RENAL SYSTEMGLOMERULAR DISEASE

GLOMERULAR DISEASES

• Nephrotic syndrome-Idiopathic-Secondary-Congenital

• Hemolytic uremic syndrome• Alport syndrome• IgA nephropathy• Glomerular Nephritis

NEPHROTIC SYNDROMEIt is a manifestation of glomerular disease, characterized by:

- Nephrotic range proteinuria (Nephrotic range proteinuria - protein excretion of > 40 mg/ m2 /hr or a first morning protein: creatinine ratio of >2-3 : 1)

- the triad of clinical findings associated with massive proteinuriao hypoalbuminemiao edemao hyperlipidemia.

INCIDENCE:

The annual incidence is 2-3 cases per 100,000 children per year in most Western countries and higher in underdeveloped countries resulting predominantly from malaria. Minimal change nephrotic syndrome constitutes 80% of nephrotic syndrome cases.

ETIOLOGY• Idiopathic nephrotic syndrome

⁻ focal segmental glomerulosclerosis⁻ membranoproliferative glomerulonephritis⁻ membranous nephropathy ⁻ diffuse mesangial proliferation

• Associate with glomerular damage - Systemic lupus erythematosus- Lymphoma- Leukemia & infections

• Hereditary proteinuria syndromes - Alport syndrome- Sickle cell anemia.

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TYPES

Idiopathic

Secondary

Congenital

Nephrotic syndrome

1.Idiopathic Nephrotic syndrome

• It is the primary disease which also known as childhood nephrosis, or minimal change nephrotic syndrome.

• Approximately 90% of children with nephrotic syndrome have idiopathic nephrotic syndrome.

• Idiopathic nephrotic syndrome is associated with primary glomerular disease without evidence of a specific systemic cause.

2.Secondary Nephrotic Syndrome• It is a secondary disorder that occurs as a

clinical manifestation after or in association with glomerular damage. It occurs secondary to systemic diseases and infections.

• Nephrotic syndrome has also developed during therapy with numerous drugs and chemicals.

3.Congenital Nephrotic Syndrome

It is inherited as autosomal recessive disorder. It is defined as nephrotic syndrome manifesting at birth or within the first 3 months of life. Congenital nephrotic syndrome may be classified as:

1. Primary2. Secondary

• Primary congenital nephrotic syndrome is due to a variety of syndromes inherited as autosomal recessive disorders.- present at birth with edema due to massive proteinuria- delivered with an enlarged placenta (>25% of the

infant’s weight).- Severe hypoalbuminemia, hyperlipidemia, and

hypogammaglobulinemia result from loss of filtering selectivity at the glomerular filtration barrier.

• Secondary congenital nephrotic syndrome can be

occurred from underlying causes such as syphilis.

PATHOPHYSIOLOGY

CLINICAL MANIFESTATIONS• Weight gain• Puffiness on face (facial

edema)o Especially around the eyeso Apparent on arising the

morningo Subsides during the day

• Abdominal swelling(ascitis)• Pleural effusion• Labial or scrotal swelling• Edema of intestinal mucosal

which result in:o Diarrhea

o Anorexiao Poor intestinal absorption

• Ankle / leg swelling• Irritability• Easily fatigued• Lethargic• BP normal or slightly

increased• Susceptible to infections• Urine alterations

o Decreased volumeo frothy

DIAGNOSTIC EVALUATION• History collection

weight gain, anorexia, irritability, less active• Physical examination

Clinical manifestations• Urine dipstick test for protienuria• Blood tests

₋ Serum protein low concentration₋ Presence of casts, RBC₋ Reduced albumin₋ GFR normal or high₋ Hb normal or elevated₋ Elevated platelet count

• Renal biopsy if not respond to steroid treatment

THERAPEUTIC MANAGEMENT

GOALS• Reduce excrtion of urinary protein• Reduce fluid retension• Preventing infection• Minimize complications related to treatment DIETARY MANAGEMENT• Low salt diet• Fluid restriction

PHARMACOLOGICAL MANAGEMENT

• Diuretic therapy for temporary relief from edema• Infections are treated with

appropriate antibiotics• Corticosteroids for MCNS• Prednisolone – 2mg/kg body weight/day in one

or two divide doses• Relapse is treated with high dose steroid therapy• For children who do not respond to steroid

therapy, immune - suppressants are given.

