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Papillon Lefevre Disease PubMed articles on BioPortfolio. Our PubMed references draw on over 21 million records from the medical literature. Here you can see the latest Papillon Lefevre Disease articles that have been published worldwide.
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Papillon-Lefèvre syndrome (PLS) is characterized by nonfunctional neutrophil serine proteases (NSPs) and fulminant periodontal inflammation of unknown cause. Here we investigated neutrophil extracellular trap (NET)-associated aggregation and cytokine/chemokine-release/degradation by normal and NSP-deficient human and mouse granulocytes. Stimulated with solid or soluble NET inducers, normal neutrophils formed aggregates and both released and degraded cytokines/chemokines. With increasing cell density, prote...
Given ocean warming, the GOLT predicts a reduction of the size of fish due to their inability to compensate, via their gill surface, for the increased metabolic rate that results from higher temperatures (Cheung et al., 2013). Lefevre et al. (2017, 2018) objected to this because they believe that fish can grow gills as needed, such that oxygen supply cannot be limiting their metabolism. Pauly & Cheung (2017) responded to this claim, refuted its various elements, and reiterated the basic dimensional argume...
Behçet's disease is a multi-system disease with inflammatory infestations, in particular of the small and middle arteries and veins. Histologically, the disease presents with the image of leukocytoclastic vasculitis. The etiology of Behçet disease is unknown, but genetic factors are discussed of playing a role in its pathogenesis. There is no known autoantibody, which could serve as a diagnostic tool. The disease usually affects patients in the 3. life decade and is rare in pediatrics. A very rare clinica...
Inflammatory bowel disease (IBD) is characterized by uneven disease courses with various clinical outcomes. A few prognostic markers of disease severity may help stratify patients and identify those who will benefit the most from early aggressive treatment. The concept of disease severity remains too broad and vague, mainly because the definition must embrace several disease mechanisms, mainly inflammation and fibrosis, with various rates of disease progression. The magnitude of inflammation is an obvious k...
Huntington's disease is a rare, neurodegenerative disease caused by an expanded CAG repeat mutation in the huntingtin gene. Compared with adult-onset Huntington's disease, juvenile Huntington's disease (onset ≤20 years) is even rarer and has not been studied extensively. We aimed to further characterise juvenile Huntington's disease by examining the effect of CAG repeat size on disease presentation, progression, and survival.
Behçet's disease is an autoimmune disease with diverse clinical manifestations with vasculitis being the hallmark of the disease. The aim of this work is to study the genetic association between human leukocyte antigen (HLA) class-I molecules of Egyptians with Behçet's disease and the disease susceptibility and clinical patterns.
Kawasaki disease presents many challenges to the diverse group of physicians who care for these patients including infectious disease specialists, rheumatologists, and cardiologists. Here we review some of the progress being made toward improved understanding of disease pathogenesis, treatment, and long-term outcomes.
Venous disease is more common than peripheral arterial disease. Pathophysiologically, venous disease can be associated with obstruction, reflux, or both. A common feature in chronic venous disease is ambulatory venous hypertension. Inflammatory and pro-thrombotic mechanisms can be activated. The current therapies, including compression, ablation, and recanalization are discussed.
Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease.
Visual hallucinations (VHs) are common in Parkinson's disease (PD), with prevalence ranging from 27-50% in cross-sectional cohorts of patients with well-established disease. However, minor hallucinations may occur earlier in the disease process than has been previously reported.
Powassan virus (POWV) is a tick-borne flavivirus that causes rare, but often severe, disease in humans. POWV neuroinvasive disease was added to the U.S. nationally notifiable disease list in 2001 and nonneuroinvasive disease was added in 2004. The only previous review of the epidemiology of POWV disease in the United States based on cases reported to the Centers for Disease Control and Prevention (CDC) covered the period from 1999 through 2005.
Crohn's disease (CD) patients who undergo ileocolonic resection frequently have disease recurrence. The aim of this preliminary study was to identify urinary metabolomic profiles associated with disease recurrence in order to identify underlying mechanisms of recurrence and possible disease biomarkers.
