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Dysphagia lusoria: uncommon cause of dysphagia in children
Get PDFDysphagia lusoria (lusus naturae, latin for "freak of nature") describes dysphagia because of vascular compression of the esophagus. Symptoms, when present, occur at the two extremes of life. Owing to the more flexible and compressible nature of the trachea, children usually present with respiratory symptoms, in contrast to adults, who more often present with dysphagia. Here, we report the case of a six-year-old child presenting with a history of progressive dysphagia without respiratory symptoms. A barium esophagogram showed a diagonal impression in the proximal esophagus, while at esophagogastroduodenoscopy there was an extrinsic pulsatile bulging area suggesting an extrinsic compression by an aberrant vessel. Angio-CT (computed tomography) scan confirmed the presence of an aberrant right subclavian artery.info:eu-repo/semantics/publishedVersio
Portuguese consensus document statement in diagnostic and management of atypical hemolytic uremic syndrome
Get PDFAmong thrombotic microangiopathies (TMA), the hemolytic uremic syndrome associated with dysregulation of the alternative complement pathway (aHUS) is one of the most challenging diseases a nephrologist can face. By the end of the XXth century, the complement’s role was unraveled with the discovery that mutations in the factor H coding gene were responsible for aHUS. But it was the acknowledgment that pharmacological C5-9 blockage provided a cure for aHUS that fostered the interest of the nephrology community in the genetics, pathophysiology and therapeutics of, not only of aHUS, but TMA in general. The molecular genetics of aHUS is technically demanding and, as such, in Portugal (alike many other European countries) a single laboratory emerged as a national reference center. The fact that all samples are evaluated in a single center provides a unique opportunity for data collection and a forum for discussion for all those interested in the field: immunologists, molecular geneticists, pathologists and nephrologists. The current consensus document emerged from such a discussion forum and was sponsored by the Portuguese Society of Nephrology. The goal is more to portray the Portuguese picture regarding the diagnostic approach and therapeutic options than to extensively review the state of the art of the subject. The accompanying documents that are published as supplementary data are in line with that goal. They range from the informed consent and clinical form to be sent together with the biological samples for genetic testing, to the appendix regarding the actual sampling and storing conditions. The document is also intended to set an example for future documents and independente discussion forums on other kidney diseases for which emerging diagnostic and/or therapeutic strategies are reaching clinical practice.info:eu-repo/semantics/publishedVersio
Artroplastia total do joelho em doente previamente patelectomizado: reconstrução patelar com técnica original utilizando autoenxerto de prato tibial
Get PDFIntrodução: os autores pretendem demonstrar uma técnica original de reconstrução patelar, apresentando para isso um caso clínico. Trata-se de uma doente previamente submetida a patelectomia por artrose patelofemoral, que acabou por desenvolver artrose femoro-tibial que condicionava dor resistente ao tratamento conservador. Perante uma doente com diminuição de força do aparelho extensor, consideramos que seria benéfica a reconstrução patelar no momento da artroplastia.
Caso Clínico: neste trabalho apresentamos o estado funcional da doente, incluindo as mobilidades e scores funcionais pré e pós-operatórios. São descritos em pormenor todos os passos que constituíram o procedimento cirúrgico, incluindo a colheita do enxerto, o ajuste do mesmo às dimensões e forma necessárias e a forma de integração em bolsa subsinovial confecionada.
Discussão-Conclusão: a patelectomia não deve excluir a indicação para artroplastia do joelho, não só pelos bons resultados que pode ter como também pela diversidade de técnicas de reconstrução patelar que existe. Apesar dos bons resultados com as técnicas já conhecidas, todas acabam por ter desvantagens nem sempre desprezíveis. O uso de prato tibial externo parece-nos uma excelente opção com morbilidade mínima que além dos bons resultados obtidos no imediato, demonstrou vantagem clínica a médio prazo.Introduction: the authors aim to demonstrate an original technique for patellar reconstruction, presenting a clinical case. It’s a patient previously patellectomized for patelofemoral osteoarthritis, with progressive symptomatic femorotibial compartment osteoarthritis in the ipsilateral knee. Given the decreased extensor strength of extensor apparatus of the knee, we considered it would be important to reconstruct the patella at the time of arthroplasty
Case Presentation: in this paper, we present patient functional status, including pre- and post-operative range of motion and functional scores. We describe in detail all the steps constituting the surgical procedure, including graft harvesting, the graft adjustment to size and shape needed and how it was integrated in a subsynovial pouch.
