Exam 3, deck 3 Flashcards
1
Q
Inborn errors of metabolism are frequent causes of
A
sepsis like presentations
Intellectual disability
seizures
sudden infant death
neurologic impairment
2
Q
infants who have an inborn error of metabolism often present a few hours to weeks after birth often mimicking late onset sepsis. If they survive the neonatal period they often experience
A
intermittent illness separated by periods of being well
3
Q
metabolic disorders should be considered in all neonates presenting with
A
lethargy
poor tone
poor feeding
hypothermia
irritability
seizures
4
Q
metabolic disorders should be evaluated by what 3 specific labs
A
plasma ammonia
blood glucose
anion gap
5
Q
Significant ketosis in neonate is unusual and suggests what type of disorder
A
organic acid disorder
6
Q
The introduction of fructose or sucrose in diet may lead to decompensation in
A
hereditary fructose intolerance
7
Q
In older children, increased protein intake may unmask disorders of
A
ammonia detoxification
8
Q
The toxic metabolic compensation often presents as ________, may be precipitated by
A
encephalopathy
fever
infection
fasting
or other catabolic stresses
9
Q
If an infant or child presents with features of toxic encephalopathy, what metabolic complication should be on your differential
A
Hyperammonemia
10
Q
presentation of severe neonatal hyperammonemia
A
blood ammonia >1,000
poor feeding
hypotonia
apnea
hypothermia
vomiting
resp alkalosis
progresses to
coma
intractable seizures
leads to
death if not corrected
11
Q
Moderate neonatal hyperammonemia range
A
200-400 umol/L
12
Q
Severe neonatal hyperammonemia range
A
> 1,000 umol/L
13
Q
clinical features of Moderate neonatal hyperammonemia
A
depression of CNS
poor feeding
vomiting
may have Resp alkalosis
seizures not characteristic
14
Q
Later infancy
Infants who are affected by defects in the urea cycle need what type of diet to do well?
Clinical hyperammonemia may occur when what happens?
plasma ammonia level during a crisis?
A
low protein intake of breast milk
clinical hyperammonemia when dietary protein is increased or when catabolic stress occurs (Vomiting and lethargy that can progress to coma)
200-500 umol/L
ammonia decreases when protein intake decreases …..condition may go unrecognized for years
Older children may present with neuropsych or behavioral abnormalities
15
Q
A child who has a defect in the urea cycle that has a crisis during an epidemic of influenza, the child may mistakenly thought to have what syndrome?
A
Reye syndrome
16
Q
organ presentation in metabolic disorders…
Nervous system
liver
eye
kidney
heart
A
Nervous system: seizures, coma, ataxia
Liver: Hepatocellular damage
eye: cataracts, dislocated lens
kidney: tubular dysfunction, cysts
heart: cardiomyopathy, pericardial effusion
17
Q
metabolic disorders that result in energy deficiency?
general symptoms that can manifest
A
disorders of fatty acid oxidation
mitochondrial function/oxidative phosphorylation
carbohydrate metabolism
myopathy
CNS dysfunction
ID
seizures
cardiomyopathy
vomiting
hypoglycemia
renal tubular acidosis
18
Q
basic breakdown of a metabolic dysfuction
A
deficiency of an enzyme complex results in accumulation of metabolites proximal to the blocked metabolism and deficiency of the product of the reaction.
19
Q
metabolic disorder:
In medium-chain and long chain fatty acid oxidation defects
you are deficient in?
accumulation of what toxic compound?
result?
A
Medium and long you are deficient in fat for energy
in long your accumulation of toxic compound is long chain fats. none in medium-chain.
for both the result is use of glucose with consequent hypoglycemia, however
in long-chain, you also have resultant mitochondrial dysfunction in liver, heart, ect that leads to organ dysfunction
20
Q
metabolic disorder:
Glycogen storage disease
you are deficient in?
accumulation of what toxic compound?
result?
A
you are deficient in?
glucose to prevent fasting hypoglycemia
accumulation of what toxic compound?
glycogen resulting in storage in liver, muscle and heart
result?
risk of hypoglycemic brain injury and dysfunction of tissue with storage
21
Q
metabolic disorder:
Ketone utilization disorder
you are deficient in?
accumulation of what toxic compound?
result?
A
deficient in fat for energy
accumulation of ketones
risk of hypoglycemic brain injury; profound metabolic acidosis and reversible neurologic dysfunction
Cyclic vomiting
22
Q
metabolic disorder:
Galactosemia
you are deficient in?
accumulation of what toxic compound?
result?
A
you are deficient in?
nothing listed
accumulation of what toxic compound?
Galactose
result?
elevated galactose level leads to severe hepatic dysfunction, neurologic injury and impaired immune response
23
Q
metabolic disorder:
Urea cycle defects
you are deficient in?
accumulation of what toxic compound?
result?
A
you are deficient in?
none listed
accumulation of what toxic compound?
Ammonia
result?
CNS dysfunction
probably mediated through glutamine
24
Q
metabolic disorder:
Propionic acidemia, methylmalonic acidemia, other organic acidemias
you are deficient in?
accumulation of what toxic compound?
result?
A
you are deficient in?
none listed
accumulation of what toxic compound?
organic acids
result?
systemic or local impairment of mitochondrial function; impaired neurotransmission; impairment of urea cycle
25
metabolic disorder:
Phenylketonuria
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
Tyrosine
accumulation of what toxic compound?
Phenylalanine
result?
impairment of tryptophan metabolism leading to serotonin deficiency
defective neurotransmission and white matter damage
26
metabolic disorder:
Maple syrup urine disease
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
none listed
accumulation of what toxic compound?
Leucine
result?
Leucine toxicity leading to cerebral edema
27
metabolic disorder:
mitochondrial disease
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
deficiency of ATP (energy) in affected tissues
accumulation of what toxic compound?
none listed
result?
Failure of affected tissues to carry out normal functions (ie: muscle weakness, failure of relaxation of blood vessel muscles)
failure of Cori cycle leading to lactate accumulation, cardiomyopathy
28
metabolic disorder:
peroxisomal disorders
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
defect in peroxisomal B-oxidation. deficiency of steroid hormones necessary for signaling
accumulation of what toxic compound?
saturated very long chain fatty acids
result?
Aberrant embryonic patterning and hormone deficiency, defects in maintenance of myelin and white matter
29
metabolic disorder:
Lysosomal storage disorders
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
none listed
accumulation of what toxic compound?
tissue specific accumulation of compound not metabolized by lysosome
result?
cell type specific damage and dysfunction as a result lysosomal failure and reaction to waste product buildup
30
metabolic disorder:
Disorders of creatinine biosynthesis
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
deficiency of cerebral creatine
accumulation of what toxic compound?
guanidoacetate in AGAT deficiency leads to seizures
result?
global brain energy defect leads to severe cognitive delays
31
metabolic disorder:
cholesterol biosynthesis disorders
you are deficient in?
accumulation of what toxic compound?
result?
you are deficient in?
steroid hormones
accumulation of what toxic compound?
none listed
result?
