Pediatrics Flashcards
Transactional Model for Child/Adolescent Disorders (3 components)
CNS development/maturation, Bidirectional feedback mechanisms, and Academic/behavioral/psychological manifestations
CNS Development/maturation
Biogenetic factors (chromosomal abnormalities, temperament) and environmental factors (pre/postnatal toxins, birth complications)
Bidirectional feedback mechanisms
Intellectual, cognitive, and perceptual capacity, subcortical structures (RAS, thalamus) and Cortical Structures (frontal, temporal, parietal, occipital, association regions)
Academic, behavioral, and psychological manifestations
Home environment (parents, SES), social environment (peers) and school/classroom (expectations, teaching, reinforcement)
Folate supplementation
Recommended during pregnancy to prevent neural tube defects
Risk factors associated with premature birth
- periventricular leukomalacia (PVL)
- hypoxic ischemic encephalopathy (HIE)
- Intraventricular hemorrhage (IVH)
Key window of brain development
ages 0-6
Tourette’s syndrome
- Tic disorder marked by multiple motor and vocal tics
- Tics increase with stress
- Symptoms present before age 18
Dopamine hypothesis of Tourette’s
Deficiency in dopamine in the prefrontal lobes corresponding with sensitivity of dopamine receptors in the striatal and nucleus accumbens region, results in disinhibition of subcortical regions
lack of DA in frontal lobes + sensitive DA receptors in striatal and nucleus accumbens = disinhibition of subcortical regions
excess dopamine in the striatum is thought to excite the thalamo-cortical circuits, producing tics. When external stressors activate the hypothalamic-pituitary-adrenal (HPA) axis, more dopamine is produced, furthering the excitation of tic-producing pathways
Coprolalia
Involuntary/repetitive use of obscene language
(rare symptom of Tourette’s)
Echolalia
Repetition of words just spoken by another person (symptom of Tourette’s)
Tourette’s is commonly comorbid with
OCD
Autism (brain areas)
Abnormalities in the cerebellum, brain stem, limbic system, and temporal lobes
Enlargement of lateral ventricles
Delayed maturation of frontal lobes
Reduced activity in the amygdala during processing of facial emotions
Children with autism would be expected to be below expected performance on
Executive control and frontal lobe function tasks, perseverative errors
- Tower of Hanoi
Asperger’s
Marked by impaired social interactions, normal development of age-appropriate self-help/adaptive bx, restricted/repetitive/stereotyped patterns of bx/interests/activities
Flat/dull affect, monotonous speech, little reciprocity, lack of interest in others
Appears before age 4
ADHD
3-5% school age children
Disturbance of stimulus boundedness, disturbances in attention span, self-reg, activity level, and impulse control
Conduct disorder
3-7% in general pop, 3x more likely in males
Marked by aggressive bx without taking into consideration the feelings of others, physical/verbal aggression, stealing, lying
Negative bias on testing with the tendency to interpret ambiguous stimuli as hostile/negative
Sociopathic “Macdonald” Triad
1) Arson/obsession with fire starting
2) Animal cruelty
3) Enuresis
Language disorders
Symptoms include stuttering/stammering, not understanding words, inability to express themselves, difficulty speaking words/making sentences, delayed speech
Auditory Processing Disorder
Appears to have combination of many symptoms including:
- Difficulty understanding speech
- Difficulty following multi-step directions presented verbally
- Difficulty attending to long lectures
- Difficulty remembering/summarizing info presented verbally
- Difficulty reading, spelling, writing
- Trouble following abstract thought/ideas
- Delayed/misunderstanding jokes, sarcasm, etc.
