Age-related diseases of

urinary system in children

Prof. Rusudan Karseladze

Head of Pediatric Department
Tbilisi State University
Nelson, textbook of pediatrics, p.1779
Content
Nephrotic syndrome
Hematuria and investigations
UTI
Eneuresis
Renal investigations
Nephrotic syndrome

• Nephrotic range proteinuria (> 200mg/mmol)

• Hypoalbuminaemia (<25 g/l)
• Oedema
 Typical features Atypical features

Age 1- 10 y <1 >10 y

Normotensive Hypertensive
Normal renal functions Abnormal renal
functions
Microscopic haematuria Macroscopic haematuria
Initial investigation

Blood: FBC, U+E’s; Creatinine; LFT’s; ASOT; C3/C4; Varicella titres

Urine: Urine culture andUrinary protein/creatinine ratio
 BP
 Urinalysis including glucose
A urinary sodium concentration can be helpful in those at risk of
hypovolaemia.
Complications
1) Hypovolemia – abdominal pain, unwell, tachycardia, poor perfusion,
High Hb, high urea
2) Peritonitis – difficult to recognise and may be masked due to
steroids.
3) Thrombosis – renal, pulmonary and cerebral Veins
Fall in platelets, raised FDP, abnormal PTT,
abnormal dopplers
Treatment
Strict input out put – Oliguric patients need fluid
restriction.400ml/m2
Prednisolone 60mg/m2 – till negative or trace proteins in urine, then
40mg/m2 on alternate days for 4 weeks
Penicillin prophylaxis
Advice on immunisations and contact
Teach dipstick technique
Some definitions
Remission – trace or no protein on 3 consecutive days
Relapse – 3+ or more protein on 3 consecutive days
Steroid resistance- failure of remission for 4 weeks
How often do we check urine :
Urine should be checked initially twice weekly, then weekly after the
first episode, and the families instructed to get in contact should a
relapse of proteinuria occur, or if there is ++ for more than 1 week.
When to refer to a nephrologist

• Age < 1 yr
• Age > 10-12 yrs
• Persistent hypertension
• Macroscopic haematuria
• Low C3/C4
• Failure to respond to steroids within 4 weeks
Hematuria – AGN
• Hematuria, proteinuria, odema, hypertension and renal insufficiency
• Symptoms and signs
• Macroscopic hematuria
• Oedema
• Breathlessness
• Headaches
• Weight gain, B.P, JVP, signs of cardiac failure, oliguria
Management
• Urine dip
• U&E,bicarbonate,phosphate,albumin,C3 and C4
• FBC
• ASO, Throat swab
• Treatment
• Fluid restrict
• Monitor BP, weight
• Penicillin prophylaxis
 Urinary tract infection (UTI)

3% of girls and 1 % of boys suffer from Urinary tract infection (UTI) during childhood
UTI may be defined in terms of the presence of symptoms (dysuria, frequency, loin pain)
plus the significant culture of organisms urine :

 Any growth on culture of suprapubic aspirate

 >10 0 organisms/mL in pure growth from carefully collected urine
(midstream urine, clean catch urine)

Eschrichia coli – in 75-90% cause of infection in girls

Nelson, textbook of pediatrics, p.1829

 Urinary tract infection (UTI)- Pyelonephritis

Clinical features

Symptoms are varies;

in infants may be non-specific

Fever (may be only manifestation)

Abdominal or back pain
Vomiting/diarrhea
Poor feeding/failure the thrive
Prolonged neonatal jaundice

Nelson, textbook of pediatrics, p.1829

 Urinary tract infection (UTI)-Cystitis
Indicate bladder involvement:
dysuria, frequency, mild abdominal – suprapubic pain, enuresis

Ask about urinary stream in boys and family history of

vesicoureteric reflux (VUR) or other urinary tract abnormality!

Bacteriuria in the absence of symptoms does not necessarily

need treatment,
but needs to be considered in the clinical context
It is most common in girls.
 UTI – Treatment and Prevention
Treatment:
•TMP_SMX
•Nitrofuran 5-7 mg/kg/24hr in3-4 divided doses
•Amoxicillin 50 mg/kg /24 hr
•in Hospital:
•IV ceftriaxone 100 mg/kg /24hr or ampicillin 100 mg/kg /24hr +amioglycoside (3-5 mg/kg /24hr )
•Rehydration if patent is dehydrated
•Oral 3rd generation of cephalosporin's (cefexime) is also effective

 Treat and prevent constipation

 Hygiene: clean perineum front to back
 Avoid nylon underwear and bubble bath
 Encourage fluid intake and regular toileting with micturition

