First Step Research Articles

Understanding real-world treatment patterns of patients with advanced ovarian cancer in the first-line maintenance setting: The FIRST STEP Study

Understanding real-world treatment patterns of patients with advanced ovarian cancer in the first-line maintenance setting: The FIRST STEP Study

KAZAKHSTAN EXPERIENCE OF VENTURE CAPITAL FINANCING OF TECHNOLOGY ENTREPRENEURS

The economy cannot function without a high-quality technology production sector. To do this, a connection between science and business must be established. Supporting technology entrepreneurship is the fi rst step in building communication amongst them.Technological entrepreneurship is a high level of commercialization of high-tech technologies with high added value. Technology-based entrepreneurship needs an eff ective ecosystem where venture capital investment is developing, as it is a high-risk investment tool. The following indicators demonstrate the relevance of the study: R&D expenditures in the country’s GDP have signifi cantly decreased over the past 10 years; the rating of the Republic of Kazakhstan in GEM, according to the indicator «Access to entrepreneurial fi nance» – 38th place out of 45 countries; the rating in the Venture Capital & Private Equity Country Attractiveness Index – 54th place.The purpose of the study is an analytical and empirical review of venture investment in technological entrepreneurship in the Republic of Kazakhstan.The research methodology is based on the methods of basic statistics, logical analysis, correlation and regression analysis, questionnaires, and content analysis.The originality of the work lies in the study of a phenomenon of gaining momentum in the country as venture fi nancing of technological entrepreneurship, through a survey of technological entrepreneurs of the Republic of Kazakhstan.The results of the study show that venture fi nancing signifi cantly aff ects the development of technological entrepreneurship. However, this method of fi nancing is mainly available only to large enterprises. The authors come to the conclusion that techno-trainers fi nd access to venture fi nancing moderately diffi cult and mainly rely on personal fi nances.

Assessment of biomechanical parameters of feet in patients with multiple sclerosis during walking

Rationale. Walking and balance disorders, modi fi ed foot muscles kinematics and walking stereotypes are typical for the patients with Multiple Sclerosis (MS).Objective. To assess the biomechanical parameters of the feet in MS patients with diff erent neurological status during walking.Material and methods. Data analysis of 102 patients with relapsing-remitting MS was carried out for two groups depending on the value of the Expanded Disability Status Scale (EDSS). Plantar pressure distribution was measured on with the pedographic platform emed, novel gmbh, Munich, Germany, using a “fi rst step” protocol.Results and conclusion. Loading of the heel, the area of central metatarsal heads is decreased and loading of the toes is increased during walking in MS patients. Diff erences were found for the spreading coeffi cient (the ratio of the width to the length of the foot), the dynamic width of the foot in patients with an EDSS ≥ 4 is signi fi cantly less in the instep, in the middle and in the narrowest parts of the foot, which leads to the decrease of the contact area. It has been shown that patients with MS who are mobile or walk without assistance are more likely to have pes cavus foot compared to MS patients with minor impairments. The load of the medial side of the foot is greater than the lateral side, which indicates the predominance of the valgus foot. The pronation-supination index is signi fi cantly higher in the patients with moderate impairments in toe-off phase. Evaluation of foot deformity based on the measurement of biomechanical parameters of plantar pressure distribution makes it possible to plan the treatment and rehabilitation tactics in MS patients.

THE FIRST STEP TO THE SOUTH AFTER HALF A CENTURY. CAN THE "SUNSHINE" POLICY MELT FROZEN RELATIONS?

THE FIRST STEP TO THE SOUTH AFTER HALF A CENTURY. CAN THE "SUNSHINE" POLICY MELT FROZEN RELATIONS?

