Você está na página 1de 25

Neonatal Condition

Description

Signs/symptoms Preterm birth, IUGR, microcephaly, poor feeding, lethargy, petechiae/purpura, blueberry muffin spots, jaundice, HSM Elevated liver enzymes, anemia, thrombocytopenia intracranial (esp periventricular) calcifications, seizures, chorioretinitis

Ddx

Treatment

Complications

CMV

Transmission during pegnancy/exposure to maternal fluids during birth. Higher transmission rate if mother acquires disease during pregnancy

CMV in urine within first 2 wks of life Supportive; ganciclovir

MCC of infectious sensorineural hearing loss Developmental delay, MR, CP, dental defects

HSV

Perinatal transmission; higher rates if mother has primary infection during pregnancy. Most are born to women who have never experienced symptoms

First 4 wks: 1) isolated mucocutaneous lesions, keratoconjunctivitis 2) encephalitis 3) disseminated disease involving mult organs Hydrops fetalis Mostly asymptomatic LAD/HSM, FTT, DD, encephalopathy, bacterial infections, opportunistic infection, lyphoid interstitial pneumonitis

Viral cx, DFA testing of scrapings. CSF: PCR EEG

IV acyclovir.

Local disease: recurrent mucocutaneous lesions Encephalitis: cataracts/bindness, microcephaly, DD/learning disabilities Disseminated: severe neuro impairment, death None

Parvovirus

Maternal IgM, IgG levels

Intrauterine blood transfusions, supportive Prevention: zidovudine to mother during pregnancy, zidovudine to infant for first 6 wks of life.

HIV

HIV culture, PCR

VZV

Congenital: anytime during pregnancy. Acquired: mothers who develop lesions 5d before- 2d after delivery Transmission at any time during pregnancy

Congenital: IUGR, "zigzag" scarring, limb atrophy, ocular abnormalities Acquired: widespread cutaneous lesions, PNA, hepatitis, death Congenital rubella syndrome: cataracts, sensorineural hearing loss, congenital cardiac defects, DD Maternal antibody titers during pregnancy After birth: elevated rubella IgM or IgG

Rubella

Syphilis

Premature delivery, asymptomatic at birth Transplacental transmission; any Skin lesions, anemia, thrombocytopenia, jaundice, time during pregnancy esp 1st/2nd trimesters "snuffles", HSM, elevated liver enzymes, skeletal abn (osteochondritis, periostitis),

Penicillin G

Neurosyphilis, deafness

Toxoplasmosis

Dermatology Condition

Description

Signs/symptoms

Hand-foot-mouth

Coxsackie A

Ddx Viral infections Prodrome: fever, anorexia, oral pain Crops of ulcers on tongue/oral mucosa, Vesicular rash on hands/feet/buttocks/ thighs - football shaped vesicles Asymptomatic erythematous papular eruption - symmetrically distributed on face, extensor surfaces of arms, legs, buttocks - spares trunk incubation 10-21d prodrome: fever, malaise, anorexia, morbilliform rash next day: pruritic rash on trunk, spreading peripherally. - red papules --> clear vesicles (dewdrop on rose petal) --> crusts - occurs in crops

Treatment

Complications

Supportive

Giannoti-Crosti Syndrome (papular acrodermatitis)

Occurs 1-6 yrs, after URI hepatitis B, EBV, varicella

Order hep B serologies

Supportive - takes up to 8 wks

Supportive - Antipyretics, daily bathing Clinical diagnosis - Tzanck smear - DFA, viral culture, PCR

Primary infection with VZV Varicella fatal disseminated disease in immunocompromised

Progressive varicella: meningoencephalitis, Administer VZV vaccine within 72h of hepatitis, pneumonitis in immunocompromised exposure Acyclovir/valacyclovir/famciclovir not Reye syndrome if indicated for children with taking aspirin uncomplicated primary varicella Antivirals for immunocompromised, pts >12 yrs, children with chronic disease, and those who have received systemic steroids

Herpes zoster

Pain/pruritus Vesicular eruption in crops, confined to dermatome - clears up in 7-14d Uncommon in children <10 Pain persists for wks-months (postherpetic neuralgia) Reactivation of VZV from dorsal root ganglia Poxvirus Small, flesh colored, pearly, umbilicated, dome-shaped papules in moist areas - axilla, buttocks, groin - resolve in 1-2 yrs

Molluscum contagiosum

Common in childhood, wrestlers, sauna bathers HPV

Curretage, cryotherapy, cantharidin, PO cimetidine, imiquimod cream Topical salicylic acid, liquid nitrogen, imiquimod cream, PO cimetidine, PO zinc sulfate, injected immunotherapies (candida antigen), squaric acid dibutylester

Verrucae

Verruca vulgaris: common wart Verruca plantaris: plantar wart Spread by skin-skin contact Verruca plana: flat wart or fomites Condylomata accuminata: genital wart Presumed viral exanthems Viral URI prodrome sometimes Rash begins as herald patch, 2-10cm oval salmon plaque on trunk, neck, UE, or thigh --> smaller lesions in christmas tree pattern over trunk and UE. - fades over 4-12 wks

Pityriasis Rosea

Self limited Antihistamines, topical steroids for pruritis sunlight

Dermatology Condition Unilateral thoracic exanthem (asymmetric periflexural exanthem of childhood)

Description

Signs/symptoms

Ddx

Treatment

Complications

Children 1-5 yrs Winter and spring

Exanthem on one side of trunk that spread centripetally Self limited - 6-8 wks Often confused with - variable: erythematous macules/papules Antihistamines, topical steroids for contact dermatitis with surrounding halo, morbilliform, pruritis eczematous, scarlatiniform, reticulate Skin manifestations of bacterial infections red macules --> bullous (fluid filled) eruptions on erythematous base Can be mistaken for cigarette burns S. aureus can be cultured from vesicle fluid Limited: Muciprocin ointment Numerous: cephalexin (1st gen) - covers staph and strep Suspect MRSA: clindamycin or TMPSMX Remove honey colored crusts with warm compress

Bullous impetigo

S. aureus toxin

Nonbullous impetigo

group A beta hemolytic strep, s. aureus

papules --> vesicles --> 5mm pustules --> rupture --> honey colored crust over ulcerated base Organism can be isolated from lesions

Staph scalded skin syndrome

S. aureus Common in infancy, rare beyond 5 yrs

abrupt onset erythema, skin tenderness, irritability, fever --> flaccid bullae --> rupture --> beefy red, weeping surface - periorificial areas of face, flexural areas around neck, axillae, inguinal creases positive Nikolsky sign: separation of epidermis after light rubbing Unruptured bullae contain sterile fluid

Mild-moderate: PO antistaphylococcal medication Severe: treat like 2nd degree burn

Folliculitis

Buttocks/lower legs of girls who shave S. aureus Deep forms: Infection of hair follicle shaft - furuncle (boil): areas of friction - scalp, buttocks, axillae Hot tubs: pseudomonas - carbuncles: collections of furuncles

antiseptic cleansers, topical muciprocin

Dermatology Condition

Description

Signs/symptoms Silvery scales, tends to be on extensor surfaces, symmetric, Auspitz sign Koebner phenomenon: psoriasis appears at sites of physical/thermal/ mechanical trauma Nail pitting, onycholysis (detachment of nail plate), accumulation of subungual debris. Group A strep: common exacerbation

Ddx

Treatment

Complications

Eczema: pruritic, flexural creases Scalp lesions may look like seborrheic dermatitis or tinea capitis

Psoriasis

Family history, HLA inheritance

Keep skin well hydrated Topical steroids Vit D, UVB light Severe: MTX, immunosuppressants

Psoriatic arthritis

Eruptions secondary to allergic reactions Polycyclic urticaria: can appear Erythematous macules, papules, plaques, targetoid, but vesicles, target lesions lesions are not fixed and do not have - evolve over days PO antihistamines, moist necrotic centers. - fixed, develop necrotic centers compresses, oatmeal baths Edematous, - dorsum of hands and feet, palms and erythematous soles, extensor surfaces, may spread to borders with central trunk clearing, resolving in 12-24 hrs Prodrome 1-14d: fever, malaise, myalgias, arthralgias, HA, emesis, diarrhea Sudden onset fever, erythematous purpuritic macules with duscky centers, inflammatory bullae of mucous membranes - GI, resp, GU tract involvement if severe Positive Nikolsky sign Elevated liver enzymes, renail failure, fluid and electrolyte imbalance Hospitalization, fluid and electrolyte support, moist compresses, oatmeal baths Oral mucosal: mouthwashes with lidocain, diphenhydramine, Maalox Ophthalmology consult Fluid therapy, treat like a burn IVIG: may affect binding/effect of Fas ligand

Acute, self limited hypersenitivity reaction Erythema multiforme - herpesvirus, adenovirus, EBV - recurrent EM: HSV 1

10% mortality if untreated Stevens Johnson syndrome NSAIDs, penicillins, sulfonamides, antiepileptic meds, mycoplasma, immunizations Severe form of SJS involving 30% of body 30% mortality if untreated Upregulated expression of Fas-ligand in epidermis

Toxic epidermal necrolysis

Pulmonology Condition

Definition

Epidemiology

Etiology

Signs/symptoms

Ddx Obstructive lung diseases

Treatment

Complications

High Yield facts

Upper airway obstructive disease

Upper airway: nostril to thoracic inlet - obstruction leads to inspiratory obstruction - obstruction below thoracic inlet leads to expieratory obstruction

Choanal atresia: narrowing of nasal passages Laryngomalacia: large, floppy arytenoid cartilage, MCC congenital stridor Suspect subglottic hemangioma in child with persistent stridor and cutaneous hemangioma Older child: large adenoids and tonsils laryngotracheitis, peritonsillar or retropharyngeal abscess

Noisy inspiration, increased work of breathing (nasal flaring, accessory muscles), retractions (suprasternal) Obstruction of subglottic space: high pitched, monophonic stridor Obstruction above glottis: more variable, fluttering stridor

Flexible bronchoscopy often required to evaluate anatomy and dynamics of Tracheostomy upper airway

BL choanal atresia: presents with life threatening respiratory distress in delivery room. Oxygenation improves when infant is crying.

