scholarly journals Alagille Syndrome: About Two Cases and Literature Review

2020 ◽  
pp. 1-6
Author(s):  
M. Akhrif ◽  
A. Radi ◽  
M. Kmari ◽  
A. Ourrai ◽  
A. Hassani ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Family History ◽  
Bile Ducts ◽  
Heart Diseases ◽  
Genetic Disorder ◽  
Alagille Syndrome ◽  
Facial Features ◽  
Clinical Expression ◽  
Intrahepatic Bile Ducts

Alagille syndrome is a multi-systemc genetic disorder with variable phenotypic penetrance that was first described in 1969 by Daniel Alagille.It is  characterized by anomalies of the intrahepatic bile ducts, heart, eye and skeleton, which are associated with facial features . The prognosis depends on the severity of the liver and heart diseases.  The authors reported  two  cases characterized by the  variability of clinical expression and evolution. The study concerned two girls aged  of 2 and 4 months  with no family history, who developed cholestatic jaundice evolving from the first month of life. The aim of this work is to remind the different clinical expressivity and the differentmodalities to manage the patients in order to ensure a best quality of life.

Development of a Multidimensional Measure to Examine Fear of Dementia

2018 ◽  
Vol 89 (2) ◽  
pp. 187-205 ◽  
Author(s):  
Kyle S. Page ◽  
Bert Hayslip ◽  
Dee Wadsworth ◽  
Philip A. Allen
Keyword(s):  
Quality Of Life ◽  
Older Adults ◽  
Family History ◽  
Health Behaviors ◽  
Factor Structure ◽  
Multidimensional Measure ◽  
Valid Instrument ◽  
History Of ◽  
Areas Of Concern

Persons with and without a family history of dementia report concerns for developing this syndrome; yet, less is known about the specific aspects of dementia that are feared. The Fear of Dementia (FOD) scale was created to assess these concerns. This study examined the psychometric properties of the FOD scale using a sample of middle-aged and older adults ( N = 734). We then explored the factor structure of the scale 2 years later using a smaller sample from the first study ( N = 226). Three factors emerged, highlighting several main areas of concern: Burden and Loss, Quality of Life, and Perceived Social and Cognitive Loss. Preliminary data suggest that the FOD scale is a reliable and valid instrument for assessing the multidimensional nature of the concern about developing dementia. Attention to what specifically is feared may help further our understanding of health behaviors, coping, and targeted supports.

scholarly journals For the 30th anniversary of laparoscopic cholecystectomy: problems remain

2019 ◽  
Vol 100 (3) ◽  
pp. 537-541
Author(s):  
I V Fedorov ◽  
A N Chugunov ◽  
L E Slavin ◽  
D A Slavin ◽  
V I Fedorov
Keyword(s):  
Laparoscopic Cholecystectomy ◽  
Bile Ducts ◽  
Perioperative Complications ◽  
Open Cholecystectomy ◽  
Intrahepatic Bile Ducts ◽  
Negative Effect ◽  
Iatrogenic Damage ◽  
Extrahepatic Bile Ducts ◽  
Legal Costs

The review describes perioperative complications of laparoscopic cholecystectomy. Over the past 30 years, laparoscopy has become the «gold standard» for cholecystectomy and one of the most frequently performed procedures in abdominal surgery. Nevertheless, despite the advantages of the method, it has an «Achilles heel» - the frequency of iatrogenic damage to the extrahepatic bile ducts is 3-5 times higher than with an open cholecystectomy. This complication has a negative effect on the survival of patients after surgery, leads to deterioration in the quality of life and is a major source of legal costs in many countries. In general, the total range for any damage to the biliary tract during laparoscopic cholecystectomy is 0.32-0.52%, while the complication rate and mortality rate are 1.6-5.3% and 0.08-0.14%, respectively. Patients who have undergone a complete intersection of the hepaticoholedochus, become «bile cripples» for life. Recurrent cholangitis, strictures of anastomoses with a possible outcome in liver cirrhosis are quite likely in later periods after damage to the intrahepatic bile ducts. Technological efforts to improve the results of laparoscopic cholecystectomy reside. These include the routine use of intraoperative cholangiography, infrared fluorescent cholangiography, etc. Nevertheless, despite the growing number of methods designed to reduce these complications, evidence of their effectiveness remains limited. The most important factors ensuring the safety of laparoscopic cholecystectomy are recognized: understanding of anatomy, adequate exposure when using electrosurgery, psychological readiness to invite a senior colleague in time for help, the ability to recognize a situation that requires conversion and rejection of laparoscopy.

scholarly journals To the issue of the health comprehensive assessment of children who underwent surgical correction of congenital heart diseases

2020 ◽  
Vol 8 (4S) ◽  
pp. 42-50
Author(s):  
L. N. Igisheva ◽  
A. A. Anikeenko ◽  
S. A. Shmulevich ◽  
I. N. Sizova
Keyword(s):  
Quality Of Life ◽  
Postoperative Period ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Rehabilitation Program ◽  
Comprehensive Assessment ◽  
Surgical Correction ◽  
Long Time

