[Budd Chiari syndrome secondary to antiphospholipid syndrome and systemic lupus erythematosus in an adolescent: a case report]. / Síndrome de Budd Chiari secundario al síndrome antifosfolipídico y lupus eritematoso sistémico en una adolescente: reporte de un caso.

Budd-Chiari syndrome is caused by an obstruction of blood flow to the liver. Published cases of the antiphospholipid syndrome associated with BCS are limited in the pediatric population. We report a 15-year-old adolescent who presented with fever, ascites, and hepatosplenomegaly. Hepatic Doppler ultrasound revealed no flow in the right and middle hepatic veins and in the inferior vena cava. Abdominal tomography revealed extensive thrombosis of the inferior vena cava. During hospitalization, she was diagnosed with antiphospholipid syndrome and systemic lupus erythematosus. She was given treatment with unfractionated heparin, low molecular weight heparin, and anticoagulants. Budd-Chiari syndrome secondary to the antiphospholipid syndrome is a life-threatening disease. Timely diagnosis and treatment improve the quality of life of the patient.

[Cytomegalovirus hepatitis in a 2-month-old infant: a case report]. / Hepatitis por citomegalovirus en una lactante de 2 meses: reporte de un caso.

Congenital cytomegalovirus infection is a cause of neonatal infection that can be asymptomatic; however, its complications are possible in some organs such as the nervous system and the liver. We report a case of a 2-month-old female infant with cytomegalovirus hepatitis characterized by abdominal distention, cholestasis, irritability, and poor breastfeeding. In the tests, liver involvement, periventricular microcalcifications, and sensorineural hearing loss were found. The diagnosis was determined by serum antibodies, confirmed with viral load in blood, urine, and liver tissue. Treatment with ganciclovir, vitamin K, and ursodeoxycholic acid was administered with adequate evolution. Cytomegalovirus hepatitis is a complication that may be associated with the involvement of other organs. Antiviral treatment for liver involvement is still controversial; however, it is recommended in children under one month of age if it is associated with neurological damage.

Association between social determinants of health and trends in prevalence of hypertension in patients of the Peruvian Ministry of Health.

OBJECTIVE: To evaluate the association between social determinants of health and trends in the prevalence of hypertension among patients of the Peruvian Ministry of Health 2007-2016. METHODS: We conducted an ecological study with secondary data sources using health care and death records obtained from the Peruvian Ministry of Health, data from the Peruvian National Household Survey and data from the Regional Information System for Decision Making. We determined the standardised prevalence of hypertension at national and region level, conducted a geospatial exploratory analysis at region level, and applied generalised linear mixed models to evaluate the association between social determinants of health and the prevalence of HT, according to the domains suggested by Healthy People 2020. RESULTS: The prevalence of hypertensive patients of the Peruvian Ministry of Health increased from 966.8/100 000 in 2007 to 1619.1/100 000 in 2016. The prevalence of hypertension rose by 17.7/100 000 per 1% increase of insurance coverage and by 2.2/100 000 per 1% increase in the number of hospitals. In contrast, it decreased by 12.3/100 000 per 1% increase of the poverty rate, by 9.8/100 000 per 1% increase of the proportion of people with native language, by 3.6/100 000 per 1% increase of GDP per capita and by 3/100 000 per 1% increase in the number of local health centres. CONCLUSIONS: The growing trend of HT prevalence in Peru is directly associated with insurance coverage and number of hospitals, and inversely associated with poverty rate, proportion of people with native language, GDP per capita and number of local health centres.

