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Učinak toksina botulinuma tipa – A na nemotoričke simptome u bolesnika s fokalnom distonijom
Background: Traditionally, isolated adult-onset cervical dystonia (IAOCD) is considered as a ‘pure’ motor disorder. However, non-motor symptoms (NMS) are increasingly recognized as a part of IOACD phenotype. Cognitive functions in IAOCD were rarely investigated and poorly understood making this issue still a matter of debate. The aim of this prospective study was to investigate, for the first time, the effect of acute and long-term therapeutic applications (more than one year) of botulinum toxin type-A (BoNT-A) on NMS and cognitive functions in IAOCD patients previously not treated with BoNT-A. Design/Methods: The study included 51 IAOCD patients (29 females and 22 males) and 90 (50 female i 40 males) age, gender and education matched healthy controls. Questionnaires used in this study included: Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Beck Depression Inventory – Second Edition (BDI-II), Beck Anxiety Inventory (BAI), Starkstein Apathy Scale (AS), Pitsburgh Sleep Quality Index (PSQI), Fatigue Severity Scale (FSS), Short Form-36 Health Survey (SF-36). Cognitive functions were assessed using Cogtest®, a computerized neurocognitive battery set of 5 tests: Auditory Number Sequencing (ANS), Spatial Working Memory (SWM), Strategic Target Detection (STD), Continuous Performance Test – Flanker version (Flanker CPT) and Tower of London (ToL). All of the above mentioned investigations were performed prior to initial BoNT-A application (first test). The follow-up was performed after 4 month (second test) and 12 to 16 months after initial BoNT-A application (third test). During the follow-up period the patients received 3 BoNT-A applications.
Results: On initial investigation 46 (90,2%) IAOCD patients had at least one psychiatric disorder in comparison to 36 (40%) healthy controls. Mean BDI-II score, BAI score and AS score were significantly higher in IAOCD patients than in healthy controls (12,59 ± 6,28 vs. 6,11 ± 4,26, p0,05). However, IAOCD patients had worse performance in STD, ToL, and Flanker CPT test (p<0,05). IAOCD patients had significantly reduced quality of life (QoL) determined by SF-36 questionnaire. During follow-up period, we found no significant difference in total BDI-II, BAI, AS, PSQI and FSS score between all three tests. Still, no difference in total AS score on third test was observed between IAOCD and control group, and improvement in total PSQI score was observed in second and third test when compared to initial one. Significant pain amelioration, unrelated to improvement of motor symptoms, was observed during follow-up period. Long-term BoNT-A applications had no effect on cognitive performance as measured by ANS, SWM, Flanker CPT and ToL test. Significant improvement was detected in variables of strategic efficiency and total perseverative errors of STD test, designed for evaluation of complex attention and processing speed. Improvement of QoL was observed in two SF-36 domains: bodily pain and general health perception.
Conclusion: Spectrum of IAOCD symptoms includes various NMS such as pain, psychiatric comorbidities, impaired sleep quality, pathological fatigue, and cognitive dysfunction, especially in the domains of attention and executive functioning, thus implicating shared neurobiology of motor symptoms, NMS and cognitive dysfunction. Except for major analgesic effect and mild positive effect on sleep quality and apathy, long-term BoNT-A treatment had no statistically significant impact on other NMS. In addition, long-term BoNT-A application showed modest improvement of attention without any negative implication on other cognitive domains. Finally, we can say that in the dosages applied in this study (150-200U per application), BoNT-A is significantly effective in reducing pain and motor symptoms, probably minimally effective in improvement of sleep quality and apathy, ineffective in reducing anxiety, depression and fatigue. We did not find any negative influence of BoNT-A on cognitive functions with the applied objectivization methods, moreover, a slight improvement in complex attention and processing speed was found
Imunizacija djece s kongenitalnim srčanim grješkama – klinički i kritički osvrt [Immunisation of children with congenital heart defects – clinical and critical overview]
Numeruous scientific articles indicate the benefit of immunisation when dealing with children with congenital heart defects. High possibility of gaining infections can be related to compromised cardiopulmonary status, changed pressures in pulmonary circulation (including pulmonary hypertension) as well as ventilation-perfusion mismatch. Many children with heart anomalies within heterotaxy syndrome have functional hyposplenism with high susceptibilty to infections. Children with chromosomopathies and many other syndromes have a high frequency of immunodeficiency states of various intensity, alongside hemodynamically significant, often complex heart defects and numerous anomalies of other organs. Cardiopulmonary bypass and ECMO device are inevitably used for operative corrections and palliative procedures, however, main issue is their tendency to decrease the circulating antibodies that have occurred during active immunisation, thereby compromising the acquired immune system. Protecting such patients by systematic immunisation of children and their family members should be the main interest of scientific community. This paper gives a systematic overview of the above mentioned controversy
