Page kidney after pediatric kidney transplantation: a case report. / Riñón de Page tras trasplante renal pediátrico: informe de un caso.

INTRODUCTION: Page kidney (PK) is a rare condition caused by parenchymal compression due to a subcapsular hematoma. Irreversible damage of the graft may occur if this condition is not recognized and treated properly. CLINICAL CASE: We describe the case of a 16-year-old man with chronic renal failure secondary to corticosteroid-resistant nephrotic syndrome (CRNS) caused by NPHS2 mutations. The patient underwent a 5th fifth living-related KT. The graft was placed intraperitoneally and reperfused well without complications. On the 4th postoperative day his labs demonstrated raising creatinine associated with refractory hypertension, gross hematuria and anemia. Urgent ultrasound revealed a subcapsular hematoma with signs of parenchymal compression. PK phenomenon was suspected and urgent surgical intervention decided. COMMENTS: PK is a rare but an emergence potentially treatable and reversible complication after pediatric KT. Early diagnosis based on clinical suspicion and suggestive imaging are the key points for a favorable outcome.

INTRODUCCION: El riñón de Page (RP) es una enfermedad rara provocada por compresión parenquimatosa debido a un hematoma subcapsular. El injerto puede sufrir daños irreversibles si la enfermedad no se reconoce y se trata de forma adecuada. CASO CLINICO: Describimos el caso de un varón de 16 años con fallo renal crónico secundario a síndrome nefrótico corticorresistente (SNCR) provocado por mutaciones del gen NPHS2. El paciente se somete a un quinto TR de donante vivo. El injerto se coloca en posición intraperitoneal, con una adecuada reperfusión, sin complicaciones. Al cuarto día postoperatorio, sus análisis revelan una subida de la creatinina asociada a hipertensión refractaria, hematuria macroscópica y anemia. La ecografía de urgencia revela hematoma subcapsular con signos de compresión parenquimatosa. Se sospecha RP y se decide intervención quirúrgica de urgencia. COMENTARIOS: El RP es una complicación rara y urgente, aunque a su vez tratable y reversible, tras TR pediátrico. El diagnóstico precoz basado en la sospecha clínica y las imágenes son claves a la hora de conseguir un resultado favorable.

Treatment of balanitis xerotica obliterans in pediatric patients. / Tratamiento de la balanitis xerótica obliterante en pacientes pediátricos.

OBJECTIVES: Balanitis xerotica obliterans (BXO) is a chronic inflammatory disease with a little known incidence in pediatric population. The objective of this work was to describe our experience in the treatment of BXO. MATERIALS AND METHODS: Retrospective study carried out in 419 patients undergoing circumcision surgery between January 2014 and January 2017. Demographic, clinical, therapeutic, and anatomical and pathological variables, as well as complications during follow-up, were analyzed. RESULTS: Of the 419 patients, 41 (9.78%) were diagnosed with BXO. 6 patients were excluded owing to lack of follow-up, so 35 patients were analyzed. Mean age at diagnosis was 8.6 years. Suspicion diagnosis was clinical at physical exploration in 17 patients (48.6%), and at surgery in 18 patients (51.4%). Anatomical and pathological confirmation was performed in a total 35 patients (100%). During follow-up, 6 patients (17.14%) had lesions in the glans, 3 (8.57%) in the urethra, and 9 (25.71%) in both. 6 meatotomies (17.14%) and 5 new circumcisions (14.28%) had to be carried out. Mean recurrence time was 32.43 months. In 19 patients (54.28%), topical corticoids - ointment - were applied, and 1 patient (2.85%) received topical immunosuppressants. CONCLUSIONS: A close follow-up of patients with clinical or anatomical and pathological diagnosis of BXO is required given its high morbidity. The complications described in pediatric population include meatal and urethral stenosis, as well as recurrent phimosis, unless a sufficient amount of foreskin is resected.

OBJETIVOS: La balanitis xerótica obliterante (BXO) es una enfermedad crónica inflamatoria de incidencia poco conocida en la población pediátrica. El objetivo de este trabajo es describir nuestra experiencia en el tratamiento de las BXO. MATERIAL Y METODOS: Estudio retrospectivo de 419 pacientes intervenidos de circuncisión en el periodo comprendido entre enero de 2014 y enero de 2017. Analizamos variables demográficas, clínicas, anatomopatológicas, terapéuticas y complicaciones durante el seguimiento. RESULTADOS: De los 419 pacientes, 41 fueron diagnosticados de BXO (9,78%). Seis pacientes fueron excluidos por falta de seguimiento, por lo que se analizaron 35 pacientes. La media de edad al diagnóstico fue de 8,6 años. El diagnóstico de sospecha fue clínico durante la exploración física en 17 pacientes (48,6%) y durante la intervención en 18 (51,4%), realizando la confirmación anatomopatológica en un total de 35 pacientes (100%). Durante el seguimiento 6 pacientes (17,14%) presentaron lesiones en glande, 3 (8,57%) en uretra y 9 (25,71%) en ambas localizaciones, siendo necesaria la realización de 6 meatotomías (17,14%) y de nueva circuncisión en 5 (14,28%). El tiempo medio de recidiva fue de 32,43 meses. En 19 pacientes (54,28%) se aplicaron corticoides tópicos en pomada y en 1 paciente inmunosupresores tópicos (2,85%). CONCLUSIONES: Es necesario un seguimiento estrecho de los pacientes con diagnóstico clínico o anatomopatológico de BXO dada su elevada morbilidad. Las principales complicaciones descritas en la población pediátrica son la estenosis meatal y uretral, así como la recidiva de la fimosis si no se reseca el prepucio suficientemente.

