Articles: disease.
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Vitamin D-dependent rickets type I (VDDR-I) is a rare form of rickets, which is an autosomal recessive disease caused by 1α-hydroxylase enzyme deficiency. However, long-term dental management and microscopic morphology of teeth remain largely unclear. ⋯ This case is the first to confirm amelogenesis imperfecta in a patient with VDDR-I that was not prevented by drug intervention. Importantly, it provides evidence that long-term dental intervention in patients with VDDR-I can result in an acceptable oral hygiene status. Therefore, early and long-term dental intervention is necessary in VDDR-I patients.
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Observational Study
Early achievement of ASDAS clinical response is associated with long-term improvements in metrological outcomes in patients with ankylosing spondylitis treated with TNF-α blockers.
The aim of this study was to investigate the relationship between long-term spinal mobility improvements and early disease activity changes or achievement of clinical response criteria in patients with ankylosing spondylitis (AS) treated with tumor necrosis factor (TNF-α) blockers. This retrospective study included 112 patients with AS treated with TNF-α blockers for up to 33 months. The paired t-test was used to compare outcome measures between visits. ⋯ Responders according to Assessment of Spondylo Arthritis international Society 40 at month 3 showed significant 33-month improvements in BASMI10, but not chest expansion, compared to non-responders. The degree of early changes in disease activity outcomes influenced the extent of long-term metrological improvements in AS treated with TNF-α blockers. Additionally, the achievement of ASDAS- major improvement at month 3 predicted significant metrological improvements throughout long-term TNF-α-blocker therapy.
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Observational Study
IKZF1 polymorphisms are associated with susceptibility, cytokine levels, and clinical features in systemic lupus erythematosus.
Ikaros family zinc finger 1(IKZF1) encodes a lymphoid-restricted zinc finger transcription factor named Ikaros that regulates lymphocyte differentiation and proliferation as well as self-tolerance. Increasing evidence indicates that IKZF1 could contribute to the pathogenesis of autoimmune diseases. Recent research has provided evidence that IKZF1 might correlate with Systemic lupus erythematosus (SLE), but no clear definition has been made yet. ⋯ In addition, significant relationships were observed between rs4917014 and susceptibility, granzyme B level, and hematological disorder in SLE (P = .005, P = .03 and P = .005, respectively). The results further support that IKZF1 may have an important role in the development and pathogenesis of SLE. Allele G of rs4132601 and rs4917014 is related to a decreased risk of SLE occurrence and associated with clinical features in SLE patients, including CD40 ligand level, granzyme B level, malar rash, and hematological disorder, which play important roles in disease progression.
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Observational Study
The impact of the coronavirus disease 2019 pandemic on a central Italy transplant center.
Coronavirus disease 2019 (COVID-19) is challenging health care systems worldwide, raising the question of reducing the transplant program due to the shortage of intensive care unit beds and to the risk of infection in donors and recipients. We report the positive experience of a single Transplant Center in Rome, part of the National Institute for Infectious Diseases Lazzaro Spallanzani, one of the major national centers involved in the COVID-19 emergency.
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The importance of monocyte/lymphocyte ratio (MLR) has been indicated in the initiation and progression of coronary artery disease. However, few previous researches demonstrated the relationship between MLR and plaque vulnerability. We aimed to investigate coronary non-culprit plaque vulnerability in patients with acute coronary syndrome (ACS) by optical coherence tomography (OCT). ⋯ MLR could differentiate TCFA with a sensitivity of 60.0% and a specificity of 85.1%. Circulating MLR level has potential value in identifying the presence of vulnerable plaque in patients with ACS. MLR, as a non- invasive biomarker of inflammation, may be valuable in revealing plaque vulnerability.