Legis Actio in Rem and Adolf Reinach’s Theory on Social Acts

In an essay published in 2002, Ulrich Manthe analysed the ancient legis actio sacrament in rem in the light of the speech act theory shaped, above all, by John L. Austin (1911–1960). Almost half a century before, Austin Adolf Reinach, an outstanding member of the phenomenological school founded by Edmund Husserl, developed a theory of illocutionary acts from the perspective of realistic legal philosophy. One of the essential elements of the model developed by Reinach is the theory of social acts. In the fi rst step, the paper focuses on the opening part of the legis actio sacramento in rem. It shows that concerning the activity of the parties during the initial ritual of the law enforcement process, we discover the development from the acts of conviction and assertion, which still do not arrive at the state of social acts, to the social act of informing the other party about the claim. The paper also tries to approach the activity of the praetor and the judge through the theory of social acts. The “mittite ambo rem” on behalf of the praetor is qualifi ed technically as the social act of a command. Without arriving at a fi nal result, the paper makes a first attempt to apply the categories of commandment and enactment to the activities of the praetor and the judge. It leaves this item open to further research underlying at the same time that the application of the social acts might contribute to understanding the relationship between the two actors in ancient law.

Evaluation of the Eff ectiveness of the Copper (II) Complex with a New Ligand derived from Benzothiazole and Anthranilic Acid as Anticancer and Antioxidant

Four steps were used to convert 2-mercaptobenzothiazole into the new ligand (2-((2-((4-(1-((4’-(benzothiazol-2-ylamino)- [1,1’-biphenyl]-4-yl)imino) ethyl) phenyl)imino)-1,2diphenylethylidene) amino)benzoic acid (LH) type of schiff bases: The fi rst step included preparing compound (A) N-(benzothiazol-2-yl)-[1, 1’-biphenyl]-4, 4’-diamine by the reaction 2-mercaptobenzothiazole with benzidine. The second step was prepared the compound(B) 2 OXO-1, 2-diphenylethylidene) amino) benzoic -(( 2- acid by the reacting anthranilic acid with benzil As for the third step, it included the reaction of the product of the fi rest step with 4-aminoacitophenon and the formation compound (C). The fourth step was the preparation ligand (LH) by reacting compound(B) with compound (C). Fourier-transform infrared spectroscopy (FTIR), proton nuclear magnetic resonance (1H-NMR), UV-vis, melting points, molar conductivity, CHN, atomic absorption, magnetic sensitivity, and other measurements were used to determine the structure of the ligand (LH) and its complex with copper (II). The MTT method was used to evaluate the activity of the ligand (LH) and its complex with copper (II) in-vitro as an anticancer (human breast cancer (MCF7). Nd (MTT) assay was used to evaluate the ligand schiff base in-vitro anticancer activity of a Cu(II) complex against human breast cancer MCF7

Middle East in a framework within the Global geopolitical transformation

Against a background of the substantial geopolitical changes, which are, symbolized breaking with westcentrism unipolarity, fi rst of all US supremacy in world aff airs and transition to a new more balanced global order. The Arab community is in search of its own place, which is correspond with its achieved mighty level. All is said in favor of this: the real practice of the Middle East States as far as is concerned foreign policy adjusting to the new realities and in vital interest of their own, having been proamericanly imbalanced during long period. This process is not simple: during it diff erent changes are quite possible even retreats to the previous stands. Mush is dependent on the force of Washington counteraction and of course dependent on political will and fi rmness of purpose of ruling circles of the Arab States in the Middle East. But most important is that the fi rst steps are already have made, fi rst of all in energy security. All of this is in favour of Russia, vitally interest in such world architecture of the global policy which is to work for majority of States on the Earth and be more justice. As far as the current situation is concerned Arad States of the Middle East even nowadays might be cold supportive partners of our country. Beside energy background of this partnership are ethics, social and confessional values, first of all in family relations and gender sphere. The most common factor, which gets as closer is our long non conflict multi centuries history of the mutual intercourse: we never fought each other under battle ground. On the contrary there are a lot of examples of our beneficial cooperation in economy, investment activity, medicine and so on and so forth, and in recent years — common struggle against national terrorism.

