Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Introducción: la proteinuria en la edad pediátrica es una entidad relativamente frecuente, la cual puede ser fisiológica o patológica. La segunda, por una alteración a nivel glomerular con pérdida de proteínas de gran tamaño o a nivel tubular, caracterizada por pérdida de proteínas de bajo peso molecular y alteraciones en la excreción de iones. Entre las enfermedades hereditarias que cursan con proteinuria tubular, se ha descrito la enfermedad de Dent, una patología ligada al cromosoma X. Esta enfermedad se manifiesta principalmente en varones, pero las mujeres pueden ser portadoras y tener manifestaciones clínicas leves de la enfermedad. La primera descripción de esta enfermedad fue hecha por Dent y Friedman en 1964. La mayoría de los casos recientemente reportados han sido en China y Alemania. Objetivo: realizar una revisión general de la enfermedad de Dent y del enfoque diagnóstico de la proteinuria en la infancia con base en nuestro caso, para así, sospechar de esta enfermedad. Descripción del caso: se presenta el caso de un paciente masculino sin antecedentes prenatales ni personales de importancia, quien presenta proteinuria persistente desde los primeros meses de vida y a quien, a los 7 años de edad, se le documenta la presencia de una variante ya conocida en el gen CLCN5, causante de la enfermedad de Dent tipo 1. Discusión: la proteinuria persistente patológica en la infancia debe ser estudiada debido a su posible relación con patologías que pueden afectar la función renal. Además de la diferenciación de la proteinuria persistente, de origen glomerular y tubular, la evaluación de alteraciones en la excreción de electrolitos, puede guiarnos hacia la realización de estudios genéticos y, por ende, al diagnóstico de patologías infrecuentes como la enfermedad de Dent. Conclusión: el enfoque diagnóstico de causas poco frecuentes de proteinuria tubular en la infancia, como la enfermedad de Dent, requiere de la valoración conjunta entre nefrología pediátrica y genética clínica.

Background: In pediatric patients, proteinuria is a relatively frequent entity that can be physiological or pathological. The second one, due to an alteration at the glomerular level with the loss of large proteins or at the tubular level, characterized mainly by the loss of low molecular weight proteins and changes in the excretion of ions. Among the hereditary diseases that present with tubular proteinuria, Dent disease is a disease linked to the X chromosome. Therefore, it manifests essentially in males, but women can be carriers and have minor clinical manifestations of the disease. Dent and Friedman made the first description of this disease in 1964. Recently, most of the cases have been reported in China and Germany. Objective: To perform a revision of Dent disease, as well as the diagnostic approach of childhood proteinuria based in our case in order to suspect this disease. Case description: This is the case of a masculine patient, without relevant prenatal and personal antecedents, the son of a father with polycystic renal disease, who presents persistent proteinuria from the first months of life, and who, at seven years old, the presence of a variant in the CLCN5 gene -causing of type 1 Dent disease- was documented. Discussion: The persistent pathological proteinuria in childhood must be studied due to its possible relation with pathologies that could affect renal function. Moreover, the differentiation among glomerular and tubular proteinuria can guide us to perform additional studies, including genetic tests to diagnose infrequent pathologies like Dent disease. Conclusion: The diagnostic approach to rare causes of tubular proteinuria in childhood, such as Dent's disease, requires joint assessment between pediatric nephrology and clinical genetics.

Renal volume of five-year-old preterm children are not different than full-term controls

Abstract Objective: In previous studies, smaller renal volumes were reported in prematurely born infants, however, these renal volumes were not corrected for body surface area, the main determinant of renal size. Given the rapid growth of the renal cortex after premature birth, the authors hypothesized that corrected volumes would not differ from healthy controls. Methods: Ambispective cohort study with prospective follow-up of prematurely born babies in a large specialized center and retrospectively recruited healthy control group. Children were assessed for renal length and renal volumes at age 5 by three independent ultrasonographers. Detailed anthropometry, blood pressure and renal function were also obtained. Age independent z-scores were calculated for all parameters and compared using descriptive statistics. Results: Eighty-nine premature study participants (median 32 weeks gestational age) and 33 healthy controls (median 38 weeks gestational age) were studied. Study participants did not differ in age, sex, Afro-Colombian descent, height, blood pressure, serum creatinine, or new Schwartz eGFR. Premature study participants had a significantly lower weight (17.65 ± 2.93 kg) than controls (19.05 ± 2.81 kg, p = 0.0072) and lower body surface area. The right renal volumes were significantly smaller (39.4 vs 43.4 mL), but after correction for body surface area, the renal volume and renal length z-scores were identical for both kidneys (mean right kidney -0.707 vs -0.507; mean left kidney -0.498 vs -0.524, respectively). Conclusion: Renal volumes need to be corrected to body surface area. After correction for body surface area, 5-year-old healthy and prematurely born children have comparable renal volumes.

