UW Peds [email protected]

UWorld notes

Pediatrics

1

UW Peds Adolescence/misc

o _{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:

2 o

 Gonococcal conjunctivitis:

o _{Prevention: Application of topical erythromycin w/in 1 hour of birth}

o

 _{Primary amenorrhea:}

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

3 o

o _{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 Buccal smear for Barr bodies (inactivated X chromosomes) is an outdated and unreliable screening test for turner.

4  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)

o

o _{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

5  Osteomyelitis:

o

o _{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:

o

o _{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

6  Cat bites:

o _{Most cat bites will become infected w/o abx prophylaxis}

o

o _{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

o

o 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 <25, new or multiple sexual partners)

7  Lymphadenopathy:

o

o _{Palpable supraclavicular lymph nodes should also be considered pathologic until} proven otherwise.

 Androgen insensitivity:

o Cryptorchid gonads have a 1%-5% risk of developing a dysgerminoma or gonadoblastoma after puberty.

o _{Most 46,XY individuals with complete AIS identify with the female gender identity,} but the decision for gonadectomy is based on concern for malignancy and not the patient's gender identity. Patients should receive age-appropriate disclosure and families should be offered psychosocial support

o _{Benefits from gonad-stimulated puberty ( eg, attainment of adult height) outweigh} the low risk of malignancy. Therefore, a gonadectomy can be deferred until

completion of puberty

o _{Gonadectomy is performed before or during puberty in cases of incarcerated or} symptomatic inguinal masses or suspected malignancy (based on imaging or tumor markers)

o _{In contrast, gonadal dysgenesis in Turner syndrome is associated with a higher} risk of malignancy (15%-30%), requiring closer surveillance and earlier

8  Congenital infections:

o

o _{Congenital syphilis: penicillin therapy is curative and prevents the development of} late manifestations (eg, frontal bossing, saddle nose, Hutchinson teeth)

o _{Suspect HIV infection when an infant has failure to thrive, LN, and thrush in the} setting of maternal IVDU. Pregnant pts with risky behavior should undergo HIV antibody testing in the 1st and 3rd trimesters as it can take up to 3 months to develop detectable antibody (giving initial false negative result). Dx in infants (from birth to 18 months): PCR



9  Strabismus:

o

10  Retinoblastoma:

o _{Every case of leukocoria is considered a retinoblastoma, until proven otherwise.} Rb is the most common intraocular tumor of childhood

 Prepubertal vaginal bleeding:

o

o The most common cause of vaginal bleeding and discharge in the neonatal period is withdrawal of maternal estrogen.

o

 Universal screening for dyslipidemia is recommended at age 9-11 and again at age 17-21 as lipid levels are relatively stable just prior to and after puberty.

 Screen visual development in every well-child examination. Observe fixation and tracking. Perform cover-uncover test in older infants and children to assess for strabismus. Test visual acuity at age 3 w/ the tumbling E or Snellen chart.

11  Neonatal sepsis:

o

 Bacterial rhinosinusitis:

o

o _{Streptococcus pneumoniae (-30%), nontypeable Haemophifus influenzae (-30%),} and Moraxefla catarrhatis (-10%).

o _{Pseudomonas aeruginosa is common in nosocomial sinusitis, especially in} immunocompromised patients with nasal tubes or catheters.

12  Bacterial meningitis:

o

o Neonates do not herniate after LP, as their open fontanelles serve to relieve intracranial pressure

o _{Infants who are critically ill (eg, status epilepticus, septic shock) may require Abx} before LP

 Entrobius vemicularis: all household contacts should be treated  Granulosa cell tumor:

o

 Abnormal uterine bleeding:

o _{Adolescents w/ heavy bleeding should also be evaluated for bleeding disorders if} clinically appropriate.

o _{Sexually active adolescents should be for pregnancy} o _{Rx options:}

 High-dose intravenous or oral estrogen  High-dose combined OCP

 High-dose progestin pills if contraindication to estrogen or to treat anovulatory bleeding

13  Cavernous sinus thrombosis (vs orbital cellulitis):

o _{Headache is the most common early symptom.}

o _{There's also periorbital edema, exophthalmos, and chemosis, but fundoscopy} typically reveals papilledema and dilated tortuous retinal veins.

o _{Eye swelling in CST usually begins as a unilateral but quickly (24-48 hours)} involves the other eye.

o _{Cranial nerves 3,4,5,6 involvement (eg, ptosis)}  Croup:

o _Rx:

 Mild (no stridor at rest): single dose of steroids (dexa) to decrease airway edema.

