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Effects of the Infant Stool Color Card Screening

Program on 5-Year Outcome of Biliary


Atresia in Taiwan
Tien-Hau Lien,1 Mei-Hwei Chang,1 Jia-Feng Wu,1 Huey-Ling Chen,1 Hung-Chang Lee,2 An-Chyi Chen,3
Mao-Meng Tiao,4 Tzee-Chung Wu,5 Yao-Jong Yang,6 Chieh-Chung Lin,7 Ming-Wei Lai,8 Hong-Yuan Hsu,1
Yen-Hsuan Ni,1 and the Taiwan Infant Stool Color Card Study Group*

In Taiwan, a screening system using an infant stool color card to promote the early diagno-
sis of biliary atresia (BA) was established in 2002. This study aimed to investigate the
5-year outcome of BA before and after using the screening program. BA patients were
divided into three cohorts according to their birth dates. The patients in cohort A (n 5 89)
were born before the stool card screening program (1990-2000); those in cohort B (n 5
28) were screened by the stool card regional screening program (2002-2003); and those in
cohort C (n 5 74) were screened by the stool card universal screening program (2004-
2005). The relative odds ratios were computed using logistic regression to compare the dif-
ferent factors affecting survival time. The rate of age at Kasai operation <60 days was
49.4% and 65.7% in cohorts A and B1C, respectively (P 5 0.02). The jaundice-free (total se-
rum bilirubin <2.0 mg/dL) rate 3 months after surgery was 34.8% and 60.8% in cohorts A
and B1C, respectively (P < 0.001). The 3-year jaundice-free survival rate with native liver was
31.5% in cohort A and 56.9% in cohort B1C (P < 0.001), whereas the 3-year overall survival
rates were 64.0% and 89.2%, respectively (P < 0.001). The 5-year jaundice-free survival rate
with native liver was 27.3% in cohort A and 64.3% in cohort B (P < 0.001), and the 5-year
overall survival rates were 55.7% and 89.3%, respectively (P < 0.001). Conclusion: The stool
color card screening program for BA allows for earlier Kasai operation, which increases the
jaundice-free rate at 3 months postsurgery. With higher surgical success rates, the 3- and 5-year
outcome of BA patients in Taiwan improves remarkably. (HEPATOLOGY 2011;53:202-208)

B
iliary atresia (BA) is an inflammatory, progres- ease such that it has become the first-line treatment. The
sive fibro-sclerosing cholangiopathy of infancy Kasai operation can restore bile flow through a recon-
that variably affects both the extrahepatic and structed hepatic portoenterostomy to a jejunal loop.
intrahepatic bile ducts,1,2 resulting in the destruction Once the cholestasis progresses and/or complications of
and obstruction of the biliary tract.2-4 If untreated, BA liver cirrhosis occur, liver transplantation remains the
progresses to cirrhosis with portal hypertension and salvage way for BA. Although ongoing cholestasis, which
liver failure leading to death within 2 to 3 years. Since further aggravates liver cirrhosis, is present in most BA
the Kasai operation was first used for BA in 1959, children,5 a successful Kasai operation may still delay or
there have been encouraging results in treating this dis- even decrease the need for liver transplantation.

