1. Polygrandular Autoimmune Syndrome 내분비대사 내과 R1 권성진

2. 11623653 이 O 화 (F/43) OPD : 2006-4-10 Chief complaint - Black pigmentation on skin & oral mucus Chief complaint - Black pigmentation on skin & oral mucus Present illness Present illness - F/43, 특이병력 없던 환자로 2005. 6. 27 갑상선 검사에서 - F/43, 특이병력 없던 환자로 2005. 6. 27 갑상선 검사에서 hypothyriodism 으로 진단 후 Levothyroxin medication 하며 hypothyriodism 으로 진단 후 Levothyroxin medication 하며 F/U 중, 수개월전부터 생긴 피부와 입안의 black pigmentation, F/U 중, 수개월전부터 생긴 피부와 입안의 black pigmentation, general weakness 생겨 내분비내과 외래 방문함 general weakness 생겨 내분비내과 외래 방문함

3.  Past medical history DM / HTN / Hepatitis / TB (-/-/-/-) OP Hx : tubal ligation, 20 년전 Hypothyroidism(+) : 2005, 6 월 Levothyroxine 0.1mg qd Personal history alcohol (-) Personal history alcohol (-)smoking(-)  Family history HTN Lung Ca Thyroid Ca →OP

4. Review of systems General : fatigue (+) fever (-) chills (-) General : fatigue (+) fever (-) chills (-) Skin : pigmentation (+); oral mucosa, extremity, hand crease Skin : pigmentation (+); oral mucosa, extremity, hand crease axillary hair loss(+); 1 Y ago axillary hair loss(+); 1 Y ago Head / Neck : headache (-) hoarseness (-) sore thraot (-) pain(-) Head / Neck : headache (-) hoarseness (-) sore thraot (-) pain(-) Respiratory : cough (-) dyspnea (-) hemoptysis (-) Respiratory : cough (-) dyspnea (-) hemoptysis (-) GI : A/N/V/D/C (-/-/-/-/-) diarrhea (-) constipation(-) GI : A/N/V/D/C (-/-/-/-/-) diarrhea (-) constipation(-) Gynecological: amenorrhea(+); 3 Y ago Gynecological: amenorrhea(+); 3 Y ago Musculoskeletal : pain(-) cramps(-) weakness(+) swelling(-) Musculoskeletal : pain(-) cramps(-) weakness(+) swelling(-) Metabolic : weight loss(-) hair change(+) Metabolic : weight loss(-) hair change(+) temperature tolerance(-)

5. Physical examination (1) Vital sign 110/70 mmHg – 84/min – 20/min – 36.4 °C Vital sign 110/70 mmHg – 84/min – 20/min – 36.4 °C General appearance - Alert consciousness mentality - Chronic ill looking appearance General appearance - Alert consciousness mentality - Chronic ill looking appearance Skin - No rash Skin - No rash - Pigmentation at both buccal side, gum, face, both upper and lower extremity, crease of palm and sole lower extremity, crease of palm and sole Eyes and ENT - Isocoric pupils with pupilary light reflex (++/++) - Whitish sclera - Pinkish conjunctivae Eyes and ENT - Isocoric pupils with pupilary light reflex (++/++) - Whitish sclera - Pinkish conjunctivae

6. Physical examination (2) Head & Neck Head & Neck - No neck vein engorgement (-/-) - No neck vein engorgement (-/-) - No cervical lymph node enlargement - No cervical lymph node enlargement Chest - Clear breath sounds without crackle or wheezing - Regular heart beat without murmur Chest - Clear breath sounds without crackle or wheezing - Regular heart beat without murmur Abdomen - Soft and flat abdomen - Normoactive bowel soud - No tenderness or rebound tenderness Abdomen - Soft and flat abdomen - Normoactive bowel soud - No tenderness or rebound tenderness

