하지 부종을 주소로 내원한 17 세 남자환자 경희의료원 신장내과 R3 황진경 /Prof 정경환

심 O 민 17/M Chief Complaint 양 하지 부종 O/S) 1 년 전 Present Illness 2 년 전 학교에서 시행한 소변검사에서 단백뇨가 있다는 이야기 들었으나 특별한 검사 없이 지내던 자로, 약 1 년 전부터 시작된 양 하지 부종 및 활동 시 피로감을 증상으로 개인 의원 방문하였으나 큰 병원 진료 권유 받고 본원 신장내과 방문하여 입원함.

Past Medical History HTN/DM/Tb/Hepatitis (-/-/-/-) Operation History none Familial History unremarkable Personal History Smoking/Alcohol (-/-)

Review of System General fever (-) chill (-) fatigue (+) wt change (+) : 76  80kg/6months Skin rash (-) pigmentation (-) itching (-) Head/ENT headache (-) sore throat (-) Repiratory cough (-) sputum (-) rhinorrhea (-) dyspnea (-) hemoptysis (-)

Review of System Cardiovascular chest pain (-) palpitation (-) DOE (+) GI anorexia(-) nausea(-) vomiting(-) diarrhea(-) constipation(-) abdominal pain (-) Urogenital urgency (-) frequency (-) foamy urine (+) Neuromuscular numbness (-) dizziness (-) sleep disturbance(-) swelling of both leg (+)

Physical Examination Ht : 180 cm BW : 82 kg BMI : 23.5 kg/m 2 Initial V/S 155/97mmHg-75/min-20/min-36.6°C General alert consciousness chronic ill looking appearance Head/ENT isocoric pupil c pupil light reflex (++/++) neck vein engorgement (-/-) neck lymph node enlargement (-/-)

Physical Examination Chest symmetric chest expansion regular heart beat without murmur clear breathing sound without rale or wheezing Abdomen soft and flat abdomen no shifting dullness normoactive bowel sound abdominal tenderness/rebound tenderness (-/-)

Physical Examination Back/Ext costovertebral angle tenderness (-/-) pretibial pitting edema (++/++) Motor/Sensory V V VV 100

Initial Lab Findings CBC/DC 5,950 / μ L-11.2 g/dL-34.5%-257,000 (seg. 71.3%) PT 13.1 sec (INR 0.96) / aPTT 35.3 sec Chemistry TB0.57 mg/dLBUN/Cr26/2.2 mg/dL ALP/rGT66/42 U/LNa/K/Cl141/5.0/103 mmol/L Prot/Alb4.5/2.5 g/dLCa/P/Mg9.2/3.8/1.9 mg/dL AST/ALT19/30 U/LCRP<0.3 mg/dL LD/CK480/59 U/LT-chol232 mg/dL TG143 mg/dL Urinalysis RBC many /HPFWBC 0~1 /HPF pH 5.5 SG 1.08glucose - protein ++++ (Urine PCR 8.82 mg/g) ketone - nitrite -

Initial EKG

Initial Chest X-ray

Initial Problem Lists #1. Pitting edema of both leg with weight gain #2. Renal insufficiency (eGFR 42.2 mL/min/1.73m 2 ) #3. HTN #4. Microscopic hematuria #5. Proteinuria (Urine PCR 8.82 mg/g) #6. Hypoalbuminemia #7. Dyslipidemia #8. Anemia

Initial Assessment #1. Pitting edema of both leg with weight gain #2. Renal insufficiency (eGFR 42.2 mL/min/1.73m 2 ) #3. HTN #4. Microscopic hematuria #5. Proteinuria (Urine PCR 8.82 mg/g) #6. Hypoalbuminemia #7. Dyslipidemia #8. Anemia Harrison’s Principle of Internal Medicine, 18 th edition #1. Acute nephritic syndrome

Initial Assessment #1. Pitting edema of both leg with weight gain #2. Renal insufficiency (eGFR 42.2 mL/min/1.73m 2 ) #3. HTN #4. Microscopic hematuria #5. Proteinuria (Urine PCR 8.82 mg/g) #6. Hypoalbuminemia #7. Dyslipidemia #8. Anemia #2. Nephrotic syndrome Harrison’s Principle of Internal Medicine, 18 th edition

Initial Assessment #1. Acute nephritic syndrome r/o IgA nephropathy r/o ANCA small-vessel vasculitis r/o Lupus nephritis r/o PSGN ProteinuriaHematuriaVascular injury Harrison’s Principle of Internal Medicine, 18 th edition

Initial Assessment #1. Acute nephritic syndrome r/o IgA nephropathy r/o ANCA small-vessel vasculitis r/o Lupus nephritis r/o PSGN #2. Nephrotic syndrome r/o MCD r/o MGN r/o FSGS ProteinuriaHematuriaVascular injury Harrison’s Principle of Internal Medicine, 18 th edition

