 0.03, and 6.74 ± 0.12 days respectively. The adjusted length of stay was 0.62 days longer in the HBV cohort and 0.61 days longer in the coinfection cohort, compared to the HCV cohort (P < 0.001). Adjusted hospital charges were $15112 higher in the HBV cohort and $ 6312 higher in the coinfection cohort, compared to the HCV cohort (P < 0.001). Patients with HBV had a higher risk of mortality compared to HCV infection (AOR 1.35, [1.22-1.48], P < 0.001); However, patients with coinfection had no difference in mortality compared to HCV infection. CONCLUSION: Cirrhosis with HBV and coinfection is associated with increased duration of hospital stay and cost when compared to HCV infection. There is a higher risk of mortality in cirrhotic patients with HBV infection compared to HCV; however, no significant difference in mortality for coinfection compared to HCV.

Endoscopic sleeve gastroplasty reintervention score using supervised machine learning.

BACKGROUND AND AIMS: Reintervention after endoscopic sleeve gastroplasty (ESG) can be indicated because of postprocedural adverse events from various preinterventional or postprocedural comorbidities. We developed and internally validated an ESG reintervention score (ESG-RS) that determines the individualized risk of reintervention within the first 30 days after ESG. METHODS: We used data from a sample of 3583 patients who underwent ESG in the Metabolic and Bariatric Surgery Accreditation Quality Improvement Program database (2016-2021). The least absolute shrinkage and selection operator (LASSO)-penalized regression was used to select the most promising predictors of reintervention after ESG within 30 days. The predictive variables extracted by LASSO regression were entered into multivariate analysis to generate an ESG-RS by using the coefficients of the statistically significant variables. The model performance was assessed using receiver-operator curves by 10-fold cross-validation. RESULTS: Eleven variables were selected by LASSO regression and used in the final multivariate analysis. The ESG-RS was inferred using 5 factors (history of previous foregut surgery, preoperative anticoagulation use, female gender, American Society of Anesthesiologists class ≥II, and hypertension) weighted by their regression coefficients in the multivariable logistic regression model. The area under the curve of the ESG-RS was .74 (95% confidence interval, .70-.78). For the ESG-RS, the optimal cutpoint was 67.9 (high risk vs low risk), with a sensitivity of .76 and specificity of .71. CONCLUSIONS: The ESG-RS aids clinicians in preoperative risk stratification of patients undergoing ESG while clarifying factors contributing to a higher risk of reintervention.

Racial and gender-based disparities and trends in common psychiatric conditions in liver cirrhosis hospitalizations: A ten-year United States study.

BACKGROUND: Chronic liver disease is associated with various neuropsychiatric conditions. There are currently no large studies assessing and comparing the prevalence of psychiatric illnesses based on patient profiles and the etiology of cirrhosis. AIM: To examine the trends of hospitalizations among psychiatric conditions in cirrhosis. METHODS: We used the National Inpatient Sample database 2016-2019 for the primary diagnosis of liver cirrhosis. The outcomes included the prevalence, trends, and associations of psychiatric diagnoses in these hospitalizations. Chi-square for categorical variables and the Wilcoxon rank test for continuous variables were utilized. RESULTS: The prevalence of generalized anxiety disorder (GAD) in liver cirrhosis hospitalizations increased from 0.17% in 2009 to 0.92% in 2019 (P < 0.001). The prevalence of depression increased from 7% in 2009 to 12% in 2019 (P < 0.001). Attention deficit hyperactivity disorder (ADHD) prevalence increased from 0.06% to 0.24%. The prevalence of schizophrenia increased from 0.59% to 0.87% (P < 0.001). Schizoaffective disorder prevalence increased from 0.10% to 0.35% (P < 0.001). Post-traumatic stress disorder (PTSD) prevalence displayed increasing trends from 0.36% in 2009 to 0.93% in 2019 (P < 0.001). The prevalence of suicidal ideation increased from 0.23% to 0.56% in 2019. Cirrhosis related to alcoholic liver disease [adjusted odds ratios (aOR) 1.18, 95%CI 1.08-1.29, P < 0.001] and non-alcoholic fatty liver disease (NAFLD) (aOR 1.14, 95%CI 1.01-1.28, P = 0.025) was associated with depression more than other causes. Alcohol- and NAFLD-associated cirrhosis had a stronger link to psychiatric disorders. Females had a higher association with GAD (aOR 2.56, 95%CI 2.14-3.06, P < 0.001), depression (aOR 1.78, 95%CI 1.71-1.84, P < 0.001), bipolar disorder (aOR 1.64, 95%CI 1.52-1.77, P < 0.001] and chronic fatigue (aOR 2.31, 95%CI 1.31-4.07, P < 0.001) when compared to males. Blacks, Hispanics, and Asian/Native Americans had a significantly lower association with GAD, depression, bipolar disorder, PTSD, and ADHD when compared to the white race. CONCLUSION: The prevalence of psychiatric comorbidities in liver cirrhosis hospitalizations has increased over the last decade. Females had a higher association with psychiatric disorders compared to males. Blacks, Hispanics, and Asian/Native Americans had lower associations with psychiatric comorbidities compared to the white race.