Abbreviations: BA, biliary atresia; CI, confidence interval; OR, odds ratio.
From the 1Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; the 2Department of Pediatrics, Mackay Memorial Hospital, Taipei,
Taiwan; the 3Department of Pediatrics, China Medical University Hospital, Taichung, Taiwan; the 4Department of Pediatrics, Chang Gung Memorial Hospital-
Kaohsiung Medical Center, Kaohsiung, Taiwan; the 5Department of Pediatrics, Taipei Veterans General Hospital & National Yang Ming University, Taipei, Taiwan;
the 6Department of Pediatrics, National Cheng Kung University Hospital, Tainan, Taiwan; the 7Department of Pediatrics, Taichung Veterans General Hospital,
Taichung, Taiwan; and the 8Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan.
Received May 4, 2010; accepted September 22, 2010.
Supported by grants of the Bureau of Health Promotion of the Department of Health, Taiwan (Project No. 9804004A).
The members of the Taiwan Infant Stool Color Card Study Group are: Wan-Hsin Wen (Cardinal Tien Hospital), Chun-Hsien Yu (Taipei City Hospital,
Branch for Women and Children), I-Hsien Lee (Changhua Christian Hospital), Lung-Huang Lin (Cathay General Hospital), Wen-Terng Lin (En Chu Kong
Hospital), Hsiang-Hung Shih (Kaohsiung Medical University), Pi-Feng Chang (Far Eastern Memorial Hospital), Ching-Feng Huang (Tri-Service General
Hospital), I-Fei Huang (Kaohsiung Veterans General Hospital), Chun-Yan Yeung (Mackay Memorial Hospital, Tamshui Branch), Shan-Ming Chen (Chung Shan
Medical University Hospital), and Te-Kuei Hsieh (Hsin Chu General Hospital, Department of Health, Executive Yuan).

202
HEPATOLOGY, Vol. 53, No. 1, 2011 LIEN ET AL. 203

It is generally accepted that the Kasai operation is were divided into three cohorts by their birth date.
more successful in children when performed earlier The historical control cohort was derived from the 96
than 60 days of age.6,7 The reported successful bile cases diagnosed as BA at the National Taiwan Univer-
flow rates were 91%, 56%, 38%, and 17% in sity Hospital from January 1990 to December 2000.
patients receiving an operation before 60 days of age, Five patients who did not receive Kasai operation and
at 61-70 days of age, at 71-90 days of age, and two patients who underwent Kasai operation but were
beyond 90 days of age, respectively.8 However, early not followed up for at least 3 years postoperatively
identification and timely surgery, which are crucial were excluded. The remaining 89 patients became
for better prognosis, remain challenging. cohort A. All of these patients were followed up for at
In Taiwan, a pilot regional study using the infant least 5 years, except one patient who was followed up
stool color card to increase the efficacy of early identifi- for only 3 years postoperatively. Cohort A represented
cation of BA was started in 2002. Universal screening patients born before the stool card screening program.
for BA using the infant stool color card was launched There were 29 BA patients born between 2002 and
in 2004. This is the first nationwide screening program 2003. One patient who did not receive Kasai opera-
for BA using an infant stool color card. The present tion was excluded. The other 28 patients who were
study aimed to compare the outcome of the BA patients followed up for at least 5 years postoperatively were
after Kasai operation before versus after the launch of enrolled in cohort B, which represented the period of
the infant stool color card screening program. the regional pilot study of the stool card screening pro-
gram in Taiwan.
Seventy-five BA patients were born between 2004
Patients and Methods and 2005. After excluding one patient without Kasai
Infant Stool Color Card. An infant stool color card operation, the 74 patients who were followed up for at
was designed with six photographs of different colored least 3 years postoperatively were enrolled in cohort C, which
stool samples from Taiwanese infants. Three colors on represented the nationwide screening data in Taiwan. Cohort
this card were labeled abnormal (clay-colored, pale yel- BþC was the merged data of cohorts B and C and repre-
lowish, and light yellowish), whereas the other three sented the era of the stool color card screening program.
were labeled normal (yellowish, brown, and greenish). Statistical Methods and Data Analysis. Statistical
Telephone and fax numbers for consultation were also analyses were performed using Stata software (Stata-
printed on this card, and parents, guardians, and med- Corp LP, College Station, TX). A chi-square test was
ical personnel were instructed to inform the stool card used to compare categorical variables, including age at
registry center if abnormal stool colors were noticed. Kasai operation <60 days, jaundice-free rates at 3
There are professional personnel in the stool card cen- months after Kasai operation, 3- and 5-year survival rates
ter who respond to every related phone call or fax with native liver, 3- and 5-year jaundice-free survival rates
within 24 hours. Instructions and follow-up were given with native liver, and 3-year and 5-year overall survival
to every reported case. In 2002, 47,180 newborns rates between different cohorts. Overall survival included
from 49 hospitals and clinics in northern and central those patients who survived with either their native liver
Taiwan were enrolled. In 2003, the range of coopera- or a transplanted liver. Jaundice-free was defined as total
tion extended to southern and eastern Taiwan, and serum bilirubin <2.0 mg/dL (34 lmol/L).
72,793 newborns from 96 hospitals were enrolled. In Quality outcome was defined as jaundice-free sur-
2004, the universal stool color screening program was vival with native liver. All survival time was calculated
launched, and the stool color card was integrated into after the date of the Kasai operation. Relative odds
the child health booklet. All neonates born in Taiwan ratios were computed using logistic regression models
participated in the screening program since then. to compare the different factors affecting survival time.
Patients. All of the patients had a diagnosis of BA The Kaplan-Meier method and a log-rank test were
made using clinical data, biochemical data, imaging also used to assess factors affecting survival. P < 0.05
data, surgical findings, and liver histology. The patients was considered statistically significant.

