Cardiac findings in pediatric patients with spinal muscular atrophy types 2 and 3.

BACKGROUND: It is unclear whether the heart is affected in pediatric patients with milder forms of spinal muscular atrophy (SMA). Therefore, we aimed to determine the presence of any cardiac abnormalities in these patients. METHODS: We conducted a cross-sectional study of children and adolescents with SMA types 2 and 3 between July 2018 and July 2019. All patients underwent a comprehensive cardiac evaluation, including history-taking, physical examination, electrocardiography, echocardiography, measurement of cardiac biomarkers (cardiac troponin T [cTnT] and N-terminal pro-brain natriuretic peptide [NT-proBNP]), and 24-hour Holter monitoring. RESULTS: In total, 42 patients were enrolled (27 and 15 with SMA type 2 and 3, respectively). No patient had structural heart disease, except for one with mitral valve prolapse. None had signs of ventricular dysfunction on echocardiography. Both cTnT and NT-proBNP levels were normal in all patients. Electrocardiography showed sinus tachycardia in seven patients (16.7%), and prolonged P-R interval in one (2.4%). Holter monitoring detected benign ventricular arrhythmias in two patients (4.8%), and rare supraventricular premature beats in one. The mean 24-hour heart rate was elevated in six patients (14.3%), whereas both the minimum 24-hour heart rate and the maximum R-R interval were increased in 23 (54.8%). DISCUSSION: The prevalence of cardiac disease in pediatric patients with SMA types 2 and 3 is low; however, these patients may have increased resting heart rates. A complete cardiac history and physical examination are a useful screen. Additional cardiac investigations may be performed as needed.

Analysis of PMP22 duplication and deletion using a panel of six dinucleotide tandem repeats.

BACKGROUND: Charcot-Marie-Tooth type 1A (CMT1A) is the most common type of hereditary motor and sensory neuropathies (HMSN), caused by the duplication of the 17p11.2 region that includes the PMP22 gene. Reciprocal deletion of the same region is the main cause of hereditary neuropathy with liability to pressure palsies (HNPP). CMT1A accounts for approximately 50% of HMSN patients. Diagnostics of CMT1A and HNPP are based on quantitative analysis of the affected region or RFLP detection of breakage points. The aim of this study was to improve the sensitivity and efficiency of CMT1A and HNPP genetic diagnostics by introducing analysis of six STR markers (D17S261-D17S122-D17S839-D17S1358-D17S955-D17S921) spanning the duplicated region. METHODS: Forty-six CMT1A and seven HNPP patients, all genetically diagnosed by RFLP analysis, were tested for duplication or deletion using six STR markers. RESULTS: In all CMT1A and HNPP patients, microsatellite analysis comprising six STR markers confirmed the existence of a duplication or deletion. In 89% (41/46) CMT1A patients the confirmation was based on detecting three alleles on at least one locus. In the remaining 11% (5) CMT1A patients, duplication was also confirmed based on two peaks with clear dosage difference for at least two different markers. All HNPP patients (7/7) displayed only one allele for each analyzed locus. CONCLUSIONS: Microsatellite analysis using six selected STR loci showed a high level of sensitivity and specificity for genetic diagnostics of CMT1A and HNPP. The results here strongly suggest STR marker analysis as a method of choice in PMP22 duplication/deletion testing.

Organizational commitment and job satisfaction among nurses in Serbia: a factor analysis.

BACKGROUND: One of the basic prerequisites of efficient organizational management in health institutions is certainly monitoring and measuring satisfaction of employees and their commitment to the health institution in which they work. The aim of this article was to identify and test factors that may have a predictive effect on job satisfaction and organizational commitment. METHODS: We conducted a cross-sectional study that included 1,337 nurses from Serbia. Data were analyzed by using exploratory factor analysis, multivariate regressions, and descriptive statistics. RESULTS: The study identified three major factors of organizational commitment: affective commitment, disloyalty, and continuance commitment. The most important predictors of these factors were positive professional identification, extrinsic job satisfaction, and intrinsic job satisfaction (p < .0001). Predictors significantly affecting both job satisfaction and organizational commitment were identified as well; the most important of which was positive professional identification (p < .0001). CONCLUSIONS: This study identified the main factors affecting job satisfaction and organizational commitment of nurses, which formed a good basis for the creation of organizational management policy and human resource management policy in health institutions in Serbia.