Outcome of kidney transplantation from young pediatric donors (aged less than 6 years) to young size-matched recipients.

INTRODUCTION: Pediatric donation is underutilized because of presumed increased risk of vascular thrombosis (VT) and graft loss. Using young pediatric donors (YPDs) for young pediatric recipients (YPRs) is suggested to be even at greater risk and therefore precluded in many centers. The aim of this study was to analyze the outcome of kidney transplantation (KT) from YPD to age-matched YPR. PATIENT AND METHODS: A retrospective study of 118 pediatric KT performed between January 2007-July 2017. The authors identified KT with YPD (considered as those aged <6 years) and age-matched YPR. Organ allocation was performed based on the best paired size (YPR for YPR). Data were collected regarding donor and recipient characteristics, surgical and urological complications, graft loss, and outcomes. RESULTS: Forty cases of YPD to age-matched YPR were identified (33.89% of the cohort). Mean recipient and donor age were 2.9 years (SD 1.68) and 2.24 years (SD 1.5), respectively. Mean recipient and donor weight were 12.7 kg (SD 4.1) and 13.7 kg (SD 4.15), respectively. Thirty of those young recipients (75%) weighed <15 kg. The most frequent primary renal disease was the congenital nephrotic syndrome. Nine out of 40 patients (22.5%) had received a previous KT before. Three received a combined liver-KT. Eight (20%) were classified as high immunological risk and 19 (47.5%) as high thrombotic risk. All allografts were implanted extraperitoneally and anastomosed to the iliac vessels. Major complications requiring reintervention occurred in seven patients (17.5%): three VT, three bleeding episodes, and one ureteral necrosis. Remarkably, only one surgical complication (VT) resulted in graft loss. Regarding long-term urological complications, four patients (10%) all with obstructive uropathy-developed vesicoureteral reflux to the graft. Actuarial graft survival at 1,5, and 10 years in the YPD to age-matched YPR cohort was 83% -78% -78%, respectively. Mean follow-up was 3.6 years (SD 3.2) (r = 7-10). Over time, eight patients lost their graft, not related to surgical factors in seven out of eight cases. CONCLUSION: The authors suggest that KT using YPD for age-match YPR yields good results in expert centers, even in high-risk patients and is associated with good graft survival. In this series, surgical complications were rarely related to graft loss.

[Is the Mathieu urethroplasty a useful technique for urethrocutaneous fistula repair after hypospadias surgery?] / ¿Es la uretroplastia Mathieu una técnica útil para la reparación de fístulas uretrocutáneas en pacientes operados de hipospadias?

AIM: There are multiple surgical techniques for distal urethrocutaneous fistulas (DUCF) repair after hypospadias surgery. Our aim was to analyze our results of simple closure (SC) and salvage mathieu urethroplasty (MU). MATERIAL AND METHODS: Retrospective study (2014-2017) of pediatric patients with DUCF who underwent SC and MU. Only subcoronal to distal fistulas were collected, excluding the proximal ones non-tributary of MU. Demographics, clinical data, treatment and results were registered. MAIN RESULTS: Over 4 years, 177 hypospadias and 28 UCFs were repaired. 15/28 met the inclusion criteria. Hypospadias type: anterior (10, 66.7%), midshaft (3, 20%) and posterior (2, 13.3%). Mean age at primary urethroplasty was 31.9 months (SD: 16.2). The most common techniques in primary surgery were Thiersch-Duplay (6) and Snodgrass (4). Median time from primary surgery to apparition of the fistula was 5 months (rank: 0-14). The surgical technique used for fistula repair was: SC (7) and MU (8). DUCF succeeded in all patients treated with MU, while among those treated with SC, 71.4% recurred, and only 4 patients underwent finally successful repair with a median of 1.5 interventions. Median follow-up was 25 months (rank: 12-55). CONCLUSIONS: MU is a useful, effective and safe technique for DUCF repair. Most importantly, it is easily reproducible. The results of MU seem to be superior to CS and could be considered as a first choice.

OBJETIVO: Existen múltiples técnicas quirúrgicas para el cierre de fístulas uretrocutáneas distales (FUCD) secundarias a la corrección de hipospadias. Nuestro objetivo fue analizar nuestros resultados mediante el cierre simple (CS) y cierre con nueva uretroplastia por técnica de Mathieu (CUM). MATERIALES Y METODOS: Estudio retrospectivo (2014-2017) de pacientes pediátricos con FUCD tratadas mediante CS y CUM. Se incluyeron solo fístulas desde la región subcoronal a distal, excluyendo las fístulas proximales no candidatas de cierre por CUM. Se recogieron datos demográficos, clínicos, tratamiento y resultados. RESULTADOS: Durante 4 años se intervinieron 177 hipospadias y 28 FUC. 15/28 cumplieron los criterios de inclusión. Tipo hipospadias: distal (10; 66,7%), mediopeneano (3; 20%) y proximal (2; 13,3%). La media de edad de la cirugía primaria fue de 31,9 meses (DS:16,2). Las técnicas primarias más utilizadas fueron el Thiersch-Duplay (6) y el Snodgrass (4). La mediana de tiempo desde la cirugía primaria a la aparición de la fístula fue de 5 meses (rango: 0-14). La técnica utilizada para el cierre fue: CS (7) y CUM (8). Todos los pacientes tratados con CUM resolvieron la fístula en un solo tiempo; de los tratados con CS, recidivaron el 71,4%, resolviéndose finalmente 4 con una mediana de 1,5 intervenciones. La mediana de seguimiento fue de 25 meses (rango: 12-55). CONCLUSIONES: El CUM es una técnica útil, eficaz y segura para el tratamiento de la FUCD y, además, fácilmente reproducible. Los resultados del cierre por CUM parecen ser superiores al CS y podría considerarse la técnica de primera elección.

