ZEB1 as an additional predictor of tumor progression in Ewingʼs sarcoma. Results of a morphological study on a population of children and adolescents

Relevance. Ewingʼs sarcoma (ES) is a classic representative of the extensive family of ES tumors, which occupies one of the leading positions among the malignant pathology of the musculoskeletal system in children and adolescents. This group is characterized by an extremely large variety of morphological, immunohistochemical, and molecular genetic characters among its representatives. The absence of specific pathognomonic markers for ES, as well as the presence of wide variability of clinical manifestations complicates the differential diagnosis. Materials and methods. The study included patients of childhood and adolescence with a localized and generalized form of ES/PNEТ of various localizations undergoing treatment in the conditions of the Department of Pediatric Oncology of the Federal State Budgetary Research Center for Oncology from 2009 to 2019. As the material, the tissue of the primary tumor of ES/PNEТ from paraffin blocks was used, obtained from 67 patients during the primary biopsy, as well as after the surgical stage as part of a combined or complex treatment. The expression of ZEB1 was determined immunohistochemically.Results. The highest average level of expression of ZEB1 protein was observed in group 4 with a generalized form of ES (surgical material) and amounted to 60.8 ± 2.2 %, the minimum level was detected in group 2 with a localized form of ES (surgical material) and amounted to 29.2 ± 3.0 %. Between groups 2 (localized form) and 4 (generalized form) statistically significant differences were noted (p = 0.026).Conclusion. As a result of an immunohistochemical study, the ZEB1 protein showed its prognostic significance when comparing groups with a localized and generalized form of ES (p = 0.026). The predominance of the expression level of ZEB1 protein in the group with the generalized form statistically significantly increased the chances of metastasis by 3.6 times (95 % CI 1.13-11.8).

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Epicardial obesity as a significant preclinical predictor of atherosclerosis of brachiocephalic arteries

European Heart Journal ◽

10.1093/ehjci/ehaa946.3026 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Ott ◽

G.A Chumakova

Keyword(s):

Cardiometabolic Risk ◽

Subclinical Atherosclerosis ◽

Prognostic Significance ◽

Clinical Manifestations ◽

Area Under The Curve ◽

Visceral Obesity ◽

Threshold Value ◽

Group 2 ◽

Brachiocephalic Arteries ◽

Group 1

Abstract Obesity is one of the significant factors of cardiovascular risk. Nowadays it is understood that it is visceral obesity (VO), which has metabolic activity due to the synthesis of adipokines, that determines cardiometabolic risk. The effect of epicardial obesity (EO), as a variant of VO on the formation of cardiometabolic risk (in particular coronary atherosclerosis) is being actively studied. The role of EO in the development of atherosclerosis of other localizations has been little studied. Objective To study the predictor value of EO as well as traditional criteria for obesity: body mass index (BMI) and waist circumference (WC) for the formation of atherosclerosis of brachiocephalic arteries (BCA). Materials and methods The study included 140 men 45.2±4.3 years old with arterial hypertension (AH) of the 1–3 degree and the absence of clinical manifestations and anamnesis of atherosclerosis of any localizations with a BMI of 20–35 kg /m2 and abdominal obesity according to WC ≥94 cm. Patients were divided into two groups depending on the thickness of the epicardial adipose tissue (EAT) measured behind the free wall of the right ventricle by echocardiography. Group 1 consisted of 60 patients with epicardial obesity (EAT ≥7 mm), group 2 included patients without epicardial obesity (EAT <7 mm). Subclinical atherosclerosis of BCA was evaluated in all subjects using duplex brachiocephalic arteries (BCA). Results When assessing the thickness of the intima-media of the carotid arteries (TIM), a subclinical marker of BCA atherosclerosis, higher average TIM values in group 1 (EAT ≥7 mm) were revealed (1.09±0.34 mm versus 0.74±0, 05 mm in group 2 (EAT <7 mm) (p=0.0001). Prevalence of subclinical BCA atherosclerosis from (20–45%) in group 1 patients was found in 57%, in group 2 only 4% (p=0.01). In the first group, hemodynamically significant asymptomatic BCA stenosis (50–65%) was found in 8% of patients. No hemodynamically significant BCA stenosis was detected in the second group. Using ROC analysis, the threshold value of EAT (9.25 mm) was obtained as a risk factor for hemodynamically significant stenoses of BCA (50% or more) with high prognostic significance (the area under the curve was 0.92). Using multivariate analysis of variance, the effect of various criteria of obesity on the formation of BCA atherosclerosis was studied. As a result of the analysis it was revealed that only EAT (p=0.02) influenced the development of BCA atherosclerosis (TIM more than 1.3 mm). WC and BMI did not affect the development of BCA subclinical atherosclerosis (p=0.21; p=0.24, respectively). Conclusions EO (EAT ≥7 mm) is an early marker of BCA subclinical atherosclerosis in contrast to the traditional criteria for obesity (BMI, WC). Patients with EAT of 9.25 mm or more need additional examinations and the appointment of pharmacotherapy aimed at the prevention of secondary complications. Funding Acknowledgement Type of funding source: None

