Safety and efficacy of EsoFLIP dilation in patients with esophageal dysmotility: a systematic review.

Esophageal manometry is utilized for the evaluation and classification of esophageal motility disorders. EndoFlip has been introduced as an adjunctive test to evaluate esophagogastric junction (EGJ) distensibility. Treatment options for achalasia and EGJ outflow obstruction (EGJOO) include pneumatic dilation, myotomy, and botulinum toxin. Recently, a therapeutic 30 mm hydrostatic balloon dilator (EsoFLIP, Medtronic, Minneapolis, MN, USA) has been introduced, which uses impedance planimetry technology like EndoFlip. We performed a systematic review to evaluate the safety and efficacy of EsoFLIP in the management of esophageal motility disorders. A systematic literature search was performed with Medline, Embase, Web of science, and Cochrane library databases from inception to November 2022 to identify studies utilizing EsoFLIP for management of esophageal motility disorders. Our primary outcome was clinical success, and secondary outcomes were adverse events. Eight observational studies including 222 patients met inclusion criteria. Diagnoses included achalasia (158), EGJOO (48), post-reflux surgery dysphagia (8), and achalasia-like disorder (8). All studies used 30 mm maximum balloon dilation except one which used 25 mm. The clinical success rate was 68.7%. Follow-up duration ranged from 1 week to a mean of 5.7 months. Perforation or tear occurred in four patients. EsoFLIP is a new therapeutic option for the management of achalasia and EGJOO and appears to be effective and safe. Future comparative studies with other therapeutic modalities are needed to understand its role in the management of esophageal motility disorders.

Hemorrhagic Ascites Is Associated With Reduced Survival in Cirrhosis: A Systematic Review and Meta-Analysis.

Background: Hemorrhagic ascites is characterized as red blood cell count greater than 10,000/mm3. In cirrhosis, ascites is an event of decompensation, and associated with poor prognosis. However, significance of hemorrhagic ascites is unclear. We conducted a systematic review and meta-analysis to evaluate the significance of hemorrhagic ascites in cirrhotic patients. Methods: We conducted a systematic search in Embase, MEDLINE, Cochrane Central Register of Controlled Trials, the World Health Organization (WHO) International Clinical Trial Registry, and Web of Science Core Collection to identify studies till March 2021, which, in patients with cirrhosis, compared outcomes amongst those with hemorrhagic ascites to those with non-hemorrhagic ascites. The primary outcome was 3-year mortality, and secondary outcomes were acute kidney injury (AKI), hepatic encephalopathy (HE), spontaneous bacterial peritonitis (SBP) and portal vein thrombosis (PVT). Results: Four studies, with 2,058 cirrhosis patients, were included. Among these, 1,488 patients had non-hemorrhagic ascites and 570 had hemorrhagic ascites. We observed no significant differences in AKI (odds ratio (OR) = 2.55; confidence interval (CI): 0.58 - 11.24), HE (OR = 2.52; CI: 0.70 - 9.05), SBP (OR = 1.66; CI: 0.12 - 22.83) and PVT (OR = 0.99; CI: 0.71 - 1.39). Intensive care unit (ICU) stay was significantly higher in patients with hemorrhagic ascites compared to those with non-hemorrhagic ascites (OR = 1.79; CI: 1.37 - 2.36; I2 = 56%). Pooled 3-year mortality was significantly higher in those with hemorrhagic (72.5% (CI: 68.2-76.4%)) when compared to non-hemorrhagic ascites (57.9% (CI: 55.2-60.6%)) (OR = 2.17; CI: 1.71 - 2.74) with low heterogeneity (I2 = 15%). Conclusions: In patients with cirrhosis, hemorrhagic ascites is a poor prognostic marker, which is associated with increased ICU stay and mortality. Prospective studies are needed to further evaluate significance of hemorrhagic ascites in patients with cirrhosis.

Safety and Efficacy of Endoscopic Retrograde Cholangiopancreatography in Nonagenarians: A Systematic Review and Meta-Analysis.

