Title: Renal Problems in children
1Renal Problems in children
- Dr. Rim El-Rifai
- Consultant Paediatrician
- QMHC
- October 2005
2Outline
- Renal malformations and Common Urological
problems - Common Renal Problems
- Summary
3Introduction
- Developmental disorders account for a wide
spectrum of kidney diseases that cause
considerable morbidity and mortality in the first
years of life - Childhood kidney disorders can predispose to
adult morbidity and mortality - Chronic renal diseases can affect growth
4Renal Malformations and Common Urological Problems
5Renal malformations
- The major causes of end-stage renal failure in
children - Can be diagnosed Antenatally
- Can be part of a syndrome
- Some have a genetic basis
- Vesico-ureteric reflux
6Types of abnormalities detected antenatally
- Abnormalities in the size of the kidneys
- Abnormalities in the texture of the renal
parenchyma - Visible cysts
- Hydronephrosis
7Abnormal renal size
- Large
- Polycystic kidney disease
- Multicysticdysplastic
- Cystic dysplasia
- Congenital nephrotic syndrome
- Renal tumour
- Compensatory hypertrophy
- Small
- Renal dysplasia/hypoplasia
- Damage from obstructive uropathy
8Abnormal Parenchyma
- Echobright
- Polycystic disease
- Cystic dysplasia
- Damage from obstructive uropathy
- Glomerulo-cystic disease
- Congenital Nephrotic syndrome
- Macrocysts
- AD PCKD
- TS
- Multicystic dysplastic
- Cystic dysplasia
9Hydronephrosis
- Obstruction
- PUJO
- VUJO/ Megaureter
- PUV
- Ureterocele
- Non-obstruction
- VUR,
- prune-belly s.
10Other renal problems detected antenatally
- Duplication of the upper tract
- Renal agenesis
- Renal fusion and ectopia
11Diagnosis And Management of Antenatal
Hydronephrosis
- Diagnosis
- Antenatal screening
- Postnatal KUB Ultrasound
- MCUG
- MAG3 renogram/ DMSA
- Management
- Prophylactic antibiotics
- Early treatment of UTI and complications
- surgery
12Urinary Tract Infections
13Urinary tract infections
- One of the most common bacterial infections in
childhood (7 of girls- 2 of boys) - Most present to the primary care physician
- Complications can result in end-stage renal
disease and hypertension - Can be as a result of underlying anatomical
abnormalities - E-Coli cause 80-90 of first time UTI
14Risk Factors associated with permanent renal
damage
- Obstruction
- Vesico-ureteric reflux with dilatation
- Younger age (lt 4 years)
- Delay of treatment
- Number of pyelonephritis attacks
- Uncommon bacteria
15Long-term consequences of reflux nephropathy
- Chronic renal failure
- Reflux nephrophathy in 13 of patients 5-44 years
of age with ESRF (Australia and New Zealand) - 30 of CRF in children (Wales)
- 39 of renal transplants (Ireland)
- Hypertension
- Need for annual BP monitoring for life
- Complications of pregnancy
- UTI during pregnancy
- Pregnancy induced hypertension
- Complicated pregnancies, and worse fetal outcome
16Diagnosis and management of childhood UTI
- MSU
- Early antibiotic therapy
- Prophylactic antibiotics
- Imaging USS, MCUG, DMSA, other
- Surgery
- Monitoring of BP annually
17Voiding Dysfunction and The Wet Child
18Normal Sequence of Developing Bladder and Bowel
Control
- Nocturnal bowel control
- Daytime bowel control
- Daytime bladder control
- Nocturnal bladder control
19Classification Primary vs secondary
- Primary mono-symptomatic nocturnal enuresis
- Primary Diurnal enuresis
- structural urological abnormalities.
- Neuropathic Bladder
- Secondary Diurnal enuresis
- UTI
- Dysfunctional voiding
- Concentration abnormalities IDDM, Diabetes
insipidus - Neuropathic bladder
20Characterization of Voiding Dysfunction
- Storage Problem Failure to Store normal volumes
of urine at low pressure without leakage - Non compliant bladder
- overactive bladder
- Inadequate sphincter tone during filling
- Emptying Problem Failure to empty completely, on
command, efficiently at low pressures - Failure of neurological control of bladder
- Bladder muscle failure
- Failure of sphincter relaxation during voiding
21Evaluation of Dysfunctional Voiding
- History
- Physical Exam
- Laboratory Tests
- Imaging and Urodynamics
22Management of the wet child
- Treatment of underlying causes
- UTI
- anatomical abnormalities
- Bladder training (and bowel)
- Drugs
- Anticholinergics
- Desmopressin
- Other
- Alarms, star charts,
- surgery
23Common Renal Problems
24Features of Renal problems
- Clinical
- Oedema
- Polyuria and polydypsia
- Failure to thrive/ short stature
- Hypertension
- Abnormal investigations
- Blood
- UE, Albumin, Bone
- Urinalysis
- Proteinuria
- Haematuria
25Haematuria
26Detection of Haematuria
- Visual examination
- Dipsticks
- Microscopy
27Causes of gross Haematuria in 150 children
Pediatrics 1977
- UTI
- Proven 39
- Suspected 35
- Perineal irritation 16
- Trauma 10
- Acute nephritis 6
- Coagulopathy 5
- Stones 3
- Tumour 1
- Other 35
28IgA Nephropathy
- Male predominance
- More common in 2nd- 3rd decades
- 2-10 of glomerulonephritides in UK
29IgA Nephropathy
- Diagnosis
- macroscopic haematuria,
- asymptomatic microscopic haematuria and
proteinuria, - acute nephritis,
- nephrotic syndrome,
- mixed nephritic-nephrotic synd.
