Determinants of length of stay after cesarean sections in the Friuli Venezia Giulia Region (North-Eastern Italy), 2005-2015.

Since Italy has the highest cesarean section (CS) rate (38.1%) among all European countries, the containment of health care costs associated with CS is needed, along with control of length of hospital stay (LOS) following CS. This population based cross-sectional study aims to investigate LoS post CS (overall CS, OCS; planned CS, PCS; urgent/emergency CS, UCS), in Friuli Venezia Giulia (a region of North-Eastern Italy) during 2005-2015, adjusting for a considerable number factors, including various obstetric conditions/complications. Maternal and newborn characteristics (health care setting and timeframe; maternal health factors; child's size factors; child's fragility factors; socio-demographic background; obstetric history; obstetric conditions) were used as independent variables. LoS (post OCS, PCS, UCS) was the outcome measure. The statistical analysis was conducted with multivariable linear (LoS expressed as adjusted mean, in days) as well as logistic (adjusted proportion of LoS > 4 days vs. LoS ≤ 4 days, using a 4 day cutoff for early discharge, ED) regression. An important decreasing trend over time in mean LoS and LoS > ED was observed for both PCS and UCS. LoS post CS was shorter with parity and history of CS, whereas it was longer among non-EU mothers. Several obstetric conditions/complications were associated with extended LoS. Whilst eclampsia/pre-eclampsia and preterm gestations (33-36 weeks) were predominantly associated with longer LoS post UCS, for PCS LoS was significantly longer with birthweight 2.0-2.5 kg, multiple birth and increasing maternal age. Strong significant inter-hospital variation remained after adjustment for the major clinical conditions. This study shows that routinely collected administrative data provide useful information for health planning and monitoring, identifying inter-hospital differences that could be targeted by policy interventions aimed at improving the efficiency of obstetric care. The important decreasing trend over time of LoS post CS, coupled with the impact of some socio-demographic and obstetric history factors on LoS, seemingly suggests a positive approach of health care providers of FVG in decision making on hospitalization length post CS. However, the significant role of several obstetric conditions did not influence hospital variation. Inter-hospital variations of LoS could depend on a number of factors, including the capacity to discharge patients into the surrounding non-acute facilities. Further studies are warranted to ascertain whether LoS can be attributed to hospital efficiency rather than the characteristics of the hospital catchment area.

Author Correction: A Systematic Evaluation of Hospital Performance of Childbirth Delivery Modes and Associated Factors in the Friuli Venezia Giulia Region (North-Eastern Italy), 2005-2015.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Understanding Factors Leading to Primary Cesarean Section and Vaginal Birth After Cesarean Delivery in the Friuli-Venezia Giulia Region (North-Eastern Italy), 2005-2015.

