Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Discopatía calcificante de la infancia: una infrecuente causa de tortícolis adquirida. Caso clínico / Pediatric intervertebral disc calcification: A rare cause of acquired torticollis. Case report

Introducción: El dolor y la contractura muscular cervical son motivos de consulta usuales en los servicios de urgencia pediátrica. El primer enfrentamiento es la anamnesis y examen físico minucioso. Ante la sospecha de etiología musculoesquelética se debe solicitar radiografía de columna cervical. El hallazgo de calcificación de los discos intervertebrales, en ausencia de otras lesiones radiológicas, nos debe hacer sospechar de una discopatía calcificante de la infancia. Objetivo: Presentar un caso de discopatía calcificante de la infancia, enfermedad infrecuente, que se debe tener en cuenta como diagnóstico diferencial de tortícolis y dolor cervical en la infancia. Caso clínico: Paciente varón de 7 años, sin antecedentes mórbidos ni historia de traumatismo o deportes bruscos. Consultó por historia de dolor y contractura cervical de 6 días de evolución. La velocidad de eritrosedimentación y proteína C reactiva estaban discretamente elevadas. La radiografía de columna cervical y la tomografía computarizada mostraron calcificación discal C5-C6 y protrusión discal anterior. Se hospitalizó para estudio y tratamiento del dolor, con buena respuesta clínica, continuando el manejo ambulatorio con antiinflamatorios no esteroidales y collar blando. Evolucionó con resolución de la sintomatología clínica y de las calcificaciones a 6 meses de seguimiento. Conclusiones: El hallazgo de calcificaciones de los discos intervertebrales es una infrecuente causa de tortícolis adquirida, de evolución benigna y autolimitada. Se recomienda manejo conservador por tiempo acotado y seguimiento clínico e imagenológico.

Introduction: Pain and cervical muscle spasm are common reasons why parents bring children to the pediatric emergency department. The first steps are the gathering of medical history of the patient and a physical examination. If musculoskeletal damage is suspected, cervical spine x-rays should be obtained. An intervertebral disc calcification finding, in the absence of other radiological lesions should suggest pediatric intervertebral disc calcification. Objective: To present a case of intervertebral disc calcification, a rare condition that must be considered in the differential diagnosis of torticollis and neck pain in childhood. Case report: A seven-year-old male patient without morbid history and no history of trauma or rough sport practice. He consulted the emergency room for pain and cervical contracture for the last six days. C reactive protein and red cell sedimentatio rates were slightly elevated. Imaging studies showed calcification of the C5-C6 intrvertebral disc and anterior disc protrusion. The patient was hospitalized for evaluation and pain management, with good clinical response and continue afterwards with non-steroidal anti-inflammatory drugs and a soft collar. At the 6-month-follow up, the patient had resolved symptoms and calcifications. Conclusions: Pediatric intervertebral disc calcification is a rare cause of acquired torticollis, with a benign and self-limited outcome. Conservative management, as well as clinical and imaging follow-up is recommended.

Piomiositis en niños: Reporte de 2 casos / Pyomyositis in children: Report of two cases

Pyomyositis (PM) is an uncommon pyogenic infection of skeletal muscle and, when not properly treated, it can progress to a high-risk clinical situation with high mortality. Because it usually has a subacute presentation, diagnosis is often delayed. We present two cases of PM of the paraspinal muscles in healthy children and discuss the current state of knowledge of this disease.

La piomiositis (PM) es una infección piógena infrecuente del músculo estriado y, cuando no es tratada adecuadamente, puede evolucionar hasta una situación clínica de alto riesgo vital. Debido a su presentación usualmente subaguda, el diagnóstico es a menudo tardío. Presentamos dos casos clínicos de PM de la musculatura paraespinal en escolares sanos y se describe el estado actual del conocimiento de esta enfermedad.

Mediciones de adiposidad intraabdominal por ultrasonido y factores asociados con riesgo cardiovascular en niños obesos / Ultrasound measurements of intra-abdominal adiposity and factors associated with cardiovascular risk in obese children

Background: Cardiovascular risk factors are commonly present in obese children. Aim: To evaluate the association among radiological measurements of intra-abdominal adipose tissue, and cardiovascular risk factors, in prepuberal obese children. Patients and Methods: We evaluated 30 obese (body mass index > p95) children aged 6 to 12 years (15 males). Anthropometry and blood pressure were measured. Subcutaneous and intra-abdominal fat thickness and fat area were measured by ultrasound (US) and computed tomography. Serum insulin, glucose and lipid profile were measured in a fasting blood sample. Homeostasis model assessment (HOMA) was calculated as an index of insulin resistance. Results: There was a significant correlation between US intra-abdominal fat thickness and HOMA (r = 0.47, p < 0.01), serum triglycerides (r = 0.46, p < 0.05) and with positive criteria for metabolic syndrome (r = 0.66, p < 0.01). A receiver operating curve (ROC) analysis showed that, above a cut-off of 45 mm for intra-abdominal fat thickness, US was able to identify insulin resistance with a sensibility and specificity of 79 and 69 percent respectively and metabolic syndrome with sensibility and specificity of 100 and 67 percent respectively. US and computed tomography measurements for intra-abdominal fat thickness were significantly correlated (r= 0.62, p < 0.01). Conclusions: US measurements of intra-abdominal fat thickness identify obesity-associated damage in childhood. Age-specific measurements of intra-abdominal adipose tissue may improve the detection power of this approach.

