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BACKGROUND: Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS: We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS: Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P<0.001). A total of 88% of patients who received the selected propranolol regimen showed improvement by week 5, versus 5% of patients who received placebo. A total of 10% of patients in whom treatment with propranolol was successful required systemic retreatment during follow-up. Known adverse events associated with propranolol (hypoglycemia, hypotension, bradycardia, and bronchospasm) occurred infrequently, with no significant difference in frequency between the placebo group and the groups receiving propranolol. CONCLUSIONS: This trial showed that propranolol was effective at a dose of 3 mg per kilogram per day for 6 months in the treatment of infantile hemangioma. (Funded by Pierre Fabre Dermatologie; ClinicalTrials.gov number, NCT01056341.).
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Antagonistas Adrenérgicos beta/administración & dosificación , Hemangioma/tratamiento farmacológico , Propranolol/administración & dosificación , Administración Oral , Antagonistas Adrenérgicos beta/efectos adversos , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Esquema de Medicación , Femenino , Humanos , Hipotensión/inducido químicamente , Lactante , Masculino , Propranolol/efectos adversos , Resultado del TratamientoRESUMEN
We present a case of a 4-year-old girl with ocular venous malformation in her left eye. Over a one-year follow up, the symptoms progressed and sclerotherapy with bleomycin was performed. The bleomycin solution was injected directly into the abnormal vessel under visual control, which lead to a complete obliteration of malformation in a long-term follow-up.
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Bleomicina/uso terapéutico , Oftalmopatías/terapia , Escleroterapia , Malformaciones Vasculares/terapia , Venas/efectos de los fármacos , Preescolar , Femenino , HumanosRESUMEN
BACKGROUND: In the process of diagnosis and treatment of fractures, an X-ray study is typically performed. In modern medicine very important is the development of new diagnostic methods without adverse effects on the body. One of such techniques is ultrasound imaging. It has a high value in imaging most areas of the body, including the musculoskeletal system. Reports on the use of ultrasound in the evaluation of the callus are rare and this could be a method equivalent to or even better than standard radiographs. The aim of the study was to analyze the correlation of ultrasound with radiographs in imaging of callus formation after fractures of long bones in children and to analyze the correlation of vascular resistance index (RI) and the degree of vascularization of the callus with a subjective radiological assessment of the bone union quality. MATERIAL/METHODS: The prospective study was planned to qualify 50 children treated for long bones fractures of the arm, forearm, thigh and lower leg. Ultrasound diagnosis was carried out using a Philips iU22 camera equipped with a linear probe with 17-5-MHz resolution and MSK Superficial program. During ultrasound examination measurements of the callus were performed. Using the Power Doppler callus vascularity was visualized and vascular resistance index (RI) was measured. The same measurements were made within the corresponding area of the healthy limb. The results obtained by ultrasound were compared with radiograph measurements and with the subjective assessment of the callus quality. RESULTS: Preliminary results were developed on a group of 24 patients, where 28 fractured bones and 28 corresponding healthy bones were examined. Fifteen boys and 9 girls participated in the study. The average age at injury was, respectively, 11 and 9 years. In both groups fractures without displacement were the most frequent. A similar frequency was observed in fractures requiring reposition and subperiosteal fractures. In contrast, fractures with a slight displacement of the fragments, were 3 times more common in girls. Statistical analysis of the measurements of length and width of the callus demonstrated that the differences between results obtained in the ultrasound in comparison with X-rays were not statistically significant. Moreover, preliminary results showed a significantly higher degree of vascularization of the callus than of the healthy periosteum. CONCLUSIONS: Preliminary results indicate the high efficacy of ultrasound in the evaluation of callus formation after fractures of long bones in children and the possibility of its alternative use to X-ray examinations.
