case conference case conference Department of nephrology R2 하상진

ID number; Sex/Age; male/23  C. C. : fever Onset : 14 days ago  P. I. :10 년전 H-S purpura-nephritis 진단후 98 년 10 월 시행한 renal biopsy 결과 RPGN 진단 99 년 3 월 hemodialysis 시작 2000 년 7 월 13 일 KTP (donor; mother) 받음 2002 년 3 월 이식신에 대해 renal biopsy 시행 chronic rejection, 2005 년 12 월 부터 hemodialysis 다시 시작함 2 주전부터 시작된 fever 로 AKU 에서 p.o antibiotics 복용하였으나 호전없이 fever 지속되어 입원 Present Illness (1)

 PMHx. : HTN (+) DM (-) TB (-) viral Hepatitis (-) op. Hx (+) KTP (donor; mother)  Family Hx. : unremarkable  Personal Hx. : alcohol (-) smoking (-) Present Illness (2)

General fever(+) chill(+) fatigue(+) myalgia(-) skin rash(-) H & ENT headache(-) dizziness(-) sore throat(-) neck stiffness(-) Respiratory cough(-) sputum (-) hemoptysis (-) Cardiac chest discomfort(-) dyspnea(-) orthopnea(-) palpitation(-) Gastrointestinal anorexia(-) nausea(-) vomiting(+) diarrhea(-) constipation(-) hiccup (+) Genitourinary frequency(-) urgency(-) dysuria(-) urine ; 종이 한컵 / 하루 Musculoskeletal Rt big toe pain(+) ; 1 주일전, 현재는 호소하지않음 Review of systems

V/S : 130/80mmHg –88/min– 24/min – 38.3°C G/A : alert, oriented, acutely ill-looking appearance Skin : rash (-) pigmentation (-) H/N : palpable LNs (-), thyroid enlargement (-) neck stiffness (-) E/E/N/T : isocoric pupil with PLR (++/++) pale conjuntiva, whitish sclera PI (-), PTH (-) Chest : regular heart beat without murmur clear breathing sound without crackle Physical examination

Abdomen Soft and flat Normoactive bowel sound No hepatosplenomegaly Td / RT (-/-) Muscle guarding(-) Back & extremities CVAT (-/-) no pretibial pitting edema Neurology Motor & sensory ;intact Babinski sign (-/-) Physical examination

’96. H-S purpura - nephritis 진단 ( 서울 중앙병원 ) RPGN’ renal biopsy; RPGN ‘ hemodialysis initiation KTP (donor ;mother)’ KTP (donor ;mother) ‘ biopsy ; 1. chronic tubulointertial inflammation 2. glomerular sclerosis (3/7) 2. glomerular sclerosis (3/7) ’ Herpes zoster 로 입원 iv MPD pulse Tx hemodialysis’ hemodialysis 재개 ’ discontinue immunosuppressive agent Chart review

’ KTP Gum hypertrophy Old chart review Bx PDL MPM cyclosporinFK506 MIZOBMPM cyclosporin PDLMPL FK506 HDHD

 CBC with diff 6840/mm 3 – 5.9 g/dL – 19.6% - 456,000/mm 3 (Seg. 54%)  ESR / CRP 52 mm/hr / 12.5 mg/dL  PT(INR) /aPTT 1.22 / 43 c 33  Blood chemistry Total bil./Direct bil. 0.55/0.11 mg/dL ALP/ GGT 31/11 IU/L Protein/Albumin 6.4/1.8 g/dL AST/ALT 20/26 IU/L LDH/CK 389/43 IU/L BUN/Creatinine 52/10.8mg/dL Na/K/Cl 131/4.8/93 mmol/dL  U/A : RBC 0~4/HPF, WBC 0~1/HPF, Initial lab. data

E K G

Initial chest Xray

Initial simple abd

# 1. fever # 2. anemia # 3. ESRD on HD # 4. chronic rejection state in KTP Initial Problem list

Initial Assessment & Plan # 1. fever A > fever unspecified D. P. > blood CX, urine CX abdominal ultrasonography T. P. > antibiotics (amp/sulbactam3.0g iv qd) # 2. anemia A> anemia d/t chronic disease EPO resistant anemia D.P.> reticulocyte, iron, TIBC, ferritin T.P.> 1. iron supply  if needed iv iron supply 2. transfusion if needed

Initial Assessment & Plan # 3.ESRD on HD A> ESRD on HD P> scheduled hemodialysis # 4. chronic rejection state

Abdominal US

Clinical course (HD #5) S > fever (+), chill(-) sore throat (-) O > V/S :100/70mmHg –78/min– 20/min – 39°C clear breathing sound without crakcles Lab : CBC with diff 8,710/mm 3 – 6.0 g/dL – 18.9% -443k (Seg.58.2%, eos; 5.7%) CRP 14.5 mg/dL blood CX ; no growth urine Cx ; no growth A>fever unspecified d/t r/o atypical infection r/o acute on chronic rejection r/o adrenal insufficiency P>1. urine AFP & TB culture, CMV IgM/IgG, EBV Ig M/EBNA chlamydia, mycoplasma, pneumococcal Ag 2. rapid ACTH stimulation test 3. PDL 15mg QD

chest Xray f/u

 Urine culture ; no growth  Blood culture ; no growth  Urine pneumococcal Ag ; negative  CMV Ig M ; negative  Urine AFB ; negative  Cold agglutitnin ; <1:4  Mycoplasma Ab ; 1;40 (  1;80 ) Infection study

A-SAMP PDL

 Hb / Hct ; 5.9 g/dL – 19.6%  Iron /TIBC ;15 /158 μg/dL, Tranferin saturation; 9.4 %  Ferritin ; 336 ng/mL  Reticulocyte ;2.08  Erythropoeitin supply 06.4 ~ EPO2 3 회 /week  06.5 ~ EPO4 3 회 /week anemia

Final diagnosis # 1. acute on chronic rejection # 2. EPO resistant anemia # 3. ESRD on hemodialysis