Case Review

Division of GastroenterologyDepartment of Pediatrics, YUMC R1 이 순 민

E.○ M/ 2/12(7wks) C.C: evaluation for acholic stool & jaundice Duration: 1 months ago PHx: NSVD at IUP 40wks, BW 3400g No neonatal asphyxia No neonatal hyperbilirubinemia Vaccination Hx: Done as scheduled Admisson Hx: noneFHx: only 1st male baby no known DM/HTN/pul Tbc/hepatitis

Present illness

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

ROSPoor 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 ( 50-75P ) 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 13040/11.5/33/679k(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%/29sec

Blood culture no growth

U/A S.G 1.010 pH 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(8wks)

HD1 HD7 HD16

OP

HD5

HD3

sono MRCP

0123456789

10

HD1 HD2 HD3 HD7 HD8 HD12 HD15

T.bil

D.bil

Kasai operation (Resection of remnant biliary tract, Roux-en-Y portojejunostomy) Bx; * Liver, porta hepatitis, biopsy Fibrous septa containing ductular structures, measuri

ng 40 um in the maximal dimension * Gallbladder, cholecystectomy

Chronic nonspecific inflammation, mild * Liver, biopsy Periportal fibrosis with marked autolytic change.

Operation

MedicationSolu-medrol 20mg(POD #4,5,6)Solu-medrol 10mg(POD #7)Solu-medol 5mg(POD #8)Solu-medrol 2.5mg(POD #9)Ursa 100mg Phenobarbital 30mg* Discharge med Delta-cortef 10mg * 4days Ursa 100mg Phenobarbital 30mg Cefaclor 3ml

MRCP

Abd. U/S

Final Diagnosis

Biliary Atresia