Nephrotic Syndrome and Acute Glomerulonephritis

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ACUTE NEPHROTIC SYNDROME 

CLINICAL DESCRIPTION

This condition is associated with proteinuria in excess of 3-3.5 g/1.73 m2 daily accompanied by hypoalbuminemia, oedema, hyperlipidemia and hypercoagulable state. Most often caused as a complication of a streptococcal infection. Usually manifests itself 1 – 5 weeks after an episode of pharyngitis, impetigo or infected scabies.

CLINICAL FEATURES

SIGNS AND SYMPTOMS

  • Oedema, low albumin, ascites, periorbital oedema, pleural effusion, genital oedema, protein energy malnutrition (especially in children).

INVESTIGATIONS

  • Urinalysis, Urine microscopy (look for casts and check for Schistosoma ova), MPs and PCV, Electrolytes, Urea and Creatinine, Imaging on individual basis: renal USS, CXR, cardiac Echo.

TREATMENT

NON-PHARMACOLOGIC

  • Monitor BP, urine output, weight
  • Avoid added salt
  • Treatment is usually supportive

PHARMACOLOGICAL

Adult:

For control of oedema:

  • Give Frusemide 40-80 mg 24 hourly (may require intravenous frusemide)

If hypertension is present:

  • Treat accordingly (hypertension treatment section)

For control of proteinuria:

Use Angiotensin Converting enzyme inhibitors (i.e. Enalapril)

Note: Renal diseases can easily be mistaken for malnutrition

 

NEPHROTIC SYNDROME IN CHILDREN

CLINICAL DESCRIPTION

Presence of nephrotic range proteinuria, oedema, hyperlipidemia, and hypoalbuminemia

Criteria

  1. Edema
  2. Proteinuria (at least 3+)
  3. Hypoalbuminemia usually plus Hyperlipidemia

Causes

Primary:

  • Minimal change disease, Focal segmental glomerulosclerosis, Membranoproliferative glomerulonephritis

Secondary:

  • Hepatitis B, HIV, Lupus, Postinfectious GN, Subacute infective endocarditis

Congenital:

  • Occur in children less than 1 year of age

CLINICAL FEATURES

SIGNS AND SYMPTOMS 
  • Signs of systemic disease like joint complaints, rash, fever
  • Preceding symptoms or illnesses e.g. respiratory, skin or urinary tract infections
  • Oedema
  • Abdominal pain, flank pain
  • Breathlessness and cough
  • Past medical history of similar problem
  • Family history of renal problems
  • Ascites
INVESTIGATIONS
  • Urine dipstick and microscopy
  • FBC,
  • Urea, electrolytes and Creatinine
  • albumin
  • HIV test
  • Hepatitis B and C
  • VDRL
  • Malaria parasites
  • Stool microscopy
  • Kidney ultrasound

TREATMENT

First episode

  • Admit
  • Salt restriction
  • fluid restriction if fluid overload
  • Stat dose Praziquantel
  • Monitor daily: blood pressure, weight and urine dipstick until normal
  • Encourage mobilization (bed rest may increase risk of venous thrombosis.)
  • Steroids: Prednisolone 2mg/kg/day for 4-6 weeks, If no response in 4 -6 weeks, refer to tertiary facility.
  • Diuretics: if severely fluid overloaded 
    • Furosemide 1mg/kg IV(maximum 40mg) 12-8 hourly
    • If no response after few days, refer to tertiary facility
  • Pneumococcal vaccine, if available, and not previously immunized
  • Follow up all patients in paediatric/renal clinic

Relapses

Proteinuria at least ++ for more than 3 days

  • Start treatment as above
  • Refer to tertiary facility
  • The same as above, except

Complications

  • Infections
  • Thromboembolism (Doppler Ultrasound)
  • Acute kidney injury
  • Hypovolemia
  • Protein malnutrition
  • Hyperlipidemia
  • Spontaneous bacterial peritonitis

Referral criteria 

  • Not responding after 4 -6 weeks of steroids
  • All cases of relapse
  • Severe fluid overload not responding to diuretics
  • Patients with complications
NEPHROTIC SYNDROME IN ADULTS 
Adults
  • Give Frusemide 40-80 mg as a single dose each morning
  • Give Enalapril 10-20 mg  every day (use  with caution, stop if renal function deteriorates)
  • A trial of steroids is indicated (responsiveness to steroids in adults is less than in children). Give Prednisolone 50-60mg every day for up to two months, tapering off is required after response
  • Give Proton pump inhibitors and calcium carbonate plus vitamin D tablets for bone protection if long term steroid use is indicated

If Schistosomiasis is diagnosed or suspected as cause

  • Give Praziquantel 40 mg/kg single dose

Referral Criteria: 

Refer all patients to a physician specialist, pediatrician or nephrologist immediately after diagnosis and stabilization.

If presentation is acute:

  • Give Phenoxymethyl-Penicillin 500mg 6 hourly for 7 days
  • Refer to Nephrologist

Note: Furosemide should not be given to children as routine treatment. Steroids use in nephrotic syndrome should be discussed with nephrologist.

 

ACUTE GLOMERULONEPHRITIS IN CHILDREN (NEPHRITIS SYNDROME)

CLINICAL DESCRIPTION

Acute glomerular injury, defined by:

  • Hematuria (may be macroscopic) and RBC casts in urine
  • Hypertension
  • Oliguria and increasing creatinine
  • Mild Proteinuria (not nephrotic range)
  • Oedema

CLINICAL FEATURES

SIGNS AND SYMPTOMS 
  • Preceding symptoms or illnesses (Infections, bloody diarrhea, joint pains, rash):
  • Oliguria or anuria
  • Haematuria
  • Abdominal pain
  • Breathlessness, cough
  • Headache, convulsions (hypertension) hypertension
  • Past medical history of similar problem
  • Family history of kidney diseases
  • Oedema and pulmonary oedema
  • Ascites

Causes 

  • Mostly post-infectious glomerulonephritis Membranoproliferative glomerulonephritis (MPGN)
  • Lupus nephritis
  • Haemolytic uraemic syndrome
  • Vasculitides, such as Henoch Schonlein Purpura (IgA nephropathy)
  • Sickle cell disease
INVESTIGATIONS
  • Blood pressure
  • Urine dipstick and microscopy – Haematuria with red cell casts on microscopy,
  • proteinuria and leucocyturia can be present
  • FBC
  • Creatinine, urea and electrolytes
  • Albumin
  • HIV- test, Hepatitis B and C
  • Sickle cell test
  • Renal ultrasound
    • chest X-ray and Cardiac echo

TREATMENT

  • Salt restriction
  • Furosemide 1-2mg/kg IV, if moderate/ severe fluid overload and hypertension
  • Treat blood pressure Start Nifedipine 0.25-0.3mg/kg 6-8 hourly, if hypertensive.
  • Give Praziquantel 40mg/kg PO STAT.
  • When poststreptococcal glomerulonephritis is suspected, give Amoxicillin 15mg/kg PO 8 hourly for 10 days
  • Follow up in pediatric or renal clinic

Complications:

  • Hypertensive emergency
  • Acute kidney injury
  • Chronic kidney disease

Referral

  • Hypertensive emergency
  • Persistent uncontrolled hypertension
  • Acute kidney injury