WorldWideScience

Sample records for juvenile localized scleroderma

  1. BILATERAL CHOROIDAL EXCAVATION IN JUVENILE LOCALIZED SCLERODERMA.

    Science.gov (United States)

    Franklin, Mackenzie L; Day, Shelley

    2018-01-01

    To describe a case of bilateral choroidal excavation in a patient with juvenile localized scleroderma. Case report. An asymptomatic 12-year-old boy with localized scleroderma presented for examination and was found to have bilateral areas of choroidal excavation temporal to the fovea. Previous reports of ocular complications of localized scleroderma have primarily described adnexal and anterior segment changes. This is the second report of choroidal changes in a patient with localized scleroderma, and the first in a pediatric patient.

  2. Musculoskeletal MRI findings of juvenile localized scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Eutsler, Eric P. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Wilmington, DE (United States); Washington University School of Medicine, Mallinckrodt Institute of Radiology, St. Louis, MO (United States); Horton, Daniel B. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Division of Rheumatology, Department of Pediatrics, Wilmington, DE (United States); Rutgers Robert Wood Johnson Medical School, Department of Pediatrics, New Brunswick, NJ (United States); Epelman, Monica [Nemours Children' s Health System/Nemours Children' s Hospital, Department of Medical Imaging, Orlando, FL (United States); Finkel, Terri [Nemours Children' s Health System/Nemours Children' s Hospital, Department of Pediatrics, Orlando, FL (United States); Averill, Lauren W. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Wilmington, DE (United States)

    2017-04-15

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol. (orig.)

  3. Musculoskeletal MRI findings of juvenile localized scleroderma

    International Nuclear Information System (INIS)

    Eutsler, Eric P.; Horton, Daniel B.; Epelman, Monica; Finkel, Terri; Averill, Lauren W.

    2017-01-01

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol. (orig.)

  4. Musculoskeletal MRI findings of juvenile localized scleroderma.

    Science.gov (United States)

    Eutsler, Eric P; Horton, Daniel B; Epelman, Monica; Finkel, Terri; Averill, Lauren W

    2017-04-01

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol.

  5. [Clinical characteristics of patients with juvenile localized scleroderma].

    Science.gov (United States)

    Sun, Qiu-Ning; Du, Wei; Hu, Bin; Liu, Pai; Yuan, Xie

    2009-02-01

    To investigate the clinical characteristics of juvenile localized scleroderma (JLS). The clinical data of 100 outpatients with JLS who were admitted to PUMC Hospital from 2000 to 2008 were retrospectively analyzed. Of a total of 100 cases, 51 (51%) were confirmed as linear scleroderma, 26 (26%) as plaque morphea, 26 (26%) as deep morphea, 12 (12%) as generalized morphea, and 15 (15%) as a mixed subtype. Nine patients (9%) had family histories of rheumatic or autoimmune diseases, while 16 (16%) might be triggered by unknown factors. Totally 84 patients underwent antinuclear antibody tests and 38 patients (45.2%) had positive results. Linear scleroderma are the most frequent subtype of JLS. Localized scleroderma may be associated with some autoimmune-related causes.

  6. Predictors of Longitudinal Quality of Life in Juvenile Localized Scleroderma.

    Science.gov (United States)

    Ardalan, Kaveh; Zigler, Christina K; Torok, Kathryn S

    2017-07-01

    Localized scleroderma can negatively affect children's quality of life (QoL), but predictors of impact have not been well described. We sought to identify predictors of QoL impact in juvenile localized scleroderma patients. We analyzed longitudinal data from a single-center cohort of juvenile localized scleroderma patients, using hierarchical generalized linear modeling (HGLM) to identify predictors of QoL impact. HGLM is useful for nested data and allows for evaluation of both time-variant and time-invariant predictors. The number of extracutaneous manifestations (ECMs; e.g., joint contracture and hemifacial atrophy) and female sex predicted negative QoL impact, defined as a Children's Dermatology Life Quality Index score >1 (P = 0.019 for ECMs and P = 0.002 for female sex). As the time since the initial visit increased, the odds of reporting a negative QoL impact decreased (P scleroderma than cutaneous features. Further study is required to determine which ECMs have the most impact on QoL, which factors underlie sex differences in QoL in localized scleroderma, and why increasing the time since the initial visit appears to be protective. An improved understanding of predictors of QoL impact may allow for the identification of patients at risk of poorer outcomes and for the tailoring of treatment and psychosocial support. © 2016, American College of Rheumatology.

  7. New developments in juvenile systemic and localized scleroderma.

    Science.gov (United States)

    Foeldvari, Ivan

    2013-11-01

    Juvenile localized scleroderma (jLS) and juvenile systemic sclerosis (jSS) are both orphan diseases, with jLS around 10 times more frequent than jSS. In recent years the time gap between the appearance of symptoms and diagnosis has become significantly shorter. This review focuses on the new classifications of jSS and jLS, and on the developments and adaptations of the outcome measures for certain organ involvements whereby progress has been made regarding pediatric patients. Copyright © 2013 Elsevier Inc. All rights reserved.

  8. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma.

    Science.gov (United States)

    Li, Suzanne C; Torok, Kathryn S; Pope, Elena; Dedeoglu, Fatma; Hong, Sandy; Jacobe, Heidi T; Rabinovich, C Egla; Laxer, Ronald M; Higgins, Gloria C; Ferguson, Polly J; Lasky, Andrew; Baszis, Kevin; Becker, Mara; Campillo, Sarah; Cartwright, Victoria; Cidon, Michael; Inman, Christi J; Jerath, Rita; O'Neil, Kathleen M; Vora, Sheetal; Zeft, Andrew; Wallace, Carol A; Ilowite, Norman T; Fuhlbrigge, Robert C

    2012-08-01

    Juvenile localized scleroderma (LS) is a chronic inflammatory skin disorder associated with substantial morbidity and disability. Although a wide range of therapeutic strategies has been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment has made it difficult to compare approaches and identify optimal therapy. Our objective was to develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile LS. A core group of pediatric rheumatologists, dermatologists, and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for juvenile LS using consensus methods/nominal group techniques. Recommendations were validated in 2 face-to-face conferences with a larger group of practitioners with expertise in juvenile LS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the US and Canada. Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members. Standardized clinical assessment methods and provisional treatment response criteria were also developed. Greater than 90% of pediatric rheumatologists responding to a survey (66% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with juvenile LS. Using consensus methodology, we have developed standardized treatment plans and assessment methods for juvenile LS. The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of juvenile LS. Copyright © 2012 by the American College of Rheumatology.

  9. Juvenile localized scleroderma with port wine stain: Coincidental or possible common pathogenetic association

    Directory of Open Access Journals (Sweden)

    Seval Dogruk Kacar

    2015-01-01

    Full Text Available Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain. Coexistence of both diseases is not found yet. We herein present a case of juvenile localized scleroderma on the left side of trunk, with congenital port wine stain located on the ipsilateral face at V1-V2 distribution.

  10. Juvenile localized scleroderma with port wine stain: coincidental or possible common pathogenetic association.

    Science.gov (United States)

    Kacar, Seval Dogruk; Ozuguz, Pinar; Polat, Serap; Kacar, Emre; Polat, Onur; Tokyol, Cigdem

    2015-01-01

    Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain. Coexistence of both diseases is not found yet. We herein present a case of juvenile localized scleroderma on the left side of trunk, with congenital port wine stain located on the ipsilateral face at V1-V2 distribution.

  11. Localized Scleroderma

    Science.gov (United States)

    ... How Is Localized Scleroderma Diagnosed? Doctors who are familiar with scleroderma, or who are experts at examining ... systemic treatment with a medication or other treatment interventions (for example, ultraviolet light), are reserved for more ...

  12. Development of Consensus Treatment Plans for Juvenile Localized Scleroderma

    Science.gov (United States)

    Li, Suzanne C.; Torok, Kathryn S.; Pope, Elena; Dedeoglu, Fatma; Hong, Sandy; Jacobe, Heidi T.; Rabinovich, C. Egla; Laxer, Ronald M.; Higgins, Gloria C.; Ferguson, Polly J.; Lasky, Andrew; Baszis, Kevin; Becker, Mara; Campillo, Sarah; Cartwright, Victoria; Cidon, Michael; Inman, Christi J; Jerath, Rita; O'Neil, Kathleen M.; Vora, Sheetal; Zeft, Andrew; Wallace, Carol A.; Ilowite, Norman T.; Fuhlbrigge, Robert C

    2013-01-01

    Objective To develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile localized scleroderma (jLS). Background jLS is a chronic inflammatory skin disorder associated with substantial morbidity and disability. Although a wide range of therapeutic strategies have been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment of have made it difficult to compare approaches and identify optimal therapy. Methods A core group of pediatric rheumatologists, dermatologists and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for jLS using consensus methods/nominal group techniques. Recommendations were validated in two face-to-face conferences with a larger group of practitioners with expertise in jLS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the U.S and Canada. Results Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members. Standardized clinical assessment methods and provisional treatment response criteria were also developed. Greater than 90% of pediatric rheumatologists responding to a survey (67% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with jLS. Conclusions Using consensus methodology, we have developed standardized treatment plans and assessment methods for jLS. The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of jLS. PMID:22505322

  13. Clinicohistopathological correlations in juvenile localized scleroderma: studies on a subset of children with hypopigmented juvenile localized scleroderma due to loss of epidermal melanocytes.

    Science.gov (United States)

    Sung, Joanne J; Chen, Tina S; Gilliam, Anita C; McCalmont, Timothy H; Gilliam, Amy E

    2011-08-01

    Localized scleroderma or morphea is a connective tissue disorder characterized by fibrosis of the skin and subcutaneous tissue. Excessive accumulation of collagen underlies the fibrosis, yet the pathogenesis is unknown. A subset of localized scleroderma/morphea, juvenile localized scleroderma (JLS), affects children and adolescents. The clinical and microscopic features of JLS have not been fully characterized. The goal is to better characterize the microscopic features of JLS. We collected a distinctive data set of 35 children with JLS, 19 (54%) of whom presented with hypopigmented lesions, and performed a retrospective chart and pathology review. We had adequate tissue for immunostaining studies on 8 of these individuals. We found that: (1) CD34 and factor XIIIa immunostaining, reported previously in adult morphea and scleroderma, when used with clinical information, is valuable for confirming a diagnosis of JLS; and (2) presence of hypopigmented lesions in JLS correlates with immunostaining studies. Decreased numbers of MelanA(+) melanocytes were present at the dermoepidermal junction in lesional skin in two of 3 children with hypopigmented JLS and in two of 4 children with nonhypopigmented JLS. The number of cases is small, a function of the small number of children who have biopsy specimens with material sufficient for multiple immunostaining procedures. These results provide a useful immunostaining method for confirmation of the diagnosis of JLS. They suggest a complex autoimmune phenotype in some children with JLS. Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  14. Peripheral blood cytokine and chemokine profiles in juvenile localized scleroderma

    Science.gov (United States)

    Torok, Kathryn S.; Kurzinski, Katherine; Kelsey, Christina; Yabes, Jonathan; Magee, Kelsey; Vallejo, Abbe N.; Medsger, Thomas; Feghali-Bostwick, Carol A.

    2015-01-01

    Objective To evaluate peripheral blood T-helper (TH) cell associated cytokine and chemokine profiles in localized scleroderma (LS), and correlate them with clinical disease features, including disease activity parameters. Methods A 29-plex Luminex platform was used to analyze the humoral profile of plasma samples from 69 pediatric LS patients and 71 healthy pediatric controls. Cytokine/chemokine levels were compared between these two groups and within LS patients, focusing on validated clinical outcome measures of disease activity and damage in LS. Results Plasma levels of IP-10, MCP-1, IL-17a, IL-12p70, GM-CSF, PDGF-bb, IFN-α2, and IFN-γ were significantly higher in LS compared to healthy controls. Analysis within the LS group demonstrated IP-10, TNF-α and GM-CSF correlated with clinical measures of disease activity. Several cytokines/chemokines correlated with anti-histone antibody, while only a few correlated with positive ANA and single-stranded DNA antibody. Conclusion This is the first time that multiple cytokines and chemokines have been examined simultaneously LS. In general, a TH-1 (IFN-γ) and TH-17 (IL-17a) predominance was demonstrated in LS compared to healthy controls. There is also an IFN–γ signature with elevated IP-10, MCP-1 and IFN-γ, which has been previously demonstrated in systemic sclerosis, suggesting a shared pathophysiology. Within the LS patients, those with active disease demonstrated IP-10, TNF-α and GM-CSF, which may potentially serve as biomarkers of disease activity in the clinical setting. PMID:26254121

  15. Successful combination treatment of a patient with progressive juvenile localized scleroderma (morphea) using imatinib, corticosteroids, and methotrexate.

    Science.gov (United States)

    Inamo, Yasuji; Ochiai, Toyoko

    2013-01-01

    We report a case of progressive juvenile localized scleroderma (JLS or morphea) treated with a combination of imatinib, corticosteroids, and methotrexate. This therapy halted the progressive skin thickening and the hand and finger joint deformity in the early stages of the disease. We conclude that imatinib used in addition to standard treatment with systemic corticosteroids and methotrexate may be of therapeutic benefit for individuals with JLS. © 2012 Wiley Periodicals, Inc.

  16. Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation.

    Science.gov (United States)

    Borowiec, A; Dabrowski, R; Wozniak, J; Jasek, S; Chwyczko, T; Kowalik, I; Musiej-Nowakowska, E; Szwed, H

    2012-02-01

    The aim of the present study was non-invasive evaluation of the cardiovascular system in asymptomatic young adult patients with juvenile localized scleroderma (JLS) and juvenile systemic sclerosis (JSS). A group of 34 consecutive children with scleroderma were prospectively observed in the study. The control group (CG) consisted of 20 healthy subjects. In each subject 12-lead electrocardiographic, echocardiographic, ECG Holter, and ambulatory blood pressure monitoring examinations were performed at the baseline visit and after 10 years. Additionally, B-type natriuretic peptide (BNP) concentrations were measured after 10 years. Examinations were performed in 13 patients with JLS and 15 with JSS at the final visit. Two children had died (one from each group). Four patients were alive but refused the final visit. After 10 years, a higher prevalence of ventricular extrasystoles (p = 0.01) and an elevated pulmonary arterial pressure (JLS: p = 0.04, JSS: p = 0.03) were observed in both groups, but in comparison with the controls there was no significant difference at the final visit. In JLS patients more cases of left ventricle diastolic dysfunction, hypertension, and sinus tachycardia were diagnosed at the final visit (p ≤ 0.05). More atrioventricular block episodes in both groups of scleroderma patients were observed. Over the 10 years, arterial hypertension was diagnosed in three patients from the JLS group and in two with JSS. There were no significant differences in BNP concentrations at the final visit. The results of the present study show that juvenile scleroderma seems to be more benign than adult-onset disease. This observational study shows subclinical, not severe, cardiac abnormalities in adult patients with juvenile-onset disease.

  17. Esophageal abnormalities in juvenile localized scleroderma: is it associated with other extracutaneous manifestations?

    Science.gov (United States)

    Valões, Clarissa C M; Novak, Glaucia V; Brunelli, Juliana B; Kozu, Katia T; Toma, Ricardo K; Silva, Clovis A

    To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. For a period of 30 years, 5881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring. JLS was observed in 56/5881 patients (0.9%), mainly linear morphea subtype. EI was observed in 23/56(41%) of JLS patients. Eight(35%) of 23 EI patients with JLS were symptomatic and presented heartburn(5/8), solid and liquid dysphagia(3/8), nausea and epigastralgia(1/8). The frequency of any cumulative extracutaneous manifestations (calcinosis, arthritis/arthralgia, central nervous system, interstitial pneumonitis, mesangial nephritis and/or arrhythmia) was significantly higher in JLS patients with EI compared to those without this complication (56% vs. 24%, p=0.024). No differences were evidenced in demographic data, JLS subtypes and in each extracutaneous manifestation in both groups (p>0.05). The frequency of methotrexate use was significantly higher in JLS patients with EI compared to those without (52% vs. 12%, p=0.002). Autoantibody profile (antinuclear antibodies, anti-SCL-70, rheumatoid factor, anticentromere, anti-cardiolipin, anti-Ro/SSA and anti-La/SSB) was similar in both groups (p>0.05). Our study demonstrated that EI was frequently observed in JLS patients, mainly in asymptomatic patients with linear subtype. EI occurred in JLS patients with other extracutaneous manifestations and required methotrexate therapy. Copyright © 2016. Published by Elsevier Editora Ltda.

  18. Esophageal abnormalities in juvenile localized scleroderma: is it associated with other extracutaneous manifestations?

    Directory of Open Access Journals (Sweden)

    Clarissa C.M. Valões

    Full Text Available Abstract Objective: To assess esophageal involvement (EI in juvenile localized scleroderma (JLS population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. Methods: For a period of 30 years, 5881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring. Results: JLS was observed in 56/5881 patients (0.9%, mainly linear morphea subtype. EI was observed in 23/56(41% of JLS patients. Eight(35% of 23 EI patients with JLS were symptomatic and presented heartburn(5/8, solid and liquid dysphagia(3/8, nausea and epigastralgia(1/8. The frequency of any cumulative extracutaneous manifestations (calcinosis, arthritis/arthralgia, central nervous system, interstitial pneumonitis, mesangial nephritis and/or arrhythmia was significantly higher in JLS patients with EI compared to those without this complication (56% vs. 24%, p = 0.024. No differences were evidenced in demographic data, JLS subtypes and in each extracutaneous manifestation in both groups (p > 0.05. The frequency of methotrexate use was significantly higher in JLS patients with EI compared to those without (52% vs. 12%, p = 0.002. Autoantibody profile (antinuclear antibodies, anti-SCL-70, rheumatoid factor, anticentromere, anti-cardiolipin, anti-Ro/SSA and anti-La/SSB was similar in both groups (p > 0.05. Conclusions: Our study demonstrated that EI was frequently observed in JLS patients, mainly in asymptomatic patients with linear subtype. EI occurred in JLS patients with other extracutaneous manifestations and required methotrexate therapy.

  19. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea).

    Science.gov (United States)

    Zulian, Francesco; Vallongo, Cristina; Patrizi, Annalisa; Belloni-Fortina, Anna; Cutrone, Mario; Alessio, Maria; Martino, Silvana; Gerloni, Valeria; Vittadello, Fabio; Martini, Giorgia

    2012-12-01

    Recent studies report that methotrexate (MTX) is beneficial in the treatment of juvenile localized scleroderma (JLS) but little is known about its long-term effectiveness. We assessed the therapeutic role of MTX in children with JLS who were followed up for a prolonged period. A cohort of patients with JLS, previously enrolled in a double-blind, randomized controlled trial and treated with oral MTX (15 mg/m(2)/wk) and prednisone (1 mg/kg/d, maximum 50 mg) for the first 3 months, were prospectively followed up. Lesions were evaluated clinically, with infrared thermography, and by a computerized skin score. Response to treatment was defined as: (1) no new lesions; (2) skin score rate less than 1; and (3) decrease in lesion temperature by at least 10% compared with baseline. Clinical remission (CR) on medication was defined when response was maintained, on treatment, for at least 6 months, and complete CR when response was maintained, without treatment, for at least 6 months. Of 65 patients treated with MTX, 48 (73.8%) were responders, 10 (15.4%) relapsed by 24 months since MTX start, and 7 (10.8%) were lost to follow-up. Among the responders, 35 (72.9%) maintained CR for a mean of 25 months and 13 (27.1%) were in CR on medication. Adverse effects seen in 28 patients (48.3%) were generally mild and never required treatment discontinuation. The use of objective measures not widely available, such as infrared thermography and computerized skin score, makes it difficult to compare data from previous studies. Long-term MTX therapy is beneficial and well tolerated for JLS. Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  20. Initial evaluation of an ultrasound measure for assessing the activity of skin lesions in juvenile localized scleroderma.

    Science.gov (United States)

    Li, S C; Liebling, M S; Haines, K A; Weiss, J E; Prann, A

    2011-05-01

    To evaluate the construct validity of 2 proposed measures (the Ultrasound Disease Activity [U-DA] and the Tissue Thickness Score [TTS]) for evaluating sonographic differences in juvenile localized scleroderma skin lesions. We conducted a retrospective review of juvenile localized scleroderma patients who had ultrasound scans of their skin lesions between October 2005 and February 2009. Imaged lesions were classified as active or inactive based upon clinical assessment. Lesions had to have been imaged within 1 month of a clinic visit or have the same clinical assessment during both the visit before and the visit after the scan. Two physicians scored the scans using the U-DA, which scores for differences in lesion echogenicity and vascularity compared with normal tissue. Tissue thickness differences were evaluated by percent differences and by using the TTS. Wilcoxon's rank sum test was performed to assess differences. We studied 52 scans from 21 patients, 32 scans of active skin lesions and 20 scans of inactive skin lesions. Features reported by clinicians as indicative of active disease included erythema, warmth, violaceous color, new lesion, expansion of lesion, and induration. The U-DA was significantly different between active and inactive skin lesions (P = 0.0010) with significant differences found for the parameters of total echogenicity, hypodermis echogenicity, and deep tissue layer vascularity (P = 0.0014, P = 0.0023, and P = 0.0374, respectively). No significant differences were found for tissue layer thickness or TTS. The U-DA may be a useful tool in the identification of localized scleroderma activity. Further study is needed to prospectively evaluate the validity, reliability, and sensitivity of this potential monitoring tool. Copyright © 2011 by the American College of Rheumatology.

  1. JUVENILE SCLERODERMA-what has changed in the meantime?

    Science.gov (United States)

    Adrovic, Amra; Sahin, Sezgin; Barut, Kenan; Kasapcopur, Ozgur

    2018-04-22

    Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition. Skin manifestations are most prominent feature of the systemic form, followed by musculoskeletal and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in childhood. Combination of disease modifying antirheumatic drugs (methotrexate, mycophenolate-mofetil, cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a useful treatment option in most severe form of the disease. Localized scleroderma is characterized with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the local scleroderma is made according to the size and localization of the skin changes. There are few different therapeutical options but there is no specific therapy for the localized scleroderma. Many data regarding disease features and treatment options in juvenile scleroderma are based on studies among adults. There is a striking need for multicentric, prospective studies among children with juvenile scleroderma.Emerging biological agents and new treatment options are showing promising results. Anyhow, juvenile scleroderma remains a mystery with many aspects of the disease waiting to be solved. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  2. Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease.

    Science.gov (United States)

    Hedrich, Christian Michael; Fiebig, Barbara; Hahn, Gabriele; Suttorp, Meinolf; Gahr, Manfred

    2011-07-01

    Juvenile scleroderma is a rare connective tissue disease that involves the skin and subcutaneous tissue. Among all presentations of juvenile scleroderma, localized scleroderma (JLSc) is the most frequent, followed by systemic disease (JSSc) and eosinophilic fasciitis (EF). In posttransplantation chronic graft-versus-host disease (GvHD), scleroderma-like skin involvement can occur. Systemic forms of juvenile scleroderma and GvHD can affect the internal organs, such as the lungs, the gastrointestinal tract, the heart, and kidneys and cause disability and severe, sometimes lethal, complications. Here, the authors give an overview of different presentations of juvenile scleroderma. They report their experience with the different forms and presentations of scleroderma, diagnostic workups, treatment, and outcome of all forms of childhood scleroderma in the context of the existing literature.

  3. Localized scleroderma: imaging features

    International Nuclear Information System (INIS)

    Liu, P.; Uziel, Y.; Chuang, S.; Silverman, E.; Krafchik, B.; Laxer, R.

    1994-01-01

    Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with ''en coup de sabre'' facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients. (orig.)

  4. Localized scleroderma: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Liu, P. (Dept. of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)); Uziel, Y. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada)); Chuang, S. (Dept. of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)); Silverman, E. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada)); Krafchik, B. (Div. of Dermatology, Dept. of Pediatrics, Hospital for Sick Children, Toronto, ON (Canada)); Laxer, R. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada))

    1994-06-01

    Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with ''en coup de sabre'' facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients. (orig.)

  5. Access to care for children and young people diagnosed with localized scleroderma or juvenile SSc in the UK.

    Science.gov (United States)

    Hawley, Daniel P; Baildam, Eileen M; Amin, Tania S; Cruikshank, Mary K; Davidson, Joyce E; Dixon, Jennifer; Martin, Neil S; Ohlsson, Victoria; Pilkington, Clarissa; Rangaraj, Satyapal; Riley, Philip; Sundaramoorthy, Chitra; Walsh, Jo; Foster, Helen E

    2012-07-01

    To describe pathways of care and referral to paediatric rheumatology from onset of first symptom (noticed by the patient or their family) to diagnosis for children and young people diagnosed with localized scleroderma (LS) or juvenile SSc (jSSc). Retrospective case note audit of patients under paediatric rheumatology care who presented during January 2005-January 2010. Data included disease subtype, sex, age at key points in the referral pathway and health care professional (HCP) contact. All patient and HCP data were pseudo-anonymized in accordance with good clinical practice. Data were from eight UK centres that saw 89 cases: 62 females, 26 males; 73 LS, 16 jSSc. Median time from first symptom to first HCP review was 4 (range 0-72) months (LS) and 1 (range 0-50) month (jSSc). Median time from first symptom to paediatric rheumatology review was 15 (range 1-103) months (LS) and 7 (range 0-50) months (jSSc). Median time from first HCP review to first paediatric rheumatology review was 11 (range 0-103) months (LS) and 2 (range 0-10) months. First HCP seen (74%) was usually a general practitioner. The referring HCP to paediatric rheumatology was usually a dermatologist (56%) for LS. Median time from first symptom to diagnosis was 13 (range 1-102) months (LS) and 8 (range 1-50) months (jSSc). A prolonged interval occurs from first symptom to definitive diagnosis, which may adversely affect outcome. There is a need to raise awareness of this rare diagnosis and facilitate earlier recognition.

  6. Surgical Management of Localized Scleroderma.

    Science.gov (United States)

    Lee, Jae Hyun; Lim, Soo Yeon; Lee, Jang Hyun; Ahn, Hee Chang

    2017-09-01

    Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients.

  7. [Localized scleroderma (morphea)].

    Science.gov (United States)

    Bono, Waafa; Dupin, Nicolas

    2006-12-01

    DEFINITION AND FREQUENCY: Localized scleroderma, also known as morphea, is a sclerotic condition limited to the skin. The specific clinical entity depends on the extent, linear disposition and depth of the lesions. Morphea is ten times more prevalent than systemic sclerosis, and its prognosis is generally good: superficial forms resolve within 3 years. In the absence of symptoms, examinations to detect systemic involvement are purposeless. Plaque morphea is the most frequent clinical presentation. Serious manifestations include extensive morphea that may involve the entire skin or linear forms, especially in children, where they may be severe, especially on the face. There are no immunological markers clearly associated with morphea and no causative agents have been implicated in its pathogenesis, although sclerodermiform dermatitis is reported to be associated with some drugs and toxic agents. There is no consensual treatment for morphea. Treatment should be decided according to severity and extent of lesions. Limited lesions may be treated with local steroids such as class IV corticosteroids. Systemic treatment (methotrexate) should be discussed in extensive and linear forms when there is a risk of functional or esthetic complications.

  8. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

    Science.gov (United States)

    McCann, Liza J; Pain, Clare E

    2016-02-01

    Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

  9. [Localized scleroderma (morphea) in childhood].

    Science.gov (United States)

    Weibel, L

    2012-02-01

    Localized scleroderma or morphea is a sclerosing connective tissue disease of the skin, which may affect underlying tissues such as subcutis, muscle and bone. Many patients show extracutaneous symptoms and antinuclear antibodies, however, secondary transformation into systemic sclerosis does not occur. Localized scleroderma usually begins in childhood with a wide variation in its clinical spectrum. The linear variant is the most common subtype in children, associated with a progressive course and increased risk of complications. The disease may progress over years and result in severe functional and cosmetic disability. The etiology of localized scleroderma remains unknown. A genetic background is suspected, while triggers such as trauma, vaccinations and infections may lead to secondary immunologic phenomena. Localized scleroderma often remains unrecognized for a long time, resulting in substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as first-line therapy for progressive (usually linear) disease, whereas phototherapy (UVA-1 or UVB-narrow band) is suitable for adolescents with superficial circumscribed subtypes.

  10. [Morphea or juvenile localised scleroderma: Case report].

    Science.gov (United States)

    Strickler, Alexis; Gallo, Silvanna; Jaramillo, Pedro; de Toro, Gonzalo

    2016-01-01

    Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy. We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. [Nodules on localized scleroderma or morphea].

    Science.gov (United States)

    Bayle, P; Bazex, J; Marguery, M-C; Lamant, L

    2005-02-01

    Localized scleroderma or morphea usually appears as flat or depressed lesions. We report 3 cases of morphea with atypical appearance, alternating pigmented and depigmented patches with nodules or sclerous bands, occurring in adult men. The occurrence of nodular elements on generalized or localized scleroderma, although rare, was first reported in the literature by Addisson in 1884. These nodules usually appear during evolution. These scleroderma are then described as being keloidal or nodular. We report 3 cases of nodules on localized scleroderma which appeared at the beginning of the dermatosis and where the scleroderma had a similar unusual irregularly pigmented appearance.

  12. Localized Scleroderma: A Clinical Review.

    Science.gov (United States)

    Tratenberg, Mark; Gutwein, Farrah; Rao, Varuni; Sperber, Kirk; Wasserrman, Amy; Ash, Julia

    2017-01-01

    Localized scleroderma (LS) is characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissue or both. The outcome for most patients with localized scleroderma is directly related to the type and stage of the affected tissue. The major challenge for untreated patients is not increased mortality risk, rather deformity and growth defects from skin, muscle and bone abnormalities. Treatment is individualized to type and stage of the lesion and may include pharmacologic and non-pharmacologic therapies. Among the pharmacologic modalities, methotrexate with systemic glucocorticoids is currently the mainstay of treatment. More controlled trials are needed to determine the length of treatment and the maintenance dose of this combination therapy. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  13. Localized scleroderma: clinical spectrum and therapeutic update.

    Science.gov (United States)

    Careta, Mariana Figueiroa; Romiti, Ricardo

    2015-01-01

    Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

  14. Localized scleroderma: clinical spectrum and therapeutic update*

    Science.gov (United States)

    Careta, Mariana Figueiroa; Romiti, Ricardo

    2015-01-01

    Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma. PMID:25672301

  15. Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review.

    Science.gov (United States)

    Martini, Giorgia; Fadanelli, Gloria; Agazzi, Anna; Vittadello, Fabio; Meneghel, Alessandra; Zulian, Francesco

    2018-05-03

    Juvenile Localized Scleroderma (JLS) is a rare disorder that may cause severe aesthetic sequelae and functional disability. To date, data on natural history and long-term outcome are discordant and difficult to compare due to the heterogeneity of clinical subtypes, treatments and methods to evaluate activity and outcome in previous studies. A retrospective and cross-sectional study including 133 patients followed between January 1991 and December 2016 was conducted at our Pediatric Rheumatology Centre. Disease course was drawn by retrospective analysis of patients' clinical features, treatment, disease course and outcome at the last evaluation. Disease activity and severity of tissue damage were assessed by using parameters derived from the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and thermography. Most patients achieved complete remission, as only 12.5%, all with the linear subtype, had still active disease after over 10 years of follow-up. At least one disease relapse occurred in 22.2% of patients and first flare was observed 20 months after first treatment discontinuation. Mild tissue damage was observed in more than half of patients, in 25.4% was moderate and in 23.0% severe; 19.8% presented a functional limitation. The entity of skin and subcutaneous fat loss established at the early stages of the disease as 27.8% of patients with shorter disease duration had severe damage and the rates remained constant in patients with longer follow-up. The delay in start of systemic treatment was associated with longer disease activity and higher relapse rate. Patients with linear scleroderma (LS), pansclerotic morphea (PM) and mixed subtype (MS) presented more severe aesthetic and functional damage but did not differ from other subtypes as for rate of complete remission. JLS in some patients can be a very aggressive disease with persistent activity after >10 years and/or several disease relapses. As tissue damage establishes early in disease course a

  16. Localized scleroderma and regional inflammatory myopathy.

    Science.gov (United States)

    Zivković, Saša A; Freiberg, William; Lacomis, David; Domsic, Robyn T; Medsger, Thomas A

    2014-05-01

    Inflammatory myopathy is rare in localized scleroderma. We report 2 new cases of regional inflammatory myopathy associated with localized scleroderma and review 10 reported cases of localized scleroderma associated with an inflammatory myopathy with regional muscle involvement, more often in the upper extremities. Serum creatine kinase was mildly elevated or normal. Histopathology often showed perimysial inflammation and plasma cell infiltration. These cases demonstrate that inflammatory myopathy should be considered in patients with localized scleroderma and regional muscle weakness, pain or atrophy. Muscle biopsy can confirm the diagnosis of myositis, which if identified, will require anti-inflammatory and/or immunosuppressive therapy. Published by Elsevier B.V.

  17. Localized severe scleroderma: a retrospective study of 26 pediatric patients.

    Science.gov (United States)

    Beltramelli, Matilde; Vercellesi, Paolo; Frasin, Adina; Gelmetti, Carlo; Corona, Fabrizia

    2010-01-01

    Juvenile localized scleroderma includes different conditions characterized by skin hardening with increased collagen deposition. Although juvenile localized scleroderma is considered a relatively benign disease, lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Furthermore, extracutaneous manifestations are described. We retrospectively analyzed a cohort of 26 patients with severe Juvenile localized scleroderma with particular attention to clinical features, therapy, and long-term outcome. A subgroup of three patients has been further evaluated with infrared thermography. Our findings were consistent with the current literature for demographic, laboratory, and clinical characteristics at disease onset, but, with our patients, the prevalence of extracutaneous manifestations was higher, thus confirming the potential for severe juvenile localized scleroderma to affect organs other than the skin, without increased risk of development toward systemic sclerosis. Correlation between various treatments and clinical endpoint showed that systemic therapy lead to a better outcome: in particular, methotrexate appeared the most effective drug, capable in halting the progression of the disease and sometimes inducing its regression. © 2010 Wiley Periodicals, Inc.

  18. Localized scleroderma: a series of 52 patients.

    Science.gov (United States)

    Toledano, C; Rabhi, S; Kettaneh, A; Fabre, B; Fardet, L; Tiev, K P; Cabane, J

    2009-05-01

    Localized scleroderma also called morphea is a skin disorder of undetermined cause. The widely recognized Mayo Clinic Classification identifies 5 main morphea types: plaque, generalized, bullous, linear and deep. Whether each of these distinct types has a particular clinical course or is associated with some patient-related features is still unclear. We report here a retrospective series of patients with localized scleroderma with an attempt to identify features related to the type of lesion involved. The medical records of all patients with a diagnosis of localized scleroderma were reviewed by skilled practitioners. Lesions were classified according to the Mayo Clinic Classification. The relationship between each lesion type and various clinical features was tested by non-parametrical methods. The sample of 52 patients included 43 females and 9 males. Median age at onset was 30 y (range 1-76). Frequencies of patients according to morphea types were: plaque morphea 41 (78.8%) (including morphea en plaque 30 (57.7%) and atrophoderma of Pasini-Pierini 11 (21.1%)), linear scleroderma 14 (26.9%). Nine patients (17.3%) had both types of localized scleroderma. Median age at onset was lower in patients with linear scleroderma (8 y (range 3-44)) than in others (36 y (range 1-77)) (p=0.0003). Head involvement was more common in patients with linear scleroderma (37.5%) than in other subtypes (11.1%) (p=0.05). Atrophoderma of Pasini-Pierini was never located at the head. Systemic symptoms, antinuclear antibodies and the rheumatic factor were not associated with localized scleroderma types or subtypes. These results suggest that morphea types, in adults are not associated with distinct patient features except for age at disease onset (lower) and the localization on the head (more frequent), in patients with lesions of the linear type.

  19. Periostin in Mature Stage Localized Scleroderma.

    Science.gov (United States)

    Kim, Min-Woo; Park, Jung Tae; Kim, Jung Ho; Koh, Seong-Joon; Yoon, Hyun-Sun; Cho, Soyun; Park, Hyun-Sun

    2017-06-01

    Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare. To investigate the expression of periostin and other molecules in localized scleroderma. A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken. Fourteen age-matched and biopsy site-matched subjects with normal skin were included as controls. Collagen fiber deposition, periostin, procollagen, transforming growth factor-β, and matrix metalloproteinase (MMP)-1 expression were assessed and compared between the two groups. Co-localization of α-smooth muscle actin and periostin was evaluated using confocal microscopy. Periostin was predominantly expressed along the dermo-epidermal junction in the controls. Conversely, patients with localized scleroderma demonstrated increased collagen fiber deposition and periostin expression that was more widely distributed along the entire dermis. MMP-1 staining showed increased expression in the epidermis and dermis of patients compared to scanty expression in the controls. A semi-quantitative evaluation showed a higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, p =0.013), diffuse periostin positivity (42.9% vs. 0%, p =0.016), and moderate MMP-1 positivity (71.4% vs. 7.1%, p =0.001) in patients than in the controls. Compared to the controls, patients with localized scleroderma had enhanced periostin expression corresponding to increased collagen fiber deposition and unexpected overexpression of MMP-1. The results of this human in vivo study may implicate the pathogenesis of localized scleroderma.

  20. Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk

    DEFF Research Database (Denmark)

    Hesselvig, Jeanette Halskou; Kofoed, Kristian; Wu, Jashin J

    2018-01-01

    Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18...... and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint. A total of 697 patients with localized scleroderma and 1......,962 patients with systemic sclerosis were identified and compared with 5,428,380 people in the reference population. In systemic sclerosis, the adjusted HR was 2.22 (95% confidence interval 1.99-2.48). No association was seen between patients with localized scleroderma and cardiovascular disease. In conclusion...

  1. Localized scleroderma of the breast

    International Nuclear Information System (INIS)

    Opere, Elisa; Oleaga, Laura; Ibanez, Teresa; Grande, Domingo

    2002-01-01

    We report a 44-year-old patient with right-breast morphea. Mammography, MRI and needle biopsy were used for assessment of the case. Mammography demonstrated thickening of the skin and the subcutaneous tissue. The MRI showed replacement of the subcutaneous and breast fat by a low signal intensity, non-enhancing tissue. Skin biopsy confirmed the histological features of scleroderma. (orig.)

  2. Localized scleroderma of the breast

    Energy Technology Data Exchange (ETDEWEB)

    Opere, Elisa; Oleaga, Laura; Ibanez, Teresa; Grande, Domingo [Department of Radiology, Hospital de Basurto, Bilbao (Spain)

    2002-06-01

    We report a 44-year-old patient with right-breast morphea. Mammography, MRI and needle biopsy were used for assessment of the case. Mammography demonstrated thickening of the skin and the subcutaneous tissue. The MRI showed replacement of the subcutaneous and breast fat by a low signal intensity, non-enhancing tissue. Skin biopsy confirmed the histological features of scleroderma. (orig.)

  3. Pediatric scleroderma: systemic or localized forms.

    Science.gov (United States)

    Torok, Kathryn S

    2012-04-01

    Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of patients showing extracutaneous disease manifestations such as arthritis and uveitis. Vascular, cutaneous, gastrointestinal, pulmonary, and musculoskeletal involvement are most commonly seen in children with SSc. Treatment of both forms targets the active inflammatory stage and halts disease progression; however, progress needs to be made toward the development of more effective antifibrotic therapy to help reverse disease damage. Copyright © 2012 Elsevier Inc. All rights reserved.

  4. Pathogenesis and treatment modalities of localized scleroderma.

    Science.gov (United States)

    Valančienė, Greta; Jasaitienė, Daiva; Valiukevičienė, Skaidra

    2010-01-01

    Localized scleroderma is a chronic inflammatory disease primarily of the dermis and subcutaneous fat that ultimately leads to a scar-like sclerosis of connective tissue. The disorder manifests as various plaques of different shape and size with signs of skin inflammation, sclerosis, and atrophy. This is a relatively rare inflammatory disease characterized by a chronic course, unknown etiology, and insufficiently clear pathogenesis. Many factors may influence its appearance: trauma, genetic factors, disorders of the immune system or hormone metabolism, viral infections, toxic substances or pharmaceutical agents, neurogenic factors, and Borrelia burgdorferi infection. Various therapeutic modalities are being used for the treatment of localized scleroderma. There is no precise treatment scheme for this disease. A majority of patients can be successfully treated with topical pharmaceutical agents and phototherapy, but some of them with progressive, disseminated, and causing disability localized scleroderma are in need of systemic treatment. The aim of this article is not only to dispute about the clinical and morphological characteristics of localized scleroderma, but also to present the newest generalized data about the possible origin, pathogenesis, and treatment modalities of this disease.

  5. The frequency of pulmonary hypertension in patients with juvenile scleroderma

    Directory of Open Access Journals (Sweden)

    Amra Adrovic

    2015-08-01

    Full Text Available Juvenile scleroderma (JS represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests, Forced vital capacity (FVC and Carbon monoxide diffusion capacity (DLCO in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP and risk factors for pulmonary arterial hypertension (PAH were made by the measurement of maximum tricuspid insufficiency (TI, end-diastolic pulmonary insufficiency (PI, ratio of acceleration time (AT to ejection time (ET (AT/ET, right atrial pressure (RAP and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years. The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years and 3.57 years (median 2 years, range 0.5-14.5 years, respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

  6. The frequency of pulmonary hypertension in patients with juvenile scleroderma.

    Science.gov (United States)

    Adrovic, Amra; Oztunc, Funda; Barut, Kenan; Koka, Aida; Gojak, Refet; Sahin, Sezgin; Demir, Tuncalp; Kasapcopur, Ozgur

    2015-08-22

    Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

  7. Morphea and other localized forms of scleroderma.

    Science.gov (United States)

    Vasquez, Rebecca; Sendejo, Chelsea; Jacobe, Heidi

    2012-11-01

    Morphea, also known as localized scleroderma, is a disorder of excessive collagen deposition leading to thickening of the dermis and/or subcutaneous tissues and may cause significant morbidity. This review will describe new developments in the evaluation and management of morphea as well as its pathophysiology. The reader will be able to apply these findings to patient management. The recent development of validated outcome measures (i.e. the localized scleroderma cutaneous assessment tool) as well as consensus treatment recommendations provide a platform for collaboration among specialties to develop both standardized assessment tools and therapeutic trials. New studies have also begun to investigate the immunological underpinnings of morphea. The promise of evidence-based treatments for morphea in the near future will provide better care for patients with morphea and understanding its pathophysiology will lay groundwork for the development of new treatments.

  8. Case for diagnosis. Localized scleroderma or morphea.

    Science.gov (United States)

    Tomiyoshi, Carolina; Wojcik, Adma Silva de Lima; Vencato, Elisa Milani Oba; Taques, Guilherme Ribas; Fillus Neto, José; Brenner, Fabiane Andrade Mulinari

    2010-01-01

    Localized scleroderma or morphea is a chronic disease of the connective tissue. Its etiology may be autoimmune and the condition results from a disturbance in collagen synthesis and deposition, clinically represented by sclerotic skin lesions. Some plaques may be yellowish, which can be misleading at diagnosis. This article reports the case of an adolescent girl who concomitantly presented erythematous lesions and yellowish lesions, both of which constitute clinical manifestations of the disease.

  9. Systemic involvement in localized scleroderma/morphea.

    Science.gov (United States)

    Gorkiewicz-Petkow, Anna; Kalinska-Bienias, Agnieszka

    2015-01-01

    Localized scleroderma (LoSc), also known as morphea, is a rare fibrosing disorder of the skin and underlying tissues. Sclerosis is mainly limited to the skin, but subcutaneous tissue, fascia, and underlying muscles and bone may also be involved. In some cases, systemic manifestation with visceral abnormalities may occur. Several publications have focused on significant aspects of LoSc: genetics, immunity, epidemiology, scoring systems, and unification of classifications. Clinical studies featuring large cohorts with the disease published by various international study groups have been of great value in furthering the diagnostic and therapeutic management of LoSc. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Scleroderma

    Science.gov (United States)

    Scleroderma means hard skin. It is a group of diseases that cause abnormal growth of connective tissue. ... their shape and helps keep them strong. In scleroderma, the tissue gets hard or thick. It can ...

  11. Oxidative stress parameters in localized scleroderma patients.

    Science.gov (United States)

    Kilinc, F; Sener, S; Akbaş, A; Metin, A; Kirbaş, S; Neselioglu, S; Erel, O

    2016-11-01

    Localized scleroderma (LS) (morphea) is a chronic, inflammatory skin disease with unknown cause that progresses with sclerosis in the skin and/or subcutaneous tissues. Its pathogenesis is not completely understood. Oxidative stress is suggested to have a role in the pathogenesis of localized scleroderma. We have aimed to determine the relationship of morphea lesions with oxidative stress. The total oxidant capacity (TOC), total antioxidant capacity (TAC), paroxonase (PON) and arylesterase (ARES) activity parameters of PON 1 enzyme levels in the serum were investigated in 13 LS patients (generalized and plaque type) and 13 healthy controls. TOC values of the patient group were found higher than the TOC values of the control group (p < 0.01). ARES values of the patient group was found to be higher than the control group (p < 0.0001). OSI was significantly higher in the patient group when compared to the control (p < 0.005). Oxidative stress seems to be effective in the pathogenesis. ARES levels have increased in morphea patients regarding to the oxidative stress and its reduction. Further controlled studies are required in wider series.

  12. [Localized scleroderma: a retrospective study about 92 cases].

    Science.gov (United States)

    El Fékih, Nadia; Réjaibi, Iménc; Kamoun, Hajer; Zéglaoui, Faten; Fazaa, Bécima; Kharfi, Monia; Kamoun, Mohamed Ridha

    2009-09-01

    Sclerodermas are rare affections which can be located or generalized. Localized form is the most frequent. The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature. We have performed a retrospective study on all patients followed in the department of dermatology of the Hospital Charles Nicole during 14 years period. Our study was about 92 cases of localized scleroderma (73 were females and 19 males). The mean age was 35 years (between 2 and 72 years). The majority of localised sclerodermas (66.2% of the cases) appeared before 40 years with a maximum of frequency between 10 and 30 years (41.6%). Only 11.9% of the cases were observed before 10 years. They were 51 cases (55%) of morphea, 35 cases (38%) of scleroderma in bands including 32 linear scleroderma and 3 scleroderma en coup de sabre, 5 cases (5.5%) of generalized morphea and 1 case (0.15%) of deep morphea. Average therapeutic was specified among 63 patients (87%), and the evolution could be appreciated among 45 patients. The epidemiologic data observed in our series are comparable with those reported in the literature. Therapeutic difficulties and risks of functional after-effects, particular in scleroderma in bands, remain the principal concern for all the authors.

  13. German guidelines for the diagnosis and therapy of localized scleroderma.

    Science.gov (United States)

    Kreuter, Alexander; Krieg, Thomas; Worm, Margitta; Wenzel, Jörg; Moinzadeh, Pia; Kuhn, Annegret; Aberer, Elisabeth; Scharffetter-Kochanek, Karin; Horneff, Gerd; Reil, Emma; Weberschock, Tobias; Hunzelmann, Nicolas

    2016-02-01

    Localized scleroderma designates a heterogeneous group of sclerotic skin disorders. Depending on the subtype, severity, and site affected, adjacent structures such as adipose tissue, muscles, joints, and bones may be involved. This is an update of the existing German AWMF (Association of the Scientific Medical Societies in Germany) guidelines (classification: S2k). These guidelines provide an overview of the definition, epidemiology, classification, pathogenesis, laboratory workup, histopathology, clinical scoring systems, as well as imaging and device-based workup of localized scleroderma. Moreover, consensus-based recommendations are given on the management of localized scleroderma depending on its clinical subtype. Treatment recommendations are presented in a therapeutic algorithm. No financial support was given by any pharmaceutical company. The guidelines are valid until July 2019. © 2016 The Authors | Journal compilation © Blackwell Verlag GmbH, Berlin.

  14. Evaluation of mean platelet volume in localized scleroderma.

    Science.gov (United States)

    Bahali, Anil Gulsel; Su, Ozlem; Emiroglu, Nazan; Cengiz, Fatma Pelin; Kaya, Mehmet Onur; Onsun, Nahide

    2017-01-01

    Localized scleroderma is a chronic inflammatory skin disease characterized by sclerosis of the dermis and subcutaneous tissue. Platelets play an important role in inflammation. Following activation, platelets rapidly release numerous mediators and cytokines, which contribute to inflammation. To evaluate whether there was any relation between localized scleroderma and platelet parameters. Forty-one patients with localized scleroderma were enrolled in the study. The control group consisted of 30 healthy subjects. The mean platelet volume level in the patient group was 9.9 ± 1.3 fl and in the control group was 7.6 ± 1.1 fl. This difference was statistically significant (pscleroderma. Platelet parameters may be used as markers for evaluating disease severity and inflammatory processes. Thus, there is a need for more detailed and prospective studies.

  15. Parry-Romberg Syndrome Associated with Localized Scleroderma

    Directory of Open Access Journals (Sweden)

    Jelena Maletic

    2010-06-01

    Full Text Available Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea.

  16. Parry-Romberg Syndrome Associated with Localized Scleroderma

    Science.gov (United States)

    Maletic, Jelena; Tsirka, Vassiliki; Ioannides, Panos; Karacostas, Dimitrios; Taskos, Nikolaos

    2010-01-01

    Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea). PMID:20671858

  17. A Patient with Localized Scleroderma Successfully Treated with Etretinate

    Science.gov (United States)

    Shima, Tomoko; Yamamoto, Yuki; Ikeda, Takaharu; Furukawa, Fukumi

    2014-01-01

    There are several treatment methods for localized scleroderma, but treatment is difficult when the lesion is widely distributed. We encountered a case who was treated successfully with etretinate, a vitamin A derivative. The usefulness of this agent is discussed. PMID:25408646

  18. A Patient with Localized Scleroderma Successfully Treated with Etretinate

    Directory of Open Access Journals (Sweden)

    Tomoko Shima

    2014-09-01

    Full Text Available There are several treatment methods for localized scleroderma, but treatment is difficult when the lesion is widely distributed. We encountered a case who was treated successfully with etretinate, a vitamin A derivative. The usefulness of this agent is discussed.

  19. Clinicopathological study of 81 cases of localized and systemic scleroderma.

    Science.gov (United States)

    Succaria, F; Kurban, M; Kibbi, A-G; Abbas, O

    2013-02-01

    Scleroderma is a connective tissue disease that includes localized and systemic forms. Our recent encounter with a morphea case exhibiting prominent perineural inflammation microscopically prompted us to assess the features of all patients diagnosed with morphea/scleroderma at our institution. To describe the clinicopathological features of all patients diagnosed with morphea/scleroderma at American University of Beirut Medical Center (AUB-MC) between 1999 and 2010, and compare our findings with those published in the literature. A total of 81 cases (63 women and 18 men) were identified, of which 73 were localized (morphea) and eight were systemic scleroderma. Clinically, plaque type morphea was the most common variant both in adults and children, and seven (9%) cases of morphea were associated with lichen sclerosis et atrophicus (LSA). Histopathologically, perineural inflammation was observed in 49% of cases, and may serve, in addition to other features including lichen sclerosis-like changes (observed in exclusively nine cases of morphea), more diffuse dermal and less subcutaneous sclerosis, and intense inflammation, as clues favouring diagnosis of morphea over systemic sclerosis. The features of morphea/scleroderma patients in this study are generally comparable to those published in the literature, with few differences. Clinically, plaque type morphea was the most common variant both in adults and children and LSA was a frequent association. Histopathologically, perineural inflammation was commonly observed and may serve in addition to lichen sclerosis-like changes and intense inflammation as clues favouring diagnosis of morphea over systemic sclerosis. © 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.

  20. Update on the classification and treatment of localized scleroderma.

    Science.gov (United States)

    Bielsa Marsol, I

    2013-10-01

    Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach. Copyright © 2011 Elsevier España, S.L. and AEDV. All rights reserved.

  1. A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide.

    Science.gov (United States)

    Ferguson, Ian D; Weiser, Peter; Torok, Kathryn S

    2015-01-01

    Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments. Infliximab is a potential treatment option for pediatric localized scleroderma patients who have progression of disease or who are unable to tolerate the side effect profile of more standard systemic therapy. Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

  2. Pediatric Scleroderma –Systemic and Localized Forms

    Science.gov (United States)

    Torok, Kathryn S.

    2012-01-01

    Synopsis statement Pediatric scleroderma includes two major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, with an initial inflammatory phase associated with endothelial activation, and a later fibrotic phase evidenced by collagenization of tissue and appreciable skin thickness, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, and though not fatal like SSc, up to a quarter of the patients may have extracutaneous disease manifestations, such as arthritis and uveitis. While any organ may be affected in SSc, vascular (Raynaud’s phenomenon), cutaneous (skin thickening), GI, pulmonary and musculoskeletal involvement are most commonly seen in children. Auto-antibody profiles in childhood onset SSc can assist in predicting internal organ involvement. Treatment for both forms of scleroderma targets the active inflammatory stage and halts disease progression; however, progress still needs to be made towards the development of a more effective anti-fibrotic therapy to help reverse disease damage. PMID:22560576

  3. Cytokine profiles in localized scleroderma and relationship to clinical features.

    Science.gov (United States)

    Kurzinski, Katherine; Torok, Kathryn S

    2011-08-01

    Localized scleroderma (LS) is a disfiguring autoimmune disease of the skin and underlying tissue that mainly affects the pediatric population. Inflammation of the tissue leads to fibrosis and atrophy, causing physical and psychological disability that can continue throughout childhood into adulthood. Available therapies for LS have had variable effects and are associated with morbidity themselves. A better understanding of the pathophysiology of LS, especially during the active inflammatory phase, would lead to more directed and efficacious therapies. As in systemic sclerosis (SSc), the other form of scleroderma, T-helper (Th) cells and their associated cytokines have been suggested to contribute significantly to the pathophysiology of LS supported by the presence of cytokines from these lineages in the sera and tissue of LS patients. It is postulated that the imbalance between Th1/Th2/Th17 cell subsets drives inflammation in the early stages of disease (Th1 and Th17 predominant) and fibrosis in the later stages of scleroderma (Th2 predominant). We review the available experimental data regarding cytokines in LS and compare them to available clinical disease severity and activity features. This provides the platform to launch further investigations into the role of select cytokines in the pathogenesis of LS and to provide directed therapeutic options in the future. Published by Elsevier Ltd.

  4. Treatment of morphea or localized scleroderma: review of the literature.

    Science.gov (United States)

    Vilela, Fernanda Aguiar Santos; Carneiro, Sueli; Ramos-e-Silva, Marcia

    2010-10-01

    Scleroderma is a disease that affects the microvasculature and the connective tissue. These alterations produce fibrosis and blood vessel occlusion. Its cause is still unknown, although the exaggerated synthesis of collagens I and IV, detected in skin and vessels, may be related to genetic, immunologic and, less frequently, exogenous factors as inhalation of silica and polyvinyl chloride. There is a localized and a systemic form, which affects both adults and children. The treatment of the localized form, also called morphea, is still controversial, and, in this article, the authors will discuss the main agents that were found to improve the lesions and symptoms.

  5. Localized scleroderma and its implications on quality of life

    Directory of Open Access Journals (Sweden)

    Nathalia Mebius

    2014-12-01

    Full Text Available Scleroderma is a disease of unknown etiology, which affects the connective tissue, is characterized histologically by a change in collagen synthesis with increased deposition, and dermal infiltration and thickening of the skin, asymmetric distribution without involvement of internal organs in this way (localized of the disease, but can lead, in some cases, important aesthetic and functional impairment of the patient. In our case, it is believed that the disease has already developed completely and now there is an overlap disease of old age, which makes their daily activities and independence.

  6. Localized scleroderma en coup de sabre in the Neurology Clinic.

    Science.gov (United States)

    Pinho, João; Rocha, João; Sousa, Filipa; Macedo, Cristiana; Soares-Fernandes, João; Cerqueira, João; Maré, Ricardo; Lourenço, Esmeralda; Pereira, João

    2016-07-01

    Localized scleroderma en coup de sabre (LScs) is a form of localized scleroderma thought to be an autoimmune disorder. Central nervous system involvement is not rare and neurological manifestations include seizures, focal neurological deficits, headache and neuropsychiatric changes. Patients attending the Neurology Clinic with the final diagnosis of LScs with neurological manifestations were identified and clinical and imagiological records reviewed. Five patients (0.024%) had LScs with neurological involvement, presenting with transient focal neurologic deficits, seizures, headache or migraine with aura. Neuroimaging studies confirmed localized skin depression and showed bone thinning, white matter lesions, brain calcifications, sulcal effacement and meningeal enhancement. Three patients experienced clinical improvement after immunosuppressive therapy, and in two of these patients neuroimaging findings also improved. Recognizing typical dermatologic changes is keystone for the diagnosis of LScs with neurological involvement. It is a diagnosis of exclusion and extensive etiological diagnostic evaluation should be performed. Treatment options, including conservative follow-up or immunosuppressive therapy, should be carefully considered. Copyright © 2016 Elsevier B.V. All rights reserved.

  7. Evaluation of heart rhythm variability and arrhythmia in children with systemic and localized scleroderma.

    Science.gov (United States)

    Wozniak, Jacek; Dabrowski, Rafal; Luczak, Dariusz; Kwiatkowska, Malgorzata; Musiej-Nowakowska, Elzbieta; Kowalik, Ilona; Szwed, Hanna

    2009-01-01

    To evaluate possible disturbances in autonomic regulation and cardiac arrhythmias in children with localized and systemic scleroderma. There were 40 children included in the study: 20 with systemic and 20 with localized scleroderma. The control group comprised 20 healthy children. In 24-hour Holter recording, the average rate of sinus rhythm was significantly higher in the groups with systemic and localized scleroderma than in the control group, but there was no significant difference between them. The variability of heart rhythm in both groups was significantly decreased. In the group with systemic scleroderma, single supraventricular ectopic beats were observed in 20% and runs were seen in 40% of patients. In the group with localized scleroderma, supraventricular single ectopic beats occurred in 35% of patients and runs in 45% of those studied. Ventricular arrhythmia occurred in 2 children with systemic scleroderma, but in 1 child, it was complex. The most frequent cardiac arrhythmias in both types of scleroderma in children were of supraventricular origin, whereas ventricular arrhythmias did not occur very often. There were no significant differences in autonomic disturbances manifesting as a higher heart rate and decreased heart rate variability between localized and systemic scleroderma.

