UMEM Educational Pearls - Critical Care

Background: Conventional medical wisdom long held that patients with pneumothorax (PTX) who require positive pressure ventilation (PPV) should undergo tube thoracostomy to prevent enlarging or tension pneumothorax, even if otherwise they would be managed expectantly.¹

• Small retrospective and observational studies have demonstrated safety to an observational approach for both occult (only detectable on CT) and larger PTXs even in patients requiring noninvasive or invasive mechanical ventilation, whether traumatic/iatrogenic or spontaneous.^2-6
• The Western Trauma Association recently released a guideline for the management of traumatic PTX, which includes observation with 6-hour follow up CXR for patients with small (<20% aka <2cm from chest wall on CXR or <35 mm on CT scan) hemodynamically stable pneumothoraces, even if mechanical ventilation is required.⁷
  • They note a 10% subsequent failure rate (i.e. chest tube requirement) with no difference between patients who do or do not undergo PPV. 
• The OPTICC trial, found however, that while the rate of respiratory distress development was not different between those randomized to observation vs initial chest tube management, there was an increase from a 25% chest tube requirement in the obs group to a 40% failure rate in patients requiring >4 days of mechanical ventilation.⁸ 

Bottom Line: The cardiopulmonar-ily stable patient with small PTX doesn’t need empiric tube thoracostomy simply because they’re receiving positive pressure ventilation. If you are unlucky enough to still have them in your ED at day 5 in these COVID times, provide closer monitoring as the observation failure rate may increase dramatically around this time.

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   There are several well known medications that we tend to give by default during cardiac arrests.  It seems like for each of them, every few years someone does an RCT to see if they really help anybody, and we're all disappointed by what they find.  Well... prepare to be disappointed again, I'm afraid.

   These Danish authors randomized 391 patients in cardiac arrest to either calcium or saline (given IV or IO).  They gave 2 doses of either calcium chloride or saline, with the first dose being along with the first epi dose.  Primary outcome was ROSC.  They also looked at modified Rankin at 30 and 90 days.

  The trial was stopped early for harm.  Now, we all know the dangers of interpreting studies that were stopped early, but this doesn't look great for calcium.  19% of the calcium group had ROSC compared to 27% of the saline group (p = 0.09).  Percentage of patients alive, and with favorable mRS at 30 days also both favored the saline group (although also not statistically significantly).  By the way, of the patients who had calcium levels sent, 74% in the calcium group, vs 2% in the saline group, were hypercalcemic.  Whether that had anything to do with the outcome, we may never know.

Bottom Line:  Is this saying that calcium hurts patients in cardiac arrest?  Maybe... but I don't think this is high quality enough data to draw that conclusion.  At the very least, however, just giving everyone in arrest calcium is probably not terribly helpful.  If you have a reason to give it (known severe hypocalcemia, recent parathyroid surgery, suspected hyperkalemia, etc) then go for it, otherwise you can probably focus your resus on more important things.

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The BOUGIE Trial

• More than 1 million patients undergo endotracheal intubation each year in the US.
• Up to 20% of intubations fail on the first attempt, thereby increasing the risk of adverse outcome.
• Over the past several years, many have become comfortable using the bougie as a rescue device when the first attempt at intubation fails with an endotracheal tube with stylet.
• In contrast to its use as a rescue device, should the bougie be used during the first attempt rather than an endotracheal tube with a malleable stylet?
• The BOUGIE Trial compared the effect of using the bougie to an endotracheal tube with stylet on first attempt success in critically ill patients.
• The trial enrolled 1106 patients in 7 EDs and 8 ICUs at 11 hospitals.
• The primary outcome of first pass success was not statistically different between those randomized to bougie and those randomized to endotracheal tube with stylet for the first attempt at intubation.. 
• Though the trial did not find a statistical difference in first pass success rates, the bougie remains an important device in our management of the critically ill airway.

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When we initiate the sepsis bundle in the ED for patients with suspected sepsis, what probability that those patients who received broad spectrum antibiotics in the ED would have bacterial infection.

This study (Shappell et al) provides us with a glimpse of those number.

Settings: Retrospective study of adults presenting to 4 EDs in Massachusetts.

Patients: patients with suspected serious bacterial infection in ED, defined as blood cultures and initiation of at least one broad spectrum antibiotics.  Random selection of 75 patients per hospital.

