Paediatric renal and urology Flashcards
What is cystitis?
Inflammation of the bladder and can be a result of a bladder infection
What are the symptoms of UTI in babies?
Fever
Lethargy
Irritability
Vomiting
Poor feeding
Urinary frequency
What are the symptoms of UTI in older children?
Fever
Abdominal, suprapubic pain
Vomiting
Dysuria
Urinary frequency
Incontinence
When is a diagnosis of pyelonephritis made?
A temperature greater than 38 degrees
Loin pain or tenderness
What does the presence of nitrites in urine suggest and why?
Bacteria as gram negative bacteria (such as E.coli) break down nitrates, a normal waste product of urine into nitrites
What do leukocytes in the urine suggest?
Leukocytes are white blood cells and normally found in urine. A significant rise however can indicate infection. Urine dipstick tests for leukocyte esterase which is a product of leukocytes
Are nitrites or leukocytes a better indication of UTI?
Nitrites- if just increased leukocytes then don’t treat for UTI unless there is clinical evidence
What is sent to the lab when testing for UTI?
Midstream urine
What is the management for children under 3 months with a fever?
Immediate IV abx (e.g. ceftriaxone) and full septic screen, blood cultures and lactate, maybe also a lumbar puncture
What are typical antibiotic choices for management of UTI in children over 3 months?
Trimethoprim
Nitrofurantoin
Cefalexin
Amoxicillin
Which children with UTI should have an ultrsound?
All children under 6 months with their first UTI
Children with recurrent UTIs within 6 weeks
Children with atypical UTIs
When are Dimercaptosuccinic acid scans used in UTI in children?
4-6 months after illness to assess for damage from recurrent or atypical UTIs.
How do DMSA scans work?
Involves injecting a radioactive material (DMSA) and using a gamma camera to assess how well material is taken up by the kidneys. Where there are patches of kidney that have not taken up the material, this indicates scarring that may be the result of previous infection
What is vesico-ureteric reflux and what does it predispose people to?
Where urine has the tendency to flow from the bladder back into the ureters predisposing to UTIs and subsequent renal scarring
How is vesico-ureteric reflux diagnosed?
Micturating cystourethrogram
What is the management of vesico-ureteric reflux?
Avoid constipation
Avoid an excessively full bladder
Prophylactic antibiotics
Surgical input from paediatric urology
When should micturating cystourethrogram used?
To investigate atypical or recurrent UTIs in children under 6 months. Also used where there is family history of vesico-ureteric reflux, dilation of the ureter on ultrasound or poor urinary flow
What does micturating cystourethrogram involve?
Catheterising the child, injecting contrast into the bladder and taking a series of xray films to determine whether the contrast is refluxing. Children are usually given prophylactic antibiotics for 3 days around the time of the investigation
What is vulvovaginitis?
Inflammation and irritation of the vulva and vagina
Who does vulvovaginitis affect?
Girls aged 3 to 10 years
What can vulvovaginitis be exacerbated by?
Wet nappies, Chemicals or soaps, tight clothing that traps moisture or sweat in the area, poor toilet hygiene, constipation, threadworms, pressure on the area e.g. horse riding, heavily chlorinated pools
Why is vulvovaginitis less common after puberty?
Oestrogen helps keep the skin and vaginal mucosa healthy and resistant to infection
What does vulvovaginitis present with?
Soreness
Itching
Erythema around the labia
Vaginal discharge
Dysuria
Constipation
Why is vulvovaginitis often misdiagnosed as a UTI?
Because a urine dipstick may show leukocytes but will not show nitrites
What is the management for vulvovaginitis?
Usually no treatment required
Avoid washing area with harsh soaps and perfumes
Keep the area dry
Emollients
Loose cotton clothing
What is nephrotic syndrome?
When the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine.
At what ages is nephrotic syndrome most common?
2 to 5 years
What does nephrotic syndrome present with?
Frothy urine, generalised oedema and pallor
What is the classic triad of features for nephrotic syndrome?
