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1.
Clin Endocrinol (Oxf) ; 101(2): 99-107, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38935859

RESUMEN

OBJECTIVE: To investigate the clinical, laboratory findings and signal intensity index (SII) on magnetic resonance imaging (MRI) of patients with bilateral and unilateral macronodular mild autonomous cortisol secretion (MACS). PATIENTS AND MEASUREMENTS: Clinical and laboratory findings of 81 patients with MACS were examined from retrospective records. SII of adenomas and internodular areas were evaluated by MRI. The unilateral group included patients with an adrenal macronodule (≥1 cm) in a single adrenal gland, while the bilateral group included patients with at least one macronodule in both adrenal glands. RESULTS: In total, 46 patients were in the unilateral (57%), while 35 (43%) patients were in the bilateral groups. The dehydroepiandrosterone sulphate (DHEA-S) level was lower in the unilateral than in the bilateral group (p < .001). The presence of type 2 diabetes mellitus (T2DM), glycosylated haemoglobin (HbA1c) and low-density lipoprotein (LDL) concentrations were higher in the bilateral group (p < .05). However, no significant difference was detected in terms of adrenocorticotropic hormone (ACTH) and overnight 1 mg dexamethasone suppression test (DST) between the two groups (p > .05). There was no difference in SII between adenomas within the same patient, as well as between the unilateral and bilateral groups (p > .05). Logistic regression analysis based on the differentiation between unilateral and bilateral macronodular MACS demonstrated that DHEA-S, HbA1c and LDL concentrations were associated factors. CONCLUSION: DHEA-S levels may not be as suppressed in patients with bilateral macronodular MACS as compared to those with unilateral adenoma. T2DM and hypercholesterolaemia have a higher frequency in bilateral patients. However, ACTH, overnight 1 mg DST and SII may not provide additional information for differentiation of bilaterality and unilaterality.


Asunto(s)
Hidrocortisona , Imagen por Resonancia Magnética , Humanos , Femenino , Hidrocortisona/sangre , Hidrocortisona/metabolismo , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto , Anciano , Sulfato de Deshidroepiandrosterona/sangre , Hormona Adrenocorticotrópica/sangre , Glándulas Suprarrenales/metabolismo , Glándulas Suprarrenales/diagnóstico por imagen , Glándulas Suprarrenales/patología , Diabetes Mellitus Tipo 2/metabolismo , Diabetes Mellitus Tipo 2/sangre , Neoplasias de las Glándulas Suprarrenales/metabolismo , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/sangre
2.
Fetal Pediatr Pathol ; 36(6): 472-474, 2017 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-29220605

RESUMEN

BACKGROUND: Nuclear protein of the testis (NUT) midline carcinoma is genetically defined by rearrangement of NUT or by immunohistochemical expression of NUT. FINDINGS: A 6-year old child had a NUT midline carcinoma of the lung. Despite aggressive therapy, the child died. CONCLUSION: NUT carcinoma, which can be diagnosed immunohistochemically, remains an aggressive tumor.


Asunto(s)
Carcinoma/genética , Neoplasias Pulmonares/genética , Proteínas Nucleares/genética , Proteínas de Fusión Oncogénica/genética , Apoptosis , Carcinoma/terapia , Niño , Diagnóstico Diferencial , Progresión de la Enfermedad , Resultado Fatal , Femenino , Reordenamiento Génico , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , Neoplasias Pulmonares/terapia , Necrosis
3.
Pediatr Int ; 58(5): 379-382, 2016 May.
Artículo en Inglés | MEDLINE | ID: mdl-26541772

RESUMEN

Congenital diarrheal disorders are caused by disruption in nutrient digestion, absorption, or transport, enterocyte development and functioning, or enteroendocrine functioning. Many additional rare forms of congenital diarrhea are expected to be linked to genes associated with appropriate intestinal fluid and electrolyte balance. Neurogenin-3 mutation, a very rare form of congenital diarrhea, disrupts enteroendocrine cell differentiation and is characterized by malabsorption and the absence of pancreatic islet cells. Diabetes mellitus is typically associated with malabsorptive diarrhea at early onset or at later presentation in neurogenin-3 mutation. Here, we describe the case of an infant with homozygous neurogenin-3 mutation who had severe malabsorptive diarrhea and episodes of hyperchloremic metabolic acidosis after birth. Remarkably, cholestyramine was effective at reducing stool volume and frequency and improved the consistency of the stools; diabetes was not present in this patient.

