[Clinical characteristics and complications in patients with OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome. Our experience]. / Síndrome de OHVIRA: características clínicas y complicaciones, nuestra experiencia.

INTRODUCTION: Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rare variant of Müllerian anomalies. Delay in diagnosis and treatment can produce serious complications in patient's life and fertility. The aim of this study is to present our experience in clinical presentation, diagnosis and complications of patients with OHVIRA syndrome in a 20-year period. PATIENTS AND METHODS: Retrospective analysis of 9 women under 25 years old with OHVIRA syndrome between 1997 and 2017. Analysis included clinical characteristics, demographic, diagnostic methods, follow-up, treatment and complications. RESULTS: A total of 9 patients were studied, 5 diagnosed in prepuberal age and 4 postpuberal. 55% had prenatal diagnosis of renal agenesis and in 3 was incidentally diagnosed. Follow up period for single kidney to diagnosis was 3.6 years (range: 0-13,4 years). Right side was affected in 55%. Symptoms were dysmenorrea (44%), recurrent abdominal pain (22%), urinary dysfunction (22%). Complications were present in postpuberal patients and included hydrometrocolpos (22%) and pyocolpos (11%) requiring septostomy or septum resection. Surgical removal of the septum was performed in 44%). Prepuberal patients were asymptomatic. CONCLUSIONS: OHVIRA syndrome is a rare anomaly in the development of Müllerian ducts with variable clinical presentation. High suspect in patients with single kidney is necessary to avoid potential complications by providing surgical treatment.

INTRODUCCION: El síndrome de OHVIRA (SO) es una rara y compleja variante de las anomalías de los conductos de Müller caracterizado por hemivagina obstruida, útero didelfo y anomalía renal ipsilateral. Su diagnostico prepuberal es raro y el retraso en su diagnóstico y tratamiento compromete la vida y fertilidad de la paciente. MATERIAL Y METODOS: Análisis retrospectivo de las historias clínicas de las pacientes diagnosticadas en nuestro centro en un período de tiempo de 20 años. Los datos se recogen en un protocolo que incluye: características clínicas, diagnóstico, tratamiento y complicaciones. RESULTADOS: Nueve pacientes en total, 55% diagnosticadas en edad prepuberal y 45% postmenarquia. En el 55% se realizó diagnóstico prenatal de malformación renal. En el 33% antecedentes familiares de primer grado de enfermedades nefrourológicas. La media de seguimiento por riñón único, previo al diagnóstico, fue de 3,6 años (rango 0-13,4 años). En el 55% de los casos el lado afecto fue el derecho. Los síntomas fueron ginecológicos en el 44%, miccionales en el 22% y dolor abdominal recurrente en el 22%. Presentaron complicaciones en el 33%, correspondiendo a hidrometrocolpos (22%) y piocolpos (11%). Se realizó septostomía y resección del tabique vaginal como tratamiento en el 44%, todas en edad postpuberal. Las pacientes prepuberales continúan en seguimiento con actitud conservadora y están asintomáticas. CONCLUSIONES: El SO es una rara anomalía del desarrollo de los conductos müllerianos de presentación clínica variable. El alto índice de sospecha y el diagnóstico precoz en niñas con anomalías renales, facilita el tratamiento quirúrgico antes de la presentación de complicaciones.

Congenital malformations of the urinary tract: progression to chronic renal disease. / Malformaciones congénitas del tracto urinario (CAKUT): evolución a enfermedad renal crónica.

INTRODUCTION: Congenital nephrourological abnormalities (CAKUT) are a particularly relevant group of diseases due to their high prevalence and the fact they are the main cause of chronic renal disease (CRD) in the pediatric population. Our objective was to determine the characteristics and prevalence of CAKUT in our setting, while identifying the factors associated with the occurrence of renal damage. MATERIALS AND METHODS: A retrospective, descriptive, analytical, cross-sectional study of patients seen in the Pediatric Nephrology Department of a third-level hospital from January 1 to December 31, 2018 was carried out. Epidemiological, clinical, and analytical variables were assessed, and potential risk factors associated with CRD were searched for. RESULTS: The study involved 685 patients with 827 kidney units affected by CAKUT with a mean age of 9.98 ± 5.12 years. 62.2% were male, and the mean follow-up period after diagnosis was 9.95 ± 5.09 years. 58.8% were non-obstructive dilations, followed by renal dysplasia, obstructive dilations, and number and position abnormalities. The most frequent malformation was vesicoureteral reflux (VUR). The most commonly affected side was the left (47.5%). 55% of the diagnoses were prenatal. 172 patients underwent surgery. The initially chosen treatment for VUR was endourological. Overall re-intervention rate was 20%. Of the total number of patients, glomerular filtration rate was analyzed in 383, 95 (24.8%) of whom had CRD (86% in stage 2). Male sex, bilaterality, and proteinuria were risk factors associated with CRD. CONCLUSIONS: Knowledge of the epidemiological and clinical characteristics of children with CAKUT and the factors associated with CRD helps to individualize the clinical follow-up of these patients, thus customizing diagnostic tests and healthcare resources.

