my mumps,......my mumps my mumps my mumps...check it out.

Please pardon the horrible Black Eyed Peas reference.

A great "Advanced Morning Report" today. We discussed a case that was likely Mumps.

More details can be found here.

HoPingKong-isms

(hyperthermia can cause rhabdomyolysis)

1. "....What are you lying on? This is a giant question...a maximus question....are you crushed by this?"..... this was in the context of patients found either unconscious or unable to get up for prolonged periods of time. He is referring to rhabdomyolysis. The 'maximus' comment must refer to the gluteus maximus, and the 'are you crushed?'....for sure that is for crush injuries. We discussed rhabdomyolysis here.

2. "...Let's think about cardiac causes of syncope...hey....Adam is here today...". Yep, this is a Stokes-Adams Attack, named after William Stokes and Robert Adams - two Irish physicians.

3. "...Sometimes you have overt alcoholics, then there are social drinkers...I'm thinking of the smallest room in the house..." So what's the smallest room in the house? The closet. He was referring to "closet alcoholics". We discussed alcohol related issues here, seizure disorders here, and toxic alcohol ingestions here.

4. "....Think about Captain Morgan!...". This was said in the context of him shaking his arms and head - convulsing. "Yes...this is a 'Rum Fit'". Also known as an alcohol withdrawal seizure. Remember your CAGE questionnaire to screen for alcohol-related problems:

• do you feel the need to Cut back your drinking?
• do you ever feel Annoyed at people critiquing your drinking?
• do you ever feel Guilty about your alcohol consumption?
• do you ever have an Eye opener? (Alcohol first thing in the morning)

• 1 point is given every time your patient answers "yes" to a question. ....then you just add up the points.
  

• Most patients with alcohol dependence will have a score of 2 or greater. About 80% of patients without alcoholism will score 0 points.
  

Here is a paper from JAMA written by local talent on the utility of alcohol screening tools.

There is the artist formerly known as Prince...

....and the pneumonia formerly known as Pneumocystis Carinii Pneumonia. Many people still refer to it as PCP , but you will also hear it called Pneumocystis Jirovecii Pneumonia (PJP). Either is fine. It is a common respiratory opportunistic infection in HIV+ individuals. Those with a CD4 count less than 200 are at the greatest risk. It is a protozoa, and is found ubiquitously in soil - we are all exposed, but this organism poses few problems to healthy immune systems.

The classic clinical presentation is dyspnea with subacute onset, and a dry cough. Patients may have a low-grade fever, tachycardia, and tachypnea. The chest exam is variable - you may hear crackles...you may have a normal exam (in up to 50% of cases). The Chest X-ray often reveals bilateral interstitial infiltrates, but virtually any abnormality may be seen.

Remember, we can make the diagnosis roughly 90% of the time with history and physical exam alone. Still, it is nice to confirm your diagnosis by isolating an organism. Methenamine silver or Immunofluorescent stains on induced sputum (or bronchoalveolar lavage) has a high sensitivity and specificity.

Treatment: TMP-SMX in high doses. This has some interesting complications associated with it (see below). If patients are allergic to sulfa drugs or have complications, other agents can be used, such as TMP-Dapsone, or Atovaquone.

Steroids? Yep. If the PaO2 is less than 70, this is very helpful. Of note, this was a major breakthrough in medicine and was discovered by local talent here in Toronto.

What else? Watch these patients closely. There is often a profound inflammatory reaction to the dying organisms, and patients often get worse on day 2-ish of treatment. That is why the steroids are added in severe disease.

Links:

• Treatment guidelines for HIV can be found here.
• Treatment guidelines for Opportunistic infections are here.
• A great review of PCP from NEJM here.
• Local talent publishing on an interesting complication from high dose trimethoprim-sulfamethoxazole.
  

Below: Methenamine silver (top) and Immunofluorescent (bottom) stains on induced sputum showing PCP.

Would you like fries with that?

Today we discussed Non-Alcoholic Fatty Liver Disease.

We have previously discussed the etiology of liver diseases here, and some complications of liver disease like ascites here, and spontaneous bacterial peritonitis here.

I don't know about you, but I'm dying for a burger right now.

Check out this review from CMAJ on Non-Alcoholic Fatty Liver Disease.

No Country for Old ________........

(Does this man have acromegaly?)











Multiple Endocrine Neoplasia (MEN) syndromes can often be a bit confusing. It should be considered in those with....uhhhh....endocrine neoplasia - at multiple sites.

