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Critical Care 2 > Exam 3, deck 3 > Flashcards

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

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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

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3
Q

metabolic disorders should be considered in all neonates presenting with

A

lethargy

poor tone

poor feeding

hypothermia

irritability

seizures

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4
Q

metabolic disorders should be evaluated by what 3 specific labs

A

plasma ammonia

blood glucose

anion gap

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5
Q

Significant ketosis in neonate is unusual and suggests what type of disorder

A

organic acid disorder

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6
Q

The introduction of fructose or sucrose in diet may lead to decompensation in

A

hereditary fructose intolerance

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7
Q

In older children, increased protein intake may unmask disorders of

A

ammonia detoxification

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8
Q

The toxic metabolic compensation often presents as ________, may be precipitated by

A

encephalopathy

fever
infection
fasting
or other catabolic stresses

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9
Q

If an infant or child presents with features of toxic encephalopathy, what metabolic complication should be on your differential

A

Hyperammonemia

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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

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11
Q

Moderate neonatal hyperammonemia range

A

200-400 umol/L

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12
Q

Severe neonatal hyperammonemia range

A

> 1,000 umol/L

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13
Q

clinical features of Moderate neonatal hyperammonemia

A

depression of CNS
poor feeding
vomiting
may have Resp alkalosis

seizures not characteristic

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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

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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

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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

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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

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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.

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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

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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

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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

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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

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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

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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

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25
Q

metabolic disorder:
Phenylketonuria

you are deficient in?

accumulation of what toxic compound?

result?

A

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

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26
Q

metabolic disorder:
Maple syrup urine disease

you are deficient in?

accumulation of what toxic compound?

result?

A

you are deficient in?
none listed

accumulation of what toxic compound?
Leucine

result?
Leucine toxicity leading to cerebral edema

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27
Q

metabolic disorder:
mitochondrial disease

you are deficient in?

accumulation of what toxic compound?

result?

A

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

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28
Q

metabolic disorder:
peroxisomal disorders

you are deficient in?

accumulation of what toxic compound?

result?

A

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

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29
Q

metabolic disorder:
Lysosomal storage disorders

you are deficient in?

accumulation of what toxic compound?

result?

A

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

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30
Q

metabolic disorder:
Disorders of creatinine biosynthesis

you are deficient in?

accumulation of what toxic compound?

result?

A

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

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31
Q

metabolic disorder:
cholesterol biosynthesis disorders

you are deficient in?

accumulation of what toxic compound?

result?

A

you are deficient in?
steroid hormones

accumulation of what toxic compound?
none listed

result?
Endocrinopathies
disordered cellular signaling leading to aberrant organogenesis

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32
Q

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

A

Ketothiolase deficiency

Succinyl-CoA:3 ketoacid CoA transferase (SCOT) deficiency

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33
Q

common condition in which tolerance for fasting is impaired.

A

ketotic hypoglycemia

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34
Q

features of ketotic hypoglycemia

A

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

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35
Q

treatment of ketotic hypoglycemia

A

frequent snacks and glucose during stress

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36
Q

Ketonuria is normal to prolonged fasting in older infants and children, however if found in neonates, investigate for

A

metabolic disease

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37
Q

a high anion gap metabolic acidosis +/- ketosis suggests

A

a metabolic disorder

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38
Q

Genetic inheritance of IEMs

A

most are autosomal recessive
also x -linked

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39
Q

labs to order in suspected IEM

A

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

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40
Q

in general carriers of AR or x-linked IEM are asymptomatic/symptomatic

A

asymptomatic

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41
Q

Most treatable metabolic disorders are on the newborn screen. Most states use what testing for this?

A

tandem mass spectrometry

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42
Q

Proposed additions or removal of conditions to newborn screen are evaluated by who?

