Peds

Question 1

% children with myelo who have hydrocephalus at birth

Answer 1

What percentage of myelomeningoceles (MMCs) are associated with hydrocephalus at birth?

Question 2

sexual development of females with MMC

Answer 2

Between 12% and 15% of girls with MMC show precocious puberty; 95% have menses.

Question 3

Which is the major cause of calcaneal deformity in myelomeningocele (MMC)?

Answer 3

unopposed ankle dorsiflexors

Question 4

Milestones

Answer 4

Palmar grasp involves picking up an object with the whole hand. Scissor grasp is achieved at 8 months and uses all four fingers and the side of the thumb. Pincer grasp uses the thumb and index finger. Fine pincer grasp is achieved at 12 months and uses the thumb and DIP of the index finger.

Question 5

Myotonic muscular dystrophy

Answer 5

Myotonic muscular dystrophy affects skeletal muscle, smooth muscle, myocardium, brain, and ocular structures. Associated findings include frontal pattern baldness, gonadal atrophy (in males), cataracts, insulin insensitivity, and cardiac dysrhythmias.

Question 6

AIDP

Answer 6

Although all of the answer choices can be seen, motor involvement predominates. Campylobacter jejuni, Mycoplasma pneumoniae, cytomegalovirus, and Epstein-Barr virus are common known causes.

Question 7

MMC incidence

Answer 7

1 per 1,000 births

Question 8

monophasic, single quantum version of a biphasic end-plate action potential of summated quanta, both of which can be found in either children or adults.

Answer 8

Miniature end-plate potentials

Question 9

Milestones

Answer 9

Palmar grasp involves picking up an object with the whole hand. Scissor grasp is achieved at 8 months and uses all four fingers and the side of the thumb. Pincer grasp uses the thumb and index finger. Fine pincer grasp is achieved at 12 months and uses the thumb and DIP of the index finger.

A child should be able to pivot circles while prone at 5 months of age. They can maintain their heads in the midline position at 2 months of age. By 3 months, they should be able to prone prop on extended elbows. At 4 months, they can roll prone to supine.

You observe a child who is able to crawl on his or her hands and knees (“creeps”). At what age should this milestone be met? 9 months

Question 10

Neural tube defects occur between

Answer 10

The nervous system is derived from ectoderm. The anterior neuropore closes around 23rd day of intrauterine life. The posterior neuropore closes at 26 and 27 days of intrauterine life. (18 to 30 days)

Question 11

The most common identifiable risk factor for childhood ischemic stroke is:

Answer 11

congenital heart disease

Question 12

Poor outcome in JIA

Answer 12

Indicators of poor outcome of juvenile idiopathic arthritis include greater severity or extension of arthritis at onset, symmetrical disease, early wrist or hip involvement, presence of rheumatoid factor, persistent active disease, and early radiographic changes,unremitting polyarticular disease, early hip or wrist involvement

Question 13

Which dislocation common after birth brachial plexus injury

Answer 13

Glenoid dysplasia with posterior shoulder subluxation is frequently a complication of children after birth brachial plexus palsy.

Question 14

Surgical referral for Erb’s palsy in infants

Answer 14

Patients with Erb’s palsy who had surgery at 6 months did better than those who spontaneously recovered elbow flexion at 5 months. Surgical intervention is commonly recommended for those having less-than-antigravity strength in elbow flexion at 6 months of age.

Question 15

Elevated transaminases and toe walking can be found in

Answer 15

DMD

Question 16

What is the most common congenital musculoskeletal deformity associated with myelomeningocele?

Answer 16

Equinovarus foot (downward and inward) . The foot is generally in equinus, with forefoot and hindfoot varus and severe adduction.

Question 17

Glycogen phosphorylase deficienc

Answer 17

associated with McArdle’s disease.

Question 18

Glycogen phosphorylase deficiency

Answer 18

associated with McArdle’s disease.

Question 19

The bladder capacity in children is based on the Berger equation –

Answer 19

age in years plus 2 equals bladder capacity in ounces up until around age 12 years when the bladder capacity equals an adult size….
2 year old is 4 oz

Question 20

risk factor that is associated with development of a venous thromboembolism (VTE) in a child with traumatic brain injury (TBI).

