28 Flashcards
Corrected age for premature babies
In general, when looking at growth and development in former premature infants, pediatricians use the “corrected age.”
To calculate corrected age:
Subtract gestational weeks of a premature infant from the average gestational period of 40 weeks.
Subtract the result from the chronological age to obtain the “corrected age.”
1. Use corrected age to plot growth parameters.
According to the U.S. Centers for Disease Control and Prevention (CDC), use of corrected age on growth charts should be discontinued after the child reaches 2 years.
Most premature infants have caught up by then, especially in terms of height; some still lag behind their same-age peers in weight.
2. Also use corrected age for charting developmental progress.
Milestones in speech development
7 days - 2 years old
Before 7 days of age
Can distinguish mother’s voice from another woman’s voice.
Before 2 weeks of age
Can distinguish father’s voice from another man’s voice
At 6-8 months
Has added a few consonant sounds to the vowel sounds.
May say “mama” or “dada” but does not attach them to individuals.
At 1 year
Will attach “mama” or “dada” to the correct person.
Responds to one-step commands such as “Give it to me.”
At 15 months
Continues to string vowel and consonant sounds together (gibberish), but may imbed real words.
May say as many as 10 different words.
At 18 months
Can say nouns (ball, cup), names of special people, and a few action words or phrases.
Can add gestures to her speech, and may be able to follow a two-step command.
At 2 years
Can combine words, forming simple sentences like “Daddy go.”
Developmental delays - 3 examples and the delays they cause
Determining the extent and nature of a child’s developmental delay is important not only in establishing a diagnosis but also in matching therapeutic interventions to the child’s specific needs. Many developmental disorders affect some domains of development and not others. For example:
Autism spectrum disorders primarily interfere with social interactions - screen in 18 and 24 month olds
Cerebral palsy is characterized by motor dysfunction, with variable impact on other domains.
Genetic and metabolic disorders may cause global delay.
The timing of developmental delay (i.e., delayed or failed acquisition of milestones vs. acquisition followed by loss of milestones, as occurs in neurodegenerative disorders) is also important in differentiating causes of developmental delay.
Psychosocial stressors, depending on severity, may cause or contribute to developmental delay.
Risk Factors for Developmental Delay 4
Bronchopulmonary dysplasia (BPD)
BPD (or chronic lung disease) may cause poor growth in part due to increased caloric requirements, repeated pulmonary infections or congestive heart failure.
Development in these infants may be delayed because of prolonged or repeated hospitalization due to illness, underlying neurological disease, or both.
Retinopathy of prematurity (ROP)
All premature infants, particularly those weighing less than 1500 g are at risk of ROP.
Approximately 50% of infants weighing less than 1200 g develop ROP.
ROP is characterized by extraretinal fibrovascular proliferation and, in severe cases, may cause retinal detachment and blindness.
Development in children with ROP may be delayed depending on the degree of visual impairment.
Hyperbilirubinemia
Bilirubin is a potential neurotoxin, particularly in preterm or critically ill infants.
Severe hyperbilirubinemia may lead to kernicterus, which is characterized by abnormal motor development (choreoathetoid cerebral palsy) and sensorineural hearing loss.
Largely because of advances in preventive management, kernicterus is rare.
Periventricular leukomalacia (PVL)
PVL is the result of damage to the white matter surrounding the ventricles in the brain as a result of hypoxia, ischemia, and inflammation.
It is correlated with intraventricular hemorrhage (bleeding from the delicate vessels of the neuronal and glial proliferation zone—germinal matrix—that surrounds the lateral ventricles in preterm infants and fetuses); it is likely that damage to the white matter is actually more widespread, but not easily visualized using current clinical imaging techniques.
PVL with cysts (cystic PVL) is highly correlated with cerebral palsy.
Effects of stress on childhood development
Neurodegenerative disorder effects on development
Behavioral changes may occur in response to stress within families. This is a normal adaptive response and parents should be encouraged to support the children through the stress.
Most often, a regression in previously achieved behaviors is seen in response to stress:
For toddlers, temper tantrums, sleep disturbances and refusal to eat are common adaptations.
Language acquisition may be slowed during periods of stress.
During hospitalization (an extreme form of stress) children may develop increased dependency, enuresis or encopresis.
If a child has recently gained motor milestones and is ill, the achievements may be temporarily lost.
Regression of milestones
Signs of muscle tone abnormalities 2
Spasticity
Spasticity is defined as increased muscle resistance (muscles are continuously contracted) that is velocity-dependent. This means that resistance is greater when passive movement is rapid and less when the passive movement is slow. People often have both increased tone and spasticity. Toe-walking can be a sign of increased calf tone. Check for decreased ankle dorsiflexion in children who toe-walk.
Low tone
Low tone is manifested as slumped posture, poor head control, soft muscles and the patient slipping through your hands when held under the armpits. Beyond the first few months of life, persistently closed hands or cortical thumbs (thumbs held in the palm within the fingers) are signs of CNS dysfunction.
What diagnosis would you give to an ex-29-week preemie who has delayed acquisition of developmental milestones and hypertonia and spasticity on exam?
