The implantable cardioverter defibrillator may prolong survival and prevent sudden cardiac arrest in patients with ventricular arrhythmias. Whereas previous research focused on mortality, attention is now turning to the impact of the device on the recipient. We reviewed the literature on the effects of the implantable cardioverter defibrillator on recipients and their families; our aim was to consolidate our current understanding and guide further research in this area. We found a number of effects on which researchers agree. Primarily, recipients are happy to have the device and generally feel more confident. However, recipients experience many psychological reactions such as fear and anxiety that can be related to the unpredictable nature of the shocks. Recipients also are affected by intellectual changes. Both recipients and their families experience similar effects and use a range of coping mechanisms; the most effective is optimism. Only one nursing intervention has been investigated that directly addresses these effects. Areas that need more research are the effects of the implantable cardioverter defibrillator during childhood, adolescence, and pregnancy; the impact of vigilance on recipients and their families; costs; and nursing interventions. More consistent, standardized quality-of-life measures are needed so that comparisons can be made between samples.

Mitral annulus calcification may directly cause cerebrovascular accidents by serving as a source of calcific or thrombotic emboli. This hypothesis has been supported by recent reports of mobile components associated with mitral annulus calcification detected by echocardiography. Cardiovascular calcifications, including mitral annulus calcification, are common in end-stage renal disease and may develop and progress over a short period of time. We report two patients with mobile components associated with mitral annulus calcification and end-stage renal disease. Serial echocardiography documented that the mitral annulus calcification in these two patients had developed rapidly. Follow-up echocardiography in one patient demonstrated resolution of the mobile elements.

Background Autosomal recessive (AR) complete IRF8 deficiency is a rare severe inborn error of immunity underlying an absence of blood myeloid mononuclear cells, intracerebral calcifications, and multiple infections. Only three unrelated patients have been reported. Materials and Methods We studied an Argentinian child with multiple infectious diseases and severe pulmonary alveolar proteinosis (PAP). We performed whole-exome sequencing (WES) and characterized his condition by genetic, immunological, and clinical means. Results The patient was born and lived in Argentina. He had a history of viral pulmonary diseases, disseminated disease due to bacillus Calmette-Guérin (BCG), PAP, and cerebral calcifications. He died at the age of 10 months from refractory PAP. WES identified two compound heterozygous variants in IRF8: c.55del and p.R111*. In an overexpression system, the p.R111* cDNA was loss-of-expression, whereas the c.55del cDNA yielded a protein with a slightly lower molecular weight than the wild-type protein. The mutagenesis of methionine residues downstream from c.55del revealed a re-initiation of translation. However, both variants were loss-of-function in a luciferase assay, suggesting that the patient had AR complete IRF8 deficiency. The patient had no blood monocytes or dendritic cells, associated with neutrophilia, and normal counts of NK and other lymphoid cell subsets. Conclusion We describe the fourth patient with AR complete IRF8 deficiency. This diagnosis should be considered in children with PAP, which is probably due to the defective development or function of alveolar macrophages.

Introduction: An effective and standardized communication anticipates and limits the appearance of possible adverse events. Objective: To evaluate the effect of the implementation of a handoff program in reducing the frequency of adverse events (AE) in Paediatric Intensive Care Units (PICUs). Methods: Facility-based, cluster randomised stepped wedge trial in six Argentine PICUs, with more than 20 admissions per month. The intervention comprised a Spanish version on the I-PASS Handoff Bundle consisting of a written and verbal handoff using mnemonics, an introductory workshop with teamwork training, an advertising campaign, simulation exercises and observation and standardized feedback of handoffs. Results: We recruited 6 cluster PICUs in 5 hospitals. We reviewed 1465 medical records (MR). We did not observed differences in the rates of preventable AE per 1000 days of hospitalization (control 60.4 [37.5 - 97.4] vs. intervention 60.4 [33.2 - 109.9], p=0.3568, RR:1.21 [CI95%:0.80 - 1.83]), and no changes in the categories or types of AE. We evaluated 847 handoffs. Compliance with all items in verbal and written handoff was significantly higher in the intervention group. We observed a longer time per patient to complete the handoff in the intervention group (7.29 minutes [5.77 - 8.81] vs. 5.96 [4.69 - 7.23]; p <0.0002, RR:1.33 [CI95%:0.64 - 2.02]), without changes in the whole time used for handoff (control: 35.7 [29.6 - 41.8] vs. intervention: 34.7 [26.5 - 42.1]; p = 0.4900, RR:1.43 [CI95%:-2.63 - 5.49]). Perception of improved communication from provider did not show changes. Conclusions: After the implementation of the I-PASS bundle, improvement in the quality of handoffs was observed. Nevertheless, no differences were observed in the frequency of AE, nor in the perception of improved communication.

