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Billiary Atresia

0603961

-


58
:

: 38w,
B: 3270 gr, M: 51,5cm, : 34cm
G6PD: (-)
25
: (-)

2y
4y




:
2-3 :
.


.
U/S
, o ,
, /
,
()

.

: , .

: 36,3o C

: 100/min

. : 95/64 mmHg

: 4480 gr (50 )

: 54cm (50 )

: 38cm (75 )

: ,
. , ,
.
: , . < 2,
.
: . , - .
: S1 , S2 , ,
~ 1-2/6, .
: , , , , ~5cm
, ~ 2cm .

: .

. : . .
: .

4/1/2013 ( 1 )

: WBC 23.400 (=21%, =69%, =9% S=1% BAS=0%),


Hgb=10gr/dl, HCT=28,7%, MCV=92,7 fl, MCH=32,3 pg, MCHC=34,8 g/dl,
RDW=17,7%, PLT=610.000, =3,29%, 19mm
: CRP=0,4 mg/dl , 86mg/dl, 24mg/dl,
TRPO=5,4 g/dl, Alb=3,67 g/dl, SGOT=407 IU/L, SGPT=311 IU/L, GT=435
U/L, ALP=694 IU/L, K=5,0 mmol/L, Na=132mmol/L, Ca()=9,5 mmol/L,
TBIL=15,11 mg/dl, DBIL=7,9 mg/dl, LDH 321 UI/L, CPK=80 U/L.
: PT=11,5 sec, INR=0,98, APTT=27,1 sec, FIB=211 mg/dl, Ddimers=637
ng/ML
: , , : 60-70 ,
0-1 . Glu(trace), bil(+++), ket(-), EBO=1025, pH=5,5, Pro(+), leuk (-), nit(-)
.
. : pH=7,41, pCO2 33mmHg, PO2 =90mmHg HCO3 =20,9
mmol/L, BEecf =3,7 mmol/L, BE(B)= 3,1 mmol/L, SatO2 =97%
K/ : .
6/1/2013 (2 )
Mayer (-) , :
.

7/1/2013 (3 )
:
o CMV IgG (33, pos>6), IgM(-),
o EBV IgG (38.44 pos>15) ,IgM (-),
o COXSACKIE IgG(-), IgM(-), IgA(-),
o ECHO IgG (-), IgM (-) ,IgA (-),
o HSV1/2 IgG(-) IgM(-),
o PLASMA IgG(-) IgM (-).
Io CMV :IgG 241,6 (pos>6) IgM 1,82 (pos>1).
: BsAg (-) Anti-HBs(-), Anti-HBc(-), HBeAg(-) Anti-
Hbe(-), Anti-HAV(-) Anti-HCV (-)
Coombs (-)
:: ,

8/1/2013 (4 )
PCR CMV: 1) 2) () .

U/S (5/1/2013)

,
,

( )
-

:
, .

4/1/2013
8/1/2013 .
:
, ,
, ,
.
u/s
PCR ( CMV) .
.

8/1/13


/ 14/1/2013
13-15/03/2013

05/04/2013 3
:
SGOT : 463 IU/I, SGPT: 394 IU/I , TBIL: 16,91 mg/dl, DBIL:13,09
mg/dl
06-09/04/2013

WBC:24.800(:29% ,:65%, M:4%), TKE :51mm
CRP:2,1mg/dl, SGOT:572 IU/I, SGPT:439 IU/I, ALP:386 IU/I
,GGT: 543 U/I ,DBIL:15,34 mg/dl, TBIL:20,99mg/dl
2 / :
/ :
:

H
, ,
:

1) ,

2)

3)

Kasper S. WangPediatrics. 2015 December ; 136(6): e1663e1669. doi:10.1542/peds.2015-3570



Jessica A. Zagory, MD et al Curr Opin Pediatr. 2015 June ; 27(3): 389394.


doi:10.1097/MOP.0000000000000214.


( Kasai),
2



Kasai
2

80%

Kasper S. WangPediatrics. 2015 December ; 136(6): e1663e1669. doi:10.1542/peds.2015-3570



:
(~90%)

. BASM (biliary atresia splenic
malformation) /
, :
(100%),
(50%),
(60%),

(40%),
(50%)

Davenport M J Pediatr. 2006 Sep;149(3):393-400


(//
Kasai)
: ,
( )

: ,
( ).

o :
( , )
o : ,
.

: ,
( )

, ( 73% ),
( 18%).
( 6%) ( 3%)
.
Barbara E. Wildhaber ISRN Surg. 2012; 2012: 132089


.
.
:
, 5/100.000 32/100.000
1/18.000
5 / 100.000 .
5.1 /100.000 .
6 /100.000
6.5-7.5 /100.000
7 /100.000
7.4 /100.000
10.1 / 100.000
32 /100.000

. Chardot C. Biliary atresia. Orphanet J Rare Dis. 2006; 1:28


Jessica A. Zagory, MD et al Curr Opin Pediatr. 2015 June ; 27(3): 389394.


doi:10.1097/MOP.0000000000000214.

