Brown Pediatrics

Brown's Pediatric Residency Blog

Author: Brian Lee (page 2 of 3)

“Your skin,… And it was all yellow”



Your next patient is a 17 day old presenting for follow-up. During the visit you note that the baby’s eyes and skin are yellow.  Labs show a total bilirubin of 7 mg/dL, with direct component being 5mg/dl. What are your next steps?

What is Jaundice?

  • A yellowish discoloration of the skin and sclera due to elevated bilirubin levels in the blood.
    • Associated findings include: pale stool, dark urine
    • Becomes clinically apparent at total levels >2.5mg/dL (Fawaz  et al, 2016)
  • Very common finding; found in up 15% of infants at 2 week visit (Kelly et al, 1995)

What is Bilirubin?

  • Breakdown product of heme metabolism
  • Conjugated in the Liver
  • Excreted in feces (majority) and urine

Approach to A Jaundiced Neonate (adapted from NASPGHAN guidelines; Moyer et al, 2004)

Step 1: Is the baby sick?
  • Differential includes: infection (especially urinary tract), inborn errors of metabolism, acute hemolysis, etc
  • Most important: manage acute illness
Step 2:  What type of hyperbilirubinemia?
  1. Direct
    • Defined as a serum conjugated bilirubin >1.0mg/dl if serum total bilirubin is <5.0 mg/dl or >20% of total bilirubin if >5 mg/dL. (Shulman et al, 2016)

Figure 1: Most Common Etiologies of Neonatal Cholestasis

Etiology Number of Cases (Total: 1692) % of Cases
Idiopathic Neonatal Hepatitis 440 26
Extrahepatic Biliary Atresia 438 25.9
Infection 194 11.5
Metabolic Disease 74 4.4

Adapted from Gottesman et al, 2015

    2.) Indirect Hyperbilirubinemia

  • Most commonly due breast milk jaundice, though larger differential exists. Will not be discussed during this post.
Step 3: Other Labs and Studies
  1. Important Imaging Studies
    • Ultrasound- wide estimates of sensitivity
      • 73-100% for absent gallbladder and 83-100% for “triangular cord” sign depending on study cited.
  2. Other labs/blood to obtain
    • Review Newborn Screen: Congenital hypothyroidism and galactosemia can present with cholestasis.
    • GGTP, LFTs, CBC, Coags
    • α-1 antitrypsin (If low, order Pi Typing)
 Next Steps
  • Next steps largely depend on findings of workup listed above
  • As biliary atresia (BA) can cause neonatal cholestasis, this MUST be ruled out
    • Outcome of patients diagnosed with biliary atresia is inversely correlated with age of diagnosis (specifically the age at which they undergo the Kasai hepatoportoenterostomy (Balistreri et al, 1996)
    • If ultrasound non-diagnostic, hepatobiliary scintigrapy (HDS), magnetic resonance cholangiopancreatography (MRCP), liver biopsy may need to be undertaken to rule out BA
      • Note: Gold standard is intra-op cholangiogram (Shulman et al, 2016)
Faculty Reviewer: Jason Shapiro, MD
Resident Reviewer: Meg Gibson, MD


  • Balistreri WF et al. “Biliary Atresia: Current Concepts and Research Directions.” Hepatology. 1996;23(6)1682-1692.
  • Fawaz R et al. “Guideline for the Evaluation of Cholestatic Jaundice in Infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.” Journal of Pediatric Gastroenterology and Nutrition. 2016; (Published ahead of print)
  • Gottesman LE, Del Vecchio MT, Aronoff SC. “Etiologies of conjugated hyperbilirubinemia in infancy: A systematic review of 1692 subjects.” BMC Pediatrics 2015; 15:192
  • Kelly DA et al. “Jaundice in Babies: Implications for Community Screening for Biliary Atresia.” The British Medical Journal. 1995;310(6988)1172-1173.
  • Moyer M et al. “Guideline for the Evaluation of Cholestatic Jaundice in Infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.”Journal of Pediatric Gastroenterology and Nutrition. 2004;39(2)115-128
  • Shulman RJ et al. “Approach to Neonatal Cholestasis.” Accessed July 5th, 2016.

Image of the Week: 10/17

While evaluating an otherwise asymptomatic, healthy adolescent in clinic, you note an irregular rhythm on exam. Given this finding, you order an EKG (shown below). What do you think?


EKG Courtesy of Life in the Fast Lane:

Diagnosis:  AV block: 2nd degree, Mobitz 1 (Wenckebach Phenomenon)


What is it?

