Hospital de Santa Maria
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Lysophosphatidic acid enhances survival of human CD34(+) cells in ischemic conditions
Get PDFSeveral clinical trials are exploring therapeutic effect of human CD34(+) cells in ischemic diseases, including myocardial infarction. Unfortunately, most of the cells die few days after delivery. Herein we show that lysophosphatidic acid (LPA)-treated human umbilical cord blood-derived CD34(+) cells cultured under hypoxic and serum-deprived conditions present 2.2-fold and 1.3-fold higher survival relatively to non-treated cells and prostaglandin E2-treated cells, respectively. The pro-survival effect of LPA is concentration- and time-dependent and it is mediated by the activation of peroxisome proliferator-activator receptor γ (PPARγ) and downstream, by the activation of pro-survival ERK and Akt signaling pathways and the inhibition of mitochondrial apoptotic pathway. In hypoxia and serum-deprived culture conditions, LPA induces CD34(+) cell proliferation without maintaining the their undifferentiating state, and enhances IL-8, IL-6 and G-CSF secretion during the first 12 h compared to non-treated cells. LPA-treated CD34(+) cells delivered in fibrin gels have enhanced survival and improved cardiac fractional shortening at 2 weeks on rat infarcted hearts as compared to hearts treated with placebo. We have developed a new platform to enhance the survival of CD34(+) cells using a natural and cost-effective ligand and demonstrated its utility in the preservation of the functionality of the heart after infarction.info:eu-repo/semantics/publishedVersio
Overlap between Frontotemporal Dementia and Bipolar Disorder: a case report
Get PDFA variante comportamental da demência frontotemporal (DFTvc) apresenta-se
frequentemente com sintomas psiquiátricos, entre os quais alterações comportamentais
e da personalidade. Antes do estabelecimento dos défices cognitivos, a diferenciação
entre a DFTvc e outras perturbações psiquiátricas pode ser desafiante.
Este artigo apresenta um caso de um episódio maníaco com sintomas psicóticos
num doente de 61 anos cujas alterações da personalidade e as dificuldades na realização
das tarefas diárias não só se mantiveram, como se agravaram após o tratamento da
sintomatologia maniforme e psicótica. Após estudo posterior com avaliação neuropsicológica
formal, tomografia computorizada crânio-encefálica (TC-CE) e SPECT
cerebral, estabeleceu-se o diagnóstico provável de DFTvc.
O presente caso atenta para a possível progressão de formas atípicas e tardias
de Perturbação Bipolar (PB) para Demências Frontotemporais. Têm sido propostas
várias ligações entre DFT e PB, entre as quais se destacam o envolvimento do gene
C9ORF72 em alguns doentes com PB que posteriormente evoluem para demência.info:eu-repo/semantics/publishedVersio
Endogenous endophthalmitis secondary to erysipelas
Get PDFA 64-year-old woman with chronic right arm lymphoedema presented with progressive and painful vision loss in the right eye following diagnosis of erysipelas in the ipsilateral arm. Visual acuity was light perception. Biomicroscopy revealed marked conjunctival injection, decreased corneal transparency and an inflammatory mass in the anterior chamber, which precluded fundoscopy. The ocular ultrasonography features were consistent with acute endophthalmitis, and the patient was admitted to the hospital. A systemic evaluation, including complete physical examination, echocardiography and blood tests, ruled out other sources of infection besides the cutaneous site. Blood cultures were positive for group A Streptococcus. A diagnosis of unilateral acute endophthalmitis due to group A Streptococcus bacteraemia secondary to erysipelas was made and successfully treated with optimal medical care, including prompt intravitreal and systemic antibiotic administration. Despite resolution of the infectious process, visual acuity did not improve.info:eu-repo/semantics/publishedVersio
Doença de Fabry, revisão de uma doença olho-rim
No full textFabry’s disease is a recessive X -linked disorder that results from a deficiency of the hydrolase alpha-
-galactosidase A (α -Gal A). The absence of α -Gal A enzyme activity leads to accumulation of glycosphingolipid
globotryaosylceramide (GL -3) in the lysosomes of a variety of cell types. It can cause skin and ocular lesions,
progressive renal, cardiac or cerebrovascular disorders. The authors report the case of a 39 -year -old female
who was referred to a nephrology appointment by her ophthalmologist, after the diagnosis of cornea verticillata
and posterior subcapsular cataract. This case illustrates the importance of a multidisciplinary evaluation
to an effective clinical screening. In males, most symptoms begin in childhood; in females the onset
can be observed later and presentation is more variable. Various manifestations often lead to misdiagnosis
or are frequently delayed for many years. Enzyme replacement therapy highlights the importance of early
diagnosis so that treatment can be initiated before irreversible organ damage occurs
Mode I fracture characterization of human bone using the DCB test
Get PDFPurpose
– Fracture characterization of human cortical bone under pure mode I loading was performed in this work. The purpose of this paper is to validate the proposed test and procedure concerning fracture characterization of human cortical bone under pure mode I loading.
