[Beckwith-Wiedemann syndrome: study of eleven cases]. / Síndrome de Beckwith-Wiedemann: estudio de once casos.

In this paper we report a review of the omphalic pathology that was admitted to our hospital from january 1973 through december 1990. Eleven cases of Beckwith-Wiedemann Syndrome were diagnosed during this period of time. Our eleven cases comply with at least three of the four major criteria (omphalocele, macroglossia, gigantism and neonatal hypoglycemia) and several of the minor criteria. The interest of this paper is based on the rareness of this syndrome and its association in two of our cases with Cacchi-Ricci Syndrome. Moreover, one of these cases later presented with a Wilms' tumor. Finally, we emphasize the importance of an early diagnosis of this syndrome in order to avoid the metabolic disturbances (hypoglycemia), to establish prompt treatment of the serious anomalies (omphalocele) and to control and follow these patients since they have increased risk to develop neoplastic disease.

[Lymphoblastic non-Hodgkin's lymphoma: estimation of the survival of patients with the disseminated form of the disease (stages III and IV) treated by the LSA2-L2 protocol]. / Linfoma no Hodgkin de tipo linfoblástico: evaluación de la supervivencia en pacientes con enfermedad diseminada (estadío III y IV) tratados con el protocolo LSA2-L2.

We analyze the survival rate in 17 pediatric patients (twelve males and five females) that were diagnoses of non-Hodgkin's lymphoblastic lymphoma (cases with Burkitt's lymphoma were excluded) during the period from 1982 to 1990. All of them were treated using the LSA2-L2 therapeutic protocol. The follow-up period ranged from 14 to 99 months (follow-up median 43 months). Staging of our newly diagnosed patients, we found 9 patients in stage III and 8 in stage IV disease. Survival was analyzed by the Kaplan-Meier method. The overall survival rate of our patients is 69.5% and the disease free survival is 62.6%. The disease free survival for separated stages is 66.5% for stage III and 58.3% for stage IV. We compare our results with other casuisties published in literature.