Rheum with A View
Liv-er Let Die
Heme-agen Dragons
I've Got Rhythm
Smorgaas-Board
Final Jeopardy
100
The most likely diagnosis for a 40 year old woman with pain, tenderness and swelling of the small joints of the hand and foot. It is accompanied by patches of erythema and scaling behind the ear and at the occiput of the scalp.
Changes of the nail are seen below:
What is psoriatic arthritis?
Key Point
Psoriatic arthritis is associated with psoriasis, enthesitis, dactylitis, tenosynovitis, arthritis of the distal interphalangeal joints, asymmetric oligoarthritis, and spondylitis.
Nails should be examined for pitting or onycholysis.
The recently developed Classification Criteria for Psoriatic Arthritis (CASPAR) have a sensitivity and specificity of more than 90%, especially for the diagnosis of early psoriatic arthritis.
100
According to the American Association for the Study of Liver Disease, this disorder is projected to become the leading cause of liver cirrhosis/transplantation in the US by 2030.
What is NASH/NAFLD?
Key Point:
NAFLD is the most common cause of abnormal liver test results in the United States. Approximately 30% of the U.S. population has NAFLD, some of whom have normal liver enzyme levels. Most patients with NAFLD have insulin resistance associated with obesity, hypertriglyceridemia, and/or type 2 diabetes mellitus. Approximately 20% of patients with NAFLD have nonalcoholic steatohepatitis (NASH), which is characterized by hepatic steatosis accompanied by inflammation and often fibrosis.
100
The underlying disorder for this case presentation:
61 year old man presents with transient visual disturbances, hypertension, erythromelalgia, aquagenic pruritus. On exam patient is found to have facial plethora and splenomegaly. Labs show +JAK2 mutation.
What is Polycythemia Vera (PV)?
PV is a myeloproliferative disorder due to clonal rbc proliferation that is EPO-independent. Most patients have a low EPO level and +JAK2 mutation testing. Patients at low risk for thrombosis (<60y/o, no prior thrombosis) are treated with serial phlebotomy, low-dose aspirin, and symptom control. Those at greater thrombus risk or active thrombus also require myelosuppression.
100
The drug used to initially treat a patient with dizziness and syncope, normal blood pressure, and this rhythm: 
What is atropine?
Atropine is used for treating symptomatic sinus bradycardia and may be beneficial for any type of AV block at the nodal level.
The recommended dose for bradycardia is 0.5mg IV every 3 to 5 minutes, up to 3mg.
100
This is the treatment of choice for idiopathic hyperaldosteronism
What is spironolactone/eplerenone?
Medical therapy with an aldosterone receptor antagonist (spironolactone or eplerenone) is the treatment of choice for primary aldosteronism due to idiopathic hyperaldosteronism, or when patients with APA are not candidates for, or do not wish to undergo, surgery. Spironolactone is often preferred over eplerenone because it is less expensive and more potent. However, patients on spironolactone are more likely to develop dose-dependent side effects of gynecomastia and erectile dysfunction in men and menstrual irregularities in women. Hypokalemia almost always resolves with treatment, but blood pressure control may require additional agents. No studies clearly show superiority of adrenalectomy compared to medical therapy for APA, but surgery may be more cost-effective in the long term.
100
This physical exam skin finding can be found in patients with profound azotemia:
What is uremic frost?
Uremic frost is the dermatologic manifestation of profound azotemia and occurs when urea and other nitrogenous waste products accumulate in sweat and crystallize after evaporation. This clinical finding is very uncommon because of the earlier detection and treatment of end-stage kidney disease.
200
A 40 year old man with history of well controlled chronic plaque psoriasis who suddenly develops painful joints and worsening psioratic plaques of the trunk and extremities as picture, should be tested for this infection.
What is HIV testing?
Key Point
The development of explosive onset or severe flare of psoriatic arthritis should raise suspicion for concomitant HIV infection.
200
The most likely diagnosis in a 58 year old women with hypercholesterolemia, progressive fatigue and generalized pruritis without rash, elevated LFTs, and Antimitochondrial Ab 1:640.
