Toward Telemonitoring in Immune-Mediated Inflammatory Diseases: Protocol for a Mixed Attention Model Study.

BACKGROUND: Rheumatic and musculoskeletal diseases (RMDs) are chronic diseases that may alternate between asymptomatic periods and flares. These conditions require complex treatments and close monitoring by rheumatologists to mitigate their effects and improve the patient's quality of life. Often, delays in outpatient consultations or the patient's difficulties in keeping appointments make such close follow-up challenging. For this reason, it is very important to have open communication between patients and health professionals. In this context, implementing telemonitoring in the field of rheumatology has great potential, as it can facilitate the close monitoring of patients with RMDs. The use of these tools helps patients self-manage certain aspects of their disease. This could result in fewer visits to emergency departments and consultations, as well as enable better therapeutic compliance and identification of issues that would otherwise go unnoticed. OBJECTIVE: The main objective of this study is to evaluate the implementation of a hybrid care model called the mixed attention model (MAM) in clinical practice and determine whether its implementation improves clinical outcomes compared to conventional follow-up. METHODS: This is a multicenter prospective observational study involving 360 patients with rheumatoid arthritis (RA) and spondylarthritis (SpA) from 5 Spanish hospitals. The patients will be followed up by the MAM protocol, which is a care model that incorporates a digital tool consisting of a mobile app that patients can use at home and professionals can review asynchronously to detect incidents and follow patients' clinical evolution between face-to-face visits. Another group of patients, whose follow-up will be conducted in accordance with a traditional face-to-face care model, will be assessed as the control group. Sociodemographic characteristics, treatments, laboratory parameters, assessment of tender and swollen joints, visual analog scale for pain, and electronic patient-reported outcome (ePRO) reports will be collected for all participants. In the MAM group, these items will be self-assessed via both the mobile app and during face-to-face visits with the rheumatologist, who will do the same for patients included in the traditional care model. The patients will be able to report any incidence related to their disease or treatment through the mobile app. RESULTS: Participant recruitment began in March 2024 and will continue until December 2024. The follow-up period will be extended by 12 months for all patients. Data collection and analysis are scheduled for completion in December 2025. CONCLUSIONS: This paper aims to provide a detailed description of the development and implementation of a digital solution, specifically an MAM. The goal is to achieve significant economic and psychosocial impact within our health care system by enhancing control over RMDs. TRIAL REGISTRATION: ClinicalTrials.gov NCT06273306; https://clinicaltrials.gov/ct2/show/NCT06273306. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/55829.

Clinical presentation of pediatric patients with symptomatic SARS-CoV-2 infection during the first months of the COVID-19 pandemic in a single center in Mexico City.

Background: The clinical spectrum of COVID-19 is broad, from asymptomatic to severe cases and death. The objective of this study is to analyze the clinical course of patients attended during the first months of the SARS-CoV-2 pandemic in a third-level pediatric hospital. Methods: Design: prospective cohort study. Patients with viral respiratory disease or suspected cases of COVID-19 were evaluated at the Pediatric Hospital, National Medical Center XXI Century, Mexico City, from 21 March 2020 to 13 January 2021. Statistical analysis: Chi-square test and Fisher's exact test were used for comparisons; a logistic regression model was constructed to identify clinical or laboratory characteristics associated with critical disease. A p-value < 0.05 was considered statistically significant. Results: A total of 697 patients met the operational definition of viral respiratory disease or suspected cases of COVID-19 and underwent real-time reverse transcription polymerase chain reaction (rRT-PCR) SARS-CoV-2 testing. Patients with a positive result were included. Of the 181 patients (26%), 121 (66.8%) had mild disease and were treated as outpatients and 60 (33.1%) were hospitalized. A total of six patients met the criteria for multisystem inflammatory syndrome in children (MIS-C). Of the 60 inpatients, 65% were males, and 82% had one or more comorbidities. The main comorbidities were cancer (42%) and overweight (15%). The median hospital stay was 9 days. The inpatients had a higher frequency of fever, general malaise, dyspnea, chills, polypnea, and cyanosis than the outpatients (p < 0.05). Only 21.4% of the outpatients had one or more comorbidities, which were lower than in the hospitalized patients (p < 0.001). Laboratory data at admission were similar between critically ill and those with moderate and severe disease. The patients who developed pneumonia were at higher risk of critical disease, while older age was associated with a better prognosis. A total of 13 of the 60 inpatients died (mortality 7.1%). All but one had one or more comorbidities: four had cancer, four congenital heart disease, one chronic kidney disease and epilepsy, one Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis, one obesity, and one diabetes mellitus. Conclusion: Hospital mortality is high, especially in children with comorbidities. Despite 2 years having passed since the beginning of the COVID-19 pandemic, the epidemiological and clinical data on children are still helpful to improve their prognosis.