Low serum albumin, High urine protein content (>3 + protein on urine dipstick), oedema
Apart from the classic triad, what other three symptoms occur in patients with nephrotic syndrome?
Deranged lipid profile with high levels of cholesterol., triglycerides and low density lipoproteins
High BP
Hyper-coagulability with an increased tendency for blood clots
What is the most common cause of nephrotic syndrome in children?
Minimal change disease causes over 90% of cases in children under 10
What are the secondary causes of nephrotic syndrome?
Intrinsic kidney disease: focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis
Systemic illness: Henoch schonlein purpura, Diabetes, Infection (HIV, hepatitis and malaria)
What is the diagnosis for minimal change disease?
Renal biopsy and standard microscopy
Urinanalysis will show small molecular weight proteins and hyaline cysts
What is the management of minimal change disease?
Corticosteroids and most children make a full recovery.
What is the management for nephrotic syndrome?
Corticosteroids
Low salt diet
Diuretics for oedema
Albumin infusions in severe hypo
Antibiotic prophylaxis if severe
What is the treatment of nephrotic syndrome using corticosteroids?
High dose are given for 4 weeks and then gradually weaned over the next 8 weeks
What does steroid sensitive mean?
They respond to steroids (80% of children)
What does steroid dependent mean?
Patients struggle to wean steroids due to relapses
What is steroid resistant?
Patients that do not respond to steroids
What is used for nephrotic syndrome in steroid resistant children?
ACE inhibitors and immunosuppressants such as cyclosporine, tacrolimus or rituximab
What are the complications of nephrotic syndrome?
Hypovolemia- fluid from intravascular space leaks into interstitial space, causing oedema and low BP
Thrombosis- proteins that prevent blood clotting are lost in the kidneys and liver responds to low albumin by producing pro-thrombotic proteins
Infection- kidneys leak immunoglobulins, exacerbated by immunosuppressant meds such as steroids
Acute or chronic renal failure
Relapse
What is nephritis?
Inflammation within the nephrons of the kidneys
What does nephritis cause?
Reduction in kidney function
Haematuria: invisible or visible amounts of blood in the urine
Proteinuria: although less than nephrotic syndrome
What are the two most common causes of nephritis in children?
Post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease)
When does post-streptococcal glomerulonephritis occur?
1-3 weeks after a beta-haemolytic streptococcus infection e.g. tonsilitis caused by streptococcus pyogenes.
How does strep infection cause glomerulonephritis?
Immune complexes of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation. Leading to an acute deterioration in renal function causing acute kidney injury.
What could suggest tonsilitis caused by streptococcus clinically?
History, throat swab or anti-streptolysin antibody titres on blood test
What is the management of post-streptococcal glomerulonephritis?
Usually supportive. May need treatment with anti-HTN medications and diuretics if they develop complications e.g. HTN and oedema
What is IgA nephropathy also known as?
Berger’s disease
What is IgA nephropathy related to?
Henoch-Schonlein purpura which is an IgA vasculitis
How is IgA nephropathy related to Henoch-Schonlein purpura?
IgA deposits in the nephrons of the kidney cause inflammation (nephritis).
What will be seen in the renal biopsy of IgA nephropathy?
IgA deposits and glomerular mesangial proliferation
When does IgA nephropathy usually present?
teenagers or young adults
How is IgA nephropathy treated?
Supportive treatment and immunosuppressant medications such as steroids and cyclophosphamide to slow progression
What is haemolytic-uraemic syndrome?
Occurs when there is thrombosis within the small blood vessels throughout the body.
What is HUS usually triggered by?
Shiga toxin produced by e.coli 0157 or shigella
What is the classic triad of HUS?
Haemolytic uraemia
Acute kidney injury: failure to excrete waste products
Thrombocytopenia: low platelets
What can increase the risk of developing HUS?
antibiotics and anti-motility meds such as loperamide for gastroenteritis caused by pathogens
What is the timeline of presentation in HUS?