Lipid peroxidative damage on Cisplatin exposure and alterations in antioxidant defense system in rat kidneys: a possible protective effect of selenium.

Cisplatin (Cis-diamminedichloroplatinum II, CP) is an important chemotherapeutic agent, useful in the treatment of several cancers, but with several side effects such as nephrotoxicity. The present study investigated the possible protective effect of selenium (Se) against CP-induced oxidative stress in the rat kidneys. Male Wistar albino rats were injected with a single dose of cisplatin (7 mg CP/kg b.m., i.p.) and selenium (6 mg Se/kg b.m, as Na(2)SeO(3), i.p.), alone or in combination. The obtained results showed that CP increased lipid peroxidation (LPO) and decreased reduced glutathione (GSH) concentrations, suggesting the CP-induced oxidative stress, while Se treatment reversed this change to control values. Acute intoxication of rats with CP was followed by statistically significant decreased activity of antioxidant defense enzymes: superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GSH-Px), glutathione reductase (GR) and glutathione-S-transferase (GST). Treatment with Se reversed CP-induced alterations of antioxidant defense enzyme activities and significantly prevented the CP-induced kidney damage.

Towards Identifying Optimal Biased Feedback for Various User States and Traits in Motor Imagery BCI.

OBJECTIVE: Neural self-regulation is necessary for achieving control over brain-computer interfaces (BCIs). This can be an arduous learning process especially for motor imagery BCI. Various training methods were proposed to assist users in accomplishing BCI control and increase performance. Notably the use of biased feedback, i.e. non-realistic representation of performance. Benefits of biased feedback on performance and learning vary between users (e.g. depending on their initial level of BCI control) and remain speculative. To disentangle the speculations, we investigate what personality type, initial state and calibration performance (CP) could benefit from a biased feedback. METHODS: We conduct an experiment (n = 30 for 2 sessions). The feedback provided to each group (n = 10) is either positively, negatively or not biased. RESULTS: Statistical analyses suggest that interactions between bias and: 1) workload, 2) anxiety, and 3) self-control significantly affect online performance. For instance, low initial workload paired with negative bias is associated to higher peak performances (86%) than without any bias (69%). High anxiety relates negatively to performance no matter the bias (60%), while low anxiety matches best with negative bias (76%). For low CP, learning rate (LR) increases with negative bias only short term (LR = 2%) as during the second session it severely drops (LR = -1%). CONCLUSION: We unveil many interactions between said human factors and bias. Additionally, we use prediction models to confirm and reveal even more interactions. SIGNIFICANCE: This paper is a first step towards identifying optimal biased feedback for a personality type, state, and CP in order to maximize BCI performance and learning.

Child Neurology: Bartonella henselae Neuroretinitis in 2 Patients.

Neuroretinitis due to Bartonella henselae infection is a rare cause of vision loss in children. Two pediatric cases of acute unilateral vision loss accompanied by edema of the optic nerve on fundoscopic examination are presented. Severe causes of vision loss were excluded. During the course of the disease, macular stellate exudates emerged on control fundoscopic examinations, and diagnosis of neuroretinitis was made. A causative agent was confirmed by serologic examination, as high titers of IgM and IgG antibodies to Bartonella henselae were detected. Both patients significantly recovered after oral antibiotic treatment.

Relationships between Milk and Blood Biochemical Parameters and Metabolic Status in Dairy Cows during Lactation.

