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Moffitt DOUBLE Pearls 4.11.18

Double Pearls!

CARDS Report

Thank you to Alayn for presenting the case of a middle aged man with Hennekam syndrome c/b by protein losing enteropathy, immunodeficiency, anasarca and recurrent pericardial effusions. The patient has had a prolonged course with recurrent infections and an untappable pericardial effusion. We discussed clinical and radiographical evidence of tamponade vs constrictive pericardial disease and focused on the management of his complex effusion w/ the possibility of a window vs pericardial stripping.

Key Pearls

  • In any patient presenting with ACS (UA, NSTEMI or STEMI) the standard of care is DAPT for 1 year regardless of management (medical vs interventional) AND regardless of type of stent placed.
  • We learned that Hennekam syndrome is rare autosomal recessive intestinal lymphagiectasia-lymphedema syndrome associated with mental delay, immune deficiency and recurrent effusions. See this article for more info!
  • Despite radiographic evidence suggestive of tamponade in this case, the diagnosis should be made clinically. Beck’s triad of 1) elevated JVP 2) tachycardia/hypotension and 3) distant heart sounds are components with a pulses paradox > 10 mmHg is suggestive tamponade physiology.
  • A patient with tamponade will have a normal BNP (as the myocardium is not stretched).
  • Make sure to measure a pulses correct! It is the systolic BP drop with inspiration and is positive when > 10 mmHg. Here is the link on YouTube how to measure this correctly.

More on Tamponade

  • In patient with concern for tamponade a positive pulses > 10 mmHg is 98% sensitive and 84% specific (Circulation 64: 663-640).
  • Physiology includes increased intrapericardial pressures that then impair diastolic filling and result in a drop in cardiac output.
  • Other causes of pulses paradox include the following:
    • Tamponade
    • Constrictive Pericarditis
    • Asthma/COPD Exacerbations
    • Large PE
    • Tension PTX
    • Large Pleural Effusions

GI Report

Thank you to Lev for presenting the sad case of a middle aged woman with NASH cirrhosis and decompensation transferred from LTU given she is not a transplant candidate 2/2 to a BMI > 40. She remains on pressors with c/f rarer cause of refractory shock including AI or thiamine deficiency.

  • In any patient with cirrhosis remember the pneumonic VIBES to help with relevant history taking = Volume (ascites), Infection (SBP), Bleeding, Esophageal (Varices), Screening (HCC).
  • The original Model for End Stage Liver Disease score is made up of a patient’s serum bilirubin, INR and Cr. This score is a validated predictor of survival among patients with advanced liver disease and is used to determine allocation of organs for transplant.
  • The MELD-Na score has since replaced the original MELD scoring system given improved predictive accuracy, especially for those with ascites. There is an increase in mortality of ~5% for each millimole decrease in serum sodium between 125 and 140 (Clinical Liver Disease, April 2015).
  • A BMI > 40 is often a contraindication for transplantation given prior studies in the 1990s showing worse outcomes. These studies are felt to be flawed given they did not account for fluid overload which may have been driving these results.
  • The BMI > 40 cut off is still debated and practice may be changing with newer data supporting expanding this cut-off – a 2015 meta-analysis in Liver International looked at 13 studies spanning a 13 year period and found no difference in mortality b/t obese patients and controls (Saab S, et al. The impact of obesity on patient survival in liver transplant recipients: A meta-analysis. Liver International. 2015;35:164.
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Moffitt Pearls 4/4/18 – Calcinosis Cutis, Calciphylaxis, NMS and Serotonin Syndrome

Thank you to Andrew for presenting an interesting case of a young women with ESRD 2/2 lupus nephritis and failed transplant on PD presenting with painful purpura c/f calcinosis cutis. The patient subsequently developed AMS, sinus tachycardia, clonus and hyperreflexia c/f serotonin syndrome.

Calcinosis Cutis

  • Calcinosis cutis is described as the deposition of insoluble calcium salts in the skin and subcutaneous tissue.
  • There are five subtypes of calcinosis cutis: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis.
  • There is an association with autoimmune disorders – mostly dermatomyositis and mixed connective tissue disorders. It is less commonly associated with SLE.
  • Associated with trauma, underlying Calcium and Phosphorus metabolism disorders: the higher the Ca++ and Phos the higher the risk (one rule of thumb is Ca++xPhos > 55 puts this patient at high risk)

calcinosis cutisNEJM

Calciphylaxis

  • Calciphylaxis is a rare and serious disorder that presents with skin ischemia and necrosis and is characterized histologically by calcification of dermal arterioles
  • Main risk factor is ESRD and is more formally know as calcific uremic arteriolopathy (CUA)

