Vagal Asystoles in a Boy With Prader-Willi Syndrome.

Prader-Willi syndrome (PWS) is a genetic hormonal disorder of the hypothalamic-pituitary-axis resulting in mental retardation, muscle hypotonia, hypogonadism, and hyperphagia leading to significant obesity. Cardiovascular morbidity and mortality in adult patients with PWS is higher than in healthy controls and mainly secondary to massive obesity. In childhood, mortality may result from respiratory or gastrointestinal illnesses. We present a case of a 10-year-old boy with PWS who experienced recurrent and asymptomatic episodes of sinus pauses caused by the ingestion of large gulps of apple juice, which could be provoked and reproduced. The asystoles could not be provoked by any other vagal maneuvers and an initial diagnostic workup revealed no indication for structural heart disease. Because of the asymptomatic character of the asystoles, no treatment was initially provided. When he re-presented 3 months later after a clinically relevant syncope at school, pacemaker therapy was initiated, and he has demonstrated no subsequent sinus pauses or syncopes. Regarding the rising awareness of subtle cardiac alterations including autonomic dysfunction and electrocardiogram changes in young patients with PWS and especially the occurrence of unexplained sudden deaths in childhood that may be precipitated by arrhythmia, we suggest that the utility of periodic screening for arrhythmia risk should be evaluated in children with PWS.

Conversion to everolimus in pediatric heart transplant recipients is a safe treatment option with an impact on cardiac allograft vasculopathy and renal function.

BACKGROUND: Cardiac allograft vasculopathy (CAV) and nephrotoxicity affect long-term survival after heart transplantation (HTX). Studies, mostly conducted in adults, showed a positive effect of everolimus (EVL) on these problems. We describe the effects of conversion of the immunosuppressive therapy to an everolimus including regime on CAV, renal function, and safety in heart transplanted children/adolescents. METHODS: This retrospective single-center study included 36 participants (mean time after HTX 6.3 ± 4.7 years). Descriptive pre/post-comparisons were performed with an observation period partially up to 4 years. Impact on CAV was assessed based on intravascular imaging and Stanford grading. Safety analysis included cytomegalovirus (CMV)-infection and acute rejection. RESULTS: In terms of CAV (9 out of 36 patients) four showed no progression, three an improvement, one a worsening; one new diagnosis. The average CrCl showed a significant improvement 6, 12, and 24 months after conversion regarding all patients (n = 29). There was no acute rejection or CMV-infection. CONCLUSION: Conversion to an EVL-based therapy after pediatric HTX is a safe immunosuppressive regime without increasing risk of acute rejection or CMV-infection. There was some evidence of reduction in progression of CAV and a significant improvement of the renal function.

Intermediate outcomes of transcatheter pulmonary valve replacement with the Edwards Sapien 3 valve - German experience.

Background: After encouraging results with the Edwards Sapien and XT valves, this study aimed to review procedural data and early outcomes for the Sapien 3 valves for transcatheter pulmonary valve replacement (TPVR). Methods: We performed a multicenter, retrospective analysis of cases who underwent a Sapien 3 TPVR between 2015 and 2017 in 7 centers in Germany with a follow-up of up to 2 years. Results: 56 patients could be enrolled (weight 58,5 ± 25,0 kg; 53% Tetralogy of Fallot, 45% native RVOT). Most procedures were two-stage procedures (82,1%) with 100% prestenting. Valve sizes were 20 mm (n = 1), 23 mm (n = 15), 26 mm (n = 27), 29 mm (n = 13). Procedural success rate was 96.4%. Two patients underwent surgical valve implantation after balloon rupture during TPVR. Follow-up data were available up to 24-month post TPVR. The rate of patients with ? moderate and severe pulmonary regurgitation decreased to 0% after TPVR, peak systolic gradient decreased from 24,2 (SD±20,9) mmHg to 7,1 mmHg (SD±5,0). There were no endocarditis, severe tricuspid valve impairment or stent fractures. Conclusions: With the Edwards Sapien 3 valve, the patient pool for TPVR can be substantially extended. Continued data collection is necessary to verify long-term results.

The Creation of an Interatrial Right-To-Left Shunt in Patients with Severe, Irreversible Pulmonary Hypertension: Rationale, Devices, Outcomes.