Although there is no evidence that elevated rates of cesarean sections (CS) translate into reduced maternal/child perinatal morbidity or mortality, CS have been increasingly overused almost everywhere, both in high and low-income countries. The primary cesarean section (PCS) has become a major driver of the overall CS (OCS) rate, since it carries intrinsic risk of repeat CS (RCS) in future pregnancies. In our study we examined patterns of PCS, pl compared with planned TOLAC anned PCS (PPCS), vaginal birth after 1 previous CS (VBAC-1) and associated factors in Friuli Venezia Giulia (FVG), a region of North-Eastern Italy, collecting data from its 11 maternity centres (coded from A to K) during 2005-2015. By fitting three multiple logistic regression models (one for each delivery mode), we calculated the adjusted rates of PCS and PPCS among women without history of CS, whilst the calculation of the VBAC rate was restricted to women with just one previous CS (VBAC-1). Results, expressed as odds ratio (OR) with 95% confidence interval (95%CI), were controlled for the effect of hospital, calendar year as well as several factors related to the clinical and obstetric conditions of the mothers and the newborn, the obstetric history and socio-demographic background. In FVG during 2005-2015 there were 24,467 OCS (rate of 24.2%), 19,565 PCS (19.6%), 7,736 PPCS (7.7%) and 2,303 VBAC-1 (28.4%). We found high variability of delivery mode (DM) at hospital level, especially for PCS and PPCS. Breech presentation was the strongest determinant for PCS as well as PPCS. Leaving aside placenta previa/abuptio placenta/ante-partum hemorrhage, further significant factors, more importantly associated with PCS than PPCS were non-reassuring fetal status and obstructed labour, followed by (in order of statistical significance): multiple birth; eclampsia/pre-eclampsia; maternal age 40-44 years; placental weight 600-99 g; oligohydramios; pre-delivery LoS 3-5 days; maternal age 35-39 years; placenta weight 1,000-1,500 g; birthweight < 2,000 g; maternal age ≥ 45 years; pre-delivery LoS ≥ 6 days; mother's age 30-34 years; low birthweight (2,000-2,500 g); polyhydramnions; cord prolaspe; ≥6 US scas performed during pregnancy and pre-term gestations (33-36 weeks). Significant factors for PPCS were (in order of statistical significance): breech presentation; placenta previa/abruptio placenta/ante-partum haemorrhage; multiple birth; pre-delivery LoS ≥ 3 days; placental weight ≥ 600 g; maternal age  40-44 years; ≥6 US scans performed in pregnancy; maternal age ≥ 45 and 35-39 years; oligohydramnios; eclampsia/pre-eclampsia; mother's age 30-34 years; birthweight <2,000 g; polyhydramnios and pre-term gestation (33-36 weeks). VBAC-1 were more likely with gestation ≥ 41 weeks, placental weight <500 g and especially labour analgesia. During 2005-2015 the overall rate of PCS in FVG (19.6%) was substantially lower than the corresponding figure reported in 2010 for the entire Italy (29%) and still slightly under the most recent national PCS rate for 2017 (22.2%). The VBAC-1 rate on women with history of one previous CS in FVG was 28.4% (25.3% considering VBAC on all women with at least 1 previous CS), roughly three times the Italian national rate of 9% reported for 2017. The discrepancy between the OCS rate at country level (38.1%) and FVG's (24.2%) is therefore mainly attributable to RCS. Although there was a marginal decrease of PCS and PPCS crudes rates over time in the whole region, accompained by a progressive enhancement of the crude VBAC rate, we found remarkable variability of DM across hospitals. To further contain the number of unnecessary PCS and promote VBAC where appropriate, standardized obstetric protocols should be introduced and enforced at hospital level. Decision-making on PCS should be carefully scrutinized, introducing a diagnostic second opinion for all PCS, particularly for term singleton pregancies with cephalic presentation and in case of obstructed labour as well as non-reassuring fetal status, grey areas potentially affected by subjective clinical assessment. This process of change could be facilitated with education of staff/patients by opinion leaders and prenatal counseling for women and partners, although clinical audits, financial penalties and rewards to efficient maternity centres could also be considered.

A Systematic Evaluation of Hospital Performance of Childbirth Delivery Modes and Associated Factors in the Friuli Venezia Giulia Region (North-Eastern Italy), 2005-2015.

