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1.
J. coloproctol. (Rio J., Impr.) ; 43(2): 139-151, Apr.-June 2023. ilus
文章 在 英语 | LILACS | ID: biblio-1514434

摘要

Pseudomyxoma peritonei (PMP) refers to a growth disorder characterized by glycoprotein neoplasm in the peritoneum, where mucin oversecretion occurs. The tumors of the appendix region are well associated with PMP; however, ovarian, colon, stomach, pancreas, and urachus tumors have also been linked to PMP. Other mucinous tumors in the pelvis, paracolic gutters, greater omentum, retrohepatic space, and Treitz ligament can be the reason for PMP. Despite being rare and having a slow growth rate, PMP can be lethal without treatment. It is treated with neoadjuvant chemotherapy with the option of cytoreductive surgery and intraperitoneal chemotherapy. In the current study, we hypothesize that there may be novel gentle ways to inhibit or eliminate the mucin. Dr. David Morris has used mucolytics - such as bromelain and N-acetyl cysteine to solubilize mucin. In the present review, we aimed to study the regulation of mucin expression by promoter methylation, and drugs that can inhibit mucin, such as boldine, amiloride, naltrexone, dexamethasone, and retinoid acid receptors antagonist. This review also explored some possible pathways, such as inhibition of Na + , Ca2+ channels and induction of DNA methyltransferase along with inhibition of ten-eleven translocation enzymes, which can be good targets to control mucin. Mucins are strong adhesive molecules that play great roles in clinging to cells or cell to cell. Besides, they have been greatly involved in metastasis and also act as disease markers for cancers. Diagnostic markers may have exclusive roles in disease initiation and progression. Therefore, the present review explores various drugs to control and target mucin in various diseases, specifically cancers. (AU)


Subject(s)
Pseudomyxoma Peritonei/drug therapy , Aporphines/therapeutic use , Retinoids/therapeutic use , Dexamethasone/therapeutic use , Calcium , Amiloride/therapeutic use , Methylation/drug effects , Mucins/drug effects , Naltrexone/therapeutic use
2.
Prensa méd. argent ; Prensa méd. argent;105(9 especial): 576-581, oct 2019.
文章 在 英语 | LILACS, BINACIS | ID: biblio-1046621

摘要

This work is aimed at studying the problems of timely diagnostics and therapy of various forms of rosacea, identifying the factors that influence the compliance, prognosis, and quality of life of the patients, as well as the stages of combination therapy. The efficiency of rosacea therapy is determined by the timely identification of patients, as well as the clinical variety of the disease. Complex therapy of rosacea includes identification of the precipitating factors, basic skincare, and the use of systemic and local pathogenetic preparations. The "Gold Standard" of topical rosacea therapy is the antimicrobial and antiprotozoal drug called metronidazole. An important role in disease therapy is played by active cooperation between the doctor and the patient. Comprehensiveness, timeliness, and rationality of rosacea therapy are defined not only by the mechanisms of the disease development but also by aggravating factors, the need for basic care and photosensitivity of the patients


Subject(s)
Photosensitivity Disorders , Retinoids/therapeutic use , Isotretinoin/therapeutic use , Patient Compliance , Tacrolimus/therapeutic use , Rosacea/diagnosis , Combined Modality Therapy , Metronidazole/therapeutic use
3.
Arch. pediatr. Urug ; 88(5): 279-283, oct. 2017. tab, ilus
文章 在 西班牙语 | LILACS | ID: biblio-887794

摘要

Resumen Pitiriasis rubra pilaris es una dermatosis eritematoescamosa infrecuente, de etiología desconocida producida por una alteración en la queratinización de la epidermis. Presenta una distribución bimodal con mayor incidencia en la primera y sexta década de vida. Posee una clínica heterogénea clasificada en 6 subtipos según Griffiths, de acuerdo a su presentación clínica y pronóstico. Sus principales hallazgos son pápulas hiperqueratósicas foliculares, queratodermia palmoplantar y placas eritematoesmamosas rojo-anaranjadas que pueden progresar a eritrodermia, con islas de piel sana. En niños las manifestaciones clínicas más frecuentes son la III y IV de la clasificación de Griffiths, según distintos estudios. La histología no es específica pero apoya el diagnóstico. Existen múltiples opciones terapéuticas según la extensión y severidad del cuadro. Presentamos el caso de un preescolar de 5 años de edad con diagnóstico de PRP atípica asociado a eritema extenso, con buena respuesta a corticoides sistémicos y posteriormente a retinoides tópicos.


