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.

Risk factors associated with SARS-CoV-2 pneumonia in the pediatric population.

INTRODUCCIÓN: Hasta ahora existen pocos estudios sobre las características clínicas de la población pediátrica con neumonía por COVID-19. El objetivo de este estudio fue analizar los datos que se asocian con el desarrollo de neumonía en niños y adolescentes con infección por SARS-CoV-2 en México. MÉTODOS: Se llevó a cabo un análisis secundario de la base de datos de la Dirección General de Epidemiología del Gobierno Mexicano. Se incluyeron menores de 19 años con infección por SARS-CoV-2 confirmada mediante la prueba de retrotranscripción acoplada a la reacción en cadena de la polimerasa (RT-PCR). La variable dependiente fue el diagnóstico de neumonía. Se calcularon las razones de momios (RM) y los intervalos de confianza del 95% (IC95%). Se realizaron modelos de regresión logística múltiple para ajustar los factores asociados a neumonía. RESULTADOS: Se incluyeron 1443 niños con diagnóstico de COVID-19. La mediana de edad de los participantes fue de 12 años (rango intercuartilar 25-75: 5-16). La neumonía se presentó en 141 niños (9.8%). Los principales factores de riesgo fueron edad < 3 años (RM: 3.5; IC95%: 2.45-5.03), diabetes u obesidad (RM: 12.6; IC95%: 4.62-34.91) e inmunocompromiso (RM: 7.03; IC95%: 3.97-13.61). CONCLUSIONES: Los niños con COVID-19 menores de 3 años y con comorbilidad, especialmente diabetes u obesidad, y los inmunocomprometidos presentan mayor riesgo de desarrollar neumonía. BACKGROUND: Currently, there are a few studies on the clinical characteristics of the pediatric population with COVID-19 and pneumonia. This study aimed to analyze data associated with the development of pneumonia in children and adolescents with SARS-CoV-2 infection throughout Mexico. METHODS: We conducted a secondary analysis of the database of the Dirección General de Epidemiología of the Mexican Government. We included children under the age of 19 who were confirmed with SARS-CoV-2 infection by reverse transcription-polymerase chain reaction (RT-PCR) test. The dependent variable was the diagnosis of pneumonia. Odds ratios (OR) and 95% confidence intervals (95% CI) were calculated. Multiple logistic regression models were performed to adjust factors associated with pneumonia. RESULTS: A total of 1443 children with a COVID-19 diagnosis were included. The median age of the participants was 12 years (interquartile range 25, 75: 5, 16). Pneumonia occurred in 141 children (9.8%). The main risk factors were age < 3 years (OR, 3.5; 95%CI, 2.45-5.03); diabetes or obesity (OR, 12.6; 95%CI, 4.62-34.91); and immunocompromise (OR, 7.03; 95%CI, 3.97-13.61). CONCLUSIONS: Children < 3 years with COVID-19 and comorbidities, especially diabetes or obesity, and immunocompromised patients have a higher risk of developing pneumonia.

Risk factors associated with SARS-CoV-2 pneumonia in the pediatric population / Factores de riesgo asociados con neumonía por SARS-CoV-2 en población pediátrica

Abstract Background: Currently, there are a few studies on the clinical characteristics of the pediatric population with COVID-19 and pneumonia. This study aimed to analyze data associated with the development of pneumonia in children and adolescents with SARS-CoV-2 infection throughout Mexico. Methods: We conducted a secondary analysis of the database of the Dirección General de Epidemiología of the Mexican Government. We included children under the age of 19 who were confirmed with SARS-CoV-2 infection by reverse transcription-polymerase chain reaction (RT-PCR) test. The dependent variable was the diagnosis of pneumonia. Odds ratios (OR) and 95% confidence intervals (95% CI) were calculated. Multiple logistic regression models were performed to adjust factors associated with pneumonia. Results: A total of 1443 children with a COVID-19 diagnosis were included. The median age of the participants was 12 years (interquartile range 25, 75: 5, 16). Pneumonia occurred in 141 children (9.8%). The main risk factors were age < 3 years (OR, 3.5; 95%CI, 2.45-5.03); diabetes or obesity (OR, 12.6; 95%CI, 4.62-34.91); and immunocompromise (OR, 7.03; 95%CI, 3.97-13.61). Conclusions: Children < 3 years with COVID-19 and comorbidities, especially diabetes or obesity, and immunocompromised patients have a higher risk of developing pneumonia.

