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BACKGROUND: Cystic fibrosis (CF) is a severe systemic disease that affects many aspects of patients' lives. It is known that the progression of the disease adversely affects lower and upper airways including the paranasal sinuses. However, its impact on sinus development in the pediatric population is not fully examined. The purpose of this study was to evaluate the development of the paranasal sinuses in a pediatric population with CF and compare it to a control group consisting of healthy children. METHODS: The results of computed tomography (CT) scans of children with the disease and the control group were evaluated. The study included 114 CT images of children in the study group and 126 images of healthy children aged 0-18 years. The volumes of maxillary, frontal, and sphenoid sinuses were analyzed. The obtained results were compared with those of the control group and analyzed statistically. RESULTS: The volume and the development of the paranasal sinuses in both groups increased with age, but statistically significant differences were found between the study and the control group. CONCLUSIONS: The obtained results provide valuable knowledge regarding the impact of the CF on sinuses development. Also, they may be important in understanding the progression of the disease and its influence on the quality and length of life of patients. The results may contribute to enhanced diagnostics and have implications for improving therapy for patients with chronic sinusitis associated with CF.
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Fibrose Cística , Seios Paranasais , Sinusite , Humanos , Criança , Fibrose Cística/complicações , Fibrose Cística/diagnóstico por imagem , Seios Paranasais/diagnóstico por imagem , Sinusite/diagnóstico por imagem , Sinusite/complicações , Seio Esfenoidal , Tomografia Computadorizada por Raios XRESUMO
Central diabetes insipidus (CDI) is a disorder in the pediatric population resulting from antidiuretic hormone deficiency. The excessive production of dilute urine characterizes it and manifests with polyuria, nocturia, and polydipsia. The diagnostics of CDI is often challenging, especially concerning the underlying condition of the disease. This article highlights the diverse clinical presentation of children with CDI and diagnostic difficulties among patients with polyuria and polydipsia. The article also reviews the etiology, symptoms, diagnostic workup, and management of CDI. We present 4 pediatric patients (aged 3-13.5 years) diagnosed with CDI of different etiology: 1 due to septo-optic dysplasia/optic nerve hypoplasia and 3 due to acquired processes such as Langerhans cell histiocytosis and germ cell tumor in 2 patients. Central diabetes insipidus was the first manifestation of a tumor or granuloma in all presented patients with acquired pathology. The patients sometimes need long-term follow-up to establish the proper final diagnosis.
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Background: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract, usually found in elderly adults. It is infrequent among pediatric patients and usually differs from adult-type disease in terms of histopathology and molecular features. Case Description: We describe the management of the disease in a 10-year-old female patient diagnosed with a GIST of the stomach. In total, she has undergone successively total tumor resection, unsuccessful imatinib treatment and subtotal gastric resection at relapse. The first genetic test from primary tumor confirmed KIT mutations in exons 13 and 17, while the repeated genetic screening using tumor sample from subtotal gastric resection revealed no KIT or platelet-derived growth factor receptor α (PDGFRA) genes mutations. Such dedifferentiation from adult type (thus KIT-mutated) into wild-type (without KIT or PDGFRA mutation) has not been reported so far to the best of our knowledge. Currently, the patient is observed, and no further pharmacological nor surgical treatment has been administered. Conclusions: The case underlines the importance of genetic profiling combined with non-standard diagnostics (both histopathological and radiological) due to the treatment efficacy prediction. We moreover emphasize the necessity to create worldwide standards on the diagnostics and treatment of GIST in pediatric patients that would include options of targeted therapies.
