Breast density in NF1 women: a retrospective study.

Neurofibromatosis type 1 (NF1) is an autosomal dominant condition caused by neurofibromin haploinsufficiency due to pathogenic variants in the NF1 gene. Tumor predisposition has long been associated with NF1, and an increased breast cancer (BC) incidence and reduced survival have been reported in recent years for women with NF1. As breast density is another known independent risk factor for BC, this study aims to evaluate the variability of breast density in patients with NF1 compared to the general population. Mammograms from 98 NF1 women affected by NF1, and enrolled onto our monocentric BC screening program, were compared with those from 300 healthy subjects to verify differences in breast density. Mammograms were independently reviewed and scored by a radiologist and using a Computer-Aided Detection (CAD) software. The comparison of breast density between NF1 patients and controls was performed through Chi-squared test and with multivariable ordinal logistic models adjusted for age, body mass index (BMI), number of pregnancies, and menopausal status.breast density was influenced by BMI and menopausal status in both NF1 patients and healthy subjects. No difference in breast density was observed between NF1 patients and the healthy female population, even after considering the potential confounding factors.Although NF1 and a highly fibroglandular breast are known risk factors of BC, in this study, NF1 patients were shown to have comparable breast density to healthy subjects. The presence of pathogenic variants in the NF1 gene does not influence the breast density value.

Treatment discontinuation following low-dose TKIs in 248 chronic myeloid leukemia patients: Updated results from a campus CML real-life study.

TKIs long-term treatment in CML may lead to persistent adverse events (AEs) that can promote relevant morbidity and mortality. Consequently, TKIs dose reduction is often used to prevent AEs. However, data on its impact on successful treatment-free remission (TFR) are quite scarce. We conducted a retrospective study on the outcome of CML subjects who discontinued low-dose TKIs from 54 Italian hematology centers participating in the Campus CML network. Overall, 1.785 of 5.108 (35.0%) regularly followed CML patients were treated with low-dose TKIs, more frequently due to relevant comorbidities or AEs (1.288, 72.2%). TFR was attempted in 248 (13.9%) subjects, all but three while in deep molecular response (DMR). After a median follow-up of 24.9 months, 172 (69.4%) patients were still in TFR. TFR outcome was not influenced by gender, Sokal/ELTS risk scores, prior interferon, number and last type of TKI used prior to treatment cessation, DMR degree, reason for dose reduction or median TKIs duration. Conversely, TFR probability was significantly better in the absence of resistance to any prior TKI. In addition, patients with a longer DMR duration before TKI discontinuation (i.e., >6.8 years) and those with an e14a2 BCR::ABL1 transcript type showed a trend towards prolonged TFR. It should also be emphasized that only 30.6% of our cases suffered from molecular relapse, less than reported during full-dose TKI treatment. The use of low-dose TKIs does not appear to affect the likelihood of achieving a DMR and thus trying a treatment withdrawal, but might even promote the TFR rate.

Cardiopulmonary testing in adult patients with ß-thalassemia major in comparison to healthy subjects.

ß-Thalassemia patients often have a reduced capacity of exercise and abnormal respiratory function parameters, but the reasons are unclear. In order to identify the causes of the exercise limitation, we performed a cardiopulmonary exercise testing (CPET) in a group of 54 adult ß-thalassemia major (TM) patients without pulmonary arterial hypertension and in a group of healthy control subjects. All subjects underwent cardiac echocardiography and carried out pulmonary function tests. TM patients also filled an IPAQ questionnaire on usual physical activity (PA).Overall, TM patients have a diminished exercise performance in comparison to control subjects. In fact, peak oxygen uptake (V'O2 peak), expressing maximum exercise capacity, was decreased in 81.5% of the patients; similarly, anaerobic threshold (V'O2@AT) and O2 pulse also resulted lowered. In multivariable regression models adjusted for gender, age, BMI, and mean haemoglobin, V'O2 peak and O2 pulse were positively associated with cardiac iron overload (T2*). No ventilatory limitation to exercise was observed. The most important causes of exercise limitation in these patients were muscular deconditioning and reduced cardiac inotropism due to iron deposition. Only 15/54 (27.8%) TM patients used to perform vigorous physical activity. These results suggest that a program of regular physical activity may be useful to increase the tolerance to effort and therefore to improve the quality of life in these patients.

