Renal and Urology Flashcards
What is the most common abdominal tumour in children under 5?
Wilm’s tumour is the most common abdominal tumour in children. It is also known as nephroblastoma and is most common in children under 5 with a peak incidence between 3-4 years of age.
Presentation of Wilm’s tumour
It presents with a palpable abdominal mass, distension and haematuria
Wilm’s tumours typically do not cross the midline but in up to 5% of cases they may be bilateral.
What is a urinary tract infection?
The urinary tract includes the urethra, bladder, ureters and kidneys. Urinary tract infections are infections anywhere along this pathway.
What is acute pyelonephritis?
Acute pyelonephritis is when the infection affects the tissue of the kidney. It can lead to scarring in the tissue and consequently a reduction in kidney function.
What is cystitis?
Cystitis means inflammation of the bladder, and can be the result of a bladder infection.
Babies will present with very non-specific symptoms of UTI, such as?
Fever
Lethargy
Irritability
Vomiting
Poor feeding
Urinary frequency
Signs and symptoms of UTI in older infants and children are more specific, and inlcude what?
Fever
Abdominal pain, particularly suprapubic pain
Vomiting
Dysuria (painful urination)
Urinary frequency
Incontinence
When is the diagnosis of acute pyelonephritis made and why is this significant?
The diagnosis of acute pyelonephritis is made if either there is:
- A temperature greater than 38°C
- Loin pain or tenderness
This is a very important point to note, as it affects the way you would investigate the child for recurrent infections.
Urine dipstick - ?UTI
The ideal urine sample is a clean catch sample, avoiding contamination. This can be tricky in younger children and babies, particularly girls. This often involves the parent sat with the infant without a nappy and a urine pot held ready to catch the sample if it occurs. A clean catch sample is important to avoid contamination and unreliable microbiology results.
Nitrites – gram negative bacteria (such as E. coli) break down nitrates, a normal waste product in urine, into nitrites. The presence of nitrites suggest bacteria in the urine.
Leukocytes – leukocytes are white blood cells. There are normally a small number of leukocytes in the urine, however a significant rise can be the result of an infection or another cause of inflammation. A urine dipstick tests for leukocyte esterase, a product of leukocytes that give an indication about the number of leukocytes in the urine.
Nitrites are a better indication of infection than leukocytes. If both are present the patient should be treated as a UTI. If only nitrites are present it is worth treating as a UTI. If only leukocytes are present the patient should not be treated as a UTI unless there is clinical evidence they have one.
If nitrites or leukocytes are present, the urine should be sent to the microbiology lab. If neither are present the patient is unlikely to have a UTI.
Send a midstream urine (MSU) sample to the microbiology lab to be cultured and have sensitivity testing.
Managing UTIs in children?
All children under 3 months with a fever should start immediate IV antibiotics (e.g. ceftriaxone) and have a full septic screen, including blood cultures, bloods and lactate.
A lumbar puncture should also be considered.
Oral antibiotics can be considered in children over 3 months if they are otherwise well. Children with features of sepsis or pyelonephritis will require inpatient treatment with IV antibiotics. Always follow local guidelines. Typical antibiotic choices in urinary tract infections in children are:
Trimethoprim
Nitrofurantoin
Cefalexin
Amoxicillin
Typical antibiotic choices in urinary tract infections in children?
Trimethoprim
Nitrofurantoin
Cefalexin
Amoxicillin
Over what age can oral antibiotics be considered for a child with UTI
3 months
Recurrent UTIs should be investigated for an underlying cause and renal damage. What kind of investigations might be undertaken?
Ultrasound Scans
DMSA (Dimercaptosuccinic Acid) Scan
Micturating Cystourethrogram (MCUG)
Investigating recurrent UTIs in children - when do NICE recommend USS?
All children under 6 months with their first UTI should have an abdominal ultrasound within 6 weeks, or during the illness if there are recurrent UTIs or atypical bacteria
Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks
Children with atypical UTIs should have an
abdominal ultrasound during the illness
Investigating recurrent UTIs in children - what is a DSMA scan when do NICE recommend them?
