SJS/TEN Teaching Pearls:

• SJS/TEN are severe mucocutaneous lesions
  • <10% of epidermal involvement categorized as SJS.
  • >30% of epidermal involvement categorized as TEN
  • 10-30% of epidermal involvement categorized as SJS/TEN
• Most commonly caused by:
  • Medications (allopurinol, anticonvulsants, sulfonamides, NSAIDS)
  • Infectious (mycoplasma pneumonia, CMV)
• Clinical Manifestations:
  • Prodrome of fever and flu-like symptoms 1-3 days
  • Development of mucocutaneous and skin lesions that start as erythematous macules.
  • Progress to vesicles and bullae formation within days prior to sloughing.
  • Mucocutaneous involvement occurs in >90% of patients (oral, ocular, urogenital).
• Complications
  • Fluid balance and electrolyte abnormalities
  • Infections
    • Staph Aureus and Pseudomonas
    • Fungemia
    • No role for empiric antibiotics.
  • Pulmonary
    • ARDS
    • Pneumonia
• Management
  • High level of suspicion as early withdrawal of medications important for treatment.
  • Symptomatic care
    • Consult ophtho, burn, and derm
    • Patient should be transferred to ICU burn care if moderate to high severity of disease.

Teaching Points:

• Normal Intracardiac Pressures
  • Right Atrium – 0-6
  • Right Ventricle: 15-30/0-6
  • Pulmonary Artery: 15-30/6-12
  • Left Atrium: 6-12
  • Left Ventricle: 100-130/6/12
• Heart failure exacerbations first results from elevated Left ventricular End Diastolic Pressure (LVEDP).
• Increased LVEDP –> increased LA pressures –> increased pulmonary pressure
  • Most common cause of right heart failure is left heart failure
• Mitral Stenosis heart sounds
  • Loud S1
    • Due to forceful closure of mitral valve.
  • Opening Snap (OS) with diastolic rumbling after S2
    • OS occurs later than along the cycle compared to a split S2 sound.
• Most common cause of mitral stenosis is rheumatic heart disease.
• Patients with mechanical prosthetic valves should be on Coumadin for anticoagulation due to increased thrombotic risk.
• Newer anticoagulants including anti-X and anti-II inhibitors have been shown to increase bleeding and thrombotic risks.

Teaching Pearls:

• In the setting of normal renal and endocrine function, you can utilize your urine studies to help determine the etiology of Hyponatremia
• If Urine osmolality is low, consider the following two etiologies:
  • Primary polydipsia
    • Clinical history becomes important to differentiate.
  • Low solute diet (tea toast diet, beer potomania)
    • Clinical history becomes important to differentiate.
• If urine osmolality is high, this suggests an abnormal feature as kidneys would normally increase free water excretion.
  • Check urine sodium. If high, consider:
    • medication use (SSRI), hypothyroidism, adrenal insufficiency, siADH (if others ruled out).
  • If low, then renal perfusion appears to be poor. This can be seen in some of the following conditions:
    • True hypovolemia
    • Hypervolemic states including CHF (cardiorenal syndrome), cirrhosis, etc

• The most common SVT is AVNRT (~60%), followed by AVRT (~30%), and Atrial Tachycardia (~10%).
• AVNRT is more common in women (~70%), mean age of onset is 32 years
• AVRT is more common in men, mean age of onset 23 years
• Atrial Tachycardia is more common with older age and structural heart disease
• Common symptoms of SVT include palpitations with bounding neck pulsations, exertional fatigue, dyspnea, chest discomfort, and pre-syncope (rarely syncope).
• Management of SVT includes vagal maneuvers followed by adenosine (6mg IV push followed by 20cc of NS) through a peripheral line. (If using a central line, the dose is lower).
• For unstable SVT, management includes synchronized cardioversion.
• A modified Valsalva maneuver was shown to be more effective than the traditional Valsalva maneuver (Appelboam, Andrew, et al. “Postural modification to the standard Valsalva manoeuvre for emergency treatment of supraventricular tachycardias (REVERT): a randomised controlled trial.” The Lancet 386.10005 (2015): 1747-1753) which includes placing the patient supine and doing a passive leg raise in addition to the Valsalva.
• For some videos about AVNRT and AVRT check out:
  • http://www.blaufuss.org
  • Khan Academy AVRT and AVNRT

Thanks to Brian Sun for presenting!