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Purpose: The aim of the study was to analyse magnetic resonance imaging (MRI) of paediatric patients referred because of back pain. Material and methods: The retrospective analysis included the medical records of 328 patients referred in 2020-2022 to the Department of Paediatric Radiology for spine examination. The criterion for inclusion in the analysed group was back pain as the dominant symptom. This symptom occurred in 20% (68 patients) of referrals for MRI examinations. The examination was performed with the 3T Magnetom Spectra. Results: In 68 patients aged 2 to 17 years, with back pain as the first diagnosis, 53% (36 patients - 16 girls and 20 boys) showed abnormalities. The rest of the tests were assessed as normal. Among the patients with an abnormal MR image, the largest group were children with degenerative changes diagnosed: 10 children (28%) aged 13-17 years. In 9 patients (25%) aged 2-16 years the final diagnosis qualified the patients to the group of oncological diagnoses. Another group of 7 (19%) patients, aged 6-14 years, comprised children diagnosed with inflammation. The group of 5 patients, aged 3-17 years, presented symptoms most likely related to the trauma. One 7-year-old boy was diagnosed with large calcifications within the intervertebral disc. Conclusions: Back pain, with accompanying neurological symptoms, should not be underestimated. Although in most clinical situations the MR image is normal, in the case of persistent symptoms and neurological abnormalities confirmed by the clinician, extending the diagnostics with MR imaging should be considered. This imaging can accelerate the correct diagnostic path or make a very precise diagnosis.
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Magnetic resonance imaging (MRI) of the chest is becoming more available in the detection and monitoring of early changes in lung function and structure in patients with cystic fibrosis (CF). The aim of this study was to assess the relationship between pulmonary function tests (PFT) and perfusion deficits in CF children measured by MRI. We performed a retrospective analysis of the perfusion lung MRI scans and the results of spirometry, oscillometry, body plethysmography, single-breath carbon monoxide uptake, and multiple-breath washout technique (MBW). There were statistically significant correlations between the MRI perfusion scores and MBW parameters (2.5% LCI, M1/M0, M2/M0), spirometry parameters (FEV1, FVC, FEF25/75), reactance indices in impulse oscillometry (X5Hz, X10Hz), total lung capacity (TLC) measured in single breath carbon monoxide uptake, markers of air-trapping in body plethysmography (RV, RV/TLC), and the diffusing capacity of the lungs for carbon monoxide. We also observed significant differences in the aforementioned PFT variables between the patient groups divided based on perfusion scores. We noted a correlation between markers of functional lung deficits measured by the MRI and PFTs in CF children. MRI perfusion abnormalities were reflected sooner in the course of the disease than PFT abnormalities.
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Lymphomas are the ninth most common malignant neoplasms as of 2020 and the most common blood malignancies in the developed world. There are multiple approaches to lymphoma staging and monitoring, but all of the currently available ones, generally based either on 2-dimensional measurements performed on CT scans or metabolic assessment on FDG PET/CT, have some disadvantages, including high inter- and intraobserver variability and lack of clear cut-off points. The aim of this paper was to present a novel approach to fully automated segmentation of thoracic lymphoma in pediatric patients. Manual segmentations of 30 CT scans from 30 different were prepared by the authors. nnU-Net, an open-source deep learning-based segmentation method, was used for the automatic segmentation. The highest Dice score achieved by the model was 0.81 (SD = 0.17) on the test set, which proves the potential feasibility of the method, albeit it must be underlined that studies on larger datasets and featuring external validation are required. The trained model, along with training and test data, is shared publicly to facilitate further research on the topic.
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Ataxia-telangiectasia (AT) is a multisystemic neurodegenerative inborn error of immunity (IEI) characterized by DNA repair defect, chromosomal instability, and hypersensitivity to ionizing radiation. Impaired DNA double-strand break repair determines a high risk of developing hematological malignancies, especially lymphoproliferative diseases. Poor response to treatment, excessive chemotherapy toxicities, and the need for avoiding exposure to ionizing radiation make the successful clinical management of patients with AT challenging for oncologists. We describe the favorable outcome of the LBCL with IRF4 rearrangement at stage III in a 7-year-old female patient diagnosed with AT. The patient was treated according to the B-HR arm of the INTER-B-NHL-COP 2010 protocol, including the administration of rituximab, cyclophosphamide, methotrexate, prednisone, etc. She presented excessive treatment toxicities despite individually reduced doses of methotrexate and cyclophosphamide. However, in the MRI there was no significant reduction in pathologic lymph nodes after three immunochemotherapy courses. Therefore, a lymph node biopsy was taken. Its subsequent histopathological examination revealed tuberculosis-like changes, though tuberculosis suspicion was excluded. After two following immunochemotherapy courses, PET-CT confirmed complete remission. From March 2022 onwards, the patient has remained in remission under the care of the outpatient children's oncology clinic.
