Influence of the physical pressure of an ultrasound probe on shear-wave elastography measurements of the gastrocnemius muscle in a paediatric population: a non-interventional cohort study.

Purpose: The aim of our study is to evaluate the impact of ultrasound probe mechanical pressure on the stiffness of the gastrocnemius muscle in a healthy paediatric population. As far as we know, there has been no previous qualitative in vivo study on the impact of probe pressure on muscle shear-wave elastography results with objective evaluation of compression in the paediatric population. Material and methods: In this cohort study, a group of 22 children (mean age 8.99 years, SD 2.74, 11 males) underwent elastography of the gastrocnemius muscle of the dominant leg. A custom-made, 3-dimensional printed probe cover was used to measure the mechanical pressure of the probe on tissues. Results: The obtained results were related to the age, sex, BMI, and calf circumference of the subjects. We observed a significant difference in the stiffness parameter at a pressure of 1 N, with a further increase if force was increased (p < 0.001). A significant, very weak positive correlation of age and stiffness was observed (p < 0.001, r2 = 0.022). There was no significant correlation of stiffness, BMI, and calf circumference. Conclusions: The use of compression during muscle elastography in children causes a significant bias in results, regardless of age, sex, BMI, or calf size.

Analysis of 622 paediatric hospitalisations due to arterial ischaemic stroke in Poland - National Health Fund registry-based study from 2011 to 2020.

Introduction: The present study aimed to evaluate the prevalence of arterial ischaemic stroke (AIS) in Polish children, as well as to analyse the parameters related to AIS hospitalisation, including age, gender, region, month and season of the year at admission, duration, and costs, based on data from National Health Fund (NHF) registry in 2011-2020. Material and methods: Data from the NHF were analysed statistically. The disease was identified according to the codes I63 and I64 of the International Classification of Diseases, 10th Revision (ICD-10), and patients included only individuals up to 18 years of age. Results: We identified 622 hospitalisations due to paediatric AIS in Poland in the study period. The most frequent age subgroups were adolescents, followed by toddlers or pre-school children (34.73% and 24.12%, respectively), while the least frequent were neonates or infants (9.81%). ICD-10 procedures did significantly affect the duration and costs of hospitalisation (p < 0.001). The highest costs of hospitalisations concerned the I63.1 procedure (cerebral infarction due to embolism of precerebral arteries), which included thrombectomy. The duration and costs of hospitalisation were positively correlated with each other (r = 0.525, p < 0.001). Age correlated negatively with duration of hospitalisation (r = -0.154, p < 0.001) and positively with costs of hospitalisation (r = 0.133, p = 0.008). Conclusions: Data from the NHF registry proved that AIS occurs more often in boys than in girls and is more common in adolescents (15-18 years) than in younger children.

Polish recommendations for diagnosis and therapy of paediatric stroke.

Stroke remains one of the greatest health challenges worldwide, due to a high mortality rate and, despite great progress in its treatment, the significant disability that it causes. Studies conducted around the world show that the diagnosis of stroke in children is often significantly delayed. Paediatric ischaemic arterial stroke (PAIS) is not only a problem that varies greatly in frequency compared to the adult population, it is also completely different in terms of its risk factors, clinical course and outcome. The main reason for the lack of a rapid diagnosis of PAIS is a lack of access to neuroimaging under general anaesthesia. The insufficient knowledge regarding PAIS in society as a whole is also of great importance. Parents and carers of children should always bear in mind that paediatric age is not a factor that excludes a diagnosis of stroke. The aim of this article was to develop recommendations for the management of children with acute neurological symptoms suspected of ischaemic stroke and further treatment after confirmation of the ischaemic aetiology of the problem. These recommendations are based on current global recommendations for the management of children with stroke, but our goal was also to match them as closely as possible to the needs and technical diagnostic and therapeutic possibilities encountered in Poland. Due to the multifactorial problem of stroke in children, not only paediatric neurologists but also a neurologist, a paediatric cardiologist, a paediatric haematologist and a radiologist took part in the preparation of these recommendations.