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BACKGROUND: Vascular anomalies are usually diagnosed through their clinical picture and history. The purpose of this study was to assess the role of MR imaging in initial assessment of cervicofacial vascular anomalies in children. MATERIAL/METHODS: Twenty pediatric patients with vascular anomalies located in the cervicofacial region underwent MRI examination in our department. Images were evaluated for lesion detectability and its signal characteristics (on T1w, T2w images with fat suppression and contrast enhanced T1w sequences); the extent of the lesions and surrounding tissue involvement were also assessed. RESULTS: In the studied group MR images revealed all anomalies and provided information of their anatomic extent and invasion of surrounding anatomic structures. Nine hemangiomas and six venous malformations were found among studied patients. Two children had multiloculated lesions corresponding to lymphatic malformations. One examination visualized a lesion consisting mainly of dilated vascular channels with an apparent feeding artery, which was consistent with arteriovenous malformation. Two remaining lesions were mixed malformations. Nine patients had lesions limited to subcutaneous tissue. Two masses infiltrated bone structures. There was muscle involvement found in nine cases. CONCLUSIONS: MR imaging is a well-established method for detection and monitoring of vascular anomalies in children. With ultrasound used mostly for initial diagnosis and additional flow assessment, angiography viewed as an invasive therapeutic method and computed tomography used only in specific situations due to its high irradiation dose, magnetic resonance is the best imaging method used in differential diagnosis and topographical characterization of vascular malformations and tumors of cervicofacial area in pediatric patients. Noninvasively and without irradiation, it enables evaluation of the extent and characteristics of lesions and planning proper therapeutic strategy.
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<b><br>Aim:</b> The aim of this retrospective study was to evaluate our treatment for pilonidal disease in adolescent patients, which uses ultrasonography, minimally invasive pit-picking, and Nd:YAG laser therapy.</br> <b><br>Material and methods:</b> We included 52 of 147 patients treated between June 2017 and December 2020. The patients underwent pit-picking procedures and 6-10 Nd:YAG treatments. The remnants of the cysts were removed by laser therapy, which provided easy epilation. Each patient underwent multiple ultrasound examinations during the therapy to uncover any potential newly formed asymptomatic sinuses. When such issues were identified, the pit-picking procedure was repeated on those sinuses.</br> <b><br>Results:</b> A total of 52 patients were included in this study with a 1-year follow-up following the procedure. There were 49 symptom-free patients (96%). One patient underwent surgery in another hospital because of a recurrence and 1 had a pilonidal disease relapse (4%). In the follow-up period, asymptomatic cysts were found in 2 other patients by ultrasound examination. They were all treated with a pit-picking procedure in the outpatient department with good results.</br> <b><br>Conclusions:</b> Combining sequentially repeated pit-picking procedures and Nd:YAG laser therapy is an effective treatment method for adolescent pilonidal disease. Simultaneous Nd:YAG laser therapy enables efficacious epilation of the intergluteal cleft. Repeatable ultrasonography examinations allow for early diagnosis of possible pilonidal sinus relapse.</br>.
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Remoción del Cabello , Láseres de Estado Sólido , Seno Pilonidal , Adolescente , Humanos , Remoción del Cabello/métodos , Láseres de Estado Sólido/uso terapéutico , Estudios Retrospectivos , Recurrencia Local de Neoplasia , Seno Pilonidal/diagnóstico por imagen , Seno Pilonidal/cirugía , Ultrasonografía , Recurrencia , Ultrasonografía IntervencionalRESUMEN
Systemic inflammatory response syndrome (SIRS) is defined as the systemic host response to infection or a non-infectious factor. The purpose of this study was to evaluate the involvement of reactive oxygen species (ROS) in severe inflammation and to assess the discrimination strength of the neutrophil BURSTTEST assay regarding its etiology in three groups of patients (sepsis, burns, and bone fractures) who met the SIRS criteria. The neutrophil activation (respiratory burst of granulocytes as well as p55 and p75 tumor necrosis factor (TNF-α) receptor expression) was evaluated twice using flow cytometry, and the results were compared with healthy controls and among SIRS subjects. A decreased oxygen metabolism in neutrophils after E.coli stimulation and increased TNF-α receptor expression were found in septic and burned patients on admission, while ROS production augmented and TNF-α receptor expression diminished with the applied therapy. The significant differences in neutrophil respiratory burst intensity among septic and burned patients and those with sepsis and bone fractures were found (however, there were not any such differences between patients with thermal and mechanical injuries). This study indicates that the neutrophil BURSTTEST evaluation might be a clinically reliable marker for differentiating the SIRS etiology.
