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Flashcards in Pediatrics Deck (108)
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1
Q

Weight trends

A

infant loses up to 10% birth weight, regained by 2 weeks

birth weight doubled by 4 mo, tripled by 12 mo, quadrupled by 24 mo

Age 2-13 annual wt gain 5 pounds

Inadequate wt gain:
poor intact - feeding techniques, neglect
Vomiting - pyloric stenosis, reflux
Malabsorption
neoplasma
infection
congenital diseases - cardiac or endocrine

Failure to thrive - less than 2nd percentile or consistently low weight

2
Q

Height trends

A

increases 50% at 1 yr, doubles by 4 y, triples by 13
Annual increase of 2 in from age 2-adolescence

Greater than normal height:
familial tall stature
precocious puberty
gigantism - excessive GH
hyperthyroidism
Klinefelter syndrome, Marfan syndrome
obesity
Lower than normal height:
familial short stature
constitutional growth delay
neglect
Turner syndrome
chronic renal failure
asthma, CF
IBD
immunologic disease
GH deficiency
hypothyroidism
glucocorticoid excess
skeletal dysplasias
neoplasm
3
Q

causes of macrocephaly

A

cerebral metabolic disease - Tay-Sachs, maple syrup urine disease
neurocutaneous syndromes - neurofibromatosis, tuberous sclerosis
hydrocephalus
increased ICP
Skeletal dysplasia
acromegaly
Intracranial hemorrhage

4
Q

causes of microcephaly

A
fetal toxin exposure - fetal alcohol syndrome
chromosomal trisomies
congenital infection
cranial anatomic abnormalities
metabolic disorders
neural tube defects
5
Q

Car seats

A

under 2 - rear facing, middle back seat

2-5 - forward facing, harness as long as possible

5-12 - booster until seat belt fits properly - 4’9” between 8-12

Younger than 13 - back seat

6
Q

APGAR score

A

Appearance: 0 blue, 1 acrocyanosis, 2 pink all over
Pulse: 0 absent, 1 less than 100, 2 over 100
Grimace: 0 absent, 1 grimace, 2 strong cry
Activity: 0 absent, 1 some movement, 2, vigorous movement
Respiration: 0 absent, 1 irregular, 2 regular

normal 7-10
abnormal - resuscitate

7
Q

Calorie content per ounce of breastmilk vs formula

A

20 kcal/oz each

8
Q

Caloric needs in infant less than 6 mo

A

100-120 kcal/kg/day

9
Q

Single umbilical artery

A

get renal u/s - check for abnomalies

10
Q

Anterior fontanelle closure

A

normal by 24 mo

Delayed consider: downs, achondroplasia, rickets, congenital hypothyroidism, increased ICP

Early - monitor head circumference for craniosynostosis

11
Q

Craniotabes

A

soft occipital bone - like ping-pong ball
3-12 mo
highly suspicious for rickets

12
Q

Signs of dehydration in child - mild

Skin turgor
mucous membranes
Tears
Fontanelle
CNS
HR
Capillary refill
Urine output
A

Infant - 5%; 50 ml/kg

Skin turgor - normal
mucous membranes - moist
Tears - present
Fontanelle - flat
CNS - consolable
HR - normal
Capillary refill - less than 2 sec
Urine output - normal
13
Q

Signs of dehydration in child - moderate

Skin turgor
mucous membranes
Tears
Fontanelle
CNS
HR
Capillary refill
Urine output
A

infant - 10%, 100 ml/kg

Skin turgor - tenting
mucous membranes - dry
Tears - reduced
Fontanelle - soft
CNS - irritable
HR - mild increase
Capillary refill - about 2 sec
Urine output - decreased
14
Q

Signs of dehydration in child - severe

Skin turgor
mucous membranes
Tears
Fontanelle
CNS
HR
Capillary refill
Urine output
A

Infant - 15%, 150 ml/kg

Skin turgor - none
mucous membranes - parched/cracked
Tears - none
Fontanelle - sunken
CNS - lethargic
HR - increased
Capillary refill - >3 sec
Urine output - anuric
15
Q

Failure to thrive in under 2 yo

A

2nd percentile in weight or less on more than 1 measurement
weight less than 80% of ideal weight for sex and age
Weight crosses 2 major percentiles downward on growth chart over time
weight for length ratio less than 10th percentile
Rate of daily weight gain less than expected for age

16
Q

tanner stages for penile/testicular development

A

(pubic hair in gyn lecture)

1- prepubertal
2 - testicular and scrotal growth with skin coarsening
3- penile enlargement and further testicular growth
4- further growth glans, and darkening of scrotal skin
5 -adult genitalia

17
Q

Early adolescence (10-13) psychosocial development

A

concrete thinking and early independent behavior

18
Q

Middle adolescence (14-16) psychosocial development

A

emergency of sexuality - identity, beginnings of sexual activity
increased desire for independence (conflict with parents, need for guidance, self absorption)
development of abstract thought

