scholarly journals Fibrous dysplasia patients with and without craniofacial involvement report reduced quality of life inclusive of stigma, depression, and anxiety

2021 ◽  
pp. 174239532110494
Author(s):  
Amanda Konradi

Objectives Fibrous dysplasia is a rare bone disorder that causes deformity, fractures, and pain that typically manifests in childhood and persists as a chronic illness. This study evaluates adult patients with fibrous dysplasia and McCune Albright syndrome to determine whether their quality of life differs from the general population and varies in relation to disease severity and lesion location. Methods This study uses data from the online self-report Fibrous Dysplasia Foundation Patient Registry and operationalizes quality of life using PRO measures: SF-36, Hospital Anxiety and Depression scale, Neuro-Quality of Life Stigma scale, and the Brief Pain Inventory. Results One hundred and ninety seven adults, 90% white, 84% women, constitute the sample. Mean scores for all SF-36 domains and the Neuro Q stigma scale were significantly below population benchmarks. A large minority registered moderate to severe levels of anxiety and depression. Group differences were not significant across most of the SF-36 domains but were associated with experienced stigma. Discussion This study demonstrates a social psychological impact of fibrous dysplasia on adults, in those with and without craniofacial involvement and with mild and severe forms of the disease. Clinical treatment should encompass assessment of quality of life issues and ensure access to psychosocial treatment resources for all fibrous dysplasia/McCune-Albright syndrome patients.

2015 ◽  
Author(s):  
Damla Gokeen ◽  
Samim Ozen ◽  
Nurhan Ozcan ◽  
Sukran Darcan

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1011.2-1011
Author(s):  
Y. Olyunin ◽  
V. Rybakova ◽  
E. Likhacheva ◽  
E. Nasonov

Background:The patient-reported outcomes are important components of quantitative methods of rheumatoid arthritis (RA) activity assessment which are used to choose the appropriate drug therapy. The value of these parameters can be significantly affected not only by the inflammatory process, but also by the psychological characteristics of the patient and, in particular, by hardiness [1].Objectives:To study the relationship between psychological factors and signs of RA activity.Methods:Patients with RA who met the EULAR/ACR 2010 criteria, and observed at the V. A. Nasonova Research Institute of Rheumatology were included. Clinical examination was performed including patient global assessment (PGA), physician global assessment (PhGA), pain measurement on a visual analog scale, tender joint count (TJC), swollen joint count (SJC). The functional status was determined by HAQ, the quality of life – by SF-36 EQ-5D, the nature of pain – by painDETECT, the presence of anxiety and depression – by HADS. Patients also completed Hardiness Survey questionnaire to assess hardiness (HDS) and 3 components of the HDS – commitment (CMT), control (CT) and challenge (CLN). Disease activity was evaluated with DAS28, CDAI, and RAPID3. All patients signed informed consent to participate in the study. Analysis of the data was performed using Spearman’s rank test, Fisher exact test, qui-square and t-tests.Results:85 patients with RA were included. There were 69 women and 16 men. Mean age was 56.7±13.1 years, disease duration – 7.6±2.7 years. 72 patients were positive for rheumatoid factor, 75 – for anti-cyclic citrullinated peptide antibody. CDAI showed high activity in 15, moderate – in 37, low – in 30, and remission in 3 patients, DAS 28 – in 10, 55, 12, and 8, and RAPID3 – in 24, 25, 15, and 21, respectively. 24 patients had subclinically or clinically expressed anxiety and 15 –subclinically or clinically expressed depression (≥8 according to HADS). In 31 patients, the painDETECT questionnaire revealed possible or probable neuropathic pain. Mean HDS was 84.8±21.7, CMT – 38.9±9.2, CT – 29.4±8.6, CLN – 17.3±7.1. These values were comparable with the corresponding population data for this age group. There was a significant inverse correlation between HDS and RA activity measures, including SJC, TJC, DAS28 (p<0.05), pain, PGA, PhGA, CDAI, RAPID3, and HAQ (p<0.01). In addition, HDS and all its components positively correlated with quality of life, assessed by SF-36 and EQ-5D (p<0.01). In patients with subclinically and clinically expressed anxiety and depression, HDS, CMT, and CT were significantly lower than in patients without anxiety and depression (p<0.01), while the values of CLN in these groups did not differ significantly.Conclusion:The results of the present study suggest that low HDS may be one of the significant factors determining RA activity level because it does not allow patients to adapt adequately to a stressful situation produced by the disease.References:[1]Maddi SR. Am Psychol. 2008 Sep;63(6):563-4.Disclosure of Interests:None declared


Oral Diseases ◽  
2004 ◽  
Vol 10 (1) ◽  
pp. 36-43 ◽  
Author(s):  
SO Akintoye ◽  
LL Otis ◽  
JC Atkinson ◽  
J Brahim ◽  
H Kushner ◽  
...  