Endocrinopathies
disordered cellular signaling leading to aberrant organogenesis
32
2 disorders of ketone utilization mentioned by nelson in which severe ketosis may result frequently presenting in context of fasting, infection with fever or decreased intake secondary to vomiting and diarrhea with profound hypoglycemia. As ketone bodies accumulate, cyclic vomiting may ensue
Ketothiolase deficiency
Succinyl-CoA:3 ketoacid CoA transferase (SCOT) deficiency
33
common condition in which tolerance for fasting is impaired.
ketotic hypoglycemia
34
features of ketotic hypoglycemia
symptomatic hypoglycemia with seizures or coma occurs when child encounters catabolic stress.
Hypoglycemia with significant stress (viral infection with vomiting) less common following minor stress (longer than normal overnight fast)
first appears in 2nd year of life and occurs in otherwise healthy children
35
treatment of ketotic hypoglycemia
frequent snacks and glucose during stress
36
Ketonuria is normal to prolonged fasting in older infants and children, however if found in neonates, investigate for
metabolic disease
37
a high anion gap metabolic acidosis +/- ketosis suggests
a metabolic disorder
38
Genetic inheritance of IEMs
most are autosomal recessive
also x -linked
39
labs to order in suspected IEM
ABG
electrolytes -anion gap
glucose
ammonia
liver enzymes
CBC with diff
Lactate, pyruvate
organic acids
Acylcarnitines
carnitine
urine
Glucose
pH
ketones
reducing substances
organic acids
Acylglycines
orotic acid
40
in general carriers of AR or x-linked IEM are asymptomatic/symptomatic
asymptomatic
41
Most treatable metabolic disorders are on the newborn screen. Most states use what testing for this?
tandem mass spectrometry
42
Proposed additions or removal of conditions to newborn screen are evaluated by who?
federal advisory committee on heritable disorders in the newborn and child (ACHDNC)
43
when are newborns tested for newborn screen panel
24-48 hours
44
Neonatal screening has what purpose
early detection and rapid treatment of disorders before onset of symptoms to prevent morbidity and mortality.
designed to maximize detection of affected infants but it is not diagnostic
45
The plasma amino acid profile is most useful in identifying disorders of
amino acid catabolism
can also be helpful in disorders of organic acid degradation (often normal and not diagnostic)
46
The urine amino acid profile is helpful in diagnosing
primary and secondary orders of renal tubular function
disorders of amino acid transport
Not test of choice for amino acid or organic acid metabolism
47
Urine acylglycine profile and plasma acylcarnitine profile reflect
markers of disordered fatty acid oxidation
48
plasma free fatty acid to 3-OH butyrate suggests a
fatty acid oxidation disorder
49
Excess 3-OH butyrate suggests a disorder of
ketone metabolism
50
Absence of ketones or decreased 3-OH-butyrate suggests a
fatty acid oxidation disorder
51
treatment of IEM basic guidelines
1) toxic with encephalopathy - first goal is removal of toxic compound (hemodialysis, hemovenovenous filtration and administration of alternate pathway agents
2) enhance deficient enzyme activity
-administration of enzyme cofactors (pyridoxine in homocystinuria , tetrahydrobiopterin in PKU)
3) If deficiency of pathway product plays an important role, providing missing products is helpful (tyrosine in treatment of PKU)
4) decrease flux through the deficient pathway by restricting precursors in diet (ex: protein restriction in disorders of ammonia detox, Phenylalanine in PKU, and of amino acid precursors in the organic acid disorder)
52
odor of sweaty feet,
think _______ in metabolic acidosis etiologies caused by IEM in infants
isovaleric acidemia
53
many glycogen storages diseases are characterized by
hypoglycemia and
hepatomegaly
54
Glycogen (storage form of glucose) is found most abundantly where?
Liver - where it modulates blood glucose levels and in muslces where it facilitates anaerobic work
55
what glycogen storage disease type falls into this category?
diseases that predominantly affect the liver and have a direct influence on blood glucose
Types I VI, and VIII
56
what glycogen storage disease type falls into this category?
diseases that predominantly involve muscles and affect the ability to do anaerobic work
Types V and VII
57
what glycogen storage disease type falls into this category?
Diseases that can affect the liver and muscles and directly influence blood glucose and muscle metabolism
Type III
58
what glycogen storage disease type falls into this category?
Diseases that affect various tissues but have no direct effect on blood glucose or on the ability to do anaerobic work
types II and IV
59
The diagnosis of Glycogen storage disease type I or type III is suggested by what?
elevated uric acid, lactate and triglycerides in blood
confirmed by DNA testing
If DNA test is unavailable or inconclusive then enzyme measurements in tissue from affected organ confirm the dx
Lasty metabolic challenge and exercise testing
60
Treatment in Glycogen storage disease
aimed at maintaining satisfactory blood glucose levels or supplying alt energy sources to muscle.
In glucose-6-phospatase deficiency (type I) -> tx usually requires nocturnal intragastric feedings of glucose during the 1st 1-2 yrs of life. After snacks and uncooked cornstarch may be fine.
Hepatic tumors (sometimes malignant) are a threat in adolescence and adult life
No treatment for the diseases of muscle that impair skeletal muscle ischemic exercise.
Enzyme replacement early in life is effective in Pompe disease (type II) which involves cardiac and skeletal muscle
61
Galactosemia genetic
Autosomal Recessive
62
Galactosemia is an AR disease cause by deficiency of
Galactose-1-phosphate uridyltransferase
63
Clinical manifestations of Galactosemia
most striking in neonate who when fed milk generally exhibits evidence of liver failure
-hyperbilirubinemia
-disorders of coagulation
-hypoglycemia
disordered renal tubular function
-acidosis
-glycosuria
-aminoaciduria
cataracts
albuminuria
64
The neonatal screening test is imperative that it has a rapid turnaround time bc affected infants with Galactosemia may die how quickly
within the first week of life
65
Infants with Galactosemia are at increased risk for what type of sepsis
Severe neonatal Escherichia coli sepsis
66
When galactose is ingested as lactose in a galactosemia, what may be detectable
levels of plasma galactose
erythrocyte galactose 1-phosphate
elevated
67
How do you confirm the diagnosis of Galactosemia
DNA testing for pathogenic variants in galactose-1-phosphate uridyltransferase
68
Renal tubular dysfunction may be evidenced by what type of acidosis
Normal anion gap hyperchloremic metabolic acidosis
69
Treatment of galactosemia
eliminate dietary galactose
Infants who are extremely ill before treatment may die before the therapy is effective
70
Galactosemia complications longer term
first few years of life
-major effects on liver and kidney function
-development of cataracts
older children
-learning disabilities despite compliance
Girls
-develop premature ovarian failure despite treatment
71
Galactokinase deficiency genetics
Autosomal recessive disorder
72
Clinical manifestations of Galactokinase deficiency
-leads to accumulation of galactose in body fluids
Cataract formation
rarely for increased ICP
Homozygous - develop cataracts after neonatal period
Heterozygous - risk for cataracts as adults
73
Hereditary fructose intolerance
what happens?
