Estudio del daño renal en el paciente pediátrico con vejiga neurógena y su relación con el patrón de funcionamiento vesical y el tratamiento recibido / Study of kidney damage in paediatric patients with neurogenic bladder and its relationship with the pattern of bladder function and treatment received

Objetivos: El fallo renal es la principal causa de morbimortalidad en pacientes con mielodisplasia. Analizamos la presencia de lesiones renales en la gammagrafía (DMSA) de estos pacientes y la relacionamos con el tipo de funcionamiento vesical, así como con el retraso en recibir un manejo adecuado. Material y métodos: Realizamos un estudio retrospectivo de pacientes con mielodisplasia en nuestro hospital desde 2004. Analizamos datos epidemiológicos, clínicos y el patrón de funcionamiento vesical según estudios urodinámicos. Clasificamos a los pacientes en 4 patrones urodinámicos según el comportamiento del detrusor y del esfínter; y lo relacionamos con la función renal en la gammagrafía y el manejo recibido desde el nacimiento. Resultados: Estudiamos 39 pacientes con mielodisplasia. El patrón vesical más frecuente fue el tipo A (61,5%) con hiperactividad del esfínter y del detrusor, seguido del D (20,5%), C (7,8%) y B (5,1%). El 38% de nuestros pacientes (n = 15) presenta algún tipo de nefropatía. El 92,9% de los niños que reciben tratamiento adecuado en el primer año de vida, no presentan lesiones renales en la gammagrafía. Encontramos algún tipo de nefropatía en el 56% de los pacientes en los que el tratamiento adecuado se demora más de un año; siendo la nefropatía más severa cuanto más tarde se inicia el manejo. Conclusiones: Existe una relación estadísticamente significativa entre el retraso en el tratamiento y la alteración en la gammagrafía renal en pacientes con vejiga neurógena. Es fundamental el estudio y tratamiento precoz de los pacientes para disminuir el deterioro renal, disminuir la necesidad de cirugía y mejorar las opciones de continencia

Objectives: Kidney failure is the main cause of morbidity and mortality in patients with myelodysplasia. We analysed the presence of renal lesions in these patients using dimercaptosuccinic acid scintigraphy and related their presence with the type of vesical function and the delay in receiving appropriate management. Material and methods: We performed a retrospective study of patients with myelodysplasia treated in our hospital since 2004. We analysed the epidemiological and clinical data and the pattern of bladder function according to urodynamic studies. We classified the patients into 4 urodynamic patterns according to detrusor and sphincter behaviour. We linked this behaviour to renal function in the scintigraphy and the care received since birth. Results: The study included 39 patients with myelodysplasia. The most common bladder pattern was type A (61.5%), with sphincter and detrusor hyperactivity, followed by type D (20.5%), C (7.8%) and B (5.1%). Some 38% of our patients (n = 15) had some type of nephropathy. Some 92.9% of the children who were properly treated during the first year of their life had no renal lesions in the scintigraphy. We found some type of nephropathy in 56% of the patients for whom appropriate treatment was delayed for more than a year. The nephropathy was more severe the later the management was started. Conclusions: There is a statistically significant relationship between a delay in treatment and impairment in renal scintigraphy in patients with neurogenic bladders. The early study and treatment of patients is essential for decreasing renal impairment, reducing the need for surgery and improving the continence options

Study of kidney damage in paediatric patients with neurogenic bladder and its relationship with the pattern of bladder function and treatment received.

OBJECTIVES: Kidney failure is the main cause of morbidity and mortality in patients with myelodysplasia. We analysed the presence of renal lesions in these patients using dimercaptosuccinic acid scintigraphy and related their presence with the type of vesical function and the delay in receiving appropriate management. MATERIAL AND METHODS: We performed a retrospective study of patients with myelodysplasia treated in our hospital since 2004. We analysed the epidemiological and clinical data and the pattern of bladder function according to urodynamic studies. We classified the patients into 4 urodynamic patterns according to detrusor and sphincter behaviour. We linked this behaviour to renal function in the scintigraphy and the care received since birth. RESULTS: The study included 39 patients with myelodysplasia. The most common bladder pattern was type A (61.5%), with sphincter and detrusor hyperactivity, followed by type D (20.5%), C (7.8%) and B (5.1%). Some 38% of our patients (n=15) had some type of nephropathy. Some 92.9% of the children who were properly treated during the first year of their life had no renal lesions in the scintigraphy. We found some type of nephropathy in 56% of the patients for whom appropriate treatment was delayed for more than a year. The nephropathy was more severe the later the management was started. CONCLUSIONS: There is a statistically significant relationship between a delay in treatment and impairment in renal scintigraphy in patients with neurogenic bladders. The early study and treatment of patients is essential for decreasing renal impairment, reducing the need for surgery and improving the continence options.

