Klippel Feil Syndrome presenting with tricuspid regurgitation and cardiopulmonary distress secondary to dysplastic thoracic cage and spine: A case report.

Background: Klippel Feil syndrome is a rare multifactorial disease that occurs due to a combination of genetic and environmental factors. It is a complex disease that requires lifelong treatment by multidisciplinary teams. Case report: We present a case of a 15-year-old girl who presented with fever and shortness of breath and was found to have Klippel Feil Syndrome with a unique presentation of tricuspid regurgitation with cardiopulmonary distress secondary to dysplastic thoracic cage and spine. Discussion: Patients with Klippel feil syndrome are at increased risk for infection and cardiovascular problems. Proper surgical and medical management are required for patient wellbeing. Delay in diagnosis and management can be fatal with worse outcome.

Acute pancreatitis as a rare adverse event among cannabis users: A systematic review.

BACKGROUND: Cannabis use has been steadily rising in the United States and can have multiple adverse effects, including cannabis-induced acute pancreatitis. This study aims to collate and highlight the significant demographics, clinical presentation, and outcomes in patients with cannabis-induced acute pancreatitis. METHOD: A systematic literature search of electronic databases for peer-reviewed articles was conducted. After an initial search, we found 792 articles through different electronic databases. After manually removing duplicates and articles that did not meet the criteria, 25 articles were included in our review. RESULTS: A total of 45 patients were studied, 35 (78%) cases were male and 10 (22%) cases were female, showing male predominance. The mean age of all participants was 29.2 ± 10.3 years. The most common presenting symptoms were abdominal pain 21 of 21 (100%), nausea 17 of 21 (81%), and vomiting 12 of 20 (60%). Ultrasound was normal in the majority of patients, with findings of mild pancreatitis. Computerized tomography scans revealed pancreatic edema and inflammation in 7 of 20 (35%) patients, and findings of necrotizing pancreatitis and complex fluid collection were visualized in 3 of 20 (15%) patients. Dilatation of intrahepatic or extrahepatic biliary ducts was not seen in any patients. The overall prognosis was good, with reported full recovery. CONCLUSIONS: Cannabis should be included in the differential diagnosis for the etiology of acute pancreatitis, which would help in early intervention and treatment for the mitigation of the rapidly progressive disease.

Update on the applications and limitations of alpha-fetoprotein for hepatocellular carcinoma.

Alpha-fetoprotein (AFP) is an oncofetal glycoprotein that has been used as a tumor marker for hepatocellular carcinoma (HCC) in combination with ultrasound and other imaging modalities. Its utility is limited because of both low sensitivity and specificity, and discrepancies among the different methods of measurements. Moreover, its accuracy varies according to patient characteristics and the AFP cut-off values used. Combination of AFP with novel biomarkers such as AFP-L3, Golgi specific membrane protein (GP73) and des-gamma-carboxyprothrombin significantly improved its accuracy in detecting HCC. Increased AFP level could also signify severity of hepatic destruction and subsequent regeneration and is commonly observed in patients with acute and chronic liver conditions and cirrhosis. Hereditary and other non-hepatic disorders can also cause AFP elevation.

Does the Route of Cocaine Use Affect the Mortality and Outcomes of Cocaine-Induced Intestinal Ischemia? A Systematic Review.

Intestinal ischemia results from diminished perfusion of the colon resulting in tissue hypoxia. Anecdotal reports suggest that cocaine-induced intestinal ischemia has the highest mortality and longer length of stay among the vasoconstrictors. The present study aimed to summarize the available studies in the literature to assess the effect of routes of consumption on the outcomes of cocaine-induced intestinal ischemia. We conducted a systematic search of MEDLINE from inception through October 2019. Studies of cocaine-induced intestinal ischemia were included if data were available on comorbidities, mortality, and hospital length of stay (LOS). The study's primary outcomes were mortality and need for surgery, while secondary outcomes included the hospital length of stay, LACE index, and hospital score. Statistical tests used included linear and binary logistic regression. STATA 2015 was used, and P < 0.05 was statistically significant. Of the 304 studies, 8 case series and 45 case reports (n = 69 patients) met the inclusion criteria. Different routes of cocaine use had similar mortality odds and the need for surgery for intestinal ischemia. Hospital LOS showed significant difference among the subgroups. Readmission scores (LACE and hospital score) were higher for intravenous and smoking than ingestion and intranasal use (P < 0.05). In conclusion, different routes of cocaine use appear to have similar mortality odds for intestinal ischemia, which vary significantly among the different routes of cocaine consumption for the length of stay and readmission scores. Prompt recognition of the route of cocaine use is vital to improve the outcome. Large-scale and well-designed observational studies are needed to investigate this topic further.

