Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016.

Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries.

Hepatobiliary scintigraphy for early diagnosis of biliary atresia.

Introduction: The aim of this study was to evaluate the validity of (99m)Technetium-trimethylbromo-iminodiacetic acid hepatobiliary scintigraphy (HS) for the diagnosis of biliary atresia (BA).

Methods: From January 2005 to December 2009, a total of 47 infants with conjugated hyperbilirubinaemia (> 20 micromol/l total bilirubin of which 20% is conjugated) underwent HS. BA was suspected if no tracer was visualised in the gut 24 hours post-injection.

Prophylactic Dosing of Vitamin K to Prevent Bleeding.

Background And Objectives: Based on a high incidence of Vitamin K deficiency bleeding (VKDB) in breastfed infants with thus far unrecognized cholestasis, such as biliary atresia (BA), the Dutch regimen to prevent VKDB in breastfed infants was changed from a daily oral dosage of 25 µg to 150 µg vitamin K. Infants continued to receive 1 mg of vitamin K orally at birth. We compared the efficacy of the 150-µg regimen with the 25-µg regimen and with the Danish regimen of a single intramuscular (IM) dose of 2 mg vitamin K at birth.

Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia.

Introduction: Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia.

[The plasma anion gap is a useful tool for evaluating children with metabolic acidosis].

Metabolic acidosis occurs frequently in hospitalized children. The causes are many and often apparent from the history and physical examination. However, if the aetiology is unclear, the plasma anion gap is a useful tool for evaluating patients with metabolic acidosis.

[Acute, severe liver insufficiency caused by extrahepatic biliary atresia in a newborn].

A newborn female was hospitalized due to metabolic acidosis and conjugated hyperbilirubinaemia. Extrahepatic biliary atresia (EHBA) was suspected why a (99m)Tc-mebrofenin cholescintigraphy was performed. It showed poor hepatocyte tracer uptake and no drainage to the gut.

Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis--a Danish study.

Background/purpose: The aims of this study are as follows:

Method: From 1979 to 2003, 57 children have been operated by the Kasai procedure. Only 40 of these have had their portal plate removed for histologic examination. We divided the patients according to clinical outcome into a successful and a failure group and compared the histologic features of the portal plates in the 2 groups.

Congenital biliary atresia: liver injury begins at birth.

Background: The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth.

Methods: The study involved retrospective case-note review for infants with definite BA who underwent laparotomy within first week of life.

Results: Three infants were identified who had occlusive BA evident on the first day of life.

[Surgical treatment of children with hepatic tumours].

Introduction: In this paper we review the results of surgical treatment of children with hepatic tumours.

Materials And Methods: The study comprises 33 children who have undergone lever resection or liver transplantation since 1990. 26 patients had hepatoblastoma, 3 had hepatocellular carcinoma, 2 had rhabdomyosarcoma, 1 had a mesenchymal tumour, and 1 had a giant haemangioma.

Prevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries.

Objective: Newborns routinely receive vitamin K to prevent vitamin K deficiency bleeding. The efficacy of oral vitamin K administration may be compromised in infants with unrecognized cholestasis. We aimed to compare the risk of vitamin K deficiency bleeding under different prophylactic regimens in infants with biliary atresia.