 

  • Clinically may present in a serpiginous pattern as livedo reticularis and/or violaceous, painful, plaque–like subcutaneous nodules.
  • The optimal therapy is not known. See uptodate for a helpful flow chart on management principles.

calcphylasxis

Serotonin Syndrome vs NMS

NMS vs SS

SS diagnosis

Moffitt Pearls – 4/2/18 – PPP Syndrome

Thank you to Emma for presenting a case of a young woman presenting with chronic bilateral ankle pain, recurrent pancreatitis, and multiple subcutaneous nodules ultimately diagnosed with the very rare condition of PPP syndrome! HH and Andrew were onto this early based on the odd dark, brown fluid drainage from her wounds.

  1. You can organize the causes of polyarthritis in several large buckets: infectious arthritis, postinfectious or reactive arthritis, rheumatologic conditions, and 2/2 other systemic illness. See below for more details!
  2. ANA-negative Lupus is extremely rare! Previously, it was reported as high as 5% of patients, but it turns out that the negative finding occurred as a consequence of testing practices rather than the absence of the autoantibodies. Previous ANA indirect immunoflouresence used to use rodent tissues as substrate and were less sensitive than modern testing practices.
  3. What the heck is PPP syndrome? This is an extremely rare syndrome in which patients’ with Pancreatitis have translocation of pancreatic enzymes into the blood which can cause Panniculitis, Polyarthritis and multi-focal intraosseous fat necrosis. Interestingly, while the systemic manifestations are thought to be a consequence of high blood levels of pancreatic enzymes, abdominal symptoms are often mild or absent!
  4. Here’s a case report and review on this rare condition: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5288313/pdf/main.pdf

 

Major Causes of Inflammatory Polyarthropaties: (adapted from UpToDate)

Infectious arthritis Bacterial
Lyme disease
SBE
Viral – EBV, CMV
Postinfectious Rheumatic fever
Reactive arthritis
Enteric infection
Rheumatologic Other seronegative spondyloarthritides
Ankylosing spondylitis
Psoriatic arthritis
IBD associated
Rheumatoid arthritis
Inflammatory osteoarthritis
Crystal-induced arthritis
Systemic rheumatic illnesses
SLE
Systemic vasculitis
Systemic sclerosis
Polymyositis/dermatomyositis
Stills
Behcets
Relapsing polychondritis
Other Systemic Illness Sarcoid
Pallindromic rheumatism
Familial Mediterranean fever
Malignancy
Hyperlipoproteinemias

Evernote: https://www.evernote.com/shard/s462/sh/3b42c894-2f53-49e6-a801-2763ce8a9383/bff0b477bdd7520bbf45b515607492bc

 

Moffit Pearls 3.27.18 – Cards Report – Collapse

Thank you to Anne Thorson + Satvik for joining us Tuesday as Albert Liu presented the fascinating case of a middle aged man with hx of CAD + HFrEF presenting after chest pain and collapse found to be in VT/VF arrest in the field. He was coded in the field and quickly had ROSC with subsequent full neurological function after the amazing management by the primary team. It is felt that an arrhythmia (possibly monomorphic VT from prior scar) led to collapse given mild troponin rise, unchanged TTE + EKG.

Key Pearls!

  1. A summary of the top articles for cooling are below. Note: Improved outcomes for cooling in post-arrest patients are in the setting of out of hospital VT/VF.
  2. In a patient with a hx of CAD a wide complex tachycardia is VT/VF until proven otherwise. “When in doubt shock it out – anonymous.” Prior cardiac disease or MI has the highest predicative value for recurrent VT/VF.
  3. Digoxin used for AF in the outpatient is associated with INCREASE overall mortality. Indications for digoxin use are those patient with RV dysfunction and AF as it improves symptoms and can help with rate control.
  4. HH Pearl!! Normalization of ventricular response in a patient with AF should prompt investigation for digoxin toxicity especially in renal dysfunction.

 

More on Cooling Patients (see attached doc made by incoming chief Laura Huppert!!)

  • Contraindications to cooling: If patient is following commands post-arrest, coaguloapathy, active infection can sometimes limit things (although to be honest I’m not sure how/why, will ask more…)
  • Complications: Electrolyte disturbances, coagulopathy, bradycardia
  • SUMMARY: Cooling is indicated in VT/VF arrests. The data is limited for cooling after PEA arrest but because there is some indication of benefit it’s our neurology department’s current practice to offer it. So call neuro STAT after EITHER PEA arrests or Vfib/Vtach arrests to discuss indications for cooling.