:
CMV
REO
ROTA C




-

, ,

Jessica A. Zagory, MD et al Curr Opin Pediatr. 2015 June ; 27(3): 389394.


doi:10.1097/MOP.0000000000000214.

.
2 w - 2 months
(
)
-






(ADEK)

Kasper S. WangPediatrics. 2015 December ; 136(6): e1663e1669. doi:10.1542/peds.2015-3570


Davenport M J Pediatr. 2006 Sep;149(3):393-400
-
(>12
>21 )


>1mg /dl <5 mg/dl
>20% >5 mg/dl


Thomas Gtze1 et al Neonatal cholestasis differential diagnoses, current diagnostic procedures, and
treatment Frontiers in Pediatrics June 2015 doi: 10.3389/fped.2015.00043

237 studies
identified from
EMBASE and
Pubmed

17 studies
encompassing 1692
infants were
selected

-
1. / /
2.
3.
: WBC,Hb, MCV,,PLT,
: , CRP, LDH, , GT, AST,
ALT
: PT, aPTT, INR, D-dimers,
:
coombs
TORCH : CMV, Toxo, HSV, ,

: a1 , ,
, ,

Thomas Gtze1 , Holger Blessing1 et al frontiers of Pediatrics June 2015 doi: 10.3389/fped.2015.00043
-
4.
- :
,
: , , triangular cord sign

(, , )
,
MRCP : / / /
-
ERCP
99m-TC-Br-IDA :
-

U/S :
Triangular cord sign:
. >4mm

Larger hepatic arterial calibre:



/

Gallbladder ghost triad


I. < 19mm
II.
III.

DehghaniSM et al.Comparisonof differentdiagnosticmethodsininfantswithcholestasis. WorldJGastroenterol (2006) 12(36):58936. 23.


TakamizawaS etal.Canbiliary atresia be diagnosed by ultrasonography alone ?JPediatrSurg(2007)42(12):20936. doi:10.1016/j.jpedsurg.2007.08.032
Triangular cord sign

Sensitivity :
(73100%) and
specificity :
(98100%)

. Lee HJ, Lee SM, Park WH et-al. Objective criteria of triangular cord sign in biliary atresia on
US scans. Radiology. 2003;229 (2): 395-400. doi:10.1148/radiol.292020472
Triangular cord sign and Larger hepatic arterial calibre
Normal gallbladder


MRCP :
/

F. Ali, W. Akhter, M. Arshad Magnetic resonance cholangiopancreatography in diagnosis of biliary disordersin childrenSharing our experience J Pak Med Assoc. 2016 jan;66(1):27-9

ERCP:

: 8692% : 7394%
KeilR,SnajdaufJ etal.Diagnostic efficacyofERCPincholestaticinfantsandneonatesaretrospectivestudyon a largeseries. Endoscopy (2010) 42(2):1216. doi:10.1055/s-0029-1215372
PetersenC,MeierPN,SchneiderA,TurowskiC,PfisterED,MannsMP, et al.Endoscopicretrogradecholangiopancreaticographypriortoexplorative
laparotomyavoidsunnecessarysurgeryinpatientssuspectedforbiliaryatresia. J Hepatol (2009) 51(6):105560. doi:10.1016/j.jhep.2009.06.025

99m-TC-Br-IDA :

24 h
(83100%) (3380%)

FeldmanAG,SokolRJ.Neonatalcholestasis. Neoreviews (2013) 14(2):e6373. doi:10.1542/neo.14-2-e63


high sensitivity (83100%) but lacks specificity (3380%),
limiting its usefulness to discriminate between BA and other non-surgical
conditions
-
5.
gold standard
(11).
7998% .

,

,

6.
:

Thomas Gtze1 et al Neonatal cholestasis differential diagnoses, current diagnostic procedures, and
treatment Frontiers in Pediatrics June 2015 doi: 10.3389/fped.2015.00043

2 :
(-
- Kasai) /

Kasai hepato-portoenterostomy
(HPE)
1 1959 Morio Kasai

:



, 4050 cm, Roux

( ) 4765%

< 2.0 mg/dL 3


Jessica A. Zagory, MD et alRecent Advances in the Pathogenesis and Management of Biliary Atresia Curr
Opin Pediatr. 2015 June ; 27(3): 389394. doi:10.1097/MOP.0000000000000214.
(a) Complete biliary atresia with a shrunken, fibroticgall bladder (arrow) and biliary remnant (white
star) in a 2-month-old baby. Note the already cirrhotic aspect of the liver. (b) Hilar region after removal
of the biliary remnant (white star), surrounded by the hepatic artery's right branch (fine arrow) and the
portal vein's left branch (large arrow). (c) Final aspect of the Kasai hepato-porto-enterostomy with the
Roux-en-Y limb (black star) anastomosed to the hilar region.
Kasai hepato-portoenterostomy
(HPE)

1-2


.
( )

(,)

Kasper S. Wang et al Newborn Screening for Biliary Atresia Pediatrics. 2015 December; 136(6):e1663e1669.doi:10.1542/peds.2015-
3570.

