Browsing by Author "Coimbra, Miguel T"
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- Accidental Diagnosis of Isolated Persistent Left Superior Vena Cava After an Elective Central Venous Access Procedure for Chronic Hemodialysis: Clinical Implications and Precautions From a Case ReportPublication . Coimbra, Miguel T; Braga, Beatriz; Silva, Adriana; Sousa, Fernanda; Queirós, JoséPersistent left superior vena cava (PLSVC) is the most frequent thoracic venous anatomical variant in the general population. Isolated PLSVC, without formation of the right superior vena cava, is described in 10% of cases of PLSVC only. While it can be associated with congenital heart disease, arrhythmias, and premature death, adult patients with PLSVC are mostly asymptomatic, and the diagnosis is usually accidental. We present the case of a 72-year-old male with end-stage renal disease who was started on urgent hemodialysis through a temporary non-tunneled femoral central venous catheter (CVC) in the SLED (slow low-efficiency dialysis) modality and later remained dependent on hemodialysis. At this stage, placement of a tunneled CVC in the right internal jugular vein was necessary and fluoroscopy guidance was not available. There were no complications during the procedure, but postoperative conventional chest radiography revealed an inadequate positioning of the CVC tip in the left hemithorax, crossing the midline. Subsequently, the diagnosis of PLSVC was obtained by performing a thoracic angio-CT scan, confirming CVC tip positioning inside the PLSVC, and also excluded the presence of cardiac defects or additional anatomical variations of the great vessels of the thorax. Early evaluation for the creation of autologous vascular access was started under our care, and there were no mechanical or other complications associated with hemodialysis sessions during early follow-up after discharge.
- Medical Challenges of a Common Variable Immunodeficiency With a TNFRSF13B Gene Mutation in a Simultaneous Kidney and Pancreas Transplant RecipientPublication . Coimbra, Miguel T; Silvano, José; Martins, La SaleteCommon variable immune deficiency (CVID) is a primary immunodeficiency disorder, with hypogammaglobulinemia and increased susceptibility to recurrent infections, autoimmune disorders, granulomatous diseases and malignancy. Among the solid organ transplant (SOT) recipient population, those with primary immunodeficiency disorders under chronic immunosuppression therapy can theoretically be at higher risk of atypical infections, autoimmune complications and disease recurrence with suboptimal long term graft survival, but literature is scarce. Here, we report a 27-year-old female with type 1 diabetes mellitus, complicated with nephropathy that progressed to end-stage renal disease (ESRD), who had a history of a chronic inflammatory response dysregulation, with chronic monoarthritis, persistent elevation of inflammation markers, recurrent infections, low immunoglobulin G (IgG) and A (IgA) serum levels, a slightly decreased population of memory B cells at flow cytometric immunophenotyping, and a confirmed pathological heterozygous mutation in the tumor necrosis factor receptor superfamily 13B (TNFRSF13B), with a suspected diagnosis of CVID. Whilst on hemodialysis, she received a simultaneous kidney and pancreas transplant from a standard criteria donor (SCD), and our induction and maintenance immunosuppression protocol and prophylaxis regimen allowed for a successful transplant with immediate pancreatic function, with no evidence of renal graft rejection upon biopsy in the early post-transplant period, and no novel episodes of serious infectious complications were recorded during a follow-up period of six months