• Complete atelectasis/lung collapse can occur from mucous plugging or endobronchial lesions, look for tracheal deviation towards the side of atelectasis
• Consider atypical Legionella coverage for HCAP in patients with risk factors such as on glucocorticoids, transplant patients, GI symptoms, especially when not improving with standard antibiotics
• Drugs covering Pseudomonas include Imipenem, Meropenem (not ertapenem!), Fluoroquinolones, Aminoglycosides, 4th generation pencillins, and 3/4th generation cephalosporins (Ceftriaxone, Ceftazidime, Cefepime)

• Tumor lysis is most commonly seen in Burkitt’s lymphoma and other hematologic malignancies after initiation of cytotoxic chemotherapy
• Spontaneous tumor lysis is rare
• Risk factors for TLS include chemosensitive malignancies, bulky Dz > 10 cm, pretreatment hyperuricemia, and dehydration
• Evaluation of the calcium, phosphorous, uric acid level, and potassium levels are the first step in diagnosis
• Treatment consists of intravenous fluids, uric acid lowering agents, and possibly dialysis
• Allopurinol decreases production of uric acid while rasburicase shunts uric acid into allantoin to minimize uric acid precipitation in the renal tubules
• Can’t use Rasburicase in patients with G6PD deficiency
• Stages of hyperkalemia
  • > 5.5 peaked Ts
  • >6.5 atrial paralysis –> p wave widens/flattens, prolonged PR, then P’s disappear
  • > 7.0 conduction abnormalities, bradycardia, wide QRS with bizzare morphology, sine wave
  • > 9.0 cardiac arrest, asystole, V fib

Teaching Pearls:

• Emergent clinical manifestations of diabetes includes diabetic ketoacidosis (DKA) and hyperosmolar hyperglycemic state (HHS).
• DKA Manifestations
  • Presence of ketones
  • Glucose usually less than 700
  • Can present with N/V and abdominal pain
  • Acute presentation
• HHS Manifestations
  • Lack of ketones present
  • Glucose usually greater than 700
  • Usually present with neurological changes including stupor and coma.
  • Subacute presentation
• A small subset of DKA can present with euglycemic DKA.
  • Patients present with presence of ketones and a normal to near normal glucose levels.
  • More commonly seen in patients with poor PO intake, recent insulin use prior to ED arrival, and pregnancy state
  • Management is geared towards to decreasing ketones.
  • Other causes of anion gap are ruled out, with presence of ketones.
• Management
  • Similar for DKA (including euglycemic) and HHS
  • Fluid resuscitation as many of these patients are hypovolemic.
  • Insulin gtt for moderate to severe manifestations
    • 0.1units/kg bolus followed by 0.1units/kg/hour or:
    • start at 0.14units/kg/hour without a bolus

Teaching Pearls:

• Intrinsic Coagulation Pathway
  • XII, XI, IX, VIII
  • Manifested by PTT
• Extrinsic Coagulation Pathway
  • Factor VII
  • Coumadin decreases production of Factors 2, 7, 9, and 10. Factor VII has the shortest half-life so PT/INR changes are seen first.
  • Manifested by PT/INR
• Factor VIII Deficiency (Hemophilia A)
  • Genetic
    • X-link recessive trait
      • Present mostly in males at younger age
    • Due to decreased ability to produce factor VIII
    • Elevated PTT due to factor VIII deficiency
    • Treated with prophylactic Factor XIII infusion
    • Clinically presents with hemarthrosis, which can be very severe causing chronic disabling joint disease.
  • Acquired
    • Can be seen in malignancy, post-partum state, or with other autoimmune diseases
    • Can also be idiopathic
    • Elevated PTT due to inhibitor protein against factor VIII
    • Treatment depends on concentration of inhibitor
      • Fast Responder (high inhibitor) – treat with PCC/FFP if actively bleeding, and immunosuppression.
      • Slow Responder (low inhibitor)  – can be treated with factor VIII infusion.
    • Clinical bleeding can manifest in a variety of ways as compared to congenital factor VIII deficiency.
• Von Willebrand’s Disease (vWF)
  • Most common genetic cause of bleeding.
  • vWF protects factor VIII from being degraded.
    • vWF deficiency can lead to PTT prolongation.
  • vWF binds to a receptor G1b on the platelets to achieve primary hemostasis.
    • vWF disease results in increased bleeding time, which is a result of poor platelet binding and aggregation.

