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[PMID]:29237532
[Au] Autor:Peng SH; Yang KY; Chen SY; Ji Y
[Ad] Endereço:Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China. jijiyuanyuan@163.com.
[Ti] Título:[Research progresses in the pathogenesis, diagnosis and treatment of infantile hemangioma with PHACE syndrome].
[So] Source:Zhongguo Dang Dai Er Ke Za Zhi;19(12):1291-1296, 2017 Dec.
[Is] ISSN:1008-8830
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:Infant hemangioma, the most common benign tumor in children, is characterized by rapid proliferation, followed by slower spontaneous involution. However, some patients with facial segmental hemangioma are associated with PHACE syndrome. PHACE syndrome is characterized by vascular nerve and vascular cutaneous lesions of multiple systemic systems, often resulting in structural and functional impairments. Recent studies have demonstrated that the possible pathogeneses of PHACE syndrome mainly include hypoxia, abnormality of mesodermal vascular endothelial cells, genetic abnormality, and abnormality of interstitial mesenchymal stem cells. The current medications for hemangioma with PHACE syndrome include beta blockers, glucocorticoids, and mTOR inhibitors. This review article mainly describes the pathogenesis, diagnoses and treatments of PHACE syndrome, in order to provide directions for diagnosis and treatment of this disorder.
[Mh] Termos MeSH primário: Anormalidades Múltiplas/terapia
Anormalidades do Olho/terapia
Cardiopatias Congênitas/terapia
Hemangioma/terapia
[Mh] Termos MeSH secundário: Anormalidades Múltiplas/diagnóstico
Anormalidades Múltiplas/etiologia
Anormalidades do Olho/diagnóstico
Anormalidades do Olho/etiologia
Cardiopatias Congênitas/diagnóstico
Cardiopatias Congênitas/etiologia
Hemangioma/diagnóstico
Hemangioma/etiologia
Seres Humanos
Lactente
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180306
[Lr] Data última revisão:
180306
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171215
[St] Status:MEDLINE


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[PMID]:29429507
[Au] Autor:Léauté-Labrèze C
[Ad] Endereço:Unité de dermatologie pédiatrique et centre de référence des maladies rares de la peau, hôpital Pellegrin-Enfants, CHU de Bordeaux, 33076 Bordeaux Cedex, France. Electronic address: christine.labreze@chu-bordeaux.fr.
[Ti] Título:[What's new in pediatric dermatology?]
[Ti] Título:Quoi de neuf en dermatologie pédiatrique ?.
[So] Source:Ann Dermatol Venereol;143 Suppl 3:S29-S36, 2016 Dec.
[Is] ISSN:0151-9638
[Cp] País de publicação:France
[La] Idioma:fre
[Ab] Resumo:The association of a birth defect and a segmental hemangioma is well established, a consensus concerning evaluation and monitoring of infants with PHACE or LUMBAR syndromes has been published. The efficacy of propranolol in infantile hemangioma is proven; however there were still unresolved issues concerning the safety in children; after 8 years of use on thousands of children safety data collection did not show any unexpected side effects. Topical treatment of infantile hemangiomas with beta-blockers, such as timolol, is very popular, but recent publications revealed a significant systemic absorption that could be responsible for severe side effects, such as bradycardia, in low birthweight infants. As a consequence, this therapeutic option should be considered with caution. In the last 2 years mTOR inhibitors have been tested in low-flow vascular malformations with varying success, but progress remains to be done in the treatment of vascular abnormalities. Today, genetics has led to advances in the understanding of the pathophysiology and in the future targeted therapies could probably be feasible. Skin barrier deficiency is responsible for the development of allergic phenomena in atopic patients, since it has been shown that sensibilisation, even to food, could probably be induced by skin contact. Unfortunately, the topical treatment with crisaborole, a phosphodiesterase 4 inhibitor, does not look like a revolution in children atopic dermatitis, its efficacy seems equivalent to emollient application. In the field of infectious diseases, changes in viral outbreaks are the most reported. Furthermore epidemic Zika virus, enteroviruses are responsible for expanded dermatological manifestations and also severe meningoencephalitis. Paraviral character of various eruptions, such as gloves and socks syndrome or eruptive pseudoangiomatosis is challenged.
