UW Peds Notes_@Pedunculated

UWorld notes Pediatrics

3/1/17

UW Peds Adolescence/misc Wednesday, February 15, 2017 12:58 AM 

Emergency contraception

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Ulipristal (like mefipristone in some sense), an antiprogestin that delays follicular rupture, inhibits ovulation, and impairs implantation Danazol is a testosterone derivative with progestin-like effects. This medication, in addition to GnRH agonists and progestins, is used for the treatment of endometriosis Dysmenorrhea:

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Gonococcal conjunctivitis: o Prevention: Application of topical erythromycin w/in 1 hour of birth

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Primary amenorrhea: o Isolated amenorrhea with well-developed 2ry sexual characteristics can be considered normal up to the age of 16.

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If karyotype is normal but clinical suspicion for Turner $ is high → FISH to detect mosaicism (eg, 45,X0/46,XX) that is beyond the resolution of standard karyotyping. Buccal smear for Barr bodies (inactivated X chromosomes) is an outdated and unreliable screening test for turner.

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Measles: o Rash spares the palms and soles o Rx: is typically supportive only. However, immunocompromised, pregnant, at extremes of age (eg, infants or the elderly), or who are vitamin A deficient are at greater risk for complications such as pneumonia, encephalitis, or blindness. o Treatment with vitamin A reduces the morbidity and mortality rates for patients with severe measles (eg, those requiring hospitalization) through the promotion of antibody-producing cells and regeneration of epithelial cells (eg, in the gut, lungs, and retina). HSV: o Fingers can get infected as well (+ lesions on the lips) forming what's called herpetic whitlow (vesiculopustular lesion)

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Kaplan says don't incise/drain the lesion for fear of delayed resolution, bacterial superinfection and systemic dissemination of disease. Conjunctivitis DD: o Trachoma:  Chlamydia trachomatis serotype A-C.  Major cause of blindness worldwide.  Active phase of the disease is characterized by follicular conjunctivitis and pannus (neovascularization) formation in the cornea.  Concurrent infection occurs in the nasopharynx→ nasal discharge.  Dx: Giemsa stain examination of conjunctival scrapings.  Rx: Topical tetracycline or oral azithromycin.  Repeated infections can lead to scarring of the cornea. o Herpes simplex keratitis:  Dendritic ulcer is the most common presentation. + pain, photophobia, and ↓ vision o Viral conjunctivitis:  Adenovirus type 3  Conjunctiva is red with copious, watery discharge  Children are more commonly infected through contaminated swimming pools

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Osteomyelitis:

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Empirical Rx in sickle cell disease pt w/ osteomyelitis = third generation cephalosporin (ceftriaxone) + anti staphylococcal (oxacillin, vancomycin) Vaginal F.B.: o Vaginal foreign bodies in children often present with foul-smelling vaginal discharge and vaginal spotting or bleeding. o Bimanual exam should never be performed in a prepubertal child as this may result in significant discomfort and trauma as the prepubertal vaginal introitus is narrow and the hymenal tissue is very sensitive. o Sedation/General anesthesia may be required to remove large foreign bodies, but a bimanual examination is not. o Toilet paper is the most common foreign body. Removal with a calcium alginate swab or irrigation with warmed fluid should be attempted after a topical anesthetic has been applied. Most common risk factor for orbital cellulitis? Bacterial sinusitis. Breast fibroadenoma:

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Although simple cysts are indistinguishable on physical exam from fibroadenomas, a cyst is most common in women age 35-50. In an adolescent, this diagnosis is less likely than a fibroadenoma. Fibroadenoma is the most common cause of breast mass in an adolescent

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Cat bites: o Most cat bites will become infected w/o abx prophylaxis

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N.B. cat scratch disease is treated w/ azithromycin (has activity against bartonella henselae). However, no need for prophylaxis in immunocompetent pt like in cat bites o Minor human or dog bites that aren't located on hands, feet, or genetalia in an immunocompetent pt can be observed w/ close f/u w/o Abx prophylaxis. #Ig hands, feet, and genetalia bites introduce bacteria on the skin as these areas are commonly contaminated :/ Neonatal conjunctivitis: o Dacryostenosis is the most common cause of neonatal eye discharge due to poor drainage of tears through the nasolacrimal duct. Rx: gentle massage

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CDC recommends that all pregnant women should be screened for chlamydia at the first prenatal visit. Repeat testing in the third trimester in all high- risk women (eg, age Lt renal artery. o Angiography shows a "string of beads" pattern to the renal artery. Viral myocarditis: o Viral injury → myocyte necrosis w/ systolic and diastolic dysfunction.

