Utilización de escalas no invasivas en la detección de varices esofágicas en pacientes con trombosis venosa portal / Use of non-invasive scales for detecting esophageal varices in paediatric patients with portal vein thrombosis

Introducción La trombosis portal (TVP) es la causa más frecuente de hipertensión portal en población pediátrica. El Consenso de Baveno VI considera la ligadura endoscópica de varices como segunda opción terapéutica tras el meso-Rex-bypass (shunt quirúrgico). Objetivo Analizar la rentabilidad diagnóstica de escalas no invasivas para predecir el riesgo de varices esofágicas en niños con TVP. Material y métodos Estudio descriptivo retrospectivo donde se incluyeron endoscopias digestivas altas (EDA) en pacientes<15 años con TVP no cirróticos. Se dividieron según la presencia de varices esofágicas y se estudiaron sexo, etiología, edad, hemorragia digestiva o tratamientos previos, resultados de EDA y las escalas (Regla Predicción Clínica-CPR, Regla Predicción Varices-VPR, King's Variceal Prediction Score-K-VaPS y ratio plaquetas/bazo-RPB). Las variables cualitativas se expresaron mediante frecuencia absoluta y porcentaje, y las cuantitativas mediante mediana y rango intercuartílico. Para las comparaciones se emplearon los test U de Mann-Whitney y Hanley-McNeil. Resultados Se realizaron 45 EDA. Un 80%(n=36) presentaron varices esofágicas: mediana de 3(2 – 3) y un 33,3%(n=12) precisó ligadura endoscópica de varices. Se demostraron diferencias estadísticamente significativas entre ambos grupos: CPR (142,39 [132,22 - 166,53] vs. 122,75 [115,24 – 133,15] p=0,003), VPR (9,91 [9,36 – 11,75] vs. 5,6 [3,34 – 8,39] p=0,001), K-VaPS (117,86 [99,66 - 126,58] vs. 99,64 [94,88 - 110,18] p=0,019), RPB (2384,62 [1902,22 - 3201,63] vs. 1252,5 [579,6 - 2144,42] p=0,05), con un área bajo la curva>75%, sin demostrarse diferencias entre escalas. Conclusiones En pacientes pediátricos con TVP no cirróticos se pueden emplear escalas no invasivas como herramienta para predecir la presencia de VE y plantear con ello la indicación de EDA. (AU)

Introduction Portal vein thrombosis (PVT) is the most frequent cause of portal hypertension in paediatric population. Baveno VI Consensus considers endoscopic variceal ligation as the second therapeutic option after meso-Rex bypass (surgical shunt). Aim Analyse the diagnostic profitability of non-invasive scales in order to predict the risk of oesophageal varices (OV) in children with PVT. Material and methods Descriptive retrospective study where every upper gastrointestinal endoscopy (UGE) carried on patients <15 years old with non-cirrhotic PVT were included. There were divided according to the presence of OV and sex, cause, age, previous gastrointestinal bleeding or treatments, results of UGE and scales (Clinical Prediction Rule – CPR), Varices Prediction Rule – VPR), King's Variceal Prediction Score – K-VaPS) and Platelet count/Spleen diameter Ratio – PSR). Qualitative variables were expressed as absolute frequency and percentage, and quantitative variables as median and interquartile range. U Mann–Whitney and Hanley–McNeil tests were used for comparisons. Results Forty-five UGE were analysed. 80% (n=36) presented OV: median of 3 (2–3) and 33.3% (n=12) required endoscopic variceal ligation. Statistical differences were demonstrated between both groups: CPR (142.39 [132.22-166.53] vs. 122.75 [115.24-133.15]; p=0.003), VPR (9.91 [9.36-11.75] vs. 5.6 [3.34-8.39]; p=0.001), K-VaPS (117.86 [99.66-126.58] vs. 99.64 [94.88-10.18]; p=0.019), PSR (2384.62 [1902.22-3201.63] vs. 1252.5 [579.6-2144.42]; p=0.05), with and area under the curve AUROC>75%, without statistical differences between scales. ConclusionsIn paediatric patients with non-cirrotic PVT non-invasive scales can be used as a tool to predict the presence of OV and raise the indication of UGE. (AU)

Use of non-invasive scales for detecting esophageal varices in paediatric patients with portal vein thrombosis. / Utilización de escalas no invasivas en la detección de varices esofágicas en pacientes con trombosis venosa portal.

