Case Review Division of Gastroenterology Department of Pediatrics

Case Review Division of Gastroenterology Department of Pediatrics, YUMC R 1 이 순 민

E. ○ M/ 2/12(7 wks) C. C: evaluation for acholic stool & jaundice Duration: 1 months ago PHx: NSVD at IUP 40 wks, BW 3400 g No neonatal asphyxia No neonatal hyperbilirubinemia Vaccination Hx: Done as scheduled Admisson Hx: none FHx: only 1 st male baby no known DM/HTN/pul Tbc/hepatitis

Present illness 본 2개월된 남자환아는 내원 1달전부터의 yellowish skin color, acholic stool 보여 몽고 local clinic 내원 후 R/O obstructive jaundice imp 하에 further tx위해 내원함

ROS Poor oral intake(-) Lethargy(-) Fever/chill(-/-) Headache/Dizziness(-/-) Cough/Sputum/Rhinorrhea(-/-/-) Anorexia/Nausea/Vomiting(-/-/-) Constipation/Diarrhea(-/-) acholic stool(+) Chest pain/Chest discomfort(-/-) Cyanosis/Respiratory difficulty(-/-) Abd. Pain(-) Oliguria/Frequency(-/-) Hematuria/Dysuria(-/-)

Physical examination BP 94/50 ( 50 -75 P ) PR 130/min G/A Not so ill looking appearnace Alert mental status Skin Warm & dry No abnormal skin rash c yellowish skin color HEENT Not pale conjunctiva Icteric sclera No dried lips & tongue No throat injection Neck Supple Not palpable cervical LN

Chest Symmetric expansion without retraction CBS without rale RHB without murmur No thrill and heaving Abd. Soft & flat Normoactive bowel sound No organomegaly Back & Ext. No LOM No CVA tenderness No pitting edema

Impression Neonatal cholestasis R/O biliary atresia R/O Neonatal hepatitis

Laboratory finding CBC 13040/11. 5/33/679 k(25/61/7) Electrolyte 136/5. 5/108/18(100) Ca/P 10. 4/4. 6 Uric acid 2. 4 BUN/Cr 4. 8/0. 4 T. protein/albumin 6. 4/3. 8 Cholesterol 202 OT/PT 149/107 ALP 1239 T. bil/D. bil 9. 3/8. 6 PT/PTT 100%/29 sec Blood culture no growth

U/A S. G 1. 010 p. H 5. 0 WBC - RBC - protein (-) Urobilinogen(-) Viral study: HBs Ag (-) HBc Ab(+) HBs Ab(-) Anti-HAV Ig M(-) EBV EA Ig M(-) Mycoplasma Ab(-) Toxoplasma Ab(-) VDRL(-) Rubella Ab(-) CMV Ig M (-) HSV Ig M (-)

Progression * HD #1 CXR, F/U abd * HD#3 Abd sono * HD#5 MRCP * HD#7 Operation(8 wks)

HD 1 HD 3 sono HD 5 MRCP HD 7 HD 16 OP

Operation Kasai operation (Resection of remnant biliary tract, Roux-en-Y portojejunostomy) Bx; * Liver, porta hepatitis, biopsy Fibrous septa containing ductular structures, measuring 40 um in the maximal dimension * Gallbladder, cholecystectomy Chronic nonspecific inflammation, mild * Liver, biopsy Periportal fibrosis with marked autolytic change.

Medication Solu-medrol 20 mg(POD #4, 5, 6) Solu-medrol 10 mg(POD #7) Solu-medol 5 mg(POD #8) Solu-medrol 2. 5 mg(POD #9) Ursa 100 mg Phenobarbital 30 mg * Discharge med Delta-cortef 10 mg * 4 days Ursa 100 mg Phenobarbital 30 mg Cefaclor 3 ml

MRCP

Abd. U/S

Final Diagnosis Biliary Atresia