Study of 52 Pediatric Patients Who Required 78 Hospital Admissions for Cerebral Venous Sinus Thrombosis from 2013 to 2020 Using Data from the Polish National Health Fund Registry.

BACKGROUND Cerebral venous sinus thrombosis (CVST) in children is a rare disease with a complex, multifactorial etiopathogenesis. The Polish National Health Fund (NHF) Registry [Narodowy Fundusz Zdrowia (NFZ)], contains health insurance data from all 16 national provinces, or voivodeships. This study used data from the Polish NHF Registry to evaluate 52 pediatric patients who required 78 hospital admissions for CVST from 2013 to 2020. MATERIAL AND METHODS The data in the Polish NHF Registry were acquired based on the disease code I67.6 from the International Classification of Diseases, Tenth Revision (ICD-10), and the patients' age (up to 18 years old). RESULTS We identified 78 hospitalizations of 52 pediatric patients due to CVST in Poland from 2013 to 2020 (63.5% boys and 36.5% girls, mean age 9.7±5.8 years old). The mean duration of hospitalization was 10.5±11.7 days, the mean cost of hospitalization was 3273±2191 Polish zloty (PLN). The most common age subgroup was adolescents (27%). Ten percent of patients were hospitalized in a region other than their region of residence. The duration and cost of hospitalization were positively correlated with each other (r=0.512, P<0.001). The most common type of admission was an emergency (51%), and the most common discharge was referral for further outpatient treatment (50%). CONCLUSIONS Polish registry data showed that from 2013 to 2020, CVST was more commonly diagnosed in male adolescents from 15 to 18 years of age who presented as emergency hospital admissions. There were regional differences in incidence and duration of hospital stay and healthcare costs between patients.

Selected Factors of Vascular Changes: The Potential Pathological Processes Underlying Primary Headaches in Children.

BACKGROUND: The prevalence, social consequences and complicated pathogenesis make headaches in children a significant clinical issue. Studies in adults suggest that primary headaches could be the first sign of atherosclerosis and platelet aggregation. AIM: To analyze the blood levels of selected biomarkers of vascular changes potentially associated with a higher risk of atherosclerosis in children with primary headaches. METHODS: The medical family history, brain-derived neurotrophic factor (BDNF), soluble CD40 ligands (sCD40L), endothelial plasminogen activator inhibitor (PAI I), vascular endothelial growth factor (VEGF) and intima-media thickness (IMT) measurements were performed in the 83 children (52 with primary headaches, 31 controls). Selected factors were compared with basic laboratory parameters that are potentially related to atherosclerosis: C-reactive protein (CRP) and lipid concentration. RESULTS: There were no significant differences in biomarkers of vascular changes in the study group and controls in general. In the study group, boys had a higher BDNF level than girls (p = 0.046). Normal-weight migraine patients had significantly higher PAI-I levels than controls (p = 0.034). A positive correlation between PAI-1 and triglycerides (TG) was observed. IMT did not differ between children with primary headaches and controls; however, IMT showed a positive correlation with BMI z-score and TG. Children with headaches had, more often, a positive family history of cardiovascular disease (p = 0.049). CONCLUSIONS: There were no clear clinical changes indicative of atherosclerosis in the study population. However, some trends are visible. Primary headaches are more often related to a family history of cardiovascular diseases. IMT is associated with TG levels and BMI z-score. The measured biomarkers of vascular changes show mutual relations.

Do Antiepileptic Drugs Change the Levels of Arginine Derivatives in Epileptic Children Treated with Polytherapy? The Results of a Case-Control Study.

