A5041154337- Pediatric Hepatobiliary Diseases and Treatments
- Liver Diseases and Immunity
- Liver Disease Diagnosis and Treatment
- Hepatitis B Virus Studies
- Hepatitis C virus research
- Genetic and Kidney Cyst Diseases
- Drug Transport and Resistance Mechanisms
- Congenital Anomalies and Fetal Surgery
- Celiac Disease Research and Management
- Metabolism and Genetic Disorders
- Pharmacological Effects and Toxicity Studies
- Microscopic Colitis
- Gallbladder and Bile Duct Disorders
- Diabetes and associated disorders
- Endoplasmic Reticulum Stress and Disease
- Genetics and Neurodevelopmental Disorders
- Immunodeficiency and Autoimmune Disorders
- Gastrointestinal disorders and treatments
- Liver Disease and Transplantation
- Drug-Induced Hepatotoxicity and Protection
- Hepatitis Viruses Studies and Epidemiology
- Eosinophilic Esophagitis
- Organ Transplantation Techniques and Outcomes
- Sphingolipid Metabolism and Signaling
- Autoimmune and Inflammatory Disorders Research
Children's Memorial Health Institute
2013-2024
National Institute of Public Health
2008-2019
Jagiellonian University
2017
Instytut Matki i Dziecka
2000-2016
Medical University of Lublin
2016
Creative Research Enterprises (United States)
2016
Lurie Children's Hospital
2014
Oxford University Hospitals NHS Trust
2014
Toho University Medical Center Sakura Hospital
2014
Toho University
2014
Hepatocellular carcinoma (HCC) is rare in young children. We attempted to see if immunohistochemical and mutational-analysis studies could demonstrate that deficiency of the canalicular bile acid transporter salt export pump (BSEP) mutation ABCB11 , encoding BSEP, underlay progressive familial intrahepatic cholestasis (PFIC)—or “neonatal hepatitis” suggesting PFIC—that was associated with HCC studied 11 cases pediatric setting PFIC or PFIC. Archival liver were retrieved immunostained for...
The aim of the study was to investigate toxic effects aflatoxin B₁ (AFB₁) and efficacy a probiotic preparation containing L. reuteri, plantarum, pentosus, rhamnosus paracasei Saccharomyces cerevisiae yeasts ameliorate their in broiler chickens. A total 168 one-day-old female Ross 308 broilers were randomly allocated six groups. Three wheat soybean meal-based diets prepared: Control diet contaminated with 1 or 5 mg/kg AFB₁ supplied moldy wheat. All unsupplemented supplemented probiotic, cold...
Respiratory tract infections constitute the most frequent manifestation of X-linked agammaglobulinemia (XLA). There are not many papers elucidating gastrointestinal (GI) disorders, including inflammatory bowel disease (IBD), in such patients. The aim study was to evaluate occurrence disorders and IBD compared respiratory XLA individuals.Of 1563 patients with primary immunodeficiencies diagnosed Department Immunology, Children's Memorial Health Institute (CMHI), 66 boys had a provisional...
Objective: To evaluate the clinical utility of panel-based NGS in diagnostic approach monogenic cholestatic liver diseases. Study design: Patients with diagnosis chronic disease an unknown etiology underwent targeted genes panel. Group 1 included five patients (prospectively recruited) hospitalized from January to December 2017 while group 2 seventeen (retrospectively 2010 presenting low-GGT PFIC phenotype (group 2a, 11 patients) or indeterminant cirrhosis 2b, 6 patients). Results: Among 22...
To investigate IL-18 mRNA expression in the gastric mucosa Helicobacter pylori-infected children and its association with macrophage infiltration, IL-8, IL-1 beta expression.From 39 children, blood samples were taken for gene polymorphism analysis antral biopsies obtained histology (including immunostaining), culture semiquantitative of IL-18, beta, CD14 by reverse transcription-PCR (RT-PCR). RT-PCR was used H. pylori ureA cagA detection tissue.H. patients had significantly higher transcript...