Cesarean sections (CS) have become increasingly common in both developed and developing countries, raising legitimate concerns regarding their appropriateness. Since improvement of obstetric care at the hospital level needs quantitative evidence, using routinely collected health data we contrasted the performance of the 11 maternity centres (coded with an alphabetic letter A to L) of an Italian region, Friuli Venezia Giulia (FVG), during 2005-15, after removing the effect of several factors associated with different delivery modes (DM): spontaneous vaginal delivery (SVD), instrumental vaginal delivery (IVD), overall CS (OCS) and urgent/emergency CS (UCS). A multivariable logistic regression model was fitted for each individual DM, using a dichotomous outcome (1 = each DM; 0 = rest of hospital births) and comparing the stratum specific estimates of every term with their respective reference categories. Results were expressed as odds ratios (OR) with 95% confidence intervals (95%CI). The Benjamini-Hochberg (BH) false discovery rates (FDR) approach was applied to control alpha error due to the large number of statistical tests performed. In the entire FVG region during 2005-2015, SVD were 75,497 (69.1% out of all births), IVD were 7,281 (6.7%), OCS were 26,467 (24.2%) and UCS were 14,106 (12.9% of all births and 53.3% out of all CS). SVD were more likely (in descending order of statistical significance) with: higher number of previous livebirths; clerk/employed occupational status of the mother; gestational age <29 weeks; placentas weighing <500 g; stillbirth; premature rupture of membranes (PROM). IVD were predominantly more likely (in descending order of statistical significance) with: obstructed labour, non-reassuring fetal status, history of CS, labour analgesia, maternal age ≥35 and gestation >40 weeks. The principal factors associated with OCS were (in descending order of statistical significance): CS history, breech presentation, non-reassuring fetal status, obstructed labour, multiple birth, placental weight ≥ 600 g, eclampsia/pre-eclampsia, maternal age ≥ 35 and oligohydramnios. The most important risk factors for UCS were (in descending order of statistical significance): placenta previa/abruptio placenta/ antepartum hemorrage; non-reassuring fetal status, obstructed labour; breech presentation; PROM, eclampsia/pre-eclampsia; gestation 33-36 weeks; gestation 41+ weeks; oligohydramnios; birthweight <2,500 g, maternal age ≥ 35 and cord prolapse. After removing the effects of all other factors, we found great variability of DM rates across hospitals. Adjusting for all risk factors, all hospitals had a OCS risk higher than the referent (hospital G). Out of these 10 hospitals with increased adjusted risk of OCS, 9 (A, B, C, D, E, F, I, J, K) performed less SVD and 5 (A, C, D, I, J) less IVD. In the above 5 centres CS was therefore probably overused. The present study shows that routinely collected administrative data provide useful information for health planning and monitoring. Although the overall CS rate in FVG during 2005-15 was 24.2%, well below the corresponding average Italian national figure (38.1%), the variability of DM rates across FVG maternity centres could be targeted by policy interventions aimed at reducing the recourse to unnecessary CS. In some clinical conditions such as obstructed labor, non-reassuring fetal status, breech presentation, history of CS, higher maternal age and multiple birth, consideration may be given to more conservative DM. The overuse of CS in nulliparas and repeat CS (RCS) should be carefully monitored and subject to audit.

Immediate delivery or expectant management in gestational diabetes at term: the GINEXMAL randomised controlled trial.

OBJECTIVE: To evaluate maternal and perinatal outcomes after induction of labour versus expectant management in pregnant women with gestational diabetes at term. DESIGN: Multicentre open-label randomised controlled trial. SETTING: Eight teaching hospitals in Italy, Slovenia, and Israel. SAMPLE: Singleton pregnancy, diagnosed with gestational diabetes by the International Association of Diabetes and Pregnancy Study Groups criteria (IADPSGC), between 38+0 and 39+0 weeks of gestation, without other maternal or fetal conditions. METHODS: Patients were randomly assigned to induction of labour or expectant management and intensive follow-up. Data were analysed by 'intention to treat'. MAIN OUTCOME MEASURES: The primary outcome was incidence of caesarean section. Secondary outcomes were maternal and perinatal mortality and morbidity. RESULTS: A total of 425 women were randomised to the study groups. The incidence of caesarean section was 12.6% in the induction group versus 11.7% in the expectant group. No difference was found between the two groups (relative risk, RR 1.06; 95% confidence interval, 95% CI 0.64-1.77; P = 0.81). The incidence of non-spontaneous delivery, either by caesarean section or by operative vaginal delivery, was 21.0 and 22.3%, respectively (RR 0.94; 95% CI 0.66-1.36; P = 0.76). Neither maternal nor fetal deaths occurred. The few cases of shoulder dystocia were solved without any significant birth trauma. CONCLUSIONS: In women with gestational diabetes, without other maternal or fetal conditions, no difference was detected in birth outcomes regardless of the approach used (i.e. active versus expectant management). Although the study was underpowered, the magnitude of the between-group difference was very small and without clinical relevance. TWEETABLE ABSTRACT: Immediate delivery or expectant management in gestational diabetes at term?

Does EMLA cream application interfere with the success of venipuncture or venous cannulation? A prospective multicenter observational study.

