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ORAL EXAM CASES > Chronically-ill child > Flashcards

Chronically-ill child Flashcards

1
Q

What are the causes of chronically ill child?

A

Congenital/Neuromuscular disorders

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2
Q

What are congenital/ neuromuscular disorders?

A

CH ?: CONGENITAL/ NEUROMUSCULAR DISORDERS
According to WHO/CDC, the ff are the most common congenital/NM disorders:
1. Cleft lip and cleft palate
2. Neural tube defects
3. Limb deficiencies

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3
Q

What is the patho of cleft lip/palate?

A

CH 310: CLEFT LIP AND PALATE
Patho
- Hypoplasia of the mesenchymal layer during
embryogenesis results to failure of the medial nasal and maxillary processes to join (cleft lip) or the palatal shelves (cleft palate)
- Cleft lip may be associated with other facial anomalies; cleft palate may be associated with CNS anomalies

Etiology
- Maternal drug exposure, genetics
- Incidence highest among Asians and native Americans

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4
Q

What are the clinical manifestations of cleft lip/palate?

A

CM
1. Cleft lip – small notch in the vermilion border to a
complete separation involving the skin, muscle,
mucosa, tooth, and bone. May be unilateral (L>R) and
involve the alveolar ridge
2. Cleft palate – occur in the midline involving the uvula
or extend into or through the soft and hard palate to
the incisive foramen
3. Cleft lip + palate – involve midline of soft palate to the
hard palate on one or both sides, exposing the nasal
cavity

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5
Q

Diagnostics for cleft lip/palate?

A
  • Clinical, by direct observation and palpation
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6
Q

What is the management of cleft lip/palate?

A

Mgt
1. Mgt of feeding difficulties – breastfeeding/obturator, soft artificial nipples with large openings, squeezable bottle: assist feedings; aspiration precautions

  1. Refer to ENT/Pedia Surg
  2. Surgical closure of cleft lip (Millard technique) – done at 3 mos age. May require revision at 4-5yo.
  3. Surgical closure of cleft palate – before 1yo.
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7
Q

Complications of cleft lip/palate?

A

Complications
- Recurrent otitis media, hearing loss, malposition of
teeth, speech difficulty

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8
Q

What are neural tube defects and causes?

A

CH 591: NEURAL TUBE DEFECTS
- Spina bifida and anencephaly
Spinal cord malformations
- Due to failure of the neural tube to close spontaneously between the 3rd and 4th week of in utero
development
- RF: hyperT, drugs, malnutrition, chemicals, maternal
obesity or DM, genetic determinants adversely affect
CNS development from conception
- 75% are lumbosacral (may be located anywhere along
the neuroaxis)

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9
Q

What is spina bifida occulta?

A

-midline defect of the vertebral bodies w/o protrusion of spinal cord or meninges
-most are asymptomatic
-rule out occult spinal dysraphism which usually presents with cutaneous skin manifestations (hemangiomas, discoloration, pit, lump, hairy patch, dermal sinus)
-associated with more significant anomalies (syringomyelia, diastomyelia, lipoma, tethered cord)

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10
Q

what is meningocele?

A

-meninges herniate through a defect in the posterior vertebral arches or anterior sacrum
-spinal cord is usually normal
-fluctuant midline mass that might transilluminate usually in the lower back
-most are well covered with skin
-look out for urologic manifestations such as bladder/bowel incontinence or dysfunction

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11
Q

What is myelomeningocele?

A

-most severe form of dysraphism
-meninges, along with SC elements, herniate through the defect
-affects many structure (skeleton, GIT, GUT)
-In newborns: sac-like cystic structure covered by a thin layer of skin or partially epithelialized tissue
-may have paralysis of the lower extremities, areflexia, sensory abnormalities and bladder and bowel abnormalities
-Chiari II malformation presents with hydrocephalus and myelomenigocele (80% of the time)

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12
Q

Diagnostics for neural tube defects

A

Dx
1. XR – assess vertebral bodies and bony structures
2. UTZ – assess soft tissue structures (SC)
3. MRI – diagnostic test of choice, expensive, requires sedation
4. Cystometogram – ID neurogenic bladder
5. Cranial CT scan – r/o hydrocephalus

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13
Q

Managament of NTDs

A

Mgt
1. Prevention
- Prenatal screening of maternal serum for AFP in the
16th-18th wk AOG: ID pregnancies at risk for fetuses with NTD in utero
- Maternal periconceptual use of folic acid 0.4mg OD reduces incidence (started 3 mos before conception
until at least the 12 wks of gestation when neurulation is complete)

  1. Surgery – immediate surgery is indicated for CSF leaks
    to prevent complications
    a. Repair of myelomeningocele
    b. Shunting procedure for hydrocephalus
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14
Q

Prognosis for NTDs

A

Prognosis
- With aggressive tx: 10-15% mortality rate
- 70% of survivors have N intelligence

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15
Q

What is Juvenile Myasthenia gravis?