4.
Ren Fail ; 38(8): 1283-90, 2016 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-27402370

RESUMEN

BACKGROUND: In this study, it was aimed to determine the effects of alfuzosin on experimentally generated unilateral partial ureteropelvic junction obstruction (UPO) in rats. MATERIALS AND METHODS: Thirty Long-Evans rats were randomly allocated into five groups. In control group (C), nothing was performed; in group Sham (S) only laparotomy was done; in Alfuzosin group (A) only alfuzosin was administered for two weeks (10 mg/kg/day p.o.) without any surgery; in UPO group, unilateral UP junction obstruction was produced; and in the Group UPT (ureteropelvic obstruction + treatment), alfuzosin was administered for two weeks (10 mg/kg/day p.o.) in addition to UPO production. Renal pelvic anteroposterior diameters were determined with ultrasonography (USG) and renal arterial resistivity indexes by color Doppler USG. Urine was collected both at the beginning and at the end of the experiment for 24 h in all the groups and at the end of the experiment, blood samples were obtained. Blood and urine electrolytes and TGF-ß1, urine density, urine ß2 microglobulin levels were determined. Renal tissue samples harvested from all of the rats were histopathologically evaluated. Results were determined using one-way ANOVA t-test; p < 0.05 was accepted as significant. RESULTS: Urine density in the UPT group was lower with respect to UPO group and blood electrolytes were preserved as close to normal (p < 0.05). In the UPT group, urine TGF-ß1 and blood TGF-ß1, blood ß2 microglobulin levels and histopathologic damage scores were lower compared to the UPO group (p < 0.05). CONCLUSION: It is shown in this experimental unilateral partial UPO model that alfuzosin treatment prevents obstructive renal damage.


Asunto(s)
Electrólitos/orina , Riñón/patología , Quinazolinas/administración & dosificación , Factor de Crecimiento Transformador beta1/orina , Obstrucción Ureteral/terapia , Microglobulina beta-2/orina , Animales , Modelos Animales de Enfermedad , Pelvis Renal/diagnóstico por imagen , Masculino , Distribución Aleatoria , Ratas , Ratas Long-Evans , Arteria Renal/diagnóstico por imagen , Factor de Crecimiento Transformador beta1/sangre , Ultrasonografía Doppler , Microglobulina beta-2/sangre
5.
Eur J Pediatr ; 174(7): 903-10, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25567795

RESUMEN

UNLABELLED: The link between Helicobacter pylori and iron deficiency (ID) or iron deficiency anemia (IDA) has been investigated recently. We suggested that IDA/ID associated with H. pylori infection might be mediated by inflammation-driven hepcidin production. Patients with complaints of recurrent abdominal pain and dyspepsia aged between 7-16 years were included in this study. Patients were divided into two groups according to H. pylori status in upper gastrointestinal endoscopy. Group I who had H. pylori gastritis (n=50) received triple antibiotic therapy. Group II (n=50) who had H. pylori-negative gastritis only received proton pump inhibitor. Thirty healthy children with the similar age and gender were included in the study as a control group. Complete blood count, serum iron levels, iron-binding capacity, ferritin levels, prohepcidin and interleukin-6 (IL-6) values were evaluated in all children at the first visit. Initial tests were repeated after H. pylori eradication. Initial levels of ferritin (p=0.002), prohepcidin (p=0.003), and IL-6 (p=0.004) were found significantly lower in group I compared to group II and the control group. The mean prohepcidin level was lower in the anemic H. pylori-positive group than in non-anemic H. pylori-positive group; however, the difference was not statistically significant. While significant increases in hematocrit and mean corpuscular volume were observed, no significant difference was found in serum ferritin, prohepcidin, or IL-6 level after eradication treatment in H. pylori-positive group. CONCLUSION: H. pylori-induced gastritis appears to cause an increase in prohepcidin levels and a decrease in ferritin levels, supporting our hypothesis; but this relationship has not been proven.


