UMEM Educational Pearls - Pediatrics

• 4-5% of Prepubertal Vaginal Complaints are the result of foreign body.
• Vaginal bleeding is the most sensitive (93%), and specific (82%)
• Discharge usually foul-smelling is only seen in 18% of patients
• Undiagnosed symptoms may be chronic, (case reports lasting years).
• Complications of delayed removal include infection, toxic shock syndrome, fistulas, adhesions and even infertility
• Exam in knee chest position, and removal with irrigation or tissue forceps.
• Failure to remove FB may require exam under anesthesia.

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The Emergency Department is often the first line in detecting the sexual abuse of a child.  Unfortunately, what you do or don't say/ask/test can significantly affect the legal protection of the abused child.

1. Know your region's dedicated sexual abuse center, if one exists.  These centers have personnel trained in interviewing and forensic evidence collection.  There may be different centers for children of different ages.

2. Know your state laws regarding what is and is not admissible as evidence of sexual abuse.  GC/CT urine testing (NAAT), though more sensitive than swab cultures, is not currently admissible as evidence in many states.

3. Withhold prophylactic antibiotic treatment when possible - antibiotics work well, and often eliminate evidence.  Withholding antibiotics is acceptable if the child is asymptomatic or only has very mild symptoms.

4. Any sexually transmitted disease in a child warrants further workup and investigation.  Primary genital HSV in a young child warrants testing for Gonorrhea and Chlamydia, and appropriate referral as well as police involvement.

5. Finally, if trained personnel is available to conduct the interview of a child, limit the questions you ask the child directly.  Any evidence in your note that you may have suggested something to the child in your line of questioning could negate the validity of their testimony.

Ductal-Dependent Cardiac Lesions in the Neonate

• Often present in the first 1-2 weeks of life (children born prematurely tend to be at the upper end of the spectrum as they may have delayed closure of the ductus arteriosus)
• May present with tachypnea, sudden onset of cyanosis or pallor (often worse with crying), diaphoresis with feeds, lethargy, or failure to thrive
• Oxygen challenge - place baby on 100% 02 via NRB;  10% improvement in SpO2 (or 30mmHg increase in PaO2 on ABG) suggests a pulmonary issue;  no or minimal change suggests a congenital heart defect
• If congenital heart disease is suspected, start PGE-1 infusion at a rate of 0.05-0.1ug/kg/minute;  improvement may be drastic and is usually seen within 15 minutes
• Side effects of PGE-1 infusion include apnea, fever, hypotension, and seizures;  have your code cart and intubation equipment ready to go prior to beginning infusion

Ring-removal is a dreaded problem in pediatric hand and finger injuries.  Removal can be difficult and time consuming.  The relatively recent introduction of Tungsten into the jewelry market has further complicated this problem:

• The hardest metal used in jewelry - cannot be scratched, much less cut, by common tools
• Cheap, easy to buy online, attractive to adolescents

However, it is:

• Extremely brittle
• May be safely and quickly broken with locking pliers (also cheap), by sequentially, gradually tightening the locking plier grip

This video explains how.  Of course, this works on adults as well.

http://www.youtube.com/watch?v=poM423pewRE

_{I have no relationship with the copany which made this video - it was simply chosen for its clear explanation of the solution described in this pearl.}

Conjunctivitis in Children:

• Usually a self-limiting process, very common in school-aged children and children in daycare or group home settings
• Crusting/swelling treated with warm compresses
• Antibiotics are not necessary outside of the neonatal period, but they do speed recovery
• Neonates with conjunctivitis - send cultures (gonococcal ophthalmia neonatorum);  all others - no cultures necessary
• Treatment with erythromycin ointment, Polytrim solution, ciprofloxacin solution (usually practicioner preference unless dealing with a recurrent/resistant case)

HOWEVER... remember to consider other common etiologies of a red eye in a child!

• Corneal abrasions
• Acute angle closure glaucoma (sickle cell patients)
• Allergic sources

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• comprehensive history and thorough external genital exam (often without direct visualization of the cervix) will lead to appropriate diagnosis
• estrogen withdrawal following birth or ingestion of oral contraceptives
• vaginal foreign bodies (such as toilet paper, small toys)
• bacterial infections (strep and shigella)
• trauma from sexual abuse or straddle injuries
• vascular lesions such as hemangiomas
• be careful to differentiate urethral bleeding from vaginal bleeding

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• characterized by paroxysms of severe vomiting without apparent cause separated by periods of complete health
• typically begins between 3 and 7 years of age
• family or patient history of migraine or irritable bowel syndrome often noted
• intentse vomiting with lethargy, fever, and headache preceding the onset of emesis
• episodes last up to 48 hours (but may last up to one week) with 4-12 episodes per hour, and end suddenly often after sleep
• two thirds of children become so dehydrated they require intravenous fluids
• most patients have stereotypic patterns of onset and triggering events
• rapid treatment with IVF and glucose, along with migraine treatments such as cyproheptadine, propanolol, and TCA's
• antiemetics often not effective

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While it is often ok to defer removal of pesky nasal foreign bodies until ENT follow up, if the foreign body may be a button battery, emergent identification and removal is indicated.

