Prevalence of dyssynergic defecation in children with constipation evaluated by high-resolution anorectal manometry.

Dyssynergic defecation, defined as the incoordination of rectoanal and abdominal muscles and the pelvic floor which are necessary for the appropriate relaxation, is characterized by paradoxical anal contraction, inadequate anal relaxation, or abnormal rectal propulsion; it is considered a cause of refractory primary constipation. The prevalence of dyssynergic defecation in the pediatric age is still little known. The studies that have evaluated the defecation dynamics through anorectal manometry suggest that 36.8% to 80.9% of children with functional constipation (FC) present dyssynergic defecation. High-resolution Anorectal Manometry (HRAM) is a tool for the evaluation of the sensitivity and defecation dynamics; it allows to establish the diagnosis of dyssynergia and its classification. The objective of this study was to determine the prevalence of dyssynergic defecation in children with FC and characterize the most common type of dyssynergia evaluated through a HRAM. In this study, 63 files of pediatric patients with FC diagnoses were included. Of these, 41.3% (n=26) were female and 58.7% (n=37) were male. The median age in the group of dyssynergia was 8 years, while for the FC group it was 9 years; the distribution by sex was similar. Of the included patients, 41.3% (n=26) showed dyssynergic defecation, and 58.7% (n=37) showed normal anorectal manometry. Regarding the type, 84.6% (n=22) were of type I, 7.7% (n=2) was the percentage for both types III and IV, and no patients were reported for type II.

Acute pancreatitis in children and adolescents: diagnostic and therapeutic approach according to management guidelines in a group of pediatricians.

BACKGROUND: Acute pancreatitis is observed more frequently in the pediatric age. Currently, there are recommendation guidelines for its proper diagnosis and treatment. The objective of this study was to evaluate the level of knowledge of the international recommendations on acute pancreatitis in pediatrics of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition in a group of pediatricians. METHODS: Observational, multicenter study, through a survey applied to pediatricians and pediatric residents. RESULTS: 48.8% of physicians had prior knowledge of the guidelines for the treatment of acute pancreatitis in children. 72.4% knew the current criteria for the diagnosis of acute pancreatitis. There were no differences in the majority of responses between pediatricians and pediatric residents. CONCLUSIONS: Although only half of the respondents followed the guidelines for diagnosis and treatment of acute pancreatitis, about three-quarters adequately use the criteria for diagnosis. There is adequate knowledge about the prescription of antibiotics and pancreatitis follow-up. There is lack of knowledge on the recommendation of monitoring vital signs and the precise time to perform cholecystectomy in the pancreatitis of biliary origin.

INTRODUCCIÓN: La pancreatitis aguda se observa con mayor frecuencia en la edad pediátrica. Actualmente existen guías de recomendaciones para su adecuado diagnóstico y tratamiento. El objetivo de este estudio fue evaluar el nivel de conocimiento de las recomendaciones internacionales sobre pancreatitis aguda de la North American Society for Pediatric Gastroenterology, Hepatology and Nutrition en un grupo de pediatras. MÉTODOS: Estudio observacional, multicéntrico, mediante una encuesta aplicada a médicos pediatras y médicos pediatras en formación. RESULTADOS: El 48.8% de los médicos tenían conocimiento de las guías para tratamiento de pancreatitis aguda en niños. El 72.4% conocían los criterios actuales para el diagnóstico de pancreatitis aguda. No hubo diferencias en la mayoría de las respuestas entre médicos pediatras y médicos pediatras en formación. CONCLUSIONES: Aunque solo la mitad de los encuestados conocían la guía para el diagnóstico y el tratamiento de la pancreatitis aguda, cerca de tres cuartas partes utilizan adecuadamente los criterios para el diagnóstico. Existe adecuado conocimiento sobre la prescripción de antibióticos y el seguimiento posterior a la pancreatitis aguda. Hay déficit en el conocimiento sobre las recomendaciones de la monitorización de los signos vitales y el momento adecuado para realizar la colecistectomía ante una pancreatitis de origen biliar.