Neuroma en la incisión de Pfannestiel como causa de un dolor abdominal intratable / Traumatic neuroma under Pfannestiel’s incision as a cause of untreatable abdominal pain

El neuroma es un crecimiento disperso y desordenado de las fibras nerviosas entremezcladas en tejido fibroso durante su proceso de regeneración. Es una patología poco común en la práctica clínica, y su diagnóstico en ocasiones resulta dificultoso y demorado en el tiempo. Presentamos el caso de una paciente de 13 años de edad con un cuadro de dolor abdominal incoercible y sin alteraciones en las pruebas diagnósticas realizadas, cuya causa resultó un neuroma en la cicatriz quirúrgica previa (AU)

The neuroma is a dispersed and disordered growth of the nervous fibers intermingled with fibrous tissue during its process of regeneration. It is a fairly uncommon pathology in the clinical practice and the diagnosis sometimes proves to be difficult and delayed in time. The author presents the clinical case of a 13-year-old patient with a severe abdominal pain, all diagnostic tests being normal without any alterations. The cause of the abdominal pain was a neuroma in a previous surgical scar (AU)

Megauréter obstructivo primario. Tratamiento endoscópico con balón de dilatación: nuestros primeros resultados / Primary obstructive megaureter. Endoscopic treatment with balloon dilatation: our first results

Tras los grandes avances endourológicos producidos en los últimos años, ha surgido una alternativa mínimamente invasiva a la cirugía abierta en el tratamiento del megauréter obstructivo primario (MOP). Presentamos nuestra experiencia con la dilatación endoscópica de la estenosis uretero vesical durante los 4 últimos años. Material y métodos: Entre los años 2005 y 2009 se han tratado en nuestro servicio mediante esta técnica un total de 10 pacientes. Todos ellos cumplían los criterios diagnósticos de MOP sin reflujo asociado. El procedimiento consistió en la realización de cistoscopia, comprobación de la estenosis y dilatación endoscópica de ésta con balón. Se deja introducido un tutor ureteral doble J, que se retirará en el primer mes post operatorio. El seguimiento posterior se lleva a cabo mediante una ecografía y una cistografía al cabo de 1 mes, un renograma a los 3 y 9 meses, y ecografías posteriores de manera seriada. Resultados: El 60% de nuestros casos corresponden a niños y el 40% a niñas. La localización izquierda del MOP representa el 60% de los casos. En el 70% de los pacientes el diagnóstico se realizó bajo una sospecha prenatal, en el 30% se halló tras una infección del tracto urinario (ITU). La media de edad durante la intervención quirúrgica fue de 14,4 meses. El tiempo de seguimiento evolutivo fue de 29 meses. Todos los pacientes mejoraron su patrón obstructivo. Como complicaciones, un paciente precisó un cambio de doble J a las 12 horas por encontrarse éste en la vejiga, y dos pacientes presentaron una ITU durante el postoperatorio. Conclusiones: El manejo endoscópico del MOP sin reflujo vesicoureteral asociado es un nuevo planteamiento terapéutico que evita una cirugía agresiva en el niño para realizar la reimplantación ureteral. Se trata de una técnica factible y efectiva, que precisará estudios a más largo plazo para demostrar su eficacia (AU)

Introduction: With the great advances in the urological endoscopic surgery in the last years, a new minimal invasive alternative to open surgery in the treatment of primary obstructive megaureter has been used. We present the results of our experience in the endoscopic dilatation of the uretro vesiclestenosis during the last 4 years. Material and method: Between the years 2005 to 2009 we present a total of ten have been treated through this technique. All of them complied with the criteria of the POM without associated reflux. In all cases the procedure consisted in a cytoscopy and the verification of stenosis and endoscopic dilatation of the same with a balloon. A double-J catheter was positioned and then withdrawn 1 month after the procedure. As a followup, an ultrasound monitoring is done as well as a cystographya month after, a renography at 3 and 9 months and posterior ultrasound monitoring in a serial manner. Results: 60% of our patients were boys and 40% were girls. In 60% of them the primary obstructive megaureter was on the left side. 70% of the patients were diagnosed due to a prenatal suspicion and 30% after an ITU. The mean intervention age was of 14.4 months. The mean follow-up was of 29 months. All the patients improved their obstructive curves. As complications: one patient required the removal of the double-J catheter, 12 hours after, because it was located in the bladder, and two patients showed a ITU during the post operatory. Conclusions: The endoscopic management of primary obstructive megaureter without uretro vesicle reflux is a new therapeutic approach which avoids an aggressive surgery in a child to do a urethral reimplantation. It is a feasible and effective technique which will need studies to a long term to be able to demonstrate its effectiveness (AU)

Tratamiento ortopédico de las malformaciones craneales posturales / Orthopedic treatment of the postural cranial malformations

Introducción: En 1992, la Academia Americana de Pediatría recomendó que los bebés durmieran boca arriba o de lado para reducir el riesgo de síndrome de muerte súbita del lactante. Desde entonces, la incidencia de las malformaciones craneales posturales ha aumentado de forma considerable y, con ello, sus consecuencias estéticas e incluso psicológicas. Gran parte de los niños afectados precisarán tratamiento ortopédico de su deformidad mediante ortesis craneal. Realizamos un estudio sobre la eficacia, las indicaciones y el resultado de dichas ortesis según la experiencia y el protocolo de tratamiento en la unidad de malformaciones craneales de nuestro hospital. Material y métodos: Analizamos los niños tratados por malformaciones craneales posturales durante 5 años. Recogemos los datos clínicos que nos han resultado más importantes para el diagnóstico diferencial con las sinostosis y la necesidad de pruebas complementarias. Valoramos la morfología de la lesión, su gravedad, los factores etiológicos asociados, la edad del niño en el momento del diagnóstico, la duración del tratamiento postural u ortésico y la eficacia de éste según nuestro registro fotográfico, así como la opinión subjetiva de los padres. Realizamos una revisión bibliográfica al respecto (AU)