 _{Moderate/severe (stridor at rest): steroids + nebulized epinephrine}  Rabies:

o

o

14

UW Peds cardiology

o _{Nielsen has sensory neural deafness}

o _{Inherited syndromes are due to defect in K}+ _{channels. Mutation in the LQT1 gene.}

o _{Rx: avoid electrolyte derangements and meds that block K}+ _{channels. Beta blockers} with pacemaker placement can prevent cardiac arrest.

o

15 o

 Sudden cardiac death:

o _{HCOM is more prevalent than long QT $. A question stem w/o enough clues to} differentiate, choose HCOM.

o _{HCOM peaks during puberty (↑ myocyte hypertrophy and fibrosis)}

o

 Congenital heart diseases:

o _{Pearl: Large (symptomatic) VSD classically cause a pansystolic murmur that is} loudest at the left lower sternal border and a diastolic rumble at the apex due to increased flow across the mitral valve.

16 o

o Cyanotic heart diseases

o

17 o

o _{Tricuspid atresia ECG}



18

 Hypertension in children:

o _{The most common cause of 2ry HTN in children is fibromuscular dysplasia (20% of} all cases) (seen in premenopausal women).

o _{Physical examination reveals a hum or bruit in the costovertebral angle due to} well-developed collaterals.

o _{Rt renal artery is affected > 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.}

o

o _{Chest x-ray cardiomegaly}

o (N.B. age <1 yo nl transverse cardiothoracic ratio is 56%. age >1 year and adults nl cardiothoracic ratio is 55%)

19 o

 Heart murmurs: benign vs pathologic

o _{Maneuvers that ↓ VR (eg, standing, Valsalva maneuvers) typically ↓ innocent} murmurs.

20  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

 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.

21  Atopic dermatitis:

o

o

22 o _{Eczema herpeticum}

o

o

 _{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.

23  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}



o _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 anti-staphylococcal 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.

24 o

 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.

25 o

o

versicolor

26 o

 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.

27

UW Peds endocrinology

 _{Constitutional short stature vs familial short stature growth charts}

o

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} life-threatening 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.

o _{Prevention: Avoid aggressive initiation of nutrition without adequate electrolyte} repletion

28 o

 Precocious puberty:

29  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 o GnRH agonist therapy to prevent premature epiphyseal plate fusion and

maximize adult height potential. Girls age <6 who undergo GnRH agonist

treatment have the potential to grow an additional 10 cm; girls age 6-8 can grow an additional 4-7 cm

o _{Reassurance can be provided in cases of isolated premature thelarche or} adrenarche when bone age is normal

o

o _{Isolated premature adrenarche:}

 _{Caused by ↑ adrenal androgen secretion. More in black & hispanics}

 Lab: may show slightly ↑ DHEA-S, but testosterone levels are usually low.  Generally benign but is a significant risk factor for PCOS, type 2 DM, and

metabolic syndrome, especially in obese pts.

 _{Gynecomastia:}

o _{Pubertal gynecomastia:}

 2/3 of adolescent boys during puberty. Unilateral, bilateral, or painful.

 _{Due to transient ↑ in testicular production of estrogen over testosterone and} peripheral conversion of prohormones to estrogen

 No workup or treatment is necessary as it usually resolves within a few months to 2 years

30 o

 Congenital hypothyroidism:

o _{Most common is Thyroid dysgenesis (85%) then defect in hormone synthesis} (10%) then maternal receptor blocking antibodies (5%)

o _{Initially asymptomatic due to presence of moderate amount of maternal hormones} o _{Symptoms may also include: abdominal bloating, resistant macrocytic anemia,}

noisy breathing

31  Kallmann $ is an X linked recessive disorder yet it can manifest in females

o

 _{Turner $: estrogen replacement to nearly all pts to promote normal maturation and to} ↓ risk of osteoporosis

 _{McCune-Albright $:}

o _{Characterized by precocious puberty (endocrine abnormalities), cafe au lait spots} and multiple bone defects (polyostotic fibrous dysplasia)

32

UW peds GIT

Saturday, January 21, 2017 12:52 AM

 Neonatal IO suspected (bilious vomiting). First step? cessation of enteral feeding + decompression + IVF. Xray to rule out perforation.