PURPOSE OF THE REVIEW: Targeted pharmacotherapies did improve survival rates, exercise capacity, and quality of life (QoL) of PAH patients. However, these pharmacological interventions are expensive and not always accessible. In addition, not all patients do respond similarly to these medications and many will continue to deteriorate. This review aims to discuss the beneficial role of an artificial right-to-left shunt and highlights current interventional devices and outcomes. RECENT FINDINGS: Since patients with preexisting atrial shunts or patients with Eisenmenger's disease show better survival rates, improved exercise capacity, and QoL, PAH patients clinically do benefit from an atrial septostomy by reducing signs of right heart failure, improving left heart filling, cardiac output, and systemic oxygen transport despite hypoxia. However, an uncontrolled septostomy with unrestricted right-to-left shunt bears the risk of acute severe desaturation and death. The Atrial Flow Regulator (AFR device, Occlutech®, Sweden) provides an adjustable shunt size with restricted flow and excellent short-term outcomes. Interventional strategies for PAH palliation are on the rise. The novel AFR device provides a durable and safe option for a controlled right-to-left shunting, thus enabling an individualized management.

Guiding Antiepileptic Therapy in a Pediatric Patient with Severe Meningoencephalitis and Decompressive Craniectomy with the Use of Amplitude-Integrated Electroencephalography.

Introduction Amplitude-integrated electroencephalography (aEEG) is one of the most widely used neuromonitoring tools in neonatology today. However, little is known about its clinical indications and potential benefits in pediatric intensive care patients. Based on limited experience, its impact on therapeutic decision-making in this patient population is unclear. Case Description We report the case of a 16-year-old boy who, after a pansinusitis, developed a severe meningoencephalitis and intracranial empyema with increased intracranial pressure that required drainage and decompressive craniectomy. He subsequently developed status epilepticus despite a combination of various anticonvulsants. Only after the initialization of an aEEG, we were able to adequately diagnose and continuously monitor his seizure activity and titrate the effect of the antiepileptic drugs. During his hospital stay, we were able to clearly monitor and guide our therapy by accurately identifying the termination of status epilepticus and the recurrence of seizures. Discussion With the help of aEEG, it was easy to identify the nonconvulsive status epilepticus (NCSE) and the ongoing seizure activity in this teenage patient. NCSE is a clinical problem with an effect on the outcome of the patient and is often underdiagnosed. AEEG enabled a rapid detection and management of seizure activity and thereby reduced the overall seizure burden, which was associated with better neurologic outcome.

[Community transmission of pandemic influenza (H1N1) 2009 from School Outbreaks in the Madrid region, Spain: May-June 2009]. / Transmisión comunitaria de gripe pandémica (H1N1) 2009 a partir de brotes en centros escolares de la Comunidad de Madrid: Mayo-junio de 2009.

BACKGROUND: The first pandemic influenza (H1N1) 2009 confirmed case acquired in the Madrid Region was diagnosed in May 20th. This was the index case of the first outbreak in a school in the Madrid Region (MR). The aim of this paper is to describe school outbreaks that took place during the spring in 2009, the control measures implemented and the beginning of the community transmission of this virus in the Region. METHODS: Descriptive observational study of school outbreaks due to pandemic (H1N1) 2009 virus reported between May 20th and June 27th, and total investigated cases in this period. It was used the definition of cluster of influenza cases adopted by National Authority (three or more cases). Clinical, epidemiological and microbiological data of cases were analyzed. RESULTS: There were identified 1.349 cases of pandemic (H1N1) 2009. They were associated with 77 school centres with three or more reported cases, 47,4% of total cases in this period (n = 2.844). 36 outbreaks were detected in Leganés (more than one third of all schools in the municipality), generating the highest cumulative incidence rates in the Basic Health Zone. There were differences by symptoms according to age. During this period, 949 samples were analyzed and 555 (58.5%) tested positive. CONCLUSIONS: The transmission of pandemic (H1N1) 2009 virus in the MR occurred steadily during the spring of 2009 due to schools outbreaks, meaning the onset of community transmission in Spain.

[Evaluation of a schoolbased intervention to promote smokefree areas]. / Evaluación de una intervención de promoción de espacios sin humo en el ámbito educativo.

