Consenso colombiano de la enfermedad inflamatoria intestinal pediátrica

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Introduction: Pediatric ulcerative colitis (CUP), pediatric Crohn's disease (PCD), and pediatric inflammatory bowel disease not classifiable (PIDNCID) have clinical and psychosocial particularities that differentiate them from those of adults and may condition different therapeutic approaches due to possible nutritional, growth and developmental repercussions, representing a challenge for the pediatrician and gastroenterologist. Objective: Develop expert consensus evidence-based recommendations for the timely and safe diagnosis and treatment of Pediatric Inflammatory Bowel Disease (PID) in children under 18 years of age for professionals caring for these patients and healthcare payers. Methodology: Through a panel of experts from the Colombian College of Pediatric Gastroenterology, Hepatology and Nutrition (COLGAHNP) and a multidisciplinary group, 35 questions were asked regarding the clinical picture, diagnosis, and treatment of PID. Through a critical review and analysis of the literature with particular emphasis on the main clinical practice guidelines (CPGs), randomized clinical trials (RCTs), and meta-analyses of the last ten years, from which the experts made 77 recommendations that responded to each of the research questions with their respective practical points. Subsequently, each of the statements was voted on within the developer group, including the statements that achieved > 80%. Results: All statements scored > 80%. PID has greater extension, severity, and evolution towards stenosis, perianal disease, extraintestinal manifestations, and growth retardation compared to adult patients, so its management should be performed by multidisciplinary groups led by pediatric gastroenterologists and prepare them for a transition to adulthood. Porto's criteria allow a practical classification of PID. In CPE, we should use the Paris classification and perform ileocolonoscopy and esophagogastroduodenoscopy, since 50% have upper involvement, using the SES-CD (UCEIS/Mayo in CUP) and taking multiple biopsies. Initial labs should include inflammatory markers and fecal calprotectin and rule out intestinal infections. Treatment, induction, and maintenance of PID should be individualized and decided according to risk stratification. Follow-up should use PCDAI and PUCAI for the last 48 hours. Immunologists and geneticists should evaluate patients with early and infantile PID. Conclusion: A consensus guideline is provided with evidence-based recommendations on timely and safe diagnosis and treatments in patients with ILD.

Case Report: Duodenal Papillary Stenosis Secondary to Strongyloides stercoralis Infection in a Non-Immunocompromised Host.

Strongyloides stercoralis infection is a common infection in tropical and subtropical regions and is considered one of the "neglected tropical diseases." Owing to its life cycle, this infection can remain undetectable for years, preventing an early diagnosis and prompt treatment. We report the case of a 65-year-old woman who presented to us with a history of nausea, abdominal pain, bloating, and weight loss and, after initial radiologic and laboratory workup, was diagnosed with a periampullary mass with no secondary spread. She underwent an uneventful pylorus-preserving pancreatoduodenectomy, and on histopathological study of the lesion, a diagnosis of S. stercoralis infection was confirmed. This case stands out because of the importance of keeping S. stercoralis infections as a differential diagnosis of periampullary masses, particularly when the patient comes from regions where S. stercoralis is prevalent.

Pediatric patients with lysosomal acid lipase deficiency.

Lysosomal acid lipase (LAL) deficiency is a rare, autosomal recessive disease caused by mutations in the LIPA gene, which produces cholesteryl ester and triglyceride accumulation predominantly in hepatocytes, adrenal glands, and gastrointestinal tract. We describe two new cases occurring in siblings, aged 5 and 7 years, who presented with hepatomegaly, dyslipidemia, and abnormal liver function. Percutaneous liver biopsy revealed portal inflammation, hypertrophic Kupffer cells with a foamy appearance and microvesicular steatosis with fibrosis. Immunostaining for lysosomal markers, cathepsin D and LAMP1 reflected the lysosomal nature of the lipid vacuoles. After enzymatic confirmation, enzyme replacement therapy was initiated for both siblings. Follow-up transaminase levels and lipid profiles showed a notable decrease in AST and ALT and a slight increase in HDL cholesterol. It is crucial to increase awareness of this rare condition among clinicians and pathologists. The expression of lysosomal markers around the lipid vacuoles might help diagnose LAL deficiency in pediatric patients.

Lesión de la mucosa intestinal asociada a cristales: serie de casos y revisión de la literatura / Intestinal mucosal lesion associated with crystals: Case series and literature review