  • Progressively lengthening of the PR interval and eventual “dropped QRS” complex, caused by increased refractory period of the AV node . The entire process tends to occur over 3-6 cardiac cycles (Doniger et al, 2006)
  • Following “dropped” beat, there is a diastolic pause, and cycle continues
  • Characteristically thought of as a “benign rhythm” found in asymptomatic individuals and there is low risk of progressing to third degree heart block. In fact, up to 10% of healthy will episodes of type 1, second degree heart block (Dickinson, 2005).
    • However, this may be seen in myocarditis, congenital heart disease, and following cardiac surgery

Created by:  Jason Mandell, MD

Reviewed by: Brian Lee, MD


Dickinson DF. “The Normal ECG in Childhood and Adolescence.” Heart. 2005;91:1626-1630

Doniger SJ et al. “Pediatric Dysrhythmias.” Pediatric Clinics of North America. 2006;53(1)85-105

Life in The Fast Lane:


Trouble in Paradise



Case: George is a 5 year-old boy presenting to an Emergency Department (ED) complaining of abdominal pain and loose stools following a recent tropical vacation. How should we proceed? Is there any way that we could have prevented this?

What is traveler’s diarrhea?

    1. Classic Definition: ≥ 3 unformed stools in 24 hour period with nausea, vomiting, cramps, fever, blood in stool (Stauffer et al, 1990)
      • For infants and young children, some authors define diarrhea as ≥ 2-fold increase in unformed stool (Ashkenazi et al, 2016)
    2. Moderate diarrhea: 1-2 loose stools per 24 hour period
    3. Mild diarrhea: 1 loose stool per 24 hour period
    4. Duration (CDC, 2016)
      • Viral : 2-3 days
      • Bacterial: 3-7 days
      • Protozoal: weeks to months

Etiology (Ashkenazi et al., 2016)


Electron Microscope Image of E. Coli (Pixabay Image)

    1. Bacterial: E. Coli (ETEC, EHEC, EAEC, etc), Campylobacter jejuni, Salmonella spp., Shigella spp.,  are the most commonly seen, though Aeromonas spp. increasingly noted (CDC, 2016).
      • Of note, E. Coli (O157:H7) [associated with hemolytic uremic syndrome] has not been described in traveling children (Mackell, 2005)
    2. Viral: rotavirus, norovirus, adenovirus
    3. Parasite: Giardia (most common), Cryptosporidium, Cyclospora, entamoeba (uncommon)
    4. Etiologic agent generally identified in less than ⅓ of cases



    1. General incidence: 10-40% of travelers (Pitzinger B et al, 1991), though can affect up to 70% of travelers depending on the location they were traveling in (CDC Yellow Book)
      • Highest risk in Asia, Sub-Saharan Africa, and Latin America (Hagmann et al, 2010)
    2. Young children at the highest risk and manifest most severely (Ashkenazi et al, 2016)
    3. Children visiting family and/or friends are at higher risk as compared to tourists


    1. Microbiologic identification is generally unnecessary
    2. If fever and colitis  think Campylobacter, Shigella, EHEC
    3. Predominance of upper GI symptoms  Giardia, isospora, cyclospora
    4. If recent antimicrobials  -> C. diff
    5. If ill, send cultures for salmonella

Role for Prevention? (Connor, 2015)

  1. Choosing food and beverages wisely while traveling has been the cornerstone of advice
    • Unfortunately, studies do not show benefit to this practice (Steffen et al, 2004)
  2. Hand hygiene very important
  3. For children older than 12 years old, bismuth subsalicylate has been shown to reduce incidence of traveler’s diarrhea by 50%
    • Inconvenient dosing: 2 tabs, four times daily
  4. Prophylactic antibiotics are not generally recommended
    • May be considered in “high-risk hosts” (e.g. immunosuppressed)

His dad asks: how should he treat this?


    1. Maintaining hydration is the most important treatment
      • Use urine output as a guide (if normal urine output, diarrheal illness is mild)
    2. If evidence of dehydration: Preferentially use oral rehydration solution (Desforges, 1990)
      • WHO solution made with: Glucose (20g/L), 3 salts (3.5g/L) [sodium chloride, potassium chloride, and sodium bicarbonate]
      • Rationale for use is intestinal co-transport of glucose and sodium
    3. Role of antibiotics
      • Warranted in severe diarrhea (>4 stools in 24 hr period, fever, blood/pus in stool)
        1. Azithromycin is treatment of choice (Ashkenazi et al., 2016)
        2. Rifaxamin for children ≥ 12 years old
        3. Fluoroquinolones (Note: not FDA approved for children)


      1. Diarrheal illness in children returning from travel is not uncommon
      2. Younger children at higher risk of significant morbidity
      3. Maintaining hydration is essential; utilize oral route
      4. Antibiotics not well studied, beneficial in severe cases
      5. For all traveler’s, utilize CDC’s Website to provide resources and guidance

Online Resources

  • CDC:

Faculty Reviewer: Michael Koster, MD


Ashkenazi S et al. “Travelers’ Diarrhea in Children: What have we learnt?” The Pediatric Infectious Disease Journal. 2016;35(6)698-700.

