Diagnosis of primary immunodeficiencies in Peru.

PURPOSE OF REVIEW: Primary immunodeficiencies (PIDs) are human inborn errors of immunity, leading to an increased susceptibility to infections, inflammatory manifestations, and malignancy. We estimate around 16 000 individuals with PIDs living in Peru who are still undiagnosed. The purpose of this review is to make a situational analysis of the diagnosis of PIDs in Peru. RECENT FINDINGS: There is an evident underdiagnosis of PIDs in Peru. Insufficient awareness and lack of diagnostic tools can be solved partially by expanding the number and expertise of Clinical Immunologists and specialized medical centers. The availability of molecular testing at reasonable costs is mandatory to improve the diagnostic approach to patients with suspected PID. The development of didactic and innovative educational tools has been a critical strategy to improve PID awareness and diagnosis in Peru. SUMMARY: Developing countries like Peru still have critical limitations to diagnose patients with PIDs such as insufficient awareness in physicians, lack of specialized reference centers, and unavailability of confirmatory genetic testing. Joint work between government, health professionals, patient organizations, and society is essential to overcome these limitations and provide a better future for patients with inborn errors of immunity.

Diagnostic Delay of Primary Immunodeficiencies at a Tertiary Care Hospital in Peru- Brief Report.

OBJECTIVE: The aim of the study was to assess the diagnostic delay in pediatric patients with primary immunodeficiencies (PID) at a tertiary care hospital in Peru. METHODS: A descriptive study was carried out in which patients from a third-level referral center in Peru were included. Those without a specific diagnosis of PID were excluded. Data was collected by reviewing the medical records and interviewing patients' family members. RESULTS: A total of 45 patients with a mean of 7.4 years (SD = 4.3) were studied. The most frequent diagnosis was predominant antibody defects (35.5%), and the diagnostic delay had a median of 12.17 months (IQR 5.1-30.3). CONCLUSIONS: The most frequently diagnosed group of PID was predominant antibody deficiency. The overall median diagnostic delays for PID and predominant antibody deficiency were 12 and 14 months, respectively. Even though early detection of PIDs is crucial for effective treatment, current available laboratory tests required for PID diagnosis are both complex and expensive. Early detection and management of these pathologies cannot be achieved without training non-specialist health professionals in the diagnosis of PID, as well as integrating multidisciplinary and multi-center cooperation at both national and international levels.

Ranolazine for stable angina pectoris.

BACKGROUND: Stable angina pectoris is a chronic medical condition with significant impact on mortality and quality of life; it can be macrovascular or microvascular in origin. Ranolazine is a second-line anti-anginal drug approved for use in people with stable angina. However, the effects of ranolazine for people with angina are considered to be modest, with uncertain clinical relevance. OBJECTIVES: To assess the effects of ranolazine on cardiovascular and non-cardiovascular mortality, all-cause mortality, quality of life, acute myocardial infarction incidence, angina episodes frequency and adverse events incidence in stable angina patients, used either as monotherapy or as add-on therapy, and compared to placebo or any other anti-anginal agent. SEARCH METHODS: We searched CENTRAL, MEDLINE, Embase and the Conference Proceedings Citation Index - Science in February 2016, as well as regional databases and trials registers. We also screened reference lists. SELECTION CRITERIA: Randomised controlled trials (RCTs) which directly compared the effects of ranolazine versus placebo or other anti-anginals in people with stable angina pectoris were eligible for inclusion. DATA COLLECTION AND ANALYSIS: Two authors independently selected studies, extracted data and assessed risk of bias. Estimates of treatment effects were calculated using risk ratios (RR), mean differences (MD) and standardised mean differences (SMD) with 95% confidence intervals (CI) using a fixed-effect model. Where we found statistically significant heterogeneity (Chi² P < 0.10), we used a random-effects model for pooling estimates. Meta-analysis was not performed where we found considerable heterogeneity (I² ≥ 75%). We used GRADE criteria to assess evidence quality and the GRADE profiler (GRADEpro GDT) to import data from Review Manager 5.3 to create 'Summary of findings' tables. MAIN RESULTS: We included 17 RCTs (9975 participants, mean age 63.3 years). We found very limited (or no) data to inform most planned comparisons. Summary data were used to inform comparison of ranolazine versus placebo. Overall, risk of bias was assessed as unclear.For add-on ranolazine compared to placebo, no data were available to estimate cardiovascular and non-cardiovascular mortality. We found uncertainty about the effect of ranolazine on: all-cause mortality (1000 mg twice daily, RR 0.83, 95% CI 0.26 to 2.71; 3 studies, 2053 participants; low quality evidence); quality of life (any dose, SMD 0.25, 95% CI -0.01 to 0.52; 4 studies, 1563 participants; I² = 73%; moderate quality evidence); and incidence of non-fatal acute myocardial infarction (AMI) (1000mg twice daily, RR 0.40, 95% CI 0.08 to 2.07; 2 studies, 1509 participants; low quality evidence). Add-on ranolazine 1000 mg twice daily reduced the fervour of angina episodes (MD -0.66, 95% CI -0.97 to -0.35; 3 studies, 2004 participants; I² = 39%; moderate quality evidence) but increased the risk of non-serious adverse events (RR 1.22, 95% CI 1.06 to 1.40; 3 studies, 2053 participants; moderate quality evidence).For ranolazine as monotherapy compared to placebo, we found uncertain effect on cardiovascular mortality (1000 mg twice daily, RR 1.03, 95% CI 0.56 to 1.88; 1 study, 2604 participants; low quality evidence). No data were available to estimate non-cardiovascular mortality. We also found an uncertain effect on all-cause mortality for ranolazine (1000 mg twice daily, RR 1.00, 95% CI 0.81 to 1.25; 3 studies, 6249 participants; low quality evidence), quality of life (1000 mg twice daily, MD 0.28, 95% CI -1.57 to 2.13; 3 studies, 2254 participants; moderate quality evidence), non-fatal AMI incidence (any dose, RR 0.88, 95% CI 0.69 to 1.12; 3 studies, 2983 participants; I² = 50%; low quality evidence), and frequency of angina episodes (any dose, MD 0.08, 95% CI -0.85 to 1.01; 2 studies, 402 participants; low quality evidence). We found an increased risk for non-serious adverse events associated with ranolazine (any dose, RR 1.50, 95% CI 1.12 to 2.00; 3 studies, 947 participants; very low quality evidence). AUTHORS' CONCLUSIONS: We found very low quality evidence showing that people with stable angina who received ranolazine as monotherapy had increased risk of presenting non-serious adverse events compared to those given placebo. We found low quality evidence indicating that people with stable angina who received ranolazine showed uncertain effect on the risk of cardiovascular death (for ranolazine given as monotherapy), all-cause death and non-fatal AMI, and the frequency of angina episodes (for ranolazine given as monotherapy) compared to those given placebo. Moderate quality evidence indicated that people with stable angina who received ranolazine showed uncertain effect on quality of life compared with people who received placebo. Moderate quality evidence also indicated that people with stable angina who received ranolazine as add-on therapy had fewer angina episodes but increased risk of presenting non-serious adverse events compared to those given placebo.