Characteristics of 1573 healthcare workers who underwent nasopharyngeal swab testing for SARS-CoV-2 in Milan, Lombardy, Italy.

OBJECTIVES: The management of healthcare workers (HCWs) exposed to confirmed cases of coronavirus disease 2019 (COVID-19) is still a matter of debate. We aimed to assess in this group the attack rate of asymptomatic carriers and the symptoms most frequently associated with infection. METHODS: Occupational and clinical characteristics of HCWs who underwent nasopharyngeal swab testing for the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a university hospital from 24 February 2020 to 31 March 2020 were collected. For those who tested positive and for those who tested positive but who were asymptomatic, we checked the laboratory and clinical data as of 22 May to calculate the time necessary for HCWs to then test negative and to verify whether symptoms developed thereafter. Frequencies of positive tests were compared according to selected variables using multivariable logistic regression models. RESULTS: There were 139 positive tests (8.8%) among 1573 HCWs (95% confidence interval, 7.5-10.3), with a marked difference between symptomatic (122/503, 24.2%) and asymptomatic (17/1070, 1.6%) workers (p < 0.001). Physicians were the group with the highest frequency of positive tests (61/582, 10.5%), whereas clerical workers and technicians had the lowest frequency (5/137, 3.6%). The likelihood of testing positive for COVID-19 increased with the number of reported symptoms; the strongest predictors of test positivity were taste and smell alterations (odds ratio = 76.9) and fever (odds ratio = 9.12). The median time from first positive test to a negative test was 27 days (95% confidence interval, 24-30). CONCLUSIONS: HCWs can be infected with SARS-CoV-2 without displaying any symptoms. Among symptomatic HCWs, the key symptoms to guide diagnosis are taste and smell alterations and fever. A median of almost 4 weeks is necessary before nasopharyngeal swab test results are negative.

Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia.

BACKGROUND: Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary dyskinesia (PCD), a rare genetic disease due to malfunctioning of motile cilia. Chronic infections and persistent inflammation of the respiratory system result in progressive lung disease. Aim of the study was to highlight the main factors associated with clinical, functional and anatomical deterioration in PCD patients. METHODS: We retrospectively analyzed data from 58 patients with PCD, 37 adults and 21 children. The demographic and clinical data, forced expiratory volume at 1 s (FEV1) and forced vital capacity (FVC), sputum microbiology and imaging results (chest CT scores-modified Bhalla) were recorded. Patients were stratified according to the number of exacerbations (< 2/year vs ≥ 2/year) and chronic Pseudomonas aeruginosa (PA) colonization. The possible correlations between lung function and chest CT scores were assessed; we also evaluated the correlation between these parameters and the severity scores for bronchiectasis (BSI, FACED and e-FACED). RESULTS: Chest CT scores showed a significant correlation with FEV1 (p = 0.0002), age (p <  0.0001), BMI (p = 0.0002) and number of lung lobes involved (p <  0.0001). PA colonization had an overall prevalence of 32.6%: no significant difference in FEV1 between PA colonized and non-colonized patients was found (p = 0.70), while chest CT score was significantly worse in chronic PA colonized patients (p = 0.009). Patients with a high number of exacerbation (≥ 2/year) were older (p = 0.01), had lower FEV1 (p = 0.03), greater number of lobes involved (p < 0.001) and worse CT score than patients with low number of exacerbations (p = 0.001); they also had higher prevalence of PA chronic bronchial infection (33.3% versus 13.6%, p = 0.10). Multivariable linear regression analyses adjusted for gender, age and BMI showed positive associations between PA colonization and number of exacerbations with severity of disease (number of lobes involved, CT score, BSI, FACED, and e-FACED). CONCLUSIONS: In our PCD population the number of exacerbations (≥ 2/year) and PA colonization were the two most relevant factors associated with severity of disease.

