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.

Hemangioendothelioma of bone in a patient with a constitutional supernumerary marker

A 13-year old girl was diagnosed as having a bone hemangioendothelioma. Cytogenetic studies identified the presence of a small supernumerary marker chromosome in this patient. Classical cytogenetic methods using G-, C-, Ag-NOR-banding were supplemented by spectral karyotyping (SKY) and fluorescence in situ hybridization to reveal a karyotype 47,XX,+mar.ish der(22)(D22S543+) karyotype in cells derived from the tumor and lymphocytes. These findings suggest that the supernumerary marker chromosome originated from the proximal centromeric region of chromosome 22, and that trisomy of the region 22q11: was not associated with adverse phenotypic effects, but that the presence of trisomy 22q11 may be related to the development of this tumor. (C) Elsevier B.V., 1999. All rights reserved.

Soldiers initiate foraging activities in the subterranean termite, Heterotermes tenuis

Caste polyethism has been recorded in some termite species, however the foraging behavior of subterranean termites remains poorly known. Heterotermes tenuis Hagen (Isoptera: Rhinotermitidae) is a subterranean termite that is native to Brazil and is an agricultural and urban pest. The aim of this study was to investigate which caste acts as scouts when searching for food sources and determinate the percentages of each caste present in the foraging territories of field colonies of H. tenuis. Our results showed no significant differences among the caste proportions present in the foraging territories of the three colonies studied in the field. Laboratory experiments showed that minor soldiers were the most frequent initiators of foraging activities. This result suggests that the exploratory phase of the foraging behavior may be regulated by the number of soldiers present in the foraging territories of each colony.

Double teeth in primary dentition: Report of two clinical cases

Odontogenic anomalies can occur as a result of conjoining or twinning defects. These include fusion, gemination and concrescence. This article presents two case reports of double teeth. In the first case reported, a 4-year-old white boy presented primary double teeth associated to the absence of the right permanent mandibular lateral incisor. In the second case, a 5-year-old white girl had a family history of anomaly in primary dentition. The girl and her mother presented double teeth in the primary dentition. Her mother showed hypodontia in the permanent dentition. Extra and intra oral clinical examination was made in both cases. Radiographic analyses showed the involvement of the permanent tooth. Authors conclude that double teeth in primary dentition have to be carefully analysed as they may be associated with anomalies in the permanent dentition. Correct diagnosis of the condition implicates in a better prognosis for the patient.

A militarização da infância: expressões do nacionalismo na cultura brasileira

O texto compreende um estudo sobre as práticas de militarização da infância, isto é, práticas de natureza patriótica, cívico-militar que predominaram no ensino primário, no início do século XX. Nesse sentido, destaca a introdução da disciplina Ginástica e exercícios militares nos programas de ensino e seus desdobramentos mediante a criação dos Batalhões Infantis. Analisa, também, o escotismo escolar, movimento efervescente no estado de São Paulo nas décadas de 1910 e 1920, identificando-o como mais uma expressão do militarismo e do nacionalismo na educação brasileira. Para a realização deste estudo foram utilizadas fontes manuscritas encontradas no Arquivo do Estado de São Paulo e periódicos educacionais da época.

Tricotilomania: dificuldades diagnósticas e relato de dois casos

OBJETIVO: Relatar dois casos de tricotilomania, um transtorno psiquiátrico ainda subdiagnosticado e que pode estar associado a problemas sociais e clínicos relevantes. Pretende-se destacar as características clínicas, discutindo as implicações do diagnóstico precoce para a evolução dos pacientes. DESCRIÇÃO do CASO: Uma adolescente com diagnóstico de tricotilomania pura e outra menina cujo quadro estava associado ao transtorno obsessivo-compulsivo. Embora com o tratamento, a evolução de ambas tenha sido favorável, houve demora significativa para estabelecer o diagnóstico e encaminhá-las a um serviço de saúde mental, com prejuízos escolares e sociais. COMENTÁRIOS: A tricotilomania difere dos quadros benignos e transitórios de arrancar cabelos observados nos primeiros anos de vida e ainda é subdiagnosticada. A vergonha dos sintomas observada nos portadores e o desconhecimento por parte dos profissionais de saúde contribuem para essa situação. O quadro pode ser grave, particularmente se acompanhado de tricofagia. Profissionais da saúde precisam identificar o transtorno precocemente e encaminhar as crianças para tratamento especializado antes das possíveis complicações clínicas e repercussões psicossociais