Previously, a relation between therapy with antiepileptic drugs (AEDs) and the levels of biochemical parameters was observed in adult patients suffering from epilepsy. Among these biochemical factors, arginine derivatives are often analyzed, i.e., asymmetric dimethylarginine (ADMA), symmetric dimethylarginine (SDMA), and homoarginine (hArg) as they may be linked with increased risk for cardiovascular disease (CVD). Since the levels of arginine derivatives may increase during therapy, and the treatment of epilepsy often lasts many years, patients may experience CVD faster. The aim of the present study was to analyze the levels of arginine derivatives in children with epilepsy who were treated with multiple AEDs to answer the question whether pediatric patients may be at increased risk of CVD in the future. We prospectively analyzed 21 children suffering from epilepsy who took ≥2 AEDs for at least 6 months and 22 children without epilepsy (reference group). The levels of the arginine derivatives, e.g., ADMA, SDMA, and hArg, were determined in the blood serum using the HPLC method. No differences in both the mean levels of ADMA and SDMA, as well as in the mean values of the arginine derivative ratios, were observed between the groups. The tendency toward a lower level of hArg was found in epileptic patients more than in the reference group (p = 0.091). Epileptic children receiving three or more AEDs had significantly lower concentrations of hArg and values of the hArg/ADMA ratio than the reference group (p = 0.023 and p = 0.006, respectively). In turn, the mean hArg/ADMA ratio was lower in children receiving three or more AEDs compared to children receiving two AEDs (p = 0.002). There was also a positive correlation between the hArg and ADMA concentrations in children with epilepsy taking two AEDs; the higher the level of hArg, the greater the level of ADMA on average (r = 0.650, p = 0.022). Taking three or more AEDs by epileptic children resulted in lower levels of both hArg and the value of the hArg/ADMA ratio.

Introduction to the Special Issue on Ischemic Stroke in Children.

The occurrence of arterial ischemic stroke (AIS) is a serious medical problem due to the deleterious neurological consequences that affect the daily functioning of the patient as well as the costs of medical care and rehabilitation [...].

International Prevalence and Mechanisms of SARS-CoV-2 in Childhood Arterial Ischemic Stroke During the COVID-19 Pandemic.

BACKGROUND: Data from the early pandemic revealed that 0.62% of children hospitalized with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had an acute arterial ischemic stroke (AIS). In a larger cohort from June 2020 to December 2020, we sought to determine whether our initial point estimate was stable as the pandemic continued and to understand radiographic and laboratory data that may clarify mechanisms of pediatric AIS in the setting of SARS-CoV-2. METHODS: We surveyed international sites with pediatric stroke expertise to determine numbers of hospitalized SARS-CoV-2 patients <18 years, numbers of incident AIS cases among children (29 days to <18 years), frequency of SARS-CoV-2 testing for children with AIS, and numbers of childhood AIS cases positive for SARS-CoV-2 June 1 to December 31, 2020. Two stroke neurologists with 1 neuroradiologist determined whether SARS-CoV-2 was the main stroke risk factor, contributory, or incidental. RESULTS: Sixty-one centers from 21 countries provided AIS data. Forty-eight centers (78.7%) provided SARS-CoV-2 hospitalization data. SARS-CoV-2 testing was performed in 335/373 acute AIS cases (89.8%) compared with 99/166 (59.6%) in March to May 2020, P<0.0001. Twenty-three of 335 AIS cases tested (6.9%) were positive for SARS-CoV-2 compared with 6/99 tested (6.1%) in March to May 2020, P=0.78. Of the 22 of 23 AIS cases with SARS-CoV-2 in whom we could collect additional data, SARS-CoV-2 was the main stroke risk factor in 6 (3 with arteritis/vasculitis, 3 with focal cerebral arteriopathy), a contributory factor in 13, and incidental in 3. Elevated inflammatory markers were common, occurring in 17 (77.3%). From centers with SARS-CoV-2 hospitalization data, of 7231 pediatric patients hospitalized with SARS-CoV-2, 23 had AIS (0.32%) compared with 6/971 (0.62%) from March to May 2020, P=0.14. CONCLUSIONS: The risk of AIS among children hospitalized with SARS-CoV-2 appeared stable compared with our earlier estimate. Among children in whom SARS-CoV-2 was considered the main stroke risk factor, inflammatory arteriopathies were the stroke mechanism.

Effectiveness of ACTH in Patients with Infantile Spasms.

