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1.
An Bras Dermatol ; 94(5): 521-526, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31777351

RESUMO

BACKGROUND: The use of monoethanolamine oleate 5% is effective for the treatment of vascular malformations with low blood flow. OBJECTIVES: To report a case series of vascular malformations in the mouth and oral cavity treated with monoethanolamine oleate 5%. METHODS: A retrospective descriptive study was performed in electronic patient charts covering seven years. Patient demographics, diagnostic resources, lesion site, size, and number of applications of monoethanolamine oleate 5% were collected. RESULTS: A total of 21 vascular malformations were recorded, located mostly on the lower lip (52.3%) and resolved in a single application in 14 patients. The authors found 19 patients treated with sclerotherapy. Thirteen were women and six were men, with a mean age of 61 years. STUDY LIMITATION: Small sample size. CONCLUSIONS: Sclerotherapy is an effective treatment for vascular malformations of the lips and oral cavity, with resolution after only one or two applications (n=16).


Assuntos
Doenças da Boca/terapia , Ácidos Oleicos/administração & dosagem , Soluções Esclerosantes/administração & dosagem , Escleroterapia/métodos , Malformações Vasculares/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estética , Feminino , Humanos , Injeções Intralesionais , Lábio/irrigação sanguínea , Lábio/patologia , Masculino , Pessoa de Meia-Idade , Doenças da Boca/patologia , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Malformações Vasculares/patologia , Adulto Jovem
2.
Medicine (Baltimore) ; 98(44): e17798, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689858

RESUMO

RATIONALE: Dieulafoy disease of the bronchus is a rare vascular deformity. To the best of our knowledge, reports of these involving both lung vascular are hitherto absent. PATIENT CONCERNS: A 67-year-old male was admitted to our department due to agnogenic hemoptysis. DIAGNOSES: Bronchoscopy was performed and some smooth, pulsatile nodular lesions were found in the middle and lower lobes, Computed tomography angiography of the bronchial artery confirmed a left bronchial artery arising from the aortic arch at T4 level, and both bronchial arteries were dilated and tortuous. INTERVENTIONS: Bronchial artery embolization was performed successfully. OUTCOMES: The patient was discharged with no hemoptysis. In addition, patient is under follow-up until today without any further incidents. LESSONS: This case reminds us that Dieulafoy disease of the bronchus could be a potential etiology for unexplained hemoptysis. The clinician should be aware of this disease when bronchoscopy revealed multiple some smooth, pulsatile nodular lesions, thereafter, bronchoscope biopsy should be avoided, as it could lead to fatal hemoptysis.


Assuntos
Artérias Brônquicas/anormalidades , Broncopatias/complicações , Hemoptise/etiologia , Malformações Vasculares/complicações , Idoso , Artérias Brônquicas/cirurgia , Broncopatias/patologia , Broncopatias/cirurgia , Broncoscopia/métodos , Angiografia por Tomografia Computadorizada , Hemoptise/cirurgia , Humanos , Pulmão/patologia , Masculino , Malformações Vasculares/patologia , Malformações Vasculares/cirurgia
3.
Medicine (Baltimore) ; 98(33): e16802, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31415390

RESUMO

Impact of arterial stiffness on aortic morphology has not been well evaluated. We sought to investigate the association of brachial-ankle pulse wave velocity (baPWV) with aortic calcification and tortuosity.A total of 181 patients (65.4 ±â€Š10.4 years, males 59.7%) who underwent computed tomographic angiography and baPWV measurement within 1 month of study entry were retrospectively reviewed. Aortic calcification was quantified by the calcium scoring software system. Aortic tortuosity was defined as the length of the midline in the aorta divided by the length of linear line from the aortic root to the distal end of the thoraco-abdominal aorta. In simple correlation analyses, baPWV was correlated with aortic calcification (r = 0.36, P < .001) and tortuosity (r = 0.16, P = .030). However, these significances disappeared after controlling for confounders in multivariate analyses. Factors showing an independent association with aortic calcification were age (ß = 0.37, P < .001), hypertension (ß = 0.19, P = .003), diabetes mellitus (ß = 0.12, P = .045), smoking (ß = 0.17, P = .016), and estimated glomerular filtration rate (ß = -0.25, P = .002). Factors showing an independent association with aortic tortuosity were age (ß = 0.34, P < .001), body mass index (ß = -0.19, P = .018), and diabetes mellitus (ß = -0.21, P = .003).In conclusion, baPWV reflecting arterial stiffness was not associated with aortic calcification and tortuosity. Traditional cardiovascular risk factors were more influential to aortic geometry. Further studies with a larger sample size are needed to confirm our results.


