Morphological aspects of the gingiva in children with Down's syndrome during experimental gingivitis

In a previous investigation, children with Down's syndrome (DS) showed an earlier, more rapid and more extensive gingival inflammation than normal healthy control children. These differences in gingival inflammation may be the result of aberrant morphology of the gingiva related to the genetic disorder in DS children. The aims of the present study were (i) to describe the structural composition of "normal" gingiva in DS compared to control children, (ii) to analyse the histological changes in the gingiva during plaque development and (iii) to investigate whether the clinical findings could be supported by morphological observations. The study was carried out in 8 DS and 8 matched control children. Their ages ranged from 5-10 years. Gingival normality was guaranteed by strict oral hygiene procedures. During a period of 21 days in which oral hygiene was abolished, gingival biopsies were taken from buccal sites of deciduous teeth following a predetermined schedule on days 0, 7, 14 and 21. Results on day 0 showed no morphological differences between the DS and control children regarding oral epithelium, junctional epithelium or connective tissue. During the experimental phase of the study, the amount of plaque accumulation in the DS children gave rise to a more extensive gingival inflammation than in the control children. The gingival inflammation in the DS group started earlier and included: (1) an acute inflammatory response, (2) an increase of the junctional epithelium area, (3) an increase of the infiltrated connective tissue area (ICT) and (4) a decrease in collagen fibre density of about 35-40% compared to day 0. The same phenomena were not seen until 7 days later in the control group. Conversely, the development of a perivascular lymphocyte infiltrate (LI) in the DS children was delayed compared to the control group. This may be caused by the impaired delayed-type hypersensitivity response in DS children. The development of 2 separate infiltrates (ICT and LI) in this age group and the different temporal development of ICT (day 7 for the DS and day 14 for the control group) and LI (day 14 for the DS and day 7 for the control group) does suggest different immunological mechanisms for both areas and both groups

Doença oclusiva progressiva das artérias cerebrais associada a síndrome de Down: considerações sobre a etiopatogenia

Registro de um caso que pode ser o segundo na literatura mundial de associação de "moyamoya" com síndrome de Down, caracterizando-se pela permeabilidade das artérias carótidas internas, cerebrais médias e anteriores. O comprometimento da circulação distal poderia levantar a possibilidade de uma alteração da microcirculação capilar e, inclusive, da ação de alguma substância não determinada, fatores que poderiam explicar a localização e bilateralidade das lesões

Síndrome de Down e Moyamoya: estudo através de metanálise Moyamoya and Down syndrome: study conducted by meta-analysis

Apresentamos o estudo clínico-epidemiológico de dois pacientes e metanálise (período 1977-2000) sobre a comorbidade entre síndrome de Down (SD) e síndrome de "moyamoya" (SMM). Entre os 42 pacientes catalogados no presente estudo, a metanálise permitiu verificar: maior número de publicações de pesquisadores do Japão e Estados Unidos, seguidos pelo Brasil e Itália; predomínio do acidente vascular cerebral (AVC) em lactentes e pré-escolares; sintomatologia inaugural, hemiparesia (78,6%), distúrbio da fala (26,2%); predomínio de infarto isquêmico (76,2%); episódios isquêmicos recorrentes (62%); comprometimento bilateral (83,3%). Esta análise permitiu concluir que, na investigação clínico-neurológica de pacientes com SD e episódios de hemiparesia aguda, a SMM deve ser incluída como diagnóstico mais provável .<br>We present a clinic-epidemiological study of two patients and meta-analysis (period 1977-2000 ) of the co-morbidity of the Down syndrome (DS) and moyamoya syndrome (MMS). Among the 42 patients listed in this survey, meta-analysis permitted to find the highest number of publications by researchers from Japan and United States, followed by Brazil and Italy; prevalence of cerebrovascular disease in suckling and pre school children; first symptomatology was hemiparesis (78.6%), speech disorders (26.2%); ischemic infarction (76.2%); recurring ischemic episodes (62%); bilateral impairment (83.3%). This analysis led to the conclusion that in the clinic-neurological investigation of DS patients with acute hemiparesis episodes, MMS should be included as the most probable diagnosis