Caracterización clínica de pacientes chilenos con displasia fibrosa/síndrome de McCune-Albright / Clinical features of Chilean patients with Fibrous Dysplasia/McCune-Albright Syndrome

BACKGROUND: Fibrous Dysplasia/McCune-Albright Syndrome (FD/MAS) is characterized by a spectrum of manifestations that may include fibrous dysplasia of bone and multiple endocrinopathies. AIM: To describe the clinical spectrum, the study and follow-up of patients with FD/MAS cared at our institution. MATERIAL AND METHODS: Review of medical records of 12 pediatric and adult patients (11 women) who met the clinical and genetic diagnostic criteria for FD/ MAS. RESULTS: The patients' mean age at diagnosis was 4.9 ± 5.5 years. The most common initial clinical manifestation was peripheral precocious puberty (PPP) in 67% of patients and 75% had café-au-lait spots. Fibrous dysplasia was present in 75% of patients and the mean age at diagnosis was 7.9 ± 4.7 years. Ten patients had a bone scintigraphy, with an age at the first examination that varied between 2 and 38 years of age. The most frequent location of dysplasia was craniofacial and appendicular. No patient had a recorded history of cholestasis, hepatitis, or pancreatitis. In four patients, a genetic study was performed that was positive for the pathogenic variant of guanine nucleotide binding protein, alpha stimulating (GNAS). CONCLUSIONS: These patients demonstrate the variable nature of the clinical presentation and study of FD/MAS. It is essential to increase the index of diagnostic suspicion and adherence to international recommendations.

Factibilidad, eficacia y satisfacción lograda por la intervención transdisciplinaria de un comité de ética clínico-hospitalario. Un estudio cualicuantitativo / Feasibility, effectiveness, and satisfaction achieved by the transdisciplinary intervention of a clinical-hospital ethics committee. A qualitative and quantitative study

Introducción. Las funciones del Comité de Ética Clínica (CEC) son educativas, normativas, consultivas, mediadoras y reflexivas. Como cualquier servicio de salud, las consultas de casos a un CEC deben ser sujetas a revisión y mejora de la calidad. Los objetivos fueron evaluar la factibilidad, la efectividad y la satisfacción de las recomendaciones ético-asistenciales dadas por el CEC, y evaluar su impacto en el equipo tratante y en la familia. Población y métodos. Estudio descriptivo, retrospectivo, cualicuantitativo, de casos clínicos presentados en el Comité de Ética Clínica del hospital desde el 1 de enero de 2013 hasta el 31 de diciembre de 2017, según datos del libro de actas, historias clínicas, registros de la Oficina de Comunicación a Distancia y entrevistas semiestructuradas al equipo de salud. Resultados. Se analizaron 108 casos (106 pacientes), 73 casos con encuesta y registros, y 35 solamente con registros. Los motivos principales más frecuentes de consulta fueron los siguientes: adecuación del esfuerzo terapéutico (46/42,6 %), compromiso neurológico grave (15/13,9 %), calidad de vida del paciente (11/10,2 %), conflictos entre el paciente, la familia y el sistema de salud (7/6,5 %), y rechazo del tratamiento por la familia (6/5,6 %). Se hallaron niveles altos de satisfacción (> 95 %) y niveles moderados de factibilidad (> 74 %) y efectividad (> 85 %). Solo en 50/108 casos (46,3 %) quedó registro en la historia clínica de la consulta al CEC y el 44 % de los profesionales opinó que la consulta tuvo un impacto positivo en la familia y en el paciente. Conclusiones. Los resultados contribuyeron a descubrir oportunidades de mejora, especialmente en la documentación y en la comunicación en el proceso de consulta.

Introduction. A clinical ethics committee (CEC) has educational, regulatory, advisory, mediation, and reflexive functions. As any health care service, the consults with the CEC should be subjected to review and quality improvement. The study objectives were to assess the feasibility, effectiveness, and satisfaction with the bioethical recommendations made by the CEC and assess their impact on the treating team and the patient's family. Population and methods. Descriptive, retrospective, qualitative, and quantitative study of clinical cases submitted to the hospital's CEC between January 1 st, 2013 and December 31, 2017 using data from the CEC minute book, medical records, registries from the Office for Remote Communication, and semi-structured interviews with health care team members. Results. A total of 108 cases (106 patients) were analyzed: 73 cases with survey and registries and 35 with registries only. The main most frequent reasons for consultation were adequacy of therapeutic effort (46/42.6%), severe neurological involvement (15/13.9%), patient's quality of life (11/10.2%), patient-family-health system conflict (7/6.5%), and family's refusal of treatment (6/5.6%). High levels of satisfaction (> 95%) and moderate levels of feasibility (> 74%) and effectiveness (> 85%) were observed. In only 50/108 cases (46.3%), the consultation with the CEC was registered in the medical record, 44% of health care providers stated that the consultation had a positive impact on the patient and their family. Conclusions. Results helped to establish improvement opportunities, especially in terms of documentation and communication in the consultation process.