A

CH 612: JUVENILE MYASTHENIA GRAVIS
- Chronic autoimmune disease of neuromuscular blockade characterized by rapid fatigability of striated muscle (esp extraocular and palpebral muscles of swallowing)

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16
Q

What is the pathophysiology of Juvenile Myasthenia Gravis?

A

Patho
- The release of acetylcholine (ACh) into the synaptic cleft by the axonal terminal is normal, but the postsynaptic muscle membrane (i.e., sarcolemma) or motor end plate is less responsive than normal. A decreased number of available ACh receptors is as a result of circulating receptor-binding antibodies in most cases of autoimmune myasthenia.

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17
Q

What are the clinical manifestations of Juvenile Myasthenia Gravis?

A

CM
1. Muscle weakness – earliest and most constant sign.
Unilateral or bilateral, progressive to life-threatening; usually asymmetrical ptosis and extraocular ms, respi
ms involvement

  1. Diplopia, ptosis
  2. Dysphagia, facial weakness
  3. Infancy – feeding difficulties: cardinal sign; aspiration,
    airway obstruction, poor head control
    - Progressive weakness: bulbar muscles –> limb-girdle
    ms –> distal hand muscles
  4. No muscle fasciculation, myalgias or sensory sx
  5. Rapid fatigue of muscles – characteristic feature
    - Unsustained upward gaze, unsustained head holding
    from supine, inability to elevate arms for >1-2min, more sx’c at PM
  6. Myesthenic crisis – acute or subacute severe increase
    in weakness usually precipitated by an intercurrent infection, surgery, or emotional stress
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18
Q

Diagnostics

A

Dx
1. Electromyography (EMG) – more diagnostic than muscle biopsy;
- Unique pattern: decremental response to repetitive nerve stimulation; N motor nerve conduction velocity:
correlates to the fatigable weakness

  1. Muscle biopsy – not required; lymphorrhages, type II muscle fiber atrophy
  2. Anti-AchR Ab – inconsistent
  3. ANA – r/o other autoimmune ds
  4. TFT, CK N – r/o other causes
  5. Clinical test for MG – administration of short acting
    cholinesterase inhibitor (edrophonium chloride): ptosis
    and ophthalmoplegia improve within a few sec, fatigability of other ms dec
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19
Q

What is the management of Juvenila Myasthenia gravis?

A

Mgt
1. Cholinesterase inhibiting drugs – primary tx:
neostigmine methylsulfate 0.04mg/kg IM q4-6h, neostigmine bromide po 0.4mg/kg q4-6h; pyridostigmine

  1. Prednisone
  2. Plasmapheresis, IVIG
  3. Myesthenic crisis – IV cholinesterase inhibitors, IVIG,
    plasma exchange, gavage feeding, transitory MV

Complication
- Do NOT give succinylcholine or pancuronium –> paralysis for weeks after a SD
- Aminoglycosides (gentamicin) potentiate MG

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20
Q

What is GBS?

A

CH 616: GUILLAIN-BARRE SYNDROME (GBS)
Etiopatho
- Autoimmune disorder thought to be a post-infectious polyneuropathy (symmetric ascending muscle
weakness or paralysis)
- Etiology: autoimmune reaction that develops in response to a previous infection leading to aberrant
demyelination of peripheral nerves and ventral motor nerve roots

21
Q

What are the manifestations GBS?