[Mh] Termos MeSH primário: Dermatopatias
[Mh] Termos MeSH secundário: Coartação Aórtica/terapia
Doenças Autoimunes/genética
Criança
Dermatologia
Anormalidades do Olho/terapia
Hipersensibilidade Alimentar/imunologia
Hemangioma/terapia
Seres Humanos
Síndromes Neurocutâneas/terapia
Pediatria
Fator de Transcrição STAT3/genética
Dermatopatias/diagnóstico
Dermatopatias/etiologia
Dermatopatias/terapia
Fenômenos Fisiológicos da Pele
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (STAT3 Transcription Factor); 0 (STAT3 protein, human)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180221
[Lr] Data última revisão:
180221
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180213
[St] Status:MEDLINE


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[PMID]:29384951
[Au] Autor:Ouyang L; Yang F
[Ad] Endereço:Department of Child Healthcare, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China.
[Ti] Título:Cole-Carpenter syndrome-1 with a de novo heterozygous deletion in the P4HB gene in a Chinese girl: A case report.
[So] Source:Medicine (Baltimore);96(52):e9504, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Cole-Carpenter syndrome-1 (CLCRP1) is an independent osteogenesis imperfect (OI)-like disorder that manifests as bone fragility, craniosynostosis, ocular proptosis, hydrocephalus, and distinctive facial features. Only 2 types of mutation sites in the P4HB and CRTAP genes have been reported. PATIENT CONCERNS: A 14-month-old Chinese girl presented with prominent ocular proptosis, frontal bossing, craniosynostosis, plump anterior fontanel, growth retardation, osteopenia, and distinctive facial features that were strikingly similar to those in the original 2 cases. DIAGNOSES: Whole-exome sequencing revealed a novel deletion variation in exons 5 to 8 of the P4HB gene, which was found to be heterozygous using fluorogenic quantitative-polymerase chain reaction. LESSONS: This de novo deletion mutation in exons 5 to 8 of the P4HB gene advances our understanding of CLCRP1, expands the mutation spectrum of P4HB, and diversifies the cases reported for this condition.
[Mh] Termos MeSH primário: Craniossinostoses/genética
Anormalidades do Olho/genética
Hidrocefalia/genética
Osteogênese Imperfeita/genética
Pró-Colágeno-Prolina Dioxigenase/genética
Isomerases de Dissulfetos de Proteínas/genética
[Mh] Termos MeSH secundário: Grupo com Ancestrais do Continente Asiático
China
Craniossinostoses/patologia
Anormalidades do Olho/patologia
Feminino
Seres Humanos
Hidrocefalia/patologia
Lactente
Osteogênese Imperfeita/patologia
Deleção de Sequência
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
EC 1.14.11.2 (Procollagen-Proline Dioxygenase); EC 5.3.4.1 (P4HB protein, human); EC 5.3.4.1 (Protein Disulfide-Isomerases)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180220
[Lr] Data última revisão:
180220
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180201
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009504


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[PMID]:29394916
[Au] Autor:Chen Y; Ma X; Hua R
[Ad] Endereço:Department of Ophthalmology, First Hospital of China Medical University, No. 155 Nanjingbei Street, Heping District, Shenyang, Liaoning Province, People's Republic of China.
[Ti] Título:Multi-modality imaging findings of huge intrachoroidal cavitation and myopic peripapillary sinkhole.
[So] Source:BMC Ophthalmol;18(1):24, 2018 Feb 02.