Answer 20

TBI due to non-accidental trauma

Question 21

Purpose of orthosis in neuromuscular scoliosis

Answer 21

Neuromuscular curve progression is rarely slowed by bracing. Orthoses may be beneficial because they can stabilize a weak trunk, and improve sitting posture in those using wheelchairs.

Question 22

splinting position for wrist in JRA

Answer 22

The correct position for splinting an involved hand in a child with juvenile rheumatoid arthritis is with the wrist in 15° of extension, the metacarpophalangeal joints in 25° of flexion, and the thumb in opposition. This position provides support for weakened structures and helps to reduce contractures.

Question 23

L4 vs L5 muscle contractures in spina bifida

Answer 23

The knee extensors (quadriceps) are innervated at the L3-4 level, while the knee flexors (hamstrings) are innervated at the L5-S1 level. A child with L4 preserved level would have quadriceps muscles that work, while hamstrings will either be weak or absent. Foot muscles are innervated at the L5-S2 levels. Equinus and cavus feet result from asymmetric pull of foot muscles, which would be seen in levels of spina bifida L5 and distally.

Question 24

For patients with peripheral vascular disease, the best method of assessing their walking endurance is:

Answer 24

Constant load treadmill test

Question 25

only clear benefits for creatine for increasing performance in exercise have been found in

Answer 25

short duration, high intensity activity.

Question 26

Massage techniques

Answer 26

Petrissage is a method of compression massage that is used to break up tissue and muscle adhesions. effleurage massage the practitioner uses a stroking motion that is beneficial for vascular and lymphatic drainage. Friction massage is a method of massage that uses circular motions to small areas of tissue to help with tendonitis and fasciitis. Acupressure is a massage method in which pressure is applied on specific body points to help reduce pain.

Question 27

MET activity

Answer 27

Lying quietly is 1.0 MET. Climbing stairs is equivalent to 3-4 METs, and heavy gardening is equivalent to 4-5 METs.

Question 28

Muscle fiber types

Answer 28

Type 1 muscle fibers are slow-twitch with oxidative metabolic pathways. Type 2 muscle fibers are fast-twitch fibers. The type 2 fibers can then be further divided into fast-twitch with both oxidative and glycolytic metabolism (type 2a) and fast-twitch glycolytic (type 2b).

Question 29

Energy transfer types

Answer 29

Conduction is a process of transferring thermal energy between 2 entities placed in direct contact with each other, for example cold packs on skin. Convection is a process of using a medium to transfer energy. Examples of convection include the use of husks with fluidotherapy, and the use of water with whirlpool therapy. Vapocoolant sprays are an example of evaporation, not conduction.

Question 30

Scheuermann kypohsis surgery if

Answer 30

curve >75 deg, refractory pain, or neuro deficit...less than 50 deg conservative, 50-75 deg TLSO and conservative NSAID, PT, Ice

Question 31

Scoliosis idiopathic- infantile (left thoracolumbar), juvenile (right thoracic or double curve), adolescent (most common) >11 yrs old, M=F, right thoracic, right thoracic/left lumbar congenital acquired

Answer 31

prominence of posterior trunk correlates to the convex side of curve, restrictive lung, decr TVC, the higher the curve the more the lung is compressed on the convex side, observe if less than 20 deg, brace is 20-40 unless neurmuscular etiology than sooner....surgery generally if curve over 40, but in CP may wait even longer.

Question 32

syndromes associated with limb deficiencies (5)

Answer 32

thrombocytopenia with absense of radius (TAR) syndrome (thrombocytopenia) Fanconi syndrome (anemia, leukopenia) Hold oram syndrome (heart) Baller-Gerold synd (craniosynostosis) VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo esophag fistula, renal dysplasia, limb deficiency

Question 33

upward slant palpebral fissues, brushfield spots, third fontanelle, protruding tongue, low set ears, excess nuchal skin, single palmar crease, CHD (endocardial cushion defect), VSD

Answer 33

trisomy 21

Question 34

Intrauterine growth retardation, short palpebral fissues, small mouth, micrognathia, low set ears, prominent occiput, short sternum, hypolastic fingrenails, rocker bottom feet, CHD

Answer 34

trisomy 18 (edward)

