Naso-orbicular tissue necrosis by Streptococcus parasanguis in a patient with Fanconi anemia: Clinical and laboratory aspects

Fanconi anemia (FA) is a rare autosomal recessive disorder, characterized by pancytopenia and progressive hypoplasia of the bone marrow. A 23-year-old woman with FA showed severe pancytopenia and developed an abscess on the infraorbicular region on the right side of the face that progressed to phlegmon and caused tissue necrosis of the nostrils, nasal septum, nasal fossa, and posterior orbital region. Laboratory examination showed Streptococcus parasanguis as the etiologic agent of the phlegmon. Supportive treatment was recommended due to donor incompatibility for bone marrow transplant. The intraoral examination showed spontaneous gingival bleeding, edema of the interdental papillae, hematomas on the superior and inferior lips, bacterial and fungal infections, and adequate oral hygiene. The patient was treated with the administration of an antibiotic (imipenem), an antifungal (amphotericin B), and mouth washing with antiseptic solutions. Periodontal prophylaxis and orientation to and control of oral hygiene and diet were also used during the remission period. For functional and esthetic rehabilitation of the alar regions and nasal dorsum, an acrylic resin nasal prosthesis was made, supported by a spectacle frame.

Interdisciplinary approach to treat dyskeratosis congenita associated with severe aplastic anemia: A case report

This paper reports on a 4-year-old male who had dyskeratosis congenita and who acquired severe aplastic anemia. The patient developed hyperpigmentation of the face, neck and chest region, arms, shoulders and legs. In addition, he had dry skin, deformed fingernails and toenails, sparse hair and eyebrows and hyperkeratosis of the dorsum of the hands and feet. Laboratory and histological analysis revealed severe pancytopenia and dyserythropoiesis of red blood cells, hypocellularity of white blood cells and decreased megakaryocytes with dysplasia. The intraoral examination identified bleeding gums; petechiae of the palate, tongue and cheek mucosa; and an atrophic, smooth and shining dorsal surface of the tongue. There were deep carious lesions in the deciduous mandibular molars and maxillary anterior teeth; as well as mobility of mandibular left canine, which had bone loss. The treatment for oral lesions included diet changes, improved oral hygiene, and extraction of the deciduous teeth destroyed by caries.

Incidência de malformações congênitas em crianças concebidas através de injeção intracitoplasmática de espermatozóides

Purpose: to evaluate the incidence and types of major congenital malformations (MCM) in liveborn children conceived by intracytoplasmic sperm injection (ICSI). Methods: a total of 680 liveborn children resulted from 511 couples submitted to ICSI from January, 1999 to December, 2002. Data collection of the children was performed through standardized questionnaire and clinical examination. Of the 511 couples, 366 had been contacted for a sampling of 371 gestations. Of the 680 liveborn, 520 had been evaluated, 250 of them (48.1%) through questionnaire and 270 (51.9%) through questionnaire and physical examination. Two hundred and fifty children were from singleton pregnancies and 270 from multiple pregnancies. Malformations were classified according to the 10th revision of the International Statistical Classification of Diseases and Related Health. Only MCM were analyzed in this study. The incidence of MCM was compared with that of the general population obtained by the Latin American Collaborative Study of Congenital Malformations. The statistical analysis was performed by the χ 2 test (level of significance p<0.05). Results: of the 520 children, 15 presented MCM, resulting in an incidence of 2.9%. There was no difference in relation to the control group (p>0.05), which showed 2.6% incidence of MCM. The most frequent malformations were of cardiac origin (four isolated and two associated), corresponding to 40% of the total. The other types of MCM were: renal (three), neural tube (two), skull (one), cleft lip (one), genital (one), Down syndrome (associated with cardiac malformations) (two), and musculoskeletal (one). Six MCM occurred in children from singleton pregnancies and nine in children from multiple pregnancies. Conclusion: the liveborn children conceived by ICSI presented incidence of major congenital malformations (2.9%) near to the expected for the general population (2.6%). However, to establish the risks of MCM with precision it is necessary to continue the evaluation of the children conceived by ICSI.