Rare spontaneous development of nasal septal abscess in end-stage kidney disease

@#The spontaneous development of a nasal septal abscess in patients with chronic kidney disease is hardly described in the literature. A 58-year-old man with long-standing type 2 diabetes mellitus and a history of rectal adenocarcinoma was admitted for resection of tumor recurrence. He was initiated on hemodialysis post-operatively due to worsening kidney function. He was discharged on thrice-weekly dialysis but was readmitted two months after for progressive shortness of breath. Further examination revealed severe nasal congestion from a nasal septal abscess which prompted mouth-breathing. Incision and drainage and anterior nasal packing were done, and the patient was discharged improved on broad-spectrum oral antibiotics. This case report highlights the possibility of developing nontraumatic nasal infections in patients with chronic kidney disease due to compromised host defenses.

Abdominal angiostrongyliasis, report of two cases and analysis of published reports from Colombia / Angiostrongiliasis abdominal, reporte de dos casos y análisis de los casos publicados en Colombia

Abdominal angiostrongyliasis is a parasitic zoonosis, endemic in the American continent. Its etiological agent is Angiostrongylus costaricensis, a nematode whose definitive hosts are rats and other rodents and the intermediate hosts, slugs. Mammals acquire the infection by consuming vegetables contaminated with L3 larvae. The disease shows a heterogeneous clinical spectrum and given its low incidence its diagnosis is a great challenge. In Colombia, the first case was reported in 1979 and until 1998, only five additional cases have been reported. However, in the last two decades, no new cases were reported. Here we discuss two cases of children from Huila and Caquetá departments who developed the disease. Both cases required long in-patient care and multiple surgical interventions. The diagnosis was achieved by histopathological observation of parasitic elements inside the mesenteric arteries. One of the children died while the other fully recovered. We discuss the epidemiology, pathogenic cycle, clinical presentation, diagnosis, and prevention strategies of this disease paying particular attention to our patients' features and the Colombian context.

La angiostrongiloidiasis abdominal es una zoonosis parasitaria endémica en el continente americano. Su agente etiológico es el nematodo Angiostrongylus costaricensis, cuyos huéspedes definitivos son los roedores y, los intermediarios, los caracoles y las babosas, por lo que se adquiere al consumir vegetales contaminados con larvas en estadio 3. La presentación clínica es muy variada y, dada su rareza, su diagnóstico es un desafío. En Colombia el primer caso se reportó en 1979 y, desde entonces hasta 1998, se han informado cinco casos más, aunque en las últimas dos décadas no se había reportado ningún caso. Se describen aquí dos casos de angiostrongiloidiasis en niños provenientes de los departamentos de Huila y Caquetá que requirieron una larga hospitalización y múltiples intervenciones quirúrgicas. El diagnóstico se logró al observar en los especímenes quirúrgicos larvas and huevos cuya morfología sugería una infección por nematodos; uno de los pacientes murió y el otro se recuperó satisfactoriamente. Se discuten la epidemiología, la patogenia, la presentación clínica, el diagnóstico y las estrategias de prevención de esta parasitosis, con énfasis en las características particulares de los casos descritos y en el contexto colombiano.

Relación del reflujo gastroesofágico y manifestaciones respiratorias, desde el punto de vista de la gastroenterología pediátrica / Relationship between gastroesophageal reflux and respiratory manifestations, from the pediatric gastroenterologist point of view

Gastroesophageal reflux is a frequent condition in the daily life of infants and older children. When reflux causes symptoms, it is called gastroesophageal reflux disease. Different extraesophageal symptoms have been frequently attributed to gastroesophageal reflux, however, new diagnostic techniques available, such as pHmetry with impedance measurement, have allowed us to evaluate and eventually dismiss such relationships. In this article we review the relationship between gastroesophageal reflux and laryngeal pathology, chronic cough, asthma and aspiration. In general terms, the empirical treatment of a presumed reflux is not recommended in asymptomatic patients, in whom its presence is not demonstrated by techniques such as pHmetry with impedance, given that therapeutic response is low and similar to placebo, with potential adverse effects.

El reflujo gastroesofágico es una condición frecuente en la vida diaria de lactantes y niños mayores. Cuando produce síntomas, se denomina enfermedad por reflujo gastroesofágico. Se ha atribuido frecuentemente diferentes síntomas extraesofágicos al reflujo, sin embargo, nuevas técnicas diagnósticas disponibles, como la pHmetría con medición de impedanciometría, han permitido evaluar y eventualmente descartar tales asociaciones. En este artículo se revisa la relación entre el reflujo gastroesofágico y patología laríngea, tos crónica, asma y aspiración. En términos generales, no se recomienda el tratamiento empírico de un supuesto reflujo en pacientes asintomáticos, en quienes tampoco esté demostrada su presencia por técnicas como la pHmetría con impedanciometría, dado que la respuesta terapéutica es baja y similar a placebo, con potenciales efectos adversos.

