UMEM Educational Pearls - Pediatrics

Passive leg raise (PLR) has been studied in adults as a bedside tool to predict volume responsiveness (see previous pearls from 5/7/13 and 6/17/2008). Can this be applied to children?
 
A single center prospective study looked at 40 intensive care patients ranging in age from 1 month to 12.5 years.  They used a noninvasive monitoring system that could measure heart rate, stroke volume and cardiac output.  These parameters were measured at a baseline, after PLR, after another baseline and after a 10 ml/kg bolus.
 
Overall, changes in the cardiac index varied with PLR.  However, there was a statistically significant correlation in children over 5 years showing an increase in cardiac index with PLR and with a fluid bolus.
 
Bottom line:  In children older then 5 years, PLR can be a quick bedside tool to assess for fluid responsiveness, especially if worried about fluid overload and in an under served area.

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Inborn errors of metabolism (IEM) are rare, each typically affecting 1 in 5000 to 1 in 100,000 children, BUT collectively these disorders are more common because there are so many. If you are lucky…when they present to the ED they come with a letter from Dr. Greene (our world renowned metabolic geneticist) detailing exactly what to do. The rest of the time…you are on your own. Think about IEM in any neonate or child with history of feeding difficulties, failure to thrive, recurrent vomiting, unexplained altered mental status and/or acidosis. Pay particular attention to feeding difficulties that appear with changes in diet: switch from soy to cow’s milk formula (galactose), addition of juice or fruit or certain soy formulas (fructose), switch from breast milk to formula or foods (increased protein load), and longer fasting periods from sleeping or illness.

For this pearl, we will focus on primary hyperammonemia from an enzymatic block in ammonia metabolism within the urea cycle. It is important to remember that secondary hyperammonemia can result from metabolic defects such as organic acid disorders, fatty acid oxidation disorders, drugs that interfere with urea cycle, or severe liver disease. Amino acids liberated from excess protein breakdown (stress of newborn period, infection, injury, dehydration, surgery, or increased intake) release nitrogen which circulates as ammonia. Ammonia is then converted to urea via the urea cycle and excreted in the urine. With urea cycle defects (UCD) there is an enzymatic block in the cycle that results in accumulation of ammonia which has toxic effects on the CNS especially cerebral edema. The most common UCD is ornithine transcarbamylase deficiency followed by argininosuccinic academia, and citrullinemia.

Clinical presentation includes poor feeding, lethargy, tachypnea, hypothermia, irritability, vomiting, ataxia, seizures, hepatomegaly, and coma. Hyperammonemic crises in neonates mimic sepsis! If you think about an IEM in your differential, send plasma ammonia (1.5 mL sodium-heparin tube on ice STAT), plasma amino acids, and urine organic acids. Other helpful labs include blood gas, CMP, urinalysis (looking at ketones), lactate, plasma acylcarnitines, and newborn screen if not already sent. Plasma ammonia is a direct index of CNS toxicity and important to follow for acute management. Serum level > 150 in sick neonate or > 100 in sick infant/child is concerning for IEM. The presence of hyperammonemia and respiratory alkalosis suggest urea cycle defect. The presence of metabolic acidosis and hyperammonemia suggests organic acid disorder.

Immediate treatment of hyperammonemia is critical to prevent neurologic damage. Cognitive outcome is inversely related to the number of days of neonatal coma caused by the cerebral edema.

1. Stop all protein intake! You need to stop catabolism.

2. Start D10 at 1.5 times maintenance rate with GIR at least 6-8. Start intralipids 1-3g/kg/day when able (typically in the ICU after central line placed).

3. Give ammonia scavenger medications sodium benzoate and sodium phenylacetate. These are available commercially as Ammonul.

     a. 0-20kg: 2.5mL/kg IV bolus over 90 min followed by same dose as 24 hr infusion

     b. >20kg: 55 mL/m2 IV bolus over 90 min followed by same dose as 24 hr infusion

4. HEMODIALYSIS! Dialysis is the most effective way to remove ammonia and should be done when level > 300. The decision to hemodialyze is crucial in preventing irreversible CNS damage; when in doubt in the face of elevated ammonia, HEMODIALYZE!

• Much attention has been paid towards early goal-directed therapy for sepsis in adult ED patients, but there has not been as much consideration for the pediatric ED patient. 
• R-C analyses and M&M reviews have consistently identified system difficulties  recognizing sepsis in children, especially cases of compensated shock, and subsequent management.
• Protocols beginning in triage to recognize abnormal vital signs, followed by timely execution of interventions especially antibiotic and fluid administration are worthwhile to reduce overall morbidity and mortality.
• Protocols should include 3 major goals:

1. Triage vital signs adjusted for age, and corrected heart rate for pyrexia to recognize sepsis.
2. Obtain vascular access within 5 minutes followed by a 20mL/kg bolus of IV fluids administered within 15 minutes in cases of volume depletion.
3. Antibiotic administration within 30 minutes.

