Publications by authors named "Stephanie Nguyen"

Among repairs for ventriculoarterial discordance, ventricular septal defect, and pulmonary stenosis, aortic root translocation (Nikaidoh operation) offers the most anatomic result. With a diminutive pulmonary annulus or hypoplastic left ventricular outflow tract, the distance gained posteriorly with aortic translocation is negligible. We developed the "hemi-Nikaidoh" procedure as an alternative.

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Context: Mucopolysaccharidosis (MPS) requires urgent treatment to prevent neurological damage. While gene therapy holds promise for effectively treating these diseases with minimal toxicity, access remains limited for most patients. Consequently, advancing allogeneic hematopoietic stem cell transplantation (HSCT) for young children is crucial.

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BACKGROUND Focal segmental glomerulosclerosis (FSGS) very rarely occurs in patients with multiple myeloma. Much more common are renal impairments secondary to monoclonal light-chain tubulopathy, AL amyloidosis, light-chain deposition disease, and the so-called monoclonal gammopathy of renal significance. CASE REPORT We report the case of a 79-year-old myeloma patient without noticeable medical problems but with a long history of myeloma treatment beginning 13 years ago.

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Despite the growing evidence supporting the existence of CNS involvement in acute and chronic graft-versus-host disease (CNS-GvHD), the characteristics and course of the disease are still largely unknown. In this multicenter retrospective study, we analyzed the clinical, biological, radiological, and histopathological characteristics, as well as the clinical course of 66 patients diagnosed with possible CNS-GvHD (pCNS-GvHD), selected by predetermined diagnostic criteria. Results were then contrasted depending on whether pCNS-GvHD occurred before or after day 100 following allogeneic hematopoietic stem cell transplantation.

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  • Surgeons often leave a residual atrial-level communication during Tetralogy of Fallot (TOF) repairs, and this study aimed to see how closing this communication affects patient recovery.
  • The researchers reviewed records of 132 TOF patients who had surgery before 12 months old, comparing outcomes between those with and without the communication.
  • The results showed no significant differences in key recovery metrics like cardiac output, drug therapy duration, or intensive care stay, suggesting that closing the communication doesn't significantly alter short-term or mid-term recovery outcomes.
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Organoids are self-organizing 3D cell culture models that are valuable for studying the mechanisms underlying both development and disease in multiple species, particularly, in humans. These 3D engineered tissues can mimic the structure and function of human organs in vitro. Methods to generate organoids have substantially improved to better resemble, in various ways, their in vivo counterpart.

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JCO Primary analysis of the phase III randomized AATT study showed that younger patients with peripheral T-cell lymphoma (PTCL) consolidated with autologous or allogeneic transplantation (alloSCT) had similar event-free survival (EFS) and overall survival (OS). Seven-year EFS of patients randomly assigned to alloSCT was 38% (95% CI, 25 to 52) compared with 34% (95% CI, 22 to 47) for patients randomly assigned to autologous transplantation of hematopoietic stem cells (autoSCT); OS was 55% (95% CI, 41 to 69) and 61% (95% CI, 47 to 74). Among patients undergoing alloSCT (n = 26) or autoSCT (n = 41) on study, the cumulative progression/relapse rate was 8% (95% CI, 0 to 19) and 55% (95% CI, 35 to 74).

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Allogeneic hematopoietic stem cell transplantation (alloHSCT) is a potentially curative treatment for acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS). However, these transplants are complicated by a high rate of relapse in very high cytogenetic risk or refractory diseases. The benefit of this therapeutic strategy for these serious malignant hemopathies could therefore be reassessed.

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  • Lymphangioleiomyomatosis (LAM) is a rare lung disease mostly affecting women, often leading to complications like pneumothorax and chylothorax, so surgical management options were examined.
  • The study reviewed records of 326 patients suspected of having LAM, revealing that 40.5% underwent surgical procedures, with significant instances of pneumothorax and associated treatments like pleurodesis.
  • Findings suggest a high occurrence of pleural disease in LAM patients, indicating a need for thoracic surgeons to be aware of these issues and highlighting the potential benefits of using tunneled indwelling pleural catheters for better management.
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  • This study examines the effects of IDH1 and IDH2 mutations on adult AML patients who received allogeneic hematopoietic cell transplantation, finding that 15.91% had IDH1 mutations and 26.27% had IDH2 mutations.* -
  • Patients with IDH1 and IDH2 mutations experienced lower rates of acute graft-versus-host disease (GVHD) compared to those without mutations, with significant improvements in overall survival (OS) linked to IDH1 mutations.* -
  • IDH2 mutations were associated with a lower relapse rate and better leukemia-free survival (LFS) and OS, particularly in patients without NPM1 mutations, indicating these mutations may lead to better outcomes post
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  • The study focuses on the long-term survival rates of adult patients with congenital heart disease who underwent orthotopic heart transplantation, distinguishing between those with single-ventricle versus biventricular circulation.
  • A total of 61 patients were analyzed, with single-ventricle patients being younger and having more complications like cirrhosis and protein-losing enteropathy compared to biventricular patients.
  • Five-year survival rates were significantly lower for single-ventricle patients (66%) than for biventricular patients (91%), indicating that the type of congenital heart disease affects post-transplant outcomes.
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Objective: To determine the influence of coronary anatomy on long-term outcomes of the arterial switch operation (ASO).

Methods: We retrospectively reviewed patients with transposition of the great arteries or Taussig-Bing anomaly who underwent ASO at our institution between 1992 and 2022. The primary endpoint was freedom from a composite of death, transplantation, and coronary reintervention.

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  • Therapeutic monoclonal antibodies have been effective against SARS-CoV-2 in at-risk groups, but their effectiveness is declining due to new variants.
  • Health authorities are using in vitro tests to guide treatment recommendations, but these tests may not accurately reflect clinical efficacy in real-world situations.
  • A study using hamsters shows that while AZD7442 retains some effectiveness against specific variants like BA.1 and BA.2, its reliability drops significantly against BA.5, underscoring the need for animal studies to enhance understanding of antibody performance in humans.
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There is renewed interest in septation of the double-inlet ventricle as an alternative to Fontan palliation. We examined our septation experience with over 30 years of follow-up. We retrospectively reviewed patients with double-inlet ventricle from 1990 to 2011.

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The photovoice literature has focused mainly on research collaborations between researchers in academia and community partners. There is limited evidence of undergraduate students facilitating photovoice research projects with underserved Black, Indigenous, and persons of color (BIPOC). Students' participation in photovoice research increases their understanding of local issues and can empower them to engage with local communities to create change.

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Ventricular septation of the double-inlet ventricle is a largely abandoned operation due to poor historical outcomes. However, there has been renewed interest in septation as an alternative to Fontan palliation given its long-term sequelae. As one of the few centers to revisit septation in the early 1990s, our institution has long-term data on a series of patients with a double-inlet ventricle who underwent biventricular repair.

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In this SFGM-TC registry study, we report the results after stem cell transplantation (HSCT) in 305 myelofibrosis patients, in order to determine potential risk factors associated with outcomes, especially regarding previous treatment with ruxolitinib. A total of 102 patients were transplanted from an HLA-matched-sibling donor (MSD), and 143 patients received ruxolitinib. In contrast with previous studies, our results showed significantly worse outcomes for ruxolitinib patients regarding overall survival (OS) and non-relapse mortality (NRM), especially in the context of unrelated donors (URD).

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