1. 2014.8.18 신장내과 R3 강현준 /Prof. 이태원 MGR 양측 하지부종을 주소로 내원한 48 세 남자

2. Chief complaint both leg edema onset) 2014.4 Present illness 48/M, DM,HTN 있는 자로 내원 3 개월전 발생한 both leg edema 로 원주기독병원 입원하여 renal Bx 시행한 결과 DM nephropathy 진단받고 보존적 치료하였으나 호전 없어 2014/5/26~30 본원 신장내과 입원하여 약물조정 후 퇴원했 던 자로 both leg edema 및 nephrotic range proteinuria 지속 되어 신장질환 재평가 위해 입원함 M/48 김 O 규 12412346 admission date : 2014/07/07

3. Past Hx  DM/HTN/Tb/Hepatitis (+ /+ /- /-)  DM, HTN : 10 년 전 원주기독병원에서 진단  Pacemaker insertion (1996, 원주기독병원 )  OP Hx (-) Medication Hx  Furosemide 80mg bid  Spironolactone 25mg bid  hydrochlorothiazide 25mg bid  gliclazide 6mg qd  Linagliptin 5mg qd  Atorvastatin 40mg qd Personal Hx  Alcohol (-) 과거음주 (5 년전 금주 ), 소주 0.5 병, 주 1 회  Smoking (-) Family Hx  어머니, 형 DM

4. Review of System General Chill(-) Fatigue(-) Headache(-) both leg edema(+) Wt change(+) : 평상시 79Kg -> 86Kg(2014/5/27) ->79.5Kg(5/30) -> 80.5Kg(7/7) Skin Rashes (-) Itching(-) Jaundice(-) Cardiac Chest Pain(-) Palpitations(-) Respiratory Cough(-) Sputum(-) Dyspnea(-) Hemoptysis (-) Gastrointestinal Anorexia(-) Nausea(-) Vomiting(-) Diarrhea(-) Genitourinary Flank pain(-) Dysuria(-) Urgency(-) foamy urine(+) Nervous Dizziness(-) Sensory loss(-) paresthesia (-)

5. Physical exam Vital sign 100/60 mmHg – 90 /min – 20 /min – 36.9 ℃ Height : 167.8 cm Weight : 80.3 kg General Alert mentality Chronic ill looking appearance Skin Rash(-) Striae (-) Pigmentation(-) Petechiae(-) Cyanosis(-) Chest Clear breathing sound without rale Regular heart beat s murmur Abdomen Soft & flat abdomen Td/rTd (-/-) Back&Ext. CVA Td (-/-) Pretibial Pitting edema (+++/+++) Clubbing (-) Neurology Motor, sensory : intact

6. Lab findings (2014.7.7)  CBC/DC 7570/uL – 16.2 g/dL – 47.1 % – 296,000 /uL (Seg.neutrophil 51.7%, Lymphocyte 38.4%,Monocyte 4.8%, Eosinophil 2.4%) PT / INR 12.3 sec / 0.91 aPTT 39.1 sec  Chemistry TB 0.4 mg/dL BUN/ Cr 23/ 0.9 mg/dL Total chol. 365 mg/dL Ca/ P 7.6 / 3.7 mg/dL Prot/ Alb 4.0/ 1.9 g/dL Na /K /Cl 133 / 3.5 /93 mEq/L ALP 84 IU/L AST/ALT 21 / 12 IU/L Total CO2 30.6 CRP < 0.3 mg/dL  Urine analysis pH 6.5S.G 1.043 Occult blood - / Glucose + / Ketone - / protein ++++ RBC / WBC0~1/ 0~1

7. Lab findings (2014.7.7)  Urine Protein/Cr ratio Urine Protein 532.0 (mg/dL) Urine Creatinine 38.0 (mg/dL) Urine Protein/Cr Ratio 16.63 (mg/mg)

8. Chest X-ray (2014.7.7)

9. EKG (2014.7.7)

10. 2014/5/6~25 원주기독병원 입원  타병원 기록지 Serum albumin : 2.1 g/dL (2014/5/6) Urine PCR : 11.36 mg/mg (2014/5/6) 24hr urine protein : 21756 mg/day (2014/5/7)  Renal Bx(2014/5/13 ) : suggestive of eary diabetic change  Medication Metformin 1000mg Glimepiride 2mg Irbesartan 150mg Furosemide 40mg Atorvastatin 20mg Aspirin 100 mg Pre - Clinical course