Asunto(s)
Anemia Ferropénica/diagnóstico , Gastritis/microbiología , Infecciones por Helicobacter/complicaciones , Hepcidinas/sangre , Interleucina-6/sangre , Adolescente , Antibacterianos/uso terapéutico , Estudios de Casos y Controles , Niño , Índices de Eritrocitos , Femenino , Ferritinas/sangre , Gastritis/tratamiento farmacológico , Infecciones por Helicobacter/tratamiento farmacológico , Helicobacter pylori , Hematócrito , Hemoglobinas/análisis , Humanos , Masculino , Inhibidores de la Bomba de Protones/uso terapéutico
6.
Appl Immunohistochem Mol Morphol ; 32(6): 285-291, 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38721798

RESUMEN

Knowledge of the molecular pathways of pediatric high-grade gliomas is increasing. Gliomas with mismatch repair deficiency do not currently comprise a distinct group, but data on this topic have been accumulating in recent publications. Immunohistochemistry can effectively determine mismatch repair status, indirectly suggesting the microsatellite instability of the tumor. This study aimed to determine the number of mismatch repair-deficient pediatric high-grade gliomas in a tertiary institution and assess the relationship between the survival and mismatch repair status of the patients. It also aimed to assess the potential for further clinical studies including immunotherapy. Of 24 patients with high-grade gliomas, 3 deceased patients were mismatch repair-deficient. Mismatch repair deficiency was significantly associated with shorter survival ( P =0.004). Immunotherapy trials need to progress, and patients with mismatch repair-deficient pediatric high-grade gliomas are the most suitable candidates for such studies.


Asunto(s)
Neoplasias Encefálicas , Reparación de la Incompatibilidad de ADN , Glioma , Inmunohistoquímica , Humanos , Glioma/metabolismo , Glioma/patología , Glioma/genética , Niño , Femenino , Masculino , Preescolar , Adolescente , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/metabolismo , Neoplasias Encefálicas/genética , Lactante , Clasificación del Tumor , Inestabilidad de Microsatélites , Síndromes Neoplásicos Hereditarios/patología , Síndromes Neoplásicos Hereditarios/metabolismo , Neoplasias Colorrectales
7.
Int J Rheum Dis ; 27(5): e15187, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38742741

RESUMEN

Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient. To our knowledge, this is the first case pediatric thymic carcinoma accompany with severe polyarthritis and myopathy, thus we have reviewed the current literature regarding the cases of thymic malignancies coexisting with paraneoplastic syndromes in children.


Asunto(s)
Artritis , Miositis , Síndromes Paraneoplásicos , Timoma , Neoplasias del Timo , Humanos , Masculino , Miositis/diagnóstico , Miositis/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/etiología , Neoplasias del Timo/complicaciones , Neoplasias del Timo/diagnóstico , Adolescente , Artritis/diagnóstico , Artritis/etiología , Timoma/complicaciones , Timoma/diagnóstico , Resultado del Tratamiento , Timectomía , Biopsia
8.
Pediatr Int ; 55(4): e86-9, 2013 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-23910814

RESUMEN

Rectal duplication (RD) cysts are rare congenital anomalies that can be diagnosed with the presence of another opening in the perineum. They seldom accompany anorectal malformations (ARM). Two cases of RD accompanying ARM at opposite ends of the phenotypic spectrum, are described. A 3-month-old baby and a 2-year-old girl with ARM were scheduled for posterior sagittal anorectoplasty. The infant had an orifice at the anal dimple and the other had an orifice at the vestibulum posterior to the rectovestibular fistula. The infant presented with no other anomalies whereas the older one presented with an unusual coexistence of caudal duplication and caudal regression syndromes. Perioperatively both orifices were found to be related to retrorectal cysts, and were excised. Clinicians should always be alert when dealing with complex malformations. Because these malformations have variable anatomical and clinical presentations, they can represent a diagnostic and therapeutic challenge.


Asunto(s)
Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Procedimientos de Cirugía Plástica/métodos , Fístula Rectal/cirugía , Recto/anomalías , Preescolar , Colostomía , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Fístula Rectal/diagnóstico , Recto/cirugía
9.
Turk J Pediatr ; 55(2): 222-5, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24192687

RESUMEN

Food allergies are often seen in infancy and usually show the clinical signs in the form of type I hypersensitivity reaction. However, a combination of food allergies can sometimes be found in the same patient. In this report, we present an 11-month-old infant who had a combination of anaphylaxis and eosinophilic esophagitis induced by cow's milk. Diagnosis of eosinophilic esophagitis is done by examination of esophageal biopsy material histologically. This is a very rare combination of food allergy. Improvement in clinical and histological findings of eosinophilic esophagitis was achieved by removal of cow's milk and dairy products from the infant's diet.


