SAT0441 Juvenile Onset Systemic Sclerosis: Clinical and Serological Features, and Mortality in Comparison with Adult Onset Disease

2013 ◽  
Vol 72 (Suppl 3) ◽  
pp. A731.1-A731
Author(s):  
J. G. Ovalles-Bonilla ◽  
F. J. López-Longo ◽  
I. Monteagudo ◽  
E. Naredo ◽  
C. M. Gonzalez ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Adult Onset ◽  
Juvenile Onset

scholarly journals Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort

2018 ◽  
Vol 4 (1) ◽  
pp. 43-48
Author(s):  
Percival D Sampaio-Barros ◽  
Adriana B Bortoluzzo ◽  
Ana Paula T Del Rio ◽  
Ana Paula Luppino-Assad ◽  
Danieli CO Andrade ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Age At Onset ◽  
Adult Onset ◽  
Juvenile Onset ◽  
Clinical Pattern ◽  
Diffuse Systemic Sclerosis ◽  
Laboratory Profile ◽  
Brazilian Cohort

Objective: To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was <16 years. Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.

Characteristics of Patients with Juvenile Onset Systemic Sclerosis in an Adult Single-center Cohort

2010 ◽  
Vol 37 (11) ◽  
pp. 2422-2426 ◽  
Author(s):  
IVAN FOELDVARI ◽  
SVETLANA I. NIHTYANOVA ◽  
ANGELA WIERK ◽  
CHRISTOPHER P. DENTON
Keyword(s):  
Systemic Sclerosis ◽  
Survival Rates ◽  
Disease Onset ◽  
High Survival ◽  
Adult Onset ◽  
Single Center ◽  
Juvenile Onset ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Onset Group

Objective.Systemic sclerosis (SSc) is a rare connective tissue disease in childhood. We compared the characteristics of adult patients with juvenile-onset SSc (jSSc) from a single-center cohort to an adult-onset group.Methods.Patients with disease onset before the age of 17 years were included in the jSSc cohort, while subjects with SSc onset after age 17 formed the adult-onset cohort.Results.We identified 52 adult subjects with jSSc and compared them to 954 patients with adult-onset SSc. The mean ± SD age at disease onset of the patients with jSSc was 14 ± 2 years, 39 (75%) of them were women, and 24 (46%) had the diffuse cutaneous subset of SSc (dcSSc). There were no differences between the 2 cohorts in terms of sex and disease subset. Overlaps were significantly more frequent among the jSSc cohort (37%) compared to the adult-onset group (18%; p = 0.002). Autoantibody analysis demonstrated significantly more antitopoisomerase I antibody-positive subjects (33% vs 20%; p = 0.034) and significantly fewer anticentromere antibody-positive subjects (2% vs 25%; p < 0.001) in the jSSc cohort. Compared to the adult-onset group at 10 years from disease onset, survival was significantly higher among the subjects with jSSc (98% vs 75%; p = 0.001), pulmonary arterial hypertension had a significantly lower incidence (2% vs 14%; p = 0.032), and there was no difference in terms of pulmonary fibrosis (22% vs 21%) and cardiac scleroderma (3% vs 2%) between the 2 groups.Conclusion.The high survival rates and lower proportion of dcSSc in the adult jSSc cohort may represent a survival bias.

scholarly journals One repeated transplantation of allogeneic umbilical cord mesenchymal stromal cells in type 1 diabetes: an open parallel controlled clinical study

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Jing Lu ◽  
Shan-mei Shen ◽  
Qing Ling ◽  
Bin Wang ◽  
Li-rong Li ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Stromal Cells ◽  
Treated Group ◽  
Control Group ◽  
Adult Onset ◽  
Β Cell ◽  
Juvenile Onset ◽  
C Peptide