(1) Background: West syndrome is a severe, refractory, epileptic syndrome that usually appears in infancy or early childhood. ACTH is one of the more effective drugs for treating this condition. (2) Aim of the study and methods: The objective of our study was to examine short-term efficacy (during treatment schedule) and long-term outcome of intramuscular 0.02 mg/kg/day ACTH (tetracosactide) depot, used concomitantly with other antiepileptic drugs (AEDs) in patients with infantile spasms who did not achieve seizure cessation or relapse when taking only the AEDs. The drug efficacy was evaluated in retrospective and prospective analyses of 50 patients diagnosed with infantile spasms. (3) Results: Complete cessation of spasms was achieved in 42 cases (84%). EEG improvement was seen in 41 (82%) patients who responded to ACTH therapy. Information on the clinical course of 28 patients was obtained duringlong-term follow-up. In 17 (60.7%) cases, seizures were still present. Normal or near-normal development was observed in 11 out of 28 children (39%). ACTH used concomitantly with other AEDis a highly effective treatment with acceptable side effects. (4) Conclusion: Randomized controlled clinical trialswith long-term follow-up are needed to compare the effectiveness of ACTH in polytherapy and monotherapy. Dyskinesias as a potential side effect observed in our study group should be investigated in the following studies.

Serum Levels of Lipids and Selected Aminothiols in Epileptic Children-A Pilot Case-Control Study.

BACKGROUND: Standard treatment of epileptic seizures involves the use of antiepileptic drugs (AEDs). Both AEDs themselves and treatment duration may influence the levels of biochemical parameters, e.g., lipids or homocysteine (HCys), that may increase the risk of cardiovascular diseases. The aim of the present study was to compare the levels of lipid parameters, as well as the concentrations of selected aminothiols (i.e., HCys, cysteine, and glutathione) between epileptic children treated with multiple AEDs and children without epilepsy. METHODS: In the study, 21 children with epilepsy treated with two or more AEDs for at least 6 months (8 girls and 13 boys, mean age 7.03 ± 4.51) and 23 children without epilepsy (7 girls and 16 boys, mean age 7.54 ± 3.90) were prospectively analyzed. Lipid parameters, i.e., total cholesterol (TC), triglycerides (TG), low density lipoprotein (LDL) and high density lipoprotein (HDL), and levels of selected aminothiols were determined in the blood serum. RESULTS: No differences in the mean levels of lipid parameters and in the mean values of lipid ratios (TC/HDL, TG/HDL, LDL/HDL) were observed between the total groups as well as in the sex subgroups. HCys and cysteine levels did not differ between the patients and controls. We observed significantly lower levels of glutathione in children with epilepsy than in children without epilepsy (1.49 ± 0.35 µmol/L vs. 2.39 ± 1.17 µmol/L, respectively) (p < 0.001). Glutathione level was also lower in boys with epilepsy than in boys without epilepsy (p = 0.007). Similarly, epileptic girls had statistically decreased levels of glutathione when compared to girls without epilepsy (p = 0.006). CONCLUSIONS: A lower level of glutathione is observed in pediatric patients with epilepsy treated with two or more AEDs for at least 6 months. This indicates the oxidative stress of the patients treated with AEDs, which in turn may affect their well-being, and in the case of chronic occurrence resulting from long-term treatment, also on the function of the liver and the condition of the cardiovascular system.

Medico-legal analysis of cases of children who died suddenly due to pneumonia undiagnosed in their lifetime. / Sadowo-lekarska analiza przypadków dzieci zmarlych nagle z powodu nierozpoznanego za zycia zapalenia pluc.

Pneumonia is one of the most common causes of children's hospitalization and death. The aim of the study was a medico-legal analysis of children who died suddenly due to pneumonia undiagnosed in their lifetime. The research was of a retrospective character and consisted in an analysis of prosecution files. The study included 47 children who died between 2011-2018 in whom pneumonia after post-mortem examination was indicated as the cause of death, as well as children in whom the cause of death, despite additional post-mortem examinations, including histopathological tests, was not established. In some cases, under additional post-mortem examinations, additional targeted immunohistochemical staining of selected lung sections was performed to establish the diagnosis. In children with prodromal symptoms, histopathological examination showed significantly more frequent atelectasis than in children without prodromal symptoms. Pneumonia is a significant clinical problem. Especially in young children, it may proceed with- out any symptoms that would cause such a diagnosis to be made. A properly conducted post-mortem diagnosis supplemented by immunohistochemical examinations allows to reduce the number of unexplained deaths in children.