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Neutrófilos , Sepsis , Niño , Humanos , Inflamación , Estallido Respiratorio , Factor de Necrosis Tumoral alfaRESUMEN
UNLABELLED: Vascular endothelial growth factor (VEGF) and its receptors were postulated to be involved in pathogenesis of infantile hemangioma. The aim of this study was to determine the serum levels of VEGF and soluble VEGF receptors (sVEGFR1/sVEGFR2) in children with hemangiomas. MATERIALS AND METHODS: Thirty-eight children with infantile hemangiomas (25 proliferating, 13 involuting) and 34 healthy children were included in the study. sVEGFR1 and sVEGFR2 serum levels in peripheral blood and in vascular tumors were determined with ELISA test. RESULTS: sVEGFR1 serum levels were slightly lower in hemangioma patients (p = 0.049). No significant differences in sVEGFR2 levels were observed in any study group. VEGF levels did differ significantly, with median level being 364.05 pg/ml in hemangioma patients and 107.40 pg/ml in the control group (p < 0.0001). CONCLUSIONS: The obtained results suggest that VEGF is involved in hemangioma angiogenesis but that soluble VEGFRs marginally influence this process. Lower serum levels of sVEGFR1 in hemangioma patients indicate the possible dysregulation between VEGFR1 and VEGFR2 receptors.
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Hemangioma/sangre , Factor A de Crecimiento Endotelial Vascular/sangre , Receptor 1 de Factores de Crecimiento Endotelial Vascular/sangre , Receptor 2 de Factores de Crecimiento Endotelial Vascular/sangre , Biomarcadores , Ensayo de Inmunoadsorción Enzimática , Femenino , Hemangioma/patología , Humanos , Lactante , Masculino , Neovascularización Patológica , SolubilidadRESUMEN
UNLABELLED: The pathogenesis of hemangiomas still remains poorly understood. Dysregulation of angiogenesis has been proposed to play a central role in hemangioma pathogenesis. The aim of our study was to determine the peripheral and local serum levels of VEGF in patients with hemangiomas and vascular malformations. MATERIAL AND METHODS: The study group consisted of 52 children with infantile hemangioma (33 with proliferative lesions, 19 with involuting lesions), 14 children with vascular malformations and 36 healthy children. VEGF serum levels were analyzed by an ELISA assay and the values between the groups were compared. RESULTS: The serum peripheral VEGF concentrations in children with proliferative hemangiomas were significantly higher than in patients with involuting hemangiomas, vascular malformations and controls. There was no correlation between the measured cytokine level, hemangioma size, and the age of the patients. The local serum VEGF levels in 29 children with hemangiomas were distinctly lower than in the peripheral blood, both in 20 proliferating hemangiomas (p<0.0001) and 9 involuting ones (p=0.007); and the difference between females and males was non-significant (NS p=0.06). CONCLUSIONS: (1) VEGF serum levels vary in the different phases of hemangioma growth and may help to distinguish hemangiomas from vascular malformations; (2) obtained local results may support the intrinsic theory of endothelial cell proliferation in hemangiomas.