LESSONS LEARNED FROM the FIRST STEPS of IMPLEMENTING VALUE-BASED ORAL HEALTH CARE: A CASE STUDY FROM the MEDICAL UNIVERSITY of VIENNA

Value-based oral healthcare (VBOHC) has two fundamental components, the assessment of patients' dental outcomes and the measurement of the costs to achieve those outcomes. The aim of this article is to describe challenges and opportunities of implementing dental patient-reported outcomes (dPROs) in clinical care at the University Clinic of Dentistry, Medical University of Vienna, in Austria, to determine lessons learned and describe next steps forward to VBOHC implementation. A case study determining lessons learned based on an implementation process to incorporate a dental patient-reported outcome measure (dPROM) in routine clinical care was conducted. The German version of the five items Oral Health Impact Profile (OHIP-5), a dPROM was selected and integrated into the general anamnesis including dental and medical history for patients aged ≥16 years. The anamnesis is paper based and is to be completed by each new patient during the registration process. Thereafter, it is uploaded to the patients' dental record via scan by the main central admission. However, it is then the treating dentist's task to transfer the data into the digital system. Data accuracy between digital and paper forms was investigated, and lessons learned regarding the first steps of implementing VBOHC were summarized based on the implementation process findings. To date, 8,147 patients were approached to fill in OHIP-5. However, only 266 patients´ OHIP- 5 files were transferred into the digital system by the dentist. To explore the accuracy between the manual transfer of data from paper forms to digital format, the data of 89 randomly selected patients was compared. Of this sample, 74 (83.1%) patient's data sets were found to be identical. Lessons learned included the importance of institutional dedication, stakeholders' engagement, dPROMs integration in follow up visits, the significance of digital solutions, and the continuous monitoring and evaluation. Integrating dPROMs in clinical settings is achievable and is the first important step to move forward with VBOHC implementation.

MYKHAILO HRUSHEVSKY IN THE CIRCLE OF SCANDINAVIAN SLAVICISTS: COOPERATION AND RECEPTION

The purpose of the research – the scientifi c novelty of the research consists in a comprehensive attempt to comprehensively analyze the multifaceted relations of M. Hrushevsky with his Scandinavian colleagues during the end of the 19th – the fi rst third of the 20th centuries. The research methodology relies on the use of an interdisciplinary approach. Based on the principles of objectivity and historicism, the research has d the structuralfunctional systematic analysis of historiographical facts and the comparative-historical method. The article has used the heuristic possibilities of periodisation, classifi cation and typology methods. The scientifi c novelty of the research consists in a comprehensive attempt to comprehensively analyze the multifaceted relations of M. Hrushevsky with his Scandinavian colleagues during the end of the 19th – the fi rst third of the 20th centuries Conclusions. In conclusion, we would like to note that Ukrainian intellectuals, as well as M. Hrushevsky, had almost the most harmonious relations with their Scandinavian colleagues. After all, unlike, say, Polish and Russian scientists, they were not divided by the complex issues of the common past and the often tragic circumstances of the revolutionary era. Thus, fruitful scientifi c relations were established between Ukrainian and Scandinavian Slavists, which had benefi cial consequences for both sides. The most important achievement of the time was the involvement of Scandinavian colleagues in the study of Ukrainian historiographicaland linguistic issues. Studying it from the standpoint of Ukrainian cultural interest, they popularized the vision of our history and spiritual heritage as unique civilizational phenomena. In addition, the relationship led to mutual scientifi c reception, which contributed to the intellectual rapprochement of East Slavic and Scandinavian cultures. As a result, thanks to M. Hrushevsky and his colleagues, an important international dialogue was started, which is still the foundation of the Ukrainian-Scandinavian partnership. Finally, we consider the attempted reconstruction of M. Hrushevsky’s cooperation with his Swedish and Norwegian colleagues to be only the fi rst step in the direction of clarifying a larger problem, which can be described as “Ukrainian and Scandinavian science in mutual infl uences and representations”.

Cultural Sensitivity in Preventive Infant Mental Health Care: An Example From the Developmental Guidance Project FIRST STEPS in Belgium.