This study aimed to determine blood and milk metabolic parameters and their correlations for the purpose of evaluating metabolic status in dairy cows. Blood and milk samples were collected from 100 Holstein dairy cows during morning milking. The cows were allocated to four groups according to the production period, including cows in early (n = 18), full (n = 26), mid (n = 25) and late (n = 31) lactation. The value of non-esterified fatty acids (NEFA), ß-hydroxybutyrate (BHB), glucose, triglycerides (TG), total cholesterol (TChol), total protein (TP), albumin, globulin, urea, total bilirubin (TBil), aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), and lactate dexydrogenase (LDH) in the blood were determined. The following milk parameters were measured: fat, protein, lactose, urea, AST, ALT, ALP, GGT, LDH and BHB. Blood serum NEFA, BHB, TBil, AST, ALT, ALP and LDH were higher in early lactation cows, whereas glucose, TP, globulin and urea levels were significantly lower in early lactation cows. Milk fat and lactose levels were lower in early lactation cows, whereas milk protein and the activities of AST, ALT, ALP and LDH in milk were highly greater in early lactation cows. Milk fat was positively correlated with glucose, TP and TG, and negatively correlated with BHB, NEFA, TBil, ALT, LDH and ALP levels in the blood. Enzyme activities in milk were positively correlated with those in blood and with blood NEFA, BHB and TBil levels, and negatively correlated with blood glucose, TChol and TG. A significant positive correlation existed between blood and milk BHB values. Many correlations showed the same slope during all lactation periods. In conclusion, similar changes in blood and milk metabolite concentration during lactation and milk to blood correlations confirm that milk has great potential in predicting of blood metabolites and metabolic status of cows.

LTBP4, SPP1, and CD40 Variants: Genetic Modifiers of Duchenne Muscular Dystrophy Analyzed in Serbian Patients.

BACKGROUND: Clinical course variability in Duchenne muscular dystrophy (DMD) is partially explained by the mutation location in the DMD gene and variants in modifier genes. We assessed the effect of the SPP1, CD40, and LTBP4 genes and DMD mutation location on loss of ambulation (LoA). METHODS: SNPs in SPP1-rs28357094, LTBP4-rs2303729, rs1131620, rs1051303, rs10880, and CD40-rs1883832 were genotyped, and their effect was assessed by survival and hierarchical cluster analysis. RESULTS: Patients on glucocorticoid corticosteroid (GC) therapy experienced LoA one year later (p = 0.04). The modifying effect of SPP1 and CD40 variants, as well as LTBP4 haplotypes, was not observed using a log-rank test and multivariant Cox regression analysis. Cluster analysis revealed two subgroups with statistical trends in differences in age at LoA. Almost all patients in the cluster with later LoA had the protective IAAM LTBP4 haplotype and statistically significantly fewer CD40 genotypes with harmful T allele and "distal" DMD mutations. CONCLUSIONS: The modifying effect of SPP1, CD40, and LTBP4 was not replicated in Serbian patients, although our cohort was comparable in terms of its DMD mutation type distribution, SNP allele frequencies, and GC-positive effect with other European cohorts. Cluster analysis may be able to identify patient subgroups carrying a combination of the genetic variants that modify LoA.

Standardization of protocol design for user training in EEG-based brain-computer interface.

Brain-computer interfaces (BCIs) are systems that enable a person to interact with a machine using only neural activity. Such interaction can be non-intuitive for the user hence user training methods are developed to increase one's understanding, confidence and motivation, which would in parallel increase system performance. To clearly address the current issues in the BCI user training protocol design, here it is divided intointroductoryperiod and BCIinteractionperiod. First, theintroductoryperiod (before BCI interaction) must be considered as equally important as the BCI interaction for user training. To support this claim, an extensive literature review demonstrates that BCI performance can depend on the methodologies presented in such introductory period. To standardize its design, the user training models from human-computer interaction field are adjusted to the BCI context. Second, during the user-BCI interaction, the interface can take a large spectrum of forms (2D, 3D, size, colour etc) and modalities (visual, auditory or haptic etc) without following any design standard or guidelines. Namely, studies that explore perceptual affordance on neural activity, show that motor neurons can be triggered from a simple observation of certain objects, and depending on objects' properties (size, location etc) neural reactions can vary greatly. Surprisingly, the effects of perceptual affordance were not investigated in the BCI context. Both inconsistent introductions to BCI as well as variable interface designs make it difficult to reproduce experiments, predict their outcomes and compare results between them. To address these issues, a protocol design standardization for BCI user training is proposed.

