Explanation: Bipolar I is characterized by extreme mania that includes symptoms such as lack of sleep, impulsivity, risky behavior activities, high volume and rate of speech, flight of ideas, elated and expansive mood, increased activity, psychomotor agitation, grandiosity, and self perceived talents. The patient in this question has been exhibiting excessive risk taking behaviors for over 7 days that include excessive spending of money, hypersexual tendencies, and increased impulsivity. Choice A- Schizophrenia Paranoid Type is incorrect because this diagnosis requires the presence of active delusions and hallucinations, disorganized behavior and speech, and negative symptoms. Choice B- Cyclothymia is incorrect because it is a less severe form of bipolar disorder, with alternating periods of hypomania and moderate depression. Symptoms also must be present for at least 2 years. Choice D- Borderline Personality Disorder is incorrect because this diagnosis requires a pattern of unstable personal relationships, self-image, and affect that are seen as efforts to avoid abandonment, unstable relationships, and recurrent suicidal behavior.
Discussion:
Bipolar Disorder, also formerly known as manic-depressive disorder is a mental illness that results in dramatic shifts in mood and energy that may disrupt a patient’s ability to complete their day-to day tasks (1). This mental condition causes mood swings that are more excessive than the typical ups and downs, they are characterized by extreme emotional highs known as mania or hypomania and extreme lows that are consistent with depression (2). Although the average age of onset is 25, patients’ can be diagnosed at any age, most uncommonly during childhood. Bipolar disorder is equally as common in men as in women. In the U.S 2.6% of the population has been diagnosed with bipolar disorder, although this number is thought to be widely underreported. Within the number of diagnosed cases 83% have been classified as severe. Suicide rates are currently higher in men. (3).
Currently, there are four different basic subcategories of bipolar disorder. Bipolar I disorder is diagnosed by manic episodes that at minimum lasts 7 days, or symptoms that are severe enough to require hospitalization. Typically, depressive symptoms are also present and lasting about two weeks. In this category it is also possible for depression with mixed features to also occur which is having manic and depressive symptoms at the same time. Bipolar II disorder is diagnosed by a patient having a pattern of depressive episodes and hypomanic episodes, but absence of full blown manic episodes. Cylcothymic Disorder is diagnosed by frequent periods of hypomanic symptoms as well as frequent periods of depressive symptoms lasting for at least 2 years. The symptoms of this disorder do not meet the diagnostic criteria for a hypomanic episode or a depressive episode. The final subcategory is just a category that doesn’t fit any of the above mentioned criteria. (1)
To date a single cause of Bipolar Disorder has not been found but there are several factors that are thought to contribute to the disorder. The first factor is genetics, it is thought that a family history of bipolar disorder increases a patient’s overall risk. The second factor is stress, it has been found that a stressful event in one’s life may trigger a manic or depressive episode. The third factor is brain structure and function, currently there isn’t a brain scan that can diagnose bipolar disorder but researchers have found that people with bipolar versus people without have subtle difference in average size and activation of some brain structures. The fourth factor is biochemical factors, there is thought to be an up regulation of monoamine neurotransmission and receptor function as well as changes in the limbic system and prefrontal cortex in patients with Bipolar Disorder. (3)
Diagnosis of a manic state in a patient with bipolar disorder can be characterized by lack of sleep, impulsivity, risky behavior activities, high volume and rate of speech, flight of ideas, elated and expansive mood, increased activity, psychomotor agitation, grandiosity, and self perceived talents. Bipolar II is mostly characterized by Major Depressive Disorder and at least one hypomanic episode during the patient’s life. Rapid cycling may be more common in Bipolar I than Bipolar II. Differential diagnosis and etiology is very similar is Bipolar I versus Bipolar II (1).
For Bipolar I and Bipolar II, treatment options include mood stabilizers which are the mainstay and can be used for both acute and maintenance treatment. These include lithium which is gold standard, valproic acid, and lamotrigine/lamictal. Antipsychotics such as Zyprexa, Risperdal, and Seroquel may also be used especially for mania with psychosis since it is faster acting than a mood stabilizer. Antidepressants may also be prescribed but not usually as a monotherapy. For patients on Lithium antidepressants are useful in the severely depressed. Some non pharmalogical treatment include electroconvulsive therapy, psychotherapy such as CBT, good social support, and economic stability. It has been found that psychopharmatherapeutics and psychotherapy leads to the best outcome. For these patients the prognosis varies on level of treatment and adherence, but most will have a second episode 2-4 years after the first event (2).
References:
Bipolar Disorder. National Institute of Mental Health. https://www.nimh.nih.gov/health/topics/bipolar-disorder/index.shtml. Accessed September 23, 2017.

Bipolar disorder. Mayo Clinic. http://www.mayoclinic.org/diseases-conditions/bipolar-disorder/home/ovc-20307967. Published February 15, 2017. Accessed September 23, 2017.

NAMI. Bipolar disorder | NAMI: National Alliance on Mental Illness. https://www.nami.org/Learn-More/Mental-Health-Conditions/Bipolar-Disorder. Accessed September 23, 2017.