Oral antibiotic prophylaxis is required (nitrofurantoin 1 mg/kg or trimethoprim 2 mg/kg) if:

 VUR
 Recurrent UTIs (more than 2-3 episodes)

Nelson, textbook of pediatrics, p.1837

 Glomerulonephritis
Combination of haematuria, oliguria, oedema and hypertension with variable proteinuria
• Majority of cases post-infectios
• Usually presents 1-2 weeks after a URTI and sore throat
• Symmetrically enlarged kidneys

Causes of glomerulonephritis:
• Bacterial: St. commonest, St. aureus
• Mycoplasma pneumonia, Salmonella
• Virus: EBV, varicella, CMV
• Fungi: candida, aspergillus
• Parasites: toxoplasma, malaria, schisosomiasis
Other:
• MPGN
• IgA nephropathy
• Systemic lupus erythematosus
• Sub acute endocarditis
• Shunt nephritis

Nelson, textbook of pediatrics, p.1779

Glomerulonephritis
 Glomerulonephritis

Investigations:
Urine: urinalysis by dipstick – haematuria+/-proteinuria
microscopy-casts, mostly red cells
Throat swabs: culture
Bloods:
• FBC
• U&I including creatinine, bicarbonate, calcium, phosphate
• Albumin
• ASOT/antiDNAase B
• Complement
• Autoantibody screen Renal US
• C×R
 Glomerulonephritis
Management:
Infection
• 10-day course of penicillin
Hypertension
• Α-blockers and Ca channel blocker
Treat life complications first:
• Hyperkalemia
• Hypertension
• Acidosis
• Seizures
• Hypocalcemia
Otherwise supportive treatment
• Fluid balance: weight daily
• No add/restricted salt diet
• If oliguric, fluid restrict to insensible losses + urine
• Furosemide 1-2 mg/kg bd if fluid overloaded
Alport syndrome or hereditary nephritis

Alport syndrome or hereditary nephritis is a genetic disorder characterized

by glomerulonephritis, end-stage kidney disease, and hearing loss.
Alport syndrome can also affect the eyes, causing eye abnormalities
including cataracts, lenticonus, kerataconus, as well as retinal flecks in
the macula and mid-periphery.
Visibly bloody urine and protein in the urine are common features of this
condition.

Mostly - it is inherited in an X-linked pattern,

Alport syndrome can also be inherited in an autosomal recessive pattern.

Nelson, textbook of pediatrics, p.1782

At least four of the following ten criteria must be met to diagnose an individual with Alport syndrome:
1. Family history of nephritis of unexplained hematuria in a first degree relative of the index case or in a
male relative linked through any numbers of females.
2. Persistent hematuria without evidence of another possibly inherited nephropathy such as thin
glomerular basement membrane disease, polycystic kidney disease or IgA nephropathy.
3. Bilateral sensorineural hearing loss in the 2000 to 8000 Hz range. The hearing loss develops gradually, is
not present in early infancy, and commonly presents before the age of 30 years.
4.Immunohistochemical evidence of complete or partial lack of the Alport epitope in glomerular, or
epidermal basement membranes, or both.
5.Widespread glomerular basement membrane ultrastructural abnormalities, in particular thickening,
thinning and splitting.
6. Eye lesions including anterior lenticonus, kerataconus, posterior subcapsular cataract, posterior
polymorphous dystrophy and retinal flecks.
7. Gradual progression to end-stage kidney disease in the index case of at least two family members.
8. Macrothrombocytopenia or granulocytic inclusions, similar to the May-Hegglin anomaly.
9. Diffuse leiomyomatosis of esophagus or female genitalia, or both
Hemolytic-uremic syndrome (or haemolytic-uraemic
syndrome) - HUS

Main symptoms:
•hemolytic anemia
•acute kidney failure (uremia)
•Thrombocytopenia

HUS was first defined as a syndrome in 1955.

Nelson, textbook of pediatrics, p.1791

Hemolytic anemia plus one or more of the following:
•neurological symptoms (e.g., confusion, cerebral convulsions, seizures);
•renal impairment (e.g., elevated creatinine, decreased estimated glomerular
filtration rate [eGFR],
• abnormal urinalysis ;
•gastrointestinal (GI) symptoms (e.g., diarrhea, nausea/vomiting, abdominal
pain, gastroenteritis
Nelson, textbook of pediatrics, p.1791
UTI
• Common cause of fever
• Important to recognise this – as implications for further investigations
and management
• Recognise different urine collection methods
• History important
 Imaging
Recommended imaging schedule for infants younger
than 6 months
Test Responds well to Atypical UTI Recurrent UTI
treatment within
48 hours
Ultrasound during No Yes Yes
the acute infection
Ultrasound within Yes No No
6 weeks
DMSA 4–6 months No Yes Yes
following the acute
infection
MCUG No Yes Yes
 Imaging
Recommended imaging schedule for infants and
children 6 months and older but younger than 3 years