Familial chylomicronemia syndrome: a case report.

BACKGROUND: Familial chylomicronemia is an extremely rare disease. Lipoprotein lipase deficiency, lipoprotein defect or lipoprotein receptor defect are the main genetic causes of familial chylomicronemia. CASE PRESENTATION: We report a rare case of hypertriglyceridemia which was diagnosed at 24 days after birth. A newborn south east Asian baby born for G3P2A1 mother was presented with hematuria at 24 days at the hospital. The patient's family history is significant for pink blood in an elder sibling who died within a few months of birth without a proper diagnosis. Physical examination was not significant for any findings. Urinalysis revealed numerous RBC in the urine. Blood draw to perform renal function test revealed a characteristic pink blood. Baby's blood was normal and red in color at the time of birth. During the present visit, although most of the blood test were not able to be carried out by the regular laboratory instruments, the patient's lipid profile was alarmingly high with triglyceride levels over 4000 mg/dL. Due to a very high triglyceride level in a neonate and a significant familial history, a genetic cause of hypertriglyceridemia is suspected. Upon diagnosis, baby was discontinued of breast feeding completely and was given a special diet devoid of triglyceride and containing medium chain fatty acid diet and was also started with fenofibrate. After a month and a half, follow up tests were conducted which showed the triglyceride level was reduced to 1300 and a reversal of the blood color from pink to red. Since the imported diet was extremely expensive for the family, the patient was put on skimmed milk with medium-chain triglyceride (MCT) oil. With 6 weeks of treatment, baby's condition has improved and is thriving well. CONCLUSIONS: Our case reports an extremely rare and fatal condition and illustrated the significance of timely diagnosis and intervention for saving the life of the baby.

Percutaneous Intervention or Bypass Graft for Left Main Coronary Artery Disease? A Systematic Review and Meta-Analysis.

BACKGROUND: The safety and efficacy of percutaneous coronary intervention (PCI) versus coronary artery bypass grafting (CABG) for stable left main coronary artery disease (LMCAD) remains controversial. METHODS: Digital databases were searched to compare the major adverse cardiovascular and cerebrovascular events (MACCE) and its components. A random effect model was used to compute an unadjusted odds ratio (OR). RESULTS: A total of 43 studies (37 observational and 6 RCTs) consisting of 29,187 patients (PCI 13,709 and CABG 15,478) were identified. The 30-day rate of MACCE (OR, 0.56; 95% CI, 0.42-0.76; p = 0.0002) and all-cause mortality (OR, 0.52; 95% CI, 0.30-0.91; p = 0.02) was significantly lower in the PCI group. There was no significant difference in the rate of myocardial infarction (MI) (p = 0.17) and revascularization (p = 0.12). At 5 years, CABG was favored due to a significantly lower rate of MACCE (OR, 1.67; 95% CI, 1.18-2.36; p = <0.04), MI (OR, 1.67; 95% CI, 1.35-2.06; p = <0.00001), and revascularization (OR, 2.80; 95% CI, 2.18-3.60; p = <0.00001), respectively. PCI was associated with a lower overall rate of a stroke, while the risk of all-cause mortality was not significantly different between the two groups at 1- (p = 0.75), 5- (p = 0.72), and 10-years (p = 0.20). The Kaplan-Meier curve reconstruction revealed substantial variations over time; the 5-year incidence of MACCE was 38% with CABG, significantly lower than 45% with PCI (p = <0.00001). CONCLUSION: PCI might offer early safety advantages, while CABG provides greater durability in terms of lower long-term risk of ischemic events. There appears to be an equivalent risk for all-cause mortality.

Recurrent Pheochromocytoma in an Elderly Patient.

