NEPHROTIC SYNDROME

- A PRESENTATION BY NAYEEM AHMED

What is Nephrotic Syndrome ?

NEPHROTIC SYNDROME IS A NONSPECIFIC KIDNEY DISORDER CHARACTERISED BY A NUMBER OF SIGNS OF DISEASE: PROTEINURIA, HYPOALBUMINEMIA AND EDEMA.

NEPHROTIC SYNDROME (NS) RESULTS FROM INCREASED PERMEABILITY OF GLOMERULAR BASEMENT MEMBRANE (GBM) TO PLASMA PROTEIN.

Nephrotic Syndrome (Nephrosis)

The Main Trigger Of primary Nephrotic Syndrome and Fundamental and highly

important change of pathophysiology :-

Proteinuria

In Short

It is clinical and laboratory syndrome characterized by massive proteinuria, which lead to hypoproteinemia (hypo-albuminemia), hyperlipidemia and pitting edema.

NS in a nutshell• Proteinuria

• Protenima (Hypoalbuminemia)

• Hyperlipidemia

• Generalized edema (Anasarca)

• Oliguria

• Hypertension

Causes Amyloidosis :     A group of diseases resulting from abnormal deposition of certain proteins (amyloids) in various bodily areas.

Membranous Glomerulopathy:     Is a kidney disorder that leads to changes and inflammation of the structures inside the kidney that help filter wastes and fluids.

Diabetes Foot Process Disease:In minimal change disease, there is damage to the glomeruli 

ClassificationPrimary Idiopathic NS (INS): majority The cause is still unclear up to now. Recent 10 years

evidence has suggested that INS may result from a primary disorder of T– cell function.

Accounting for 90% of NS in child.

Secondary NS: Congenital NS: rare 1st 3 month of life, only treatment renal transplantation.

Secondary NS Drug Toxicity Allergy Infection: HBV, HIV, leprosy, syphilis, Schistosomiasis. Autoimmune or collagen-vascular diseases Metabolic disease: Diabetes mellitus.

Neoplasma: Hodgkin’s disease, carcinoma. Genetic Disease: Sickle cell disease, Amyloidosis, Congenital nephropathy.

Idiopathic NS (INS)• Minimal Change Nephropathy (MCN): <80%

• Non—Minimal Change Nephropathy：＜ 20%

Some FactsNephrotic syndrome is 15 times more common in children than in Nephrotic syndrome is 15 times more common in children than in adults. adults.

Most cases of primary nephrotic syndrome are in children and are Most cases of primary nephrotic syndrome are in children and are due to minimal-changedue to minimal-change disease. disease.

Nephrotic criteria*Massive proteinuria : qualitative proteinuria: 3+ or 4+, quantitative proteinuria : more than 3.5gms/day in children (selective).

*Hypo-proteinemia : total plasma proteins < 5.5g/dl and serum albumin : < 2.5g/dl.

*Hyperlipidemia : serum cholesterol : > 5.7mmol/L

*Edema : pitting edema in different degree

Pathophysiology

Glomerular Damage Proteinuria Hypoproteinemia Edema Hyperlipidemia

Proteinuria

Reduced Albumin

Reduced oncotic pressure

Edema

Reduced blood volume

Activation of RAAS

Edema

Sequelae. because of NS

Hyperlipidemia; Pathogenesis of:Response to Hypoproteinemia → reflex to liver --→ synthesis of generalize protein ( including lipoprotein ) and lipid in the liver ,the lipoprotein high molecular weight no loss in urine → hyperlipidemia

*Diminished catabolism of lipoprotein

Clinical Manifestations1.Main manifestations: Edema (varying degrees) is the common symptom

Local edema: edema in face , around eyes( Periorbital swelling) , in lower extremities. Generalized edema (anasarca), edema in penis and scrotum.

2.Non-specific symptoms:Fatigue and lethargyloss of appetite nausea and vomiting abdominal pain diarrheabody weight increase urine output decrease pleural effusion (respiratory distress)

Examples of Clinical MenifestationsPeriorbital swellingAnasarca

Management of Nephrotic Syndrome    

 General (non specific)

 *Corticosteroid therapy 

General (non-specific)HospitalizationDiet : Hypertension and edema: Low salt diet. Severe edema: Restricting fluid intake Avoiding infectionDiuresisVaccination

Corticosteroid- Prednisone therapy:

Prednisone is a corticosteroid. It reduces swelling. It is used for many conditions among them: Allergic reactions Skin diseases Breathing problems Certain Cancers Blood disorders Eye problems Hormone replacement

Thank you for you Patience and concentration