o

33  Both have empty rectum on PR exam but the anal tone is increased in HS.  Both can have narrow colon, but it's usually recto-sigmoid in HS and diffuse

in meconium ileus

o Neonatal IO/malrotation: barium swallow is the fastest and most accurate test to order: ligament of Tretiz on the right side of the abdomen (malrotation) or

corkscrew (vovulus). US has high false -ve. Barium enema has unacceptable false +ve and -ve

 Beckwith Wiedemann $:

o _{the hypoglycemia is usually temporary during neonatal period and kids grow out} of it later on, hence, no need for ongoing glucose monitoring.

o

o _{Screening recommendation for BWS} 

Age Test freq

0-4 yr Abdominal US a-fetoprotein

Every 3 m 4-8 yr Abdominal US Every 3 m 8-18 yr Renal US

 Patients with isolated hemihyperplasia are also at increased risk for Wilms tumor and hepatoblastoma. These patients should undergo frequent screening as in BWS.  Constipation Rx in kids: Sorbitol-containing juices (eg, prune, pear, apple) can

increase stool water content Juice is less effective than laxatives, and excessive consumption increases the risk for obesity.

34 o

 Laxatives: polyethylene glycol, mineral oil

 Enema is Last resort

 Bilious emesis in the neonate is an ominous sign of intestinal obstruction and requires immediate workup. Exclude pneumoperitonium first by abd x ray then proceed with enema or upper GI series.

o

 Breastfeeding failure jaundice can be caused by both maternal (eg, inadequate milk supply, cracked/clogged nipples, engorgement, infrequent feeding) and infant (eg, poor latch, ineffective suck, falling asleep) factors.

35 o _{Signs of dehydration are: decreased urine output, weight loss more than}

expected, brick-red urate crystals in baby diapers, delayed passage of yellow stools.

o Rx: in an otherwise healthy full-term newborns is to increase the frequency and duration of feeds to stimulate milk production, maintain adequate hydration, and promote bilirubin excretion.

o _{Monitor bili w/in 2 days and If continued to rise, supplement w/ formula but don't} stop breastfeeding as that will cause further decrease in milk production.

o

 Biliary atresia presents with conjugated hyperbilirubinemia (direct bilirubin >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

36

o

 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")

37 o

o

 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

38 o

 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

39

o

o _{Can be congenital or aquired.} o _{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)

40  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

o

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

o

o _{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.

41 o

 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.

o

42  Cystic fibrosis

o

o _{Remember Vitamin deficiencies} A

K recurrent bleeding E

43  Water soluble vitamins:

o

 _{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

44  Necrotizing enterocolitis

o

45  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 x-ray shows a "triple bubble" sign and gasless colon. Risk factors include prenatal exposure to cocaine and other vasoconstrictive drugs.

o

 _{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.

46 o

47

 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.

o

o _{Kasai is a Temporary procedure: ↓ morbidity and mortality after liver} transplantation

48 o

 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

o

 _{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.

49 o

 Iron poisoning:

o

o _{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.

50  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

o

51 o _{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}

o _{Dimercaprol (BAL) + EDTA should be administered on an emergency basis for} levels ≥70 or acute encephalopathy

o _{Rx of mild cases → remove from toxic environment}

UW Peds ENT

Saturday, February 11, 2017 7:12 AM

 Otalgia:

o

 _{Acute otitis media:}

52 o _{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}

o

 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

53 o _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:

o

o _{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}

o _{Rx: Augmentin® + intranasal steroid (for pt w/ hx of allergic rhinitis). Antihistamine} is a wrong answer.

UW Peds Hem/onc

 _{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

 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.

54

o _{Periodic blood transfusions are used primarily for treatment (eg, acute stroke,} acute

o chest syndrome, acute multi-organ failure, acute symptomatic anemia, aplastic crisis);

o _{transfusions are indicated for stroke prevention.}

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

o _{Hydroxyurea has been shown to decrease pain crises, the need for transfusions,} and episodes of acute chest syndrome.

o _{Side effects: bone marrow suppression (Leukopenia, anemia, and}

thrombocytopenia). These effects are generally temporary and reversible but may predispose the patient to infection.

o 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

o

o

o _{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

55 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.

o

 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 protein-induced colitis.

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

56

o _{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.

 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.

 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.



 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

57 o _{Dx can be made clinically, but gene testing allows screening of other family}

members.

o _{Rx: stem cell transplantation}



 Neonatal polycythemia:

o

 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.

58  Bone tumors:

o

o _{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

59 o

 Vit K deficiency:

60  Anemia evaluation:

o

 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.