Assuntos
Aorta/patologia , Artérias/anormalidades , Instabilidade Articular/fisiopatologia , Dermatopatias Genéticas/fisiopatologia , Calcificação Vascular/fisiopatologia , Malformações Vasculares/fisiopatologia , Rigidez Vascular/fisiologia , Idoso , Índice Tornozelo-Braço , Aorta/fisiopatologia , Artérias/patologia , Artérias/fisiopatologia , Índice de Massa Corporal , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Instabilidade Articular/patologia , Masculino , Pessoa de Meia-Idade , Análise de Onda de Pulso , Estudos Retrospectivos , Dermatopatias Genéticas/patologia , Calcificação Vascular/patologia , Malformações Vasculares/patologia
4.
Urology ; 131: 223-227, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31163184

RESUMO

Glanular venous malformations are uncommon in pediatric patients. The diagnosis can be easily achieved by observation, even if color Doppler ultrasound is useful for a better characterization. Abdomino-pelvic MRI is necessary to assess the extension of complex lesions and check for associated anomalies. Several therapeutic options are reported in literature. We report 3 paediatric cases successfully treated by surgery with no complications and functional sequelae. Cosmetic results were satisfactory, with minimal surgical scarring. In our opinion, surgery for small glanular venous malformations is indicated within puberty to prevent traumatic bleeding and psychological impact.


Assuntos
Doenças do Pênis/patologia , Doenças do Pênis/cirurgia , Pênis/irrigação sanguínea , Malformações Vasculares/patologia , Malformações Vasculares/cirurgia , Criança , Pré-Escolar , Humanos , Masculino
5.
Semin Ophthalmol ; 34(5): 353-358, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31165650

RESUMO

Purpose: To investigate the clinical characteristics of perifoveal exudative vascular anomalous complex (PEVAC) in Korean patients. Methods: This retrospective study included 8 eyes (8 patients) diagnosed with PEVAC. Demographic characteristics of the patients, fundus photography, optical coherence tomography (OCT), fluorescein angiography, and indocyanine-green angiography (ICGA) findings were analyzed. Available follow-up data were also reviewed. Results: The mean ± standard deviation age was 61.0 ± 11.0 years and mean logarithm of minimal angle of resolution (logMAR) best-corrected visual acuity was 0.15 ± 0.14. PEVAC was noted in 2 eyes with coincident age-related macular degeneration (AMD) and in the fellow eye of 1 eye with unilateral type 3 neovascularization. On fundus photography, PEVAC presented as a perifoveal isolated aneurysm associated with retinal exudation and hemorrhage. Well-defined hyperfluorescent lesions with leakage were noted on fluorescein angiography, but no leakage was observed on ICGA. Anti-vascular endothelial growth factor (VEGF) therapy was performed for 2 patients. However, there was no notable improvement after treatment. Conclusions: The clinical characteristics of PEVAC in Korean patients were similar to those reported in patients of other ethnicities. Further studies are needed to better understand the nature of this newly described clinical entity.


Assuntos
Fóvea Central/patologia , Doenças Retinianas , Vasos Retinianos/anormalidades , Malformações Vasculares , Adulto , Idoso , Exsudatos e Transudatos , Feminino , Humanos , Coreia (Geográfico) , Masculino , Pessoa de Meia-Idade , Doenças Retinianas/patologia , Doenças Retinianas/fisiopatologia , Neovascularização Retiniana/patologia , Neovascularização Retiniana/fisiopatologia , Estudos Retrospectivos , Malformações Vasculares/patologia , Malformações Vasculares/fisiopatologia , Acuidade Visual
6.
Pathologica ; 111(1): 48-50, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31217623

RESUMO

In the last 30 years a revolution has occurred in the diagnosis and management of vascular anomalies. The great changes began with Mulliken and Glowacki separation of hemangiomas and vascular anomalies. Their work has now morphed into the ISSVA classification. Subsequently the discovery of the significance of the presence of GLUT-1 in the diagnosis of the hemangiomas of infancy gave us a new marker in our quest for accurate classification. Now the genetic breakthroughs have led us into a "Star Wars" like environment in the experimental laboratory. During all these events the critical role of the pathologist has become more evident. Understanding the histopathology of anomalies has greatly aided in our approach to therapies. Moreover, genetic findings do not have full significance without the morphologic framework.


