[Neutropenic enterocolitis in the pediatric cancer patient]. / Enterocolitis neutropénica en el paciente oncológico pediátrico.

Neutropenic enterocolitis (NEC) is a heterogeneous disease of the gastrointestinal tract with systemic response, that corresponds to a severe and life-threatening clinical condition in immunocompromised patients, especially in childhood cancer. The pathologic features are poorly understood, although its multifactorial cause of NEC is well established and it is associated with the cytotoxic effects of the chemotherapy agents used and recognized by the classic triad of fever, neutropenia, and abdominal pain, secondary to gastrointestinal injuries that alters mucosal permeability and helps intramural bacterial invasion. NEC is truly a clinical challenge that requires an early diagnosis and a multidisciplinary approach including basic laboratory and imagological tests in high complexity centers. We present a current review, adding epidemiological aspects, risks factors, diagnostic support elements, therapeutic considerations, and preventive measures in order to provide knowledge of this disease and help to reduce morbidity and mortality associated with it.

Enterocolitis neutropénica en el paciente oncológico pediátrico / Neutropenic enterocolitis in the pediatric cancer patient

Resumen La enterocolitis neutropénica (ECN) es una enfermedad heterogénea de foco digestivo, pero afectación sistémica, que corresponde a una condición clínica grave que amenaza la vida de pacientes inmunocomprometidos, particularmente oncológicos pediátricos. De patogenia aún poco definida y aunque de causa multifactorial, la ECN se asocia a los efectos citotóxicos de la quimioterapia empleada y se caracteriza por la triada clásica que incluye fiebre, neutropenia y dolor abdominal, donde la principal injuria se localiza en la mucosa intestinal, provocando su alteración como barrera y facilitando la invasión bacteriana intramural. La ECN constituye un reto diagnóstico para el equipo tratante, que requiere ser oportuno y contar con apoyo de un óptimo laboratorio general e imagenológico, para iniciar un completo manejo multidisciplinario en unidades y centros de alta complejidad. Se presenta una revisión actualizada del tema incorporando aspectos epidemiológicos, factores de riesgo, elementos de apoyo diagnóstico, consideraciones terapéuticas y medidas de prevención a fin de aportar en el conocimiento de esta patología, y reducir morbimortalidad en estos pacientes.

Abstract Neutropenic enterocolitis (NEC) is a heterogeneous disease of the gastrointestinal tract with systemic response, that corresponds to a severe and life-threatening clinical condition in immunocompromised patients, especially in childhood cancer. The pathologic features are poorly understood, although its multifactorial cause of NEC is well established and it is associated with the cytotoxic effects of the chemotherapy agents used and recognized by the classic triad of fever, neutropenia, and abdominal pain, secondary to gastrointestinal injuries that alters mucosal permeability and helps intramural bacterial invasion. NEC is truly a clinical challenge that requires an early diagnosis and a multidisciplinary approach including basic laboratory and imagological tests in high complexity centers. We present a current review, adding epidemiological aspects, risks factors, diagnostic support elements, therapeutic considerations, and preventive measures in order to provide knowledge of this disease and help to reduce morbidity and mortality associated with it.

Interpretación de patrones radiológicos sugerentes de COVID-19 en pediatría / Interpretation of radiological patterns suggesting COVID-19 in pediatrics

The CoVID-19 pandemic has impacted in a lesser extent and intensity to patients younger than 15 years. The role of different imaging studies of lung involvement has been extensively addressed, from the first cases of severe pneumonia and respiratory distress syndrome in adults. There are fewer reports of the comparative usefulness of conventional radiology, ultrasound, and computed axial tomography in children. Of those, ground glass opacities, crazy paving pattern and surrounding halo consolidation are the most characteristic. Even though none of them allows diagnostic confirmation, their correct interpretation helps in decision flows. Computed axial tomography is more accurate for defining the type and extent of lung parenchymal involvement. The role of the ultrasound in early stages in the emergency department is clearer in adults than in children, in whom there is a good correlation with chest tomography. This article addresses the different radiological patterns, their pathophysiological representation and differential diagnoses, in order to alert pediatricians of their interpretation, as well as the potential role of imaging diagnoses most frequently used in children with low acute respiratory infection.