Variación estacional de 25-hidroxi-vitamina D3, hormona paratiroidea y fosfatasa alcalina en niños escolares / Seasonal variations in 25-hydroxy vitamin D3, parathormone and alkaline phosphatase in school-aged children

INTRODUCCIÓN: El principal rol de la vitamina D es la regulación del metabolismo del calcio, cuya principal fuen te es la vitamina D3 que se obtiene principalmente por la acción de la luz ultravioleta (UV) en la piel. OBJETIVO: Evaluar las diferencias estacionales en las concentraciones de 25-hidroxi-vitamina D3 (25OHVitD3), hormona paratiroidea (PTH), fosfatasa alcalina (FA) y calcio en niños en edad esco lar. SUJETOS Y MÉTODO: Se midieron las concentraciones de 25OHVitD3, PTH, FA y calcio en niños de 5 a 8 años, sin suplementación de Vitamina D, reclutados en Santiago de Chile (latitud -33.4372) en distintas estaciones del año. El estatus de VitD fue definido como suficiente con concentraciones de 25OHVitD3 > 20 ng/mL (50 nmol/L), insuficiente 12-20 ng/mL (30-50 nmol/L) y deficiente 20 ng/mL) en verano, lo que disminuyó significativamente en invierno (54,3%, p < 0,0001). CONCLUSIONES: Las concentraciones de 25OHVitD3 disminuyeron en aproximadamente la mitad de los niños durante el invierno, lo que se vio acompañado de un aumento de la PTH y FA, asociado a concentraciones normales de calcio. De acuerdo a nuestros resultados, la suplementación con VitD en niños podría ser necesaria durante otoño e invierno.

INTRODUCTION: The main role of Vitamin D is to regulate calcium metabolism, whose main source is vitamin D3 ob tained mostly from the action of ultraviolet (UV) light on the skin. OBJECTIVE: To evaluate the seaso nal differences in the concentrations of 25-hydroxy-vitamin D3 (25OHVitD3), parathyroid hormone (PTH), alkaline phosphatase (ALP), and calcium in school-age children. SUBJECTS AND METHOD: The concentrations of 25OHVitD3, PTH, ALP, and calcium were measured in children from Santiago, Chile (latitude -33.4372), aged 5 to 8 years, without Vitamin D supplementation, in different seasons of the year. VitD status was defined as sufficient with concentrations of 25OHVitD3 >20 ng/mL (50 nmol/L), insufficient 12-20 ng/mL (30-50 nmol/L) and deficient 20 ng/mL), which decreased significantly in winter to 54.3% (p <0.0001). CONCLUSIONS: In winter, 25OHVitD3 concentrations decreased in approximately half of the children, which was associated with an increase in PTH and ALP, and normal calcium concentrations. According to our results, children may need VitD supple mentation during fall and winter.

Perfil clínico de niños con cetoacidosis diabética en una unidad de paciente crítico / Clinical profile of children with diabetic ketoacidosis in a critical care unit

INTRODUCCIÓN: La cetoacidosis (CAD) es la principal causa de morbimortalidad en niños con diabetes mellitus tipo 1 (DM1) debido a las alteraciones bioquímicas asociadas, siendo el más temido el edema cerebral, con altas tasas de mortalidad y secuelas neurológicas a largo plazo. OBJETIVO: caracterizar el perfil clínico y las complicaciones de pacientes con CAD ingresados en una unidad de paciente crítico pediátrico. PACIENTES Y MÉTODO: Revisión retrospectiva de pacientes con CAD atendidos en el Hospital Clínico de la Pontificia Universidad Católica de Chile (UPCPUC) entre los años 2000 y 2015. Se evaluaron características demográficas, manifestaciones clínicas, alteraciones bioquímicas, tratamiento, complicaciones y pronóstico. Se compararon pacientes con debut de DM1 versus diabéticos conocidos, analizándose variables según distribución. RESULTADOS: Se identificaron 46 episodios de CAD. El 67% de éstos correspondió a un debut de DM1. El 66% de los diabéticos conocidos ingresaron por mala adherencia al tratamiento. Los principales síntomas de presentación fueron: 63% polidipsia, 56% poliuria, 48% vómitos, 39% pérdida de peso y 35% dolor abdominal, con medias de Glicemia 522 mg/dL, pH 7,17 y osmolaridad plasmática 305 mOsm/L. El 89% recibió insulina en infusión. El 37% presentó hipokalemia. No se registraron episodios de edema cerebral ni muertes. CONCLUSIONES: La mayoría de los ingresos por CAD correspondió a debut de DM1. En el grupo de diabéticos conocidos, la mala adherencia al tratamiento fue la principal causa de descompensación. No se presentaron complicaciones graves ni muertes asociadas al manejo de la CAD durante el período estudiado. El diagnóstico precoz y el tratamiento adecuado y estandarizado pudieran contribuir a reducir la morbilidad y mortalidad en niños con CAD.