Test Responds well to Atypical UTI Recurrent

treatment within UTI
48 hours
Ultrasound during the No Yes No
acute infection

Ultrasound within No No Yes

6 weeks
DMSA 4–6 months No Yes Yes
following the acute
infection
MCUG No No No
 Imaging
Recommended imaging schedule for children
3 years and older
Test Responds well Atypical Recurrent
to treatment within UTI UTI
48 hours
Ultrasound during No Yes No
the acute infection

Ultrasound within No No Yes

6 weeks
DMSA 4– No No Yes
6 months following
the acute infection
MCUG No No No
Imaging tests: atypical UTI
• Atypical UTI is defined as any of the following:

• Seriously ill (for more information refer to ‘Feverish illness in children’ (NICE
clinical guideline 47)
• Poor urine flow
• Abdominal or bladder mass
• Raised creatinine
• Septicaemia
• Failure to respond to treatment with suitable antibiotics within 48 hours
• Infection with non-E. coli organisms.
The Final Urological Diagnosis of 426
live-born Infants with Significant
Prenatally Detected Uropathy

British Journal of Urology volume 81 Page 8 - April 1998

32
Grades of Hydronephrosis
• Mild hydronephrosis:
• Pelvic APD <=1.5 cm and normal calyces
• Moderate hydronephrosis
• Pelvic APD > 1.5 cm and caliectasis with no parenchymal atrophy
• Severe hydronephrosis:
• Pelvic APD > 1.5 cm, caliectasis and cortical atrophy

BJU Inter volume 85 Page 987 - May 2000

33
Prognosis & Severity of ANH
• Prognosis & severity of hydronephrosis: (% needed surgery or prolonged follow-
up):
• RPD > 20 mm, 94%
• RPD 10–15 mm 50%
• RPD was < 10 mm 3%

Grignon A, Filion R, Filiatrault D, et al: Radiology 1986 Sep; 160(3): 645-7

 Outcome of fetal renal pelvic dilatation (Surgery or UTI):

• Mild dilation 0%
• Moderate dilatation 23%
• Severe hydronephrosis 64%

Ultrasound Obstet Gynecol. 2005

May;25(5):483-8. 34
Eneuresis
• Involuntary wetting during sleep without any
inherent suggestions of frequency of bedwetting or
pathophysiology
• Prevalence decreases with age
• Causes not fully understood
• Treatment has a positive effect on the self-esteem
of children and young people. Healthcare
professionals should persist in offering treatments
Principles of care
• Inform children and young people with bedwetting and their
parents or carers that it is not the child or young person’s
fault and that punitive measures should not be used in the
management of bedwetting
 Assessment and
investigation: 1
History taking

Ask about onset of bedwetting, pattern of

bedwetting, daytime symptoms, toileting
patterns, fluid intake and practical issues.

Assess for comorbidities and other factors

that may be associated with bedwetting.
 Advice on fluid intake, diet
and toileting patterns
• Address excessive or insufficient fluid intake or abnormal
toileting patterns before starting other treatment for
bedwetting in children and young people (KPI)
• Adequate daily fluid intake is important
Age Sex Total drinks per day
4 – 8 years Female 1000 – 1400 ml
Male 1000 – 1400 ml

9 – 13 years Female 1200 – 2100 ml

Male 1400 – 2300 ml

14 – 18 years Female 1200 – 2500 ml

Male 2100 – 3200 ml
 Reward systems
• Explain that reward systems with positive rewards for agreed
behaviour rather than dry nights should be used either alone
or in conjunction with other treatments for bedwetting

• Inform parents or carers that they should not use

systems that penalise or remove previously gained
rewards

• Advise parents or carers to try a reward system

alone for the initial treatment of bedwetting in young
children who have some dry nights
Initial treatment: alarms
Who to consider
• Offer an alarm as the first-line treatment to children and
young people whose bedwetting has not responded to
advice on fluids, toileting or an appropriate reward system,
unless the alarm is inappropriate or undesirable.
• Alarm may be inappropriate when:
• bedwetting is very infrequent (that is, less than 1–2 wet
beds per week)
• the parents or carers are having emotional difficulty
coping with the burden of bedwetting
• the parents or carers are expressing anger,
negativity or blame towards
the child or young person
 Initial treatment:
desmopressin
• Offer desmopressin to children and young people over
7 years, if:
• rapid-onset and/or short-term improvement in
bedwetting is the priority of treatment or

• an alarm is inappropriate or undesirable