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Fundamentals of psychological correction of children and adolescents

10.12737/1085328 ◽

2021 ◽

Author(s):

Stanislav Savinkov ◽

Ekaterina Barysheva ◽

Veronika Zolotova ◽

Valentina Kozyreva ◽

Elena Pozdnyakova ◽

...

Keyword(s):

Higher Education ◽

Children And Adolescents ◽

Educational Institutions ◽

Federal State ◽

Childhood And Adolescence ◽

Educational Standards ◽

Joint Work ◽

Wide Range ◽

Psychological Assistance ◽

State Educational

The textbook, which is the result of the joint work of the author's team, examines various approaches to the nature of the child's psyche, the principles, tasks and specifics of psychological correction in childhood and adolescence. The main scientific and most practice-oriented areas of psychotherapy and development correction, as well as specific techniques and methods for working with children and adolescents, are presented. Meets the requirements of the federal state educational standards of higher education of the latest generation. For students of higher educational institutions studying in the direction of "Psychological and pedagogical education" (qualification "bachelor"), as well as for a wide range of specialists dealing with the problems of correcting the development of children and adolescents, providing psychological assistance.

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Clinical Significance of Homozygous D13S319 Deletion in B-Cell Chronic Lymphocytic Leukemia (B-CLL).

Blood ◽

10.1182/blood.v104.11.2799.2799 ◽

2004 ◽

Vol 104 (11) ◽

pp. 2799-2799 ◽

Cited By ~ 2

Author(s):

Stephanie Fink ◽

Susan Geyer ◽

Tait Shanafelt ◽

Stephanie Smoley ◽

Sarah Paternoster ◽

...

Keyword(s):