Etiologia e incidência das fraturas faciais em adultos e crianças: Experiência em 513 casos

Facial trauma has presented an increasing occurrence in the last four decades, due especially to the growth of accidents with automobiles as well as to the urban violence. Both of which continue being the main cause of such traumas. Aim: To evaluate the features of the population victim of facial trauma as to gender, age, occupation, origin, type of fracture and its cause. Design study: retrospective clinical with transversal cohort. Material and Method: Retrospective study consulting hospital registers of 513 patients victms of the facial trauma. Results: There was a higher incidence of facial trauma on men (84,9%), white (82,7) and with an average age of 29. Regarding occupation, the trauma was mostly occurred to students (16,6%) and Masons (11,2%). The jaw was the most affected place (35%), followed by zygoma (24%) and by the nose (23%), though most patients presented a single facial fracture (82,5%). Among the causes, accidents with automobiles (28,3%), aggressions (21%) and accidental fall s (19,5%) were the most common. Conclusions: Accidents with automobiles continue being the main cause of facial trauma, especially of multiple factures due to the great transmission of kinetic energy.

Componente lento do V̇O2 em crianças durante exercício pesado de corrida: Análise com base em diferentes modelos matemáticos

The purpose of this study was to identify and quantify the magnitude of the slow component of VO2 (SC) in children during running exercise, performed at heavy intensity domain (75%Δ), using two different mathematical models: a) three-exponential model and; b) ΔVO2 6-3 min. Eight healthy male children (11.92 ± 0.63 years; 44.06 ± 13.01 kg; 146.63 ± 7.25 cm; and sexual maturity levels 1 and 2), not trained, performed in different days the following tests: 1) incremental running treadmill test to determine the peak oxygen uptake (VO2peak) and the lactate threshold (LT); and 2) two transitions from baseline to 75%Δ [75%Δ = LT + 0.75 x (VO2 peak - LT)] for six minutes on treadmill. The SC was determined by two models: a) three-exponential model (Exp3); and b) the VO2 difference between the sixth and the third exercise minute (ΔVO2 6-3min). The SC was expressed as the absolute (ml/min) and percent contribution (%) to the total change in VO 2. The SC values determined by model Exp3 (129.69 ± 75.71 ml/min and 8.4 ± 2.92%) and ΔVO2 6-3 min (68.69 ± 102.54 ml/min and 3.6 ± 7.34%) were significantly different. So, the SC values in children during running exercise performed at heavy intensity domain (75%Δ) are dependent of the analysis model (Exp3 x ΔVO2 6-3 min).

Incidência e evolução da polipose nasal em crianças e adolescentes com fibrose cística

Nasal polyps are a clinical sign of alert for investigating Cystic Fibrosis (CF). Aims: To study the incidence of nasal polyps in children and adolescents with cystic fibrosis, its possible association with age, gender, clinical manifestations, genotype and sweat chlorine level, and its evolution with topical steroid therapy. Methods: Clinical symptoms, sweat chlorine level and genotype were studied in 23 cystic fibrosis patients. Nasal polyps were diagnosed by nasal endoscopy and treated with topical steroids during 6 months, followed by a second nasal endoscopy. Fisher test was used for statistical analysis. Results: Nasal polyps were found in 39.1% of the patients (five bilateral, four unilateral), all older than six years, recurrent pneumonia in 82.6%, pancreatic insufficiency in 87% and malnutrition in 74%. No association was seen between nasal polyps and sweat chlorine level, genotype, clinical sings of severity and nasal symptoms. Seven patients improved in their nasal polyps with topical steroids, six showed complete resolution. Conclusion: The study showed a high incidence of nasal polyps in older children, who span the entire range of clinical severity, even in the absence of clinical nasal symptoms. Topical steroid therapy showed good results. An interaction among pediatricians and otolaryngologists is necessary for diagnosis and follow-up. 2008 © Revista Brasileira de Otorrinolaringologia. All Rights reserved.

Abnormal hemoglobin phenotypes in carriers of mild anemia in Latin America.

We looked for abnormal hemoglobins in blood samples sent for diagnosis of anemia. Identification of the hemoglobins was made using electrophoretic, chromatographic and molecular procedures. The 2020 blood samples were of patients from various regions of Brazil and from some other Latin American countries. Among the abnormal hemoglobins that we found, 3.5% are known to be rare, while 51% had an electrophoretic profile similar to that of Hb S at alkaline pH. Differentiation was possible only by combining electrophoretic and chromatographic methods. Hb Hasharon, an alpha globin chain mutant, was the most frequently found variant hemoglobin; it accounted for 14.3% of the abnormal DNA samples. The other abnormal hemoglobin phenotypes displayed distinct electrophoretic profiles; most of them migrated faster than Hb A. The frequencies of the different abnormal hemoglobin profiles that we found reflect the miscegenation of the Latin American population and indicate the importance of hemoglobin studies using various methods in combination for accurate diagnosis and appropriate counseling of carriers and their families.