OBJECTIF: Evaluer l'association entre les déterminants sociaux de la santé et les tendances de la prévalence de l'hypertension chez les patients du Ministère de la Santé du Pérou 2007-2016. MÉTHODES: Nous avons mené une étude écologique avec des sources de données secondaires utilisant des données de santé et de décès obtenues du ministère péruvien de la santé, des données de l'enquête nationale sur les ménages péruviens et du système d'information régional pour la prise de décision. Nous avons déterminé la prévalence standardisée de l'hypertension (HT) aux niveaux national et régional, mené une analyse exploratoire géospatiale au niveau régional et appliqué des modèles mixtes linéaires généralisés pour évaluer l'association entre les déterminants sociaux de la santé et la prévalence de l'HT, selon les domaines suggérés par Healthy People 2020. RÉSULTATS: La prévalence des patients HT du Ministère de la Santé du Pérou est passée de 966,8/100.000 en 2007 à 1.619,1/100.000 en 2016. La prévalence de l'HT a augmenté de 17,7/100.000 pour 1% d'augmentation de la couverture d'assurance et de 2,2/100.000 pour 1% d'augmentation du nombre d'hôpitaux. En revanche, elle a diminué de 12,3/100.000 pour 1% d'augmentation du taux de pauvreté, de 9,8/100.000 pour 1% d'augmentation de la proportion de personnes de la langue locale, de 3,6/1000.000 pour 1% d'augmentation du PIB par habitant et de 3/100.000 pour 1% d'augmentation du nombre de centres de santé locaux. CONCLUSIONS: La tendance croissante de la prévalence de l'HT au Pérou est directement liée à la couverture d'assurance et au nombre d'hôpitaux et inversement associée au taux de pauvreté, à la proportion de personnes de la langue locale, au PIB par habitant et au nombre de centres de santé locaux.

[Mortality attributable to diabetes mellitus as recorded by the Ministry of Health of Peru, 2005-2014Mortalidade atribuída à diabetes mellitus nos registros do Ministério da Saúde do Peru, 2005-2014]. / Mortalidad atribuida a diabetes mellitus registrada en el Ministerio de Salud de Perú, 2005-2014.

OBJECTIVE: To estimate the mortality attributable to diabetes mellitus (DM) as recorded by Peru's Ministry of Health and its association with the human development index (HDI). METHODS: This was an ecological study based on a secondary analysis of death records belonging to the Ministry of Health for the period from 2005 to 2014. A death was considered attributable to DM if the underlying cause of death given in the death record was DM. Mortality attributable to DM has been presented descriptively and in terms of geospatial analyses, and Spearman's rho was used to test for an association between the difference in the mortality attributable to DM (between 2005-2006 and 2013-2014) and the HDI in Peru's various departments. RESULTS: In the 10-year period under evaluation, 25 074 records listed DM as the underlying cause of death. The mortality rate attributable to DM per 100 000 -population increased from 5.7 in 2005 to 9.5 in 2014. This accounted for 2.7% of the deaths recorded during the study period: 3.5% in coastal areas, 1.4% in the highlands, and 2.5% in the rainforest region. A direct association was found between the HDI and the difference in mortality attributable to DM (Spearman's rho = 0.41; p = 0.04). CONCLUSIONS: Mortality attributable to DM increased over the study period. It was highest in coastal areas, intermediate in the rainforest region, and lowest in the highlands. A direct association was found between the HDI and the difference in mortality attributable to DM.

OBJETIVOS: Estimar a mortalidade atribuída à diabetes mellitus com base em registros do Ministério da Saúde do Peru e examinar a associação com o índice de desenvolvimento humano (IDH). MÉTODOS: Estudo ecológico realizado com dados de uma análise secundária dos registros de óbitos do Ministério da Saúde para o período 2005 a 2014. Foi considerada mortalidade atribuída à diabetes mellitus os registros de óbitos com a diabetes como causa básica de morte. A mortalidade atribuída à diabetes foi apresentada de forma descritiva e com análises geoespaciais e foi feita uma análise com o uso do coeficiente de correlação (rho) de Spearman da associação entre a diferença da mortalidade associada à diabetes (entre 2005­2006 e 2013­2014) e o IDH nas províncias peruanas. RESULTADOS: No período estudado de 10 anos, foram registados 25.074 óbitos com a diabetes como causa básica de óbito. Observou-se um aumento da mortalidade atribuída à diabetes por 100 mil habitantes, de 5,7 em 2005 a 9,5 em 2014. Ela foi responsável por 2,7% dos óbitos registrados no período estudado: 3,5% na região litorânea, 1,4% na região serrana e 2,5% na região de floresta. Verificou-se uma associação direta entre o IDH e a diferença de mortalidade atribuída à diabetes (rho de Spearman = 0,41; p = 0,04). CONCLUSÕES: A mortalidade atribuída à diabetes aumentou no período estudado, sendo mais elevada na região litorânea, intermediária na região de floresta e mais baixa na região serrana. Verificou-se uma associação direta entre o IDH e a diferença de mortalidade atribuída à diabetes.

Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020.

BACKGROUND: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. METHODS: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. RESULTS: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. CONCLUSIONS: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.

INTRODUCCIÓN: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. MÉTODOS: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. RESULTADOS: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. CONCLUSIONES: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.

Massive pleural empyema secondary to amoebic liver abscess in a child.

BACKGROUND: Pleural empyema secondary to a ruptured amoebic liver abscess is a rare complication in the pediatric population. CASE REPORT: We report the case of a 13-year-old male with right flank abdominal pain, productive cough with foul-smelling sputum, fever, and respiratory distress. Physical examination revealed breathlessness, decreased vesicular murmur in the right hemithorax, abdominal distension, hepatomegaly, and lower limb edema. Laboratory tests revealed mild anemia, leukocytosis without eosinophilia, elevated alkaline phosphatase, hypoalbuminemia, and positive immunoglobulin G antibodies against Entamoeba histolytica in pleural fluid. He required a chest tube and treatment with metronidazole. After 2 months of follow-up, the abscesses disappeared, and the empyema decreased. CONCLUSIONS: Massive pleural empyema secondary to a ruptured liver abscess is a rare complication. The epidemiological link associated with the symptoms and serological tests can help in the diagnosis.

INTRODUCCIÓN: El empiema pleural secundario a ruptura de absceso amebiano hepático es una complicación poco frecuente en la población pediátrica. CASO CLÍNICO: Se reporta el caso de un paciente de sexo masculino de 13 años que presentó dolor abdominal en flanco derecho, tos productiva con esputo de mal olor, fiebre y dificultad respiratoria. Al examen físico se encontró amplexación y murmullo vesicular disminuido en hemitórax derecho, distensión abdominal, hepatomegalia y edema de miembros inferiores. Los resultados del laboratorio evidenciaron anemia leve, leucocitosis sin eosinofilia, elevación de fosfatasa alcalina, hipoalbuminemia y anticuerpos IgG contra Entamoeba histolytica positivo en líquido pleural. Requirió tubo de drenaje torácico y tratamiento con metronidazol. A los dos meses de seguimiento los abscesos desaparecieron y el empiema disminuyó. CONCLUSIONES: El empiema pleural masivo secundario a ruptura de absceso hepático es una complicación poco frecuente. El nexo epidemiológico asociado con la sintomatología y pruebas serológicas pueden ser de ayuda en el diagnóstico.

Hyperferritinemic sepsis secondary to invasive Toxoplasma gondii in a child with untreated HIV.

Background: Untreated human immunodeficiency virus (HIV)-immunosuppressed pediatric patients show high morbidity and mortality from opportunistic infections. Limited cases of hyperferritinemic sepsis have been described in patients with toxoplasmosis. Case report: We describe the case of a 13-year-old female patient with a history of untreated HIV who presented with hyperferritinemic sepsis secondary to Toxoplasma gondii infection and Pneumocystis jirovecci pneumonia. She received ventilatory support, inotropic drugs, treatment for opportunistic germs, and high-dose corticosteroids, but with unfavorable evolution. Conclusions: The global approach to sepsis with elevated ferritin guides to using of therapies aimed at neutralizing the severe inflammatory response. A timely diagnosis would allow prompt treatment and minimize complications.