  8. Measurement of transepidermal water loss in localized scleroderma.

    Science.gov (United States)

    Ďurčanská, Veronika; Jedličková, Hana; Vašků, Vladimír

    2016-05-01

    Localized scleroderma (LS) is a disease characterized by fibrotic changes in the dermis. Connective tissue growth factor and transforming growth factor β2 are the main mediators of fibrogenesis; this, along with excessive connective tissue production, affects epidermal keratinocytes, and thereby contributes to the changed quality of skin barrier. The objective of this article was to study the objective measurement of the skin barrier quality in LS with transepidermal water loss (TEWL) meter. The measurements of TEWL were performed on LS plaques in all three stages of various body locations. Control measurements were made on the contralateral side of healthy skin. The difference between TEWL in LS area and the contralateral side of the healthy skin was evaluated. A higher average TEWL 7.86 g/m(2) /h (SD 5.29) was observed on LS plaques compared with the control measurements on healthy skin 6.39 g/m(2) /h (SD 2.77). TEWL average values decreased from the inflammatory stage, through the sclerotic and to the atrophic stage. The mean difference 1.301 g/m(2) /h (SD 5.16) was found between TEWL on LS plaques and on the contralateral healthy skin in 82 measurements, i.e., a higher TEWL was observed in LS. The difference was statistically significant with p = 0.0250. Although fibrogenesis in scleroderma is localized in dermis, the skin barrier changes can be detected. © 2016 Wiley Periodicals, Inc.

  9. Prevalence of localized scleroderma in the Krasnodar territory

    Directory of Open Access Journals (Sweden)

    M. M. Tlish

    2015-01-01

    Full Text Available The authors conducted a study of particular features of the course of localized scleroderma in the south of Russia (Krasnodar territory. 65 case histories were analyzed. Women suffered from the disease 2.8 times more often than men; one half of the sample (55.4% included patients aged 35-55; the disease duration of over a year prevailed (69.8%; the disease occurred against the background of an endocrine pathology in one third of all cases; one third of all women developed the disease during the postmenopausal period. Plaque scleroderma (56.9%, white spot disease (von Zumbusch psoriasis (16.9% and idiopathic atrophoderma of Pasini and Pierini (12.3% belonged to the key clinical forms. The average number of lesions was 2.4 ± 1.2 while almost a half of the patients (47.7% had three or more lesions. The clinical manifestations localized mostly on the back (46.5% and stomach (34.9%.

  10. A Case of Localized Scleroderma in a Sculptor and His Wife

    Science.gov (United States)

    Bakst, Richard; Kovarik, Carrie; Werth, Victoria P.

    2011-01-01

    SUMMARY The etiology of localized scleroderma is unknown, and its pathogenetic relationship to its systemic counterpart is unclear. Environmental exposures, notably to silica dust, have long been suspected in the pathogenesis of the disorder. However, its’ relationship to the localized variant has not been well described. Here we present two cases of localized scleroderma in a sculptor and his wife who have extensive exposure to silica dust. PMID:19955998

  11. From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution.

    Science.gov (United States)

    Dilia, Giuggioli; Michele, Colaci; Emanuele, Cocchiara; Amelia, Spinella; Federica, Lumetti; Clodoveo, Ferri

    2018-01-01

    Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients. We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Eight (2.4%) female patients presented both the two diagnoses in their clinical histories. Six developed LoS prior to SSc; in 4/6 cases, the presence of autoantibodies was observed before SSc diagnosis. Overall, the median time interval between LoS and SSc diagnosis was 18 (range 0-156) months. LoS and SSc are two distinct clinical entities that may coexist. Moreover, as anecdotally reported in pediatric populations, we suggested the possible development of SSc in adult patients with LoS, particularly in presence of Raynaud's phenomenon or antinuclear antibodies before the SSc onset.

  12. Choroidal sclerosis in localized scleroderma (morphea en plaque).

    Science.gov (United States)

    Milenkovic, S; Petrovic, L; Risimic, D; Kosanovic-Jakovic, N; Jaksic, V; Djakovic, Z; Stojkovic, M; Risovic, D; Ivankovic, Lj; Ivancevic-Milenkovic, M

    2008-01-01

    Plaque morphea is a superficial type of morphea (localized scleroderma) which is characterized by various fibrotic areas of the dermis without systemic features. We present a 63-year-old man with morphea en plaque. The skin on his forearms and feet was taut, thickened and hidebound with scattered telangiectatic changes. Autoantibody profile was obtained and only ANA were positive (1:80). The patient had a decreased vision in the only functional, left eye. Our case is specific because the patient negated any kind of health problem, meaning the morphea and visual deterioration were of outstanding importance for him. Choroidal sclerosis and fundus appearance was extremely impressive and, to our knowledge, this is the first report of such unique case of ocular involvement in the literature. (c) 2008 S. Karger AG, Basel

  13. Alternatively activated macrophages (M2 macrophages) in the skin of patient with localized scleroderma.

    Science.gov (United States)

    Higashi-Kuwata, Nobuyo; Makino, Takamitsu; Inoue, Yuji; Takeya, Motohiro; Ihn, Hironobu

    2009-08-01

    Localized scleroderma is a connective tissue disorder that is limited to the skin and subcutaneous tissue. Macrophages have been reported to be particularly activated in patients with skin disease including systemic sclerosis and are potentially important sources for fibrosis-inducing cytokines, such as transforming growth factor beta. To clarify the features of immunohistochemical characterization of the immune cell infiltrates in localized scleroderma focusing on macrophages, skin biopsy specimens were analysed by immunohistochemistry. The number of cells stained with monoclonal antibodies, CD68, CD163 and CD204, was calculated. An evident macrophage infiltrate and increased number of alternatively activated macrophages (M2 macrophages) in their fibrotic areas were observed along with their severity of inflammation. This study revealed that alternatively activated macrophages (M2 macrophages) may be a potential source of fibrosis-inducing cytokines in localized scleroderma, and may play a crucial role in the pathogenesis of localized scleroderma.

  14. Juvenile Scleroderma

    Science.gov (United States)

    ... of the tongue. Obvious cases of en coup de sabre and Parry Romberg syndrome differ significantly, but many children present with cross-over manifestations, which can make it difficult to determine with certainty which form of ... en coup de sabre is mixed. If the lesions are confined ...

  15. Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma

    Directory of Open Access Journals (Sweden)

    Jolanta Budzyńska-Włodarczyk

    2016-02-01

    Full Text Available Introduction : Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components. Aim: To determine concentrations of interleukin (IL-27, transforming growth factor (TGF-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers. Material and methods: The study encompassed 17 females with localized scleroderma (aged 25–67. The control group consisted of 30 age-matched healthy women. The blood was sampled from the basilic vein. Serum levels of cytokines were determined using ELISA. Results : The TGF-β2 levels were found to be significantly lower in patients with localized scleroderma compared to controls. Concentrations of TGF-β1 were decreased in scleroderma patients when compared to controls but without statistical significance. There were no significant differences in serum IL-6, sIL-6R and IL-27 levels between patients and the control group; however, we found a significant positive correlation between the level of sIL-6 and ESR among subjects with localized scleroderma. Conclusions : The findings of decreased serum levels of TGF-β1 and TGF-β2 in patients with localized scleroderma demonstrate a possible association of these cytokines with pathogenesis of the disease. The results suggest also that sIL-6R is likely to be involved in inflammation in patients with localized scleroderma.

  16. Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma.

    Science.gov (United States)

    Budzyńska-Włodarczyk, Jolanta; Michalska-Jakubus, Małgorzata M; Kowal, Małgorzata; Krasowska, Dorota

    2016-02-01

    Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components. To determine concentrations of interleukin (IL)-27, transforming growth factor (TGF)-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers. The study encompassed 17 females with localized scleroderma (aged 25-67). The control group consisted of 30 age-matched healthy women. The blood was sampled from the basilic vein. Serum levels of cytokines were determined using ELISA. The TGF-β2 levels were found to be significantly lower in patients with localized scleroderma compared to controls. Concentrations of TGF-β1 were decreased in scleroderma patients when compared to controls but without statistical significance. There were no significant differences in serum IL-6, sIL-6R and IL-27 levels between patients and the control group; however, we found a significant positive correlation between the level of sIL-6 and ESR among subjects with localized scleroderma. The findings of decreased serum levels of TGF-β1 and TGF-β2 in patients with localized scleroderma demonstrate a possible association of these cytokines with pathogenesis of the disease. The results suggest also that sIL-6R is likely to be involved in inflammation in patients with localized scleroderma.

  17. Localized scleroderma: a clinical study at a single center in Korea.

    Science.gov (United States)

    Noh, Jung Won; Kim, Jinseok; Kim, Jae-Wang

    2013-08-01

    Localized scleroderma (morphea) is a rare autoimmune disease limited to the skin, characterized by cutaneous fibrosing and obstructive vasculopathy. Localized scleroderma may invade into the subcutaneous fat layer and cause permanent functional disability. Because of its rarity, there have been few clinical surveys of patients with localized scleroderma in Korea. The aim of this study was to elucidate the clinical presentation, serological data, and clinical outcomes of localized scleroderma. This was a retrospective survey conducted by reviewing available medical records during a 7 year-period from 2004 to 2010 in a single medical center in Jeju Island, South Korea. In total 43 patients with localized scleroderma were included. Localized scleroderma occurred primarily in females (female to male ratio 2.6 : 1.0). Most patients were between 10 and 29 years of age and the mean age at diagnosis was 26.2 years. Plaque (51.2%) and linear morphea (37.2%) were most common. No case was associated with systemic scleroderma (systemic sclerosis). The most common site of plaque morphea was the trunk (47.8%). In the linear type, the most common site was head-neck (52.9%). Fluorescent antinuclear antibody was positive in 23.3% of all cases. Treatment included systemic corticosteroids, colchicine, anti-malarial agents, D-penicillamine or intralesional triamcinolone injection. Clinical improvement, including significant and partial response, was seen in only 62.8% of treated patients. Localized scleroderma is a chronic inflammatory condition confined to the skin. In order to exclude other conditions, thorough history taking, physical examination, serologic studies and histopathologic examinations should be conducted. © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  18. [Localized eruptive juvenile xanthogranuloma].

    Science.gov (United States)

    Vanotti, S; Chiaverini, C; Rostain, G; Cardot-Leccia, N; Lacour, J-P

    2014-03-01

    Juvenile xanthogranuloma (JXG) is a non-Langerhans histiocytosis of young children characterized by solitary or multiple yellowish cutaneous nodules. Atypical skin lesions such as lichenoid eruptions, and pedunculated, maculopapular, plaque-like or linear lesions have been described. We report a case of eruptive XGJ en plaque in the left leg in an infant. A 13-month-old child presented asymptomatic eruptive, yellowish papules of the leg measuring 5 to 10mm since the age of 2months. There was no cutaneous infiltration between the lesions. Darier's sign was negative. Histological examination confirmed the diagnosis of JXG. The course of the disease comprised a gradual decrease in the number of active lesions with slight residual pigmentation. Our case was suggestive of JXG en plaque. Only 7 cases have been reported in the literature, all appearing before the age of 5months. The lesions corresponded mostly to an asymptomatic erythematous plaque studded with small yellowish/red nodules of variable localisation. Spontaneous involvement was noted in all cases. No systemic involvement was found. Herein we present a unique case of localised multiple JXG without evident clinical infiltrating plaque progressing with self-resolving flares. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  19. Detection of autoimmune antibodies in localized scleroderma by synthetic oligonucleotide antigens

    DEFF Research Database (Denmark)

    Samuelsen, Simone; Jørgensen, Christian Damsgaard; Mellins, Elizabeth D

    2018-01-01

    In this study, we developed a series of synthetic oligonucleotides that allowed us to investigate the details on the antigen recognition by autoimmune antibodies in localized scleroderma subjects. Besides dramatically improved analytical specificity of the assay, our data suggests a potential...... linking for antibodies to DNA to the biological status of disease state in localized scleroderma. Moreover, introducing chemical modifications into short synthetic deoxyribonucleic acid (DNA) and ribonucleic acid (RNA) molecules completely changed the binding titers of corresponding antibodies...... and their clinical relevance. The strongest observed effect was registered for the localized scleroderma skin damage index (LoSDI) on the IgG antibodies to TC dinucleotide-rich double-stranded antigen (p

  20. Health-related quality of life, optimism, and coping strategies in persons suffering from localized scleroderma.

    Science.gov (United States)

    Szramka-Pawlak, B; Dańczak-Pazdrowska, A; Rzepa, T; Szewczyk, A; Sadowska-Przytocka, A; Żaba, R

    2013-01-01

    The clinical course of localized scleroderma may consist of bodily deformations, and bodily functions may also be affected. Additionally, the secondary lesions, such as discoloration, contractures, and atrophy, are unlikely to regress. The aforementioned symptoms and functional disturbances may decrease one's quality of life (QoL). Although much has been mentioned in the medical literature regarding QoL in persons suffering from dermatologic diseases, no data specifically describing patients with localized scleroderma exist. The aim of the study was to explore QoL in localized scleroderma patients and to examine their coping strategies in regard to optimism and QoL. The study included 41 patients with localized scleroderma. QoL was evaluated using the SKINDEX questionnaire, and levels of dispositional optimism were assessed using the Life Orientation Test-Revised. In addition, individual coping strategy was determined using the Mini-MAC scale and physical condition was assessed using the Localized Scleroderma Severity Index. The mean QoL score amounted to 51.10 points, with mean scores for individual components as follows: symptoms = 13.49 points, emotions = 21.29 points, and functioning = 16.32 points. A relationship was detected between QoL and the level of dispositional optimism as well as with coping strategies known as anxious preoccupation and helplessness-hopelessness. Higher levels of optimism predicted a higher general QoL. In turn, greater intensity of anxious preoccupied and helpless-hopeless behaviors predicted a lower QoL. Based on these results, it may be stated that localized scleroderma patients have a relatively high QoL, which is accompanied by optimism as well as a lower frequency of behaviors typical of emotion-focused coping strategies.

  1. Thrombocytopenia Associated with Localized Scleroderma: Report of Four Pediatric Cases and Review of the Literature.

    Science.gov (United States)

    Jindal, Ankur Kumar; Gupta, Anju; Dogra, Sunil; Rawat, Amit; Suri, Deepti; Ahluwalia, Jasmina; Singh, Surjit

    2017-07-01

    We report on four children with localized scleroderma (morphea) and thrombocytopenia. All four had the en coup de sabre subtype of morphea and had varying degrees of thrombocytopenia (8 × 10 9 /L to 120 × 10 9 /L). None of them had major bleeding manifestations, and thrombocytopenia resolved with treatment of morphea. (One patient was also given an injection of anti-D immunoglobulin.) We propose that thrombocytopenia associated with localized scleroderma is usually benign and requires no specific therapy. © 2017 Wiley Periodicals, Inc.

  2. Successful autologous hematopoietic stem cell transplantation for a patient with rapidly progressive localized scleroderma.

    Science.gov (United States)

    Nair, Velu; Sharma, Ajay; Sharma, Sanjeevan; Das, Satyaranjan; Bhakuni, Darshan S; Narayanan, Krishnan; Nair, Vivek; Shankar, Subramanian

    2015-03-01

    Autologous hematopoietic stem cell transplant (HSCT) for rapidly progressive disease has not been reported in localized scleroderma. Our patient, a 16-year-old girl had an aggressive variant of localized scleroderma, mixed subtype (linear-generalized) with Parry Romberg syndrome, with no internal organ involvement, that was unresponsive to immunosuppressive therapy and was causing rapid disfigurement. She was administered autologous HSCT in June 2011 and has maintained drug-free remission with excellent functional status at almost 3.5 years of follow-up. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  3. Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk: A Danish Nationwide Cohort Study.

    Science.gov (United States)

    Hesselvig, Jeanette Halskou; Kofoed, Kristian; Wu, Jashin J; Dreyer, Lene; Gislason, Gunnar; Ahlehoff, Ole

    2018-03-13

    Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint. A total of 697 patients with localized scleroderma and 1,962 patients with systemic sclerosis were identified and compared with 5,428,380 people in the reference population. In systemic sclerosis, the adjusted HR was 2.22 (95% confidence interval 1.99-2.48). No association was seen between patients with localized scleroderma and cardiovascular disease. In conclusion, systemic sclerosis is a significant cardiovascular disease risk factor, while patients with localized scleroderma are not at increased risk of cardiovascular disease.

  4. Use of mycophenolate mofetil in patients with severe localized scleroderma resistant or intolerant to methotrexate

    NARCIS (Netherlands)

    Mertens, Jorre S.; Marsman, Diane; van de Kerkhof, Peter C M; Hoppenreijs, Esther P A H; Knaapen, Hanneke K A; Radstake, Timothy R D; de Jong, Elke M G J; Seyger, Marieke M B

    2016-01-01

    To assess the efficacy and safety of mycophenolate mofetil (MMF) in patients with localized scleroderma (LoS) resistant or intolerant to previous treatment with methotrexate (MTX). A case series of patients with LoS treated with MMF. Outcome was assessed through clinical examination. Adverse events

  5. Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases.

    Science.gov (United States)

    Matsushita, Takashi; Hasegawa, Minoru; Matsushita, Yukiyo; Echigo, Takeshi; Wayaku, Takamasa; Horikawa, Mayuka; Ogawa, Fumihide; Takehara, Kazuhiko; Sato, Shinichi

    2007-02-01

    Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.

  6. Pirfenidone gel in patients with localized scleroderma: a phase II study.

    Science.gov (United States)

    Rodríguez-Castellanos, Marco; Tlacuilo-Parra, Alberto; Sánchez-Enríquez, Sergio; Vélez-Gómez, Ezequiel; Guevara-Gutiérrez, Elizabeth

    2015-01-28

    Localized scleroderma is an inflammatory disease in its first stages and a fibrotic process in later stages, principally mediated by the transforming growth factor β. To date, there is no standard treatment. The objective of this study was to determine the effectiveness and safety of 8% pirfenidone gel in patients with localized scleroderma. This was an open phase II clinical trial that included 12 patients. Treatment with pirfenidone was indicated, three times daily for 6 months. Patients were evaluated clinically with the modified Localized Scleroderma Skin Severity Index (mLoSSI), as well with a durometer and histologically using hematoxylin and eosin stain and Masson's trichrome stain. The baseline mLoSSI average scores were 5.83 ± 4.80 vs. 0.83 ± 1.75 (P = 0.002) at 6 months. The initial durometer induration of the scleroderma plaques was 35.79 ± 9.10 vs. 32.47 ± 8.97 at 6 months (P = 0.05). We observed histopathological improvement with respect to epidermal atrophy, inflammation, dermal or adipose tissue fibrosis and annex atrophy from 12.25 ± 3.25 to 9.75 ± 4.35 (P = 0.032). The 8% pirfenidone gel application was well tolerated, and no side effects were detected. This is the first study on the therapeutic use of pirfenidone gel in localized scleroderma. It acts on both the inflammatory and the fibrotic phases. Considering its effectiveness, good safety profile and the advantage of topical application, pirfenidone is a treatment option in this condition.

  7. [Localized Scleroderma of Lower Extremities:Clinical and Magnetic Resonance Imaging Features].

    Science.gov (United States)

    Wang, Feng-dan; Wang, Hong-wei; Wu, Zhi-hong; Hou, Bo; Jiang, Bo; Zhang, Yan; Feng, Feng; Jin, Zheng-yu; Yuan, Xie

    2015-08-01

    To evaluate the clinical and musculoskeletal characteristics of localized scleroderma with lower extremities affected. All the localized scleroderma patients,who received magnetic resonance (MR ) examinations of affected lower extremities at Peking Union Medical College Hospital from April 2013 to June 2014,were retrospectively reviewed. Their clinical data and laboratory results of antinuclear antibody,anti-double stranded-DNA antibody, and anti-extractable nuclear antigen antibody were collected and analyzed. All the MR examinations were non-contrast imaging using Siemens Skyra 3.0T MR scanner. There were 16 localized scleroderma patients with lower extremities affected, 11 of whom were linear scleroderma, 4 generalized morphea, and 1 deep morphea. Female to male ratio was 1:2.2. The mean age was 22.5 years. The mean time span was 7.4 years. Four of the 14 patients (28.6%) who received antinuclear antibody test were positive. All the 10 patients who received anti-double stranded-DNA antibody test and the 7 patients who received anti-extractable nuclear antigen antibody test were negative. The most common musculoskeletal MR features were subcutaneous septal thickening (16/16) and fascial thickening (11/16). The thickened speta and fascia could either be hypointenstiy or hyperintensity on turbo inversion recovery magnitude/proton density weighted imaging. Other MR manifestations were intramuscular speta thickening (3/16), muscular abnormal signals (1/16), and bone marrow abnormal signals (2/16). Musculoskeletal manifestations of the lower extremities with localized scleroderma can be well revealed using MR imaging.

  8. Scleroderma in children: an update.

    Science.gov (United States)

    Zulian, Francesco; Cuffaro, Giorgio; Sperotto, Francesca

    2013-09-01

    Scleroderma, in its localized and systemic presentation, represents the third most frequent rheumatic condition in childhood after juvenile idiopathic arthritis and systemic lupus erythematosus. Early diagnosis, appropriate assessment and effective treatment are crucial to improve the long-term outcome. Recent studies, concerning histopathology and clinical associations with other conditions, open new horizons on the etiopathogenesis of scleroderma. New developments have been also reached in the field of outcome measures. In juvenile localized scleroderma (JLS), new techniques such as Doppler and laser Doppler imaging have shown their usefulness for the daily monitoring of the patients. In juvenile systemic sclerosis (JSSc), a new severity score has been developed and needs to be validated in future trials. Finally, a randomized, double-blind controlled trial, a multicenter consensus statement and long-term follow-up studies have confirmed the important role of methotrexate (MTX) for the treatment of JLS. Studies over recent years highlighted the role of imaging as outcome measures for JLS and introduced a severity score for JSSc. New studies on MTX confirmed its important role for the treatment of JLS.

  9. Scanning laser Doppler imaging may predict disease progression of localized scleroderma in children and young adults.

    Science.gov (United States)

    Shaw, L J; Shipley, J; Newell, E L; Harris, N; Clinch, J G; Lovell, C R

    2013-07-01

    Localized scleroderma is a rare but potentially disfiguring and disabling condition. Systemic treatment should be started early in those with active disease in key functional and cosmetic sites, but disease activity is difficult to determine clinically. Superficial blood flow has been shown to correlate with disease activity in localized scleroderma. To examine whether superficial blood flow measured by laser Doppler imaging (LDI) has the potential to predict disease progression and therefore select patients for early systemic treatment. A group of 20 individuals had clinical assessment and scanning LDI blood-flow measurements of 32 affected body sites. After a mean follow-up of 8.7 months their clinical outcome was compared with the results of the initial LDI assessment. Eleven out of 15 patients with an assessment of active LDI had progressed clinically, and 16 out of the 17 scans with inactive LDI assessment had not progressed, giving a positive predictive value of 73% and a negative predictive value of 94%. We believe that LDI can be a useful tool in predicting disease progression in localized scleroderma, and it may help clinicians to decide which patients to treat early. © 2013 The Authors BJD © 2013 British Association of Dermatologists.

  10. Scleroderma with Nodular Scleroderma

    Directory of Open Access Journals (Sweden)

    Chutika Srisuttiyakorn

    2016-11-01

    Full Text Available Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition. Although no additional treatment was given, the lesions remained stable without progression. Conclusions: Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques.

  11. [Using the polymerase chain reaction to Borrelia burgdorferi infection in localized scleroderma injure (morphea), in Venezuelan patients].

    Science.gov (United States)

    Espinoza-León, Fabiola; Arocha, Francisco; Hassanhi, Manzur; Arévalo, Julio

    2010-09-01

    Borrelia burgdorferi is the causative agent of Lyme Borreliosis, an infectious multisystemic disease transmitted to humans by the Ixodes ticks bite. A possible association of Borrelia burgdorferi with localized scleroderma has been postulated. However, published data do not provide unequivocal results. Previous serologic analysis of patients with localized scleroderma in South American countries (including Venezuela), have been reported as yielding some reactivity. The present study looked for evidence of Borrelia burgdorferi infection in venezuelan patients with localized scleroderma, using the polymerase chain reaction to analyze 21 skin samples of patients with this skin condition. The results were negative in all the samples studied. Our data do not support an association of Borrelia burgdorferi infection and the sclerotic lesions of localized scleroderma; but do not rule out the possibility of a relationship between localized scleroderma and an unknown geno-specie of Borrelia burgdorferi sensu lato complex, a different Borrelia specie or a different spirochetal organism, as the etiological agents of the skin lesions in this area.

  12. Imaging features in calcinosis circumscripta, a rare type of subcutaneous calcification in localized scleroderma

    Directory of Open Access Journals (Sweden)

    Pratiksha Yadav

    2013-01-01

    Full Text Available Calcinosis cutis circumscripta is a rare condition in which abnormal deposition of calcium seen in the dermis and subcutaneous tissue, it is associated with localized scleroderma. A 30-year-old female presented with an area of extensive calcification involving the right gluteal region, lateral aspect of right thigh and a small area on left thigh detected on radiograph with atrophy of subcutaneous tissue. Magnetic resonance imaging and computed tomography were done for further evaluation and the findings were of calcification and atrophy involving the skin and subcutaneous tissue.

  13. Esclerodermia localizada: Diagnósticos diferenciales Localized scleroderma: Differential diagnosis

    Directory of Open Access Journals (Sweden)

    MB Leroux

    2011-09-01

    Full Text Available La esclerodermia localizada constituye un desorden autoinmune órgano específico, que compromete sobre todo la piel. Se caracteriza por inflamación seguida de esclerosis e incluye distintas formas clínicas. La etiología de la esclerodermia localizada no ha sido establecida. El diagnóstico diferencial incluye cuadros esclerodermiformes, desencadenados por factores intrínsecos y extrínsecos que están siendo estudiados. Entre ellos se incluyen: exposición a radiaciones o tóxicos, consumo de medicamentos, infecciones y enfermedades de origen endocrino-metabólico, genético e inflamatorio. En primer lugar, se debe descartar la esclerodermia sistémica. En segundo lugar, se clasifican las entidades con predominio de esclerosis o atrofia. Por último, se incluyen algunas enfermedades en un cuadro comparativo.Localized scleroderma is an autoimmune organ specific disorder with an important skin compromise. It is an inflammatory process with several distinct clinical characteristics. The etiology of localized scleroderma has not been established yet. Differential diagnosis includes sclerodermiform onset unchained by intrinsic and extrinsic factors that are presently studied. Among them must be taking into account: exposure to radiation or toxic agents, therapeutic drugs, infections and diseases of endocrine, metabolic, genetic or inflammatory etiology. Firstly, it must be point out that systemic scleroderma must be ruled out. Secondly, disorders predominantly with sclerosis and atrophy must be classified and lastly, some other diseases are included in a comparative table.

  14. Systemic sclerosis and localized scleroderma--current concepts and novel targets for therapy.

    Science.gov (United States)

    Distler, Oliver; Cozzio, Antonio

    2016-01-01

    Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Skin and organ fibrosis are key manifestations of SSc, for which no generally accepted therapy is available. Thus, there is a high unmet need for novel anti-fibrotic therapeutic strategies in SSc. At the same time, important progress has been made in the identification and characterization of potential molecular targets in fibrotic diseases over the recent years. In this review, we have selected four targeted therapies, which are tested in clinical trials in SSc, for in depths discussion of their preclinical characterization. Soluble guanylate cyclase (sGC) stimulators such as riociguat might target both vascular remodeling and tissue fibrosis. Blockade of interleukin-6 might be particularly promising for early inflammatory stages of SSc. Inhibition of serotonin receptor 2b signaling links platelet activation to tissue fibrosis. Targeting simultaneously multiple key molecules with the multityrosine kinase-inhibitor nintedanib might be a promising approach in complex fibrotic diseases such as SSc, in which many partially independent pathways are activated. Herein, we also give a state of the art overview of the current classification, clinical presentation, diagnostic approach, and treatment options of localized scleroderma. Finally, we discuss whether the novel targeted therapies currently tested in SSc could be used for localized scleroderma.

  15. Mild Cognitive Impairment as a single sign of brain hemiatrophy in patient with Localized Scleroderma and Parry-Romberg Syndrome.

    Science.gov (United States)

    Klimiec, Elzbieta; Klimkowicz-Mrowiec, Aleksandra

    2016-01-01

    Neurologic involvement is well recognized in Systemic Scleroderma and increasingly reported in Localized Scleroderma. MRI brain abnormalities are often associated with symptoms such as seizures or headaches. In some cases they may be clinically silent. We describe a 23 years old female with head, trunk and limbs scleroderma who developed Parry-Romberg Syndrome. Brain MRI showed ipsilateral temporal lobe atrophy without any prominent neurologic symptoms. Neuropsychological examination revealed Mild Cognitive Impairment. During the 7 years of follow up we have noticed progression of face atrophy but no progression of brain atrophy. Cognitive functions have been stable. This case highlight that major MRI brain abnormalities in LS may occur with only subtle clinical manifestation such as Mild Cognitive Impairment. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  16. Systemic sclerosis complicated with localized scleroderma-like lesions induced by Köbner phenomenon.

    Science.gov (United States)

    Saigusa, Ryosuke; Asano, Yoshihide; Yamashita, Takashi; Takahashi, Takehiro; Nakamura, Kouki; Miura, Shunsuke; Ichimura, Yohei; Toyama, Tetsuo; Taniguchi, Takashi; Sumida, Hayakazu; Tamaki, Zenshiro; Miyazaki, Miki; Yoshizaki, Ayumi; Sato, Shinichi

    2018-03-01

    Scleroderma is a chronic disease of unknown etiology characterized by skin fibrosis and is divided into two clinical entities: systemic sclerosis (SSc) and localized scleroderma (LSc). In general, LSc is rarely complicated with SSc, but a certain portion of SSc patients manifests bilateral symmetric LSc-like lesions on the trunk and extremities. We investigated SSc patients with LSc-like lesions to clarify the underlying pathophysiology. Nine SSc cases complicated with LSc-like lesions were clinically and histologically characterized. SSc patients with LSc-like lesions exhibited multiple progressive hyper- and/or hypo-pigmented plaques with mild sclerosis symmetrically distributed on the trunk and extremities, especially abdominal region. In histological assessment, epidermal IL-1α expression was elevated in both forearms and LSc-like lesions of these patients to a greater extent than in forearms of control patients (SSc patients without LSc-like lesions). Of note, the infiltration and degranulation of mast cells were evident throughout the dermis of LSc-like lesions, while detectable to a lesser extent in forearms of SSc patients with LSc-like lesions and control patients. The epidermis of SSc patients with LSc-like lesions seems to possess an inflammatory phenotype, leading to the activation of mast cells in the dermis of mechanically stressed skin. Köbner phenomenon may be involved in the induction of LSc-like lesions in a certain subset of SSc. Copyright © 2017 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.

  17. Evaluation of medium-dose UVA1 phototherapy in localized scleroderma with the cutometer and fast Fourier transform method

    NARCIS (Netherlands)

    de Rie, M. A.; Enomoto, D. N. H.; de Vries, H. J. C.; Bos, J. D.

    2003-01-01

    Purpose: To evaluate the efficacy of medium-dose UVA1 phototherapy in patients with localized scleroderma. Method: A controlled pilot study with medium-dose UVA1 (48 J/cm(2)) was performed. The results were evaluated by means of a skin score and two objective methods for quantifying sclerosis

  18. The Localized Scleroderma Skin Severity Index and Physician Global Assessment of Disease Activity: A Work in Progress Toward Development of Localized Scleroderma Outcome Measures

    Science.gov (United States)

    ARKACHAISRI, THASCHAWEE; VILAIYUK, SOAMARAT; LI, SUZANNE; O’NEIL, KATHLEEN M.; POPE, ELENA; HIGGINS, GLORIA C.; PUNARO, MARILYNN; RABINOVICH, EGLA C.; ROSENKRANZ, MARGALIT; KIETZ, DANIEL A.; ROSEN, PAUL; SPALDING, STEVEN J.; HENNON, TERESA R.; TOROK, KATHRYN S.; CASSIDY, ELAINE; MEDSGER, THOMAS A.

    2013-01-01

    Objective To develop and evaluate a Localized Scleroderma (LS) Skin Severity Index (LoSSI) and global assessments’ clinimetric property and effect on quality of life (QOL). Methods A 3-phase study was conducted. The first phase involved 15 patients with LS and 14 examiners who assessed LoSSI [surface area (SA), erythema (ER), skin thickness (ST), and new lesion/extension (N/E)] twice for inter/intrarater reliability. Patient global assessment of disease severity (PtGA-S) and Children’s Dermatology Life Quality Index (CDLQI) were collected for intrarater reliability evaluation. The second phase was aimed to develop clinical determinants for physician global assessment of disease activity (PhysGA-A) and to assess its content validity. The third phase involved 2 examiners assessing LoSSI and PhysGA-A on 27 patients. Effect of training on improving reliability/validity and sensitivity to change of the LoSSI and PhysGA-A was determined. Results Interrater reliability was excellent for ER [intraclass correlation coefficient (ICC) 0.71], ST (ICC 0.70), LoSSI (ICC 0.80), and PhysGA-A (ICC 0.90) but poor for SA (ICC 0.35); thus, LoSSI was modified to mLoSSI. Examiners’ experience did not affect the scores, but training/practice improved reliability. Intrarater reliability was excellent for ER, ST, and LoSSI (Spearman’s rho = 0.71–0.89) and moderate for SA. PtGA-S and CDLQI showed good intrarater agreement (ICC 0.63 and 0.80). mLoSSI correlated moderately with PhysGA-A and PtGA-S. Both mLoSSI and PhysGA-A were sensitive to change following therapy. Conclusion mLoSSI and PhysGA-A are reliable and valid tools for assessing LS disease severity and show high sensitivity to detect change over time. These tools are feasible for use in routine clinical practice. They should be considered for inclusion in a core set of LS outcome measures for clinical trials. PMID:19833758

  19. Clinical and laboratory features of systemic sclerosis complicated with localized scleroderma.

    Science.gov (United States)

    Toki, Sayaka; Motegi, Sei-ichiro; Yamada, Kazuya; Uchiyama, Akihiko; Kanai, Sahori; Yamanaka, Masayoshi; Ishikawa, Osamu

    2015-03-01

    Localized scleroderma (LSc) primarily affects skin, whereas systemic sclerosis (SSc) affects skin and various internal organs. LSc and SSc are considered to be basically different diseases, and there is no transition between them. However, LSc and SSc have several common characteristics, including endothelial cell dysfunction, immune activation, and excess fibrosis of the skin, and there exist several SSc cases complicated with LSc during the course of SSc. Clinical and laboratory characteristics of SSc patients with LSc remain unclear. We investigated the clinical and laboratory features of 8 SSc patients with LSc among 220 SSc patients (3.6%). The types of LSc included plaque (5/8), guttate (2/8), and linear type (1/8). All cases were diagnosed as having SSc within 5 years before or after the appearance of LSc. In three cases of SSc with LSc (37.5%), LSc skin lesions preceded clinical symptoms of SSc. Young age, negative antinuclear antibody, and positive anti-RNA polymerase III antibody were significantly prevalent in SSc patients with LSc. The positivity of anticentromere antibody tended to be prevalent in SSc patients without LSc. No significant difference in the frequency of complications, such as interstitial lung disease, reflux esophagitis, and pulmonary artery hypertension, was observed. The awareness of these characteristic of SSc with LSc are essential to establish an early diagnosis and treatment. © 2015 Japanese Dermatological Association.

  20. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis.

    Science.gov (United States)

    Badea, I; Taylor, M; Rosenberg, A; Foldvari, M

    2009-03-01

    SSc is a chronic progressive disorder of unknown aetiology characterized by excess synthesis and deposition of collagen and other extracellular matrix components in a variety of tissues and organs. Localized scleroderma (LS) differs from SSc in that with LS only skin and occasionally subcutaneous tissues are involved. Although rarely life threatening, LS can be disfiguring and disabling and, consequently, can adversely affect quality of life. There is no known effective treatment for LS, and various options, including, as examples, corticosteroids and other immunomodulatory agents, ultraviolet radiation and vitamin D analogues, are of unproven efficacy. Clinical trials evaluating combination therapy such as corticosteroids with MTX or UVA1 exposure with psoralens have not been established as consistently effective. New immunomodulators such as tacrolimus and thalidomide are also being evaluated. A better understanding of the molecular and cellular mechanisms of LS has led to evaluation of new treatments that modulate profibrotic cytokines such as TGF-beta and IL-4, regulate assembly and deposition of extracellular matrix components, and restore Th1/Th2 immune balance by administering IL-12 or IFN-gamma. IFN-gamma acts by directly inhibiting collagen synthesis and by restoring immune balance. In this review, we evaluate current and future treatment options for LS and cutaneous involvement in SSc. Recent advances in therapy focus mainly on anti-fibrotic agents. Delivery of these drugs into the skin as the target tissue might be a key factor in developing more effective and safer therapy.

  1. Impact of a new simplified disability scoring system for adult patients with localized scleroderma.

    Science.gov (United States)

    Okiyama, Naoko; Asano, Yoshihide; Hamaguchi, Yasuhito; Jinnin, Masatoshi; Motegi, Sei-Ichiro; Koizumi, Haruka; Hasegawa, Minoru; Ishikawa, Osamu; Sato, Shinichi; Takehara, Kazuhiko; Yamamoto, Toshiyuki; Fujimoto, Manabu; Ihn, Hironobu

    2018-04-01

    Localized scleroderma (LoS) involves dermal but not internal inflammation and fibrosis. Cosmetic changes often impact quality of life (QOL), however, impairment of activities of daily living (ADL) in LoS patients has not been investigated. To determine what factor(s) are associated with ADL in adult patients with LoS, we performed a retrospective observational study in 177 Japanese adult LoS patients using a novel LoS disability score based on Barthel's indices of ADL: feeding, bathing, grooming, dressing, bowels, bladder, toilet use, transfers, mobility and stairs. LoS disability scores increased in proportion to the number of affected body parts but were not correlated to age and duration of illness. The presence of leg lesions significantly impaired ADL of LoS patients compared with lesions on other body parts. Patients treated with systemic medications, who tended to have multiple lesions, presented higher LoS disability scores than those without systemic treatments. Our study proposes that physicians evaluate ADL, not only QOL, in LoS patients. Our findings using LoS disability scoring indicate that multiple affected body parts and leg lesions are risk factors for ADL impairment. © 2018 Japanese Dermatological Association.

  2. microRNA-7 down-regulation mediates excessive collagen expression in localized scleroderma.

    Science.gov (United States)

    Etoh, Mitsuhiko; Jinnin, Masatoshi; Makino, Katsunari; Yamane, Keitaro; Nakayama, Wakana; Aoi, Jun; Honda, Noritoshi; Kajihara, Ikko; Makino, Takamitsu; Fukushima, Satoshi; Ihn, Hironobu

    2013-01-01

    Localized scleroderma (LSc), a connective tissue disorder restricted to the skin and subcutaneous tissue, is characterized by skin fibrosis due to an excessive deposition of types I collagen. The mechanism of such fibrosis is still unknown, but epigenetics may play some roles in the excessive collagen expression. In the present study, we investigated the mechanism of fibrosis seen in LSc, focusing on microRNA (miRNA). miRNA expression was determined by PCR array, real-time PCR, and in situ hybridization. The function of miRNA was evaluated using specific inhibitor. Immunoblotting was performed to detect α2(I) collagen protein. PCR array analysis using tissue miRNA demonstrated miR-7 level was significantly decreased in LSc skin as well as keloid tissue compared to normal skin in vivo. In situ hybridization also showed miR-7 expression in dermal fibroblasts was decreased in LSc dermis. The transfection of specific inhibitor for miR-7 into cultured normal dermal fibroblasts resulted in the up-regulation of α2(I) collagen protein in vitro. Also, the serum levels of miR-7 were significantly decreased in LSc patients compared with healthy controls, but serum miR-29a levels not. Systemic or local down-regulation of miR-7 may contribute to the pathogenesis of LSc via the overexpression of α2(I) collagen, and serum miR-7 may be useful as a disease marker. Investigation of the regulatory mechanisms of LSc by miRNA may lead to new treatments by the transfection into the lesional skin of this disease.

  3. Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach.

    Science.gov (United States)

    Marrani, Edoardo; Foeldvari, Ivan; Lopez, Jordi Anton; Cimaz, Rolando; Simonini, Gabriele

    2018-03-14

    Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years. Due to a lack of validated clinical criteria, four clinical response criteria were used to assess the treatment efficacy as primary outcome. We determined a combined estimate of the proportion of children responding to MTX and UVA. A total of 19 studies was included (8 MTX; 11 UVA). In the methotrexate group, 193 children were included in the analysis; in the phototherapy group, a total of 48 treated children. For both groups age, disease subtype, glucocorticoids (GCs) use, and side effects of treatment were also analyzed. The meta-analysis suggested that UVA and MTX protocols have both a favorable effect in active lesions of childhood localized scleroderma. However, MTX resulted significantly superior to UVA, with or without Psoralen. Our study supports the combination of MTX and GCs in patients with a high risk of complication. Phototherapy with UVA1 could represent a therapeutic option in patients with limited scleroderma, where lesions do not cross joints and they do not lead to potential cosmetic changes. Copyright © 2018 Elsevier Inc. All rights reserved.

  4. Interferon-gamma inducible protein-10 as a potential biomarker in localized scleroderma

    Science.gov (United States)

    2013-01-01

    Introduction The purpose of this study was to evaluate the presence and levels of interferon-gamma inducible protein-10 (IP-10) in the plasma and skin of pediatric localized scleroderma (LS) patients compared to those of healthy pediatric controls and to determine if IP-10 levels correlate to clinical disease activity measures. Methods The presence of IP-10 in the plasma was analyzed using a Luminex panel in 69 pediatric patients with LS and compared to 71 healthy pediatric controls. Of these patients, five had available skin biopsy specimens with concurrent clinical and serological data during the active disease phase, which were used to analyze the presence and location of IP-10 in the skin by immunohistochemistry (IHC). Results IP-10 levels were significantly elevated in the plasma of LS patients compared to that of healthy controls and correlated to clinical disease activity measures in LS. Immunohistochemistry staining of IP-10 was present in the dermal infiltrate of LS patients and was similar to that found in psoriasis skin specimens, the positive disease control. Conclusions Elevation of IP-10 levels in the plasma compared to those of healthy controls and the presence of IP-10 staining in the affected skin of LS patients indicates that IP-10 is a potential biomarker in LS. Furthermore, significant elevation of IP-10 in LS patients with active versus inactive disease and correlations between IP-10 levels and standardized disease outcome measures of activity in LS strongly suggest that IP-10 may be a biomarker for disease activity in LS. PMID:24499523

  5. The Localized Scleroderma Cutaneous Assessment Tool: responsiveness to change in a pediatric clinical population.

    Science.gov (United States)

    Kelsey, Christina E; Torok, Kathryn S

    2013-08-01

    Lack of agreement on how to accurately capture disease outcomes in localized scleroderma (LS) has hindered the development of efficacious treatment protocols. The LS Cutaneous Assessment Tool (LoSCAT), consisting of the modified LS Skin Severity Index (mLoSSI) and the LS Damage Index, has potential for use in clinical trials. The goal of this article is to further evaluate the clinical responsiveness of the LoSCAT. Based on the modifiable nature of disease activity versus damage, we expected the mLoSSI to be responsive to change. At 2 study visits, a physician completed the LoSCAT and Physician Global Assessment (PGA) of Disease Activity and of Disease Damage for 29 patients with LS. Spearman correlations were used to examine the relationships between the change in the LoSCAT and the PGA scores. To evaluate contrasted group validity, patients were grouped according to disease activity classification and change scores of groups were compared. Minimal clinically important differences were calculated and compared with the standard error of measurement. Change in the mLoSSI score correlated strongly with change in the PGA of Disease Activity score, whereas change in the LS Damage Index score correlated weakly with change in the PGA of Disease Damage score. The mLoSSI and PGA of Disease Activity exhibited contrasted group validity. Minimal clinically important differences for the activity measures were greater than the respective standard errors of measurement. Only 2 study visits were included in analysis. This study gives further evidence that the LoSCAT, specifically the mLoSSI, is a responsive, valid measure of activity in LS and should be used in future treatment studies. Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  6. Supernatants from culture of type I collagen-stimulated PBMC from patients with cutaneous systemic sclerosis versus localized scleroderma demonstrate suppression of MMP-1 by fibroblasts.

    Science.gov (United States)

    Brown, Monica; Postlethwaite, Arnold E; Myers, Linda K; Hasty, Karen A

    2012-06-01

    Systemic sclerosis (SSc) is a chronic fibrosing disease characterized by vasculopathy, autoimmunity, and an accumulation of collagen in tissues. Numerous studies have shown that compared to healthy or diseased controls, the peripheral blood mononuclear cells (PBMC) from patients with SSc produce a variety of cytokines or proliferate when cultured with solubilized type I collagen (CI) or constituent α1(II) and α2(I) polypeptide chains. The purpose of this study was to determine whether PBMC isolated from patients with SSc and cultured in vitro with soluble CI elaborated soluble mediators that inhibit the production of collagenase (i.e., matrix metalloproteinase, MMP-1) by fibroblasts. Supernatants of CI-stimulated PBMC from juvenile and adult diffuse cutaneous (dc)SSc patients significantly reduced MMP-1 production by SSc dermal fibroblasts, while supernatants of CI-stimulated PBMC from patients with localized scleroderma (LS) did not. CI-stimulated PBMC culture supernatants from patients with dcSSc in contrast to patients with LS exhibited increased levels of platelet-derived growth factor (PDGF)-AA, PDGF-BB, TNF-α, IL-13, and EGF. Prolonged culture of SSc dermal fibroblasts with recombinant PDGF-BB or IL-13 inhibited the induction of MMP-1 in response to subsequent TNF-α stimulation. These data suggest that therapies aimed at reducing these cytokines may decrease collagen accumulation in SSc, preventing the development of chronic fibrosis.

  7. Risk Factors for Scleroderma

    Science.gov (United States)

    ... You are here: Home For Patients Risk Factors Risk Factors for Scleroderma The cause of scleroderma is ... what biological factors contribute to scleroderma pathogenesis. Genetic Risk Scleroderma does not tend to run in families ...

  8. Lupus erythematosus and localized scleroderma coexistent at the same sites: a rare presentation of overlap syndrome of connective-tissue diseases.

    Science.gov (United States)

    Pascucci, Anabella; Lynch, Peter J; Fazel, Nasim

    2016-05-01

    Overlap syndromes are known to occur with connective-tissue diseases (CTDs). Rarely, the overlap occurs at the same tissue site. We report the case of a patient with clinical and histopathologic findings consistent with the presence of discoid lupus erythematosus (DLE) and localized scleroderma within the same lesions. Based on our case and other reported cases in the literature, the following features are common in patients with an overlap of lupus erythematosus (LE) and localized scleroderma: predilection for young women, photodistributed lesions, DLE, linear morphology clinically, and positivity along the dermoepidermal junction on direct immunofluorescence. Most patients showed good response to antimalarials, topical steroids, or systemic steroids.

  9. Quantitative Assessment of Skin Stiffness in Localized Scleroderma Using Ultrasound Shear-Wave Elastography.

    Science.gov (United States)

    Wang, Liyun; Yan, Feng; Yang, Yujia; Xiang, Xi; Qiu, Li

    2017-07-01

    The purpose of this study was to evaluate the usefulness of ultrasound shear-wave elastography (US-SWE) in characterization of localized scleroderma (LS), as well as in the disease staging. A total of 21 patients with 37 LS lesions were enrolled in this study. The pathologic stage (edema, sclerosis or atrophy) of the lesions was characterized by pathologic examination. The skin elastic modulus (E-values including E mean , E min , E max and E sd ) and thickness (h) was evaluated both in LS lesions and site-matched unaffected skin (normal controls) using US-SWE. The relative difference of E-values (E RD ) was calculated between each pair of lesions and its normal control for comparison among different pathologic stages. Of the 37 LS lesions, 2 were in edema, 22 were in sclerosis and 13 were in atrophy. US-SWE results showed a significant increase of skin elastic modulus and thickness in all lesions (p < 0.001 in sclerosis and p < 0.05 in atrophy) compared with the normal controls. The measured skin elastic modulus and thickness were greater in sclerosis than in atrophy. However, once normalized by skin thickness, the atrophic lesions, which were on average thinner, appeared significantly stiffer than those of the sclerosis (normalized E RD : an increase of 316.3% in atrophy vs. 50.6% in sclerosis compared with the controls, p = 0.007). These findings suggest that US-SWE allows for quantitative evaluation of the skin stiffness of LS lesions in different stages; however, the E-values directly provided by the US-SWE system alone do not distinguish between the stages, and the normalization by skin thickness is necessary. This non-invasive, real-time imaging technique is an ideal tool for assessing and monitoring LS disease severity and progression. Copyright © 2017 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

  10. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

    OpenAIRE

    Bonilla Abadía, Fabio; Muñoz Buitrón, Evelyn; Ochoa, Carlos D.; Carrascal, Edwin; Cañas Dávila, Carlos Alberto

    2012-01-01

    The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our kno...

  11. Scleroderma Mimickers

    Science.gov (United States)

    Morgan, Nadia D.; Hummers, Laura K.

    2017-01-01

    Opinion statement Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. PMID:28473954

  12. [When thinking to scleroderma?].

    Science.gov (United States)

    Cogan, E

    2007-09-01

    Scleroderma encompasses an heterogeneous group of autoimmune disorders characterized by an hidebound thickened skin involvement. When the changes are limited to the skin, localized scleroderma is suspected. Although the latter is most often a benign disease, it may be exceptionally associated with involvement of multiple organs, mainly the neurological system. At the opposite, systemic sclerosis is a serious disorder associated with high morbidity and even mortality and defined by an extended skin sclerosis, multiple organ involvement and general symptoms. Raynaud phenomena is nearly always present at the beginning of the disease. Identifying initial manifestations of the disease (Raynaud phenomena, diffuse non pitting edema, symmetrical polyarthritis with tendon friction rubs, dysphagia associated with mucosal telangiectasia) may allow the clinician to rapidly transfer the patient to a specialized reference center in order to organize a multidisciplinary approach and to prompt optimum therapy.

  13. Down-regulation of microRNA-196a in the sera and involved skin of localized scleroderma patients.

    Science.gov (United States)

    Makino, Takamitsu; Jinnin, Masatoshi; Etoh, Mitsuhiko; Yamane, Keitaro; Kajihara, Ikko; Makino, Katsunari; Ichihara, Asako; Igata, Toshikatsu; Sakai, Keisuke; Fukushima, Satoshi; Ihn, Hironobu

    2014-01-01

    Localized scleroderma (LSc) exhibits fibrosis of the skin and subcutaneous tissue. LSc shows an excessive deposition of type 1 collagen. To elucidate the mechanism of type 1 collagen overexpression in LSc, we investigated the epigenetics, focusing on microRNA (miRNA). miRNA expression profile was determined by PCR array analysis. The expression of microRNA-196a (miR-196a) in the skin tissue was examined by in situ hybridization or real-time PCR. The serum levels of miR-196a were measured by real-time PCR. PCR array analysis demonstrated that the miR-196a level was markedly decreased in LSc skin tissue in vivo. The transfection of specific inhibitor for miR-196a into normal cultured human dermal fibroblasts led to the up-regulation of type 1 collagen protein in vitro. Furthermore, the serum levels of miR-196a were significantly decreased in LSc patients. Down-regulation of miR-196a and subsequent overexpression of type 1 collagen in dermal fibroblasts may play a key role in the pathogenesis of LSc. The serum levels of miR-196a may be useful as a diagnostic marker of LSc.

  14. Corticosteroids in Myositis and Scleroderma

    Science.gov (United States)

    Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

    2017-01-01

    Synopsis Idiopathic inflammatory myopathies (IIM) involve inflammation of the muscles and are classified based on the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into four categories: dermatomyositis, polymyositis, inclusion body myositis, and immune mediated necrotizing myopathy. The term “scleroderma” refers to fibrosis of the skin. Localized scleroderma (morphea) is skin-limited, while systemic sclerosis (SSc) is associated with vascular and internal organ involvement. Although there is a paucity of randomized clinical trials, treatment with systemic corticosteroids (CS) is the standard of care for IIM with muscle and organ involvement. The extra-cutaneous features of systemic sclerosis are frequently treated with CS, however high doses have been associated with scleroderma renal crisis in high-risk patients. CS monotherapy is neither recommended for the cutaneous manifestations of dermatomyositis nor scleroderma. While CS can be effective first line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. PMID:26611554

  15. Localized morphea. A rare but significant secondary complication following breast cancer radiotherapy. Case report and review of the literature on radiation reaction among patients with scleroderma/morphea

    International Nuclear Information System (INIS)

    Herrmann, Thomas; Csere, Peter; Guenther, Claudia

    2009-01-01

    Purpose and approach: to report a case of morphea (localized scleroderma) in a patient following breast cancer therapy and to summarize the current literature. Results and conclusion: the occurrence of morphea is an unexpected late effect (approximately 1 year after the end of radiation therapy) which occurs frequently in the irradiated breast in women with breast-conserving therapy. The pathogenesis is unclear. The main differential diagnoses are recurrence of carcinoma and a radiogenic subcutaneous fibrosis (in most cases, the final diagnosis can only be made by means of a biopsy). Diagnosis and therapy must be performed in cooperation between dermatologist and radiooncologist. (orig.)

  16. Localized morphea. A rare but significant secondary complication following breast cancer radiotherapy. Case report and review of the literature on radiation reaction among patients with scleroderma/morphea

    Energy Technology Data Exchange (ETDEWEB)

    Herrmann, Thomas; Csere, Peter [Dept. of Radiotherapy and Radiooncology, Medical Faculty Carl Gustav Carus, Univ. of Technology, Dresden (Germany); Guenther, Claudia [Dept. of Dermatology, Medical Faculty Carl Gustav Carus, Univ. of Technology, Dresden (Germany)

    2009-09-15

    Purpose and approach: to report a case of morphea (localized scleroderma) in a patient following breast cancer therapy and to summarize the current literature. Results and conclusion: the occurrence of morphea is an unexpected late effect (approximately 1 year after the end of radiation therapy) which occurs frequently in the irradiated breast in women with breast-conserving therapy. The pathogenesis is unclear. The main differential diagnoses are recurrence of carcinoma and a radiogenic subcutaneous fibrosis (in most cases, the final diagnosis can only be made by means of a biopsy). Diagnosis and therapy must be performed in cooperation between dermatologist and radiooncologist. (orig.)

  17. Dental Care in Scleroderma

    Science.gov (United States)

    Dental Care in Scleroderma People living with scleroderma face unique challenges while trying to maintain their oral ... They are more likely to be affected by dental conditions such as small mouth, dry mouth, jaw ...

  18. Scleroderma and Sexuality

    Science.gov (United States)

    Scleroderma and Sexuality INTRODUCTION If you or your partner have been diagnosed with scleroderma, you may be wondering how this will ... will continue to find satisfaction and enjoyment through sexuality. If you are single, you may wonder how ...

  19. Pulmonary Hypertension in Scleroderma

    Science.gov (United States)

    PULMONARY HYPERTENSION IN SCLERODERMA PULMONARY HYPERTENSION Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. If the high ... the right side of the heart. Patients with scleroderma are at increased risk for developing PH from ...

  20. Localized scleroderma: assessment of the therapeutic response to phototherapy Esclerodermia cutânea: avaliação da resposta terapêutica à fototerapia

    Directory of Open Access Journals (Sweden)

    Roberta Buense

    2012-02-01

    Full Text Available BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES: This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS: Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS: Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS: The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound.FUNDAMENTOS: A esclerodermia é uma doença autoimune caracterizada pela esclerose progressiva do tecido conjuntivo e alterações da microcirculação. A forma cutânea é considerada uma doença autolimitada. No entanto, em alguns casos, ocorrem lesões atróficas, deformantes, que dificultam o desenvolvimento normal. Relatos da literatura apontam a fototerapia como uma modalidade terapêutica com resposta favorável nas formas cutâneas da esclerodermia. OBJETIVOS

  1. Pulsed dye laser in the treatment of localized scleroderma and its effects on CD34+ and factor XIIIa+ cells: an immunohistochemical study.

    Science.gov (United States)

    Tawfik, Abeer Attia; Shokir, Hisham; Soliman, Mona; Salah, Lila; Fathy, Sahar

    2013-06-01

    Localized scleroderma (morphea) is characterized by hardening and thickening of the dermis due to excessive collagen deposition. A decreased number of CD34+ cells and an increased number of Factor XIIIa+ cells are seen in the affected skin. The flashlamp pulsed dye laser (FLPDL) has been used in the treatment of localized morphea with promising results. The purpose of this study was to evaluate the therapeutic effectiveness of the pulsed dye laser in localized scleroderma and to assess its effect on CD34+ cells, Factor XIIIa+ cells, and blood vessels. Thirty patients with plaque morphea were treated with a FLPDL (585 nm wavelength, 450 μs pulse duration). Fluence ranged from 7.5 to 8.5 J/cm(2). Sessions were performed biweekly for a maximum of 6 months. Clinical, histopathologic, and immunohistochemical assessments were performed. Patients showed varying degrees of improvement of indurated skin. There was no worsening or further improvement at the treated sites during the follow-up assessments at 3, 6, and 12 months. An increased number of CD34+ cells were found in both the upper and the lower dermis, and a decreased number of Factor XIIIa+ cells were found in the lower dermis. The FLPDL is effective in the treatment of morphea, as confirmed by the changes in the pathologic tissue and levels of CD34+ and Factor XIIIa+ cells.

  2. Challenges and strategies for coping with scleroderma: implications for a scleroderma-specific self-management program.

    Science.gov (United States)

    Milette, Katherine; Thombs, Brett D; Maiorino, Kristina; Nielson, Warren R; Körner, Annett; Peláez, Sandra

    2018-05-09

    The purpose of this study was to explore challenges faced by patients with systemic sclerosis, also called scleroderma, in coping with their disease and the strategies they used to face those challenges. Five focus groups were held with scleroderma patients (4 groups, n = 34) and health care professionals who have experience treating scleroderma (1 group, n = 8). Participants' discussions were recorded, transcribed and analyzed using thematic analysis. Participants reported challenges accessing information (e.g., knowledgeable specialists), dealing with negative emotions (e.g., stress due to misunderstandings with loved ones), and accessing resources (e.g., helpful products or devices). Strategies for overcoming challenges were also discussed (e.g., advocating for own needs). When faced with significant challenges while coping with scleroderma, patients develop strategies to manage better and improve their quality of life. To help them cope, patients would benefit from easier access to supportive interventions, including tailored scleroderma self-management programs. Although the challenges experienced by patients with scleroderma are unique, findings from this study might help better understand patients' perspectives regarding coping and disease management for other chronic diseases as well. Implications for Rehabilitation People living with rare diseases, including the rare autoimmune disease scleroderma, face unique challenges and often do not have access to disease-specific educational or other support resources. People with scleroderma report that they face challenges in accessing information, including knowledgeable healthcare providers; managing difficult social interactions and negative emotions; and accessing resources. Strategies employed by scleroderma patients to overcome these challenges include seeking connections to other people with scleroderma or scleroderma patient organizations, actively seeking out local resources, and learning to

  3. Tips for Living with Scleroderma

    Science.gov (United States)

    ... Patients Tips for Living Tips for Living with Scleroderma Ways to help manage your symptoms The Scleroderma ... help find improved therapies and a cure for scleroderma! Your gift today will be matched to have ...

  4. Genetics Home Reference: systemic scleroderma

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Systemic scleroderma Systemic scleroderma Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Systemic scleroderma is an autoimmune disorder that affects the skin ...

