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Finals > Paeds: Renal & Urology > Flashcards

Paeds: Renal & Urology Flashcards

1
Q

Haemolytic Uraemic Syndrome

what is the classic triad

A
  1. haemolytic anaemia
  2. AKI
  3. thrombocytopenia
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2
Q

Haemolytic Uraemic Syndrome

what is it

A

thrombosis within small blood vessels throughout the body

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3
Q

Haemolytic Uraemic Syndrome

what is it triggered by

A

shiga toxin produced by e.coli 0157

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4
Q

Haemolytic Uraemic Syndrome

what trx increases the risk of developing HUS

A

abx and anti-motility medications (loperamide) to treat gastroenteritis

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5
Q

Haemolytic Uraemic Syndrome

signs and sx

A
  • reduced urine output
  • haematuria or dark brown urine
  • abdo pain
  • lethargy + irritability
  • confusion
  • oedema
  • hypertension
  • bruising
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6
Q

Haemolytic Uraemic Syndrome

mnx

A

medial emergency

supportive:

  • urgent referral to paed renal unit for renal dialysis if required
  • antihypertensives if required
  • careful maintenance o fluid balance
  • blood transfusions if required
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7
Q

Nephrotic Syndrome

why does it occur

A

the BM in the glomerulus becomes highly permeable to protein

allowing proteins to leak from the blood into the urine

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8
Q

Nephrotic Syndrome

whom is it most common in

A

2-5yr olds

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9
Q

Nephrotic Syndrome

classic triad of features

A
  1. proteinuria (>3 on urine dipstick)
  2. hypoalbuminaemia
  3. oedema
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10
Q

Nephrotic Syndrome

what may be seen in the urine

A

frothy urine

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11
Q

Nephrotic Syndrome

what would the lipid profile show

A
  • high cholesterol
  • high triglycerides
  • low density lipoproteins
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12
Q

Nephrotic Syndrome

what would the blood pressure be

A

high

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13
Q

Nephrotic Syndrome

blood coagulability: high or low

A

high, with an increased tendency to form blood clots

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14
Q

Nephrotic Syndrome

most common cause in children

A

minimal change disease

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15
Q

Nephrotic Syndrome

intrinsic kidney disease causes

A
  • focal segmental glomerulosclerosis

- membranoproliferative glomerulonephritis

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16
Q

Nephrotic Syndrome

systemic illness causes

A
  • henoch schonlein purpura
  • diabetes
  • infection: hepatitis, malaria
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17
Q

Nephrotic Syndrome

is renal biopsy and standard microscopy able to detect minimal change disease

A

no

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18
Q

Nephrotic Syndrome

minimal change disease: what will urinalysis show

A

small molecular weight proteins

hyaline casts

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19
Q

Nephrotic Syndrome

minimal change disease: mnx

A

corticosteroids (i.e. prednisolone)

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20
Q

Nephrotic Syndrome

General mnx

A
  • high dose steroids
  • low salt diet
  • diuretics
  • albumin infusions
  • abx prophylaxis if severe
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21
Q

Nephrotic Syndrome

how long are high dose steroids given for

A

4w and then gradually weaned over the next 8w

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22
Q

Nephrotic Syndrome

what does steroids sensitive mean

A

children who respond to steroids

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23
Q

Nephrotic Syndrome

what does steroids dependent mean

A

patients that struggle to wean off steroids due to relapses

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24
Q

Nephrotic Syndrome

what does steroids resistant mean

A

pts that do not response to steroids

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25
Q

Nephrotic Syndrome

what do you use in steroid resistant children

A

ACEi and immunosuppressants (cyclosporine, tacrolimus, rituximab)

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26
Q

Nephrotic Syndrome

complications: why does hypovolaemia occur

A

fluid leaks from the intravascular space into the interstitial space causing oedema and low BP

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27
Q

Nephrotic Syndrome

complications: why can thrombosis occur

A

proteins that normally prevent blood clotting are lost in the kidneys

also liver responds to the low albumin by producing pro-thrombotic proteins

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28
Q

Nephrotic Syndrome

complications: why can infection occur

A

the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond

medications also suppress immune system

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29
Q

Nephrotic Syndrome

complications: hypovolaemia, thrombosis, infection and ________

A
  • acute or chronic renal failure

- relapse

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30
Q

Polycystic Kidney Disease

what are the 2 types

A

autosomal recessive polycystic kidney disease (ARPKD)

Autosomal dominant ADPKD

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31
Q

Polycystic Kidney Disease

when does ARPKD present

A

in neonates

usually picled up on antenatal USS

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32
Q

ARPKD

where is the mutation

A

in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6

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33
Q

ARPKD

what does the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome code for

A

fibrocystin/polyductin protein complex (FPC)

which is responsible for the creation of tubules and the maintenance of healthy epithelial tissue in the kidneys, liver and pancreas.