Introducción: Los pacientes pediátricos inmunodeprimidos por el virus de la inmunodeficiencia humana (VIH) sin tratamiento presentan una elevada morbilidad y mortalidad por infecciones oportunistas. Se han descrito limitados casos de sepsis hiperferritinémica en pacientes con toxoplasmosis. Caso clínico: Se describe el caso de una paciente de 13 años con antecedente de VIH sin tratamiento que presentó sepsis hiperferritinémica secundaria a una infección por Toxoplasma gondii y neumonía por Pneumocystis jirovecci. Recibió soporte ventilatorio, uso de inotrópicos, tratamiento para gérmenes oportunistas y corticoides en altas dosis, pero su evolución fue desfavorable. Conclusiones: El abordaje global de la sepsis con ferritina elevada orienta a utilizar terapias dirigidas a neutralizar la respuesta inflamatoria severa, por lo que un diagnóstico oportuno permitiría iniciar el tratamiento prontamente y minimizar las complicaciones.

Postinfectious bronchiolitis obliterans in children: case series at a pediatric hospital in Peru.

BACKGROUND: Postinfectious bronchiolitis obliterans is a rare lung disease; there are limited reports in South America. CASE REPORT: We report 10 patients with this disease diagnosed at the Instituto Nacional de Salud del Niño-Breña (Lima-Peru). The median age at diagnosis was 19 months and all patients had a history of severe acute respiratory infection. The most frequent symptoms were cough, respiratory distress, wheezing, and hypoxemia. The mosaic attenuation pattern was the most frequent on the tomography. All the patients had positive serology for adenovirus. The treatment received was methylprednisolone pulses, azithromycin, hydroxychloroquine, and inhaled corticosteroids. No patient died during the follow-up. CONCLUSIONS: In previously healthy children with a history of severe acute respiratory infection and persistent bronchial obstructive symptoms, the diagnosis of postinfectious bronchiolitis obliterans should be considered. This is the first report in Peru with a therapeutic regimen adapted to our institution.

INTRODUCCIÓN: La bronquiolitis obliterante postinfecciosa es una enfermedad pulmonar poco frecuente; existen limitados reportes en Sudamérica. CASO CLÍNICO: En esta serie se reportan 10 pacientes con esta enfermedad diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú). La mediana de edad al diagnóstico fue de 19 meses. Todos los pacientes presentaron el antecedente de infección respiratoria aguda grave. Los síntomas más frecuentes fueron tos, dificultad respiratoria, sibilancias e hipoxemia; el patrón de atenuación en mosaico fue la característica más frecuente en la tomografía. Todos tenían serología positiva para adenovirus. Se administró tratamiento con pulsos de metilprednisolona, azitromicina, hidroxicloroquina y corticoides inhalados. Ningún paciente falleció durante el seguimiento. CONCLUSIONES: En los niños previamente sanos con antecedente de infección respiratoria aguda grave y sintomatología obstructivo bronquial persistente se debe considerar el diagnóstico de bronquiolitis obliterante postinfecciosa. Este es el primer reporte en Perú con un régimen terapéutico adaptado a nuestra institución.

Lung mechanics in pediatric acute respiratory distress syndrome associated to acute COVID-19 and MIS-C: implications for therapies and outcomes. / Mecánica pulmonar en el síndrome de distrés respiratorio agudo pediátrico asociado a COVID-19 aguda y MIS-C.