treatment
leads to intracellular accumulation of fructose 1-phosphate
emesis
hypoglycemia
severe liver and kidney disease
eliminate fructose and sucrose from diet
74
Fructosuria is caused by
what happens
fructokinase deficiency
not associated with clinical consequences
75
These disorders are the result of the inability to catabolize specific amino acids derived from protein
Disorders of Amino Acid Metabolism
76
In disorders of amino acid metabolism, the amino acids accumulate in excess and are toxic to various organs such as
brain
eyes
skin
liver
77
Phenylketonuria (PKU) genetics
Autosomal recessive
78
PKU is the result from a defect in the hydroxylation of _______ to form______
phenylalanine
tyrosine
79
In PKU affected infants show what signs at birth and what happens after that?
normal at birth
untreated will cause severe Intellectual disability (IQ30) in the first year of life
80
PKU is found in what testing
dx?
screened in newborn screen
quantitative plasma amino acid analysis
Plasma phenylalanine >360 is consistent with dx of one of the hyperphenylalaninemias and requires prompt eval and tx
Plasma Phenylalanine >600 is classic PKU
premature infants and a few full term infants have transient elevations in phenylalanine
all hyperphenalaninemic infants should be tested for low tetrahydrobiopterin (cofactor for phenylalanine) -> measure dihydrobiopterin reductase in erythrocytes and anylyzing biopterin metabolites in urine
81
tx PKU
goal: maintain plasma phenylalanine in therapeutic range of 120-360 mM
using a diet specifically restricted in phenylalanine
Treatment for life is recommended to reduce risk of maternal PKU syndrome
tx with modified prep of tetrahydrobiopterin has shown good responses in some individuals with PKU
82
PKU outcome and education
Excellent
Most who are treated in the first 10 days of life achieve normal intelligence. Learning problems and problems with execute function are more frequent then unaffected peers.
Females must be educated on risks and prevention for maternal PKU (rigorous mgmt before conception and throughout pregnancy to prevent fetal brain damage, congenital heart disease and microcephaly)
83
how are tyrosinemias identified
neonatal screening program using tandem mass spectrometry to detect elevated tyrosine and/or succinylacetone
84
treatment of transient tyrosinemia of the newborn
ascorbic acid treatment
85
2 causes of elevated tyrosine levels screened on newborn screen
transient tyrosinemia of the newborn or severe liver disease
86
tyrosinemia type 1 is due to deficiency of
fumarylacetoacetate hydrolase
87
clinical signs tyrosinemia type 1 (it causes)
the accumulated metabolites produce severe liver disease associated with bleeding disorders
hypoglycemia
hypoalbuminemia
elevated transaminases
defects in renal tubular function
hepatocellular carcinoma may eventually occur
88
dx/confirmation of Tyrosinemia type 1
positive neonatal screening
diagnostic is an increased concentration of succinylacetone
DNA testing is available
89
Treatment of Tyrosinemia type 1
Nitisinone (NTBC - inhibitor of the oxidation of parahydroxyphenylpyruvic acid) whcih effectively eliminates the production of the toxic succinylacetone
diet-low phenylalanine, low tyrosine
90
which tyrosenemias are worse, which are more benign
type I worse
type II and III are more benign - blocked metabolism of tyrosine at earlier step is responsible so succinylacetone is not produced
91
clinical features of Tyrosinemias II and III
Hyperkeratosis of palms and soles
Keratitis - can cause severe visual disturbances
significant elevations of tyrosine levels associated with mild cognitive impairment and specific defects in executive function
92
treatment of Tyrosenemias II and III
phenylalanine and tyrosine restricted diet
93
genetics for Homocystinuria
Autosomal Recessive
94
Homocystinuria is an AR disorder caused by a deficiency of
cystathionine B-synthase
(when this is deficient, homocysteine accumulates in the blood and appears in the urine, also causes raise in Methionine)
95
detection of Homocystinuria
newborn screen (looks for elevated methionine in blood)
96
Homocystinuria clinical symptoms
dislocated ocular lenses
long, slender extremities
malar flushing
livedo reticularis
Arachnodactyly
scoliosis
pectus excavatum or carinatum
genu valgum
ID
psychiatric illness
major arterial or venus thromboses are a constant threat
97
confirmation of Homocystinuria
demonstration of elevated total homocysteine in the blood
plasma acid profile reveals Hypermethioninemia
Numerous pathogenic variants in the gene are known and can be tested
98
treatment of Homocystinuria
there are two forms
treatment for one is
large doses of pyridoxine (100-500mg/day)
Folate supplementation for common folate deficiency (trapped in remethylation of homocysteine to methionine) - this form is more likely to be missed on newborn screen
second form not always responsive to above
-betaine (trimethylglycine)
-some may benefit from Folate and B12 supplementation
diet also can be used to control
99
which form of homocystinuria is more likely to be missed on newborn screen bc methionine concentrations are not always above the screening cutoff
Pyridoxine-responsive Homocystinuria
100
genetics for Maple syrup urine disease (MSUD)
Autosomal Recessive
101
Maple syrup urine disease is also called
Branched chain ketoaciduria
102
Maple syrup urine disease (MSUD) is caused by a deficiency of
the decarboxylase that initiates the degradation of the ketoacid analogs of the 3 branched chain amino acids (leucine, isoleucine and valine)
103
onset of Maple syrup urine disease
classic at birth
intermittent onset and late onset forms
classic form occurs typically 1-4 weeks of birth
104
clinical s/s Maple syrup urine disease
Poor feeding
vomiting
tachypnea
hallmark
profound depression of the CNS
alternating hypotonia and hypertonia (extensor spasms)
Opisthotonos
seizures
urine may have the odor of maple syrup
labs
hypoglycemia
metabolic acidosis
positive urine ketones
no or low B-hydroxybutyrate
rapid formation of copious white precipitate when 2,4 dinitrophenylhydrazine is added to urine sample
105
definitive diagnosis of maple syrup urine disease
large increases in plasma leucine with less increases in isoleucine and valine concentrations
identification of excess alloisoleucine in the plasma
abnormal urinary organic acid profile showing the ketoacid derivatives of the branched chain amino acids
pathogenic variants in one of 3 genes
BCKDHA
BCKDHB
DBT
106
management of Maple syrup urine disease
adequate calories and protein with restriction of Leucine
catabolic stresses such as moderate infections or labor and delivery in pregnant mother with MSUD can precipitate crisis
most feared complication of metabolic decompensation is brain edema
Liver transplant effectively treats MSUD
107
Genetics for OTC (ornithine carbamoyltransferase) deficiency
x linked
variants range from whole gene deletions to single nucleotide substitutions
If no OTC activity (enzyme nonfunctional) in affected males -> likely to die in neonatal period
Affected females are heterozygous - may become symptomatic at any time in life
108
Clinical manifestations of OTC deficiency
lethal disease in males (coma, encephalopathy)
clinically normal in females
late onset forms in males also occur
manifestations in clinically affected females
-recurrent emesis
lethargy
seizures
developmental delay
psychosis
episodic confusion
-may spontaneously limit their protein intake
109
confirmation of OTC deficiency
plasma amino acid profile - may sho reduced citrulline and arginine concentrations with increased glutamate and alanine