La pandemia CoVID-19 ha impactado en una proporción e intensidad menor a los pacientes menores de 15 años. El rol de los diferentes estudios por imágenes del compromiso pulmonar ha sido extensamente abordado, desde los primeros casos de neumonías graves y síndrome de distrés respiratorio en adultos. En niños existen menos reportes de la utilidad de la radiología convencional, ecografía y tomografía axial computarizada. Sin embargo, los patrones más característicos observados en adultos se repiten en los niños. De ellos, el vidrio esmerilado, el patrón en empedrado y la consolidación con halo circundante son los más característicos. Aun cuando ninguno de ellos permite confirmación diagnóstica, su correcta interpretación ayuda en los flujos de decisiones. La tomografía axial computarizada es más certera para la definición de tipo y extensión del compromiso parenquimatoso pulmonar. El rol en los estadios tempranos en el servicio de urgencia de la ecografía es más claro en adultos que en niños, donde existe buena correlación con la tomografía de tórax. Este artículo aborda los diferentes patrones radiológicos, su representación fisiopatológica y diagnósticos diferenciales, con el objeto de apoyar a los pediatras en su interpretación, como también reconocer el rol de las técnicas de imágenes diagnósticas más frecuentemente utilizadas en niños con infección respiratoria aguda baja.

Timo cervical aberrante: masa cervical pediátrica inusual, revisión bibliográfica y reporte de caso / Aberrant cervical thymus: unusual type of pediatric cervical mass, bibliographic review and case report

Las masas cervicales representan un desafío habitual en pediatría, que en su mayoría son de etiología benigna. Una de las causas menos conocidas es el timo cervical aberrante, una entidad clínica resultante de anormalidades en el descenso del timo a lo largo de su ruta habitual (tracto timo-faríngeo). Este es un cuadro frecuentemente mal diagnosticado, del que se desconoce su incidencia real. Actualmente, su incidencia relativa parece ir en aumento en conjunto con la disponibilidad de la ecotomografía. En Chile no hay registro epidemiológico de este cuadro ni existen reportes de caso documentados. En este artículo se presenta una revisión bibliográfica sobre masa cervical pediátrica y un reporte de caso de un paciente chileno.

Cervical masses constitute a frequent challenge in pediatric care, mostly which are of benign nature. One of the lesser known causes is the aberrant cervical thymus, which results from embryological abnormalities during the thymus descent through its normal route (thymo-pharyngeal duct). This is a frequently misdiagnosed syndrome, for which its real incidence remains unknown. Nowadays, its relative incidence is on the rise along with ultrasound availability. There is no epidemiological record or documented case reports of this entity in Chile. In this article we present a bibliographic revision on cervical mass and a case report from a Chilean patient.

[Lumbar hypoplasia associated to thoracolumbar kyphosis in infants. Anatomic variant? Clinical cases]. / Hipoplasia vertebral asociada a cifosis toracolumbar en lactantes ¿variante anatómica? Casos clínicos.

INTRODUCTION: Vertebral hypoplasia is an anomaly of the vertebral bodies, in which they present a wedge shape, usually at the level of the thoracolumbar junction. Although it is associated with cer tain storage diseases and bone dysplasias, it is also possible to find isolated vertebral hypoplasia it in healthy infants or associated with thoracolumbar kyphosis. The objective of this report is to show the evolution of vertebral hypoplasia associated to kyphosis in two apparently health children. CASE REPORT: Two cases of infants diagnosed with clinically visible lumbar kyphosis when they were sitting. Spine X-rays of both showed lumbar vertebral hypoplasia at L2 level as the only finding. After ruling out other conditions associated with vertebral hypoplasia, conservative management was indicated; in the first case a clinical-radiological follow-up and in the second one, a corset given the magnitude of kyphosis. The evolution was favorable, with complete radiological clinical resolution at the age of 15 months in the first case and clinical regression in the second, in which, at 3 years and 4 months of age, an image of mild vertebral hypoplasia persisted. CONCLUSIONS: Isolated vertebral hypoplasia or associated to kyphosis may be considered a minor anomaly or anatomic variant of infant spine development, however, it requires follow-up until its normalization.