19
Q

Late adolescence (17-21) psychosocial development

A

increased self-awareness, increased confidence in own abilities, more open relationship with parents, and cognitive maturity

20
Q

Klinefelter syndrome

A
47, XXY
Tall thin body
Gynecomastia
Testicular atrophy
Infertility
Mild retardation
Psychosocial adjustment abilities
21
Q

Turner syndrome

A
45, XO
Web neck
Low occipital hairline
Short stature
Infertile
Streak ovaries
Coarctation of aorta
Horseshoe kidney

Most end in spontaneous abortion
Can have mosaicism (45, XO/46, XX)
Most common cause of primary amenorrhea

Tx:
Estrogen and progesterone
Growth hormone
Cardiac monitoring

22
Q

Down syndrome

A

Trisomy 21
First trimester ultrasound: increased nuchal translucency
Quad screening: low AFP, high b-hCG, low Estriol, high inhibin A

Features:
Intellectual disability
Small ears, Flatnose, protruding tongue
Transverse Palmar crease - simian crease
Cervical spine instability
Increase space between first and second toes
Vision and hearing loss
duodenal atresia, Hirschsprung disease, annular pancreas, celiac disease
Alzheimer’s disease 3rd decade
Endocardial cushion defect, Tetralogy of Fallot, PDA
Associated with ALL “ALL fall DOWN”

23
Q

Edward syndrome

A

Trisomy 18
Quad screening: low AFB, low b-hCG, low Estriol

Features:
Severe intellectual disability
hypertonia
Micrognathia - small mouth, small jaw
Overlapping flex fingers, rocker bottom feet
PDA, VSD
omphalocele, Meckel diverticulum

Usually miscarry in first trimester
Die within 2 weeks of life, possible to survive 1st year

24
Q

Williams syndrome

A

Elfen type face - upward turn nose, Long philtrum
Mild retardation
Cheerful
Cardiac defects

25
Q

Velocardiofacial Syndrome

A

del 22q11

DiGeorge Sn - T cell deficiency, hypocalcemia
Cleft palate
Truncus arteriosus, Tetralogy of Fallot
Mild retardation
Overbite
Speech disorder
26
Q

Patau syndrome

A

Trisomy 13

Severe intellectual disability
Cleft lip and palate
Rounded nose
Polydactyly
Cardiac and CNS defects
27
Q

Angelman syndrome

A

mom Ch15

MR
Seizures
Ataxia
Inappropriate laughter
"happy puppet"
28
Q

Prater Willi syndrome

A

dad Ch15

Hyperphagia -> obesity
Short
MR
Tantrums, skin picking, OCD
Hypogonadotropic hypogonadism
Incomplete sexual development
Osteoporosis - Child
Delayed menarche
29
Q

Cri-du-chat syndrome

A

Small head
Low birth weight
MR
FTT

High-pitched cat like cry

30
Q

Fragile X syndrome

A

Trinucleotide repeat
MC cause of MR in men

MR
Hyperactivity
Seizures
Large faced with prominent jaw
Large ears
Mild hand and foot abnormalities
macroochidism
31
Q

Tuberous sclerosis

A

Distinctive brown, fibrous plaque on the forehead in infancy
Ashleaf spots - wood’s lamp
Shagreen patch - leathery cutaneous thickening usually on the lower trunk
Facial angiofibromas - adenoma sebaceum
seizures
MR
Subependymal nodules in brain - distinguishes from neurofibromatosis

32
Q

Fetal alcohol syndrome

A

short palpebral fissures
thin upper lip
Smooth philtrum
Flattened midface

Structural brain abnormalities
10th percentile or less for head circumference
Abnormal neurologic exam
Variable MR

10 percentile or less for height and weight
FTT despite adequate intake
Disproportionately low weight to height ratio

33
Q

Branchial cleft cyst

A

Lateral neck

Does not move with swallowing

34
Q

Thyroglossal duct cyst

A

Midline neck
Moves with swallowing
Most common neck cyst
Associated with ectopic thyroid tissue

35
Q

Blood shunting in fetus

A

Ductus venosis
Foraman ovale
ductus arteriosis

36
Q

path of highest O2 blood in fetus

A

umbilical v -> ductus venosis -> IVC -> right atrium

37
Q

Patent ductus arteriosus

A
Risk factors:
F>M
Prematurity
Congenital rebel infection
Trisomy 21

Features:
May lead to heart failure ( tachypnea, respiratory distress, Poor feeding)
Continuous machinery like murmur
Wide pulse pressure and bounding pulses

