Renal Problems in children

1
Renal Problems in children

• Dr. Rim El-Rifai
• Consultant Paediatrician
• QMHC
• October 2005

2
Outline

• Renal malformations and Common Urological
  problems
• Common Renal Problems
• Summary

3
Introduction

• 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

4
Renal Malformations and Common Urological Problems
5
Renal 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

6
Types of abnormalities detected antenatally

• Abnormalities in the size of the kidneys
• Abnormalities in the texture of the renal
  parenchyma
• Visible cysts
• Hydronephrosis

7
Abnormal renal size

• Large
• Polycystic kidney disease
• Multicysticdysplastic
• Cystic dysplasia
• Congenital nephrotic syndrome
• Renal tumour
• Compensatory hypertrophy
• Small
• Renal dysplasia/hypoplasia
• Damage from obstructive uropathy

8
Abnormal Parenchyma

• Echobright
• Polycystic disease
• Cystic dysplasia
• Damage from obstructive uropathy
• Glomerulo-cystic disease
• Congenital Nephrotic syndrome
• Macrocysts
• AD PCKD
• TS
• Multicystic dysplastic
• Cystic dysplasia

9
Hydronephrosis

• Obstruction
• PUJO
• VUJO/ Megaureter
• PUV
• Ureterocele
• Non-obstruction
• VUR,
• prune-belly s.

10
Other renal problems detected antenatally

• Duplication of the upper tract
• Renal agenesis
• Renal fusion and ectopia

11
Diagnosis 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

12
Urinary Tract Infections
13
Urinary 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

14
Risk 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

15
Long-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

16
Diagnosis and management of childhood UTI

• MSU
• Early antibiotic therapy
• Prophylactic antibiotics
• Imaging USS, MCUG, DMSA, other
• Surgery
• Monitoring of BP annually

17
Voiding Dysfunction and The Wet Child
18
Normal Sequence of Developing Bladder and Bowel
Control

• Nocturnal bowel control
• Daytime bowel control
• Daytime bladder control
• Nocturnal bladder control

19
Classification 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

20
Characterization 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

21
Evaluation of Dysfunctional Voiding

• History
• Physical Exam
• Laboratory Tests
• Imaging and Urodynamics

22
Management of the wet child

• Treatment of underlying causes
• UTI
• anatomical abnormalities
• Bladder training (and bowel)
• Drugs
• Anticholinergics
• Desmopressin
• Other
• Alarms, star charts,
• surgery

23
Common Renal Problems
24
Features of Renal problems

• Clinical
• Oedema
• Polyuria and polydypsia
• Failure to thrive/ short stature
• Hypertension
• Abnormal investigations
• Blood
• UE, Albumin, Bone
• Urinalysis
• Proteinuria
• Haematuria

25
Haematuria
26
Detection of Haematuria

• Visual examination
• Dipsticks
• Microscopy

27
Causes 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

28
IgA Nephropathy

• Male predominance
• More common in 2nd- 3rd decades
• 2-10 of glomerulonephritides in UK

29
IgA 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

30
Alport Syndrome

• Inherited nephritis,
• Sensori-neural deafness,
• Occular defects,
• less commonly large Platelets.
• 80-90 X-linked dominant, 10-20 AR
• Diagnosis renal biopsy.

31
Alport 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

32
Thin 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

34
Proteinuria
35
Proteinuria 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

36
Causes 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

37
Disease related Proteinuria

• Glomerular mechanisms increased protein
  filtration
• Damage to basement membrane
• Loss of glomerular anion
• Increased glomerular permeability
• Tubular causes decreased protein reabsorption
• other

38
Proteinuria 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.

39
Disease 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

40
Nephrotic Syndrome
41
Idiopathic 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

42
Clinical features

• Oedema
• gravity related, effusions
• BP
• usually normal or low,
• paradoxically elevated in 20
• abdominal pain
• hypovolaemia, peritonitis

43
Lab. 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

44
Investigations 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

45
Treatment 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

46
Complications 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

47
Complications 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

48
Indications 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

49
Other 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

50
Acute Nephritis Syndrome
51
Acute 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

52
Other 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

53
Acute post-Streptococcal Gromelunephritis(APSGN)
54
APSGN

• 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

55
APSGN

• 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

56
Renal involvement in APSGN

• Mild asymptomatic Haematuria
• Acute renal failure with oligo-anuria
• (rarely necessitating dialysis)

57
Clinical 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

58
Investigations

• Urinalysis
• M C S,
• early morning Pr Cr ratio
• Bacteriology
• throat swab,
• ASOT,
• Streptozyme essay ( strptolysin O, streptokinase,
  DNAse B, Hyaloronidase, NADase antibodies)

59
Investigations

• 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

60
Management

• Eradication of organism
• Treatment of renal failure supportive
• Indications for in-patient management
• Hypertension
• Oedema
• Oliguria
• Elevated plasma creatinine
• Electrolyte abnormalities

61
Clinical 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

62
Renal manifestations of Systemic Disorders
63
Childhood 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

64
Henoch-Schönlein Purpura

• Multisystem small vessel systemic vascultitis
• Prominent cutaneous component
• Most common vasculitis in children
• 14/100 000 of children
• Most favourable outcome

65
HSP 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

66
Prognosis 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

67
Other vasculitis syndromes

• SLE
• Kawasaki disease
• Takayasus arteritis
• Polyarteritis nodosa
• Wegenrs granulomatosis

68
Other renal manifestations of systemic diseases

• Cystic Fibrosis
• Nephrotoxic drugs
• Tubulo-interstitial nephritis
• IgA Nephropathy
• Diabetes Mellitus
• microalbuminuria

69
Summary

• 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