El síndrome de Costello a propósito de un caso clínico / Costello syndrome based on one clinical case

El síndrome de Costello ha sido descrito en 115 pacientes hasta la fecha. Reúne una serie de características fenotípicas que sugieren el diagnóstico ya que, en el momento actual, no existe ningún estudio que lo confirme. El interés de este caso radica en el reconocimiento de este síndrome debido al importante incremento de riesgo tumoral que presentan estos pacientes y a que, por tanto, precisan ser sometidos a un protocolo de despistaje de neoplastias: ecografía abdomino-pélvica desde el nacimiento y cada 3-6 meses hasta 8-10 años con objeto de detectar rabdomiosarcoma; determinación de catecolaminas en orina cada 6-12 meses y hasta los 5 años de vida para objetivar neuroblastoma y búsqueda de hematuria por su relación con la presentaciónde cáncer de vejiga (AU)

Costello Síndrome has been described in 115 patients. It consists in different clinical data that suggest the diagnosis. It is important to recognize this syndrome early because these children have an important tumoral risk so they should undergo different tests in order to find neoplasias: an abdo-pelvic ECO should be done from birth till 8-10 years of age every months to look for rhabdomyosarcoma, urinary catecholamine excretion measurements every 6-12 months up the age of 5 years to find neuroblastoma, and annual screening for hematuria from the age of ten onwards because it is related with the bladder cancer (AU)

[Interferon alpha and infantile orbital hemangioma]. / Interferón-alfa y hemangiomas orbitarios infantiles.

OBJECTIVE: To determine the efficacy and safety of interferon alpha therapy for the treatment of infantile periorbital hemangiomas associated with visual impairment. METHODS: Retrospective study of 4 patients diagnosed of orbital hemangioma during infancy. All of them were treated with interferon alpha 2b at doses of 3 million U/m2, administered once daily by subcutaneous injection, at least during 3.5 months. The lesions were compared through clinical evaluation and diagnostic imaging (echography, CT and MRI) before, during and after treatment, estimating the percentage of lesion reduction in relation to the initial size. RESULTS: In 3 cases, a 95% volume reduction was detected, and greater than 50% in the other one. Spastic diplegia was detected in a child during treatment, but resolved without persistent neurological deficits when discontinued. Alterations in plasmatic lipid profile were detected in one patient. CONCLUSION: Interferon alpha can be a valuable treatment for periorbital hemangiomas of infancy; even though, adverse effects can be clinically significant.

Interferón-alfa y hemangiomas orbitarios infantiles / Interferon alpha and infantile orbital hemangioma

Objetivo: Estudiar la eficacia y seguridad de la terapia con interferón-alfa en el tratamiento de los hemangiomas infantiles con afectación orbitopalpebral y compromiso visual. Métodos: Estudio retrospectivo de 4 pacientes, diagnosticados de hemangioma orbitario en la infancia. Se administró interferón-alfa 2b a la dosis de 3 millones U/m2 de superficie corporal, en inyección subcutánea diaria, durante un período de tiempo mínimo de 3,5 meses. Se compararon las lesiones a través de la exploración clínica y por métodos de imagen (ecografía, TAC, RNM) antes, durante y después del tratamiento, estimándose el porcentaje de reducción de la lesión con respecto al tamaño inicial de la misma. Resultados: En 3 casos se produjo una reducción del 95 por ciento de la lesión y en el restante ésta fue de más del 50 por ciento. En un caso se detectó diplejia espástica durante el tratamiento que, al suspenderlo, revirtió sin dejar secuelas neurológicas permanentes. En un caso detectamos alteraciones en el perfil lipídico con elevación de las cifras de colesterol y, principalmente, de los triglicéridos. Conclusiones: El interferón alfa puede constituir una alternativa terapéutica eficaz para el tratamiento de los hemangiomas infantiles con participación orbitopalpebral y, compromiso visual, aunque los efectos secundarios pueden ser importantes (AU)

Ranitidine prophylaxis in acute gastric mucosal damage in critically ill pediatric patients.

We determined the ranitidine dosage necessary to maintain gastric pH at or above 4 in 40 critically ill children. The patients were divided into four groups of ten patients each. They were treated with ranitidine in the following dosages: a) 2 mg/kg by NG tube every 12 h; b) 4 mg/kg by NG tube every 12 h; c) 0.75 mg/kg iv every 6 h; d) 1.5 mg/kg iv every 6 h. The fourth group had a higher median pH than the other groups, in spite of also having the highest risk of acute gastric mucosal damage (AGMD). Eight (80%) of ten patients in the fourth group had a pH greater than or equal to 4 or more than 80% of the study period. We recommend 1.5 mg/kg iv every 6 h for gastric acid inhibition in AGMD prophylaxis in children.

[Catecholamines in pediatric intensive care]. / Catecolaminas en patología intensiva pediátrica.

IV catecholamines are a very important therapy in the Pediatric ICU. Their application is very discussed, principally in the pediatric age, where effects, beginning by a remembrance of its physiology (endogenous production, storing, destruction, with an explanation of the adrenergic system, according with Ahlquist). Pharmacologic activity and clinical characteristics of their application and basic requirements for its IV use (monitorization, exact dosification, permeable venous way) are revised. Authors analyse independently the three catecholamines of more common use: isoproterenol, dopamine and dobutamine, discussing systematically its' actions on three levels: a) heart; b) systemic circulation, and c) pulmonary circulation. They discuss their indications, contraindications, risks and precaution in their use and dosification concluding that the more common catecholamines are dopamine and dobutamine, being the last one more recommended in the future, by its' more selective effects on the heart. Isoproterenol has very specific indications, in acute phases and during short periods of time. Adrenaline and noradrenaline are also considered. Finally, association of different drugs looking for improvement of positive effects and reduction of negative ones is mentioned.