Design/methodology/approach
– A miniaturized version of the double cantilever beam (DCB) test was used for the experimental tests. A data reduction scheme based on crack equivalent concept and Timoshenko beam theory is proposed to overcome difficulties inherent to crack length monitoring during the test. The application of the method propitiates an easy determination of the Resistance-curves (R-curves) that allow to define the fracture energy under mode I loading from the plateau region. The average value of fracture energy was subsequently used in a numerical analysis with element method involving cohesive zone modelling.
Findings
– The excellent agreement obtained reveals that the proposed test and associated methodology is quite effective concerning fracture characterization of human cortical bone under pure mode I loading.
Originality/value
– A miniaturized version of traditional DCB test was proposed for cortical human bone fracture characterization under mode I loading owing to size restrictions imposed by human femur. In fact, DCB specimen propitiates a longer length for self-similar crack propagation without undertaking spurious effects. As a consequence, a R-curve was obtained allowing an adequate characterization of cortical bone fracture under mode I loading
Favorable outcome of total hip arthroplasty with insufficient bone coverage of the roof reinforcement ring
No full textINTRODUCTION:
Total hip arthroplasty in patients with developmental dysplasia of the hip can be a complex procedure due to acetabular and proximal femoral deformities.
PRESENTATION OF CASE:
A 59-year-old male patient underwent a total hip arthroplasty for the treatment of end-stage dysplastic osteoarthritis. A roof reinforcement ring, a cemented polyethylene cup, and a cementless stem were used. A portion of the superior rim of the ring was uncovered by the host bone. Morsellized autogenous femoral-head graft was impacted to fill the space between the superior rim of the ring and the superior part of the dysplastic acetabulum. At the follow-up after 5-years, the patient had no complaints and was very satisfied with the operation result. The hip radiograph revealed no signs of instability of the acetabular component, and no bone graft resorption.
DISCUSSION:
Favorable results were described using metal rings and conical femoral stems for the treatment of the developmental dysplasia of the hip. The superior rim of the metal ring should be against host bone for 60% of its support. Despite the suboptimal implantation of the ring compromising, apparently, mechanical stability of the arthroplasty, the outcome was favorable.
CONCLUSION:
This result can be supported by the good fixation of the metal ring to the pelvis with screws, the adequate orientation of both components of the total hip arthroplasty, and the bone graft incorporation
The other side of recovery: validation of the Portuguese version of the subjective experiences of psychosis scale.
Get PDFBACKGROUND:
The aim of this study was to develop and validate a Portuguese version of The Subjective Experiences of Psychosis Scale (SEPS) for use in Portuguese-speaking populations in order to provide a self-report instrument to assess and monitor dimensions of psychotic experiences, translating patient's perspective and experience in terms of recovery from psychosis.
METHODS:
The sample consisted of 30 participants with psychotic disorders who had recently experienced delusions or hallucinations. The SEPS was completed along with other observer-based assessments and self-report questionnaires, such as the Brief Psychiatric Rating Scale, the Insight and Treatment Attitudes Questionnaire and the Function Assessment Short Test.
RESULTS:
Two main factors representing the positive and negative components of each subscale were identified. We obtained good internal consistency and test-retest reliability for the positive and negative components of all subscales. The subscales of SEPS correlated with observer-based assessments and self-report questionnaires.
CONCLUSIONS:
The Portuguese version of the SEPS is a useful tool in the assessment and monitoring of psychotic symptoms
Twins prematurity--the influence of prenatal surveillance
No full textOBJECTIVE:
To evaluate the influence of the local prenatal surveillance of twin pregnancies in the obstetrical results.
