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1.
J Oral Pathol Med ; 46(5): 321-326, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-27778394

RESUMO

New targeted cancer therapies such as bisphosphonates, denosumab, and bevacizumab are routinely used in adult for the past decades. Their introduction into pediatric medicine is more recent that means there is a paucity of data on long-term effects on dental development and on the risk of osteonecrosis of jaw. This study aimed to outline adverse effects of new targeted cancer therapies on oral cavity including dental abnormalities observed in pediatric population treated with these molecules and the risk of osteonecrosis of the jaw (ONJ). The impact of bisphosphonates and denosumab on bone remodeling (inhibition of osteoclasts) could interfere with teeth exfoliation and eruption processes, causing a tooth eruption delay. This hypothesis was confirmed, bisphosphonate-treated rats presented tooth eruption delay, and bisphosphonate therapy was associated with a mean delay of 1.67 years in tooth eruption in children with osteogenesis imperfecta. Another study showed that the inhibition of RANK/RANKL by denosumab was associated with a lack of tooth eruption in animals. Several animal studies reported that bisphosphonate could also induce dental abnormalities including defective amelogenesis and dentinogenesis in rats, but there is no evidence of such effects in children; only one case of enamel hypoplasia in a child treated for idiopathic arterial calcification with bisphosphate was reported. To date, there has been no reported case of ONJ in children treated with bisphosphonates, denosumab, or bevacizumab.


Assuntos
Antinematódeos/efeitos adversos , Neoplasias Ósseas/tratamento farmacológico , Dente/crescimento & desenvolvimento , Animais , Antinematódeos/uso terapêutico , Bevacizumab/efeitos adversos , Bevacizumab/uso terapêutico , Osteonecrose da Arcada Osseodentária Associada a Difosfonatos , Criança , Denosumab/efeitos adversos , Denosumab/uso terapêutico , Difosfonatos/efeitos adversos , Difosfonatos/uso terapêutico , Humanos , Dente/efeitos dos fármacos , Erupção Dentária/efeitos dos fármacos
2.
J Med Genet ; 53(2): 98-110, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26502894

RESUMO

BACKGROUND: Orodental diseases include several clinically and genetically heterogeneous disorders that can present in isolation or as part of a genetic syndrome. Due to the vast number of genes implicated in these disorders, establishing a molecular diagnosis can be challenging. We aimed to develop a targeted next-generation sequencing (NGS) assay to diagnose mutations and potentially identify novel genes mutated in this group of disorders. METHODS: We designed an NGS gene panel that targets 585 known and candidate genes in orodental disease. We screened a cohort of 101 unrelated patients without a molecular diagnosis referred to the Reference Centre for Oro-Dental Manifestations of Rare Diseases, Strasbourg, France, for a variety of orodental disorders including isolated and syndromic amelogenesis imperfecta (AI), isolated and syndromic selective tooth agenesis (STHAG), isolated and syndromic dentinogenesis imperfecta, isolated dentin dysplasia, otodental dysplasia and primary failure of tooth eruption. RESULTS: We discovered 21 novel pathogenic variants and identified the causative mutation in 39 unrelated patients in known genes (overall diagnostic rate: 39%). Among the largest subcohorts of patients with isolated AI (50 unrelated patients) and isolated STHAG (21 unrelated patients), we had a definitive diagnosis in 14 (27%) and 15 cases (71%), respectively. Surprisingly, COL17A1 mutations accounted for the majority of autosomal-dominant AI cases. CONCLUSIONS: We have developed a novel targeted NGS assay for the efficient molecular diagnosis of a wide variety of orodental diseases. Furthermore, our panel will contribute to better understanding the contribution of these genes to orodental disease. TRIAL REGISTRATION NUMBERS: NCT01746121 and NCT02397824.


