[Dyggve-Melchior-Clausen syndrome: description of 2 further cases]. / La sindrome di Dyggve-Melchior-Clausen: descrizione di due nuovi casi clinici.

In this paper we describe the clinical and radiographic features of a spondylo-epi-methaphyseal dysplasia. Dyggve-Melchior-Clausen syndrome. In these two new cases, without severe mental retardation, we have highlighted the clinical and radiological findings, progression of the skeletal changes that have allowed us to make a diagnosis.

[Current validity of the exchange transfusion intervention by the classic indication. Our 15 years' experience]. / Validità attuale dell'intervento di exanguinotrasfusione secondo l'indicazione classica. Nostra esperienza di 15 anni.

In this paper the Authors report their personal experience of the use of exchange transfusion, secondary to classic indication, in the treatment of neonatal hyperbilirubinemia, in order to: 1) determine the trend over the past wears in the number of exchange transfused neonates, both from a global point of view and in relation to indications; 2) critically assess the risks, in terms of complications and mortality, correlated to exchange transfusion. Four hundred and eighty-eight neonates, who were subjected to 693 exchange transfusions in the Pediatric Clinic and Neonatal Division of the Policlinico Gemelli in Roma, were studied over a period of 15 years (1972-1986), according to the following indications: 214 cases of neonatal hyperbilirubinemia with MEN-Rh, 106 cases of neonatal hyperbilirubinemia with MEN-ABO and 168 cases of idiopathic hyperbilirubinemia. The total number of exchange transfused neonates decreased drastically from 304 in the period 1972-76 to 65 in the period 1982-86. The frequency of exchange transfused neonates because of idiopathic hyperbilirubinemia decreased significantly (p greater than 0.001), booth in comparison to the number of live births and in comparison to the number of exchange transfused neonates, probably due to the gradual introduction of phototherapy. The frequency of exchange transfused neonates with iso-Rh and iso-ABO decreased or remained stationary on account of the confirmed relative lesser efficacy of phototherapy on hemolytic jaundice. With regard to mortality and morbidity, 27 out of 488 neonates died during the neonatal period, but only 4 within six hours of exchange transfusion; the majority of those who died had a reduced gestational age and low birthweight, and were affected by a severe associated pathology.