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1.
Mediators Inflamm ; 2020: 5696185, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32308556

RESUMO

Changes in the liver and bile ducts observed in patients diagnosed with cystic fibrosis result from inflammatory processes as well as fibrosis, remodeling, apoptosis, and cholestasis. As a consequence, portal hypertension, cirrhosis, and hepatic failure may develop. So far, the complexity of these processes has not been elucidated. Study Objectives. The aim of the study was to evaluate the selected parameters of hepatitis and fibrosis (Fibrotest, Actitest, and APRI) in patients diagnosed with cystic fibrosis. Material and Methods. The study included 79 patients with cystic fibrosis, aged 1 to 20 years (mean age 9.8 years), 49 girls (62%) and 30 boys (38%). The analysis involved the following: age, sex, clinical manifestations, laboratory tests evaluating pancreas function, parameters of liver damage, and cholestasis. Fibrotest, Actitest, and APRI were performed in all subjects. Results. Elevated parameters of hepatic cell damage (hypertransaminasemia) were found in 31/79 (39.2%) patients, while abnormal cholestasis parameters in 21/79 (26.6%). The abnormal results of Fibrotest were reported in 15% of patients (12/79), while of Actitest in 10% (8/79). In contrast, elevated APRI values were found in only 7.6% (6/79) of subjects. There was a statistically significant correlation between APRI and age (higher values were observed in younger children) and between Fibrotest and Actitest and pancreatic insufficiency (higher values were found in subjects without this abnormality). Moreover, Fibrotest values were significantly higher in girls. There was no correlation between Fibrotest, Actitest, and APRI values and the type of mutation. Conclusion. It appears that Fibrotest may be used as an early marker of liver fibrosis in patients with cystic fibrosis. Increased APRI values were only found in subjects with advanced hepatic lesions, most often in the form of portal hypertension.


Assuntos
Fibrose Cística/imunologia , Fibrose Cística/metabolismo , Inflamação/imunologia , Inflamação/metabolismo , Cirrose Hepática/imunologia , Cirrose Hepática/metabolismo , Fígado/imunologia , Fígado/metabolismo , Adolescente , Adulto , Biomarcadores/metabolismo , Criança , Pré-Escolar , Colestase/imunologia , Colestase/metabolismo , Feminino , Humanos , Lactente , Masculino , Adulto Jovem
2.
Mediators Inflamm ; 2018: 3040346, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30057485

RESUMO

INTRODUCTION: Iron deficiency is common in patients with cystic fibrosis. Conventional iron status markers are often abnormal in patients with CF, reflecting inflammation and/or infection, rather than actual iron stores. The aim was to evaluate serum hepcidin levels against selected iron status markers, assuming that hepcidin may be a more sensitive indicator of iron management in patients with active inflammation, such as those with CF. MATERIAL AND METHODS: 46 children with cystic fibrosis and 31 healthy controls were enrolled. Hepcidin concentration was evaluated, along with the following other blood assays: full blood count, Fe, ferritin, transferrin, TIBC, liver markers, and CRP. RESULTS: Higher ferritin and CRP levels as well as lower TIBC levels significantly predicted hepcidin levels in the study group, control group, and the entire sample. There was no significant difference in hepcidin levels between the patients and controls. Children with exacerbations had significantly higher hepcidin levels than those with stable disease. These findings support the serum hepcidin level as useful in assessing iron status in children with cystic fibrosis. It may also be useful in early detection and monitoring of treatment of exacerbations.


Assuntos
Fibrose Cística/sangue , Hepcidinas/sangue , Ferro/sangue , Adolescente , Biomarcadores/sangue , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Ferritinas/sangue , Humanos , Lactente , Inflamação , Masculino , Prevalência , Curva ROC , Sensibilidade e Especificidade
3.
Postepy Dermatol Alergol ; 34(1): 52-59, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28261032

