Case Presentation 2007313075 Chief Complaint 10 moF Mild

Case Presentation 성균관대학교 의과대학 2007313075 손의영

Chief Complaint � 10 mo/F �Mild fever �Onset : 1 week ago �Associated Sx : general weakness

Present Illness � 10. 15(생후 2 mo) � 10. 25 � 11. 03. 26 -04. 05 (생후 7 mo) aggravated jaundice, whitish stool로 본원 OPD 방문 Dx. Biliary atesia Kasai OP 시행 cholangitis로 입원 후 치료받음 � 내원 1주전부터 37. 4 -37. 8정도의 mild fever � 내원 당일부터 처지는 모습 보여 본원 외래 통해 R/O cholangitis로 입원

Birth History �Full-term baby �출생 시 체중 : 2. 86 Kg (25~50 �분만형태 : Vaginal delivery percentile)

Vaccination History • 종류 BCG HBV DPT IPV Hib Pn HAV rota 1차 ● ● ● ● 2차 ● ● ● ● 3차 ● ● ● 추가1 추가2 추가3

Review of System � Activity : decreased � Oral intake : decreased � � � � � General weakness ( +) Weight change ( －) Fever ( ＋) Headache ( －) Cough ( －) Rhinorrhea ( －) Chest pain ( －) Palpitation ( －) Orthopnea ( －) Easy fatigability ( －) Chill ( －) Sputum ( －) Sore throat ( －) Discomfort ( －) Dyspnea ( －) Hemoptysis ( －)

Review of System � � � � � Abdominal pain / discomfort ( －/ - ) Anorexia ( －) N/V ( －/ - ) Diarrhea ( －) Constipation ( －) Hematemesis ( －) Melena ( －) Dysuria / hematuria / frequency ( - / - ) Hesitancy ( －) Nocturia ( －) Urgency ( －) Oliguria ( －) Myalgia ( －) Arthralgia ( －) Joint swelling ( －) Vaginal discharge ( －) Bleeding ( －)

Physical Exam � Vital Sign � 혈압 108/76 mm. Hg � 호흡 40 /min � 몸무게 8. 1 kg (25 percentile) 맥박 135. 0 /min 체온 36. 9℃ 신장 68. 0 cm (10~25 percentile) � General appearance : Chronic ill looking � Activity : moderate � Mental status : Alert � HEENT � Facial asymmetry ( －) � Conjunctival injection ( －) � TM ( －) � PI ( －) � Palpable LN ( －) Conjunctiva ( Not anemic) Sclera ( Icteric) PTH ( －)

Physical Exam � Chest wall expansion ( Symmetric) � Heart beat ( Regular) � Murmur ( －) � Breathing sound ( Vesicular) � Abdomen � Soft Distended � Tenderness : ( －) � Rebound tenderness ( －) � Bowel sound ( Normoactive) � not palpable liver, spleen � Back and Extremity � Pitting edema ( －) � Skin lesion ( －)

Lab – CBC � WBC � RBC � Hb � Hct (2011 -07 -01) ▲ 13. 16 x 10³/μL (seg. 55%) ▼ 3. 16 x 10³/μL ▼ 6. 5 g/d. L ▼ 22. 0% � PLT 341, 000/μL � Protein � Albumin � Globulin 6. 0 g/㎗ ▼ 3. 1 g/㎗ 2. 9 g/㎗ � Cholesterol 195 mg/d. L � Bilirubin(total) ▲ 128 mg/d. L � Bilirubin(direct) ▲ 8. 7 mg/d. L � AST ▲ 163 U/l � ALT ▲ 64 U/l � ALP ▲ 943 U/l � GGT ▲ 244 U/L

Lab – CBC (2) � Glucose(fasting) ▲ 114 � BUN � Cr � BUN/Cr mg/dl ▼ 7. 6 mg/dl ▼ 0. 23 mg/dl ▲ 33. 0 � Estimated GFR 100 m. L/min � CRP ▲ 5. 22 mg/d. L � Na �K � Cl ▼ 134 mmol/ℓ 4. 5 mmol/ℓ 104 mmol/ℓ � Uric acid � Ca �P ▼ 2. 0 mg/d. L 8. 5 mg/d. L 4. 0 mg/d. L

Lab – Urinalysis �Color �Turbidity �Specific Gravity �p. H �Albumin �Glucose �Ketones �Bilirubin �Blood �Urobilinogen �Nitrite �Leukocyte Esterase Amber Clear ▼ 1. 007 6. 5 neg norm – neg 3 ++ 10 TR norm – neg 100 ++

Abdomen USG � CONCLUSION: � Interval progression of ascites. � Suggestive of biliary cirrhosis with portal hypertension leading to reversed portal flow and left gastric varix. � No interval change of the presumed biloma in right lobe of the liver.

Problem List #1. known biliary atresia(10. 15) s/p kasai op. (10. 25) #2. mild fever #3. decreased activity

Assessment #1. known biliary atresia(10. 15) s/p kasai op. (10. 25) #2. known otomycosis[Aspergillus species] #3. R/O cholangitis #4. R/O Infected choledochal cysts #5. R/O Cholecystitis #6. R/O Mirizzi syndrome

Plan �치료계획 �퇴원계획 Ampicillin & sulbactam IV unasyn, cefotaxime start Cholangitis 확진 시 항생제 3주 지속 Complication monitoring LT 시기는 12개월 예방접종완료 후로 예정 fever control & 임상 양상 호전 시

신생아 황달 Sungkyunkwang Univ.

담도 폐쇄 (Biliary atresia) � 진단 � Hepatobiliary scintigraphy : HIDA, DISIDA � Liver Bx : 간의 섬유화, 간내 담도의 증식, 간내 담관 내 담즙 정체 � 초음파 검사 � Intraoperative cholangiogram (수술적 담도 조영술) � Magnetic resonance cholangiopancreatography � 조기 감별 진단이 중요 : 치료가 빠를 수록 담즙 배출이 잘 됨

References �Nelson textbook of pediatrics, 18 th Ed. � 102. 3 Jaundice and Hyperbilirubinemia in the Newborn �홍창의 소아과학, 안효섭 편저, 9 th Ed. � Ch 13. 소화기 질환