A. Mild cases are asymptomatic with less proteinuria than found in
nephrotic syndrome. Moderate cases have nephrotic syndrome with or
without hematuria. Severe cases have acute renal failure, severe
hypertension, or cardiorespiratory complications of hypoalbuminemia
related to hypovolemia, pleural effusions, and/or ascites.
B. Hypoalbuminemia can cause severe edema, ascites, pleural
effusions, and hypovolemia with azotemia. Treat hypoalbuminemia with
1 to 2 g/kg of 25 percent salt-poor albumin intravenously over
several hours depending on circulatory volume status followed by
furosemide 1 to 2 mg/kg intravenously. When hypovolemia results in
hypotension, do riot use furosemide.
C. Hypertension should be treated with appropriate medication (see p
84). When decreased renal profusion is contributing to hypertension,
establish urine output with furosemide and then consider an albumin
infusion.
D. Treat children with presumed idiopathic nephrotic syndrome of
childhood initially with prednisone 2 mg/kg per day (maximum 60mg),
until the urine is protein-free for 5 consecutive days or for a
maximum of 6 weeks. Many (73 percent) cases of idiopathic nephrotic
syndrome respond within 2 weeks and most (94 percent) respond within
1 month. After remission has been achieved, maintain the patient
with 2 mg/kg on alternate days for 1 to 2 months. During the third
month of therapy, taper the dose by 5 mg every 2 weeks. Monitor
proteinuria daily with an albumin-sensitive dipstick. Relapse is
defined as 3 consecutive days with proteinuria measuring 3+ or more.
Relapses are often associated with respiratory infections (virus,
Mycoplasma, and Chlamydia).
E. If the nephrotic syndrome recurs as the prednisone dosage is
tapered, steroid-dependent disease is present. These patients, as
well as frequent relapsers who experience three or more relapses per
year, require long-term alternate-day prednisone. A maintenance dose
of 1.4 mg/kg every other day controls most patients and produces
only minimal steroid toxicity However, attempts should be made to
accomplish complete discontinuance of steroids whenever possible.
Alkylating agents, such as cyclophosphamide and chlorambucil, may
decrease the relapse rate but are associated with significant side
effects, including abnormal gonadal function, alopecia, leukopenia,
hemorrhagic cystitis, and predisposition to overwhelming infection
with varicella or measles. Cyclosporin A is now being examined for
use in steroid-dependent or resistant cases.
Nephrotic Syndrome Terminology
vNephrotic syndrome:
Edema; plasma albumin <25 g/L; proteinuria >40 mg/m2/hr or protein:
creatinine ratio >200 mg/mmol.
vRemission:
Urinary protein excretion <4 mg/hr/m2 or Albustix = 0/trace for 3
consecutive days.
vSteroid responsive:
Remission achieved with steroid therapy alone.
vLate responder:
Remission occurring after 4 weeks prednisolone 60 mg/m2/without
other drugs
vRelapse:
Urinary protein excretion >40 mg/hr/m2 or Albustix = ++ or more for
3 consecutive days, having previously been in remission.
vFrequent relapses:
Two or more relapses within 6 months of initial response or 4 or
more relapse within any 12-month period
vSteroid dependence:
Two consecutive relapses occurring during corticosteroid treatment
or within 14 days of its cessation.
vSteroid resistance:
Failure to achieve response in spite of 4 weeksأ¢â‚¬â„¢
prednisolone 60 mg/m2/day
vEarly nonresponder:
Steroid resistance in the initial episode
vLate nonresponder:
Steroid resistance developing in a patient who had previously been
steroid responsive
Management of Minimal Lesion Glomerulonephritis
Treatment of minimal lesion glomerulonephritis in children
v Treatment of the first attack
Prednisone at 60 mg/m2/day (up to a maximum of 80 mg/day) for four
to six weeks and then 40 mg/m2 of prednisone every other day to four
to six weeks are recommended.
Treatment of Relapse
For patients with minimal lesion disease who relapse after initial
treatment, prednisone should be given at 60 mg/m2/day (up to 80
mg/day) until the urine is protein free; then 40 mg/m2 should be
given every other day for 4 weeks.
Treatment of Frequently Relapsing Minimal Lesion Disease
Patients with minimal lesion disease who relapse frequently should
be treated with one of these regimens.
1. Cyclosphosphamide or chlorambucil for 8 wks
2. Repeat relapse therapy with prednisone
3. Symptomatic treatment only (Na restriction, diuretics
4. Long term alternate day prednisone
and/or
5. Levamisole
Treatment of Steroid-dependent Minimal Lesion Disease
Children with steroid-dependent minimal lesion should be treated
with:
1.) 2 mg/kg/day of cyclophosphamide for 12
weeks or
2.) 6 mg/kg/day of cyclosporine for children and
5 mg/kg/day for adults, with the duration
being uncertain
Conservative Treatment to Slow Deterioration of Renal
FunctionProtein-restricted Diets
1. Protein-restricted diets (PRDs) cannot be recommended for all
patients with renal disease.
2. PRDs should be considered for patients with severe renal
impairment (serum creatinine of more than 350 mmol/liter. However,
there is concern that such a restrictive diet may not be
nutritionally sufficient, and there is conflicting evidence on the
ability of patients to adhere to a very low protein diet.
3. Diet
ط£آ¨ Protein intake ط¢آ» 130-140% of RDA.
ط£آ¨ Salt restriction - prevention and treatment of edema.
ط£آ¨ Fluid restriction - moderate-severe hyponatremia <125
mcq/L.
ط£آ¨ Saturated fat reduction.
ط£آ¨ Carbohydrate - starch or dextrose - maltose
- avoid sucrose
Indications for Alternative Therapy
1. Relapse on prednisone dosage >0.5 mg/kg body weight/alternate
days plus one or more of the following:
a. Unacceptable side effects of corticosteroid therapy
b. High risk of toxicity - boys approaching puberty, or diabetes.
c. Unusually severe relapses; hypovolemia, thrombosis,severe sepsis,
or acute renal failure.
d. Inadequate facilities for follow-up or concern about compliance
2. Relapse on prednisolone dosage > 1 mg/kg body weight/alternate
days.
Indications for Renal Biopsy
Pretreatment
Recommended:
1. Onset age <6 mo
2. Macroscopic hematuria
3. Microscopic hematuria & persistent hypertension
4. Low plasma C3
5. Renal failure not because of hypovolemia.
