scholarly journals Epidemiologi ved sklerodermi hos barn

2009 ◽  
Vol 18 (1) ◽  
Author(s):  
Odd Vinje ◽  
Berit Flatø

<li>Localized scleroderma is an infrequent disease in children, but still ten times more frequent than systemic sclerosis. The incidence is probably 5-10/100,000 per year. The disease is classified into five subgroups. Paediatric rheumatologists report linear scleroderma as the most frequent subgroup whereas plaque morphea is found most frequently by dermatologists, linear scleroderma being in second place. The risk of transforming into systemic sclerosis is minimal. The mortality risk is not found increased compared with the normal population. Systemic sclerosis is the most infrequent systemic connective tissue disease in children, the incidence is less than 1/1,000,000 per year. The outcome in children is considered to be generally better than in adults. However, deaths is caused most frequently by heart failure with or without pulmonal hypertension, but also by renal or respiratory insuffiency, CNS disease and sepsis. Cumulative survival after 5, 10, 15 and 20 years of disease is reported to be 89%, 80%, 74% and 69%. For the age group less than 16 years the median age of death is reported to be 10.4 years, range 5 to 15 years. The disease in the children who die is often rapidly progressing with early involvement of internal organs. </li>


2021 ◽  
Vol 97 (3) ◽  
pp. 129-143
Author(s):  
Gábor Bali ◽  
◽  
Bernadett Hidvégi ◽  
Miklós Sárdy

Systemic sclerosis is a collagen vascular disease which could lead to fibrosis of the skin and internal organs. It is characterized by a high disease burden and mortality. The 2013 classification criteria system helps to recognize the disease early, which allows prompt intervention and a better survival. In the past few year the recognition of the association between the presence of RNA polimerase III antibodies and malignancies was important. Treatment options of SSc have been improved. Early, intensive immunosuppressive treatment (cyclophosphamide, mycophenolate mofetil) of interstitial lung disease inhibits progression. There are several treatment modalities for the treatment of pulmonary arterial hypertension and digital ulcers (endothelin receptor antagonists, phosphodiesterase 5 inhibitors, prostaglandin analogues). Antifibrotic drugs (nintedanib, riociguate) are also available among former immunosuppressive and vasoactive agents. Lenabasum is a new promising selective cannabinoid receptor 2 agonist, which inhibits inflammation and fibrosis. Localized scleroderma presents a diverse clinical picture. The disease leads to fibrosis of the skin but it is not accompanied with serious inner organ involvement typically seen in systemic sclerosis. Capillaroscopy abnormalities, sclerodactyly and SSc specific antibodies are absent in localized scleroderma . The 2017 EDF guideline formulates a very useful classification, checkup and treatment recommendations.



2015 ◽  
Vol 34 (2) ◽  
pp. 95-102 ◽  
Author(s):  
Francisca Caetano ◽  
Paula Mota ◽  
Inês Almeida ◽  
Andreia Fernandes ◽  
Ana Botelho ◽  
...  


1979 ◽  
Vol 22 (2) ◽  
pp. 130-140 ◽  
Author(s):  
Gerald P. Rodnan ◽  
Esther Lipinski ◽  
Joan Luksick




1994 ◽  
Vol 35 (4) ◽  
pp. 329-334 ◽  
Author(s):  
M. Yamamoto ◽  
Y. Iimuro ◽  
M. Mogaki ◽  
K. Kachi ◽  
H. Fujii ◽  
...  

In trying to clarify the high recurrence rate after removal of small hepatocellular carcinoma (HCC), we assessed the postoperative evolution of minute hepatic Lipiodol deposits which had been diagnosed as artifacts on the preoperative Lipiodol-CT. Of 27 patients with solitary HCC less than 5 cm in diameter, 14 had such Lipiodol deposits in the preoperative CT and 9 of them (64%) developed recurrent tumors. On the other hand, 6 of the 13 patients without deposits (46%) suffered recurrence, but in 5 of these 6 patients the HCC was metachronous multicentric. The cumulative survival rate of the non-deposit group was better than that of the deposit group (p < 0.1). The present study suggested that, even in patients with small HCC, minute concomitant tumors invisible by conventional imaging techniques may exist at the time of surgery. Some of these lesions without sufficient tumor vasculature showing a hypervascular blush on angiography appear to retain small, vague Lipiodol deposits.



2018 ◽  
Vol 98 (3) ◽  
pp. 361-365 ◽  
Author(s):  
J Hesselvig ◽  
K Kofoed ◽  
J Wu ◽  
L Dreyer ◽  
G Gislason ◽  
...  


