Publications by authors named "D S Peck"

Purpose: This prospective, longitudinal study was designed to determine the natural history of Fabry disease (FD) in early pediatric patients across the disease spectrum.

Methods: In this observational study of children under 5 years of age with variants in the  gene, prospective phenotypic and urinary biomarker data were collected annually over 5 years.

Results: The study population included 40 participants (35 male, 5 female) with variants including 15 with classic pathogenic variants (CFD), 6 with nonclassic pathogenic variants (NFD), and 19 with a variant of uncertain significance.

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  • - The text discusses a rare complication called pseudoaneurysm that can occur in the right ventricular outflow tract (RVOT) after cardiac surgeries, particularly in a 3-month-old infant with tetralogy of Fallot and pulmonary atresia.
  • - The patient was critically ill with septic shock and very high pressure in the right ventricle, requiring urgent treatment to avoid high-risk surgery.
  • - The successful management involved using a Gore VBX balloon expandable covered stent to exclude the pseudoaneurysm and stenting the pulmonary arteries to relieve pressure, highlighting the importance of recognizing this condition and available treatment options.
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  • Endomyocardial biopsy is the standard method for monitoring heart transplant rejection, but this study explored a non-invasive index for assessing left ventricular relaxation in pediatric patients.
  • The researchers analyzed data from 65 pediatric heart transplant patients who underwent 246 procedures to compare the new left ventricular relaxation index with traditional biopsy results for detecting cellular rejection.
  • The findings showed that while the index had good sensitivity (100%) for identifying higher grades of rejection (2R and 3R), it lacked specificity (23%), indicating it may not be a reliable predictor of cardiac rejection in these patients.
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  • Blackwater rivers and streams have tea-colored water due to tannins from decaying plants, and these waters typically have lower pH and oxygen levels compared to non-blackwater systems.
  • While blackwaters are most common in the eastern U.S., differences in water chemistry were found between blackwater and non-blackwater sites, but these differences varied by ecoregions, making it hard to generalize nationally.
  • The study suggests the need for improved understanding and management of blackwaters through better definitions, identification of stressors, and consistent assessment methods to protect their unique ecological conditions.
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