X-ray findings for a tension pneumothorax would show increased radiolucency on the side of the pneumothorax where the air is collecting in the pleural space, tracheal and mediastinal deviation to the contralateral side of the pneumothorax due to the compression caused by the air leak. Because of this, answer A is correct, as it is not a radiologic finding consistent with a tension pneumothorax. Choices B, C, and D are all radiologic findings in a tension pneumothorax.

DISCUSSION:
The patient in the above case was diagnosed with a spontaneous tension pneumothorax. A pneumothorax occurs when there is a presence of air in the pleural cavity and can occur for a variety of different reasons and can have different levels of severity. A tension pneumothorax is “a life-threatening condition that develops when air is trapped in the pleural cavity under positive pressure, displacing mediastinal structures and compromising cardiopulmonary function” (Daley, 2016). Patients are at the highest risk for developing a pneumothorax during the neonatal period. The incidence in neonates is 1-2% (Daley, 2016). Neonates who are male, preterm, delivered by c-section, low birth weight, low 1 minute Apgar scores, require resuscitation at birth, have RDS, PIE and receive ventilator treatment are at high risk for developing pneumothoraxes as represented by the patient in the above case. Other risk factors include receiving positive pressure ventilation or CPAP, vacuum extraction, pneumonia, pulmonary hypoplasia, CPR, perinatal asphyxia and urinary tract anomalies. The incidence of pneumothorax development has increased since the start of ventilator use in neonates. In those receiving positive pressure ventilation, the risk is 15-30% (Gomella, 2013). The incidence of tension pneumothorax is unknown.
Pneumothorax can either be spontaneous or due to trauma. There are two types of spontaneous pneumothorax (primary and secondary). Primary spontaneous pneumothoraxes are without known pulmonary disease and usually occur in tall, thin younger males. They are more at risk if they are smokers, or have a positive family history however; this cause is rare during the neonatal period. A secondary spontaneous pneumothorax occurs in the presence of pulmonary disease, such as Pulmonary Interstitial Emphysema or RDS as seen in the above case. PIE is seen almost exclusively in very low birth weight infants on ventilatory support like Twin Boy B and usually develops in the first 48-72 hours of life (Gomella, 2013). In adults, diseases such as COPD put you at increased risk for a pneumothorax. A pneumothorax can also be iatrogenic from ventilation use or traumatic.
In a pneumothorax, air gets displaced into the pleural space due to the pressure gradient of either the alveoli and pleural space or the pleural space and the atmospheric pressure (traumatic). Alveoli are under a greater pressure than the intrapleural space, with the pressure in the intrapleural space being less than atmospheric pressure. Normally, a pneumothorax will “seal itself off” when the pressure between the two is equalized. Tension pneumothorax can result from any of these when the intrapleural pressure exceeds the atmospheric or alveolar pressure (Epocrates, 2017). This acts as a one-way valve and does not allow for air to escape (back into the lung or through a trauma induced puncture) with exhalation (Gamella, 2013). The pressure in your thoracic cavity increases causing collapse of the lung tissue/volume. If it is a tension pneumothorax, the pressure continues to collapse the lung and causes a shift of the trachea and mediastinum to the contralateral side. This further causes an increase in pulmonary vascular resistance and central venous pressure. With the heart compressed, cardiac output and the venous return to the heart can become decreased. The result is hypoxia and acute respiratory failure (Gamella, 2013).
As for history and physical findings, it becomes more challenging to catch with neonatal patients. Generally, a tension pneumothorax presents itself as a medical emergency with sudden and rapid deterioration of the patient. Especially in neonates, this is not always that apparent and can present more subtly as with the patient’s presentation in the above case. Signs to look for in the infant include grunting, irritability, and/or restlessness. Most commonly, a patient will present with worsening chest pain, dyspnea, anxiety or fatigue and occasionally epigastric pain. However, neonates cannot provide you with this information so you have to rely on physical exam. Physical exam findings include cyanosis, hypoxia, tachypnea, increased work of breathing, retractions, diminished or absent breath sounds, and an asymmetric chest rise. If mechanically ventilated, you may see an increase in oxygen demand. The cardiovascular exam can show tachycardia or bradycardia, an increase in blood pressure with narrowed pulse pressure and eventually hypotension (Gomella, 2013). The PMI will be displaced to the contralateral side. Infants may present with a “cyanotic upper half and pale lower half” of their body (Gomella, 2013). Not as commonly in patients you may see JVD and tracheal shift and have hyper resonance on percussion (Epocrates 2016). Pneumothoraxes are found incidentally in 1-2% of neonates without any suggesting signs or symptoms (Gomella 2013). When looking at labs, a blood gas may show respiratory acidosis as seen with the above patient giving a decreased PaO2 and and increased PCO2.
Imaging that is useful in the diagnosis of a pneumothorax include ultrasound, xray and on rare occasions CT if patient is hemodynamically stable with a high index of suspision but a pneumothorax cannot be ruled out by CXR/US. In neonates, transillumination of the thorax may also be used. Ultrasound will show an absence of lung sliding/absence of the normal “sea shore” sign. In a tension pneumothorax a CXR will show a shift in the mediastinum and trachea to the contralateral side of the air leak, depression of the diaphragm on the ipsilateral side, a radiolucent band of air with a lack of lung markings on the ipsilateral side of the air leak and decreased radiolucency on the contralateral side of the air leak where the lungs are being collapsed (Gorrochategui, 2017).
Treatment differs for a tension vs. nontension pneumothorax. If suspecting a tension pneumothorax, do not delay for confirmation on CXR. If the air is not evacuated immediately it can be fatal. Treatment includes a needle decompression followed by chest tube placement on the side of the air leak with a confirmatory CXR after decompression/tube placement. Chest tube placement is not always necessary for pneumothorax following needle decompression, especially if the patient is not using any form of mechanical ventialation. The needle can be placed at either the second intercostal space – midclavicular line or the fourth intercostal space – anterior axillary line with needle insertion on top of the rib. Removal of the chest tube requires pain medication and can be done once there is no longer bubbling of the underwater seal or presence of air on xray for 24-48 hours. If hemodynamicaly stable and a non-tension pneumothorax, conservative measures such as oxygen supplementation may be all that is necessary. If patient is currently on or requiring mechanical ventilation, High Frequency Oscillatory Ventilation is preferred as it has lower mean airway pressures and is a gentler mode of ventilation for these patients (Gomella, 2013). If patient is hyperinflated or has atelectasis after reinflation, bronchodilators can be used as well as proper positioning of the infant (hyperinflated side down/atelectatic side up).
With the above case the differential diagnosis included pneumothorax, displaced ET tube, pneumonia, pneumomediastinum, pleural effusion, pericardial effusion, pneumopericardium, anemia. Although pneumothorax was high on suspicion, due to baby’s risk factors of RDS, PIE and mechanical ventilation, the diagnosis of tension pneumothorax was quite surprising. The infant had subtle signs of respiratory distress but was not at all hemodynamically unstable. Bacteremia/sepsis would have been considered however this could be ruled out with blood cultures showing no growth for 48 hours. Obstruction of the infant’s nares by a mucus plug was ruled out after no relief after suction by the nurse. The displaced ET tube was the most likely and most easily correctable diagnosis second to a pneumothorax but this was ruled out with the XRAY along with the pleural effusion, pericardial effusion and pneumopericardium. Anemia could have contributed to some of the symptoms but not all and could not be the cause of the pneumothorax; this was eventually corrected with a blood transfusion. Pneumomediastinum would have resulted in an elevated appearance of the thymus with air outlining the heart. Pneumopericardium and tension pneumothoraxes can both present with rapid deterioration of the patient although the patient did not present in this way. Pneumothorax is the more common of these diagnoses and the diagnosis can be differentiated on CXR. Pneumonia was a likely diagnosis before the XRAY as the patient had multiple risk factors and had a subtle presentation of symptoms. Infants with pneumonia may also have decreased breath sounds, rales, and tachypnea but may also present with increased respiratory secretions, fever/hypothermia and an altered white blood cell count.