Obstructive sleep apnea

Pickwickian syndrome: OSA associated with obesity

Restless sleep with position Changes in upper airway changes, irregular snoring, tone during sleep, anatomic daytime somnolence, poor obstruction growth, enuresis

Polysomnography: measure respiratory muscle activity, air flow, oxygenation, sleep stage, and heart rate

Remove tonsils/adenoids, CPAP

severe OSA --> CHF, death Pickwickian syndrome: chronic hypoventilation results in pulmonary hypertension and CHF

Prolonged respiratory infections, Methacholine challenge test decr exerise tolerance, persistent CXR: hyperinflation, increased bronchial markings day/nighttime coughing 15-20% children in US 25% in blacks and hispanics 1) Reversible airway obstruction 2) inflammation 3) bronchial hyperresponsivenes s most common cause of hospitalization >50% present before age 6 Risk factors: genetic, atopy, cigarette exposure, urban impoverished areas, rhinovirus/RSV URIs Acute exacerbation: wheezing, subcostal retractions, nasal flaring, tracheal tugging, prolonged expiratory phase resulting from obstruction - absence of wheezing/decr breath sounds indicate severe airway obstruction - CXR: hyperinflation, focal atelectasis Severity/Control: determined by impairment and risk - Impairment: nighttime awakenings, interference with normal activity, lung function - Risk: exacerbations requiring oral systemic corticosteroids Nasal polyps in any pediatric pt should prompt further testing for CF. Frequent pneumonias: S aureus, H influenza, Pseudomonas aeruginosa (>90%), Burkholderia (associated with accelerated pulmonary deterioration and early death) GI: pancreatic insufficiency, bowel obstruction, rectal prolapse, diabetes, diabetes, hepatic cirrhosis. failure to thrive is most common manifestation of untreated CF. Meconium ileus is pathognomonic Ddx: - intraluminal inflammation or failure to clear secretions: bronchiolitis, GER with aspiration, cystic fibrosis, TE fistula, primary ciliary dyskinesia - intraluminal mass effets: foreign body aspiration, tracheal or brochial tumors or granulation tissue - dynamic arway collapse: tracheobronchomalacia - intrinsic narrowing of airway: congenital/acquired stenosis - extrinsic compression: masses, LN - cough variant asthma: chronic cough triggered by exercise or noted primarily at night during sleep. +/wheezing. Improves with corticosteroids

Remove inciting agents from patient's environment, maintence antiinflammatory medication Acute exacerbation: inhaled bronchodilator Persistent asthma: Inhaled corticosteroids - symptom control, avoidance of exacerbations Also b2 agonists, leukotriene receptor antagonists Theophylline: add on therapy in pts who do not respond to conventional therapy, severe exacerbation. Omalizumab: monoclonal ab vs IgE, use in pts >12 yrs with severe allergic asthma Corticosteroids PO or IV

Asthma

Control vs severity: severity = degree of impairment prior to treatment, control = monitoring impairment and risk of future exacerbations - assessing/maintaining control is more important than assessing severity

Cystic Fibrosis

CFTR channel: cAMP activated chloride channel AR, chromosome 7 on apical surface of delta F508 deletion epithelial cells in respiratory (single nucleotide tract, pancreas, sweat and deletion) salivary glands, intestines, and repro system. Lifespan mid-late 30s Abnormally viscid secretions and impairment of mucociliary clearnace

Elevated sweat chloride (>60mEq/L), pancreatic insufficiency, chronic pulmonary disease. - lower airways: bronchiectasis, parenchymal loss, bleb on CXR Newborn screen

Maintain effective airway clearance, bronchodilators, abx, regular inhaled tobramycin for pts with Pseudomonas, azithromycin, inhaled hypertonic saline

hemoptysis occurs in pts with severe bronchiectasis. Frequent coughin and inlfammation --> erosion of walls of bronchial arteries --> sputum streaked with blood. Blood loss >500mL in 24h requires arterial embolization

spontaneous pneumothorax: place chest tube. 50% recurrence unless Pancreatic enzyme replacement, high pleurodesis is performed calorie high protein diets. Maintain height/weight >25th percentile. chronic pulmonary HTN: results from progressive airway obstruction/hypoxia in advanced disease.

Pulmonology Condition

Definition

Epidemiology

Etiology

Signs/symptoms

Ddx

Treatment

Complications

High Yield facts

Primary ciliary dyskinesia

Impaired mucociliary clearance

DNA11 and DNAH5 mutations

bronchial obstruction, sinusitis, chronic otitis media, recurrent respiratory infections sxs same as asthma, CF

Light microscopy: abnormal ciliary beat Ultrastructural changes in ciliary cells from nasal/bronchial scrapings

Same as pulmonary CF treatment, except do not need pseudomonas treatment.

Bronchiectasis in 2nd or 3rd decade

Pts do not have propensity to Pseudomonas infection

Congenital tracheal stenosis

Tracheal cartilage rings that completely encircle trachea and grow more slowly than the rest of trachea

"washing machine" inspiratory and expiratory noise, hypoxemia, failure to thrive

Tracheomalacia

Widening of posterior membranous portion fo trachea with dynamic collapse during exhalation

wheezing made worse by treatment with bronchodilator Esophageal atresia with TE - bronchodilator makes the fistula is a common cause posterior tracheal membrane more flaccid and more likely to collapse

Bronchomalacia

Poor cartilage or poor elastic recoil in surrounding tissues

Dynamic bronchial collapse on exhalation --> wheezing upon forced exhalation, failure to respond to bronchodilators or steroids

Cardiology Condition

Description

Cardiac exam Cyanotic Congenital Heart Disease: Ductal-independent lesions Systolic ejection murmur at left sternal border - loud ejection click - single S2 - widened pulse pressure; bounding pulses CXR: cardiomegaly, incr pulm vascularity, R aortic arch (30%) ECG: biventricular hypertrophy (70%) Loud, single S2 VSD +/- pulm stenosis: systolic murmur CXR: mild cardiomegaly, incr pulm vascularity. "eggshaped silhouette" - ant aorta superimposed on posterior pulm artery results in narrower mediastium ECG: R. ventricular hypertrophy No obstruction: progressive CHF - R. ventricular heave - wide and fixed split S2 with loud pulmonary component - systolic ejection murmur at L. upper sternal border - CXR: cardiomegaly, snowman contour - ECG: R. axis deviation, R. ventricular hypertrophy

Treatment

Truncus arteriosus

Single arterial vessel arising from base of heart - VSD is always present - Nonspecific murmur at birth - CHF develops in weeks - 22q11 microdeletion (DiGeorge): tetralogy of Fallot, interrupted aortic arch, VSD

Surgery: - close VSD - separate pulmonary arteries from truncal vessel - place conduit between R. ventricle and pulmonary arteries

Parallel systemic and pulm circuits - Most common form of cyanotic CHD in first 24 h of life D-transposition of - patent foramen ovale required to mix circuits great arteries - intact ventricular septum (60%), VSD (30%), VSD + pulm stenosis (10%) Pulmonary veins not connected to L. atrium. - supracardiac (50%): drain into SVC or brachiocephalic vein - cardiac (20%): drain into coronary sinus or R. atrium - infradiagphragmatic (20%): drain into portal or hepatic veins - mixed (10%)

PGE1 Balloon atrial septostomy (Rashkind procedure) - enlarge PFO, incr atrial level mixing Arterial switch procedure

Total anomalous pulmonary venous connection

Surgery

Obstruction: cyanosis, resp distress - Loud, single/narrowly split S2 Obstruction: vein enters at acute - Tachypnea angle. pulm venous HTN and severe - CXR: normal heart size, diffuse pulmonary edema cyanosis - ECG: R ventricular hypertrophy Cyanotic Congenital Heart Disease: Ductal-dependent pulmonary blood flow Complete atresia of tricuspid valve. hypoplasia of R. ventricle. VSD. PFO - Normally related great arteries (NRGA) - ductal independent - Transposition of great arteries (TGA) (30%) - ductal dependent systemic blood flow - VSD in 90% - allows blood to pass from L. ventricle to R. outflow chamber and pulmonary arteries - pulmonary blood flow depends on size of VSD and degree of pulm stenosis Significant pulm stenosis: progressive cyanosis No pulm stenosis: no symptoms TGA: cyanosis, poor feeds Holosystolic VSD murmur at L. lower sternal border Continuous PDA murmur CXR: normal heart size. - TGA: cardiomegaly ECG: Left-axis deviation, R. atrial enlargement, L. ventricular hypertrophy NRGA, pulm stenosis: - PGE1 - Blalock-Taussig shunt (neonate): connect subclavian and pulmonary artery - Cavopulmonary anastomosis (infancy): connect SVC to pulmonary artery - Fontan (2-5 yrs): connect IVC and hepatic vein to pulmonary circulation TGA: - PGE1 - severe arch obstruction: reconstruct aortic arch

Tricuspid atresia

1) VSD 2) Pulm valve stenosis 3) R. ventricular hypertrophy 4) overriding aorta - R-->L shunt through VSD results in cyanosis. Timing/severity depends on Tetralogy of fallot degree of R. ventricular outflow R. ventricular heave obstruction Loud systolic ejection murmur at L. upper sternal border 22q11 microdeletion, most common CHD presenting in childhood CXR: boot shaped heart ECG: R. axis deviation, R. ventricular hypertrophy

Tet spells: episodes of cyanosis, rapid/deep breathing, agitation - incr in R. ventricular outflow tract resistance --> incr shunt across VSD - squatting increases venous return and systemic perfusion

Tet spells: squatting, vagal maneuvers (knee-chest position), O2, morphine sulfate - volume expansion, vasoconstrictors to increase systemic vascular resistance - beta blockers to decr infundibular spasm - sodium bicarb to reduce acidosis and decr pulmonary vascular resistance