A bio-psycho-social approach in elderly population: outcome of adapted physical activity in patients with osteoarthritis.

BACKGROUND: The beneficial effect of physical activity on a large spectrum of diseases is well known, with particular importance for elderly people. Among the different types of activity, adapted physical activity (APA) has been applied in a number of disease-related physical deficit. OBJECTIVE: The purpose of this study is to determine the outcome of a six months APA program in elder patients with osteoarthritis concerning physical and functional health and as second endpoint to determine the potential effect of AFA on reducing the risk of institutionalization. METHODS: The clinical indexes used to assess the outcome included the Blaylock Risk Assessment Screening Score (BRASS), the Psychological General Well Being Index (PGWBI), the Cumulative Illness Rating Scale (CIRS), the Short Physical Performance Battery (SPPB), and the visual analogue scale (VAS). RESULTS: A significant difference (p = 0.047) between the pre-APA and the post-APA value was found for BRASS, and highly significant differences (p <0.0001) were found for SPBB and VAS. CONCLUSIONS: These findings show that a six months APA program in elder patients with osteoarthritis improves physical function, reduces pain intensity and decreases the risk of institutionalization. Moreover the positive outcome of APA we found in elder patients with osteoarthritis suggests a more frequent use of such rehabilitation approach, also evaluating its economic impact on this disease.

The effect of delayed umbilical cord clamping on cord blood gas analysis in vaginal and caesarean-delivered term newborns without fetal distress: a prospective observational study.

OBJECTIVE: To determine variations in cord blood gas (CBG) parameters after 3-minute delayed cord clamping (DCC) in vaginal deliveries (VDs) and caesarean deliveries (CDs) at term without fetal distress. DESIGN: Prospective observational study. SETTING: University hospital. SAMPLE: CBG from 97 VDs and 124 CDs without fetal distress. METHODS: Comparison of paired arterial-venous CBG parameters drawn at birth from the unclamped cord and after 3-minutes DCC for VDs and CDs. MAIN OUTCOME MEASURES: Base excess, bicarbonate, haematocrit and haemoglobin from both arterial and venous cord blood, lactate, neonatal outcomes, partial pressure of oxygen (pO2 ), partial pressure of carbon dioxide (pCO2 ), pH, and postpartum haemorrhage. RESULTS: Arterial cord blood pH, bicarbonate ( HCO3- , mmol/l), and base excess (BE, mmol/l) decreased significantly after 3-minute DCC both in VDs (pH = 7.23 versus 7.27; P < 0.001; HCO3-  = 23.3 versus 24.3; P = 0.004; BE = -5.1 versus -2.9; P < 0.001) and CDs (pH = 7.28 versus 7.34; P < 0.001; HCO3-  = 26.2 versus 27.2; P < 0.001; BE = -1.5 versus 0.7; P < 0.001). After 3-minute DCC, pCO2 increased in CDs only (57 versus 51; P < 0.001), whereas lactate increased more in CDs compared with VDs (lactate, +1.1 [0.9, 1.45] versus +0.5 [-0.65, 2.35]; P = 0.01). Postpartum maternal haemorrhage, neonatal maximum bilirubin concentration, and need for phototherapy were similar between the two groups. Newborns born by CD more frequently required postnatal clinical monitoring or admission to a neonatal intensive care unit. CONCLUSIONS: After 3-minute DCC, the acid-base status shifted towards mixed acidosis in CDs and prevalent metabolic acidosis in VDs. CDs were associated with a more pronounced increase in arterial lactate, compared with VDs. TWEETABLE ABSTRACT: By 3-minute DCC, acid-base status shifts towards mixed and metabolic acidosis in caesarean and vaginal delivery, respectively.