Dx:
CXR: cardiomegaly
ECHO: shows defect, enlarged LA and LV

Tx:
Indomethacin
Surgical ligation

38
Q

Atrial septal defect

A

left to right shunt

Risk factors:
Trisomy 21

Presentation:
\+/- heart failure - SOB, FTT, cyanosis
Eisenmenger syndrome -> cyanosis
Fixed splitting of S2
\+/- midsystolic pulmonary flow murmur

Dx:
ECHO - shows defect, enlarged RA and RB

Tx:
Small: monitor for sxs
Large or HF/cyanosis - surgical repair

39
Q

Ventricular septal defect

A

Left to right shunt

Risk factors:
Trisomy 21

presentation:
+/- heart failure - tachypnea, poor feeding, failure to thrive
Eisenmenger Sn -> cyanosis
Holosystolic murmur along LLSB

Dx: ECHO shows defect

Tx:
Diuretic (furosemide) +/- inotropic drug (dopamine)
large defect - repair

40
Q

Eisenmenger sequence of events

A

left to right shunt
Pulmonary hypertension
RV hypertrophy
Right heart pressure exceeds left heart pressure -> reverse workflow across the defect
Deoxygenated blood enters the systemic circulation
-> cyanosis, dyspnea, digital clubbing

41
Q

Coarctation of the aorta

A

Usually near the ductus arteriosus

Risk: Turner syndrome

Presentation:
high BP in the arms and low BP In the legs
Neonates may develop heart failure and shock when the ductus arteriosus closes

Dx:
CXR - notching of ribs
ECHO shows defect

Tx:
Prostaglandin E keeps the ductus open - avoid heart failure and cardiogenic shock
Surgical correction

42
Q

Truncus arteriosus

A

Failure of truncus arteriosus to divide into the aorta and pulmonary trunk
-associated with a VSD

Features:
Neonatal cyanosis (immediately), tachypnea, respiratory distress
Poor feeding, FTT
Harsh systolic murmur at the LLSB (due to VSD)

DX: ECHO

Tx: repair

43
Q

Transposition of the great vessels

A

RV empties into the aorta and the LV empties into the pulmonary trunk
-incompatible with life without ASD or VSD

Features:
Neonatal cyanosis and tachypnea
Murmur if VSD

ECHO

Tx:
Prostaglandin E - keeps ductus arteriosus patent
Balloon atrial septoplasty
Surgical repair

44
Q

Tricuspid atresia

A

Tricuspid valve does not develop

  • RV often hypoplastic
  • incompatible without ASD and VSD

ECHO

Tx:
Prostaglandin E
Surgical repair

45
Q

Ebstein anomaly

A

Tricuspid leaflets displaced into RV
Hypoplastic RV
Tricuspid valvular regurgitation or stenosis
PFO with right to left shunt

Associated with maternal lithium use

Features:
Sinuses and heart failure
Increased risk of SVT and WPW
Widely split S2
murmur -  tricuspid regurgitation (holosystolic, LLSB)

ECHO

Tx:
Prostaglandin E
Diuretic +/- digoxin
Radiofrequency ablation for arrhythmias
Surgical repair or replacement of tricuspid valve
46
Q

Tetralogy of Fallot

A
MC
'IHOP'
Interventricular septal defect - VSD
Hypertrophy of RV
Overriding aorta
Pulmonic stenosis

Risk factors:
Trisomy 21
DiGeorge Sn

Features:
Episodes of cyanosis, dyspnea, and fatigue - ‘tet spells’
-relieved by squatting
Pulmonic stenosis murmur - systolic at LUSB

CXR: RVH - Boot shaped heart
ECHO

Tx:
Acute cyanotic spells - knees to chest, O2, morphine, propranolol
Neonates: prostaglandin E
Surgical repair

47
Q

Total anomalous pulmonary venous return

A

Pulmonary veins empty into the systemic venous circulation - SVC, coronary sinus, RA

Features:
cyanosis and tachypnea

ECHO

Tx:
Prostaglandin E
Repair

48
Q

Tracheoesophageal fistula

A

Blind upper esophageal pouch
Lower esophagus attached to trachea

Features:
Coughing and cyanosis during feedings
Abdominal distention
Aspiration pneumonia

CXR with NG tube
Tx: surgery

49
Q

Pyloric stenosis

A

Hypertrophy of the pyloric sphincter causes obstruction of the gastric outlet

Presentation:
Non-bilious projectile vomiting
Palpable epigastric olive sized mass

Lab: hyperchloremic, hypokalemic metabolic alkalosis

Rad: barium show string sign
US Study of choice

Tx: pyloromyotomy

50
Q

Necrotizing enterocolitis

A

Risk factors:
Preterm
Low birth weight

Presentation:
Bilious vomiting
Lethargy
Poor feeding
Diarrhea
hematochezia
Abdominal distention
Abdominal tenderness
Shock
Metabolic acidosis
Hyponatremia

