Derrame pericárdico masivo tras infección por Neisseria meningitidis / Massive pericardial effussion after a Neisseria meningitidis infection

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Alcalosis metabólica de origen dietético en un lactante / Infant metabolic alkalosis of dietetic origin

La alcalosis metabólica (AlcM) puede ser sensible o resistente al tratamiento con cloruro sódico en función de su respuesta a éste. La AlcM resistente presenta elevada excreción urinaria de cloro y es secundaria a tubulopatías o al uso de diuréticos; la AlcM sensible incluye vómitos, fibrosis quística y déficit de ingesta. A continuación se presentan los casos de 2 lactantes alimentadas con leche de almendras que presentaron deshidratación y fallo de medro. En ambos casos se observó AlcM hipoclorémica e hipopotasémica, que respondió satisfactoriamente a la reposición hidroelectrolítica. Tras descartar vómitos, ingesta de fármacos, tubulopatías y fibrosis quística se llegó al diagnóstico de déficit en la ingesta debido al escaso aporte de cationes sodio (Na+) y aniones cloro (Cl−) que proporciona la leche de almendras. Este déficit induce un incremento de la reabsorción tubular proximal del anión bicarbonato, y en las porciones distales de la nefrona induce un aumento de la reabsorción de Na+ y Cl−, que se intercambian con cationes potasio y cationes hidrógeno, lo que favorece la aparición de alcalosis hipoclorémica e hipopotasémica. Asimismo, en uno de los casos se constató hipotiroidismo secundario (aparentemente debido al nulo aporte de yodo de la leche de almendras) y trastorno de la mielinización caracterizado por neuritis óptica e hipoacusia. Estos trastornos se resolvieron al administrar una alimentación con fórmula humanizada (AU)

Cases of metabolic alkalosis are divided into susceptible or resistant to treatment with sodium chloride, depending on the response to it. The resistant cases present with high urinary excretion of chloride, and are secondary to tubular disease or use of diuretics. Included among the sensitive cases are, vomiting, cystic fibrosis and low intake. Two infants were fed with “almond milk” and showed clinical symptoms of dehydration and failure to thrive. Hypochloraemic and hypokalaemic metabolic alkasosis was seen in both cases, which responded satisfactorily to water and electrolyte replacement. After ruling out vomiting, ingestion of drugs, tubular disease, and cystic fibrosis, the diagnosis was low intake, due to poor contribution of Na+ and Cl− provided by the “almond milk”. This deficit induces an increase in proximal tubular reabsorption of H−CO3 and in parts of the distal nephron, an increase in reabsorption of Na+ and Cl− which are exchanged with K+ and H+, which can give rise to a hypochloraemic alkalosis and hypokalaemia. Secondary hypothyroidism was found in one case, apparently due to the lack of iodine in the almond milk, and disorders of the myelination characterized by optic neuritis and hearing loss. These disorders were resolved when feeding with human formula was established (AU)

[Infant metabolic alkalosis of dietetic origin]. / Alcalosis metabólica de origen dietético en un lactante.

Cases of metabolic alkalosis are divided into susceptible or resistant to treatment with sodium chloride, depending on the response to it. The resistant cases present with high urinary excretion of chloride, and are secondary to tubular disease or use of diuretics. Included among the sensitive cases are, vomiting, cystic fibrosis and low intake. Two infants were fed with "almond milk" and showed clinical symptoms of dehydration and failure to thrive. Hypochloraemic and hypokalaemic metabolic alkasosis was seen in both cases, which responded satisfactorily to water and electrolyte replacement. After ruling out vomiting, ingestion of drugs, tubular disease, and cystic fibrosis, the diagnosis was low intake, due to poor contribution of Na+ and Cl(-) provided by the "almond milk". This deficit induces an increase in proximal tubular reabsorption of H(-)CO3 and in parts of the distal nephron, an increase in reabsorption of Na+ and Cl(-) which are exchanged with K+ and H+, which can give rise to a hypochloraemic alkalosis and hypokalaemia. Secondary hypothyroidism was found in one case, apparently due to the lack of iodine in the almond milk, and disorders of the myelination characterized by optic neuritis and hearing loss. These disorders were resolved when feeding with human formula was established.