BackgroundThere are only a few studies on handoff quality and adverse events (AEs) rigorously evaluating handoff improvement programmes’ effectiveness. None of them have been conducted in low and middle-income countries. We aimed to evaluate the effect of a handoff programme implementation in reducing AE frequency in paediatric intensive care units (PICUs).MethodsFacility-based, cluster-randomised, stepped-wedge trial in six Argentine PICUs in five hospitals, with >20 admissions per month. The study was conducted from July 2018 to May 2019, and all units at least were involved for 3 months in the control period and 4 months in the intervention period. The intervention comprised a Spanish version of the I-PASS handoff bundle consisting of a written and verbal handoff using mnemonics, an introductory workshop with teamwork training, an advertising campaign, simulation exercises, observation and standardised feedback of handoffs. Medical records (MR) were reviewed using trigger tool methodology to identify AEs (primary outcome). Handoff compliance and duration were evaluated by direct observation.ResultsWe reviewed 1465 MRs: 767 in the control period and 698 in the intervention period. We did not observe differences in the rates of preventable AE per 1000 days of hospitalisation (control 60.4 (37.5–97.4) vs intervention 60.4 (33.2–109.9), p=0.99, risk ratio: 1.0 (0.74–1.34)), and no changes in the categories or AE types. We evaluated 841 handoffs: 396 in the control period and 445 in the intervention period. Compliance with all items in the verbal and written handoffs was significantly higher in the intervention group. We observed no difference in the handoff time in both periods (control 35.7 min (29.6–41.8) vs intervention 34.7 min (26.5–42.1); difference 1.43 min (95% CI −2.63 to 5.49, p=0.49)). The providers’ perception of improved communication did not change.ConclusionsAfter the implementation of the I-PASS bundle, compliance with handoff items improved. Nevertheless, no differences were observed in the AEs’ frequency or the perception of enhanced communication.Trial registration numberNCT03924570

Presentación de casos clínicos RESUMENEl incremento del amonio en sangre, hiperamoniemia, es pasible de provocar compromiso neurológico al atravesar la barrera hematoencefálica. La causa más frecuente y conocida de hiperamoniemia es la alteración en la función hepática. Sin embargo, se deben considerar otras patologías, de menor frecuencia y poco conocidas. La infección del tracto urinario por gérmenes productores de ureasa debe ser contemplada a pesar de ser infrecuente en pediatría. Se reporta el caso de un niño con encefalopatía aguda grave, con niveles elevados de amonio en sangre, en quien, luego de descartar otros diagnósticos diferenciales, se asumió el cuadro como hiperamoniemia secundaria a infección del tracto urinario por Corynebacterium riegelii, un germen productor de ureasa. Se implementaron medidas generales de tratamiento para la encefalopatía hiperamoniémica y tratamiento antibiótico específico, con buena evolución el paciente. Palabras clave: encefalopatía, hiperamoniemia, germen productor de ureasa, pediatría. ABSTRACTElevated level of ammonia in the blood, defined as hyperammonemia, is feasible to cause neurological symptoms when crossing the blood-brain barrier. The most frequent and studied cause of hyperammonemia is liver failure. Nevertheless, other less frequent and known etiologies must be considered. Urinary tract infection caused by urea-splitting bacteria, despite being unusual in pediatric patients, must be taken into account. We report a pediatric patient with severe acute encephalopathy and high levels of ammonia in blood. After ruling out other causes of hyperammonemia, it was assumed secondary to urinary tract infection by Corynebacterium riegelii, a ureasplitting bacteria. General treatment for hyperammonemic encephalopathy was established, as well as specific treatment with antibiotics. The patient evolved favorably. Cómo citar: Acosta P, Nogueira M, Gallagher R, et al. Encefalopatía hiperamoniémica secundaria a infección urinaria por germen productor de ureasa. Caso clínico pediátrico. Arch Argent Pediatr 2017;115(6):e454-e457.

Purpose: Patients with heart failure is one of the largest patient groups in the health care system and will probably continue to increase. One half of the patients are taken care of in Primary care. However the largest cost for heart failure is for hospital care. Improved symptom control is suggested to prevent readmissions to hospital and ESC guidelines recommend patients as symptom control to record daily weight as weight gain can be a sign of deterioration in heart failure. Adherence to daily weight is estimated to be about 50%. The aim of this pilot-study was to examine the feasibility of a weight monitoring system for patients with heart failure in order to improve patients' symptom control. Methods: Patients with heart failure in 2 Primary health care centres in Stockholm were included in this pilot study. Electronic scales were placed in patients home and patients weighed themselves as a daily routine and the weight was transmitted via the telephone network to a computer in the Primary health care centre. In case of weight gain >2 kg in 3 days the heart failure nurse contacted the patient. If there were signs of deterioration in heart failure the patients GP was informed for further measures. Results: Twelve patients were included and participated between 8-12 weeks. Two patients were receiving changes in medication due to weight gain. One patient was followed-up after being hospitalized for weight gain and deterioration in heart failure and 2 severely ill patients were also monitored successfully. The remaining 7 patients were in relatively stable condition and needed no interventions. The transmitting of weights from patients' home to the Primary health care centres worked satisfactory and only 1 patient had trouble using the electronic scale. Conclusions: In this pilot study there were signs that weight monitoring was feasible and relatively simple to use for both patients and nurses. Weight monitoring might be a useful tool to improve symptom control among patients with heart failure but further research and larger studies in this topic will be needed.

Los pacientes en estado crítico con COVID-19 sufren hiperglucemias sostenidas de difícil manejo. A esto se suma el desafío de minimizar la exposición al contagio.En el presente artículo analizamos la evolución metabólica de dos pacientes pediátricos con COVID-19 admitidos en unidad de cuidados intensivos (UCI) para pacientes COVID-19 del Hospital “Prof. Dr. Juan P. Garrahan” de la Ciudad Autónoma de Buenos Aires, Argentina, que requirieron tratamiento con insulina endovenosa y cuya glucemia fue monitoreada de manera remota con la plataforma InsuMate® desarrollada en la Universidad Nacional de La Plata.Los pacientes requirieron tasas de infusión de insulina en dosis marcadamente mayores que las de otros pacientes críticos que impresionaron relacionadas con los valores de marcadores de inflamación. La infusión pudo ajustarse con cuatro monitoreos diarios de glucosa y las métricas obtenidas con el monitor de glucosa. El uso del sistema de monitoreo remoto continuo de glucosa permitió disminuir la frecuencia de monitoreo glucémico durante el tratamiento.