Asma: uso adecuado de dispositivos para inhalación / Asthma: correct use of inhalation drug-delivery devices

Resumen La terapia inhalada se considera la piedra angular del manejo del asma. Sin embargo, a pesar de ser la forma ideal de administración de estos medicamentos, solamente el 70% de los pacientes cumple el tratamiento adecuadamente y sólo del 39 al 67% de los médicos conocen y pueden explicar de forma adecuada las distintas técnicas de inhalación. La terapia inhalada tiene características muy particulares. El depósito pulmonar de un medicamento inhalado a través del tracto respiratorio es más complejo que cuando se administra por vía oral, y varía dependiendo de varios factores, tanto inherentes al medicamento como a la forma de administrarlo. Para que la terapia inhalada sea exitosa, se requiere que se generen partículas del medicamento de un tamaño apropiado que penetren más allá de la orofaringe y la laringe, y que puedan depositarse en los pulmones. Existen múltiples dispositivos para la administración de medicamentos en la vía respiratoria baja. Cada uno ha probado tener una eficacia similar, siempre y cuando se utilicen con la técnica correcta. La decisión para su uso se realiza con base en la edad del paciente, la capacidad de coordinar entre la inhalación y la activación del dispositivo y la presencia de síntomas agudos. La elección del dispositivo a utilizar siempre deberá hacerse de forma conjunta, evaluando pros y contras de cada uno de los dispositivos y siempre de forma individualizada.

Abstract Inhaled therapy is considered the cornerstone of asthma treatment. However, despite being the ideal form of drug delivery, it is recognized that only 70% of patients have an adequate attachment to their treatment and only 39-67% of physicians can explain the optimal inhaler technique. Inhaled therapy has very specific characteristics. Pulmonary deposit of an inhaled medication through the respiratory tract is more complex than when administered orally and depends on several factors inherent to both the medication and the administration. For successful inhaled therapy, the drug needs to be converted into particles of an appropriate size, which can enter beyond the oropharynx and larynx, and be deposited in the lungs. There are multiple devices for the administration of drugs in the lower respiratory tract, each one with a similar efficacy as long as it is used with the correct technique. The decision of which device should be used is made based on the age of the patient, the ability to coordinate between the inhalation and activation of the device, and the presence of acute symptoms. The choice of the device must be evaluated individually.

A systematic review on Vitex negundo (NIRPROMP formulations) for the treatment of acute cough of mild to moderate severity in pediatric patients

Objective@#The aim of this systematic review was to evaluate the efficacy and safety of Vitex negundo (lagundi) for the treatment of acute cough of mild to moderate severity among pediatric patients.@*Methods@#A systematic review of randomized controlled trials (RCTs) comparing lagundi to placebo for acute cough of mild to moderate severity in children and adolescents aged two to twenty one years in ambulatory settings was performed. Authors of unpublished clinical trials with existing patent numbers were contacted and permission was obtained to access and include their studies in this review. @*Results@#Seven studies were included, with a total of 308 participants. Lagundi increased the peak exploratory flow rate (PEFR) at the end of therapy compared with placebo but this was not statistically significant (p=0.36). The increase in PEFR was consistent with the results of the mechanistic isolated tissue studies that showed bronchodilating effect of lagundi. Studies using lagundi syrup showed decrease in the frequency of cough by 44–71%.@*Conclusion@#Lagundi therapy in acute cough of mild to moderate severity among pediatric patients has a bronchodilating effect and decreases the frequency and duration of coughing, with no serious adverse effects.

Dolor y distensión abdominal: abordaje diagnóstico a distintas edades / Abdominal pain and distention: diagnostic approach at different ages

Fluctuating abdominal pain and bloating suggest gastrointestinal origin with multiple causes. In adults, patients fulfilling the Rome criteria for irritable bowel syndrome (IBS) have a low probability of neoplasms or intestinal inflammatory diseases. In these patients it is cost effective to request fecal calprotectin and celiac disease serology. Due to the high probability of nocebo effect, the diagnosis of sensitivity to non celiac and food allergies should require a blind rechallenge. It is recommended to evaluate other non ominous diagnostic options in a second stage if there is not good control of symptoms. In adults that do not fulfil the criteria of IBS or in adults older than 50 it is often necessary to request more studies, including endoscopic examinations. In children, abdominal pain and bloating occur frequently in the context of excessive consumption of sugar (including fructose, lactose and sorbitol). In infants it can occur in the context of congenital malformations, infant colics and food allergies. An active search for symptoms and signs of alarm is recommended. In their absence the performance of an endoscopic study is low. The use of celiac disease serology is also recommended, but the use of fecal calprotectin has the limitation that normal values are not present below age 4, so its use is not recommended under that age.

