Publications by authors named "Wojciech Młynarski"

288 Publications

Successful Salvage Haploidentical Bone Marrow Transplantation in a Child With Hemophagocytic Lymphohistiocytosis, When the Previously Matched Unrelated Donor Tested Positive for SARS-CoV-2 on the Day of Stem Cells Collection.

Transplant Proc 2021 Apr 27. Epub 2021 Apr 27.

Department of Pediatric Bone Marrow Transplantation, Oncology, and Hematology, Wroclaw Medical University, Wroclaw, Poland. Electronic address:

The 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. We decided to perform salvage haploidentical bone marrow transplant from the patient's mother because it was the only possible option. Now, in a 5-month observation period after the hematopoietic stem cell transplantation, our patient is in good general condition. His case convinced us to redirect our approach to transplant procedure preparation. Following the European Group of Blood and Marrow Transplantation recommendations, we use cryopreserved apheresis materials to ensure the availability of stem cell products before the start of a conditioning regimen.
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http://dx.doi.org/10.1016/j.transproceed.2021.04.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8078905PMC
April 2021

Neutrophil Elastase Defects in Congenital Neutropenia.

Front Immunol 2021 22;12:653932. Epub 2021 Apr 22.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.

Severe congenital neutropenia (SCN) is a rare hematological condition with heterogenous genetic background. Neutrophil elastase (NE) encoded by gene is mutated in over half of the SCN cases. The role of NE defects in myelocytes maturation arrest in bone marrow is widely investigated; however, the mechanism underlying this phenomenon has still remained unclear. In this review, we sum up the studies exploring mechanisms of neutrophil deficiency, biological role of NE in neutrophil and the effects of mutation and neutropenia pathogenesis. We also explain the hypotheses presented so far and summarize options of neutropenia therapy.
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http://dx.doi.org/10.3389/fimmu.2021.653932DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8100030PMC
April 2021

Ovarian carcinoma in children with constitutional mutation of SMARCA4: single-family report and literature review.

Fam Cancer 2021 Apr 28. Epub 2021 Apr 28.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, ul. Sporna 36/50, 91-738, Lodz, Poland.

Ovarian carcinoma is an extremely rare malignancy in children, often developing on the underlying inherited background. Female carriers of pathogenic germline mutations of SMARCA4 are at risk of an aggressive type of undifferentiated ovarian cancer called small cell carcinoma of the ovary, hypercalcemic type (SCCOHT). Regardless of age of the patient, stage of the disease, and oncological treatment, the prognosis for SCCOHT is poor. Therefore, early intervention with risk-reducing surgeries is recommended for these patients. In this study, we report genetic testing of a family with two children carrying pathogenic germline mutations of SMARCA4 and summarize the course of SCCOHT in all pediatric patients reported in the literature with constitutional defects identified within the SMARCA4 locus.
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http://dx.doi.org/10.1007/s10689-021-00258-wDOI Listing
April 2021

Maintenance Therapy With Everolimus for Subependymal Giant Cell Astrocytoma in Patients With Tuberous Sclerosis - Final Results From the EMINENTS Study.

Front Neurol 2021 9;12:581102. Epub 2021 Apr 9.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.

The aim of this EMINENTS prospective, single-center, open-label, single-arm study was to evaluate the cumulative efficacy and safety of reduced doses of everolimus (maintenance therapy) in patients with tuberous sclerosis and subependymal giant cell astrocytoma (SEGA). The trial included 15 patients who had undergone at least 12 months of treatment with a standard everolimus dose. The dose of everolimus was reduced to three times a week, with a daily dose as in standard regimen. Data of 14 patients were analyzed. SEGA volume (SV) was evaluated at study entry and subsequent time points by an experienced radiologist. Adverse events (AEs) noted during maintenance therapy were compared to the AEs of standard dose period. Patients were followed over a mean duration 58.37 months (95%CI: 45.95-70.78). The differences in SEGA volume between subsequent time points (0, 3, 6,12, 18, 24, 36, 48, and 60 months) were not statistically significant ( = 0.16). At the end of the study, 7 out of 10 patients had stable SEGA volume. No clinical symptoms of progression were observed in any patients. No patient or tumor-related risk factors of progression were identified. Regarding AEs, infections (stomatitis, bronchitis, diarrhea) and laboratory abnormalities (neutropenia, anemia, hyperglycemia) occurred less frequently during maintenance therapy compared to the standard dose regimen. Final results from EMINENTS study confirm that maintenance therapy with everolimus might represent a rational therapeutic option for patients TSC and SEGA after effective full dose treatment. It could be an option for patients who experienced everolimus-related AEs, instead of discontinuation of therapy. Careful evaluation of possible progression, especially concerning first six months of maintenance therapy should be advised. www.drks.de, identifier DRKS00005584.
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http://dx.doi.org/10.3389/fneur.2021.581102DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8062974PMC
April 2021

Clinical heterogeneity among pediatric patients with autoimmune type 1 diabetes stratified by immunoglobulin deficiency.

Pediatr Diabetes 2021 Apr 10. Epub 2021 Apr 10.

Department of Pediatrics, Oncology, and Hematology, Medical University of Łódź, Łódź, Poland.

Background: Type 1 diabetes (T1D) may coexist with primary immunodeficiencies, indicating a shared genetic background.

Objective: To evaluate the prevalence and clinical characteristics of immunoglobulin deficiency (IgD) among children with T1D.

Methods: Serum samples and medical history questionnaires were obtained during routine visits from T1D patients aged 4-18 years. IgG, IgA, IgM, and IgE were measured by nephelometry and enzyme-linked immunosorbent assay (ELISA). IgG and IgM deficiency (IgGD, IgMD) were defined as IgG/IgM >2 standard deviations (SD) below age-adjusted mean. IgE deficiency was defined as IgE <2 kIU/L. IgA deficiency (IgAD) was defined as IgA >2 SD below age-adjusted mean irrespective of other immunoglobulin classes (absolute if <0.07 g/L, partial otherwise) and as selective IgAD when IgA >2 SD below age-adjusted mean with normal IgG and IgM (absolute if <0.07 g/L, partial otherwise).