11. Serologic markers(5/26) IgG/A/M/E 239/382/149 /107 C3 (88 ~ 201mg/dL) 153 C4 (16 ~ 47mg/dL) 31.3 C-ANCANegative P-ANCANegative RA factor (~ 20IU/mL ) <10 CryoglobulinNegative ASO titer (~ 200IU/mL) 57.5 ANANegative viral markers(5/27) HBs AgNegative Anti-HBs AbNegative Anti-HCV AbNegative Anti-HIV AbNegative Urine dysmorphic RBC <5 S-PEP No restriction S-IFE No restriction U-PEP prominent albuminuria U-IFE No restriction

12. Pre - Clinical course 안저검사 당뇨병성 망막병증 -/- 당뇨병성 황반부종 -/-

13. Pre - Clinical course 본원입원 (5/26~30) OPD(6/9) 24hr protein : 18.5g/day uPCR : 13.5 Diuretics OHA 24hr protein : 18.5g/day uPCR : 13.5 Diuretics OHA uPCR 12.8 Diuretics 유지 OHA 유지 uPCR 12.8 Diuretics 유지 OHA 유지 입원 (7/7) 24hr protein : 10.7g/day Diuretics 유지 OHA 유지 24hr protein : 10.7g/day Diuretics 유지 OHA 유지 OPD(6/23)

14. Initial Problems #1. Nephrotic range proteinuria #2. Pitting edema #3. Hyperlipidemia #4. Hypoalbuminemia #5. DM #6. HTN #7. Pacemaker insertion state d/t sick sinus syndrome

15. Initial assessment & plan #1. Nephrotic range proteinuria #2. Pitting edema #3. Hyperlipidemia #4. Hypoalbuminemia  Assessment) Nephrotic syndrome d/t DM nephropathy, r/o d/t MCD r/o d/t MGN r/o d/t FSGS  Diagnostic plan) Abdomen & pelvis US, urine chemistry, 24h urine collection renal biopsy  Therapeutic plan) Treatment underlying disease, diuretics,I/O control

16. Initial assessment & plan #5. DM  Diagnostic plan> HbA1C check, GMT monitoring  Therapeutic plan> OHA 용량 조절, 필요시 insulin 사용 #6. HTN  Diagnostic tic plan> BP monitoring  Therapeutic plan> anti-hypertensives modification

17. #7. Pacemaker insertion state d/t sick sinus syndrome  Diagnostic plan> EKG check  Therapeutic plan> 필요시 IC consult

18. Clinical course  2014/7/8 AP-US :

19. Clinical course Kidney biopsy(2014.7.8 )

21.  I. Glomerulus  1. Number of Glomerulus: 17  2. Glomerulosclerosis: Present ( 2/17)  Segmental glomerulosclerosis: 0/17  Global glomerulosclerosis: 2/17  3. Size of Glomerulus: Increased (moderately)  4. Pericapsular fibrosis: Absent  5. Tuft adhesion: Absent  6. Crescent: Absent  7. Cellularity: Normal  8. Mesangial widening: Absent  9. Mesangial cellularity: Normal  10. Capillary wall changes: Absent or minimal  11. Wire-loop formation of capillary wall: Not identified  12. Capillary luminal occlusion: Absent  13. Capillary necrosis: Not identified  II. Interstitium  1. Inflammatory cell infiltration:  -Mild (10-25%)  -Lymphocytes  2. Fibrosis: Absent  III. Tubules  1. Tubular atrophy: No (<5%)  2. Acute tubular injury: Absent  3. Tubulitis: Absent  IV. Vessels  1. Arterio- or Arteriolosclerosis: Absent  2. Hyalinosis: Absent  3. Fibrinoid necrosis: Absent  4. Vasculitis: Absent LM findings

22. IF findings Total No. Glomerulus Mesangium GCWTubuleVesselOthers IgG4----- IgA4----- IgM4----- C34----- C1q4----- Kappa4----- Lambda4-----