STEROID THERAPY

• Extended APN schedule• 60mg/m2/day as a single dose for 6 weeks• If remission is present , then 40 mg/m2/every

other day for next 6 weeks• If remission is maintained, taper steroids in

EOD in 2 weeks

Definition of response• Remission

No albumin on three consecutive early morning urine samples

• Relapse2+ or more albumin on 3 consecutive early morning urine samples

• Frequent relapse3 or more relapses in 6 months or 4 or more relapses in 1 year

• Steroid resistanceNo remission after 8 weeks of adequate daily steroids

• Steroid dependenceRelapse within 15 days of stopping steroids after inducing remission or on tapering, on more than 2 occasions

NURSING MANAGEMENTa. Focus Assessment

• Urinary System (oliguric, urine retention, proteinuria and urine discoloration).

• Fluid and electrolyte balance (excess fluid, edema, ascitis, weight gain, dehydration)

• Circulation (increased blood pressure)• Neurology (decreased level of consciousness due to

dehydration)• Breathing (shortness of breath, tachypnea)• Mobility (redness, malaise)

b. Nursing Diagnosis

• Impaired Urinary Elimination related to Na and water retention.

• Excess Fluid Volume related to edema• Imbalanced Nutrition Less Than Body

Requirements related to damage protein metabolism

• Ineffective Breathing Pattern related to suppression of the diaphragm due to ascites

c. Nursing interventions

• Administer medications• Stress the importance of adhering to the special

diet• meticulous skin care• Encourage activity and exercise• Frequently check of the patient’s urine• Monitor and document about edema.• Measure blood pressure• Monitor intake and output hourly.• Assess the patient’s response to prescribed

medications.

COMPLICATIONS1.Due to drugs

Toxicity of drugs may occur– Furosemide,

siranolactone– Steroids– Cyclophosphamide– Levamisole– Anticoagulants

2.Due to the disease– Edema

– Biochemical hypothyroidism

– Hypocalcemic tetany– Anemia– Hypercoagulable states– Acute renal failure– Infection– Thromboembolic events– Cardiovascular disease– Steroid therapy

IMMUNISATIONS• The children with NS should receive:• 23- serotype pneumococcal vaccine• 7-valent conjugate pneumococcal vaccine• routine childhood immunization schedule( for child is in

remission and off daily prednisone therapy)• Live virus vaccines should not be administered to children

who are receiving daily or alternate-day high-dose steroids• Vaccines can be administered after corticosteroid therapy has

been discontinued to nephrotic children in relapse• if exposed to varicella, should receive varicella-zoster

immunoglobulin• Influenza vaccine should be given on a yearly basis

PROGNOSIS

Ultimate recovery in most cases is good. It is a self timing disease. In children who receives steroid therapy the tendency to relapse decreases with time. With early detection and treatment, the membrane damage could be minimized. About 80% of affected children have favorable prognosis.

HEMOLYTIC-UREMIC SYNDROME (HUS)

It is one of the most common causes of community-acquired acute kidney failure in young children. It is characterized by the triad of:– micro angiopathic hemolytic anemia– thrombocytopenia– renal insufficiency

CLINICAL DESCRIPTIONHUS is characterized by the acute onset of

microangiopathic hemolytic anemia, renal injury, and a low platelet count. Most cases of HUS occur after an acute gastrointestinal illness (usually diarrheal).

INCIDENCEOccurs in infants and small children between the ages of 6 months and 5 years.

CASE CLASSIFICATION• Probable

An acute illness diagnosed as HUS that(a) has onset within 3 wk after onset of an acute or bloody diarrhea(b) meets the laboratory criteria except that microangiopathic changes are not confirmed.

• ConfirmedAn acute illness diagnosed as HUS that both meets the laboratory criteria and began within 3 wk after onset of an episode of acute or bloody diarrhea

CLASSIFICATION OF HEMOLYTIC UREMIC SYNDROME

1.Infection Induced• Verotoxin-producing

Escherichia coli• Human immunodeficiency

virus2.Genetic• von Willebrand factor-

cleaving protease deficiency• Complement factor H

deficiency or mutation3.Other Diseases Associated

With Microvascular Injury

• Systemic lupus erythematosus

• Primary glomerulopathy• HELLP (hemolytic anemia,

elevated liver enzymes, low platelet count) syndrome

4.Medication-Induced• Calcineurin inhibitors• Cytotoxic, chemotherapy

agents• Quinine

ETIOLOGY

• Rickettsia• Bacterial toxins (e-coli, salmonella, pneumococci)• Chemicals• Viruses (coxsackie virus, echovirus, adenovirus)• Usually transmitted by undercooked meat or

unpasteurized milk or apple cider• HUS outbreaks have also been associated with

municipal water supply; petting farms; swimming in contaminated ponds and consuming cheese, lettuce, or raw spinach contaminated with toxin

