scholarly journals Photodermatoses in Childhood

2021 ◽  
Vol 20 (5) ◽  
pp. 360-369
Author(s):  
Nikolay N. Murashkin ◽  
Eduard T. Ambarchian ◽  
Roman V. Epishev ◽  
Alexander I. Materikin ◽  
Leonid A. Opryatin ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Clinical Symptom ◽  
Metabolic Disorders ◽  
Connective Tissue Diseases ◽  
Heterogeneous Group ◽  
Diverse Group ◽  
Skin Hypersensitivity ◽  
Timely Diagnosis ◽  
Laboratory Features

Photodermatoses is a heterogeneous group of diseases resulting from abnormal skin hypersensitivity to sunlight and presented as local or generalized rashes. Specific sensitivity of children's skin to ultraviolet is often the first sign or clinical symptom of photodermatosis. Abnormal photosensitivity can be represented by diverse group of primary idiopathic conditions or photo-mediated aggravation of existing dermatosis. Number of genetic genodermatoses, metabolic disorders and connective tissue diseases is also widely known. These conditions can manifest with photosensitivity associated to other extracutaneous clinical and laboratory features. Timely diagnosis of photosensitivity in childhood allows to minimize long-term complications associated with insufficient photoprotection.

scholarly journals Hereditary Connective Tissue Diseases in Young Adult Stroke: A Comprehensive Synthesis

2011 ◽  
Vol 2011 ◽  
pp. 1-18 ◽  
Author(s):  
Olivier M. Vanakker ◽  
Dimitri Hemelsoet ◽  
Anne De Paepe
Keyword(s):  
Connective Tissue ◽  
Metabolic Disorders ◽  
Type Iv Collagen ◽  
Connective Tissue Diseases ◽  
Haemorrhagic Stroke ◽  
Monogenic Disease ◽  
Connective Tissue Disorders ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Though the genetic background of ischaemic and haemorrhagic stroke is often polygenetic or multifactorial, it can in some cases result from a monogenic disease, particularly in young adults. Besides arteriopathies and metabolic disorders, several connective tissue diseases can present with stroke. While some of these diseases have been recognized for decades as causes of stroke, such as the vascular Ehlers-Danlos syndrome, others only recently came to attention as being involved in stroke pathogenesis, such as those related to Type IV collagen. This paper discusses each of these connective tissue disorders and their relation with stroke briefly, emphasizing the main clinical features which can lead to their diagnosis.

Autoimmune connective tissue diseases

2013 ◽  
Vol 6 (3) ◽  
pp. 159-171
Author(s):  
Ishita Patel ◽  
Alia Ahmed
Keyword(s):  
Primary Care ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
The Body ◽  
Diverse Group ◽  
Connective Tissues

Connective tissue diseases are a rare and diverse group of disorders that result in pathology of the connective tissues of the body. This article focuses on the systemic autoimmune connective tissue diseases, and aims to provide a practical overview of these conditions for use in primary care.

Vasculitis

2018 ◽  
Author(s):  
Raashid Luqmani
Keyword(s):  
Connective Tissue ◽  
Blood Vessel ◽  
Blood Vessels ◽  
Vessel Wall ◽  
Connective Tissue Diseases ◽  
Blood Vessel Wall ◽  
Target Organs ◽  
Laboratory Features ◽  
Life Threatening ◽  
Pulmonary Capillaritis

The vasculitides are a heterogeneous group of disorders that can range from mild inflammation of blood vessels in the skin, to organ- and life-threatening diseases. The term ‘vasculitis’ is a pathological description of blood vessel wall inflammation which leads to ischaemia and infarction of the target organs. Definitions and classifications of the primary vasculitides are mainly based on the predominant calibre of the blood vessels involved but incorporate clinical, pathological, and laboratory features. The secondary vasculitides usually occur in the context of other connective tissue diseases and are not discussed further in this section. Goodpasture’s disease is not usually included in the primary vasculitides, but has compatible clinical features of pulmonary capillaritis and glomerulonephritis.

scholarly journals Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases

1994 ◽  
Vol 37 (10) ◽  
pp. 1528-1533 ◽  
Author(s):  
Jose De La Mata ◽  
Miguel A. Gomez-Sanchez ◽  
Mercedes Aranzana ◽  
Juan J. Gomez-Reino
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Infusion Therapy ◽  
Severe Pulmonary Hypertension ◽  
Iloprost Infusion

scholarly journals Chemerin, Resistin, and Adiponectin in Patients with Connective Tissue Diseases

2018 ◽  
Vol 37 (2) ◽  
pp. 148-154 ◽  
Author(s):  
Bogna Grygiel-Górniak ◽  
Teresa Grzelak ◽  
Krystyna Czyźewska ◽  
Mariusz Puszczewicz
Keyword(s):  
Connective Tissue ◽  
Roc Curve ◽  
Metabolic Disorders ◽  
Connective Tissue Diseases ◽  
Area Under The Curve ◽  
Control Group ◽  
Reactive Protein ◽  
State Present ◽  
Inflammatory State ◽  
Wbc Count

SummaryBackground:The exact role of cytokines in inflammation and metabolic disorders in case of connective tissue diseases (CTDs) is under discussion.Methods:In this study, we intended to find the relationship between the selected cytokines in inflammatory and metabolic disorders in patients with CTDs (n=55) and compared the results with those of control group subjects (n=25) matched by age and body mass. We estimated their nutritional status by the bioimpedance method. The levels of basic biochemical parameters and the levels of adiponectin, resistin, and chemerin were also estimated. Multiple regressions and area under the curve in receiver operating characteristic (AUC–ROC) curve were used to find the associations of aforementioned parameters.Results:Patients with CTDs exhibited higher levels of chemerin than that of control group subjects. We found an inverse relationship between chemerin, RBC count, and hemoglobin levels. The concentration of adiponectin inversely correlated with the levels of platelets and concentrations of glucose and triglycerides as well as the erythrocyte sedimentation rate, whereas the concentration of resistin was positively correlated with WBC count, C-reactive protein (CRP), and the amount of used oral glucocorticosteroids. The mean ± standard deviation for the AUC–ROC curve in case of chemerin was the highest (AUC–ROC=0.714,p=0.0005) than that of both resistin and adiponectin.Conclusions:Chemerin and resistin levels are related to the inflammatory state in patients with CTDs, whereas adiponectin levels seem to be correlated with a protective effect. Chemerin can be considered as a marker differentiating a proinflammatory state present in CTDs.

scholarly journals Pauci-Immune Crescentic Glomerulonephritis in Connective Tissue Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-6
Author(s):  
Supraja Yeturi ◽  
Mary Cronin ◽  
Robin Adam ◽  
Campbell Lorna ◽  
Ann K. Rosenthal
Keyword(s):  
Renal Transplant ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Crescentic Glomerulonephritis ◽  
Connective Tissue Diseases ◽  
Small Population ◽  
Rare Entity ◽  
Separate Entity ◽  
Anca Associated Vasculitis ◽  
Laboratory Features

Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren’s syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus. In this small population of patients there is no correlation with ANCAs. Most of the patients were treated with aggressive immunosuppression and did well if they were treated early in the course of their disease. One of our patients required renal transplant, but she presented late in the course of her disease, as evidenced by chronicity on her renal biopsy. Whether these patients are overlap of vasculitis and other connective tissue diseases or to be considered as a separate entity is yet to be described. Clinicians must be aware of these presentations because initial presentation can be severe.

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