The European Registry for Patients with Mechanical Circulatory Support (EUROMACS)

OBJECTIVES: A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (≤19 years of age) performed from 1 January 2000 to 1 July 2019 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-nine hospitals contributed 398 registered implants in 353 patients (150 female, 203 male) to the registry. The most frequent aetiology of heart failure was any form of cardiomyopathy (61%), followed by congenital heart disease and myocarditis (16.4% and 16.1%, respectively). Competing outcomes analysis revealed that a total of 80% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 20% died while on support. At 12 months, 46.7% received transplants, 8.7% were weaned from their device and 18.5% died. The 3-month adverse events rate was 1.69 per patient-year for device malfunction including pump exchange, 0.48 for major bleeding, 0.64 for major infection and 0.78 for neurological events. CONCLUSIONS: The overall survival rate was 81.5% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age) (P = 0.01) and lower weight (<20 kg) (P = 0.015). Transplant rates at 6 months contin

Outcomes of patients after successful left ventricular assist device explantation: a EUROMACS study

Aims: Sufficient myocardial recovery with the subsequent explantation of a left ventricular assist device (LVAD) occurs in approximately 1–2% of the cases. However, follow-up data about this condition are scarcely available in the literature. This study aimed to report the long-term outcomes and clinical management following LVAD explantation. Methods and results: An analysis of the European Registry for Patients with Mechanical Circulatory Support was performed to identify all adult patients with myocardial recovery and successful explantation. Pre-implant characteristics were retrieved and compared with the non-recovery patients. The follow-up data after explantation were collected via a questionnaire. A Kaplan–Meier analysis for freedom of the composite endpoint of death, heart transplantation, LVAD reimplantion, or heart failure (HF) relapse was conducted. A total of 45 (1.4%) cases with myocardial recovery resulting in successful LVAD explantation were identified. Compared with those who did not experience myocardial recovery, the explanted patients were younger (44 vs. 56 years, P < 0.001), had a shorter duration of cardiac disease (P < 0.001), and were less likely to have ischaemic cardiomyopathy (9% vs. 41.8%, P < 0.001). Follow-up after explantation could be acquired in 28 (62%) cases. The median age at LVAD implantation was 43 years (inter-quartile range: 29–52),

Social cognition in pervasive developmental disorders [Yaygin gelişimsel bozukluklarda sosyal biliş]

Pervasive developmental disorders (PDDs) and autistic disorder (AD) are neurodevelopmental disorders with genetic basis and abnormal brain development, and characterized by severe and permanent deficits in many interpersonal relation areas like communication, social cognition and processing of emotional signs. Cognitive impairments in AD and other PDDs are tried to be explained by neuropsychiatric models like theory of mind deficits, executive dysfunction and weak central coherence. This article aimed to review neurobiological bases of social cognition and theory of mind which try to explain social cognition. PubMed medical search engine was queried to find out the studies and review articles on social cognition and theory of mind in AD and PDDs. Social cognition may be defined as the ability to interact in complex social areas with understanding the others' intentions and thoughts. The mind deficit is theorized to be one of the basic difficulties in autism. Individuals with autism have deficits in recognizing mental processes and mental representations of self and others'. Patients with AD have deficits in social functions which an important part of interpersonal interactions and functioning within a social group; like face recognition, eye contact and emotional expression recognition. Frontal lobe, temporal lobe, anterior cingulate cortex, fusiform gyrus, amygdala, posterior association cortex and their internal associations are brain areas associated with social cognition. Fusiform gyrus and amygdala are effective in face perception and recognition. Studies suggest that a deficit in amygdala may lead to social perceptional deficits like face identity and emotional expression recognition. It is determined that individuals with PDDs have hypoactivation in fusiform gyrus during perception of faces. Amygdala has a regulatory effect on fusiform gyrus and in lesions of amygdala, the hypoactivation of fusiform gyrus for emotional salient faces are parallel to the level of amygdala lesion. The common result of many studies is that the hypoactivation of fusiform gyrus is based on some processes related to amygdala. Superior temporal sulcus hypoactivations and abnormal volume measures were found in patients with autistic disorder. Superior tempral sulcus has a role in perception of social stimulus from gaze directions, and eye and body movements of others'. Autism can be defined as a social cognition disorder and is caused by deficits at microscopic and/or macroscopic levels in these brain systems. The review of the neurobiology of social cognition in AD and other PPDs defisits in amygdala and in connections of amygdala with other temporal areas including fusiform gyrus, superior temporal sulcus in early infancy and that leads to a deficit or absence of infant's interest for faces and other stimuli which are socially significant. This causes abnormal development of brain areas like fusiform gyrus which needs a stimulus dependent activation. When studying social cognition deficits, it is important to note that autism is not a disorder of a unique neuroanatomical system or cyclus; but it is a neurodevelopmental disorder in which many pervasive neural systems are affected