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Chest x-ray cardiomegaly (N.B. age 1 year and adults nl cardiothoracic ratio is 55%)

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Heart murmurs: benign vs pathologic o Maneuvers that ↓ VR (eg, standing, Valsalva maneuvers) typically ↓ innocent murmurs.

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Carotid pulse with dual upstroke on physical exam? HCOM, due to midsystolic obstruction during cardiac contraction Turner $ has aortic root dilatation w/ increased risk of dissection.

UW Peds dermatology Saturday, February 11, 2017 7:04 AM 

Child abuse burns: o Deliberate scald injuries are characterized by:  sharp lines of demarcation  uniform burn depth  spared flexor surfaces  Delayed seeking medical advice o In contrast, splash marks, poorly defined wound margins. non-uniform burn depth. and immediate presentation after injury are all features of accidents.

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Atopic dermatitis:

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Eczema herpeticum

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Scabies: caused by Sarcoptes scabiei infestation. Small pruritic papules in a linear arrangement (burrows) favor the web spaces, wrists and ankles, genitals, nipples, and waistline. o

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Neonatal rashes: o N.B. neonatal varicella: post-exposure prophylaxis (exposed to infected mother) → VZIG but in neonatal varicella (actual infection with vesicles etc) → acyclovir o Erythema toxicum: spares palms and soles. Can change appearance 

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SSSS:  The toxins target desmoglein 1, which is responsible for keratinocyte adhesion in the superficial epidermis  Prodrome of fever, irritability, and skin tenderness.  Erythema starts on the face, and generalizes w/in 24-48 hrs.  Subsequent desquamation for about 5 days, and the entire process usually resolves within 1-2 weeks.  Cultures from intact bullae are usually sterile, because this is a toxin mediated process  The goal of treatment is to eliminate any focus of infection with antistaphylococcal antibiotics, and supportive wound care.  SSSS is primarily a disease of children, but adults with renal disease or immunocompromised may also be affected. The mortality rate is low in pediatric patients, but can be quite high in adults.

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Tinea o All tineas (corporis, cruris, mannum, pedis) are treated w/ local therapy except capitis (hair) and unguinum (nail) which need systemic therapy. Dx can be made by KOH prep of skin scraping.

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versicolor

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Sunburn o Facts:  All skin colors are at risk of cancer when exposed to UV light  Water washes off sunscreen, even those that are water-resistant  No sunscreen for babies < 6 months. Due to ↑ exposure to chemicals in high surface area to body wt ratio  No evidence that steroids are helpful in Rx of sunburn.  Sunscreen protection factor SPF: ↑ SPF = more protection from UV rays. However, SPF > 50% adds very little value.  UV rays are reflected off cement, water, and snow and can penetrate clouds. You can get burned even if standing in the shade.  Tanning bed significantly increase risk of melanoma.  Dark fabrics offer greater protection from UV rays.

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UW Peds endocrinology Wednesday, February 8, 2017 6:28 AM 

Constitutional short stature vs familial short stature growth charts

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Constitutional growth delay is characterized by a delayed growth spurt, delayed puberty, and delayed bone age. Refeeding $: o Risk factors: Chronic starvation and acute refeeding are potentially lifethreatening in pts w/ anorexia nervosa. o

Pathophysiology: Phosphorus is the primary deficient electrolyte as it is required for energy (adenosine triphosphate). Deficiencies in potassium and magnesium potentiate cardiac arrhythmias in a heart that is already atrophic from prolonged malnutrition.