Resumen La lesión de la mucosa asociada a cristales es un cuadro clínico importante en un subconjunto de pacientes urémicos a los que se les administran resinas de intercambio catiónico como el sulfonato de poliestireno sódico (Kayexalate) y sevelamer para el tratamiento de la hiperpotasemia y la hiperfosfatemia, respectivamente. La necrosis colónica en estos pacientes es rara, pero puede estar asociada a una lesión gastrointestinal mortal, con una tasa de mortalidad del 33%. Los secuestrantes de ácidos biliares son otro tipo de resina que teóricamente son biológicamente inertes. Se presentan dos casos de colitis asociada a cristales. El primer paciente tenía antecedentes de múltiples intervenciones quirúrgicas y patologías del tracto gastrointestinal, y estaba en manejo con colestiramina. Se realizó una sigmoidectomía en la que se encontraron varios focos de cristales. El segundo paciente tenía antecedente de enfermedad renal crónica que requirió el uso de Kayexalate y se presentó al servicio de urgencias con una hemorragia grave del tracto gastrointestinal inferior. Se realizó una colectomía parcial en la que se detectaron cambios morfológicos relacionados con el depósito de cristales. Las resinas pueden producir un amplio espectro de lesiones mortales de la mucosa, por lo que el diagnóstico precoz es fundamental para disminuir la mortalidad y mejorar el pronóstico. Sin embargo, es incierto si el consumo de colestiramina y Kayexalate, así como el depósito de sus cristales en el tracto gastrointestinal son el factor causal de la lesión de las mucosas. Por tanto, la presencia de resinas debería ayudar a establecer el diagnóstico correcto y el tratamiento médico oportuno para evitar resultados nocivos.

Abstract Crystal-associated mucosal injury is a crucial clinical picture in a subset of uremic patients who are given cation exchange resins such as sodium polystyrene sulfonate (Kayexalate) and sevelamer to treat hyperkalemia and hyperphosphatemia, respectively. Colonic necrosis in these patients is rare but may be associated with fatal gastrointestinal injury, with a mortality rate of 33%. Bile acid sequestrants are another type of resin that is theoretically biologically inert. Two cases of colitis associated with crystals are presented. The first patient had a history of multiple surgeries and pathologies of the gastrointestinal tract and was treated with cholestyramine. A sigmoidectomy was performed in which several crystal foci were found. The second patient had a history of chronic kidney disease requiring Kayexalate and attended the emergency department with severe lower GI bleeding. A partial colectomy was performed in which morphological changes related to the deposit of crystals were detected. Resins can cause a broad spectrum of malignant mucosal lesions, so early diagnosis is essential to reduce mortality and improve prognosis. However, it is uncertain whether the consumption of cholestyramine and kayexalate, as well as the deposition of their crystals in the GI tract, are the causative factor of mucosal damage. Therefore, resins should help establish the correct diagnosis and prompt medical treatment to avoid harmful results.

Characteristics of Inflammatory Bowel Disease Compared to Other National Centers in Colombia / Características de la enfermedad inflamatoria intestinal con respecto a otros centros nacionales de Colombia

Abstract Introduction: Inflammatory bowel disease (IBD) is an immune-mediated disease whose incidence in Latin America has increased in recent years. Aim: To analyze the demographic and clinical characteristics of patients with IBD treated in a university hospital and present the epidemiological data compared to other centers in Colombia. Patients and methods: Descriptive study of patients with IBD (1996-2019) at the Hospital Universitario Fundación Santa Fe de Bogotá. Analysis of data from centers in Medellín, Cali, Bogotá, and Cartagena. Results: Of 386 patients, 277 presented with ulcerative colitis (UC), 102 with Crohn's disease (CD), and seven with unclassifiable colitis. IBD was more frequent in women (53 %). Mortality was less than 1 %. The involvement of UC was mainly pancolitis (42.6 %). The greater the extent of the disease, the higher the hospitalization and surgery rates (OR 3.70, P < 0.01). Thirteen percent of patients with UC received biologics. Compromise due to CD was mainly ileocolonic (43.6 %) and ileal (43.6 %). The predominant clinical pattern of CD was structuring (50%). Forty-five percent received biologicals and 56 % surgery. Primary sclerosing cholangitis (PSC) was found in 4 % of patients (n = 15). Two patients with PSC developed colorectal cancer (OR 4.18; p 0.008), while 13 patients with UC developed colon cancer and seven dysplastic changes. Three patients with CD developed colon cancer. Conclusions: The results were compared to other reference centers. We found similar trends in the clinical behavior and treatment of IBD, with higher hospitalization and surgery rates in our cases.

Resumen Introducción: la enfermedad inflamatoria intestinal (EII) es una enfermedad inmunomediada, cuya incidencia en Latinoamérica ha aumentado en los últimos años. Objetivo: analizar las características demográficas y clínicas de los pacientes con EII tratados en un hospital universitario y presentar los datos epidemiológicos con respecto a otros centros en Colombia. Pacientes y métodos: estudio descriptivo de pacientes con EII (1996-2019) en el Hospital Universitario Fundación Santa Fe de Bogotá. Análisis de datos de centros de Medellín, Cali, Bogotá y Cartagena. Resultados: de 386 pacientes, 277 presentaron colitis ulcerativa (CU), 102 enfermedad de Crohn (EC) y 7 colitis no clasificable. La EII fue más frecuente en mujeres (53 %). La mortalidad fue menor de 1 %. El compromiso de la CU fue principalmente la pancolitis (42,6 %). Entre mayor la extensión de la enfermedad, más alta fue la tasa de hospitalización y cirugías (OR 3,70; p < 0,01). El 13 % de los pacientes con CU recibió biológicos. El compromiso por la EC fue principalmente ileocolónico (43,6 %) e ileal (43,6 %). El patrón clínico predominante de la EC fue estenosante (50%). El 45 % recibió biológicos y 56% cirugía. La colangitis esclerosante primaria (CEP) se encontró en 4 % de los pacientes (n = 15). Dos pacientes con CEP desarrollaron cáncer colorrectal (OR 4,18; p 0,008), mientras que 13 pacientes con CU desarrollaron cáncer de colon y 7 cambios displásicos. 3 pacientes con EC desarrollaron cáncer de colon. Conclusiones: se compararon los resultados en relación con otros centros de referencia. Encontramos tendencias similares en el comportamiento clínico y en el tratamiento de la EII, con mayores tazas de hospitalizaciones y cirugías en nuestros casos.

