Certification QBank Pediatrics 31-60 Flashcards

1
Q

a 6-year-old boy with Down syndrome presents for evaluation of torticollis. Mother reports that he often tilts his head to the right, and it is especially notable when he is watching TV. He denies pain. What is your main concern about etiology of his head tilting before starting therapy?

A. Congenital shortening of the right sternocleidomastoid

B. Atlantoaxial rotatory fixation

C. Ocular superior oblique muscle palsy

D. Arnold-Chiari malformation

A

B. Atlantoaxial rotatory fixation (AARF)

AARF associates with syndromes like trisomy 21, Klippel-Feil, Marfan, Larsen and Morquio. Bony abnormalities such as AARF can be the etiology for torticollis in Down syndrome patients, and in this case occupational therapy will be contraindicated. Cervical radiographs (AP-L views) his first-line tests. Add open mouth odontoid view if AARF is suspected. Dynamic CT is useful in assessing C1-2 rotation in different positions of neck rotation. If cervical CT is positive, do a cervical MRI/MRA to check for spinal cord, ligamentous and vascular injuries

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2
Q

according to the American disabilities act of 1990, a person with a disability who applies for a job as typically have access to the employer which of the following question?

A. Employment coaching

B. Low cost health insurance

C. Worker’s Compensation

D. Adaptive transportation

A

C. Worker’s Compensation

The American with disabilities act of 1990, and mended 2008, states that it covered entity she will not discriminated against a qualified individual with a disability. This applies to job application procedures, hiring, advancement and discharged employees, Worker’s Compensation, and job training and other terms, conditions and privileges of employment. This gives similar protections against discrimination to individuals with disability as though stipulated in the civil rights at 1964, which may discrimination based on race, religion, sex, national origin and other characteristics of legal. Benefits such as insurance or transportation to work, need to be consistent with the company’s policy is for all employees and in accordance with state law. Public transportation does need to accommodate people with disabilities.

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3
Q

what is the mode of inheritance of spinal muscular atrophy?

A. Autosomal dominant

B. Autosomal recessive

C. sex-linked

D. Mitochondrial

A

B. autosomal recessive

spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by degeneration of anterior horn cells of the spinal cord and brainstem motor nuclei with resultant muscle atrophy and progressive weakness. SMA has traditionally been classified into 3 categories by age of onset and highest level of motor function achieved. SMA is an autosomal recessive disorder, with ileal on chromosome 5q13, although his phenotypic variability results in part from the number of repeats of the SMN2 gene

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4
Q

which joint is most commonly affected in patients with juvenile idiopathic arthritis (JIA?)

A. Hip

B. Cervical spine

C. Knee

D. Wrist

A

C. Knee

Cervical spine involvement occurs more often in children with JIA in adults, but in the is the most commonly affected JIA.

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5
Q

bracing is recommended for adolescent scoliosis with curves between

A. 5-10°

B. 20-40°

C. 50-70°

D. 70-75

A

B. 20-40°

More than 90% adolescent scoliosis can be controlled effectively with bracing for curves between 20 and 40°. Bracing is recommended for curves over 20° in a skeletally immature patient in whom only 5-10° of progression has been noted over 6 month period. Brace wears discontinued in lieu of surgical intervention generally the curve exceeds 40° or more rapid reaction is noted. In neuromuscular scoliosis, while orthoses may improve trunk control in sitting posture, the less often slow curve progression and do not prevent the need

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6
Q

a 20-year-old male with Duchenne muscular dystrophy has an FEV1 of 20% predicted and end tidal CO2 of 58 mmHg, complains of breathlessness when speaking, daily headache and fatigue. What treatment would you recommend?

A. Flu and pneumonia vaccines, aggressive pulmonary toilet and improved positioning

B. Nocturnal BiPAP with CoughAssist during the day

C. Tracheostomy and ventilator

D. Daytime BiPAP/sip and puff withcough

A

D. Per DMD care guidelines, and daytime ventilation is indicated for:

  • self extension of the nocturnal ventilation into waking hours
  • Abnormal deglutition due to dyspnea, which is relieved by ventilatory assistance
  • Inability to speak a full sentence without breathlessness and/or
  • Symptoms of hypoventilation when awake, resting on SpO2 <95% and/or large were end tidal CO2 > 45 mmHg whenweek
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7
Q

a patient with myelomeningocele presents with low back pain, decreased lower limb strength, and increased spasticity. What is most likely diagnosis?

A. Urinary tract infection

B. Intracranial hemorrhage

C. Vertebral compression fracture

D. Tethered cord

A

D. Tethered cord

Tethered cord often occurs during growth spurts of adolescence, and presents with pain, weakness, change in bladder habits, and is in spasticity. Evaluation includes MRI and neurosurgical intracranial hemorrhage is not a commoncombination of MMC, although shunt malfunction can be. UTI should not cause decreased strength

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8
Q

What lower limb deformity in children with L4 spina bifida most likely to happen?