UNLABELLED: Venipuncture and intravenous cannulation are the most common painful procedures performed on children. The most widely used topical anesthetic is eutectic mixture of local anesthetics (EMLA). EMLA use is associated with a transient cutaneous vasoconstriction which can make it difficult to identify veins. We assessed with a prospective, multicenter, observational study whether EMLA interferes with venipuncture and intravenous cannulation. The primary study outcome was a success at first attempt in the course of venipuncture or venous cannulation. The study enrolled 388 children; 255 of them received EMLA and 133 did not. Eighty-six percent of procedures were successful at the first attempt in the EMLA group and 76.7 % in the no EMLA group. CONCLUSION: In this study, EMLA use did not interfere with the success of venipuncture or venous cannulation in children.

Diagnosed child, treated child: food challenge as the first step toward tolerance induction in cow's milk protein allergy.

BACKGROUND: Food challenge is required to assess tolerance in cow milk (CM) allergy. A positive challenge contraindicates the reintroduction of CM. Specific oral tolerance induction (SOTI) is a promising treatment. METHODS: All children admitted for a challenge were prospectively enrolled. To those tolerating between 2 and 150 ml a SOTI protocol was offered. Outcome, adverse reactions, parents' satisfaction were recorded. RESULTS: Out of 245 challenged patients, 175 reacted 122 out of 125, able to tolerate a minimum dose of 2 ml, underwent SOTI. After one year 75.4% were in an unrestricted diet, 16.1% tolerated between 5 and 150 ml, 8.5% stopped SOTI. Side effects were mild, parents' satisfaction was very high. CONCLUSIONS: The majority of children tolerating limited amounts of CM at the challenge acquires tolerance with SOTI without relevant side effects. Maintaining on an exclusion diet partially tolerant children should be considered debatable.

Videogame playing as distraction technique in course of venipuncture.

BACKGROUND: Needle-related procedures (venipuncture, intravenous cannulation) are the most common source of pain and distress for children. Reducing needle related pain and anxiety could be important in order to prevent further distress, especially for children needing multiple hospital admissions. The aim of the present open randomized controlled trial was to investigate the efficacy of adding an active distraction strategy (videogame) to EMLA premedication in needle-related pain in children. METHODS: One-hundred and nine children (4 -10 years of age) were prospectively recruited to enter in the study. Ninety-seven were randomized in two groups: CC group (conventional care: EMLA only) as control group and AD group (active distraction: EMLA plus videogame) as intervention group. Outcome measures were: self-reported pain by mean of FPS-R scale (main study outcome), observer-reported pain by FLACC scale, number of attempts for successful procedure. RESULTS: In both groups FPS-R median rate was 0 (interquartile range: 0-2), with significant pain (FPS-R > 4) reported by 9% of subjects. FLACC median rate was 1 in both groups (interquartile range 0-3 in CC group; 0-2 in AD group). The percentage of children with major pain (FLACC > 4) was 18% in CC group and 9% in AD group (p = 0.2). The median of necessary attempts to succeed in the procedures was 1 (interquartile range 1-2) in both groups.. CONCLUSION: Active distraction doesn't improve EMLA analgesia for iv cannulation and venipuncture. Even though, it resulted in an easily applicable strategy appreciated by children. This technique could be usefully investigated in other painful procedures.

First trimester maternal serum PIGF, free ß-hCG, PAPP-A, PP-13, uterine artery Doppler and maternal history for the prediction of preeclampsia.

OBJECTIVE: To evaluate the detection of pregnancy hypertensive disorders by integrating maternal history, serum biomarkers and uterine artery Doppler in the first trimester. METHODS: We prospectively recruited 2118 women that underwent an 11-13 weeks aneuploidy screening. We gathered information on maternal history, uterine artery Doppler and serum biomarkers (PAPP-A, PlGF, PP-13 and free ß-hCG). Models were developed for the prediction of overall preeclampsia (PE), early-onset PE, late-onset PE and gestational hypertension (GH). For each outcome, we performed a multivariate logistic regression starting from the saturated model: adopting a step-down procedure we excluded all factors not statistically significant (p > 0.05). Sensitivity models only for statistically significant parameters were calculated from the ROC curves for fixed false-positive rates (FPR). RESULTS: Among 2118 women, 46 (2.17%) developed GH and 25 (1.18%) were diagnosed with PE, including 12 (0.57%) early-onset PE and 13 (0.61%) late-onset PE. For a fixed FPR of 10 and 5%, serum PlGF, free ß-hCG and chronic hypertension identified respectively 67 and 75% of women who developed early-onset PE. In the model for the prediction of overall PE the combination of the uterine artery Doppler pulsatility index (UtA PI) with PlGF and chronic hypertension reached a sensitivity of 60% for a 20% of FPR. CONCLUSION: An integration of maternal characteristics and first trimester maternal serum biomarkers (free ß-hCG and PlGF) provided a possible screening for early-onset PE. In the overall PE model, UtA PI turned out to be statistically significant but did not improve the detection rate.