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Prevention: Avoid aggressive initiation of nutrition without adequate electrolyte repletion

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Precocious puberty:

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MRI w/ contrast is indicated in all pts w/ central precocious puberty even in absence of neurological/visual symptoms and despite the fact that most central precocious puberty is idiopathic premature activation in girls GnRH agonist therapy to prevent premature epiphyseal plate fusion and maximize adult height potential. Girls age 20% of total bilirubin) in the first 2 months of life, clay-colored stools, dark urine, and an enlarged liver. Dubin-Johnson syndrome is usually asymptomatic with mild scleral icterus. Vomiting in babies

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Physiologic reflux  Clinical Dx  Rx: Small frequent feeds, Upright for 25 min after feeds  Prone position while awake (not asleep). Avoid tight diapers, knee chest position.  Improves @ 6 m when baby sits, resolves @ 1 yr  Pathologic GERD  Clinical Dx  Don't use Thickened feeds (obesity) or PPI (pneumonia, diarrhea) for physiologic reflux. pH probe & endoscope only for severe cases Pyloric stenosis: o Formula feeding is thought to cause gradual hypertrophy of the pylorus until symptom onset at age 3-5 weeks. Compared to breastfed infants, formula-fed infants have slower gastric emptying and consume more volume in less time. The increased gastric burden may stimulate growth of the pylorus muscle. Pyloric stenosis presents with postprandial projectile vomiting followed by hunger (e.g., "hungry vomiter")

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Meconium ileus is virtually diagnostic for CF. Although only 20% of patients with CF develop meconium ileus, almost all newborns with meconium ileus have CF Celiac disease:

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Iron deficiency anemia:  From malabsorption. Duodenal atrophy leads to decreased iron absorption. Rx: gluten free diet + iron supp  Dermatitis herpetiformis:  On knees, elbows, forearms, and buttocks Biliary cyst: choledocal cyst

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Can be congenital or aquired. Right upper quadrant mass o Slightly elevated amylase and lipase like any other biliary disease o Risk factor for cholangiocarcinoma, infectioin, and pancreatitis Radiolucent foreign body suspected and not seen on xray. Next step? CT Esophageal coins in asymptomatic patients can be observed for up to 24 hours after ingestion. If the patient is symptomatic (cough, vomiting, dysphagia) or the time of ingestion is unknown, the coin should be removed promptly by flexible endoscopy. Smooth F.B. located distal to the esophagus pass uneventfully in most cases and need only to be observed with stool examination and/or follow up x-rays to confirm excretion. Don't induce emesis or try to push it into the stomach (likely unsuccessful and causes more esophageal injury) o

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Milk protein allergy o The diagnosis is clinical and confirmed when bleeding ceases in response to dietary modifications. Visible bleeding should resolve within 3 days, but complete resolution of occult blood may take up to 2 weeks. Parents should be reassured that the prognosis is excellent and that almost all affected infants can tolerate dairy and soy products by age 1 year

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Painless hematochezia DD: milk protein allergy (v. common) vs meckle's diverticulum (rare)

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Ectopic gastric tissue secretes acid → mucosal ulceration → bleeding → can lead to severe anemia and hemorrhagic shock Cyclic vomiting syndrome: o Functional syndrome diagnosed after exclusion of all possible causes. Overlapping with migraine headache and abdominal migraine (cyclic functional abdominal pain with criteria similar to that of CMS. Family hx of migraine headache. Rx: hydration, antiemetic (odansetron), and reassurance; triptans can help pts w/ family hx of migraine. Resolves by age 5 -10 yo in 65% of cases. o

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Choanal atresia: first step is passing a catheter, next is to do a CT to reveal the bone structure. Next look for associated anomalies CHARGE.

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Cystic fibrosis

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Remember Vitamin deficiencies A K recurrent bleeding E D fractures/osteopenia

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Water soluble vitamins:

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Reye syndrome o In the setting of influenza B or varicella zoster infection aspirin is a mitochondrial toxin that can cause acute liver dysfunction in young ages

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Necrotizing enterocolitis

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Although neonates frequently are colonized with C difficile, symptomatic disease is rare in the first year of life, possibly due to an absence of intestinal receptors to the bacterial toxins. Jejunal atresia presents with bilious vomiting and abdominal distension. Abdominal xray shows a "triple bubble" sign and gasless colon. Risk factors include prenatal exposure to cocaine and other vasoconstrictive drugs.