Question 35

IUGR, coloboma of iris *keyhold pupil", capillary hemangioma, skil defect of skull, polydactyly, rocker bottom feet, cleft palate, CHD, urinary tract abnormalities

Answer 35

trisomy 13 patau syndrome (survival less than 3 days)

Question 36

short stature, triangular face, webbed neck, shield/broad chest, wide set nipples, amenorrhea, coarctation of aorta

Answer 36

turner synd 45,x

Question 37

tall statur, small testicles, gynecomastia, incr risk for mitral regurg, learning problems, infertility

Answer 37

klinefelter's syndrome 47 x,x,y

Question 38

height in kid *** by 4 years weight end of first year

Answer 38

doubles triples

Question 39

``` head midline head held when pulled to sit in prone lifts head and chest slightly turns to supine hands midline open crude palmar grasp ```

Answer 39

4 months

Question 40

maintains sitting, rolls to prone, transfers cube from hand to hand, intermediate grasp, single word

Answer 40

7 months

Question 41

stands momentarily, cruises, pincer grasp, peek a boo, finger feeds, waves bye bye, uses mama and dada with meaning, can retrieve an object hidden from view

Answer 41

10 months

Question 42

walks alone, piles two cubes, scribbles, uses spoon with spilling

Answer 42

14 months

Question 43

heel strike, mature supporting base, arms lower while walking, points to named body part

Answer 43

18 months

Question 44

begins running, walking up and down stairs, jumps on both feet in place, builds 8 cube tower

Answer 44

2 years

Question 45

triccyle, runs well, copies circle, three word sentence

Answer 45

3 years

Question 46

hops on 1 foot, walks down stairs with alternating feet, copies a cross

Answer 46

4 years

Question 47

skips, tiptoes, drows man with head and body

Answer 47

5 years

Question 48

adduction of forefoot on hind foot with heel in slightly valgus

Answer 48

metatarsus varus

Question 49

Malalignment of talocalcaneonavicular complex CAVE midfoot CAVus forefoot adductus hindfoot EQUINUS VARUS

Answer 49

talipes equinovarus conservative serial casting ponseti method.surgical correction is last resort

Question 50

excessive dosiflexion and eversion of ankle/foot

Answer 50

talipes calcaneovalgus

Question 51

Galeazzi test

Answer 51

flex hip and knees and look at level of knees, if knee is lower its either hip dysplasia or short femur

Question 52

hip dysplasia treatment

Answer 52

closed reduction and use of pavlik harness or hip spica to keep hip reduced and to limited adduction..if diagnosis delayed may need surgery.

Question 53

causes of torticollis

Answer 53

muscular fibrosis, ( intrauterine position causing ischemia), cervical hemi vertebrae, atalntoaxial rotary subluxation

Question 54

treatment for transient synovitis, legg calve perthes, scfe

Answer 54

transient synovitis: rest, nsaids, full activity avoided until hip is pain free LCP: conservative abduction brace, continue weight bearing but with femur in abducted position so head is well contained by acetabulum; surgery is varus osteotomy if needed SCFE: surgical pinning....pain with abduction, IR and flexion of hip.... no pain with adduction

Question 55

result of vertebral body slippage due to a spondylolysis (fx of pars)

Answer 55

isthmic spondylolisthesis, most common in children, pars defect usually L5, then L4 then L3.

Question 56

Dysplastic (congenital spondylolisthesis-

Answer 56

congenital malformation of facet joint and may cause spondylolysis as slippage increases

Question 57

surgery in children with spondylolisthesis

Answer 57

over 50% slippage, progrss neuro deficits, unstable spondy

Question 58

Juvenile idiopathic arthritis (JRA) | cervical spine is often affected more than it is in adults

Answer 58

Polyarticular, pauciarticaular, systemic poly can be RF- or RF+....RF+ worse, F>M Pauci/Oligo- 50 percent develop iridocyclitis, most are +ANA and that incr risk for the ididocyclitis...routine eye exams!!!! most common joint knee -> ankle , wrist, elbows Systemic- 10-20% develop iridocyclitis, fever, rash, hepatosplenomeg, lymphadenopathy, serositis...macrophage activation syndrome can develop!!!! Enthesitis related- HLAB27, boy

Question 59

AS in kids

Answer 59

hip and lower extremitity....peripheral joint involvement...hip disease is poor predictor

Question 60

Most common primary malignant tumor in children

Answer 60

