Correlations between selected parameters of nasal cavity in neonates and young infants — computed tomography study

  Background: Correlations between selected metric parameters of the nasal cavity and nasopharynx in children without atresia may be useful for anticipating probable dimensions of this region in living subjects, in terms of changes with age. Materials and methods: One hundred and eighty children, age range 0–3 years, were divided into five age groups, and measurements of 18 distances between structures of nasal cavity and nosopharynx were performed on computed tomography scans. Correlation coefficients and relations between parameters have been determined. Results: Our study confirmed the existence of statistically significant correlations between linear dimensions within nasal cavity in children. The analysis demon­strated that for the values of following indexes: nasal septum length/piriform aperture width, and maximum length of the nasal septum/posterior nares width no statistically significant differences have been noted between age groups of children. All correlations have been positive. No statistically significant differences have been noted between the maximum width of the vomer and osseous parameters measured, both in the anterior and posterior part of the nasal cavity, and nasal septum length. Conclusions: The size of posterior nares changed with age in children by a constant value. So far, no such an analysis has been carried out assessing potential correlations between linear dimensions for the entire nasal cavity, nasopharynx, length of the nasal septum in children, as well as proportions of individual linear dimensions of the anatomical structures analysed, in various age groups

The treatment of primary cutaneous lymphoma. Recommendations of the Polish Lymphoma Research Group (PLRG)

Primary cutaneous lymphoma is a heterogenous group of lymphoid neoplasms, mostly of T-cell origin, defined by the initial and clinically dominant involvement of skin. Clinical course and prognosis of these diseases is distinct from other nodal or extranodal lymphomas. Cutaneous lymphomas have usually very prolonged course and are incurable in most of the cases. The diagnosis and treatment planning is best applied if multidisciplinary approach involving dermatologists, oncologists, hematologists and pathologists is adopted. Cutaneous Lymphoma Section, created within the Polish Lymphoma Research Group in 2009 set out to reach a consensus on treatment guidelines that would be acknowledged by major specialties involved in the management of these patients. The guidelines are consistent with published international recommendations and supported by current practice of the Polish dermatology and hemato-oncology centers.Pierwotne chłoniaki skóry stanowią heterogenną grupę nowotworów układu limfoidalnego, wśród których około 75% wywodzi się z limfocytów T i których pierwotnym i dominującym klinicznie umiejscowieniem jest skóra. Pierwotne chłoniaki skóry cechuje odmienny przebieg kliniczny i rokowanie w porównaniu z chłoniakami układowymi. Chłoniaki skóry mają najczęściej przebieg przewlekły, ale całkowite wyleczenie chorego rzadko jest możliwe. Diagnostyka i leczenie pierwotnych chłoniaków skóry jest zagadnieniem interdyscyplinarnym, w które powinni być zaangażowani - oprócz dermatologów - również onkolodzy, hematolodzy i patomorfolodzy. Powstała w 2009 roku Sekcja Chłoniaków Skóry Polskiej Grupy Badawczej Chłoniaków podjęła próbę opracowania jednolitych zaleceń terapeutycznych, które byłyby uznane i stosowane w środowiskach wszystkich zaangażowanych specjalności. Są one oparte na dostępnych europejskich i światowych rekomendacjach oraz na doświadczeniu polskich ośrodków dermatologicznych i hematoonkologicznych w zakresie leczenia chłoniaków skóry

Stanowisko polskich ekspertów dotyczące zastosowania leku brentuksymab vedotin w leczeniu chorych na pierwotne chłoniaki skóry CD30+

The group of primary cutaneous T-cell lymphomas (CTCLs) expressing CD30 + is consisted of primary cutaneous anaplastic T-cell lymphoma (pcACTL), lymphomatoid papulosis (LyP), some cases of mycosis fungoides (MF) and Sezary syndrome (SS). It is well known that patient cannot be cured completely by available therapeutic methods. In addition, the effectiveness of available therapies is especially limited in advanced stages of the disease. Based on the results of the most recent trials, the experts recommend that brentuximab vedotin (BV) should be reimbursed in Poland for the treatment of adult patients with CTCL expressing CD30 who have had at least one prior systemic treatment. In case of MF BV should be preferred to bexarotene or MTX therapy due to the higher efficacy in stage IIB or higher. BV treatment should be also considered as an alternative to bexarotene (after ineffectiveness of local treatment, phototherapy and IFN/MTX therapy) in early stages of MF (IB-IIA).Do pierwotnych chłoniaków skóry T-komórkowych (CTCL) z ekspresją CD30+ należą pierwotny skórny chłoniak anaplastyczny z dużych komórek (pcALCL), lymphomatoid papulosis (LyP), a także ziarniniak grzybiasty (MF) i zespół Sézary’ego (SS), spośród których u części można stwierdzić ekspresję CD30+. Istniejące metody terapii nie pozwalają na wyleczenie pacjenta cho­rującego na wymienione wyżej odmiany chłoniaków. Ponadto skuteczność dostępnych metod jest szczególnie ograniczona w zaawansowanych stadiach choroby. Na podstawie wyników najnowszych badań eksperci rekomendują, aby brentuksymab vedotin (BV) był dostępny w Polsce do leczenia do­rosłych pacjentów z CTCL z ekspresją CD30, u których uprzednio stosowano co najmniej jedno le­czenie systemowe. W stadium MF IIB i wyższym leczenie BV powinno być preferowane w stosunku do terapii beksarotenem lub metotreksatem (MTX) ze względu na wyższą skuteczność nowego leku. We wczesnych stadiach MF (IB–IIA) należy rozważyć leczenie BV alternatywnie do stosowania bek­sarotenu (po nieskuteczności leczenia miejscowego, fototerapii i terapii interferonem i/lub MTX). Skuteczność leczenia BV wykazano u chorych z pcALCL zarówno we wczesnej fazie z obecnością zmian ograniczonych do skóry, jak i w postaci zaawansowanej z pozaskórną lokalizacją zmian