Discussion-Conclusion: patellectomy should not exclude a patient from a total knee arthroplasty, not only for the possible good results, but also for the diversity of surgical techniques for patella reconstruction. Despite the good results with recognized techniques, all of them have some disadvantages. The use of external tibial plateau seems to be an excellent option, with minimum morbidity and, beyond the immediate good results, demonstrated mid-term clinical advantages.info:eu-repo/semantics/publishedVersio
Diagnosis of monoclonal gammopathy of renal significance
Get PDFMonoclonal gammopathies are a heterogeneous group of disorders characterized by clonal proliferation of immunoglobulin produced by B-lymphocytes or plasma cell clone.
The term monoclonal gammopathy of renal significance (MGRS) was introduced to distinguish monoclonal gammopathies that result in the development of kidney disease from those that are benign.
Screening for monoclonal immunoglobulin and an appropriate hematologic workup are fundamental and sometimes a difficult challenge, with therapeutic and prognostic implications. Kidney biopsy is essential to determine the exact nature of the lesion and to evaluate the severity of renal disease.
In this review we discuss the clinical and pathologic features of MGRS, highlighting the most diagnostic difficulties and current therapeutic options.info:eu-repo/semantics/publishedVersio
A pilot study on the usefulness of peripheral blood flow cytometry for the diagnosis of lower risk myelodysplastic syndromes: the "MDS thermometer"
Get PDFBackground:
Immunophenotypic analysis of the bone marrow (BM) cells has proven to be helpful in the diagnosis of Myelodysplastic Syndromes (MDS). However, the usefulness of flow cytometry (FCM) for the detection of myelodysplasia in the peripheral blood (PB) still needs to be investigated. The aim of this pilot study was to evaluate the value of FCM-based PB neutrophil and monocyte immunophenotyping for the diagnosis of lower risk MDS (LR-MDS).
Methods:
We evaluated by 8-color FCM the expression of multiple cell surface molecules (CD10, CD11b, CD11c, CD13, CD14, CD15, CD16, CD34, CD45, CD56, CD64 and HLA-DR) in PB neutrophils and monocytes from a series of 14 adult LR-MDS patients versus 14 normal individuals.
Results:
Peripheral blood neutrophils from patients with LR-MDS frequently had low forward scatter (FSC) and side scatter (SSC) values and low levels of CD11b, CD11c, CD10, CD16, CD13 and CD45 expression, in that order, as compared to normal neutrophils. In addition, patients with LR-MDS commonly display a higher fraction of CD14+CD56+ and a lower fraction of CD14+CD16+ monocytes in the PB. Based on these results, we proposed an immunophenotyping score based on which PB samples from patients with LR-MDS could be distinguished from normal PB samples with a sensitivity 93% and a specificity of 100%. In addition, we used this score to construct the MDS Thermometer, a screening tool for detection and monitoring of MDS in the PB in clinical practice.