A review of user training methods in brain computer interfaces based on mental tasks.

Mental-tasks based brain-computer interfaces (MT-BCIs) allow their users to interact with an external device solely by using brain signals produced through mental tasks. While MT-BCIs are promising for many applications, they are still barely used outside laboratories due to their lack of reliability. MT-BCIs require their users to develop the ability to self-regulate specific brain signals. However, the human learning process to control a BCI is still relatively poorly understood and how to optimally train this ability is currently under investigation. Despite their promises and achievements, traditional training programs have been shown to be sub-optimal and could be further improved. In order to optimize user training and improve BCI performance, human factors should be taken into account. An interdisciplinary approach should be adopted to provide learners with appropriate and/or adaptive training. In this article, we provide an overview of existing methods for MT-BCI user training-notably in terms of environment, instructions, feedback and exercises. We present a categorization and taxonomy of these training approaches, provide guidelines on how to choose the best methods and identify open challenges and perspectives to further improve MT-BCI user training.

Long-Term BCI Training of a Tetraplegic User: Adaptive Riemannian Classifiers and User Training.

While often presented as promising assistive technologies for motor-impaired users, electroencephalography (EEG)-based Brain-Computer Interfaces (BCIs) remain barely used outside laboratories due to low reliability in real-life conditions. There is thus a need to design long-term reliable BCIs that can be used outside-of-the-lab by end-users, e.g., severely motor-impaired ones. Therefore, we propose and evaluate the design of a multi-class Mental Task (MT)-based BCI for longitudinal training (20 sessions over 3 months) of a tetraplegic user for the CYBATHLON BCI series 2019. In this BCI championship, tetraplegic pilots are mentally driving a virtual car in a racing video game. We aimed at combining a progressive user MT-BCI training with a newly designed machine learning pipeline based on adaptive Riemannian classifiers shown to be promising for real-life applications. We followed a two step training process: the first 11 sessions served to train the user to control a 2-class MT-BCI by performing either two cognitive tasks (REST and MENTAL SUBTRACTION) or two motor-imagery tasks (LEFT-HAND and RIGHT-HAND). The second training step (9 remaining sessions) applied an adaptive, session-independent Riemannian classifier that combined all 4 MT classes used before. Moreover, as our Riemannian classifier was incrementally updated in an unsupervised way it would capture both within and between-session non-stationarity. Experimental evidences confirm the effectiveness of this approach. Namely, the classification accuracy improved by about 30% at the end of the training compared to initial sessions. We also studied the neural correlates of this performance improvement. Using a newly proposed BCI user learning metric, we could show our user learned to improve his BCI control by producing EEG signals matching increasingly more the BCI classifier training data distribution, rather than by improving his EEG class discrimination. However, the resulting improvement was effective only on synchronous (cue-based) BCI and it did not translate into improved CYBATHLON BCI game performances. For the sake of overcoming this in the future, we unveil possible reasons for these limited gaming performances and identify a number of promising future research directions. Importantly, we also report on the evolution of the user's neurophysiological patterns and user experience throughout the BCI training and competition.

Active inference as a unifying, generic and adaptive framework for a P300-based BCI.