 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) <13 (vs iron def it's > 13 due to ↓ RBCs). The hematocrit is usually >30% in patients with thalassemia minor.

61 o _{Usually follows an infection. Dx: clinical in children w/ classic signs and}

symptoms.

o _{Dx requires the presence of lower-extremity purpura or petechiae PLUS at least} one of the following

 _{arthritis or arthralgia}  renal involvement  abdominal pain

 positive histopathology

o _{Laboratory testing is not required but typically demonstrates a normal platelet} count and mild ↑ creatinine.

o _{In children with atypical presentations, a renal biopsy to confirm the Dx(deposition} of lgA in the mesangium will be seen.

o

 Purpura fulminans = purpura that happens w/ meningococcal meningitis.  Henoch schonlein purpura:

o _{↑ risk for intussusception due to bowel wall edema and localized hemorrhage →} act as lead-points for the intussusception

o _{Unlike most cases of intussusception in children, which are ileo-colic,}

intussusceptions in HSP are more likely to be small-bowel or ilea-ileal (60% of cases).

o Because of their location, small-bowel intussusceptions cannot be seen on contrast enema and are diagnosed by the presence of a "target" sign on ultrasound.

o _{Ileocolic intussusceptions can be treated with air or contrast enema, but ilea-ileal} intussusceptions that do not reduce spontaneously often require surgical

62 o

o

63 o

Osteosarcoma

The most common 1ry bone tumor in children and young adults (13-16 yo have higher risk)

Risk factors:

Paget disease (old age), Retinoblastoma

Li Fraumeni $ Presentation:

Pain and swelling

The most important physical exam finding is tender soft tissue mass.

Normal range of motion, no constitutional symptoms (fever, wt loss, night sweats). Workup:

lab: ↑ Alkaline phosphatase and LDH (high levels correlate w/ adverse prognosis) Imaging: x-ray: metaphyses of long bones such as the distal femur proximal tibia, and proximal humerus. Spiculated "sunburst" pattern (yellow arrow) and periosteal elevation known as the Cadman triangle (red arrow)

64 Rx:

excision and chemotherapy

Ewing sarcoma

Although overall rare, Ewing sarcoma is the 2nd most common 1ry bone malignancy in adolescent males.

Risk factors:

Associated with t(11;22) translocation Presentation:

Pain, mild fever, erythema and swelling, anemia (20% have systemic symptoms). Extremely aggressive with early metastases

Workup:

X-ray: Commonly appears in diaphysis of long bones, pelvis, scapula, ribs. Osteolytic lesion with a periosteal reaction that produces layers of reactive bone, giving the classic "onion skin" appearance

Also read here

65

UW Peds ID

66 o

67 o

 _{Cervical adenitis in children}

o

68 o

 Lyme disease

o Borrelia burgdorferi

o Ixodes scapularis transmits Lyme disease (as well as anaplasmosis and babesiosis)

o _{The rash is not particularly painful but may occasionally burn or itch.} o _Rx:

 Oral doxycycline, amoxicillin, and cefuroxime have equivalent efficacy for treating early localized Lyme disease. Doxycycline is used because it is effective in treating potential coexisting Anaplasma.

 _{IV ceftriaxone is reserved for Lyme meningitis and heart block,} manifestations of early disseminated Lyme disease.

69 o

70 o

o

71 o

 _{Cystic fibrosis}

72 o

o

73 o  _Pertussis: o

o _{Dx: clinical, start Rx and don't wait for confirmatory tests. PCR used in uncertain} cases and for epidemiological studies.

o _{Prevention: previous vaccination or infection can decrease disease severity but} doesn't provide lifelong immunity. Adolescents who didn't get a booster @ 11-18 yr are at risk of waning immunity. Give Tdap booster w/ each pregnancy.

