Incremento de la incidencia de los derrames pleurales en el Área de Salud de Albacete: Revisión de 12 años / Increase in the incidence of pleural effusion in the Health Care Area of Albacete: A 12 year review

Introducción. En los últimos años se ha constatado un aumento en la incidencia de derrames pleurales (DP) para-neumónicos. El objetivo del estudio es conocer las características epidemiológicas, clínicas y microbiológicas de los derrames, su incidencia y los cambios producidos desde la introducción de la vacuna antineumocócica. Material y métodos. Estudio retrospectivo y descriptivo de pacientes ingresados por DP en el Servicio de Pediatría del Complejo Hospitalario Universitario de Albacete desde enero de 1996 hasta diciembre de 2007. Resultados. Grupo de estudio: 71 pacientes. La incidencia anual de DP ha aumentado desde el año 2000, con una incidencia máxima de 24 caos por 100.000 niños menores de 14 años del Área de Albacete. El 72,4% de los niños no habían recibido la vacuna antineumocócica. Se practicó 23 (32,3%). Se identificó el microorganismo responsable en 19 casos (26,7%). 13 fueron por Neumococo (18,5%). El 57% de los neumococos fueron sensibles a penicilina. 15 casos (21,1%) precisaron tubo de drenaje pleural y un 22,5% (16 casos) toracoscopia. Conclusiones. Desde el año 2000 observamos un aumento de la incidencia global de los DP y de los derrames por Neumococo. Las características clínicas de nuestros pacientes son similares a las descritas en otras publicaciones. La rentabilidad de los cultivos y la resistencia de Neumococo a la penicilina han sido bajas (AU)

Introduction. An increased incidence of parapneumonic pleural effusions has recently been reported. The aim of this study is to identify the epidemiological, clinical and microbiological characteristics of this illness, to determine the impact of the antipneumococcal vaccine and any changes occurred since its introduction. Material and methods. Retrospective analysis of patients hospitalized due to a pleural effusion in the Department of Paediatrics of the Universitary Hospital of Albacete form January 1996 to December 2007. Results. Study group: 71 patients. The anual incidence of pleural effusions has increased since 2000, with a peak incidence of 24 cases per 100,000 children under 14 years old in the Area of Albacete. 72,4% of children had not received the pneumococcal vaccine. Thoracocentesis was performed in 56,3%. 23 cases fulfilled criteria for empyema (32.3%). The involved microorganism was isolated in 19 case (26.7%), 13 were Pneumococcus (18.5%). 57% of Pneumococci were sensitive to Penicilli. 15 children (21.1%) needed a pleural tube, 16 (22.5%) a thoracoscopy. Conclusins. We observed and increase in the overall incidence of pleural effusions since 200, most of them due to Pneumococcus. The clinical features of our patients are similar to others reported in the literature. The sensitivity of microbiological cultures is low as well as the Pneumococcus resitance to penicillin (AU)

[Hemophagocytic syndrome secondary to visceral leishmaniasis]. / Síndrome hemofagocítico secundario a leishmaniasis visceral.

Haemophagocytic syndrome is a disease diagnosed according to clinical and analytical criteria, related to many infectious diseases. It is exceptionally described in patients infected with Leishmania. Visceral leishmaniasis is an uncommon disease in our country except in some areas where it is endemic. Its diagnosis is sometimes difficult and the use of other methods currently available is needed. Haemophagocytic syndrome treatment is based on established chemotherapy protocols, but when it is secondary to Visceral Leishmaniasis, it may be an exception, since the abnormalities can be resolved by treatment of the infection itself. This treatment has improved recently as Liposomal Amphotericin B has replaced classic antimonials, being more beneficial due to less adverse effects and a shorter treatment time.

Síndrome hemofagocítico secundario a leishmaniasis visceral / Hemophagocytic syndrome secondary to visceral leishmaniasis

El síndrome hemofagocítico es una enfermedad diagnosticada basándose en criterios clínicos y analíticos, relacionada con numerosas entidades infecciosas. De forma excepcional se ha descrito en pacientes infectados con el parásito Leishmania. La leishmaniasis visceral es una patología infrecuente en nuestro país, salvo en zonas concretas donde es endémica. Su diagnóstico en ocasiones es difícil y hay que recurrir a varios de los métodos actualmente disponibles. El tratamiento del síndrome hemofagocítico se fundamenta en pautas quimioterápicas protocolizadas, aunque puede representar una excepción cuando es secundario a la leishmaniasis visceral, ya que el tratamiento antiinfeccioso suele resolver las alteraciones por sí mismo. Dicha terapia ha evolucionado en los últimos tiempos al sustituir la anfotericina B liposomal a las pautas clásicas con antimoniales y logrando beneficios por sus menores efectos secundarios y por acortar el tiempo de tratamiento (AU)