BACKGROUND: Recent advances in modern medicine have translated into increase in life expectancy in the USA and with that, a rise in the demand for invasive procedures in elderly patients. Endoscopic retrograde cholangiopancreatography (ERCP) is the procedure of choice for managing various benign and malignant pancreatobiliary conditions and can be associated with various adverse events. AIM: We performed a systematic review and meta-analysis to evaluate outcomes of ERCP in nonagenarians. METHODS: A comprehensive literature search was performed in Embase, MEDLINE, Web of Science, and Cochrane Review library until July 2020. Our primary outcomes were the rate of technical success and adverse events in nonagenarians. Secondary outcomes were comparison of technical success and adverse events compared with younger patients. RESULTS: The initial search revealed 4933 studies, of which 24 studies with 5521 patients met our inclusion criteria. Pooled technical success rate of ERCP in nonagenarians was 92%, and pooled adverse event rate was 7.8%. There was no significant difference in technical success rate and overall rate of adverse events comparing ERCP outcomes in nonagenarians with a relatively younger population. The risk of post-ERCP bleeding was significantly higher in nonagenarians compared to younger patients with OR = 1.986 [1.113-3.544], I2 = 0. ERCP-related mortality was also significantly higher in nonagenarians compared to younger patients with OR = 4.720 [1.368-16.289], I2 = 0. CONCLUSION: There was no significant difference in technical success rate and risk of adverse events related to ERCP in nonagenarians compared to younger patients. However, the risk of bleeding and procedure-related mortality was significantly higher.

Acute Gastrointestinal Bleeding in COVID-19 Patients: A Systematic Review and Meta-Analysis.

BACKGROUND/AIMS: More than 100 million people to date have been affected by the coronavirus disease-2019 (COVID-19) pandemic. Patients with COVID-19 have a higher risk of bleeding complications. We performed a systematic review and meta-analysis to evaluate the outcomes of COVID-19 patients with signs and symptoms of acute gastrointestinal bleeding (GIB). METHODS: A systematic literature search was carried out for articles published until until November 11, 2020, in the Embase, MEDLINE, Web of Science, and Cochrane Library databases. We included studies on COVID-19 patients with signs and symptoms of GIB. RESULTS: Our search yielded 49 studies, of which eight with a collective 127 patients (86 males and 41 females) met our inclusion criteria. Conservative management alone was performed in 59% of the patients, endoscopic evaluation in 31.5%, and interventional radiology (IR) embolization in 11%. Peptic ulcer disease was the most common endoscopic finding, diagnosed in 47.5% of the patients. Pooled overall mortality was 19.1% (95% confidence interval [CI]; 12.7%-27.6%) and pooled mortality secondary to GIB was 3.5% (95% CI; 1.3%-9.1%). The pooled risk of rebleeding was 11.3% (95% CI; 6.8%-18.4%). CONCLUSION: The majority of COVID-19 patients with GIB responded to conservative management, with a low mortality rate associated with GIB and the risk of rebleeding. Thus, we suggest limiting endoscopic and IR interventions to those with hemodynamic instability and those for whom conservative management was unsuccessful.

Safety and Efficacy of Nitazoxanide-Based Regimen for the Eradication of Helicobacter pylori Infection: A Systematic Review and Meta-Analysis.

