UMEM Educational Pearls

Category: Administration

Title: Personal growth, not goal setting

Keywords: Personal growth. (PubMed Search)

Posted: 12/31/2023 by Robert Flint, MD (Updated: 4/19/2024)
Click here to contact Robert Flint, MD

As the calendar flips to a new year, consider not setting goals or resolutions. Studies show unmet goals or having too many half finished projects leads to increased stress, anxiety and depression. Instead, consider approaching the new year looking for growth, introspection, and  striving to achieve excellence.  Understanding the why and what motivates you will lead to the correct what and how. Here are some questions to get you thinking about the why.  May your New Year be filled with growth and excellence!  



For the agitated geriatric patient, if verbal deescalation, distraction, and providing a safe quiet area do not work and you require chemical sedation use oral antipsychotics first.  Follow this with IV or IM antipsychotics. Avoid benzodiazepines due to often worsening delirium or respiratory depression. For dosing, start low and go slow.

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NEXUS criteria for blunt chest trauma patients who are over 14 years old, not intubated:

  • >60 years old

  • rapid deceleration defined as fall > 6 meters or motor vehicle crash >64 km/hour

  • chest pain

  • intoxication

  • abnormal alertness or mental status

  • distracting painful injury

  • tenderness to chest wall palpation

    If abnormal chest X-Ray proceed to chest CT.  Negative predictive value of 99.9% excluding major injury.

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Estimating the size of knee effusions

  • Small effusions (5 to 10 mL) will fill the peripatellar dimples with the knees extended and quadriceps relaxed.
  • The ballottement sign is positive when there is at least 10 to 15 mL of intraarticular fluid.
  • Large effusions (20 to 30 mL) fill the suprapatellar space. 

While this size range is typically easily detectable on exam. This may not apply to patients who are either very muscular or obese.

If the detection of a small to moderate sized effusion would change patient management 

  • For example, ones confidence to successfully drain a knee effusion knee based on a physical exam

Consider ultrasound: 

As compared to MRI (sensitivity of 81.3 % and a specificity of 100 %)

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Category: EMS

Title: Does EMS diversion impact the number of ambulances that arrive at a particular facility?

Keywords: EMS, red, yellow, divert, capacity (PubMed Search)

Posted: 12/20/2023 by Jenny Guyther, MD (Updated: 4/19/2024)
Click here to contact Jenny Guyther, MD

US hospitals have traditionally been concerned that without an ambulance diversion protocol that they would be overrun with EMS arrivals.  EMS had been concerned that without diversion there would be extended wait times at the hospital.  This study looked at EMS arrivals one year (2021) before the elimination of diversion and compared the number to one year after diversion elimination (2022).  

This study of a single level 1 trauma center showed that there was NO difference between the number of EMS arrivals per day (84 vs 83, p = 0.08), time to room for ESI 2 patients, time to head CT in acute stroke patients OR ambulance turn around time (16 min vs 17 min, p = 0.15).

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Category: Critical Care

Title: Acute-On-Chronic Liver Failure

Posted: 12/19/2023 by Mike Winters, MD (Updated: 4/19/2024)
Click here to contact Mike Winters, MD

Acute-On-Chronic Liver Failure

  • Acute-on-chronic liver failure (ACLF) is defined as an acute deterioration of liver function in a patient with cirrhosis that is associated with organ failure and has high short-term mortality.
  • Key extrahepatic organ failures in ACLF include the renal, CNS, respiratory, circulatory, and coagulation systems.
  • With respect to CNS failure in ACLF:
    • Hepatic encephalopathy (HE) is the most common manifestation
    • A normal ammonia level makes HE unlikely
    • Benzodiazepines should be avoided
    • Primary triggers for HE include infection, GIB, and aggressive diuresis
    • Treatment of HE primarily consists of lactulose and rifaximin

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Category: Ultrasound

Title: How to Perform a Transvaginal Ultrasound for OB

Keywords: Obstetrics; POCUS; Transvaginal Ultrasound (PubMed Search)

Posted: 12/18/2023 by Alexis Salerno, MD
Click here to contact Alexis Salerno, MD

By performing a Point-of-Care Transvaginal Ultrasound (TVUS), we can decrease length of stay for patients with early pregnancy. Moreover, if an ectopic pregnancy is identified, we can decrease time to the OR for these patients. 

Begin by discussing the exam with the patient and ensuring they have emptied their bladder.  Apply a probe cover and add sterile lubricant to the outside of the probe tip. You can save time by performing a TVUS immediately after the pelvic speculum exam for swab collection.

