To Wean or Not to Wean: The Role of Autologous Reconstructive Surgery in the Natural History of Pediatric Short Bowel Syndrome on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP)

Pediatric Short Bowel Syndrome (SBS) can require prolonged parenteral nutrition (PN). Over the years, SBS management has been implemented by autologous gastrointestinal reconstructive surgery (AGIR). The primary objective of the present review was to assess the effect of AGIR on weaning off PN. We also evaluated how AGIR impacts survival, the need for transplantation (Tx) and the development of liver disease (LD). We conducted a systematic literature search to identify studies published from January 1999 to the present and 947 patients were identified. PN alone was weakly associated with higher probability of weaning from PN (OR = 1.1, p = 0.03) and of surviving (OR = 1.05, p = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, p = 0.03). Finally, adjusting for age and primary diagnosis (gastroschisis), any association was lost. The prevalence of TX and LD did not differ by groups. In conclusion, in view of the low benefit in terms of intestinal adaptation and of the not negligible rate of complications (20%), a careful selection of candidates for AGIR should be required. Bowel dilation associated with failure of advancing EN and poor growth, should be criteria to refer for AGIR

Incidence of Isolated Biliary Atresia during the COVID Lockdown in Europe: Results from a Collaborative Project by RARE-Liver

Background: Biliary atresia (BA) is a rare cholangiopathy where one of the proposed aetiological mechanisms is an infectious viral trigger. Coronavirus disease-19 (COVID) lockdown restrictions were implemented to reduce the transmission of infections. Strictness of lockdown varied across European countries. This study aimed to investigate if there was an association between strictness of lockdown and change in isolated BA (IBA) incidence in Europe. Methods: We approached European centres involved in the European Reference Network RARE-LIVER. We included IBA patients born between 2015 and June 2020. We calculated the number of IBA patients born per centre per month. The Stringency Index (SI) was used as lockdown strictness indicator. The association between percentage change of mean number of IBA patients born per month and the SI was assessed. Results: We included 412 IBA patients from thirteen different centres. The median number of patients per month did not change: 6 (1–15) pre-lockdown and 7 (6–9) during lockdown (p = 0.34). There was an inverse association between SI and percentage change in IBA (B = -0.73, p = 0.03). Median age at Kasai portoenterostomy (days) did not differ between time periods (51 (9–179) vs. 53 (19–126), p = 0.73). Conclusion: In this European study, a stricter COVID-lockdown was seemingly accompanied by a simultaneous larger decrease in the number of IBA patients born per month in the lockdown. Results should be interpreted with caution due to the assumptions and limitations of the analysis

Monogenic diseases that can be cured by liver transplantation

While the prevalence of most diseases caused by single-gene mutations is low and defines them as rare conditions, all together, monogenic diseases account for approximately 10 in every 1000 births according to the World Health Organisation. Orthotopic liver transplantation (LT) could offer a therapeutic option in monogenic diseases in two ways: by substituting for an injured liver or by supplying a tissue that can replace a mutant protein. In this respect, LT may be regarded as the correction of a disease at the level of the dysfunctional protein. Monogenic diseases that involve the liver represent a heterogeneous group of disorders. In conditions associated with predominant liver parenchymal damage (i.e., genetic cholestatic disorders, Wilson's disease, hereditary hemochromatosis, tyrosinemia, α1 antitrypsin deficiency), hepatic complications are the major source of morbidity and LT not only replaces a dysfunctional liver but also corrects the genetic defect and effectively cures the disease. A second group includes liver-based genetic disorders characterised by an architecturally near-normal liver (urea cycle disorders, Crigler-Najjar syndrome, familial amyloid polyneuropathy, primary hyperoxaluria type 1, atypical haemolytic uremic syndrome-1). In these defects, extrahepatic complications are the main source of morbidity and mortality while liver function is relatively preserved. Combined transplantation of other organs may be required, and other surgical techniques, such as domino and auxiliary liver transplantation, have been attempted. In a third group of monogenic diseases, the underlying genetic defect is expressed at a systemic level and liver involvement is just one of the clinical manifestations. In these conditions, LT might only be partially curative since the abnormal phenotype is maintained by extrahepatic synthesis of the toxic metabolites (i.e., methylmalonic acidemia, propionic acidemia). This review focuses on principles of diagnosis, management and LT results in both paediatric and adult populations of selected liver-based monogenic diseases, which represent examples of different transplantation strategies, driven by the understanding of the expression of the underlying genetic defect. © 2013 European Association for the Study of the Liver

Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children

Intestinal failure-associated liver disease (IFALD) is a threatening complication for children on long-term parenteral nutrition because of intestinal failure. When progressive and intractable, it may jeopardize intestinal rehabilitation and lead to combined liver and intestinal transplantation. The institution of dedicated intestinal failure centers has dramatically decreased the incidence of such complication. IFALD may rapidly fade away if very early management aimed at preventing progression to end-stage liver disease is provided. In this review, we address the etiology and risk factors of IFALD in order to introduce pillars of prevention (nutritional management and catheter-related infections control). The latest evidence of therapeutic strategies, such as medical and surgical treatments, is also discussed

