Nephrotic Syndrome Disease

BASICS

DESCRIPTION

A syndrome comprising glomerular proteinuria (3.5 g per 1.73m2 body-surface area/day), hypoalbuminemia, lipiduria, hypercholesterolemia and edema as a result of a primary renal disease or secondary to another disease process.

System(s) affected: Renal/Urologic, Endocrine/Metabolic
Genetics: N/A
Incidence/Prevalence in USA:
- Children - 2:100,000 new cases/year
- Adults - 3:100,000 new cases/year
Predominant age:
- Children - 1.5-6 years (MCD)
- Adults - all ages (FGS, MGN more common USA; IgG-IgA worldwide)
Predominant sex: Male = Female

SIGNS AND SYMPTOMS

Fluid retention: abdominal distention, ascites, edema, puffy eyelids, scrotal swelling, weight gain, shortness of breath
Anorexia
Hypertension
Oliguria
Orthostatic hypotension
Retinal sheen
Skin striae

CAUSES

Primary renal disease
- Fibrillary glomerulopathy (primary)
- Focal glomerulonephritis
- Focal glomerulosclerosis (FGS)
- IgA nephropathy
- Membranoproliferative glomerulonephritis (MPGN)
- Membranous glomerulonephritis (MGN)
- Mesangial proliferative glomerulonephritis
- Minimal change disease (MCD)
- Rapidly progressive glomerulonephritis (RPGN)
- Congenital nephrotic syndrome
Secondary renal disease. Associated primary renal disease shown in brackets:
- Allergens (snake venoms, antitoxins, poison ivy, insect stings)
- Carcinoma (bronchogenic, breast, colon, stomach, kidney) [MGN, etc]
- Diabetes mellitus (most common)
- Erythema multiforme
- Fibrillary glomerulopathy (secondary: amyloid, cryoglobulins, multiple myeloma, chronic lymphocytic leukemia [CLL])
- Henoch-Schönlein purpura
- Heredofamilial (Alport's syndrome, Fabry's disease)
- HIV infection
- Hodgkin's lymphoma [MCD]
- Infections : Ventriculoatrial shunt infection, Bacterial endocarditis [MPGN], Viral (hepatitis B [MPGN, mesangial, MGN]) Other viral (hepatitis C), protozoal and helminthic
- Leukemias
- Lymphomas [MGN]
- Non-Hodgkin's lymphoma
- Focal glomerulosclerosis (Reflux nephropathy, Heroin abuse, Nephron ablation, Extensive glomerular scarring in acute glomerulonephritis, Chronic renal allograft rejection, End-stage kidney, Morbid obesity, A thromboembolism)
- Malignant hypertension
- Melanoma
- Nephrotoxins and drugs (gold, penicillamine, mercury [MGN])
- Nonsteroidal anti-inflammatory drug induced nephrotic syndrome [MCD] and interstitial nephritis
- Polyarteritis nodosa
- Post-streptococcal glomerulonephritis [PSGN] - 20% are nephrotic
- Sarcoid
- Serum sickness
- Sjögren's syndrome
- Systemic lupus erythematosus (SLE) [MGN, FGS, focal, mesangial, diffuse, proliferative]
- Toxemia of pregnancy

RISK FACTORS

Drug addiction (e.g., heroin [FGS])
Hepatitis B and C, HIV, other infections
Immunosuppression
Nephrotoxic drugs
Vesicoureteral reflux [FGS]
Cancer (usually MGN, may be nil disease [MCD])
Chronic analgesic abuse

DIAGNOSIS

LABORATORY

Hypoalbuminemia
Hyperlipidemia
Lipiduria
Low complement in some diseases
Azotemia
Hypercholesterolemia
Increased serum beta-globulin
Increased serum IgG
Urine
- Proteinuria (> 3 gm/24 hr)
- Glycosuria
- Hematuria
- Aminoaciduria
- RBC casts
- Granular casts
- Proteinuria
- Hyaline casts
- Fatty casts
- Foamy appearance