Results: Among 395 patients (53.4% boys) with the median age of 11.2 (8.4-13.7) and diabetes duration 3.6 (1.1-6.0) years, 90 (22.8%) were found to have hypogammaglobulinemia. The IgGD and IgAD were the most common each in 40/395 (10.1%). Complex IgD was found in seven patients. Increased odds of infection-related hospitalization (compared to children without any IgD) was related to having any kind of IgD and IgAD; OR (95%CI) = 2.1 (1.2-3.7) and 3.7 (1.8-7.5), respectively. Furthermore, IgAD was associated with having a first-degree relative with T1D OR (95%CI) = 3.3 (1.4-7.6) and suffering from non-autoimmune comorbidities 3.3 (1.4-7.6), especially neurological disorders 3.5 (1.2-10.5).

Conclusions: IgDs frequently coexist with T1D and may be associated with several autoimmune and nonimmune related disorders suggesting their common genetic background.
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http://dx.doi.org/10.1111/pedi.13208DOI Listing
April 2021

Vaccination in children with chronic severe neutropenia - review of recommendations and a practical approach.

Cent Eur J Immunol 2020 27;45(2):202-205. Epub 2020 Jul 27.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Poland.

While the management of childhood neutropenia associated with a modifiable factor should be appropriate for the primary cause, there are misconceptions regarding the management of severe congenital neutropenia, immune neutropenia and cases classified as "idiopathic". Antibiotic prophylaxis or granulocyte-colony stimulating factor (G-CSF) are prescribed by specialists in pediatric hematology or immunology, whereas immunization may be conducted by primary care physicians should clear recommendations by provided. There is a belief that severe neutropenia, as an immunodeficiency, is associated with compromised effectiveness and increased rate of complications of immunization. The immunization might be delayed or omitted, increasing the risk of unnecessary infection. We discuss the available data and recommendations regarding vaccination of children with chronic severe neutropenia. While there are virtually no studies addressing the safety and effectiveness of vaccination in neutropenia, expert opinions provide information on immunization policy in "phagocytic cells defects" or explicitly neutropenia. There are no contraindications for inactivated vaccines in neutropenia. Live bacterial vaccines are contraindicated. While in general the vaccination with live viral vaccines is encouraged, occasionally neutropenia might be associated with defects of adaptive immunity, which would preclude the administration of such vaccines. Although this should be easily phenotypically identified, we propose assessing immunoglobulin levels and performing a low-cost flow cytometry test for major lymphocyte subpopulations to exclude significant defects in adaptive immunity before administration of live viral vaccines to such patients. This can improve the adherence of patients' guardians and physicians to proposed vaccination policy and the professional and legal safety associated with the procedure.
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http://dx.doi.org/10.5114/ceji.2020.97910DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7792435PMC
July 2020

Clinical Outcome in Pediatric Patients with Philadelphia Chromosome Positive ALL Treated with Tyrosine Kinase Inhibitors Plus Chemotherapy-The Experience of a Polish Pediatric Leukemia and Lymphoma Study Group.

Cancers (Basel) 2020 Dec 13;12(12). Epub 2020 Dec 13.

Department of Pediatrics, Oncology & Hematology, Medical University of Lodz, 90-647 Lodz, Poland.

The treatment of children with Philadelphia chromosome positive acute lymphoblastic leukemia (ALL Ph+) is currently unsuccessful. The use of tyrosine kinase inhibitors (TKIs) combined with chemotherapy has modernized ALL Ph+ therapy and appears to improve clinical outcome. We report herein the toxicity events and results of children with ALL Ph+ treated according to the EsPhALL2010 protocol (the European intergroup study of post-induction treatment of Philadelphia chromosome positive ALL) in 15 hemato-oncological centers in Poland between the years 2012 and 2019. The study group included 31 patients, aged 1-18 years, with newly diagnosed ALL Ph+. All patients received TKIs. Imatinib was used in 30 patients, and ponatinib was applied in one child due to T315I and M244V mutation. During therapy, imatinib was replaced with dasatinib in three children. The overall survival of children with ALL Ph+ treated according to the EsPhALL2010 protocol was 74.1% and event-free survival was 54.2% after five years. The cumulative death risk of the study group at five years was estimated at 25.9%, and its cumulative relapse risk was 30%. Our treatment outcomes are still disappointing compared to other reports. Improvements in supportive care and emphasis placed on the determination of minimal residual disease at successive time points, which will impact decisions on therapy, may be required.
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http://dx.doi.org/10.3390/cancers12123751DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7763070PMC
December 2020

Nivolumab for the Treatment of Advanced Pediatric Malignancies.

Anticancer Res 2020 Dec;40(12):7095-7100

Department of Pediatric, Hematology and Oncology, Nicolaus Copernicus University Torun, Jurasz University Hospital, Collegium Medicum, Bydgoszcz, Poland.

Background/aim: Nivolumab is an immune checkpoint inhibitor with high antitumor activity in selected neoplasms. The aim of the study was to evaluate the efficacy and safety of nivolumab in pediatric patients with various types of highly malignant advanced tumors.

Patients And Methods: Ten patients with a median age of 15.1 years were included in the study. The indications for treatment were: malignant skin melanoma (n=5), brain tumor (n=2), malignant melanoma of the brain (n=1), Hodgkin lymphoma (n=1) and soft tissue sarcoma (n=1).

Results: Complete disease remission was observed in 4 patients. Overall survival at 24 months from diagnosis for the entire group was 0.36. Two patients receiving combination therapy of nivolumab and ipilimumab did not achieve a remission. Adverse events of immunotherapy were observed in 4/10 patients.