Hipoplasia vertebral asociada a cifosis toracolumbar en lactantes: ¿variante anatómica? casos clínicos / Lumbar hypoplasia associated to thoracolumbar kyphosis in infants: anatomic variant? clinical cases

INTRODUCCIÓN: La hipoplasia vertebral es una anomalía de los cuerpos vertebrales, en la que estos tienen forma de cuña, habitualmente a nivel de la unión toracolumbar. Si bien se asocia a ciertas enfermedades de depósito y displasias óseas, es posible encontrar una hipoplasia vertebral aislada en lactantes sanos, o en presencia de cifosis toracolumbar. El objetivo del presente manuscrito es dar a conocer la evolución de la hipoplasia vertebral asociada a cifosis en dos lactantes aparentemente sanos. CASOS CLÍNICOS: Se presentan dos casos de lactantes en que se pesquisó cifosis lumbar clínicamente visible durante la sedestación. Las radiografías de columna de ambos mostraron hipoplasia vertebral lumbar a nivel de L2 como único hallazgo en la morfología vertebral. Luego de descartar causas asociadas, se indicó manejo conservador: en el primer caso, en que la cifosis era de 18° se indicó seguimiento clínico-radiológico; mientras que, en el segundo caso, que tenía una angulación de 57° en sedestación, se indicó tratamiento con corsé. La evolución en ambos fue favorable, con re solución clínica radiológica a la edad de 15 meses en el primer caso y en el segundo caso, a los 3 años y 4 meses se constató regresión clínica de la cifosis con persistencia de una imagen de leve hipoplasia vertebral. CONCLUSIONES: La hipoplasia vertebral aislada o asociada a cifosis puede considerarse una anomalía menor o bien una variante anatómica del desarrollo de la columna del lactante, no obstan te, requiere seguimiento hasta su normalización.

INTRODUCTION: Vertebral hypoplasia is an anomaly of the vertebral bodies, in which they present a wedge shape, usually at the level of the thoracolumbar junction. Although it is associated with cer tain storage diseases and bone dysplasias, it is also possible to find isolated vertebral hypoplasia it in healthy infants or associated with thoracolumbar kyphosis. The objective of this report is to show the evolution of vertebral hypoplasia associated to kyphosis in two apparently health children. CASE REPORT: Two cases of infants diagnosed with clinically visible lumbar kyphosis when they were sitting. Spine X-rays of both showed lumbar vertebral hypoplasia at L2 level as the only finding. After ruling out other conditions associated with vertebral hypoplasia, conservative management was indicated; in the first case a clinical-radiological follow-up and in the second one, a corset given the magnitude of kyphosis. The evolution was favorable, with complete radiological clinical resolution at the age of 15 months in the first case and clinical regression in the second, in which, at 3 years and 4 months of age, an image of mild vertebral hypoplasia persisted. CONCLUSIONS: Isolated vertebral hypoplasia or associated to kyphosis may be considered a minor anomaly or anatomic variant of infant spine development, however, it requires follow-up until its normalization.

Hiperuricemia y componentes del síndrome metabólico en niños y adolescentes obesos / Hyperuricaemia and metabolic syndrome in obese children and adolescents

Introducción: La hiperuricemia se ha observado como una alteración metabólica adicional en pacientes adultos obesos, pero es poco conocida su magnitud en pacientes pediátricos. Objetivos: Analizar la asociación entre ácido úrico sérico (AUS) con magnitud de la obesidad general y visceral y con otras mediciones bioquímicas en niños y adolescentes obesos de Santiago, Chile. Sujetos y método: En un estudio transversal se evaluaron 770 niños (edades: 6-15 años) de un colegio público de Santiago, Chile, encontrando 227 obesos (29%) (IMC > 2 DE, estándares OMS). Se seleccionaron aleatoriamente 90 niños y aceptaron participar 77, sin otras enfermedades crónicas. Se evaluó: peso, talla, perímetro abdominal, adiposidad visceral con ultrasonografía abdominal y mediciones metabólicas: insulinemia, glucemia (HOMA), lípidos séricos, aspartato aminotransferasa, alanina aminotransferasa (ALT) y AUS. Resultados: Las concentraciones de AUS fueron 0,200 ± 0,065 mmol/l. El AUS fue mayor en niños con valores de hiperinsulinismo (ajustado según edad): 0,221 ± 0,075 vs 0,183 ± 0,054 mmol/l (p < 0,01), sin diferencias según HOMA; las diferencias también se observaron según ALT (> vs < 26 U/ml): 0,238 ± 0,070 vs 0,178 ± 0,054 mmol/l, p < 0,001; la regresión logística controlando por sexo, edad e intensidad de la obesidad mostró solo las ALT asociadas a aumento de AUS. No hubo asociación de mayor AUS con magnitud de IMC, adiposidad visceral o hígado graso. Conclusiones: Los niños y adolescentes obesos de Santiago, Chile, tienen una uricemia mayor en asociación a un aumento de la actividad de la enzima ALT e hiperinsulinismo. Se justifica analizar uricemia en el estudio de niños obesos y en su seguimiento.

