23 resultados para Geniculohypothalamic tract


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PURPOSE: This study was designed to compare baseline data and clinical outcome between patients with prostate enlargement/benign prostatic hyperplasia (PE/BPH) who underwent unilateral and bilateral prostatic arterial embolization (PAE) for the relief of lower urinary tract symptoms (LUTS). METHODS: This single-center, ambispective cohort study compared 122 consecutive patients (mean age 66.7 years) with unilateral versus bilateral PAE from March 2009 to December 2011. Selective PAE was performed with 100- and 200-μm nonspherical polyvinyl alcohol (PVA) particles by a unilateral femoral approach. RESULTS: Bilateral PAE was performed in 103 (84.4 %) patients (group A). The remaining 19 (15.6 %) patients underwent unilateral PAE (group B). Mean follow-up time was 6.7 months in group A and 7.3 months in group B. Mean prostate volume, PSA, International prostate symptom score/quality of life (IPSS/QoL) and post-void residual volume (PVR) reduction, and peak flow rate (Qmax) improvement were 19.4 mL, 1.68 ng/mL, 11.8/2.0 points, 32.9 mL, and 3.9 mL/s in group A and 11.5 mL, 1.98 ng/mL, 8.9/1.4 points, 53.8 mL, and 4.58 mL/s in group B. Poor clinical outcome was observed in 24.3 % of patients from group A and 47.4 % from group B (p = 0.04). CONCLUSIONS: PAE is a safe and effective technique that can induce 48 % improvement in the IPSS score and a prostate volume reduction of 19 %, with good clinical outcome in up to 75 % of treated patients. Bilateral PAE seems to lead to better clinical results; however, up to 50 % of patients after unilateral PAE may have a good clinical outcome.

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Using 49 prostatectomized patients as experimental subjects, we studied the effects of Inclometnacin and acetylsalicylic acid — accredited prostaglandin synthetase inhibitors — from a urodynamic and clinical standpoint. Relevant urodynamic data was gathered 1 hr 30 mi after the patients had taken the drugs and placebo. Clinical results were further scrutinized after 8 days of treatment, at which time a new urodynamic workup was again performed on some patients. Results were again studied shortly after the end of treatment. The effect of the drugs on bladder and urethral structures was borne out by clear-ct!t clinical and urodynamic changes. After statistically analyzing such changes, we concluded that prostaglandin synthesis inhibition resulting in the inhibition of prostaglandin action had, at least in part, led to the changes noted. In the present report we shall discuss the role played by the highly complex mechanisms at work.

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BACKGROUND: Both primary and secondary gynaecological neuroendocrine (NE) tumours are uncommon, and the literature is scarce concerning their imaging features. METHODS: This article reviews the epidemiological, clinical and imaging features with pathological correlation of gynaecological NE tumours. RESULTS: The clinical features of gynaecological NE tumours are non-specific and depend on the organ of origin and on the extension and aggressiveness of the disease. The imaging approach to these tumours is similar to that for other histological types and the Revised International Federation of Gynecology and Obstetrics (FIGO) Staging System also applies to NE tumours. Neuroendocrine tumours were recently divided into two groups: poorly differentiated neuroendocrine carcinomas (NECs) and well-differentiated neuroendocrine tumours (NETs). NECs include small cell carcinoma and large cell neuroendocrine carcinoma, while NETs account for typical and atypical carcinoids. Cervical small cell carcinoma and ovarian carcinoid are the most common gynaecological NE tumours. The former typically behaves aggressively; the latter usually behaves in a benign fashion and tends to be confined to the organ. CONCLUSION: While dealing with ovarian carcinoids, extra-ovarian extension, bilaterality and multinodularity raise the suspicion of metastatic disease. NE tumours of the endometrium and other gynaecological locations are very rare. TEACHING POINTS: • Primary or secondary neurondocrine (NE) tumours of the female genital tract are rare. • Cervical small cell carcinoma and ovarian carcinoids are the most common gynaecological NE tumours. • Cervical small cell carcinomas usually behave aggressively. • Ovarian carcinoids tend to behave in a benign fashion. • The imaging approach to gynaecological NE tumours and other histological types is similar.