Summary Pityriasis rubra pilaris (PRP) is an unusual erythematous squamous dermatosis of unknown etiology, caused by an alteration of keratinization in the epidermis. This disease presents a bimodal distribution, being its incidence greater in the first and sixth decade of life. It has a heterogeneous clinical manifestation, and, according to Griffiths, has been classified into 6 subtypes, based on clinical features and prognosis. The typical manifestations of this disease are follicular hyperkeratotic papules, palmoplantar keratoderma and orange-red scaly plaques that can progress to erythroderma, with islands of sparing. According to different studies, the most frequent clinical manifestations in children are type III and IV according to Griffiths classification. Histology is not specific but supports diagnosis. There are multiple therapeutic options, depending on the extension and severity of the disorder. This review presents the case of a 5-year- old child case with a diagnosis of atypical PRP associated with extensive erythema, his response to treatment of systemic corticosteroids and later to topical retinoids being good.


Subject(s)
Humans , Male , Pityriasis Rubra Pilaris , Pityriasis Rubra Pilaris/diagnosis , Retinoids/therapeutic use , Prednisone/therapeutic use , Glucocorticoids/therapeutic use , Pityriasis Rubra Pilaris/complications , Erythema/etiology
4.
文章 在 英语 | IMSEAR | ID: sea-163481

摘要

Ultraviolet light has a wide spectrum of effects on human skin depending on the wavelength. Ultraviolet light often used for therapy of psoriasis comprises of electromagnetic spectrum ranging from 200nm – 400nm. Phototherapy of psoriasis is frequently used in combination regimens to achieve higher clearance rates, longer disease-free intervals, and to reduce the patient`s cumulative radiation dose thereby lowering the carcinogenic risk. Phototherapy may be combined with topical or systemic agents. Topical agents most commonly used are anthralin, tar, vitamin D analogues and tazarotene. Among systemic agents, retinoids are the most widely used. Ultraviolet B wavelengths in the range of 300-313nm is more efficacious than conventional broad-band UV-B [Philips TL 40W/12 lamp] and causes greater remission of psoriatic lesions. 311nm narrow-band UV-B (TL-01) phototherapy is more effective and probably has no greater risk than conventional UV-B (TL-12) phototherapy in the treatment of psoriasis.


Subject(s)
Humans , Phototherapy/methods , Phototherapy/therapeutic use , Phototherapy/trends , Phototherapy/statistics & numerical data , Psoriasis/radiotherapy , Psoriasis/therapy , Retinoids/administration & dosage , Retinoids/therapeutic use
5.
Rev. bras. cir. plást ; 29(3): 324-327, jul.-sep. 2014. ilus
文章 在 英语, 葡萄牙语 | LILACS | ID: biblio-713

摘要

A Síndrome de Vohwinkel ou ceratodermia hereditária mutilante é uma ceratodermia palmoplantar rara, que se manifesta na infância e se torna mais evidente nas fases de adolescência e idade adulta. Doença de herança preferencialmente autossômica dominante, acomete mais mulheres e caucasianos. A hiperceratose palmoplantar difusa, com aparência de favo de mel; as faixas constritivas digitais conhecidas como pseudoainhum, e as placas ceratósicas em forma de estrela-do-mar no dorso de mãos e pés, podendo acometer cotovelos e joelhos, são os achados clínicos característicos da Síndrome. O presente trabalho trata de um relato de caso de uma paciente com Síndrome de Vohwinkel e a terapêutica cirúrgica realizada nas faixas constritivas.


Vohwinkel syndrome, also known as hereditary mutilating keratoderma, is a rare palmoplantar keratoderma that manifests in childhood and becomes more evident in adolescence and adulthood. This preferential autosomal dominant disease affects more women and Caucasians. Its clinical features are diffuse palmoplantar keratoderma with the appearance of honeycomblike constricting rings in the fingers and toes known as pseudo-ainhum, and starfish-shaped keratotic plaques on the dorsal aspect of the hands and feet that can affect the elbows and knees. The present report describes a case report of a patient with Vohwinkel syndrome and surgical correction of the constrictive bands.