RESUMO

INTRODUCTION: Eosinophilic colitis, which is a rare form of eosinophilic gastrointestinal diseases, occurs as primary and secondary allergic eosinophilic colitis of the gastrointestinal tract infection, inflammatory bowel disease, celiac disease, and vasculitis. The diagnosis is based on a significant amount of eosinophils in the inflammatory infiltrate of the colon wall. AIM: To analyze the clinical picture taking into account comorbidities and endoscopic picture in children with eosinophilic colitis. MATERIAL AND METHODS: The test group consisted of 43 children, the average age - 12.1 years diagnosed with eosinophilic colitis (according to the Whitington scale) hospitalized in the Gastroenterology Unit, Department of Pediatrics of the Medical University of Silesia in Katowice. Testing for food allergies, celiac disease, inflammatory bowel disease, gastrointestinal diseases and parasitic diseases was performed in the group of children and the analysis concerned the intensity of eosinophilic infiltration of the colon mucosa with the severity of clinical symptoms, endoscopic picture, the presence of inflammatory bowel disease, and food allergy. RESULTS: Half of the tested children suffered from isolated eosinophilic colitis but the rest of them had eosinophilic infiltrate with inflammatory bowel disease more often, however, the Crohn's disease. The endoscopic image was uncharacteristic, and grade III in the Whitington scale was predominant in the histopathological examination, in most cases located in the entire large intestine. The higher level of total IgE was found in less than half of the patients and it did not correlate with the severity of eosinophilic infiltration. It was shown that the severity of eosinophilic infiltration correlated with exacerbation of clinical symptoms, endoscopic image, and the presence of inflammatory bowel disease. The higher level of antibodies of ASCA and ANCA was found in approximately 20% of the children with isolated eosinophilic colitis and 63% of children with Crohn's disease. CONCLUSIONS: The higher concentration of total IgE in less than half of the patients with eosinophilic colitis indicates the need for improving allergy diagnosis also in terms of IgE-independent allergy. The presence of higher levels of antibodies of ASCA and ANCA in some of the patients with isolated eosinophilic colitis indicates the need for further observation for the occurrence of inflammatory bowel disease.

4.
J Pediatr Hematol Oncol ; 38(8): e298-e302, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-26989913

RESUMO

Primary heart tumors are extremely rare, constituting approximately 0.02% of all malignancies. Inflammatory myofibroblastic tumor (IMT) constitutes <5% of primary heart tumors. Until now, IMT of the heart has been described in 21 infants below 1 year of age. Its etiology remains unknown. IMT usually develops within the right atrial and ventricular endocardium. The main clinical symptoms reported in the affected infants involved increasing respiratory failure, cyanosis, and heart murmurs. Histopathologically, IMT is characterized by the myofibroblast proliferation with inflammatory infiltrates composed of plasmocytes, lymphocytes, and histiocytes. Tumor resection is the treatment of choice in IMT. Such tumor location is associated with the high risk of perioperative failure. Steroid therapy and chemotherapy is reported in the literature as a nonsurgical treatment alternative. Here, we present a review of clinical symptoms, diagnostic and treatment options, based on published case reports of IMT in infants, including our 11-month-old patient with IMT located within the pericardium.


Assuntos
Neoplasias Cardíacas/patologia , Miofibroblastos/patologia , Humanos , Lactente , Inflamação/patologia , Masculino , Esteroides/uso terapêutico , Tomografia Computadorizada por Raios X
5.
Childs Nerv Syst ; 32(8): 1431-40, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27325303

RESUMO

AIM: The aim of the study was to evaluate renal function and to assess the usefulness of the following nephrotoxicity markers: cystatin C (CYS C), beta-2 microglobulin (B2MG) and neutrophil gelatinase-associated lipocalin (NGAL) in 38 (18 girls, 20 boys) children previously treated for central nervous system malignancy. MATERIAL: Median age at evaluation was 13.7 years (range 2.1-22 years). The mean follow-up time after the completion of chemotherapy was 3.2 years (range 0.16-6.5 years). RESULTS: Subclinical chronic kidney disease (estimated glomerular filtration rate: eGFR 90-60 ml/min/1.73 m(2)) was found in 22 patients (58 %), while renal insufficiency (eGFR 30-60 ml/min/1.73 m(2)) was found in six children (16 %). It has been demonstrated statistically significant negative correlation between the eGFR and cystatin C concentration (p < 0.0001) and eGFR and beta-2 microglobulin concentration (p < 0.02). Conversely, there was no correlation between eGFR and NGAL. Thirteen children (34 %) developed drug-induced tubulopathy: decreased tubular reabsorption of phosphate (TRP) and renal tubular threshold for phosphate (Tmp/GFR). CONCLUSION: Children treated for CNS tumours often develop drug-induced chronic renal disease, involving the glomeruli and/or renal tubules. Cystatin C and beta-2 microglobulin seemed to be good markers for chronic kidney damage in these patients, which is probably not true for NGAL.