A

Symptoms:
*Initial symptoms include numbness and paresthesia followed by progressive weakness and areflexia
*weakness begins in the lower extremities and progressively involves the trunk, upper limb and bulbar muscles (Landry ascending paralysis)

Signs:
*Cranial nerve deficits leading to dysphagia, dysarthria, facial weakness, papilledema, autonomic dysfunction, respiratory muscle paralysis
*Dysphagia and facial weakness: signs of impending respiratory failure
*Miller-Fisher syndrome: acute ophthalmologic, ataxia, areflexia

22
Q

Give the course of GBS

A

*Onset of weakness usually follows a nonspecific viral infection (GI or respiratory tract) by 10 days (Campylobacter jejuni, H. pylori, Mycoplasma pneumonia, Zika virus)
*Maximal severity of weakness is reached by 4 weeks after onset
*Benign clinical course with spontaneous recovery within 2-3 weeks
*tendon reflexes usually the last function to recover and lower extremity weakness last to resolve

23
Q

Diagnostics

A

CSF analysis: increased protein, normal glucose, no pleocytosis
-dissociation between high CSF protein and a lack of cellular response is diagnostic (albuminocytologic dissociation)

Electrodiagnostic tests:
reduced motor and sensory nerve conduction velocity
EMG may show acute denervation of musclesM

24
Q

Management of GBS

A

ACUTE STAGE:
*admit for observation because ascending paralysis may occur within 24 hrs
*Monitor respiratory effort by spirometry to identify hypoventilation and respiratory failure
*For rapidly progressive ascending paralysis; may give IVIG (0.4 grams/kg/day for 5 consecutive days)

SUPPORTIVE CARE
prevention of ulcers
pain management
nutritional support

25
Q

What is HIE?

A

CH 99: HYPOXIC-ISCHEMIC ENCEPHALOPATHY (HIE)
- Important cause of permanent damage to the CNS tissues that may result in neonatal death or manifest
later as cerebral palsy or developmental delay
- Death 20-30% in neonatal period, 33-50% with permanent neurodevelopmental abnormalities (CP, MR)
- Greatest RF: 1. Acidosis (pH <6.7), 2. Base deficit
>25mmol/L

Etiology
- Fetal hypoxia secondary to anesthesia, CHD, respiratory failure, maternal and placental hypoxic
events
- Postnatal hypoxic events such as CHD, respiratory failure, severe anemia from hemorrhage or hemolytic disease, shock

26
Q

What is pathophysiology of HIE?

A

Pathophysio
- Hypoxia, ischemia –> anaerobic metabolism –> increased lactate and inorganic phosphates –>
glutamate accumulate in damaged tissue –> inc amounts of intracerebral Na and Ca –> tissue swelling and cerebral edema –> inc fee radicals and NO –> inc shunting through the ductus venosus, DA, FO with transient maintenance of the perfusion of brain, heart, adrenals > lungs, liver, kidneys, intestines

27
Q

What are the clinical manifestations of HIE?

A

CM
1. Pallor, cyanosis, apnea, bradycardia, unresponsiveness
to stimulation

  1. Cerebral edema and brainstem depression – sz
  2. Systemic organ dysfunction – heart failure, cardiogenic shock, persistent pulmonary HTN, RDS, GI perforation,
    acute kidney injury
  3. Depressed level of consciousness, periodic breathing with apnea or bradycardia
  4. CN function often spared
  5. Hypotonia
  6. Severe encephalopathy – coma, apnea, absence of oculocephalic reflexes, refractory sz (poor prognosis)
  7. Microcephaly, poor head growth – indicate damage to basal ganglia and white matter
  8. Brain death – coma unresponsive to pain, auditory, or visual stimulation, apnea with PCO2 rising from 40->60mmHg without ventilatory support, absence of
    brainstem reflexes (pupillary, oculocephalic, oculovestibular, corneal, gag, sucking)
  9. Sarnat staging
28
Q

What are the diagnostics for HIE?

A

Dx
1. Diffusion-weighted MRI – preferred method. Earliest to be positive. Inc signal intensity and swelling of grey
matter

  1. CT scan – ID focal hemorrhagic lesions, diffuse cortical injury, damage to basal ganglia. Diffuse edema with effacement of CSF-containing spaces, dec cortical grey
    matter attenuation with loss of N grey-white differentiation, dec B basal ganglia attenuation,
    reversal sign (reversal of N attenuation of grey and
    white matter). T1 hyperintensities (cortical laminar necrosis)
  2. Amplitude-integrated electroencephalography (aEEG)
    – determine highest risk for long-term brain injury
29
Q

What is the management of HIE?