  5. Unilateral Enophthalmos in Linear Scleroderma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Cho, So hyun; Hwang, Hee Young; Choi, Hye Young; Kim, Hyung Sik [Gachon University, Gil Hospital, Incheon (Korea, Republic of)

    2010-04-15

    Linear scleroderma is an uncommon subtype of localized scleroderma, which is characterized by a linear streak that crosses dermatomes and is associated with the tracking of fibrosis from the skin into deeper tissues, including muscle and fascia. A severe form of this condition sometimes causes growth atrophy of bone and supporting tissue in the affected area. Enophthalmos as a manifestation of linear scleroderma is very rare and occurs due to the replacement of orbital fat and muscle with collagen, which finally leads to atrophy of the affected orbit. This report introduces imaging findings of two cases of enophthalmos caused by linear scleroderma

  6. Unilateral Enophthalmos in Linear Scleroderma: A Case Report

    International Nuclear Information System (INIS)

    Cho, So hyun; Hwang, Hee Young; Choi, Hye Young; Kim, Hyung Sik

    2010-01-01

    Linear scleroderma is an uncommon subtype of localized scleroderma, which is characterized by a linear streak that crosses dermatomes and is associated with the tracking of fibrosis from the skin into deeper tissues, including muscle and fascia. A severe form of this condition sometimes causes growth atrophy of bone and supporting tissue in the affected area. Enophthalmos as a manifestation of linear scleroderma is very rare and occurs due to the replacement of orbital fat and muscle with collagen, which finally leads to atrophy of the affected orbit. This report introduces imaging findings of two cases of enophthalmos caused by linear scleroderma

  7. Paraneoplastic Scleroderma: Are There Any Clues?

    Science.gov (United States)

    Jedlickova, Hana; Durčanská, Veronika; Vašků, Vladimír

    2016-04-01

    localized scleroderma was seen in 3 patients and generalized localized scleroderma in one case. All patients had a histological picture consistent with scleroderma, negative ANA and ENA antibodies (Table 1, Figure 1). A 66-year-old woman presented with a 10 months history of sclerodermatous plaques on her neck, trunk, and upper and lower extremities. The skin on her breasts and cheeks was diffusely indurated. Examination showed thrombocytopenia, elevated transaminases, Cancer antigen 19-9 (Ca 19-9), thyroid stimulating hormone (TSH), and anti-thyroid peroxidase antibodies, dysmotility of the lower part of esophagus, hepatosplenomegaly, cholecystolithiasis, and benign polyps of colon. She was given prednisone 40 mg/day but did not return for follow up. After 6 months she was diagnosed with cholangiogenic carcinoma with metastatic disease and died shortly afterwards. A 74-year-old woman had localized scleroderma on the trunk for three years. She was treated with procaine penicillin for positive borrelia Immunoglobulin M (IgM) antibodies. Her condition worsened suddenly with confluent scleroderma plaques on her trunk, extremities, and genital region, and vasoneurosis on her lower extremities; she was started on prednisone 35 mg/day. Examination revealed endometrial cancer. The patient underwent a hysterectomy, adnexectomy, and radiotherapy with curative effect. Scleroderma patches softened with residual hyperpigmentation, and prednisone was stopped two years later. A 80-year-old man had a month-long history of diffuse thickening and toughening of the skin on the forearms and lower legs and scleroderma patches on the thighs and shins. Examination revealed prostate adenocarcinoma, and therapy with antiandrogen bicalutamide and prednisone 15 mg/day was started. Two years after the diagnosis he continues with bicalutamide treatment, prednisone 5 mg q.a.d. and has residual toughening of the skin on his lower legs. A 62-year-old woman with seronegative rheumatoid arthritis presented

  8. Assessment of esophageal involvement in systemic sclerosis and morphea (localized scleroderma) by clinical, endoscopic, manometric and pH metric features: a prospective comparative hospital based study.

    Science.gov (United States)

    Arif, Tasleem; Masood, Qazi; Singh, Jaswinder; Hassan, Iffat

    2015-02-15

    Systemic sclerosis (SSc) is a generalized disorder of unknown etiology affecting the connective tissue of the body. It affects the skin and various internal organs. Gastrointestinal tract involvement is seen in almost 90% of the patients. Esophagus is the most frequently affected part of the gastrointestinal tract. Esophageal motility disturbance classically manifests as a reduced lower esophageal sphincter pressure (LESP) and loss of distal esophageal body peristalsis. Consequently, SSc patients may be complicated by erosive esophagitis and eventually by Barrett's esophagus and esophageal adenocarcinoma. Morphea, also known as localized scleroderma, is characterized by predominant skin involvement, with occasional involvement of subjacent muscles and usually sparing the internal organs. The involvement of esophagus in morphea has been studied very scarcely. The proposed study will investigate the esophageal involvement in the two forms of scleroderma (systemic and localized), compare the same and address any need of upper gastrointestinal evaluation in morphea (localized scleroderma) patients. 56 and 31 newly and already diagnosed cases of SSc and morphea respectively were taken up for the study. All the patients were inquired about the dyspeptic symptoms (heartburn and/or acid regurgitation and/or dysphagia). Upper gastrointestinal endoscopy, esophageal manometry and 24-hour pH monitoring were done in 52, 47 and 41 patients of SSc; and 28, 25 and 20 patients of morphea respectively. Esophageal symptoms were present in 39 cases (69.6%) of SSc which were mild in 22 (39.3%), moderate in 14 (25%), severe in three (5.3%); while only four cases (7.1%) of morphea had esophageal symptoms all of which were mild in severity. Reflux esophagitis was seen in 17 cases (32.7%) of SSc and only two cases (7.14%) of morphea. Manometric abnormalities were seen in 32 cases (68.1%) of SSc and none in morphea. Ambulatory 24-hour esophageal pH monitoring documented abnormal reflux in

  9. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

    Science.gov (United States)

    Knobler, R; Moinzadeh, P; Hunzelmann, N; Kreuter, A; Cozzio, A; Mouthon, L; Cutolo, M; Rongioletti, F; Denton, C P; Rudnicka, L; Frasin, L A; Smith, V; Gabrielli, A; Aberer, E; Bagot, M; Bali, G; Bouaziz, J; Braae Olesen, A; Foeldvari, I; Frances, C; Jalili, A; Just, U; Kähäri, V; Kárpáti, S; Kofoed, K; Krasowska, D; Olszewska, M; Orteu, C; Panelius, J; Parodi, A; Petit, A; Quaglino, P; Ranki, A; Sanchez Schmidt, J M; Seneschal, J; Skrok, A; Sticherling, M; Sunderkötter, C; Taieb, A; Tanew, A; Wolf, P; Worm, M; Wutte, N J; Krieg, T

    2017-09-01

    The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum. © 2017 European Academy of Dermatology and Venereology.

  10. Conformal radiotherapy for locally advanced juvenile nasopharyngeal angio-fibroma

    Directory of Open Access Journals (Sweden)

    Supriya Mallick

    2015-01-01

    Full Text Available Purpose: To assess the efficacy of radiation in the treatment of juvenile nasopharyngeal angiofibroma (JNA. Materials and Methods: Data were retrieved for JNA treated with radiotherapy from 1987-2012. The demographics, treatment and outcome data were recorded in predesigned proforma. Results: Data of 32 patients were retrieved. Median age was 17 years (range: 12-33 years. All patients received radiation because of refractory, residual or unresectable locally advanced disease. All patients were planned with a three-dimensional conformal technique (3DCRT. The median radiation dose was 30 Gray (range: 30-45 Gray. Median follow-up was 129 months (range: 1-276 months. At the last follow-up, 13 patients were found to have a radiological complete response. Two patients progressed 38 and 43 months after completion of treatment and opted for alternative treatment. One patient developed squamous cell carcinoma of the nasal ale 15 years after radiation. Conclusion: Conformal radiotherapy shows promise as an alternative treatment approach for locally advanced JNA and confers long-term disease control with minimal toxicity.

  11. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.

    Science.gov (United States)

    Ferreli, Caterina; Gasparini, Giulia; Parodi, Aurora; Cozzani, Emanuele; Rongioletti, Franco; Atzori, Laura

    2017-12-01

    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct

  12. Scleroderma Research Foundation

    Science.gov (United States)

    ... will continue. Learn more The SRF: A Four Star Charity The SRF has achieved the highest possible ... Saget share about his connection to scleroderma and what he is doing to make a difference. Click ...

  13. Neurological abnormalities in localized scleroderma of the face and head: a case series study for evaluation of imaging findings and clinical course.

    Science.gov (United States)

    Lis-Święty, Anna; Brzezińska-Wcisło, Ligia; Arasiewicz, Hubert

    2017-09-01

    Localized scleroderma (LoS) of the face and head is often associated with neurological manifestations and/or imaging abnormalities in the central nervous system (CNS). We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e.g. headaches), serious neurological complications probably in the course of vasculitis were revealed: epilepsy (in two patients), epilepsy and pyramidal sings (in one patient). Neurological disorders and LoS occurred at the same time (in three patients) or at the course of the disease (nine patients) and no later than 29 years since the onset of the disease. No link between neurological disorders and the LoS clinical morphology, immunological and other laboratory parameters has been established. CNS involvement is not correlated with the clinical course of the facial and head LoS and may occur years after the disease initial symptomatology. Imaging follow-up is not required if there is not any emerging neurological symptom. In some cases, however, both HRCT and MRI are useful for monitoring disease evolution and addressing therapeutic choices.

  14. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

    Science.gov (United States)

    Bonilla-Abadía, Fabio; Muñoz-Buitrón, Evelyn; Ochoa, Carlos D; Carrascal, Edwin; Cañas, Carlos A

    2012-12-20

    The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  15. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

    Directory of Open Access Journals (Sweden)

    Bonilla-Abadía Fabio

    2012-12-01

    Full Text Available Abstract Background The localized scleroderma (LS known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  16. Fractional carbon dioxide laser versus low-dose UVA-1 phototherapy for treatment of localized scleroderma: a clinical and immunohistochemical randomized controlled study.

    Science.gov (United States)

    Shalaby, S M; Bosseila, M; Fawzy, M M; Abdel Halim, D M; Sayed, S S; Allam, R S H M

    2016-11-01

    Morphea is a rare fibrosing skin disorder that occurs as a result of abnormal homogenized collagen synthesis. Fractional ablative laser resurfacing has been used effectively in scar treatment via abnormal collagen degradation and induction of healthy collagen synthesis. Therefore, fractional ablative laser can provide an effective modality in treatment of morphea. The study aimed at evaluating the efficacy of fractional carbon dioxide laser as a new modality for the treatment of localized scleroderma and to compare its results with the well-established method of UVA-1 phototherapy. Seventeen patients with plaque and linear morphea were included in this parallel intra-individual comparative randomized controlled clinical trial. Each with two comparable morphea lesions that were randomly assigned to either 30 sessions of low-dose (30 J/cm 2 ) UVA-1 phototherapy (340-400 nm) or 3 sessions of fractional CO 2 laser (10,600 nm-power 25 W). The response to therapy was then evaluated clinically and histopathologically via validated scoring systems. Immunohistochemical analysis of TGF-ß1 and MMP1 was done. Patient satisfaction was also assessed. Wilcoxon signed rank test for paired (matched) samples and Spearman rank correlation equation were used as indicated. Comparing the two groups, there was an obvious improvement with fractional CO 2 laser that was superior to that of low-dose UVA-1 phototherapy. Statistically, there was a significant difference in the clinical scores (p = 0.001), collagen homogenization scores (p = 0.012), and patient satisfaction scores (p = 0.001). In conclusion, fractional carbon dioxide laser is a promising treatment modality for cases of localized morphea, with proved efficacy of this treatment on clinical and histopathological levels.

  17. Mimics of scleroderma

    Directory of Open Access Journals (Sweden)

    Kaveri K Nalianda

    2017-01-01

    Full Text Available Systemic sclerosis is a rare autoimmune connective tissue disorder characterised typically by tightening and tethering of skin. However, several other disorders are also characterised by hardening and thickening of skin. These mimics can be potentially confused with systemic sclerosis, leading to a misdiagnosis. This review describes the aetiopathogenesis, clinical features and treatment of Morphea (localised scleroderma, Scleredema, Scleromyxoedema, Eosinophilic fasciitis, Nephrogenic Systemic Fibrosis, Diabetic Cheiroarthropathy, chronic GVHD, POEMS syndrome and drug induced scleroderma like illness. A careful and thorough clinical assessment is essential in order to differentiate these mimics from each other and from systemic sclerosis, establish the diagnosis, and initiate appropriate treatment.

  18. Development and initial validation of the Localized Scleroderma Skin Damage Index and Physician Global Assessment of disease Damage: a proof-of-concept study

    Science.gov (United States)

    Vilaiyuk, Soamarat; Torok, Kathryn S.; Medsger, Thomas A.

    2010-01-01

    Objective. To develop and assess the psychometric properties of the Localized Scleroderma (LS) Skin Damage Index (LoSDI) and Physician Global Assessment of disease Damage (PGA-D). Methods. Damage was defined as irreversible/persistent changes (>6 months) due to previous active disease/complications of therapy. Eight rheumatologists assessed the importance of 17 variables in formulating the PGA-D/LoSDI. LS patients were evaluated by two rheumatologists using both tools to assess their psychometric properties. LoSDI was calculated by summing three scores for cutaneous features of damage [dermal atrophy (DAT), subcutaneous atrophy (SAT) and dyspigmentation (DP)] measured at 18 anatomic sites. Patient GA of disease severity (PtGA-S), Children's Dermatology Life Quality Index (CDLQI) and PGA-D were recorded at the time of each examination. Results. Thirty LS patients (112 lesions) and nine patient-visit pairs (18 lesions) were included for inter- and intra-rater reliability study. LoSDI and its domains DAT, SAT, DP and PGA-D demonstrated excellent inter- and intra-rater reliability (reliability coefficients 0.86–0.99 and 0.74–0.96, respectively). LoSDI correlated moderately with PGA-D and poorly with PtGA-S and CDLQI. PGA-D correlated moderately with PtGA-S, but poorly with CDLQI. Conclusions. To complete the LS Cutaneous Assessment Tool (LoSCAT), we developed and evaluated the psychometric properties of the LoSDI and PGA-D in addition to the LS Skin Severity Index (LoSSI). These instruments will facilitate evaluation of LS patients for individual patient management and clinical trials. LoSDI and PGA-D demonstrated excellent reliability and high validity. LoSCAT provides an improved understanding of LS natural history. Further study in a larger group of patients is needed to confirm these preliminary findings. PMID:20008472

  19. The downregulation of microRNA let-7a contributes to the excessive expression of type I collagen in systemic and localized scleroderma.

    Science.gov (United States)

    Makino, Katsunari; Jinnin, Masatoshi; Hirano, Ayaka; Yamane, Keitaro; Eto, Mitsuhiko; Kusano, Takamitsu; Honda, Noritoshi; Kajihara, Ikko; Makino, Takamitsu; Sakai, Keisuke; Masuguchi, Shinichi; Fukushima, Satoshi; Ihn, Hironobu

    2013-04-15

    Systemic and localized scleroderma (SSc and LSc) is characterized by excessive deposition of collagen and tissue fibrosis in the skin. Although they have fundamental common characteristics including autoimmunity, little is known about the exact mechanism that mediates the excessive collagen expression in these disorders. In the current study, we tried to evaluate the possibility that microRNAs (miRNAs) play some roles in the pathogenesis of fibrosis seen in these diseases. miRNA expression patterns were evaluated by miRNA array analysis, real-time PCR, and in situ hybridization. The function of miRNAs in dermal fibroblasts was assessed using miRNA inhibitors, precursors, or protectors. In the mouse model of bleomycin-induced dermal sclerosis, the overexpression of miRNAs was performed by i.p. miRNA injection. We demonstrated let-7a expression was downregulated in SSc and LSc skin both in vivo and in vitro, compared with normal or keloid skin. The inhibition or overexpression of let-7a in human or mouse skin fibroblasts affected the protein expression of type I collagen or luciferase activity of collagen 3'-untranslated region. Also, we found let-7a was detectable and quantitative in the serum and investigated serum let-7a levels in patients with SSc or LSc. let-7a concentration was significantly decreased in these patients, especially in LSc patients. Moreover, we revealed that the intermittent overexpression of let-7a in the skin by i.p. miRNA injection improved the skin fibrosis induced by bleomycin in mice. Investigation of more detailed mechanisms of miRNA-mediated regulation of collagen expression may lead to new therapeutic approaches against SSc and LSc.

  20. Coexistence of lichen sclerosus and morphea: a retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center.

    Science.gov (United States)

    Kreuter, Alexander; Wischnewski, Jana; Terras, Sarah; Altmeyer, Peter; Stücker, Markus; Gambichler, Thilo

    2012-12-01

    The coexistence of lichen sclerosus (LiS) and localized scleroderma (LoS) has sporadically been reported in the literature. Recently, a prospective multicenter study demonstrated a surprisingly high percentage of genital LiS in patients with morphea. The aim of this study was to determine the prevalence of LiS in a cohort of patients with LoS who presented at a tertiary referral medical center for connective tissue diseases in Germany. We retrospectively evaluated the prevalence of genital and extragenital LiS in adult and pediatric patients with different subtypes of LoS. Secondary outcome measures included demographic characteristics and prevalence of other concomitant autoimmune diseases. Of the 472 patients (381 adults, 91 children; mean age: 46 years; range, 4-88 years; female to male ratio: 3.5:1 in adults and 8:1 in children) with LoS, 27 (5.7%) also presented with LiS (19 extragenital and 8 genital lesions). LiS exclusively occurred in patients with plaque-type (morphea) and generalized LoS. Twenty-six of the 27 (96.2%) patients with concomitant LoS and LiS were adults. Compared with LiS in the general population, LiS was significantly more frequent in LoS as indicated by an odds ratio of 18.1 (95% confidence interval 2.6-134.2; P morphea, should be carefully screened for concomitant LiS, including inspection of the anogenital region. Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  1. Malignancy in scleroderma patients from south west England: a population-based cohort study.

    LENUS (Irish Health Repository)

    Siau, Keith

    2010-01-08

    The pathophysiological relationship between scleroderma and malignancy remains poorly understood. Although some previous studies have demonstrated an increased malignancy risk in patients with scleroderma, others have been inconclusive. We aimed to determine if patients with scleroderma had an increased risk of malignancy compared to an age- and sex-matched local South West England population, and if there were any important differences between scleroderma patients with and without malignancy. Methods of this study are as follows. Notes were obtained on all local scleroderma patients (n = 68) locally, and those diagnosed with malignancy verified by contacting each patient\\'s general practitioner. Expected malignancy figures were obtained from age- and sex-stratified regional prevalence data provided by the South West Cancer Intelligence Service registry. Among the patients, 22.1% with scleroderma were identified with concurrent malignancy. Affected sites were of the breast (n = 5), haematological system (n = 5), skin (n = 4), and unknown primary (n = 1). Overall, malignancy risk was found to be increased in scleroderma (RR = 3.15, 95% CI 1.77-5.20, p = 0.01). In particular, this risk was the highest for haematological malignancies (RR = 18.5, 95% CI 6-43, p = 0.03), especially for non-Hodgkin\\'s lymphoma (RR = 25.8, 95% CI 5-75, p = 0.10). The majority of patients (86.7%) developed malignancy after the onset of scleroderma (mean = 6.9 years). Age of >70 and patients with limited scleroderma were significant risk factors for a patient with scleroderma to have a concurrent malignancy; however, no increased risk was found in patients with any particular pattern of organ involvement, cytotoxic usage or serology. To conclude, in this small patient cohort, we have found that scleroderma is associated with an increased risk of malignancy. This risk is statistically significant in patients with limited scleroderma. Patients who are elderly and those with limited disease

  2. Esclerodermia localizada na criança: aspectos clínicos, diagnósticos e terapêuticos Localized scleroderma in children: clinical, diagnostic and therapeutic aspects

    Directory of Open Access Journals (Sweden)

    Pedro C. Q. Zancanaro

    2009-04-01

    Full Text Available A esclerodermia localizada, ou morféia, acomete crianças em idade escolar e, em geral, é autolimitada, apesar de localmente desfiguradora. A literatura descreve inúmeros fatores etiopatogênicos, bem como modalidades de investigação e tratamento. Este artigo reúne os estudos mais recentes e discute sua aplicação clínica.Localized scleroderma or morphea affects school-aged children, is usually self-limited and a disfiguring condition. Several etiopathogenic factors, investigations and treatment options are described. This article reviews the recent literature and discusses its clinical applications.

  3. Scleroderma: a case report

    Science.gov (United States)

    Harahap, T. A.; Marpaung, B.

    2018-03-01

    Scleroderma is a complex disease in which extensive fibrosis, vascular alterations, and autoantibodies against various cellular antigens are among the principal features.[1,2] The prevalenceranging from 50 to 300 cases per 1 million persons with women are at much higher risk. The average age of diagnosis is the fifth decades of life.[2] There is no cure for scleroderma, but many of its problems and complications can be treated.[3-7]A 54-year-old female patient with main complains limitation of motion and mouth, stiffness and painful joints in the hands and feet, thickening on the skin in the chest and trunk for eight years, purplish red spots on arms and legs intermittent for tenyears. On physical examination found sclerosis lesions, sclerodactyly on fingers and toes, telangiectasias in the antebrachii and cruris region. On laboratory, examination showed ANA test 10.7 and Anti DS DNA 123. The histopathological of the skin result is scleroderma. The patient was diagnosed with scleroderma and treated with methotrexate 7.5 mg/weeks, ciclosporin 2×100 mg/day, omeprazole 2×20 mg. After seven days of therapy, there is aclinical improvement, and the patient becomes anoutpatient treatment.

  4. Prospective study to evaluate the clinical and radiological outcome of patients with scleroderma of the face.

    Science.gov (United States)

    Careta, Mariana Figueiroa; Leite, Claudia da Costa; Cresta, Fernando; Albino, Jose; Tsunami, Mirian; Romiti, Ricardo

    2013-09-01

    Scleroderma featuring rare connective tissue disease that manifests as skin sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement and localized scleroderma or morphea which classically presents benign evolution and self-limited, confined to the skin and/or underlying tissue. Recent studies show that the localized form may possibly course with involvement of internal organs and variable morbidity. This study aimed to determine the demographic characteristics, the prevalence of systemic manifestations and laboratory findings, as well as the association with autoimmune diseases, and the evolution of neurological findings, both clinical as brain MRI in patients with scleroderma of the face and its relation with the activity skin. Patients with localized scleroderma with facial involvement were evaluated and underwent neurological examination, magnetic resonance imaging and ophthalmology evaluation. After 3years, the patients were subjected again to MRI. We studied 12 patients with localized scleroderma of the face. Of this total, headache being the most frequent complaint found in 66.7% of patients, 33.3% had neurological changes possibly associated with scleroderma. As for ophthalmologic evaluation, 25% of patients showed abnormalities. The most frequent parenchymal finding was the presence of lesions with hyperintense or hypointense signal in 75% of patients, followed by ventricular asymmetry at 16.7%. Of the patients who had neurological deficits, 75% also had a change to MRI. In all patients, imaging findings after 3years were unchanged. During this interval of 3years, 25% of patients showed signs of activity of scleroderma. Patients with localized scleroderma of the face have a high prevalence of neurological and ophthalmological changes. Based on these findings, we suggest that all cases of localized scleroderma of the face should be thoroughly

  5. Evaluation of liver function tests in scleroderma patients.

    Science.gov (United States)

    Salem, Gehan Ibrahim Abdelrazek; Abdulrahman, Awni Ali

    2012-08-01

    Systemic sclerosis is a clinically heterogeneous, systemic disorder which affects the connective tissue of the skin, internal organs, and the walls of blood vessels. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen and other matrix substances in the connective tissue. This study was done to evaluate the frequency of liver disease in patients with scleroderma and, secondarily, to study the frequency of infection of hepatitis B and C virus in these patients and determine frequency of serum auto-antibodies in this disease. We studied patients with scleroderma, localized or systemic, in the outpatient clinic of rheumatology and dermatology departments, at King Khalid University Hospital. As for a comparison, healthy persons coming to the clinic with the same mean age were considered as control group. Forty patients with the diagnosis of scleroderma included in this work, 35% had elevated gamma-glutamyl-transferase (γ-GT), 30% had elevated alkaline phosphatase (AP) and in 17.5%, the alanine-amino-transferase (ALT) was above the reference values. The ALT had changed to be more in scleroderma patients than in controls. Twenty percent (20%) of the patients tested positive for anti-smooth muscle antibodies (anti-SMA) and only one patient had anti-mitochondrial antibodies (AMA). There was no statistical difference between the two groups regarding antibody testing. Anti-HCV antibodies were observed in one patient, and HBsAg was detected in another scleroderma patient. There was no patient with clinically significant hepatic disease. In this study, although changes in liver enzymes in patients with scleroderma were not uncommon, there was no scleroderma patient with clinical manifestations of liver disease.

  6. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland.

    Science.gov (United States)

    Herrick, Ariane L; Ennis, Holly; Bhushan, Monica; Silman, Alan J; Baildam, Eileen M

    2010-02-01

    Childhood scleroderma encompasses a rare, poorly understood spectrum of conditions. Our aim was to ascertain the incidence of childhood scleroderma in its different forms in the UK and Ireland, and to describe the age, sex, and ethnicity of the cases. The members of 5 specialist medical associations including pediatricians, dermatologists, and rheumatologists were asked to report all cases of abnormal skin thickening suspected to be localized (including linear) scleroderma or systemic sclerosis (SSc) in children scleroderma and 7 (7%) with SSc. This gave an incidence rate per million children per year of 3.4 (95% confidence interval [95% CI] 2.7-4.1) for localized scleroderma, including an incidence rate of 2.5 (95% CI 1.8-3.1) for linear scleroderma, and 0.27 (95% CI 0.1-0.5) for SSc. Of the 87 localized cases, 62 (71%) had linear disease. Of localized disease cases, 55 (63%) were female, 71 (82%) were classified as white British, and the patients' mean age when first seen in secondary care was 10.4 years. Of the 7 SSc cases, all were female, 6 (86%) were white British, and the mean age when first seen was 12.1 years. The median delay between onset and being first seen was 13.1 months for localized scleroderma and 7.2 months for SSc. These data provide additional estimates of the incidence of this rare disorder and its subforms.

  7. Neurologic involvement in scleroderma: a systematic review.

    Science.gov (United States)

    Amaral, Tiago Nardi; Peres, Fernando Augusto; Lapa, Aline Tamires; Marques-Neto, João Francisco; Appenzeller, Simone

    2013-12-01

    To perform a systematic review of neurologic involvement in Systemic sclerosis (SSc) and Localized Scleroderma (LS), describing clinical features, neuroimaging, and treatment. We performed a literature search in PubMed using the following MeSH terms, scleroderma, systemic sclerosis, localized scleroderma, localized scleroderma "en coup de sabre", Parry-Romberg syndrome, cognitive impairment, memory, seizures, epilepsy, headache, depression, anxiety, mood disorders, Center for Epidemiologic Studies Depression (CES-D), SF-36, Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Patient Health Questionnaire-9 (PHQ-9), neuropsychiatric, psychosis, neurologic involvement, neuropathy, peripheral nerves, cranial nerves, carpal tunnel syndrome, ulnar entrapment, tarsal tunnel syndrome, mononeuropathy, polyneuropathy, radiculopathy, myelopathy, autonomic nervous system, nervous system, electroencephalography (EEG), electromyography (EMG), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Patients with other connective tissue disease knowingly responsible for nervous system involvement were excluded from the analyses. A total of 182 case reports/studies addressing SSc and 50 referring to LS were identified. SSc patients totalized 9506, while data on 224 LS patients were available. In LS, seizures (41.58%) and headache (18.81%) predominated. Nonetheless, descriptions of varied cranial nerve involvement and hemiparesis were made. Central nervous system involvement in SSc was characterized by headache (23.73%), seizures (13.56%) and cognitive impairment (8.47%). Depression and anxiety were frequently observed (73.15% and 23.95%, respectively). Myopathy (51.8%), trigeminal neuropathy (16.52%), peripheral sensorimotor polyneuropathy (14.25%), and carpal tunnel syndrome (6.56%) were the most frequent peripheral nervous system involvement in SSc. Autonomic neuropathy involving cardiovascular and gastrointestinal systems was regularly described

  8. Scleroderma, Stress and CAM Utilization

    Directory of Open Access Journals (Sweden)

    Ka-Kit Hui

    2009-01-01

    Full Text Available Scleroderma is an autoimmune disease influenced by interplay among genetic and environmental factors, of which one is stress. Complementary and alternative medicine (CAM is frequently used to treat stress and those diseases in which stress has been implicated. Results are presented from a survey of patients with scleroderma. Respondents were a convenient sample of those attending a national conference in Las Vegas in 2002. Findings implicate stress in the onset, continuation and exacerbation of scleroderma. The implication is that CAM providers may be filling an important patient need in their provision of services that identify and treat stress and its related disorders.

  9. International Team Identifies Biomarker for Scleroderma

    Science.gov (United States)

    ... Spotlight on Research International Team Identifies Biomarker for Scleroderma By Kirstie Saltsman, Ph.D. | May 5, 2014 ... molecule correlates with a more severe form of scleroderma, a chronic autoimmune disorder that involves the abnormal ...

  10. Scleroderma in hospital settings in Lomé: 50 cases.

    Science.gov (United States)

    Akakpo, A S; Teclessou, J N; Mouhari-Touré, A; Saka, B; Matakloe, H; Kakpovi, K; Kombate, K; Pitché, P

    2017-11-01

    The aim of this study was to document the epidemiological and clinical profile, treatment used, and outcome of patients with scleroderma in hospital settings in Lomé. This descriptive study examined the records of all patients seen as outpatients or admitted for scleroderma in hospital dermatology and rheumatology departments in Lomé during the 20-year period of 1993-2012. During the study period, 50 (0.04%) of the 121,021 patients seen in these departments had scleroderma. There were 29 cases of localized scleroderma and 21 systemic cases, predominantly women (sex-ratio=0.2). The patients' mean age was 36 years. All patients with systemic scleroderma had speckled achromia (100%), and most (90.48%) had cutaneous sclerosis. After a mean follow-up period of 43.5 days, 71.43% of the patients had been lost to follow-up. All of the patients with localized scleroderma had cutaneous sclerosis, and the rate of loss to follow-up (after a mean of 17 days) was 96.55%. The results of this study confirm the extreme rarity of scleroderma in the teaching hospitals in Lomé and a clear female predominance. It points out the difficulty of management, which both influences and is aggravated by the high rate of loss to follow-up.

  11. [Microstomia as a complication of scleroderma

    NARCIS (Netherlands)

    Phoa, K.H.; Baat, P. de; Baat, C. de

    2003-01-01

    Scleroderma is a rare connective tissue disease of unknown origin. It is characterized by increased collagen deposition leading to fibrosis and degeneration of the skin and sometimes of internal organs. There are two main groups of scleroderma: circumscribed scleroderma and systemic sclerosis.

  12. [3H]thymidine labeling of dermal endothelial cells in scleroderma

    International Nuclear Information System (INIS)

    Fleischmajer, R.; Perlish, J.S.

    1977-01-01

    The purpose of this study was to estimate [ 3 H] thymidine labeling of endothelial cells of skin capillaries in localized scleroderma (LS) and systemic scleroderma (SS). Skin specimens from 14 patients with SS, 5 with LS, and 9 matched controls were studied by in vitro autoradiography. Capillaries from patients with SS showed a statistically significant increase in endothelial cell labeling when compared to vessels from controls

  13. A case of localized juvenile periodontitis: treatment and 3 years follow-up with superimposable radiographs.

    Science.gov (United States)

    Dubrez, B; Baehni, P; Cimasoni, G

    1996-06-01

    A 17-year-old male patient with localized juvenile periodontitis was treated by subgingival instrumentation with full thickness flap on the lower molars, combined with a 3-week course of systemic tetracycline, and a programme of supervised oral hygiene. The treatment was rapidly followed by dramatic clinical and microbiological improvement. However, despite good oral hygiene, gingival inflammation recurred at regular intervals. It was necessary to maintain the clinical results by periodic subgingival instrumentation with an ultrasonic scaler. Healing of alveolar bone was monitored in the lower 1st molar regions over 3 years by using superimposable radiographs. Quantitative analysis of bone density performed with a high-resolution digitalisation technique showed a considerable improvement 1 year after therapy. However, continuous remodelling, probably related to variations in inflammation, occurred during the 3 postoperative years.

  14. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.

    Science.gov (United States)

    Fett, Nicole

    2013-01-01

    Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The nomenclature of scleroderma has changed dramatically in recent years, with morphea (localized scleroderma), limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma encompassing the currently accepted disease subtypes. Major advances have been made in the molecular studies of morphea and systemic sclerosis; however, their etiologies and pathogenesis remain incompletely understood. Although morphea and systemic sclerosis demonstrate activation of similar inflammatory and fibrotic pathways, important differences in signaling pathways and gene signatures indicate they are likely biologically distinct processes. Morphea can cause significant morbidity but does not affect mortality, whereas systemic sclerosis has the highest disease-specific mortality of all autoimmune connective tissue diseases. Treatment recommendations for morphea and systemic sclerosis are based on limited data and largely expert opinions. Current collaborative efforts in morphea and systemic sclerosis research will hopefully lead to better understanding of the etiology and pathogenesis of these rare and varied diseases and improved treatment options. Published by Elsevier Inc.

  15. Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports

    Directory of Open Access Journals (Sweden)

    Laxer Ronald M

    2011-07-01

    Full Text Available Abstract Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. Using a worldwide pediatric rheumatology electronic list-serve, another patient with the same 2 conditions was identified. These two patients are reported in this study. Consideration of neuroimaging studies to disclose abnormal findings in patients with linear scleroderma en coup de sabre is important for potentially preventing and treating neurological manifestations associated with this condition.

  16. Influence of childhood scleroderma on physical function and quality of life.

    Science.gov (United States)

    Baildam, Eileen M; Ennis, Holly; Foster, Helen E; Shaw, Lindsay; Chieng, Alice S E; Kelly, Jane; Herrick, Ariane L; Richards, Helen L

    2011-01-01

    there have been few studies of quality of life in childhood scleroderma and these focused predominantly on self-perception and the influence of skin lesions. Our cross-sectional study aimed to describe the influence of childhood scleroderma on physical function and quality of life in relation to clinical and demographic measures. children with either localized scleroderma or systemic sclerosis (SSc) attending pediatric rheumatology clinics, together with their parents or guardians, were asked to complete a set of 4 validated measures. Clinical and demographic data were provided by consultant pediatric rheumatologists. in total, 28 children and their parents/guardians participated in the study (68% female, median age 13 yrs; 86% localized scleroderma, 14% SSc). The median Child Health Assessment Questionnaire (CHAQ) score was 0.1 (range 0-3, 0 indicating no impairment), the median Child Dermatology Life Quality Index (CDLQI) score was 5 (range 0-30, 0 indicating no impairment), and the median Child Quality of Life Questionnaire (CQOL) function score was 26 (range 0-105, 0 indicating no impairment). Family activity, measured by the Child Health Questionnaire (CHQ-PF50), was also moderately impaired by scleroderma, with a median score of 83 (0-100, 100 indicating no impairment). scleroderma had only a moderate effect on quality of life and physical function as measured by the 4 validated instruments. Although a small number of children reported greater impairment, this is an encouraging finding, given its potential disfiguring and debilitating effects.

  17. A study of the localized humoral immune response to implicated microorganisms in juvenile periodontitis

    International Nuclear Information System (INIS)

    Hall, E.R.

    1988-01-01

    A study was undertaken using an in vitro explant culture system to determine the presence of immunoglobulins (IgG, IgA, and IgM) in the supernatant fluids (SF) of disease gingival tissue explant cultures. Studies were also undertaken to determine if the de novo biosynthesis of 14 C-immunoglobulins could be observed in the explant cultures of diseased tissues from juvenile periodontitis (JP) patients. Radiolabeled proteins were detected in the SF and immunodiffusion studies using goat antihuman gamma, alpha or mu chain serum revealed the presence of IgG and IgA but no IgM present in the SF of the JP gingival tissue explant cultures. Immunodiffusion studies using goat anti-human gamma chain serum with Staph protein A isolated IgG fractions of the SF, followed by autoradiography of the IgG precipitation lines demonstrated the biosynthesis of IgG by the JP gingival tissue explant cultures. The serological studies suggested that local immune response in JP was to a polymicrobic infection. The SF of JP showed significantly higher levels of antibody reactivity to B. intermedius, C. ochracea, E. nodatum and P. micros as compared to healthy tissues. The local antibody response to the microorganisms tested differed from that observed in the sera of the patients

  18. Ocular localization of mycobacterial lesions in tank-reared juvenile cobia, Rachycentron canadum.

    Science.gov (United States)

    Phillips, A C N; Suepaul, R; Soto, E

    2017-12-01

    Severe clinical mycobacteriosis with consistent ocular lesion localization was diagnosed in a population of 800 juvenile tank-reared Cobia (Rachycentron canadum) which experienced a sudden increase in mortality approximately 5 months after arriving into Trinidad and Tobago from Florida, USA. Moderate daily mortality (15-20 animals per day) persisted for just over 1 month. Moribund fish displayed circling behaviour and had an open-mouth gape upon death. Fish consistently presented with bilateral exophthalmia, corneal cloudiness and hyphema. Non-branching acid-fast rods were detected in aqueous humour touch preparations. Histological analysis revealed severe bilateral intra-ocular granulomatous responses in all specimens. Mycobacterium sp. was identified using a real-time PCR assay detecting the RNA polymerase β-subunit (rpoB) gene in different tissue samples. Specimens did not present with characteristic granulomatous responses usually seen in viscera. To the best of our knowledge, this represents only the third documentation of piscine mycobacterial infection presenting with only localized ocular lesions, and the second documented case of mycobacteriosis in cobia. It is, however, the first documentation of an ocular presentation of mycobacteriosis in a marine species and is the first documentation of such a presentation in cobia. © 2017 John Wiley & Sons Ltd.

  19. A study of the localized humoral immune response to implicated microorganisms in juvenile periodontitis

    Energy Technology Data Exchange (ETDEWEB)

    Hall, E.R.

    1988-01-01

    A study was undertaken using an in vitro explant culture system to determine the presence of immunoglobulins (IgG, IgA, and IgM) in the supernatant fluids (SF) of disease gingival tissue explant cultures. Studies were also undertaken to determine if the de novo biosynthesis of {sup 14}C-immunoglobulins could be observed in the explant cultures of diseased tissues from juvenile periodontitis (JP) patients. Radiolabeled proteins were detected in the SF and immunodiffusion studies using goat antihuman gamma, alpha or mu chain serum revealed the presence of IgG and IgA but no IgM present in the SF of the JP gingival tissue explant cultures. Immunodiffusion studies using goat anti-human gamma chain serum with Staph protein A isolated IgG fractions of the SF, followed by autoradiography of the IgG precipitation lines demonstrated the biosynthesis of IgG by the JP gingival tissue explant cultures. The serological studies suggested that local immune response in JP was to a polymicrobic infection. The SF of JP showed significantly higher levels of antibody reactivity to B. intermedius, C. ochracea, E. nodatum and P. micros as compared to healthy tissues. The local antibody response to the microorganisms tested differed from that observed in the sera of the patients.

  20. Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma

    DEFF Research Database (Denmark)

    Krabbe, Simon; Engelhart, Merete; Thybo, Sören

    2017-01-01

    This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal......, unfortunately with significant scarring. Immunodeficiency testing was unremarkable. In summary, an infection with Mycobacterium intracellulare was mistaken for an unusually severe progression of scleroderma....

  1. Microbiological characteristics of subgingival microbiota in adult periodontitis, localized juvenile periodontitis and rapidly progressive periodontitis subjects.

    Science.gov (United States)

    Nonnenmacher, C; Mutters, R; de Jacoby, L F

    2001-04-01

    To describe the prevalence of the cultivable subgingival microbiota in periodontal diseases and to draw attention to the polymicrobial nature of periodontic infections. The study population consisted of 95 patients, 51 females and 44 males, aged 14-62 years. Twenty-nine patients exhibited adult periodontitis (AP), six localized juvenile periodontitis (LJP), and 60 rapidly progressive periodontitis (RPP). Two to four pooled bacterial samples were obtained from each patient. Samples were collected with sterile paper points from the deepest periodontal pockets. The samples were cultured under anaerobic and microaerophilic conditions using selective and non-selective media. Isolates were characterized to species level by conventional biochemical tests and by a commercial rapid test system. Prevotella intermedia and Capnocytophaga spp. were the most frequently detected microorganisms in all diagnostic groups. Porphyromonas gingivalis and Peptostreptococcus micros were found more frequently in AP and RPP patients, while Actinobacillus actinomycetemcomitans and Eikenella corrodens were associated with AP, LJP and RPP patients. The other bacterial species, including Actinomyces spp., Streptococcus spp. and Eubacterium spp., were detected at different levels in the three disease groups. The data show the complexity of the subgingival microbiota associated with different periodontal disease groups, indicating that the detection frequency and levels of recovery of some periodontal pathogens are different in teeth affected by different forms of periodontal disease.

  2. Scleroderma mimicker – Eosinophilic fasciitis

    Directory of Open Access Journals (Sweden)

    Debanjali Sinha

    2017-01-01

    Full Text Available Eosinophilic fasciitis is an uncommon connective tissue disorder characterized by thickening of the deep fascia and overlying skin and subcutaneous tissue. It may mimic scleroderma and other scleroderma-like conditions. It may be a manifestation of paraneoplastic disorders or may be associated with hematological disorders including lymphomas. Definitive diagnosis is made on histological examination of a deep skin biopsy revealing thickened deep fascia and infiltration by lymphocytes and eosinophils. Enhancement of deep fascia on Gadolinium contrast-enhanced magnetic resonance imaging may be used as a substitute for skin biopsy. Ultrasound imaging is an evolving imaging tool for diagnosing it. Glucocorticoids with or without immunosuppressive agents remains the mainstay of therapy with good response, generally. A younger age of onset, morphea like lesions and dermal fibrosclerosis is more likely to be associated with the refractory disease. Early diagnosis and appropriate treatment may result in better outcomes in terms of morbidity and quality of life of the patients.

  3. Correlação clínica e ultra-sonográfica na esclerodermia localizada cutânea Clinical and ultrasonographic correlation in localized cutaneous scleroderma

    Directory of Open Access Journals (Sweden)

    Marcio Bouer

    2008-04-01

    Full Text Available OBJETIVO: Apresentar os aspectos ultra-sonográficos da esclerodermia localizada e relacioná-los com os aspectos clínicos. MATERIAIS E MÉTODOS: Foram analisadas 23 lesões de esclerodermia localizada em 21 pacientes. Foi utilizado equipamento Logiq 700 com transdutor linear de 6-14 MHz. Foram avaliados, pelo dermatologista, o estágio da doença (inflamatório ou atrófico, e pelo radiologista, a espessura e a ecogenicidade da derme nas regiões afetadas e sãs adjacentes. Foi feito acompanhamento de sete casos após tratamento. RESULTADOS: Todas as lesões apresentaram perda do padrão ultra-sonográfico normal da derme. Os casos de lesão clinicamente atrófica (52,2%; 12/23 corresponderam a redução da espessura e aumento da ecogenicidade da derme e os casos de lesão clinicamente inflamatória (47,8%; 11/23 corresponderam a aumento da espessura e redução da ecogenicidade da derme. Controles pós-tratamento mostraram alterações na espessura da derme. CONCLUSÃO: Os achados ultra-sonográficos nos permitem associar o aumento da espessura e a redução da ecogenicidade da derme com a fase inflamatória da doença, e a redução da espessura e o aumento da ecogenicidade da derme com a fase atrófica da doença. Notamos também que é possível quantificar a espessura da derme e usar essa informação no controle pós-tratamento associada à avaliação clínica.OBJECTIVE: To describe ultrasonographic findings of localized cutaneous scleroderma and correlating them with clinical findings. MATERIALS AND METHODS: Twenty-three lesions of localized cutaneous scleroderma in 21 patients were evaluated with a Logiq 700 equipment coupled with a 6-14 MHz linear transducer. The disease stage (athrophic or inflammatory was evaluated by a dermatologist, and the ultrasonographic findings (skin thickness and echogenicity for both the affected and adjacent healthy regions were evaluated by a radiologist. Seven of the cases underwent post

  4. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study.

    Science.gov (United States)

    Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta; Smith, Vanessa; Cutolo, Maurizio; Foeldvari, Ivan

    2015-11-01

    Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% CI 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% CI 0.28-2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34-3.56. This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. Neurologic Involvement in Scleroderma en Coup de Sabre

    Science.gov (United States)

    Amaral, Tiago Nardi; Marques Neto, João Francisco; Lapa, Aline Tamires; Peres, Fernando Augusto; Guirau, Caio Rodrigues; Appenzeller, Simone

    2012-01-01

    Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs. PMID:22319646

  6. Targeting miR-155 to Treat Experimental Scleroderma.

    Science.gov (United States)

    Yan, Qingran; Chen, Jie; Li, Wei; Bao, Chunde; Fu, Qiong

    2016-02-01

    Scleroderma is a refractory autoimmune skin fibrotic disorder. Alterations of microRNAs in lesional skin could be a new approach to treating the disease. Here, we found that expression of miR-155 was up regulated in lesional skin tissue from patients with either systemic or localized scleroderma, and correlated with fibrosis area. Then we demonstrated the potential of miR-155 as a therapeutic target in pre-clinical scleroderma models. MiR-155(-/-) mice were resistant to bleomycin induced skin fibrosis. Moreover, topical antagomiR-155 could effectively treat mice primed with subcutaneous bleomycin. In primary skin fibroblast, miR-155 silencing could inhibit collagen synthesis function, as well as signaling intensity of two pro-fibrotic pathways, Wnt/β-catenin and Akt, simultaneously. We further showed that miR-155 could regulate the two pathways via directly targeting casein kinase 1α (CK1α) and Src homology 2-containing inositol phosphatase-1 (SHIP-1), as previous reports. Mice with miR-155 knockout or topical antagomir-155 treatment showed inhibited Wnt/β-catenin and Akt signaling in skin upon bleomycin challenge. Together, our data suggest the potential of miR-155 silencing as a promising treatment for dermal fibrosis, especially in topical applications.

  7. Corticosteroids in Myositis and Scleroderma.

    Science.gov (United States)

    Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

    2016-02-01

    Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. Published by Elsevier Inc.

  8. Biomarkers in scleroderma: Current status

    Directory of Open Access Journals (Sweden)

    Latika Gupta

    2017-01-01

    Full Text Available Scleroderma is an autoimmune disease characterized by indolent obliterative vasculopathy and widespread fibrosis. The two main morphological manifestations of the disease overlap and may make it difficult to separate activity from damage. Many patients, especially those with the limited subset of the disease, have an indolent course without clear-cut inflammatory manifestations. There is a felt need for validated biomarkers, which can differentiate activity from damage, and yet be sensitive to change with therapy. Multiplex arrays of biomarkers have ushered an era of targeted or personalized medicine based on phenotypic characteristics in an individual.

  9. Subcutaneous administration of polymerized type I collagen downregulates interleukin (IL)-17A, IL-22 and transforming growth factor-β1 expression, and increases Foxp3-expressing cells in localized scleroderma.

    Science.gov (United States)

    Furuzawa-Carballeda, J; Ortíz-Ávalos, M; Lima, G; Jurado-Santa Cruz, F; Llorente, L

    2012-08-01

    Localized scleroderma (LS) is a disfiguring inflammatory autoimmune disease of the skin and underlying tissue. As in systemic sclerosis, a key feature is the presence of T cells in inflammatory lesions. To evaluate the effect of polymerized type I collagen vs. methylprednisolone (MP) in LS, and to determine the influence of this polymerized collagen (PC) on CD4+ peripheral T cells expressing interleukin (IL)-4, IL-17A, interferon-γ and Forkhead box protein (Foxp)3, and on cells expressing transforming growth factor (TGF)-β1, IL-17A, IL-22 and Foxp3 in the skin. In total, 16 patients with LS were treated for 3 months with monthly subcutaneous intralesional injections of 0.1 mL MP (giving a total dose of 20 mg/mL each month) and 15 patients were treated, with weekly subcutaneous intralesional injections of PC, ranging from 0.2 mL (equivalent to 1.66 mg collagen) for a lesion of 50 mm in size, up to a maximum of 1.0 mL (8.3 mg collagen) for a lesion > 100 mm in size, and followed up for a further 6 months. Skin biopsies were obtained from lesions at baseline (before treatment) and 9 months later (6 months after treatment end). Tissue sections were evaluated by histology and immunohistochemistry (IL-17A, IL-22, TGF-β1 and Foxp3). CD4+ T-cell subsets were determined in peripheral blood by flow cytometry. Abnormal tissue architecture was seen in the biopsies taken from patients treated with MP, whereas the PC treatment restored normal skin architecture. PC downregulated pro-inflammatory/profibrotic cytokine expression in peripheral cells, and upregulated the number of regulatory T cells (Tregs) in skin. PC was safe and well tolerated. PC is not only an antifibrotic/fibrolytic agent but also an immunomodulator biodrug that restores the balance between T helper (Th)1, Th2, Th17 and Tregs, downregulates production of pro-inflammatory or profibrogenic cytokines (IL-17A, IL-22 and TGF-β1), and renews skin architecture, without adverse effects. © The Author(s). CED

  10. Esophagectomy in Scleroderma: Report of a Case

    Directory of Open Access Journals (Sweden)

    Erdal Yekeler

    2008-12-01

    Full Text Available Scleroderma is a generalized autoimmune disease with variable involvement of the skin and major organs (esophagus, lung, heart and kidney. Scleroderma is essentially a skin disease that frequently involves the digestive system. In scleroderma, the esophagus is the most frequently affected organ of the digestive system, and esophageal dysmotility, reflux and stricture may be observed in the advanced stage. Balloon dilatation and bougienage are generally sufficient in patients developing stricture, and the number of cases in whom resection is performed is very low. In a 20-year-old patient with difficulty in taking even liquid foods, tests revealed sclerodermal involvement of the distal end of the esophagus and stricture. Esophageal resection and gastric replacement were performed. Such systemic diseases as scleroderma, although rare, must be considered in the differential diagnosis of nonmalignant dysphagia, and resection must be borne in mind as a surgical option in cases of advanced stricture.

  11. UVA1 a promising approach for scleroderma

    Science.gov (United States)

    Keyal, Uma; Bhatta, Anil Kumar; Wang, Xiu Li

    2017-01-01

    Scleroderma is a complex connective tissue disease characterized by fibrosis, vasculopathy, and immune system dysfunction. The heterogeneity of disease presentation and poorly understood etiology has made the management of scleroderma difficult. The available treatment options like immunosuppressive agents are associated with potentially hazardous side effects and physiotherapy, which to a certain degree helps to minimize the loss of function in digits and limbs, has only limited success. Also, studies investigating antifibrotic therapies have failed to report any significant improvement. Hence, there is currently no effective therapy for scleroderma. Recently, phototherapy has been extensively studied and found to be effective in treating scleroderma. Initially psoralen + ultraviolet A (PUVA) significantly enriched the therapeutic panel, but more recently ultraviolet A1 (UVA1) is seen to replace PUVA therapy. This might be because of UVA1 therapy being free of side effects seen with psoralens such as nausea, vomiting or photokeratitis. In addition, UVA1 is seen to lower risk of phototoxic reactions with deeper penetration of radiation. The present review will put some light on the use of UVA1 for treating cutaneous lesion in scleroderma and we aim to find the most benefitted group of patients and most effective dose of UVA1 for different types of scleroderma. PMID:28979701

  12. Keloidal Scleroderma: Case Report and Review

    Directory of Open Access Journals (Sweden)

    Sama Kassira

    2015-01-01

    Full Text Available Objective. We report a rare case of keloidal scleroderma and provide an analysis of similar cases. Results. A 41 year-old woman presented with dark brown, indurated, exophytic nodules over the chest along with smaller hyperpigmented plaques scattered over the abdomen, with concomitant sclerodactyly. The clinical, laboratory, and pathological findings were consistent with a diagnosis of keloidal scleroderma. The patient was treated with methotrexate, resulting in reduced firmness of her plaques and no new lesions. A literature review of previously reported cases was performed using keywords including keloidal morphea, keloidal scleroderma, nodular morphea, and nodular scleroderma. In our review, the majority of patients were African American and female. 91% of cases had nodular lesions with distribution on the trunk. The majority of patients exhibited sclerodactyly and pulmonary involvement was reported in 28%1. The majority of patients were ANA positive (63% and only 10% demonstrated anti-SCL-70 positivity. Conclusion. Keloidal scleroderma is a rare presentation, which can often be clinically confused with keloid and scar formation. Due to this being a rare variant, our knowledge of treatment options and efficacy is limited. Methotrexate could be considered as an initial treatment option for patients with progressive keloidal scleroderma.

  13. Scleroderma and dentistry: Two case reports.

    Science.gov (United States)

    Dixit, Shantanu; Kalkur, Chaithra; Sattur, Atul P; Bornstein, Michael M; Melton, Fred

    2016-10-24

    Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable. Hence, we present a case of morphea, associated with Parry-Romberg syndrome, and a second case with the classical signs of progressive systemic sclerosis. Case one: A 20-year-old man of Dravidian origin presented to our out-patient department with a complaint of facial asymmetry, difficulty in speech, and loss of taste sensation over the last 2 years. There was no history of facial trauma. After physical and radiological investigations, we found gross asymmetry of the left side of his face, a scar on his chin, tongue atrophy, relative microdontia, thinning of the ramus/body of his mandible, and sclerotic lesions on his trunk. Serological investigations were positive for antinuclear antibody for double-stranded deoxyribonucleic acid and mitochondria. A biopsy was suggestive of morphea. Hence, our final diagnosis was mixed morphea with Parry-Romberg syndrome. Case two: A 53-year-old woman of Dravidian origin presented to our out-patient department with a complaint of gradually decreasing mouth opening over the past 7 years. Her medical history was noncontributory. On clinical examination, we found her perioral, neck, and hand skin to be sclerotic. Also, her fingers exhibited bilateral telangiectasia. An oral examination revealed completely edentulous arches as well as xerostomia and candidiasis. Her serological reports were positive for antinuclear antibodies against centromere B, Scl-70, and Ro-52. A hand and

  14. Spatial distribution of limited resources and local density regulation in juvenile Atlantic salmon.

    Science.gov (United States)

    Finstad, Anders G; Einum, Sigurd; Ugedal, Ola; Forseth, Torbjørn

    2009-01-01

    1. Spatial heterogeneity of resources may influence competition among individuals and thus have a fundamental role in shaping population dynamics and carrying capacity. In the present study, we identify shelter opportunities as a limiting resource for juvenile Atlantic salmon (Salmo salar L.). Experimental and field studies are combined in order to demonstrate how the spatial distribution of shelters may influence population dynamics on both within and among population scales. 2. In closed experimental streams, fish performance scaled negatively with decreasing shelter availability and increasing densities. In contrast, the fish in open stream channels dispersed according to shelter availability and performance of fish remaining in the streams did not depend on initial density or shelters. 3. The field study confirmed that spatial variation in densities of 1-year-old juveniles was governed both by initial recruit density and shelter availability. Strength of density-dependent population regulation, measured as carrying capacity, increased with decreasing number of shelters. 4. Nine rivers were surveyed for spatial variation in shelter availability and increased shelter heterogeneity tended to decrease maximum observed population size (measured using catch statistics of adult salmon as a proxy). 5. Our studies highlight the importance of small-scale within-population spatial structure in population dynamics and demonstrate that not only the absolute amount of limiting resources but also their spatial arrangement can be an important factor influencing population carrying capacity.

  15. Validation of the Body Concealment Scale for Scleroderma (BCSS): Replication in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort

    NARCIS (Netherlands)

    Jewett, L.R.; Kwakkenbos, C.M.C.; Carrier, M.E.; Malcarne, V.L.; Harcourt, D.; Rumsey, N.; Mayes, M.D.; Assassi, S.; Körner, A.; Fox, R.S.; Gholizadeh, S.; Mills, S.D.; Fortune, C.; Thombs, B.D.

    2017-01-01

    Body concealment is an important component of appearance distress for individuals with disfiguring conditions, including scleroderma. The objective was to replicate the validation study of the Body Concealment Scale for Scleroderma (BCSS) among 897 scleroderma patients. The factor structure of the

  16. Scleroderma

    Science.gov (United States)

    ... this research; and the dissemination of information on research progress in these diseases. Contact Us NIAMS Archive Viewers and Players Social Media Moderation Policy FOIA Privacy Statement Accessibility Disclaimer Digital Strategy ...

  17. Current and future direction in the management of scleroderma.

    Science.gov (United States)

    Brady, Sean M; Shapiro, Lee; Mousa, Shaker A

    2016-09-01

    Scleroderma is a heterogeneous disease with a complex etiology. As more information is gained about the underlying mechanisms and the improved classifications of scleroderma subtypes, treatments can be better personalized. Improving scleroderma patients' early diagnosis before end organ manifestations occur should improve clinical trial design and outcomes. Two recently FDA-approved antifibrotics for idiopathic pulmonary fibrosis may be effective treatments in patients with pulmonary fibrosis secondary to scleroderma after further investigation. The potential impact of Nanobiotechnology in improving the efficacy and safety of existing antifibrotics and immunomodulators might present an exciting new approach in the management of scleroderma.

  18. Abnormalities in the Regulators of Angiogenesis in Patients with Scleroderma

    Science.gov (United States)

    HUMMERS, LAURA K.; HALL, AMY; WIGLEY, FREDRICK M.; SIMONS, MICHAEL

    2014-01-01

    Objective To determine plasma levels of regulators of angiogenesis in patients with scleroderma and to correlate those levels with manifestations of scleroderma-related vascular disease. Methods Plasma levels of vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), fibroblast growth factor-2 (FGF-2), matrix metalloproteinase-9 (MMP-9), endostatin, pro-MMP-1, hepatocyte growth factor (HGF), placental growth factor (PlGF), and FGF-4 were examined by ELISA in a cross-sectional study of 113 patients with scleroderma and 27 healthy controls. Simple and multivariate regression models were used to look for associations between factor levels and clinical disease characteristics. Results There were marked differences in the levels of pro-angiogenic growth factors between patients with scleroderma and controls, with significant elevations of VEGF, PDGF, FGF-2, and PlGF among patients with scleroderma (p scleroderma patients compared to controls (MMP-9 and pro-MMP-1) (p scleroderma, but had a positive correlation with right ventricular systolic pressure (RVSP) as measured by echocardiogram (p scleroderma (p scleroderma. The levels of some factors correlate with measures of vascular disease among patients with scleroderma. Dysregulated angiogenesis may play a role in the development of scleroderma vascular disease. PMID:19228661

  19. Caring for the Patient With Limited Systemic Scleroderma.

    Science.gov (United States)

    Lachner, Kelly Denise

    2016-01-01

    Systemic scleroderma (systemic sclerosis) is a rare, autoimmune, collagen-vascular disease of unknown etiology that affects the connective tissues of the skin, internal organs, as well as the small blood vessels. There are 3 subclasses of systemic scleroderma: limited cutaneous, diffuse cutaneous, and sine scleroderma. Prognosis depends on the extent of organ involvement. Complications of systemic scleroderma can involve the cardiovascular, pulmonary, gastrointestinal, renal, integumentary, and the skeletal-muscular systems. Because systemic scleroderma is not common, many orthopaedic nurses may be unfamiliar with how to best provide care. This article provides information about the complexity of the different types of this disease and the basic nursing care of the patient with the most common subclass of systemic scleroderma, limited cutaneous systemic scleroderma.