Assuntos
Antineoplásicos/efeitos adversos , Nefropatias/induzido quimicamente , Nefropatias/diagnóstico , Adolescente , beta-Globulinas/metabolismo , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Criança , Pré-Escolar , Cistatina C/metabolismo , Feminino , Seguimentos , Taxa de Filtração Glomerular , Humanos , Nefropatias/metabolismo , Lipocalina-2/metabolismo , Masculino , Estatísticas não Paramétricas , Adulto Jovem
6.
Dev Period Med ; 19(1): 92-7, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26003074

RESUMO

UNLABELLED: Mutation of the SERPINA1 gene is present in about 2% of patients with cystic fibrosis but is more common and accounts for about 5% in patients with cystic fibrosis and co-existing liver lesions. The SERPINA1 gene is responsible for the synthesis of a serine protease inhibitor. The protein related with this gene is accumulated within the endoplasmic reticulum of hepatocytes causing their damage, inflammation and cirrhosis. The aim was to assess the presumable effect of the SERPINA1 mutation gene in patients with diagnosed cystic fibrosis on damage to the liver and/or cholestasis. MATERIAL AND METHOD: The analysis included 30 children, 13 girls (43.3%) and 17 boys (56.6%), aged from 6 months to 18 years (the average age was 5.5 years) with diagnosed cystic fibrosis. All the patients have undergone a genetic test of the mutation of the SERPINA1 gene. The analysis included age, sex, clinical symptoms, type of mutation of the CFTR protein, abnormalities in laboratory tests (the activity of aminotransferases, GGTP, alkaline phosphatase , protein, the indicator of acid steatocrit, the rate of APRI) and abdominal ultrasonography. RESULTS: Symptoms of damaged liver were concluded in 9/30 patients (30%) with diagnosed cystic fibrosis. Most commonly observed were increased activities of aminotransferases in 9/30 patients (30%) and of gamma glutamyl transferase in 6/30 (20%) of the assessed patients. In 4/30 patients the abdominal ultrasonography revealed an enlarged liver and increased echogenicity. Mutation within the SERPINA1 gene was observed only in 1/30 patients (3.3%) with diagnosed cystic fibrosis. As far as the patient is concerned, currently the activities of aminotransferases, GGTP and AF are normal, but there has been a considerable increase in the intensity of symptoms from the respiratory system. No corelation between the mutation of the SERPINA1 gene and clinical symptoms, type of mutation of the CFTR protein, laboratory results of the functions and damage to the liver and the abdominal ultrasonography was observed. CONCLUSIONS: We did not find a more frequent occurrence of the SERPINA1 gene mutation in children with cystic fibrosis and coexisting features of damaged liver and cholestasis. The obtained results suggest the contribution of other than SERPINA1 gene mutation factors responsible for the development of changes in the liver in patients diagnosed with cystic fibrosis. The studies on the subject should be extended and performed on a larger group of patients. .


Assuntos
Colestase/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/complicações , Fibrose Cística/genética , Hepatopatias/genética , Mutação , alfa 1-Antitripsina/genética , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
7.
Dev Period Med ; 19(1): 25-31, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26003067

RESUMO

INTRODUCTION: Neonatal cystic fibrosis screening contributes to an early diagnosis of cystic fibrosis and to implementing appropriate therapeutic management. Long-standing screening tests have made it possible to identify a group of newborns in whom the diagnosis was ambiguous and required further specialised tests. AIM: The aim is to present cases of patients with a positive result of newborn screening for cystic fibrosis who were found to be carriers of the mutation in both alleles, however the lack of clinical symptoms and correct sweat testing values did not lead doctors to diagnosing cystic fibrosis and by the same token implementing the treatment. MATERIAL AND METHODS: The analysis encompassed a group of 22 infants and children 3 months to 3 years of age, in whom, in spite of a positive result of newborn screening for cystic fibrosis and the presence of 2 mutations in the CFTR gene, the diagnosis of cystic fibrosis was not made, and appropriate treatment was not administered because of diagnostic doubts (due to correct concentration of chlorides in sweat, correct IRT level and lack of clinical signs of cystic fibrosis). The control group consisted of 55 children treated in our centre, in whom neonatal screening for cystic fibrosis was positive and the diagnosis was confirmed by genetic testing, sweat chloride testing and IRT concentration. RESULTS: There were no differences in birth body weight between the groups. The differences in chlorideion levels in sweat secretion tests and mean IRT values were statistically significant and were: 97.5 for the control group and 26.4 for the test group. At the present time there are no clinical symptoms to give a diagnosis of cystic fibrosis and start treatment in the test group. CONCLUSIONS: Newborn screening contributes not only to an early diagnosis of cystic fibrosis but also to CFTR-related metabolic syndromes (CRMS), which is a phenomenon requiring further observation. This fact constitutes a definite psychological problem for the parents of these patients. .