Patients were categorized in 4 groups:

• Definite bacterial infection: clinical syndrome, pathologic diagnosis of infection (positive cultures from blood, urine; pus; radiographic evidence of abscess, consolidations in lungs)
• Likely bacterial infection: not meeting criteria for definite infection, but having a compatible clinical syndrome responsive to antibiotics and no clear etiology or reason for clinical improvement.
• Unlikely bacterial infection: clinical syndrome consistent with infection, but an alternate diagnosis is more likely.
• Definitely no bacterial infection: there was clear non-infectious diagnosis and no evidence of concurrent bacterial process.

Outcome: Prevalence of each category.

Study Results: 300 patients who received broad spectrum antibiotics.

1. Prevalence of bacterial infection:
   1. 81 (27%) had definite bacterial infection
   2. 104 (34.7%) had likely bacterial infection
   3. 55 (18.3%) had unlikely bacterial infection
   4. 49 (16.3%) with definitely no bacterial infection
2. For 96 patients with suspicion of sepsis vs. the rest of the cohort (P = 0.36)
   1. Definite 42.7%
   2. Likely 29.2%
   3. Unlikely 16.7%
   4. Definitely no 11.5%

       3. For patients who were admitted to the ICU (P = 0.26)

  a.   Definite 16.5%

                b.   Likely 8.6%

  c.   Unlikely 16.4%

                d.   Definitely no 20.4%

4. Source of infection

5.  Definite/Likely bacterial infection
   1. GU = 69 (35%)
   2. Respiratory = 48 (24.4%)
   3. Skin or soft tissue = 45 (22.8%)
   4. Bacteremia or endovascular = 42 (21.3%)
   5. Abdominal = 24 (12.2%) 
6. Unlikely/definitely not bacterial infection

1. Viral = 27%
2. Volume overload/cardiac disease = 10%
3. Hypovolemia = 8%

Discussion:

1. Slightly more than half of the patient we covered with broad spectrum antibiotics would have definitely or likely bacterial infection.
2. This study agreed with previous studies (2), which suggested that for patients treated prophylactically for sepsis, 13% had a “none” likelihood, 30% of only "possible" likelihood for bacterial infection.
3. The study highlighted that it was not easy for Emergency clinicians to recognize bacterial infection when we operate on a limited source of information and a limited timeline (think about the bundle of sepsis).
4. However, overtreatment is also bad, so we just need to be cognizant.

Conclusion:

Approximately 30% of patients who had blood cultures drawn and received broad spectrum antibiotics in ED have low likelihood of bacterial infection.

Reference:

1. Shappell CN, Klompas M, Ochoa A, Rhee C; CDC Prevention Epicenters Program. Likelihood of Bacterial Infection in Patients Treated With Broad-Spectrum IV Antibiotics in the Emergency Department. Crit Care Med. 2021 Nov 1;49(11):e1144-e1150. doi: 10.1097/CCM.0000000000005090. PMID: 33967206; PMCID: PMC8516665.

2. Klein Klouwenberg PM, Cremer OL, van Vught LA, Ong DS, Frencken JF, Schultz MJ, Bonten MJ, van der Poll T. Likelihood of infection in patients with presumed sepsis at the time of intensive care unit admission: a cohort study. Crit Care. 2015 Sep 7;19(1):319. doi: 10.1186/s13054-015-1035-1. PMID: 26346055; PMCID: PMC4562354.

Clinical Pearls for Variceal Hemorrhage

-lower mortality with “restrictive” (Hgb 7-9 g/dL) rather than liberal strategy

• although you should c/w your blood resuscitation according to hemodynamics

-antibiotic “prophylaxis” reduces mortality

• use ceftriaxone rather than quinolone 2/2 increasing resistance

-no need to correct INR with FFP

• FFP transfusions may actually be associated with worse outcomes (e.g. inc’d mortality)

-vasoactives (i.e. octreotide, somatostatin, terlipressin) alone may actually control bleeding

-for your ICU boarders...if persistent or severe rebleeding (despite endoscopic therapy), rescue TIPS is therapy of choice (call IR)

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Myocarditis is a potentially fatal inflammatory disorder of the heart. Viral infection is the most common cause but can also result from toxic, autoimmune, or other infectious etiologies. Complications include life-threatening dysrhythmias, heart failure, and fulminant myocarditis. Typically affects young patients (20-50 years old).