Cardiology Condition

Description

Cardiac exam

Treatment

Ebstein anomaly

Severe: Cyanosis and CHF Mild: fatigue, exercise intolerance, palpitations, mild Inferior displacement of tricuspid into cyanosis w/ clubbing R. ventricle, small R. ventricle, enlarged R. atrium, patent foramen Widely fixed split S2, gallop ovale (80%) Blowing holosystolic murmur (tricuspid regurg) - atrialization of R. ventricle - severe tricuspid regurg --> majority CXR: cardiomegaly with R. atrial enlargement and decr of pulm blood flow comes from PDA pulm vascularity - dilated R atrium results in tachycardia ECG: R bundle branch block with R. atrial - Wolff-Parkinson White syndrome enlargement - Maternal Lithium use - Wolff-Parkinson White: delta wave and short PR interval Cyanotic Congenital Heart Disease: Ductal-dependent systemic blood flow

PGE1 Avoid surgery Heart transplant if severe

Hypoplastic left heart syndrome

Atresia/hypoplasia of mitral valve Aortic atresia or stenosis PDA PFO with L-->R shunt Decr blood flow through left heart. Systemic blood flow is completely PDA dependent

Neonates: severely decr systemic blood flow when PDA closes. Shock, tachycardia, tachypnea Right ventricular heave Single S2 Continuous PDA murmur CXR: pulmonary edema and cardiac enlargement ECG: R. ventricular hypertrophy, poor R wave progression

PGE1 No corrective surgery available - only palliative First week: - connect pulm artery and aorta - atrial septectomy - connect R. ventricle to pulm artery (modified blalock-taussig) 3-6 months: - cavopulmonary anastamosis (hemi Fontan) 2-5 years: - modified Fontan

Extreme coarctation of aorta Type A: interruption beyond L. subclavian Interrupted aortic Type B: Betw L. subclavian and L. Same as hypoplastic left heart syndrome arch common carotid (most common) Type C: Betw L. common carotid and brachiocephalic Acyanotic Congenital Heart Disease Paradoxical embolism SVT from atrial enlargement Systolic ejection murmur in pulmonic area (pulmonary valve), middiastolic rumble in lower right sternal border (tricuspid valve) Loud S1, fixed widely split S2 CXR: enlarged heart and main pulmonary artery ECG: R ventricular enlargement

PGE1 Connect interrupted aortic segments

Atrial septal defect

Ostium secundum: midportion Ostium primum: lower septum Sinus venosus defect: Junction of R. atrium and SVC/IVC L-->R shunt: R. atrial and R. ventricular enlargement

Most common congential heart defect Muscular: muscular portion of septum Inlet: endocardial cushion defect, inlet portion of septum below tricuspid Ventricular septal Conoseptal hypoplasia: RV outflow tract below pulmonary valve defect Conoventricular: membranous portion Malalignment: infundibular septum - anterior malalignment results in TOF - posterior malalignment results in aortic stenosis

Eisenmenger Harsh systolic murmur at left sternal border CXR: mild cardiomegaly, incr pulm vascularity, ECG: L atrial, L ventricular, or biventricular hypertrophy Surgery - may not be necessary - patch closure

Cardiology Condition

Description

Cardiac exam

Treatment

Down syndrome Endocardial cushion defect --> ostium Incomplete: Same as ASD. primum ASD, inlet VSD with lack of - Blowing systolic murmur at L. lower sternal border septation of mitral and tricuspid and apex (mitral regurg) valves Common atrioventricular canal defect Incomplete: CAVV leaflets attach directly to muscular portion of ventricular septum. - ASD only. Some mitral regurg Complete: CAVV is not attached to muscular ventricular septum - ASD and large inlet VSD. L-->R shunting at ASD and VSD. Complete: CHF - blowing holosystolic murmur at L lower sternal boder - Widely split, fixed S2 - CXR: cardiac enlargement, incr pulm vascularity - ECG: superior axis (canal defect), enlargement of R and L atria CHF: ace inhibis and diuretics Repair large VSDs within 6 months to decrease risk of pulmonary artery HTN and pulmonary vascular obstructive disease

Patent ductus arteriosus

Premies Symptoms related to size of defect and direction of flow

Large, L-->R flow: CHF, FTT - bounding pulses - continuous murmur after S1, peaks at S2, trails off during diastole - CXR: cardiomegaly, incr pulm vascularity - ECG: L or biventircular hypertrophy Reversal of flow (R-->L): cyanosis Delayed/weak femoral pulses, upper extremity HTN nonspecific ejection murmur at apex bicuspid valve: apical ejection click CXR: enlarged aortic knob, cardiomegaly ECG: R ventricular hypertrophy In neonate, L ventricular hypertrophy in older pt

Indomethacin - risk of renal insufficiency Usually closes by itself in first month of life in FT infant

Coarctation of the Turner syndrome aorta

PGE1 Surgery: anastomosis or patch aortoplastic Interventional: balloon angioplastic Restenosis common

Aortic stenosis

Thickened, rigid, valvular tissue Increased pressure in L ventricle --> L ventricular hypertrophy, decr compliance

Harsh systolic ejection murmur at right upper sternal murmur Ejection click PGE1 Thrill balloon valvuloplasty CXR: cardiomegaly, pulmonary edema ECG: L ventricular hypertrophy. ST depression, inverted T waves (ischemia) Most are asymptomatic Severe: dyspnea on exertion, angina

Dysplastic valve. Incr R. ventricular afterload --> R ventricular hypertrophy Pulmonic stenosis

Ejection click varies with inspiration Harsh systolic ejection murmur on left upper sternal Critical stenosis: decr R ventricle border compliance --> incr R atrial pressure -Severe: thrill, R ventricular heave > opening of foramen ovale --> R to L shunt CXR: enlarged pulmonary artery segment

Infectious disease Fever of unknown origin: Fever >14d, T>38.3C on mult occasions, uncertain etiology Ddx includes: infection, CT disease, malignancy, other (IBD, kawasaki, drug) s/s: conjunctivitis, LAD, joint tenderness, thrush, heart murmurs, organomegaly, etc Diagnostic eval: - CBC + diff, electrolytes, BUN and creatinine, LFTs, alk phos, UA - Blood, urine, stool, CSF cultures - ESR, CRP - CXR, skin test for TB Condition Description Etiology Signs/symptoms Bacteremia and sepsis: Occult bacteremia - appears in well-appearing child with no obvious source of infection - highest in children 2-24 mo, T >39.0C, leukocytosis - MCC S. pneumo, resolves spontaneously Sepsis: bacteremia + systemic response, altered organ perfusion - Neonates: GBS, enteric gram neg, listeria - Children <5: S. pneumo, N. meningitidis - Children > 5: S. aureus - Other: Salmonella, pseudomonas, viridans strep - Evaluation: Blood, urine, CSF cultures, CXR if respiratory signs present Ddx Treatment Complications High Yield facts

Acute otitis media

Narrowing of eustachian tube by edema in URI --> Inflammation of middle ear vaccuum draws secretions from Eustachian tube in children: nasopharynx to - angle of entry (horizontal) middle ear - short length - decr tone MCC: - S. pneumo - H. influenza - Moraxella

Ear pain, fever, fussiness, URI sxs TMs bulging, opaque, aberrant light reflex, decr mobility

Otitis media with effusion: fluid behind TM but no inflammation (no fever/ear pain) - no abx Myringitis: inflammation of eardrum - no abx Otitis externa (swimmer's ear): ear pain, but TMs looks normal, erythematous canal - topical abx drops

1. High dose amoxicillin, topical abx if perforated Hearing loss, otitis media 2. augmentin, PO second or 3rd externa gen cephalosporin, or IM ceftriaxone Meningitis (MC intracranial) S. pneumo: 50% penicillin resistant. H. influenza and M. catarrhalis: beta- lactamase activity Atopic constitution increases risk for recurrent OM

Decr mobility of TM is most specific sign

Mastoiditis: severe, but uncommon Consider tympanostomy tube if - high fever, tenderness of moderate hearing loss, recurrent mastoid bone AOM Similar abx as AOM but longer treatment course (14-21d) Recurrent disease: sinus aspiration

HA, facial pain, sinus tenderness Sinusitis Same pathogens as Acute bacterial: 1) persistent AOM respiratory sxs (10-14d) 2) severe sxs (high fever, purulent nasal discharge) High fever, very sore throat Vesicular lesions progressing to ulcers - soft palate, tonsils, pharynx Viral URI, allergic rhinitis, nasal foreign body

Complications uncommon - bony erosion, orbital cellulitis, intracranial extension

Herpangina

Diagnosed during summer and fall in young children

Enteroviruses: coxsackievirus, etc

Primary herpetic gingivostomatitis:lesions are more widespread over gums, lips, mucosa Hand foot mouth dz: also lesions - HSV on palms and soles Viral pharyngitis, infectious mononucleosis Definitive diagnosis requires throat culture or antigen detection test for GAS Rapid antigen detection: high specificity, variable sensitivity (depends on quality of swab). confirm negative test with throat culture CMV, toxoplasma gondii, HHV6, adenovirus, HIV Pancytopenia indicates malignancy Severe exudative pharyngitis, fever, profound fatigue Leukocytosis or leukopenia, 50% lymphocyte, at least 10% atypical lymphocytes Heterophile antibody: limited sensitivity in pts <4 Thrombocytopenia, elevated hepatic transaminase levels
10

Self limited (5-7d)

Streptococcal pharyngitis

School aged children and adolescents, spread via oral Group A strep secretions

sore throat, f/HA/n/abd pain PE: enlarged erythematous, exudative tonsils, petechiae on soft palate No rhinorrhea, hoarseness or coughing Scarlet fever: sandpaper rash with fever and pharyngitis - begins at neck/axillae/groin, spreads to extremities, may desquamate

Suppurative: peritonsillar abscess, retropharyngeal abscess Nonsuppurative: rheumatic fever, poststreptococcal glomerulonephritis PO penicillin (10d)