Connor BA. “Traveler’s Diarrhea.” CDC Health Information for International Travelers 2016. Ed. G. Brunette. Oxford University Press, 2015.  Print and Online

Desforges JF. “Oral therapy for Acute Diarrhea- The Underutilized Simple Solution.” NEJM. 1990; 323:891- 894.

Hagmann S et al. “Illness in Children After International Travel: Analysis From the GeoSentinel Surveillance Network.” Pediatrics. 2010. 125(5)e1072-e1080

Mackell S. “Traveler’s Diarrhea in the Pediatric Population: Etiology and Impact.” Clin Infect Dis. 2005;41(Suppl 8)S547-S552.

Pitzinger B et al. “Incidence and clinical features of traveler’s diarrhea in infants and children.” The Pediatric Infectious Disease Journal. 1991;10(10)

Stauffer WM et al. “Traveling with Infants and Small Children. Part III: Traveler’s Diarrhea.” Journal of Travel Medicine. 2002;9(3):141-50

Steffen R et al. “Epidemiology of Travelers’ Diarrhea: Details of a Global Survey.” J Travel Med. 2004;11(4)231-238.



“Water, water, everywhere…”



Case: Zoe is a 10 day old ex- full term female, born to a G1P0 →1 presenting with feeding difficulties. Per her mother, she is exclusively breastfed and had initially had been doing “ok” but for the last couple days, has been more sleepy than usual and not feeding as well. She also notes that during this time, her eyes have become a bit more yellow.


On exam, you note an infant in no distress, but she sleeps comfortably through your exam. Jaundice is appreciated. Vitals are normal, but you note she has lost 12% of her birth weight. Her HEENT is notable for a sunken anterior fontanelle. Her exam is otherwise benign. Concerned for hyperbilirubinemia and dehydration, you order a complete metabolic panel, which, among other abnormalities, is significant for a serum sodium of 165 meq/L.


Why is her sodium so high?

Diagnosis: Severe neonatal hypernatremic dehydration



  • In this case, the most likely etiology is ineffective breastfeeding (also termed lactation failure), which is a rare, but increasing cause of hypernatremic dehydration (Mortiz et al, 2002)
  • In all humans (not just neonates), hypernatremia results from one of two mechanisms: inadequate access to free water and/or an inability to concentrate urine
  • Breastfeeding failure leads to inadequate fluid intake, but is also related to the higher concentration of sodium in breast milk (Morton, 1994)


How do patients present? (Moritz et al, 2005)

scaleOver 70% of patients had > 10% weight loss


Signs at Presentation

% Of Infants (n=70)

Jaundice 81
Poor PO Intake 61
Decreased Urine Output 36
Fever 20

Table Adapted from Moritz et al, 2005


How common is this problem?

  • Neonatal hypernatremic dehydration is rare. A review of admissions to a major children’s hospital found that over 4 years, 1.9% of term and near term infants were admitted for hypernatremic dehydration (Mortiz et al., 2005)
  • Most commonly affects primiparous mothers


How should we treat?

  • The goal of treatment is to lower serum sodium in a slow and controlled fashion
  • Conventional teaching states that sodium should not be lowered faster than 0.5mEq/hr and in fact, recent studies suggest that correction faster than 0.5mEq/L/hr is independently associated with poor neurologic outcomes and seizures (Bolat et al, 2013)
  • Specifics (based on protocol detailed in Bolat et al)
    • Emergency Phase
      • Correct shock immediately (within 30 mins) with 10-20 cc/kg 0.9% saline
    • Rehydration Phase
      • Calculated Free Water Deficit
      • Composition of fluid for rehydration is dependent on serum sodium; remember, in patients with high serum concentrations, “normal saline” will be hypotonic (154 meq/L)
      • Serum sodium should be decreased by 0.5meq/L/hr over the first 24-48 hours
      • If a patient is urinating, add 40 meq potassium to fluids


What are the neurological outcomes?

  • In the aforementioned study (Bolat et al, 2013), researchers found that presenting serum sodium >160 meq/L was an independent predictor of mortality (OR: 1.9) and correction faster than 0.5 meq/hr was independently associated with an increased risk of seizures (OR: 4.3)
  • At 6 months of age, patients were screened with the Denver Developemental Screening Test II. Serum sodium > 165 meq/L on presentation was associated with worse outcome.