A

federal advisory committee on heritable disorders in the newborn and child (ACHDNC)

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43
Q

when are newborns tested for newborn screen panel

A

24-48 hours

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44
Q

Neonatal screening has what purpose

A

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

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45
Q

The plasma amino acid profile is most useful in identifying disorders of

A

amino acid catabolism

can also be helpful in disorders of organic acid degradation (often normal and not diagnostic)

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46
Q

The urine amino acid profile is helpful in diagnosing

A

primary and secondary orders of renal tubular function
disorders of amino acid transport

Not test of choice for amino acid or organic acid metabolism

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47
Q

Urine acylglycine profile and plasma acylcarnitine profile reflect

A

markers of disordered fatty acid oxidation

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48
Q

plasma free fatty acid to 3-OH butyrate suggests a

A

fatty acid oxidation disorder

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49
Q

Excess 3-OH butyrate suggests a disorder of

A

ketone metabolism

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50
Q

Absence of ketones or decreased 3-OH-butyrate suggests a

A

fatty acid oxidation disorder

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51
Q

treatment of IEM basic guidelines

A

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)

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52
Q

odor of sweaty feet,
think _______ in metabolic acidosis etiologies caused by IEM in infants

A

isovaleric acidemia

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53
Q

many glycogen storages diseases are characterized by

A

hypoglycemia and
hepatomegaly

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54
Q

Glycogen (storage form of glucose) is found most abundantly where?

A

Liver - where it modulates blood glucose levels and in muslces where it facilitates anaerobic work

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55
Q

what glycogen storage disease type falls into this category?

diseases that predominantly affect the liver and have a direct influence on blood glucose

A

Types I VI, and VIII

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56
Q

what glycogen storage disease type falls into this category?

diseases that predominantly involve muscles and affect the ability to do anaerobic work

A

Types V and VII

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57
Q

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

A

Type III

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58
Q

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

A

types II and IV

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59
Q

The diagnosis of Glycogen storage disease type I or type III is suggested by what?

A

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

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60
Q

Treatment in Glycogen storage disease

A

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

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61
Q

Galactosemia genetic

A

Autosomal Recessive

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62
Q

Galactosemia is an AR disease cause by deficiency of

A

Galactose-1-phosphate uridyltransferase

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63
Q

Clinical manifestations of Galactosemia

A

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

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64
Q

The neonatal screening test is imperative that it has a rapid turnaround time bc affected infants with Galactosemia may die how quickly

A

within the first week of life

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65
Q

Infants with Galactosemia are at increased risk for what type of sepsis

A

Severe neonatal Escherichia coli sepsis

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66
Q

When galactose is ingested as lactose in a galactosemia, what may be detectable

A

levels of plasma galactose
erythrocyte galactose 1-phosphate
elevated

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67
Q

How do you confirm the diagnosis of Galactosemia

A

DNA testing for pathogenic variants in galactose-1-phosphate uridyltransferase

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68
Q

Renal tubular dysfunction may be evidenced by what type of acidosis

A

Normal anion gap hyperchloremic metabolic acidosis

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69
Q

Treatment of galactosemia

A

eliminate dietary galactose
Infants who are extremely ill before treatment may die before the therapy is effective

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70
Q

Galactosemia complications longer term

A

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

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71
Q

Galactokinase deficiency genetics

A

Autosomal recessive disorder

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72
Q

Clinical manifestations of Galactokinase deficiency

A

-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

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73
Q

Hereditary fructose intolerance
what happens?
treatment

A

leads to intracellular accumulation of fructose 1-phosphate

emesis
hypoglycemia
severe liver and kidney disease

eliminate fructose and sucrose from diet

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74
Q

Fructosuria is caused by

what happens

A

fructokinase deficiency
not associated with clinical consequences

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75
Q

These disorders are the result of the inability to catabolize specific amino acids derived from protein

A

Disorders of Amino Acid Metabolism

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76
Q

In disorders of amino acid metabolism, the amino acids accumulate in excess and are toxic to various organs such as

A

brain
eyes
skin
liver

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77
Q

Phenylketonuria (PKU) genetics

A

Autosomal recessive

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78
Q

PKU is the result from a defect in the hydroxylation of _______ to form______

A

phenylalanine
tyrosine

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79
Q

In PKU affected infants show what signs at birth and what happens after that?