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Ataxia Telangiectasia , Linfoma Difuso de Grandes Células B , Feminino , Humanos , Criança , Metotrexato/uso terapêutico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Rituximab/genética , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Ataxia Telangiectasia/tratamento farmacológico , Ataxia Telangiectasia/genética , Prednisona/uso terapêutico , Ciclofosfamida/uso terapêutico , Mutação , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Vincristina/uso terapêutico , Doxorrubicina/uso terapêutico , Proteínas Mutadas de Ataxia Telangiectasia/genéticaRESUMO
Children with cystic fibrosis (CF) suffer from chronic inflammation and recurrent pulmonary exacerbations (PEs). We aimed to test whether a specific miRNA could be associated with the occurrence of PE. We sequenced extracellular vesicle (EV)-derived miRNA in sputum (n= 20), exhaled breath condensate (EBC) (n= 11), and serum (n= 8) samples from pediatric patients during PE and the stable stage of CF. Four miRNAs: let-7c, miR-16, miR-25-3p and miR-146a, have been selected for validation in a larger group with reverse transcription quantitative real-time PCR (RT-qPCR) in sputum and serum, or droplet digital PCR (ddPCR) in EBC. Next-generation sequencing (NGS) differential expression analysis was done in Base Space, and the correlation between miRNAs expression and clinical data was calculated with Statistica. Functional annotation of selected miRNAs and their potential target genes was performed with miRDip and DAVID software. There were no differences in miRNA expression between stable and exacerbation in sputum and in serum. Validation of four selected miRNAs showed significant downregulation of miR-146a in serum. A panel of all four miRNAs (peripherally) was the best predictive model of exacerbation (p< 0.001, AUC = 0.96). Expression of airway miR-25-3p improved the diagnostic value of FEV1% pred and FVC% pred, while peripheral miR-146a improved the predictive model of C-reactive protein and neutrophilia.In silicoanalysis revealed a potential role for selected miRNAs in regulating processes associated with inflammation and tissue remodeling. We demonstrated that EVs contained in peripheral blood as well as local biomaterials can act as carriers for miRNAs with the diagnostic potential of predicting exacerbation in pediatric CF.
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Fibrose Cística , MicroRNAs , Humanos , Criança , MicroRNAs/genética , Fibrose Cística/genética , Testes Respiratórios , Pulmão , InflamaçãoRESUMO
Ataxia-telangiectasia (A-T) is a syndromic inborn error of immunity (IEI) characterized by genomic instability, defective reparation of the DNA double-strand breaks, and hypersensitivity to ionizing radiation disturbing cellular homeostasis. The role of imaging diagnostics and the conscious choice of safe and advantageous imaging technique, as well as its correct interpretation, are crucial in the diagnostic process and monitoring of children with A-T. This study aimed at defining the role of a radiologist in the early diagnosis of A-T, as well as in detecting and tracking disease complications associated with infections, inflammation, lymphoproliferation, organ-specific immunopathology, and malignancy. Based on our single-center experience, retrospective analysis of investigations using ionizing radiation-free techniques, ultrasound (US), and Magnetic Resonance Imaging (MRI), was performed on regularly followed-up 11 pediatric A-T patients, 6 girls and 5 boys, aged from 2 to 18 years, with the longest period of observation coming to over 13 years. Our attention was especially drawn to the abnormalities that were observed in the US and MRI examinations of the lungs, abdominal cavity, and lymph nodes. The abdominal US showed no abnormalities in organ dimensions or echostructure in 4 out of 11 children studied, yet in the other 7, during follow-up examinations, hepato- and/or splenomegaly, mesenteric, visceral, and paraaortic lymphadenopathy were observable. In 2 patients, focal changes in the liver and spleen were shown, and in one patient progressive abdominal lymphadenopathy corresponded with the diagnosis of non-Hodgkin lymphoma (NHL). The lung US revealed multiple subpleural consolidations and B line artifacts related to the interstitial-alveolar syndrome in 5 patients, accompanied by pleural effusion in one of them. The MRI investigation of the lung enabled the detection of lymphatic nodal masses in the mediastinum, with concomitant airway lesions characteristic of bronchiectasis and focal parenchymal consolidations in one A-T patient with chronic respiratory failure. This patient also manifested organomegaly and granulomatous liver disease in abdominal MRI examination. Our study shows that the use of modern US capabilities and MRI is safe and efficient, thereby serving as a recommended advantageous imaging diagnostic tool in monitoring children with IEI and DNA instability syndromes.