OBJECTIVE: Going adaptive is a major challenge for the field of brain-computer interface (BCI). This entails a machine that optimally articulates inference about the user's intentions and its own actions. Adaptation can operate over several dimensions which calls for a generic and flexible framework. APPROACH: We appeal to one of the most comprehensive computational approach to brain (adaptive) functions: the active inference (AI) framework. It entails an explicit (probabilistic) model of the user that the machine interacts with, here involved in a P300-spelling task. This takes the form of a discrete input-output state-space model establishing the link between the machine's (i) observations-a P300 or error potential for instance, (ii) representations-of the user intentions to spell or pause, and (iii) actions-to flash, spell or switch-off the application. MAIN RESULTS: Using simulations with real EEG data from 18 subjects, results demonstrate the ability of AI to yield a significant increase in bit rate (17%) over state-of-the-art approaches, such as dynamic stopping. SIGNIFICANCE: Thanks to its flexibility, this one model enables to implement optimal (dynamic) stopping but also optimal flashing (i.e. active sampling), automated error correction, and switching off when the user does not look at the screen anymore. Importantly, this approach enables the machine to flexibly arbitrate between all these possible actions. We demonstrate AI as a unifying and generic framework to implement a flexible interaction behaviour in a given BCI context.

Mutational analysis of GJB1, MPZ, PMP22, EGR2, and LITAF/SIMPLE in Serbian Charcot-Marie-Tooth patients.

We report the results of mutational analysis in the following genes: GJB1, MPZ, PMP22, EGR2, and LITAF/SIMPLE in 57 Charcot-Marie-Tooth (CMT) patients of Serbian origin without the PMP22 duplication. We found 10 different mutations in 14 CMT patients: 6 mutations in GJB1, 3 in MPZ, and 1 in PMP22. Five of six GJB1 mutations are reported for the first time, and the most frequent one appears to be a founder mutation in the Serbian population. No mutations were found in EGR2 or LITAF. Thus, GJB1 mutation analysis should be done in patients without the PMP22 duplication and male-to-male transmission of CMT.

Impact of Education on Hypothermia Delivery during Neonatal Transport.

BACKGROUND: Moderate therapeutic hypothermia (TH) initiated within 6 h of life reduces adverse neurodevelopmental outcomes in infants after perinatal hypoxic ischaemic insult. For infants born in non-tertiary centres, TH may be initiated manually en route to a neonatal intensive care unit (NICU). However, both over- and undercooling is reported with this strategy, precluding some infants from the benefits of TH. OBJECTIVES: To evaluate the impact of a region-wide educational programme on the safety and efficacy of manual cooling administered by the Wellington Neonatal Transport Service (NeTS). METHODS: Clinical records of infants with hypoxic ischaemic encephalopathy (HIE) retrieved by the Wellington NeTS for TH between January 2012 and June 2017 were reviewed retrospectively. Temperature outcomes of infants retrieved before and after the education programme were compared. RESULTS: A total of 101 infants were cooled manually by Wellington NeTS for TH during the study period. Education and training significantly reduced the rate of overcooling to ≤32.0°C (4/43 [9%] vs. 0/58, p = 0.02). However, there was no difference in the proportion of infants who achieved target rectal temperature within 6 h of life (29/43 [65%] vs. 35/58 [60%], p = 0.57). CONCLUSIONS: Introduction of a region-wide educational programme may have improved the safety of manual cooling during neonatal transport but it had a negligible impact on its efficacy. The use of servo-controlled cooling during transport should therefore be considered to improve access to the optimal neuroprotective benefits of TH for outborn infants with HIE.

Epidemiology of myotonic dystrophy type 1 (Steinert disease) in Belgrade (Serbia).

