bluuebunny/crossref_metadata_2025 huggingface dataset:
Thoracic Trauma
Authors: Baker William E. , Medzon Ron
Published: January 2021

More than 85% of blunt and penetrating trauma to the thorax results in injury to the lungs or ribs. Among civilians, blunt trauma is the most common mechanism, while penetrating trauma is the most common among military sectors. This review describes the assessment and stabilization, diagnosis, treatment and disposition, and outcomes of thoracic trauma. Videos shows the “lung point” sign on M-mode and two-dimensional ultrasonography, and a transthoracic echocardiogram clip of pericardial clot and tamponade due to a gunshot wound. Figures show a sonogram showing the “lung point sign”, a chest x-ray and computed tomographic scan demonstrating right-sided hemothorax in a patient with a right chest stab wound, and a three-dimensional computed tomographic scan and chest x-ray of a blunt trauma patient with displaced fractures of the left lateral sixth to ninth ribs. Tables list types of injuries, NEXUS chest decision instrument imaging criteria, level 2 evidence-based recommendations for the management of pulmonary contusion and flail chest by the Eastern Association for the Surgery of Trauma, Eastern Association for the Surgery of Trauma practice guidelines for managing issues with pulmonary contusion and flail chest, and the Vancouver simplified and University of Washington grading systems for blunt aortic injury.
This review contains 2 videos, 4 highly rendered figures, 10 tables, and 94 references.
Anticholinergic Toxicity
Authors: Lai Jeffrey T , Babu Kavita M
Published: January 2021

Anticholinergic compounds oppose the action of the endogenous neurotransmitter acetylcholine at its target receptors and are found in over-the-counter and prescription medication, natural products, and plants. Anticholinergic medications, such as atropine and scopolamine, are used for the treatment of a wide range of conditions, including bradycardia, motion sickness, and insomnia. Antihistaminergic medications, such as diphenhydramine, also possess anticholinergic activity and are used in the treatment of seasonal allergies, common cold symptoms, and allergic reactions. Other medications, such as antidepressants (especially the older tricyclic class), antipsychotics, muscle relaxants, and anticonvulsants, can act as anticholinergic agents or produce anticholinergic side effects. Toxicity can result from therapeutic misadventure, intentional overdose, recreational use, and pediatric exposures. This review covers the principles of toxicity, immediate stabilization, diagnosis and definitive therapy, and disposition and outcomes. Figures show the anticholinergic toxidrome, look-alike structures, and electrocardiographic changes in tricyclic antidepressant overdose. Tables list medications with anticholinergic activity and selected botanicals that cause anticholinergic toxicity.
Key words: anticholinergic overdose, anticholinergic toxicity, anticholinergic toxidrome, physostigmine, tricyclic antidepressant toxicity
This review contains 3 highly rendered figures, 2 tables, and 49 references.
Pediatric Procedural Sedation
Authors: Nelson Sara W. , Simmons J. Calvin
Published: January 2021

Children present to the emergency department with painful conditions or conditions that require diagnostic or therapeutic procedures every day. As emergency physicians, we need to have the skills to manage our patients’ pain and anxiety in a safe and efficient manner. Appropriately managing pain and anxiety facilitates medical interventions, decreases patients’ suffering, improves patient and parent satisfaction, and improves the quality of care. Conversely, failure to adequately provide analgesia and sedation can have negative consequences for pediatric patients. In the pediatric population, inadequate pain control not only causes immediate harm and fear but can also worsen the reaction to future medical care and potentially affect the child’s long-term psychological well-being. This review provides an overview of pediatric procedural sedation, as well as the pathophysiology and practice. Figures show the sedation continuum with associated physiologic responses, oxyhemoglobin desaturation during apnea for various types of patients, and examples of capnography waveforms in procedural sedation and analgesia (PSA). Tables list potential indications for pediatric PSA in the emergency department, American Society of Anesthesiologists’ classifications, drugs and pharmacokinetics of common agents used in PSA, focused history and physical examination for patients undergoing PSA, SOAPME (Suction, Oxygen, Airway, Pharmacy, Monitors, Equipment) acronym for PSA equipment, and suggested monitoring for PSA pre- and postprocedure.
This review contains 3 highly rendered figures, 6 tables, and 41 references.
Key words: pediatric analgesia; pediatric pain; pediatric procedural sedation; pediatric sedation; procedural pain relief; procedural sedation and analgesia
Pediatric Hematologic And Oncologic Emergencies
Authors: Milligan Rebecca , Mendelson Jenny
Published: January 2021

Hematologic and oncologic emergencies that afflict children and adolescents are important for emergency medicine physicians to recognize. Pediatric patients can present with a previous formal diagnosis and have a complication related to their disease or with new symptoms that suggest a hematologic or oncologic process. Oncologic treatments can also lead to life-threatening complications. Recognizing these emergencies is very important for emergency physicians to prevent further morbidity. This review covers common patient presentations, diagnosis, and treatments for hematologic and oncologic emergencies.
 This review contains 6 figures, 6 tables and 57 references
Pediatric Diabetic Ketoacidosis and Hypoglycemia
Authors: Williams Rachel J , Wood Samantha L.
Published: January 2021