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Endotelio Vascular , Hemangioma , Neovascularización Patológica , Neoplasias Cutáneas , Factor A de Crecimiento Endotelial Vascular/sangre , Niño , Preescolar , Endotelio Vascular/metabolismo , Endotelio Vascular/patología , Femenino , Hemangioma/metabolismo , Hemangioma/patología , Humanos , Lactante , Masculino , Neovascularización Patológica/metabolismo , Neovascularización Patológica/patología , Neoplasias Cutáneas/metabolismo , Neoplasias Cutáneas/patologíaRESUMEN
Impaired balance between proangiogenic and antiangiogenic factors has been implicated in the development of hemangiomas. Elevated vascular endothelial growth factor serum levels and basic fibroblastic growth factor urine levels in patients with proliferating hemangiomas were reported. However, whether these growth factors can be used for the differential diagnosis of vascular anomalies or assessment of the clinical course of hemangiomas has yet to be determined. We report here our preliminary results of serum vascular endothelial growth factor and basic fibroblastic growth factor levels as an aid in the diagnosis of hemangiomas and in the follow up of patients with this lesion. Twenty two children with infantile hemangioma (13 with proliferating hemangiomas, nine with involuting lesions), five children with vascular malformations, and 25 healthy children study group. Vascular endothelial growth factor and basic fibroblastic growth factor serum levels were analyzed by an ELISA assay. The serum vascular endothelial growth factor concentrations in children with proliferating hemangiomas were significantly higher than in patients with involuting hemangiomas, vascular malformations and healthy patients. The serum basic fibroblastic growth factor concentrations were low and similar in all patients with no statistical correlation between study groups. We concluded that (i) ELISA can easily determine vascular endothelial growth factor concentrations in different phases of hemangioma growth and help distinguishing them from vascular malformations. (ii) A potential role for vascular endothelial growth factor in the pathophysiology of hemangiomas is probable.
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Factor 2 de Crecimiento de Fibroblastos/sangre , Hemangioma/sangre , Hemangioma/diagnóstico , Neoplasias Cutáneas/sangre , Neoplasias Cutáneas/diagnóstico , Factor A de Crecimiento Endotelial Vascular/sangre , Malformaciones Vasculares/sangre , Adolescente , Niño , Preescolar , Diagnóstico Diferencial , Ensayo de Inmunoadsorción Enzimática , Femenino , Hemangioma/patología , Humanos , Lactante , Masculino , Concentración Osmolar , Neoplasias Cutáneas/patologíaRESUMEN
BACKGROUND: Infantile hemangioma (IH) is the most common vascular tumor of childhood and infancy. It is distinguished by rapid proliferation of endothelial cells during the first year of life followed by spontaneous regression thereafter. One of the possible factors responsible for the IH development is vascular endothelial grow factor (VEGF). The purpose of this study was to evaluate the influence of selected polymorphisms in the genes coding for VEGF-A (+405 G/C, rs2010963; +936 C/T, rs3025039) and its receptor VEGFR-2 (+1416 T/A, rs1870377; -271 G/A, rs7667298) on the susceptibility to infantile hemangioma. METHODS: We performed a case-control study of 99 Polish children hospitalized due to IH and compared them with matched healthy control subjects. The polymorphisms were ascertained through genotyping by PCR-RFLP assay, PCR-HRM, or the allelic discrimination method. RESULTS: The study revealed a lower odds of infantile hemangioma in individuals with GG genotype or G allele for +405 G/C VEGF-A polymorphism (ORdis = 0.52, P = 0.023 and ORdis = 0.63, P = 0.025, respectively). No association was observed for the remaining VEGF and VEGFR-2 polymorphisms and IH risk. CONCLUSIONS: In our study, none of the investigated VEGF-A and VEGFR-2 genes polymorphisms was found to be an independent prognostic marker for infantile hemangioma. However, there is evidence that individuals carrying at least one G allele of +405 G/C VEGF-A polymorphism have significantly lower risk of IH.