Damage can occur in 3 hours, and by 24 hours, near complete necrosis of turbinates and ala has been described.

• If the object may be a button battery, consider a plain film - if it doesn't show up, it isn't a battery, and you are in the clear.
• If you can clearly see the button battery, you can try to remove it - consider using a magnet if one is available - more on that in a future pearl.
• Lastly, if you cannot visualize the battery, if there is any evidence of content leakage, or if there is any tissue damage, emergently consult ENT for assistance - this is a surgical emergency.

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While breastfeeding is still the preferred source of infant nutrition by the AAP, a little-known fact is that breastfeeding may expose the nursing infant to environmental pollutants to which they might not normally be exposed.  If you have a mother that appears ill due to exposure to any of these agents, don't forget to have the infant examined as well for signs of intoxication.

• Breastmilk can contain approximately 20% of the maternal toxin load, which can produce more severe effects in the infant due to the vastly different dose/weight ratio
• Toxin load is usually due to the lipid solubility of agents
• Formulas are safe due to the nature of their fat sources;  cows usually have a much lower exposure rate to pollutants, and those that are ingested are much more dilute due to the volume of milk produced in comparison to a human female;  also, with non-cows'-milk formulas, the lipid components are usually plant-derived and thus also with a lower risk of exposure
• Common offending agents include:  DDT, PCBs, Dioxin, hexachlorobenzene, Halothane, carbon disulfide, nicotine, lead, methylmercury, Heptachlor, Chlordane, and tetrachloroethylene

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• although newborn screening for PKU has been routine throughout North America since the 1960's, it is not routine in undeveloped countries (beware immigrants, foreign visitors)
• PKU is caused by phenylalanine hydroxylase (PAH) deficiency which catalyzes the conversion of phenylalanine to tyrosine
• neonates with PKU typically show no physical signs of hyperphenylalaninemia
• children with untreated PKU have impaired brain development with poor brain growth, seizures, behavior problems, and severe mental retardation
• affected individuals exude a pungent, musty odor due to elevated phenylalanine levels which also causes skin conditions such as eczema
• because there is absent tyrosine production with reduced tyrosinase, the hair and skin are very lightly pigmented
• early diagnosis and management with a low-phenylalanine diet eliminates these complications; and once treated, affected children are healthy and do not require hosopitalizations

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• Although rare, it is a serious life threatening entity of pediatric emergency medicine
• Must be in the differential of those with signs of increased intracranial pressure or focal deficit and hx of sinusitis, mastoiditis or cyanotic congenital heart disease.
• Investigation and diagnosis primarily with CT scan
• CSF studies demonstrate sterile fluid with elevated protein, and mildly elevated WBC
• Antibiotic coverage should be broad Naficillin/Vanc + Ceftriaxone + Metronidazole, until speciation and susceptibilities obtained from surgical specimen
• Steroids reserved only in cases of imminent herniation
• Controversy exists over prophylactic anticonvulsants
• Mortality recently <10% attributed to early diagnosis and appropriate antibiotic coverage.

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You've probably long been taught that Sickle Cell Trait is an irrelevant piece of the PMH, unless you are a genetic counselor.  Well, thanks to Dr. Rolnick and a literature search, I (and now you) know that that is incorrect.

Though Sickle Cell Trait (SCT) does not cause exactly the same pathologies as Sickle Cell Disease (SCD), there are believed to be a variety of RBC abnormalities associated with HgbS (such as measurably lower RBC deformability, and low levels of sickling under extreme heat and exercise conditions) which contribute to increased exercise-related sudden death.  In one NEJM study of all deaths among 2 million (MILLION) military recruits over a 4 year period, the relative risk of otherwise unexplained sudden death for black recruits with HgbAS vs. black recruits without HgbS was 27.6 (p<0.001), and 39.8 (p<0.001) for all recruits (HgbAS vs. no HgbS).

I must say that this topic is not controversy-free, however, I should also note that my search for "Sickle Cell Trait and Sudden Death" turned up quite a few articles directed at plaintiff's attorneys. 

The take-home point is that SCT is likely not a benign condition, and you must be cautious in telling patients that it is.  Again, this phenomenon is best described in patients undergoing extreme physical exertion, but hopefully this will change how you think about SCT.

