scholarly journals Reducing the Burden of Care: Multidisciplinary Management of Late-Manifested Crouzon Syndrome—A Case Report

Children ◽  
2021 ◽  
Vol 8 (12) ◽  
pp. 1122
Author(s):  
Sarah Achterrath ◽  
Teresa Kruse ◽  
Julia Neuschulz ◽  
Isabelle Graf ◽  
Joachim Zöller ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Case Report ◽  
Psychosocial Development ◽  
Young Patients ◽  
Crouzon Syndrome ◽  
Burden Of Care ◽  
Short Treatment ◽  
Related Quality ◽  
Health Related

The therapy of patients with Crouzon syndrome involves a multidisciplinary team. In most cases, this therapy is extensive, time-consuming, and exhausting for the patient. This case report illustrates a temporally coordinated therapy plan that succeeds in reducing the burden of care. Showing typical extraoral characteristics of Crouzon syndrome, the patient had a frontal and left-sided crossbite, and impaction of the maxillary canines. Multidisciplinary therapy included the extraction of multiple teeth, midface distraction at Le Fort III level, and alignment of the impacted teeth. Before starting, during, and after completion of the treatment, the patient’s oral health-related quality of life was assessed using COHIP-19. The combination of different treatment steps significantly reduced the duration of therapy. The therapy improved not only the patient’s oro- and craniofacial function, but also the patient’s facial appearance in a short treatment period. The patient’s quality of life improved considerably during this time. In the treatment of severe craniofacial anomalies, the highest priority should be given to keeping the burden of care low. All measures should encourage young patients’ appropriate psychosocial development despite extensive therapies, ensuring at the same time medically satisfactory treatment results.

Clinical phenotype, fibrinogen supplementation and health-related quality of life in patients with afibrinogenemia

Blood ◽  
2021 ◽  
Author(s):  
Alessandro Casini ◽  
Sylvia von Mackensen ◽  
Cristina Santoro ◽  
Claudia Djambas Khayat ◽  
Meriem Belhani ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Family Relationships ◽  
Thrombotic Event ◽  
Young Patients ◽  
Severe Bleeding ◽  
Health Related Quality ◽  
Cross Sectional ◽  
Related Quality ◽  
Health Related

Due to its low prevalence, epidemiologic data on afibrinogenemia are limited and none are available on health-related quality of life (HRQoL). We conducted a cross-sectional international study to characterize the clinical features, the fibrinogen supplementation modalities and their impact on HRQoL in patients with afibrinogenemia. A total of 204 patients (119 adults and 85 children) from 25 countries were included. The bleeding phenotype was severe: 68 (33.3%) patients having at least one bleed per month and 48 (23%) a history of cerebral bleeding. About 35% (n=72) of patients were treated with fibrinogen concentrates or cryoprecipitates as prophylaxis, 18.1% (n=37) received more than one injection per week and 16.6% (n=34) were on home treatment. A thrombotic event was reported in venous and/or arterial territories by 37 (18.1%) patients. Thrombosis occurred even in young patients and recurrence was frequent (7.4%). The total HRQoL was lower in children than in adults. Discomfort linked to treatment and limitations to sports and leisure were the main concerns. Women and children were particularly affected in family relationships. In multivariate analyses, younger age, residence in Asia or Africa and a previous thrombotic event were statistically correlated with a worse HRQoL. In conclusion, our study underlines the severe bleeding and thrombotic phenotype and their impact on HRQoL in afibrinogenemia. The optimal strategy for fibrinogen supplementation needs to be determined.

Predictors of health-related quality of life in children with chronic heart disease

2017 ◽  
Vol 27 (8) ◽  
pp. 1455-1464 ◽  
Author(s):  
Mandy Niemitz ◽  
Diana C. M. Gunst ◽  
Hedwig H. Hövels-Gürich ◽  
Michael Hofbeck ◽  
Renate Kaulitz ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Heart Disease ◽  
Children And Adolescents ◽  
Psychosocial Development ◽  
Health Related Quality ◽  
Chronic Heart Disease ◽  
Related Quality ◽  
Health Related ◽  
Cardiac Medication

AbstractObjectiveChronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease.Patients and methodsThe Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers.ResultsGreater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart–lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child’s health-related quality of life.ConclusionsBeyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.

Impact of Oral Mucositis on Oral Health-Related Quality of Life of Young Patients Diagnosed With Cancer

2015 ◽  
Vol 120 (2) ◽  
pp. e104
Author(s):  
JAIR CARNEIRO LEÃO ◽  
ANDREZA BARKOKEBAS SANTOS DE FARIA ◽  
DEBORAH FONSECA ◽  
ADRIANA MACHADO ZARZAR ◽  
LUIZ ALCINO MONTEIRO GUEIROS ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Oral Health ◽  
Oral Mucositis ◽  
Young Patients ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

Children with a History of Premature Adrenarche Have Good Health-Related Quality of Life at the Age of 12 Years

2018 ◽  
Vol 89 (3) ◽  
pp. 184-188
Author(s):  
Jani Liimatta ◽  
Harri Sintonen ◽  
Pauliina Utriainen ◽  
Raimo Voutilainen ◽  
Jarmo Jääskeläinen
Keyword(s):  
Quality Of Life ◽  
Psychosocial Development ◽  
Pubertal Development ◽  
Reference Population ◽  
Health Related Quality ◽  
Premature Adrenarche ◽  
Related Quality ◽  
Health Related ◽  
And Control

