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Exercise may trigger bronchoconstriction, especially in a group of athletes in whom bronchospasm during exercise is reported to occur more frequently than in nonathletes. The aim of this study was to determine the prevalence and environmental risk factors contributing to exercise-induced bronchoconstriction (EIB) in adolescent athletes. A prospective study was conducted among a group of 101 adolescent athletes who underwent spirometry, exercise challenge, fractional exhaled nitric oxide (FeNO) measurements, and allergy assessment. The study group was divided into three subgroups of athletes based on the most common sports environments: swimmers, “indoor” athletes, and “outdoor” athletes. The clinical evaluation demonstrated a high frequency of EIB in the study group. Moreover, a large proportion of the athletes in whom EIB was observed reported no pre-existing symptoms suggestive of bronchospasm or asthma. Among patients without a previous diagnosis of asthma, clinical evaluation confirmed 22% with positive exercise challenges, compared with 77% of adolescents with negative test results. Moreover, among the athletes with a history of asthma, 39% had positive exercise challenges. Both EIB and asthma are common conditions that affect adolescent athletes. Physicians should pay particular attention to this group, as the symptoms can lead to under- and overdiagnosis.

Background. As diabetes affects multiple spheres of life the aim of this study was to explore the pain points of diabetes management as perceived by persons with type 2 diabetes and to identify their expectations towards new technologies. Methods. Patients with type 2 diabetes treated with oral hypoglycemic agents and/or insulin were surveyed. Respondents were asked to rate (i) the impact of diabetes on their daily life and (ii) their needs for improvements in different aspects of diabetes management on a five level Likert-type scale. Results. One hundred and fifty-four persons with type 2 diabetes were included. Most frequently reported challenges were: fear of diabetes complications development or progression (98.7% of patients), presence of diabetes complications (65.6%), frequent hyperglycemia (53.2%), and diabetes limiting one’s daily activities (50%). Most frequently expressed needs were: to evaluate glucose concentrations without finger pricking (98.1%), contact with a physician using mobile solutions and/or telemedicine (98.1%), and automation of insulin dosing (91.6%) and of calories/carbohydrates’ evaluation in meals (84.4%). Needs for telemedicine development, automation of insulin dosing and that the others help patients with diabetes management were more frequently reported by persons with: higher HbA1c, positive severe hypoglycemia history, concomitant chronic complications or diseases, and by those who were on insulin therapy. Conclusions. Although many diabetes technologies which meet the needs of patients with type 2 diabetes are already available, the study uncovers a high requirement for integrating them into disease management. The challenge pertains to implementation of the right technological solutions fulfilling needs of particular groups of patients and to helping them to embrace novelties into their daily lives.

