Objective To describe the clinical, radiological, and electromyographic features in a series of children with joint contractures (arthrogryposis) associated with congenital infection presumably caused by Zika virus.Design Retrospective case series study.Setting Association for Assistance of Disabled Children, Pernambuco state, Brazil.Participants Seven children with arthrogryposis and a diagnosis of congenital infection presumably caused by Zika virus during the Brazilian microcephaly epidemic.Main outcome measures Main clinical, radiological, and electromyographic findings, and likely correlation between clinical and primary neurological abnormalities.Results The brain images of all seven children were characteristic of congenital infection and arthrogryposis. Two children tested positive for IgM to Zika virus in the cerebrospinal fluid. Arthrogryposis was present in the arms and legs of six children (86%) and the legs of one child (14%). Hip radiographs showed bilateral dislocation in seven children, subluxation of the knee associated with genu valgus in three children (43%), which was bilateral in two (29%). All the children underwent high definition ultrasonography of the joints, and there was no evidence of abnormalities. Moderate signs of remodeling of the motor units and a reduced recruitment pattern were found on needle electromyography (monopolar). Five of the children underwent brain computed tomography (CT) and magnetic resonance imaging (MRI) and the remaining two CT only. All presented malformations of cortical development, calcifications predominantly in the cortex and subcortical white matter (especially in the junction between the cortex and white matter), reduction in brain volume, ventriculomegaly, and hypoplasia of the brainstem and cerebellum. MRI of the spine in four children showed apparent thinning of the cord and reduced ventral roots.Conclusions Congenital Zika syndrome should be added to the differential diagnosis of congenital infections and arthrogryposis. The arthrogryposis was unrelated to the abnormalities of the joints themselves, but was possibly of neurogenic origin, with chronic involvement of central and peripheral motor neurones leading to deformities as a result of fixed postures in utero. Based on the neurophysiological observations, we suggest two possible mechanisms: tropism of neurones, with involvement of peripheral and central motor neurones, or a relation with vascular disorders.

Morbid Obesity Morbid obesity is a disabling condition that has a serious negative impact on lung function, respiratory muscle function and quality of life. Inspiratory Muscle Training (IMT) is a technique which aims to improve pulmonary expansion and to prevent post surgery complications on morbid obese individuals. This study shows significantly increased on maximal inspiratory pressure, maximal voluntary ventilation and promoted changes on spirometric variables after IMT.

Introdução: a peritonite meconial, como resultado da perfuração intestinal fetal, apresenta baixa incidência (1:30.000 nascimentos) e elevada mortalidade (em torno de 50%). Os achados ecográficos pré-natais incluem ascite e calcificações intra-abdominais. Há evidências de que o diagnóstico pré-natal possa melhorar o prognóstico pós-natal. Relato do Caso: R.C.M.S., 22 anos, II gesta O para, realizou ultra-sonografia em 02/12/98 com diagnóstico de ascite fetal. Fez investigação para hidropisia fetal, afastando-se causas imunes e não-imunes. Foram realizados ecografias seriadas em que se manteve a imagem de ascite fetal acentuada, sem calcificações. Parto normal em 02/01/99, com 36 semanas, observando-se volumoso poliidrâmnio. Recém-nascido do sexo feminino pesando 2.670 gramas, com sinais de desconforto respiratório, abdome distendido e com petéquias. Apresentou aumento progressivo da distensão abdominal, palpação de massa pétrea no hipocôndrio direito e eliminação de muco branco ao toque retal. Raios-x em 04/01/99 com imagem de extensas calcificações abdominais, distensão de alças intestinais e ausência de gás na ampola retal. Hipótese diagnóstica de peritonite meconial. Indicada laparotomia exploradora em 04/01/99, encontrando-se volumoso cisto meconial e atresia ileal, realizandose lise de aderências e ileostomia em dupla boca. Evolução satisfatória nos primeiros dias de pós-operatório, complicada posteriormente por quadro séptico, verificando-se o óbito neonatal em 09/01/99. Conclusão: a peritonite meconial deve ser lembrada no diagnóstico diferencial das causas de ascite fetal. O diagnóstico pré-natal no presente caso poderia ter antecipado a indicação cirúrgica, com possível melhora da evolução neonatal.