[Is] ISSN:1471-2415
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Peripapillary intrachoroidal cavitation was described as the presence of an asymptomatic, well-circumscribed, yellow-orange, peripapillary lesion at the inferior border of the myopic conus in eyes with high myopia. CASE PRESENTATION: A 66-year-old myopic Chinese man was enrolled and his multi-color imaging examination showed a well-circumscribed, caesious, peripapillary lesion coalesced with the optic nerve head vertically rotated and obliquely tilted, together with an inferotemporal sinkhole in the myopic conus. The optical coherence tomography images showed an intrachoroidal hyporeflective space, schisis, an intracavitary septum located below the retinal pigment epithelium and inserted beneath the optic nerve head, as well as a sinkhole between the peripapillary intrachoroidal cavitation and the vitreous space. CONCLUSIONS: Both myopic colobomas and sinkhole in myopic conus may contribute the coalescence of intrachoroidal cavitation with optic nerve head. These qualitative and quantitative new findings will be beneficial for understanding its pathomorphological mechanism, and the impact on optic nerve tissue of myopic patients.
[Mh] Termos MeSH primário: Doenças da Coroide/diagnóstico por imagem
Anormalidades do Olho/diagnóstico por imagem
Miopia/diagnóstico por imagem
Disco Óptico/anormalidades
Tomografia de Coerência Óptica/métodos
[Mh] Termos MeSH secundário: Idoso
Seres Humanos
Masculino
Imagem Multimodal
Campos Visuais
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180216
[Lr] Data última revisão:
180216
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180204
[St] Status:MEDLINE
[do] DOI:10.1186/s12886-018-0681-x


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[PMID]:29238852
[Au] Autor:Maertz J; Kolb JP; Klein T; Mohler KJ; Eibl M; Wieser W; Huber R; Priglinger S; Wolf A
[Ad] Endereço:Augenklinik der Ludwig-Maximilians-Universität München, Campus Innenstadt, Mathildenstraße 8, D-80336, Munich, Germany.
[Ti] Título:Combined in-depth, 3D, en face imaging of the optic disc, optic disc pits and optic disc pit maculopathy using swept-source megahertz OCT at 1050 nm.
[So] Source:Graefes Arch Clin Exp Ophthalmol;256(2):289-298, 2018 Feb.
[Is] ISSN:1435-702X
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To demonstrate papillary imaging of eyes with optic disc pits (ODP) or optic disc pit associated maculopathy (ODP-M) with ultrahigh-speed swept-source optical coherence tomography (SS-OCT) at 1.68 million A-scans/s. To generate 3D-renderings of the papillary area with 3D volume-reconstructions of the ODP and highly resolved en face images from a single densely-sampled megahertz-OCT (MHz-OCT) dataset for investigation of ODP-characteristics. METHODS: A 1.68 MHz-prototype SS-MHz-OCT system at 1050 nm based on a Fourier-domain mode-locked laser was employed to acquire high-definition, 3D datasets with a dense sampling of 1600 × 1600 A-scans over a 45° field of view. Six eyes with ODPs, and two further eyes with glaucomatous alteration or without ocular pathology are presented. 3D-rendering of the deep papillary structures, virtual 3D-reconstructions of the ODPs and depth resolved isotropic en face images were generated using semiautomatic segmentation. RESULTS: 3D-rendering and en face imaging of the optic disc, ODPs and ODP associated pathologies showed a broad spectrum regarding ODP characteristics. Between individuals the shape of the ODP and the appending pathologies varied considerably. MHz-OCT en face imaging generates distinct top-view images of ODPs and ODP-M. MHz-OCT generates high resolution images of retinal pathologies associated with ODP-M and allows visualizing ODPs with depths of up to 2.7 mm. CONCLUSIONS: Different patterns of ODPs can be visualized in patients for the first time using 3D-reconstructions and co-registered high-definition en face images extracted from a single densely sampled 1050 nm megahertz-OCT (MHz-OCT) dataset. As the immediate vicinity to the SAS and the site of intrapapillary proliferation is located at the bottom of the ODP it is crucial to image the complete structure and the whole depth of ODPs. Especially in very deep pits, where non-swept-source OCT fails to reach the bottom, conventional swept-source devices and the MHz-OCT alike are feasible and beneficial methods to examine deep details of optic disc pathologies, while the MHz-OCT bears the advantage of an essentially swifter imaging process.
