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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
Nephrotic Syndrome what do you use in steroid resistant children
ACEi and immunosuppressants (cyclosporine, tacrolimus, rituximab)
26
Nephrotic Syndrome complications: why does hypovolaemia occur
fluid leaks from the intravascular space into the interstitial space causing oedema and low BP
27
Nephrotic Syndrome complications: why can thrombosis occur
proteins that normally prevent blood clotting are lost in the kidneys also liver responds to the low albumin by producing pro-thrombotic proteins
28
Nephrotic Syndrome complications: why can infection occur
the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond medications also suppress immune system
29
Nephrotic Syndrome complications: hypovolaemia, thrombosis, infection and ________
- acute or chronic renal failure | - relapse
30
Polycystic Kidney Disease what are the 2 types
autosomal recessive polycystic kidney disease (ARPKD) Autosomal dominant ADPKD
31
Polycystic Kidney Disease when does ARPKD present
in neonates | usually picled up on antenatal USS
32
ARPKD where is the mutation
in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6
33
ARPKD what does the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome code for
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.
34
ARPKD 3 features
- Cystic enlargement of the renal collecting ducts - Oligohydramnios, pulmonary hypoplasia and Potter syndrome - Congenital liver fibrosis
35
ARPKD what is seen on antenatal scans
oligohydramnios and polycystic kidneys
36
ARPKD what is oligohydramnios
a lack of amniotic fluid caused by reduced urine production by the fetus
37
ARPKD what does a lack of amniotic fluid (oligohydramnios) lead to
Potter syndrome
38
ARPKD what is Potter syndrome characterised by
dysmorphic features: - underdeveloped ear cartilage - low set ears - flat nasal bridge - abnormalities of the skeleton
39
ARPKD why may there be resp failure shortly after birth
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
ARPKD what mnx may be required in first few days of life
renal dialysis
41
ARPKD what do most patients develop before reaching adulthood
end stage renal failure
42
ARPKD what ongoing problems may pts have throughout life
- Liver failure due to liver fibrosis - Portal hypertension leading to oesophageal varices - Progressive renal failure - Hypertension due to renal failure - Chronic lung disease
43
ARPKD prognosis
1/3 die in neontal period 1/3 will survive to adulthood
44
Multicystic Dysplastic Kidney what is it
one of the baby’s kidneys is made up of many cysts while the other kidney is normal
45
Multicystic Dysplastic Kidney when is it diagnosed
on antenatal ultrasound scans.
46
Multicystic Dysplastic Kidney prognosis
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
Multicystic Dysplastic Kidney what is the pt at risk of later in life
- UTIs - HTN - CKD
48
Multicystic Dysplastic Kidney trx
none follow up renal USS prophylactic abx for recurrent UTIs
49
Posterior Urethral Valve what is it
tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output
50
Posterior Urethral Valve who does it occur in
newborn boys
51
Posterior Urethral Valve how does it cause hydronephrosis
obstruction to outflow of urine back pressure into bladder, ureters and up the kidneys
52
Posterior Urethral Valve why is there an increased risk of UTIs
restriction in the outflow of urine prevents bladder from fully emptying leading to a reservoir of urine
53
Posterior Urethral Valve presentation
- Difficulty urinating - Weak urinary stream - Chronic urinary retention - Palpable bladder - Recurrent UTIs - Impaired kidney function
54
Posterior Urethral Valve how may severe cases present as
bilateral hydronephrosis and oligohydramnios leading to underdeveloped fetal lungs (pulmonary hypoplasia)
55
Posterior Urethral Valve how may severe cases present on antenatal scans
oligohydramnios and hydronephrosis
56
Posterior Urethral Valve what may abdo US show in young boys
enlarged, thickened bladder and bilateral hydronephrosis
57
Posterior Urethral Valve what may Micturating cystourethrogram (MCUG) show in young boys
shows the location of the extra urethral tissue and reflux of urine back into the bladder
58
Posterior Urethral Valve why is cytoscopy used
to ablate or remove the extra tissue.
59
Posterior Urethral Valve what is the definitive mnx
ablation or removal of the extra urethral tissue, usually during cystoscopy.
60
Posterior Urethral Valve what can be inserted to bypass the valve whilst awaiting definitive management
a temporary urinary catheter
61
Wilms Tumour what is it
a specific type of tumour affecting the kidney in children, typically under the age of 5 years.
62
Wilms Tumour presentation
- mass in abdomen in child <5y - abdo pain - haematuria - lethargy - fever - hypertension - weight loss
63
Wilms Tumour initial inx
- US
64
Wilms Tumour inx to stage tumour
CT or MRI
65
Wilms Tumour definitive inx for diagnosis
biopsy to identify the histology
66
Wilms Tumour mnx
- surgical excision of tumour and nephrectomy | - adjuvant chemo/radio
67
Wilms Tumour prognosis
Early stage tumours with favourable histology hold a good chance of cure (up to 90%). Metastatic disease has a poorer prognosis.
68
Undescended Testes how do testes usually develop
- 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
Undescended Testes what are they
when the testes have not made it out of the abdomen by birth. aka cryptorchidism
70
Undescended Testes Undescended testes in older children or after puberty hold a higher risk of?
testicular torsion infertility testicular cancer.
71
Undescended Testes RFs (5)
- FH of undescended testes - Low birth weight - Small for gestational age - Prematurity - Maternal smoking during pregnancy
72
Undescended Testes at what age should they be seen by a paediatric urologist
6 months
73
Undescended Testes when should Orchidopexy (surgical correction of undescended testes) be carried out
between 6 and 12 months of age.
74
Undescended Testes what is retractile testicles
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
Enuresis what is it
involuntary urination
76
Enuresis what is the name for bed wetting
nocturnal enuresis
77
Enuresis what is the name for inability to control bladder function during the day
diurnal enuresis
78
Enuresis when do most children et control of daytime urination and nighttime urination
day: 2y night: 3-4y
79
Enuresis what is primary nocturnal enuresis
the child has never managed to be consistently dry at night.
80
Enuresis most common cause of primary nocturnal enuresis
a variation on normal development . Often patients will have a family history of delayed dry nights.
81
Enuresis causes of primary nocturnal enuresis
- overactive bladder - fluid intake - failure to wake - psychological distress - secondary causes: chronic constipation, UTI, learning disability, cerebral palsy
82
Enuresis mnx for primary nocturnal enuresis if <5y
reassure parents that it is likely to resolve without any trx
83
Enuresis mnx of primary nocturnal enuresis >5y
- 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
Enuresis what is secondary nocturnal enuresis
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
Enuresis causes of secondary nocturnal enuresis
- UTI - Constipation - Type 1 diabetes - New psychosocial problems (e.g. stress in family or school life) - Maltreatment (abuse. safeguarding)
86
Enuresis types of diurnal enuresis
- urge incontinence | - stress incontinence
87
Enuresis causes of diurnal enuresis
- recurrent UTIs - psychosocial problems - constipation
88
Enuresis what is an enuresis alarm
a device that makes a noise at the first sign of bed wetting
89
Enuresis pharmacological trx
- desmopressin (ADH) - oxybutynin - imipramine (TCA)
90
why may constipation cause urinary sx
non-voided stools in the rectum press against the bladder, decreasing its ability to hold urine

Finals (47 decks)

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