El dolor abdominal y la distensión abdominal fluctuantes sugieren origen gastrointestinal, con múltiples causas. En adultos, los pacientes que cumplen criterios de Roma para Síndrome de Intestino Irritable (SII) tienen una baja probabilidad de neoplasias o enfermedades inflamatorias intestinales (EII). En estos pacientes, es costoefectivo solicitar calprotectina fecal y serología de enfermedad celiaca. Por la alta probabilidad de efecto nocebo, el diagnóstico de sensibilidad al gluten no celiaca (SGNC) y alergias alimentarias debería requerir un rechallenge ciego. Es recomendable evaluar otras opciones diagnósticas no ominosas en una segunda etapa, si no hay buen control sintomático. En adultos que no cumplen criterios de SII o en adultos mayores de 50 años, suele requerirse más cantidad de estudios, incluyendo endoscópicos. En niños, el dolor abdominal y distensión ocurren frecuentemente en el contexto de consumo excesivo de azúcares (incluyendo fructosa, lactosa y sorbitol). En lactantes puede ocurrir también en el contexto de malformaciones congénitas, cólicos del lactante y alergia alimentaria. Se recomienda la búsqueda activa de signos y síntomas de alarma. En su ausencia el rendimiento del estudio endoscópico es bajo. También se recomienda el uso de serología de enfermedad celíaca, pero el uso de calprotectina fecal tiene la limitación de ausencia de valores de normalidad hasta los 4 años, por lo que su uso no se recomienda bajo esa edad.

Disease characteristics of Takayasu’s Arteritis among Filipino patients seen at Rheumatology Clinics

Introduction@#Takayasu’s arteritis (TA), a large vessel vasculitis has various initial presenting manifestations; making it difficult to diagnose. Hence, the number of those with the disease in the population is underestimated. The study intends to update local data and to describe different presentations of the disease to enhance awareness for TA.@*Methods@#This is a retrospective study done in a tertiary government hospital. Twenty-two out of twenty three charts of patients diagnosed with TA based on the 1990 ACR criteria were reviewed. Demographic profile, initial clinical manifestations, imaging, treatment and outcomes were collected. Descriptive statistics was applied. Institutional Review Board approval was obtained prior to study initiation.@*Results@#Majority (90.1%) were female; mean age at onset of symptoms and at diagnosis were 30.4 (+12.3)years and 33.2 (+12.0)years respectively. The common reasons for consult were hypertension (26.3%), claudication (21.1%) and abdominal pain (11%). Laboratories showed elevated erythrocyte sedimentation rate (87.5%), leukocytosis (43.8%), anemia (31%) and thrombocytosis (4.5%). Common imaging findings were cardiomegaly (27.3%), aortic regurgitation (27.3%) and carotid stenosis (18.2%). CT angiogram in 90% of cases demonstrated arterial wall narrowing. Other findings were aneurysm (31.8%), contour irregularities (13.6%) and femoral artery occlusion (4.5%). Treatment for active disease were glucocorticoids alone (44%) and combined glucocorticoids and other immunosuppressants (56%). Of the 22 records reviewed, six patients (27%) had stroke. Four (18.2 %) had different surgical procedures; ray amputation of toe for digital ischemia, embolectomy for digital gangrene, balloon angioplasty of the renal artery and renal angioplasty for stenosis. Two (9.1%) who had pregnancies after TA diagnosis had premature deliveries without neonatal complications. No mortality was recorded over the mean follow-up of 49.33 patient-years.@*Conclusion@#Clinicians should be aware of the different initial presenting signs and symptoms of TA since development of collateral circulation may mask other symptoms. Thus, thorough history and physical assessment are essential tools in the diagnosis of TA.

Fístula de líquido cefalorraquídeo del receso lateral de seno esfenoidal: ¿Es el canal de Sternberg un defecto real? / Cerebrospinal fluid leak of the lateral recess of the sphenoid sinus: is Sternberg's channel a real defect?

RESUMEN Las fístulas de líquido cefalorraquídeo (LCR) corresponden a una comunicación anómala entre el espacio subaracnoideo y la cavidad nasal. El origen de las fístulas laterales del seno esfenoidal se encuentra en un defecto congénito de la base del cráneo, con una incompleta o prematura fusión de los componentes óseos implicados en el complejo proceso de osificación del esfenoides. Ello origina un canal sin cobertura ósea, solo cubierto por tejido conectivo, denominado canal craneofaríngeo lateral o de Sternberg. Este es un punto débil donde pueden aparecer encefaloceles y/o fístulas, su ubicación lateral en el seno constituyen una zona de difícil abordaje quirúrgico. La reparación quirúrgica de fístulas de LCR ha progresado drásticamente con el desarrollo de ópticas endoscópicas y la mejora en el conocimiento de la anatomía de la base de cráneo. Los endoscopios permiten una visualización directa, localización del defecto y por ende una reparación precisa que traduce menos recidivas y menor morbilidad asociada a accesos transcraneales realizados previamente. El propósito de este artículo es presentar el caso de paciente con fístula del receso lateral del seno esfenoidal, su reparación endoscópica y discusión del origen de la persistencia del canal de Sternberg como causa del defecto.