Clinical Significance ◽

Prognostic Significance ◽

Lymphocytic Leukemia ◽

Median Percentage ◽

Time To Treatment ◽

Treatment Status ◽

Group 4 ◽

Group 3 ◽

Group 2 ◽

Group 1

Abstract Background: In B-CLL, the observation of interphase cells with hemizygous D13S319 deletion at 13q14 (13q-x1) as a sole anomaly in blood is widely considered a favorable prognosis. The observation of cells with 11q-, +12 or 17p- has been associated with a relatively poor prognosis. Over the past 1.5 yrs, 16.2% (174/1,076) of patients (pts) referred for fluorescence in situ hybridization (FISH) testing for B-CLL in our clinical practice had a clone with homozygous D13S319 deletion (13q-x2), but the prognostic significance of this observation is poorly understood. Moreover, 39.3% (142/361) of pts with unfavorable FISH anomalies have 13q-x1 and/or 13q-x2 and the clinical significance of this observation is also unknown. Thus, we investigated pts with 13q- (with or without other chromosome anomalies) to establish the relative clinical significance of 13q- in B-CLL. Methods: We studied 333 pts with B-CLL sampled between 9/1999 and 6/2004 who had FISH performed on interphase nuclei from blood. The FISH probe set was designed to detect 6q-, 11q-, +12, 13q-, 17p-, and translocations involving IgH at 14q32. We classified pts into four groups: 13q-x1 only (group 1), 13q-x1 and 13q-x2 only (group 2), 13q-x2 only (group 3) and 13q-x1 and/or 13q-x2 plus other FISH anomalies (group 4). FISH groups were compared with gender, age, Rai stage, treatment status, time to treatment, CD38 and IgVH mutation. Results: Of the 333 pts, 171 (51.3%) had a 13q-: 71 were in group 1, 25 in group 2, 26 in group 3 and 49 in group 4. %CD38+ differed significantly across FISH groups; in pairwise analyses, the proportion of pts with >30% CD38+ was significantly greater for pts in group 4 vs. group 3 (p=0.0015) although no significant differences were observed for group 3 vs. group 1 or vs. group 2. Pts in group 3 were not significantly different from other FISH groups for Rai stage, IgVH mutation or gender. The median percentage of abnormal nuclei for pts with group 1 was 54.5% vs. 79.5% for pts in group 4 (p<0.0001). The median percent abnormal nuclei for pts in groups 3 and 2 was 72.5% and 68.5%, respectively. Median % abnormal nuclei for group 3 was not significantly different than the other FISH groups. Treatment status was available on 147 pts, where the proportion of pts who had treatment in each group was as follows: group 1, 15/66; group 2, 2/22; group 3, 4/21; and group 4, 9/38. Due to limited sample size and heavy censoring, any analysis on time to treatment is preliminary; however, these early analyses suggest group 4 pts have a lower median time to treatment (9 yrs) compared to groups 3 and 1 (12.3 and 13 yrs, respectively). Conclusions: This study has generated new information about the 13q- anomaly in B-CLL. First, known prognostic markers for B-CLL pts with 13q-x2 are not significantly different than for pts with 13q-x1 or 13q-x1/13q-x2. Second, 13q-x1 and/or 13q-x2 occurring with other unfavorable FISH anomalies have an unfavorable prognosis; i.e. potential benefits of 13q- are trumped when it is observed with unfavorable FISH anomalies. Thus, patients with any form of 13q- alone may have indolent disease while patients with 13q- and unfavorable FISH anomalies should be considered to be in a more aggressive phase.

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The effect of Rosa canina L. and a polyherbal formulation syrups in patients with attention deficit/hyperactivity disorder: A study protocol for a multicenter randomized controlled trial

10.21203/rs.3.rs-345669/v1 ◽

2021 ◽

Author(s):

Haide Golsorkhi ◽

Mostafa Qorbani ◽

Mohammad Kamalinejad ◽

Saeideh Sabbaghzadegan ◽

Mohsen Bahrami ◽

...

Keyword(s):

Attention Deficit Hyperactivity Disorder ◽

Children And Adolescents ◽

Attention Deficit ◽

Clinical Manifestations ◽

Behavioral Disorder ◽

Childhood And Adolescence ◽

Rosa Canina ◽

Hyperactivity Disorder ◽

Polyherbal Formulation ◽

Multicenter Randomized Controlled Trial

Abstract Background: Attention deficit/hyperactivity disorder (ADHD) is the most common behavioral disorder in childhood and adolescence. A number of these patients do not respond to the current pharmacological treatments and there may also be drug side effects. This study aims to determine the efficacy and safety of two herbal medicine products, including Rosa canina L. (RC) and a polyherbal formulation (PHF) syrups on the clinical manifestations of ADHD in children and adolescents.Methods: Ninety ADHD patients based on DSM-5 diagnostic criteria will be randomly assigned equally to three groups: (1) RC syrup + methylphenidate (MP); (2) PHF syrup + MP; (3) placebo + MP according to the inclusion criteria (30 subjects in each group). The syrups dosage is 5 cc every 8 hours and MP has a stabilized dose for 8 weeks during the study. Moreover, Conner’s questionnaires will be completed by the teacher and parents before the intervention and then every 4 weeks. Also, the child symptom inventory-fourth edition (CSI-4) and temperament questionnaires will be completed before the intervention and every four weeks until two months.Discussion: This trial is the first experiment to determine the effects of RC and PHF syrups on the clinical manifestations of ADHD in children and adolescents. Our findings provide new insight into the effect of these herbal products on the clinical manifestations of ADHD.Trial registration: The trial was registered at https://en.irct.ir/ (Registration number: IRCT20190923044855N1). Registration date: 2020-01-14.