References
Daley BJ. Pneumothorax. http://emedicine.medscape.com/article/424547-overview. Published August 18, 2017. Accessed September 24, 2017.
Gomella TL, Cunningham MD, Eyal FG. Neonatology: management, procedures, on-Call problems, diseases, and drugs. 7th ed. New York, NY: McGraw-Hill Education Medical; 2013.
Pneumothorax History & Exam – Epocrates Online. https://online.epocrates.com/diseases/50433/Pneumothorax/History-Exam. Accessed September 24, 2017.
Gorrochategui M. Pneumothorax | Radiology Reference Article. Radiopaedia.org. https://radiopaedia.org/articles/pneumothorax. Accessed September 24, 2017.

Correct Answer: (D) The patient is presenting admitting to an acetaminophen overdose. He is stating the timeframe and the dose that was ingested. The appropriate treatment is to get an acetaminophen concentration and treat the patient with NAC if appropriate, which it most likely is in this case due to the large amount consumed.
Incorrect Answers: (A) Even though a psych eval and 72-hour hold may indeed be necessary this is not the correct treatment as this doesn’t treat the active issue of possible hepatoxicity.
(B) Discharging the patient home is incorrect because we don’t know the patient’s level of toxicity and therefore can’t predict his risk of hepatoxicity.
(C) This is incorrect because activated charcoal is usually only used within the first hour after ingestion.

