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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
Q

what could be the problems in IV formulation?

A

contraindicated excipents due to poor metabolism eg Benzyl alcohol
displacement value

26
Q

can we administer a fraction of the retal suppositries?

what is the other drawback?

A

no, cant assume homogenous distribution of drug throughout suppository
very expensive

27
Q

what are the general requiements for liqid formulation for neonates?

A

alcohol and sugar free

phenobarbital elixer contains 40% alcohol!= 2 glasses of wine in adult

28
Q

what could be the issue with liquid formulation?

A

allergy to colourings, flavourings, preservatives

29
Q

Can taste of solid formulation be disguised or masked in food, juice, milk etc.?

A

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
Q

Paediatric Pharmacokinetics - absorption w oral

A
  • 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
Q

what is Phenylketonuria? is it inherited? is it common?

A

inherited common metabolic disease inv phenyalanine hydroxylase (PAH) deficiency, this enzyme convert S-Phe to S-Try

32
Q

what is the result of PKU?

A

increased S-Phe (accumulation): >800microM

decreased S-Tyr in cells. this causes neurological deficit such as ADHD, mental retartdation

33
Q

what is the normal serum conc range for S-Phe?

A

20-150 microM

34
Q

what is the normal metabolic pathway for S-Phe? where does it occur? what does the product convert into?

A

in liver, S-Phe –> S-Tyr,
tyrosine makes amine NTs such as L-DOPA->DA->NA->A
also thyroxine

35
Q

to what extend does S-Phe converts into S-Tyr

A

90%

36
Q

what type and structure of enzyme is phenyalanine hydroxylase?

A

allosteric enzyme

tetramer

37
Q

where on PAH does the S-Phe bind?

A

S-Phe binds to orthosteric sites of PAH

BH4 (co-sub) binds to allosteric site

38
Q

what procedure all new borns have to go through regards to PKU?

A

screening for PKU

39
Q

what methods do they use in PKU screening? what they trying to find?

A

MS-MS to quantify serum S-Phe concentration, S-Tyr conc, and BH4 conc (so-sub for PHA)

40
Q

what are the consequences of PKU?not only mental retardation …

A
  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
Q

what is the standard trt for PKU?

how long does the trt last?

A

restrict S-Phe dietary intake, avoid aspartame!

lifelong trt!

42
Q

what food has high s-phe?

what other food has high aspartame that PKU pt cant have?

A

food that is rich in protein (AA) = meat

artificial sweetener containing S-Phe

43
Q

what supplement does PKU pt have to take?

A

S-Tyr and other AA supplements

44
Q

what is the MOA of large neutral AA?

A

increase neutral AA eg tyrosine, tryptophan

reduce S-Phe ABSORPTION from digestion

45
Q

what is the LNAA that we used which is derived from milk? why?

A

glycomacropeptide= naturally low in S-Phe, high in LNAA

46
Q

what is the benefit of LNAA?

A

improved executive function (more DA in mesocortical pathway?) but only a prob in pt w poor compliance to diet

47
Q

what is the BH4 supplement for PKU?

A

sapropterin

48
Q

Sapropterin works in what two type of pt?

A
  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
Q

how does the gene therapy work for PKU pt?

A
  • REPLACE missing gene for enzyme (PAH)

- use vector AAV for delivery

50
Q

drawback for gene therapy for PKU?

A
  • become ineffective in a few w, due to IMR to vector

- gender specific (works better in male)

51
Q

how does the enzyme therapy work for PKU pt? TWO method

A
  • 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
Q

drawback for PAL therapy for PKU?

A
  • an enzyme so susceptible for GI degradation by protease
  • have to use IV
  • this can be expensive, diff process
  • gender specific
53
Q

how does probiotics trt works for PKU?

A
  • 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
Q

what result can sapropterin achiveve for PKU pt?

A
  • less restricted diet

- complete discont. of dietary req!

55
Q

what is safeguarding?

A

safeguarding is to protect people’s health, rights, let them avoid harm, abuse, neglect

56
Q

who do we need to safeguard the most?

A

children and vulnerable adults

57
Q

define vulnerable adults?

A

adults at risk of abuse or neglect because of their needs for care (eg lack of mental capacity)

58
Q

what are the classified types of abuse?

A

Physical, psychological, sexual, financial, salvary, discrimination, organisational, neglect, self-neglect
Domestic – physical psychologial sexual and financial

59
Q

What to do if you suspect/ identify a safeguarding issue

A

is it an emergency? yes- call police 999

no- refer social service/ seek further advice

60
Q

where to seek for further advice if safeguarding issue identifed?

A
  • child protection professionals (local)
  • child or vulnerable adult’s GP
  • colleagues
  • NSPCC (national society for prevention of cruelty to children)
61
Q

what to do after referring social service by tel?

A

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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