Conclusion: Nivolumab is a promising option in pediatric advanced malignancies. Treatment with immunotherapy was relatively well tolerated, and emerging side-effects were manageable.
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http://dx.doi.org/10.21873/anticanres.14738DOI Listing
December 2020

Results of two consecutive treatment protocols in Polish children with acute lymphoblastic leukemia.

Sci Rep 2020 11 19;10(1):20168. Epub 2020 Nov 19.

Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Antoni Gębala Street 6, 20-093, Lublin, Poland.

The aim of the study was to retrospectively compare the effectiveness of the ALL IC-BFM 2002 and ALL IC-BFM 2009 protocols and the distribution of risk groups by the two protocols after minimal residual disease (MRD) measurement as well as its impact on survival. We reviewed the medical records of 3248 patients aged 1-18 years with newly diagnosed ALL who were treated in 14 hemato-oncological centers between 2002 and 2018 in Poland. The overall survival (OS) of 1872 children with ALL treated with the ALL IC 2002 protocol was 84% after 3 years, whereas the OS of 1376 children with ALL treated with the ALL IC 2009 protocol was 87% (P < 0.001). The corresponding event-free survival rates were 82% and 84% (P = 0.006). Our study shows that the ALL IC-BFM 2009 protocol improved the results of children with ALL compared to the ALL IC-BFM 2002 protocol in Poland. This analysis confirms that MRD marrow assessment on day 15 of treatment by FCM-MRD is an important predictive factor.
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http://dx.doi.org/10.1038/s41598-020-75860-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7678856PMC
November 2020

Next-Generation Sequencing in the Diagnosis of Patients with Bardet-Biedl Syndrome-New Variants and Relationship with Hyperglycemia and Insulin Resistance.

Genes (Basel) 2020 10 29;11(11). Epub 2020 Oct 29.

Department of Clinical Genetics, Medical University of Lodz, 92-213 Lodz, Poland.

Bardet-Biedl syndrome (BBS) is a rare autosomal recessively inherited disease with major clinical symptoms such as: obesity, retinal degeneration, polydactyly and renal abnormalities. The aim of the study was to assess the spectrum of gene variants among patients with BBS, identified on the basis of nationwide genetic studies of monogenic diabetes in Polish population. Out of 575 patients enrolled for genetic testing from February 2017 to July 2019, 25 patients with a clinical suspicion of BBS were selected. The identification of pathogenic variants was performed by using targeted next-generation sequencing (NGS) on Illumina NextSeq 550 platform involving the SureSelect assay (Agilent, Santa Clara, CA, USA). BBS was genetically confirmed in 10 of 25 suspected patients. In patients, 14 different variants were found in six genes, mainly in and gene, including two novel variants. A strong association between hyperglycemia and insulin resistance in patients and the presence of variants in gene was observed. Identification of 14 variants, including two new mutations using the NGS method, is the first molecular characteristic of Polish patients with Bardet-Biedl syndrome. It gives hope for earlier proper diagnosis of BBS in future patients selected from children with early childhood obesity and their medical multidisciplinary care.
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http://dx.doi.org/10.3390/genes11111283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7693916PMC
October 2020

Broad phenotypic spectrum of germ line 7p12.1 microdeletions encompassing the IKZF1 gene includes predisposition to acute lymphoblastic leukemia.

Genes Chromosomes Cancer 2021 Feb 18;60(2):79-87. Epub 2020 Nov 18.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.

Microdeletions of 7p12.1 encompassing the IKZF1 gene locus are rare, with few cases reported. The common phenotype includes intellectual disability, overgrowth, and facial dysmorphism accompanied, albeit rarely, by congenital anomalies. Haploinsufficiency of IKZF1 predisposes individuals to childhood acute lymphoblastic leukemia (ALL). In this study, we comprehensively analyzed the frequency of 7p12.1 deletions among 4581 Polish individuals who underwent chromosomal microarray testing for unexplained developmental delay, intellectual disability, and/or congenital anomalies. Two unrelated individuals (0.04%) with a de novo interstitial 7p12.1 microdeletion encompassing IKZF1 were identified. One developed ALL. Analysis of the incidence and the phenotype of constitutional 7p12.1 microdeletion, which based on the previously annotated patients data in public databases and literature reports, revealed 21 cases including five patients diagnosed with ALL.
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http://dx.doi.org/10.1002/gcc.22914DOI Listing
February 2021

Serum microRNA as indicators of Wolfram syndrome's progression in neuroimaging studies.

BMJ Open Diabetes Res Care 2020 11;8(2)

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.

Introduction: Patients with the ultra-rare Wolfram syndrome (WFS) develop insulin-dependent diabetes and progressive neurodegeneration. The aim of the study was to quantify microRNAs (miRNAs) in sera from patients with WFS, correlate their expression with neurological imaging over time and compare miRNA levels with those observed in patients with type 1 diabetes mellitus (T1DM).

Research Design And Methods: We quantified miRNA expression (Qiagen, Germany) in two groups of patients: with WFS at study entry (n=14) and after 2 years of follow-up and in 15 glycated hemoglobin-matched (p=0.72) patients with T1DM.

Results: We observed dynamic changes in the expression of multiple miRNAs in patients with WFS parallel to disease progression and in comparison to the T1DM patients group. Among miRNAs that differed between baseline and follow-up WFS samples, the level of 5 increased over time (miR-375, miR-30d-5p, miR-30e-30, miR-145-5p and miR-193a-5p) and was inversely correlated with macular average thickness, while the expression of 2 (let-7g-5p and miR-22-3p) decreased and was directly correlated with neuroimaging indicators of neurodegeneration.