INTRODUCTION: Diabetic ketoacidosis (DKA) is the main cause of morbidity and mortality in children with type 1 diabetes mellitus (T1DM) due to clinical and biochemical alterations associated, cerebral edema as one of the most critical because of the high mortality rates and long-term neurological se quelae. OBJECTIVE: To analyze the clinical characteristics and complications of patients with DKA ad mitted to a pediatric intensive care unit. PATIENTS AND METHODS: Retrospective study of DKA patients treated at the Hospital Clínico, Pontificia Universidad Católica de Chile (UPCPUC) between 2000 and 2015. Demographic characteristics, clinical manifestations, biochemical alterations, treatment, complications, and prognosis were assessed. Patients with T1DM onset were compared with those patients already diagnosed with diabetes, analyzing variables according to distribution. RESULTS: 46 DKA events were identified, 67% of them were the first episode of DKA. 66% of patients already diagnosed with diabetes were admitted due to poor adherence to treatment. The main symptoms described were: 63% polydipsia, 56% polyuria, 48% vomiting, 39% weight loss and 35% abdominal pain, and mean blood sugar levels of 522 mg/dL, pH 7.17, and plasma osmolality of 305 mOsm/L. 89% of patients received insulin infusion, and 37% presented hypokalemia. No episodes of cerebral edema or deaths were registered. CONCLUSIONS: Most of the DKA admissions were due to T1DM onset. In the group of patients already diagnosed with diabetes, the poor adherence to treatment was the main cause of decompensation. There were no serious complications or deaths associated with DKA management during the studied period. Early diagnosis and proper and standardized treatment contributed to reducing morbidity and mortality in children with DKA.

Tumor de las células de granulosa: pubertad precoz en lactante menor de 1 año. Caso clínico / Granulosa cell ovarian tumor: precocious puberty in infant less than 1 year of age. Case report

Resumen Introducción: Los tumores de las células de la granulosa de tipo juvenil (TCGJ) son muy poco fre cuentes, especialmente en menores de 1 año. Los signos de pubertad precoz constituyen la presenta ción clínica más importante. Objetivo: Presentar una lactante con pubertad precoz periférica, con diagnóstico de TCGJ, discutiendo las claves de su tratamiento y seguimiento. Caso Clínico: Lactante de 10 meses que presentó telarquia, vello púbico y tumor abdominal palpable acompañado de niveles plasmáticos de Estradiol aumentados, gonadotrofinas muy bajas e imágenes que mostraban masa ovárica gigante. Se realizó salpingooforectomía, obteniéndose regresión absoluta de signos y síntomas. La biopsia demostró TCGJ por lo que se tomó inhibina B (InB) como marcador después de la cirugía. Esta hormona estaba alta inicialmente, pero descendió rápidamente. El seguimiento se basó en InB, Hormona antimulleriana (AMH) y estradiol como se describe en este tipo de tumores. Conclusiones: Los TCGJ son muy infrecuentes en pediatría; deben sospecharse en niñas con puber tad precoz periférica. El tratamiento quirúrgico en la gran mayoría es curativo, pero debe mantenerse un estricto control con marcadores tumorales, siendo los más específicos la InB y la AMH y en menor escala los niveles de Estradiol.

Abstract Introduction: Juvenile granulosa cell tumors (JGCT) are very rare, especially in infants under the age of one. The most frequent presentation is with signs of precocious puberty. Objective: Present an in fant with peripheral precocious puberty, diagnosis of JGCT and follow up. Clinical case: 10-month-old female infant with thelarche, pubic hair and palpable abdominal mass accompanied with eleva ted levels of estradiol, very low gonadotrophins and images that show a very large ovarian mass. A sapingooforectomy was carried out with full regression of symptoms and signs and improvement of laboratory exams. The biopsy showed TCGJ so inhibin B (InB) was taken as tumoral marker after surgery. This hormone was high initially, but rapidly declined. Follow-up was based on InB, antimu-llerian Hormone (AMH) and estradiol as described in this type of tumors. Conclusions: Juvenil gra nulosa cell tumors are very infrequent in pediatric age, but should be suspected in girl with peripheral precocious puberty. The majority of cases improve with surgery, but strict surveillance of tumoral markers is needed. The most specific markers are inhibin B and anti mullerian hormone (AMH), followed by estradiol levels.

Opinión de los miembros de la Justicia Nacional en lo Penal y Civil (Familia) en situaciones de limitación del soporte vital en pediatría / Opinions of members of the National Civil (Family Proceedings) and Criminal Courts in withholding or withdrawing of life support situations in pediatrics