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Implementation of molecular genetic tests in the diagnostics of thyroid cancer: own research

Clinical endocrinology and endocrine surgery ◽

10.30978/cees-2021-2-9 ◽

2021 ◽

pp. 9-16

Author(s):

O. P. Nechay ◽

O. A. Tovkai ◽

V. O. Palamarchuk ◽

N. I. Belemets ◽

S. І. Nikolayenko ◽

...

Keyword(s):

Thyroid Cancer ◽

Molecular Genetic ◽

Prognostic Significance ◽

Pathogenic Mutation ◽

Endocrine Surgery ◽

Pathogenic Mutations ◽

Endocrine Organs ◽

Group 2 ◽

Group 1

Aim — to investigate the significance of BRAFV600E, NRAS, KRAS, HRAS, RET/PTC (RET/PTC1 and RER/PTC3), PAX8/PPARg mutations for the development and course of thyroid cancer with the determination of a significant predictor mutation. Materials and methods. The analysis included 63 selected case histories of patients who, at the preoperative stage, underwent molecular genetic testing (MHT) of the thyroid gland (TG) masses and subsequently underwent surgical treatment in the surgical department of the Ukrainian Scientific and Practical Center for Endocrine Surgery, Transplantation of Endocrine Organs and Tissues. The average age of the patients was 41.0 ± 1.8 years; from them 7men and56women. All patients underwent fine-needle aspiration puncture biopsy(FNAB) of thyroid nodules according to the standard method followed by a cytological conclusion in accordance with the Bethesda system.The Bethesda III category was revealed in 3 patients (4.8 %), Bethesda IV — 13 patients (20.6 %), Bethesda V — 12 patients (19.0 %), Bethesda VI in 35 (55.6 %) patients. The pathogenic mutations were detected in 47 (74.6 %) patients (group 1), among them two mutations were simultaneously found in two subjects. In 16 cases (25.4 %), no pathogenic mutation was found at all (group 2). Results. The genes that occurred most often were BRAFV600E — in 35 patients (55.6 %), NRAS — in 11 patients (17.5 %), KRAS — in 3 patients (4.8 %). In case of thyroid cancerdiagnosis, pathogenic mutations were found in 38 (79.1 %) subjects. The BRAFV600E gene mutation was observed when establishing a cytological conclusion classified as Bethesda III—V in 28.9 %, and in Bethesda V and in 77.1 %. Accordingly, the sensitivity of the test was low — 0.646, specificity — 0.733. The high prognostic significance of a positive result(PPV) value (from 0.784 to 0.943) indicated the likelihood of detecting thyroid cancer. This assumption confirms the calculation of the c-square criterion with the Yates correction, which is 5.207 (p = 0.023). The use of this test for the presence of the NRAS gene to detect thyroid cancer was ineffective, the value of the c -square criterion with Yates correction = 0.009 (p = 0.927). The incidence of thyroid cancer in group 1 in the cytological class Bethesda III—V was higher than in group 2, but it did not differ significantly between the groups. The aggressiveness of thyroid cancer in patients of group 1 with a positive MHT result did not have significant differences compared with the results obtained in group 2 (39.5 % and 33.3 %, respectively). Conclusions. The use of the kit for the determination of MHTmade it possible to identify pathogenic mutations in the genes BRAFV600E, NRAS, KRAS in 79.1 % of cases. The presence of these genes in combination with the analysis of cytomorphological findings classified according to the Bethesda III—V system did not increase the detection of thyroid cancer in the studied patients. The BRAFV600E gene, which was observed in 64.6 % of cases (PPV from 0.784 to 0.943), was a significant predictor among the studied candidate genes for establishing the diagnosis of thyroid cancer. Detection of a pathogenic mutation in patients with thyroid cancer did not indicate in favour of its aggressive course.

