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Flashcards in stats & ethics Deck (62):
1

What is the equation for specificity?

True negatives / true negative + false positive

2

What is the probability that a test will produce a true negative result when used on a population without disease?

Specificity

3

What is the probability that a test will produce a true positive result when used on a population with the disease?

Sensitivity

4

What is the probability that someone who had the disease will test positive?

Positive predictive value

5

What is the equation for positive predictive value?

True positive / true positive + false positive

6

What is the probability that someone who does NOT have a disease will have a negative test result?

Negative predictive value

7

What is the equation for negative predictive value?

True negative / true negative + false negative

8

What does the P value respresent?

The chance that the null hypothesis was rejected in error

9

What P value is significant enough to reject the null hypothesis?

<0.05

10

What type of error occurs when the null hypothesis is rejected in error?

Type 1

11

What type of error occurs when the null hypothesis was NOT rejected but the null hypothesis is not true?

Type 2

12

What number is obtained by adding the sample values and dividing by the size of the sample?

Mean

13

What number is the middle value in which half the data is larger and the other have is smaller?

Median

14

What is the most frequently occurring number in a sample?

Mode

15

What type of study follows persons with risk factors to determine the outcomes?

Cohort study

16

What type of study examined those with a disease and then checks for common risk factors?

Case control study

17

What is an analysis of data from several studies?

Meta analysis

18

Is a 15 y/o who has a child and lives on her own considered an emancipated minor?, why?

No - must be older than 16

19

In a comatose infant, what two lab values are most important in diagnosing inborn error of metabolism ?

Ammonia and anion gap

20

What inborn errors of metabolism should you consider in a patient with high ammonia and no acidosis?

Urea cycle defect or transient hyperammonemia

21

What inborn errors of metabolism would you consider in a patient with high ammonia and metabolic acidosis?

Propionic acidemia
Methylmalonic acidemia
Fatty acid oxidation defects

22

What inborn error of metabolism would you consider in a child with normal serum ammonia and metabolic acidosis?

Maple syrup urine disease

23

What inborn errors of metabolism would you consider in a patient with normal ammonia and normal abg?

Aminoacidopathy
Galactosemia
Non Ketotic hyperglycinemia

24

What is the appropriate treatment in a comatose infant with high ammonia ?

If >200, reduce ammonia with benzoate and phenylacetate
If >600, dialysis

25

What is the normal serum ammonia value in a newborn?

<110

26

What is the best initial study in a 2 day old infant with acidosis, kerosine and high ammonia?

Measure urine organic acids

27

What should be the first study in an infant who presents with the appearance of sepsis but no fever or other signs of infection?

Serum ammonia level

28

What is the first step in managing a patient with organic acidemia?

Hydration

29

When would organic acidemias present?

First 2 days of life after introduction of protein in the diet

30

What are the similarities and difference between organic acidemias and brain tumors?

Both have decreased appetite, falling down and delayed milestones but brain tumors would progressively worsen

31

What happens to the CBC in patients with metabolic acidosis?

Suppression of bone marrow with low platelets and WBC count

32

What condition should you expect in an infant with poor feeding, seizure and odor that smells of sweaty feet ?

Isovaleric acidemia

33

What is the appropriate treatment for isovaleric acidemia?

Protein restriction

34

What are the symptoms of Propionic acidemia?

Tachypnea, poor feeding and lethargy

35

What is the treatment for methylmalonic acidemia?

Vitamin b12

36

How are fatty acid oxidation defects inherited ?

Autosomal recessive

37

What would you consider in a patient who had a brief episode of illness and decreased PO intake followed by hypoglycemia and hepatoma halt?

Fatty acid oxidation defects

38

What lab findings would you suspect in a child with defects in fatty acid metabolism?

Absence of reducing substances and ketones in the urine
Normal serum amino acids

39

What is the test for definitive diagnosis of fatty acid oxidation defects?

Plasma acylcarnitine profile

40

What is the treatment for urea cycle defects?

Reduce serum ammonia by reducing protein intake and increasing glucose via IV

41

Which metabolic disorders do NOT present with hepatomegaly?

Organic acid defects
Hyperinsulinism
Urea cycle defects

42

Which syndrome should you consider in a patient with hypotonia, dysmorphic features but NO high ammonia?

Zellweger syndrome

43

If an infant presents with signs of inborn error of metabolism but has ketonuria, what disease could you rule out?

Urea cycle defects

44

What should you consider in a patient with elevated ammonia, hypotonia and coma but no acidosis?

Urea cycle defect

45

What would you suspect in a patient who is normal at birth but then develops poor feeding, abdominal distention, hypoglycemia and gram negative sepsis?

Galactosemia

46

What laboratory finding would you expect in galactosemia?

Non glucose reducing substances in the urine

47

How is definitive diagnosis of galactosemia made?

Measuring GALT in RBCs (will be deficient)

48

How are children with galactosemia treated?

Galactose free diet

49

What complications can arise with untreated galactosemia?

cataracts, intellectual disability or liver disease

50

What should you suspect in a child with seizures after each meal?

Inherited fructose intolerance

51

What is the duarte variant?

Infants with half the normal amount of GALT...newborn screen may be positive but require more specific testing

52

What is the drug of choice in refractory hypoglycemia in infants?

Diazoxide

53

How would you know the difference between Hyperinsulinism and adrenal insufficiency?

Both have hypoglycemia but adrenal insufficiency does NOT have ketonuria

54

Why do children with beckwith wiedemann syndrome develop hypoglycemia?

Islet cell hyperplasia

55

What should you suspect in a patent with lactic acidosis, alopecia and rash as well as neurological signs?

Biotinidase deficiency

56

Which amino acids are elevated in maple syrup urine disease?

Valine
Isoleucine
Alloisoleucine (not normal in infants)
Leucine

57

What is the most useful test in an infant with hypoglycemia, seizures and hepatomegaly ?

Urine ketones and reducing substances

58

What should you consider in an infant with tachypnea, shallow breathing, lethargy and hypertonicity?

Maple syrup urine disease

59

What is the treatment of alcaptonuria?

Diet low in phenylalanine and tyrosine

60

What condition results in dark sclerae, cartilage, sweat, cerumen and urine?

Alcaptonuria

61

What causes dark urine in patients with alcaptonuria ?

Homogentisic acid in urine

62

What is the formula for sensitivity?

True positives / true positives + false negatives