Subject(s)
Humans , Male , Adult , History, 21st Century , Retinoids , Callosities , Review Literature as Topic , Keratoderma, Palmoplantar , Transplants , Rare Diseases , Amputation, Surgical , Keratolytic Agents , Retinoids/therapeutic use , Retinoids/pharmacology , Callosities/surgery , Callosities/pathology , Keratoderma, Palmoplantar/surgery , Keratoderma, Palmoplantar/pathology , Transplants/surgery , Rare Diseases/history , Rare Diseases/pathology , Amputation, Surgical/adverse effects , Amputation, Surgical/methods , Keratolytic Agents/therapeutic use , Keratolytic Agents/pharmacology
6.
Indian J Cancer ; 2014 Jul-Sep; 51(3): 293-302
文章 在 英语 | IMSEAR | ID: sea-154386

摘要

Cutaneous T-cell lymphomas (CTCLs) comprise a heterogeneous group of lymphoproliferative disorders characterized by the proliferation of skin-homing post-thymic T-cells. It is the second most common extranodal non-Hodgekin's lymphoma. Many variants of mycosis fungoides and CTCLs are known to date, differing in clinical, histological, and immunophenotypic characteristics. Oral involvement has also been reported rarely in CTCLs. Treatment depends on the disease stage or the type of variant. New insights into the disease and the number of emerging novel therapeutic options have made it an interesting area for dermatologists and medical oncologists.


Subject(s)
Humans , /drug therapy , /surgery , Lymphoma, T-Cell/therapy , Mycosis Fungoides/drug therapy , Mycosis Fungoides/surgery , Mycosis Fungoides/therapy , Retinoids/therapeutic use
7.
Rev. bras. cir. plást ; 29(1): 165-168, jan.-mar. 2014.
文章 在 英语, 葡萄牙语 | LILACS | ID: biblio-110

摘要

A paquidermoperiostose primária é uma doença rara, caracterizada por aposição excessiva do periósteo do crânio, coexistindo com espessamento da epiderme e derme (paquidermia), provocando deformidades grosseiras. Devido à diversidade de estruturas acometidas, há várias opções cirúrgicas e métodos complementares que são utilizados no tratamento das alterações faciais desses pacientes. Esse trabalho apresenta o lifting subperiosteal como uma opção de tratamento estético para a face de pacientes portadores dessa síndrome, através do relato de dois casos operados no Hospital das Clínicas da Universidade Federal de Minas Gerais.


Primary pachydermoperiostosis is a rare disease characterized by excessive skull affixing of the periosteum, coexisting with thickening of the epidermis and dermis (pachydermia), thereby causing gross deformities. Owing to the variety of affected structures, there are several surgical options and complementary methods that are used in the treatment of facial alterations in these patients. This report describes the use of subperiosteal detachment as an aesthetic treatment option for the faces of two patients with primary pachydermoperiostosis, operated at the Hospital das Clínicas of the Federal University of Minas Gerais.


Subject(s)
Humans , Male , Adult , History, 21st Century , Osteoarthropathy, Primary Hypertrophic , Periosteum , Retinoids , Case Reports , Comparative Study , Isotretinoin , Rhytidoplasty , Colchicine , Evaluation Study , Dermatologic Agents , Face , Facial Bones , Osteoarthropathy, Primary Hypertrophic/surgery , Osteoarthropathy, Primary Hypertrophic/pathology , Periosteum/abnormalities , Periosteum/surgery , Periosteum/pathology , Retinoids/therapeutic use , Isotretinoin/therapeutic use , Rhytidoplasty/methods , Colchicine/therapeutic use , Rare Diseases , Rare Diseases/surgery , Rare Diseases/pathology , Dermatologic Agents/therapeutic use , Face/surgery , Facial Bones/surgery
8.
An. bras. dermatol ; An. bras. dermatol;88(4): 585-593, ago. 2013. tab
文章 在 英语 | LILACS | ID: lil-686527

摘要

Actinic keratosis is a common cause of dermatological consultations and it presents a strong association with squamous cell carcinoma. Many substances are used for treatment and prevention, such as retinoids. Nevertheless, many studies on retinoids emphasize their application in treating and preventing non melanoma skin cancers. In this article, we reviewed studies about systemic and topical retinoids used with immunocompetent patients and organ transplant recipients with actinic keratosis, as primary or secondary outcomes. The majority of these papers pointed to a reduction in actinic keratosis count after treatment with retinoids. However, studies need to be better-defined in order to address the lack of a standardized dose, the absence of control groups, the low number of patients and short follow-up periods. Blind, randomized and controlled clinical trials with adequate sample sizes, specifically focused on actinic keratosis, are needed to clarify the real benefit of topical and/or oral retinoids. Comparison of efficacy and safety between oral and topical retinoids in the prevention and treatment of non-melanoma skin cancers and actinic keratosis is an essential pre requisite to establish new strategies to control these conditions. .


