Case Conference 내분비 대사 내과 R1 최소영

11686478 정혜연 F/25 내원일 :2003년 11월 29일 CC: Alopecia O/S: 3년 전 PI : 7년전부터 Weight gain (59kg→68kg)있었고 6년전부터 Amenorrhea 있어 산부인과 방문, progestrone 주사후 소퇴성 출혈 있는 상태로 비만으로 인한 PCO 의심하에 Weight reduction 시도함. 3년 전(2000년)부터 시작된 alopecia로 03년 10월 본원 피부과 진료중 Cushing’s syndrome 의심되어 03년 11월 본원 IE로 진료 의뢰. Overnight dexamethasone supression test하여 Cushing’s syndrome 의심되어 admission Overnight dexamethasone( 1mg ) supp. Test (2003.11.14) S-ACTH : 33.7 pg/mL S-cortisol : 19.1 ug/dL 24hr urine free cortisol : 590.8 ug/day (cr: 0.7g/day)

PMHx FMHx PHx : 담배 (-) 술 (-) 고혈압(+) : 03년 5월 백병원에서 진단받고 medication 시작함 당뇨(-): OPD OGTT상 IGT 간염(-) 결핵(-) 약물복용력: amlodipine 10mg qd-03년 5월부터 시작 수술력(-) FMHx PHx : 담배 (-) 술 (-)

Review of System General : fatigue(-) fever(-) chills(-) sweat(-) weight loss(+):9kg/3Mo Skin : rash(-) abdominal puplish striae(+) thin skin(+) hirsuitism(+):귀밑, 뒷머리 acne(+):face & body alopecia(+) Head & Neck : headache(-) trauma(-) stiiffness(-) moon face(+) buffalo hump(+) Eye & ENT : sore throat(-) dry mouth(-) hoarseness(-) Respiratory : cough(-) sputum(-) dyspnea(-) Cardiac : palpitation(-) chest discomfort(-) orthopnea(-) Gastrointestinal : A/N/V/D/C(-/-/-/-/-) abdomial pain(-) Urinary : dysuria(-) nocturia(-) frequency(-) Gynecology : amenorrhea(+) -6년전 Endocrine : polydipsia(+) heat intolerance(-) Musculoskeletal : myalgia(-) weakness(-) numbness(-) back pain(-) Knee joint pain(+)

Physical Examination V/S : 140/95 mmHg - 88회/min - 20회/min - 36.7°C height: 159cm body weight: 64 kg BMI:25.3 kg/m2 General apperance alert conciousness Chronic ill appearance Skin skin turgor : normal purple striae: abdomen, extremities acne(+): face & body hirsuitism: 귀 밑, 뒷머리 Head & Neck moon face(+) no neck vein engoregement no cervical LN enlargement no thyroid gland enlargement

Eye & ENT isocoric pupil with PLR(+/+) pinkish conjunctiva clear sclera Pharyngeal injection(-) peritonsilar hypertrophy(+/+) Thorax symmetric chest expansion clear breathing sound without rale regular heart beat without murmur Abdomen abdominal striae (+) : purple color soft and flat abdomen normoactive bowel sound no tenderness , no rebound tenderness Back / Extremity CVA tenderness(-/-) pretibial pitting edema(-/-)

Impression Cushing’s syndrome

Diagnostic Plan Brain (Sella) MRI Low & high dose Dexamethasone supression test Brain (Sella) MRI Abdominal CT

Laboratory Finding ( 03.11.29 ) CBC/DC 10400/mm3 – 13.5g/dL-40.4 %-326000/mm3 Chemistry TB/DB 0.6/0.1 mg/dL AST/ALT 20/20 IU/L ALP/GGT 20/26 IU/L Prot/Alb 6.9/4.0 g/dL Glucose 109 mg/dL LD/CK 381/40 IU/L BUN/Cr 14/0.7 mg/dL Na/K/Cl 147/4.3/108mmol/L Ca/P/Mg 9.3/3.8/2.0 mg/dL UA RBC 0-1/HPF WBC 0-1/HPF

EKG (03.11.29)

Chest X-ray (03.11.29)

Abdominal CT (03.12.01)

Brain (Sella) MRI(03.12.01)

Low & High dose DMST (03-12-01~12-05)

Basal level Low dose DMST (0.5mg q6hr for 2day) High dose DMST S-ACTH 8AM-4PM 38.2 pg/mL - 38.8 pg/mL (Nl 6-76 pg/mL (8AM), 5-37pg/mL 4PM)) 57.4 pg/ml - 41.8 pg/ml 19.9 pg/ml - 20.9 pg/ml S-cortisol 8AM-4PM 9.3 ug/dL - 8.7 ug/dL (Nl 5-25 ug/dL(8AM), 3-12ug/dL(4PM)) 22.6 ug/dl -30.3 ug/dl (if suppression; <5 ug/dl) 8.2 ug/dl -13.9 ug/dl 24hr urine free cortisol 183.0→ 366 ug/day 494.5 → 706 ug/day (if suppression : <20ug/day) 191.4 → 638 ug/day (if suppression : < 90% of basal level) 17-OHCS (2~6.8 mg/day) 5.2 mg/day (nl: 2~6.8) 7.2 mg/day (10.2 mg/g Cr) (if suppression: <2.5mg/g cr) 7.4 mg/day (if suppression : <64% of basal level) 17-KS (2.9~11.1 mg/day) 2.5 mg/day (nl: 2.9~11.1) 3.0 mg/day 2.8 mg/day 24hr u Cr 0.5 g/day (Cr: 1.0g/day) 0.7 g/day 0.3 g/day

IPSS (03-12-29) CRH(1ug/kg) injection Basal ACTH IPS:PPh>2→confirm CRH induced ACTH IPS:PPh ratio≥3→confirm pituitary ACTH secreting tumor ACTH (pg/ml) basal Post CRH 2분 5분 Rt 79.6 171.8 271.8 Lt 115.2 1600.3 2002.3 Pph 22.8 32.2 25.6

Final diagnosis Cushing’ disease Therapeutic plan TSA (04-01-15)

Brain (Sella) MRI(04.1.23)

IPSS (04-05-06) ACTH (pg/ml) 2 5 10 Rt 26.1 754 363 213 Lt 252 1916 2 5 10 Rt 26.1 754 363 213 Lt 252 1916 2527 1501 Pph 10.5 13.0 22.8 24.6

Clinical course Menstruation : IPSS 후 퇴원14일째인 04년 5월 20일 vaginal spot bleeding 이후로 현재까지 regular mensturation 있음 수술후 acne, knee joint pain 등 호전되었으나 04년 11월경부터 다시 acne, knee joint pain, face edema 등 나타나고 있음

Clinical course II (LAB) 2003.11.14 2004.1.13 2004.1.17 2004.3.17 2004.5.21 2004.7.28 2004.11.16 2004.12.23 2005.3.3 ACTH(P) (pg/dl) 33.7 17.4 45.2 44.5 10.4 13.2 20.4 32.7 18.8 S-Cor (ug/dl) 19.1 2.9 60 28.4 4.8 17 21.9 39.5 U-Cor (ug/day) 590.8 54.6 1560 687.3 33 424.4 331.5 04-01-15 TSA 04.11월 경 임상증상 악화

04-11월 acne, knee joit pain, face edema 04-01-15 TSA 04-05-07 IPSS

Clinical course III Recurrence? Unsuccessful surgery?