ABSTRACT Fístulas liquid cerebrospinal (CSF) correspond to an abnormal communication between the subarachnoid space and the nasal cavity. The origin of lateral fístulas of the sphenoid sinus is a congenital defect of the skull base, with an incomplete or premature fusion of bone components involved in the complex process of ossification of sphenoid. This originates a channel without bone coverage, only covered by connective tissue, called channel craneofaringeo or Sternberg channel. This is a weak point where may appear encephaloceles and/or fístula, the lateral location in sinus is a difficult surgical approach. Surgical repair of fístulas of CSF has progressed dramatically with the development of endoscopic optics and the improvement in the knowledge of the anatomy of the skull base. Endoscopes enable a direct visualization, location of the defect and thus accurate reparation and less morbidity associated with transcraneales accesses made previously. The purpose of this article is to present the case of patient with fístula of the lateral recess of the sphenoid sinus, endoscopic repair and discussion of the origin of the persistence of the channel of Sternberg as a cause of the defect.

Sarcoma histiocítico: presentación de un caso probable / Histiocytic sarcoma: Presentation of a probable case

El sarcoma histiocítico es un desorden de células no Langerhans extremadamente raro con un curso clínico agresivo y limitadas opciones de tratamiento. Se presenta el caso de un paciente adulto joven de género masculino, con cuadro febril prolongado, compromiso hematológico persistente, hepatoesplenomegalia y linfadenopatias generalizadas con curso clínico fatal, en quien por las características clínicas, paraclínicas y de estudios histopatológicos como de inmunohistoquímica se llegó a dicha presunción diagnostica

Histiocytic sarcoma is an extremely rare non-Langerhans cell disorder with an aggressive clinical course and limited options for therapy. We present the case of a young adult male patient, with prolonged fever, persistent hematological involvement, hepatosplenomegaly, and generalized lymphadenopathy with a fatal clinical course, in whom we arrived to this presumptive diagnosis considering the clinical, paraclinical, and histopathological characteristics, as well as immunohistochemistry

Hematuria en la niñez: revisión sistemática cualitativa / Hematuria in the childhood A systematic cualitative review

Introducción: la hematuria aislada en niños es un hallazgo frecuente en la práctica clínica diaria del médico general y el pediatra, y un reto diagnóstico para averiguar su etiología y manejo. Objetivo: describir las pautas básicas para el diagnóstico y manejo de la hematuria aislada en menores de 18 años por el médico general y el pediatra. Materiales y métodos: se realizó una búsqueda bibliográfica sistemática en las bases de datos PubMed, ScienceDirect, LILACS y Embase, utilizando palabras claves del DeCS (español) y MeSH (inglés), mediante las combinaciones con la conjunción «AND¼ o la disyunción «OR¼, de manuscritos tipo estudios observacionales, ensayos clínicos, guías de práctica clínica y revisiones sistemáticas, publicados entre 1995 y 2017, que describieran el diagnóstico y el manejo básico de la hematuria. La calidad de los artículos fue evaluada por medio de los instrumentos PRISMA, CONSORT y STROBE, según correspondiera. Resultados: se identificaron 402 publicaciones, de las cuales 34 cumplieron con los criterios y 28 fueron seleccionadas para realizar esta revisión. Conclusiones: la hematuria aislada se define por el hallazgo de cinco eritrocitos por campo de alto poder en orina fresca centrifugada o más de cinco eritrocitos por microlitro en orina fresca no centrifugada. El diagnóstico y tratamiento se realiza por pasos: a) historia clínica y b) confirmación por microscopía óptica de alta resolución. Diferenciar la hematuria benigna de las que requieren paraclínicos de extensión es primordial para confirmarla más tempranamente, evitar procedimientos invasivos, disminuir el gasto en salud y hacer un seguimiento predictivo. (AU)

Introduction: Isolated hematuria in children is a frequent finding in the daily clinical practice of the general physician and pediatrician, being a diagnostic challenge to find out its etiology and management. Objective: To describe the basic guidelines for the diagnosis and management of isolated hematuria in children under 18 years of age by the general physician and the pediatrician. Materials and methods: A systematic bibliographic search was carried out in the databases PubMed, ScienceDirect, LILACS and Embase, using key words from DeCS (Spanish) and MeSH (English) through combinations with the conjunction "AND" or the disjunction " OR ", of manuscripts such as observational studies, clinical trials, clinical practice guidelines and systematic reviews, published between 1995 and 2017 that described the basic diagnosis and management of hematuria. The quality of the articles was evaluated through the PRISMA, CONSORT and STROBE instruments, as was appropriate. Results: 402 publications were identified, of which 34 met the criteria and 28 were selected to carry out this review. Conclusions: Isolated hematuria is defined by the finding of five erythrocytes per high power field in centrifuged fresh urine or more than five erythrocytes per microliter in fresh non-centrifuged urine Diagnosis and treatment are carried out in steps: a) clinical history, and b) confirmation by high resolution optical microscopy. Differentiating benign hematuria from those requiring paraclinical extension is essential to confirm it earlier, avoid invasive procedures, decrease health expenditure and make predictive follow-up. (AU)