BACKGROUND: Helicobacter pylori (HP) is the most common cause of gastritis worldwide. Clarithromycin-based triple therapy or bismuth-based quadruple therapy is usually considered the first-line treatment, however with around 30% failure rate for both regimens. Drug resistance of clarithromycin and metronidazole is a growing concern in some parts of the world. Therefore, there is a need for effective eradication regimen for HP. Nitazoxanide, a bactericidal thiazolide antibiotic, has been shown to be effective in HP infection. We conducted a systematic review and meta-analysis to evaluate the efficacy of nitazoxanide-based regimen for the eradication of HP. METHODS: We have searched PubMed, Embase, Ovid Medline and Cochrane library database from inception to December 9, 2020 to identify studies that utilized nitazoxanide in the treatment regimen for HP eradication. Our primary outcome was pooled eradication rate of HP. RESULTS: Thirteen studies including 1,028 patients met our inclusion criteria and were analyzed in a meta-analysis. HP eradication was successful in 867 patients with a pooled eradication rate of 86% (95% confidence interval (CI): 79-90%) with 84% heterogeneity. A subgroup analysis that included 230 patients who failed other prior eradication regimens revealed a pooled eradication rate of 85% (95% CI: 69-94%) without heterogeneity. In a subgroup analysis, highest eradication rates were achieved with levofloxacin, doxycycline, nitazoxanide and proton pump inhibitor with a pooled eradication rate of 92% (88-95%). CONCLUSION: Nitazoxanide-based regimen is safe and effective in the eradication of HP infection. It is also successful as a salvage therapy in patients who have failed prior treatments.

Acute Pancreatitis Secondary to Use of Appetite Suppressant: Garcinia cambogia.

Due to the global epidemic of obesity, weight loss and appetite suppressant herbal products are quite popular. As these medications are not United States Food and Drug Administration-approved and are regulated as dietary supplements, little evidence exists regarding their safety. This case discusses an 82-year-old man with the past medical history of obesity who presented to the emergency department with abdominal pain in the epigastric region. His serum lipase was elevated, and an abdominal computed tomography revealed acute pancreatitis (AP). He reported two episodes of AP in the past. He denied any alcohol use and reported no recent changes in his medications. He reported taking Garcinia cambogia (GC) recently as an appetite suppressant. Due to prior cholecystectomy, no alcohol abuse, no recent changes in medications and recent use of GC, a likely etiology of AP was thought to be secondary to the use of GC. He was treated with bowel rest and intravenous fluid hydration with significant improvement in his symptoms. He was advised to avoid GC in the future. Clinicians should be vigilant in evaluating their patients with AP and should get a meticulous history regarding their use of over-the-counter medications and herbal products.

Use of Curcumin in Achieving Clinical and Endoscopic Remission in Ulcerative Colitis: A Systematic Review and Meta-analysis.

BACKGROUND: Ulcerative Colitis (UC) is characterized by chronic inflammation of the mucosal layers of the colon. Treatment of refractory UC is challenging and has a huge healthcare burden. Although there have been advancements in immunomodulatory therapies, these require a step-up financially, and these medications are also associated with significant adverse events. Curcumin, an active ingredient of turmeric, has been studied in the past and found to be useful in the treatment of UC when used as an adjuvant along with mesalamine. We did a systematic review and meta-analysis to explore the role curcumin plays in clinical and endoscopic remission in patients with UC. MATERIALS AND METHODS: A comprehensive literature review was conducted by first searching the MEDLINE, Pubmed, and Embase databases through December 2017 to identify all studies that compared the use of curcumin when used along with mesalamine with placebo for clinical and endoscopic improvement and remission. RESULTS: Three randomized controlled trials including 142 patients were included in the study. Use of curcumin along with mesalamine was associated with increased odds of clinical remission (pooled odds ratio of 6.78, 95% CI: 2.39-19.23, P = 0.042). Clinical improvement, endoscopic remission and improvement rate also trended higher in the curcumin group compared to placebo. CONCLUSIONS: This study demonstrates higher clinical remission rates when curcumin was used in combination with mesalamine to achieve remission in patients with UC. Curcumin, due to its cost effectiveness and safer side effect profile, can decrease the healthcare burden and morbidity associated with this relapsing and remitting disease.

Safety and efficacy of a minimally invasive cell sampling device ('Cytosponge') in the diagnosis of esophageal pathology: a systematic review.

