UMEM Educational Pearls

-    The most common disease producing enzymopathy in humans

-    Affects 400 million people worldwide

-    Highest prevalence is among persons of African, Asian, and Mediterranean descent

-    Patients can be asymptomatic but may present with symptoms of acute hemolytic anemia, which may be precipitated by certain medications (Oxidative medications) or foods (some types of beans)

-    Avoid oxidative drugs (consult your PharmD when your patient has G6PDd)

-    Diagnosis: Measure the actual enzyme activity of G6PD rather than the amount of the enzyme. A more practical test is the presence of Indirect hyperbilirubinemia, but it is non specific

-    Treatment consists of oxygen and bed rest in minor cases. However, severe cases may require PRBC transfusion

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Propofol is generally a well-tolerated sedative / amnestic but occasionally it can lead to the propofol infusion syndrome (PRIS); a metabolic disorder causing end-organ dysfunction.

Suspect PRIS in patients with increasing lactate levels, worsening metabolic acidosis, worsening renal function, increased triglyceride levels, or creatinine kinase levels. End-organ effects include:

• Myocardial dysfunction / Arrhythmias
• Rhabdomyolysis
• Acute renal failure

The true incidence of PRIS is unknown, however, certain risk factors have been identified:

• Doses >4-5mg/kg/hour
• <18 years of age
• Critically-ill patients; especially receiving vasopressors or steroids
• History of mitochondrial disorders
• Infusions >48 hours

Prevent PRIS by using adequate analgesia (with morphine or fentanyl) post-intubation, which may reduce the overall dosage of propofol ultimately reducing the risk.

If PRIS develops, stop propofol and provide supportive care; IV fluids, ensuring good urine output, adequate oxygenation, dialysis (if indicated), vasopressor and inotropic support.

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Question

A 25 year-old female presents complaining of a "net-like" rash bilaterally on her medial thighs. She denies any pain but states that the rash looks “pretty scary” What's the diagnosis?

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-Common life-threatening cardiovascular effects of cocaine intoxication include tachydysrhythmias, ventricular fibrillation, myocardial ischemia, and infarction.

-Emergency management of acute cocaine intoxication relies mainly on supportive and symptomatic treatment, w/liberal use of gamma-aminobutyric acid receptor agonists such as benzodiazepines.

-Intravenous lipid emulsion (ILE) therapy has been used successfully to treat cardiac toxicity associated with a variety of lipid-soluble drugs, such as local anesthetics, calcium/beta-blockers, tricyclic anti-depressants, and cocaine. 

-The current hypothesis, called the “lipid sink” hypothesis, suggest that ILE infusion creates an expanded lipid phase in the plasma that absorbs the circulating lipophilic toxin and decreases the amount of free unbound toxin available to bind to the myocardium.

-When life-threatening cardiac arrhythmias (e.g. wide-complex tachycardia/prolonged QT) are not amenable to standard therapy (e.g. sodium bicarbonate/magnesium) consider ILE as a potential option to the current algorithm. 

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Estimated 3.8 million sport-related concussions per year (likely significantly higher due to underreporting)

Most patients recover within a 7-10 day period

** Children and teenagers require more time than college and professional athletes

This "accepted" time for recovery is not scientifically established and there is a large degree of variability based on multiple factors including age (as above), sex & history of prior concussions

Approximately 10% of athletes have persistent signs and symptoms beyond 2 weeks (which may represent a prolonged concussion or the development of post-concussion syndrome)

During this time the patient should have complete rest from all athletic activities, close follow-up with PCP and be educated re concussions.

If practical, "cognitive rest" should also be prescribed. This is one of the most frequently neglected aspects of post-concussion care and will be discussed in a future pearl.

• Found as adulterant in street drugs

• Used in bodybuilding and for weight loss

• Long acting beta-2 agonist

• Has specific anabolic activity and increases lipolysis

• Toxicity presents with tachycardia, palpitations, tremor, and myocardial ischemia

Just a quick clarification to last week's melioidosis pearl:

An astute reader noted the typo:  "The patient should also be covered for melioidosis, and infection caused by Burkholderia pseudomallei."  The sentence should read "...meliodosis, an infection caused by Burkholderia pseudomallei."

Just to clarify, melioidosis is caused by the bacteria Burkholderia pseudomallei.

Many apologies for any confusion this might have caused.

Thanks for reading!

Andi Tenner, MD, MPH

Background Information:

Active tuberculosis (TB) develops in 5-10% of individuals who become infected with M. tuberculosis, typically after a latency period of 6-18 months (but sometimes decades later).  Compliance with the 9 month self-supervised isoniazid (INH) regimen has been porr with completion rates <60%.  Until recently, daily rifampin for 4-6 months has been the only alternative when the bacterium is resistant or INH cannot be used.