In Wilson disease (WD), liver copper (Cu) excess, caused by mutations in the ATPase Cu transporting beta (ATP7B), has been extensively studied. contrast, gastrointestinal tract, responsible for dietary uptake, ATP7B malfunction is poorly explored. We therefore investigated gut biopsies from WD patients and compared intestines two rodent models human knock-out intestinal cells to their respective wild-type controls. observed (GI) inflammation patients, rats mice lacking ATP7B. Mitochondrial...
Background and rationale for the study. The aim of study was to determine prognostic value histopathological findings with special care severity liver fibrosis at moment hepatoportoenterostomy (HPE) in children biliary atresia (BA). We performed analysis 142 wedge biopsies taken time HPE. All patients were operated by same surgical team between 1995 2007. According outcome 6 months after HPE divided into groups: group 1-bilirubin level < 2 mg% (n = 65), 2-bilirubin > 77). Liver re-evaluated...
Abstract: Nonalcoholic steatohepatitis (NASH) is the most severe form of non‐alcoholic fatty liver disease (NAFLD). The aim our study was to highlight NASH as a rare but possible problem in children. We present case 13‐yr‐boy with well‐established diagnosis cirrhosis secondary NASH, who underwent orthotopic transplantation (OLT) at age 13 years. Six months after recurrence graft diagnosed. In treatment metformin used good effect.
of HBsAg expression in hepatocytes.Inflammatory infiltrates accompanied the focal HCV antigens.A direct correlation between antigen and inflammatory reaction situ was noted more often hepatitis C than B. CONCLUSION: Irrespective etiology activity hepatitis, components infiltrate liver were similar.Overall did not correlate with HCVAg; HBcAg expression, however, chronic B moderate severe activity.
DNA fragmentation factor 40 (DFF40) is a key executor of apoptosis. It localizes to the nucleus together with 45 (DFF45), which acts as DFF40 inhibitor and chaperone. B-cell lymphoma (Bcl-2) protein proven antiapoptotic present in cytoplasm. In this study, we aimed investigate DFF40, DFF45, Bcl-2 immunoexpression endometrial polyps (EPs) benign hyperplasia (BEH) tissue compared that normal proliferative endometrium (NPE) secretory (NSE) well post menopausal (NAE). This study used archived...
Functioning farnesoid X receptor (FXR; encoded by NR1H4) is key to normal bile acid homeostasis. Biallelic mutations in NR1H4 are reported a few children with intrahepatic cholestasis. We describe boy progressive familial cholestasis and homozygous mutation NR1H4.A had severe neonatal moderate hypercholanemia persistently elevated alpha-fetoprotein. Despite medical treatment, coagulopathy was uncontrollable, prompting liver transplantation at age 8 mo incidental splenectomy. The patient...
pathologists differ in their assessment of grading and staging liver biopsies; (2) inter-observer variability for is lower than that grading; (3) regardless the assessments, majority children with chronic HBV infection have mild to moderate inflammation fibrosis.
Background:Amoxicillin/clavulanic acid-induced liver injury is a well recognized complication. Presentation and outcome may vary, which related to individual maturity, genetic predisposition, enzyme heterogeneity, intensity of treatment, drug interactions. In most cases withdrawing the sufficient treatment; however, progressive damage leading transplantation have been reported.Case Report:We present case an 8-year-old patient after who developed induced (DILI) amoxicillin/clavulanic acid...
Liver biopsy is a well-known method for the diagnosis and evaluation of chronic diffuse liver diseases, especially among patients with "hepatopathy unknown origin".In years 2014-2015 we performed 259 biopsies in 28 (22 females, 6 males, aged 18-65 years, mean: 45 years) an initial origin". The these were revised by two independent pathologists.Histopathological features autoimmune conditions found 11 cases, steatohepatitis with/without Mallory bodies 7, simple steatosis without inflammation...
ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Lipiński P, Cielecka-Kuszyk J, Socha Tylki-Szymańska A. Liver involvement in NGLY1 congenital disorder of deglycosylation. Polish Journal Pathology. 2020;71(1):66-68. doi:10.5114/pjp.2020.92994. APA Lipiński, P., Cielecka-Kuszyk, J., Socha, & Tylki-Szymańska, (2020). Pathology, 71(1), 66-68. https://doi.org/10.5114/pjp.2020.92994 Chicago Patryk, Joanna Piotr and Anna Tylki-Szymańska. 2020. "Liver...