Principales causas de riesgo obstétrico en el centro de salud "Roberto Astudillo" de la cuidad de Milagro enero-junio 2019 / Main causes of obstetric risk in the center of health "Roberto Astudillo" of the city of milagro

Un factor de riesgo obstétrico es una condición médica obstétrica o sociodemográfica que, en una mujer gestante puede ocasionar un aumento en la morbimortalidad que repercute como ya se ha mencionado a nivel materno -fetal con respecto al resto de la población. Objetivo: El objetivo primordial de evaluar el riesgo obstétrico, es poder realizar acciones preventivas que encaminen a evitar complicaciones que comprometan la vida materno fetal. Materiales y Métodos: Investigación cuantitativa documental, retrospectiva de corte transversal descriptivo. La investigación es cuantitativa, porque se obtiene datos los cuales son procesados, documental, porque toma la información de historia clínica que reposa en el área de estadística del centro de Salud Roberto Astudillo. Resultados: Dentro de los factores desencadenantes del alto riesgo obstétrico se encuentra el embarazo gemelar, y los embarazos mal controlados; entendiendo que el Síndrome de Hellp puede ser prevenible si hay un control exhaustivo desde el primer trimestre del embarazo, sobre todo cuando hay antecedentes de preeclampsia. Por otro lado, en los hábitos psicobiológicos se obtuvo una incidencia alta de consumo de café, de medicamentos, de consumo de alcohol y de pacientes con hábitos tabáquicos abandonados en el primer trimestre del embarazo (hábitos tóxicos). Aunque no hay una teoría que determine que el consumo de café influye directamente sobre la tensión arterial, se sabe, que las teofilinas estimulan los receptores B1 y B2 trayendo esto como consecuencia un leve incremento de la frecuencia cardíaca, y por ende un ligero incremento de la presión arterial. Conclusiones: Mantener una política de conducta expectante, generalmente incluye la atención intrahospitalaria con corticoides para la maduración pulmonar fetal, sulfato de magnesio (según sea necesario), antihipertensivos (según sea necesario) y monitoreo fetal y materno cuidadoso para identificar las indicaciones para el parto (por ejemplo, hipertensión no controlada, deterioro del estado de la madre y del feto, incluidos disfunción orgánica y sufrimiento fetal). Como parte de la conducta expectante, debe considerarse el traslado intraútero (antes del parto) a un centro de nivel terciario con capacidad para cuidados intensivos neonatales(AU)

An obstetric risk factor is a medical condition, obstetric or sociodemographic that, in a pregnant woman, can cause a increase in morbidity and mortality that has repercussions, as already mentioned, maternal-fetal level with respect to the rest of the population. Objective: The objective essential to assess the obstetric risk is to be able to carry out preventive actions that lead to avoid complications that compromise maternal and fetal life. Materials and Methods: Documentary quantitative research, retrospective of descriptive cross section. The research is quantitative, because it is obtained data which are processed, documentary because it takes the information from clinical history that rests in the statistics area of ​​the health center Robert Astudillo. Result: Within the triggering factors of the high obstetric risk is found in twin pregnancy, and miscarriages controlled; understanding that Hellp Syndrome can be preventable if there is comprehensive control from the first trimester of pregnancy, especially when there is a history of preeclampsia. On the other hand, in habits psychobiological results, a high incidence of coffee consumption, of drugs, alcohol consumption and patients with smoking habits abandoned in the first trimester of pregnancy (toxic habits). But not there is a theory that determines that coffee consumption directly influences on blood pressure, it is known that theophyllines stimulate B1 receptors and B2 bringing this as a consequence a slight increase in the frequency heart rate, and therefore a slight increase in blood pressure. Conclusions: Maintaining a watchful waiting policy generally includes inpatient care with corticosteroids for fetal lung maturation, magnesium sulfate (as needed), antihypertensives (as needed necessary) and careful fetal and maternal monitoring to identify indications for delivery (eg, uncontrolled hypertension, impaired of the condition of the mother and fetus, including organ dysfunction and distress fetal). As part of watchful waiting, transfer should be considered. intrauterine (before delivery) to a tertiary-level facility with the capacity to neonatal intensive care(AU)

