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.

A new short and simple health-related quality of life measurement for paediatric rheumatic diseases: initial validation in juvenile idiopathic arthritis

Objective. To develop and validate a new short and simple measure of health-related quality of life (HRQL) in children with juvenile idiopathic arthritis (JIA).Methods. The Paediatric Rheumatology Quality of Life Scale (PRQL) is a 10-item questionnaire that explores HRQL in two domains: physical health (PhH) and psychosocial health (PsH). Validation of the parent proxy report and child self-report versions of the instrument was accomplished by evaluating 472 JIA patients and similar to 800 healthy children. Validation analyses included assessment of feasibility, face and content validity; construct and discriminative ability; internal structure and consistency; test-retest reliability; responsiveness to clinical change; and minimal clinically important difference.Results. The PRQL was found to be feasible and to possess both face and content validity. The PRQL score correlated in the predicted range with most of the other JIA outcome measures, thereby demonstrating good construct validity, and discriminated well between different levels of disease severity. Assessment of internal structure (factor analysis) revealed that the PhH and PsH subscales identify two unambiguously separated domains. The internal consistency (Cronbach's alpha) was 0.86. The intraclass correlation coefficient for test-retest reliability was 0.91. The PRQL revealed fair responsiveness, with a standardized response mean of 0.67 in improved patients. Overall, the PRQL appeared to be more able to capture physical HRQL than psychosocial HRQL.Conclusion. The PRQL was found to possess good measurement properties and is, therefore, a valid instrument for the assessment of HRQL in children with JIA. This tool is primarily proposed for use in standard clinical care.

Paediatric rheumatology - A global perspective

This chapter aims to give a global perspective to paediatric rheumatology. The main points covered are the incidence, recognition of paediatric autoimmune diseases, and ethnic/geographic distribution. The most prevalent disease is juvenile idiopathic arthritis; robust data are still required for childhood-onset systemic lupus erythematosus, dermatomyositis, and scleroderma. Mimicking or overlapping infections are a major challenge in developing countries, and immunization policies in our patients in these areas need specific attention. The delivery of paediatric rheumatology care is also overviewed. Discrepancies in health-care resources and priorities are found in developing countries. Although most anti-rheumatic treatments are available worldwide, they are prohibitively expensive in many countries. For more traditional antirheumatic drugs there is still an ongoing need for good core outcome data across the world to ensure valid comparisons. Parent/patient education has been implemented worldwide in paediatric rheumatology through the power of the Internet. Physician and undergraduate training goals must be met to facilitate competent musculoskeletal assessment, a proper understanding of age-dependent variations, diagnosis, referral to specialists, and improved standards of care.

Schwartz-Jampel Syndrome: a paediatric dentistry approach

Schwartz-Jampel syndrome (SJS) is a rare recessive disorder characterized mainly by myotonia. As the clinical signs and symptoms are manifested in the oromaxillofacial region, paediatric dentists may be first choice of parents that seek information and assistance to their children. A female patient diagnosed with SJS was brought to our clinic for dental treatment with main complain of difficulty on oral hygiene and mastication due to tooth crowding. The treatment included preventive measures, extraction of a supernumerary tooth and the maxillary primary second molars. Furthermore, the patient was referred to orthodontic treatment for correction of tooth crowding. When dealing with children with confirmed diagnosis of SSJ, paediatric dentists should be understand the need of planning the dental treatment considering the characteristics and possible complications associated with the syndrome in order to reduce the risks to patients, especially when surgical procedures are necessary.

DNA damage in lymphocytes and buccal mucosa cells of children with malignant tumours undergoing chemotherapy

The aim of the present study was to evaluate DNA damage (micronucleus) in cytokinesis-blocked lymphocytes and exfoliated buccal mucosa cells from children with malignant tumours and under chemotherapy. Micronucleated cells (MNCs) were assessed from children before and during chemotherapy. A total of 21 healthy children (controls), matched for gender and age, were used as control. The results pointed out higher frequencies of micronucleated lymphocytes in children with malignant tumour before any therapy when compared to healthy probands. Furthermore an increase of micronucleated lymphocytes during chemotherapy was detected when compared to the data obtained before chemotherapy. No statistically significant increases of MNCs were noticed in buccal mucosa cells at any of the timepoints evaluated. Taken together, these data indicate that the presence of malignant tumours may increase the frequency of DNA damage in circulating lymphocytes, these cells being more sensitive for detecting chromosome aberrations caused by anti-cancer drugs.

EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

Objectives To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schonlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.Methods The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES-supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis <= 18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, kappa-agreement) using as 'gold standard' the final consensus classification or original treating physician diagnosis.Results A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with kappa-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall. of 0.79 (95% CI 0.73 to 0.84).Conclusion EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

Use of extensible internal device in the femur of young dogs

An extensible internal device (EID) was developed to preserve growth plate during the treatment of fracture complications or segmental bone loss from tumour resection in children. Since this type of extensible, trans-physeal, internal fixation device has only been used in a few paediatric cases; the aim of this study was to evaluate an in vivo canine study, a surgical application of this device, and its interference with longitudinal growth of the non-fractured distal femur. Ton clinically healthy two- to three-month-old poodles weighing 1.5-2.3 kg were used. Following a medial approach to the right distal femur, one extremity of the EID, similar to a T-plate, was fixed in the femoral condyle with two cortical screws placed below the growth plate. The other extremity, consisting of an adaptable brim with two screw holes and a plate guide, was fixed in the third distal of the femoral diaphysis with two cortical screws. The EID was removed 180 days after application. All of the dogs demonstrated full weight-bearing after surgery. The values of thigh and stifle circumferences, and stifle joint motion range did not show any difference between operated and control hindlimbs. The plate slid in the device according to longitudinal bone growth, in all but one dog. In this dog, a 10.5% shortening of the femoral shaft was observed due to a lack of EID sliding. The other dogs had the some longitudinal lengths in both femurs. The EID permits longitudinal bone growth without blocking the distal femur growth plate if appropriately placed.

Salivary alpha amylase and cortisol levels in children with global developmental delay and their relation with the expectation of dental care and behavior during the intervention

The purpose of this study was to analyze the alpha-amylase (sAA) and cortisol levels in children with Global developmental delay (GDD) before and after dental treatment and its association with the children's behavior during treatment. The morning salivary cortisol levels and activity of sAA of 33 children with GDD were evaluated before and after dental treatment and were compared to 19 healthy children. The behavior of children with GDD during dental care was assessed by the Frankl scale. Children with GDD showed lower levels of sAA activity than healthy children, but this result was not significant. The salivary cortisol levels were similar between GOD and healthy children. GDD children showed increased levels of sAA (but not cortisol) prior to the dental treatment as compared to the post-treatment phase. GOD children who showed less favorable behavior during dental care had higher levels of sAA and salivary cortisol than GOD children with more favorable behavior, but only the sAA results were significant. In conclusion, GOD children show hyperactivity of the SNS-axis in anticipation of dental treatment which indicates the need for strategies to reduce their anxiety levels before and during dental care. (C) 2011 Elsevier Ltd. All rights reserved.

A combination of clinical and microbiological management of generalized aggressive periodontitis in primary teeth. A case report

International Journal of Paediatric Dentistry 2012; 22: 310316 Background. Generalized aggressive periodontitis (GAP) in primary teeth is a rare periodontal disease that occurs during or soon after eruption of the primary teeth. An association with systemic diseases is a possibility. Case Report. A 4-year-old Brazilian girl presented with GAP involving the entire primary dentition. The patient and her parents and sister were subjected to microbiological testing to identify the microorganisms involved in the disease. The patient underwent tooth extraction to eradicate the disease and received a prosthesis for the restoration of masticatory function. After the permanent teeth erupted, fixed orthodontic appliances were place to restore dental arch form and occlusion. Conclusions. The results show the importance of an early diagnosis of GAP and of a multidisciplinary approach involving laboratory and clinical management to treat the disease and to restore masticatory function, providing a better quality of life for patients.

Effect of a calcium hydroxide/chlorhexidine paste as intracanal dressing in human primary teeth with necrotic pulp against Porphyromonas gingivalis and Enterococcus faecalis

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Molar incisor hypomineralization: prevalence, severity and clinical consequences in Brazilian children

Background.The prevalence of molar incisor hypomineralization (MIH) varies considerably around the world; however, few studies have examined MIH in South American countries.Objective.To evaluate the prevalence, severity, and clinical consequences of MIH in Brazilian children residing in rural and urban areas of the municipality of Botelhos, Minas Gerais, Brazil.Methods.Children aged 6 to 12 years (n = 918) with all four-first permanent molars erupted had these teeth evaluated according to the European Academy of Paediatric Dentistry (EAPD) criteria. The examinations were conducted by two previously trained examiners, and the dental impact caused by MIH was evaluated with the Decayed, Missing and Filled Teeth (DMFT) index (WHO).Results.Molar incisor hypomineralization was present in 19.8% of the 918 children, with a higher prevalence in rural areas. The majority of the defects presented were demarcated opacities without post-eruptive structural loss, which has been considered as mild defects. Children with MIH had higher DMFT values.Conclusion.Despite the high prevalence of MIH, the severity of the defects was mild. The results indicate a positive association between MIH and the presence of dental caries.

