The past 2 weeks have seen a lot of turnover in our internal medicine program, as the INTERNS graduate and prepare for their RESIDENT roles, and our third year RESIDENTS graduate!!!
Thank you, Dr Scharman, for an exhilarating case of a 69 year old male who presented with a petechial rash covering his bilateral lower extremities in the setting of a recent incidentaloma of a mediastinal mass and new pancytopenia found to have a thymoma.
What a case-- jam-packed with items ripe for differential making! Petechial rash! Pancytopenia! Mediastinal Mass! Since the first two findings are encountered rather frequently, let’s talk about mediastinal masses.
I whipped up a quick visual aid (see below) but you can also just remember “the cartoon network” or “that crazy Neukam” or “totally cool Nikes” or any mnemonic for the letters “TCN” to help you remember the differential for anterior, middle and posterior mediastinal masses.
As we learned from Dr Scharman, the paraneoplastic syndromes associated with thymic neoplasm are many and can present in myriad ways. This table from Up to Date outlines this nicely. (annotations made to highlight what our patient had).
So the lesson from today is, if you see a mediastinal mass and well… any other symptom!... think thymoma and it’s attendant paraneoplastic syndromes.
This week we had a fantastic discussion led by Dr. Padilla about a case of recurrent symptomatic ventricular tachycardia eventually diagnosed as Giant Cell Myocarditis.
Based on symptoms of palpitations, the differential is broad and the workup dependent on clinical features and level of concern. In this case, the key piece of data that we obtained was the ambulatory ECG, which revealed marked episodes of recurrent ventricular tachycardia, longer than 1 minute!
We reviewed the different possible types of ambulatory monitoring and their indications:
The differential of ventricular tachycardia is broad, but as part of the workup, we like to think through the following framework:
- Structural heart disease
- Past ischemia, scarring
- Infiltrative heart disease
- Inherited disorders
- Brugada Syndrome
- Arrhyhtmogenic right ventricular dysplasia
- Congenital Long QT
- Hypertrophic Cardiomyopathy
- Includes acute ischemia, metabolic/electrolyte causes, drugs/medication, hypoxia
In this case, the patient underwent cardiac MRI, which revealed late gadolinium enhancement and myocardial strain, characteristic of giant cell myocarditis
Briefly, giant cell myocarditis is a rare disorder attributed to T-cell mediated autoimmunity, characterized by biventricular enlargement, refractory ventricular arrhythmias with progression to cardiac shock.
Though there are characteristic findings on cardiac MRI, an endomyocardial biopsy is diagnostic.
Patients are treated with immunosuppression, with referral to cardiac transplantation if appropriate.
On Monday Dr Li shared an exciting case of infectious endocarditis secondary to aerococcus urinae complicated by severe aortic valve insufficiency, eventually requiring valve replacement. What is aerococcus urinae, you ask? Great question.
- Rare, gram +, alpha hemolytic, catalase negative cocci found in clusters or pairs
- The Aerococcus genus was discovered in 1953 (the same year as the double helix!)
- Historically underrecognized due to inability to culture
- Risk factors: elderly (age 75), male and female affects, underlying urologic issues (e.g. BPH, prostate CA), catheterization
- Presents as typical UTI, however UA negative for nitrites while positive esterase and protein
- Management Pearl: typically RESISTANT to TMP-SMX, ciprofloxacin, but SUSCEPTIBLE to penicillins, cephalosporins. Notably some reports of VARIABLE SUSCEPTIBILITY to vancomycin
o Some sources cite the “drug of choice” to be pencillins
- It is not uncommonly associated with the complications of bacteremia and endocarditis
With increasing ability to identify this organism we may begin to see it more in our clinical experiences and it will be prudent to keep our radars up, especially considering both its potential for complication and also resistance to some of our common empiric antibiotic choices.