urine organic acid profile may show increased excretion of orotic acid after protein loading or with concurrent administration of allopurinol
known pathogenic variant testing, deletion testing and sequencing of the entire coding region of the OTC gene are available
110
ASL deficiency genetics
autosomal recessive
111
detection of ASL
newborn screen - elevated citrulline
confirmed by detection of elevated argininosuccinic acid in the urine
112
treatment of hyperammonemia
protein intake reduced
IV glucose to suppress catabolism of endogenous protein
to eliminate ammonia:
Sodium benzoate
Sodium phenylacetate
Arginine is usually deficient so given
refractory treatment
hemodialysis or hemofiltration
(not peritoneal dialysis)
monitor for cerebral edema
crystalline essential amino acids can support protein synthesis
maintenance treatment with phenylbutyrate prevents accumulation of ammonia
113
a disorder of renal tubular transport of cystine, lysine, arginine and ornithine
Cystinuria
114
diagnosis of Cystinuria
pattern of amino acid excretion in the urine
DNA testing
115
Treatment of Cystinuria
Based on increasing the solubility of cystine by complexing it with compounds such as penicillamine
116
Intestinal transport of tryptophan is impaired in this syndrome.
Pellagra like symptoms result from this deficiency.
Diagnosis is based on amino acid pattern in urine
Treatment with tryptophan improves outcomes
Hartnup Syndrome
117
in the pain mgmt lecture she talks about pre-education of pt to set expectations. What will she tell families on why they may use pills to control pain over morphine pca
lasts longer
118
pain that arises from actual or threatened damage to non-neural tissue and is due to the activation of nociceptors
Nociceptive pain
example is an incisional cut
119
type of nociceptive pain that is caused by the activation of nociceptors in either surface tissues (skin, mucosa of mouth, nose, urethra, anus, ect.) or deep tissues such as bone, joint, muscle or connective tissue
somatic pain
120
type of nociceptive pain that is caused by the activation of nociceptors located in the viscera (the internal organs of the body that are enclosed within a cavity, such as thoracic and abdominal organs)
Visceral pain
121
which type of nociceptive pain is more difficult to treat
visceral pain
122
pain pathway
1) sensory endings in skin
2) action potential in sensory axion
3) sensory axon enters the spinal cord and synapses with the brain
4) sensory pathway continues with second neuron projecting to the thalamus
5) Sensory pathway reaches the cerebral cortex for conscious perception
6) An upper motor neuron from the cortex executes a motor command
7) The upper motor neuron contacts a lower motor neuron in the spinal cord
8) The lower motor neuron causes contraction of the target skeletal muscle
123
characteristics of acute pain
clear onset
relatively clear offset
contextual factors are less relevant
"protective" - meaning going to get better
124
characteristics of recurrent pain
clear onset
relatively clear offset
Episodes of pain recur
contextual factors more relevant
125
Vaso-occlusive pain in sickle cell is categorized as what type of pain
Recurrent pain
126
Headache or migraine is categorized as what type of pain
Recurrent pain
127
cancer pain is categorized as what type of pain
ongoing pain
128
characteristics of ongoing pain
variability specificity of onset
no clear offset
contextual factors more relevant
129
what category of pain?
characteristics are...
variability specificity of onset
no clear offset
contextual factors more relevant
ongoing pain
130
what category of pain?
characteristics are...
clear onset
relatively clear offset
Episodes of pain recur
contextual factors more relevant
recurrent pain
131
what category of pain?
characteristics are...
clear onset
relatively clear offset
contextual factors are less relevant
"protective" - meaning going to get better
Acute pain
132
what category of pain?
characteristics are...
no clear onset
no offset
contextual factors extremely relevant
133
characteristics of chronic pain
no clear onset
no offset
contextual factors extremely relevant
134
goals of chronic pain
eradication to the degree possible
improve function
135
pain caused by a lesion or disease of the somatosensory nervous system
Neuropathic pain
136
how do most people describe neuropathic pain
sharp, burning, fire, electric pain
137
what type of pain medicine is used to control neuropathic pain
neuromodulators
138
what is allodynia?
lowered threshold to pain. light touch is perceived as very painful
stimulus and response mode differ
139
What is hyperalgesia
increased response over time
stimulus and response mode are the same
140
what is Hyperpathia
raised threshold: increased response
stimulus and response mode may be the same or different
141
what is hypoalgesia
raised threshold: lowered response
stimulus and response mode are the same
142
pain characterized as a temporary increase in severity of pain over and above the pre-existing baseline pain level
Breakthrough pain
143
pain that results when the blood level of the medicine falls below the minimum effective analgesic level towards the end of dosing interval
End of dose pain
144
World Health Organization (WHO) pain ladder
Mild pain treatment recommendations
Nonopiod +/- adjuvant therapy
145
World Health Organization (WHO) pain ladder
Mild to moderate pain treatment recommendations
"Weak" opioid or multimodal +/- nonopioid +/- adjuvant therapy
146
World Health Organization (WHO) pain ladder
Moderate to Severe Pain
treatment recommendations
"strong" opioid
+/- nonopioid +/- adjuvant therapy
147
World Health Organization (WHO) pain ladder
Severe to very severe pain treatment
Interventional treatments +/- nonopioid +/- adjuvant therapy
148
delivers low voltage electrical current through the skin. Activates endogenous descending inhibitory pathways
Transcutaneous electrical nerve stimulation (TENS)
149
directions for Lidoderm patch (Lidocaine 5%)
for 12 hours on and off
cannot be combined with heating pads
you can get OTC up to 4%
150
5As of opioid assessment
Analgesia
Adverse side effects
Activity
Aberrant behaviors ( are they going to ER, missing school, requesting refills early)
Affect (poor mood, and still not improving function)
151
opioid conversion for Morphine....ie...they are on 10mg IV morphine dose...what would PO be
3:1
30mg
152
Opioid conversions for IV to PO
Morphine and Hydromorphone is 3:1
Oxycodone is 2: 1
Methadone is 1:1
Tramadol is 1.2:1
153
Codeine use in kids
dont use unless know their polymorphisms are
contraindicated in pediatric pt <12 and in <18yrs following tonsillectomy or adenoidectomy
CYP2D6 causes poor metabolism of the drug
If they are a ultra rapid metabolizer, this can lead to overdose
154
a state of nociceptive sensitization caused by exposure to opioids
Opioid Induced Hyperalgesia
155
Treatment for Opioid induced hyperalgesia
Dose increase to rule out tolerance (if this is opioid induced hyperalgesia, this will make the pain worse)
Dose decrease
try other agents such as: Ketamine and methadone, clonidine, precedex
156
how much to prescribe for opioids
opioids should be prescribed in amounts to cover less than 2 weeks or 2 months at most
157
screening tool to assess opioid abuse risk
CRAFFT screening tool
158
opioid combos to beware of for abuse
Hydrocodone/acetaminophen(Norco, Vicodin, Lortab)
Oxycodone/acetaminophen (Percocet)
159
According to the Texas Prescription Monitoring Program, all Texas licensed pharmacies are required to report all dispensed controlled substances records to the Texas Prescription monitoring program (PMP) no later than the next business day after prescription is filled. This applies to what?