Is donor age 6 years or less related to increased risk of surgical complications in pediatric kidney transplantation?

INTRODUCTION: Despite the widespread organ shortage dilemma, there is hesitancy regarding utilization of young donors (aged ≤6 years) because previous reports have suggested that this is associated with an increased risk of surgical complications and graft loss. OBJECTIVE: The aim of this study was to determine if donor age ≤6 years is related to increased risk of surgical complications or allograft loss in pediatric kidney transplantation (KT). STUDY DESIGN: A retrospective study of pediatric kidney transplants (KT) undertaken between January 2000 and July 2015. The incidence of surgical and urological complications, and allograft loss were analyzed and compared between donors aged ≤6 years (Group 1) and donors aged >6 years (Group 2). RESULTS: A total of 171 pediatric KTs were performed at the current center during the study period. Twenty-eight patients were excluded; as a result, the study comprised 143 patients: 60 (Group 1) and 83 (Group 2). Mean recipient weight was 17 kg (SD 9.7; range 3.2-47) in Group 1 and 38.2 kg (SD 15.3; range 7.8-73) in Group 2. Despite a significantly higher proportion of risk factors in Group 1, no significant between-group differences were observed in terms of: surgical complications (OR 0.4; range 0.1-1.2), early urological complications (OR 2.2; range 0.4-11), late urological complications (OR 0.3; range 0.8-1.4), lymphoceles (OR 6.2; range 0.7-51.7) and allograft loss (OR 1.5; range 0.7-3.1, summary Table). Graft survival at 1 and 5 years was: 81% and 70% (Group 1) and 92% and 79% (Group 2), respectively (P = 0.093). Mean follow-up was 90.13 ± 49.7 months. DISCUSSION: The main finding of this retrospective study was that pediatric donor kidneys from donors aged ≤6 years could safely be used in pediatric recipients without an increased risk of surgical and urological complications or graft loss. Nevertheless, KT with small donor kidneys is challenging and should be performed at experienced pediatric centers. CONCLUSION: In line with these results, the outcomes of KT using donors aged ≤6 years were encouraging and similar to those obtained with older donors. Thus, this study supported using kidney grafts from young donors, given the organ shortage and potential high mortality risk while awaiting KT.

[Conservative management of liver trauma and its complications: current gold-standard]. / Tratamiento conservador de los traumatismos hepáticos y sus complicaciones: gold-standard actual.

INTRODUCTION: The liver is the second most frequently involved organ in abdominal trauma in children. The aim of this study was to review the characteristics of liver traumas (LT) in children, its treatment and complications. MATERIAL AND METHODS: Retrospective study of patients with LT treated between 2010-2014. We analyzed data regarding diagnosis, conservative management (CM), complications and treatment. RESULTS: Twenty-four patients of LT with a mean age of 9.8 years (SD: 3.4) were treated at our center. Liver injury degrees according to the Organ Injury Scale of American Association for Surgery of Trauma (AAST) were: grade I (4), grade II (6), grade III (8), grade IV (5) and grade V (1). CT angiography was performed in 23 patients. Four of the six patients with initial hemodynamic instability required blood transfusion; of those, two required angioembolization for active bleeding in extrahepatic locations and a third required damage control surgery. Three patients presented late-onset complications: 2 large size bilomas which underwent spontaneous resolution and a third patient with a right biloma and section of the left bile duct who required a multidisciplinary approach. Median hospital stay was 6 days (r 1-92). With a mean follow-up of 17.75 months (SD: 16.35) (r: 2-57) long-term survival was 96%. The patient who received surgery died due to a post-traumatic brain injury. CONCLUSION: CM seems to be the treatment of choice in LT regardless of injury degree. Surgery should be reserved for cases refractory to CM due to the associated high mortality.

INTRODUCCION: El hígado es el segundo órgano más afectado en traumatismos abdominales en edad pediátrica. El objetivo de este trabajo es revisar las características de los traumatismos hepáticos (TH), su tratamiento y complicaciones. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con TH atendidos entre 2010 -2014. Analizamos datos referentes al diagnóstico, tratamiento conservador (TC), complicaciones y tratamiento de las mismas. RESULTADOS: Se trataron 24 casos de TH con una edad media de 9,8 años (DS: 3,4). Los grados de lesiones hepáticas según la Organ Injury Scale of American Association for Surgery of Trauma (AAST) fueron: grado I (4), grado II (6), grado III (8), grado IV (5) y grado V (1). Se realizó angio-TC en 23 pacientes. Cuatro de los 6 pacientes que presentaron inestabilidad hemodinámica inicial requirieron transfusión de hemoderivados; de éstos, 2 requirieron angioembolización por sangrado activo extrahepático y un tercero se intervino de forma urgente. Tres pacientes presentaron complicaciones tardías: 2 bilomas de gran tamaño que se resolvieron espontáneamente y un biloma derecho con sección de vía biliar izquierda que requirió un abordaje multidisciplinar. En los 3 casos se trataba de traumatismos de alto grado (2 grado IV, 1 grado V). La mediana de estancia hospitalaria fue de 6 días (r: 1-92). Con una media de seguimiento de 17,75 meses (DS: 16,35) (r: 2-57) la supervivencia global fue del 96%. El paciente que requirió cirugía falleció por el traumatismo craneoencefálico asociado. CONCLUSIONES: El TC parece ser de primera elección en los TH independientemente del grado aunque no está exento de complicaciones. La cirugía debe reservarse para los casos refractarios al TC dada la alta mortalidad que conlleva.