Address reprint requests to: Mei-Hwei Chang, M.D., Department of Pediatrics, National Taiwan University Hospital, No. 7, Chung-Shan S. Rd., Taipei, 100,
Taiwan. E-mail: changmh@ntu.edu.tw; Fax: (886)-2-23114592.
Copyright VC 2010 by the American Association for the Study of Liver Diseases.

View this article online at wileyonlinelibrary.com.


DOI 10.1002/hep.24023
Potential conflict of interest: Nothing to report.
Additional Supporting Information may be found in the online version of this article.
204 LIEN ET AL. HEPATOLOGY, January 2011

From 1990 until now, there was no systemic change Table 1. General Characteristics and Outcomes in Cohort A
in post-Kasai operation care except for the concept of versus Cohort B1C
prophylactic antibiotics use in Taiwan. Since 1997, Cohort A Cohort B1C
most patients have been prescribed with prophylactic (n 5 89)* (n 5 28 1 74)y P Value

antibiotics after operation. The data of the use of pro- Male sex 38 (42.7) 56 (54.9) 0.09
phylactic antibiotics are collected by chart review, and Age 60 days at 44 (49.4) 67 (65.7) 0.02
Kasai operation
this possible confounding factor is taken into consider- Jaundice-free‡ 31 (34.8) 62 (60.8) <0.001
ation for analyses. The regimen of prophylactic antibi- 3 months after
otics is trimethoprim-sulfamethoxazole (TMP-SMZ, 7 Kasai operation
Survival
days per week) or neomycin (4 days per week). Our 3-year SNL 46 (51.7) 63 (61.8) 0.16
previous study reveals the superior effect of using pro- 3-year JFSNL 28 (31.5) 58 (56.9) <0.001
phylactic antibiotic versus not using it, and the equal 3-year OS 57 (64.0) 91 (89.2) <0.001
5-year SNL 33 (37.5)§ 18 (64.3)|| 0.01
effect for the prophylaxis of cholangitis between the
5-year JFSNL 24 (27.3)§ 18 (64.3)|| <0.001
two antibiotic regimens.9 5-year OS 49 (55.7)§ 25 (89.3)|| <0.001

Data are expressed as No. (%).