-Background -Presinusoidal portal hypertension with frequent episodes of upper gastrointestinal variceal bleeding are hallmarks of hepatosplenic Manson's schistosomiasis; a clinical form that affects about 5% of Brazilians who are infected by Schistosoma mansoni. Aims -To evaluate duplex sonography findings in patients with hepatosplenic Manson's schistosomiasis with and without upper gastrointestinal variceal hemorrhage. Methods -A cross-sectional study was performed whereby 27 consecutive patients with hepatosplenic Manson's schistosomiasis were divided into two groups: group I (six men and six women; mean age 48.7 years) with a past history of bleeding and group II (four men and eight women; mean age 44.7 years) without a past history of upper gastrointestinal bleeding, underwent duplex sonography examination. All patients underwent the same upper gastrointestinal endoscopy and laboratory examinations. Those with signs of mixed chronic liver disease or portal vein thrombosis (three cases) were excluded. Results -Group I showed significantly higher mean portal vein flow velocity than group II (26.36 cm/s vs 17.15 cm/sec). Although, as a whole it was not significant in all forms of collateral vessels (83% vs 100%), there was a significantly higher frequency of splenorenal collateral circulation type in group II compared with group I (17% vs 67%). The congestion index of the portal vein was significantly lower in group I than in group II (0.057 cm vs 0.073 cm/sec). Conclusion -Our duplex sonography findings in hepatosplenic Manson's schistosomiasis support the idea that schistosomotic portal hypertension is strongly influenced by overflow status, and that collateral circulation seems to play an important role in hemodynamic behavior.

In patients with hepatosplenic schistosomiasis with a previous history of variceal hemorrhage, splenectomy and gastric vein ligation was effective in reducing the main hemorrhagic risk factors until the sixth month of follow-up, indicating a good way to control the bleeding episodes.

-Background:In hepatosplenic schistosomiasis occurs diffuse hepatic fibrosis associated with venous congestion of the portal system resulting in hepatosplenomegaly. It can produce digestive hemorrhage caused by rupture of esophageal and stomach varices or peptic gastroduodenal mucosal lesions. Aim: To study the effects of splenectomy and ligature of the left gastric vein on portohepatic hemodynamics. Method: Twenty-three patients with hepatosplenic schistosomiasis mansoni were studied before and about two weeks after operation through angiographic diameter of the common and proper hepatic artery, splenic artery, superior mesenteric artery, portal vein, superior mesenteric vein and left gastric vein. The pressures of the inferior vena cava and central venous pressure, free hepatic vein, the hepatic sinusoidal and occluded vein were measured. Results: The splenectomy and ligature of the left gastric vein determined low morbidity and null mortality. It determined significant addition to the following variables: diameters of the common and proper hepatic artery; diameter of the superior mesenteric vein. It determined non significant increase on the following measurements: right atrial pressure and diameter of the superior mesenteric artery. It determined non significant decrease to the following variables: inferior vena cava pressure; free hepatic vein pressure; occluded hepatic vein pressure; sinusoidal pressure, diameter of the portal vein. Conclusion: Splenectomy and ligature of the left gastric vein do not determine portal hemodynamic changes capable of breaking the functional hemodinamic balance that characterizes the hepatosplenic mansoni schistosomiasis.RESUMO -Racional: Na esquistossomose mansônica na forma hepatoesplênica ocorre fibrose hepática difusa que associada à congestão venosa do sistema porta resulta em hepatoesplenomegalia. Pode produzir hemorragia digestiva alta por rotura das varizes de esôfago e do estômago ou lesões pépticas da mucosa gastroduodenal. Objetivo: Estudar os efeitos da esplenectomia e ligadura da veia gástrica esquerda sobre a hemodinâmica portohepática. Método: Vinte e três portadores de esquistossomose mansônica na forma hepatoesplênica foram estudados prospectivamente, antes e cerca de duas semanas após a operação, através de estudos angiográficos dos diâmetros da artéria hepática comum e própria, artéria esplênica, artéria mesentérica superior, veia porta, veia mesentérica superior e veia gástrica esquerda. Foram aferidas as pressões da veia cava inferior, venosa central, da veia hepática livre, da veia hepática ocluída e sinusoidal. Resultados: A ligadura da veia gástrica esquerda determinou acréscimo significante nas seguintes variáveis: diâmetros da artéria hepática comum e própria; diâmetro da veia mesentérica superior; o acréscimo não foi significante nas seguintes medidas: pressão venosa central e diâmetro da artéria mesentérica superior. Ela promoveu decréscimo não significante nas variáveis: pressão da veia cava inferior; pressão da veia hepática livre; pressã...

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The aurora sign, a sonographic sign found on the sagittal and transverse view, refers to multiple bands of ring-down artifacts posterior to the right hemidiaphragm. Parenchymal lung disease should be suspected when this is present. We report a case of type B Niemann-Pick disease with pulmonary involvement and the aurora sign on abdominal sonography. High-resolution CT of the chest showed corresponding thickened interlobular septa.