Asunto(s)
Anafilaxia/inmunología , Esofagitis Eosinofílica/inmunología , Hipersensibilidad a la Leche/complicaciones , Lactancia Materna , Esofagitis Eosinofílica/patología , Femenino , Humanos , Lactante , Membrana Mucosa/patología
10.
Turk Neurosurg ; 33(6): 1120-1125, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37846542

RESUMEN

AIM: To determine if previous histological grading systems were sufficient or unreliable with a limited repository of modern techniques. MATERIAL AND METHODS: The pathology reports of pediatric neurosurgery patients between 2019-2022 were accessed. Data on patients that needed unattainable further molecular investigation were extracted. Data were noted from electronic archives, including their sex, age, histologic grade, location, resection type, survival, and therapy. RESULTS: Out of 61 surgeries, 17 patients needed further investigation for a proper 2022 World Health Organization (WHO) diagnosis. Seven were deceased, and nine were alive. Two of 10 patients with low-grade gliomas and five of six patients with highgrade gliomas were deceased. Data on one foreign patient with high-grade glioma was inaccessible. The average survival was 9 months for the deceased. CONCLUSION: Modern molecular techniques such as next-generation sequencing and methylation profiling are the state-ofthe- art methods, but it is hard for developing and underdeveloped countries to utilize such methods. The classification schemes, diagnostic key figures, and treatment modalities are developed using these techniques, but the less developed world is incapable of achieving these. We are trying to hybridize the modern and classic modalities, and the results of our study show that for overall survival, there is still not much difference. More economic and feasible techniques should be produced and summarized for the rest of the world.


Asunto(s)
Neoplasias Encefálicas , Glioma , Humanos , Niño , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirugía , Estudios Retrospectivos , Glioma/diagnóstico , Glioma/genética , Glioma/cirugía
11.
Contemp Oncol (Pozn) ; 16(1): 26-33, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-23788851

RESUMEN

AIM OF THE STUDY: Although the survival for children with certain central nervous system (CNS) tumour types has improved through current surgical and adjuvant treatment modalities, the prognosis of many high-grade tumours remains poor despite aggressive treatment. The aim of this study is to analyse patients with high-grade brain tumours in our institution to determine the histopathology, clinical characteristics, treatment modalities, and survival. MATERIAL AND METHODS: A total of 74 patients with a diagnosis of high-grade brain tumour were analysed. There were a total of 31 patients with embryonal tumours, 27 patients with high-grade glial tumours, 12 patients with brain stem gliomas and 4 patients with other high-grade brain tumours. RESULTS: There were 48 (65%) boys and 26 (35%) girls (ratio: 1.85) with a median age of 99.7 months (range = 2-204 months). The median follow-up period was 19 months (range = 1-204 months). Tumour recurrence was observed in 38 patients (51.4%). The overall survival rate and event-free survival rate of our patients were 27% and 19.5%, respectively. CONCLUSIONS: Pediatric high-grade CNS tumours have a very aggressive behaviour and a significant number of children eventually succumb to disease despite multimodal treatment. There is a need of more effective therapeutic approaches for these tumours with poor prognosis. The future improvement in childhood high-grade brain tumour management depends on a better understanding of the molecular genetics and biology of brain tumours.