[Vascular interventional radiology: a fundamental procedure for the management of paediatric trauma]. / Radiología vascular intervencionista: un arma para el tratamiento del niño politraumatizado. Experiencia en un centro de Trauma Pediátrico.

INTRODUCTION: The management of active bleeding with haemodinamic lability in the paediatric trauma patient is difficult and generally leads to damage control surgery. Vascular Interventional Radiology (VIR) techniques are useful for the diagnosis as for the definitive treatment. AIM: The aim of our study was to describe our experience and evaluate effectiveness of VIR in the management of the paediatric trauma patient with active bleeding signs. METHODS: Retrospective analysis (2003-2014) of politraumatic patients who showed contrast blush on computed tomography and then treated by VIR techniques. RESULTS: In the reported study period 16 patients underwent VIR procedures. Medium age was 13 years (5-17). The most frequent lesion mechanism was traffic accident (8 out of 17) and 93,75% were blunt traumas. Findings on initial Computed Tomography were 12 contrast blushes and 2 absences of arterial flow. In 2 cases the contrast blush appeared 48 hours after the accident. Arteriography allowed us to localize the bleeding vessels in all the cases, performing selective or supraselective renal (7), pelvic (5), hepatic (3), splenic (1) and intercostal (1) embolization. One patient required an endoprothesis for renal revascularization. Two cases needed additional surgical procedures (2 nephrectomies) because of complete section of the renal artery (1) and disruption of the ureteropelvic junction (1). One case required hemofiltration in relation to rhabdomyolysis. CONCLUSION: In our experience VIR is a valuable diagnostic and therapeutic procedure for the management of paediatric trauma patients, with high effectiveness and a low complication rate.

INTRODUCCION: El tratamiento del sangrado activo en niños politraumatizados con labilidad hemodinámica es difícil y generalmente obliga a realizar una cirugía de control de daños. La aplicación de técnicas de Radiología Vascular Intervencionista (RVI) ayuda al diagnóstico y tratamiento definitivo. OBJETIVO: Describir nuestra experiencia y valorar la eficacia de la RVI en el tratamiento del paciente traumático pediátrico con signos de sangrado activo. MATERIAL Y METODO: Análisis retrospectivo (2003-2014) de los pacientes politraumatizados tratados mediante RVI en los cuales la AngioTC mostraba fuga de contraste o ausencia de captación. RESULTADOS: En el periodo de estudio se trataron 16 pacientes, con una media de edad de 13 años (5-17). El mecanismo lesional más frecuente fue el accidente de tráfico. El 93,75% fueron traumatismos cerrados. Los hallazgos de la angioTC inicial fueron 12 sangrados activos, 2 ausencias de flujo arterial. Dos sangrados se produjeron a las 48 horas del trauma. La arteriografía permitió localizar los vasos sangrantes, realizándose embolización selectiva o supraselectiva a nivel renal (7), pélvico (5), hepático (3), esplénico (1) e intercostal (1). Un paciente preciso revascularización renal con endoprótesis. Un paciente requirió nefrectomía urgente tras la angiografía por avulsión arterial completa y en 1 caso se realizó nefrectomía a las 48 horas por fuga de contraste y disrupción de la unión pieloureteral. En un caso se observó rabdomiólisis como complicación que requirió hemofiltración. CONCLUSION: La RVI constituye un procedimiento diagnóstico y terapéutico en el abordaje del paciente pediátrico traumático, con alta efectividad y escasas complicaciones, siendo una herramienta fundamental en un centro de trauma pediátrico.

[Rupture of a superior mesenteric artery aneurysm in pediatric age: case report and literature review]. / Rotura de un aneurisma de arteria mesentérica superior en edad pediátrica: caso clínico y revisión de la literatura.

INTRODUCTION: Splanchnic artery aneurysms are rare in children. High mortality from rupture justifies its treatment, with various therapeutic options among which stand out surgery and recently, endovascular treatment. CASE REPORT: A 11 year old girl presented with abdominal pain and sudden drop in hematocrit. The urgent abdominal CT angiography showed a saccular aneurysm of the superior mesenteric artery (SMA) at 4 cm from the ostium with dissection and active bleeding. A selective angiography was performed which confirmed the dissection. A self-expanding stent was placed in the main trunk of the SMA and a transcatheter coil and onyx embolization of the aneurysm was performed. The control angiogram showed no evidence of residual perfusion of the false lumen and demonstrated proper vascularization of the distal jejunum-ileal branches. Dual antiplatelet therapy with aspirin and dipyridamole was begun. After 24 months of follow-up the patient is asymptomatic. COMMENTS: Endovascular treatment of a SMA aneurysm is effective in the pediatric patient, even in emergency situations.

INTRODUCCION: Los aneurismas esplácnicos son excepcionales en la edad pediátrica. La elevada mortalidad por rotura justifica su tratamiento, existiendo diversas opciones terapéuticas entre las que destacan la cirugía y, recientemente, el tratamiento endovascular. CASO CLINICO: Paciente de 11 años que presentó dolor abdominal súbito y caída del hematocrito. La angio-TC abdominal urgente mostró un aneurisma sacular de la arteria mesentérica superior (AMS) a 4 cm del ostium con disección de la luz y signos de sangrado activo. Se realizó una angiografía que confirmó el aneurisma. Se colocó un stent autoexpandible en el tronco principal de la arteria mesentérica superior con repleción del aneurisma con microcoils y Onyx, sin evidenciar perfusión residual de la falsa luz y comprobando una adecuada vascularización tanto distal como de las ramas yeyuno-ileales. Se instauró doble antiagregación con AAS y dipiridamol. Tras 24 meses de seguimiento se encuentra asintomática.. COMENTARIOS: El tratamiento endovascular es efectivo en el paciente pediátrico, incluso en situaciones de emergencia.