25. EM findings  1. Epithelium  1) Foot process effacement (yes), diffuse  2) Deposits: No  2. GBM  1) Thickness(nm) - thickening  2) Contour: no pathologic abnormality  3) Texture: no pathologic abnormality  4) Deposits: No  3. Mesangium  No pathologic abnormality  4. Endothelium  No pathologic abnormality  Diagnosis  Kidney, native, biopsy: Minimal change disease

26. Clinical course PDL 60mg + CyP 100mg -> 퇴원시 PDL 40mg + CyP 100mg 24hr protein : 32mg/day(8/6) 24hr protein : 10.3g/day(7/16) 24hr protein : 18.5g/day(5/30)

27. Final diagnosis #1. Minimal change disease #2. DM #3. HTN

28. 감사합니다

32. Clinical course 입원 (7/7~17) OPD(7/25) 7/8 renal Bx 시행 7/10 MCD 진단 -> cyclophosphamide 50mg bid+PDL 60mg qd 투여시작 7/17 퇴원시 Diuretic 유지 cyclophophamide 50mg bid + PDL 40mg qd uPCR(7/7) : 16.6 uPCR(7/15) : 11.4 24hr urine protein(7/16) : 10.3g/day 7/8 renal Bx 시행 7/10 MCD 진단 -> cyclophosphamide 50mg bid+PDL 60mg qd 투여시작 7/17 퇴원시 Diuretic 유지 cyclophophamide 50mg bid + PDL 40mg qd uPCR(7/7) : 16.6 uPCR(7/15) : 11.4 24hr urine protein(7/16) : 10.3g/day Diuretics 유지 cyclophophamide 50mg bid + PDL 30mg qd uPCR 0.79 Diuretics 유지 cyclophophamide 50mg bid + PDL 30mg qd uPCR 0.79 Diuretics 유지 cyclophophamide 50mg bid + PDL 20mg qd 24hr protein : 32mg/day Diuretics 유지 cyclophophamide 50mg bid + PDL 20mg qd 24hr protein : 32mg/day OPD(8/6)

33. INSULIN ARB + CCB Kidney biopsy (2011.4.14 ) NPDR 진단

34. Final diagnosis #1. Minimal change disease #2. DM #3. HTN

35. 감사합니다

49.  I. Glomerulus  1. Number of Glomerulus: 3  2. Glomerulosclerosis: 2/3 Nodular glomerulosclerosis : 1/2 Global glomerulosclerosis : 1/2  3. Capillary wall changes: thickened, focal  4. lumen of capillary: obliteration, focal  5. Tuft adhesion: (Yes) 1/2  6. wire-loop formation : 0  7. capillary necrosis : 0  8.cellularity : normal  9. Mesangial widening: increased (moderate)  10. mesangial cellularity : normal(<3)  Crescent : (yes) 1/2 cellular crescent : 1/1  II. Tubules  1. Tubular atrophy : Mild(6-25%)  2. degeneration : 0, regeneration : 0  3. acute tubular necrosis : 0  4. tubular case : hyaline casts, a few  5. tubulitis : 0  III. Interstitium  1. edema : 1  2. Fibrosis : 1  3.inflammatory cell infiltration : Mild(11-25%), lymphocyte, plasma cell, eosinophil  4. Tamm-Horsfall protein leakage 0  IV. Vessels  1. Sclerosis:1  2. Intimal change : 1  3. medial change : 0  4. necrosis : 0  5. luminal change :0 LM findings

50. IF findings ResultG.LinearG.GranularMegansialOthersTubulesInterstitiumvessels C1q---+/----- C3--++-+/-- C4--+/------ Fibrinogen-+-+-+-+/- IgA--+/------ IgG-++---+-+/- IgM--+-----

53. EM findings  1. Epithelium  1) Foot process effacement (yes), diffuse  2) Deposits: No  2. GBM  1) Thickness(nm) - thickening  2) Contour: irregular  3) Texture: no pathologic abnormality  4) Deposits: No  3. Mesangium  Increase of mesangial matrix with mesangial sclerosis  4. Endothelium  No pathologic abnormalities > Clinical course 2

54. Final diagnosis #1. CKD d/t diabetic nephropathy #2. DM #3. HTN #4. Non- proliferative diabetic retinopathy

55. 감사합니다