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Intussusception: A pathological lead point should be suspected in older children with recurrent intussusception. Meckel's diverticulum is the most common congenital anomaly of the gastrointestinal tract and can be asymptomatic between episodes of telescoping Surgical resection is required to treat and prevent complications of obstruction.

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Gastroschisis:T2 sonography is > 95% sensitive for abdominal wall defects, and maternal serum a-fetoprotein is usually elevated. Gastroschisis results in the bowel being exposed to amniotic fluid → inflammation and edema of the bowel wall → ↑ risk of complications (eg, necrotizingenterocolitis, short bowel syndrome). Dysmotility (ileus, delayed gastric emptying, intolerance of feeds) occurs in > 50% of cases and may lead to prolonged reliance on TPN. Gastroschisis is an isolated defect >90% of the cases (no associated anomalies or neural tube defects). Rx: After delivery, the exposed bowel should be covered with sterile saline dressings andplastic wrap. Immediate surgery is required Suspect vitamin A deficiency in a 2 or 3-year-old child with impaired adaptation to darkness, photophobia, dry scaly skin, xerosis conjunctiva, xerosis cornea, keratomalacia, Bitot spots and follicular hyperkeratosis of the shoulders, buttocks, and extensor surfaces. Biliary atresia: Conjugated hyperbilirubinemia is defined as >2 mg/dl of direct bilirubin or a direct bilirubin fraction that is >20% of the total bilirubin level. Newborns with conjugated hyperbilirubinemia and hepatomegaly require immediate evaluation for biliary atresia. The first step in evaluation is abdominal ultrasound, which may show an absent or abnormal gallbladder Early treatment with a Kasai procedure dramatically improves outcomes.

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Kasai is a Temporary procedure: ↓ morbidity and mortality after liver transplantation

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Full-term infants are born with adequate iron stores to prevent anemia for the first 4-6 months of life regardless of dietary intake. Preterm infants are at significantly increased risk for iron deficiency anemia. Iron supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1 year All exclusively breastfed infants should also be started on vitamin D supplementation Crying infant: o Colic: Rx: Calming techniques include using an infant swing, swaddling, minimizing environmental stimuli (eg, quiet dark room), and holding and rocking the baby. Feeding patterns should also be reviewed to assess if there is overfeeding, underfeeding, or an inadequate burping technique

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Caustic ingestion: o Any intervention that could provoke vomiting should be avoided. This includes administration of milk, water, activated charcoal, vinegar, or nasogastric lavage, as vomiting can increase the extent of injury. 48

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Iron poisoning:

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The mechanism of iron poisoning is free radical production and lipid peroxidation, which impairs various cell processes, leading to systemic manifestations o Metabolic acidosis is due to lactic acidosis from shock and hypotension. o Dx is confirmed by measuring serum iron levels. Iron is radiopaque, and visualization of gastric tablets on abdominal x- ray further supports the diagnosis o Treatment depends on the severity of the poisoning. Whole-bowel irrigation is sometimes instituted, but other methods of decontamination (eg, activated charcoal, syrup of ipecac, gastric lavage) are not routinely recommended. Chelation therapy with intravenous deferoxamine is used in moderate-to-severe cases and would be indicated in this patient Lead poisoning: o Screening of high-risk populations is important, as most children with lead toxicity are initially asymptomatic but can have cognitive and behavioral problems that become apparent after school entry. 49

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Capillary (fingerstick) blood specimens are widely used in screening for lead poisoning, but false-positive results are common. Confirmatory venous lead measurement is required if a screening capillary lead level is ≥5 ug/dL. Lead accumulates throughout the body, but measuring it in hair, teeth, bone, or urine is not recommended