A queratose actínica é uma causa comum de consultas dermatológicas e apresenta forte associação com o carcinoma espinocelular. Muitas substâncias são utilizadas para seu tratamento e prevenção, assim como os retinoides. Entretanto, muitos estudos sobre retinoides salientam seu uso no tratamento e prevenção de cânceres de pele não melanoma. Neste artigo, nós revisamos estudos que avaliam o uso dos retinoides sistêmicos e tópicos para pacientes imunocompetentes e imunossuprimidos com queratoses actínicas, como desfechos primários e secundários. A maioria destes estudos mostra redução na contagem das queratoses actínicas após o tratamento com retinóides. Além disso, ajustes no delineamento dos estudos deveriam ser feitos quanto à falta de padronização da dose, ausência de grupos controle, número pequeno de pacientes e tempo curto de seguimento. Ensaios clínicos cegos, randomizados e controlados com tamanho amostral adequado tendo como alvo específico as queratoses actínicas são necessários para esclarecer o real benefício dos retinoides tópicos e/ou orais. A comparação da eficácia e segurança entre os retinoides orais e tópicos na prevenção e tratamento dos cânceres de pele não melanoma e queratoses actínicas é um pré-requisito essencial para o estabelecimento de novas estratégias para o controle destas condições.


Subject(s)
Humans , Keratosis, Actinic/drug therapy , Keratosis, Actinic/prevention & control , Retinoids/therapeutic use , Carcinoma, Squamous Cell/prevention & control , Skin Neoplasms/prevention & control , Treatment Outcome
9.
São Paulo med. j ; São Paulo med. j;131(3): 193-197, 2013. tab
文章 在 英语 | LILACS | ID: lil-679556

摘要

Among the current treatments available for acne vulgaris, many widely practiced options lack support from studies at the best level of scientific evidence. The aim of this narrative review was to present the very latest information on topical and systemic treatments for acne vulgaris. Information from systematic reviews and well-designed clinical trials, obtained through a systematic search of the major medical databases, is emphasized. There are important issues regarding the clinical management of acne that still lack consistent grounding in scientific evidence. Among these are the optimum dose and duration of treatment with oral antibiotics that can be given without inducing bacterial resistance, and the safety of oral isotretinoin.


Na terapêutica atual da acne vulgar, muitas opções amplamente praticadas carecem de respaldo em estudos de melhor nível de evidência científica. O objetivo desta revisão narrativa é apresentar o que há de mais recente no tratamento tópico e sistêmico da acne vulgar. São enfatizadas as informações de revisões sistemáticas e de ensaios clínicos com bom desenho metodológico, obtidas a partir de busca sistematizada nas principais bases de dados em medicina. Há importantes questões referentes à conduta clínica diante da acne que ainda requerem embasamento em evidências científicas consistentes, entre elas a dose e a duração ideais do tratamento com antibióticos orais, sem que ocorra indução de resistência bacteriana, e a segurança da isotretinoína oral.


Subject(s)
Humans , Acne Vulgaris/drug therapy , Dermatologic Agents/therapeutic use , Anti-Bacterial Agents/therapeutic use , Benzoyl Peroxide/therapeutic use , Contraceptives, Oral, Combined/therapeutic use , Evidence-Based Medicine , Isotretinoin/therapeutic use , Retinoids/therapeutic use
10.
Rev. chil. dermatol ; 28(2): 167-172, 2012. ilus
文章 在 西班牙语 | LILACS | ID: lil-718977

摘要

La Pitiriasis Rubra Pilaris (PRP) es una dermatosis papuloescamosa crónica, de etiología desconocida. Se caracteriza por la presencia de pápulas foliculares hiperqueratósicas que coalescen formando placas eritematoescamosas, dejando islotes de piel sana entre las lesiones. Puede llegar a una eritrodermia. Se clasifica en base a la edad de presentación, características morfológicas, evolución y pronóstico. Existen múltiples opciones de tratamiento descritas en la literatura, siendo los retinoides sistémicos el tratamiento de primera línea en estos pacientes. Presentamos dos casos de pacientes con PRP eritrodérmica tratados exitosamente con Acitretín y revisión de la literatura a la fecha.