Haemophagocytic syndrome is a disease diagnosed according to clinical and analytical criteria, related to many infectious diseases. It is exceptionally described in patients infected with Leishmania. Visceral leishmaniasis is an uncommon disease in our country except in some areas where it is endemic. Its diagnosis is sometimes difficult and the use of other methods currently available is needed. Haemophagocytic syndrome treatment is based on established chemotherapy protocols, but when it is secondary to Visceral Leishmaniasis, it may be an exception, since the abnormalities can be resolved by treatment of the infection itself. This treatment has improved recently as Liposomal Amphotericin B has replaced classic antimonials, being more beneficial due to less adverse effects and a shorter treatment time (AU)

Tumores de parótida en niños / Parotid gland´s tumors in children

Los tumores de glándulas salivares son infrecuentes en niños, afectando el 80% a la parótida y presentando una mayor proporción de malignidad que en adultos. Revisamos 8 casos en menores de 15 años tratados en nuestro hospital, atendiendo a sus antecedentes, expresividad clínica, tipo tumoral, diagnóstico, tratamiento empleado y seguimiento. Todos debutaron con masa parotídea asintomática y de crecimiento lento a una edad entre 10 meses y 14 años. Cuatro fueron adenomas pleomorfos, dos angiomas capilares, un quiste epidérmico y un carcinomamioepitelial. Destacamos la excepcionalidad del carcinoma por su rareza y por el alto grado de malignidad expresado. Se realizó punción-aspiración con aguja fina en cuatro pacientes, siendo concluyente sólo en tres. En todos se practicó tumorectomía, excepto en el carcinoma mioepitelialy en uno de los adenomas pleomorfos recidivado, en los que se realizó parotidectomía total. Los tumores malignos de la parótida son clínicamente indistinguibles de los benignos, por lo que ante toda masa palpable en la zona parotídea deberíamos establecer un diagnóstico sin demora. El tratamiento de elección es la exéresis quirúrgica con márgenes amplios, siendo otros tratamientos coadyuvantes poco útiles en niños (AU)

The tumors of the salivary glands are infrequent in children, and parotid gland is involved in 80% of them. When a salivary gland tumor is present, the chance of malignancy is greater in the child than in the adult. We reviewed 8 cases identified in patients aged 14 years and younger in our hospital, analyzing its antecedents, signs and symptoms, histological features, diagnosis, treatment and evolution. All the patients displayed preauricular painless, non-inflammatory and slow-growing masses to an age between 10 months and 14 years. Four of them were pleomorphic adenomas, two haemangiomas, one epidermal cysts and onemyoepithelial carcinoma. We emphasize the exceptional nature of the carcinoma for its rareness and for the high degree of malignancy expressed. We made a fine needle aspiration biopsy in four cases but they were conclusive only in three. All were treated by surgical resection of the tumour except for the myoepithelial carcinoma and the recurrent pleomorphic adenoma that were treated by total parotidectomy. The malignant tumours of the parotid gland are clinically indistinguishable of the benign ones, thus when any palpable mass appears in the zone of the parotid gland, an accurate diagnosis should be made without delay. The treatment of choice is the surgical excision with wide margins, being other adjuvant treatments less useful to this age that in the adult age (AU)

Ictericia colestásica como complicación de una pielonefritis aguda en una niña de 6 años / Cholestatic jaundice complicating acute pyelonephritis in a 6-year-old girl

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Hemangioma solitario intestinal y anemia crónica / Solitary intestinal hemangioma and chronic anemia

Los hemangiomas del tracto gastrointestinal son una causa rara pero importante de hemorragia digestiva, que se manifiesta bien por sangrado activo o bien por anemia ferropénica resistente al tratamiento. Su diagnóstico resulta con frecuencia difícil y la presencia de hemangiomas en otras localizaciones, habitualmente en la piel, hace sospechar su existencia. Presentamos el caso de una niña con anemia persistente secundaria a un hemangioma solitario en intestino delgado (AU)

[Megacalycosis diagnosed during the study of antenatal hydronephrosis]. / Megacaliosis diagnosticada en el estudio de una hidronefrosis prenatal.

We describe a case of megacalycosis in a newborn female with a probable antenatal ultrasonographic diagnosis of left multicystic dysplastic kidney. Uroradiology, ultrasonography and nuclear medicine studies were performed and diagnosis was established by excretory urogram. We have found only one previously published case of megacalycosis in which diagnosis was made during the postnatal study of antenatal hydronephrosis. Megacalycosis should be included in the differential diagnosis of prenatal hydronephrosis.