[Mh] Termos MeSH primário: Anormalidades do Olho/diagnóstico
Imagem Tridimensional
Macula Lutea/patologia
Disco Óptico/patologia
Doenças Retinianas/diagnóstico
Tomografia de Coerência Óptica/instrumentação
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Desenho de Equipamento
Feminino
Seres Humanos
Masculino
Meia-Idade
Disco Óptico/anormalidades
Projetos Piloto
Reprodutibilidade dos Testes
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180214
[Lr] Data última revisão:
180214
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171215
[St] Status:MEDLINE
[do] DOI:10.1007/s00417-017-3857-9


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[PMID]:29390414
[Au] Autor:Zhu L; Xie L
[Ad] Endereço:Ultrasound Department, Sheng Jing Hospital of China Medical University, Shenyang, Liaoning Province, People's Republic of China.
[Ti] Título:Prenatal diagnosis of Joubert syndrome: A case report and literature review.
[So] Source:Medicine (Baltimore);96(51):e8626, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Joubert syndrome (JS) is a rare autosomal recessive inherited disease belonging to ciliopathy with the causative mutation of genes. Except for X-linked inheritance, the high recurrence rate of a family is about 25%. After birth, it may cause a series of neurological symptoms, even with retina, kidney, liver, and other organ abnormalities, which is defined as Joubert syndrome and related disorders (JSRD). Molecular genetics research contributes to disease prediction and genetic counseling. Prenatal diagnosis is rare. Magnetic resonance imaging (MRI) is usually the first-choice diagnostic modality with typical brain images characterized by the molar tooth sign. We describe a case of JS prenatally and Dandy-Walker malformation for the differential diagnosis based on ultrasonograms. We also review the etiology, imaging features, clinical symptoms, and diagnosis of JSRD. CASE PRESENTATION: A 22-year-old woman was pregnant at 27 1/7 weeks' gestation with fetal cerebellar vermis hypoplasia. Fetal ultrasonography and MRI confirmed a diagnosis of JS at our center. The couple finally opted to terminate the fetus, which had a normal appearance and growth parameters. The couple also had an AHI1 gene mutation on chromosome 6. CONCLUSIONS: Currently, a diagnosis of JS is commonly made after birth. Fewer cases of prenatal diagnosis by ultrasonography have been made, and they are more liable to be misdirected because of some nonspecial features that also manifest in Dandy-Walker malformation, cranio-cerebello-cardiac syndrome, and so on.
[Mh] Termos MeSH primário: Anormalidades Múltiplas/diagnóstico
Proteínas Adaptadoras de Transdução de Sinal/genética
Cerebelo/anormalidades
Síndrome de Dandy-Walker/diagnóstico
Anormalidades do Olho/diagnóstico
Doenças Renais Císticas/diagnóstico
Retina/anormalidades
[Mh] Termos MeSH secundário: Anormalidades Múltiplas/diagnóstico por imagem
Cerebelo/diagnóstico por imagem
Síndrome de Dandy-Walker/complicações
Síndrome de Dandy-Walker/diagnóstico por imagem
Diagnóstico Diferencial
Anormalidades do Olho/complicações
Anormalidades do Olho/diagnóstico por imagem
Feminino
Aconselhamento Genético
Idade Gestacional
Seres Humanos
Doenças Renais Císticas/complicações
Doenças Renais Císticas/diagnóstico por imagem
Mutação
Gravidez
Retina/diagnóstico por imagem
Ultrassonografia Pré-Natal
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (AHI1 protein, human); 0 (Adaptor Proteins, Signal Transducing)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180213
[Lr] Data última revisão:
180213
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180203
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008626


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[PMID]:28468208
[Au] Autor:Sisti A; Freda N; Giacomina A; Gatti GL
[Ad] Endereço:Plastic and Reconstructive Surgery, Santa Chiara Hospital, Pisa, Italy.
[Ti] Título:Popliteal Pterygium Syndrome With Syngnathia.