Apoptotic colopathy as a manifestation of Good's syndrome.

The case was a 44-year-old patient with four years of evolution of respiratory infections, fever, weight loss of 30 kg and chronic diarrhea with inconclusive colonoscopy studies, managed as inflammatory bowel disease (IBD), with a history of thymomectomy four years previously. On physical examination, there was severe protein-calorie malnutrition, skin lesions compatible with herpes simplex infection and lower limb edema. Blood tests were requested when pancytopenia and hypoalbuminemia were negative for human immunodeficiency virus (HIV). Chest tomography showed a budding tree pattern and bronchiectasis, but SARS-CoV-2 was negative. A colonoscopy was performed, showing the presence of ulcers in the sigmoid colon with an infectious aspect vs IBD.

Post-transplant lymphoproliferative disorders in a cohort of adult patients with a liver transplant from a reference hospital in Bogotá, Colombia / Trastornos linfoproliferativos en una cohorte de pacientes adultos con trasplante hepático atendidos en un hospital de referencia en Bogotá, Colombia.

Introduction: The post-transplant lymphoproliferative disorders (PTLD) are characterized by an uncontrolled pathological lymphoid proliferation as a consequence of transplant immunosuppression therapy. Objective: To characterize the clinical and pathological characteristics of PTLD in a cohort of adult patients with liver transplant during a 15 year period at the Hospital Universitario Fundación Santa Fe de Bogota. Materials and methods: We conducted an observational retrospective study by searching for the PTLD cases diagnosed during the study period in the databases of the Liver Transplantation Unit and the Pathology Department. We collected the epidemiological, clinical, and pathological information and performed the corresponding statistics analyses. Results: During the research period, 572 patients were transplanted; the incidence of PTDL was 2.44%; 79% of them were man and the average age at the time of diagnosis was 62.5 years; 71% of the cases were diagnosed during the first year after the transplant and the same percentage EBV-seropositive patients. The most frequent pathological phenotype was monomorphic and the majority of tumors was detected in the hepatic hilum. The one-year survival was 50%. Conclusion: The high proportion of early cases and the high frequency of Epstein-Barr virus seropositivity both in donors and receptors drewour attention. More studies are necessary to have a better understanding of this condition in Colombia. This is the first PTLD clinical and pathological analysis in liver-transplant patients from Colombia to date.

Introducción. Los trastornos linfoproliferativos después de un trasplante se caracterizan por la proliferación descontrolada de linfocitos como consecuencia del tratamiento inmunosupresor posterior a este. Objetivo. Caracterizar clínica y patológicamente los casos de trastornos linfoproliferativos después de trasplante (Post-Transplant Lymphoproliferative Disorders, PTLD) en una cohorte de pacientes adultos con trasplante de hígado atendidos a lo largo de 15 años en el Hospital Universitario Fundación Santa Fe de Bogotá. Materiales y métodos. Se hizo un estudio observacional retrospectivo a partir de la revisión de las bases de datos de la Unidad de Trasplante Hepático y del Departamento de Patología del Hospital en busca de los casos de PTLD diagnosticados durante el periodo de estudio. Se recolectó la información epidemiológica, clínica y patológica, y se adelantaron los análisis estadísticos. Resultados. Durante el periodo de estudio, hubo 572 pacientes con trasplante de hígado, la incidencia de trastornos linfoproliferativos fue de 2,44 %, el 79 % en hombres, y la edad promedio en el momento del diagnóstico fue de 62,5 años. El 71 % de los casos se presentó durante los primeros 12 meses después del trasplante y el mismo porcentaje fue seropositivo para el virus de Epstein-Barr (EBV). El fenotipo patológico más frecuente fue el monomorfo y la mayoría de los tumores se detectaron en el hilio hepático. La supervivencia al año fue del 50 %. Conclusiones. Llamó la atención el alto porcentaje de casos de presentación temprana, así como la gran frecuencia de seropositividad para el EBV tanto en los donantes como en los receptores. Deben adelantarse estudios más detallados para una mejor comprensión de esta enfermedad en el país. Este es el primer análisis clínico y patológico de PTLD en pacientes con trasplante de hígado adelantado en Colombia hasta la fecha.