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Current prognostic classification of rhabdomyosarcoma in children requires precise measurements of the tumor. The purpose of the study was to compare the standard three-dimensional (3D) measurements with semi-automatic tumor volume measurement method concerning assessment of the primary tumor size and the degree of response to treatment for rhabdomyosarcoma in children. Magnetic Resonance Imaging data on 31 children with treated rhabdomyosarcoma based on the Cooperative Weichteilsarkom Studiengruppe (CWS) guidance was evaluated. Tumor sizes were measured by two methods: 3D standard measurements and semi-automatic tumor volume measurement (VOI) at diagnosis, and after 9 and 17/18 weeks of the induction chemotherapy. Response to treatment and prediction values were assessed. The tumor volume medians calculated using VOI were significantly higher in comparison with those calculated using the 3D method both during the diagnosis as well as after 9 weeks of the chemotherapy and during the 17-18th week of the treatment. The volume measurements based on the generalized estimating equations on the VOI method were significantly better than the 3D method (p = 0.037). The volumetric measurements alone can hardly be considered an unequivocal marker used to make decisions on modification of the therapy in patients with rhabdomyosarcoma.
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BACKGROUND: Granulomatous lymphocytic interstitial lung disease (GLILD) has been increasingly recognized in children affected with primary immunodeficiencies (PIDs). In this study, we aimed to better characterize the spectrum of pediatric PIDs coexisting with GLILD including clinical and immunological predictors, thoracic imaging findings, and histopathologic features. METHODS: We respectively reviewed records of six representative cases of children, three of them affected with common variable immunodeficiency (CVID) and three with syndromic immunodeficiencies, in whom a diagnosis of GLILD was established based on clinical, radiological, and histopathologic findings. Clinical and immunological predictors for GLILD were also analyzed in the patients studied. RESULTS: All the children with GLILD had a history of autoimmune phenomena, organ-specific immunopathology, and immune dysregulation. Defective B-cell maturation and deficiency of memory B cells were found in all the children with GLILD. The radiological and histopathological features consistent with the diagnosis of GLILD, granulomatous disease, and lymphoid hyperplasia, were accompanied by chronic airway disease with bronchiectasis in children with CVID and syndromic PIDs. CONCLUSIONS: Our study shows that both CVID and syndromic PIDs may be complicated with GLILD. Further studies are required to understand the predictive value of coexisting autoimmunity and immune dysregulation in the recognition of GLILD in children with PIDs.
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Imunodeficiência de Variável Comum , Doenças Pulmonares Intersticiais , Criança , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico , Granuloma , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Células B de Memória , RadiografiaRESUMO
The International Neuroblastoma Risk Group Staging System (INRGSS) is based on the age of patients and preoperative imaging, with attention paid to whether the primary tumor is affected by one or more of specific image-defined risk factors (IDRFs). This publication presents a 2.5-year-old boy with neuroblastoma who had an accidental ligation of the celiac trunk during tumor resection. The consequences of this complication were pancreatic and spleen ischemia and necrosis, ischemia, and perforation of the common bile duct, gallbladder, stomach, and duodenum. The aim of this publication was to highlight the great role of the radiologist in determining the indications for neuroblastoma tumor removal, especially with current vascular IDRFs, and to show how the radiologist's insightful approach can save the patient from irreversible complications.
