Renal Problems in children

Dr. Rim El-Rifai

Consultant Paediatrician

QMHC

October 2005

Outline

Renal malformations and Common Urological problems

Common Renal Problems Summary

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

Renal Malformations and Common Urological Problems

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

Types of abnormalities detected antenatally

Abnormalities in the size of the kidneys

Abnormalities in the texture of the renal parenchyma

Visible cysts Hydronephrosis

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

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

Hydronephrosis

• Obstruction: • PUJO• VUJO/ Megaureter• PUV• Ureterocele

• Non-obstruction: • VUR, • prune-belly s.

Other renal problems detected antenatally

Duplication of the upper tract Renal agenesis Renal fusion and ectopia

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

Urinary Tract Infections

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

Risk Factors associated with permanent renal damage

Obstruction Vesico-ureteric reflux with dilatation Younger age (< 4 years) Delay of treatment Number of pyelonephritis attacks Uncommon bacteria

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

Diagnosis and management of childhood UTI

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

Voiding Dysfunction and The Wet Child

Normal Sequence of Developing Bladder and Bowel Control

Nocturnal bowel control

Daytime bowel control

Daytime bladder control

Nocturnal bladder control

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

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

Evaluation of Dysfunctional Voiding

History Physical Exam Laboratory Tests Imaging and Urodynamics

Management of the wet child Treatment of underlying causes:

– UTI– anatomical abnormalities

Bladder training (and bowel) Drugs:

– Anticholinergics – Desmopressin

Other:– Alarms, star charts, – surgery

Common Renal Problems

Features of Renal problems Clinical:

– Oedema– Polyuria and polydypsia– Failure to thrive/ short stature– Hypertension

Abnormal investigations– Blood:

» U&E, Albumin, Bone

– Urinalysis» Proteinuria» Haematuria

Haematuria

Detection of Haematuria

Visual examination Dipsticks Microscopy

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

IgA Nephropathy

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

UK

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

Alport Syndrome

1. Inherited nephritis, 2. Sensori-neural deafness, 3. Occular defects, 4. less commonly large Platelets.

80-90% X-linked dominant, 10-20% AR

Diagnosis: renal biopsy.

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

Thin Basement membrane nephropathy

Haematuria Variable clinical course Biopsy DD:

–Alports S.

–benign familial haematuria

Benign familial haematuria

Microscopic haematuria AD inheritance Normal biopsy Good prognosis

Proteinuria

Proteinuria in Renal disease

Dipsticks: correlate better with level of albuminuria

24 hour urinary protein >60mg/m2/day Early morning Urine Protein/creatinine

ratio > 10 –25 mg/mmol Exercise and age related in normal

children

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 (<2 g/24 hr)

Persistent/ non-benign:– Persistent isolated– Disease related

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

Proteinuria evaluation Exclude non-renal causes History, examination, urinalysis Documentation of proteinuria:

– Dipstick diary: BD for 1/52– 24 hr urine collection– Random Pr:Cr ratio

MSU GFR measurement: est. GFR (Schwartz formula) Immunology/serology: C3-C4, ASOT, anti-hyaloronidase,

ANA, anti-DNA. Blood chemistry: prot, alb, cholest.

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

Nephrotic Syndrome

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

Clinical features

Oedema: – gravity related, effusions

BP: – usually normal or low, – paradoxically elevated in 20%

abdominal pain: – hypovolaemia, peritonitis

Lab. findings

Urine: – large amounts of albumin (>50 mg/kg/day)– microscopic haematuria (23%),

Blood: – Hypoalbuminaemia (<25 g/l), – low IgG, – increased cholesterol, – usually normal U & E, – reduced total calcium, – raised or decreased Hb and Hct

Investigations of Nephrotic Syndrome

Urine:– Urinalysis and MC&S– Quantification of proteinuria: Early morning Pr:Cr 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

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

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

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

Indications for renal biopsy in NS

Age < 12 months (continuous or congenital/ infantile NS)

Age > 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

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

Acute Nephritis Syndrome

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%

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

Acute post-Streptococcal Gromelunephritis

(APSGN)

APSGN

Post throat or skin infection – nephritogenic group A beta-haemolytic

streptococcus Risk of APSGN is 10-15%, (40% within

families)

AB’s do not prevent GN but important to prevent further spread of bacteria

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

Renal involvement in APSGN

Mild asymptomatic Haematuria

Acute renal failure with oligo-anuria

(rarely necessitating dialysis)

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

Investigations

Urinalysis:– M C &S, – early morning Pr: Cr ratio

Bacteriology: – throat swab, – ASOT, – Streptozyme essay ( strptolysin O,

streptokinase, DNAse B, Hyaloronidase, NADase antibodies)

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

Management

Eradication of organism Treatment of renal failure: supportive

Indications for in-patient management: Hypertension Oedema Oliguria Elevated plasma creatinine Electrolyte abnormalities

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

Renal manifestations of Systemic Disorders

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

Henoch-Schönlein Purpura

Multisystem small vessel systemic vascultitis

Prominent cutaneous component

Most common vasculitis in children

14/100 000 of children Most favourable outcome

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

Prognosis: HSP nephritis

Risk of chronic renal impairment <2% 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

Other vasculitis syndromes

SLE Kawasaki disease Takayasu’s arteritis Polyarteritis nodosa Wegenr’s granulomatosis

Other renal manifestations of systemic diseases

Cystic Fibrosis– Nephrotoxic drugs– Tubulo-interstitial nephritis– IgA Nephropathy

Diabetes Mellitus– microalbuminuria

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