Guidance to develop a multidisciplinary, international, pediatric registry: a systematic review.

AIM: The European Reference Network for craniofacial anomalies and ear, nose and throat disorders (ERN-CRANIO) aims to improve craniofacial care on a European scale. Within ERN-CRANIO, the cleft lip and palate (CL/P) work stream seeks to ameliorate health outcomes for patients with CL/P. This work stream acknowledged the need for a European wide registry for comparable outcome measures and therapy endpoints to achieve this goal. This review aimed to provide a scientific basis for the conceptualization of this registry by studying previous registry initiatives. METHODS: This review performed thematic analysis on twenty-four articles through narrative synthesis. An iterative process was used to identify key-themes required for prolonged registry success. RESULTS: Analysis of the literature resulted in twenty-one distinct headings including quantitative and qualitative data. Quantitative data including registry characteristics were visualized in a table. The analysis of qualitative data resulted in the identification of fourteen key-themes, which have been summarized and visualized in a guidance. CONCLUSION: This review has successfully identified key-themes required for the development of an international, multidisciplinary, pediatric registry for pan-European cleft care. The guidance provided by this review applies to the goals of ERN-CRANIO, but can be used by any initiative developing a registry.

Extender Supplementation with Antioxidants Selected after the Evaluation of Sperm Susceptibility to Oxidative Challenges in Goats.

This study aimed to detect the most deleterious ROS for goat sperm and then supplemented the extender with a proper antioxidant. For this, 12 adult goats (aged 1-7) were used. Fresh samples were submitted to challenge with different ROS (superoxide anion, hydrogen peroxide, and hydroxyl radical) and malondialdehyde (MDA-toxic product of lipid peroxidation). After experiment 1, sperms were cryopreserved in extenders supplemented to glutathione peroxidase (Control: 0 UI/mL; GPx1: 1 UI/mL; GPx5: 5 UI/mL, and GPx10: 10 UI/mL) and catalase (Control: 0 UI/mL; CAT60: 60 UI/mL; CAT120: 120 UI/mL, and CAT240: 240 UI/mL). Each sample was evaluated by motility, plasma membrane integrity (eosin/nigrosin), acrosome integrity (fast green/rose bengal), sperm morphology, assay of the sperm chromatin structure, mitochondrial activity (3,3-diaminobenzidine), and measurement of lipid peroxidation (thiobarbituric acid reactive substances [TBARS]). It was possible to observe a mitochondrial dysfunction (DAB-Class IV) and low membrane integrity after hydrogen peroxide action. However, the high rates of TBARS were observed on hydroxyl radical. CAT240 presents the lower percentage of plasma membrane integrity. It was possible to attest that hydrogen peroxide and hydroxyl radical are the more harmful for goat sperm. Antioxidant therapy must be improving perhaps using combination between antioxidants.

Low-dose combination of alendronate and atorvastatin reduces ligature-induced alveolar bone loss in rats.