[Posterior urethral valves, unilateral vesicoureteral reflux and renal dysplasia (VURD syndrome). Long-term follow-up of kidney function]. / Válvulas de uretra posterior, reflujo vesicoureteral unilateral y displasia renal (síndrome VURD). Seguimiento de la función renal a largo plazo.

Posterior urethral valves, unilateral vesicoureteral reflux and renal dysplasia (VURD syndrome) is an infrequent entity in childhood that has provoked multiple controversies. The shortage of studies that evaluate the long-term outcome in these children prompted up to write the present article. Three patients that met strict criteria for a diagnosis of VURD syndrome were retrospectively reviewed, with special emphasis on several indicators of renal function in these patients at diagnosis and in adulthood. The three patients currently have normal renal function, unlike a large percentage of patients diagnosed with posterior urethral valves with vesicoureteral bilateral reflux. Although the sample is small, our results support the hypothesis of good long-term renal function in affected children.

[Study of renal morphology and function in children with nocturnal enuresis. Relationship with sensitivity or resistance to desmopressin]. / Estudio de la morfología y la función renal en niños diagnosticados de enuresis nocturna. Su relación con la sensibilidad o la resistencia a la desmopresina.

BACKGROUND: In the last few years, there have been reports that children with nocturnal enuresis frequently have hypercalciuria. Likewise, children with desmopressin-resistant enuresis have recently been reported to have a higher renal concentration capacity than patients with desmopressin-sensitive enuresis. OBJECTIVES: To study renal function and urinary calcium excretion and to register familial history of enuresis and urolithiasis in a group of children with enuresis, whether responders or nonresponders to desmopressin, followed-up in our hospital. MATERIAL AND METHODS: A cohort of 60 patients (42 boys and 18 girls) who were referred to the hospital because of nocturnal enuresis. RESULTS: Hypercalciuria was detected in 26 children (43.3 %) and hypocitraturia in eight (13.3 %). The frequency of hypercalciuria was higher in desmopressin-resistant patients than in desmopressin-sensitive patients, but this difference was not statistically significant. Sonographic renal morphological anomalies were detected in 11 children (18.3 %). No differences in renal handling of water were detected when the patients were distributed according to the grade of sensitivity to desmopressin. CONCLUSIONS: In our cohort we found a high frequency of hypercalciuria in children with nocturnal enuresis. No differences were observed in maximal urinary osmolality among desmopressin-resistant and desmopressin-sensitive children.

Estudio de la morfología y la función renal en niños diagnosticados de enuresis nocturna. Su relación con la sensibilidad o la resistencia a la desmopresina / Study of renal morphology and function in children with nocturnal enuresis. Relationship with sensitivity or resistance to desmopressin

Antecedentes: En los últimos años, se ha descrito que, con frecuencia, los niños con enuresis nocturna presentan hipercalciuria. Así mismo, se acaba de comunicar que los niños con enuresis nocturna resistente a desmopresina tienen una capacidad de concentración renal superior a la de aquellos pacientes con enuresis sensible a ese fármaco. Objetivos: Estudiar la función renal y la eliminación urinaria de calcio y registrar los antecedentes familiares de enuresis y de litiasis en un grupo de niños enuréticos respondedores y resistentes a desmopresina controlados en nuestro hospital. Material y métodos: Cohorte de 60 pacientes, 42 varones y 18 mujeres, que fueron remitidos al hospital por padecer enuresis nocturna. Resultados: Se detectó hipercalciuria en 26 niños (43,3 %) e hipocitraturia en ocho (13,3 %). La frecuencia de hipercalciuria fue superior en los pacientes resistentes con respecto a los sensibles a desmopresina, sin diferencias estadísticamente significativas. En 11 niños se detectaron anomalías morfológicas ecográficas renales. No se comprobaron diferencias en el manejo renal del agua al distribuir a los pacientes según el grado de sensibilidad a la desmopresina. Conclusiones: En nuestra serie se demuestra la elevada frecuencia de hipercalciuria en niños con enuresis nocturna. No observamos diferencias en la osmolalidad urinaria máxima entre los niños sensibles y los resistentes a la desmopresina