Megacaliosis diagnosticada en el estudio de una hidronefrosis prenatal / Congenital megacalycosis diagnosed during the study of antenatal hydronephrosis. Case report

Se describe un caso de megacaliosis en una recién nacida con diagnóstico prenatal de probable riñón multiquístico displásico izquierdo. Se practicaron estudios urorradiológicos, ecografía renal y renograma diurético, siendo la urografía excretora la exploración diagnóstica. En la revisión bibliográfica realizada tan sólo se ha encontrado un caso documentado de megacaliosis diagnosticada en el estudio posnatal de una hidronefrosis demostrada ecográficamente en período prenatal. La megacaliosis debe figurar en el diagnóstico diferencial de la hidronefrosis prenatal (AU)

[Phenotype heterogeneity in Gitelman's syndrome]. / Variabilidad fenotípica del síndrome de Gitelman.

A family with three offspring affected of Giltelman's syndrome is reported. The phenotypic variability of this entity is emphasized. Moreover, the diagnosis criteria of the syndrome, phathophysiology, and genetics and clinical differences with Bartter's syndrome are stated.

[Neurocutaneous melanosis]. / Melanosis neurocutánea.

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi and excessive proliferation of melanotic cells in the leptomeninges. We report the case of a girl with a giant hairy nevus and numerous small nevi since birth. Within the first 2 years of life she developed clinical features of increased intracranial pressure and West s syndrome. At 2 years of age she presented a right facial palsy and myelopathy. Brain and spinal magnetic resonance imaging demonstrated meningeal infiltration. Diagnosis of NCM was established by a detailed cytologic analyses of the cerebrospinal fluid that revealed melanocytic cells. She received palliative treatment. The girl died 2 months after. Patients with large or multiple congenital melanocytic nevi should be carefully followed up with clinical examination and neuroimaging to detect NCM. At present there is no curative treatment. The association of NCM and West s syndrome has not been previously described.

Melanosis neurocutánea / Neurocutaneous melanosis. Case report and review of the literature

La melanosis neurocutánea (MNC) es un síndrome congénito poco frecuente caracterizado por la asociación de nevus cutáneos pigmentados múltiples o de gran tamaño y una excesiva proliferación de células melánicas en leptomeninges. Comunicamos el caso de una niña que al nacimiento mostró un nevus piloso gigante y múltiples nevus de menor tamaño. Durante los dos primeros años de vida desarrolló clínica de hipertensión intracraneal y síndrome de West. A los 2 años de edad presentó una mielopatía compresiva y parálisis facial derecha. Una resonancia magnética craneospinal evidenció infiltración meníngea. La citología del líquido cefalorraquídeo reveló células melánicas con signos de malignidad. Una vez establecido el diagnóstico de MNC se inició tratamiento paliativo. La niña falleció dos meses después. En los pacientes con nevus pigmentados congénitos numerosos o gigantes debe considerarse el diagnóstico de MNC por lo que es preciso un seguimiento clínico y de neuroimagen. Hasta el momento esta entidad carece de tratamiento curativo. La asociación de MNC y síndrome de West no ha sido descrita previamente (AU)

Variabilidad fenotípica del síndrome de Gitelman / Phenotype heterogeneity of Gitelman's syndrome

Presentamos una fratria afecta de síndrome de Gitelman en la que se pone de manifiesto su variabilidad fenotípica. Así mismo se exponen los criterios diagnósticos de la entidad, su fisiopatología y las diferencias genéticas y clínicas con el síndrome de Bartter (AU)

Early neonatal cutaneous candidiasis.

A premature female presented cutaneous lesions caused by Candida albicans at 12 h after delivery. Her mother carried Candida in the vagina. The lesions extended to face, both sides of the trunk and extremities, including palms and soles, and later to the mouth. The cutaneous manifestations healed in four weeks. It is proposed to use the term "early neonatal cutaneous candidiasis" to designate the forms of candidiasis which appear within the first hours after delivery, and "congenital cutaneous candidiasis" when lesions are present at delivery.

[Serum theophylline levels after oral administration in a group of asthmatic children]. / Niveles séricos de teofilina tras administración oral en un grupo de niños asmáticos.

Serum levels of theophylline were determined in 54 asthmatics children aged 3,92 +/- 2,45 years, who were taken oral theophylline at doses of 4,99 +/- 0,45 mg. The serum levels at 0 hours (9,22 +/- 4,90 mcg/ml) and at 2 hours (15,22 +/- 5,5 mcg/ml) showed a great variation, although they were taken similar doses. Two hours after ingestion, 34 patients showed levels between 10-20 mcg/ml in 10 children were less than 10 mcg/ml, and another 10 shown serum levels over 20 mcg/ml without symptoms. There was a good correlation between levels at 0 and 2 hours (p less than 0,002). These data may support the utility of doses employed. It is advisable knowing the individual response to theophylline for managing episodes of asthma in children, that can be made by only one determination 2 hours after ingestion.