scholarly journals CLINICAL COURSE OF JUVENILE IDIOPATHIC ARTHRITIS IN HLA-B27-POSITIVE PATIENTS IN ADULTHOOD

2017 ◽  
Vol 13 (1-2) ◽  
pp. 12-20
Author(s):  
M.B. Dzhus ◽  
H.V. Mostbauer ◽  
T.A. Karasevska ◽  
O.I. Ivashkivsky

Relevance. The existence of associations between histocompatibility antigens and JIA variants has been proved. There is no consensus that the JIAs associated with HLA-B27 antigen are transformed in adulthood into other diseases for which it is necessary to revise the diagnosis, according to the adult classification of rheumatic diseases. Is this one process that began in childhood and continues into adulthood, and whether these two processes that begin in childhood and adulthood have common signs and differences? There is few data about the hallmarks of the disease and outcome in adulthood.   Objective. – To investigate the frequency of HLA-B27 detection in adult patients with a history of JIA and to evaluate the clinical features of the course of arthritis in adulthood and the long-term articular and extra-articular consequences of JIA. Materials and methods. A survey of 132 young adult patients with different variants of JIA in the history (70 women, 62 males), aged – 24,3±8,3 years; disease duration – 13,6±9,3 years. We evaluated body mass index, anamnestic data, visual analogue scale, C-reactive protein quantitatively, HLA-B27, rheumatoid factor (RF), IgG-antibody to cyclic citrulline peptide (anti-CCP) and antinuclear antibody (АNА). Long-term effects JIA assessed by joint indices (JADI-A) and extraarticular (JADI-E) damage. Disease activity in childhood and adulthood was assessed on a scale JADAS (Juvenile Arthritis Disease Activity Score) and DAS 28. For statistic analises we use the Statistica 6.0 software packages Copyright © StatSoft, Inc. 1984-2001. Results. HLA-B27 was found in 38 patients with JIA (28,8 %), including 95 % of patients with enthesitis-related arthritis, 28,1 % – with persistent oligooarthritis and 36,8 % of patients with extended oligoarthritis, 8,3 % – with RF-positive JIA and 10,5 % – with the systemic onset JIA. According adult classification 61,5 % of patients with positive HLA-B27 antigen in adulthood developed ankylosing spondilitis or undifferentiated spondiloarthritis, in 22,7 % – undifferentiated arthritis, 100 % – psoriatic arthritis and 31,8 % – remission of the disease. In the childhood in HLA-B27 (+) patients, symmetrical joint damage (20,5 %, p<0,0001), enthesitis (20,5 %, p<0,05), lesion of the joints of the hands (26,4 %, p<0,05), defeat of more than 3 peripheral joints (36,8 %, p <0,05) and longer morning stiffness (Ме 40 [20; 60] min, p<0,001) were observed more often, compared with adult age. In adulthood, pain in the spine was significantly more frequent (27,5 %, p<0,01), as well as sacroilitis (15,0 %, p<0,05) and oligoarthritis (45 %, p<0,01). Only 21 % HLA-B27-positive patients received NSAIDs, 26,3 % had one DMARD and NSAIDs, 31,6 % had more than one DMARDs, and 21,1 % had a combination of different DMARDs and biological therapy (BTs). In childhood 58,3 % of patients received glucocorticoids and in adulthood only 22,2 % of patients but this difference was not significant. 42,1 % of adults needed intensification of therapy, 26,3 % of patients required BT. The most significant joint damages (JADI-A) in adulthood were found in the anti-CCP/RF-positive patients (3,04±4,90), whereas HLA-B27- positive patients had the lowest rates of this index, that shows the development of less remote negative consequences. Extra-articular damages (JADI-E) were most pronounced in АNА-positive patients (1,31±1,49), compared with a more favorable course in the groups anti-CCP/RF- (0,38±0,70; p <0,05) and HLA-B27-positive (0,50±1,06; p <0,05) patients. Conclusion. Clinical manifestations of articular syndrome have certain age-related pecularities in HLA-B27-positive patients with JIA: symmetrical joint damage, enthesitis, lesion of the joints of the hands, affections of more than 3 peripheral joints, and more prolonged morning stiffness are observed in childhood but in adulthood, pain in the spine, sacroilitis and oligoarticular lesion are more common. The presence of HLA-B27 antigen in patients with JIA is associated with the development of a smaller number of long-range articular damage (JADI-A), compared to the anti-CCP/RF-positive group (p <0,05) and less of the remote extra-articular effects (JADI- E) compared with the group of ANA-positive patients (p <0,05) in an adulthood.

