Cardiac amyloidosis can present along a spectrum from asymptomatic to severe CHF w/conduction abnormalities

ECG with low voltage + echocardiogram with thickened myocardium should heighten suspicion

Definitive Dx. is myocardial biopsy identifying the infiltrative lesion (MRI w/gad is also supportive)

AL (light chain) amyloidosis is an acquired disease from improperly functioning plasma cells

¨ Rapidly progressive and life threatening

¨ Tx. w/chemotherapeutic agents (+/- BMT)

Transthyretin-related (TTR) amyloidosis is produced by the liver (2 types)

Familial transthyretin-related amyloidosis (ATTR)

Senile systemic amyloidosis (SSA)

¨ Both are slowly progressive

¨ Tx liver transplant (ATTR) and supportive care (SSA)

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Many who work in urban EDs and have a patient population that has a high rate of methadone use have probably wondered - why don't I see many STEMIs in the ED?

One study has actually attempted to answer the question - is methadone cardioprotective? Comparing 98 decedents with known long-term methadone exposure and compared autopsy coronary artery findings to match controls without, there was significant decrease in incidence of severe CAD:

5/98 Methadone Patients post-mortem had severe CAD vs 16/97 match controls

Better than a baby ASA, who knew?

[I thank Dr. Hoffman for citing this article to me]

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Delirium in the Critically Ill

• Delirium has been shown to be an independent predictor of mortality and can occur in up to 75% of critically ill patients.
• Whether preventing or treating delirium in the critically ill patient, consider the following:
  • Minimize the use of anticholinergic medications (i.e. diphenhydramine, chlorpromazine)
  • Ensure pain is adequately controlled (avoid meperidine and tramadol)
  • Be careful with sedative medications; consider bolus dosing and daily interruption of continuous infusions
• Additional measures to treat delirious patients include reducing sensory deprivation, promoting normal sleep-wake cycles, early physical rehabilitation, and treating psychosis.

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Question

35 year-old male unrestrained driver following motor vehicle crash presents with blunt chest injury. There are multiple injuries on CXR (can you find them all?), but what's up with his right lung?

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Etiological agent is the parasite Trypanosoma cruzi

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Congratulations to today's Baltimore marathoners and the medical race staff

In honor of them:

Marathons are becoming increasingly popular with participation rising from an estimated 143,000 US marathon finishers in 1980 to a record high of 507,000 during 2010.

Most victims of exercise-related sudden cardiac arrest have NO premonitory symptoms

Autopsy reports show that

1) 65 - 70% of all adult sudden cardiac deaths are attributable to coronary artery disease.

2) 10% due to other structural heart diseases (HOCM, congenital artery abnormalities)

3) 5 - 10% due to primary cardiac conduction disorders (prolonged QT, ion channel disorders)

4) Remainder are due to non cardiac etiologies

Overall risk of sudden cardiac arrest is approximately from 1 in 57,000 and the risk of sudden cardiac death is approximately 1 in 171,000. Mortality without intervention after sudden cardiac arrest  is greater than 95%. The majority occur in middle to late aged males.

V fib/V tach are the most common arrhythmias leading to sudden cardiac arrest. Most events occur in the last 4 miles of the racecourse.

Survival decreases by 7 - 10%  with each minute of delayed defibrillation. Defibrillation within 3 minutes can produce survival rates as high as 67 - 74%. After 8 minutes, there is a dramatic decrease in survival. Prompt CPR increases survival from 2.5% to greater than 8%.

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• approximately 1% of children in DKA have some degree of cerebral edema, and up to 25% of them may die
• known risk factors include the following:

1. younger children (especially <5 years)
2. new onset or newly diagnosed
3. increased BUN at presentation
4. severity of acidosis at presentation
5. bicarbonate therapy use
6. failure of sodium to improve following therapy

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Activated charcoal is most effective if given within 1 hour of overdose.

Prehospital administration of charcoal can be challenging, but may save significant time compared to waiting until arrival to the ED. The patient has to be transported by EMS, registered, seen by a provider, order for charocal placed...

Two studies evaluated the time difference between prehospital and hospital administration of GI decontamination.

• Study 1 found median time to activated charcoal in the ED was 82 minutes.
• Study 2 found mean time to activated charcoal by EMS was 5 minutes, compared to 51 if held until arrival to ED.

Bottom line: Don't underestimate the amount of time that goes by before you evaluate non-crashing patients upon arrival to the ED. If the story supports an overdose and the patient doesn't have contraindications for receiving charcoal, recommend it be given in the prehospital setting for greatest potential benefit.

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Question

70 year-old male recently treated for community-acquired pneumonia presents with bloody diarrhea, fever, and severe abdominal pain. Abdominal Xray is shown below. Diagnosis?  

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Question

26 year-old male from Indonesia presents with severe abdominal pain and weight loss for the past two months. He also states he found this "worm" in the toilet (see below) after a bowel movement. What is the medical treatment for this condition? 

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Autoantibody-associated congenital heart block (CHB), also know as neonatal lupus, is responsible for the majority (~60-90%) of CHB

This is secondary to maternal antibodies that cross the placenta and may disappear postnatal

Neonatal lupus can result in diffuse myocardial disease both with and without conduction disturbances, structural defects, and electrophysiologic anomalies

Overall mortality is up to 30%, with 15% mortality before 3 months of age

More than 65% of surviving newborns require pacemakers

Maternal screening and fetal echocardiography has allowed routine prenatal diagnosis 

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Patients frequently report having a sulfa allergy. In most cases, the allergic reaction was secondary to a sulfonamide antimicrobial agent, such as sulfamethoxazole-trimethoprim.