Edema agudo de pulmão de pressão negativa como complicação grave de síndrome de apneia obstrutiva do sono em crianças. Relatos de casos

Introduction: Obstructive sleep apnea syndrome is related to cardiopulmonary complications in children. It is important to know its patophysiology and possible complications to help reduce risks in this group. Aims: To report three cases of severe cardiorespiratory complications of obstructive sleep apnea managed in the intensive care unit (ICU). Case report: Two children with no previous diagnosis of obstructive sleep apnea syndrome suffered acute congestive heart failure and acute lung oedema with need of ICU and improved after adenotonsillectomy. In a third case, the patient had acute lung oedema as a complication after adenotonsillectomy. Conclusions: Paediatricians and otolaryngologists must be aware of the clinical manifestations of severe sleep apnea. Early referring to treatment and special attention at pre and post surgical periods are essentials to avoid serious complications.

Caracterização de programas de intervenção com crianças e/ou adolescentes

The objective was to present a descriptive analysis of interventions to prevent and/or reduce children's and/or adolescents' problem behaviors. The study reviewed 192 abstracts from journal articles published between the 1986 and June 2006, available on the following databases: Index-Psi/Periódicos, Lilacs, PsycInfo, Scielo and Medline. Categories of analysis focused on bibliography, method, and studies' results. Data-analysis showed that most of the studies focused on groups and families, and samples with more than 30 participants. Study designs were experimental and quasi-experimental, combining observation and self report or just self report for data collection. Result analysis suggests that the intervention programs contributed specially for reducing behavior problems, and improving parental practices. Discussion evaluates contributions of the study and gaps in knowledge.

Conflitos conjugais e seus efeitos sobre o comportamento de crianças: Uma revisão teórica

Marital conflicts have been largely investigated in research, especially because of its impact on child development. Based on a literature review, this article aims to present some reflections about marital conflicts and its possible associations with children's behavior, particularly through parental practices. Emphasis is made on a systemic view of causality between marital conflict and child behavior, which offers a better comprehension of the family functioning. This perspective also allows us to enlarge the analysis scope beyond linear correlations between both variables.

Eletromiografia dos músculos temporal e masseter em crianças com mordida cruzada posterior direita

This study proposal was to evaluate the changes at a masticatory muscle temporalis and masseter, through a eletromyographic, at ponters of the right unilateral bite posterior, verifying the difference at the eletric activity between right and left sides. Twenty female volunteers, with ages between 7 and 8 (x = 7.4). The eletromyographics signals were collected in both sides in all volunteers in rest situations and in an usual isotonic mastication, right, left and bilateral and the maximun isometric constriction, which sinal was used to the eletric activity amplitude normalization. The results signed that the right masseter muscle presents an expressive statistic difference, if compared with the left masseter muscle during the left and right mastication, suggesting a right unilateral mastication. The muscle haven't gotten any expressive variance in this amplitude which remained to a proximate flat noticed in a right and habitual mastication, suggesting a right and one sided mastication during the left mastication occurs a raise of the eletric signal amplitude of the muscle left masseter. The right masseter muscle haven't gained any expressive variance, which remained in a proximate flat observed in an usual and right mastication, suggesting a muscular pattern hyperfunction next to the dental crossing.

Avaliação de grupo de intervenção para as mães/cuidadores de crianças pré-escolares com problemas de comportamento externalizantes

Negative parental practices may influence the onset and maintenance of externalizing behavior problems, and positive parenting seem to improve children's social skills and reduce behavior problems. The objective of the present study was to describe the effects of an intervention designed to foster parents' social skills related to upbringing practices in order to reduce externalizing problems in children aged 4 to 6 years. Thirteen mothers and two care taker grandmothers took part in the study with an average of four participants per group. To assess intervention effects, we used a repeated measure design with control, pre, and post intervention assessments. Instruments used were: (a) An interview schedule that evaluates the social interactions between parents and children functionally, considering each pair of child's and parent's behaviors as context for one another; (b) A Social Skills Inventory; (c) Child Behavior Checklist - CBCL. Intervention was effective in improving parent general social skills, decreasing negative parental practices and decreasing child behavior problems.

