• vasculitis of small vessels with neutrophilic infiltration of venules and arterioles
• classic triad:  painful recurrent oral and genital ulcers with inflammatory eye disease
• key finding of recurrent buccal apthous ulcers (nearly 100% of patients)
• diagnosis is made when recurrence of oral ulceration occurs at least 3 times in 1 year plus 2 of the following: recurrent genital ulceration , eye lesions, skin lesions, or positive pathergy test.
• initial ED treatment is corticosteroids (oral or topical).  Reserve colchicine and pentoxifylline for ulcerative maifestations.

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• causes gastric outlet obstruction and vomiting
• 1 in every 500 infants; with a 4:1 male-to-female ratio and a family history in another sibling
• symptoms begin 2-4 weeks after birth, with projectile NON-bilious vomiting
• firm, mobile, nontender, olive-shaped mass in right hypochondrium or epigastric area
• diagnosis confirmed with US or upper GI series
• treatment is a pyloromyotomy, but fluid and electrolyte replacement is vital in ED

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• ocurs with significant lateral traction during vaginal delivery of an infant
• results in damage to the upper part of the brachial plexus, especially the 5th and 6th cervical roots
• results in paralysis of hte shoulder and arm
• the affected arm is held in adduction and internal rotation
• most resolve spontaneoulsy, but some may require physical therapy after 2 weeks
• surgery is rarely required, and has poor results
• always palpate for ipsilateral clavicel fractures!

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• 0.3-1.5% of all pediatric DKA cases
• 21-24% mortality rate
• usually at 4-12 hours after therapy starts
• risk factors:  <5years old, new onset diagnosis, increased BUN at presentation, severity of acidosis at presentation, bicarbonate use
• have low threshold to diagnose and treat:  don't wait to treat for the CT!

• newborns have a thick right ventricle resulting in a mean QRS axis which points anteriorly and to the right demonstrating a right axis deviation (70-180degress) and large R waves in the precordium
• by 3 months of age, the QRS axis in the frontal plane shifts to the left with a mean of 65degress (0-125degress)
• by older childhood, the normal mean QRS axis is -30-100degress)
• thus, with age the R wave decreases in V1 and increases in V6
• take home:  right-axis deviation is often a normal finding in children and young adults when you see left-axis deviation in children consider tricuspid atresia, atrioventricular septal defects, and LVH as the most associated conditions

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• occurs when the small opening in the abdominal muscles which allows passage of umbilical cord does not completley close after birth
• allowing intestinal loops to pass through the opening
• 10% of all children are affected
• more common in blacks, girls, and premature infants
• most resolve by age 1year, but consider outpatient referral if becoming larger or still present after 2-3 years of age
• emergent consultation if not reducible, but rarely as most are harmless

• radial head subluxation
• usually 1-3 years of age
• often after sudden longitudinal traction on extended arm with wrist in pronation
• tearing of annular ligament attachment to radial neck, with detatched portion trapped between subluxed raidal head and capitellum
• children refuse to use affected arm and hold in a flexed pronated position
• traditionally, reduce by supination of forearm with elbow in 90degrees of flexion
• newer reduction technique, hyperpronation with elbow flexion has better success rateand less pain

• second most common vasculitis of childhood
• leading cause of acquired heart disease in children
• usually in children <5years old
• year-round with clusters in spring and winter
• highest incidence in children of asian decent
• clinical diagnosis requires fever for at least 5 days and a minimum of 4 of the following:

1. bilateral conjunctival injection without exudate
2. rash (often macular, polymorphous with no vesicles, most prominent in perineum followed by desquamation
3. changes in the skin of the lips and oral cavity (red pharynx, dry fissured lips, strawberry tongue)
4. changes in the extremities (edema, redness of hands and feet followed by desquamation)
5. cervical lymphadenopathy

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• seborrheic dermatitis is most common in infants within the first two months of birth
• appears as  erythematous, greasy yellow scales most commonly on the scalp (cradle cap), and may also occur on the face
• most cases resolve spontaneously within weeks to months, but severe cases may be treated with 1% hydrocortisone cream, sahmpooing with selenium sulfide, and using an emollient to remove scales
• in extreme cases, consider hte possibility of Langerhans cell histicytosis, especially if atrophy, ulceration, or purpura are also present
• rarely occurs in children between 1 and 12 as they do not have active sebaceous glands, but will appear as dandruff in adolesecents
   

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•  disorder in which the entire left side of the heart is underdeveloped
•  the right side of the heart is dilated and hypertrophied, and supports both the systemic and pulmonary circulations via PDA
•  accounts for nearly 1/4 of all cardiac deaths in the first year of life
•  infants present within the first days or weeks of life acutely ill with signs of CHF
• PE often shows cyanosis and poor pulses but hyperdynamic cardiac impulses
• CXR shows cardiac enlargement and prominent pulmonary vasculature
• EKG shows RA and RV hypertrophy
• echo is diagnostic
• acute treatment is PGE1 to maintain the PDA.