Assuntos
Fibrose Cística/diagnóstico , Triagem de Portadores Genéticos , Testes Genéticos , Triagem Neonatal , Pré-Escolar , Cloretos , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Diagnóstico Precoce , Humanos , Lactente , Recém-Nascido , Mutação , Suor/química
8.
Childs Nerv Syst ; 30(1): 131-6, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23780403

RESUMO

PURPOSE: To assess the nutritional status in children with central nervous system (CNS) tumours, including concentration of leptin, the neuropeptide responsible for regulation of energetic homeostasis in an organism. METHOD: The studied group comprised 44 children with brain tumours, aged (4.02-18.7). In all children during the whole therapy (from the start to the period of 1 year and more after the end of therapy), a number of standard deviations (SDs) for the body mass index (SDS BMI) was derived from anthropometric measurements. Concentrations of leptin were assayed simultaneously. RESULT: The lowest values of the anthropometric indices were found in children during the maintenance therapy. Concentrations of leptin in patients with malignant CNS tumours and significant undernutrition were slightly greater as compared to patients presenting normal nutritional status; however, without statistical significance. CONCLUSION: In children with tumours of the central nervous system, there are quantitative disorders of the nutritional status which correlate with the period of the treatment. The most significant disorders in the nutritional status are observed during maintenance chemotherapy. There was no statistically significant correlation between the concentration of leptin and nutritional status in children with malignant brain tumours during the course of treatment and after its completion.


Assuntos
Índice de Massa Corporal , Neoplasias Encefálicas/sangue , Neoplasias Encefálicas/diagnóstico , Leptina/sangue , Estado Nutricional/fisiologia , Adolescente , Antineoplásicos/uso terapêutico , Biomarcadores/sangue , Neoplasias Encefálicas/tratamento farmacológico , Criança , Feminino , Humanos , Masculino , Resultado do Tratamento
9.
J Pediatr Hematol Oncol ; 34(4): e142-4, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22258346

RESUMO

Rhabdomyolysis refers to a number of clinical and biochemical symptoms, which result from the destruction of skeletal muscles. The following triad of symptoms is considered typical: myalgia, muscle weakness, and dark urine. The most common reasons for rhabdomyolysis in children are infections. It has also been reported that rhabdomyolysis may be caused by chemotherapy drugs. The most difficult complication of rhabdomyolysis is renal failure. The authors present a 17-year-old boy diagnosed with Ewing sarcoma and a 16-year-old boy suffering from acute leukemia, both with rhabdomyolysis developed in the course of infection caused by Clostridium difficile, and drug-induced neutropenia.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Clostridioides difficile , Enterocolite Pseudomembranosa , Neutropenia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Rabdomiólise , Sarcoma de Ewing/tratamento farmacológico , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Enterocolite Pseudomembranosa/induzido quimicamente , Enterocolite Pseudomembranosa/diagnóstico , Enterocolite Pseudomembranosa/patologia , Enterocolite Pseudomembranosa/terapia , Humanos , Masculino , Neutropenia/induzido quimicamente , Neutropenia/diagnóstico , Neutropenia/microbiologia , Neutropenia/patologia , Neutropenia/terapia , Rabdomiólise/induzido quimicamente , Rabdomiólise/diagnóstico , Rabdomiólise/microbiologia , Rabdomiólise/patologia , Rabdomiólise/terapia
10.
Mediators Inflamm ; 2012: 608249, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22665952