Resumen Introducción: Hasta ahora existen pocos estudios sobre las características clínicas de la población pediátrica con neumonía por COVID-19. El objetivo de este estudio fue analizar los datos que se asocian con el desarrollo de neumonía en niños y adolescentes con infección por SARS-CoV-2 en México. Métodos: Se llevó a cabo un análisis secundario de la base de datos de la Dirección General de Epidemiología del Gobierno Mexicano. Se incluyeron menores de 19 años con infección por SARS-CoV-2 confirmada mediante la prueba de retrotranscripción acoplada a la reacción en cadena de la polimerasa (RT-PCR). La variable dependiente fue el diagnóstico de neumonía. Se calcularon las razones de momios (RM) y los intervalos de confianza del 95% (IC95%). Se realizaron modelos de regresión logística múltiple para ajustar los factores asociados a neumonía. Resultados: Se incluyeron 1443 niños con diagnóstico de COVID-19. La mediana de edad de los participantes fue de 12 años (rango intercuartilar 25-75: 5-16). La neumonía se presentó en 141 niños (9.8%). Los principales factores de riesgo fueron edad < 3 años (RM: 3.5; IC95%: 2.45-5.03), diabetes u obesidad (RM: 12.6; IC95%: 4.62-34.91) e inmunocompromiso (RM: 7.03; IC95%: 3.97-13.61). Conclusiones: Los niños con COVID-19 menores de 3 años y con comorbilidad, especialmente diabetes u obesidad, y los inmunocomprometidos presentan mayor riesgo de desarrollar neumonía.

Genetic, Immunological, and Clinical Features of the First Mexican Cohort of Patients with Chronic Granulomatous Disease.

PURPOSE: Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by an inability of phagocytes to produce reactive oxygen species, impairing their killing of various bacteria and fungi. We summarize here the 93 cases of CGD diagnosed in Mexico from 2011 to 2019. METHODS: Thirteen Mexican hospitals participated in this study. We describe the genetic, immunological, and clinical features of the 93 CGD patients from 78 unrelated kindreds. RESULTS: Eighty-two of the patients (88%) were male. All patients developed bacterial infections and 30% suffered from some kind of fungal infection. Fifty-four BCG-vaccinated patients (58%) presented infectious complications of BCG vaccine. Tuberculosis occurred in 29%. Granulomas were found in 56% of the patients. Autoimmune and inflammatory diseases were present in 15% of patients. A biological diagnosis of CGD was made in 89/93 patients, on the basis of NBT assay (n = 6), DHR (n = 27), and NBT plus DHR (n = 56). The deficiency was complete in all patients. The median age of biological diagnosis was 17 months (range, 0-186 months). A genetic diagnosis was made in 83/93 patients (when material was available), corresponding to CYBB (n = 64), NCF1 (n = 7), NCF2 (n = 7), and CYBA (n = 5) mutations. CONCLUSIONS: The clinical manifestations in these Mexican CGD patients were similar to those in patients elsewhere. This cohort is the largest in Latin America. Mycobacterial infections are an important cause of morbidity in Mexico, as in other countries in which tuberculosis is endemic and infants are vaccinated with BCG. X-linked CGD accounted for most of the cases in Mexico, as in other Latin American countries. However, a significant number of CYBA and NCF2 mutations were identified, expanding the spectrum of known causal mutations.

Clinical management of COVID-19 paediatric cases. / Manejo clínico de casos pediátricos de COVID-19.

Coronaviruses are a family of viruses present in different species of animals, which can infect humans, causing epidemics and pandemics. On January 9th, 2020, a new virus was announced as the cause of an outbreak of severe pneumonia in Wuhan, China. On March 11th, WHO declared the SARS-CoV-2 pandemic, the disease was called COVID-19. The virus is transmitted by droplets, contact and airborne during aerosol-generating procedures. In almost all pediatric cases a household contact was the source of infection. Unlike adults, the disease in children has a mild course, even in those under one year of age. The main symptoms are fever, dry or productive cough, headache, general discomfort, and shortness of breath, which is a sign of severity. The incubation period is 1-12.5 days (media 5-6 days). The most affected age group are school children. The disease may present as a mild acute upper airway infection, or as pneumonia. Very few cases progress to severe pneumonia and other complications such as acute respiratory distress syndrome, shock and multiorgan failure. There is not yet a specific antiviral treatment approved for use in children. This document aims to guide the management of suspected or confirmed pediatric patients of COVID-19.