Assuntos
Sequenciamento de Nucleotídeos em Larga Escala/métodos , Mutação , Anormalidades Dentárias/genética , Amelogênese Imperfeita/genética , Autoantígenos/genética , Deleção Cromossômica , Transtornos Cromossômicos/genética , Cromossomos Humanos Par 11/genética , Estudos de Coortes , Coloboma/genética , Displasia da Dentina/genética , França , Perda Auditiva Neurossensorial/genética , Humanos , Colágenos não Fibrilares/genética , Reprodutibilidade dos Testes , Colágeno Tipo XVII
3.
Ann Pathol ; 30(3): 240-2, 2010 Jun.
Artigo em Francês | MEDLINE | ID: mdl-20621605

RESUMO

Karyomegalic interstitial nephritis (KIN) is a rare and slowly progressive chronic interstitial nephritis (CIN) (28 cases reported), described for the first time by Mihatsch et al. in 1979. Here, we report on a 50-year-old woman who presented with asymptomatic renal failure and mild proteinuria without hematuria. Renal biopsy showed large tubulo-interstitial fibrosis and massively enlarged tubular epithelial cell nuclei, without viral inclusion. KIN is a rare CIN defined by a karyomegaly of tubular epithelial cell nuclei. Its pathogenesis remains obscure. Nevertheless, an exogenous factor is suspected, ochratoxin A particularly. The familial clustering of patients and the frequency of HLA-A9 and HLA-B35 haplotypes suggest the presence of a possible genetic susceptibility to this disorder.


Assuntos
Nefrite Intersticial/patologia , Feminino , França , Humanos , Pessoa de Meia-Idade
4.
J Clin Pediatr Dent ; 34(1): 35-42, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19953807

RESUMO

UNLABELLED: THE OBJECTIVE of this descriptive study was to define the at-risk occlusal surface to guide the practitioner in the decision of whether to seal or not. METHOD: All dentists affiliated with the French Society of Pediatric Odontology (SFOP) and general practitioners (GP) registered in postgraduate courses in three French dental schools answered the same questionnaire illustrating four occlusal surfaces of permanent molars. It was focused on obtaining an optimal definition of an at-risk occlusal surface. The corresponding four molars were later sectioned to check the answers. Univariate logistic regression analyses and multivariate logistic regression models were tested to identify the factors associated with the at-risk occlusal surface. RESULTS: Eighty-six SFOP dentists and 136 GP filled in the form. Multivariate logistic regression models stratified by type of practice demonstrated that stained fissures (p =0.001) were only associated with at-risk occlusal surface among GP and the morphology of primary fissure (p=0.001) when considering SFOP dentists alone. The multivariate analyses demonstrated that stained fissures, and primary and secondary fissures were linked to the perception of an at-risk occlusal surface. CONCLUSION: An at-risk occlusal surface has narrow and deep primary fissures. Numerous secondary fissures could increase the risk. The coloration of fissures should not be used in the definition because it depends on tooth integrity.


Assuntos
Cárie Dentária/prevenção & controle , Fissuras Dentárias/patologia , Dente Molar/patologia , Cárie Dentária/epidemiologia , Feminino , Odontologia Geral/educação , Humanos , Modelos Logísticos , Masculino , Odontopediatria/educação , Selantes de Fossas e Fissuras/uso terapêutico , Fatores de Risco , Sociedades Odontológicas , Inquéritos e Questionários
5.
Int J Clin Pediatr Dent ; 12(3): 243-246, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31708623

