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PSE329 > pse > Flashcards

pse Flashcards

1
Q

general and specific therapeutic measures of an acutely sick child

A

general = maintain vital functions (oxygenation, hydration, a-b balance)

specific = treat infection, high dose IV glucose, carnitine supplementation

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2
Q

clinical symptomatology of IEM

A

possibility
- acutely sick after being healthy
- intractable seizures
- unusual odour

strong possibility
- vomiting
- lethargy
- comas
- sepsis
- jaundice
- FTT
- apnoea
- family history

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3
Q

clinical manifestations of IEM - neurological signs

A
  • poor suck
  • lethargy
  • seizures
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4
Q

clinical manifestations of IEM - GI signs

A
  • poor feeding
  • vomiting
  • diarrhoea
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5
Q

clinical manifestations of IEM - respiratory signs

A
  • hyperpnea
  • respiratory failure
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6
Q

clinical manifestations of IEM - organomegaly

A
  • liver
  • heart
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7
Q

2 basic biochemical mechanisms of IEM

A
  • transportation defects
  • secondary inhibition
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8
Q

first 6 steps of metabolic therapy

A
  1. reduce precursor substrate load
  2. provide caloric support
  3. provide fluid support
  4. remove metabolites via dialysis
  5. divert metabolites
  6. supplement with cofactors
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9
Q

examples of newborn screens - disorders of amino acid metabolism

A
  • MSUD
  • homocystinuria
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10
Q

examples of newborn screens - disorders of fatty acid oxidation

A
  • trifunctional protein deficiency
  • carnitine uptake defect
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11
Q

examples of newborn screens - disorders of organic acid metabolism

A
  • multiple carboxylase deficiency
  • isovaleric acidaemia
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12
Q

examples of newborn screens - haemoglobinopathies

A
  • sickle cell disease
  • haemoglobin SC disease
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13
Q

examples of newborn screens - miscellaneous

A
  • galactosaemia
  • cystic fibrosis
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14
Q

match the smell to the IEM
1 - PKU
2 - tyrosinemia
3 - maple syrup urine disease
4 - isovaleric acidemia
5 - multicarboxylase deficiencies

A

1 - PKU - musty/mousy
2 - tyrosinemia - boiled cabbage
3 - maple syrup urine disease - maple syrup
4 - isovaleric acidemia - sweaty feet
5 - multicarboxylase deficiencies - cat urine

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15
Q

describe postnatal lung development from birth onwards

A
  • terminal saccules continue to divide into alveoli
  • alveoli number increase massively
  • lung enlargement
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16
Q

5 stages of lung development

A
  • embryonic
  • pseudoglandular
  • canalicular
  • saccular
  • alveolar
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17
Q

2 phases of lung development

A

structural and functional development

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18
Q

when does surfactant production begin?

A

24 weeks (canicular phase)

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19
Q

what is atelectasis?

A

collapse of entire/lobe of lung

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20
Q

how does smoking affect the respiratory system?

A
  • mucus and cilia in normal respiratory epithelium clean inhaled air
  • function declines with years of smoking
  • cigarettes have carcinogens
  • carcinogens in smoke cause progressive series of epithelial changes
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21
Q

define TB

A

chronic infection of the lungs caused by mycobacterium tuberulosis

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22
Q

condition resulting from inadequate production of surfactant and the resultant collapse of alveoli is:

A

respiratory distress syndrome

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23
Q

what is respiratory distress syndrome?

A
  • when immature lungs lack sufficient surfactant, alveoli collapse
  • high risk = <1kg, <28w
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24
Q

how is respiratory distress syndrome treated?