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Imageamento por Ressonância Magnética , Neuroblastoma , Pré-Escolar , Humanos , Masculino , Estadiamento de Neoplasias , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/patologia , Neuroblastoma/cirurgia , Radiologistas , Fatores de Risco , Tomografia Computadorizada por Raios XRESUMO
Infantile choriocarcinoma is an extremely rare disease. We present a case study of a 1-month-old male with choriocarcinoma diagnosed simultaneously with his mother. On admission to hospital, the disease was very advanced and massive progression and multi-organ failure caused the death of the patient despite the implemented treatment. It was too late to save the child's life, but early enough to save his mother. The authors believe that the serum levels of hCG should be determined in every newborn with anemia and liver tumor, especially when the mother has a positive history of miscarriage.
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Anemia , Coriocarcinoma , Neoplasias Hepáticas , Neoplasias Uterinas , Criança , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Mães , GravidezRESUMO
PURPOSE: Horner syndrome (HS) manifests in unilateral ptosis, miosis, enophthalmos, and anhedonia. It is most commonly caused by trauma or surgical procedures, but can also occur in pediatric patients as a result of tumors, especially neuroblastoma (NBL). The objective of this study was to analyze the incidence of HS in patients diagnosed with NBL. METHODS: A retrospective analysis of data collected at the Department of Pediatric Oncology, Hematology, and Transplantology from 2004 to 2019 was performed. The study group included 119 patients younger than 18 years old, with 62 girls and 57 boys. All of them were diagnosed with a neuroblastic tumor. RESULTS: Among the 119 patients, eight children (6.72%) were diagnosed with HS associated with NBL. Three of these patients presented to the clinic with HS, whereas HS developed after the surgical procedure to remove the tumor in four patients. The adrenal gland was the most frequent localization of the tumor. However, HS occurred more frequently in patients with mediastinum tumors. As a presenting symptom, HS occurred in 2 of 11 cases (18.18%) with mediastinum localization. All of the patients with HS were younger than 2 years old. CONCLUSION: Investigation of the cause of isolated HS is crucial because it can be the first symptom of NBL. However, the surgical procedure itself increases the risk of HS as a complication of NBL treatment.
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Blefaroptose , Síndrome de Horner , Neuroblastoma , Adolescente , Criança , Pré-Escolar , Feminino , Síndrome de Horner/epidemiologia , Síndrome de Horner/etiologia , Humanos , Incidência , Masculino , Neuroblastoma/complicações , Neuroblastoma/epidemiologia , Estudos RetrospectivosRESUMO
Ataxia-telangiectasia (A-T) is an autosomal recessive disorder characterized by neurodegeneration, combined immunodeficiency, and oculocutaneous telangiectasia. The hyper-IgM phenotype of A-T, correlating with a class-switch recombination defect, IgG and IgA deficiency, T helper and B cell lymphopenia, immune dysregulation, proinflammatory immune response, autoimmune disease, and a high risk of lymphomagenesis. Progressive liver disease is a hallmark of classical A-T with the hyper-IgM phenotype and manifests as non-alcoholic hepatic steatosis and fibrosis. We report a case of a 17-year-old male A-T patient, in whom a progressive granulomatous liver disease with portal hypertension, has led to massive splenomegaly and hypersplenism, metabolic liver insufficiency, bleeding from esophageal varices and pancytopenia. In this patient, an unusual severe disease course with a highly variable constellation of A-T symptomatology includes granulomatous skin, visceral, and internal organs disease with liver involvement. The liver disease is associated with the hyper-IgM immunophenotype and escalating neurodegeneration, creating a vicious circle of immune deficiency, permanent systemic inflammatory response, and organ-specific immunopathology.