Increase in severity of molar-incisor hypomineralization and its relationship with the colour of enamel opacity: a prospective cohort study

Background. Predicting risk of posteruptive enamel breakdown (PEB) of molar-incisor hypomineralization (MIH) opacity is a difficult but important clinical task. Therefore, there is a need to evaluate these aspects through longitudinal studies.Objective. The aim of this longitudinal study was to analyse the relationship between colours of MIH opacity of children aged 6-12 (baseline) and other clinical and demographic variables involved in the increase in severity of MIH.Materials and methods. A blinded prospective 18-month follow-up was conducted with 147 individuals presenting mild MIH. Tooth-based incidence of increase in severity of MIH (PEB or atypical restorations) was used as dependent measurement. Enamel opacities were recorded according to colour shades of white, yellow and brown, allowing assessment of susceptibility to structural loss over time, according to colour of MIH opacity. Poisson regression models were used to adjust the results for demographic and clinical variables.Results. Brown and yellow MIH opacities were at higher risk for PEB and atypical restorations than those of white ones, even after adjustment for clinical and demographic variables.Conclusion. Teeth presenting mild MIH severity associated with yellow and brown enamel opacities were at high risk for increase in severity of MIH than lighter ones. This result could help clinicians determine a risk-based treatment for children with MIH.

Bond strength and morphology of resin materials applied to the occlusal surface of primary molars

International Journal of Paediatric Dentistry 2012; 22: 435441 Background. Hydrophilic adhesives may be used as pit and fissure sealants (sealants), but there is concern about the ability of self-etching adhesives to bond sealants to enamel. Aim. To study the bond strength (BS) and morphology of adhesive systems used as sealants. Design. OptiBond FL, OptiBond All-in-One, combined OptiBond All-in-One + OptiBond FL adhesive, and Fluroshield were applied to the occlusal surfaces of 16 primary molars (n = 4). Teeth were stored in distilled water (24 h at 37 degrees C) and sectioned through the interface to obtain sticks (0.8 mm2) tested under a tensile load (0.5 mm/min). Failure modes were observed. Data were analysed by ANOVA and Tukeys tests (a = 5%). The morphology of 12 primary molars was examined in terms of the etching pattern and resin reproduction. Results. Differences in the BS were found (P = 0.001), with OptiBond FL showing the highest (36.84 +/- 5.7 MPa), Fluroshield (24.26 +/- 2.13 MPa) and OptiBond All-in-One (17.12 +/- 4.97 MPa) similar, and OptiBond All-in-One + OptiBond FL adhesive the lowest (9.8 +/- 2.94 MPA). OptiBond FL showed the best results in terms of morphology. Conclusion. Under the conditions of this study, OptiBond FL was the best material to be used for sealing.

Apresentação pediátrica da síndrome antifosfolípide

OBJETIVO: Descrever as características clínicas, laboratoriais e de desfecho de uma série de casos com diagnóstico definido de síndrome antifosfolípide (SAF) pediátrica. MÉTODOS: Estudo observacional-retrospectivo de referência pediátrica terciária, que identificou os casos por meio de evento vascular, trombose venosa ou oclusão arterial, determinação de anticorpos anticardiolipina (IgG e IgM) e teste do anticoagulante lúpico. RESULTADOS: Foram identificados cinco casos atendidos nos últimos cinco anos, sendo dois meninos e três meninas. A trombose venosa ocorreu em seios venosos cerebrais (2), fibular (2), poplítea (1), femoral (1), intestinal (1), renal (1), acompanhados por oclusão arterial intestinal (1), de artéria renal (1) e artéria digital (1), esta resultando gangrena periférica como evento recorrente durante anticoagulação com warfarina. Um abortamento espontâneo ocorreu em uma adolescente em vigência de púrpura trombocitopênica, evoluindo com anemia hemolítica (síndrome de Evans) e desfecho fatal por hemorragia. A investigação laboratorial em todos os casos resultou, pelo menos, uma determinação positiva de anticardiolipina IgG e/ou IgM, sendo considerados como SAF primária. Três dos casos estão em seguimento com anticoagulação oral. CONLUSÃO: A trombose venosa cerebral e de extremidades foram os eventos mais freqüentes. A presente série alerta para a investigação e o diagnóstico precoces, com abordagem multidisciplinar para o tratamento.