Conclusions: Our findings show for the first time that serum miRNAs can be used as easily accessible indicators of disease progression in patients with WFS, potentially facilitating clinical trials on mitigating neurodegeneration.
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http://dx.doi.org/10.1136/bmjdrc-2020-001379DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7607591PMC
November 2020

SARS-CoV-2 M inhibitors and activity-based probes for patient-sample imaging.

Nat Chem Biol 2021 02 22;17(2):222-228. Epub 2020 Oct 22.

Department of Chemical Biology and Bioimaging, Wroclaw University of Science and Technology, Wroclaw, Poland.

In December 2019, the first cases of infection with a novel coronavirus, SARS-CoV-2, were diagnosed. Currently, there is no effective antiviral treatment for COVID-19. To address this emerging problem, we focused on the SARS-CoV-2 main protease that constitutes one of the most attractive antiviral drug targets. We have synthesized a combinatorial library of fluorogenic substrates with glutamine in the P1 position. We used it to determine the substrate preferences of the SARS-CoV and SARS-CoV-2 main proteases. On the basis of these findings, we designed and synthesized a potent SARS-CoV-2 inhibitor (Ac-Abu-DTyr-Leu-Gln-VS, half-maximal effective concentration of 3.7 µM) and two activity-based probes, for one of which we determined the crystal structure of its complex with the SARS-CoV-2 M. We visualized active SARS-CoV-2 M in nasopharyngeal epithelial cells of patients suffering from COVID-19 infection. The results of our work provide a structural framework for the design of inhibitors as antiviral agents and/or diagnostic tests.
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http://dx.doi.org/10.1038/s41589-020-00689-zDOI Listing
February 2021

Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome.

Clin Cancer Res 2021 Jan 20;27(2):575-584. Epub 2020 Oct 20.

Research Unit Pediatric Hematology and Immunology, Division of Pediatric Hematology-Oncology, Department of Pediatrics and Adolescent Medicine, Medical University Graz, Graz, Austria.

Purpose: Nijmegen breakage syndrome (NBS) is a DNA repair disorder with a high predisposition to hematologic malignancies.

Experimental Design: We describe the natural history of NBS, including cancer incidence, risk of death, and the potential effectiveness of hematopoietic stem cell transplantation (HSCT) in preventing both pathologies: malignancy and immunodeficiency.

Results: Among 241 patients with NBS enrolled in the study from 11 countries, 151 (63.0%) patients were diagnosed with cancer. Incidence rates for primary and secondary cancer, tumor characteristics, and risk factors affecting overall survival (OS) were estimated. The cumulative cancer incidence was 40.21% ± 3.5% and 77.78% ± 3.4% at 10 years and 20 years of follow-up, respectively. Most of the tumors = 95 (62.9%) were non-Hodgkin lymphomas. Overall, 20 (13.2%) secondary malignancies occurred at a median age of 18 (interquartile range, 13.7-21.5) years. The probability of 20-year overall survival (OS) for the whole cohort was 44.6% ± 4.5%. Patients who developed cancer had a shorter 20-year OS than those without malignancy (29.6% vs. 86.2%; < 10). A total of 49 patients with NBS underwent HSCT, including 14 patients transplanted before malignancy. Patients with NBS with diagnosed cancer who received HSCT had higher 20-year OS than those who did not (42.7% vs. 30.3%; = 0.038, respectively). In the group of patients who underwent preemptive transplantation, only 1 patient developed cancer, which is 6.7 times lower as compared with nontransplanted patients [incidence rate ratio 0.149 (95% confidence interval, 0.138-0.162); < 0.0001].

Conclusions: There is a beneficial effect of HSCT on the long-term survival of patients with NBS transplanted in their first complete remission of cancer.
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http://dx.doi.org/10.1158/1078-0432.CCR-20-2574DOI Listing
January 2021

Salivary immunoglobulin A level during steroids and chemotherapy treatment administered in remission induction phase among pediatric patients with acute lymphoblastic leukemia.

Medicine (Baltimore) 2020 Oct;99(42):e22802

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Sporna 36/50, 91-738 Lodz, Poland.

The agents used in the treatment of acute lymphoblastic leukaemia (ALL) might affect the oral health of cancer patients.The study aims to assess the changes in the levels of immunoglobulin A (IgA) in saliva and blood, during first 22 days of intensive chemotherapy of ALL in children.Saliva and blood samples were taken from 24 patients, including 13 boys and 11 girls (age range: 4 - 17 years) on days 1, 8 and 22 of treatment. The levels of immunoglobulin A and total protein were estimated in samples at each time-point. The distribution of the quantitative variables was assessed using the Shapiro-Wilk test. Non-parametric statistics were used to compare the levels of repeated measurements and post hoc non-parametric analysis was applied for between time-point comparisons.A constant relationship was found between the levels of Ig A in blood and saliva (r = 0.28; P = .031). No change in salivary IgA level was observed in the prednisone-only prephase, but it dropped significantly on day 22 (10.7+/-4.8 vs 9.6+/-6.4 vs 5.7+/-3.9 ng/mL; P = .04), when chemotherapy was given (anthracycline, vincristine, L-asparaginase).In blood, the total protein level decreased significantly between day 1 and 22 (6.2+/-0.4 vs 5.1+/-0.3 g/dL; P = .001). Lymphocyte count (per microliter) also decreased (2.12+/-0.8 vs 0.41+/-0.1 vs 1.08+/-0.5; P = .002). Four children suffered from oral mucositis graded 1 or higher between days 8 and 22.Chemotherapy given during the treatment of childhood ALL is associated with a reduction in the level of salivary immunoglobulin A. Prevention of the drop of salivary IgA may diminish the risk of occurrence of acute mucosal complications.
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http://dx.doi.org/10.1097/MD.0000000000022802DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7571880PMC
October 2020

Pulmonary Exacerbation of Undiagnosed Toxocariasis in Intensively-Treated High-Risk Neuroblastoma Patients.