RESUMO

BACKGROUND: Several studies showed that cancer therapies during tooth development are associated with dental abnormalities, including enamel defects, arrested tooth development, microdontic teeth, and agenesis. STUDY DESIGN: We describe the case of a nine-year-old boy treated for acute myeloid leukemia at 15 months of age, who presents several dental abnormalities resulting from anticancer treatment. RESULTS: The patient was included and treated according to the ELAM 02 French protocol. Six years after allogenic hematopoietic stem cell transplantation, the intraoral and radiographic examination highlighted the agenesis of the second permanent molars and three of the four second premolars, microdontia of the first premolars, root stunting of the central incisors and first premolars, rootlessness of the first permanent molars, and enamel defects localized at the permanent incisors and canines. As a first step to reduce enamel defects, restorations with resin composite (Tetric EvoCeram® A2, Ivoclar Vivadent) were performed under a dental dam. Orthodontic treatment was contraindicated due to arrested tooth development, short roots, and a risk of resorption is considered too important. CONCLUSION: The young age at diagnosis (<5 years of age) and intensive chemotherapy (especially myeloablative conditioning with high doses of cyclophosphamide and Busulfan) could explain the severity of the dental abnormalities. This case illustrates the importance of systematically scheduling a dental follow-up in parallel with the onco-hematologic follow-up allowing the clinicians to prevent, detect, and propose early intervention for dental late effects. HOW TO CITE THIS ARTICLE: Hernandez M, Pochon C, et al. Long-term Adverse Effects of Acute Myeloid Leukemia Treatment on Odontogenesis in a Child. Int J Clin Pediatr Dent 2019;12(3):243-246.

6.
BMC Clin Pharmacol ; 8: 3, 2008 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-18547418

RESUMO

BACKGROUND: Conscious sedation is used in dentistry to improve access and quality of care in patients who have difficulty coping with treatment. The aim of this prospective study was to describe a postgraduate training course in conscious sedation for dentists, with specific evaluation of the safe and effective administration of a 50% nitrous oxide in oxygen premix. METHODS: 45 practitioners were trained between 2002 and 2004. They carried out 826 sessions of inhalation sedation in 662 patients. The clinical competency of this group was compared with an expert group. RESULTS: There was no difference between trainees and experts in ability to complete the planned dental treatment under sedation (89.6% vs 93.2%). Trainees were less successful than experts for patients with intellectual disability (87.4% vs 94.2%, p < 0.01). For both groups, the degree of cooperation improved between initial induction and each perioperative step (Wilcoxon test, p < 0.01). However, for trainees, Venham behaviour scores varied with the type of patient (Kruskal Wallis test, p < 0.001). No major adverse effects were recorded. Trainees reported more minor adverse effects than experts (13% vs. 5.3% respectively, Fisher exact test, p < 0.001) CONCLUSION: The trainee practitioners provided effective and safe inhalation sedation. This challenges the current French restriction of the 50% nitrous oxide in oxygen premix to the hospital setting. Further emphasis is required on the teaching of behaviour management skills for patients with intellectual disability.


Assuntos
Educação Continuada em Odontologia/métodos , Óxido Nitroso/administração & dosagem , Óxido Nitroso/efeitos adversos , Anestesia Dentária/efeitos adversos , Anestesia Dentária/métodos , Anestesia Dentária/normas , Sedação Consciente/efeitos adversos , Sedação Consciente/métodos , Sedação Consciente/normas , Odontólogos/normas , Educação Continuada em Odontologia/normas , Humanos , Estudos Longitudinais , Satisfação do Paciente , Estudos Prospectivos
7.
Hum Pathol ; 38(12): 1798-809, 2007 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17714761