  • Obtain a Sagittal View of the Uterus:

Gently introduce the transducer with the marker upward, directed towards the ceiling. As you slowly advance, the uterus will be visualized in a sagittal orientation. Fan through the uterus by moving the probe handle left and right.

Image From: doi: 10.1016/j.emc.2022.12.006.

  • Obtain a Coronal View of the Uterus:

Rotate the transducer so that the marker is directed towards the patient's right side. Fan through the uterus by lifting the probe handle up and down. 

Image From: doi: 10.1016/j.emc.2022.12.006.

  • Perform Measurements:

If a gestational sac is found, you should measure the gestational age and if present, fetal heart rate. 

  • Evaluate the Adnexa:

Tilt the transducer towards the patient's left or right side to visualize the adnexa. The adnexa will be located medially to the iliac vessels. 

  • End the Exam:

Remove the transducer and follow your department protocol for high level disinfection.

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This commentary offers another reminder that there is significant bias in which trauma patients receive alcohol testing when that decision is made on a case by case basis. Age, sex, socioeconomic, race, injury pattern, all have been shown to influence provider ordering. Trauma systems should have pre-defined ordering criteria to eliminate this bias. The importance of gathering this testing information is to provide intervention and treatment to those in need. First we have to identify all patients in need.

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Urinary tract infection (UTI) is the leading cause of fever without a source in infants younger than 3 months.  This data was collected from patients who presented to the emergency department with fever without a source over a 16 year period.  Out of 2850 patients, 20.8% were diagnosed with a UTI, the majority of which grew E coli.  Of those patients who were diagnosed with UTI, these patients were more likely to have a history of renal/GU problems, have a fever of at least 39C (38% vs 29%) or poor feeding (13% vs 8.7%).  However, 48% had none of these risk factors.  Also 6.1% of patients with a febrile UTI had another invasive bacterial infection.  These patients were more likely to be < 1 month, be "irritable" per parents and have an elevated procalcitonin and CRP.  

Bottom line:  A lack of risk factors can not exclude a UTI in febrile infants < 3 months.  A diagnosis of UTI also does not definitively exclude an additional invasive bacterial infection in a subset of these children.

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Bottom Line: Droperidol is an effective alternative to haloperidol in the treatment of gastroparesis although most patients will also receive prokinetic agents as well such as metoclopramide. It may also have some analgesic benefit.

Prior studies have demonstrated the efficacy and safety of haloperidol in the management of gastroparesis. A recent retrospective study was conducted to assess the impact of droperidol as it is an effective antiemetic similar to haloperidol.

This study enrolled 233 patients.  Visits were matched with their most recent ED visit > 7 prior where droperidol was not administered. 

Most patients were female, 51% African American, and the median age was 40.  Doses ranged from 0.625 mg – 2.5 mg with the most common dose being 1.25 mg. 

Results:

  • 46% of the droperidol visits received opioids compared to 60% when droperidol was not given (p=0.004)
  • Droperidol was noted to reduce pain scores from 9 (IQR 7-10) to 5 (IQR 0-8) (p=0.0001)
  • Droperidol visits were prescribed fewer antiemetic agents 60% vs 73% (p=0.0045)
  • There was no difference in the use of prokinetic agents (metoclopramide)
  • No difference in ED or hospital LOS or admission rates (~30%), cost, or adverse events

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Bottom line: In the 2023 updated Clinical Practice Guideline, the American Burn Association recommends 2ml/kg/%TBSA (for burns >20% TBSA)as initial starting point for fluid administration in the first 48 hours, guided by clinical factors with consideration of supplemental albumin to limit fluid administration. Fresh frozen plasma should be considered in the context of a clinical trial.  Vitamin C and advanced hemodynamic monitoring are not recommended as they have not demonstrated improved outcomes.

Summary: Burn care has a paucity of high-quality research about some of the fundamental questions for resuscitation. The American Burn Association since 2010 has endorsed fluid volumes for patients with >20% TBSA (i.e. those predicted to develop burn shock) from 2ml/kg/%TBSA to 4ml/kg/%TBSA as a starting point for fluid resuscitation. Further clinical studies since then have demonstrated that lower volumes of fluid targeting urine output and other physiological variables are effective without demonstrating clear improvement in patient centered outcomes.  Further adjuncts such as albumin or fresh frozen plasma have demonstrated reduced fluid administration but no improvement in patient-centered outcomes. While “fluid creep” is increasingly recognized, demonstrating benefits in clinical trials will likely remain elusive as overall practice continues to shift towards less fluids and the adjunctive use of colloid will likely continue to expand. In addition to ABA CPGs and resources, the Joint Trauma System also has several useful resources for burn care.

