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

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

Case Review
Division of Gastroenterology
Department 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: none
FHx: only 1st 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 ( 50-75P )
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
HD3
sono
10
9
8
7
6
5
4
3
2
1
0
HD5
HD7
MRCP
HD16
OP
T.bil
D.bil
HD1
HD2
HD3
HD7
HD8
HD12
HD15
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 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