Grand Rounds Recap 6.10.20

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Peds Sim WITH dr. shah

Status Epilepticus in Peds

  • Be aggressive with AEDs up front (anti-epileptic drugs) as recent literature shows the longer you are seizing, the harder it is to stop

  • First line Versed 0.1-0.2 mg/kg IV/IM or Lorazepam 0.1 mg/kg IV can repeat after 5 min if still seizing

    • Repetitive doses of benzos have been shown to be less effective than originally thought, especially in patients with prolonged seizure activity prior to medical care

    • Repeat benzos also increase the risk of respiratory depression and circulatory collapse

after 10 min

  • 2nd line medications (phenytoin, levetiracetam, valproate)

    • CONCEPT Trial:

      • 3mo - 16yr: given levetiracetam 40mg/kg vs phenytoin 20mg/kg in total of 233 kids

      • Outcome: seizure cessation at 5 min as reviewed by an independent observer (on video)

      • levetiracetam group 50% cessation at 5 min vs Phenytoin 60% cessation at 5 min

      • Conclusion: levetiracetam is equivalent but not superior

    • EcLiPSE Trial:

      • 6mo-18yr: levetiracetam 40mg/kg vs phenytoin 20mg/kg in 286 kids

      • Outcome: cessation judged by treating physician

      • levetiracetam time to cessation about 35 min vs phenytoin time to cessation 45 min

      • Conclusion: No significant difference

    • ESETT Trial:

      • 2-17yr: given levetiracetam 60 mg/kg vs fosphenytoin 20 mg/kg vs valproate 40 mg/kg

      • Outcome: cessation and clinical responsiveness at 60 mins

      • Results: All drugs equally effective with similar rates of adverse effects, however study stopped early

After 10 mins post-infusion completion

  • 3rd line if seizures are ongoing (midazolam, propofol, pentobarbital)

    • Midazolam: 0.2 mg/kg bolus followed by 0.05-2mg/kg/hr

    • Pentobarbital: 5-15 mg/kg bolus followed by 0.5-5mg/kg/hr

    • Propofol: keep dose <5mg/kg/hr (little evidence in children); should not be used on children who require ketogenic diet; there are however less cardiovascular effects in propofol than pentobarbital

  • Metabolic causes always need to be considered such as hyponatremia

    • Ask about free water to family in a patient who is vomiting

    • Consider giving hypertonic if Na less than 125 although usually level is much lower

    • 3% 1ml/kg aliquots given at 1 min intervals until seizure stops

  • No one knows at what time intubation is indicated in this disease process, unless there are objective criteria met (no respiratory effort, poor respiratory effort with declining sats, rising pCO2). However, some recent literature suggests intubation earlier in the course may help abort seizure, maybe relative to the RSI meds or improving acidosis.

Oral Boards Case 1: COVID associated Kawasaki

11yo M presenting with fever for 5 days. Also having abdominal pain with episodes of vomiting and diarrhea. Mom recently had COVID like symptoms. Otherwise patient healthy.

Vitals: T 102, HR 135, BP 80/48, RR 18, SpO2 98% on Room Air, Normal weight for age

Exam: Notable for mild bilateral conjunctivitis, cracked/dry lips with scattered cervical lymph nodes. Tachycardic, with no murmur. Erythema and swelling to bilateral hands and feet.

Labs: WBC: 2.3 with lymphopenia, VBG: 7.28, pCO2 34, Bicab 19, -6 Lactate: 5, CMP: normal, elevated ESR and CRP, D-dimer 2.5, troponin 0.8, BNP 2045, SARS-CoV-2 RT-PCR: +

Discussion

Differences from Kawasaki’s

  • Affected children are older: 7.5yr (Verdoni et al. Lancet), 10yr (Belhadjer et al. Circulation)

  • LV dysfunction more commonly seen than coronary artery disease

  • More severe:

    • 60% with cardiac involvement

    • 50% with Kawasaki disease shock syndrome - “systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of ≥20%, or clinical signs of poor perfusion

    • 50% with Macrophage Activation Syndrome, also known as secondary hemophagocytic lymphohistiocytosis, is characterized by, “persistence of fever with splenomegaly, hyperferritinemia, thrombocytopenia, and elevated aspartate aminotransferase (AST)