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Other risk factors include  lead piping  living near a battery recycling plant, having a parent who works with batteries or pottery  having a playmate or sibling with a history of lead poisoning Dimercaprol (BAL) + EDTA should be administered on an emergency basis for levels ≥70 or acute encephalopathy Rx of mild cases → remove from toxic environment

UW Peds ENT Saturday, February 11, 2017 7:12 AM  Otalgia:

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Acute otitis media:

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Treatment RULES:  Under 2 years → Abx. Delaying antibiotics in infants and young children could increase the risk of developing complications. Observation and analgesia is a reasonable option if the child is age >2 years, has a normal immune system, and symptoms are mild and unilateral.  Recurrent AOM (eg, >3 w/in 6 months or ≥ 4 w/in 12 months) despite appropriate antibiotic treatment → Tympanocentesis and culture during myringotomy with tube placement  Therapy with otic drops is appropriate for otorrhea from tympanostomy tubes, chronic suppurative otitis media, or external otitis, but not for AOM.  Although viral URI often precede or occur w/ AOM, it would not change management, which consists of empiric antibiotic treatment. Cholesteatoma: o congenital or acquired secondary to chronic middle ear disease. o New-onset hearing loss or chronic ear drainage despite antibiotic o Granulation tissue and skin debris may be seen within retraction pockets of the tympanic membrane on otoscopy. o Complications of cholesteatomas include hearing loss, cranial nerve palsies, vertigo, brain abscesses or meningitis Laryngomalacia: o Dx: clinical. Laryngoscope only for moderate to severe cases

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Juvenile angiofibroma: o Any adolescent who presents with a nasal obstruction, visible nasal mass, and frequent nosebleeds (epistaxis) is considered to have a juvenile angiofibroma (JNA), unless proven otherwise. o Pathophysiology:  benign growth, but is capable of eroding and locally invading (bone erosion). In the back of the nose or upper throat (nasopharynx) of adolescent boys.  Potentially very dangerous because of high vascularity which may bleed readily + occur in areas difficult to access surgically 52

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Treatment:  In some cases, no treatment is necessary.  Surgical removal  Indication: enlarging, obstructing the airway, or causing chronic nosebleeds.  Often difficult because the tumor is unencapsulated and may be deeply invasive.  Recurrence after resection is common. Bacterial rhinosinusitis:

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CT sinus scan/aspiration and Cx are not routine. X-ray sinus has low sensitivity (wrong answer)  Indication for CT imaging: If the patient develops complications such as  Periorbital edema  Vision abnormalities  Or altered mental status  Indication for aspiration and Cx: persists or worsens after 3 days of Abx Rx: Augmentin® + intranasal steroid (for pt w/ hx of allergic rhinitis). Antihistamine is a wrong answer.

UW Peds Hem/onc Sunday, February 5, 2017 5:09 AM 

Basophilic stippling are ribosomal precipitates which appear as blue granules of various sizes dispersed throughout the cytoplasm of the red cell. These are often seen with thalassemia, lead or heavy metal poisoning

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Sickle cell disease: o Stroke is a common complication of sickle cell disease secondary to sludging and occlusion in the cerebral vasculature. Exchange transfusion is the recommended treatment acutely since it helps to decrease the percentage of sickle cells and prevent a second infarct from occurring. 53

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Periodic blood transfusions are used primarily for treatment (eg, acute stroke, acute chest syndrome, acute multi-organ failure, acute symptomatic anemia, aplastic crisis); transfusions are indicated for stroke prevention.

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Hydroxyurea is indicated in patients with frequent painful episodes; history of acute chest syndrome; and severe symptomatic anemia.

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Hydroxyurea has been shown to decrease pain crises, the need for transfusions, and episodes of acute chest syndrome. Side effects: bone marrow suppression (Leukopenia, anemia, and thrombocytopenia). These effects are generally temporary and reversible but may predispose the patient to infection.

