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SIU6 Special patient group > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

why is prescribing different in children from adult? What are the 5 psysiological factor to consider when rx?

A 
children are NOT small adult 
Body weight
Body composition
Surface area
Nutritional status
Organ maturation
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2
Q

what is the most appropriate way of prescribing in children? (most used meds are liquid form)

A

to prescribe the actual dosage in mg instead of volume ml, because meds have different strength 120mg/5ml or 250mg/5ml

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3
Q

how do we monitor children who are on IV fluid?

A

daily UE

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4
Q

when rx maintenance fluid to children, what are the details you need to inc in prescribing?

A
  • Fluid type (concentration)
  • Volume (e.g. 500mL bag)
  • Rate (mL/hour)
  • Additives if required eg potassium
  • ?duration (some trusts)
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5
Q

Calculation for fluid deficit for %dehydration

- what patient facotr is the calculation based on?

A

body weight

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6
Q

what is the equation for fluid deficit in ml?

A

fluid deficit (ml)= %dehydration x weight (kg) x 10

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7
Q

how would you use the fluid deficit volume in prescribing

A

fluid deficit volume represents the EXTRA volume of fluid needs to be ADDED to total MAINTENANCE fluid requirement

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8
Q

the volume of fluid deficit need to be replaced over how long of a peroid

A

24-48 h depends on condition

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9
Q

when prescirbing fluid in neonates, what are the patient factors to be considered?

A
  • gestational age
  • neonatal age
  • weight
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10
Q

what is gestational age?

A

from first day of your last menstrual period , this inc extra 2 weeks where woman is NOT pregnant

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11
Q

what is neonatal age?

A

the actual age of baby starting at the time of conception.

neonatal age is going to be 2 weeks less than gestational age

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12
Q

why is fluid calculation in neonates complicated?

A

the fluid requirements increase over first 4-5 days of life for neonates, dose changes all the time

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13
Q

what is ususal neonatal fluid?

A

10% dextrose

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14
Q

is there a set amount of additive requirement for neonates fluid?

A

no, depends on clinical condition and actual age. and local policy

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15
Q

age divisions for pre-term new borns

A

< 37 w gestation

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16
Q

age division for neonates

A

37w gestation < neonates < 1 month post-natal

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17
Q

age divisions for infant

A

1 month age- 2 years

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18
Q

age divisions for child

A

2 -11 years

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19
Q

age divisions for adulescent

A

12- 18 years

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20
Q

definition for Unlicensed

A

not licensed for

  • age group
  • indication
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21
Q

what is PUMA?

A

paediatric use marketing authorisation (PUMA)

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22
Q

Is it necessary to obtain consent to rx unlicensed med in children if care has been taken?

A

no!

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23
Q

what is the proportion of unlicensed rx in children in european hosptial?

A

2/3

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24
Q

what does unlicensed med mean?