In the event that aerococcus misbehaves, as was the case for Dr Li’s patient, let’s explore the indications for when to replace a left sided native valve endocarditis. Our experts at the AHA and IDSA list the following indications (level of evidence in parenthesis):
- Signs or symptoms of heart failure due to valve dysfunction (1B)
- IE complicated by annular abscess, heart block, or destructive perforating lesions (1B)
- IE due to fungal infection or highly resistant organisms (1B)
- Persistent infection (bacteremia or fever; >5-7 days) after appropriate antibiotic initiation, if other sources of fever or infection have been ruled out (1B)
- Recurrent emboli or persistent/growing vegetations despite appropriate antimicrobial therapy (2a,B)
- Severe regurgitation and mobile vegetation >10mm (2a,B)
- Mobile vegetation >10mm (2b,C)
If you're visual like I am, please find below this ridiculous picture that captures these indications. Consider it a work in progress... I've been trying to spend more time on my creative expression.
Christensen and Nielsen. Aerococcus urinae. Antimicrobe http://www.antimicrobe.org/b75.asp
Higgins and Garg. Aerococcus urinae: An Emerging Cause of Urinary Tract Infection in Older Adults with Multimorbidity and Urologic Cancer. Urol Case Rep. 2017 Jul; 13: 24–25. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5393163/
Zhang et al Aerococcus urinae in Urinary Tract Infections. J. Clin. Microbiol. April 2000 vol. 38 no. 4 1703-1705 http://jcm.asm.org/content/38/4/1703.full
Baddour et al. Infective Endocarditis in Adults: Diagnosis, Antimicrobial Therapy, and Management of Compliations. AHA Scientific Statement Endorsed by Infectious Disease Society of America. Circulation. Oct 13 2015 http://circ.ahajournals.org/content/circulationaha/early/2015/09/15/CIR.0000000000000296.full.pdf
Thanks to Dr. Joel Horton for providing a great discussion last week on toxindromes in a patient with serotonin syndrome (SS). Below are some learning points from today’s case, in addition to reference to two good review articles on serotonin syndrome and toxindromes respectively. A few highlights from an NEJM review on Serotonin syndrome.
- Serotonin syndrome is thought to be caused by excess agonism of 5-HT2A Receptors
- 60% of patients present within 6 hours of change or administration of medication leading to SS.
- Clinical findings with a statistically significant association with SS include: hyperreflexia, inducible clonus, myoclonus, ocular clonus, spontaneous clonus, peripheral hypertonicity, and shivering.
- Hyperreflexia, clonus, and rigidity are more prominent in the lower extremities in SS.
- Serotonin syndrome must be distinguished from other toxindromes that present similarly: neuroleptic malignant syndrome, anticholinergic poisoning, and malignant hyperthermia
Most cases will resolve after 24 hours with initiation of treatment (see below) and discontinuation of the offending agent.
a. Removal of the offending agent
b. Supportive care (fluids, vasopressors- norepi, epi, phenylephrine preferred)
c. Control agitation (Benzodiazepines)
d. Administration of 5-HT2A Antagonists (cyproheptadine: 12- 32 mg over 24 hours will bind 85-95% of serotonin receptors)
e. Control of autonomic instability
f. Control of hyperthermia (if temp > 41°C): Paralysis with non-depolarizing agent and endotracheal intubation. No role for antipyretics as increased temp is due to muscular activity.
Want to learn more? See the following links below:
- https://www.nejm.org/doi/full/10.1056/NEJMra041867 (NEJM review on Serotonin syndrome)
- Holstege, Christopher P., and Heather A. Borek. "Toxidromes." Critical care clinics 28.4 (2012): 479-498.
Distinguished professor at Tufts University & former editor in chief of the NEJM
Dr. Kassirer (@jpkassirer) paid a visit as part of the annual Dr. Noble Wiley Jones Lectureship. He gave an excellent grand rounds, entitled "Lessons from my 60 year Quirky Career", full of excellent advice for our housestaff and faculty "The young and the old" (in his words).
Residents were then treated to a lunchtime Q&A panel between him and our very own Dr. Vinay Prasad (@VinayPrasadMD), where they touched on a number of topics, including gun control, challenging dogma, conflicts of interest, the evolution of diagnostic reasoning, and how to use your role as a physician to be an advocate.