  • Neonatal hypernatremic dehydration is a rare complication of exclusive breastfeeding, primarily seen with primiparous mothers and  can have devastating consequences
  • Clinicians need to be aware of this complication and ensure infants  who are exclusively breastfed are followed closely to ensure adequate breastfeeding and weight gain
  • If hypernatremic dehydration is encountered, it is imperative to 1.) treat shock initially and 2.) ensure that serum sodium is NOT corrected faster than 0.5 meq/hour

Resident Reviewer: Marie Carillo, MD


  • Ahmed A et al. “Complications Due to Breastfeeding Associated Hypernatremic Dehydration.” Journal of Clinical Neonatology. 2014;3(3):153-157
  • Bolat F et al. “What Is the Safe Approach for Neonatal Hypernatremic Dehydration?” Pediatric Emergency Care. 2013;29(7):808-813
  • Moritz ML et al. “Breastfeeding-Associated Hypernatremia: Are We Missing the Diagnosis?” Pediatrics. 2005;116(3):e343-e347
  • Moritz ML et al. “Disorder of Water Metabolism in Children: Hyponatremia and Hypernatremia.” Pediatrics in Review. 2002;23(11):371-380
  • Morton J. “The Clinical Usefulness of Breast Milk Sodium in the Assessment of Lactogenesis” Pediatrics. 1994;93(5):802-806

BRUE is the new Black

Post Created by: Bill Foo, MD

Case 1



3 week old ex FT baby Bruce is brought the emergency room after “mom says he stopped breathing for about 10 secs. He had associated cyanosis around the lips. His mother witnessed the entire event. He did not require any stimulation for the event to resolve. By the time EMS arrived, baby Bruce is already back to his usual self. Initial vitals and exam in the Emergency room is reassuring.


What should you do with this kid?


The American Academy of Pediatrics (AAP) released new guidelines concerning children presenting with a “Brief Resolved Unexplained Event (BRUE),” formerly referred to as an “Apparent Life Threatening Event (ALTE).” These new guidelines are meant to reduce testing in low risk infants coming in for a BRUE.


The two questions that determine if the new guideline applies to your patient are:

  • Does this event meet criteria for a BRUE?
    • There are narrow criteria for what the AAP defines as a BRUE in this guideline
      • Brief event lasting less than one minute in a child <1 year of age
      • Events can have one or more of:
        • Cyanosis or pallor
        • Absent, decreased, or irregular breathing
        • Marked change in tone (hyper- or hypotonia)
        • Altered responsiveness
      • The patient must return to their baseline after the event without any resuscitation by trained medical provider.


  • Is this the patient low risk?
    • The AAP guideline’s recommendations only apply to patients who they define as low risk!!!
      • Age >60 days
      • Gestational age >/= 32 weeks and post-conceptional age >/= 45 weeks (calculate the corrected gestation age, those NICU/ex-premie will trick you)
      • No previous BRUE episode, and occurrence of only 1 BRUE with this event (not occurring in clusters)
      • No concerning physical exam findings
      • No concerning historical feature that would suggest another diagnosis such as underlying neurological, cardiac, pulmonary or GI disorder.


If you can answer Yes to both of the questions above, you can send the patient home  with reassurance and education, but without further testing. Note, in the case described above, Bruce is 3 weeks old, therefore WOULD NOT be considered low risk. 


BRUE guideline:

“Laboratory studies, imaging studies and other diagnostic procedures (including admission to the hospital solely for cardio-respiratory monitoring as lower risk infants do not have increased risk of cardiovascular events) are unlikely to be useful”

Case 2


6 week old ex-FT baby Sarah is brought the emergency room after developing apnea for 2 minutes. She was unresponsive during this event. She also became limp and her hands and feet turned blue. EMS was called, and by the time of arrival the baby was back to her baseline self. In the ED, she is noted to have vitals appropriate for age and a reassuring exam.


What do you do for this child?


The event described does not meet criteria for BRUE based on the history provided and furthermore, Sarah is not considered “low risk” based on her age. At a minimum, she should be admitted to the floor for at least 24 hours of cardio-respiratory monitoring.


High-risk infants:

  • Age < 60 days (ALTE events may indicate occult bacteremia)
  • History of prematurity
  • Duration of event >1 minutes
  • Clustering of events.
  • History of ALTE or of ALTE or unexplained death in a sibling


Due to a paucity of evidence, the new guideline does not provide any recommendations for evaluation of high-risk BRUEs.  However it does state that some studies suggest that these patients are more likely to have “a serious underlying cause, recurrent event, or an adverse outcome.”