Learning Disorder in Reading
- Difficulty acquiring rapid, context-free word reading skills
- Associated with symmetrical planum temporale
- Reduced insular and frontal lobe volumes
- Cortical malformations in the frontal/temporal areas
Learning disorder in math
Subtypes include anarithemetria, acalculia, and spatial dyscalculia
Anarithemetria
Difficulty performing math operations
Acalculia
Deficits knowledge of math concepts
Spatial dyscalculia
difficulty organizing and manipulating numbers spatially
Written Language Expression
May be a function of bilateral hemispheric substrates
Dysfunction in complementary roles in processing:
- Right hemisphere (anterior, central, posterior)
- Left hemisphere (temporal-parietal, anterior)
Nonverbal LD
Intact left hemisphere with right hemisphere dysfunction resulting in complex social/emotional and academic difficulties
Tactile/visual interception deficits, impaired complex psychomotor skills, inattention to tactile/visual info, verbal skill deficits (prosody, semantics, content)
Most commonly associated with math LD
Developmental delay
Failure to meet expected developmental milestones in 1+ of following:
= physical, social, emotional, intellectual, speech/language, adaptive development
Child performs below 25-35% of age norms
Progress occurs at slower than expected rate
Possible causes of developmental delay
- Chromosomal abnormalities
- Genetic/congenital disorders
- Severe sensory impairments (hearing/vision)
- Inborn errors of metabolism
- Disorders relating to disturbance of the development of the nervous system
- Congenital infections
- Exposure to toxins (e.g., FAS)
Gershwind-Galaburda theory
Proposes that embryonic surges of testosterone delay the development of the left hemisphere in males
Down’s Syndrome
Common chromosomal disorder, most common is trisomy 21
mild to severe cognitive impairment, small head, flat nose, folds in corners of eyes, protruding tongue, heart/ear/eye defects
Sleep apnea common
Prone to spinal injuries
Phenylketonia (PKU)
abnormal recessive disorder affecting phenylalanine to tyrosine metabolism causing bx/antisocial problems (may look like ADHD)
Lesch-Nyhan Syndrome
Progressive metabolic disorder caused by abnomality of an enzyme (HGPRT) on the X chromosome, severe cognitive impairment and often accompanied by choreoathetoid movements (twitching/writing)
Fragile X
X chromosome compressed/broken, more common in males
females have higher rates of normal IQ, marked by long face, prominent ears/jaw/forehead
Klinefelter Syndrome
extra X chromosome on #47, marked by infertility, male breast development, underdeveloped masculine build, social/cognitive/academic difficulties, long legs, tall stature, small testes/penis, bx problems, peer relation difficulties
Noonan Syndrome
Short stature, webbed neck, ptosis (upper eyelid droop), hypertelorism (increased distance between the eyes), high arched palate, low set ears
Generally lower IQ but relatively preserved verbal skills
X-linked andenoleukodystrophy
Marked by visual-perception deficits
Neurofibromatosis/Von Recklinghausen’s
inherited, spots of skin pigmentation, benign tumors on/under skin, tumors in the iris, focal lesions (basal ganglia, subcortical white matter, brain stem, cerebellum), learning problems, anxiety due to appearance, seizures
Tuberous Sclerosis
Heart/lungs/bones/kidney problems, facial lesions, white spots on the skin
Results from abnormal proliferation of brain cells and glia during embryonic development, cognitive impairment, seizures, hemiplegia
Sturge-Webber Syndrome
Seizures, cognitive impairment, bx difficulties, infantile hemiplegia
Partial Seizures
Structural lesions, no LOC, can develop to generalized tonic-clonic (grand mal)
Simple partial seizures
Grey matter origin/motor strip with electrical discharge, no LOC
Complex partial seizures
LOC/loss of impairment
Absence Seizure
Petit mal, abrupt LOC, return to normal activity, most consistent with a neuropsychological deficit in visual memory
Tonic-clonic seizure
Grand mal, most common in children, LOC followed by a fall and crying
Febrile Seizures
Convulsion in a child that may be caused by a spike in body temperature (fever)
Can be simple or complex
Cerebral Palsy
a group of disorders that affect a person’s ability to move and maintain balance and posture
Injuries causing CP can occur pre/post natally (infancy to early childhood)
“floppy baby” with gradual increase in restricted movement
6 subtypes: spastic hemiplegia, spastic quadriplegia, spastic diplegia, extrapyramideal CP, atonic CP, ataxic CP
Fetal Alcohol Syndrome
Result from birth parent consuming EtOH during pregnancy
Side effects
- low body weight
- non-typical facial features
- vision and hearing problems
- cognitive difficulties
- executive fx challenges
Pediatric tumors brain locations
70% are posterior fossa (a small space in the skull, found near the brainstem and cerebellum)
30% supratentorial (the upper part of the brain)
Resection of a cerebellar tumor may be marked by
Mutism, ataxia, and emotional lability
Leukemia
Radiation may lead to cognitive impairment - can manifest as non-verbal LD
Can impact social relationships because of range of emotions and stress
Turner Syndrome (TS)
Affects females, results from one of the X chromosomes being missing/partially missing
Short height, failure of ovaries and heart to develop
Weakness in processing speed, motor skills, math
Landau-Kleffner Syndrome
Rare, age-related epileptic encephalopathy manifesting in children ages 3-8 despite previously normal development
Regression in language, epilepsy, and social-cognitive deficits
Acquired aphasia and paroxysms (sudden spasm)
INITIAL SYMPTOM: auditory agnosia
Flynn Effect
the substantial and long-sustained increase in both fluid and crystallized intelligence test scores over time (i.e., why someone would score lower on the WAIS-IV than the WAIS-III)
Most important factor to consider when interpreting discrepancies between test scores in children
Base rates
Should you allow others to sit on on a child assessment?
NO