A

Mgt
1. Whole body/selective cerebral therapeutic hypothermia – reduce mortality and major
neurodevelopmental impairment. Decreases apoptosis and suppress production of mediators, glutamate, NO, free fradicals, lactate. Reduce core T to 33.5C within the 1st 6 hours after birth. For stage 2 and 3.

  1. Phenobarbital – DOC for sz
  2. Supportive – NGT/OGT feeding, OT, PT
30
Q

What is static encephalopathy?

A

CH ?: STATIC EWhaNCEPHALOPATHY
- Umbrella term of chronic nonprogressive brain disorder in children – cerebral palsy, mental
retardation

31
Q

What is cerebral palsy?

A

CH 598: CEREBRAL PALSY (CP)

Etiopatho
- Group of permanent disorders of movement and posture causing activity limitation that are due to nonprogressive disturbances in the developing fetal or infant brain
- A group of “brain damage” syndromes in which a static
and nonprogressive cerebral lesion produces significant motor delay, abnormal neuromotor
findings, onset during the developmental period and
deficits in cognition and behavior
- Motor disorders are often accompanied by disturbances of sensation, perception, cognition,
communication and behavior as well as by epilepsy and secondary musculoskeletal problems
- MC chronic motor disability that begins in childhood

32
Q

What is the epidemiology of Cerebral palsy?

A

Epidemiology
- M>F
- Major lesions that contribute to CP in preterm babies
are:
o Intracerebral hemorrhage
o Periventricular leukomalacia

  • Risk factors for developing CP:
    o Congenital anomalies external to the CNS
    o Intrauterine exposure to maternal
    infections
    o LBW esp <1000 g at birth
33
Q

Clinical manifestations of Spastic hemiplegia (25%)

A

*arms>legs (shows hand preference at a very early age)
*decreased spontaneous movement on the affected side
*delayed walking or walks on tiptoes
*circumductive gait
*spasticity apparent especially in the ankles
*seizures (1/3 of patients) and cognitive impairment (25%)

34
Q

Clinical manifestations of spastic diplegia (35%)

A

*bilateral spasticity of the legs (legs >arms)
*damage to the immature white matter (during the 20-34th week AOG)
*commando crawl
*Increased DTRs, (+) Babinski sign and ankle clonus
*Scissoring posture of extremities is seen when child is suspended by te axilla
*normal intellect
*periventricular leukomalacia (most common neuropathologic finding)

35
Q

Clinical manifestations of spastic quadriplegia (20%)

A

*most severe form of CP due to marked motor impairment of all extremities and high association with intellectual disabilities and seizures
*swallowing difficulties die to supranuclear bulbar palsy
*severe periventricular leukomalacia and multicystic encephalomalacia (most common nueropathologic lesions)
*increased tone and spasticity in all extremities, brisk reflexes and plantar extensor responses

36
Q

Clinical manifestations of Athetoid/Dyskinetic/extrapyramidal (15-20%)

A

*hypotonic with poor head control and marked head lag
UE&raquo_space; LE affected with dystonia (rigid muscles throughout their range of motion and involuntary contractions can occur)
*feeding difficulties, tongue thrust, drooling
*absent or slurred speech
*Intellect is preserved; seizures are uncommon
*can also be caused by kernicterus

37
Q

Levine (Poster) criteria: >4/6 strongly point to CP

A

Levine (Poster) criteria: >4/6 strongly point to CP
Posturing/abnormal movement
Oropharyngeal problems (tongue thrusts, swallowing abN)
Strabismus
Tone (hypo/hypertonia)
Evolutional maldevelopment (primitive reflex)
Reflexes (inc DTR/(+) Babinski)

38
Q

Diagnostics for CP

A

Dx
1. EEG – baseline; to determine the extent and location of structural lesions and associated congenital
malformations
2. Cranial MRI – same as EEG
3. Hearing and visual function tests

39
Q

Management of CP

A

Mgt
1. Multidisciplinary approach in the assessment and treatment
2. For tight heel cord – tenotomy of Achilles tendon
3. Drugs for spasticity
a. Diazepam po 0.01-0.3 mkd BID or QID
b. Baclofen 0.2-2mkd BID or TID
c. Dantrolene 0.5-1 mkd BID
d. Levodopa (for dystonia) 0.5-2 mkd
e. Reserpine or Tetrabenazine (for
hyperkinetic movements)
f. Botulinum toxin
g. Deep brain stimulation
4. PT, OT, speech therapy

40
Q

What is intellectual disability?