Efecto de la hospitalización sobre la mantención de la lactancia materna en niños menores de 6 meses de edad / Hospitalization and continuity of breastfeeding in infants less than 6 months of age

Objective: To assess the effect of hospitalization for acute morbidity on the continuity of breastfeeding. Patients and Method: Concurrent cohort study of infants under 6 month of age admitted to the Hospital Sótero del Río, with follow-up at 2 and 6 weeks post discharge. Results: 72 patients entered the study. At the time of admission, 91.7 percent of patients were receiving breastfeeding (BF); 19.4 percent of them was exclusive breastfeeding (BFe). At 2 weeks after discharge, BF and BFe was 86 percent and 29 percent (p = NS), respectively. At 6 weeks post discharge, BF and BFe was 80 percent (p = 0.021 compared to admission) and 33 percent (p = NS) respectively. In patients admitted to the pediatric unit, there was an increase in the BFe between the second and sixth week of follow up (29.7 percent and 40.5 percent respectively, p = 0.008). Conclusions: After discharge, there was a decrease in the prevalence of BF similar to that observed in the National Breast Feeding Survey. The hospitalization did not negatively affect the maintenance of breastfeeding, on the contrary, there was an increase in those receiving exclusive breastfeeding, which could be explained by the education on breast feeding that mothers received within the hospital.

Objetivo: Evaluar el efecto de la hospitalización por morbilidad aguda sobre la continuidad de la lactancia materna (LM) en menores de 6 meses de edad. Pacientes y Método: Estudio de cohorte concurrente en pacientes ingresados al Hospital Sótero del Río, con seguimiento de lactancia materna al ingreso, a las 2 y 6 semanas post-alta. Resultados: 72 pacientes ingresaron al estudio. Al momento de la admisión, 91,7 por ciento de los pacientes recibía LM, de ellos, 19,4 por ciento era lactancia materna exclusiva (LME). A las 2 semanas post alta, LM y LME fue de 86 por ciento y 29 por ciento (p = NS), respectivamente. A las 6 semanas post alta, LM y LME fue de 80 por ciento (p = 0,021 respecto al ingreso), y 33 por ciento (p = NS) respectivamente. En los pacientes ingresados a sala, se observó un aumento en la LME entre la segunda y la sexta semana de seguimiento (29,7 por ciento y 40,5 por ciento, respectivamente, p = 0,008). Conclusiones: Posterior al alta, se observó una disminución en la prevalencia de LM similar a la caída observada en la Encuesta Nacional de Lactancia Materna. La hospitalización no afectó negativamente la mantención de la lactancia materna, al contrario, se observó un incremento en aquellos que recibían lactancia materna exclusiva, lo que podría explicarse por la educación en lactancia que reciben las madres durante la hospitalización.

Avaliação de anticoagulantes naturais e de fatores da coagulação em pacientes com distúrbios congênitos de glicosilação (DCG) tipo I / An evaluation of natural anticoagulants and coagulation factors in patients with congenital disorders of glycosylation type I

Defeitos na incorporação de N-glicanos nas proteínas humanas ocasionam um grupo de doenças multissistêmicas denominadas coletivamente distúrbios congênitos de glicosilação (DCG). Os DCG manifestam-se na infância com sintomas neurológicos que incluem principalmente atraso psicomotor, ataxia, hipotonia e episódios de acidente vascular cerebral. Várias proteínas do sistema hemostático somente tornam-se biologicamente ativas após a glicosilação. O objetivo deste estudo foi avaliar os anticoagulantes naturais (proteína S livre, proteína C e antitrombina) e os fatores da coagulação (VIII, IX e XI) em pacientes com DCG tipo I. Foram avaliados 11 pacientes com diagnóstico positivo para DCG tipo I (três do gênero masculino e oito do gênero feminino), idade média de 5,6 anos; e oito pacientes com diagnóstico negativo para DCG(quatro do gênero masculino e quatro do gênero feminino), idade média de 4,5 anos (grupo-controle). O diagnóstico de DCG tipo I foi realizado pela identificação do padrão de hipoglicosilação da transferrina plasmática. Na avaliação dos anticoagulantes naturais pode-se observar redução dos valores de PS livre e PC e uma redução marcante de AT, quando comparados com o grupo controle. Em relação aos fatores de coagulação não houve diferença significativa para os fatores VIII e IX e houve redução marcante do fator XI. Os resultados do presente estudo sugerem que a deficiência combinada de anticoagulantes naturais é responsável pelo estado pró-trombótico observado em pacientes com DCG. Sugerimos também que a análise dos parâmetros hemostáticos seja realizada para pacientes com DCG quando apresentarem sintomas clínicos de alteração do sistema hemostático e antes de procedimentos invasivos.

