Multifaceted Fontan Patients and Their Response to Pregnancy.

We present 4 patients with Fontan circulation who underwent successful pregnancies, albeit with complications that required close monitoring and timely intervention. Each Fontan patient presents with a unique clinical picture, making risk stratification challenging but all the more important.

Education and support needs of the older adult with congenital heart disease.

AIM: This article is a report of a study exploring health-related quality of life in adults with congenital heart disease and the extent to which it is associated with patients' illness beliefs and emotional health. BACKGROUND: A reduction in mortality in patients with congenital heart disease has led to an increasingly older population that faces new challenges. Studies in a younger adult population have reported inconsistent findings regarding health-related quality of life. Factors such as, the complexity of the congenital heart defect, have not been found to be associated with quality of life. The association between illness beliefs and health-related quality of life has not previously been reported. METHOD: A cross-sectional questionnaire study of adults with congenital heart disease attending an outpatient clinic in a specialist centre in the United Kingdom between October 2007 and May 2008. RESULTS: The mean age of the study population was 37·2 years. Participants reported poorer physical functioning, role functioning and general health than a general population. High levels of anxiety were reported in 38% and high levels of depression in 17%. In multivariate analysis, higher levels of anxiety and depression were associated with poorer mental functioning and higher levels of depression with poorer physical quality of life. CONCLUSION: We have reported that high levels of anxiety and depression in an older population of patients with congenital heart disease are associated with poorer quality of life. This highlights the need to routinely assess anxiety and depression in this patient group and to provide psychological support appropriately.

Patient monitoring and education over a tailored digital application platform for congenital heart disease: A feasibility pilot study.

BACKGROUND: Patients with adult congenital heart disease (ACHD) are a rapidly growing cardiovascular population with increasing health needs and co-morbidities. Furthermore, their management requires frequent and ongoing hospital visits which can be burdensome. Digital health and remote monitoring have been shown to have a vast potential to enhance delivery of healthcare for patients, reducing their need for travel to clinic appointments therefore reducing costs to the patient and the healthcare service. METHODS: Patients over the age of 16 with a diagnosis of ACHD were invited to use the tailored digital application too. They were monitored for a period of 6 months. Information on patient demographics, time using the application, flagged events that prompted clinical reviews and their feedback through patient surveys were collected. RESULTS: A total of 103 patients were enrolled and registered to use the digital application tool. There were 57 (56%) males, median age at the time of enrolment was 39 (16-73) years. The majority (96%) had a moderate or complex ACHD according to the ACC/AHA classification. There was a total of 7 modules that were completed on a weekly basis. The median length of a participant session was 2.2 min and the mean time to complete a module was 21 s. In total, 35 (67%) felt that the application helped them better manage their cardiac condition. Almost all (94%) of patients expressed that they would like to continue using the application beyond the pilot. There were 18 flagged events during the 6 month observation period, and 50% of received early clinical intervention. CONCLUSION: Application based remote monitoring in this select group was well received and potentially holds large benefit to patients both clinically and economically. There were no safety concerns in our pilot feasibility study. Our data may inform much needed and timely investment in digital health.

Self-Care Practices and Related Factors in Patients with Multiple Sclerosis (MS) Based on the Health Belief Model.

Introduction: Self-care programs can raise health in patients with Multiple Sclerosis (MS). This study aimed to identify the self-care behaviors and determinants in patients with MS according to the Health Belief Model (HBM). Methods: In this cross-sectional study, we included 280 MS patients through convenience sampling method. The collection tool was a self-administered questionnaire based on HBM. The participants were the members of MS society in Kerman, Iran. The data were analyzed using descriptive statistics, path analysis, and multivariable linear regression in SPSS software Version 22. Results: The mean (SD) score for self-care practices was 2.86 (0.64), and medication adherence was the most conducted practice. The perceived benefits and cues to action exerted positive influence on self-care practices. The most frequent symptoms experienced by the participants were fatigue (82.5%), visual impairment (76.4%), headaches (72.1%), and muscle weakness (71.4%). The most important cues to action for self-care behavior were the physician (77%), media (52%), and other MS patients (32%). Conclusion: The quality of life (QOL) of MS patients is heavily influenced by self-care behaviors. In this study, only about half of the patients accomplished self-care behaviors, which seems to be insufficient. Since the perceived benefits and cues to action are the main predictors of self-care practices, intervention based on these two constructs can be utilized to promote self-care programs and QOL in MS patients. Health-care providers should pay more attention to these factors for promoting self-care behaviors.