C4d as a Practical Marker for Cutaneous Amyloidosis.

ABSTRACT: Cutaneous amyloidosis (CA) is defined by the accumulation of amyloid in the dermis; it might be primary or secondary. The diagnosis is based on histopathological findings with the demonstration of amyloid deposits, confirmed by Congo red stain under the polarized light. Studies on other diagnostic markers are ongoing in the literature. The aim of this study was to demonstrate the utility of C4d staining in the recognition of amyloid in CA and using it as an alternative or substitute marker for the diagnosis. In this retrospective study, 199 skin biopsies with a clinical provisional diagnosis of CA were analyzed, the Congo red stain was performed, and, in a subgroup (n = 97) with histopathological findings probably for CA, C4d immunohistochemistry was assessed. Forty-eight cases of CA were detected. Congo red birefringence was positive in all cases, whereas in 14 cases, it was faded. In these 14 cases, the diagnosis of CA was made by means of Congo red fluorescence and Thioflavin T because the histopathological findings were highly suggestive for CA. All CA cases were positive with C4d, and in 12 of the 49 inflammatory dermatoses, C4d was positive. The interpretation of C4d immunohistochemistry can be performed more easily and rapidly than Congo red evaluation. The sensitivity and specificity of C4d were 100% and 75.5%, respectively. In our experience, C4d staining was a useful method for detecting amyloid deposits in CA. Although Congo red staining is the gold standard for amyloid detection, we propose C4d immunohistochemistry as a routine screening method or hybrid transition while further investigations are completed.

Prevalence of inhibitors and clinical characteristics in patients with haemophilia in a middle-income Latin American country.

INTRODUCTION: Development of inhibitors is the most serious complication in patients with haemophilia (PWH). The prevalence of inhibitors in patients with severe haemophilia A (HA) is approximately 25%-30%. Inhibitor prevalence differs among populations. Some studies report a prevalence of almost twice in Hispanic as compared to Caucasian patients. Most data available, on the prevalence of inhibitors and their predisposing factors, originate from centres in developed countries. AIM: Establish the prevalence of inhibitors of FVIII and FIX in Mexico. METHODS: This was an observational, cross-sectional and descriptive study. The records of all patients diagnosed with haemophilia A (HA) or B (HB), with and without inhibitors, were included. Clinical and demographical characteristics of patients with inhibitors were assessed. Statistical analysis was performed using IBM SPSS version 22. The Ethics Committees of the various participating institutions approved this study. RESULTS: A total of 1455 patients from the 20 participating centres were recruited, from which 1208 (83.02%) had HA and 247 (16.97%) were diagnosed with HB. The presence of inhibitors in severe HA was reported in 93/777(11.96%), and 10/162 (6.17%) in severe HB. Of them, 91.7% exhibited high titres in HA and 100% in HB. CONCLUSION: In Mexico, the general prevalence of inhibitors varies considerably among centres. This study established a basis of comparison for future development and advances in the treatment and follow-up of patients. These findings also augment our understanding of risk factors related to inhibitor development.

Expansion of the variable expression of Muenke syndrome: Hydrocephalus without craniosynostosis.