Background/Aim: Children with premature adrenarche (PA) are taller and more overweight than their healthy peers, and PA girls have a slightly accelerated pubertal development. There is some evidence that early exposure to androgens may have an influence on psychosocial development. The aim of this cross-sectional case-control study was to evaluate health-related quality of life (HRQoL) in PA children at the age of 12 years. Methods: The HRQoL was assessed for 43 PA (36 girls) and 63 control children (52 girls) at the median age of 12.0 years using the standardized 16D instrument, and the scores of the PA children were compared to those of the control children and reference population. Results: The mean overall HRQoL scores did not differ between PA and control girls, PA and control boys, or all PA and control children or the reference population. Independently of PA, overweight girls had a lower mean overall HRQoL score than lean girls, and both overweight girls and boys were on average worse off on the dimension of appearance than their lean peers. Conclusions: PA children have as good self-rated HRQoL as their peers at the age of 12 years. Overweight is associated with a worse HRQoL profile independently of PA.

scholarly journals Health-Related Quality of Life in Patients with Chronic Myeloid Leukemia Treated with First- Versus Second-Generation Tyrosine Kinase Inhibitors

2020 ◽  
Vol 9 (11) ◽  
pp. 3417
Author(s):  
Adi Shacham Abulafia ◽  
Sivan Shemesh ◽  
Lena Rosenmann ◽  
Tamar Berger ◽  
Avi Leader ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Kinase Inhibitors ◽  
Young Patients ◽  
Current Treatment ◽  
European Organization ◽  
Related Quality ◽  
Health Related

The life expectancy of patients with chronic myeloid leukemia (CML) approaches that of the age-matched population and quality of life (QOL) issues are becoming increasingly important. To describe patients’ characteristics and assess QOL, we delivered a 30-item core questionnaire, a 24-item CML-specific questionnaire, both from the European Organization for Research and Treatment of Cancer (EORTC), and additional health-related items to 350 patients. Among 193 patients who completed the questionnaires, 139 received either imatinib (n = 70, 33%), dasatinib (n = 45, 23%) or nilotinib (n = 24, 12%). Patients’ median age was 58 (range: 23 to 89) years and 86 (63%) were males. Stratifying patients by treatment, we recognized two distinct populations. In comparison to patients on dasatinib and nilotinib, patients on imatinib were two decades older, had a longer duration of disease and current treatment, experienced fewer limitations on daily activities (p = 0.02), less fatigue (p = 0.001), lower degree of impaired body image (p = 0.022) and less painful episodes (p = 0.014). Similarly, they had better emotional functioning, were less worried, stressed, depressed or nervous (p = 0.01) and were more satisfied with their treatment (p = 0.018). Not only does age associate with current treatments, but it also predicts how patients perceive QOL. Young patients express impaired QOL compared with elderly patients.

Quality of life in young people with congenital heart disease is better than expected

2018 ◽  
Vol 104 (2) ◽  
pp. 124-128 ◽  
Author(s):  
Barbara Reiner ◽  
Renate Oberhoffer ◽  
Peter Ewert ◽  
Jan Müller
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Young Patients ◽  
Control Group ◽  
Healthy Children ◽  
Related Quality ◽  
Health Related

ObjectivesImproved treatments for patients with congenital heart disease (CHD) have led to a growing interest in long-term functional outcomes such as health-related quality of life (HRQoL). Studies on HRQoL in children with CHD have contradicting results. Therefore, we compared HRQoL of children with CHD with that of current healthy peers and stratify CHD cases by severity and diagnostic subgroups.MethodsWe included 514 patients (191 girls) aged 7–17 (12.9±3.1) years who were recruited at our institution between July 2014 and May 2017. The self-reported and age-adapted KINDL questionnaire was used to assess HRQoL. Patient data were compared with that of a recent control group of 734 healthy children (346 girls, 13.4±2.1 years).ResultsPatients with CHD scored at least as high as healthy peers in HRQoL (CHD: 78.6±9.8; healthy: 75.6±10.1; P<0.001). After correction for sex and age, patients with CHD presented a 2.3-point higher HRQoL (P<0.001). The sex-specific and age-specific analyses showed that there were no differences between boys with and without CHD in childhood (P=0.255), but in adolescence, boys with CHD had on average 3.9-point higher scores (P=0.001), whereas girls with CHD had statistically higher HRQoL perception than healthy girls in childhood (4.2 points; P=0.003) and adolescence (4.2 points; P=0.005). There were no differences between the severity classes or diagnostic subgroups in the total HRQoL score or in the six subdomains.ConclusionThe high HRQoL in young patients with CHD suggests that they can cope well with their disease burden. This holds true for all severity classes and diagnostic subgroups.

scholarly journals Parent and Self-Report Health-Related Quality of Life Measures in Young Patients With Tourette Syndrome

2012 ◽  
Vol 28 (10) ◽  
pp. 1305-1308 ◽  
Author(s):  
Andrea E. Cavanna ◽  
Chiara Luoni ◽  
Claudia Selvini ◽  
Rosanna Blangiardo ◽  
Clare M. Eddy ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tourette Syndrome ◽  
Young Patients ◽  
Self Report ◽  
Health Related Quality ◽  
Quality Of Life Measures ◽  
Related Quality ◽  
Health Related
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