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Hemangioma Capilar/genética , Síndromes Neoplásicos Hereditarios/genética , ARN Mensajero/metabolismo , Factor A de Crecimiento Endotelial Vascular/genética , Receptor 2 de Factores de Crecimiento Endotelial Vascular/genética , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Expresión Génica , Predisposición Genética a la Enfermedad , Genotipo , Humanos , Lactante , Recién Nacido , Masculino , Polimorfismo de Nucleótido Simple , Factores de RiesgoRESUMEN
BACKGROUND AND OBJECTIVES: There is no consensus on optimal treatment duration for propranolol in infantile hemangioma (IH). We evaluated the efficacy and safety of oral propranolol solution administered for a minimum of 6 months up to a maximum of 12 months of age in high-risk IH. METHODS: This single-arm, open-label, phase 3 study was conducted in patients aged 35 to 150 days with high-risk IH in 10 hospitals between 2015 and 2017. The study comprised a 6-month initial treatment period (ITP) plus continuation up to 12 months of age if complete success was not achieved, a follow-up, and a retreatment period. Patients received oral propranolol twice daily (3 mg/kg per day). The primary end point was the success rate at the end of the ITP. Furthermore, the persistence of IH response and efficacy of retreatment was evaluated. RESULTS: The success rate after 6 months of treatment was 47%, increasing to 76% at the end of the ITP. Of the patients who achieved success, 68% sustained success for 3 months without treatment, and 24% required retreatment. Of the 8 patients who were retreated, 7 achieved success. Adverse events, reported by 80% of patients, were mild, which were expected in this population or known propranolol side effects. CONCLUSIONS: Oral propranolol administered beyond 6 months and up to 12 months of age meaningfully increases the success rate in high-risk IH. Success was sustained in most patients up to 3 months after stopping treatment. Retreatment was efficacious, and the safety profile satisfactory.
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Antagonistas Adrenérgicos beta/administración & dosificación , Hemangioma/diagnóstico , Hemangioma/tratamiento farmacológico , Propranolol/administración & dosificación , Administración Oral , Esquema de Medicación , Femenino , Humanos , Lactante , Masculino , Factores de Riesgo , Resultado del TratamientoRESUMEN
Propranolol is a widely-known beta-blocker approved for treating infantile hemangiomas (IH). The mechanisms behind the spectacular IH involution after propranolol treatment remain unclear. Recently, there is strong evidence of overexpression of numerous angiogenic factors in IH tissues, and it is reported that propranolol influences their pathways. However, a number of MMPs studies is highly limited. Here, for the first time, we propose a comprehensive approach by analyzing the expression levels of metalloproteinases-2/9 (MMPs-2/9) and tissue metalloproteinase inhibitor-2 (TIMP-2) in vivo on both, molecular and immunohistochemical levels, and in both, IH tissues and in the serum of IH patients, and relates the obtained results to the tumor's biology and systemic propranolol treatment. MATERIAL AND METHODS: MMPs-2/9 and TIMP-2 were analyzed in 71 IH tissue samples using immunohistochemistry and real-time PCR, and in 50 serum samples of IH patients by ELISA. RESULTS: Significantly lower MMPs-2/9 and higher TIMP-2 levels were observed in IH tissues on the mRNA level as well as lower serum MMP-2 concentration among the treated individuals. CONCLUSION: MMPs-2/9 and TIMP-2 are both involved in the biology of IH and the propranolol pathways enabling their antiangiogenic properties. The most reliable method of IH examination appears to be direct MMPs-2/9 mRNA evaluation in tumor tissue; and MMP-2 evaluation in patients' serum is a valuable complement to it. Tissue and serum mRNA MMPs assessment may represent a suitable novel biomarker identifying tumor progression and involution processes with potential clinical impact in IH as well as in cancer disease.