Preventive developmental guidance programmes have been applied on a large scale for several decades now in many western countries. But how do we adapt these programs to families with very different ethnic backgrounds? How can we concretise the concept of cultural sensitivity into that context? The plea of von Klitzing, the former President of the World Association for Infant Mental Health, for further reflection on the concretization of cultural sensitivity in the context of infant mental health care is the main source of inspiration to this article. von Klitzing speaks out against the point of view in which universal children’s rights or conditions that are seen as promoting a child’s development all around the globe, are criticized as being only western conceptions and thereby culturally biassed. Following this kind of reasoning, a culturally relativistic stance on what a facilitating environment is, should be adopted. Such a discussion in terms of universalism versus relativism, though, as argues von Klitzing, is an old antagonism that cannot inspire us for the adaptation of early health care practices or developmental guidance programs that are fitting in to the needs of families and their babies that are living in multicultural contexts. Although it could be interesting to think about how certain universal principles of good-enough child care can be formulated or embodied in an intercultural variety of ways, these variable expressions of the worldwide agreed upon principles of good early mental health care will only be culture-specific translations of these universal principles. They can’t in any way be seen as an argument against the universality of these principles, as argues von Klitzing, who is describing another way of solving the problem of the cultural relativist criticism on the universality of principles of good infant mental health: culture sensitive infant mental health services. But, to make the principle of cultural sensitivity work in practice, we need to fill it with content. This article is mainly on what it concretely means to work in a culture sensitive way in our preventive practice within the FIRST STEPS program, a Belgian project for immigrant and exiled mothers and their children from zero to three.

Fostering Emotional Availability in Mother-Child-Dyads With an Immigrant Background: A Randomized-Controlled-Trial on the Effects of the Early Prevention Program First Steps.

BackgroundIn many Western countries like Germany, the social integration of children with an immigrant background has become an urgent social tasks. The probability of them living in high-risk environments and being disadvantaged regarding health and education-related variables is still relatively higher. Yet, promoting language acquisition is not the only relevant factor for their social integration, but also the support of earlier developmental processes associated with adequate early parenting in their first months of life. The Emotional Availability Scales (EAS) measure the quality of caregiver-child-interactions as an indicator of the quality of their relationship and thus of such early parenting, focusing on mutual and emotional aspects of their interaction.MethodThis pilot study examined in a randomized controlled trial the effects of the prevention project First Steps regarding the hypothesis that the Emotional Availability (EA) improved to a greater extent in “difficult-to-reach” immigrant mother-child dyads in a psychoanalytically oriented early intervention (A, FIRST STEPS) compared to a usual care intervention (B) offered by paraprofessionals with an immigrant background. A sample of N = 118 immigrant women in Germany from 37 different countries and their children was compared with regard to the parental EA-dimensions sensitivity, structuring, non-intrusiveness and non-hostility and the child dimensions responsiveness to and involvement of the caregiver in the pre-post RCT design.Results and ConclusionDifferent from what was expected, repeated ANOVAs revealed no significant pre-post group differences for the parental dimensions. For the child dimensions the effect of time of measurement was highly significant, which can be interpreted as mostly natural developmental effects. Still, on the level of simple main effects for each intervention, only in the FIRST STEPS groups child responsiveness significantly improved. When controlled for confounding variables, a significant interaction effect for maternal sensitivity in favor of the FIRST STEPS intervention was found. The systematic group differences indicate that the more extensive and professional intervention, focusing on the individual needs of the participants, is more suitable to support the quality of the mother-child-relationship amongst immigrant mother-child dyads than usual care. The results are discussed taking into account the context of the maternal migration process and potential maternal traumatization.Clinical Trial Registration[https://clinicaltrials.gov], identifier [DRKS00004632].

Early and late outcomes of combined and staged surgeries in patients with concomitant atherosclerotic lesions of the coronary and brachiocephalic arteries