Teaching Pearls:

• Common causes of pancreatitis:
  • gallstones, alcohol, hypertriglyceridemia, hypercalcemia, medications, infectious, trauma, scorpion bites, autoimmune, idiopathic, etc
• Not recommended to trend lipases
  • Although on certain occasions, decrease in lipase may help suggest a certain etiology.
  • Fast decrease in lipase level can be associated with passed gallstone.
• Normal CBD normal is <6mm. Can widen in non-pathologic conditions including age and post-cholecystectomy.
• LFT Abnormalities
  • Hepatocellular – defined by severe elevation in AST and ALT in proportion to Alk phos
  • Cholestatic – defined by severe elevation in alkaline phosphatase compared to AST and ALT
  • Both can have elevated bilirubin levels
• Causes of cholestatic jaundice includes:
  • Primary biliary cirrhosis, primary sclerosing cholangitis, large bile duct obstruction, infiltrative liver disease, malignancy (pancreatic carcinoma, ampullary carcinoma, cholangiocarcinoma), cholangitis, extrahepatic pancreatic mass, choledocholithiasis.
• MRCP is good for detecting proximal bile duct lesions, but sensitivity worsens distally.
• EUS has good sensitivity for distal bile duct lesions and obstructions.
• Autoimmune Pancreatitis
  • Can manifest by a pancreatic mass that is often confused with pancreatic carcinoma.
  • Recurrent pancreatitis is common and occurs more frequently in focal disease.
  • May lead to pancreatic duct strictures.
  • Most common presentation is obstructive jaundice.
  • A pancreatic biopsy is usually required to establish the diagnosis.
  • Important to check IgG4 levels and CA 19-9.
    • Higher levels of IgG4 levels decreases sensitivity, but increases specificity of autoimmune pancreatitis.

• Thyroid storm is a clinical diagnosis. Severe thyrotoxicosis + systemic decompensation
• The degree of TSH suppression/T4 elevation in patient’s with thyroid storm is comparable to patients with uncomplicated overt hyperthyroidism
• Thyroid storm can be differentiated from thyrotoxicosis by the presence of temperature elevation, significant tachycardia, heart failure, gastrointestinal disorders, diarrhea, nausea, vomiting, and sometimes jaundice. Neurologically, agitation and disorientation can occur.
• Etiology of Thyroid Storm
  • Thyroid storm most commonly occurs in Graves disease and has a higher frequency in younger women, but it also can be due to a toxic adenoma or multinodular goiter.
  • Can occur in long-standing, untreated hyperthyroidism but usually is precipitated by an underlying condition, such as surgery, infection, trauma, parturition, acute iodine exposure, radioactive iodine (131I) therapy, or ingestion of medications, including amiodarone, salicylates and pseudoephedrine.
• Even with aggressive therapy and supportive measures, mortality after thyroid storm is as high as 15% to 20%.
• Geriatric (Apathetic) Hyperthyroidism
  • Hyperthyroidism in elderly patients may be apathetic, rather than having hyperactivity, tremor, and other symptoms of sympathetic hyperactivity.
  • Older patients more commonly present with atrial fibrillation as well.
  • Take Home Point: In the elderly patient with new onset atrial fibrillation or depression/somnolence, make sure to consider hyperthyroidism.