Publications by authors named "M A Merideth"

Article Synopsis
  • Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder that impacts the development of lysosome-related organelles, leading to varied clinical symptoms based on its genetic type, with HPS-1 and HPS-4 being more severe compared to HPS-3, HPS-5, and HPS-6.
  • This study aimed to improve understanding of kidney issues associated with HPS, using medical records and renal function tests from patients evaluated at the National Institutes of Health from 1995 to 2020.
  • Results showed that adults with HPS-1 and HPS-4 had significantly lower kidney function compared to those with HPS-3, HPS-5, or HPS-6
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Article Synopsis
  • - Patients with GATA2 haploinsufficiency face higher risks for HPV-related anogenital cancers, with the study showing that HPV appeared at a median age of 18.9 in females and 25.6 in males, often leading to significant health issues.
  • - The research conducted a thorough review of 68 patients over 17 years, revealing that 76% had HPV-related squamous intraepithelial lesions (SIL), with females showing a higher prevalence and severity of high-grade lesions compared to males.
  • - Among patients receiving hematopoietic stem cell transplants for GATA2-linked diseases, some still experienced persistent HPV issues post-treatment; however, those who received vaccinations afterward showed minimal evidence
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BACKGROUNDWarts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a primary immunodeficiency disorder caused by heterozygous gain-of-function CXCR4 mutations. Myelokathexis is a kind of neutropenia caused by neutrophil retention in bone marrow and in WHIM syndrome is associated with lymphopenia and monocytopenia. The CXCR4 antagonist plerixafor mobilizes leukocytes to the blood; however, its safety and efficacy in WHIM syndrome are undefined.

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Candida albicans causes debilitating, often azole-resistant, infections in patients with chronic mucocutaneous candidiasis (CMC). Amphotericin B (AMB) resistance is rare, but AMB use is limited by parenteral administration and nephrotoxicity. In this study, we evaluated cochleated AMB (CAMB), a new oral AMB formulation, in mouse models of oropharyngeal candidiasis (OPC) and vulvovaginal candidiasis (VVC) and in patients with azole-resistant CMC.

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Pulmonary fibrosis is characterized by abnormal interstitial extracellular matrix and cellular accumulations. Methods quantifying fibrosis severity in lung histopathology samples are semi-quantitative, subjective, and analyze only portions of sections. We sought to determine whether automated computerized imaging analysis shown to continuously measure fibrosis in mice could also be applied in human samples.

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