Children (Basel) 2020 Oct 5;7(10). Epub 2020 Oct 5.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Sporna 36/50 St., 91-738 Lodz, Poland.

Toxocariasis is one of the most common zoonoses, with high seroprevalence in apparently healthy individuals. Neuroblastoma is an aggressive childhood cancer. The cure rates are improving due to dose-dense chemotherapy, progress in surgical practice, myeloablative therapy with autologous stem cell transplantation, and recently, anti-GD2 immunotherapy. This is associated with a burden of complications, some of which are relatively specific for neuroblastoma treatment. Based on previous reports of infection in high-risk neuroblastoma patients and cases of pulmonary exacerbation from our center in this disease, we propose that toxocariasis is a specific complication of intensive pediatric cancer treatment and advocate for active surveillance.
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http://dx.doi.org/10.3390/children7100169DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7600921PMC
October 2020

Genetic Association Study of IL2RA, IFIH1, and CTLA-4 Polymorphisms With Autoimmune Thyroid Diseases and Type 1 Diabetes.

Front Pediatr 2020 21;8:481. Epub 2020 Aug 21.

Department of Pediatrics, Endocrinology, Diabetology With Cardiology Division, Medical University of Bialystok, Bialystok, Poland.

Autoimmune thyroid diseases (AITDs) which include Graves' disease (GD) and Hashimoto's thyroiditis (HT) as well as type 1 diabetes (T1D) are common autoimmune disorders in children. Many genes are involved in the modulation of the immune system and their polymorphisms might predispose to autoimmune diseases development. According to the literature genes encoding IL2RA (alpha subunit of Interleukin 2 receptor), IFIH1 (Interferon induced with helicase C domain 1) and CTLA-4 (cytotoxic T cell antigen 4) might be associated with autoimmune diseases pathogenesis. The aim of the study was to assess the association of chosen single nucleotide polymorphisms (SNPs) of IL2RA, IFIH1, and CTLA-4 genes in the group of Polish children with AITDs and in children with T1D. We analyzed single nucleotide polymorphisms (SNPs) in the IL2RA region (rs7093069), IFIH1 region (rs1990760) and CTLA-4 region (rs231775) in group of Polish children and adolescents with type 1 diabetes ( = 194) and autoimmune thyroid diseases (GD = 170, HT = 81) and healthy age and sex matched controls for comparison ( = 110). There were significant differences observed between T1D patients and control group in alleles of IL2RA (rs7093069 T > C) and CTLA-4 (rs231775 G > A). In addition, the study revealed T/T genotype at the IL2RA locus (rs7093069) and G/G genotype at the CTLA-4 locus (rs231775) to be statistically significant more frequent in children with T1D. Moreover, genotypes C/T and T/T at the IFIH1 locus (rs1990760) were significantly more frequent in patients with T1D than in controls. We observed no significant differences between AITD patients and a control group in analyzed SNPs. In conclusion, we detected that each allele T of rs7093069 SNP at the IL2RA locus and G allele of rs231775 SNP at the CTLA-4 locus as well as C/T and T/T genotypes of rs1990760 SNP at the IFIH1 locus are predisposing in terms of T1D development. Thereby, we confirmed that IL2RA, IFIH1, and CTLA-4 gene locus have a role in T1D susceptibility. The analysis of selected SNPs revealed no association with AITDs in a group of Polish children and adolescents.
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http://dx.doi.org/10.3389/fped.2020.00481DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7473350PMC
August 2020

Evaluation of Three Lancing Devices: What Do Blood Volume and Lancing Pain Depend On?

J Diabetes Sci Technol 2020 Aug 17:1932296820949930. Epub 2020 Aug 17.

Department of Pediatrics, Diabetology, Endocrinology and Nephrology, Medical University of Lodz, Poland.

Background: Globally millions of people with diabetes still prick their fingers to measure blood glucose. The aim of this study was to comprehensively evaluate and to compare three lancing devices set at the minimum ("1") and at the maximum ("5") lancing depth with respect to blood volume (BV) and pain related to lancing.

Methods: Lancing devices tested were A-, B- (both: HTL-Strefa S.A., Poland), and C- (Ascensia Diabetes Care, Switzerland), all used with personal lancets of three sizes 28G, 30G, and 33G. BVs were measured with calibrated capillaries. Pain related to lancing was expressed as a derivative of pain rating with visual analog scale.

Results: In 90 participants with diabetes, 360 lancing procedures were performed. Overall, BV and pain were higher for "maximum" compared to "minimum" lancing depth (for both < .001). Pain differed between devices ( ≤ .001), overall was higher for device A compared to B or C; in paired comparisons differences were significant for the following settings: A > B for 28G/1 and 33G/1, B > C for 30G/1, and A > C for 28G/1, 30G/1, and 33G/1. In aggregated comparison we did not prove a significant effect of lancet size on either BV nor pain ( = .1109, = .4966, respectively).

Conclusions: BV depended mainly on lancing depth. Pain depended on lancing depth and to some degree on device type. The results may serve as a source of comparative data of lancing devices performance for studies in which other lancing devices and/or lancets would be tested.The study was registered at ClinicalTrials.gov: NCT03479619.
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http://dx.doi.org/10.1177/1932296820949930DOI Listing
August 2020

The Broad Variability in Dental Age Observed among Childhood Survivors Is Cancer Specific.

Cancer Res Treat 2021 Jan 24;53(1):252-260. Epub 2020 Aug 24.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.

Purpose: The study aimed to assess the differences in dental maturation between childhood cancer survivors and healthy children.