  20. An unusual dental phenomenon in systemic scleroderma

    International Nuclear Information System (INIS)

    ElCridly, Mahmoud

    1989-01-01

    Systemic scleroderma is a disease of unknown etiology characterized by excessive deposition of collagen and other connective tissue components in the skin and several internal organs. The most common oral findings are rigidity and thinness of lips, circumoral fibrosis which causes microstomia and inability to open the mouth widely. Involvement of tongue may lead to its decreased mobility and diminished size. Radiographic findings that have been classically associated with systemic scleroderma are widening of the periodontal ligament spaces and destruction of lamina dura, usually in posterior teeth. Intraoral radiographs of presented cases revealed apical resorption of the roots and destruction of the lamina dura verified on extraction of the teeth. This oral finding appears not to have been recorded by previous investigators of scleroderma. (author)

  1. Linear scleroderma following Blaschko′s lines

    Directory of Open Access Journals (Sweden)

    Mukhopadhyay Amiya

    2005-01-01

    Full Text Available Blaschko′s lines form a pattern, which many diseases are found to follow, but linear scleroderma following Blaschko′s lines is a controversial entity rarely reported in the literature. A 24-year-old man presented with multiple linear, atrophic, hyperpigmented lesions punctuated by areas of depigmentations on the left half of the trunk distributed on the anterior, lateral and posterior aspects. The lesions were distributed in a typical S-shaped line. Antinuclear antibody and antihistone antibody tests were negative. Histopathological examination of the skin from the affected area showed features suggestive of scleroderma. Here, we present a case of linear scleroderma following Blaschko′s lines in a male patient - an entity reported only three times so far.

  2. Nodular Scleroderma - Successful Treatment With Extracorporeal Photochemotherapy

    Directory of Open Access Journals (Sweden)

    Wollina U

    2001-01-01

    Full Text Available Nodular seleroderma is rare variant of circumscribed scleroderma (morphea. Treatment is often unsatisfactory. This report is on the use of extracorporeal photochemotherapy. A 12 year old girl and a 49 year old woman have been treated once a month on two consecutive days. A complete remission was achieved in one patient after 10 months and an almost complete remission in the young girl after 6 months. The treatment was well-tolerated and no severe side â€" effects occurred. In contrast to previous attempts in treating nodular scleroderma with different modalities, ECP seems to be an effective therapy.

  3. Acute and regressive scleroderma concomitant to an acute CMV primary infection.

    Science.gov (United States)

    Goulabchand, Radjiv; Khellaf, Lakhdar; Forestier, Amandine; Costes, Valerie; Foulongne, Vincent; le Quellec, Alain; Guilpain, Philippe

    2014-12-01

    To describe the pathophysiological mechanisms involving cytomegalovirus (CMV) primary infection and natural killer (NK) cell expansion in the development of localized scleroderma. A 43-year-old woman presented acute erythematous discoloration and skin thickening concerning face, neck, trunk, abdomen, and the four limbs, predominantly in proximal areas. Our case did not respond to systemic sclerosis criteria diagnosis. However, skin and muscle biopsy revealed early scleroderma associated with capillary thrombi, and tissue infiltration with NK cells (CD56+/Granzyme B). Scleroderma was attributed to CMV primary infection responsible for cytolytic hepatitis (7-fold over the limit) and circulating NK cell excess. After 6 months of prednisone and a 2-year follow-up, a complete resolution of symptoms was observed. Our observation suggests a potential triggering role of CMV primary infection in the development of scleroderma. Histological features from our observation addresses the role of CMV and NK cells in the development of endothelial damage and fibrotic process. Copyright © 2014 Elsevier B.V. All rights reserved.

  4. Association of Immunological Cell Profiles with Specific Clinical Phenotypes of Scleroderma Disease

    Science.gov (United States)

    Calzada, David; Mayayo, Teodoro; González-Rodríguez, María Luisa; Rabasco, Antonio María; Lahoz, Carlos

    2014-01-01

    This study aimed to search the correlation among immunological profiles and clinical phenotypes of scleroderma in well-characterized groups of scleroderma patients, comparing forty-nine scleroderma patients stratified according to specific clinical phenotypes with forty-nine healthy controls. Five immunological cell subpopulations (B, CD4+ and CD8+ T-cells, NK, and monocytes) and their respective stages of apoptosis and activation were analyzed by flow cytometry, in samples of peripheral blood mononuclear cells (PBMCs). Analyses of results were stratified according to disease stage, time since the diagnosis, and visceral damage (pulmonary fibrosis, pulmonary hypertension, and cardiac affliction) and by time of treatment with corticosteroids. An increase in the percentages of monocytes and a decrease in the B cells were mainly related to the disease progression. A general apoptosis decrease was found in all phenotypes studied, except in localized scleroderma. An increase of B and NK cells activation was found in patients diagnosed more than 10 years ago. Specific cell populations like monocytes, NK, and B cells were associated with the type of affected organ. This study shows how, in a heterogeneous disease, proper patient's stratification according to clinical phenotypes allows finding specific cellular profiles. Our data may lead to improvements in the knowledge of prognosis factors and to aid in the analysis of future specific therapies. PMID:24818126

  5. Esophageal dysmotility in scleroderma: a prospective study of 183 cases.

    Science.gov (United States)

    Lahcene, M; Oumnia, N; Matougui, N; Boudjella, M; Tebaibia, A; Touchene, B

    2009-01-01

    The goal of the study was to evaluate the prevalence and risk factors of esophageal motor disorders in systemic sclerosis. In 183 consecutive cases of scleroderma, as diagnosed by American College of Rheumatology criteria (1980). Patients' mean age was 40.6+/-13.3 years, the gender ratio was 0.13 and the average duration of disease was 6.8+/-7.5 years. A localized, cutaneous form was observed in 148 patients (81%) and a diffuse form in 35 (19%). All patients underwent upper gastrointestinal endoscopy and standard esophageal manometry. Esophageal symptoms and reflux esophagitis were found in 108 (59%) and 68 (37%) of patients, respectively. Esophageal motor disorders were present in 148 patients (81%), and were associated with a hypotensive lower esophageal sphincter in 114 (62%). The presence of these motor abnormalities was not related to age, gender, skin extension or duration of disease. Esophageal motor disorders were present in almost all patients with esophageal symptoms or esophagitis, and were also found in 48 (64%) of the asymptomatic patients. Esophageal motor disorders are frequently seen in scleroderma, especially in cases with clinical symptoms, but are not associated with a specific form of the disease.

  6. Juvenile angiofibroma

    Science.gov (United States)

    Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor; Juvenile nasal angiofibroma; JNA ... Juvenile angiofibroma is not very common. It is most often found in adolescent boys. The tumor contains many blood ...

  7. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis

    DEFF Research Database (Denmark)

    Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta

    2015-01-01

    OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus on capillar......OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus...... on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. RESULTS: 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy...... in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34-3.56. CONCLUSION: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern...

  8. Aberrant Recapitulation of Developmental Program: Novel Target in Scleroderma

    Science.gov (United States)

    2015-12-01

    AWARD NUMBER: W81XWH-12-1-0472 TITLE: “Aberrant Recapitulation of Developmental Program: Novel Target in Scleroderma ” PRINCIPAL INVESTIGATOR...SUBTITLE Aberrant Recapitulation of Developmental Program: Novel Target in Scleroderma 5a. CONTRACT NUMBER W81XWH-12-1-0472 5b. GRANT NUMBER 5c. PROGRAM...SPONSOR/MONITOR’S REPORT NUMBER(S) 12. DISTRIBUTION / AVAILABILITY STATEMENT 13. SUPPLEMENTARY NOTES 14. ABSTRACT Fibrosis in scleroderma is associated

  9. Rare Infratentorial and Supratentorial Localization of Juvenile Angiofibroma: A Case Report.

    Science.gov (United States)

    Pašalić, Ivan; Trninić, Ines; Nemir, Jakob; Jednačak, Hrvoje; Žarković, Kamelija; Mrak, Goran

    2016-01-01

    Angiofibromas are rare tumors of the head and neck that mostly occur in the sphenopalatine region. We present a case of angiofibroma in a young male patient with an unusual and extremely rare localization, which to our knowledge has not been described before. It was situated in the tentorium and spread to the supratentorial and infratentorial regions. The patient initially presented with symptoms of increased intracranial pressure. After a diagnostic evaluation was done, the whole tumor was successfully removed using the supratentorial and infratentorial approach and the microsurgical technique. © 2016 S. Karger AG, Basel.

  10. Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma

    Directory of Open Access Journals (Sweden)

    Palasciano Giuseppe

    2008-02-01

    Full Text Available Abstract Background Studies on gastrointestinal symptoms, dysfunctions, and neurological disorders in systemic scleroderma are lacking so far. Methods Thirty-eight scleroderma patients (34 limited, 4 diffuse, 60 healthy controls and 68 dyspeptic controls were scored for upper and lower gastrointestinal symptoms (dyspepsia, bowel habits, gastric and gallbladder emptying to liquid meal (functional ultrasonography and small bowel transit (H2-breath test. Autonomic nerve function was assessed by cardiovascular tests. Results The score for dyspepsia (mainly gastric fullness was greater in scleroderma patients than healthy controls, but lower than dyspeptic controls who had multiple symptoms, instead. Scleroderma patients with dyspepsia had a longer disease duration. Fasting antral area and postprandial antral dilatation were smaller in scleroderma patients than dyspeptic and healthy controls. Gastric emptying was delayed in both scleroderma patients (particularly in those with abnormal dyspeptic score and dyspeptic controls, who also showed a larger residual area. Despite gallbladder fasting and postprandial volumes were comparable across the three groups, gallbladder refilling appeared delayed in dyspeptic controls and mainly dependent on delayed gastric emptying in scleroderma. Small intestinal transit was also delayed in 74% of scleroderma and 66% of dyspeptic controls. Bowel habits were similar among the three groups. Autonomic neuropathy was not associated with dyspepsia, gastric and gallbladder motility and small intestinal transit. Conclusion In scleroderma patients dyspepsia (mainly gastric fullness, restricted distension of the gastric antrum and diffuse gastrointestinal dysmotility are frequent features. These defects are independent from the occurrence of autonomic neuropathy.

  11. Linear scleroderma en coup de sabre including abnormal dental development

    DEFF Research Database (Denmark)

    Hørberg, M; Lauesen, S R; Daugaard-Jensen, J

    2015-01-01

    BACKGROUND: Linear scleroderma en coup de sabre (SCS) is a rare skin condition, where dense collagen is deposited in a localised groove of the head and neck area resembling the stroke of a sabre. The SCS may involve the oral cavity, but the severity and relation to this skin abnormality is unknow...... with a left-sided skin defect (SCS) and a left-sided local malformation in her dentition. It is possible that there is a developmental connection between these two left-sided defects, both with an ectodermal origin.......-UP: The patient has been regularly controlled and treated since she was first diagnosed. A surgical and orthodontic treatment was performed to ensure optimal occlusion, space and alveolar bone development. The present age of the patient is 14 years and 10 months. CONCLUSION: This case demonstrated a patient...

  12. Polymyositis and the Spectrum of Scleroderma Disorders

    Directory of Open Access Journals (Sweden)

    Joana Cochicho

    2015-12-01

    Full Text Available Polymyositis (PM is usually associated to other autoimmune or connective tissue diseases. The authors report the case of a 59-year-old man with pulmonary fibrosis, who presented with constitutional symptoms and gradually developed proximal muscle weakness, Raynaud phenomenon, and dysphagia. Besides creatine kinase (CK elevation, he had positive anti-Polymyositis-Scleromyositis (PM-Scl and anti-Sjögren's-syndrome A (SSA antibodies. Nailfold capillaroscopy showed a scleroderma pattern and muscle biopsy revealed necrosis, regeneration of muscle fibers, and inflammatory infiltrate. Prednisolone was started, with great improvement. Taking into account the overlap features between PM and systemic sclerosis sine scleroderma, it is important to closely monitor the patient for signs of pulmonary and cardiac decompensation.

  13. Tumor-like calcifications with scleroderma

    International Nuclear Information System (INIS)

    Meyer, E.; Kulenkampff, H.A.; Kortenhaus, H.

    1987-01-01

    In patients with progressive scleroderma, interstitial calcifications are present to a varying extent. They are mostly located in the soft tissues of the fingers, resembling points, commas or dashes. They may also appear as 'calcinosis universalis' and reach a considerable size. Thus they mimic proliferative tumors. Scintigraphy, proving the existence of further calcifications can be helpful. We report the case of a female patient who presented with such a 'pseudotumor' of unusual size, site and extent in the lumbar region. (orig.) [de

  14. Progressive systemic scleroderma (A case report)

    International Nuclear Information System (INIS)

    Han, Jung Suh; Kwon, Chung Sik

    1974-01-01

    This is a report of a rare case of progressive systemic scleroderma involving the skin, lungs, bones, esophagus, duodenum, and colon in a 27 year old Korean female whose chief complaints were generalized edema, knee joint pain, hard and indurated skin lesions on the anterior chest, neck, both upper and lower extremities with loss of pain and touch sensation for one year. A review of literature is submitted

  15. Progressive systemic scleroderma (A case report)

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jung Suh; Kwon, Chung Sik [Chonnam University College of Medicine, Kwangju (Korea, Republic of)

    1974-10-15

    This is a report of a rare case of progressive systemic scleroderma involving the skin, lungs, bones, esophagus, duodenum, and colon in a 27 year old Korean female whose chief complaints were generalized edema, knee joint pain, hard and indurated skin lesions on the anterior chest, neck, both upper and lower extremities with loss of pain and touch sensation for one year. A review of literature is submitted.

  16. Gastroesophageal reflux demonstrated by hepatobiliary imaging in scleroderma

    International Nuclear Information System (INIS)

    Sawaf, N.W.; Orzel, J.A.; Weiland, F.L.

    1987-01-01

    Radionuclide hepatobiliary imaging was performed on a patient with a longstanding history of scleroderma who presented with abdominal pain suggestive of biliary disease. Cystic duct patency was documented after 10 min with tracer accumulation in the second portion of the duodenum which failed to progress consistent with the duodenal hypomotility of scleroderma. The patient was given intravenous Kinevac resulting in gastroesophageal reflux of radionuclide

  17. Gastroesophageal reflux demonstrated by hepatobiliary imaging in scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Sawaf, N.W.; Orzel, J.A.; Weiland, F.L.

    1987-03-01

    Radionuclide hepatobiliary imaging was performed on a patient with a longstanding history of scleroderma who presented with abdominal pain suggestive of biliary disease. Cystic duct patency was documented after 10 min with tracer accumulation in the second portion of the duodenum which failed to progress consistent with the duodenal hypomotility of scleroderma. The patient was given intravenous Kinevac resulting in gastroesophageal reflux of radionuclide.

  18. Phenomenon of isomorphic provoking responses in cases of limited scleroderma

    Directory of Open Access Journals (Sweden)

    Talnikova Е.Е.

    2015-09-01

    Full Text Available The article presents the historical origin of the term "Koebner phenomenon". The literature data reflect the etiology, pathogenesis and epidemiology of isomorphic mechanisms provoking responses in lichen planus, psoriasis, scleroder-ma, syphilis. Variants of the Koebner phenomenon's classifications are given. The clinical cases of limited scleroderma after mechanical injury are described.

  19. Atypical Neuroimaging Manifestations of Linear Scleroderma “en coup de sabre”

    Science.gov (United States)

    M. ALLMENDINGER, Andrew; A. RICCI, Joseph; S. DESAI, Naman; VISWANADHAN, Narayan; RODRIGUEZ, Diana

    2015-01-01

    Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. Patients often present with neurologic symptoms. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Infratentorial lesions and other lesions not closely associated with the overlying scalp abnormality, such as those found in the cerebellum, have been reported, but are extremely uncommon. We present a case of an 8-year-old boy with a left fronto-parietal “en coup de sabre” scalp lesion and describe the neuroimaging findings of a progressively enlarging left cerebellar lesion discovered incidentally on routine magnetic resonance imaging. Interestingly, the patient had no neurologic symptoms given the size of the mass identified. PMID:26401155

  20. Capillary regeneration in scleroderma: stem cell therapy reverses phenotype?

    Directory of Open Access Journals (Sweden)

    Jo N Fleming

    2008-01-01

    Full Text Available Scleroderma is an autoimmune disease with a characteristic vascular pathology. The vasculopathy associated with scleroderma is one of the major contributors to the clinical manifestations of the disease.We used immunohistochemical and mRNA in situ hybridization techniques to characterize this vasculopathy and showed with morphometry that scleroderma has true capillary rarefaction. We compared skin biopsies from 23 scleroderma patients and 24 normal controls and 7 scleroderma patients who had undergone high dose immunosuppressive therapy followed by autologous hematopoietic cell transplant. Along with the loss of capillaries there was a dramatic change in endothelial phenotype in the residual vessels. The molecules defining this phenotype are: vascular endothelial cadherin, a supposedly universal endothelial marker required for tube formation (lost in the scleroderma tissue, antiangiogenic interferon alpha (overexpressed in the scleroderma dermis and RGS5, a signaling molecule whose expression coincides with the end of branching morphogenesis during development and tumor angiogenesis (also overexpressed in scleroderma skin. Following high dose immunosuppressive therapy, patients experienced clinical improvement and 5 of the 7 patients with scleroderma had increased capillary counts. It was also observed in the same 5 patients, that the interferon alpha and vascular endothelial cadherin had returned to normal as other clinical signs in the skin regressed, and in all 7 patients, RGS5 had returned to normal.These data provide the first objective evidence for loss of vessels in scleroderma and show that this phenomenon is reversible. Coordinate changes in expression of three molecules already implicated in angiogenesis or anti-angiogenesis suggest that control of expression of these three molecules may be the underlying mechanism for at least the vascular component of this disease. Since rarefaction has been little studied, these data may have

  1. Scleroderma renal crisis and ovarian hyperstimulation syndrome related to the use of clomiphene in a patient with scleroderma.

    Science.gov (United States)

    Kobak, Senol; Hacivelioglu, Servet; Gungor, Selen

    2014-01-01

    This paper presented a 28-year-old female with systemic sclerosis who developed scleroderma renal crisis and ovarian hyperstimulation syndrome following clomiphene administration. Urgent therapy including angiotensin-converting enzyme (ACE) inhibitors and supportive care resulted in regression and eventually resolution of all the clinical and laboratory symptoms. Although scleroderma renal crisis is a fatal complication of high-dose corticosteroids, rarely is this seen with the use of ACE inhibitors. This case report aimed to investigate the potential capacity of the selective oestrogen receptor modulator clomiphene to induce scleroderma renal crisis as well as corticosteroids. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  2. Recurrent ovary cancer presenting with scleroderma - A rare case report

    OpenAIRE

    Sargin, Betul; Gurer, Gulcan; Bozbas, Gulnur; Noyan, Fatih; Barut, Kayra; Tataroglu, Canten

    2017-01-01

    Scleroderma is a chronic autoimmune multisystem disorder which is characterizedby progressive fibrosis of the skin and internal organs. Ovary cancers with sclerodermahave been reported in the literature. But recurrent ovary cancer with sclerodermahas not been reported before. Here, we report a 65 -year old female patient presentingwith recurrent ovary cancer and subsequently diagnosed with scleroderma. Due toliterature sources, this is the first case of presenting with recurrent ovary cancera...

  3. Echocardiographic study of the cardiac state in systemic scleroderma

    International Nuclear Information System (INIS)

    Kotel'nikova, G.P.; Guseva, N.G.

    1986-01-01

    Echocardiographic investigation in 65 patients with systemic scleroderma showed that the left ventricular syes and indices of central hemodynamics were frequently lowered in them; an increase in sizes was observed mainly in the patients with stage 2-3 of disease. Echocardiography made it possible to specify the nature of cardiac pathology and brought to light some additional features of the involvement of different heart membranes in systemic scleroderma

  4. Validation of the Social Interaction Anxiety Scale in scleroderma: A Scleroderma Patient-centered Intervention Network cohort study

    NARCIS (Netherlands)

    Gholizadeh, S.; Kwakkenbos, C.M.C.; Carrier, M.E.; Mills, S.D.; Fox, R.S.; Jewett, L.R.; Gottesman, K.; Roesch, S.C.; Thombs, B.D.; Malcarne, V.L.

    2018-01-01

    Introduction: Individuals with visible differences due to medical conditions, such as systemic sclerosis (SSc; scleroderma), have reported difficulty navigating social situations because of issues such as staring, invasive questions, and rude comments. Fears or anxiety linked to situations in which

  5. Juvenile Arthritis

    Science.gov (United States)

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

  6. Treatment of GI dysmotility in scleroderma with the new enterokinetic agent prucalopride

    NARCIS (Netherlands)

    Boeckxstaens, G. E.; Bartelsman, J. F. W. M.; Lauwers, L.; Tytgat, G. N. J.

    2002-01-01

    Scleroderma is a multisystem disorder frequently resulting in disturbed GI motility. Although, especially early in the disease, symptomatic improvement is achieved with prokinetic agents, more severe GI manifestations of scleroderma may be difficult to treat, leading to parenteral feeding and

  7. Novel Insights Into Causes of Scleroderma Offer Potential New Treatment Strategies

    Science.gov (United States)

    ... Spotlight on Research Novel Insights Into Causes of Scleroderma Offer Potential New Treatment Strategies By Kirstie Saltsman, Ph.D. | December 15, 2013 In scleroderma, immune cells invade the skin, but the role ...

  8. Juvenile Justice in Mexico

    Directory of Open Access Journals (Sweden)

    Martha Frías Armenta

    2014-08-01

    . Juveniles lack real access to an adequate defense because they cannot afford to pay lawyers. This disconnection between rights and reality undermines the new system, raising the question of whether recent modifications to bring laws in line with international norms are in fact advancing juvenile justice. By approaching the Mexican juvenile justice systems as a single, multilayered system combining international, federal and local laws and procedures, we can better describe some of the substantive inconsistencies that continue to prevail, even as new ones develop in terms of children’s rights.

  9. Lung diffusion of soluble radioaerosols in scleroderma

    International Nuclear Information System (INIS)

    Chopra, S.K.; Taplin, G.V.; Tashkin, D.P.; Elam, D.

    1978-01-01

    Diffusion rates of soluble radioaerosols of sodium pertechnetate (/sup 99m/TcO 4 ; mol. wt. 163) and diethylentriaminepentaacetate (/sup 99m/Tc-DTPA; mol. wt. 492) were determined in ten normal subjects and ten patients with scleroderma having lung involvement. Twenty millicuries (mCi) each of /sup 99m/TcO 4 and /sup 99m/Tc-DTPA in 5 ml saline were aerosolized and inhaled on two different days. Initial lung retention after three minutes of administration was approximately 2 mCi. Two regions of interest over each posterior lung field were monitored with a scintillation camera and data were stored on magnetic tape. Decreasing levels of radioactivity were plotted semilogarithmically and half time (T 1 / 2 ) removal rates were calculated

  10. Juvenile Firesetting.

    Science.gov (United States)

    Peters, Brittany; Freeman, Bradley

    2016-01-01

    Juvenile firesetting is a significant cause of morbidity and mortality in the United States. Male gender, substance use, history of maltreatment, interest in fire, and psychiatric illness are commonly reported risk factors. Interventions that have been shown to be effective in juveniles who set fires include cognitive behavior therapy and educational interventions, whereas satiation has not been shown to be an effective intervention. Forensic assessments can assist the legal community in adjudicating youth with effective interventions. Future studies should focus on consistent assessment and outcome measures to create more evidence for directing evaluation and treatment of juvenile firesetters. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  12. Biomarkers in Scleroderma: Progressing from Association to Clinical Utility.

    Science.gov (United States)

    Ligon, Colin; Hummers, Laura K

    2016-03-01

    Scleroderma is a heterogenous disease characterized by autoimmunity, a characteristic vasculopathy, and often widely varying extents of deep organ fibrosis. Recent advances in the understanding of scleroderma's evolution have improved the ability to identify subgroups of patients with similar prognosis in order to improve risk stratification, enrich clinical trials for patients likely to benefit from specific therapies, and identify promising therapeutic targets for intervention. High-throughput technologies have recently identified fibrotic and inflammatory effectors in scleroderma that exhibit strong prognostic ability and may be tied to disease evolution. Increasingly, the use of collections of assayed circulating proteins and patterns of gene expression in tissue has replaced single-marker investigations in understanding the evolution of scleroderma and in objectively characterizing disease extent. Lastly, identification of shared patterns of disease evolution has allowed classification of patients into latent disease subtypes, which may allow rapid clinical prognostication and targeted management in both clinical and research settings. The concept of biomarkers in scleroderma is expanding to include nontraditional measures of aggregate protein signatures and disease evolution. This review examines the recent advances in biomarkers with a focus on those approaches poised to guide prospective management or themselves serve as quantitative surrogate disease outcomes.

  13. Race and Association With Disease Manifestations and Mortality in Scleroderma

    Science.gov (United States)

    Manno, Rebecca L.; Shah, Ami A.; Woods, Adrianne; Le, Elizabeth N.; Boin, Francesco; Hummers, Laura K.; Wigley, Fredrick M.

    2013-01-01

    Abstract Experience suggests that African Americans may express autoimmune disease differently than other racial groups. In the context of systemic sclerosis (scleroderma), we sought to determine whether race was related to a more adverse expression of disease. Between January 1, 1990, and December 31, 2009, a total of 409 African American and 1808 white patients with scleroderma were evaluated at a single university medical center. While the distribution by sex was virtually identical in both groups, at 82% female, African American patients presented to the center at a younger mean age than white patients (47 vs. 53 yr; p scleroderma-specific autoantibody status, and for the socioeconomic measures of educational attainment and health insurance status, diminished these risk estimates (RR, 1.3; 95% CI, 1.0–1.6). The heightened risk of mortality persisted in strata defined by age at disease onset, diffuse cutaneous disease, anticentromere seropositivity, decade of care at the center, and among women. These findings support the notion that race is related to a distinct phenotypic profile in scleroderma, and a more unfavorable prognosis among African Americans, warranting heightened diagnostic evaluation and vigilant care of these patients. Further, we provide a chronologic review of the literature regarding race, organ system involvement, and mortality in scleroderma; we furnish synopses of relevant reports, and summarize findings. PMID:23793108

  14. Cancer and Scleroderma: A Paraneoplastic Disease with Implications for Malignancy Screening

    Science.gov (United States)

    Shah, Ami A.; Rosen, Livia Casciola

    2015-01-01

    Purpose of review Recent data suggest a paraneoplastic mechanism of scleroderma pathogenesis in unique subsets of scleroderma patients. In this article, we review these data, explore potential links between cancer and scleroderma, and propose an approach to malignancy screening in scleroderma. Recent findings Emerging data have demonstrated that patients with scleroderma and RNA polymerase III autoantibodies have a significantly increased risk of cancer within a few years of scleroderma onset. Genetic alterations in the gene encoding RNA polymerase III (POLR3A) have been identified, and patients with somatic mutations in POLR3A have evidence of mutation specific T cell immune responses with generation of cross-reactive RNA polymerase III autoantibodies. These data strongly suggest that scleroderma is a by-product of anti-tumor immune responses in some patients. Additional epidemiologic data demonstrate that patients developing scleroderma at older ages may also have a short cancer-scleroderma interval, suggestive of paraneoplastic disease. Summary Scleroderma may be a paraneoplastic disease in unique patient subsets. Aggressive malignancy screening in these patients may aid in early cancer detection. Further study is required to determine whether cancer therapy could improve scleroderma outcomes in this patient population. PMID:26352736

  15. Scleroderma of geriatric age and scleroderma-like paraneoplastic syndrome – description of two cases

    Directory of Open Access Journals (Sweden)

    Magdalena Marek

    2016-06-01

    Full Text Available Systemic sclerosis (Ssc is an autoimmune connective tissue disease of unknown origin, characterized by progressive fibrosis of the skin and internal organs. Immune reactions taking part in Ssc pathogenesis may contribute to cancer development; therefore patients with risk factors for this disease require observation for a neoplastic process. On the other hand, symptoms of Ssc may be a mask of various cancers. Differentiating between the idiopathic form of Ssc and scleroderma-like paraneoplastic syndrome often causes a lot of difficulties. The article presents two cases of Ssc at the beginning of the disease after 60 years of age. The first case was diagnosed as Ssc, whereas in the second case the defined diagnosis was scleroderma-like syndrome in the course of colorectal cancer. This paper presents an analysis of differential diagnostic procedures which were performed and led to the final diagnosis, mentions types of cancers co-occurring with Ssc and suggests a screening scheme for cancer development in patients with a diagnosis of Ssc.

  16. Molecular subsets in the gene expression signatures of scleroderma skin.

    Directory of Open Access Journals (Sweden)

    Ausra Milano

    2008-07-01

    Full Text Available Scleroderma is a clinically heterogeneous disease with a complex phenotype. The disease is characterized by vascular dysfunction, tissue fibrosis, internal organ dysfunction, and immune dysfunction resulting in autoantibody production.We analyzed the genome-wide patterns of gene expression with DNA microarrays in skin biopsies from distinct scleroderma subsets including 17 patients with systemic sclerosis (SSc with diffuse scleroderma (dSSc, 7 patients with SSc with limited scleroderma (lSSc, 3 patients with morphea, and 6 healthy controls. 61 skin biopsies were analyzed in a total of 75 microarray hybridizations. Analysis by hierarchical clustering demonstrates nearly identical patterns of gene expression in 17 out of 22 of the forearm and back skin pairs of SSc patients. Using this property of the gene expression, we selected a set of 'intrinsic' genes and analyzed the inherent data-driven groupings. Distinct patterns of gene expression separate patients with dSSc from those with lSSc and both are easily distinguished from normal controls. Our data show three distinct patient groups among the patients with dSSc and two groups among patients with lSSc. Each group can be distinguished by unique gene expression signatures indicative of proliferating cells, immune infiltrates and a fibrotic program. The intrinsic groups are statistically significant (p<0.001 and each has been mapped to clinical covariates of modified Rodnan skin score, interstitial lung disease, gastrointestinal involvement, digital ulcers, Raynaud's phenomenon and disease duration. We report a 177-gene signature that is associated with severity of skin disease in dSSc.Genome-wide gene expression profiling of skin biopsies demonstrates that the heterogeneity in scleroderma can be measured quantitatively with DNA microarrays. The diversity in gene expression demonstrates multiple distinct gene expression programs in the skin of patients with scleroderma.

  17. Limited scleroderma and early detection of visceral changes

    International Nuclear Information System (INIS)

    Kalyuzhnaya, L.D.; Potsibina, V.V.; Stychinskaya, L.P.; Turik, N.V.

    1989-01-01

    The state of liver, kidneys, osteoarticular apparatus at the early stages of development of limited scleroderma and with the exclusion of visceral changes on the basis of clinical-laboratory studies is investigated. 11 patients with scleroderma in the age of 7-18 years were examined. Osteoscintigraphy with 99m TC-phosphone and dynamic scintigraphy of kidneys without additional introduction of RF, and hepatocholecyctoscintigraphy with 99m tc-HIPA of the patients were realized. The conclusion is made that radionuclide investigation methods permit to reveal various visceral changes, which are not recognizable by clinical methods

  18. Juvenile angiofibromer

    DEFF Research Database (Denmark)

    Thuesen, Anne Daugaard; Jakobsen, John; Nepper-Rasmussen, Jørgen

    2005-01-01

    Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through...... the years, the treatment of juvenile angiofibroma has included many methods, including surgical excision, electrocoagulation, interstitial or external radiation therapy, cryosurgery, hormone administration and chemotherapy. Radiation, chemotherapy and surgery have proven to be the most effective treatments...

  19. Sex-specific effects of the local social environment on juvenile post-fledging dispersal in great tits

    NARCIS (Netherlands)

    Michler, Stephanie P. M.; Nicolaus, Marion; Ubels, Richard; van der Velde, Marco; Komdeur, Jan; Both, Christiaan; Tinbergen, Joost M.; Gibson, R.

    2011-01-01

    An individual's decision to disperse from the natal habitat can affect its future fitness prospects. Especially in species with sex-biased dispersal, we expect the cost benefit balance for dispersal to vary according to the social environment (e.g., local sex ratio and density). However, little is

  20. Exploring Sources of Emotional Distress among People Living with Scleroderma: A Focus Group Study.

    Directory of Open Access Journals (Sweden)

    Stephanie T Gumuchian

    Full Text Available Systemic sclerosis, or scleroderma, is a chronic and rare connective tissue disease with negative physical and psychological implications. Sources of emotional distress and the impact they have on the lives of people with scleroderma are not well understood.To gain an in-depth understanding of the emotional experiences and sources of emotional distress for women and men living with scleroderma through focus group discussions.Three semi-structured focus group discussions were conducted (two in English, one in French with a total of 22 people with scleroderma recruited through the Scleroderma Society of Ontario in Hamilton, Ontario and a scleroderma clinic in Montreal, Canada. Interviews were recorded, transcribed, and then coded for emerging themes using thematic inductive analysis.Core themes representing sources of emotional distress were identified, including: (a facing a new reality; (b the daily struggle of living with scleroderma; (c handling work, employment and general financial burden; (d changing family roles; (e social interactions; and (f navigating the health care system. Collectively, these themes refer to the stressful journey of living with scleroderma including the obstacles faced and the emotional experiences beginning prior to receiving a diagnosis and continuing throughout the participants' lives.Scleroderma was portrayed as being an unpredictable and overwhelming disease, resulting in many individuals experiencing multiple sources of emotional distress. Interventions and supportive resources need to be developed to help individuals with scleroderma and people close to them manage and cope with the emotional aspects of the disease.

  1. A Giant Juvenile Nasopharyngeal Angiofibroma

    Science.gov (United States)

    Yüce, Salim; Uysal, İsmail Önder; Doğan, Mansur; Polat, Kerem; Şalk, İsmail; Müderris, Suphi

    2012-01-01

    Juvenile nasopharyngeal angiofibroma (JNA) are locally growing highly vascular tumours. They are treated primarily by surgical excision ranging from open approach to endoscopic approach. We presented a 20-year-old male with a giant nasopharyngeal juvenile angiofibroma obliterating the pterygopalatine fossa bilaterally, invasing the sphenoid bone and extending to the left nasal passage. His complaints were epistaxis and nasal obstruction. After embolization, the patient was treated surgically with endoscopic approach and discharged as cured without any complication. PMID:23714961

  2. Primary biliary cirrhosis and scleroderma complicated by Barrett's ...

    African Journals Online (AJOL)

    1991-04-06

    Apr 6, 1991 ... primary biliary cirrhosis, CREST syndrome, and chronic pancreatitis. Thorax. 1983; 38: 316-317. 9. Okano Y, Nisbikai M, Sato A. Scleroderma, primary biliary cirrhosis, and. Sjogren's syndrome after cosmetic breast augmentation with silicone injec- tion: a case reporfof possible human adjuvant disease.

  3. Pediatric morphea (localized scleroderma): review of 136 patients.

    Science.gov (United States)

    Christen-Zaech, Stéphanie; Hakim, Miriam D; Afsar, F Sule; Paller, Amy S

    2008-09-01

    Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement. We sought to determine the clinical features of morphea in a large pediatric cohort. We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006. Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients. Two patients with rapidly progressing generalized or extensive linear morphea and arthralgias developed restrictive pulmonary disease. Initial oral corticosteroid treatment and long-term methotrexate administration stabilized and/or led to disease improvement in most patients with aggressive disease. Retrospective analysis, relatively small sample size, and risk of a selected referral population to the single site are limitations. These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. Visceral manifestations rarely occur; the presence of progressive problematic cutaneous disease and arthralgias should trigger closer patient monitoring.

  4. MRI findings in deep and generalized morphea (localized scleroderma).

    Science.gov (United States)

    Horger, Marius; Fierlbeck, Gerhard; Kuemmerle-Deschner, Jasmin; Tzaribachev, Nikolay; Wehrmann, Manfred; Claussen, Claus D; Fritz, Jan

    2008-01-01

    Our objective was to describe the spectrum of MRI features in patients with deep and generalized morphea. Imaging features of morphea are not specific and usually overlap with those of other disorders involving the skin, fascia, and musculature, such as some types of fasciitis, myositis, and so forth. Nevertheless, the imaging features of morphea reflect pathomorphologic changes of this rare disorder and enable a complete assessment of the disease extent, including depth of infiltration and disease activity.

  5. Effects of replacing fishmeal with wastes derived from local fisheries on the growth of juvenile tilapia, Oreochromis niloticus

    Directory of Open Access Journals (Sweden)

    Chanagun Chitmanat

    2009-01-01

    Full Text Available A feeding trial was conducted to investigate the effects of partially and totally replacing fishmeal with by-product derived from local fisheries on growth performances of tilapia (Oreochromis niloticus. Tilapia fingerlings (average initialweight 0.38±0.05 g were fed with 5 different diet formulas composing of fish meal protein replacement levels of 0%, 25%,50%, 75, and 100%. Tilapia were raised in 80 x 80 x 80 cm3 hapa in an earthen pond for 8 weeks. Each treatment contained3 replications. No feeding trial was applied as negative control. The result showed that fish by-product powder could substitute for fishmeal on a crude protein basis at a level of not more than 25%. As a result, feed cost can only be cut down approximately 3 US cents/kg. Specific growth rate, weight gain, survival rate and feed conversion ratio were not significantly different between the fish fed with the 75% and 100% fishmeal containing diets (P>0.05. The outcome would beapplied to reduce the solid wastes from fish processing and partially replace the imported fishmeal. This can also be used as guideline for farmers in small communities to produce their own tilapia feed.

  6. Clinical and Autoimmune Profile of Scleroderma Patients from Western India

    Directory of Open Access Journals (Sweden)

    Vandana Pradhan

    2014-01-01

    Full Text Available Background. Systemic sclerosis (SSc, scleroderma is a disorder characterized by fibrosis of skin and visceral organs. Pathogenesis of scleroderma is complex and is incompletely understood as yet. Autoantibodies in SSc represent a serologic hallmark which have clinical relevance, with diagnostic and prognostic potential. Objectives. To study distribution of clinical manifestations and to identify frequency of autoantibodies among subtypes of scleroderma patients from Western India. Methodology. One hundred and ten scleroderma patients were clinically classified according to the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR criteria. All these patients were in active stage of disease. Clinical manifestations were recorded at the time of presentation. Autoantibodies were tested in them by indirect immunofluorescence test and ELISA. Immunoglobulin levels were estimated by nephelometer. These parameters were further correlated with clinical presentation of the disease. Results. Scleroderma patients had M : F ratio of 1 : 10 where mean age at evaluation was 34.7±10.7 years and a mean disease duration was 43.7±35 months. Clinical subtypes showed that 45 patients (40.9% had diffused cutaneous (dcSSc lesions, 32 patients (29.1% had limited cutaneous (lcSSc lesions, and 33 patients (30% had other autoimmune overlaps. The overall frequency of ANA in SSc patients studied was 85.5%. The frequency of anti-Scl70, anti-centromere, anti-endothelial cell antibodies (AECA, and anti-keratinocyte antibodies (AKA was 62.7%, 22.7%, 30%, and 40.9%, respectively. Anti-Scl70 antibodies were significantly high (75.6% versus 46.9% among dcSSc patients (P<0.0115 whereas anti-centromere antibodies were significantly high (9% versus 38% among lcSSc patients when these two subtypes were compared (P<0.0044. Conclusion. This study supports that there are geoepidemiological variations among scleroderma patients for their clinical

  7. What Is Juvenile Arthritis?

    Science.gov (United States)

    ... Initiative Breadcrumb Home Health Topics English Español Juvenile Arthritis Basics In-Depth Download Download EPUB Download PDF What is it? Points To Remember About Juvenile Arthritis Juvenile arthritis is the term used to describe ...

  8. Juvenile rheumatoid arthritis

    Science.gov (United States)

    ... joints. This form of JIA may turn into rheumatoid arthritis. It may involve 5 or more large and ... no known prevention for JIA. Alternative Names Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ...

  9. Predictive value of European Scleroderma Group Activity Index in an early scleroderma cohort.

    Science.gov (United States)

    Nevskaya, Tatiana; Baron, Murray; Pope, Janet E

    2017-07-01

    To estimate the effect of disease activity, as measured by the European Scleroderma Research Group Activity Index (EScSG-AI), on the risk of subsequent organ damage in a large systemic sclerosis (SSc) cohort. Of 421 SSc patients from the Canadian Scleroderma Research Group database with disease duration of ⩽ 3 years, 197 who had no evidence of end-stage organ damage initially and available 3 year follow-up were included. Disease activity was assessed by the EScSG-AI with two variability measures: the adjusted mean EScSG-AI (the area under the curve of the EScSG-AI over the observation period) and persistently active disease/flare. Outcomes were based on the Medsger severity scale and included accrual of a new severity score (Δ ⩾ 1) overall and within organ systems or reaching a significant level of deterioration in health status. After adjustment for covariates, the adjusted mean EScSG-AI was the most consistent predictor of risk across the study outcomes over 3 years in dcSSc: disease progression defined as Δ ⩾ 1 in any major internal organ, significant decline in forced vital capacity and diffusing capacity of carbon monoxide, severity of visceral disease and HAQ Disability Index worsening. In multivariate analysis, progression of lung disease was predicted solely by adjusted mean EScSG-AI, while the severity of lung disease was predicted the adjusted mean EScSG-AI, older age, modified Rodnan skin score (mRSS) and initial severity. The EScSG-AI was associated with patient- and physician-assessed measures of health status and overpowered the mRSS in predicting disease outcomes. Disease activity burden quantified with the adjusted mean EScSG-AI predicted the risk of deterioration in health status and severe organ involvement in dcSSc. The EScSG-AI is more responsive when done repeatedly and averaged. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email

  10. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis

    Directory of Open Access Journals (Sweden)

    Serena Bonin

    2011-09-01

    Full Text Available Lichen sclerosus et atrophicans (LSA is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. The case was associated with Borrelia infection. The lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr /day i.v for 21 days associated with UVA-1 therapy and local and systemic vitamin E supply (400 mg 2x/day per os for 3 months. This case stresses the importance of identifying clinical manifestations associated with Lyme disease and the use of tissue PCR to detect borrelial DNA in patients with these lesions, but characterized by negative serology for Borrelia.

  11. Barrett esophagus in scleroderma: Increased incidence and radiographic findings

    International Nuclear Information System (INIS)

    Recht, M.P.; Levine, M.S.; Katzka, D.A.; Reynolds, J.C.; Saul, S.H.; Ouyang, A.; Cohen, S.

    1986-01-01

    Thirteen patients with scleroderma underwent esophagography and endoscopy because of symptoms of reflux esophagitis or dysphagia, or both. Five had biopsy-proved Barrett esophagus, and two of those five had esophageal adencarcinomas. In a blinded review of the barium studies, Barrett esophagus was thought to be probable in one case, possible in seven, and unlikely in five. Five of eight patients in the first two groups had Barrett esophagus on endoscopy, but none in the third group had this disease. The author's initial experience, therefore, suggests that a definitive radiologic diagnosis of Barrett esophagus is not possible in most patients with scleroderma. However, endoscopy may be avoided when Barrett esophagus is considered unlikely on esophagography

  12. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  13. Use of the international systemic scleroderma activity index

    Directory of Open Access Journals (Sweden)

    Maya Nikolayevna Starovoitova

    2013-01-01

    Full Text Available Up to now, it is difficult to determine systemic scleroderma (SSD activity because of the lack of validated tools to estimate changes in the pathological process. Attempts have been made to develop unified activity assessing methods for many years. The indices proposed by the European SSD Group are most popular today. This paper gives the results of using this index in a cohort of Russian patients.

  14. The skeletal changes of progressive systemic scleroderma (PSS), of exclusive Raynaud phenomenon and of circumscript scleroderma in a gamma camera scintigraph

    International Nuclear Information System (INIS)

    Ulbrich, T.

    1987-01-01

    The bases of this study are the skeletal scintigraphs of altogether 34 patients with the three forms of scleroderma mentioned in the title. The PSS shows a polytropic, symmetric distribution pattern, the exclusive Raynaud's phenomenon shows a basic skeleton which is unchanged in contrast to circumscript scleroderma, with which also the larger joints are relatively more frequently attacked than the little ones. As a result of high sensitivity skeletal scintiscanning makes possible an early recognition of osseous changes and is there with superior to X-radiology and should then see an increased use for the clarification of the diagnosis of arthropathy in scleroderma. (TRV) [de

  15. The EULAR Scleroderma Trials and Research Group (EUSTAR): an international framework for accelerating scleroderma research.

    Science.gov (United States)

    Tyndall, Alan; Ladner, Ulf M; Matucci-Cerinic, Marco

    2008-11-01

    Systemic sclerosis has a complex pathogenesis and a multifaceted clinical spectrum without a specific treatment. Under the auspices of the European League Against Rheumatism, the European League Against Rheumatism Scleroderma Trials And Research group (EUSTAR) has been founded in Europe to foster the study of systemic sclerosis with the aim of achieving equality of assessment and care of systemic sclerosis patients throughout the world according to evidence-based principles. EUSTAR created the minimal essential data set, a simple two-page form with basic demographics and mostly yes/no answers to clinical and laboratory parameters, to track patients throughout Europe. Currently, over 7000 patients are registered from 150 centres in four continents, and several articles have been published with the data generated by the minimal essential data set. A commitment of EUSTAR is also to teaching and educating, and for this reason there are two teaching courses and a third is planned for early in 2009. These courses have built international networks among young investigators improving the quality of multicentre clinical trials. EUSTAR has organized several rounds of 'teach the teachers' to further standardize the skin scoring. EUSTAR activities have extended beyond European borders, and EUSTAR now includes experts from several nations. The growth of data and biomaterial might ensure many further fruitful multicentre studies, but the financial sustainability of EUSTAR remains an issue that may jeopardize the existence of this group as well as that of other organizations in the world.

  16. Esclerose sistêmica e sarcoidose Scleroderma and sarcoidosis

    Directory of Open Access Journals (Sweden)

    Fernanda Guidolin

    2005-10-01

    Full Text Available Os autores descrevem o caso de uma paciente com esclerose sistêmica (ES - forma limitada - com comprometimento pulmonar tipo fibrose intersticial. Após sete anos sem acompanhamento, foram identificados gânglios mediastinais e esplenomegalia. A biópsia de linfonodos mostrou granuloma não caseoso sugestivo de sarcoidose. Estamos mostrando, neste caso, a associação de ES e sarcoidose, para chamar a atenção para esse fato e enfatizar que a sarcoidose deve ser lembrada no diagnóstico diferencial das complicações pulmonares da esclerodermia.The authors describe the case of a patient with limited scleroderma and interstitial lung disease. Follow-up was lost for seven years, when patient returned presenting nodular mediastinal enlargement and splenomegaly. Lymph node biopsy showed granulomatous lesions without caseum suggestive of sarcoidosis. This case is being presented to remind the association of scleroderma and sarcoidosis as a possible differential diagnosis of scleroderma pulmonary complications.

  17. Scleroderma in the Caribbean: characteristics in a Dominican case series.

    Science.gov (United States)

    Gottschalk, Paola; Vásquez, Ricardo; López, Persio David; Then, Jossiell; Tineo, Carmen; Loyo, Esthela

    2014-01-01

    Scleroderma is a rare disease with limited data in Latin America. Preliminary genetic studies suggest a strong African ascendance in the Dominican Republic, which could modulate the expression of the disease. The objective of this study is to describe the clinical and demographic characteristics of scleroderma in a series of 26 Dominican patients. Patients who fulfilled the EULAR/ACR criteria for scleroderma were selected from the Rheumatology Department of a tertiary health center; systemic sclerosis subtypes were defined according to the EULAR classification. Clinical and demographic information was obtained retrospectively from clinical records. Mean age at time of onset was 32.6±15 years; 68% of patients had 40 years of age or less. 73% of patients was feminine, with a female:male ratio of 2.7:1. The most affected systems were pulmonary and gastrointestinal; renal affection was scarce. Anti-Scl-70 antibodies were positive in 64% of patients, sometimes in coexistence with anti-centromere antibodies. The prevalence of systemic sclerosis is lower in the Dominican population than the reported elsewhere. The age of onset of the disease seems to be lower in the Dominican population than that reported in literature. A different pattern of autoantibodies is observed in this population. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  18. Discovery or Extinction of New Scleroderma Species in Amazonia?

    Directory of Open Access Journals (Sweden)

    Iuri G Baseia

    Full Text Available The Amazon Forest is a hotspot of biodiversity harboring an unknown number of undescribed taxa. Inventory studies are urgent, mainly in the areas most endangered by human activities such as extensive dam construction, where species could be in risk of extinction before being described and named. In 2015, intensive studies performed in a few locations in the Brazilian Amazon rainforest revealed three new species of the genus Scleroderma: S. anomalosporum, S. camassuense and S. duckei. The two first species were located in one of the many areas flooded by construction of hydroelectric dams throughout the Amazon; and the third in the Reserva Florestal Adolpho Ducke, a protected reverse by the INPA. The species were identified through morphology and molecular analyses of barcoding sequences (Internal Transcribed Spacer nrDNA. Scleroderma anomalosporum is characterized mainly by the smooth spores under LM in mature basidiomata (under SEM with small, unevenly distributed granules, a characteristic not observed in other species of the genus, the large size of the basidiomata, up to 120 mm diameter, and the stelliform dehiscence; S. camassuense mainly by the irregular to stellate dehiscence, the subreticulated spores and the bright sulfur-yellow colour, and Scleroderma duckei mainly by the verrucose exoperidium, stelliform dehiscence, and verrucose spores. Description, illustration and affinities with other species of the genus are provided.

  19. End-Stage Kidney Disease From Scleroderma in the United States, 1996 to 2012

    Directory of Open Access Journals (Sweden)

    Donal J. Sexton

    2018-01-01

    Conclusion: The incidence of ESKD from scleroderma appears to have declined in the United States since 1996. ESKD from scleroderma is associated with an enhanced likelihood of recovery of kidney function and death, a reduced likelihood of transplantation, and similar outcomes after transplantation.

  20. Exploring sources of emotional distress among people living with scleroderma: A focus group study

    NARCIS (Netherlands)

    Gumuchian, S.T.; Peláez, S.; Delisle, V.C.; Carrier, M.E.; Jewett, L.R.; El-Baalbaki, G.; Fortune, C.; Hudson, M.; Impens, A.; Körner, A.; Persmann, J.; Kwakkenbos, C.M.C.; Bartlett, S.J.; Thombs, B.D.

    2016-01-01

    Background: Systemic sclerosis, or scleroderma, is a chronic and rare connective tissue disease with negative physical and psychological implications. Sources of emotional distress and the impact they have on the lives of people with scleroderma are not well understood. Objectives: To gain an

  1. Assessment of Microvascular Abnormalities by Nailfold Capillaroscopy in Juvenile Dermatomyositis After Medium- to Long-Term Followup.

    Science.gov (United States)

    Barth, Zoltan; Witczak, Birgit N; Flatø, Berit; Koller, Akos; Sjaastad, Ivar; Sanner, Helga

    2018-05-01

    In juvenile dermatomyositis (DM), microvascular abnormalities, measured by nailfold capillaroscopy (NFC), are common early in the disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to long-term juvenile DM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. Fifty-eight juvenile DM patients with a median disease duration of 16.8 (range 2-38) years were clinically examined and compared with matched controls. By NFC, we assessed nailfold capillary density (NCD), giant capillaries, scleroderma, and neovascular pattern (defined as scleroderma active or late pattern). NFC was analyzed with researchers blinded to patient/control identity and disease characteristics. We measured disease activity and damage by validated tools, and patients were categorized as having active or inactive disease according to the Paediatric Rheumatology International Trials Organisation criteria. Compared to controls, patients had decreased NCD (mean ± SD 6.4 ± 2.1/mm versus 7.6 ± 0.8/mm; P = 0.001) and showed more abnormality in all other NFC parameters; 36% of patients versus 4% of controls had NCD <6/mm (P < 0.001). Giant capillaries, scleroderma, and neovascular pattern were found in 9%, 84%, and 41% of patients, respectively. Patients with active disease (n = 30) presented more frequently with neovascular pattern than patients with inactive disease (n = 28) (P = 0.041). Decreased NCD and neovascular pattern were associated with higher levels of disease activity and impaired muscle function. After medium- to long-term followup, juvenile DM patients had decreased NCD and, often, neovascular pattern; both were associated with higher levels of disease activity and impaired muscle function. This suggests that NFC can be a biomarker for disease activity in longstanding juvenile DM too. © 2017, American College of Rheumatology.

  2. [Clinical, endoscopic and morphological manifestations of oesophageal lesion in systemic scleroderma].

    Science.gov (United States)

    Karateev, A E; Movsiian, A E; Anan'eva, M M; Radenska-Lopovok, S G

    2014-01-01

    Oesophageal lesion is the commonest visceral manifestation of systemic scleroderma (SSD) affecting the quality of life and fraught with serious complications. The aim of this study was to evaluate clinical, endoscopic andmorphological manifestations of oesophageal lesion in systemic scleroderma and its relationships with other clinical symptoms and pharmacotherapy of the disease. 479 patients with SSD (93.7% women, 6.3% men, mean age 48.7 +/- 19.2 yr). All of them underwent EGDS in 2005-2010. 123 patients were examined for the detection of Barrett's oesophagus (BO), total screening regardless of complaints was conducted in 2010. Control group included 1018 age and sex-matched patients with RA who underwent EGDS in 2008-2009. Oesophageal lesions occurred much more frequently in SSD than in RA. Oesophageal symptoms were documented in 70.0 and 29.9% cases, non-erosive oesopahgitis in 28.8 and 1.5%, erosive esophagitis in 22.5 and 2.2% ulcers in 0.8 and 0% (p < 0.001). BO manifested as intestinal metaplasia (histological study of mucosal biopsy) was found in 30 SSD patients (4.2%). Screening revealed BO in 8.9% of the patients. The development of erosive oesophagitis was unrelated to the age of the patients, duration of the disease and its form (localized or diffusive), lung pathology or Sjogren's syndrome. Cytotoxic medicines significantly increased the frequency of erosive oesophagitis, it tended to increase under effect of NSAID and low doses of aspirin. Long-term intake of PPI did not reduce the risk of oesophagitis and BO. Half of the patients with SSD have oesophagitis. Over 20% of them suffer its complications (erosion and ulcers) and 9% have BO. All such patients need endoscopic study ofoesophagus regardless of clinical symptoms.

  3. Oligosaccharide modification by N-acetylglucosaminyltransferase-V in macrophages are involved in pathogenesis of bleomycin-induced scleroderma.

    Science.gov (United States)

    Kato, Arisa; Yutani, Mizuki; Terao, Mika; Kimura, Akihiro; Itoi, Saori; Murota, Hiroyuki; Miyoshi, Eiji; Katayama, Ichiro

    2015-08-01

    Oligosaccharide modification by N-acetylglucosaminyltransferase-V (GnT-V), which catalyses the formation of β1,6 GlcNAc (N-acetylglucosamine) branches on N-glycans, is associated with various pathologies, such as cancer metastasis, multiple sclerosis and liver fibrosis. In this study, we demonstrated the involvement of GnT-V in the pathophysiology of scleroderma. High expression of GnT-V was observed in infiltrating cells in skin section samples from systemic and localized patients with scleroderma. Most of the infiltrating cells were T cells and macrophages, most of which were CD163(+) M2 macrophages. To determine the role of GnT-V in scleroderma, we next investigated skin sclerosis in GnT-V knockout (MGAT5(-/-) ) mice. Expression of GnT-V was also elevated in bleomycin (BLM)-injected sclerotic skin, and MGAT5(-/-) mice were resistant to BLM-induced skin sclerosis with reduced collagen type 1 α1 content, suggesting the biological significance of GnT-V in skin sclerosis. Furthermore, the number of CD163(+) M2 macrophages and CD3-positive T cells in BLM-induced skin sclerosis was significantly fewer in MGAT5(-/-) mice. In bone marrow-derived macrophages (BMDMs), IL-4-induced expressions of Fizz1 and Ym1 were significantly reduced in MGAT5(-/-) mice-derived BMDMs. Taken together, these results suggest the induction of GnT-V in skin sclerosis progression is possibly dependent on increased numbers of M2 macrophages in the skin, which are important for tissue fibrosis and remodelling. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  4. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    Directory of Open Access Journals (Sweden)

    Kimberly P. Liang

    2011-01-01

    Full Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse; ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43% died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

  5. Scleroderma Renal Crisis: A Reversible Cause of Left Ventricular Dysfunction.

    Science.gov (United States)

    Martínez-Milla, Juan; Gaebelt, Hans Paul; Sánchez-Pernaute, Olga; Kallmeyer, Andrea; Romero, José; Farré, Jerónimo

    2018-05-02

    We report a case of acute left ventricular dysfunction due to myocarditis, in the setting of a scleroderma renal crisis. The case is particularly intriguing for the favorable outcome of both symptoms and heart function following immunosuppressive therapy. We also highlight the changes observed over time with image techniques as well as in electrocardiograms. Copyright © 2018 Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Tumor-like calcifications with scleroderma. Thibierge-Weissenbach-Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Meyer, E.; Kulenkampff, H.A.; Kortenhaus, H.

    1987-12-01

    In patients with progressive scleroderma, interstitial calcifications are present to a varying extent. They are mostly located in the soft tissues of the fingers, resembling points, commas or dashes. They may also appear as 'calcinosis universalis' and reach a considerable size. Thus they mimic proliferative tumors. Scintigraphy, proving the existence of further calcifications can be helpful. We report the case of a female patient who presented with such a 'pseudotumor' of unusual size, site and extent in the lumbar region.

  7. Renal diagnostic nuclear medicine procedures in progressive systemic scleroderma (PSS)

    Energy Technology Data Exchange (ETDEWEB)

    Ammari, B.; Hotze, A.; Gruenwald, F.; Biersack, H.J.; Blitz, H.; Kuester, W.; Kreysel, H.W.

    1989-02-01

    The involvement of kidneys in progressive systemic scleroderma (PSS) is one of the most frequent causes of death in this disease. Using clinical criteria and laboratory tests only the frequency of kidney involvement would be clearly underestimated. Invasive diagnostic procedures such as biopsy and angiography can not be applied in those patients. Nuclear medicine techniques (hippurate clearance, DMSA-scan), however, offer non invasive and sensitive methods in the diagnosis of renal involvement in PSS patients. In our study 46 of 76 patients (60%) revealed pathologic findings. The mentioned diagnostic techniques show a high sensitivity and are in agreement with pathological findings described in PSS.

  8. Renal diagnostic nuclear medicine procedures in progressive systemic scleroderma (PSS)

    International Nuclear Information System (INIS)

    Ammari, B.; Hotze, A.; Gruenwald, F.; Biersack, H.J.; Blitz, H.; Kuester, W.; Kreysel, H.W.