OBJECTIVE: To determine the effect of an intervention in secondary schools on visible tobacco use, the prevalence and intensity of smoking, and the attitudes and behaviors of teachers and students. METHODS: We performed a quasiexperimental study in 18 public secondary schools that carried out the program and in 18 that did not (control group). Information was obtained on visible tobacco use and the number of ashtrays and smokefree posters through direct observation. Data were collected on tobacco use, the number of heavy smokers, smoking in schools, opinions on smokefree schools, smoking in front of the students, and attempts to quit smoking through questionnaires to teachers and students in the third year of compulsory secondary education (15-year-olds) and in the final year of secondary school (18-year-olds). RESULTS: Visible tobacco use was lower in the intervention group (27.9 vs. 45.6%) and twice as many posters were displayed. No differences were found in the prevalence of daily tobacco use, but the percentage of heavy smokers was lower in teachers and students in the intervention group. Smoking was lower in students in the third year of compulsory secondary education in schools carrying out the program. Opinions about smokefree schools were favorable in both groups. CONCLUSIONS: The program contributed to reducing visible tobacco use and in decreasing the number of heavy smokers among teachers and students.

[Mortality and tuberculosis: analysis of multiple causes in the Community of Madrid (1991-1998)]. / Mortalidad y tuberculosis: análisis por causas múltiples en la Comunidad de Madrid (1991-1998).

BACKGROUND: Mortality from tuberculosis (TBC) is of characteristics such as to be studied with a multiple cause focus, as this disease is not generally fatal in itself, but is however linked to many deaths. This study is aimed at describing TBC as a cause of death in the Autonomous Community of Madrid throughout the 1991-1998 period. METHOD: A descriptive study of deaths in the Autonomous Community of Madrid (1991-1998) entailing TBC as the main cause or as a diagnosis mentioned in the Death Statistics Bulletin (DSB). The data was taken from the Autonomous Community of Madrid Death Registry. Variables analyzed: sex, age, year deceased, main cause and other diagnoses mentioned in the DSB. Crude annual rates, standardized rates by age and specific rates by age and sex were calculated. A comparison was made as to the average number of diagnoses per death. RESULTS: A total of 1,206 deaths "with TBC", 915 males and 291 females, were recorded. The main cause was TBC in 566 of these cases, HIV/AIDS in 442 and other diseases in 198 cases. The "with TBC" death rates showed peak values in 1995 among both males and females. There were an average of 3.4 diagnoses per death, this average having undergone an increase throughout the period under study (p < 0.05) CONCLUSIONS: With a multi-cause focus, TBC doubles in death rate statistics. Its presence is associated with other diseases, especially with HIV/AIDS for the period analyzed.

[The Madrid autonomous community epidemiological bulletin. A survey on its dissemination and opinion thereof on among primary care physicians for the year 2000]. / Boletín epidemiológico de la Comunidad de Madrid: encuesta sobre su difusión y percepción entre los médicos de atención primaria en el año 2000.

BACKGROUND: The Autonomous Community of Madrid Epidemiological Bulletin is the main communications link between epidemiological monitoring system and health care professionals. The purpose of this study is that of ascertaining the dissemination and opinion of this Autonomous Community of Madrid Epidemiological Bulletin among primary care physicians for the purpose of adapting this publication to its readers' interests. METHOD: A telephone survey among primary care physicians in the Autonomous Community of Madrid, asking how often they read the Bulletin, the interest and usefulness of the information included in it. The sample size was estimated at 346 physicians. A two-stage sampling process was carried out-by cluster sampling in the first stage, randomly selecting 125 health care centers and 2.7 physicians per center, 17% being primary care team coordinators. A comparison is made of the results among physicians and coordinators by means of the Chi-square and Fisher's Exact Test method, with Epi-Info v.6. RESULTS: A total of 305 surveys were conducted (245 physicians and 60 coordinators). There was an awareness of the existence of the Autonomous Community of Madrid Epidemiological Bulletin on the part of 91.5% (CI 95%: 88.1-94.8), and 27.2% (CI 95%: 21.9-32.5) were familiar with more than 50% of the last issues published. A total of 92.4% (CI 95%: 89.4-95.8) considered the Bulletin to be interesting or highly interesting, grading its usefulness an average of 3.5 on a maximum scale of 5. Of the permanent sections, the most highly-valued was Epidemic Outbreaks, those reports related to meningococcal infection, tuberculosis and HIV/AIDS being the most highly-valued. CONCLUSIONS: The Autonomous Community of Madrid Epidemiological Bulletin is a publication which, although not widely-known by the primary care physicians in the Community, is well-valued when it is read, thus being a useful feedback tool within the Epidemiological Monitoring System.