NEPHROTIC SYNDROME

Ms. Harpreet Kaur SNI, KURALI

DEFINITION

The nephrotic syndrome (nephrosis) is a clinically defined state characterized by proteinuria, hypoalbu-minemia, hyperlipidemia, and edema. It is sometimes accompanied by hematuria, hypertension, and reduced glomerular filtration rate (GFR).

PATHOPHYSIOLOGY

PATHOPHYSIOLOGY

The glomeruli clean the blood and make urine. Normally, the glomeruli prevent the passage of protein from blood into urine, and in adults less than 150mg of protein is lost each day. But in nephrotic syndrome, the glomeruli become leaky and more than 3.5g - that's 25 times the normal amount - of protein is passed out every 24 hours (the equivalent in children is 0.05g/kg body weight/24 hours). This loss of serum proteins produces a decrease in intravascular oncotic pressure and a resulting shift of fluid from the intravascular compartments to the interstitial space. The result is edema

PATHOPHYSIOLOGY CONTD..

Loss of protein leads to: Low levels of protein in the blood. Salt and water accumulating, leading to tissue swelling. The second factor involves excessive renal tubular resorption of salt and water as a response to the depletion of plasma volume. This contraction of intravascular volume stimulates the production of aldosterone, which increases salt and water reabsorption in the renal tubules.

INCIDENCE

Nephrotic syndrome can present at any age, but the onset is usually between 2 and 7 years, with a male-to-female ratio of 2 to 1. In adolescents and adults, this ratio comes closer to even. Incidence peaks between 2 and 3 years.

CAUSES

Primary renal causes Minimal change nephropathy Immune complex glomerulonephritis Membranoproliferative glomerulonephritis Acute poststreptococcal glomerulonephritis Membranous nephropathy Congenital nephrosis

CAUSES

CONTD

Systemic Causes Infections Toxins Allergies: oak, bee sting, inhaled pollens, food allergy Cardiovascular: sickle cell disease, renal vein thrombosis, passive congestive heart failure Malignancies: Hodgkin disease, leukemia, carcinoma Other: multiple myeloma, systemic lupus erythematosus.

Types

Congenital Rare, Serious & Fatal problem associated with other anomalies of kidney, Inherited autosomal recessive disease, Renal insufficiency and urinary infections Idiopathic Most common (90%), respond to immunosuppressive therapy Secondary 10% cases occurs as secondary disease to HIV/AIDS, DM, Drug toxicity, malaria etc

CLINICAL MANIFESTATIONS

puffiness of the face around the eyes (called periorbital oedema, which decreases during the day but then appears in the abdomen and lower extremities. massive edema (anasarca), with resulting respiratory distress and diarrhea due to edema of the intestinal mucosa. marked edema of the labia or scrotum child is pale and irritable and has lost appetite but gained weight history of recent infections and a decrease in urinary output.

Symptoms of Nephrotic syndrome

Hypoalbuminemia (low levels of the protein albumin in the blood - this causes fluid to move from the blood into the tissues). Urine that looks 'frothy'. Tiredness. Increased susceptibility to infections. Increased risk of thrombosis (formation of blood clots).

DIAGNOSIS

Complete history. The child with nephrosis should have a urinalysis, complete blood count, and evaluation of serum electrolytes, calcium, phosphorus, blood urea nitrogen, creatinine, total protein, albumin, globulin, cholesterol, triglycerides, and complement (C3). Proteinuria is reflected by urinary excretion of protein that frequently reaches levels in excess of 2 gm/m2/24 hours. The urine specific gravity may be high and proportionate to the amount of protein concentration.

DIAGNOSIS

CONTD..

The serum cholesterol may be elevated above 220 mg/dl. Serum sodium is generally low, approximately 130 to 135 mEq/L, because of its retention in the interstitial compartment. The hemoglobin and hematocrit are usually normal but may be elevated during hemoconcentration or reduced during hemodilution. A renal biopsy may be indicated if the child does not respond to initial steroid therapy.

MEDICAL MANAGEMENT

The goal of medical management is reduction of protein excretion. Prednisone is the drug of choice. Initially, a daily dose of 2 mg/kg (maximum 80 mg) or 60 mg/m2 is administered in divided doses throughout the 24 hours. When children have a relapse, which is defined as the reappearance of proteinuria for 3 consecutive days, alkylating agents such as cyclophosphamide have been effective in reducing the relapse rate and inducing long-term remission

DIETARY MANAGEMENT

Children should have a well-balanced, ageappropriate diet rich in protein. Sodium restriction may be indicated when there is marked edema, but otherwise the salt intake should be adjusted to the child's taste. Water restriction may be indicated if decreasing sodium does not control marked edema. Diuretics and salt-poor albumin may be indicated in severe edema.

NURSING MANAGEMENT

The goals of nursing management include (1) providing care during hospitalization (2) administering medications (3) maintaining proper fluid balance and assessing edema (4) providing a nutritious diet (5) preventing infection (6) preventing skin breakdown (7) promoting optimal psychosocial growth (8) providing emotional support and education for all family members.

NURSING DIAGNOSIS

Alteration in nutrition: Less than body requirements related to anorexia Disturbance in self-concept: body image related to physical changes secondary to edema Potential interference in skin integrity related to generalized edema and immobility

CONTD

Alteration in family process related to chronicity of child's illness Anxiety related to separation from family Altered growth and development related to effects of steroid therapy Potential for infection related to effects of steroid therapy Potential impaired physical mobility related to decreased strength Potential self-care deficit related to decreased endurance

Nursing Management

Bed rest & high protein diet Steroid Therapy with oral prednisolone in 2-3 divided doses for 4-6 weeks and then tapered off along with Antacid Antibiotic therapy for any additional infection Diuretics for severe edema, albumin infusion(1gm/kg/day), immunosupressive drugs Renal Transplant in end stages Urine testing for albumin Maintaining intake output and body weight

Nursing Management

Examining child fir any sign/symptom of infections Preventing infection as child is on immunosupressive drugs Allowing parental involvement Discussing about treatment options, prognosis rate and chances of reocurrence