84. Objawy uszkodzenia centralnego układu nerwowego, będące maską rozpoznania sarcoma granulocytium

Przedstawiono przypadek 26 letniego chorego z rozpoznaniem Sarcoma granulocyticum (SG), u którego pierwsze objawy pod postacią dolegliwości bólowych, okolicy krzyżowobiodrowej, wystąpiły w przebiegu urazu, powtarzając się regularnie co ok. 3 tygodnie. Badanie rezonansu magnetycznego (NMR) w 7 miesięcy później wykazało guzowatą, masę na poziomie S1-2 o wymiarach 7×1,5 cm wrastającą do kanału kręgowego, wypełniającą jego światło i uciskającą korzenie nerwowe. Po wykonaniu laminectomi L5 -S2 z podejrzeniem rozrostu (na podstawie wyniku badania histopatologicznego) z komórek układu chłonnego pacjenta przeniesiono do dalszego leczenia Wdrożono 2-etapową radioterapię na okolicę krzyżową w łącznej dawce 72 Gy. Dwa miesiące później stwierdzono bolesność uciskową skóry głowy, a w 6 tyg. później doszło do nagłej utraty przytomności. Badanie CT mózgu uwidoczniło patologiczną przymózgową masę o wymiarach 3,1×2,5 cm przylegającą do kości pokrywy czaszki w okolicy ciemieniowo-skroniowej. Przeprowadzono kraniotomię skroniowo-ciemieniową lewostronnąW wyniku konsultacji wszystkich preparatów obraz histologiczny i fenotyp odpowiadały rozpoznaniu SG. Po miesiącu, na podstawie przeprowadzonych badań nie stawierdzono zmian dla ostrej białaczki, a jedynie wykryto duży guz części przymostkowej obojczyka lewego. Materiał z pobranej biopsji potwierdził rozpoznanie SG. Pacjent otrzymał 3 cykle chemioterapii: Daunorucicyna – 120 mg / 24 h + Arabinozyd Cytarabiny – 400 mg – 3 g / 24 h, po których uzyskano całkowitą remisję. Z uwagi na przynależność do, grupy wysokiego ryzyka” (kariotyp: 46, XY, del(7q), del(20q)/ 47, idem,+mar/46, XY [21]) chory został zakwalifikowany do zabiegu transplantacji szpiku kostnego od dawcy niespokrewnionego. Sarcoma granulocyticum to typ nowotworu, w którym allogeniczny przeszczep szpiku może dać pacjentowi szansę na wyleczenie

83. Astrocytoma u chorego z wieloletnim przebiegiem białaczki włochatokomórkowej

WstępNowotwory wtórne centralnego układu nerwowego u chorych z chorobami rozrostowymi układu krwiotwórczego należą do rzadkości.Celem pracyBył opis przypadku chorego E.R. 1.51 z przewlekłą białaczką. włochatokomórkową, u którego po 58 miesiącach od rozpoznania choroby stwierdzono astrocytoma mózgu.Opis przypadkuRozpoznanie przewlekłej białaczki włochatokomórkowej postawiono na podstawie badań trepanobiopsji szpiku (obecność rozległych nacieków “hairy cells”: L26 i DBA44-dodatnie), dodatniego wyniku fosfatazy kwaśnej opornej na winian (w 60% komórek), a w badaniu klinicznym – splenomegalii (163 mm). U pacjenta wdrożono leczenie 2-Chlorodeoksyadenozyną (2-CdA) w dawce 0,1 mg/kg/24 h przez 5 dni uzyskując częściową remisję (PR) po 2 cyklach leczenia trwającą 30 m-cy, a następnie po stwierdzeniu wznowy choroby stosowano Roferon w dawce 3–6 mln j. × 3 w tygodniu przez 6 m-cy, wykonano splenektomię i ponownie leczono za pomocą. 2-CdA w dawce 0,1 mg/kg podawanej raz w tygodniu (6 dawek w 1 cyklu) – łącznie 4 cykle. Ponownie uzyskano PR, która trwała 2 m-ce. W okresie trwania II PR tj. Po 58 m-cach od rozpoznania u chorego pojawiły się. zaburzenia orientacji co do czasu i przestrzeni, bóle głowy, omamy słuchowe oraz niedowład prawostronny. W badaniu MR mózgu stwierdzono w obu półkulach mózgu w istocie białej w płatach czołowych ogniska o podwyższonej intensywności sygnałów w czasie T2 i obniżonej w czasie T1 oraz nacieczenie ciała modzelowatego. Nie było widocznego efektu masy- obraz MR odpowiadał naciekom białaczkowym. W badaniu CT mózgu w płatach czołowych obu półkul mózgu wykryto 2 ogniska hypodensyjne w istocie białej płatów czołowych: w prawej ognisko o średnicy 15–20 mm, a w lewej 50 mm. U chorego wykonano biopsję, sterotaktyczną, mózgu, a po ocenie histopatologicznej pobranych wycinków postawiono rozpoznanie Astrocytoma. U pacjenta wykonano radykalną operację guzów. Chory zmarł w drugiej dobie po zabiegu operacyjnym.WniosekU pacjentów z chorobami rozrostowymi układu krwiotwórczego po wieloletnim leczeniu immunosupresyjnym mogą wystąpić wtórne nowotwory centralnego układu nerwowego

