Pediatrics Notes

Neonatal Condition

Description

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

Signs/symptoms

Ddx

Treatment

Preterm birth, IUGR, microcephaly, poor feeding, lethargy, petechiae/purpura, blueberry muffin spots, jaundice, HSM

Complications

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

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

First 4 wks: 1) isolated mucocutaneous lesions, keratoconjunctivitis 2) encephalitis 3) disseminated disease involving mult organs

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

Parvovirus

Hydrops fetalis

Maternal IgM, IgG levels

Intrauterine blood transfusions, supportive

None

HIV

Mostly asymptomatic LAD/HSM, FTT, DD, encephalopathy, bacterial infections, opportunistic infection, lyphoid interstitial pneumonitis

HIV culture, PCR

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

CMV

Elevated liver enzymes, anemia, thrombocytopenia intracranial (esp periventricular) calcifications, seizures, chorioretinitis

HSV

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

VZV

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

Congenital: IUGR, "zigzag" scarring, limb atrophy, ocular abnormalities Acquired: widespread cutaneous lesions, PNA, hepatitis, death

Rubella

Transmission at any time during pregnancy

Congenital rubella syndrome: cataracts, sensorineural hearing loss, congenital cardiac defects, DD

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),

Maternal antibody titers during pregnancy After birth: elevated rubella IgM or IgG

Penicillin G

Toxoplasmosis

1

Neurosyphilis, deafness

Dermatology Condition

Description

Hand-foot-mouth

Coxsackie A

Giannoti-Crosti Syndrome (papular acrodermatitis)

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

Primary infection with VZV Varicella

Herpes zoster

fatal disseminated disease in immunocompromised

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

Order hep B serologies

Clinical diagnosis - Tzanck smear - DFA, viral culture, PCR

Pain/pruritus Vesicular eruption in crops, confined to dermatome - clears up in 7-14d Uncommon in children 12 yrs, children with chronic disease, and those who have received systemic steroids

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

Presumed viral exanthems

Pityriasis Rosea

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

2

Complications

Supportive - Antipyretics, daily bathing

Reactivation of VZV from dorsal root ganglia

Poxvirus Molluscum contagiosum

Signs/symptoms

Self limited Antihistamines, topical steroids for pruritis sunlight

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

Bullous impetigo

Nonbullous impetigo

Description

Signs/symptoms

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

S. aureus toxin

group A beta hemolytic strep, s. aureus

Ddx

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

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

Treatment

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

Limited: Muciprocin ointment Numerous: cephalexin (1st gen) - covers staph and strep Suspect MRSA: clindamycin or TMPSMX Remove honey colored crusts with warm compress

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

Unruptured bullae contain sterile fluid

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

3

antiseptic cleansers, topical muciprocin

Complications

Dermatology Condition

Description

Signs/symptoms

Ddx

Treatment

Complications

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

Psoriatic arthritis

Silvery scales, tends to be on extensor surfaces, symmetric, Auspitz sign

Psoriasis

Family history, HLA inheritance

Eczema: pruritic, flexural creases

Koebner phenomenon: psoriasis appears at sites of physical/thermal/ mechanical trauma Nail pitting, onycholysis (detachment of nail plate), accumulation of subungual debris.

Scalp lesions may look like seborrheic dermatitis or tinea capitis

Group A strep: common exacerbation Eruptions secondary to allergic reactions

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

10% mortality if untreated Stevens Johnson syndrome

Toxic epidermal necrolysis

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

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

Hospitalization, fluid and electrolyte support, moist compresses, oatmeal baths Oral mucosal: mouthwashes with lidocain, diphenhydramine, Maalox Ophthalmology consult

Positive Nikolsky sign

Fluid therapy, treat like a burn

Elevated liver enzymes, renail failure, fluid and electrolyte imbalance

IVIG: may affect binding/effect of Fas ligand

4

Pulmonology Condition

Upper airway obstructive disease

Obstructive sleep apnea

Definition

Epidemiology

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

Etiology

Signs/symptoms

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

Noisy inspiration, increased work of breathing (nasal flaring, accessory muscles), retractions (suprasternal)