Discussion:
Acetaminophen is one of the most widely used over the counter oral analgesic and antipyretic. It is usually well tolerated and very safe when administered in the therapeutic dose range. When the dosage exceeds the therapeutic range, hepatotoxicity can occur. This is becoming increasingly common in the United States and acetaminophen toxicity has now replaced viral hepatitis as the most common cause of acute hepatic failure (1). Acetaminophen hepatotoxicity is estimated to account for approximately 48% of all acute liver failure diagnoses. In addition, some studies have shown that 29% of patients with acute liver failure secondary to acetaminophen toxicity end up undergoing a liver transplant, and ultimately have a 28% mortality rate (3).
On average it takes approximately 4 hours from ingestion to peak acetaminophen level in the blood, with liquid formulations peaking somewhat quicker. Even the extended-release preparations are almost entirely absorbed by the body at the 4-hour mark. Primarily the liver metabolizes acetaminophen, which is why in overdose it is hepatotoxic. The majority of the metabolism is completed via Glucuronidation and sulfation. The remaining 5%-15% is oxidized through the p450 system, largely CYP2E1 (2).
Acetaminophen overdoses happen both intentionally and unintentionally. The signs and symptoms can be pretty difficult to catch if the patient doesn´t present admitting to the overdose. Acute ingestions of acetaminophen are single ingestions occurring within a 4-hour period. When the patient presents with a clear history and a timeframe of less than 4 hours from ingestion, the protocol for treatment is pretty clear. Chronic ingestions on the other hand are not so clear-cut. A chronic ingestion is defined as multiple ingestions or a timeframe greater than 4 hours (2). Patients are usually asymptomatic for the first 24 to 48 hours. When they do start showing clinical signs it is due to end organ toxicity and the patient´s life is in danger (1). It is important for the clinician to know the time at which the overdose occurred and how much was ingested if possible. Minimum toxic doses of acetaminophen in a single ingestion that have severe risk of hepatotoxicity are 7.5 to 10g in adults and 150 to 200mg-kg in healthy children aged 1 to 6 years (1). Poor prognostic signs include multi-organ failure, which can include cerebral edema, renal failure, profound hypoglycemia and lactic acidosis, any signs of which should prompt an immediate liver transplant evaluation. As stated before most of the signs of unintentional toxic ingestion typically present later on, when the complications and end organ damage is already apparent (3).
Treatment for acetaminophen overdoses consists of a few different things based on the time of ingestion, the amount the patient ingested or the level in the patient’s blood and the liver function. A nomogram called the Rumack-Matthew nomogram is used to calculate PO/IV N-Acetylcysteine (NAC) dosing for acetaminophen overdose and to determine toxic 4-hour acetaminophen levels. It looks at the acetaminophen concentration and the time post ingestion to give an estimate about possible toxicity and if to treat (4). Activated charcoal can be used as a gastrointestinal decontamination agent during the immediate postingestion time, ideally within 1 hour. If the patient has an acetaminophen concentration below the “possible toxicity” line on the Rumack-Matthew nomogram, they can be discharged home after they are cleared medically. However, if the patient falls above the “possible toxicity” line, they need to be treated with NAC and hospitalized for observation. NAC is close to 100% hepatoprotective when administered within 8 hours of ingestion of acetaminophen, but can also still be beneficial to patients who present more than 24 hours post ingestion. NAC can be given in both oral and IV form. If the damage is too much for the liver to handle and the NAC to overcome, the only other option is liver transplantation (1).

References:
Farrell SE. Acetaminophen Toxicity. Practice Essentials, Background, Pathophysiology. http://emedicine.medscape.com/article/820200-overview?pa=Nr1y61Ko11XC7v8pvuZCnb8l1YOpVLo7yOfSsThn6h%2FAMipBdjDfyXPfOm8VaALLeiwSmQLHuFrfjJk2gj%2F4wy8HoCpv88EfsR0xVZ6JHe4%3D. Published August 17, 2017. Accessed September 12, 2017.
Mottram AM, Kumar AM. ACEP. Focus On: Acetaminophen Toxicity and Treatment // ACEP. https://www.acep.org/Clinical—Practice-Management/Focus-On–Acetaminophen-Toxicity-and-Treatment/. Published May 2007. Accessed September 12, 2017.
Yoon E, Babar A, Choudhary M, Kutner M, Pyrsopoulos N. Acetaminophen-Induced Hepatotoxicity: a Comprehensive Update. Journal of Clinical and Translational Hepatology. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4913076/. Published June 28, 2016. Accessed September 12, 2017.
Rumack BM. Acetaminophen Overdose and NAC Dosing. MDCalc. https://www.mdcalc.com/acetaminophen-overdose-nac-dosing. Accessed September 13, 2017.

B is the correct answer. PPI to decrease risk of GI bleed (upper: mainly due to patients presumed varices, from portal HTN). Folic acid and Thiamine for malnutrition and as a preventative measure for Wernicke’s encephalopathy. If there is any indication of alcohol withdrawal or DTs a CIWA should be initiated and Benzodiazepines used as indicated.
A is incorrect because the patient doesn’t have any known symptoms of acute cholecystitis or biliary cholic, cholestyramine can also be used in indicated.
C is incorrect because these supplements are not currently necessary.
D is incorrect because it would be used in place of prednisolone, if used at all. This drug was more common in the past but it not frequently used due to side effects. Recent studies show that this drug can prevent AKI during a hospital stay but the longer a patient is on it mortality increases.