Do not treat pharyngitis with abx empirically bc most causes are viral Rheumatic fever: 3wk following make therapeutic pharyngitis. Penicillin decisions based on prophylaxis to prevent throat culture or rapid recurrent ARF antigen detection Poststrep glomerulonephritis: results occurs following either pharyngitis or skin infection - penicillin therapy and

Primary EBV infection in older children and adolescents Majority of people are Infectious mononucleosis infected with EBV and seroconvert in early childhood (usu asymptomatic) EBV

Rare but serious: upper airway obstruction (rx: Self limited, supportive care corticosteroids), splenic Activity restrictions due to risk of rupture, meningoencephalitis splenic rupture Immunocompromised at risk for disseminated disease and lymphoproliferative disorders

Pts infected with EBV who receive amoxicillin (for misdiagnosed bacterial infection) may have maculopapular rash

Condition

Description

Etiology

Signs/symptoms

Ddx AP neck and chest radiographs show steeple sign (<50%) Ddx: epiglottitis, bacterial tracheitis, foreign body aspiration, anaphylaxis, angioneurotic edema - Epiglottitis: life-threatening emergency, thumbprint sign. Child drools and leans forward with chin extended. Emergent intubation or cricothyroidotomy. IV ampicillinsulbactam. Biggest risk factor is failure to maintain Hib vaccination status

Treatment

Complications

High Yield facts

Croup Acute laryngotracheo Most pronounced in young bronchitis children due to narrow caliber of subglottic region. Peaks in late fall/winter

Virus induced inflammation of laryngotracheal tissues, resulting in upper airway obstruction

Paramyxovirus (parainfluenza?), can also result from influenza or RSV

Hoarse voice, barky/seal like cough, inspiratory stridor which may progress to respiratory distress Prodrome: low grade fever and rhinorrhea 12-24h prior to onset of stridor

Cool night air/humidity for cough and stridor Resolves in 4-7d ER: give cool mist, racemic epinephrine, PO/IV/IM corticosteroids

Acute viral lower respiratory tract infection resulting in inflammatory obstruction of the peripheral airways At least 50% of children are infected before 1 yr of age, recurrent infections are common. 3% of infants in first 12 mo of life are hospitalized for bronchiolitis Chornic lung disease, congenital heart disease, immunodeficiencies are risk factors RSV, also parainfluenza, influenza, human metapneumovirus, adenovirus Infected neonates may develop life threatening apnea Initial fever, cough, rhinorrhea followed by respiratory distress PE: wheezing, rhonchi, crackles, accessory muscle use

Bronchiolitis

Rapid assays from nasal secretions for RSV, influenza A and B, etc CXR for ill or hypoxic pts or recurrent wheezing - lung hyperinflation, peribronchial thickening (cuffing), incr interstitial markings

Supportive, self limited Pavalizumab (IM RSV More airway monoclonal ab): passive hyperresponsiveness later in prophylaxis. Give in winter to at life risk pts <2 yrs (heart dz, chronic lung disease of prematurity, premies <35 wks)

Pertussis

Almost all are afebrile Catarrhal phase: 7-10d incubation, 1-2 wks low-grade URI and persistent cough in fever, cough, coryza adults, life threatening Bordetella pertussis Paroxysmal phase: spasms of disease in neonates/infants coughing followed by sudden inhalation ("whoop") Convalescent phase: most sxs resolve by cough persists 2-8wks C. trachomatis: afebrile, conjunctivitis, staccato cough M pneumo and C. pneumo: f/HA/myalgia M pneumo: macular/erythematous rash, erythema multiforme

Leukocytosis with lymphocyte predominance PCR or culture of organisms in nasopharyngeal secretions CXR usually normal, infiltrates may be seen

Hospitalized to manage apnea, cyanosis, hypoxia, and feeding difficulties Erythromycin or azithromycin only in catarrhal phase, decreases infectivity, prophylaxis in close contacts

If pleural effusion: drain, cell count, gram stain, culture If high fever in child: blood culture rapid influenza test, DFA/PCR/tissue culture for C. trachomatis M pneumo: PCR or cold agglutinins

Pneumonia

Young children: viruses MCC acute inflammatory process C. trachomatis at 2-3 occuring in lungs Viral: diffuse wheezing and mo crackles S. pneumo, etc Bacterial: focal crackles/decr percussion/egophony/bronchoph ony Young child: tachypnea out of proportion to fever

High dose amoxicillin or amox/clav for most bacterial pna. Erythromycin/azithromycin/ clarithromycin for M pneumo or C pneumo. Azithromycin/Erythromycin for C. trachomatis Neonates: ampicillin and cefotaxime (or gentamicin)

Pleural effusion --> compromise respiratory effort. Chest tube - most large pleural effusions are caused by s. aureus pneumonia Lung abscesses due to anaerobic infections

Cold agglutinin titers are elevated in M. pneumo, many viral and some bacterial pneumonias

11

Condition

Description

Etiology

Signs/symptoms n/v, photophobia, irritability, lethargy, HA, stiff neck

Ddx

Treatment

Complications

High Yield facts

Meningitis

Viral prodrome: f/malaise, sore throat, myalgias. Usually resolves Infection of leptomeninges in 2-4d and CSF MCC enterovirus Bacterial: no prodrome. High Bacterial: S. pneumo fever Neonates: low birth weight, and N. meningitidis - HTN, bradycardia, apneic, incr prolonged rupture of (neonates and ICP membranes, children <3 are Lyme: low grade fever, HA, stiff chorioamnionitis predispose highest risk) neck, photophobia over 1-2 wks to septicemia and meningitis Kernig: knee extension --> flexion of hip with pain Brudzinski: passive neck flexion --> involuntary leg flexion Excessive stooling --> dehydration, inadequate nutrition, Salmonella, Shigella, electrolyte abnormalities E. Coli, Yersinia enterocolitica, Bacterial diarrhea: fever, Campylobacter abdominal cramping, malaise, jejuni, Vibrio cholera tenesmus, vomiting is less common. Stools w/ mucus, guiaic Rotavirus: major positive cause of nonbacterial Shigella: neurologic sxs gastroenteritis in Salmonella: extraintestinal infants and toddlers disease (meningitis and osteomyelitis) Giardiasis: MC Shigella and E. Coli: hemolytic intestinal disease in uremic syndrome US Yersinia: erythema nodosum, pseudoappendicitis Enterobius vermicularis

Encephalitis, drug intoxiation or s/e, anoxia/hypoxia, primary or metastatic CNS malignancy, bacterial endocarditis with septic embolism, intracrainial hemorrhage/hematoma, malignant HTN, demyelination disorders

Neonates: ampicillin for GBS and Listeria + cefotaxime Child: vancomycin + 3rd gen cephalosporin Abx usually 10-14d

CSF analysis diagnostic: cell counts rifampin prophylaxis in close + diff, gram stain, glucose and contacts protein levels, culture PCR for HSV and enteroviruses

10-20% develop persistent neurologic deficit: hearing loss, developmental delay, motor incoordination, seizures, hydrocephalus

Dp not attempt LP in a child with focal neurologic deficits and/or increased ICP until an expanding mass lesion is excluded by CT or MRI

Gastroenteritis

Electrolyte and renal function studies Blood culture at time of initial evaluation

Abx prolongs Salmonella shedding, increases risk of hemolytic uremic syndrome. TMP SMX or azithromycin for shigella, erythromycin or azithromycin for C. jejuni

Pinworm

Perianal, vulvar itching

Adhesive tape stool O&P not recommended - few ova in stool Ddx: EBV, CMV, enterovirus, other viral infections. Autoimmune heptatitis, metabolic liver disease, biliary tract disorders, drug ingestions

reinfections are common mebendazole, pyrantel pamoate, handwashing is best or albendazole prevention HAV: fulminant hepatitis rare but mortality up to 50% HBV: chronic infection HDV: HDV and HBV simultaneously infection puts pt at greater risk for more severe chronic hepatitis B and higher mortality rate. HDV superinfection on top of exisiting HBV results in acute exacerbation and acclerated course result. HCV: 50% develo[ chronic hepatitis

Perinatally infected: asymptomatic HAV and HEV: diarrhea Hepatitis Carrier state associated with hepatocellular carcinoma Scleral icterus or jaundice, hepatomegaly, RUQ tenderness, benign-appearing rash in early HBV

HAV: immune globulin, HAV: anti-HAB IgM antibody = administer within 14d of infection exposure HCV antibody: present in acute and chronic, 12 wk window period HCV RNA: positive wtihin 1 wk - HCV RNA with negative antibody = acute infection (window period) - negative HCV RNA = recovery VDRL and RPR: tests for antibodies to lipoidal molecule rather than organism itself. - False positives in infectious mono, connective tissue disease, endocarditis, and TB IM or IV penicillin G FTA-ABS and particle agglutination: fewer false positives Newborns: lumbar puncture to identify neurosyphilis - pleocytosis and elevated protein
12

Syphilis

Congenital or sexually acquired. Commonly coinfection with other STD.

