Paediatrics: Nephrotic syndrome: complications and follow-up

Nephrotic syndrome: complications and follow-up

Complications

Complications are 2^o to the relative hypovolaemic state and to impaired immunity.

Infection

Predisposition to infection is  2^o to decreased IgG levels, and to impaired opsonization due to steroid immunosuppression. Bacterial peritonitis (especially Streptococcus pneumoniae) is an important complication and should be considered in any child with nephrotic syndrome who com-plains of abdominal pain. Urgent assessment, cultures, and IV antibiotic therapy are required.

Thrombosis

Nephrotic syndrome produces a hypercoagulable state and predisposition to both arterial and venous thrombosis is recognized.

Hypovolaemia

Suggested by development of oliguria and or presence of low BP. Patients may also complain of abdominal pain. If present, administration of an in-fusion of 20% human albumin solution 1g/kg over 2hr with furosemide (2mg/kg IV) should be given.

Acute renal failure 

This is pre-renal and  2^o to hypovolaemia.

Indications for renal biopsy

The majority of patients will have MCD and will respond to steroids.

Biopsy is therefore reserved for those with atypical features:

·  Age <12mths or >12yrs.

·  Increased BP.

·  Macroscopic haematuria.

·  Impaired renal function.

·  Decreased C3/C4.

·  Failure to respond after 1mth of daily steroid therapy.

Follow-up

Prognosis

·  30% single relapse.

·  30% occasional relapses.

·  30% steroid dependence.

Relapse

·  Many patients with steroid-sensitive nephrotic syndrome will relapse. A relapse is defined as detection of urine dipstick ++ proteinuria for >3 days.

·  Frequent relapse is defined as >2 relapses within 6mths of initial response or 4 or more relapses in any 12mths.

Management of relapses

Each relapse is treated with oral steroids in a similar manner to above. Alternative strategies for frequent relapsers include a trial of therapy with other agents such as:

·Cyclophosphamide.

·Levamisole.

·Ciclosporin A.

·Other agents including the immunosuppressants tacrolimus, mycophenolate mofetil and anti-CD20 monoclonal antibody (rituximab) may be considered.