Clinical presentation and salivary gland histopathology of paediatric primary Sjogren's syndrome

ObjectiveExplore the presentation, diagnostic criteria and exocrine gland histopathology of paediatric primary Sjogren's syndrome (PPSjS).MethodsA case series of 8 children is reported and American-European Consensus Group (AECG-2002) criteria were examined, as well as minor labial salivary and lachrymal gland biopsies, which were scored by a pathologist blinded to outcome. For all cases, connective tissue diseases and parotid-related infectious disease were excluded.ResultsAge at onset varied from 5-13 years old; 6 were females, all followed from diagnosis up to the last visit (1-10 years). The main features at presentation were recurrent tender parotid swelling and sialectasis imaging, with decreased salivary function assessed by Tc-99 scintigraphy. Mild sicca symptoms were observed in 4/8 cases. Systemic features, including fatigue, myalgia, arthritis, tenosynovitis, joint contractures, transient Raynaud's and high ESR, were recorded at onset. Autoantibody profile was unremarkable for diagnosis, while lymphocytic infiltration of labial salivary glands and sialectasis were observed in all biopsies (8/8). In lachrymal glands, massive lymphocytic infiltration and lymphocytic gastritis were observed during complementary assessment. Flares were treated with low dose steroids and long-term use of hydroxychloroquine (5/8), although only 318 fulfilled AECG-2002 diagnostic criteria, throughout the disease course.ConclusionPPSjS is rare, slowly progressive and its early presentation is variable. Standardised diagnostic algorithms should include recurrent parotid swelling and early diagnosis should rely mostly on salivary and lachrymal gland histopathology in this age group.

SINDROME DE DUBOWITZ

A presentation of two cases of Dubowitz syndrome in monozigous twin girls. The syndrome is a rare congenital disorder, the main clinical aspects of which include retarded intrauterine and post-natal growth, microcephaly, peculiar face and an eczemic rash, resulting from photosensitivity of the regions exposed to sunlight.

Sindrome das bandas ou constricoes amnioticas: Relato de caso

The authors present a case of constriction band syndrome in a 20-year-old male. There were constriction rings in the interphalangeal joints of hands and in the thigh, shortening of the lower limb and atrophic left gastrocnemius muscle. These findings belong to the described syndrome, which is uncommon and has no genetic implication. Personal antecedent: convulsive syndrome and corrective surgery for congenital bent foot.

Factores que afectan el rendimiento escolar de los pobres: revisión de investigaciones educacionales, 1980-1995

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Capital social y políticas públicas en Chile: investigaciones recientes: volumen I

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Capital social y políticas públicas en Chile: investigaciones recientes: volumen II

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Aproximación al estado de situación de la oferta y demanda de información de investigaciones sobre drogas en Chile

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Utilización de informaciones estadísticas en investigaciones demográficas sobre Chile realizadas por CELADE

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Algunos hallazgos de investigaciones de CELADE: utilidad de estos hallazgos para mejorar programas de planificación familiar en América Latina

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Las investigaciones sobre tierras indígenas en Brasil: una evaluación crítica

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