Introducción. La posibilidad de sostener artificialmente las funciones vitales hace más difícil diferenciar al paciente en agonía terminal del paciente con posibilidades de supervivencia, lo que pone al grupo que lo rodea frente a un dilema. Por un lado, se presenta la continuación de soporte que solo prolongue un proceso irreversible, que causa daños físicos, psíquicos y a su dignidad. Por otro, la abstención o retiro de soporte vital sin la reflexión y el esfuerzo diagnóstico-terapéutico apropiado puede dejar sin esperanza y llevar a la muerte a un niño potencialmente recuperable. Además, la toma de decisiones, en estas circunstancias, enfrenta diversas barreras que dificultan lograr el mejor interés del paciente. Entre ellas, los temores legales son un factor importante. ¿En qué medida esos temores están justificados? Objetivo. Explorar la opinión del Poder Judicial de la Nación respecto al enfoque que, desde el derecho, se da a situaciones de limitación de soporte vital. Población y métodos. Profesionales activos del ámbito penal, civil y médico forense. Encuesta semiestructurada sobre tres casos hipotéticos con decisiones sobre la limitación del soporte vital. Resultados. Se repartieron 185 encuestas; se contestaron 68 (36,76%) y 51 (30,3%) fueron respondidas en forma completa. No tipificaron ningún delito en ninguno de los tres casos 28 (55%) encuestados. Trece (25%) interpretaron como delitos las decisiones de los tres casos; 6 (12%), alguno de los casos; y 4 (8%), 2 de los 3 casos. Los delitos seleccionados por los encuestados incluyeron homicidio doloso, homicidio culposo y abandono de persona. Conclusiones. El 45% de los encuestados consideraron que hubo alguna forma de delito en las decisiones tomadas.

Introduction. The possibility of sustaining life functions makes it difficult to distinguish between a dying patient and a patient with chances of survival, raising a dilemma for everyone around them. On the one side, continuing with life support techniques that would only extend an irreversible process and result in physical and psychological damage and harm their dignity. On the other side, withholding or withdrawing life support without an adequate reflection and diagnostic-therapeutic effort which may lead to the death of a potentially recoverable child. In addition, making decisions in this context implies facing barriers that hinder the possibility of pursuing the patient's best interest. Among such barriers, the fear of litigation plays a major role. To what extent is this fear justified? Objective. To explore the opinions of the members of the National Judiciary regarding the approach to withholding or withdrawing of life support from a legal stance. Population and methods. Professionals working in the criminal, civil and forensic medicine settings. Semistructured survey on three hypothetical case histories that implied making a decision to withhold or withdraw life support. Results. One hundred and eighty-five surveys were distributed; 68 (36.76%) were partially completed and 51 (30.3%), in full. Twenty-eight (55%) survey respondents did not criminalize any of the three cases presented. Thirteen (25%) respondents considered that the decisions made in the three cases constituted a crime; 6 (12%), only in one case; and 4 (8%), in two out of the three. Crimes described by survey respondents included intentional homicide, wrongful death, and failure to render assistance. Conclusions. Forty-five percent of survey respondents considered that decisions made involved some form of crime.

II Consenso de la Sociedad Chilena de Endocrinología y Diabetes sobre resistencia a la insulina

Insulin resistance is a prevalent condition commonly associated with unhealthy lifestyles. It affects several metabolic pathways, increasing risk of abnormalities at different organ levels. Thus, diverse medical specialties should be involved in its diagnosis and treatment. With the purpose of unifying criteria about this condition, a scientific-based consensus was elaborated. A questionnaire including the most important topics such as cardio-metabolic risk, non-alcoholic fatty liver disease and polycystic ovary syndrome, was designed and sent to national experts. When no agreement among them was achieved, the Delphi methodology was applied. The main conclusions reached are that clinical findings are critical for the diagnosis of insulin resistance, not being necessary blood testing. Acquisition of a healthy lifestyle is the most important therapeutic tool. Insulin-sensitizing drugs should be prescribed to individuals at high risk of disease according to clinically validated outcomes. There are specific recommendations for pregnant women, children, adolescents and older people.

Mercado de formación y disponibilidad de profesionales de ciencias de la salud en el Perú / Demand for training and availability of health science professionals in Peru

Objetivos. Describir el mercado de formación y la disponibilidad de profesionales de ocho carreras de ciencias de la salud en Perú. Además, examinar los perfiles de los médicos, enfermeros y obstetras que se forman y sus competencias para trabajar en el primer nivel de atención. Materiales y métodos. Estudio transversal utilizando datos de volumen de postulantes, ingresantes y graduados de ocho profesiones durante el periodo de 2007û2011. También se analizaron los planes de estudio de programas de formación de médicos, enfermeros y obstetras de universidades públicas y privadas, y los perfiles de competencias elaborados por los colegios profesionales y el Ministerio de Salud (MINSA). Resultados. Las tasas de ingreso en universidades públicas y privadas varían según la carrera: 4 y 28% respectivamente para Medicina Humana, y 18 y 90% para Enfermería. Además, se estima que se gradúan aproximadamente 43 y 53% de los estudiantes que ingresan a Medicina y Enfermería, respectivamente. El análisis del perfil de los profesionales recientemente graduados en Medicina, Enfermería y Obstetricia, al ser contrastados con el perfil de competencias priorizadas para los profesionales que laboran en el primer nivel de atención elaborado por el MINSA, indican que no están necesariamente ni específicamente formados para trabajar en este nivel de atención. Conclusiones. Existe demanda de formación para profesionales de ciencias de la salud y predomina la oferta por parte de universidades privadas. Los perfiles de competencias desarrollados por el MINSA muestran un claro divorcio con respecto a la oferta actual de profesionales formados.