El 9 de enero de 2020 fue anunciado un nuevo virus como la causa de un brote de neumonía grave en Wuhan, China. El 11 de marzo, la Organización Mundial de la Salud declaró la pandemia por SARS-CoV-2, virus causante de la enfermedad COVID-19. El virus se transmite por gotas, contacto y por vía aérea en procedimientos que generan aerosoles. En el caso de los niños, la fuente de contagio casi siempre es un contacto intradomiciliario. En los pacientes pediátricos, la enfermedad tiene un curso leve, incluso en los menores de un año. Los principales síntomas son: fiebre, tos seca o productiva, cefalea, malestar general y dificultad respiratoria, que es un signo de gravedad. El período de incubación es de 1 a 12.5 días (media 5-6). El grupo de edad más afectado son los escolares. La enfermedad puede presentarse como un cuadro leve de infección aguda de vías aéreas superiores o como neumonía. Muy pocos casos progresan a neumonía grave y a complicaciones como síndrome de distrés respiratorio agudo, choque y falla multiorgánica. Aún no hay tratamiento antiviral específico aprobado para su uso en niños. El presente documento tiene como objetivo guiar el manejo de los pacientes pediátricos sospechosos o confirmados de COVID-19.

Piperacillin/tazobactam plus amikacin vs. piperacilin/tazobactam: treatment for children with febrile neutropenia / Piperacilina/tazobactam más amikacina vs. piperacilina/tazobactam: tratamiento en niños con neutropenia febril

Background: Pediatric patients with febrile neutropenia usually receive a combination of broad spectrum antimicrobials. Treatment without aminoglycoside seems to have advantages. Objective: To compare the efficacy of piperacillin/tazobactam plus amikacin versus piperacillin/tazobactam. Methods: Randomized, open label, controlled clinical trial. Sample size for an efficacy of 55%, and delta of 25%; 80 episodes were required for each group. Selection criteria were patients with febrile neutropenia, candidates to receive parenteral antimicrobial treatment; they were randomized to one of two groups, piperacillin/tazobactam plus amikacin (Group A), or piperacillin/tazobactam (Group B). The outcomes were failure, adverse events and death. Mantel-Haenszel chi squaretest and exact Fisher test were used. Reduction of relative and absolute risk (RRR and ARR), 95% confidence intervals (CI 95%) and number needed to treat (NNT) were calculated. Results: 88 Episodes were analyzed in group A and 76 in group B. There was no statistical difference in general characteristics of patients or type of infections. There was not significant statistical difference in: failure 31.8% group A, 30.2% group B (RR 1.05, CI 95% 0.66-1.66, p = 0.86), or adverse events (one in each group). The RRR was 1.5%, and ARR 2%, with a NNT of 67. Conclusion: Piperacillin/tazobactam without amikacin was as effective as combination therapy in pediatric patients with febrile neutropenia.

Introducción: los pacientes pediátricos con neutropenia febril habitualmente reciben una combinación de antimicrobianos de amplio espectro. La terapia sin aminoglucósido parece tener ventajas. Objetivo: comparar la eficacia de piperacilina/tazobactam más amikacina frente a la de piperacilina/tazobactam. Métodos: ensayo clínico controlado aleatorizado. Tamaño de muestra para una eficacia de 55%, y delta de 25%; se calcularon 80 episodios por grupo. Fueron seleccionados pacientes con neutropenia febril, candidatos a recibir antimicrobiano parenteral; se aleatorizaron a recibir piperacilina/tazobactam más amikacina (grupo A) o piperacilina/tazobactam (grupo B). Los desenlaces fueron falla, eventos adversos y muerte. Se emplearon las pruebas Chi cuadrada de Mantel-Haenszel y exacta de Fisher. Se calculó la reducción de riesgo relativo y absoluto (RRR y RRA), intervalos de confianza 95% (IC 95%) y número necesario a tratar (NNT). Resultados: se analizaron 88 episodios en el grupo A y 76 en el grupo B. No hubo diferencias estadísticas en características generales ni en el tipo de infecciones. No se encontró diferencia significativa en: falla 31.8% grupo A, 30.2% grupo B (RR 1.05, IC 95% 0.66-1.66, p = 0.86), ni en los eventos adversos (uno en cada grupo). La RRR fue de 1.5%, RRA de 2%, con un NNT de 67. Conclusión: la terapia con piperacilina/tazobactam sin amikacina fue tan efectiva como la terapia combinada para pacientes pediátricos con neutropenia febril.