Initial Plans #1. Acute nephritic syndrome #2. Nephrotic syndrome Diagnostic Plans 24hrs urine collection Urine electroporesis Viral marker, Serologic marker (ANA, ANCA, IgA/G/E/M…) Kidney USG with doppler Renal biopsy Diagnostic Plans 24hrs urine collection Urine electroporesis Viral marker, Serologic marker (ANA, ANCA, IgA/G/E/M…) Kidney USG with doppler Renal biopsy

Initial Plans #1. Acute nephritic syndrome #2. Nephrotic syndrome Therapeutic Plans Diuretics ARB Therapeutic Plans Diuretics ARB

Work Up 24hrs urine collectionViral marker Serology marker 24hr urine Protein13520 mg/day 24hr urine Creatinine1825 mg/day 24hr urine Uric acid446 mg/day 24hr urine Urea Nitrogen5 g/day CrCl52.8 Serum Cr2.4 Urine Cr108 Urine Vol.1690 mL 24hr urine Ca29 mg/day 24hr urine Na200 mmol/day Urine PCR8.82 mg/g HBs Ag- Anti HBS- HCV Ab- HIV Ab- VDRL- IgG/A/M/E-/-/-/- C3- C4- C-ANCA- P-ANCA- RA factor- Cryoglobulin-

Work Up Urine EP

Kidney USG and doppler 11.9 cm11.7 cm

Renal Biopsy

Ⅰ. Glomerulus 1. Number of Glomerulus: 7 2. Glomerulosclerosis: 7/7 (%) Segmental: 4/7 (%) Global: 3/7 (%) 3. Crescent: 4/7 (%) 4. Capillary wall thickening: 0 5. Mesangial matrix expansion: 2 6. Mesagnial cell proliferation: 2 7. Inflammatory cell infiltration: 1 8. Additional pathologic findings: 0 Ⅰ. Glomerulus 1. Number of Glomerulus: 7 2. Glomerulosclerosis: 7/7 (%) Segmental: 4/7 (%) Global: 3/7 (%) 3. Crescent: 4/7 (%) 4. Capillary wall thickening: 0 5. Mesangial matrix expansion: 2 6. Mesagnial cell proliferation: 2 7. Inflammatory cell infiltration: 1 8. Additional pathologic findings: 0 Ⅱ. Tubulointerstitium 1.Mononuclear cell infiltration: 3 2.Neutrophilic infiltration: 0 3.Interstitial fibrosis: 2 4.Tubular atrophy:1 5.Acute tubular necrosis: 0 6.Tubular cast: 1 7.Tubulitis (No/tubule): 0 Ⅲ. Vessels 1.Vasculitis, endotheliitis: 0 2.Necrosis: 0 3.Intraluminal thrombi: 0 4.Art. intimal hyalinosis: 0 5.Art. medial nodular hyalinosis: 0 6.Fibrous wall thickening: 0 *Score - 0: absent, 1: focal segmental, diffuse (2: mild, 3: moderate, 4: marked) Ⅱ. Tubulointerstitium 1.Mononuclear cell infiltration: 3 2.Neutrophilic infiltration: 0 3.Interstitial fibrosis: 2 4.Tubular atrophy:1 5.Acute tubular necrosis: 0 6.Tubular cast: 1 7.Tubulitis (No/tubule): 0 Ⅲ. Vessels 1.Vasculitis, endotheliitis: 0 2.Necrosis: 0 3.Intraluminal thrombi: 0 4.Art. intimal hyalinosis: 0 5.Art. medial nodular hyalinosis: 0 6.Fibrous wall thickening: 0 *Score - 0: absent, 1: focal segmental, diffuse (2: mild, 3: moderate, 4: marked) 1. Crescenteric glomerulonephritis, consistent with 2. Chronic tubulointerstitial inflammation 1. Crescenteric glomerulonephritis, consistent with 2. Chronic tubulointerstitial inflammation

C3 IgAIgM IgA : granular (++), mesangial (++) IgM/C3 : mesangial (+), granular(+)

[ Electron microscopic findings ] Glomerulus 1. Epithelium 1) Foot process effacement (N) 2) Deposits (N) 2. GBM: diffuse sclerosis with ruptured of membrane 3. Mesangium: diffuse mesangial sclerosis Diagnosis: diffuse glomerulosclerosis

Final Diagnosis

Clinical Course Discharge 02/06 Discharge 02/06 02/10 02/ Admission 01/30 Admission 01/30 02/02 ~ 02/04 Steroid puls for 3days (MPD 500mg/day IV) Steroid puls for 3days (MPD 500mg/day IV) PDL 60mg/day 50mg/day 40mg/day Urine PCR : 8.82 mg/gUrine PCR : 7.69 mg/g Madipine 10mg/day Torsemide 10mg/day