Defects in the biosynthesis of N-linked human protein glycosylation leads to a group of multisystem disorders collectively called congenital disorders of glycosylation (CDG). CDG present in infancy with neurologic symptoms that include psychomotor retardation, ataxia, hypotonia and stroke-like episodes. Many haemostatic system proteins only present biological activity after glycosylation. The aim of this study was to evaluate coagulation inhibitors (free protein S, protein C and antithrombin) and coagulation factors (VIII, IX and XI) in CDG type I patients. Eleven patients with CDG type I (three males and eight females) with a mean age of 5.6 years old, and eight patients without CDG (four males and four females) with a mean age of 4.5 years old (control group) were evaluated. The diagnoses of CDG type I were confirmed by isoelectric focusing of serum transferrin. When coagulation inhibitors were evaluated, decreased activity of free protein S and protein C, and a pronounced reduction of antithrombin were observed compared to the control group. There was no significant difference for coagulation factors VIII and IX but a markedly decrease in factor XI. The present results suggest that a combined deficiency of coagulation inhibitors is responsible for the pro-thrombotic state observed in CDG patients. We recommend that a haemostatic analysis should be performed in CDG patients with clinical haemostatic manifestations before invasive procedures are performed.

Severe infection with H1N1 requiring intensive care--lessons for preparedness programmes

<p><b>INTRODUCTION</b>The influenza pandemic has generated much interest in the press and the medical world. We report our experience with 15 cases of severe novel influenza A H1N1 (2009) infections requiring intensive care. The aim of this review is to improve our preparedness for epidemics and pandemics by studying the most severely affected patients.</p><p><b>CLINICAL PICTURE</b>During the epidemic, hospitals were required to provide data on all confirmed H1N1 cases admitted to an intensive care unit (ICU) to the Ministry of Health. We abstracted information from this dataset for this report. To highlight learning points, we reviewed the case notes of, and report, the fi ve most instructive cases.</p><p><b>TREATMENT</b>There were 15 cases admitted to an ICU from July 4, 2009 to August 30, 2009. Two patients died.</p><p><b>CONCLUSIONS</b>The lessons we wish to share include the following: preparedness should include having intermediate-care facilities that also provide single room isolation and skilled nursing abilities, stringent visitor screening should be implemented and influenza may trigger an acute myocardial infarction in persons with risk factors.</p>

Curvas manométricas rectales en niños: estudio promano fase I / Rectal mamometric curves in children: phase I study

Aim of this study: To evaluate the role of anorectal manometry (ARM) and determine normal ARM's parameters in a group of children referred for evaluation of defecation disorders. Patients and Methods: A retrospective review of pediatric ARMs performed over an 8-year period. Results: Records from 789 children (52.6 percent male) were reviewed. Patients were classified in four groups according to the main ARM's findings in: "Control group" (CL), "Probable megarectum group" (PMG), "Abnormalities of intrinsic innervation group" (All) and "Abnormalities of extrinsic innervation group" (AET). 79 percent of them had been referred for evaluation of chronic constipation and 10 percent because of suspicion of abnormalities of intrinsic innervation. In 94 and 83 percent of them respectively, the ARM ruled out organic causes. ARM's findings distribution was: CL (48.0 percent), PMG (42.6 percent), All (7.5 percent) and AEI (1.5 percent). CL and PMG showed age-dependent differences in manometric parameters. Differences in manometric parameters between CL, PMG, API and AEI were found. Conclusions: Four distinctive patterns were described in 789 patients referred for evaluation of their presumed defecation disorder. The main indication of ARM was study of chronic constipation, where organic problems were confirmed only in a few of them.

Propósito del estudio: Evaluar el rol de la manometría rectoanal (MRA) y establecer valores de normalidad en un grupo de niños referidos por desórdenes de defecación. Pacientes y Métodos: Revisión retrospectiva de MRA efectuadas durante un período de 8 años. Resultados: Se analizaron los resultados obtenidos de 789 niños (52,6 por ciento hombres). Éstos se clasificaron en cuatro grupos según resultado de la MRA como "Grupo control" (CL), "Grupo con probable megarrecto" (PMG), "Grupo con anormalidades de la inervación intrínseca" (All) y "Grupo con anormalidades de la inervación extrínseca" (ALE). Setenta y nueve por ciento de los pacientes fueron referidos para evaluación de constipación crónica y 10 por ciento por sospecha de anormalidades de inervación intrínseca; en 94 por ciento y 83 por ciento de ellos respectivamente, la MRA descartó causas orgánicas. Los niños se distribuyeron en: CL (48,0 por ciento), PMG (42,6 por ciento), All (7,5 por ciento) y AIE (1,5 por ciento). El grupo CL y PMG mostraron diferencias edad-dependiente en algunos parámetros manométricos. Además se encontró diferencias en parámetros manométricos entre CL, PMG, All y AIE. Conclusiones: La principal indicación de MRA fue para estudio de constipación crónica, siendo las alteraciones orgánicas confirmadas sólo en un bajo porcentaje. Se describió cuatro patrones diferentes en 789 pacientes referidos para evaluación de dificultades en la defecación.