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Biomarcadores de Tumor/metabolismo , Hemangioma/diagnóstico , Metaloproteinasa 2 de la Matriz/metabolismo , Metaloproteinasa 9 de la Matriz/metabolismo , Propranolol/uso terapéutico , Biomarcadores de Tumor/genética , Preescolar , Femenino , Hemangioma/tratamiento farmacológico , Humanos , Inmunohistoquímica , Lactante , Recién Nacido , Masculino , Metaloproteinasa 2 de la Matriz/genética , Metaloproteinasa 9 de la Matriz/genética , Medicina de Precisión , Inhibidor Tisular de Metaloproteinasa-2/genética , Inhibidor Tisular de Metaloproteinasa-2/metabolismo , TranscriptomaRESUMEN
BACKGROUND: Infantile hemangiomas (IHs) are the most common benign tumors of childhood. They are characterized by a unique clinical course with two phases, proliferation and involution, which are followed by regression. The therapy of infantile hemangiomas was revolutionized in 2008 by the introduction of propranolol, however, the mechanism of its influence on hemangiomas remains unclear. METHODS: The study included 71 patients with IHs, 27 of whom were treated with propranolol while the remaining 44 were used as a comparative group. The expression of Bcl-2, Bax and Caspase3 was determined with immunohistochemistry and mRNA of Bax, Bcl-2 and Caspase3 were assessed with the use of RT-PCR. RESULTS: Both methods revealed a statistically significant decrease in Bcl-2 expression and an increase in Bax in IHs tissues after propranolol treatment. CONCLUSIONS: The results obtained for Bax and Bcl-2 proteins may indicate a link between the effect of propranolol and apoptosis. Higher Bax and lower Bcl-2 expression in the propranolol treated group indicates a strong pro- apoptotic action countering any anti-apoptotic activity; apoptosis was indicted in IH tissue as a potential result of propranolol treatment, with potential clinical impact in other tumors.
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Inhibidores de la Angiogénesis/uso terapéutico , Caspasa 3/metabolismo , Hemangioma/tratamiento farmacológico , Neovascularización Patológica/tratamiento farmacológico , Propranolol/uso terapéutico , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo , Proteína X Asociada a bcl-2/metabolismo , Apoptosis/efectos de los fármacos , Caspasa 3/genética , Células Cultivadas , Femenino , Humanos , Inmunohistoquímica , Lactante , Recién Nacido , Masculino , Proteínas Proto-Oncogénicas c-bcl-2/genética , Proteína X Asociada a bcl-2/genéticaRESUMEN
UNLABELLED: In the last few years propranolol has revolutionized infantile hemangioma therapy. This nonselective ß bloker has been proven to be safe and effective but the molecular bases of its actions remain unclear. One of debated theories holds that propranolol may inhibit angiogenesis and induce apoptosis. To investigate this claim, this study aims to analyze the serum and tissue profiles of VEGF and VEGRR1/2 in patients treated with propranolol. MATERIALS AND METHODS: To assess the expression if VEGF and VEGRR1/2 we used three independent methods. First we analyzed serum VEGF levels in 50 children with IH before and 3 months after the therapy using ELISA test (I.). Then we used immunohistochemistry to evaluate tissue expression of VEGF and VEGFR1/2 in IH treated (n=27) and not treated (n=45) with propranolol (II.). Finally we assessed mRNA of VEGF and VEGFR1/2 in the same patients as in part II (III.). RESULTS: (I) There was no distinct decrease of VEGF level in children with IH after propranolol treatment. (II) We found no significant difference in VEGFR1 and VEGFR2 expression in hemangiomas from the study and control group. The expression of VEGF was even higher than before therapy. (III) VEGF and VEGFR1 mRNA expression was significantly lower in IH tissue after propranolol treatment compared to those without treatment. VEGFR2 demonstrated no differences in expression between the two groups. CONCLUSIONS: The obtained results show distinct discrepancies between in vitro and clinical studies as well as among different methods used for analyzing the same phenomenon. Only VEGF and VEGFR1 expression in mRNA studies may prove the proposed theory of antiangiogenic properties of propranolol. Other results do not confirm it and remain inconsistent with the fantastic clinical response to this medication.