RESUMO

INTRODUCTION: The aim of the study was to assess the usefulness of the FC measurement in children with various types of IBD and relation to the disease activity. PATIENTS AND METHODS: 91 patients (49 boys: 53.85% and 42 girls: 46.15%, mean age: 13.38 years, range 6-18 years) were included in the analysis. Patients were divided into the groups: B1-24 children with CD, B2-16 patients with UC, and a group comprising 31 children with other types of colitis; the control group (K) comprised 20 healthy children. FC was assayed by ELISA method, using Phical test (Calpro). RESULTS: The mean faecal calprotectin concentrations were higher in children with CD and UC as compared to healthy controls, patients with eosinophilic, lymphocytic, and nonspecific colitis. A positive correlation was observed between FC concentrations and the disease activity (the PCDAI scale, the Truelove-Witts Scale, and the endoscopic Rachmilewitz Index). CONCLUSION: It seems that the FC concentrations can be a useful, safe, and noninvasive test in children suspected for IBD, since FC concentration is higher in children with CD and UC than in patients with other inflammatory diseases.


Assuntos
Fezes/química , Doenças Inflamatórias Intestinais/metabolismo , Complexo Antígeno L1 Leucocitário/metabolismo , Adolescente , Criança , Colite Ulcerativa/metabolismo , Colite Ulcerativa/patologia , Feminino , Humanos , Doenças Inflamatórias Intestinais/patologia , Complexo Antígeno L1 Leucocitário/análise , Masculino
11.
Wiad Lek ; 65(2): 77-83, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23289251

RESUMO

INTRODUCTION: The aim of this study was to evaluate the prevalence of selected risk factors of weight deficiency in children with chronic metabolic diseases. MATERIAL AND METHODS: The study group involved 160 children, from 2 months to 15 years (mean age 3.14 years), with diseases of the nervous system and body weight deficiency. According to the type of neurological disease the following groups of patients were separated: static encephalopathies, progressive encephalopathies, disorders of mental development of undetermined etiology, genetically determined diseases. As the exponent of malnutrition, z-score of weight-for-age standards was used. An inclusion criterion for the study group was z-score of weight-for-age < - 2SD. The analysed risk factors of body weight deficiency were: mode of feeding children, neurological disorders, oral motor dysfunction, diseases of other organs, gastrointestinal motility disorders (oral cavity, esophagus, intestines) and type of nutritional therapy. RESULTS: The most advanced malnutrition was in children with progressive encephalopathies and genetically determined diseases. Seizures and muscular hypotonia were most common neurological disorders. Oral motor dysfunctions were observed in 40% of patients. CONCLUSIONS: Malnutrition in children with neurological disorders is associated mainly with neurological deficits. In this group of children monitoring of somatic development and early nutritional intervention are necessary.


Assuntos
Transtornos da Nutrição Infantil/prevenção & controle , Métodos de Alimentação/classificação , Transtornos da Alimentação e da Ingestão de Alimentos/terapia , Transtornos da Nutrição do Lactente/prevenção & controle , Doenças do Sistema Nervoso/epidemiologia , Terapia Nutricional/métodos , Adolescente , Causalidade , Criança , Transtornos da Nutrição Infantil/epidemiologia , Transtornos da Nutrição Infantil/etiologia , Pré-Escolar , Comorbidade , Constipação Intestinal/epidemiologia , Constipação Intestinal/prevenção & controle , Doenças do Sistema Endócrino/epidemiologia , Doenças do Sistema Endócrino/prevenção & controle , Transtornos da Alimentação e da Ingestão de Alimentos/epidemiologia , Feminino , Gastroenteropatias/epidemiologia , Gastroenteropatias/prevenção & controle , Humanos , Lactente , Transtornos da Nutrição do Lactente/epidemiologia , Masculino , Estado Nutricional , Prevalência , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/prevenção & controle , Fatores de Risco , Infecções Urinárias/epidemiologia , Infecções Urinárias/prevenção & controle
12.
Nutrients ; 14(21)2022 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-36364923

RESUMO

BACKGROUND: The issue of vitamin metabolism in children with cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is not well known. The aim of this study was to determine the status of vitamins A, D, E, and C in the blood of a group of children with CFSPID. MATERIAL AND METHODS: A total of 89 children were enrolled in the study (Me: 3.6 years, 52.8% boys), as follows: 28 with CFSPID, 31 with CF (cystic fibrosis), and 30 HC (healthy children). Their blood concentrations of vitamins A, D, E, and C, and their dietary intake of these vitamins were analysed in the study groups on the basis of a three-day food diary. RESULTS: The patients with CFSPID had significantly higher serum vitamin D (p = 0.01) and E (p = 0.04) concentrations, compared to the children with CF. None of the children with CFSPID revealed vitamin A or E deficiencies. Patients with CF had been consuming significantly higher vitamin D and E amounts (p = 0.01). The vitamin concentrations did not depend either on the pancreatic/liver function or on anthropometric parameters. In total, 32.14% of patients with CF did not cover the baseline recommended calorie intake, and 53.6% and 36% did not take the recommended vitamin E and vitamin A intake, respectively. CONCLUSION: Children with CF and CFSPID did not fully cover the dietary recommendations for vitamin supply, but vitamin deficiency was found only in CF.


Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística , Fibrose Cística , Feminino , Humanos , Masculino , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Mutação , Triagem Neonatal , Vitamina A , Vitamina D , Vitaminas , Pré-Escolar
13.
Dis Markers ; 2022: 4798136, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35345868

RESUMO

Background: Complications of cystic fibrosis-associated liver disease (CFLD) are a leading nonpulmonary cause of death. Noninvasive tests enabling early detection of liver changes, especially in children are sought. The aim of the study was to assess the scale of liver fibrosis with the use of elastography in paediatric patients with diagnosed cystic fibrosis (CF) and its comparison with other tests (APRI and Fibrotest). Methods: We examined 41 children, in the age range 2-21 years, with diagnosed CF. The analysis a included clinical picture, laboratory parameters of liver damage, and cholestasis. Aspartate aminotransferase-to-platelet ratio index (APRI) and Fibrotest were done in all patients. Liver stiffness measurements were acquired using shear-wave elastography (SWE). Results: CFLD was diagnosed in 16/41 patients (39%). Abnormal elastography was observed in 19/41 patients (46.3%), and in 5/41 (12.2%), the changes were advanced (F4). Abnormal elastography was observed in 12/16 (75%) of the patients with CFLD, and in 7/25 (28%), there were no lesions observed in the liver in the course of cystic fibrosis. In all patients with F4, we observed abnormal results of the APRI and Fibrotest. In most patients with small changes in elastography, we found normal results of the APRI and Fibrotest. Conclusion: Elastography seems to be a noninvasive examination useful in everyday clinical work in detecting early liver changes and monitoring of progression in paediatric patients with diagnosed cystic fibrosis, ahead of changes in laboratory tests. The cost-effectiveness of this test, the possibility of its repetition, and its availability are additional benefits.


Assuntos
Fibrose Cística , Técnicas de Imagem por Elasticidade , Hepatopatias , Adolescente , Adulto , Criança , Pré-Escolar , Fibrose Cística/complicações , Fibrose Cística/diagnóstico por imagem , Fibrose Cística/patologia , Técnicas de Imagem por Elasticidade/métodos , Humanos , Fígado/diagnóstico por imagem , Fígado/patologia , Cirrose Hepática/diagnóstico , Índice de Gravidade de Doença , Adulto Jovem
14.
J Nutr Sci Vitaminol (Tokyo) ; 67(3): 189-195, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34193678

RESUMO

One component of a correctly balanced diet is dietary fibre. Fibre acts protectively-it improves the functioning of the intestines, regulates the rhythm of bowel movements, inhibits the absorption of sugar and also lowers the level of cholesterol. The aim of the research was to determine the intake of fibre in relation to the occurrence of excessive weight and obesity among children and adolescents living in rural areas. The research was conducted using an authored questionnaire. The study questionnaire was completed by the study participants and their parents over 7 d. The anthropometric measurements were carried out on pupils in their underwear in conditions of privacy. Based on the results obtained, the BMI index was calculated for each pupil and then ranked according to WHO reference values. Among the pupils in the study group, the intake of fibre was at a very low level. The lowest amount of fibre in the diet was found among those with excessive weight and with obesity. Over 39% pupils never consumed wholegrain bread. Fruit and vegetables were consumed most seldom by pupils with excessive body weight. Knowledge about the lifestyles of children and adolescents is of crucial importance in taking multidirectional preventative actions to make changes to such lifestyles.