A. Equinas but

B. Cavus foot

C. Knee flexion contractures

D. Knee extension Contractures

A

D. Knee extension contracture

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. Equinas and cavus feet results from asymmetric pull of foot muscles which would be seen in levels of spina bifida L5 and distally

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9
Q

a 6-month-old with L5 spina bifida and shunted hydrocephalus presents to your clinic. On examination, you find that he is all hip flexion against gravity and his knee extension strength is at least 4 out of 5. The infant has ankle dorsiflexion but not plantar flexion. His feet are in neutral position. Hip examination is symmetric. What will most likely be this child’s primary means of mobility as he grows older?

A. Community ambulation without assistive device

B. Short distance household ambulation with a cane

C. Use of bilateral crutches for all mobility

D. Independent manual wheelchair mobility

A

A. Community ambulation without assistive device

This child has a strong quadriceps muscle and no deformities noted at 6 months of age. He is reported to be healthy. Sitting balance and neurologic level were good predictors of ambulation potential the best early predictor of ambulation children with spina bifida is a strong quadriceps muscle. Negative predictors are spine and lower extremity deformities and obesity. Children do not typically learn to use crutches until 3-5 years of age or older

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10
Q

in the newborn with severe pan-brachial plexopathy, wears a lesion located the results in Horner syndrome?

A. C5–6 nerve roots

B. Brainstem

C. C7 nerve root

D. C8–T1 nerve roots

A

D. C8–T1 nerve roots

Horner syndrome is associated with injury of the C8-T1nerve roots with injury of the superior cervical sympathetic ganglion. Horner syndrome is associated with more severe root avulsion injuries in a poor prognosis for spontaneous recovery

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11
Q

which Maternal factors associated with an increased risk of spina bifida?

A. Anticonvulsant medications in pregnancy

B. Upper socioeconomic class

C. Asian descent

D. Folic acid 4 mg a day prior to and during pregnancy

A

A. Anticonvulsant medications in pregnancy

The etiology of spina bifida is multifactorial. Both polygenic inheritance and environmental influences contribute. Several studies have shown that the incidence of spina bifida is reduced if food is fortified with folic acid or if mother take folic acid prior to conception and during pregnancy. Recommended doses of folic acid are 0.4 mg per dayin women who are not at high risk and 4 mg per day in women at high risk (eg, those with family history of spina bifida). Some studies have also implicated lower socioeconomic class and in utero exposure to anticonvulsant medication is being risk factors. Fetal alcohol syndrome has an association with increased risk for abnormal CNS development, including NTDs. NTDs very and prevalence, depending on race and ethnicity, with women of African-American and Asian descent having the lowest while the highest is noted among women of Hispanic ethnicity

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12
Q

which the following sports is most commonly associated with pediatric SCI?

A. Basketball

B. Soccer

C. American football

D. Ice skating

A

C. American football

the sports most commonly associated with SCI R American football, ice hockey, wrestling, diving, skiing, snowboarding, rugby, and cheerleading

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13
Q

as compared to children with severe traumatic brain injuries, children with severe anoxic encephalopathy are more likely to:

A. Regain consciousness

B. Have a longer survival time

C. Develop profound rigidity

D. Recover language skills

A

C. Develop profound rigidity

Compared to children with severe traumatic brain injury, children with severe anoxic encephalopathy demonstrate a worse outcome. They are less likely to regain consciousness, become ambulatory and also have shorter survival time and often have profound rigidity

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14
Q

a 5-year-old girl has spasticity in her legs and decreased fine motor coordination. She is ambulatory with orthosis and crutches. What would be the most likely finding on a magnetic resonance scan of her brain?

A. Periventricular leukomalacia

B. Parasagittal cerebral necrosis

C. Multifocal brain necrosis

D. Basal ganglia leukomalacia

A

A. Periventricular leukomalacia

PVL is commonly seen following intraventricular hemorrhage and preterm infants. It is characterized by bilateral White Matter necrosis adjacent to the lateral ventricles. This lesion causes the clinical picture of spastic diplegic cerebral palsythat is described question. Parasagittal cerebral injury and multifocal brain necrosis usually cause spastic tetraplegic CP. Neuronal injury within the basal ganglia results in choreoathetosis.

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15
Q

an 11-year-old boy with autism and speech impairment is referred to you for evaluation of limping for 2 months. You observe he is overweight and has Trendelenburg gait. He suspects he has slipped capital femoral epiphysis (SCFE). Which findings may be present in your clinical exam

A. Increased hip internal rotation

B. Increased hip flexion

C. Outgoing gait

D. Weakness at the hip extensors

A

C. Outgoing gait

SCFE quickly occurs in boys between age 12-16 years. The 2 most common features of presentation are pain and altered gait. External rotation of the leg is common with some shortening and antalgic, Trendelenburg gait. Physical examination demonstrates a loss of internal rotation, diminished flexion, shortening of the leg, and atrophy of the thigh if the symptoms have been long-standing.