Results Abbreviations: JFSNL, jaundice-free survival with native liver, OS, overall sur-
vival; SNL, survival with native liver.
Comparisons of Cohort A versus Cohort *Includes patients born before the institution of the stool color card screen-
ing program (1990-2000).
(B1C). There was no significant difference in the sex †The data of cohort B and cohort C were merged to represent the outcomes
distribution between cohort A and cohort (BþC). The in the era of the stool color card screening program. Cohort B (n ¼ 28)
rates of Kasai operation performed before 60 days of includes patients born during the regional study of the stool color card screen-
ing program (2002-2003). Cohort C (n ¼ 74) includes patients born after the
age were 49.4% in cohort A and 65.7% in cohort launch of the nationwide study of the stool color card screening program
BþC (P ¼ 0.02). At 3 months after Kasai operation, (2004-2005).
the jaundice-free rate was significantly higher in cohort ‡Total serum bilirubin <2.0 mg/dL.
BþC than in cohort A (60.8% versus 34.8%; P < §One patient was lost to follow-up. Only 88 patients completed the 5-year
follow-up.
0.001). The 3-year survival rates with native liver in ||
Only cohort B (n ¼ 28) was followed up for more than 5 years and
cohort A and cohort BþC were 51.7% and 61.8%, calculated in this data.
respectively. The 3-year jaundice-free survival rate with
native liver was significantly higher in cohort BþC card screening (Supplement Table 1). The results are
than in cohort A (56.9% versus 31.5%; P < 0.001). similar to the comparisons of cohort A and cohort
The 3-year overall survival rate was 64.0% in cohort A BþC (Table 1).
and 89.2% in cohort BþC (P < 0.001). Jaundice-Free at 3 Months After Kasai Opera-
The 5-year follow-up was not yet finished in cohort tion. Logistic regression analyses revealed that patients
C; therefore, only cohort B was compared with cohort who underwent Kasai operation before 60 days of age
A in the analyses of 5-year survival time. The 5-year had a higher jaundice-free rate at 3 months after sur-
survival rates with native liver in cohorts A and B were gery compared with those who underwent surgery after
37.5% and 64.3%, respectively (P ¼ 0.01). The 60 days of age (odds ratio [OR] 2.62; P ¼ 0.001)
5-year jaundice-free survival rate with native liver was (Table 2). Patients born in the stool card screening era
significantly higher in cohort B than in cohort A (cohort BþC) had a significantly higher jaundice-free
(64.3% versus 27.3%; P < 0.001) and the 5-year rate at 3 months postsurgery than patients born before
overall survival rates were 89.3% and 55.7%, respec- the screening era (cohort A) (OR 2.90; P < 0.001).
tively (P < 0.001). Survival with Native Liver The 3-year survival
However, 15 cases in cohort BþC, despite their rates with native liver in patients who received Kasai
birth after the launch of the stool card screening pro- operation before 60 days of age and after 60 days of
gram, were not successfully screened using the stool age were 64.9% and 46.3%, respectively (OR 2.15;
card. In order to clearly demonstrate the effect of the 95% confidence interval [CI] 1.19-3.86; P ¼ 0.01).
stool card screening program, we further analyzed the The 5-year survival rates with native liver in patients
outcome by redividing our total cases from 1990 to who underwent surgery before 60 days old and after
2005 into two groups for comparison: one group rep- 60 days old were 55.0% and 32.1%, respectively (OR
resenting BA children without the screening program 2.58; 95% CI 1.21-5.50; P ¼ 0.01). The 3-year sur-
or not screened out by the stool card, the other group vival rates with native liver in patients who were and
representing BA children who benefited from stool were not jaundice-free at 3 months after Kasai
HEPATOLOGY, Vol. 53, No. 1, 2011 LIEN ET AL. 205