Assuntos
Técnicas de Laboratório Clínico , Malformações Vasculares , Diagnóstico Diferencial , Hemangioma/patologia , Humanos , Malformações Vasculares/diagnóstico , Malformações Vasculares/patologia
8.
Diagn Interv Radiol ; 25(3): 225-230, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31063139

RESUMO

PURPOSE: We aimed to evaluate the safety and effectiveness of cryoablation in the treatment of low-flow malformations, specifically venous malformation (VM) and fibroadipose vascular anomaly (FAVA). METHODS: We conducted a retrospective review of 11 consecutive patients with low-flow malformations (14 lesions; 9 VM, 5 FAVA), median lesion volume 10.8 cm3, (range, 1.8-55.6 cm3) with a median age of 19 years (range, 10-50 years) who underwent cryoablation to achieve symptomatic control. Average follow-up was at a median of 207 days postprocedure (range, 120-886 days). Indications for treatment included focal pain and swelling. Technical success was achieved if the cryoablation ice ball covered the region of the malformation that corresponded to the patient's symptoms. Clinical success was considered complete if all symptoms resolved and partial if some symptoms persisted but did not necessitate further treatment. RESULTS: The technical success rate was 100%. At 1-month follow-up, 13 of 14 lesions (93%) had a complete response and one (7%) had a partial response. At 6-month follow-up 12 of 13 (92%) had a complete response and 1 (8%) had a partial response. A total of 6 patients underwent primary cryoablation. Out of 9 VM cases, 7 had prior sclerotherapy and 2 had primary cryoablation. Out of the 5 FAVA cases, 1 had prior sclerotherapy and the remaining 4 cases underwent primary cryoablation. There were 3 minor complications following cryoablation including 2 cases of skin blisters and 1 case of transient numbness. These complications resolved with conservative management. CONCLUSION: Cryoablation is safe and effective in the treatment of low-flow vascular malformations, either after sclerotherapy or as primary treatment.


Assuntos
Criocirurgia/métodos , Criocirurgia/estatística & dados numéricos , Malformações Vasculares/terapia , Adolescente , Adulto , Vesícula/etiologia , Criança , Criocirurgia/efeitos adversos , Feminino , Humanos , Hipestesia/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Escleroterapia/métodos , Escleroterapia/normas , Resultado do Tratamento , Malformações Vasculares/patologia , Adulto Jovem
9.
Virchows Arch ; 474(6): 755-761, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30810814

RESUMO

Vascular malformations (VMs) are rare congenital anomalies that develop during embryogenesis in different types of vessels. Several triggering factors of cutaneous VMs include trauma, infections, or hormonal changes. We investigated the expression of hormonal receptors (androgen, estrogen, progesterone) in tissue samples of well-characterized VMs. A secondary objective was to identify self-reported triggering factors for these VMs, including hormonal changes, in the cohort of patients. We included patients with VM samples obtained in the tertiary center for vascular anomalies of the University Hospital Center of Tours, France, from January 1, 2007, to August 1, 2018. Immunohistochemistry was used to detect the expression of hormonal receptors (estrogen, progesterone, androgens). We obtained 51 samples from 51 patients: 13 cystic lymphatic malformations (CLMs), 16 venous malformations (VeMs), 11 arteriovenous malformations (AVMs), 4 combined VMs, 4 PIK3CA-related overgrowth spectrum, 1 Parkes-Weber syndrome, 1 Gorham syndrome, and 1 multiple lymphangioendotheliomatosis with thrombopenia. In total, 38 (74.5%) samples were positive for androgen receptor: 11 (84.6%) CLMs, 12 (75.0%) VeMs, 8 (72.2%) AVMs, and 7/11 (63.5%) other samples. All samples were negative for estrogen and progesterone receptors. Triggering factors were self-reported in 7 cases and were most frequently hormonal changes (n = 6, 18.2%). Hormonal triggers were frequent in AVMs (n = 4). Among patients with identified hormonal triggers, VM samples were positive for androgen receptor in 3 and negative in 3. Three-quarters of our VM samples expressed androgen receptor, and most CLM, VeM, and AVM samples were positive. Hormonal triggers were identified in 6/33 patients, mostly with AVMs.