Congenital: hepatomegaly, splenomegaly, mucocutaneous lesions, jaundice, LAD, snuffles (bloody, mucopurulent nasal discharge) Treponema pallidum Sexually acquired: primary chancre that heals in 3-6 wks, secondary dermatologic involvement (palm and sole rash), tertiary gummas in skin, bone heart, CNS

Untreated infants may develop anemia, thrombocytopenia, and radiography abnormalities of long bones

Condition Genital HSV infection

Description

Etiology HSV 2 or 1

Signs/symptoms

Ddx

Treatment

Complications

High Yield facts

Pelvic inflammatory Disease

Usually polymicrobial

C. trachomatis, N. gonorrheae - also anaerobes and enteric gram negs

NAAT tests for gonorrhea and chlamydia Rx for N. gonorrhea, C. Either cervical motion tenderness - must also offer testing for syphilis, trachomatis, anaerobes or uterine/adnexal tenderness HIV, and other STI - pregnancy test C trachomatis and N. gonorrhea: Additional criteria: oral T>101, Single dose IV ceftiaxone + PO elevated ESR or CRP, WBCs in Gyn ddx: mucupurulent cervicitis, doxycycline vaginal secretions, mucopurulent ectopic pregnancy, ruptured ovarian discharge, lab evidence cyst, septic abortion, endometriosis Anaerobes: metronidazole Nongyn ddx: appendicitis, pyelonephritis, IBD Trichomonas: most asymptomatic, malodorous, frothy gray discharge and vaginal Trichomonas: PMNs and discomfort. Penile discharge trichomonads on wet prep

Decreased fertility, ectopic pregnancy, dyspareunia, chronic pelvic pain, adhesions N. gonorrhea: arthritis C. trachomatis: Reiter syndrome Both: Fitz-Hugh-Curtis syndrome (perihepatitis)

Candida occurs in women with abx use, pregnancy, Vulvovaginosis diabetes, immunosuppression, OCP use

Candida, Trichomonas, bacterial

Bacterial: thin, white, foulBacterial: vaginal pH > 4.5, clue smelling discharge with fishy odor cells when mixed with KOH Candida: yeast and pseudohyphae Candida: thick white vaginal on wet prep with KOH discharge with vaginal itching and burning Diagnosis: mucoid or purulent urethral discharge, pos leuk esterase, WBCs on first-void urine, gram neg intracellular diplococci on gram stain - test pts for other STDs

Trichomonas: PO metronidazole Bacterial: PO metronidazole Candida: antifungal creams or single dose of PO fluconazole

Urethritis

N gonorrhea and C trachomatis - also mycoplasma genitalium and trichomonas vaginalis AIDS: AIDS-defining illness occurs or when CD4+ lymphocyte count is less than a defined number for age >90% in utero or perinatal Risk of transmission is 25% if untreated

Urethral discharge, itching, dysuria, frequency.

Gonococcal: 230mg IM ceftriaxone AND either 1 dose PO azithromycin OR 7d of PO doxycycline - azithromycin preferred because it covers mycoplasma genitalium Pregnant women: zidovudine (AZT), followed by treatment of infant for first 6 wks of life reduces vertical transmission to 2% NRTI, NNRTIs, PIs TMP-SMX prophylaxis against PCP

HIV and AIDS

Generalized LAD, hepatomegaly, splenomegaly, failure to thrive, diarrhea, cadidiasis, parotitis, and ELISA and Western blot not useful developmental delay in children <18 months Resp: lymphoid interstitial PNA, PCP PNA Fever, HA, rash 7d after tick bite - f/c/HA/n/v/myalgias - rash on 2nd-5th day, blanching erythematouc macular lesions that prgress to petechiae or purpura - starts on wrists/ankles and spreads inward Erythema migrans (early localized disease, 3-30d after bite), f, HA, myalgias Borrelia burgdorferi No reliable diagnostic test. Abs confirm diagnosis 10d after sxs Thrombocytopenia and hyponatremia

Rocky Mountain Spotted Fever

Tick bite (wood tick, dog tick, lone star tick) April-Sept

Empiric rx - need to cover erlichiosis and N. meningitidis

Rickettsia rickettsii

doxycycline add cefotaxime or ceftriaxone if Ddx: erlichiosis, meningococcemia, suspecting meningococcemia atypical measles Atypical rash may be confused with erythema multiforme or erythema marginatum

Lyme disease

Tick bite (deer ticks, black legged ticks) - infected tick must feed for >48h

Treatment prevents early disseminated and late disease Ddx arthritis: juvenie idiopathic Early (days--> wks): multiple arthritis, reactive arthritis, Reiter Young children: amoxicillin or erythema migrans lesions, cranial syndrome. cefuroxime nerve palsy meningitis Ddx meningitis: aseptic meningitis Children >8yrs: PO doxycycline Late (>6wks): arthritis, usually involving knee Clinical diagnosis, elevated IgM titer wks after tick bite

13

Heme Condition

Description

Signs/symptoms

Ddx Microcytic anemias

Treatment

Complications

Occurs as early as 3 mo in premie. Risk factors: extended exclusive Mild (6-8 g/dL): decr appetite, irritability, fatigue, decr breastfeeding (>6 mo), low-iron formula, exercise tolerance. Skin/mucous membrane pallor, low-iron solids, excessive cow milk. tachycardia, systolic ejection murmur along left sternal border Response to appropriate iron Occult blood loss: GI anomalies (meckel, supplementation is best diagnostic test juvenile polyps) Severe (<3 g/dL): CHF, tachycardia, S3 gallop, Overt loss: bloody stools or traumatic cardiomegaly, hepatomegaly, distended neck veins, rales hemorrhage Glossitis, angular stomatitis, koilonychia in children with isolated iron def anemia in developed nations Hb Barts: Gamma-globin tetramers. High affinity for oxygen --> hypoxia, heart failure, HSM, edema, hydrops Alpha thal major: HbBarts fetalis HbH disease: 10-40% HbH, 60-90% HbA Alpha thal trait: normal electrophoresis, HbH: beta globin tetramer. HbH disease: deletion of 3 similar to Fe deficiency out of 4 alpha genes. - at birth: HbBarts predominates Beta thal major: normal blood counts at - first few months: HbH predominates birth. - anemia, HSM, require intermittent transfusions - hypochromia, microcytosis, anisocytosis, poikilocytosis Alpha thal trait: deletion of 2 genes. Blacks and - elevated HbF Mediterranean. Often confused wtih Fe-def anemia

Iron deficiency anemia

Iron supplement: reticulocyte count incr within 3d, Hb concentration normalizes within a month. Continue therapy for 2-3 mo to replenish stores and prevent future occurrence.

Alpha thal: deletions Beta thal: point mutations Alpha and Beta thalassemia Imbalance between alpha and beta chains --> excess of one type --> unstable monomers that precipitate and damages membrane

Thalassemia major: RBC transfusion to eliminate anemia, suppress extramedullary erythropoiesis, decr iron overload - goal is to maintain Hb >10g/dl - Splenectomy with incr transfusion requirement

Thalassemia intermedia (alpha or beta): folic acid supplements for pts not on Beta thal minor: elevations of HbA2 and/or transfusion therapy Beta thal: normal blood counts at birth (mostly fetal Hb), severe anemia, organomegaly, growth failure during HbF first year. Extramedullary hematopoiesis --> frontal bossing, maxillary hypertrophy, overbite TIBC low, ferritin normal or incr Mild anemia (8-10 g/dL) BM exam: Incr in storage iron, decr in ironTreat underlying inflammation containing erythroblasts Usually normocytic initially Nonmegaloblastic macrocytic anemias Presents in first year of life. Macrocytosis, reticulocytopenia elevated HbF 25% have associated anomalies: short stature, web neck, Elevated RBC adenosine deaminase cleft lip, shield chest, triphalangeal thumb Macrocytosis Elevated HbF 10% develop leukemia Confirm diagnosis by demonstrating increased chromosomal breakage with exposure to diepoxybutane (DEB) CBC: cytopenia, microcytosis Anemia, thrombocytopenia, neutropenia Diagnosis: peripheral pancytopenia + hypocellular bone marrow Disorders of hemostasis PO corticosteroids BM transplant

Beta thal major: Iron overload due to hyperabsorption of dietary iron or transfusional iron loading - cardiomyopathy, CHF - cardiac disease is main cause of death

Anemia of inflammation

Inflammation --> incr hepatic production of hepcidin --> internalization/degradation of ferroportin --> impaired release of iron from macrophages and absorption of iron from gut

Diamond-Blackfan Anemia

Congenital pure red cell aplasia mutation in ribosomal protein S19 (RPS19)

Fanconi anemia

AR or X linked, pancytopenia Defect in DNA repair

Hyperpigmentation, caf au lait spots, microcephaly, microphthalmia, short stature, horseshoe/absent kidney, absent thumbs

RBC transfusions Hematologic improvement with androgen therapy SC transplant - decrease doses of radiation/chemo bc they damage DNA

Severe Aplastic Anemia

Failure of hematoietic stem cells --> pancytopenia - due to chemicals (benzene), drugs (chloramphenicol, sulfonamides), infectious agents (hepatitis), ionizing radiation. Usually idiopathic.

BM transplant Immunosuppression if no donor can be found

Immune thrombocytopenia

Antiplatelet autoantibodies --> destruction by RES - primary, or secondary to SLE or HIV

CBC: normal, except thrombocytopenia - large, young platelets Abrupt onset petechiae/bruising 1-4wks after febrile/viral illness Diagnosis based on history, physical, blood count. Does not require BM exam, lab testing, or antibody detection

Usually self limited Corticosteroids, IVIG, anti-D immunoglobulin can temporarily increase platelet count Factor therapy to prevent joint damage from recurrent hemarthrosis ( hemophilic arthropathy) DDAVP: releases factor VIII from endothelial cells

Hemophilia A and B

Hemophilia A: factor VIII Hemophilia B: factor IX

Unexplained post-op bleeding Newborns: intracranial bleeding from delivery, bleeding after circumcision (avoid circumcision)

Older pts: received blood products with HIV --> AIDS is MCC death Formation of inhibitors: neutralizing IgG abs vs factor VIII or IX

14

Aminocaproic acid: inhibits fibrinolysis

Onc Condition

Description

Signs/symptoms

Ddx Leukemia

Treatment

Complications

Neutropenia (ANC < 500/mm3) predisposes to serious bacterial and fungal infections. Induction therapy: 28d of vincristine, steroids, intrathecal MTX, asparaginase. Add daunomycin for high risk pts. Consolidation: intensification of therapy to kill additional leukemic cells if induction fails Interim maintenance: vincristine, 6MP, MTX Radiation therapy for CNS and testicular disease, CNS prophylaxis. High risk of tumor lysis syndrome: hyperuricemia, hyperphosphatemia, hyperkalemia - especially in T cell ALL or Burkitt lymphoma - greatest risk in first 3d of chemo - maintain Hb <10mg/dL Hyperleukocytosis (WBC>200,000): can cause vascular stasis --> mental status changes, HA blurry vision, dizziness, sz, dyspnea Mediastinal mass --> SVC syndrome: distended neck veins, swelling of face/neck/upper limbs, cyanosis, conjunctival injection