Terbium luminescence in synthetic peptide loops from calcium-binding proteins with different energy donors.

Fourteen 14-mer peptides corresponding to a consensus sequence of metal-binding loops from proteins of the calmodulin family were synthesized. The effect of varying both the position in the binding loop, and the type of aromatic side chains as energy donors for enhancement of terbium luminescence, was studied. It was concluded that tryptophan in loop position 7 gave optimal luminescence enhancement, and that the additional inclusion of a tyrosine in the loop at positions 2 or 4 could further boost emission from the bound terbium. In all further cases energy transfer from aromatic residues at positions other than 7 was markedly less efficient. These results suggest that the peptides assume a configuration which allows a hexadentate ligand structure around the bound terbium ion. This is consistent with a Dexter-type electron exchange model of energy transfer

Pain as a mediator in the temperament-alexithymia relationship in individuals suffering from rheumatoid arthritis

[Abstract] OBJECTIVE: The study aims to establish a relationship between temperament traits, symptoms of alexithymia, and pain intensity in rheumatoid arthritis. Despite the significant progress seen in the area of RA treatment, pain, often life-long, remains the predominant symptom. This constant pain and progressing disability, as well as dependence upon other people cause RA patients to experience psychological stress that can be modified by individual patient traits. Recently, several authors have underlined the need to relate personality and temperament constructs to neurobiological processes that may underlie individual differences. It seems then that patient characteristics may play a significant role in the course of the disease. PATIENTS AND METHODS: The study was performed on a group of patients (N=317) with rheumatoid arthritis diagnosed according to the current criteria of the American-European Consensus of 2010. All patients expressed voluntary consent to participate, and the study protocol was approved by the Local Ethics Committee. This was a survey-based study. It involved the application of the adult version of the Buss and Plomin EAS Temperament Questionnaire (EAS-D), which tests 3 main temperament domains: sociability, activity, and emotionality. The pain was measured on the Visual Analogue Scale (VAS). VAS is used to measure pain intensity. The level of alexithymia was tested using the Toronto Alexithymia Scale-20. The scale consists of 20 statements and includes 3 subscales that measure difficulty in describing feelings/emotions, difficulty in identifying feelings/emotions, and operational externally oriented thinking. RESULTS: The analysis revealed that alexithymia is positively correlated only with one dimension of temperament, i.e., emotionality, and with pain intensity. Moreover, high emotionality was positively correlated with pain. A simple mediation analysis revealed that pain intensity functioned as a mediator in the emotionality-alexithymia relationship. CONCLUSIONS: The observed correlations indicate that RA patients with a high level of emotionality exhibit high alexithymia as they perceive pain related to the disease symptoms more intensely. The observed mediation is partial, meaning that there are also other mediating factors in this relationship