74 Prophylaxis macrolide (azithro for <1 y/o bcz erythro → pyloric stenosis; and

clarithro not well studied) to all contacts despite their vaccination status. Unvaccinated contacts should receive vaccines according to schedule. Respiratory isolation is necessary only during the first 5 days of antibiotics. o _{Rx: 100 day cough but still, as a general rule; Over-the-counter antitussive}

medications should be avoided due to lack of proven efficacy and risk of toxicity, especially in children age <6 years

o _{Hospitalization if}

 _{Infants <3 months (due to high risk of apnea)}  Severe paroxysms that impair feeding

 Complicated by pneumonia, seizures, or other comorbidities.

o

 Inflamed joint DD:

o Bacterial joint infection that is often preceded by skin or upper respiratory tract infections (source of infection + hematogenous spread) → DD 1) transient

synovitis (same local sings and symptoms but no systemic symptoms like fever or significant lab derangements; Rx: bed rest + NSAIDs), and 2) rheumatic fever (migratory, look for associated findings w/ RF; Rx: rest + NSAIDs). Suspect septic arthritis → arthrocentesis.

o

75 o

76 o

o _{Septic arthritis is often preceded by a seemingly mild infection (e.g. cellulitis) in} which there may be intermittent bacteremic showers, allowing for hematogenous spread of bacteria into the synovial fluid.

o _{Gram stains and cultures can be falsely negative and confounded in pts} pretreated w/ antibiotics for his recent skin infection. (focus on WBCs in joint analysis)

o _{Rx: Debridement and irrigation of the joint space is the most important intervention} in preventing long-term disability. A delay of even 4-6 hours in treatment can lead to joint necrosis and requirement for a prosthetic. If no improvement w/

appropriate Abx and still febrile → MRI to look for concomitant osteomyelitis.  Meningitis:

The long-term neurologic sequelae associated with bacterial meningitis are: o _{Hearing loss}

o _{Loss of cognitive functions (due to the neuronal loss in the dentate gyrus of the} hippocampus)

o _Seizures

o _{Mental retardation} o Spasticity or paresis  Pharyngitis DD:

77 o

78 o _{Pearls: Note that Centor criteria includes only ANTERIOR cervical LN+ and}

tonsillar EXUDATES. Posterior LN+ and any tonsillar changes (ulcers, erythema..etc) other than exudates are not included.

o

o

o Herpangia also causes bilateral LN+

79 o o _EBV   _{Heterophile antibody}

 Initial negative test does not rule out the IM diagnosis as it takes wks to develop. EBV-specific antibody testing can be ordered in those with more prolonged illness and negative heterophile antibody testing

 Atypical lymphocytes

 May also be present in pts w/ toxoplasmosis, rubella, roseola, viral hepatitis, mumps, CMV, acute HIV infection, and some drug reactions.

80 o _{causes a milder pharyngitis and lymphadenopathy than EBV-induced IM.}

o _{CMV lgM titers can be considered in patients with a negative heterophile antibody} test

o Rapid influenza testing (high specificity and low to moderate sensitivity) can be performed in high-risk children in whom treatment would be indicated (eg, age <2, history of pulmonary/cardiac disease, need for hospitalization) Influenza typically presents with fever, cough, and rhinitis rather than pharyngitis.

o _{Retropharyngeal abscess: Dx by clinical + lateral X-ray. In pts with no signs of} respiratory compromise, do CT w/ contrast to confirm presence and size of the abscess.

o _{scan with contrast should be performed to confirm the presence and size of the} abscess

o

Nonetheless, you have to cover pseudomonas in your empiric Abx

 Epiglottitis:

o _{Incidence was reduced due to widespread Hib vaccination, but Hib remains the} most common cause, even in immunized children.

o Symptoms often develop over several hours without a significant prodrome (eg, cough, congestion, rhinorrhea) vs croup that develops over days w/ prodrome. Signs of impending airway obstruction include restlessness, anxiety, worsening stridor, and a muffled "hot potato" voice

81 o

82 o

83 o

 HUS:

o _{Vascular damage, microthrombi formation. Platelet consumption but normal} PT/PTT. No fever. Abx are CI

o

 Acute rheumatic fever: o Rx:

84  Even in the absence of active pharyngitis, pts with ARF should still be treated

with IM benzathine penicillin G until adulthood to eradicate bacterial carriage to prevent recurrent ARF and worsening rheumatic heart disease.

 Pts w/ severe chorea may require steroids, and those w/ pericarditis and arthritis may need NSAIDs

o

 Most common organism causing pericarditis or myocarditis? Coxsackievirus  Impetigo:

Risk factors: warm/humid climate, poverty/crowding, poor personal hygiene, and pre-existing skin trauma/ inflammation (eg,insect bite, eczema) Colonization w/

staphylococci or streptococci.

o

85 o

o

 Varicella:

o Vesicles are pruritic.

o Potential complications include (bacterial superinfection in kids and pneumonia in adults. lmmunocompromised patients are at risk for potentially fatal disseminated disease. Patients are contagious from 2 days prior to rash onset until all vesicles are crusted (isolate the pt for this entire period)

86 o

o _{Immunocompetent pts who are asymptomatic and nonimmune should receive} varicella vaccine for post-exposure prophylaxis within 3-5 days of exposure. lmmunocompromised (<1 yo & pregnant included) who are asymptomatic and nonimmune should receive VZIG w/in 10 days of exposure. Although VZIG may not prevent infection, it can reduce disease severity. Those who receive it require close monitoring for development of varicella infection as VZIG can prolong the incubation period beyond 1 month. Immune pts are ok, and symptomatic pts get acyclovir.