Trastornos linfoproliferativos en una cohorte de pacientes adultos con trasplante hepático atendidos en un hospital de referencia en Bogotá, Colombia / Post-transplant lymphoproliferative disorders in a cohort of adult patients with a liver transplant from a reference hospital in Bogotá, Colombia

Introducción. Los trastornos linfoproliferativos después de un trasplante se caracterizan por la proliferación descontrolada de linfocitos como consecuencia del tratamiento inmunosupresor posterior a este. Objetivo. Caracterizar clínica y patológicamente los casos de trastornos linfoproliferativos después de trasplante (Post-Transplant Lymphoproliferative Disorders, PTLD) en una cohorte de pacientes adultos con trasplante de hígado atendidos a lo largo de 15 años en el Hospital Universitario Fundación Santa Fe de Bogotá. Materiales y métodos. Se hizo un estudio observacional retrospectivo a partir de la revisión de las bases de datos de la Unidad de Trasplante Hepático y del Departamento de Patología del Hospital en busca de los casos de PTLD diagnosticados durante el periodo de estudio. Se recolectó la información epidemiológica, clínica y patológica, y se adelantaron los análisis estadísticos. Resultados. Durante el periodo de estudio, hubo 572 pacientes con trasplante de hígado, la incidencia de trastornos linfoproliferativos fue de 2,44 %, el 79 % en hombres, y la edad promedio en el momento del diagnóstico fue de 62,5 años. El 71 % de los casos se presentó durante los primeros 12 meses después del trasplante y el mismo porcentaje fue seropositivo para el virus de Epstein-Barr (EBV). El fenotipo patológico más frecuente fue el monomorfo y la mayoría de los tumores se detectaron en el hilio hepático. La supervivencia al año fue del 50 %. Conclusiones. Llamó la atención el alto porcentaje de casos de presentación temprana, así como la gran frecuencia de seropositividad para el EBV tanto en los donantes como en los receptores. Deben adelantarse estudios más detallados para una mejor comprensión de esta enfermedad en el país. Este es el primer análisis clínico y patológico de PTLD en pacientes con trasplante de hígado adelantado en Colombia hasta la fecha.

Introduction: The post-transplant lymphoproliferative disorders (PTLD) are characterized by an uncontrolled pathological lymphoid proliferation as a consequence of transplant immunosuppression therapy. Objective: To characterize the clinical and pathological characteristics of PTLD in a cohort of adult patients with liver transplant during a 15 year period at the Hospital Universitario Fundación Santa Fe de Bogota. Materials and methods: We conducted an observational retrospective study by searching for the PTLD cases diagnosed during the study period in the databases of the Liver Transplantation Unit and the Pathology Department. We collected the epidemiological, clinical, and pathological information and performed the corresponding statistics analyses. Results: During the research period, 572 patients were transplanted; the incidence of PTDL was 2.44%; 79% of them were man and the average age at the time of diagnosis was 62.5 years; 71% of the cases were diagnosed during the first year after the transplant and the same percentage EBV-seropositive patients. The most frequent pathological phenotype was monomorphic and the majority of tumors was detected in the hepatic hilum. The one-year survival was 50%. Conclusion: The high proportion of early cases and the high frequency of Epstein-Barr virus seropositivity both in donors and receptors drewour attention. More studies are necessary to have a better understanding of this condition in Colombia. This is the first PTLD clinical and pathological analysis in liver-transplant patients from Colombia to date.

Lipomatosis gástrica: reporte de un caso / Gastric lipomatosis: a case report

Resumen La lipomatosis gástrica es una enfermedad infrecuente caracterizada por múltiples lesiones tumorales benignas (lipomas), que acorde a su tamaño pueden producir una variedad de síntomas. Por lo general, la enfermedad es documentada incidentalmente en estudios imagenológicos que se realizan para estudiar otras enfermedades y los hallazgos patológicos contribuyen a tener una certeza en el diagnóstico de esta patología. Por el momento, no hay un tratamiento definido para las masas pequeñas y asintomáticas, mientras que para las masas grandes (> 3-4 cm) o sintomáticas se sugiere la resección quirúrgica de las lesiones.

Abstract Gastric lipomatosis is a rare disease characterized by multiple lipomas, benign tumors which can produce a variety of symptoms according to their size. In general, the disease is incidentally documented in imaging studies done to study other diseases. Pathological findings can contribute to the certainty of diagnosis. At the moment, there is no definite treatment for small, asymptomatic masses, but surgical resection is suggested for masses that are larger than 3 or 4 cm and for those that are symptomatic.

Merkel Cell Carcinoma and Diagnostic Experience in a Reference Hospital: A Case Series.

Merkel cell carcinoma (MCC) is a rare poorly differentiated neuroendocrine tumor, usually located in sun-exposed skin, with aggressive behavior and with high recurrence risk and metastatic disease. In Latin America, case series have been published, and it does not exceed 32 patients in 10 years, and in Colombia, there are case reports. We present a descriptive retrospective cross-sectional study in patients diagnosed with MCC in the Department of Pathology and Laboratories at the University Hospital Fundación Santa Fe de Bogotá(FSFB) between January 2003 and December 2018. We present the demographic, clinical, and pathological variables of these patients, as well as a literature review.