INTRODUCCION: Las anomalías nefrourológicas congénitas (CAKUT), constituyen un grupo de enfermedades de gran relevancia por su alta prevalencia y por ser la principal causa de enfermedad renal crónica (ERC) en la población pediátrica. Nuestro objetivo es conocer las características y la prevalencia de CAKUT en nuestro medio, identificando los factores asociados a la aparición de daño renal. MATERIAL Y METODOS: Estudio retrospectivo, descriptivo, analítico y transversal, que incluyó los pacientes atendidos en la consulta de Nefrología Pediátrica de un hospital de tercer nivel desde el 1 de enero al 31 de diciembre de 2018. Se analizaron variables epidemiológicas, clínicas y analíticas, y se buscaron posibles factores de riesgo asociados a ERC. RESULTADOS: Se incluyeron 685 pacientes con 827 unidades renales con CAKUT con una edad media de 9,98 ± 5,12 años. El 62,2% fueron varones y el seguimiento medio desde el diagnóstico fue de 9,95 ± 5,09 años. El 58,8% fueron dilataciones no obstructivas, seguido por displasia renal, dilataciones obstructivas y anomalías de número y posición. La malformación más frecuente fue el reflujo vesicoureteral (RVU). El lado más afectado fue el izquierdo (47,5%). El 55% de los diagnósticos fueron prenatales. Fueron intervenidos 172 pacientes. El tratamiento inicial de elección en el RVU fue endourológico. La tasa de reintervención global alcanzó el 20%. Del total de pacientes, se pudo analizar el filtrado glomerular en 383 pacientes, de los cuales 95 (24,8%) tenían ERC (86% en estadio 2). El sexo masculino, la bilateralidad y la proteinuria fueron factores de riesgo asociados a la ERC. CONCLUSIONES: El conocimiento de las características epidemiológicas y clínicas de los niños con CAKUT y de los factores asociados a la ERC ayuda a individualizar el seguimiento clínico de estos pacientes adecuando las pruebas diagnósticas y los recursos sanitarios.

[Organisation of paediatric resident rotations by specific areas]. / Organización de las rotaciones del residente de pediatría por las áreas específicas.

INTRODUCTION: Pediatric resident training is based on rotations. The tutor and the head of the area are responsible for planning the rotation. MATERIAL AND METHODS: An organisational model of rotation for a Pediatric residents program is presented. It includes educational objectives, theoretical contents, abilities and attitudes that must be acquired by the resident, training activities to achieve such objectives, strategies of stimulus and evaluation tools. COMMENTS: The aim of this model is to improve the resident teaching-learning process and help in the planning of resident training for those in charge of pediatric areas.

[Acute renal failure in critically-ill children. A preliminary study]. / Insuficiencia renal aguda en niños críticamente enfermos. Estudio preliminar.

OBJECTIVE: To analyze the characteristics of acute renal failure (ARF) in critically-ill children and develop a protocol for a multicenter study. METHODS: A prospective, descriptive study was performed in four pediatric intensive care units (PICU) over 5 months. Epidemiological, clinical and laboratory data from children aged between 7 days and 16 years with ARF were analyzed. Premature neonates were excluded. RESULTS: There were 16 episodes of ARF in 14 patients and 62.5 % were male (mean 6 SD age: 50 +/- 49 months). The incidence of ARF was 2.5 % of PICU patients. The most frequent primary diseases were nephro-urological (50 %) and heart disease (31 %). The main risk factors for ARF were hypovolemia (44 %) and hypotension (37 %). Six patients (37.5 %) developed ARF following surgery (cardiac surgery in four, kidney transplantation in one and urological surgery in one). Furosemide was used in 13 patients (as continuous perfusion in nine), inotropes in nine and renal replacement therapy in 12. Medical complications were found in 94 % and some organic dysfunction was found in 81 %. The length of stay in the PICU was 21 +/- 21 days. The probability of death according to the Pediatric Risk of Mortality was 14 +/- 8 %. Five patients died (36 % of the patients and 31.2 % of ARF episodes). CONCLUSIONS: The incidence of ARF in critically-ill children is low but remains a cause of high mortality and prolonged stay in the PICU. Mortality was caused not by renal failure but by multiple organ failure.

[Fatal outcome after severe cerebellar infarction due to spontaneous dissection of the vertebral artery]. / Evolución fatal tras infarto cerebeloso masivo por disección espontánea de arteria vertebral.