Adverse effects during specific oral tolerance induction: in home phase.

BACKGROUND: Specific oral tolerance induction (SOTI) is a promising approach for severe food allergies. There are little data in the literature regarding the home-phase of SOTI, not only with regard to type and frequency of adverse reactions but also regarding the most suitable treatment and protocol. AIMS: To define the incidence and severity of adverse reactions, possible risk factors, and the safety and effectiveness of the home-phase of an original SOTI protocol in a large group of children with severe cow's milk (CM) allergy, after the hospital "rush" phase. METHODS: The study was conducted by recording in-home phase adverse events, success and failure as reported by parents, and calling families. Adverse reactions were treated following the International Guidelines, arbitrarily modified by introducing nebulised epinephrine for respiratory reactions, oral beclomethasone for acute gastric pain and oral cromolyn for recurrent gastric pain. RESULTS: Out of 140 patients, 132 were contacted; eight were inaccessible (follow-up 2-84 months). The number of adverse reactions was 1 in every 100 doses. The reactions were treated with nebulised epinephrine (221 reactions), IM epinephrine (6 reactions), and other drugs. Patients with high specific IgE levels (greater than 100 kU(A)/L) and lower CM dose (less than 5 ml) at the end of in-hospital phase showed a higher risk both for number of reactions and use of nebulised epinephrine. CONCLUSIONS: The home phase of SOTI was characterised by a significant number of adverse reactions, mostly managed with an acceptable rate of side effects. Nebulised epinephrine played a pivotal role in respiratory reactions.

Interventions for the prevention of overweight and obesity in preschool children: a systematic review of randomized controlled trials.

The objective of this study was to analyse interventions for the prevention of overweight and obesity in children under 5 years of age. We carried out a systematic review focusing exclusively on randomized controlled trials (RCTs). Data sources include Medline, Cochrane Library, EMBASE, CINHAL, PsychInfo and Web of Science. Data were extracted from seventeen articles describing seven RCTs identified through electronic search, screening of references in systematic reviews, own files and contact with authors. RCTs were assessed with the Jadad scale. Four trials were carried out in preschool settings, one with an exclusive educational component, two with an exclusive physical activity component and one with both. Two trials were family-based, with education and counselling for parents and children. The remaining trial was carried out in maternity hospitals, with a training intervention on breastfeeding. None of the interventions had an effect in preventing overweight and obesity. The failure to show an effect may be due to the choice of outcomes, the quality of the RCTs, the suboptimal implementation of the interventions, the lack of focus on social and environmental determinants. More rigorous research is needed on interventions and on social and environmental factors that could impact on lifestyle.

Gestational diabetes and fetal growth acceleration: induction of labour versus expectant management.

AIM: The aim of the study was to compare elective induction of labour at 38 weeks versus expectant management in A1 and A2 gestational diabetes (GDM) pregnancies with fetal growth acceleration. Primary outcome of the study was C-section (CS) rate, while secondary outcomes were macrosomia incidence and adverse perinatal outcomes. METHODS: A retrospective cohort study was carried out. Data were collected between 1996 and 2006 and evaluated through patients' records analysis. Differences between the two study groups were investigated using non-parametric tests for continuous variables and χ2 test for categorical ones. RESULTS: There was no significant difference between induction and expectant management in terms of caesarean section rate. A trend favoring women in the induction group in terms of incidence of macrosomia and neonatal outcomes was identified, but results were not statistically significant. CONCLUSION: Labour induction at 38 weeks in GDM patients with fetal growth acceleration does not seem to determine an increased incidence of C-section in comparison to expectant management, particularly in case of maternal obesity.