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RETROSPECTIVE ANALYSIS OF FEATURES OF DETECTION OF NEW CORONOVIRAL INFECTION COVID-19 IN PATIENTS WITH SURGICAL PROFILE ARRIVING IN A EMERGENCY

СИСТЕМНЫЙ АНАЛИЗ И УПРАВЛЕНИЕ В БИОМЕДИЦИНСКИХ СИСТЕМАХ ◽

10.36622/vstu.2021.20.3.019 ◽

2021 ◽

pp. 139-148

Author(s):

Дмитрий Валериевич Судаков ◽

Евгений Владимирович Белов ◽

Олег Валериевич Судаков ◽

Ольга Игоревна Гордеева ◽

Оксана Александровна Андросова

Keyword(s):

Clinical Symptoms ◽

Positive Impact ◽

Clinical Manifestations ◽

Reactive Protein ◽

Group 4 ◽

Group 3 ◽

Ct Data ◽

Group 2 ◽

Group 1

В статье рассматриваются особенности своевременного выявления новой короновирусной инфекцией (НКИ) у пациентов хирургического профиля на уровне приемного отделения областной клинической больницы. Представленная работа является в настоящее время крайне актуальной, в виду продолжающейся в мире пандемии НКИ - Covid-19. Важное значение данному исследованию придает то, что оно направлено на как можно более раннюю диагностику Covid-19, уже на уровне нахождения в приемном отделении при поступлении в многопрофильный стационар. Подобные меры могут существенно помочь в сохранении здоровья медицинских работников и могут оказать положительное влияние на эпидемиологическую обстановку в целом. Интересной составляющей данного исследования является и то, что диагностика Covid-19 основывается лишь на тех лабораторных анализах, которые можно быстро определить в условиях приемного отделения. Объектами исследования послужило 200 пациентов, поступающих в приемное отделение БУЗ ВО ВОКБ №1 исключительно с хирургической патологией. Все больные были подразделены на 4 группы по 50 человек, всем им был поставлен сопутствующий диагноз Covid-19. В 1 группу вошли пациенты, поступающие в приемное отделение без сознания, сбор анамнеза и сбор жалоб у которых был невозможен. 2 группу составили пациенты, у которых НКИ была диагностирована непосредственно после оперативного вмешательства. В 3 группу вошли пациенты, у которых была выявлена НКИ, но объем оперативного вмешательства и объем поражения легких позволял им дальнейшее амбулаторное лечение. 4 группу составили пациенты, у которых Covid-19 выявлялся в течение 3 дней после оперативного вмешательства. У всех больных подробно собирался анамнез жизни и эпидемиологический анамнез (за исключением 1 группы), анализировались наиболее частые клинические проявления и показатели общего и биохимического анализа крови, обрабатывались данные КТ легких. Были определены основные клинические симптомы НКИ, такие как: повышенная температура тела, кашель, слабость и утомляемость. Наиболее важными лабораторными показателями стало определение уровня лейкоцитоза, с уровнем нейтрофилов и лимфоцитов, а также СОЭ, D-димера, С-реактивного белка. Представленная статья представляет большой интерес для врачей, работающих в условиях приемных отделений многопрофильных стационаров. По результатам работы планируется продолжение исследования с большей выборкой пациентов и большим количеством изучаемых параметров The article discusses the features of the timely detection of a new coronavirus infection (NCI) in surgical patients at the level of the admission department of a regional clinical hospital. The presented work is currently extremely relevant, in view of the ongoing NСI pandemic in the world - Covid-19. The importance of this study is attached to the fact that it is aimed at the earliest possible diagnosis of Covid-19, already at the level of being in the admission department upon admission to a multidisciplinary hospital. Such measures can significantly help preserve the health of medical workers and can have a positive impact on the epidemiological situation in general. An interesting component of this study is the fact that the diagnosis of Covid-19 is based only on those laboratory tests that can be quickly determined in the conditions of the admission department. The objects of the study were 200 patients admitted to the admission department of BUZ VO VOKB № 1 exclusively with surgical pathology. All patients were divided into 4 groups of 50 people, all of them were diagnosed with a concomitant Covid-19 diagnosis. Group 1 consisted of patients admitted to the emergency department unconscious, and it was impossible to collect anamnesis and collect complaints. Group 2 consisted of patients in whom NCI was diagnosed immediately after surgery. Group 3 included patients who had NCI, but the volume of surgery and the volume of lung lesions allowed them further outpatient treatment. Group 4 consisted of patients in whom Covid-19 was detected within 3 days after surgery. In all patients, a detailed life history and epidemiological history were collected (except for group 1), the most frequent clinical manifestations and indicators of general and biochemical blood tests were analyzed, and CT data of the lungs were processed. The main clinical symptoms of NCI were identified, such as: fever, cough, weakness and fatigue. The most important laboratory indicators were the determination of the level of leukocytosis, with the level of neutrophils and lymphocytes, as well as ESR, D-dimer, C-reactive protein. The presented article is of great interest to doctors working in the admission departments of multidisciplinary hospitals. Based on the results of the work, it is planned to continue the study with a larger sample of patients and a large number of studied parameters