Assuntos
Dieta , População Rural , Adolescente , Peso Corporal , Criança , Fibras na Dieta , Frutas , Humanos , Obesidade/epidemiologia , Verduras
15.
Pediatr Hematol Oncol ; 27(5): 393-404, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20615068

RESUMO

Increased angiogenesis is observed both in the inflammatory and in the neoplasmatic tissue. The aim of the study was to assess the diagnostic significance of serum concentration of vascular-endothelial growth factor (sVEGF) and basic fibroblast growth factor (sbFGF) in the various forms of lymphadenopathy in children. Ninety-four children with lymphadenopathy were studied: group A, 52 patients with lymphadenitis; group B, 42 patients with lymphomas. Group B was divided into subgroups: B(P), children with lymphomas in peripheral lymph nodes and B(M), children with lymphomas in peripheral lymph nodes and mediastinal tumor. The healthy control group was 20 children. Using enzyme-linked immunosorbent assays the authors quantified VEGF and bFGF in serum of healthy children and of children with lymphadenopathy. The sVEGF in group A was significantly higher than controls (313.8 versus 44.6 pg/mL; P <.05) and in group B was 633.4 pg/mL and was significantly higher than controls (P <.0001). The sVEGF and bFGF in group A versus subgroup B(P) were significantly lower (P(VEGF) <.05, P(bFGF) <.05), and sVEGF in subgroup B(P) versus B(M) was significantly lower (P <.05). These results show that the evaluation of serum VEGF concentration might be useful as noninvasive diagnosis of some chronic peripheral lymphadenopathies in children.


Assuntos
Proteínas Angiogênicas/sangue , Doenças Linfáticas/sangue , Doenças Linfáticas/diagnóstico , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Fator 2 de Crescimento de Fibroblastos/sangue , Humanos , Linfadenite/sangue , Linfadenite/diagnóstico , Linfoma/sangue , Linfoma/diagnóstico , Masculino , Neovascularização Patológica/sangue , Fator A de Crescimento do Endotélio Vascular/sangue
16.
Pediatr Blood Cancer ; 53(7): 1195-9, 2009 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-19621434

RESUMO

BACKGROUND: Angiogenesis and proangiogenic cytokines are involved in neoplastic development. The role of these processes in lymphoma formation has not been established. The aim of the study was to assess angiogenesis on the basis of serum levels of vascular-endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in childhood lymphomas. The prognostic value of these parameters was determined in the examined children. PROCEDURE: Forty-two children with lymphomas (Hodgkin and non-Hodgkin Lymphomas) were studied (group A). Group A was divided into two subgroups: A(CR)-30 children with complete remission (CR) and A(PR+P+ER)-12 children with partial remission (PR), progressive disease (P), and early relapse (ER). The control group (group C) consisted of healthy 20 children. Using enzyme-linked immunosorbent assays we quantified VEGF and bFGF in serum of the healthy children and of the children with lymphomas. RESULTS: The serum VEGF concentration in group A was 633.4 pg/ml (24.0-1,210.5) and was significantly higher (P = 0.0001) in comparison with group C (144.6 pg/ml; 32.3-734.8). In subgroup A(PR+P+ER), the baseline serum VEGF concentration was 865.0 pg/ml (205.8-1,209.2) and was significantly higher (P = 0.02) than in subgroup A(CR) (564.0 pg/ml; 24.0-1,210.5). The high serum VEGF concentration in children with lymphomas was the only independent risk factor for treatment failure (OR = 8.64; 95 CI: 1.51-49.34; P = 0.01). The cutoff point for the serum VEGF level >or=633.4 pg/ml as a parameter predicting treatment failure was established (P = 0.01). CONCLUSION: Baseline serum VEGF concentration constitutes a prognostic marker for the progression of Hodgkin and non-Hodgkin Lymphomas in children.


Assuntos
Fator 2 de Crescimento de Fibroblastos/sangue , Linfoma/sangue , Proteínas de Neoplasias/sangue , Neovascularização Patológica/sangue , Fator A de Crescimento do Endotélio Vascular/sangue , Adolescente , Biomarcadores , Criança , Pré-Escolar , Progressão da Doença , Feminino , Doença de Hodgkin/sangue , Doença de Hodgkin/mortalidade , Doença de Hodgkin/fisiopatologia , Humanos , Linfoma/mortalidade , Linfoma/fisiopatologia , Linfoma não Hodgkin/sangue , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/fisiopatologia , Masculino , Prognóstico , Indução de Remissão , Fatores de Risco , Análise de Sobrevida , Falha de Tratamento
17.
J Pediatr Hematol Oncol ; 31(8): 599-601, 2009 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-19641469