Abnormalities of serum glucose in pediatric patients are commonly encountered in the emergency department and represent an acute threat to life and neurologic function. Rapidly identifying and aggressively treating hyperglycemia with diabetic ketoacidosis and hypoglycemia are critical to ensure the best possible outcome. This review will guide the emergency provider in the identification, resuscitation, workup, and disposition of these critically ill patients.
This review contains 6 figures, 13 tables, and 50 reviews.
Key Words: Cerebral edema, diabetic ketoacidosis, hyperglycemia, hypoglycemia
Platelet Disorders
Authors: Perry Michael , Bedolla John , Milling Truman John
Published: January 2021

Hemostasis occurs in two steps: platelet plug formation followed by fibrin deposition. Platelet disorders cause incomplete or absent platelet plug formation. Platelets form in bone marrow and have an 8- to 9-day life span. A careful history and physical examination can distinguish between platelets or the coagulation cascade as the cause of deranged hemostasis. Platelet disorders are characterized by mucosal and small vessel bleeding, and petechiae are characteristic. A complete blood count and prothrombin time/international normalized ratio testing reveal most causes. Many drugs can alter platelet production, function, and longevity, but antiplatelet therapy is the most common cause. Other causes include congenital, medication side effects, sepsis, bone marrow suppression, and systemic disease. The platelet count may be low, elevated, or normal. Bleeding time is virtually always prolonged in platelet dysfunction. Therapy for platelet dysfunction depends on the etiology and severity. Platelets are short-lived, and platelet transfusion may be necessary in the unstable or actively bleeding patient. In most other cases, removing the cause or treating the systemic disease will improve hemostasis. Emergency physicians treating patients with abnormal bleeding should understand the coagulation cascade and drug-induced coagulopathy from older and newer agents.
Key words: bleeding time, coagulopathy, drug induced, hemostasis, megakaryocyte, mucosal bleeding, petechiae, platelet plug, platelet transfusion, platelets, thrombocytopenia
Mesenteric Ischemia
Authors: Ezenkwele Ugo A.
Published: January 2021

Acute mesenteric ischemia is interruption of intestinal blood flow by embolism, thrombosis, or a low-flow state. Bowel infarction is the end result of a process initiated by mediator release and inflammation. On clinical assessment, the early hallmark is severe abdominal pain but minimal physical findings. The abdomen remains soft, with little or no tenderness. Mild tachycardia may be present. Early diagnosis is difficult, but selective mesenteric angiography and computed tomographic angiography have the most sensitivity; other imaging studies and serum markers can show abnormalities but lack sensitivity and specificity early in the course of the disease, when diagnosis is most critical. Treatment is by embolectomy, anticoagulation, revascularization of viable segments, or resection; sometimes vasodilator therapy is successful. If diagnosis and treatment take place before infarction occurs, mortality is low; after intestinal infarction, mortality approaches 30 to 70%. For this reason, in the emergency department, clinical diagnosis should supersede diagnostic tests, which may delay treatment.
Key words: acute mesenteric ischemia; bowel necrosis; chronic mesenteric ischemia; mesenteric occlusive disease; mesenteric venous thrombosis; nonocclusive mesenteric ischemia; postprandial abdominal pain; superior mesenteric artery thromboembolism
Acute Respiratory Failure And ­Mechanical Ventilation
Authors: DeLuca, Jr Lawrence A.
Published: January 2021

Patients with acute respiratory failure present to the emergency department (ED) on a regular basis, and emergency physicians (EPs) are expected to be skilled in endotracheal intubation. Historically, although a significant portion of emergency medicine residency training focuses on airway management, extended management of the ventilated patient has received relatively short shrift. Recent data indicate that not only is endotracheal intubation one of the most commonly performed ED procedures, but also that in the initial hours of care, it is also often the EP rather than the intensivist who provides the bulk of critical care to the patient. It is therefore critical that EPs are skilled in ongoing management of the ventilated patient in the early hours as inappropriate management of the ventilator or sedation/analgesia can have a significant impact on complications such as ventilator-induced lung injury, ventilator-associated pneumonia (VAP), ventilator weaning, and delirium. This review outlines basic strategies for the physiologic management of respiratory failure patients to reduce periintubation complications and discuss ventilation strategies, appropriate use of analgesia/sedation, and prevention of secondary complications such as VAP and delirium. Basic troubleshooting of common ventilator problems is also reviewed. Although it is not expected that the EP will replace the intensivist, the goal of this review is to optimize patient management early in the ED stay, to facilitate the transition between the ED and the intensive care unit, and to reduce preventable complications by optimizing the care of ventilated patients in the ED.

This review contains 9 figures, 6 tables and 48 references.
Key words: acute respiratory distress syndrome, analgesia, chronic obstructive pulmonary disease, delirium, hypercapnia, hyperventilation, hypoxia, patient-ventilator dyssynchrony, pulmonary edema, respiratory failure, sedation, ventilator-associated pneumonia
Human Trafficking 2: Approach To The Patient
Authors: Macias-Konstantopoulos Wendy
Published: January 2021

Trafficking survivors most commonly cite the emergency department (ED) as their health care access point while previously trafficked. A majority of trafficking survivors surveyed report accessing medical care at least once while trafficked. ED physicians should adhere to guiding principles of care, keeping in mind at all times the need for patient privacy and the use of paced evaluations and neutral language. It is incumbent upon ED physicians to recognize both the clinician-related barriers to helping trafficked patients (which may include an underappreciation of the relevance of trafficking to clinical practice and a lack of education and training) and patient-related barriers (which range from restriction and confinement by their abuser to the patient’s own shame, guilt, and self-blame; distrust of authorities; and fear). Once the trafficked patient presents to the ED, the physician should acknowledge that the identification of at-risk and trafficked patients can be the first step toward prevention and assistance, respectively. No singular or defined set of diagnostic signs or symptoms has been shown to cut across all forms of trafficking with any degree of sensitivity or specificity, but familiarity with the potential indicators of human trafficking can help the emergency provider recognize patterns and raise the suspicion of trafficking. If a clinician suspects that a patient might be trafficked, the clinician should engage the patient in a trauma-informed and culturally sensitive assessment.