12.
Nucl Med Commun ; 43(7): 778-786, 2022 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-35506271

RESUMEN

PURPOSE: The aim of this study was to evaluate the prognostic value of PET-derived metabolic features and textural parameters of primary tumors in pediatric sarcoma patients. METHODS: The imaging findings of 43 patients (14 girls and 29 boys; age 11.4 ± 4.4 years) who underwent 18-fluorodeoxyglucose positron emission tomography (PET)/computed tomography for primary staging prior to therapy between 2005 and 2020 were retrospectively evaluated. The diagnoses were osteosarcoma in 10, rhabdomyosarcoma in 10, and Ewing sarcoma in 23 patients. PET metabolic data and textural features of primary tumors were obtained. Cox proportional hazards regression models were used to identify predictors for progression-free survival and overall survival. Survival curves were estimated by using the Kaplan-Meier method. RESULTS: Distant metastases were detected in primary staging in 13 patients (30.2%). The median follow-up duration after diagnosis was 28 months (range: 10-171 months). In multivariate Cox regression analysis, the presence of distant metastasis and neighborhood grey-level difference matrix_Contrast (ngldm_Contrast) were found as independent predictors for both progression-free survival and overall survival. Grey-level zone length matrix_Zone-length nonuniformity (glzlm_ZLNU) was also found as an independent predictor for overall survival. The Kaplan-Meier survival analysis showed that higher ngldm_Contrast and glzlm_ZLNU values of primary tumors were significantly associated with shorter progression-free survival and overall survival. CONCLUSION: In addition to the presence of distant metastasis at initial diagnosis, textural features of primary tumors may be used as prognostic biomarkers to identify patients with worse prognosis in pediatric sarcoma. Higher tumor heterogeneity is significantly associated with shorter progression-free survival and OS.


Asunto(s)
Tomografía de Emisión de Positrones , Sarcoma , Adolescente , Niño , Femenino , Fluorodesoxiglucosa F18 , Humanos , Masculino , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Pronóstico , Radiofármacos , Estudios Retrospectivos , Sarcoma/diagnóstico por imagen
13.
J Oncol Pharm Pract ; 17(4): 444-7, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-21282299

RESUMEN

Mucinous breast carcinoma (MBC) is a rare histological type of breast cancer and rarely associated with advanced disease. We report a case that had MBC with an isolated adrenal metastasis which was removed by laparoscopic adrenelectomy. This case is unique due to the unexpected metastasis of pure mucinous carcinoma developed after 4 years of hormone therapy.


Asunto(s)
Adenocarcinoma Mucinoso/diagnóstico , Adenocarcinoma Mucinoso/secundario , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/secundario , Neoplasias de la Mama/diagnóstico , Anciano , Femenino , Humanos
14.
Mikrobiyol Bul ; 45(2): 344-52, 2011 Apr.
Artículo en Turco | MEDLINE | ID: mdl-21644078

RESUMEN

Pulmonary aspergillosis which is an important opportunistic infection in neutropenic patients, is usually caused by Aspergillus fumigatus. Since the pathogenesis of disease is not well understood, the main proposed mechanism is thought to be cell-mediated immunity and cytokine response. The aim of this study was to investigate the local production of cytokines in the lung tissues of rats with experimentally developed aspergillosis, by reverse transcriptase-polymerase chain reaction (RT-PCR). A total of 33 Wistar albino type rats were included in the study with the consent of Experimental Animal Ethics Committee. Twenty-five of the rats were infected with A.fumigatus by intratracheal way, while 8 animals were used as controls. The presence of A.fumigatus in the lung tissues of infected rats was confirmed with the use of quantitative culture and histologic staining methods. RNA isolation from the lung tissue samples of both groups were performed by a commercial kit (Qiagen, Germany). After obtaining complementary DNAs from the genomic RNAs, in-house qualitative and quantitative (real-time) PCR methods were used to amplify the target regions for interleukin-10 (IL-10), tumor necrosis factor-alpha (TNF-?) and interferon-gamma (IFN-?) by using specific primers (Tib Molbiol, Germany). Mean mRNA levels achieved by real-time PCR for IL-10, TNF-? and IFN-? in aspergillosis group were 6.5 x 106 copies/ml, 7.9 x 105 copies/ml and 2.2 x 103 copies/ml, respectively, while those values in control group were 4.3 x 102 copies/ml, 5.6 x 103 copies/ml and 1.3 x 102 copies/ml, respectively. Our data indicated that rat model of aspergillosis was associated with significantly increased expression of mRNA encoding IL-10 and TNF-? than controls (p< 0.05), however there was no statistically significant difference between the groups with respect to IFN-? expression (p= 0.53). In conclusion, the production of proinflammatory cytokines which mediate the influx of phagocytic cells might account for the localization of Aspergillus infection to the upper respiratory tract. The up-regulation of the expression of the immunomodulatory cytokine TNF-? and IL-10 in lung tissue from infected rats might be important to limit the extent of local tissue destruction, but might also account for the fact that infected rats are generally unable to clear the infection spontaneously.