A

normal at birth
untreated will cause severe Intellectual disability (IQ30) in the first year of life

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80
Q

PKU is found in what testing
dx?

A

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

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81
Q

tx PKU

A

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

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82
Q

PKU outcome and education

A

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)

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83
Q

how are tyrosinemias identified

A

neonatal screening program using tandem mass spectrometry to detect elevated tyrosine and/or succinylacetone

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84
Q

treatment of transient tyrosinemia of the newborn

A

ascorbic acid treatment

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85
Q

2 causes of elevated tyrosine levels screened on newborn screen

A

transient tyrosinemia of the newborn or severe liver disease

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86
Q

tyrosinemia type 1 is due to deficiency of

A

fumarylacetoacetate hydrolase

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87
Q

clinical signs tyrosinemia type 1 (it causes)

A

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

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88
Q

dx/confirmation of Tyrosinemia type 1

A

positive neonatal screening

diagnostic is an increased concentration of succinylacetone

DNA testing is available

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89
Q

Treatment of Tyrosinemia type 1

A

Nitisinone (NTBC - inhibitor of the oxidation of parahydroxyphenylpyruvic acid) whcih effectively eliminates the production of the toxic succinylacetone

diet-low phenylalanine, low tyrosine

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90
Q

which tyrosenemias are worse, which are more benign

A

type I worse
type II and III are more benign - blocked metabolism of tyrosine at earlier step is responsible so succinylacetone is not produced

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91
Q

clinical features of Tyrosinemias II and III

A

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

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92
Q

treatment of Tyrosenemias II and III

A

phenylalanine and tyrosine restricted diet

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93
Q

genetics for Homocystinuria

A

Autosomal Recessive

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94
Q

Homocystinuria is an AR disorder caused by a deficiency of

A

cystathionine B-synthase
(when this is deficient, homocysteine accumulates in the blood and appears in the urine, also causes raise in Methionine)

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95
Q

detection of Homocystinuria

A

newborn screen (looks for elevated methionine in blood)

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96
Q

Homocystinuria clinical symptoms

A

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

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97
Q

confirmation of Homocystinuria

A

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

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98
Q

treatment of Homocystinuria

A

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

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99
Q

which form of homocystinuria is more likely to be missed on newborn screen bc methionine concentrations are not always above the screening cutoff

A

Pyridoxine-responsive Homocystinuria

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100
Q

genetics for Maple syrup urine disease (MSUD)

A

Autosomal Recessive

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101
Q

Maple syrup urine disease is also called

A

Branched chain ketoaciduria

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102
Q

Maple syrup urine disease (MSUD) is caused by a deficiency of

A

the decarboxylase that initiates the degradation of the ketoacid analogs of the 3 branched chain amino acids (leucine, isoleucine and valine)

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103
Q

onset of Maple syrup urine disease

A

classic at birth
intermittent onset and late onset forms

classic form occurs typically 1-4 weeks of birth

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104
Q

clinical s/s Maple syrup urine disease

A

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

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105
Q

definitive diagnosis of maple syrup urine disease

A

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

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106
Q

management of Maple syrup urine disease

A

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

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107
Q

Genetics for OTC (ornithine carbamoyltransferase) deficiency

A

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

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108
Q

Clinical manifestations of OTC deficiency

A

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

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109
Q

confirmation of OTC deficiency

A

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

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110
Q

ASL deficiency genetics

A

autosomal recessive

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111
Q

detection of ASL

A

newborn screen - elevated citrulline
confirmed by detection of elevated argininosuccinic acid in the urine

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112
Q

treatment of hyperammonemia

A

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

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113
Q

a disorder of renal tubular transport of cystine, lysine, arginine and ornithine

A

Cystinuria

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114
Q

diagnosis of Cystinuria

A

pattern of amino acid excretion in the urine

DNA testing

115
Q

Treatment of Cystinuria

A

Based on increasing the solubility of cystine by complexing it with compounds such as penicillamine

116
Q

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

A

Hartnup Syndrome

117
Q

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

A

lasts longer

118
Q

pain that arises from actual or threatened damage to non-neural tissue and is due to the activation of nociceptors