Analysis of Selected Risk Factors Depending on the Type of Cerebral Palsy.

Background: Cerebral palsy (CP) is not a defined, separate disease classification, but a set of etiologically diverse symptoms that change with the child's age. According to the up-to-date definition, CP is a group of permanent but not unchanging disorders of movement and/or posture and motor function, which are due to a nonprogressive interference, lesion, or abnormality of the developing/immature brain. CP is one of the most frequent causes of motor disability in children. The aim of the present study was to analyze whether selected risk factors may vary depending on particular types of CP. Methods: 181 children with CP (aged 4-17 years), hospitalized at the Department of Pediatrics and Developmental Age Neurology in Katowice in the years 2008-2016 were retrospectively analyzed in the present study. The assumed risk factors of CP were divided into two groups: 1-pre-conception and prenatal (mother's age, family history of epilepsy, burdened obstetric history, mother's systemic diseases, pregnancy order, multiple pregnancy, duration of pregnancy, bleedings from the genital tract during gestation, arterial hypertension during pregnancy, infections during pregnancy, preterm contractions, maintained pregnancy, premature rupture of membranes, abruptio placentae, and others), 2-perinatal and postnatal (mode of delivery, birth weight, Apgar score at the first and fifth minute, neonatal convulsions, respiratory failure, infections in neonatal and infant period, and intraventricular bleeding). The division into particular CP types was based on Ingram's classification. Results: The following risk factors were the most frequent in the total group: respiratory failure, infections, intraventricular bleeding, and prematurity. Among the analyzed preconception and prenatal factors, the duration of pregnancy and preterm contractions during pregnancy significantly differentiated the subgroups of patients depending on the type of CP. The prevalence of almost all analyzed perinatal, neonatal, and infant-related risk factors (i.e., birth weight, Apgar score at the first and fifth minute, neonatal convulsions, respiratory failure, infections in neonatal and infant period, and intraventricular bleeding) significantly differed between CP types, apart from the mode of delivery. However, in multivariate regression, only intraventricular bleeding was an independent predictor for tetraplegic CP type when compared to joined extrapyramidal and ataxic types (OR = 2.801, p = 0.028). Conclusions: As CP is a syndrome of multifactorial etiology, the identification of CP risk factors entails the need for careful observation and comprehensive care of children in the risk group. The presence of certain risk factors may be a prognostic indicator for particular types of CP. The knowledge about the association between the risk factor(s) and the CP type could be a very useful tool for pediatricians looking after the child at risk of developmental disorders.

Muscle Ultrasonographic Elastography in Children: Review of the Current Knowledge and Application.

Ultrasonographic elastography is a relatively new imaging modality for the qualitative and quantitative assessments of tissue elasticity. While it has steadily gained use in adult clinical practice, including for liver diseases, breast cancer, thyroid pathologies, and muscle and tendon diseases, data on its paediatric application is still limited. Moreover, diagnosis of muscular diseases in children remains challenging. The gold standard methods, namely biopsy, electroneurography, and electromyography, are often limited owing to their invasive characteristics, possible contraindications, complications, and need for good cooperation, that is, a patient's ability to perform certain tasks during the examination while withstanding discomfort, which is a significant problem especially in younger or uncooperative children. Genetic testing, which has broad diagnostic possibilities, often entails a high cost, which limits its application. Thus, a non-invasive, objective, repeatable, and accessible tool is needed to aid in both the diagnosis and monitoring of muscle pathologies. We believe that elastography may prove to be such a method. The aim of this review was to present the current knowledge on the use of muscle elastography in the paediatric population and information on the limitations of elastography in relation to examination protocols and factors for consideration in everyday practice and future studies.