METHODS:
A prospective cohort study of multiple pregnancies delivered over a period of 16 years in a tertiary centre was conducted. In this study 861 twin pregnancies were included. They were compared for obstetric complications, gestational age at delivery, mode of delivery and birthweight, according to the place of the surveillance.
RESULTS:
Of the 861 cases examined, the following obstetric complications were significantly different: metrorrhagia (p = 0.039), infections (p < 0.001), HELLP (p = 0.007), PROMPT (p < 0.001) and fetal death (p = 0.024). The mode of delivery was similar but occurred mostly ≤32 weeks (p < 0.001), the birthweight was mostly <2000 g and occurred more NICU admission (p < 0.001) when surveillance was outside the MPC-MDM.
CONCLUSION:
Our results demonstrate the crucial importance of prenatal surveillance be carried in a differentiated referral centers with specific/strict protocols or the urgent implementation of same protocols in all other places of surveillance, since this straight surveillance greatly reduces the occurrence of prenatal complications, mainly PROMPT, PTD
RETINAL ANGIOMATOUS PROLIFERATION: A Quantitative Analysis of the Fundoscopic Features of the Fellow Eye
No full textPURPOSE:
To quantitatively analyze and compare the fundoscopic features between fellow eyes of retinal angiomatous proliferation and typical exudative age-related macular degeneration and to identify possible predictors of neovascularization.
METHODS:
Retrospective case-control study. Seventy-nine fellow eyes of unilateral retinal angiomatous proliferation (n = 40) and typical exudative age-related macular degeneration (n = 39) were included. Fundoscopic features of the fellow eyes were assessed using digital color fundus photographs taken at the time of diagnosis of neovascularization in the first affected eye. Grading was performed by two independent graders using RetmarkerAMD, a computer-assisted grading software based on the International Classification and Grading System for age-related macular degeneration.
RESULTS:
Baseline total number and area (square micrometers) of drusen in the central 1,000, 3,000, and 6,000 μm were considerably inferior in the fellow eyes of retinal angiomatous proliferation, with statistically significant differences (P 510,196 μm2 in the central 6,000 μm was associated with an increased risk of neovascularization (hazard ratio, 4.35; 95% confidence interval [1.56-12.15]; P = 0.005).
CONCLUSION:
Baseline fundoscopic features of the fellow eye differ significantly between retinal angiomatous proliferation and typical exudative age-related macular degeneration. A large area (>510,196 μm2) of soft drusen in the central 6,000 μm confers a significantly higher risk of neovascularization and should be considered as a phenotypic risk factor
Familial thrombotic risk based on the genetic background of Protein C Deficiency in a Portuguese Study
Get PDFINTRODUCTION:
Inherited protein C (PC) deficiency is a well-known risk factor for venous thrombosis (VT). Plasma PC levels are reliable in moderate to severe deficiencies; however, in mildly deficient individuals, the levels may overlap with those considered normal. Genetic studies of PROC, which encodes PC, could help identify carriers; genome-wide association studies (GWAS) have shown that approximately 50% of phenotypic variation in PC deficiency is caused by the cumulative effects of mutations in several other loci, namely in the PROCR.
PATIENTS AND METHODS:
With the main objective of determining the genotype/phenotype correlation in 59 Portuguese individuals from 26 unrelated families with history of thrombosis and repeatedly low/borderline PC plasma levels, we conducted a molecular study by direct sequencing of PROC; PROC promoter haplotypes and PROCR c.4600A>G polymorphism (rs867186), which are known to influence plasma PC concentrations, were also screened.
RESULTS:
Twelve different PROC mutations were identified, one of them not previously reported, p.Cys105Arg. The mutation types and locations as well as haplotype combinations correlated with the phenotypic severity. The most frequent mutation, p.Arg199X, correlated with the CGTC haplotype and was identified in nine families containing patients with higher numbers of VT episodes. This mutation in homozygous individuals for the CGTC haplotype is a significant risk factor for VT in Portuguese.
CONCLUSION:
These genetic family studies allowed the identification of the unknown carriers and individuals at a higher thrombotic risk within each family, thus permitting the evaluation of the need for prophylactic measures, particularly in at-risk situations.info:eu-repo/semantics/publishedVersio