Substance abuse and inpatient outcomes in inflammatory bowel disease hospitalizations in the United States: a propensity matched analysis.

Background: Inflammatory bowel disease (IBD) is a chronic intestinal inflammation resulting in a genetically susceptible population. The present study aimed to look at the effect of substance abuse on IBD hospitalizations in the United States. Methods: We identified primary IBD hospitalizations with substance abuse using the National Inpatient Sample database (2016-2019). A matched comparison cohort of IBD hospitalizations without substance abuse was identified by 1:N propensity score matching using the nearest-neighbor method, based on demographics, hospital-level factors, and comorbidities. Results: We matched 4437 IBD hospitalizations with a diagnosis of substance abuse to 4528 hospitalizations without abuse. The median age was higher in the substance abuse group than no abuse (44 vs. 38 years, P<0.001). There was a higher prevalence of discharge to care facilities (2.9% vs. 2.2%) and against medical advice (4.9% vs. 1.8%) in the substance abuse group compared to the no abuse (P<0.001). The median length of hospital stays (LOS) (P=0.74) and hospitalization charge did not differ significantly (P=0.57). There was no significant difference in 30-day inpatient mortality among cohorts (adjusted hazard ratio 0.74, 95% confidence interval 0.32-1.81; P=0.54). There was a higher prevalence of psychoses (2.5% vs. 1.3%) and depression (18.8% vs. 15.7%) in IBD hospitalizations with substance abuse compared to those without abuse (P<0.001). Conclusions: This study reports no difference in median LOS, hospitalization charge, or mortality risk in IBD hospitalizations based on substance abuse. There is a higher prevalence of psychoses and depression in IBD patients, requiring screening for substance abuse to improve overall outcomes.

Inpatient outcomes and racial disparities of palliative care consults in mechanically ventilated patients in the United States.

Intensive care units (ICUs) account for a disproportionately large share of healthcare utilization. Our study examined the association between palliative care consults (PCC) and hospital outcomes in mechanically ventilated patients. We analyzed patients admitted from 2016 to 2019 using the National Inpatient Sample database. The primary outcome was the association of PCC and length of stay; secondary outcomes included the impact of PCC on total hospital costs. Of the 2,351,503 patients included, 15.5% had a PCC, with a male predominance (53%, P < 0.001). Whites had a higher PCC rate, at 167 per 1000 ICU cases, vs. Blacks, at 25 per 1000 cases (P < 0.001). Adjusted length of stay was 2.0 days less in patients with PCC (P < 0.001), and adjusted inpatient hospital cost was $12,942 lower in patients with PCC (P < 0.001). Whites had a larger decrease in length of stay and costs compared to blacks, Hispanics, and Asians (P < 0.001). In conclusion, PCC was associated with a shorter length of stay and lower inpatient hospital costs in critically ill ICU patients. Black patients saw a lower impact of PCC on LOS and hospital costs, as well as a lower rate of PCC.