Objective. To study the early and late outcomes of surgical treatment of patients with concomitant atherosclerotic lesions of coronary and brachiocephalic arteries while choosing a treatment strategy based on the developed diff erentiated approach algorithm.Material and Methods. The study comprised 243 patients with combined atherosclerotic lesions of the coronary and carotid arteries examined during the period from 01.07.2014 to 01.01.2021. Patients underwent revascularization surgeries based on the algorithm for choosing the volume and stages of surgical intervention, which was previously developed in the Federal Center for Cardiovascular Surgery named after S.G. Sukhanov. A single-stage combined surgery of coronary artery bypass grafting (CABG) and carotid endarterectomy (CEE) was performed in 104 patients (42.8%); 139 patients (57.2%) received staged revascularization including 102 patients (73.4%) who received CABG as the fi rst step and 37 patients (26.6%) who received CEE as the fi rst step of surgery. The endpoints for both early and late results were death from all causes, stroke, transient ischemic attack (TIA), acute myocardial infarction (AMI), and combined endpoint that included all of the above. Average follow-up time was 41.1 ± 21.8 months.Results. No fatal outcomes were in any group during the early postoperative period. At the hospital stage, there were 5 cases (2.1%) of stroke, 1 case (0.4%) of TIA, and 3 cases (1.2%) of acute MI. Long-term results were evaluated in 225 patients (92.3%). The overall survival rate was 93.8%. There were 5 cases (2.4%) of MI, 11 cases (4.9%) of stroke, and 1 case (1.0%) of TIA. No signifi cant diff erences were observed in immediate and long-term endpoints between the groups of staged and combined interventions as compared to immediate (AMI: p = 0.680; TIA: p = 0.500; acute cerebrovascular events: p = 0.567; combined: p = 0.940) and long-term results (deaths: 0.860; AMI: p = 0.906; TIA: p = 0.528; acute cerebrovascular events: p = 0.378; combined: p = 0.669).Conclusion. Based on successful experience with treating the concomitant atherosclerotic lesions of the coronary and brachiocephalic arteries, the proposed algorithm allowed to perform safe procedures in both arterial basins and to achieve satisfactory results in in-hospital and long-term periods in the Federal Center for Cardiovascular Surgery named after S.G. Sukhanov (Perm).

ПРИМЕНЕНИЕ ФИЗИЧЕСКИХ МЕТОДОВ ОБРАБОТКИ ДЛЯ ОБЕЗЗАРАЖИВАНИЯ ПОЧВЫ

To reduce the chemical load on the soil, plants and fruits, it is important to use alternative methods of soil and plant cultivation, which include irradiation with non-ionizing radiation. To establish the range of technological parameters and operating modes of the developed machines for tillage with non-ionizing radiation, the authors developed a technique and an experimental installation capable of providing infrared and ultraviolet radiation. To study the soil intended for use as protected greenhouse ground, standard methods for determining the physical, microbiological, and qualitative properties of the soil were applied. In the course of research, fi ve modes of soil cultivation were implemented: infrared radiation for three hours with and without the mechanical mixing of the soil; ultraviolet radiation for one hour; exposure for one hour to one infrared and one ultraviolet emitters with the mechanical mixing of the soil; processing for one hour with three infrared emitters and one ultraviolet with the mechanical mixing of the soil. Microbiological studies of the soil treated at the experimental unit were carried out within one hour after the selection of the material according to the presented method. It has been experimentally established that, depending on the modes of soil cultivation, the number of microorganisms decreases by 14…98%. The combination of infrared and ultraviolet radiation with a simultaneous threefold increase in the thermal power of radiation for one hour helps to reduce the number of microorganisms up to 98%. The decrease in the content of microorganisms in the soil due to non-ionizing radiation is the fi rst step to establish the design and technological parameters and operating modes of the developed machines used for tillage in protected greenhouse conditions.

The system of mental health care and psychoeducation as a form of counteracting the psychological stress related to the profession of a police officer