Drug-Induced Hyponatremia: NSAIDs, a Neglected Cause that Should Be Considered.

Hyponatremia is the most common electrolyte disorder. It may have serious consequences in asyntomatic patients with a mild disease. Therefore, an evaluation of unsual causes is of paramount importance. Polypharmacy is highly prevalent in older people and many drugs can cause hyponatremia as a collateral effect. In our retrospective analysis of geriatric medical records dated 2015 we found that 39 out of the 273 hospitalized patients had hyponatremia. Polipharmacy was highly prevalent, especially in hyponatremic patients. Non-steroidal anti-inflammatory drugs, which are seldom considered as a cause of hyponatremia were instead found to be associated to an increased risk of the disorder (adjustedOR 3.61, 95% CI 1 - 12.99, p = 0.05). In-hospital mortality was higher in patients with moderate or severe hyponatremia at hospital admission. Our study underlines the importance of considering rare but potentially reversible causes of hyponatremia, which can lead to serious consequences.

Hair as a matrix to evaluate cumulative and aggregate exposure to pesticides in winegrowers.

INTRODUCTION: Vineyard is a crop where a large number of pesticides are applied; exposure to pesticides may occur in farmers and the general population living close to the treated area. This work aimed to investigate hair as a matrix for the assessment of cumulative and aggregate exposure to pesticides in potentially exposed individuals. METHODS: Twenty agricultural workers (AW), 4 agricultural worker relatives (AR), and 5 research staff members (RS) were involved in the study. Hair samples were collected before and after the application season (PRE- and POST-EXP samples) to obtain 18 paired samples. Records with the name and the quantity of applied pesticides were obtained; twenty-seven pesticides were measured in hair by solvent extraction and LC-MS/MS. RESULTS: During the study season, AW applied 14 different pesticides with median amount ranging from 12 to 7200 g. The most popular pesticides were dimethomorph, penconazole, cyazofamid, fenamidone and quinoxyfen, applied from 94 to 69% of AW. In AW, in PRE-EXP samples the majority of used pesticides was detectable (with detection rates from 6 to 88%), with median concentrations of few pg/mg hair; in the POST-EXP samples the frequency of detected values increased (from 25 to 100%), with median concentrations up to two orders of magnitude higher. In AR, most pesticides were quantifiable only in POST-EXP samples and with lower concentration in comparison with AW; in RS, in both PRE- and POST-EXP samples only a few pesticides were quantifiable with very low levels. In AW, a linear correlation (r = 0.682 on log-transformed data, p < 0.01) was found between the total amounts of applied pesticides during the season and their concentration in hair. CONCLUSION: The study shows that the majority of assessed pesticides was incorporated into hair of AW and AR. The increased frequency of detection and level at the end of the season and the correlation between pesticide in hair and the amount of applied pesticides, reinforce the use of hair for quantitative biomonitoring of cumulative exposure to pesticides.

Voiding cystourethrography and 99MTC-MAG3 renal scintigraphy in pediatric vesicoureteral reflux: what is the role of indirect cystography?