Comparative analysis and trends in liver transplant hospitalizations with Clostridium difficile infections: A 10-year national cross-sectional study.

GOALS AND BACKGROUND: Clostridium difficile infection (CDI) is the leading cause of antibiotic-associated diarrhea in the United States. We aimed to determine comparative trends in inpatient outcomes of liver transplant (LT) patients based on CDI during hospitalizations. METHODS: The national inpatient sample database was used to conduct the present retrospective study regarding CDI among the LT hospitalizations from 2009 to 2019. Primary outcomes included 10-year comparative trends of the length of stay (LOS) and mean inpatient charges (MIC). Secondary outcomes included comparative mortality and LT rejection trends. RESULTS: There was a 14.05% decrease in CDI in LT hospitalizations over the study period (p = .05). The trend in LOS did not significantly vary (p = .9). MIC increased significantly over the last decade in LT hospitalizations with CDI (p < .001). LT hospitalizations of autoimmune etiology compared against non-autoimmune did not increase association with CDI, adjusted odds ratio (aOR) 0.97 (95% confidence interval [CI] 0.75-1.26, p = .87). CDI was associated with increased mortality in LT hospitalizations, aOR 1.84 (95% CI 1.52-2.24, p < .001). In-hospital mortality for LT hospitalizations with CDI decreased by 7.75% over the study period (p = .3). CDI increased transplant rejections, aOR 1.3 (95% CI 1.08-1.65, p < .001). There was a declining trend in transplant rejection for LT hospitalization with CDI from 5% to 3% over the study period (p = .0048). CONCLUSION: CDI prevalence does not increase based on autoimmune LT etiology. It increases mortality in LT hospitalizations; however, trend for mortality and transplant rejections has been declining over the last decade.

Crohn's Disease with Fistula: 10 Year Trends and Mortality in the United States.

Background/Aims: Crohn's disease (CD) results in significant morbidity, mortality, and healthcare burden. This study evaluated the temporal trends of CD hospitalizations with a fistula over the last decade to understand the outcomes of severe CD. Methods: National Inpatient Sample database from 2009 to 2019 was used to identify CD hospitalizations with a fistula. The outcomes of interest included temporal trend analysis of length of stay (LOS), mean inpatient cost (MIC), and mortality. Results: There was an increase in the total number of fistulizing CD hospitalizations from 5,386 in 2009 to 12,900 in 2019 (p<0.01). The mean age decreased from 44.9±0.6 to 41.8±0.4 years for the study period (p<0.01). Caucasians were the predominant ethnicity, followed by Africans, Hispanics, and others (p<0.001). The mean LOS for fistulizing CD hospitalizations decreased significantly from 11.57±0.09 days in 2009 to 7.20±0.05 days in 2019 (p<0.001). There was a significant decrease in inpatient mortality from 1.72% in 2009 to 0.73% in 2019 (p<0.01). The MIC did not have a significant trend. There was a decreasing trend toward partial/total colectomies, rectosigmoid, and small bowel surgeries from 2009 to 2019 (p<0.001). Conclusions: There has been a rise in CD hospitalizations with fistulizing disease in the last decade. Despite this, the mortality and inpatient LOS have been decreasing significantly. In addition, the increase in CD hospitalizations with fistulizing disease has had no significant effect on hospital costs.

Ten-year trends and prediction model of 30-day inpatient mortality for alcoholic hepatitis in the United States.