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Inhibidores de la Angiogénesis/uso terapéutico , Hemangioma/tratamiento farmacológico , Neoplasias de la Parótida/tratamiento farmacológico , Propranolol/uso terapéutico , Factor A de Crecimiento Endotelial Vascular/metabolismo , Receptor 1 de Factores de Crecimiento Endotelial Vascular/metabolismo , Receptor 2 de Factores de Crecimiento Endotelial Vascular/metabolismo , Femenino , Perfilación de la Expresión Génica , Hemangioma/diagnóstico , Hemangioma/mortalidad , Humanos , Inmunohistoquímica , Lactante , Masculino , Neoplasias de la Parótida/diagnóstico , Neoplasias de la Parótida/mortalidad , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
The large neurofibromatic tumours developing in head, neck and abdominal cavity in children with neurofibromatosis type 1 make a serious clinical problem. In this report the case of 12 years old boy with benign abdominal tumor leading from nerve roots (S1-S2) is presented. He has been treated for 6 years. The other malignant neoplasms, additional congenital defects or neurological dysfunctions were not confirmed. The primary resection was not radical and adjuvant therapy (chemotherapy, hormonotherapy) was ineffective. Due to the progression and not coming to hospital in next 2 years severe obstructive uropathy developed leading to complete destruction of the left kidney. Bilateral ureterocutaneostomies were performed. Despite of slow tumor grow the patient is stable now. However he needs regular calibrations of the right ureterocutaneostomy due to the progressive contraction. Further treatment of this case remains open question.
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Neoplasias Abdominales/complicaciones , Hidronefrosis/etiología , Neurofibromatosis 1/complicaciones , Neoplasias Abdominales/diagnóstico , Neoplasias Abdominales/terapia , Niño , Humanos , Hidronefrosis/diagnóstico , Hidronefrosis/terapia , Masculino , Neurofibromatosis 1/terapia , UreterostomíaRESUMEN
BACKGROUND: Venous malformations are the second most common congenital vessel anomaly. In our hospital, we conduct up to 30 sclerotherapies with 1-3% aethoxysclerol annually in children of all ages. The procedure is invasive and painful and therefore requires general anaesthesia. CASE REPORT: A 16-year-old girl underwent sclerotherapy of a vast vascular malformation of her left leg, pelvis, abdominal cavity and thorax. After induction of general anaesthesia and positioning for the procedure, she presented with hypotonic shock with sinus tachycardia and sudden decrease in her ETCO2. Her skin became pale and cold. The venous malformation became distended. The incident was caused by redistribution of the blood to the malformation, which is believed to have been triggered by the volatile anaesthetic. After discontinuation of the sevoflurane, modification of anaesthesia and the administration of ephedrine and fluids, hypotonia was successfully treated. The patient's state was stabilised, her clinical measurements returned to normal, and the procedure was continued. Her later course was uneventful. Blood gas analysis in post-anaesthesia care unit revealed mild, compensated metabolic acidosis. No electrolyte abnormalities were present. CONCLUSION: Volatile anaesthetics and propofol decrease the systemic vascular resistance and cause vasodilatation. Our patient presented with hypotonic shock due to the redistribution of blood to the dilated venous malformation, which developed after the use of standard concentration of sevoflurane. Intravenous anaesthetics were administered during induction and might have increased that effect. Although we found no similar reports, we believe that patients with vast venous malformations can experience such complications after the use of volatile anaesthetics, especially in high concentrations.
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Anestesia General/efectos adversos , Anestésicos por Inhalación/efectos adversos , Anestésicos Intravenosos/efectos adversos , Éteres Metílicos/efectos adversos , Propofol/efectos adversos , Choque/inducido químicamente , Malformaciones Vasculares/complicaciones , Adolescente , Femenino , Humanos , Escleroterapia , Sevoflurano , Choque/complicacionesRESUMEN
BACKGROUND: Infantile haemangiomas are the most common vascular tumours in children. Since 2008 the application of propranolol has been a promising therapy in the management of haemangiomas. AIM OF THE STUDY: Analysis of the patients with infantile haemangiomas treated with propranolol. MATERIAL AND METHODS: Between June 2009 and December 2010 in Department of Pediatric Surgery and Oncology Medical University of Lodz, 35 children with infantile haemangiomas (29 females and 6 males) were treated with propranolol. Therapy was initiated in age of 2-15 months (mean 4.5). All infantile haemangiomas were in a proliferative phase. In 27 children lesions were located in the head and neck, 2 of them were located in the orbital region and 3 penetrated into the orbit. In 5 children haemangiomas were located in the trunk, with 3 in the perineum and 3 in limbs. In 2 children the PHACE Syndrome was diagnosed. In one of these cases exploratory laparatomy revealed jejunal haemangiomatosis. The indication for propranolol application was impairment of physiological functions in 23 cases, cosmetic defect in 8 and ulceration in 4. The duration of treatment was 4 to 12 months (mean 7.5-months). The change of haemangioma volume, density and colour were evaluated. Reduction of Ë haemangioma volume was assessed as very good response, 1/3 as good response, and 1/4 as poor. RESULTS: In 7 patients therapy has been finished. In all cases decrease in density, volume and fading was observed. Very good result was achieved in 27 patients, good in 5, poor in 3. Recurrence of haemangioma appeared in two patients after termination of treatment. A spectacularly good result was achieved in the child with PHACE syndrome and in one with jejunal haemangiomatosis. CONCLUSIONS: Propranolol therapy is safe and effective in children with infantile proliferating haemangiomas. It can be the treatment of choice in cases with impairment of physiological functions or severe cosmetic defect. Election and therapy of the children should be carried out in highly reference centres.