Sources:

https://doi.org/10.1093/jbcr/irad125

https://jts.health.mil/assets/docs/cpgs/Burn_Care_11_May_2016_ID12.pdf



Category: Trauma

Title: Morel-Lavallée Lessions

Keywords: soft tissue injury, trauma, (PubMed Search)

Posted: 12/10/2023 by Robert Flint, MD (Updated: 4/19/2024)
Click here to contact Robert Flint, MD

Here are three good resources to learn about a soft tissue injury seen in high velocity blunt trauma patients called Morel-Lavallee lessions.

“Morel Lavallee lesions are soft tissue injuries seen in high-velocity trauma and are usually associated with underlying fractures of the pelvis, acetabulum, or proximal femur. Often these injuries are not immediately diagnosed due to the distracting concomitant bony injuries. However, identification of such injuries is important as they may pose as an independent risk factor for surgical site infection. The clinical findings include soft tissue swelling, bruise/ ecchymosis, fluctuance, and compressibility in the swelling. The diagnosis is usually established on physical examination, however, radiological investigations including ultrasonography and CT might help. The management options include nonoperative treatment, percutaneous aspiration, and open debridement.” 1

“Morel-Lavallée lesions are often the result of skin and subcutaneous tissue quickly tearing away from the underlying fascia. This allows a range of fluids to fill the space in the form of hemolymphatic masses. The two most common sites are the prepatellar plate of the knee and the lateral fascia of the hip.” 2

“ML lesion is often undiagnosed during initial presentation of a trauma patient, and emergency physicians and trauma surgeons should be aware of the possibility of occurrence of this injury. MRI is the imaging modality of choice, and the presence or absence of a capsule is an important imaging finding that guides appropriate therapy. Early diagnosis and management will help prevent long-term morbidity and complications in these patients.”3

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Category: EMS

Title: There's a Doctor on Board! Physician Staffed EMS and Trauma Care in Japan

Keywords: EMS, trauma, emergency medical services, (PubMed Search)

Posted: 12/6/2023 by Ben Lawner, DO (Updated: 4/19/2024)
Click here to contact Ben Lawner, DO

BACKGROUND
 
EMS systems differ in staffing and composition. The Japanese model utilizes “doctor cars” which bring a physician and nurse to the scene of a critical patient encounter. Personnel on the “doctor cars” are able to perform advanced therapies such as REBOA, finger thoracostomy, and chest tube thoracostomy. As physician EMS fellowships continue to expand in the United States, it is helpful to examine the utility of physician response incorporated into prehospital emergency care. 

 
THE STUDY

A nationwide retrospective cohort study including over 370,000 patients examined the impact of Japan “doctor cars” upon in hospital survival. Doctor cars responded to 2361 trauma patients, and traditional Ground Emergency Medical Services (GEMS) units cared for 46,783 trauma patients.  The study’s primary outcome was survival to discharge.  

The adjusted odds ratio for survival was significantly higher in the exposure group served by the doctor cars. The study suggests that there may be a role for augmenting ground EMS personnel in the response to critical injuries. Via logistic regression, the study controlled for multiple other variables such as age, sex, prehospital vital signs, out of hospital time, and injury severity score (ISS).  

  • At hospitals caring for >50 trauma patients per year, the impact of doctor cars upon in hospital survival was not statistically significant 
  • Not surprisingly, patients cared for by the doctor car team had a longer time to hospital arrival 
  • Adult patients with higher ISS scores had a significant improvement in survival  

BOTTOM LINE
 
This study is far from definitive but contributes to a growing body of literature addressing how EMS physicians integrate into prehospital systems.

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Category: Critical Care

Title: To Start Or Not To Start Vasopressor????

Keywords: vasopressor, norepinephrine, timing, septic shock (PubMed Search)

Posted: 12/5/2023 by Quincy Tran, MD (Updated: 4/19/2024)
Click here to contact Quincy Tran, MD

Settings: systemic review and meta-analysis

Participants: 2 RCTs, 21 observational studies. Fifteen studies were published between 2020-2023.