Background: Alcoholic hepatitis (AH) results in significant morbidity, mortality and healthcare burden. We aimed to evaluate the temporal trends of AH hospitalizations in the last decade and to devise a mortality scoring system for risk stratification. Methods: National Inpatient Sample (NIS) databases from 2009-2019 were used to identify AH hospitalizations. Outcomes of interest included temporal trend analysis of length of stay (LOS), mean inpatient cost (MIC), mortality, and mortality predictors. A mortality scoring system was derived using multivariate Cox regression and validated using receiver operating characteristic curves. Results: There was an increase in total AH hospitalizations, from 67,070 in 2009 to 125,540 in 2019 (P=0.004). The inpatient mortality increased from 2.48% in 2009 to 3.78% in 2019 (P=0.008). The MIC was $31,189 in 2009 and $62,229 in 2019 (P<0.001). A trend for LOS was not significant. Ten variables were selected for incorporation into a risk score, including anemia, age >60 years, female sex, mechanical ventilation, vasopressor use, spontaneous bacterial peritonitis, hepatorenal syndrome, acute renal failure, coagulopathy (thrombocytopenia), and hepatic encephalopathy. The score has a maximum of eight points, and the cutoff for predicting mortality was set as 4 points. The area under the curve (AUC) of the derivation cohort was 0.8766 (95% confidence interval [CI] 0.865-0.888) and AUC 0.862 (95%CI 0.855-0.868) for a 30-day period. Conclusions: There has been an increase in AH hospitalizations and mortality in the last decade. The Tahira score provides an easy objective method to estimate inpatient 30-day mortality for AH hospitalizations.

Effect of constipation on outcomes in mechanically ventilated patients.

Constipation can be a significant clinical challenge that can compromise management plans and prolong hospital stays. Our goal was to examine the effects of constipation on mechanically ventilated patients, with outcomes related to inpatient stays. We retrospectively analyzed critically ill patients hospitalized with constipation in the 2016 to 2019 National Inpatient Sample (NIS) database. Constipation was defined using Rome IV criteria. Critically ill patients were defined as mechanically ventilated from admission day 1. Our primary outcome was length of stay (LOS) and total hospital charge. Secondary outcomes included predictors of mortality in critically ill patients with constipation. The study included 2,351,119 weighted discharges of mechanically ventilated patients in the NIS database. Of these, 3.7% had constipation. The adjusted LOS was 3.4 days longer in patients with constipation vs those without it (P < 0.001). The adjusted inpatient hospital cost was $31,762 higher in patients with constipation (P < 0.001). Men had higher LOS and inpatient costs. Constipation was not associated with increased inpatient mortality (P < 0.001). Several conditions increased mortality in critically ill patients with constipation, including peritonitis, fecal impaction, and bowel obstruction.

Relation between inflammatory bowel disease, depression, and inpatient outcomes in the United States.

The relationship between inflammatory bowel disease (IBD) and depression is complicated. The effect of depression on ulcerative colitis (UC) and Crohn's disease (CD) among the inpatient US population has not previously been studied. We retrospectively analyzed patients admitted with UC and CD from 2016 to 2019 using the National Inpatient Sample database. Our primary outcome was the effect of depression on hospital length of stay (LOS), costs, and mortality. Secondary outcomes included the comparison between UC and CD cases. In the UC population, 13.4% had depression, compared to 14.9% in the CD population. LOS was longer in UC and CD patients with depression (P < 0.001). Subgroup analysis revealed that LOS was longer in CD patients than UC patients in the depressed cohort (P < 0.001). Inpatient hospital costs were lower in IBD patients with depression (P < 0.001). Subgroup analysis revealed that hospital cost was $17,974 higher in CD patients than UC patients (P < 0.001). Depression did not increase mortality in the IBD population but increased LOS, with a greater impact on CD than UC. White women were found to have an increased prevalence of depression in the IBD population.

Inpatient outcomes of Dieulafoy's lesions in the United States.