[Congenital portosistemic shunt. Importance of early treatment]. / Shunt portosistémico congénito. Importancia del tratamiento precoz.

AIM: Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition. PATIENTS AND METHODS: Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007. The mean age at diagnosis was 5.5 years (1 month-15 years). Five patients had associated comorbidities. RESULTS: Five patients had developed hyperammonemia and intellectual impairment, one of those manifested with an onset of coma. Four patients have developed at diagnosis liver tumors, including focal nodular hyperplasia/regenerative nodules (n=3) and adenomas (n=3). One patient with multiple tumors required a hepatectomy owing to compressive symptoms. Two patients, developed malignant degeneration, a child under five years treated with liver transplantation and another in adulthood treated with hepatectomy. In one patient, diagnosed in the neonatal period, the shunt occlusion occurred spontaneously. Direct portography with the occlusion test was performed in five patients, the shunt was occluded with interventional radiology in three cases, surgery in one and liver transplantation in the remaining. CONCLUSIONS: The treatment of the SPSC must be early to prevent and even to reverse its complications, avoiding liver transplantation. Currently, interventional radiology is essential in the strategy to follow and treatment of these patients.

OBJETIVOS: El shunt porto-sistémico congénito (SPSC) es una patología infrecuente que puede producir complicaciones graves, como encefalopatía y tumores hepáticos con riesgo de degeneración maligna. La oclusión del shunt por cirugía o radiología intervencionista puede evitar, e incluso mejorar, las complicaciones. En algunos casos el trasplante hepático es la única opción. Describimos nuestra experiencia con esta patología. PACIENTES Y METODO: Entre 1992 y 2013, ocho pacientes en edad pediátrica (cuatro varones y cuatro mujeres) fueron diagnosticados de un SPSC (cuatro extrahepáticos y cuatro intrahepáticos), de los cuales siete fueron diagnosticados después del año 2007. La mediana de edad al diagnóstico fue 5,5 años (1 mes-15 años). Cinco pacientes tenían patología asociada. RESULTADOS: Cinco pacientes presentaban hiperamoniemia y afectación intelectual. Una niña debutó con coma. Cuatro pacientes presentaron tumoraciones hepáticas, incluyendo hiperplasia nodular focal/nódulos de regeneración (n=3), y adenomas (n=3). Una paciente con tumoraciones múltiples requirió una hepatectomía por síntomas compresivos. En dos pacientes se produjo degeneración a hepatocarcinoma, un niño de 5 años tratado con trasplante y otro en edad adulta tratado con hepatectomía. En un paciente de diagnóstico neonatal, el shunt cerró espontáneamente en seis meses. En cinco pacientes se ha realizado portografía directa con test de oclusión, realizándose cierre del shunt en tres casos por radiología intervencionista, uno con cirugía y en otro, con trasplante. CONCLUSIONES: El tratamiento del SPSC ha de ser precoz para prevenir, e incluso revertir, las complicaciones, evitando el trasplante hepático. En la actualidad la radiología intervencionista juega un papel fundamental en la estrategia y el tratamiento de estos pacientes.

[Laryngotracheal traumatic injuries (LTI) in children: when to operate?] / Lesiones laringotraqueales traumáticas en niños ¿cuándo operar?

INTRODUCTION: Paediatric LTI is associated with significant morbid-mortality. Although historically first line treatment was surgical, conservative management is making headway. The purpose of this study was to analyze the management and surgical indications of LTI treated at our institution. MATERIAL AND METHODS: Retrospective study of patients with LTI treated between 2007 and 2014. RESULTS: Six out of seven patients, with a median age of 4 years (2-15), had blunt traumas and one had an open trauma. Respiratory distress, pain and subcutaneous emphysema were presented in 71.4% of the patients. Five children were intubated (3 pre-hospital care and 2 after reaching the hospital), all of them underwent fibrobronchoscopy, determining the location, the extention, and assessing the possibility of extubation. Esophagoscopy was made in 2 cases. The remaining 2 cases were bounded to observation as they did not have respiratory symptoms or progressive emphysema, precluding bronchoscopy. Conservative management was followed in 71.4% of the patients, with a 100% success rate. Conservative management criteria were no progression of emphysema or respiratory distress, regardless the degree and location of the lesions. Two patients needed surgery: one tracheoesophageal fistula caused by a button battery, and the other was a tracheal open section associated with vascular injury. Both of them recovered uneventfully. CONCLUSIONS: If the respiratory symptoms or the emphysema do not progress, patients with LTI can be managed conservatively, regardless of size or location of the injury. Associated esophageal lesions and open injuries require surgical management. An early diagnosis is mandatory in order to minimize morbidity and mortality.

