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Nephrotic syndrome

Definition

It is a kidney disease with proteinuria (proteins in the urine), hypoalbuminemia (blood contains less albumin, and edema (body swelling).

Criteria: range of proteinuria is 3 grams per day or more. On a single, "spot" urine collection, it is 2 grams of protein per gram of urine creatinine.

Causes:

Kidney diseases: minimal-change nephropathy
Focal glomerulosclerosis
Membranous nephropathy.
Systemic diseases: diabetes, amyloidosis, and lupus erythematosus.
Nephrotic syndrome may affect adults and children, of both sexes and of any race. It may occur in typical form, or in association with nephritic syndrome. The latter has glomerular inflammation, with hematuria and impaired kidney function.

Classification

1. Primary: disease specific to the kidneys
2. Secondary: renal manifestation of a systemic general illness.

In all cases, injury to glomeruli is an essential feature.

Primary causes of nephrotic syndrome:

• Minimal-change nephropathy
• Focal glomerulosclerosis
• Membranous nephropathy
• Hereditary nephropathies

Secondary causes:
• Diabetes mellitus
• Lupus erythematosus
• Amyloidosis and paraproteinemias
• Viral infections (eg, hepatitis B, hepatitis C, human immunodeficiency virus [HIV] )
• Preeclampsia

Nephrotic-range proteinuria may occur in other kidney diseases like IgA nephropathy.

Nephrotic syndrome may occur in persons with sickle cell disease and evolve to renal failure.

Membranous nephropathy may complicate bone marrow transplantation

Kidney diseases that affect tubules and interstitium, such as interstitial nephritis, will not cause nephrotic syndrome.

Nephrotic syndrome in infancy and childhood
almost 20 cases per million children under age 15 years.
congenital nephrotic syndrome may occur in 1 in 10,000 or 1 in 500 births, respectively.
84.5% of all children with primary nephrotic syndrome have minimal-change nephrotic syndrome (MCNS)
9.5% have focal segmental glomerulosclerosis (FSGS)
2.5% have mesangial proliferation
3.5% have membranous nephropathy or another cause of the disease.
MCNS remains the most important cause of chronic renal disease in children.
From a therapeutic perspective classification is :
• Steroid sensitive
• Steroid resistant
• Steroid dependent or frequently relapsing.
Frequency
Diabetic nephropathy with nephrotic syndrome is most common
The rate of end-stage renal disease from diabetes has reached 100 cases per million

Mortality/Morbidity

• The prognosis may worsen because of (1) an increased incidence of renal failure and the complications secondary to nephrotic syndrome, including thrombotic episodes and infection, or (2) treatment-related conditions, such as infectious complications of immunosuppressive treatments.
• In secondary nephrotic syndromes, morbidity and mortality are related to the primary disease process, such as diabetes or lupus, although in diabetic nephropathy, the magnitude of proteinuria itself relates directly to mortality.

Sex

• There is a male predominance
• Lupus nephritis affects mostly women.

Age

Affects as per the types of nephrotic syndrome
1. Congenital right from childhood
2. Minimal change, focal glomerulosclerosis till 10 yrs
3. Minimal change diabetic type 1 nephropathy type between 20 -40 yrs
4. Amyaladotic membranous nephropathy from 40 – 60 yrs

Symptoms of Nephrotic syndrome

The following are some of the symptoms of Nephrotic syndrome:

• The first sign of nephrotic syndrome in children is usually swelling of the face; this is followed by swelling of the entire body.
• Adults can present with dependent edema.
• Foamy urine may be a presenting feature.
• A thrombotic complication, such as deep vein thrombosis of the calf veins or even a pulmonary embolus, may be the first clue indicating nephrotic syndrome.
• Additional historical features that appear can be related to the cause of nephrotic syndrome. Thus, the recent start of a nonsteroidal anti-inflammatory drug (NSAID) or a 10-year history of diabetes may be very relevant.

Physical
• Edema is the predominant feature of nephrotic syndrome and initially develops around the eyes and legs.
• Increase in weight
• The development of ascites, or pleural effusions.
• Hematuria and hypertension manifest in a minority of patients.
• Diabetic retinopathy, hypertension and/or anemia.

Pathophysiology

Glomerular permeability
In a healthy individual, less than 0.1% of plasma albumin may traverse the glomerular filtration barrier.
The glomerular structural changes that may cause proteinuria are (1) damage to the endothelial surface, (2) damage to the glomerular basement membrane, and/or (3) damage of the podocytes. One or more of these mechanisms may be seen in any one type of nephrotic syndrome. Albuminuria alone may occur, or, with greater injury, leakage of all plasma proteins, (ie, proteinuria) may take place.

Proteinuria that is more than 85% albumin is selective proteinuria.

Pathogenesis of edema

An increase in glomerular permeability leads to albuminuria and eventually to hypoalbuminemia. In turn, hypoalbuminemia lowers the plasma colloid osmotic pressure, causing greater transcapillary filtration of water throughout the body and thus the development of edema.

Metabolic consequences of proteinuria
A) Levels of serum lipids are usually elevated.
This can occur via (1) hypoproteinemia that stimulates -lipoprotein, synthesis by the liver (2) Diminution of lipid catabolism caused by reduced plasma levels of lipoprotein lipase.
B) The loss of antithrombin III and plasminogen via urine
C) Increase in clotting factors, especially factors I, VII, VIII, and X, increases the risk for D) Venous thrombosis and pulmonary embolism.
In the first 6 months that a patient has nephrotic syndrome, the occurrence rate of venous thrombosis may reach 10%.
E) Vitamin D–binding protein may be lost in the urine, leading to hypovitaminosis D, with malabsorption of calcium and development of bone disease.
F) Urinary immunoglobulin losses may lower the patient's resistance to infections and increase the risk of sepsis and peritonitis.

Homeopathic treatment for Nephrotic syndrome

Treatment options vary as per the underlying cause and type of nephrotic syndrome

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