DMSA scans should be used 4 – 6 months after the illness to assess for damage from recurrent or atypical UTIs.
This involves injecting a radioactive material (DMSA) and using a gamma camera to assess how well the 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 how is it diagnosed?
Vesico-ureteric reflux (VUR) is where urine has a tendency to flow from the bladder back into the ureters. This predisposes patients to developing upper urinary tract infections and subsequent renal scarring.
This is diagnosed using a micturating cystourethrogram (MCUG).
Management of vesico-ureteric reflux
Avoid constipation
Avoid an excessively full bladder
Prophylactic antibiotics
Surgical input from paediatric urology
Investigating recurrent UTIs in children - what is an MCUG and when do NICE recommend them?
Micturating cystourethrogram (MCUG) should be used to investigate atypical or recurrent UTIs in children under 6 months.
It is also used where there is a family history of vesico-ureteric reflux, dilatation of the ureter on ultrasound or poor urinary flow.
A MCUG is used to diagnose VUR.
It involves catheterising the child, injecting contrast into the bladder and taking a series of xray films to determine whether the contrast is refluxing into the ureters.
Children are usually given prophylactic antibiotics for 3 days around the time of the investigation.
What is vulvovaginitis?
Vulvovaginitis refers to inflammation and irritation of the vulva and vagina. It is a common condition often affecting girls between the ages of 3 and 10 years.
Which age group are commonly affected by vulvovaginitis and why?
Ages 3-10 years
This irritation is caused by sensitive and thin skin and mucosa around the vulva and vagina in young girls. The vagina is more prone to colonisation and infection with bacteria spread from faeces.
Vulvovaginitis improves and is much less common after puberty, as oestrogen helps keep the skin and vaginal mucosa healthy and resistant to infection.
What may exacerbate vulvovaginitis?
Wet nappies
Use of chemicals or soaps in cleaning the area
Tight clothing that traps moisture or sweat in the area
Poor toilet hygiene
Constipation
Threadworms
Pressure on the area, for example horse riding
Heavily chlorinated pools
How does vulvovaginitis present?
Vulvovaginitis is a common presentation in young girls before puberty. It presents with:
Soreness
Itching
Erythema around the labia
Vaginal discharge
Dysuria (burning or stinging on urination)
Constipation
A urine dipstick may show leukocytes but no nitrites. This will often result in misdiagnosis as a urinary tract infection.
Management of vulvovaginitis
Often patients have already been treated for urinary tract infections and thrush, usually with little improvement in symptoms. It is unusual for girls to develop thrush before puberty.
Generally no medical treatment is required and management focuses on simple measures to improve symptoms:
- Avoid washing with soap and chemicals
- Avoid perfumed or antiseptic products
- Good toilet hygiene, wipe from front to back
- Keeping the area dry
- Emollients, such as sudacrem can sooth the area
- Loose cotton clothing
- Treating constipation and worms where applicable
- Avoiding activities that exacerbate the problem
In severe cases an experienced paediatrician may recommend oestrogen cream to improve symptoms.
What is nephrotic syndrome?
Nephrotic syndrome occurs when the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine. It is most common between the ages of 2 and 5 years. It presents with frothy urine, generalised oedema and pallor.
What is wrong with the basement membrane in the glomerulus in nephrotic syndrome?
Nephrotic syndrome occurs when the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine.
Nephrotic syndrome is the classic triad of what features?
Low serum albumin
High urine protein content (>3+ protein on urine dipstick)
Oedema
Features of nephrotic syndrome?
It presents with frothy urine, generalised oedema and pallor.
Nephrotic syndrome features a classic triad of:
- Low serum albumin
- High urine protein content (>3+ protein on urine dipstick)
- Oedema
There are three other features that occur in patients with nephrotic syndrome:
Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins
High blood pressure
Hyper-coagulability, with an increased tendency to form blood clots
Causes of nephrotic syndrome?
The most common cause in children is minimal change disease. In minimal change disease, nephrotic syndrome occurs in isolation, without any clear underlying condition or pathology.
There are a number of secondary causes of nephrotic syndrome, where it occurs due to an underlying condition.
It can be secondary to intrinsic kidney disease:
Focal segmental glomerulosclerosis
Membranoproliferative glomerulonephritis
It can also be secondary to an underlying systemic illness:
Henoch schonlein purpura (HSP)
Diabetes
Infection, such as HIV, hepatitis and malaria
Most common cause of nephrotic syndrome in children?
Minimal change disease
What might nephrotic syndrome occur secondary too?
Intrinsic kidney disease:
- Focal segmental glomerulosclerosis
- Membranoproliferative glomerulonephritis
Underlying systemic illness:
- Henoch schonlein purpura (HSP)
- Diabetes
- Infection, such as HIV, hepatitis and malaria
What is the most likely underlying diagnosis in a 2 – 5 year old child with oedema, proteinuria and low albumin?
Minimal change disease
How is minimal change disease managed?
Management of minimal change disease is with corticosteroids (i.e. prednisolone). The prognosis is good and most children make a full recovery, however it may reoccur.
Minimal change disease is the most common cause of nephrotic syndrome in children. It can occur in otherwise healthy children, without any clear risk factors or reason for developing the condition. It is not clear why it occurs in most cases.
What abnormalities may be seen on investigation?
A renal biopsy and standard microscopy in minimal change disease is usually not able to detect any abnormality.
Urinalysis (analysis of the urine) will show small molecular weight proteins and hyaline casts.
Nephrotic syndrome should be managed by experienced paediatricians with input from renal specialists. General management includes what?
High dose steroids (i.e. prednisolone)
Low salt diet
Diuretics may be used to treat oedema
Albumin infusions may be required in severe hypoalbuminaemia
Antibiotic prophylaxis may be given in severe cases
Steroid management of nephrotic syndrome?
High dose steroids are given for 4 weeks and then gradually weaned over the next 8 weeks:
80% of children will respond to steroids, and are referred to as steroid sensitive
80% of steroid sensitive patients will relapse at some point and need further steroids
Patients that struggle to wean steroids due to relapses are referred to as steroid dependant
Patients that do not respond to steroids are referred to as steroid resistant
What management for nephrotic syndrome may be used in steroid resistant children?
In steroid resistant children, ACE inhibitors and immunosuppressants such as cyclosporine, tacrolimus or rituximab may be used.
Potential complications of nephrotic syndrome?
Hypovolaemia occurs as fluid leaks from the intravascular space into the interstitial space causing oedema and low blood pressure.
Thrombosis can occur because proteins that normally prevent blood clotting are lost in the kidneys, and because the liver responds to the low albumin by producing pro-thrombotic proteins.
Infection occurs as the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond. This is exacerbated by treatment with medications that suppress the immune system, such as steroids.
Acute or chronic renal failure
Relapse
Why might hypovolemia occur as a complication of nephrotic syndrome?
Fluid leaks from the intravascular space into the interstitial space causing oedema and low blood pressure
Why might thrombosis occur as a complication of nephrotic syndrome?
Because proteins that normally prevent blood clotting are lost in the kidneys, and because the liver responds to the low albumin by producing pro-thrombotic proteins.
Why might infection occur as a complication of nephrotic syndrome?
The kidneys leak immunoglobulins, weakening the capacity of the immune system to respond.
This is exacerbated by treatment with medications that suppress the immune system, such as steroids.
What is a hydrocele, and what structures are involed?
A hydrocele is a collection of fluid within the tunica vaginalis that surrounds the testes.
The tunica vaginalis is a sealed pouch of membrane that surrounds the testes.
Originally the tunica vaginalis is part of the peritoneal membrane, but during development of the fetus it becomes separated from the peritoneal membrane and remains in the scrotum, partially covering each testicle.
What are simply hydroceles?
They occurs where fluid is trapped in the tunica vaginalis.
Usually this fluid gets reabsorbed over time and the hydrocele disappears.
What type of hydrocele is common in the newborn male?
Simple hydroceles
What is a communicating hydrocele?
Communicating hydroceles occur 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 seen O/E in a hydrocele?
Hydroceles cause a soft, smooth, non-tender swelling around one of the testes.
The swelling will be IN FRONT OF AND BELOW the testicle.
Simple hydroceles remain one size, whereas communicating hydroceles can fluctuate in size depending on the volume of fluid from the peritoneal cavity.
They transilluminate with light. To transilluminate the hydrocele, hold a pen torch flat against the skin and watch as the whole thing lights up like a bulb.
Simple vs communicating hydrocele
Simple hydroceles remain one size, whereas communicating hydroceles can fluctuate in size depending on the volume of fluid from the peritoneal cavity.
Simple hydrocele occurs where fluid is trapped in the tunica vaginalis, communicating hydrocele occurs where the tunica vaginalis around the testicle is connected with the peritoneal cavity via a pathway called the processus vaginalis
What are the key differential diagnoses of a scrotal or inguinal swelling in a neonate?
Hydrocele
Partially descended testes
Inguinal hernia
Testicular torsion
Haematoma
Tumours (rare)
Management of hydrocele
Ultrasound is a useful investigation for confirming the diagnosis and excluding other causes.
Simple hydroceles will usually resolve within 2 years without having any lasting negative effects. Parents can be reassured and followed up routinely. They may require surgery if they are associated with other problems, such as a hernia.
Communicating hydroceles can be treated with a surgical operation to remove or ligate the connection between the peritoneal cavity and the hydrocele (the processus vaginalis).
What is hypospadias?
Hypospadias is a condition affecting males, where the urethral meatus (the opening of the urethra) is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum.
This might be further towards the bottom of the glans (in 90% of cases), halfway down the shaft or even at the base of the shaft.
Epispadias is where the meatus is displaced to the dorsal side (top side) of the penis.
Usually, the foreskin is abnormally formed to match the position of the meatus.
There can also be an associated condition called chordee, where the head of the penis bends downwards.
Hypospadias is a congenital condition affecting babies from birth and is usually diagnosed on the examination of the newborn.
What is epispadias?
Epispadias is where the urethral meatus is displaced to the dorsal side (top side) of the penis.
What associated condition can be present alongside hypospadias?
Chordee, where the head of the penis bends downwards.
How is hypospadias managed?
Hypospadias requires referral to a paediatric specialist urologist for ongoing management. It is important to warn parents not to circumcise the infant until a urologist indicates this is ok.
- Mild cases may not require any treatment
- Surgery is usually performed after 3 – 4 months of age
- Surgery aims to correct the position of the meatus and straighten the penis
Hypospadias complications?
Difficulty directing urination
Cosmetic and psychological concerns
Sexual dysfunction
Basic normal development pathway of the testes?
The testes develop in the abdomen and then gradually migrate down, through the inguinal canal and into the scrotum. They have normally reached the scrotum prior to birth.
In about 5% of boys the testes have not made it out of the abdomen by birth. Where may they be palpable?
They might be palpable in the inguinal canal (in the inguinal region), which is not technically classed as undescended testes, although they have not fully descended at that point.
What is cryptorchidism?
Undescended testes - where the testes have not made it out of the abdomen by birth
The longer the testes take to descend, the less likely it is this will happen spontaneously. Undescended testes in older children or after puberty can cause what problems?
The longer the testes take to descend, the less likely it is this will happen spontaneously.
Undescended testes in older children or after puberty hold a higher risk of testicular torsion, infertility and testicular cancer.
Cryptorchidism (undescended testes) risk factors
Family history of undescended testes
Low birth weight
Small for gestational age
Prematurity
Maternal smoking during pregnancy
Cryptorchidism (undescended testes) management?
Watching and waiting is appropriate in newborns. In most cases the testes will descend in the first 3 – 6 months.
If they have not descended by 6 months they should be seen by a paediatric urologist.
Orchidopexy (surgical correction of undescended testes) should be carried out between 6 and 12 months of age.
Orchidopexy (surgical correction of undescended testes) should be carried out between which ages?
6 and 12 months of age
If the testes have not descended by what age they should be seen by a paediatric urologist?
6 months
What are retractile testes?
It is normal in boys that have not reached 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.