Esophageal adenocarcinoma is an increasingly common cause of morbidity and mortality in developed countries. Most cases are considered the consequence of chronic gastroesophageal reflux disease, with subsequent Barrett's metaplasia and dysplasia. Because progression from Barrett's metaplasia to cancer occurs over many years, endoscopic screening and surveillance programs have been established, albeit with little or no consideration for cost-effectiveness. As an alternative to the expensive and resource-demanding endoscopic surveillance, the Cytosponge has been developed to sample the esophageal mucosa efficiently. The device is a compressed mesh sponge encapsulated in an ingestible gelatin pill attached to a string. After swallowing, the capsule dissolves allowing the sponge to expand in the stomach. As it is pulled out, cells are collected from the esophagogastric junction and throughout the esophagus. The cellular samples can be analyzed by cytology, immunohistochemistry, and molecular markers. We conducted a systematic review of all recent relevant studies to help define the role of this novel technology, including studies of screening and surveillance of Barrett's esophagus, esophageal squamous dysplasia detection, detection of eosinophilic esophagitis, and evaluation of benign esophageal diseases. With the major limitation that most studies were performed by a single investigative group that developed the technology, the device yielded overall impressive results against the endoscopy/biopsy gold standard. Patient acceptability was high. If these promising early results are validated by other investigators in other populations, the Cytosponge represents an important new advance in the detection of esophageal pathology that could potentially decrease the burden of endoscopic esophageal sampling.

Ringer's lactate versus normal saline in acute pancreatitis: A systematic review and meta-analysis.

OBJECTIVE: Aggressive i.v. hydration with crystalloids is the first step in managing acute pancreatitis (AP) and is associated with improved survival. Guidelines about the choice of crystalloids to use are unclear. This systematic review and meta-analysis was aimed to discern whether the choice of fluids in managing pancreatitis was associated with patients' outcomes. METHODS: A comprehensive literature review was conducted by searching the Embase, MEDLINE, PubMed and Google Scholar databases to December 2017 to identify all studies that compared normal saline (NS) with Ringer's lactate (RL) for managing AP. The characteristics of the participants, outcome measurements (including mortality, the development of systemic inflammatory response syndrome [SIRS] on admission and at 24 h, and pancreatic necrosis) were analyzed. RESULTS: Five studies (three randomized controlled trials and two retrospective cohort studies) with 428 patients were included in this analysis. Mortality trended lower in the RL group but this was not statistically significant (pooled odds ratio [OR] 0.61, 95% CI 0.28-1.29, P = 0.20). Patients in the RL group had significantly decreased odds of developing SIRS at 24 h (pooled OR 0.38, 95% CI 0.15-0.98, P = 0.05). CONCLUSIONS: RL has anti-inflammatory effects and is associated with decreased odds of persistent SIRS at 24 h, which is a marker of severe disease in AP patients. Although mortality trended lower in the RL group this did not achieve statistical significance and hence larger randomized controlled trials are needed to evaluate this association.

Does Early Endoscopy Improve Mortality in Patients with Acute Non-variceal Gastrointestinal Bleeding? A Retrospective review.

Introduction Initial management of acute upper gastrointestinal bleeding (UGIB) aims towards aggressive fluid resuscitation to maintain hemodynamic stability. Existing evidence regarding the benefit of early endoscopy is unclear with some studies suggesting mortality benefits and some suggesting otherwise. The purpose of this study is to evaluate if there is any mortality benefit of doing early endoscopy within 24 hours of presentation. Methods From July 2013 to July 2016, 179 patients admitted with a diagnosis of non-variceal UGIB were retrospectively reviewed. Clinical variables including 30-day mortality were then compared between the patients who had endoscopy within 24 hours with those who had endoscopy after greater than 24 hours. Results Out of 179 patients admitted for non-variceal UGIB, 146 underwent endoscopy within 24 hours of presentation and 33 underwent endoscopy after 24 hours. The overall mortality associated with UGIB was 6.7% (12/179). There was no statistically significant difference found in 30-day mortality between the two groups (6.8% within 24 hours vs 6.1% after 24 hours). There was also no difference in 30-day readmission or rates of rebleeding among the two groups. The length of stay was also similar in both groups (6.0 days vs 6.1 days). Conclusion This study did not find any advantage of endoscopy within 24 hours on length of stay, rate of complications, and 30-day mortality. As hemostasis is achieved in almost 90% of patients with supportive management without any endoscopic intervention, focus should be made on aggressive fluid resuscitation to achieve hemodynamic stability before endoscopy.