Age-dependent determinants of infectious complications profile in children and adults after hematopoietic cell transplantation : lesson from the nationwide study

Incidence and outcome of microbiologically documented bacterial/viral infections and invasive fungal disease (IFD) in children and adults after hematopoietic cell transplantation (HCT) were compared in 650 children and 3200 adults in multicenter cross-sectional nationwide study. Infections were diagnosed in 60.8% children and 35.0% adults, including respectively 69.1% and 63.5% allo-HCT, and 33.1% and 20.8% auto-HCT patients. The incidence of bacterial infections was higher in children (36.0% vs 27.6%; p  21 days were risk factors for death from infection. In conclusion, pediatric patients have 2.9-fold higher incidence and 2.5-fold better outcome of infections than adults after HCT

Current status and achievements of Polish haemato-oncology

The number of newly diagnosed haematological malignancies in Polish adults and children is about 9,000 a year, which constitutes about 5.5% of all malignancies in the country. Adult patients with haematological malignancies are diagnosed and treated in 42 institutions in Poland. The scientific and educational support for this activity is provided under the umbrella of the Polish Society of Haematologists and Transfusiologists (PTHiT, Polskie Towarzystwo Hematologów i Transfuzjologów), the Polish Adult Leukemia Group (PALG), the Polish Lymphoma Research Group (PLRG), the Polish Myeloma Study Group (PMSG), the Polish Myeloma Consortium (PMC), and consultants in haematology. The aim of this position paper is to present the current status and progress in therapy of haematological malignancies in Polish haematology adult centres, focusing on the activity of PALG, PLRG, and PMSG. The achievements of Polish haemato-oncology at the beginning of the third decade of the 21st century are set out in this paper. Polish haemato-oncology today has an important international position based on contributions to the development of knowledge, international cooperation, and a high quality of patient care. In many instances, clinical trials run by Polish collaborative groups have influenced international standards. Polish haematologists have been the authors of treatment recommendations, and their research has indicated areas for further research

Clinical features, etiology, and survival in patients with restrictive cardiomyopathy: A single-center experience

Background: Numerous prognostic factors have been proposed for cardiac amyloidosis (CA). The knowledge about other subtypes of restrictive cardiomyopathy (RCM) is scant.Aims: This study aimed to elucidate the etiology and prognostic factors of RCM as well as assess cardiac biomarkers: high-sensitive troponin T (hs-TnT), growth differentiation factor-15 (GDF-15), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and soluble suppression of tumorigenicity 2, as mortality predictors in RCM.Methods: We enrolled 36 RCM patients in our tertiary cardiac department. All patients were screened for CA. Genetic testing was performed in 17 patients without CA.Results: Pathogenic or likely pathogenic gene variants were found in 86% of patients, including 5 novel variants. Twenty patients died, and 4 had a heart transplantation during the study. Median overall survival was 29 months (8–55). The univariate Cox models analysis indicated that systolic and diastolic blood pressure, GDF-15, hs-TnT, NT-proBNP, left ventricular stroke volume, the ratio of the transmitral early peak velocity (E) estimated by pulsed wave Doppler over the early mitral annulus velocity (e’), tricuspid annulus plane systolic excursion, early tricuspid valve annular systolic velocity, the presence of pulmonary hypertension, and pericardial effusion influenced survival (P <0.05). A worse prognosis was observed in patients with GDF-15 >1316 pg/ml, hs-TnT >42 ng/l, NT-proBNP >3383 pg/ml, and pericardial effusion >3.5 mm (Kaplan-Meier analysis, log-rank test, P <0.001).Conclusions: Genetic testing should be considered in every RCM patient where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF-15, hs-TNT, NT-proBNP, and pericardial effusion are associated with worse prognosis. Further studies are warranted

The CLIC Potential for New Physics

The Compact Linear Collider (CLIC) is a mature option for the future of high energy physics. It combines the benefits of the clean environment of $e^+e^-$ colliders with operation at high centre-of-mass energies, allowing to probe scales beyond the reach of the Large Hadron Collider (LHC) for many scenarios of new physics. This places the CLIC project at a privileged spot in between the precision and energy frontiers, with capabilities that will significantly extend knowledge on both fronts at the end of the LHC era. In this report we review and revisit the potential of CLIC to search, directly and indirectly, for physics beyond the Standard Model

Angular Momentum of Early- and Late-type Galaxies: Nature or Nurture?