Caracterización clínica e histopatológica de niños con hepatitis autoinmune en un centro de referencia de Bogotá, Colombia / Clinical and histopathological characterization of children with autoimmune hepatitis at a referral center in Bogotá, Colombia

Resumen La hepatitis autoinmune (HAI) es una enfermedad hepática inflamatoria progresiva poco frecuente en niños y adolescentes, la cual es un reto diagnóstico para clínicos y patólogos. Describimos las características clínicas, bioquímicas e histopatológicas de 21 pacientes pediátricos con HAI diagnosticados en los últimos 14 años. Las biopsias hepáticas se reevaluaron para analizar detalladamente los hallazgos histopatológicos. De los 21 casos evaluados, 12 (57,1%) fueron mujeres, la mediana de edad fue 14 años, y 17 (80,9%) tenían HAI tipo 1. Los signos clínicos más frecuentes fueron ictericia (66,7%) y coluria (44,4%); también hubo evidencia de hipertensión portal con várices esofágicas (47,1%) y esplenomegalia (41,2%). El 11,8% de los pacientes tenía antecedentes de otras enfermedades autoinmunes. El 89,5%, 88,9% y 60,0% de los casos tenía elevación de aminotransferasas, hiperbilirrubinemia y bajos niveles de albúmina sérica, respectivamente. Las biopsias reevaluadas mostraron infiltrado linfoplasmocitario portal (94,4%), hepatitis de interfase (77,8%) y formación de rosetas (50,0%). En el 42,9% de las biopsias se hallaron inclusiones hialinas en las células de Kupffer. Cerca del 33,5% de los casos mostró cirrosis en la biopsia inicial. A pesar del tratamiento inmunosupresor, 4 pacientes requirieron trasplante hepático y 2 están en lista de espera. La HAI en niños puede manifestarse con ictericia y coluria, signos de hipertensión portal, aminotransferasas elevadas, hiperbilirrubinemia y anticuerpos circulantes. Las inclusiones hialinas en las células de Kupffer pueden ser un hallazgo útil en el diagnóstico histopatológico de la HAI en niños.

Abstract Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease. It is uncommon in children and adolescents, and is a diagnostic challenge for clinicians and pathologists. We describe the clinical, biochemical and histopathological characteristics of 21 pediatric patients with AIH diagnosed in the last 14 years. Liver biopsies were reassessed to analyze histopathological findings in detail. Of the 21 cases evaluated, 12 (57.1%) were girls and young women, the median age was 14 years old, and 17 (80.9%) had type 1 AIH. The most frequent clinical signs were jaundice (66.7%), choluria (44.4%), evidence of portal hypertension with esophageal varices (47.1%), and splenomegaly (41.2%). Histories of other autoimmune diseases were found in 11.8% of these patients. Elevated levels of aminotransferases were found in 89.5% of the patients, hyperbilirubinemia was found in 88.9%, and 60.0% of the cases had low levels of serum albumin. Reassessed biopsies showed portal lymphoplasmocytic infiltrate (94.4%), interface hepatitis (77.8%) and rosette formation (50.0%). Hyaline inclusions were found in Kupffer cells in 42.9% of the biopsies. About 33.5% of the cases showed cirrhosis at the initial biopsy. Despite immunosuppressive treatment, four patients required liver transplantation and two are on the waiting list. AIH in children can manifest with jaundice, choluria, signs of portal hypertension, elevated aminotransferases, hyperbilirubinemia and circulating antibodies. Hyaline inclusions in Kupffer cells may be a useful finding in the histopathological diagnosis of AIH in children.

Unusual Course of an Aggressive Pituitary Prolactinoma: Case Report and Review of the Literature.

Pituitary carcinomas are rare tumors with heterogeneous behaviors. Their carcinogenesis is still unknown. Consequently, treatment is multimodal and not standardized. Dopamine (DA) agonists are used as first-line treatments, while radiotherapy and surgery may be used for local control of invasive tumors. We describe the case of a 35-year-old male who presented with an invasive prolactinoma, managed initially with a transsphenoidal resection, postsurgical radiotherapy and DA agonists. The patient posteriorly presented a sole metastatic lesion to the lumbar spine that was later managed with local radiotherapy. Due to pituitary recurrence of the lesion, multiple surgical resections were needed until further treatment was declined. The clinical course in this patient was unusual. He lived for 13 years after initial diagnosis, with a very invasive tumor without systemic chemotherapy. Radiotherapy is used in pituitary tumors in which surgery fails; we hypothesize that it contributed to the malignant transformation and the late resistance to DA agonists in our patient. Several biomarkers in tumoral tissue have been evaluated regarding their association with malignancy and aggressive behaviors, although more studies are still needed. Therapeutic strategies are limited, without evidence on the impact on overall survival and prognosis. Risk factors associated with early malignancy in pituitary prolactinomas include recurrent behavior, increase in prolactin levels with a stable sellar mass, and secondary development of DA agonist resistance. However, there are still no conclusive answers as to whether physicians should rigorously follow up these patients or provide direct therapy with aggressive approaches.