PATHOPHYSIOLOGY

Primary injury in endothelial lining of small glomerular arterioles

Deposits of platelets and fibrin clots

Swelling in the glomerular arterioles

RBC damage resulted by the attempt to move through occluded blood vessels

Spleen removes the damage

Results in hemolytic anemia

Result in characteristic thrombocytopenia

CLINICAL MANIFESTATIONS

• History of a prodromal disease (gastroenteritis or an upper respiratory infection)

• Acquired hemolytic anemia• Sudden onset of hemolysis• Thrombocytopenia• Renal injury• Central nervous system symptoms

DIAGNOSTIC EVALUATION

• History collectuion• Clinical examination:– Triad of anemia, thrombocytopenia and renal failure

• Laboratory examination:o Urine - proteinuria, hematuria, urinary cast

presenceo Blood - Elevated blood urea, nitrogen, creatinine - Low hemobglobin, hematocrit - High reticulocyte count

THERAPEUTIC MANAGEMENT• early recognition of the disease• monitoring for potential complications• meticulous supportive care.

– careful management of fluid and electrolytes– correction of volume deficit– control of hypertension– early institution of dialysis if the patient becomes anuric or

significantly oliguric– Red cell transfusions are usually required because hemolysis can be

brisk and recurrent until the active phase of the disease has resolved.

• Blood transfusion– Fresh, washed packed cells for anemic child with caution to prevent

circulatory overload– Fresh frozen plasma and plasma pherisis

PROGNOSIS

Most recover renal function completely, but of surviving patients, 5% remain dependent on dialysis, and up to 20-30% are left with some level of chronic renal insufficiency. The recovery rate is about 95%, but residual renal impairment ranges from 10% to 50% in various cases.

Immunoglobulin A Nephropathy (Berger Nephropathy)

IgA nephropathy is the most common chronic glomerular disease. The disease derives its name from deposits of Immunoglobulin A (IgA) in a granular pattern in the mesangium a region of the renal glomerulus.

INCIDENCE

It is seen more often in male than in female patients.-is often benign in childhood in comparison to that of adults.-is an uncommon cause of end-stage renal failure during childhood.

CLINICAL MANIFESTATIONS

• Gross hematuria associated with loin pain.• Proteinuria often <1000 mg/24 hr.• Mild to moderate hypertension.• Normal serum levels of C3

DIAGNOSIS

• History collection• Physical examination (clinical features)• Laboratorical investiagations• Renal biopsy

TREATMENT

• The primary treatment is appropriate blood pressure control

• Fish oil, which contains anti-inflammatory omega-3 polyunsaturated fatty acids

• Immunosuppressive therapy with corticosteroids• Angiotensin-converting enzyme inhibitors and

angiotensin II receptor antagonists are effective in reducing proteinuria and retarding the rate of disease progression.

• Kidney transplantation

PROGNOSIS

Most children with IgA nephropathy do not display progressive renal dysfunction until adulthood, prompting the need for careful long-term follow-up. Poor prognostic indicators at presentation or followup include persistent hypertension, diminished renal function, and heavy or prolonged proteinuria.

Alport Syndrome (hereditary nephritis)It is a genetically heterogeneous disease

caused by mutations in the genes coding for type IV collagen, a major component of basement membranes.

These genetic alterations are associated with marked variability in clinical presentation, natural history, and histologic abnormalities.

GENETICS

Approximately 85% of patients have X-linked disease caused by a mutation. Autosomal recessive forms of AS are caused by mutations in the COL4A3 and COL4A4 genes on chromosome 2 encoding the α3 and α4 chains, respectively, of type IV collagen. An autosomal dominant form of AS linked to the COL4A3-COL4A4 gene locus occurs in 5% of cases.

CLINICAL MANIFESTATIONS

• Asymptomatic microscopic Hematuria• Single or recurrent episodes of gross hematuria

commonly occurring 1-2 days after an upper respiratory infection.

• Proteinuria• Bilateral sensorineural hearing loss• Ocular abnormalities• Leiomyomatosis of the esophagus,

tracheobronchial tree, and female genitals in association with platelet abnormalities is rare.