Stenoza spinalnog kanala slabinske kralježnice – od epidemiologije do rehabilitacije [Lumbar spinal stenosis – from epidemiology to rehabilitation]
Lumbar spinal stenosis (LSS) is becoming an increasingly prevalent condition due to trends of aging population. That is because, although etiologically heterogenous, it is most often the consequence of degenerative changes of the spine. There is no generally accepted classification of LSS. To make the diagnosis of LSS it is crucial to interrelate history, clinical examination and imaging alongside with neurophysiological findings. The treatment of patients with LSS may be conservative (pharmacological and non-pharmacological) and surgical, and in this respect in the latter minimally invasive techniques are preferred lately. In this paper we comprehensively present a state-of-the-art view on LSS
Ponavljane luksacije peronealnih tetiva: prikaz uzastopne serije bolesnika i pregled literature [Recurrent peroneal tendon dislocation: a consecutive case series and review of the literature]
The aim of this paper was to give a complete review of the current literature on surgical treatment of peroneal tendon dislocation and to present the results of surgical treatment in our consecutive case series of 7 patients in order to create a treatment strategy for optimal functional outcomes. The results of current research on surgical treatment of peroneal tendon dislocation as well as the outcome of treatment in our patients indicate that the repair of the superior peroneal retinaculum (the Singapore operation) is highly effective with excellent func-tional results after long term follow up. The great advantage of this technique is the possibility of combining this method with other procedures, such as retromalleolar groove deepening in the presence of a shallow retro-malleolar groove. Despite the success of open surgery in the treatment of peroneal tendon dislocation due to the rapid development of tendoscopy of peroneal tendons as a minimally invasive surgical technique, it is be-lieved that treatment of recurrent dislocation of peroneal tendons will be performed completely tendoscopical-ly in the near future
Nastava kemije i biokemije na Medicinskom fakultetu u Zagrebu u doba Frana Bubanovića [Chemistry and biochemistry education at School of medicine in Zagreb at the time of Fran Bubanović]
Exactly a hundred years ago the Department of Chemistry and Biochemistry was established within the newly founded School of Medicine in Zagreb. Its founder, first Department Head and Professor was Fran Bubanović. Education of future physicians in medical chemistry and biochemistry is continuously performed since 1918. This paper aims to provide an insight in teaching activities at the Department throughout almost forty years of Bubanović’s university career. The contribution of professor Bubanović is unmeasurable not only in the early years of the Department teaching activity, but also to the organisation and management of courses and the Department throughout many years, as well as to university literature in chemistry.
We believe that professor Bubanović has made a profound impact on many generations of students with his dedicated work, and us, his successors, he left forever indebted
Preporuke za prevenciju i liječenje pothranjenosti u bolesnika s kroničnom opstruktivnom plućnom bolesti [Recommendations for prevention and treatment of malnutrition in patients with chronic obstructive pulmonary disease]
Chronic obstructive pulmonary disease (COPD) represents a significant public health problem with estimated prevalence of 10-15% in adult population in Croatia. COPD is a chronic inflammatory disease characterized by significant systemic disorders that can lead to poorer quality of life and increased morbidity and mortality. Protein- -energy malnutrition can be found in 25-40% of patients with COPD, with higher prevalence as disease progresses. Thus, COPD is defined as a multicomponent disease and treatment should be based on correction of all systemic disorders. Cachexia and muscle wasting is a frequent complication in COPD patients, an independent predictor of morbidity and mortality, and can accelerate frequency and severity of exacerbation. The aim of the Croatian Thoracic Society with these recommendations is to emphasize the importance of timely determination of the nutritional status of COPD patients by using a validated tool and defining a specific phenotype of patients. Adequate nutritional care needs to be provided in malnourished patients and those at risk of developing malnutrition
Obrazovanje medicinskih sestara u Republici Hrvatskoj [Nursing education in Croatia]
