Paediatric Nephrology Flashcards
Presentation of UTIs in children
May only oresent with fever
Babies:
- lethargy
- irritability
- vomiting and poor feeding
- urinary frequency
Infants and children:
- abdo pain
- vomiting
- dysuria
- urinary frequency
- incontinence
How is pyelonephritis diagnosed
Temperature > 38 degrees celsius
Loin pain or tenderness
Investigations for a UTI in children
Obs Clean catch (mid stream) urine sample - MCS - positive nitrites if bacterial, WCC
Management of UTIs in children
All children under 3 months old with a fever - IV abx - ceftriaxone, full sepsis screen
Over 3 months old: oral abx
- trimethoprim
- nitrofurantoin
- cefalexin
- amoxicillin
Investigating recurrent UTIs
Under 6 months with first UTI - abdominal USS within 6 weeks or during illness, micturating cystourethrogram (MCUG)
Recurrent UTIs - abdominal USS within 6 weeks, DMSA scan 4 - 6 months after illness
Atypical UTIs - abdominal USS during illness, DMSA scan 4 - 6 months after illness
How to test for renal scarring
DMSA scan
How does a DMSA scan work
Injecting a radioactive material (DMSA) and using a gamma camera to assess how well the material is taken up by the kidneys
Pathophysiology of vesico-ureteric refoux
When urine has a tendency to flow from the bladder back into the ureters, predisposing patients to developing upper UTIs and renal scarring
How to diagnose vesico-ureteric reflux
Micturating cystourethrogram
Managment of vesico - ureteric reflux
Avoid constipation
Avoid an excessively full bladder
Prophylactic abx
Surgical input may be needed from paediatric urology
How does a MCUG
Catheterising a child and injecting contrast into the bladder whilst taking a series of xray films to determine whether the contrast is refluxing into the ureters
Prophylactic abx for 3 days before the investigation
Vulvovaginitis pathophysiology
Inflammation and irritation of the vulva and vagina caused by sensitive and thin skin mucosa around the vulvovaginal area
How is vulvovaginitis exacerbated
- Wet nappies
- Use of chemicals or soaps
- Tight clothing
- Poor toilet hygiene
- Constipation
- Threadworms
- Pressure on the area - horse riding
- Chlorinated pools
Who commonly gets vulvovaginitis
Young girls between the ages of 3 and 10 years old
After puberty, oestrogen helps keep the skin healthy and resistant to infection
Presentation of vulvovaginitis
Soreness Itching Erythema around the labia Vaginal discharge Dysuria Constipation
Investigations for vulvovaginitis
Urine dipstick may show higher leucocytes but no nitrites
Managment of vulvovaginitis
No medication required
Advice:
- avoid over washing the area with soap and chemicals
- good toilet hygiene - wipe front to back
- Keep area dry
- Emollients can sooth the area
- Loose cotton clothing
- Treat constipation and threadworms
Severe - oestrogen cream given by paediatrician
Nephrotic syndrome triad
Hypoalbuminaemia
Proteinuria
Oedema
Presentation of nephrotic syndrome in children
Normally occurs within the ages of 2 - 5 years old
Frothy urine
Pallor
Generalised oedema
Hypertension
High lipids
Hypercoaguable
Common cause of nephrotic disease in children
Minimal change disease
Secondary causes of nephrotic disease
Henoch schonlein purpura
Diabetes
Infection - HIV, hepatitis and malaria
Intrinsic kidney disease:
- focal segmental glomerulosclerosis
- membranoproliferative glomerulonephritis
How to investigate minimal chamge disease
Urinanalysis - small molecular weight proteins and hyaline casts
Renal Biopsy and microscopy - normal
Treatment for minimal change disease
Prednisolone - high dose for 4 weeks then weaned for 8 weeks
Restrict salt intake
Diuretics - oedema
Severe hyperalbuminaemia - albumin infusions
Abx prophylaxis
Nephritic disease triad
Haematuria
Hypertension
Low GFR
(Also may have proteinuria but less than nephrotic disease)
Common causes of nephritis in children
IgA nephropathy
Post streptococcal glomerulonephritis
When does post streptococcal glomerulonephritis commonly occur
1 - 3 weeks after a beta haemolytic streptococcal infection such as tonsillitis caused by Strep pyogenes
Pathophysiology of post streptococcal gonerulonephritis
Imunne complexes made up of streptococcal antigens, antibodies and complement proteins accumulate and and stick to the renal glomeruli which causes inflammation
Management of post streptococcal glomerulonephritis
Supportive, may need diuretics for oedema and hypertension
IgA nephropathy pathophysiology
Caused by IgA vasculitis where IgA deposits in the glomeruli causing inflammation
Renal biopsy shows IgA deposits and glomerular mesangial proliferation
Managment of IgA nephropathy
Supportive management of renal failure
Immunosuppressant medication - cyclophosphamide
Pathophysiology of haemolytic uraemic syndrome
Thrombus formation within small blood vessels throughout the body
Normally triggered by shiga toxin produced by E. coli or Shigella
Or use of antibiotics such as loperamide which slows down gut motility
Haemolytic uraemic syndrome triad
Haemolytic anaemia
AKI
Thrombocytopenia
Presentation of haemolytic uraemic syndrome
Gastroenteritis with blood diarrhoea
5 days after onset of diarrhoea:
- reduced urine output - AKI
- haematuria
- abdo pain
- lethargy and irritability
- bruising - thrombocytopenia