OBJECTIVE: To describe lung mechanics in Pediatric Acute Respiratory Distress Syndrome (PARDS) associated with acute COVID-19 and MIS-C with respiratory failure. METHODS: A concurrent multicenter observational study was performed, analyzing clinical variables and pulmonary mechanics of PARDS associated with COVID-19 in 4 Pediatric intensive care units (PICU) in Peru. The subgroup analysis included PARDS associated with multisystem inflammatory syndrome in children (MIS-C), MIS-PARDS, and PARDS with COVID-19 primary respiratory infection, C-PARDS. In addition, receiver operating characteristic (ROC) curve analysis for mortality and lung mechanics was performed. RESULTS: 30 patients were included. The age was 7.5 (4-11) years, 60% were male, and mortality was 23%. 47% corresponded to MIS-PARDS and 53% to C-PARDS groups. C-PARDS had positive RT-PCR in 67% and MIS-PARDS none (p < 0.001). C-PARDS group had more profound hypoxemia (P/F ratio < 100, 86% vs. 38%, p < 0.01) and higher driving-pressure [14(10-22) vs 10(10-12) cmH2O], and lower compliance of the respiratory system (CRS) [0.5 (0.3-0.6) vs 0.7(0.6-0.8) ml/ kg/cmH2O] compared with MIS-PARDS (all p < 0.05). The ROC analysis for mortality showed that driving pressure had the best performance [AUC 0.91(95%CI0.81-1.00), with the best cut-off point of 15 cmH2O (100% sensitivity and 87% specificity). Mortality in C-PARDS was 38% and 7% in MIS-PARDS (p = 0.09). MV-free days were 12(0-23) in C-PARDS and 23(21-25) in MIS-PARDS (p = 0.02). CONCLUSION: Patients with C-PARDS have lung mechanics characteristics similar to classic moderate to severe PARDS. This was not observed in patients with MIS-C. As seen in other studies, a driving pressure ≥ 15 cmH2O was the best discriminator for mortality. These findings may help guide ventilatory management strategies for these two different presentations.

Pulmonary embolism in an adolescent with COVID-19 pneumonia.

BACKGROUND: Pulmonary embolism (PE) is a complication reported in the adult population with coronavirus disease 2019 (COVID-19); however, its documentation in the pediatric population is limiteda. CASE REPORT: We report the case of a 15-year-old male with obesity and Down syndrome who was admitted for severe COVID-19 pneumonia. On day 7 of admission, he presented with chest pain, hemoptysis, respiratory distress, and marked elevation of D-dimer. Pulmonary CT angiography found an extensive thrombus in the right lower lobar artery. He received treatment with enoxaparin and rivaroxaban and had a favorable clinical outcome. In the tomographic control 1 month after treatment, thrombus was not evidenced and was successfully resolved. CONCLUSIONS: There are few reports of PE in children with COVID-19. Prompt diagnosis and early anticoagulant treatment are important to avoid life-threatening complications.

INTRODUCCIÓN: El tromboembolismo pulmonar es una complicación reportada en la población adulta con COVID-19; sin embargo, en la población pediátrica, su descripción es limitada. CASO CLÍNICO: Se reporta el caso de un varón de 15 años con antecedente de obesidad y síndrome de Down que fue hospitalizado por neumonía COVID-19 severa. En el séptimo día de hospitalización presentó dolor torácico, hemoptisis, dificultad respiratoria y elevación del dímero D. En la angiotomografía pulmonar se encontró un extenso trombo en la arteria lobar inferior derecha. Recibió tratamiento con enoxaparina y rivaroxabán evolucionando favorablemente. La resolución al mes de tratamiento fue existosa, ya que el control tomográfico no evidenció más el trombo. CONCLUSIONES: El tromboembolismo pulmonar es una complicación poco reportada en niños con neumonía COVID-19. El diagnóstico oportuno y tratamiento anticoagulante es importante para evitar complicaciones mortales.

Characteristics of hemolytic uremic syndrome in patients from a pediatric hospital in Peru, 2010-2020. / Características del síndrome urémico hemolítico en pacientes de un hospital pediátrico de Perú, 2010-2020.

OBJECTIVES.: Motivation for the study. There are few studies in Peru on hemolytic uremic syndrome. Main findings. Between the years 2010 to 2020, the age at diagnosis has not changed; however, more patients presented oliguria and required more renal replacement therapy (peritoneal dialysis) compared to previous years. Implications. This syndrome is an important cause of renal damage in children; therefore, its surveillance and notification are necessary. In addition, measures of prevention and early recognition of the disease must be implemented, since this condition is generally caused by consumption of contaminated food.

