The outlook for children with rhabdomyosarcoma has change significantly in these last years. With an adeguate combined modality therapy more than 50% of these children may be cured. The results of the IRS-I indicate that the 3 year relapse-free survival rates are 85% for patients in group I 70% for those in group II, 45% for those in group III and 15% for those in group IV. In addition to the clinical group other significant prognostic factors are histologic cell type (alveolar, unfavorable) and primary site (disease in extremities and in retroperitoneal area, unfavorable). The chemiotherapy must be used in all patients for 12-24 months. The effective drugs are VCR, ACT-D, CTX, ADR combined in different schedules. It has been demonstrated that the effective doses of radiotherapy range from 4000 to 5000 rad and that radiotherapy may be omitted in patients in group I. Now a less aggressive surgical procedures may be employed, and patients with primary tumor in the orbit or in the pelvic organs may be cured saving the eye, or the bladder, the vagina and the uterus.

Adolescent↗

PubMedDate not supplied

[Congenital diaphragmatic hernia: the use of ECMO and other modern therapeutic strategies].

Congenital diaphragmatic hernia (CDH) with severe respiratory failure is still associated with significant mortality. Modern treatment of CDH is now widely accepted to be delayed repair after stabilization. Availability of Extracorporeal Membrane Oxygenation (ECMO) led up to real improvement in survival. Several others modalities have been recently used in attempting to reduce the need for ECMO or, otherwise, to improve outcome. Multicenter controlled trial of high-frequency oscillatory ventilation (HFOV), exogenous surfactant replacement, nitric oxide (NO) inhalation and, more recently, liquid ventilation have been reported. We describe four cases of CDH treated in our ECMO-centre from 1993 to date, 25% surviving. One patient died by pulmonary hypertension and multiorgan failure while on ECMO; one by pulmonary hypertension and cardiac failure and one by sepsis, both ones far from effective ECMO weaning. All patients underwent extracorporeal bypass because of Oxygenation Index (OI) ranging 65-215. Venovenous has been always made but one patient needed early switching on venoarterial. Several trials with surfactant and nitric oxide were performed during extracorporeal bypass. In survived patient, diaphragmatic defect was repaired out of ECMO. Patients survived to the weaning underwent vascular reconstruction. Our ECMO data confirm worse prognosis for CDH rather than other ECMO requiring diseases (we report 66.7% surviving in overall ECMO application); we underline real improvement by using alternative therapies together with extracorporeal bypass and primary role of OI as predicting index for ECMO.

Administration, Inhalation↗

PubMedDate not supplied

[Cystic dilatation of the choledochus: the diagnostic problems].

Choledochal cyst is a rare pathology in western countries and the typical signs and symptoms are not always present at the onset. The diagnosis is often difficult and it needs a long time to be clear. In this paper we review our recent experience with this pathology (8 patients) and particularly we point out the problems of the delate in the diagnosis and the best choice to study these patients.

Choledochal Cyst↗