The hairy elbows syndrome: clinical and neuroradiological findings.

The hairy elbows syndrome (HES) is a rare congenital phenotype characterized by an abnormal increase in long hairs localized on the upper limbs extensor surfaces. This feature is often associated with short stature, facial asymmetry, dysmorphisms, intrauterine growth retardation (IUGR), and mental and speech delay. We report a case with hypertricosis cubiti associated with infantile spasms, behaviour disorders and cerebral hemisphere asymmetry. Although these findings have not been previously described we are uncertain whether they are unusual or underestimated. However, it is likely that these neurological findings are strongly interrelated leading to a more severe phenotype of the syndrome.

Clinical approach to the analysis of causes of death in the first two years of life of very-low-birthweight infants in a multicentre setting.

Mortality in the first 2 years of 634 very-low-birthweight infants admitted to eight neonatal intensive care units in Italy, and the factors associated with the net probability of death from each cause, were studied by means of the Cox proportional hazard model. A clinical classification of the causes of death was used. Overall mortality was 33.7% (intercentre range 12.6-52.9%). The highest cause-specific mortality rates were observed for respiratory problems, intra-ventricular haemorrhage (IVH) and infections (14.5%, 6.3% and 5.7% respectively). The leading causes of death were respiratory problems and IVH in the first week of life, infections from the second week up to the end of the first month, and bronchopulmonary dysplasia (BPD) afterwards. Birthweight < 1000 g, gestational age < 30 weeks, absence of spontaneous respiratory activity, unknown body temperature and pH < 7.20 at admission were associated with death from respiratory problems and IVH. Male sex, birthweight < 1000 g and unknown body temperature at admission were associated with death from BPD. Mortality from infections was higher in one centre; no other differences emerged among the eight NICUs. The classification of the causes of death employed and the use of the net probabilities of death appear as practical and useful instruments to study the relationship between specific aspects of medical care and mortality, and to investigate the reasons for differences in performance between neonatal units.

[Incidence and evolution of retinopathy of prematurity. A six year experience in a neonatal intensive care unit]. / Incidenza ed evoluzione della retinopatia del pre-termine. Esperienza di sei anni in un Centro di Terapia Intensiva Neonatale.

The incidence and the evolution of ROP in 324 infants with birthweight < or = 2500 g is report. The highest incidence was among the 750 and 1250 g birthweight infants. 5 infants < 750 g survived without signs of ROP. When present the ROP was within the 3rd stage and had a favourable outcome: spontaneous resolution without cicatricial lesions.

The use of intravenous gamma-globulin for prevention of sepsis in pre-term infants. A controlled clinical trial.

This paper reports a randomized clinical trial to study the effect of an intravenous gamma-globulin preparation to prevent sepsis in pre-term newborn infants. 80 infants were enrolled: 37 of birthweight less than or equal to 1500 g and 43 of birthweight 1501-2000 g. In each group 20 infants received an intravenous preparation of gamma-globulin (0.5 g/kg/wk); the remaining 17 and 20, respectively, served as control cases. No significant differences in the occurrence of sepsis were observed between the group receiving prophylactively intravenous gamma-globulin and the control group. This is particularly evident in infants under intensive care (35% of the total population): in this group 2/3 of sepsis occurred in infants who received IgG. Among the infants with sepsis, the presence of an umbilical artery catheterization represented a significant risk-factor. The post-dose increment of serum IgG did not differ significantly in infants with and without sepsis; the post-dose serum disappearance rate in concentration appears identical in the two groups.