Conclusions:
Peripheral blood neutrophil and monocyte immunophenotyping provide useful information for the diagnosis of LR-MDS, as a complement to cytomorphology. If validated by subsequent studies in larger series of MDS patients and extended to non-MDS patients with cytopenias, our findings may improve the diagnostic assessment and avoid invasive procedures in selected groups of MDS patients.info:eu-repo/semantics/publishedVersio
The role of endoscopic ultrasound in a case of lung cancer with jaundice
Get PDFLung cancer is one of the major causes of death in the world. Small cell carcinoma is the most aggressive type and can spread rapidly. The association of a small cell carcinoma with hepatic hilar metastasis and biliary obstruction is rare. Endoscopic ultrasound allows the aspiration of a cytology sample from adenopathies for diagnostic purpose. We present the case of a patient with lung cancer, with lymph node metastasis to the hepatic hilum and extrinsic biliary tree compression. Endoscopic ultrasound allowed the definitive diagnosis of hepatic hilar metastasis of a lung small cell carcinoma. To the author's knowledge it was the first time that endoscopic ultrasound was used for the diagnosis of hepatic hilar lymph node metastasis of lung cancer.info:eu-repo/semantics/publishedVersio
Glycans as Key Checkpoints of T Cell Activity and Function
Get PDFThe immune system is highly controlled and fine-tuned by glycosylation, through the addition of a diversity of carbohydrates structures (glycans) to virtually all immune cell receptors. Despite a relative backlog in understanding the importance of glycans in the immune system, due to its inherent complexity, remarkable findings have been highlighting the essential contributions of glycosylation in the regulation of both innate and adaptive immune responses with important implications in the pathogenesis of major diseases such as autoimmunity and cancer. Glycans are implicated in fundamental cellular and molecular processes that regulate both stimulatory and inhibitory immune pathways. Besides being actively involved in pathogen recognition through interaction with glycan-binding proteins (such as C-type lectins), glycans have been also shown to regulate key pathophysiological steps within T cell biology such as T cell development and thymocyte selection; T cell activity and signaling as well as T cell differentiation and proliferation. These effects of glycans in T cells functions highlight their importance as determinants of either self-tolerance or T cell hyper-responsiveness which ultimately might be implicated in the creation of tolerogenic pathways in cancer or loss of immunological tolerance in autoimmunity. This review discusses how specific glycans (with a focus on N-linked glycans) act as regulators of T cell biology and their implications in disease.The Institute of Molecular Pathology and Immunology of the University of Porto integrates the i3S research unit, which is partially supported by the Portuguese Foundation for Science and Technology (FCT). This article is a result of the project NORTE-01-0145-FEDER-000029, supported by the Norte Portugal Regional Programme (NORTE 2020) under the PORTUGAL 2020 Partnership Agreement through the European Regional Development Fund. This work was also funded by Fundo Europeu de Desenvolvimento Regional (FEDER) funds through the COMPETE 2020—Operacional Programme for Competitiveness and Internationalization (POCI), Portugal 2020, and by Portuguese funds through the FCT in the framework of the project (POCI-01/ 0145-FEDER-016601 and PTDC/DTP-PIC/0560/2014, as well as POCI-01-0145-FEDER-028772). SSP acknowledges the European Crohn's and Colitis Organization (ECCO) for ECCO Grant 2017, the Broad Medical Research Program at the Crohn's and Colitis Foundation of America, and the Portuguese Group of Study in IBD (GEDII) for funding. MSP [SFRH/BD/110148/2015], IA [SFRH/BD/128874/2017], MV [PD/BD/135452/2017], received funding from the FCinfo:eu-repo/semantics/publishedVersio
Alternative method of outside-in meniscal repair for anterior horn tears
Get PDFTreatment of symptomatic meniscal tears continues to evolve as we improve our understanding of the biomechanical role of the meniscus and its long-term importance to the health of the knee joint. Suture repair of meniscal tears is challenging and continues to rise as we aim to preserve meniscal tissue. Outside-in meniscal suture techniques may involve using expensive equipment that is not readily available for immediate use in most operating rooms. Aware of the different techniques available, the authors describe a fast and reproducible technique that does not require the use of specific material or equipment.info:eu-repo/semantics/publishedVersio
Pulmonary hypertension: Real-world data from a Portuguese expert referral centre
Get PDFPulmonary hypertension (PH) is a heterogeneous, debilitating condition with highly relevant impact on functional capacity, quality of life, and life-expectancy.info:eu-repo/semantics/publishedVersio
Pulmonary function and respiratory muscle strength after arthrodesis of the spine in patients who have adolescent idiopathic scoliosis
No full textinfo:eu-repo/semantics/publishedVersio