• 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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• CNSD, also known as toddler's diarrhea
• the most common cause of chronic diarrhea in childhood
• possibly a variant of irritable bowel syndrome, family history of IBS is common
• children are 6-58 months of age, most commonly 11-24 months old)
• otherwise in good health with normal weight gain, without signs of malabsorption syndrome or enteric infection
• morning stool is the most formed, with the stools becoming progressively looser through the day that are malodorous with food particles
• most often due to dietary factors causing altered gastrointestinal motility such as reduced fat intake or excessive fluid intake, especially sucrose-containing fruit juices
• treatment is based on modification of the contributing dietary factors

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Noninvasive ventilation use in children has been shown in some trials to be a useful tool to avoid intubation in children with asthma.

Since children with asthma who are intubated have a much higher risk for complications including pneumotharaces and pneumomediastinum this can be a very useful tool.

Bi-Pap is usually started with typical settings of 10 for IPAP and 5 for EPAP and can be titrated up as tolerated to levels of up to 25/20 cm H2O and can be delivered with a set rate or a back up rate.

Albuterol and nebulized epiephrine may be delivered through newer BiPAP machines.  

Signs that BiPAP is working include decreased Respiratory Rate, decreased retractions and accesory muscle use, improved oxygenation saturation

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• Characterized by proteinuria, hypoalbuminemia, edema, and hypercholesterolemia
• Abnormal Vitals:  tachypnea (due to pulmlonary edema); tachycardia (intravascular depletion); hypertension
• Abnormal PE:  peripheral edema, ascites, S3 on ausculation
• UA demonstrates significant proteinuria.
• TX is uniformly with oral steroids.

• Rates are highest for children <5yrs and between 15-24 yrs old.
• Most of pathology is related to duration of asphyxia from time of submersion until adequate respiration is restored.
• The brain and heart are most vulnerable to anoxic and ischemic injury.
• Prognosis for near-drowning depends primarily on the degree of brain anoxia.
• Prolonged submersion (>25 min); apnea or coma at presentation to ED; and initial arterial pH <7.0 are all poor prognostic indicators.
• 96% of victims who require <10min of CPR survive with no or only mild neurologic impariment.

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Classic presentation:  breastfeeding failure with umbilical stump and gastrointestinal bleeding by postnatal day 7.  Oozing from circumcision, venipuncture, and heel sticks is also common.  Beware bleeding into the scalp or intracranial space.

Due to essential vitamin K deficiency which exists at birth as the fetus receives little vitamin K from the uteroplacental circulation.  It is responsible for impaired neonatal clotting function (deficiency of factors II, VII, IX, and X).

Prevented by a single intramuscular dose of 1mg vitamin K in the first few hours following delivery.

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Pancytopenia manifests as a decrease in the erythroid, myeloid, and megakaryocytic cell lines that appears as a decrease in red blood cells, white blood cells, and platelents on complete blood count analysis. 

• Indicates bone marrow failure
• May be due to invasion of marrow by nonneoplastic (such as drugs, chemicals, irradiation, or infections) or neoplastic conditions
• Clinically manifests as pallor, easy fatigability, and weakness due to anemia; purpura, epistaxis, and bruising due to thrombocytopenia; and increased susceptibility to infection due to leukoopenia.

Pancytopenia is an absolute indication for bone marrow aspiration and biopsy to delineate and treat the cause.

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• Increasing use of OTC meds is a worldwide occurence with $3.5 billion each year spent in the US.
• About 4 million children younger than 12 yrs are treated with these meds each week in the US.
• In 2007 the FDA recommended that the use of OTC cold meds (antihistamines-brompheniramine, chlorpheniramine, diphenhydramine, doxylamine; antitussive-dextromethorphan; expectorant-guaifenesin; and decongestants-pseudoephedrine and phenylephrine) be prohibited in children < 6 yrs.
• A recent review of 103 childhood deaths due to OTC meds found that most deaths were from product misuse rather than adverse effects resulting from recommended doses particularly when the product was used with the intent to sedate a child. 
• Children less than 2 years old were most susceptible to death using these products which is why manufacturers voluntarily withdrew the use of OTC meds in this age group.