Rad: bowel distention, pneumatosis intestinalis, free air

Tx:
TPN
IV broad spectrum abx
NG suction - decompress bowel
Resect affected bowel
51
Q

Hirschsprung disease

A
Features:
Vomiting
Obstipation
Failure to pass meconium/stool
Abdominal distention
FTT
"blast sign" - DRE -> bowel movement

Bx: absence of ganglia
XR: dilated bowel
Barium enema: proximal distention with distal narrowing

Tx: colostomy and resection of effected area

52
Q

Intussusception

A

MC cause of bowel obstruction the first 2 years of life
Telescoping of bowel into itself

Risk factors:
Meckel diverticulum
Henoch-Schonlein purpura
Adenovirus - inflamed peyers patches
CF
Presentation:
Sudden abdominal pain (less than 1 min)
pallor
Sweating
Vomiting
"current jelly stool" - bloody mucus in stool

CT: target

Tx: barium enema, surgery
Complication: bowel ischemia

53
Q

Meckel diverticulum

A

Remnant of vitelline duct

2's:
2 inches long
2 ft from ileocecal valve
2% of population
first 2 years of life
2 types of epithelium - pancreatic, gastric -> painless lower GI bleed

Scintigraphy - ectopic gastric mucosa

Risk factor for intussusception

54
Q

Respiratory distress syndrome in newborn

A

surfactant deficiency - type II pneumocytes immature
a lecithin/shpingomyeline ratio greater than 2 = maturity

S/S:
Tachypnea - RR >60
Nasal flaring
Expiratory grunting
intercostal retractions
hypoxia

Lung exam: crackles, areas of decreased breath sounds

Give IM corticosteroids for labor prior to 34 weeks

CPAP for all babies

If weak respiratory drive or FiO2>0.4 then intubate and give exogenous surfactant via ET tube

55
Q

CXR in respiratory distress of newborn

A

low long volumes

Diffuse groundglass appearance with air bronchograms

56
Q

CXR in transient tachypnea of the newborn

A

Increased lung volumes with flattening of the diaphragm
Prominent vascular markings from the hilum - sunburst pattern
Fluid streaking in interlobular fissures +/- pleural effusions

57
Q

Meconium aspriation syndrome

A

Risk: postterm infants and in fetal distress during labor (intestinal contractions in neonate)

Presentation:
Meconium staining of skin
Respiratory distress

CXR: streaky linear densities, hyper inflated, Flat diaphragms

No intrapartum suction
No suction on vigorous infant
Suction on non-vigorous infants - depressed respiration, decrease tone, HR less than 100
O2 as needed
Intubation for respiratory distress
Enteric antibiotics for pneumonia or sepsis

Short-term complications:
PTX, pulmonary hypertension
Reactive airway disease, asthma

58
Q

Cystic fibrosis

A

CFTR mutation
Sweat chloride >60 on 2 occasions

Features:
meconium Ileus at first
FTT
Pancreatic insufficiency
Steatorrhea
Chronic productive cough
Hyper inflated lungs
PFTs - obstructive lung disease
Recurrent infections: S. aureus, nontype H flu, pseudomonas
Chronic rhinosinusitis

Tx:
CFTR modulators -ivacaftor, lumacaftor - improve function of CFTR protein
B2- agonist - albuterol, salmeterol, formoterol
Deoxyribonuclease (DNase, dornase alfa) - decrease sputum viscosity
Hypertonic saline for chronic cough
Physiotherapy - aerobic exercise - increased mucus clearance - percussion vests
Azithromycin liberally when lung function decreases - slow decline in lung function, treat any pseudomonas
Fluoroquinolones used in children with CF

Pancreatic enzyme supplements
ADEK supplements

59
Q

FB aspiration

A

6mo-4 y
Stridor
Coins usually esophagus

Right main stem bronchus - more vertical

60
Q

Croup

A

parainfluenza virus 1/2
-less common: RSV, influenza, adenovirus, Mycoplasma

3m-3 yr, fall or early winter

Presentation:
Nasal congestion
\+/- fever
Barky seal like cough
Inspiratory stridor
"steeple sign" on CXR

Tx:
Cool mist humidifier, steam from hot shower, cold night air, popsicle
Mild: dexamethasone IM/PO x1
Moderate to severe: keep patient calm, dexamethasone Im/PO x1, nebulized (racemic) epi, humidified oxygen
Severe with respiratory compromise - intubate

61
Q

Epiglottitis

A

HIB, Staph, strep, nontypable H flu
2-7 yo

Acute onset high fever, poor oral intake, Drooling, severe sore throat, dysphasia, toxic appearance, respiratory distress, stridor, hypoxia

“hot potato voice”
“tripoding”

Lateral neck XR: thumb sign

CBC: leukocytosis with bandemia

Tx:
Minimize stress/anxiety
Intubate or tracheostomy ASAP
Cx and sensitivity from blood and epiglottis surface