RESUMO

Ectopic cervical location of the thymus is a very rarely diagnosed developmental disorder in children. This anomaly is usually manifested clinically as a neck tumor suggesting lymphadenopathy, which is also differentiated from more frequently occurring cervical cysts, angiomas, or malignant neoplasms. A decisive examination is the histopathologic assessment of the tumor, supported by necessary immunoassays. A diagnostic process is sometimes very difficult and the results are ambiguous; what is more, this process has a decisive impact on the child's future life. Therefore, a histopathologic evaluation performed by 2 independent pathologists should become the standard procedure. We present the case of a 9-month-old girl, in whom, after a difficult diagnostic process, ectopic cervical location of the thymus was diagnosed.


Assuntos
Coristoma/diagnóstico , Neoplasias de Cabeça e Pescoço/diagnóstico , Pescoço , Timo , Diagnóstico Diferencial , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Lactente
18.
Mediators Inflamm ; 2009: 512695, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-20037744

RESUMO

The aim of the study was the evaluation of frequency and titre of IgA ASCA and IgG ASCA and p-ANCA, c-ANCA in children with IBD and occurrence of ASCA antibodies in relation to coexistence of FA. Patients and methods. The study comprised 95 children at the ages of 2 to 18 years. The diagnosis of IBD was established on the basis of Porto criteria. Tests of blood serum were performed in all children: IgA and IgG ASCA, p-ANCA, c-ANCA using ELISA method. Results. IgE-dependent FA was found in 32.5% children with UC and in 21% with CD. We did not observe any relation between the occurrence of FA and the frequency and ASCA titre. p-ANCA were significantly more frequent in the group of children with UC. The occurrence of ASCA antibodies was observed in 73.7% of children with CD, 17.5% with UC and almost 30% with allergic colitis. Conclusions. Patients with CD and the presence of ASCA revealed a significantly more frequent localization of lesions within the small bowel and a tendency towards older age. We observed a connection between the occurrence of antibodies and the examined mutations of gene NOD2/CARD15.


Assuntos
Hipersensibilidade Alimentar/sangue , Doenças Inflamatórias Intestinais/sangue , Adolescente , Anticorpos Anticitoplasma de Neutrófilos/sangue , Criança , Pré-Escolar , Doença de Crohn/sangue , Doença de Crohn/genética , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Mutação
19.
Pol Merkur Lekarski ; 24(141): 185-9, 2008 Mar.
Artigo em Polonês | MEDLINE | ID: mdl-18634281

RESUMO

Angiogenesis is a process of creating new vessels which is necessary in pathogenesis of many diseases and disorders like inflammatory or malignancies. A lot of markers, both stimulating and inhibiting can influence on this process. Knowledge about angiogenesis and its markers, e.g. Vascular Endothelial Growth Factor (VEGF), basic Fibroblast Growth Factor (bFGF) can be used in diagnosis and treatment of diseases like malignancies. For these reasons summary of these processes seems to be very important and valid.


Assuntos
Fator 2 de Crescimento de Fibroblastos/metabolismo , Inflamação/metabolismo , Neoplasias/metabolismo , Neovascularização Patológica , Fator A de Crescimento do Endotélio Vascular/metabolismo , Doença Crônica , Humanos
20.
Przegl Lek ; 65(7-8): 353-7, 2008.
Artigo em Polonês | MEDLINE | ID: mdl-19004235

RESUMO

The new blood vessels formation in the body is through vasculogenesis, angiogenesis, lymphangiogenesis and arteriogenesis processes. A process of creating new blood vessels is necessary for normal organism function, and is necessary too in pathogenesis of many diseases and disorders like inflammatory or malignancies. Vascular-Endothelial Growth Factor (VEGF) and basic Fibroblast Growth Factor (bFGF) are the main stimulators blood vessels formation. Knowledge about properties and function these markers can be used in diagnosis and treatment of diseases like malignancies. These article is summary knowledge about structure and function VEGF and bFGF.


Assuntos
Fator 2 de Crescimento de Fibroblastos/metabolismo , Fator A de Crescimento do Endotélio Vascular/metabolismo , Animais , Fator 2 de Crescimento de Fibroblastos/química , Humanos , Neovascularização Patológica/metabolismo , Neovascularização Fisiológica/fisiologia , Fator A de Crescimento do Endotélio Vascular/química
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