1: http://www.nejm.org/doi/full/10.1056/NEJMoa012689#t=abstract

European study, cooling 32 to 34 degrees for 24h after out of hospital cardiac arrest (OHCA)

– 6 month favorable neurologic outcome in cooling group was 55%, compared to 39%…. number needed to treat of 6 !!

2:  http://www.nejm.org/doi/full/10.1056/NEJMoa003289

Australian study, OHCA, vfib arrest, cooling to 33 degrees within 2h after ROSC for 12 hours total

– 49% of patients in hypothermia group compared to 26% of normothermia group… number needed to treat of 4 !!!!!

3: http://www.nejm.org/doi/pdf/10.1056/NEJMoa1310519

TTM study, comparing targeted temperature management (TTM) to 33 degrees vs 36 degrees

– OHCA, presumed cardiac cause, but irrespective of initial rhythm

– no difference in mortality or neurologic function at 180 days

REMEMBER THOUGH, that everyone was kept at these specific temps, and 36 degrees controlled for 24h is NOT the same as “normothermia”

4: http://jamanetwork.com/journals/jama/fullarticle/2565288

Retrospective cohort study of successfully resuscitated patients from in-hospital cardiac arrest from March 2002 and December 2014

– found that the use of therapeutic hypothermia among patients with in-hospital cardiac arrest compared with usual care was associated with LOWER likelihood of favorable neurologic survival and likelihood to survival to discharge

– A big issue is that this is not a prospective randomized study with pre-specified outcomes. For me the biggest issue: 20.9% of cooling patients had temps lower than 32 degrees

 

https://www.evernote.com/shard/s307/sh/2f83acf2-8348-47c8-b851-f7b86152506c/55b02fe5b19cb3c911f4c1b13a3b2a07

Moffitt Pearls 3.23.18 – Chlamydia Proctitis and Syphilis

Thank you to Michelle for presenting the case of a young man presenting with symptomatic anemia found to have chlamydia proctitis and a positive RPR.

Key Pearls

  1. The diagnostic test of choice for chlamydial infection of the genitourinary tract is nucleic acid amplification testing (NAAT) of vaginal swabs for women or urine for men. Many laboratories have also validated NAAT on rectal swabs to diagnose chlamydial proctitis
  2. CDC recommended regimen for treatment of chlamydia proctitis is CTX 250 mg IM x1 PLUS Doxycyline 100 mg PO x 7 days
  3. In high risk patients diagnosed with chlamydia proctitis it is important to treat them for gonorrhea b/c of the risk of co-infections (hence the dual coverage above).
  4. Patients with severe proctitis may have lymphogranuloma venereum (LGV) which requires a full 3 weeks of therapy. See more info on LGV from prior blogs!
  5. As in this case, if the timing of syphilis is not known, late latent syphilis is presumed. Penicillin G IM once weekly for 3 weeks is the treatment of choice.

 

Proctitis:

Inflammation of the lining of the rectum within 10-12 cm of the dentate line.

Differential Diagnosis:

# Infectious

  • Most commonly gonorrhea or chlamydia (including LGV strains with rq longer duration of therpay)
  • Other causes include HSV (more common in immunosuppressed patients), syphilis (usually secondary), C. difficile or parasites (amebiasis).

# Inflammatory

  • Crohn’s disease and Ulcerative colitis

# Ischemia

# Radiation or Chemical

# Trauma/instrumentation

 

Review of Syphilis and Therapy can be found in table form here.

See prior blog from the amazing Grant Smith for more on LGV

Moffitt Pearls 2.26.18 – Hypophosphatemia

Thank you, Matt, for presenting a case of an elderly woman with a history of recent diagnosis of lung cancer s/p chemo and XRT, weight loss, and multiple recent falls who presented after another mechanical fall with post-traumatic emesis.

Key Pearls:

    1. Check out this fantastic prior chief post from Christy that covers an approach to falls in the elderly. Christy also outlines how to perform and interpret the Get up and Go Test!
    2. Emesis after head trauma is an important historical feature. By some definitions, post-traumatic vomiting is diagnostic of concussion. Furthermore, in this case series of over 5000 adults and children presenting to the ED for head injury, post-traumatic vomiting was associated with a fourfold increase in the relative risk for a skull fracture.
    3. Undetectable phosphate?? What causes that? Big categories include poor nutrition or malabsorption, GI losses, cellular shifts, and renal losses. See more below.

 

 

Clinical manifestations of severe hypophosphatemia are widespread. They can include anorexia, muscle weakness, osteomalacia, progressive encephalopathy, seizures, coma, and death. Hematologic disturbances include hemolytic anemia, decreased release of O2 from Hb, and impaired leukocyte and platelet function.