Dieulafoy's lesions are arguably underidentified rather than an infrequent cause of gastrointestinal bleeding. No population-based study exists regarding its inpatient outcomes in the United States. We evaluated the characteristics and inpatient outcomes for Dieulafoy's lesions using the National Inpatient Sample from 2016 to 2019. We identified 30,015 weighted hospitalizations for Dieulafoy's lesions. An initial diagnosis of Dieulafoy's lesions was established for 53.85% of patients on admission. The mean age was 68.7 ± 0.04 years, with male (56%) and white race predominance (70%). The mean length of stay and hospital cost were 7.87 days and $111,914, respectively. Significant predictors of inpatient mortality included heart failure, cardiac arrhythmias, coagulopathy, protein-calorie malnutrition, and alcoholism (P < 0.001). During inpatient hospitalization, 78% of patients underwent endoscopies, and 11% had colonoscopies. Inpatient mortality was 4.65%. Common comorbidities in Dieulafoy's lesions patients included heart failure (34%), cardiac arrhythmias (41%), hypertension (32%), chronic obstructive pulmonary disorders (25%), coagulopathic disorders (22%), and alcohol abuse (12%). Dieulafoy's lesions have a significant effect on length of stay and hospital cost. Endoscopies were used substantially more than colonoscopies for Dieulafoy's lesions, indicating a predominant presentation as upper gastrointestinal bleed. Cardiac disorders increase mortality in patients with Dieulafoy's lesions.

Diagnostic Accuracy for Per-Patient Polyp Detection of Second-Generation Capsule Endoscopy Compared to Colonoscopy: A Meta-Analysis of Multicenter Studies.

Colon capsule endoscopy (CCE) or capsule colonoscopy can be used as colorectal cancer (CRC) screening option. We intended to analyze the concerning literature that compared second-generation CCE to standard colonoscopy for multicenter studies only. A literature search was performed in PubMed, Embase, and Web of Science. Study characteristics related to our research including sensitivity and specificity for per-patient polyps detection (size: ≥ 10 mm and ≥ 6 mm). Meta-analysis was performed using an open meta-analyst. Our research included five studies, involving a total of 1518 patients, with a total of 1305 analyzed patients. The adequate bowel preparation rate ranged from 70% to 90%. The rates of complete CCE transit fluctuated from 80% to 100%. Our meta-analysis illustrated that mean (95% confidence interval) per-patient sensitivity, specificity, and diagnostic odds ratio were: 0.86 (0.82-0.91) (p < 0.001), 0.88 (0.72-0.96) (p < 0.001), and 50.7 (18.5-138.9) (p < 0.001), respectively, for polyps ≥ 6 mm; and 0.86 (0.8-0.91) (p < 0.001), 0.96 (0.92-0.98) (p < 0.001), and 173.5 (98.4-305.8) (p < 0.001), respectively, for polyps ≥ 10 mm. We concluded that CCE had high sensitivity and specificity for per-patient polyps vs. standard colonoscopy. Nevertheless, the comparatively higher rate of unfinished CCEs limits the utilization of CCE for CRC screening.

Pancreatic cancer incidence trends in the United States from 2000-2017: Analysis of Surveillance, Epidemiology and End Results (SEER) database.

Background: Recent incidence trends of pancreatic cancers were reviewed by demographics and histologic type to observe any new findings. Methods: Data was used from the Surveillance, Epidemiology, and End Results (SEER) registry 18 (2000-2017) and it underwent temporal trend analysis. Pancreatic cancer incidence rates were reported based on histological subtype and demographics. Results: The incidence rate of white males increased significantly during 2000-2017 (annual percent change (APC) = 3.5%) compared to previously reported APCs. The incidence of white females grew from an APC of 1.29% to 2.9%. Rates among black ethnicity increased with an APC of 4.2%. Rates among Hispanics and other ethnicities also showed increment. The rates for ductal adenocarcinoma showed a positive trend in all races, with the APC ≥ 6 % for females and APC ≥ 6.5 % for males. The rates of non-secretory endocrine tumors showed a decline in both genders of all five races in recent years after showing an initial positive trend till 2010. Rates for pancreatic adenocarcinoma continued to rise in all ethnicities from 2000-2017. Interestingly, there was a rise in carcinoid type pancreatic neuroendocrine tumors (PNETs) in all ethnicities. Cumulatively, males had a higher incidence than females; male to female Incidence Risk Ratio (IRRs) was 1.32. The IRR was > 1 for age groups ≥ 35 years. The male to female IRRs was less than 1 for cystic adenocarcinoma, secretory endocrine, and solid pseudopapillary carcinomas (IRR = 0.5, 0.9, and 0.2 respectively, confidence intervals 0.4-0.6 and 0.9-1.3, 0.2-0.3, respectively). Conclusion: Pancreatic cancer incidence continued to rise in the years 2000-2017. However, incidence differed by demographics and histologic type. Interestingly, recent years discerned a rise in PNETs (carcinoid type) which has not been reported previously.