Medulloblastoma is the most common primary malignant tumor in children. Over 85% of children with this type of tumor have long-term learning and memory deficits. The highest incidence of medulloblastoma occurs in children less than 10 years old. These tumors arise in the cerebellum and have a long-term survival rate of approximately 80%.

Question 61

Rheumatic fever

Answer 61

prior strep infection Jones criteria: Carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules....treatmenet anti inflammatory salicylates and steroids and PT

Question 62

Septic arthritis in children

Answer 62

monoarticular involvement most common; H influenzae and staph aureus Newborn- Staph aureus, less commonly G- enteric bacteria 2 months - 2 yrs: H flu >2 yrs: Staph A Sexual active adolescents- Gonococcal disease

Question 63

Hemophilia

Answer 63

A: factor VIII def B (christmas): Factor IX def C: XI def hemarthrosis...replace missing factor

Question 64

Erythema migrans, arthritis, cardiac disease, neuro disease...heart block, bells palsy

Answer 64

Lyme disease Borrelia burgdorferi; tx doxycyline, amoxicillin, erytheromycin, ceftriaxone

Question 65

Systemic vasculitis that affects young children, high grade fever, strawberry tongue, red, chapped lips, pharyngeal erytehma, conjunctival injection, edema of hands/feet, erythema of palms/soles with desquamation, truncal rash, cervical lymphadenopathy

Answer 65

Kawasaki disease

Question 66

Most common cause of ped burn

Answer 66

scald burn

Question 67

Rule of 9's burn for kid

Answer 67

age 1-4: trunk 32, legs 15/15, arms 9/9, head 19, pubic 1, head 19 age 5-14: trunk 32, legs 18/18, arms 9/9, pubic 1, head 13 Adult: trunk 36, arm 9/9, leg 18/18, head 9, pubic 1 page 764

Question 68

NUMBER 1 COMMON CANCER CHILDREN NUMBER 2 NUMBER 3

Answer 68

LEUKEMIA (ALL), BRAIN CANCER (MEDULLOBLASTOMA), NEUROBLASTOMA (ARISES FROM SYMPATHETIC GANGLIA AND ADRENAL MEDULLA)

Question 69

MOST COMMON MALIGNANT SOFT TISSUE TUMOR IN CHILDREN

Answer 69

RHABDOMYOSARCOMA...NEUROFIBROMATOSIS ASSOCIATION, MOST COMMONLY OCCURS IN HEAD AND NECK

Question 70

osteosarcoma location

Answer 70

metaphysis of long bones, distal femur , followed by proximal tib and proximal humerus

Question 71

ewing sarcoma location

Answer 71

long and flat bones, pelvis and most typically diaphysis

Question 72

Neuroendocrine dysfunction s/p TBI peds

Answer 72

DI SIADH, HYPOPITUITARISM: growth failure delay arrested puberty, GH, LH, FSH, CSW, Precocious puberty (2-17 months s/p TBI) shortened stature secondary to epiphyseal closure

Question 73

hearing impairment in CP

Answer 73

sensory neural, fourfold increase in athetosis than in spasticity; kernicterus is the most common cause of sensory neural hearing loss in athetosis....other causes are intrauterine infxn rubella, CMV, toxo, syphilis, meningitis, otoxoci drugs

Question 74

Asymmetric tonic neck reflex

Answer 74

suppression around 6-7 months...stimulus is turning head to one side

Question 75

Palmar grasp reflex suppression

Answer 75

5-6 months

Question 76

spina bifida area of highest incidence

Answer 76

british isles, ireland, wales, scotland....lowest in Japan

Question 77

foot form in spina bifida

Answer 77

T6-T12: equinus foot

Question 78

complication of myelomeningocele

Answer 78

ACM II with hydrocephalus

Question 79

tethered cord most common complication

Answer 79

wakness, then scoli, then pain, then ortho deformity, then urologic dysfunction

Question 80

Urinary dsyfunction very common in myelomeningocele

Answer 80

T10-L2 sympathetic adrenergic innervation S2-4: Parasympathetic cholinergic innervation S2-5: Somatic innervation thru pudendal plexus

Question 81

most common cause of death in myelodysplasia

Answer 81

central respiratory dysfunction

Question 82

proteus UTI in kids associated with

Answer 82

calcinosis

Question 83

higher the SCI dysfunction

Answer 83

the lower the IQ

Question 84

Self cath at what age

Answer 84

5-6

Question 85

Crutches, house hold ambulation, wheelchair.... in spina bifida, what level

Answer 85

L1,2,3,4 L5 community ambulation with cruches , braces S1 community ambulation..only half need AD ``` Functional community ambulation Thoracic lesions 0-33% High lumbar 31% low lumbar 38% before age of 15 SacrAL :Full community ambulation ```

Question 86

employment rate of spina bifida

Answer 86

25-50%

Question 87

Myopathic gait