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34
Q

ARPKD

3 features

A
  • Cystic enlargement of the renal collecting ducts
  • Oligohydramnios, pulmonary hypoplasia and Potter syndrome
  • Congenital liver fibrosis
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35
Q

ARPKD

what is seen on antenatal scans

A

oligohydramnios and polycystic kidneys

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36
Q

ARPKD

what is oligohydramnios

A

a lack of amniotic fluid caused by reduced urine production by the fetus

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37
Q

ARPKD

what does a lack of amniotic fluid (oligohydramnios) lead to

A

Potter syndrome

38
Q

ARPKD

what is Potter syndrome characterised by

A

dysmorphic features:

  • underdeveloped ear cartilage
  • low set ears
  • flat nasal bridge
  • abnormalities of the skeleton
39
Q

ARPKD

why may there be resp failure shortly after birth

A

oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia), resulting in respiratory failure

large cystic kidneys take up space in the abdomen so hard to breathe adequately.

40
Q

ARPKD

what mnx may be required in first few days of life

A

renal dialysis

41
Q

ARPKD

what do most patients develop before reaching adulthood

A

end stage renal failure

42
Q

ARPKD

what ongoing problems may pts have throughout life

A
  • Liver failure due to liver fibrosis
  • Portal hypertension leading to oesophageal varices
  • Progressive renal failure
  • Hypertension due to renal failure
  • Chronic lung disease
43
Q

ARPKD

prognosis

A

1/3 die in neontal period

1/3 will survive to adulthood

44
Q

Multicystic Dysplastic Kidney

what is it

A

one of the baby’s kidneys is made up of many cysts while the other kidney is normal

45
Q

Multicystic Dysplastic Kidney

when is it diagnosed

A

on antenatal ultrasound scans.

46
Q

Multicystic Dysplastic Kidney

prognosis

A

Usually the single healthy kidney is sufficient to lead a normal life. Often the cystic kidney will atrophy and disappear before 5 years of age.

47
Q

Multicystic Dysplastic Kidney

what is the pt at risk of later in life

A
  • UTIs
  • HTN
  • CKD
48
Q

Multicystic Dysplastic Kidney

trx

A

none

follow up renal USS

prophylactic abx for recurrent UTIs

49
Q

Posterior Urethral Valve

what is it

A

tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output

50
Q

Posterior Urethral Valve

who does it occur in

A

newborn boys

51
Q

Posterior Urethral Valve

how does it cause hydronephrosis

A

obstruction to outflow of urine

back pressure into bladder, ureters and up the kidneys

52
Q

Posterior Urethral Valve

why is there an increased risk of UTIs

A

restriction in the outflow of urine prevents bladder from fully emptying

leading to a reservoir of urine

53
Q

Posterior Urethral Valve

presentation

A
  • Difficulty urinating
  • Weak urinary stream
  • Chronic urinary retention
  • Palpable bladder
  • Recurrent UTIs
  • Impaired kidney function
54
Q

Posterior Urethral Valve

how may severe cases present as

A

bilateral hydronephrosis and oligohydramnios

leading to underdeveloped fetal lungs (pulmonary hypoplasia)

55
Q

Posterior Urethral Valve

how may severe cases present on antenatal scans

A

oligohydramnios and hydronephrosis

56
Q

Posterior Urethral Valve

what may abdo US show in young boys

A

enlarged, thickened bladder and bilateral hydronephrosis

57
Q

Posterior Urethral Valve

what may Micturating cystourethrogram (MCUG) show in young boys

A

shows the location of the extra urethral tissue and reflux of urine back into the bladder

58
Q

Posterior Urethral Valve

why is cytoscopy used

A

to ablate or remove the extra tissue.

59
Q

Posterior Urethral Valve

what is the definitive mnx

A

ablation or removal of the extra urethral tissue, usually during cystoscopy.