Intervenção fonoaudiológica na síndrome de kabuki: relato de caso

TEMA: este trabalho teve como objetivo descrever o processo de intervenção fonoaudiológica em um caso de síndrome de Kabuki, com ênfase na terapia de linguagem. PROCEDIMENTOS: trata-se de relato de caso de uma criança de seis anos e seis meses de idade, gênero feminino, com diagnóstico de síndrome de Kabuki, que realizou terapia fonoaudiológica durante três anos em uma clínica-escola. Foi realizada análise documental dos dados do prontuário relativos à anamnese, avaliação e relatórios terapêuticos fonoaudiológicos, exames e avaliações multidisciplinares. Aos três anos e cinco meses, a criança passou por avaliação fonoaudiológica e apresentou, como histórico, características típicas da síndrome de Kabuki: alteração cardíaca, episódios de pneumonia, otite média secretora recorrente, alterações ortopédicas e crises convulsivas. Na avaliação fonoaudiológica, apresentou tempo de atenção auditiva e visual reduzido, dificuldade de compreensão oral, comunicação por meio de poucos gestos e vocalizações e problemas de comportamento. RESULTADOS: o processo terapêutico teve como objetivo principal promover o desenvolvimento da linguagem com ênfase na interação social por meio de atividades lúdicas, orientação familiar e escolar. Foi observada melhora na compreensão e emissão oral, no contato social e manutenção da atenção. CONCLUSÃO: este estudo permitiu divulgar o percurso de atuação fonoaudiológica na síndrome de Kabuki, do processo diagnóstico à terapia de linguagem. Verificou-se que a terapia foi eficaz no que se refere às habilidades comunicativas e de interação social. Esses dados podem contribuir para elucidar lacunas existentes na literatura acerca da terapia fonoaudiológica na síndrome em questão e subsidiar intervenções clínicas nesses casos.

DUPLICATION OF THE SHORT ARM OF THE X-CHROMOSOME IN MOTHER AND DAUGHTER

An 11-year-old girl with short stature, mental retardation, and mild dysmorphic features was found to have an inverted duplication of most of the short arm of the X chromosome [dic inv dup(X)(qter --> p22.3 = p22.3 --> cen:)]. Her mother, who is also short and retarded, carries the same duplication. Fluorescence in situ hybridization with an X chromosome library, and with X centromere-specific alpha satellite and telomere probes, was useful in characterizing the duplication. In most females with structurally abnormal X chromosomes, the abnormal chromosome is inactivated. Although the duplicated X was consistently late replicating in the mother, X chromosome inactivation studies in the proband indicated that in 11 % of her lymphocytes the duplicated X was active.

MACROSOMIA, OBESITY, MACROCEPHALY AND OCULAR ABNORMALITIES (MOMO SYNDROME) IN 2 UNRELATED PATIENTS - DELINEATION OF A NEWLY RECOGNIZED OVERGROWTH SYNDROME

We describe 2 unrelated patients, a boy and a girl, with an overgrowth syndrome and the following common characteristics: macrocrania, obesity, ocular abnormalities (retinal coloboma and nystagmus), downward slant of palpebral fissures, mental retardation, and delayed bone maturation. Both cases are of sporadic occurrence with no consanguinity between the parents. We suggest that this syndrome is due to a new autosomal dominant mutation and propose to designate it with the acronym of ''MOMO syndrome'' (Macrosomia, Obesity, Macrocrania, Ocular anomalities).