Safety and efficacy of encapsulated fecal microbiota transplantation for recurrent Clostridium difficile infection: a systematic review.

BACKGROUND AND AIMS: Fecal microbial transplantation (FMT) has been shown to be effective for the treatment of recurrent clostridium difficile infection (CDI). The efficacy and safety of freeze-dried encapsulated FMT for the treatment of recurrent CDI is unclear. We performed a systematic review to evaluate and analyze the current evidence in this respect. MATERIALS AND METHODS: A systematic literature search was performed using the PubMed, Embase, and Medline databases until December 2017 to identify all original studies that investigated the role of administration of encapsulated FMT in recurrent CDI. The study included patients of all ages. Two independent reviewers extracted data and assessed the quality of publications; a third investigator resolved any discrepancies. RESULTS: A total of six studies, five case series and one randomized-controlled trial, were included in this review. Overall, 341 patients completed treatment with encapsulated FMT. Only three major adverse events were reported and no deaths occurred directly related to FMT. In all, 285 patients responded to the first treatment, with no recurrence during the specified follow-up period set to meet the primary endpoint. Forty-two patients underwent a second treatment, with resolution of symptoms in 28 patients. At least five patients were reported to undergo a third treatment, with resolution in three of them. Only one patient was reported to have received four treatments without long-term resolution of symptoms. CONCLUSION: Low-quality to moderate-quality evidence showed that encapsulated FMT is safe and cost-effective for the treatment and prevention of recurrent CDI. Its efficacy is not inferior to FMT performed through the nonoral route. Randomized-controlled trials are necessary to compare its efficacy with oral antimicrobial drugs and also to evaluate the potential adverse effects associated with the treatment.

Atypical Colonoscopic Presentation of Lymphocytic Colitis Mimicking Hyperplastic Polyposis Syndrome.

Lymphocytic colitis is a chronic inflammatory disease of colon usually presented in middle age female as chronic watery diarrhea. Diagnosis is made on biopsy as colonoscopy usually revealed normal appearing colonic mucosa. We present here an unusual case of a 25-year-old female with past medical history of asthma was evaluated for one year of non-bloody watery diarrhea. The symptoms started after a course of antibiotics for upper respiratory tract infection a year back. The serum chemistries, including liver enzymes, were unremarkable. Stool culture, ova, and parasites were unremarkable. Stool Clostridium difficile was also negative. Celiac disease antibodies were unremarkable. Stool occult blood test was positive. The patient underwent colonoscopy for the evaluation of chronic diarrhea and revealed multiple polyps throughout the colon with inflamed surfaces which were biopsied. The concern was for hyperplastic polyposis syndrome. Genetic testing for adenomatous polyposis gene was done and came back negative. Biopsy from polyps revealed lymphocytic colitis. The patient was started on budesonide which resulted in marked improvement in her symptoms. Our case highlighted an atypical endoscopic finding of lymphocytic colitis which mimic hyperplastic polyposis syndrome.

Allopurinol-Induced Granulomatous Hepatitis: A Case Report and Review of Literature.