Can’t remember all the specifics? Well there is a smart phrase in EPIC you use: .BRUEVSALTE

Faculty Reviewer: Dan Coghlin, MD


Tieder, J. S., Bonkowsky, J. L., Etzel, R. A., Franklin, W. H., Gremse, D. A., Herman, B., . . . Smith, M. B.  “Brief Resolved Unexplained Events (Formerly Apparent Life-Threatening Events) and Evaluation of Lower-Risk Infants: Executive Summary.” Pediatrics. 2016(5)137. doi:10.1542/peds.2016-0591

Commercial Sexual Exploitation of Children (CSEC)

Post Created by: Anish Raj, MD

Case: A 15-year-old female, with a history of PTSD, presents to the ED for medical clearance after being AWOL (absent from care) from her group home for the past 3 weeks. What are critical components you should consider prior to making any disposition plans for this patient?

I.) Background (Smith, 2014)

  • Commercial Sexual Exploitation of Children (CSEC): the sexual abuse of a minor (< 18 years old) with remuneration in money, goods, or services—or the promise of money, goods, or services—to the child or a third-party for the sexual use of that child.
  • Note: CSEC is an umbrella term that encompasses child sex trafficking, escorting, survival sex, child pornography, stripping, etc.
  • Domestic Minor Sex Trafficking (DMST): the inducement of a commercial sex act of anyone under the age of eighteen by a controlling party (i.e. trafficker/exploiter/pimp) that takes place within U.S. borders and involves a child who is a U.S. citizen.
  • Note: Per the Trafficking Victims Protection Act (TVPA), in cases of child sex trafficking, the inducement of a commercial sex act of a minor (< 18 years old) is enough to meet criteria for trafficking. Force, fraud, or coercion do NOT have to be demonstrated.


II.) Epidemiology (IOM, 2013)

  • No consensus on estimates of incidence and prevalence
  • Most widely cited national estimate: 244,000-325,000 children are at risk for CSEC
  • Average age of initial involvement: 15 years old (Gibbs et al., 2015)
  • Rhode Island preliminary data: ~70 suspected cases over the past 3 years


III.) Risk Factors

  • *History of sexual abuse*: up to 70-90% of CSEC victims (Bagley & Young, 1987)
  • History of running away and/or truancy: 70% of street youth estimated to be involved in CSEC at some point (Estes & Weiner, 2001)
  • Child welfare (e.g. DCYF) involvement: 50-80% of CSEC victims (Walker, 2013)
  • Juvenile justice system (e.g. RITS) involvement
  • Adult (> 18 years old) “boyfriend”/“girlfriend”
  • Multiple sexual partners at present
  • Positive STI testing
  • Substance use


IV.) Physical Exam


  • Observation: are clothing and accessories congruent with age/time/season?
  • Tattoos: name branding? explicit? gang insignia?
  • Note: In Rhode Island, individuals must be > 18 years old to receive a tattoo or piercing in a licensed parlor.
  • Signs of physical abuse (including but not limited to head trauma, oral trauma, genital trauma, and cutaneous injuries)


V.) Screening Examples (no brief, validated screening tool currently exists)

  • Have you or any of your friends ever exchanged sex for money, a place to stay, food, or drugs?
  • Has anyone ever asked you to have sex with someone else or made you have sex when you didn’t want to?
  • Has anyone ever taken sexual pictures of you or posted such pictures on the internet? (Greenbaum et al., 2015)


VI.) What To Do If You Suspect CSEC

  • Understand that it is very common for patients to NOT disclose
  • Notify Aubin Center (i.e. page on-call physician) of suspected CSEC
  • Determine need for forensic evidence collection if acute assault has taken place
  • Consult Psychiatry for evaluation
  • Confirm with law enforcement that missing persons report was filed if patient had been missing
    • If no report was filed, communicate this information to DCYF due to concern for possible neglect by caregiver
  • File report expressing concern for suspected CSEC to DCYF Hotline
  • Complete PRE without hold and fax to DCYF
  • Order screening labs: urine pregnancy, urine gonorrhea, urine chlamydia, urine trichomonas, urine toxicology screen, RPR, hepatitis C, hepatitis B, and HIV
  • Consider administration of empiric STI antibiotic treatment (e.g. ceftriaxone, azithromycin, and metronidazole)
  • Consider administration of Plan B
  • Consider administration of HIV post-exposure prophylaxis (PEP) on a case-by-case basis in coordination with Aubin Center +/- Infectious Disease team
  • Determine safe disposition plan
    • Ensure patient has an outpatient appointment at Aubin Center if being discharged
  • Consult social work and Aubin Center if patient is admitted