Resultados: Hemos registrado un total de 105 niños con malformaciones craneales posturales, la gran mayoría con plagiocefalia. Entre los factores asociados podemos indicar que 12 pacientes estuvieron ingresados durante largo tiempo en la unidad de neonatos, 16 con una desproporción materno fetal, 8 niños padecían tortícolis congénita y, curiosamente, 12 niños eran de procedencia china por adopción, 8 con antecedente departo instrumental. La media de edad en el momento del diagnóstico en nuestra consulta fue de 6,3 meses, la misma media de edad en que se indicó una ortesis, ya que en general los pacientes acuden con un diagnóstico demasiado tardío como para poder instaurar el tratamiento postural, y sólo 16 niños acudieron o fueron diagnosticados antes de los 3 meses de edad. La ortesis se mantuvo durante una media de 4,5 meses. El porcentaje de malformación facial apreciable subjetivamente fue del 28%. El porcentaje de niños diagnosticados en nuestra consulta que no precisaron ortesis dentro del mismo tipo de deformidad fue nulo, dado el mencionado diagnóstico tardío. Ninguna malformación postural precisó cirugía. Se realizó una radiografía craneal al 20% de los niños, todas ellas solicitadas por médicos no especialistas. La cuantificación subjetiva de la mejoría fue mayor en los niños diagnosticados precozmente. El tiempo medio de resolución fue de 4,5 meses. Conclusiones: Las malformaciones craneales, dada su elevada incidencia actual, precisan un adecuado conocimiento, protocolo de tratamiento y diagnóstico precoz, ya que éste facilita la probabilidad de éxito con un tratamiento sencillo y corto; provocan una no despreciable incidencia de deformidad facial. El desconocimiento de esta patología conlleva la realización de pruebas complementarias innecesarias (AU)

Introduction: In 1992, the American Academy of Pediatrics (AAP) recommended for babies to sleep face up or sideways to reduce the risk of sudden infant death syndrome, since then, the incidence of cranial malformations has increased considerably and thus its esthetic and psychological consequences. Many affected children require orthopedic treatment of their cranial deformities through an orthosis helmet. We make a study of effectiveness, indications and results of these orthosis according to our experience and our treatment protocol in the unit of cranial malformations of our hospital. Material and methods: We studied children treated for postural cranial malformations during 5 years. We analyze the most important clinical data for differential diagnosis with synostosis and the need of additional studies. We appreciate themorphology of the lesion, severity, the associated etiological factors, child’s age at diagnosis, duration of conservative or orthotic treatment and their effectiveness in our photographic files and in the subjective opinion of the parents. We have made a bibliographic review on this matter (AU)

Results: We have registered a total of 105 children with positional cranial malformations, most of them with morphologically with plagiocephaly. The associated factors were: 12 patients stayed long time in our newborn unit, 16 had maternal fetal disproportion, 8 children suffered from congenital torticollis, oddly 12 children whom were adopted had a Chinese origin, 8 had history of instrumental delivery. The average age of diagnosis in our department was of 6.3 months, same mean age in which an orthosis was indicated, because they generally arrive with a late diagnosis to be able to establish the positional therapy, only 16 children attended or were diagnosed at ages younger than 3 months. The orthosis helmet was kept for an average of 4.5 months. The percentage of subjective facial malformation was of 28%. The number of children diagnosed in our department who did not require helmets within the same type of deformity was of zero because of such a late diagnosis. None of them needed surgery, skull radiography was performed in 20% of the children, all of them requested by a non-specialist doctors. The quantification of subjective improvement was greater in children with an early diagnosis. The mean time of resolution was of 4.5 months. Conclusions: Positional cranial malformations, because of their high incidence require a complete knowledge, treatment protocol and early diagnosis, as this provides higher probability of successful, simple a short treatment. They cause a non negligible incidence of facial deformity. The ignorance of this condition causes the consumption of unnecessary tests (AU)

[Quality of life in children operated on for anal atresia]. / Calidad de vida en los pacientes intervenidos de atresia de ano.

INTRODUCTION: Most children with anorectal malformations have some type of intestinal dysfunction. A correct follow up in this aspect after surgery affects their quality of life. MATERIAL AND METHODS: We gathered a sample of 20 children that were lost in their follow up after posterior sagittal anorectoplasty (PSARP). We got contact with them and they were interviewed and examined in our department. RESULTS: We collected 12 girls and 8 boys. Age range was between 3 and 14 years. 70% had good prognosis for continence (low fistula) and 30% poor prognosis (high fistula). 35% suffered from postoperative complications being the most frequent prolapse especially in high atresias. 85% had good rectal sensitivity, 15% had poor sensitivity that was directly related to incontinence. Anal tone was decreased in our exploration in 35% of patients which was not directly related to their continence. 65% suffered constipation with or without fecaloma. 5% of cases had intestinal hipermotility. The total incidence of fecal loose was 40%. More than a half (62,5%) kept on loosing stool despite treating their constipation or hipermotility, so we consider them true incontinents (no voluntary bowel movements). 67% of children with high fistula were true incontinents, just 7% of those with low fistula. Subjective quality of life in patients with soling was 6.4. In clean patients it was 9.3. Objective quality of life (Score/13) in dirty patients was 6.6. In Clean patients: 11.9. After our bowel management protocol we got 100% of patients clean during school time, thereby improving their quality of life. CONCLUSIONS: Incontinence determines the long-term quality of life in our patients in addition to the psycho-social consequences. They are clearly more frequent in patients with high fistula. Much assume incontinence as an unavoidable part of their disease so do not always demand treatment if they are not followed by a surgeon.