MEN 1: An autosomal dominant disease with mutations in the MENIN gene.
Remember "the 3 P's":

• Pituitary tumors: particularly the anterior pituitary.
• Pancreas: islet cell tumors like Insulinomas, Glucagonomas, and VIPomas.
• Parathyroid hyperplasia, usually in all glands.

MEN 2: Also an autosomal dominant disease. The mutation is in the RET proto-onco gene.

• MEN 2A: aka Sipple Syndrome
  
• Medullary thyroid cancer; typically bilateral. In non-MEN disease this is mostly unilateral.
  
• Pheochromocytoma in about 50%. Also can be bilateral.
• Parathyroid hyperplasia

• Men 2B
• Medullary thyroid cancer: as above.
• Parathyroid hyperplasia: as above.
• Marfanoid habitus: high arched palate, pectus excivatum.
  

HoPingKong-isms

(Modigliani's Paul Guillaume Novo Pillota. In Paris' Musee d'Orangerie)

1. "....if you are anemic with your chronic renal failure, are you drinking the Tour de France drink?..." Okay...this one isn't too tough. He is referring to erythropoietin, a glycoprotein hormone produced by interstitial fibroblasts in the renal cortex, responsible for stimulating red blood cell production. Patients with chronic renal failure commonly have anemia secondary to low levels of this hormone. It is also a common performance enhancing drug which some famous athletes on the Tour de France were caught with recently.

2. "....is is hard to diagnose rheumatoid arthritis? No, they are usually deviant...". I liked this one. He is referring to ulnar deviation of the metacarpal bones in RA. Other common findings in the rheumatoid hand include swelling of the small joints - namely the MCPs and the PIPs, swan neck and boutonniere deformities in the fingers, median nerve entrapment, and trigger fingers (from nodules forming on tendon sheaths).

3. "...do you do math? Adding and subtracting? C'mon...this is a crisis!..." This was mentioned in the context of an elderly person on longstanding prednisone for a hemolytic anemia. "Adding"? "Crisis"? In a person on steroids? Yes, this is an Addisonian Crisis (adrenal crisis). Patients may present with clinical evidence of volume depletion, nausea, vomiting, shock, abdominal pain, and hyperkalemia with or without hyponatremia and hypoglycemia.

4. "...is it dangerous to take antibiotics? Is this a difficult hospital...?" Hmmm...'antibiotics'? 'Difficult'? Yep, this has got to be Clostridium difficile infection. A good review by local talent can be found here.

5. "...you look unflappable....is it frosty in here?...." I really liked this one. We were discussing a case of an elderly gentleman who presented with 1 week of malaise. He is referring to some signs of renal failure, including asterixis (unflappable - may also be seen in hepatic encephalopathy or hypercarbia), and the uremic frost. We don't really see uremic frost all that much - it's from severe uremia such that there are nitrogenous deposits on the skin Here's a case from NEJM with picture below.

Oh Schist!(....ocyte)

Microangiopathic Hemolytic Anemia (MAHA):

It is important to rule out the microangiopathic hemolytic anemia's when you see a CBC with anemia and thrombocytopenia. After a very detailed history and physical exam, a few additional tests must be done to clinch the diagnosis:

• Hemolyitic work up: LDH, unconjugated bilirubin, haptoglobin
• Coomb's test: to assess for autoimmune hemolytic anemia
• Blood film: looking for schistocytes
  

If there your patient has biochemical evidence of hemolysis and schistocytes on the blood film then this is likely MAHA. Note that a positive Coomb's test in autoimmune hemolysis will typically be associated with spherocytes on the blood film - this is not MAHA

What are some of the more important causes of MAHA?

1. Thrombotic Thrombocytopenia Purpura: presents with a classic pentad of fever, neurologic changes, renal failure, hemolytic anemia, and thrombocytopenia....although most patients have only 2-3 of these features. A great case-based approach to TTP can be found here.
   
2. Hemolytic Uremic Syndrome: on the same spectrum as TTP, more common in children, and as the name implies it has a renal failure phenotype. Here is a cool paper from JAMA looking at the risk of developing HUS after treating E. coli 0157:H7 strains.
   
3. Disseminated Intravascular Coagulation: check for coagulopathy with an INR, PTT, fibrinogen, and fibrin degradation products.
   
4. HELLP Syndrome: a variant of pre-eclampsia with hemolysis, elevated liver enzymes, and low platelets. Read more about this here.
   