2014 ◽  
Vol 48 (spe) ◽  
pp. 137-144
Author(s):  
Renata Laszlo Torres ◽  
Suely Itsuko Ciosak

Objective To describe the profile of Hospitalizations by Amulatory Care Sensitive Conditions (HACSC), in the Municipality of Cotia, from 2008 to 2012. Method ecological, exploratory, longitudinal study with a quantitative approach. Data on HACSC, by age group and sex, were obtained from the Department of the Unified Health System. For data analysis descriptive statistics were used. Results During the period, there were 46,676 admissions, excluding deliveries, 7,753 (16.61%) by HACSC. The main causes were cerebrovascular diseases, 16.96%, heart failure, 15.50%, hypertension, 10.80% and infection of the kidney and urinary tract, 10.51%. Regarding gender, HACSC occurred predominantly in males. There was a greater number of HACSC at extreme age ranges, especially in the elderly. Conclusion Chronic diseases predominate among the leading causes of HACSC and there was no significant difference between sex.







2017 ◽  
Vol 7 (2) ◽  
pp. 188-194 ◽  
Author(s):  
Yasuhiro Nagahama ◽  
Tomoko Okina ◽  
Norio Suzuki

Background/Aims: To examine the influence of age on neuropsychological performances in dementia with Lewy bodies (DLB) and Alzheimer disease (AD) patients. Methods: We examined memory, executive, and visuo-constructional performances in 202 DLB patients and 236 AD patients. We divided the subjects into three age groups (65–74, 75–84, and 85–95 years old), and evaluated the differences in neuropsychological performances. Results: Recent memory in the DLB group was significantly better than that in the age-matched AD group when comparing the age groups 65–74 years and 75–84 years; however, memory impairment in the DLB patients in the age group 85–95 years was comparable with that in the age-matched AD patients. In contrast to recent memory, the other assessed neuropsychological performances, such as visuospatial and executive functions, showed no significant change in differences between the DLB and AD groups with advancing age. Conclusion: Our study revealed that the nature of memory impairment in DLB patients changes according to age. DLB patients in the young-old and old-old age groups showed significantly better memory performance than the age-matched AD patients, whereas memory performance of the DLB patients in the oldest-old age group was similar to that of the age-matched AD patients. This may be associated with the increased rate of coexisting AD pathology in DLB patients with older age.



2018 ◽  
Vol 28 (10) ◽  
pp. 106312 ◽  
Author(s):  
Philine Granitza ◽  
Jan F. Kraemer ◽  
Christoph Schoebel ◽  
Thomas Penzel ◽  
Jürgen Kurths ◽  
...  


2019 ◽  
Vol 12 (9) ◽  
pp. 1491-1498 ◽  
Author(s):  
Yu Vatnikov ◽  
A. Rudenko ◽  
P. Rudenko ◽  
Ev Kulikov ◽  
A. Karamyan ◽  
...  

Background: Dilated cardiomyopathy is common in dogs. This form of cardiomyopathy is the main cause of death due to heart disease in dogs. Death can occur suddenly in clinically normal animals as a result of the progression of congestive heart failure (CHF). The pathogenesis of heart failure syndrome in dogs with dilated cardiomyopathy involves activation of the neurohumoral system and immune-mediated inflammation, which leads to further progression of the condition. Heart failure syndrome in dogs with dilated cardiomyopathy is caused by the progressive loss of cardiomyocytes, apoptosis, remodeling of the left ventricle, systolic and diastolic dysfunction, arrhythmias, reduced cerebral blood flow, the involvement of other key internal organs, and intestinal dysbiosis. Aim: This study aimed to determine the immunological and inflammatory mechanisms surrounding the development of heart failure syndrome in dogs with dilated cardiomyopathy. Materials and Methods: The subjects of this study were dogs with a dilated form of cardiomyopathy (n=159), complicated by various functional classes of heart failure syndrome. Evaluation of myocardial remodeling, systolic function, and systemic hemodynamics was performed using EMP-860 Vet and PU-2200V ultrasound scanners according to the standard technique. Electrocardiography was performed with all dogs in right lateral recumbency using the EK1T-04 Midas electrocardiograph (50 mm/s speed and 1 mV gain = 1 cm). Results: In some affected animals, especially in cases of compensated dilated cardiomyopathy, leukocytosis was noted. In patients with dilated cardiomyopathy complicated by heart failure syndrome of various functional classes, the number of neutrophils was significantly increased, and the number of lymphocytes was decreased by 1.9-2.1 times when compared with those in clinically normal animals. In dogs with dilated cardiomyopathy, neutrophilic leukocytosis develops with a simple regenerative shift to the left. The results of immunological studies indicate that dogs with dilated cardiomyopathy develop T lymphocytopenia as compared with clinically normal animals. Conclusion: The central component of heart failure syndrome in dogs with dilated cardiomyopathy is the activation of the neurohumoral system and immune-mediated inflammation. The development of CHF in dogs with dilated cardiomyopathy is caused by the progressive loss of cardiomyocytes, apoptosis, remodeling of the left ventricle, systolic and diastolic dysfunction, arrhythmias, reduced cerebral blood flow, involvement of other key internal organs, and intestinal dysbiosis.



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