RESUMO

We retrospectively reviewed the clinicopathological features of a series of 68 renal AA amyloidosis observations collected between 1990 and 2005. The amyloidogenic disease was a chronic infection (40.8%), a chronic inflammation (38%), a tumor (9.9%), a hereditary disease (9.9%), or was undetermined in 1.4% of cases. Nephrotic syndrome and renal insufficiency were noted in 63.1% and 75% of patients, respectively. The distribution pattern of glomerular amyloid deposits was mesangial segmental (14.7%), mesangial nodular (26.5%), mesangiocapillary (32.3%), and hilar (26.5%). Glomerular form was observed in 80.9% of cases and vascular form in 19.1%. AA amyloidosis-related inflammation was noted in 30 patients (44.1%) and appeared as a multinucleated giant cell reaction (27.9%) or a glomerular inflammatory infiltrate (25%), including glomerular crescents (17.6%). At the end of follow-up, 26 patients (38.2%) showed end-stage renal disease. The clinical presentation of glomerular and vascular forms was distinct with a clear predominance of proteinuria in glomerular form. Inflammatory reaction was preferentially observed in biopsies with a codeposition of immunoglobulin chains and/or complement factors in AA amyloid deposits. The distribution pattern of glomerular amyloid deposits and glomerular inflammatory reaction were independent factors influencing proteinuria level. Tubular atrophy, abundance, and distribution pattern of glomerular amyloid deposits at the time of biopsy were independent predictors of renal outcome. In conclusion, the glomerular involvement appeared as the determining histological factor for clinical manifestations and outcome of renal AA amyloidosis. AA amyloidosis-related inflammation could partly result from an immune response directed against AA fibrils and could induce amyloid resolution and crescents.


Assuntos
Amiloidose/patologia , Inflamação/patologia , Nefropatias/metabolismo , Nefropatias/patologia , Proteína Amiloide A Sérica/metabolismo , Adulto , Idoso , Amiloidose/metabolismo , Feminino , Hematúria/etiologia , Humanos , Hipertensão/etiologia , Inflamação/metabolismo , Nefropatias/complicações , Masculino , Pessoa de Meia-Idade , Proteinúria/etiologia , Estudos Retrospectivos
8.
Clin Infect Dis ; 36(8): 1070-3, 2003 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-12684922

RESUMO

We report 3 cases of renal toxicity associated with use of the antiviral agent tenofovir. Renal failure, proximal tubular dysfunction, and nephrogenic diabetes insipidus were observed, and, in 2 cases, renal biopsy revealed severe tubular necrosis with characteristic nuclear changes. Patients receiving tenofovir must be monitored closely for early signs of tubulopathy (glycosuria, acidosis, mild increase in the plasma creatinine level, and proteinuria).


Assuntos
Adenina/análogos & derivados , Adenina/efeitos adversos , Fármacos Anti-HIV/efeitos adversos , Diabetes Insípido Nefrogênico/etiologia , Síndrome de Fanconi/etiologia , Organofosfonatos , Compostos Organofosforados/efeitos adversos , Insuficiência Renal/etiologia , Acidose/etiologia , Adulto , Creatinina/sangue , Diabetes Insípido Nefrogênico/patologia , Monitoramento de Medicamentos , Síndrome de Fanconi/patologia , Glicosúria/etiologia , Infecções por HIV/tratamento farmacológico , Humanos , Pessoa de Meia-Idade , Proteinúria/etiologia , Insuficiência Renal/patologia , Tenofovir
9.
Am J Kidney Dis ; 40(6): 1179-84, 2002 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-12460036

RESUMO

BACKGROUND: The association of systemic lupus erythematosus (SLE) with minimal change disease (MCD) and/or focal and segmental glomerulosclerosis (FSGS) has been described in isolated case reports. The relevance of this association is still debated. METHODS: We performed a retrospective and descriptive study of 11 patients with SLE who experienced idiopathic nephrotic syndrome (iNS) in an effort to determine the relevance of this unusual combination. RESULTS: All patients fulfilled at least four criteria (renal abnormalities excluded) of the American Rheumatologic Association for the diagnosis of SLE, and all had severe nephrotic syndrome (mean proteinuria, 9.23 +/- 6 g of protein/24 h; serum albumin concentration, 1.48 +/- 0.6 g/dL). None had a past medical history of lupus nephritis or a cause for secondary FSGS. Renal histological examination showed MCD (4 patients) or FSGS (7 patients) without mesangial proliferation. Immunofluorescence was negative in 8 patients. In 3 patients, immune deposits (immunoglobulin G, immunoglobulin M, C3, and C1q) were present, but confined to the mesangium without glomerular changes on light microscopy. The abrupt onset of nephrotic syndrome coincided with the appearance of SLE in 6 patients (group 1) and recurrence of SLE in 3 patients (group 2). Two patients in group 1 experienced SLE recurrence with concomitant relapse of nephrotic syndrome. In only 2 patients (group 3) were the two diseases independent. CONCLUSION: These results suggest that a relevant association exists between both diseases, and SLE could be a precipitating factor for iNS.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Síndrome Nefrótica/etiologia , Adolescente , Adulto , Idade de Início , Criança , Ciclofosfamida/uso terapêutico , Progressão da Doença , Feminino , Glomerulosclerose Segmentar e Focal/patologia , Humanos , Imunossupressores/uso terapêutico , Falência Renal Crônica , Glomérulos Renais/patologia , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/complicações , Síndrome Nefrótica/tratamento farmacológico , Recidiva , Indução de Remissão/métodos , Estudos Retrospectivos , Esteroides/uso terapêutico , Resultado do Tratamento
10.
Am J Kidney Dis ; 40(5): 1091-6, 2002 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-12407656