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Urofacial syndrome (UFS) is an autosomal recessive congenital disease featuring grimacing and incomplete bladder emptying. Mutations of HPSE2, encoding heparanase 2, a heparanase 1 inhibitor, occur in UFS, but knowledge about the HPSE2 mutation spectrum is limited. Here, seven UFS kindreds with HPSE2 mutations are presented, including one with deleted asparagine 254, suggesting a role for this amino acid, which is conserved in vertebrate orthologs. HPSE2 mutations were absent in 23 non-neurogenic neurogenic bladder probands and, of 439 families with nonsyndromic vesicoureteric reflux, only one carried a putative pathogenic HPSE2 variant. Homozygous Hpse2 mutant mouse bladders contained urine more often than did wild-type organs, phenocopying human UFS. Pelvic ganglia neural cell bodies contained heparanase 1, heparanase 2, and leucine-rich repeats and immunoglobulin-like domains-2 (LRIG2), which is mutated in certain UFS families. In conclusion, heparanase 2 is an autonomic neural protein implicated in bladder emptying, but HPSE2 variants are uncommon in urinary diseases resembling UFS.

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The Ross procedure has been used in children and young adults for aortic valve replacement and the correction of complex obstruction syndromes of the left ventricular outflow tract. We report the mid-term results of the Ross procedure in a single institution and performed by the same surgical team. Population: Between March 1999 and December 2005, 18 patients were operated on using the Ross procedure. The mean age at the time of surgery was 12 years, being 12 patients male (67%). The primary indication for surgery was isolated aortic valve disease, being the predominant abnormality in 58% of cases aortic regurgitation and in 42% left ventricular outflow tract obstruction. Associated lesions included sub-aortic membrane in 3 patients (16%), small VSD in 2 patients (11%), bicuspid aortic valve in 4 patients (22%) and severe left ventricular dysfunction and mitral valve regurgitation in 1 patient (6%). Ten of the 18 patients (56%) had been submitted to previous surgical procedures or percutaneous interventions. Results: Early post-operative mortality was not seen, but two patients (11%), had late deaths, one due to endocarditis, a year after the Ross procedure, and the other due to dilated cardiomiopathy and mitral regurgitation. The shortest time of follow-up is 6 months and the longest 72 months (median 38 months). Of the 16 survivors, 14 patients are in class I of the NYHA and 2 in class II, without significant residual lesions or need for re-intervention. The 12 patients with more than a year of follow up revealed normal coronary perfusion in all patients and no segmental wall motion abnormalities. Nevertheless, two of the 12 patients developed residual dynamic obstruction of LVOT and in three patients aortic regurgitation of a mild to moderate degree was evident. Significant gradients were not verified in the RVOT. Conclusions: The Ross procedure, despite its complexity, can be undertaken with excellent immediate results. Aspects such as the dilation of the neo aortic root and homograft evolution can not be considered in a study of this nature, seeing that the mean follow up time does not exceed 5 years.

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Objectives: Literature review of classification, epidemiology, pathophysiology, microbiology, clinical presentation, diagnosis, complications, treatment and prevention of urinary tract infections (UTI) in pregnancy. Data Sources and Review Methods: Bibliographic research in Medline, through PubMed and Medscape, of systematic reviews, observational studies, clinical guidelines, meta-analyses and randomized controlled trials published between January 1992 and December 2010. Results: Asymptomatic bacteriuria occurs in 2 to 10% of pregnant women, 30% of which will develop acute pyelonephritis if left untreated. Treatment of asymptomatic bacteriuria has been shown to reduce the risk of pyelonephritis in pregnancy. Therefore, screening and treatment of this form of UTI has become a standard of obstetrical care, although the ideal duration of the treatment is still controverse. Acute pyelonephritis is one of the most common medical complications of pregnancy and may be associated with maternal, obstetric and perinatal complications. There is no clear consensus in the literature regarding antibiotic choice or duration of therapy for symptomatic UTI. Increasing antibiotic resistance complicates the choice of empirical regimens and local resistance rates need to be taken into account when deciding the therapy. Considering the high rate of recurrence of UTI during pregnancy, prophylactic measures need to be taken in pregnant women who have already experienced UTI during their current pregnancy. Conclusions: Although UTI is a generally benign condition in non-pregnant women, it may be a potentially serious complication during pregnancy. Early diagnosis and treatment of UTI during pregnancy are mandatory and can prevent severe maternal and perinatal complications.