This review contains 2 tables and 34 references
Key words: commercial sexual exploitation, debt bondage, domestic servitude, forced labor, forced substance use, HIV, modern-day slavery, neutral language, patient privacy, posttraumatic stress disorder, trafficking in persons
Update on Imaging Modalities for Inflammatory Bowel Diseases
Authors: Alessandrino Francesco , Mortele Koenraad J.
Published: January 2021

Inflammatory bowel diseases (IBDs) are a group of chronic inflammatory disorders that are mainly represented by Crohn disease and ulcerative colitis. Over the last decade, conventional fluoroscopic barium studies used for the imaging of the gastrointestinal tract have been replaced with newer techniques, such as multidetector-row computed tomography (CT), dual-energy CT, and positron emission tomography (PET)/CT. Also increasingly seen is the use of magnetic resonance enterography, representing a robust, highly accurate, and radiation-free imaging method. This review covers these newer imaging modalities for IBD and more, as well as proposed techniques that could potentially help monitor and guide new antiinflammatory treatment strategies in the future. Figures include examples of images conducted via CT, magnetic resonance imaging, and PET/CT. Table compares different imaging modalities for the evaluation of IBD.
This review contains 13 highly rendered figures, 1 table, and 127 references.
Medical Therapy of Ulcerative Colitis
Authors: Burke Kristin E. , Feuerstein Joseph D. , Cheifetz Adam S.
Published: January 2021

Ulcerative colitis (UC) is a chronic idiopathic inflammatory bowel disease (IBD) characterized by continuous colonic mucosal inflammation that extends proximally from the rectum. Most patients present with bloody diarrhea, abdominal pain, tenesmus, and urgency. The course of UC is characterized by periods of flares and remission, and can significantly impact quality of life. Most patients are successfully treated medically, though 15 to 25% of patients require total proctocolectomy. Treatment of UC depends on the extent and severity of the disease, and response to prior therapies. This review outlines the medical treatment of UC and the therapies available for the induction and maintenance of remission. Figures include endoscopic and histologic images of UC, and algorithms cover outpatient inductions for mild-to-moderate disease and moderate-to-severe-disease, outpatient management of known steroid-refractory/steroid-intolerant patient, and approach to inpatient management of acute severe UC. Tables list disease severity by Truelove and Witts criteria, IBD medication adverse effects and monitoring parameters, induction dosing of 5-aminosalicylates, and rates of clinical response, remission, and mucosal healing in patients receiving anti-tumor necrosis factor (TNF) agents versus placebo by trial.
This review contains 5 highly rendered figures, 4 tables, and 118 references.
Microscopic Colitis
Authors: O’Connor Anthony , Moss Alan C.
Published: January 2021

Microscopic colitis (MC) is defined by the presence of specific histologic abnormalities in patients with diarrhea and a normal-appearing colon at endoscopy. MC can be categorized into two types: collagenous colitis (CC) and lymphocytic colitis (LC). CC is characterized by a thickened subepithelial collagen band in the colonic mucosa of at least 10 mm in thickness, whereas LC describes infiltration of the colonic epithelium with lymphocytes (intraepithelial lymphocytes), without excess collagen in the mucosa. This review of MC addresses the epidemiology, pathogenesis, etiology/genetics, diagnosis, differential diagnosis, treatment, complications, and prognosis between the two types. Tables list potential etiologic factors in MC, medications associated with MC, and treatments used for MC and evidence base.
This review contains 3 tables and 55 references.
Diverticulosis and Diverticulitis
Authors: Duloy Anna M. , Makrauer Frederick L
Published: January 2021

Diverticular disease has been considered a disease of the elderly, but recently, an increased incidence has been noted in younger patients. Diverticulosis is asymptomatic; however, when symptomatic, it is referred to as diverticular disease. When associated with any inflammation, it is diverticulitis. Diverticulitis is an acute illness, but symptoms may become chronic with recurrent episodes. When mucosal inflammation is present, segmental colitis associated with diverticula (SCAD) is identified. SCAD is a distinct, but uncommon, disorder sharing histological and clinical features of ulcerative colitis and Crohn disease. Only 1 to 2% of patients with diverticulosis will develop diverticulitis. This review covers the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, management, complications and prognosis of diverticulosis, and diverticulitis. Figures show diverticulosis, symptomatic uncomplicated diverticular disease, diverticulitis, computed tomography scan of acute diverticulitis, and a management algorithm. Tables list definitions, risk factors, pathophysiology, modified Hinchey classification, and acute diverticulitis differential diagnosis.
 
