Atypical Hemolytic Uremic Syndrome: A Nationwide Colombian Pediatric Series.

Objectives. Atypical hemolytic uremic syndrome (aHUS) is a rare complement-mediated kidney disease with genetic predisposition and represents up to 10% of pediatric hemolytic uremic syndrome (HUS) cases. Few studies have evaluated aHUS in Latin American population. We studied a Colombian pediatric cohort to delineate disease presentation and outcomes. Methods. A multicenter cohort of 27 Colombian children with aHUS were included. Patients were grouped by age at onset. Clinical features were compared using analysis of variance (ANOVA) and Fisher exact tests. Renal biopsy was performed on 6 patients who were suspected of having other renal diseases before aHUS diagnosis. Results. Most patients were male (70%). The onset of aHUS occurred frequently before age 4 years (60%) and followed gastroenteritis as the main triggering event (52%). Age groups showed comparable clinical presentation, disease severity, treatment, and outcomes. Pulmonary involvement (67%) was the main extrarenal manifestation, particularly in the 1 to 7 age group (P = .01). Renal biopsies were as follows: 3 had membranoproliferative glomerulonephritis (MPGN) type I, one MPGN type III, one C3-glomerulonephritis, and one rapidly progressive GN. Genetic screening was available in 6 patients and identified 2xCFHR5, 2xMCP, 1xADAMTS13/THBD, and 1xDGKE mutations. A total of 15 relapses were seen, of which 8 (72%) occurred in the 1 to 7 age group. The renal outcome was not significantly different regardless of age group. Conclusion. In our cohort, we observed a relatively high frequency of extrarenal involvement at first presentation represented by pulmonary manifestations. The renal prognosis at initial presentation was worse than in previous reports.

Sepsis por Lactobacillus reuteri en un recién nacido pretérmino: reporte de un caso / Sepsis due to Lactobacillus reuteri in an extreme preterm newborn: case report

La utilización de probióticos en la población neonatal ha contribuido a una disminución de la morbilidad y mortalidad en esta población a través de la reducción en la incidencia de entidades como enterocolitis necrosante, infecciones asociadas al cuidado de la salud y sepsis neonatal. A pesar de los múltiples efectos beneficiosos descritos, este tipo de medicamentos debe ser administrado con precaución en los pacientes de alto riesgo, debido a que se han documentado reportes de casos de sepsis neonatal por Lactobacillus rhamnosus, Saccharomyces boulardii y Bifidobacterium, entre otros. Se presenta un caso atípico de sepsis neonatal por Lactobacillus reuteri en un recién nacido pretérmino extremo en una institución de alta complejidad en la ciudad de Bogotá D. C., Colombia. El desenlace, en nuestro caso, fue fatal, pese al manejo médico establecido.

The use of probiotics in the neonatal population has contributed to a decrease in morbidity and mortality in this population through the reduction in the incidence of entities such as necrotizing enterocolitis, infections associated with health care and neonatal sepsis. Despite the multiple beneficial effects described due to the use of probiotics, this type of medication should be administered with caution in high-risk patients because reports of cases of neonatal sepsis by Lactobacillus rhamnosus, Saccharomyces boulardii and Bifidobacterium, among others, have been documented. We present an atypical case of neonatal sepsis due to Lactobacillus reuteri in an extreme preterm newborn in a highly complex institution in the city of Bogotá D. C., Colombia. The outcome in our case was fatal despite the established medical management.

[Sepsis due to Lactobacillus reuteri in an extreme preterm newborn: case report]. / Sepsis por Lactobacillus reuteri en un recién nacido pretérmino: reporte de un caso.

The use of probiotics in the neonatal population has contributed to a decrease in morbidity and mortality in this population through the reduction in the incidence of entities such as necrotizing enterocolitis, infections associated with health care and neonatal sepsis. Despite the multiple beneficial effects described due to the use of probiotics, this type of medication should be administered with caution in high-risk patients because reports of cases of neonatal sepsis by Lactobacillus rhamnosus, Saccharomyces boulardii and Bifidobacterium, among others, have been documented. We present an atypical case of neonatal sepsis due to Lactobacillus reuteri in an extreme preterm newborn in a highly complex institution in the city of Bogotá D. C., Colombia. The outcome in our case was fatal despite the established medical management.

La utilización de probióticos en la población neonatal ha contribuido a una disminución de la morbilidad y mortalidad en esta población a través de la reducción en la incidencia de entidades como enterocolitis necrosante, infecciones asociadas al cuidado de la salud y sepsis neonatal. A pesar de los múltiples efectos beneficiosos descritos, este tipo de medicamentos debe ser administrado con precaución en los pacientes de alto riesgo, debido a que se han documentado reportes de casos de sepsis neonatal por Lactobacillus rhamnosus, Saccharomyces boulardii y Bifidobacterium, entre otros. Se presenta un caso atípico de sepsis neonatal por Lactobacillus reuteri en un recién nacido pretérmino extremo en una institución de alta complejidad en la ciudad de Bogotá D. C., Colombia. El desenlace, en nuestro caso, fue fatal, pese al manejo médico establecido.