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Infantile Spasms (West Syndrome):

• Are brief contractions of the neck, trunk, arm, and leg muscles that last 2-10 seconds
• Are NOT seizures, but 86% of children with infantile spasms go on to develop a seizure disorder before 1 year of age
• Usually occur as the child is going to sleep or waking up
• Most commonly seen between 3 and 8 months of age
• Often mistakenly diagnosed as colic
• Poor prognosis as infantile spasms usually indicate an underlying genetic, metabolic, or developmental abnormality

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• Status epilepticus is defined as either a continuous convulsion or serial convulsions without loss of consciousness that lasts 30 minutes.
• First line treatment:  benzodiazepine because it is absorbed rapidly into the nervous system; lorazepam (0.05 to 0.1 mg/kg) is preferred over diazepam (0.2 to 0.5 mg/kg) because of its longer half-life in the CNS; rectal administration of the intravenous formulation or the commercially available gel at the same doses may be subsitutued if no IV is attainable.
• if seizure activity persists beyond 10 - 15 min, a longer acting anticonvulsant such as phenytoin (18 -20 mg/kg), fosphenytoin, or phenobarbital (18 - 20 mg/kg) is administered; they take longer to penetrate the CNS, but have much longer half-lives than the benzodiazepines.  Phenobarbital is given to infants while phenytoin or fosphenytoin is given to older children.
• Fosphenytoin, a prodrug to phenytoin, increasingly is replacing phenytoin as the drug of choice.  It can be administered at two to three times the rate of phenytoin and is less caustic to skin in teh event of vein extravasation.  It can als be given intramuscularly, while phenytoin can't.

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Hypertensive encephalopathy is generally seen in children with renal disease, e.g. acute glomerulonephritis or ESRD. 

Signs and symptoms include bp >99th percentile for age and height and neurologic impairment.  May present acutely with seizure or coma, or subacute with HA, vomiting, lethargy, blurry vision or change in mental status.  Exam findings may also include papilledema.

MRI may show increased signal in occipital lobes of T2 weighted images, known as reversible posterior leukoencephalopathy.

Treatment is to lower BP by 20-25% for the first 8 hours and to normative levels over 24-48 hrs.  IV therapy with esmolol drip, labetalol or nicardapine are the treatments of choice.  Nitroprusside prudent in most hypertensive adult emergencies must be used cautiously  if history of renal disease secondary to cyanide toxicity. Seizure should also be treated as you would with status epilepticus.

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PEARL: Any patient that in your Emergency Department with a sickle cell disease (SCD)-related diagnosis requires incentive spirometry and frequent monitoring for acute chest syndrome (ACS).

BRIEF WHY: ACS is the most common cause of hospitalization and death in patients with SCD.^1,2 

Nearly half of all patients who develop ACS are admitted for diagnoses other than ACS.  Of those not admitted with ACS, radiographic and clinical findings of ACS appeared a mean of 2.5 days after admission.² It is because of this that all patients with SCD related diagnoses at presentation, must be treated as though they are in the prodrome stage of ACS, and all require incentive spirometry to reduce the risk of progression to ACS.²

More to come... 

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Pertussis (Whooping Cough):

• Caused by B.pertussis and B.parapertussis
• Incubation period = 6 days
• Three stages:  Catarrhal (low-grade fever, rhinorrhea);  Paroxysmal (classic "whooping" cough);  Convalescent (resolution of symptoms over a ~2wk period)
• Full course of the disease = on average 6-8 weeks, although convalescent stage may last MONTHS
• Erythromycin may be effective early on, but no effect once in the paroxysmal stage
• Complications are most common in neonates and infants, and notably, the elderly
• Complications include apnea, hypoxia, pneumonia, encephalopathy, pneumothorax/pneumomediastinum (from paroxysms in the setting of severe mucus plugging)

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• peak age of coin ingestion is 1-3 years, 60% being males
• CXR is recommended, and if in esophagus the flat surface of coin is seen on the AP view with the edge seen on the lateral view
• if in the stomach, expectant observation (3-4 days) in the absence of abdominal pain and vomiting
• 20% of coins will lodge in the esoophagus at the level of the cricopharyngeus muscle, aortic arch, and lower esophageal sphincter
• the change in composition of pennies from cooper to zinc in recent years has increased the potential for mucosal corrosion
• all coins lodged in the proximal esophagus should be removed endoscoopically as soon as possible
• coins in the mid- to lower esophagus may be observed for 12-24 hours if asymptomatic, but should undergo endoscopy if the coin fails to pass in that time period

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• triad of chronic sinusitis, bronchiecctasis, and situs inversus
• recurrent acute otitis media and nasl polyps also common
• syndrome is due to an ultrastructural abnormality of the cilia in which dynein arms are absent resulting in ciliary dysfunction and mucus stasis and frequent sinopulmonary infections
• situs inversus is due to the absence of the influence of cilia function on viscera development
• diagnosed by bronchoscopy for cilia biopsy

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• results from failure of the upper femoral epiphysis, which allows displacement of the femoral head on the femoral neck
• onset may be sudden, but more often is gradual
• pain frequently is referred to the knee, but can also occur in the hip
• limp and out-toeing are common, with loss of medial hip rotation
• majority of patients are 7-15 years old, and are aboce the 95th percentile for weight
• AP or frog-leg lateral xrays of the hip are diagnostic

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