87 o

88 o

o

 Rashes

o _{scarlet fever: Rash initially appears on neck, axillae, groin then generalizes.} Towards the end of the 1st week, desquamation begins in the face, progresses down the trunk, and finally extends to the hands and feet

o Kawasaki: can be confused w/ scarlet since it has strawberry tongue and other features. Key is to stick to Kawasaki criteria for Dx. Rapid response to penicillin confirms scarlet. Nikolsky sign -ve

89 o _{SSSS: exfoliative strains of S. aureus, characterized bullae followed by an}

exfoliation of the skin. Rare after 5 yo. Nikolsky sign +ve

o _{Toxic epidermal necrolysis: hypersensitivity; considered to be a variant of} Stevens-Johnson syndrome. 2ry to medications (sulfa drugs, anticonvulsants, and NSAIDs). Similar to SSSS, it presents with extensive erythema, tenderness, and blister formation, followed by exfoliation. Mucous membranes are severely affected, and shedding of the nails may occur. Nikolsky sign +ve

o _{Stevens-Johnson $: severe variant of erythema multiforme (target lesions),} followed by bullae of two or more mucous membranes. Sometimes, the Gl, respiratory, or GU tracts may also be involved.

 _{Sickle cell:}

o _{Strept pneumococci is the most common causes of sepsis (fever w/o origin) in} those pts, despite vaccination (vaccine doesn't cover all serotypes; doesn't provide full immunity). On the other hand, H influenza and meningococcal vaccines are fully protective.

o

 _{Osteomyelitis:}

o _{Staphylococcus aureus is the most common organism in infants and children.} Other commons are GBS and E coli in infants, and Streptococcus pyogenes in children. Staphylococcus epidermidis is a frequent cause of osteomyelitis associated with prosthetic devices.

90 o

 Meningitis:

o _{Bacterial meningitis:}

 Neisseria meningitides is the most common cause of bacterial meningitis in children and young adults in the United States. The mortality rate of

meningococcal meningitis is v. high (15%). Children age < 2 are at greatest risk for contracting this illness, with rates 10 times higher than those of the general population. Seventy-five percent of patients with Meningococcus meningitis present with a petechial rash that is prominent on the axilla, wrists, flanks and ankles. It appears within 24 hours of the infection.

o _{Viral meningitis:}

 The most common causes of viral meningitis are the non-polio enteroviruses, such as echoviruses and coxsackieviruses. Start w/ constitutional and upper respiratory symptoms w/ low-grade fever. Over the next 36-48 hours, the patient develops a high fever, headache, irritability, and nuchal rigidity. Other symptoms of enteroviral infection may also be seen such as pharyngitis, rash, or herpangina. self-limited Rx: supportive, resolves in 10 days

91 o

 Neonatal sepsis:

o Early onset sepsis: < 7 days o _{Late onset sepsis: later}

o _{Pregnant women with listeriosis have nonspecific flulike symptoms such as fever.} body aches. and fatigue prior to delivery

92 o

o

 Measles:

o _{Measles (rubeola) is extremely contagious, spreads by droplets from respiratory} secretions. The virus can remain airborne for several hours, and unvaccinated patients can contract it by entering a room that an infected person has exited.

93 o _{Patients are most contagious during the prodrome but can spread disease for}

several days even after the resolution of the rash. Patients with known or suspected measles should be isolated and placed on airborne precautions (negative pressure room, N95 facemask (not surgical masks) for health care personnel)

o

 Neonatal conjunctivitis:

o _{Blood-stained eye discharge is highly characteristic of chlamydia! conjunctivitis}

o

 STDs screening:

o _{Screening for chlamydia trachomatis and Neisseria gonorrhoeae in the following} pts:

94  _{All sexually active women age ≤24}

 _{Any person with a new partner in past 2 months}  Multiple partners

 History of sexually transmitted infections

 illicit drug use, incarceration, and contact with sex workers. o Best screening test:

 Nucleic acid amplification test (high sensitivity and specificity).