Pityriasis Rubra Pilaris is a chronic inflammatory dermatosis of unknown etiology, characterized by the presence of multiple follicular papules that coalesce into large erythematous or salmon colored plaques with islands of non-involved skin between them. It can eventually evolve into erythroderma. Descriptions and therapeutic experiences are mainly based on case reports. Today retinoids have become de first line treatment in these patients. We present two cases of erythrodermic PRP treated successfully with Acitretin and an updated review of the literature.


Subject(s)
Humans , Male , Female , Adolescent , Middle Aged , Pityriasis Rubra Pilaris/diagnosis , Pityriasis Rubra Pilaris/pathology , Pityriasis Rubra Pilaris/drug therapy , Acitretin/therapeutic use , Cetirizine/therapeutic use , Dermatitis, Exfoliative , Diagnosis, Differential , Pityriasis Rubra Pilaris/classification , Keratolytic Agents/therapeutic use , Retinoids/therapeutic use
11.
An. bras. dermatol ; An. bras. dermatol;86(4,supl.1): 72-75, jul,-ago. 2011. ilus, tab
文章 在 葡萄牙语 | LILACS | ID: lil-604125

摘要

A hiperceratose epidermolítica é uma forma de ictiose geralmente resistente a tratamentos tópicos. Relata-se um caso de paciente feminina , em acompanhamento na dermatologia desde 1978, com diagnóstico de hiperceratose epidermolítica. Foi tratada inicialmente com queratolíticos, vitamina A oral, ácido tartárico e emolientes tópicos, porém sem melhora no quadro clínico, já que não haviam disponíveis outros tratamentos na época. Em 1986, com o advento dos retinóides orais, foi introduzido o etretinato, e em 1998, foi substituído pelo acitretin, apresentando excelente resposta terapêutica. No momento a paciente está em uso de acitretin 25 mg/dia, completando 23 anos de uso de retinóides orais, com mínimos efeitos adversos e melhora significativa na qualidade de vida.


Epidermolytic hyperkeratosis is a form of ichthyosis normally resistant to topical treatments. Female patient monitored since 1978 diagnosed with epidermolytic hyperkeratosis. Clinical examination showed generalized hyperkeratosis and scaling. Given that no other treatments were available at the time, the patient was initially treated with keratolytic, systemic vitamin A and moisturizers, with no improvement. In 1986, with the development of oral retinoids, etretinate was introduced. In 1998 this was replaced by acitretin. The patient is receiving 25 mg/day after 23 years of using oral retinoids. Significant improvement of the condition and patient's quality of life has been noted.


Subject(s)
Female , Humans , Middle Aged , Acitretin/therapeutic use , Hyperkeratosis, Epidermolytic/drug therapy , Retinoids/therapeutic use , Hyperkeratosis, Epidermolytic/pathology , Time Factors
12.
Rev. clín. pesq. odontol. (Impr.) ; 6(2): 149-154, maio-ago. 2010. ilus
文章 在 英语 | LILACS, BBO | ID: lil-617378

摘要

OBJECTIVE: The purpose of this paper is to review the literature regarding the topical use of the retinoids in the treatment of oral leukoplakia, discussing their mechanisms of action, indications, effectiveness, and adverse effects. DISCUSSION: Leukoplakia is defined as white lesion in oral mucous membrane that cannot be characterized as any other entity and it has potential for malignancy. It does not present specific histological pattern. Due to its potential for malignancy and high prevalence, dentists should know how to diagnose and treat it correctly. The retinoids are chemopreventive agents derived from the vitamin A. They could be either natural or synthetic and their main role is to suppress cellular mitosis. Retinoid derivatives have been used as agents for chemoprevention of malignant transformation of leukoplakias due to its potential effect on the control of the differentiation and proliferation of the epithelial cells, as well as their induction of apoptosis. CONCLUSION: The topical use of retinoids to treat oral leukoplakia is safe, convenient and effective, with minor side effects than the systemic administration.


OBJETIVO: A proposta deste artigo é revisar a literatura referente ao uso de retinoides no tratamento de lecoplasiasbucais e discutir o mecanismo de ação, indicações, efetividade e efeitos adversos. DISCUSSÃO: Leucoplasia é definida como lesão branca na mucosa bucal que não pode ser caracterizada como qualquer outra entidade e tem potencial de malignização. Não apresenta padrão histológico específico e, por causa de seu potencial de malignização e alta prevalência, os cirurgiões-dentistas devem conhecer como diagnosticá-la e tratá-la corretamente. Os retinoidestêm sido usados como agentes quimio-profiláticos para a transformação maligna das leucoplasias por seu efeito potencial no controle da diferenciação e proliferação da células epiteliais, bem como capazes de indução de apoptose. CONCLUSÃO: O uso tópico de retinoides para tratar leucoplasias é seguro, conveniente e efetivo, com efeitos colaterais menores do que na administração sistêmica.