This review contains 5 highly rendered figures, 5 tables and 105 references
Functional Dyspepsia
Authors: Chan Walter W , Mahmud Nadim
Published: January 2021

Functional dyspepsia (FD), formerly “nonulcer dyspepsia,” makes up approximately 50 to 75% of dyspepsia presentations. These presentations are defined by having no identifiable structural disease that would explain the symptoms. Recognizing the heterogeneity within the diagnosis of FD, the Rome III criteria further classify FD based on primary symptoms: postprandial distress syndrome and epigastric pain syndrome. The former term refers to bothersome fullness or early satiety with regular meals, whereas the latter is characterized by intermittent epigastric pain or burning that is not predictably meal associated. This review addresses FD, detailing the epidemiology, pathophysiology and pathogenesis, differential diagnosis, diagnosis, treatment, and prognosis. A figure shows the diagnostic algorithm for new-onset dyspepsia. Tables list the Rome III criteria for functional dyspepsia, alarm features in dyspepsia, and medications commonly associated with dyspepsia.
This review contains 1 highly rendered figure, 3 tables, and 27 references.
Gastroparesis
Authors: Kuo Braden , Guay Laurence
Published: January 2021

The gastric phase of digestion requires a tightly coordinated neuromuscular apparatus to permit appropriate timing for each step. Dysregulations in this apparatus may be related to the Cajal cells, the intrinsic enteric nervous system, the extrinsic nervous system, the muscle cells, or any combination of structures and may lead to abnormal gastric emptying, referred to as gastroparesis. Gastroparesis is idiopathic in 35 to 49.4% of cases but may also be related to diabetes, autoimmune and inflammatory conditions, multiple sclerosis, use of certain medications, and infections, among other factors. This review describes the epidemiology, etiology, pathophysiology, clinical manifestations, diagnosis, treatment, and prognosis of gastroparesis. The figure shows symptom mechanisms related to gastric physiology in gastroparesis patients. Tables list the physiopathologic mechanisms and symptoms associated with defective gastric physiologic phenomena, the classification of gastroparesis according to affected structures, and pharmacologic treatments for gastrointestinal dysmotility.
This review contains 1 highly rendered figure, 3 tables, and 56 references.
Irritable Bowel Syndrome with Constipation
Authors: Tang Loni , Cash Brooks D.
Published: January 2021

Irritable bowel syndrome (IBS) is characterized by recurrent abdominal pain or discomfort that has occurred at least 3 days per month in the 3 months prior to diagnosis. One of the subtypes of this disorder is IBS with constipation (IBS-C), where individuals experience hard or lumpy stools at least 25% of the time and loose or watery stools less than 25% of the time with defecation. This review addresses IBS-C, detailing the epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, and prognosis. A figure shows the Bristol stool form scale. Tables list IBS subtypes, components of digital rectal examination, differential diagnoses for IBS and IBS-C, alarm features, and the American College of Gastroenterology Recommendations.
This review contains 1 highly rendered figure, 6 tables, and 71 references.
Celiac Disease, Refractory Celiac Disease, and Tropical Sprue
Authors: Trier Jerry S.
Published: January 2021

Celiac disease, often also termed celiac sprue, is an immune-mediated disorder triggered by ingestion of wheat, barley, rye, and triticale gluten proteins in genetically predisposed individuals. The offending glutens induce a characteristic but variable and nonspecific lesion of the small intestinal mucosa that improves following gluten withdrawal. Patients with refractory celiac disease (RCD) have persistent or recurrent symptomatic malabsorption together with biopsy-documented enteropathy that has not responded to 6 to 12 months of strict gluten withdrawal. Tropical sprue is an illness that occurs in individuals living in tropical countries; clinical features include anorexia, diarrhea, and intestinal malabsorption, leading to weight loss accompanied by a characteristic although nonspecific lesion of the mucosa of the small intestine. This review addresses the epidemiology, etiology/genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of these related diseases. Figures show the celiac disease iceberg, duodenal mucosal surface seen at endoscopy, duodenal mucosal biopsies from a patient with celiac disease, higher magnification micrograph of the duodenal mucosal surface from a patient with untreated celiac disease, duodenal biopsies from a patient with refractory celiac disease, and duodenal biopsy obtained from an Indian expatriate with diarrhea, weight loss, and folate deficiency. Tables list selected extraintestinal manifestations of celiac disease, prevalence of celiac disease in selected conditions, disorders with duodenal lesions that may mimic those of celiac diseases, initial treatment for celiac disease, organizations that provide helpful information for gluten-intolerant patients, considerations in a patient not responding to a gluten-free diet, and features that help distinguish tropical sprue from celiac disease.
This review contains 6 highly rendered figures, 7 tables, and 96 references.
Short Bowel Syndrome
Authors: Burakoff Robert , Goldin Alison
Published: January 2021