Alcoholic Hepatitis is due to increased alcohol consumption over decades; binge drinking is less commonly the cause. Many hepatic conditions, alcoholic fatty liver disease, alcoholic hepatitis, and cirrhosis, are caused by alcohol abuse and can present with or without steatohepatitis. The amount of alcohol intake is thought to be about 3oz to 4oz daily for decades. Currently alcohol liver disease is the most common cause of chronic and end-stage liver disease with about 10% to 35% of patients who abuse alcohol having changes in liver histology.
On physical exam abdominal pain and jaundice are common. Hepatomegaly is occasionally observed; from fatty liver, hepatocyte injury and protein retention. A liver bruit, gynecomastia and spider angiomatas are features of severe alcoholic hepatitis. Ascites may also be present, due to underlying cirrhosis and portal hypertension, and would be an indication to preform a paracentesis for question of SBP.
A clinical diagnosis is made if the patient presents with jaundice, moderately elevated aminotransferases, an AST:ALT ratio ≥2, an increased bilirubin and a high INR. The addition of leukocytosis or a fever only strengthens the diagnosis. There are several clinical scoring systems that can be used to judge outcomes. Discriminate function, INR & Bili, predicts prognosis and the need pharmacotherapy. A value >32 requires corticosteroids or pentoxifylline and implies a 30d mortality ranging between 35% to 45%. LILLE score, age, albumin, bili days 1 & 7, creatinine and PT, predicts mortality in patients who do not respond to 7d steroid treatment. A score >0.45 indicates a poorer outcome. Child-Pugh score, tBili, albumin, INR, ascites, and encephalopathy, determines the necessity of liver transplant. MELD score, bili, creatinine & INR, is used to predict survival post transplant.
The main treatment is prednisolone. Prednisone is not used due to the known hepatic metabolism. If patients are unstable and corticosteroids are not improving symptoms patients should be worked up for a liver transplant. The work up is quiet extensive and can take some time. That is why it is important to get the ball rolling before the patient direly needs a transplant. Mortality rates among patients who do not receive pharmacologic therapy are variable. Risk factors for increased mortality are: age, AKI, high bilirubin, high INR, Leukocytosis, Alcohol consumption >4 oz/d, presence of infection, hepatic encephalopathy and UGIB. The primary causes of death are hepatic failure, GIB, and sepsis.
Recurrence is a possibility but the possibility of more severe complications is more striking. This is why it is so important to attend alcohol rehab and have GI follow up; screen for cirrhosis and other such complications. The screening may include; abdominal ultrasounds/MRI every 6 months and yearly EGD for varices if the patient has portal HTN.
Resources
Evangelos Akriviadis, Ravi Botla, William Briggs, Steven Han, Telfer Reynolds, Obaid Shakil. “Pentoxifylline improves short-term survival in severe acute alcoholic hepatitis: A double-blind, placebo-controlled trial” Gastroenterology. Volume 119, Issue 6, December 2000, Pages 1637-1648 http://www.sciencedirect.com/science/article/pii/S0016508500511834

Lucey, M.R., M.D., Mathurin, P., M.D., Ph.D., & Morgan, T.R., M.D. “Alcoholic Hepatitis”. New England Journal on Medicine 2009; 360:2758-2769June 25, 2009DOI: 10.1056/NEJMra0805786 p. 2758-2769. http://www.nejm.org/doi/pdf/10.1056/NEJMra0805786

Maddrey W.C. MD., Boitnott J.K. MD., Bedine M.S. MD., Webber F.L. MD., Mezey E. MD., White R.I. MD., “Corticosteroid therapy of alcoholic hepatitis” 1978 Gastroenterology Vol 75 No 2 p193-199 http://www.gastrojournal.org/article/0016-5085(78)90401-8/pdf

National institute on Alcohol abuse and alcoholism. “Drinking Levels Defined” https://www.niaaa.nih.gov/alcohol-health/overview-alcohol-consumption/moderate-binge-drinking

Thurzs, M.R, MD., Richardson, P, MD., Allison, M, Ph.D., Austin, A, MD., … Forrest, E.H, MD., “Prednisolone or Pentoxifylline for Alcoholic Hepatitis”. N Engl J Med 2015; 372:1619-1628April 23, 2015DOI: 10.1056/NEJMoa1412278. http://www.nejm.org/doi/pdf/10.1056/NEJMoa1412278

©2017 UpToDate, Inc. All Rights Reserved. “Alcoholic Hepatitis Clinical Manifestations and Diagnosis”. Topic 86934 Version 13.0 https://www.uptodate.com/contents/alcoholic-hepatitis-clinical-manifestations-and-diagnosis?source=search_result&search=alcohol%20hepatitis&selectedTitle=2~150

©2017 UpToDate, Inc. All Rights Reserved. “Management and Prognosis of Alcoholic Hepatitis”. Topic 87052 Version 22.0 https://www.uptodate.com/contents/management-and-prognosis-of-alcoholic-hepatitis?source=search_result&search=alcohol%20hepatitis&selectedTitle=1~150