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Purple urine bag syndrome (PUBS) was first reported in 1978. PUBS is rare, occurs predominantly in constipated women, chronically catheterized and associated with some bacterial urinary infections that produce sulphatase/phosphatase. The etiology is due to indigo (blue) and indirubin (red) or to their mixture that becomes purple. A chain reaction begins in the gastrointestinal tract with tryptophan as described in the article.

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Clinical history - A 4-year-old boy, born prematurely at 29 weeks (twin pregnancy), with periventricular leukomalacia and epilepsy underwent brain MRI. Neurological examination showed severe developmental retardation with axial hypotonia, spastic tetraparesis and convergent strabismus. Imaging findings - Cranial MRI revealed typical aspects of partial rhombencephalosynapsis with vermian hypoplasia, midline fusion of the cerebellar hemispheres and transversely oriented folia and fissures. There was also mild dilatation and dysmorphism of the ventricular system, the septum pellucidum was absent, the hippocampi were malrotated and had vertical orientation and additional finding of associated periventricular cystic leukomalacia. Discussion - Rhombencephalosynapsis (RS) is a rare congenital defect of the cerebellum classically characterised by vermian agenesis or hypogenesis, fusion of the hemispheres, and closely apposed or fused dentate nuclei. It is now considered to result from an absence of division of the cerebellar hemispheres, following an insult between the 28th and 44th day of gestation (i.e., before the formation of the vermis). Other features have also been described such as fusion of the thalami and cerebral peduncles, malrotated hippocampi, corpus callosum agenesis, hypoplastic chiasm, absence of the septum pellucidum, ventriculomegaly, agenesis of the posterior lobe of the pituitary and cortical malformations. Musculoskeletal, cardiovascular, urinary tract, and respiratory abnormalities have been reported. Typical symptoms consist of swallowing difficulties, delayed motor acquisitions, muscular hypotonia, spastic quadriparesis, cerebellar signs including dysarthria, gait ataxia, abnormal eye movements, and seizures and hydrocephalus. The major MRI signs consist of fused cerebellar hemispheres, with absent or hypoplastic vermis, narrow diamond-shaped fourth ventricle and fused dentate nuclei. In a minority of cases, partial RS has been identified by MRI, demonstrating the presence of the nodulus and the anterior vermis and absence of part of the posterior vermis with only partial fusion of the hemispheres in the inferior part. Other cerebellar malformations involving vermian agenesis or hypoplasia include the Dandy–Walker continuum, Joubert syndrome, tectocerebellar dysraphy or pontocerebellar hypoplasias, and are now easily distinguished from RS by both brain MRI and morphology.

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Perinatal bacterial infection may be caused by any microorganism colonizing the vaginal tract. Neonatologists and paediatricians are especially concerned about group B Stretpococcus (GBS). However, Enterobactereacea, mainly E.coli and Proteus, are also responsible for infection. GBS screening may be accomplished in over 90% of pregnant women. In our maternity in 2007-2008, 85% of the mothers had been screened. Screening and prophylaxis were responsible for a decreasing incidence of neonatal infection - from 0.6/1000 to 0.15/1000 live births in Portugal, from 2002 to 2007. However there are some difficulties related to screening. In the second Portuguese study 16/57 NB with early-onset infection (28%) were born to “negative” mothers. Several factors illustrate how difficult is to draw national screening policies: a wide range of carrier’s state rate throughout a country - in Portugal from 12% to 30%. The success of any screening policy may also be affected by additional technical and organizational problems. In countries where home delivery is a tradition or a trend intrapartum GBS prophylaxis requires a very well organized assistance.. Moreover factors usually accepted as protective are not so effective. In the Portuguese study 24/57 infected newborns (42%) were delivery by caesarean section. Another subject deals with the workload in the postnatal ward generated by deficient compliance to the guidelines a problem not confirm by a study of our group. Decreasing the importance of GBS, highlight the importance of E. coli in perinatal infection. From the 16 340 registrations of the National Registry 1676 were newborns with mother-related infection. Applying the same reasoning to E.coli as to GBS and Listeria monocytogenes – that is considering all of them are of maternal origin - 6.7% of these infections were due to E. coli, 4.6% to SGB and 0.5% to Listeria monocytogenes. In conclusion screening and prophylaxis may be not the best way to prevent all GBS neonatal infections but by now it is the only available procedure. The other bacteria continue to demand a high suspicion level and immediate intervention.

