Sickle Cell Intrahepatic Cholestasis: Extremely Rare but Fatal Complication of Sickle Cell Disease.

Sickle cell intrahepatic cholestasis (SCIC) is a rare but potentially fatal complication of sickle cell disease (SCD), with high mortality, observed mainly in patients with homozygous sickle cell anemia. Herein, we have reported a case of severe SCIC with a poor outcome despite aggressive measures including exchange transfusion and use of vasopressors. The patient was admitted with generalized weakness, confusion, rigors, chills, and signs of hepatic failure, such as hyperbilirubinemia, hypoalbuminemia, and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. The patient received two exchange transfusions, but he continued to deteriorate clinically despite exchange transfusion and developed hemorrhagic shock and multiorgan failure. The patient was made comfort care as per family wishes. This case emphasizes the importance of early diagnosis of sickle cell intrahepatic cholestasis and poor prognosis despite aggressive measures.

The Effect of Atrial Fibrillation on Inpatient Outcomes of Patients with Acute Pancreatitis: A Two-year National Inpatient Sample Database Study.

Limited published data exist regarding the association of atrial fibrillation (AF) and acute pancreatitis. To test our hypothesis that AF increases mortality and clinical outcomes in patients with acute pancreatitis, we conducted a cross-sectional data review of the National Inpatient Sample (NIS) database. The NIS database was reviewed for the collection of data on patient hospitalizations in 2016 and 2017. Patients diagnosed with acute pancreatitis with and without concomitant AF were included in the analysis. The International Classification of Diseases, 10th revision coding system was used for the variables of interest. The Stata software program (StataCorp LLC, College Station, TX, USA) was used to perform statistical analyses. The chi-squared test or analysis of variance was used to identify differences in demographic characteristics between the groups. The study population included two groups of patients: those with acute pancreatitis only (n = 542,440) and those with both acute pancreatitis and AF (n = 32,790). The group with acute pancreatitis and AF had a two- to threefold higher rate of mortality [adjusted odds ratio (OR): 2.59; 95% confidence interval (CI): 2.04-3.28] and increased length of stay (adjusted OR: 1.28; 95% CI: 1.08-1.48). Also, significantly higher odds of sepsis (adjusted OR: 2.49; 95% CI: 2.06-3.01), congestive heart failure (adjusted OR: 3.16; 95% CI: 2.87-3.49), acute coronary syndrome (adjusted OR: 1.61; 95% CI: 1.17-2.21), stroke (adjusted OR: 3.94; 95% CI: 1.42-10.93), and acute kidney injury (adjusted OR: 1.42; 95% CI: 1.30-1.55) were observed in patients with acute pancreatitis and AF relative to in patients with acute pancreatitis only. Our results suggest AF increases mortality in patients with acute pancreatitis and that patients with acute pancreatitis and AF are at greater risk of worse clinical outcomes.

Coexisting Cirrhosis Worsens Inpatient Outcomes in Patients With Infective Endocarditis: A Cross-Sectional Analysis of the National Inpatient Sample 2013-2014.

Introduction Cirrhosis is known to be an important prognostic factor in determining morbidity and mortality in preoperative cardiac risk assessment for cardiac surgery. Data is limited on outcomes in patients with infective endocarditis (IE) and comorbid liver cirrhosis. The objective of our study is to evaluate the clinical outcomes in patients suffering from IE both with and without underlying liver cirrhosis as well as to determine rates of in-hospital mortality and factors that contribute to this outcome. Hypothesis Liver cirrhosis worsens clinical outcomes in patients with IE. Materials and methods Patients with a principal diagnosis of IE with and without liver cirrhosis were identified by querying the Healthcare Cost and Utilization (HCUP) database, specifically the National Inpatient Sample for the years 2013 and 2014 using International Classification of Diseases, Ninth Revision (ICD-9) codes. Results During 2013 and 2014, a total of 17,952 patients were admitted with a diagnosis of IE, out of whom 780 had concurrent liver cirrhosis. There was increased in-hospital mortality [15.6% vs 10.2%, aOR = 1.57 (1.27-1.93)], acute kidney injury [41.4% vs 32.6%, aOR = 1.45 (1.24-1.69)], and hematologic complications [32.1 vs 14.7%, aOR = 2.87 (2.44-3.37)] in patients with IE with liver cirrhosis when compared to patients with IE without liver cirrhosis. Patients having IE without liver cirrhosis underwent an increased number of interventions, i.e. aortic (7.2 vs 3.7%, aOR = 0.51 (0.34-0.76)) and mitral (4.9% vs 3.4%, aOR = 0.39 (0.23-0.69)) valvular replacements as compared to those with liver cirrhosis, which may explain the increased mortality seen in patients with liver cirrhosis. Conclusion Liver cirrhosis is an important prognostic risk factor for in-hospital mortality in patients with IE. The coagulopathic state in addition to increased rates of bleeding complications and renal dysfunction make these patients poor surgical candidates thus contributing to higher mortality. Further research into the individual risk factors contributing to the increased mortality rates in patients with IE and cirrhosis is required.

GILBERT'S SYNDROME - A CONCEALED ADVERSITY FOR PHYSICIANS AND SURGEONS.

Gilbert's syndrome (often abbreviated as GS) is most common hereditary cause of mild unconjugated (indirect) hyperbilirubinemia. Various studies have been published depicting clinical and pharmacological effects of Gilbert's syndrome (GS). However GS as a sign of precaution for physician and surgeons has not been clearly established. A systematic study of the available literature was done. Key words of Gilbert's syndrome, hyperbilirubinemia and clinical and pharmacological aspects of GS were searched using PubMed as search engine. Considering the study done in last 40 years, 375 articles were obtained and their abstracts were studied. The criterion for selecting the articles for through study was based on their close relevance with the topic. Thus 40 articles and 2 case reports were thoroughly studied. It was concluded that Gilbert's syndrome has immense clinical importance because the mild hyperbilirubinemia can be mistaken for a sign of occult, chronic, or progressive liver disease. GS is associated with lack of detoxification of few drugs. It is related with spherocytosis, cholithiasis, haemolytic anaemia, intra-operative toxicity, irinotecan toxicity, schizophrenia and problems in morphine metabolism. It also has profound phenotypic effect as well. The bilirubin level of a GS individual can rise abnormally high in various conditions in a person having Gilbert's syndrome. This can mislead the physicians and surgeons towards false diagnosis. Therefore proper diagnosis of GS should be ascertained in order to avoid the concealed adversities of this syndrome.