2018 ◽  
Vol 13 (3-4) ◽  
pp. 28-37
Author(s):  
M.B. Dzhus ◽  
T.V. Marushko ◽  
H.V. Mostbauer ◽  
О.І. Ivashkivsky ◽  
I.V. Kurylchyk ◽  
...  

Relevance. It is known that the poly-articular variant of JIA is associated with significant articular and extra-articular damages with predominant lesions of small and medium joints with limitation of their function and the development of ankylosis, requiring surgical intervention in these patients. Objective. The aim of the study was to evaluate the risk factors of articular and extra-articular damages in adults with the poly-articular variant of the JIA and to work out a prognostic model for their development. Materials and methods. The study included 45 adult patients with poly-articular (RF+ and RF-) variant of the JIA. Retrospective analysis of medical records of patients with poly-articular variant of JIA was made, taking into account clinical manifestations in the onset of the disease, laboratory parameters and response to therapy. In adulthood, the integral index of articular (JADI-A) and extra-articular (JADI-E) damages was used to assess the long-term effects of JIA. Results. Unfavorable prognostic factors were revealed by method of binary logistic regression. Mathematical model for predicting the probability of long-term negative articular and extra-articular damages of poly-articular JIA was developed. Conclusions 1. The risk factors for the development of articular and extra-articular damages in adulthood in patients with a poly-articular variant of the JIA are female sex, the presence of lymphadenopathy and/or splenomegaly in childhood, the level of ESR during treatment in childhood, the activity of JADAS in childhood, the presence of symmetric arthritis in childhood, the RF positivity in the debut of the disease, the treatment with glucocorticoids, the cumulative dose of the DMARDs, and lack of therapy by DMARDs. 2. A prognostic model for the development of articular and extra-articular damages in adult patients with a poly-articular variant of the JIA is developed. This model is effective and allows determine the adverse course of the disease and can be the basis for personalized treatment for the prevention of the development of significant articular and extra-articular damages of JIA.


Haemophilia ◽  
2021 ◽  
Author(s):  
Víctor Jiménez‐Yuste ◽  
Hortensia de la Corte‐Rodríguez ◽  
María Teresa Álvarez‐Román ◽  
Mónica Martín‐Salces ◽  
Felipe Querol ◽  
...  

2021 ◽  
Author(s):  
Haron Obaid ◽  
Stephan Milosavljevic ◽  
Udoka Okpalauwaekwe ◽  
Brenna Bath ◽  
Catherine Trask ◽  
...  

Abstract Background. Detection of ankylosing spondylitis (AS) in the preclinical stage could help prevent long term morbidity in this patients’ population. The aim of this study was to examine the prevalence of active sacroiliitis in first-degree relatives of AS patients using MRI with clinical and laboratory correlations as these patients may benefit from MRI screening and early treatment.Methods. Seventeen first-degree relatives of AS patients were recruited prospectively. AS screening questionnaires (Ankylosing Spondylitis Disease Activity Score, Bath Ankylosing Spondylitis Disease Activity Index & Visual Analogue Scale), blood tests (C-Reactive Protein, HLA-B27), and an MRI of the SIJs were taken. Two musculoskeletal radiologists interpreted the MRI scans, and two physiotherapists applied four symptom provocation tests (Gaenslen's test, posterior pelvic pain provocation test, Patrick's Faber (PF) test and palpation of the long dorsal SIJ ligament test), and two functional movement tests (active straight leg raise and Stork test). Results. Seven (41%) of the 17 participants demonstrated MRI evidence of active sacroiliitis. Of the 7 participants with active sacroiliitis, two (29%) had no history of recent low back pain (LBP), two (29%) had negative HLA-B27, and one (14%) participant had neither back pain nor positive HLA-B27. The Cohen's Kappa score for the interobserver agreement between the radiologists was 1.00 (p-value <0.0001). Despite fair to strong between therapist agreement for the physical test outcomes (Kappa 0.26 to 1.00), the physical test results per se did not have any predictive association with a positive MRI.Conclusions. MRI detected active sacroiliitis in 41% of first-degree relatives of AS patients. The lack of a history of prior LBP or positive HLA-B27 in active sacroiliitis participants might suggest that MRI screening for this high-risk population is warranted; however, further larger studies are needed to help elucidate its cost-effectiveness and long-term benefits.


Diagnostics ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. 686
Author(s):  
Anna Degtyareva ◽  
Alexander Razumovskiy ◽  
Nadezhda Kulikova ◽  
Sergey Ratnikov ◽  
Elena Filippova ◽  
...  

This prospective study enrolled 144 patients after surgical treatment of biliary atresia in early infancy. We analyzed the immediate effectiveness of the surgery and the age-related structure of complications in the up to 16-year follow-up. The immediate 2-year survival rate after the surgery constituted 49.5%. At the time of this writing, 17 of the patients had celebrated their 10th birthdays with good quality of life and no indications for transplantation of the liver. The obtained results underscore the critical importance of surgical correction of biliary atresia by Kasai surgery in the first 60 days of life and subsequent dynamic follow-up of patients for the purpose of the early detection and timely correction of possible complications.


RMD Open ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. e000852 ◽  
Author(s):  
Hilal Ince-Askan ◽  
Erica L T van den Akker ◽  
Yolanda B de Rijke ◽  
Elisabeth F C van Rossum ◽  
Johanna M W Hazes ◽  
...  

ObjectivesTo identify whether children with antenatal prednisone exposure have chronically elevated cortisol and cortisone concentrations, an altered body composition or higher blood pressure. In addition, to identify whether maternal rheumatoid arthritis disease (RA) activity is associated with these alterations.MethodsIn this prospective study, 56 children (mean age=10.0 years) with and 61 children (mean age=9.6 years) without antenatal prednisone exposure, born to women with RA, were included. Hair cortisol and cortisone were analysed using liquid chromatography–tandem mass spectrometry. Linear regression models were built to analyse differences between the two groups, corrected for relevant covariates. Hair cortisol concentrations were also compared between the study population and an age-matched healthy reference group(n=150 children, mean age=9.8 years).ResultsHair cortisol and cortisone concentrations were similar in children with and without antenatal prednisone exposure (median cortisol 1.14 pg/mg (IQR 0.67–1.75) and 1.15 pg/mg (IQR 0.65–2.21) and median cortisone 6.76 pg/mg (IQR 5.42–8.86) and 7.40 pg/mg (IQR 5.39–10.73), respectively). Antenatal prednisone exposure and maternal RA disease activity were also not associated with body composition or blood pressure. Hair cortisol concentrations were not different in children born to mothers with RA compared with children from the reference group.ConclusionThis, in its kind, large and unique long-term prospective study demonstrates that low-dose antenatal prednisone exposure and maternal RA disease activity are not associated with negative consequences in prepubertal childhood. The findings of this study are reassuring and support the assumption that low-dose maternal prednisone use during pregnancy is safe for the offspring, at least until the age of approximately 10 years.


2006 ◽  
Vol 8 (4) ◽  
pp. 750-755 ◽  
Author(s):  
Marja Äikiä ◽  
Leena Jutila ◽  
Tuuli Salmenperä ◽  
Esa Mervaala ◽  
Reetta Kälviäinen

Nutrients ◽  
2020 ◽  
Vol 12 (5) ◽  
pp. 1416
Author(s):  
Hideaki Oike ◽  
Yukino Ogawa ◽  
Kayo Azami