Answer is C. Supraglottitis, inflammation of the epiglottis and adjacent structures, can present with a spectrum of chief complaints from sore throat to fever and airway compromise.2,7 It is most commonly of infectious etiology and in the past two decades became more common in the adult population.5,6 In some cases, the diagnosis can be made clinically, but radiographs, ultrasound and direct visualization aid in the evaluation of this condition.3 Even a low suspicion for supraglottitis warrants further assessment, as a patient’s clinical condition can rapidly decline.8 A management plan should be decided at initial presentation and centered around airway management.3 If a patient presents with drooling and signs of respiratory distress, as in this case, emergently securing the airway is a priority.1,8 This should be done with a skilled critical care team and otolaryngologist present, and include direct endoscopic evaluation (C) if the patient is currently able to maintain their own away.8 With potential for difficulty and complications, bedside intubation (B) should be avoided in cases of suproglottitis and the procedure should take place in a well-controlled, closely monitored environment.5 All patients presenting with infectious supraglottitis should receive empiric antibacterial coverage with IV ceftriaxone (A), however administration of this medication should not prolong initiation of airway intervention.3,5 Some studies advise the use of nebulized racemic epinephrine (D), IV steroids and supplemental oxygen but recent literature suggests that it has no effect on clinical outcome or length of ICU stay.3,4,8

References
1. Bizaki AJ, Numminen J, Vasama J-P, Laranne J, Rautiainen M. Acute supraglottitis in adults in Finland: Review and analysis of 308 cases. The Laryngoscope. 2011; 121(10):2107-2113.
2. Galitz YS, Shoffel-Havakuk H, Cohen O, Halperin D, Lahav Y. Adult acute supraglottitis: Analysis of 358 patients for predictors of airway intervention. The Laryngoscope. 2017; 127(9):2106-2112.
3. Lindquist B. Adult Epiglottitis: A Case Series. The Permanente Journal. November 2016.
4. Navaratnam AV, Smith ME, Majeed A, Mcferran DJ. Adult supraglottitis: a potential airway emergency that can present in primary care. British Journal of General Practice. 2015; 65(631):99-100.
5. Abdallah C. Acute epiglottitis: Trends, diagnosis and management. Saudi Journal of Anaesthesia. 2012; 6(3):279.
6. Woods, CR. Epiglottitis (supraglottitis): Clinical features and diagnosis. Up to Date. May 2017. Retrieved from https://www.uptodate.com/contents/epiglottitis-supraglottitis-clinical-features-and-diagnosis?source=search_result&search=epiglottitis&selectedTitle=1~42
7. Takata M, Fujikawa T, Goto R. Thumb sign: acute epiglottitis. BMJ Case Reports. 2016.
8. Woods, CR. Epiglottitis (supraglottitis): Management. Up to Date. September 2017. Retrieved from https://www.uptodate.com/contents/epiglottitis-supraglottitis-management?source=search_result&search=epiglottitis&selectedTitle=2~42

Answer is C. Polycythemia vera is a myeloproliferative disorder with an increase in Hgb, Hct, WBC’s and plateletes. It is most commonly seen in men ages 50-60 yrs old. Signs/symptoms are due to increase in RBC mass and can also include headaches, dizziness, blurred vision, pruritis after hot bath, fatigue, early saiety, flushed face and splenomegaly.
Essential thrombocythemia is also a myeloproliferative disorder that can have an increase in WBC and marked elevation in platelets (>600,000), but no increase in Hct. Symptoms include headache and dizziness.
Chronic myeloid leukemia is another myeloproliferative disorder with a large increase in WBC (>30,000/mcL). Patients may also have an increase in platelets, but a normal Hct. Symptoms include fatigue, SOB, weight loss, or abdominal discomfort from splenomegaly.
Secondary polycythemia is caused by hypoxia (ie: smoking). It results in an increase in Hct with normal WBC’s and platelets. Symptoms are usually related to the underlying cause. Physical exam may show cyanosis and clubbing. Splenomegaly would be absent.1
Reference: Hammond DB. Myeloproliferative Disorder, Bone Marrow Failure & the Myelodysplastic Syndrome. MCPHS University: Clinical Medicine III. October 2016.