Caracterización clínico-patológica de la esofagitis eosinofílica en niños y adolescentes en el Hospital Universitario Fundación Santa Fe de Bogotá / Clinical Pathology Characterization of Eosinophilic Esophagitis in Children and Adolescents at Hospital Universitario Fundación Santa Fe de Bogotá

Resumen Introducción: la esofagitis eosinofílica (EEo) es una enfermedad emergente, crónica e inmunomediada. Clínicamente se caracteriza por síntomas asociados con disfunción esofágica e histológicamente por infiltrado inflamatorio predominante de eosinófilos. Objetivo: describir las características clínicas, endoscópicas e histopatológicas de niños y adolescentes con diagnóstico de EEo en el Hospital Universitario Fundación Santa Fe de Bogotá entre 2007 y 2017. Métodos: estudio observacional descriptivo de corte transversal que incluyó pacientes menores de 18 años con diagnóstico histopatológico de EEo. Resultados: se incluyeron 46 pacientes, 31 de sexo masculino, con una edad promedio de 11,8 años (rango: 11 meses-18 años). El 70 % presentó dolor abdominal, el 37 % pirosis, 28 % emesis y el 22 % náuseas y disfagia. Los antecedentes más referidos fueron asma (41 %), rinitis alérgica (37 %), enfermedad por reflujo gastroesofágico (22 %) y dermatitis atópica (15 %). El hallazgo endoscópico más frecuente fue los exudados blanquecinos, descritos en el 35 %. La sospecha endoscópica de EEo se describió en el 50 % de los casos. El estudio histopatológico mostró de 15 a 40 eosinófilos por campo de alto poder (CAP) en el 52 %, 41-60 en el 19,5 % y 61-80 en el 15,2 %. Se encontró hiperplasia de la lámina basal en el 95,6 % de los casos. Conclusiones: los pacientes en su mayoría eran adolescentes (69 %), el síntoma más frecuente fue el dolor abdominal (70 %) y el 40 % de los casos tenía antecedente de atopia. Solamente el 50 % tenía hallazgos endoscópicos sugestivos de EEo. El presente trabajo corresponde al primer análisis clínico y patológico de casos de EEo en niños y adolescentes en Colombia.

Abstract Introduction: Eosinophilic esophagitis (EoE) is an emerging, chronic and immune-mediated disease. Clinically it is characterized by symptoms associated with esophageal dysfunction, and histologically by predominantly inflammatory eosinophil infiltrate. Objective: The aim of this study was to describe the clinical, endoscopic and histopathological characteristics of children and adolescents diagnosed with EoE at the Hospital Universitario Fundación Santa Fe de Bogotá between 2007 and 2017. Methods: This is a cross-sectional, descriptive and observational study that included patients under 18 years of age with histopathological diagnoses of EoE. Results: Forty-six patients were included, 31 were male, and the average age was 11.8 years (range 11 months - 18 years). Seventy percent presented abdominal pain, 37% presented heartburn, 28% suffered vomiting, 22% had nausea and dysphagia. The most common antecedents were asthma (41%), allergic rhinitis (37%), gastroesophageal reflux disease (22%) and atopic dermatitis (15%). The most frequent endoscopic finding consisted of whitish exudates found in 35% of the cases. Endoscopic suspicion of EoE was described in 50% of the cases. The histopathological study showed 15 to 40 eosinophils per high power field (HPF) in 52%, 41-60/HPF in 19.5%, and 61-80/HPF in 15.2%. Hyperplasia of the basal lamina was found in 95.6% of the cases. Conclusions: The majority of patients were adolescents (69%), the most frequent symptom was abdominal pain (70%), and 40% of cases had histories of atopy. Only 50% had endoscopic findings suggestive of EoE. This study is the first clinical and pathological analysis of EoE cases in children and adolescents in Colombia.

New Regenerative Vascular Grafts for Hemodialysis Access: Evaluation of a Preclinical Animal Model.

The purpose of this study was to evaluate a suitable animal model for the in vivo evaluation of patency and vascular tissue regeneration in small intestinal submucosa (SIS) vascular grafts for hemodialysis access. First, a 4-mm U-shaped SIS vascular graft was implanted between the internal carotid artery (CA) and the external jugular vein (JV) in five sheep and six swine. The U-shape grafts remained functional for 53 ± 4 days in sheep and 32 ± 2 days in swine. The sheep model presented exaggerated inflammation, so the swine model was selected for the in vivo study. Based on these initial results, a 4-mm C-shape SIS vascular graft with SIS circumferential reinforcement was developed to mechanically improve the vascular graft and manage complications identified during surgery in both sheep and swine. The C-shape vascular graft was implanted in a swine model (n = 3) between the CA and JV. GORE-TEX® vascular grafts were used as controls in the contralateral side of the neck. C-shape grafts remained patent for 47 ± 4 days, whereas the GORE-TEX® grafts were patent for 30 ± 15 days. The C-shape vascular graft was easier to handle during surgery, and its circumferential reinforcement improved in vivo patency, avoiding kinks in the graft after implantation. Histological results showed neovascularization and some regeneration with the alignment of endothelial cells in the vascular wall of the grafts. The model developed may be helpful in other research involving in vivo studies of vascular grafts for hemodialysis access.