Ameloblastic fibro-odontosarcoma: a case report

This paper reports one case, of an ameloblastic fibro-odontosarcoma (AFOS) affecting the mandible, in a 12-year-old girl. This neoplasm is a rare odontogenic neoplasm. To the authors' knowledge this is the fifteenth case of AFOS reported in English. The patient's chief complaint was a swelling in the face For 6 months. An incisional biopsy was performed diagnosing the case as all ameloblastic fibroma. After radiography ameloblastic fibro-odontoma was diagnosed. Computed tomography was performed and a stereolithography model made to plan the surgical procedures. A hemimandibulectomy followed by a vascularized fibular flap was then proposed. The surgery was uneventful. Microscopic features diagnosed an AFOS. After 23 months of close follow-up there is no sign of recurrence or metastasis. Dental implants were recently placed in the fibular flap.

Cholesteryl ester storage disease. Report of a case.

Cholesteryl ester storage disease (CESD) is a rare disorder of familial incidence characterized by the accumulation of cholesteryl ester and triglycerides in the liver, intestine and bone marrow. Until now only 21 cases have been reported in the literature. We present a 9 months old girl presenting with increased abdominal girth. She had normal liver function tests and increased cholesterol and triglycerides serum levels. The liver biopsy showed many cholesterol cristals seen as needle shaped cristals under polarized light. This is the youngest patient being diagnosed clinically in the literature.

A case of terminal long arm deletion of chromosome 6

A four-year-old girl with deletion of chromosomal band 6q24 → qter is described. Clinical features include growth and psychomotor retardation, microcephaly, convergent strabismus, bulbous nose, long philtrum, short neck and cardiopathy.

Molecular and Cytogenetic Analyses on Brazilian Youths with Pervasive Developmental Disorders

The Pervasive Developmental Disorders (PDDs) constitute a group of behavioral and neurobiological impairment conditions whose main features are delayed communicative and cognitive development. Genetic factors are reportedly associated with PDDs and particular genetic abnormalities are frequently found in specific diagnostic subgroups such as the autism spectrum disorders. This study evaluated cytogenetic and molecular parameters in 30 youths with autism or other PDDs. The fragile X syndrome was the most common genetic abnormality detected, presented by 1 patient with autism and 1 patient with PPD not-otherwise specified (PPD-NOS). One girl with PDD-NOS was found to have tetrasomy for the 15q11-q13 region, and one patient with autism exhibited in 2/100 metaphases an inv(7)(p15q36), thus suggesting a mosaicism 46,XX/46,XX,inv(7)(p15q36) or representing a coincidental finding. The high frequency of chromosomopathies support the hypothesis that PDDs may develop as a consequence to chromosomal abnormalities and justify the cytogenetic and molecular assessment in all patients with PDDs for establishment of diagnosis.

Surgical and clinical management of a patient with Glanzmann thrombasthenia: A case report

A 6-year-old girl with Glanzmann thrombasthenia presented with caries and periapical lesions in the primary mandibular second molars and moderate gingivitis of the maxillary and mandibular anterior teeth. Dental extraction was recommended, and before every surgical intervention, the patient underwent platelet-concentrate transfusion to prevent hemorrhage. Epsilon aminocaproic acid was administered 6 hours before, and 48 hours after every dental procedure to prevent bleeding. In this case, treatment was effective in the prevention of hemorrhagic complications, during the required dental procedures.

Regional odontodysplasia: Case report

Regional odontodysplasia (RO) is a rare developmental anomaly involving both mesodermal and ectodermal dental components in a group of contiguous teeth. It affects the primary and permanent dentitions in the maxilla and mandible or both jaws. Generally it is localized in only one arch. The etiology of this dental anomaly is uncertain. Clinically, affected teeth have an abnormal morphology, are soft on probing and typically discolored, yellow or yellowish-brown. Radiographically, the affected teeth show a ghostlike appearance. This paper reports the case of a 5-year-old girl presenting this rare anomaly on the left side of the maxillary arch, which crossed the midline. The primary maxillary left teeth (except for the canine) and the primary maxillary right central incisor were missing due to previous extractions. The permanent teeth had a ghostlike appearance radiographically. The treatment performed was rehabilitation with temporary partial acrylic denture and periodic controls. In the future, the extraction of affected permanent teeth and rehabilitation with dental implants will be evaluated. The presentation of this case adds valuable information to pediatric dentists to review special clinical and radiographic features of RO, which will facilitate the diagnosis and treatment of patients with this condition.