Early Deaths after Arterial Ischemic Stroke in Pediatric Patients: Incidence and Risk Factors.

In developed countries, cerebrovascular diseases are among the 10 most common causes of death in both the pediatric and adult population. The prevalence of fatal outcomes following arterial ischemic stroke (AIS) in various groups of pediatric patients ranges from 1% to almost 32%. However, a constant improvement in stroke mortality among children has been observed. The extent of the decline differs among studies (from nearly tenfold to twofold decline), as it depends on the study population. While a portion of this variability might be explained by factors such as health care access, population age, diseases related to ethnicity, and different etiologies of stroke in studied populations, the understanding of such differences is still insufficient. Risk factors for death in the early stages of the disease are poorly understood and are usually based on the clinical presentations of relatively small groups of pediatric patients. Familiarity with these factors may be of significant importance for prognosis, but also for the early selection of patients requiring careful supervision. The present study aimed to analyze and discuss the current literature data on the incidence of early death and risk factors for early death in children suffering from stroke.

Assessment of Post-Stroke Consequences in Pediatric Ischemic Stroke in the Context of Neuroimaging Results-Experience from a Single Medical Center.

Arterial ischemic stroke (AIS) in children is a rare condition; its frequency is estimated at 0.58 to 7.9 new onsets in 100,000 children per year. The knowledge on risk factors, clinical outcomes and consequences of pediatric AIS is increasing. However, there are still many unknowns in the field. The aim of the study was to analyze the clinical presentation of pediatric AIS and its consequences according to the neuroimaging results and location of ischemia. The research was retrospective and observational. The analyzed group consisted of 75 AIS children (32 girls, 43 boys), whereby the age of the patients ranged from 9 months to 18 years at stroke onset. All the patients were diagnosed and treated in one tertiary center. The most frequent stroke subtype was total anterior circulation infarct (TACI) with most common ischemic focus location in temporal lobe and vascular pathology in middle cerebral artery (MCA). The location of ischemic focus in the brain correlated with post-stroke outcomes: intellectual delay and epilepsy, hemiparesis corresponded to the location of vascular pathology. A correlation found between ischemic lesion location and vascular pathology with post-stroke consequences in pediatric AIS may be important information and helpful in choosing proper early therapy. The expected results should lead to lesser severity of late post-stroke outcomes.

The Impact of Sex on Arterial Ischemic Stroke in Young Patients: From Stroke Occurrence to Poststroke Consequences.

The male sex has been suggested to predominate in paediatric patients with arterial ischemic stroke (AIS), especially in newborns. The explanation for this phenomenon remains unsatisfactory since it focuses on the analysis of the potential relationship with trauma and arterial dissection. In turn, in some populations of young adults, men suffer from AIS more frequently than women, which may be related to the protective role of oestrogen. On the other hand, certain data indicate that women dominate over men. Some of the disparities in the frequencies of particular symptoms of AIS and poststroke consequences in both children and young adults have been suggested; however, data are scarce. Unfortunately, the low number of studies on the subject does not allow certain conclusions to be drawn. For adults, more data are available for patients aged over 60 years, the results of which are more obvious. The present literature review aimed to discuss available data on the prevalence of AIS, its clinical presentations, and poststroke consequences in regard to the sex of young patients. We considered young patients to be children from birth up to the age of 19 years of life and young adults to be individuals up to the age of 55 years. The role of sex hormones in AIS and possible gender differences in genetic risk factors for AIS were also discussed briefly.

Levels of Lipid Parameters in Children with Arterial Ischemic Stroke and Headache: Case-Control Study and Meta-Analysis.