Toracoscopia en niños con empiema pleural en fase II. ¿Lavado y desbridamiento de tabiques de fibrina o decorticación? / Thorascoscopy in children with pleural empyema in 2nd stage, breaking and washing fibrinous loculations or decortication?

Introducción: Ante el aumento de incidencia de derrames pleurales paraneumónicos complicados en niños, ha aumentado considerablemente el número de toracoscopias realizadas, ya sea para el lavado y el desbridamiento de los tabiques de fibrina para liberar el líquido acumulado, o completando la intervención con decorticación completa de la fibrina aún no organizada adherida a la pared pulmonar. Material y métodos: Estudiamos una muestra de 31 niños tratados toracoscópicamente por un derrame pleural complicado en fase II. Estudiamos diversas variables: edad, tiempo de clínica y de tratamiento antibiótico preoperatorios, composición del líquido pleural, germen, opción quirúrgica, tiempo operatorio, días con drenaje pleural mayor de 20 mL/día o fiebre y complicaciones. Resultados: De 31 toracoscopias, el 52% son descorticaciones pulmonares y el 48% desbridamientos y lavados. Mediante el test de la t de Student comprobamos que el tiempo de drenaje pleural en los pacientes con desbridamiento simple es estadísticamente superior, al igual que los días de fiebre (sin diferencias significativas), y se requieren más fibrinolíticos y reintervención. No observamos una mayor incidencia de fuga aérea en la decorticación, aunque sí de íleo paralítico posquirúrgico. Conclusiones: La decorticación pulmonar en los derrames pleurales complicados en fase II conlleva un menor tiempo de drenaje torácico y clínica febril. En manos entrenadas no aumenta el tiempo operatorio ni el número de complicaciones graves (AU)

Introduction: Before the increase of incidence of complicated pleural parapneumonic effusion in children, has increased the number of Video Assisted Thoracoscopy (VATS) considerably. Whether it is for the washing or breaking of the nasal bone fibrin to liberate the accumulated liquid, or complete the intervention with complete decortication of a non-organized fibrin wall attached to the lung. Material and methods: We study a sample of 31 children whounder went VATS for complicated pleural effusion in 2nd stage. We study: age, clinical time and pre-surgical symptoms and antibiotics, composition of the pleural liquid, infectious agent, surgical option, operative time, days with fever and pleural drainage more than 20 cc/day or fever and complications. Results: From 31 VATS, 52 % were pulmonary decortications and 48 % debridement and wash. Using the t-student we get statistically significative difference in time of pleural drainage, longer in patients with simple debridement, as in days with fever, not being this difference significant. There is more need of fibrinolysis and reintervention in this group. We do not see more incidence of air leak in the decortication group, but there is more post surgery paralytic ileus. Conclusions: Lung decortication in pleural complicated effusions2nd stage entails less time with thoracic drainage and fever. In trained hands it does not increase neither the operative time nor the number of serious complications (AU)

Calidad de vida en los pacientes intervenidos de atresia de ano / Quality of life in children operated on for anal atresia

Introducción. El utrículo prostático consiste en una regresión incompleta de las estructuras Müllerianas. Puede presentar varios signos y síntomas, como incontinencia e infecciones del tracto urinario. Presentamos nuestra experiencia personal en la extirpación laparoscópica de lutrículo prostático en pacientes con sintomatología de ITU e incontinencia. Material y Métodos. La cirugía consiste en una cistouretroscopia y canalización del utrículo con colocación de sonda de Fogarty nº4 o del cistoscopio en el interior, si este atraviesa el orificio. Esta maniobra ayuda a la localización. La laparoscopia se lleva a cabo con un trocar de 10 mm y dos de 5 mm. Gracias a la tras iluminación, la localización es más sencilla. La resección se realiza con electrocoagulación y extirpación tras puntos transfixivos. Resultados. Se operaron cinco varones con una media de edad de7 (2-11) años. En todos la laparoscopia fue satisfactoria excepto en uno, que necesitó la reconversión a cirugía abierta. La media de duración fue de 110 (90-210) min. Conclusión. La exéresis laparoscópica del utrículo prostático es una vía segura y sencilla de tratamiento. La iluminación directa con el cistoscopio ayuda a la visualización y disección del mismo (AU)

Introduction. The prostatic utricle is an embryological remnantfrom Müllerian duct tissue. Most prostatic utricles are asympomatic, but they may manifest as a urinary tract infection or an incontinence. Material and method. Surgery consists in a cysto-urethroscopy and cannulation of the prostatic utricle with a ureteral catheter (Fogarty ner 4) and the cystoscopy left in situ to facilitate identification and mobilization. A 10 mm port through a umbilical incision and two more5 mm working ports were inserted. After mobilization, the ureteral defect was closed by an absorbable suture and ultrasonic coagulation. Results. Five boys with symptomatic prostatic utricles underwent surgery at a mean (range) age of 7 (2-11) years. The laparoscopic escisión was successful in all but one due to a bleeding. The mean (range) operative duration was 165 (120-240) min. Conclusion. Laparoscopic escision under cystoscopic guidance offers a good surgical view and allowing easy dissection (AU)

[Correction of lop ear using minimally invasive approach]. / Corrección de las orejas procidentes mediante un abordaje mínimamente invasivo.