Pancreatitis aguda en niños con enfermedades hematooncológicas: aspectos clínicos y tratamiento / Acute pancreatitis in children with hemato-oncological diseases: clinical aspects and treatment

Resumen La pancreatitis aguda es una enfermedad inflamatoria del páncreas. Se observa con mayor frecuencia en niños bajo tratamiento por alguna enfermedad hematooncológica y se asocia principalmente con la administración de L-asparaginasa. Identificar esta complicación de forma temprana y establecer un plan terapéutico adecuado puede mejorar el pronóstico y reducir el riesgo de otras complicaciones. En este trabajo se realizó una revisión crítica de la literatura actual, con especial énfasis en los aspectos clínicos, el diagnóstico y el tratamiento de la pancreatitis aguda en niños con cáncer.

Abstract Acute pancreatitis is an inflammatory disease of the pancreas. It is currently seen more frequently in children undergoing treatment for a hemato-oncological disease and it is mainly associated with the administration of L-asparaginase. The early identification of this complication and the establishment of an appropriate therapeutic plan can improve its prognosis and reduce the risk of other complications. In this article, we make a critical review of the current literature, with special emphasis on the clinical aspects, diagnosis, and treatment of acute pancreatitis in children with cancer.

Síndrome antifosfolípido catastrófico "seronegativo" en pediatría: caso clínico / "Seronegative" catastrophic antiphospholipid syndrome in pediatrics: clinical case

INTRODUCCIÓN: El síndrome antifosfolípido es una trombofilia adquirida autoinmune, caracterizada por trombosis arteriales y/o venosas. En raras ocasiones este cuadro puede tener una presentación catastrófica, de elevada mortalidad, con presencia de microangiopatia y compromiso de tres o más órganos. OBJETIVO: Describir la presentación clínica y evolución de una paciente pediátrica con síndrome antifosfolípido catastrófico, con forma de inicio seronegativa, cuya respuesta a terapia agresiva fue favorable. CASO CLÍNICO: Paciente femenina adolescente, que debutó cuadro de una semana de evolución de dolor, incremento del volumen abdominal y edema en extremidades inferiores. Se diagnosticó lupus eritematoso generalizado y se descartó proceso neoplásico. Durante su evolución pre sentó diversos eventos trómboticos, al inicio con presencia de anticuerpos antifosfolípido negativos, que posteriormente fueron positivos. Cursó con deterioro multisistémico secundario a trombosis multiorgánica, requirió soporte hemodinámico y ventilatorio. Se manejó con heparina de bajo peso molecular, plasmaféresis, anticoagulación, inmunosupresión y bolos de rituximab con excelente respuesta. CONCLUSIONES: Consideramos este caso de interés por tratarse de un diagnóstico infrecuente en la edad pediátrica y cuya sospecha, manejo intensivo y oportuno, puede cambiar el pronóstico sombrío y de alta mortalidad de estos pacientes.