RESUMO

Renal involvement has been described rarely in monoclonal (type I) cryoglobulinemia, although this complication is frequent among patients with mixed (type II or III) cryoglobulin. We report two patients with glomerulonephritis and monoclonal IgGkappa cryoglobulin. Both patients presented with nephrotic syndrome, microscopic hematuria, and impaired renal function. Hepatitis C serology was negative, bone marrow aspiration was normal, and the renal biopsy specimen showed membranoproliferative glomerulonephritis with glomerular subendothelial deposits of monoclonal IgGkappa. In both cases, circulating cryoglobulin and monotypic tissue deposits were found to be IgG3kappa, suggesting that this isotype may have a particular propensity to cause this type of membranoproliferative glomerulonephritis. Although 18 cases of type I cryoglobulinemia with biopsy-proven glomerulonephritis have been reported to date, this is the first characterization of immunoglobulin heavy-chain isotype in this disease.


Assuntos
Anticorpos Monoclonais/sangue , Crioglobulinemia/classificação , Imunoglobulina G/sangue , Cadeias kappa de Imunoglobulina/sangue , Rim/patologia , Idoso , Crioglobulinemia/patologia , Glomerulonefrite Membranoproliferativa/sangue , Glomerulonefrite Membranoproliferativa/patologia , Humanos , Rim/irrigação sanguínea , Rim/imunologia , Masculino , Pessoa de Meia-Idade
11.
Ann Pathol ; 22(3): 222-5, 2002 Jun.
Artigo em Francês | MEDLINE | ID: mdl-12410107

RESUMO

We report the case of an intravascular large B-cell lymphoma in a 49-year-old woman, which was revealed by proteinuria. This type of lymphoma is very rare and corresponds to the proliferation of malignant lymphoid cells within the lumina of small vessels, resulting in ischemic lesions involving mainly brain and skin. Renal involvement is quite rare and remains asymptomatic, being discovered at autopsy. In this case, the renal biopsy demonstrated the presence of large malignant B cells in the glomerular lumina, associated with minimal glomerular damage. The mechanisms of the proteinuria occurring during intravascular lymphoma remain to elucidate.


Assuntos
Linfoma de Células B/diagnóstico , Proteinúria , Neoplasias Vasculares/diagnóstico , Linfócitos B/patologia , Biópsia , Capilares/patologia , Divisão Celular , Feminino , Humanos , Imuno-Histoquímica , Imunofenotipagem , Rim/irrigação sanguínea , Rim/patologia , Glomérulos Renais/patologia , Linfoma de Células B/patologia , Pessoa de Meia-Idade , Neoplasias Vasculares/patologia
12.
J Dent Child (Chic) ; 71(1): 41-4, 2004.
Artigo em Inglês | MEDLINE | ID: mdl-15272655

RESUMO

This study investigated sealant penetration and dye microleakage of a resin composite system, a compomer system, and a resin-modified glass-ionomer cement in artificially grooved fissures in human molars. Ionosit Seal penetrated 99% of the artificial crevices, whereas Dyract Seal penetrated 97%. The penetration of Helioseal F at 90% was statistically different (P<.0001) from the other 2 materials. Microleakage dye penetration occurred in 22% of the Dyract Seal samples, while it occurred in 5% of Healioseal F and 7% of Ionosit Seal samples. The viscosity and flow properties of the 3 sealants allowed the materials to penetrate the artificial grooves, but they did not seem to affect their sealing capacity.