- Renal biopsy deposits of IgA (plasma IgA raised
in 20) - Prognosis
- clinical course variable,
- 50 recurrence in transplanted kidney
30Alport Syndrome
- Inherited nephritis,
- Sensori-neural deafness,
- Occular defects,
- less commonly large Platelets.
- 80-90 X-linked dominant, 10-20 AR
- Diagnosis renal biopsy.
31Alport Syndrome
- Treatment
- ACE inhib. or Angiotensin recept. Blockers,
- blood pressure control,
- Renal transplantation
- Prognosis Poor in boys
- proteinuria, hypertension and renal impairment in
late teens. - Hearing loss in adolescent years
32Thin Basement membrane nephropathy
- Haematuria
- Variable clinical course
- Biopsy DD
- Alports S.
- benign familial haematuria
33 Benign familial haematuria
- Microscopic haematuria
- AD inheritance
- Normal biopsy
- Good prognosis
34Proteinuria
35Proteinuria in Renal disease
- Dipsticks correlate better with level of
albuminuria - 24 hour urinary protein gt60mg/m2/day
- Early morning Urine Protein/creatinine ratio gt 10
25 mg/mmol - Exercise and age related in normal children
36Causes of proteinuria on dipstick
- Artefect alk. Urine, contamination by vag.
Secretions - Benign
- Functional exercise, cold, fever, congestive
heart failure - Idiopathic incidental finding Transient-
Intermittent - Orthostatic transient- fixed (lt2 g/24 hr)
- Persistent/ non-benign
- Persistent isolated
- Disease related
37Disease related Proteinuria
- Glomerular mechanisms increased protein
filtration - Damage to basement membrane
- Loss of glomerular anion
- Increased glomerular permeability
- Tubular causes decreased protein reabsorption
- other
38Proteinuria evaluation
- Exclude non-renal causes
- History, examination, urinalysis
- Documentation of proteinuria
- Dipstick diary BD for 1/52
- 24 hr urine collection
- Random PrCr ratio
- MSU
- GFR measurement est. GFR (Schwartz formula)
- Immunology/serology C3-C4, ASOT,
anti-hyaloronidase, ANA, anti-DNA. - Blood chemistry prot, alb, cholest.
39Disease related proteinuria
- Renal disease
- Glomerular causes
- Tubular causes hereditary, ATN, heavy metal
poisoning - Secretory proteinuria neonates, lower urinary
tract - Other diseases
- Overflow proteinuria
- Histuria
- other
40Nephrotic Syndrome
41Idiopathic Nephrotic syndrome
- More common in boys
- More common in Arabs and people from Indian
subcontinent - Peak incidence 2-5 years
- Minimal change disease the most common
- Genetic component
42Clinical features
- Oedema
- gravity related, effusions
- BP
- usually normal or low,
- paradoxically elevated in 20
- abdominal pain
- hypovolaemia, peritonitis
43Lab. findings
- Urine
- large amounts of albumin (gt50 mg/kg/day)
- microscopic haematuria (23),
- Blood
- Hypoalbuminaemia (lt25 g/l),
- low IgG,
- increased cholesterol,
- usually normal U E,
- reduced total calcium,
- raised or decreased Hb and Hct
44Investigations of Nephrotic Syndrome
- Urine
- Urinalysis and MCS
- Quantification of proteinuria Early morning
PrCr ratio, 24 hr collection - U- Na if hypovolaemia suspected
- Plasma
- U, Cr E
- Albumin, T. Prot., Ca, Phos.