    1989-01-01

    The involvement of kidneys in progressive systemic scleroderma (PSS) is one of the most frequent causes of death in this disease. Using clinical criteria and laboratory tests only the frequency of kidney involvement would be clearly underestimated. Invasive diagnostic procedures such as biopsy and angiography can not be applied in those patients. Nuclear medicine techniques (hippurate clearance, DMSA-scan), however, offer non invasive and sensitive methods in the diagnosis of renal involvement in PSS patients. In our study 46 of 76 patients (60%) revealed pathologic findings. The mentioned diagnostic techniques show a high sensitivity and are in agreement with pathological findings described in PSS. (orig.) [de

  9. A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face

    Science.gov (United States)

    Kumar, M Hari; Kumar, M Siva; Kumar, Sabitha Hari; Kumar, Kingsly Selva

    2016-01-01

    Limited cutaneous scleroderma is a subtype of scleroderma limited to the skin of the face, hands, feet and forearms. We present a case of a 45-year-old woman affected by limited cutaneous systemic scleroderma involving the orofacial region and causing restricted mouth opening. The patient showed noteworthy improvement of the skin lesion by use of a combination of intralesional corticosteroid with hyaluronidase and various multiantioxidants, resulting in amelioration of her mouth opening problem. The patient gave her full informed written consent to this report being published. PMID:27033280

  10. A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face.

    Science.gov (United States)

    Kumar, M Hari; Kumar, M Siva; Kumar, Sabitha Hari; Kumar, Kingsly Selva

    2016-03-31

    Limited cutaneous scleroderma is a subtype of scleroderma limited to the skin of the face, hands, feet and forearms. We present a case of a 45-year-old woman affected by limited cutaneous systemic scleroderma involving the orofacial region and causing restricted mouth opening. The patient showed noteworthy improvement of the skin lesion by use of a combination of intralesional corticosteroid with hyaluronidase and various multiantioxidants, resulting in amelioration of her mouth opening problem. The patient gave her full informed written consent to this report being published. 2016 BMJ Publishing Group Ltd.

  11. X-ray findings in scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Kauffmann, G.W.; Reinbold, W.D.; Hagedorn, M.

    1983-05-01

    In 28 patients with progressive systemic sclerosis clinical symptoms are correlated with roentgen findings. The systemic disease of connective tissue shows typical roentgenological signs, such as soft tissue calcifications, generalized or localized osteoporosis, arthritis, dysfunction of oesophagus, small and large bowel, pulmonary fibrosis and cardiomegaly. A limited prognostic statement according to a clinical and radiological classification seems possible.

  12. X-ray findings in scleroderma

    International Nuclear Information System (INIS)

    Kauffmann, G.W.; Reinbold, W.D.; Hagedorn, M.; Freiburg Univ.

    1983-01-01

    In 28 patients with progressive systemic sclerosis clinical symptoms are correlated with roentgen findings. The systemic disease of connective tissue shows typical roentgenological signs, such as soft tissue calcifications, generalized or localized osteoporosis, arthritis, dysfunction of oesophagus, small and large bowel, pulmonary fibrosis and cardiomegaly. A limited prognostic statement according to a clinical and radiological classification seems possible. (orig.) [de

  13. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, M.

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted.

  14. Systemic scleroderma diagnosed after undergoing radiation therapy for breast cancer

    International Nuclear Information System (INIS)

    Sasaki, Tetsuo; Kakei, Masae

    1994-01-01

    A case of systemic scleroderma in which the symptoms became prominent after undergoing radiation therapy for breast cancer is reported. A 68-year-old woman, who had undergone a radical mastectomy for breast carcinoma at the age of 63 and thereafter received radiotherapy at 65, 66 and 67 years of age, visited our clinic complaining of skin sclerosis and Raynaud's phenomenon which she had noticed since the age of 65. The physical examination revealed not only postirradiation fibrosis and pigmentation, but also edematous sclerosis and the pigmentation of her extremities, as well as short frenulum of the tongue and digital pitting scars. She demonstrated serum anti-topoisomerase I antibodies, esophageal dysfunction and lung fibrosis. The histopathology of the forearm skin showed edema of the upper dermis as well as increased and homogenized collagen bundles in the middle and lower dermis. Since similar cases have been reported, it is considerable that radiation therapy may have thus worsened the lesions of scleroderma in this patient as well. (author)

  15. Systemic scleroderma diagnosed after undergoing radiation therapy for breast cancer

    Energy Technology Data Exchange (ETDEWEB)

    Sasaki, Tetsuo; Kakei, Masae (Yokohama City Univ. (Japan). Faculty of Medicine)

    1994-10-01

    A case of systemic scleroderma in which the symptoms became prominent after undergoing radiation therapy for breast cancer is reported. A 68-year-old woman, who had undergone a radical mastectomy for breast carcinoma at the age of 63 and thereafter received radiotherapy at 65, 66 and 67 years of age, visited our clinic complaining of skin sclerosis and Raynaud's phenomenon which she had noticed since the age of 65. The physical examination revealed not only postirradiation fibrosis and pigmentation, but also edematous sclerosis and the pigmentation of her extremities, as well as short frenulum of the tongue and digital pitting scars. She demonstrated serum anti-topoisomerase I antibodies, esophageal dysfunction and lung fibrosis. The histopathology of the forearm skin showed edema of the upper dermis as well as increased and homogenized collagen bundles in the middle and lower dermis. Since similar cases have been reported, it is considerable that radiation therapy may have thus worsened the lesions of scleroderma in this patient as well. (author).

  16. Juvenile Court Statistics - 1972.

    Science.gov (United States)

    Office of Youth Development (DHEW), Washington, DC.

    This report is a statistical study of juvenile court cases in 1972. The data demonstrates how the court is frequently utilized in dealing with juvenile delinquency by the police as well as by other community agencies and parents. Excluded from this report are the ordinary traffic cases handled by juvenile court. The data indicate that: (1) in…

  17. Juvenile Court Statistics, 1974.

    Science.gov (United States)

    Corbett, Jacqueline; Vereb, Thomas S.

    This report presents information on juvenile court processing of youth in the U.S. during 1974. It is based on data gathered under the National Juvenile Court Statistical Reporting System. Findings can be summarized as follows: (1) 1,252,700 juvenile delinquency cases, excluding traffic offenses, were handled by courts in the U.S. in 1974; (2) the…

  18. Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins Scleroderma Center and review of the literature.

    Science.gov (United States)

    Gelber, Allan C; Manno, Rebecca L; Shah, Ami A; Woods, Adrianne; Le, Elizabeth N; Boin, Francesco; Hummers, Laura K; Wigley, Fredrick M

    2013-07-01

    Experience suggests that African Americans may express autoimmune disease differently than other racial groups. In the context of systemic sclerosis (scleroderma), we sought to determine whether race was related to a more adverse expression of disease. Between January 1, 1990, and December 31, 2009, a total of 409 African American and 1808 white patients with scleroderma were evaluated at a single university medical center. While the distribution by sex was virtually identical in both groups, at 82% female, African American patients presented to the center at a younger mean age than white patients (47 vs. 53 yr; p scleroderma-specific autoantibody status, and for the socioeconomic measures of educational attainment and health insurance status, diminished these risk estimates (RR, 1.3; 95% CI, 1.0-1.6). The heightened risk of mortality persisted in strata defined by age at disease onset, diffuse cutaneous disease, anticentromere seropositivity, decade of care at the center, and among women. These findings support the notion that race is related to a distinct phenotypic profile in scleroderma, and a more unfavorable prognosis among African Americans, warranting heightened diagnostic evaluation and vigilant care of these patients. Further, we provide a chronologic review of the literature regarding race, organ system involvement, and mortality in scleroderma; we furnish synopses of relevant reports, and summarize findings.

  19. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children

    NARCIS (Netherlands)

    E.F. Elst (Elisabeth); L.W.A. van Suijlekom-Smit (Lisette); A.P. Oranje (Arnold)

    1999-01-01

    textabstractLinear scleroderma is a connective tissue disorder that characteristically involves the skin. Skin induration and pigmentary changes present in a linear distribution. Severe functional and cosmetic disability may occur, especially in growing children. No effective therapy for the

  20. Predictors of end stage lung disease in a cohort of patients with scleroderma

    OpenAIRE

    Morgan, C; Knight, C; Lunt, M; Black, C; Silman, A

    2003-01-01

    Objectives: To estimate the incidence of severe lung disease in patients with scleroderma and identify the combination(s) of features present at first assessment which would be useful to predict future risk of severe lung disease.

  1. INTERSTITIAL LUNG-DISEASE AND MYOSITIS IN A PATIENT WITH SIMULTANEOUSLY OCCURRING SARCOIDOSIS AND SCLERODERMA

    NARCIS (Netherlands)

    GROEN, H; POSTMA, DS; KALLENBERG, CGM

    1993-01-01

    A patient initially presented with sarcoidosis in combination with myositis of sarcoid origin and Raynaud's phenomenon. During the course of his disease, he additionally developed scleroderma. Bronchoalveolar lavage, performed because of increase of interstitial markings in the presence of enlarged

  2. Emergency Surgery in a Patient with Scleroderma - Anaesthetic Challenges: A Case Report

    Directory of Open Access Journals (Sweden)

    Teena Bansal

    2013-06-01

    Full Text Available Scleroderma (progressive systemic sclerosis is a multisystem disease involving the skin, airway, musculoskeletal, gastrointestinal, pulmonary, renal and cardiac systems that can pose a significant challenge for the anaesthetist. The multisystem involvement of scleroderma can impact on every aspect of anaesthetic care especially airway management. There are no specific contraindications to the use of any type of anaesthesia, although the selection must be guided by identification of organ dysfunction. The anaesthetist must be aware of the organs involved, the severity of the disease and the associated anaesthetic considerations and potential risks in order to safely & skilfully manage the patient with scleroderma. We hereby present a case report of a patient with scleroderma for emergency orbital decompression because of orbital cellulitis.

  3. First record of Scleroderma polyrhizum Pers. (Gasteromycetes from Brazil Primeiro registro de Scleroderma polyrhizum Pers. (Gasteromycetes para o Brasil

    Directory of Open Access Journals (Sweden)

    Iuri Goulart Baseia

    2000-08-01

    Full Text Available The ectomycorrhizal, gasteroid fungus, Scleroderma polyrhizum is recorded from Brazil for the first time, growing under Caryocar brasiliense Camb. (Caryocaraceae a widespread native tree of the Brazilian "cerrado" vegetation. Macro and microscopic features were described using basidiocarps from fresh and dried material treated according to the traditional methodology for Gasteromycetes. The characteristics of the material were close to those of the original description given by Persoon. All material collected was associated with roots of C brasiliense.Scleroderma polyrhizum, um gasteromiceto ectomicorrízico, é registrado pela primeira vez para o Brasil, crescendo sob Caryocar brasillense Camb. (Caryiocaraceae espécie arbórea comum e nativa da vegetação de cerrado brasileiro. Os caracteres macro e microscópicos foram descritos a partir de basidiocarpos frescos e secos, segundo a metodologia tradicional utilizada em estudos taxonómicos de Gasteromycetes. As características do material analisado foram similares à descrição original fornecida por Persoon. Todo material coletado estava associado a raízes de C. brasillense.

  4. New directions for patient-centred care in scleroderma: the Scleroderma Patient-centred Intervention Network (SPIN)

    Science.gov (United States)

    Thombs, Brett D.; Jewett, Lisa R.; Assassi, Shervin; Baron, Murray; Bartlett, Susan J.; Costa Maia, Angela; El-Baalbaki, Ghassan; Furst, Daniel E.; Gottesman, Karen; Haythornthwaite, Jennifer A.; Hudson, Marie; Ann Impens, PhD; Korner, Annett; Leite, Catarina; Mayes, Maureen D.; Malcarne, Vanessa L.; Motivala, Sarosh J.; Mouthon, Luc; Nielson, Warren R.; Plante, Diane; Poiraudeau, Serge; Poole, Janet L.; Pope, Janet; Sauve, Maureen; Steele, Russell J.; Suarez-Almazor, Maria E.; Taillefer, Suzanne; van den Ende, Cornelia H.; Erin Arthurs, BSc; Bassel, Marielle; Delisle, Vanessa; Milette, Katherine; Leavens, Allison; Razykov, Ilya; Khanna, Dinesh

    2014-01-01

    Systemic sclerosis (SSc), or scleroderma, is a chronic multisystem autoimmune disorder characterised by thickening and fibrosis of the skin and by the involvement of internal organs such as the lungs, kidneys, gastrointestinal tract, and heart. Because there is no cure, feasibly-implemented and easily accessible evidence-based interventions to improve health-related quality of life (HRQoL) are needed. Due to a lack of evidence, however, specific recommendations have not been made regarding non-pharmacological interventions (e.g. behavioural/psychological, educational, physical/occupational therapy) to improve HRQoL in SSc. The Scleroderma Patient-centred Intervention Network (SPIN) was recently organised to address this gap. SPIN is comprised of patient representatives, clinicians, and researchers from Canada, the USA, and Europe. The goal of SPIN, as described in this article, is to develop, test, and disseminate a set of accessible interventions designed to complement standard care in order to improve HRQoL outcomes in SSc. PMID:22244687

  5. Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

    DEFF Research Database (Denmark)

    Jacobsen, Søren; Halberg, P; Ullman, S

    1998-01-01

    To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement.......To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement....

  6. Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma

    Directory of Open Access Journals (Sweden)

    Fatemeh Mokhtari

    2018-01-01

    Full Text Available Subcorneal pustular dermatosis (SPD or Sneddon-Wilkinson disease is a rare, benign, chronic, sterile pustular eruption which is associated with various systemic diseases including immunoglobinopathies, neoplasms, and autoimmune disorders. This paper reports a case of SPD in a patient with diffuse scleroderma in a 37-year-old woman. The hypothesis that immune dysregulation may play a role in the pathogenesis of SPD was supposed by the coexistence of diffuse scleroderma and SPD in our patient.

  7. Determination of muscle microcirculation of the limbs of healthy persons and patients with scleroderma by means of /sup 133/Xe clearance. 2. Examination of patients with progressive scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Poenitzsch, I.; Wiemers, U.; Haustein, U.F.; Schneider, G. (Karl-Marx-Universitaet, Leipzig (German Democratic Republic). Radiologische Klinik)

    1984-01-01

    By means of /sup 133/Xe muscle clearance the blood flow of the musculus tibialis anterior and the musculus opponens pollicis was determined during nonischemic work and after 3 minutes ischemia in patients with progressive scleroderma and additionally of the ischemic musculus opponens pollicis following contrast baths. In relation to 53 patients with normal vessels the reactive hyperemia of the musculus tibialis anterior after ischemia and of the musculus opponens pollicis after heat, cold as well as arterial flow decreasing was significantly decreased in patients with scleroderma and was as a disturbance of the microcirculation realized. For scientific problems in progressive scleroderma the /sup 133/Xe muscle clearance is suitable as to the musculus opponens pollicis.

  8. Systemic Sclerosis Sine Scleroderma in Mexican Patients. Case Reports.

    Science.gov (United States)

    Vera-Lastra, Olga; Sauceda-Casas, Christian Alexis; Domínguez, María Del Pilar Cruz; Alvarez, Sergio Alberto Mendoza; Sepulceda-Delgado, Jesús

    2017-01-03

    Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  9. Porphyria Cutanea Tarda Presenting with Scleroderma, Ichthyosis, Alopecia, and Vitiligo

    Directory of Open Access Journals (Sweden)

    Megan E. MacGillivray

    2018-05-01

    Full Text Available Porphyria cutanea tarda (PCT is a cutaneous porphyria that presents later in life with cutaneous findings in sun-exposed sites. We report a complex case of PCT in a 67-year-old woman with an unusual constellation of cutaneous findings: scleroderma, acquired ichthyosis, and nonscarring alopecia. Possible triggers for her PCT include tamoxifen treatment for breast cancer and carrier status of the hemochromatosis gene. High-dose chloroquine was used to successfully achieve clinical remission and normalize her uroporphyrins. While on chloroquine she developed extensive classic vitiligo. It is not clear if this is another feature of her complex and unusual PCT, or a consequence of her antimalarial therapy.

  10. A cross-sectional electromyography assessment in linear scleroderma patients

    Science.gov (United States)

    2014-01-01

    Background Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. Methods We conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997–2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern. Results Nine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement. Conclusion In our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed. PMID:25053924

  11. Crecimiento somático y relación ARN/ADN en estadios juveniles de Eucinostomus argenteus (Pisces: Gerreidae en dos localidades del Caribe de Venezuela Somatic growth and RNA/DNA rate of Eucinostomus argenteus (Pisces: Gerreidae juveniles stages at two localities of the Venezuelan

    Directory of Open Access Journals (Sweden)

    Ana Teresa Herrera-Reveles

    2012-03-01

    Full Text Available Con la finalidad de evaluar la asociación de índices de crecimiento en estadios tempranos de peces marinos, se estimó la tasa de crecimiento somático y las condiciones fisiológicas de Eucinostomus argenteus en dos zonas del nor-oriente venezolano: Bahía de Mochima y Golfo de Cariaco. La edad y el crecimiento fueron estimados basados en análisis de otolitos sagitta. Las condiciones fisiológicas fueron evaluadas por medio de las concentraciones de proteínas y la relación ARN/ADN, empleando técnicas espectofotométricas y fluorométricas sobre tejido muscular. Las relaciones entre tallas con la edad y el diámetro de los otolitos resultaron positivas, significativas y ajustadas a un modelo de regresión lineal. Los valores de la tasa de crecimiento reciente oscilaron entre 0.178 y 0.418mm día-1, la tasa de crecimiento retrocalculado varió entre 0.295 y 0.393mm día-1, y la tasa ARN/ADN osciló entre 1.65 y 6.97. No se registraron diferencias entre las zonas de estudio, sin embargo se reportaron diferencias entre localidades. A pesar de no encontrarse correlación entre la tasa de crecimiento y la relación ARN/ADN, los valores reportados sugieren crecimiento positivo de los individuos silvestres en las localidades evaluadas. No obstante, ciertas localidades mostraron valores que indican pobres condiciones nutricionales, pudiendo afectarse a futuro otras tasas vitales.Somatic growth and RNA/DNA rate of Eucinostomus argenteus (Pisces: Gerreidae juveniles stages at two localities of the Venezuelan Caribbean. In order to evaluate the association among growth indices of marine fishes at early life stages, the somatic growth rate and physiological conditions of Eucinostomus argenteus were estimated at two Venezuelan North-East zones: Mochima Bay and Cariaco Gulf. The age and somatic growth rate were estimated based on daily growth increments in sagitta otoliths. The physiological conditions were evaluated with proteins concentrations and RNA

  12. The spectrum of muscle histopathologic findings in 42 weak scleroderma patients

    Science.gov (United States)

    Paik, Julie J.; Wigley, Fredrick M.; Lloyd, Thomas E.; Corse, Andrea M.; Casciola-Rosen, Livia; Shah, Ami A.; Boin, Francesco; Hummers, Laura K.; Mammen, Andrew L.

    2015-01-01

    Objective To determine if distinct muscle pathological features exist in scleroderma subjects with weakness. Methods This retrospective study included weak scleroderma subjects with muscle biopsies available for review. Biopsies were systematically assessed for individual pathologic features including inflammation, necrosis, fibrosis, and acute neurogenic atrophy. Based on the aggregate individual features, biopsies were assigned a histopathologic category of polymyositis, dermatomyositis, necrotizing myopathy, non-specific myositis, “acute denervation”, “fibrosis only”, or “other”. Clinical data analyzed included autoantibody profiles, scleroderma subtype and disease duration, Medsger muscle severity scores, creatine kinase (CK), electromyography (EMG), and muscle magnetic resonance imaging (MRI). Results 42 subjects (79% female and 64% diffuse scleroderma) were included in this study. Necrosis (67%), inflammation (48%), acute neurogenic atrophy (48%), and fibrosis (33%) were the most prevalent pathologic features. The presence of fibrosis was strongly associated with anti-PM-Scl antibodies. Histopathologic categories included non-specific myositis (36%), necrotizing myopathy (21%), dermatomyositis (7%), “acute denervation” (7%), “fibrosis only” (7%), and polymyositis (5%). Disease duration of scleroderma at the time of muscle biopsy was shorter in polymyositis than other histopathologic categories. Patients with anti-PM-Scl and Scl-70 antibodies also had a shorter disease duration than those with other auto-antibody profiles. Conclusion Non-specific myositis and necrotizing myopathy were the most common histopathologic categories in weak scleroderma subjects. Surprisingly, nearly half of the subjects studied had histological evidence of acute motor denervation (acute neurogenic atrophy); this has not been previously reported. Taken together, these observations suggest that a variety of pathologic mechanisms may underlie the development of

  13. Autoantigens targeted in scleroderma patients with vascular disease are enriched in endothelial lineage cells

    Science.gov (United States)

    McMahan, Zsuzsanna H.; Cottrell, Tricia R.; Wigley, Fredrick M.; Antiochos, Brendan; Zambidis, Elias T.; Park, Tea Soon; Halushka, Marc K.; Gutierrez-Alamillo, Laura; Cimbro, Raffaello; Rosen, Antony; Casciola-Rosen, Livia

    2016-01-01

    Objective Scleroderma patients with autoantibodies to centromere proteins (CENPs) and/or interferon-inducible protein 16 (IFI16) are at increased risk of severe vascular complications. We set out to define whether these autoantigens are enriched in cells of the vasculature. Methods Successive stages of embryoid bodies (EBs) as well as vascular progenitors were used to evaluate the expression of scleroderma autoantigens IFI16 and CENP by immunoblotting. CD31 was included to mark early blood vessels. IFI16 and CD31 expression were defined in skin paraffin sections from scleroderma patients and from healthy controls. IFI16 expression was determined by flow cytometry in circulating endothelial cells (CECs) and circulating progenitor cells (CPCs). Results Expression of CENP-A, IFI16 and CD31 was enriched in EBs at days 10 and 12 of differentiation, and particularly in cultures enriched in vascular progenitors (IFI16, CD31, CENPs A and-B). This pattern was distinct from that of comparator autoantigens. Immunohistochemical staining of skin paraffin sections showed enrichment of IFI16 in CD31-positive vascular endothelial cells in biopsies from scleroderma patients and normal controls. Flow cytometry analysis revealed IFI16 expression in CPCs, but minimal expression in CECs. Conclusion Expression of scleroderma autoantigens IFI16 and CENPs, which are associated with severe vascular disease, is increased in vascular progenitors and mature endothelial cells. High level, lineage-enriched expression of autoantigens may explain the striking association between clinical phenotypes and the immune targeting of specific autoantigens. PMID:27159521

  14. Juvenile Confinement in Context

    Science.gov (United States)

    Mendel, Richard A.

    2012-01-01

    For more than a century, the predominant strategy for the treatment and punishment of serious and sometimes not-so-serious juvenile offenders in the United States has been placement into large juvenile corrections institutions, alternatively known as training schools, reformatories, or youth corrections centers. America's heavy reliance on…

  15. Juvenile giant fibroadenoma

    Directory of Open Access Journals (Sweden)

    Vipul Yagnik

    2011-07-01

    Full Text Available Fibroadenomas are benign solid tumor associated with aberration of normal lobular development. Juvenile giant fibroadenoma is usually single and >5 cm in size /or >500 gms in weight. Important differential diagnoses are: phyllodes tumor and juvenile gigantomastia. Simple excision is the treatment of choice.

  16. Juvenile mammary papillomatosis; Papilomatosis juvenil mamaria

    Energy Technology Data Exchange (ETDEWEB)

    Alvarez, M.; Jimenez, A. V. [Hospital Reina Sofia. Cordoba (Spain)

    2001-07-01

    Juvenile mammary papillomatosis is a benign proliferative disease of young patients, generally under 30 years of age. The most frequent clinical presentation is the existence of an elastic and mobile lymph node of the breast. Anatomopathologically, it is characterized because it presents ductal epithelial hyperplasia, sometimes with marked atypia, and there are numerous cysts having different sizes among the findings. It has been associated with an increase in the incidence of breast cancer, both in the patient herself as well as her family. We review the literature on the subject and present the mammographic and ultrasonographic findings of a 22 year old woman diagnosed of juvenile mammary papillomatosis. (Author) 12 refs.

  17. Hippocampal atrophy and developmental regression as first sign of linear scleroderma "en coup de sabre".

    Science.gov (United States)

    Verhelst, Helene E; Beele, Hilde; Joos, Rik; Vanneuville, Benedicte; Van Coster, Rudy N

    2008-11-01

    An 8-year-old girl with linear scleroderma "en coup de sabre" is reported who, at preschool age, presented with intractable simple partial seizures more than 1 year before skin lesions were first noticed. MRI revealed hippocampal atrophy, controlaterally to the seizures and ipsilaterally to the skin lesions. In the following months, a mental and motor regression was noticed. Cerebral CT scan showed multiple foci of calcifications in the affected hemisphere. In previously reported patients the skin lesions preceded the neurological signs. To the best of our knowledge, hippocampal atrophy was not earlier reported as presenting symptom of linear scleroderma. Linear scleroderma should be included in the differential diagnosis in patients with unilateral hippocampal atrophy even when the typical skin lesions are not present.

  18. Experiencing work as a daily challenge: the case of scleroderma.

    Science.gov (United States)

    Mendelson, Cindy; Poole, Janet L; Allaire, Saralynn

    2013-01-01

    Little is known about the physical and discretionary aspects of work that people with scleroderma (SSc) find difficult. This article describes the findings from a study that explored the challenges and adaptations made by individuals with SSc to continue to work. Thirty-two employed individuals with SSc participated. Participants were predominantly women (82%), white (79%), and well educated (M = 16.9 years). The average age was 47.3 years, and 60.6% were married. Mean disease duration was 9.7 years, and 56.2% had diffuse SSc. Mean years on the job was 10.2 (SD ± 8.8), and 71.9% worked at least 35 hours per week. Participants engaged in a single structured interview about work-related challenges and adaptations. Content and thematic analysis was used to identify key themes across the interviews. Employees with SSc experienced Work as a daily challenge. This central theme described the general work experience for most participants. Three subthemes described their specific experiences: The work environment: Opportunities, challenges, and accommodations; Career planning; and Supportive others. The participants were anxious to find scenarios that allowed them to continue to work. Worksite accommodations and flexibility in scheduling can make the difference between working and disability.

  19. Scleroderma and the temporomandibular joint: reconstruction in 2 variants.

    Science.gov (United States)

    MacIntosh, Robert Bruce; Shivapuja, Prasanna-Kumar; Naqvi, Rabia

    2015-06-01

    This article reviews the pathophysiology of scleroderma (systemic sclerosis [SSc]) and its destructive effects on the mandible in general and the temporomandibular joint (TMJ) in particular. It discusses the considerations of operating on patients with devastating chronic disease and presents 2 cases of TMJ reconstruction in patients with the diagnosis. Two patients with different degrees of SSc involvement underwent TMJ reconstruction with costochondral grafts. The patients represent the surgical considerations pertinent to this disease and different outcomes as determined by the variance in severity of their afflictions. The 2 patients tolerated the surgeries well and exhibited improvement in function in the long-term. One patient thrives and continues to do well despite her SSc approximately 10 years postoperatively; the second patient died of her disease approximately 9 years after her initial surgical care. The experience with these 2 cases showed that patients with SSc can safely undergo TMJ reconstruction with anticipated good results, but that the overall severity of the disease remains paramount in determining the feasibility of corrective surgery under this diagnosis. Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  20. Role of radiation therapy for 'juvenile' angiofibroma

    Energy Technology Data Exchange (ETDEWEB)

    Gudea, F.; Vega, M.; Canals, E.; Montserrat, J.M.; Valdano, J. (Univ. Autonoma de Barcelona (Spain). Hospital de la Santa Creu i Sant Pau (Spain))

    1990-09-01

    Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign neoplasm which occurs primarily in male adolescents and is characterized by aggressive local growth. The controversy concerning appropriate treatment for patients with juvenile angiofibroma persists. Radiation therapy and survival resection have both been reported to be effective to control a high proportion of these tumours. The case reported here demonstrates a locally advanced JNA controlled by radiation therapy. (author).

  1. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

  2. Juvenile Rockfish Recruitment Cruise

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — In 1983, the groundfish analysis project began a series of yearly cruises designed to assess the annual abundance of juvenile rockfish along the central California...

  3. Juvenile polyposis syndrome

    OpenAIRE

    Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

    2016-01-01

    Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male...

  4. Parenting and juvenile delinquency

    OpenAIRE

    Hoeve, M.

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. The first study addressed a meta-analysis on parenting characteristics and styles in relation to delinquency. In this meta-analysis, previous manuscripts were systematically analyzed, computing mean ...

  5. local

    Directory of Open Access Journals (Sweden)

    Abílio Amiguinho

    2005-01-01

    Full Text Available The process of socio-educational territorialisation in rural contexts is the topic of this text. The theme corresponds to a challenge to address it having as main axis of discussion either the problem of social exclusion or that of local development. The reasons to locate the discussion in this last field of analysis are discussed in the first part of the text. Theoretical and political reasons are there articulated because the question is about projects whose intentions and practices call for the political both in the theoretical debate and in the choices that anticipate intervention. From research conducted for several years, I use contributions that aim at discuss and enlighten how school can be a potential locus of local development. Its identification and recognition as local institution (either because of those that work and live in it or because of those that act in the surrounding context are crucial steps to progressively constitute school as a partner for development. The promotion of the local values and roots, the reconstruction of socio-personal and local identities, the production of sociabilities and the equation and solution of shared problems were the dimensions of a socio-educative intervention, markedly globalising. This scenario, as it is argued, was also, intentionally, one of transformation and of deliberate change of school and of the administration of the educative territoires.

  6. Development and preliminary validation of the Scleroderma Support Group Leader Self-efficacy Scale

    NARCIS (Netherlands)

    Pal, N.E.; Gumuchian, S.T.; Delisle, V.C.; Pé pin, M.; Malcarne, V.L.; Carrier, M.E.; Kwakkenbos, C.M.C.; Pelá ez, S.; El-Baalbaki, G.; Thombs, B.D.

    2018-01-01

    Support groups are an important resource for people living with systemic sclerosis (SSc; scleroderma). Peer support group leaders play an important role in the success and sustainability of SSc support groups, but face challenges that include a lack of formal training. An SSc support group leader

  7. Nailfold capillaroscopy in systemic sclerosis: data from the EULAR scleroderma trials and research (EUSTAR) database.

    Science.gov (United States)

    Ingegnoli, Francesca; Ardoino, Ilaria; Boracchi, Patrizia; Cutolo, Maurizio

    2013-09-01

    The aims of this study were to obtain cross-sectional data on capillaroscopy in an international multi-center cohort of Systemic Sclerosis (SSc) and to investigate the frequency of the capillaroscopic patterns and their disease-phenotype associations. Data collected between June 2004 and October 2011 in the EULAR Scleroderma Trials and Research (EUSTAR) registry were examined. Patients' profiles based on clinical and laboratory data were obtained by cluster analysis and the association between profiles and capillaroscopy was investigated by multinomial logistic regression. 62 of the 110 EUSTAR centers entered data on capillaroscopy in the EUSTAR database. 376 of the 2754 patients (13.65%) were classified as scleroderma pattern absent, but non-specific capillary abnormalities were noted in 55.48% of the cases. Four major patients' profiles were identified characterized by a progressive severity for skin involvement, as well as an increased number of systemic manifestations. The "early" and "active" scleroderma patterns were generally observed in patients with mild/moderate skin involvement and a low number of disease manifestations, while the "late" scleroderma pattern was found more frequently in the more severe forms of the disease. These data indicate the importance of capillaroscopy in SSc management and that capillaroscopic patterns are directly related to the extent of organ involvement. Copyright © 2013 Elsevier Inc. All rights reserved.

  8. Frequency of motor alterations detected through manometry in patients with esophageal symptoms and scleroderma.

    Science.gov (United States)

    Pérez Y López, N; Lugo-Zamudio, G; Barbosa-Cobos, R E; Wong-Lam, A; Torres-López, E

    Scleroderma can present with esophageal involvement causing important morbidity. To describe the manometric findings and clinical characteristics of patients with scleroderma and esophageal symptoms. Patients with scleroderma and esophageal symptoms were evaluated through esophageal manometry within the time frame of one year. Descriptive statistics were carried out and the continuous variables were expressed as means and standard deviation. Frequencies were expressed as percentages. The study included 24 female patients with a mean age of 53.5 years and mean disease progression of 7.84 years. The most frequent findings were short and hypotonic lower esophageal sphincter (mean length 1.58cm and mean tone 9.49mmHg) and ineffective esophageal motility (mean non-transmitted waves 92.91%, mean effective primary peristalsis 40.05%, and mean amplitude 13.11mmHg). The most frequent symptom was dysphagia. Scleroderma is associated with lower esophageal sphincter alterations and symptomatic ineffective esophageal motility. Copyright © 2017 Asociación Mexicana de Gastroenterología. Publicado por Masson Doyma México S.A. All rights reserved.

  9. U3 snoRNP associates with fibrillarin a component of the scleroderma clumpy nucleolar domain

    DEFF Research Database (Denmark)

    Herrera-Esparza, Rafael; Kruse, Lars; von Essen, Marina

    2002-01-01

    by ELISA was recognized by the clumpy scleroderma serum from the majority of patients. In situ hybridization assays showed that the fibrillarin tagged by the elicited antibodies was colocalized with U3 snoRNP in the nucleolus in a clumpy manner and coprecipitated the U3 snoRNP. In conclusion...

  10. Reduced coronary flow and resistance reserve in primary scleroderma myocardial disease

    International Nuclear Information System (INIS)

    Nitenberg, A.; Foult, J.M.; Kahan, A.; Perennec, J.; Devaux, J.Y.; Menkes, C.J.; Amor, B.

    1986-01-01

    The maximum coronary vasodilator capacity after intravenous dipyridamole (0.14 mg X kg-1 X min-1 X 4 minutes) was studied in seven patients with primary scleroderma myocardial disease and compared to that of seven control subjects. Hemodynamic data and left ventricular angiographic data were not different in the two groups. The coronary flow reserve was evaluated by the dipyridamole/basal coronary sinus blood flow ratio (D/B CSBF) and the coronary resistance reserve by the dipyridamole/basal coronary resistance ratio (D/B CR). Coronary reserve was greatly impaired in the group with primary scleroderma myocardial disease: D/B CSBF was lower than in the control group (2.54 +/- 1.37 vs 4.01 +/- 0.56, respectively; p less than 0.05) and D/B CR was higher than in the control group (0.47 +/- 0.25 vs 0.23 +/- 0.04, respectively; p less than 0.05). Such a decreased coronary flow and resistance reserve in patients with primary scleroderma myocardial disease was not explained by an alteration of left ventricular function. It may be an important contributing factor in the pathogenesis of primary scleroderma myocardial disease

  11. Parapiptadenia rigida MYCORRHIZATION WITH SPORES OF Scleroderma citrinum

    Directory of Open Access Journals (Sweden)

    Gerusa Pauli Kist Steffen

    2017-06-01

    Full Text Available Ectomycorrhizal fungal inoculation in forestry seedlings aids plant establishment and growth in the field. The objectives of this study were: to determine the mycorrhizal capacity of the ectomycorrhizal fungus Scleroderma citrinum in Parapiptadenia rigida (red angico seedlings and to evaluate the viability of a mycorrhizal inoculation technique for forest seedlings involving the use of spores. Mature spores were inoculated in the substrate (75% soil and 25% carbonized rice husk, totaling 1.5 grams of fungal spores per liter of substrate. P. rigida seeds were sown in substrates inoculated or not inoculated with fungal spores in presence or absence of Pinus echinata and Eucalyptus citriodora essential oil: not inoculated (T1, inoculated (T2, inoculated more pine essential oil (T3, inoculated more eucalyptus essential oil (T4. Seedlings of Pinus elliottii were used for a positive control of mycorrhizal inoculation (T5 and not inoculated (T6 with fungal spores. At 90 days after sowing, the base stem diameter, height, fresh and dry weight of shoots and roots, percentage of root colonization and Dickson Index were determined. The presence of fungal structures in P. rigida and P. elliottii roots inoculated with S. citrinum spores was observed, demonstrating the occurrence of an ectomycorrhizal association. The application of pine and eucalyptus essential oils in the substrate increased the percentage of ectomycorrhizal colonization in P. rigida seedlings. The addition of S. citrinum mature spores in the substrate used for seedling production is a viable practice for ectomycorrhizal inoculation and it can be used in forest nurseries in controlled mycorrhization programs.

  12. Fox-2 protein regulates the alternative splicing of scleroderma-associated lysyl hydroxylase 2 messenger RNA.

    Science.gov (United States)

    Seth, Puneet; Yeowell, Heather N

    2010-04-01

    Scleroderma (systemic sclerosis [SSc]) is a complex connective tissue disorder characterized by hardening and thickening of the skin. One hallmark of scleroderma is excessive accumulation of collagen accompanied by increased levels of pyridinoline collagen crosslinks derived from hydroxylysine residues in the collagen telopeptide domains. Lysyl hydroxylase 2 (LH2), an important alternatively spliced enzyme in collagen biosynthesis, acts as a collagen telopeptide hydroxylase. Changes in the pattern of LH2 alternative splicing, favoring increased inclusion of the alternatively spliced LH2 exon 13A, thereby increasing the levels of the long transcript of LH2 (LH2[long]), are linked to scleroderma disease. This study was undertaken to examine the role played by RNA binding protein Fox-2 in regulating exon 13A inclusion, which leads to the generation of scleroderma-associated LH2(long) messenger RNA (mRNA). Phylogenetic sequence analysis of introns flanking exon 13A was performed. A tetracycline-inducible system in T-Rex 293 cells was used to induce Fox-2 protein, and endogenous LH2(long) mRNA was determined by reverse transcriptase-polymerase chain reaction. An LH2 minigene was designed, validated, and used in Fox-2 overexpression and mutagenesis experiments. Knockdown of Fox-2 was performed in mouse embryonic fibroblasts and in fibroblasts from SSc patients. Overexpression of Fox-2 enhanced the inclusion of exon 13A and increased the generation of LH2(long) mRNA, whereas knockdown of Fox-2 decreased LH2(long) transcripts. Mutational analysis of an LH2 minigene demonstrated that 2 of the 4 Fox binding motifs flanking LH2 exon 13A are required for inclusion of exon 13A. In early passage fibroblasts derived from patients with scleroderma, the knockdown of Fox-2 protein significantly decreased the endogenous levels of LH2(long) mRNA. Our findings indicate that Fox-2 plays an integral role in the regulation of LH2 splicing. Knockdown of Fox-2 and other methods to decrease

  13. "The City of Colors": a good practical experience of youth integration in the local context of a marginal neighborhood in the city of Alicante / “La Ciudad de los Colores”: una experiencia de buena práctica de integración juvenil en el marco local de un barrio marginal en la ciudad de Alicante

    Directory of Open Access Journals (Sweden)

    Virgilio Francisco Candela Sevila

    2015-10-01

    Full Text Available This article explains the birth, evolution and subsequent project management of a juvenile intervention in the Northern of the city of Alicante, born in 2007 and remains in effect within a specific Municipal Comprehensive Plan. A project managed by a local social organization nonprofit (Danae Cultural Association and is formed by a group of 80 people, including students, parents or guardians, monitors and coordinators. The program is developed with the official support of the City Council of Alicante and the Generalitat Valenciana in a neighborhood context degraded and economically disadvantaged (North Zone of the city which hosts a significant number of immigrants that in some neighborhoods, exceeds 45% and live more than 120 nationalities. It is a very socially recognized project and has been awarded by municipal and regional institutions.

  14. Pigmentation abnormalities in systemic scleroderma examined by using a colorimeter (Choromo Meter CR-200).

    Science.gov (United States)

    Maeda, M; Kachi, H; Matubara, K; Mori, S; Kitajima, Y

    1996-03-01

    Cutaneous colors of the dorsum of the hands (A), the distal forearms (B; 5 cm from the wrists), the proximal forearm (C; proximal 1/3 from the elbow) and sternal skin region (D) in patients with systemic scleroderma (73 cases; M:F = 16:57) systemic lupus erythematosus (SLE) or dermatomyositis (27 cases; M:F = 7:20) and healthy controls (HC) (36 cases; M:F = 8:28) was characterized by a XYZ colorimetric system (CIE, 1931) using a colorimeter (Choromo Meter CR-200, Minolta Camera Co. Ltd., Osaka). The index Y, which means color value shows a lower value in male HC and in patients with systemic scleroderma, especially in the more severe type with hyperpigmentation (score 5-6; the system proposed by Ishikawa) than that of female HC. The values of indices x and y, which relate to reddish (erythema with hyperpigmentation) and greenish color (pale), respectively, were higher in the exposed portion of the severe type of systemic scleroderma with hyperpigmentation, especially male and older patients, and in unexposed portions of the female group without hyperpigmentation. Histopathologically, there was prominent pigmentation in the upper dermis of the forearm in the severe type of systemic scleroderma, so that melanin quantity may be closely related to the decrease in index Y. There was no statistical significance in the value of indices Y, x and y between HC, SLE and dermatomyositis. This method may contribute not only to diagnosis of systemic scleroderma and differentiation from other collagen diseases, but also studies of clinical follow-up and effects of medication.

  15. β-thymosins and interstitial lung disease: study of a scleroderma cohort with a one-year follow-up

    Directory of Open Access Journals (Sweden)

    Messana Irene

    2011-02-01

    Full Text Available Abstract Background β-thymosins play roles in cytoskeleton rearrangement, angiogenesis, fibrosis and reparative process, thus suggesting a possible involvement in the pathogenesis of systemic sclerosis. The aim of the study was to investigate the presence of thymosins β4, β4 sulfoxide, and β10 in bronchoalveolar lavage fluid of scleroderma patients with interstitial lung disease and the relation of these factors with pulmonary functional and radiological parameters. Methods β-thymosins concentrations were determined by Reverse Phase-High Performance Liquid Chromatography-Electrospray-Mass Spectrometry in the bronchoalveolar lavage fluid of 46 scleroderma patients with lung involvement and of 15 controls. Results Thymosin β4, β4 sulfoxide, and β10 were detectable in bronchoalveolar lavage fluid of patients and controls. Thymosin β4 levels were significantly higher in scleroderma patients than in controls. In addition, analyzing the progression of scleroderma lung disease at one-year follow-up, we have found that higher thymosin β4 levels seem to have a protective role against lung tissue damage. Thymosin β4 sulfoxide levels were higher in the smokers and in the scleroderma patients with alveolitis. Conclusions We describe for the first time β-thymosins in bronchoalveolar lavage fluid and their possible involvement in the pathogenesis of scleroderma lung disease. Thymosin β4 seems to have a protective role against lung tissue damage, while its oxidation product mirrors an alveolar inflammatory status.

  16. Extending juvenility in grasses

    Energy Technology Data Exchange (ETDEWEB)

    Kaeppler, Shawn; de Leon Gatti, Natalia; Foerster, Jillian

    2017-04-11

    The present invention relates to compositions and methods for modulating the juvenile to adult developmental growth transition in plants, such as grasses (e.g. maize). In particular, the invention provides methods for enhancing agronomic properties in plants by modulating expression of GRMZM2G362718, GRMZM2G096016, or homologs thereof. Modulation of expression of one or more additional genes which affect juvenile to adult developmental growth transition such as Glossy15 or Cg1, in conjunction with such modulation of expression is also contemplated. Nucleic acid constructs for down-regulation of GRMZM2G362718 and/or GRMZM2G096016 are also contemplated, as are transgenic plants and products produced there from, that demonstrate altered, such as extended juvenile growth, and display associated phenotypes such as enhanced yield, improved digestibility, and increased disease resistance. Plants described herein may be used, for example, as improved forage or feed crops or in biofuel production.

  17. Miastenia gravis juvenil Juvenile myasthenia gravis

    OpenAIRE

    Oscar Papazian; Israel Alfonso; Nayle Araguez

    2009-01-01

    La miastenia gravis juvenil (MGJ) es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos) o sostenidos (posturas) y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nerv...

  18. Estudio de la migración y de la actividad metabólica de los polimofonucleares neutrófilos en pacientes con periodontitis juvenil localizada Migration and metabolic activity of polymorphonuclear neutrophils in patients with localized juvenile periodontitis

    Directory of Open Access Journals (Sweden)

    Pablo Javier Patiño Grajales

    1993-03-01

    -family: Arial">Localized Juvenile periodontitis (LJP is characterized by a rapid loss of connective tissue insertion and of alveolar bone around the first molars and incisors of the permanent teeth. This research was carried out in order to establish a relationship between in vitro behavior of PMN and the different clinical types of the disease. Twenty-one patients were studied; they Included incipient, moderate and advanced LJP. Before any kind of dental treatment was applied PMN were isolated from each patient to determine their chemotaxis under agarose and their metabolic activity (luminoidependent- chemiluminiscence, LDCL. A group of healthy control individuals was similarly studied.

     

    Patients with advanced LJP had significantly lower chemotaxis (p=O.O23; on the other hand, LDCL of phorbol-myristate-acetate stimulated PMN was normalin the three groups as compared with controls; PMN stimulation with opsonized zimosan revealed significantly decreased metabolic activity in patients with incipient and advanced disease (p=O.O12. These findings indicate that patients with LJP behave as a heterogeneous group regarding PMN function. In order to define PMN response in each type of this disease It Is necessary to study the relationship between genetic components, environment and clinical phenotypes of the disease.

  19. Occupational and environmental scleroderma. Systematic review and meta-analysis.

    Science.gov (United States)

    Rubio-Rivas, Manuel; Moreno, Rafael; Corbella, Xavier

    2017-03-01

    The etiology of systemic sclerosis (SSc) remains unknown; however, several occupational and environmental factors have been implicated. Our objective was to perform a meta-analysis of all studies published on SSc associated with occupational and environmental exposure. The review was undertaken by means of MEDLINE and SCOPUS from 1960 to 2014 and using the terms: "systemic," "scleroderma," or "systemic sclerosis/chemically induced" [MesH]. The Newcastle-Ottawa Scale was used for the qualifying assessment. The inverse variance-weighted method was performed. The meta-analysis of silica exposure included 15 case-control studies [overall OR 2.81 (95%CI 1.86-4.23; p < 0.001)] and 4 cohort studies [overall RR 17.52 (95%CI 5.98-51.37; p < 0.001)]; the meta-analysis of solvents exposure included 13 case-control studies (overall OR 2.00 [95%CI 1.32-3.02; p = 0.001); the meta-analysis of breast implants exposure included 4 case-control studies (overall OR 1.68 (95%CI 1.65-1.71; p < 0.001)) and 6 cohort studies (overall RR 2.13 (95%CI 0.86-5.27; p = 0.10)); the meta-analysis of epoxy resins exposure included 4 case-control studies (overall OR 2.97 (95%CI 2.31-3.83; p < 0.001)), the meta-analysis of pesticides exposure included 3 case-control studies (overall OR 1.02 (95%CI 0.78-1.32; p = 0.90)) and, finally, the meta-analysis of welding fumes exposure included 4 studies (overall OR 1.29 (95%CI 0.44-3.74; p = 0.64)). Not enough studies citing risks related to hair dyes have been published to perform an accurate meta-analysis. Silica and solvents were the two most likely substances related to the pathogenesis of SSc. While silica is involved in particular jobs, solvents are widespread and more people are at risk of having incidental contact with them.

  20. Comparing HLA shared epitopes in French Caucasian patients with scleroderma.

    Directory of Open Access Journals (Sweden)

    Doua F Azzouz

    Full Text Available Although many studies have analyzed HLA allele frequencies in several ethnic groups in patients with scleroderma (SSc, none has been done in French Caucasian patients and none has evaluated which one of the common amino acid sequences, (67FLEDR(71, shared by HLA-DRB susceptibility alleles, or (71TRAELDT(77, shared by HLA-DQB1 susceptibility alleles in SSc, was the most important to develop the disease. HLA-DRB and DQB typing was performed for a total of 468 healthy controls and 282 patients with SSc allowing FLEDR and TRAELDT analyses. Results were stratified according to patient's clinical subtypes and autoantibody status. Moreover, standardized HLA-DRß1 and DRß5 reverse transcriptase Taqman PCR assays were developed to quantify ß1 and ß5 mRNA in 20 subjects with HLA-DRB1*15 and/or DRB1*11 haplotypes. FLEDR motif is highly associated with diffuse SSc (χ(2 = 28.4, p<10-6 and with anti-topoisomerase antibody (ATA production (χ(2 = 43.9, p<10-9 whereas TRAELDT association is weaker in both subgroups (χ(2 = 7.2, p = 0.027 and χ(2 = 14.6, p = 0.0007 respectively. Moreover, FLEDR motif- association among patients with diffuse SSc remains significant only in ATA subgroup. The risk to develop ATA positive SSc is higher with double dose FLEDR than single dose with respectively, adjusted standardised residuals of 5.1 and 2.6. The increase in FLEDR motif is mostly due to the higher frequency of HLA-DRB1*11 and DRB1*15 haplotypes. Furthermore, FLEDR is always carried by the most abundantly expressed ß chain: ß1 in HLA DRB1*11 haplotypes and ß5 in HLA-DRB1*15 haplotypes.In French Caucasian patients with SSc, FLEDR is the main presenting motif influencing ATA production in dcSSc. These results open a new field of potential therapeutic applications to interact with the FLEDR peptide binding groove and prevent ATA production, a hallmark of severity in SSc.

  1. Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Kenan Barut

    2017-04-01

    Full Text Available Juvenile idiopathic arthritis is the most common chronic rheumatic disease of unknown aetiology in childhood and predominantly presents with peripheral arthritis. The disease is divided into several subgroups, according to demographic characteristics, clinical features, treatment modalities and disease prognosis. Systemic juvenile idiopathic arthritis, which is one of the most frequent disease subtypes, is characterized by recurrent fever and rash. Oligoarticular juvenile idiopathic arthritis, common among young female patients, is usually accompanied by anti-nuclear antibodie positivity and anterior uveitis. Seropositive polyarticular juvenile idiopathic arthritis, an analogue of adult rheumatoid arthritis, is seen in less than 10% of paediatric patients. Seronegative polyarticular juvenile idiopathic arthritis, an entity more specific for childhood, appears with widespread large- and small-joint involvement. Enthesitis-related arthritis is a separate disease subtype, characterized by enthesitis and asymmetric lower-extremity arthritis. This disease subtype represents the childhood form of adult spondyloarthropathies, with human leukocyte antigen-B27 positivity and uveitis but commonly without axial skeleton involvement. Juvenile psoriatic arthritis is characterized by a psoriatic rash, accompanied by arthritis, nail pitting and dactylitis. Disease complications can vary from growth retardation and osteoporosis secondary to treatment and disease activity, to life-threatening macrophage activation syndrome with multi-organ insufficiency. With the advent of new therapeutics over the past 15 years, there has been a marked improvement in juvenile idiopathic arthritis treatment and long-term outcome, without any sequelae. The treatment of juvenile idiopathic arthritis patients involves teamwork, including an experienced paediatric rheumatologist, an ophthalmologist, an orthopaedist, a paediatric psychiatrist and a physiotherapist. The primary goals

  2. DERMATOMIOSITIS JUVENIL Y EMBARAZO

    OpenAIRE

    Evans M,Gregorio; Poulsen R,Ronald; Blanco R,Romiely; Luna V,Viviana

    2002-01-01

    La dermatomiositis juvenil es un desorden inflamatorio crónico multisistémico del tejido conectivo. Tiene una incidencia de 2-3/100.000/año. Con la disminución en la mortalidad experimentada en los últimos decenios, la atención está cifrada en la morbilidad a largo plazo y en las alteraciones funcionales. Con un tratamiento agresivo los niños con dermatomiositis juvenil generalmente tienen un futuro promisorio, sin incapacidad o con incapacidad mínima. La mortalidad actualmente se estima cerc...

  3. Juvenile polyposis syndrome

    Science.gov (United States)

    Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

    2016-01-01

    Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male infant who presented with chronic anemia and gastrointestinal bleeding initially. Panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception. Technetium-99m-labeled red blood cell (RBC) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum. Histopathological examination on biopsy samples showed Peutz-Jeghers syndrome was excluded, whereas the diagnosis of juvenile polyposis syndrome was established. Results: Enteroscopic polypectomy is the mainstay of the treatment. However, polyps recurred and occupied the majority of the gastrointestinal tract in 6 months. Supportive management was given. The patient expired for severe sepsis at the age of 18 months. Conclusion: Juvenile polyposis syndrome is an inherited disease, so it is not possible to prevent it. Concerning of its poor outcome and high mortality rate, it is important that we should increase awareness and education of the parents at its earliest stages. PMID:27631205

  4. [ABOUT JUVENILE NASOPHARYNGEAL ANGIOFIBROMA].

    Science.gov (United States)

    Urbain, V; Meunier, P; Otto, B

    2015-09-01

    We report the case of a young man with a juvenile nasopharyngeal angiofibroma. In this paper, we will first remind the clinical signs of this pathology and its radiological appearance (localisation and extensions). Then we will explain how radioembolisation techniques were used to facilitate the surgical intervention. Finally we will discuss the histology of this tumor.

  5. Naevoxanthoendothelioma (Synonym: Juvenile Xanthogranuloma

    Directory of Open Access Journals (Sweden)

    F Handa

    1978-01-01

    Full Text Available A case of naevoxanthoendothelioma juvenile xanthogranuloma is reported with rare features like late onset of the disease, involvement of liver and diffuse cutaneous lesions including cafe au lait spots and pigmented naevus. Final diagnosis could be achieved only on histopathology report.

  6. Scleroderma renal crisis in a case of mixed connective tissue disease

    Directory of Open Access Journals (Sweden)

    Mukul Vij

    2014-01-01

    Full Text Available Mixed connective tissue disease (MCTD is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma and polymyositis. Scleroderma renal crisis (SRC is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

  7. Scleroderma renal crisis in a case of mixed connective tissue disease.

    Science.gov (United States)

    Vij, Mukul; Agrawal, Vinita; Jain, Manoj

    2014-07-01

    Mixed connective tissue disease (MCTD) is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma) and polymyositis. Scleroderma renal crisis (SRC) is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

  8. Netrin-1 regulates fibrocyte accumulation in the decellularized fibrotic scleroderma lung microenvironment and in bleomycin induced pulmonary fibrosis

    Science.gov (United States)

    Sun, Huanxing; Zhu, Yangyang; Pan, Hongyi; Chen, Xiaosong; Balestrini, Jenna L.; Lam, TuKiet T.; Kanyo, Jean E.; Eichmann, Anne; Gulati, Mridu; Fares, Wassim H.; Bai, Hanwen; Feghali-Bostwick, Carol A.; Gan, Ye; Peng, Xueyan; Moore, Meagan W.; White, Eric S.; Sava, Parid; Gonzalez, Anjelica L.; Cheng, Yuwei; Niklason, Laura E.; Herzog, Erica L.

    2017-01-01

    Objectives Fibrocytes are collagen-producing leukocytes that accumulate in Scleroderma-associated interstitial lung disease (SSc-ILD) via unknown mechanisms. The extracellular matrix (ECM) influences cellular phenotypes. However, a relationship between the lung ECM and fibrocytes in Scleroderma has not been explored. This study uses a novel translational platform based on decellularized human lungs to determine whether the scleroderma lung ECM controls fibrocyte development from peripheral blood mononuclear cells. Methods Decellularized scaffolds prepared from healthy and fibrotic Scleroderma lung explants underwent biomechanical evaluation using tensile testing and biochemical analysis using proteomics. Cells from healthy and SSc-ILD subjects were cultured on these scaffolds, and CD45+Pro-ColIα1+ cells meeting criteria for fibrocytes were quantified. The contribution of Netrin-1 to fibrosis was assessed using neutralizing antibodies in this system and via the inhalational administration of bleomycin to Netrin-1+/− mice. Results Compared to control lung scaffold, SSc-ILD lung scaffolds showed aberrant anatomy, enhanced stiffness, and abnormal extracellular matrix composition. Culture of control cells in Scleroderma scaffolds increased Pro-ColIα1+ production, which was stimulated by enhanced stiffness and abnormal ECM composition. SSc-ILD cells demonstrated increased Pro-ColIα1 responsiveness to Scleroderma lung scaffolds, but not enhanced stiffness. Enhanced Netrin-1 expression was seen on CD14lo SSc-ILD cells and antibody mediated Netrin-1 neutralization attenuated CD45+Pro-ColIα1+ detection in all settings. Netrin-1+/− mice were protected from bleomycin induced lung fibrosis and fibrocyte accumulation. Conclusion Factors present in Scleroderma lung matrices regulate fibrocyte accumulation via a Netrin-1-dependent pathway. Netrin-1 regulates bleomycin induced murine pulmonary fibrosis. Netrin-1 might be a novel therapeutic target in SSc-ILD. PMID:26749424

  9. Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis.

    Science.gov (United States)

    Ghossein, Cybele; Varga, John; Fenves, Andrew Z

    2016-01-01

    Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.

  10. Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

    Directory of Open Access Journals (Sweden)

    Irina Khamaganova

    2018-01-01

    Full Text Available Similar clinical and histhopathological features in progressive hemifacial atrophy and linear scleroderma en coup de sabre are well known. Trauma may predispose to the development of both diseases. The lack of association with anti-Borrelia antibodies was shown in both cases as well. The otolaryngological and endocrine disorders may be associated findings in both diseases. However, there are certain differences in neurological and ophthalmological changes in the diseases.

  11. Mechanical properties of the gastro-esophageal junction in health, achalasia, and scleroderma.

    Science.gov (United States)

    Mearin, F; Fonollosa, V; Vilardell, M; Malagelada, J R

    2000-07-01

    Manometric assessment of the gastro-esophageal junction (GEJ) is deceptive in that ignores key dynamic properties of the junction, such as resistance to flow and compliance. Our aim was to investigate the mechanical properties of the GEJ comprising intraluminal pressure (measured by manometry), resistance to flow and compliance (measured by resistometry). We studied 8 healthy subjects, 11 patients with achalasia and 11 patients with scleroderma. We used a pneumatic resistometer, previously developed and validated in our laboratory. The resistometer consists of a flaccid polyurethane 5-cm cylinder connected to an electronically regulated nitrogen-injection system; the instrument records nitrogen flow through the cylinder while maintaining a constant pressure gradient between its proximal and distal ends. By placing the cylinder successively in the proximal stomach and along the GEJ we measured the GEJ-gastric resistance gradient (GEJ resistance minus gastric resistance) and were able to calculate the cumulative resistance (sum of resistance exerted at each pressure level), peak resistance (at any injection pressure), nil resistance point (injection pressure in mmHg at which GEJ resistance equals gastric resistance), and compliance slope (flow/pressure relationship). We found that GEJ resistance to flow (cumulative resistance, peak resistance, and nil resistance point) is significantly increased in achalasia and decreased in scleroderma (P < 0.05 versus health) while GEJ compliance is diminished in achalasia (P < 0.05 versus health) and normal in scleroderma. Achalasia is a disease characterized by increased GEJ resistance and rigidity. By contrast, although scleroderma is characterized by decreased GEJ resistance, GEJ compliance may be normal.

