Ectopia cordis: prenatal diagnosis, perinatal outcomes, and postnatal follow-up of an international multicenter cohort case series.

OBJECTIVE: This study aimed to analyze prenatal diagnosis, perinatal outcomes, and postnatal follow-up in fetuses with ectopia cordis (EC). METHODS: This retrospective analysis accessed 31 patients with EC who were either diagnosed or referred to a tertiary Fetal Medicine centers for EC diagnosis in Brazil, Germany, Italy, and Poland. We analyzed prenatal diagnosis, perinatal outcomes, and follow-up in these patients. RESULTS: Our study included a cohort of 31 fetuses with EC, 4 and 27 of whom had partial and complete protrusion of the heart through a ventral defect in the thoracoabdominal wall, respectively. EC was diagnosed by fetal echocardiography at a mean gestational age of 20.3 ± 8.6 weeks (range, 8-35 weeks). Of the four cases, in which the karyotype was performed, all of them had a normal result (1 - 46,XX and 3 - 46,XY). Five patients showed conotruncal abnormalities and six ventricular septal defects. Termination of pregnancy (TOP) was performed in 15 cases (48%) and seven pregnant women had spontaneous fetal demise (22.5%). Of the seven fetuses that were born alive, four of them died, and three infants underwent surgery. Among these three infants, all of them survived, one was 5 months, 13 years old and 29 years old at the time of study completion. CONCLUSIONS: Ectopia cordis is associated with high mortality rates and intracardiac/extra-cardiac defects. Ventricular septal defects and conotruncal anomalies were the more common intracardiac defects associated with EC. However, in this cohort of fetuses with EC the incidence of PC was lower than reported in the literature.

Clinical and Electrodiagnostic Findings in Patients with Peripheral Neuropathy and Inflammatory Bowel Disease.

BACKGROUND: Several neurological diseases, especially different types of peripheral neuropathy (PN) are common in inflammatory bowel disease (IBD). METHODS: We prospectively evaluated the presence of PN in 121 patients with IBD (51 with Crohn's disease [CD] and 70 with ulcerative colitis [UC]) and 50 controls (gastritis and dyspepsia) over 3.5 years. RESULTS: A total of 15 patients (12.4%) with small-fiber neuropathy and IBD (7 CD and 8 UC) and 24 patients (19.8%) with large-fiber PN (12 CD and 12 UC) were diagnosed. Small-fiber neuropathy affected 6% and large-fiber PN affected 4% of the control patients. Patients with CD with PN were older, had more metabolic complications and more severe motor involvement than patients with UC with PN. Carpal tunnel syndrome was more common in patients with UC. Sural and median sensory nerves were the most commonly and severely affected sensory responses. Tibial, peroneal, median, and ulnar compound muscle action potential amplitudes were also significantly decreased in patients with CD and UC. In general, sensory and motor amplitudes were a more sensitive marker for PN in patients with IBD than conduction velocities. CONCLUSIONS: In summary, PN is common in patients with IBD. It may be primarily related to IBD, phenotypically modified by metabolic complications. Its phenotype is diverse (most commonly small to predominantly axonal sensory large-fiber), but usually more severe in CD. It also includes ataxic and demyelinating forms. Results from our 10-year follow-up will elucidate the PN clinical course and the real impact of the comorbidities and new therapies.

Pioderma gangrenoso / Pyoderma gangrenosum

É descrito caso de paciente de sete meses de idade com Pioderma gangrenoso em coxa direita. Não há associação com doença sistêmica. O tratamento foi feito com corticóide oral e sulfadiazina de prata. Obteve-se remissão da lesão.

The case is described of a 7 months old patient that presented Pyoderma gangrenosum in the right thigh. There isn't association with systemic desease. It was treated with oral corticoid and silver sulfadiazine.

Tinea capitis em João Pessoa: visão socioeconômica / Tinea capitis in João Pessoa: a social and economic view

FUNDAMENTOS: A distribuição das espécies de dermatófitos varia ao longo do tempo e de acordo com a região, refletindo as condições socioeconômicas da populaçãoOBJETIVOS: Estudar a Tinea Capitis quanto ao agente etiológico, ao sexo, à idade e ao grupo étnicodos doentes em João Pessoa, PB, Brasil e salientar a importância socioeconômica desses dados, com-parando-os aos de regiões mais ricas do país.MÉTODOS: Foram avaliados o perfil e os exames micológicos direto e cultura de 82 pacientes com suspeita clínica de T. capitis em João Pessoa.RESULTADOS: A freqüência de T. capitis incluiu 64,6 por cento das suspeitas clínicas. O dermatófito isolado com maior freqüência foi o T. rubrum (37,7 por cento), seguido por T. tonsurans (28,3 por cento), M. canis (24,5 por cento), T. verru c o s u m (7,5 por cento) e T. mentagrophytes (1,9 por cento). Não houve predileção quanto ao sexo. A faixa etária mais acometida foi a de 0 a 10 anos, e 71,7 por cento dos doentes são caucasóides.CONCLUSÃO: Comparando os resultados obtidos com publicações anteriores da Região Sudeste, os autores salientam as diferenças das variantes socioeconômicas na epidemiologia da doença.

Estudo retrospectivo de 145 casos de Tinea capitis na população de João Pessoa - Paraíba / Retrospective study in 145 cases by Tinea capitis from the population in João Pessoa - Paraíba

Foram analisados 145 casos de pacientes atendidos no Hospital Universitário Lauro Wanderley, da Universidade Federal da Paraíba, em João Pessoa - PB, Brasil, com diagnóstico de Tinea capitis conforme idade, sexo, número de casos/ano e doenças associadas. Houve maior incidência no sexo feminino e na faixa escolar. A média de casos/ano foi de 14,5. Verificou-se associação de Tinea capitis com dermatite seborréica, Tinea corporis e pediculose.

We studied 145 patients with Tinea capitis from Lauro Wanderley's University Hospital of Paraíba Federal University, João Pessoa-PB, Brasil, considering age, sex, number of cases/year and associated diseases...