Objectives. To describe the availability and demand of professional training programs for eight health science professions in Peru. Study the profiles of the physicians, nurses and midwives that these programs train and their competencies to work at the primary health care level. Materials and methods. Cross-sectional study using data on the volume of applicants, students and graduates of these eight professional training programs during the period 2007 - 2011. In addition, the curricula of professional training programs for physicians, nurses and midwives from public and private universities were analyzed, along with competency profiles developed by Professional Colleges and the Ministry of Health. Results. Admission rates in public and private universities vary by program: 4% and 28% respectively for medical schools, and 18% and 90% for nursing. Graduation rates were estimated at approximately 43% and 53% of students entering medicine and nursing training programs respectively. Contrasting the profiles of recently graduated professionals in medicine, nursing and midwifery, with the skills required by the Ministry of Health for professionals working in primary care the first level of care, indicate that these recently graduated professionals are not necessarily or specifically trained to work in primary care. Conclusions. Demand for professional training in health sciences exists and its supply is met predominantly by private universities. Competency profiles developed by the MOH for the basic professional health team in primary care shows a clear disconnect regarding the current supply of trained professionals.

Experiencia del Comité de Ética asistencial de un hospital pediátrico de referencia / Experience of an Ethics Committee of a pediatric reference hospital

Desde los años 60, ha habido un interés creciente por la complejidad de los problemas éticos que plantea la práctica de la medicina. Desde entonces, numerosas teorías éticas han intentado fundamentar la bioética justificando las consideraciones que han sido necesarias para la toma de decisiones. El objetivo del artículo es exponer brevemente la historia y el funcionamiento del Comité de Ética de un hospital pediátrico, así como la evolución experimentada desde sus comienzos. A lo largo de la trayectoria como Comité de Ética, se fueron realizando progresivos cambios en la forma de trabajo. En los primeros años, se exigía el cumplimiento de una serie de formalidades en la presentación de los pacientes, pero esto se fue modificando con el tiempo hasta llegar a una presentación menos rígida y más reflexiva y plural. En el caso de nuestro Comité Hospitalario de Ética, la deliberación es la herramienta principal y más valorada de su actividad en la búsqueda de la mejor opción para la asistencia de los casos problemáticos que se plantean.

Since 1960, there has been a growing interest in the complexity of the ethical problems posed by medical practice. Ever since then, many ethical theories have attempted to support bioethics, setting the necessary grounds for decision making process. The aim of this article is to briefly present the history and working of a pediatric hospital's Assistance Ethics Committee, as well as its evolution from the very beginning. Throughout the Committee's career, progressive changes were made in the way of working. During its first years, the fulfillment of certain formalities was demanded when presenting patients, but this was modified overtime towards a less rigid and more reflexive and pluralistic presentation. Regarding our Ethics Committee, deliberation is the main and most valuable tool in the search for the best option when dealing with harsh and problematic cases that are presented.

La razón cintura estatura en escolares no varía con el género, la edad ni la maduración puberal / Waist-to-height ratio does not change with gender, age and pubertal stage in elementary school children

Background: Waist-to-height ratio (WHtR) is a cardiometabolic risk indicator in children. A value greater than or equal to 0.55 is an effective screening tool for identifying obese children with metabolic syndrome. However, it is unclear whether this cutoff can be applied equally to any age or gender. Aim: To analyze the variability of WHtR by age, gender and pubertal stage in elementary school children. Patients and Methods: Cross-sectional study in 2,980 school children (6-14 years old, 51% male) of Santiago, Chile. We measured weight, height and waist circumference and calculated body mass index and WHtR. Pubertal stage was assessed and classified as peripubertal (Tanner I and II) and pubertal (Tanner III, IV and V). Results: The mean age was 9.9 ± 2.3 years, with no gender difference (p = 0.5). Eighty one percent of boys and 59.4% of girls were peripubertal (p < 0.001). The association between age-adjusted WHtR by gender and pubertal stage was not significant (p = 0.409). Therefore mean, standard deviation and percentiles of WHtR were calculated without sex and pubertal stage segmentations. Conclusions: Since WHtR does not vary with age, gender and pubertal status in elementary school children, it is possible to use a single cutoff value, previously defined in this population, to identify children with cardiometabolic risk.

Linfomas asociados a infección por VIH en pacientes del Hospital Clínico San Borja Arriarán / Fundación Arriarán2001-2008 / Lymphomas associated with HIV infection in patients at the Hospital San Borja Arriarán /Fundación Arriarán 2001-2008, Santiago, Chile