Skin autofluorescence (sAF) represents tissue accumulation of advanced glycation end products (AGEs) and correlates with cardiovas-cular morbidity and diabetes risk.To assess sAF in Polish children without diabetes and to investigate whether sAF values in children with chronic diseases (but without glucose metabolism disorders) differ from sAF in healthy children.Children without diseases known to influence sAF results (diabetes, renal failure) and with HbA1c5.7% (39 mmol/mol) were includ-ed, and the total study group was divided into two subgroups: with and without chronic conditions. Skin autofluorescence was meas-ured with an AGE Reader (Diagnoptics BV, Groningen, Netherlands). Data were presented as medians; Mann-Whitney U-test, Kruskall Wallis test, and Spearman's correlation coefficients were used in statistical analyses.The study group included 86 children (41 girls; mean age 10.1 ±4.2 years). Median sAF was 1.20 AU (25th-75th centile: 1.06-1.30). There was a positive correlation between sAF and age (R = 0.37, p = 0.0005). Skin autofluorescence values were higher in children with chronic diseases than in healthy children (1.23 AU [25th-75th centile: 1.10-1.40], n = 51 vs. 1.16 AU [1.06-1.26], n = 36, p = 0.0272).To our knowledge we present the first data on sAF values in Polish children without glucose metabolism disorders. We suggest that larger, homogenous populations of different ages should be studied to determine if and which diseases affect sAF measurements, and to develop pediatric reference values for sAF. This will allow a wider use of sAF measurement in the assessment of cardiovascular risk in the paediatric population.Autofluorescencja skóry (skin autofluorescence – sAF) odzwierciedla zawartość końcowych produktów zaawansowanej glikacji (ad-vanced glycation end products – AGEs) w organizmie i wyniki jej pomiaru korelują z obecnością i nasileniem zaburzeń metabolizmu glukozy i z występowaniem chorób sercowo-naczyniowych.Ocena sAF u polskich dzieci oraz zbadanie, czy wartości sAF u dzieci z chorobami przewlekłymi (ale bez zaburzeń metabolizmu gluko-zy) różnią się od wartości sAF u dzieci zdrowych.Badaniami objęto dzieci z prawidłowym stężeniem hemoglobiny glikowanej [HbA1c5,7% (39 mmol/mol)]. Grupę badaną podzielono na dwie podgrupy: dzieci z chorobami przewlekłymi (z wykluczeniem chorób o znanym wpływie na sAF – cukrzyca, niewydolność nerek) oraz dzieci zdrowych. Pomiar sAF wykonywano w sposób nieinwazyjny, za pomocą urządzenia AGE Reader (Diagnoptics BV, Groningen, Holandia). Wyniki przedstawiono jako mediany; w analizie statystycznej wykorzystano test U Manna-Whitneya, test Kru-skalla Wallisa oraz współczynniki korelacji Spearmana.Grupę badaną stanowiło 86 dzieci (41 dziewczynek i 45 chłopców; średni wiek 10,1 ±4,2 roku) – 36 dzieci zdrowych i 51 ze schorze-niami przewlekłymi (u większości rozpoznano choroby alergiczne, choroby krwi, choroby tarczycy). Mediana sAF wynosiła 1,20 AU (25.–75. centyl: 1,06–1,30). Stwierdzono dodatnią korelację między sAF a wiekiem (R = 0,37, p = 0,0005). Wartości sAF były wyższe u dzieci z chorobami przewlekłymi niż u dzieci zdrowych [1,23 AU (25.–75. centyl: 1,10–1,40) vs 1,16 AU (1,06–1,26) p = 0,0272].Zgodnie z wiedzą autorów jest to pierwsze podsumowanie prezentujące wartości sAF u polskich dzieci w różnym wieku, bez zaburzeń metabolizmu glukozy. Wskazane są dalsze badania obejmujące większe, jednorodne populacje, które pozwolą ocenić, czy i jakie choroby wpływają na wyniki pomiaru sAF i umożliwią opracowanie wartości referencyjnych sAF dla polskiej populacji pediatrycznej. Pozwoli to na szersze zastosowanie pomiarów sAF w ocenie ryzyka sercowo-naczyniowego u dzieci.

The aim of this EMINENTS prospective, single-center, open-label, single-arm study was to evaluate the cumulative efficacy and safety of reduced doses of everolimus (maintenance therapy) in patients with tuberous sclerosis and subependymal giant cell astrocytoma (SEGA).Methods: The trial included 15 patients who had undergone at least 12 months of treatment with a standard everolimus dose. The dose of everolimus was reduced to three times a week, with a daily dose as in standard regimen. Data of 14 patients were analyzed. SEGA volume (SV) was evaluated at study entry and subsequent time points by an experienced radiologist. Adverse events (AEs) noted during maintenance therapy were compared to the AEs of standard dose period.Results: Patients were followed over a mean duration 58.37 months (95%CI: 45.95–70.78). The differences in SEGA volume between subsequent time points (0, 3, 6,12, 18, 24, 36, 48, and 60 months) were not statistically significant (p = 0.16). At the end of the study, 7 out of 10 patients had stable SEGA volume. No clinical symptoms of progression were observed in any patients. No patient or tumor-related risk factors of progression were identified. Regarding AEs, infections (stomatitis, bronchitis, diarrhea) and laboratory abnormalities (neutropenia, anemia, hyperglycemia) occurred less frequently during maintenance therapy compared to the standard dose regimen.Conclusions: Final results from EMINENTS study confirm that maintenance therapy with everolimus might represent a rational therapeutic option for patients TSC and SEGA after effective full dose treatment. It could be an option for patients who experienced everolimus-related AEs, instead of discontinuation of therapy. Careful evaluation of possible progression, especially concerning first six months of maintenance therapy should be advised.Clinical Trial Registration:www.drks.de, identifier DRKS00005584.