Pheochromocytomas are rare neuroendocrine tumors that can affect people of all ages and are commonly diagnosed in the 4th and 5th decades of life. Familial pheochromocytomas are diagnosed mostly between the 2nd and 3rd decades of life. They can be benign or metastatic and often present as isolated tumors or along with other neuroendocrine syndromes. We present a case of an elderly man who underwent laparoscopic adrenalectomy for pheochromocytoma at the age of 60 years but developed recurrence of metastatic pheochromocytoma after ten years. We also conducted a literature review to understand the epidemiology and presentation of the tumor and to emphasize that there should be a low threshold of suspicion for timely diagnosis and management of recurrent pheochromocytoma.

Diagnosis and Management of Lemierre's Syndrome Presented with Multifocal Pneumonia and Cerebral Venous Sinus Thrombosis.

A 27-year-old female patient initially presented with fever, myalgia, sore throat that progressed to multifocal pneumonia, and cerebral sinus venous thrombosis. A combination of upper respiratory symptoms with tooth infection, positive blood culture for Fusobacterium nucleatum, computed tomography (CT) chest finding of multifocal pneumonia, and magnetic resonance imaging (MRI) finding of internal jugular vein thrombosis (IJVT) and cerebral venous sinus thrombosis (CVST) suggested Lemierre syndrome. The patient was managed with fluids, antibiotics, and anticoagulants. The patient survived and discharged from the hospital. The patient's symptoms improved at 2 months of follow-up.

Comparison of Glycemic Variability Indices: Blood Glucose, Risk Index, and Coefficient of Variation in Predicting Adverse Outcomes for Patients Undergoing Cardiac Surgery.

OBJECTIVES: Fluctuations in blood glucose (glycemic variability) increase the risk of adverse outcomes. No universally accepted tool for glycemic variability exists during the perioperative period. The authors compared 2 measures of glycemic variability-(1) coefficient of variation (CV) and (2) the Blood Glucose Risk Index (BGRI)-in predicting adverse outcomes after cardiac surgery. DESIGN: Prospective, observational study. SETTING: Single-center, teaching hospital. PARTICIPANTS: A total of 1,963 adult patients undergoing cardiac surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative blood glucose levels were measured hourly for the first 24 hours and averaged every 4 hours (4, 8, 12, 16, 20, and 24 hours). Glycemic variability was measured by CV and the BGRI. The primary outcome, major adverse events (MAEs), was a predefined composite of postoperative complications (death, reoperation, deep sternal infection, stroke, pneumonia, renal failure, tamponade, and myocardial infarction). Logistic regression models were constructed to evaluate the association. Predictive ability was measured using C-statistics. Major adverse events were seen in 170 (8.7%) patients. Only the fourth quartile of CV showed association (odds ratio [OR] 1.91; 95% confidence interval [CI] [1.19-3.14]; p = 0.01), whereas BGRI was related significantly to MAE (OR 1.20; 95% CI [1.10-1.32]; p < 0.0001). The predictive ability of CV and BGRI increased on adding the standard Society of Thoracic Surgeons (STS) risk index. The C-statistic for STS was 0.68, whereas STS + CV was 0.70 (p = 0.012) and STS + BGRI was 0.70 (p = 0.012). CONCLUSION: Both CV and the BGRI had good predictive ability. The BGRI being a continuous variable could be a preferred measure of glycemic variability in predicting adverse outcomes (cutoff value 2.24) after cardiac surgery.

Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.

Guided meditation as an adjunct to enhance postoperative recovery after cardiac surgery: study protocol for a prospective randomized controlled feasibility trial.

BACKGROUND: Cardiac surgical procedures are associated with postoperative neurological complications such as cognitive decline and delirium, which can complicate recovery and impair quality of life. Perioperative depression and anxiety may be associated with increased mortality after cardiac surgeries. Surgical prehabilitation is an emerging concept that includes preoperative interventions to potentially reduce postoperative complications. While most current prehabilitation interventions focus on optimizing physical health, mind-body interventions are an area of growing interest. Preoperative mind-body interventions such as Isha Kriya meditation, may hold significant potential to improve postsurgical outcomes. METHODS: This is a prospective, randomized controlled feasibility trial. A total of 40 adult patients undergoing cardiac surgery will be randomized to one of three study groups. Participants randomized to either of the two intervention groups will receive meditative intervention: (1) commencing two weeks before surgery; or (2) commencing only from the day after surgery. Meditative intervention will last for four weeks after the surgery in these groups. Participants in the third control group will receive the current standard of care with no meditative intervention. All participants will undergo assessments using neurocognitive, sleep, depression, anxiety, and pain questionnaires at various time points in the perioperative period. Blood samples will be collected at baseline, preoperatively, and postoperatively to assess for inflammatory biomarkers. The primary aim of this trial is to assess the feasibility of implementing a perioperative meditative intervention program. Other objectives include studying the effect of meditation on postoperative pain, sleep, psychological wellbeing, cognitive function, and delirium. These will be used to calculate effect size to design future studies. DISCUSSION: This study serves as the first step towards understanding the feasibility of implementing a mind-body intervention as a prehabilitative intervention to improve postoperative surgical outcomes after cardiac surgery. TRIAL REGISTRATION: Clinicaltrials.gov, NCT03198039 . Registered on 23 June 2017.