Most common pediatric cancer (75%)

Normochromic normocytic anemia, low retic count, low WBC count variable

ALL

Most common is precursor B cell HSM and cervical LAD at diagnosis. (80%) good prognosis; T cell ALL Extramedullary involvement in CNS, (19%), Mature B cell (Burkitt) (1%) skin, testicles (5%). - HA emesis, papilledema, CN6 palsy Poor prognosis: age >10 yrs or <1 yr, WBC >50,000 at Fever, mediastinal mass (mostly T diagnosis, failure to respond to cell), induction therapy, Philadelphia chromosome

20% childhood leukemias Best prognosis: M3 AML (APML) and trisomy 21 pts with M7 AML AML Worse prognosis: WBC >100,000/mm3 Low risk: inv 16, t16;16, t8;21 High risk: monosome 5 or 7, or no remission Lympoblastic (50%): pre-T or preB Burkitt (35%) or large B cell T cell lymphoblastic lymphoma: Anaplastic large cell (15%) mediastinal mass B cell lymphoblastic lymphoma: bone 3rd most common malignancy in involvement, isolated lymph nodes, skin childhood, 10% of childhood Burkitt: abdominal tumor w/ n/v, cancers. Boys 3x as many as girls intussusception, tonsils, bone marrow, CNS Risk factors: congenital Anaplastic large: slowly progressive immunodeficiency (Wiskott disease with fever Aldrich, SCID), acquired immunodeficiency, Bloom syndrome, ataxia telangiectasia Chemotherapy more intense than ALL Induction: anthracycline + arabinoside Low risk: chemo only High risk: BM transplant Hyperleukocytosis (5-22%): dyspnea, hypoxemia, etc - treat earlier than ALL bc AML cells are larger and stickier than lymphocytes in ALL. - maintain Hb < 10 mg/dL

Chloroma: soft tissue tumor in spinal cord, brain, skin Leukemia cutis: Neonates, Blueberry muffin spots Gingival hypertrophy

Non-Hodgkin lymphoma

CBC: leukocytosis, thrombocytopenia, anemia CMP for tumor lysis CXR for mediastinal mass BM aspiration and biopsy with flow cytometry LP for CNS involvement CT scan to assess extent ot disease

Combination chemo Burkitt: surgical resection - tumor lysis: careful management with incr fludi intake, alkalinzation of urine, electrolyte observation, allopurinol - high risk of developing kidney failure requiring dialysis from tumor lysis

Mediastinal mass: SVC syndrome Burkitt: tumor lysis syndrome - even before chemo is started.

15

Onc Condition

Description

Signs/symptoms

Ddx Ddx reactive/inflammatory nodes: bacterial lymphadenitis, infectious mono, TB, atypical mycobacterial infection, cat scratch, HIV, histo, toxo

Treatment

Complications

Increase incidence of immune dysregulation Association with EBV Incr risk in ataxia teleangiectasia, Wiskott-Aldrich, Bloom Bimodal distribution Hodgkin lymphoma Subtypes: - Nodular sclerosing (40-55%) - Lymphocyte predominant (1015%) - Mixed (30%) - Lymphocyte depleted (5%)

Painless, rubbery, cervical LAD (80%) B symptoms: fever, night sweats, weight loss Enlargement of liver/spleen in advanced disease

1. CXR for mediastinal Multiagent chemo involvement, airway compromise Lymphocyte-predominance has best 2. Pulmonary function test, prognosis ECHO before anesthesia in pts with mediastinal mass 3. Excisional lymph node biopsy required for diagnosis: Reed sternberg cells Eosinophilia (15-30%) CNS tumors

Secondary malignant neoplasms (breast, thyroid, sarcomas), cardiac toxicity (anthracyclines), pulm (bleomycin), hypothyroidism (XRT)

Neuroblastoma

Childhood embryonal malignancy of postganglionic sympathetic nervous system. 8% of all childhood cancers <15yrs, most common solid tumor outside CNS. - abdominal tumors (70%) from sympathetic ganglia/adrenal medulla, thoracic masses (20%) from paraspinal ganglia, neck (5%) involves cervical ganglion

Abdominal tumors: hard smooth, nontender abdominal masses palpated in flank, displace kidney. Neck: Horner syndrome, heterochromia of iris on affected side Epidural invasion --> back pain and cord compression sxs Metastatic sequelae: - Hutchinson syndrome: cortical bone pain causing limp - Pepper syndrome: liver infiltrate causing hepatomegaly Raccoon eyes: periorbital infiltratex

Ddx: adrenal hemorrhage, hydronephrosis, polycystic kidney disease, splenomegaly, renal cell ca, wilms tumor, hepatoblastoma, leukemia, lymphoma, retroperitoneal rhabdomyosarcoma

Prognosis: INSS staging, DNA index of tumor, MYCN gene amplification - Stage I, II, IVS have good prognosis. Stage III, IV have poor prognosis Surgery, chemo, radiation, etc. Chemo: vincristine, cyclophosphamide, doxorubicin, cisplatin

Confirm mass by CT of chest/abd/pelvis tumor cells on BM aspirate + elevated urinary catecholamines Paraneoplastic effects: watery diarrhea Tissue biopsy for histology, in tumors secreting VIP, opsoclonus DNA ploidy, and MYCmyoclonus (chaotic eye movements, related oncogene myoclonic jerking, truncal ataxia)

16

Onc Condition

Description

Signs/symptoms Associated anomalies: sporadic aniridia, hemihypertrophy, cryptoorchidism, hypospadias, other GU anomalies

Ddx

Treatment

Complications

Wilms tumor

Most common renal tumor in children, chromosomal loci 11p13 and 11p15 - most are unilateral - most common renal tumor - usually diagnosed in first 5 yrs

Beckwith-Wiedemann: hemihypertrophy, macroglossia, omphalocele, GU abn WAGR: Wilms, aniridia, GU abn, mental retardation Perlman syndrome: unusual facies, islet cell hypertrophy, macroscomia, hamartomas Other findings: hematuria, HTN, varicocele Von willebrand's disease in 8%

Ddx: hydronephrosis, PKD, Surgical removal of kidney splenomegaly Radiation therapy can be used for metastatic sites Abd US, CT, or MRI Good prognosis: small size, younger Staging done after age, no LN/metastases, no capsular/vascular invasion exploratory laparotomy

Invasion of renal capsule, extension through adjacent vessels (IVC), regional nodes, lung, liver Lung most common site of metastasis

Bone tumors Ddx: osteomyelitis, eosinophilic granuloma (langerhans cell histiocytosis), osteosarcoma, neuroblastoma or rhabdomyosarcoma metastasis to bone Radiographs: lytic bone lesion with calcified periosteal elevation (onion skin) and/or soft tissue mass Ddx: ewing sarcoma, benign bone tumors, chronic osteomyelitis Pain and localized swelling Systemic manifestations rare (vs Ewing) 20% have mets: lung most common Gait disturbance, pathologic fractures lytic bone lesion with periosteal reaction (sunburst appearance) MRI to assess extent, CT to detect pulm mets (calcified nodules), bone scan for mets DDx: congenital cataract, medulloepithelioma, Toxocara canis endophthalmitis, persistent hyperplastic primary vitreous, Coats disease Ophthalmologic exam, MRI Soft tissue sarcomas
17

Ewing sarcoma

Undifferentiated sarcoma that arises primarily in bone - t(11;22) in 85% of pts Pain and localized swelling adolescents Systemic: fever, weight loss, fatigue - flat and long bones: femur (20%), pelvis (20%), fibula (12%), humurs and tibia (12%) - begins midshaft in long bones

Chemo + radiation - chemo: reduce size and treat mets (almost all pts have mets)

Osteosarcoma

Tumor of bone-producing mesenchymal stem cells - arises in medullary cavity or periosteum - usually at metaphysis of bones with maximum growth velocity: distal femur, proximal tibia, proximal humerus

Surgical removal of primary tumor Resistant to radiation therapy (vs ewing) Chemo: cisplatin, doxorubicin, MTX

Retinoblastoma

Tumor of embryonic neural retina Chromosome 13q14, BR1 locus - 60% unilateral

Leukocoria (absent red reflex) Trilateral rb: involvement of pineal or parasellar sites

Enucleation, chemotherapy, local therapies, radioplaques, external beam radiation - treatment depends on ReeseEllsworth classification Child born to parent with BL retinoblastoma or unilateral rb with known genetic mutation should be screened for rb at birth and at regular intervals until 4 or 5 yrs

Onc Condition

Description

Signs/symptoms Head and neck (35%), GU (22%), extremeties (20%) 25% have distant mets, lung most common site

Ddx

Treatment

Complications

Most common STS in children <10 - Associated familial syndromes: neurofibromatosis, Li Fraumeni Rhabdomyosarcoma - 2 subtypes: embryonal (53%), alveolar (21%) - t(2;13) and t(1;13) Heterogenous Malignant peripheral nerve sheath Non tumors: associated with NF type I rhabdomyosarcoma - Malignant fibrous histiocytoma or leiomyosarcoma

CT or MRI of site, chest CT Surgery, radiation, chemo and bone scan for mets - chemo helps reduce tumor size and bone marrow biopsy mets required

CT or MRI of site, chest CT Surgery, radiation, chemo and bone scan for mets

18

Immunology, Allergy, Rheum Condition Description

Signs/symptoms

Ddx Immunology Decreased absolute lymphocyte counts and T cells. Absent T cell function: in vitro mitogen stimulation, intradermal delayed hypersensitivity testing Absent antibody function (measure after 6 mo) DiGeorge: absent thymic shadow on CXR, FISH

Treatment

Complications

Viruses, mycobacteria infections, fungi, PCP X linked SCID: presents in first 6 mo with 1. X-linked: IL2RG gene - gamma viral/bacterial infections, diarrhea, failure to thrive. chain of IL-2 receptors Lymphopenia, absent CD4 cells 2. DiGeorge Digeorge: congenital heart disease, hypocalcemic tetany, malformed ears/face

