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.

A interação escolar de uma criança com Sindrome de Tourette, segundo as percepções de pais e educadores um estudo de caso exploratório

The syndrome of Gilles de la Tourette (ST) can cause marked distress in several areas of functioning. The literature indicates that children with TS may present diffi culties in relationships with teachers and peers. This case study exploration aimed to discover and describe the perceptions of parents and teachers of child with TS enrolled in regular school, regarding the child-school relationships. Interviews were conducted with parents of a 8 years-old child, diagnosed with ST and three educators. Participant observation of everyday children’s school was also performed. The set of reports reveals doubts on the part of respondents to identify involuntary behaviors of the child or to identify intentional actions. It was observed that there is still no connection between parents and educators in meeting the needs of the child. It is assumed that intervention strategies in a multidisciplinary perspective could help parents, educators and health care professionals act in a coordinated way in order to improve the school and social interaction of the child.

Análise microbiológica e imunologica do fluido gengival e proteoma salivar de individuos com Sindrome de Down com doença periodontal

Pós-graduação em Odontologia - FOAR

Tratamento da sindrome da apnéia e hipopnéia obstrutiva do sono (sahos) com aparelhos intrabucais

The apnea and hypopnea sleep obstructive syndrome is a disorder that affects part of adult population. Some characteristics can come with this syndrome that cause social problems such as snoring and excessive daytime sleepiness, associated many times with pulmonary hypertension and cardiac insufficiency. The dentist who is inserted into a multidisciplinary team is a professional highly regarded for diagnosis and treatment of sleep disorders. Among treatments recommended, therapy with intraoral appliance shows an excellent non invasive alternative, achieving satisfactory results.