NEPHROTIC SYNDROME

Ndayambaje Israel

Introduction Nephrotic syndrome isn't a disease but is a condition Cxtxd by marked proteinuria, hypoalbuminemia, hyperlipidemia, lipiduria, increased coagulation, & edema. It results from a glomerular defect that affects the vessels' permeability & indicates renal damage. Some forms of nephrotic syndrome may eventually progress to end-stage renal failure.

Anatomy

Nephrotic SyndromeChronic renal disorder in which the basement membrane surfaces of the glomeruli are affected, cause loss of protein in the urine.

Nephrotic criteria Massive proteinuria:Qualitative proteinuria: 3+ or 4+, Quantitative proteinuria : more than 40 mg/m2/hr in children (selective). 2. Hypo-proteinemia : total plasma proteins < 5.5g/dl & serum albumin : < 2.5g/dl.3. Hyperlipidemia: Serum cholesterol : > 5.7mmol/L 4. Edema: pitting edema in different degree

Causes Glomerulonephritis Metabolic diseases: diabetes mellitusCollagen-vascular disorders: systemic lupus erythematosus,periarteritis nodosa.Circulatory diseases: heart failure, sickle cell anemia, and renal vein thrombosis.Nephrotoxins: mercury, gold, and bismuth.Infections: tuberculosis, enteritis; allergic reactions; pregnancy; hereditary nephritisNeoplastic: multiple myeloma

Types Idiopathic nephritic syndrome The most common in children for about 90%Congenital Nephrotic SyndromeRare in children & congenital It is inherited by an autosomal recessive gene, meaning that males & females are equally affected

Pathophysiology I. Proteinuria :Increase glomerular permeability for proteins due to loss of negative charged glycoproteinDegree of protineuria:-Mild: less than 0.5g/m2/dayModerate: 0.5 2g/m2/daySevere: more than 2g/m2/dayType of proteinuria:-A-Selective proteinuria: where proteins of low molecular weight, such as albumin, are excreted more readily than protein of HMWB-Non selective : LMW+HMW are lost in urine

Pathophysiology /2II. Hypoalbinaemia Due to hyperproteinuria----- Loss of plasma protein in urine mainly the albumin.Increased catabolism of protein during acute phase.

Pathophysiology /3III. Edema Reduction plasma colloid osmotic pressure secondary to hypoalbuminemia Edema & hypovolemia*Intravascular volume antidiuretic hormone (ADH ) & aldosterone(ALD) water & sodium retention Edema *Intravascular volume glomerular filtration rate(GFR) water & sodium retention Edema

Signs & symptoms 1.Main manifestations: Edema (varying degrees) is the common symptomLocal edema: edema in face , around eyes( Periorbital swelling) , in lower extremities. Generalized edema (anasarca), edema in penis and scrotum.

The symptoms of nephrotic syndromeFatigue & malaise. Decreased appetiteWeight gain from excess fluid Dull hair Foamy urine, decrease in frequency of urinationPale fingernail beds. Ears cartilage may feel less firmFood intolerances or allergiesProteinuria, high levels Low levels of proteinemia due to its loss in the urineHigh cholesterol levels in the blood

Massive proteinuria Hypoalbuminemia (K+ normal & BP normal)Edema usually starts in periorbital area & dependent areas of the body and progresses to generalized, massive edema. Pitting edema of 4+. Caused by hypo albumin which causes shift of fluids to extracellular space. There is an insidious weight gain- shoes don't fit, etc..Hyperlipidemia

* NB: there is no hematuria or hypertension! Four most common characteristics:

Diagnosis Urine tests (to check for protein) >40mg/m2/hr Blood tests for levels of cholesterol & albuminRenal ultrasound.Renal biopsy

Blood specimenSerum protein: decrease >5.5gm/dL , Albumin levels are low (2.5gm/dL). Serum cholesterol & triglycerides: Cholesterol 5.7mmol/L (220mg/dl).

ESR100mm/hr during activity phase Serum complement: Vary with clinical type. Renal function

Medications CorticosteroidsImmunosuppressive drug therapyDiuretics (to reduce the edema)Restriction to fluids intake IV albuminSpecial diet that restricts salt intake

Nrsg care Assess and Document the location and character of the patient's edema.Vital signs: BP, PulseMonitor & record intake and output & weigh the patient accurately each morning after s/he voids & before S/he eats. Make sure S/he's wearing the same amount of clothing each time you weigh him/her.Careful monitoring of IV fluids Plan a low-sodium diet with moderate amounts of protein.Frequently check urine for protein.

Nrsg care 7. Monitor plasma albumin & transferrin concentrations to evaluate overall nutritional status.8. Provide meticulous skin care 9. Use a reduced-pressure mattress or padding to help prevent pressure ulcers.10. To prevent the occurrence of thrombophlebitis, encourage activity & exercise11. Psychological care - Give the patient & family reassurance & support12. Provide appropriate provisions corticosteroids13. Give diuretic if prescribed

Complications Infections: Infections is a major complication in children with NS. It frequently trigger relapses. Common infections: URI, peritonitis, cellulitis &UTIHypercoagulability (Thrombosis)Cardiovascular disease :-Hyperlipidemia, may be a risk factor for cardiovascular disease.Hypovolemic shockOthers: growth retardation, malnutrition, adrenal cortical insufficiency