[So] Source:J Craniofac Surg;28(3):e250-e251, 2017 May.
[Is] ISSN:1536-3732
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Popliteal pterygium syndrome is a condition characterized by skin webs on the popliteal fossa, which may impair mobility unless surgically repaired. Affected individuals may also have syndactyly on the fingers and/or toes. Most people with this disorder present cleft lip and cleft palate and they can have syngnathia, that is a congenital adhesion between maxilla and mandible by fibrous bands, which affects the opening of the mouth. The case that we report is about a 2-month-old male, who presented skin webs bilaterally on the popliteal fossa, syndactyly between the IV and the V toe of the right foot and between the III and the IV toe of the left foot, and genital malformations. He was born with complete bilateral cleft lip and complete cleft palate on the left side and incomplete cleft palate on the right side and syngnathia with 4 fibrous bands between the mandibular arch and the maxilla arch on the right side, which affected the opening of the mouth. The case of our patient is very interesting because there have been few reported patients affected by popliteal pterygium syndrome with syngnathia.
[Mh] Termos MeSH primário: Anormalidades Múltiplas/diagnóstico
Fenda Labial/diagnóstico
Fissura Palatina/diagnóstico
Anormalidades do Olho/diagnóstico
Dedos/anormalidades
Articulação do Joelho/anormalidades
Deformidades Congênitas das Extremidades Inferiores/diagnóstico
Mandíbula/anormalidades
Maxila/anormalidades
Sindactilia/diagnóstico
Anormalidades Urogenitais/diagnóstico
[Mh] Termos MeSH secundário: Seres Humanos
Lactente
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180202
[Lr] Data última revisão:
180202
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000003473


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[PMID]:29224766
[Au] Autor:Bangiyev JN; Gurgel R; Vanderhooft SL; Grimmer JF
[Ad] Endereço:University of Utah, Division of Otolaryngology, United States. Electronic address: jbangiyev@gmail.com.
[Ti] Título:Reversible profound sensorineural hearing loss due to propranolol sensitive hemangioma in an infant with PHACE syndrome.
[So] Source:Int J Pediatr Otorhinolaryngol;103:55-57, 2017 Dec.
[Is] ISSN:1872-8464
[Cp] País de publicação:Ireland
[La] Idioma:eng
[Ab] Resumo:PHACE syndrome is the association of large or segmental infantile hemangiomas of the face or scalp with abnormalities within the posterior fossa, arteries, cardiovascular system, and eyes. We present a case of reversible profound sensorineural hearing loss due to a cerebellopontine angle infantile hemangioma that was successfully treated with propranolol.
[Mh] Termos MeSH primário: Antagonistas Adrenérgicos beta/uso terapêutico
Coartação Aórtica/complicações
Anormalidades do Olho/complicações
Perda Auditiva Neurossensorial/etiologia
Hemangioma/complicações
Síndromes Neurocutâneas/complicações
Propranolol/uso terapêutico
[Mh] Termos MeSH secundário: Hemangioma/tratamento farmacológico
Seres Humanos
Lactente
Imagem por Ressonância Magnética
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Adrenergic beta-Antagonists); 9Y8NXQ24VQ (Propranolol)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171226
[Lr] Data última revisão:
171226
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171212
[St] Status:MEDLINE


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[PMID]:29202717
[Au] Autor:Giocanti-Aurégan A; Chbat EA; Morel CGH; Morin BR; Conrath JG; Devin FC
[Ad] Endereço:Ophthalmology department, Avicenne hospital, 125 rue de Stalingrad, 93000, Bobigny, France. audreygiocanti@yahoo.fr.
[Ti] Título:Ten years follow-up after surgery for a foveal detachment due to optic disc pit: a case report of outer retinal layer healing.
[So] Source:BMC Ophthalmol;17(1):231, 2017 Dec 04.
