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This document discusses renal system diseases including glomerular diseases and nephrotic syndrome. It defines nephrotic syndrome as a manifestation of glomerular disease characterized by nephrotic range proteinuria, edema, hypoalbuminemia, and hyperlipidemia. It describes the types of nephrotic syndrome as idiopathic, secondary, and congenital. It also discusses hemolytic-uremic syndrome which is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency.
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few glomerular diseases
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- 2. GLOMERULAR DISEASES • Nephroticsyndrome -Idiopathic -Secondary -Congenital • Hemolytic uremic syndrome • Alport syndrome • IgA nephropathy • Glomerular Nephritis
- 3. NEPHROTIC SYNDROME It isa 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 proteinuria o hypoalbuminemia o edema o hyperlipidemia.
- 4. INCIDENCE: The annual incidenceis 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.
- 5. ETIOLOGY • Idiopathic nephroticsyndrome ⁻ 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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- 7. 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.
- 8. 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.
- 9. 3.Congenital Nephrotic Syndrome Itis 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. Primary 2. Secondary
- 10. • Primary congenitalnephrotic 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.
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- 13. CLINICAL MANIFESTATIONS • Weightgain • Puffiness on face (facial edema) o Especially around the eyes o Apparent on arising the morning o Subsides during the day • Abdominal swelling(ascitis) • Pleural effusion • Labial or scrotal swelling • Edema of intestinal mucosal which result in: o Diarrhea o Anorexia o Poor intestinal absorption • Ankle / leg swelling • Irritability • Easily fatigued • Lethargic • BP normal or slightly increased • Susceptible to infections • Urine alterations o Decreased volume o frothy
- 14. DIAGNOSTIC EVALUATION • Historycollection 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
- 15. THERAPEUTIC MANAGEMENT GOALS • Reduceexcrtion of urinary protein • Reduce fluid retension • Preventing infection • Minimize complications related to treatment DIETARY MANAGEMENT • Low salt diet • Fluid restriction
- 16. PHARMACOLOGICAL MANAGEMENT • Diuretictherapy 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.
- 17. STEROID THERAPY • ExtendedAPN 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
- 18. Definition of response •Remission No albumin on three consecutive early morning urine samples • Relapse 2+ or more albumin on 3 consecutive early morning urine samples • Frequent relapse 3 or more relapses in 6 months or 4 or more relapses in 1 year • Steroid resistance No remission after 8 weeks of adequate daily steroids • Steroid dependence Relapse within 15 days of stopping steroids after inducing remission or on tapering, on more than 2 occasions
- 19. NURSING MANAGEMENT a. FocusAssessment • 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)
- 20. 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
- 21. 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.
- 22. COMPLICATIONS 1.Due to drugs Toxicityof drugs may occur – Furosemide, siranolacto ne – 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
- 23. IMMUNISATIONS • The childrenwith 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
- 24. PROGNOSIS Ultimate recovery inmost 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.
- 25. HEMOLYTIC-UREMIC SYNDROME (HUS) Itis 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
- 26. CLINICAL DESCRIPTION HUS ischaracterized 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). INCIDENCE Occurs in infants and small children between the ages of 6 months and 5 years.
- 27. CASE CLASSIFICATION • Probable Anacute 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. • Confirmed An 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
- 28. CLASSIFICATION OF HEMOLYTIC UREMICSYNDROME 1.Infection Induced • Verotoxin-producing Escherichia coli • Human immunodeficiency virus 2.Genetic • von Willebrand factor- cleaving protease deficiency • Complement factor H deficiency or mutation 3.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
- 29. ETIOLOGY • Rickettsia • Bacterialtoxins (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
- 30. PATHOPHYSIOLOGY Primary injury inendothelial 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
- 31. CLINICAL MANIFESTATIONS • Historyof a prodromal disease (gastroenteritis or an upper respiratory infection) • Acquired hemolytic anemia • Sudden onset of hemolysis • Thrombocytopenia • Renal injury • Central nervous system symptoms
- 32. DIAGNOSTIC EVALUATION • Historycollectuion • Clinical examination: – Triad of anemia, thrombocytopenia and renal failure • Laboratory examination: o Urine - proteinuria, hematuria, urinary cast presence o Blood - Elevated blood urea, nitrogen, creatinine - Low hemobglobin, hematocrit - High reticulocyte count
- 33. THERAPEUTIC MANAGEMENT • earlyrecognition 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
- 34. PROGNOSIS Most recover renalfunction 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.
- 35. Immunoglobulin A Nephropathy (BergerNephropathy) 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.
- 36. INCIDENCE It is seenmore 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.
- 37. CLINICAL MANIFESTATIONS • Grosshematuria associated with loin pain. • Proteinuria often <1000 mg/24 hr. • Mild to moderate hypertension. • Normal serum levels of C3
- 38. DIAGNOSIS • History collection •Physical examination (clinical features) • Laboratorical investiagations • Renal biopsy
- 39. TREATMENT • The primarytreatment 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
- 40. PROGNOSIS Most children withIgA 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.
- 41. Alport Syndrome (hereditarynephritis) 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.
- 42. GENETICS Approximately 85% ofpatients 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.
- 43. CLINICAL MANIFESTATIONS • Asymptomaticmicroscopic 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.
- 44. 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.
- 45. TREATMENT • No specifictherapy 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.
- 46. PROGNOSIS The risk ofprogressive 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.
- 47. Acute Glomerulo Nephritis AGNis 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.
- 48. ETIOLOGY It is animmune 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.
- 49. PHASES • Phase 1– edema and oliguria present • Phase 2 – edema reduces and urine output increases
- 50. PATHOPHYSIOLOGY Streptococcal infection Production ofantigen 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
- 51. CLINICAL MANIFESTATIONS • Sorethroat/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
- 52. 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.
- 53. COMPLICATIONS • CCF • Acyterenal failure • Hypertensive encephalopathy • Persistent hypertension • Anemia • Growth failure • Chronic glomerulonephritis
- 54. MANAGEMENT -Bedrest for weekstill 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
- 55. NURSING MANAGEMENT • Carefulassessment 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
- 56. PROGNOSIS Almost all childrencorrectly 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.
- 57. Chronic Glomerulo Nephritis Itis 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.
- 58. CLINICAL MANIFESTATIONS • edema •Severe hypertension • Hematuria • Nocturia • Persistent anemia • Bone pain • Bony deformities • Failure to thrive
- 59. DIAGNOSIS • Urine examination •Blood examination- increase urea, creatinine • Urine analysis – protein, RBC, cast • USG
- 60. MANAGEMENT • It canbe done with steroid therapy and other immune suppressive drugs. • Anti hypertensive drugs and antibiotics are useful for symptomatic measurement.