Introduction: Hodgkin lymphomas (HL) and non Hodgkin lymphomas (NHL) are frequently associated to acquired immunodeficiency syndrome in adults. Objective: To systematize the clinical features and histological characteristics of lymphomas in AIDS patients, its treatment and outcomes in our institution. Patients and Methods: Retrospective analysis of patients with HIV-associated lymphoma between January 2001 and December 2008 at the San Borja Arriarán Hospital complex. Results: Information was obtained from 30 patients with NHL and 7 with HL, with a median of 40 years. The majority of tumors were Burkitt lymphoma (47%), diffuse large cell lymphoma B-cell (37%) and NHL of T lineage (10%). There was no CNS or cavities lymphoma. Almost all patients (86.7%) with NHL were treated with CHOP chemotherapy, 57% of those receiving treatment had progression or relapse from complete remission. A rescue chemotherapy was indicated in 4 patients. 73% of patients receiving CHOP, complete 5 to 6 cycles of chemotherapy. The use of CHOP chemotherapy for the subgroup of patients with Burkitt lymphoma achieved low rates of complete remission and frequent relapse and disease progression, showing that CHOP was ineffective in improving survival, especially in high risk patients. We found statistically significant differences in survival according to IPIae (International prognostic Index age-adjusted). Conclusion: Non-Hodgkin lymphoma in HIV patients treated with chemotherapy protocols PAlNDA persists in our environment as a disease with a poor prognosis compared with findings in the international literature. The incorporation of new drugs of proven utility as rituximab and specific schemes chemotherapy could improve these results. The establishment of prognostic groups established by IPIae can guide clinical work for the use of chemotherapy tailored to their specific risk and optimized according to histological type.

Introducción: Los linfomas de Hodgkin (LH) y no Hodgkin (LNH) se asocian con alta frecuencia al síndrome de inmunodeficiencia humana en adultos. Objetivo: Sistematizar los aspectos clínicos e histológicos de los linfoma que afectan a pacientes con SIDA, su tratamiento y resultados globales en nuestra institución. Pacientes y Métodos: Análisis retrospectivo de pacientes con linfoma asociado a VIH entre enero de 2001 y diciembre de 2008 en el complejo hospitalario San Borja Arriarán. Resultados: Se obtuvo información de 30 pacientes con LNH y 7 LH, con una mediana de 40 años. Los tipos histológicos predominantes fueron linfoma de Burkitt (47 %), linfoma difuso de células grandes de estirpe B (37 %) y LNH de estirpe T (10%). No se diagnosticaron LNH del SNC ni linfoma de cavidades. Casi la totalidad de los pacientes (86,7%) con LNH se trataron con esquema CHOP, 57% de quienes recibieron tratamiento presentaron progresión o recaída desde remisión completa, ofreciéndoles una quimioterapia de rescate a cuatro pacientes. El 73% de los pacientes que recibieron CHOP lograron completar entre cinco y seis ciclos de quimioterapia. El uso de quimioterapia CHOP para el subgrupo de pacientes con linfoma de Burkitt alcanzó bajos porcentajes de remisión completa y mayoritariamente progresó la enfermedad, siendo esta quimioterapia, inefectiva para mejorar la sobrevida, especialmente en los pacientes de riesgo alto. Se encontraron diferencias estadísticamente significativas en sobrevida según el IPIae (índice internacional pronóstico ajustado por edad) al ingreso. Conclusión: El LNH en los pacientes con VIH tratados con los protocolos de quimioterapia PANDA persiste en nuestro medio como una enfermedad de muy mal pronóstico comparado con los resultados en la literatura internacional. La incorporación de nuevos fármacos de demostrada utilidad como rituximab y esquemas específicos de quimioterapia podrían mejorar estos resultados. El establecimiento de grupos pronósticos establecidos por IPIae puede orientar el trabajo clínico para el uso de quimioterapia ajustada a su riesgo específico y optimizado según tipo histológico.

Marcadores de síndrome metabólico como predictores de elevación de alanino aminotransferasa en niños / Association of metabolic syndrome markers with abnormal alanine aminotransferase levels in healthy children

Background: There is a high prevalence of non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) among pediatric patients. The identification of clinical predictors of these conditions would allow a timely treatment. Aim: To evaluate the relationship between serum alanine aminotransferase levels and parameters of metabolic syndrome in asymptomatic school students without hepatic illness. Subjects and Methods: A randomized sample of 175 children aged between 9 and 14 years (54% females) was selected, from a database of 3010 students living in Santiago, Chile. Weight, height, abdominal circumference, systolic and diastolic blood pressure were measured. A fasting blood sample was obtained to measure glucose, total cholesterol, HDL, LDL-cholesterol, triglycerides, alanine aminotransferase (ALT) and insulin levels. Results: Forty percent of participants were obese, 17% had metabolic syndrome and 13.1% had abnormal ALT levels. Compared with children with normal ALT levels, the latter had significantly higher waist obesity, body mass index, systolic and diastolic blood pressure and triglycerides. However on multiva-riate analysis, only waist obesity was independently associated with abnormal ALT levels (adjusted odds ratio 3.93, 95% confidence intervals 1.44-10.78, p = 0.008). Conclusions: Only waist obesity was independently associated with abnormal ALT levels in this sample of children.