Early-life determinants of overweight and obesity: a review of systematic reviews.

The aim of this paper was to review the evidence for early-life (from conception to 5 years of age) determinants of obesity. The design is review of published systematic reviews. Data sources included Medline, Embase, Web of Science, Cochrane Library, CINAHL, PsycINFO. Identification of 22 eligible reviews from a database of 12,021 independent publications. Quality of selected reviews assessed using the Assessment of Multiple Systematic Reviews score. Articles published after the reviews were used to confirm results. No review was classified as high quality, 11 as moderate and 11 as low. Factors associated with later overweight and obesity: maternal diabetes, maternal smoking, rapid infant growth, no or short breastfeeding, obesity in infancy, short sleep duration, <30 min of daily physical activity, consumption of sugar-sweetened beverages. Other factors were identified as potentially relevant, although the size of their effect is difficult to estimate. Maternal smoking, breastfeeding, infant size and growth, short sleep duration and television viewing are supported by better-quality reviews. It is difficult to establish a causal association between possible determinants and obesity, and the relative importance of each determinant. Future research should focus on early-life interventions to confirm the role of protective and risk factors and to tackle the high burden obesity represents for present and future generations.

Respiratory training with a specific device in cystic fibrosis: a prospective study.

INTRODUCTION: Chest physiotherapy (CP) is used in cystic fibrosis (CF) even if there is no robust scientific evidence of a beneficial effect. We investigated the effects of a training with a specific device (SpiroTiger) in a group of CF patients. This device, developed for respiratory training through maximal inspirations and espirations without hypocarbia, may improve respiratory function and mucus clearance. Patients where instructed and trained by a physiotherapist with individualized settings of training parameters. METHODS: Twenty-four patients were enrolled in an open-label 1 year observational study. Baseline and post intervention measurements were determined by lung function (FVC, FEV1, FEF 25-75), patients' opinions on physiotherapy (questionnaires), need for antibiotic treatment (clinical follow-up and records) and perception of physical fitness (questionnaires) in the year before and in the year of intervention. Adherence to physiotherapy was monitored by means of a specific device software. RESULTS: Increased lung function (FEV1 p<0.01), perception of physical fitness (p<0.001) and a reduction in the need for intravenous antibiotic treatment (p<0.001) were reported. Adherence to treatment was good/acceptable in 92% of patients. CONCLUSIONS: This study shows an association between training through a specific device and improved lung function. Further trials are needed to confirm this report.

Efficacy and safety of thalidomide in children and young adults with intractable inflammatory bowel disease: long-term results.

BACKGROUND: Anti-tumour necrosis factor-alpha antibodies are useful for the treatment of refractory Crohn's disease and ulcerative colitis. Thalidomide is another agent with tumour necrosis factor-alpha suppressive properties. AIM: To investigate the long-term efficacy and safety of thalidomide in a group of children and young adults with refractory inflammatory bowel disease. METHODS: Twenty-eight patients with refractory moderate-severe inflammatory bowel disease (19 Crohn's disease, 9 ulcerative colitis) received thalidomide 1.5-2.5 mg/kg/day. Patients were assessed at baseline, at weeks 2, 4, 8 and 12, and then every 12 weeks by patient's diary, physical examinations, laboratory analyses and scoring on activity indexes. Primary outcomes were: (i) efficacy in inducing remission; and (ii) efficacy in maintaining remission. RESULTS: Remission was achieved with thalidomide in 21 of 28 (75%) patients (17 with Crohn's disease, 4 with ulcerative colitis). Mean duration of remission was 34.5 months. Sixteen of 20 (80%) patients suspended steroids. Reversible neuropathy occurred in seven of 28 (25%) patients, but only with cumulative doses over 28 g. Other side effects requiring thalidomide suspension were vertigo/somnolence (one of 28), and agitation/hallucinations (one of 28). CONCLUSIONS: Thalidomide seems to be effective in inducing long-term remission in children and adolescents with intractable inflammatory bowel disease. Neuropathy is the main adverse effect, but appears to be cumulative dose-dependent, thus allowing long-term remission before drug suspension.

