Peds Flashcards
% children with myelo who have hydrocephalus at birth
What percentage of myelomeningoceles (MMCs) are associated with hydrocephalus at birth?
sexual development of females with MMC
Between 12% and 15% of girls with MMC show precocious puberty; 95% have menses.
Which is the major cause of calcaneal deformity in myelomeningocele (MMC)?
unopposed ankle dorsiflexors
Milestones
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.
Myotonic muscular dystrophy
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.
AIDP
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.
MMC incidence
1 per 1,000 births
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.
Miniature end-plate potentials
Milestones
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
Neural tube defects occur between
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)
The most common identifiable risk factor for childhood ischemic stroke is:
congenital heart disease
Poor outcome in JIA
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
Which dislocation common after birth brachial plexus injury
Glenoid dysplasia with posterior shoulder subluxation is frequently a complication of children after birth brachial plexus palsy.
Surgical referral for Erb’s palsy in infants
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.
Elevated transaminases and toe walking can be found in
DMD
What is the most common congenital musculoskeletal deformity associated with myelomeningocele?
Equinovarus foot (downward and inward) . The foot is generally in equinus, with forefoot and hindfoot varus and severe adduction.
Glycogen phosphorylase deficienc
associated with McArdle’s disease.
Glycogen phosphorylase deficiency
associated with McArdle’s disease.
The bladder capacity in children is based on the Berger equation –
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
risk factor that is associated with development of a venous thromboembolism (VTE) in a child with traumatic brain injury (TBI).
TBI due to non-accidental trauma
Purpose of orthosis in neuromuscular scoliosis
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.
splinting position for wrist in JRA
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.
L4 vs L5 muscle contractures in spina bifida
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.
For patients with peripheral vascular disease, the best method of assessing their walking endurance is:
Constant load treadmill test
only clear benefits for creatine for increasing performance in exercise have been found in
short duration, high intensity activity.
Massage techniques
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.
MET activity
Lying quietly is 1.0 MET. Climbing stairs is equivalent to 3-4 METs, and heavy gardening is equivalent to 4-5 METs.
Muscle fiber types
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).
Energy transfer types
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.
Scheuermann kypohsis surgery if
curve >75 deg, refractory pain, or neuro deficit…less than 50 deg conservative, 50-75 deg TLSO and conservative NSAID, PT, Ice
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
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.
syndromes associated with limb deficiencies (5)
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
upward slant palpebral fissues, brushfield spots, third fontanelle, protruding tongue, low set ears, excess nuchal skin, single palmar crease, CHD (endocardial cushion defect), VSD
trisomy 21
Intrauterine growth retardation, short palpebral fissues, small mouth, micrognathia, low set ears, prominent occiput, short sternum, hypolastic fingrenails, rocker bottom feet, CHD
trisomy 18 (edward)
IUGR, coloboma of iris *keyhold pupil”, capillary hemangioma, skil defect of skull, polydactyly, rocker bottom feet, cleft palate, CHD, urinary tract abnormalities
trisomy 13 patau syndrome (survival less than 3 days)
short stature, triangular face, webbed neck, shield/broad chest, wide set nipples, amenorrhea, coarctation of aorta
turner synd 45,x
tall statur, small testicles, gynecomastia, incr risk for mitral regurg, learning problems, infertility
klinefelter’s syndrome 47 x,x,y
height in kid *** by 4 years
weight end of first year
doubles
triples
head midline head held when pulled to sit in prone lifts head and chest slightly turns to supine hands midline open crude palmar grasp
4 months
maintains sitting, rolls to prone, transfers cube from hand to hand, intermediate grasp, single word
7 months
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
10 months
walks alone, piles two cubes, scribbles, uses spoon with spilling
14 months
heel strike, mature supporting base, arms lower while walking, points to named body part
18 months
begins running, walking up and down stairs, jumps on both feet in place, builds 8 cube tower
2 years
triccyle, runs well, copies circle, three word sentence
3 years
hops on 1 foot, walks down stairs with alternating feet, copies a cross
4 years
skips, tiptoes, drows man with head and body
5 years
adduction of forefoot on hind foot with heel in slightly valgus
metatarsus varus
Malalignment of talocalcaneonavicular complex
CAVE
midfoot CAVus
forefoot adductus
hindfoot EQUINUS VARUS
talipes equinovarus
conservative serial casting ponseti method.surgical correction is last resort
excessive dosiflexion and eversion of ankle/foot
talipes calcaneovalgus
Galeazzi test
flex hip and knees and look at level of knees, if knee is lower its either hip dysplasia or short femur
hip dysplasia treatment
closed reduction and use of pavlik harness or hip spica to keep hip reduced and to limited adduction..if diagnosis delayed may need surgery.
causes of torticollis
muscular fibrosis, ( intrauterine position causing ischemia), cervical hemi vertebrae, atalntoaxial rotary subluxation
treatment for transient synovitis, legg calve perthes, scfe
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
result of vertebral body slippage due to a spondylolysis (fx of pars)
isthmic spondylolisthesis, most common in children, pars defect usually L5, then L4 then L3.
Dysplastic (congenital spondylolisthesis-
congenital malformation of facet joint and may cause spondylolysis as slippage increases
surgery in children with spondylolisthesis
over 50% slippage, progrss neuro deficits, unstable spondy
Juvenile idiopathic arthritis (JRA)
cervical spine is often affected more than it is in adults
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
AS in kids
hip and lower extremitity….peripheral joint involvement…hip disease is poor predictor
Most common primary malignant tumor in children
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%.