Materials And Methods: Fifty-nine cancer patients including 16 (27.1%) girls and 43 (72.8%) boys, aged between 4 and 16 years, underwent dental and radiographic examinations. The mean duration of anticancer therapy was 16.8 months (range, 1 to 47 months), and 4.6 years (range, 8 to 123 months) had passed since the termination of disease. The control group consisted of 177 panoramic radiographs of age- and sex-matched healthy individuals. Dental age (DA) was estimated with Demirjian's scale and delta age, i.e., DA-chronological age (CA), was used to compare groups.

Results: The DA of cancer survivors was accelerated by almost 1 year compared to their CA (9.9±3.1 vs. 8.9±2.8, p=0.040). The greatest difference was observed among patients with brain tumor: delta (DA-CA) was 2.2±1.1 years. Among all cancer patients, only children with familial adenomatous polyposis (FAP)-associated hepatoblastoma (HP) demonstrated delayed DA, with regard to both other cancer survivors (p=0.011) and healthy patients (p=0.037). All four patients with HP suffered from FAP, and three of them had documented adenomatous polyposis coli (APC) genes mutation. The DA of cancer patients having teeth with short roots was significantly greater than that of the cancer survivors without this anomaly (12.8±3.2 vs. 9.0±2.4, p < 0.001).

Conclusion: DA in children may be altered by cancer disease.
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http://dx.doi.org/10.4143/crt.2020.275DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7812002PMC
January 2021

Multiple Retinal Anomalies in Wfs1-Deficient Mice.

Diagnostics (Basel) 2020 Aug 19;10(9). Epub 2020 Aug 19.

Department of Pediatrics, Oncology and Hematology, Medical University of Łódź, Sporna 36/50 Street, 91-738 Łódź, Poland.

Background: Wolfram syndrome (WFS, OMIM: #222300) is an ultrarare autosomal recessive disorder characterized by diabetes insipidus, diabetes mellitus, optic nerve atrophy and deafness. It has been reported that the average retinal thickness in WFS patients decreases with the progression of the disease.

Aim: To investigate retinal thickness and wolframin expression disorders in Wolfram syndrome 1 gene knockout (Wfs1KO) mice compared to their wild-type (WT) littermates.

Materials And Methods: Both bulbs with optic nerves of three mice Wfs1WT and three Wfs1KO were taken for the histopathological examination. A strain of knockout mice with mutation in exon 8 was used.

Results: No expression of wolframin protein in the retina and neurodegeneration of the optic nerve of Wfs1KO mice as compared among Wfs1WT mice was observed. The mean central retinal thickness was thinner and the retinal thickness/longitudinal diameter ratio was significantly lower in hte Wfs1KO as compared to the Wfs1WT mice. In four (67%) eyeballs of Wfs1KO mice, intra-retinal neovessels were observed.

Conclusions: Wfs1KO mice retina with mutation in exon 8 present similar clinical features as patients with WFS in the form of reduced retinal thickness and neurodegeneration of the optic nerve. The presence of proliferative retinopathy observed in Wfs1KO mice requires further investigation.
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http://dx.doi.org/10.3390/diagnostics10090607DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7555979PMC
August 2020

Novel mutation in the first fully-diagnosed patient with Fanconi anemia in Polish population - case report.

Mol Cytogenet 2020 10;13:33. Epub 2020 Aug 10.

Department of Clinical Genetics, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, ul. Sklodowskiej-Curie 9, 85-094 Bydgoszcz, Poland.

Background: Fanconi anemia is a rare genetic disorder caused by mutations in genes which protein products are involved in replication, cell cycle control and DNA repair. It is characterized by congenital malformations, bone marrow failure, and high risk of cancer. The diagnosis is based on morphological and hematological abnormalities such as pancytopenia, macrocytic anaemia and progressive bone marrow failure. Genetic examination, often very complex, includes chromosomal breakage testing and mutational analysis.

Case Presentation: We present a child with clinical diagnosis of Fanconi anemia. Although morphological abnormalities of skin and bones were present from birth, diagnosis was only suspected at the age of 8. Chromosome breakage test in patient's lymphocytes showed increased level of aberrations (gaps, chromatid breaks, chromosome breaks, radial figures and rearrangements) compared to control. Next generation sequencing revealed presence of two pathogenic variants in gene, one of which was not previously reported

Conclusions: The article provides additional supportive evidence that compound biallelic mutations of are associated with Fanconi anemia. It also illustrates the utility of combination of cytogenetic and molecular tests, together with detailed clinical evaluation in providing accurate diagnosis of Fanconi anemia. This report, to the best of our knowledge, describes the first fully diagnosed FA patient in Polish population.
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http://dx.doi.org/10.1186/s13039-020-00503-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7418427PMC
August 2020

Advantages and Limitations of SNP Array in the Molecular Characterization of Pediatric T-Cell Acute Lymphoblastic Leukemia.

Front Oncol 2020 17;10:1184. Epub 2020 Jul 17.

Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Lublin, Poland.

T-cell acute lymphoblastic leukemia (T-ALL) is a highly heterogeneous disease, and numerous genetic aberrations in the leukemic genome are responsible for the biological and clinical differences among particular ALL subtypes. However, there is limited knowledge regarding the association of whole-genome copy number abnormalities (CNAs) in childhood T-ALL with the course of leukemia and its outcome. The aim of this study was to identify the pattern of whole-genome CNAs in 86 newly diagnosed childhood T-ALL cases using a high-density single-nucleotide polymorphism array. We analyzed the presence of whole-genome CNAs with respect to immunophenotype, clinical features, and treatment outcomes. A total of 769 CNAs, including trisomies, duplications, deletions, and segmental loss of heterozygosity, were detected in 86 analyzed samples. Gain or loss of chromosomal regions exceeding 10 Mb occurred in 46 cases (53%), including six cases (7%) with complex chromosomal alterations. We observed that microdeletions in selected genes (e.g., and ) were related to the clinical features. Interestingly, 13% of samples have a duplication of the two loci ( and 6q23.3), which never occurred alone. Single-nucleotide polymorphism array significantly improved the molecular characterization of pediatric T-ALL. Further studies with larger cohorts of patients may contribute to the selection of prognostic CNAs in this group of patients.
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http://dx.doi.org/10.3389/fonc.2020.01184DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7379740PMC
July 2020

High Frequency of Fusion Gene Transcript Resulting From t(10;11)(p12;q23) Translocation in Pediatric Acute Myeloid Leukemia in Poland.