The main task of this publication is to present the psychological care and psycho-education system in the Polish Police. The aim of the mental health care system is to provide help and psychological support to police officers and police employees, and to relieve their tensions caused by negative external factors, called stressors. This help and support is necessary due to the fact that the profession of a police offi cer is considered a ‘high-risk’ job, one of the most stressful in the world and resulting in many diffi cult experiences, burdens and problems in the psychological sphere. Persistent mental disorders are often the cause of somatic diseases. Police offi cers on duty are confronted with many diffi cult situations, stressful events and they are exposed to numerous challenging and risky situations, which in consequence are the cause of the professional stress in offi cers. This, in turn, leads to psychological tensions and various ailments, somatic and mental illnesses. The article presents a number of factors that occur in the external service that are sources of work stress. Reference has also been made to the fact that a very common source of this stress is an inappropriate management process within the Police. The reason for numerous tensions are often confl icts between superiors and subordinates. However, the key to the publication is the description of the functioning of the mentioned system. The history of its creation is the fi rst step in its presentation here. The organisational structure of the system, the relatively full range of tasks assigned to offi cers, the organisation of their work, as well as the catalogue of events requiring their intervention and the forms of psychological assistance have all been presented. The paper also focuses on the psycho-education system — the one carried out directly by police psychologists, and this performed in a broader sense, called the psychological education which is carried out by the lecturers and management of the Police. As part of this psychological education process, future and current managers are equipped with a wide range of psychological knowledge. It is necessary for managers at various levels to be the fi rst to provide the necessary psychological support and ‘fi rst aid’ to their subordinates in cases of occupational stress and psychological load caused by service conditions. The conclusion shows that a well-functioning system of psychological care and psycho-education has a positive impact on the mental condition of police offi cers and employees, on their level of satisfaction with police service, and indirectly on the quality and effectiveness of the police work.

The genetics of congenital heart disease: The role of advanced genomic approaches

As the leading non-infectious cause of paediatric morbidity and mortality worldwide, congenital heart disease (CHD) is a significant social and healthcare burden, especially in low- and lower-middle-income countries, including South Africa. The aetiology of CHD is poorly understood, though heritable genetic factors have been shown to contribute to the risk of CHD in individuals of European ancestry. In this review, we highlight the impact that advanced genomic approaches have had on the understanding of the role of genetics in CHD. We also summarise current knowledge of the genetics of CHD in Africa, and the challenges and opportunities for conducting genomic research in these populations. Chromosomal microarrays and next-generation sequencing platforms allow for high-throughput screening of patients for genetic mutations and show great potential for the identification of genetic causes of CHD. Advancing our understanding of the genetic architecture and risk factors associated with CHD in patients of African descent is the fi rst step towards improving CHD diagnosis and management. Therefore, exploring the genetics of CHD has the potential to improve the quality of life for children born with CHD in Africa, by enabling clinicians to identify familial inheritance patterns and predict recurrent risks and prognostic outcomes pre- and post-surgical intervention.

International legal and national regulation of digital currency: the experience of China and the EU

The article discusses the issue of legal regulation of the digital currency of central banks. The experience of international organizations, as well as the fi rst steps in this direction from China, represented by the People’s Bank of China and the European Union, represented by the European Central Bank, are examined.

O12 - Topic: AS06-Prognosis/AS06a-Prognostic factors of outcome and risk assessment: CLINICAL OUTCOMES AND MOLECULAR PROFILE OF OLIGOMONOCYTIC CHRONIC MYELOMONOCYTIC LEUKEMIA SUPPORT ITS CONSIDERATION AS THE FIRST STEP IN THE PROLIFERATIVE CONTINUUM OF CHRONIC MYELOMONOCYTIC LEUKEMIA

O12 - Topic: AS06-Prognosis/AS06a-Prognostic factors of outcome and risk assessment: CLINICAL OUTCOMES AND MOLECULAR PROFILE OF OLIGOMONOCYTIC CHRONIC MYELOMONOCYTIC LEUKEMIA SUPPORT ITS CONSIDERATION AS THE FIRST STEP IN THE PROLIFERATIVE CONTINUUM OF CHRONIC MYELOMONOCYTIC LEUKEMIA