Liver enzyme elevation is a common reason for referral to a gastroenterologist. Drugs are one of the most common reasons for asymptomatic elevation of liver enzymes. We present here a case of granulomatous hepatitis (GH) secondary to long-term use of allopurinol. An 83-year-old male with a history of chronic gout and hypertension was evaluated for elevation of liver enzymes. He denies any complaints of abdominal pain, nausea, fever, chills, weight loss, night sweats, or yellowness of skin. He denies any use of herbal medications. He was on losartan and allopurinol for years. No new medications reported. Physical examination was unremarkable. Labs showed aspartate transaminase 101 U/L, alanine transaminase 81 U/L, and alkaline phosphatase 645 U/L. Ultrasound of the abdomen showed coarse liver texture. Liver biopsy was done that showed mixed GH. Given negative autoimmune and viral serologies, allopurinol-induced GH was suspected. Allopurinol was held, and repeat liver enzymes were checked in 3 months, which showed improvement in transaminase and alkaline phosphatase levels. This case highlights the importance of reviewing medications carefully when evaluating a patient with liver enzymes elevation, as stopping the offending drug can normalize the abnormalities in liver chemistries and can prevent subsequent expensive testing.

Richter's Hernia Presenting As Faecal Fistula In Female; A Rare Entity.

Strangulated hernia in quite common in surgical practice and always present with its typical symptoms. In this hardly found unique case, a 76-year's old female with no co morbid presented to us with abdominal pain, vomiting and diarrhoea initially and later developed left groin abscess after 4 days of swelling. It was assumed to be inguinal lymphadenitis leading to abscess formation but after complete evaluation on digital examination, diagnosis of Strangulated Femoral hernia of Richter type was made. This distinguishing presentation among the list of all hernias has to be discussed for the differential diagnosis.

Does Right Ventricular Dysfunction Predict Mortality in Hemodynamically Stable Patients With Acute Pulmonary Embolism?

BACKGROUND: Acute pulmonary embolism (APE) is directly responsible for 100,000 deaths annually. Right ventricular dysfunction (RVD) on admission is considered a poor prognostic factor in these patients, though existing evidence of its significance in predicting mortality in hemodynamically stable patients is still unclear. We attempted to clarify this association by doing a retrospective review. METHODS: We retrospectively reviewed electronic medical records of hemodynamically stable patients older than 18 years of age with APE who were admitted to a tertiary care hospital in rural Upstate New York from July 2014 to July 2016. One hundred thirty-four patients were reviewed in two groups: patients who presented with computed tomography (CT) or echocardiographic evidence of RVD, and those without RVD. To identify differences in mortality between the two groups, the Chi-square/Fisher's exact test and t-tests were used. All variables with P < 0.2 in the initial analysis were included in a stepwise multivariable logistic regression model to predict RVD. RESULTS: No statistically significant difference was found in 30-day mortality between the groups (7.8% in RVD and 5.3% in no RVD, P = 0.563). The overall prevalence of RVD was found to be 57% (77/134). Troponin elevation (53.2% in RVD group vs. 19.3 in the no RVD group with P < 0.01) and central location of thrombus (53.1% vs. 32.1% with P = 0.016) were more prevalent in RVD group. A marginally significant difference was found in length of hospital stay among those with RVD versus no RVD (7.13 days vs. 5.46 days; P = 0.061). The multivariable analysis shows that the odds of RVD were greater for patients with elevated troponin levels (odds ratio = 7.8). CONCLUSION: There was no difference in 30-day mortality in hemodynamically stable patients with APE having RVD compared to patients with no RVD. On the basis of this study, we do not suggest the routine use of systemic fibrinolysis in hemodynamically stable patients with radiographic evidence of RVD alone.

Prognostic Significance of Elevated Cardiac Troponin in Acute Gastrointestinal Bleeding.