All pharmacists and prescribers (other than veternarians) are required to check the patient's PMP history at what point?
schedule II, III, IV, and V controlled substances
before dispensing or prescribing opioids, Benzodiazepines, barbiturates or carisoprodol
160
what level of sedation?
Purposeful response to verbal or tactile stimulation
no airway intervention required
Adequate spontaneous ventilation
cardiovascular function usually maintained
Moderate sedation/analgesia
161
what level of sedation?
Normal responsiveness
unaffected airway
unaffected spontaneous ventilation
Unaffected cardiovascular function
minimal sedation
162
what level of sedation
Purposeful response after repeated or painful stimulation
Airway intervention may be required
Spontaneous ventilation may be adequate
cardiovascular function usually maintained
Deep sedation/analgesia
163
what level of sedation?
Unarousable even with painful stimulus
airway intervention often required
spontaneous ventilation frequently inadequate
cardiovascular function may be impaired
General anesthesia
164
loss of sensation of pain, usually from following administration of a medication, that results from an interruption in the nervous system pathway between sense organ and brain
analgesia
165
Benzodiazepines Mechanism of action
acts centrally and activates GABAa neuronal receptors in the brain
-acts as agonists in the brain to cause and inhibitory effect
166
Benzodiazepines therapeutic effects
induce anterograde amnesia
anticonvulsant properties
muscle relaxant properties
167
Benzodiazepines adverse effects
over sedation or prolonged sedation
hypotension
Paradoxical excitation (agitation and delirium)
resp depression
physiologic dependence
168
sedation dosing for Midazolam continuous infusion
0.01-0.05mg/kg/hr to start
up to 0.3mg/kg/hr
169
sedation dosing for Lorazepam intermittent dosing
0.05-0.1mg/kg/dose every 4-6 hours
170
IV intermittent dosing and cont infusion dosing for Propofol in ICU sedation .also for amnesia
IV intermittent: 1-2mg/kg/dose
contin: 5-50mcg/kg/min
171
dosing for sedation using Dexmedetomidate cont infusion
0.2-0.7 mcg/kg/hr up to 1mcg/kg/hr
172
explain state behavioral scale
sedation assessment in young and sedated patients on mechanical ventilation
-3 unresponsive
-2 responsive to noxious stimuli
-1 responsive to gentle touch or voice
0 - awake and able to calm
+1 restless and difficult to calm
+2 Agitated
in ICU we usually aim or 0 to -1, sometimes -2. Rare to aim for -3 in ICU
173
most valid and reliable sedation assessment tools for measuring quality and depth of sedation in ICU patients (on adult slide in pain and sedation in ICU)
Richmond Agitation Sedation Scale (RASS)
Sedation-Agitation Score (SAS)
174
explain Richmond Agitation Sedation Scale (RASS)
+4 Combative (violent, immediate danger to staff)
+3 Very agitated (pulls or removes tubes or catheters, aggressive)
+2 Agitated (Frequent non purposeful movement, fights ventilator)
+1 Restless (Anxious, apprehensive, but movements not aggressive or vigorous)
0 - Alert and calm
-1 Drowsy (not fully alert but has sustained awakening to voice (eye opening and contact >=10 sec)
-2 light sedation (briefly awakens to voice (but no eye contact)
-3 moderate sedation (movement or eye opening to voice (but no eye contact)
-4 deep sedation (no response to voice, but movements or eye opening to physical stimulation
-5 unarousable (no response to voice or physical stimulation)
175
what class of med?
act selectively on neuron that transmit and modulate nociception, leaving other sensory modalities and motor fx intact
Opioid analgesics
176
what is more potent?