[MRI role in Creutzfeldt-Jakob disease: about a case]. / Apport de l'IRM dans la maladie de Creutzfeldt-Jakob: à propos d'un cas.

Creutzfeldt-jakob disease (CJD) is a very rare and fatal disease affecting the central nervous system. It is characterized by mental deterioration leading to progressive dementia, pyramidal and extrapyramidal symptoms as well as myoclonus. Early diagnosis is essential to prevent human-to-human transmission. We here report the case of a 62-year old patient in whom the diagnosis of sporadic CJD was retained based on his clinical status including dementia-related deterioration with myoclonus preceded by behavioral disorders, hallucinations and depression and on brain MRI data showing hyperintensities at the level of the striatum and the cortex on FLAIR and diffusion-weighted imaging sequences.

Process of Transition for Congenital Heart Patients: Preventing Loss to Follow-up.

PURPOSE: The aim of this article is to provide an overview of our nurse-led transition clinic provided to congenital heart disease patients moving from pediatric into adult care setting. DESCRIPTION OF THE SERVICE: Nurse-led transition clinic was analyzed at various stages of young adult care from an early stage of 12 to 14 years to entering adult setting at 16 years or older. METHODS: Overview of current transition service for young adults being transferred from pediatric into adult services highlights the integral role of clinical nurse specialist as a coordinator of care. RESULTS: The result of the service overview indicates that nurse-led transition service enables patients to build on their knowledge. This is achieved by providing them time and the opportunities to develop an understanding of their condition and the attitudes required to engage with the adult care setting as indicated in the psychology questionnaire from transition day. CONCLUSION: A nurse-led transition clinic enhances long-term care of patients by supporting the young adults and their family/carer through the transition and transfer of the care to promote the young adult's understanding of their condition and to prevent any lost to follow-up.

Significant Reduction in Helicobacter pylori Load in Humans with Non-viable Lactobacillus reuteri DSM17648: A Pilot Study.

Reducing the amount of Helicobacter pylori in the stomach by selective bacterial-bacterial cell interaction was sought as an effective and novel method for combating the stomach pathogen. Lactobacillus reuteri DSM17648 was identified as a highly specific binding antagonist to H. pylori among more than 700 wild-type strains of Lactobacillus species. Applying a stringent screening procedure, the strain DSM17648 was identified as selective binder to H. pylori cells under in vivo gastric conditions. The strain DSM17648 co-aggregates the pathogen in vivo and in vitro. The specific co-aggregation occurs between Lact. reuteri DSM17648 and different H. pylori strains and serotypes, as well as H. heilmannii, but not with Campylobacter jejuni or other commensal oral and intestinal bacteria. Lact. reuteri DSM17648 was shown in a proof-of-concept single-blinded, randomized, placebo-controlled pilot study to significantly reduce the load of H. pylori in healthy yet infected adults. Reducing the amount of H. pylori in the stomach by selective bacterial-bacterial cell interaction might be an effective and novel method for combating the stomach pathogen. Lact. reuteri DSM17648 might prove useful as an adhesion blocker in antibiotic-free H. pylori therapies.

Lack of stage-specific proteins in coccoid Helicobacter pylori cells.

Helicobacter pylori exists in two distinct forms, rod shaped or coccoid, in stomachs of infected patients. Based on in vitro proteome comparisons, there are no detectable coccoid-specific proteins, which argues against the specific adaptation of coccoid Helicobacter to distinct biological functions, such as enhanced persistence or transmission to other hosts.

Proteome comparison of Vibrio cholerae cultured in aerobic and anaerobic conditions.

The pathogen Vibrio cholerae causes severe diarrheal disease in humans. This environmental inhabitant has two distinct life cycles, in the environment and in the human small intestine, in which it differs in its multiplication behavior and virulence expression. Anaerobiosis, limitation of some nutrient elements, and excess burden from host metabolism reactants are the major stresses for V. cholerae living in intestine, in comparison to conditions in the environment and laboratory medium. For an insight into the response of V. cholerae to different microenvironments, we cultured the bacteria in aerobic and anaerobic conditions, and compared the whole cell proteome by two-dimensional electrophoresis. Among the protein spots identified, some protein species involved in aerobic respiration and the nutrient carbohydrate transporters were found to be more abundant in aerobic conditions, and some enzymes for anaerobic respiration and some stress response proteins were found more abundant in anaerobic culture. One spot corresponding to flagellin B subunit was decreased in anaerobic conditions, which suggests correlation with the meticulous regulation of bacterial motility during infection in the host intestine. This proteome analysis is the starting point for in-depth understanding of V. cholerae behavior in different environments.