• Diagnosis can be challenging. Presentation can range from nonspecific symptoms and normal hemodynamics to cardiogenic shock.
• Dyspnea was found to be the most common presenting symptom in one study
• Other symptoms include fever, malaise, chest pain, palpitations, fatigue, nausea, vomiting
• Consider the diagnosis in young patient with suspected sepsis but worsens with IV fluids with signs of volume overload
• Initial assessment should include ECG, CBC, CMP, inflammatory markers, cardiac biomarkers, CXR. Obtaining an echo is important. Perform POCUS to assess for global hypokinesis, reduced EF, wall motion abnormalities, pericardial effusion, B-lines.

ED management pearls

• Initiate vasopressors and inotropic support if hemodynamically unstable: norepinephrine + inotropic agent (e.g. milrinone, dobutamine) is recommended. In a few studies, epinephrine was associated with increased mortality when used in cardiogenic shock.
• Diurese if evidence of volume overload
• NIPPV or intubation if respiratory failure
• Avoid NSAIDs which may worsen mortality
• Consider mechanical circulatory support (e.g. ECMO, IABP, VAD) in refractory hypotension despite appropriate medical therapy

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Fever has long been understood to be associated with worse outcomes in patients post-cardiac arrest. Whether ascribing to the goal of 33-34°C, 36°C, or simply <38°C, close monitoring and management of core temperatures are a tenet of post-cardiac arrest care.

A recently published study compared the effectiveness of several methods in maintaining temperatures <38°C…

• Both ICHA and OHCA, shockable and unshockable, nontraumatic arrests
• Single center retrospective cohort study looking at 1/2012 – 9/2015
• Treatment and temperatures over first 48 hours

Results:

Maintenance of temp <38°C:

• Antipyretics only group: 57.7% 
• Invasive cooling by intravascular catheter +/- antipyretics:  82.1%

Mean change in temp from baseline:

• Antipyretics only: +1.1°C
• Intravascular alone: -3.4°C
• Antipyretics + Intravascular cooling: -5.2°C

Limitations:

• Varied range of antipyretic dosing per body weight
• No mention of noninvasive cooling methods (cooling pads, ice packs, etc.)
• Patients w/ intravascular cooling likely getting more aggressive care in general
• Not powered for clinical outcomes assessment

Bottom Line:

• Antipyretics alone greatly ineffective at preventing fever 
• Even with invasive cooling -- not meeting goal 18% of the time
• With longer ED boarding times nationwide, we must pay active attention to body temperature management and not assume that that we can set it and forget it, even with techniques as invasive as intravascular cooling.

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The debate around post-arrest management recently has revolved around whether therapeutic hypothermia should go cold, or LESS cold.  But what if we went MORE cold?  While recent TTM trials have compared temps such as 33 to 36 and 33 to 37.5 or less, a recent trial called CAPITAL CHILL looked at 34C vs 31C.  There is a solid physiologic basis for cooling post-arrest patients, so do they do better if we lower their temp even further?  Maybe we're not going cold enough with 33?

Bottom Line: No, 31C is not better than 34C for post-arrest patients.  This study compared death and poor neurologic outcome at 180 days with 31 and 34C targets for post-arrest patients, and found no difference (in fact the 31C group did slightly, but not significantly, worse on the primary outcome, and worse on a few secondary outcomes).  

While debate remains for 33 vs 36 vs afebrile, the literature does not currently support consideration of temps below 33.  

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Initial Mechanical Ventilation Settings for the Intubated Asthmatic

• Approximately 2% of adult patients who present with an acute asthma exacerbation will require intubation and mechanical ventilation.
• It is critical to provide the intubated asthmatic with sufficient time for exhalation.
• Initial recommended settings for mechanical ventilation include:
  • Tidal volume: 6-8 ml/kg ideal body weight
  • Respiratory rate: 6-10 breaths per minute
  • PEEP: 0-5 cm H2O
  • Inspiratory flow rate: 80-120 L/min
• Permissive hypercapnea is tolerated to a pH of approximately 7.15

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Background: A cornerstone of therapy for cardiogenic shock is inotropic support with medications including dobutamine, epinephrine and milrinone.  Few studies have examined these head-to-head and between dobutamine and milrinone (including only one RCT of 36 patients)

The investigators conducted a RCT of milrinone versus dobutamine for cardiogenic shock in a single quaternary care center cardiac ICU.