BACKGROUND: Primary vesicoureteral reflux (VUR) is the most common urological anomaly in children. Voiding cystourethrography (VCUG) is considered the reference standard for the diagnosis of VUR. Even if it is a secure and standardized technique, it is still an invasive method, hence, the effort to find an alternative method to diagnose VUR. The aim of the study is to evaluate the diagnostic accuracy of 99mTC-MAG3 scintigraphy with indirect cystography in detecting VUR and to estimate any interobserver variability in 99mTC-MAG3 scintigraphy interpretation. METHODS: The authors retrospectively reviewed all the pediatric patients who underwent both a VCUG and a 99mTC-MAG3 renal scintigraphy at the study institution between 2012 and 2016. RESULTS: A total of 86 children (and 168 renal units) were included. MAG3 scan revealed a sensitivity of 54% and a specificity of 90% with positive predictive value of 79% and negative predictive value of 73%. Each MAG3 scintigraphy was then independently and blindly evaluated by a pediatric urologist and two nuclear physicians. After revision, the concordance between VCUG and MAG3 in reflux cases dropped from 54% to 27% (on average), and the reviewers reclassified most examinations as non-conclusive. CONCLUSIONS: 99mTC-MAG3 renal scintigraphy with indirect cystography showed low sensitivity in detecting VUR of any grade and cannot, therefore, be proposed as completely alternative to VCUG in the diagnosis of VUR. Moreover, MAG3 scintigraphy interpretation for the diagnosis of VUR has a very high interobserver variability, mostly because of the lack of a correct and complete voiding phase.

Clinical outcomes of Clostridium difficile infection according to strain type. A prospective study in medical wards.

OBJECTIVES: To describe clinical characteristics and outcome of Clostridium difficile infection (CDI) patients in Internal Medicine, to identify ribotypes (RTs); to evaluate the association between RT and patient clinical characteristics and report outcome. METHODS: One year prospective cohort study. Clinical data, Barthel Index (BI) and outcomes were collected for all inpatients suffering from CDI (n = 148) in hospital wards in Northern Italy. 84 fecal samples were analysed for molecular typing. RESULTS: 12 RTs were identified, predominantly RT018 (42.9%, n = 36/84) and RT356/607 (40.5%, n = 34/84). Patients with dementia were more frequent among those infected by RT018 [55.6% (n = 20/36) vs. 32.4% (n = 11/34), p = 0.05]. The median BI score of patients with RT018 was lower than BI score of patients with RT356/607 [10 (IQR 0-32) vs. 15 (IQR 5-50), p = 0.06]. RT018 infection was associated to higher levels of C-reactive protein [7.2 mg/dl (IQR 4.1-14.7) vs. 4.0 mg/dl (IQR 2.2-6.8), p = 0.01] and white blood cells ≥15,000/dl [33.3% (n = 12/36) vs. 14.7% (n = 5/34) of patients, p = 0.07]. Higher mortality was noted among RT018 infected patients. We found a continuous mortality increase according to the ATLAS score. CONCLUSIONS: Our results confirm that RT018 and RT356/607 are the two major RTs causing CDI in older patients with a high degree of disability in Northern Italy and RT018 is associated with more serious outcomes.

Could seasonal allergy be a risk factor for acute rhinosinusitis in children?

OBJECTIVE: To evaluate the incidence of acute rhinosinusitis in children with grass pollen induced rhinitis during the period of grass pollinosis. METHODS: Children with nasal symptoms from grass pollen induced rhinitis but without rhinosinusitis symptoms were selected. Their parents were asked to complete a diary during pollen exposure to report nasal symptoms and drugs used daily. When rhinosinusitis was suspected, the confirmatory diagnosis of acute rhinosinusitis was made by fibro-endoscopy. Children without inhalant allergy served as controls. RESULTS: Seventeen out of 242 children (7.0 per cent) had a diagnosis of acute rhinosinusitis, confirmed by fibro-endoscopy, during grass pollination, compared to 3 out of 65 (4.6 per cent) in the control group (p = 0.49). Among allergic children, those with acute rhinosinusitis had symptoms for a greater number of days and/or a higher symptoms score than children without acute rhinosinusitis. CONCLUSION: Children with grass pollen induced rhinitis during exposure to pollen have an incidence of endoscopically confirmed acute rhinosinusitis comparable to non-allergic children. This suggests that grass pollen induced rhinitis is a negligible risk factor for acute rhinosinusitis.

Increased risk of placental abruption after solomon laser treatment of twin-twin transfusion syndrome.