Short bowel syndrome (SBS) is a state of malabsorption resulting from physical or functional loss of large portions of the small intestine, and is the most common cause of intestinal failure. The average length of a human’s small intestine is between 3 and 8 m, depending on the type of measurements made (surgical, radiologic, or autopsy); SBS occurs when less than 200 cm of small bowel remains. SBS may be congenital (intestinal atresia) or acquired. Physical losses usually occur from surgical resection for Crohn disease (CD), vascular insufficiency, radiation, malignancy, trauma, or volvulus. The site of intestinal resection helps to determine the degree of intestinal capacity. Functional losses, on the other hand, are less common and occur in the setting of a nonfunctioning, but intact, small intestine. Examples include radiation enteritis, congenital defects, and inflammatory bowel disease (IBD). This review addresses the epidemiology, pathophysiology and pathogenesis, clinical manifestations, treatment, complications, and prognosis of SBS. A figure shows sites of intestinal nutrient absorption. A table lists potential complications of SBS in patients receiving parenteral nutrition (PN).
This review contains 1 highly rendered figure, 1 table, and 67 references.
Systemic Mastocytosis
Authors: Hamilton Matthew J.
Published: January 2021

Systemic mastocytosis (SM) is a disease characterized by an abnormal proliferation and accumulation of a clonal population of mast cells in the tissues including the bone marrow, gastrointestinal tract, and skin. In the most common form, indolent mastocytosis, the symptoms are most often due to the consequences of mast cell activation and include flushing, abdominal pain, and diarrhea. The diagnosis is made using a defined set of criteria based on a tissue biopsy, with special staining and a blood test for tryptase. Treatment is directed at the mast cell activation and specific symptoms such as nausea and abdominal bloating. Differential diagnosis includes the non-clonal form of mast cell activation syndrome and IgE-mediated allergy.
Keywords: mast cell, mast cell activation, mastocytosis, tryptase, flushing, urticaria pigmentosa, dermatographism
This review contains 5 highly rendered figures, 4 tables, and 35 references.
Malignant Lesions of the Pancreas
Authors: Basar Omer , Kadayifci Abdurrahman , Brugge William R.
Published: January 2021

Malignant lesions of pancreas are the fourth most common cause of cancer death in men and women. The majority of pancreatic cancer results from malignant transformation of the exocrine pancreas, and nearly 90% are ductal adenocarcinomas (pancreatic ductal adenocarcinoma, PDAC). Patients typically present at an advanced stage with a poor prognosis. PDAC is acquired through the accumulation of multiple genetic mutations. The major risk factors for PDAC include age, smoking, chronic pancreatitis, diabetes mellitus (DM), male gender, and African American race. Less commonly, hereditary syndromes may be implicated. The clinical presentation may involve weight loss, abdominal discomfort, and or jaundice. Painless jaundice, depression, and new-onset DM can suggest the diagnosis. Cross-sectional imaging has utility in diagnosis and staging. Endoscopic ultrasound (EUS) with fine needle aspiration (FNA) is a standard approach to tissue diagnosis. Endoscopic retrograde cholangiopancreaticography with palliative stenting can relieve obstructive jaundice. Surgical resection is the only potentially curative option in the management of PDAC but only a minority of patients are candidates for resection. The prognosis for most patients with pancreatic adenocarcinoma is poor. Less common pancreatic malignant lesions such as neuroendocrine tumors (NETs) have a much more favorable prognosis.
 
Key words: Pancreatic cancer; Pancreatic ductal adenocarcinoma; Pancreatic NETs (PNETs); Pancreatic neuroendocrine tumors
Benign and Premalignant Lesions of the Pancreas
Authors: Kadayifci Abdurrahman , Brugge William R.
Published: January 2021

Pancreatic cancer is among the most aggressive human cancers, with few symptoms and clinical findings until it is detected in an advanced stage. Today, the only favorable treatment is surgical resection before progression to an invasive stage. There are a variety of benign and premalignant conditions of the pancreas that may appear similar to malignant tumors; early detection and management of those premalignant pancreatic lesions may provide a significant improvement in patient outcome. This review covers cystic neoplasms of the pancreas, premalignant solid lesions of the pancreas, and inflammatory lesions of the pancreas. Figures show a 30 mm diameter hypoechoic lesion without septae in the pancreatic head, an oval 20 mm diameter pancreatic mass with mixed solid and cystic components, a 24 mm diameter hypoechoic mass with well-defined borders in the tail of the pancreas, a 22 × 18 mm cystic lesion with a thick wall and internal debris in the head of the pancreas, and an endoscopic cyst gastrostomy after balloon dilation. Tables list World Health Organization (WHO) 2010 classification of pancreatic tumors, classification of pancreatic cystic lesions, main characteristics of common pancreatic cystic lesions, high-risk stigmata and worrisome features of intraductal papillary mucinous neoplasm on cross-sectional imaging, and WHO classification of pancreatic neuroendocrine tumors.
 