Inclusion: Patients over 18 with cardiogenic shock (largely clinical determination)

Exclusion: Out-of-hospital cardiac arrest, pregnancy, prior initiation of dobutamine or milrinone, or physician discretion.

Methods: 1:1 randomization stratified by affected ventricle (LV vs RV). Primary outcome was a composite of in-hospital death, resuscitated cardiac arrest, cardiac transplant, mechanical circulator support, nonfatal MI, TIA, stroke, or renal replacement therapy. Powered to detect a 20% improvement in this measure in the milrinone group (192 pts).

Results:  192 patients enrolled (96 in each arm). Average age was 70, 36% female, 90% LV dysfunction, 67% ischemic disease, 33% non-ischemic, average LVEF 25%, 68% on vasopressors. ICU admission to randomization was 23+/-92.6h for dobutamine and 17.6+/-50.6h for milrinone arms. 80% were SCAI class C shock.

Primary outcome for milrinone 49% versus dobutamine 54%, HR 0.9(0.69-1.19), p=0.47, death was the primary driver of the composite (37% vs 43%).  Arrythmia requiring intervention was not different between groups (50% vs 46%). No difference in a host of other endpoints including AKI (92% vs 90%), RRT (22% vs 17%), HR, lactate, MAP, UOP, and creatinine.

Discussion: No significant differences observed in outcomes for patients with cardiogenic shock randomized to milrinone versus dobutamine.  The trial addressed an important clinical question for management of cardiogenic shock and relied largely on clinical diagnosis for inclusion and likely reflected a somewhat broad range of patients. The trial was too small given observed treatment effects and few patients with RV failure. Notably, similar rates of adverse events observed in each group.  

Many limitations for practice including a single specialized ICU setting, limited information on events leading to ICU admission including invasive or medical interventions during the index visit and no long term follow-up.  Time to randomization, exclusion of cardiac arrest, and lack of reporting pre-ICU setting (ED, floor, cath lab) also significantly limits utility in an emergency setting.

Bottom Line: 192 patient single-center cardiac ICU-based trial shows no difference in composite or secondary endpoints between milrinone and dobutamine for cardiogenic shock, adds to a body of very limited RCTs comparing inotropes in cardiogenic shock but provides no practice changing evidence.

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Intubation considerations

• Use large ET tube (at least 8.0 if possible): minimizes airway resistance, facilitates aggressive pulmonary toilet and bronchoscopy if needed
• Consider using ketamine as induction agent as it has bronchodilator properties and can maintain blood pressure
• Appropriate choices for initial sedation includes propofol, fentanyl, and ketamine

Vent management strategies

• No overall outcome differences between volume vs pressure control modes. Volume control has been recommended as initial mode due to familiarity and ensures your set tidal volume will be delivered.
• Goal is to minimize autoPEEP, which occurs from incomplete exhalation prior to initiation of next inhaled breath. This can be achieved by adjusting a few vent settings: decreasing RR, decreasing I:E ratio, decreasing inspiratory time, or increasing inspiratory flow rate. Allow for permissive hypercapnia, pH >7.2 has been advocated though precise target is unknown.
• If patient becomes hemodynamically unstable, consider first disconnecting the ventilator from the ET tube and manually decompress the chest to facilitate exhalation.
• Peak inspiratory pressures are expected to be high in the acute severe asthmatic. More important is to keep plateau pressures <30 cm H₂O to prevent lung injury.
• Don't forget to continue asthma-directed therapy. Administer albuterol via in-line nebulization unit of the vent.

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Management of Intermediate-High Risk PE Patients

• Though there are varying definitions, intermediate-high risk patients with a PE are generally defined as those who are hemodynamically stable, have radiographic or laboratory evidence of right heart strain, and an elevated PE risk score.
• A few key management pearls include:
  • Be judicious with IVFs to avoid worsening septal shift and fruther decreases in LV cardiac output.
  • Consider dobutamine for severe RV dysfunction.
  • Administer norepinephrine, if needed, to achieve a MAP of 65 mm Hg.
  • Avoid initiation of positive pressure ventilation, if possible. 
  • If intubation is needed for clinical deterioriation avoid propofol for RSI.  Propofol has been associated with increased mortality in this patient population.

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Background: Interest in moving to balanced fluid administration has grown after publication of the SPLIT trial and SALT-ED/SMART trials, which showed respectively evidence of benefit to balanced crystalloid over normal saline on mortality and major adverse kidney events at 30 days.