The aim of this study was to estimate the incidence and prevalence of myotonic dystrophy type 1 (DM1) in Belgrade during the period 1983-2002. The patients who had DM1 were ascertained through hospital records from all neurological departments in Belgrade during 1983-2002. The molecular genetic analysis was performed in all patents included in the study. We identified 101 DM1 patients (52 males and 49 females). The average annual incidence rate of DM1 in Belgrade for the period observed was 2.0/1,000,000 (95% confidence interval (CI), 0.3-8.3), 2.1/1,000,000 (95% CI, 0.3-8.3) for males and 2.0/1,000,000 (95% CI, 0.3-8.3) for females. The highest age-specific DM1 incidence was registered in the age group 20-49: 3.4/1,000,000 (95% CI, 0.5-7.6), 4.0/1,000,000 (95% CI, 1.1-10.2) in males and 2.5/1,000,000 (95% CI, 0.5-7.6) in females. In the population of Belgrade, a cumulative probability of acquiring DM1 was 1 per 8621 for men and 1 per 9259 for women (1 per 8940 of the population for both sexes). The prevalence of DM1 in Belgrade on 31 December 2002 was 5.3/100,000 (95% CI, 4.2-6.6).

250 CTG repeats in DMPK is a threshold for correlation of expansion size and age at onset of juvenile-adult DM1.

Myotonic dystrophy type 1 (DM1) is associated with an expansion of CTG repeats in the 3'UTR of the DMPK gene. It is accepted, as in other trinucleotide diseases, that the number of the repeats is correlated with age at onset and severity of the disease. However, assessment of genotype-phenotype correlation in DM1 is complicated with the expansion-biased somatic instability of mutant alleles over time and difficulties in precise assessment of the number of repeats by standard Southern blot hybridization. In order to clarify this issue we defined DM1 expansion size in lymphocytes by three parameters: size of progenitor, average, and largest allele, using a more precise small-pool/long-range PCR technique. We found a negative linear correlation of age at onset and average expansion size in juvenile-adult DM1 patients (35 out of 46) whose progenitor allele is less than 245 repeats long. Our result favors the hypothesis of the existence of a threshold in the progenitor allele size beyond which number of CTG repeats does not influence age at onset. Potential clinical significance is that the average allele size could be a useful indicator for the age at onset in juvenile-adult DM1 patients with relatively short progenitor allele. To test whether somatic instability of mutant alleles influences the progression of DM1, patients were divided in three phenotypic classes according to the severity of neuromuscular symptoms. We showed that the largest expansion in each DM1 phenotypic class reflects somatic instability of mutant allele over time independently of progenitor allele size and patient's age at sampling. The mean of the largest expansion was significantly different between phenotypic classes, implying the possible association between expansion-biased somatic instability of mutant alleles over time and progression of neuromuscular symptoms.

Protective effects of oestradiol against cadmium-induced changes in blood parameters and oxidative damage in rats.

The aim of this study was to investigate the protective effects of oestradiol (E2, 4 mg kg-1 b.w. i.p.) against cadmium-induced (Cd, 2 mg kg-1 b.w. i.p.) blood changes in rats. Cadmium induced a significant decline in haemoglobin, haematocrit, and total erythrocyte, lymphocyte, and thrombocyte count, whereas total leukocytes and granulocytes increased. A significant increase was also observed in serum cholesterol, triglycerides, glucose, AST, and ALT activities, whereas total protein and albumin levels dropped significantly. Administration of E2 in combination with Cd alleviated most of these adverse effects. In terms of oxidative stress, Cd significantly increased oxygen-free radicals (O2 •- and H2O2) in neutrophils and lipid peroxidation in erythrocytes, whereas E2 treatment reversed these changes to control values. Acute Cd poisoning significantly lowered antioxidant enzyme (SOD and CAT) activity and the level of non-enzymatic antioxidants (GSH and vitamin E), while increasing in GSSG. Treatments with E2 reversed Cd-induced effects on the antioxidant defences and significantly lowered Cd-induced oxidative damage in erythrocytes. This study suggests that exogenous E2 effectively restores redox balance in rat erythrocytes and counters adverse haematological and biochemical effects of Cd poisoning. It also improves the antioxidant capacity of erythrocytes, acting in synergy with endogenous antioxidants.

An algorithm for genetic testing of Serbian patients with demyelinating Charcot-Marie-Tooth.