This is described as retractile testicles and is considered a normal variant.
This usually resolves as they go through puberty and the testes settle in the scrotum.
Occasionally they may fully retract or fail to descend and require surgical correction with orchidopexy.
What is nephritis and what does it cause?
Nephritis refers to inflammation within the nephrons of the kidneys. It causes:
- Reduction in kidney function
- Haematuria: invisible or visible amounts of blood in the urine
- Proteinuria: although less than in nephrotic syndrome
Most common causes of nephritis in children?
Post-streptococcal glomerulonephritis
IgA nephropathy (Berger’s disease).
When does post-streptococcal glomerulonephritis occur?
Post-streptococcal glomerulonephritis occurs 1 – 3 weeks after a β-haemolytic streptococcus infection, such as tonsillitis caused by Streptococcus pyogenes.
Why does post-streptococcal glomerulonephritis cause AKI?
Immune complexes made up of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation.
This inflammation leads to an acute deterioration in renal function, causing an acute kidney injury.
When should a differential of post-streptococcal glomerulonephritis be considered?
Where there is evidence of recent tonsillitis caused by streptococcus, such as:
History of tonsillitis
Positive throat swab results
Anti-streptolysin antibody titres found on a blood test
How is post-streptococcal glomerulonephritis managed?
Management is supportive and around 80% of patients will make a full recovery.
In some cases patients can develop a progressive worsening of their renal function.
They may need treatment with antihypertensive medications and diuretics if they develop complications such as hypertension and oedema.
What is IgA nephropathy and which condition is it related to?
IgA nephropathy is also known as Berger’s disease.
This condition is related to Henoch-Schonlein Purpura, which is an IgA vasculitis.
How does IgA nephropathy cause nephritis?
IgA deposits in the nephrons of the kidney causes inflammation (nephritis).
Which age group are typically affected by IgA nephropathy?
It usually presents in teenagers or young adults.
What would be seen on histology in renal biopsy in IgA nephropathy?
IgA deposits and glomerular mesangial proliferation
What is seen here on this renal biopsy?
IgA deposits and glomerular mesangial proliferation - IgA nephropathy
What is seen here on this renal biopsy?
IgA deposits and glomerular mesangial proliferation - IgA nephropathy
Management of IgA nephropathy?
Management involves supportive treatment of the renal failure and immunosuppressant medications such as steroids and cyclophosphamide to slow the progression of the disease.
What is HUS?
Haemolytic uraemic syndrome (HUS) occurs when there is thrombosis within small blood vessels throughout the body.
This is usually triggered by a bacterial toxin called shiga toxin. It leads to the classic triad of:
- Haemolytic anaemia: anaemia caused by red blood cells being destroyed
- Acute kidney injury: failure of the kidneys to excrete waste products such as urea
- Thrombocytopenia: low platelet count
What triad of symptoms occur in haemolytic uraemic syndrome?
Haemolytic anaemia
- anaemia caused by red blood cells being destroyed
Acute kidney injury
- failure of the kidneys to excrete waste products such as urea
Thrombocytopenia
- low platelet count
What usually causes HUS?
SHIGA TOXIN
e. coli 0157 bacteria - most commonly producer
also produced by SHIGELLA
What medications increase the risk of patients developing HUS?
The use of antibiotics and anti-motility medications such as loperamide to treat gastroenteritis caused by Shiga producing bacteria (e. coli 0157 and shigella) increases the risk of developing HUS.
How does haemolytic uraemic syndrome present?
E. coli 0157 causes a brief gastroenteritis, often with bloody diarrhoea.
The symptoms of haemolytic uraemic syndrome typically start around 5 days after the onset of the diarrhoea.
Signs and symptoms of HUS may include:
Reduced urine output
Haematuria or dark brown urine
Abdominal pain
Lethargy and irritability
Confusion
Oedema
Hypertension
Bruising
HUS signs and symptoms?
Reduced urine output
Haematuria or dark brown urine
Abdominal pain
Lethargy and irritability
Confusion
Oedema
Hypertension
Bruising
The symptoms of haemolytic uraemic syndrome typically start around how long following the onset of the diarrhoea.
5 days
Management of HUS?
HUS is a medical emergency and has a 10% mortality. It needs to be managed by experienced paediatricians under the guidance of a renal specialist. The condition is self limiting and supportive management is the mainstay of treatment:
- Urgent referral to the paediatric renal unit for renal dialysis if required
- Antihypertensives if required
- Careful maintenance of fluid balance
- Blood transfusions if required
- 70 to 80% of patients make a full recovery.
What is Wilms’ tumour and what age is typically affected?
Wilms’ tumour is a specific type of tumour affecting the kidney in children, typically under the age of 5 years.
What should be considered in a child under the age of 5 years presenting with a mass in the abdomen?
Wilms’ tumour
How might Wilms’ tumour present?
Child <5 presenting with mass in abdomen +
Abdominal pain
Haematuria
Lethargy
Fever
Hypertension
Weight loss
How is Wilms’ tumour diagnosed?
Abdominal USS - INITIAL INVESTIGATION
Initial investigation is an ultrasound of the abdomen to visualise the kidneys.
STAGING - CT OR MRI
A CT or MRI scan can be used to stage the tumour.
DEFNITIVIE DIAGNOSIS - BIOPSY
Biopsy to identify the histology is required to make a definitive diagnosis.
Wilms’ tumour - initial investigation?
Abdominal USS
Wilms’ tumour - definitive diagnosis
Biopsy
Mgx of Wilms’ tumour?
NEPHERECTOMY - surgical excision of the tumour along with the affected kidney (nephrectomy).
Adjuvant treatment refers to treatment that is given after the initial management with surgery. This depends on the stage of the disease, the histology and whether it has spread. The main options are:
- Adjuvant chemotherapy
- Adjuvant radiotherapy
Prognosis of Wilms’ tumour?
Early stage tumours with favourable histology hold a good chance of cure (up to 90%). Metastatic disease has a poorer prognosis.
What is enuresis?
Involuntary urination
What is nocturnal enuresis?
Bedwetting
What is diurnal enuresis?
Inability to control bladder function during the day
By what age do most children get control of daytime urination?
2 years
By what age do most children get control of nighttime urination?
2-4 years
What is primary nocturnal enuresis?
Child has never managed to be consistently dry at night.
What is secondary nocturnal enuresis?
Child begins wetting the bed when they have previously been dry for at least 6 months.
Which type of nocturnal enuresis is more indicative of an underlying illness?
Secondary nocturnal enuresis (child wetting the bed when they have previously been dry for 6 months)
What is the most common cause of primary nocturnal enuresis?
The most common cause of primary nocturnal enuresis is a variation on normal development, particularly if the child is younger than 5 years.
Often patients will have a family history of delayed dry nights.
In this situation reassurance is important, and there is no need to jump to further investigations or management.
Causes of primary nocturnal enuresis?
Normal development (particularly if <5 years)
Overactive bladder. Frequent small volume urination prevents the development of bladder capacity.
Fluid intake prior to bedtime, particularly fizzy drinks, juice and caffeine, which can have a diuretic effect
Failure to wake due to particularly deep sleep and underdeveloped bladder signals
Psychological distress, for example low self esteem, too much pressure or stress at home or school
Secondary causes such as
- Chronic constipation
- Urinary tract infection
- Learning disability
- Cerebral palsy
Investigating primary nocturnal enuresis?
- The initial step in management of primary nocturnal enuresis is to establish the underlying cause.
- 2 week diary of toileting, fluid intake and bedwetting episodes.
This helps establish any patterns and identifies areas that may be changed, such as fluid intake before bed.
It is important to take a history and examination to exclude underlying physical or psychological causes.
Managing primary nocturnal enuresis?
Reassure parents of children under 5 years that it is likely to resolve without any treatment
Lifestyle changes: reduced fluid intake in the evenings, pass urine before bed and ensure easy access to a toilet
Encouragement and positive reinforcement. Avoid blame or shame. Punishment should very much be avoided.
Treat any underlying causes or exacerbating factors, such as constipation
Enuresis alarms
Pharmacological treatment
Causes of secondary nocturnal enuresis include what?
