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Published in Samar Razaq, Difficult Cases in Primary Care, 2021
Biliary atresia is a disease of unknown cause in which there is inflammatory obstruction and destruction of the intra-and extrahepatic bile ducts. It is the most common cause of neonatal liver disease. Early identification is necessary for the neonate to have the best chance of recovery. Kasai portoenterostomy is effective in achieving good biliary drainage, thus improving the chances of the neonate reaching adolescence without the need for a liver transplantation. It should be suspected in all neonates with prolonged jaundice, raised conjugated bilirubin level and pale stools, triggering an urgent referral to a paediatrician.
Paper 1
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
The baby has typical radiological features of biliary atresia with hepatomegaly and the ‘triangular cord’ sign which is caused by fibrous echogenic tissue anterior to the portal vein. Intrahepatic biliary duct dilatation may or may not be present. The inability to see the gallbladder is suspicious and the common bile duct may also not be visible. Biliary atresia most commonly presents within the first couple of months of life with jaundice, pale stools and dark urine. Prompt diagnosis and surgical treatment is important to avoid cirrhosis and liver transplant.
The cases
Published in Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young, Paediatric Radiology for MRCPCH and FRCR, 2020
Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young
Treatment of biliary atresia is surgical, either with a portoenterostomy (Kasai procedure—only 12% cases are suitable for direct anastamotic drainage) or liver transplantation (for unsuitable cases or those with end-stage liver disease).
Does Arterialization of Portal Vein Have Any Effects in Large-for-Size Liver Transplantation? Hemodynamic, Histological, and Biomolecular Experimental Studies
Published in Journal of Investigative Surgery, 2022
Rafael Rodrigues Torres, Ana Cristina Aoun Tannuri, Suellen Serafini, Alessandro Belon, Josiane Oliveira Gonçalves, Celso di Loreto, Uenis Tannuri
Orthotopic liver transplantation is a universally accepted treatment approach for progressive irreversible liver diseases. Among these, biliary atresia corresponds to approximately 60% of liver transplant indications in the pediatric population and most cases require liver replacement before 12 months of life when children usually weigh less than 10 kilograms. In untreated children, BA leads to death by 18 to 24 months of life in most cases. Therefore, liver transplant in this population often shows an imbalance between graft and recipient sizes due to the difficulties in obtaining size-matched livers that are small enough for the recipient. This condition is known as “large-for-size” and may be linked to higher morbidity, mortality, and graft loss rates. In liver transplantation, the optimal size of the transplanted liver ranges between 0.8% and 4.0% of the recipient’s body weight (graft to body weight ratio – GBWR) [1–3] but it is not uncommon to find grafts that reach 5.0% of the recipient’s weight.
Coexistence of Three Different Mutations in a Male Infant: neurofibromatosis Type 1, Progressive Familial Intrahepatic Cholestasis Type 2 and LPIN3
Published in Fetal and Pediatric Pathology, 2022
Derya Altay, Orhan Gorukmez, Duran Arslan
Cholestatic jaundice is a complex clinical condition with multiple causes that affects approximately 1 in 2500 infants. Biliary atresia, which can be treated surgically, is ruled out first when approaching a patient with cholestasis. This is then followed by excluding infectious, metabolic, and genetic diseases. Genetic testing of the patient revealed PFIC2 that is caused by the mutation of the ABCB11 gene on chromosome 2 and is characterized by a defect in bile acid transport from hepatocyte to canalicular system [1]. Pruritus in PFIC 2 is very severe and occurs as a result of stimulation of unmyelinated free nerve endings with serum bile acids. In PFIC 2, liver biopsy shows cholestasis, giant cell hepatitis, hepatocellular necrosis, and portal fibrosis [1]. The liver biopsy of the patient also revealed giant cell hepatitis. In the management of PFIC 2, the first-line treatment is ursodeoxycholic acid; however, if this does not yield any results, partial biliary diversion can be tried. If this procedure fails and end-stage liver failure develops, liver transplantation should be performed [1].
Pharmacological management of portal hypertension and its complications in children: lessons from adults and opportunities for the future
Published in Expert Opinion on Pharmacotherapy, 2021
Sarah Henkel, Carol Vetterly, Robert Squires, Patrick McKiernan, James Squires
Despite decades of patient care, optimal management of pediatric PHT remains undefined and subject to individual experience and institutional practices. In large part, this can be directly linked to a lack of basic understanding related to the unique pathophysiology and natural history that defines PHT in children. As a result, meaningful research into the utility and effectiveness of pharmacotherapy in children with PHT remains in its infancy. Large, multi-center, prospective studies are needed to establish an infrastructure on which a pediatric-specific research agenda can be built. Through these collaborative efforts, well designed, clinical trials can be conducted, and ideal treatment strategies will become clearer. The complexity of the design of these trials has been noted in adults [130], and similar intricacies should be expected in children. Notably, it would be prudent for trials to start with a single unifying diagnosis, such as biliary atresia, to most accurately examine treatment effects.