This review contains 5 highly rendered figures, 5 tables and 55 references
Primary Sclerosing Cholangitis
Authors: Navaneethan Udayakumar
Published: January 2021

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disease characterized by inflammation and fibrosis that may involve the entire biliary tree. The fibrosis causes diffuse narrowing of the intrahepatic and extrahepatic bile ducts, and the resulting biliary stasis leads to secondary biliary cirrhosis and associated complications. This review addresses PSC through its epidemiology, etiopathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Figures show pouchoscopy, liver biopsy, and endoscopic retrograde cholangiopancreatography; direct peroral cholangioscopy visualization of cholangiocarcinoma; and algorithms depicting diagnosis of cholangiocarcinoma and screening for inflammatory bowel disease (IBD) in patients with PSC. Tables list characteristics of IBD in PSC, prevalence of antibodies in PSC, differential diagnosis of PSC, diagnostic approach in PSC, and risk of cancers in PSC.
This review contains 6 highly rendered figures, 5 tables, and 91 references.
Metabolic Liver Diseases: Wilson Disease and alpha 1-Antitrypsin Deficiency
Authors: Avissar Uri
Published: January 2021

Two significant metabolic diseases of the liver are Wilson disease (WD) and α1-antitrypsin deficiency (AATD). WD is defined as an autosomal recessive disorder caused by mutations in the ATP7B gene of copper metabolism, leading to copper accumulation in multiple organs, notably the liver and brain, with ensuing injury giving way to hepatic and neuropsychiatric symptoms. AATD is also a genetic disorder but is distinguished by the production of a defective protease inhibitor, which leads to hepatic and pulmonary injury. This review of WD and AATD addresses their respective epidemiologies, genetics, pathophysiologies, diagnoses, differential diagnoses, managements, complications, and prognoses. Figures show the pathophysiology, clinical presentation, and liver histology of WD; suggested approaches to the diagnosis of WD, suspected WD, and family screening of first-degree relatives of WD patients; monitoring therapy in WD disease; schematic representation of the α1-antitrypsin (AAT) mechanism of action and polymerization of the Z mutation variant; pathophysiology and clinical presentation of AATD; periodic acid–Schiff–positive diastase-resistant globules in AATD; and suggested approach to AAT diagnosis and management. Tables list the causes of low ceruloplasmin, medications used in WD, and AAT variants and risk of disease.
This review contains 12 highly rendered figures, 3 tables, and 167 references.
Alcoholic Liver Disease
Authors: Krisko Tibor , Baffy György , Vogel Alexander S.
Published: January 2021

Alcohol-associated liver disease encompasses all forms of liver injury related to the consumption of alcohol, one of the most common hepatotoxic agents in the world. The spectrum of this disease ranges from steatosis, which is present in everyone who drinks alcohol in excess, to cirrhosis, which occurs in approximately 10 to 15% of individuals with alcohol abuse and conveys an annual risk of 1 to 2% for the development of hepatocellular carcinoma. Despite the prevalence of alcohol-associated liver disease and its profound impact on health, questions remain surrounding its pathogenesis and management. This review of alcohol-associated liver disease addresses the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis and comorbidities, treatment, complications, measures of quality of care, and prognosis and outcome measurements.
This review contains 6 highly rendered figures, 6 tables, and 50 references.
Keywords: Alcohol-associated Liver Disease (ALD), alcohol, cirrhosis, liver injury, Alcohol Use Disorder (AUD), alcohol-associated hepatitis, substance abuse
Nonalcoholic Fatty Liver Disease
Authors: Fuchs Michael , Puri Puneet
Published: January 2021

Nonalcoholic fatty liver disease (NAFLD) is defined by the pathologic accumulation of fat in more than 5% of hepatocytes in the absence of significant alcohol consumption (daily intake < 20 g in women and < 30 g in men) and by excluding secondary causes of hepatic steatosis. NAFLD can be categorized into two principal phenotypes: nonalcoholic fatty liver (NAFL) and nonalcoholic steatohepatitis (NASH). NAFL is defined by the presence of macrovesicular steatosis without inflammation, whereas NASH sees inflammation and hepatocyte ballooning injury, is associated with varying degrees of fibrosis, and can progress to cirrhosis and end-stage liver disease. This review addresses the epidemiology, etiology, pathophysiology, diagnosis, treatment, and prognosis of NAFLD. Figures show the spectrum of fatty liver disease, hepatic consequences of insulin resistance, role of liver biopsy in evaluation of NAFLD, histologic features of NASH, and principles of NAFLD. Tables list risk factors and clinical manifestations for NAFLD, physical examination findings in NAFLD, comparison of imaging modalities, and noninvasive fibrosis markers.

This review contains 6 figures, 5 tables and 166 references
Key words: Nonalcoholic fatty liver disease, nonalcoholic steatohepatitis, non-invasive assessment, vibration controlled transient elastography, magnetic resonance elastography, diet and lifestyle treatment
Hereditary Hemochromatosis
Authors: Chang Matthew S , Smith Benjamin N
Published: January 2021

Hereditary hemochromatosis is an inherited iron overload disorder that can result in liver and other end-organ involvement and injury. The phenotypic expression ranges from asymptomatic to end-stage liver disease and can be separated into three stages. This review covers the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, treatment, complications, and prognosis of hereditary hemochromatosis. Figures show the fraction of patients with mutations for hemochromatosis and clinical manifestations, the regulation of iron by hepcidin, physical examination findings in hemochromatosis, a diagnostic algorithm for hemochromatosis, and a treatment algorithm for hemochromatosis. Tables list hemochromatosis disease stage according to the European Association for the Study of the Liver, genetic mutations in hemochromatosis, secondary (non-hemochromatosis-related) causes of iron overload, indications for liver biopsy in patients with hemochromatosis, and clinical manifestations in hemochromatosis.
This review contains 5 highly rendered figures, 5 tables, and 32 references
Key words: Hemochromatosis; Hereditary hemochromatosis; Iron overload; Iron regulatory pathways; Hepatic  iron; Hepcidin
Drug-Induced Liver Injury
Authors: Tran Thanh , Lee William M.
Published: January 2021