Percutaneous transhepatic treatment of a unique portal vein malformation with portal hypertension in a pediatric patient

Abstract Background Anomalies of the portal venous system can be congenital or acquired, the latter being related to spontaneous thrombosis or iatrogenic alterations such as complications of perinatal catheterization of the umbilical vein. These conditions can be clinically silent for years and then manifest abruptly causing severe clinical emergencies. Case presentation This case report describes the diagnosis and interventional management of a singular abnormality in the portal venous system of an 8-year-old female that led to severe portal hypertension and acute variceal bleeding. Peculiar imaging findings were not pathognomonic for any of the known congenital and acquired portal vein anomalies: absence of a normal extrahepatic portal vein; splenic and mesenteric veins merging into a dilated left gastric vein; presence of an aberrant mesenteric venous collateral with a stenotic connection with the intrahepatic right portal branch; and absence of porto-systemic shunt. The case was successfully managed with percutaneous transhepatic portography and angioplasty. Conclusions Prompt non-invasive imaging characterization allowed to understand the singular vascular abnormality and mini-invasive interventional radiology management resolved portal hypertension and variceal bleeding

Incidence of Cholangitis and Sepsis Associated with Percutaneous Transhepatic Cholangiography in Pediatric Liver Transplant Recipients

Background. Percutaneous transhepatic cholangiography (PTC) is an established treatment in the management of biliary strictures. The aim of our study was to determine the incidence of PTC-related infectious complications in transplanted children, and identify their precise aetiol-ogy. Methods. We retrospectively reviewed all PTC performed from January 2017 to October 2020 in our center. Before the procedure, all patients received antibiotic prophylaxis defined as first line, while second line was used in case of previously microbiological isolation. Cholangitis was defined as fever (>38.5°) and elevated inflammatory markers after PTC, while sepsis included hemodynamic instability in addition to cholangitis. Results. One hundred and fifty-seven PTCs from 50 pediatric recipients were included. The overall incidence of cholangitis and sepsis after PTC was 44.6% (70/157) and 3.2% (5/157), respectively, with no fatal events. Blood cultures yielded positive results in 15/70 cases (21.4%). Enterococcus faecium and Pseudomonas aeruginosa were the most common isolated pathogens. Multidrug-resistant (MDR) pathogens were found in 11/50 patients (22%). Conclusion. PTC is associated with a relatively high rate of post-procedural cholangitis, although with low rate of sepsis and no fatal events. Blood cultures allowed to find a precise aetiology in roughly a quarter of the cases, showing prevalence of Enterococcus faecium and Pseudomonas aeruginosa

Under-Vaccination in Pediatric Liver Transplant Candidates with Acute and Chronic Liver Disease—A Retrospective Observational Study of the European Reference Network TransplantChild

Infection is a serious concern in the short and long term after pediatric liver transplantation. Vaccination represents an easy and cheap opportunity to reduce morbidity and mortality due to vaccine-preventable infection. This retrospective, observational, multi-center study examines the immunization status in pediatric liver transplant candidates at the time of transplantation and compares it to a control group of children with acute liver disease. Findings show only 80% were vaccinated age-appropriately, defined as having received the recommended number of vaccination doses for their age prior to transplantation; for DTP-PV-Hib, less than 75% for Hepatitis B and two-thirds for pneumococcal conjugate vaccine in children with chronic liver disease. Vaccination coverage for live vaccines is better compared to the acute control group with 81% versus 62% for measles, mumps and rubella (p = 0.003) and 65% versus 55% for varicella (p = 0.171). Nevertheless, a country-specific comparison with national reference data suggests a lower vaccination coverage in children with chronic liver disease. Our study reveals an under-vaccination in this high-risk group prior to transplantation and underlines the need to improve vaccination

The Role of Imaging in Portal Vein Thrombosis: From the Diagnosis to the Interventional Radiological Management

Purpose: To illustrate diagnostic and interventional imaging for the characterization and treatment of portal vein thrombosis (PVT). Introduction: The broad spectrum of congenital and acquired PVT manifestations is illustrated, with a focus on the pediatric population; diagnostic and interventional imaging techniques are described. Description: PVT frequently presents as an incidental finding at imaging in the screening for liver diseases or for other unrelated reasons. PVT can be classified based on: extension (intrahepatic, extrahepatic, involving the spleno-mesenteric tract, etc.); degree (partial or complete); onset (acute or chronic); and with or without cavernomatous transformation. This comprehensive review relies on the experience gained from a large series of congenital and acquired PVT in a referral center for pediatric and adult liver transplantation. Diagnostic and interventional imaging techniques are described, including: color–Doppler and contrast-enhanced Ultrasound; CT and MR angiography; retrograde portography; percutaneous transhepatic, transplenic, and transmesenteric portography; transjugular intrahepatic portosystemic shunt creation. Pre- and post-operative imaging assessment of the surgical meso-rex bypass is discussed. The description is enriched with an original series of pictorial imaging findings. Conclusion: PVT is a clinical condition associated with significant morbidity and mortality. Diagnostic and interventional imaging plays a crucial role in both conservative and operative management