Finally, he participated in Diagnostic Rounds led by Dr. Peter Sullivan, before setting off to visit our beautiful Oregon coast!
From Dr. Max Gordon, we learned about a fascinating case of hemoptysis arising from Williams-Campbell Syndrome first diagnosed in an older patient. In this condition, a cartilaginous deficiency in the 4th to 6th order bronchi prevent adequate clearance of respiratory secretions, resulting in recurrent infections and thus bronchiectasis.
Bronchiectasis arising from this chronic progressive process is diffuse, cystic, and has a central predominance on imaging, characterized by air-fluid levels.
Below is some representative imaging similar to that of the case discussed:
We discussed some initial considerations when encountering a new case of hemoptysis:
Classification of Massive vs. Submassive: generally considered “massive” if >100cc/24 hrs
Location of bleed and potential etiologies:
- Lung parenchyma (Abscess, pneumonia, tuberculosis, Goodpasture, granulomatosis with polyangiitis)
- Subglottic airways (Bronchitis, bronchiectasis, tumor)
- Pulmonary vasculature (AVM, pulmonary embolism, mitral stenosis, left sided heart failure)
- Mimickers (GI tract, Nasopharynx, Serratia marcescens pneumonia (due to staining of pigmented bacteria))
Mechanism: Usually from bronchial arterial source (ie non-gas exchange vasculature).
- Either compromised pulmonary circulation in anastamotic vessels leading to rupture
- Chronic inflammatory or neoplastic related- angiogenic growth factors leading to proliferation of fragile new vessels which are prone to rupture
Thanks again to Dr. Gordon for this fascinating case and imaging.
Additional information above compiled from dynamed
This afternoon Dr Feng shared the tale of a middle-aged man who presented with acute on chronic dyspnea felt to be secondary to acute decompensated heart failure. Seems easy, right? Diuresis, diuresis, diuresis… but, what if that’s not enough? What if you come up against…
… diuretic resistance!
Thanks to our very own, Dr David Ellison (nephrology), we have guidance. If you’ll allow me, I’d like to share some highlights from his 2017 NEJM review article.
How to Recognize Diuretic Resistance In the Labs:
- Low urine sodium despite maximum recommended dose of diuretic
- Lack evidence of “contraction” on your chemistry panel (increasing creatinine or bicarbonate)
The 3 D’s of Diuretic Resistance*:
As eloquently explained by Dr Ellison, “A dose of a loop diuretic increases urinary excretion of sodium chloride for several hours, but this is then followed by a period of very low sodium excretion, often termed “post-diuretic sodium retention.” To induce negative sodium chloride balance, the excretion of sodium chloride during 24 hours must exceed its intake. When dietary sodium chloride intake is high, post-diuretic sodium retention will offset the initial natriuresis, especially if the dosing interval is long. In contrast, low intake of sodium chloride permits urinary sodium excretion to exceed intake. The difference in these effects on extracellular fluid volume underscores the importance of dietary intake of sodium chloride, the drug half-life, and the dosing interval, especially in patients with chronic heart failure.”\
In abbreviated terms, when thinking about how to avoid diuretic resistance and improve natriuresis, consider:
- Dietary Sodium
- Drug half-life
- Dosing Interval
*Note: This ridiculous alliteration is all mine, do not hold it against Dr Ellison.
Management of Acute Decompensated Heart Failure
With this background of diuretic resistance in mind, the question remains for how we practically apply this it our patients. You should find the following tips helpful:
- Initial Diuretic Dose: IV (ggt schedule as below) or bolus (2.5x previous oral dose, BID)
- Subsequent Doses: increase daily to reach goal UOP
- Goal UOP: 3-5 liters urine daily until clinical euvolemia is reached
- Backup Plan: if UOP remains < 3 liters per day, add sequential blockade
- Sequential Blockade Options: metolazone || HCTZ 50 mg BID || chlorthalidone 50 mg QD
Citation: Ellison, D and Felker, G. Diuretic Treatment in Heart Failure https://www.nejm.org/doi/full/10.1056/nejmra1703100 Nov 16 2017