  12. Antitopoisomerase antibody positivity predates nailfold capillaroscopy abnormalities in scleroderma. Postulated classification of 'prescleroderma'.

    Science.gov (United States)

    Englert, H; Champion, D; Wu, J C; Giallussi, J; McGrath, M; Manolios, N

    2011-02-01

    In a patient with early topoisomerase antibody-positive scleroderma, antinuclear antibody positivity was fortuitously observed to predate nailfold capillaroscopy changes. Using this case as a template, the prediagnostic phase of the presumed multifactorial disease may be divided into 5 temporal phases--phase 1 representing conception and intrauterine environment, phase 2 representing the extrauterine environment predating environmental exposure; phase 3 representing the early post-environmental exposure interval with no detectable perturbed body status; phase 4 representing the post-environmental exposure interval characterized by autoantibody production and microvascular changes, and phase 5, the symptomatic clinical prediagnostic interval (Raynaud's, skin, musculoskeletal, gastrointestinal, cardiorespiratory) prompting scleroderma diagnosis. Temporal classification of prescleroderma aids in both the understanding and definition of scleroderma 'onset'. If altered nailfold capillaries and autoantibodies develop at comparable rates, and if the findings from this case--that autoantibody changes precede microvascular changes--are truly representative of the preclinical disease phase, then these findings argue that the evolution of the disease is from within the vessel outwards, rather than vice versa. © 2011 The Authors. Internal Medicine Journal © 2011 Royal Australasian College of Physicians.

  13. Radiotherapy for malignancy in patients with scleroderma: The Mayo Clinic experience

    International Nuclear Information System (INIS)

    Gold, Douglas G.; Miller, Robert C.; Petersen, Ivy A.; Osborn, Thomas G.

    2007-01-01

    Purpose: To determine the frequency of acute and chronic adverse effects in patients with scleroderma who receive radiotherapy for treatment of cancer. Methods and Materials: Records were reviewed of 20 patients with scleroderma who received radiotherapy. Acute and chronic toxic effects attributable to radiotherapy were analyzed, and freedom from radiation-related toxicity was calculated. Results: Of the 20 patients, 15 had acute toxic effects, with Grade 3 or higher toxicity for 3 patients. Seven patients had self-limited Grade 1 or 2 radiation dermatitis, and no patient had Grade 3 or higher radiation dermatitis. Thirteen patients had chronic toxic effects, with Grade 3 or higher chronic toxicity for 3 patients. The median estimated time to any grade chronic toxicity was 0.4 years, and the median estimated time to Grade 3 or higher chronic toxicity has not been reached. Conclusions: The results suggest that although some patients with scleroderma treated with radiation experience considerable toxic effects, the occurrence of Grade 3 or higher toxicity may be less than previously anticipated

  14. Autologous fat transplantation for depressed linear scleroderma-induced facial atrophic scars.

    Science.gov (United States)

    Roh, Mi Ryung; Jung, Jin Young; Chung, Kee Yang

    2008-12-01

    Facial linear scleroderma results in depressed atrophic scars. Autologous fat transplantation has been widely used, and fat appears to be an ideal material for filling depressed atrophic scars and contour deformities, but long-term results for autologous fat transplantation are controversial. To review the short- and long-term results of 20 patients who underwent multiple autologous fat transplantations for depressed atrophic scar correction. Twenty patients with clinically inactive facial linear scleroderma were included. They received at least two transplantations and had a 12-month follow-up evaluation. On the forehead, 51% to 75% improvement (average grading scale: 2.4) was achieved when observed at least 12 months after the last treatment. For the chin, correction was poor (average grading scale: 0.7) with less than 25% improvement. The infraorbital area showed fair correction, but the nose showed poor correction. Two of three patients with scalp reduction surgery showed excellent results, showing only slight scar widening. Autologous fat transplantation is an effective method for long-term correction of depressed atrophic scars left by linear scleroderma on the forehead but is less effective for corrections on the nose, infraorbital area, and chin.

  15. Preventing Juvenile Delinquency

    Directory of Open Access Journals (Sweden)

    Carolina dos Reis

    2016-04-01

    Full Text Available This article aims to problematize discourses about protection and care that have surrounded compulsory hospitalization by evidencing its use as a control and punishment mechanism that increases the social vulnerability of young drug users. For such, we analyze lawsuits involving juveniles who were consigned to psychiatric institutions for drug addiction treatment as a protection measure in the state of Rio Grande do Sul, in Brazil. The analysis of the materials has evidenced discourses that have circumscribed young drug users and constructed this population as potentially dangerous subjects as well as a population category at risk. In this sense, we point out how compulsory hospitalization has emerged out of the lawsuits as a tool for prevention of juvenile delinquency.

  16. Late Onset Juvenile Xanthogranuloma

    Directory of Open Access Journals (Sweden)

    Punithwavathy K

    1999-01-01

    Full Text Available A 19 year old female was seen with multiple skin coloured and hyperpigmented macules, discrete as well as grouped papules and nodules of varying sizes distributed over the face, neck, extensor and flexor aspects of both upper and lower extremities including joints. The trunk was spared. Some of the lesions showed features of spontaneous regression. Investigations confirmed the diagnosis of juvenile xanthogranuloma. Lesions regressed satisfactorily with liquid nitrogen cryotherapy.

  17. Paraneoplastic scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia: a report of 10 cases.

    Science.gov (United States)

    Magro, Cynthia M; Iwenofu, Hans; Nuovo, Gerard J

    2013-07-01

    Systemic plasma cell dyscrasias have diverse manifestations in the skin and include an inflammatory paraneoplastic process. We encountered cases of scleroderma and eosinophilic fasciitis in the setting of an underlying plasma cell dyscrasia. Ten cases of scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia were encountered. The biopsies were stained for Transforming growth factor (Transforming growth factor) beta, IgG4, kappa, and lambda. Patients presented with a sclerodermoid reaction represented by eosinophilic fasciitis (5 cases), morphea (3 cases), and systemic scleroderma (2 cases). The mean age of presentation was 70 years with a striking female predominance (4:1). Acral accentuation was noted in 8 cases. In 6 of the cases, the cutaneous sclerosis antedated (4 cases) by weeks to 2 years or occurred concurrently (2 cases) with the initial diagnosis of the plasma cell. The biopsies showed changes typical of eosinophilic fasciitis and/or scleroderma. In 5 cases, light chain-restricted plasma cells were present on the biopsy. There was staining of the plasma cells for Transforming growth factor beta in 3 out of 5 cases tested. In any older patient presenting with a sudden onset of eosinophilic fasciitis or scleroderma especially with acral accentuation, investigations should be conducted in regards to an underlying plasma cell dyscrasia.

  18. Evaluation of Peripheral Blood Circulation Disorder in Scleroderma Patients Using an Optical Sensor with a Pressurization Mechanism.

    Directory of Open Access Journals (Sweden)

    Yoshiki Yamakoshi

    Full Text Available Blood circulation function of peripheral blood vessels in skin dermis was evaluated employing an optical sensor with a pressurization mechanism using the blood outflow and reflow characteristics. The device contains a light source and an optical sensor. When applied to the skin surface, it first exerts the primary pressure (higher than the systolic blood pressure, causing an outflow of blood from the dermal peripheral blood vessels. After two heartbeats, the pressure is lowered (secondary pressure and blood reflows into the peripheral blood vessels. Hemoglobin concentration, which changes during blood outflow and reflow, is derived from the received light intensity using the Beer-Lambert law. This method was evaluated in 26 healthy female volunteers and 26 female scleroderma patients. In order to evaluate the blood circulation function of the peripheral blood vessels of scleroderma patients, pressurization sequence which consists of primary pressure followed by secondary pressure was adopted. Blood reflow during the first heartbeat period after applying the secondary pressure of 40mmHg was (mean±SD 0.059±0.05%mm for scleroderma patients and 0.173±0.104%mm for healthy volunteers. Blood reflow was significantly lower in scleroderma patients than in healthy volunteers (p<0.05. This result indicates that the information necessary for assessing blood circulation disorder of peripheral blood vessels in scleroderma patients is objectively obtained by the proposed method.

  19. Metabolism of carbon-14 labelled l-tryptophan, l-kynerenine and hydroxy-l-kynerenine in miners with scleroderma

    International Nuclear Information System (INIS)

    Hankes, L.V.; De Bruin, E.; Jansen, C.R.; Voster, L.; Schmaeler, M.

    1977-01-01

    Six South African white miners were studied with the 2-g l-tryptophan load test and tracer doses of L-tryptophan-7a-carbon-14, L-kynurenine-keto-carbon-14 and hydroxy-L-kynerenine-keto-carbon-14. The breath 14 CO 2 and 14 urinary metabolites were measured. When they were compared with a previous study of American women with scleroderma, similar 14 CO 2 and tryptophan metabolite excretion patterns were observed in the data from the miners. The labelled quinolinic acid excretion was more significantly elevated in the South African miners' urine than in the urine of the American women. The data from both studies suggest that some patients with scleroderma have an altered step in the tryptophan metabolic pathway after hydroxy-anthranilic acid. What relationship exists between the induction of pulmonary silicosis and the subsequent development of scleroderma, requires additional human studies

  20. Timing of natal nests is an important factor affecting return rates of juvenile Great Reed Warblers

    Czech Academy of Sciences Publication Activity Database

    Sosnovcová, Kateřina; Koleček, Jaroslav; Požgayová, Milica; Jelínek, Václav; Šulc, Michal; Steidlová, Petra; Honza, Marcel; Procházka, Petr

    2018-01-01

    Roč. 159, č. 1 (2018), s. 183-190 ISSN 0021-8375 R&D Projects: GA ČR GA13-06451S Institutional support: RVO:68081766 Keywords : Acrocephalus arundinaceus * Juvenile condition * Juvenile survival * Local dispersal distances * Natal philopatry Subject RIV: EG - Zoology OBOR OECD: Ornithology Impact factor: 1.468, year: 2016

  1. Miastenia gravis juvenil Juvenile myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Oscar Papazian

    2009-01-01

    Full Text Available La miastenia gravis juvenil (MGJ es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos o sostenidos (posturas y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA. El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y

  2. Juvenile psittacine environmental enrichment.

    Science.gov (United States)

    Simone-Freilicher, Elisabeth; Rupley, Agnes E

    2015-05-01

    Environmental enrichment is of great import to the emotional, intellectual, and physical development of the juvenile psittacine and their success in the human home environment. Five major types of enrichment include social, occupational, physical, sensory, and nutritional. Occupational enrichment includes exercise and psychological enrichment. Physical enrichment includes the cage and accessories and the external home environment. Sensory enrichment may be visual, auditory, tactile, olfactory, or taste oriented. Nutritional enrichment includes variations in appearance, type, and frequency of diet, and treats, novelty, and foraging. Two phases of the preadult period deserve special enrichment considerations: the development of autonomy and puberty. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Juvenile Dermatomyositis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Anthony Emeka Madu

    2013-01-01

    Full Text Available Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  4. Radioangiography in diagnosis of juvenile angiofibroma

    International Nuclear Information System (INIS)

    Kalantarov, K.D.; Anyutin, R.G.; Ashikhmina, I.G.

    1979-01-01

    To study the blood supply of juvenile angiofibroma of the nasopharynx 10 patients were examined (2 of them were examined twice). Four patients with the deseases of the nose and the nasopharynx of a nonvascular nature were in the control group. The authors used sup(99m)Tc for radioangiography. The investigations were made on the gamma-chamber ''Nuclear-Chicago'' connected to the computer ''Cina-200''. Ten patients with juvenile angiofibroma of the nasopharynx showed rapid accumulation of the radioactive substance at the site of the tumour exceeding its concentration in the carotic arteries by 4-10 times. In unilateral localization of angiofibroma, its blood supply was provided mainly through the external carotic artery at the affection site or evenly through the both external carotic arteries. The data on dynamic distribution of the radioactive substance, scintiphotograms and clinical observations allow a distinct differentiation between juvenile angiofibroma of the nasopharynx the adenoid tissue or any other tumour to determine the angiofibroma size as well as to follow the results of surgical and radiation treatment

  5. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension.

    Science.gov (United States)

    Fox, Benjamin D; Shimony, Avi; Langleben, David; Hirsch, Andrew; Rudski, Lawrence; Schlesinger, Robert; Eisenberg, Mark J; Joyal, Dominique; Hudson, Marie; Boutet, Kim; Serban, Alexandrina; Masetto, Ariel; Baron, Murray

    2013-10-01

    Our study aimed to determine the prevalence of occult left-heart disease in patients with scleroderma and pulmonary hypertension. In patients with pulmonary hypertension (mean pulmonary artery pressure (mean PAP)≥25 mmHg), differentiation between pre- and post-capillary pulmonary hypertension has been made according to pulmonary artery wedge pressure (PAWP) less than or more than 15 mmHg, respectively. We performed a retrospective chart review of 107 scleroderma patients. All patients with suspected pulmonary hypertension had routine right or left heart catheterisation with left ventricular end-diastolic pressure (LVEDP) measurement pre-/post-fluid challenge. We extracted demographic, haemodynamic and echocardiographic data. Patients were classified into one of four groups: haemodynamically normal (mean PAP15 mmHg); occult PVH (mean PAP≥25 mmHg, PAWP≤15 mmHg, LVEDP>15 mmHg before or after fluid challenge); and pulmonary arterial hypertension (PAH) (mean PAP≥25 mmHg, PAWP≤15 mmHg and LVEDP≤15 mmHg before or after fluid challenge). 53 out of 107 patients had pulmonary hypertension. Based on the PAWP-based definition, 29 out of 53 had PAH and 24 out of 53 had PVH. After considering the resting and post-fluid-challenge LVEDP, 11 PAH patients were reclassified as occult PVH. The occult PVH group was haemodynamically, echocardiographically and demographically closer to the PVH group than the PAH group. PVH had high prevalence in our scleroderma-pulmonary hypertension population. Distinguishing PAH from PVH with only PAWP may result in some PVH patients being misclassified as having PAH.

  6. Scleroderma and pulmonary hypertension Esclerodermia e hipertensão pulmonar

    Directory of Open Access Journals (Sweden)

    Karen A. Fagan

    2003-10-01

    Full Text Available Patients with scleroderma are at increased risk for the development of pulmonary hypertension, and the development of unexplained dyspnea or an isolated decrease in diffusing capacity should prompt evaluation. Echocardiography is often helpful in this situation, with further testing being performed as indicated. Because the prognosis of untreated pulmonary hypertension occurring in the setting of scleroderma is generally quite poor, vigilance is required on the part of physicians following this "at risk" group of patients. The past decade has seen important advances in the treatment of pulmonary arterial hypertension, including intravenous epoprostenol, oral bosentan and subcutaneously infused treprostinil. As new therapies are developed for the treatment of pulmonary arterial hypertension, it is essential that patients with scleroderma-related disease are included in clinical trials.Pacientes com esclerodermia têm risco aumentado para desenvolver hipertensão pulmonar. O aparecimento de dispnéia e/ou a diminuição da capacidade de difusão devem levar à suspeita imediata dessa complicação. A ecodopplercardiografia é importante para o diagnóstico e o seguimento desses casos. Os casos não tratados de hipertensão pulmonar em esclerodermia têm mau prognóstico, daí a necessidade em manter sob vigilância estes pacientes. Na última década surgiram avanços para o tratamento da hipertensão arterial pulmonar, incluindo os medicamentos epoprostenol EV, bosentan VO e treprostinil SC. À medida que novas terapias vão sendo desenvolvidas, torna-se necessário a realização de estudos clínicos de maior validade.

  7. Scleroderma "en coup de sabre" With Epilepsy and Uveitis Successfully Treated With Tocilizumab.

    Science.gov (United States)

    Osminina, Maria; Geppe, Nathalia; Afonina, Elena

    2018-06-18

    Case history of a small girl outlet with epilepsy, followed by scleroderma skin damage and uveitis, neurovasculitis with white matter foci in brain on the side of skin lesion in two months, immunologic disease activity. Resistance to conventional immunosuppressive therapy forced us to initiate the treatment with tocilizumab. It was well tolerated and led to significant improvement of brain, ocular and skin manifestations. Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  8. The preliminary evaluation on sclerodermas of newborn baby with thyroid hormone and β2-MG ria

    International Nuclear Information System (INIS)

    Liu Junchi; Du Sujun

    1995-01-01

    The combined determination of thyroid hormone and β 2 -MG may be used as the sensitive indication of the early degeneration of liver and kidney function. It is probably due to the damage of liver and kidney by scleroderma in newborn baby. The observation of 68 cases show that the serum levels of T 3 ,FT 3 in the concentration of thyroid hormone decreased significantly, and syndrome of the low serum T 3 , high serum rT 3 are taken place. Whereas, the serum level of β 2 -MG is increased significantly

  9. Spinal subarachnoid hemorrhage caused by scleroderma-induced aneurysm: a case report

    International Nuclear Information System (INIS)

    Mueller, J.; Neidl, K.; Contier-Dippel, B.; Huber, G.; Ernst, E.

    1995-01-01

    We introduce a 58-year-old woman who suffered from progressive systemic scleroderma (PSS) associated with trigeminal sensory neuropathy for approximately 10 years. She then had a stroke from spinal subarachnoid hemorrhage (SSAH). Spinal digital subtraction angiography (DSA) revealed two aneurysms and smaller dilations of the afferent vessel that could also be seen by MRI. Three asymptomatic brain infarctions in different vascular regions could be revealed by CCT. The SSAH, ischemic lesions and aneurysms were probably caused by vasculitic affections induced by PSS. (orig.)

  10. Spinal subarachnoid hemorrhage caused by scleroderma-induced aneurysm: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, J. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Neidl, K. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Contier-Dippel, B. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Huber, G. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Ernst, E. [Neurologische Abt., Caritas Krankenhaus, Dillingen (Germany)

    1995-11-01

    We introduce a 58-year-old woman who suffered from progressive systemic scleroderma (PSS) associated with trigeminal sensory neuropathy for approximately 10 years. She then had a stroke from spinal subarachnoid hemorrhage (SSAH). Spinal digital subtraction angiography (DSA) revealed two aneurysms and smaller dilations of the afferent vessel that could also be seen by MRI. Three asymptomatic brain infarctions in different vascular regions could be revealed by CCT. The SSAH, ischemic lesions and aneurysms were probably caused by vasculitic affections induced by PSS. (orig.)

  11. Juvenile hyperthyroidism: an experience.

    Science.gov (United States)

    Bhadada, S; Bhansali, A; Velayutham, P; Masoodi, S R

    2006-04-01

    To analyze the clinical profile of juvenile hyperthyroidism at presentation, their treatment outcome; predictors of remission and relapse. Retrospective analysis of medical records of 56 patients with juvenile hyperthyroidism seen over a period of 16 years. A cohort of 38 females and 18 males with mean (+/-SD) age of 14.9 +/- 3.4 years (range 3 to 18 years) was analyzed. Majority of patients was in the age group of 12-16 years. Common symptoms observed at presentation were weight loss (82.1%), excessive sweating (78.6%), heat intolerance (76.8%), increased appetite (73.2%) and diarrhea in 48.2%. In addition, accelerated linear growth was observed in 7.1% of patients. Goiter was present in 98.2% of children; 94.5% of which was diffuse and 4.8% was multinodular. The mean ((+/-SD) T3 was 4.8 +/- 3.4 ng/mL (N, 0.6-1.6), T4 was 218 +/- 98 ng/mL (N, 60-155) and TSH was 0.44 +/- 0.36 (N, 0.5-5.5 microIU/mL). TMA positivity seen in 36.9% of patients. All patients were treated with carbimazole; subsequently 4 patients required thyroidectomy and one required radioactive iodine ablation. Mean (+/-SD) duration of follow-up in our patients was 4.9 +/- 3 years, ranging between 1.6 to 16 years and mean (+/-SD) duration of treatment was 34.4 +/- 22.6 months (range 12 to 120 months). Mean (+/-SD) duration to achieve euthyroidism was 5.2 +/- 4.7 months, ranging between 1-33 months. On intention to treat analysis, remission with carbimazole was achieved in 47.6%, remaining patients failed to achieve remission with drug treatment. Graves disease is the commonest cause of juvenile hyperthyroidism. Carbimazole is safe, effective, cheap, and easily available form of therapy. It is occasionally associated with serious side effects but requires prolonged follow up.

  12. Juvenile prison in parallel legislation

    Directory of Open Access Journals (Sweden)

    Lutovac Mitar

    2016-01-01

    Full Text Available The need for punishment of juveniles occurred from the time when there was no clear line separating them from the adult criminal population. At the same time, the evolution of the juvenile punishment is not in itself involve substantial changes to their criminal status. On the contrary, the status of minors in society did not show serious differences regarding the status of young adults, as well as the adult elderly. On the other hand, on the ground of their punishment is recorded deviations that go in the direction of application of mild corporal punishment. Closing the minor was performed in a physically separate parts of the general penal institutions with the use of a lower degree of restrictions while serving juvenile prison. Due to the different treatment of minors during the evolution of their criminal status leads to their different treatment in comparative law. That is why we are witnessing the existence of numerous differences in the juvenile punishment in some countries in the world. On the European continent there is a wide range of different legal solutions when it comes to punishing juveniles. There are considerable differences in the procedure pronouncing juvenile prison and in particular penal treatment of juveniles in penitentiary institutions. For these reasons, the author has decided to show the basic statutory provisions in the part that relates to the issue of punishment of minors in the legislation of individual countries.

  13. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

    NARCIS (Netherlands)

    Kowal-Bielecka, O.; Landewé, R.; Avouac, J.; Chwiesko, S.; Miniati, I.; Czirjak, L.; Clements, P.; Denton, C.; Farge, D.; Fligelstone, K.; Földvari, I.; Furst, D. E.; Müller-Ladner, U.; Seibold, J.; Silver, R. M.; Takehara, K.; Toth, B. Garay; Tyndall, A.; Valentini, G.; van den Hoogen, F.; Wigley, F.; Zulian, F.; Matucci-Cerinic, Marco

    2009-01-01

    The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group

  14. Inhibition of collagen production in scleroderma fibroblast cultures by a connective tissue glycoprotein extracted from normal dermis

    International Nuclear Information System (INIS)

    Maquart, F.X.; Bellon, G.; Cornillet-Stoupy, J.; Randoux, A.; Triller, R.; Kalis, B.; Borel, J.P.

    1985-01-01

    It was shown in a previous paper that a connective tissue glycoprotein (CTGP) extracted from normal rabbit dermis was able to inhibit total protein and collagen syntheses by normal dermis fibroblast cultures. In the present study, the effects of CTGP on scleroderma fibroblasts were investigated. [ 14 C]Proline incorporation into total proteins of the supernatant was not significantly different from that found in controls. By contrast, the amount of collagen, expressed as percentage of total secreted protein, was far higher in scleroderma cultures than in normal ones (14.4% +/- 6.0% vs 4.6% +/- 0.9%). Addition of CTGP to the medium induced a concentration-dependent inhibition of [ 14 C]proline incorporation into proteins from both control and scleroderma cells. In control cultures, no significant decrease of the percentage of collagen was observed, but over 60 micrograms/ml, both cytotoxic effects and inhibition of protein synthesis occurred. In scleroderma cultures, the inhibition was twice as effective on collagen as on noncollagen protein synthesis. The inhibition of collagen secretion was not related either to changes in collagen hydroxylation or to the intracellular catabolism of newly synthesized procollagen

  15. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study

    DEFF Research Database (Denmark)

    Peytrignet, Sébastien; Denton, Christopher P; Lunt, Mark

    2018-01-01

    Objectives: Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods: Patients completed questionnaires at study entry, 12 and 24 months, including the HA...

  16. Validation of the Self-Efficacy for Managing Chronic Disease Scale: A Scleroderma Patient-Centered Intervention Network cohort study

    NARCIS (Netherlands)

    Riehm, K.E.; Kwakkenbos, C.M.C.; Carrier, M.E.; Bartlett, S.J.; Malcarne, V.L.; Mouthon, L.; Nielson, W.R.; Poiraudeau, S.; Nielsen, K.; Baron, M.; Frech, T.; Hudson, M.; Pope, J.; Sauvé, M.; Suarez-Almazor, M.E.; Wigley, F.M.; Thombs, B.D.

    2016-01-01

    Objective: Self-management programs for patients with chronic illnesses, including rheumatic diseases, seek to enhance self-efficacy for performing health management behaviors. No measure of self-efficacy has been validated for patients with systemic sclerosis (SSc; scleroderma). The objective of

  17. Characteristic of x-ray picture of urolithiasis in children and juveniles

    International Nuclear Information System (INIS)

    Kramnij, Yi.O.; Bortnij, M.O.; Voron'zhev, Yi.O.; Ul'yanchenko, Yi.M.

    1999-01-01

    More exactly the characteristics of urolithiasis x-ray picture in children and juveniles was defined. X-ray study of 42 patients. Mainly x-ray positive calculi localized in the pelvis and ureters occur in children patients

  18. Recurrent giant juvenile fibroadenoma

    Directory of Open Access Journals (Sweden)

    Kathryn S. King

    2017-11-01

    Full Text Available Breast masses in children, though rare, present a difficult clinical challenge as they can represent a wide variety of entities from benign fibroadenomas to phyllodes tumors. Rapidly growing or recurrent masses can be particularly concerning to patients, families and physicians alike. Clinical examination and conventional imaging modalities are not efficacious in distinguishing between different tumor types and surgical excision is often recommended for both final diagnosis and for treatment of large or rapidly growing masses. While surgical excision can result in significant long-term deformity of the breast there are some surgical techniques that can be used to limit deformity and/or aid in future reconstruction. Here we present a case of recurrent giant juvenile fibroadenoma with a review of the clinical presentation, diagnostic tools and treatment options.

  19. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Anon.

    1981-01-01

    A number of studies conducted under this project have demonstrated that many of the biological parameters used to calculate permissible levels of exposure of adults to radioactive materials are inappropriate for the rapidly growing infant or child or for the pregnant female. These include age-related differences in radionuclide deposition, distribution, and retention and associated differences in microdosimetry, as well as the greater intrinsic radiosensitivity of the immature organism. These findings emphasize the need for more detailed information on the metabolism and toxicity of radionuclides in the prenatal and juvenile mammal. The continuing objective of this project is to obtain such information, which is needed to establish appropriate exposure limits for radionuclides of greatest potential hazard to these age groups

  20. JUVENILE DELINQUENCY: TRENDS (REGIONAL ASPECT

    Directory of Open Access Journals (Sweden)

    I. G. Selezneva

    2017-01-01

    Full Text Available he article analyzes the nature and internal structure of various types of crimes in which involved minors. Describes the main social factors contributing to this anomaly in the period of development of society. Investigated the motivation, the system and the types of crimes of minors in the Volgograd region, are the main trends of development of this phenomenon. The study also discusses the theoretical basis of the problem of the influence of economic stability on the species structure of juvenile delinquency. In this study the analysis of various types of deviance minors in different areas of the city of Volgograd. In the process of rapid modernization of communication processes most of today’s youth have not been able to quickly rebuild their behavior. Currently, the value-perception of the adolescents focused on the material benefits in terms of expanded economic interactions. In these conditions, social processes become increasingly removed from humane and spiritual orientations. The effective functioning of society in its interaction based on cooperation and understanding is of great importance to stimulate the positive trends in social sphere in modern Russia. The modern period of development, coupled with a drastic breaking of the foundations of life, the formation of new social relations and institutions and the destruction of the old, inevitably contributes to social tension, the reassessment of social and moral values and development of deviant behavior of minors. The advantages of this study are the involvement of local archives regional committees on Affairs of minors, was first introduced to active scientific revolution, as well as logical structuring and grouping of the main issues related to the dynamics and changes in the species structure of juvenile crime, which allowed us to perform a fairly extensive archive of statistical material. Based on this analysis, the authors made a

  1. Juvenile delinquency and correctional treatment in Britain

    OpenAIRE

    堀尾, 良弘; ホリオ, ヨシヒロ; Yoshihiro, Horio

    2006-01-01

    Japanese modernistic culture is influenced not a little from Britain. In looking at the Juvenile Law and the history of correctional treatment in Britain, understanding of today's juvenile delinquency and treatment deepen. Moreover, the background and issue of juvenile delinquency in Britain are also discussed. As a feature of the juvenile delinquency in Britain, the common field with Japan and the field peculiar to Britain became clear in each. It is common to the world that the juvenile del...

  2. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma)

    International Nuclear Information System (INIS)

    Tagliafico, Alberto; Panico, Nicoletta; Resmini, Eugenia; Derchi, Lorenzo E.; Ghio, Massimo; Martinoli, Carlo

    2011-01-01

    Background: Patients affected by scleroderma may complain of sensory disturbances especially in the hands. Purpose: To study the imaging features of upper limb nerves in patients affected by scleroderma (SSc). Materials and method: Twenty-five patients affected only by SSc were prospectively evaluated with high-resolution US and magnetic resonance (MRI) or computer tomography (CT) when necessary (2 patients). Median and ulnar nerves were evaluated bilaterally. Nerve conduction studies were performed in the symptomatic patients (n = 10). Results of imaging studies were correlated with disease duration, autoimmunity and immunosuppression. Nerves of SSc patients were compared with a control group of 90 patients matched for age and body mass index. Results: The prevalence of sensory disturbances revealed by clinical examination was 40%. In symptomatic SSc patients (n = 10) US evaluation revealed nerve abnormalities in 70% of cases (n = 7/10). n = 2 had a carpal tunnel syndrome. n = 5 had cubital tunnel syndrome. In two of them CT and MR were necessary to identify the compressed nerve at the level of the elbow due to the presence of calcifications. There was no association between the presence of an entrapment neuropathy and disease duration, autoantibodies and immunosuppression. Conclusion: Ultrasound, CT and MR may detect nerve abnormalities in 70% of SSc patients complaining of neurologic disturbances in the hands. The results of imaging studies support the hypothesis of a vascular dependent neuropathy in SSc.

  3. Esophageal function scintigraphy as parameter for organ involvement of progressive systemic scleroderma

    International Nuclear Information System (INIS)

    Leisner, B.; Koenig, G.; Hundegger, K.; Luderschmidt, C.

    1986-01-01

    Involvement of internal organs such as lungs, heart and kidneys, is a life-threatening complication in progressive systemic scleroderma (PSS). However, the earliest and the most frequent internal manifestation of PSS is that of esophageal dysfunction. This study was undertaken to determine whether the new, sensitive and noninvasive esphageal function scintigraphy (EFS) enables us to identify patients likely to develop pulmonary interstitial fibrosis. 131 patients with PSS of different clinical types and courses underwent EFS. The esophageal clearance of a sup(99m)Tc-tagged 15 ml water bolus was measured. In normals, 91 +- 4.8% of the maximal rate were cleared from the ROI comprising the whole esophagus 10 sec after T max was reached. For comparison, chest X-rays and pulmonary function data were used. In the presence of interstitial lung disease EFS gave normal results in four patients only (sensitivity, 89%). All cases with either severely impaired lung function or signs of fibrosis on X-ray films showed esophageal clearance values below 40%. Moreover, esophageal motility disorders were seen in 32 among 43 patients with normal lung function. There was a correlationi between the stage and progression (reflected by unspecific signs of inflammation) and the incidence and severity of both lung function impairment and esophageal dysfunction. In conclusion, functional scintigraphy proved to be a very sensitive diagnostic procedure in the screening for internal manifestations of progressive systemic scleroderma. (Author)

  4. Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma

    International Nuclear Information System (INIS)

    Follansbee, W.P.; Curtiss, E.I.; Medsger, T.A. Jr.; Steen, V.D.; Uretsky, B.F.; Owens, G.R.; Rodnan, G.P.

    1984-01-01

    To investigate cardiopulmonary function in progressive systemic sclerosis with diffuse scleroderma, we studied 26 patients with maximal exercise and redistribution thallium scans, rest and exercise radionuclide ventriculography, pulmonary-function testing, and chest roentgenography. Although only 6 patients had clinical evidence of cardiac involvement, 20 had abnormal thallium scans, including 10 with reversible exercise-induced defects and 18 with fixed defects (8 had both). Seven of the 10 patients who had exercise-induced defects and underwent cardiac catheterization had normal coronary angiograms. Mean resting left ventricular ejection fraction and mean resting right ventricular ejection fraction were lower in patients with post-exercise left ventricular thallium defect scores above the median (59 +/- 13 per cent vs. 69 +/- 6 per cent, and 36 +/- 12 per cent vs. 47 +/- 7 per cent, respectively). The authors conclude that in progressive systemic sclerosis with diffuse scleroderma, abnormalities of myocardial perfusion are common and appear to be due to a disturbance of the myocardial microcirculation. Both right and left ventricular dysfunction appear to be related to this circulatory disturbance, suggesting ischemically mediated injury

  5. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma)

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto, E-mail: atagliafico@sirm.org [Department of Radiology, University of Genova, Genova (Italy); Panico, Nicoletta [Division of Immunology, Department of Internal Medicine, University of Genoa, Genoa (Italy); Resmini, Eugenia [Department of Endocrinological and Medical Sciences (DiSEM), Center of Excellence for Biomedical Research, University of Genova, Genova (Italy); Derchi, Lorenzo E. [Department of Radiology, University of Genova, Genova (Italy); Ghio, Massimo [Division of Immunology, Department of Internal Medicine, University of Genoa, Genoa (Italy); Martinoli, Carlo [Department of Radiology, University of Genova, Genova (Italy)

    2011-03-15

    Background: Patients affected by scleroderma may complain of sensory disturbances especially in the hands. Purpose: To study the imaging features of upper limb nerves in patients affected by scleroderma (SSc). Materials and method: Twenty-five patients affected only by SSc were prospectively evaluated with high-resolution US and magnetic resonance (MRI) or computer tomography (CT) when necessary (2 patients). Median and ulnar nerves were evaluated bilaterally. Nerve conduction studies were performed in the symptomatic patients (n = 10). Results of imaging studies were correlated with disease duration, autoimmunity and immunosuppression. Nerves of SSc patients were compared with a control group of 90 patients matched for age and body mass index. Results: The prevalence of sensory disturbances revealed by clinical examination was 40%. In symptomatic SSc patients (n = 10) US evaluation revealed nerve abnormalities in 70% of cases (n = 7/10). n = 2 had a carpal tunnel syndrome. n = 5 had cubital tunnel syndrome. In two of them CT and MR were necessary to identify the compressed nerve at the level of the elbow due to the presence of calcifications. There was no association between the presence of an entrapment neuropathy and disease duration, autoantibodies and immunosuppression. Conclusion: Ultrasound, CT and MR may detect nerve abnormalities in 70% of SSc patients complaining of neurologic disturbances in the hands. The results of imaging studies support the hypothesis of a vascular dependent neuropathy in SSc.

  6. Group Work with Juvenile Delinquents.

    Science.gov (United States)

    Zimpfer, David G.

    1992-01-01

    Reviews group work literature on juvenile delinquents. Presents overview of interventions, including positive peer culture, cognitive-behavioral treatment, psychoeducational treatment, treatment of learned behavior, action-oriented treatment, milieu therapy, parental involvement, assertiveness training, and music therapy. Discusses outcome…

  7. Juvenile Angiofibroma: Evolution of Management

    Science.gov (United States)

    Nicolai, Piero; Schreiber, Alberto; Bolzoni Villaret, Andrea

    2012-01-01

    Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment. PMID:22164185

  8. Juvenile Angiofibroma: Evolution of Management

    Directory of Open Access Journals (Sweden)

    Piero Nicolai

    2012-01-01

    Full Text Available Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment.

  9. Bilateral, independent juvenile nasopharyngeal angiofibroma

    DEFF Research Database (Denmark)

    Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T

    2015-01-01

    BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating...... from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning...... embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. CONCLUSION: This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral...

  10. Nasal juvenile angiofibroma: Current perspectives with emphasis on management.

    Science.gov (United States)

    López, Fernando; Triantafyllou, Asterios; Snyderman, Carl H; Hunt, Jennifer L; Suárez, Carlos; Lund, Valerie J; Strojan, Primož; Saba, Nabil F; Nixon, Iain J; Devaney, Kenneth O; Alobid, Isam; Bernal-Sprekelsen, Manuel; Hanna, Ehab Y; Rinaldo, Alessandra; Ferlito, Alfio

    2017-05-01

    Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor. It is found almost exclusively in young men. Common presenting symptoms include nasal obstruction and epistaxis. More advanced tumors may present with facial swelling and visual or neurological disturbances. The evaluation of patients with juvenile angiofibroma relies on diagnostic imaging. Preoperative biopsy is not recommended. The mainstay of treatment is resection combined with preoperative embolization. Endoscopic surgery is the approach of choice in early stages, whereas, in advanced stages, open or endoscopic approaches are feasible in expert hands. Postoperative radiotherapy (RT) or stereotactic radiosurgery seem valuable in long-term control of juvenile angiofibroma, particularly those that extend to anatomically critical areas unsuitable for complete resection. Chemotherapy and hormone therapy are ineffective. The purpose of the present review was to update current aspects of knowledge related to this rare and challenging disease. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1033-1045, 2017. © 2017 Wiley Periodicals, Inc.

  11. Juvenile psammomatoid ossifying fibroma: An unusual case report

    Directory of Open Access Journals (Sweden)

    Nidhi Yadav

    2013-01-01

    Full Text Available Ossifying fibromas are well-demarcated benign fibro-osseous tumors of craniofacial skeleton most often in the jaws. It has two types, conventional and juvenile ossifying fibroma (JOF. JOF is considered a separate entity from ossifying fibroma due to its locally aggressive behavior and tendency to occur at a young age. Two subtypes of JOF, i.e., juvenile psammomatoid ossifying fibroma (JPOF and juvenile trabecular ossifying fibroma have been identified on the basis of histology 70% of the JPOF occur in the paranasal sinuses, 20% in the maxilla and only 10% in the mandible. Here, we report a case of JPOF in a 14-year-old girl causing an asymptomatic expansile swelling in the right mandibular posterior region, which is a rare site for this tumor.

  12. Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma

    International Nuclear Information System (INIS)

    Follansbee, W.P.; Curtiss, E.I.; Medsger, T.A. Jr.; Owens, G.R.; Steen, V.D.; Rodnan, G.P.

    1984-01-01

    Myocardial function and perfusion were evaluated in 22 patients with progressive systemic sclerosis with the CREST syndrome using exercise and radionuclide techniques, pulmonary function testing, and chest roentgenography. The results were compared with a similar study of 26 patients with progressive systemic sclerosis with diffuse scleroderma. The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01). Reperfusion thallium defects in the absence of extramural coronary artery disease were seen in 38 percent of patients with diffuse scleroderma. This finding was not seen in any of the patients with the CREST syndrome. In diffuse scleroderma, abnormalities of both right and left ventricular function were related to larger thallium perfusion defects. In the CREST syndrome, abnormalities of left ventricular function were minor, were seen only during exercise, and were unrelated to thallium perfusion defects. Abnormal resting right ventricular function was seen in 36 percent of the patients with the CREST syndrome and was associated with an isolated decrease in diffusing capacity of carbon monoxide. It is concluded that the cardiac manifestations of the CREST syndrome are distinct from those found in diffuse scleroderma. Unlike diffuse scleroderma, abnormalities of left ventricular function in the CREST syndrome are minor and are unrelated to abnormalities of coronary perfusion. Right ventricular dysfunction in the CREST syndrome appears to be primarily related to pulmonary vascular disease

  13. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Sikov, M.R.

    1985-01-01

    This project is directed at obtaining detailed comparative information on the deposition, distribution, retention, and toxicity of radionuclides in the prenatal and juvenile mammal. Because quantitative data cannot necessarily be extrapolated to man, emphasis is also directed toward establishing patterns, phenomenologic interactions, and relationships which will be useful in determining appropriate exposure levels for rapidly growing infants or children and for pregnant women. Further dosimetry for an experiment to evaluate the effects of foster-rearing of newborn rats on the lifetime effects of 239 Pu exposure has demonstrated that most of the lifetime burden is derived from prenatal exposure and that milk contributes little in addition. Other measurements have confirmed a tentative observation that the lifetime burden in offspring is greater with near-term exposure than with exposure earlier in gestation. Additional results from a comparison of the embryotoxicity of 239 Pu and 241 Am have confirmed that, on the basis of dose administered to the dam, the former has a greater effect on the conceptus. Pilot studies indicate that 233 U is teratogenic, acting as a chemical rather than as a radiological teratogen. Studies with 239 Pu-exposed pregnant rabbits have shown that maternal distribution differs from that in rodents; concentration patterns in the placenta and membranes also differed. 4 figures, 1 table

  14. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Sikov, M.R.

    1982-01-01

    This project is directed at obtaining detailed comparative information on the deposition, distribution, retention, and toxicity of radionuclides in the prenatal and juvenile mammal. Because quantitative data cannot necessarily be extrapolated to man, our emphasis is directed toward establishing patterns, phenomenologic interactions, and relationships which will be useful in determining appropriate exposure levels for the rapidly growing infant or child, and for pregnant women. Recent results demonstrated that injection of pregnant rats with 23 Pu had the greatest effect on longevity and bone-tumor incidence of the offspring when exposure occurred at 19 days of gestation (dg); less effect at 15 dg and the least effect at 9 dg. Ongoing distribution studies are providing data which confirm our tentative explanation that marked variations in the anatomic distributions of bone tumors, with age at the time of injection, were attributable to age-related differences in 239 Pu microdosimetry and concentrations among skeletal components. Other studies, using a placental perfusion technique, have demonstrated that intravenous injection of 239 Pu in pregnant guinea pigs leads to a marked decrease in maternal blood flow to the placenta

  15. JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    I N Sartika

    2012-11-01

    Full Text Available Juvenile rheumatoid arthritis (JRA is the most common rheumatic condition in children. JRA is defined as persistent arthritis in 1 or more joints for at least 6 weeks, with the onset before age 16 years. The etiology of JRA is unknown. Antigen activated CD4+ T cell stimulate monocytes, macrophages, and synovial fibroblasts to produce the cytokines Interleukin-1 (IL-1, IL-6, and tumor necrosis factor ? (TNF-? and to secrete matrix metalloproteinases, which lead to chronic inflammation due to infiltration of inflammatory cell, angiogenesis, destruction of cartilage and bone with pannus formation. The 3 major subtypes of JRA are based on the symptoms at disease onset and are designated systemic onset, pauciarticular onset, and polyarticular onset. For all patients, the goals of therapy are to decrease chronic joint pain and suppress the inflammatory process. Poor prognostic have been observed in patients with polyarticular onset, rheumatoid factor, persistent morning stiffness, tenosynovitis, involvement of the small joints, rapid appearance of erosions, active late onset childhood, subcutaneous nodules, or antinuclear antibody.

  16. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Sikov, M.R.

    1983-01-01

    Comparative information on the deposition, distribution, retention, and toxicity of radionuclides in the prenatal and juvenile mammal is reported. Emphasis is toward establishing patterns, phenomenologic interactions, and relationships which will be useful in determining appropriate exposure levels for the rapidly growing infant or child and for pregnant women. Recent results have shown that injection of pregnant rats with 239 Pu increases the incidence and severity of adenomatous hyperplasia of the liver in the offspring; the magnitude of these effects is relatd to dose and prenatal age at exposure. Analysis of combined data from several experiments leads to the conclusion that perinatal rats are more sensitive to bone tumor induction by 239 Pu alpha-particle irradiation than are adults. Further histopathologic evaluations of material from earlier experiments have demonstrated that most of the increased incidence of thyroid tumors following 131 I exposure is attributable to follicular tumors. An analysis of the literature led to the conclusion that prenatal irradiation can lead to an increased or decreased incidence of tumors, depending on the specific details of the experimental design and system

  17. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Sikov, M.R.

    1984-01-01

    This project is directed at obtaining detailed comparative information on the deposition, distribution, retention, and toxicity of radionuclides in the prenatal and juvenile mammal. Because quantitative data cannot necessarily be extrapolated to man, emphasis is also directed toward establishing patterns, phenomenologic interactions, and relationships which will be useful in determining appropriate exposure levels for the rapidly growing infant or child and for pregnant women. An experiment to evaluate the effects of foster-rearing of newborn rats on the lifetime effects of 239 Pu exposure has demonstrated that, while longevity is primarily dependent on radiation history, growth rate and adult body weight are related to the exposure and fitness of the foster dam. Results from an ongoing comparison of the dosimetry and embryotoxicity of 239 Pu and 241 Am confirm that the former has a greater effect on the conceptus, on the basis of dose administered to the dam. Studies in the guinea-pig perfusion system have confirmed that maternal blood flow to the placenta is decreased by intravenous doses of 30 nCi/g 239 Pu and suggest that the threshold lies at approximately 5 nCi/g body weight. A dose of 30 nCi/g of 241 Am does not affect blood flow. Clearance of the two actinides is similar when blood flow effects are not considered. 3 figures, 3 tables

  18. PSYCHOSOCIAL PROFILE OF JUVENILE DIABETES

    Science.gov (United States)

    Dass, Jyoti; Dhavale, H.S.; Rathi, Anup

    1999-01-01

    A study of the complex relationships between the patient characteristics, family and environmental influences, physician's behaviour and the demands of the disease with its management in Juvenile Diabetics was taken up at a general hospital. 90 subjects were selected for the study and grouped into three. Group A consisted of 30 Juvenile Diabetics, Group B of 30 Adult Diabetics and Group C of 30 Normal healthy adolescents. The impact of the illness was measured on the Diabetes Impact Measurement Scale (DIMS), the behavioural deviations and the parental attitudes towards child rearing on the Fallstrom's Questionnaire (FQ) and the family environment on the Family Climate Scale (FCS). Psychiatric morbidity was assessed using DSM-IV criteria. Group A & B were compared on the DIMS and Group A & C on FQ & FCS. Adult diabetics had a greater impact of diabetes. Juvenile diabetics had significantly higher frequency of behavioural deviations as compared to controls. Also there was a higher number of responses on questions indicating an overprotecting attitude amongst parents of juvenile diabetics. There was an increased incidence of psychiatric morbidity in juvenile diabetics as compared to normal adolescents irrespective of the family environment. The results are discussed in relation to current literature. PMID:21430802

  19. Juvenile rheumatoid arthritis

    International Nuclear Information System (INIS)

    Naz, S.; Mushtaq, A.; Bari, A.; Maqsud, A.; Khan, M. Z.; Ahmad, T. M.; Saira Rehman

    2013-01-01

    Objective: To determine the spectrum of clinical presentation, laboratory parameters and drug therapy in patients with Juvenile Rheumatoid Arthritis (JRA). Study Design: Case series. Place and Duration of Study: The Children's Hospital and The Institute of Child Health, Lahore, from October 2008 to October 2011. Methodology: All patients who fulfilled the American College of Rheumatology criteria for JRA were enrolled. Their clinical features, investigations done and treatment received for JRA were noted. Statistical analysis of data was done on SPSS version 16.0 for obtaining descriptive statistics. Results: Out of 185 patients, 50.3% (n = 93) were females; 54% (n = 100) were between 10 - 15 years of age. Polyarthritis was found in 71.9% (n = 133) followed by oligoarthritis (22.7%, n = 42) and systemic onset disease (5.4%, n = 10). Morning stiffness (78%) and fever (68%) were the most common clinical presentations. All patients with systemic onset disease had fever (n = 10) followed by skin rash, hepatosplenomegaly and lymphadenopathy. Uveitis was found in 2 patients, and both belonged to the oligoarticular group. Rheumatoid factor was found in 10.27% (n = 19) of all patients. All patients were given non-steroidal anti-inflammatory drugs (NSAIDs). Disease modifying agents (methotrexate) were given to 43.8% (n = 81). Steroids were used in 61% (n = 113) of patients either with NSAIDs alone or NSAIDs plus methotrexate. Conclusion: Disease profile of JRA at the study centre showed that polyarthritis is the commonest type. Recognition of subtypes will help in planning the management of these patients. (author)

  20. Do scleroderma patients look young?: Evaluation by using facial imaging system.

    Science.gov (United States)

    Sawamura, Soichiro; Jinnin, Masatoshi; Kajihara, Ikko; Makino, Katsunari; Aoi, Jun; Ichihara, Asako; Makino, Takamitsu; Fukushima, Satoshi; Ihn, Hironobu

    2017-01-01

    These days various collagen supplements have widely been marketed. However, it has not been scientifically proved whether increasing collagen can actually prevent skin aging. Systemic sclerosis (SSc) is an autoimmune disease that is characterized by thickening of the skin caused by accumulation of collagen. In this study, we tried to evaluate facial skin characteristics and skin aging of SSc patients by using digital imaging system. As the result, the severity of wrinkles, texture and pores were significantly lower in SSc patients than control subjects. Among them, wrinkles showed better correlation with skin thickness score. Therefore, increased amount of collagen in scleroderma skin may directly affect wrinkles. In conclusion, attempt on collagen induction itself is reasonable and effective strategy in order to keep young appearance, although oral collagen supplementation may not directly reach to the skin.

  1. Microcirculatory disorders in scleroderma systematica: an association with vascular wall stiffness

    Directory of Open Access Journals (Sweden)

    Ulyana Yuryevna Ruzhentsova

    2013-01-01

    Full Text Available Objective: to study the specific features of regulation of peripheral vascular tone and their association with the endothelial structure and function of large vessels in patients with scleroderma systematica (SDS. Subjects and methods. The investigation enrolled 25 patients with SDS (mean age, 47±2.6 years; mean disease duration, 8.3+1.7 years and 15 apparently healthy individuals matched for age and gender. Comprehensive examination involved laboratory and instrumental studies, laser Doppler study to evaluate endothelium-dependent and endothelium-independent vasodilation, as well as applanation tonometry calculating the pulse wave velocity and augmentation index. Results. All the patients were found to have impaired peripheral vascular responsiveness as compared to the controls. The examination established a relationship between the magnitude of endothelium-dependent vasodilation and the stiffness index of large vessels. There was no association between endothelium-independent vasodilation and vascular elasticity parameters.

  2. The expression of myocardial injury in cold induced myocardial imaging and echocardiography of systematic scleroderma

    International Nuclear Information System (INIS)

    Liang Jiugen; Zhu Xiaojun; Jiang Ningyi; Chen Shaoxiong

    1999-01-01

    The study was performed with cold-induced 99m Tc(MIBI) myocardial imaging (MI) in 23 patients with systematic scleroderma. The left ventricular function and wall motion were also observed by dimensional echocardiography (UCG). 14 patients had myocardial perfusion abnormalities visualized by MI, including 5 cases with fixed defects of 9 segments, 3 cases with reversible defects of 6 segments and 6 cases with both fixed and reversible one of 14 segments. The positive rate in myocardial imaging had no significant differences between patients with and without Raynaud's phenomenon (0.5>P>0.25). Compared with baseline, the ejection fraction, stroke volume, cardiac output were significantly decreased during cold-induced in patients with abnormal myocardial scintigraphy (P<0.05), and had significant difference compared with normal group (P<0.05). 4 cases with cold-induced reversible perfusion defects had anatomically correlated regional ventricular hypokinesia in UCG

  3. Scleroderma keratinocytes promote fibroblast activation independent of transforming growth factor beta.

    Science.gov (United States)

    McCoy, Sara S; Reed, Tamra J; Berthier, Celine C; Tsou, Pei-Suen; Liu, Jianhua; Gudjonsson, Johann E; Khanna, Dinesh; Kahlenberg, J Michelle

    2017-11-01

    SSc is a devastating disease that results in fibrosis of the skin and other organs. Fibroblasts are a key driver of the fibrotic process through deposition of extracellular matrix. The mechanisms by which fibroblasts are induced to become pro-fibrotic remain unclear. Thus, we examined the ability of SSc keratinocytes to promote fibroblast activation and the source of this effect. Keratinocytes were isolated from skin biopsies of 9 lcSSc, 10 dcSSc and 13 control patients. Conditioned media was saved from the cultures. Normal fresh primary fibroblasts were exposed to healthy control and SSc keratinocyte conditioned media in the presence or absence of neutralizing antibodies for TGF-β. Gene expression was assessed by microarrays and real-time PCR. Immunocytochemistry was performed for α-smooth muscle actin (α-SMA), collagen type 1 (COL1A1) and CCL5 expression. SSc keratinocyte conditioned media promoted fibroblast activation, characterized by increased α-SMA and COL1A1 mRNA and protein expression. This effect was independent of TGF-β. Microarray analysis identified upregulation of nuclear factor κB (NF-κB) and downregulation of peroxisome proliferator-activated receptor γ (PPAR-γ) pathways in both SSc subtypes. Scleroderma keratinocytes exhibited increased expression of NF-κB-regulated cytokines and chemokines and lesional skin staining confirmed upregulation of CCL5 in basal keratinocytes. Scleroderma keratinocytes promote the activation of fibroblasts in a TGF-β-independent manner and demonstrate an imbalance in NF-κB1 and PPAR-γ expression leading to increased cytokine and CCL5 production. Further study of keratinocyte mediators of fibrosis, including CCL5, may provide novel targets for skin fibrosis therapy. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  4. REFORMATIONS IN ZIMBABWE'S JUVENILE JUSTICE SYSTEM

    African Journals Online (AJOL)

    Mugumbate

    1996-05-23

    May 23, 1996 ... The article is based on a desk review of existing literature on juvenile crime in the country. ... that Zimbabwe's juvenile justice system is transforming from being ... recommendations include expanding the Pre-trial Diversion ...

  5. Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

    Directory of Open Access Journals (Sweden)

    Rohit Tewari

    2016-01-01

    Full Text Available Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.

  6. Litter-forager termite mounds enhance the ectomycorrhizal symbiosis between Acacia holosericea A. Cunn. Ex G. Don and Scleroderma dictyosporum isolates.

    Science.gov (United States)

    Duponnois, Robin; Assikbetse, Komi; Ramanankierana, Heriniaina; Kisa, Marija; Thioulouse, Jean; Lepage, Michel

    2006-05-01

    The hypothesis of the present study was that the termite mounds of Macrotermes subhyalinus (MS) (a litter-forager termite) were inhabited by a specific microflora that could enhance with the ectomycorrhizal fungal development. We tested the effect of this feeding group mound material on (i) the ectomycorrhization symbiosis between Acacia holosericea (an Australian Acacia introduced in the sahelian areas) and two ectomycorrhizal fungal isolates of Scleroderma dictyosporum (IR408 and IR412) in greenhouse conditions, (ii) the functional diversity of soil microflora and (iii) the diversity of fluorescent pseudomonads. The results showed that the termite mound amendment significantly increased the ectomycorrhizal expansion. MS mound amendment and ectomycorrhizal inoculation induced strong modifications of the soil functional microbial diversity by promoting the multiplication of carboxylic acid catabolizing microorganisms. The phylogenetic analysis showed that fluorescent pseudomonads mostly belong to the Pseudomonads monteillii species. One of these, P. monteillii isolate KR9, increased the ectomycorrhizal development between S. dictyosporum IR412 and A. holosericea. The occurrence of MS termite mounds could be involved in the expansion of ectomycorrhizal symbiosis and could be implicated in nutrient flow and local diversity.

  7. Evolution of Juvenile Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Ye.V. Prohorov

    2013-02-01

    Full Text Available Evolution of juvenile ankylosing spondylitis tend to follow a more frequent involvement in the pathological process of elbow and ankle joints, development of enthesiopathies, changes of intraarticular meniscal horns, forming of Baker’s cysts, cartilage flaps and systemic osteoporosis, and total value of all these signs 13 times exceeds thereof in patients with with the debut of disease in adulthood, but for juvenile ankylosing spondylitis vertebral lesion is less common. Age dimorphism of the use of certain groups of drugs and physiotherapy facilities is observed.

  8. Juvenile technologies in foreign publications

    Directory of Open Access Journals (Sweden)

    Shpagina E.M.

    2012-09-01

    Full Text Available The article provides the review of foreign publications, concerning the juvenile technologies used in France, Canada, Germany and Switzerland. The paper presents legal, social and psychotherapeutic aspects of juvenile judiciary in foreign countries. The authors paid special attention to the complexity of approaches to young children and teenagers who found themselves in complicated life circumstances or got into trouble with the law. The article gives examples of using the following techniques: cognitive-behavioral intervention, mediation, family therapy (including family background and family history, relations theory, narrative practices, utilization of «emotional intelligence» resources.

  9. Nailfold capillaroscopy in children and adolescents with rheumatic diseases.

    Science.gov (United States)

    Piotto, Daniela Gerent Petry; Len, Cláudio Arnaldo; Hilário, Maria Odete Esteves; Terreri, Maria Teresa Ramos Ascensão

    2012-10-01

    To assess nailfold capillaroscopy in children and adolescents with autoimmune rheumatic diseases (juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma and mixed connective tissue disease) and relate it to clinical and laboratory findings and disease activity. Cross-sectional study assessing 147 patients by use of nailfold capillaroscopy as follows: 60 with juvenile idiopathic arthritis; 30 with systemic lupus erythematosus; 30 with juvenile dermatomyositis; 20 with localized scleroderma; four with systemic sclerosis; and three with mixed connective tissue disease. Clinical and laboratory tests and nailfold capillaroscopy were performed in all patients. The nailfold capillaroscopy was performed with an optical microscope (at 10- and 16-time magnifications) by the same observer. Most patients (76.2%) had normal nailfold capillaroscopy. The major changes in nailfold capillaroscopy, characterizing the scleroderma pattern, were observed in patients with juvenile dermatomyositis, systemic scleroderma and mixed connective tissue disease. There was no association between nailfold capillaroscopy and disease activity in patients with juvenile idiopathic arthritis, systemic lupus erythematosus and localized scleroderma. Disease activity and capillaroscopy were associated in patients with juvenile dermatomyositis. Nailfold capillaroscopy is a useful method to diagnose autoimmune rheumatic diseases and monitor disease activity.

  10. Juvenile Courts. Creation and development

    Directory of Open Access Journals (Sweden)

    Montserrat GONZÁLEZ FERNÁNDEZ

    2013-11-01

    Full Text Available This paper studies the creation of Juvenile or Children's Courts in Spain, analysing their reasons and aims, as well as the ethical and political connotations present on their way of acting. Their history and the one of the institutions that complement them is built from the legislation, writings and ideas of their promoters.

  11. Juvenile Justice: A Bibliographic Essay.

    Science.gov (United States)

    Kondak, Ann

    1979-01-01

    Provides information on the background and legal framework of the juvenile justice system, the issues that confront it, and the pressures for change, as well as noting some sources of information on the system. Available from American Association of Law Libraries, 53 West Jackson Blvd., Suite 1201, Chicago, Illinois 60604; sc $4.00. (Author/IRT)

  12. [Sex-linked juvenile retinoschisis].

    Science.gov (United States)

    François, P; Turut, P; Soltysik, C; Hache, J C

    1976-02-01

    About 13 observations of sexe linked juvenile retinoschisis, the authors describe the ophthalmoscopic, fluorographic and functional aspects of the disease whose caracteristics are:--its sexe linked recessive heredity; --its clinical characterestics associating: a microcystic macular degeneration, peripheral retinal lesions, vitreous body alterations, --an electroretinogram of the negative type.