E.coli 0157 caused by brief gastroenteritis, often with bloody diarrhoea. Symptoms of HUS typically start around 5 days after the onset of the diarrhoea
What are the signs and symptoms of HUS?
Reduced urine output
Haematuria or dark brown urine
Abdominal pain
Lethargy and irritability
Confusion
Oedema
Hypertension
Bruising
What is the prognosis of HUS?
Medical emergency and has 10% mortality
70-80% make a full recovery
What is the management of HUS?
Condition is self-limiting
Renal dialysis may be required
Maybe anti-HTN
Maybe blood transfusions
Careful fluid balance maintenance
What is enuresis?
Involuntary urination
What is diurnal enuresis?
Inability to control bladder function during the day
When do most children have control of urination the day?
2 years
When do most children have control of urination at night?
3-4 years
What is primary nocturnal enuresis?
Where the child has never managed to be consistently dry at night
What is the most common cause of primary nocturnal enuresis?
Variation in normal development especially if child is under 5
What are other causes of primary nocturnal enuresis?
Overactive bladder
Fluid intake prior to bedtime
Failure to wake due to deep sleep and underdeveloped bladder signals
Psychological distress e.g. low self esteem
Secondary causes such as chronic constipation, UTI, learning disability or cerebral palsy
What is the management of primary nocturnal enuresis?
reassurance
lifestyle changes
pharmacological treatment
What are the two types of polycystic kidney disease?
autosomal dominant and autosomal recessive
Which type of PKD presents in neonates?
Autosomal recessive
What mutation results in ARPKD and what does this code for?
polycystic and hepatic disease 1 genes (PKHD1) on chromosome 6. this codes for the fibrocystin/polyductin protein complex (FPC), which is responsible for the creation fo tubules and the maintenance of healthy epithelial tissue in the kidneys, liver and pancreas
What does the underlying pathology cause in ARPKD (3 things)
Cystic enlargement of the renal collecting ducts
Oligohydramnios, pulmonary hypoplasia and potter syndrome
Congenital liver fibrosis
What is usually seen on antenatal scans in ARPKD?
oligohydramnios and polycystic kidneys
What is oligohydraminos?
lack of amniotic fluid caused by reduced urine production by the fetus
what does a lack of amniotic fluid lead to?
Potter syndrome which si charcterised by dysmorphic features such as underdeveloped ear cartilage, low set ears, a flat nasal bridge and abnormalities of the skeleton
What does oligohydromnias lead to apart from Potter syndrome?
Underdeveloped lungs (pulmonary hypoplasia), resulting in respiratory failure shortly after birth. Also large polycystic kidneys take up a lot of space so hard for neonate to breathe
What might patients reuqire in teh first few days of life with ARPKD?
renal dialysis
What is the prognosis of those with ARPKD?
most patients develop end stage renal failure before reaching adulthood. Around 1/3 will die in neonatal period. Around 1/3 will survive to adulthood
What are the ongoing problems patients with ARPKD experience?
Liver failure due to liver fibrosis
Portal HTN leading to oesophageal varices
Progressive renal failure
HTN due to renal failure
Chronic lung disease
What is multicystic dysplastic kidney? (MCDK)
Where one of the baby’s kidneys is made up of many cysts while the othe rkidney is normal
How is MCDK diagnsoed?
antenatal ultrasoun scans
What treatment is required for MCDK?
no treatment, usually the single kidney is sufficient.
What is Wilm’s tumour?
Tumour affecting the kidney in children, usually under 5 years
What is the presentation of a Wilm’s tumour?
Abdominal pain
Haematuria
Lethargy
Fever
Hypertension
Weight loss
Under 5 with a mass in the abdomen
What is the investigation for a Wilm’s tumour?
Ultrasound
A CT or BMI can be used to stage the tumour
What is the treatment for Wilm’s tumour?
Surgical excision (nephrectomy)
Adjuvant chemotherapy or radiotherapy
What is the prognosis of Wilm’s tumour?
Early stage has a good chance of cure (up to 90%)
What is a posterior urethral valve?