Older child: large adenoids and tonsils laryngotracheitis, peritonsillar or retropharyngeal abscess

Pickwickian syndrome: OSA associated with obesity

Ddx Obstructive lung diseases

Obstruction of subglottic space: high pitched, monophonic stridor

Asthma

most common cause of hospitalization >50% present before age 6 Risk factors: genetic, atopy, cigarette exposure, urban impoverished areas, rhinovirus/RSV URIs

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

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

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 tonsils/adenoids, CPAP

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

Remove inciting agents from patient's environment, maintence antiinflammatory medication Acute exacerbation: inhaled bronchodilator Persistent asthma: Inhaled corticosteroids - symptom control, avoidance of exacerbations

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

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

Nasal polyps in any pediatric pt should prompt further testing for CF.

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

High Yield facts

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

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

Prolonged respiratory infections, Methacholine challenge test decr exerise tolerance, persistent CXR: hyperinflation, increased bronchial markings day/nighttime coughing

1) Reversible airway obstruction 2) inflammation 3) bronchial hyperresponsivenes s

Complications

Obstruction above glottis: more variable, fluttering stridor

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

15-20% children in US 25% in blacks and hispanics

Treatment

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.

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

failure to thrive is most common manifestation of untreated CF. Meconium ileus is pathognomonic

5

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

Primary ciliary dyskinesia

Impaired mucociliary clearance

Epidemiology

Etiology

DNA11 and DNAH5 mutations

Signs/symptoms

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

Congenital tracheal stenosis

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

Tracheomalacia

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

Bronchomalacia

Poor cartilage or poor elastic recoil in surrounding tissues

Ddx

Treatment

Complications

High Yield facts

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

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

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

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

6

Cardiology Condition

Truncus arteriosus

Description

Cardiac exam Cyanotic Congenital Heart Disease: Ductal-independent lesions

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

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%)

Total anomalous pulmonary venous connection

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%)

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%)

Treatment

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

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

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

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

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

Tricuspid atresia

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

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

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

7

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

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

Ebstein anomaly

Description

Cardiac exam

Treatment

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

PGE1 Avoid surgery Heart transplant if severe

Cyanotic Congenital Heart Disease: Ductal-dependent systemic blood flow

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

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

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

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

PGE1 Connect interrupted aortic segments

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

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

8

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.

Patent ductus arteriosus

Premies Symptoms related to size of defect and direction of flow

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

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

CHF: ace inhibis and diuretics Repair large VSDs within 6 months to decrease risk of pulmonary artery HTN and pulmonary vascular obstructive disease

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

Reversal of flow (R-->L): cyanosis Delayed/weak femoral pulses, upper extremity HTN

Coarctation of the Turner syndrome aorta

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

Aortic stenosis

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

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

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

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

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

9

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

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. aureus - Other: Salmonella, pseudomonas, viridans strep - Evaluation: Blood, urine, CSF cultures, CXR if respiratory signs present

Condition

Ddx

Acute otitis media

Description

Etiology

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

Signs/symptoms

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

Treatment

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

HA, facial pain, sinus tenderness Same pathogens as Acute bacterial: 1) persistent AOM respiratory sxs (10-14d) 2) severe sxs (high fever, purulent nasal discharge)

Sinusitis

Herpangina

Streptococcal pharyngitis

Diagnosed during summer and fall in young children

Enteroviruses: coxsackievirus, etc

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

High fever, very sore throat Vesicular lesions progressing to ulcers - soft palate, tonsils, pharynx

Primary herpetic gingivostomatitis:lesions are more widespread over gums, lips, mucosa Hand foot mouth dz: also lesions - HSV on palms and soles

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

EBV

Severe exudative pharyngitis, fever, profound fatigue

S. pneumo: 50% penicillin resistant. H. influenza and M. catarrhalis: beta- lactamase activity

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

Leukocytosis or leukopenia, 50% lymphocyte, at least 10% atypical lymphocytes Heterophile antibody: limited sensitivity in pts 48h

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

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

Urethral discharge, itching, dysuria, frequency.