Assuntos
Fissuras Dentárias/terapia , Infiltração Dentária/diagnóstico , Selantes de Fossas e Fissuras , Corantes , Compômeros , Resinas Compostas , Cárie Dentária/prevenção & controle , Cimentos de Ionômeros de Vidro , Humanos , Teste de Materiais , Dente Molar , Reologia , Viscosidade
15.
Orphanet J Rare Dis ; 4: 6, 2009 Feb 21.
Artigo em Inglês | MEDLINE | ID: mdl-19232125

RESUMO

BACKGROUND: Hypophosphatasia (HP) is a rare inherited disorder characterized by a wide spectrum of defects in mineralized tissues and caused by deficiency in the tissue non-specific alkaline phosphatase gene (ALPL). The symptoms are highly variable in their clinical expression, and relate to numerous mutations in this gene. The first clinical sign of the disease is often a premature loss of deciduous teeth, mostly in the moderate forms. AIM: The purpose of this study was to document the oral features of HP patients and to relate theses features to the six recognized forms of HP in 5 patients with known genotype and to investigate the genotype-phenotype correlations. METHODS: Clinical and radiographic examinations were carried out. We collected medical and dental history in the kindred and biochemical data. Finally, mutations in the ALPL gene were tested by DNA sequencing in SESEP laboratory. RESULTS: We have for the first time related the known dental anomalies which occur as integral features of HP to the recognized clinical forms of HP. We also pointed out striking dental abnormalities which were never described in association with this rare disease. Accurate genotype-phenotype severity correlations were observed. CONCLUSION: This work allowed us to compare orodental manifestations in all the clinical forms of HP within the patient's sample. According to the severity of the disorder, some dental defects were infrequent, while other were always present. The long term prognosis of the permanent teeth varies from a patient to another. As premature loss of primary teeth is often the first, and sometimes the only visible symptom of the milder forms, the paediatric dentist plays a critical role in the detection and diagnosis of the disease.


Assuntos
Fosfatase Alcalina/genética , Hipofosfatasia/genética , Hipofosfatasia/fisiopatologia , Anormalidades Dentárias/genética , Anormalidades Dentárias/fisiopatologia , Adulto , Criança , Pré-Escolar , Feminino , Genótipo , Humanos , Hipofosfatasia/diagnóstico , Hipofosfatasia/diagnóstico por imagem , Lactente , Masculino , Mutação , Fenótipo , Radiografia , Análise de Sequência de DNA , Índice de Gravidade de Doença , Anormalidades Dentárias/diagnóstico , Anormalidades Dentárias/diagnóstico por imagem
16.
J Dent Child (Chic) ; 75(2): 125-33, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18647507