To evaluate correlation between the lateral ventricle ratio (LVR) and the risk of conservative treatment failure (CTF) among patients with acute subdural hematoma (ASDH) after non-severe traumatic brain injury (TBI), we retrieved from the hospital database and performed a retrospective analysis of 1339 cases with TBI treated during the 2008-2016 period.112 patients with ASDH, GCS≥ 9 and initial conservative treatment were enrolled. They were divided according to the final treatment method applied (surgical or conservative). Clinical and radiological data was evaluated. We used ROC curve analysis and multivariate logistic regression model to identify risk factors of CTF.LVR higher than 1.48 calculated on admission CT scans was the strongest predictor of CTF, with sensitivity of 78.9% and specificity of 93.5% (AUC: 0.774-0.994). LVR, prolonged prothrombin time and coexisting traumatic subarachnoid hemorrhage were independent risk factors.Despite limitations, study results support the view that patients after non-severe TBI with ASDH and with lateral ventricle asymmetry, defined as LVR 1.48, require surgical treatment. LVR seems to be indirect, but still the closest method to quantify intracranial compliance. Thus, in the selected group of patients without clinical symptoms of critically diminished compensatory reserve, LVR could indicate those who need a surgical decompression.

The aim of this study was to evaluate a cost-effective diagnostic strategy for identification of casual variants for tuberous sclerosis complex (TSC) in the Polish population and to correlate the genetic results with selected phenotypic features. Fifty-five patients, aged 3–44 years, with a clinical diagnosis of TSC were enrolled into the study. All patients received a three-step analysis: next generation sequencing screening (NGS), multiplex ligation-dependent probe amplification (MLPA) and deep sequencing. This multistep approach obtained positive results in 51/55 (93%) patients: of the 51 positives TSC1 variants were observed in 16 (31%) and TSC2 variants in 35 (69%); these included 13 novel variants and two patients with mosaicism. Four patients (7%) had no mutation identified (NMI). Among the TSC1 gene variants, there were five nonsense, four frameshift, three large deletions, two missense and two splicing variants. For the TSC2 gene, 11 were missense, eight splicing, six frameshift, four large deletions, two in-frame deletions and four nonsense variants. The patients with TSC2 changes had their clinical diagnosis of TSC at a younger age than those with TSC1 changes (one year vs three years, p = 0.041). The TSC2 group demonstrated a higher number of major symptoms per patient (p = 0.04). Subependymal giant cell astrocytoma with concomitance of other brain lesions was more common in patients with missense mutations in either gene (23% vs 0%, p = 0.02). Such a multistep molecular diagnostic strategy could increase the possibility of detecting causal variants for TSC and may allow detection of mosaicism at low levels. Missense pathogenic variants in TSC1 or TSC2 gene might be associated with a higher risk of brain lesions.

Background/aim Nivolumab is an immune checkpoint inhibitor with high antitumor activity in selected neoplasms. The aim of the study was to evaluate the efficacy and safety of nivolumab in pediatric patients with various types of highly malignant advanced tumors. Patients and methods Ten patients with a median age of 15.1 years were included in the study. The indications for treatment were: malignant skin melanoma (n=5), brain tumor (n=2), malignant melanoma of the brain (n=1), Hodgkin lymphoma (n=1) and soft tissue sarcoma (n=1). Results Complete disease remission was observed in 4 patients. Overall survival at 24 months from diagnosis for the entire group was 0.36. Two patients receiving combination therapy of nivolumab and ipilimumab did not achieve a remission. Adverse events of immunotherapy were observed in 4/10 patients. Conclusion Nivolumab is a promising option in pediatric advanced malignancies. Treatment with immunotherapy was relatively well tolerated, and emerging side-effects were manageable.