Faculty Reviewer: Christine Barron, MD



  • Smith, Holly Austin. Walking Prey: How America’s Youth Are Vulnerable to Sex Slavery. New York: St. Martin’s, 2014. Print.
  • Institute of Medicine and National Research Council. Confronting commercial sexual exploitation and sex trafficking of minors in the United States. Washington, DC: The National Academies Press; 2013. Print.
  • Gibbs, D., Walters, J., Lutnick, A., Miller, S., & Kluckman, M. (2015). Evaluation of Services for Domestic Minor Victims of Human Tracking. Manuscript submitted for publication. Retrieved August 12, 2016, from
  • Bagley, C., & Young, L. (1987). Juvenile Prostitution and Child Sexual Abuse: A Controlled Study. Canadian Journal of Community Mental Health, 6(1), 5-26. doi:10.7870/cjcmh-1987-0001.
  • Estes, R., & Weiner, N. (2001). The Commercial Sexual Exploitation of Children in the U.S., Canada and Mexico. Retrieved August 12, 2016 from
  • Walker, K. (2013). Ending the Commercial Sexual Exploitation of Children: A Call for Multi-System Collaboration in California (USA, California Child Welfare Council). Retrieved August 12, 2016, from Welfare/Ending CSEC-ACallforMulti-SystemCollaborationinCA-February2013.pdf.
  • Greenbaum, J., & Crawford-Jakubiak, J. E. (2015). Child Sex Trafficking and Commercial Sexual Exploitation: Health Care Needs of Victims. Pediatrics, 135(3), 566-574. doi:10.1542/peds.2014-4138.

Image of the Week: 8/9/2016


A 16 year-old male presents to the ED after a syncopal episode. He currently feels well and wants to go home. Below is his EKG, what do you think?





The above presentation (syncopal event) paired with this EKG is concerning for the Brugada syndrome.


  • The Brugada syndrome was first described in 1992 by Drs. Pedro and Josep Brugada in a case report of 8 patient with aborted sudden cardiac death (SCD), though cases of sudden unexplained nocturnal death (SUND) had been described previously in various southeast Asian populations (Juang JM et al, 2004).
  • Some estimate that 4-12% of SCD can be attributed to the syndrome, with a higher prevalence in SE Asian populations (Gourraud JB et al, 2016)
  • Median age of presentation is 41, though has be reported in neonates through the elderly.
  • While many mutations have been described, the Brugada syndrome appears to result from mutations in the Na+ channel, affecting the SCN5A gene (Behere SP et al, 2015)
    • Note: Only 20% of patients with Brugada syndrome have a documented mutation in the SCN5A gene. Studies show a autosomal dominant transmission with incomplete penetrance  (Juang JM et al, 2004).

Clinical Presentation:

  • In the original description of the syndrome, the Brugada brothers noted that all patients presented with syncope, with 5/7 progressing immediately to cardiac arrest and the other 2 progressing to cardiac arrest over the next 2 weeks
    • Registry data indicates that almost 1/3 present with syncope, 6% with SCD, though >60% were asymptomatic at the time of diagnosis (Probst V et al, 2010)
      • NOTE: Registry listed above excluded children, so interpret with caution
  • Sustained Polymorphic Ventricular Tachycardia is the most common rhythm noted to precipitate SCD

EKG Findings and Diagnosis

  • ST elevation in V1-3 with right bundle block morphology is common, with “coved pattern” in V1-3  negative T wave (referred to as “Type 1;” this is the only pathognomonic EKG pattern)
    • In most cases, no reciprocal ST-depression noted (Juang JM et al, 2004).
  • EKGs in Brugada syndrome tend to be variable and at times are even normal
  • In patients with normal EKG, class Ia or Ic antiarrhythmic drugs ( potently blocks Na channel) may unmask the Brugada pattern (Juang JM et al, 2004).
  • Two other patterns have been described, though are considered less significant
  • To be considered diagnostic of Brugada syndrome the EKG findings must be combined with the following clinical findings:
    • Polymorphic ventricular tachycardia
    • syncope
    • ventricular fibrillation
    • Family history of early sudden cardiac death (<45)
  • Workup must also exclude other causes that lead to ST-elevation (e.g. acute pericarditis, arrhythmogenic right ventricular dysplasia (ARVD), Prinzmetal angina, etc).


  • Implantable cardioverter-defibrillator (ICD) is the only strategy to prevent SCD
  • Patients should also be advised to avoid alcohol, certain drugs, and treat fevers aggressively (Refaat MM, 2016).
Resident Reviewer: Vanessa Tommey, MD


  1. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a multicenter report. J Am Coll Cardiol 1992;20:1391–6
  2. Juang JM et al. “Brugada syndrome–an under-recognized electrical disease in patients with sudden cardiac death.” Cardiology. 2004;101(4):157-69
  3. Gourraud JB et al. “The Brugada Syndrome: A Rare Arrhythmia Disorder with Complex Inheritance.” Front Cardiovasc Med. 2016 Apr 25;3:9.
  4. Behere SP et al. “Inherited arrhythmias: The cardiac channelopathies.” Ann Pediatr Cardiol. 2015 Sep-Dec; 8(3): 210–220.
  5. Probst V et al. “Long-Term Prognosis of Patients Diagnosed With Brugada Syndrome.” Circulation. 2010;121(5):635-643.
  6. Alley P. “What is Brugada Syndrome?”