Evaluación de los índices de monitoreo de pH esofágico y su relación con indicaciones clínicas: MePeache Fase I / Evaluation of the esophageal pH monitoring and its relation with clinical indications: MePeache Phase I

Objectives: Establish mean values and normality range for pH metric parameters, different to reflux index (RI), in esophageal pH monitoring (EpHm) according to new RI cut- off values; besides, correlate them with clinical diagnosis. Method: Retrospective study of EpHm parameters including RI, number and average length of episodes (DPE), number of episodes > 5 minutes and duration of the longest episode. 153 patients were classified according to age (Group I < 1 year and Group II > 1 year) and RI cut-off values: Normal group (NL) with physiological range (RI < 12 percent GI and < 6 percent GII) and Pathological group (PT) when RI was higher. Results: The most frequent EpHm indications were gastroesophageal reflux (GER) study (41.7 percent), airway diseases (19.9 percent) and otolaryngologic diseases (17.2 percent). There was no correlation between EpHm parameters in GI (n = 53) when analyzed by gender, referring physician or EpHm indication. In GII (n = 100), the RI for children referred by pediatricians was higher than the one for those referred by gastroenterologists in Group NL (p = 0.002). There were differences in all pH metric parameters according to NL and PT groups, except DPE. 84.9 percent and 77 percent of the EpHms in GI and GII respectively were normal. Conclusions: The EpHm parameters allow the division in NL and PT groups according to new RI cut-off values. There is a weak correlation between clinical suspicion and pHmetric diagnosis, sugesting the necessity to reevaluate EpHm indications.

Objetivos: Establecer valores promedio y rangos de normalidad de parámetros pHmétricos diferentes al índice de Reflujo (IR) en monitoreos de pH esofágico (MpHe), según nuevos valores de IR y correlacionarlos con el diagnóstico clínico. Pacientes y Método: Estudio retrospectivo de parámetros de MpHe incluyendo IR, n° y duración de episodios (DPE), n° episodios > 5 min y duración episodio mayor. Se reagrupó a los 153 pacientes según edad (Grupo I < 1 año y Grupo II > 1 año) y según IR como: Grupo Normal (NL) a aquéllos con IR en rango fisiológico (IR < 12 por ciento GI y < 6 por ciento GII) y Grupo Patológico (PT), cuando el IR superaba estos valores. Resultados: Las indicaciones más frecuentes fueron estudio de RGE (41,7 por ciento), patología de vías aéreas (19,9 por ciento) y otorrinolaringológicas (17,2 por ciento). Al comparar MpHes en GI (n = 53) no se encontraron diferencias significativas según género, médico referente o indicación. En el Grupo NL de GII (n = 100) se encontró un IR en niños referidos por pediatras mayor al de aquellos referidos por gastroenterólogos (p = 0,002). Según grupos NL y PT, todos los parámetros pHmétricos resultaron significativos (p < 0,005), excepto la DPE. 84,9 por ciento y 77 por ciento de los MpHes en GI y GII, respectivamente fueron normales. Conclusión: Los índices de MpHe permiten separar los grupos NL y PT bajo los nuevos puntos de corte del IR. Existe escaso correlato entre sospecha clínica y diagnóstico pHmétrico lo que sugiere la necesidad de reevaluar sus indicaciones clínicas.

HygR and PurR plasmid vectors for episomal transfection of Trypanosoma cruzi

This work describes the development and functional testing of two episomes for stable transfection of Trypanosoma cruzi. pHygD contained the 5'- and 3'- flanking regions of the gene encoding the cathepsin B-like protease of T. cruzi as functional trans-splicing and polyadenylation signals for the hygR ORF. Evidence is presented to support extrachromosomal maintenance and organization as tandem repeats in transfected parasites. pPac was derived from pHygD by replacement of the entire hygR ORF with a purR coding region. The ability to modify pHygD and the availability of the complete DNA sequence make these plasmids useful tools for the genetic manipulation of T. cruzi.

Actores, actoras emergentes en el campo de los derechos humanos: comunidades de las diversidades sexuales uniones de trabajadoras sexuales comerciales asociaciones de personas viviendo con el VIH sida / sss

Contiene: esta publicación contiene un estudio realizado a comunidades sexuales (gays, lesbianas, bisexuales, transexuales, transgéneros, travestis, heterosexuales), asociaciones de personas viviendo con el vih y sida (pvvs) y uniones de trabajadoras sexuales comerciales (etc). Esta investigación se basa en una lectura de los derechos universales, de los derechos ciudadanos y de los derechos fundamentales de la persona reconocidos en la Constitución Política del Estado boliviano.