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Patients with a Wilms tumor are often admitted to the hospital accidentally, with an abdominal mass causing asymmetry of the abdominal wall. Hypertension accompanying a Wilms tumor occurs in about 10-27% of children, but cardiomyopathy associated with a Wilms tumor is very rarely described. This publication presents a case of a 9-month-old girl with a bilateral Wilms tumor accompanied by dilated cardiomyopathy since her initial cancer diagnosis, as well as her off-protocol treatment. The severe condition of the child forced the application of off-protocol treatment, i.e., accelerated resection of a larger tumor, which enabled the improvement of heart performance and made subsequent therapy possible. In the course of the presented treatment, a gradual normalization of cardiac ventricular function and contractility was observed. In conclusion, a massive abdominal tumor associated with abdominal compartment syndrome compromised the functioning of the cardiovascular system in the young child. Therefore, earlier removal of Wilms tumors in patients with heart failure should be considered. This may result in the improvement of cardiovascular function and the possibility of further therapy.
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Cardiomiopatia Dilatada , Neoplasias Renais , Tumor de Wilms , Criança , Feminino , Humanos , Lactente , Neoplasias Renais/cirurgia , Masculino , Função Ventricular Esquerda , Vincristina , Tumor de Wilms/cirurgiaRESUMO
INTRODUCTION: The development of granulomas is a well-recognized manifestation of immunodeficiency in ataxia-telangiectasia (A-T), resulting from lymphocyte developmental abnormalities, impaired immunosurveillance, and inappropriate innate immune response-driven inflammation. AIM: To better understand pathological and immunological phenomena involved in development of cutaneous and visceral granulomatosis observable in patients with ataxia-telangiectasia. MATERIAL AND METHODS: We retrospectively reviewed medical records of eight A-T children, aged from 2 to 13 years, with regard to clinical, immunological and histopathological features of cutaneous and visceral granulomatosis. RESULTS: In four out of eight A-T patients studied, cutaneous granulomas clinically presented as skin nodules and ulcerated erythematous plaques disseminated on the face, and on trauma-prone areas of upper and lower extremities. Visceral granulomatosis had a severe clinical course and involved the lungs, the spleen, the liver and the larynx. Histologically, cutaneous and laryngeal granulomas showed extensive cellular infiltrations containing T lymphocytes with predominating CD8+ phenotype and with CD68+ histiocytes. The immunological profile with the hyper-IgM phenotype, markedly reduced numbers of B and naive CD4+ and CD8+ T cells with predominating IgM-only memory B cells and skewed repertoire of a T cell receptor was observable in patients with skin and visceral granulomatosis. CONCLUSIONS: In the setting of combined immunodeficiency in A-T, cutaneous and systemic granulomatosis reflects a granulomatous reaction pattern, as a result of inappropriate immune regulation.
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MicroRNAs are small non-coding RNAs that regulate immune response and inflammation. We assumed that miRNAs may be involved in the immune response during cystic fibrosis pulmonary exacerbations (CFPE) and that altered expression profile in the airways and blood may underlie clinical outcomes in CF pediatric patients. METHODS: We included 30 pediatric patients diagnosed with cystic fibrosis. The biologic material (blood, sputum, exhaled breath condensate) was collected during pulmonary exacerbation and in stable condition. The miRNA expression profile from blood and sputum (n = 6) was done using the next-generation sequencing. For validation, selected four miRNAs were analyzed by qPCR in exosomes from sputum supernatant and exhaled breath condensate (n = 24). NGS analysis was done in Base Space, correlations of gene expression with clinical data were done in Statistica. RESULTS: The miRNA profiling showed that four miRNAs (miR-223, miR-451a, miR-27b-3p, miR-486-5p) were significantly altered during pulmonary exacerbation in CF patients in sputum but did not differ significantly in blood. MiRNA differently expressed in exhaled breath condensate (EBC) and sputum showed correlation with clinical parameters in CFPE. CONCLUSION: MiRNA expression profile changes in the airways during pulmonary exacerbation in CF pediatric patients. We suggest that miRNA alterations during CFPE are restricted to the airways and strongly correlate with clinical outcome.