Subject(s)
Humans , Leukoplakia, Oral/drug therapy , Retinoids/therapeutic use , Retinoids/pharmacology , Vitamin A/therapeutic use
13.
Rev. chil. dermatol ; 26(3): 272-278, 2010. ilus
文章 在 西班牙语 | LILACS | ID: lil-569983

摘要

Las queratodermias palmo plantares son un grupo diverso de trastornos caracterizados por el engrosamiento anormal de la piel de las palmas y pantas, que puede ser hereditario o adquirido. Este últimos se define como una hiperqueratosis no hereditaria, no friccionar, de las palmas y/o las plantas que afecta al 50% o más de estas superficies ocrales. Es un trastorno de múltiples etiologías, entre las cuales destacan: el climaterio, asociada a malignidad, a otras dermatosis, acuagénica, causada por infecciones, asociada a medicamentos, a enfermedades sistémicas, y la hiperqueratosis palmo plantar filiforme (queratodemia espinosa). El tratamiento tiende a ser sintomático y puede variar desde medidas simples a queratoliticos tópicos, retinoides sistémicos o cirugía reconstructiva. En algunas queratodermias especificas, como la climatérica y aquellas asociadas a hipotiroidismo, el tratamiento indicado es el reemplazo hormonal, y en las asociadas a malignidad, la extirpación del tumor habitualmente produce la mejoría de to hiperqueratosis.


Palmoplantar keratodermas are a diverse group of disorders characterized by an abnormal thickening of the skin of the palms and soles, which can be hereditary or acquired. The last form above mentioned is defined as a non-hereditary, non-frictional hyperkeratosis of the palms and/or soles that involves 50 percent or more of the surface of these acral areas. This is a disorder produced by multiple causes, among which highlight climacteric, associated with malignancies, related with another dermatoses, aquagenic, infectious, drug-related, related with systemic disease and palmar/plantar filiform hyperkeratosis (spiny keratoderma). Treatment tends to be symptomatic and may vary from simple advices to topical keratolytics, systemic retinoids or reconstructive surgery. In some specific keratodermas like the climacteric one and in those associated with hypothyroidism the recommended treatment corresponds to hormonal replacement and in those associated with malignancies the removal of the tumor usually results in improvement of the hyperkeratosis.


Subject(s)
Humans , Keratoderma, Palmoplantar/etiology , Keratoderma, Palmoplantar/therapy , Keratolytic Agents/therapeutic use , Adrenal Cortex Hormones/therapeutic use , Dermabrasion , Communicable Diseases/complications , Skin Diseases/complications , Neoplasms/complications , PUVA Therapy , Pharmaceutical Preparations/adverse effects , Retinoids/therapeutic use
14.
Dermatol. pediatr. latinoam. (Impr.) ; 7(3): 48-50, sept.-dic. 2009. ilus
文章 在 西班牙语 | LILACS | ID: lil-556371

摘要

Los avances recientes en el campo de la terapia génica, tales como el desarrollo de ARN de cadena corta inhibitorios (ARNsi) capaces de silenciar la expresión de alelos mutados dominantes, ofrecen nuevas expectativas para el tratamiento de la queratodermia plantar en pacientes con paquioniquia congénita. Revisamos brevemente las características clínicas de esta entidad y describimos un estudio de fase 1 que arrojó resultados prometedores. Los mismos serían aplicables a todo el campo de las enfermedades monogénicas.


Recent advances in the genetic therapy field like development of short inhibitory RNA (RNAsi) capable of silencing mutant dominant alleles, offer new expectations for treatment of plantar keratoderma in patients with pachyonichya congenita. We briefly review clinical features of this entity and describe a fase 1 study that showed promising results that may be usefull for the whole monogenic diseases field.