Tuberculosis course in a patient with Fahr’s disease (a clinical case report)

Abstract In the present context, pulmonary tuberculosis, especially its resistant forms, is an urgent problem of society, not only inUkraine, but also throughout the world. As is known, comorbidities make difficult not only tuberculosis diagnostics, but also its treatment. Tuberculosis can either develop on the background of any diseases or cause their development and exacerbations. On account of this there are rare diseases of various organs and systems which are characterized by a long absence of clinical symptoms. Work objective is to draw the attention of doctors to the combined course of tuberculosis and rare neurological diseases such as Fahr’s disease. Materials and methods. The article deals with the clinical case based on own observations of the pulmonary multidrug-resistant tuberculosis (MRTB) development in a patient with Fahr’s disease diagnosed in the process of tuberculosis treatment. The patient received inpatient treatment in the Department of Pulmonary Tuberculosis No. 3 of the clinical base of the Phthisiology and Pulmonology Department of the ZSMU in the Communal Institution “Zaporizhzhia Regional Tuberculosis Clinical Dispensary”. Results of own observations. It has been defined that the patient had experienced slow asymptomatic course of Fahr’s disease prior to the onset of pulmonary MRTB. Probably, the toxic effect of antimycobacterial drugs provoked clinical manifestations of Fahr’s disease in the form of neurological symptoms. If Fahr’s disease had been timely diagnosed, antimicrobial therapy would have been prescribed with appropriate initial treatment correction through the exclusion of antimycobacterial drugs with neurotoxic effects from the chemotherapy regimen. Such an approach might not have exacerbated the underlying disease. Conclusions. In a patient with Fahr’s disease the pulmonary MRTB can be treated provided constant monitoring by a neuropathologist with the prescription of adequate symptomatic and pathogenetic therapy that could prevent severe neurological disorders development

A five-year hedonic price breakdown for desktop personal computer attributes in Brazil

The purpose of this article is to identify the attributes that discriminate the prices of personal desktop computers. We employ the hedonic price method in evaluating such characteristics. This approach allows market prices to be expressed as a function, a set of attributes present in the products and services offered. Prices and characteristics of up to 3,779 desktop personal computers offered in the IT pages of one of the main Brazilian newspapers were collected from January 2003 to December 2007. Several specifications for the hedonic (multivariate) linear regression were tested. In this particular study, the main attributes were found to be hard drive capacity, screen technology, main board brand, random memory size, microprocessor brand, video board memory, digital video and compact disk recording devices, screen size and microprocessor speed. These results highlight the novel contribution of this study: the manner and means in which hedonic price indexes may be estimated in Brazil

Uniparental Genetic Heritage of Belarusians: Encounter of Rare Middle Eastern Matrilineages with a Central European Mitochondrial DNA Pool

Ethnic Belarusians make up more than 80% of the nine and half million people inhabiting the Republic of Belarus. Belarusians together with Ukrainians and Russians represent the East Slavic linguistic group, largest both in numbers and territory, inhabiting East Europe alongside Baltic-, Finno-Permic- and Turkic-speaking people. Till date, only a limited number of low resolution genetic studies have been performed on this population. Therefore, with the phylogeographic analysis of 565 Y-chromosomes and 267 mitochondrial DNAs from six well covered geographic sub-regions of Belarus we strove to complement the existing genetic profile of eastern Europeans. Our results reveal that around 80% of the paternal Belarusian gene pool is composed of R1a, I2a and N1c Y-chromosome haplogroups – a profile which is very similar to the two other eastern European populations – Ukrainians and Russians. The maternal Belarusian gene pool encompasses a full range of West Eurasian haplogroups and agrees well with the genetic structure of central-east European populations. Our data attest that latitudinal gradients characterize the variation of the uniparentally transmitted gene pools of modern Belarusians. In particular, the Y-chromosome reflects movements of people in central-east Europe, starting probably as early as the beginning of the Holocene. Furthermore, the matrilineal legacy of Belarusians retains two rare mitochondrial DNA haplogroups, N1a3 and N3, whose phylogeographies were explored in detail after de novo sequencing of 20 and 13 complete mitogenomes, respectively, from all over Eurasia. Our phylogeographic analyses reveal that two mitochondrial DNA lineages, N3 and N1a3, both of Middle Eastern origin, might mark distinct events of matrilineal gene flow to Europe: during the mid-Holocene period and around the Pleistocene-Holocene transition, respectively