INTRODUCTION: Helix valgus or procident ears is a common problem that affects about 5% of the population. The folds of the antehelix and the overdevelopment of the concha are the most commonly found anatomic alterations of the ear pavilion. In children this pathology usually causes anxiety and an emotional trauma that may interfere in their normal development. MATERIALS AND METHODS: There are a few tipes of techniques to correct helix valgus. We present the application of the technique in our service. We conduct the otoplastia with an outer puntiform technique which allows us to cut the cartilage partially from the outside. Next we fold from the rear the antehelix and hide the concha. RESULTS: We analysed 7 years of the application of this technique and we now present 87 otoplastias conducted to 44 children. The 97% of them were bilateral. No precocious complications have been observed after the surgery. All cases except for one of them have been bilateral. All the patients were satisfied with the aesthetic results. None of them showed relapse. In one case there was a hypertrophic scar that required cutting and in 2 of the cases there was a slight hypercorrection. CONCLUSIONS: Procident ears may occasion a psychological trauma in children. We believe that this technique, which is minimally invasive, provides very satisfactory aesthetic results, the puntiform scar being hardly noticed fifteen days before surgery. The patients need to stay in hospital for a short period, 24-48 hours, and complications are very rare, recidiva has not been described. We strongly recommend this technique for the correction of procident ears.

Leishmania infantum: antiproliferative effect of recombinant plant cystatins on promastigotes and intracellular amastigotes estimated by direct counting and real-time PCR.

Two recombinant barley cystatins, HvCPI5 and HvCPI6, have been tested in vitro against promastigotes and intracellular amastigotes of Leishmania infantum in the J774 monocytic cell line. Toxicity of cystatins for J774 cells was also determined. In addition, a comparison between direct counts of intracellular amastigotes and quantitation of burden by Q-PCR was carried out. Low concentrations (2 microM) from both cystatins were unable to inhibit promastigote replication. HvCPI5 was toxic for mammalian cells; 0.1 microM reduced by more than 50% the cell viability. On the contrary, HvCPI6 did not exhibit any toxicity for J774 cells up to 6 microM and inhibited the intracellular amastigote multiplication. Dose-response analysis showed that 4.8 microM HvCPI6 reduced by >90% the intracellular parasite load and had an approximate IC(50) value of 1.5 microM. Comparable results were obtained by direct counting of intracellular amastigotes and Q-PCR. Results point towards the direct inhibition of amastigote multiplication by HvCPI6 and the interest of this recombinant cystatin in the chemotherapy of leishmaniasis.

[Non-palpable testes: laparoscopic or inguinal approach?]. / Testes no palpables: abordaje laparoscópico o inguinal?

INTRODUCTION: Twenty per cent of the operated patients suffering from cryptorchidisim show no palpable testis in the physical check-up. The use of a non-palpable testis in the initial stages is considered to be controversial when deciding between a laparascopic or an inguinal approach. Our aim is to compare the results obtained with these two approaches and evaluate which one of them would be the most relevant as an initial option. MATERIALS AND METHODS: We examined the patients who have been subjected to surgical intervention for non-palpable testis in the last three years. We had a sample of 53 patients. Bilateral non-palpable testes were disregarded. In the case of 35 patients the initial approach was through the groins while in 18 of them the approach was laparascopic. Relevant data were recorded, such as the age of the patient, right or left side, surgical findings, need for a laparascopic or groin approach and associated hernia. RESULTS: The average age of the patients at the time of the surgical treatment was 3.7 years R (1-13 years). 42% of the testes were on the right side and 58% on the left. In an initial stage the inguinal approach was used with 35 patients, 2 of these requiring laparascopic exploration due to a non-concluding check-up; it was concluded that they were 2 cases of anorchia. An initial laparoscopic approach was used with 18 patients. Internal vessels in the canal were found which required an inguinal approach. In 12 patients normal or atrophic testes were observed. Testicular descent was achieved through the groin in 10 of them while in the remaining 2 a combined approach was adopted. CONCLUSIONS: 80% of the patients subjected to a first laparoscopic approach needed a groin approach later on. 11% of the patients subjected to a first inguinal approach required laparascopic examination. In view of the results obtained, we conclude that inguinal exploration diminishes the need for second procedures.

[Therapeutic approach of enuresis based on the differentiation of the enuretic syndrome and monosymptomatic enuresis]. / Enfoque terapéutico de la enuresis basándose en la diferenciación del síndrome enurético y la enuresis monosintomática.

INTRODUCTION: Enuresis affects 15% of the children under 5. Possible etiopatogeny explaining the mechanism of production of nocturnal enuresis has been described, resulting in different terapeutical approaches; however, we cannot speak up to now of general guidelines for its treatment. MATERIALS AND METHODS: On the basis of 544 children who were treated in our hospital in the last 12 years, we analysed a sample of 124 patients corresponding to the last two years. We now present the therapeutical protocol used and analyse the results. In the initial therapeutical approach a distinction is made between monosymptomatic enuresis and eneuretic syndrome. RESULTS: We studied a total of 120 patients (89 boys and 31 girls). 63% of them showed monosymptomatic enuresis while 37% suffered from enuretic syndrome. In the case of 15% of them, this was associated with heavy sleep and difficulties to wake up. 92% was the general percentage of recovery. In the patients suffering from nonosymptomatic eneuresis, the problem was solved with desmopressin in 87% of them (demospressin on its own in 65% or in combination with oxibutine in the remaining 35%). In the group of enereutic symdrome, the problem was solved with oxibutine in 71% (on its own in 40% or in combination with desmopressin in 54%. CONCLUSIONS: The treatment and solution of enuresis improve the child's self-esteem and the anxiety caused in the family. A good medical history with a clear distinction between enuretic syndrome and monosyntomatic enuresis leads us to a suitable therapeutical approach for every patient, allowing us to find earlier the right treatment for every individual.