Vancomycin or clindamycin + ceftriaxone or cefotaxime

62
Q

Pertussis

A

under 4 mo risk respiratory failure

Incubation 7-10 days
Catarrhal stage 7-10 d - mild URI symptoms
paroxysmal stage 2-8 wks: paroxysms of cough with inspiratory whoop - worse at night, often with post-tussive emesis and exhaustion
-often confused with acute bronchitis
Convalescent stage 2-3 weeks - waning sxs

Dx: clinical
Nasopharyngeal swab for PCR

Tx:
Azithromycin x5 d, TMP-SMX x 14 d
ppx close contacts - azithromycin

Isolation from school/day car x5 days of abx or 3 weeks after onset of sxs in untreated pt

Hospital admit:
Respiratory distress
Pneumonia
Inability to feed
Cyanosis or apnea - with or without coughing
seizures
63
Q

RSV bronchiolitis

A

RSV (MC), flue, parainfluenza type 3, adenovirus, metapneumovirus

Presentation
1-3 days of mild URI: copious rhinorrhea, mild cough, mild fever, Poor feeding

Cough with tachypnea, expiratory wheezing, crackles and hyper resonance from air trapping

Possible respiratory distress with nasal flaring, tachypnea, retractions, grunting

Possible apnea in neonates

CXR: hyperinflation, patchy perihilar infiltrates

Tx:
supportive, O2 and IVF prn
Steroids NOT effective
Abx and ribavirin NOT USED

ppx: palivizumab in premies to decrease risk of hospitalization

64
Q

Rubeola - measles

A

Prodrome 2-3 days: fever (persists), malaise, anorexia, 3 C’s (cough, coryza, conjunctivitis)
Koplik spots - white-gray spots with red base on buccal mucosa - 48 hours before rash
Rash 5 days after prodrome onset: erythematous, maculopapular rash, Head and spread to feet, last 4 to 5 days, resolves from head down - darkens and coalesces

Tx:
Supportive 
Risk superinfections - treat PNA or OM
Vitamin A
Ribavirin (not standard of car, harms measles virus in vitro)
65
Q

Rubella virus - German measles

A

Low-grade fever, LAD, rash
Prodrome: malaise, fever, anorexia 1-5 days before rash

LAD: suboccipital and posterior cervical nodes

Rash: erythematous, tender, maculopapular, starts on face then generalizes - lasts 5 days, does not darken or coalesce like measles

Fever mild, only day 1
Polyarthritis up to 1 mo in women and adolescents

66
Q

Erythema infectiosus (fifth disease)

A

Parvovirus B19
Prodrome: sore throat, headache, cough, coryza, diarrhea x2-5 days
Slap cheek rash -> trunk and extremities - area around lips spared
Rash resolves within two weeks

Sickle cell - risk aplastic anemia
Pregnant - hydrops fetalis and intrauterin fetal death

67
Q

Roseola infantum (sixth disease)

A

HHV-6
Sudden high fever >102 x3-4 days - no other s/s
Rash appears as fever dissipates - starts on trunk then spreads over body, lasts 24 hrs

Erythematous papules on soft palate and uvula, mild cervical LAD, edematous eyelids, bulging anterior fontanelle in infants

Commonly misdiagnosed as acute OM and subsequent antibiotic allergy

Tx: antipyretics as needed

68
Q

Scarlet fever

A

S. pyogenes
Rash:
-“sandpaper like” - erythematous and blanching “sunburn-like”
-Starts on trunk then generalizes - spares palms and soles
-prominent in creases of axilla and groin - Pastia’s lines/sign

Strawberry tongue, beefy-red pharnyx, cervical LAD
Fever/chills

later desquamation of hands and feet

Positive throat cx or rapid strep test

69
Q

Desquamation of hands and feet - ddx

A

Scarlet fever
kawasaki disease
Toxic shock syndrome
acrodyna of mercury poisoning

70
Q

Coxsackie - hand, foot, mouth disease

A

Constitutional fever and anorexia
Oral vesicles on buccal mucosa and tongue - rupture -> shallow ulcers

Small, tender, maculopapular/vesicular rash on the hands and feet, sometimes buttocks

duration 3-5 days without complications

71
Q

acute and bilateral cervical LAD in child

A

usually viral

URI: rhinovirus, adenovirus, influenza, group A strep
Mono - EBV or CMV or Mycoplasma
Other: HIV, HSV

72
Q

Acute and unilateral cervical LAD in child

A

usually bacterial

S. aureus, group A strep > anaerobes, group B strep

73
Q

Chronic and unilateral cervical LAD in child

A

Bartonella henseale - cat scratch fever
Toxoplasmosis
TB -> scrofula
Actinomyces israelii -> sinus tracts drain pus