INTRODUCCION: Las lesiones laringotraqueales traumáticas pediátricas están asociadas a una importante morb-imortalidad. Aunque históricamente el tratamiento ha sido quirúrgico, en la actualidad el enfoque conservador ha adquirido una mayor importancia. Valoramos el tratamiento y las indicaciones quirúrgicas de los traumatismos laringotraqueales tratados en nuestro centro. MATERIAL Y METODOS: Análisis retrospectivo de pacientes con traumatismos laringotraqueales del 2007 al 2014. RESULTADOS: Siete pacientes, mediana de edad 4 años (r: 2-15), 6 traumatismos cerrados y 1 abierto. El 71,4% presentaban dificultad respiratoria, dolor y enfisema subcutáneo. Cinco fueron intubados (3 en prehospitalaria y 2 en hospital). En todos ellos se realizó fibrobroncoscopia determinando la localización, la extensión de la lesión y la posibilidad de extubación. En dos de ellos se asoció esofagoscopia. Dos casos no presentaban clínica respiratoria y el enfisema no progresó por lo que se tomó una actitud expectante y no precisaron broncoscopia. El 71,4% se trataron conservadoramente, siendo el criterio de tratamiento conservador la no progresión del enfisema ni empeoramiento del estado respiratorio, independientemente del grado y localización de la lesión, con una tasa de éxito del 100%. Dos pacientes se intervinieron: una fístula tráqueo-esofágica producida por una quemadura (pila de botón) que requirió resección traqueal; y una sección traqueal abierta asociada a lesión vascular, ambas con correcta evolución posterior. CONCLUSIONES: En caso de no progresión de la clínica respiratoria o del enfisema, e independientemente del grado y localización, las lesiones laringotraqueales pueden tratarse de manera conservadora con o sin intubación. Lesiones esofágicas asociadas y lesiones abiertas obligan a un abordaje quirúrgico. Un diagnóstico precoz minimiza la morbimortalidad.

[Salivaly gland tumors in childhood: importance of an early diagnosis and treatment]. / Tumores de glándulas salivales en la infancia. Importancia de un diagnóstico y tratamiento precoz.

OBJECTIVE: Review of our series of salivaly gland tumors at pediatric age, given its low frequency and their higher rate of malignancy compared with the adult population. MATERIAL AND METHODS: Retrospective study of the medical records of all pediatric patients with salivaly gland tumors treated in our center between 1998 and 2013. RESULTS: We identified 12 patients. The most common site of tumor location was the parotid gland in 11 patients followed by the sublingual gland in 1 patient. Three patients had previous cancer and radiotherapy history. The diagnosis (histological or radiological) were: 4 hemangiomas, 2 pleomorphic adenomas, 4 mucoepidermoid carcinomas, one acinar cell carcinoma and 1 primitive neuroectodermal tumor (PNET). No patient had metastasis at diagnosis. Preoperative fine needle aspiration (FNA) was performed in 6 patients with solid masses, being diagnostic in all cases. Eight patients underwent surgery. In two cases of total parotidectomy initial neuroapraxia appeared but none had permanent facial paralysis. There were no cases of Frey syndrome. The aesthetic results were good. There was only one tumor recurrence and all patients, except one who died of leukemia relapse, survive today. CONCLUSIONS: Salivaly gland tumors are uncommon in pediatric age but their high rate of malignancy makes it necessary to establish a rapid diagnosis. In our experience, FNA showed a high sensitivity. For malignant tumors, definitive treatment is surgical excision, preserving the facial nerve whenever possible. For vascular tumors, surgery is reserved for cases refractory to medical treatment.

OBJETIVO: Revisión de nuestra serie de neoplasias de glándulas salivales en pediatría, dada su baja frecuencia y mayor tasa de malignidad en comparación con la población adulta. MATERIAL Y METODOS: Estudio retrospectivo de las historias clínicas de los casos pediátricos de tumores de glándulas salivales tratados en nuestro centro entre los años 1998 y 2013. RESULTADOS: Se estudian 12 pacientes. Los tumores afectaban a la glándula parótida en 11 casos y en uno a la glándula sublingual. Tres pacientes presentaban historia oncológica previa y antecedentes de radioterapia. El diagnóstico (histológico o radiológico) fue de: 4 hemangiomas, 2 adenomas pleomorfos, 4 carcinomas mucoepidermoides, 1 carcinoma de células acinares y 1 tumor primitivo neuroectodérmico (PNET). Ningún paciente presentaba metástasis al diagnóstico. Se realizó PAAF preoperatoria en 6 pacientes que presentaban masas sólidas, siendo diagnóstica en todos ellos. Ocho pacientes fueron intervenidos. En dos casos de parotidectomía total apareció neuroapraxia inicial pero ninguno tuvo parálisis facial definitiva. No hubo ningún caso de síndrome de Frey. Los resultados estéticos fueron buenos. Un solo caso presentó recidiva tumoral y todos los pacientes, excepto uno que falleció por recaída de leucemia, sobreviven en la actualidad. CONCLUSIONES: Los tumores de glándula salival son raros en pediatría pero su alta tasa de malignidad hace necesario establecer un diagnóstico rápido. En nuestra experiencia, la PAAF presentó elevada sensibilidad. En los tumores malignos el tratamiento es la exéresis, preservando el nervio facial siempre que sea posible. En los tumores vasculares la cirugía se reserva para casos refractarios al tratamiento médico.

[Exceptional causes of hematuria in childhood and infancy]. / Causas excepcionales de hematuria en la infancia y en la adolescencia.

INTRODUCTION: Hematuria is not uncommon in pediatric patients, even though it remains a diagnostic challenge in many cases. In this study we aim to draw attention to health professionals to have in mind rare diseases as a cause of hematuria. CASE REPORTS: We report 3 representative clinical cases of hematuria from very different origins: traumatic, congenital and infectious, illustrating the broad spectrum of causes of hematuria. COMMENTS: There are exceptional causes of hematuria which should be considered in clinical practice. The study of hematuria should always include a complete personal and familiar medical history, a careful physical examination and urine cultures. It is important to try to propose an algorithm study, avoiding unnecessary and invasive diagnostic tests that can be associated with complications.