[Is] ISSN:1471-2415
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: To report a case of complete progressive visual recovery and healing of outer retinal layers after vitrectomy for foveal detachment associated with optic disc pit. CASE PRESENTATION: Optical coherence tomography (OCT) follow-up was performed on a 15-year-old boy with deep optic disc pit and foveal detachment, before and for 10 years after vitrectomy with gas. The foveal detachment was successfully reattached with complete reapplication of the retina. OCT scans showed a progressive long-term retinal healing with reappearance of the ellipsoid line and visual acuity improved from 20/100 before surgery to 20/25, 10 years after surgery. CONCLUSIONS: Photoreceptor regeneration after foveal detachment surgery has been already described only in zebrafish but never humans. However, we highlight with this case that in humans, a healing process of the outer retinal layers can occur with reappearance of the ellipsoid zone on OCT. This healing process may take several years and allow a complete functional restoration.
[Mh] Termos MeSH primário: Anormalidades do Olho/complicações
Fóvea Central
Disco Óptico/anormalidades
Retina/fisiologia
Descolamento Retiniano/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Fóvea Central/cirurgia
Seres Humanos
Masculino
Regeneração
Perfurações Retinianas/cirurgia
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171226
[Lr] Data última revisão:
171226
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171206
[St] Status:MEDLINE
[do] DOI:10.1186/s12886-017-0626-9


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[PMID]:26782916
[Au] Autor:Krásný J; Cakrtová M; Kletenský J; Novák V; Sach J
[Ti] Título:[About Cryptophthalmos (2nd Czech Study)].
[Ti] Título:O KRYPTOFTALMU (2. ceská studie)..
[So] Source:Cesk Slov Oftalmol;71(6):278-86, 2015.
[Is] ISSN:1211-9059
[Cp] País de publicação:Czech Republic
[La] Idioma:cze
[Ab] Resumo:AIM: To get acquainted with the 2nd Czech study about cryptophthamos and with self-surgical methods. MATERIAL: The boy with unilateral complete cryptophthalmos of left eye was treated from 2 to 20 years. The girls was treated from 4 month to 5 year yet for right abortive cryptophthalmos with microblepharon and left complete type still waiting for solutions.Surgical methods and results: Authors present a surgical procedures for correction of the upper and lower eyelids and ocular anomalies both patients studied. Successful reconstruction of palpebral fissure took place in several stages at the boy. The surgical procedure gradually contained: the insertion of gradually increased convex concave circular-shaped implant (silicone ruber) due a modeling of palpebral fissure, an enucleation of rudimentary eye, a reconstruction of bottom palpebral fissure by retro-auricular skin graf and a releasing of the lower transitory fold by the cul-de-sac method. An adequate depth of palpebral fissure to allow perfect position of an aesthetic protesis. Enucleated eye was atypically shaped, remiding partly sand-glass clock. The cornea was replaced by thick fibrous membrane, the iris and the lens were not revealed. Gliomatic retina was detached nearly totaly and the optic nerv was rudimental. The repairing the upper lid coloboma of girl by a lid rotation flap reconstruction using the inferior eyelid was performed at the age 17 month. Corneal dermoid simultaneously removed (histologically verified). Upper conjunctival fornix was formed using the spherical covering foil (silicone rubber) before and after the reconstruction of the lid. CONCLUSIONS: Plastic reconstructions required the need for patient access without trying immediate effect. An important role played silicone rubber implants (elastomer medical grade) which used temporarily. KEY WORDS: cryptophthalmos, microblepharon, relief surgery, silicon ruber implants.
[Mh] Termos MeSH primário: Anormalidades do Olho/cirurgia
Pálpebras/anormalidades
Procedimentos Cirúrgicos Oftalmológicos
Procedimentos Cirúrgicos Reconstrutivos
[Mh] Termos MeSH secundário: Túnica Conjuntiva/cirurgia
República Tcheca
Pálpebras/cirurgia
Feminino
Seguimentos
Seres Humanos
Masculino
Próteses e Implantes
Retalhos Cirúrgicos
Acuidade Visual/fisiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171205
[Lr] Data última revisão:
171205
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160120
[St] Status:MEDLINE



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