A

Nociceptive pain
example is an incisional cut

119
Q

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

A

somatic pain

120
Q

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)

A

Visceral pain

121
Q

which type of nociceptive pain is more difficult to treat

A

visceral pain

122
Q

pain pathway

A

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
Q

characteristics of acute pain

A

clear onset
relatively clear offset
contextual factors are less relevant
“protective” - meaning going to get better

124
Q

characteristics of recurrent pain

A

clear onset
relatively clear offset
Episodes of pain recur
contextual factors more relevant

125
Q

Vaso-occlusive pain in sickle cell is categorized as what type of pain

A

Recurrent pain

126
Q

Headache or migraine is categorized as what type of pain

A

Recurrent pain

127
Q

cancer pain is categorized as what type of pain

A

ongoing pain

128
Q

characteristics of ongoing pain

A

variability specificity of onset
no clear offset
contextual factors more relevant

129
Q

what category of pain?
characteristics are…
variability specificity of onset
no clear offset
contextual factors more relevant

A

ongoing pain

130
Q

what category of pain?
characteristics are…
clear onset
relatively clear offset
Episodes of pain recur
contextual factors more relevant

A

recurrent pain

131
Q

what category of pain?
characteristics are…
clear onset
relatively clear offset
contextual factors are less relevant
“protective” - meaning going to get better

A

Acute pain

132
Q

what category of pain?
characteristics are…

A

no clear onset
no offset
contextual factors extremely relevant

133
Q

characteristics of chronic pain

A

no clear onset
no offset
contextual factors extremely relevant

134
Q

goals of chronic pain

A

eradication to the degree possible

improve function

135
Q

pain caused by a lesion or disease of the somatosensory nervous system

A

Neuropathic pain

136
Q

how do most people describe neuropathic pain

A

sharp, burning, fire, electric pain

137
Q

what type of pain medicine is used to control neuropathic pain

A

neuromodulators

138
Q

what is allodynia?

A

lowered threshold to pain. light touch is perceived as very painful

stimulus and response mode differ

139
Q

What is hyperalgesia

A

increased response over time

stimulus and response mode are the same

140
Q

what is Hyperpathia

A

raised threshold: increased response

stimulus and response mode may be the same or different

141
Q

what is hypoalgesia

A

raised threshold: lowered response

stimulus and response mode are the same

142
Q

pain characterized as a temporary increase in severity of pain over and above the pre-existing baseline pain level

A

Breakthrough pain

143
Q

pain that results when the blood level of the medicine falls below the minimum effective analgesic level towards the end of dosing interval

A

End of dose pain

144
Q

World Health Organization (WHO) pain ladder
Mild pain treatment recommendations

A

Nonopiod +/- adjuvant therapy

145
Q

World Health Organization (WHO) pain ladder
Mild to moderate pain treatment recommendations

A

“Weak” opioid or multimodal +/- nonopioid +/- adjuvant therapy

146
Q

World Health Organization (WHO) pain ladder
Moderate to Severe Pain
treatment recommendations

A

“strong” opioid
+/- nonopioid +/- adjuvant therapy

147
Q

World Health Organization (WHO) pain ladder
Severe to very severe pain treatment

A

Interventional treatments +/- nonopioid +/- adjuvant therapy

148
Q

delivers low voltage electrical current through the skin. Activates endogenous descending inhibitory pathways

A

Transcutaneous electrical nerve stimulation (TENS)

149
Q

directions for Lidoderm patch (Lidocaine 5%)

A

for 12 hours on and off
cannot be combined with heating pads

you can get OTC up to 4%

150
Q

5As of opioid assessment

A

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
Q

opioid conversion for Morphine….ie…they are on 10mg IV morphine dose…what would PO be

A

3:1
30mg

152
Q

Opioid conversions for IV to PO

A

Morphine and Hydromorphone is 3:1

Oxycodone is 2: 1

Methadone is 1:1

Tramadol is 1.2:1

153
Q

Codeine use in kids

A

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
Q

a state of nociceptive sensitization caused by exposure to opioids

A

Opioid Induced Hyperalgesia

155
Q

Treatment for Opioid induced hyperalgesia

A

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
Q

how much to prescribe for opioids

A

opioids should be prescribed in amounts to cover less than 2 weeks or 2 months at most

157
Q

screening tool to assess opioid abuse risk

A

CRAFFT screening tool

158
Q

opioid combos to beware of for abuse

A

Hydrocodone/acetaminophen(Norco, Vicodin, Lortab)
Oxycodone/acetaminophen (Percocet)

159
Q

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?