Background: Abnormalities in levels of lipid parameters are one of the main causes of cardiovascular and cerebrovascular disease in adults. There are limited data on the role of disturbances of lipid metabolism in the etiopathogenesis of arterial ischemic stroke (AIS) in children and the results provided are ambiguous. The aim of the study was to compare the levels of lipid parameters (total cholesterol [TC], triglycerides [TG], high-density lipoprotein [HDL] and low-density lipoprotein [LDL]) between children with AIS, children with headache and healthy children. In addition, we performed meta-analysis of available data on lipid parameters in young patients with AIS. Methods: We retrospectively analyzed 218 children hospitalized between 2002 and 2018 in the Upper-Silesian Child's Health Center (n = 82 children with AIS, n = 45 children with headache, n = 91 healthy children) with available data on lipid levels, i.e., TC, TG, and HDL. The levels of LDL, non-HDL cholesterol, and a very-low density lipoprotein (VLDL) were calculated. The ratios of TC/HDL, TG/HDL and LDL/HDL were also assessed. Data between cases and controls were analyzed using STATISTICA 13.0 whereas meta-analysis was performed with RevMan version 5.4 software. Results: Children with headache were significantly older than children with AIS (p = 0.001). Ten percent of children with AIS had posterior stroke. The mean TC level was significantly higher in the AIS children than in controls or in children with headache. Mean TG and VLDL levels were significantly different between all groups (p < 0.001 each). The hypertriglyceridemia was more prevalent in AIS children than in children with headache (39% vs. 13%, OR = 4.16 95% CI 1.58-10.94, p = 0.004). Similarly, the frequency of dyslipidemia was higher in children with AIS compared to children with headache (38% vs. 22%, OR = 2.13 95% CI 0.93-4.89, p = 0.078). The meta-analysis was conducted based on data from 4 studies (3 studies published previously plus the results we obtained in the present case-control analysis) with total number of 236 young patients with AIS and 272 healthy controls. Significant Standard Mean Difference (SMD) was found in triglycerides level between young patients with AIS and controls (0.78 95%CI 0.30-1.26 p = 0.002). Conclusions: Lipid abnormalities, especially levels of triglycerides, seem to be of particular importance in children with AIS, as confirmed in meta-analysis. The results of the present study may be a significant contribution to the further research on the role of lipid metabolism disorders in the development of childhood stroke.

Nusinersen treatment of Spinal Muscular Atrophy Type 1 - results of expanded access programme in Poland.

AIM OF THE STUDY: This study aimed to evaluate the effects of nusinersen therapy in Polish children with SMA type 1. CLINICAL RATIONALE OF STUDY: Spinal muscular atrophy (SMA) is a neuromuscular disorder that is characterised by the loss of motor neurons, progressive muscle weakness and atrophy, leading to increased disability and mortality. Nusinersen, an antisense oligonucleotide that promotes production of the functional survival motor neuron protein is approved for the treatment of SMA 5q in the European Union. In 2017, an early access programme (EAP) for nusinersen was launched in Poland. In this study, we present the results of nusinersen treatment in Polish patients participating in the EAP. MATERIALS AND METHODS: We collected prospectively clinical data including mutational analysis of SMN1 and SMN2 genes, motor function outcomes as measured on a standardized scales, ventilatory and nutritional status, on SMA type 1 patients receiving nusinersen in three EAP centres in Poland. Scores on the CHOP-INTEND scale after 18-26 months of treatment were compared to baseline. RESULTS: We analysed data from 26 patients with SMA type 1, mean age 4.79 (2-15) years. The mutational analysis revealed two SMN2 gene copies in the majority of patients (61.54%). Three and four copies were found in 34.62% and 3.84%, respectively. Median disease duration was 21 months. Half (n = 13) of the patients required mechanical ventilation at baseline and 57.69% (n = 15) were fed by nasogastric tube or percutaneous endoscopic gastrostomy. No patient worsened during the follow-up. Mean improvement in CHOP-INTEND from baseline to the last follow-up was 7.38 points (p < 0.001). CHOP-INTEND scores did not decline for any patient. Patients with three or more SMN2 gene copies had higher scores than did the patients with two copies (p = 0.013), and they tended to show greater improvement over time, but the difference was not significant (p = 0.324). Shorter disease duration and higher CHOP-INTEND baseline score were associated with a better response (p = 0.015). Patients with a CHOP-INTEND score above the median had higher scores overall than the rest (p < 0.0013), and they improved significantly more than the rest (p = 0.037). Nusinersen was well tolerated, no new safety findings were identified. CONCLUSIONS AND CLINICAL IMPLICATIONS: Our data indicates that nusinersen treatment might be effective in SMA type 1 patients, regardless of their age and functional status.