morphine or Fentanyl
Fentanyl is 100xs more potent than morphine
177
dosing for continuous infusion of Fentanyl
0.5-4mcg/kg/hr
178
what opioid analgesic is good to use in renal dysfunction
Hydromorphone
no active metabolites
179
what does FLACC stand for
pain scale
Face
Legs
Activity
Cry
consolability
0-2 for each category
0-3 mild
4-7 moderate pain
7-10 score is severe pain
180
best method for pain monitoring
Ask the patient/self report
181
Behavioral pain scale
evaluating
Facial expression
Upper limb movements
Compliance with mechanical ventilation
Each category is 1-4 points
Facial expression
relaxed -1
partially tightened (brow lowering) - 2
Fully tightened (eyelid closing) - 3
Grimacing - 4
no upper limb movements -1
partially bent upper limb - 2
full bent w/finger flexion - 3
permanently retracted -4
tolerating movement (compliance with mechanical vent) - 1
Coughing but tolerating most of the time - 2
fighting ventilator - 3
unable to control ventilation - 4
182
restore trial
objective
result
Determine if critically ill children managed with a nurse implemented, goal directed sedation protocol experienced fewer days of mechanical ventilation than usual care
Although protocolized sedation did not decrease the duration of mechanical ventilation, it was not harmful to pt and allowed them to have decreased exposure to opioid and sedative medications as well as spend more days awake and calm
183
Basic principles of CVICU sedation
1) assess SBS according to ordered goal
2) use rescue bolus doses to maintain SBS goal PRN every 20 min with re-eval of SBS after each bolus
3) Bolus dose should = 1 hr of infusion (ie) fentanyl gtt 2mcg/kg/hr so bolus should be Fentanyl 2 mcg/kg)
4) if use 2-3 rescue boluses every 10 min within the same hour, then provider called to have infusion rate increased
5) Goal is to have better steady state
6) Dedicated line (PIV or CVL for infusions recommended to encourage bolus from the pump
184
Neonate <30 days sedation 1st line and second line
try to avoid
non pharm first of course
Fentanyl
Dexmedetomidine as adjunct therapy
try to avoid Benzos but can be used - shown to affect neurodevelopmental outcomes
185
Age 30 days to 15 years sedation pathway fist line, second
Start with Fentanyl and Dexmedetomidine
with incremental increases
-Fentanyl increase by 0.5mcg/kg/hr
-Dex increase by 0.25 mg/kg/hr
Bolus Fentanyl
second line - Midazolam Bolus for sedation
Dont bolus Dex
-Replace Dex with Midazolam for those not tolerating Dex
186
Age > 15 yrs or >50 kg sedation pathway first line, second line
not weight based, infusions dose per hour (except Dex)
Starts with Fentanyl and Dexmedetomidine
with incremental increases
-Fentanyl by 10mcg/hr
- Dex by 0.25 mg/kg/hr
Bolus dose Fentanyl
Bolus Midaz for sedation
dont bolus the Dex
187
what is PTSD from their ICU experience
Post-Intensive Care Syndrome (PICS) seen in >50% of critical illness survivors
188
Midazolam exposure is associated with alterations in _______ development and _____
hippocampal development
poorer neurodevelopmental outcomes
(can affect brain development)
189
new or increased physical, cognitive or mental health impairment in a pt after hospitalization in a critical care unit
Post intensive care syndrome (PICS)
190
Children suffering from Post intensive care syndrome (PICS) may experience
muscle weakness
delayed developmental milestones
learning problems
sadness
191
a group of symptoms that can occur upon abrupt discontinuation or decreased intake of certain medications due to medical treatment
iatrogenic withdrawal
192
risk factors for iatrogenic withdrawal syndrome
Higher pre-weaning mean daily opioid dose
Longer duration of sedation >5 days
-low risk <5 days
-moderate risk 5-14 days
-high risk 14-21 days
-very high risk >21 days
receipt of 3+ pre-weaning sedative classes
193
classic signs of opioid withdrawal
neurological excitability
GI dysfunction (Vomiting, loose stool)
autonomic instability (sweating, elevated temp)
Poor organization of sleep state
194
Benzodiazepine withdrawal symtpoms
Agitation
visual hallucinations
facial grimacing
small amplitude choreic or choreoathetoid movements
Seizures
195
Dexmedetomidine withdrawal symptoms
Agitation
irritability
headache
rebound hypertension
196
Dexmedetomidine withdrawal is most likely to be seen when greater than _____ of therapy have been received
>24 hours
197
what is a tool to assess withdrawal?
Withdrawal Assessment Tool-1 (WAT-1)
198
WAT-1 consists of
a review of the patients record for the past 12 hours
direct observation of the patient for 2 min
patient assessment using a progressive stimulus
routinely performed to assess level of consciousness
assess of post stimulus recovery
199
sedation wean principle for time to wean
duration of wean should be less time than the patient received indicated medication
200
evaluation before sedation
Evaluate injury/condition and urgency of procedure needing to be performed
obtain a comprehensive history
Determine an American Society of Anesthesiologist (ASA) physical status classification
Choose anticipated category/level of sedation
Assess the timing and nature of oral intake
-optimal to be NPO if can wait for moderate and above
-clear liquid - 1 hour min
-breastmilk - 4 hours
infant formula or non-human meal - 6 hours
light meal - 6 hours
heavy meal - 8 hours
201
American Society of Anesthesiologist (ASA) physical status classification
ASA I A normal healthy patient - excellent suitability for sedation
ASA II A patient with mild systemic disease - generally good suitability for sedation
ASA III A patient with severe systemic disease - Intermediate to poor suitability for sedation; consider benefits to relative risks
ASA IV A patient with severe systemic disease that is a constant threat to life. Poor suitability for sedation; benefits rarely outweigh risks
ASA V A moribund patient who is not expected to survive without the operation, extremely poor suitability for sedation
ASA VI A declared brain-dead patient whose organs are being removed for donor purposes
202
NPO guidelines for sedation
-clear liquid - 1 hour min (lecture, Lippincott has 2 hours)
-breastmilk - 4 hours
infant formula or non-human milk - 6 hours
light meal - 6 hours (lecture)
heavy meal - 8 hours
Lippincott has 8 hours for all solid food
203
developmental understanding of pain at 2-7 yrs old
preoperational thought
-relates pain primarily as physical, concrete experience
-Thinks in terms of magical disappearance of pain
-may view pain as punishment for wrongdoing
-Tends to hold someone accountable for own pain and my strike out at person
204
what developmental understanding of pain
age?
-relates pain primarily as physical, concrete experience
-Thinks in terms of magical disappearance of pain
-may view pain as punishment for wrongdoing
-Tends to hold someone accountable for own pain and my strike out at person
preoperational thought
2-7 yrs old
205
What is the developmental understanding of pain at 7-12 yrs old
Concrete operational thought
Relates pain physically (ie: headache, stomachache)
is able to perceive psychosocial pain (ie: someone dying)
Fears bodily harm and annihilation (body destruction and death)
206
what developmental understanding of pain?
age?
Concrete operational thought
Relates pain physically (ie: headache, stomachache)
is able to perceive psychosocial pain (ie: someone dying)
Fears bodily harm and annihilation (body destruction and death)
Concrete operational thought
7-12 yrs old
207
what developmental understanding of pain?
age?
Is able to give reason for pain (ie fell and hit nerve)
perceives several types of psychological pain
has limited life experiences to cope with pain as adult might cope despite mature understanding of pain
fears losing control during painful experience
Formal operational thought
>=12 yrs
208
what is the developmental understanding of pain >=12 yrs old
Formal operational thought
Is able to give reason for pain (ie fell and hit nerve)
perceives several types of psychological pain
has limited life experiences to cope with pain as adult might cope despite mature understanding of pain
fears losing control during painful experience
209
techniques for non-pharm intervention in pain mgmt
Infants (0-12 months)
Parents voice (talking, singing on tape)
touching (holding, rocking)
pacifier
music
swaddling
massage
210
techniques for non-pharm intervention in pain mgmt
toddlers (12-36 months)
same as infant +
Pinwheels
storytelling
peek-a-boo
busy box
infant: Parents voice (talking, singing on tape)
touching (holding, rocking)
pacifier
music
swaddling
massage
211
techniques for non-pharm intervention in pain mgmt
Preschoolers 3-5 years
pinwheels
party blowers
feathers
pop up books
storytelling
comfort item
music
singing
manipulatives
212
techniques for non-pharm intervention in pain mgmt
School age 6-12 yrs
electronic toys (nintendo DS, psp, iopd)
pop up books
i spy books
participation in procedure
imagery
storytelling
breathing techniques
muscle relaxation
213
techniques for non-pharm intervention in pain mgmt
adolescents 13-18 yrs
music
comedy tapes
imagery
massage
muscle relaxation
TV
video
other electronics
214
cognitive behavioral interventions
non pharm
distraction
relaxation
guided imagery
thought stopping - like I know it hurts but this wont last long
behavioral contracting - Beads of courage example
215
type of pain
pain due to the abnormal presence of, or inappropriate activation of, abnormal pain pathways within the nervous system.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1168-1169). Wolters Kluwer Health. Kindle Edition.