Charcot-Marie Tooth (CMT) is a clinically and genetically heterogeneous group of diseases with rough genotype-phenotype correlation, so the final diagnosis requires extensive clinical and electrophysiological examination, family data, and gene mutation analysis. Although there is a common pattern of genetic basis of CMT, there could be some population differences that should be taken into account to facilitate analyses. Here we present the algorithm for genetic testing in Serbian patients with demyelinating CMT, based on their genetic specificities: in cases of no PMP22 duplication, and if -X-linked CMT (CMTX) is not contraindicated by pattern of inheritance (male-to-male transmission), one should test for c.94A>G GJB founder mutation, first. Also, when a patient is of Romani ethnicity, or if there is an autosomal recessive inheritance in a family and unclear ethnicity, c.442C>T mutation in NDRG1 should be tested.

Phenolic compounds and biological activity of Kitaibelia vitifolia.

This study was aimed at evaluating the antioxidant activity and efficacy of the ethanolic extract of the endemic plant species Kitaibelia vitifolia in inhibiting the growth of selected fungi and bacteria. Antimicrobial activity was tested using the broth dilution procedure for determination of minimum inhibitory concentration (MIC). MICs were determined for eight selected indicator strains. The highest susceptibility to K. vitifolia ethanolic extract among the bacteria tested was exhibited by Bacillus subtilis ATCC 6633, Staphylococcus aureus ATCC 25923, and Klebsiella pneumoniae ATCC 13883 (MIC=15.62 µg/mL), followed by Escherichia coli ATCC 25922 and Proteus mirabilis ATCC 14153 (MIC=31.25 µg/mL), and Proteus vulgaris ATCC 13315 (MIC=62.50 µg/mL). Of the fungi, Candida albicans ATCC 10231 (MIC=15.62 µg/mL) showed the highest susceptibility, and Aspergillus niger ATCC 16404 (MIC=31.25 µg/mL) had the lowest. Results showed that K. vitifolia extract possesses antioxidant activity, with total antioxidant capacity of 75.45±0.68 µg of ascorbic acid/g and 50% inhibition concentration values of 47.45±0.55 µg/mL for 2,2-diphenyl-1-picrylhydrazyl free radical scavenging activity, 35.35±0.68 µg/mL for inhibitory activity against lipid peroxidation, 95.25±0.52 µg/mL for hydroxyl radical scavenging activity, and 31.50±0.35 µg/mL for metal chelating activity. Total phenolics, flavonoids, condensed tannins, and gallotannins were 85.25±0.69 mg of gallic acid (GA)/g, 45.32±0.55 mg of rutin/g, 54.25±0.75 mg of GA/g, and 41.74±0.55 mg of GA/g, respectively. The phenolic composition of K. vitifolia extract was determined by high-performance liquid chromatography. Rosmarinic acid was found to be the dominant phenolic compound of the extract.

Childhood cerebral X-linked adrenoleukodystrophy more than 5 years after hematopoietic cell transplantation: the first case from Serbia and southeastern Europe.

We report the clinical course, brain magnetic resonance imaging (MRI), and proton magnetic resonance spectroscopy findings in a boy with childhood cerebral X-linked adrenoleukodystrophy whose neurological disease keeps progressing more than 5 years after conventional hematopoietic cell transplantation with full donor-derived engraftment accomplishment. The described clinical and radiological findings follow all phases of this childhood cerebral X-linked adrenoleukodystrophy: from the clinically asymptomatic pretransplant stage to the present day. This is the first patient not only from Serbia but from the entire area of Southeastern Europe who underwent hematopoietic cell transplantation for childhood cerebral X-linked adrenoleukodystrophy. The presented disease course and the posttransplant outcome in the only case of transplanted adrenoleukodystrophy from Serbia enhances the overwhelming appeal for better X-linked adrenoleukodystrophy screening, earlier disease detection, and contributes to the well-known anticipation of the refined hematopoietic cell transplantation eligibility criteria in future adrenoleukodystrophy treatment.