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Head lice infestation is a common problem in the United States with treatment costs estimated at 1 billion dollars and cases affecting millions of children each year.  Many of these children present to the ED for care...lucky us!  Traditional therapies containing permethrin and pyrethrins are having increased rates of treatment failure likely secondary to increasing resistance and medication noncompliance.  The typical first line agents require multiple doses.  There are safety concerns regarding therapies that contain malathion and lindane in children.  Is there another option?  Topical ivermectin 0.5% lotion applied to scalp in a single dose has been shown to be effective and safe for treatment of head lice infestation in children older than 6 months.  It was FDA approved at the end of 2012.  Considerations include cost. Sklice lotion is expensive!  

The NEJM article was considered an "editors pick"  by the AAP as one of the best articles of 2012-2013.

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• Significant morbidity and mortality has been consistently documented in pediatric sickle cell patients due to overwhelming sepsis from encapsulated organisms, especially S. pneumoniae
• All pediatric sickle cell patients presenting with fevers greater than 101.5F (38.6C) should receive antibiotics within 60 minutes of triage.
• Historically, and still in many pediatric sickle cell centers, ceftriaxone (75mg/kg/dose) is administered
• However, reported cases of deadly intravascular hemolysis in pediatric sickle cell patients whom had recieved multiple doses of ceftriaxone has led to new recommendations for antibiotic coverage to include cefuroxime (200mg/kg/day) or ampicillin/sulbactam (200mg/kg/day)

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• High or normal BP
• Normal heart rate after ROSC
• Sinus rhythm after ROSC
• Urine output >1 ml/kg/hr
• Noncyanotic skin color
• GCS >7 on arrival

Question

Case: A 3 year 9 month female presents with fever to 39.4 C and intermittent abdominal pain worsening over 2 days.  The patient had been tolerating food and had no change in her bowel habits.  Based on the imaging below, what is your diagnosis and treatment?

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How many times have you been frustrated in the peds ED when you have a child with a URI that has a significant night time cough and you feel like you have nothing to offer them for symptom control?  The parent is frustrated because the child is not sleeping which means they are not sleeping and they are looking at you for help.  We all know that OTC cough and cold medications are not helpful and may be harmful in children <2 yrs old and should be used with caution in children <6 yrs old.  So what can you do?  You can recommend a course of HONEY at night.  Of course this does not apply to children < 1 yr who are at increased risk of botulism.  A recent double-blind placebo-controlled trial published in Pediatrics in 2012 demonstrated reduced night time cough and subjective improved sleep quality in children age 1-5 who were given honey compared to placebo.  This study supports previous less rigorous publications that found honey was an effective remedy on cough in children.  Mechanism for honey's beneficial effect on cough is unknown but possibly related to close anatomic relationship between sensory nerve fibers that initiate cough and gustatory nerve fibers that taste sweetness.  Of note, a recently published survey in Pediatric Emergency Care revealed that 2/3 of parents were unaware of the FDA guidelines regarding OTC cough and cold remedies in children!  After you recommend HONEY for night time cough, take an extra minute and educate your parents about the potential dangers of cough and cold medicines in small children!

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Pediatric patients with an isolated skull fracture and normal neurological exam have a low risk of neurosurgical intervention and outpatient follow up may be appropriate (assuming no suspicion of abuse and a reliable family).  In a study published in 2011, a retrospective review over a 5 year period at a level 1 trauma center showed that 1 out of 171 admitted patients with isolated skull fractures developed vomiting.  This patient had a follow up CT showing a small extra-axial hematoma that did not require intervention.  58 patients were discharged from the ED within 4 hours.

You can also check out another recent article published in Annals of Emergency Medicine on the same topic this month!

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Should you be concerned about erythema around the umbilical stump?!

Yes!

Often parents will bring their neonate to the ED with concerns about the umbilical cord and it is just a simple granuloma or normal detachment. But is it omphalitis???

Omphalitis incidence is low in developed countries, but that means it’s easier, and no less catastrophic, to miss!

Omphalitis is a superficial cellulitis of the umbilical cord, but 10-16% progress to necrotizing fasciitis of the abdominal wall!!!

Always ADMIT and consider consulting surgery early in case of rapid progression…

Most often polymicrobial and should be treated with:

• Anti-staphylococcal PCN,  Vanc, & an Aminoglycoside
• Also consider adding Metronidazole or Clindamycin for anaerobic coverage
• Anti-pseudomonal coverage if toxic

Should notice improvement within 12-24 hours, so if don’t or begin to observe

• Fever
• Induration
• Peau d’orange tisse
• Tenderness
• Violaceous discoloration
• Crepitace
• Increased erythema
• Systemic signs of toxicity/shock

CONSULT SURERY for concern of necrotizing fasciitis which has a mortality rate of close to 60%!!!