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• Scabiess requires sensitization to the organism, Sarcoptes scabiei. 
• It may take weeks before pruritus develps in a child infested for the first time.  On the next exposure, however, INTENSE itching will occur within 24 hours. 
• Burrows in the webs of fingers and toes are common.
• Treatment:  Firstline is permethrin 5% cream on the entire body from the neck down, and wash off after 12 hours.  Alternative is lindane 1% (1oz of lotion or 30g of cream) applied in a thin layer over the entire body from the neck down, and thoroughly washed off after 8 hours OR ivermectin 200ug/kg orally repeated in 2 weeks.
• Many avoid lindane because of neurotoxicity.  Do not apply it after a bath, or to someone with extensive atopic dermatitis as seizures have been reported.
• Decontaminate all bedding and cloting.
• Warn patients that the rash and itching may persist for up to 2 weeks after treatment.

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As we head into the warmer months we should remember that every year there are reports of a toddler left in his car seat for 15 min who comes in uresponsive with hyperthermia. 

Heat related illnesses are a continuum from heat cramps to heatstroke.  The hallmark of heatstroke is hyperthermia with mental status changes and when identified rapid cooling must be initiated.  Mortality for heatstroke is reported as high as 80%. 

Children are more susceptible to heat stroke because of a greater surface area to body mass ratio, higher metabolic rates, less developed sweating mechanisms, and inability to always remove themselves from the hot environment.

The quickest and easiest way to cool a conscious patient is by evaporation.  Changing water from a liquid to a vapor is an endothermic process.  Removal of all clothes, followed by misting or wiping with tepid water of the entire skin is very effective.  Having a fan pointed at the child can enhance this method.   

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Hemolytic-uremic syndrome (HUS)

• Characterized by hemolytic anemia (pallor on exam), acute renal failure (oliguria or anuria by history), and thrombocytopenia (petechiae).
• HUS is one of the most common causes of acute renal failure in children.
• Two types: diarrhea-associated (shiga toxin+ or D+) which is more common and has a more favorable prognosis, and non diarrhea-associated (atypical or sporadic or D-).
• Most common age at presentation is during infancy or young childhood.
• Pediatric HUS is a true medical emergency.
  • Resuscitation with blood products frequently is required, but it is crucial to provide volume carefully because renal function may be severely compromised.
  • Dialysis is required if anuria persists for 12+ hours or for severe hyperkalemia (>6.5mEq/L) Some patients may benefit from plasmapheresis, but full renal recovery is not certain.

Parainfluenza viruses (types 1, 2, 3) account for more than 65% of all cases. The different serotypes have seasonal patterns, with type 1 and 2 occuring in the autumn and being the most common pathogens associated with croup while type 3 is more frequent in the spring and summer and is associated with pneumonia and bronchiolitis.

Infections are rarely associated with high fever and usually last 4 to 5 days. There are no distinctive laboratory abnormalities, and diagnosis is generally made clinically.  Chest and neck xray may demonstrate a “steeple sign” from narrowing of the subglottic region.  Viral cultures and immunofluorescent rapid antigen identification can be obtained from respiratory secretions.  Specific antiviral therapy is not available. Aerosolized epinephrine can be given to severely affected, hospitalized patients to decrease airway obstruction.  Parental (>0.3mg/kg) and oral ((0.15mg/kg) dexamethasone have been demonstrated to lessen the severity and duration of symptoms and hospitalization in patients with moderate to severe croup.  
 

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• For children under 5 years of age the rate of missing an appendicitis remains very high.  (57%-67%)
• The rate of misdiagnosis increases as the age decreases. 
• In cases of missed appendicitis the most common incorrect diagnosis is gastroenteritis.
• Think twice before you label vomiting alone, or diarrhea alone as gastroenteritis.
• If an appendicitis is missed there is an increased risk of perforation, abscess formation, and higher morbidity. 

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Rocky Mountain spotted fever (RMSF)

Systemic small vessel vasculitis caused by R rickettsii which is transmitted by a tick bite.

Clinical features: fever, headache, myalgia, nausea, vomiting, and characteristic rash. Rash usually appears before the sixth day of the illness initially on the wrists and ankles, and spreads to the trunk within hours.  Initially. It is erythematous and macular, later becoming petechial.

Laboratory findings: thrombocytopenia, anemia, and hyponatremia.

Complications: meningitis, multiorgan involvement, DIC, shock, and death. 