A

schedule II, III, IV, and V controlled substances

before dispensing or prescribing opioids, Benzodiazepines, barbiturates or carisoprodol

160
Q

what level of sedation?
Purposeful response to verbal or tactile stimulation
no airway intervention required
Adequate spontaneous ventilation
cardiovascular function usually maintained

A

Moderate sedation/analgesia

161
Q

what level of sedation?
Normal responsiveness
unaffected airway
unaffected spontaneous ventilation
Unaffected cardiovascular function

A

minimal sedation

162
Q

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

A

Deep sedation/analgesia

163
Q

what level of sedation?
Unarousable even with painful stimulus
airway intervention often required
spontaneous ventilation frequently inadequate
cardiovascular function may be impaired

A

General anesthesia

164
Q

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

A

analgesia

165
Q

Benzodiazepines Mechanism of action

A

acts centrally and activates GABAa neuronal receptors in the brain
-acts as agonists in the brain to cause and inhibitory effect

166
Q

Benzodiazepines therapeutic effects

A

induce anterograde amnesia
anticonvulsant properties
muscle relaxant properties

167
Q

Benzodiazepines adverse effects

A

over sedation or prolonged sedation

hypotension

Paradoxical excitation (agitation and delirium)

resp depression

physiologic dependence

168
Q

sedation dosing for Midazolam continuous infusion

A

0.01-0.05mg/kg/hr to start
up to 0.3mg/kg/hr

169
Q

sedation dosing for Lorazepam intermittent dosing

A

0.05-0.1mg/kg/dose every 4-6 hours

170
Q

IV intermittent dosing and cont infusion dosing for Propofol in ICU sedation .also for amnesia

A

IV intermittent: 1-2mg/kg/dose
contin: 5-50mcg/kg/min

171
Q

dosing for sedation using Dexmedetomidate cont infusion

A

0.2-0.7 mcg/kg/hr up to 1mcg/kg/hr

172
Q

explain state behavioral scale

A

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
Q

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)

A

Richmond Agitation Sedation Scale (RASS)

Sedation-Agitation Score (SAS)

174
Q

explain Richmond Agitation Sedation Scale (RASS)

A

+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
Q

what class of med?
act selectively on neuron that transmit and modulate nociception, leaving other sensory modalities and motor fx intact

A

Opioid analgesics

176
Q

what is more potent?
morphine or Fentanyl

A

Fentanyl is 100xs more potent than morphine

177
Q

dosing for continuous infusion of Fentanyl

A

0.5-4mcg/kg/hr

178
Q

what opioid analgesic is good to use in renal dysfunction

A

Hydromorphone
no active metabolites

179
Q

what does FLACC stand for

A

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
Q

best method for pain monitoring

A

Ask the patient/self report

181
Q

Behavioral pain scale

A

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
Q

restore trial
objective
result

A

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
Q

Basic principles of CVICU sedation

A

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
Q

Neonate <30 days sedation 1st line and second line

try to avoid

A

non pharm first of course
Fentanyl
Dexmedetomidine as adjunct therapy

try to avoid Benzos but can be used - shown to affect neurodevelopmental outcomes

185
Q

Age 30 days to 15 years sedation pathway fist line, second

A

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
Q

Age > 15 yrs or >50 kg sedation pathway first line, second line

A

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
Q

what is PTSD from their ICU experience

A

Post-Intensive Care Syndrome (PICS) seen in >50% of critical illness survivors

188
Q

Midazolam exposure is associated with alterations in _______ development and _____