Subject(s)
Humans , RNA, Small Interfering/therapeutic use , Callosities/therapy , Pachyonychia Congenita/therapy , Keratoderma, Palmoplantar/therapy , Phenytoin/therapeutic use , Retinoids/therapeutic use
16.
São Paulo; s.n; 2009. 111 p. ilus, Cd Rom, tab, graf. (BR).
学位论文 在 葡萄牙语 | LILACS, BBO | ID: lil-558041

摘要

O carcinoma epidermóide de boca, neoplasia maligna de boca mais comum, pode originar-se de lesões potencialmente malignas. O ácido retinóico, que atua no crescimento e diferenciação celular, tem sido comumente estudado como um possível quimioterápico na prevenção dessa progressão. Embora o mecanismo pelo qual o ácido retinóico previne essa progressão, e promove a parada do crescimento celular, não esteja estabelecido, sabe-se que os genes homeobox são importantes alvos do ácido retinóico durante o desenvolvimento embrionário e diferenciação tecidual. Este estudo visa determinar se a modulação da expressão desses genes está envolvida na inibição do crescimento pelo ácido retinóico em carcinoma epidermóide de boca. Para isso, foi realizado PCR array para avaliar a expressão de 84 genes homeobox na linhagem celular de carcinoma epidermóide de boca sensível ao ácido retinóico SSC-25, comparando com a linhagem resistente, SSC-9, após o tratamento com ácido retinóico por sete dias. Os resultados mostraram nove genes com perda de expressão e quatro com alta expressão. A validação por qPCR de 7 desses genes confirmou os resultados. Desses, três genes(ALX1, DLX3, TLX1) foram selecionados para terem a expressão avaliada em amostras tratadas por 3, 5 e 7 dias. O gene ALX1 apresentou baixa expressão apenas no dia 7. O gene DLX3 apresentou baixa expressão no terceiro dia com maior decréscimo no sétimo. Já o gene TLX1, mostrou baixa significativa no quinto dia, com valores semelhantes nosétimo. Os dados mostram genes homebox são modulados pelo ácido retinóico em linhagens de carcinoma epidermóide de boca. No entanto, esses genes não parecem ser alvo direto da inibição do crescimento promovida pelo ácido retinóico.


Oral squamous cell carcinoma, the most frequent oral cancer, may arise from potentially malignant oral lesions. Retinoic acid, which plays a role in cell growth and differentiation, has been frequently studied as a possible chemotherapeutic agent in the prevention of this progression. While the mechanism by which retinoic acid prevents progression and suppresses cell growth has not been completely elucidated, it is known that homeobox genes represent important targets of retinoic acid during embryogenesis and differentiation. The present study aims to determine if modulation of the expression of these genes is involved in inhibition of OSCC cell growth by retinoic acid. In order to achieve this goal a PCR array was performed to evaluate the expression of 84 homeobox genes in retinoic acid sensitive SCC-25 cells compared to retinoic acid resistant SCC-9 cells following treatment with retinoic acid for 7 days. Results showed that 9 homeobox genes are downregulated and 4 are upregulated by retinoic acid. The validation confirmed these results. Three genes (ALX1, DLX3, TLX1) were selected for having their expression evaluated on samples treated with retinoic acid for 3, 5 and 7 days. Three different patterns of gene expression were observed. Gene ALX1 showed down-regulation only on day 7. Homeobox gene DLX3 showed reduced expression on day 3 and decreased expression on day 7. TLX1 showed a substantial down-regulation on day 5 with similar values on. The data presented show that a number of homeobox genes aremodulated by retinoic acid in oral squamous cell carcinoma cell lines. However, these genes do not appear to be direct targets of growth suppression trigged by retinoic acid.


Subject(s)
Carcinoma , Genes, Homeobox , Retinoids/therapeutic use
17.
Tunisie Medicale [La]. 2007; 85 (10): 849-856
在 法语 | IMEMR | ID: emr-180185

摘要

Background: With its varied clinical presentation, psoriasis is actually considered as a public health care problem. Severe psoriasis remains difficult to treat


Methods: Our study was retrospective involving 50 patient with severe psoriasis treated in the dermatology unit of military hospital of Tunis from January 1990 to December 2003


Aim of our study was to evaluate actual different therapeutic attitudes and new treatments in case of severe psoriasis


