In this sample of children with SBS, the IP was within normal limits but did not correlate with age. LGG therapy had no consistent effects on IP. These findings do not support empiric LGG therapy to enhance IP in children with SBS.

Introduction Hepatoportoenterostomy (HPE) is the first-line treatment for biliary atresia (BA) patients. This study aims to describe perioperative complications after HPE and to analyze their impact on outcome. Materials and Methods Patients with HPE (Swiss National Biliary Atresia Registry, 1994–2017) were retrospectively analyzed. Perioperative complications were defined as complications occurring up to 30 days after surgery. Surgical complications were defined as directly related to the surgical act; medical complications were defined as any other deviation from the uneventful postoperative course. Results Sixty-two patients were included. Median age at HPE was 63 days (18–126). Twenty six patients out of 62 (42%) had ≥ 1 complications: 6/62 (10%) surgical, 24/62 (39%) medical, that is, we observed 7 surgical and 28 medical complications. As for medical complications, cholangitis was the most frequent: 19/28 (68%). Lower gestational age at birth correlated with more overall complications (p = 0.02). Age, weight at HPE, syndromic BA, and postoperative steroid administration were not significantly correlated. There were no perioperative deaths. Perioperative complications did not correlate with overall survival (p = 0.14) and survival with native liver (p = 0.55). Conclusion HPE is often associated with perioperative medical complications. Lower gestational age at birth was significantly associated with more complications. Perioperative complications had no impact on overall outcome.

PurposeBiliary atresia can easily be screened using a stool colour card (SCC) and has shown to significantly reduce time to diagnosis, improving children’s outcome. Despite the general approval of the clinical usefulness of the SCC, physicians remain reluctant: it might unnecessarily worry parents. This study aimed to analyse the parental reaction to this screening method and if it evokes parental stress.MethodsA semistructured questionnaire was sent to parents with one or more healthy child to inquire about reactions on receipt and use of the SCC.Results109/256 questionnaires were returned and evaluated (43%). 107/107 parents considered the SCC as helpful, a simple screening method and easy to use (100%). 26/43 were reassured when receiving the SCC (60%), 2 were worried (5%) and 9 had no particular feelings (21%). In 41/49, emotions experienced during its use were positive or neutral (84%), and 3 were worried (6%). In 41/50, the discussion with the paediatrician about stool colour-linked pathologies was neutral (82%), and 9 felt uneasy (18%).ConclusionA vast majority of parents appreciate the SCC. It creates uneasiness in a minority of parents. Our results are encouraging and argue in favour of implementing the regular distribution of the SCC in antenatal, postnatal and newborn infant clinics.

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Background Congenital portosystemic shunts (CPSS) are rare vascular malformations associated with the risk of life-threatening systemic conditions, which remain underdiagnosed and often are identified after considerable diagnostic delay. CPSS are characterized by multiple signs and symptoms, often masquerading as other conditions, progressing over time if the shunt remains patent. Which patients will benefit from shunt closure remains to be clarified, as does the timing and method of closure. In addition, the etiology and pathophysiology of CPSS are both unknowns. This rare disorder needs the strength of numbers to answer these questions, which is the purpose of the international registry of CPSS (IRCPSS). Method A retrospective and prospective registry was designed using secuTrial® by the ISO certified Clinical Research Unit. Given that a significant number of cases entered in the registry are retrospective, participants have the opportunity to use a semi-structured minimal or complete data set to facilitate data entry. In addition, the design allows subjects to be entered into the IRCPSS according to clinically relevant events. Emphasis is on longitudinal follow-up of signs and symptoms, which is paramount to garner clinically relevant information to eventually orient patient management. The IRCPSS includes also three specific forms to capture essential radiological, surgical, and cardiopulmonary data as many times as relevant, which are completed by the specialists themselves. Finally, connecting the clinical data registry with a safe image repository, using state-of-the-art pseudonymization software, was another major focus of development. Data quality and stewardship is ensured by a steering committee. All centers participating in the IRCPSS have signed a memorandum of understanding and obtained their own ethical approval. Conclusion Through state-of-the-art management of data and imaging, we have developed a practical, user-friendly, international registry to study CPSS in neonates, children, and adults. Via this multicenter and international effort, we will be ready to answer meaningful and urgent questions regarding the management of patients with CPSS, a condition often ridden with significant diagnostic delay contributing to a severe clinical course.