DIAGNOSIS

• Family history• Screening urinalysis of first-degree relatives• Audiogram,• Ophthalmologic examination• Diagnostic renal biopsy• Mutation screening or linkage analysis is not readily

available for routine clinical use.• Prenatal diagnosis is available for families with

members who have X-linked AS and who carry an identified mutation.

TREATMENT

• No specific therapy is available to treat AS• Angiotensin converting enzyme inhibitors can

slow the rate of progression• Careful management of renal failure

complications such as hypertension, anemia, and electrolyte imbalance is critical.

• Patients with ESRD are treated with dialysis and kidney transplantation.

PROGNOSIS

The risk of progressive renal dysfunction leading to end-stage renal disease (ESRD) is highest among hemizygotes and autosomal recessive homozygotes. Risk factors for progression are gross hematuria during childhood, nephrotic syndrome, and prominent GBM thickening.

Acute Glomerulo Nephritis

AGN is an immune mediated inflammatory disease of the capillary loops in the renal glomeruli. AGN may be a primary event or a manifestation of a systemic disorder that can range from minimal to severe.

INCIDENCE-Acute post streptococcal glomerulo nephritis (APGN) is

the most common of post infectious renal disease in childhood.

-It is common in early school age children.-And male female ratio is 2:1.

ETIOLOGY

It is an immune complex disease that occurs after an antecedent streptococcal infection with certain strains of group A β-hemolytic streptococcal infection. Acute post streptococcal glomerulonephritis is the most common. A latent period of 10 to 21 days occurs for the onset of clinical manifestations.

PHASES

• Phase 1 – edema and oliguria present• Phase 2 – edema reduces and

urine output increases

PATHOPHYSIOLOGYStreptococcal infection

Production of antigen antibody complex in glomerular loops

Inflammatory reaction

Proliferation and swelling of endothelial cells

Diminish the amount of glomerular filtrate and allow the

passage of blood cells and protein in the filtrate

Sodium and water retention

Damage of glomerular membrane

Progressive renal failure

CLINICAL MANIFESTATIONS

• Sore throat/pyoderma/scabies/impetigo

• Oliguria• Edema• Periorbital puffiness• Pedal edema• Rapid weight gain• Hypertension• Circulatory congestion• Hematuria

• Proteinuria• Fever • Headache• Nausea and vomiting• Anorexia• Abdominal pain• Malaise• Hypertension

DIAGNOSIS• History collection• Physical examination• Urine examination• Blood examination

-increased level of urea, creatinine, ESR, decreased Hb, hyponatremia, hyperkalemia, reduced C3(serum complement) in early stages.

• Throat swab culture-Streptococci from culture of the pharynx

• Chest X-ray-Cardiac enlargement, pulmonary congestion, pleural effusion.

COMPLICATIONS

• CCF• Acyte renal failure• Hypertensive encephalopathy• Persistent hypertension• Anemia• Growth failure• Chronic glomerulonephritis

MANAGEMENT-Bedrest for weeks till urine got free from RBC-Diet management-Daily weight recording to assess increase and decrease edema- Regular measurement of vital signs, body weight and input and output-administration of antibiotic-symptomatic management-Antihypertensive drugs-Tranquilisers for convulsions and encephalopathy-dialysis may be needed in renal failure and severe electrolyte imbalance-management of complications

NURSING MANAGEMENT• Careful assessment of diseases status• Regular monitoring of vital signs• Maintain input and output• Children with restricted fluid intake and those

who does not have much edema should be observed for signs of dehydration if he lost weight.

• Administer antibiotics• Promote rest, sleep and comfortable position• Skin care for edematous part• Dietary management

PROGNOSIS

Almost all children correctly diagnosed as having APSGN recover completely and specific immunity is conferred, so that subsequent recurrences are uncommon. A few of these children have been reported to develop chronic disease, but most of these cases are now believed to be different glomerular diseases misdiagnosed as post streptococcal disease.

Chronic Glomerulo Nephritis

It is an advanced irreversible impairment of renal function with or without symptoms. It may develop as a primary disease or may occur in SLE or drug induced nephropathies.

CLINICAL MANIFESTATIONS

• edema• Severe hypertension• Hematuria• Nocturia• Persistent anemia• Bone pain• Bony deformities• Failure to thrive

DIAGNOSIS

• Urine examination• Blood examination- increase urea, creatinine• Urine analysis – protein, RBC, cast• USG

MANAGEMENT

• It can be done with steroid therapy and other immune suppressive drugs.• Anti hypertensive drugs and

antibiotics are useful for symptomatic measurement.

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