What is primary biliary cirrhosis?
Primary biliary cirrhosis (PBC), now termed “primary biliary cholangitis”, is a chronic cholestatic liver disease of unknown cause. PBC mainly affects middle-aged women. Most patients are asymptomatic at presentation but develop symptoms of PBC within 10 years; symptoms include fatigue, dry eyes, dry mouth, and pruritus. Hyperlipidemia is common, but jaundice, cutaneous hyperpigmentation, hepatosplenomegaly, and xanthelasmas are rarely observed at diagnosis. PBC is diagnosed by serum alkaline phosphatase levels greater than 1.5 times the upper limit of normal and typically normal serum total bilirubin levels. Serum aspartate (AST) and alanine (ALT) aminotransferase levels are typically less than five times the upper limit of normal. Serum antimitochondrial antibody is present in 90% to 95% of patients. For patients with undetectable serum antimitochondrial antibody levels, a liver biopsy is required for diagnosis. Treatment with ursodiol slows disease progression and may prevent or delay advanced disease and the need for liver transplantation.
200
This is the standard long-term treatment to prevent recurrent thromboembolism in cancer patient.
What is Enoxaparin?
Low molecular weight heparin (LMWH), e.g. enoxaparin, is more effective than vitamin K antagonists (e.g. warfarin) in reducing the risk of recurrent venous thromboembolism in cancer patients. However, LMWH is not associated with improved survival in these patients.
200
This device is indicated for secondary prevention in a patient having an episode of hemodynamic instability, and this rhythm strip
:
What is an Implanted cardioverter-defibrillator (ICD or AICD)?
ICDs are indicated as secondary prevention in patients with prior episdoe of resuscitated VT/VF or sustained hemodynamically unstable VT in whom a completely reversible cause cannot be identified, or paitents with episodes of spontaneous sustained VT in presence of heart disease.
They are indicated as primary prevention in patients with prior MI and LVEF <30%, NYHA II/III with LVEF <35% after 3 months of optimized medical therapy, or other high risk conditions such as congenital long QT syndrome, HOCM or Brugada syndrome.
200
Patients with bullous emphysema are at risk of this medical emergency while traveling by air.
What is pneumothorax?
During air travel, pneumothorax is most likely to occur at cruising altitude in patients with bullous lung disease, particularly those with a recent exacerbation of airways disease who are, therefore, more prone to air trapping.
300
The most likely diagnosis in a 44 year old male who has color change in the hands when exposed to the cold, finger skin tightness with palpable nodules, fatigue, and pruritus.
His physical exam shows skin thickening of the face and finger. There are a few firm, gritty nodules on the palmar aspect of the digits. There is no rash or digital pits.
There are no telangectasias or esophageal dysmotility present. 
What is limited cutaneous systemic sclerosis?
Key Point:
Limited cutaneous systemic sclerosis is characterized by distal skin thickening (face, neck, and hands) and is typically not accompanied by internal organ fibrosis; patients may display features of the CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome.
300
The most likely diagnosis of an 18 year old woman hospitalized with confusion and jaundice after taking tylenol, and physical exam including asterixis and palpable spleen, and the picture eye exam:
What is Wilson's Disease?
Wilson disease is a rare congenital disorder of copper excretion that occurs in approximately 1 in 30,000 live births. Young patients with Wilson disease tend to present with acute liver failure; older patients present with chronic liver disease and/or neurologic manifestations. Wilson disease should be considered in all patients younger than 40 years of age who have unexplained liver disease.
When Wilson disease causes acute hepatitis, usually in young patients, the sudden release of copper from liver cells can also induce hemolytic anemia.
Kayser-Fleischer rings, noted on ophthalmologic examination, indicate copper deposition in the Descemet membrane of the iris. Patients with acute liver failure due to Wilson disease rarely recover and should be urgently referred for liver transplantation.
300
This enzyme is deficient in a patient presenting with jaundice, dark urine, fatigue and "bite cells" on peripheral smear after taking trimethoprim-sulfamethoxazole.
