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Pediatric Renal Diseases

Pediatric Renal Diseases. Developmental and Physiological Aspects 1. Urine volume: Newborns 1~3 ml/kg/h 3~10 d 100~300 ml/d ~2 m 250~400 ml/d ~1 y 400~500 ml/d .

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Pediatric Renal Diseases

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  1. Pediatric Renal Diseases

  2. Developmental and Physiological Aspects1. Urine volume: Newborns 1~3 ml/kg/h 3~10 d 100~300 ml/d ~2 m 250~400 ml/d ~1 y 400~500 ml/d

  3. ~3 y 500~600 ml/d ~5 y 600~800 ml/d ~8 y 600~1000 ml/d ~14 y 800~1400 ml/d >14 y 1000~1600 ml/d

  4. ▲Oliguria (low urine output): Newborns < 1ml/kg/h Infant & infancy <200ml/m2/d Pre-school age <300ml/m2/d School age <400ml/m2/d ▲Anuria: < 50 ml/m2/d (newborns < 0.5 ml/kg/h)

  5. 2.Routine urine test 2.1. Urine color--normally yellow, color changes may be normal or abnormal2.2. PH: normal range 5~72.3. Specific gravity newborns – 1.006~1.008 , >1 year old – 1.011~1.025

  6. 2.4. Urine analysis – freshly collected and centrifugal urine● RBC < 3/hpf● WBC < 5/hpf● Casts–cellular (RBC, WBC) and granular casts are abnormal, hyaline casts can be normal

  7. ●Crystals – phosphate and urate crystals may be normal ● Protein(Pro) – negative ● Sugar (Glu) ●Ketones (Ket) ● Urobilinogen (Uro) ● Bilirubin (Bil)

  8. 3. Addis countRBC < 50,0000, WBC <1,000,000, Casts < 50004. 24h total urinary protein less than 100 mg/m2/d, or <4 mg/m2/h, or <100 mg/L, or <150 mg/d

  9. 5. Renal function tests: BUN, Cr6. Imaging procedures X-ray, Ultrasound, VCUG, Nuclear medicine (99mTc DMSA, 99mTc DTPA), IVP etc.7. Renal Biopsy

  10. Glomerular Diseases

  11. Classify▲Clinical classify 1. Primary glomerular diseases 1.1. Glomerulonephritis (Nephritis) ﹉Acute glomerulonephritis

  12. ﹉Rapidly progressive glomerulo- nephritis (RPGN)﹉Persistent glomerulonephritis ﹉Chronic glomerulonephritis

  13. ﹉Rapidly progressive glomerulo- nephritis (RPGN)﹉Persistent glomerulonephritis ﹉Chronic glomerulonephritis

  14. 1.2. Nephrotic syndrome (NS) ﹉Simple tape NS﹉Nephritic tape NS

  15. 1.3. Asymptomatic (isolated) hematuria or proteinuria 1.4. Familial nephritis 2. Secondary glomerular diseases– it is part of mul- tisystem disorder, e.g. –

  16. 2.1. Hepatitis B virus related glomerulonephritis (HBV-GN) 2.2.Purpuric nephritis 2.3.Lupus nephritis (LN)▲Pathologic classify ▲Immunopathology classify

  17. Acute Glomerulonephritis (AGN)

  18. DefinitionGlomerulonephritis is a various group of diseases– acute nephritic syndrome.★Acute poststreptococcal glomerulonephritis, APSGN(acute nephritis)

  19. ●Incidence age: in 5 ~14 years old● peak age: 3~7 years old ●Boys > girls = 2:1 ●Incidence peak: Jan. Feb. Sep. and Oct.

  20. Etiology & Pathogenesis●Bacterial: ▲ group Aβ- hemolytic streptococci,Staphylococci, Pneumococci, Gˉ bacilli

  21. ● Viral: influenza virus, mumps virus , Coxsackie virus, ECHO virus and EBV● Other pathogens fungi etc.