Treatment: doxcycycline (even despite the risk of dental staining in children younger than 8 years old)

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• Pediatric seizures are common and 4-6% of all children will have a seizure by the time they are 16 years old.
• Afebrile neonatal seizures require an evaluation of electrolytes, glucose, calcium, magnesium, LP, blood and urine cultures.
• Simple Febrile seizures usually do not require any lab testing or admission if the child appears well.
• Dilution of formula with too much water is a common cause of hyponatremic seizures in infants.  (Treat with 3ml/kg of 3% hypertonic saline)
• Complex febrile seizures have a higher risk for meningitis than simple febrile seizures, so perform an LP, give antibiotics, and admit.
• When intubating for Status Epilepticus consider using thiopental or propofol for induction given their antiepileptic properties.

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• An acute bacterial infection of a joint.
• Peak incidence in children is younger than 2 years of age.
• Risk factors:
  • history of trauma
  • preceding URI
  • immunodeficiency
  • hemoglobinopathy
  • Diabetes.
• Age is the most important determinant of cause.
  • In all age groups, S aureus is the primary organism accounting for more than 50% of cases.
  • Among neonates, enteric gram-negative organisms and group B Streptococcus are the most frequent causes.
  • Group A Streptococcus, S pneumoniae, and K kingae are common causes in children younger than 5 years old.
• Blood culture, joint fluid aspiration and analysis, gram stain, and culture of fluid is recommended.
• In pyogenic arthritis, the joint fluid is usually cloudy and has a leukocyte count of at least 50 x 10000/mcL, with a predominance of polymorphonuclear cells, low glucose concentrations, and high protein values.
• Treatment involves a combination of parenteral antibiotics, surgical drainage, and decompression of the affected joint.
• All children who have pyogenic arthritis of the hip or shoulder require prompt open surgical drainage and irrigation to prevent permanent joint damage as the increased intra-articular pressure can compromise blood flow resulting in avascular necrosis of the femoral or humeral head and predisposing the patient to dislocations.
• Open surgical drainage of other joints usually is not required.

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Epstein-Barr virus (EBV)-associated infectious mononucleosis (IM) 

Most commonly presenting clinical findings: fever, fatigue, exudative pharyngitis, lymphadenopathy, and hepatosplenomegaly. 

Self-limited illness that lasts an average of 2 - 3 weeks. 

Treatment is primarily supportive.  Use of ampicillin, amoxicillin, or penicillin during the acute phase not indicated and may result in the development of a morbilliform rash, which studies have suggested may occur in more than 50% of the cases.  Antiviral therapy is not recommended. Splenic rupture occurs in about 1 - 2:1000 cases.  Therefore, avoidance of activities that increase the risk for injury is recommended until splenomegaly has resolved. 

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Bradycardia in children is most often caused by hypoxemia but can also be caused by acidosis, elevated ICP, vagal stimulation, heart blocks or overdoses. 

First degree heart block in otherwise healthy children can be caused by infectious diseases, myocarditis, rheumatic fever, Lyme disease and congenital heart disease.

Third degree heart block can be congenital, caused by maternal connective tissue disorders such as Lupus, or may result from cardiac surgery.

Any infant presenting with a third degree heart block should have an investigation for neonatal lupus. 

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The most common arrhythmias in children presenting to the ED are:

• Sinus tachycardia (50%)
• SVT (13%)
• Bradycardia (6%)
• Atrial Fibrillation (4.6%)

Atrial fibrillation in children is irregularly irregular with disorganized atrial activity with atrial rates ranging from 350-600 BPM. 

Children at increased risk of developing atrial fibrillation include those with underlying structural heart defects and hyperthyroidism.

Hemodynamically stable children have several treatment options including digoxin, amiodarone, propranolol, esmolol, or procainamide for ventricular rate control.

Hemodynamically unstable children need immediate synchronized cardioversion with 0.5 - 1 J/kg.  (don't forget light sedation.)

References:

Sacchetti A, Moyer V, Baricella R, et al. Primary cardiac arrhythmias in children. Pediatr Emerg Care 1999;15:95-98

Doniger S. Pediatric Dysrhythmias. Pediatric Emergency Medicine Reports. Sept 2008. Vol 13, No 9 (This was edited by a UMMS Combined EM/PEDS graduated Dr. Jim Colletti who is Associate Residency Director, Emergency Medicine, Mayo Clinic College of Medicine, Rochester, MN.)

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