Mechanisms for Hypophosphatemia

Less Coming In Cellular Shifts More Going Out
Poor Nutrition

–          alcoholics

Refeeding syndrome GI Losses

–          chronic diarrhea

–          chronic phosphate-binding aluminum (antacids)

Malabsorption

–          short gut

–          Med effects: PPIs

Acid-base abnormalities

–          Severe respiratory alkalosis

 

Renal Losses

–          Fanconi syndrome (+/- Bcell neoplasm)

–          Hyper PTH (or PTHrP)

–          Diuretics

–          Vitamin D deficiency

 

Moffitt Pearls 2/14/18 – GI Report – Liver Masses

Thank you to Bennett and Scott for presenting the case of an middle aged man presenting from an outside hospital with RUQ pain found to have a rapidly growing hepatic mass (10 -> 20 cm in 6 weeks) extending into the R pleural cavity. We had a great discussion of possible liver lesions summarized below and are awaiting the pathology review from OSH biopsy. Given the rapid growth we discussed the possibility of a vascular component to this mass in addition to infectious causes (s/p biopsy) vs. lymphoma of the liver. Keep us updated!!

Key Pearls

  1. In the majority of patients (as Dr. Ostroff was alluding to), a proper diagnosis can be made based on the characteristics on imaging modalities.
  2. The majority of lesions < 1.0 cm are benign. Benign liver lesions are found in more than 20% of the general population[1], including haemangioma (4%), focal nodular hyperplasia (FNH, 0.4%) and hepatic adenomas (0.004%).
  3. Liver mets in a normal liver usually come from colon, stomach, lung and prostate. Importantly, mets are a rare finding in a cirrhotic liver.

Liver Lesions

Benign

 

Risk Factors Symptoms Dx Tx
Hepatic hemangioma Most common liver lesion; F>M Rarely causes pain US – well-circumscribed; peripheral enhancement None; no risk of bleed or CA
Cyst 5% of individuals; F>M Asx Rapid arterial uptake; hypodense lesions None
Focal Nodular Hyperplasia Hyperplastic growth around a preexisting arterial malformation Asx Solitary lesion None
 

Hepatic adenoma

Uncommon; M>F RUQ pain; palpable mass; fevers leukocytosis US or CT – no uptake with contrast Surgery (emergent if converts to bleeding)
Pyogenic Liver Abscess Ass w/ biliary stenting or acute ascending cholangitis; higher risk in DM Fevers, tender liver, leukocytosis Aspirate and Cx; US or CT; loculated single or multiple rim enhancement 4-6 weeks of abx

 

+/- Perc drainage

Amebic liver abscess Amoebiasis; endemic to Mexico Fevers, tender liver, leukocytosis Can’t culture; serology & empiric abx; Halo sign on CT “rim enhancement” Cholestatic LFTs; r/o IgG Echinococcal Metronidazole

 

If persistent then drain

Echinococcal cyst Ingestion of tapeworm eggs (fecal-oral); infected dogs/livestock If cysts rupture -> 2° echinococcis or anaphylactic shock Cholesttaic LFTs; eosinophilia; Test for IgG echinococcal Mebendazole

 

Avoid puncturing cyst

 

 

Malignant Risk Factors Symptoms Dx Tx
HCC Cirrhotics (EtoH, HBV, HCV, NASH…) Wt. loss, RUQ discomfort; HSM; jaundice; ascites – U/S or CT

-AFP – trend & prognosticate; -CEA (non-specific)

Resection, transplant, chemo
Biliary Tract Cancer CA of GB or intra-or extra hepatic biliary tract Abd pain, biliary obstruction, LFTs abnl Intrahepatic Ca; solid mass withing liver, Extra-hepatic: duct dilation, rim-enhancing (unlike HCC) Surgical resection, but often too large, embolization + chemo
Liver mets 20:1 more common than HCC, but uncommon in cirrhotic livers

-CRC, gastric, pancrearic, neuroendocrine

Looks for rise in CEA or LFTs in pt with hx of CRC or other CA CT or U/S Resection is confined to one lobe; radioablation
Heptic angiosarcoma 2% of primary liver cancers; Abd pain, weakness, wt loss, HSM, jaundice, CHF 31%; hepatic failure; intra-abd bleeding LFTs abnl

Ateriography “vascular lakes”

Mean life expectancy 6 months +/- chemo therapy or surgery
Lymphoma   B-symptoms, weight loss, fevers, RUQ abd pain Multiple, fast growing lesions Chemotherapy

 

References: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2710776/