Risk factors, types and outcomes of arterial ischemic stroke in Polish pediatric patients: a retrospective single-center study.

INTRODUCTION: Various neurological complications may occur as a consequence of arterial ischemic stroke (AIS) and have an impact on daily activity of the patients, costs of their medical care and rehabilitation. The aim of this study was to analyze risk factors, stroke symptoms and post-stroke consequences in Polish pediatric patients depending on stroke subtype. MATERIAL AND METHODS: We retrospectively reviewed 77 children under the age of 18 years following their first AIS. Patients were white, Polish Caucasians, recruited in the Department of Pediatric Neurology at the Medical University of Silesia in Katowice (Poland). Statistical analysis was performed using Statistica 12.0. RESULTS: Gender differed significantly between stroke subgroups (p = 0.030). The presence of focal cerebral arteriopathy (FCA) and chronic diseases was associated with type of AIS (p = 0.003 and p = 0.050, respectively). An outcome without neurological deterioration (normal outcome) was observed in 43% of children with lacunar anterior circulation infarct (LACI). Hemiparesis was present in almost all children with total anterior circulation infarct (TACI), in two thirds of children with partial anterior circulation infarct (PACI) and in almost 50% of children with LACI or posterior circulation infarct (POCI). In every child with hemiplegia the stroke symptom evolved into hemiparesis at follow-up. Additionally, patients with a normal outcome were older at the time of AIS than those with at least one neurological consequence (OR = 0.894, p = 0.034). CONCLUSIONS: The presence and number of neurological outcomes depend on stroke subtypes. A relation between the presence of post-stroke deficits and age at onset was observed. The odds of deficit after ischemic stroke decreases by an average of 10.6% if the child is 1 year older at the time of AIS.

Does the Occurrence of Particular Symptoms and Outcomes of Arterial Ischemic Stroke Depend on Sex in Pediatric Patients?-A Pilot Study.

Arterial ischemic stroke (AIS) in childhood is reported to occur more frequently in boys, which may lead to the assumption that the prevalence of post-stroke deficits is sex related. The present study aimed to evaluate sex-related differences in functional outcomes (hemiparesis, seizures, aphasia, and motor disturbances other than hemiparesis) in pediatric patients with AIS. A total of 89 children (52 boys and 37 girls; mean age at stroke onset: 8.4 ± 5.6 years) were evaluated retrospectively based on data from medical records. The patients were divided into subgroups according to age (i.e., infants and toddlers, children, and adolescents), stroke subtype (i.e., lacunar anterior circulation infarct (LACI), total anterior circulation infarct (TACI), partial anterior circulation infarct (PACI), posterior circulation infarct (POCI)) and stroke location (i.e., anterior stroke, posterior stroke). Significant differences in the prevalence of stroke subtypes between girls and boys were observed (p = 0.034). POCI stroke were found to be more frequent in boys than in girls (OR = 8.57 95%CI 1.05-70.23, p = 0.023). Males predominated in the total group and in all analyzed age subgroups. The proportions of boys within the subgroups according to stroke subtype were extremely high for the POCI and TACI stroke subgroups. On the other hand, girls predominated in the LACI stroke subgroup. Frequency of central type facial nerve palsy and other symptoms of AIS were found to significantly differ between male subgroups according to stroke subtype (p = 0.050 and p < 0.001, respectively), as well as between children with anterior stroke and those with posterior stroke (p = 0.059 and p < 0.001, respectively). Post-stroke seizures appeared significantly more commonly in girls with TACI and POCI stroke than in girls with LACI and PACI stroke (p = 0.022). In turn, the prevalence of post-stroke hemiparesis differed between stroke subtypes in boys (p = 0.026). In conclusion, sex may have an impact in predisposing to a certain type of AIS in the patient. Post-stroke seizure may be related to stroke subtype in girls and hemiparesis in boys. However, further studies are needed to confirm the results.