Introduction: Inborn errors of primary bile acid (BA) synthesis are rare autosomal recessive disorders responsible for 1–2% cases neonatal cholestasis. Among them, cerebrotendinous xanthomatosis (CTX) is caused by mutations in the CYP27A1 gene resulting impairment sterol 27-hydroxylase enzyme activity. Patients and Methods: Here we present study on two siblings with cholestasis diagnosed deficiency. The clinical, biochemical, histological, molecular presentation at time diagnosis detailed...
BackgroundCongenital disorders of glycosylation (CDG) result from defects in the synthesis glycans and their attachment to proteins lipids. Histologically, liver steatosis, fibrosis cirrhosis have been reported CDG.The aim study was characterize histopathological ultrastructural changes CDG patients hospitalized our Institute, find most characteristic features, as articles concerning microscopic features are sparse.MethodsOut 32 diagnosed followed-up biopsy performed 4 them, including 2 with...
Figure 1.. Liver biopsies after LTx – Case 1. (A) Mixed macro- and microvesicular steatosis (95%) portal tract without fibrosis, ductular proliferation (H&E staining). (B) (70%), periportal fibrosis (Azan (C) (40%), proliferation, mild inflammatory infiltrates (D) macro-and (30%), fibrous septa, filtrates
ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Janowski K, Goliszek M, Cielecka-Kuszyk J, Jankowska I, Pawłowska J. Congenital hepatic fibrosis in a 9-year-old female patient – case report. Clinical and Experimental Hepatology. 2017;3(3):176-179. doi:10.5114/ceh.2017.70299. APA Janowski, K., Goliszek, M., Cielecka-Kuszyk, J., Jankowska, I., & Pawłowska, (2017). Hepatology, 3(3), 176-179. https://doi.org/10.5114/ceh.2017.70299 Chicago Kamil, Maria Joanna...
1. McCarthy MI, Hattersley AT. Molecular diagnostic in monogenic and multifactorial forms of type 2 diabets. Expert Rev Mol Diagn. 2001; 1: 403–412. CrossRef Google Scholar
ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Cielecka-Kuszyk J, Lipiński P, Szymańska S, Ismail H, Jankowska I. Long-term follow-up in children with progressive familial intrahepatic cholestasis type 2 after partial external biliary diversion focus on histopathological features. Polish Journal of Pathology. 2019;70(2):79-83. doi:10.5114/pjp.2019.87099. APA Cielecka-Kuszyk, J., Lipiński, P., Szymańska, S., Ismail, H., & Jankowska, (2019). Pathology, 70(2),...
Lysosomal acid lipase (LAL) deficiency (LAL-D) is a lysosomal lipid storage disorder in which the accumulation of cholesteryl esters and triglycerides predominantly hepatocytes cells macrophage-monocyte system observed. The disturbance synthesis trafficking cholesterol other lipids (triglycerides as well phospholipids) systemic lipoprotein dysregulation, reflects pathophysiology LAL-D. aim this study was to present occurrence macrophage derived structures LAL-D patient, provide an overview...
Biliary atresia (BA) is a severe cholangiopathy of newborns with fibrotic obliteration the intrahepatic and extrahepatic bile ducts resulting in progressive liver injury. This most common cause neonatal cholestasis major indication for transplantation children. The introduction Kasai hepatportoenterostomy (HPE) dramatically changed fatal prognosis BA, if surgery performed within first weeks life, there chance delay or even avoidance up to 10% 20% patients. coexistence inborn anomalies,...
The diagnosis of celiac disease (CD) at the first diagnostic step requires detection specific class A antibodies to tissue transglutaminase type-2 (TG2 IgA) and measurement total immunoglobulin (tIgA) exclude IgA deficiency. aim study was evaluate new quantitative immunoassay panel allowing for celiac-specific with simultaneous determination tIgA from same sample blood one time. This retrospective included 104 pediatric patients divided into groups recognized CD deficiency (n = 20; 19%),...