/ Kasai

251
10
(73%) <60
(11%) >91
Ohi R, Nio M, Chiba T, Endo N, Goto M, Ibrahim M. Long-term follow-up after surgery for patients with biliary atresia. J Pediatr Surg. 1990;
25(4):442445

1
<30 <45
Marie-Odile Serinet, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia
screening. Pediatrics. 2009; 123(5):12801286.

, 159 National Institutes of Health


Kasai <75

Superina R, et al. Childhood Liver Disease Research and Education Network. The anatomic pattern of biliary atresia identified at time of
Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann
Surg. 2011; 254(4):577585
METHODS :
1986 2002 .
follow-up : 7

RESULTS :
743 , 695 asai
2-, 5-, 10-, 15- 57.1%, 37.9%, 32.4%,
28.5%,
Kasai 60
follow-up (2, 5, 10, 15 ),
(30, 3145, 4660, 61-75, 7690 )
<45
, 15 12,1%
Variations of 2-, 5-, 10- and 15-year SNL according to age at Kasai operation.

Marie-Odile Serinet et al. Pediatrics 2009;123:1280-1286

2009 by American Academy of Pediatrics


Comparison of the 15-year SNL between patients with BA having undergone a Kasai operation
before and after 45 days of life.

Marie-Odile Serinet et al. Pediatrics 2009;123:1280-1286

2009 by American Academy of Pediatrics


screening
2 screening tests :
a)
Harpavat et al
100% 96 ,

96

screening ?

Harpavat S, Finegold MJ, Karpen SJ. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics. 2011; 128(6)
screening
b)
Taiwan 2004.


Taiwan screening :
89.7%, 99.9%
28.6% 99.9%


HPE <60 66 % 49%
3 57% 31.5% controls

2 ,
cost-effectivness . 2 :
A

Chen SM,, et al. Screening for biliary atresia by infant stool color card in Taiwan. Pediatrics. 2006; 117(4):11471154.
Lien TH et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology (Baltimore, Md). 2011; 53(1):202208
Schreiber RA, Masucci L, Kaczorowski J, et al. Home-based screening for biliary atresia using infant stool colour cards: A large-scale prospective cohort study and cost-effectiveness
analysis. Journal of medical screening. 2014
asai

: 60% (
3,6 , 2 )
: Klebsiella pneumonia,
Enterococcus, Escherichia coli Pseudomonas aeruginosa.
Lactobacillus


2/3


98% >5 Kasai

Jessica A. Zagory, MD et alRecent Advances in the Pathogenesis and Management of Biliary Atresia Curr
Opin Pediatr. 2015 June ; 27(3): 389394. doi:10.1097/MOP.0000000000000214.

50%


Kasai
( ) /
,

:
1) :
( 2 3)
( 2, 3, 4)
.
2) :
.
2 3.
10 %

: , ,

Davenport M J Pediatr. 2006 Sep;149(3):393-400


Whats new
H BA
?




( )

IgG

:

,

1( ICAM-1)

CD4+ and CD8+ T-cell
T-

, FDA
1/2a
Kasai

Jessica A. Zagory, MD et alRecent Advances in the Pathogenesis and Management of Biliary Atresia Curr Opin Pediatr. 2015 June ; 27(3): 389394.doi:10.1097/MOP.0000000000000214.
Mechanisms of action of high dose IgG in murine BA
(1) Inhibition of VCAM-1 expression on vascular endothelium, resulting in decreased portal tract extravasation of T cells and
monocytes. (2) Inhibition of Th1 cell activation, leading to decreased IFN- associated macrophage stimulation, IL-2
associated T cell expansion, cytotoxic TNF- production and CD4+ T cell-mediated stimulation of cytotoxic CD8+ T cells. (3)
Treg expansion resulting in T cell inhibition

Erika K. Fenner et al High Dose IgG Therapy Mitigates Bile Duct Targeted Inflammation and Obstruction in a Mouse Model of Biliary Atresia
Pediatr Res. 2014 July ; 76(1): 7280. doi:10.1038/pr.2014.46.