Trichotillomania in celiac disease patient refractory to iron replacement

PubMed ID: 28779566Trichotillomania is characterized by recurring hair pulling behavior and repeated attempts to decrease or stop the behavior. This behavior can cause a trichobezoar, which is a mass of hair found in the stomach. Patients with diagnosed celiac disease may have an urge to swallow their hair due to iron or folat deficiency, which is called pica. We report a case of an 11-year old girl with celiac disease who has had trichotillomania for a duration of 2 years, and required an operation due to residual gastric hair mass. In this case trichotillomania was more likely caused by behavioral disorders secondary to celiac disease, rather than the iron deficiency due to malabsorption. © 2017 Walter de Gruyter GmbH, Berlin/Boston

Psychiatric evaluation of children with vernal keratoconjunctivitis [Vernal keratokonjonktivitli çocuklarda psikiyatrik degerlendirme]

Objective: To evaluate the psychiatric effects of vernal keratoconjunctivitis (VKC) in children. Material and Methods: Psychiatric evaluation forms were filled out through an interview with 25 children with vernal keratoconjunctivitis (VKC, study group) and 36 myopic (-0.50 and -1.50 D) and otherwise healthy children (control group). Both groups' mothers filled the Child Behavior Check List (CBCL). All children filled the Children Depression Inventory (CDI), the State-Trait Anxiety Inventory (STAI), and were interviewed using Kiddie Schedule for Affective Disorders and Schizophrenia. The Diagnostic and Statistical Manual for Psychiatric Disorders, 4th edition was used for psychiatric diagnosis. Results: The CBCL scores of activities (p<0.0001), and sociability (p=0.003) were significantly lower, and the scores of withdrawn (p=0.48), somatization (p<0.0001), anxiety depression (p=0.045), attention problems (p=0.001), total problems (p=0.020), internalizing (p=0.001) were significantly higher in the study group. The scores of STAI I (p<0.0001) and STAI II (p<0.0001) were significantly higher in the study group. The CDI scores of the study group were significantly higher (p<0.0001). A psychiatric diagnosis was made in 64.00% of the study group, and in 2.77% of the control group patients (p< 0.0001). Conclusion: VKC is a chronic disorder which affects the activity and sociability of children, and tends to cause depression and anxiety disorders. Ophthalmologists should be aware of the possible psychiatric effects of VKC in pediatric population, and should remember to consult these children to child psychiatrists. © 2013 by Türkiye Klinikleri

Hepatitis B virus among adolescents with substance use disorder: Prevalence, risks, vaccinization [Madde kullanim bozuklugu olan ergenlerde HBV: Yayginlik, riskler, aşilama]

Objective: Drug abuse and sexual intercourse have been reported to have equal level of effects on hepatitis B virus (HBV) contamination. Drug abuse prevalence increases in adolescence period in Turkey. The aim of present study was to determine the prevalence of HBV infection and risk factors among adolescent drug users, an important health problem in our country. Methods: In the study, the medical records of the patients who were hospitalized between October 2004 and November 2007 at the inpatient service of Ege University Child and Adolescent Addiction Investigation and Practice Center (EGEBAM) were evaluated. From a total of 206 patients hospitalized within this period, 188 patients whose serological results were available, were reviewed in terms of socio-demographic features (n=188), clinical properties (n=188) and risk factors (n=41). For the comparison of categorical variables chi-square test and for the risk assessment logistic regression analysis was used. Results: All of the 188 subjects recruited in the study were male and the mean age of the sample was 16.4±1.4 years. 69.1% (n=130) of the subjects were using cannabis, 56.4% (n=106) were using inhalants, 46.8% (n=88) were using ecstasy, and 42.6% (n=80) were using alcohol. Forty-five patients (23.9%) had been exposed with the virus. Among these subjects, 14.9% (n=28) of them have been exposed to the virus via immunization while 9% of the cases have been exposed to the virus via viral infection. Anti-HBc positivity was negatively correlated with education status of the mother (p=0.005) and the father (p=0.010) and economic levels of parents (p<0.001). The number of cases with Anti-HBc positivity was found to be smaller among the patients with three or less siblings compared to the ones with 4 or more siblings. Immunization via vaccination was higher among patients with higher income (p=0.033). Discussion: The prevalence of HBV infection among adolescents with substance use disorders in our country is quite lower than other countries which might be a result of lower intravenous drug users in this age group in this country. As the prevalence of HBV infection increases with age, utmost care should be provided to avoid the spread of infection in this age group