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

Generalized LAD, hepatomegaly, splenomegaly, failure to thrive, diarrhea, cadidiasis, parotitis, and ELISA and Western blot not useful developmental delay in children 90% in utero or perinatal Risk of transmission is 25% if untreated

Rocky Mountain Spotted Fever

Ddx

Trichomonas: most asymptomatic, malodorous, frothy gray discharge and vaginal Trichomonas: PMNs and discomfort. Penile discharge trichomonads on wet prep

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

HIV and AIDS

Signs/symptoms

Complications

HSV 2 or 1

Resp: lymphoid interstitial PNA, PCP PNA

Rickettsia rickettsii

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

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

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

No reliable diagnostic test. Abs confirm diagnosis 10d after sxs

Empiric rx - need to cover erlichiosis and N. meningitidis

Thrombocytopenia and hyponatremia

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

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

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

High Yield facts

Heme Condition

Description

Signs/symptoms

Ddx Microcytic anemias

Treatment

Complications

Occurs as early as 3 mo in premie.

Iron deficiency anemia

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 ( excess of one type --> unstable monomers that precipitate and damages membrane

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

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

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 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.

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

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

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

AR or X linked, pancytopenia Defect in DNA repair

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

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

Anemia, thrombocytopenia, neutropenia

Macrocytosis Elevated HbF 10% develop leukemia Confirm diagnosis by demonstrating increased chromosomal breakage with exposure to diepoxybutane (DEB) CBC: cytopenia, microcytosis Diagnosis: peripheral pancytopenia + hypocellular bone marrow

PO corticosteroids BM transplant

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

BM transplant Immunosuppression if no donor can be found

Disorders of hemostasis

Immune thrombocytopenia

Hemophilia A and B

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

Hemophilia A: factor VIII Hemophilia B: factor IX

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)

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

DDAVP: releases factor VIII from endothelial cells 14

Aminocaproic acid: inhibits fibrinolysis

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

Onc Condition

Description

Signs/symptoms

Ddx Leukemia

Treatment

Complications

Neutropenia (ANC < 500/mm3) predisposes to serious bacterial and fungal infections.

Most common pediatric cancer (75%)

ALL

Induction therapy: 28d of vincristine, steroids, intrathecal MTX, asparaginase. Add daunomycin for high risk pts.

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

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 50,000 at Fever, mediastinal mass (mostly T diagnosis, failure to respond to cell), induction therapy, Philadelphia chromosome

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 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

20% childhood leukemias Best prognosis: M3 AML (APML) and trisomy 21 pts with M7 AML AML

Worse prognosis: WBC >100,000/mm3

Chemotherapy more intense than ALL

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

Induction: anthracycline + arabinoside Low risk: chemo only High risk: BM transplant

Low risk: inv 16, t16;16, t8;21 High risk: monosome 5 or 7, or no remission

Non-Hodgkin lymphoma

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

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

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

15

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.

Onc Condition

Description

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%)

Signs/symptoms

Ddx

Treatment

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

Neuroblastoma

Childhood embryonal malignancy of postganglionic sympathetic nervous system. 8% of all childhood cancers 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

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

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

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

Onc Condition

Description

Signs/symptoms

Ddx

Treatment

Complications

Associated anomalies: sporadic aniridia, hemihypertrophy, cryptoorchidism, hypospadias, other GU anomalies

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

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

Other findings: hematuria, HTN, varicocele Von willebrand's disease in 8% Bone tumors

Ewing sarcoma

Osteosarcoma

Retinoblastoma

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

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

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

Ddx: osteomyelitis, eosinophilic granuloma (langerhans cell histiocytosis), osteosarcoma, neuroblastoma or rhabdomyosarcoma metastasis to bone

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

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)

lytic bone lesion with periosteal reaction (sunburst appearance)

20% have mets: lung most common Gait disturbance, pathologic fractures

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

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

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

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

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

Onc Condition

Description

Most common STS in children