Nephritic syndrome for C1 new 2016 one of best presentations.pptx

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1. Nephritic syndrome, AGN Meron Shimeles Assist Prof Pediatrics and Child Health

2. Outline Anatomy of glomerulus Clinical manifestation of Nephritic syndrome Acute post streptococcal glomerulonephritis

3. Anatomy of glomerulus Kidneys lie in retroperitoneal space. In newborns – length ~ 6cm & Wt ~ 24 gm In adults – length ~ 12 cm & Wt ~ 150 gm It has an outer layer – the cortex & inner layer – the medulla Each kidney contains approximately 1 million nephrons (each consisting of a glomerulus and associated tubules).

4. Anatomy … ctd The cortex contains: Glomeruli, proximal & distal convoluted tubules, and collecting ducts The medulla contains: Straight portion of the tubules, the loops of Henle , vasa recta & terminal collecting ducts Glomerulus: a tuft or bunch of capillaries that lie between two arterioles Arranged in lobules and supported by a stalk consisting mesangial cells and extra-cellular matrix

5. Glomerular Basement Membrane (GBM) – Forms a continuous layer between the endothelial and mesangial cells on one side and the epithelial cells on the other. GBM has three layers: A central electron dense lamina densa (LD) Lamina rara interna (b/n LD and endothelial cells) Lamina rara externa (LRE) (b/n LD and epithelial cells) Bowman’s capsule (BC) – surrounds the glomerulus and is composed of the BM and proximal tubule; and it is lined by epithelial cells (parietal and visceral)

6. Glomerular Filtration (GF) Plasma is filtered through glomerular capillary walls to ultra filtrate collected in BC. Ultra filtrate is cell free and contains: All plasma substances except proteins ( like albumin and globulins) having a molecular Wt > 68,000 Major factors restricting filtration include: Size and ionic charge GBM is site for size selective filtration and Endothelial, epithelial cells and BM have negative ionic charges repelling plasma proteins

7. GFR = 0.413 x height(cm)/Serum creatinine (mg/dl) GF approximates that of adult at 3 years of age

8. Pathogenesis Glomerular injury may be a result of genetic, immunologic, perfusion, or coagulation disorders. The whole mark of glomerulonephritis is inflammation of the glomerulus- proliferation of cellular elements secondary to an immunologic mechanism.

9. Clinical Manifestation Acute nephritic syndrome is characterized by the sudden onset of hematuria, oliguria, edema and hypertension. Acute renal failure may develop with variable presence of oliguria and elevated serum Creatinine and BUN. Hyperkalemia may sometimes develop

10. Clinical course Less fulminant course : characterized by nephritic sediment with mild or no impairment in renal function and mild or no recognizable clinical symptoms. Rapidly progressing course : with rapid deterioration of renal function within few days with abnormal urinary sediment and dramatic presentation.

11. Causes With low serum complement level Acute post streptococcal glomerulonephritis SLE Sub acute bacterial endocarditis Membranoproliferative glomerulonephritis (type I and II) Shunt nephritis With normal complement level Diarrhea associated Hemolytic Uremic syndrome IgA nephropathy Henoch-Schonlein purpura Alport's syndrome

12. Acute post-streptococcal glomerulonephritis It is the most common form of acute glomerulonephritis in children. It is typically found in preschool and school age children; commonly between the ages of 5-12 years. It is uncommon in under 3 years of age because of infrequency of Group A streptococcal pharyngitis and impaired immunogenictiy of patients.

13. Acute poststreptococcal glomerulonephritis (APSGN) is a classic example of the acute nephritic syndrome characterized by the sudden onset of gross hematuria, edema, hypertension, and renal dysfunction.

14. Etiology and Epidemiology APSGN follows infection of the throat or skin by certain nephritogenic strains of group A β-hemolytic streptococci.

15. Pathology Glomeruli appear enlarged and relatively bloodless and show a diffuse mesangial cell proliferation, with an increase in mesangial matrix . Crescents and interstitial inflammation may be seen in severe cases

16. Pathogenesis Mediated by immune complexes. Molecular mimicry whereby circulating antibodies elicited by streptococcal antigens react with normal glomerular antigens, in situ immune complex formation of antistreptococcal antibodies with glomerular deposited antigen, and complement activation by directly deposited streptococcal antigens continue to be considered as probable mechanisms of immunologic injury.

17. Clinical Manifestation most common in children aged 5-12 yr and uncommon before the age of 3 yr. Develops 1-2 wk after an antecedent streptococcal pharyngitis or 3-6 wk after a streptococcal pyoderma . The severity of kidney involvement varies from asymptomatic microscopic hematuria with normal renal function to gross hematuria with acute renal failure. Depending on the severity of renal involvement, patients can develop various degrees of edema, hypertension, and oliguria.

18. Patients are at risk for developing encephalopathy and/or heart failure secondary to hypertension or hypervolemia. Hypertensive encephalopathy must be considered in patients with blurred vision, severe headaches, altered mental status, or new seizures. Peripheral edema typically results from salt and water retention and is common.

19. The acute phase generally resolves within 6-8 wk. Although urinary protein excretion and hypertension usually normalize by 4-6 wk after onset, C3 level returns to normal within 6-8 weeks; Proteinuria resolves within 3-6 months; persistent microscopic hematuria can persist for 1-2 yr after the initial presentation.

