Two histologically different tumours in a neonate born from an assisted reproductive technology pregnancy

The first case of a female neonate born from an in vitro fertilization with embryo transfer (IVF-ET) and intracytoplasmic sperm injection (ICSI) (IVF-ET (ICSI) with two histologically different tumours (craniopharyngioma and hepatoblastoma) is described. Anti-neoplasmatic therapy was abandoned due to the significant extent of the disease (craniopharyngioma, 15×12 cm in diameter with active internal hydrocephalus; and right liver lobe hepatoblastoma, 5 cm in diameter) and the severely impaired general condition of the neonate. The neonate died on the 30th day of life due to cerebellar and brainstem herniation, followed by circulatory and respiratory failure

Safety and feasibility of Lin- cells administration to ALS patients : a novel view on humoral factors and miRNA profiles

Therapeutic options for amyotrophic lateral sclerosis (ALS) are still limited. Great hopes, however, are placed in growth factors that show neuroprotective abilities (e.g., nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF), and vascular endothelial growth factor (VEGF)) and in the immune modulating features, in particular, the anti-inflammatory effects. In our study we aimed to investigate whether a bone marrow-derived lineage-negative (Lin-) cells population, after autologous application into cerebrospinal fluid (CSF), is able to produce noticeable concentrations of trophic factors and inflammatory-related proteins and thus influence the clinical course of ALS. To our knowledge, the evaluation of Lin- cells transplantation for ALS treatment has not been previously reported. Early hematopoietic Lin- cells were isolated from twelve ALS patients’ bone marrow, and later, the suspension of cells was administered into the subarachnoid space by lumbar puncture. Concentrations of selected proteins in the CSF and plasma were quantified by multiplex fluorescent bead-based immunoassays at different timepoints post-transplantation. We also chose microRNAs (miRNAs) related to muscle biology (miRNA-1, miRNA-133a, and miRNA-206) and angiogenesis and inflammation (miRNA-155 and miRNA-378) and tested, for the first time, their expression profiles in the CSF and plasma of ALS patients after Lin- cells transplantation. The injection of bone marrow cells resulted in decreased concentration of selected inflammatory proteins (C3) after Lin- cells injection, particularly in patients who had a better clinical outcome. Moreover, several analyzed miRNAs have changed expression levels in the CSF and plasma of ALS patients subsequent to Lin- cells administration. Interestingly, the expression of miR-206 increased in ALS patients, while miR-378 decreased both in the CSF and plasma one month after the cells’ injection. We propose that autologous lineage-negative early hematopoietic cells injected intrathecally may be a safe and feasible source of material for transplantations to the central nervous system (CNS) environment aimed at anti-inflammatory support provision for ALS adjuvant treatment strategies. Further research is needed to evaluate whether the observed effects could significantly influence the ALS progression

Część I: Wytyczne postępowania w hemofilii A i B niepowikłanej inhibitorem czynnika VIII i IX (wydanie zaktualizowane)

In this document, Working Group for Haemostasis of the Polish Society of Haematology and Blood Transfusion updates the principles of diagnosis and treatment of haemophilia A and B, published for the first time in 2008. In a companion paper (part II), the management of haemophilia A and B complicated by factor VIII and IX inhibitors will be covered

Część II: Wytyczne postępowania w hemofilii A i B powikłanej inhibitorem czynnika VIII i IX (2 wydanie)

In this article, the Working Group for Haemostasis of the Polish Society of Haematology and Blood Transfusion updates the principles of diagnosis and treatment of haemophilia A and B complicated by factor VIII or IX inhibitors, which were published for the first time in 2008. In the first part of the Guidelines published in 2016, the management of haemophilia A and B without factor VIII and IX inhibitors was dealt with

Epidemiology and prevention strategies of SARS-CoV-2 infection in pediatric hematology and oncology centers in Poland

IntroductionEpidemiological analysis of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infections in pediatric hematology and oncology (PHO) and hematopoietic cell transplant (HCT) centers in a Polish nationwide study, as well as analysis of the preventive strategies in these centers. MethodsAll of the 18 PHO/HCT centers participated in eight surveys and questionnaires conducted over the first 5 months of the SARS-CoV-2/coronavirus disease 2019 (COVID-19) pandemic in Poland. Epidemiological data were collected at eight regular time points, and the strategy of preventive management was done once after 4 months of the pandemic. ResultsDuring this analyzed period, eight patients were positive for SARS-CoV-2. The estimated incidence of SARS-CoV-2 positivity in Polish PHO/HCT centers was 0.5%. After exclusion of HCT patients (with one patient being infected), the estimated incidence of SARSCoV-2 positivity was between 0.5 and 0.6%. In all but one case, the course of COVID-19 was asymptomatic or mild, and it was moderate in one case. None of them developed SARS or respiratory insufficiency, none of them required treatment in the intensive care unit (ICU), and no patient died due to SARS-CoV-2 infection. As of July 1, parents staying in the hospital together with their children were regularly tested for the virus in 13 centers. Asymptomatic healthcare personnel were regularly tested for the virus in seven centers. ConclusionsThe estimated incidence of SARS-CoV-2 infection among PHO/HCT patients is lower than in Western Europe; however, these patients cannot be regarded as a low-risk group. The low COVID-19 incidence should be interpreted as a result of strictly and continuously implemented detailed preventive measures in the PHO/HCT wards and in hospitals

Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL

Wstęp: Zróżnicowane raki tarczycy (DTC, differentiated thyroid carcinoma) występują u dzieci rzadko. Większość przypadków wykrywanych jest w wieku 11–17 lat. W odróżnieniu od dorosłych DTC u dzieci prezentują odmienne zachowanie biologiczne. Mała liczba przypadków DTC w poszczególnych ośrodkach oraz względnie łagodny ich przebieg utrudniają ocenę występowania i leczenia DTC u dzieci w Polsce, uzależniając ją od wysiłków włożonych w uzyskanie rzetelnych danych. Autorzy przedstawiają wstępne wyniki analizy wieloośrodkowej dotyczące występowania, diagnostyki i leczenia DTC u dzieci. Materiał i metody: Podjęte badania są retrospektywną analizą obejmującą lata 2000–2005, opartą na danych z historii chorób uzyskanych z ankiet rozesłanych do ośrodków dla dzieci i dorosłych podejmujących leczenie DTC. Do analizy zgłoszono 107 pacjentów z 14 ośrodków akademickich w Polsce. Analizie poddano wiek i płeć dzieci z DTC, wielkość i lokalizację zmian w tarczycy, sposoby rozpoznawania DTC, rodzaje i zakres wykonywanych zabiegów operacyjnych oraz leczenie uzupełniające izotopem J131. Wyniki: Raka brodawkowatego stwierdzono u 83 dzieci, pęcherzykowego u 10 dzieci, a rdzeniastego u 14 dzieci. Częstość występowania DTC u dzieci w Polsce wahała się między 18 a 23 przypadkami rocznie. W województwach: mazowieckim i połączonych wielkopolskim i lubuskim wykazano w okresie 2000–2005 wyższą (24 i 25) częstość występowania DTC, w pozostałych województwach wykazywano od 2 do 10 przypadków DTC. Największą grupę pacjentów stanowiły dzieci w wieku 11–15 lat, a stosunek dziewcząt do chłopców wynosił 3,3 : 1. Klinicznie DTC prezentowały się najczęściej jako pojedyncze guzki tarczycy. Limfadenopatię szyjną w badaniu klinicznym stwierdzono u 42% pacjentów, a śródoperacyjnie u 50% dzieci. U większości pacjentów dominowały niższe stopnie zaawansowania DTC (T1 u 36% i T2 u 26% dzieci). Operacje jednoetapowe wykonano u 65% dzieci, operacje dwuetapowe u 25% dzieci, a profilaktyczne tyreoidektomie u 79% dzieci z grupy pacjentów z rakiem rdzeniastym tarczycy (MTC, medullary thyroid cancinoma) i mutacją genu Ret. Leczenie izotopowe J131 podjęto u 80% dzieci. Przerzuty do płuc w scyntygrafii poterapeutycznej wykazano u 14% dzieci z DTC. Wnioski: We wnioskach podkreśla się konieczność wdrożenia na terenie całego kraju ujednoliconego i ocenianego na podstawie obiektywnych przesłanek sposobu postępowania z dziećmi z DTC.Introduction: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children. Material and methods: The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children. Results: Papillary thyroid cancer was diagnosed in 83 children, follicular thyroid cancer in 10 children and medullary thyroid cancer in 14 children. Incidence of DTC in children was estimated between 18 and 23 cases per year. The biggest group of patients consisted of children between 11 and 15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC in children presented most often as solitary thyroid nodule. Cervical lymphadenopathy was observed in 42% of patients. Intraoperative verification indicated metastatic nodes in 50% of children. Low stage DTC predominated (T1 in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery in 25% of patients. I131 therapy was undertaken in 80% of children. Lung metastases were indicated in post therapeutic studies in 14% of children with DTC. Prophylactic thyroidectomies were performed in 79% of children in the group of patients with MTC and RET gene mutations. Conclusions: The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined

Badania obrazowe u 17-letniego chłopca z chorobą Bourneville'a-Pringle'a : opis przypadku

Background: Tuberous sclerosis complex is e genetic disorder characterized by lesions affecting the brain, skin, eyes and internal organs - kidneys, heart, liver and lungs. Case Report: The case of a 17-year-old boy with delayed proper diagnosis of tuberous sclerosis complex and multiple lesions in brain, lungs, heart and kidneys has been described. Conclusions: The authors would like to underline the very rare incidence of sclerosis tuberous complex and the need for periodic follow-up imaging of central nervous system, chest (with thorough evaluation of heart and lungs), and abdominal cavity. Moreover, the authors would like to underline a significantl delay in a process of reaching a final diagnosis of the disease

Novel Evidence of the Increase in Angiogenic Factor Plasma Levels after Lineage-Negative Stem/Progenitor Cell Intracoronary Infusion in Patients with Acute Myocardial Infarction

Cell therapy raises hope to reduce the harmful effects of acute myocardial ischemia. Stem and progenitor cells (SPCs) may be a valuable source of trophic factors. In this study, we assessed the plasma levels of selected trophic factors in patients undergoing application of autologous bone marrow (BM)-derived, lineage-negative (Lin−) stem/progenitor cells into the coronary artery in the acute phase of myocardial infarction. The study group consisted of 15 patients with acute myocardial infarction (AMI) who underwent percutaneous revascularization and, afterwards, Lin− stem/progenitor cell administration into the infarct-related artery. The control group consisted of 19 patients. BM Lin− cells were isolated using immunomagnetic methods. Peripheral blood was collected on day 0, 2, 4, and 7 and after the first and third month to assess the concentration of selected trophic factors using multiplex fluorescent bead-based immunoassays. We found in the Lin− group that several angiogenic trophic factors (vascular endothelial growth factor, Angiopoietin-1, basic fibroblast growth factor, platelet-derived growth factor-aa) plasma level significantly increased to the 4th day after myocardial infarction. In parallel, we noticed a tendency where the plasma levels of the brain-derived neurotrophic factor were increased in the Lin– group. The obtained results suggest that the administered SPCs may be a valuable source of angiogenic trophic factors for damaged myocardium, although this observation requires further in-depth studies