Corrección de las orejas procidentes mediante un abordaje mínimamente invasivo / Correction of lop ear using minimally invasive approach

Introducción. El hélix valgus u orejas procidentes es un problema común que afecta a un 5% de la población. Las alteraciones anatómicas del pabellón que más frecuentemente se encuentran son el defecto en el desarrollo de los pliegues de antehélix y el sobre desarrollo dela concha. Esta patología en los niños suele causar ansiedad y un trauma emocional que puede interferir en su desarrollo. Material y métodos. Existen múltiples técnicas descritas para la corrección del hélix valgus. Describimos la técnica realizada en nuestro servicio durante los últimos siete años analizando los resultados. Realizamos la otoplastia mediante un abordaje anterior puntiforme que nos permite realizar de una manera poco agresiva varios cortes parciales del cartílago por vía anterior. Por vía posterior realizamos la (..) (AU)

Introduction. Helix valgus or procident ears is a common problem that affects about 5% of the population. The folds of the antehelix and the overdevelopment of the concha are the most commonly found anatomic alterations of the ear pavilion. In children this pathology usually causes anxiety and an emotional trauma that may interfere in their normal development. Materials and methods. There are a few tipes of techniques to correct helix valgus. We present the application of the technique in our (..) (AU)

Testes no palpables: ¿abordaje laparoscópico o inguinal? / Non-Palpable testes: Laparoscopic or inguinal approach?

Introducción: El 20% de los pacientes intervenidos por criptorquidia presentan teste no palpable en la exploración física. El manejo inicial de estos pacientes sigue siendo controvertido. Nuestro objetivo es comparar el abordaje inguinal y el laparoscópico y valorar cuál de ellos es el más resolutivo como opción inicial. Material y métodos: Revisamos los pacientes intervenidos por teste no palpable unilateral en los últimos tres años en nuestro servicio. Tras descartar los testes no palpables bilaterales entramos en el estudio53 pacientes. En 35 el abordaje inicial realizado fue por vía inguinal y en 18 laparoscópico. Los datos recogidos para el estudio son la edad del paciente en el momento de la intervención, lado del teste no palpable, hallazgos quirúrgicos, necesidad de abordaje combinado laparoscópico o inguinaly presencia o no de hernia asociada. Resultados: La edad media de los pacientes en el momento de la intervención quirúrgica fue de 3,7 años (R: 1-13 años). El 42% de los testes no palpables correspondían al lado derecho y el 58% al izquierdo. El 65 % de los pacientes se abordaron inicialmente por vía inquinal. En tan sólo el 11% debido a una exploración no concluyente se necesitó realizar una exploración laparoscópica que concluyó en todos los casos anorquia. En el 33% el abordaje inicial fue laparoscópico. En (..) (AU)

Introduction: Twenty per cent of the operated patients suffering from cryptorchidisim show no palpable testis in the physical check-up. The use of a non-palpable testis in the initial stages is considered to be controversial when deciding between a laparascopic or an inguinal approach. Our aim is to compare the results obtained with these two approaches and evaluate which one of them would be the most relevant as an initial option. Materials and methods: We examined the patients who have been subjected to surgical intervention for non-palpable testis in the last three years. We had a sample of 53 patients. Bilateral non-palpable testes were disregarded. In the case of 35patients the initial approach was through the groins while in 18 of them the approach was laparascopic. Relevant data were recorded, such as the age of the patient, right or left side, surgical findings, need for alaparascopic or groin approach and associated hernia. Results: The average age of the patients at the time of the surgical treatment was 3.7 years R ( 1-13 years). 42% of the testes were on the right side and 58% on the left. In an initial stage the inguinal approach was used with 35 patients, 2 of these requiring laparascopic exploration due to a non-concluding check-up; it was concluded that they were 2 cases of anorchia. An initial laparoscopic approach (..) (AU)

[25 years experience in cerebrospinal shunt. Are new systems better?]. / 25 años de experiencia en válvulas de derivación ventrículo-peritoneal. Son mejores los nuevos sistemas?

INTRODUCTION: Since the introduction of the first ventricular shunt in 1949, the cerebrospinal shunts are the most used choice for the management of hydrocephalus in children. With the technological advance, systems have been developing increasingly sophisticated. Our aim is to study the incidence of complications after the implantation of these shunts and the variables associated to it. MATERIAL AND METHODS: We perform a retrospective study between 1981 and 2006, gathering the patients between 0 and 15 years treated at our Hospital for the placement of a cerebrospinal shunt or as consequence of a problem of the device. We analyze the tipe of shunt (Hakim, Pudentz, Delta, Orbis-Sigma, Strata, Codman), etiology of hydrocephalus, age, immediate and late complications, permanence of the system, changes of the system and cerebrospinal fluid. The qualitative data were analyze by c2 test. We also estimate survival of shunts with Kaplan-Meier stimator and make a multivariant Cox regression analysis. RESULTS: A total of 75 patients. The main etiology for hydrocephalous was myelomeningocele, followed by congenital and posthemorrhagic. Most of the patients presented complications of the system in the follow-up. They were adjusted for age, sex, etiology, shunt and surgeon. The variable that had an independent effect for predicting the presence of a complication was the type of shunt, being the most ancient systems those with major survival. The most frequent complication in the immediate period (< 3 month) was the obstruction and infection of the proximal catheter or shunt. Distal catheter disconnection or break prevailed in the late one. We got few functional complications, these were not related with the type of shunt. CONCLUSIONS: In our experience, the shunts with the less short-term probability of complication (< 3 months) have been old simple systems like Hakim and Pudentz. The functional complications, that are those which presumably should be reduced by the new programmable systems, don,t seem do it, neither mean longer survival of the system.

