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A lower secondary school education was reported by 3 children, and comprehensive school education by 2 children. No child has gone on to higher education, while 2 children did attend a special formm school.

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AsnAsn hom synonymous c. As a treatment strategy, UDCA has been used in most patients soon after diagnosis. In total, all 38 patients were analyzed for ABCB4 variants. Epidemiologic data for PFIC3 do not exist. At the time of diagnosis, only 7 patients had elevated serum bilirubin concentrations. ArgGln het missense c.

ProLeu hom missense 13 c. A positive response to UDCA was defined as normalization of aminotransferase activities and total bilirubin; no improvement constituted a negative response. The family background revealed consanguinity in six of 19 families.

Genetic predisposition of the intrahepatic pregnancy scholestasis: In 12 patients of the study cohort, the diagnosis of ABCB4 deficiency was made in adulthood.


All exons and exon—intron boundaries of the ABCB4 gene were sequenced as described. However, current medical therapy is not satisfactory for the majority of patients in the long term.

Thirteen patients underwent liver transplantation 4. There is a period of latency between the onset of symptoms and diagnosis. Children surviving liver transplantation are at risk of prolonged cognitive and academic deficits.

The Human Gene Mutation Database. Laboratory parameters collected at the first available documentation were alanine aminotransferase, aspartate aminotransferase, GGT, serum bilirubin, and bile acid concentrations.

Supported by travel grants provided by the patient organization Billy Rubin eV. Clin Genet ; Moreover, histopathologic changes were not assessed systematically due to the heterogeneity of biopsy sampling at different time points, when performed, reflecting various stages of disease progression. Despite the retrospective and multicenter nature of this study, its strength is the large number of patients who were included and treated successfully.

The wide spectrum of multidrug resistance 3 deficiency: Central nervous system involvement, mostly concentration disturbances, was reported by the parents of 9 patients. Due to the rare nature of mutations in the ABCB4 gene, cases with genetically proven ABCB4 gene mutations were systematically collected in a multicenter study with 10 participating hospitals in Germany. AlaPro het missense 36 c. GlyAla het missense c.


Dig Liver Dis ; Severe hepatocellular dysfunction in obstetric cholestasis related to combined genetic variation in hepatobiliary transporters. The first laboratory values were determined at a median age of 0. ABCB4 deficiency with onset in younger patients caused a more severe PFIC type 3 phenotype with the need for liver transplantation in half the children.

Phenotypic spectrum and diagnostic pitfalls of ABCB4 deficiency depending on age of onset

SerPhe het missense 26 c. ProLeu hom missense 7 c. A functional classification of ABCB4 variations causing progressive familial intrahepatic cholestasis type 3. Treatment in 1 of the patients with ICP resulted in a complete response while pregnant, and the second patient responded partially. J Pediatr ; In patients with disease onset before the age of 18 years, 20 different mutations were found. Clinical diagnosis followed symptoms 1.

J Pediatr Surg ; Fluctuating activities of transaminases and even normal GGT or bilirubin levels might contribute to the delay in correct diagnosis. Outcome was analyzed regarding treatment with ursodeoxycholic acid UDCArifampicin, or phenobarbital and was assessed by liver function tests and development of pruritus.

Presentation of progressive familial intrahepatic cholestasis type 3 mimicking Wilson disease: Pro95Ser hom missense 23 c.