functional
216
any pain (e.g., nociceptive, neuropathic, or functional) that lasts longer than 1 month is considered
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1169). Wolters Kluwer Health. Kindle Edition.
chronic pain
217
topical anesthetic should allow ___ -____ min for skin penetration with a duration effect < ___ hrs
30-60 min
<4 hours
218
when using topical lidocaine, monitor for ______ if used over significant body surface area
toxicity
219
intradermal onset of action is ___ to ____ min with duration ____
1-2 min
duration <1 hr
220
state of sedation that provides anxiolysis
minimal sedation
221
Midazolam is used for what type of sedations
minimal
moderate
222
Midazolam IV in sedation
IM
Intranasal
PO
dose
onset
duration
IV
0.05-0.1mg/kg IV
onset 2-3 min
duration 1-2 hr
IM
0.1-0.15mg/kg
onset 5-15 min
duration 2-6 hrs
use the 5mg/ml concentration
intranasal
O.2-0.3mg/kg
onset 4-8 min
duration 20-30 min
use the 5mg/ml concentration
PO
0.5-0.75mg/kg
5-15 min
30 min-1 hr
max dose 20mg
223
type of sedation
drug-induced depressed consciousness, but patient is able to respond purposefully to verbal commands or light physical stimulation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1170). Wolters Kluwer Health. Kindle Edition.
moderate sedation
224
lorazepam used in what type of sedation
minimal
225
Dose
onset
duration
Lorazepam IV in minimal sedation
0.01-0.05mg/kg
onset 15-20 min
duration 6-8 hr
reduce when used in combo with opioids
use with caution in renal/liver impairment pts
226
type of sedation?
drug-induced depressed consciousness from which patient is not easily aroused, and has partial or complete
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1170). Wolters Kluwer Health. Kindle Edition.
deep sedation
227
type of sedation?
drug-induced loss of consciousness; patient is not arousable even to painful stimuli.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1170). Wolters Kluwer Health. Kindle Edition.
General anesthesia
228
propofol is used in what type of sedations
moderate
deep
229
Propofol dosing in sedations
onset
duration
IV Bolus - 0.5-1mg/kg
onset 15-45 sec
duration 3-10 min
IV continuous
50-200 mcg/kg/min
max 200 mcg/kg/min
onset 15-45 sec
duration 3-10 min
230
if using ketamine for moderate or deep sedation recommendations to use 0.5mg/kg boluses of ____
Propofol
231
Avoid _Propofol for sedation in patients with _____ or _____ allergy
egg
soy
232
can a nurse administer propofol in Texas
no
233
Dexmedetomidine is used in what level of sedations
minimal
moderate
234
Dexmedetomidine
IV bolus
IV continuous
Intranasal
dose
onset
duration
IV Bolus
1-3 mcg/kg
onset 2-3 min
duration 1-2 hrs
give over 5 min
IV continuous
1 mcg/kg/hr (max 2 mcg/kg/hr)
onset 2-3 min
duration 60-12 min
continuous may be restricted at various institutions
IN
1-4 mcg/kg
15-60 min
60-90 min
max dose 200 mcg (100 mcg per nare)
235
Pentobarbital is used in what type of sedations
moderate
236
Pentobarbital
dose
onset
duration
IV
PO
IV 1-3 mg/kg
(max 6mg/kg, 200mg)
onset 1-5 min
duration 15-45 min
PO 4mg/kg
(max 6mg/kg, 200mg)
onset 20 min
duration 30 min-90 min
237
When is etomidate need to be avoided
if sepsis is suspected
238
AMPLE history
Allergies
Medications: current, previous sedation/anesthetic history
Past medical history: Gen health, risk factors for sedation, airway problems, hepatic or renal dysfunction
Last meal: NPO status, volume status
Events leading up to scenario: why are they being sedated
239
Classifies the size of the tongue by opening the mouth as wide as possible and examining the oropharynx to determine the degree of visualization of uvula and tonsillar pillars.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1177). Wolters Kluwer Health. Kindle Edition.
Mallampati Classification
240
used in combination with other screening tools to evaluate airway for degree of intubation difficulty.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1177). Wolters Kluwer Health. Kindle Edition.
Mallampati Classification
241
Mallampati class I
242
Mallampati Class II
243
Mallampati class III
244
Mallampati class IV
245
categorizes patients based on general health status to determine suitability for sedation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1178). Wolters Kluwer Health. Kindle Edition.
ASA physical status classification
246
_____________ consult recommended if the patient has an ASA classification of 3 or has significant risk factors that may result in airway or hemodynamic compromise with the administration of moderate sedation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1178). Wolters Kluwer Health. Kindle Edition.
Anesthesiologist
247
oral sucrose is safe and effective in reducing signs of distress associated with minor, painful procedures in what ages
neonates
and moderately effective in infants up to 6 months of age
248
Single agents are often used for diagnostic procedures; however, moderate sedation for therapeutic procedures is often best achieved with a combo of
Benzodiazepine and an opioid
Lippincott
249
discharge criterion after sedation
monitor until pt fully recovered and sedation risks no longer exist
No evidence based set of clinical indicators exist for safe discharge following procedural sedation, but general criteria include stable vital signs, pain well controlled, return to baseline LOC, n/v controlled
and pt adequately tolerating oral intake to maintain hydration
250
regional anesthetic technique to provide analgesia below the umbilicus when severe pain is expected only for 24 hours post op
Caudal block
251
How to treat resp depression in sedation/analgesia
* Decrease PCA dosing.
* Increase the lockout interval.
* Decrease/discontinue the basal rate if present.
* Low-dose naloxone 2–10 µg/kg/dose IV.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1181). Wolters Kluwer Health. Kindle Edition.
252
How to treat apnea in sedation/analgesia
* Assist ventilation as needed.
* Stop infusion.
* Reverse the opioid with naloxone 0.1 mg/kg/dose IV (maximum dose: 2 mg).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1181). Wolters Kluwer Health. Kindle Edition.