Assuntos
Malformações Arteriovenosas/patologia , Receptores Androgênicos/metabolismo , Receptores de Progesterona/metabolismo , Malformações Vasculares/patologia , Malformações Arteriovenosas/diagnóstico , Feminino , Humanos , Imuno-Histoquímica/métodos , Lactente , Masculino , Pessoa de Meia-Idade , Receptores Estrogênicos/metabolismo , Malformações Vasculares/diagnóstico , Malformações Vasculares/metabolismo
10.
Arterioscler Thromb Vasc Biol ; 39(3): 496-512, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30626204

RESUMO

Objective- Venous malformations (VMs) arise from developmental defects of the vasculature and are characterized by massively enlarged and tortuous venous channels. VMs grow commensurately leading to deformity, obstruction of vital structures, bleeding, and pain. Most VMs are associated with the activating mutation L914F in the endothelial cell (EC) tyrosine kinase receptor TIE2. Therapeutic options for VM are limited and ineffective while therapy with the mammalian target of rapamycin inhibitor rapamycin shows moderate efficacy. Here, we investigated novel therapeutic targets promoting VM regression. Approach and Results- We performed an unbiased screen of Food and Drug Administration-approved drugs in human umbilical vein ECs expressing the TIE2-L914F mutation (HUVEC-TIE2-L914F). Three ABL (Abelson) kinase inhibitors prevented cell proliferation of HUVEC-TIE2-L914F. Moreover, c-ABL, common target of these inhibitors, was highly phosphorylated in HUVEC-TIE2-L914F and VM patient-derived ECs with activating TIE2 mutations. Knockdown of c-ABL/ARG in HUVEC-TIE2-L914F reduced cell proliferation and vascularity of murine VM. Combination treatment with the ABL kinase inhibitor ponatinib and rapamycin caused VM regression in a xenograft model based on injection of HUVEC-TIE2-L914F. A reduced dose of this drug combination was effective in this VM murine model with minimal side effects. The drug combination was antiproliferative, enhanced cell apoptosis and vascular channel regression both in vivo and in a 3-dimensional fibrin gel assay. Conclusions- This is the first report of a combination therapy with ponatinib and rapamycin promoting regression of VM. Mechanistically, the drug combination enhanced AKT inhibition compared with single drug treatment and reduced PLCγ (phospholipase C) and ERK (extracellular signal-regulated kinase) activity.


Assuntos
Imidazóis/uso terapêutico , Inibidores de Proteínas Quinases/uso terapêutico , Piridazinas/uso terapêutico , Sirolimo/uso terapêutico , Malformações Vasculares/tratamento farmacológico , Animais , Apoptose/efeitos dos fármacos , Divisão Celular/efeitos dos fármacos , Células Cultivadas , Quimiotaxia , Avaliação Pré-Clínica de Medicamentos , Quimioterapia Combinada , Xenoenxertos , Células Endoteliais da Veia Umbilical Humana/transplante , Humanos , Imidazóis/administração & dosagem , Imidazóis/farmacologia , Sistema de Sinalização das MAP Quinases/efeitos dos fármacos , Masculino , Camundongos , Camundongos Nus , Mutação de Sentido Incorreto , Fosfolipase C gama/antagonistas & inibidores , Inibidores de Proteínas Quinases/administração & dosagem , Inibidores de Proteínas Quinases/farmacologia , Proteínas Proto-Oncogênicas c-abl/antagonistas & inibidores , Proteínas Proto-Oncogênicas c-akt/antagonistas & inibidores , Piridazinas/administração & dosagem , Piridazinas/farmacologia , Receptor TIE-2/genética , Transdução de Sinais/efeitos dos fármacos , Sirolimo/administração & dosagem , Sirolimo/farmacologia , Malformações Vasculares/patologia
11.
J Cutan Pathol ; 46(5): 368-371, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30666667

RESUMO

A 52-year-old man presented with a rapidly growing red tumor on the central neckline. It had appeared over a congenital flat and pinkish vascular lesion that involved the shoulder and the upper anterior area of his chest. Intermingled with the pinkish stain, there were also some blue nodules several millimeters in diameter. Histopathologic examination revealed that the full lesion was a mixed venous-capillary malformation. The red tumor was excised and diagnosed as a pyogenic granuloma developing over the venous component of the vascular malformation. To our knowledge, a pyogenic granuloma growing over a venous malformation has not been previously described.