25 años de experiencia en válvulas de derivación ventrículo-peritoneal. ¿Son mejores los nuevos sistemas? / 25 years experience in cerebrospinal shunt. Are new systems better?

Introducción. Desde la introducción del primer shunt ventricular en 1949, las válvulas de derivación ventrículo-peritoneal constituyen la forma de derivación más utilizada en niños y adultos con hidrocefalia. Con el avance tecnológico se han ido desarrollando sistemas cada vez más sofisticados. Nuestro objetivo es determinar la incidencia de complicaciones tras la implantación de válvulas ventrículo-peritoneales y las variables asociadas a las mismas. Material y métodos. Realizamos un estudio retrospectivo entre1981 y 2006, recogiendo a los pacientes entre 0 y 15 años ingresados en nuestro Hospital para colocación de válvula de derivación o como consecuencia de un problema del dispositivo. Realizamos una recogida de datos incluyendo tipo de válvula (Hakim®, Pudenz®, Delta®, Orbis-Sigma®, Strata®, Codman®), causas de la hidrocefalia, edad, complicaciones inmediatas y tardías, durabilidad de los sistemas, recambios,análisis de LCR. La asociación de variables cualitativas se realizó por medio del estadístico Chi cuadrado. Se hizo a su vez un análisis de supervivencia con la metología Kaplan-Marie y un análisis multivariado de regresión de Cox. Realizamos además una revisión bibliográfica al respecto. Resultados. Se recogieron 75 pacientes. La causa más frecuente de hidrocefalia ha sido la espina bífida, seguida de malformaciones congénitas y hemorragia intraventricular. La mayoría presentan complicaciones en el seguimiento. Ajustando por edad y por tipo de válvula la variable que tiene un efecto independiente para predecir la presencia de una complicación es el tipo de válvula, siendo los sistemas más antiguos los de mayor supervivencia. El sexo y la edad no modifican dicha probabilidad. La complicación más frecuente fue de tipo obstructivo e infeccioso en el período precoz y desconexión-rotura del catéter distal en el tardío. Las complicaciones funcionales fueron escasas y no dependieron del tipo de válvula. Conclusiones. Las válvulas con menos probabilidad a corto plazo de presentar complicaciones (< de 3 meses) han resultado ser los antiguos sistemas Hakim® y Pudenz®. Las complicaciones funcionales, que son las que presumiblemente evitarían los nuevos sistemas, no parecen mejorar con ellos y no conllevan una mayor supervivencia del sistema (AU)

Introduction. Since the introduction of the first ventricular shunt in 1949, the cerebrospinal shunts are the most used choice for the management of hydrocephalus in children. With the technological advance, systems have been developing increasingly sophisticated. Our aim is to study the incidence of complications after the implantation of these shunts and the variables associated to it. Material and methods. We perform a retrospective study between1981 and 2006, gathering the patients between 0 and 15 years treated at our Hospital for the placement of a cerebrospinal shunt or as consequence of a problem of the device. We analyze the type of shunt (Hakim®,Pudentz®, Delta®, Orbis-Sigma®, Strata®, Codman®), etiology of hydrocephalus, age, immediate and late complications, permanence of the system, changes of the system and cerebrospinal fluid. The qualitative data were analyze by c2 test. We also estimate survival of shunts with Kaplan-Meier stimator and make a multivariant Cox regression analysis. Results. A total of 75 patients. The main etiology for hydrocephalous was myelomeningocele, followed by congenital and posthemorrhagic. Most of the patients presented complications of the system in the follow-up. They were adjusted for age, sex, etiology, shunt and surgeon. The variable that had an independent effect for predicting the presence of a complication was the type of shunt, being the most ancient systems those with major survival. The most frequent complication in the immediate period (< 3 month) was the obstruction and infection of the proximal catheter or shunt. Distal catheter disconnection or break prevailed in the late one. We got few functional complications, these were not related with the type of shunt. Conclusions. In our experience, the shunts with the less short-term probability of complication (< 3 months) have been old simple systems like Hakim® and Pudentz® The functional complications, that are those which presumably should be reduced by the new programmable systems, don’t seem do it, neither mean longer survival of the system (AU)

Ternary complex formation between HvMYBS3 and other factors involved in transcriptional control in barley seeds.