T cell immunity: SCID

Immunoglobulin replacement, aggressive identification and treatment of infections BM transplant

Vaccine associated diarrhea with live/attenuated rotavirus vaccine

Diagnostic evaluation: - IgG, IgA, IgM levels - serum protein screen - albumin or X linked agammaglobulinemia transferrin to r/o other etiologies (Bruton): no mature B cells. Life - antibody titers after immunization threatening enterovirus infections (tetanus and diptheria = protein antigens; pneumococci and H CVID: hypogammaglobulinemia, Humoral immunity: Recurrent infections with encapsulated organisms influenza = carb antigens) esp IgG and IgA. Decr antibody Antibody deficiency after 6 mos- otitis media, sinusitis, pneumonia formation to vaccines. Incr syndromes Transient lymphoma and autoimmune dz hypogammaglobulinemia of infancy: delayed acquisition of Selective IgA deficiency: IgA <5 normal infant immunoglobulin levels mg/dl, bacterial infections of - most develop normal levels by 2-5 respiratory, GI, and urinary tracts yrs, have intact responses to vaccination Incr susceptibility to virulent and opportunistic Wiskott-Aldrich: X linked, B and infections, autoimmunity T cell disorder. Host's abs do not respond to carbohydrate antigens Wiskott Aldrich: atopic dermatitis, thrombocytopenia. Hyper IgM: CD40L mutation. Failure of class switching. Hyper IgM: recurrent sinopulm infections, PCP Gingivitis, skin infections, rectal inflammation, otitis media, pneumonia, sepsis. S. aureus and gram neg Insufficient number of PMNs infections. (neutropenia), cell dysfunction, or - absense of inflammatory response --> no migration defect erythema, warmth, or swelling Phagocytic immunity Chronic granulomatous disease: most common. Failure to generate superoxide CGD: chronic/recurrent pyogenic infections by catalase pos organisms, abscesses. Failure to thrive, diarrhea, persistent candidiasis of motuh Neutropenia ddx: infection (esp viruses), medication administration (penicillin, sulfonamides, anticonvulsants), malignancy in BM, aplastic anemia - severe: ANC <0.5x10^3 - chronic: >2-3 months CGD: WBC betw 10,000-20,000, leukoerythroblastic response - nitroblue tetrazolium test, dihydrorhodamine reduction (DHR)

IVIG

Combined immunodeficiency

Acute neutropenia: no treatment Chronic neutropenia/infections: recombinant human gametocyte colony stim factor (rhG-CSF) CGD: prophylactic TMP-SMX. BM transplant LAD: BM transplant.

Leukocyte adhesion deficiency: LAD: WBC counts 5-10x normal, unable to form defect in adhesion to endothelial granulomas. Severe gingivitis, intestinal fistuals, cells, most commonly CD18 poor wound healing, delayed separation fo umbilica LAD: flow cytometry analysis of cord CD18 Complement immunity Impaired opsonization Bacterial infections, rheumatologic disease C5-C9 def: Neisseria meningitidis infections C1-C4 def: SLE Allergy Chronic relapsing/remitting reaction to allergens (food/env) 10% of pediatric population genetic predilection 50% develop allergic rhinitis and/or asthma Pruritic, erythematous, weeping papulovesicular reaction --> scaling, hypertrophy, lichenification

Atopic dermatitis

DDx: contact dermatitis, psoriasis <2 yrs: extensor surfaces >2 yrs: flexor surfaces, neck, wrists, ankles

Termination of itch-scratch-itch cycle: lotions, topical corticosteroids, Bacterial superinfection pimecrolimus cream (inhibits T cell activation), topical tacrolimus

19

Immunology, Allergy, Rheum Condition Description

Signs/symptoms

Ddx Ddx: - Infectious rhinitis: more common in infants/toddlers, mucopurulent - Sinusitis: chronic rhinorrhea, postnasal drip w/ facial tenderness, cough, and/or HA - Nasal foreign body: unilateral, thick, foul discharge - Vasomotor rhinitis (idiopathic nonallergic): exaggerated vascular respons to irritant - Rhinitis medicamentosa: overuse of topical decongestants Elevated nasopharyngeal eosinophils, serum radioallergosrobant (RAST) test, direct skin testing

Treatment

Complications

T1 hypersensitivty, IgE seasonal (hay fever): limited to pollination months, uncommon betfore 4-5 yrs Allergic rhinitis Risk factors: atopy, genetic, smoking - heavy exposure to animal dander early in life reduces risk

Nasal congestion, rhinorrhea, postnasal drainage, sneezing, itching Nasal mucosa appears boggy and bluish 1. allergic shiners: dark circles under eyes secondary to venous congestions 2. allergic salute: horizontal crease across middle of nose due to upward wiping motion with hand

Allergen avoidance H1 blocker: PO or intranasal Intranasal cromolyn: preventive Nasal topical steriods PO leukotriene receptor antagonists Topical/inhaled sympathomimetics pseudoephrine

Recurrent sinusitis, otitis media with effusion

Urticaria and angioedema

Hereditary angioedema: C1 esterase deficiency

Urticaria: raised edematous hives on skin/mucous membranes resulting from vascular dilation and increased permeability - itch, blanch, resolve Angioedema: lower dermis and subcutaneous areas, well demarcated area, no pruritis/erythema/warmth

Subcutaneous epinephrine: emergency Also IV diphenhydramine and steroids PO antihistamines, sympathomimetics, PO steriods C1 esterase replacement for hereditary angioedema Avoid offending food - Cow milk, egg, soy, and wheat allergies can be outgrown - peanut, nut, and fish allergies usually persist

Food allergies

Ddx: - Food intolerance: nonimmunologic (caffeine-induced tachycardia, 80% present in 1st year Isolated cutaneous rxns, GI sxs, respiratory sxs, lifelactose intolerance) - peanuts, eggs, milk, soy, wheat, threatening anaphylaxis. tree nuts, fish Double-blind placebo challenge food challenge: gold standard. Rheumatology Oligoarticular JIA: most common. - girls 2-4yrs - large joints (knee, ankle) - joint contractures, muscle atrophy, increased extremity growth in affected limb - 75% have positive ANA test -- associated with chronic nongranulomatous anterior uveitis asymptomatic, detect on slit lamp

Juvenile Idiopathic Arthritis

Chronic arthritis > 6 weeks in individuals <16 yrs synovitis: inflammation and hypertrophy of synovium - TNF alpha mediated

Ddx: posinfectious arthritis (acute rheumatic fever), systemic inflammatory conditions (IBD, connective tissue diseases, HSP), Polyarticular JIA infections (septic arthritis, viral - RF positive: resembles adult rheumatoid arhtritis arthritis, Lyme disease), malignancy - RF neg: adolescents, large and small joints, better (leukemia, neuroblastoma, bone prognosis tumors) Systemic JIA: autoinflammatory disorder - extraarticular, intermittent high fever, salmon colored rash, HSM, LAD, pericarditis. - large and small joints - acute phase rxn: leukocytosis, thrombocytosis, anemia, incr ESR and CRP, incr ferritin. Enthesitis-related arthritis: sacroiliac joints, occurs at tendon insertions (achilles, plantar fascia, ASIS) Psoriatic arthritis: nail pitting, onycholysis, dactylitis due to flexor tendon tenosynovitis
20

Single large joint: intra-articular corticosteroid injection Multiple joints: methotrexate - TNF-alpha, IL-L, IL-6, T-cell costimulation Bony erosions, deformities, growth disturbances

Diagnosis: - acute phase rxn - ANA associated with anterior uveitis - RF only in 5% - Synovial fluid: WBC > 2000/mm3 with mononuclear cells

Immunology, Allergy, Rheum Condition Description

Signs/symptoms

Ddx Anemia, leukopenia, thrombocytopenia, elevated ESR, CRP normal Positive coombs --> hemolytic anemia Decr complemet levels ANA, antiphospholipid abs, anti smith, anti-dsDNA, anti-RNP, antiRo, anti-La Ddx: polymyositis (no skin findings, CD8 lymphocytes infiltrate muscle fascicles and attack muscle fibers directly), less common in children

Treatment

Complications

Systemic lupus erythematosus

Abnormal cell death --> incr exposure to self nuclear components

Painless oral ulcers, malar rash, discoird lupus, photosensitivity. Renal failure in Type IV LN (diffuse proliferative)

Aovid sun hydroxychloroquine for preventing disease flares NSAIDs, corticosteroids

Dermatomyositis

autoimmune disease involving skin/skeletal muscles

Violaceous dermatitis of eyelids (heliotrope), hands, elbows, knees, ankles. Gottron papules: scaly erythematous papules on extensor surfaces of fingers, elbows, knees

elevated serum creatine phosphokinase (released during muscle breakdown), other muscle enzymes. Elevated VWF. Definitive diagnosis: muscle biopsy perivascular inflammatory infiltrate, perifascicular atrophy

Corticosteroid therapy, IVIG, cyclosporine hydroxychloroquine: treats cutaneous manifestations

Spontaneous perforation of bowel is leading cause of death

Henoch Schonlein purpura

IgA immune complexes in vessel walls

Skin, joint, GI, kidney disease. Supportive, Spontaneous resolution Nonthrombocytopenic purpura on lower extremities, within 4 wks, sxs may persist for 12 buttocks. Scroal edema, extremity swelling wks Fever with abrupt onset and termination Familial Mediterranean Fever: MEFV gene, most common. Peritonitis, erysipelas-like rash, oligoarthritis

Periodic Fever Syndromes

TNF receptor-associated Period Fever Syndrome WBC, CRP, ESR elevated during (TRAPS): AD, fevers 7-21d, 2-3x/yr. Abd pain, fever attacks severe muscle aches with overlyting erythema, conjunctivitis, periorbital edema, large joint arthritis Periodic Fever, Apthous stomatitis, pharyngitis, adenitis syndrome (PFAPA): 3 or more episodes of fever, tender cervical LAD, pharyngitis/apthous ulcers, normal WBC, CRP, ESR