  13. Juvenile European anchovy otolith microstructure

    Directory of Open Access Journals (Sweden)

    Pablo Cermeño

    2006-09-01

    Full Text Available Juvenile European anchovy (Engraulis encrasicolus has a complex incremental growth pattern that was studied using scanning electron microscope (SEM and optical microscope observations. Daily increments were identified and related to rhythmic growth patterns while double-band structures were identified as one increment. The causes of these growth patterns are discussed.

  14. CT appearance of juvenile angiofibroma

    Energy Technology Data Exchange (ETDEWEB)

    Ueda, Jun; Hara, Kazuo (Sumitomo Hospital, Osaka (Japan)); Fukuzumi, Akio; Uchida, Hideo

    1983-06-01

    Three verified cases of juvenile angiofibroma were presented. All of them were young and adolescent male CT proved to be an ideal tool in evaluating the extension of this tumor. The appearance on plain CT was multilobulated with displacement of the adjacent bony structures. On enhancement, there was intense staining of the tumor.

  15. What is Justice for Juveniles?

    Science.gov (United States)

    Rothwell, Jennifer Truran

    1997-01-01

    Provides background information and related learning activities for three areas of inquiry involving youth and violence: (1) "Evolution of the Juvenile Justice System"; (2) "The Literature of Crime and Poverty"; (3) "Youth Crime and Public Policy." Includes a list of six recommended Web sites. (MJP)

  16. Juvenile Ossifying Fibroma of the Mandible: a Case Report

    Directory of Open Access Journals (Sweden)

    Bahar Keles

    2010-04-01

    Full Text Available Background: Fibro-osseous lesions of the jaws, including juvenile ossifying fibroma, pose diagnostic and therapeutic difficulties due to their clinical, radiological and histological variability. The aim of this study was to report the outcome of a 9 years old girl with diagnosed juvenile ossifying fibroma treatment.Methods: A 9 years old girl presented with a 6 x 8 cm sized hard fixed tumour on right ramus and corpus of the mandible. On the radiological examination tumour showed an irregular but well bordered, unilocular and expansive lesion on the right corpus and ramus of the mandible. There was no teeth displacement or teeth root resorbtion. Microscopically, the tumour had trabeculae, fibrillary osteoid and woven bone. After the clinical, radiological (panoramic radiography, computed tomography and magnetic resonance imaging and histologic analysis it was diagnosed juvenile ossifying fibroma. In the history of the patient there has been an acute lymphocytic leukaemia in the remission for 3 years.Results: Because of large size of mandibular tumour, resultant expansion and destruction of mandibular cortex, the patient underwent right hemimandibulectomy using transmandibular approach. There was no recurrence or complications for two years follow-up.Conclusions: Although juvenile ossifying fibroma is an uncommon clinical entity, its aggressive local behaviour and high recurrence rate means that it is important to make an early diagnosis, apply the appropriate treatment and, especially, follow-up the patient over the long-term.

  17. Juvenile Nasophryngeal Angiofibroma,New Aspects in Management

    Directory of Open Access Journals (Sweden)

    Mojtaba Mohammadi Ardehali

    2011-03-01

    Full Text Available Juvenile nasopharyngeal angiofibroma (JNA is a rare, benign and locally invasive tumor. Massive bleeding because of vascularity of tumor and postoperative recurrence are potential problems of management. Endonasal approach reduces the rate of postoperative recurrence, intraoperative bleeding, and complications of surgery, time of operation and duration of hospitalization. In this article, we review the indications and contraindications of endoscopic surgery, indications of radiotherapy in treatment of JNA and our policy in postoperative follow-up and management of patients.

  18. Juvenile primary extranasopharyngeal angiofibroma, presenting as cheek swelling

    Science.gov (United States)

    Nandhini, J; Ramasamy, S; Kaul, Ronak Nazir; Austin, Ravi David

    2018-01-01

    Angiofibroma is a locally advancing immensely vascular tumor that essentially arises from the nasopharynx. The clinical characteristics of extranasopharyngeal angiofibroma (ENA) do not accord to that of nasopharyngeal angiofibroma and can present a diagnostic confront. We describe a case of primary juvenile ENA in a 19-year-old patient who presented with a rapidly enlarging mass of the cheek region. The case is unusual because of its anatomic location. The diagnostic and management particulars are sketched. PMID:29491611

  19. Radiotherapy of early breast cancer in scleroderma patients: our experience with four cases and a short review of the literature

    Directory of Open Access Journals (Sweden)

    Kyrgias G

    2012-01-01

    Full Text Available George Kyrgias1,2, Kiki Theodorou3,4, Anna Zygogianni1, Konstantinos Tsanadis2, Stefanos Zervoudis5, John Tzitzikas6, Michael Koukourakis71Academic Radiotherapy, University of Thessaly, Medical School, Greece; 2Radiation Oncology Department, University Hospital of Larissa, Greece; 3Academic Medical Physics, University of Thessaly, Medical School, Greece; 4Medical Physics Department, University Hospital of Larissa, 5Breast Unit, REA Hospital, Athens, Greece; 6Radiation Oncology Department, AHEPA University Hospital of Thessaloniki, Greece; 7Radiotherapy-Oncology Department, University Hospital of Alexandroupolis, GreecePurpose: Connective vascular diseases (CVD, including scleroderma, are reported to represent for some researchers a relative contraindication and for others absolute contraindication for radiotherapy. The purpose of our study is to add four new cases to the existing body of international literature and to determine whether women with pre-existing scleroderma who have been surgically treated for early breast cancer could undergo postsurgical radiotherapy without serious early and late complications.Patients and methods: From May 1998 to November 2010, we irradiated for early breast cancer four patients suffering from pre-existing scleroderma; after conservative surgery, we performed whole breast postoperative radiotherapy of 50.4 Gy total dose to the whole breast plus a 9 Gy boost to the tumor bed. We reviewed the records of all four patients and evaluated the early and late reactions using acute radiation morbidity scoring criteria (Radiation Therapy Oncology Group [RTOG], American College of Radiology, Philadelphia, PA and late radiation morbidity scoring scheme (European Organisation for Research and Treatment of Cancer [EORTC], Brussels, Belgium and RTOG.Results: After a median follow-up of 105 months (range 12–155 months the early and late toxicity concerning the skin, the subcutaneous tissues, the lungs, and the heart have

  20. Autoantibodies in scleroderma and their association with the clinical profile of the disease. A study of 66 patients from southern Brazil.

    Science.gov (United States)

    Skare, Thelma Larocca; Fonseca, Adriano Erlon; Luciano, Alan Campos; Azevedo, Pedro Ming

    2011-01-01

    Scleroderma is a fairly rare connective tissue disease whose autoantibody profile is associated with different clinical manifestations. The prevalence of autoantibodies in scleroderma is influenced by race and genetics. To study the prevalence of anti-Scl-70, anti-centromere (ACA) and anti-U1-RNP antibodies in patients with scleroderma in southern Brazil and verify their association with clinical manifestations of the disease. A retrospective study involving 66 patients with scleroderma for the presence of anti-Scl-70, anti-centromere and anti-U1-RNP and of clinical manifestations such as Raynaud's phenomenon, digital micro scars, digital necrosis, telangiectasias, calcinosis, pulmonary fibrosis, pleuritis, pericarditis, cardiomyopathy, arthralgia and arthritis, skin sclerosis, joint contractures, tendon friction rubs, pulmonary hypertension, esophageal disorders and renal crisis. The prevalence of anti-Scl-70 was 17.8% , that of ACA was 33.3% and the prevalence of U1 RNP was 11.8%. Anti-Scl-70 was associated with the diffuse form of the disease (p = 0.015), presence of cardiomyopathies (p = 0.016) and digital micro scars (p = 0.05). Anti-centromere was more common in the limited form, although it was not statistically significant, and had a protective role associated with cardiomyopathies (p = 0.005). Anti-U1-RNP was more common in the overlap forms (p = 0.0004). The prevalence and profile of clinical associations of autoantibodies in Brazilian patients with scleroderma are similar to those found in the literature.

  1. The thickness of the A1 pulleys reflects the disability of hand mobility in scleroderma. A pilot study using high-frequency ultrasound

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto, E-mail: atagliafico@sirm.org [Department of Radiology, National Institute for Cancer Research, Genova (Italy); Panico, Nicoletta [Division of Immunology, Department of Internal Medicine, University of Genova, Genova (Italy); Serafini, Giovanni [Department of Radiology, Santa Corona Hospital, Pietra Ligure (Italy); Ghio, Massimo [Division of Immunology, Department of Internal Medicine, University of Genova, Genova (Italy); Martinoli, Carlo [Department of Radiology, University of Genova, Genova (Italy)

    2011-02-15

    Background: Hand involvement in scleroderma is a serious concern. Clinical tests to asses hand dysfunction are based on the experience of the clinician. Objective: To asses if utrasonographic (US) measurement of A1 pulley thickness may be used as an indicator of hand mobility in scleroderma. Materials and methods: Institutional review board approval and patient informed consent was obtained. Twenty-eight patients affected suffering from scleroderma and 40 healthy controls were prospectively evaluated by two blinded radiologists with US, with a transducer operating at 17 MHz. A1 pulley thickness was measured and correlated with the Hand Mobility in Scleroderma Test (HAMIS) and disease duration. Results: The thickness of the A1 pulley was greater in sclerodermic patients than in controls (p < 0.05). Intra and inter-observer agreement were better for ultrasound (0.94;0.88) than for HAMIS tests (0.71;0.70). A good correlation between pulley thickness, hand mobility and disease duration was found (r = 0.78, p < 0.018; r = 0.54, p < 0.05). Conclusion: A1 pulley thickness measured on ultrasound correlates with hand mobility and disease duration. Ultrasound is an useful tool to evaluate hand disability in scleroderma.

  2. The thickness of the A1 pulleys reflects the disability of hand mobility in scleroderma. A pilot study using high-frequency ultrasound

    International Nuclear Information System (INIS)

    Tagliafico, Alberto; Panico, Nicoletta; Serafini, Giovanni; Ghio, Massimo; Martinoli, Carlo

    2011-01-01

    Background: Hand involvement in scleroderma is a serious concern. Clinical tests to asses hand dysfunction are based on the experience of the clinician. Objective: To asses if utrasonographic (US) measurement of A1 pulley thickness may be used as an indicator of hand mobility in scleroderma. Materials and methods: Institutional review board approval and patient informed consent was obtained. Twenty-eight patients affected suffering from scleroderma and 40 healthy controls were prospectively evaluated by two blinded radiologists with US, with a transducer operating at 17 MHz. A1 pulley thickness was measured and correlated with the Hand Mobility in Scleroderma Test (HAMIS) and disease duration. Results: The thickness of the A1 pulley was greater in sclerodermic patients than in controls (p < 0.05). Intra and inter-observer agreement were better for ultrasound (0.94;0.88) than for HAMIS tests (0.71;0.70). A good correlation between pulley thickness, hand mobility and disease duration was found (r = 0.78, p < 0.018; r = 0.54, p < 0.05). Conclusion: A1 pulley thickness measured on ultrasound correlates with hand mobility and disease duration. Ultrasound is an useful tool to evaluate hand disability in scleroderma.

  3. Colony size-frequency distribution of pocilloporid juvenile corals along a natural environmental gradient in the Red Sea

    KAUST Repository

    Lozano-Corté s, Diego; Berumen, Michael L.

    2015-01-01

    Coral colony size-frequency distributions can be used to assess population responses to local environmental conditions and disturbances. In this study, we surveyed juvenile pocilloporids, herbivorous fish densities, and algal cover in the central

  4. Antifibrotic effects of crocetin in scleroderma fibroblasts and in bleomycin-induced sclerotic mice

    Directory of Open Access Journals (Sweden)

    Yinghua Song

    2013-10-01

    Full Text Available OBJECTIVE: To investigate the antifibrotic effects of crocetin in scleroderma fibroblasts and in sclerotic mice. METHODS: Skin fibroblasts that were isolated from three systemic scleroderma (SSc patients and three healthy subjects were treated with crocetin (0.1, 1 or 10 μM. Cell proliferation was measured with an MTT assay. Alpha-smooth muscle actin was detected via an immunohistochemical method. Alpha 1 (I procollagen (COL1A1, alpha 1 (III procollagen (COL3A1, matrix metalloproteinase (MMP-1 and tissue inhibitor of matrix metalloproteinase (TIMP-1 mRNA levels were measured using real-time PCR. SSc mice were established by the subcutaneous injection of bleomycin. Crocetin (50 mg/kg/d was injected intraperitoneally for 14 days. Dermal thickness and lung fibrosis were assessed with Masson's trichrome staining. Plasma ET-1 was detected with an enzyme-linked immunosorbent assay (ELISA. Skin and lung ET-1 and COL1A1 mRNA levels were measured via real-time PCR. RESULTS: Crocetin inhibited the proliferation of SSc and normal fibroblasts, an effect that increased with crocetin concentration and incubation time. Crocetin decreased the expression of α-SMA and the levels of mRNA for COL1A1, COL3A1 and matrix metalloproteinase-1, while crocetin increased TIMP-1 mRNA levels in both SSc and normal fibroblasts. Skin and lung fibrosis was induced, and the levels of ET-1 in the plasma, skin and lungs were elevated in bleomycin-injected mice. Crocetin alleviated the thickening of the dermis and lung fibrosis; decreased COL1A1 mRNA levels in the skin and lung; and simultaneously decreased ET-1 concentrations in the plasma and ET-1 mRNA levels in the skin and lungs of the bleomycin-induced sclerotic mice, especially during the early phase (weeks 1-3. CONCLUSION: Crocetin inhibits cell proliferation, differentiation and collagen production in SSc fibroblasts. Crocetin alleviates skin and lung fibrosis in a bleomycin-induced SSc mouse model, in part due to a

  5. Determination of muscle microcirculation of the limbs of healthy persons and patients with scleroderma by means of 133Xe clearance. 2

    International Nuclear Information System (INIS)

    Poenitzsch, I.; Wiemers, U.; Haustein, U.F.; Schneider, G.

    1984-01-01

    By means of 133 Xe muscle clearance the blood flow of the musculus tibialis anterior and the musculus opponens pollicis was determined during nonischemic work and after 3 minutes ischemia in patients with progressive scleroderma and additionally of the ischemic musculus opponens pollicis following contrast baths. In relation to 53 patients with normal vessels the reactive hyperemia of the musculus tibialis anterior after ischemia and of the musculus opponens pollicis after heat, cold as well as arterial flow decreasing was significantly decreased in patients with scleroderma and was as a disturbance of the microcirculation realized. For scientific problems in progressive scleroderma the 133 Xe muscle clearance is suitable as to the musculus opponens pollicis. (author)

  6. Metabolism of /sup 14/C-labelled L-tryptophan, L-kynurenine, and hydroxy-L-kynurenine in miners with scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Hankes, L.V.; De Bruin, E.; Jansen, C.R.; Vorster, L.; Schmaeler, M.

    1977-03-19

    Six South African white miners were studied with the 2-g L-tryptophan load test and tracer doses of L-tryptophan-7a-/sup 14/C, L-kynurenine-keto-/sup 14/C and hydroxy-L-kynurenine-keto-/sup 14/C. The breath /sup 14/CO/sub 2/ and 14 urinary metabolites were measured. When they were compared with a previous study of American women with scleroderma, similar /sup 14/CO/sub 2/ and tryptophan metabolite excretion patterns were observed in the data from the miners. The labelled quinolinic acid excretion was more significantly elevated in the South African miners' urine than in the urine of the American women. The data from both studies suggest that some patients with scleroderma have an altered step in the tryptophan metabolic pathway after hydroxy-anthranilic acid. What relationship exists between the induction of pulmonary silicosis and the subsequent development of scleroderma, requires additional human studies.

  7. Marcadores de inmunorrespuesta en la periodontitis juvenil

    Directory of Open Access Journals (Sweden)

    Amparo Pérez Borrego,

    2002-12-01

    Full Text Available La periodontitis juvenil es una enfermedad del periodonto propia de adolescentes y adultos jóvenes, afecta principalmente los primeros molares y los incisivos y se caracteriza por la pérdida severa del hueso alveolar alrededor de dientes permanentes sin correspondencia entre la rapidez y severidad de la destrucción con los factores locales. En la causa de la enfermedad se citan factores genéticos, infecciosos e inmunológicos. Estudiamos algunos marcadores de inmunorrespuesta en 6 adolescentes que acudieron a nuestro servicio con el diagnóstico de periodontitis juvenil, además de su valoración clínica y radiológica. Ambos sexos se afectaron por igual, la movilidad dentaria y el sangramiento al sondeo fueron los hallazgos clínicos más relevantes y el índice de higiene bucal fue adecuado en todos los casos. No encontramos homogeneidad en las alteraciones inmunológicas, pero todos los pacientes estuvieron afectados en más de un marcador. Predominaron las alteraciones funcionales de linfocitos T en los estudios celulares. La hipogammaglobulinemia y la IgM elevada fueron las alteraciones más frecuentes en la inmunidad de anticuerpos. Se señala la dificultad que aún existe para explicar la patogenia de la enfermedad basándose solamente en un único factor de riesgo, así como la importancia de la valoración individual de cada enfermo.Juvenile periodontitis is a disease of the periodontium inherent to adolescents and young adults, affecting mainly the first molars and incisives and characterized by the severe loss of the alveolar bone sorrounding the permanent teeth with no correspondance between the celerity and severity of the destruction and the local factors. Genetic, infectious and immunological factors are considereed as the causes of the disease. Some immunoresponse markers were studied in 6 adolescents that were seen at our service with the diagnosis of juvenile periodontitis in addition to their clinical and radiological

  8. Soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) in scleroderma skin

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Deleuran, Mette; Heickendorff, Lene

    1998-01-01

    In order to investigate whether soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) were present in scleroderma skin, and to compare their levels to concentrations measured in plasma and clinical parameters, we examined suction blister fluid and plasma...... from 13 patients with systemic sclerosis and 11 healthy volunteers. Suction blisters and biopsies were from the transition zone between normal skin and scleroderma, and uninvolved abdominal skin. The levels of sICAM-1 and sIL-2R were significantly increased in both plasma and suction blister fluid from...

  9. Parasitization by Scleroderma guani influences expression of superoxide dismutase genes in Tenebrio molitor.

    Science.gov (United States)

    Zhu, Jia-Ying; Ze, Sang-Zi; Stanley, David W; Yang, Bin

    2014-09-01

    Superoxide dismutase (SOD) is an antioxidant enzyme involved in detoxifying reactive oxygen species. In this study, we identified genes encoding the extracellular and intracellular copper-zinc SODs (ecCuZnSOD and icCuZnSOD) and a manganese SOD (MnSOD) in the yellow mealworm beetle, Tenebrio molitor. The cDNAs for ecCuZnSOD, icCuZnSOD, and MnSOD, respectively, encode 24.55, 15.81, and 23.14 kDa polypeptides, which possess structural features typical of other insect SODs. They showed 20-94% identity to other known SOD sequences from Bombyx mori, Musca domestica, Nasonia vitripennis, Pediculus humanus corporis, and Tribolium castaneum. Expression of these genes was analyzed in selected tissues and developmental stages, and following exposure to Escherichia coli and parasitization by Scleroderma guani. We recorded expression of all three SODs in cuticle, fat body, and hemocytes and in the major developmental stages. Relatively higher expressions were detected in late-instar larvae and pupae, compared to other developmental stages. Transcriptional levels were upregulated following bacterial infection. Analysis of pupae parasitized by S. guani revealed that expression of T. molitor SOD genes was significantly induced following parasitization. We infer that these genes act in immune response and in host-parasitoid interactions. © 2014 Wiley Periodicals, Inc.

  10. Parasitization by Scleroderma guani influences protein expression in Tenebrio molitor pupae.

    Science.gov (United States)

    Zhu, Jia-Ying; Wu, Guo-Xing; Ze, Sang-Zi; Stanley, David W; Yang, Bin

    2014-07-01

    Ectoparasitoid wasps deposit their eggs onto the surface and inject venom into their hosts. Venoms are chemically complex and they exert substantial impact on hosts, including permanent or temporary paralysis and developmental arrest. These visible venom effects are due to changes in expression of genes encoding physiologically relevant proteins. While the influence of parasitization on gene expression in several lepidopterans has been reported, the molecular details of parasitoid/beetle relationships remain mostly unknown. This shortcoming led us to pose the hypothesis that envenomation by the ectoparasitic ant-like bethylid wasp Scleroderma guani leads to changes in protein expression in the yellow mealworm beetle Tenebrio molitor. We tested our hypothesis by comparing the proteomes of non-parasitized and parasitized host pupae using iTRAQ-based proteomics. We identified 41 proteins that were differentially expressed (32↑- and 9↓-regulated) in parasitized pupae. We assigned these proteins to functional categories, including immunity, stress and detoxification, energy metabolism, development, cytoskeleton, signaling and others. We recorded parallel changes in mRNA levels and protein abundance in 14 selected proteins following parasitization. Our findings support our hypothesis by documenting changes in protein expression in parasitized hosts. Copyright © 2014 Elsevier Ltd. All rights reserved.

  11. Decreased interleukin-20 expression in scleroderma skin contributes to cutaneous fibrosis.

    Science.gov (United States)

    Kudo, Hideo; Jinnin, Masatoshi; Asano, Yoshihide; Trojanowska, Maria; Nakayama, Wakana; Inoue, Kuniko; Honda, Noritoshi; Kajihara, Ikko; Makino, Katsunari; Fukushima, Satoshi; Ihn, Hironobu

    2014-06-01

    To clarify the role of interleukin-20 (IL-20) in the regulatory mechanism of extracellular matrix expression and to determine the contribution of IL-20 to the phenotype of systemic sclerosis (SSc). Protein and messenger RNA (mRNA) levels of collagen, Fli-1, IL-20, and IL-20 receptor (IL-20R) were analyzed using polymerase chain reaction (PCR) array, immunoblotting, immunohistochemical staining, enzyme-linked immunosorbent assay, and real-time PCR. PCR array revealed that IL-20 decreased gene expression of α2(I) collagen (0.03-fold), Smad3 (0.02-fold), and endoglin (0.05-fold) in cultured normal dermal fibroblasts. Fli-1 protein expression was induced by IL-20 (~2-fold). The inhibition of collagen by IL-20, the induction of Fli-1 by IL-20, and the reduction of Smad3 and endoglin by IL-20 were also observed in SSc fibroblasts. Serum IL-20 levels were reduced only slightly in SSc patients but were significantly decreased in patients with scleroderma spectrum disorders (the prodromal stage of SSc) compared with those in normal subjects (111.3 pg/ml versus 180.4 pg/ml; P value of IL-20 and IL-20R, their function and expression in vivo should be further studied. Copyright © 2014 by the American College of Rheumatology.

  12. The Assessment of Health-Related Quality of Life in Scleroderma-Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Shahrzad M Lari

    2014-05-01

    Full Text Available Introduction: Pulmonary involvement is the most common cause of mortality and disability in patients with systemic sclerosis and it significantly affects the quality of life in these patients. Therefore, early diagnosis and treatment of pulmonary involvement seems necessary in patients with SSc. In this study, we aimed to assess the health-related quality of life (HRQoL in patients with Scleroderma-Interstitial Lung Disease (SSc-ILD and its relationship with pulmonary function parameters. Materials and Methods: Considering the inclusion and exclusion criteria, 25 patients with SSc-ILD were enrolled in this cross-sectional study from April 2012 to June 2013. Full tests of lung function, including body plethysmography and diffusing capacity of the lungs for carbon monoxide (DLCO, 6-minute walk distance (6MWD, and pulse oximetry were performed. The HRQoL was assessed using St. George’s and CAT questionnaires; also, dyspnea was evaluated for all the patients, using modified medical research council (MMRC scale. Afterwards, the relationship between the total scores of HRQoL questionnaires and the severity of lung disease was analyzed, based on the recorded variables. Results: The mean age of the patients was 40.36±9.50 years and the mean duration of the disease was 7.16±4.50 years. A statistically significant inverse correlation was observed between 6MWD (r=-0.50, P=0.01, DLCO (r=-0.67, P

  13. Assessment of extent of skin involvement in scleroderma using shear wave elastography

    Directory of Open Access Journals (Sweden)

    Anupam Wakhlu

    2017-01-01

    Full Text Available Introduction: Scleroderma (systemic sclerosis [SSc] is a rare autoimmune disease which manifests as fibrosis in the skin and other internal organs. Conventionally, the modified Rodnan skin score (MRSS has been used to quantify the extent of skin fibrosis (resulting in skin tightness in SSc. This technique, although widely validated, is limited by the requirement of a trained, experienced assessor. Recent literature suggests that utilization of the objective ultrasound-based assessment of skin fibrosis utilizing shear wave elastography (SWE may be a more robust technique to detect early skin tightness in SSc. Methods: We evaluated the use of SWE (assessed by an experienced radiologist in 24 patients with SSc compared with 16 healthy controls. Results: Our patients were predominantly females, with median disease duration of 1.5 years and median MRSS of 17. There was minimal intraobserver variation in the assessment of SWE. Patients with SSc had higher SWE values (mean elasticity [Emean] compared to healthy controls at most assessed sites for the MRSS. The Emeancorrelated significantly at all sites with the MRSS scores. At the sites where MRSS was scored as 0 (normal, the Emeanin patients with SSc was higher when compared with similarly clinical normal skin in patients with SSc, suggesting potential early involvement of these areas of the skin with fibrosis. Conclusion: SWE is a promising tool to objectively assess skin fibrosis in SSc and may be useful in detecting early, subclinical skin involvement in this disease.

  14. Mortality Risk Prediction in Scleroderma-Related Interstitial Lung Disease: The SADL Model.

    Science.gov (United States)

    Morisset, Julie; Vittinghoff, Eric; Elicker, Brett M; Hu, Xiaowen; Le, Stephanie; Ryu, Jay H; Jones, Kirk D; Haemel, Anna; Golden, Jeffrey A; Boin, Francesco; Ley, Brett; Wolters, Paul J; King, Talmadge E; Collard, Harold R; Lee, Joyce S

    2017-11-01

    Interstitial lung disease (ILD) is an important cause of morbidity and mortality in patients with scleroderma (Scl). Risk prediction and prognostication in patients with Scl-ILD are challenging because of heterogeneity in the disease course. We aimed to develop a clinical mortality risk prediction model for Scl-ILD. Patients with Scl-ILD were identified from two ongoing longitudinal cohorts: 135 patients at the University of California, San Francisco (derivation cohort) and 90 patients at the Mayo Clinic (validation cohort). Using these two separate cohorts, a mortality risk prediction model was developed and validated by testing every potential candidate Cox model, each including three or four variables of a possible 19 clinical predictors, for time to death. Model discrimination was assessed using the C-index. Three variables were included in the final risk prediction model (SADL): ever smoking history, age, and diffusing capacity of the lung for carbon monoxide (% predicted). This continuous model had similar performance in the derivation (C-index, 0.88) and validation (C-index, 0.84) cohorts. We created a point scoring system using the combined cohort (C-index, 0.82) and used it to identify a classification with low, moderate, and high mortality risk at 3 years. The SADL model uses simple, readily accessible clinical variables to predict all-cause mortality in Scl-ILD. Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  15. Gammadelta receptor bearing T cells in scleroderma: enhanced interaction with vascular endothelial cells in vitro.

    Science.gov (United States)

    Kahaleh, M B; Fan, P S; Otsuka, T

    1999-05-01

    In view of the documented perivascular mononuclear cell infiltration in the involved organs in scleroderma (SSc) and the reported accumulation of gammadelta-T cells in SSc skin and lung, we evaluated gammadelta-T cell interaction with endothelial cells (EC) in vitro. gammadelta- and alphabeta-T cells were isolated from BPMN of SSc patients with early diffuse disease and of matched control subjects by an immunomagnetic method after stimulation with mycobacterium lysate and interleukin-2 for 2 weeks. Lymphocyte adhesion, proliferation, and cytotoxicity to EC were investigated. SSc gammadelta-T cells adhered to cultured EC and proliferated at higher rates than control cells. Furthermore, significant EC cytotoxicity by SSc gammadelta was seen. The cytotoxicity was blocked by addition of anti-gammadelta-TCR antibody and by anti-granzyme A antibody but not by anti-MHC class I and II antibodies. Expression of granzyme A mRNA was seen in five/five SSc gammadelta-T cells and in one/five control cells. alphabeta-T cells from both SSc and control subjects were significantly less interactive with EC than gammadelta-T cells. The data demonstrate EC recognition by SSc gammadelta-T cells and propose gammadelta-T cells as a possible effector cell type in the immune pathogenesis of SSc. Copyright 1999 Academic Press.

  16. Pulmonary clearance of sup 99m Tc-DTPA aerosol in patients with progressive systemic scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Tateno, Madoka; Nakano, Akihiko; Hasegawa, Akira; Watanabe, Naoyuki; Oriuchi, Noboru; Inoue, Tomio; Endo, Keigo (Gunma Univ., Maebashi (Japan). School of Medicine); Sasaki, Yasuhito

    1992-05-01

    Alveolar epithelial permeability was assessed in 32 patients with progressive systemic scleroderma (PSS), using {sup 99m}Tc-DTPA aerosol. Immediately after the inhalation of {sup 99m}Tc-DTPA aerosol for 3 to 6 minutes under normal tidal breathing, lung was imaged sequentially for 30 minutes from the posterior by a gamma camera and exponential fitting was processed on the time activity curve. T{sub 1/2} (min) was used as a parameter for the evaluation of permeability of alveolar epithelium. Patients with collagen disease showed shorter T{sub 1/2} (T{sub 1/2}=43.7{+-}23.8 min) than the normal volunteers (T{sub 1/2}=76.8{+-}8.7 min). No significant difference was observed between patients with or without interstitial changes on the chest CT. Significant correlation was not observed between T{sub 1/2} and %VC or %DLco. In 8 cases, studies were repeated in the interval of 3 to 19 months. Improvement of T{sub 1/2} was seen in 4 cases, independent of CT findings. These results suggest that {sup 99m}Tc-DTPA aerosol clearance study provides information independent from other lung examinations, and may be useful for the assessment of lung interstitial changes in patients with PSS. (author).

  17. Effects of bosentan on collagen type I synthesis on in vitro culture of scleroderma skin fibroblasts

    Directory of Open Access Journals (Sweden)

    S. Soldano

    2011-01-01

    Full Text Available The present study evaluated the effects of a non-selective endothelin (ETA/B receptors antagonist, on collagen type I (COLI synthesis on in vitro culture of scleroderma (SSc skin fibroblasts (Fb. Fb were obtained from skin biopsies of 6 female SSc patients (mean age 64. 1±6 years, after informed consent and Ethical Committee Approval. Cells were treated with endothelin-I [ET-I, 100nM] for 24 and 48 hrs, pre-treated for I hr with ETA/B receptors antagonist [10nM] alone or followed by ET-I for 24 and 48 hrs. Untreated Fb were used as controls. Immunocytochemistry and western blot analysis were performed to evaluate COLI synthesis. ET-I increased COLI synthesis both at 24 and 48 hrs when compared to controls. ETA/B receptor antagonost blocks the increased COLI synthesis ET-I-mediated both at 24 and 48 hrs vs. ET-I. Results showed that ET-I receptors blockage by ETA/B receptors antagonist might prevent the excessive synthesis of COLI, supporting its positive action in the management of skin fibrosis.

  18. Pulmonary clearance of 99mTc-DTPA aerosol in patients with progressive systemic scleroderma

    International Nuclear Information System (INIS)

    Tateno, Madoka; Nakano, Akihiko; Hasegawa, Akira; Watanabe, Naoyuki; Oriuchi, Noboru; Inoue, Tomio; Endo, Keigo; Sasaki, Yasuhito.

    1992-01-01

    Alveolar epithelial permeability was assessed in 32 patients with progressive systemic scleroderma (PSS), using 99m Tc-DTPA aerosol. Immediately after the inhalation of 99m Tc-DTPA aerosol for 3 to 6 minutes under normal tidal breathing, lung was imaged sequentially for 30 minutes from the posterior by a gamma camera and exponential fitting was processed on the time activity curve. T 1/2 (min) was used as a parameter for the evaluation of permeability of alveolar epithelium. Patients with collagen disease showed shorter T 1/2 (T 1/2 =43.7±23.8 min) than the normal volunteers (T 1/2 =76.8±8.7 min). No significant difference was observed between patients with or without interstitial changes on the chest CT. Significant correlation was not observed between T 1/2 and %VC or %DLco. In 8 cases, studies were repeated in the interval of 3 to 19 months. Improvement of T 1/2 was seen in 4 cases, independent of CT findings. These results suggest that 99m Tc-DTPA aerosol clearance study provides information independent from other lung examinations, and may be useful for the assessment of lung interstitial changes in patients with PSS. (author)

  19. Juvenile morphology in baleen whale phylogeny.

    Science.gov (United States)

    Tsai, Cheng-Hsiu; Fordyce, R Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  20. An unusual presentation of juvenile lupus nephritis

    Directory of Open Access Journals (Sweden)

    Malleshwar Bottu

    2016-01-01

    Full Text Available The incidence of juvenile lupus varies widely ranging between 4 and 250 per 100,000 population. Most common organ involvement in juvenile lupus is kidney. Neurological, cutaneous and hematological involvements are also involved. Skeletal muscle involvement in the form of myositis is rare. Myositis as presenting manifestation in juvenile lupus is also unusual. Herein, we report one such case wherein myositis preceded the onset of lupus nephritis

  1. Juvenile offenders: competence to stand trial.

    Science.gov (United States)

    Soulier, Matthew

    2012-12-01

    This article details the legal background and assists the reader in the preparation and practical conduct of evaluations regarding juvenile adjudicative competency. The material is presented to be useful as a guide to direct questions of competency and covers aspects of evaluation that include: legal standard for competency to stand trial, developmental immaturity, current practice in juvenile competency to stand trial, forensic evaluation of juvenile competency to stand trial, organizing the evaluation, collateral sources of information, psychiatric evaluation of juvenile adjudicative competency, assessment of mental disorder and intellectual disability, assessment of developmental status, assessment of functional abilities for adjudicative competence, and reaching the forensic opinion. Copyright © 2012 Elsevier Inc. All rights reserved.

  2. Radiation-induced skin injury in the animal model of scleroderma: implications for post-radiotherapy fibrosis

    International Nuclear Information System (INIS)

    Kumar, Sanath; Kolozsvary, Andrew; Kohl, Robert; Lu, Mei; Brown, Stephen; Kim, Jae Ho

    2008-01-01

    Radiation therapy is generally contraindicated for cancer patients with collagen vascular diseases (CVD) such as scleroderma due to an increased risk of fibrosis. The tight skin (TSK) mouse has skin which, in some respects, mimics that of patients with scleroderma. The skin radiation response of TSK mice has not been previously reported. If TSK mice are shown to have radiation sensitive skin, they may prove to be a useful model to examine the mechanisms underlying skin radiation injury, protection, mitigation and treatment. The hind limbs of TSK and parental control C57BL/6 mice received a radiation exposure sufficient to cause approximately the same level of acute injury. Endpoints included skin damage scored using a non-linear, semi-quantitative scale and tissue fibrosis assessed by measuring passive leg extension. In addition, TGF-β1 cytokine levels were measured monthly in skin tissue. Contrary to our expectations, TSK mice were more resistant (i.e. 20%) to radiation than parental control mice. Although acute skin reactions were similar in both mouse strains, radiation injury in TSK mice continued to decrease with time such that several months after radiation there was significantly less skin damage and leg contraction compared to C57BL/6 mice (p < 0.05). Consistent with the expected association of transforming growth factor beta-1 (TGF-β1) with late tissue injury, levels of the cytokine were significantly higher in the skin of the C57BL/6 mouse compared to TSK mouse at all time points (p < 0.05). TSK mice are not recommended as a model of scleroderma involving radiation injury. The genetic and molecular basis for reduced radiation injury observed in TSK mice warrants further investigation particularly to identify mechanisms capable of reducing tissue fibrosis after radiation injury

  3. IMPORTANCE OF CYCLOPHOSPHANUM IN THE TREATMENT OF INTERSTITIAL LUNG LESION IN PATIENTS WITH SCLERODERMA SYSTEMATICA (A REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Olga Aleksandrova Koneva

    2010-01-01

    Full Text Available Interstitial lung diseases (ILD are a common manifestation of scleroderma systematica (SSD that along with pulmonary arterial hypertension remains the leading cause of death in this nosological entity. As of now, cyclophosphanum remains the only immunosuppressant recommended by the European League against Rheumatism for the treatment of ILD in SSD. The paper analyzes the papers providing evidence for the efficacy of cyclophosphanum in ILD in patients with SSD. It also considers the regimens and duration of treatment with cyclophosphanum, ways of evaluating its efficacy and effects on extrapulmonary manifestations of SSD. It is concluded that cyclophosphanum has a positive, predominantly stabilizing, effect on the course of ILD in SSD.

  4. Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.

    Science.gov (United States)

    Lazzaroni, Maria-Grazia; Cavazzana, Ilaria; Colombo, Enrico; Dobrota, Rucsandra; Hernandez, Jasmin; Hesselstrand, Roger; Varju, Cecilia; Nagy, Gabriella; Smith, Vanessa; Caramaschi, Paola; Riccieri, Valeria; Hachulla, Eric; Balbir-Gurman, Alexandra; Chatelus, Emmanuel; Romanowska-Próchnicka, Katarzyna; Araújo, Ana Carolina; Distler, Oliver; Allanore, Yannick; Airò, Paolo

    2017-05-01

    To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. (3) A Delphi exercise was performed by 82 experts to reach consensus for cancer screening in anti-RNAP3+ patients. In the EUSTAR registry, anti-RNAP3 were associated in multivariable analysis with renal crisis and diffuse cutaneous involvement. In the case-control study, anti-RNAP3 were associated with gastric antral vascular ectasia, rapid progression of skin involvement, and malignancies concomitant to SSc onset (OR 7.38, 95% CI 1.61-33.8). When compared with other anti-RNAP3+ patients, those with concomitant malignancies had older age (p < 0.001) and more frequent diffuse cutaneous involvement (p = 0.008). The Delphi exercise highlighted the need for malignancy screening at the time of diagnosis for anti-RNAP3+ patients and tight followup in the following years. Anti-RNAP3+ patients with SSc have a high risk of concomitant malignancy. These results have implications for clinical practice and suggest regular screening for cancer in anti-RNAP3+ patients.

  5. Renal Shielding and Dosimetry for Patients With Severe Systemic Sclerosis Receiving Immunoablation With Total Body Irradiation in the Scleroderma: Cyclophosphamide or Transplantation Trial

    International Nuclear Information System (INIS)

    Craciunescu, Oana I.; Steffey, Beverly A.; Kelsey, Chris R.; Larrier, Nicole A.; Paarz-Largay, Cathy J.; Prosnitz, Robert G.; Chao, Nelson; Chute, John; Gasparetto, Cristina; Horwitz, Mitchell; Long, Gwynn; Rizzieri, David; Sullivan, Keith M.

    2011-01-01

    Purpose: To describe renal shielding techniques and dosimetry in delivering total body irradiation (TBI) to patients with severe systemic sclerosis (SSc) enrolled in a hematopoietic stem cell transplant protocol. Methods and Materials: The Scleroderma: Cyclophosphamide or Transplantation (SCOT) protocol uses a lymphoablative preparative regimen including 800 cGy TBI delivered in two 200-cGy fractions twice a day before CD34 + selected autologous hematopoietic stem cell transplantation. Lung and kidney doses are limited to 200 cGy to protect organs damaged by SSc. Kidney block proximity to the spinal cord was investigated, and guidelines were developed for acceptable lumbar area TBI dosing. Information about kidney size and the organ shifts from supine to standing positions were recorded using diagnostic ultrasound (US). Minimum distance between the kidney blocks (dkB) and the lumbar spine region dose was recorded, and in vivo dosimetry was performed at several locations to determine the radiation doses delivered. Results: Eleven patients were treated at our center with an anteroposterior (AP)/posteroanterior (PA) TBI technique. A 10% to 20% dose inhomogeneity in the lumbar spine region was achieved with a minimum kidney block separation of 4 to 5 cm. The average lumbar spine dose was 179.6 ± 18.1 cGy, with an average dkB of 5.0 ± 1.0 cm. Kidney block shield design was accomplished using a combination of US and noncontrast computerized tomography (CT) or CT imaging alone. The renal US revealed a wide range of kidney displacement from upright to supine positions. Overall, the average in vivo dose for the kidney prescription point was 193.4 ± 5.1 cGy. Conclusions: The dose to the kidneys can be attenuated while maintaining a 10% to 20% dose inhomogeneity in the lumbar spine area. Kidneys were localized more accurately using both US and CT imaging. With this technique, renal function has been preserved, and the study continues to enroll patients.

  6. DNA fingerprint similarity between female and juvenile brown-headed cowbirds trapped together

    Science.gov (United States)

    Hahn, D.C.; Fleischer, R.C.

    1995-01-01

    This DNA fingerprinting study investigates whether females of the brood parasite brown-headed cowbird, Molothrus ater, associate with their own juvenile offspring at feeding sites more often than would be expected by chance. Cowbirds lay their eggs in the nests of a variety of host species and, as far as is known, leave them to the care of foster parents. Using baited walk-in funnel traps, 36 adult female-juvenile pairs (or trios) of cowbirds were trapped. Blood samples were collected from these individuals to conduct DNA fingerprinting analyses, calculate similarity indices, and to compare S-values for the 11 comparisons of juveniles and the females with which they were caught with S-values of random pairings of juveniles and the females in adjacent gel lanes with which they were not caught. Overall band-sharing was significantly higher for the individuals trapped together than for the random pairings. These associations between juvenile cowbirds and their mothers could occur as a result of female cowbirds monitoring the development of their young in the nests where they have laid. Alternatively, nestling cowbirds in the nest could become familiar visually and locally with a female parent that is frequently in their territory and could follow her when she departs for feeding grounds. In either case these data suggest that adult cowbirds associate with juveniles, in some cases their own offspring, and that offspring may learn to function as cowbirds in part from this association.

  7. Juvenile hyaline fibromatosis. Radiological diagnosis

    International Nuclear Information System (INIS)

    Fuentes, R.; Sar, V.; Cabrera, J.J.; Diaz, L.; Hernandez, B.; Valeron, P.; Baez, O.; Rodriguez, M.

    1993-01-01

    Juvenile hyaline fibromatosis (JHF) is a rare disorder of unknown etiology, very few cases of which have been reported in the literature. It presents similarities to other fibromatosys, but has its particular radiological features which differentiate it from them. The clinical findings consist of several, slow growing, subcutaneous nodules, flexion contractures of the joints which can lead to disability, gingival hypertrophy and muscular atrophy. The suspected radiological diagnosis is confirmed by electron microscopy study of the nodules, although light microscopy can also reveal suggestive images. Author (9 refs.)

  8. Juvenile eye growth, when completed?

    DEFF Research Database (Denmark)

    Fledelius, Hans C; Christensen, Anders S; Fledelius, Christian

    2014-01-01

    PURPOSE: To test Sorsby's classical statement of axial eye growth as completed at the age of 13 years, with a view also to differentiating between basic eye growth and juvenile elongation associated with eventual refractive change towards myopia. METHODS: (i) A total of 160 healthy eyes close...... about age 13 as general limit found support from the cross-sectional data, which suggested stable emmetropic eye size from about 11-12 years, with an average apparently outgrown male emmetropic value of 23.5 mm versus females' 22.9 mm. The longitudinal data, however, showed emmetropic growth also beyond...

  9. Juvenile ossifying fibroma: Psammamatoid variant

    Directory of Open Access Journals (Sweden)

    Shivani Aggarwal

    2012-01-01

    Full Text Available Juvenile ossifying fibroma is a rare fibro-osseous lesion containing variable amount of calcified masses, which resembles bone or cementum within a fibrocellular connective tissue stroma. It has variable clinical behavior, highly aggressive in nature including invasion and destruction of adjacent anatomic structures with a strong tendency to recur. We reported a 28-year-old female patient with a growth in the upper left vestibule region extending from canine to molar region with clinical, histopathological, and radiological features are presented. Surgical management was done, and regular follow-up was advised.

  10. THE STUDY OF FEATURES OF GUILT OF JUVENILE OFFENDERS IN THE CONTEXT OF JUVENILE JUSTICE

    Directory of Open Access Journals (Sweden)

    Natalija Vladimirovna Galkina

    2015-08-01

    Full Text Available The article is devoted to the results of empirical studies of the experiences of guilt of juvenile offenders in the context of juvenile justice where a minor appears as the subject of legal relations. Restorative approach of juvenile justice is based on an admission of guilt to the victim. In connection with it, the research of features of the guilt of minors who have committed an offence and the conditions for the development of the subjectivity will enhance understanding of the possibilities of restorative juvenile justice system in the prevention of juvenile delinquency.Thus, the results of empirical research presented in the article are important for determining of the psychological bases of realization of rehabilitation programs in the context of juvenile justice. In particular, the results are important for the organization and conduct of psychological work to overcome the psychological barriers in the behavior of juveniles having inherently maladaptive guilt and destructive psychological defense mechanisms.

  11. Juveniles' Motivations for Remaining in Prostitution

    Science.gov (United States)

    Hwang, Shu-Ling; Bedford, Olwen

    2004-01-01

    Qualitative data from in-depth interviews were collected in 1990-1991, 1992, and 2000 with 49 prostituted juveniles remanded to two rehabilitation centers in Taiwan. These data are analyzed to explore Taiwanese prostituted juveniles' feelings about themselves and their work, their motivations for remaining in prostitution, and their difficulties…

  12. Moral development of solo juvenile sex offenders

    NARCIS (Netherlands)

    van Vugt, E.; Stams, G.J.; Dekovic, M.; Brugman, D.; Rutten, E.; Hendriks, J.

    2008-01-01

    This study compared the moral development of solo juvenile male sex offenders (n = 20) and juvenile male non-offenders (n = 76), aged 13-19 years, from lower socioeconomic and educational backgrounds. The Moral Orientation Measure (MOM) was used to assess punishment- and victim-based moral

  13. Juvenile Obesity, Physical Activity, and Lifestyle Changes.

    Science.gov (United States)

    Bar-Or, Oded

    2000-01-01

    Because many obese children become obese adults, the recent rapid increase in juvenile obesity poses a major public health challenge. Enhanced physical activity is a cornerstone in a multidisciplinary approach to preventing and treating juvenile obesity. Giving exercise recommendations focused for obese youth is critical. Cutting down on sedentary…

  14. Juvenile dispersal in Calomys venustus (Muridae: Sigmodontinae)

    Science.gov (United States)

    Priotto, José; Steinmann, Andrea; Provensal, Cecilia; Polop, Jaime

    2004-05-01

    Both spacing behaviour and dispersal movement are viewed as hierarchical processes in which the effects may be expressed at spatial scale. This research was carried out to examine the hypothesis that the presence of parents promotes the dispersal of juveniles from their natal nest and their father or mother home-range, in Calomys venustus.The study was carried out in four 0.25 ha fences (two controls and two experimentals), in a natural pasture. This study had two periods: Father Removal (FR) (August and December 1997; year one) and Mother Removal (MR) (August 1998 and January 1999; year two). For the FR treatment fathers were removed after juveniles were born, but in the MR treatment mothers were removed after the juveniles were weaned. The effect of parents on the dispersal distance of juveniles was analysed with respect to their natal nest and their mother and father home-range. Dispersal distance from the nest of C. venustus was independent of either male or female parent. Juveniles were more dispersing in relation to the centre of activity of their mothers than to that of their fathers, and females were more dispersing than males. Female juveniles overlap their home-range with their parents less than male juveniles do. The differences observed between female and male juveniles would be related to their different sexual maturation times, as well as to the female territoriality.

  15. Using the Juvenile Justice Poster. Teaching Strategy.

    Science.gov (United States)

    Update on Law-Related Education, 2000

    2000-01-01

    Presents a lesson that can help students review and summarize what they have learned about the juvenile justice system. Explains that the students discuss how the juvenile justice system can be improved and conduct a survey on how it might be changed in the future. Provides a copy of the survey and directions. (CMK)

  16. Intelligence Score Profiles of Female Juvenile Offenders

    Science.gov (United States)

    Werner, Shelby Spare; Hart, Kathleen J.; Ficke, Susan L.

    2016-01-01

    Previous studies have found that male juvenile offenders typically obtain low scores on measures of intelligence, often with a pattern of higher scores on measures of nonverbal relative to verbal tasks. The research on the intelligence performance of female juvenile offenders is limited. This study explored the Wechsler Intelligence Scale for…

  17. Reformations in Zimbabwe's juvenile justice system | Ruparanganda ...

    African Journals Online (AJOL)

    Children in conflict with the law are often stigmatized and shunned by society as they are perceived as a threat to society. Historically, Zimbabwe's juvenile justice system has been retributive and focused on punishing the juvenile offender. As a result, it has been criticised from a number of viewpoints, including the need to ...

  18. Transcriptomic immune response of Tenebrio molitor pupae to parasitization by Scleroderma guani.

    Directory of Open Access Journals (Sweden)

    Jia-Ying Zhu

    Full Text Available BACKGROUND: Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. METHODOLOGY/PRINCIPAL FINDINGS: In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26% showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. CONCLUSIONS/SIGNIFICANCE: obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular

  19. Transcriptomic immune response of Tenebrio molitor pupae to parasitization by Scleroderma guani.

    Science.gov (United States)

    Zhu, Jia-Ying; Yang, Pu; Zhang, Zhong; Wu, Guo-Xing; Yang, Bin

    2013-01-01

    Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE) analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26%) showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular understanding of the host-parasitoid interaction.

  20. Transcriptomic Immune Response of Tenebrio molitor Pupae to Parasitization by Scleroderma guani

    Science.gov (United States)

    Zhu, Jia-Ying; Yang, Pu; Zhang, Zhong; Wu, Guo-Xing; Yang, Bin

    2013-01-01

    Background Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE) analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. Methodology/Principal Findings In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26%) showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. Conclusions/Significance obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular understanding of the host

  1. Quantitative nanohistological investigation of scleroderma: an atomic force microscopy-based approach to disease characterization

    Directory of Open Access Journals (Sweden)

    Strange AP

    2017-01-01

    Full Text Available Adam P Strange,1 Sebastian Aguayo,1 Tarek Ahmed,1 Nicola Mordan,1 Richard Stratton,2 Stephen R Porter,3 Susan Parekh,4 Laurent Bozec1 1Department of Biomaterials and Tissue Engineering, UCL Eastman Dental Institute, 2Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, UCL Medical School, 3UCL Eastman Dental Institute, 4Department of Pediatrics, UCL Eastman Dental Institute, London, UK Abstract: Scleroderma (or systemic sclerosis, SSc is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. A more detailed analysis of SSc may provide better future treatment options and information of disease progression. Recently, the histological stain picrosirius red showing collagen register has been combined with atomic force microscopy (AFM to study SSc. Skin from healthy individuals and SSc patients was biopsied, stained and studied using AFM. By investigating the crosslinking of collagen at a smaller hierarchical stage, the effects of SSc were more pronounced. Changes in morphology and Young’s elastic modulus were observed and quantified; giving rise to a novel technique, we have termed “quantitative nanohistology”. An increase in nanoscale stiffness in the collagen for SSc compared with healthy individuals was seen by a significant increase in the Young’s modulus profile for the collagen. These markers of stiffer collagen in SSc are similar to the symptoms experienced by patients, giving additional hope that in the future, nanohistology using AFM can be readily applied as a clinical tool, providing detailed information of the state of collagen. Keywords: rheumatology, adjunct diagnosis, picrosirius red, collagen, nanohistology

  2. Is occupational exposure to solvents associated with an increased risk for developing systemic scleroderma?

    Directory of Open Access Journals (Sweden)

    Drexler Hans

    2006-07-01

    Full Text Available Abstract Background Our study was aimed to investigate in a German collective if there are any hints for an increased occupational or environmental risk to develop systemic sclerosis, especially, focussing on work-related exposure to solvents. Moreover, we tried to evaluate the feasibility of a sampling method addressing support groups. Methods A standardised questionnaire was published in two journals subscribed by members of two different support groups and all members were asked to complete the questionnaire and to return it anonymously. The subjects were not informed on the scientific hypotheses, nor did they know who of them belonged to the case group (scleroderma or to the control group (multiple sclerosis. Results 175 questionnaires could be included in the statistical analysis. As expected, a female predominance was in our collective. In the male subpopulation, the occupational exposure to solvents was higher in the case group than in the control-group (70% versus 45.8%. Based only on the male subgroup, a tendency for an association between occupational exposure to solvents and the risk to develop systemic sclerosis was found. Conclusion According to our experience in this case-control-study exposure misclassification, qualitative or quantitative, was an eminent problem. Within such a setting, it is generally very difficult to establish an exact dose-response relationship due to incomplete, imprecise or missing data concerning duration of exposure, frequency of use and kind of solvent. Additionally, a well-known problem in studies based on self-reported questionnaires is the so-called volunteer bias. Unfortunately, but similar to other studies assessing epidemiologic factors in such a rare disease, our study was of limited power, especially in the subgroups defined by gender.

  3. Valuation of scleroderma and psoriatic arthritis health states by the general public

    Directory of Open Access Journals (Sweden)

    Hays Ron D

    2010-10-01

    Full Text Available Abstract Objective Psoriatic arthritis (PsA and scleroderma (SSc are chronic rheumatic disorders with detrimental effects on health-related quality of life. Our objective was to assess health values (utilities from the general public for health states common to people with PsA and SSc for economic evaluations. Methods Adult subjects from the general population in a Midwestern city (N = 218 completed the SF-12 Health Survey and computer-assisted 0-100 rating scale (RS, time trade-off (TTO, range: 0.0-1.0 and standard gamble (SG, range: 0.0-1.0 utility assessments for several hypothetical PsA and SSc health states. Results Subjects included 135 (62% females, 143 (66% Caucasians, and 62 (28% African-Americans. The mean (SD scores for the SF-12 Physical Component Summary scale were 52.9 (8.3 and for the SF-12 Mental Component Summary scale were 49.0 (9.1, close to population norms. The mean RS, TTO, and SG scores for PsA health states varied with severity, ranging from 20.2 to 63.7 (14.4-20.3 for the RS 0.29 to 0.78 (0.24-0.31 for the TTO, and 0.48 to 0.82 (0.24-0.34 for the SG. The mean RS, TTO, and SG scores for SSc health states were 25.3-69.7 (15.2-16.3 for the RS, 0.36-0.80 (0.25-0.31 for the TTO, and 0.50-0.81 (0.26-0.32 for the SG, depending on disease severity. Conclusion Health utilities for PsA and SSc health states as assessed from the general public reflect the severity of the diseases. These descriptive findings could have implications regarding comparative effectiveness research for tests and treatments for PsA and SSc.

  4. Scleroderma dermal microvascular endothelial cells exhibit defective response to pro-angiogenic chemokines

    Science.gov (United States)

    Rabquer, Bradley J.; Ohara, Ray A.; Stinson, William A.; Campbell, Phillip L.; Amin, M. Asif; Balogh, Beatrix; Zakhem, George; Renauer, Paul A.; Lozier, Ann; Arasu, Eshwar; Haines, G. Kenneth; Kahaleh, Bashar; Schiopu, Elena; Khanna, Dinesh; Koch, Alisa E.

    2016-01-01

    Objectives. Angiogenesis plays a critical role in SSc (scleroderma). The aim of this study was to examine the expression of growth-regulated protein-γ (Gro-γ/CXCL3), granulocyte chemotactic protein 2 (GCP-2/CXCL6) and their receptor CXCR2 in endothelial cells (ECs) isolated from SSc skin and determine whether these cells mount an angiogenic response towards pro-angiogenic chemokines. The downstream signalling pathways as well as the pro-angiogenic transcription factor inhibitor of DNA-binding protein 1 (Id-1) were also examined. Methods. Skin biopsies were obtained from patients with dcSSc. ECs were isolated via magnetic positive selection. Angiogenesis was measured by EC chemotaxis assay. Results. Gro-γ/CXCL3 and GCP-2/CXCL6 were minimally expressed in both skin types but elevated in SSc serum. Pro-angiogenic chemokine mRNA was greater in SSc ECs than in normal ECs. SSc ECs did not migrate to vascular endothelial growth factor (VEGF), Gro-γ/CXCL3, GCP-2/CXCL6 or CXCL16. The signalling pathways stimulated by these chemokines were also dysregulated. Id-1 mRNA in SSc ECs was lower compared with normal ECs, and overexpression of Id-1 in SSc ECs increased their ability to migrate towards VEGF and CXCL16. Conclusion. Our results show that SSc ECs are unable to respond to pro-angiogenic chemokines despite their increased expression in serum and ECs. This might be due to the differences in the signalling pathways activated by these chemokines in normal vs SSc ECs. In addition, the lower expression of Id-1 also decreases the angiogenic response. The inability of pro-angiogenic chemokines to promote EC migration provides an additional mechanism for the impaired angiogenesis that characterizes SSc. PMID:26705326

  5. Orofacial Manifestations and Temporomandibular Disorders of Systemic Scleroderma: An Observational Study

    Directory of Open Access Journals (Sweden)

    Vito Crincoli

    2016-07-01

    Full Text Available Scleroderma is a disorder involving oral and facial tissues, with skin hardening, thin lips, deep wrinkles, xerostomia, tongue rigidity, and microstomia. The aim of this study was to investigate the prevalence of oral manifestations and temporomandibular disorders (TMD in Systemic Sclerosis (SSc patients compared with healthy people. Eighty patients (6 men, 74 women fulfilling ACR/EULAR SSc Criteria were enrolled. A randomly selected group of 80 patients, matched by sex and age served as control group. The examination for TMD signs and symptoms was based on the standardized Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD through a questionnaire and clinical examination. SSc patients complained more frequently (78.8% of oral symptoms (Xerostomia, dysgeusia, dysphagia and stomatodynia than controls (28.7% (χ2 = 40.23 p = 0.001. TMD symptoms (muscle pain on chewing, difficulty in mouth opening, headaches were complained by 92.5% of SSc patients and by 76.2% of controls (χ2 = 8.012 p = 0.005. At the clinical examination, 85% of SSc patients showed restricted opening versus 20.0% of controls (χ2 = 67.77 p = 0.001, 81.2% of SSc showed reduced right lateral excursion versus 50% of controls (χ2 = 17.316 p = 0.001; 73.8% of SSc showed limited left lateral excursion versus 53.8% of controls (χ2 = 6.924 p = 0.009; and 73.8% of SSc had narrow protrusion versus 56.2% of controls (χ2 = 5.385 p = 0.02.

  6. Do juveniles bully more than young offenders?

    Science.gov (United States)

    Ireland, Jane L

    2002-04-01

    This study compares bullying behaviour among juvenile and young offenders and incorporates two different methods to measure bullying. Ninety-five male juvenile and 196 male young offenders completed two questionnaires, one that measured bullying directly and one that measured behaviours indicative of "being bullied" or of "bullying others". Juveniles perceived a higher extent of bullying than young offenders. Juveniles reported significantly more physical, psychological or verbal and overall direct forms of bullying behaviour than young offenders. A number of differences were found between juveniles and young offenders with regard to the types of prisoners likely to become victims, who they would advise a victim to speak to and how bullying could be prevented. The results are discussed in relation to developmental theories of aggression and how bullying behaviour can be defined and measured among prisoners. Copyright 2002 The Association for Professionals in Services for Adolescents. Published by Elsevier Science Ltd. All rights reserved.