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Introduction: Rhinoscleroma is a rare, chronic, granulomatous disease that most frequently affects the upper respiratory tract, especially the nasal cavity and sometimes extends through the lower respiratory tract. Is associated with Klebsiella rhinoscleromatis, which is endemic in certain geographic regions namely Central America. The pathogenesis and risk factors remain unclear. Clinical case: We report a five years Old Portuguese boy, previously healthy, brought to the Emergency Department with epistaxis, without other accompanying signs or symptoms. The Otorhinolaryngologist (ORL) performed rhinoscopy and identified an intranasal bleeding mass. The MRI revealed an intranasal mass with extension to the ethmoid bone sinus, and performed biopsy. The histopathology was vital, making the diagnosis of Rhinoscleroma. The child had traveled abroad for the first time on vacations a year before to Dominican Republic. The bacteriologic exam identified a Klebsiella spp. sensible to the association of amoxicillin and clavulanic acid. Blood test performed excluded association of immunodeficiency. Since it’s a rare disease genetic study are under course. Monthly evaluation by ORL and pediatrician was performed which documented progressive reduction until total disappearing of the macro and microscopic lesion, and negative bacteriologic exam. Six months of antibiotic therapy were completed without any known secondary effects. The child remained asymptomatic up to the last visit, 3 months following treatment and has shown no evidence of recurrence. Conclusion: Globalization and free transit of people to areas far from origin countries here some rare diseases are endemic brings a new challenge to modern medicine. Sometimes vacations bring more than memories.

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Precocious puberty, defined as the development of secondary sexual characteristics before the age of 8, often leads to anxiety in patients and their families but also in clinicians searching for the final diagnosis. After adequate investigation, the majority of the cases in girls turn out to be idiopathic. The authors present a case of McCune Albright syndrome in order to call attention to a rare cause of sexual precocity and the value of ultrasound in the evaluation of these situations. 10 years old infant girl admitted in our department due to irregular menstrual bleeding. She experienced a vaginal bleeding by the age of 3 which led to the diagnosis of McCune Albright Syndrome after a complete evaluation. Pubertal assessment revealed a reversed sequence in the remaining events with adrenarche at 5 and thelarche at 8. Hormonal evaluation demonstrated low FSH and LH levels (11,2 and 6,72 respectively) with high estrogen (204). Pelvic ultrasound showed a normal sized uterus (73x 29x32 mm), endometrial thickness of 5 mm and ovaries with several microfollicles and a copus luteum measuring 23 mm in the right ovary. McCune Albright syndrome is a very uncommon cause of sexual precocity that should, however, be suspected in all infant girls who present with vaginal bleeding. It is characterized by a triad: polyostotic fibrous dysplasia, gonadotropin-independent precocious puberty and café-au-lait skin spots. Due to autonomous production of estrogen by the ovaries, ultrasound image of the female reproductive tract is inconsistent with chronologic age. Pelvic ultrasound demonstrates a normal sized uterus with a well defined cervix and clearly identified ovaries with several follicles, similar to adult women of reproductive age. Ultrasonography of the pelvis has also an important role excluding other causes of GnRH-independent precocious puberty conditions like ovarian cysts or tumors.

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Background: The unique clinical syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly is very rare and can be quite difficult to recognize because of the enormous heterogeneity in its clinical presentation. There are few long-term reports of the reproductive performance of women with this syndrome following treatment, or about the location of subsequent pregnancies. Case: A case in which two spontaneous pregnancies occurred alternatively in both hemiuteri: one despite a previous ipsilateral large hematometra and hematocolpos and the other, 8 years after, simultaneously with contralateral hematometra and hematocolpos(because of vaginal restenosis), is reported. Drainage of hematocolpos was performed at 14 weeks of pregnancy with immediate pain relief. Results: Pregnancy proceeded without complications. Eight month after delivery, a vaginoplasty was performed by excising the longitudinal vaginal septum, and marsupializing the vaginal cuff. Conclusions: This case highlights the importance of a correct and early diagnosis of developmental anomalies of the urogenital tract, as well as how a conservative approach in a Mullerian anomaly with unilateral obstruction led to two successful pregnancies occurring alternatively in the unaffected and in the previously blocked side. This is additional information supporting that every effort should be made to preserve the obstructed uterus.