Where there is tissue at the proximal end of the urethra that causes obstruction of urine output
Who does a posterior urethral valve occur in?
newborn boys
What does a posterior urethral valve cause?
Hydronephrosis- a restriction in the outflow of urine prevents the bladder from fully emptying, leading to a reservoir of urine that increases the risk of UTI
What is the presentation of posterior urethral valve
Can be asymptomatic
Difficulty urinating
weak urinary system
chronic urinary retention
palpable bladder
recurrent UTI
impaired kidney function
What can severe obstruction from posterior urethral valve cause?
Bilateral hydronephrosis
oligohydramnios
What can be seen on antenatal scans with severe psoterior urethral valve?
Oligohydramnios and hydroneohrosis
What investigations are there for posterior urethral valve presenting after birth?
Abdominal ultrasound- may show enlarged, thickened bladder and bilateral hydronephrosis
Micturating cystourehtrogram (MCIG) shows location of the extra urethral tissue
Cystoscopy can be used to ablate or remove extra tissue
How do the testes develop normally?
Develop in the abdomen and then migrates down, through the inguinal canal and into the scrotum
What are undescended testes?
When the testes have not made it out of the abdomen by birth (around 5% of boys)
What is undescended testes also known as?
Cryptorchidism
What is it called when the testes have not descended fully but are palpable in the inguinal canal?
Not undescended testes technically
What are the RF for undescended testes?
Family history of undescended testes
Low birth weight
small for gestational age
prematurity
maternal smoking during pregnancy
What do undescended testes in older children or after puberty hold a higher risk of?
testicular torsion, infertility and testicular cancer
What is the management for undescended testes?
most will descend in first 3-6 months. Orchidopexy should be carried out between 6 to 12 months of age
What is retractile testicles?
normal before puberty for the testes to move out of the scrotum and into the inguinal canal when it is cold or the cremasteric reflex is activated. usually resolves
What is hypospadias?
affects males, where the urethral meatus is abnormally displaced to the ventral side of the penis, towards the scrotum
Where is the urethral meatus in hypospadias?
towards the bottom of the glans (90%)
halfway down the shaft or base of the shaft
What is epispadias?
where the urethral meatus is displaced to the dorsal side (top) of the penis.
What is the foreskin like in hypospadias?
usually abnormally formed
What condition may be associated with hypospadias?
chordee, where the head of the penis bends downwards
When is hypospadias usually diagnosed?
on examination of the newborn
What type of condition is hypospadias?
congenital
What is the management if hypospadias?
mild cases may not require treatment
surgery is usually peformed after 3-4 months of age
correct the position of the meatus and straighten the penis
What are the complications of hypospadias?
difficulty directing urination
cosmetic and pscyhological concerns
sexual dysfunction
What is a hydrocele?
A collection of fluid within the tunica vaginalis that surrounds the testes.
What is the tunica vaginalis?
A sealed pouch of membrane that surrounds the testes
What is the tunica vaginalis originally part of?
peritoneal membrane
What is a simple hydrocele and who are they common in?
common in newborn males. occur where fluid is trapped in the tunica vaginalis. usually this fluid gets reabsorbed over time
what is a communicating hydrocele?
where the tunica vaginalis around the testicle is connected with the peritoneal cavity via a pathway called the processus vaginalis. This allows fluid to travel from the peritoneal cavity into the hydrocele, allowing the hydrocele to fluctuate in size
What is a hydrocele like on examination?
soft, smooth, non-tender swelling in front of and below the testicle. They transilluminate with light with a pen torch
What are the differential diagnosis for a scrotal or inguinal swelling in a neonate?
Hydrocele
partially descended testes
Inguinal hernia
Testicular torsion
Haematoma
Tumours (rare)
What is a useful investigation for a hydrocele to rule out other causes?
Ultrasound
How long does it take for a simple hydrocele to resolve?
within 2 years
How is a communicating hydrocele managed?
surgical operation to remove or ligate the connection between the peritoneal cavity and the hydrocele (the processus vaginalis)