Population/Intervention: The BaSICs trial is an RCT in 75 ICUs in Brazil, testing P-Lyte versus NS (with each arm getting two different infusion rates that were analyzed as a separate trial) for volume administration per protocol.

--10,520 ICU patients requiring fluid expansion, expected ICU stay >1 day, and 1 additional risk factor for AKI (age>65, hypotension, sepsis, MV, NIV, oliguria, elevated creatinine, cirrhosis, or acute liver failure).

--Exclusions: severe dysnatremia, expected RRT within 6 hours, expected death.

--Average age was 61, with a SOFA score of 4, and 48% on were elective surgical admissions.

Outcome:

--No difference in 90 days mortality (P-Lyte 26.4% v NS 27.2, aHR p=0.47), AKI or RRT out to 7-days, or in duration of MV, ICU LOS or hospital LOS

--Median study fluid by day 3 was 2.9L in each group

--Higher neurological SOFA score observed in P-Lyte group

--Higher mortality seen with P-Lyte in TBI subgroup (P-Lyte 31.3% vs NS 21.1%, p=0.02)

Discussion:

--Adds contrasting negative data to previous large positive RCTs showing benefit of balanaced fluids

--Expect further reanalysis/metanalysis of BSS versus NS trials

--Signal for harm in TBI pts with P-Lyte correlates with SMART point estimates that were not significant

--Compared to SMART trial population BaSICs had: 2x higher mortality, more planned surgery, received about 1L more study fluid in the first 3ds

Takeaway:

--Balanced crystalloid versus normal saline debate will continue considering this large negative trial

--Signal for possible harm in TBI population with balanced crystalloids compared to normal saline

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Background: SOFA score has been used as a predictor for poor outcomes in patients with sepsis.  However, the original SOFA score utilizes PaO2/FiO2 ratio to calculate the SOFA’s respiratory component.  When there are no ABG, thus no PaO2, we have to convert patients’ spO2 to PaO2, and the amount of oxygen support to FiO2 (for example, 2 liters of oxygen via nasal cannula = 0.27).  This is cumbersome.

Objective: This study assessed whether spO2 can be used instead of PaO2/FiO2 ratio for SOFA’s respiratory score.

Settings: 8 hospitals across Sweden and Canada

Patients: Adults with sepsis.  19396 patients were included for the derivation group while there were 10586 patients for the validation cohort.

Study Results:

• When PaO2 was not measured, assigning respiratory SOFA score of 1 for spO2 94% and respiratory SOFA score of 2 for spO2 < 90% had good discriminatory capability.
• The AUROC with SOFA score using spO2 threshold as above was 0.783 (0.767-0.798), which was slightly increased from the model using previous methods to calculate respiratory SOFA scores (0.781 [0.765-0.796]).

Discussion:

• For Emergency Medicine, using a cut-off threshold  for spO2 of 94% (respiratory SOFA of 1) and 90% (respiratory SOFA of 2) can simplify how to calculate the SOFA score.  
• It’s also simpler to do research when we collect SOFA score retrospectively.

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Background:

There are also no clear guidelines regarding how fast fluid boluses should be administered, and there has been debate about whether different infusion rates could lead to different outcomes in patients receiving intravenous fluid (IVF) boluses (i.e. fast infusions may cause more third spacing due to the rapidity of the expansion of the intravascular space compared to fluid administered more slowly). A recent study compared IVF infusion rates in ICU patients.

-- Unblinded, randomized

-- 10,520 patients clinically requiring a fluid challenge, from 75 ICUs in Brazil

-- Infusion rate 333 mL/hr vs 999 mL/hr

   * (Trial also compared plasmalyte vs 0.9% saline, analyzed in separate study)

-- Some notable exclusion criteria: severe hypo/hypernatremia, AKI or expected to need RRT 6 hrs after admission

--Other caveats:

   * Faster infusion rates allowed at physician discretion in patients with active bleeding or severe      hypotension (SBP < 80 or MAP < 50 mmHg); patient was returned to assigned rate after condition resolved

   * Almost 1/2 the patients received at least 1L of IVF in 24 hours prior to enrollment

-- Results: No sig difference in 90-day survival, use of RRT, AKI, mechanical ventilator free days, ICU/hospital mortality/LOS 

Bottom Line: There is not yet compelling evidence that there are differences in patient outcomes in patients receiving fluid boluses given at 333 cc/hr vs. 999 cc/hr.