To evaluate the relative risk of placental abruption in monochorionic (MC) twin pregnancies complicated with twin-to-twin transfusion syndrome (TTTS) and treated with endoscopic laser coagulation of placental vessels (ELCPV). A retrospective analysis from January 2004 and December 2015 of 373 TTTS pregnancies, treated with selective ELCPV until January 2012 (287 cases), after which the Solomon technique was introduced (86 cases), compared with 243 normal MC pregnancies. A significant improvement in perinatal survival was observed after the introduction of the Solomon technique when compared to the selective procedure (77% vs 54%, p < 0.001). The rate of placental abruption was 1% (3/243) in normal MC pregnancies, 6% (21/373) in TTTS group, increased with Solomon technique (12/86, 14%, vs 9/287, 3%, p < 0.001). MC twin pregnancies treated with laser coagulation of placental vascular anastomoses could be at increased risk of placental abruption, especially when the Solomon technique is used.

Fine airborne particles: when alarming levels are the standard.

OBJECTIVES: To quantify the contribution of each individual month to the annual mortality burden attributable to particulate matter (PM) in 2015 in Milan, Italy, after authorities and media considered December 2015 as an outlying month carrying an exceptional population exposure to PM. STUDY DESIGN: We used routinely available daily time series of air pollution and mortality to perform an assessment of the impact of PM exposure on population health. METHODS: By combining daily death counts with daily PM levels, as well as the yearly average of the number of deaths with the yearly average of PM concentrations, impact estimates were calculated in terms of deaths attributable (AD) to levels of PM10 and PM2.5 exceeding the daily or the annual European Union (EU) exposure limits. RESULTS: On a monthly basis, the estimated AD for exceeding the daily EU limits for more than 35 days were 18.4 (PM10) and 33.2 (PM2.5) between January and March, and 20.0 and 31.9 between October and December, respectively. On an annual basis, the EU limit for PM10 was almost met and, therefore, the estimated impact in terms of AD was practically null. CONCLUSIONS: Impact results should be interpreted in the light of the skewness of the daily PM concentration distribution. The number of days above the limits is more important than the average annual concentration in determining the number of attributable deaths. The impact of PM on mortality is substantial during the whole winter season irrespective of its annual average concentration. Our estimates further stress the need for a revision of the current European air quality standards.

Selective intrauterine growth restriction in monochorionic twins: changing patterns in umbilical artery Doppler flow and outcomes.

OBJECTIVES: To describe changes in umbilical artery (UA) Doppler flow in monochorionic diamniotic (MCDA) twins affected by selective intrauterine growth restriction (sIUGR), to correlate Doppler findings with pregnancy course and perinatal outcome, and to report postnatal follow-up. METHODS: This was a retrospective study of 140 MCDA twins with sIUGR. UA end-diastolic flow, defined as Doppler waveform pattern Type I (persistently positive), Type II (persistently absent or persistently reversed) or Type III (intermittently absent or intermittently reversed), was recorded at first examination and monitored weekly until double or single intrauterine fetal death (IUFD), bipolar cord coagulation or delivery. All neonates had an early neonatal brain scan, magnetic resonance imaging, when indicated, and neurological assessment during infancy. Rates (per 100 person-weeks) and hazard ratios (HR) of IUFD in the IUGR twin in each pregnancy were calculated considering UA Doppler pattern as a time-dependent variable. RESULTS: At first examination, there were 65 cases with UA Doppler waveform pattern Type I, 62 with Type II and 13 with Type III. Of the 65 Type-I cases, 48 (74%) remained stable, while 17 (26%) changed to either Type II absent (14%), Type II reversed (9%) or Type III (3%). Of 62 Type-II cases (47 with absent and 15 with reversed flow), 33 (53%) remained stable (18 with absent and all 15 with reversed flow). The 29 Type-II absent cases which changed became Type II reversed (24/47, 51%) or Type III (5/47, 11%). All 13 Type-III cases remained stable. Compared with Type I, the risk of IUFD (adjusted for estimated fetal weight discordance and amniotic fluid deepest vertical pocket) was highest when the pregnancy was or became Type II reversed (HR, 9.5; 95% CI, 2.7-32.7) or Type II absent (HR, 4.3; 95% CI, 1.3-14.3). Mild neurological impairment was more prevalent in the IUGR twin than in the large cotwin (7% vs 1%, P = 0.02). CONCLUSIONS: Risk stratification based on UA Doppler is useful for planning ultrasound surveillance. However, patterns can change over time, with important consequences for management and outcome. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Clostridium difficile infection epidemiology and management: Comparison of results of a prospective study with a retrospective one in a reference teaching and research hospital in Northern Italy.

