Paeds: Renal & Urology Flashcards
Haemolytic Uraemic Syndrome
what is the classic triad
- haemolytic anaemia
- AKI
- thrombocytopenia
Haemolytic Uraemic Syndrome
what is it
thrombosis within small blood vessels throughout the body
Haemolytic Uraemic Syndrome
what is it triggered by
shiga toxin produced by e.coli 0157
Haemolytic Uraemic Syndrome
what trx increases the risk of developing HUS
abx and anti-motility medications (loperamide) to treat gastroenteritis
Haemolytic Uraemic Syndrome
signs and sx
- reduced urine output
- haematuria or dark brown urine
- abdo pain
- lethargy + irritability
- confusion
- oedema
- hypertension
- bruising
Haemolytic Uraemic Syndrome
mnx
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
Nephrotic Syndrome
why does it occur
the BM in the glomerulus becomes highly permeable to protein
allowing proteins to leak from the blood into the urine
Nephrotic Syndrome
whom is it most common in
2-5yr olds
Nephrotic Syndrome
classic triad of features
- proteinuria (>3 on urine dipstick)
- hypoalbuminaemia
- oedema
Nephrotic Syndrome
what may be seen in the urine
frothy urine
Nephrotic Syndrome
what would the lipid profile show
- high cholesterol
- high triglycerides
- low density lipoproteins
Nephrotic Syndrome
what would the blood pressure be
high
Nephrotic Syndrome
blood coagulability: high or low
high, with an increased tendency to form blood clots
Nephrotic Syndrome
most common cause in children
minimal change disease
Nephrotic Syndrome
intrinsic kidney disease causes
- focal segmental glomerulosclerosis
- membranoproliferative glomerulonephritis
Nephrotic Syndrome
systemic illness causes
- henoch schonlein purpura
- diabetes
- infection: hepatitis, malaria
Nephrotic Syndrome
is renal biopsy and standard microscopy able to detect minimal change disease
no
Nephrotic Syndrome
minimal change disease: what will urinalysis show
small molecular weight proteins
hyaline casts
Nephrotic Syndrome
minimal change disease: mnx
corticosteroids (i.e. prednisolone)
Nephrotic Syndrome
General mnx
- high dose steroids
- low salt diet
- diuretics
- albumin infusions
- abx prophylaxis if severe
Nephrotic Syndrome
how long are high dose steroids given for
4w and then gradually weaned over the next 8w
Nephrotic Syndrome
what does steroids sensitive mean
children who respond to steroids
Nephrotic Syndrome
what does steroids dependent mean
patients that struggle to wean off steroids due to relapses
Nephrotic Syndrome
what does steroids resistant mean
pts that do not response to steroids
Nephrotic Syndrome
what do you use in steroid resistant children
ACEi and immunosuppressants (cyclosporine, tacrolimus, rituximab)
Nephrotic Syndrome
complications: why does hypovolaemia occur
fluid leaks from the intravascular space into the interstitial space causing oedema and low BP
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
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
Nephrotic Syndrome
complications: hypovolaemia, thrombosis, infection and ________
- acute or chronic renal failure
- relapse
Polycystic Kidney Disease
what are the 2 types
autosomal recessive polycystic kidney disease (ARPKD)
Autosomal dominant ADPKD
Polycystic Kidney Disease
when does ARPKD present
in neonates
usually picled up on antenatal USS
ARPKD
where is the mutation
in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6
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.
ARPKD
3 features
- Cystic enlargement of the renal collecting ducts
- Oligohydramnios, pulmonary hypoplasia and Potter syndrome
- Congenital liver fibrosis
ARPKD
what is seen on antenatal scans
oligohydramnios and polycystic kidneys
ARPKD
what is oligohydramnios
a lack of amniotic fluid caused by reduced urine production by the fetus
ARPKD
what does a lack of amniotic fluid (oligohydramnios) lead to
Potter syndrome
ARPKD
what is Potter syndrome characterised by
dysmorphic features:
- underdeveloped ear cartilage
- low set ears
- flat nasal bridge
- abnormalities of the skeleton
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.
ARPKD
what mnx may be required in first few days of life
renal dialysis
ARPKD
what do most patients develop before reaching adulthood
end stage renal failure
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
ARPKD
prognosis
1/3 die in neontal period
1/3 will survive to adulthood
Multicystic Dysplastic Kidney
what is it
one of the baby’s kidneys is made up of many cysts while the other kidney is normal
Multicystic Dysplastic Kidney
when is it diagnosed
on antenatal ultrasound scans.