Background: In the last few years, there have been reports that children with nocturnal enuresis frequently have hypercalciuria. Likewise, children with desmopressin-resistant enuresis have recently been reported to have a higher renal concentration capacity than patients with desmopressin-sensitive enuresis. Objectives: To study renal function and urinary calcium excretion and to register familial history of enuresis and urolithiasis in a group of children with enuresis, whether responders or nonresponders to desmopressin, followed-up in our hospital. Material and methods: A cohort of 60 patients (42 boys and 18 girls) who were referred to the hospital because of nocturnal enuresis. Results: Hypercalciuria was detected in 26 children (43.3 %) and hypocitraturia in eight (13.3 %). The frequency of hypercalciuria was higher in desmopressin-resistant patients than in desmopressin-sensitive patients, but this difference was not statistically significant. Sonographic renal morphological anomalies were detected in 11 children (18.3 %). No differences in renal handling of water were detected when the patients were distributed according to the grade of sensitivity to desmopressin. Conclusions: In our cohort we found a high frequency of hypercalciuria in children with nocturnal enuresis. No differences were observed in maximal urinary osmolality among desmopressin-resistant and desmopressin-sensitive children

Comparación de la desmopresina intranasal y oral en la determinación de la capacidad de concentración renal en la infancia / Determination of the renal concentrating ability in childhood: intranasal desmopressin versus oral desmopressin

Antecedentes: En los últimos años, se ha descrito que uno de los parámetros más sensibles para estimar la función renal es la prueba de concentración urinaria realizada con estímulo de desmopresina (DDAVP). Objetivo: Estudiar la capacidad de concentración urinaria en un grupo de pacientes controlados en nuestro hospital comparando los resultados de la osmolalidad urinaria máxima obtenida con desmopresina administrada tanto por vía oral como nasal. Material y métodos: Cohorte de 53 pacientes (30 niños y 32 niñas) que están en seguimiento en las consultas externas de Nefrología Pediátrica. Resultados: La osmolalidad urinaria máxima obtenida con desmopresina intranasal fue 726,4 ± 163,8 mOsm/kg y con desmopresina oral 782,1 ± 198,5 mOsm/kg (p = 0,003). Se comprobó una correlación directa entre ambas variables (r = 0,75; p < 0,001). En 40 niños (75,5%) coincidían los valores de osmolalidad urinaria máxima, es decir, el que concentraba con un método lo hacía con el otro y a la inversa. 32 de ellos (80%) presentaban defecto de concentración urinaria. En 13 pacientes (24,5%) los valores no fueron coincidentes; todos ellos concentraban adecuadamente con uno u otro método, pero en 12 (92,3%) el valor máximo se alcanzó con desmopresina vía oral. Conclusiones: Los hallazgos más significativos de este estudio radican en el hecho de que si existe defecto de concentración urinaria, éste se constata independientemente de la vía de administración de la desmopresina. En aquellos casos en los que no existió coincidencia entre ambas determinaciones, la vía oral es la que valida la prueba

Background: In the last few years, it has been described that one of the best renal function parameters is the renal concentrating test with desmopressin. Objective: The aim of the present study was the evaluation of the renal function comparing the results of the urine concentration test with desmopressin administrated by oral or intranasal way in a group of children followed up in our hospital. Material and methods:Aseries of 53 patients (30 males and 23 females) who consulted at the hospital. Results: The maximum urinary osmolality was 726,4 ± 163,8 mOsm/kg with desmopressin administrated by intranasal way and 782,1 ± 198,5 mOsm/kg (p = 0,003) with desmopressin administrated by oral way. It was observed a direct correlation between both parameters (r = 0,75; p < 0,001). In 40 children (75,5%) were coinciding the maximum urinary osmolality, that is, the one that he was concentrating with a method did it with the other and the other way around. 32 of them (80%) had a defect in the urinary concentration test. In 13 patients (24,5%) the values were not coincident; all of them presented a normal urinary concentration test but in 12 the maximum value was obtained with oral desmopressin. Conclusions: The most meaning results of this study are that if there is a defect in the renal concentrating capacity, this will be present independently of the desmopressin administration method. On the other hand, in those cases where there are not coincidence between both determinations, the oral way is the reference method

Ectasia de la pelvis renal en la infancia. ¿Sabemos ya lo que significa y cómo debe estudiarse? / Pelvic ectasia in chilhood. Do we know what it means and how it should be investigated?

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Enfermedad por arañazo de gato con afectación hepatoesplénica / Cat scratch disease with hepatosplenic involvement

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