Early discharge of pediatric patients with cancer, fever, and neutropenia with low-risk of systemic infection / Egreso temprano en pacientes pediátricos con cáncer, fiebre y neutropenia con bajo riesgo de infección sistémica

Abstract: Background: Ambulatory therapy in low-risk patients with cancer, fever, and neutropenia seems to be a secure and effective alternative. This study aimed to compare the effectiveness and safety of the antimicrobial treatment in early discharge vs. in-hospital treatment in children with cancer and febrile neutropenia (FN) with low risk of invasive bacterial infection (IBI). Methods: Quasi-experimental design with a historical cohort control group. Children with cancer during an episode of FN and low risk of IBI were included. The control group were inpatient children that received intravenous piperacillin/tazobactam. The experimental group was early discharge patients, who received 48 h of IV treatment and were switched to oral treatment. Outcomes: fever resolution, readmissions, and mortality. Results: Eighty low-risk FN episodes were included; the median age was 6 years old (2.6-11 years), and 43 (54%) were female. Main diagnoses were solid tumors (52 patients) and leukemia or lymphoma (28 patients). Forty-three patients received in-hospital treatment, and 37 were selected for early discharge (31 patients received ciprofloxacin and six received amoxicillin/clavulanate). Two patients were readmitted, one due to a relapse of fever with tumor progression and the other due to epistaxis. Adverse effects occurred in 21.6% of the early discharge group and 12% of the inpatient treatment group (p = 0.04). Conclusions: Early discharge in pediatric patients with cancer, fever, and neutropenia is an acceptable and safe alternative for low-risk patients.

Resumen: Introducción: El tratamiento ambulatorio en pacientes con cáncer, fiebre y neutropenia de bajo riesgo parece ser una alternativa segura y efectiva. El objetivo de este trabajo fue comparar la efectividad y la seguridad del tratamiento antimicrobiano en la modalidad de egreso temprano vs. el tratamiento intrahospitalario en niños con cáncer y neutropenia febril (NF), con bajo riesgo de infección bacteriana invasiva (IBI). Métodos: Diseño cuasi-experimental con un grupo control histórico. Se incluyeron niños con cáncer durante un episodio de NF con bajo riesgo de IBI. El grupo control fue constituido por pacientes que recibieron tratamiento hospitalario con piperacilina-tazobactam intravenosa. Los pacientes en el grupo de egreso temprano recibieron 48 horas de tratamiento intravenoso y egresaron con antimicrobianos por vía oral. Desenlaces: resolución de la fiebre, reingreso al hospital y muerte. Resultados: Se incluyeron 80 pacientes con NF de bajo riesgo; la mediana de edad fue de 6 años; 43 pacientes (54%) eran de sexo femenino. Los diagnósticos principales fueron tumores sólidos (52) y leucemia o linfoma (28). Cuarenta y tres pacientes recibieron tratamiento hospitalario y 37 fueron seleccionados para egreso temprano. En el grupo de egreso temprano, 31 pacientes recibieron ciprofloxacino y 6 recibieron amoxicilina-clavulanato. Dos pacientes reingresaron, uno por fiebre secundaria a progresión tumoral y otro por epistaxis. Los efectos adversos se presentaron en el 21.6% de los pacientes en el grupo de egreso temprano y en el 12% del grupo de tratamiento hospitalario (p = 0.04). Conclusiones: El egreso temprano para niños con cáncer y NF de bajo riesgo es una alternativa aceptable y segura.

Early discharge of pediatric patients with cancer, fever, and neutropenia with low-risk of systemic infection.