Introduction: Hyperuricaemia has been suggested as an additional metabolic factor in adult obese patients, but it has not been sufficiently studied in paediatric. Objectives: To assess the relationship between serum uric acid levels (SUAL) with the level of general and visceral obesity, and other biochemical parameters in children and adolescents of Santiago, Chile. Subjects and method: A cross sectional study was conducted on 770 children and adolescents (ages: 6-15 y.) from a public school in Santiago, Chile, of whom 227 (29%) were obese (BMI > 2 SD, WHO growth standards). Ninety subjects were randomly selected and 77 with no other chronic disease (41 males) accepted to participate. Data was collected on weight, stature, abdominal circumference (AC), visceral adiposity using ultrasound, and other biochemical measurements including fasting glucose, insulin, serum lipids, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and SUAL. Results: The mean SUAL was 0.200 ± 0.065 mmol/L, and was increased in children with hyperinsulinism (adjusted by age: 0.221 ± 0.075 vs 0.183 ± 0.054 mmol/L; P < .01), with no significant differences according to HOMA. Differences were also found between children with ALT > or < 26 U/mL: 0.238 ± 0.070 vs 0.178 ± 0.054 mmol/L, P < .001. The logistic regression showed the increased SUAL was only associated with increased ALT. No significant differences were found in general or visceral adiposity measurements or fatty liver. Conclusions: Children and adolescents from Santiago, Chile have higher uric acid serum uric acid levels as well as an association with increased ALT and insulin. It is demonstrated in this study that uric acid should be measured in obese children and adolescents, and in their follow up.

[Hyperuricaemia and metabolic syndrome in obese children and adolescents]. / Hiperuricemia y componentes del síndrome metabólico en niños y adolescentes obesos.

INTRODUCTION: Hyperuricaemia has been suggested as an additional metabolic factor in adult obese patients, but it has not been sufficiently studied in paediatric. OBJECTIVES: To assess the relationship between serum uric acid levels (SUAL) with the level of general and visceral obesity, and other biochemical parameters in children and adolescents of Santiago, Chile. SUBJECTS AND METHOD: A cross sectional study was conducted on 770 children and adolescents (ages: 6-15 y.) from a public school in Santiago, Chile, of whom 227 (29%) were obese (BMI>2 SD, WHO growth standards). Ninety subjects were randomly selected and 77 with no other chronic disease (41 males) accepted to participate. Data was collected on weight, stature, abdominal circumference (AC), visceral adiposity using ultrasound, and other biochemical measurements including fasting glucose, insulin, serum lipids, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and SUAL. RESULTS: The mean SUAL was 0.200±0.065 mmol/L, and was increased in children with hyperinsulinism (adjusted by age: 0.221±0.075 vs. 0.183±0.054 mmol/L; P<.01), with no significant differences according to HOMA. Differences were also found between children with ALT>or<26 U/mL: 0.238±0.070 vs. 0.178±0.054 mmol/L, P<.001. The logistic regression showed the increased SUAL was only associated with increased ALT. No significant differences were found in general or visceral adiposity measurements or fatty liver. CONCLUSIONS: Children and adolescents from Santiago, Chile have higher uric acid serum uric acid levels as well as an association with increased ALT and insulin. It is demonstrated in this study that uric acid should be measured in obese children and adolescents, and in their follow up.

Secuencia de regresión caudal: caso clínico-radiológico / Caudal regression sequence: clinical-radiological case

El síndrome de regresión caudal es una malformación congénita poco frecuente que abarca un gran espectro de formas de presentación. Caracterizado por el compromiso musculoesquelético caudal, se puede asociar a defectos neurológicos, gastrointestinales, renales y genitourinarios. Aunque su etiología aún no se encuentra aclarada, se ha asociado a la presencia de diabetes materna y a mutaciones en el gen homeobox HBLX9. Su diagnóstico se basa en un buen estudio ecográfico prenatal, un examen físico detallado y estudio imagenológico posnatal con radiografía y/o resonancia magnética. El síndrome de regresión caudal requiere un manejo multidisciplinario en el cual el control metabólico de la diabetes gestacional constituye la mejor medida preventiva disponible en la actualidad. Se presenta el caso e imágenes de un recién nacido de término de sexo masculino, hijo de madre diabética pregestacional con mal control metabólico y diagnóstico ecográfico prenatal de malformación de columna lumbar, huesos iliacos y extremidades inferiores. Nace en buenas condiciones y se confirma el diagnóstico de síndrome de regresión caudal mediante estudio radiográfico y resonancia magnética compatibles.