A

CH 36: INTELLECTUAL DISABILITY
- Refers to a group of disorders that have in common deficits of adaptive and intellectual function and an
age of onset before maturity is reached.
- Neurodevelopmental d/o’s that begin in childhood and are characterized by intellectual difficulties as well as difficulties in conceptual, social, and practical areas of living
- Onset of sx before 18yo or adulthood
- Significant impairment = IQ <70, or 2 SD below mean
- Must have a significant delay in 1 of 3 areas:
conceptual skills (language and comprehension), social
skills, representative practical skills (ADL)
- “Global developmental delay” if not yet officially
diagnosed, usually 3yo

41
Q

Etiology of Intellectual disability

A

Etiology
- Mild: IQ 50-70
Severe: IQ<70
- Genetic/chromosomal syndromes (MC for severe ID),
IUGR, PT, maternal drug or alcohol use
- M>F 2:1

42
Q

Clinical manifestations of intellectual disability

A

CM
1. Dysmorphisms – earliest sign (NB period)
2. Infancy – gross motor delay
3. Toddler – language delay
4. Preschool – language delay, motor delay
5. School age – academic underachievement, behavior
problems
6. Associated disorders – sz, CP, hypotonia, autism
7. At 6-18mos old – gross motor delay (lack of sitting, crawling, walking): MC sx
8. DSM-V criteria:
- Deficits in intellectual functioning – “reasoning, problem solving, planning, abstract thinking,
judgement, academic learning, and learning from experience” – confirmed by clinical evaluation and
individualized standard IQ testing

  • Deficits in adaptive functioning that significantly hamper conforming to developmental and sociocultural standards for the individual’s
    independence and ability to meet their social responsibility
  • The onset of these deficits during childhood
43
Q

Diagnostics

A

Dx
1. MRI
2. Metabolic, genetic, and chromosomal testing
3. EEG
4. Bayley Scales of Infant Development (BSID-III) – MC
infant intelligence scale
5. Wechsler scales – MC psychologic test for >3yo
6. Vineland Adaptive Behavior Scale (VABS) – MC
adaptive behavior test

44
Q

Management of intellectual disability

A

Mgt
1. Special education – single most important discipline involved in the tx of children with ID
2. Continuous support and supervision
3. Family counseling

45
Q

What is BPD?

A

CH 416: BRONCHOPULMONARY DYSPLASIA (BPD)/ NEONATAL
CHRONIC LUNG DISEASE
- Pathologic process leading to s/sx of chronic lung disease that originates in the neonatal period
- Oxygen requirement for 28d postnatally
- Mild, moderate, or severe based on supplemental oxygen requirement and GA

46
Q

What is the pathophysiology of BPD?

A

Patho
- Neonates weighing <1000g includes the contribution of immature development of airway and vascular
structures of the lung

  • New BPD: impaired alveolar devt
  • Classic BPD: airway injury, inflammation, alveoloseptal
    fibrosis due to O2 toxicity, barotrauma, infection
47
Q

what are the clinical manifestations of BPD?

A

CM
1. Tachypnea

  1. Mouth breathing – due to narrowed nasal passages
    and high arched palate
  2. Increased anteroposterior diameter – air trapping
  3. Intercostal retractions
  4. Baseline wheeze or coarse crackles, persistent fixed
    wheeze or stridor (if with subglottic stenosis) or large airway malacia, fine crackles (fluid overload)
  5. Severe – prolonged mechanical ventilation to achieve
    acceptable gas exchange
  6. Growth failure – from elevated energy expenditure
  7. Chronic respiratory insufficiency, elevated serum bicarbonate, elev CO2 pressure, polycythemia
  8. Pulmonary exacerbation during RTIs, OM, weather changes, cigarette smoke exposure and GER – inc work
    of breathing, tachypnea, retractions more prominent
48
Q

STUDY THE BPD CLASSIFICATIONS

A
49
Q

Management of BPD

A

Mgt
Goal: Dec work of breathing and normalize gas exchange
1. Inhaled B-agonist – increase air movement and
improve comfort of breathing
2. Ipratropium bromide, oral methylxanthines – second
line
3. Inhaled glucocorticoids, LRTIs – for inflammatory
triggers

ORAL EXAM CASES (51 decks)

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