A

surfactant and oxygen

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25
Q

7 possible factors affecting lung development in infancy and childhood

B3RRESD

A
  • birth weight
  • premature birth
  • breast feeding
  • respiratory infection
  • recurrent wheezing
  • environmental factors
  • surfactant deficiency
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26
Q

how is bone metabolism altered in COPD

A
  • bone loss
  • vitamin D depletion
  • osteoporosis affects chest wall
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27
Q

3 enzymes that appear in plasma and indicate what theyre a result of

A

increase liver ALP - due to increased canalicular pressure in obstructive biliary disease (disease)

increased y-GT - due to phenobarbital (induction by drugs)

increased serum ACP - due to prostate cancer (carcinogens)

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28
Q

6 factors that affect enyzme levels in blood

A
  • clearance of enzymes from blood
  • entry of enzymes into blood
  • leakage of enzymes from cells
  • efflux of enzymes from cells
  • enzyme changes leading to altered enzyme production
  • non-specific causes
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29
Q

briefly discuss the clinical diagnostic importance of alkaline phosphatase

A

ps = prostate, erythrocytes
ca = carcinoma of prostate

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30
Q

briefly discuss the clinical diagnostic importance of aspartate aminotransferase

A

ps = liver, skeletal muscle, heart, kidney, erythrocytes
ca = liver disease, muscle disease, myocardial infarction

31
Q

briefly discuss the clinical diagnostic importance of lactate dehydrogenase

A

ps = heart, liver, skeletal muscle
ca = myocardial infarction, haemolysis

32
Q

briefly discuss the clinical diagnostic importance of choline esterase

A

ps = liver
ca = insecticide, organo-phosphorus poisoning, liver disease, drug sensitivity

33
Q

5 causes of cell damage or death that would result in high levels of enzymes in blood

GNIPI

A

genetic factors - diabetes, gout

nutritional disorders - P-E malnutrition, micronutrient deficiencies

infectious agents - viruses, bacteria

physical agents = trauma, radiation

immune mechanisms - anaphylaxis, immunity

34
Q

define congenital disease

A
  • disease that is present at birth
  • acquired during development in uterus, not hereditary
35
Q

define genomic imprinting

A
  • during recombination when portions of chromosome break away or are deleted
  • effects depend on if abnormal gamete is produced through oogenesis or spermatogenesis
36
Q

describe the diagnostic application of PCR in fragile-x syndrome

A
  • differences in size of CGG repeat between normal and pre-maturation allow for products of different sizes and mobility
  • with full maturation, region between primers is too large to be amplified by PCR
37
Q

foodborne illness causes by foodservice worker coughing/sneezing on food

A

staphylococcus aureus

38
Q

consumption of improperly canned foods can result in a deadly foodborne illness which is caused by

A

clostridium botulinum

39
Q

Listeria monocytogenes is a harmful microorganism commonly found in

A

soft cheeses and unpasteurised milk

40
Q

A family of two adults and three children develop gastroenteritis while vacationing in New York. The onset is abrupt with abdominal cramps and watery diarrhoea. None of the family members has a fever or nausea; nobody experiences vomiting. Within 24 hr, the symptoms have resolved. The family notes that the condition developed 10h after they had eaten a buffet dinner in a roadside café. When they report the disease to the public health office, an investigator is assigned to investigate the restaurant. The investigation reveals that one of food products eaten by the family was contaminated with
a high concentration of type A Clostridium perfringens. Which food is the most likely source of infection?

A

beef stew

41
Q

list the functions of the skin

A
  • protection from infection
  • sensory organ
  • control loss and movement of fluids
  • insulation from trauma
  • temperature regulation
42
Q

name the pathophysiological stages or phases during burns

A

emergent, fluid shift, hypermetabolic, resolution

43
Q

name the chemicals released during burn injury that will ultimately lead to burn shock

A

histamine, eicosanoids, kinins, serotonin

44
Q

name the ointment used to treat burns

A

silver sulfadiazine

45
Q

4 signs of premature labour

A
  • low, dull backache
  • regular uterus contractions
  • watery vaginal discharge
  • pelvic pressure
46
Q

5 measures to take care of premature baby

A
  • learn to recognise babys signs
  • change environment to quiet and dim
  • stay calm when baby is irritable
  • use car seats correctly
  • make appointments with babys caregivers
47
Q

management of a mother suffering from premature uterine contractions

A
  • sometimes fluid and bed rest
  • sometimes meds to relax smooth muscle and block calcium channels
48
Q

developmental age vs gestational age

A

gestational age = how many weeks since being born

developmental age = for premature babies, counts down weeks they should have been in the womb

49
Q

what is respiratory alkalosis?