74
Q

cervical LAD in child without infectious cause

A

Kawasaki disease

Hodgkin lymphoma

75
Q

PFAPA syndrome

A

Periodic fever, aphthous ulcers, pharyngitis, and adenitis
-occurs monthly

2-5 yo
Benign, self limited
lasts 4-8 yrs

Tx: glucocorticoids relieve sxs in hours
Tonsillectomy may be beneficial

76
Q

Fanconi anemia

A
Presentation:
Pancytopenia
Short stature
Hyperpigmented spots and café au lait spots
Abnormality of the thumbs - dangling
Microcephaly or hydrocephaly
Developmental delay

Labs:
Pancytopenia
Increased AFP
Bone marrow: hypocellularity, increased chromosome of breakage in lymphocytes

Tx: hematopoietic cell transplantation

Complications:
Death from bone marrow failure
Increased risk of cancer- leukemia, head and neck

77
Q

Diamond-Blackfan anemia

A

ribosome mutation

Presentation:
RBC anemias
Craniofacial abnormalities
Ophthalmologic abnormalities
Neck anomalies
Cardiac anomalies
Thumb anomalies
GU malformations
Pre and postnatal growth failure

labs:
Macrocytic anemia, decreased reticulocytes
Normal WBC and platelets
BM: normal cellularity, decreased or absent erythroid precursors

Tx: corticosteroid, blood transfusion
Increased risk of cancer

78
Q

Neuroblastoma

A

MC adrenal tumor (or sympathetic ganglion) and children

Presentation:
Abdominal distention and pain
Weight loss
Malaise
Bone pain
Diarrhea
Abdominal mass
Hypertension
Horner syndrome
Proptosis
movement disorders
hepatomegaly
Fever
periorbital bruising

Labs:
increased vanillylmandelic acid (VMA) and homovanillic acid in 24 hr urine

Dx: CT
Tx: surgery

No episodic hypertension, diaphoresis like pheo

79
Q

Rhabdomyosarcoma

A

MC soft tissue sarcoma in kids - striated muscle
Majority - extremities - 50% thigh, buttock, groin

Presentation:
Gradually enlarging painless mass
Hematogenous spread to lung

Dx: MRI and bx

Tx: chemo, rad, surgery
75% at 5 yr if localized

80
Q

Developmental dysplasia of hip

A

Risk factors:
Female - breech, FHx
Male - breech

Displacement of femoral head from acetabulum

Hip click on Barlow and Ortolani
Asymmetric skin folds/ creases
On equal leg length - glotsy sign
Trendelenburg gait

Dx: hip u/s, XR after 4 mo

Tx:
Pavlik harness - maintain hip in abduction - until 6 mo
dx after 6 mo - closed (or open) reduction and spica casting
After 2 yr uncorrected - open reduction
after 8 yo - no attempt to correct

81
Q

Slipped capital femoral epiphysis (SCFE)

A

Early adolescent
Separation of femoral epiphysis from the metaphysis

Presentation:
Overweight or athletic teen boy
Limp, pain on hip movement
limited internal rotation and abduction of hip

Dx: XR - ice cream falling off cone

Tx: operative stabilization - avoid weight bearing

Complications: avascular necrosis, premature OA

82
Q

Legg-Clave-Perthes disease

A

3-8 yo
Avascular necrosis of capital femoral epiphysis - unknown cause
Gradually worsening limp, decreased range of motion

Dx: XR - icecream melting

Tx: non-weight bearing, usually no surgery
50% recovery fully
increased risk of OA and early hip replacement

83
Q

Osgood-Schlatter disease

A

Inflammation of the insertion of the patellar tendon at the tibial tuberosity

MC sxs: anterior knee pain, increases over time and and worsened by quadriceps contraction (running, jumping)

Signs:
tibial tuberosity - soft tissue swelling, palpable bony mass and/or pain upon quadriceps flexion

Tx:
can continue sports despite pain
Rehab - stretch hamstrings and quads, strengthening quads
protective pad over tibial tuberosity
ICE after activities
NSAIDs for pain

DO NOT USE knee immobilizers

84
Q

Nursemaid’s elbow - radial head subluxation

A

Subluxation of radial head out of the annular ligament
ages 1-4

Pulling or traction on outstretched arm - lifting child by arm

young child holding arm pronated and partially flexed or extended at elbow

Dx: XR

Tx: supination/flexion or hyperpronation

85
Q

Duchenne muscular dystrophy

A

Becker MD - less severe, slower progression

X linked - dystrophin gene

Presentation:
2-3 yrs old
Progressive proximal muscle weakness, clumsy, difficulty with getting up from seated position (Gower sign)
Braces by 10, wheelchair by 12
Skeletal abnormalities - curvature of spine
Dilated cardiomyopathy, conduction abnormalities - extensive fibrosis of LV wall
Pseudohypertrophy of calg - fibro-fatty infiltrate
Shortening of the Achilles tendon
Waddling gait
Hyporeflexia or areflexia