Childhood obesity: Aetiology, comorbidities, and treatment.

Obesity is currently considered a global epidemic, and its implications in mortality and morbidity in a paediatric patient and in adulthood are increasingly important. The objective of this article is to review in detail the definition of obesity according to age group, and, in turn, the epidemiology of this entity worldwide and in South America. Available evidence about pathophysiology and, additionally, associated comorbidities are reported in some of the most important and clinically relevant body systems. The recommendations on pharmacological and nonpharmacological management through changes in lifestyle and relevant aspects of bariatric surgery in the paediatric population are also described.

Derrame pericárdico maligno secundario a adenocarcinoma de pulmón: Reporte de caso / Malignant Pericardial Effusion Secondary to Lung Adenocarcinoma: Case Report

Introduction: Malignant pericardial effusion is the accumulation of liquid in the pericardial space secondary to advanced stage neoplasia, becoming in an indicator of bad prognosis. Malignant tumors that are most often related to this entity are the ones from pulmonary, mammary, and hematolymphoid origin. Clinically, it may present imminent signs of cardiac tamponade and hemodynamic instability, so that it merits an early diagnosis and management with emerging decompression maneuvers. Objective: This article presents a malignant pericardial effusion case, since this clinical condition implies a diagnostic challenge. Case presentation and Conclusions: A case of a 63 year old male patient is presented. He has one month of clinical evolution characterized by cervical adenopathies, also in the past fifteen days has been presenting precocious satiety, nocturnal sweating, sensation of distended abdomen, and functional class deterioration to having dyspnea during small physical efforts. This was the reason why he went to the emergency room. During the medical observation, the patient quickly deteriorates to hemodynamic instability, refractory to treatment. Some imaging studies were performed, which show severe pericardial effusion so a decompressive pericardiocentesis and a multidisciplinary integral management were performed...(AU)

Introducción: El derrame pericárdico maligno es la acumulación de líquido en el espacio pericárdico secundario a neoplasias en estadios avanzados, siendo así, un indicador de mal pronóstico. Los tumores malignos que con mayor frecuencia se relacionan con esta entidad son los de origen pulmonar, mamario y hematolinfoides. Clínicamente puede cursar con signos inminentes de taponamiento cardiaco e inestabilidad hemodinámica, por lo que amerita un diagnóstico temprano y manejo urgente con maniobras de descompresión. Objetivo: Este articulo busca presentar un caso de derrame pericárdico maligno, debido a que esta condición clínica implica un reto diagnóstico. Presentación del caso y conclusiones: Presentamos el caso de un paciente masculino de 63 años de edad, con cuadro clínico de 1 mes de evolución, caracterizado por presencia de adenopatías cervicales, quien en los últimos 15 días presenta saciedad precoz, sudoración nocturna, sensación de distensión abdominal y deterioro de la clase funcional hasta disnea de pequeños esfuerzos, motivo por el cual consulta al servicio de urgencias. Durante la observación médica rápidamente progresa a inestabilidad hemodinámica, refractaria a manejo; se realizan estudios imagenológicos, en donde se evidencia derrame pericárdico severo, por lo que se procede a realizar pericardiocentesis descompresiva emergente y manejo integral multidisciplinario...(AU)

Introdução: o derrame pericárdico maligno é o acúmulo de líquido no espaço pericárdico secundário a neoplasias em estádios avançados, sendo, portanto, um indicador de mau prognóstico. Os tumores malignos, frequentemente associados a esta categoría, são aqueles de origem pulmonar, mamária e hematolinfóides. Clinicamente, podem apresentar sinais iminentes de tamponamento cardíaco e instabilidade hemodinâmica, o que justifica um diagnóstico precoce e tratamento urgente com manejo de descompressão. Objetivo: Este artigo tem como objetivo apresentar um caso de derrame pericárdico maligno, pois esta condição clínica implica um desafio diagnóstico. Apresentação e conclusões do caso: apresentamos o caso de um paciente do sexo masculino de 63 anos, com o quadro clínico de 1 mês de evolução, caracterizado pela presença de linfadenopatia cervical, que nos últimos 15 dias apresenta saciedade precoce, sudação noturna, distensão abdominal e deterioração da classe funcional até a dispnéia de pequenos esforços, motivo pelo qual consulta o serviço de emergência. Durante a observação médica, ele progride rapidamente para a instabilidade hemodinâmica, refratária ao manejo; Estudos de imagem são realizados, nos quais o derrame pericárdico grave é evidente, por isso é necessário realizar pericardiocentese descompressiva emergente e gerenciamento integral multidisciplinar...(AU)