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Antagonistas Adrenérgicos beta , Propranolol , Hemangioma , Humanos , Lactante , Recurrencia Local de Neoplasia/tratamiento farmacológicoRESUMEN
OBJECTIVES: Dysregulation of angiogenesis has been proposed to play a central role in hemangioma pathogenesis. The aim of the study was to determine the peripheral and local serum levels of bFGF in patients with hemangiomas and vascular malformations (VM). DESIGN AND METHODS: The study group consisted of 52 children with infantile hemangioma, 14 with VM and 36 healthy patients. bFGF serum levels were analyzed by an ELISA assay. Urinary bFGF was determined in 11 individuals with hemangioma. RESULTS: The serum peripheral bFGF concentrations in children with proliferating hemangiomas were lower than in healthy controls (p=0,03). There was no correlation between the measured cytokine level and hemangioma size, as well as patients' age. The serum local bFGF levels in 29 children with hemangiomas were higher than in the peripheral blood (p=0.022). Urinary bFGF in hemangioma patients did not differ statistically from healthy controls. CONCLUSIONS: (1) Determination of bFGF serum levels is not helpful in differentiating the phases of hemangioma growth and distinguishing hemangiomas from VM; (2) serum levels of bFGF cannot distinguish between extrinsic and intrinsic theories of endothelial cell proliferation in hemangiomas.
Asunto(s)
Células Endoteliales/patología , Factor 2 de Crecimiento de Fibroblastos/sangre , Hemangioma Cavernoso/sangre , Malformaciones Vasculares/sangre , Adolescente , Proliferación Celular , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Femenino , Factor 2 de Crecimiento de Fibroblastos/orina , Hemangioma Cavernoso/diagnóstico , Hemangioma Cavernoso/orina , Humanos , Lactante , Masculino , Malformaciones Vasculares/diagnóstico , Malformaciones Vasculares/orinaRESUMEN
PURPOSE: The aim of this study was to investigate expression of estrogen receptor alpha (ERalpha) and progesterone receptor (PR) in paratesticular tissues obtained from boys with undescended testes. MATERIALS AND METHODS: A total of 65 boys with unilateral cryptorchidism and failed human chorionic gonadotropion treatment underwent orchiopexy. A small sample of gubernaculum, cremasteric muscle and processus vaginalis was obtained. A total of 57 boys who underwent inguinal hernia repair served as the control group. All boys in the control group had testes in the scrotum. The expression of estrogen receptor alpha and progesterone receptor was measured by counting the number of ERalpha or PR positive cells detected by immunohistochemical analysis. RESULTS: ERalpha and PR density was higher in cremasteric muscle and processus vaginalis obtained from boys with undescended testes than in the control group. Density of progesterone receptor in the examined groups was lower than the density of estrogen receptor. CONCLUSIONS: ERalpha and PR are expressed in paratesticular tissues important for normal testicular descent. ERalpha was over expressed in cremasteric muscle and processus vaginalis in boys with undescended testes previously treated with human chorionic gonadotropin.