INTRODUCTION: The antiphospholipid syndrome is an acquired autoimmune thrombophilia, characterized by arterial and/or venous thrombosis. Rarely, this condition can have a catastrophic presenta tion, with high mortality, and presence of microangiopathy and involvement of three or more organs. OBJECTIVE: To describe the clinical presentation and evolution of a pediatric patient with catastrophic antiphospholipid syndrome, with a seronegative onset form, whose response to aggressive therapy was favorable. CLINICAL CASE: Adolescent female, with a one-week history of pain, increased abdo minal volume and edema in the lower extremities. Generalized lupus erythematosus was diagnosed and the neoplastic process was ruled out. During its evolution, she presented various thrombotic events, initially with the presence of negative antiphospholipid antibodies, which were subsequently positive. The patient presented multisystemic failure secondary to multiorgan thrombosis, required hemodynamic and ventilatory support. It was managed with low molecular weight heparin, plas mapheresis, anticoagulation, immunosuppression and boluses of rituximab with excellent response. CONCLUSIONS: We consider this case interesting because it is an infrequent diagnosis in the pediatric age and whose suspicion, timely and aggressive intensive management, can change the poor progno sis and high mortality of these patients.

Probiotics and non-alcoholic fatty liver disease in children and adolescents: a systematic review

Background: non-alcoholic fatty liver disease (NAFLD) in childhood is an increasing global public health issue with significant long-term consequences. NAFLD management mainly consists of lifestyle modifications, however, adjunct pharmacological therapies are currently lacking. Gut microbiota manipulation via probiotics may alter the course of pediatric NAFLD. The objective of this systematic review was to synthesize all the available literature on the use of probiotics in children and adolescents with NAFLD. Methods: PubMed, EBSCOhost, Scopus, Web of Science, and Cochrane Library were systematically searched for trials on the use of probiotics in pediatric NAFLD. A quantitative DerSimonian Laird random effects meta-analysis was performed when possible; otherwise, a narrative summary of the study outcomes was presented and discussed. A separate search was completed to include all the ongoing registered trials on probiotics use in pediatric NAFLD. Results: five randomized controlled trials met the inclusion criteria. Of these, four trials were included in the final quantitative analysis. Probiotic therapy significantly reduced the levels of alanine aminotransferase (ALT) (mean difference: -10.39 [-19.85, -0.93]), however significant heterogeneity between studies was identified (I2, 93 %). Conclusions: there is insufficient evidence to support probiotics in the treatment of pediatric NAFLD given the substantial degree of discordance amongst the available trials. Lifestyle modifications focusing on maintaining a normal BMI and regular exercise continue to be the gold standard approach to treating NAFLD in children (AU)

Colestasis por deficiencia de 3β-Δ5-C27-hidroxiesteroide deshidrogenasa en un paciente con alteración en la síntesis de ácidos biliares / Cholestasis due to deficiency of 3β-Δ5-C27-hydroxysteroid dehydrogenase in a patient with altered bile acid synthesis

Resumen: Introducción: Los errores innatos en la síntesis de ácidos biliares son un grupo de defectos genéticos que representan del 1 al 2% de las enfermedades colestásicas crónicas en lactantes, niños y adolescentes. La deficiencia de 3β-Δ5-C27-hidroxiesteroide oxidoreductasa (3β-HSDH) es el defecto más comúnmente reportado. El cuadro clínico característico consiste en hepatitis neonatal, hepatomegalia, esplenomegalia, malabsorción, desnutrición y enfermedad hepática de aparición tardía. Caso clínico: Lactante masculino con antecedente de ictericia en escleras a los 4 meses que se resolvió espontáneamente; posteriormente, a los 18 meses, presentó enfermedad colestásica. Durante su abordaje se documentó gamma-glutamil transpeptidasa normal, hallazgo que es altamente sugestivo de alteración en la síntesis de ácidos biliares. El diagnóstico se realizó con espectrometría de masas en orina. Se inició tratamiento con ácido cólico oral, y presentó mejoría inmediata. Conclusiones: El resultado en los ácidos biliares urinarios es definitivo para el defecto genético y consistente con mutaciones homocigotas en el gen HSD3B7. Este padecimiento constituye un diagnóstico de exclusión en las enfermedades colestásicas de la infancia, particularmente el hallazgo de gamma-glutamil transpeptidasa normal o levemente aumentada, y responde adecuadamente al tratamiento oral, por lo que debe identificarse de forma temprana.