5. Malignant Hypertension. More on this here.
   

Mitral Regurgitation

Mitral regurgitation is common. When you hear this murmur and are trying to determine the underlying cause, think about the individual components of the mitral valve, and particular disease states which may affect them. Let's start at the annulus, and work our way down:

1. Annulus: This may be dilated from cardiomyopathies, or calcified in diseases like rheumatic fever or chronic renal failure.
2. Leaflets: The mitral leaflets can fail in a number of disease states, including infectious endocarditis (acute or chronic), rheumatic fever, autoimmune conditions (SLE, scleroderma), myxomatous degeneration (MVP), connective tissue diseases (eg. Marfans), and congenital malformations.
3. Chordae: These can be damaged or rupture under ischemic, infected, or traumatic conditions and in rheumatic heart disease.
4. Papillary Muscle: These muscles can rupture after trauma or infarct. They become 'dysfunctional' under ischemic conditions, or when the LV becomes dilated (myopathy or aneurysm). Papillary muscle can also become infected, and rarely can have infiltration with amyloid deposits or granuloma (eg. sarcoid).

Here is a good review on the evaluation and management of mitral regurgitation from NEJM.

"Shot through the heart, and you're to blame.....You give love a bad name "......Bon Jovi, circa 1986

Pericarditis: an approach....

1. Idiopathic: many patients do not have an underlying etiology established. We assume that many of these patients have a 'viral' pericarditis.
2. Infectious:
   • Viral: classically Coxsackie, Echovirus, and Adenovirus, but certainly others.
   • Bacterial: think about Staph and Strep species, and never forget TB (mycobacterial)
   • Fungal: Histoplasmosis, Aspergillosis, Blastomycosis
   • Parasitic: Toxoplasomosis, Echinococcus
3. Malignant: usually metastatic disease like lung or breast cancers, or lymphoma.
4. Autoimmune: Think about lupus, rheumatoid arthritis, and mixed connective tissue diseases.
5. Metabolic: Uremic pericarditis is common, and hypothyroidism can cause a pericardial effusion.
6. Cardiac: a pericarditis can be seen early after an infarction, or sometimes within 3-4 weeks afterwards....the so called Dressler's Syndrome.
7. Drugs: can cause a drug-induced lupus. Common culprits include procainamide, INH, and hydralazine.
8. Other things: Radiation, Trauma.

COPD

Some principles in the management of acute COPD exacerbations:

1. Stability: patients should be in a setting where their vital signs can be monitered closely.

2. Oxygen: can be delivered with increasing capacity. Note that if you are requiring more intense oxygenation, consider other etiologies of hypoxia such as pulmonary embolism. Oxygen can be delivered by....

• nasal prongs
• venturi masks (delivered in increasing FiO2's)
• non-rebreathing masks
• non-invasive positive pressure ventilation
• intubation

3. Anticholinergic Agents: short acting ones like Ipratropium Bromide, in frequent and large doses.

4. Beta Agonists: also short acting ones like Salbutamol, also in frequent and large doses.

5. Steroids: administered PO for most, or IV if your patient is very ill and/or will not tolerate PO meds. Commonly used doses include Prednisone 40-60 mg PO daily, or Methylprednisolone 125 mg IV given 2 times per day. Treatment usually lasts from 5-10 days.

6. Antibiotics: Indicated in moderate to severe exacerbations. This includes patients who have 2/3 of:

• increased dyspnea
  
• increased sputum purulence
  
• increased sputum production

or those requiring invasive ventilation.

7. When the dust settles: After the acute event it is important to counsel patients on smoking cessation, ensure they have their proper vaccines, re-evaluate their medications and ensure they are taking them properly, and possibly arrange for pulmonary rehabilitation.

Links:
A good paper on infections in COPD from NEJM.
A review on the management of COPD exacerbations in a case-based approach.

Serum-Ascites Albumin Gradient

Calculate your Serum-Ascites Albumin Gradient:

If greater that 11 g/L: This is considered a transudate and is likely related to portal hypertension. Think about cirrhosis, CHF (right sided), constrictive pericarditis, or Budd-Chiari syndrome.

If less than 11 g/L: This is considered an exudate and is not related to portal hypertension. Think about pancreatitis, peritoneal carcinomatosis, peritoneal TB, serositis, or low albumin states like nephrotic syndrome.

(Physical exam manoeuvres for ascites, from the JAMA rational clinical exam. Link provided below).

1. JAMA's, Does This Patient Have Ascites?
   
2. Here is a great article on managing cirrhosis and ascites from NEJM.

Follow the arrows...it's that easy

An interesting Morning Report today on DKA - first presentation.












1. Here is a good approach to the confused patient.

2. You can read about precipitants to DKA here.

3. Check out the management of DKA/HONK here.

(the great Shane McConkey, pictured below)