INTRODUCCION: La hematuria es un signo frecuente en pediatría, siendo un reto diagnóstico en muchos casos. El objetivo de este trabajo es llamar la atención a los profesionales de la salud sobre la necesidad de tener presentes enfermedades infrecuentes como causa de hematuria. CASOS CLINICOS: Exponemos 3 tres casos clínicos representativos de formas de hematuria de orígenes muy distintos: traumático, congénito e infeccioso, que ilustran el amplio espectro de causas en la hematuria. COMENTARIOS: Existen causas de hematuria de presentación excepcional que debemos tener en cuenta en la práctica clínica. El estudio de la hematuria debe incluir siempre una completa anamnesis personal y familiar, una cuidada exploración física, análisis y cultivo de la orina. Es importante intentar proponer un algoritmo de estudio, evitando pruebas diagnósticas innecesarias e invasivas que no están exentas de complicaciones.

[Robotic pyeloplasty: first experiences]. / Pieloplastia robótica: primeras experiencias.

OBJECTIVE: To analyze our initial experience with the practice of robotic pyeloplasty and describe its advantages over laparoscopic and open surgery. PATIENTS AND METHODS: Retrospective study of the pediatric cases of pyeloplasty assisted by the robot da Vinci between March 2010 and May 2013. In all cases a transperitoneal approach was used with placement of 4 trocars (3 robotic trocars and 1 conventional laparoscopic). The technique used was the dismembered pyeloplasty Anderson-Hynes type. RESULTS: We operated 15 patients (12 men and 3 women) with a median age at surgery of 11 years (r: 5-18). The median weight was 40 kilos (r: 19-82). Five patients had a previous open pyeloplasty performed several years ago. All patients except but one were completed by robot. The only conversion to open surgery was performed because of difficulty in placing the double-J catheter due to a stenosis at the ureterovesical junction. They were no other intraoperative complications. The median operative time was 180 minutes (r: 110-252) and the median time for the robot docking 14 minutes (r: 4-50). The average hospital stay was 3.47 days (SD: 3). Mean follow-up was 16.97 months (SD: 10.24) and currently all patients are asymptomatic. CONCLUSIONS: Our initial experience seems to demonstrate that, coinciding with the literature, robotic pyeloplasty improves the results of laparoscopic pyeloplasty, retaining all its advantages and radically shortening the learning curve.

Determination of the translational and rotational stiffnesses of an L4-L5 functional spinal unit using a specimen-specific finite element model.

The knowledge of spinal kinematics is of paramount importance for many aspects of clinical application (i.e. diagnosis, treatment and surgical intervention) and for the development of new spinal implants. The aim of this study was to determine the translational and rotational stiffnesses of a functional spinal unit (FSU) L4-L5 using a specimen-specific finite element model. The results are needed as input data for three-dimensional (3D) multi-body musculoskeletal models in order to simulate vertebral motions and loading in the lumbar spine during daily activities. Within the modelling process, a technique to partition the constitutive members and to calibrate their mechanical properties for the complex model is presented. The material and geometrical non-linearities originating from the disc, the ligaments and the load transfer through the zygapophysial joints were considered. The FSU was subjected to pure moments and forces in the three anatomical planes. For each of the loading scenarios, with and without vertical and follower preload, the presented technique provides results in fair agreement with the literature. The novel representation of the nonlinear behaviour of the translational and rotational stiffness of the disc as a function of the displacement can be used directly as input data for multi-body models.

Stability of PreservCyt for Hybrid Capture (HC II) HPV test.

The Food and Drug Administration (FDA) has approved the Hybrid Capture II (HC II) assay to test for the presence of high-risk types of human papilloma virus (HPV) DNA using specimens in PreservCyt fixative for up to 21 days after collection. The ability of HC II to determine the presence of HPV DNA in actual patient samples after longer periods of storage has not been shown. To determine if specimens older than 21 days can yield useful results, 207 patient specimens that had been tested for HPV DNA by HC II (primary test) were tested again after a significant period of storage ranging from approximately 2.5 to 13.5 mo (retest). The results of the primary test and the retest agreed in 86% of the cases. The high level of agreement in the results suggests that the presence of high-risk types of HPV DNA can be determined from actual cervical cytology material in PreservCyt with the HC II assay for at least 3 mo after specimen collection.

Molecular characterization of rabbit scavenger receptor class B types I and II: portal to central vein gradient of expression in the liver.

To further elucidate the role of scavenger receptor class B type I (SR-BI) in reverse cholesterol transport and in atherogenesis, we performed studies in the rabbit, an animal model displaying a lipoprotein profile similar to that of human, expressing cholesteryl ester transfer protein in plasma and having been demonstrated to be susceptible to atherosclerosis. In this report, we describe for the first time the isolation and characterization of rabbit cDNA fragments encoding SR-BI and scavenger receptor class B type II (SR-BII). Development of an isoform-specific Taqman Real Time PCR system and generation of isoform-specific polyclonal antibodies allowed us to measure SR-BI and SR-BII expression in various rabbit organs on mRNA and protein levels, respectively. We found the highest expression of SR-BI in adrenal gland, liver, and proximal intestine; lesser expression was found in appendix and spleen. Immunohistochemical staining of frozen sections showed SR-BI expression in the cortex but not in the medulla of adrenal gland. An increasing portal to central vein gradient of expression was found within the hepatic lobule. As shown in this report, identification and characterization of SR-BI expression in the rabbit affords a powerful tool to elucidate the role of SR-BI in cholesterol homeostasis and atherogenesis in human.