Utilidad de la determinación basal de la hormona luteinizante para determinar la activación del eje hipotalamo-hipofisiario-gonadal (HHG) / Basal luteinizing hormone values to determine activation of the hypothalamic-pituitary-gonadal axis

Background: The stimulation test with gonadotropin-releasing factor (GnRH) is considered the gold standard to study the activation of the hypothalamic-pituitary-gonadal axis (HPG). A maximum luteinizing hormone (LH) response of more than > 5 mIU/min in girls confirms the activation of the HPG axis. With the advent of more sensitive methods of LH measurement, such as immuno chemiluminescence (ICMA), basal LH values have been proposed to predict the response and replace the GnRH test. Aim: To determine which basal LH value correlates with a GnRH test > 5 uUI/ml. Material and Methods: We retrospectively analyzed LH values, measured by ICMA, in the basal period and after GnRH stimulation, in 120 girls aged 8 +/- 1 years (60 with activated axis and 60 without activation), during 2009. The sensitivity and specificity of the different values were determined by receiver operating characteristic (ROC) curves. Results: In patients with and without activation of the HPG axis, basal LH was 0.5 +/- 0.53 and 0.15 +/- 0.12 mUI/ml, respectively (p < 0.01). According to ROC curves, a basal LH value greater or equal than 0.4 mUI/ml had a specificity of 93.2 percent and a sensitivity of 61 percent to determine activation of the HPG axis. Conclusions: A basal LH > 0.4 mUI/ml in girls with precocious puberty has a specificity of 93.2 percent to determine activation of the HPG axis.

Prevalencia de hipertensión arterial y su asociación con la obesidad en edad pediátrica / Prevalence of hypertension in school age children and its association with obesity

Background: Hypertension in children is a frequently overlooked problem that is an important cardiovascular risk factor. Aim: To determine the prevalence of hypertension among school age children. Material and Methods: Cross-sectional study of 2980 children aged 10 ± 2years (48 percent females) from 10 schools of middle and lower class in Metropolitan Santiago. Blood pressure (BP) was measured in the sitting position on three occasions after a rest period, using a mercury sphygmomanometer with appropriate cuff arm diameter, averaging the results of the measurements. Systolic and diastolic hypertension were defined as blood pressure values over 95percentilefor age, sex and height. Results: The overall prevalence of hypertension was 12.2 percent in women and 15 percent in men (p < 0.05). According to nutritional status, the prevalence was 6.7, 8.9,13.6 and 26 percent in underweight, eutrophic, overweight and obese children, respectively (p < 0.01). Compared with normal weight children, the risk of being hypertensive for overweight children was 1.6 (95 percent confidence intervals (CI) 1.2-2.3) and for obese children was 3.6 (95 percent CI 2.8-4.7). Conclusions: The studied children had a high prevalence of hypertension, that was directly related to a higher body mass index.

Prevalencia de síndrome metabólico en niños y adolescentes que consultan por obesidad / Prevalence of metabolic syndrome in children and adolescents who consult with obesity

Background: The higher prevalence of chüdhood obesity has led to search for metabolic syndrome (MS) in this age group. Aim: To study the prevalence of MS in obese children and adolescents. Material ana Methods: Cross sectional study of 255 obese children and adolescents aged 11.3 ± 2.4 years, 45 percent males, 60 percent pubertal, with a body mass Índex (BMI) z score of 2.7 ± 0.6, who were evaluated for obesity. MS was defined as the presence of at least three of the following criteria, according to Ferranti: fasting glucose (FG) ≥ 100 mg/dl, triglycerides (TG) ≥ 100 mg/dl, HDL < 50 mg/dl, waist circumference (WC) > percentile (p) 75 and blood pressure (BP) > p90. Patients were also classified using Cook criteria: FG ≥100 mg/dl, TG ≥ 110 mg/dl, HDL < 40 mg/dl, WC > p 90, BP > p 90. Results: MS was observed in 45 and 22.7 percent of patients, according to Ferranti and Cook definitions, respectively. WC was the most frequent criteria and glucose was the most uncommon. Males had higher body mass Índex, WC and TG levéis than femóles. According to Ferranti and Cook áefinitions, MS prevalence was 53.5 and 28 percent in males and 37.6 andl8.4 percent in fernales (p < 0.05). Fifty and 26.1 percent of pubertal patients exhibited MS vs 36.9 and 17.5 percent in pre-pubertal subjects (p < 0.05) using Ferranti and Cook criteria, respectively. The frequency of MS increased along with a higher BMI. Conclusions: MS is a prevalent condition in obese children and adolescents, especially in males and pubertal children. It is necessary to have a better and universal definition for MS in pediatrics including all ages, in order to be focused in obesity prevention and treatment.