The effect of a one-time 15-minute guided meditation (Isha Kriya) on stress and mood disturbances among operating room professionals: a prospective interventional pilot study.

Background: Operating room professionals are exposed to high levels of stress and burnout. Besides affecting the individual, it can compromise patient safety and quality of care as well. Meditation practice is getting recognized for its ability to improve wellness among various populations, including healthcare providers. Methods: Baseline stress levels of perioperative healthcare providers were measured via an online survey using a Perceived Stress Scale (PSS) questionnaire. An in-person meditation workshop was demonstrated during surgical grand rounds and an international anesthesia conference using a 15-minute guided Isha Kriya meditation. The participants were then surveyed for mood changes before and after meditation using a Profile of Mood States (POMS) questionnaire.  Results: Surgeons and anesthesiologists were found to have higher median (interquartile range) Perceived Stress Scores as compared to nurses respectively (17 [12, 20] and 17 [12, 21] vs 14 [9, 19]; P = 0.01). Total mood disturbances were found to be significantly reduced after meditation in both the surgical grand rounds (pre-meditation median [IQR] 99 [85, 112] vs 87 [80, 93] post-meditation; P < 0.0001) and anesthesia conference cohorts (pre-meditation 92 [86, 106] vs 87 [81, 92] post-meditation; P < 0.0001). Conclusions: Isha Kriya, a guided meditation, is easy to learn and takes less than 15 minutes to complete. This meditation technique improves mood changes and negative emotions among operating room professionals and could be used as a potential tool for improving wellness.

Significance of intra-operative blood pressure data resolution: A retrospective, observational study.

Background: With evolving techniques for analysis of blood pressure (BP) variability, the importance of sampling resolution for intra-operative BP still remains to be examined. This study aims at comparing BP data with beat-by-beat vs. 15 second resolution. Methods: This is a retrospective analysis of intra-arterial BP data obtained from cardiac surgical patients from the intra-operative period. Data was collected from two sources for each patient, one with beat-by-beat frequency, other at a frequency of once every 15 seconds. The fraction of time and area under the curve beyond systolic BP thresholds of 95 - 135 mmHg were calculated using data from both sources, for each patient. These were compared using Wilcoxon ranked sum test for paired samples using R-statistics version 3.4.3. Results: There was a statistically significant difference (P < 0.001) between the parameters from the two sources. This was especially true for parameters below and outside the thresholds. Only time fraction showed significant difference above the 135 mmHg threshold. Conclusion: Our preliminary analysis shows a definitive difference between BP descriptors, depending on sampling resolution. But the impact of this difference on the outcome predicting models of the parameters stands to be ascertained. Future larger studies, powered to examine the impact of sampling resolution on outcome predictive ability of BP descriptors, with special emphasis on dynamic markers of complexity are warranted.

Acute hepatitis E superinfection leading to chronic hepatitis B reactivation.

Reactivation of chronic hepatitis B (CHB) can be associated with significant morbidity and mortality. There are many different causes of hepatitis B reactivation. This case describes an Asian woman with stable CHB presenting with significant hepatitis flare with markedly elevated serum aminotransferases and hepatitis B virus DNA level. The clinical symptoms were subtle with fatigue and vague right upper quadrant tenderness. We ruled out drug-associated hepatotoxicity and screened for common causes of acute hepatitis. Interestingly, she was noted to have reactive anti-hepatitis E virus (HEV) IgM at initial presentation followed by anti-HEV IgG positivity a month later. The serological pattern confirmed the diagnosis of acute hepatitis E. The combination of antiviral therapy for hepatitis B and resolution of acute hepatitis E resulted in normalisation of serum aminotransferases. This case illustrates the importance of taking a careful history and having a high index of suspicion for various aetiologies when evaluating patients with reactivation of CHB.

