Francisco Barriga C

Leucoencefalopatía reversible posterior: cuatro casos clínicos

Reversible posterior leukoencephalopathy syndrome (PLS) ischaracterized by headache, clouding of sensorium, visual disturbances and seizures. It isassociated to hypertension, renal disease or immunosuppressive therapy. We report three males,aged 9, 12 and 16 years and one female, aged 5 years wih PLS associated toimmunosuppressive therapy. All had seizures and three had headache and clouding ofsensorium. One case was associated to an hypertensive emergency, one to liver failure and oneto high tacrolimus levels. Magnetic resonance imaging showed lesions in the white matter intwo patients and in the gray matter in the other two. The lapse between the start ofimmunosuppressive treatment and neurological symptoms ranged from 4 days to 6 months. Allreceived antiepileptic drugs and immunosuppresive therapy was changed or decreased, withcomplete clinical recovery (Rev Med Chile 2008, 136: 93-8).(

Tratamiento de las infecciones asociadas a catéteres venosos centrales

El tratamiento de las infecciones del torrentesanguineo (ITS) asociadas a cateteres venososcentrales (CVC) esta basado en su mayor parteen series clinicas, recomendaciones de expertos,guias clinicas y un reducido numero de estudioscaso-control. No se dispone lamentablemente deensayos clinicos randomizados que permitan efec-tuar recomendaciones solidas para el manejo deestas complicaciones. Por ello, la calidad de laevidencia corresponde mayoritariamente a unade tipo III.La metodologia utilizada para analizar este temaconsistio en el analisis de las principales pregun-tas que enfrentan los clinicos cuando sospechano confirman una ITS asociada a CVC. Lasinterrogantes que el grupo estimo necesario res-ponder se presentan en la Tabla 1.

Pancreatitis aguda grave y pseudoquiste pancreático por uso de drogas en niños: Presentación de tres casos clínicos y revisión de la literatura

Acute severe pancreatitis associated with drugs is an uncommon disorder in children but its incidence is increasing with more extensive use of certain drugs. We present three children with severe drug induced pancreatitis caused by valproic acid and L-asparaginase who developed pancreatic pseudocysts. The children required medical supportive therapy in the Intensive Case Unit and one needed surgical intervention for the pseudocyst. National and international reports were reviewed. The aetiology, clinical presentation, diagnostic methods and current management of acute severe pancreatitis and pseudocysts in children are discussed.

Bronquiolitis obliterante con neumonía en organización secundaria a quimioterapia, en un niño con sarcoma pericárdico primario

The uncommon bronchiolitis obliterans organizing pneumonia can be idiopathic or caused by infection or medications. We report a 5 year old boy with pericardial sarcoma that was treated with chemotherapy (vincristine, doxorubicin, etoposide, cyclophosphamide) and radiotherapy. During the third cycle of chemotherapy, he developed progressive cough and dyspnea, needing oxygen therapy. Chest X-ray examination showed bilteral infiltrates. After discarding infectious etiologies, an open lung biopsy was performed, and the pathological examination showed a bronchiolitis obliterans organizing pneumonia. He was treated with prednisolone for 7 days, followed by prednisone for additional 45 days. He continued with fluticasone propionate as maintenance and remains in good condition with a ches X ray showing mild interstitial images, after seven months of follow up. (Rev Med Chile 2000; 128: 633-40).

Infección diseminada por adenovirus de curso fatal en un niño inmunocomprometido

Las infecciones graves por adenovirus (ADV) tienen una importancia creciente en pacientes inmuno-comprometidos, en especial en ninos sometidos a trasplante de precursores hematopoyeticos (TPH). Se reporta un caso de infeccion por ADV inicialmente gastrointestinal y luego diseminada, de curso fatal, en un nino de 12 anos, post LPH. El diagnostico se confirmo mediante aislamiento viral y deteccion de genoma viral en tejidos y sangre. Se revisan los principales aspectos de la infeccion por ADV, su diagnostico y las posibilidades terapeuticas. Este caso debe alertar a los medicos clinicos para sospechar y estudiar este agente viral en pacientes de alto riesgo y enfatiza la importancia de disponer en Chile de antivirales para su tratamiento

Manejo multidisciplinario de tumores hepáticos malignos en el niño: una experiencia nacional reciente