Use of Endovascular Coiling to Stop Gastric Ulcer Bleeding in a Patient With Extensive Risk Factors.

Peptic ulcer disease (PUD) can lead to life-threatening bleeding. Endoscopy is a primary intervention used to locate the site of bleeding and maintain hemostasis. When considering multiple risk factors to operative intervention or failed initial endoscopic procedure in patients, the preferred treatment for acute gastrointestinal bleeding remains endovascular coiling to embolize the culprit's vessel. We report a case of a 57-year-old female who presents with melena secondary to gastric ulcer not amenable to endoscopic interventions. Various embolization techniques are available demanding clinicians' attention towards their role in managing ulcer bleeds and their impact on the controlling bleeds.

Role of Prucalopride in Treating Functional Constipation and Gastroparesis: A Systemic Review.

Prucalopride is a selective serotonin receptor agonist that can be used to treat chronic constipation. This article reviews the clinical efficacy side effects of prucalopride, assessing its role in constipation and gastroparesis. Relevant published medical literature was identified by using the search terms "constipation," "gastroparesis," and "prucalopride" from 2010 and onwards. The databases included PubMed/MEDLINE and EMBASE. Bibliographies from published literature and websites were also reviewed. Results were filtered for English language and randomized controlled trials. Out of the 18 results, abstracts were manually reviewed for studies with similar statistical methodology; eight studies were selected for constipation and two studies for gastroparesis. In two four-week trials, prucalopride showed improvement in gastric emptying and the gastroparesis cardinal symptom index over placebo, with a 1-4 mg/day dosage. In seven 12-week trials in patients with chronic constipation, oral prucalopride 2-4 mg/day was more significant than placebo to improve the number of bowel movements and symptoms. One study showed no significant bowel function differences when prucalopride was compared to placebo over 12 or 24 weeks. Prucalopride was generally well-tolerated, and the most common adverse events reported were headache, nausea, diarrhea, and abdominal pain. Further long-term and comparative data would be beneficial to show that prucalopride can be an advantageous treatment option for patients with chronic idiopathic constipation (CIC) or gastroparesis. Additionally, it would be interesting to see its effect on irritable bowel syndrome-constipation predominant, as it has some overlap with idiopathic constipation.

Psychological, physical, and sleep comorbidities and functional impairment in irritable bowel syndrome: Results from a national survey of U.S. adults.

BACKGROUND/AIMS: Patients with irritable bowel syndrome (IBS) in referral practice commonly report mental disorders and functional impairment. Our aim was to determine the prevalence of mental, physical and sleep-related comorbidities in a nationally representative sample of IBS patients and their impact on functional impairment. METHODS: IBS was defined by modified Rome Criteria based on responses to the chronic conditions section of the National Comorbidity Survey-Replication. Associations between IBS and mental, physical and sleep disorders and 30-day functional impairment were examined using logistic regression models. RESULTS: Of 5,650 eligible responders, 186 met criteria for IBS {weighted prevalence 2.5% (SE = 0.3)}. Age >60 years was associated with decreased odds (OR = 0.3; 95% CI:.1-.6); low family income (OR = 2.4; 95% CI:1.2-4.9) and unemployed status (OR = 2.3; 95% CI:1.2-4.2) were associated with increased odds of IBS. IBS was significantly associated with anxiety, behavior, mood disorders (ORs 1.8-2.4), but not eating or substance use disorders. Among physical conditions, IBS was associated with increased odds of headache, chronic pain, diabetes mellitus and both insomnia and hypersomnolence related symptoms (ORs 1.9-4.0). While the association between IBS and patients' role impairment persisted after adjusting for mental disorders (OR = 2.4, 95% CI 1.5-3.7), associations with impairment in self-care, cognition, and social interaction in unadjusted models (ORs 2.5-4.2) were no longer significant after adjustment for mental disorders. CONCLUSION: IBS is associated with socioeconomic disadvantage, comorbidity with mood, anxiety and sleep disorders, and role impairment. Other aspects of functional impairment appear to be moderated by presence of comorbid mental disorders.