Assuntos
Granuloma Piogênico/patologia , Neoplasias Cutâneas/patologia , Malformações Vasculares/patologia , Granuloma Piogênico/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/metabolismo , Malformações Vasculares/metabolismo
12.
Biochem Biophys Res Commun ; 509(4): 898-902, 2019 02 19.
Artigo em Inglês | MEDLINE | ID: mdl-30638931

RESUMO

The endothelial receptor tyrosine kinase Tie2 plays an important role in vascular formation and maintenance. Mutations in Tie2 lead to vascular malformations, which are painful vascular lesions that cause disfigurement, bleeding, and thrombosis. R849W Tie2 is the most common mutation implicated in an inherited form of vascular malformations and has been shown to be activating, though little is known about the kinetic features of catalysis. Here we undertake a steady-state kinetic analysis of heterologously expressed and purified wild type (WT) and R849W Tie2. While the catalytic efficiencies of the two forms are not significantly different, the observed maximal rate of phosphorylation, kcat,obs, is > 3-fold higher for R849W Tie2 compared to WT. Notably, steady-state catalysis by R849W Tie2 has more striking sigmoidal features compared to WT, suggesting enhanced positive cooperativity. We propose that activating catalytic features are one important consequence of the R849W mutation, though likely other factors such as increased protein binding affinity also contribute to the phenotypes observed in patients.


Assuntos
Endotélio Vascular/patologia , Mutação , Receptor TIE-2/genética , Malformações Vasculares/patologia , Biocatálise , Endotélio Vascular/anormalidades , Humanos , Cinética , Fosforilação , Ligação Proteica , Malformações Vasculares/genética
13.
Cardiovasc Pathol ; 38: 39-41, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30447516

RESUMO

We report here a case of capillary vascular malformation developed in a bicuspid aortic valve incidentally detected during valve replacement in a 67-year-old male patient. The International Society for the Study of Vascular Anomalies (ISSVA) classification was used to classify this vascular lesion instead of using the term hemangioma. The differential diagnosis and the literature are reviewed.


Assuntos
Valva Aórtica/anormalidades , Capilares/anormalidades , Anomalias dos Vasos Coronários/diagnóstico , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Achados Incidentais , Malformações Vasculares/diagnóstico , Idoso , Valva Aórtica/cirurgia , Biópsia , Capilares/patologia , Anomalias dos Vasos Coronários/patologia , Diagnóstico Diferencial , Doenças das Valvas Cardíacas/diagnóstico , Humanos , Masculino , Valor Preditivo dos Testes , Malformações Vasculares/patologia
14.
Am J Forensic Med Pathol ; 40(1): 68-71, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30359337

RESUMO

Venous malformations (VMs) are the most common vascular malformations, and their diagnosis can be challenging. They may develop in any region of the body, with highly variable clinical presentations. Although they typically present early in life, many case reports have documented the sudden appearance of a previously unrecognized venous malformation in adulthood. Pain is the major complaint in most of the cases, and other complications include phlebolith formation and bleeding. To our knowledge, fatal hemorrhage from a VM has not previously been reported in the medical literature. We present a case of exsanguination from a previously undiagnosed lower limb superficial VM. This case stresses the importance of proper diagnosis and management of VM.


Assuntos
Exsanguinação/etiologia , Veia Safena/anormalidades , Malformações Vasculares/patologia , Idoso de 80 Anos ou mais , Feminino , Humanos , Úlcera da Perna/patologia
15.
Virchows Arch ; 474(1): 47-57, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30357455

RESUMO

Congenital portosystemic shunt (CPSS) is a congenital anomaly resulting in partial or complete diversion of the portal blood into the systemic circulation. The literature on the histological changes in livers of patients with CPSS is limited. Liver histology of 22 consecutive patients managed in our institution between 2001 and 2016 was reviewed. Twenty-one patients were children at the time of diagnosis. Thirty-two specimens were available and consisted of three explant livers and 29 biopsy samples from 19 patients. Sixteen samples were from wedge biopsies taken at the time of shunt closure. Thirteen were from core needle biopsies taken during clinical work-up. A variable proportion of portal tracts contained prominent thin-walled channels (PTWCs) and arterio-biliary dyads. The proportion of portal tracts containing triads, arterio-biliary dyads and biliary monads varied considerably in the different samples. Dilated inlet venules, increase in the number of portal arteries or the presence of portal arteries of increased size, deposition of copper-associated protein, sinusoidal dilatation, capillarization and intralobular individual arteries were present. Physiological nuclear vacuolation of periportal hepatocytes was absent in most samples from our paediatric patients. Presence of PTWCs, arterial-biliary dyads, increased arterial profiles in portal tracts and lobule and lack of the physiological periportal vacuolated hepatocytes in children are the most characteristic histological changes of CPSS in the liver periphery.