TGF-beta cytokines increase senescence-associated beta-galactosidase activity in human prostate basal cells by supporting differentiation processes, but not cellular senescence.

The family of transforming growth factors betas (TGF-betas) comprises molecules involved in growth inhibition, stress-induced premature senescence, epithelial mesenchymal transition and differentiation processes. The aim of this study was to clarify the effect of long term exposure of human prostate basal cells to TGF-betas, which are found in high concentrations in prostatic fluid and areas of benign prostatic hyperplasia (BPH). Basal cell cultures established from prostate explants (n=3) were either grown into cellular senescence, or stimulated with TGF-beta1, beta2 and beta3. Similar to cellular senescence, TGF-beta stimulation resulted in an increase of SA-beta galactosidase (SA-beta-gal) activity, flattened and enlarged cell morphology, and down-regulation of the inhibitor of differentiation Id-1. TGF-beta-treated prostate epithelial cells neither showed terminal growth arrest nor induction of important senescence-relevant genes, such as p16(INK4A), IFI-6-16, IGFBP-3 or Dkk-3. Cells stained positive for cytokeratins 8/18, but did not express other lumenal markers, such as prostate-specific antigen and androgen-receptors. TGF-betas increased also the expression of the mesenchymal marker vimentin, indicating that basal epithelial cells underwent differentiation with lumenal and mesenchymal features. In contrast, in vitro-differentiated neuroendocrine-like cells from prostate organoide cultures, expressing chromogranin A and cytokeratin 18, strongly stained positive for SA-beta-gal. Thus, SA-beta-gal activity is not only a marker for senescence, but also for differentiation of human prostate epithelial cells. With regard to the in vivo situation, in addition to cellular senescence, TGF-beta could contribute to the increased number of SA-beta-gal positive epithelial cells in BPH.

Phospholipid and cholesteryl ester transfer are increased in lipoprotein lipase deficiency.

OBJECTIVES: Phospholipid transfer protein (PLTP) and cholesteryl ester transfer protein (CETP) are key enzymes in lipoprotein metabolism by mediating the transfer and exchange of phospholipids (PL) and neutral lipids between lipoproteins. Lipoprotein lipase (LPL) deficiency is associated with low HDL-cholesterol (HDL-C) levels in both, the homozygous and heterozygous state. In the present study we set out to investigate the role of lipid transfer proteins, which are known to strongly determine HDL-C levels, in LPL deficiency. DESIGN/SUBJECTS: Phospholipid acceptor and donor properties of lipoproteins, PLTP activity, CETP mass, activity and cholesteryl ester (CE) transfer were determined in two homozygous and six heterozygous LPL-deficient subjects and in 10 healthy, normolipidaemic controls, respectively. RESULTS: The HDL isolated from LPL-deficient subjects showed strongly increased PL-acceptance when compared with controls (homozygotes versus heterozygotes versus control: 26.46 +/- 15.26 vs. 3.41 +/- 1.61 vs. 1.89 +/- 0.33 micromol mL-1 h-1/micromol mL-1 PL; all P < 0.05). Phospholipid transfer from apolipoprotein B containing lipoproteins was increased in heterozygotes when compared with controls (46.66 +/- 23.3 vs. 28.91 +/- 18.05 micromol mL-1 h-1/micromol mL-1 PL, P = 0.05). PLTP activity, however, was similar in LPL-deficient subjects and controls. CETP mass was highest in homozygotes, whilst enzyme activity was similar in LPL-deficient subjects and controls. CE transfer was highest in homozygotes (72.5 +/- 8.8%) and lowest in controls (28.7 +/- 5.2%, P < 0.01). CONCLUSIONS: In conclusion, PL and CE transfer are increased in LPL deficiency and thus, partly explain low HDL-levels in LPL-deficient subjects. Enhanced transfer seems rather to be the result of altered lipoprotein composition and concentration than altered enzyme activity. Our findings on mechanisms leading to low HDL-C levels might show another aspect in atherogenesis in LPL deficiency.

Influence of leptin and insulin on lipid transfer proteins in human hepatoma cell line, HepG2.

AIM: Phospholipid transfer protein (PLTP) and cholesteryl ester transfer protein (CETP) are key enzymes in lipoprotein metabolism facilitating the transfer and exchange of cholesteryl esters, triglycerides and phospholipids between lipoproteins. In the study presented here, we investigated the influence of two hormones-the adipocyte-derived hormone leptin as well as insulin on the hepatic secretion of both, PLTP and CETP. METHODS: PLTP activity and CETP concentration-measured by exogenous substrate assay and enzyme-linked immunosorbent assay-were determined in supernatant of human hepatoma cell line HepG2 after single or combined exposure to leptin and insulin at physiological and supraphysiological concentrations, respectively. Messenger-RNA of PLTP and CETP was quantified by Northern blot analysis. RESULTS: Leptin suppressed PLTP activity and CETP-concentration by up to 33% and 23%, respectively. Insulin also suppressed PLTP activity by up to 11% and CETP-concentration by up to 16%. In combination, the two hormones had additive suppressive effects for both, PLTP activity and CETP-concentration. Northern blot analysis showed no difference in m-RNA levels after exposure to leptin or insulin. CONCLUSIONS: Leptin and insulin, both known to increase with body fat mass, suppress production of PLTP and CETP in HepG2 cells. When extrapolated to the in vivo situation, this suppressive effect may constitute a mechanism counteracting the potentially harmful action of lipid transfer proteins, particularly reduction of HDL-cholesterol, in conditions frequently associated with increased plasma triglyceride levels such as obesity and insulin resistance.