The spectrum of glomerular disease between the years 2003 and 2015 in Columbia: A review of 12,613 cases.

BACKGROUND: The prevalence of glomerular disease (GD) varies according to the different socio-demographic characteristics of each population. For the first time we present the prevalence of the different forms of GD among patients from several different areas of Columbia. METHODS: Data from 12,613 renal biopsies studied at our University Hospital between 2003 and 2015 was reviewed. Pathology results were classified according to a list of renal diseases proposed by various authors. RESULTS: Focal segmental glomerulosclerosis was present in 22%, IgA disease in 21%, Lupus nephritis in 17%, membranous glomerulonephritis in 13% and thin basal membrane disease in 9%. CONCLUSIONS: This study confirms that focal segmental glomerulosclerosis and IgA disease are the most prevalent GDs found in Columbian patients. This is the first study to analyze GDs in a Columbian population and we recommend that a national registry system be created to collect comprehensive information from future research.

Guía colombiana para el diagnóstico de la deficiencia de lipasa ácida / Colombian Guidelines for Diagnosis of Lipase Acid Deficiency

Resumen Introducción: la deficiencia de lipasa ácida lisosomal (LAL-D) es una entidad de herencia autosómica recesiva que lleva a la acumulación de esteres de colesterol y triglicéridos en el hígado, bazo y otros órganos. La edad de inicio y la tasa de progresión son muy variables, lo que posiblemente sea explicado por las mutaciones presentes en el gen LIPA. Las manifestaciones clínicas son las mismas que para otras patologías hepáticas, cardiovasculares y metabólicas, lo que hace difícil reconocerla en la práctica clínica. Objetivo: proveer una guía que permita a los clínicos reconocer los principales grupos de riesgo en los cuales se debe sospechar de LAL-D y mejorar su diagnóstico. Metodología: este documento se diseñó como un consenso de expertos en el cual participaron médicos especialistas en gastroenterología, hepatología, endocrinología, genética, patología y pediatría. Se realizó una revisión de la literatura acerca de las manifestaciones clínicas y de las herramientas para el diagnóstico de LAL-D y se siguió la metodología de técnica de grupo nominal. Resultados: se generaron algoritmos diagnósticos por consenso para cada uno de los grupos de riesgo, que facilitaran la sospecha y el diagnóstico de LAL-D. Conclusiones: esta guía propone algoritmos para el diagnóstico de LAL-D con base en el consenso clínico, que buscan optimizar la ruta diagnóstica en los pacientes con dicha patología.

Abstract Introduction: Lysosomal acid lipase deficiency (LAL-D) is an inherited autosomal recessive entity that leads to the accumulation of cholesterol and triglyceride esters in the liver, spleen and other organs. The age of onset and rate of progression vary greatly, possibly explained by mutations of the LIPA gene. Clinical manifestations are the same as those of other hepatic, cardiovascular and metabolic pathologies which makes it difficult to recognize in clinical practice. Objective: The objectives of these guidelines is to help clinicians recognize the major groups at risk for LAL-D and to improve its diagnosis. Methodology: This document was designed as a consensus of experts in gastroenterology, hepatology, endocrinology, genetics, pathology and pediatrics. A review of the literature regarding clinical manifestations and tools for diagnosis of LAL-D was conducted and the nominal group technique was followed. Results: Diagnostic algorithms which facilitate suspicion and diagnosis of LAL-D were generated by consensus for each of the risk groups. Conclusions: This guide proposes algorithms for the diagnosis of LAL-D based on clinical consensus. The algorithms seek to optimize diagnosis for patients with this pathology.

Algoritmos útiles para el diagnóstico histopatológico de la enfermedad hepática con base en los patrones de daño hepático / Useful Algorithms for Histopathological Diagnosis of Liver Disease Based on Patterns of Liver Damage

En los anteriores artículos, se revisaron las patologías hepáticas más frecuentes desde el punto de vista morfológico y la importancia de una adecuada correlación, para lograr un mejor entendimiento entre clínicos y patólogos. El ejercicio que se hará a continuación, se basa en establecer una aproximación al diagnóstico histopatológico de algunas de las patologías hepáticas a las que se les realiza biopsias con mayor frecuencia, teniendo en cuenta algoritmos basados en patrones de daño hepático

Previous articles have reviewed the most frequent liver pathologies from the morphological point of view and looked at the importance of adequate correlation for obtaining better understanding between clinicians and pathologists. The next exercise is directed toward histopathological diagnosis of some of the liver diseases for which biopsies are performed most frequently. It takes into account algorithms based on patterns of liver damage

