8 research outputs found
Whose Land Is It Anyway? Navigating Ghana\u27s Complex Land System
This Article dives into Ghana’s complex land-registration system, which is influenced by both statutory and customary law. Section II discusses Ghana’s statutory land laws. Section III provides a brief overview of Ghana’s customary land laws. Section IV discusses several obstacles within Ghana’s land-administration system
Newborn Screening for X-Linked Adrenoleukodystrophy: The Initial Illinois Experience
X-linked adrenoleukodystrophy (X-ALD) is a genetic neurodegenerative disorder with an approximate incidence of 1 in 14,700 births. Both males and females are affected. Approximately one-third of affected males develop childhood cerebral adrenoleukodystrophy, which progresses rapidly to severe disability and death. In these cases, early surveillance and treatment can be lifesaving, but only if initiated before the onset of neurologic symptoms. Therefore, X-ALD was added to the Recommended Uniform Screening Panel. We report outcomes of the initial screening of approximately 276,000 newborns in Illinois. The lipid C26:0 lysophosphatidylcholine (C26:0-LPC) was measured in dried blood spots (DBS) using liquid chromatography with tandem mass spectrometry. Results ≥ 0.28 µmol/L were considered screen positive. Of 18 screen positive results detected, 12 cases were confirmed. Results were reported as borderline if initial and repeat analyses were ≥0.18 and <0.28 µmol/L. Of the 73 borderline screen results, 57 were normal after analysis of a second sample. Five X-ALD cases were identified from borderline screens. Newborn screening of X-ALD was successfully implemented in Illinois, and results were comparable to reports from other states. Early identification of infants with this potentially life-threatening disorder will significantly improve outcomes for these children
Prevalence and predictors of cancer-related worry and associations with health behaviors in adult survivors of childhood cancer
BACKGROUND: Cancer-related worry (CRW) is common among cancer survivors; however, little is known about factors associated with CRW or its impact on health behaviors in adult survivors of childhood cancer. METHODS: Survivors in the St. Jude Lifetime Cohort Study (n=3211; 51% male; mean[SD] age 31.2[8.4] years; 22.8[8.3] years post-diagnosis) underwent medical evaluations and completed ratings of CRW, psychological symptoms, and health behaviors. Multivariable modified Poisson regression models examined associations between CRW and treatment exposures, chronic health conditions, psychological symptoms, and health behaviors. RESULTS: Sixty-four percent, 95% CI [61.5, 64.8], of survivors reported worry about subsequent malignancy, 45% [43.0, 46.4] reported worry about physical problems related to cancer, and 33% [30.8, 34.1] reported worry about relapse. Multiple psychological symptoms, treatment exposures, and chronic conditions each significantly increased the risk of CRW. Survivors reporting CRW were at increased risk for substance use, inadequate physical activity, and increased health care utilization, after adjusting for chronic conditions. For example, when adjusting for chronic conditions, those who endorsed CRW were more likely to have ≥5 cancer-related physician visits, ≥5 physician visits related to cancer, and ≥5 calls to a physician’s office in the previous 2 years compared to survivors who were not worried. CRW was also associated with increased risk of current tobacco use, past marijuana use, and current marijuana use. CONCLUSIONS: A substantial proportion of adult survivors of childhood cancer reported CRW that is associated with increased health care utilization. CRW may serve as an intervention target to promote well-being and adaptive health behaviors
Identification of redundancy between human Fc epsilon RI beta and MS4A6A proteins points toward additional complex mechanisms for Fc epsilon RI trafficking and signaling
Background: Allergic diseases are triggered by signaling through the high-affinity IgE receptor, FcεRI. In both mast cells (MCs) and basophils, FcεRI is a tetrameric receptor complex comprising a ligand-binding α subunit (FcεRIα), a tetraspan β subunit (FcεRIβ, MS4A2) responsible for trafficking and signal amplification, and a signal transducing dimer of single transmembrane γ subunits (FcεRIγ). However, FcεRI also exists as presumed trimeric complexes that lack FcεRIβ and are expressed on several cell types outside the MC and basophil lineages. Despite known differences between humans and mice in the presence of the trimeric FcεRI complex, questions remain as to how it traffics and whether it signals in the absence of FcεRIβ. We have previously reported that targeting FcεRIβ with exon-skipping oligonucleotides eliminates IgE-mediated degranulation in mouse MCs, but equivalent targeting in human MCs was not effective at reducing degranulation. Results: Here, we report that the FcεRIβ-like protein MS4A6A exists in human MCs and compensates for FcεRIβ in FcεRI trafficking and signaling. Human MS4A6A promotes surface expression of FcεRI complexes and facilitates degranulation. MS4A6A and FcεRIβ are encoded by highly related genes within the MS4A gene family that cluster within the human gene loci 11q12-q13, a region linked to allergy and asthma susceptibility. Conclusions: Our data suggest the presence of either FcεRIβ or MS4A6A is sufficient for degranulation, indicating that MS4A6A could be an elusive FcεRIβ-like protein in human MCs that performs compensatory functions in allergic disease