60
Q

Posterior Urethral Valve

what can be inserted to bypass the valve whilst awaiting definitive management

A

a temporary urinary catheter

61
Q

Wilms Tumour

what is it

A

a specific type of tumour affecting the kidney in children, typically under the age of 5 years.

62
Q

Wilms Tumour

presentation

A
  • mass in abdomen in child <5y
  • abdo pain
  • haematuria
  • lethargy
  • fever
  • hypertension
  • weight loss
63
Q

Wilms Tumour

initial inx

A
  • US
64
Q

Wilms Tumour

inx to stage tumour

A

CT or MRI

65
Q

Wilms Tumour

definitive inx for diagnosis

A

biopsy to identify the histology

66
Q

Wilms Tumour

mnx

A
  • surgical excision of tumour and nephrectomy

- adjuvant chemo/radio

67
Q

Wilms Tumour

prognosis

A

Early stage tumours with favourable histology hold a good chance of cure (up to 90%).

Metastatic disease has a poorer prognosis.

68
Q

Undescended Testes

how do testes usually develop

A
  • testes develop in the abdomen
  • then gradually migrate down, through the inguinal canal and into the scrotum.
  • They have normally reached the scrotum prior to birth.
69
Q

Undescended Testes

what are they

A

when the testes have not made it out of the abdomen by birth.

aka cryptorchidism

70
Q

Undescended Testes

Undescended testes in older children or after puberty hold a higher risk of?

A

testicular torsion

infertility

testicular cancer.

71
Q

Undescended Testes

RFs (5)

A
  • FH of undescended testes
  • Low birth weight
  • Small for gestational age
  • Prematurity
  • Maternal smoking during pregnancy
72
Q

Undescended Testes

at what age should they be seen by a paediatric urologist

A

6 months

73
Q

Undescended Testes

when should Orchidopexy (surgical correction of undescended testes) be carried out

A

between 6 and 12 months of age.

74
Q

Undescended Testes

what is retractile testicles

A

the testes moves out of scrotum into the inguinal canal when it is cold or the cremasteric reflex is activated

it is normal in boys that have not reached puberty

75
Q

Enuresis

what is it

A

involuntary urination

76
Q

Enuresis

what is the name for bed wetting

A

nocturnal enuresis

77
Q

Enuresis

what is the name for inability to control bladder function during the day

A

diurnal enuresis

78
Q

Enuresis

when do most children et control of daytime urination and nighttime urination

A

day: 2y
night: 3-4y

79
Q

Enuresis

what is primary nocturnal enuresis

A

the child has never managed to be consistently dry at night.

80
Q

Enuresis

most common cause of primary nocturnal enuresis

A

a variation on normal development

. Often patients will have a family history of delayed dry nights.

81
Q

Enuresis

causes of primary nocturnal enuresis

A
  • overactive bladder
  • fluid intake
  • failure to wake
  • psychological distress
  • secondary causes: chronic constipation, UTI, learning disability, cerebral palsy
82
Q

Enuresis

mnx for primary nocturnal enuresis if <5y

A

reassure parents that it is likely to resolve without any trx

83
Q

Enuresis

mnx of primary nocturnal enuresis >5y

A
  • 2w toilet + fluid diary
  • lifestyle: reduce fluids in evening, pass urine before bed, easy access to toilet
  • encouragement + positive reinforcement
  • trx underlying cause e.g. constipation
  • enuresis alarms
  • pharmacological trx
84
Q

Enuresis

what is secondary nocturnal enuresis

A

where a child begins wetting the bed when they have previously been dry for at least 6 months.

more indicative of an underlying illness

85
Q

Enuresis

causes of secondary nocturnal enuresis

A
  • UTI
  • Constipation
  • Type 1 diabetes
  • New psychosocial problems (e.g. stress in family or school life)
  • Maltreatment (abuse. safeguarding)
86
Q

Enuresis

types of diurnal enuresis

A
  • urge incontinence

- stress incontinence

87
Q

Enuresis

causes of diurnal enuresis

A
  • recurrent UTIs
  • psychosocial problems
  • constipation
88
Q

Enuresis

what is an enuresis alarm

A

a device that makes a noise at the first sign of bed wetting

89
Q

Enuresis

pharmacological trx

A
  • desmopressin (ADH)
  • oxybutynin
  • imipramine (TCA)
90
Q

why may constipation cause urinary sx

A

non-voided stools in the rectum press against the bladder, decreasing its ability to hold urine

Finals (47 decks)

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