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Approximately 15,000 children experience an in hospital cardiac arrest (IHCA) with little improvement in outcomes over the last two decades. During that time, epinephrine has been the constant basis for resuscitation of these patients. Current recommendations by the AHA recommend bolus dosing of epinephrine every 3-5 minutes in a pediatric cardiac arrest. Animal studies suggest that more frequent dosing of epinephrine may be beneficial. 

This was a retrospective study of 125 pediatric IHCAs with 33 receiving “frequent epinephrine” interval (≤2 minutes). Pediatric CPC score 1-2 or no change from baseline was used as primary outcome to reflect favorable neurologic outcome, with frequent dosing associated with better outcome (aOR 2.56, 95%CI 1.07 to 6.14). Change in diastolic blood pressure was greater after the second dose of epinephrine among patients who received frequent epinephrine (median [IQR] 6.3 [4.1, 16.9] vs. 0.13 [-2.3, 1.9] mmHg, p=0.034). 

This study is subject to all sorts of confounding and should be studied more rigorously, but suggests that more frequent dosing for pediatric IHCA may be of benefit.

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Resuscitative thoracotomy is a dramatic and heroic procedure used in the emergency department in an attempt to resuscitate a patient in arrest due to trauma. There are a few techniques commonly used, but due to the extreme nature of the procedure no prior randomized controlled trials (RCTs) have been done.

The modified clamshell thoracotomy (MCT) is a technique in which the standard left anterolateral thoracotomy (LAT) is extended across the sternum, but does not involve surgical opening of the right chest. The MCT allows for increased visualization of the mediastinum and thoracic cavity structures. 

Sixteen Emergency trained physicians (approximately half attending and half senior residents) from a level 1 trauma center underwent didactic and skill based training on both the MCT and LAT techniques using fresh, human cadavers. Following training they were randomized based on order of intervention, performing both techniques.

Their thoracotomies were assessed by a board certified surgeon and “success” was determined based on the complete delivery of the heart and cross clamping of the descending aorta. 

Primary outcome: time to successful completion of procedure

Secondary outcomes: successful delivery of the heart from the pericardial sac (as well as time to delivery),  cross clamping of the aorta (and time to clamping),  procedural completion and number of iatrogenic injuries. 

Overall, there was no statistical difference in primary outcome or successful completion between the MCT compared to the LAT (67% vs. 40%). However, 100% of the LAT resulted in some form of iatrogenic injury (rib fractures, lacerations of the diaphragm,/esophagus/heart/lung) compared to 67% of the MCT technique. There was no associated difference in success when previous experience (attending vs. senior resident) were compared. Lastly, MCT was the favored technique of the majority of the study subjects. 

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The much anticipated REMAP-CAP trial was epublished ahead of print July 12th in Intensive Care Medicine.  It was an RCT investigating four antiviral strategies in critically ill adults with COVID-19: lopinavir-ritonavir, hydroxychloroquine, a combination of the two, and no antiviral therapy (control group).  

Despite the hype around protease inhibitors, hydroxychloroquine, and other unproven therapies in COVID (lookin at you next, Ivermectin...), all three strategies had WORSE outcomes than placebo.  They all decreased organ-support-free days (all reaching statistical significance), which was the primary outcome.  They also all led to longer ICU time, longer time to hospital discharge, and reduced 90 day survival.  Not only does this study show no benefit, it shows fairly convincing signs of harm to these therapies.

Bottom Line: Protease inhibitors (e.g. lopinavir-ritonavir) and hydroxychloroquine are unproven therapies for critical COVID-19 infection, and are not recommended.  Providers should focus on interventions with demonstrated benefit, most notably steroids and good supportive/critical care.  

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Hemophagocytic Lymphohistiocytosis (HLH)

• HLH is a hematologic disorder that results from overactivation of the immune response (macrophages and cytotoxic T cells).
• HLH is often underrecognized and has a mortality that can be as high as 75%.
• Secondary HLH is most commonly associated with infection (sepsis), malignancy (lymphoma), and autoimmune disorders (SLE, RA).
• Hallmark features of HLH include fever, splenomegaly, hepatomegaly, cytopenias, coagulopathy, elevated ferritin, elevated triglycerides, and decreased fibrinogen levels.
• ED resuscitation of patients with suspected HLH includes Hematology consultation, treatment of the underlying disorder (infection), and potentially corticosteroids and chemotherapeutic agents.

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