Rheumatic fever
prior strep infection
Jones criteria: Carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules….treatmenet anti inflammatory salicylates and steroids and PT
Septic arthritis in children
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
Hemophilia
A: factor VIII def
B (christmas): Factor IX def
C: XI def
hemarthrosis…replace missing factor
Erythema migrans, arthritis, cardiac disease, neuro disease…heart block, bells palsy
Lyme disease Borrelia burgdorferi; tx doxycyline, amoxicillin, erytheromycin, ceftriaxone
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
Kawasaki disease
Most common cause of ped burn
scald burn
Rule of 9’s burn for kid
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
NUMBER 1 COMMON CANCER CHILDREN
NUMBER 2
NUMBER 3
LEUKEMIA (ALL), BRAIN CANCER (MEDULLOBLASTOMA), NEUROBLASTOMA (ARISES FROM SYMPATHETIC GANGLIA AND ADRENAL MEDULLA)
MOST COMMON MALIGNANT SOFT TISSUE TUMOR IN CHILDREN
RHABDOMYOSARCOMA…NEUROFIBROMATOSIS ASSOCIATION, MOST COMMONLY OCCURS IN HEAD AND NECK
osteosarcoma location
metaphysis of long bones, distal femur , followed by proximal tib and proximal humerus
ewing sarcoma location
long and flat bones, pelvis and most typically diaphysis
Neuroendocrine dysfunction s/p TBI peds
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
hearing impairment in CP
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
Asymmetric tonic neck reflex
suppression around 6-7 months…stimulus is turning head to one side
Palmar grasp reflex suppression
5-6 months
spina bifida area of highest incidence
british isles, ireland, wales, scotland….lowest in Japan
foot form in spina bifida
T6-T12: equinus foot
complication of myelomeningocele
ACM II with hydrocephalus
tethered cord most common complication
wakness, then scoli, then pain, then ortho deformity, then urologic dysfunction
Urinary dsyfunction very common in myelomeningocele
T10-L2 sympathetic adrenergic innervation
S2-4: Parasympathetic cholinergic innervation
S2-5: Somatic innervation thru pudendal plexus
most common cause of death in myelodysplasia
central respiratory dysfunction
proteus UTI in kids associated with
calcinosis
higher the SCI dysfunction
the lower the IQ
Self cath at what age
5-6
Crutches, house hold ambulation, wheelchair…. in spina bifida, what level
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
employment rate of spina bifida
25-50%
Myopathic gait
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
DMD
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!!!!!
DMD
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
Congenital muscular dystrophy
hypotonia and weakness, arthrogryposis, dislocation of hips, autosomal recessive
Facioscapulohumeral Muscular Dystrophy
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
Emery Dreifuss MD (lots of contractures)
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
Limb girdle syndrome
Auto recessive
limb girdle MD have FKRP is a known gene mutation causing limb-girdle muscular dystrophy type 2I.
Myotonic myopathies (MMD, myotonia congenita, Schwartz Jampel, congenital myotonic dystrophy)
Myotonic muscular dystrophy/steinerts disease: AD, long thin face with masseter and temporal wasting
Myotonic Muscular Dystrophy
FAutosomal dominant; rontal baldness, gonadal atrophy, cataracts, cardiac dysrhythmia, long thing face with temporal and masseter muscle wasting, IQ reduced, greater DISTAL > PROX weakness
Myotonia Congenita Little Hercules
Autosomal dominant vs recessive; stiffness esp in cold, difficulty releasing grip, myotonic dive bomber EMG
Schwartz jampel
hypotonia, dwarfism, bone disease
Congenital Myotonic Dystrophy
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…
Mitochondrial myopathies
Disease severity variable vc mitochondria….Auto Recessive or Maternal pattern……
Neonatal myesthenia, transient
born to MG moms so AChR Receptor +++++, difficult feed, weak, hypotonia, resp difficulties, facial weakness and ptosis, self limiting
Congenital or infantile myasthenia
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
Infantile botulism
10 days-6 mos, preserved DTR interestingly
AIDP in kids
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
CIDP in kids
EMG shows focal conduction block, temporal dispersion of CMAPs, prolonged distal motor latencies, slow conduction velocities, and absent/prolongedH and F wave
HMSN (CMT) I II III IV
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
toxic neuropathies
arsenic: axonal or demyelinating
vincristine: pure motor axon polyneuropathy
N hexane: distal motor and sensory demyelinating PN
Mercury: distal motor axon neuropathy
ESRD and DM neuropathy
distal motor and sensory PN with both axonal and demyeilnating features …stocking glove distribution
SMA I, II, III
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
Friedreichs ataxia
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.
Exercise in NM disease
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
Number one cause of mortality in childhood NMJ disorder
pulmonary complications
Uhthoff’s phenomenon
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
safe treatment option for MS during pregnancy
Glatiramer acetate is a safe treatment option during pregnancy
dermatomyositis vs polymyositis biopsy
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.
Reduce nursemaid elbow by
Pronating the flexed arm
abnormal cobb angle
anything greater than 10deg
abnormal cobb angle
anything greater than 10deg
surgery for greater than 45 deg