BACKGROUND: Clostridium difficile-associated disease (CDAD) is the most common infectious antibiotic-associated diarrhea and is a growing health care problem. Prevention of Clostridium difficile infection focuses on clinical and epidemiologic infection control measures. METHODS: Between 2008 and 2009, we conducted a retrospective study that showed an incidence of CDAD among the highest reported in the literature. Subsequently, we developed a preventive protocol that was adopted in our hospital in 2010. We then conducted a prospective study to investigate prevalence, incidence, and mortality of CDAD and to compare the results with those of the retrospective study, evaluating adherence to preventive measures and their efficacy. RESULTS: In both studies, prevalence and incidence significantly increased in older patients. Crude prevalence was similar in the 2 studies. The incidence rate increased by 36%, with a significant increase only in the C and D wards. In-hospital mortality rose in both prevalent and incident cases. Regarding adhesion to hospital protocol, 77% of prevalent cases were treated with the required procedure. The highest percentage of isolated patients was achieved in C and D wards. In these wards we detected lower training hours per nurse. However, in 2013, we observed a significant decrease in incidence of CDAD and found a hospital prevalence of 0.33%. CONCLUSIONS: Health care personnel education could be more important than the possibility of isolating infected patients in single rooms.

Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study.

BACKGROUND AND PURPOSE: To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. METHODS: Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up. RESULTS: Our study on 545 incident cases confirmed that King's College stages occurred at predictable times and were quite evenly spaced out throughout the disease course (occurring at approximately 40%, 60% and 80% of the disease course), whereas MITOS stages were mostly skewed towards later phases of the disease. In the King's College system there was a decrease in survival and an increase in deaths with escalating stages, whereas in the MITOS system survival curves pertaining to intermediate stages overlapped and the number of deaths was fairly homogenous throughout most stages. CONCLUSIONS: The King's College staging system had a higher homogeneity (i.e. smaller differences in survival among patients in the same stage) and a higher discriminatory ability (i.e. greater differences in survival among patients in different stages), being more suitable for individualized prognosis and for measuring efficacy of therapeutic interventions.

Sustained lung inflation in late preterm infants: a randomized controlled trial.

OBJECTIVE: To assess the need for respiratory support in late preterm infants treated with sustained lung inflation (SLI) at birth. STUDY DESIGN: In this controlled trial, we randomly assigned infants born at 34(+0) to 36(+6) weeks of gestation to receive SLI (25 cmH2O for 15 s) at birth, followed by continuous positive airway pressure (CPAP) or assistance according to the recommendations of the American Academy of Pediatrics. The primary outcome was the need for any type of respiratory support. The secondary outcomes included neonatal intensive care unit (NICU) admission for respiratory distress and length of stay. The risk ratios (RRs) and 95% confidence intervals (CIs) of the outcomes were calculated for the SLI group in reference to the control group. RESULTS: A total of 185 infants were enrolled: 93 in the SLI group and 92 in the control group. No difference was found in the need for any type of respiratory support between the infants treated with SLI and the control group (10.6 vs 8.7%, RR 1.24, 95% CI 0.51 to 2.99). The NICU admission for respiratory distress and the length of stay did not differ between the groups. CONCLUSION: Providing SLI at birth in late preterm infants does not affect their need for respiratory support.