Caudal regression syndrome is an uncommon congenital malformation that includes a wide spectrum of clinical presentations. Characterised by caudal musculoskeletal compromise, it can be associated to neurological, gastrointestinal, renal and genitourinary defects. Although the specific aetiology has not been clarified, it has been associated with the presence of maternal diabetes and mutations in homeobox gene HBLX9. Its diagnosis is based on a good prenatal ultrasound detection, detailed physical examination, and post-natal imaging study using radiography and magnetic resonance. Caudal regression syndrome requires multidisciplinary management, and it seems that good metabolic control of gestational diabetes constitutes the best preventive measure available. We present the clinical case and images of a male term newborn, born to a pregestational diabetic mother with poor metabolic control and a prenatal ultrasound diagnosis of lumbar spine, iliac bones and lower limbs malformation. Born in good conditions, the diagnosis was confirmed using X-rays and magnetic resonance.

[Caudal regression sequence: clinical-radiological case]. / Secuencia de regresión caudal: caso clínico-radiológico.

Caudal regression syndrome is an uncommon congenital malformation that includes a wide spectrum of clinical presentations. Characterised by caudal musculoskeletal compromise, it can be associated to neurological, gastrointestinal, renal and genitourinary defects. Although the specific aetiology has not been clarified, it has been associated with the presence of maternal diabetes and mutations in homeobox gene HBLX9. Its diagnosis is based on a good prenatal ultrasound detection, detailed physical examination, and post-natal imaging study using radiography and magnetic resonance. Caudal regression syndrome requires multidisciplinary management, and it seems that good metabolic control of gestational diabetes constitutes the best preventive measure available. We present the clinical case and images of a male term newborn, born to a pregestational diabetic mother with poor metabolic control and a prenatal ultrasound diagnosis of lumbar spine, iliac bones and lower limbs malformation. Born in good conditions, the diagnosis was confirmed using X-rays and magnetic resonance.

Ultrasonido en tórax pediátrico / Ultrasound in pediatric chest

Ultrasound is a second line technique in the pediatric chest imaging evaluation. In some specific conditions like pleural effusions, diaphragmatic motility and chest injuries, ultrasound provides outstanding and sometimes better information safely and efficiently as it can be performed bedside, usually without sedation and as a non-ionizing radiation alternative. The main pediatric chest ultrasound applications are reviewed and shown in a pictogram assay.

Para el estudio del tórax en pediatría, el ultrasonido es una herramienta diagnóstica complementaria. Las ventajas propias de la técnica como ausencia de radiación, la evaluación de la motilidad en tiempo real y alta resolución de estructuras de la pared y espacio pleural, muestran claras ventajas en patologías específicas como el derrame pleural, la motilidad diafragmática y las lesiones de la pared del tórax. Se revisa en una muestra pictográfica las principales indicaciones y utilidad de esta técnica.

Caso clínico-radiológico para diagnóstico / Radiological case report

La disostosis cleidocraneal (DCC) es un trastorno congénito poco frecuente, que sigue un patrón de herencia autosómico dominante con expresividad variable. Clínicamente se caracteriza por agenesia o disgenesia clavicular, Tórax acampanado, fontanelas amplias, retraso o ausencia en la osificación de las ramas del pubis e hipoplasia de las alas ilíacas. La erupción dental en el período lactante es de aparición tardía al igual que la erupción de la dentición definitiva. La dislocación hombros y la artrosis en el adulto son las complicaciones descritas. No existe un tratamiento específico para esta patología. Se presenta caso clínico y visión de la literatura.

Cleidocranial dysostosis (CCD) is a rare inherited congenital disorder that presents an autosomal dominant pattern with variable expressivity. Clinically, it is characterized by agenesis or dysgenesis of clavicle, bell shaped thorax, large fontanelles, delayed or absent ossification of pubic bones and hypoplasia of iliac wings. Both infant and permanent teeth eruption is delayed. Shoulder dislocation and osteoarthritis are complications described in adults. There is no specific treatment for this condition. Case report and literature reviews are presented.