DISCUSSION
Polycythemia vera (PCV) is an acquired chronic myeloproliferative disorder.2 Myeloproliferative disorders are caused by clonal defects of hematopoietic stem cells. These stem cells produce myeloid, erythroid, and platelet cells. Polycythemia vera causes an overproduction of all three of these cell lines, with erythrocytes, or red blood cells (RBC), being the most prominent.3 Red blood cells are produced from bone marrow when erythropoietin is secreted by the kidneys, in response to cellular hypoxia. The exact etiology of PCV is unknown, however most believe it is caused by a mutation in exon 14 of Janus Kinase (JAK2 V617F), which is a signling molecule found in 95% of PCV cases.4 JAK2 may play a role in causing an increase in the sensitivity of erythroid cells to erythropoietin.3 PCV occurs in all ages. It is commonly seen in 50-60 year old patients, with the average age of diagnosis 60 years. Sixty percent of patients are men. It occurs in all populations. The incidence is lower in Japan when compared to the United States and Europe. The incidence is 2/100,000 – 18/100,000 in adults and increases with age.1
PCV is also known as primary erythrocytosis, which means an increase in hematocrit or red blood cell mass in the absence of hypoxia. Patients should have a normal oxygen saturation on exam.3 This is different from secondary erythrocytosis, which is an increase in hematocrit from a response to another process (ie: hypoxia from smoking).2 Majority of patients with PCV are discovered incidentally. Symptoms occur from the increase in red blood cell mass. This includes hyperviscosity or thrombosis. Also headache, dizziness, tinnitus, blurred vision, early satiety, fatigue, epistaxis, easy bruising, or abdominal discomfort due to splenomegaly.3 Pruritis is a common symptom, especially after a hot bath or shower. This is due to the increase in WBC’s, specifically the basophils releasing histamine.4 Physical exam findings include splenomegaly, facial flushing/ruddy complexion, engorged retinal veins or injection of the conjunctiva, excoriation of the skin, prior thrombotic event (ie: stroke, DVT/PE, myocardial infarction, mesentaric or hepatic venous thrombosis, superficial thrombophlebitis).1
PCV should be suspected in any patient with an elevated hemoglobin/hematocrit or red blood cell mass and a normal O2 saturation. A hemoglobin >18.5 g/dL (or hematocrit >56%) in men or a hemoglobin >16.5 g/dL (or hematocrit >50%) in women is considered an elevated red cell mass. There is also a mild increase in the WBC’s and platelets.4 The white blood cell count is usually elevated to 10,000-20,000/mcL and the platelet count is variably increased.1 Upon obtaining elevated blood counts, it is important to repeat a CBC to see if the hemoglobin and hematocrit return to normal.3 If the level remain elevated, a serum erytropoietin level can be obtained. This level should be low because the bone marrow is overproducing RBC’s in the absence of erythropoietin. Low erythropoietin levels is highly specific for PCV. A splenic ultrasound can also be done to show splenomegaly.4 Peripheral blood should be tested for the JAK2 mutation, which is on chromosome 9p24. A bone marrow biopsy would show hypercellularity in all three cell lines.3 The diagnostic criteria for PCV is as followed: Major criteria includes increase in RBC mass demonstrated by an increase in hemoglobin and hematocrit, bone marrow biopsy shows hypercellularity, and JAK2 mutation presence. Minor criteria includes a decrease in serum erythropoietin levels. A proper diagnosis is required to have all three major or the first 2 major and the minor.2 It is also important to rule out other causes of erythrocytosis, such as hypoxia, familial polycythemic disorder, damaged EPO receptors, and tumor producing EPO inappropriately.3
There is no cure for PCV at this time. However, it is important to reduce the risk of thrombosis, decrease the symptoms, and lower the transition to AML/MDS.1 Therapeutic phlebotomy is the management of choice. It is done until a hematocrit of less than 45 is obtained.4 A standard 1 unit phlebotomy (500 mL) should decrease the hematocrit by 3%. Men can lose 1.5 – 2 units per week and women can lose 0.5 units per week. Phlebotomies are repeated as often as it is necessary to maintain a normal hematocrit level. Patients are at risk of venous thrombosis. Low-dose aspirin should be given to all patients, unless contraindicated, to help prevent thrombosis. High dose aspirin should be avoided due to increase in GI bleed. If thrombosis has occurred, than anticoagulation should be started. Anagrelide can be helpful in thrombocythemia, to reduce the amount of platelets. However, it is not first line treatment for PCV because it causes an increased risk of arterial thrombosis and bleeding. Myelosuppression therapy is also part of treatment for high-risk PCV patient or those who cannot tolerate phlebotomies (ie: poor venous access). High-risk patients are >60 years old or have history of thrombosis. Most commonly used is hydroxyurea.5 It inhibits cells with a high division rate.2 It works by inhibiting ribonucleotide reductase that interferes with DNA repair. Side effects from hydroxyurea include cytopenias, mucositis, diarrhea, peripheral neuropathy, and skin cancer. Also can use interferon-alpha, an immunotherapy, as an alternative. Interferon-alpha may be considered better for younger patients and those who want to get pregnant. For patients who are still symptomatic (ie: extreme pruritus and symptomatic splenomegaly) despite phlebotomy, aspirin, and myelosuppression therapy, ruxolitinib may be offered. Ruxolitinib is a JAK inhibitor, therefore it can only be used with patient who have the JAK2 mutation. It causes a decrease in platelets and help reduce the size of the spleen. It may cause severe anemia. For the pruritis, patients can try antihistamines, moisturizing cream, lowering water temperature while bathing, and patting skin dry rather than rubbing. It is also important to advise patients the importance of weight loss, diet, blood pressure control, and smoking cessation.5 Prognosis is good for patients with PCV. Median survival is 15 years. The major cause of death is venous and arterial thrombosis.4 PCV may transition to myelofibrosis, AML, or myelodysplastic syndrome which can also limit life expectancy.5