Abstract Background The preservation or restoration of β cell function in type 1 diabetes (T1D) remains as an attractive and challengeable therapeutic target. Mesenchymal stromal cells (MSCs) are multipotent cells with high capacity of immunoregulation, which emerged as a promising cell-based therapy for many immune disorders. The objective of this study was to examine the efficacy and safety of one repeated transplantation of allogeneic MSCs in individuals with T1D. Methods This was a nonrandomized, open-label, parallel-armed prospective study. MSCs were isolated from umbilical cord (UC) of healthy donors. Fifty-three participants including 33 adult-onset (≥ 18 years) and 20 juvenile-onset T1D were enrolled. Twenty-seven subjects (MSC-treated group) received an initial systemic infusion of allogeneic UC-MSCs, followed by a repeat course at 3 months, whereas the control group (n = 26) only received standard care based on intensive insulin therapy. Data at 1-year follow-up was reported in this study. The primary endpoint was clinical remission defined as a 10% increase from baseline in the level of fasting and/or postprandial C-peptide. The secondary endpoints included side effects, serum levels of HbA1c, changes in fasting and postprandial C-peptide, and daily insulin doses. Results After 1-year follow-up, 40.7% subjects in MSC-treated group achieved the primary endpoint, significantly higher than that in the control arm. Three subjects in MSC-treated group, in contrast to none in control group, achieved insulin independence and maintained insulin free for 3 to 12 months. Among the adult-onset T1D, the percent change of postprandial C-peptide was significantly increased in MSC-treated group than in the control group. However, changes in fasting or postprandial C-peptide were not significantly different between groups among the juvenile-onset T1D. Multivariable logistic regression assay indicated that lower fasting C-peptide and higher dose of UC-MSC correlated with achievement of clinical remission after transplantation. No severe side effects were observed. Conclusion One repeated intravenous dose of allogeneic UC-MSCs is safe in people with recent-onset T1D and may result in better islet β cell preservation during the first year after diagnosis compared to standard treatment alone. Trial registration ChiCTR2100045434. Registered on April 15, 2021—retrospectively registered, http://www.chictr.org.cn/

scholarly journals Differences in Early Childhood Risk Factors for Juvenile-Onset and Adult-Onset Depression

2002 ◽  
Vol 59 (3) ◽  
pp. 215 ◽  
Author(s):  
Sara R. Jaffee ◽  
Terrie E. Moffitt ◽  
Avshalom Caspi ◽  
Eric Fombonne ◽  
Richie Poulton ◽  
...  
Keyword(s):  
Risk Factors ◽  
Early Childhood ◽  
Adult Onset ◽  
Juvenile Onset ◽  
Childhood Risk Factors

scholarly journals Brief Report: HLA-DRB1, DQA1, and DQB1 in Juvenile-Onset Systemic Sclerosis

2016 ◽  
Vol 68 (11) ◽  
pp. 2772-2777 ◽  
Author(s):  
Anne M. Stevens ◽  
Sami B. Kanaan ◽  
Kathryn S. Torok ◽  
Thomas A. Medsger ◽  
Maureen D. Mayes ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Juvenile Onset

Myelin basic protein and myelin oligodendrocyte glycoprotein T-cell repertoire in childhood and juvenile multiple sclerosis

2006 ◽  
Vol 12 (4) ◽  
pp. 412-420 ◽  
Author(s):  
Jorge Correale ◽  
Silvia N Tenembaum
Keyword(s):  
Multiple Sclerosis ◽  
T Cell ◽  
Myelin Basic Protein ◽  
Basic Protein ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
T Cell Repertoire ◽  
Adult Onset ◽  
Cell Repertoire ◽  
Juvenile Onset ◽  
Exon 2

Multiple sclerosis (MS) is usually a disease of young adulthood, its clinical onset occurring between 20 and 40 years of age; however, today there is general consensus that MS can also occur in children, adolescents and even in infants. In order to gain further insight into the T-cell repertoire present in this particular group of patients myelin basic protein (MBP)-, MBP exon-2- and myelin oligodendrocyte glycoprotein (MOG)Igd-specific T-cell lines (TCLs) were isolated from 18 patients whose symptoms had started before the age of 16. Epitope specificity was established by measuring proliferative responses, and interferon-g (IFN-g) secretion by using a panel of overlapping synthetic peptides. For MOGIgd, the T-cell response was focused on three main immunodominant epitopes comprising residues 1-26, 36-60 and 63-87. For MBP the predominant immune responses were directed against peptides 83-102, 139-153 and 146-162. When compared to those observed in adult-onset MS patients, anti-MOGIgd specificity and anti-MBP responses showed similar results. Moreover, the number of MBP exon-2 TCLs isolated, and the magnitude of the specific IFN-g secretion induced were similar, both in childhood/juvenile-onset and adult-onset MS patients. Thus, despite differences in the clinical and neuroimaging manifestations of MS, these results would seem to indicate that both the spectrum of MBP found, as well as the MOGIgd epitopes recognized by peripheral blood T cells in MS, appear to be similar for childhood/juvenile-onset and adult-onset patients.

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