2016 ◽  
Vol 89 (4) ◽  
pp. 559-564
Author(s):  
Iulian Raus ◽  
Roxana Elena Coroiu

McCune–Albright syndrome is a rare sporadic disease characterized by bone fibrous dysplasia, café-au-lait skin spots and a variable association of hyperfunctional endocrine disorders. Fibrous dysplasia (FD), which can involve the craniofacial, axial, and appendicular skeleton, may range from an isolated, asymptomatic monostotic lesion to a severe disabling polyostotic disease involving the entire skeleton. A twenty-five-year old male patient presented to our clinic with recently developed heart palpitations. He had also been feeling pain in the right femur since he was younger, without any trauma history, leading to difficulties of ambulation and limping occasionally. His physical examination revealed café-au-lait spots with irregular borders and right testicular agenesis. Laboratory findings identified hyperthyroidism with hyperparathyroidism. Radiographs of the pelvis revealed multiple lytic lesions of the right femur and magnetic resonance imaging (MRI) characterized these lesions as specific to fibrous dysplasia of the bone, without any insufficiency fracture at this level.The association of café-au-lait skin spots with bone fibrous dysplasia, and hyperthyroidism in this patient suggested the diagnosis of McCune – Albright syndrome.


2021 ◽  
Vol 13 (1S) ◽  
pp. 45-49
Author(s):  
M. E. Guseva ◽  
O. V. Matvievskaya ◽  
A. N. Boyko

Objective: to study the effectiveness of art therapy methods in the complex treatment of neuropsychological disorders and quality of life improvement in patients with relapsing-remitting and secondary-progressive multiple sclerosis (MS).Patients and methods. A group of 42 patients (5 men and 37 women, mean age 32.9 years, mean EDSS severity 3.8 points) with MS underwent outpatient continuous active art therapy cycle. The mean group training cycle duration was six months. The comparison group included 15 age- and sex-adjusted individuals without neurological disorders. Tests on various drawing topics were carried out before and after a three-month therapy course. We used Hospital Anxiety and Depression Scale (HADS) to assess the severity of anxiety and depression and the SF-36 scale to evaluate the quality of life (QoL).Results and discussion. Before the art therapy start, light blue, yellow, pink, blue, and green colors prevailed in the drawings. There was no predominance of any color depending on MS severity or course. The green color predominated in this test in the control group, and the light blue color was significantly less represented. The art therapy course did not lead to a significant change in the severity of MS according to the Expanded Disability Status Scale (EDSS). After the art therapy course, the color scheme of the drawings was mainly represented by red, light blue, green, yellow, and blue colors. In addition, a red-orange color appeared in the drawings, which was completely absent before the course. Positive trends were noted according to HADS: a decrease in anxiety by 23% and depression by 19%. The physical component of QoL assessed by the SF-36 scale did not change substantially, but the psychological component significantly improved by 11%.Conclusion. The predominance of light blue color observed in MS is associated with damage to the nervous and musculoskeletal systems, and blue color – with psychological problems and depression. However, after a course of art therapy for MS, red, red-orange, yellow, and green colors representation increased significantly, which indicates an increase in positive attitude, a decrease in the level of depression, and an improvement in QoL.


2018 ◽  
Author(s):  
Jinrong Zhao ◽  
Jinguo Yu

Abstract Background: McCune-Albright syndrome (MAS) is a rare disease defined by the triad of polyostotic fibrous dysplasia of bone, skin spots, and precocious puberty. No available treatment is effective in changing the course of fibrous dysplasia of bone, but symptomatic patients require the rapeutic support to reduce bone pain and prevent fractures and deformities.we reported 1 case of McCune-Albright syndrome with exophthalmos in clinical practice. Case presentation:A 35-year-old female was admitted to our hospital who complained about “skin pigmentation for 35 years, vaginal bleeding for 30 years and progressive skeletal deformity for 28 years and exophthalmos for 2 years. And after the examination, she was been diagnosed with“McCune-Albright syndrome with exophthalmos”.We highlighted the pathogenesis and development of the disease in this rare condition. Conclusion: McCune-Albright syndrome with exophthalmos due to multiple fibrous dysplasia is rare but can be seen in clinical practice.


2020 ◽  
Vol 40 (2) ◽  
pp. 134-137
Author(s):  
Subhana Thapa Karki ◽  
Vandana Jain

McCune Albright syndrome (MAS) is a very rare genetic disease characterised by any two of the following three findings: café au lait spots, polyosteotic fibrous dysplasia and endocrine disorders. The clinical presentation of MAS may vary depending on which of the various components of the syndrome predominate. Here, we report one case of MAS presenting with precocious puberty.


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