Costos médicos directos de enfermedades neumocócicas invasoras y neumonías con diagnóstico radiológico en niños chilenos / Direct medical costs of invasive pneumococcal disease and radiologically-diagnosed pneumonia among Chilean children

OBJETIVOS: Determinar los costos médicos directos relacionados con la atención sanitaria de los casos de enfermedades neumocócicas invasoras (ENI) y neumonías adquiridas en la comunidad confirmadas mediante radiología (NAC-Rx) en niños chilenos. MÉTODO: Estudio de seguimiento prospectivo de las prestaciones de salud entregadas a 594 niños de 0 a 35 meses con ENI y 1489 niños de 1 a 35 meses con NAC-Rx, diagnosticados y tratados en establecimientos de la red pública de salud de la Región Metropolitana de Chile. Las prestaciones se valoraron según las tarifas del Fondo Nacional de Salud (FONASA) y los precios de dos clínicas privadas. Se estimó la incidencia nacional anual de ENI y NAC-Rx para calcular la carga económica total nacional de la población afiliada al seguro de salud estatal. RESULTADOS: Los costos promedio de los casos que requirieron hospitalización fueron US$ 1056,20 para las ENI y US$ 594,80 para las NAC-Rx, mientras que para los casos tratados en forma ambulatoria fueron US$ 77,70 y US$ 65,20, respectivamente. Los precios por los mismos servicios de internación fueron US$ 4484,10 y US$ 2962,70 en una de las clínicas privadas y US$ 9967,50 y US$ 6578,40 en la otra. El costo anual nacional estimado de la atención de los niños menores de 5 años según las tarifas de FONASA fue de US$ 789045 para las ENI y US$ 13823289 para las NAC-Rx. CONCLUSIONES: La alta demanda asistencial y económica por NAC-Rx en niños de 0 a 3 años es una razón de salud pública tremendamente poderosa que apoya el uso sistemático de la vacunación antineumocócica en niños chilenos.

OBJECTIVES: To determine the direct medical costs of health care services for cases of invasive pneumococcal disease (IPD) and pneumonia acquired in the community and confirmed by radiology (NAC-Rx) among Chilean children. METHODS: A prospective follow-up study of the health services delivered to 594 children 0-35 months of age with IPD and 1 489 children 1-35 months with NAC-Rx, diagnosed and treated by organizations within public health network of the Región Metropolitana de Chile. The value of the health services was established according to rates supplied by the Fondo Nacional de Salud (FONASA, the National Health Fund) and prices charged by two private clinics. The national IPD and NAC-Rx rates were estimated to calculate the total national economic burden for the population covered by state health insurance. RESULTS: The mean cost of cases requiring hospitalization was US$ 1056.20 for IPD and US$ 594.80 for NAC-Rx, while that of cases treated by out-patient services was US$ 77.70 and US$ 65.20, respectively. The cost of the same services for in-patient care at the private clinics was US$ 4484.10 and US$ 2962.70 at one clinic and US$ 9967.50 and US$ 6578.40 at the other. The estimated national annual cost of services for children under 5 years of age, according to FONASA rates, was US$ 789045 for IPD and US$ 13823289 for NAC-Rx. CONCLUSION: The high demand for services and financial resources for NAC-Rx in children 0-3 years of age is a tremendously powerful public health reason to support the routine use of pneumococcal vaccination in Chilean children.

Adiposidad visceral y su asociación con lípidos séricos e insulinemis en adolescentes obesas / Visceral adiposity and its association with serum lipids in female obese teenagers

Background: Increased visceral or abdominal adipose tissue in children and adults is strongly associated with metabolic and a variety of chronic diseases. Aim: To study the association between visceral or external body measurements of adiposity with blood lipids, glucose and insulin levels, in obese female adolescents. Material and methods: In a cross-sectional study, 47 obese female adolescents (body mass index (BMI) >95th percentile) aged 10 to 15 years, were analyzed. Weight, height, BMI, Tanner pubertal stages, skinfold thickness, waist circumference, waist-to-hip ratio, fasting and 120 min post prandial blood glucose, serum insulin, and lipid profile were studied. Visceral fat was assessed by computed tomography at the L4-L5 level, measuring the fat area or the length of a straight drawn line between the spine and the internal border of the rectus abdominus muscle. Results: No association between lipid profile and BMI or external body measurements (skinfold thickness, waist circumference, waist-to-hip ratio) was observed. Total serum cholesterol >170 mg/dL was positively associated with the straight line over 63 mm (a cut-off obtained by ROC analysis (RR 2.64; 1.15-6.08). This association was statistically significant in girls in Tanner I + II (n =21; Fisher, p <0.023), but not with Tanner III + IV (n =26) stages. Increased cholesterol (>170 mg/dL) was also positively associated with a serum insulin >17 uU/mL in the Tanner I + II group (Fisher p<0.05), but not with the homeostasis model assessment of insulin resistance (HOMA). Conclusions: No external body measurement of adiposity was associated to increased serum cholesterol in these obese female adolescents. Increased total cholesterol (>170 mg/dL) was associated with visceral fat (evaluated through the straight line spina-rectus abdominus muscle), and also with a serum insulin >17 uU/ml in those teenagers with Tanner I or II pubertal stages.

Infección genital a virus herpes / Genital infection caused by herpes virus

Se efectúa un estudio bibliográfico de la infección genital por virus herpes simple destacando su sintomatología, su tratamiento en sus distintos tipos clínicos, su prevención y sus relaciones con el cáncer cérvicouterino, destacando la importancia de su sinergismo como los papilomavirus