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ACUTE NEPHRITIC SYNDROME By Hakimah KhaniBintiSuhaimi
Introduction Synonyms: acute nephritis,                            acute nephritic syndrome An immunologic mechanism: the result of 	an immune process that injures the 	glomeruli of the kidney. Clinical features: A sudden onset of hematuria,  Proteinuria, Edema,  Oliguria and volume overload, Hypertension,  Azotemia is another common but inconstant finding.
ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
Outline Definition Epidemiology Etiology and Pathogenesis Clinical Features Complication Investigation Diagnosis Differential Diagnosis Treatment and Management Prognosis
Definition 		AGNthat follows an infection with a nephritogenic strain of group A beta hemolytic streptococci. 		The classic example of the acute nephriticsyndrome. Nelson Textbook of Pediatrics, 7th Edition
Epidemiology 121 of the 124 patients had poststreptococcal nephritis.  Department of Pediatrics, HUSM, July 1987- June 1988 Globally - incidence has decreased in the past three decades.  Most commonly – sporadic. Despite that, epidemics and clusters of cases - in some poor or rural communities © 2008 American Society of Nephrology Peak incidence - age 5-12 y/o, uncommon <3y/o. Male : female ratio is 2 : 1. 	Nelson Textbook of Pediatrics, 7th Edition
Etiology and Pathogenesis The child gets gets throat or skin infection bynephritogenic strain of group A beta hemolytic streptococci - serotype 12 , 4 and 1 Antibodies to streptoccocus (egantistreptolysin O) are formed in his circulation Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM).
Streptococcal infection immune complex formation + deposited in GBM complement system activated immune injuriescellular proliferation                   GBM fracturecapillary lumen narrowed                       hematuriaglomerular blood flow decreased               proteinuriaoliguria	GFR	distal sodium reabsorptionretention of water & sodiumblood volume  edema    hypertension Low serum  complement
Clinical Features
Typical Manifestation  Develop acute nephritic syndrome 1–2 wk after an antecedent streptococcal pharyngitis or 3–6 wk after a streptococcal pyoderma. Edema 75%   of the patients Face,periorbital area lowerextremities generalized  (ascites, pleural effusions) 2.	Proteinuria– usu. normalize after 4 weeks 3. 	Oliguria school child    <  400ml/day 	preschool child   <  300ml/day 	infant & toddler  <  200ml/day
Typical Manifestation (2) Gross hematuria(65% of patients) 	Smoky, tea-colored, cola-colored, or fresh bloody urine Microscopical hematuria (almost all patients) 	The urine appears normal, but >3 RBCs/HP are found in centrifuged urine sediment examined microscopically. Hypertension (50%) – mild to moderate, typically subsides promptly after diuresis Nonspecific symptoms:  	Such as anorexia, vomiting, general malaise, lethargy, abdominal or flank pain, low-grade fever and weight gain.
Clinical course Spontaneous improvement typically begins within 1 wk with resolution of edema in 5-10 days and hypertension in 2-3 wk, but urinalysis may be abnormal (persistent microscopic hematuria) for a year.
Complications in severe cases ,[object Object]
Encephalopathy
Acute renal failure,[object Object]
Laboratory Investigations Urinalysis ,[object Object]
Microscopy: leukocytes, red blood cell casts (pathognomonic), and granular casts.
Proteinuria 2+. (Nephrotic-range proteinuria occurs in <5% of patients.)
Pyuria. The urine contains large amounts of fibrin degradation products, and fibrinopeptides.,[object Object]
Throat or skin cultures.
Antistreptolysin O (ASO) titer. Pharyngitis (80%),  skin infections (<50%).
Anti-deoxyribonuclease (DNase) B level. Pharyngitis (98%),  skin infections (80%).
Renal function Test
The BUN concentration is elevated in 75% of patients, and serum creatinine level is increased in one half of the patients, but profound decrease in GFR is uncommon in children.
Hyperkalemia, hypocalcaemia, hyponatremia, and metabolic acidosis are seen only in severe patients.,[object Object]
A mild normochromic anemia may be present from hemodilution and low-grade hemolysis.
Leococytosis maybe present.
Activation of complements
Serum C3 level, decrease (90%), return to normal within 6 weeks.
Serum C4 levels are typically normal.
Kidney ultrasound
Not necessary if patient has clear cut acute nephritic syndrome,[object Object]

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ACUTE NEPHRITIC SYNDROME

  • 1. ACUTE NEPHRITIC SYNDROME By Hakimah KhaniBintiSuhaimi
  • 2. Introduction Synonyms: acute nephritis, acute nephritic syndrome An immunologic mechanism: the result of an immune process that injures the glomeruli of the kidney. Clinical features: A sudden onset of hematuria, Proteinuria, Edema, Oliguria and volume overload, Hypertension, Azotemia is another common but inconstant finding.
  • 3.
