Constitutional mismatch repair deficiency (CMMRD) syndrome, caused by biallelic mutations in mismatch repair genes, is one of the most aggressive hereditary cancer syndromes. This report presents the clinical course of two brothers diagnosed with CMMRD. The first patient was diagnosed with T-cell lymphoma at the age of three and a half years, a relapse, and synchronous glioblastoma at the age of seven and a half years.
View Article and Find Full Text PDFCD19-targeted CAR-T cell therapy has revolutionized the treatment of relapsed/refractory (r/r) pre-B acute lymphoblastic leukemia (ALL). However, it can be associated with acute toxicities related to immune activation, particularly cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). Cytokines released from activated immune cells play a key role in their pathophysiology.
View Article and Find Full Text PDFThe optimal bridging therapy before CAR-T cell infusion in pediatric relapsed or refractory B-cell precursor acute lymphoblastic leukemia (r/r BCP-ALL) still remains an open question. The administration of blinatumomab prior to CAR-T therapy is controversial since a potential loss of CD19+ target cells may negatively impact the activation, persistence, and, as a consequence, the efficacy of subsequently used CAR-T cells. Here, we report a single-center experience in seven children with chemorefractory BCP-ALL treated with blinatumomab before CAR-T cell therapy either to reduce disease burden before apheresis (six patients) or as a bridging therapy (two patients).
View Article and Find Full Text PDFHematopoietic stem cell transplantation (HSCT) is a curative therapy for an increasing number of nonmalignant indications. Its use is restricted by severe transplant-related complications, including CMV infection; despite various prophylactic and therapeutic strategies, CMV reactivation has remarkable morbidity and mortality. The analysis included 94 children with nonmalignant disorder who underwent allogeneic HSCT in the Department of Pediatric Hematology, Oncology, and Bone Marrow Transplantation in Wrocław during years 2016-2020.
View Article and Find Full Text PDFThe coronavirus disease 2019 pandemic has made us adjust our standards and cope with unpredictable circumstances affecting the whole world, including the medical field. A 2-year-old boy diagnosed with X-linked lymphoproliferative disease type 2 with concomitant positive polymerase chain reaction test for Epstein-Barr virus-DNA was admitted to our transplant ward. His treatment scheme had to be modified at the last moment because of a donor disqualification due to a positive polymerase chain reaction result for severe acute respiratory syndrome coronavirus 2 just before the apheresis.
View Article and Find Full Text PDFBackground: Kidney diseases are the main causative factors of secondary hypertension (HTN) in children. Although primary HTN is less common in the pediatric population, its increasing prevalence, especially among teenagers, makes early diagnosis an emerging issue.
Objectives: To analyze the potential differences between primary HTN and HTN secondary to renal diseases, in order to tailor diagnostic procedures to pediatric patients with suspicion of HTN.
Background: The effect multiple sclerosis (MS) has on the social functioning and integration of patients has been recently considered as an important factor of the disease.
Objectives: To assess social participation of MS patients with regard to demographic and disease-related variables.
Material And Methods: The study comprised 201 MS patients: 140 women, 61 men, aged 24-69 years.
Objective: To assess the type and frequency of early postoperative complications in patients after open surgical infrarenal aneurysms repair and determination of their risk factors.
Study Design: An observational study Place and Duration of Study: The 4th Military Teaching Hospital in Wroclaw, Poland, from January 2012 to December 2016.
Methodology: Analyses of medical records of 205 patients treated for infrarenal aneurysm with open surgery.
The way that bad news is disclosed to a cancer patient has a crucial impact on physician-patient cooperation and trust. Consensus-based guidelines provide widely accepted tools for disclosing unfavorable information. In oncology, the most popular one is called the SPIKES protocol.
View Article and Find Full Text PDFBackground: Peripheral arterial disease (PAD) affects 3%-10% of the population before the age of 70 years and 15%-20% after that age.The aim of the study was to compare the incidence of complications and secondary interventions in patients who underwent each type of treatment.
Methods: We analyzed 734 medical records of the Department of Surgery at the 4th Military Teaching Hospital in Wroclaw, In total, 394 were operated on with open surgery; an endarterectomy (59.