Drug-induced liver injury (DILI) refers to liver damage caused by over-the-counter and prescription drugs as well as herbal and dietary supplements. Hepatocytes, the main factory cells of the liver, are the primary targets of drug-related injury. During hepatocyte injury, loss of cell integrity results in a leak of liver contents, including a variety of enzymes, into the circulation. Currently, more than 1,000 drugs and herbal products have recognized hepatotoxic properties, with acetaminophen (APAP) being the most common cause of acute liver failure in the United States and amoxicillin-clavulanate the most common cause of severe liver injury worldwide. This review of DILI addresses the epidemiology, classification, pathophysiology, clinical manifestations, diagnosis, common idiosyncratic drug reactions, unusual drug reactions, treatment, and prognosis, with special sections on APAP hepatotoxicity and the approval of prescription drugs by the Food and Drug Administration. Figures show histologic features of DILI, mechanisms of liver injury, genome-wide association study results for lumiracoxib, APAP metabolism, and the Rumack-Matthew nomogram. Tables list classification of DILI, features of idiosyncratic drug reactions, histologic patterns of DILI, haplotypes associated with specific drug-related disorders, and the Roussel-Uclaf causality assessment method.
This review contains 5 highly rendered figures, 5 tables, and 54 references.
Primary Biliary Cirrhosis
Authors: Pratt Daniel S. , King Lindsay Y.
Published: January 2021

Primary biliary cirrhosis (PBC) is a progressive autoimmune disease of the liver. It is the most common cause of chronic intrahepatic cholestatic liver disease in adults. This review addresses the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of PBC. Figures show the pathogenesis and natural history of PBC and histologic features of the four stages of PBC. Tables list diagnostic criteria for PBC via the American Association for the Study of Liver Diseases, differential diagnosis for PBC, medications used to treat PBC, secondary therapy for PBC, and follow-up of patients with PBC.
This review contains 2 highly rendered figures, 5 tables, and 45 references.
Failure to Thrive in Infants and Toddlers
Authors: Phadke Madhura Y , Porto Anthony F
Published: January 2021

Failure to thrive (FTT) is a broad term that is used to document an abnormal pattern of weight gain over time. There is no single definition for FTT, but all proposed definitions use anthropometric parameters such as weight gain or weight for length. The term FTT has been falling out of favor, and the term weight/growth faltering is becoming more common to describe this clinical entity. The underlying problem in FTT is inadequate usable calories. The primary mechanisms leading to FTT are impaired absorption, increased metabolic demands, and inadequate caloric intake. Inadequate caloric intake is the most common of these mechanisms, although FTT is often a combination of the three. The diagnostic evaluation of FTT must take into account the multifactorial nature of this clinical sign. A comprehensive history is essential for diagnosis and should include specific questions about the child’s living situation and feeding habits. The physical examination must include accurate weight and length measurements. Clinicians should look for signs of abuse or neglect, dysmorphic features, abnormal skin or nail findings, digital clubbing, or other signs of chronic disease. Laboratory investigations are rarely revealing in FTT but should be considered if there is a high index of suspicion for underlying disease. Treatment in FTT favors a multidisciplinary approach. The primary goal of treatment is restoration of normal growth velocity. Children with FTT are at increased risk for growth and cognitive problems in later childhood, although the clinical significance of these findings is not well understood. The mainstay of treatment is increasing calories in the diet. Enteral feeding, orally or via a tube, is always preferred over parenteral feeding due to a better safety profile, ease of feeding, and lower cost. Parenteral nutrition is an acceptable way to meet caloric needs in infants and children when enteral nutrition is not possible. Children with FTT and malnutrition should be monitored closely for refeeding syndrome, which results from fluid and electrolyte shifts in malnourished children. In general, FTT can be treated on an outpatient basis with close follow-up. Indications for hospitalization include severe malnutrition/dehydration and concern for child endangerment.
Key words: enteral feeding, failure to thrive, growth charts, nutrition, parenteral nutrition, poor weight gain, tube feeding, weight loss
Parenteral Nutrition Associated Liver Toxicity: Prevention, Diagnosis and Management
Authors: Baker Meredith A. , Anez-Bustillos Lorenzo , Dao Duy T. , Fell Gillian L. , Gura Kathleen M. , Puder Mark
Published: January 2021

Long-term parenteral nutrition (PN) treatment is limited by parenteral nutrition–associated liver disease (PNALD), which is characterized initially by intrahepatic cholestasis, typically defined as a direct bilirubin greater than 2 mg/dL in the absence of other causes of liver disease. PNALD is typically less common and less severe and progresses more slowly in older children and adults than in infants. The etiology of PNALD is multifactorial. Key factors include immature liver function, sepsis, and a lack of enteral nutrition. Additionally, nearly every component of PN has been attributed to or exacerbated hepatotoxicity. PN preparations must be carefully individualized and monitored to minimize hepatotoxicity from its various components. Although many hepatotoxic components or imbalances have been recognized, soybean oil–based lipid emulsions continue to be widely used as they are the only lipid emulsions currently approved for PN by the Food and Drug Administration. Fish oil–based lipid emulsions have been shown to reverse PNALD, with an associated decrease in mortality. As such, fish oil therapy should be considered early once biochemical cholestasis is detected in PN-dependent patients. Studies investigating the use of novel lipid emulsions for prevention and treatment of PNALD are ongoing.  