A

pCO2 <35
carbonic acid defect

50
Q

causative factors of respiratory alkalosis

A
  • hyperventilation
  • pulmonary disease
  • cerebrovascular accident
  • severe anxiety
51
Q

how does body compensate for respiratory alkalosis

A

kidneys conserve H+ and excrete HCO3-

52
Q

what is respiratory acidosis

A

CO2 > 45
hypercapnia

53
Q

how does body compensate for respiratory acidosis

A

kidneys eliminate H+ and retain HCO3-

54
Q

hypoventilation leads to: respiratory alkalosis/acidosis

A

respiratory acidosis

55
Q

what is metabolic alkalosis

A

HCO3- >26

56
Q

how does the body compensate for metabolic alkalosis

A

most commonly occurs with renal dysfunction

57
Q

what is metabolic acidosis?

A

HCO3 < 22
pH < 7.35

58
Q

how does the body compensate for metabolic acidosis?

A
  • increased ventilation
  • renal excretion of H+
  • K+ changes with excess H+ in ECF
59
Q

a person with poorly controlled diabetes mellitus with develop: metabolic acidosis/alkalosis

A

metabolic acidosis

60
Q

a person who consumes large amounts of sodium bicarbonate (“baking soda”) to settle an upset stomach, risks: metabolic acidosis/alkalosis

A

metabolic acidosis

61
Q

name the different buffer systems in the body

A

bicarb-, phosphate, and protein-buffer system
respiratory and renal mechanism

62
Q

Osmolarity vs Osmolality

A

osmolarity = number of solutes per 1L solvent
osmolality = number of particles per 1kg solvent

63
Q

define dehydration

A

excessive water loss in the body

64
Q

4 symptoms associated with severe dehydration

A
  • lethargic
  • sunken eyes
  • drinking poorly
  • skin pinch goes back slowly
65
Q

what is the structure of DNA?

A
  • cylindrical nucleiod
  • bullet shaped core
  • 2 identical RNA
  • 9 regulatory genes (6 reg, 3 structural)
66
Q

how do you diagnose HIV

A

sample blood tests - see if blood contains human
antibodies specific to HIV

key tests = enzyme-linked immunosorbent assay, western blot

67
Q

How does HIV progress to AIDS?

A
  • HIV destroys CD4+ Th cells (important for immunity) and suppresses the immune response to other pathogens
  • over time, HIV levels increase and CD4+ T cell count declines in blood
68
Q

3 causes of hyponatraemia

A
  • excessive sodium losses and replaced with sodium-free water
  • excess water intake in relation to output
69
Q

3 causes of hyperkalaemia

A
  • insulin deficiency
  • addisons disease
  • renal disease
70
Q

hyperkalaemia symptoms

A
  • muscle cramps
  • urine abnormalities
  • respiratory distress
  • decreased cardiac contractility
  • EKG changes
  • reflexes
71
Q

hyperkalaemia treatment

A

decrease intake and increase renal secretion of insulin, glucose, bicarb and Ca++

72
Q

hyponatraemia symptoms

A
  • stupor
  • anorexia
  • lethargy
  • tendon reflexes
  • limp muscles
  • orthostatic hypotension
  • seizures
  • stomach cramps
73
Q

hyponatraemia treatment

A

water intoxication = limit water, admin diuretics

sodium deficiency = admin saline solution

PSE329 (2 decks)

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