Assuntos
Ductos Biliares Intra-Hepáticos/patologia , Capilares/patologia , Artéria Hepática/patologia , Fígado/irrigação sanguínea , Fígado/patologia , Veia Porta/anormalidades , Veia Porta/patologia , Malformações Vasculares/patologia , Adolescente , Biópsia com Agulha de Grande Calibre , Criança , Pré-Escolar , Feminino , Hepatectomia , Humanos , Imuno-Histoquímica , Lactente , Fígado/cirurgia , Masculino , Veia Porta/cirurgia , Malformações Vasculares/genética , Malformações Vasculares/cirurgia
16.
Sci Rep ; 8(1): 17987, 2018 12 20.
Artigo em Inglês | MEDLINE | ID: mdl-30573741

RESUMO

Notch expression has been shown to be aberrant in brain arteriovenous malformations (AVM), and targeting Notch has been suggested as an approach to their treatment. It is unclear whether extracranial vascular malformations follow the same patterning and Notch pathway defects. In this study, we examined human extracranial venous (VM) (n = 3), lymphatic (LM) (n = 10), and AV (n = 6) malformations, as well as sporadic brain AVMs (n = 3). In addition to showing that extracranial AVMs demonstrate interrupted elastin and that AVMs and LMs demonstrate abnormal α-smooth muscle actin just as brain AVMS do, our results demonstrate that NOTCH1, 2, 3 and 4 proteins are overexpressed to varying degrees in both the endothelial and mural lining of the malformed vessels in all types of malformations. We further show that two gamma secretase inhibitors (GSIs), DAPT (GSI-IX) and RO4929097, cause dose-dependent inhibition of Notch target gene expression (Hey1) and rate of migration of monolayer cultures of lymphatic endothelial cells (hLECs) and blood endothelial cells (HUVEC). GSIs also inhibit HUVEC network formation. hLECs are more sensitive to GSIs compared to HUVEC. GSIs have been found to be safe in clinical trials in patients with Alzheimer's disease or cancer. Our results provide further rationale to support testing of Notch inhibitors in patients with extracranial vascular malformations.


Assuntos
Terapia de Alvo Molecular , Receptores Notch/fisiologia , Malformações Vasculares/genética , Malformações Vasculares/terapia , Inibidores da Angiogênese/farmacologia , Inibidores da Angiogênese/uso terapêutico , Sobrevivência Celular/efeitos dos fármacos , Sobrevivência Celular/genética , Células Cultivadas , Regulação da Expressão Gênica/efeitos dos fármacos , Células Endoteliais da Veia Umbilical Humana/efeitos dos fármacos , Células Endoteliais da Veia Umbilical Humana/fisiologia , Humanos , Recém-Nascido , Vasos Linfáticos/efeitos dos fármacos , Vasos Linfáticos/metabolismo , Neovascularização Fisiológica/efeitos dos fármacos , Neovascularização Fisiológica/genética , Receptores Notch/antagonistas & inibidores , Transdução de Sinais/efeitos dos fármacos , Transdução de Sinais/genética , Malformações Vasculares/patologia
19.
BMC Pediatr ; 18(1): 371, 2018 11 27.
Artigo em Inglês | MEDLINE | ID: mdl-30482204

RESUMO

BACKGROUND: The diagnosis of obscure gastrointestinal bleeding (OGIB) which is defined as bleeding of unknown origin of the small bowel by routine evaluation in childhood is a challenge. CASE PRESENTATION: Here we report a one-year-old Chinese girl who was suspected with idiopathic pulmonary haemosiderosis (IPH) and referred to our department for further diagnosis. Finally she was diagnosed with vascular malformations (VM) by exploratory laparoscopy combined with pathological examination. CONCLUSIONS: Children OGIB could be easily misdiagnosed in the beginning, and OGIB children with active ongoing bleeding may benefit from proceeding directly to exploratory laparoscopy, followed by pathological confirmation of the diagnosis.


Assuntos
Hemorragia Gastrointestinal/etiologia , Jejuno/irrigação sanguínea , Jejuno/patologia , Laparoscopia , Malformações Vasculares/diagnóstico , Anemia Ferropriva/diagnóstico , Diagnóstico Diferencial , Feminino , Hemorragia Gastrointestinal/patologia , Hemossiderose/diagnóstico , Humanos , Lactente , Jejuno/cirurgia , Pneumopatias/diagnóstico , Malformações Vasculares/patologia , Malformações Vasculares/cirurgia
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