INTRODUCTION: Portal vein thrombosis (PVT) is the most frequent cause of portal hypertension in paediatric population. Baveno VI Consensus considers endoscopic variceal ligation as the second therapeutic option after meso-Rex bypass (surgical shunt). AIM: Analyse the diagnostic profitability of non-invasive scales in order to predict the risk of oesophageal varices (OV) in children with PVT. MATERIAL AND METHODS: Descriptive retrospective study where every upper gastrointestinal endoscopy (UGE) carried on patients <15 years old with non-cirrhotic PVT were included. There were divided according to the presence of OV and sex, cause, age, previous gastrointestinal bleeding or treatments, results of UGE and scales (Clinical Prediction Rule - CPR), Varices Prediction Rule - VPR), King's Variceal Prediction Score - K-VaPS) and Platelet count/Spleen diameter Ratio - PSR). Qualitative variables were expressed as absolute frequency and percentage, and quantitative variables as median and interquartile range. U Mann-Whitney and Hanley-McNeil tests were used for comparisons. RESULTS: Forty-five UGE were analysed. 80% (n=36) presented OV: median of 3 (2-3) and 33.3% (n=12) required endoscopic variceal ligation. Statistical differences were demonstrated between both groups: CPR (142.39 [132.22-166.53] vs. 122.75 [115.24-133.15]; p=0.003), VPR (9.91 [9.36-11.75] vs. 5.6 [3.34-8.39]; p=0.001), K-VaPS (117.86 [99.66-126.58] vs. 99.64 [94.88-10.18]; p=0.019), PSR (2384.62 [1902.22-3201.63] vs. 1252.5 [579.6-2144.42]; p=0.05), with and area under the curve AUROC>75%, without statistical differences between scales. CONCLUSIONS: In paediatric patients with non-cirrotic PVT non-invasive scales can be used as a tool to predict the presence of OV and raise the indication of UGE.

Dermatofibrosarcoma protuberans en pacientes pediátricos / Dermatofribosarcoma protuberans in pediatric patients

Antecedentes y Objetivos. Presentamos 3 casos de dermatofibrosarcoma protuberans (DFSP) en pacientes pediátricos para documentar la presentación clínica poco frecuente de esta patología. Pacientes y Método. Realizamos un análisis descriptivo retrospectivo de los 3 últimos casos de DFSP en nuestro centro durante los años 2010 - 2013. Resultados. Una niña de 12 años remitida desde otra institución por DFSP en parte distal del muslo, en la que ampliamos bordes quirúrgicos de resección en 3 cm y practicamos cobertura con dermis artificial e injerto autólogo; tras 1 año de observación sin recidiva, realizamos reconstrucción del defecto mediante injertos autólogos de grasa. Otra niña de 12 años diagnosticada de Síndrome de Hamartomatosis Múltiple asociado al gen PTEN, que presentó DFSP en labio mayor derecho y en mama derecha, recidivante en esta última localización. Por último, un DFSP congénito en una paciente remitida a los 3 meses de vida con tumoración gigante de pared abdominal. El diagnóstico orientativo inicial con resonancia magnética con gadolino, solo se confirmó finalmente por los hallazgos histopatológicos de la pieza de resección; ni la punción aspiración ni la biopsia cutánea lograron confirmar el diagnóstico. Conclusiones. El DFSP es un tipo de sarcoma raro de la piel del niño y del adulto, localmente agresivo y con una alta tasa de recidiva. La escisión local amplia puede provocar mutilaciones y desfigurar al paciente; por ello la cirugía micrográfica de Mohs permite reducir los márgenes quirúrgicos (AU)