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16
Q

age appropriate independence with activities of daily living in children with spina bifida are more likely to occur when:

A. Parents insist that they will not help their children

B. The child’s equipment is in poor repair

C. The child has a lower level of spine lesion

D. The child has motor planning challenges

A

C. The child has a lower level of spine lesion

Children with spina bifida require proper equipment, in good repair. These individuals frequently have motor planning challenges, and visual perceptual issues. The lower the level of the lesion the more likely they will have better hand use and mobility

17
Q

what best describes a teenager with cerebral palsy who is a gross motor functional classification level of IV?

A. Independent ambulation in the household

B. Independent ambulation in the community

C. No independent mobility in the household

D. Wheelchair use in the community

A

D. Wheelchair use in the community

The gross motor function classification system (GMFCS) classifies mobility of people with cerebral palsy from I to V. Level 1 is independent ambulation indoors and outdoors with no assistive device. Level IV requires wheelchair for household and community

18
Q

a 10-year-old boy is referred to you because of weakness noted by his gym teacher. On examination you find mild weakness of extremity muscles, scapular winging, and calf hypertrophy. The most likely diagnosis is:

A. Duchenne muscular dystrophy

B. Emery Dreyfuss muscular dystrophy

C. Becker muscular dystrophy

D. Merosin-deficient muscular dystrophy

A

C. Becker muscular dystrophy

Becker muscular dystrophy (BMD) usually presents later then DMD, ages 4–12, with milder cases presenting later. It is milder than Duchenne muscular dystrophy (DMD) which usually presents before the age of 6 years. Dystrophin is absent in muscles in DMD, whereas in BMD it is decreased or abnormal. Patients with Emery-Dreifuss muscular dystrophy have weakness, elbow contractures, and normal dystrophin on muscle biopsy. Patients with merosin-deficient muscular dystrophy usually have severe contractures and do not attain ambulation

19
Q

what is the most common location for brachial plexus palsy and children?

a. Upper trunk

B. Lower trunk

C. Posterior cord

D. Medial cord

A

A. upper trunk

Injury of the upper trunk, C5-6, is called Erbs palsy, sometimes called Erb-Duchenne palsy. This is the most common level of involvement, present in approximately 3/4ths of those with birth brachial plexus palsy

20
Q

what is the strongest predictor of a seizure related motor vehicle crash?

A. Nocturnal only seizures

B. History of partial complex seizures

C. Duration of seizure free interval

D. Current medication transition

A

C. duration of seizure-free interval

Seizures are responsible for 1% of all motor vehicle collisions. Minimum requirements for length of seizure-free time before a person can drive very from state to state range from 3-12 months. The strongest literature confirmed predictor of a seizure related more to vehicle collision is duration of seizure-free interval. A 12 month seizure-free interval prevents 80% of crashes.a 3 month interval presents 50% of crashes. Other risk factors for motor vehicle collisions in individuals with seizures include age, gender, generalized or complex seizures, partial complex seizures, history of multiple seizures, and noncompliance with antiepileptic medications. The probability of a seizure related collision is decreased by long. Of seizure free, reliable aura, and the use of the least sedating but most effective medication

21
Q

what is the pathophysiological disturbance in Mucopolysaccaridoses?

A degradation of glycosaminoglycan’s

B. Glycogen phosphorylase deficiency

C. Dystrophic and dysfunction

D. Loss of anterior horn cells

A

A. Degradation of glycosaminoglycans

Mucopolysaccharidosis consists of several different types. There is now enzyme replacement available for many of the types. Cervical spine is frequently unstable, and MPS is the most common cause of carpal tunnel syndrome in children. Dystrophin dysfunction is associated with Duchenne muscular dystrophy. Loss of anterior horn cells is associated with spinal muscle atrophy. Glycogen phosphorylase deficiency is associated with McArdle’s disease.

22
Q

which of the following distinguishes the asymmetric tonic neck reflex (ATNR) from the symmetric tonic neck reflex (STNR)?

A. Present at birth and disappearance by 6 months

B. Provides further postural stability as the child goes from crawling to standing

C. Appears at 6 months of age and disappearance by 1 year

D. Obligatory and all children at certain ages

A

A. Present at birth and disappearance by 6 months

The ATNR is also known as the fencer position and is in neonatal reflex that disappears by 6 months of age. It is never obligatory in normal children. The STNR appears about 6 months and disappears by one year, and it provides postural stability as a child makes a transition from crawling to standing

23
Q

which statement is true regarding oligoarthritis in children?