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Tem sido descrita uma correlação estreita entre infecção urinária, refluxo vesico-ureteral e disfunção miccional na criança. A obstrução funcional causada pela disfunção vesical/uretral representa um elevado risco de recorrência de infecção urinária, indução e perpetuação do refluxo (mesmo após correcção cirúrgica) é de lesão renal permanente. A normalização da alteração da micção como problema primário, é crítica na resolução de problemas secundários tais como a infecção urinária e o refluxo vesico-ureteral. Trinta e sete crianças com refluxo vesicoureteral secundário a disfunção miccional foram detectadas, avaliadas e tratadas entre 1990 e 1995 (5 anos). Foram estudados 49 ureteres. A infecção urinária foi o sintoma revelador em todas as crianças, ocorrendo entre 1 mês e os 13 anos de idade (mediana de 3,5 anos). Todas as crianças eram neurologicamente e estruturalmenle normais, detectando-se sintomatologia sugestiva de instabilidade ou imaturidade vesical em 34 (91,9%) e sugestiva de obstrução esfiocteriana funcional em três (8,1 %). Os estudos ecográfico e cistográfico efectuado em todas as crianças, com o apoio do estudo urodinâmico em 17 (45,9%) confirmaram o diagnóstico clínico. Em 29 (78,4%) das crianças foi efectuada cintigrafia com DMSA (Addo Dimercaptosuccínico), revelando cicatriz renal em 26 (89,6%) dos exames. Foi incentivado um programa de reeducação vesical e regularização dos hábitos intestinais em todas as crianças, associado a terapêutica anticolinérgica em 23 (62,2%) e/ou relaxantes musculares em três (8,1%) e fenoxibenzamina e algaliação intermitente (1,5 mês) em uma (2,7%), para além da quimioprofilaxia da infecção urinária instituida em 34 (91,9%) das crianças. Houve resolução completa da infecção urinária em 35 (94,6%) com redução da sua frequência nas outras duas (5,4%), cura do RVU em 32 (86,5%) e melhoria em quatro (10,8%). Verificou-se desaparecimento dos sinais de disfunção vesical em 22 (59,5%) casos com redução na intensidade e frequência em 14 (37,8%), mantendo-se uma criança (2,7%) com síndrome de urgência e refluxo vesico-ureteral inalterado. Estes dados implicam que a detecção e traramento da disfunção vesical/esfincteriana, são essenciais em todas as crianças com o complexo infecção urinária recorrente e refluxo vesicoureteral.

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OBJECTIVES: To evaluate the short- and medium-term results of prostatic arterial embolisation (PAE) for benign prostatic hyperplasia (BPH). METHODS: This was a prospective non-randomised study including 255 patients diagnosed with BPH and moderate to severe lower urinary tract symptoms after failure of medical treatment for at least 6 months. The patients underwent PAE between March 2009 and April 2012. Technical success is when selective prostatic arterial embolisation is completed in at least one pelvic side. Clinical success was defined as improving symptoms and quality of life. Evaluation was performed before PAE and at 1, 3, 6 and every 6 months thereafter with the International Prostate Symptom Score (IPSS), quality of life (QoL), International Index of Erectile Function (IIEF), uroflowmetry, prostatic specific antigen (PSA) and volume. Non-spherical polyvinyl alcohol particles were used. RESULTS: PAE was technically successful in 250 patients (97.9 %). Mean follow-up, in 238 patients, was 10 months (range 1-36). Cumulative rates of clinical success were 81.9 %, 80.7 %, 77.9 %, 75.2 %, 72.0 %, 72.0 %, 72.0 % and 72.0 % at 1, 3, 6, 12, 18, 24, 30 and 36 months, respectively. There was one major complication. CONCLUSIONS: PAE is a procedure with good results for BPH patients with moderate to severe LUTS after failure of medical therapy. KEY POINTS: • Prostatic artery embolisation offers minimally invasive therapy for benign prostatic hyperplasia. • Prostatic artery embolisation is a challenging procedure because of vascular anatomical variations. • PAE is a promising new technique that has shown good results.