BACKGROUND: Acute gastrointestinal bleeding (AGIB) is responsible for over 140,000 hospitalizations annually. Cardiovascular-related deaths account for 30% of the patients surviving the initial episode of AGIB. The purpose of this study was to identify the impact of elevated troponin on short-term mortality and length of stay (LOS) of these patients. METHODS: From July 2013 to July 2016, 290 patients admitted with a diagnosis of AGIB and who had cardiac troponin I measured within 24 h of presentation were retrospectively reviewed. Clinical variables including 30-day mortality, 30-day readmission and LOS were then compared between the groups of troponin elevation and no troponin elevation. RESULTS: The overall 30-day mortality among patients with AGIB was 6.5% (19/290). Cardiac troponin was elevated in 10% of patients (29/290). Among patients with normal troponin, 5% (13/261) died within 30 days. In patients with troponin elevation, 21% died in the same period (6/29, P = 0.001). The LOS was also higher in patients with troponin elevation (6 vs. 5 days, P = 0.02). There was no difference in 30-day readmission among the two groups. Past history of coronary artery disease, congestive heart failure, hypertension, aspirin use and elevated creatinine was more common in patients with troponin elevation. On multivariate analysis, troponin elevation on presentation is associated with increased mortality (odds: 5.50, CI: 1.73 - 17.47, P = 0.004). CONCLUSION: In patients admitted to the inpatient service with AGIB, elevated troponin I on presentation is associated with high short-term mortality and longer hospital stay.

Recurrent Metatarsal Fractures in Postmenopausal Woman With Low Serum Alkaline Phosphatase: A Rare Diagnosis Not to Miss.

Hypophosphatasia (HPP) is a rare inborn error of metabolism due to a loss-of-function mutation in the gene for tissue nonspecific isoenzyme of alkaline phosphatase (ALP) that results in low levels of ALP. The clinical presentation of HPP is variable and in adults can easily be misdiagnosed as other forms of osteomalacia. We present a case of a 53-year-old Caucasian female who was evaluated for recurrent metatarsal fractures. She reported her first metatarsal fracture at age 21, and since then had at least 8 more metatarsal fractures over her lifetime, most without injury other than weight bearing. She reported history of gait disturbance as a child and dental issues (spacing and loosening). Laboratory tests showed normal serum calcium, phosphorus, and parathyroid hormone, but low serum ALP <20 IU/L and elevated N-telopeptide. Foot X-ray showed several healed and nonhealed metatarsal fractures, and bone densitometry revealed osteopenia. She was treated with calcium and vitamin D. A year later she had a new metatarsal fracture and a nontraumatic pelvic fracture. Teriparatide therapy was attempted but not tolerated. Due to suspicion of HPP vitamin B6 levels were checked and found to be elevated at 263 µg/L. Given her clinical presentation and low ALP levels with elevated vitamin B6, the diagnosis of HPP was made. Clinicians should be attentive to a history of recurrent low trauma fractures, premature loss of deciduous teeth, and persistently low serum ALP to suspect this diagnosis. Early case detection with the availability of recent Food and Drug Administration-approved asfotase alfa may avoid years of undiagnosed morbidity.

Acute Pancreatitis Secondary to Severe Hypertriglyceridemia: Management of Severe Hypertriglyceridemia in Emergency Setting.

Hypertriglyceridemia (HTG) is the third most common cause of acute pancreatitis (AP). The incidence of AP is around 10-20% with levels > 2,000 mg/dL. We present here a case of a 44-year-old male with history of uncontrolled diabetes mellitus and HTG admitted with severe abdominal pain. Labs revealed elevated lipase and amylase. CT of abdomen with contrast showed AP. He was found to have a triglyceride (TG) level of 6,672 mg/dL. Besides conventional treatment for AP with intravenous (IV) hydration, he was started on IV regular insulin along with dextrose saline. He had marked improvement in his TG level the next day. He was continued on insulin and dextrose saline with hourly glucose monitoring until TG was < 500 mg/dL. He was discharged on statins and fenofibrate. The goal of management of AP secondary to severe HTG in emergency setting is to lower the TG levels to less than 500 as quickly as possible as lower levels are associated with good clinical outcomes. Apheresis and IV insulin are both helpful in lowering TG levels with no randomized controlled trials showing greater efficacy of one over other. Further episodes of AP can be prevented by lifestyle modification and lipid lowering drugs to keep TG levels below 500 mg/dL. Fibrates are first-line drugs to lower TG and used either alone or in conjunction with statins. Periodic plasmapheresis can also be considered in some non-compliant patients with recurrent episodes of pancreatitis.