NEPHROTIC SYNDROME, ACUTE

GLOMERULO-NEPHRITIS
Learning Outcomes
1. Describe the common causes of proteinuria and haematuria
2. Describe the epidemiology, pathophysiology, clinical features of
nephrotic syndrome and acute glomerulonephritis
3. Describe the relevant investigations and findings
4. Describe the complications
5. Outline the principles of management of nephrotic syndrome and
acute glomerulonephritis

Causes of proteinuria

Causes of haematuria

Non-glomerular

Orthostatic proteinuria

Glomerular abnormalities
(disruption of normal glomerular barrier to protein filtration)
Minimal change disease
Glomerulonephritis
Abnormal glomerular basement membrane (familial nephritides)
Increased glomerular filtration pressure
Reduced renal mass
Hypertension
Tubular proteinuria(impaired reabsorption or secretion of
protein)
Transient proteinuria : fever,exercise,dehydration ,seizure

Infection (bacterial, viral, TB, schistosomiasis)

Trauma to genitalia, urinary tract or kidneys
Renal Stones
Renal Tumours
Sickle cell disease
Thrombocytopenia
Renal vein thrombosis
Hypercalciuria.

Glomerular
APSGN and NAPSGN
IgA nephropathy

Primary
renal
disease

Familial nephritis, e.g. Alport syndrome

Thin basement membrane disease
SLE,HSP,HUS,subacute bacterial endocarditis (secondary due to
systemic ds)

Epidemiology
Nephrotic
syndrome

AGN

The incidence is approximately 2/100,000 children with a peak age

of onset

Children aged <6yrs.(preschool age)

Boys are more commonly affected than girls (2:1)

There is an increased frequency in certain ethnic groups, e.g. Indian

subcontinent.

PSGN 6-10yr of age (school age)

Common in boys

10 days (5-21 days) streptococcal pharyngitis ; 4-6 weeks after

impetigo

PIGN- same manner following infection with other bacterial and viral
pathogens

IgA nephropathy- hematuria concurrent with URTI

Etiology
Nephrotic syndrome

AGN
Post-infectious
Bacterial: streptococcal commonest, Staphylococcus aureus,

Unknown

Mycoplasma pneumoniae, Salmonella

secondary to systemic diseases such as

Virus: herpesviruses (EBV, varicella, CMV)

HSP

Fungi: candida, aspergillus

Other vasculitides (SLE)

Parasites: toxoplasma, malaria, schistosomiasis

Infections (e.g. malaria)

Allergens (e.g. bee sting).

Others (less common)

MPGN
IgA nephropathy
Systemic lupus erythematosis
Subacute bacterial endocarditis
Shunt nephritis

Pathophysiology
Nephrotic syndrome

Injury to the podocyte appears to be the main cause of proteinuria,

podocytes wrap around the glomerular capillaries and maintain the
filtration barrier, preventing large molecular weight proteins from
entering the urine.
Effacement of the foot processes or loss of podocytes can cause heavy
protein loss.

AGN

Glomerulonephritis simply means inflammation of the glomeruli and

nephrons

when you remember this you can appreciate what the consequences
of this inflammation are:

Damage to the glomerulus restricts blood flow, leading to

compensatory INCREASING BP
Damage to the filtration mechanism allows protein and blood to enter
the urine
Loss of the usual filtration capacity leads to acute kidney injury

Clinical
features
Nephrotic
AGN

syndrome
Generali
sed
oedema

Puffy face ( hypoproteinemia due to massive proteinuria )

{scrotal/ vulval}, leg and ankle oedema

Ascites

Breathlessness (due to pleural effusions and abdominal

distension.)

FORTHY urine ( proteinuria)

Hyperlipidaemia

Haematuria(tea-coloured or smoky urine )

Transient renal impairment (e. g Oliguria ,azotaemia , uraemia

)

Hypertension(may cause seizures)

Localised oedema (e.g. facial puffiness.)

Relevant investigation and findings

Urine

Nephrotic syndrome

Urinalysis: protein +++.

Microscopy: haematuria/casts (suggest causes other than
MCD).
Na+: If <10mmol/L suggests hypovolaemia. (Note: If patient
has received diuretics this is not accurate.)
Protein: creatinine ratio (early morning urine specimen).
Bloods
Serum albumin (reduced, <25g/L).
U&E/creatinine (decreased sodium and total calciumwith
normal ionized Ca).

AGN
Urine:
urinalysis by dipstick: haematuria +/ proteinuria;
microscopycasts (mostly red cell casts).

Throat swab: culture.

Bloods:

FBC; Anaemia (mainly dilutional).

U&E, including creatinine, bicarbonate, calcium,
phosphate
albumin;

C3/C4 (if decreased suggests not MCD).

ASOT/antiDNAase B;

Consider ANF, ASOT, ANCA, Ig if mixed nephritic/ nephrotic

picture.

complement (expect low C3, normal C4);

Lipids: total cholesterol , LDL , VLDL

Renal US (urgent).

Haemoglobin may be {IN/DE}crease depending on plasma

volume.

CXR (if fluid overload suspected).

Hepatitis B and C screen

autoantibody screen (include ANA).

Complication
Nephrotic syndrome

Bacteraemia and peritonitis (strep pneumoniae , E.coli, Klebsiella )

loss of Ig & complement

Steroid side effect common in steroid-dependent and relapsing pt

TE(thromboembolism) urine loss antithrombin, plasminogen

coagulation factor.

Hypovolaemia shift of fluid from intravascular space, symptoms

oliguria, abd pain, anorexia, postural hypotension

Acute renal failure This is pre-renal and s to hypovolaemia.

Atherosclerotic vascular ds hyperlipidaemia

AGN

Hypertensive encephalopathy usually presenting with seizures

Pulmonary oedema (acute left ventricular failure)
Acute renal failure

ESRF

Prognosis
NS

MOST children remission

80% with MCNS experience NS relapse(Heavy proteinuria

persists for 3 or more consecutive days)

Steroid responsive pt little risk of CRF

AGN
95% with post-streptococcal glomerular nephritis (GN) show
complete recovery.
Microscopic haematuria may persist for 12yrs.
Discharge from follow-up once urinalysis, BP, and creatinine
are normal.

IgA nephropathy- high risk to ESRF if BP increase

Principal of Management
Nephrotic syndrome

AGN

Treat hypovolaemia if present but albumin infusion is not routine.

Fluid restriction to 8001000mL/24hr.
Diuretics if very oedematous and no evidence of hypovolaemia.
Furosemide/spironolactone.

Most require admission because of fluid balance, worsening renal

function, or HTN. Treat life-threatening complications first:
hyperkalaemia, acidosis , seizures , hypocalcaemia

Steroid therapy:

oral prednisolone 60mg/m2/day for 4wks;

followed by 40mg/m2/alternate days for 4wks; then
stopslow wean over next 4mths with slow taper, but need to consider
side-effects of steroids.
Dietary sodium restriction
Prophylactic antibiotics (oral penicillin V) until oedema-free
Immunize with pneumococcal vaccine.

Dietary sodium restriction

Diuretic, antihypertensive agents

-blockers and calcium channel blocker usual first choice;

****Note: Do not use ACE inhibitor (may worsen renal function).

Antibiotic treatment for active streptococcal infection

Corticosteroid, immunosuppressant used

Key msg