There was a total of 25721 patients with septic shock

Outcome measurement: Primary outcome was short-term mortality (ICU, hospital, 28-day, 30-day). Secondary outcomes included ICU LOS, Hospital LOS, time to achieve MAP > 65 mm Hg,

Study Results:

Composite outcome of short term mortality

  • 20 studies and 17470 patients. Early initiation of vasopressors was associated with lower odds of short term mortality (OR 0.775, 95% CI 0.673-0.893, P<0.001, I2 = 68%).
  • Early initiation of norepinephrine was associated with lower odds of short term mortality (OR 0.656, 95% CI = 0.544 to 0.790, P <0.001, I2 = 57.2%)
  • Early initiation of vasopressin was also associated with lower odds of short term mortality (OR 0.685, 95% CI 0.558-0.840, P < 0.001, I2= 57%)

 Secondary outcome:

  • Early vasopressor group was associated with lower odds of RRT use (OR 0.796, 95% CI 0.654-0.968, P = 0.022, I2 = 0%)
  • Mean Serum lactate levels at 6 hours was similar in early vasopressor group (Mean Difference 0.218, 95% CI -0.642 to 1.079, P = 0.619).
  • However, mean serum lactate levels at 6 hours was lower in early norepinephrine subgroup (mean difference -0.489, 95% CI -0.863 to -0.115, P = 0.01).

Discussion:

  • This appears to be a hot topic. When our group did this topic in 2020, there were 8 or 9 studies. Since 2020, there has been a significant increase in the number of publications, although most publications were observation studies.
  • Early initiation of norepinephrine may reduce fluid overload, not by reducing fluid input, but by improving host inflammatory response, improving endothelial cell barrier stability.
  • Counter-intuitively, early vasopressor was also found to be associated with lower incidence of arrhythmia, which the authors attributed to shorter duration of vasopressors and lower total dosage.

Conclusion

More and more studies, although a RCT is still necessary, are showing that early initiation of vasopressor within 1-6 hours of septic shock would be more beneficial to patients with septic shock.

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Category: Vascular

Title: Aortic Root Measurement

Keywords: aortic aneurysm; point-of-care ultrasound; pocus; aortic dissection (PubMed Search)

Posted: 12/4/2023 by Alexis Salerno, MD (Updated: 4/19/2024)
Click here to contact Alexis Salerno, MD

Point-of-Care Ultrasound can help to identify signs of thoracic aortic dissection.

One view to help in your assessment is the Parasternal Long Axis View.

  • The aortic root should be in a 1:1:1 ratio with the left atrium and the right ventricle.
  • The aortic root should be less than 4 cm (4.5 cm considered aneurysmal)

To correctly measure the aortic root:

  • Measure at the Sinus of Valsalva
  • Measure during diastole (when the aortic valve is closed)
  • Measure leading edge to leading edge

Here is an example of an aortic root aneurysm: 

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A retrospective study of 2 years of data from 24 trauma centers looking at end tidal CO2 as a predictor of mortality in trauma patients found:

"A total of 1,324 patients were enrolled. ETCO2 was better in predicting mortality than shock index (SI) and systolic blood pressure (SBP).  Prehospital lowest ETCO2 , SBP , and SI  were all predictive of Mass Transfusion."

 

Another data point to consider when setting up trauma triage protocols and looking for patients who will require intensive interventions early. 

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The Bottom Line: Investigators studying the use of a pre-intubation checklist versus "usual care" found no differences in important outcomes such as oxygen saturation and first pass success. However, the study was conducted as a part of a larger study, was unblinded, and not well controlled. 

The investigators who conducted the multicenter CHECK UP trial, a study of head up intubation in ICU patients, reviewed the care of 262 ICU patients who were intubated. Some intubation attempts were guided by a pre-intubation checklist and some were not. The authors found no difference between the groups in lowest SPO2, number of intubation attempts, etc. However, the study was unblinded and largely observational. In many cases, the elements of a checklist had been incorporated into routine practice. 

Take Home Point: While the authors found no differences in outcomes, this study does little to prove or disprove the value of pre-intubation checklists. Not only was the study essentially uncontrolled, the untoward events being studied are unusual in the hands of experienced clinicians.

Comment: Pre-procedural checklists make intuitive sense to me. They help us to avoid cultural drift. I am certainly not ready to abandon the use of a pre-intubation checklist based upon this study

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Since Christmas is coming up, let's talk about Hemophilia A (factor VIII deficiency) and Hemophilia B (factor IX deficiency, also known as Christmas disease)

Deficiencies in Factors VIII and IX are the most common severe inherited bleeding disorders.