RESUMO

PURPOSE: The aim of this in vitro study was to evaluate the polymerization shrinkage and the microleakage of direct resin-based restorative materials commonly used in pediatric dentistry. METHODS: Standardized Class V cavities overlapping the cementoenamel junction were prepared on the buccal and the lingual surfaces of 40 extracted human mandibular third molars (36 specimens, 4 controls). The cavities were restored with 4 different materials: a packable resin composite (Filtek P60), a compomer (Compoglass F), an ormocer (Admira) and their associated bonding agents (Scotchbond 1, Excite, and Admira Bond, respectively), and a resin-modified glass ionomer (Fuji II LC). The teeth were then immersed in methylene blue solution for 48 hours. Dye penetration was evaluated for all materials, which were analyzed using a multivariate model (alpha=0.05): influence of microleakage score, margin location (enamel/cementum), and preparation location (buccal/lingual). Multivariate analysis was performed using a polychotomous logistic regression. Polymerization shrinkage was evaluated by the disk deflective method. The percentage of polymerization shrinkage (N=3) was evaluated by ANOVA and Tukey test. RESULTS: Regarding polymerization shrinkage, the P60 demonstrated the lowest value, followed by ADM and COF, whereas FLC presented the highest shrinkage-strain (P<.0001). The preparation location had no significant effect on dye penetration (P=.86). Margin location (enamel or cementum) had a significant effect on microleakage (odds ratio [OR]=24.61). Significant differences in the microleakage patterns and scores were also observed between the 4 restorative materials. Admira exhibited the lowest overall microleakage. In comparing Filtek P60, Compoglass F, and Fuji II LC to Admira, P60 showed significantly less microleakage (OR=1.30) than Fuji II LC (OR=1.47), whereas Compoglass F demonstrated the greatest significant overall microleakage (OR=3.15). CONCLUSION: Within the experimental conditions of this in vitro study, the microleakage was significantly lower at the enamel margins than at the cementum margins for the four restorative materials tested. The ormocer and the packable resin composite exhibited the best sealing ability, as well as the lowest polymerization shrinkage. It could not be demonstrated in this study, however, that the higher the polymerization shrinkage was, the lower the marginal sealing ability was.


Assuntos
Infiltração Dentária/classificação , Materiais Dentários/química , Resinas Sintéticas/química , Cerâmica/química , Corantes , Compômeros/química , Resinas Compostas/química , Preparo da Cavidade Dentária/classificação , Cemento Dentário/patologia , Esmalte Dentário/patologia , Adaptação Marginal Dentária , Restauração Dentária Permanente/classificação , Adesivos Dentinários/química , Cimentos de Ionômeros de Vidro/química , Humanos , Teste de Materiais , Metacrilatos/química , Azul de Metileno , Cerâmicas Modificadas Organicamente , Polímeros/química , Cimentos de Resina/química , Silanos/química , Siloxanas/química , Propriedades de Superfície , Fatores de Tempo , Colo do Dente/patologia
17.
Dev Med Child Neurol ; 49(8): 621-5, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17635209

RESUMO

Persons with intellectual disability have difficulty in cooperating with outpatient care, and many are referred for general anaesthesia. Intellectual disability has traditionally been a contraindication for conscious sedation. We evaluated the behavioural impact, effectiveness, and tolerance of sedation in this population using a fixed 50% nitrous oxide/oxygen mixture as a single agent. We used dental treatment as a model of outpatient care; 349 patients (192 males, 157 females; mean age 22y [SD 14]; range 3-81y) were recruited over a 12-month period at seven centres. Sedation was deemed successful if planned dental treatment was completed. Behaviour was scored with the modified Venham scale. Out of 605 sessions, 91.4% were successful. No serious adverse effects occurred. Minor adverse events (such as nausea) occurred in 10.1% of sessions. We conclude that the use of safe and effective conscious sedation may reduce the indications for general anaesthesia.


Assuntos
Anestesia Dentária , Pessoas com Deficiência/psicologia , Óxido Nitroso , Oxigênio , Doenças Dentárias/psicologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Ansiedade ao Tratamento Odontológico/prevenção & controle , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Pacientes Ambulatoriais , Doenças Dentárias/terapia
18.
Am J Respir Crit Care Med ; 173(7): 777-80, 2006 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-16399989

RESUMO

Light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic kappa light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.