Drugs that may alter lab results: See description
Disorders that may alter lab results: Many

PATHOLOGICAL FINDINGS

Light microscopy
- May see nothing (e.g., MCD)
- Disease specific: sclerosis (e.g., FGS in diabetes)
Immunofluorescence : Mesangial IgA (Schönlein-Henoch, IgG-IgA nephropathy). Other patterns specific for disease
Electron microscopy (Specific for disease, e.g., sub-epithelial deposits of IgG in MGN

SPECIAL TESTS

Complement levels
Antinuclear antibody (ANA)
Serum protein electrophoresis / quantitative immunoglobulins
Urine immune electrophoresis
Blood cultures
Renal venogram for thrombosis

IMAGING

X-ray
Ultrasound
CT
MRI or venography for renal vein thrombosis

DIAGNOSTIC PROCEDURES

History, physical, basic laboratory including electrolytes, renal biopsy with light, immunofluorescence, electron microscopy for definitive diagnosis

TREATMENT

APPROPRIATE HEALTH CARE

Outpatient

GENERAL MEASURES

Treat infections vigorously (especially bacteriuria, endocarditis, peritonitis)
Anticoagulant therapy (heparin and warfarin) if thromboses occur
Vaccinations: pneumococcal, influenza, and Haemophilus influenzae
Avoid excess sunlight
Avoid nephrotoxic drugs
Judicious use of diuretics
Severe anemia may be treated with erythropoietin

SURGICAL MEASURES

N/A

ACTIVITY

Bedrest as tolerated

DIET

Normal protein intake: 1 g/kg/day
Low fat diet (to control cholesterol)
Reduced sodium intake
Liberal potassium intake (unless hyperkalemic)
Supplemental multivitamins and minerals, especially vitamin D and iron
Fluid restriction if hyponatremic
Caloric restriction if obese or diabetic
For hypercholesterolemia: low-fat soy-protein diet, 7 g protein/kg/day

PATIENT EDUCATION

Printed material for patients: National Kidney Foundation, 30 E. 33rd Street, Suite 1100, NY, NY 10016, (800)622-9010
- "Childhood Nephrotic Syndrome" (Order #02-23NN)
- "Diabetes and Kidney Disease" (Order #02-09CP) and "Focal Glomerulosclerosis" (Order #02-28NN)

FOLLOW UP

PREVENTION/AVOIDANCE

Avoid causative factors whenever possible
Detect and treat infections vigorously. Infections may involve the common (Pneumococcus) to the unusual (Strongyloides), especially with immunosuppression.

POSSIBLE COMPLICATIONS

Low levels of: 25-hydroxycholecalciferol, serum calcium, adrenocortical hormones, thyroid hormones
Hypercoagulability, thrombosis
Pulmonary emboli
Hyperlipidemia/accelerated cardiovascular disease
Acute renal failure
Progressive renal failure
Renal vein thrombosis
Protein malnutrition
Infection
Pleural effusion
Ascites
Iron deficiency (uncommon)

EXPECTED COURSE AND PROGNOSIS

Varies with specific causes. Complete remission expected if basic disease is treatable (infection, malignancy, drug-induced). Otherwise may progress to dialysis dependence (e.g., diabetic glomerulosclerosis).

MISCELLANEOUS

ASSOCIATED CONDITIONS

Cancer
Diabetes mellitus
Connective tissue disease (e.g., SLE)
Multiple myeloma

AGE-RELATED FACTORS

Pediatric: Relatively common in children aged 1.5-4 years (MCD)
Geriatric: Occurs in this age group. Prognosis is worse.
Others: N/A

PREGNANCY

Toxemia of pregnancy may be nephrotic

OTHER NOTES

N/A

ABBREVIATIONS

FGS = focal segmental glomerulosclerosis
MGN = membranous nephropathy
MPGN = chronic hypocomplementemic glomerulonephritis, chronic mesangiocapillary glomerulonephritis
MCD = lipid nephrosis, four process disease, nil disease
RPGN = crescentic glomerulonephritis

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.