This review contains 5 figures, 5 tables, and  114 references.
Management of Cardiovascular Disease in Dialysis Patients
Authors: Roberts John K. , Middleton John P.
Published: January 2021

Cardiovascular disease is a common cause of death and disease in patients with end-stage renal disease (ESRD). Registry data show that 41% of deaths in ESRD patients are due to a variety of cardiovascular causes, such as acute myocardial infarction, congestive heart failure, arrhythmia/sudden cardiac death, and stroke. In the general population, each of these disease entities in isolation can be effectively managed according to evidence from large clinical trials and evidence-based guidelines. However, many of these trials did not include patients with ESRD, limiting the transferability of this evidence to the care of patients on dialysis. To complicate matters, cardiovascular events in ESRD patients are likely augmented from a unique interplay of cardiac risk due to both reduced kidney function and the necessity for artificial renal replacement therapies. In this light, the patient on dialysis is subjected to a series of unique factors: the continued presence of the metabolic perturbations of uremia and the peculiar environment of the dialysis treatment itself. Since the ESRD heart is under a considerable amount of strain due to chronic volume overload, rapid electrolyte and fluid shifts, and accelerated vascular calcification, management can be complex and outcomes multifactorial. In this review, we summarize the current evidence regarding management of acute myocardial infarction, heart failure, sudden cardiac death, and atrial fibrillation. We also address modifiable risk factors related to the dialysis procedure itself and highlight recent randomized controlled trials that included dialysis patients and measured important cardiovascular outcomes.
Women's Neurology
Authors: O’Neal M. Angela
Published: January 2021

This review details some of the features important to consider in women with neurologic disease, including medication and disease effects on both reproductive health and pregnancy/fetal development, as well as hormonal effects on neurologic disease. A case-based approach is used to discuss diseases that affect women throughout their life cycle (multiple sclerosis [MS] and epilepsy), disorders that affect only women (eclampsia), and those that affect women preferentially (migraine, cerebral venous thrombosis, reversible cerebral vasospasm, and Alzheimer disease). The epidemiology, differential diagnosis, pathophysiology, management, and prognosis are reviewed for each disorder. Tables include US Food and Drug Administration pharmaceutical pregnancy categories, learning objectives, migraine with aura, alternative diagnostic criteria for migraine without aura, migraine aura versus transient ischemic attacks, red flags to secondary headache, abortive headache therapy in pregnancy, migraine preventive medications and pregnancy, MS therapies in pregnancy, pregnancy consulting points for MS patients on a disease-modifying therapy, and general recommendations for women with epilepsy and pregnancy. Figures show fluid-attenuated inversion recovery and gradient echo magnetic resonance images of the right anterior parietal region; a magnetic resonance venogram demonstrating occlusion in the superior sagittal sinus; posterior reversible encephalopathy syndrome; and computed tomographic (CT) images of a hemorrhage in the left parietal region, left frontal subarachnoid bleeding, and left middle cerebral beading.
This review contains 5 highly rendered figures, 11 tables, and 29 references.
Anemia: Production Defects Generally Associated with Marrow Erythroid Hyperplasia and Ineffective Erythropoiesis
Authors: Berliner Nancy , Gansner John M
Published: January 2021

This review focuses on anemia resulting from production defects generally associated with marrow erythroid hyperplasia and ineffective erythropoiesis. The definition, epidemiology, etiology, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis of the following production defects are discussed: Megaloblastic anemias, megaloblastic anemia caused by cobalamin deficiency, megaloblastic anemia caused by folic acid deficiency, copper deficiency, and sideroblastic anemias. Figures depict intracellular interdependent cofactor activity of cobalamin and folic acid, synthesis of succinyl–coenzyme A from methylmalonyl–coenzyme A, folic acid functions as a coenzyme in single-carbon transfer reactions, cobalamin assimilation, peripheral blood smear in folic acid or cobalamin deficiency, and a Prussian blue stain showing ring sideroblasts. Tables list causes of cobalamin deficiency and folic acid deficiency.
This review contains 5 figures; 2 tables; 95 references.
Occupational and Environmental Lung Diseases Selected Pneumoconiosis
Authors: Sack Cora S , Vedal Sverre , Kaufman Joel D
Published: January 2021

Environmental and occupational lung diseases encompass a diverse group of lung diseases caused by the inhalation of potentially harmful substances. This review provides an in-depth discussion of the pneumoconioses, hypersensitivity pneumonitis, chronic beryllium disease, and other occupational lung diseases that affect the parenchyma. For each disease, the review presents the epidemiology, biologic mechanisms when known, diagnosis, and clinical care. The review includes tables with different pneumoconioses, occupations and industries associated with silicosis and asbestosis, and some selected causes of hypersensitivity pneumonitis, as well as chest radiographs, computed tomographic scans, and/or pathologic slides of some selected diseases.
This review contains 9 figures, 4 tables, and 68 references.
Key words: asbestosis, asbestos-related pleural plaque, coal worker’s pneumoconiosis, chronic beryllium disease, hard metal pneumoconiosis, hypersensitivity pneumonitis, pneumoconiosis, progressive massive fibrosis, silicosis