What is Glucose-6-Phosphate Dehydrogenase Deficiency?
Onset of acute hemolysis after treatment with TMP-SMX, negative direct and indirect anti-globulin (Coombs') test, and the presence of "bite cells" on peripheral smear is consistent with a diagnosis of Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency. It is a x-linked disorder, commonly induced by oxidative stress via medications (e.g. dapsone, antimalarials, sulfonamide antibiotics), ingestion of fresh fava beans, infections, metabolic abnormalities (e.g. DKA). Typically presents 2-4 days after an oxidatively stressful event with evidence of hemolysis. Testing for G6PD deficiency during an acute hemolytic episode may produce a false negative result as the older and more deficient erythrocytes are destroyed and replaced by reticulocytes that commonly have normal G6PD levels. If a false negative is suspected, it is recommended to recheck the G6PD level 2-3 months after the hemolytic episode.
300
This is the recommended treatment for the patient with BP 78/45 and the following rhythm:
What is synchronized cardioversion?
Hemodynamically unstable SVT should be treated with synchronized cardioversion. Stable patients may be initially treated with vagal maneuvers, followed by adenosine to ascertain the underlying arrhythmia if there is no response.
300
The most likely diagnosis in a patient with abdominal pain and constipation and the following xray.
What is a sigmoid volvulus?
Xray imaging with classic "coffee bean" sign is indicative of signoid volvulus. Risk factors include males, elderly age, chronic constipation.
It is hypothesized that chronic fecal overloading from constipation may cause elongation and dilatation of the sigmoid colon, predisposing patients to sigmoid volvulus, thereby explaining its higher incidence in older institutionalized adults with constipation.
400
This is the most appropriate treatment for a 24 year old woman with tender nodules over the legs, stiffness of ankles, and productive cough. Her labs show ESR of 38mm/hr, HCT of 35% and normal WBC and platelet counts.
Joint xrays show no bony erosions.
Chest xray appears as this:
What are NSAIDS?
Key Point:
This patient has Löfgren syndrome, a self-limiting form of sarcoidosis characterized by a triad of acute arthritis in combination with bilateral hilar lymphadenopathy and erythema nodosum. The “arthritis” associated with Löfgren syndrome is actually a nondestructive periarthritis of the soft tissue, entheses, and tenosynovium around the joints.
When the triad of features occurs, it has a 95% specificity for diagnosis, and further diagnostic tests (such as radiography or serologic testing) are unnecessary.
Ninety percent of patients remit within 12 months.
400
These two are the most appropriate treatments for a 56 year old woman hospitalized with confusion who has a hx of decompensated hepatitis B cirrhosis and chronic ascites.
Physical exam shows disorientation to time and date, asterixis and moderately distended abdomen with ascites.
Labs show T bili 2.8, Cr 1/3, BUN 34.
Paracentesis shows 640 PMN cells.
What are 3rd gen cephalosporin (e.g. cefotaxime) and albumin?
Key point:
This patient has been hospitalized with overt hepatic encephalopathy, which in many instances may be the sole presenting feature of spontaneous bacterial peritonitis (SBP). It is important to perform a diagnostic paracentesis in patients with ascites who are admitted to the hospital, especially those with overt hepatic encephalopathy. Ascitic fluid analysis with PMN >250 is diagnostic of SBP.
Patients with a serum creatinine level greater than 1 mg/dL (88.4 µmol/L), a serum bilirubin level greater than 4 mg/dL (68.4 µmol/L), or a blood urea nitrogen level greater than 30 mg/dL (10.7 mmol/L) should receive 1.5 g/kg of intravenous albumin (25%) on the day of diagnosis and 1 g/kg of albumin on day 3; this practice has demonstrated a survival benefit.
400
This condition should be considered in a 22 year old pregnant woman with fever, confusion, thrombocytopenia, schistocytes on blood smear, and renal insufficiency.
What is Thrombotic Thrombocytopenic Purpura (TTP)?