21

Endocrine Condition

Description

Signs/symptoms

Ddx

Treatment

Complications

22

Nephrology and Urology Condition Description

Signs/symptoms

Ddx Renal dysplastic and cystic diseases

Treatment

Complications

High Yield facts

Renal Dysplasia: Renal parenchymal tissue does not form correctly throughout, usually bilateral. incr risk of abnormal development elsewhere - inability of kidney to concentrate urine - impaired ability to resorb fluids --> frequency, incontinence, susceptibility to dehydration - often does not present with HTN - Renal US: kidneys appear hyperechoic Failure of one or both kidneys to form - Bilateral --> oligohydramnios --> Potter sequence (clubbed feet, cranial abnormalities) Kidney consists of numerous noncommunicating fluid filled cysts. - Almost always unilateral Most common renal cystic dz of childhood, most common abdominal mass in newborn ARPKD: dilated renal collecting tubules --> small cysts - dilated hepatic bile ducts ADPKD: usually not detected until adulthood Palpable renal mass in infant ARPKD: HTN, decline in renal function ADPKD: HTN, hematuria

Renal agenesis

Multicystic dysplastic kidney

Postnatal US: noncommunicating cysts Renogram: lack of function

Most cases undergo spontaneous involution Nephrectomy if kidney changes in size or appearance, persistent HTN

Polycystic kidney disease

Increased echogenicity on US

ARPKD: dialysis

MCC of hydronephrosis in childhood - Primary: intrinsic narrowing at junction of renal pelvis and ureter Ureteropelvic or angulation of ureter from a junction obstruction crossing renal vessl - Secondary: scarring, angulation secondary to ureteral dilation (stones) Length of tunnel of ureter through bladder submucosa is Vesicoureteral reflux insufficient to prevent retrograde flow of urine - unilateral or bilateral

Newborns: palpable abdominal mass Older children: abd or flank pain, Renal US cyclic vomiting, hematuria + mass

Surgical correction: minimally invasive surgery or open pyeloplasty

VCUG: voiding cystourethrogram detects abnormalities at erteral Frequent UTIs insertion sites and allows Retrograde flow of infected urine classification of grade of reflux -> pyelonephritis - high grade: large tortuous ureters, distortion of renal pelvis and calyces

Antibiotic prophylaxis for UTIs - < 3yrs: amoxicillin - older: bactrim or nitrofurantoin Surgery: lengthen intravesicular segment of tunnel (ureteral reimplantation)

Posterior urethral valves

Obstructing leaflets within posterior urethra --> partial-tocomplete bladder outlet obstruction Distended bladder or renal mass - urethral dilation, bladder neck hypertrophy, bladder trabeculation Older infants: weak/dribbling - males only urinary stream or unexplained daytime wetting One of the most common causes of end-stage renal disease in male child Incomplete development of distal urethra --> malposition of urethral meatus along ventral side of penis toward perineum - chordee: associated curvature of penis

Prenatal US: hydronephrosis and bladder distention Renal US: distended bladder with thickened walls and trabeculation. Bilateral hydronephrosis. VCUG: visualization of posterior urethral valvesx - dilated bladder, hypertrophied neck, dilated posterior urethra with shield-shape (spinnaker-sail)

Ablation of obstructing valve leaflets Abx prophylaxis if VUR is present

Chronic kidney disease Early surgical correction of reflux is requires long discouraged: bladder pathophys term follow up changes over time, early surgery has high failure rate

Hypospadias

Hypospadias + cryptorchidism Circumcision is contraindicatedprompts ambiguous genitalia workup surgical repair may require preputial (genetic karyotyping) tissue

23

Nephrology and Urology Condition Description

Signs/symptoms

Ddx Ddx: - epididymitis (infectious or secondary to epididymal appendix) - appendix testes: "blue dot" sign + normal cremasteric reflex clinical diagnosis

Treatment Surgical emergency - surgery within 6 hrs - remove necrotic testes, fix CL testis to scrotal envelope Testicular or epididymal appendix: torsion resolves spontaneously Epididymitis: abx

Complications

High Yield facts

Testicular torsion

Acute onset unilateral scrotal Lack of posterior attachment to pain, n/v tunica vaginalis --> mobile testis -- Swollen, erythematous scrotum > bell-clapper deformity Absent cremasteric reflex

Hydroceles and varicoceles

Hydroceles: fluid-filled sacs in scrotal cavity consisting of remnants of processus vaginalis - communicate with peritoneal cavity through patent processus, risk for incarceration Varicocele: dilation of testicular veins + enlargement of pampiniform plexus

Varicoceles: detectable in boys during adlescence, more common on left, nontender - evident when pt is standing: veins distend, "bag of worms"

Hydroceles: repair communicating hydroceles and hernias to prevent incarcerated hernia Varicoceles: surgical repair - unrepaired varicoceles incr risk for infertility

UTI DDx: - Adenovirus: self-limited hemorrhagic cystitis that does not respond to abx - Posterior urethralgia: benign, selflimiting inflammation of posterior Infants: fever may be only sign urethra in boys - hematogenous seeding of - Lower lobe PNA in febrile child: kidney f/chills/flank pain - Urolithiasis: dysuria, hematuria, Older children: same as in adults flank pain - upper tract involvement: incr WBC, ESR, CRP All children <24 months must undergo renal US to r/o hydronephrosis or structural lesions. VCUG if hydronephrosis or nonresponders to abx. DMSA if suspected pyelonephrosis

Pts with positive leukocyte esterase should be treated for presumed UTI until culture results are available

UTI

First year of life: girls = boys After first year: girls have 10x incidence

Cystitis: amoxicillin, ampicillin, nitrofurantoin, or TMP-SMX Pyelonephritis: PO cephalosporin or IV ampicillin + gentamicin

Pyelonephritis: perinephric abscess, renal scarring, renal failure

Bagged specimens are inadequate for UTI evaluation

Marked proteinuria: > 1000 mg/m2/day, spot urinary protein:creatinine ratio > 2.0 - results in hypoalbuminemia (<2.5 g/dL) Proteinuria, hypoalbuminemia, hyperlipidemia, edema - Primary: MCD (most common), FSGS, MPGN, MN Nephrotic syndrome - Secondary: Infections, systemic dz (SLE, HSP, IgA), drugs (NSAIDs, heroin), malignancies, genetic Nonspecific illness a few weeks prior Periorbital edema: first abnormality noted Dependent edema, weight gain FSGS, secondary etiologies, glomerulonephritis: gross hematuria, HTN Hyperlipidemia (lipases are lost in urine) Microscopic hematuria, mild hyponatremia (fluid overload), hypocalcemia (albumin loss), incr creatinine (renal hypoperfusion) MCD: effacement of foot processes FSGS: mesangial hypertrophy, tubular atrophy Diffuse MPGN: incr mesagnial cellularity, glomerular BM thickening MN: diffuse thickening of capillary walls Dietary salt restriction, oral steroid therapy - stronger immunosuppressants in nonresponders (cyclophosphamide, calcineurin inhibitors) IV albumin: induces temporary diuresis

Spontaneous bacterial peritonitis - incr risk of encapsulated organisms due to loss of proteins involved in phagocytosis of capsule Thromboembolic events, persistent hyperlipidemia, steroid toxicities

24

Ophthalmology Condition

Description 4% of children Esotropia: inward Exotropia: outward Associated with cerebral palsy, down syndrome, hydrocephalus, brain tumors Reduced vision in otherwise normal eye - strabismic amblyopia: suppression of retinal images in misaligned eye - anisometropic amblyopia: unequal refractive errors in two eyes

Signs/symptoms

Ddx

Treatment

Complications

Strabismus

Amblyopia found in most pts with esotropia

Corneal light reflex, cover test

Corrective lenses, occlusion, atropine penalization, surgery

Amblyopia, reduced stereopsis

Correct refractive errors Occlusion of better eye Subnormal vision Treatment is unsuccessful beyond 8 yrs

Amblyopia

Permanent vision loss, diminished stereopsis

Leukocoria

White pupil/absence of red reflex

Retinoblastoma, cataracts (most common), retinopathy of prematurity, congenital glaucoma, ocular toxocariasis

Enucleation, chemo, radiation, cryo Cataract surgery ROP: laser ablation of retina or cryo reduces progression to retinal detachment/scarring 96% resolve spontaneously in 1 yr Probing of nasolacrimal duct system at 12-15 mo.

Amblyopia ROP: Retinal detachment, scarring

Congenital nasolacrimal duct obstruction (dacryostenosis)

Failure of distal membranous end of nasolacrimal duct to open Chronic tearing in absence of - Common cause of overflow conjunctival injection tearing - 25% of neonates

Infectious conjunctivitis

Inflammation in conjunctiva Viral: adenovirus

H. influenza: same-sided otitis media

Antibiotic drops: polymyxinbacitracin, erythromycin - abx limit infectivity and decrease Corneal abrasion: Painful, tearing, duration by 2 days photosensitivity. Examination under - Neisseria: IV ceftriaxone blue light with fluorescein reveals - H. influenza: amoxicillin-clavulanic abrasion. Treat with eye patching. acid Heals in 24h - HSV1: do not give steroidcontaining abx. Risk of more severe disease and visual impairment

Hordeolum: acute infection of glands around eyelas follicle - S. aureus Styes

Hordeolum: localized tender swelling, then rupture

Hordeolum: warm compresses, abx Chalazion: excision may be required

Chalazion: sterile Chalazion: firm, nontender area lipogranulomatous reaction within glands in tarsal plate Bacterial infection of eyelids/skin anterior to orbital septum - breaks in skin: s. aureus, group A strep - hematogenous: s. pneumo, h. influenza - sinuses/resp: s. pneumo, h. influenza, moraxella Orbital cellulitis: severe pain with eye movement, proptosis, vision changes, decr ocular mobility. Confirm with CT. Trauma, edema, allergies, tumor
25

Periorbital cellulitis

Skin around eye is indurated, warm, tender

IV abx Breaks in skin: penicillin or 1st gen cephalosporin Cefuroxime 3rd gen ceph: prevent extension to meninges