Úlcera de cameron como causa incomum de anemia por deficiência de ferro

Cameron ulcers are chronic linear lesions willing on the mucosal folds, the level of diaphragmatic impression, in patients with hiatal hernia. Its clinical relevance is due to the potential risk of gastrointestinal complications, such as acute or chronic bleeding and anemia. Usually, the diagnosis is incidental by upper gastrointestinal endoscopy. Proton pump inhibitors are essential for the conduct of cases and the administration of iron when the anemia is associated. Already, the benefit of surgery is in refractory cases. We present a case of this entity of a 50-year-old patient in postmenopausal who developed anemia due to iron deficiency and normal initial endoscopy.

Assistência Odontológica em Crianças com Paralisia Cerebral e Perspectivas Tecnológicas de Reabilitação

The improvement of oral health and quality of life is closely related to transdisciplinary dialogue, technological development and social responsibility. In patients with cerebral palsy, the lesion of motor areas of the brain compromises the development and function of the craniofacial complex. Considering all the ethiopathogenic conditions, the treatment of such patients involves great difficulties. The dentist and other professionals related to their rehabilitation need to deal with difficulty in chewing, respiration, phonation, besides the poor oral hygiene resulted from abnormal involuntary movements of facial and masticatory musculature, tongue, and upper limb. It is also relevant the lack of understanding about the importance of oral health care due to mental deficits of these individuals. This study aims to review some aspects of oral health in patients with cerebral palsy proposing rehabilitation associated to technology. Few studies concerned about the effectiveness of therapies for oral rehabilitation in patients with cerebral palsy. Laser therapy, electromyography, electrostimulation and LED therapy should be analyzed as options for treatment of patients with cerebral palsy. Following research projects should focus more attention on the dynamic and oral function of these patients to achieve positive repercussions in their overall health. © ArquiMed, 2010.

Planejamento, síntese e avaliação farmacológica de compostos híbridos potencialmente ativos para o tratamento da anemia falciforme

Sickle cell disease (SCD) is a hereditary hemolytic anemia caused by the inheritance of one S hemoglobin gene from each ancestor. Patients with SCD present increased circulating levels of cytokines, including TNF-alpha (TNF-α). Hydroxyurea (HU) is the available therapeutically strategy for treatment; it acts as a source of nitric oxide and benefits patients by increasing the levels of fetal hemoglobin (HbF). Thus, within one research line that aims at finding new drugs, a series of compounds with TNF-α inhibition and nitric oxide donation properties have been synthesized in order to explore possible synergism of actions beneficial in the treatment of the disease. Six compounds were synthesized: five derivatives of organic nitrates and one of sulfonamide. The compounds, (1,3-dioxo-1,3-dihydro-2Hisoindol-2-yl) methyl nitrate (compound I); (1,3-dioxo-1,3-dihydro-2H-isoindol-2-yl) ethyl nitrate (compound II); 3-(1,3-dioxo-1,3-dihydro-2H-isoindol-2-yl) benzyl nitrate (compound III);4-(1,3-dioxo-1,3-dihydro-2H-isoindol-2-yl)-N-hydroxybenzenesulfonamide (compound IV); 4-(1,3-dioxo-1,3-dihydro-2H-isoindol-2-yl) benzyl nitrate (compound V) and 2-[4-(1,3-dioxo-1,3-dihydro-2H-isoindol-2-yl) phenyl]ethyl nitrate (compound VI), were synthesized using linear synthetic methodology, with excellent overall yields. All compounds showed anti-inflammatory and analgesic effects with a reduction in 43%-65% of ear edema in mice and a reduction of 25%-42% of writhing induced by acetic acid. All compounds showed comparable reductions in the leukocyte infiltration capacity and ability to generate nitric oxide. The aryl compounds (III, IV and V) presented less mutagenic activity compared to compounds I, II and VI according to the salmonella mutagenicity assay (Ames test). Compounds IV and VI showed activity in K562 culture cells, with increases in gamma globin gene expression to levels higher than with hydroxyurea suggesting a potential to increase fetal hemoglobin. This data set characterizes new potentially useful drug candidates for the treatment of symptoms of sickle cell anemia.