[Short-stay observation in the Pediatric Emergency Room: a 2003-1993 comparison]. / L'osservazione temporanea in Pronto Soccorso pediatrico. Confronto 2003-1993.

AIM: To evaluate the effectiveness of Short-Stay Observation (SSO) in the paediatric Emergency Room (ER) according to changes over time of guidelines. METHODS: This retrospective study analyses the probability of discharge immediately from ER or after SSO and of hospital admission in Trieste during 2003 as compared with 1993. Subjects aged under 18 with respiratory, urinary tract, neurological and gastrointestinal pathologies, selected symptoms and injuries were studied. RESULTS: In 2003 the 86.2% of 6 350 patients enrolled in the study were discharged immediately, compared with the 81.7% of 5475 subjects in 1993 (RR 1.05, IC 95% 1.04-1.07), the 10.0% were discharged after SSO, compared with the 5.9% in 1993 (RR 1.71, IC 95% 1.50-1.95). The 3.8% of the subjects were admitted in 2003, compared to the 12.4% in 1993 (RR 0.30, IC 95% 0.26-0.35). The role of SSO was particularly significant in case of pneumonia, URI, urinary tract infections, seizures, gastroenteritis, abdominal pain, infant fever, injuries. CONCLUSIONS: The decrease of hospital admissions, pointed out in 2003, are related to a better choice of candidates for SSO and to changes in guidelines concerning the approach to urinary tract infections and seizures, the treatment of respiratory distress and gastroenteritis dehydration, and the early identification of predictors of injuries complications. The study confirms the effectiveness of SSO in both avoiding inappropriate admissions and reducing those with limited needs of hospital assistance, assuring the quality of care. The reduced hospital stay decreases child and his family discomfort and favours the care continuity due to the involvement of family paediatrician.

Gaucher disease and bone: laboratory and skeletal mineral density variations during a long period of enzyme replacement therapy.

The usefulness of bone turnover markers in Gaucher disease is still unclear and their utility in monitoring the effects of enzyme replacement therapy (ERT) on bone metabolism has not yet been investigated exhaustively. Skeletal involvement seems to improve slowly during ERT, but only a few studies evaluating bone mineral density (BMD) changes during a long follow-up period have been reported. The aim of this study was to assess the efficacy of ERT on bone involvement in a group of 12 type I Gaucher disease (GD I) patients by monitoring biochemical indices of bone resorption/formation and BMD measured by dual energy x-ray absorptiometry (DEXA). Serum (calcium, phosphorus, bone alkaline phosphatase isoenzyme, carboxyterminal propeptide of type I procollagen (PICP), carboxyterminal telopeptide of type I collagen (ICTP), osteocalcin, intact parathyroid hormone) and urinary (calcium, phosphorus, hydroxyproline and free deoxypyridinoline) markers of bone metabolism and lumbar BMD were measured at baseline, after 6 and 12 months, and then every year for a mean ERT follow-up period of 4.5 years (range 4.4-6 years). Twelve healthy adult subjects matched for age and sex were tested as negative controls. A significant decrease of PICP was detected in the patient group at baseline (mean value 100.52 ng/ml vs 142.45 ng/ml, p = 0.017), while ICTP was remarkably higher: mean value 3.93 ng/ml vs 2.72 ng/ml, p = 0.004 (two-sided Student's t-test). No changes in bone formation indices were observed during the follow-up period, while urinary calcium excretion increased significantly from 0.065 to 0.191 mg/mg creatinine (p = 0.0014) (repeated measures ANOVA). A significant BMD improvement was also detected after an average ERT period of 4.5 years: Z-score increased from -0.81 to -0.56 (p = 0.005) (two-sided Student's t-test). These data evidenced the ineffectiveness of the biochemical markers used in monitoring ERT efficacy in GD I skeletal involvement, whereas DEXA was demonstrated to be a reliable method with which to follow up BMD improvement.