  • Signs and Symptoms (Bhelhadjer et al. Circulation): fatigue (100%), fever (100%), diarrhea/vomiting (83%), respiratory distress (65%), rhinorrhea (43%), adenopathy (60%), skin rash (57%), meningismus (31%)

  • Cardiac Findings: cardiogenic shock with collapse (80%), coronary artery dilation (17%), LV dysfunction (72%)

CDC Definition

  • An individual aged <21 years presenting with fever, laboratory evidence of inflammation, and evidence of clinically severe illness requiring hospitalization, with multisystem (>2) organ involvement (cardiac, renal, respiratory, hematologic, gastrointestinal, dermatologic or neurological) AND no alternative diagnoses AND positive SARS-CoV-2 infection

Tests to Order

  • CBC with diff, blood cultures, BMP, hepatic panel, ESR, CRP, Procalcitonin, LDH, Fibrinogen, D-dimer, Troponin, UA, SARS-CoV-2 RT-PCR, EKG, CXR, Echo (can be done inpatient)

Treatment

  • Still being defined, but similar to Kawasaki’s management

Oral Boards Case 2: Congenital Adrenal Hyperplasia

3 week old M presenting with poor feeding, vomiting, and lethargy. Over the past 3 days has been more tired and less feeding. Only 2 wet diapers in 24 hours, now refusing to breast feed. Born at 39w C/S. Uncomplicated pregnancy or delivery.

Vitals: T: 36.1, BP: 62/44, HR: 175, RR: 44, O2 sat 100% on RA, Weight: 7lbs (birth weight 6lb 14oz)

Exam: Notable for lethargic on exam, sunken fontanelle, dry mucous membranes, tachycardic with delayed cap refill

Labs: Glucose 50, Na 121, K 6.5

Discussion

  • Caused by impaired steroidogenesis. 21-hydroxylase deficiency responsible for 75% of cases of primary adrenal insufficiency and ~90% of causes of congenital adrenal hyperplasia. 21-hydroxylase deficiency is tested on newborn screen

  • 3 phenotypes:

    • 1) classic salt-losing

    • 2) classic non-salt losing (virilization)

    • 3) non-classic (late-onset)

  • Clinical Manifestations:

    • Females with salt-losing or non-salt losing will have ambiguous genitalia (clitoral enlargement)

    • Males with salt-losing present as neonates (FTT, dehydration, hyponatremia, hyperkalemia), usually between day 7-14

    • Males with non-salt losing present with early virilization

    • Late-onset present with early puberty, hirsutism and menstrual irregularity

  • Differential Diagnosis: Gastroenteritis, sepsis, pyloric stenosis, CHD, inborn errors of metabolism, malroation/volvulus

  • Evaluation: (Ideally labs collected prior to steroids): POC Glucose, electrolytes, cortisol, adrenal steroids (17-hydroxyprogeterone), ACTH, renin. Other labs can be drawn inpatient.

  • Treatment:

    • Ask family for action plan

    • Stress dose steroid: IM/IV hydrocortisone

      • 25mg for <3yr old

      • 50mg for 3-12yr old

      • 100mg for >12yr old

    • Hyperkalemia: Get EKG, can consider calcium, usually responds to IVF and hydrocortisone, rarely need insulin and glucose in infants

    • Hypoglycemia: Treat with dextrose bolus

    • Shock: Fluids will help but will need steroids

Global Health grand rounds: tales from the residents WITH drs. modi, jarrell, harty

Schistosomiasis US with Dr. Modi

  • Approximatey 229 million people require preventative treatment

  • Two main forms: 1) intestinal, 2) urogenital

  • Contracted by walking in shallow water where snails (as carriers) live where then the cercariae circulate in the body and find home in bladder and liver

  • Treatment: Praziquantel 20mg/kg x2 doses

  • US findings:

    • periportal fibrosis, ascites, hepatomegaly

    • will also see bladder wall thickening and in later stages can see pseudopolyp formation with increased vascularity

Burkitt Lymphoma with Dr. Jarrell

  • Patient was an 8-9 yo male with intermittent fevers, weight loss and mass on cheek

  • Non-Hodgkin Lymphoma of germinal B cells. Most aggressive NHL. Mostly localized to facial bones and jaw.

