paediatric clinical chem Flashcards

1
Q

what are the common problems of LBW babies

A

resp distress syndrome (RDS)
retinopathy of prematurity - SE of oxygen toxicity
intraventricular haemorrhage
patent ductus arteriosus
necrotising enterocolitis

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2
Q

what is respiratory distress syndrome

A

breathing problem
common less than 34wks
lack surfactant

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3
Q

rx of RDS

A

surfactant
oxygen
mechanical ventilation

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4
Q

what is intraventricular haemorrhage

A

usually happens in 1st 3 days
most are mild and self resolve
some cause pressure -> brain damage
then need to drain blood/use med to reduce fluid build up

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5
Q

when does NEC normally develop

A

2-3 wks after birth

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6
Q

what is retinopathy of prematurity

A

an abnormal growth of blood vessels in the eye -> vision loss.
less than 32 weeks.
Most cases heal themselves with little or no vision loss.
In severe cases - laser or with cryotherapy (freezing) to preserve vision.

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7
Q

what is NEC

A

inflammation bowel wall -> necrosis and perf

bloody stools

abdo distension

intramural air

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8
Q

when does renal function develop

A

nephrons from wk 6
produce urine from wk 10
full complement from wk 36

functional maturity of GFR not reached until 2yrs

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9
Q

consequence of renal function developing late

A

low GFR for surface area ->
* slow excretion of solute load
* limited Na for H+ change -> acidosis

short prox tubule ->
* lower reabsorptive capacity (although adequate for the small filtered load)
* renal threshold for glycosuria is lower – plasma glucose >7
* Reabsorption of bicarb is not as effective -> risk of acidosis

Loops of Henle/distal collecting ducts are short and juxtaglomerular ->
* reduced concentrating ability with a maximum urine osmolality of 700 mmol/kg

Distal tubule is relatively unresponsive to aldosterone:
* leads to a persistent loss of sodium of c.1.8 mmol/kg/day
* -> reduced K secretion

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10
Q

proportion of ECF in adult, term baby and prem

A

Babies more extracellular fluid than an adult does
When born - Pul resistance goes down - Release AMP – redistribution of fluid -> wht loss in 1st wk of life.

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11
Q

how much wht loss is allowed in the 1st week of life

A

10%

should regain by day 7-10

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12
Q

na and k requirements in neonates

A

Requirements build up with days

Na higher when less than 30wks – because of leak from the kidney – persistent Na loss

Check K daily in prem infant when we give fluids

Don’t rely on spot urine in neonate

Only give K after achieved a urine output – if give before then you risk hypernatraemia and all those difficulties

They need about 6x more fluid than adult etc

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13
Q

causes of electolyte disturbances in neonates

A

high water loss from high
* surface area
* skin blood flow
* metabolic/resp rate
* transepidermal fluid loss (skin isnt keritinised at birth)

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14
Q

drug causes of electrolyte disturbance in neonates

A

Bicarbonate (for acidosis)
* High Na content

Antibiotics
* high Na content

Caffeine/theophylline (for apnoea)
* Renal Na loss

Indomethacin (forPDA)
* oliguria

note: if keep replacing Na you risk hypernatraemia

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15
Q

causes of hypernatraemia in neonates

A

rare after 2 wks - normally due to dehydration
rarely - salt poisening and osmoregulatory dysfunction - consider if repeated w/o obv cause

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16
Q

how do you differentiate rare causes of hypernatraemia in the neonate

A

Routine measurement of urea, creatinine and electrolytes on paired urine and plasma on admission

17
Q

pathway of aldosterone production

18
Q

cause of hyponatraemia in neonate

A

rare
congenital adrenal hyperplasia
loss of 21-hydroxylase
* -> low aldosterone
-> Na loss (hypoNa)

  • also loss of cortisol -> hypoglycaemia
  • high level precursers - high androgens
    -> ambiguous genitalia
    growth accelaration - rare presentation
19
Q

physiological causes of high BR in neonate

A

ucBR
* High level of synthesis (rbc breakdown)
* Low rate of transport into liver
* Enhanced enterohepatic circulation

20
Q

problem with high BR in neonate

A

1g/L albumin binds 10micromol/L BR
Average albumin at term 34 g/l (lower in prem)
Free bilirubin crosses the blood brain barrier and causes Kernicterus (bilirubin encephalopathy)
in prem the levels for exchange transfusion and phototherapy are much lower

21
Q

causes of high BR less than 24hrs

A

Haemolytic disease (ABO, rhesus etc)
G-6-PD deficiency
Crigler-Najjar syndrome – metabolic high BR, deficiency of conjugation

22
Q

definition of prolonged jaundice

A

jaundice that lasts for more than
* 14 days in term babies
* 21 days in preterm babies.

23
Q

causes of prolonged jaundice

A

Prenatal infection/sepsis/hepatitis
Hypothyroidism - screened in Guthrie
Breast milk jaundice

24
Q

causes of raised cBR >20umol/L

A

always pathological

biliary atresia

ascending cholangitis in TPN - related to lipid content
metabolic disorder
* galactosaemia,
* alpha-1-AT deficiency
* tyrosinaemia 1 - plasma amino acids
* peroxisomal dis - very long chain fatty acid profile

25
associations and rx of biliary atresia
cardiac malformations, polysplenia, sinus inversus rx - surgery less than 6mo *most common cause of high cBR*
26
what is galactasaemia
urine reducing substances, red cell Gal-1-PUT
27
calcium and phos in fetus
Ca and phos laid down in last trimester of pregnancy PTH like activity in placenta – baby acquire ca and phos at mothers expense
28
ca and phos in neonate
**At birth all ca will fall – kick start the parathyroid hormone** Therefore lower limit for ca is much lower than in adults Phosphate is higher – **babies good at reabsorbing phosphate**
29
bone features of osteopenia of prematurity
fraying, splaying and cupping of long bones
30
biochem features of osteopenia of prematurity
Ca normal - last thing to change phos - low Alk phos - higher then adult ULN
31
Rx of osteopenia of prematuritty
Phosphate / calcium supplements (1 alpha calcidol) *cant give Ca and phos at the same time*
32
what is rickets
osteopenia due to deficiency of vit D
33
presentation of rickets
Frontal bossing Bowlegs/knock knees Muscular hypotonia Tetany / hypocalcaemic seizure Hypocalcaemic cardiomyopathy
34
transient hyperphosphatasaemia of infancy
benign distinguishable from rickets by electrophoresis
35
genetic causes of rickets
Pseudo vitamin D deficiency I * Defective renal hydroxylation Pseudo vitamin D deficiency II * Receptor defect Familial Hypophosphataemias * Low tubular maximum reabsorption of phosphate * Raised urine phosphoethanolamine