European Union (EU) has defi ned the process and norms of nursing education. The aim of this project is to describe the nursing education in Croatia and compare it with education of nurses in the United Kingdom (UK) as a cradle of modern nursing, and in Slovenia as a transition country. As a source of data we used Directive 2005/36/EC and Directive 2013/55/EU, and also laws, regulations and documents from the mentioned countries which are related to nursing education. Nursing education in Croatia is carried out through high school education and also at the university level. Slovenian education of medical nurses starts after twelve years of general education and it is carried out at the level of college education. In the UK education is being carried out at the college level after twelve years of general education, and the possibility of acceptance into nursing involves professional qualifications at a similar academic level and courses of higher education. Experience in nursing education in the UK and Slovenia can be useful in the improving of nursing education in Croatia
Hypermethylation of Secreted Frizzled Related Protein 1 gene promoter in different astrocytoma grades
Aim: To identify the involvement of Secreted Frizzled Related Protein 1 (SFRP1) promoter hypermethylation in different malignancy grades of astrocytoma and assess its association with beta-catenin, lymphoid-enhancer factor 1, and T-cell factor 1. Methods: Twenty-six astrocytoma samples were collected from 2008-2015. Promoter hypermethylation was evaluated by methylation-specific polymerase-chain-reaction and protein expression by immunohistochemistry and stereological analysis. The staining intensity was scored by comparing immunoreactivity with normal tissue and by using 10% and 50% cut-offs. Results: SFRP1 promoter methylation was found in 32% of astrocytomas. The number of hypermethylated samples increased in higher astrocytoma grades and was the highest in glioblastoma (P = 0.042 compared to other astrocytoma grades). There was 45.8% of samples with the lack of or weak expression of SFRP1 protein and 29.2% with strong expression. Samples with methylated promoter expressed significantly less SFRP1 than samples with unmethylated promoter (P = 0.031). Beta-catenin expression levels were elevated. Yet, glioblastomas with unmethylated SFRP1 promoter had significantly less beta-catenin (P = 0.033). Strong expression of lymphoid-enhancer factor 1 was associated to higher astrocytoma grades (P = 0.006).
Conclusion: SFRP1 gene was epigenetically silenced in glioblastomas when compared to low astrocytoma grades, which may suggest that the lack of its protein is involved in astrocytoma progression
Transplantation of neural stem cells in the mouse model of ischemic brain stroke and expression of genes involved in programmed cell death
Aim: To analyze how neural stem cells (NSC) transplantation in the stroke-affected mouse brain influences the expression of genes involved in apoptosis-inducing factor (AIF)-mediated cell death – apoptosis inducing factor mitochondria associated 1 (Aifm1), ring finger protein 146 (Rnf146, Iduna), and cyclophilin A (CypA); necroptosis –receptor interaction protein kinase 1 (Ripk1), Ripk3, and mixed-lineage kinase domain-like protein (Mlkl); and apoptosis – Caspase 3 (Casp3) and Casp8. Methods: Four groups of animals were used to obtain mRNA for quantitative reverse transcription polymerase chain reaction analysis: healthy animals (n = 3), animals with stroke (n = 4), animals with stroke treated by stem cell transplantation (n = 7), and animals with stroke treated by proliferation-supporting medium (n = 5). Ischemic brain injury was induced by transient left middle cerebral artery occlusion. Statistical analysis was performed using one-way analysis of variance with post-hoc Tukey test. Results: NSC transplantation in the stroke-affected mouse brain significantly increased the expression of Iduna (P < 0.05), a gene-encoding protein with well-known protective effects on hypoxic damage, and significantly down-regulated the expression of damage-supportive genes, Casp3 (P < .01) and Aifm1 (P < 0.001). We were able to distinguish between the effect produced by stem cell transplantation (Iduna, Aifm1, Ripk3, Mlkl) and the effect produced by supporting the tissue with proliferation-supporting medium (Ripk1, Casp8). Conclusion: Beside revealing some clearly positive effects of stem cells transplantation on the stroke-affected brain, our results suggest that the tissue response triggered by stem cells points toward the desired, regeneration-supporting levels of expression of a certain gene at a certain time point
International bullous diseases group: consensus on diagnostic criteria for epidermolysis bullosa acquisita
BACKGROUND:
Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests, making an international consensus on the diagnosis of EBA essential. -----
OBJECTIVES:
To obtain an international consensus on the clinical and diagnostic criteria for EBA. -----
METHODS:
The International Bullous Diseases Group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. For the final voting exercise, 22 experts from 14 different countries voted on 50 different items. When > 30% disagreed with a proposal, a discussion was held and re-voting carried out. -----
RESULTS:
In total, 48 of 50 proposals achieved consensus after discussion. This included nine diagnostic criteria, which are summarized in a flow chart. The IBDG was unable to determine one procedure that would be applicable worldwide. A limitation of the study is that differential diagnosis of bullous systemic lupus erythematosus has not been addressed. -----
CONCLUSIONS:
This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting