scholarly journals Clinical Courses of IKAROS and CTLA4 Deficiencies: A Systematic Literature Review and Retrospective Longitudinal Study

2022 ◽  
Vol 12 ◽  
Author(s):  
Akihiro Hoshino ◽  
Etsushi Toyofuku ◽  
Noriko Mitsuiki ◽  
Motoi Yamashita ◽  
Keisuke Okamoto ◽  
...  

IKAROS and CTLA4 deficiencies are inborn errors of immunity and show similar clinical phenotypes, including hypogammaglobulinemia and autoimmune diseases (ADs). However, the differences in clinical features and pathogenesis of these are not fully understood. Therefore, we performed systematic literature reviews for IKAROS and CTLA4 deficiencies. The reviews suggested that patients with IKAROS deficiency develop AD earlier than hypogammaglobulinemia. However, no study assessed the detailed changes in clinical manifestations over time; this was likely due to the cross-sectional nature of the studies. Therefore, we conducted a retrospective longitudinal study on IKAROS and CTLA4 deficiencies in our cohort to evaluate the clinical course over time. In patients with IKAROS deficiency, AD and hypogammaglobulinemia often develop in that order, and AD often resolves before the onset of hypogammaglobulinemia; these observations were not found in patients with CTLA4 deficiency. Understanding this difference in the clinical course helps in the clinical management of both. Furthermore, our results suggest B- and T-cell-mediated ADs in patients with IKAROS and CTLA4 deficiencies, respectively.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ellen E. Lee ◽  
Tushara Govind ◽  
Marina Ramsey ◽  
Tsung Chin Wu ◽  
Rebecca Daly ◽  
...  

AbstractThere is growing interest in the role of compassion in promoting health and well-being, with cross-sectional data showing an inverse correlation with loneliness. This is the first longitudinal study examining both compassion toward others (CTO) and compassion toward self (CTS) as predictors of mental and physical health outcomes including loneliness, across adult lifespan. We followed 552 women and 538 men in San Diego County for up to 7.5 (mean 4.8 and SD 2.2) years, using validated rating scales for CTO, CTS, and loneliness. Linear mixed-effects models were employed to examine age- and sex-related trajectories of CTO and CTS over time. Linear regression models were used to evaluate baseline and longitudinal relationships of CTO and CTS with mental well-being, physical well-being, and loneliness. CTS and CTO were weakly intercorrelated. Women had higher baseline CTO than men. While CTO was stable over time and across the lifespan, CTS scores had an inverse U-shaped relationship with age, peaking around age 77. There were significant baseline × slope interactions of both CTO and CTS predicting improvements in physical well-being in adults <60 years old. Increases in CTO and CTS predicted improvements in mental well-being. Higher baseline CTO and CTS as well as increases in CTO and CTS scores predicted lower loneliness scores at follow-up. Thus, CTO and CTS were associated with better mental well-being and loneliness across the adult lifespan, and physical well-being in younger adults, and are promising targets for interventions to improve health outcomes.


Lupus ◽  
2020 ◽  
Vol 29 (10) ◽  
pp. 1216-1226
Author(s):  
Beatriz Frade-Sosa ◽  
Javier Narváez ◽  
Tarek Carlos Salman-Monte ◽  
Raul Castellanos-Moreira ◽  
Vera Ortiz-Santamaria ◽  
...  

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.


2019 ◽  
Vol 48 (6) ◽  
pp. 766-776
Author(s):  
Andrew P. Hill ◽  
Karen Burland ◽  
Elaine C. King ◽  
Stephanie E. Pitts

Research has shown that perfectionism predicts emotional experiences among amateur, professional and adolescent musicians. In examining these relationships, previous research has measured trait perfectionism and employed cross-sectional designs. The current study builds on existing research by examining whether perfectionistic self-presentation (as opposed to trait perfectionism) predicts negative and positive emotional experiences in music students over time. One hundred and forty-three music students ( M age 18.92 years, SD = 2.96) enrolled in music-related degree programs completed measures of perfectionistic self-presentation (perfectionistic self-promotion, non-display of imperfection, and non-disclosure of imperfection) and emotional experiences (positive and negative feelings) at the start, middle and the end of the academic year. Path analysis revealed that perfectionistic self-promotion at the start of the year predicted lower positive feelings in the middle of the year, and non-disclosure of imperfection in the middle of the year predicted lower positive feelings at the end of the year. In addition, negative feelings in the middle of the year also predicted higher non-disclosure of imperfection at the end of the year. The findings suggest that the desire to present oneself perfectly and avoid disclosure of imperfections may contribute to less positive emotional experiences among music students.


Author(s):  
Judith G. Chipperfield ◽  
Betty Havens ◽  
Wendy D. Doig

ABSTRACTThe Aging in Manitoba (AIM) project includes nearly 9,000 seniors who have been interviewed. Three independent cross-sectional samples (i.e. 1971, 1976, 1983) were subsequently followed (1983–1984, 1990), producing cross-sectional and longitudinal data. A major goal of this paper was to examine non-response over time and representativeness of the panel of AIM survivors. This involved analysis of non-response, calculations of completion/response rates, and comparisons of demographic variables across the AIM sample and the broader Manitoba and Canadian populations. Non-response was very low (4.6% in 1983–1984, 5.0% in 1990) and did not appear to be dependent on such factors as location, gender, education level, or nationality descent. The 95.0 per cent completion rate compares favourably with other longitudinal studies, demonstrating the value of employing rigorous tracking procedures. These findings suggest that attempts to minimize bias and selective attrition in the AIM study have been successful.


2020 ◽  
Vol 25 (1) ◽  
pp. 71-74
Author(s):  
A. I. Bulgakova ◽  
Z. R. Hismatullina ◽  
M. V. Zatsepina ◽  
J. A. Kudryavtseva

Relevance. Multiform exudative erythema is a polyetiological disease, which is characterized by damage not only to the skin, but also to the mucous membranes, mainly the mucous membrane of the oral cavity. The article reviews the literature on the clinical course of multiform exudative erythema of the oral cavity.Purpose. Study the clinical course of erythema multiforme.Materials and methods. We conducted a review of studies of the etiology, pathogenesis, prevalence of erythema multiforme according to domestic and foreign authors, and also reflected the identification of various forms and clinical manifestations of this disease.Results. The analysis of clinical manifestations of erythema multiforme exudative in the oral cavity, the most common symptoms and manifestations on the oral mucosa.Conclusion. The clinical picture of lesions of the oral mucosa in multiform exudative erythema is very diverse, which is important for the timely diagnosis of the disease.


Author(s):  
G. R. Kozonov

The article presents the literature review of current conception of development, etiology, pathogenesis, clinical manifestations and management of discoordination in labor activity. Special attention is payed to the clinical course of the labor and its outcome and for morhpofunctional features of the myometrium in this category of patients.


2020 ◽  
Vol 46 (3) ◽  
Author(s):  
V. A. Kashirin ◽  
A. N. Shcherbina ◽  
N. V. Rudik

Abstract Sinonasal neuroblastoma is a rare malignant tumor with a wide variability of clinical manifestations, which may cause diagnostic difficulty and have hindered the progress in understanding the clinical course and improving outcomes of treatment. Patients often present with nasal obstruction, rhinorrhea, recurrent epistaxis, hyposmia, or anosmia. Treatment options consist of surgical resection and before or followed radiation and chemotherapy. We present a case report and short literature review about diagnostic, clinical and histological classification and treatment of this tumor. Keywords: neuroblastoma, nasal cavity, diagnostic, classification, treatment.


2007 ◽  
Vol 21 (4) ◽  
pp. 471-485 ◽  
Author(s):  
Abraham P. Buunk ◽  
José Maria Peíró ◽  
Isabel Rodríguez ◽  
M. Jesús Bravo

This longitudinal study among Spanish teachers (N = 558) examined burnout from an evolutionary social rank perspective. The types of stress differed among various school levels, and a sense of defeat was highest among teachers in primary schools. A low status, a loss of status and a sense of defeat were independent cross‐sectional predictors of burnout. Longitudinally a sense of defeat predicted an increase in burnout over time, especially among males and teachers in high schools. The status related variables were in general more important predictors of burnout than stressors that are usually associated with burnout. Copyright © 2007 John Wiley & Sons, Ltd.


2021 ◽  
pp. 194855062098846
Author(s):  
Alexandra N. Fisher ◽  
Danu Anthony Stinson ◽  
Joanne V. Wood ◽  
John G. Holmes ◽  
Jessica J. Cameron

Romantic relationships activate a process of psychological attunement whereby self-esteem becomes responsive to the romantic bond, thereby potentially benefitting relationship quality and bolstering self-esteem. Yet some people are romantically single, raising the question: Do single people also exhibit psychological attunement? In a 2-year longitudinal study of young adults ( N = 279), we test whether singles psychologically attune to their friendships. Multilevel modeling revealed that within-person fluctuations in friendship quality predicted within-person fluctuations in self-esteem, and this association was stronger for singles than for partnered people. A cross-sectional mediation analysis also revealed that singles invested more in their friendships than partnered people, and greater friendship investment predicted greater friendship quality and self-esteem later on. Finally, singles maintain their friendship quality over time while partnered people experience declines. Taken together, these results suggest that singles are psychologically attuned to their friendships, and such attunement may benefit their belongingness and self-esteem.


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Vishaka K. Chetram ◽  
Akram I. Ahmad ◽  
Saira Farid ◽  
Tanuj Sood

The viral infection causing COVID-19 most notably affects the respiratory system but can result in extrapulmonary clinical manifestations as well. Rhabdomyolysis-associated acute kidney injury (AKI) in the setting of COVID-19 is an uncommon complication of the infection. There is significant interest in this viral infection given its global spread, ease of transmission, and varied clinical manifestations and outcomes. This case report and literature review describes the symptoms, laboratory findings, and clinical course of a patient who developed AKI secondary to rhabdomyolysis and COVID-19, which will help clinicians recognize and treat this condition.


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