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• syndrome of hematuria at the END of urination
• evidenced by spotting of blood in underwear
• occurs only in boys
• may last up to a year or longer
• symptoms are usually intermittent and recurrent
• physical examination is normal
• renal ultrasound usually helps rule out structural anomalies, but will usually be normal
• self-limited, with no specific therapy other than reassurance
   

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• occurs in 1/1000 live births, but found in 15% of neonatal autopsies
• usually weigh <2500 grams at birth with prematurity the most common risk factor
• present with bleeding from the nose and mouth with severe respiratory distress
• immediate treatment with tracheal suctioning, oxygen, and positive-pressure ventilation
• ventilation goal is to maintain a high, positive expiratory pressure of at least 6-10cm H20
• also check for and correct any underlying bleeding disorders
• extremely high mortality, but no long-term pulmonary deficits if the infant survives

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Necrotizing Enterocolitis

• NEC is an inflammatory lesion of bowel which can progress to intestinal gangrene, with perforation, and /or peritonitis
• characterized by abdominal distension, feeding difficulties, and GI bleeding
• mainly affects pre-term infants, and most commonly affects distal ileum and proximal colon
• usually presents during the first 2 weeks of life, but may occur up to 3 months of age in infants who who born weighing <1000grams
• classic finding on abdominal XR is pneumatosis intestinalis or air in the bowel wall (pathognomonic) and is present 50-75% of the time
• treat emergently with nasogastric decompression, IVF recussitation, NPO, and IV antibiotics

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Colic

• excessive, unexplained paroxysms of crying in an otherwise well-nourished normal infant
• lasts >3 hours/day, and occurs >3 days/week...ughh!
• usually occurs at the same time of the day or evening
• usually resistant to most attempts to quell it
• infant may have excess flatus and draw legs up during episodes (but don't change formulas)
• beings in first week of life and ends by 4 months of age

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• most common cause of low platelets in children
• immune-mediated destruction of circulating platelets
• acute ITP peak incidence between 2-5 years of age; chronic ITP peaks in adolescence
• recent history (1-6 weeks) of viral infection or immunization is common
• no hepatosplenomegaly
• low platelets with megathrombocytes on smear, with normal hemoglobin (which differentiates from TTP, HUS, and DIC)
• nearly 90% of children will have normal platelet counts in 6 months
• treatment reserved for platelet counts <20,000 or significant bleeding:  IVIG (best response rate of 95%), corticosteroids (79% resposne rate), anti-rH (D) immunoglobulin (82% reesponse reate)

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• newborns have increased rates of bilirubin production due to RBC's with shorter life spans, and a decreased rate of bilirubin elimination due to decreased ability of the neonatal liver to conjugate bilirubin
• about 60% of newborns will become clinically jaundiced
• bilirubin levels peat at 4 days of life,  and may not decline before day 7
• admission and treatment should be considered urgently when serum total bilirubin >25mg/dL, with exchange transfusion if it is >30mg/dL or the infant has signs of kernicterus
• there are nomograms which plot the bilirubin level according to the infant's age in hours to determine if an infant is at risk for being at toxic levels
• the most common pathologic etiologies are due to increased bilirubin production: blood-group incompatibilities, RBC-enzyme deficiency, and RBC structural defects
• when jaundice occurs between days 4-7, strongly consider sepsis, UTI, congenital infection (syphilis, CMV, etc)

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• autossomal dominant disorder most commonly in Scandinavian and British descent due to deficiency of HMB-synthetase
• most heterozygotes are asymptomatic unless some factor increases the production of pyrogens, usually medications
• common drugs include steroids, alcohol, low calorie diets, and drugs (barbituates, sulfonamide antibiiotics, grisefulvin, and synthetic estrogens (birth-control)
• attacks of abdominal pain lasting several hours is the most common symptom and may be secondary to ileus or distension, but tenderness on exam and fever are absent
• peripheral neuropathy and muscle weakness improves over days, but may take years to return to normal
• diagnose: gold standard test measures RBC HMB-synthetase, screening test of normal PBG (porphobilinogen) level in urine rules out the condition
• treatment: narcotics, IV glucose (300g/day), and IV heme (4g/day)
   

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• in the US, the right of an adolescent (<18yrs) to seek and receive treatment without parental consent varies from state to state.
• usually, the right to self-consent for treatment is specified through public health statutes when there is clinical suspicion of a STD
• many states allow minors to seek help for pregnancy, contraception, substance abuse, and mental health issues without parental consent

some absolutes or almost always cases include the following:

1. emancipated minors: moved outside of the home and support themselves financially, married, in the military, or has a child
2. emergencies: patient is unconscious or unable to give consent
3. mature-minor: possess the ability to comprehend the risks and benefits of treatment/therapy

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• seizure disorder occuring in infants and children <1 year of age
• mostly occur between ages 4-8 months
• classic spasm is sudden, simultaneous flexion of the head and trunk with felxion and adduction of the extremities (salaam attack Blitz-krampf)
• occurs in clusters of diminishing severity
• initiated or aggrevated by transition from sleep to wakefulness or emotions
• EEG demonstrates hypsarrhythmia: high-voltage, irregular, slow waves occuring out of synch with multiple foci
• most resolve over time without therapy, but most children have some level of mental retardation or other seizure disorder

• also known as acute cerebellitis of childhood
• most commonly affects children 2-6 years old
• about 50%  have a history of recent URI or viral GI illness
• abrupt onset of ataxia which may be mild to severe, and findings usually include hypotonia, tremor, horizontal nystagmus, and dysarthria
• child often is irritable with nausa/vomiting
• sensory exam and DTR's are normal
• CT and MRI are normal
• CSF usually demonstrates an increase in WBC, with a predemonance of lymphocytes
• 90% recover without any specific therapy in 6-8 weeks (steroids are not indicated).