Sarcoidosis sistémica: un reto diagnóstico en pediatría. Reporte de un caso / Systemic sarcoidosis: a diagnostic challenge in pediatrics. Case report

La sarcoidosis es una enfermedad inflamatoria granulomatosa de etiología desconocida, evidenciada con mayor frecuencia en adultos jóvenes, que puede comprometer diversos órganos, principalmente, el pulmón, los ganglios linfáticos, los ojos y la piel. La presencia de dicha enfermedad enla población pediátrica es poco frecuente y su denominación en pediatría se realiza basada en la edad y las manifestaciones clínicas del paciente. El objetivo del siguiente reporte es presentar un caso de sarcoidosis sistémica en un paciente adolescente sin compromiso pulmonar, con manifestaciones extrapulmonares graves. Se trata de un paciente de 15 años, quien debutó con síntomas inespecíficos (emesis, pérdida de peso). Estudios radiológicos, sin compromiso pulmonar aparente; sin embargo, con presencia de hallazgos sugestivos de histiocitosis de células de Langerhans. Se realizó un estudio histopatológico de componente óseo y linfático, cuyo resultado evidenció la presencia de una enfermedad granulomatosa crónica no necrotizante tipo sarcoidosis. La sarcoidosis infantil es una entidad rara y difícil de diagnosticar, dada, principalmente, la ausencia de biomarcadores específicos para su diagnóstico.

Sarcoidosis is a granulomatous inflammatory disease of unknown etiology, evidenced most often in young adults, which can compromise several organs, mainly lung, lymph nodes, eyes and skin. The presence of the disease in pediatric population is rare. Denomination in pediatrics is made based on age and clinical manifestations of the patient. The aim of this report is to present a case of systemic sarcoidosis in a pediatric patient without lung involvement with severe extrapulmonary manifestations. Fifteen year old patient who debuted with nonspecific symptoms (emesis, weight loss). Radiographic studies without lung involvement but with findings suggestive of Langerhans cell histiocytosis; however, histopathological report (bone and lymph) showed the presence of chronic granulomatous nonnecrotizing disease, sarcoidosis type. Childhood sarcoidosis is a rare and difficult entity to diagnose, mainly by the lack of specific biomarkers for diagnosis.

[Systemic sarcoidosis: a diagnostic challenge in pediatrics. Case report]. / Sarcoidosis sistémica: un reto diagnóstico en pediatría. Reporte de un caso.

Sarcoidosis is a granulomatous inflammatory disease of unknown etiology, evidenced most often in young adults, which can compromise several organs, mainly lung, lymph nodes, eyes and skin. The presence of the disease in pediatric population is rare. Denomination in pediatrics is made based on age and clinical manifestations of the patient. The aim of this report is to present a case of systemic sarcoidosis in a pediatric patient without lung involvement with severe extrapulmonary manifestations. Fifteen year old patient who debuted with nonspecific symptoms (emesis, weight loss). Radiographic studies without lung involvement but with findings suggestive of Langerhans cell histiocytosis; however, histopathological report (bone and lymph) showed the presence of chronic granulomatous nonnecrotizing disease, sarcoidosis type. Childhood sarcoidosis is a rare and difficult entity to diagnose, mainly by the lack of specific biomarkers for diagnosis.

La sarcoidosis es una enfermedad inflamatoria granulomatosa de etiología desconocida, evidenciada con mayor frecuencia en adultos jóvenes, que puede comprometer diversos órganos, principalmente, el pulmón, los ganglios linfáticos, los ojos y la piel. La presencia de dicha enfermedad enla población pediátrica es poco frecuente y su denominación en pediatría se realiza basada en la edad y las manifestaciones clínicas del paciente. El objetivo del siguiente reporte es presentar un caso de sarcoidosis sistémica en un paciente adolescente sin compromiso pulmonar, con manifestaciones extrapulmonares graves. Se trata de un paciente de 15 años, quien debutó con síntomas inespecíficos (emesis, pérdida de peso). Estudios radiológicos, sin compromiso pulmonar aparente; sin embargo, con presencia de hallazgos sugestivos de histiocitosis de células de Langerhans. Se realizó un estudio histopatológico de componente óseo y linfático, cuyo resultado evidenció la presencia de una enfermedad granulomatosa crónica no necrotizante tipo sarcoidosis. La sarcoidosis infantil es una entidad rara y difícil de diagnosticar, dada, principalmente, la ausencia de biomarcadores específicos para su diagnóstico.