[Influence of prolonged treatment with octreotide on GH, IGF I, insulin, ACTH, cortisol, T3, T4 and TSH secretion in a case of congenital chylothorax]. / Influenza del trattamento prolungato con octreotide sulla secrezione di GH, IGFI, insulina, ACTH, cortisolo, T3, T4 e TSH in un caso di chilotorace congenito.

Congenital chylothorax is a rare condition characterized by the accumulation of lymph fluid in the pleural space that causes respiratory and circulatory dysfunctions, immune deficiencies, hypoalbuminemia, electrolyte imbalance and alterations of the coagulation. Mortality rates are elevated and can rise to 50%. Therapy consists in conservative treatment based on thoracic drainage combined with total parenteral nutrition or use of low-fat high-protein diet supplemented with medium chain triglycerides. In case of failure surgical intervention may be considered. During the last years some authors have experienced the use of octreotide with doubtful results. In no case the drug impact on insulin, GH and cortisol secretion in neonatal age has been investigated and only in one case the effect on thyroid hormones has been assessed. We report the case of a 36-week baby with congenital chylothorax treated with octreotide for 42 days. The drug was well tolerated but hormonal level measurements showed a deep depression of insulin secretion unaccompanied by alterations of glucose levels. Levels of GH and TSH showed only a transitory decrease. ACTH and cortisol remained normal. At 5 months, the measurements of hormonal levels did not show significant alterations. It is not possible to determine if such a drug played an essential role in the solution of the pleural effusion, but it is important to emphasize that a prolonged treatment with octreotide has not caused, in our case, persistent hormonal alterations.

[Quantitative ultrasound for the assessment of bone in the preterm infant]. / Utilizzo dell'ultrasonografia quantitativa nella valutazione dello stato minerale osseo del neonato pretermine.

AIM: Preterm infants are at risk of osteopathy of prematurity (OP). The present study aims to assess quantitative ultrasound as a safe and non-invasive method in evaluating bone mineral status in the critically ill preterm infant. METHODS: Sixty preterm infants (27 females) have been evaluated in the Neonatal Intensive Care Unit of the University Hospital of Siena. Gestational age was between 22 and 36 weeks and birth weight ranged between 610 and 3240 g. A single operator did all the measurements on the second metacarpus by the DBM Sonic BP (IGEA). Ultrasound parameters were bone transmission time (BTT, micros) and speed of sound (SOS, m/s). RESULTS: BTT positively increased with birth gestational age (r=0.72, P<0.001, F-Ratio=62.4428), birth weight (r=0.7827, P<0.001, F-Ratio=91.7274) and birth length (r=0.7729, P<0.001, F-Ratio=86.0748). SOS was also related with gestational age (r=0.2937, P<0.023, F-Ratio=5.4768), birth weight (r=0.2634, P<0.042, F-Ratio=4.3250) and birth length (r=0.3252, P<0.011, F-Ratio=6.8596). Small infants for gestational age showed BTT values lower then infants with appropriate size for gestational age (P<0.03). There was no difference between male and female infants. CONCLUSION: Quantitative ultrasound is a non-invasive method, which is easy to perform in a Neonatal Intensive Unit, safe, harmless and gentle. This method detects subtle differences in bone mineral status, according to gestational age, weight and length. Quantitative ultrasound is a useful screening tool for early detection of bone status in newborns and a valid method for the longitudinal assessment of bone in growing children.

[Survival rates in a single neonatal intensive care unit (period 2002-2007). Neuro-developmental outcome and survival for infants born at 23-25 weeks of gestation]. / Sopravvivenza in una unità di terapia intensiva neonatale (periodo 2002-2007). Outcome neurologico e sopravvivenza dei nati di età gestazionale 23-25 settimane.

