RESUMEN

Paroxysmal cold hemoglobinuria (PCH) is a form of cold autoimmune hemolytic anemia characterized by the presence of the Donath-Landsteiner antibody, which triggers complement-mediated intravascular hemolysis when the body temperature changes from cold to warm. PCH occurs primarily in children as a rare, self-limiting disease following viral infections. In contrast, adult-onset PCH is very rare and associated with a diverse range of underlying conditions, which complicates its management and treatment. We describe a case of adult-onset PCH following COVID-19, effectively managed with a single dose of sutimlimab, a selective classical complement pathway inhibitor. This intervention was performed during a life-threatening hemolytic crisis, at a time requiring swift decision-making when specific tests to differentiate from other hemolytic anemias were not readily available. This case illustrates the potential of using a single dose of sutimlimab to manage life-threatening hemolytic crises in PCH, highlighting the significance of inhibiting the classical complement pathway.

RESUMEN

OBJECTIVES: Tracheal regeneration is challenging owing to its unique anatomy and low blood supply. Most tracheal regeneration applications require scaffolds. Herein, we developed bio-three-dimensional-printed scaffold-free artificial tracheas. METHODS: We fabricated bio-three-dimensional-printed artificial tracheas. Their anterior surface comprised hyaline cartilage differentiated from mesenchymal stem cells, and their posterior surface comprised smooth muscle. Human bone marrow-derived mesenchymal stem cells were cultured and differentiated into chondrocytes using fibroblast growth factor-2 and transforming growth factor-beta-3. Initially, horseshoe-shaped spheroids were printed to cover the anterior surface of the artificial trachea, followed by the application of human bronchial smooth muscle cells for the posterior surface. After a 3-week maturing process, the artificial trachea was subjected to histological and immunohistochemical analyses. RESULTS: The anterior surface of the artificial trachea comprised well-differentiated hyaline cartilage from human bone marrow-derived mesenchymal stem cells. Immunohistochemistry revealed that the smooth muscle expressed α-smooth muscle actin and smooth muscle myosin heavy chain 11. CONCLUSIONS: A bio-three-dimensional-printed scaffold-free artificial trachea comprising different tissues at the front and back was successfully fabricated.

Asunto(s)

RESUMEN

Ureteral strictures, which can be caused by ureteral injury, radiation therapy, ureterolithiasis, urinary tract infections, and ureteral endometriosis, typically require ureteral reconstruction. Although tissue engineering, autologous alternative tissue transplantation, and surgical techniques applying various flaps have been carried out for ureteral regeneration, all with some success, each method has its advantages and disadvantages. As an alternative, we created the first artificial ureter structures using only live cells and grafted them into healthy rat ureters. Spheroids were created using normal human dermal fibroblasts and human umbilical vein endothelial cells and subsequently laminated using a bio-three-dimensional printer. After molding the laminated spheroids into tubular structures, the artificial ureters were transplanted into live rats. After 2-12 weeks, the animals were sacrificed and their gross and pathological features were examined. In the artificial ureteral lumen of rats with Grade 0-1 hydronephrosis, regeneration of the ureteral epithelium was observed, the thickness of which increased over the course of the experiment. Regeneration of the muscular layer was also observed, extending from the normal ureteral side toward the artificial ureter structure over time. However, complete regeneration was not observed at the end of 12 weeks. Although ureteral peristalsis was noted in all cases, it was weaker than expected. Therefore, we achieved short-segment ureteral regeneration using a cell-only structure. This finding suggests that by applying alternative strategies to this method, such as changing the cell type and composition, regeneration over the entire length of the ureter may be possible in the future. STATEMENT OF SIGNIFICANCE: Until now, ureteral regeneration techniques have been dominated by the use of high-molecular-weight compounds and autologous tissues, and there have been no reports of regeneration using structures made entirely of cells. This is the first report of ureteral regeneration using a tubular structure made from stacked spheroids. Although this study only attained short-segment ureteral regeneration, regeneration of the ureter over a much longer proportion of its length can be achieved in the future by applying other strategies, such as changing the cell type. This study provides a foundation to achieve the future goal of complete regeneration.

Asunto(s)

RESUMEN

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by infiltration of extensive IgG4-positive plasma cells and lymphocytes. Although IgG4-RD has been observed in almost all organs, it rarely affects the myocardium. Cardiovascular lesions of IgG4-RD appear as aortic (aortic aneurysm and aortitis) and pericardial (constrictive pericarditis) lesions as well as pseudotumors around the coronary arteries. We herein report a case of IgG4-RD with a cardiac mass in the right atrium involving a sinus node. This condition caused arrhythmia and repeated strokes. We successfully treated the patient through resection of the cardiac mass, catheter ablation and immunosuppressive therapy.

Asunto(s)

RESUMEN

We describe the use of veno-arterial extracorporeal membrane oxygenation (ECMO) in a 35-year-old female with severe fixed pulmonary hypertension who went into cardiogenic shock during a Cesarean section. Pregnancy in the presence of severe pulmonary hypertension is typically contraindicated due to high maternal mortality rates. This patient visited our hospital at 37 weeks of gestation after experiencing dyspnea and chest pain. Clinical evaluation revealed severe fixed pulmonary hypertension. At the time of the planned delivery, femoral lines were placed; in case of emergency, ECMO became necessary during the delivery. During delivery, the patient developed sudden hemodynamic collapse necessitating rapid cannulation and initiation of ECMO. She was stabilized pharmacologically and separated from ECMO after 2 days. The baby was delivered uneventfully, and the mother and child were discharged 1 month after delivery.

Asunto(s)

RESUMEN

The patient was a 58-year-old man with advanced gastric cancer with multiple liver metastases. He received TXL/TS-1 therapy during February 2009, but treatment was stopped immediately when he developed anorexia, diarrhea, and numbness in his fingers. Therefore, only TS-1 was administered. Following treatment initiation, tumor marker levels promptly dropped. The gastric lesion disappeared and, to date, only a slight scar remains since April 2010. Similarly, liver metastases have not been detected since August 2011. There has been no lesion progression for 6 years since the start of the chemotherapy.

Asunto(s)

RESUMEN

Lung herniation is rare. We describe two cases; one cured by surgery, and the other observed without surgery. A 61-year-old man underwent minimally invasive cardiac surgery for mitral valve plasty. Four weeks postoperatively, chest computed tomography (CT) revealed exacerbating lung herniation and emergency surgery was performed. A 75-year-old man with metastatic tumor underwent partial resection of the left lower lobe through a 10-cm access window. Three months postoperatively, follow-up chest CT revealed prolapse of a small part of the upper lobe at the site of incision. However, he remained asymptomatic and was observed on an outpatient basis.

Asunto(s)