10. Lab results (06.4.10) CBC/DC 5,910/mm3 – 12.0 g/dl – 35.3 % - 189k ( seg. 54.1 % ) 5,910/mm3 – 12.0 g/dl – 35.3 % - 189k ( seg. 54.1 % ) MCV : 87.7 fl (80~96) MCH : 29.8 pg ( 27~33) MCV : 87.7 fl (80~96) MCH : 29.8 pg ( 27~33)Chemistry T/D–bilirubin0.68/ 0.19 mg/dL T/D–bilirubin0.68/ 0.19 mg/dL AST / ALT33 / 15 U/L AST / ALT33 / 15 U/L T-protein / albumin 6.1 / 3.3 g/dL T-protein / albumin 6.1 / 3.3 g/dL BUN / Cr5 / 0.5 mg/dL BUN / Cr5 / 0.5 mg/dL Na/ K /Cl140 / 3.9 / 105 mmol/L Na/ K /Cl140 / 3.9 / 105 mmol/L Ca/P 8.7 / 3.8 mg/dL Ca/P 8.7 / 3.8 mg/dL Glucose 104 mg/dl Glucose 104 mg/dl T-cholesterol /TG 144 / 128 mg/dl T-cholesterol /TG 144 / 128 mg/dl HDL/LDL 19/119 mg/dl HDL/LDL 19/119 mg/dl

11. Follow up of TFT 05.06.2706.04.1006.05.1506.06.27 T3 (80~200ng/dl) FT4 (0.77~1.94ng/dl) 0.940.921.09 TG-Ab (<100IU/ml) <50 TMS (<8 UG/ml) 26 TBII (<10%) 0.1 TSH (0.3~4.0 u/nl) 16.5218.3616.10 05-6-30 Levothy -roxine 0.1mg qd

12. Neck & Thyroid Sono (2006-5-9)

14. Lab results (06.6.29) CBC/DC 7,340/mm3 – 11.6 g/dl – 35.2 % - 202k ( seg. 53.9 % ) 7,340/mm3 – 11.6 g/dl – 35.2 % - 202k ( seg. 53.9 % ) MCV : 90.9 fl (80~96) MCH : 30.0 pg ( 27~33) MCV : 90.9 fl (80~96) MCH : 30.0 pg ( 27~33)Chemistry T/D–bilirubin0.47/ 0.02 mg/dL T/D–bilirubin0.47/ 0.02 mg/dL AST / ALT24 / 14 U/L AST / ALT24 / 14 U/L T-protein / albumin 6.2 / 3.5 g/dL T-protein / albumin 6.2 / 3.5 g/dL BUN / Cr9 / 0.6 mg/dL BUN / Cr9 / 0.6 mg/dL Na/ K /Cl140 / 3.9 / 105 mmol/L Na/ K /Cl140 / 3.9 / 105 mmol/L Ca/P 8.7 / 3.8 mg/dL Ca/P 8.7 / 3.8 mg/dL Glucose 160 mg/dl Glucose 160 mg/dl T-cholesterol /TG 187 / 68 mg/dl T-cholesterol /TG 187 / 68 mg/dl HDL/LDL 20/152 mg/dl HDL/LDL 20/152 mg/dl

15. Chest PA

16. 부신기능검사 02.10.0806.04.1006.07.0606.07.1206.07.13 ACTH (10~60 pg/ml) 632.61160.3? Cortisol (S) (3.5~25 ug/dl) 1.72.21.2 Cortisol (U) (75~270 ug/m 2 /day) Un- detected 682.6 Creatinine (U) (1.0~1.15 g/day) 0.7 Aldosterone (50~194 pg/ml) 25 PRA (0.8~1.36 ng/ml/hr) ? 06-4-10 PDL 7.5mg

17. Abd CT (2006-6-20)

18. OGTT (06.6.29) 0min30min60min90min120min Glucose(mg/dl)101174136131107 Insulin10.295.6 C-pep1.25.2 Insulinogenic index : 1.16 HOMA – IR : 2.54

19. FSH (1.7~8.5 mlU/ml) 16.6 LH (2~15.4 mlU/ml) 25.4 PRL (0.2~9.4 ng/ml) 5.5 E2 (19~255 pg/ml) 50.4

20. Final Diagnosis and plan #1. Hypothyroidism - Levothyroxine 0.1mg qd supply - Levothyroxine 0.1mg qd supply #2. Primary adrenal insufficiency can not r/o polygrandular autoimmune syndrome can not r/o polygrandular autoimmune syndrome - Prednisolone 7.5mg + 5mg qd supply