Asunto(s)
Aspergillus fumigatus/inmunología , Interferón gamma/análisis , Interleucina-10/análisis , Pulmón/inmunología , Aspergilosis Pulmonar/inmunología , Factor de Necrosis Tumoral alfa/análisis , Animales , Aspergillus fumigatus/genética , Aspergillus fumigatus/aislamiento & purificación , Modelos Animales de Enfermedad , Femenino , Regulación Fúngica de la Expresión Génica , Interferón gamma/genética , Interleucina-10/genética , Pulmón/microbiología , ARN Mensajero/aislamiento & purificación , ARN Mensajero/metabolismo , Ratas , Ratas Wistar , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Factor de Necrosis Tumoral alfa/genética
15.
Kulak Burun Bogaz Ihtis Derg ; 21(5): 298-302, 2011.
Artículo en Turco | MEDLINE | ID: mdl-21919839

RESUMEN

Paragangliomas are very rarely seen in the nose, paranasal sinuses and nasopharynx. The development of paraganglioma concurrently with nasopharyngeal carcinoma in the same region has not been reported to date. In this study we reported a 59-years-old a female case of nasopharyngeal carcinoma who underwent radiotherapy six years ago and got the diagnosis of sinonasal paraganglioma in the same region during follow-up and underwent nasal endoscopic excision.


Asunto(s)
Neoplasias Nasofaríngeas/diagnóstico , Paraganglioma/diagnóstico , Neoplasias de los Senos Paranasales/diagnóstico , Carcinoma , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Carcinoma Nasofaríngeo , Neoplasias Nasofaríngeas/complicaciones , Neoplasias Nasofaríngeas/diagnóstico por imagen , Neoplasias Nasofaríngeas/radioterapia , Neoplasias Nasofaríngeas/cirugía , Neoplasias Primarias Múltiples , Paraganglioma/complicaciones , Paraganglioma/diagnóstico por imagen , Paraganglioma/radioterapia , Paraganglioma/cirugía , Neoplasias de los Senos Paranasales/complicaciones , Neoplasias de los Senos Paranasales/diagnóstico por imagen , Neoplasias de los Senos Paranasales/radioterapia , Neoplasias de los Senos Paranasales/cirugía , Tomografía Computarizada por Rayos X
16.
Turk J Pediatr ; 63(5): 758-766, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34738358

RESUMEN

BACKGROUND: Programmed death 1 (PD-1) is a co-receptor which is located at the surface of cells like natural killer, monocytes, T and B cells. It has two ligands including programmed death ligand-1 (PD-L1) and ligand-2 (PD-L2). T cell functions are inhibited by activation of PD-1/PD-L1 pathway and this pathway is used by viruses and some tumor cells in order to escape from immune eradication. In our study we evaluated PD-L1 expression in the tissue specimens of patients with Wilms tumor, neuroblastoma and other renal tumors. METHODS: Totally 60 patients who were followed up at Gazi University Hospital with the diagnosis of neuroblastoma, Wilms tumor and other renal tumors were included. PD-L1 expression was examined in tumor samples of the patients. RESULTS: Positive staining with PD-L1 was detected only in two male patients. Both of them had neuroblastoma and advanced stage disease. None of the patients with Wilms tumor and other renal tumors had positive PD-L1 staining. CONCLUSIONS: Unlike adult tumors, PD-L1 expression is not common in childhood tumors due to differences in immune system between children and adults. Further studies are needed to establish the importance and effects of PD-1/PD-L1 pathway in pediatric tumors.