Cerebellar infarction due to vertebrobasilar ischemia in spontaneous vertebrobasilar dissection is infrequent in children and adolescents. The commonest pathogenic mechanism of cerebellar infarction is arterial occlusion. Although usually favorable, outcome can be fatal, as in the present case. A previously healthy 4-year-old girl was admitted to the emergency room with sensory failure and trembling. The previous week she had suffered from a transitory ischemic attack that resolved spontaneously. On admission to the emergency department, the Glasgow Coma Scale score was 10/15 and she presented irregular polypneic breathing, anisocoric reactive pupils, enhanced reflexes, lower limb clonus and bilateral pyramidal signs. Cranial computed tomography revealed marked hypodensity in both cerebellar hemispheres and white supratentorial substance with obliteration of the quadrigeminus cisterns. Cerebral angiography showed left vertebral artery dissection with distal pseudoaneurysm and marked basilar occlusion. Despite aggressive antiedemic treatment and early instauration of intravenous continuous sodium heparin infusion, the patient developed a bilateral cerebellar infarction and supratentorial hydrocephalus. Brain death was diagnosed after 48 hours. Postmortem pathologic examination revealed previous thrombus the in superior left cerebellar artery and basilar artery occlusion. We discuss the absence of predisposing factors and the transitory ischemic attack in this patient 1 week before she developed massive cerebellar infarction.

[Prolonged Guillain-Barré syndrome]. / Síndrome de Guillain-Barré prolongado.

Outcome in Guillain-Barré syndrome (GBS) in childhood is usually favorable although prolonged and severe forms may develop. We report the case of a 12-year-old boy with rapidly ascending, slowly-progressive GBS with severe cranial nerve and autonomic dysfunction. Diagnosis was based on clinical presentation, electromyography, cerebrospinal fluid analysis and plasmatic antiganglioside GM1 antibodies. Early treatment with an initial loading dose of 2 g/kg of intravenous gammaglobulins over 5 days was given with a significant but slow clinical improvement. A repeat infusion of gammaglobulins was given two months later. Response was satisfactory with motor, sensory and autonomic dysfunction recovery. A third course of immunoglobulins 28 days later produced little benefit. At the present time, 5 months after the onset of the disease, flaccid paralysis of the lower limbs and the distal portion of the upper limbs persists but autonomic instability has disappeared. The patient has been decannulated and is following an ambulatory rehabilitation program. Although repeated intravenous immunoglobulins may be useful in the treatment of prolonged GBS, the key issue is excellent intensive care unit management. Further research examining potentially more effective treatment such as beta-interferon and immunosuppressive agents is required.

[Renal function and functional renal reserve in adolescents who acquired minimal change nephrotic syndrome in childhood]. / Función renal y reserva funcional renal en jóvenes que padecieron síndrome nefrótico a cambios mínimos en la infancia.

OBJECTIVE: The aim of this study was to evaluate glomerular filtration rate (GFR) and renal functional reserve (RR) in young patients after diagnosis of minimal change nephrotic syndrome (MCNS) during childhood. PATIENTS AND METHODS: GFR and RR were evaluated in 15 young patients (10 female) diagnosis of childhood MCNS 18.5 +/- 4 years before. Creatinine clearance (CC) was measured before and after an acute protein load to determine GFR and RR. Based on the tendency towards relapses, the study subjects were divided into two groups: Group A had less than five relapses and group B five or more relapses. Study subjects (groups A and B) and control subjects (group C) were matched for sex and age. RESULTS: Group B showed a higher GFR than groups A and C (group B = 133.9 +/- 16.26 ml/min/1.73 m2, group A = 107.91 +/- 18.19 ml/min/1.73m2; p = 0.014, group C = 113.89 +/- 13.17 ml/min/1.73m2, p = 0.015). RR was significantly lower (absolute and relative) in group B than in group C (group B = 10.9 +/- 15.46 ml/min/1.73m2, group C = 38.58 +/- 21.47 ml/min/1.73m2, p = 0.016 and group B = 8.56 +/- 11.75%, group C = 34.35 +/- 21.43%, p = 0.016, respectively). CONCLUSIONS: After childhood MCNS, young patients who presented more than five relapses showed an increase in GFR and a decrease in RR.

[Blood pressure in childhood and adolescence. Its relationship with growth and maturation variables]. / Presión arterial en la infancia y la adolescencia. Estudio de su relación con variables de crecimiento y maduración.

AIM: To analyze the relationship between blood pressure in childhood and adolescence and different variables including gender, age, weight, height, body mass index and sexual maturation. METHODS: Cross-sectional study conducted in 1278 boys and girls aged 6 to 18 years old. Two blood pressure measurements were obtained. Mean value in the two measurements was obtained for each of the pressures: systolic (SBP), diastolic IV (DBPIV) and diastolic V (DBPV). Simple and multiple regression analysis were done to evaluate association between variables. RESULTS: 14 years 0.33; SBP boys > 14 years 0.34; DBPIV 0.25; DBPV 0.24) and height (SBP 6-13 years 0.33; SBP girls > 14 years 0.23; SBP boys > 14 years 0.29; DBPIV 0.25; DBPV 0.24) than for age (SBP 6-13 years 0.27; SBP girls > 14 years 0.17; SBP boys > 14 years 0.15; DBPIV 0.23; DBPV 0.22) for each of the blood pressures. Multiple regression analysis demonstrated a significant association between body mass index, height and SBP in the group of boys and girls aged 6-13 years old. No other significant associations were obtained. CONCLUSIONS: Blood pressure values in childhood and adolescence show a positive association with growth and maturation variables. It's of capital importance to considerate not only age, but also height, weight and body mass index when evaluating the blood pressure value of a child.