Front Pediatr 2020 10;8:278. Epub 2020 Jul 10.

Department of Pediatrics, Hematology and Oncology, City Hospital, Chorzow, Poland.

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). It is considered as the second most common translocation detected in pediatric 11q23/-rearranged (present ) AML, after t(9;11)(p22;q23). The presence of the above translocation was previously identified as an unfavorable prognostic factor. Since June 2015, the Polish Pediatric Leukemia/Lymphoma Study Group has applied the therapeutic protocol requiring extensive diagnostics of genetic changes in pediatric AML. Until November 2019, molecular genetic studies were performed in 195 children with diagnosed AML to identify carriers of fusion gene transcripts for 28 most common chromosomal translocations in acute leukemia. The fusion gene transcript for translocation t(10;11)(p12;q23) involving gene was detected with unexpectedly high frequency (8.9%) in our research. It was the highest frequency of all detected rearrangements, as well as other detected fusion gene transcripts from chromosomal aberrations characteristic for AML. It seems that chromosomal aberration between chromosomes 10 and 11 can be relatively frequent in some populations. Paying attention to this fact and ensuring proper genetic diagnosis seem to be important for appropriate allocation of patients to risk groups of pediatric AML treatment protocols.
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http://dx.doi.org/10.3389/fped.2020.00278DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7366384PMC
July 2020

Retrospective Analysis of the Treatment Outcome in Myeloid Leukemia of Down Syndrome in Polish Pediatric Leukemia and Lymphoma Study Group From 2005 to 2019.

Front Pediatr 2020 19;8:277. Epub 2020 Jun 19.

Department of Pediatric Oncohematology, Clinical Province Hospital of Rzeszów, Rzeszow, Poland.

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. There were 34 children treated with Acute Myeloid Leukemia-Berlin-Frankfurt-Munster 2004 Interim Protocol (group I) and 20 patients treated with ML-DS 2006 Protocol (group II). In the first protocol, there was reduction of the antracyclines doses and intrathecal treatment for ML-DS compared to non-DS patients. In the second protocol, further reduction of the treatment was introduced (omission of etoposide in the last cycle, no maintenance therapy). Probabilities of 5-year overall survival (OS), event-free survival (EFS), and relapse-free survival in the whole analyzed group were 0.85 ± 0.05, 0.83 ± 0.05, and 0.97 ± 0.03, respectively. No significant differences were found between two protocols in the terms of OS and EFS (0.79 ± 0.07 vs. 0.95 ± 0.05, = 0.14, and 0.76 ± 0.07 vs. 0.95 ± 0.05, = 0.12, respectively). All deaths were caused by the treatment-related toxicities. Reduction of the treatment-related mortality was noticed (20% in group I and 5% in group II). The only one relapse in the whole cohort occurred in the patient from group I, older than 4 years, without gene mutation. He was treated successfully with IdaFLA cycle followed by hematopoietic stem cell transplantation from matched sibling donor. No significant prognostic factor was found in the study group probably due to low number of patients in the subgroups. The study confirms that the reduced intensity protocols are very effective in ML-DS patients. The only cause of deaths was toxicities; however, systematic decrease of the treatment-related mortality was noticed.
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http://dx.doi.org/10.3389/fped.2020.00277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7317010PMC
June 2020

Fetuin-A and Interleukine-8 in Children with the Clinical Remission of Type 1 Diabetes.

Iran J Immunol 2020 Jun;17(2):144-153

Department of Pediatrics, Silesian Medical University in Katowice, Katowice, Poland.

Background: Clinical partial remission (CPR) in most patients with type 1 diabetes (T1D) is observed shortly after clinical diagnosis. Increasing body weight and impaired insulin sensitivity may play a role in the pathogenesis of CPR. Several cytokines can also participate in the development of insulin resistance.

Objective: To evaluate the relationship between birth weight, body mass index, and the concentrations of IL-8 and Fetuin-A, and the presence of clinical partial remission in children at the T1D onset.

Methods: The study group consisted of 134 children with a newly diagnosed T1D in whom the presence of CPR was evaluated in a further 2-year course of diabetes. The control group included 47 children without glucose tolerance disorders. The concentrations of IL-8 and Fetuin-A were determined by the ELISA method.

Results: CPR occurred in 75.34% of T1D patients. At T1D onset, higher values of BMI SDS in the remitters as compared to the patients without remission were observed. At the T1D onset, the concentrations of Fetuin-A (p=0.031) and IL-8 (p=0.042) were significantly higher in patients compared to those without CPR.

Conclusion: Evaluation of Fetuin-A and IL-8 levels in patients with a newly diagnosed T1D can differentiate between patients with or without CPR.
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http://dx.doi.org/10.22034/iji.2020.82797.1595DOI Listing
June 2020

Six molecular patterns leading to hemophilia A phenotype in 18 females from Poland.

Thromb Res 2020 09 28;193:9-14. Epub 2020 May 28.

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Poland.

Introduction: Female hemophilia is an intriguing rare disorder and few larger reports on its genetic etiology are available. While historically the diagnosis was satisfactorily reached by factor VIII activity assays, the clinical and potentially therapeutic heterogeneity of female hemophilia calls for comprehensive molecular diagnosis in each case. Currently, the genetic investigations are not a part of routine, state-funded, diagnostics in Poland, and thus molecular epidemiological data are missing.