We investigate the origin, the shape, the scatter, and the cosmic evolution in the observed relationship between specific angular momentum $j_\star$ and the stellar mass $M_\star$ in early-type (ETGs) and late-type galaxies (LTGs). Specifically, we exploit the observed star-formation efficiency and chemical abundance to infer the fraction f_\rm inf of baryons that infall toward the central regions of galaxies where star formation can occur. We find f_\rm inf\approx 1 for LTGs and $\approx 0.4$ for ETGs with an uncertainty of about $0.25$ dex, consistent with a biased collapse. By comparing with the locally observed $j_\star$ vs. $M_\star$ relations for LTGs and ETGs we estimate the fraction $f_j$ of the initial specific angular momentum associated to the infalling gas that is retained in the stellar component: for LTGs we find $f_j\approx 1.11^+0.75_-0.44$, in line with the classic disc formation picture; for ETGs we infer $f_j\approx 0.64^+0.20_-0.16$, that can be traced back to a $z<1$ evolution via dry mergers. We also show that the observed scatter in the $j_\star$ vs. $M_\star$ relation for both galaxy types is mainly contributed by the intrinsic dispersion in the spin parameters of the host dark matter halo. The biased collapse plus mergers scenario implies that the specific angular momentum in the stellar components of ETG progenitors at $z\sim 2$ is already close to the local values, in pleasing agreement with observations. All in all, we argue such a behavior to be imprinted by nature and not nurtured substantially by the environment

Diagnosis of myelodysplastic syndromes in Poland: Polish Adult Leukemia Group (PALG) 2021 recommendations

Myelodysplastic syndromes (MDS) are a heterogeneous group of neoplastic diseases of the hematopoietic cells manifested by ineffective hematopoiesis and a tendency to transform into acute myeloid leukemia. MDS should be considered in the differential diagnosis of cytopenia, especially in the elderly. This article presents the recommendations of MDS experts of the Polish Adult Leukemia Group (PALG) for the diagnosis of myelodysplastic syndromes. We present current classifications and prognostic indices, as well as diagnostic examinations recommended for MDS: cytological, histopathological, immunophenotypic, cytogenetic and molecular tests. The aim of the study is to implement up-to-date knowledge about myelodysplastic syndromes into routine clinical practice, from the diagnosis of cytopenia to the specific diagnosis and prognosis in MDS patients.  Myelodysplastic syndromes (MDS) are a heterogeneous group of neoplastic diseases of the hematopoietic cells manifested by ineffective hematopoiesis and a tendency to transform into acute myeloid leukemia. MDS should be considered in the differential diagnosis of cytopenia, especially in the elderly. This article presents the recommendations of MDS experts of the Polish Adult Leukemia Group (PALG) for the diagnosis of myelodysplastic syndromes. We present current classifications and prognostic indices, as well as diagnostic examinations recommended for MDS: cytological, histopathological, immunophenotypic, cytogenetic and molecular tests. The aim of the study is to implement up-to-date knowledge about myelodysplastic syndromes into routine clinical practice, from the diagnosis of cytopenia to the specific diagnosis and prognosis in MDS patients.

Autologous hematopoietic cell transplantation for relapsed multiple myeloma performed with cells procured after previous transplantation–study on behalf of CMWP of the EBMT

Autologous hematopoietic cell transplantation (auto-HCT) may be performed in multiple myeloma (MM) patients relapsing after a previous auto-HCT. For those without an adequate dose of stored stem cells, remobilization is necessary. This retrospective study included patients who, following disease relapse after the first auto-HCT(s), underwent stem cell remobilization and auto-HCT performed using these cells. There were 305 patients, 68% male, median age at salvage auto-HCT was 59 years. The median time to relapse after the first-line penultimate auto-HCT(s) was 30.6 months, the median follow-up after salvage auto-HCT 31 months. The 2- and 4-year non-relapse mortality (NRM) after the salvage auto-HCT was 5 and 9%, the relapse incidence 56 and 76%, respectively. Overall survival (OS) after 2 and 4 years was 76 and 52%, progression-free survival (PFS) 39 and 15%. In multivariable analysis an increasing interval between the penultimate auto-HCT and relapse was associated with better OS and PFS, later calendar year of salvage auto-HCT with better OS. In conclusion, salvage auto-HCT performed with cells remobilized after a previous auto-HCT was associated with acceptable NRM. The leading cause of failure was disease progression of MM, which correlated with a shorter interval from the penultimate auto-HCT to the first relapse