Neuroimaging in Parkinson's disease is an evolving field, providing in-vivo insights into the structural and biochemical changes of the condition, although its diagnosis remains clinical. Here, we aim to summarize the most relevant recent advances in neuroimaging in Parkinson's disease to assess the underlying disease process, identify a biomarker of disease progression and guide or monitor therapeutic interventions.
Behçet's disease is a systemic inflammatory disease that may affect multiple organs. However, intraabdominal complications requiring surgical intervention are rare in the natural course of the disease. A 32-year-old male patient with Behçet's disease who had been followed for 5 years with a diagnosis of acute abdominal syndrome was operated on twice in 18 days. Intestinal ischemia was identified in different segments of the jejenum during each operation. Recurrent segmental intestinal ischemia within a sh...
Peripheral arterial disease (PAD) is the third most common manifestation of cardiovascular disease (CVD), following coronary artery disease (CAD) and stroke. PAD remains underdiagnosed and under-treated in women.
Extramammary Peget disease (EMPD) is a rare tumor, which typically occurs in the perianal regions. Perianal Paget disease (PPD) was first reported in 1893, and which has only 180 cases that have been reported in literature. The rarity of the disease means that no large studies have been made, and so the optimal treatment for this disease still remains controversial.
Crohn's disease is a chronic inflammatory bowel disease that requires lifelong multidisciplinary management. Seventy percent of patients affected by Crohn's disease will require at least one surgical procedure over their lifetime. The aim of this retrospective study was to present our series of patients suffering from Crohn's disease who were scheduled for surgery by a multidisciplinary team.
There is evidence that Alzheimer's disease (AD) has significant cerebrovascular etiopathogenesis. Understanding potentially modifiable risk factors for vascular disease can help design long-term intervention strategies for controlling or preventing cognitive dysfunction attributable to cerebrovascular disease.
Thyroid-associated orbitopathy (TAO) is the most common autoimmune disease of the orbit. It occurs more often in patients presenting with hyperthyroidism, characteristic of Graves' disease, but may be associated with hypothyroidism or euthyroidism. The diagnosis of TAO is based on clinical orbital features, radiological criteria, and the potential association with thyroid disease. To date, there is no specific marker of the orbital disease, making the early diagnosis difficult, especially if the orbital inv...
Mild cognitive impairment is a common feature of Parkinson's disease, even at the earliest disease stages, but there is variation in the nature and severity of cognitive involvement and in the risk of conversion to Parkinson's disease dementia. This review aims to summarise current understanding of mild cognitive impairment in Parkinson's disease. We consider the presentation, rate of conversion to dementia, underlying pathophysiology and potential biomarkers of mild cognitive impairment in Parkinson's dise...
Alzheimer's disease is a multifactorial disorder for which there is no disease-modifying treatment yet. CB2 receptors have emerged as a promising therapeutic target for Alzheimer's disease because they are expressed in neuronal and glial cells and their activation has no psychoactive effects.
The purposes of this study were to discern imaging findings that distinguish Behçet disease from small-bowel Crohn disease, to find initial performance estimates for these findings, and to evaluate the diagnostic value of MR enterography (MRE) for detecting intestinal Behçet disease.
Behçet's disease is a rare disorder of unknown etiology that is classified as a systemic vasculitis. The prevalence of the disease is high in countries in the Far East, Mediterranean Basin, and East Asia. Thus, it is also known as the Silk Road Disease. Behçet's disease is characterized by recurrent oral aphthous ulcers, genital sores, and ocular lesions. However, it can present with severe clinical manifestations as a result of cardiovascular system, central nervous system, and gastrointestinal tract inv...
Treat-to-target strategies have improved outcomes in rheumatic diseases. In psoriatic arthritis (PsA), the proposed targets are the multidimensional target Minimal Disease Activity (MDA) and the articular target Disease Activity index for PsA (DAPSA). We aimed to compare burden of PsA in patients with low disease activity according to the two definitions MDA and DAPSA-Low Disease Activity (DAPSA-LDA), one year after diagnosis.