High-fat diets (HFD) have been thought to increase the risk of obesity and metabolic syndrome, as well as shorten lifespan. On the other hand, chrono-nutritional studies have shown that time-restricted feeding during active phase significantly suppresses the induction of HFD-induced obesity in mouse model. However, the long-term effects of time-restricted HFD feeding on aging are unknown. Therefore, in this study, we set up a total of four groups: mutual combination of ad libitum feeding or night-time-restricted feeding (NtRF) and an HFD or a control diet. We examined their long-term effects in a senescence-accelerated mouse strain, SAMP8, for over a year. Hearing ability, cognitive function, and other behavioral and physiological indexes were evaluated during the study. Unexpectedly, SAMP8 mice did not show early onset of death caused by the prolonged HFD intake, and both HFD and NtRF retarded age-related hearing loss (AHL). NtRF improved grip strength and cognitive memory scores, while HFD weakly suppressed age-related worsening of the appearance scores associated with the eyes. Notably, the HFD also retarded the progression of AHL in both DBA/2J and C57BL/6J mice. These results suggest that HFD prevents aging unless metabolic disorders occur and that HFD and NtRF are independently effective in retarding aging; thus, the combination of HFD and chrono-nutritional feeding may be an effective anti-aging strategy.


2020 ◽  
Vol 12 (11) ◽  
pp. 4699 ◽  
Author(s):  
Cristina Bianca Pocol ◽  
Valentina Marinescu ◽  
Antonio Amuza ◽  
Roxana-Larisa Cadar ◽  
Anda Anca Rodideal

The importance of studying the aspects related to the sustainable food consumption behaviour of students lies in the fact that, at this age, they begin to develop certain consumption patterns that will have long-term effects. The study aimed to identify the type of eating behaviour—sustainable vs. unsustainable—and the socio-demographic factors that influence it, among students in Romania, Bulgaria, and Moldova. The research method was a survey based on a questionnaire administered to a sample of 2378 subjects in the 2017–2018 period. The ANOVA test and simple linear regression were used to identify the correlation between the various variables analysed. The results indicate both positive aspects, which characterise a sustainable diet—high consumption of fruits and vegetables, and negative—the adoption of a mixed diet, which will have a long-term impact on the environment. Age is a good predictor of unhealthy eating habits among students, as this behaviour leads to weight gain. Gender, number of household members, rural/urban and country residence are also influencing factors for food consumption behaviour. Eating while standing and having the last meal of the day after 23:00 are practices that also have negative consequences for health. Conducting campaigns to educate students on the adoption of sustainable food consumption is necessary for all three countries to empower them to choose a healthy lifestyle.


Author(s):  
Brian O'Mahony ◽  
Gerard Dolan ◽  
Diane Nugent ◽  
Clifford Goodman

INTRODUCTION:Hemophilia is a rare, inherited bleeding disorder affecting an estimated 400,000 people worldwide (1). Characterized by spontaneous bleeding and long-term, irreversible joint damage, persons with hemophilia are often limited in normal day-to-day activities, including work/school, and require comprehensive care at specialized treatment centers. With replacement therapies extending survival by decades and vastly improving quality of life (QoL), routine prophylaxis is considered the standard-of-care in developed countries. However, due to the cost of replacement factor, access to treatment remains a challenge, and increased scrutiny over funding has been augmented by growing demands on healthcare budgets (2). Thus, the hemophilia community shares a unified goal of objectively defining patient-centered value in hemophilia care.METHODS:Using a three-tiered outcomes hierarchy model initially described by Porter (3), an international, multidisciplinary panel of health economics outcomes researchers and hemophilia experts developed a value framework for decision makers to assess value of various healthcare interventions in hemophilia.RESULTS:The three tiers for assessing value are: (i) Health status achieved/retained; (ii) Process of recovery; and (iii) Sustainability of health. Tier one measures survival, quality of life (QoL), and hemophilia-specific outcomes of bleeding frequency, musculoskeletal complications, and severe bleeds, as well as function/activity (that is, lifestyle impairment). Tier two measures time to initial treatment or recovery and time missed at education/work, as well as disutility of care (that is, inhibitor development, pathogen transmission/infections, orthopedic intervention, and venous access). Tier three measures avoidance of bleeds, maintenance of productive lives, and long-term health, while capturing long-term consequences of insufficient therapy or age-related complications. Applicability of the framework can be demonstrated in areas of healthcare delivery, treatment regimen, and innovation for new therapies.CONCLUSIONS:This value framework represents an initial collaboration with stakeholders to define and organize an array of patient-centric outcomes of importance in hemophilia into a practical tool that can influence treatment and funding decisions in hemophilia care.


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