20. Diagnosis Urinalysis… RBCs, RBC cast, proteinuria, polymorphonuclear leukocytes mild normochromic anemia Serum C3… reduced … returns to normal 6-8 wks after the onset. Serum C4… normal or mildly depressed. Positive throat culture… support diagnosis A rising antibody titer to streptococcal antigen(s) confirms a recent streptococcal infection. ASO antideoxyribonuclease B level

21. Blood chemistry: BUN, Cr, Na+, K Renal ultrasound may show mildly enlarged and echogenic kidneys MRI … posterior reversible encephalopathy syndrome Chest x ray… heart failure, pulmonary edema

22. DDx systemic lupus erythematosus endocarditis membranoproliferative GN acute exacerbation of chronic GN. IgA nephropathy Goodpasture syndrome Idiopathic RPGN

23. Complications Hypertension Acute renal dysfunction heart failure Hyperkalemia Hyperphosphatemia Hypocalcemia acidosis Seizure Uremia

24. Prevention Early systemic antibiotic therapy for streptococcal throat and skin infections does not eliminate the risk of GN. Family members of patients with acute GN, especially young children, should be considered at risk and be cultured for group A β-hemolytic streptococci and treated if positive.

25. Treatment A 10-day course of systemic antibiotic therapy with penicillin is recommended to limit the spread of the nephritogenic organisms. Sodium and fluid restriction, diuretics, and pharmacotherapy with calcium channel antagonists, vasodilators, or angiotensin-converting enzyme inhibitors are standard therapies used to treat hypertension.

26. Prognosis Complete recovery occurs in > 95% of children with APSGN. Recurrences are extremely rare. Mortality in the acute stage can be avoided by appropriate management of acute renal failure, cardiac failure, and hypertension. Infrequently, the acute phase is severe and leads to glomerulosclerosis and chronic renal disease in < 2% of affected children.

27. Thank You!

28. Urinary Tract Infection

29. Objectives Prevalence and Etiology Risk Factors Classification and clinical manifestation Investigation Management

30. Prevalence most common in children under age 1 year. First year …. M:F = 2.8:5.4 Beyond 1 to 2 yr …. M:F = 1:10 more common in uncircumcised males In females, peaks during infancy, toilet training and onset of sexual activity.

31. Etiology caused primarily by colonic bacteria. Escherchia coli …. 54 – 67% of all UTIs; followed by Klebsiella spp , and Proteus spp , Enterococcus and Pseudomonas . Staphylococcus saprophyticus , group B Streptococcus and Stapphylococcus aureus , Candida spp , and Salmonella spp .

32. Risk Factors Female gender Uncircumcised male Vesicoureteral reflux Toilet training Voiding dysfunction Obstructive uropathy Urethral instrumentation Sources of external irritation (such as tight clothing, pinworm infestation) Constipation Anatomic abnormality (labial adhesion) Neuropathic bladder Sexual activity Pregnancy

33. Classification Pyelonephritis Cystitis Asymptomatic bacteriurea

34. Pyelonephritis characterized by abdominal, back or flank pain; fever, malaise; nausea; vomiting; and occasionally diarrhea. Fever may be the only manifestation. New born: poor feeding, irritability, jaundice and weight loss. Can result in renal injury … pyelonephritic scarring.

35. Cystitis indicates there is only bladder involvement. Symptoms: dysuria, urgency, frequency, suprapubic pain, incontinence and malodorous urine. doesn’t cause high fever or any renal scarring. Acute hemorrhagic cystitis – often caused by E. coli, adenovirus types 11 and 21.

36. Pathogenesis Ascending infection Rarely hematogenous

37. Diagnosis Urinalysis / symptoms … entry point nitrites, leukocyte esterase, WBC, microscopic hematuria Urine culture midstream/ suprapubic aspirate/ catheterized adhesive, sealed, sterile collection bag after disinfection of the skin.

38. If the culture shows > 50,000 colony-forming units/mL of a single pathogen ( suprapubic or catheter sample) and the urinalysis has pyuria or bacteriuria in a symptomatic child, the child is considered to have a UTI. In a bag sample, if the urinalysis result is positive and the patient is symptomatic, a catheter sample should be obtained for culture. Leukocytosis, neutrophilia , elevated ESR, prolactinin level and C-reactive protein Bacteremia in the setting of pyelonephritis is reported to occur in 3–20% of patients and is most common in infants less than 90 days old.

39. Imaging acute lobar nephronia or renal abscess ultrasound, CT The AAP practice parameter recommends initial ultrasound of the kidneys, ureters, and bladder for children 2-24 mo with a first episode of UTI. VCUG is indicated only if the ultrasound study indicates hydronephrosis , scarring or other findings suggestive of reflux or obstructive uropathy , or if the patient has other atypical complex features. Further, they recommend VCUG if the child has a recurrent febrile UTI.

40. Treatment Acute cystitis trimethoprim- sulfamethoxazole Nitrofurantoin Amoxacilline Acute febrile UTI IV rehydration IV antibiotic … ceftriaxone, cefepime , cefotaxime PO… cefixime , cephalaxine , ciproofloxacine Duration… 7 to 14 days