Urinary tract infection
Constipation
Type 1 diabetes
New psychosocial problems (e.g. stress in family or school life)
Maltreatment
Management of secondary nocturnal enuresis?
Always think about abuse and safeguarding, particularly with deliberate bedwetting, punishment for bedwetting (despite parental education) or unexplained secondary nocturnal enuresis.
Management of secondary nocturnal enuresis is based on treating the underlying cause.
The most common and easily treatable secondary causes are urinary tract infections and constipation.
Other problems may require referral to secondary care for further management.
Types of diurnal enuresis?
Urge incontinence is an overactive bladder that gives little warning before emptying
Stress incontinence describes leakage of urine during physical exertion, coughing or laughing.
In which sex is diurnal enuresis more common?
Female
Potential causes of diurnal enuresis?
Up to 2 years - normal development
Recurrent urinary tract infections
Psychosocial problems
Constipation
Stress incontinence
Overactive bladder
What is an enuresis alarm and how is it used?
An enuresis alarm is a device that makes a noise at the first sign of bed wetting, waking the child and stopping them from urinating.
It requires quite a high level of training and commitment and needs to be used consistently for a prolonged period (i.e. at least 3 months).
Some families may find them very helpful, whereas others may find they add to the burden and frustration and are counter productive.
Pharmacological management of enuresis?
Medication for nocturnal enuresis is usually initiated by a specialist:
DESMOPRESSIN
Desmopressin is an analogue of vasopressin (also known as anti-diuretic hormone). It reduces the volume of urine produced by the kidneys. It is taken at bedtime with the intention of reducing nocturnal enuresis.
OXYBUTININ
Oxybutinin is an anticholinergic medication that reduces the contractility of the bladder.
It can be helpful where there is an overactive bladder causing urge incontinence.
IMIPARAMINE
Imipramine is a tricyclic antidepressant. It is not clear how it works, but it may relax the bladder and lighten sleep.
What is a posterior urethral valve and how does it occur in?
A posterior urethral valve is where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output.
It occurs in newborn boys.
The obstruction to the outflow of urine creates a back pressure into the bladder, ureters and up to the kidneys, causing 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 urinary tract infections.
Severity of a posterior urethral valve varies. Mild cases may be asymptomatic or present how?
Difficulty urinating
Weak urinary stream
Chronic urinary retention
Palpable bladder
Recurrent urinary tract infections
Impaired kidney function
Severity of a posterior urethral valve varies. Severe cases may be asymptomatic or present how?
Severe cases can cause obstruction to urine outflow in the developing fetus resulting in bilateral hydronephrosis and oligohydramnios (low amniotic fluid volume).
The oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia) with respiratory failure shortly after birth.
How is posterior urethral valve investigated/diagnosed?
Severe cases may be picked up on antenatal scans as oligohydramnios and hydronephrosis.
To investigate cases presenting after birth, for example young boys presenting with urinary tract infections:
- Abdominal ultrasound may show an enlarged, thickened bladder and bilateral hydronephrosis
- Micturating cystourethrogram (MCUG) shows the location of the extra urethral tissue and reflux of urine back into the bladder
- Cystoscopy involves a camera inserted into the urethra to get a detailed view of the extra tissue. - -
- Cystoscopy can be used to ablate or remove the
extra tissue.
Management of posterior urethral valve?
Mild cases may simply be observed and monitored.
If required a temporary urinary catheter can be inserted to bypass the valve whilst awaiting definitive management.
Definitive management is by ablation or removal of the extra urethral tissue, usually during cystoscopy.
In what patients foes a posterior urethral valve occur?
Newborn boys
What are the two types of polycystic kidney disease and what ages do they present at?
Autosomal recessive polycystic kidney disease (ARPKD) - (ARPKD) presents in neonates and is usually picked up on antenatal ultrasound scans.
Autosomal dominant, known as ADPKD, presents later in life, usually in adults.
What genetic abnormality cause autosomal recessive polycystic kidney disease (ARPKD)?
Mutation in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6
This gene codes for the 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.