Aim: We set out to perform a comprehensive genetic analysis of Polish females with hemophilia A.

Patients/methods: Eighteen females with hemophilia A (including 2 with severe and 5 with moderate hemophilia phenotype) consented for genetic diagnostics. To establish F8 mutations, we used next-generation sequencing of a panel of genes associated with hematological disorders, standard assays for recurrent intragenic F8 inversions and MLPA when deletions were suspected. When appropriate we also used karyotyping, genomic microarrays and X chromosome inactivation assays.

Results: While abnormally skewed X-chromosome inactivation combined with a F8 variant on the active allele was, as expected, the most common genetic etiology, a number of other genetic scenarios were unraveled. This included: misdiagnosis (molecular diagnosis of vWd), Turner syndrome, compound heterozygosity and androgen insensitivity syndrome (a phenotypical 46,XY female with a novel androgen receptor gene mutation). We report 3 novel F8 mutations.

Conclusion: Every case of female hemophilia warrants full genomic diagnostics, as this may change the diagnosis or reveal broader morbidity than a coagulation disorder (Turner syndrome, androgen insensitivity, or cardiovascular morbidity that we described previously in a SHAM syndrome carrier).
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http://dx.doi.org/10.1016/j.thromres.2020.05.041DOI Listing
September 2020

HLA-A gene variation modulates residual function of the pancreatic β-cells in children with type 1 diabetes.

Pediatr Endocrinol Diabetes Metab 2020 ;26(2):73-78

Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Poland.

Aim Of The Study: The study aimed to analyze an association between the HLA-A gene variation and a risk of type 1 diabetes development and to evaluate the association of HLA class I and class II alleles with β-cell destruction.

Material And Methods: A group of 108 children with type 1 diabetes were genotyped in HLA-A, -DRB1, and -DQB1 genes using hybridization with oligonu-cleotides probes. Plasma C-peptide concentration was assessed by radioimmunoassay method.

Results: No differences in allele HLA-A distribution between type 1 diabetes patients and healthy individuals were found. Among "low C-peptide"(< 0.28 pmol/ml) individuals, the frequency of HLA-A*02 allele was 41.3%, whereas only one HLA-A*26 allele was detected in this group (0.7%). Conversely, among "high C-peptide"( 0.28 pmol/ml) probands the prevalence of A*02 allele was 19.7% (Pc = 0.008, OR = 1.4, 95% CI: 1.2-1.7) and A*26 10.5 % (Pc < 0.007, OR = 0.15, 95% CI: 0.02-0.9). Genotype analysis showed that A*02/*02 and A*02/X children were more likely to have "low" C-peptide at the onset compared to those with non-A*02/non-A*02 genotype (p = 0.008, OR = 1.6, 95% CI: 1.3-2.0 and p = 0.015, OR = 1.4, 95% CI: 1.1-1.9, respectively). A02 phenotype individuals had lower median C-peptide (0.17 pmol/ml) than non-A02 patients (0.26 pmol/ml, p = 0.008). Median C-peptide was higher in the A26-positive group comparing to A26-negative (0.40 and 0.20, respectively, p = 0.04). No association between HLA class II and C-peptide levels was observed.

Conclusions: HLA-A alleles are not associated with disease development nevertheless strongly influence a residual pancreatic β-cell function. The results suggest a different role of HLA class I and class II in type 1 diabetes pathogenesis.
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http://dx.doi.org/10.5114/pedm.2020.95617DOI Listing
May 2021

Prevalence, Epidemiology, Etiology, and Sensitivity of Invasive Bacterial Infections in Pediatric Patients Undergoing Oncological Treatment: A Multicenter Nationwide Study.

Microb Drug Resist 2021 Jan 20;27(1):53-63. Epub 2020 May 20.

Department of Pediatric Stem Cell Transplantation, Hematology and Oncology, Medical University, Wroclaw, Poland.

Infectious complications (IC) caused by bacterial strains often impede anticancer therapy. The study aimed to retrospectively analyze bacterial IC that could help predict the risk and optimize the empirical treatment for bacterial infections in pediatric cancer patients. Over a 72-month period, all-in 5,599 children with cancer: 2,441 patients with hematological malignancy (HM including acute leukemias, Hodgkin and non-Hodgkin lymphomas [NHLs], and Langerhans cell histiocytosis) and 3,158 with solid tumors (STs including central nervous system tumors, neuroblastoma, Wilms' tumor, soft tissue sarcoma, germ cell tumors, Ewing sarcoma, osteosarcoma, hepatoblastoma, and others) were enrolled into the study. Episodes of bacterial infectious complications (EBICs) confirmed by microbiological findings were reported by each hospital and analyzed centrally. At least 1 EBIC was diagnosed in 2,155 (36.8%) children (1,281 [59.4%] with HM and 874 [40.6%] with ST;  < 0.001). All-in 4,860 EBICs were diagnosed including 62.2% episodes in children with HM and 37.8% in children with ST ( < 0.001). Having analyzed the source of infections, blood stream infections predominated, apart from NHL patients in whom the most common type was gut infections. The profile of bacteria strains was different in HM and ST groups ( < 0.001). However, in both groups the most common Gram-negative pathogen was Enterobacteriaceae, with the rate being higher in the HM group. Among Gram-negative strains low susceptibility to ceftazidime, whereas among spp. low susceptibility to vancomycin was noticed. The rate of multidrug-resistant (MDR) pathogens was high, especially for Gram negatives (47.7% vs. 23.9%;  < 0.001). The survival after infections was comparable for HM and ST patients ( = 0.215). The risk of bacterial IC in HM patients was higher than in the ST group. The high rate of MDR strains was detected in pediatric cancer patients, especially in those with HM.
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http://dx.doi.org/10.1089/mdr.2019.0393DOI Listing
January 2021