A

hippocampal development
poorer neurodevelopmental outcomes

(can affect brain development)

189
Q

new or increased physical, cognitive or mental health impairment in a pt after hospitalization in a critical care unit

A

Post intensive care syndrome (PICS)

190
Q

Children suffering from Post intensive care syndrome (PICS) may experience

A

muscle weakness
delayed developmental milestones
learning problems
sadness

191
Q

a group of symptoms that can occur upon abrupt discontinuation or decreased intake of certain medications due to medical treatment

A

iatrogenic withdrawal

192
Q

risk factors for iatrogenic withdrawal syndrome

A

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
Q

classic signs of opioid withdrawal

A

neurological excitability

GI dysfunction (Vomiting, loose stool)

autonomic instability (sweating, elevated temp)

Poor organization of sleep state

194
Q

Benzodiazepine withdrawal symtpoms

A

Agitation

visual hallucinations

facial grimacing

small amplitude choreic or choreoathetoid movements

Seizures

195
Q

Dexmedetomidine withdrawal symptoms

A

Agitation
irritability
headache
rebound hypertension

196
Q

Dexmedetomidine withdrawal is most likely to be seen when greater than _____ of therapy have been received

A

> 24 hours

197
Q

what is a tool to assess withdrawal?

A

Withdrawal Assessment Tool-1 (WAT-1)

198
Q

WAT-1 consists of

A

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
Q

sedation wean principle for time to wean

A

duration of wean should be less time than the patient received indicated medication

200
Q

evaluation before sedation

A

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
Q

American Society of Anesthesiologist (ASA) physical status classification

A

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
Q

NPO guidelines for sedation

A

-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
Q

developmental understanding of pain at 2-7 yrs old

A

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
Q

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

A

preoperational thought
2-7 yrs old

205
Q

What is the developmental understanding of pain at 7-12 yrs old

A

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
Q

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)

A

Concrete operational thought
7-12 yrs old

207
Q

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

A

Formal operational thought
>=12 yrs

208
Q

what is the developmental understanding of pain >=12 yrs old

A

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
Q

techniques for non-pharm intervention in pain mgmt

Infants (0-12 months)

A

Parents voice (talking, singing on tape)

touching (holding, rocking)

pacifier

music

swaddling

massage

210
Q

techniques for non-pharm intervention in pain mgmt

toddlers (12-36 months)

A

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
Q

techniques for non-pharm intervention in pain mgmt

Preschoolers 3-5 years

A

pinwheels
party blowers
feathers
pop up books
storytelling
comfort item
music
singing
manipulatives

212
Q

techniques for non-pharm intervention in pain mgmt

School age 6-12 yrs

A

electronic toys (nintendo DS, psp, iopd)
pop up books
i spy books
participation in procedure
imagery
storytelling
breathing techniques
muscle relaxation

213
Q

techniques for non-pharm intervention in pain mgmt
adolescents 13-18 yrs

A

music
comedy tapes
imagery
massage
muscle relaxation
TV
video
other electronics

214
Q

cognitive behavioral interventions
non pharm

A

distraction

relaxation

guided imagery

thought stopping - like I know it hurts but this wont last long

behavioral contracting - Beads of courage example

215
Q

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.

A

functional

216
Q

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.

A

chronic pain

217
Q

topical anesthetic should allow ___ -____ min for skin penetration with a duration effect < ___ hrs

A

30-60 min
<4 hours

218
Q

when using topical lidocaine, monitor for ______ if used over significant body surface area

A

toxicity

219
Q

intradermal onset of action is ___ to ____ min with duration ____

A

1-2 min
duration <1 hr

220
Q

state of sedation that provides anxiolysis

A

minimal sedation

221
Q

Midazolam is used for what type of sedations

A

minimal
moderate

222
Q

Midazolam IV in sedation
IM
Intranasal
PO

dose
onset
duration

A

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
Q

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.

A

moderate sedation

224
Q

lorazepam used in what type of sedation

A

minimal

225
Q

Dose
onset
duration
Lorazepam IV in minimal sedation

A

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
Q

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.