Assuntos
Fibrose Cística/etiologia , Hipergamaglobulinemia/complicações , Cadeias Leves de Imunoglobulina/metabolismo , Adulto , Biópsia , Fibrose Cística/diagnóstico , Fibrose Cística/cirurgia , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/metabolismo , Cadeias Leves de Imunoglobulina/ultraestrutura , Transplante de Pulmão , Masculino , Microscopia Eletrônica , Tomografia Computadorizada por Raios X
19.
J Am Soc Nephrol ; 14(2): 359-66, 2003 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-12538736

RESUMO

Autosomal dominant polycystic kidney disease (ADPKD) is a genetically heterogeneous disorder characterized by focal cyst formation from any part of the nephron. The molecular bases include germinal mutation of either PKD1 or PKD2 genes, enhanced expression of several protooncogenes, alteration of the TGF-alpha/EGF/EGF receptor (EGFR) axis, and disturbed regulation of proliferative/apoptosis pathways. To identify new locations of ADPKD related oncogenes and/or tumor suppressor genes (TSG), comparative genomic hybridization (CGH) and loss of heterozygosity (LOH) analyses were performed for a series of individual cysts (n = 24) from eight polycystic kidneys. By CGH, imbalances were detected predominantly on chromosomes 1p, 9q, 16p, 19, and 22q in all tissues. DNA copy number gain was seen on chromosomes 3q and 4q in five samples. The CGH data were supplemented by LOH analysis using 83 polymorphic microsatellite markers distributed along chromosomes 1, 9, 16, 19, and 22. The highest frequency of LOH was found on the 1p35-36 and 16p13.3 segments in cysts from seven samples. Allelic losses on 9q were detected in six, whereas deletions at 19p13 and 22q11 bands were observed in three polycystic kidneys. These results indicate that the deleted chromosomal regions may contain genes important in ADPKD initiation and progression.


Assuntos
Aberrações Cromossômicas , Rim Policístico Autossômico Dominante/genética , Cromossomos/genética , Análise Citogenética , DNA/genética , Feminino , Dosagem de Genes , Humanos , Perda de Heterozigosidade , Masculino , Hibridização de Ácido Nucleico , Rim Policístico Autossômico Dominante/patologia
20.
J Am Soc Nephrol ; 13(2): 379-387, 2002 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-11805165

RESUMO

Fourteen cases of mesangial IgG glomerulonephritis characterized by exclusive or predominant mesangial IgG deposits are reported. The median age at onset was 19 yr (range, 13 to 47 yr). No patient exhibited evidence of systemic lupus erythematous or other systemic diseases. Proteinuria was present in all cases (median, 2.4 g/d; range, 1 to 13 g/d), microscopic hematuria in 12 cases, and macroscopic hematuria in two cases. Five patients were hypertensive at the time of referral. In all cases, renal biopsies revealed mesangial IgG deposits and varying degrees of mesangial matrix expansion, in the absence of significant mesangial cell proliferation. Complement component (mainly C3) deposits were present in virtually all cases. Subepithelial deposits were also noted in nine cases. IgG deposits were polyclonal and consisted mainly of IgG1 and IgG3 subclasses. In electron-microscopic analyses, deposits were electron dense and granular. Treatment was purely supportive. After a mean follow-up period of 11 yr, seven patients had experienced progression to chronic renal failure, including four who had reached end-stage renal failure. Three patients exhibited persistently normal renal function. For one patient, a symptomatic recurrence of mesangial IgG deposits in the renal graft was diagnosed 4 yr after renal transplantation. Such a recurrence highlights the specificity of this type of glomerulonephritis. Mesangial IgG glomerulonephritis is a distinct, albeit rare, type of glomerulonephritis that exhibits far from benign outcome and may recur in renal transplants.


Assuntos
Mesângio Glomerular , Glomerulonefrite/metabolismo , Imunoglobulina A/metabolismo , Adolescente , Adulto , Biópsia , Feminino , Seguimentos , Mesângio Glomerular/metabolismo , Mesângio Glomerular/patologia , Glomerulonefrite/classificação , Glomerulonefrite/patologia , Glomerulonefrite/terapia , Humanos , Rim/patologia , Transplante de Rim , Masculino , Microscopia Eletrônica , Pessoa de Meia-Idade
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