 _{The test can be performed on urine, endocervical, vaginal, or urethral} specimens with similar accuracy.

 Immunizations:

o _{All stable preterm infants should receive the first dose of the hepatitis B vaccine} at birth ,unless the infant weighs <2 kg (4 lb 6 oz) → wait until they weigh 2 kg then vaccinate

o _{All vaccines should be administered according to chronological rather than} gestational age in preterm infants.

o Laws concerning anti-vaccines people:

 _{All states allow medical exemption from vaccination (eg, allergy to vaccine} components)

 Some states also allow for exemption based on a parent's religious and/or personal beliefs.

 If a child is unvaccinated and is not exempt, he/she may not be able to enroll in day care or school, depending on the state.

 The physician must respect the mother's decision but is obligated to inform her about the health-associated risks and benefits as well as the potential consequences (school enrollment)

 The discussion should be fully documented in the medical record.  Physicians do not report cases of vaccination refusal.

95 o _{Rotavirus vaccine: live attenuated vaccine, typically administered 2-6 months of}

age



o _{Administration of multiple vaccinations in a single office visit is safe and increases} vaccine compliance and optimal protection at a young age. The exception is live-virus vaccines, which should be administered 4 weeks apart due to possible interference of immune response.

o _{Vaccines can be administered safely during minor illnesses (eg, upper respiratory} infections). They should be postponed until recovery from moderate or severe illnesses as manifestations of the underlying illness can be incorrectly attributed to the vaccine and vice versa.

o Contraindication and precaution to DTaP:

 Encephalopathy within a week of DTaP vaccine administration is a

contraindication for further administration of the combination vaccine, and diphtheria and tetanus toxoids should be administered without pertussis.  Infants with progressive neurologic disorders should also avoid pertussis

immunization to prevent confusion about causality until treatment is established and the condition has stabilized.

96  o + Abx

Immunizations

97

UW Peds immunology

 Do a quick step1 review here  Bruton's agammaglobulinemia

Defect in tyrosine kinase that prevents the development of mature B cells

98  Pts w/ T cell disorders may have Humoral immunodeficiency due to defective T-cell help

in B-cell activation for antibody production.

 All patients with Digeorge $ should receive routine killed or subcomponent vaccines. However, the safety of live vaccines depends on the degree of immunodeficiency. Decisions regarding administration should be tailored to the patient's immune status.  Hereditary angioedema:

Presents in late childhood. Episodes usually follow an infection, dental procedure, or trauma. Defect or deficiency of C1 inhibitor leads to ↑ edema-producing factors C2b and bradykinin

 Rapid onset of:

 _{Noninflammatory edema of the face, limbs, and genitalia}  Laryngeal edema

 Edema of the intestines resulting in colicky abdominal pain  No evidence of urticaria



Hereditary angioedema Acquired angioedema C1 inhibitor deficiency ACE-I → ↑ bradykinin Normal Cq1 levels ↓ Cq1 levels

↓ C4 levels ↓ C4 levels  CGD:

o CGD pts should receive lifelong antimicrobial prophylaxis, and interferon gamma can be used to boost intracellular killing in severe cases.

o

 Transient hypogammaglobulinemia of infancy: Pts present with increased sinopulmonary and gastrointestinal infections that are usually mild rather than life-threatening. The

normal physiologic nadir of maternally-derived antibody is 3-6 months of age in term infants. Low serum lgG levels after age 6 months is termed transient

hypogammaglobulinemia of infancy as it usually resolves by age 12 months. B and T lymphocyte counts and lgA and lgM levels are normal

99 o

 Humoral immune deficiencies:

o

 LAD

o

Read that TERC thingy

100

UW Peds Misc

o Most children are ready to begin toilet-training at age ≥ 2with the acquisition of the following skills:

 Voluntarily control sphincters  _Walk

 Remove pants

 Follow 2-step commands

 Communicate the need to urinate and stool  Imitate actions of other people (e.g. sit on toilet)

o Premature initiation of toilet-training can prolong the duration of training. Don't to rush the child, bedwetting is normal < 5. Most children master daytime continence within months, but nighttime continence can sometimes take years to accomplish.