  7. Trigeminocardiac reflex during endoscopic juvenile nasopharyngeal angiofibroma surgery: an appraisal.

    Science.gov (United States)

    Sharma, Shilpee Bhatia; Janakiram, Trichy Narayanan; Baxi, Hina; Chinnasamy, Balamurugan

    2017-07-01

    Juvenile nasopharyngeal angiofibroma is a locally aggressive benign tumour which has propensity to erode the skull base. The tumour spreads along the pathways of least resistance and is in close proximity to the extracranial part of trigeminal nerve. Advancements in expanded approaches for endoscopic excision of tumours in infratemporal fossa and pterygopalatine fossa increase the vulnerability for the trigeminocardiac reflex. The manipulation of nerve and its branches during tumour dissection can lead to sensory stimulation and thus inciting the reflex. The aim of our study is to report the occurrence of trigeminocardiac reflex in endoscopic excision of juvenile nasopharyngeal angiofibroma. To describe the occurence of trigeminocardiac reflex during endoscopic endonasal excision of juvenile nasopharyngeal angiofibroma. We studied the occurrence of TCR in 15 patients (out of 242 primary cases and 52 revision cases) operated for endoscopic endonasal excision of JNA. The drop in mean arterial blood pressure and heart rate were observed and measured. To the best of our knowledge of English literature, this is the first case series reporting TCR as complication in endoscopic excision of JNA. occurence of this reflex has been mentioned in various occular, maxillofacial surgeries but its occurence during endoscopic excision of JNA has never been reported before. Manifestation of trigeminocardiac reflex during surgery can alter the course of the surgery and is a potential threat to life. It is essential for the anesthetist and surgeons to be familiar with the presentations, preventive measures and management protocols.

  8. Family transitions and juvenile delinquency.

    Science.gov (United States)

    Schroeder, Ryan D; Osgood, Aurea K; Oghia, Michael J

    2010-01-01

    There is a large body of research that shows children from non-intact homes show higher rates of juvenile delinquency than children from intact homes, partially due to weaker parental control and supervision in non-intact homes. What has not been adequately addressed in the research is the influence of changes in family structure among individual adolescents over time on delinquent offending. Using the first and third waves of the National Youth Study, we assess the effect of family structure changes on changes in delinquent offending between waves through the intermediate process of changes in family time and parental attachment. Although prior research has documented adolescents in broken homes are more delinquent than youth in intact homes, the process of family dissolution is not associated with concurrent increases in offending. In contrast, family formation through marriage or cohabitation is associated with simultaneous increases in offending. Changes in family time and parental attachment account for a portion of the family formation effect on delinquency, and prior parental attachment and juvenile offending significantly condition the effect of family formation on offending.

  9. Juvenile Gaucher disease simulating osteomyelitis

    International Nuclear Information System (INIS)

    Miller, J.H.; Ortega, J.A.; Heisel, M.A.

    1981-01-01

    A case in which several imaging procedures suggested juvenile Gaucher disease in a child who presented with symptomatology of osteomyelitis is discussed. The 20-month girl was given a Technetium-99m radionuclide skeletal examination which revealed intense uptake of tracer agents along the shaft of the right femur. It was also found that the liver and spleen were dramatically Ga-67 avid. The bone pain symptomatology suggested an osteomyelitis of the femur, but skeletal scintigraphy with Tc-99m-labeled bone tracer demonstrated photopenic areas involving the femur, suggesting that the bone pain may have been due to marrow packed with Gaucher cells. This overexpansion of the marrow may lead to microfractures with remodeling seen radiographically as periosteal new bone and scintigraphically as increased periosteal deposition of tracer agent. The radiogallium study was useful to exclude an underlying osteomyelitis in the involved femurs. Although juvenile Gaucher disease is unusual, it should be considered in any child who presents with the constellation of hepatosplenomegaly and bone pain simulating osteomyelitis

  10. Juvenile Gaucher disease simulating osteomyelitis

    Energy Technology Data Exchange (ETDEWEB)

    Miller, J.H.; Ortega, J.A.; Heisel, M.A.

    1981-10-01

    A case in which several imaging procedures suggested juvenile Gaucher disease in a child who presented with symptomatology of osteomyelitis is discussed. The 20-month girl was given a Technetium-99m radionuclide skeletal examination which revealed intense uptake of tracer agents along the shaft of the right femur. It was also found that the liver and spleen were dramatically Ga-67 avid. The bone pain symptomatology suggested an osteomyelitis of the femur, but skeletal scintigraphy with Tc-99m-labeled bone tracer demonstrated photopenic areas involving the femur, suggesting that the bone pain may have been due to marrow packed with Gaucher cells. This overexpansion of the marrow may lead to microfractures with remodeling seen radiographically as periosteal new bone and scintigraphically as increased periosteal deposition of tracer agent. The radiogallium study was useful to exclude an underlying osteomyelitis in the involved femurs. Although juvenile Gaucher disease is unusual, it should be considered in any child who presents with the constellation of hepatosplenomegaly and bone pain simulating osteomyelitis.

  11. Atherosclerosis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2012-01-01

    Full Text Available Atherosclerosis is a chronic inflammatory disease of the arteries. Clinical consequences of the atherosclerotic process occur in the adult population, however atherosclerotic process begins in childhood. The classic risk factors for atherosclerosis include obesity, dyslipidaemia, age, gender or family history. In recent years, attention has been drawn to the similarity between atherosclerotic inflammatory processes and inflammatory changes in the course of systemic connective tissue disease, in particular systemic lupus etythematosus (SLE or rheumatoid arthritis (RA. There is also observed the similarity of the pathogenetic background of development of atherosclerosis and juvenile idiopathic arthritis (JIA. Elevated levels of pro-inflammatory cytokines are observed in the course of juvenile idiopathic arthritis. Also homocysteine concentrations, which may play a significant role in the development of atherosclerotic lesions, are observed higher in patients with JIA. Some studies revealed higher carotid intima-media thickness (IMT index values in children with JIA. In view of the fact that atherosclerotic process begins as early as in childhood, the introduction of appropriate preventive measures in children is a matter of utmost importance.

  12. Characterization of juvenile maritime pine (Pinus pinaster Ait.) ectomycorrhizal fungal community using morphotyping, direct sequencing and fruitbodies sampling.

    Science.gov (United States)

    Pestaña Nieto, Montserrat; Santolamazza Carbone, Serena

    2009-02-01

    Using ectomycorrhizal root tip morphotyping (anatomical and morphological identification), molecular analysis (internal transcribed spacer region amplification and sequencing), and fruitbody sampling, we assessed diversity and composition of the ectomycorrhizal fungal community colonizing juvenile Pinus pinaster Ait. under natural conditions in NW Spain. Overall, we found 15 Basidiomycetes and two Ascomycetes. Members of the family Thelephoraceae represented up to 59.4% of the samples. The most frequent species was Tomentella sublilacina followed by Thelephora terrestris, Russula drimeia, Suillus bovinus, and Paxillus involutus, while the less frequent were Pseudotomentella tristis, Lactarius subdulcis, Russula ochroleuca, and Entoloma conferendum. From October 2007 to June 2008, we sampled 208 sporocarps belonging to seven genera and nine species: Thelephora terrestris, Paxillus involutus, Suillus bovinus, Xerocomus badius, Scleroderma verrucosum, Amanita gemmata, A. rubescens, Amanita sp., and Russula sp. The species belonging to the genus Amanita, X. badius and S. verrucosum were not found on root samples. By comparing our results with a bibliographic review of papers published from 1922 to 2006, we found five genera and six species which have not been previously reported in symbiosis with P. pinaster. This is the first time that the diversity of the ectomycorrhizal fungal community associated with P. pinaster was investigated using molecular techniques. Considering that only 38% of the genera found by sequencing were found as fruitbodies, we conclude that integrating morphotyping and sporocarps surveys with molecular analysis of ectomycorrhizas is important to documenting the ectomycorrhizal fungus community.

  13. Angiofibroma juvenil nasofaríngeo Juvenile nasopharyngeal angiofibroma

    Directory of Open Access Journals (Sweden)

    Juan Gualberto Lescaille Torres

    2012-06-01

    Full Text Available Se presenta el caso de un angiofibroma juvenil nasofaríngeo, en un paciente del consultorio médico No. 9, que pertenece al Policlínico Integral Docente "Carlos Manuel Portuondo" de Marianao. Se reconoce la importancia de realizar una historia clínica detallada, así como un minucioso examen físico, por el médico de familia y el otorrinolaringólogo del área de salud, que incluyó la rinoscopia posterior, para poder llegar al diagnóstico de esa patología, y realizar la extirpación precoz del angiofibroma, mediante el proceder quirúrgico. Se concluyó que el diagnóstico clínico se correspondió con el histopatológico, y que la conducta quirúrgica temprana es resolutiva en la afección.It is presented a case of juvenile nasopharyngeal angiofibroma in a patient with this pathology, from the clicial practice No. 9, in Comprehensive Teaching Polyclinic "Carlos Manuel Portuondo" in Marianao. It recognizes the importance of a thorough clinical history and careful physical examination by the family physician and the otolaryngologist in this health area, including a posterior rhinoscopy, to diagnose this disease and to achieve early removal of the angiofibroma, by a surgical procedure. It was concluded that the clinical diagnosis corresponded to the histopathological diagnosis, and that early surgical treatment is resolute in this condition.

  14. Vegetative propagation of mature and juvenile northern red oak

    Science.gov (United States)

    James J. Zaczek; K. C. Steiner; C. W., Jr. Heuser

    1993-01-01

    Rooting trials were established to evaluate rooting success of cuttings from mature and juvenile, grafted and ungrafted northern red oak (NRO). Buds from 4 mature NRO ortets and juvenile seedlings were grafted onto juvenile and mature rootstock. Cuttings were collected from the grafts and from juvenile and mature shoots developed in situ and...

  15. Juvenile recurrent parotitis in children: diagnosis and treatment using sialography.

    Science.gov (United States)

    Narsimha Rao, Vanga V; Putta Buddi, Jai Shankar Homberhali; Kurthukoti, Ameet J

    2014-01-01

    Juvenile recurrent parotitis (JRP) is a nonobstructive, nonsuppurative parotid inflammation in young children. Causative factors are many such as allergy, infection, local autoimmune manifestations, and genetic inheritance have been suggested, but none have been proved. Parotid sialography is a hallmark in the diagnosis of JRP but newer modalities such as ultrasonography, computed tomography and magnetic resonance imaging-sialography are noninvasive investigative techniques. Recurrent attacks are often managed conservatively. Here we report a case of a 5-year-old child with JRP. Sailography can be used as both diagnostic and therapeutic modality.

  16. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Westergaard, Jytte; Stoltze, Kaj

    2006-01-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile...... idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases....

  17. Chromosome 16 microdeletion in a patient with juvenile neuronal ceroid lipofuscinosis (Batten disease)

    NARCIS (Netherlands)

    Taschner, P. E.; de Vos, N.; Thompson, A. D.; Callen, D. F.; Doggett, N.; Mole, S. E.; Dooley, T. P.; Barth, P. G.; Breuning, M. H.

    1995-01-01

    The gene that is involved in juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease--CLN3--has been localized to 16p12, and the mutation shows a strong association with alleles of microsatellite markers D16S298, D16S299, and D16S288. Recently, haplotype analysis of a Batten patient from a

  18. Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Ødum, Niels; Morling, Niels; Friis, J

    1986-01-01

    Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in controls...

  19. Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Ødum, Niels; Morling, Niels; Friis, J

    1986-01-01

    Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in contro...

  20. Perineural inflammation in morphea (localized scleroderma): systematic characterization of a poorly recognized but potentially useful histopathological feature.

    Science.gov (United States)

    Dhaliwal, Catharine A; MacKenzie, Andrew I; Biswas, Asok

    2014-01-01

    The association between morphea and perineural inflammation has been reported sporadically but never studied systematically. To assess the prevalence and nature of perineural inflammation in various clinicopathologic stages of morphea and a cohort of other inflammatory dermatoses, 80 morphea and 36 control skin biopsies were studied using hematoxylin/eosin and S100 stains. Perineural inflammation was semiquantitatively analyzed (scored), which along with the pattern (concentric vs. marginal) and cellular composition was compared in the two groups. Perineural inflammation was identified in 84% and 61% of morphea and control cases, respectively. Examination of only routinely stained sections could still detect this feature in 58% of morphea and 33% of control biopsies. Mean perineural inflammation score in morphea (0.65) was significantly higher than in the control group (0.23) (p morphea cases. Although perineural inflammation is common in morphea, it is not unusual to find this feature in other inflammatory conditions. Nevertheless, perineural inflammation can serve as an important diagnostic adjunct in difficult cases of morphea if one considers its greater intensity, predominantly concentric pattern and the tendency to show plasma cell neurotropism. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Juvenile fibromyalgia syndrome. Interdisciplinary treatment

    Directory of Open Access Journals (Sweden)

    Hanna Siuchnińska

    2014-11-01

    Full Text Available Fibromyalgia syndrome (FM belongs to soft tissue pain syndromes of unknown cause, also referred to as “soft tissue rheumatism”. It is characterized by chronic widespread pain as well as additional symptoms such as fatigue, sleep and mood disturbance and cognitive problems. There is more and more data showing that this condition may start at a young age or even in childhood, adversely affecting development processes and resulting in dysfunctional social and family relationships. Because of the multifaceted character of fibromyalgia the efficient treatment of this disorder can be difficult and requires comprehensive care. This work reviews most recommended procedures used in integrated treatment programmes for juvenile fibromyalgia syndrome (JFM.

  2. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  3. Law & psychiatry: punishing juveniles who kill.

    Science.gov (United States)

    Appelbaum, Paul S

    2012-10-01

    Punishment of juvenile murderers forces policy makers to weigh the developmental immaturity of adolescents against the heinousness of their crimes. The U.S. Supreme Court has progressively limited the severity of punishments that can be imposed on juveniles, holding that their impulsivity, susceptibility to peer pressure, and more fluid character render them less culpable for their actions. Having eliminated the death penalty as a punishment, the Court recently struck down mandatory life sentences without prospect of parole. The decision is interesting for its emphasis on rehabilitation, opening the door to further restrictions on punitive sentences for juveniles-and perhaps for adults too.

  4. Spatial distribution of juvenile and adult female Tanner crabs (Chionoecetes bairdi) in a glacial fjord ecosystem: Implications for recruitment processes

    Science.gov (United States)

    Nielsen, J.K.; Taggart, S. James; Shirley, Thomas C.; Mondragon, Jennifer

    2007-01-01

    A systematic pot survey in Glacier Bay, Alaska, was conducted to characterize the spatial distribution of juvenile and adult female Tanner crabs, and their association with depth and temperature. The information was used to infer important recruitment processes for Tanner crabs in glaciated ecosystems. High-catch areas for juvenile and adult female Tanner crabs were identified using local autocorrelation statistics. Spatial segregation by size class corresponded to features in the glacial landscape: high-catch areas for juveniles were located at the distal ends of two narrow glacial fjords, and high-catch areas for adults were located in the open waters of the central Bay. Juvenile female Tanner crabs were found at nearly all sampled depths (15–439 m) and temperatures (4–8°C), but the biggest catches were at depths <150 m where adults were scarce. Because adults may prey on or compete with juveniles, the distribution of juveniles could be influenced by the distribution of adults. Areas where adults or predators are scarce, such as glacially influenced fjords, could serve as refuges for juvenile Tanner crabs.

  5. Conceptualizing juvenile prostitution as child maltreatment: findings from the National Juvenile Prostitution Study.

    Science.gov (United States)

    Mitchell, Kimberly J; Finkelhor, David; Wolak, Janis

    2010-02-01

    Two studies were conducted to identify the incidence (Study 1) and characteristics (Study 2) of juvenile prostitution cases known to law enforcement agencies in the United States. Study 1 revealed a national estimate of 1,450 arrests or detentions (95% confidence interval [CI]: 1,287-1,614) in cases involving juvenile prostitution during a 1-year period. In Study 2, exploratory data were collected from a subsample of 138 cases from police records in 2005. The cases are broadly categorized into three main types: (a) third-party exploiters, (b) solo prostitution, and (c) conventional child sexual abuse (CSA) with payment. Cases were classified into three initial categories based on police orientation toward the juvenile: (a) juveniles as victims (53%), (b) juveniles as delinquents (31%), and (c) juvenile as both victims and delinquents (16%). When examining the status of the juveniles by case type, the authors found that all the juveniles in CSA with payment cases were treated as victims, 66% in third-party exploiters cases, and 11% in solo cases. Findings indicate law enforcement responses to juvenile prostitution are influential in determining whether such youth are viewed as victims of commercial sexual exploitation or as delinquents.

  6. The semantic sphere of juvenile offenders

    Directory of Open Access Journals (Sweden)

    Oshevsky D.S.

    2017-01-01

    Full Text Available The article presents the results of a preliminary empirical study aimed to identify features of the semantic sphere of adolescents who have committed illegal, including aggressive acts. The study included 50 male juveniles aged of 16 - 17 years. The first group consisted of adolescents convicted of aggressive and violent crimes; the second – of property socially dangerous acts (SDA. It is shown that evaluation of such adolescents is generally categorical and polar, the semantic field is subdifferentiable, less hierarchic, and has not enough realistic structure of meanings. Developed structure of motives and meanings is the basis of voluntary regulation of socially significant behavior. Thus, assessing the semantic sphere of juvenile offenders we can highlight its characteristics as risk factors of unlawful behavior, as well as the resource side, that will contribute to addressing issues of prevention and correction of unlawful behavior. Key words: juvenile offenders, semantic field of juvenile offenders, unlawful behavior.

  7. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... disease to fight microbial invaders and facilitate tissue repair. Normally, the body stops the inflammatory response after healing is complete to prevent damage to its own cells and tissues. In people with juvenile idiopathic arthritis , the inflammatory ...

  8. Juvenile Salmonid Metrics - Ocean Survival of Salmonids

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — A study to evaluate the role of changing ocean conditions on growth and survival of juvenile salmon from the Columbia River basin as they enter the Columbia River...

  9. Juvenile Pacific Salmon in Puget Sound

    National Research Council Canada - National Science Library

    Fresh, Kurt L

    2006-01-01

    Puget sound salmon (genus Oncorhynchus) spawn in freshwater and feed, grow and mature in marine waters, During their transition from freshwater to saltwater, juvenile salmon occupy nearshore ecosystems in Puget Sound...

  10. The Impact of Banning Juvenile Gun Possession.

    OpenAIRE

    Marvell, Thomas B

    2001-01-01

    A 1994 federal law bans possession of handguns by persons under 18 years of age. Also in 1994, 11 states passed their own juvenile gun possession bans. Eighteen states had previously passed bans, 15 of them between 1975 and 1993. These laws were intended to reduce homicides, but arguments can be made that they have no effect on or that they even increase the homicide rate. This paper estimates the laws' impacts on various crime measures, primarily juvenile gun homicide victimizations and suic...

  11. Juvenile Delinquency in Romania: The Indirect Result of the Transition Process

    Directory of Open Access Journals (Sweden)

    Andrea Fabian

    2010-11-01

    Full Text Available The general purpose of the research is to present the evolution of juvenile delinquency in Romania after 1989, with some of its social and psychological aspects. We use a comparative perspective: the general, Romanian framework will be compared with local situation occurred in Cluj county.The presentation is based on an extensive study of 420 delinquent juveniles, whose data on family situation, education, age, etc were analyzed and the youngsters were also tested with psychological tests. Looking at the data of the psychological tests (Nowicki & Strickland's Internal-External Control Scale for Children, McGuire & Priestley's Testing Your Reaction, Zuckerman-Kuhlman's Personality Questionnaire and Tucker's Inmate Dilemma Test and the descriptors of the social situation of juvenile delinquents, it looks like personality factors are associated with low school performance and school drop-out, as important determinants of delinquency. An integrative model was tested, to analyze the social and cognitive determinants of delinquency.

  12. Results of RNV studies and /sup 201/Tl myocardial scintigraphy in patients with progressive systemic scleroderma (PSS)

    Energy Technology Data Exchange (ETDEWEB)

    Ammari, B.; Hotze, A.; Gruenwald, F.; Biersack, H.J.; Biltz, H.; Kuester, W.

    1988-12-01

    Prognosis of progressive systemic scleroderma (PSS) depends directly on the extent of visceral organ involvement, and in particular, on the cardiac, renal and pulmonary appearance. Therapeutic approaches therefore require periodic followup with non-invasive methods to evaluate the actual course of disease and the success of therapy. Tl-201 scintigraphy showed pathologic heart abnormalities in 47% and RNV in 23% of the PSS patients. Our results and published data reveal the sensitivity of both myocardial scans and RNV in the evaluation of patients with PSS. Other current methods for the diagnosis of heart diseases, however, such as echocardiography should also be performed. In patients with PSS coronary angiography, however, usually shows normal coronary vessels.

  13. Reduction of regulatory T cells in skin lesions but not in peripheral blood of patients with systemic scleroderma.

    Science.gov (United States)

    Klein, S; Kretz, C C; Ruland, V; Stumpf, C; Haust, M; Hartschuh, W; Hartmann, M; Enk, A; Suri-Payer, E; Oberle, N; Krammer, P H; Kuhn, A

    2011-08-01

    To determine the frequency and suppressive capacity of regulatory T cells (T(reg)) and their association with clinical parameters in patients with systemic scleroderma (SSc). Peripheral blood from 25 patients with SSc, 15 patients with localised scleroderma (LS) and 29 healthy controls (HC) was studied. Analysis of CD4(+) forkhead box P3 (Foxp3)(+) and CD4(+)CD25(++)Foxp3(+) T(reg) subpopulations was carried out by flow cytometry and cell proliferation was quantified by (3)H-thymidine incorporation. Quantitative analysis of T(reg) was further performed in skin biopsies from 17 patients with SSc and 21 patients with LS using anti-CD4 and anti-Foxp3 monoclonal antibodies for immunohistochemistry. The frequency of CD4(+)Foxp3(+) and CD4(+)CD25(++)Foxp3(+) T(reg) in peripheral blood from patients with SSc was not significantly different from that of patients with LS or HC. The suppressive capacity of CD4(+)CD25(++) T(reg) in SSc was also found to be similar to that of HC. Phenotypic and functional data revealed no significant difference between the limited or diffuse form of SSc. Moreover, therapy with bosentan showed no significant effect on the frequency of T(reg) during the course of the disease. However, the frequency of T(reg) in skin lesions from patients with SSc or LS, determined as the percentage of CD4(+) cells expressing Foxp3 in the inflammatory infiltrate, was significantly reduced compared with other inflammatory skin diseases. These results indicate that although the authors found no defect in the frequency or function of peripheral T(reg) subpopulations, the reduction of CD4(+)Foxp3(+) T(reg) in the skin of patients with SSc may be important in the pathogenesis of the disease.

  14. [Bacteriological study on juvenile periodontitis].

    Science.gov (United States)

    Han, N

    1991-02-01

    The predominant cultivable microflora of 23 pockets in 15 juvenile periodontitis (JP) patients was studied for the first time in China using the current anaerobic methodology. Samples were taken with sterile paper points and dispersed on a vortex mixer. Then the diluted samples were plated on the non-selective blood agar plates and selective MGB medium which favors the growth of Actinobacillus actimycetemcomitans (Aa) and incubated in anaerobic chamber for 5 days. From each sample 15 or more isolated colonies were picked in sequence without selection and subcultured. The isolates were identified mainly by Schrechenberger's 4 hour rapid methods for biochemical and fermentative tests and the chromatographic analysis of acid end products using ion-chromatography. The results were as follows: 1. The microflora of healthy sulci of 7 healthy young subjects was significantly different from that in the pocket of JP patients. The predominant species in healthy sulci were Streptococcus spp and Capnocytophaga gingivalis. 2. The species increased significantly in JP patients in prevalence and proportions was Eubacterium. Other species in high proportions were Bacteroides oris, B. melaninogenicus, B. gingivalis, Capnocytophaga sputigena, and Actinomyces meyeri, etc. 3. Actinobacillus actinomycetemcomitans was not detected in any of the samples.

  15. First results of functional RES scintigraphy using sup(99m)-Tc-labeled human serum albumin millimicrospheres in progressive scleroderma: Possibility of early diagnosis of lung involvement

    International Nuclear Information System (INIS)

    Munz, D.L.; Altmeyer, P.; Ehrenheim, C.; Tuengerthal, S.; Holzmann, H.; Hoer, G.; Frankfurt Univ.

    1984-01-01

    Functional RES scintigraphy with sup(99m)Tc-labeled human serum albumin millimicrospheres (sup(99m)Tc-HSA-MM) was conducted in 11 female patients suffering from progressive scleroderma. The RES scan revealed abnormalities in the bone marrow in eight patients as well as pathological changes of the liver in 2 cases. 7 patients showed diffusely enhanced concentrations of sup(99m)Tc-HSA-MM in the lung, whereas X-ray picture and pulmonary function test revealed pathological findings in only 4 patients, respectively. Humoral inflammatory and immunologic parameters, too, indicated abnormalities less frequently than the lung scan. Thus functional RES scintigraphy seems to be a very sensitive approach to the assessment and possibly to the early diagnosis of lung involvement in progressive scleroderma. (orig.) [de

  16. First results of functional RES scintigraphy using sup(99m)-Tc-labeled human serum albumin millimicrospheres in progressive scleroderma: Possibility of early diagnosis of lung involvement

    Energy Technology Data Exchange (ETDEWEB)

    Munz, D.L.; Altmeyer, P.; Ehrenheim, C.; Tuengerthal, S.; Holzmann, H.; Hoer, G.

    1984-01-01

    Functional RES scintigraphy with sup(99m)Tc-labeled human serum albumin millimicrospheres (sup(99m)Tc-HSA-MM) was conducted in 11 female patients suffering from progressive scleroderma. The RES scan revealed abnormalities in the bone marrow in eight patients as well as pathological changes of the liver in 2 cases. 7 patients showed diffusely enhanced concentrations of sup(99m)Tc-HSA-MM in the lung, whereas X-ray picture and pulmonary function test revealed pathological findings in only 4 patients, respectively. Humoral inflammatory and immunologic parameters, too, indicated abnormalities less frequently than the lung scan. Thus functional RES scintigraphy seems to be a very sensitive approach to the assessment and possibly to the early diagnosis of lung involvement in progressive scleroderma.

  17. Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids.

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-10-01

    We would like to report our experience of using rituximab in cyclophosphamide refractory, rapidly progressive interstitial lung disease (ILD) in a patient with limited scleroderma. A 40-year-old man presented with 10-week history of inflammatory polyarthritis, which responded to a short course of oral corticosteroids. However, 3 weeks later, he developed new onset of exertional dyspnoea. High-resolution CT of the thorax was suggestive of early ILD. Surgical lung biopsy showed features of fibrotic non-specific interstitial pneumonia. He was diagnosed with scleroderma on the basis of: presence of anticentromere antibodies, Raynaud\\'s phenomenon, pulmonary fibrosis, digital oedema and hypomotility along with a dilated oesophagus. He was treated aggressively with pulse doses of corticosteroids and cyclophosphamide; however, his ILD continued to deteriorate. At this stage, he received rituximab (two pulses of 1 g each), which led to a gradual clinical improvement. Now, 12 months since his rituximab infusion, he walks 2 miles daily without any exertional dyspnoea.

  18. Results of domestic migration on juvenile delinquency in Adana, Turkey.

    Science.gov (United States)

    Firat, Sunay; Iltas, Yigit; Gulmen, Mete K

    2017-07-01

    The aim of this study is to determine the effect of migration on children in the juvenile justice system. The study takes into account whether these children arrived in the city via migration or not, the types of crimes against property and persons committed by the children brought before the Juvenile Courts, and the types, frequency, and durations of punishments and precautionary decisions they received. In addition, the study examines the children's ability to realize the meaning and consequences of their crimes and to lead their future behaviors effectively. Data gathered from children between 3 and 18 years of age who appeared before the 1, 2, and 3 numbered Juvenile Courts of Adana Courthouse after January 2004 on charges of committing a crime, and/or children who were sent to or who applied to the Provincial Directorate for National Education based on the decision of the Court has been evaluated retrospectively. Six hundred and eighty children were studied. Of these, 602 (88.5%) were male and 78 (11.5) were female. The average age of the child at the time of the commitment of the crime was 13 years and 8 months ± 2.18 years. It has been observed that there are statistically meaningful differences for children involved in delinquency between cases whether they come via migration or regardless of migration and types of crimes committed (p = 0.004). When cities faced with migration from other places and types of crime committed by children are compared, it has been observed that there are meaningful differences between the types of crimes committed by non-migrant versus migrant children (p = 0.012). It is important to state the reasons for delinquency and to obtain data to prevent future delinquency and to put forward regional and local recommendations within the scope of the data. Copyright © 2017 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

  19. [Juvenile-onset ankylosing spondylitis].

    Science.gov (United States)

    Menkes, C J; Job-Deslandre, C; Feldmann, J L

    1984-02-16

    Ankylosing spondylitis (AS) with juvenile onset (under 17 years of age) is not infrequent. Thirty-six cases were studied, amounting to 18% of patients hospitalized between 1977 and 1981. The following criteria were used for diagnosis: radiologic sacroiliitis (typical AS), presence of HLA B27 and/or pelvic or vertebral clinical manifestations (possible AS). 31 patients (85%) were boys. Mean age at onset was 12.3 +/- 2.8 years. In three cases, AS was found in a member of the family of the propositus and in one case there was cutaneous psoriasis. Usually (29 cases) onset was in the lower limbs: arthritis of the knee (14 cases), hip (9 cases), ankle (7 cases) or painful heel (4 cases). During the course (with a mean follow-up of 11.2 +/- 7 years), 35 patients exhibited peripheral joint diseases and 25 had axial involvement. Ocular involvement was present in 5 cases. 10 patients had a modification of respiratory function. Radiologic sacroiliitis was found in 31 patients but with a delay of 5.3 +/- 2.6 years. Vertebral radiologic lesions were only seen in 11 patients. Radiologic hip involvement was frequent (20 cases) with complete destruction in 6 patients. Erosion and ossification of the calcaneum were observed in 15 cases. The ESR was above 20 mm/first hour in 26 cases (72%). 81% of these patients were HLA B27 positive. Functional prognosis was good: 16 patients (51.6%) led an almost normal life, 6 were bedridden (Steinbrocker's grade IV), 3 had severe impairment (grade III) and 6 had slight impairment (grade II).(ABSTRACT TRUNCATED AT 250 WORDS)

  20. Juvenile idiopathic arthritis – an update on its diagnosis and ...

    African Journals Online (AJOL)

    2015-12-03

    Dec 3, 2015 ... Juvenile idiopathic arthritis (JIA) is the most common form of chronic arthritis in children and the most ... A swollen knee and uveitis in a young girl, for instance, is ..... Methotrexate for treating juvenile idiopathic arthritis.

  1. 83 CHALLENGES AND PROSPECTS OF THE JUVENILE JUSTICE ...

    African Journals Online (AJOL)

    Fr. Ikenga

    Juvenile justice administration in Nigeria is weak and has been given very .... The Nigerian criminal justice system, of which the juvenile justice system is an integral part, ... as instruments of security and justice but as weapons of oppression8.

  2. AFSC/ABL: Juvenile rockfish DNA species identification

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Many pelagic juvenile rockfish (Sebastes) were collected in juvenile salmonid surveys in the Gulf of Alaska (GOA) from 1998 to 2002. Often species identification of...

  3. Juvenile Fibromyalgia: A Multidisciplinary Approach to Treatment.

    Science.gov (United States)

    Tesher, Melissa S

    2015-06-01

    A 14-year-old boy presented with months of severe widespread musculoskeletal pain. He was profoundly fatigued and unable to attend school. Laboratory evaluation, including complete blood count, comprehensive metabolic panel, inflammatory markers, and thyroid function, was unrevealing. Physical examination was also normal except for multiple tender points. The patient was diagnosed with juvenile primary fibromyalgia syndrome and referred for multidisciplinary treatment including physical therapy, exercise, and counseling, and his daily functioning gradually improves. Juvenile fibromyalgia is a complex syndrome that often severely limits patients' activities and can impede normal adolescent development. Effective treatment requires an understanding of the biologic, psychologic, and social factors contributing to the perpetuation of chronic pain. The author reviews the diagnostic criteria, pathophysiology, and treatment of juvenile fibromyalgia. Medications, particularly antidepressants and anticonvulsants, can be useful adjuncts to therapy. However, multimodal pain management including intensive physical therapy, exercise, counseling, and sleep hygiene is most effective in treating fibromyalgia. Copyright 2015, SLACK Incorporated.

  4. Effect of TBT on Ruditapes decussatus juveniles.

    Science.gov (United States)

    Coelho, M R; Langston, W J; Bebianno, M J

    2006-06-01

    The effects of sublethal concentrations of tributyltin (TBT) on growth of juvenile clams Ruditapes decussatus were determined during exposure to TBT concentrations of 50, 100 and 250 ng l(-1) (as Sn) for a period up to two years. Length and weight of clams increased continuously in all treatments throughout the experimental period, and, overall, rates were not significantly influenced by TBT exposure, although final length and weight were inversely related to increasing TBT concentration. Juvenile R. decussatus therefore appear to be less sensitive to TBT than larval stages. Some juveniles exposed to TBT developed abnormal shell growth, laterally, changing the typical flattened shape of clams into a more "rounded" form. This characteristic was more visible in the anterior margins of valves than posteriorly, and mainly observed in clams exposed to TBT at 50 ng l(-1) (as Sn).

  5. Juvenile xanthogranuloma of the corneoscleral limbus.

    Science.gov (United States)

    Yanoff, M; Perry, H D

    1995-07-01

    Juvenile xanthogranuloma is a rare and usually benign skin disease of unknown cause that occurs in infants and young children. We studied a case of juvenile xanthogranuloma of the corneoscleral limbus in a 17-year-old black boy, who presented with a 5-month history of a lump in the right eye. The lesion extended from the superior limbus, fanning out as it proceeded posteriorly for 6 mm with a width of 9 mm and a height of 2 to 3 mm. This yellow-orange mass was vascular and firmly fixed to the underlying tissue. The lesion was diagnosed as a dermoid and observed for 7 months without documented growth before an uneventful excisional biopsy was performed. The pathologic diagnosis showed the characteristic picture of juvenile xanthogranuloma with numerous Touton giant cells. Lipid stains provided further confirmation.

  6. Corporal and capital punishment of juveniles.

    Science.gov (United States)

    Frazier, H C

    1990-01-01

    There is a previously unobserved connection between corporal punishment of public school children and capital punishment of juveniles. Both are barometers of acceptable levels of violent punishment and their elimination is a hallmark of a maturing and decent society. Within a majority of the eighteen states where school authorities most frequently strike children are housed 25 of the nation's 28 juvenile death row inmates. On average, the homicide rates of these jurisdictions are two and a half times greater than those that have abolished both state-sanctioned corporal and capital punishment or limit death sentences to those age eighteen and older at the time of their crime(s). Most of the eighteen state abolitions of corporal punishment occurred in the 1980's. The US Supreme Court has ruled both corporal and capital punishment of juveniles constitutional. Additional state legislative abolition of both is anticipated in the 1990s.

  7. Delincuencia y responsabilidad penal juvenil en Colombia

    Directory of Open Access Journals (Sweden)

    Cristina Montalvo Velásquez

    2011-01-01

    Full Text Available ResumenEl término «delincuencia juvenil» fue acuñado en Inglaterra en el año 1815, “Se entiende por delincuencia juvenil el conjunto de delitos, contravenciones o comportamientos socialmente reprochables, que cometen las personas consideradas como jóvenes por la ley”1 . Cada Estado está sujeto a su propio sistema jurídico, para algunos es delincuente juvenil el adolescente que comete acciones sancionadas por la ley sin importar su gravedad, otros Estados sólo consideran como delincuente juvenil al joven que comete un acto delictivo grave.El fenómeno de la delincuencia juvenil es algo que se inscribe en los espacios de una sociedad en la cual su estructura material, y su formación social consecuente, se halla en una profunda crisis. Que jóvenes conformen bandas de delincuencia organizada nos está indicando que son el resultado de la misma criminalidad general que se ha apoderado de la sociedad en la perspectiva de lograr sobrevivir materialmente. El capitalismo no es sólo acumulación de riqueza sino concentración de la misma en muy pocas manos; y todo el sistema institucional y legal tiende a favorecer ese fenómeno porque éste constituye la supra estructura del modo de producción capitalista. Así como los adultos se organizan para delinquir, lo hacen los niños y los jóvenes a partir de una edad en la cual pueden percibir que la sociedad no es sana y no tienen porvenir humano en ella. Abandonados y sujetos a la violencia que engendra el sistema, ellos simplemente responden en una manifestación de reflejos condicionados que sostienen la sobrevivencia en forma instintiva; “los niños no saben de normas legales sino de formas de sobrevivir a semejante situación; el instinto de sobrevivencia no tiene edades ni la normatividad puede incidir en él”.Palabras ClavesDelincuencia juvenil, Jóvenes, Criminalidad, Familia, Factores, Acto delictivo, Responsabilidad Penal.AbstractThe term “juvenile delinquency” was coined in

  8. Imágenes juveniles, medios y nuevos escenarios

    Directory of Open Access Journals (Sweden)

    Oscar Aguilera Ruiz

    2015-01-01

    Full Text Available Este artículo nace del análisis de los discursos radiales producidos exclusivamente para jóvenes en Santiago de Chile. Aborda además las categorías comprensivas de la vida juvenil, la vida juvenil de los años 90, el imaginario juvenil des-simbolizado, hacia una comprensión de lo juvenil, dinámica social propuesta por los medios y estrategias comunicacionales.

  9. A juvenile subfossil crocodylian from Anjohibe Cave, Northwestern Madagascar

    Directory of Open Access Journals (Sweden)

    Joshua C. Mathews

    2016-09-01

    Full Text Available Madagascar’s subfossil record preserves a diverse community of animals including elephant birds, pygmy hippopotamus, giant lemurs, turtles, crocodiles, bats, rodents, and carnivorans. These fossil accumulations give us a window into the island’s past from 80,000 years ago to a mere few hundred years ago, recording the extinction of some groups and the persistence of others. The crocodylian subfossil record is limited to two taxa, Voay robustus and Crocodylus niloticus, found at sites distributed throughout the island. V. robustus is extinct while C. niloticus is still found on the island today, but whether these two species overlapped temporally, or if Voay was driven to extinction by competing with Crocodylus remains unknown. While their size and presumed behavior was similar to each other, nearly nothing is known about the growth and development of Voay, as the overwhelming majority of fossil specimens represent mature adult individuals. Here we describe a nearly complete juvenile crocodylian specimen from Anjohibe Cave, northwestern Madagascar. The specimen is referred to Crocodylus based on the presence of caviconchal recesses on the medial wall of the maxillae, and to C. niloticus based on the presence of an oval shaped internal choana, lack of rostral ornamentation and a long narrow snout. However, as there are currently no described juvenile specimens of Voay robustus, it is important to recognize that some of the defining characteristics of that genus may have changed through ontogeny. Elements include a nearly complete skull and many postcranial elements (cervical, thoracic, sacral, and caudal vertebrae, pectoral elements, pelvic elements, forelimb and hindlimb elements, osteoderms. Crocodylus niloticus currently inhabits Madagascar but is locally extinct from this particular region; radiometric dating indicates an age of ∼460–310 years before present (BP. This specimen clearly represents a juvenile based on the extremely small

  10. Bacterial acquisition in juveniles of several broadcast spawning coral species.

    Directory of Open Access Journals (Sweden)

    Koty H Sharp

    Full Text Available Coral animals harbor diverse microorganisms in their tissues, including archaea, bacteria, viruses, and zooxanthellae. The extent to which coral-bacterial associations are specific and the mechanisms for their maintenance across generations in the environment are unknown. The high diversity of bacteria in adult coral colonies has made it challenging to identify species-specific patterns. Localization of bacteria in gametes and larvae of corals presents an opportunity for determining when bacterial-coral associations are initiated and whether they are dynamic throughout early development. This study focuses on the early onset of bacterial associations in the mass spawning corals Montastraea annularis, M. franksi, M. faveolata, Acropora palmata, A. cervicornis, Diploria strigosa, and A. humilis. The presence of bacteria and timing of bacterial colonization was evaluated in gametes, swimming planulae, and newly settled polyps by fluorescence in situ hybridization (FISH using general eubacterial probes and laser-scanning confocal microscopy. The coral species investigated in this study do not appear to transmit bacteria via their gametes, and bacteria are not detectable in or on the corals until after settlement and metamorphosis. This study suggests that mass-spawning corals do not acquire, or are not colonized by, detectable numbers of bacteria until after larval settlement and development of the juvenile polyp. This timing lays the groundwork for developing and testing new hypotheses regarding general regulatory mechanisms that control bacterial colonization and infection of corals, and how interactions among bacteria and juvenile polyps influence the structure of bacterial assemblages in corals.

  11. Bacterial acquisition in juveniles of several broadcast spawning coral species.

    Science.gov (United States)

    Sharp, Koty H; Ritchie, Kim B; Schupp, Peter J; Ritson-Williams, Raphael; Paul, Valerie J

    2010-05-28

    Coral animals harbor diverse microorganisms in their tissues, including archaea, bacteria, viruses, and zooxanthellae. The extent to which coral-bacterial associations are specific and the mechanisms for their maintenance across generations in the environment are unknown. The high diversity of bacteria in adult coral colonies has made it challenging to identify species-specific patterns. Localization of bacteria in gametes and larvae of corals presents an opportunity for determining when bacterial-coral associations are initiated and whether they are dynamic throughout early development. This study focuses on the early onset of bacterial associations in the mass spawning corals Montastraea annularis, M. franksi, M. faveolata, Acropora palmata, A. cervicornis, Diploria strigosa, and A. humilis. The presence of bacteria and timing of bacterial colonization was evaluated in gametes, swimming planulae, and newly settled polyps by fluorescence in situ hybridization (FISH) using general eubacterial probes and laser-scanning confocal microscopy. The coral species investigated in this study do not appear to transmit bacteria via their gametes, and bacteria are not detectable in or on the corals until after settlement and metamorphosis. This study suggests that mass-spawning corals do not acquire, or are not colonized by, detectable numbers of bacteria until after larval settlement and development of the juvenile polyp. This timing lays the groundwork for developing and testing new hypotheses regarding general regulatory mechanisms that control bacterial colonization and infection of corals, and how interactions among bacteria and juvenile polyps influence the structure of bacterial assemblages in corals.

  12. The World of Juvenile Justice According to the Numbers

    Science.gov (United States)

    Rozalski, Michael; Deignan, Marilyn; Engel, Suzanne

    2008-01-01

    Intended to be an instructive, yet sobering, introduction to the complex and disturbing nature of the juvenile justice system, this article details the "numbers," including selected percentages, ratios, and dollar amounts, that are relevant to developing a better understanding of the juvenile justice system. General statistics about juvenile and…

  13. Challenges and prospects of the juvenile justice administration in ...

    African Journals Online (AJOL)

    Juvenile justice administration in Nigeria is weak and has been given very little priority, despite Nigeria being signatory to the major international instruments relevant to the administration of juvenile justice. This is attributable to the history of the penal system of Nigeria, with laws guiding juvenile justice administration having ...

  14. Criminal Profiles of Violent Juvenile Sex and Violent Juvenile Non-Sex Offenders: An Explorative Longitudinal Study

    Science.gov (United States)

    van Wijk, Anton Ph.; Mali, Bas R. F.; Bullens, Ruud A. R.; Vermeiren, Robert R.

    2007-01-01

    Few studies have longitudinally investigated the criminal profiles of violent juvenile sex and violent juvenile non-sex offenders. To make up for this lack, this study used police records of juveniles to determine the nature of the criminal profiles of violent sex offenders (n = 226) and violent non-sex offenders (n = 4,130). All offenders…

  15. Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

    Science.gov (United States)

    Hockenberry, Sarah; Sickmund, Melissa; Sladky, Anthony

    2013-01-01

    This bulletin is part of the "Juvenile Offenders and Victims National Report Series." The "National Report" offers a comprehensive statistical overview of the problems of juvenile crime, violence, and victimization and the response of the juvenile justice system. During each interim year, the bulletins in the "National…

  16. Enhancement of juvenile Caribbean spiny lobsters: an evaluation of changes in multiple response variables with the addition of large artificial shelters.

    Science.gov (United States)

    Briones-Fourzán, Patricia; Lozano-Alvarez, Enrique; Negrete-Soto, Fernando; Barradas-Ortiz, Cecilia

    2007-03-01

    Shortage of natural crevice shelters may produce population bottlenecks in juvenile Caribbean spiny lobsters (Panulirus argus), a socially gregarious species. We conducted a field experiment to test enhancement of a local population of juvenile P. argus with the addition of artificial shelters ("casitas") that mimic large crevices (1.1 m(2) in surface area and 3.8 cm in height). Changes in density and biomass of juvenile lobsters 15-50 mm carapace length (CL) were assessed with a multiple before-after control-impact (MBACI) analysis. Separate analyses were also conducted on small (15-35 mm CL) and large (35.1-50 mm CL) juveniles to assess size-related effects. First, we carried out 13 lobster surveys on nine fixed 1-ha sites over a shallow reef lagoon ("before" period). Then, we deployed ten casitas in each of five sites and left four sites as controls, and conducted 22 further surveys ("after" period). Deployment of casitas resulted in a sixfold increase in juvenile density (76% contributed by small and 24% by large juveniles) and a sevenfold increase in biomass (40 and 60%, respectively). Capture-recapture results revealed that enhancement was achieved not by promoting individual growth but by increasing survival, persistence, and foraging ranges of small and large juveniles. Casitas both mitigated shortage of natural shelter and increased sociality, allowing for cohabitation of smaller, more vulnerable juveniles with larger conspecifics that have greater defensive abilities. Casitas may help enhance local populations of juvenile P. argus in Caribbean seagrass habitats, typically poor in natural crevice shelters. The use of MBACI and the simultaneous assessment of multiple interrelated response variables may be a powerful analytical approach to test shelter limitation in other species and to examine the function of structural habitat in other systems.

  17. Exercise therapy in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

  18. Program Performance Inventory: Six Juvenile Offender Programs.

    Science.gov (United States)

    Thomalla, Terri Groff; Dougherty, Victoria J.

    This report describes the performance of 6 Connecticut juvenile justice alternative sanction programs in 14 qualitative areas: community reintegration; outcomes and evaluation; assessment methods; risk factors; escalation of criminal activity; family involvement; community involvement; work ethic and vocational training; education and life skills;…

  19. Radium rentention and dosimetry in juvenile beagles

    International Nuclear Information System (INIS)

    Lloyd, R.D.; Jones, C.W.; Bruenger, F.W.; Atherton, D.R.; Mays, C.W.

    1983-01-01

    Retention of administered 226 Ra was substantially greater in beagles injected as 3-month-old juveniles than as 1.4-year-old adults, but the measured 222 Rn/ 226 Ra ratio in bone was significantly less in juveniles for about the first 600 days after injection. An equation that describes the total-body biological retention R in beagles injected with 226 Ra at 3 months of age at any time t (in days) after injection during the first 6.6 years is R = 0.331e/sup -0.206t/ + 0.245e/sup -0.00374t/ + 0.424e/sup -0.000114t/. The rate constant of the final term in the equation for juveniles is similar to that for young adults, suggesting that this component reflects the net turnover rate in the slowly remodeling component of adult bone. Compared to young adult beagles, animals injected as juveniles had a greater fraction of their retained 226 Ra in parts of the skeleton containing much cortical bone, such as paws, and a smaller fraction in those parts containing much trabecular bone

  20. Alteracioness cognitivas en familias con Parkinson juvenil

    Directory of Open Access Journals (Sweden)

    Francisco Javier Lopera Restrepo

    2005-03-01

    Full Text Available El Grupo de Neurociencias de la Universidad de Antioquia reportó por primera vez en Colombia cuatro familias afecatas por la Enfermedad de Parkinson Familiar Juvenil portadoras de la mutación G736A en el gen Parkin.

  1. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

    BACKGROUND AND OBJECTIVE: Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currently...

  2. Antibodies in juvenile-onset myositis.

    Science.gov (United States)

    Tansley, Sarah L

    2016-11-01

    Juvenile-onset myositis is a highly heterogeneous disease. Myositis-specific and associated autoantibodies provide a potential means of subdividing patients into clinically homogenous subgroups. Given the increasing availability of autoantibody testing, this review explores the phenotypes associated with different autoantibodies in juvenile-onset myositis and the potential clinical utility of autoantibody testing. Autoantibodies can be identified in 60-70% of children with myositis and the recent discovery of novel myositis-associated autoantibodies in adult patients suggests this may increase in the near future. Detailed phenotype descriptions are now known for several autoantibodies commonly identified in juvenile-onset disease. Whilst there is insufficient evidence to recommend a differential treatment approach based on autoantibody status, it is becoming increasingly clear that some autoantibody subgroups are often treatment resistant and may benefit from a more aggressive approach. The validation of nonspecialised methods for myositis-specific autoantibody detection should lead to more widely available testing. In juvenile-onset disease, this will provide detailed prognostic information and in the future may also influence approach.

  3. Do burn centers provide juvenile firesetter intervention?

    Science.gov (United States)

    Ahrns-Klas, Karla S; Wahl, Wendy L; Hemmila, Mark R; Wang, Stewart C

    2012-01-01

    Juvenile firesetting activity accounts for a significant number of annual injuries and property damage, yet there is sparse information on intervention in the burn literature. To quantify juvenile firesetting intervention (JFSI) in burn centers, a 23-question survey was sent to all directors listed in the American Burn Association Burn Care Facilities Directory.Sixty-four out of 112 (57%) surveys were returned. This represents responses from 79% of currently verified burn centers. When queried on interventions provided to a juvenile firesetter admitted to their unit, 38% report having their own JFSI program and 38% refer the child to fire services. Two thirds of units without a JFSI program treat pediatric patients. Units that previously had a JFSI program report lack of staffing and funding as most common reasons for program discontinuation. Almost all (95%) stated that a visual tool demonstrating legal, financial, social, future, and career ramifications associated with juvenile firesetting would be beneficial to their unit. Many burn units that treat pediatric patients do not have JFSI and rely on external programs operated by fire services. Existing JFSI programs vary greatly in structure and method of delivery. Burn centers should be involved in JFSI, and most units would benefit from a new video toolkit to assist in providing appropriate JFSI. Study results highlight a need for burn centers to collaborate on evaluating effectiveness of JFSI programs and providing consistent intervention materials based on outcomes research.

  4. Retrocalcaneal bursitis in juvenile chronic arthritis.

    OpenAIRE

    Goldenstein-Schainberg, C; Homsi, C; Rodrigues Pereira, R M; Cossermelli, W

    1992-01-01

    Retrocalcaneal bursitis has been described in various adult rheumatic diseases and septic bursitis unrelated to previous bursal disease has been reported in children. The case is reported here of a girl with juvenile chronic arthritis who developed non-septic retrocalcaneal bursitis; the diagnosis was suggested by a combination of clinical and radiographic studies and was confirmed by ultrasonography.

  5. Retrocalcaneal bursitis in juvenile chronic arthritis.

    Science.gov (United States)

    Goldenstein-Schainberg, C; Homsi, C; Rodrigues Pereira, R M; Cossermelli, W

    1992-01-01

    Retrocalcaneal bursitis has been described in various adult rheumatic diseases and septic bursitis unrelated to previous bursal disease has been reported in children. The case is reported here of a girl with juvenile chronic arthritis who developed non-septic retrocalcaneal bursitis; the diagnosis was suggested by a combination of clinical and radiographic studies and was confirmed by ultrasonography. Images PMID:1444631

  6. Bilateral, independent juvenile nasopharyngeal angiofibroma: case report.

    Science.gov (United States)

    Mørkenborg, M-L; Frendø, M; Stavngaard, T; Von Buchwald, C

    2015-10-01

    Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning and surgical strategy. A 21-year-old male presented with increasing bilateral nasal obstruction and discharge. Examination revealed tumours bilaterally and imaging demonstrated non-contiguous tumours. Pre-operative angiography showed strictly ipsilateral vascular supplies requiring bilateral embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral symptoms. Our management, including successful pre-operative planning, enabled one-step total removal of both tumours and rapid patient recovery.

  7. Perceived Competence of Juvenile Delinquents and Nondelinquents.

    Science.gov (United States)

    Cole, Peter G.; And Others

    1989-01-01

    Thirty male juvenile delinquents and 90 male high achievers, low achievers, and students with behavior problems were compared using an adapted version of Harter's Perceived Competence Scale for Children. The Australian students (aged 12-15) were compared on 4 different domains of perceived competence--cognitive competence, social competence,…

  8. Factors Involved in Juveniles' Decisions about Crime.

    Science.gov (United States)

    Cimler, Edward; Beach, Lee Roy

    1981-01-01

    Investigated whether delinquency is the result of a rational decision. The Subjective Expected Utility (SEU) model from decision theory was used with male juvenile offenders (N=45) as the model of the decision process. Results showed that the SEU model predicted 62.7 percent of the subjects' decisions. (Author/RC)

  9. Juvenile Court Commitment Rates: The National Picture.

    Science.gov (United States)

    Sosin, Michael

    There is less geographic variation in the commitment rate of juvenile offenders than is commonly assumed. Apparently, judges across the country develop a similar standard of what percentage of youths they face should be committed. This standard may be similar across the country because it represents broadly shared ideals. However, there is much…

  10. Smerte og smertemestring ved juvenil idiopatisk artritis

    DEFF Research Database (Denmark)

    Herlin, Troels; Thastum, Mikael

    2008-01-01

    Pain is one of the primary symptoms of juvenile idiopathic arthritis (JIA). JIA patients have reduced pain tolerance and pain threshold compared to healthy controls. In children with JIA the greater use of coping strategies such as problem-solving, positive self-statements and distraction consist...

  11. Biologisk terapi ved juvenil idiopatisk artritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2008-01-01

    In recent years the treatment of juvenile idiopathic arthritis (JIA) has undergone marked changes. There is substantial evidence that inhibitors of tumor necrosis factor alpha (TNFalpha) like etanercept, infliximab and adalimumab show significant efficacy when standard therapy fails, and long-ter...

  12. Nasopharyngeal juvenile angiofibroma: updating of radiotherapy results

    International Nuclear Information System (INIS)

    Alert Silva, Jose; Caballero Aguirrechu, Iraida; Reno Cespedes, Jesus; Perez Penna, Lourdes

    2010-01-01

    The nasopharyngeal juvenile angiofibroma is a uncommon benign tumor composed of fibrous connective tissue and many vascular spaces covered by endothelium. It is almost exclusive of male sex and of adolescents. Choice treatment is the exeresis without obviate other possibilities as the radiotherapy. The aim of present study was to show the results of this latter as therapeutical option

  13. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Background: Acute Lymphoblastic Leukaemia in children commonly presents with osteo articular manifestations that may mimic Juvenile Idiopathic Arthritis. This may create considerable diagnostic difficulty and lead to delay in commencing appropriate treatment. Case: An eight year old boy who presented with multiple ...

  14. Molecular basis of juvenile hormone signaling

    Czech Academy of Sciences Publication Activity Database

    Jindra, Marek; Bellés, X.; Shinoda, T.

    2015-01-01

    Roč. 11, Oct 09 (2015), s. 39-46 ISSN 2214-5745 R&D Projects: GA ČR GA15-23681S Institutional support: RVO:60077344 Keywords : juvenile hormone * JH receptor * Drosophila melanogaster Subject RIV: ED - Physiology Impact factor: 2.719, year: 2015 http://www.sciencedirect.com/science/article/pii/S2214574515001297

  15. Metamorphosis: How Missouri Rehabilitates Juvenile Offenders

    Science.gov (United States)

    Dubin, Jennifer

    2012-01-01

    Juveniles convicted of serious offenses usually end up in large correctional facilities that focus on punishment--not rehabilitation. The state of Missouri, however, has found a better way to help end the cycle of crime: by creating a network of small facilities that provide therapy and educational opportunities, it has dramatically reduced…

  16. Shortening the juvenile phase for flowering

    NARCIS (Netherlands)

    Higazy, M.K.M.T.

    1962-01-01

    Higazy tried to determine whether the duration of the juvenile phase for flowering was a fixed character or whether it could be influenced by external growth factors.

    Lunaria biennis was chosen as a cold-requiring biennial, Silene armeria as a long-day plant and Salvia

  17. The Diversity of Juvenile Sarcoidosis Symptoms

    Directory of Open Access Journals (Sweden)

    O. Vougiouka

    2012-01-01

    Full Text Available We report a case of juvenile sarcoidosis, emphasizing the variety of clinical manifestations. The child had uveitis, which is among the most common manifestations of the disease. However, fever of unknown origin, glomerulonephritis and lymphadenopathy were also noticed, underscoring the diversity of the clinical spectrum of the disease.

  18. Juvenile nasopharyngeal angiofibroma: Clinical diagnosis and treatment experience

    Directory of Open Access Journals (Sweden)

    Sladoje Radmila

    2002-01-01

    Full Text Available Juvenile nasopharyngeal angiofibroma is an infrequent epi-pharyngeal tumor necessitating particular diagnostic and therapeutic procedures in comparison to other benign epipharyngeal tumors due to its expansive growth tendency. Our retrospective study is aimed at presenting clinical casuistry of the tumor in order to evaluate modern diagnostic and therapeutic possibilities. The study included 13 male patients, aged 13-24 years, who were hospitalized, diagnostically assessed and surgically treated at the Institute of Otorhinolaryngology and Maxillofacial Surgery, Clinical Centre of Serbia over the period 1990 - June 2001. The following parameters were analyzed: sex, age groups, preoperative symptoms of the disease, diagnostic methods, embolization, local tumor spreading, number and time of tumor relapses and surgical approach.

  19. Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma

    Directory of Open Access Journals (Sweden)

    Satyaranjan Mishra

    2013-01-01

    Full Text Available Juvenile nasopharyngeal angiofibroma (JNA is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in adolescent males. Usually, the presenting symptom is a painless nasal obstruction or epistaxis; however, other symptoms may develop depending on the size and extent of the tumor mass. Owing to the vascularity of the tumor, incisional biopsy is not attempted. The diagnosis is dependent on multiplanar imaging modalities like Computed Tomography (CT, Magnetic Resonance Imaging (MRI, and Angiography. These imaging modalities help in assessing the tumor mass, pre-operative embolization of the feeder vessel, and treatment planning. Usually, patients with JNA are diagnosed by otorhinolaryngologists, but here, we present a rare case of JNA reporting to the dental hospital due to a tender palatal swelling.

  20. Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma

    OpenAIRE

    Pantalon, Anca; Ștefănache, Tudor; Danciu, Mihai; Zurac, Sabina; Chiseliță, Dorin

    2017-01-01

    Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these...