Asunto(s)
Neoplasias Renales , Neuroblastoma , Antígeno B7-H1 , Humanos , Masculino , Receptor de Muerte Celular Programada 1
17.
J Pediatr Urol ; 17(3): 292.e1-292.e7, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33608226

RESUMEN

INTRODUCTION: Penile tourniquet (Pt) application aims to work in a bloodless field in penile surgery. When the tourniquet is released, reperfusion injury occurs with the resumption of blood flow. Molecular hydrogen can easily attach to biomembranes and enter cytosol, mitochondria and other organelles of the cell and convert the formed OH- to H2O to prevent cell and tissue damage. AIM: We investigated the effects of hydrogen rich saline solution (HRSS) on penile Mathieu type flap tissue with Pt application in rats. STUDY DESIGN: Thirty-six Wistar-albino male rats were randomly divided into six groups. No operations were performed in the Sham group. Ventral penile Mathieu type flap was prepared and Pt was applied to the root of the penis with a plastic band in other groups. Pt was applied 10 and 30 min in the PT1° and PT³° groups. HRSS was injected intraperitoneally (ip) 5 ml/kg just before Pt was released in the HRSS1° and HRSS³° groups. In the HRSSB group, HRSS was injected 1 h before 10 min of Pt application. At the 4th hour of experiments the rats were sacrificed and tissue samples were taken for biochemical and histopathological studies. Tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), myeloperoxidase (MPO), malondialdehyde (MDA) and glutathione (GSH) levels were determined in the penile tissue. The results were analyzed with one-way ANOVA and Pearson's Chi-Squared test. RESULTS: Tissue MDA, MPO, IL-6 and TNF-α values were significantly lower in all HRSS groups compared to PT1° and PT³° groups. Tissue GSH levels of HRSS groups were higher compared to PT groups. Histopathologically, inflammation was found to be higher in PT groups compared to HRSS groups. Interestingly, in the HRSSB group with HRSS administration prior to Pt, the damage was less in grade, but not statistically different than the other HRSS groups (p > 0.05). DISCUSSION: In previous studies, damage in histopathological examinations after Pt could only be demonstrated long after tourniquet applications such as 24 h and with longer duration of Pt such as 30 min. Structural changes in different Pt application times could be demonstrated at 60 min by electron microscopy and 48 h by light microscopy. In this study, the histopathological effect of Pt application could be demonstrated at the 4th hour after release and HRSS was observed to reduce the damage histopathologically as well as biochemically with its anti-inflammatory and antioxidant effects. It was observed that administration of HRSS either before or following Pt did not cause an alteration statistically. CONCLUSION: HRSS reduces tissue oxidative stress and inflammation on the flap tissue and has a protective effect in Pt applied to the hypospadias model created with a penile flap.


Asunto(s)
Daño por Reperfusión , Solución Salina , Animales , Hidrógeno , Masculino , Pene/cirugía , Ratas , Ratas Wistar , Daño por Reperfusión/prevención & control , Torniquetes
18.
Clin Rheumatol ; 40(9): 3817-3825, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-33590421

RESUMEN

Autoimmune pancreatitis (AIP) type 1 is an IgG4-related disease (IgG4-RD), characterized by inflammatory pseudotumors and histologically by dense lymphoplasmacytic infiltrates rich in IgG4 positive plasma cells, storiform fibrosis, and obliterative phlebitis. Although quite rare, IgG4-RD was found to be associated with medium or small vessel vasculitides. A new overlap syndrome between IgG4-RD and ANCA-associated vasculitis (AAV) has recently been described in the adult population. Here we present a 16-year-old adolescent girl admitted with abdominal pain, episcleritis, palpable purpura, salivary gland enlargement, and bloody diarrhea. Laboratory investigations revealed findings of glomerulonephritis. Abdominal imaging surprisingly revealed a focal mass in the pancreatic tail, while the c-ANCA level was found to be quite high as well as serum IgG4 level. Biopsy of the pancreatic mass showed lymphoplasmacytic IgG4 positive cells infiltrating the pancreas with storiform fibrosis compatible with IgG4-related AIP. The renal biopsy that was done simultaneously showed necrotizing granulomatous vasculitis indicating AAV. Renal biopsy showed IgG4 positive plasma cells very rarely by immunohistochemical examination, which does not indicate any significance for IgG4-RD. Our diagnosis was IgG4-related AIP and AAV overlap syndrome, which has not been reported in the pediatric populations yet. IgG4-RD should be investigated in patients with ANCA-associated vasculitis who shows atypical organ involvement. We searched the Pubmed/Medline and Google Scholar databases to identify clinical findings, treatment, and outcome of the patients with IgG4-related AIP and AAV.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Enfermedades Autoinmunes , Enfermedad Relacionada con Inmunoglobulina G4 , Adolescente , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Anticuerpos Anticitoplasma de Neutrófilos , Enfermedades Autoinmunes/diagnóstico , Femenino , Humanos , Inmunoglobulina G , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico
19.
J Pediatr Surg ; 55(8): 1574-1578, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31466816