A. Symptoms present after age 6 years

B. Upper limbs demonstrate the majority of findings

C. Silent uveitis may lead to blindness

D. ANA is negative and the majority

A

C. silent uveitis may lead to blindness

the most ominous finding in children with oligo arthritis is silent uveitis this condition presents before age 6 years and is typically symmetric prominent in females. ANA is detected in the majority

24
Q

a 7-year-old boy is referred for EMG lab. He presents with a reflexia, bilateral hand intrinsic muscle atrophy, toe walking, and pes cavus his nerve conduction study and EMG will likely show

A. Uniform conduction slowing in all nerves

B. Multifocal conduction blocks

C. Low amplitude short duration action potentials

D. Normal study

A

A. Uniform conduction slowing in all nerves

The patient’s clinical features are consistent with Charcot-Marie-Tooth (CMT). The demyelinating form CMT 1 typically has onset in early childhood. Marked slowing of motor conduction velocities, usually to less than 50% of normal, is often present in early childhood. Needle EMG abnormalities include fibrillation with positive sharp waves, decreased interference pattern, and large amplitudepolyphasic motor unit action potentials (MUAPs)

25
Q

which condition is most associated with brachial plexus palsy in newborns?

A. Low birth weight

B. Shoulder dystocia

C. Lifelong impairment in most

D. Nulliparous

A

B. Shoulder dystocia

Shoulder dystocia (ie. arm first) during delivery is a significant risk factor for brachial plexus injury. As opposed to adults with brachial plexus palsy, the mechanism of injury makes C5-6 most vulnerable. Those infants with large birth weight are more susceptible and spontaneous recovery is found in the majority. Those that do not recover spontaneously, rehabilitation and surgical intervention are used to normalize them function and optimize nerve regeneration

26
Q

what percentage of teenagers with myelomeningocele require a bowel program?

A. 10%

B. 90%

C. 50%

D. 25%

A

B. 90%

The caregiving burden for parents of children with myelomeningoceleand social alienation experienced by teenagers with poorly managed neurogenic bowel are profound

27
Q

which of the following conditions is least likely to confound the assessment of child abuse

A. Von Willebrand’s disease

B osteogenesis imperfecta

C. Seizure disorder

D. Hemophilia

A

C. Seizure disorder

Children with hemophilia and von Willebrand’s disease may have significant bruising disproportionate to the reported trauma. Children with osteogenesis imperfecta and those with metabolic bone disease can have fractures of varying ages that is out of proportion to the reported trauma. A child with child abuse is unlikely to present with a nicely seizure disorder and no other signs of injury

28
Q

which salivary glands should be targeted with botulinum toxin to control anterior drooling and there is no food stimulation?

A. sublingual

B. Parotid

C. Submandibular

D. Buccal glands

A

C. Submandibular

Targeting the appropriate salivary gland is essential. Since the submandibular glands are the major producers of saliva when the patient is not stimulated by food, and the parotid glands produce mainly during feeding or other oral motor stimulation, these are the glands to target. Because sublingual glands produce less than 5% of saliva, they typically are not injected. The Buccal glandsare located in the mucous membrane lining the cheeks and the mouth. This gland produces only a small amount of saliva. The parotid glands can be localized by surface anatomy, whereas the submandibular glands require ultrasound guidance. Current study shows significantly decreased anterior drooling after botulinum toxin a injections to the submandibular and parotid glands. It should be noted that use of botulinum a is off label for those under 18years of age

29
Q

which Federal act delineated the provision of special education and related services for children with disabilities

A. Americans with disabilities act

B. Individuals with disability education act

C. Individual education program act

D. Rehabilitation act

A

B. Individuals with disability education act (IDEA)

Speech, occupational therapies and physical therapies are provided to students in school because they are related to the students education, as mandated in section 504 of the rehabilitation at of 1973. This law states that children with chronic conditions and disabilities are entitled to appropriate modifications within their educational program to accommodate their individual needs.the provision of special education and related services was further delineated Individuals with Disabilities Education Act (IDEA) in December 2004. As defined by part a of the IDEA, “related services” include speech-language pathology and audiology services, psychological services, physical and occupational therapy, recreation, social work services, rehabilitation counseling, and medical diagnostic services to assist the child to benefit from special education. The Americans with disabilities act (ADA) was signed into law on July 26, 1990 president George HW Bush. The ADA is 1 of America is most comprehensive pieces of civil rights legislation prohibits discrimination and guarantees that people with disabilities have the same opportunities as everyone else to participate in mainstream of American life. The IEP provides for individualized programming for any student receiving special education services

30
Q

in indicator of a poor outcome or juvenile idiopathic arthritis (JIA) is:

A. Asymmetrical disease

B. Late onset of wrist involvement

C. Absence of rheumatoid factor

D. Early radiographic changes

A

D. Early radiographic changes

indicators of poor outcome of J IA 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 change