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Case Review Division of Gastroenterology Department of Pediatrics, YUMC R1 이 순 민.

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Presentation on theme: "Case Review Division of Gastroenterology Department of Pediatrics, YUMC R1 이 순 민."— Presentation transcript:

1 Case Review Division of Gastroenterology Department of Pediatrics, YUMC R1 이 순 민

2 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: none FHx: only 1 st male baby no known DM/HTN/pul Tbc/hepatitis

3 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

4 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(-/-)

5 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

6 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

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

8 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

9 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 (-)

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

11 HD1HD7HD16 OP HD5HD3 sonoMRCP

12 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. Operation

13 Medication Solu-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

14 MRCP

15

16 Abd. U/S

17 Final Diagnosis Biliary Atresia


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