Table 2. Factors Affecting Jaundice-Free Status 3 Months liver than patients who received an operation after 60
After Kasai Operation in Patients with BA days of age (OR 3.25, P < 0.001 and OR 2.63, P ¼
Jaundice-Free* 3 Months 0.02, respectively). Patients who had used prophylactic
After Kasai Operation OR (95% CI) P Value antibiotics had better 3- and 5-year jaundice-free sur-
Age at Kasai operation 0.001 vival with native liver than patients who had not (OR
60 days 65/111 (58.6%) 2.62 (1.45-4.76) 3.03, P ¼ 0.009 and OR 2.79, P ¼ 0.01, respectively).
>60 days 28/80 (35%) 1.00
Stool card program <0.001 Moreover, patients who were jaundice-free at 3 months
Cohort BþC† 62/102 (60.8%) 2.90 (1.61-5.23) postsurgery had better 3- and 5-year jaundice-free sur-
Cohort A ‡ 31/89 (34.8%) 1.00 vival with native liver than those who were not jaun-
*Total serum bilirubin <2.0 mg/dL. dice-free (OR 39.34, 95% CI 17.00-97.06, P < 0.001
†The data of cohort B and cohort C were merged to represent the outcomes and OR 21.43, 95% CI 7.90-58.16, P < 0.001, respec-
in the era of the stool color card screening program. Cohort B (n ¼ 28)
includes patients born during the regional study of the stool color card screen-
tively). Sex did not affect outcome. Intervention by the
ing program (2002-2003). Cohort C (n ¼ 74) includes patients born after the stool card screening program, Kasai operation before 60
launch of the nationwide study of the stool color card screening program days of age, the use of prophylactic antibiotics, and
(2004-2005). jaundice-free at 3 months postsurgery were the predic-
‡Cohort A (n ¼ 89) includes patients born before the institution of the stool
color card screening program (1990-2000). tors of quality outcome for BA patients.
Overall Survival. Cohort BþC had better 3- and
5-year overall survival rates than cohort A (OR 4.64,
operation were 84.9% and 30.6%, respectively (OR P < 0.001 and OR 6.63, P ¼ 0.003, respectively)
12.79; 95% CI 6.27-26.08; P < 0.001). The 5-year (Figs. 1 and 2; Table 4). Patients who had used pro-
survival rates with native liver in patients who were phylactic antibiotics had better 3- and 5-year overall
and were not jaundice-free at 3 months postsurgery survival rates than those who did not (OR 5.33, P <
were 77.6% and 19.4%, respectively (OR 14.35; 95% 0.001 and OR 6.31, P < 0.001, respectively). Those
CI 5.81-35.43; P < 0.001). who were jaundice-free at 3 months after Kasai oper-
Jaundice-Free Survival with Native Liver. Jaun- ation had better 3- and 5-year overall survival rates
dice-free survival with native liver was considered as than those who were not jaundice-free (OR 11.15, P
quality outcome. Cohort BþC had higher rates of < 0.001 and OR 10.85, P < 0.001, respectively).
3- and 5-year jaundice-free survival with native liver
than cohort A (OR 2.87, P ¼ 0.001, and OR 4.80,
P ¼ 0.001, respectively) (Table 3). Patients who
Discussion
received Kasai operation before 60 days of age had Biliary atresia (BA) is an obliterative cholangiopathy
better 3- and 5-year jaundice-free survival with native of unknown etiology. It is the most common cause of

Table 3. Jaundice-Free Survival with Native Liver in Patients with BA


3-Year Jaundice-Free* 5-Year Jaundice-Free
Survival with Native Liver OR (95% CI) P Value Survival with Native Liver OR (95% CI) P Value

Age at Kasai operation <0.001 0.02


60 days 63/111 (56.8%) 3.25 (1.76-6.00) 28/60 (46.7%) 2.63 (1.19-5.78)
>60 days 23/80 (28.8%) 1.00 14/56 (25%) 1.00
Stool card program 0.001 0.001
Cohort BþC† 58/102 (56.9%) 2.87 (1.58-5.21) 18/28 (64.3%) 4.80 (1.94-11.86)
Cohort A‡ 28/89 (31.5%) 1.00 24/88 (27.3%) 1.00
Prophylactic antibiotics 0.009 0.01
Used 70/132 (53.0%) 3.03 (1.56-5.88) 30/65 (46.2%) 2.79 (1.25-6.20)
No use 16/59 (27.1%) 1.00 12/51 (23.5%) 1.00
Sex 0.44 0.58
Male 45/94 (47.9%) 1.25 (0.71-2.22) 21/54 (38.9%) 1.24 (0.58-2.65)
Female 41/97 (42.3%) 1.00 21/62 (33.9%) 1.00

*Total serum bilirubin <2.0 mg/dL.