FIRST STEP Next 프로그램이 유아의 문제행동 및 사회정서역량과 교사의 사회정서교수역량에 미치는 효과

본 연구는 ‘FIRST STEP Next’(FSN) 프로그램의 유아 문제행동 감소, 유아 사회정서역량 및 교사의 사회정서교수역량에 대한 효과 파악을 목적으로 한다. 대상은 만 5-6세의 심각한 문제행동이 있는 학령전기 유아 5명과 그 유아가 속한 학급 유아 43명, 그리고 그들의 담임교사 5명이다. 유아가 갖는 문제행동에 대한 단일대상분석법, 학급 전체 유아들의 사회정서역량 변화에 대한 실험통제집단 사전사후분석, 교사의 사회정서교수역량 변화에 대한 사전 사후 그래프 분석을 하였다. 연구결과 첫째, 프로그램은 연구 참여 유아의 문제행동 감소에 효과가 있는 것으로 나타났고, 이는 중재 철회 후에도 그 효과가 유지되었다. 둘째, 프로그램에 참여한 학급 유아들의 사회정서역량증진에 효과가 있는 것으로 나타났다. 마지막으로, 프로그램은 참여교사의 사회정서교수역량을 촉진하는데 효과가 있었다. 본 연구결과를 기반으로 유아를 위한 문제행동 중재 방향 및 사회정서역량 향상을 위한 적용방안을 논의하였다. 추가적으로 연구의 제한점과 후속연구를 제시하였다.

Protocol for a randomized pilot study (FIRST STEPS): implementation of the Incredible Years-ASLD\xae program in Spanish children with autism and preterm children with communication and/or socialization difficulties

Having access to parenting interventions in the early years is key to improve the developmental outcomes of children with neurodevelopmental problems. The Incredible Years® (IY) Parent Program is a group intervention that has demonstrated efficacy in terms of reducing stress in parents, as well as improving behavioral, emotional, and social outcomes in children. The program has been recently adapted for families of children with autism or language delays (IY-ASLD®). This intervention has not yet been implemented in the Spanish Public Health System, where there is a scarcity of evidence-based interventions being offered to families with young children presenting neurodevelopmental problems. The main aims of this study are to determine the feasibility of implementing the IY-ASLD® program within Spanish Child Mental Health Services and to examine parents’ acceptability and satisfaction with the intervention. As a secondary objective, we aim to evaluate its preliminary effectiveness in terms of reducing parental stress and behavioral difficulties in their children. The FIRST STEPS study is a multicenter, pilot randomized controlled trial comparing the IY-ASLD® program with a treatment-as-usual (TAU) condition. Approximately 70 families of children with autism spectrum disorder (ASD) and preterm children with communication and/or socialization difficulties (aged 2–5 years) will be recruited. Families will be assessed prior to randomization and after the intervention. Due to the COVID-19 pandemic, the intervention will consist of 22 weekly online sessions (approximately 6 months).The FIRST STEPS pilot trial will demonstrate the feasibility and acceptability of reliably implementing the IY-ASLD® program within the Spanish Public Health System. The results of this study could represent the first step to inform policymakers in Spain when designing evidence-based healthcare pathways for families of children presenting ASD symptoms or neurodevelopmental difficulties at early stages.Trial registrationClinicalTrials.gov NCT04358484. Registered on 04 April 2020

Rana dijagnoza neuronske* ceroidne lipofuscinoze tip 2 - mit ili stvarnost?

Neuronal ceroid lipofuscinosis type 2 (CLN2) is the most common childhood progressive neurodegenerative disease. The main features of CLN2 disease are language delay that precedes epileptic seizures, motor disorders, progressive deterioration of vision and cognition with death in early adolescence. The average time from the onset of fi rst symptoms to diagnosis is about two years. Delay in diagnosis postpones therapeutic approach involving multidisciplinary health care and effi cacious enzyme replacement therapy with cerliponase alpha (Brineura®). The availability of enzyme replacement therapy classifi es CLN2 disease in the group of treatable neurodegenerative diseases. Recognition of early symptoms is crucial to direct clinicians to appropriate examinations or seek additional expert opinion. Timely diagnosis is imperative because it allows treatment at an early stage of the disease. In all children with the fi rst unprovoked epileptic seizures between the ages of two and four, and particularly in those children with a history of language delay and motor impairment, the fi rst step is to measure the TTP1 enzyme activity by dried blood spot testing, which is available in our setting. Genetic testing and a fi nding of pathogenic mutations in the CLN2 gene confi rm the diagnosis. Raising awareness of CLN2 disease as a treatable and potentially curable disease and following the proposed guidelines will certainly contribute to early diagnosis of CLN2 disease to become our reality rather than a myth. Timely and accurate diagnosis is the fi rst step in improving the overall care of children with CLN2 disease and their parents.