AIM: The aim of this study was to evaluate survival rates in a single Neonatal Intensive Care Unit (NICU) (period 2002-2007), with a special focus on the survival data and outcome at one-year of corrected age for infants born at 23-25 weeks of gestation. METHODS: All infants who had evidence of heart activity at birth were actively resuscitated, regardless of birth weight or gestational age. Survival rate was calculated as a function of the following variables: birth weight and gestational category; gender in infants of birth weight < or = 1000 g ; appropriate (AGA) or small (SGA) weight for gestational age; inborn or outborn. Twenty-eight newborns (23-25 weeks of gestation) completed follow-up at one-year of corrected age. RESULTS: During the examined period, no infants died in the delivery room; 833 newborns were admitted to the NICU. Overall survival rates were as following: <500 g (37%), 501-750 g (59%), 751-1,000 g (82%), 1,001-1,250 g (96%), 1251-1,500 g (97%), 1,501-2,000 g (100%), 2,001-2,500 g (98%), >2,500 g (99%); 23-25 weeks of gestation (50%); 26-27 weeks (77%), 28-32 weeks (90%); males < or = 1,000 g (68%), females < or = 1,000 g (68%); AGA < or = 1,000 g (63%), SGA < or = 1,000 g (79%), AGA < or =28 weeks (63%), SGA < or = 28 weeks (67%); inborn (54%), outborn (25%). A fraction of 64% (infants of 23-25 weeks of gestation) did not show handicap at one-year of corrected age, while 25% presented severe, 7% moderate, and 4% mild handicaps. CONCLUSION: High rate of survival without handicap at one-year of corrected age at extremely low gestational age and the chance of improvements in neonatal care for newborn < or = 24 weeks, indicate the appropriateness for our strategy of resuscitating all newborns with evidence of heart activity in the delivery room.

[Insulin and glucagon plasma levels in very low birth weight preterm infants of appropriate weight for gestational age]. / Livelli plasmatici di insulina e glucagone in neonati pretermine di peso molto basso appropriato per l'eta gestazionale.

OBJECTIVES: Prematurity is a known risk factor for hypoglycaemia, hyperglycemia, neonatal sepsis and other common neonatal complications, possibly associated with glucoregolatory hormone (insulin and glucagon) alterations. Insulin and glucagon levels change also in relation to gender, mode of delivery and postnatal clinical severity. Because of the lack of reference range in literature, the aim of this study is to assess plasma insulin and glucagon levels in preterm appropriate for gestational age (AGA) infants of birth weight <1500 g (very low birth weight, VLBW) as a function of gestation, birth weight, gender and mode delivery. METHODS: The authors examined 48 preterm AGA infants (mean birth weight 1 163+/-286 g, mean gestational age 28.2+/-2.4 weeks). The infant population was subdivided in relation to gestational age, weight, gender, mode of delivery and assisted ventilation at 5-7(th) days. Plasma glucose, insulin and glucagon levels were assessed in all newborns at birth and at 5-7(th) days of life. Data were analyzed using t-test. RESULTS: A negative correlation between insulin and gestational age was observed (P<0.05). At birth, no significant differences regarding plasma glucose, insulin and glucagon levels were observed as a function of the examined category variables. At the 5-7(th) days of life, insulin levels were significantly higher in newborns with gestational age =or<27 weeks (P<0.02), in the female gender (P<0.02) and in the infants born to emergency Cesarean delivery (P<0.05). CONCLUSIONS: These findings indicate potentially useful reference range values for plasma insulin and glucagon in the VLBW population.

Frequent de novo monoallelic expression of beta-spectrin gene (SPTB) in children with hereditary spherocytosis and isolated spectrin deficiency.

This report represents an attempt to define the rate of beta-spectrin de novo mutations affecting mRNA accumulation in patients with hereditary spherocytosis (HS). 19 HS children with haematologically normal parents and varying degrees of spectrin deficiency were studied. 13 of the 19 cases who were heterozygous at the genomic level for polymorphisms in the beta-spectrin coding region were further studied. However, in an analysis of reverse-transcripted amplified cDNA from the regions of the polymorphisms, seven patients appeared to be homozygous, suggesting the occurrence of de novo mutational events affecting expression of one beta-spectrin allele. We conclude that in HS patients with isolated spectrin reduction and normal parents the apparently recessive pattern of inheritance may frequently be associated with de novo monoallelic expression of beta-spectrin.