Pertinent Study Design and Conclusions:

• Another rifamycin class antibiotic, Rifapentine (RPT) is approved for MDR-TB but had not been approved for latent TB treatment.
• Recent RCTs show 12 weekly doses of INH-RPT administered as directly observed therapy (DOT) are efficacious in preventing active disease and are better tolerated.
• CDC now recommends the 12 week INH-RPT DOT regimen as an equal alternative to 9 months of self supervised daily INH in patients aged >12 years who have a high likelihood of developing active TB.

Bottom LIne:

A substantially shorter course of therapy with INH-RPT is now the recommended treatment for latent TB.

University of Maryland Section of Global Emergency Health

Author: Emilie J. B. Calvello, MD, MPH

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Needle Decompression - Are we Teaching the Right Location?

• Tension pneumothorax frequently results in circulatory collapse and may lead to cardiopulmonary arrest.
• In the event that tube thoracostomy cannot be immediately performed, traditional teaching is to perform needle decompression in the second intercostal space, mid-clavicular line using a 5-cm angiocath needle.
• Recent literature, however, has challenged the traditional location for needle decompression.  In fact, researchers found:
  • Needles placed in the second intercostal space often failed to enter the chest cavity and relieve tension physiology.
  • Needles placed in the fifth intercostal space in the anterior axillary line were more likely to enter the chest cavity with a lower failure rate.
• Take Home Point: It may be time to reconsider the optimal position for needle decompression of tension pneumothorax.

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Question

40 year-old male presents with fever, chills, & cough. What’s the diagnosis and the MOST likely cause? 

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• Early CPR performed by laypersons can double the chances of survival in out-of-hospital cardiac arrest (OHCA)
• A retrospective cohort that combined 2 RCT compared the survival effects of dispatcher CPR instruction consisting of chest compression alone or chest compression with rescue breathing
• There was a lower risk of death after adjustment for confounders (adjusted hazard ratio 0.91, 95% confidence interval 0.83-0.99, p=0.02)
• Findings strongly support a long-term mortality benefit of dispatcher CPR instruction strategy consisting of chest compression alone rather than chest compression plus rescue breathing

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Intraosseus (IO) access has become quite popular in critically ill patients requiring immediate resuscitation. In a patient responsive to pain, however, pain and discomfort is associated with the force of high-volume infusion through the established line.

• Before flushing the line, consider administering preservative-free 2% lidocaine (without epinephrine) for patients responsive to pain prior to flush.

• The suggested dose is 20-40 mg (1-2 mL) of the 2% lidocaine, followed by the 10 mL saline flush.

If preservative-free 2% lidocaine is not stocked in your ED, now is the time to consider adding it.

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This winter season has brought a rise in influenza and RSV activity in Maryland and in many parts of the country. It is also important to remember other potentially lethal infections that are prevalent in the winter and early spring months, such as Neisseria meningitidis. In fact, a recent study2 showed a potential increase in meningococcal disease when influenza and RSV activity is high.

What:
Encapsulated, gram-negative diplococcus
Where:
Found in nasopharyngeal secretions, carrier rates 2-30% in normal populations
Who:
Age of incidence has 2 peaks: children < 2 years old, teens 15-19 years old
Young adults who live in shared housing, such as college dorms and military recruits

Clinical Presentation:
Early non-specific symptoms of URI, fever, malaise, myalgias
Meningitis: non-specific prodrome + headache, stiff neck (not found in younger children who often present atypically with irritability and/or vomiting)
Meningococcemia: above symptoms + hypotension + petechial rash (>60% of patients)

Treatment:
Early (!) antibiotics: 3rd generation cephalosporins (<3mo: cefotaxime; older infants, children, and teens: ceftriaxone); PCN G is antibiotic of choice for susceptible isolates
Early and aggressive management of shock

Prevention:
Tetravalent vaccine, MCV4 (Menactra, Menveo), available for serogroups A, C, Y and W-135 is given routinely at age 11-12 years old with an additional booster at 16-17 years old. MCV4 does not protect against serogroup B which accounts for 30% of infections.
 

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There have been many attempts to reduce the incidence of contrast-induced nephropathy. Mechanism usually centers around antioxidant properties or free radical scavengers that prevent the acute kidney injury that may result after intravenous contrast. IV Fluid hydration, sodium bicarbonate and acetycysteine have been studied with only some evidence. There is also some controversial data that is beginning to surface regarding the use of atorvastatin with a recent article in Circulation 2012 that showed high dose atorvastatin (80mg) 24 hrs prior to angiography prevented contrast-induced acute kidney injury in patients with mild to medium risk. Link to article has been provided:

http://circ.ahajournals.org/content/126/25/3008

Case Presentation:

A 43 year old diabetic woman presents with dyspnea and a dry cough. Her vital signs are:  BP 84/42, HR 135 RR 37 T 38.5.  Lobar consolidation is seen on chest xray.  She decompensates and is intubated, a central line is placed, and IV fluids are started.  Her husband reports that they had just returned from  a vacation in Thailand one week earlier.