EKG Basics

Created on 8/4/2016 by Hiral Mehta, MD


Griffin is a 5 y/o boy who comes into the ER. Parents state that earlier today he began complaining of his “heart running fast” and his chest hurting. His cardiac exam is unremarkable. You decide to get an EKG as part of your work up.

What should I know when interpreting a pediatric EKG?

EKGs are simple, relatively straightforward to obtain, and provide a great deal of important information- if you are able to interpret them!

First: Lead placement

There are several different types of lead set ups. Intuitively, more leads generally provide more information. Remember, EKGs are vector representations of electrical depolarization. The more “viewpoints” you have, the more information you can gather.


5 Lead-ECG: This is what we generally have on our hospitalized kids who are on CRM (cardio-respiratory monitoring).



Think “smoke (black) over fire (red), snow (white) over grass (green)” and “white on right”.

12-lead EKG: This is the standard 12 lead EKG that we order. In pediatrics, we use the “V4R lead” to better assess the right ventricular potentials because the RV extends to the right of the sternum in children. This gets confusing- you should still use the leads that the machine labels “V1”-”V6” in that order (R->L anatomically), but start V1 further to the right (and relabel the EKG when it prints out).


How do I interpret this EKG?

Good general guidelines: have a systematic approach. Look at as many EKGs as possible to start getting a sense of what is “normal”, especially at various ages. One standard approach is to think through EKGs as an approach of “rate, rhythm, and axis” but really, everyone tends to develop their own methodology.

It is helpful to think of the EKG as a “graph” of electrical activity on the “Y-axis” and time on the “X-axis.” (For more in depth explanation, please see videos at end of the post). 

  • The standard speed of paper is 25mm/sec, but it is always helpful to make sure EKG strips you are looking at are run at the standard speed
  • A “small box” is 0.04 seconds on the time axis and 0.1mV on the electrical axis
  • A “big box” is 0.2 seconds on the time axis and 0.5mV on the electrical axis
Basic components of Interpretation:


  1. For regular rhythms (more below on how to determine this), Rate = 300/ #of big boxes between 2 consecutive R waves
  2. For faster rhythms (infants), Rate = 1500/ #small boxes between 2 consecutive R waves
  3. For irregular rhythms, Rate = number of complexes on rhythm strip (usually lead II), multiplied by 6


  1. P waves: absent or present?
  2. Relationship between p- waves and QRS
    1. For sinus rhythm, there is a 1-1 relationship between p waves and QRS
  3. Regular vs Irregular (are intervals consistent across the rhythm strip)?
    1. If Irregular, is it regularly irregular (classically A-flutter) or irregularly irregular (classical atrial fibrillation)
  4. QRS morphology
    1. Narrow (sinus or junctional origin)
    2. Wide (ventricular origin)

Axis: There are multiple ways of determining the “axis.” Below highlights 1 of these methods. “Normal” axis changes with age, with infants having a “rightward” axis. As children grow older, the axis becomes more leftward.


Waves and Intervals:



  • P wave- represents atrial depolarization
  • QRS complex- represents ventricular depolarization
  • T wave- represents ventricular repolarization

(Note: In case you were wondering, there is an atrial repolarization wave, but it is “hidden” in the QRS complex).

Note: Remember, standard speed of paper is 25mm/sec, therefore, a “small box” is 0.04 seconds on the time axis and 0.1mV on the electrical axis and a “big box” is 0.2 seconds on the time axis and 0.5mV on the electrical axis


PR interval: This reflects conduction from the SA node AV node.

  • Normally thought of as 0.12- 0.2 sec (one “big box”) for adults; however in children it varies with age and HR.

QRS interval: Represents ventricular depolarization. The Q wave reflects depolarization from L -> R across the interventricular septum, the R wave reflects depolarization through the thick ventricular walls, and the S wave reflects depolarization of the Purkinje fibers. Again, normal intervals vary by age.