References
Hammond DB. Myeloproliferative Disorder, Bone Marrow Failure & the Myelodysplastic Syndrome. MCPHS University: Clinical Medicine III. October 2016.
Williams DA. PANCE Prep Pearls: a medical study and review guide for the PANCE, PANRE & medical examinations. 2nd ed. North Charleston, SC: CreateSpace Independent Publishing Platform; 2017.
Tefferi A. Clinical manifestations and diagnosis of polycythemia vera. UpToDate. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-polycythemia-vera?source=search_result&search=polycythemia vera&selectedTitle=1~104. Accessed September 27, 2017.
Papadakis MA, McPhee SJ, Rabow MW. 2015 Current Medical Diagnosis & Treatment. New York: McGraw-Hill Education/Medical; 2015.
Tefferi A. Clinical manifestations and diagnosis of polycythemia vera. UpToDate. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-polycythemia-vera?source=search_result&search=polycythemia vera&selectedTitle=1~104. Accessed September 27, 2017.
Tefferi A. Prognosis and treatment of polycythemia vera. UpToDate. https://www.uptodate.com/contents/prognosis-and-treatment-of-polycythemia-vera?source=search_result&search=polycythemia vera&selectedTitle=2~104. Accessed September 27, 2017.

Answer D.
The most common nerve injured during an anterior shoulder dislocation is the axillary nerve. The axillary nerve branches off the posterior cord of the brachial plexus and C5 and C6 nerve roots. The nerve then courses anterior to the subscapularis and exits out axilla posteriorly through the quadrangular space. The nerve then divides into the anterior, posterior, and articular branches. The anterior branch wraps around the surgical neck of the humerus beneath the deltoid muscle. The posterior branch innervates the teres minor and posterior deltoid muscle. The smaller articular branch enters the shoulder behind the subscaupularis muscle and innervates the shoulder joint itself. 1, 2
The mechanism of injury during a low velocity shoulder dislocation is traction on the axillary nerve wrapped around the humerus as the nerve is pulled forward beyond physiological limits. Symptomatic axillary nerve injury occurs in around 10% of patients after an anterior shoulder dislocation. However, electromyography studies have reported much higher rates of nerve injury with some studies approaching 55%. Symptoms of an axillary nerve injury include numbness and a dull achy pain over the lateral and posterior deltoid. A nerve injury should be suspected with any numbness to the lateral arm after a shoulder dislocation. In patients over 40 years old who sustain a dislocation, it is important to know that weak arm abduction is more likely related to a torn rotator cuff than a nerve injury. Axillary nerve injuries have a very good prognosis with full recovery expected between 12 and 45 weeks. Electromyography studies are generally not warranted as the prognosis is straight forward. Physical therapy should be started early after the injury to help regain motion and strength of the shoulder. 1
References
1. Visser CPJ, Coene LNJEM, Brand R, Tavy. The Incidence of Nerve Injury in Anterior Dislocation of the Shoulder and its Influence of Functional Recovery. JBJS (Br). Vol 81-B, No. 4. July 1999. 679-685.
2. Cetik O, Uslu M, Acar HI, Comert A, Tekdemir I, Cift H. Is there a safe area for the axillary nerve in the deltoid muscle? A cadaveric study. JBJS. 2006. 88-A (11). 2395-2399.

Answer A.
The anterior approach is an increasingly popular approach to total hip replacement. Recent advances in operating room tables and implant designs have made the anterior approach more surgeon friendly. The anterior approach is unique to the posterior approach in that the anterior approach advances through a true internervous and intermuscular plane. The posterior approach requires cutting through muscles to get to the joint capsule which can increase postoperative pain and slow recovery time as compared to the anterior approach. Patients who undergo the anterior approach tend to have shorter hospitals stays, less postoperative narcotic use, less postoperative hip precautions, and a quicker return to walking independently without a walker or crutches. Several studies have shown a lower postoperative dislocation rate with the anterior approach, although other studies have refuted this data with equal rates for both anterior and posterior.
One disadvantage of the anterior approach is the steep learning curve it takes for orthopedic surgeons to master the technique. This learning curve often leads to increased operating room times and operative blood loss. It generally takes 50-100 operative cases before orthopedic surgeons are proficient with the anterior approach. The anterior approach is often avoided in obese patients as a large pannus can fold over the anterior incision and increase infection potential.

References
1. Zawadsky MW, Paulus MC, Murray PJ, Johansen MA. Early Outcome Comparison Between the Direct Anterior Approach and the Mini-Incision Posterior Approach for Primary Total Hip Arthroplasty: 150 Consecutive Cases. June 2014Volume 29, Issue 6, Pages 1256–1260.
2. Higgins BT, Barlow DR, Heagerty NE, Lin TJ. Anterior vs. Posterior Approach for Total Hip Arthroplasty, a Systematic Review and Meta-analysis. The Journal of Arthroplasty. Volume 30, Issue 3, March 2015, Pages 419–434