- confusion
- oedema
- hypertension
Management of heamolytic uraemic syndrome
Medical emergency - urgent referral to the paediatric renal unit for renal dialysis if required
Supportive management:
- antihyoertensives
- fluid control
- blood transfusion if required
Normal age of urination control
Daytime - 2 years old
Night time - 3 - 4 years old
Primary nocturnal enuresis
Child has nerver managed to consistently be dry at night
Causes of primary nocturnal enuresis
Overactive bladder - frequent small volume urination prevents the development of bladder capacity
Fluid intake - at night, especially fizzy drink, caffeine and juice
Failure to wake - deep sleep
Psychologic stress
Secondary causes:
- constipation
- UTI
- learning disability
- cerebral palsy
Investigations for enuresis
2 week diary - toileting, bed wetting and fluid intake
Urine dipstick
Pre and post void bladder scan
Management of primary nocturnal enuresis
Reassure parents of children < 5 yo that its likely to resolve without treatment
Advice - reduce fluid intake at night, regular toilet visits
Positive reinforcement
Enuresis alarm
Treat underlying cause such as constipation
Medication 2nd line - desmopressin
Secondary nocturnal enuresis meaning
Bed wetting when a child has previously been dry for at least 6 months
Causes of secondary nocturnal enuresis
T1DM UTI Constipation New psychological problem Maltreatment
Diurnal eneuresis cause
Urge incontinence - overactive bladder
Stress incontinence
Recurrent UTIs
Psychosocial problems
Constipation
Medication for enuresis
Desmopressin - vasopressin (ADH) analogue
Oxybutynin - anticholinergic for urge incontinence
Imipramine - tricyclic antidepressant
Which type of polycystic kidney disease presents in neonates and how is it identified?
Autosomal recessive PKD - picked up on antenatal USS
Pathophysiology of PKD
- Mutation of the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6
- Impaired fribrocystin/polyductin protein complex which normally creates tubules
Features of polycystic kidney disease
Cystic enlargement of the renal collecting ducts Oligohydramnios Pulmonary hypoplasia Congenital liver fibrosis Potters syndrome
Potters syndrome features
Underdeveloped ear cartilage
Low set ears
Flat nasal bridge
Abnormalities of the skeleton
Complications of PKD
End stage renal failure
Pulmonary hypoplasia due to oligohydramnios
Liver failure
Portal hypertension due to oesophageal varices
Hypertension
Chronic lung disease
Wilms tumour
A tumour affecting the kidneys in children that typically presents under the age of 5 years old
Presentation of a Wilms tumour
Mass in the abdomen
- abdo pain
- haematuria
- lethargy
- fever
- hypertension
- weight loss
Investigations for a Wilms tumour
USS abdomen
Staging CT
Kidney biopsy and histology
Management of a Wilms tumour
Surgical excision and nephrectomy (removing affected kidney)
Adjuvant chemotherapy and radiotherapy
Pathophysiology of a posterior urethral valve
Tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output in newborn babies
This causes back pressure into the bladder, ureters and the kidneys, causing hydronephrosis
Presentation of a posterior urethral valve
Difficulty urinating Weak urinary stream Recurrent UTIs Chronic urinary retention Palpable bladder Impaire kidney function
Investigations for a posteior uretheral valve
Abdominal USS - enlarged thickened bladded and bilateral hydronephrosis
Micturating cystourethrogram (MCUG) - location of extra tissue and reflux
Cystoscopy - camera inserted into the urethra to directly visualise the extra tissue, can ablate at same time
Management of posterior urethral valve
Observe and monitor if not causing too much of a problem
Cystoscopy and ablation
Undescended testes pathophysiology
Failure of the testes to migrate throught the inguinal canal into the scrotum by birth, therefore the testes is still in the abdomen
Also called cryptochidism
Risk of undescended testes
Testicular torsion
Infertility
Testicular cancer
Risk factors for undedescended testes
FHx Low birth weight Small for gestational age Prematurity Maternal smoking during pregnancy
Management of undescended testes
Watching and waiting until 6 months - as often descends spontaneously up to 6 moths
Not descended by 6 months - refer to paediatric urologist - orchidopexy
Hypospadias pathophysiology
Urethral meatus is abnormally displaced ventrally towards the scrotum - normally near glans
Management of hypospadias
Refer to paediatric urologists
- do not circumcise infant before urologists approves
- surgery at 3 - 4 months
Complications of hypospadias
Difficulty directing urination
Sesual dysfunction
Psychological concerns
Hydrocele pathophysiology
Collection of fluid within the tunica vaginalis that surrounds the testes
Communicating hydrocele
Where the fluid in the tunica vaginalis is connected to the peritonela cavity via the processus vaginalis (remnant of the gubernaculum) therefore hydrocoele fluctuates in size
Examination findings of a hydrocele
Transilluminates
Non tender, smooth swelling - anterior and inferior to testes
Management of a hydrocele
USS - conform diagnosis
Simple hydrocele - normally resolves within 2 years - reassurance and monitoring
Communicating hydrocele - surgery to remove processus vaginalis