[Transfusions in paediatric emergencies]. / Terapie trasfusionali nelle emergenze pediatriche.

The interest in pathological emergencies and their therapeutical options has underscored the need for immediate, precise and evidence based criteria in the administration of blood products. In fact these often are an extremely important therapeutical option, above all for children less than 3 years old. Therefore our work pointed out a precise and easy to follow diagnostic and therapeutic alghoritm as already done for other clinical emergencies. We tried to supply all the indications for the correct use of whole blood, red packed cells, platelets, granulocytes, plasma and albumin. This alghoritm includes the history, the ethiological and clinical aspects, the domiciliary interventions, the indications to the admission, the diagnostic investigations, the therapeutical aspects and the indications to the discharge. Starting from our assigned aims and following the above mentioned alghoritm, we also try to give some practical updated and evidence based hints to face the difficult situations arisen by the anxious attentions of the relatives. In fact these can sometimes be cause of mistakes. Medicine is, however, a ever changing science. The new knowledges and technologies and the changes according to the new acquisitions are at the basis of the continuous development of new diagnostic and therapeutical approaches. Our aim was therefore to provide a state of art in this field using the data published in the most recent and authoritative literature.

[Efficacy of early cerebral MR in the detection of brain lesions in high risk preterm infants compared with conventional US]. / Apporti della Risonanza Magnetica cerebrale precoce rispetto all'ecografia nella sorveglianza neurologica del grande prematuro.

To identify the efficacy of early cerebral MR, performed in the first month of birth, in the detection of brain lesions in high risk preterm infants, compared with conventional US, we recruited into the study a group of 30 preterm infants born at or below a gestational age of 30 weeks, who had a pathologic scan. The findings on US were compared with those of the early MR scan, performed in the same days, the results of which were considered as the final diagnosis. The value of cranial US as a predictor of MR signal intensity was assessed by calculating sensitivity, specificity, positive and negative predictive values. Agreement between two investigations was evaluated by calculating the K coefficient. US showed 33 haemorrhagic lesions in 25 preterms; MR showed 27 haemorrhagic lesions in 22 infants: in 16 cases MR gave the same results of US. Cranial US was reliable in detecting lesions such as GLH and IVH, but less sensitive in the definition of their size and distribution. Sensitivity of US for haemorrhagic lesions was 96.3%, PPV 78.8%, K coefficient 0.55 (p < 0.001). About the White Matter, cranial US demonstrated 20 lesions in 20 preterms; MR showed 16 lesions in 16 infants: in 3 cases MR was agree to US. US showed high reliability in the detection of cystic lesions, but significant limitations in the demonstration of non-cystic injury. We founded that normal WM echogenicity on US is not a good predictor of normal WM signal intensity on MR (30%). Sensitivity of US for WM lesions was 81.3%, PPV 65%, K coefficient 0.23 (p = 0.04). Finally US showed 4 lesions in other brain locations, MR confirmed 3 of them and discovered other 10. Sensitivity of US for these lesions was 23.1%, PPV 75%, K coefficient 0.21 (p = 0.11). We founded that cranial US is a good method for detecting GLH, IVH, HPI and severe WM lesions (cystic PVL), but it can miss non-cystic PVL, punctate haemorrhages, WMD and lesions in other brain locations, that, on the other hand, MR detects clearly.

[Vesico-ureteral reflux: diagnostic and therapeutic procedures]. / Il reflusso vescico-ureterale: approccio diagnostico clinico-strumentale e terapeutico.

The vesico ureteral reflux (VUR) is the most common uropathy associated to urinary tract infections (UTI). The identification of V.U.R. is very important to avoid reflux nephropathy and it's complications. To avoid the exposure to unnecessary and injurious x-rays, the authors propose patient selection based on the presence or absence of distal ureteral dilatation and on the time elapsed between typical or atypical symptoms of u.t.i. and fever. First of all, it is essential to diagnose pielonephritis. After ultrasound and radionuclide scintigram with DMSA they select the infants who need cystourethrography. The infants with V.U.R. undergo antibiotic prophylaxis until spontaneous or surgical recovery. The duration of prophylaxis depends on the degree of V.U.R. and the age of the infant at diagnosis.

Liposomal amphotericin B treatment for neonatal fungal infections.

BACKGROUND: Disseminated fungal infections are a major problem in high risk neonates. Conventional antifungal agents are often unsatisfactory and have a high incidence of severe adverse effects. METHODS: We administered liposomal encapsulated amphotericin B (AmBisome), which is an alternative to conventional amphotericin B, to 40 preterm (mean birth weight, 1090 +/- 313.6 g; mean gestational age, 28.35 +/- 2.13 weeks) and 4 full term (mean birth weight, 3080 +/- 118 g; mean gestational age, 39 +/- 0.7 weeks) newborn infants with a severe fungal infection. RESULTS: Candida albicans was the most frequent fungus isolated (70%). The duration of intravenous AmBisome therapy ranged from 7 to 49 days; the cumulative dose ranged from 7 to 138.8 mg/kg (median, 45.2 mg/kg). Administration of AmBisome was effective in 72.7% of patients; 5 of 6 cases of meningitis also recovered; 63.6% of 33 very low birth weight infants survived. No side effects were observed. CONCLUSIONS: To our knowledge this is the largest study of the treatment of neonates with liposomal amphotericin B, and the results confirm its effectiveness and safety. However, randomized clinical trials are required to establish the most effective administration protocol for AmBisome, i.e. the starting dosage, the maximum effective dosage and the cumulative dosage, and to verify whether the preparation should be associated with another antifungal agent.

Phenotypic variability in the chromosome 9 ring.

The syndrome associated to the 9 ring is not commonly observed. The first remark was by Kistenmacher (1970) who examined a male. Later observation of other cases has allowed the syndrome to be described, so that it can be said to be characterized by constant signs, such as microcephaly, psychomotor retardation of varying entity and facial dysmorphism corresponding to that observed in 9 p monosomy. The variability of the phenotype has to be compared with the entity of the telomeric deletion, since the clinical outlook, especially the entity of retardation, could be less serious in case of small deletions.