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AIM: The aim of the study was to assess long-term consequences of central nervous system (CNS) prophylaxis in patients with high-risk ALL (HR-ALL) treated according to ALL IC-BFM 2002 and to compare observed abnormalities in patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) with those who received only prophylactic CNS irradiation (12 Gy) and with control group. PATIENTS AND METHODS: We studied 29 patients with HR-ALL in CR1 after treatment according to protocol ALL IC-BFM 2002 (14 with allo-HSCT conditioned with fractionated total body irradiation [FTBI] and 15 without HSCT) and 16 children with newly diagnosed ALL (control group). The median time from therapy completion to evaluation was 5 years. To assess brain status, volumetric T1-weighted sequences of magnetic resonance imaging were used. Neuropsychological assessment based on battery neuropsychological tests. RESULTS: Transplanted patients had significantly lower volumes of white and gray matter (P = .048 and P < .001) and also of subcortical structures, including the thalamus (P < .001), the hippocampus (P = .007), the putamen (P = .011), the globus pallidus (P = .001), and the accumbens (P < .001). In addition, these patients had generally lower cognitive performance, especially in vocabulary (P = .011), visuospatial ability (P = .047), executive functions and attention (P = .034; P = .002; P = .048), and processing speed (P = .049 and P = .037). The thalamus volume is correlated with neuropsychological performance in verbal functions (P < .001), executive functions (P < .001 and P = .024), and processing speed (P < .001). CONCLUSIONS: In pediatric patients treated for ALL, FTBI-based preparative regimen preceding allo-HSCT causes reduction of subcortical structure volumes and decline in cognitive performance. The observed long-term structural and functional CNS sequelae are significantly more pronounced in transplanted HR-ALL patients than in those treated with prophylactic CNS- radiotherapy only.
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Encéfalo/patologia , Disfunção Cognitiva/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Irradiação Corporal Total/efeitos adversos , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Disfunção Cognitiva/patologia , Terapia Combinada , Feminino , Seguimentos , Humanos , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Prognóstico , Transplante HomólogoRESUMO
BACKGROUND: In the postnatal period, some sexual dimorphism is observed from the moment of birth, but it is most pronounced from puberty. The aim of the study based on CT images was to assess possible sexual dimorphism of the maxillary sinus in children aged 0-18. METHODS: The retrospective analysis of CT images of the head (assessed as normal by radiologists) of 170 patients aged 0-18 (85 females and 85 males) was performed. The maxillary sinuses of every patient were bilaterally measured in three planes. Three diameters were obtained: maximum transverse (horizontal) diameter (the maxillary sinus width, MSW); maximum vertical diameter (the maxillary sinus height, MSH) and maximum antero-posterior diameter (maxillary sinus length, MSL). The automatic CT image segmentation algorithm was used (Syngo Via for Oncology, Siemens) to establish maxilla retrospective analysis of CT images of the head (assessed as normal by radiologists) of 170 patients aged 0-18 (85 females and 85 males) was performed. The maxillary sinuses of every patient were bilaterally measured in three planes. Three diameters were obtained: maximum transverse (horizontal) diameter (the maxillary sinus width, MSW); maximum vertical diameter (the maxillary sinus height, MSH) and maximum antero-posterior diameter (maxillary sinus length, MSL). The automatic CT image segmentation algorithm was used (Syngo Via for Oncology, Siemens) to establish maxillary sinus volume (MSV). The coefficient of variation used in the study allowed the biological variation between sexes to be observed. RESULTS AND CONCLUSIONS: The sexual dimorphism of maxillary sinus parameters is variable and depends on the phase of ontogenesis. All investigated parameters of maxillary sinuses are larger in males at 2-3 years (except for the length, which is larger in females by the end of three years, between six and nine years and after 15 years). The sexual dimorphism is less evident during the first year of age, and the most evident between 15 and 16 years. During the first two years, females are dominant with all parameters being larger than in males. The maxillary sinus length is developmentally the most stable parameter of maxillary sinuses, because throughout the investigated ontogenesis, the sexual differences are very similar. The most evident sexual dimorphism is observed in the volume of the maxillary sinus.