- C3, C4, ASOT, ANA
- Hep B serology
- Varicella IgG status
- FBC
45Treatment of SSNS
- Admit to hospital
- Treat associated infection Penicillin
- Steroids
- evidence that longer initial course ( 6-7 mo vs
2-3 mo) protects from frequent relapses - Supporting treatments diuretics
- Diet
- no-added salt, healthy eating, fluid restriction
46Complications of SSNS
- Infection
- Low IgG and serum factor B (C3 proactivator),
impaired opsonisation and lymphocyte
transformation, immunosupression - Thrombosis
- Thrombocytosis, increased clotting factors (V,
VII, VIII, X, Fibrinogen), reduced Antothrombin
III, hypovolaemia, steroids
47Complications of SSNS
- Acute renal failure
- pre-renal commonly, less common ATN
- Hyperlipidaemia
- mechanism poorly understood
- Malnutrition
- Side effects of treatment
- steroids, alkylating agents, Cyclosporin A,
Levamisole
48Indications for renal biopsy in NS
- Age lt 12 months (continuous or congenital/
infantile NS) - Age gt 16 years
- Persistent hypertension
- macroscopic haematuria
- Impaired renal function unresponsive to
correction of hypovolaemia - Low C3, C4
- Failure to respond to initial course of steroids
49Other Nephrotic Syndromes
- Steroid-resistant NS
- Focal segmental glomerulosclerosis and minimal
change disease rising incident in
African-American children - Membranoproliferative (mesangiocapillary)
glomerulonephritis uncommon - Membranous nephropathy very rare
- Congenital/ Infantile NS
50Acute Nephritis Syndrome
51Acute Nephritis Syndrome
- Acute glomerular injury and inflammation with
decreased GFR and Na and water retention - Urinalysis
- Haematuria Albuminuria red cell casts
- Most common cause
- Acute post-streptococcal Glomerulo-nephritis
(APSGN) 80
52Other causes of acute nephritis
- IgA Nephropathy
- HSP
- Membranoproliferative Glomerulonephritis
- Lupus nephritis
- ANCA-positive vasculitis
- Chronic infections
- Shunt nephritis, Infective endocarditis, Hep B,
Hep C, HIV nephropathy
53Acute post-Streptococcal Gromelunephritis(APSGN)
54APSGN
- Post throat or skin infection
- nephritogenic group A beta-haemolytic
streptococcus - Risk of APSGN is 10-15, (40 within families)
- ABs do not prevent GN but important to prevent
further spread of bacteria
55APSGN
- Age 5-15 years
- More common in males
- Antigen-antibody related nephritis
- Abrupt onset 7-14 days after throat infection and
3-6 weeks after skin infection
56Renal involvement in APSGN
- Mild asymptomatic Haematuria
- Acute renal failure with oligo-anuria
- (rarely necessitating dialysis)
57Clinical features of APSGN
- Acute fluid overload
- Peripheral oedema
- Pulmonary oedema
- Congestive heart failure
- Hypertension
- Haematuria (micro /- macro)
- Proteinuria
- Renal function impairment
- Oliguria
- Elevated plasma creatinine
58Investigations
- Urinalysis
- M C S,
- early morning Pr Cr ratio
- Bacteriology
- throat swab,
- ASOT,
- Streptozyme essay ( strptolysin O, streptokinase,
DNAse B, Hyaloronidase, NADase antibodies)
59Investigations
- Immunology
- C3 and C4
- (ANCA, ANA and double straded DNA Ab, GBM Ab)
- Renal function
- U Cr E, acid-base, plasma proteins, Ca and phos.
- Haematology
- FBC, blood film
60Management
- Eradication of organism
- Treatment of renal failure supportive
- Indications for in-patient management
- Hypertension
- Oedema
- Oliguria
- Elevated plasma creatinine
- Electrolyte abnormalities
61Clinical course and long term prognosis
- Most symptoms subside in 2-3 weeks
- C3 back to normal in 8-12 weeks
- Microscopic haematuria /- low grade proteinuria
can persist for 1-2 years - Excellent prognosis overall in children
62Renal manifestations of Systemic Disorders
63Childhood vasculitis
- Systemic symptoms malaise, fever weight loss
- purpuric skin rash
- Haematuria and red cell casts
- Arthropathy
- Serositis
- Unexplained cardiac or pulmonary disease
- Lab anaemia, leukocytosis, thrombocytosis,
raised ESR or CRP, ANCA ve
64Henoch-Schönlein Purpura
- Multisystem small vessel systemic vascultitis
- Prominent cutaneous component
- Most common vasculitis in children
- 14/100 000 of children
- Most favourable outcome
65HSP nephritis
- Incidence varies greatly (20-61)
- Up to 2 months from presentation
- Isolated haematuria- acute nephritis picture
- Treatment supportive
- Steroids in severe GI,
- Immunosuppressant for severe renal involvement
66Prognosis HSP nephritis
- Risk of chronic renal impairment lt2 overall
- CRF
- up to 10 in patients referred to Nephrologist
- Picture of nephritic/nephrotic nature and
crescentic changes on biopsy - Late deterioration in renal function well
recognised long term FU needed
67Other vasculitis syndromes
- SLE
- Kawasaki disease
- Takayasus arteritis
- Polyarteritis nodosa
- Wegenrs granulomatosis
68Other renal manifestations of systemic diseases
- Cystic Fibrosis
- Nephrotoxic drugs
- Tubulo-interstitial nephritis
- IgA Nephropathy
- Diabetes Mellitus
- microalbuminuria
69Summary
- Antenatal screening can detect a significant
number of renal and urological abnormalities - Prevention and treatment of reflux nephropathy
can prevent ESRD - Nephro-Urological problems in childhood are
reasonably common - Spectrum of childhood nephro-urological problems
extend through adolescent age necessitating close
collaboration with adult nephrologists