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Irena Jankowska

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Articles 88
Citations 742
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Recent Articles
1.
Jankowska I, Socha P, Gliwicz D, Lipinski P, Rokicki D, Kalicinski P, et al.
Pediatr Transplant . 2024 Jul; 28(5):e14825. PMID: 39001656
Background: Liver transplantation (LTx) constitutes a major life-saving routine treatment for children with end-stage liver disease. However, the analysis of LTx registries in children provides much information about changes in...
2.
Hansen B, Vandriel S, Vig P, Garner W, Mogul D, Loomes K, et al.
Hepatology . 2023 Dec; 79(6):1279-1292. PMID: 38146932
Background And Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for...
3.
Krawczyk M, Kulaga Z, Niewiadomska O, Jankowska I, Lebensztejn D, Wiecek S, et al.
Clin Res Hepatol Gastroenterol . 2023 Sep; 47(8):102204. PMID: 37678608
Introduction: Gallstone disease (GD) is increasingly common among children, possibly caused by an unhealthy food environment and the associated unhealthy lifestyle. In this study, we investigate the association between body...
4.
Lipinski P, Ciara E, Jurkiewicz D, Mekrouda M, Cielecka-Kuszyk J, Jurkiewicz E, et al.
Diagnostics (Basel) . 2023 Jun; 13(11). PMID: 37296768
Introduction: The increasing usage of NGS technology has enabled the discovery of new causal genes in ciliopathies, including the gene. The aim of our study was to present the clinical,...
5.
Felzen A, van Wessel D, Gonzales E, Thompson R, Jankowska I, Shneider B, et al.
JHEP Rep . 2023 Jan; 5(2):100626. PMID: 36687469
Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are...
6.
Lipinski P, Ciara E, Jurkiewicz D, Pronicki M, Jurkiewicz E, Bogdanska A, et al.
Front Pediatr . 2023 Jan; 10:1061043. PMID: 36589157
Adenosine kinase (ADK) deficiency is a rare inborn error of methionine and adenosine metabolism. So far, a total of 27 patients with ADK deficiency have been reported. Here, we describe...
7.
Krawczyk M, Niewiadomska O, Jankowska I, Jankowski K, Swiderska J, Lebensztejn D, et al.
J Pediatr Gastroenterol Nutr . 2022 Sep; 75(6):692-694. PMID: 36084219
Gallstones are increasingly frequent in children. In this candidate gene study, we genotyped 5 gene variants ( ANO1 , SPTLC3 , TMEM147 , TNRC6B , rs12532734) from a recent gallstone...
8.
Vandriel S, Li L, She H, Wang J, Gilbert M, Jankowska I, et al.
Hepatology . 2022 Aug; 77(2):512-529. PMID: 36036223
Background And Aims: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real-world data are lacking. This study aimed...
9.
Loomes K, Squires R, Kelly D, Rajwal S, Soufi N, Lachaux A, et al.
Hepatol Commun . 2022 May; 6(9):2379-2390. PMID: 35507739
Children with progressive familial intrahepatic cholestasis, including bile salt export pump (BSEP) and familial intrahepatic cholestasis-associated protein 1 (FIC1) deficiencies, suffer debilitating cholestatic pruritus that adversely affects growth and quality...
10.
Montano-Loza A, Ronca V, Ebadi M, Hansen B, Hirschfield G, Elwir S, et al.
J Hepatol . 2022 Feb; 77(1):84-97. PMID: 35143897
Background & Aims: Autoimmune hepatitis can recur after liver transplantation (LT), though the impact of recurrence on patient and graft survival has not been well characterized. We evaluated a large,...