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We have learned how to diagnose compartment syndrome in adults, but how do you determine the early warning signs in a nonverbal or even frightened child?  

Rising compartment pressures are related to increasing anxiety and agitation in children.  A Boston study in 2001 showed that increasing pain medication requirements were detected 7 hours earlier than a vascular exam change.  90% of the patients with compartment syndrome in this study reported pain, but only 70% had another ‘P” (pallor, parasthesia, paralysis or pulselessness).

This has led to the proposal of the 3 “A”s for early identification of compartment syndrome in children: increasing anxiety, agitation and analgesia requirement.

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A facial laceration on a child can present a unique challenge which is not limited to the initial visit.  The traditional teaching has been to use nonabsorbable sutures and have the patient return in 5 days for removal.  A recent study compared the cosmetic outcome of linear facial lacerations 1 to 5 cm that were closed with either Ethicon fast absorbing surgical gut or monocryl nonabsorbable sutures.  Patients were randomized and returned to the ED in 4-7 days and 3-4 months. Scars were assessed by caregivers and blinded physicians.  Results showed that caregivers preferred absorbable sutures.  Visual analog scores as given by caregivers were not statistically different between the 2 groups at the 3 month mark.  The blinded physicians did give better cosmetic outcome scores to the absorbable suture group which differs from previous studies that had shown equivocal results.  Of note, all absorbable sutures were no longer visible after 14 days.

Bottom line:  Try absorbable sutures the next time you are suturing a child and the parents may be happier and you will not have to try and take out your sutures from a squirming, screaming child.

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Clinically important traumatic brain injuries are rare in children.  The PECARN study provides decision rules for when to avoid unnecessarily obtaining a CT for children who have suffered head trauma.

For children < 2 years old: <0.02% risk of clinically important TBI

• Normal mental status
• No scalp hematoma, except frontal
• Loss of consciousness < 5 seconds
• No palpalble skull fracture
• Normal behavior
• Nonsevere mechanism (fall < 3ft, pedestrian struck, rollover MVC)

For children > 2 years old: <0.05% risk of clinically important TBI

• Normal mental status
• No signs of basilar skull fracture
• No loss of consciousness
• No vomiting
• No severe headache
• Nonsever mechanism (fall < 5ft, pedestrian struck, rollover MVC)

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Cringing at the thought of sewing up another screaming 2 year old?

Consider intranasal fentanyl.

Who: Young, otherwise healthy pediatric patients undergoing minor procedures (laceration repair, fracture reduction/splinting, etc...)

What: Fentanyl (2mcg/kg)

When: 5 minutes pre-procedure

Where: Intranasal

Why: More effective than PO, less invasive than IV while being equally efficacious.

How: Use an atomizer, splitting the dose between each nostril.

Lactate is commonly used in the adult ED when evaluating septic patients, but there is a lack of literature validating its use in the pediatric ED.  Pediatric studies have suggested that in the ICU population, elevated lactate is a predictor of mortality and may be the earliest marker of death.
 
A retrospective chart review over a 1 year period showed that one elevated serum lactate correlated with increased pulse, respiratory rate, white blood cell count and platelets.  Serum lactate had a negative correlation with BUN, serum bicarbinate and age.  Elevated lactate levels were higher for admitted patients. However, the mean serum lacate level was not statistically different between those diagnosed with sepsis and those that were not.
 
The study included 289 patients less then 18 years who had both blood cultures and lactate drawn.  This community hospital had a sepsis protocol in place that automatically ordered a lactate with blood cultures.  Only previously healthy children were included.
 
The study is limited by its small sample size and overall low lactate levels.  Despite having a protocol in place, only 39% of patients who had blood cultures drawn had lactate levels available for analysis.  The mean serum lacate in this study was 2.04 mM indicating that the study population may not have been sick enough to determine mortality implications.  There were no serial measurements.

 
Bottom line:  Consider measuring serum lacate in your pediatric patient with suspected sepsis.  Pediatric ICU literature does suggest that an serum lactate as low as 3mM is associated with an increased mortality in the ICU.

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Risk stratisfication score introducted by Maden Samuel in 2002.

The Pediatric Appendicitis Score had a sensitivity of 1, speciificity of 0.92, positive predictive value of 0.96, and negative predictive value of 0.99

Signs:

• Right lower quadrant tenderness = 2 points
• Cough/Percussion/Hop RLQ tenderness = 1 point
• Pyrexia = 1 point

Symptoms:

• RLQ migration of pain = 1 point
• Anorexia = 1 point
• Nausea/Vomiting = 1 point

Laboratory Values:

• Leukocytosis = 2 points
• Polymorphonuclear neutrophiia = 1 point

Scores of 4 or less are least likely to have acute appendicitis, while scores of 8 or more are most likely.

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