What it is, its definition, prevalence, types, functional challenges
Cerebral Palsy
Definition
The diagnosis of cerebral palsy (CP) represents a heterogeneous group of non-progressive disorders, characterized by motor and postural dysfunction.
These conditions, which range in severity, are due to abnormalities of the developing brain resulting from a variety of causes.
Prevalence
The overall prevalence of cerebral palsy in western countries is 2/1000.
Types
Historically, the different types of cerebral palsy have been grouped together because they all involve motor abnormalities and are treated somewhat similarly.
Spastic diplegia is characterized by increased tone and spasticity predominantly involving the lower extremities. The incidence of spastic diplegia is greatly increased in premature infants.
Functional Challenges
Many individuals with cerebral palsy (CP) face significant challenges related to muscle control, including:
Mobility
Speech production
Self-care
Risks for cerebral palsy 4 and which risk has the highest incidence
The multifactorial etiology of cerebral palsy was illustrated in a series of 213 children diagnosed with CP in Australia.
Major CP-associated pathologies other than acute intrapartum hypoxia were identified in 98 percent of cases.
Some children had more than one associated pathology.
The risk of each of the above pathologies is as follows:
Perinatal asphyxia* 10% Chorioamnionitis 28% Prematurity 78% Intrauterine growth retardation 34% *Two large studies have been published about the percentage of infants diagnosed with CP who experienced a recognizable adverse event during delivery. The first was a large North American cohort study known as the Collaborative Perinatal Project; the second was the Australian study noted above.
Assessment of child with CP - 6
H&P: All children should undergo a detailed history and physical examination. It is particularly important to determine that the condition is static rather than progressive or degenerative. It is also important to classify the type of CP.
Screening: Screening for developmental delays, ophthalmologic abnormalities, hearing impairment, speech and language disorders, and disorders of oral-motor function are warranted as part of the initial assessment because these problems are commonly associated with CP.
EEG: An EEG is recommended when there are features suggestive of epilepsy.
Neuroimaging: Neuroimaging is recommended to both establish an etiology and for prognostic purposes. MRI is preferred to CT scan and does not expose the child to radiation.
Metabolic and genetic testing: Metabolic and genetic testing are considered if the clinical history or findings on neuroimaging do not determine a specific structural abnormality or if there are additional and atypical features in the history or clinical examination.
Developmental testing: The recommended in-depth developmental testing and assessment typically cannot be done in a busy outpatient clinic. Referral to a specialist will help with formal developmental testing and with coordinating services for the child. (Federally funded Early Intervention Programs are required to provide timely evaluations and interventions for infants and toddlers at risk for developmental problems.)
Intellectual disability- def, prevalence, therapy, associated syndromes 3
Intellectual Disability
Definition
The definition of intellectual disability has three components:
Significant degree of cognitive impairment (typically defined as peforming at least two standard deviations below the mean on an intelligence test, i.e., an intelligence quotient [IQ] < 70)
Significant degree of impaired adaptive function (ability to peform everyday tasks)
Both impairments manifesting before age 18
Prevalence
While prevalence depends on the definition and the population studied, it is estimated that approximately 2.5% of the population has an intellectual disability. This diagnosis may range from mild to severe.
Therapy
Intellectual disability is not treatable. However, early identification allows for the introduction of therapies or changes in environment that can improve behavior and adaptive functioning.
Associated Syndromes
Syndromes commonly associated with intellectual disability include:
Down syndrome
Fetal alcohol syndrome
Fragile X syndrome (see more below)
Fragile X Syndrome
Intellectual disability is more common in males than females. This is due in part to the frequency of X-linked syndromes. The most common of these syndromes is Fragile X syndrome:
Fragile X syndrome occurs approximately in 1/4,000 to 1/6,000 males.
Fragile X syndrome is a difficult diagnosis to make in early childhood.
A helpful finding is macroorchidism, usually documented after puberty.
Behavioral disturbances-such as hyperactivity, gaze avoidance, or autistic behavior-have been described.
It is generally accepted that males with intellectual disability be referred for evaluation of Fragile X syndrome.
Types of CP 5
A has been selected by the expert.
A. This choice is correct because dyskinetic CP is associated with kernicterus, due to hyperbilirubinemia, as well as findings of basal ganglia pathology on imaging. Patients typically have motor abnormalities throughout the body. Dyskinetic CP is also associated with perinatal asphyxia and can involve the thalamus and cerebellum on imaging.
B. This choice is incorrect because spastic diplegia is classically associated with premature birth and specific MRI findings of periventricular white matter abnormalities. Patients present with motor involvement that is more prominent in the legs than the arms.
C. This choice is incorrect because patients with spastic quadiplegia have spasticity, clonus, and exaggerated tendon jerks throughout their bodies. Imaging would show global brain abnormalities.
D. This choice is incorrect because spastic hemiplegia—associated with a stroke damaging a unilateral upper motor neuron tract—should present with spasticity of the contralateral arm and leg, not the entire body.
E. This choice is incorrect because ataxic cerebral palsy should show cerebellar abnormalities on imaging, while Amy’s MRI shows only basal ganglia involvement.