BACKGROUND AND OBJECTIVE: Atorvastatin (ATV) has bone anabolic properties, and alendronate (ALD) is an important antiresorptive drug. This study aimed to evaluate the effects of the combination of ALD and ATV on ligature-induced alveolar bone loss in rats. MATERIAL AND METHODS: Periodontitis was induced by ligature in 78 Wistar rats. Groups of six rats prophylactically received 0.9% saline (SAL), ALD (0.01 or 0.25 mg/kg subcutaneously) or ATV (0.3 or 27 mg/kg by gavage). Then, groups of six rats received the combination of ALD+ATV (0.25 mg/kg + 27 mg/kg, 0.01 mg/kg + 0.3 mg/kg, 0.25 mg/kg + 0.3 mg/kg or 0.01 mg/kg + 27 mg/kg) prophylactically. An extra group of six rats received therapeutic SAL or a lower-dose combination of ALD+ATV (0.01 mg/kg + 0.3 mg/kg, respectively) therapeutically. Three extra groups of six rats each received SAL or a lower-dose combination of ALD+ATV (0.01 mg/kg + 0.3 mg/kg, respectively) prophylactically or therapeutically for histometric and immunohistochemical analyses. The rats were killed on day 11 after ligature placement, and the maxillae were removed and processed for macroscopic, histomorphometric and TRAP immunohistochemical analyses. Gingival samples were collected to evaluate myeloperoxidase (MPO) activity. Blood samples were collected to measure serum bone-specific alkaline phosphatase (BALP) and transaminase levels and for hematological studies. Rats were weighed daily. RESULTS: All combined therapies prevented alveolar bone loss when compared with SAL or low doses of monotherapy (ALD or ATV) (p < 0.05). The lower-dose combination of ALD+ATV (0.01 mg/kg + 0.3 mg/kg, respectively), administered either prophylactically (39.0%) or therapeutically (53.5%), prevented alveolar bone loss. Decreases in bone and cementum resorption, in leukocyte infiltration and in immunostaining for TRAP and MPO activity corroborated the morphometric findings. The lower-dose combination of ALD+ATV (0.01 mg/kg + 0.3 mg/kg, respectively) prevented BALP reduction (p < 0.05) and did not alter the level of serum transaminases. Moreover, the lower-dose combination of ALD+ATV (0.01 mg/kg + 0.3 mg/kg, respectively) also reduced neutrophilia and lymphomonocytosis and did not cause weight loss when compared with administration of SAL. CONCLUSION: The lower-dose combination of ALD+ATV (0.01 mg/kg + 0.3 mg/kg, respectively) demonstrated a protective effect on alveolar bone loss.

The prevalence and severity of dental pain in 14-15 year old Brazilian schoolchildren.

OBJECTIVE: To assess the prevalence and severity of dental pain and associated factors in Brazilian schoolchildren. METHODS: The study was a cross-sectional survey conducted in Recife, Brazil. The data were collected through questionnaires, personal interviews and clinical dental examinations of a random sample of 14-15 years old schoolchildren. The prevalence of dental pain and its characteristics were recorded using standard measures of pain. Dental caries, dental trauma and dental plaque were assessed using WHO criteria. Multiple logistic and ordinal polytomous regression were used to assess which factors were associated with the dental pain outcomes. RESULTS: 1,052 individuals participated in the study. The prevalence of reported toothache in schoolchildren in the last six months was 33.6% (31.1-36.8, 95% CI). The fully adjusted regression models showed a significant relationship between lower social class, later birth order, failure at school and attendance at the dentist only when in trouble with both the prevalence and severity of dental pain. The major predictor of the prevalence and severity of pain was pattern of dental attendance (p<0.001). CONCLUSIONS: The prevalence of toothache in 14-15 years old schoolchildren was high. The major predictor of the prevalence and severity of pain was the pattern of dental attendance.

Knowledge and clinical expertise regarding oral cancer among Brazilian dentists.

The aim of the present work was to assess the knowledge and attitudes of Brazilian General Dental Practitioners with regard to aspects of the aetiology, early diagnosis and prevention of oral cancer relevant for general dental practice. A convenience sample of 129 dentists in Brazil was randomly selected and asked to complete a questionnaire. Results of bi-variated analysis revealed that a self-perceived good knowledge regarding relevant aspects of oral cancer was statistically significantly associated with male gender (Chi square=4.59, P<0.05). Simple logistic regression revealed that male professionals were 2.9 times more knowledgeable than their female colleagues (OR=2.9, 1.1-8.1 95% CI, P=0.037), although, this association was not statistically significant when adjusted for age using multiple regression analysis (OR=2.7, 0.9-7.5 95% CI, P=0.059). There were no significant differences in relevant knowledge and attitudes of private compared with public dentists. Importantly, General Dental Practitioners who spent more than half of time of a patient's initial consultation undertaking a clinical examination were the more likely to detect oral cancer (Chi square=4.245, P=0.039) than those who set aside little time examining their patients. It is concluded that the knowledge of dentists in Brazil regarding relevant aspects of oral cancer is suboptimal, and may not be associated with any examined social-demographic variables. Further research is required to clarify the role of continuing professional education in improving the knowledge and attitudes of dentists relevant to oral cancer in Brazil.

[Sexual disorders in epilepsy. Results of a multidisciplinary evaluation]. / Alterações sexuais na epilepsia. Resultados de uma avaliação multidisciplinar.