RESUMEN

AIM: We investigated the effects of hydrogen-rich saline solution (HRSS) on intestinal anastomosis performed after intestinal ischemia reperfusion injury (IRI). MATERIALS AND METHODS: Thirty Wistar albino female rats were randomly divided into five groups. Only laparotomy was performed in the Sham group. In the other four groups, an intestinal IRI was performed for 45 min by clamping the superior mesenteric artery. After intestinal IRI, anastomosis was performed by cutting the intestine from the proximal 15 cm of the ileocecal valve at the first and 24th hours. HRSS was given intraperitoneally 5 ml/kg before reperfusion and for four more days in the HRSS1 and HRSS24groups, while no treatment was given to the I/R1 and I/R24 groups. After 5 days, all groups underwent relaparotomy. The anastomotic bursting pressures were measured in all groups, except the Sham group. The tumor necrosis factor-α (TNF-α), interleukin 6 (IL-6), myeloperoxidase (MPO) and malondialdehyde (MDA) levels were measured in the tissues taken from the anastomosis line. The tissue sections were evaluated histopathologically and the apoptosis index was determined by applying the TUNEL method. The results were analyzed one-way analysis of variance (ANOVA) and Pearson's chi-squared test. RESULTS: Although the MPO, MDA, IL-6 and TNF-α tissue values were not statistically significant among the groups, the degree of tissue damage and apoptosis levels were lower and the anastomotic bursting pressures values were higher in the HRSS1 and HRSS24 groups compared to the I/R1 and I/R24 groups. CONCLUSION: HRSS is effective in reducing the intestinal damage caused by an IRI: HRSS has the potential to reduce the detrimental effects of intestinal anastomosis performed after an intestinal IRI.


Asunto(s)
Anastomosis Quirúrgica , Procedimientos Quirúrgicos del Sistema Digestivo , Intestinos , Daño por Reperfusión/cirugía , Solución Salina/farmacología , Animales , Femenino , Hidrógeno , Intestinos/efectos de los fármacos , Intestinos/cirugía , Ratas , Ratas Wistar , Solución Salina/química
20.
J Surg Res ; 155(1): 116-24, 2009 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-19111325

RESUMEN

BACKGROUND: Thyroid hormone acts on structural and functional maturation of the mammalian small intestine, mitochondrial pathways, and several protein-gene interactions. Therefore, it is one of the most important regulators of intestinal epithelial differentiation. The aim of the study was to evaluate the effects of thyroid hormone on the adaptation in an experimental model of short bowel syndrome. METHODS: Rats were divided into three groups: sham (bowel transection and anastomosis), short bowel syndrome-saline (75% bowel resection and anastomosis), and short bowel syndrome-thyroid hormone (75% bowel resection and anastomosis, and was administered triiodothyronine). The evaluation of adaptation parameters, histopathological and biochemical analysis were performed in all groups. RESULTS: Triiodothyronine treatment resulted in a significant increase in adaptation parameters, villus height-crypt depth, and enterocyte proliferation, whereas significant decrease was seen in apoptotic index in jejunum. Enterocyte proliferation and most of the adaptation parameters changed significantly in ileum following the treatment with triiodothyronine as in jejunum. The changes in ileal villus height-crypt depth and apoptotic index were not statistically significant. Serum levels of free triiodothyronine were lower in the short bowel syndrome-saline group. CONCLUSIONS: Our results suggest that thyroid hormone treatment in the hypothyroid phase of SBS enhances intestinal adaptive response.


Asunto(s)
Adaptación Fisiológica/efectos de los fármacos , Apoptosis/efectos de los fármacos , Enterocitos/efectos de los fármacos , Síndrome del Intestino Corto/tratamiento farmacológico , Triyodotironina/uso terapéutico , Anastomosis Quirúrgica , Animales , Proliferación Celular/efectos de los fármacos , Íleon/patología , Íleon/cirugía , Yeyuno/patología , Yeyuno/cirugía , Masculino , Ratas , Ratas Wistar , Síndrome del Intestino Corto/patología , Triyodotironina/farmacología
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