Archives francaises de pediatrie · 1990
[Neonatal Pepper's syndrome. Apropos of a series of 22 cases].
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Labreveux de Cervens C, Hartmann O, Valteau D, Brugières L, de Cervens T, Kalifa C, Patte C, Flamant F, Lemerle J
Аннотация
From 1960 to 1987, 22 neonates were treated at the Institut Gustave-Roussy for a Pepper' syndrome. During this period of time, 83 children were treated for the same syndrome, the neonatal type representing 26% of cases. Twelve of the 22 neonates presented with the rapidly evolving type, requiring an immediate aggressive treatment. In 4 of them death occurred early despite treatment. The 10 other neonates presented with a slowly progressive disease. In 5 of them treatment of the metastatic disease was not necessary to obtain remission. The survival rate of disease without relapses in 77%, with a median follow-up of 8 years. So, despite the high incidence of rapidly progressive forms, the prognosis of this disease remains favorable in neonates, provided an accurate treatment initiated early.