Eleven epileptic men who complained of epilepsy and sexual dysfunction were submitted to a multidisciplinary evaluation. Mean age was 27 years (20-34), mean epilepsy duration was 19 years (0.5-32) and the mean seizure frequency was two by week (0-7). Ten patients had partial seizures and one other had myoclonic epilepsy. Ten patients were treated with antiepileptic drugs (phenytoin--1, carbamazepine--8, clonazepam--3, clobazam--2, valproic acid--3, vigabatrin--1). As defined in the DSM III-R, the complaints were: erectile disorder (9), hypoactive sexual desire disorder (4), frotteurism (4), inhibited orgasm (3), premature ejaculation (3), fetishism (2), voyeurism (2), exhibitionism (2), pedophilia (1) and sexual aversion disorder (1). Two patients showed hypogonadotropic hypogonadism on endocrinologic screening. Urological evaluation disclosed organic erectile dysfunction in other two. One patient had a diagnosis of psychogenic sexual disorder. In six patients a conclusive etiologic diagnosis was not reached. This report shows the multifactorial nature of sexual disorder in epilepsy and underlies the need of a multidisciplinar evaluation.

[Mortality descriptors in HIV inpatients]. / Infecção pelo HIV: descritores de mortalidade em pacientes hospitalizados.

OBJECTIVE: To assess the clinical-epidemiological descriptors of inpatient mortality in persons infected with the human immunodeficiency virus (HIV). METHOD: All adult HIV/AIDS patients hospitalized at a university hospital in 1990, 1992 and 1994 were studied retrospectively. Descriptive statistics for all variables of interest were generated. Chi-square test and Fisher's exact test were performed to compare categorical variables. Means were compared using the Student t test. Stepwise logistic regression was used to identify the odds of dying associated with each risk factor. RESULTS: Two hundred and forty patients were included in the study. Between 1990 and 1994 the mean age rose from 35 to 36.9 years, the male/female ratio decreased from 9.8 to 2.0, the non-white proportion increased from 18.5 to 41.3% and the mean time between detection of HIV infection and hospitalization rose from 0.7 to 2.5 years. In addition, length of stay dropped from 31.3 to 25.3 days and the proportion of patients being followed up in the outpatient clinic of the University Hospital increased from 47.8 to 83.3%. Respiratory infections were the main cause of hospitalization (58.0%). Oral candidiasis (27.1%), tuberculosis (18.3%). Pneumocystis carinii pneumonia (15.4%) and toxoplasmic encephalitis (10.4%) were the most frequent opportunistic infections. Multivariate analysis showed that the factors associated with a worse outcome included the length of stay less than or equal to 7 days (Odds Ratio [OR] = 3.88; p = 0.02) and no outpatient follow-up at the Hospital (OR = 3.29; p = 0.01). CONCLUSION: Identification of independent risk factors for death may help in the implementation of more efficient interventions directed towards inpatients with HIV/AIDS.

Analysis of Fusarium causing dermal toxicosis in marram grass planters.

In the European coastal dunes, marram grass (Ammophila arenaria) is planted in order to control sand erosion. In the years 1986 to 1991, workers on the Wadden islands in the Netherlands planting marram grass showed lesions of skin and mucous membranes, suggesting a toxic reaction. Fusarium culmorum dominated the mycoflora of those marram grass culms that were used for planting. This plant material had been cut and stored for more than one week in the open. The Fusarium toxin deoxynivalenol (DON) was detected in the suspect marram grass culms. Isolated F. culmorum strains were able to produce DON in vitro in liquid culture as well as in experimentally inoculated wheat heads. Pathogenicity tests, toxin test as well as RAPD analysis showed that the F. culmorum strains were not specialized for marram grass but may form part of the West-European F. culmorum population infecting cereals and grasses. Storage on old sand-dunes with plant debris may have led to the high occurrence of F. culmorum and contamination with DON. Marram grass culms should be obtained from young plantings on dunes on the seaward slopes and cut culms should not be stored.

Objective criteria in the long-term evaluation of penile venous surgery.