†The data of cohort B and cohort C were merged to represent the outcomes in the era of the stool color card screening program. Cohort B (n ¼ 28) includes
patients born during the regional study of the stool color card screening program (2002-2003). Cohort C (n ¼ 74) includes patients born after the launch of the
nationwide study of the stool color card screening program (2004-2005).
‡Cohort A (n ¼ 89) includes patients born before the institution of the stool color card screening program (1990-2000). Only 88 patients completed the 5-year
follow-up.
206 LIEN ET AL. HEPATOLOGY, January 2011

Fig. 1. The 5-year overall survival rates in cohort B (dotted line), which Fig. 2. The 5-year overall survival rates in subjects who were jaun-
represents the era of stool color card screening program, and in cohort A dice-free (dotted line) versus those who were not jaundice-free (solid
(solid line), which represents the era before the stool color card screening line) at 3 months after Kasai operation was 89.8% and 44.8%,
program, were 89.3% and 55.7%, respectively (P ¼ 0.002). respectively (P < 0.001).

end-stage liver disease in children, with an incidence of 75 days (P ¼ 0.02). In long-term follow-up, the 20-
0.51 per 10,000 in France,10 0.60 per 10,000 in the year survival with native liver is significantly better in
United Kingdom,11 0.70 per 10,000 in Sweden,12 and patients who receive the operation before the age of 90
0.85 per 10,000 in North America.12 There is a higher days than in those who receive it after 90 days (28%
incidence in Asia, including 1.04 per 10,000 in versus 13%; P ¼ 0.006).5 In the current study, patients
Japan13,14 and 1.78 per 10,000 in Taiwan.7 Taiwan is who underwent Kasai operation before the age of 60
one of the areas with the highest incidence in the world. days had significantly better survival with native liver
Kasai operation is the primary surgical therapy for than those receiving the operation after 60 days of age.
BA, even in the era of liver transplantation.15 Survival The earlier age at Kasai operation is indeed an im-
of BA patients with their native liver relies mainly on portant predictive factor of better long-term survival
the success of the Kasai operation,16 which is correlated with native liver. For early diagnosis of BA, the stool
with age at surgery.13 In the Swiss national study,12 4- card screening program was started in Taiwan in re-
year survival with native liver is 75% in patients who gional areas in 2002 and extended nationwide in
receive the Kasai operation before 46 days, 33% in 2004. The rate of children with Kasai operation before
patients receiving the operation between 46 and 75 60 days of age significantly improved in the stool card
days, and 11% in patients receiving the operation after screening era.

Table 4. Overall Survival with or Without Liver Transplantation in Patients with BA


3-Year Overall Survival OR (95% CI) P Value 5-Year Overall Survival OR (95% CI) P Value

Age at Kasai operation 0.30 0.15


60 days 89/111 (80.2%) 1.44 (0.73-2.85) 42/60 (70%) 1.75 (0.81-3.76)
>60 days 59/80 (73.8%) 1.00 32/56 (57.1%) 1.00
Stool card program <0.001 0.003
Cohort BþC* 91/102 (89.2%) 4.64 (2.17-9.94) 25/28 (89.3%) 6.63 (1.86-23.60)
Cohort A† 57/89 (64.0%) 1.00 49/88 (55.7%) 1.00
Prophylactic antibiotics <0.001 <0.001
Used 115/132 (87.1%) 5.33 (2.60-10.92) 53/65 (81.5%) 6.31 (2.75-14.48)
No use 33/59 (55.9%) 1.00 21/51 (41.2%) 1.00
Sex 0.28 0.32
Male 76/94 (80.9%) 1.47 (0.74-2.91) 37/54 (68.5%) 1.47 (0.68-3.16)
Female 72/97 (74.2%) 1.00 37/62 (59.7%) 1.00