What pathology does autosomal recessive polycystic kidney disease (ARPKD) cause?
Cystic enlargement of the renal collecting ducts
Oligohydramnios, pulmonary hypoplasia and Potter syndrome
Congenital liver fibrosis
When and how does autosomal recessive polycystic kidney disease present?
ANETNATAL SCANNING
- oligohydramnios and polycystic kidneys seen
POTTERS SYNDROME
A lack of amniotic fluid leads to Potter syndrome, which is characterised by dysmorphic features such as:
- underdeveloped ear cartilage
- low set ears
- a flat nasal bridge
- abnormalities of the skeleton.
RESPIRATORY FAILURE SHORTLY AFTER BIRTH - pulmonary hypoplasia + large cystic kidneys
*The oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia) resulting in respiratory failure shortly after birth
*Additionally, large cystic kidneys can take up so much space in the abdomen it becomes hard for the neonate to breath adequately
Prognosis of ARPKD?
Patients may require renal dialysis within the first few days of life.
Most patients develop end stage renal failure before reaching adulthood.
The prognosis is poor. Survival depends of very extensive interventions from a number of different specialties both in the neonatal period and throughout life.
Around 1/3 will die in the neonatal period.
Around 1/3 will survive to adulthood.
How does ARPKD resulting in respiratory failure shortly after birth?
- The oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia)
- Large cystic kidneys can take up so much space in the abdomen it becomes hard for the neonate to breath adequately
Patients with polycystic kidney disease have a number of ongoing problems throughout life, such as?
Liver failure due to liver fibrosis
Portal hypertension leading to oesophageal varices
Progressive renal failure
Hypertension due to renal failure
Chronic lung disease
What is multicystic dysplastic kidney and how is it normally diagnosed??
Multicystic dysplastic kidney (MCDK) is a separate condition to PKD,
One of the baby’s kidneys is made up of many cysts while the other kidney is normal.
In rare cases it can be bilateral, which inevitably leads to death in infancy. MCDK is normally diagnosed on antenatal ultrasound scans.
Multicystic dysplastic kidney - prognosis/disease course/complications?
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.
Having a single kidney can put the person at risk of urinary tract infections, hypertension and chronic kidney disease later in life.
How is multicystic dysplastic kidney managed?
No treatment is required for MCDK.
Followup renal ultrasound scans can be used to monitor the abnormal kidney.
Prophylactic antibiotics are occasionally used to prevent urinary infections that may affect the working kidney.
What is the investigation of choice to look for renal scarring in a child with vesicoureteric reflux?
Radionuclide scan using dimercaptosuccinic acid (DMSA)
Typical VS atypical presentaton of minimal change disease
When and why might antibiotics be utilised in nephtrotic syndrome
Oral penicillin V should be prescribed as antibiotic prophylaxis
to oedematous patients to protect against pneumococcal infection.
What are criteria for renal ultrasound (US) in a child with a UTI?
- Infant <6 months old. Renal US within 6 weeks.
- Atypical UTI as defined by:
* Seriously ill.
* Poor urine flow.
* Abdominal/bladder mass.
* Raised creatinine.
* Septicaemia.
* Failure to respond to antibiotics within 48 hours.
* Infection with non-E. coli organism - Recurrent UTI:
* 2 or more upper UTI.
* 1 upper and one or more lower UTIs.
* 3 or more lower UTIs
Glomerular haematuria - characteristics
Rusty or Coca-Cola colour
Usually painless
Oliguria and hypertension may be present
Urine microscopy:
* Small and dysmorphic RBC
* Red cell casts
* Spikes/blebs on RBC
* Loss of RBC circumferential halo
Non-glomerular haematuria - characteristics
Red or cranberry colour
Usually painful, but also painless
Urine microscopy:
* Monomorphic RBC
* No casts
* No spikes
* RBC circumferential halo present
CKD complications
Bleeding tendency
Secondary hyperparathyroidism
Intellectual impairment
Hyperuricaemia
Anaemia
CVD
Electrolyte abnormalities. Metabolic acidosis with low sodium and increased potassium.
Growth impairment