Background: Malignant hepatic tumors (Mht) are rare in children. Among them hepatoblastoma (HB) is the most common. Aim: To report the results of the multidisciplinary management in 6 consecutive children: five HB and one metastatic Wilms tumor (MWT). Patients and methods: The mean age of patients was 42 months. All HB patients had elevated serum alfafetoprotein (median 150,000 ng/ml). All patients received preoperative chemotherapy: HB patients received carboplatin/doxorrubicin alternating with cisplatin, and the MWT patient, vincristine alone. Surgery included two formal right and two formal left hepatectomies, one extensive central resection with partial left segmentectomy, and one lateral segmentectomy. Extracorporeal circulation was used in the child with atrial involvement. All patients received postoperative chemotherapy. Results: All tumors had variable regresion on preoperative chemotherapy. Complete resection with negative margins was achieved in all patients. The degree of tumor necrosis on histology ranged from 60% to 90%. Alfafetoprotein levels fell to under 10 ng/ml in all HB cases, one to three months after surgery. All patients survive free of disease at a median follow up of 19 months. Conclusion: A multidisciplinary approach including the well timed used of chemotherapy and surgery is highly effective in the management of pediatric malignant tumors.

Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich

Wiskott-Aldrich syndrome (WAS) is an X linkedcongenital disease that presents as eczema, thrombocytopenia and immune deficiency. Theonly curative procedure for this illness is hematopoietic stem cell transplant (HSCT),preferably from a healthy HLA identical sibling donor. Cord blood is becoming an excellentalternative as stem cell source from unrelated donors.

Progresos en el tratamiento y seguimiento de hepatoblastoma: Análisis de una serie de 9 niños

Resumen Introduccion: El hepatoblastoma es el tumor hepatico maligno mas frecuente del nino. La extirpacion radical del tumor es el elemento central para lograr su curacion. Objetivo: Evaluar el seguimiento y sobrevida delineando las caracteristicas clinicas, biodemograficas y terapeuticas de pacientes portadores de hepatoblastoma. Pacientes y metodo: Analisis retrospectivo de 9 ninos tratados por hepatoblastoma entre mayo 1997 y diciembre 2000. Se analizan la quimioterapia preoperatoria, tecnicas quirurgicas, anatomia patologica, nivel de alfafetoproteina y curva antropometrica en ninos con hepatoblastoma. Se uso la clasificacion SIOPEL 1 (Liver Tumour Study Group of the International Society of Paediatric Oncology). Resultados: La edad media al diagnostico fue de 24 meses, todos con una masa abdominal palpable y niveles de alfafetoproteina aumentados. Todos recibieron quimioterapia preoperatoria y cirugia hepatica radical tumoral. La anatomia patologica confirmo margenes libres. No hubo mortalidad perioperatoria. Una paciente presento recidiva tumoral a los 9 meses, falleciendo 6 meses despues. La sobrevida libre de enfermedad fue de 89% a tres anos. El desarrollo ponderal retorno a percentiles previos a su enfermedad. Conclusiones: El manejo multidisciplinario, planificacion temprana, entrenamiento quirurgico hepatico especializado son los pilares para el exito en esta patologia compleja

Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos

Severe acquired aplastic anemia (SAA) is an uncommon disease of childhood. Patients with SAA receive supportive care with transfusions and timely treatment of opportunistic infections, along with specific therapies, which may be allogenic stem cell transplantation (SCT) from a matched sibling or immunosupressive therapy (IT). Aim: To report the experience in the management of SAA. Patients and methods: Twenty five children with acquired SAA were treated from July 1992 to September 2005. Patients with full matched sibling donors received allogenic SCT after conditioning with a cyclophosphamide containing regimen. The other patients received immune suppression with cyclosporine plus methylprednisolone (n= 18) plus ATG (n=17). All received supportive care until recovery of hematopoietic function. Those who had severe opportunistic infections at diagnosis or did not respond to two cycles of ATG were evaluated for unrelated donor SCT. Results: Seven patients received sibling donor SCT and 18 IT, which was repeated in six. Three patients received mismatched related (1) or unrelated (2) SCT. Nineteen patients survived with a median follow up time of 4 years, 14 with full hematologic recovery. Six patients died: four due to infections after IT or SCT, one due to intracranial hemorrhage and one with secondary myelodysplasia 12 years after IT. Conclusions: Most children with SAA can be treated successfully with sibling donor SCT or IT. Patients without a histocompatible sibling who fail to respond to IS have a worse prognosis