Muscle-Specific Tyrosine Kinase-Associated Myasthenia Gravis: A Neuromuscular Surprise.

Myasthenia gravis is a neuromuscular autoimmune disease that results in skeletal muscle weakness that worsens after periods of activity and improves after rest. Myasthenia gravis means "grave (serious), muscle weakness." Although not completely curable, it can be managed well with a relatively high quality of life and expectancy. In myasthenia gravis, antibodies against the acetylcholine receptors at the neuromuscular junction interfere with regular muscular contraction. Although most commonly caused by antibodies to the acetylcholine receptor, antibodies against MuSK (muscle-specific kinase) protein can also weaken transmission at the neuromuscular junction. Muscle-specific tyrosine kinase myasthenia gravis (MuSK-Ab MG) is a rare subtype of myasthenia gravis with distinct pathogenesis and unique clinical features. Diagnosis can be challenging due to its atypical presentation as compared to seropositive myasthenia gravis. It responds inconsistently to steroids, but plasma exchange and immunosuppressive therapies have shown promising results. We report a case of a 49-year-old female who presented with acute hypoxic respiratory failure. Our patient experienced progressive, undiagnosed MuSK-Ab MG for years without a diagnosis.

Comparing Clinical, Imaging, and Physiological Correlates of Intestinal Pseudo-Obstruction: Systemic Sclerosis vs Amyloidosis and Paraneoplastic Syndrome.

INTRODUCTION: Intestinal pseudo-obstruction is characterized by impaired transit and luminal dilation in the absence of mechanical obstruction. Our study aims to describe the clinical, radiographic, and physiological findings in pseudo-obstruction associated with systemic sclerosis (SSc), amyloidosis, and paraneoplastic syndrome. METHODS: A retrospective cohort of patients evaluated at our institution between January 1, 2008, and August 1, 2018, was assembled. Clinical, imaging, and physiological characteristics were abstracted from electronic medical records. RESULTS: We identified 100 cases of pseudo-obstruction (55 SSc, 27 amyloidosis, and 18 paraneoplastic). Female population predominance was seen in SSc (71%) vs male population in amyloidosis (74%). Most common symptom was abdominal bloating in all 3 groups. Vomiting was more common in SSc than amyloidosis (73% vs 46%, P = 0.02). Diarrhea was more common in amyloidosis and SSc compared with paraneoplastic (81% and 67% vs 28%, P < 0.01). Weight loss (>5%) was more common in SSc compared with amyloidosis and paraneoplastic (78% vs 31% and 17%, P < 0.0001). Only small bowel dilation was seen in 79%, 40%, and 44% and only large bowel dilation in 2%, 44%, and 44% of patients in SSc, amyloidosis, and paraneoplastic, respectively. Five of 8 SSc patients had myopathic and 3 of 5 paraneoplastic had neuropathic involvement on gastroduodenal manometry. DISCUSSION: SSc-associated pseudo-obstruction demonstrates female population predominance and presents with vomiting, diarrhea, and weight loss. Amyloidosis-associated pseudo-obstruction shows male population predominance. Small bowel is more commonly involved than large bowel on both imaging and transit studies in SSc. Myopathic involvement was more common in SSc, contrary to neuropathic in paraneoplastic syndrome.