Factors associated with mortality in patients hospitalized with COVID-19: A prospective cohort in a Peruvian national referral hospital. / Factores asociados a mortalidad en pacientes hospitalizados con COVID-19: cohorte prospectiva en un hospital de referencia nacional de Perú.

OBJECTIVES: To describe and assess clinical characteristics and factors associated with mortality in adult patients with COVID-19 admitted to a national referral hospital in Peru. METHODS: We conducted a prospective cohort study that included hospitalized patients older than 18 years with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection diagnosis. Patients with a positive rapid serological test on admission but no respiratory symptoms nor compatible images were excluded. We collected the data from clinical records. RESULTS: A total of 813 adults were included, 544 (66.9%) with confirmed COVID-19. The mean age was 61.2 years (standard deviation: 15.0), and 575 (70.5%) were male. The most frequent comorbidities were hypertension (34.1%) and obesity (25.9%). On admission, the most frequent symptoms were dyspnea (82.2%) and cough (53.9%). A total of 114 (14.0%) patients received mechanical ventilation, 38 (4.7%) were admitted to the intensive care unit, and 377 (46.4%) died. The requirement for ventilatory support, greater lung involvement, and inflammatory markers were associated with higher mortality. It was found that for every 10-year age increase, the risk of dying increased 32% (relative risk: 1.32; 95% confidence interval: 1.25 to 1.38). Those who were admitted to the intensive care unit and and were placed on mechanical ventilation had 1.39 (95% confidence interval: 1.13 to 1.69) and 1.97 (95% confidence interval: 1.69 to 2.29) times the risk of dying compared to those who did not, respectively. CONCLUSION: We found a high mortality rate among hospitalized patients associated with older age, higher inflammatory markers, and greater lung involvement.