Aim. To find out the problems in children health in long-time postoperative period after cardiosurgery using the comprehensive method for creating rehabilitation program.Methods. A prospective investigation of group of children was done before (n = 88) and in a year (n = 115), in 2 years (n = 90) and in 3 years (n = 58) after the surgical correction of congenital heart defects on the base of Kuzbass cardiological center. The anamnesis, clinical and hemodynamic aspects were studied as well as the postoperative period, residual problems after the correction, social status of the family and the comprehensive assessment was done in the both groups.Results. Before the correction the most part of children had low and very low levels of physical functioning, but there was a positive dynamic right after the surgery: the most part of children had high and middle levels (13% and 44% in a year), while the amount of children with low and very low data were reduced. Nevertheless, in 3 years after the surgery the amount of children with low and very low data was increased while the hemodynamic became better. Such tendency was mentioned with all aspects of the health.Conclusion. Despite of hemodynamic normalization the quality of life with all aspects still suffers. It predicts dangers in development and quality of life in general.

Association of traditional complementary and alternate medicine usage with quality of life of diabetic patients in a Malaysian tertiary hospital

2021 ◽  
Vol ahead-of-print (ahead-of-print) ◽  
Author(s):  
Chinedum Ogbonnaya Eleazu ◽  
Aniza Abd Aziz ◽  
Tay Chuu Suen ◽  
Lam Chun-Hau ◽  
Chin Elynn ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Diabetic Foot ◽  
Heart Diseases ◽  
Tertiary Hospital ◽  
Foot Ulcers ◽  
Diabetic Patients ◽  
Diabetic Foot Ulcers ◽  
Content Type ◽  
Significant Difference

Purpose This study aims to design to assess the traditional, complementary and alternate medicine (TCAM) usage and its association with the quality of life (QOL) of Type 2 diabetic patients in a tertiary hospital (Hospital Universiti Sains Malaysia) in Malaysia. Design/methodology/approach A total of 300 respondents included in this study were divided into the following two major categories: TCAM (34.33% of respondents) and non-TCAM users (65.67% of the respondents), respectively. The mean ages of the respondents were 59.3 ± 10.2 for the TCAM users and 57.7 ± 12.0 for the non-TCAM users. Findings A greater percentage of non-TCAM users reported poor control of diabetes (14.7%) and blood glucose (55.8%) compared with the TCAM users (9.7% and 48.5%, respectively). Further, the diabetic patients on TCAM reported lower rates of coma, stroke and kidney problems but higher rates of diabetic foot ulcers, heart diseases and retinopathy than the non-TCAM users. Additionally, the diabetic patients with TCAM usage had a significantly better physical (p = 0.02) and overall (p = 0.03) qualities of life compared to the non-TCAM users. However, psychological, social and environmental health did not show any significant difference. Originality/value The prevalence of TCAM usage among diabetic patients was lower than in other comparable studies. Diabetic patients on TCAM reported lower rates of coma, stroke and kidney problems but higher rates of diabetic foot ulcers, heart diseases and retinopathy than the non-TCAM users. Further, diabetes patients on TCAM reported better QOL compared to non-TCAM users especially in terms of physical health.

scholarly journals Hereditary angioedema: an update on causes, manifestations and treatment

2019 ◽  
Vol 80 (7) ◽  
pp. 391-398 ◽  
Author(s):  
Hilary J Longhurst ◽  
Konrad Bork
Keyword(s):  
Quality Of Life ◽  
Hereditary Angioedema ◽  
Genetic Disorder ◽  
Treatment Options ◽  
Correct Diagnosis ◽  
Self Administration ◽  
Diagnosis And Management ◽  
Life Threatening ◽  
Short And Long Term

Hereditary angioedema is a rare genetic disorder caused by deficiency of C1 esterase inhibitor (C1-INH) and characterized by recurrent episodes of severe swelling that affect the limbs, face, intestinal tract and airway. Since laryngeal oedema can be life-threatening as a result of asphyxiation, correct diagnosis and management of hereditary angioedema is vital. Hereditary angioedema attacks are mediated by bradykinin, the production of which is regulated by C1-INH. Hereditary angioedema therapy relies on treatment of acute attacks, and short- and long-term prophylaxis. Acute treatment options include C1-INH concentrate, icatibant and ecallantide. Self-administration of treatment is recommended and is associated with increased quality of life of patients with hereditary angioedema. Advances in diagnosis and management have improved the outcomes and quality of life of patients with hereditary angioedema.

Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: a single-centre study

2020 ◽  
Vol 30 (4) ◽  
pp. 539-548
Author(s):  
Raphael D. Oberhuber ◽  
Sonja Huemer ◽  
Rudolf Mair ◽  
Eva Sames-Dolzer ◽  
Michaela Kreuzer ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Hypoplastic Left Heart Syndrome ◽  
Heart Diseases ◽  
Left Heart ◽  
Health Related Quality ◽  
Hypoplastic Left Heart ◽  
Related Quality ◽  
Health Related ◽  
Left Heart Syndrome

AbstractData from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, heart defects in total). Children with hypoplastic left heart syndrome (aged 6.3–16.9 years) under compulsory education requirements, who were treated at the Children’s Heart Center Linz between 1997 and 2009 (n = 74), were surveyed. Totally, 41 children and 44 parents were examined prospectively by psychologists according to Pediatric Quality of Life Inventory, a health-related quality of life measurement. The results of the self-assessments of health-related quality of life on a scale of 1–100 showed a wide range, from a minimum of 5.00 (social functioning) to a maximum of 100 (physical health-related summary scores, emotional functioning, school functioning), with a total score of 98.44. The parents’ assessments (proxy) were quite similar, showing a range from 10 (social functioning) up to 100. Adolescent hypoplastic left heart syndrome patients rated themselves on the same level as healthy youths and youths with different heart diseases. The results show that patients with hypoplastic left heart syndrome aged 6–16 years can be successfully supported and assisted in their psychosocial development even if they show low varying physical and psychosocial parameters. The finding that adolescent hypoplastic left heart syndrome patients estimated themselves similar to healthy individuals suggests that they learnt to cope with a severe heart defect.

Symptoms, Hope, Self-Management Behaviors, and Quality of Life Among Chinese Preoperative Patient With Symptomatic Valvular Heart Diseases

2019 ◽  
Vol 31 (3) ◽  
pp. 284-293 ◽  
Author(s):  
Yongfeng Chen ◽  
Guifen Fu ◽  
Fang Liang ◽  
Jing Wei ◽  
Jing He ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Rural Areas ◽  
Heart Diseases ◽  
Adult Population ◽  
Food Culture ◽  
Self Management ◽  
Valvular Heart Diseases ◽  
Loss Of Appetite ◽  
Management Behaviors

Introduction: More than 42 million people are estimated to suffer from valvular heart disease (VHD) worldwide with a prevalence of 5.3% to 7.7% in the Chinese adult population. The purpose of this study was to examine the associations between symptoms, hope, self-management behaviors, and quality of life (QOL) for preoperative patients with symptomatic VHD in a rural area of China. Method: This was a descriptive comparative study that took place in Nanning, China, between January 2015 and March 2016. The sample was 128 preoperative patients with symptomatic VHD. Data were collected using the Symptom Distress Questionnaire, Herth Hope Index, Self-Management Scale, and Minnesota Living with Heart Failure Questionnaire. Results: Data from 122 patients were included in the final analysis. Mean scores of hope, self-management, and QOL were 36.71, 55.27, and 55.56. Worse total scores of symptom severity ( r = 0.57 to 0.69, p < .001) and self-management behaviors ( r = −0.22 to −0.25, p < .05) were associated with poorer QOL. Fatigue, loss of appetite, and self-management behaviors explained 49.90% variance of QOL ( p < .001). Discussion: Fatigue, loss of appetite, and self-management influenced QOL of patients with symptomatic VHD. Interventions aimed at strengthening self-management and relieving symptoms should be tailored for patients with symptomatic VHD base on their traditional animist belief and food culture in rural areas of China such as the Zhuang Autonomous Region.

scholarly journals Socioeconomic characteristics and quality of life of urban and rural elderly people with heart disease

2015 ◽  
Vol 36 (3) ◽  
pp. 21-27 ◽  
Author(s):  
Darlene Mara dos Santos Tavares ◽  
Glendha Oliveira Arduini ◽  
Nayara Paula Fernandes Martins ◽  
Flavia Aparecida Dias ◽  
Lucia Aparecida Ferreira
Keyword(s):  
Quality Of Life ◽  
Heart Disease ◽  
Linear Regression ◽  
Social Relations ◽  
Urban Areas ◽  
Heart Diseases ◽  
Household Survey ◽  
Chi Square ◽  
Socioeconomic Characteristics

Objective: To compare the socioeconomic variables and quality of life scores (QOL) of elderly residents with heart diseases in urban and rural areas.Method: household survey with 829 urban and 220 rural elderlies. The data were collected using: Brazilian Questionnaire for Functional and Multidimensional Assessment, WHOQOL-BREF and WHOQOL-OLD. The collection in the urban area was from June to December of 2008 and, in the rural area, from June 2010 to March 2011. Chi-square, t-student and multiple linear regression (p <0.05) tests were used.Results: The proportion of women and elderlies with 75 years of age and over was higher in urban areas. Lower scores among urban elderlies physical and social relations were observed, and; facets autonomy, past, present and future activities and intimacy; for the rural elderlies, the environment, sensory abilities, death and dying.Conclusion: the urban elderlies showed a lower QOL score in most areas and facets compared to rural elderlies.

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