Background and Objectives. We present 3 cases of dermato fibro sarcoma protuberans (DFSP) to document the unusual clinical presentation of this condition in pediatric patients. Patients and Methods. We conduct a retrospective descriptive analysis of the last 3 cases of DFSP treated in our institution during years 2010-2013. Results. A 12-year-old girl referred from another institution for DFSP in distal thigh; we practised surgical margins extended 3 cm resection and cover with artificial dermis and autologous graft; after 1 year follow up without recurrence, reconstruction of the defect was performed using autologous fat grafts. Another 12-year-old girl diagnosed of Multiple Hamartoma Syndrome associated with PTEN gene, who presented DFSP at the right labia major and at the right breast, being recurrent in this last location. Finally, a congenital DFSP in a patient referred at 3 months with giant tumour of abdominal wall. The initial diagnosis was made by MRI with gadolinium. The final diagnosis of DFSP was made based on histopathological findings of the piece following surgical resection, because percutaneous biopsy or skin biopsy couldn´t confirm the diagnostic. Conclusions. DFSP is a rare type of sarcoma of the skin in children and adults, locally aggressive, with a high rate of recurrence. Wide local excision may cause maiming and disfiguring to the patient, thereby Mohs micrographic surgery (CMM) enables to reduce surgical margins (AU)

Clinical outcome of hepatocyte transplantation in four pediatric patients with inherited metabolic diseases.

Hepatocyte transplantation (HT) has become an effective therapy for patients with metabolic inborn errors. We report the clinical outcome of four children with metabolic inborn errors that underwent HT, describing the cell infusion protocol and the metabolic outcome of transplanted patients. Cryopreserved hepatocytes were used as this allows scheduling of treatments. Functional competence (viability, cell attachment, major cytochrome P450 and UDP-glucuronosyltransferase 1A1 activities, and urea synthesis) and microbiological safety of cell batches were assessed prior to clinical use. Four pediatric patients with liver metabolic diseases [ornithine transcarbamylase (OTC) deficiency, Crigler-Najjar (CNI) syndrome, glycogen storage disease Ia (GSD-Ia), and tyrosinemia type I (TYR-I)] underwent HT. Indication for HT was based on severity of disease, deterioration of quality of life, and benefits for the patients, with the ultimate goal to improve their clinical status whenever liver transplantation (LT) was not indicated or to bridge LT. Cells were infused into the portal vein while monitoring portal flow. The protocol included antibiotic prophylaxis and immunosuppressant therapy. After HT, analytical data on the disease were obtained. The OTC-deficient patient showed a sustained decrease in plasma ammonia levels and increased urea production after HT. Further cell infusions could not be administered given a fatal nosocomial fungus sepsis 2 weeks after the last HT. The CNI and GSD-Ia patients improved their clinical status after HT. They displayed reduced serum bilirubin levels (by ca. 50%) and absence of hypoglycaemic episodes, respectively. In both cases, the HT contributed to stabilize their clinical status as LT was not indicated. In the infant with TYR-I, HT stabilized temporarily the biochemical parameters, resulting in the amelioration of his clinical status while diagnosis of the disease was unequivocally confirmed by full gene sequencing. In this patient, HT served as a bridge therapy to LT.

Donantes de ayer y de hoy: ¿han cambiado las características de los donantes de hígado durante los últimos 15 años? / Donors yesterday and today: have the characteristics of liver donors changed over the last 15 years?