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The Modulating Effect of Azacitidine (Aza C) on the Cytogenetically Tracked MDS Clone Impact Survival.

Blood ◽

10.1182/blood.v104.11.1429.1429 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1429-1429 ◽

Cited By ~ 5

Author(s):

Vesna Najfeld ◽

Angela Scalise ◽

Rosalie Odchimer-Reissig ◽

Lewis R. Silverman

Keyword(s):

Therapy Group ◽

Bone Marrow Failure ◽

Prognostic Significance ◽

Survival Group ◽

Hematopoietic Stem ◽

Group 4 ◽

Clinical Consequences ◽

Group 3 ◽

Group 5 ◽

Group 2

Abstract MDS is a clonal hematopoietic stem cell disorder characterized by progressive bone marrow failure leading to death from infections and bleeding in the majority of patients. Transformation to acute leukemia occurs in 35-40%. Most treatments, other than allogeneic SCT, have been ineffective. Treatment with Aza C has been shown to be clinically effective and alters the natural history of disease (Silverman et al JCO 2002). We have previously demonstrated Aza C modulation of the MDS clone and established five different cytogenetic categories based on its modulating effect (Najfeld et al, ASH 2002). The goal of this extended study was to determine biological and clinical consequences of Aza C modulation on the five cytogenetic categories. Among 224 patients treated with AzaC, multiple sequential studies were performed in187 pts, while 41 pts without follow up studies, were excluded from this report. The initial karyotype prior to therapy with Aza C was normal in 99 pts (53%) and abnormal in 88 pts (47%). These latter patients were not further subdivided into good, intermediate or poor risk according to IPSS. The table below shows the Kaplan Meier median survival of the 5 subcategories based on the modulating effects of Aza C on the cytogenetically identified MDS clone. Cyto Category No of Frequency of Hematological Survival Group Pts Emerging Response In No Chromosome Abnormalities Months 1 Normal->Normal 67 None CR:3, PR:10, IMP:31 29.3 2 Normal->Abnormal 32 +8:25%l+1q:25% CR:1, PR:6, IMP:18 28.1 +21:9% 3 Abnormal-> Abnormal 50 −7/del(7q):24%; CR:1, PR:4, IMP:30 11.7 −5/del(5q):20%,+1q:14% 4 Abnormal->Clonal 20 −7/del(7q):25%;+21:20% IMP:8 9.1 5 Abnormal->Normal 18 del(7q):33%,+8,28% CR:1, PR:1,IMP:8 22.7 When survival was compared between cytogenetic subgroups there was a statistical difference between some groups. Survival of patients with normal cytogenetics (group 1) was superior when compared to either group 3 (p=.0001) or group 4 (p=.00015). Even for pts who developed an abnormal clone while on AzaC (group 2) survival was significantly longer when compared to either group 3 (p=0004) or group 4 (p=00001). Moreover, prognostic significance was observed for pts who initially had an abnormal karyotype that was diminished or eradicated on AZA C therapy (group 5) when compared to groups 3 (p=0.009) and 4 (p-0.0006). The emergence of a cytogenetically abnormal clone while on treatment with Aza C is not associated with shortened survival compared to those who remain cytogenetically normal. Response to Aza C can occur even with the persistence of the MDS clone or with the emergence of a new clone. This suggest that AZA C can modulate the responsivness of MDS hematopoietic progenitors. Our findings demonstrate that AZA C modulation of the cytogenetically marked MDS clone is associated with distinct subgroups with differing survival characteristics.