Background: Ambulatory therapy in low-risk patients with cancer, fever, and neutropenia seems to be a secure and effective alternative. This study aimed to compare the effectiveness and safety of the antimicrobial treatment in early discharge vs. in-hospital treatment in children with cancer and febrile neutropenia (FN) with low risk of invasive bacterial infection (IBI). Methods: Quasi-experimental design with a historical cohort control group. Children with cancer during an episode of FN and low risk of IBI were included. The control group were inpatient children that received intravenous piperacillin/tazobactam. The experimental group was early discharge patients, who received 48 h of IV treatment and were switched to oral treatment. Outcomes: fever resolution, readmissions, and mortality. Results: Eighty low-risk FN episodes were included; the median age was 6 years old (2.6-11 years), and 43 (54%) were female. Main diagnoses were solid tumors (52 patients) and leukemia or lymphoma (28 patients). Forty-three patients received in-hospital treatment, and 37 were selected for early discharge (31 patients received ciprofloxacin and six received amoxicillin/clavulanate). Two patients were readmitted, one due to a relapse of fever with tumor progression and the other due to epistaxis. Adverse effects occurred in 21.6% of the early discharge group and 12% of the inpatient treatment group (p = 0.04). Conclusions: Early discharge in pediatric patients with cancer, fever, and neutropenia is an acceptable and safe alternative for low-risk patients.

Introducción: El tratamiento ambulatorio en pacientes con cáncer, fiebre y neutropenia de bajo riesgo parece ser una alternativa segura y efectiva. El objetivo de este trabajo fue comparar la efectividad y la seguridad del tratamiento antimicrobiano en la modalidad de egreso temprano vs. el tratamiento intrahospitalario en niños con cáncer y neutropenia febril (NF), con bajo riesgo de infección bacteriana invasiva (IBI). Métodos: Diseño cuasi-experimental con un grupo control histórico. Se incluyeron niños con cáncer durante un episodio de NF con bajo riesgo de IBI. El grupo control fue constituido por pacientes que recibieron tratamiento hospitalario con piperacilina-tazobactam intravenosa. Los pacientes en el grupo de egreso temprano recibieron 48 horas de tratamiento intravenoso y egresaron con antimicrobianos por vía oral. Desenlaces: resolución de la fiebre, reingreso al hospital y muerte. Resultados: Se incluyeron 80 pacientes con NF de bajo riesgo; la mediana de edad fue de 6 años; 43 pacientes (54%) eran de sexo femenino. Los diagnósticos principales fueron tumores sólidos (52) y leucemia o linfoma (28). Cuarenta y tres pacientes recibieron tratamiento hospitalario y 37 fueron seleccionados para egreso temprano. En el grupo de egreso temprano, 31 pacientes recibieron ciprofloxacino y 6 recibieron amoxicilina-clavulanato. Dos pacientes reingresaron, uno por fiebre secundaria a progresión tumoral y otro por epistaxis. Los efectos adversos se presentaron en el 21.6% de los pacientes en el grupo de egreso temprano y en el 12% del grupo de tratamiento hospitalario (p = 0.04). Conclusiones: El egreso temprano para niños con cáncer y NF de bajo riesgo es una alternativa aceptable y segura.

Análisis de una serie de casos de tuberculosis en pacientes pediátricos atendidos en un hospital de tercer nivel / A case-series analysis of tuberculosis in pediatric patients treated in a tertiary level hospital

Resumen: Introducción: La tuberculosis (TB) continúa siendo un reto ya que las formas graves se presentan con mayor frecuencia en los menores de 5 años y el diagnóstico es complejo. El objetivo del presente trabajo fue describir las formas de presentación clínica, frecuencia, métodos de diagnóstico empleados y respuesta al tratamiento en niños con TB atendidos en un hospital de tercer nivel. Métodos: Se diseñó un estudio retrospectivo, descriptivo, de una cohorte de casos consecutivos atendidos desde enero de 2010 hasta diciembre de 2013. Se revisaron 93 expedientes clínicos de niños con diagnóstico de TB de acuerdo con la definición de la NOM-006-SSA2-2013. Se utilizó estadística descriptiva para el análisis. Resultados: El 58% de 93 niños fueron pacientes de sexo masculino con una media de edad de 7 años. El 97% contaba con antecedente de vacunación BCG; el 6% tuvo contacto con algún caso de TB. Las formas clínicas más frecuentes fueron la TB pulmonar (30.1%), ganglionar (24.7%), miliar/diseminada (16.1%), meníngea (13%) y ósea (7.5%). Los síntomas más comunes fueron fiebre y pérdida de peso (50% y 40%, respectivamente). El BAAR y el cultivo fueron positivos en el 26% y el 7% de todos los casos, respectivamente. El estudio histopatológico fue concluyente en el 90%. El tratamiento fue exitoso en el 94.6%, sin mortalidad asociada. Conclusiones: La asociación del cuadro clínico con las alteraciones en la radiografía de tórax y PPD positivo son útiles para establecer el diagnóstico presuntivo e iniciar el manejo oportuno.