Introducción. Los cambios sociosanitarios y demográficos de nuestra población y el crecimiento de las listas de pacientes en espera de un trasplante han condicionado ciertas variaciones en los criterios de selección de los donantes de órganos. Material y método. Estudio retrospectivo, descriptivo y comparativo de las características de los donantes cadáver de hígado utilizados en la Unidad de Trasplante Hepático del Hospital Universitario La Fe de Valencia en 2 períodos diferentes. Distribuimos los casos en 2 grupos: grupo A, los primeros 200 donantes (entre enero de 1991 y junio de 1995) y grupo B, 200 donantes más recientes (desde febrero de 2004 hasta diciembre de 2005). Resultados. El número de donaciones se ha incrementado desde los 18 donantes durante 1991 a los 106 en 2006. En el grupo A la edad media fue de 32,4 años, frente a 52,3 años del grupo B. En la serie A, la principal causa de muerte fueron los traumatismos craneoencefálicos y en el grupo B, los accidentes cerebrovasculares. El tiempo en la unidad de cuidados intensivos fue mayor en el segundo grupo, con una media de 67,2 h. En el grupo B el 17% presentaba aterosclerosis considerable de la aorta y el 29,5%, esteatosis hepática macroscópica, frente al 5 y el 12%, respectivamente, en el grupo A. Conclusiones. Actualmente, los donantes de hígado son de mayor edad, presentan más enfermedades crónicas, mueren por enfermedades cerebrovasculares, permanecen más tiempo en cuidados intensivos y sus hígados son macroscópicamente peores que los de donantes de años anteriores (AU)

Introduction. The social, medical and demographic changes of our population and the increase in the number of patients on waiting lists have led to some changes in the selection criteria of organ donors. Material and method. A retrospective, descriptive and comparative study of the liver cadaveric donors features accepted in the Liver Transplant Unit in La Fe University Hospital of Valencia (Spain) in 2 different periods. We distributed the cases into 2 groups, including in group A the first 200 first donors (from January 1991 to June 1995) and in group B the last 200 donors (from February 2004 to December 2005). Results. The number of donors increased from 18 during 1991 to 106 in 2006. In group A the mean age was 32.4 years, compared to 52.3 years in group B. In group A, the main cause of death was craneoencephalic traumatism and in group B cerebrovascular accidents. The mean time in the intensive care unit was longer in the second group with 67.2 hours. In group B, considerable atherosclerosis was reported in 17% of cadaveric donors and macroscopic liver steatosis in 29.5%, compared to 5 and 12%, respectively, in group A. Conclusions. Nowadays, cadaveric liver donors are older, suffer more chronic diseases, die due to cerbrovascular diseases, remain longer in intensive care units and the livers are macroscopically worse compared to donors accepted 15 years ago (AU)

[Donors yesterday and today: have the characteristics of liver donors changed over the last 15 years?]. / Donantes de ayer y de hoy: han cambiado las características de los donantes de hígado durante los últimos 15 años?

INTRODUCTION: The social, medical and demographic changes of our population and the increase in the number of patients on waiting lists have led to some changes in the selection criteria of organ donors. MATERIAL AND METHOD: A retrospective, descriptive and comparative study of the liver cadaveric donors features accepted in the Liver Transplant Unit in La Fe University Hospital of Valencia (Spain) in 2 different periods. We distributed the cases into 2 groups, including in group A the first 200 first donors (from January 1991 to June 1995) and in group B the last 200 donors (from February 2004 to December 2005). RESULTS: The number of donors increased from 18 during 1991 to 106 in 2006. In group A the mean age was 32.4 years, compared to 52.3 years in group B. In group A, the main cause of death was craneoencephalic traumatism and in group B cerebrovascular accidents. The mean time in the intensive care unit was longer in the second group with 67.2 hours. In group B, considerable atherosclerosis was reported in 17% of cadaveric donors and macroscopic liver steatosis in 29.5%, compared to 5 and 12%, respectively, in group A. CONCLUSIONS: Nowadays, cadaveric liver donors are older, suffer more chronic diseases, die due to cerbrovascular diseases, remain longer in intensive care units and the livers are macroscopically worse compared to donors accepted 15 years ago.