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Assessment of the quality of routine ambulatory healthcare for common disorders in children and adolescents in Germany: study protocol for a retrospective medical record review (QualiPäd)

BMJ Open ◽

10.1136/bmjopen-2021-048782 ◽

2021 ◽

Vol 11 (11) ◽

pp. e048782

Author(s):

Viktor Tischlik ◽

Claudia Mehl ◽

Dominik Ewald ◽

Monika Heinzel-Gutenbrunner ◽

Max Geraedts ◽

...

Keyword(s):

Quality Of Care ◽

Children And Adolescents ◽

Quality Indicators ◽

Quality Of Healthcare ◽

Federal State ◽

Childhood And Adolescence ◽

Oppositional Defiant ◽

Child Psychiatric ◽

Clinical Conditions

IntroductionThe quality of healthcare in childhood and adolescence is of key importance, in order to foster a healthy development and to avoid chronic health problems. Yet, data for Germany regarding the quality of healthcare for this patient group are lacking. The QualiPäd research project aims to estimate the quality of outpatient healthcare for children and adolescents in Germany, focusing on common psychiatric and physical disorders.Methods and analysisQuality indicators for seven common physical and mental childhood and adolescent clinical conditions (attention deficit/hyperactivity disorder, asthma, atopic dermatitis, depression, otitis media, conduct disorder/oppositional defiant disorder, tonsillitis) will be developed and ratified by experts, using the RAND/UCLA Appropriateness Method.Initially, 1400 medical records of children and adolescents with one of the aforementioned clinical conditions will then be randomly drawn from 40 outpatient practices in the German federal state of Hessen. The records will then be assessed regarding their adherence to the respective quality indicators. Based on this, the percentage of appropriate and inappropriate (eg, wasteful) healthcare of all clinical conditions (primary endpoint) will be estimated. Additionally, possible factors influencing the quality of care (eg, patient characteristics, type of condition, type of practice) will be identified using generalised estimation equation models.Ethics and disseminationThis study will show for which of the studied clinical conditions and/or patients improvement of quality of care is necessary within the German health system. Also, the quality indicators designed for the study can afterwards be implemented in regular care and thus enable regular reporting of the outpatient care of this target group. The authors plan to disseminate their findings through international, peer-reviewed scientific publications, and through presentations at national and international paediatric and child psychiatric conferences.Trial registration numberDRKS00022408.

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Characterization of Anti–Interferon-γ Antibodies in HIV-Negative Patients Infected With Disseminated Talaromyces marneffei and Cryptococcosis

Open Forum Infectious Diseases ◽

10.1093/ofid/ofz208 ◽

2019 ◽

Vol 6 (10) ◽

Cited By ~ 2

Author(s):

Wen Zeng ◽

Ye Qiu ◽

Shudan Tang ◽

Jianquan Zhang ◽

Mianluan Pan ◽

...

Keyword(s):

Clinical Laboratory ◽

Critical Role ◽

Clinical Manifestations ◽

Interferon Γ ◽

White Cell ◽

Talaromyces Marneffei ◽

Group 4 ◽

Group 2 ◽

Routine Clinical Laboratory ◽

Hiv Negative

Abstract Background Few reports of Talaromyces marneffei (TM) or cryptococcosis infections among HIV-negative patients with high-titeranti–IFN-γautoantibodies (nAIGAs) have been published. We investigated the clinical manifestations of patients with nAIGAs and TM infections. Methods HIV-negative adults (≥18 years) were enrolled if they haddisseminated TM infection (group 1; further divided into nAIGAs positive [group 1P] and negative [group 1N]); cryptococcosis(pulmonary cryptococcosis and/or cryptococcosis of the brain)(group 2); pulmonary tuberculosis (group 3); and healthy controls (group 4) with nAIGAs detected. Complete histories, physical examinations, and routine clinical laboratory tests were obtained at baseline. Results Overall, 88 participants were in the four groups (20,13,23, and 32 in groups 1 to 4, respectively). Significant differences occurred between groups with higher nAIGAs titers (P < 0.001), and higher total white-cell and absolute neutrophil counts (P < 0.001) in group1. Lungs (90.0%), lymph nodes (60.0%), skin (55.0%), and bones (50.0%) were most common sites of involvement. Significant differences in total white-cell and absolute neutrophil counts occurred between groups IP and 1N.Patients with recurrent TM infections, particularly group 1P, had higher initial nAIGA titer. Conclusions Patients with persistent infection who died tended to have positive initial nAIGA titer. It suggests that nAIGAs may play a critical role in the pathogenesis of TM infections, and may be associated with more severe, refractory infection.