QT interval: Represents the time it takes for ventricular depolarization and repolarization. This is one of the intervals that can’t really be obtained just by reading off what the EKG machine calculates, since QT varies with the HR and the individual RR intervals. This will be expanded upon in a future topic, but for now:

  • The calculated “corrected QT” interval (QTc) is most commonly derived with Bazett’s Formula:
    • QTc=QT√RR
    • Use the RR interval preceding the QT interval that you’re looking at. This formula is most accurate for HRs 60-100!
    • Infants normally have a longer QTc (under 6 months, QTc of <490 msec is considered normal). Children older than 6 months should have a QTc <440msec.
    • MD Calc


For reference, here is a table with some normal EKG values for children (adapted from Sharieff et al, 2006):



Videos From around the web:

Basics of EKG leads

The Limb Leads

The Precordial Leads


Faculty Reviewer: Sara Ford, MD

Resident Reviewer: Brian Lee, MD


“ECG Basics.”

“Paediatric ECG Interpretation.”

Sharieff GQ, Rao SO. The pediatric ECG. Emerg Med Clin North Am 2006;24:196.


Image of the Week: 8/3

Created on 8/3/2016 by Vanessa Hand, MD


A 17 year-old male in obvious distress is brought the ED by his sister. She states that  this morning he woke up with a fever and a sore throat. However, over the next few hours his voice has been changing and is now more “hoarse.” She notes that during this time he also developed difficulty breathing. Below is an x-ray obtained upon presentation. What are you most concerned about?


Case courtesy of Dr Maxime St-Amant, From the case rID: 26840

 Radiology Findings: Typical findings of epiglottitis with enlarged epiglottis and aryepiglottic folds.

Diagnosis: Acute Epiglottitis


  • Combination of sore throat, dysphagia, “hot potato” voice and high fevers classically described
  • Difficulty with breathing may be most common chief complaint (Mayo-Smith et al, 1995)
  • Symptoms progress rapidly, usually over hours (Stroud et al, 2001)
  • Physical Exam Shows:
    • Vitals: Febrile, Tachypnea
    • Visibly Distressed Child; “Tripoding” position
    • Muffled or hoarse voice

Epidemiology (Shah at al, 2004)

  • Historically caused by H. Influenza type B, however vaccination has largely shifted etiology to other organisms
  • Rate dropped from 5/100,000 to 0.6-0.8/100,000 (immunized)
  • Increased age of presentation from 3 yo to 6-12 yo 

Causative Organisms

  • H. influenzae, penicillin resistant S. pneumoniae, S. Aureus, β-hemolytic strep


  • Minimize stimuli, stressful procedures
  • Maintain airway, anesthesia/ENT intubate in OR
  • Antibiotics: Ceftriaxone or Ampicillin/Sulbactam; add Vancomycin or Clindamycin if concern for MRSA

Faculty Reviewer: Brian Alverson, MD


Mayo-Smith MF et al. “Acute Epiglottitis. An 18-year experience in Rhode Island.” Chest. 1995;108(6):1640-7

Shah RK et al. “Epiglottitis in the Hemophilus influenzae Type B Vaccine Era: Changing Trends.” The Laryngoscope. 2004;114(3): 557-60

Stroud RH et al. “An update on inflammatory disorders of the pediatric airway: epiglottitis, croup, and tracheitis.” Am J Otolaryngology.  2001;22(4):268-275

Image of the Week: 7/24


A 6 month-old male presents to the emergency room with progressively increasing work of breathing. A chest x-ray obtained shows the following:


Chest Xray to review

Case courtesy of Dr Jeremy Jones, From the case rID: 24086

What are you concerned about?




This X-ray demonstrates cardiomegaly with prominent lung markings, which is suggestive of heart failure as the etiology of this infant’s respiratory distress.


Heart Failure in Children: The Basics

  • In the US, the most common cause of pediatric heart failure is structural congenital heart disease. In children with structurally normal hearts, cardiomyopathy is the most common cause of heart failure. [Hsu et al, 2009]
  • Heart failure presents when the metabolic demands of the body exceed cardiac output.
  • Clinical Presentation is Diverse & Age related. [Madriago et al, 2010]
    • Neonates: Feeding Difficulties and ultimately Failure to Thrive
    • Older Children: Fatigue, GI symptoms, and exercise intolerance
  • Physical Exam:
    • “Pulmonary”: Tachypnea, Respiratory Distress (retractions, grunting), crackles on auscultation
    • “Cardiac”: Tachycardia, gallop rhythm
    • “GI”:  hepatomegaly

Faculty Reviewer: Kristin Lombardi, MD
Radiology Reviewer: Laura Sternick, MD


Hsu DT et al. “Heart Failure in Children, Part I: History, etiology, and pathophysiology.” Circ Heart Fail. 2009;2:63-70

Madriago E et al. “Heart Failure in Infants and Children.” Pediatrics in Review. 2010;31(1)4-12.

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