  • 5. Outline Definition Epidemiology Etiology and Pathogenesis Clinical Features Complication Investigation Diagnosis Differential Diagnosis Treatment and Management Prognosis
  • 6. Definition AGNthat follows an infection with a nephritogenic strain of group A beta hemolytic streptococci. The classic example of the acute nephriticsyndrome. Nelson Textbook of Pediatrics, 7th Edition
  • 7. Epidemiology 121 of the 124 patients had poststreptococcal nephritis. Department of Pediatrics, HUSM, July 1987- June 1988 Globally - incidence has decreased in the past three decades. Most commonly – sporadic. Despite that, epidemics and clusters of cases - in some poor or rural communities © 2008 American Society of Nephrology Peak incidence - age 5-12 y/o, uncommon <3y/o. Male : female ratio is 2 : 1. Nelson Textbook of Pediatrics, 7th Edition
  • 8. Etiology and Pathogenesis The child gets gets throat or skin infection bynephritogenic strain of group A beta hemolytic streptococci - serotype 12 , 4 and 1 Antibodies to streptoccocus (egantistreptolysin O) are formed in his circulation Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM).
  • 9. Streptococcal infection immune complex formation + deposited in GBM complement system activated immune injuriescellular proliferation GBM fracturecapillary lumen narrowed hematuriaglomerular blood flow decreased proteinuriaoliguria GFR distal sodium reabsorptionretention of water & sodiumblood volume  edema hypertension Low serum complement
  • 11. Typical Manifestation Develop acute nephritic syndrome 1–2 wk after an antecedent streptococcal pharyngitis or 3–6 wk after a streptococcal pyoderma. Edema 75% of the patients Face,periorbital area lowerextremities generalized (ascites, pleural effusions) 2. Proteinuria– usu. normalize after 4 weeks 3. Oliguria school child < 400ml/day preschool child < 300ml/day infant & toddler < 200ml/day
  • 12. Typical Manifestation (2) Gross hematuria(65% of patients) Smoky, tea-colored, cola-colored, or fresh bloody urine Microscopical hematuria (almost all patients) The urine appears normal, but >3 RBCs/HP are found in centrifuged urine sediment examined microscopically. Hypertension (50%) – mild to moderate, typically subsides promptly after diuresis Nonspecific symptoms: Such as anorexia, vomiting, general malaise, lethargy, abdominal or flank pain, low-grade fever and weight gain.
  • 13. Clinical course Spontaneous improvement typically begins within 1 wk with resolution of edema in 5-10 days and hypertension in 2-3 wk, but urinalysis may be abnormal (persistent microscopic hematuria) for a year.
  • 14.
  • 16.
  • 17.
  • 18. Microscopy: leukocytes, red blood cell casts (pathognomonic), and granular casts.
  • 19. Proteinuria 2+. (Nephrotic-range proteinuria occurs in <5% of patients.)
  • 20.
  • 21. Throat or skin cultures.
  • 22. Antistreptolysin O (ASO) titer. Pharyngitis (80%), skin infections (<50%).
  • 23. Anti-deoxyribonuclease (DNase) B level. Pharyngitis (98%), skin infections (80%).
  • 25. The BUN concentration is elevated in 75% of patients, and serum creatinine level is increased in one half of the patients, but profound decrease in GFR is uncommon in children.
  • 26.
  • 27. A mild normochromic anemia may be present from hemodilution and low-grade hemolysis.
  • 30. Serum C3 level, decrease (90%), return to normal within 6 weeks.
  • 31. Serum C4 levels are typically normal.
  • 33.
  • 34.
  • 35. Diffuse mesangial cell proliferation with an increase in mesangial matrix.
  • 36.
  • 37.
  • 38.
  • 41. Treatment Treatment of APSGN is largely that of supportive care. Usually, patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring – nephrotic chart + fluid restriction until diuresis Management is directed at treating the acute effects of renal insufficiency and hypertension
  • 42. Diuretics Diet Fluid restriction – during oliguric phase Sodium restriction is necessary Protein restriction is unnecessary Antibiotics A 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organisms. Antibiotic therapy does not affect the natural history of glomerulonephritis. Paediatric Protocols, 12th Edition
  • 43. Treatment for complications Significant asymptomatic hypertension symptomatic, severe hypertension or hypertensive emergency / encephalopathy Paediatric Protocols, 12th Edition
  • 45. Treatment for complications Acute pulmonary edema Paediatric Protocols, 12th Edition
  • 46. Treatment for complications Acute Renal Failure Paediatric Protocols, 12th Edition
  • 47. Follow-up • for at least 1 year. • monitor BP at every visit • do urinalysis and renal func􀆟 on to evaluate recovery. • repeat C3 levels 6 weeks later if not already normalised by time of discharge Paediatric Protocols, 12th Edition
  • 48. Prognosis short term outcome: excellent, mortality <0.5%. long term outcome: 1.8% of children develop chronic kidney disease following post streptococcal AGN. These children should be referred to the paediatric nephrologists for further evaluation and management. Paediatric Protocols, 12th Edition