The question is: Can I use furosemide (or other non-antimicrobial agents containing a sulfa component)?

• There is minimal evidence of cross-reactivity between sulfonamide antimicrobials and non-antimicrobials.

• Despite this, the U.S. FDA-approved product information for many non-antimicrobial sulfonamide drugs contains warnings concerning possible cross-reactions.

Bottom line: If a patient had a true IgE-mediated anaphylatic reaction to a sulfonamide antimicrobial, it may be best to avoid other sulfa-related medications (use ethacrynic acid if a loop diuretic is needed). Otherwise, the available literature does not support cross-reactivity between sulfonamide antimicrobials and non-antimicrobials.

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We often ask our pediatric patients if there vaccines are up to date, but what does this mean?

Hepatitis B: birth, 2 and 6 months

Diphtheria/Tetanus and Acellular Pertussis: 2, 4 and 6 months

Pneumococcal vaccine: 2, 4 and 6 months

Haemophilus influenzae B : 2, 4 and 6 months

Polio: 2, 4 and 6 months

Rotavirus: 2 and 4 months or 2, 4 and 6 months depending on the brand. 

Influenza: 6 months and older

Children less than 8 years old should receive 2 doses of flu vaccine at least 4 weeks apart during the first flu season that they are immunized.  Children older than 2 years are eligible for the nasal vaccine if they do not have asthma, wheezing in the past 12 months or other medical conditions that predispose them to flu complications.

To see the full vaccine schedule including exact time frames between doses and catch up schedules, see: http://www.cdc.gov/vaccines/schedules/downloads/child/0-6yrs-schedule-pr.pdf

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Ever have that alcholic who requires lorazapam doses that start to approach 10mg? 20mg? or even higher. The next step is usually a lorazepam infusion and then send them to the ICU. In the ICU,  the patient develops an unexplained anion gap lactic acidosis.

Check a Lactate - lorazepam has 80% propylene glycol (PG). PG is metabolized to lactate which can accumulate when a lorazepam infusion at an elevated dose is running constantly.  Hypotension, bradycardia and even other EKG changes have been reported. Simply discontinue the infusion and assess your acid-base status. 

Other IV meds that contain PG:

lorazepam - 80% PG

Phenytoin - 40% PG

Phenobarbital - 67.8%

Diazepam - 40% PG

• Is associated with chronic use of marijuana

• Patients typically present with severe, recurrent nausea, vomiting, and abdominal pain, usually in the morning

• Temporary relief of symptoms is achieved by taking hot showers or baths

• Diagnostic work up is negative

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Thrombotic Thrombocytopenic Purpura (TTP)

• TTP is a true hematologic emergency.  As a result of delays in diagnosis and initiation of treatment, mortality remains around 20%.
• Often, patients present with nonspecific symptoms that include weakness, anorexia, nausea, vomiting, and diarrhea.
• Recall that the textbook pentad is rarely present upon presentation.  In fact, renal failure and neurologic deficits are late findings.
• Plasma exchange remains the treatment of choice for critically ill ED patients with TTP.
• If plasma exchange is not immediately available, consider FFP (15-30 ml/kg) and methylprednisolone (10 mg/kg).

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Do you place central-lines?

Do you suture your central-lines into place?

Do you ever get worried that you are going to stick yourself with that needle?

If you answered yes to any of these questions, then maybe this pearl is for you; click here

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Aortic valve (AV) stenosis associated with gastrointestinal angiodysplasia

Proteolysis of Von Willebrand (type 2A) as it passes through the stenotic valve is one culprit of bleeding

Hemostatic abnormalities e.g. GI bleed are often corrected after AV replacement

Valve replacement is only recommended for cardiac symptoms

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Fight Bites

• Any abrasion or laceration over the knuckles should be presumed to be a fight bite.  Patients will often lie about the circumstances of the laceration.
• Radiographs should be obtained on all of these patients to exclude
  • Retained foreign bodies (e.g., tooth fragments)
  • Fracture of the metacarpal head
• Place patient on amoxicillin/clavulanic acid or clindamycin to cover mouth flora
• Irrigate wound well, and explore through the fingers full range of motion to exclude joint or tendon injury.
• Refrain from suturing the wound, as this will increase the risk of infection.
• Splint in position of function and have them follow up in 2 days.

The incidence of pediatric syncope is common with 15%-25% of children and adolescents experiencing at least one episode of syncope before adulthood. Incidence peaks between the ages of 15 and 19 years for both sexes.

Although most causes of pediatric syncope are benign, an appropriate evaluation must be performed to exclude rare life-threatening disorders. In contrast to adults, vasodepressor syncope (also known as vasovagal) is the most frequent cause of pediatric syncope (61%–80%).  Cardiac disorders only represent 2% to 6% of pediatric cases but account for 85% of sudden death in children and adolescent athletes.  17% of young athletes with sudden death have a history of syncope.

Key features on history and physical examination for identifying high-risk patients include exercise-related symptoms, a family history of sudden death, a history of cardiac disease, an abnormal cardiac examination, or an abnormal ECG.