Pathophysiology: 

  • Factors VIII and IX are required for activation of factor X.
  • In patients with Hemophilia A (factor VIII deficiency) or Hemophilia B (factor IX deficiency, also known as Christmas disease), after an injury, clot formation is delayed. 
  • Inadequate thrombin generation leads to failure to form a tightly crosslinked fibrin clot to support platelet plug, which leads to easy bleeding.
  • Clot that is formed may be friable and rebleeding occurs during physiologic lysis of clots or with minimal new trauma

Clinical Manifestations:

  • 2% of neonates with hemophilia have intracranial hemorrhages
  • 30% of male infants with hemophilia bleed with circumcision
  • Continued bleeding from umbilical stump in neonate
  • In absence of positive family history (hemophilia has high rate of spontaneous mutation), hemophilia may go undiagnosed in a newborn
  • Easy bruising, intramuscular hematomas, and hemarthroses (hallmark for hemophilic bleeding) begin when child begins to cruise
  • Bleeding from minor traumatic lacerations of the mouth (e.g. torn frenulum) can persist for hours or days
  • Iliopsoas hemorrhage: patient may lose large volumes of bleed into the muscle, leading to hypovolemic shock, with only a vague complaint of area of referred pain in the groin. Hip is held in a flexed, internally rotated position, due to irritation of the iliopsoas.
    • Confirmed on CT or US
    • Clinically unable to extend hip
  • Hemarthrosis rare in patients with acquired hemophilia

Lab findings and diagnosis

  • Reduced levels of factor VIII or factor IX will cause higher PTT 
  • PTT is usually 2-3x upper limit of normal in patients with severe hemophilia.
  • Platelet count, bleeding time, prothrombin time, and thrombin time are all normal
  • If PTT is not corrected after administration of factor VIII or IX, an inhibitor may be present.
    • 25-35% of patients with hemophilia who received infusions of factor VIII or factor IX, a factor specific antibody may develop (inhibitor)

Genetics

  • Hemophilia occurs in 1:5000 males, with 85% having factor VIII deficiency and 10-15% having factor IX deficiency
  • No apparent racial predilection, appearing in all ethnic groups

 

Classification

  • Severe hemophilia: <1% activity of specific clotting factor and bleeding is often spontaneous
  • Moderate hemophilia: 1-5% activity and require mild

trauma to induce bleeding

  • Mild hemophilia: >5% activity and can go many years before diagnosis and usually require significant trauma to induce bleeding.

Treatment

  • Ask patient or family if they brought their dosing information with them or their factor replacement with them. In many cases, they have it!
  • For life-threatening or major hemorrhages, dose should aim to achieve levels of 100% activity
    • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
    • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
    • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
    • If you don’t have factor-specific products:
      • Hemophilia A
        • can give 1U cryoprecipitate (~80U of factor VIII) or try PCC (as it contains factors II, VII, IX, and X)
        • activated PCC (FEIBA) 75-100U/kg
      • Hemophilia B
        • FFP NO LONGER RECOMMENDED (volume of FFP required has high risk of volume overload)
        • Cryoprecipitate does NOT contain factor IX, so will not work.
  • For acute bleeding in patients with mild hemophilia A:
    • Can give DDAVP: increases factor VIII by 3-5x by encouraging release of endogenous factor VIII. Recommended dose: 0.3mcg/kg/dose IV
  • For mild bleeding:
    • TXA (clot stabilizer)
    • Desmopressin
    • Aminocaproic acid
  • If patient has inhibitors:
    • Hemophilia A: 
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    • Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

Summary:

  • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
  • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
  • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
  • Treatment if patient has no inhibitors:
    • Hemophilia A: 
      • Severe bleed: Give full dose factor XIII (50U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor XIII replacement (25U/kg), TXA, DDAVP, aminocaproic acid
    • Hemophilia B: 
      • Severe bleed: Give full dose factor IX (100U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor IV replacement (50U/kg), TXA, aminocaproic acid
  • Treatment if patient has inhibitors:
    • Hemophilia A: 
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    •  Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

Show References



Since Christmas is coming up, let's talk about Hemophilia A (factor VIII deficiency) and Hemophilia B (factor IX deficiency, also known as Christmas disease)

Deficiencies in Factors VIII and IX are the most common severe inherited bleeding disorders.