101 o _{Chronic constipation can reduce bladder capacity and contribute to urinary}

incontinence. Stool burden can be assessed by abdominal x-ray but is unnecessary in the setting of soft, regular bowel movements.

o Urinalysis performed only when children are > 5 to screen for urinary tract infection, diabetes mellitus, and diabetes insipidus

o _{Alarm or desmopressin therapy are unwarranted for children <5.}

 Infant development progresses from cephalic to caudal; from proximal to distal; and from stimulus-based reflexes to specific, goal-oriented reactions that become

increasingly precise with age  Child abuse:

o

o

102 o

o _{Mental health services is also available for minors w/o parental notification.} o _{Minors who are parents may consent for the treatment of their own minor}

children.

o Parents are not allowed to refuse life-saving treatment for a child, including refusal for religious reasons.

o _{If parents refuse to consent to treatment of their child for a non-emergency but} fatal medical condition (chemotherapy for ALL), the physician should seek a court order mandating treatment

o _{Only one parent is required to consent to the minor treatment. Even if the other} parent disagrees. And remember that consent isn't required at all in emergency situations.

 Small for gestational age infants:

o _{Small for gestational age infants have a weight under the 10th percentile for} gestational age

o _{SGA infants are at risk for several complications due to their size, including} hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia (low glycogen stores, Rx: early and frequent feeds), hyperglycemia (infants w/ very low birth wt due to low insulin secretion), hypocalcemia (↓ Ca transfere across the placenta), and polycythemia (↑ erythropoietin secretion in response to fetal hypoxia)

103 o

o

104 o

 Beckwith-Wiedemann $:

o _{Disregulation of imprinted gene expression in chromosome 11 p15.}  Marfan:

o _{Fibrillin-1 mutation.}

o _{Lens dislocation (ectopia lentis), iridodonesis (a rapid contraction and dilation of} the iris), and myopia (from elongation of the globe) are typical

o First-degree relatives should undergo genetic testing

105 o

 Ehlers-Danlos syndrome:

o _{Collagen disorder characterized by scoliosis, joint laxity, and aortic dilation.} o _{Pts do not have the disproportionately tall stature, lens dislocation, or pectus}

carinatum seen in Marfan syndrome  Congenital contractural arachnodactyly:

o _{Autosomal dominant condition resulting from mutations of the fibrillin-2 gene.} o _{Tall stature, arachnodactyly, and multiple contractures involving large joints} o _{Ocular and cardiovascular symptoms are not present in congenital contractural}

arachnodactyly. In addition, pts with Marfan $ do not have joint contractures.

UW Peds Neuro

 Congenital rubella is most dangerous during T1. Positive IgG later in pregnancy can't differentiate between infection in early pregnancy and immunization as IgG will appear a few weeks after infection.

 _{Neuroblastoma: The levels of serum and urine catecholamines and their metabolites} (i.e., HVA and VMA) are usually elevated; however, patients do not present with fainting spells, sweating, palpitations and hypertension, as in pheochromocytoma  Wilms tumor arise from metanephros, which is the embryologic precursor of the renal

106  Fragile X chromosome pts may display features of ADHD and Autism spectrum

disorder.

 Absence seizures:

o

o _{DD: Pts w/ inattentive subtype of ADHD often present with staring spells.}

However, these spells tend to occur with boredom or during sedentary activities and are typically responsive to vocal or tactile stimulation.

o _{ADHD is common w/ absence seizures or w/ other forms of epilepsy. These} patients typically fall under the inattentive subtype of ADHD rather than the

hyperactive subtype. The Dx of ADHD is important to make in children w/ epilepsy as effective behavioral (eg, minimization of distractions) and pharmacologic (eg, methylphenidate) therapies exist. Unrecognized or untreated ADHD leads to decreased health-related quality of life in children with epilepsy.

o

Many children with absence seizures have a personal history of febrile seizures and/or a family history of a seizure disorder.

o _{Rx: First-line treatment is with ethosuximide. Valproic acid is a 2nd line Rx for} absence seizures. Used less frequently than ethosuximide due to side effects (eg, hair loss, tremor, thrombocytopenia).

 _{Undetected hearing impairment: Undetected hearing impairment can lead to poor}

language skills and social isolation, easily confused with certain pervasive (autism) and behavioral (ADHD) disorders. Hearing tests should be routinely conducted in all

children with any behavioral concerns. ADHD doesn't have problems w/ language skills.

 Selective mutism: characterized by failure to speak in one or more specific social  situations. These children have normal language skills and speak normally in other  _{situations or at home.}