Search Results - "Jankowska, Irena"
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The microstructure and thermal stability of the two‐phase amorphous melt‐spun alloys ejected from a double‐chamber crucible
Published in Journal of microscopy (Oxford) (01-05-2023)“…This work presents the microstructure and properties of two‐phase amorphous melt‐spun alloys ejected from the crucible with partition between liquids. The…”
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2
The complexity of structural phase transitions in Pb(Hf0.92Sn0.08)O3 single crystals
Published in Journal of the American Ceramic Society (01-11-2021)“…Pb(Hf1−xSnx)O3 single crystals with x = 0.08 were characterized using single‐crystal X‐ray diffraction and Raman scattering, in a wide temperature range. The…”
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3
Management of gallstone disease in children
Published in Pediatria polska (2023)“…In recent years an increase in incidence of cholelithiasis in children has been observed. Among the various factors predisposing to the occurrence…”
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Progressive familial intrahepatic cholestasis type 3: Report of four clinical cases, novel ABCB4 variants and long-term follow-up
Published in Annals of hepatology (01-11-2021)“…Progressive familial intrahepatic cholestasis type 3 (PFIC-3) is a rare autosomal recessive cholestatic liver disorder caused by mutations in the ABCB4 gene…”
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5
The Influence of Measurement Conditions on the Electrocaloric Effect in Ferroelectric Ceramics of Modified Barium Titanate
Published in Materials (05-07-2024)“…In this work, the electrocaloric effect (ECE) and electrocaloric strength (Δ / ) were measured and thermal and dielectric studies were performed on Pb-modified…”
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6
Maralixibat for the treatment of PFIC: Long‐term, IBAT inhibition in an open‐label, Phase 2 study
Published in Hepatology communications (01-09-2022)“…Children with progressive familial intrahepatic cholestasis, including bile salt export pump (BSEP) and familial intrahepatic cholestasis–associated protein 1…”
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Coronavirus Disease 2019-Liver Injury-Literature Review and Guidelines Based on the Recommendations of Hepatological Societies
Published in Pediatric gastroenterology, hepatology & nutrition (01-03-2021)“…The aim of our paper was to present current knowledge, review literature and available practice guidelines of international hepatological associations…”
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8
Acute liver failure in children-Is living donor liver transplantation justified?
Published in PloS one (23-02-2018)“…Living donor liver transplantation (LDLT) in patients with acute liver failure (ALF) has become an acceptable alternative to transplantation from deceased…”
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9
Differences in presentation and progression between severe FIC1 and BSEP deficiencies
Published in Journal of hepatology (01-07-2010)“…Background & Aims Progressive familial intrahepatic cholestasis (PFIC) with normal serum levels of gamma-glutamyltranspeptidase can result from mutations in…”
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10
NBAS deficiency due to biallelic c.2809C > G variant presenting with recurrent acute liver failure with severe hyperammonemia, acquired microcephaly and progressive brain atrophy
Published in Metabolic brain disease (01-10-2021)“…Biallelic pathogenic variants in the neuroblastoma amplified sequence ( NBAS ) gene were firstly (2015) identified as a cause of fever-triggered recurrent…”
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Special Issue: Emerging Dielectric, Piezoelectric, and Ferroelectric Ceramic and Crystalline Materials and Their Applications
Published in Materials (23-07-2022)“…Materials Physics: Emerging Dielectric, Piezoelectric, and Ferroelectric Ceramic and Crystalline Materials and their Applications is an open Special Issue of…”
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12
Coronavirus Disease 2019-Liver Injury-Literature Review and Guidelines Based on the Recommendations of Hepatological Societies
Published in Pediatric gastroenterology, hepatology & nutrition (2021)“…The aim of our paper was to present current knowledge, review literature and available practice guidelines of international hepatological associations…”
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13
Composition-Related Dielectric, Ferroelectric and Electrocaloric Properties of Pb5Ge3O11 Single Crystals Modified by Ba Ions
Published in Materials (01-01-2023)“…In this paper, we studied some ferroelectric properties of archetypal oxide uniaxial ferroelectric single crystals of Pb5Ge3O11 modified by Ba ions. They…”
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14
An Overview of Some Nonpiezoelectric Properties of BaTiO3 Ceramics Doped by Eu Ions
Published in Materials (04-08-2022)“…Ferroelectric ceramics BaTiO3:x%Eu (x = 0, 0.1, 1, 2, 3) were synthesized by a conventional method. Structural investigation confirmed that all ceramics…”
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Case Report: Adenosine kinase deficiency diagnosed 10 years after liver transplantation: Novel phenotypic insights
Published in Frontiers in pediatrics (14-12-2022)“…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…”
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Long-term follow-up in children with progressive familial intrahepatic cholestasis type 2 after partial external biliary diversion with focus on histopathological features
Published in Polish journal of pathology (01-01-2019)“…Progressive familial intrahepatic cholestasis (PFIC) comprises a group of rare cholestatic liver disorders of childhood that could lead to liver cirrhosis…”
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DCDC2 -Related Ciliopathy: Report of Six Polish Patients, Novel DCDC2 Variant, and Literature Review of Reported Cases
Published in Diagnostics (Basel) (30-05-2023)“…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…”
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Sterol 27-Hydroxylase Deficiency as a Cause of Neonatal Cholestasis: Report of 2 Cases and Review of the Literature
Published in Frontiers in pediatrics (13-01-2021)“…Inborn errors of primary bile acid (BA) synthesis are rare autosomal recessive disorders responsible for 1-2% of cases of neonatal cholestasis. Among them,…”
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Successful Liver Transplantation in Two Polish Brothers with Transaldolase Deficiency
Published in Children (Basel) (01-09-2021)“…Transaldolase deficiency (TALDO; OMIM 606003) is a rare inborn autosomal-recessive error of the pentose phosphate pathway. It is an early-onset multisystem…”
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Successful pregnancy after ileal exclusion in progressive familial intrahepatic cholestasis type 2
Published in Annals of hepatology (01-07-2015)“…AbstractProgressive familial intrahepatic cholestasis type 2 (PFIC 2) results from mutations in ABCB11 gene coding bile salt export pump (BSEP). Medical…”
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