Clinical partial remission (CPR) in most patients with type 1 diabetes (T1D) is observed shortly after clinical diagnosis. Increasing body weight and impaired insulin sensitivity may play a role in the pathogenesis of CPR. Several cytokines can also participate in the development of insulin resistance.To evaluate the relationship between birth weight, body mass index, and the concentrations of IL-8 and Fetuin-A, and the presence of clinical partial remission in children at the T1D onset.The study group consisted of 134 children with a newly diagnosed T1D in whom the presence of CPR was evaluated in a further 2-year course of diabetes. The control group included 47 children without glucose tolerance disorders. The concentrations of IL-8 and Fetuin-A were determined by the ELISA method.CPR occurred in 75.34% of T1D patients. At T1D onset, higher values of BMI SDS in the remitters as compared to the patients without remission were observed. At the T1D onset, the concentrations of Fetuin-A (p=0.031) and IL-8 (p=0.042) were significantly higher in patients compared to those without CPR.Evaluation of Fetuin-A and IL-8 levels in patients with a newly diagnosed T1D can differentiate between patients with or without CPR.

Background: From 1983, standardized therapeutic protocols for pediatric acute myeloid leukemia (AML) based on the BFM group experience were introduced in Poland. We retrospectively analyzed the results of pediatric AML treatment in Poland from 1983 to 2019 (excluding promyelocytic, therapy-related, biphenotypic, and Down syndrome AML). Methods: The study included 899 children suffering from AML treated with the following: AML-PPPLBC 83 (1983–1993, n = 187), AML-PPGLBC 94 (1994–1997, n = 74), AML-PPGLBC 98 (1998–2004, n = 151), AML-BFM 2004 Interim (2004–2015, n = 356), and AML-BFM 2012 (2015–2019, n = 131). Results: The probability of three-year overall survival was 0.34 ± 0.03, 0.37 ± 0.05, 0.54 ± 0.04, 0.67 ± 0.03, and 0.75 ± 0.05, event-free survival was 0.31 ± 0.03, 0.34 ± 0.05, 0.44 ± 0.04, 0.53 ± 0.03, and 0.67 ± 0.05, and relapse-free survival was 0.52 ± 0.03, 0.65 ± 0.05, 0.58 ± 0.04, 0.66 ± 0.03, and 0.78 ± 0.05, respectively, in the subsequent periods. A systematic reduction of early deaths and deaths in remission was achieved, while the percentage of relapses decreased only in the last therapeutic period. Surprisingly good results were obtained in the group of patients treated with AML-BFM 2012 with unfavorable genetic abnormalities like KMT2A-MLLT10/t(10, 11)(p12, q23) and DEK-NUP214/t(6, 9)(p23, q24), while unsatisfactory outcomes were found in the patients with FLT3-ITD. Conclusions: The use of standardized, systematically modified therapeutic protocols, with the successive consideration of genetic prognostic factors, and advances in supportive care led to a significant improvement in AML treatment outcomes over the last 40 years.

In this study, we compare the prevalence of alcohol and cigarette use among Polish adolescents with type 1 diabetes mellitus (T1DM) (n = 209), aged 15-18 years, with that of a large cohort of their healthy peers, using standardized questionnaire used in the European School Survey Project on Alcohol and Drugs (ESPAD). The lifetime, previous year, and past 30-day prevalence of alcohol consumption was high among adolescents with T1DM but lower than in the controls (82.8 vs 92.0%, 71.7 vs 85.6%, and 47.5 vs 69.7%, respectively, p 10Although alcohol and cigarette consumption is lower than in controls, it is common among teenagers with type 1 diabetes, effecting metabolic control and causing the risk of acute diabetes complications. Better prevention strategies should be implemented in this group of patients in their early teen years. What is Known: • Substance use remains a significant cause of morbidity and mortality among teenagers with type 1 diabetes. • Current medical literature contains inconsistent data on the prevalence of alcohol and cigarette use among adolescents with type 1 diabetes, mostly due to methodological problems with conducting such surveys. What is New: • Methodological approach: we used a validated questionnaire from the European School Survey Project on Alcohol and Other Drugs (ESPAD) and compared the results to a large national control group of 12,114 healthy students who took part in ESPAD in 2011.