Clinical Question:

Does the recent travel change your choice of empiric antibiotics?

Answer:

The patient should also be covered for melioidosis, and infection caused by Burkholderia pseudomallei.

• Infection can occur via direct contact with, inhalation of, or ingestion of the bacteria.
• B. pseudomallei is highly endemic in Thailand and Northern Australia, but melioidosis has been contracted in the Americas and other parts of Asia and Australia. (True epidemiology is unknown due to difficulties in culturing the bacteria)
• Clinical presentation most frequently involves pulmonary infection, abscess formation, or bacteremia.
• Labs that don't have experience with this bacteria have difficulty culturing it and it is often misidentified.
• Treatment is 10-14 days of ceftazidime or a carbapenem.
• After recovery, the patient requires TMP-SMX for 3-6 months for bacterial eradication. 

Bottom Line:

Patients presenting with severe infections and recent travel to an endemic area should receive emperic antibiotics with ceftazidime or a carbapenem until another source is identified. 

University of Maryland Section of Global Emergency Health

Author: Jenny Reifel Saltzberg, MD, MPH

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The updated Surviving Sepsis Guidelines have been released (click here) and here are some recommendations as they pertain to hemodynamic management (grades of recommendations in parenthesis).

Fluid therapy

• An initial fluid bolus of at least 30 mL/kg is recommended; crystalloids should be the initial fluids (1B).
• Consider albumin when “substantial” amounts of crystalloid have been given (2C).
• Use of hydroxyethyl starch is not recommended (1B)

Vasopressors (targeting MAP of at least 65 mmHg)

• Norepinephrine (NE) is the vasopressor of choice (1B)
• Epinephrine (EPI) if an additional agent is required; can be added to or substituted for NE (2B)
• Vasopressin (0.03 units/minute) can be added to NE; it should not be titrated or used as a single agent (ungraded).
• In selected patients (e.g., bradycardia or low-risk of tachyarrhythmia), dopamine may be considered (2C). Low-dose dopamine (for renal protection) should not be used (1A).
• Phenylephrine (PE) is not recommended, except if (1C):
  • Serious NE associated arrhythmias
  • Cardiac output can be measured and is increased with low MAP (PE can reduce cardiac output)
  • Other therapies cannot achieve the target MAP

Corticosteroids

• Use if fluids and vasopressors cannot restore adequate perfusion
• Total daily dose of 200 mg (2C) administered by continuous infusion (2D)
• ACTH stimulation test is not recommended (2B)
• Tapering hydrocortisone when vasopressors have been discontinued (2D)

Inotropic Therapy

• Administer dobutamine if it is believed that cardiac filling pressures are elevated, cardiac output is low, or persistent signs of hypoperfusion despite other therapies (1C)

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Question

40 year-old female drove into a ditch. Right sided chest pain and stable vitals. Here's the CT but what do you think the initial CXR showed (Hint: it's a trick)?

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• Many infants w/cyanotic heart disease only survive w/early surgical intervention
• The most rapid & effective first-line therapy for stabilization of the crashing neonate is IV prostaglandin E1 (PGE1)
• PGE1 serves to reopen the ductus arteriosus allowing partially desaturated systemic arterial blood to enter the pulmonary artery and be oxygenated
• The widespread use of this agent has profoundly decreased morbidity & mortality 
• The initial dose of PGE1 is 0.1 mg/kg/min
• ADR for PGE1 include: apnea, hypotension, edema, and low grade fever

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Hematoma Block

Provides good aesthesia for reduction of fractures. Onset in approximately 5 minutes

Benefits:  No need for NPO, simple and easy to perform & can be done without additional personnel (unlike w/ procedural sedation)

Contraindications: Open fractures, dirty or infected overlying skin

1) Identify fracture site with x-ray and palpation

2) Clean skin w/ Betadine

3) Insert needle into the hematoma. * Confirm placement by aspirating blood *

4)  Inject anesthetic (lidocaine 1 or 2%) into the fracture cavity and adjacent periosteum

http://www.youtube.com/watch?v=tjnsdjfwMmY

Cyclophosphamide-induced hemorrhagic cystitis is a well known to oncologists. This unique complication of this chemotherapeutic drug has a defined mechanism and could be seen in your Emergency Department.

- Hemorrhagic cystitis occurs in 46% of patients that receive cyclophosphamide

- Can occur even months after administration

- 5% can actually die from the hemorrhage

- Treatment: Bladder irrigation, hydration, supportive. Oral adminsitration of MESNA (2mercaptoethan sulfonate) and bladder irrigation with prostaglandins and even methylene blue have been attempted.