TTP is characterized by the formation of small-vessel, platelet-rich thrombi due to severe deficiency of the vonWillebrand factor-cleaving protein ADAMTS13. TTP can be associated with pregnancy due to the formation of autoantibodies (acquired) or the emergence of a previously unknown heredity case. Patients with TTP can be minimally symptomatic or critically ill but classically have: Severe thrombocytopenia ~10,000 platelet count, Microangiopathic hemolytic anemia (MAHA) - schistocytes on peripheral blood smear, organ dysfunction (thrombi-indued ischemia can lead to neurologic manifestations and mild renal insufficiency. Diagnostic studies will reveal normal coagulation studies negative Coombs testing, and ADAMTS13 levels of <10%. Urgent treatment with plasma exchange, glucocorticoids, and rituximab are required to prevent death
400
This affliction should be suspected in a patient with sudden shortness of breath, chest pain, hemodynamic instability and the following EKG:
What is Pulmonary Embolism/Massive PE/Saddle emobolus?
Pulmonary embolism can result in EKG changes including sinus tachycardia, RBBB, right ventricular strain and the "classic SI QIII TIII" pattern.
400
The classic radiographic finding in aortic coarctation.
What is rib notching (Roesler sign)/figure 3 sign?
Figure 3 sign results from contour abnormality of the aorta
Inferior rib notching, or Roesler sign, occurs from dilated intercostal collateral vessels
500
The is the underlying disorder of a 46 year old woman who has a summer time rash and noted physical findings of eyelid swelling and a periorbital violaceous rash, erythema and poikiloderma of the anterior chest and upper back, and an erythematous papular rash on the hands picture below; Muscle strength and reflexes are normal.
Labs show:
Normal CBC and Chem Panel
Creatinine kinase 100
ANA titre 1:640
What is amyopathic dermatomyositis?
Key Point:
Amyopathic dermatomyositis refers to dermatomyositis with cutaneous involvement in the absence of clinical, laboratory, electromyogram, or biopsy evidence of myositis.
500
This typical treatment for pulmonary hypertension should be avoided in patients with PAH who present with hx of liver cirrhosis and have portal hypertension.
What are calcium channel blockers?
Key points:
Portopulmonary hypertension is a Group 1 PAH that occurs in present of CLD with portal HTN. It has poor vasoactive response and can cause hypotension with splanchnic vasodilation.
Preferred medical treatments are prostacyclin analogues (epoprostenol), endothelin antagonists (bosenten), or phosphodiesterase inhibitors (sildenafil).
Other treatments to avoid:
Beta blockade
TIPS
500
This test should be ordered in a patient with recent NSTEMI (6wks ago) whose platelets decrease from 210K to 62K while receiving enoxaparin DVT prophylaxis on the sixth day of subsequent hospitalization for acute CHF exacerbation.
What is Platelet Factor 4 (PF4) Antibody?
Heparin-induced thrombocytopenia (HIT) should be suspected in patients treated (or recently treated) with heparin who develop unexplained thrombocytopenia (or a > 50% decrease in platelet count from baseline) or newly diagnosed thrombosis (venous or arterial). Once HIT is suspected, all forms of heparin must be stopped immediately, laboratory testing for HIT should be obtained, and alternate anticoagulants (eg, direct thrombin inhibitors; e.g. Argatroban) should be started even if thrombosis has not occurred.
500
This is the most appropriate treatment for a patient on adequate medical therapy having continued symptoms and the following EKG:
What is catheter ablation?
Atrial flutter is typically characterized on ECG as sawtooth pattern with inverted flutter waves in lead II,III, and aVF, and positive flutter waves in lead V1. Catheter ablation is the definitive treatment for typical atrial flutter and is indicated when patient does not respond to rate control/anti-arrhythmic treatments.
500
This protein present in CSF is suggestive of sporadic Creutzfeld-Jakob disease
What is T-tau / 14-3-3 protein?
CJD is the most common form of prion disease and is characterized by involvement of several neurological symptoms and rapid onset of apparent dementia. Phys ex may show ataxia, myoclonus as well as dementia. Definitive diagnostic test is brain biopsy showing widespread spongiform changes with gliosis.