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.
Multicystic Dysplastic Kidney
what is the pt at risk of later in life
- UTIs
- HTN
- CKD
Multicystic Dysplastic Kidney
trx
none
follow up renal USS
prophylactic abx for recurrent UTIs
Posterior Urethral Valve
what is it
tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output
Posterior Urethral Valve
who does it occur in
newborn boys
Posterior Urethral Valve
how does it cause hydronephrosis
obstruction to outflow of urine
back pressure into bladder, ureters and up the kidneys
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
Posterior Urethral Valve
presentation
- Difficulty urinating
- Weak urinary stream
- Chronic urinary retention
- Palpable bladder
- Recurrent UTIs
- Impaired kidney function
Posterior Urethral Valve
how may severe cases present as
bilateral hydronephrosis and oligohydramnios
leading to underdeveloped fetal lungs (pulmonary hypoplasia)
Posterior Urethral Valve
how may severe cases present on antenatal scans
oligohydramnios and hydronephrosis
Posterior Urethral Valve
what may abdo US show in young boys
enlarged, thickened bladder and bilateral hydronephrosis
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
Posterior Urethral Valve
why is cytoscopy used
to ablate or remove the extra tissue.
Posterior Urethral Valve
what is the definitive mnx
ablation or removal of the extra urethral tissue, usually during cystoscopy.
Posterior Urethral Valve
what can be inserted to bypass the valve whilst awaiting definitive management
a temporary urinary catheter
Wilms Tumour
what is it
a specific type of tumour affecting the kidney in children, typically under the age of 5 years.
Wilms Tumour
presentation
- mass in abdomen in child <5y
- abdo pain
- haematuria
- lethargy
- fever
- hypertension
- weight loss
Wilms Tumour
initial inx
- US
Wilms Tumour
inx to stage tumour
CT or MRI
Wilms Tumour
definitive inx for diagnosis
biopsy to identify the histology
Wilms Tumour
mnx
- surgical excision of tumour and nephrectomy
- adjuvant chemo/radio
Wilms Tumour
prognosis
Early stage tumours with favourable histology hold a good chance of cure (up to 90%).
Metastatic disease has a poorer prognosis.
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.
Undescended Testes
what are they
when the testes have not made it out of the abdomen by birth.
aka cryptorchidism
Undescended Testes
Undescended testes in older children or after puberty hold a higher risk of?
testicular torsion
infertility
testicular cancer.
Undescended Testes
RFs (5)
- FH of undescended testes
- Low birth weight
- Small for gestational age
- Prematurity
- Maternal smoking during pregnancy
Undescended Testes
at what age should they be seen by a paediatric urologist
6 months
Undescended Testes
when should Orchidopexy (surgical correction of undescended testes) be carried out
between 6 and 12 months of age.
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
Enuresis
what is it
involuntary urination
Enuresis
what is the name for bed wetting
nocturnal enuresis
Enuresis
what is the name for inability to control bladder function during the day
diurnal enuresis
Enuresis
when do most children et control of daytime urination and nighttime urination
day: 2y
night: 3-4y
Enuresis
what is primary nocturnal enuresis
the child has never managed to be consistently dry at night.
Enuresis
most common cause of primary nocturnal enuresis
a variation on normal development
. Often patients will have a family history of delayed dry nights.
Enuresis
causes of primary nocturnal enuresis
- overactive bladder
- fluid intake
- failure to wake
- psychological distress
- secondary causes: chronic constipation, UTI, learning disability, cerebral palsy
Enuresis
mnx for primary nocturnal enuresis if <5y
reassure parents that it is likely to resolve without any trx
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
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
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)
Enuresis
types of diurnal enuresis
- urge incontinence
- stress incontinence
Enuresis
causes of diurnal enuresis
- recurrent UTIs
- psychosocial problems
- constipation
Enuresis
what is an enuresis alarm
a device that makes a noise at the first sign of bed wetting
Enuresis
pharmacological trx
- desmopressin (ADH)
- oxybutynin
- imipramine (TCA)
why may constipation cause urinary sx
non-voided stools in the rectum press against the bladder, decreasing its ability to hold urine