  • Three different types: 1) endemic, 2) sporadic, 3) immunodeficiency related

  • Biopsy features “starry sky” appearance

  • Short duration, high intensity chemotherapy associated with excellent prognosis

Rheumatic Heart Disease with Dr. Harty

  • Incidental finding on young healthcare worker volunteering for echo and found to have rheumatic heart disease with a thickened immobile mitral valve and dilated left atrium.

  • Definition: Chronic valvular disase that develops over years after rheumatic carditis

  • 3.4 cases per 100k in non-endemic areas vs 444 cases per 100k in endemic areas

    • Primarily affects those living in poverty with inadequate access to health care

  • Mitral valve most commonly affected followed by aortic valve; mitral regurgitation most common early finding where as mitral stenosis most common after 30

hemorrhage control WITH dr. kenji inaba

 Pressure

  • Most important aspect of hemorrhage control, but has to be done correctly. Often we just put a large wad of gauze over area of bleeding without identifying exact area of the bleeding.

  • Better to use one piece of gauze with pinpoint finger exactly to where bleeding is. In vast majority of cases this is very effective.

  • Some will utilize granules however difficult to control dispersion

  • Combat gauze: fibrin scaffold gauze that triggers intrinsic clotting pathway

    • Pros: pre-packaged, convenient, easy to deploy

    • Cons: increased cost, unclear benefit over regular gauze for most in-hospital applications

Tourniquets

  • Highly effective for compressible distal extremity. Little downside unless not used when should have been.

  • Use early, use liberally.

  • Hands free

  • Minimal risk of complications or limb loss with large potential benefit

  • One may not always be enough (especially for upper extremity bleeding). May have to use 2 or 3.

Junctional Bleeding

  • Defined as bleeding from a junction to the torso (ex. neck, armpit, groin). These areas bleed a lot!

  • Best technique to start with is gauze and pressure.

  • Junctional tourniquets are external devices designed to help occlude bleeding from junctional areas. These are often big and clunky and most places do not have them.

  • A Foley catheter can be used to temporize bleeding by placing in the hole and blowing up the balloon (difficult to use in subclavian area).

  • There is an FDA approved product called XStat that can be used in field setting that is a compressed pack of foam like material that swells when hit by blood to help compress area.

Pelvic Fractures

  • Bleeding from bad pelvis fractures are often retroperitoneal and can be difficult to access and difficult to control.

  • What is actually bleeding? Pelvic venous plexus (80-85%), internal iliac branches (5-10%), major iliac veins (10%), external iliac branches (3-4%)

  • Unstable pelvic fracture priorities:

    • Resuscitation

    • Look for associated injuries

    • Mechanical stabilization (external device such at T pod)

    • Embolization

    • Packing

    • REBOA

  • Binders are effective at reducing anatomical volume of the pelvis, but unclear on impact of decreasing bleeding. They do however reduce pain, splint fracture and facilitate movement.

    • Can use a sheet and kelly clamps to hold in place if do not have a T pod

    • Do not put on certain pelvic fractures such as isolated iliac wing or acetabular fractures as these can make them worse.

  • Definitive treatment is packing, angiography or operative stabilization

REBOA

  • Percutaneous intra-aortic balloon that occludes the distal thoracic aorta

  • In hypotensive patient, decreases blood loss to abdomen and pelvis therefore increasing flow to coronaries and brain.

  • What does it do?

    • Resuscitation adjunct, facilitates volume loading

    • Limits initial capacity to upper torso and allows controlled increase remainder of body

    • Maintains perfusion and prevents progression to arrest

  • Placed in Zone I or III

    • Zone I: ranging from left subclavian artery to celiac artery; placed here if no idea where bleeding is coming from

    • Zone III: just above iliac bifurcations; placed here if known pelvic fracture

  • Important note is that you do not have definitive hemorrhage control. This is a bridge to definitive therapy.

  • Data is very poor, no class I or II studies

  • Dr. Inaba’s practice: used in blunt trauma without evidence of intrathoracic source, refractory hypotension (especially with known pelvic fracture)