Publications by authors named "Teofila KsiAZek"

Background: A personalised approach to the treatment of acute myeloid leukemia (AML) in children and adolescents, as well as the development of supportive therapies, has significantly improved survival. Despite this, some patients still die before starting treatment or in an early phase of therapy before achieving remission. The study analysed the frequency, clinical features and risk factors for early deaths (ED) and treatment related deaths (TRD) of children and adolescents with AML.

View Article and Find Full Text PDF

Background: The reports of studies that compare the survival of adolescents and young adults with younger children with acute myeloid leukemia (AML) are contradictory.

Patients And Methods: We retrospectively analyzed 220 AML patients aged 0-18 years treated in pediatric oncologic centers in Poland from 2015 to 2022. The evaluated group included 31 infants (below 1 year), 91 younger children (1-9.

View Article and Find Full Text PDF

Background: Gemtuzumab ozogamicin (GO), one of the first targeted drugs used in oncology, consists of an anti-cluster of differentiation 33 (CD33) monoclonal antibody bound to a derivative of cytotoxic calicheamicin. After the drug withdrawn in 2010 due to a significantly higher rate of early deaths, GO regained approval in 2017 for the treatment of newly diagnosed, refractory, or relapsed acute myeloid leukemia (AML) in adults and children over 15 years of age. The objective of the study was a retrospective analysis of clinical characteristics, treatment outcomes, and GO toxicity profile in children with primary refractory or relapsed (R/R) AML treated in Poland from 2008 to 2022.

View Article and Find Full Text PDF
Article Synopsis
  • The FLT3 gene mutation occurs in 10-15% of pediatric acute myeloid leukemia (AML) cases and is linked to poorer outcomes in patients.
  • A study analyzed data from 2005 to 2022, revealing that FLT3-ITD-positive patients had significantly lower survival rates compared to those without the mutation.
  • Results indicated that treatment advancements, like FLT3 inhibitors and stem cell transplants, improved survival rates, while additional genetic mutations (WT1 and NPM1) impacted prognosis significantly.
View Article and Find Full Text PDF

Background: Numerous studies have shown that in Crohn's disease (CD), the gut microbiota is of great importance in the induction and maintenance of inflammation in the gastrointestinal tract. Until recently, studies have focused almost exclusively on bacteria in the gut. Lately, more attention has been paid to the role of intestinal fungi.

View Article and Find Full Text PDF

Acute P./myeloid leukemia post cytotoxic therapy (AML-pCT) is rare complication of cancer treatment in childhood. The objective of the study was to identify clinical characteristics and provide an analysis of the outcomes in pediatric AML-pCT.

View Article and Find Full Text PDF

Measurable residual disease (MRD) is a well-known tool for the evaluation of the early response to treatment in patients with acute lymphoblastic leukemia (ALL). In respect to predicting the relapse the most informative cut-off and time point of MRD measurement during therapy were evaluated in our study. Between 1 January 2013 and 31 December 2019, multiparametric flow cytometry (MFC) MRD was measured in the bone marrow of 140 children with ALL treated according to the ALL IC-BFM2009 protocol.

View Article and Find Full Text PDF

This is the first report of the concurrent development of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) and Crigler-Najjar syndrome type 1 (CNs1) inherited via uniparental disomy of chromosome 2, which are both autosomal recessive pathologies. Through an expanded newborn metabolic panel, a male infant was identified as having an acylcarnitine pattern typical for LCHADD, later confirmed to be caused by a well-characterized pathogenic variant in the HADHA gene located at 2p23. Prolonged non-hematologic jaundice requiring repetitive phototherapy prompted further genetic analysis, leading to the identification of another genetic abnormality consistent with CNs1, which was caused by a novel pathogenic variant in the UGT1A1 gene located at 2q37.

View Article and Find Full Text PDF
Article Synopsis
  • From 1983 to 2019, Poland implemented standardized protocols for treating pediatric acute myeloid leukemia (AML), analyzing treatment results from 899 children over various time periods.
  • Survival rates improved significantly, with three-year overall survival increasing from 34% to 75% across the different therapeutic protocols, along with reductions in early deaths and deaths in remission.
  • Advances in treatment strategies, focusing on genetic factors and supportive care, contributed to better outcomes, particularly for patients with challenging genetic profiles in the most recent treatment period.
View Article and Find Full Text PDF
Article Synopsis
  • C1-inhibitor deficiency (C1-INH-HAE) type I and II is a rare genetic disorder linked to mutations in the SERPING1 gene, leading to severe health risks.
  • A study analyzed genetic variations in the SERPING1 gene among 41 Polish patients with C1-INH-HAE, revealing that 82.9% had mutations in various gene regions, primarily in exon 3 and exon 8.
  • Despite identifying 20 different mutations, including 9 novel variants, no clear correlation was found between mutation characteristics and patient phenotypes, such as family history or severity of the disease.
View Article and Find Full Text PDF

11q23/ rearrangements are frequently detected in pediatric acute myeloid leukemia. The analysis of their clinical significance is difficult because of the multitude of translocation fusion partners and their low frequency. The presence of t(10;11)(p12;q23) translocation was previously identified in pediatric acute myelogenous leukemia (AML).

View Article and Find Full Text PDF

Children with Down syndrome (DS) have increased risk of myeloid leukemia (ML), but specific treatment protocols ensure excellent outcome. This study was a retrospective analysis of the treatment results and genetic characteristics of ML of DS (ML-DS) in Poland from 2005 to 2019. All 54 patients with ML-DS registered in the Polish Pediatric Leukemia and Lymphoma Study Group in analyzed period were enrolled to the study.

View Article and Find Full Text PDF
Article Synopsis
  • The study investigates how exposure to high oxygen levels affects retinal gene expression in mice with a model of oxygen-induced retinopathy (OIR), which resembles retinopathy of prematurity.
  • Mice were subjected to 75% oxygen for 5 days, and their retinal samples were analyzed for gene expression to identify changes during neovascularization and regression phases.
  • Key findings highlight the enrichment of specific signaling pathways, including those related to apoptosis and immune responses, suggesting these pathways play a significant role in the disease's development and progression.
View Article and Find Full Text PDF
Article Synopsis
  • This study investigates gene expression patterns in blood and retina of mice with oxygen-induced retinopathy (OIR), a model for retinopathy of prematurity, to better understand the disease and identify potential blood biomarkers.
  • 120 C57BL/6J mice were used, divided into an OIR group exposed to high oxygen levels and a control group, with gene expression analyzed from blood and retinal cells over a period of 28 days using microarrays.
  • Results revealed significant changes in gene expression, especially in the retina, with specific genes (Deptor and Nol4) showing decreased expression in both blood and retina on day 17, suggesting they could be linked to retinal neovascularization and warrant further exploration.
View Article and Find Full Text PDF

Background: Bronchopulmonary dysplasia (BPD) is a common cause of abrupted lung development after preterm birth. BPD may lead to increased rehospitalization, more severe and frequent respiratory infections, and life-long reduced lung function. The gene regulation in lungs with BPD is complex, with various genetic and epigenetic factors involved.

View Article and Find Full Text PDF

Aim Of The Study: Neuroblastoma (NBL) is one of the most common extracranial tumours occurring in children with gene amplification, acknowledged as a marker of poor prognosis. We assessed the frequency of amplification and its impact on NBL markers and on the treatment outcome.

Material And Methods: Among 160 children with NBL treated from 1991 to 2015 in one centre 140 patients had known gene status, and they were enrolled in the study.

View Article and Find Full Text PDF

To examine the gene expression regarding pulmonary vascular disease in experimental bronchopulmonary dysplasia in young mice. Premature delivery puts babies at risk of severe complications. Bronchopulmonary dysplasia (BPD) is a common complication of premature birth leading to lifelong affection of pulmonary function.

View Article and Find Full Text PDF
Article Synopsis
  • - The study aimed to analyze the plasma proteomes in cord blood samples from preterm infants of varying gestational ages to find proteins that may be linked to complications at birth.
  • - Preterm infants were divided into three groups based on their gestational age and compared to healthy, full-term newborns regarding their plasma protein levels.
  • - Results indicated that preterm delivery alters the abundance of various proteins related to inflammation, coagulation, and immune function, suggesting key pathways that could be targeted for new treatments.
View Article and Find Full Text PDF
Article Synopsis
  • The study aimed to assess how gene expression in preterm neonates changes over time and how factors like development and environment affect this expression after birth.
  • Blood samples were taken from full-term and preterm newborns at birth and again at 36 weeks postmenstrual age to analyze whole genome expression.
  • Results showed that preterm infants have a different gene expression profile than term infants, with many genes down-regulated at birth and changing as the newborn matures.
View Article and Find Full Text PDF

We present a case of autologous bone marrow recovery after allogeneic hematopoietic stem cell transplantation (HSCT) in a 7-year old girl who was treated due to acute myelocytic leukemia. First complete remission is lasting for 81 months after the allo-HSCT. Presented case constitutes an exceptional clinical situation and it indicates that diagnosis of leukemia relapse should be cautiously considered once the autologous bone marrow recovery is observed after allogeneic HSCT.

View Article and Find Full Text PDF

The aim of the paper is to present the initial results of molecular examination which was started in 2006 for children with acute myeloid leukemia. Better knowledge of biology of this disease, can result in establishing of new risk factors what allows more precise patient stratification to different therapeutic groups. Study was obtained patients until to 18 years of age treated according to AML-BFM 2004 INTERIM protocol in 14 centers of the Polish Pediatric Leukemia/Lymphoma Study Group.

View Article and Find Full Text PDF