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The high rate of heme catabolism and short red blood cell life span (-20 days) results in elevated lactate dehydrogenase, unconjugated hyperbilirubinemia, and compensatory reticulocytosis Excess hemoglobin (from extravascular and intravascular hemolysis) binds to haptoglobin ↓ haptoglobin

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Sickle trait: pts are at increased risk for renal issues, the most common of these is painless microscopic or gross hematuria that results from sickling in the renal 54

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medulla. Isosthenuria (impairment in concentrating ability) is also common and can present as nocturia and polyuria. Spherocytosis: Dx is confirmed by acidified glycerol lysis test and eosin-5-maleimide binding test. It can present in neonates, however, they are unable to produce appropriate erythropoiesis in response to anemia ==> NORMAL reticulocyte count), thus predisposed to severe anemia and often require transfusions.

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Review Glycogen storage diseases Iron deficiency: o The most common nutritional deficiency in children o Often caused by excessive consumption of cow's milk (>24 ounces [700 ml] per day).  The anemia is secondary to the low iron content of milk, poor bioavailability of iron from milk, and increased intestinal blood loss from cow's milk proteininduced colitis. o

RDW values >20% are suggestive of iron deficiency anemia; normal values of 12%-14% are seen in thalassemia.

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Iron deficiency in child < 2 yo  Children with iron deficiency are often asymptomatic and may not have tell tale symptoms such as pica, fatigue, or pallor. Therefore, universal screening is recommended by age 1 year, and the diagnosis is typically based on a complete blood count. Further testing is not necessary in children with the classic presentation. 

The most cost-effective approach to treatment is empiric oral iron therapy → recheck Hb in 4 wks → if Hb ↑ by 1g/dL → continue therapy for 2 - 3 months after Hb normalizes to replete iron stores.

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If the anemia does not resolve w/ empiric iron therapy and appropriate dietary changes, other causes must be considered. Hemoglobin electrophoresis (for hereditary anemias), colonoscopy (for lower gastrointestinal tract bleeding), or serum creatinine measurement (for renal disease) may be indicated.

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Blood transfusions are rarely necessary in the treatment of dietary iron deficiency anemia, even when the hemoglobin is as low as 4 g/dL Iron therapy should be sufficient.

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ALL: Lymphoblasts lack peroxidase positive granules of AML but often contain cytoplasmic aggregates of periodic acid Schiff (PAS) positive material. lmmunostaining for terminal deoxynucleotidyltransferase (TdT) is positive in more than 95% of patients TdT is expressed only by pre B and pre T lymphoblasts

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Wiskott-Aldrich syndrome: 56

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Dx can be made clinically, but gene testing allows screening of other family members. Rx: stem cell transplantation

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Neonatal polycythemia:

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Erythrocyte CD55 and CD59 protein testing? Paroxysmal nocturnal hemoglobulinuria Hemophilia: Hemophilic arthropathy is a delayed consequence of recurrent hemarthrosis. Hemosiderin deposition leading to synovitis and fibrosis within the joint → chronic pain, limited range of motion. The risk of hemophilic arthropathy can be significantly reduced by prophylactic treatment with factor concentrates. 57

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Bone tumors:

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osteosarcoma vs ewing sarcoma o Osteoid osteoma appears as a sclerotic, cortical lesion on imaging with a central nidus of lucency. It typically causes pain that is worse at night and unrelated to activity. However, the pain is quickly relieved by nonsteroidal anti-inflammatory medications. ITP: o BM biopsy is not required in well-appearing children with isolated thrombocytopenia and no other symptoms (eg, fever of unknown origin, leukopenia, anemia, unexplained weight loss, fatigue) o Splenectomy is a last resort for catastrophic bleeding or chronic ITP that is refractory to IVIG and glucocorticoids

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Vit K deficiency:

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Anemia evaluation:

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Pappenheimer bodies are iron-containing inclusion bodies found in peripheral red blood cells. They result from phagosomes that engulf excessive amounts of iron and are typically seen in sideroblastic anemia. Aplastic anemia: o Acquired aplastic anemia should be suspected in any patient with pancytopenia following drug intake, exposure to toxins or viral infections.

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Both types of thalassemia minor are characterized by abnormally small RBCs. RDW is normal, and total RBC count is normal or elevated. This typically results in a Mentzer index (MCV/RBC)