A

deep sedation

227
Q

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.

A

General anesthesia

228
Q

propofol is used in what type of sedations

A

moderate
deep

229
Q

Propofol dosing in sedations

onset
duration

A

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
Q

if using ketamine for moderate or deep sedation recommendations to use 0.5mg/kg boluses of ____

A

Propofol

231
Q

Avoid _Propofol for sedation in patients with _____ or _____ allergy

A

egg
soy

232
Q

can a nurse administer propofol in Texas

A

no

233
Q

Dexmedetomidine is used in what level of sedations

A

minimal
moderate

234
Q

Dexmedetomidine
IV bolus
IV continuous
Intranasal

dose
onset
duration

A

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
Q

Pentobarbital is used in what type of sedations

A

moderate

236
Q

Pentobarbital
dose
onset
duration

IV
PO

A

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
Q

When is etomidate need to be avoided

A

if sepsis is suspected

238
Q

AMPLE history

A

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
Q

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.

A

Mallampati Classification

240
Q

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.

A

Mallampati Classification

241
Q
A

Mallampati class I

242
Q
A

Mallampati Class II

243
Q
A

Mallampati class III

244
Q
A

Mallampati class IV

245
Q

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.

A

ASA physical status classification

246
Q

_____________ 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.

A

Anesthesiologist

247
Q

oral sucrose is safe and effective in reducing signs of distress associated with minor, painful procedures in what ages

A

neonates
and moderately effective in infants up to 6 months of age

248
Q

Single agents are often used for diagnostic procedures; however, moderate sedation for therapeutic procedures is often best achieved with a combo of

A

Benzodiazepine and an opioid

Lippincott

249
Q

discharge criterion after sedation

A

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
Q

regional anesthetic technique to provide analgesia below the umbilicus when severe pain is expected only for 24 hours post op

A

Caudal block

251
Q

How to treat resp depression in sedation/analgesia

A
  • 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
Q

How to treat apnea in sedation/analgesia

A
  • 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
Q

How to treat Pruritis in sedation/analgesia

A
  • 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
Q

How to treat Nausea/Vomiting in sedation and analgesia

A
  • 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
Q

How to treat constipation in sedation and analgesia

A

*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
Q

How to treat urinary retention in sedation and analgesia

A
  • 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
Q

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.

A

anxiety

258
Q

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.

A

Agitation

259
Q

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.

A

Delirium

260
Q

Delirium is characterized by what 4 features

A

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
Q

risk factors in sedation

A

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
Q

Sedation assessment tools mentioned in Lippincott

A

Ramsey Sedation Scale
Comfort Scale
State Behavioral scale

263
Q

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.

A

Delirium

264
Q

Delirium tools are still being developed. Currently what is most commonly used

A

Pediatric Confusion Assessment Method

265
Q

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.

A

Benzodiazepine
ie: Midazolam

266
Q

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.

A

Barbiturate
ie) pentobarbital

267
Q

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.

A

Selective A2 Agonist
ie) Dexmedetomidine

268
Q

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.

A

Benzodiazepine

269
Q

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.

A

Ketamine

270
Q

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.

A

Etomidate

271
Q

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.

A

Propofol

272
Q

what are some nonpharm ways to manage delirium

A

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
Q

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.

A

Tolerance

274
Q

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.

A

Dependence

275
Q

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.

A

Withdrawal

276
Q

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.

A

opioids
benzodiazepines

277
Q

withdrawal assessment tools

A

<1 month - neonatal assessment scale
>1 month - WAT 1

278
Q

*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.

A

ATC long-acting oral agents.

279
Q

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.

A

methadone
diazepam

280
Q

If symptoms of withdrawal are observed during weaning -

A

IV dose of rescue morphine (0.05-0.1mg/kg) or lorazepam (0.05-0.1mg/kg) given for opioid or benzodiazepine withdrawal

281
Q

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.

A

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.

282
Q

Adverse effects of Clonidine

A

sedation
bradycardia
hypotension

283
Q

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.

A

Neuromuscular blocking agents

Critical Care 2 (14 decks)

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