  22. The immunoreaction caused by group Aβ- hemolytic strep-tococci－nephritogenic strans

  23. Circulating immunecomplexes (CIC)Antigens+antibodies In situ immunecomplexes

  24. →deposited on glomerular capillaries → complement system activated→immune mediators and inflammatory mediators

  25. Pathology1. The feature of pathological changes: Diffus, exudative and proliferative inflammation of the glomerulus

  26. 2. Chief varietyEndothelial and mesangial cells proliferation with leukocyte infiltration; immunofluorescence shows granular IgG & C3 deposits

  27. Electron microscopy ★Hump-like electron dense deposits on epithelial side of GBMPathophysiology (Figure)

  28. Infection of streptococciImmune complexes Local immune inflammation in glomerular capillaries Stenosis of blood Glomerular filtration capillary cavity membrane injury GFR ↓ Hematuria Proteinuria Oliguria Cylindruria Blood volume↑ Venous pressure↑ Edema Circulatory load↑ Hypertension

  29. Clinical Manifestations● Prodromal infectionspharyngitis, scarlet fever, Angina, and pyoderma● Incubation period: about 10 days for pharyngitis, 14~20 days for skin infection

  30. 1.Typical findings (general case) 1.1. Ordinary symptoms: low grade fever, nausea, debility, malaise, anorexia and vomiting, etc.

  31. 1.2 Principal symptoms (nephric signs) a. Edema (nonpiting edema, nephritic edema) Edema is the most common initial sign– Periorbital edemaOliguria may be present

  32. b. HematuriaMicroscopic ~ (most of cases) – >5/hpf,Gross~ (1/3~1/2 cases) – usually tea or cola colored (brownish) urine, continue 1~2 w

  33. 肉眼血尿

  34. c.Hypertension:1/3~2/3 cases Pre-school age>120/80mmHg School age>130/90 mmHg Headache may be present d. Proteinuria:<3+

  35. 2.Severe findings (Severe case) Appear the following symp- toms within 2 w of the onset.

  36. a. Circulatory congestionRR↑, HR↑, fidget , hepa- tomegaly→→dyspnea, jugular phlebectasia, pulmonary edema, gallop rhythm and cardiac dilation

  37. Chest X-ray: Enlarged cardiac silhouette, lung markings coarsen (pulmonary vascular congestion)

  38. b.Hypertensive encepha- lopathy BP↑→brain hypoxia and edema

  39. Smart headache, nausea, vomiting and diplopia or transient blindness→convulsion, coma●Hypertensive crisis

  40. c. Acute renal insufficiency Severe oliguria or anuria→ temporary azotemia, distur- bance of electrolytes and metabolic acidosis

  41. 3. Atypical findings (Atypical case) ▲Extrarenal symptomatic nephritis▲ Acute nephritis with neph- rotic manifestation ▲ Asymptomatic AGN

  42. Laboratory investigations1.Routine urinalysis RBC↑, 2+~ 3+, > 5/hpf, protein 1+ ~ 3+, may occur hyaline (or granular or red cell) casts, +/- WBC

  43. 2.Blood exam 2.1. Hemogram: initial mild anemia ( due to hemodilution), WBC↑ or normal2.2. ESR↑ 3.Renal functions: BUN and Cr are normal or slight increase

  44. 4.Immunologic examEvidence of recent streptococcal infection—4.1. ASO↑: 70%~80% of patients, 10~14 days after infected, incidence peak at 3~5w , normal after 3~6 m

  45. 4.2. ADNase-B: positive rate is high (more than 90% cases )4.3. ADPNase4.4. Ahase

  46. 5.Serum complement 80%~90% cases– low CH50 and low C3 (within 2 w of the onset), normalized in 6~8 w●If C3 still low after 8 w — other etiology?

  47. Course & Prognosis▲Course: About 2 w▲Routine urine test: returns to normal within 4~6 w▲ESR: returns to normal within 2~3 m

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