Abstract: Background: Tuberculosis (TB) remains a challenge because severe forms occur most frequently in children under 5 years of age and the diagnosis is complex. The objective of this paper was to describe the clinical presentation, frequency, diagnostic methods used and response to treatment in children with TB treated at a tertiary level hospital. Methods: The study was retrospective and descriptive of a cohort of consecutive cases treated from January 2010 to December 2013. Ninety-three medical records of children diagnosed with TB according to the definition of the NOM-006-SSA2-2013 were reviewed. Descriptive statistics were used for the analysis. Results: From 93 children, 58% were male (mean age of 7 years), 97% with a history of BCG vaccination, and 6% had contact with a TB case. The most frequent clinical forms were pulmonary (30.1%), lymph node (24.7%), miliary/disseminated (16.1%), meningeal (13%), and osteal TB (7.5%). The most common symptoms were fever and weight loss (50% and 40%, respectively). BAAR and culture were positive in 26% and 7% of all cases, respectively. The histopathological study was conclusive in 90% of the cases. The treatment was successful in 94.6%, with not associated mortality. Conclusions: The association of clinical symptoms with alterations in chest radiography and positive PPD are useful in establishing the presumptive diagnosis and an early and appropriate treatment.

Lipomatosis encefalocraneocutánea: reporte de caso.

The encephalocraniocutaneous lipomatosis (ECCL), also known as Fishman or Haberland syndrome, is a rare neurocutaneous syndrome of unknown etiology. Clinically characterized by skin, eye and central nervous system lesions. We present the case of a 7-year-old female who presents to the outpatient clinic of Pediatric Neurology because of the presence of seizures detecting clinical and neuroimaging manifestations compatible with ECCL. The objective of this article is to present the case because of its rare presentation and the variety of alterations found in the tomography.

[A case-series analysis of tuberculosis in pediatric patients treated in a tertiary level hospital]. / Análisis de una serie de casos de tuberculosis en pacientes pediátricos atendidos en un hospital de tercer nivel.

BACKGROUND: Tuberculosis (TB) remains a challenge because severe forms occur most frequently in children under 5 years of age and the diagnosis is complex. The objective of this paper was to describe the clinical presentation, frequency, diagnostic methods used and response to treatment in children with TB treated at a tertiary level hospital. METHODS: The study was retrospective and descriptive of a cohort of consecutive cases treated from January 2010 to December 2013. Ninety-three medical records of children diagnosed with TB according to the definition of the NOM-006-SSA2-2013 were reviewed. Descriptive statistics were used for the analysis. RESULTS: From 93 children, 58% were male (mean age of 7 years), 97% with a history of BCG vaccination, and 6% had contact with a TB case. The most frequent clinical forms were pulmonary (30.1%), lymph node (24.7%), miliary/disseminated (16.1%), meningeal (13%), and osteal TB (7.5%). The most common symptoms were fever and weight loss (50% and 40%, respectively). BAAR and culture were positive in 26% and 7% of all cases, respectively. The histopathological study was conclusive in 90% of the cases. The treatment was successful in 94.6%, with not associated mortality. CONCLUSIONS: The association of clinical symptoms with alterations in chest radiography and positive PPD are useful in establishing the presumptive diagnosis and an early and appropriate treatment.

Síndrome de Wiskott-Aldrich: Caso clínico / Wiskott-Aldrich syndrome: Case report

El síndrome de Wiskott-Aldrich es una inmunodeficiencia primaria; con una incidencia de 3,5 a 5,2 por cada millón de recién nacidos masculinos. Se caracteriza por tener un patrón de herencia recesiva ligada al cromosoma X. En estos pacientes; se ha descrito la tríada clásica de inmunodeficiencia; microtrombocitopenia y eczema. Presentamos un paciente de 5 años de edad; hispánico; con antecedentes de numerosas infecciones desde el primer año de vida. Actualmente; presenta desnutrición crónica; talla baja secundaria y retraso en el desarrollo del lenguaje. Se diagnosticó una mutación poco frecuente del gen asociado al síndrome de Wiskott-Aldrich.