Pathophysiology:

  • Factors VIII and IX are required for activation of factor X.
  • In patients with Hemophilia A (factor VIII deficiency) or Hemophilia B (factor IX deficiency, also known as Christmas disease), after an injury, clot formation is delayed.
  • Inadequate thrombin generation leads to failure to form a tightly crosslinked fibrin clot to support platelet plug, which leads to easy bleeding.
  • Clot that is formed may be friable and rebleeding occurs during physiologic lysis of clots or with minimal new trauma

Clinical Manifestations:

  • 2% of neonates with hemophilia have intracranial hemorrhages
  • 30% of male infants with hemophilia bleed with circumcision
  • Continued bleeding from umbilical stump in neonate
  • In absence of positive family history (hemophilia has high rate of spontaneous mutation), hemophilia may go undiagnosed in a newborn
  • Easy bruising, intramuscular hematomas, and hemarthroses (hallmark for hemophilic bleeding) begin when child begins to cruise
  • Bleeding from minor traumatic lacerations of the mouth (e.g. torn frenulum) can persist for hours or days
  • Iliopsoas hemorrhage: patient may lose large volumes of bleed into the muscle, leading to hypovolemic shock, with only a vague complaint of area of referred pain in the groin. Hip is held in a flexed, internally rotated position, due to irritation of the iliopsoas.
    • Confirmed on CT or US
    • Clinically unable to extend hip
  • Hemarthrosis rare in patients with acquired hemophilia

Lab findings and diagnosis

  • Reduced levels of factor VIII or factor IX will cause higher PTT
  • PTT is usually 2-3x upper limit of normal in patients with severe hemophilia.
  • Platelet count, bleeding time, prothrombin time, and thrombin time are all normal
  • If PTT is not corrected after administration of factor VIII or IX, an inhibitor may be present.
    • 25-35% of patients with hemophilia who received infusions of factor VIII or factor IX, a factor specific antibody may develop (inhibitor)

Genetics

  • Hemophilia occurs in 1:5000 males, with 85% having factor VIII deficiency and 10-15% having factor IX deficiency
  • No apparent racial predilection, appearing in all ethnic groups

 

Classification

  • Severe hemophilia: <1% activity of specific clotting factor and bleeding is often spontaneous
  • Moderate hemophilia: 1-5% activity and require mild

trauma to induce bleeding

  • Mild hemophilia: >5% activity and can go many years before diagnosis and usually require significant trauma to induce bleeding.

Treatment

  • Ask patient or family if they brought their dosing information with them or their factor replacement with them. In many cases, they have it!
  • For life-threatening or major hemorrhages, dose should aim to achieve levels of 100% activity
    • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
    • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
    • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
    • If you don’t have factor-specific products:
      • Hemophilia A
        • can give 1U cryoprecipitate (~80U of factor VIII) or try PCC (as it contains factors II, VII, IX, and X)
        • activated PCC (FEIBA) 75-100U/kg
      • Hemophilia B
        • FFP NO LONGER RECOMMENDED (volume of FFP required has high risk of volume overload)
        • Cryoprecipitate does NOT contain factor IX, so will not work.
  • For acute bleeding in patients with mild hemophilia A:
    • Can give DDAVP: increases factor VIII by 3-5x by encouraging release of endogenous factor VIII. Recommended dose: 0.3mcg/kg/dose IV
  • For mild bleeding:
    • TXA (clot stabilizer)
    • Desmopressin
    • Aminocaproic acid
  • If patient has inhibitors:
    • Hemophilia A:
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    • Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

Summary:

  • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
  • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
  • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
  • Treatment if patient has no inhibitors:
    • Hemophilia A:
      • Severe bleed: Give full dose factor XIII (50U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor XIII replacement (25U/kg), TXA, DDAVP, aminocaproic acid
    • Hemophilia B:
      • Severe bleed: Give full dose factor IX (100U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor IV replacement (50U/kg), TXA, aminocaproic acid
  • Treatment if patient has inhibitors:
    • Hemophilia A:
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    •  Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

 

Show References



Background: 
-Initial lactate clearance over 2 hours has been used to measure pt response to resuscitation in sepsis. However, data supporting its prognostic use is lacking.
 
Study:
-Single center, retrospective cohort study in large academic center
-Adult patients (4,775) admitted through ED with suspected infection
-Lactate clearance defined as 10% decrease in 2 to 12 hrs
-Multivariable logistic regression adjusting for age, sex, cirrhosis, ESRD
 
Results:
-Lactate trajectories highly variable
-Lactate clearance was highly confounded by patient comorbidities (particularly cirrhosis)
-24-h change in peak lactate was a better prognostic indicator (but still poor)
 
Takeaway:
-In the ED, caution should be used when using lactate clearance as a single marker for assessment/prognosis, particularly if the pt has other comorbidities such as cirrhosis

Show References