Dx:
Elevated CK before sxs (like polymyositis)
muscle bx: muscle degeneration, isolated opaque hypertrophic fibers, replacement of muscle with fat and connective tissue
EMG: poly phasic potentials, increased fiber recruitment
Dystrophin analysis - decreased on immunoblots
Genetic analysis - definitive

Tx:
Prednisone at 5
ECHO q1 yr starting at 10

86
Q

Osteogenesis imperfecta

A

Type 1 collagen disorder

Presentation:
Leg deformities - fractures in utero
Blue sclera
Pliable skin and joint hypermobility
Teeth deformities
Deafness or hearing loss - fractures of ossicles

Tx:
No contact sports, restrict activity
Bisphosphonates

87
Q

Rickets

A

Open growth plate
Deficient mineralization of the growth plate
Calcipenic - calcium deficiency due to vitamin D deficiency MC
Phosphopenic - due to phosphate wasting in kidneys

Risk: exclusively breast fed, dark skin and pigmentation, living in extreme North or South

Presentation:
Delayed closure of the fontanelles
Parietal and frontal bossing - softened skull bones
Enlargement of the costochondral junction with beading - rachitic rosary
Widening of the wrists, bowing of the distal radius and ulna
Lateral bowing of the femur and tibia
Bone pain
Delayed walking
kyphoscoliosis
Limb weakness
Decreased height

Tx: vit D supplements
Infants 400 IU/d (32 oz formula)

88
Q

Scoliosis

A

Curvature of the spine in the coronal plane, rotation of spinal column

Adolescent idiopathic scoliosis - after 10, most asx

Dx: plain XR - Cobb angle >10 degrees

Tx: observation (most), bracing, surgery

89
Q

Spondylolisthesis

A

MC L5-S1
Anterior slip of vertebrae -> palpable step off

Subacute back pain, worse with exertion or hyper extension
Knee flexed, hip flexed gait

Neurologic dysfunction - rare urinary incontinence

90
Q

Juvenile idiopathic arthritis

A
Late childhood, early teens
Nonmigratory arthropathy (1+ joint) for more than 3 mo

Subtypes:
Pauciarticular - few joints - 3-4, never hip
Polyarticular - many
Systemic onset - spiking fevers, maculopapular rash, HSM, LAD, elevated WBC, anemia (looks like leukemia) - sxs start first

Tx:
NSAIDs -> corticosteroids, anakinra, canakinumab, methotrexate, cyclosporine, tacrolimus

F/u - ophtho eval for uveitis

91
Q

Childhood hydrocephalus

A

Noncommunicating: obstruction of the CSF in the fourth ventricle
Communicating: dysfunction of the subarachnoid cisterns or arachnoid granulation (problem with reabsorbing CSF)

Presentation:
bulging fontanelles
Increased Head growth
Dilated scalp veins
Lethargy, vomiting, Poor appetite, Irritability
Headache
Diplopia, papilledema
poor skull suture fusion (older)

Dx:
LP - measure pressure
US, CT, MRI - expanded ventricles

Tx: acetazolamide or furosemide temporarily
Shunting

Complications:
Increased risk of epilepsy, bacterial infection with shunt
50% mortality before 3 if untreated

92
Q

Arnold-chiari malformation

A

Downward displacement of the cerebellar tonsils and medulla through the foramen magnum

Type I: MC and often asx
-headaches and/or cerebellar symptoms - 18 yo

Type II (of IV severity)

  • other neuro anomalies - myelomeningocele
  • detected at birth
  • dysphasia, arm weakness
  • stridor, apneic speels
  • paralysis below myelomeningocele
93
Q

Cerebral palsy

A
Risk:
Preemie
IUGR
Birth trauma
Neonatal seizure
Cerebral hemorrhage
Intrapartum hypoxia
Multiple births
Intrauterine infection - chorioamnionitis

Types:
Spastic - pyramidal tracts
dyskinetic - extrapyramidal

Features:
Encephalopathy - lethargy, decreased spontaneous movement, hypotonia, suppressed primitive reflexes
Spastic features: spastic paresis of any or all limbs, clonus present
Athetosis features: slow, writing movements in distal muscles
Choreiform features: rapid, irregular, unpredictable contractions of muscles in face or extremities
Dystonic features: uncontrollable jerking, writhing, or posturing
Persistence of primitive reflexes
Involuntary grimacing
Tendency to drool
Delayed psychomotor development
Atonic features: severe hypotonia at birth - no future ability to walk or stand

Associated disorders: MR, epilepsy, impaired speech, hearing, vision

Tx:
Muscle relaxation: botox, dantrolene, baclofen, benzos
PT
Surgery
Speech therapy - dysarthria
Special ed
Social and psych support
94
Q

