Intern Seminar Presented by Int. 吳志勳 Instructed by VS. 邱元佑

Basic Information Name : 歐 x 賢 9 y /o boy Date of admission: 93/01/18 No underlying disease Normal growth and development C.C: Weight gain around 5 kg over this half a month (49.5 → 54.5 kg)

Present Illness Sore throat about 1 + week ago Increasing abdominal girth SOB easily was noted while exercise Headache (+), two times URI symptoms (+), no fever No dysuria/ grossly hematuria/ frequency forehead and bil. eyelid swelling on 1/18 → to our ER

Physical Examination ER: T/P/R:36.6/90/18, BP:162/128 puffy eyelid (+) Throat ~ non-injected Bil. clear breathing sound Abd.~ Soft, distention Extremity ~ no pitting edema Hydrocele (-)

Lab (1/18) CBC/DC WBC Hb Plt Band Seg Lymph Biochemistry CRP BUN Cr GOT GPT Na K Cl CA P

Lab ~ UA (1/18) SG PH 6.5 LEU 15 /UL NIT NEGATIVE PRO >=300 MG/DL GLU NEGATIVE MG/D KET NEGATIVE MG/D UBG 1.0 MG/DL BIL NEGATIVE MG/D ERY 200 /UL WBC 6-8 /HPF RBC >100 /HPF Epith - /HPF Cast - /HPF Crystal - /HPF Bacteria - Dysmorphic RBC 75%

Tentative diagnosis Nephrotic syndrome R/O nephritis

Admission and Plan Albumin supplement and diuretic use Check Chol/TG, IgG/transferring Throat swab ~ Group A Strep. infection 24hr urine ~ check CCr and protein loss Arrange Renal echo

Lab after admission on 1/18 Alb T-pro → hold albumin → keep lasix using

Lab (1/19) IgA 148 mg/dl C3 L 21.0 mg/dl C4 N 19.3 mg/dl ASLO H 500 IU IgG 841 mg/dl

Final Diagnosis Poststreptococcal glomerulonephritis

Clinical Course Lasix 1 A ’ qd → 1 A’ q12h → 2 A’ q12h for fluid over load and HTN Renitec 20mg 1 # qd for HTN Adalat 1 # prn for HTN Aq-penicillin 5M u q6h Low salt diet

Clinical Course 1/18 1/19 1/20 1/21 BW (49.5) AC U SBP DBP

Lab (24 hr urine) 3542 mL/24h under lasix 1 A ’ q12h CREA L 26.6 mg/dL TP 721 mg/dL Ccr 95.6 ml/min per 1.73 m 2

WBC 9.9 (1/18) 10 (1/19) CRP 13.5 (1/18) <7 (1/19) Throat swab : Normal flora isolated U/C : No bacteria was isolated B/C : No bacteria was isolated Renal Echo: normal

MBD Medication Renitec 20mg 1 # qd Lasix 1 # bid Aldalat 10mg q6h prn if BP > 140/90 Amoxil 3 # po tid

OPD ( ) BW 49.5 kg (baseline) Edema (-) Urine output ok s/p lasix using Renitec 20mg 1 # qd * 2wks Lasix 1 # bid * 1wk

Discussion Poststreptococcal glomerulonephritis

Etiology occurs 7 to 14 days after infection of group A beta haemolytic streptococcus Throat and skin infection Latent period 10 + days

Nephritogenic strains Group A β- hemolytic Respiratory tract - M1, 2, 4, 12, 18, 25 Skin – M49, 55, 57, 60 Group C Streptococci Streptococcus zooepidermicus

Epidemiology accounts for 90% of acute GN in chikdren mostly in the under fives, but may occur in early adolescence and in adults Male : female = 2:1

Clinical Features Sudden, painless, gross hematuria Tea or cola-colored urine Edema, puffy eye, hydrocele HTN Proteinuria, oligouria Heart failure, ARF, encephalopathy

Lab Finding Hematuria, dysmorphic RBC, cast Hypertension Proteinuria BUN, Cr ↑ C3 ↑, C4 normal Strp. inf. ~ antistreptozyme 、 ASLO …

Pathophysiology Complement, alternative pathway ↑ Glomerular proliferative and inflammatory response Antigen-antibody complexes in basement membrane Induce complement activation GFR 、 filtration ↓→ Na + reabsortion ↑

Pathology Proliferative GN Kidney symmetrically enlarged The basement membrane is swollen mesangial cell proliferation PMN infiltration C3 and IgG deposition Subepi. Electron dense deposits (Humps)

Diagnosis History ~ sore throat, skin inf. PE ~ HTN, fluid overload Urine sample ~ U/A, 24hr urine Biochemistry ~ albumin, protein, cholesterol complement ~ C3, C4 Antistrep. Ab ~ ASLO, streptomzyme collagen vascular disease screen throat swab and skin culture

Renal Biopsy Unresolved ARF Nephrotic syndrome C3 normal Absence evidence of strep. Inf.

Treatment Essentially supportive Diuresis Antihypertensive agent Fluid and sodium restriction Treatment for ARF Antibiotics within 36~72 hr of inf.

Treatment, still controversial Steroid Bed rest → severe, ie. encephalopathy → outcome of proteinuria Antibioyics → 36~72 hr of nephritogenic strep. Inf → family, 20% asymptomatic PSGN

Prognosis 92~98% recover completely GFR 10~14 days Gross hematuria 2~3 wks BUN/Cr 1~4 wks C3 6~8 wks Proteinuria 3~6 months Microscopic Hematuria months to years

Poor Prognosis Factors Old age Renal insufficiency at the onset degree of proeinuria

Nephrotic Proteinuria in PSGN Insidious edema Even microhematuria only HTN and azotemia

Nephrotic Proteinuria in PSGN Glomerulosclerosis and CRF → degree of proeinuria correlated with histological grade of renal biosy → crescents in more then 1/3 of glomeruli