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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
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
IV acyclovir.
Local disease: recurrent mucocutaneous lesions Encephalitis: cataracts/bindness, microcephaly, DD/learning disabilities Disseminated: severe neuro impairment, death None
Parvovirus
Intrauterine blood transfusions, supportive Prevention: zidovudine to mother during pregnancy, zidovudine to infant for first 6 wks of life.
HIV
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
Supportive - Antipyretics, daily bathing Clinical diagnosis - Tzanck smear - DFA, viral culture, PCR
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
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
Description
Signs/symptoms
Ddx
Treatment
Complications
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
papules --> vesicles --> 5mm pustules --> rupture --> honey colored crust over ulcerated base Organism can be isolated from lesions
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
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
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
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
Pulmonology Condition
Definition
Epidemiology
Etiology
Signs/symptoms
Treatment
Complications
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.
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
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
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
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
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
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
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
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
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
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
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
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
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
Herpangina
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
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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
Croup Acute laryngotracheo Most pronounced in young bronchitis children due to narrow caliber of subglottic region. Peaks in late fall/winter
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
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Condition
Description
Etiology
Ddx
Treatment
Complications
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
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: 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
Description
Etiology HSV 2 or 1
Signs/symptoms
Ddx
Treatment
Complications
Usually polymicrobial
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
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
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
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
Tick bite (wood tick, dog tick, lone star tick) April-Sept
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
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 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
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
Failure of hematoietic stem cells --> pancytopenia - due to chemicals (benzene), drugs (chloramphenicol, sulfonamides), infectious agents (hepatitis), ionizing radiation. Usually idiopathic.
Immune thrombocytopenia
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
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
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
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
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
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
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
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
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
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
Painless oral ulcers, malar rash, discoird lupus, photosensitivity. Renal failure in Type IV LN (diffuse proliferative)
Dermatomyositis
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
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
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
Signs/symptoms
Treatment
Complications
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
Most cases undergo spontaneous involution Nephrectomy if kidney changes in size or appearance, persistent HTN
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
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)
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)
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
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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
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: 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
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
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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
Correct refractive errors Occlusion of better eye Subnormal vision Treatment is unsuccessful beyond 8 yrs
Amblyopia
Leukocoria
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.
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
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
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
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Periorbital cellulitis
IV abx Breaks in skin: penicillin or 1st gen cephalosporin Cefuroxime 3rd gen ceph: prevent extension to meninges