Аксіологічні виміри душпастирства у творах Іоана Золотоустого

Стаття Світлани Білоус "Аксіологічні виміри душпастирства у творах Іоана Золотоустого" присвячена пошуку джерел духовної опіки над людиною, витоки яких автор бачить у християнському середньовіччі. Життя і творча спадщина Іоана Золотоустого – яскравий приклад пояснення сутності й визначення ціннісної природи душпастирювання крізь призму поняття священства.Статья Светланы Билоус "Аксиологические измерения душпастирства в произведениях Іоана Золотоустого" посвящена поиску источников духовной опеки над человеком, истоки которіх автор видит в христианском средневековье. Жизнь и творческое наследство Иоанна Золотоустого – яркий пример объяснения сущности и определение ценностной природы душпастирства сквозь призму понятия священства.The article by Svitlana Bilous "Axiological dimensions of priesthood in the Ioan Zolotoustyi’s works" is dedicated to finding sources of spiritual care over a human, the origin of which the author sees in the Christian Middle Ages. The life and literary heredity of Ioan Zolotoustyi is a brilliant pattern of explaining the essence and definition of valuable ​​nature of pastoral care through the prism of the concept of the priesthood

Neuropathological consensus criteria for the evaluation of Lewy pathology in post-mortem brains: a multi-centre study

Currently, the neuropathological diagnosis of Lewy body disease (LBD) may be stated according to several staging systems, which include the Braak Lewy body stages (Braak), the consensus criteria by McKeith and colleagues (McKeith), the modified McKeith system by Leverenz and colleagues (Leverenz), and the Unified Staging System by Beach and colleagues (Beach). All of these systems use semi-quantitative scoring (4- or 5-tier scales) of Lewy pathology (LP; i.e., Lewy bodies and Lewy neurites) in defined cortical and subcortical areas. While these systems are widely used, some suffer from low inter-rater reliability and/or an inability to unequivocally classify all cases with LP. To address these limitations, we devised a new system, the LP consensus criteria (LPC), which is based on the McKeith system, but applies a dichotomous approach for the scoring of LP (i.e., “absent” vs. “present”) and includes amygdala-predominant and olfactory-only stages. α-Synuclein-stained slides from brainstem, limbic system, neocortex, and olfactory bulb from a total of 34 cases with LP provided by the Newcastle Brain Tissue Resource (NBTR) and the University of Pennsylvania brain bank (UPBB) were scanned and assessed by 16 raters, who provided diagnostic categories for each case according to Braak, McKeith, Leverenz, Beach, and LPC systems. In addition, using LP scores available from neuropathological reports of LP cases from UPBB (n = 202) and NBTR (n = 134), JT (UPBB) and JA (NBTR) assigned categories according to all staging systems to these cases. McKeith, Leverenz, and LPC systems reached good (Krippendorff’s α ≈ 0.6), while both Braak and Beach systems had lower (Krippendorff’s α ≈ 0.4) inter-rater reliability, respectively. Using the LPC system, all cases could be unequivocally classified by the majority of raters, which was also seen for 97.1% when the Beach system was used. However, a considerable proportion of cases could not be classified when using Leverenz (11.8%), McKeith (26.5%), or Braak (29.4%) systems. The category of neocortical LP according to the LPC system was associated with a 5.9 OR (p < 0.0001) of dementia in the 134 NBTR cases and a 3.14 OR (p = 0.0001) in the 202 UPBB cases. We established that the LPC system has good reproducibility and allows classification of all cases into distinct categories. We expect that it will be reliable and useful in routine diagnostic practice and, therefore, suggest that it should be the standard future approach for the basic post-mortem evaluation of LP