Answer 87

hyperlordotic, waddling gait (weak back/hip extensors) causing anterior pelvic tilt....patients compensate by decreasing knee flexion and posturing ankle into plantar flexionon initial contact during stance phase of gait cycle which produces a knee extension moment to maintain knee stability

Question 88

DMD

Answer 88

X linked recessive, xp21 deletion, dilated cardiomyopathy, some have intellectual retardation, CK high, degenerating fibers in clustors with necrotic fibers surrounded by macrophages/lymphocytes can have lower cognition!!!!!

Question 89

DMD

Answer 89

X linked recessive, xp21 deletion, dilated cardiomyopathy, some have intellectual retardation, CK high, degenerating fibers in clustors with necrotic fibers surrounded by macrophages/lymphocytes consider spine surgery when curve more than 20 prednisone or deflazcort muscle group earliest affected neck flexors, pelvic girdle precedes shoulder, ankle dorsiflexion weaker than plantar flexion, evertors weaker than inverters, knee extensors weaker than knee flexors, hip extensors weaker than flexors, hip abductors weaker than adductors. FVC peaks in second decade then starts to go down, FVC <40% contraindication to surgical spinal arthrodesis

Question 90

Congenital muscular dystrophy

Answer 90

hypotonia and weakness, arthrogryposis, dislocation of hips, autosomal recessive

Question 91

Facioscapulohumeral Muscular Dystrophy

Answer 91

Autosomal dominant, facial muscle weakness, then shoulder girdle; sensory neural hearing deficit, scapular winging, no cog deficit NO cardiac problems!!!! inability to extend wrist...deltoid surprisingly preserved

Question 92

Emery Dreifuss MD (lots of contractures)

Answer 92

X linked recessive also, Emerin is the protein that is deficient, atrophy in upper arms and legs due to focal wasting of calf and biceps, early presence of contractures of elbow flexors with limitation of full elbow extension Cardiac arrhythmia

Question 93

Limb girdle syndrome

Answer 93

Auto recessive limb girdle MD have FKRP is a known gene mutation causing limb-girdle muscular dystrophy type 2I.

Question 94

Myotonic myopathies (MMD, myotonia congenita, Schwartz Jampel, congenital myotonic dystrophy)

Answer 94

Myotonic muscular dystrophy/steinerts disease: AD, long thin face with masseter and temporal wasting

Question 95

Myotonic Muscular Dystrophy

Answer 95

FAutosomal dominant; rontal baldness, gonadal atrophy, cataracts, cardiac dysrhythmia, long thing face with temporal and masseter muscle wasting, IQ reduced, greater DISTAL > PROX weakness

Question 96

Myotonia Congenita Little Hercules

Answer 96

Autosomal dominant vs recessive; stiffness esp in cold, difficulty releasing grip, myotonic dive bomber EMG

Question 97

Schwartz jampel

Answer 97

hypotonia, dwarfism, bone disease

Question 98

Congenital Myotonic Dystrophy

Answer 98

Auto Dominant; almost always born to mothers with affected with myotonic dystrophy...hypotonia at birth, facial weakness, arthrogryposis, variable breathing and swallowing difficulties ...HYPOTONIA, equinovarus talipes...

Question 99

Mitochondrial myopathies

Answer 99

Disease severity variable vc mitochondria....Auto Recessive or Maternal pattern......

Question 100

Neonatal myesthenia, transient

Answer 100

born to MG moms so AChR Receptor +++++, difficult feed, weak, hypotonia, resp difficulties, facial weakness and ptosis, self limiting

Question 101

Congenital or infantile myasthenia

Answer 101

AChR antibodies usually absent (- - - - -), usually from nonmyasthenia mothers, AR inheritance....ptosis, ophalmoplegia, fatigability of muscle after rep stim, responds to IV edrophonium, single fiber EMG, pyridostigmine...thymectomy, steroid, azathioprine

Question 102

Infantile botulism

Answer 102

10 days-6 mos, preserved DTR interestingly

Question 103

AIDP in kids

Answer 103

prodromal resp / GI preceding infxn, myocoplasma, CMV, EBV, campylobacter, vaccines, weakness begins distal lower ext and ascends.....cranial nerve abnormal is ipsilateral or bilateral LMN facial paralysis...compelte recovery in most children (milder course than adults)....tx: plasma exchange and IV immune globulin

Question 104

CIDP in kids

Answer 104

EMG shows focal conduction block, temporal dispersion of CMAPs, prolonged distal motor latencies, slow conduction velocities, and absent/prolongedH and F wave

Question 105