Tay-Sachs disease

A

AR - hexosaminidase A def
Progressive deterioration of nerve cells

Risk:
Ashkenazi Jew, Cajun, French Canadian

Presentation:
Normal development for first 6 months
Blindness
Deafness
Dysphasia
Muscle weakness
Paralysis
Death before 4
Cherry red macula

Dx: decreased hexosaminidase activity, DNA analysis

Tx: supportive

95
Q

Retinoblastoma

A

MC intraocular tumor in kids, less than 2 yo
Rb gene - risk osteosarcoma

Presentation:
Decreased vision
eye inflammation
leukocoria - white reflex

Dx:
Chalky light gray retinal mass on dilated eye exam
-soft friable consistency
No CT - risk 2nd hit
MRI

Tx: enucleation, chemo, rad, cryotherapy, laserphotoablation

94% at 5 yr

96
Q

When is a renal or bladder US (RBUS) indicated

A

child under 2 with febrile UTI
any age with recurrent febrile UTIs
any age with FHx of urologic disease, poor growth, or HTN
UTI does not respond to abx

97
Q

when is a voiding cystourethrogram (VCUG) indicated

A

any age with 2 or more febrile UTIs

any age with first febrile UTI + anomalies on US or temp over 39C and pathogen other than E. coli or poor growth or htn

98
Q

posterior urethral valves

A

MC obstructive lesion

Abnormal tissue folds in the distal prostatic urethra
Thickened bladder wall, b/l hydronephrosis, megaureter

Suspected in utero with distended bladder and hydronephrosis (b/l) +/- oligohydramnios

Dx: VCUG

Tx: cystoscopic ablation of valves

99
Q

Urethral displacement

A

Episadias - open on dorsum (top), associated with extrophy of bladder

Hypospadias - opening on ventrum (bottom)

Decreased foreskin ventrally - partial circumcision

DO NOT circumcise
Tx: surgical repair

100
Q

Enuresis

A

after 5, treat after 7
Motivational thearpy
Restrict fluids before bed - increase daytime fluids
Enuresis alarm for classic conditioning - most effective
Oral desmopressin DDAVP

Secondary - psychologic or infection

101
Q

Beckwith-Weidman Syndrome

A

hemihypertrophy
abdominal wall hernias
new born hypoglycemia
risk wilms tumor

102
Q

Wilms Tumor

A
MC 2-4 yo
Palpable flank mass
Abdominal pain
Hematuria
Hypertension

Dx: renal fx
CBC
CT scan - abd for mets
CXR - mets

Tx: resect/nephrectomy
Chemo

10% B/L

103
Q

WAGR syndrome

A

Wilms tumor
Aniridia
GU abnormalities
Retardation - mental

104
Q

Autism spectrum disorder

A

Clinical features:
Deficits in communication and social interaction
-lack of responsiveness to others
-delayed language skills, difficulty with nonverbal communication, Poor eye contact, absence of social smile

Restricted or repetitive patterns of behavior:

  • peculiar repetitive motor behaviors - rocking, spinning, hand flapping
  • insistence on sameness, inflexible adherence to routine
  • fixation or fascination with specific interests or objects
  • hyperactivity or hyporeactivity to sensory input

Tx:
Behavioral therapy
Speech therapy
Social psychotherapy with peers and family

105
Q

ADHD

A

Clinical features:
Attention deficit - decreased attention span, inability to complete tasks, forgetfulness
Hyperactivity - increased energy and impulsiveness, excessive talking, excessive motor activity

Diagnosis - six symptoms before 12, multiple settings

Tx: stimulants, SNRIs, alpha 2 agonists

106
Q

Conduct disorder

A
Features:
Persistent pattern and behaviors that violate social norms and the rights of others
Aggressive behavior
Violence
Threatening/bullying
Destruction of property
Deceitfulness
Theft
Risk of drug use

Diagnosed in patients under 18

Tx:
Psychotherapy
Moods stabilizers

107
Q

Oppositional defiant disorder

A

Clinical features:
Persistent pattern of disobeying authority and hostile behavior
No serious violations of social norms
No disregard for the rights of others
No aggression or violence
No illegal activity
No destruction of property or cruelty to animals

Tx:
Strict clear-cut rules and consequences
Well defined boundaries

108
Q

Tourette’s syndrome

A

Features:
Vocal tics
Motor tics
Tics occur daily and are worsened by stress
Present for at least one year
Diagnosed 10-11 yo, usually resolves by 18

Tx:
Behavioral therapy
Pharmacotherapy - alpha2 agonists (clonidine), Neuroleptics (fluphenazine or pimozide)

If refractory - deep brain stimulation of the globus pallidus, thalamus, or other subcortical targets