[Crouzon's craniofacial dysostosis]. / La disostosi craniofacciale di crouzon. Contributo casistico.

A brief account of craniofacial synostosis is presented. The cranium is acrocephalic owing to the early closure of the coronal and lambdoid sutures. The crown is typically rudimentary where the anterior fontanel is still open. Cranial radiography shows digitate markings. Skeletal changes lead to hypertelorism, exophthalmos and strabismus. The nose is of the parrot's beak type and the hypoplasic upper jaw is in contrast with the lower jaw, resulting in pseudo-prognathism. These basic malformations are accompanied by several other deformities. The disease may appear as a hereditary or as a sporadic occurrence. The cases described in the present paper confirm the hereditary interpretation of the syndrome, since both were accompanied by positive familial histories. In the first case, the signs of the disease were less evident, whereas in the second the picture was complete and both mother and daughter presented impressive examples of the frog face sign.

[Exsanguinotransfusion at the Holy Cross Hospital, Cuneo]. / La pratica exsanguinotrasfusionale presso l'Ospedale S. Croce di Cuneo.

A brief reference to the criteria employed in the selection of candidates for exsanguinotransfusion (bilirubinaemia threshold), and a short account of the medical and physical treatment of serious neonatal icterus are followed by a description of personal exsanguinotransfusion experiences. 57 cases have been treated in this fashion in 3 1/2 years, 20 from centres in the province and 37 (0.72% of births) from children both at the Obstetrics Division of the Cuneo Hospital. 32 patients were born at term, the remainder were immature. The indications for treatment included 4 cases of pathological icterus resulting from the reabsorption of haematoma (3 of these cases presented indirect bilirubin values above the 20 mg % threshold). The mean mortality index was 3.5%. Complications were confined to: (immediate), one case of thromboembolism of a lower limb; (late) frequent chronic exudative-type enteropathy. Of 20 subjects whose indirect bilirubin values had surpassed the 20 mg% threshold (with values as high as 36 mg %) before treatment, one only presented signs of cerebral lesion (aphasia, with intelligence unimpaired).

[Phototherapy in the treatment and prevention of serious icterus in the neonate]. / La fototerapia nel trattamento e nella prevenzione dell'ittero grave del neonato.

Ten years ago, treatment of serious icterus in the neonate was made more effective by the addition of a new method: the employment of the hypobilirabinaemising effect of light. The mechanism of degradation lying behind this action is not vet fully clear; there can be no doubt, however, that the method is convenient, practical and harmless. Personal results obtained in 1 yr of work in this field are reported. 113 subjects (77% of whom were immature) were treated. The ratio of treated subjects to number of births was 7% (ratio between exsanguinotransfusion and birth values, 0.7%). The means consecutive exposure time has been 48 hours but they arrived also 7 days exposure and there were no cases of immediate or long-term damage. 25% of the treated subjects presented an almost always slight bilirubinaemic rebound. The normal degradation rhythm was 0.2 mg/hr and 0.1 mg/hr on the first and second day respectively. In cases of icterus due to reabsorption of haematoma, however, a 25% above-average degradation rhythm was observed.