Abstract: Background: Inborn errors in bile acid synthesis are a group of genetic defects accounting for 1 to 2% of chronic cholestatic diseases in infants, children and adolescents. Deficiency of 3β-Δ5-C27-hydroxysteroid dehydrogenase (3β-HSDH) is the most common defect in this disease. Clinical features consist of neonatal hepatitis, hepatomegaly, splenomegaly, malabsorption, malnutrition, and late-onset liver disease. Case report: A male infant who presented jaundice in sclera at 4 months that resolved spontaneously, later presented cholestatic disease at 18 months. During his approach, normal gamma-glutamyl transpeptidase was documented, a finding that is highly suggestive of alteration in the synthesis of bile acids. The diagnosis was made using urine mass spectrometry. Oral colic acid treatment was started, presenting immediate improvement. Conclusions: The result in urinary bile acids is definitive for the genetic defect and consistent with homozygous mutations in the HSD3B7 gene. This condition is a diagnosis of exclusion in childhood cholestatic diseases, particularly in the presence of normal or mildly enlarged gamma-glutamyl transpeptidase, and responds adequately to oral treatment; it should be identified early.

Hepatitis autoinmune en la edad pediátrica / Autoimmune hepatitis in the pediatric age

Resumen: En pediatría, la hepatitis autoinmune y la colangitis esclerosante son patologías de afección hepática cuyo mecanismo de daño es inmunológico. La hepatitis autoinmune es una enfermedad de etiología desconocida, caracterizada por hepatitis de interfase, hipergammaglobulinemia, autoanticuerpos circulantes y una respuesta favorable a la inmunosupresión. Es una enfermedad eminentemente pediátrica, con una afección prevalente hacia mujeres jóvenes. La terapia debe ser instituida con prontitud para prevenir el deterioro rápido, promover la remisión de la enfermedad y la supervivencia a largo plazo. La falta persistente de respuesta o la falta de adherencia al tratamiento dan como resultado una enfermedad hepática terminal. Los pacientes que desarrollan esta patología, y aquellos con insuficiencia hepática fulminante en el momento del diagnóstico, requerirán trasplante hepático.

Abstract: In pediatrics, autoimmune hepatitis and sclerosing cholangitis are liver disorders with an immunological damage mechanism. Autoimmune hepatitis is a disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinemia, circulating autoantibodies and a favorable response to immunosuppression. It is an eminently pediatric disease with a prevalent condition in young women. Therapy should be instituted promptly to prevent rapid deterioration, promote remission of disease and long-term survival. The persistent lack of response or lack of adherence to treatment results in terminal liver failure; these patients, and those with fulminant hepatic insufficiency at the time of diagnosis, will require liver transplantation.

Acrodermatitis enteropática / Acrodermatitis enteropathica

Resumen: Introducción: La acrodermatitis enteropática es una enfermedad de baja incidencia que ocurre por deficiencia de zinc; puede ser hereditaria o adquirida. Se caracteriza por dermatitis acral, alopecia, diarrea y problemas de crecimiento. La afección dermatológica puede simular una infección micótica cutánea u otras enfermedades de la piel relacionadas con patógenos. Caso clínico: Se informa el caso de una paciente de sexo femenino de 7 meses de edad, que fue enviada al Centro Médico Nacional 20 de Noviembre por sospecha de inmunodeficiencia y micosis cutánea. Inició con un cuadro clínico caracterizado por dermatosis diseminada a cabeza, tronco y región genital, por lo que recibió tratamiento inicial con un antifúngico y antibióticos de amplio espectro, sin presentar mejoría. A su ingreso se descartaron inmunodeficiencia e infección micótica. Se sospechó acrodermatitis enteropática, corroborándose el diagnóstico al encontrar niveles séricos de zinc disminuidos. La paciente presentó mejoría inmediata tras el inicio del tratamiento con zinc por vía oral. Conclusiones: El diagnóstico diferencial de acrodermatitis enteropática es amplio e incluye infecciones cutáneas, por lo que el reconocimiento temprano de las características de las lesiones favorece la sospecha, el diagnóstico y el tratamiento adecuado.