Patients with common variable immunodeficiency paradoxically have increased rates of autoimmune disorders.

Common variable immunodeficiency (CVID), characterised by disordered B cell function, is one of the most common primary immunodeficiency disorders. Patients with CVID are at lifelong risk of recurrent infections, particularly of the respiratory and gastrointestinal tracts. Paradoxically, given their immunocompromised state, patients with CVID are also at significantly increased risk of autoimmune disorders, which are seen in almost 25% of cases. The authors report a 24-year-old female patient with CVID, manifested as severe hypogammaglobulinaemia with recurrent sinopulmonary infections and enterocolitis, who presented with transaminitis, chronic diarrhoea and haematemesis. No infectious aetiologies were identified. She was diagnosed with coeliac disease after a small bowel biopsy and positive response to gluten-free diet. Haematemesis was attributed to portal hypertension due to liver cirrhosis, which was confirmed via liver biopsy. Coeliac disease can be a cause of diarrhoea in patients with immunodeficiency disorders and is often underdiagnosed. It can also be the underlying source of liver disease and is an often under-recognised cause of cirrhosis. The case presented emphasises the paradoxical and challenging relationship that patients with CVID face between immunodeficiency and autoimmune disorders, and also highlights that coeliac disease is an under-recognised cause of liver disease.

The use of dexmedetomidine and intravenous acetaminophen for the prevention of postoperative delirium in cardiac surgery patients over 60 years of age: a pilot study.

Background: Delirium is associated with many negative health outcomes. Postoperative sedation and opioid administration may contribute to delirium. We hypothesize that the use of dexmedetomidine and Intravenous acetaminophen (IVA) may lead to reduced opioid consumption and decreased incidence of postoperative delirium. This pilot study aims to assess feasibility of using dexmedetomidine and IVA in cardiac surgical patients, and obtain effect size estimates for incidence and duration of delirium. Methods: A total of 12 adult patients >60 years of age undergoing cardiac surgery were recruited for the study after IRB approval and randomized into 4 groups: Propofol only (P), Propofol with IVA (P+A), Dexmedetomidine only (D), Dexmedetomidine with IVA (D+A). Preoperative baseline cognition and postoperative delirium was assessed daily until discharge. The feasibility was assessed by the number of patients who successfully completed the study. Results: All patients completed the study protocol successfully. The total incidence of delirium in the study population was 42% (5/12):  67% (2/3) in the group P, and 67% (2/3) in the group D, 33% (1/3) in  D+A group and 0%(0/3)  P+A group. The incidence of delirium was 17% (1/6) in the group receiving IVA compared to 67% (4/6) that did not receive IVA. The mean duration of delirium was 0-1 days. One patient expired after surgery, unrelated to the study protocol. One patient in the D group experienced hypotension with systolic blood pressure <90 mm of Hg. Conclusions: The feasibility of performing a large-scale project is ascertained by the study. Patients receiving IVA had lower incidence of delirium compared to patients not receiving IVA which suggests that IVA may have a role in reducing the incidence of delirium. A prospective randomized, placebo-controlled trial will be the next step in investigating the role of dexmedetomidine and IVA in reducing the incidence of delirium.

Extended-Duration Thromboprophylaxis Among Acute Medically Ill Patients: An Unmet Need.

Acute medical illnesses are associated with a prolonged elevation in inflammatory markers that predisposes patients to thrombosis beyond the duration of their hospital stay. In parallel, both observational and randomized data have demonstrated a rate of postdischarge venous thromboembolic events that often exceeds that observed in the hospital setting. Despite this significant residual risk of venous thromboembolic events following discharge among acute medically ill patients, no therapeutic strategies have been recommended to address this unmet need. Available randomized trials have demonstrated the efficacy of extending the duration of thromboprophylaxis with available anticoagulants; however, the efficacy is offset, at least in part, by an increase in bleeding events. Identification of the optimal therapeutic strategies, treatment duration, and risk assessment tools that reconcile both efficacy and safety of extended-duration thromboprophylaxis among acute medically ill patients is an area of ongoing investigation.