*The data of cohort B and cohort C were merged to represent the outcomes in the era of the stool color card screening program. Cohort B (n ¼ 28) includes
patients born during the regional study of the stool color card screening program (2002-2003). Cohort C (n ¼ 74) includes patients born after the launch of the
nationwide study of the stool color card screening program (2004-2005). Only cohort B was followed up longer than 5 years and calculated in 5-year survival
analyses.
†Cohort A (n ¼ 89) includes patients born before the institution of the stool color card screening program (1990-2000). Only 88 patients completed the 5-year
follow-up.
HEPATOLOGY, Vol. 53, No. 1, 2011 LIEN ET AL. 207

The 3-year survival rate with native liver in the era card alerts the parents, medical personnel, and guardi-
before the stool card screening program was 51.7%, ans to find BA patients and send them for Kasai oper-
which increased to 61.8% in the stool card screening ation earlier when their hepatic damage is milder. Our
era (Table 1). Why is this improvement not as evident data indeed supports that BA patients born in the era
as expected? Persistent and/or progressive jaundice is of stool card screening program have significantly bet-
usually the first alarm of impaired bile flow and pro- ter quality outcome than those born before the era.
gressive liver cirrhosis. In the years before the stool Overall survival is significantly better in the era of
card screening program, the skills and care involved in the stool card screening program. Other studies show
liver transplantation were not as fully developed as that the better the results of the Kasai operation, the
they are now. Moreover, the concept of a living-related better the overall survival.16,18 Although more devel-
donor had not yet been accepted by the general popu- oped transplantation techniques in the stool card
lation. The requirement and timing of liver transplan- screening era partly contribute to survival, the need for
tation was therefore more conservative and delayed. liver transplantation still adds risk to impair the prog-
Some patients, however, lived with their native liver nosis. Successful Kasai operation still provides patients
despite severe jaundice-related complications. with the best chance of survival, and every effort
In the era of the stool card screening program, liver should be made to improve its results.16 The stool
transplantation has become more polished and has card screening program is a step in this direction,
gained more social acceptance. Pediatricians and sur- because it efficiently increases the success rate of Kasai
geons in recent years have preferred to choose an operation and contributes to better overall survival.
appropriate but earlier timed liver transplantation for The 5-year survival rate with native liver and 5-year
those patients with persistent jaundice, before many overall survival rate in other studies range from 30.1%
complications occur. Hence, the 3-year survival rate to 59.7% and from 75.5% to 85%, respectively.13,19,20
with native liver in the stool card screening era is only In Taiwan, these rates are 64.3% and 89.3%, respec-
slightly better than that of the era without screening. tively (Table 1). This corroborates the promising results
As time goes by, fewer and fewer patients can survive of intervention using the stool card screening program.
without transplantation if their jaundice is persistent. In conclusion, the stool card screening program for
In the analyses of 5-year survival with native liver, BA enhances early Kasai operation and increases the
those born in the stool card screening era already show jaundice-free rate at 3 months postsurgery, which is a
significantly better results. valuable predictor of 5-year outcome. In Taiwan, the
We believe that jaundice-free survival rate with native infant stool color card screening program has markedly
liver can reflect the true outcome of BA without the in- improved the 5-year outcome of BA patients.
terference of liver transplantation during time change.
Acknowledgments: We appreciate the valuable con-
Our study defined patients who had jaundice-free sur-
tribution of the members of the Taiwan Infant Stool
vival with native liver as a quality outcome. In our anal- Color Card Study Group and thank Li-Chin Fan,
yses, we found that use of the stool card screening pro- Cheng-Hui Hsiao, Yu-Ru Tseng, and Szu-Ta Chen for
gram and Kasai operation before 60 days of age both assistance in preparing this article.
contribute to quality outcome in BA patients.
In the study by Shneider et al.,17 jaundice-free at 3
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