253
How to treat Pruritis in sedation/analgesia
* Administer antihistamine (i.e., diphenhydramine).
* Consider low-dose naloxone 2–10 μg/kg/dose IV.
* Consider changing the opioid (e.g., hydromorphone causes a lesser degree of pruritus than morphine).
* Consider adding an NSAID.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1181-1182). Wolters Kluwer Health. Kindle Edition.
254
How to treat Nausea/Vomiting in sedation and analgesia
* Administer antiemetic (e.g., ondansetron). * Consider changing the opioid or decreasing the dose and adding an NSAID.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1182). Wolters Kluwer Health. Kindle Edition.
255
How to treat constipation in sedation and analgesia
* Stool softener and stimulant daily and adjusted as needed (e.g., docusate plus senna or polyethylene glycol).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1182). Wolters Kluwer Health. Kindle Edition.
256
How to treat urinary retention in sedation and analgesia
* Common side effect with epidural opioid infusion.
* Consider decreasing opioid dosage.
* Bladder catheterization.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1182). Wolters Kluwer Health. Kindle Edition.
257
state of apprehension that develops in response to stress and includes physiologic, behavioral, emotional responses.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1183-1184). Wolters Kluwer Health. Kindle Edition.
anxiety
258
an exaggerated physical, physiologic, behavioral, and emotional state involving excessive, often nonpurposeful motor activity.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1184). Wolters Kluwer Health. Kindle Edition.
Agitation
259
state of acute brain dysfunction characterized by four features: 1) inattention and disturbance in consciousness, 2) change in cognition (e.g., memory deficits, disorientation, language disturbances), 3) acute onset, and 4) fluctuating course. Symptoms may include agitation, lethargy, or confusion.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1184). Wolters Kluwer Health. Kindle Edition.
Delirium
260
Delirium is characterized by what 4 features
1) inattention and disturbance in consciousness,
2) change in cognition (e.g., memory deficits, disorientation, language disturbances),
3) acute onset, and
4) fluctuating course. Symptoms may include agitation, lethargy, or confusion.
261
risk factors in sedation
1) predisposing factors: psychiatric disease, genetic disposition, chronic neurologic disorders; 2) precipitating factors: sleep deprivation, oversedation, poorly controlled pain, immobilization, electrolyte imbalances, and infection.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1184). Wolters Kluwer Health. Kindle Edition.
262
Sedation assessment tools mentioned in Lippincott
Ramsey Sedation Scale
Comfort Scale
State Behavioral scale
263
is not easily recognized in children and can be mistaken for behavioral issues or oversedation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1184). Wolters Kluwer Health. Kindle Edition.
Delirium
264
Delirium tools are still being developed. Currently what is most commonly used
Pediatric Confusion Assessment Method
265
what drug class
short-acting, anxiolytic, amnestic, hypnotic, skeletal muscle relaxant, with no analgesic activity, useful to decrease anxiety associated with procedure.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1184). Wolters Kluwer Health. Kindle Edition.
Benzodiazepine
ie: Midazolam
266
what drug class?
long-acting sedative than benzodiazepine, useful for sedation prior to diagnostic imaging.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1184-1185). Wolters Kluwer Health. Kindle Edition.
Barbiturate
ie) pentobarbital
267
what drug class?
a selective α2 agonist with potent sedative and analgesic properties; use is increasing but must be administered as a continuous infusion.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1185). Wolters Kluwer Health. Kindle Edition.
Selective A2 Agonist
ie) Dexmedetomidine
268
drug class?
For antegrade amnesia and sedation in combination with an opioid.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1185). Wolters Kluwer Health. Kindle Edition.
Benzodiazepine
269
drug?
Dissociative anesthetic that has analgesic, amnesic, and sedative properties, useful for sedation for painful procedure.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1185). Wolters Kluwer Health. Kindle Edition.
Ketamine
270
drug ?
Rapidly acting sedative with no analgesic activity, useful for urgent procedures (e.g., intubation).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1185). Wolters Kluwer Health. Kindle Edition.
Etomidate
271
drug?
a general anesthetic agent that may be used for painful procedural sedation in the intensive care setting or under the guidance of an anesthesiologist/anesthesia provider.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1185). Wolters Kluwer Health. Kindle Edition.
Propofol
272
what are some nonpharm ways to manage delirium
Promote normal circadian rhythm—normal sleep, consistent orientation, family involvement, adherence to patient routines, minimal restraints when possible and early rehabilitation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1186). Wolters Kluwer Health. Kindle Edition.
273
a decrease in a drug’s effect over time or the need to increase the dose to achieve the same effect.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1187). Wolters Kluwer Health. Kindle Edition.
Tolerance
274
a decrease in a drug’s effect over time or the need to increase the dose to achieve the same effect.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1187). Wolters Kluwer Health. Kindle Edition.
Dependence
275
a constellation of physical symptoms that occurs when an opioid or benzodiazepine is abruptly discontinued in a patient who has developed tolerance.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1187). Wolters Kluwer Health. Kindle Edition.
Withdrawal
276
Withdrawal is seen in 100% of pediatric patients receiving ___ or _______ for > 9 days and will require a slow sedation taper.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1187). Wolters Kluwer Health. Kindle Edition.
opioids
benzodiazepines
277
withdrawal assessment tools
<1 month - neonatal assessment scale
>1 month - WAT 1
278
* Patients who have received opioids or other sedative agents for 5 days, benefit from switching from IV agents to
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1188). Wolters Kluwer Health. Kindle Edition.
ATC long-acting oral agents.
279
The most common conversions of IV to oral agents are fentanyl to ______ and midazolam to _________.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1188). Wolters Kluwer Health. Kindle Edition.
methadone
diazepam
280
If symptoms of withdrawal are observed during weaning -
IV dose of rescue morphine (0.05-0.1mg/kg) or lorazepam (0.05-0.1mg/kg) given for opioid or benzodiazepine withdrawal
281
The most common adjunctive therapy to smooth sedation titration and minimize withdrawal is the use of _______
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1188). Wolters Kluwer Health. Kindle Edition.
Clonidine is an α2-adrenergic medication that is available orally or as a transdermal patch. * PO starting dose ranges from 3 to 5 µg/kg/day. * Transdermal patch is available in 100 µg and 200 µg concentrations, with peak effect reached in 72 hours and remains potent for a total of 7 days.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1188). Wolters Kluwer Health. Kindle Edition.
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Adverse effects of Clonidine
sedation
bradycardia
hypotension
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Depolarizing or agonist neuromuscular blocking agent (NMBA) produces muscle paralysis through binding with acetylcholine receptor, allowing for muscle contraction and then paralysis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1188-1189). Wolters Kluwer Health. Kindle Edition.
Neuromuscular blocking agents