Abstract: Introduction: Acrodermatitis enteropathica is a low-incidence disease due to inherited or acquired zinc deficiency. It is characterized by acral dermatitis, alopecia, diarrhea and growth retardation. The dermatological condition may mimic a cutaneous fungal infection or other pathogen-related skin diseases. Case report: We report the case of a female patient of 7 months of age, who was sent to Centro Médico Nacional 20 de Noviembre for suspicion of immunodeficiency and cutaneous mycosis. Her condition began with dermatosis disseminated to the head, trunk and genital region; initial treatment with antifungal and broad spectrum antibiotics was given, without improvement. Upon admission, immunodeficiency and fungal infection were discarded. Acrodermatitis enteropathica was suspected, and corroborated later by low serum zinc levels. Immediately after the start of oral treatment with zinc, the patient showed improvement. Conclusions: There are multiple differential diagnoses of acrodermatitis enteropathica, which includes cutaneous infections. Therefore, the early recognition of the characteristic lesions favors suspicion, diagnosis and appropriate treatment.

Esophageal motility disorders in children with dysphagia: the utility of the Chicago classification

BACKGROUND: esophageal manometry is the standard criterion for the evaluation of dysphagia and the diagnosis of a primary motor disorder of the esophagus in adults and children. AIMS: to describe the diagnosis according to the Chicago classification (CC) v3.0 in children with dysphagia, in whom an esophageal motility disorder was documented. The associated comorbidities were also determined. METHODS: an observational retrospective study was performed of 54 patients evaluated for dysphagia, who had undergone a high-resolution manometry (HREM). RESULTS: a normal HREM was found in 52 % (n = 28) of the children, whereas 48 % (n = 26) had some esophageal motility disorder. The most frequent diagnosis was ineffective esophageal motility and achalasia. Excluding previously healthy children, most children had a history of autoimmune disease and intellectual disability. CONCLUSIONS: an esophageal motor disorder can be diagnosed in nearly half of infants and children with dysphagia. In this study, all esophageal diseases could be classified according to the CC v3.0. HREM should be considered for the evaluation of children with dysphagia, in addition to other studies

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Enfermedad de Crohn en una niña con asociación VACTERL: ¿puede coexistir disfunción mitocondrial? / Crohn's disease in a girl with VACTERL association: Can mitochondrial dysfunction coexist?

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Small intestinal bacterial overgrowth: could it be associated with chronic abdominal pain in children with allergic diseases?

Background and aims: small intestinal bacterial overgrowth (SIBO) is a well-known cause of chronic abdominal pain (CAP) during the pediatric age. On the other hand, children with a history of some allergic disorder present CAP more frequently. The aim of this study was to determine the association between the presence of allergic diseases and SIBO in patients diagnosed with CAP. Materials and methods: this was an observational, analytical, retrospective study. Children with CAP who had undergone a lactulose hydrogen breath test to determine the presence of SIBO were included in the study. All patients underwent an evaluation for allergies by means of a skin prick test or the determination of specific IgE, according to clinical diagnosis. The study groups were established according to the presence of SIBO and the results of the allergic evaluation were statistically compared between the groups. Results: seventy patients were included (41 females and 29 males) and SIBO was diagnosed in 35 patients. In addition, 71.4% of children with SIBO were found to have an allergic disease, in contrast with 28.6% of children without SIBO (p = 0.001). The odds ratio for having any type of allergy in patients with SIBO was 5.45 (95% CI, 1.96-15.17; p = 0.001). Conclusions: we found an association between SIBO and allergic disease, especially allergic rhinitis, cow's milk protein allergy and asthma. Thus, SIBO should be ruled out in pediatric patients with CAP and allergic disease

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