Invasive pneumococcal disease in patients from a pediatric hospital in Peru, 2017-2020. / Enfermedad neumocócica invasiva en pacientes de un hospital pediátrico de Perú, 2017-2020.

OBJECTIVES.: Motivation for the study: there are few reports describing cases of invasive pneumococcal disease after the introduction of the 13-valent conjugate vaccine in Peru. Main findings: cases of invasive pneumococcal disease are still reported in children, more frequently in children under five years of age. The most frequent clinical form was bacteremia and there was greater antibiotic resistance to erythromycin, trimethoprim-sulfamethoxazole, and penicillin. Implications: our findings suggest the need to maintain epidemiological surveillance of invasive pneumococcal disease and to measure the impact of vaccination against pneumococcus in children. This study aimed to describe the clinical characteristics, serotypes, and antibiotic susceptibility in patients with invasive pneumococcal disease (IPD). The medical records of patients with IPD who were hospitalized at the Instituto Nacional de Salud del Niño-Breña (Lima, Peru) were reviewed. We evaluated 29 patients. The median age was 1.9 years (interquartile range: 1 to 4 years). Of the sample, 51.7% were women and the most frequent clinical form of IPD was bacteremia in 18 (62.1%) patients; 65.5% had a complete vaccination schedule, according to the Peruvian Ministry of Health. Germ isolation was performed from blood samples in 82.8% of patients. Antibiotic resistance to erythromycin (55.2%) was the most frequent, followed by resistance to trimethoprim-sulfamethoxazole (48.3%) and penicillin (24.1%). The isolated serotypes were 6C, 19A, 23A and 24F. One patient died of meningitis. In conclusion, IPD was more frequent in children aged one to five years and the most frequent clinical form was bacteremia. Five serotypes reported in previous studies were found to be resistant to penicillin and erythromycin.

OBJETIVOS.: Motivación para realizar el estudio: existen pocos reportes que describan casos de la enfermedad neumocócica invasiva posterior a la introducción de la vacuna conjugada 13-valente en Perú. Principales hallazgos: aún se reportan casos de enfermedad neumocócica invasiva en niños, con mayor frecuencia en menores de cinco años. La forma clínica más frecuente fue la bacteriemia y hubo mayor resistencia antibiótica a eritromicina, trimetoprim-sulfametoxazol y penicilina. Implicancias: los hallazgos de este estudio sugieren la necesidad de mantener la vigilancia epidemiológica de la enfermedad neumocócica invasiva; además, de medir el impacto de la vacunación contra el neumococo en niños. El propósito del presente estudio fue describir las características clínicas, serotipos y susceptibilidad antibiótica en pacientes con enfermedad neumocócica invasiva (ENI). Se revisaron las historias clínicas de los pacientes con ENI hospitalizados en el Instituto Nacional de Salud del Niño-Breña (Lima, Perú). Se evaluaron a 29 pacientes. La mediana de edad fue 1,9 años (rango intercuartílico 1 a 4 años). El 51,7% eran mujeres y la forma clínica de la ENI más frecuente fue la bacteriemia en 18 (62,1%) pacientes. El 65,5% tenía el esquema de vacunación completo, según el Ministerio de Salud de Perú. El 82,8% del aislamiento del germen fue de sangre. La resistencia antibiótica fue más frecuente a la eritromicina (55,2%), trimetoprim-sulfametoxazol (48,3%) y penicilina (24,1%). Los serotipos registrados fueron 6C, 19A, 23A y 24F. Un paciente falleció por meningitis. En conclusión, la ENI fue más frecuente en niños de uno a cinco años y en la forma clínica de bacteriemia. Se encontraron cinco serotipos reportados en estudios previos con resistencia a penicilina y eritromicina.

Thrombotic thrombocytopenic purpura associated with COVID-19 in a critically ill child: a Peruvian case report.

BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease. In middle and low-income countries, specific resources are required for its diagnosis due to the lack of diagnostic tests and the variable response to plasma exchange, especially in the context of the new SARS-CoV-2 pandemic. CASE REPORT: We report the case of a 9-year-old male Hispanic patient with SARS-CoV-2 infection, atypical presentation, and multisystem involvement, thrombotic microangiopathy with dermal manifestations, hematologic, renal, and neurologic involvement. The patient was followed up after SARS-CoV-2 infection, the PLASMIC score was applied, and a genetic study was performed. Ventilation and hemodynamic support, corticotherapy, immunoglobulins, plasma exchange, renal replacement therapy, and monoclonal antibodies were given without favorable response. CONCLUSIONS: TTP associated with SARS-CoV-2 in the pediatric population is rare. However, resources for the diagnosis, support, and management of patients with TTP are required to avoid fatal outcomes.

INTRODUCCIÓN: La púrpura trombocitopénica trombótica (PTT) adquirida es una enfermedad poco frecuente. En los países de mediano y bajo estatus económico se requieren recursos para el diagnóstico de la PTT, debido a la falta de pruebas diagnósticas y a la respuesta variable al recambio plasmático, especialmente en el contexto de la pandemia por el nuevo SARS-CoV-2. CASO CLÍNICO: Paciente de sexo masculino, de 9 años, hispano, con infección por SARS-CoV-2, presentación atípica y afectación multisistémica, microangiopatía trombótica con manifestaciones dérmicas, y compromiso hematológico, renal y neurológico. Se dio seguimiento posinfección por SARS-CoV-2, se aplicó la escala PLASMIC y se realizó un estudio genético. Se aplicaron soporte ventilatorio y hemodinámico, corticoterapia, inmunoglobulinas, recambio plasmático, terapia de reemplazo renal y anticuerpos monoclonales, sin respuesta favorable. CONCLUSIONES: La PTT asociada al SARS-CoV-2 en la población pediátrica es poco frecuente. Aun así, se requieren recursos para el diagnóstico, el soporte y el manejo de los pacientes con PTT para evitar desenlaces fatales.

Trends and geographical variation in mortality from coronary disease in Peru.

BACKGROUND: Coronary disease (CD) is the main cause of mortality worldwide. Data about trends and geographical variation in CD mortality is available in some American countries. This information varies among countries since CD risk factors frequencies vary. OBJECTIVE: To describe the trend and geographical variation of coronary disease (CD) mortality in Peru, 2005-2017. METHODS: Analysis of secondary data of the Peruvian Ministry of Health's registry of deaths. We analyzed CD mortality. We described the absolute and relative frequency of deaths and age-standardized mortality rate (ASMR) by natural regions, departments, age, sex, and year. We also described the change of ASMR between two periods (2005-2010 vs. 2011-2017). RESULTS: There were 64,721 CD deaths between 2005 and 2017 (4.12% among all deaths). The absolute frequency of CD deaths was 5,665 and 6,565 in 2005 and 2017, respectively. CD mortality was more frequent in men and older adults. The ASMR varied among natural regions, being higher in the Coast (19.61 per 100,000 inhabitants). The change between the two periods revealed that almost all departments reduced their ASMRs, except for Callao, Lambayeque, and Madre de Dios. CONCLUSION: CD mortality has increased in Peru. Mortality was higher in men and older adults, and it varied among departments. More political efforts are needed to reduce these trends.

[Clinical phenotypes of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19]. / Fenotipos clínicos del síndrome inflamatorio multisistémico pediátrico asociado a COVID-19 (SIM-C).

The multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C) is infre quent but potentially lethal. There are few reports of this disease and its phenotypes in Latin America. OBJECTIVE: To describe the characteristics of the clinical phenotypes of MIS-C in hospitalized patients in Lima, Peru. PATIENTS AND METHOD: A descriptive and retrospective study in patients under 14 years old with a diagnosis of MIS-C at the Hospital Nacional Edgardo Rebagliati Martins (Lima, Perú), from April 2020 to August 2021. Clinical-demographic and microbiological variables were recorded. According to these, patients with MIS-C were classified into the shock phenotype, Kawasaki disease (KD) without shock, and the fever and inflammation phenotype, analyzing their clinical outcomes. RESULTS: 58 patients were analyzed. 32 (55.2%) presented the shock phenotype, 15 (25.8%) Kawasaki disease (KD) phenotype without shock, and 11 (19%) fever and inflammation phenotype. In the shock phenotype, 17 had KD. The mean age was 7 ± 3.5 years and 67.2% were males. Gastrointes tinal and mucocutaneous manifestations predominated in all phenotypes. The mortality was 3.5%. The frequency of coronary aneurysms was 10.2%. Most patients received immunomodulatory and antiplatelet treatment. Patients with shock phenotype showed greater involvement in inflammatory markers, hematological dysfunction, and myocardial injury, with a higher frequency of respiratory failure and invasive mechanical ventilation. CONCLUSIONS: In our case series, patients with shock phenotype were the most frequent and had worse clinical outcomes. Active surveillance of clinical phenotypes is needed to make an early diagnosis and management to improve the prognosis in these patients.

[Primary ciliary dyskinesia in children. Role of electron microscopy in countries with medium economic resources]. / Discinesia ciliar primaria en niños. Rol de la microscopia electrónica en países de medianos recursos económicos.

Primary ciliary dyskinesia (PCD) is a rare genetic disease that produces functional and structural de fects in the cilia. In Peru, no cases of this disease have been reported in the pediatric population. OBJECTIVE: To describe the clinical, radiological and ciliary ultrastructure characteristics in children with PCD, in a country with medium economic resources. CLINICAL CASE: We report 5 patients with PCD treated at the Instituto Nacional de Salud del Niño-Breña (Peru). Age range 1 to 5 years (median 3 years). Three patients were male. The most frequent clinical manifestations were chronic wet cough, rhonchi, coarse crackles, recurrent bronchial obstructive syndrome, and recurrent pneumonia. All patients had atelectasis, three had bronchiectasis, and two had dextrocardia with situs inversus. Two patients had undergone lobectomy. Other causes of recurrent pneumonias were ruled out with im munodeficiency study, chlorine test and pulmonary aspiration The electron microscopy showed ab sence of the inner arm of dynein as the most frequent pattern. All patients received treatment with antibiotics, nebulization with hypertonic saline, and respiratory physiotherapy with good adherence. CONCLUSION: In medium incomes countries, electron microscopy associated with clinical and radio logical characteristics plays an important role in the early diagnosis of this disease. This is the first Peruvian report that contributes to the casuistry and epidemiology of this rare pathology.

[Surfactant protein C dysfunction in pediatric patients: Clinical Case]. / Disfunción de la proteína surfactante C en pacientes pediátricos: Caso Clínico.

Pulmonary surfactant dysfunction disorders are caused by genetic defects that alter pulmonary surfactant metabolism. They are rare disorders and cause significant morbidity and mortality in the neonatal and pediatric populations. OBJECTIVE: To describe the clinical, histopathological, and ultrastructural findings of the lamellar body that suggest surfactant protein C (SP-C) dysfunction, where confirmatory genetic studies are not available. CLINICAL CASE: We report three pediatric cases of pul monary surfactant dysfunction disorders from a pediatric hospital in Peru. Video-assisted lung biop sy was performed in all cases. Ultrastructural studies of the lamellar body were compatible with type- C pulmonary surfactant dysfunction. The treatment used was methylprednisolone pulses monthly for six months, then every two months, varying the duration according to the clinical evolution. They also received daily hydroxychloroquine and azithromycin three times a week. Clinical evaluations, eye fundus, echocardiogram, electrocardiogram, and biochemistry were performed periodically. At follow-up, there was a good response to treatment and no adverse effects were observed. One case died despite the therapies received. CONCLUSIONS: In 3 patients with type-C surfactant dysfunction, treatment with corticosteroids, hydroxychloroquine, and azithromycin was successful in 2 of them. This is one of the first case series reported in Peru that contributes to the study of these diseases, es pecially in low- and medium-income countries.