Caverno-venous leakage is a frequent cause of impotence and, since venous network resection gives poor results, a study was performed to evaluate the prognostic factors of this surgical procedure. A total of 23 patients underwent surgery for caverno-venous leakage. With a minimum followup of 1 year, 12 patients had normal erections for sexual intercourse. No significant difference was observed in patient age or maintenance flow rates between the responders and nonresponders. Objective quantification of the percentage of smooth muscle cells using computerized image analysis after immunohistochemical staining (desmin antidesmin) for smooth muscle cells on cavernous biopsies performed during the operation allowed us to differentiate significantly between the 2 groups. All responders had greater than 29% smooth muscle cells. Biopsy of the cavernous body with quantification of the different intracavernous structures seems to be a prognostic factor in selecting impotent patients for vascular reconstruction.

Penile extensibility: to what is it related?

Penile extensibility is the difference between the length of the flaccid penis and the penis submitted to a maximal constant traction. This measurement has been proposed for use as a new potential diagnostic method to assess intracavernous fibrosis. To determine the value of this method we measured penile extensibility before and after removing the skin and cavernous tissue in 17 cadavers and 4 patients undergoing penile implantation. In all cases a cavernous tissue biopsy was performed and the percentages of the different structures were objectively quantified by computer analysis. The extensibility decreased with patient age but it was not influenced by removal of the skin and/or cavernous tissue. No correlation was observed between decreased extensibility and the increase in fibrotic elements in the penile tissue. Penile extensibility seems to reflect the elasticity of the tunica albuginea, which is the limiting factor and cannot be an expression of fibrosis of the corpora cavernosa.

Vascular impotence: focal or diffuse penile disease.

Using computerized image analysis the percentage of smooth muscle fibers was measured in several biopsies of the penis from 10 cadavers and 5 patients with vascular impotence. No significant difference was observed between the proximal and distal areas, and/or between the peripheral and central areas in 1 corpus cavernosum, and between the 2 corpora cavernosa for each patient or cadaver. The percentage of smooth muscle fibers was less in impotent patients in comparison with the cadavers, in which the erectile status was not known, and this reduction occurred throughout both cavernous bodies. Vascular impotence is a diffuse penile disease. Therefore, a cavernous body biopsy can be used to study the penile structure in the assessment of vascular impotence.

Computerized analysis of smooth muscle fibers in potent and impotent patients.

Reduction or dysfunction of the intracavernous smooth muscle fibers can provoke impotence. Computerized digital image analysis of corpus cavernosum biopsies was performed in potent and impotent patients to quantify the percentage of smooth muscle fibers. In 5 normal patients the smooth muscle area represented 40 to 52% of the specimen, in 20 patients with cavernous dysfunction it was 10 to 36% and in 10 patients with arterial disease it was 13 to 25%. This method appears to be important to understand better certain mechanisms of impotence and to approach the potential treatment.

[Stenosis of the renal artery as a cause of acute renal insufficiency after transplantation]. / La sténose de l'artère rénale comme cause d'insuffisance rénale aiguë aprés une transplantation.

Eight cases of kidney transplant recipients that developed acute renal failure are described. Arterial renal stenosis was responsabilised for the ARF. One patient was submitted to transluminar angioplasty without success and then operated. This technique was the successful treatment in another patient. Four patient were submitted to surgical correction as first therapeutic approach. Two patients received no specific treatment. From the six treated patients five had good evolution.

Tumor de testiculo em criancas: relato de tres casos. / Testicular tumor in children: report of 3 cases

Os tumores de testiculo em crianca sao uma patologia rara. Os autores apresentam 3 casos, todos tratados cirurgicamente, atraves de orquiectomia radical por via inguinal. Nao foi utilizada a radioterapia, nem a quimioterapia associada. Apos 3 anos de acompanhamento, dos 3 casos, nao se evidenciaram metastases, ou qualquer sinal de disseminacao

Congenital cyst of the seminal vesicle.

A case of congenital cyst of the seminal vesicle in association with an ectopic ureter is presented. The clinical, diagnostic and therapeutic aspects are discussed with special emphasis on the role played by ultrasound and computerized tomography in the evaluation of pelvic pain and painful ejaculation.