OBJETIVOS: Describir las características clínicas y evaluar los factores asociados con la mortalidad de los pacientes adultos con la nueva enfermedad causada por coronavirus 2019 (COVID-19) ingresados a un hospital de referencia nacional de Perú. MÉTODOS: Se realizó un estudio de cohorte prospectivo. Se incluyó a pacientes mayores de 18 años hospitalizados con el diagnóstico de infección por coronavirus 2 del síndrome respiratorio agudo severo (SARS-CoV-2). Se excluyó a quienes ingresaron con prueba rápida serológica positiva al ingreso, sin clínica sugestiva ni imágenes compatibles. Los datos se recolectaron a partir de la historia clínica. RESULTADOS: Se incluyó un total de 813 adultos, 544 (66,9%) tuvieron COVID-19 confirmado. La media de la edad fue de 61,2 años (desviación estándar: 15) y 575 (70,5%) fueron de sexo masculino. Las comorbilidades más frecuentes fueron hipertensión arterial (34,1%) y obesidad (25,9%). Los síntomas más frecuentes al ingreso fueron disnea (82,2%) y tos (53,9%). Un total de 114 (14%) pacientes recibieron ventilación mecánica, 38 (4,7%) ingresaron a unidad de cuidados intensivos y 377 (46,4%) fallecieron. Se asociaron a la mortalidad el requerimiento de soporte ventilatorio, el mayor compromiso pulmonar y los marcadores inflamatorios. Encontramos que por cada 10 años que aumentó la edad, el riesgo de morir se incrementó en 32% (riesgo relativo: 1,32; intervalo de confianza 95%: 1,25 a 1,38). Aquellos pacientes que requirieron ingreso a unidad de cuidados intensivos y ventilación mecánica tuvieron 1,39 (intervalo de confianza 95%: 1,13 a 1,69) y 1,97 (intervalo de confianza 95%: 1,69 a 2,29) veces el riesgo de morir, respectivamente. CONCLUSIÓN: La mortalidad encontrada en nuestro estudio fue alta y estuvo asociada a la edad, marcadores inflamatorios y compromiso respiratorio.

Factores asociados a mortalidad en pacientes hospitalizados con COVID-19: cohorte prospectiva en un hospital de referencia nacional de Perú / Factors associated with mortality in patients hospitalized with COVID-19: a prospective cohort in a Peruvian national referral hospital

Objetivos Describir las características clínicas y evaluar los factores asociados con la mortalidad de los pacientes adultos con la nueva enfermedad causada por coronavirus 2019 (COVID-19) ingresados a un hospital de referencia nacional de Perú. Métodos Se realizó un estudio de cohorte prospectivo. Se incluyó a pacientes mayores de 18 años hospitalizados con el diagnóstico de infección por coronavirus 2 del síndrome respiratorio agudo severo (SARS-CoV-2). Se excluyó a quienes ingresaron con prueba rápida serológica positiva al ingreso, sin clínica sugestiva ni imágenes compatibles. Los datos se recolectaron a partir de la historia clínica. Resultados Se incluyó un total de 813 adultos, 544 (66,9%) tuvieron COVID-19 confirmado. La media de la edad fue de 61,2 años (desviación estándar: 15) y 575 (70,5%) fueron de sexo masculino. Las comorbilidades más frecuentes fueron hipertensión arterial (34,1%) y obesidad (25,9%). Los síntomas más frecuentes al ingreso fueron disnea (82,2%) y tos (53,9%). Un total de 114 (14%) pacientes recibieron ventilación mecánica, 38 (4,7%) ingresaron a unidad de cuidados intensivos y 377 (46,4%) fallecieron. Se asociaron a la mortalidad el requerimiento de soporte ventilatorio, el mayor compromiso pulmonar y los marcadores inflamatorios. Encontramos que por cada 10 años que aumentó la edad, el riesgo de morir se incrementó en 32% (riesgo relativo: 1,32; intervalo de confianza 95%: 1,25 a 1,38). Aquellos pacientes que requirieron ingreso a unidad de cuidados intensivos y ventilación mecánica tuvieron 1,39 (intervalo de confianza 95%: 1,13 a 1,69) y 1,97 (intervalo de confianza 95%: 1,69 a 2,29) veces el riesgo de morir, respectivamente. Conclusión La mortalidad encontrada en nuestro estudio fue alta y estuvo asociada a la edad, marcadores inflamatorios y compromiso respiratorio.

Objectives To describe and assess clinical characteristics and factors associated with mortality in adult patients with COVID-19 admitted to a national referral hospital in Peru. Methods We conducted a prospective cohort study that included hospitalized patients older than 18 years with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection diagnosis. Patients with a positive rapid serological test on admission but no respiratory symptoms nor compatible images were excluded. We collected the data from clinical records. Results A total of 813 adults were included, 544 (66.9%) with confirmed COVID-19. The mean age was 61.2 years (standard deviation: 15.0), and 575 (70.5%) were male. The most frequent comorbidities were hypertension (34.1%) and obesity (25.9%). On admission, the most frequent symptoms were dyspnea (82.2%) and cough (53.9%). A total of 114 (14.0%) patients received mechanical ventilation, 38 (4.7%) were admitted to the intensive care unit, and 377 (46.4%) died. The requirement for ventilatory support, greater lung involvement, and inflammatory markers were associated with higher mortality. It was found that for every 10-year age increase, the risk of dying increased 32% (relative risk: 1.32; 95% confidence interval: 1.25 to 1.38). Those who were admitted to the intensive care unit and and were placed on mechanical ventilation had 1.39 (95% confidence interval: 1.13 to 1.69) and 1.97 (95% confidence interval: 1.69 to 2.29) times the risk of dying compared to those who did not, respectively. Conclusion We found a high mortality rate among hospitalized patients associated with older age, higher inflammatory markers, and greater lung involvement.