Results: During the 14 years studied the frequency of severe psoriasis was about 19,5%. Extended psoriasis was noticed on 64% of the patients. Arthropatic psoriasis was found on only 6% of the patients. Erythrodermic psoriasis, pustular psoriasis and palmoplantar psoriatic keratoderma were equally noticed in 10% of the patients. PUVA-therapy was firstly prescribed on 96,6% of the patient with extended psoriasis, bettering was noticed on 71% of all them during the 3 first cures. From the 20 patients that have received acitretin, 65% have noticed improvement. Methotrexate and RE-PUVA was secondarily used [only for the cases of psoriasic arthritis amd methotrexate] on respectively 18% and 10% of the patients. All our patients reported worsening on them quality of life


Conclusion: Cyclosporin is more and more used initially in cases of rebellious psoriasis. Actually therapeutic hope leans on biological therapies


Subject(s)
Adult , Adolescent , Child , Female , Humans , Male , PUVA Therapy , Retinoids/therapeutic use , Dermatitis, Exfoliative , Keratoderma, Palmoplantar , Arthritis, Psoriatic , Hospitals, Military , Retrospective Studies
18.
Rev. chil. dermatol ; 22(1): 10-16, 2006. ilus, tab
文章 在 西班牙语 | LILACS | ID: lil-433893

摘要

La elastolisis de la dermis media es un raro desorden idiopático del tejido elástico que se caracteriza por máculas de arrugas finas, debidas a la pérdida de fibras elásticas en la dermis media. Afecta con mayor frecuencia a mujeres de edad media, y se localiza en el cuello, las extremidades superiores y el tronco. En algunas ocasiones esta dermatosis es secundaria al daño actínico, por lo que se hipotetiza que las radiaciones ultravioleta son un factor causal mayor. Presentamos el caso de una paciente de 31 años, portadora de una elastolisis de la dermis media, y realizamos una revisión de la literatura.


Subject(s)
Adult , Humans , Female , Dermis/pathology , Skin Diseases/pathology , Skin Diseases/therapy , Phagocytosis , Elastic Tissue/pathology , Biopsy , Cutis Laxa/pathology , Diagnosis, Differential , Skin Diseases/diagnosis , Skin Diseases/enzymology , Skin Diseases/prevention & control , Matrix Metalloproteinases/metabolism , Ultraviolet Rays/adverse effects , Retinoids/therapeutic use
19.
Rev. chil. dermatol ; 20(3): 185-188, 2004.
文章 在 西班牙语 | LILACS | ID: lil-405267

摘要

La quimioprevención es una estrategia relativamente nueva que tiene como objetivo prevenir, retardar o revertir el proceso de carcinogénesis. El aumento de la incidencia del cáncer de piel observado en las últimas décadas a nivel mundial, ha estimulado la búsqueda de medidas preventivas eficaces en el control de dicho cáncer. Numerosos compuestos han mostrado tener efectos quimioprofilácticos tanto en estudios in vitro como en modelos en roedores de cáncer de piel. Sin embargo, los estudios clínicos que soporta la eficacia de estos agentes son más escasos. En este artículo revisamos algunos de estos compuestos con acción quimiopreventiva para el cáncer de piel, incluyendo melanoma y no melanoma, discutimos su mecanismo de acción y ponemos énfasis en presentar la evidencia que los respalda como tales.


Subject(s)
Humans , Sunscreening Agents/therapeutic use , Chemoprevention , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Cyclooxygenase Inhibitors/therapeutic use , Retinoids/therapeutic use , Skin Neoplasms , Retinoids/adverse effects
20.
Rev. chil. dermatol ; 20(1): 20-25, 2004. ilus
文章 在 西班牙语 | LILACS | ID: lil-407998

摘要

Las poroqueratosis son enfermedades genéticamente determinadas, definidas como desórdenes específicos de la queratinización. Se han clasificado en cinco grandes variantes clínicas: la poroqueratosis actínica superficial diseminada, la poroqueratosis clásica de Mibelli, la poroqueratosis palmoplantar diseminada, la poroqueratosis lineal y la poroqueratosis punctata, caracterizadas histológicamente por la presencia de la lámina cornoide, una columna paraqueratósica densa, bien delimitada del resto de los corneocitos. Todas las formas de presentación tienen riesgo de transformación neoplásica. Los pacientes con poroqueratosis deben fotoprotegerse adecuadamente y evitar la exposición solar excesiva y deben tener un control dermatológico periódico para pesquisar precozmente lesiones neoplásicas.


Subject(s)
Humans , Porokeratosis/etiology , Porokeratosis/therapy , Prognosis , Porokeratosis/surgery , Porokeratosis/drug therapy , Risk Factors , Ultraviolet Rays/adverse effects , Retinoids/therapeutic use
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