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To the Editor:

With great interest, we read the report by Lien et al.1 on their experience with using a stool color card (SCC) for the early identification of babies with biliary atresia. So far, Taiwan is the only country with regular, nationwide screening for this devastating disease. With this screening, the age for Kasai hepatoportoenterostomy has significantly dropped, and this has meant a significant increase in jaundice-free survival with the native liver at the age of 3 years. The same Taiwanese group has shown that the sensitivity and specificity of the SCC for the detection of biliary atresia are 97.1% and 99.9%, respectively.2

Biliary atresia is one of the most progressive fibrogenic liver diseases. The more advanced the liver fibrosis is at the time of the Kasai operation, the worse the chances are for the child to live with his or her own liver; increased age at the time of surgery has a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence.3-5

The criteria for a condition to be considered appropriate for newborn screening are as follows: (1) the condition is an important health problem; (2) there is a recognizable latent period or an early symptomatic period during which intervention may be beneficial; (3) there are suitable screening tests or examinations that are acceptable, reliable, easy to apply, and available; and (4) there are accepted treatments that are available and beneficial when they are applied early.6, 7 The implementation of screening for biliary atresia is thus justified. Moreover, the reported results convincingly demonstrate that in Taiwan, the SCC is a simple, noninvasive, efficient, low-cost, and applicable mass screening method for the early diagnosis and management of biliary atresia.

These considerations led to the design of a Swiss national biliary atresia screening pilot program, which was started in 2009. The Swiss SCC is available in German, French, Italian, and English (Fig. 1). It is explained and handed out to the parents after their child's birth by the attending pediatrician or midwife. An instructive Web site for parents and health care personal has been established (http://www.basca.ch). The SCC and the baby's stool color are checked during the first visit with the treating physician, usually at the age of 4 weeks. During the current pilot period for the screening, SCC data are immediately transmitted to the coordination center in Geneva, Switzerland, to evaluate the feasibility and acceptance of the screening program: Switzerland obviously has a culture and mentality different from those of Taiwan. If an abnormal stool color is discovered, a further evaluation of the baby is immediately performed. By signing the SCC, the parents give their informed, written consent to the physician to communicate the data to the coordination center.

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Figure 1. English version of the Swiss SCC for biliary atresia screening. Published with the permission of the program director.

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The current program is open to all interested physicians, and SCCs can be ordered for free via the Web site (http://www.basca.ch). The screening is voluntary, and money is not received by the patient, the participating physician, or the coordination center.

References

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  • 1
    Lien TH, Chang MH, Wu JF, Chen HL, Lee HC, Chen AC, et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology 2011; 53: 202-208.
  • 2
    Hsiao CH, Chang MH, Chen HL, Lee HC, Wu TC, Lin CC, et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008; 47: 1233-1240.
  • 3
    Wildhaber BE, Coran AG, Drongowski RA, Hirschl RB, Geiger JD, Lelli JL, et al. The Kasai portoenterostomy for biliary atresia: a review of a 27-year experience with 81 patients. J Pediatr Surg 2003; 38: 1480-1485.
  • 4
    Wildhaber BE, Majno P, Mayr J, Zachariou Z, Hohlfeld J, Schwoebel M, et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr 2008; 46: 299-307.
  • 5
    Serinet MO, Wildhaber BE, Broué P, Lachaux A, Sarles J, Jacquemin E, 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: 1280-1286.
  • 6
    Therrell BL Jr. U.S. newborn screening policy dilemmas for the twenty-first century. Mol Genet Metab 2001; 74: 64-74.
  • 7
    Powell JE, Keffler S, Kelly DA, Green A. Population screening for neonatal liver disease: potential for a community-based programme. J Med Screen 2003; 10: 112-116.

Barbara E. Wildhaber M.D.*, * Service of Pediatric Surgery, Children's Hospital of Geneva, University Hospital of Geneva, Geneva, Switzerland.