A

it reflects the lack of evidence from limited clinical trial

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25
what could be the problems in IV formulation?
contraindicated excipents due to poor metabolism eg Benzyl alcohol displacement value
26
can we administer a fraction of the retal suppositries? | what is the other drawback?
no, cant assume homogenous distribution of drug throughout suppository very expensive
27
what are the general requiements for liqid formulation for neonates?
alcohol and sugar free | phenobarbital elixer contains 40% alcohol!= 2 glasses of wine in adult
28
what could be the issue with liquid formulation?
allergy to colourings, flavourings, preservatives
29
Can taste of solid formulation be disguised or masked in food, juice, milk etc.?
should NEVER add drugs to baby's feeding bottle- we dont know how much they are going to drink! what % of the dose they took? disliking of the food bc of the drug inside should use minimum amount of food or drink
30
Paediatric Pharmacokinetics - absorption w oral
- neutral gastric ph at birth: increase ab for penecillin (WB) unionsed drug, faster ab decrease ab for phenytoin, phenobarbital (WA) ionised drug, reduced ab - reduced paristalsis and prolonged gastric emptying, slower ab
31
what is Phenylketonuria? is it inherited? is it common?
inherited common metabolic disease inv phenyalanine hydroxylase (PAH) deficiency, this enzyme convert S-Phe to S-Try
32
what is the result of PKU?
increased S-Phe (accumulation): >800microM | decreased S-Tyr in cells. this causes neurological deficit such as ADHD, mental retartdation
33
what is the normal serum conc range for S-Phe?
20-150 microM
34
what is the normal metabolic pathway for S-Phe? where does it occur? what does the product convert into?
in liver, S-Phe --> S-Tyr, tyrosine makes amine NTs such as L-DOPA->DA->NA->A also thyroxine
35
to what extend does S-Phe converts into S-Tyr
90%
36
what type and structure of enzyme is phenyalanine hydroxylase?
allosteric enzyme | tetramer
37
where on PAH does the S-Phe bind?
S-Phe binds to orthosteric sites of PAH | BH4 (co-sub) binds to allosteric site
38
what procedure all new borns have to go through regards to PKU?
screening for PKU
39
what methods do they use in PKU screening? what they trying to find?
MS-MS to quantify serum S-Phe concentration, S-Tyr conc, and BH4 conc (so-sub for PHA)
40
what are the consequences of PKU?not only mental retardation ...
1. irreversible mental retardation or small brain 2. increase ROS- reactive oxygen species 3. reduced monoamine NTs eg NA, DA, A -mood changes 4. change in NO level (a NT) 5. hypopigmentation 6. more porn to fractures (malnutrition?)
41
what is the standard trt for PKU? | how long does the trt last?
restrict S-Phe dietary intake, avoid aspartame! | lifelong trt!
42
what food has high s-phe? | what other food has high aspartame that PKU pt cant have?
food that is rich in protein (AA) = meat | artificial sweetener containing S-Phe
43
what supplement does PKU pt have to take?
S-Tyr and other AA supplements
44
what is the MOA of large neutral AA?
increase neutral AA eg tyrosine, tryptophan | reduce S-Phe ABSORPTION from digestion
45
what is the LNAA that we used which is derived from milk? why?
glycomacropeptide= naturally low in S-Phe, high in LNAA
46
what is the benefit of LNAA?
improved executive function (more DA in mesocortical pathway?) but only a prob in pt w poor compliance to diet
47
what is the BH4 supplement for PKU?
sapropterin
48
Sapropterin works in what two type of pt?
1. mild form of PKU, PAH still retain some activity | 2. gene mutation inv reduction of BH4 (co-sub) binding to PHA so increase BH4 will increase enzyme activity
49
how does the gene therapy work for PKU pt?
- REPLACE missing gene for enzyme (PAH) | - use vector AAV for delivery
50
drawback for gene therapy for PKU?
- become ineffective in a few w, due to IMR to vector | - gender specific (works better in male)
51
how does the enzyme therapy work for PKU pt? TWO method
- DIRECTLY REPLACE missing enzyme (PAH) but enzyme complex nature, diff to delivery to target tissue/cells - alternative enzyme used: bacteria PAL- phenylalanine AMMONIA lyase - PAL degrades S-Phe form NH3 (ammonia) which can be excreted in urine
52
drawback for PAL therapy for PKU?
- an enzyme so susceptible for GI degradation by protease - have to use IV - this can be expensive, diff process - gender specific
53
how does probiotics trt works for PKU?
- live bac with desired enzymes - survive acidic stomach, - lysis in small intestine (alkali ph) - release enzyme - reduce S-Phe level in GIT (from diet)
54
what result can sapropterin achiveve for PKU pt?
- less restricted diet | - complete discont. of dietary req!
55
what is safeguarding?
safeguarding is to protect people's health, rights, let them avoid harm, abuse, neglect
56
who do we need to safeguard the most?
children and vulnerable adults
57
define vulnerable adults?
adults at risk of abuse or neglect because of their needs for care (eg lack of mental capacity)
58
what are the classified types of abuse?
Physical, psychological, sexual, financial, salvary, discrimination, organisational, neglect, self-neglect Domestic – physical psychologial sexual and financial
59
What to do if you suspect/ identify a safeguarding issue
is it an emergency? yes- call police 999 | no- refer social service/ seek further advice
60
where to seek for further advice if safeguarding issue identifed?
- child protection professionals (local) - child or vulnerable adult's GP - colleagues - NSPCC (national society for prevention of cruelty to children)
61
what to do after referring social service by tel?
If you refer by telephone, confirm the referral in writing within 48 hours using standard local referral forms - make record of concern, action taken, decisions

SIU6 Special patient group (6 decks)

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