Background: Illness severity scores are increasingly used for risk adjustment in clinical research and quality assessment. Recently, a simplified version of the score for neonatal acute physiology (SNAPPE-II) and a revised clinical risk index for babies (CRIB-II) score have been published. Aim: To compare the discriminatory ability and goodness of fit of CRIB, CRIB-II, and SNAPPE-II in a cohort of neonates , 1500 g birth weight (VLBWI). Methods: Data from 720 VLBWI, admitted to 12 neonatal units in Lombardy (Northern Italy) participating in a regional network, were analysed. The discriminatory ability of the scores was assessed measuring the area under the receiver operating characteristic curve (AUC). Outcome measure was in-hospital death. Results: CRIB and CRIB-II showed greater discrimination than SNAPPE-II (AUC 0.90 and 0.91 v 0.84, p , 0.0004), partly because of the poor quality of some of the data required for the SNAPPE-II calculation-for example, urine output-but also because of the relative weight given to some items. In addition to each score, several variables significantly influenced survival in logistic regression models. Antenatal steroid prophylaxis, singleton birth, absence of congenital anomalies, and gestational age were independent predictors of survival for all scores, in addition to caesarean section and not being small for gestation (for SNAPPE-II) and a five minute Apgar score of > 7 (for SNAPPE-II and CRIB). Conclusions: CRIB and CRIB-II had greater discriminatory ability than SNAPPE-II. Risk adjustment using all scores is imperfect, and other perinatal factors significantly influence VLBWI survival. CRIB-II seems to be less confounded by these factors.

The need for adequate analgesia is still underestimated. Further information on the safety of analgesics in neonatology is imperative, as is an adequate education of physicians and nurses on the use of pain control guidelines as part of the standard of care in the NICU.

This study, confirms that an extremely low birth weight implies a high risk of perinatal mortality and neonatal morbidity, but that the most significant variable that can be correlated to the long-term neurological outcome is the gestational age.

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Inactivating mutations in the SLC12A3 gene that encodes the thiazide-sensitive co-transporter causes Gitelman syndrome. The main features of this syndrome include normal or low blood pressure, hypokalemia, metabolic alkalosis, hypomagnesemia, hypocalciuria, and hyperreninemia. These patients are at low risk for preterm birth and do not present with symptoms before school age. As a consequence, the condition is usually diagnosed in late childhood or in adult life. We report on four patients, two pairs of prematurely born twins, in whom hypokalemia was demonstrated early in life. In these children, a tendency towards hypokalemia was first noted during the third week of life. Overt hypokalemia subsequently appeared associated with normal blood pressure, hypochloremia, hyperreninemia, and an inappropriately high fractional excretion of potassium and chloride. Molecular biology studies failed to detect mutations in the SLC12A1, KCNJ1, and CLCNKB genes responsible for the Bartter syndromes type I, II and III, respectively. Compound heterozygous mutations in the SLC12A3 gene were detected in both pairs of twins: a frameshift mutation in exon 10 (c.1196_1202dup7bp), leading to the truncated protein p.Ser402X, and a missense mutation in exon 11, p.Ser475Cys (c.1424C>G) in the first pair; two missense mutations, p.Thr392Ile (c.1175C>T) in exon 9 and p.Ser615Leu in exon 15 (c.1844C>T), in the second pair. In conclusion, the diagnosis of Gitelman syndrome deserves consideration in infants with unexplained hypokalemia.

Aim: To reduce the problems caused by prolonged artificial ventilation in babies with Congenital Central Hypoventilation syndrome (CCHS). Methods: Two term infants with CCHS, weighing 4030 g and 3100 g, respectively, at the beginning of treatment and aged 53 and 31 d, respectively, were successfully ventilated with a Nasal Bilevel Positive Airway Pressure (N‐BiPAP) device. Results: In the first patient the tcPO2 recordings (mean ± SD) during sleep were 46 ± 12 mmHg before using N‐BiPAP and 58 ± 13 mmHg after using the device, while those for tcPCO2 were 75 ± 9 mmHg and 49 ±11 mmHg, respectively. In the second patient tcPO2 during sleep was 42 ± 3 mmHg before, and 55 ± 5 after N‐BiPAP, and for tcPCO2 the recordings were 119 ± 24 mmHg and 55 ± 6 mmHg, respectively, showing a significant improvement. One infant had persistent gastro‐oesophageal reflux, and frontal skin abrasion caused by the face mask. Nevertheless, these complications did not necessitate the discontinuation of N‐BiPAP ventilation, thus precluding prolonged use of intubation and tracheotomy. Conclusion: In infants with CCHS, early use of non‐invasive, positive‐pressure ventilation with N‐BiPAP, in association with careful monitoring, can decrease problems caused by prolonged intubation and tracheotomy.

Aim: To evaluate the incidence and duration of late‐onset neutropenia (defined as an absolute neutrophil count (ANC) <1500 mm−3 at a postnatal age of >3 wk) in a population of infants with birthweight <2000 g, and to determine whether copper deficiency, a possible cause of both anemia and neutropenia, may be associated with this complication. Methods: Complete blood cell count and differential were assessed in 247 low (LBW) and very low birthweight (VLBW) infants who were discharged after 3 wk of life. In neutropenic infants plasma copper and ceruloplasmin levels were also measured. Results: Late‐onset neutropenia was detected in 11 out of 147 VLBW infants (7.5%) and in 7 out of 127 LBW infants (5.5%). A neutrophil count of <1000 mm−3 was observed in 14 infants (5.1%). A significantly lower gestational age was found in neutropenic infants compared with non‐neutropenic infants. In neutropenic infants ANCs were significantly correlated with hemoglobin and hematocrit. In addition, a significant negative correlation was found between neutrophil and reticulocyte counts. Plasma copper concentration was significantly correlated with birthweight. Oral copper sulfate was administered to infants with plasma copper concentration <50 μg dl−1, and did not seem to affect ANC, hemoglobin, hematocrit or reticulocyte counts. Conclusion: Late‐onset neutropenia appears to be a benign condition that is not associated with any particular complication and does not require specific treatment. Reference ranges after the early neonatal period and during the first few months of life in LBW and VLBW infants should probably be set at lower values.

Our objective was to compare the effects of pressure support ventilation and synchronized intermittent mandatory ventilation on respiratory function in preterm babies. Twenty preterm infants (mean gestational age, 29 weeks; mean weight at study, 1,354 g) were evaluated. Patients received two repeated cycles of synchronized intermittent mandatory ventilation, alternated with pressure support ventilation, for a total of four alternated phases, each phase lasting 4 hr. Spontaneous respiratory rate, tidal volume, minute volume, and mean airway pressure were recorded hourly. The tidal volume released by the ventilator was limited to 6 ml/kg. During the two pressure support ventilation phases, a statistically significant reduction of respiratory rate and a significant increase of tidal and minute volume were noted, as compared to the two synchronized intermittent mandatory ventilation periods. Mean airway pressure significantly increased only after the first shift from synchronized intermittent mandatory ventilation to pressure support ventilation. The changes of minute volume and respiratory rate observed during pressure support ventilation did not persist after the return to synchronized intermittent mandatory ventilation. In conclusion, pressure support ventilation, as compared to synchronized intermittent mandatory ventilation, seemed to improve respiratory function in preterm infants.