The Wiskott-Aldrich syndrome is a rare X-linked recessive immunodeficiency, with an estimated incidence of 3.5 to 5.2 cases per million males. It is characterizedby immunodeficiency, microthrombocytopenia and eczema. We present a 5-year-old Hispanic male, with a medical history of numerous infectious diseases, compromised health, chronic malnutrition, language delay and failure to thrive. An infrequent mutation in the Wiskott-Aldrich syndrome gene was found.

[Wiskott-Aldrich syndrome: Case report]. / Síndrome de Wiskott-Aldrich: Caso clínico.

The Wiskott-Aldrich syndrome is a rare X-linked recessive immunodeficiency, with an estimated incidence of 3.5 to 5.2 cases per million males. It is characterized by immunodeficiency, microthrombocytopenia and eczema. We present a 5-year-old Hispanic male, with a medical history of numerous infectious diseases, compromised health, chronic malnutrition, language delay and failure to thrive. An infrequent mutation in the Wiskott-Aldrich syndrome gene was found.

[Serum lactate as a biomarker of severe sepsis in children with cancer, neutropenia and fever]. / Lactato sérico como biomarcador de sepsis grave en niños con cáncer, neutropenia y fiebre.

BACKGROUND: Neutropenia is a common chemotherapy-derived complication in cancer patients, in whom the prevalence of sepsis ranges from 12.9 % to 17.4 %, with a lethality rate of 16 %. The aim of this study was to determine the usefulness of serum lactate as a biomarker of severe sepsis in children with cancer, fever and neutropenia. METHODS: A phase II diagnostic test study was conducted. Lactate value was measured at admission. Neutropenia episodes were classified in three groups: I, with sepsis (patients with and without severe sepsis), II, without sepsis and III, neutropenic patients without fever (controls). Sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios were calculated. The gold standard was the clinical diagnosis of severe sepsis. RESULTS: A sample of 100 neutropenia episodes was collected: 16 were classified in group I, 73 in group II, and 11 in group III. Microbiological isolates were obtained in 11 samples, 4 from patients with severe sepsis and 6 from subjects without sepsis. A serum lactate level greater than or equal to 2 mmol/L had a sensitivity of 81 %, 83 % specificity, a positive predictive value of 48 % and a negative predictive value of 95 %; the positive and negative likelihood ratios were 4.88 and 0.23, respectively. According to the ROC curve, the area under the curve was 0.851 (95 % CI = 0.725-0.977). CONCLUSIONS: A serum lactate level greater than or equal to 2 mmol/L is consistent with severe sepsis in children with cancer, fever and neutropenia who are hemodynamically stable and without hypoperfusion.

INTRODUCCIÓN: la neutropenia es una complicación frecuente secundaria a la quimioterapia en los pacientes con cáncer, en quienes la prevalencia de sepsis es de 12.9 a 17.4 % y la letalidad es de 16 %. El objetivo de este estudio fue determinar la utilidad del lactato como biomarcador de sepsis grave en niños con cáncer, fiebre y neutropenia. MÉTODOS: se realizó un estudio de prueba diagnóstica fase II. Se midieron los niveles del lactato al ingreso. Los episodios de neutropenia se clasificaron en tres grupos: I, con sepsis II, sin sepsis; III, pacientes neutropénicos sin fiebre (controles). Se calculó sensibilidad, especificidad, valores predictivos positivo y negativo e índices de verosimilitud. El estándar de oro fue el diagnóstico clínico de sepsis grave. RESULTADOS: se recolectó una muestra de 100 episodios de neutropenia: 16 clasificados en el grupo I, 73 en el grupo II y 11 en el grupo III. Hubo aislamiento microbiológico en 11 muestras, cuatro de pacientes con sepsis grave y seis sin sepsis. El nivel de lactato mayor o igual a 2 mmol/L tuvo sensibilidad de 81 %, especificidad de 83 %, valor predictivo positivo de 48 % y valor predictivo negativo de 95 %; el índice de verosimilitud positivo fue de 4.88 y el negativo de 0.23. De acuerdo con la curva ROC, el área bajo la curva fue de 0.851 (IC 95 % = 0.725-0.977). CONCLUSIONES: el nivel de lactato mayor o igual a 2 mmol/L sirvió como indicador de sepsis grave en niños con cáncer, fiebre y neutropenia, hemodinámicamente estables y sin hipoperfusión.