Muenke syndrome (MS) is an autosomal dominant coronal craniosynostosis syndrome with variable extracranial anomalies. We studied 56 unrelated patients with non-syndromic uni- or bicoronal craniosynostosi to identify the frequency and clinical characteristics of MS in a cohort of Mexican childrens. The FGFR3 pathogenic variation p.Pro250Arg responsible for MS was characterized in all probands by PCR-restriction assay; available first-degree relatives (15 parents, 5 siblings) of the confirmed p.Pro250Arg carriers were also tested. All heterozygotes for p.Pro250Arg underwent clinical and audiologic assessment, as well as X-ray evaluations of hands and feet. Eight of 56 probands (14%) were found to carry the p.Pro250Arg variant and half of them were familial cases. Four p.Pro250Arg heterozygous familial members had been considered unaffected before the molecular testing. In one MS family, hydrocephalus without craniosynostosis, was documented as the only clinical manifestation in a previously undetected heterozygous male sibling. Hydrocephalus without craniosynostosis in a patient with the p.Pro250Arg variant suggests that some patients with MS might present only this manifestation; to our knowledge, hydrocephalus has not been described as isolated feature in MS, so we propose to consider this feature as an expansion of the MS phenotype rather than an unrelated finding. Our data also reinforce the notion that molecular testing of FGFR3 must be included in the diagnostic approach of coronal craniosynostosis. This will allow accurate genetic counseling and optimal management of MS, which might otherwise go undiagnosed because of mild manifestations and wide variability of expression. © 2016 Wiley Periodicals, Inc.

[Nosocomial infections. Trends over a 12 year-period in a pediatric hospital]. / Infecciones nosocomiales. Tendencia durante 12 años en un hospital pediátrico.

BACKGROUND: Nosocomial infections account for one of the most important health issues in hospitals; thus, prevention and control programs should be assessed through the impact on the incidence rates. The purpose of this study was to identify the trends of nosocomial infections over a 12-year period in a tertiary care pediatric hospital. METHODS: A retrospective cohort study was carried out where all hospitalized patients presenting with nosocomial infections were included. Data statistical analysis included simple frequencies, proportions and incidence density rate per year, infection site and lethality rate. RESULTS: Incidence density rate for year 2000 was 14.67 and decreased to 7.39 in 2012. Most frequent infections were those related with the intravenous line (15.4 %), mechanical ventilation-associated pneumonia (17.9 %), surgical site infection (10.3%) and urinary tract infection (6.8 %). The departments with higher rates were neonatology, the pediatric intensive care unit, medical pediatrics and neurosurgery. Nosocomial infection-associated lethality rate was 4.5 %. CONCLUSIONS: The slow decrease in the incidence density rate indicates that programs focused on training and supervision of the healthcare personnel on the appropriate placement and care of medical devices should be reinforced.

INTRODUCCIÓN: las infecciones nosocomiales constituyen uno de los principales problemas de salud en los hospitales, por ello, los programas de prevención y control deben ser evaluados con el impacto en las tasas de incidencia. El objetivo de esta investigación fue identificar la tendencia de las infecciones nosocomiales durante un periodo de 12 años en un hospital pediátrico de tercer nivel. MÉTODOS: se llevó a cabo un estudio de cohorte retrospectivo en el que se incluyeron todos los pacientes hospitalizados que presentaron infecciones nosocomiales. El análisis estadístico de los datos incluyó frecuencias simples, proporciones y densidad de incidencia por año, sitio de infección y letalidad. RESULTADOS: la densidad de incidencia para el año 2000 fue de 14.67 y disminuyó a 7.39 en 2012. Las infecciones más frecuentes fueron las relacionadas con la línea vascular (15.4 %), la neumonía relacionada con la ventilación mecánica (17.9 %), la infección de sitio quirúrgico (10.3 %) y la infección de vías urinarias (6.8 %). Los servicios con mayores tasas fueron neonatología, terapia intensiva pediátrica, pediatría médica y neurocirugía. La letalidad de la infección nosocomial fue de 4.5 %. CONCLUSIONES: la disminución lenta de la densidad de incidencia indica que deben fortalecerse los programas enfocados a la capacitación y supervisión del personal de salud sobre la correcta instalación y cuidado de dispositivos médicos.