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Summary - Restrictive Dermopathy

The text is the summary of recent articles on Restrictive Dermopathy from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.

Racial differences exist in the severity of systemic sclerosis (SSc). A 4-year-old girl was referred to the Undiagnosed Diseases Network with a history of short stature, thin and translucent skin, macrocephaly, small hands, and camptodactyly. Preterm birth is the world's leading cause of neonatal death. Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is characterized by multiple symptoms and comorbidities. To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc).A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. Earlier life stressors can increase the risk of persistent anxiety and depressive symptoms in women after cancer, though our understanding of the underlying mechanisms is limited. Although uncommon, kwashiorkor continues to occur in developed, but mainly in developing nations. Fetal akinesia deformation sequence is a clinically and genetically heterogeneous disorder characterized by a variable combination of fetal akinesia, intrauterine growth restriction, developmental abnormalities such as cystic hygroma, hydrops fetalis, pulmonary hypoplasia, occasional arthrogryposis, and pterygia. The objective of this study is to assess the association of clinical manifestations of systemic sclerosis (SSc) with the absolute count of circulating blood monocyte subpopulations according to their membrane expression of CD16. The heart may be affected directly or indirectly by a variety of protozoa and helminths. Infection of the human cornea by herpes simplex virus type-1 (HSV-1) can cause significant vision loss. Despite the importance of traditional pulmonary function tests (PFTs) in managing systemic sclerosis (SSc), many patients with pulmonary disease diagnosed by computed tomography (CT) present with normal PFTs.To evaluate the efficacy of the nitrogen single-breath washout (NCross-sectional study in which 52 consecutive SSc patients were subjected to spirometry, body plethysmography, analysis of the diffusing capacity for carbon monoxide (DLCO), analysis of respiratory muscle strength, NTwenty-eight patients had a forced vital capacity (FVC) that was <70% of the predicted value. Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with anti-RNA polymerase III (anti-RNAP III) autoantibodies as well as in patients who are triple-negative for anticentromere (anti-CENP), anti-topoisomerase I (anti-topo I), and anti-RNAP III (also known as anti-POL) autoantibodies (referred to as CTP negative). The arachidonate 5-lipoxygenase activating protein (ALOX5AP) regulates synthesis of leukotrienes (LTs), which are important mediators of inflammation and connective tissue remodelling. Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. Patients with systemic sclerosis (SSc) experience severe physical limitations and psychological morbidity, but their lived experience remains underrepresented and is reflected in the scarcity of evidence-based patient-centered interventions. Patients with progeroid syndromes such as mandibuloacral dysplasia, type B (MADB) and restrictive dermopathy (RD) harbor mutations in zinc metalloproteinase (ZMPSTE24), an enzyme essential for posttranslational proteolysis of prelamin A to form mature lamin A. Dermal fibroblasts from these patients show increased nuclear dysmorphology and reduced proliferation; however, the efficacy of various pharmacological agents in reversing these cellular phenotypes remains unknown. Previous concordance studies examining accuracy of breast diagnosis by pathologists, typically targeting difficult, histologically challenging breast lesions using artificial and restrictive environments, have reported aberrantly high levels of diagnostic discordance. In 2000, Galaria et al. proposed the designation neutrophilic dermatosis of the dorsal hands (NDDH).

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