The SHAQKYF R1MYB transcription factor (TF) HvMYBS3 from barley is an activator of gene expression both during endosperm development and in aleurone cells upon seed germination. Its mRNA was detected as early as 10 days after flowering in developing barley endosperm, with a peak at 18 days, and in aleurone cells at 8 h after water imbibition, as shown by Northern blot and in situ hybridization analyses. The HvMYBS3 protein expressed in bacteria binds to oligonucleotides containing a GATA core derived from the promoters of: (i) the developing endosperm gene Itr1 (5'-GATAAGATA-3') encoding trypsin inhibitor BTI-CMe, and (ii) the post-germinating aleurone gene Amy6.4 (5'-TATCCAC-3'/5'-GTGGATA-3') encoding a high-pI alpha-amylase. Transient expression experiments in co-bombarded developing endosperms and in barley aleurone layers demonstrated that HvMYBS3 trans-activated transcription both from Itr1 and Amy6.4 promoters, in contrast with a previously reported seed-expressed R1MYB, HvMCB1, which was an activator of Itr1 and a transcriptional repressor of the Amy6.4 gene. In the yeast three-hybrid system, the HvMYBS3 protein formed a ternary complex with BPBF and BLZ2, two important seed TFs. However, no binary interactions could be detected between HvMYBS3 and BLZ2 or between HvMYBS3 and BPBF.

Conformadores nasales en la rinoplastia primaria del paciente fisurado / Nostril reatainers in the primary cleft rhinoplasty

Introducción. Tras la realización de la rinoplastia primaria o secundaria en el paciente fisurado existe una tendencia a la depresión del cartílago alar perdiendo la nueva morfología obtenida. Por este motivo es habitual el empleo de conformadores nasales en el postoperatorio de la rinoplastia de estos pacientes, ya sea ésta abierta o cerrada. Objetivo. Presentamos nuestra experiencia en el uso de conformadores nasales para mantener en posición el cartílago alar tras la rinoplastia del paciente con fisura unilateral o bilateral, evitando así la deformación producida por la retracción cicatricial. Pacientes y métodos. Hemos colocado 18 stents nasales en 18 pacientes con fisura nasolabial intervenidos (4 bilaterales y 14 unilaterales) en el período 2001-2004. Realizamos una comparación fotográfica de la simetría nasal entre los pacientes intervenidos sin stents en un período anterior (1998-2000) y el grupo de estudio. Los conformadores nasales se fijan en su posición tras la rinoplastia mediante suturas reabsorbibles de Monocryl® 4/0. Estas suturas mantienen el stent en su lugar durante unos 20 días después de la intervención. Resultados. El tiempo medio de retención del conformador nasal fue de 3,5 meses (4 semanas a 6 meses). Los métodos empleados para mantener el stent en su lugar tras la absorción de las suturas de fijación fueron diversos y siempre en función de la comodidad de las familias. El estudio comparativo demostró una asimetría relevante de ambas narinas en el 10% de los pacientes con stents frente al 48% de aquellos sin stents (p<0,01). Conclusiones. El empleo de conformadores nasales en el postoperatorio protege la corrección quirúrgica del cartílago evitando la depresión de la narina, así como la aparición de adherencias intranasales precoces. Permite igualmente la realización sin molestias de lavados nasales y pueden mantenerse hasta la cicatrización del cartílago. Su uso implica una colaboración adecuada por parte de la familia (AU)

Introduction. The nasal cleft cartilage tends to depression after primary rhinoplasty. Nasal stents are probe to be useful in the management of these patients in order to maintain the new morphology. Aim. The purpose of this paper is to present our experience with the use of postoperative nasal splinting in the management of cleft lip nasal deformity. Patients and methods. A nostril retainer was placed in 18 cleft patients (4 bilateral, 14 unilateral) during primary repair of the cleft lip nasal deformity from 2001 to 2004. The nasal morphology in the postoperative period was compared with that of 10 control patients who were operated on without nasal stenting in a previous period. Nostril retainers were left in place after the rhinoplasty using 4/0 poliglecaprone absorbable. Those sutures fixed the stent in place during three weeks after surgery. Results. Average follow-up was 18.5 months. Retention of the splint was 3.5 months mean time (range 4 weeks to 6 months). The methods employed for retention were multiple based upon parental preferences. Photogrammetric analysis showed relevant asymmetry of the nostrils in 10% of the splinted group as compared with 48% for controls (p<0.001). Conclusions. The use of nasal stents has been found effective postoperatively after cleft primary rhinoplasty, avoiding relapse of the nasal reconstruction and drop of the nasal ala. Retainers also allows airway patent and avoid surgical adhesions due to nasal secretions and scarring. Parental collaboration is needed (AU)

[Congenital cystic adenomatoid malformation associated with pulmonary sequestration]. / Malformación adenomatoidea quística congénita asociada a secuestro pulmonar. Presentación de dos casos clínicos.

The association of congenital cystic adenomatoid malformation with pulmonary sequestration is infrequent. Has been reported less than 60 cases. We describe two patients with this association. One of this with extralobar pulmonary sequestration, and the other with an intralobar one.

Malformación adenomatoidea quística congénita asociada a secuestro pulmonar. Presentación de dos casos clínicos / Congenital cystic adenomatoid malformation associated with pulmonary sequestration

La asociación de una malformación adenomatoidea quística congénita con un secuestro pulmonar es infrecuente. Han sido descritos en la literatura menos de 60 casos de esta asociación. Presentamos los casos de dos pacientes con esta asociación, uno de ellos con secuestro pulmonar intralobar y el otro extralobar (AU)

The association of congenital cystic adenomatoid malformation with pulmonary sequestration is infrequent. Has been reported less than 60 cases. We describe two patients with this association. One of this with extralobar pulmonary sequestration, and the other with an intralobar one (AU)