Razón cintura estatura como predictor de riesgo cardiometabólico en niños y adolescentes / Waist-Height ratio as a predictor if cardio-metabolic risk in children

En niños, la obesidad general y visceral se asocian con mayor riesgo cardiometabólico. El aumento en la prevalencia del síndrome metabólico (SM) en niños y adolescentes empeora el riesgo cardiovascular. Necesitamos contar con nuevos marcadores que permitan predecir el SM en niños. Objetivo: Comparar índice de masa corporal (zIMC) con razón cintura estatura (RCE) como predictores de SM en niños chilenos. Método: Estudio transversal en 618 escolares, edad 10.8 +/- 1.9 años, 51.6 por ciento mujeres, 190 eutróficos, 174 sobrepeso, 254 obesos, estrato socioeconómico medio y medio bajo, área urbana de Santiago. Determinamos peso, talla, circunferencia de cintura, presión arterial, perfil lipídico y glicemia. Diagnóstico de SM basado en la presencia de > 3 criterios de Cook. El SM se modeló en función de RCE y z score IMC , con modelos de regresión logística. Se usaron curvas ROC para comparar RCE y zIMC como predictores de SM. Punto de corte según índice de YOUDEN. Resultados: La prevalencia de SM fue 15.37 por ciento. Promedio de z IMC + 1.22 +/- 0.90 y de RCE 0.52 +/-0.07. Punto de corte óptimo para SM: RCE 0.55 (sensibilidad 72 por ciento, especificidad 70 por ciento) yzIMC: 1.76 (sensibilidad 71 por ciento, especificidad 74 por ciento). Conclusión: RCE y zIMC predicen igualmente el riesgo cardiometabólico en niños y adolescentes. Dada la mayor facilidad de calcular RCE, la transforma en mejor herramienta definiendo riesgo en este grupo. El punto de corte > 0.55 sería buen predictor de SM en niños y adolescentes.

Among children both general and visceral obesity are associated with increased cardio-metabolic risk. The increasing prevalence of the metabolic syndrome (MS) in children and adolescents increases cardiovascular risk. Studies of new clinical markers which can easily and accurately predict MS in children and adolescents are needed. Aim: To compare zBMI and waist to height ratio (WHR) as predictors of MS in Chilean children. Method: We performed a crossectional study of 618 schoolchildren, age 11 +/- 2 years, 52 percent females, 190 eutrophic, 174 overweight, 254 obese, from middle and low social economic strata from the Santiago urban area. We determined weight, height, waist circumference, blood pressure and fasting lipids and glycemia. MS diagnosis was based on the presence of > 3 Cook criteria. MS was modeled in function of WHR and z score BMI, through logistic regression models. ROC curves were used to compare zBMI and WHR as predictors of MS. The cut off point by YOUDEN index was used. Results: The prevalence of MS was 15 percent. Mean zBMI was +1.22 +/- 0.90 and WHR 0.52 +/- 0.07. Cut off point for MS were: WHR 0,55 (sensitivity 72 percent, specificity 70 percent)and zBMI: 1.76 (sensitivity 71 percent, specificity 74 percent). Conclusions: Both WHR and zBMI similarly predicted cardio metabolic risk in children and adolescents. The greater feasibility of calculating WHR makes it a good tool for defining risk in this group. A cut off point >0.55 might be a good predictor of MS in children and adolescents.

Pubarquia precoz: Experiencia en 173 casos / Precocious pubarche: Experience in 173 cases

Background: Precocious pubarche (PP), defined as the development of sexual pubic hair before 8 years of age in females and before 9 years in males, is usually a benign condition but it can also be the first sign of an underlying disease. Aim: To analyze the etiology andperform a short term follow up in a cohort of patients with PP. Material and methods: A group of 173 patients (158 females) consulted for PP with a mean age of 7.4±0.1 years. These patients were followed between 15 to 60 months. Anthropometric measurements, bone age, serum levels of total testosterone, 17 OH progesterone (17 OHP) and dehydroepiandrosterone sulphate (DHEAS) were evaluated. Results: Mean birth weight and length was 3024.1±50.5 g and 48.5±0.3 cm,respectively. Ten percent of children were small for gestational age at birth. Bone age was accelerated by 1.1±0.01 years. One hundred and twelve patients were classified as having idiopathicPP (64.7%; 105 females), 29 as central precocious puberty (16.8%; only females), 16 as exaggerated adrenarche (EA 9.2%; 13 females) and 16 as non classical adrenal hyperplasia (9.2%; 11 females). Conclusions: PP represents a common and usually benign sign. However, 26% of cases had apathologic underlying condition. Therefore, all children with PP should be evaluated by a pediatric endocrinologist. Low birth weight was not frequent in this cohort and these patients did not show EA

¿Es la obesidad infantil un marcador independiente de riesgo de mortalidad general o cardiovascular en la vida adulta?: revisión sistemática de estudios de cohorte / Systematic review of the association between childhood obesity with adult obesity and mortality

There is still debate if childhood obesity is a risk factor for obesity or for mortality associated to cardiovascular diseases in adults. AIM: To determine if childhood obesity is a risk factor for obesity and mortality among adults, through a systematic review of the literature. MATERIAL AND METHODS: Pubmed and Lilacs databases were searched for cohort studies published until October 2006, determining an association between childhood obesity and obesity or mortality among adults. RESULTS: Eighteen articles were retrieved and in six, a consistent association between childhood and adulthood obesity was observed. In five studies, an association was found between childhood obesity and general mortality. In five of seven cohort studies, there was an association between childhood obesity and cardiovascular mortality. CONCLUSIONS: In the reviewed studies, childhood obesity is associated to obesity, cardiovascular and general mortality among adults. However the results are flawed due to the low methodological quality of the reviewed papers.