``` HMSN (CMT) I II III IV ```

Answer 105

I: Most common; hypertrophic demyelinating neuropathy (onion bulbs) on nerve bx, gene locus 17p11 2-12 II: Axonal neuropathy with low amp, inveted champagne bottle/stork leg from wasting of the calf and anterior compartment of the leg III: Severe hypertrophic demyelinating polyneuropathy IV: AR

Question 106

toxic neuropathies

Answer 106

arsenic: axonal or demyelinating vincristine: pure motor axon polyneuropathy N hexane: distal motor and sensory demyelinating PN Mercury: distal motor axon neuropathy

Question 107

ESRD and DM neuropathy

Answer 107

distal motor and sensory PN with both axonal and demyeilnating features ...stocking glove distribution

Question 108

SMA I, II, III

Answer 108

I: severe, never sit, die before 2 (weak cry, weak suck, hypotonia), hypotonia (AR inheritance) II, never stand, milder III Kugelberg Welander: mild, stand alone, die as adults

Question 109

Friedreichs ataxia

Answer 109

AUTO RECESSIVE spinal cerebellar degeneration, onset before 20, AR...gait ataxia, ascending pattern to rest of body, weakness, atrophy, dysarthria, protein abnormalitiy is in mitochrondrial protein called FRATAXIN, prev of scoliosis is 100% if onset is younger than 10...... NYSTAGMUS too HOCM too Friedreich's ataxia is an autosomal recessive disease that affects mitochondrial function, which leads to cerebellum and spinal cord degeneration. Patients exhibit abnormal gait, which leads to frequent falls. Patients also usually develop early insulin resistance, cardiomyopathies, dysarthria, scoliosis, and pes cavus. They will have bilateral muscle weakness affecting upper and lower extremities, as well as have impaired proprioception/vibratory senses. Treatment involves physical/occupational therapy and surgery if needed.

Question 110

Exercise in NM disease

Answer 110

eccentric more mechanical stress, so concentric is better, sub max strengtheneing programs and avoid muscle soreness, aerobic training good for DMD to incr CO and o2 use by muscles...wheelchair use when KE is less than antigravity or ambulation 30 ft takes more than 12 seconds

Question 111

Number one cause of mortality in childhood NMJ disorder

Answer 111

pulmonary complications

Question 112

Uhthoff's phenomenon

Answer 112

Worsening of neurological symptoms including visual problems seen with increased body temperatur This effect is thought to reflect areas of impaired but still functioning myelin that break down in transmitting electrical impulses when surrounding fluid is heated

Question 113

safe treatment option for MS during pregnancy

Answer 113

Glatiramer acetate is a safe treatment option during pregnancy

Question 114

dermatomyositis vs polymyositis biopsy

Answer 114

dermatomyositis: perivascular and interfascicular inflammatory infiltrates with adjoining groups of muscle fiber degeneration/regeneration Histologic findings in polymyositis include endomysial mononuclear inflammatory infiltrates and muscle fiber necrosis.

Question 115

Reduce nursemaid elbow by

Answer 115

Pronating the flexed arm

Question 116

abnormal cobb angle

Answer 116

anything greater than 10deg

Question 117

abnormal cobb angle

Answer 117

anything greater than 10deg surgery for greater than 45 deg