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Unfavourable Outcome and Heterogeneity of Partner Chromosomes in Chronic Lymphocytic Leukemia with 14q32/IGH Translocations.

Blood ◽

10.1182/blood.v110.11.4686.4686 ◽

2007 ◽

Vol 110 (11) ◽

pp. 4686-4686

Author(s):

Francesco Cavazzini ◽

Jose A. Hernandez ◽

Alessandro Gozzetti ◽

Antonella Russo Rossi ◽

Ruana Tiseo ◽

...

Keyword(s):

Statistical Significance ◽

Hematological Parameters ◽

Prognostic Significance ◽

Risk Groups ◽

Lymphocytic Leukemia ◽

Group 4 ◽

Group 3 ◽

The Difference ◽

Group 2 ◽

Group 1

Abstract Translocations of 14q32/IgH have a low incidence in CLL. Partner chromosomes and the prognostic significance are poorly defined. Four hundred thirty cases of CLL seen at the Hospitals of Ferrara, Salamanca and Siena between 1992 and 2006 were studied. Inclusion criteria were: diagnosis of CD5/CD19+ CLL with k/λ restriction, cytogenetic/FISH data, immunophenotypic data, complete hematological and clinical data. Lymphomas in leukemic phase were excluded. FISH was performed for 17p13/TP53, 11q22.3/ATM, 6q21, chr 12 centromere, 13q14 and 14q32/IGH. Patients with no detectable aberration or isolated 13q− were included into a favourable cytogenetic group (group 1), those with +12, 6q− or 1–2 aberrations into an intermediate risk group (group 2) and those with 17p−, 11q−, ≥ 3 aberrations into an unfavourable group (group 3). Cases with 14q32/IGH translocation as primary chromosome change represented a specific category (group 4) and were studied with FISH probes for the detection of partners (BCL1, BCL2, BCL3, BCL6, c-MYC, BCL11A, PAX5, CCND3, CDK6). One hundred eighty-six cases were allocated into group 1; 158 into group 2; 64 into group 3, and 22 into group 4. Additional aberrations were found in a minority of cells in 8 patients in group 4. Being the aim of the study to assess whether the 14q32/IGH translocation represented an unfavourable parameter as compared with cases in the favourable or intermediate cytogenetic risk groups, cases within group 3 were excluded from the analysis and the data presented here will refer to 366 patients belonging to groups 1,2 and 4. Translocation partners of 14q32/IGH were identified in 9/22 cases: 2p13/BCL11A, (n 1); 6p21/CCND3 (n 1); 7q21/CDK6 (n 1); 18q21/BCL2 (n 6). Thirteen cases did not show involvement of the loci studied. Cases with 14q32/IGH translocations were characterized by typical morphology and classical immunophenotype (score 4–5 in 92% of the cases). Unmutated IGVH genes were found in 11/18 cases tested (61%); ZAP-70 was positive in 3/5 cases tested. Median age was 63.5 years (range 18–97), male:female ratio 240/126; 345 patients were in Rai stage 0-II, 21 were in stage 3–4; CD38 was positive in 137/366 cases. There was no difference between groups 1,2 and 4 for age, stage, male:female ratio, hematological parameters at diagnosis, IGVH mutations and ZAP70. CD38 was more positive in group 4 than in group 1 (p=0.028). There was no difference in survival and treatment free interval (TFI) when comparing cases in group 4 with and without additional aberrations. Cases in group 4 had a shorter TFI and a shorter survival when compared with group 1 (p=0.02) and group 2 (p=0.02). The difference maintained its statistical significance at multivariate analysis for TFI (p=0.02) along with stage (p<0.0001) and CD38 positivity (p<0.0001) and for survival (p=0.02) along with sex (p=0.006) and stage (p=0.0001). In conclusion, the 14q32/IGH translocation in CLL shows heterogeneity of partner chromosomes and it represents a cytogenetic marker predicting for an evolutive form of CLL.

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