Objective Traumatic brain injury (TBI) remains a major cause of morbidity and mortality worldwide. The prognostic value of skull fracture (SF) remains to be clearly defined. To evaluate the need for neurosurgical intervention and determine the risk factors of conservative treatment failure (CTF), we retrieved from the hospital database the records of patients with SF after TBI. Methods We analyzed 146 consecutive patients (mean age: 49.8 ± 17.5 years) treated at the department of neurosurgery in a 5-year period. Clinical data, radiologic reports, and laboratory results were evaluated retrospectively. Results A total of 63% of patients were treated conservatively, 21.9% were operated on immediately, and 15.1% experienced CTF. Overall, 73.3% had a favorable outcome; the mortality rate was 13%. Intracranial bleeding occurred in 96.6% of cases, basilar SF in 61%, and cerebrospinal fluid (CSF) leak in 2.8%. The independent risk factors for outcome were Glasgow Coma Scale (GCS) score, age, and platelet count (PCT). The independent risk factors for CTF were epidural hematoma, subdural hematoma, mass effect, edema, international normalized ratio, PCT, mean platelet volume, and CSF leakage. The consensus decision tree algorithm used at the accident and emergency department indicated patients with no need for neurosurgical intervention with an accuracy of 91.7%, sensitivity of 88.9%, and featured the importance of mass effect, GCS, and epidural hematoma. Conclusions Tests included in the complete blood count appeared useful for predicting the course in patients with SF, although the most important factors were age and neurologic status, as well as radiologic findings. Our decision tree requires further validation before it can be used in everyday practice.

Background. The aim of the study was to explore the prevalence of illicit drug use in a group of Polish adolescents with type 1 diabetes (DM1) in comparison with a national cohort of their healthy peers.Methods. Two hundred and nine adolescents with DM1, aged 15–18 years, were studied in 2013 with an anonymous questionnaire prepared for the European School Survey Project on Alcohol and Other Drugs (ESPAD). The control group was a representative sample of 12114 students at the same age who took part in ESPAD in 2011. Metabolic control was regarded as good if self-reported HbA1c was Results. Lifetime prevalence of illicit drug use was lower among adolescents with DM1 than in the control group [58 (28%) versus 5524 (46%),p=10-5]. Cannabis preparations were the most frequently used substances [38 (18.3%) versus 3976 (33.1%),p=10-5], followed by tranquilizers, sedatives, and amphetamine. Lifetime and last 12-month use of cannabis were associated with poorer glycemic control (HbA1c ≥ 8%),p<0.01and 0.02, respectively.Conclusions. Adolescents with DM1 report using illicit drugs to a lesser extent than their healthy peers. The use of cannabis is associated with a poorer metabolic control in teens with DM1.

Background:The aim of the study was to compare the selected markers in children with and without partial clinical remission (CR) of newly diagnosed type 1 diabetes (T1D).Methods:The study group consisted of 186 patients (F/M; 87/99) at onset of T1D and 24 months of follow-up. Partial CR was defined as insulin requirement Results:Partial CR was observed in 115/186 (61.83%) of patients. At diagnosis body mass index standard deviation (BMI SDS) was higher among remitters than in non-remitters (p=0.0051) and remitters were younger (p=0.0029). In the follow-up a higher triglyceride concentration in non-remitters compared to remitters (p=0.0455) and a lower high density lipoprotein (HDL) cholesterol level (p=0.0119) were noticed.Conclusions:Younger age and higher BMI at diagnosis of T1D can predispose to partial CR in children. In patients with CR of T1D after 2 years of follow-up a lipid profile improvement is observed.