Patología del trasplante hepático, aspectos más relevantes del período postrasplante tardío / Most Relevant Pathology Issues in the Late Post Liver Transplant Period

La sobrevida de los pacientes postrasplante hepático supera el 90% al año y el 75% a los 5 años. Entender las causas de pérdida del injerto, o inclusive la muerte del paciente, es esencial para mejorar aún más los resultados a largo plazo. La evaluación de las biopsias hepáticas tiene un papel importante en la explicación y manejo de la disfunción del injerto de hígado, que ocurre después del primer año del trasplante. La interpretación de estas biopsias puede ser muy difícil, en especial por la alta incidencia de enfermedades recurrentes que pueden mostrar un cuadro clínico y unas características histopatológicas que semejan varias condiciones, especialmente cuando el rechazo agudo o crónico pueden sobreponerse a una patología ya existente o presentarse de manera simultánea y contribuir a la disfunción tardía del injerto, por lo que el análisis de la biopsia puede ayudar a determinar el componente principal de la lesión. Es indispensable la correlación clínico patológica, teniendo en cuenta la enfermedad original, el tipo de inmunosupresión, las pruebas de función hepática, las serologías virales, los autoanticuerpos y los hallazgos radiológicos. En este artículo comentaré las patologías más frecuentes y las que causan más problemas en su diagnóstico durante el período postrasplante tardío

One year survival rates of liver transplant patients exceed 90% while five year survival rates exceed 75%. Understanding the causes of graft losses and patient deaths is essential for further improvement of long-term results. Evaluation of liver biopsies has an important role in explaining liver graft dysfunction that occurs more than one year after transplantation, and thus is key for post-transplant patient management. The interpretation of these biopsies can be very difficult especially because of the high incidence of recurrent diseases that sometimes have clinical and histopathological features that resemble various other conditions. This is especially true for acute and chronic rejection which can overwhelm an existing condition and which can develop simultaneously with other conditions that contribute to late graft dysfunction. Analysis of the biopsy can help determine the main component of a lesion. Clinical findings must be correlated to pathological findings, and the correlation must take into account the original disease, the type of immunosuppression, liver function tests, viral serology, autoantibodies and radiological findings. In this article I will discuss the most common diseases and those that cause the most problems for diagnosis during the late post-transplant period

Biopsia hepática en la patología del trasplante, período postrasplante temprano, enfoque dirigido al diagnóstico histopatológico y su correlación clínico-patológica: Histopathological Diagnosis and Clinicopathological Correlation in the Early Post-Transplant Period / Liver Biopsies in Transplant Pathology

La biopsia hepática de los aloinjertos sigue siendo considerada el estándar de oro y juega un papel importante e integral en la interpretación y explicación de los cambios que puedan ocurrir en respuesta a alteraciones en las pruebas de la función o bioquímica hepática, anomalías funcionales o alteración en las imágenes diagnósticas, las cuales pueden, o no, ir acompañadas de síntomas. También es útil en el seguimiento o biopsias por protocolo (1-3). La evaluación de biopsias, después del trasplante, puede ser difícil debido a que es muy amplio el espectro de las complicaciones que pueden presentarse en el período postrasplante; más aún, cuando muchas de ellas necesitan un diagnóstico y tratamiento inmediato. La patología más frecuente es el rechazo agudo. Sin embargo, también pueden observarse cambios de perfusión/reperfusión, alteraciones funcionales, recidiva de enfermedad de base, lesión de la vía biliar, lesiones vasculares, infecciones oportunistas, patologías de novo, como la hepatitis autoinmune, hepatitis crónica idiopática postrasplante, toxicidad farmacológica o tumores, entre otras patologías (4). En este artículo relacionado con la patología del trasplante hepático se tratarán las patologías más frecuentes, no quirúrgicas, en el período postrasplante temprano, con un enfoque histopatológico dirigido a las dificultades y controversias para una adecuada correlación clínico-patológica.

Biopsies of liver allografts are still considered to be the gold standard. They play an important and integral role in the interpretation and explanation of changes that may occur in response to alterations in function tests, in the interpretation and explanation of liver biochemistry, in the interpretation and explanation of functional abnormalities, and in the interpretation and explanation of diagnostic images (whether or not accompanied by symptoms). Biopsies are also useful for monitoring and are often part of the protocol (1-3). The evaluation of biopsy samples after transplantation can be difficult especially because of the very broad spectrum of complications that may arise in the post-transplant period. Many of them require immediate diagnosis and treatment despite this difficulty. Although the most common condition is acute rejection, many other conditions and disorders can be observed. They include perfusion/reperfusion alterations, functional impairment, recurrence of underlying diseases, injury to the bile duct, vascular lesions, opportunistic infections, de novo pathologies such as autoimmune hepatitis, post-transplant idiopathic chronic hepatitis, drug toxicity, and tumors (4). This is the second article about the pathology of liver transplantation. It discusses the most common pathologies in the early post-transplant period and provides a histopathological approach towards difficulties and controversies for adequate clinicopathological correlation.