Abnormalities of acid-base balance and predisposition to metabolic acidosis in Metachromatic Leukodystrophy patients.

Metachromatic Leukodystrophy (MLD; MIM# 250100) is a rare inherited lysosomal storage disorder caused by the deficiency of Arylsulfatase A (ARSA). The enzymatic defect results in the accumulation of the ARSA substrate that is particularly relevant in myelin forming cells and leads to progressive dysmyelination and dysfunction of the central and peripheral nervous system. Sulfatide accumulation has also been reported in various visceral organs, although little is known about the potential clinical consequences of such accumulation. Different forms of MLD-associated gallbladder disease have been described, and there is one reported case of an MLD patient presenting with functional consequences of sulfatide accumulation in the kidney. Here we describe a wide cohort of MLD patients in whom a tendency to sub-clinical metabolic acidosis was observed. Furthermore in some of them we report episodes of metabolic acidosis of different grades of severity developed in acute clinical conditions of various origin. Importantly, we finally show how a careful acid-base balance monitoring and prompt correction of imbalances might prevent severe consequences of acidosis.

Invasive and non-invasive long-term mechanical ventilation in Italian children.

BACKGROUND: To date, few studies have been published regarding the number of children in Italy who require long-term mechanical ventilation (LTV) and their underlying diagnoses, ventilatory needs and hospital discharge rate. METHODS: A preliminary national postal survey was conducted and identified 535 children from 57 centers. Detailed data were then obtained for 378 children from 30 centers. RESULTS: The estimated prevalence in Italy of this population was 4.3/100000. The majority of children (72.2%) were followed in pediatric units. The primary physicians who cared for these patients were either pediatric intensivists or pediatric pulmonologists. Neurological patients (78.2% of cases) represented the principal disorder category. 57.2% of the patients were non-invasively ventilated, with a nasal mask being the most common interface (85% of cases). The presence of clinical symptoms that were associated with abnormal findings on diagnostic testing was the primary indication for ventilatory support, whereas weaning failure was the primary indication for tracheotomy. Invasive ventilation was significantly related to younger age, longer daily hours on ventilation and cerebral palsy. Ventilatory modes with guaranteed minimal tidal volume were more often used in patients with tracheotomy. Despite their age, illness severity and need for technological care, 98% of the study population were successfully home discharged. CONCLUSION: Managing pediatric home LTV requires tremendous effort on the part of the patient's family and places a significant strain on community financial resources. In particular, neurological patients require more health care than patients in other categories. To further improve the quality of care for these patients, it is essential to establish a dedicated national database.

Long-term home ventilation of children in Italy: a national survey.

BACKGROUND: Improved technology, as well as professional and parental awareness, enable many ventilator-dependent children to live at home. However, the profile of this growing population, the quality and adequacy of home care, and patients' needs still require thorough assessment. OBJECTIVES: To define the characteristics of Italian children receiving long-term home mechanical ventilation (HMV) in Italy. METHODS: A detailed questionnaire was sent to 302 National Health Service hospitals potentially involved in the care of HVM in children (aged <17 years). Information was collected on patient characteristics, type of ventilation, and home respiratory care. RESULTS: A total of 362 HMV children was identified. The prevalence was 4.2 per 100,000 (95% CI: 3.8-4.6), median age was 8 years (interquartile range 4-14), median age at starting mechanical ventilation was 4 years (1-11), and 56% were male. The most frequent diagnostic categories were neuromuscular disorders (49%), lung and upper respiratory tract diseases (18%), hypoxic (ischemic) encephalopathy (13%), and abnormal ventilation control (12%). Medical professionals with nurses (for 62% of children) and physiotherapists (20%) participated in the patients' discharge from hospital, though parents were the primary care giver, and in 47% of cases, the sole care giver. Invasive ventilation was used in 41% and was significantly related to young age, southern regional residence, longer time spent under mechanical ventilation, neuromuscular disorders, or hypoxic (ischemic) encephalopathy. CONCLUSIONS: Care and technical assistance of long-term HMV children need assessment, planning, and resources. A wide variability in pattern of HMV was found throughout Italy. An Italian national ventilation program, as well as a national registry, could be useful in improving the care of these often critically ill children.

Epidural analgesia with ropivacaine and sufentanil is associated with transient fetal heart rate changes.

AIM: Fetal heart rate (FHR) changes have been reported after regional labor analgesia. In this prospective single-blinded study, we aimed to assess whether epidural analgesia with ropivacaine and sufentanil is associated with significant changes in fetal heart rate. METHODS: Fetal heart rate traces from 120 women in active labor requesting epidural analgesia were recorded and analyzed by two reviewers 90 minutes before and after epidural analgesia for baseline fetal heart rate, accelerations, decelerations and long-term variability. RESULTS: A significantly decreased number of fetal heart rate accelerations (ANOVA P=0.0001) and a higher percentage of segments with decelerations (P<0.05) were observed in the three segments after analgesia as compared to the three preceding segments. The minimum number of accelerations occurred during the 30 minutes immediately after analgesia was initiated. The reviewers were concordant in finding a significant change from the 60 minutes before to the 60 minutes after analgesia, a period in which there CONCLUSION: Epidural analgesia with ropivacaine and sufentanil is associated with fetal heart rate changes. These modifications are transient and should be considered when evaluating fetal heart rate monitoring during labor to prevent inappropriate obstetric management decisions to proceed with operative labor.

Promoting epidural analgesia for labor: 2005-2007 diffusion in Lombardia, Italy.

BACKGROUND: Since January 2005 the Regional Government of Lombardia, a large Italian region with over 1/5 of all Italian births, allocated public funds for 3 consecutive years to help provide epidural analgesia (EA) for women in labor. The aim of the present study was to evaluate the trend of diffusion of EA in the triennium 2005-2007. METHODS: Data obtained from regional Obstetric Departments, recognized by the National Health Care System, were elaborated by the Epidemiological Service of Regione Lombardia. The software looked for specific codes for vaginal deliveries, with or without EA, and Cesarean sections included in the administrative patient records. RESULTS: A substantial increase in epidurals administered in comparison to total vaginal deliveries was recorded after assignment of regional financing: from 8.2% in 2005, to 10.4% in 2006 and 12.9% in 2007 (P<0.0001). More than 60% of epidurals were performed in 8 hospitals with >2 000 births per year. The rate of EAs in these hospitals was 18% in 2005, 22% in 2006 and 24.9% in 2007. In the 69 hospitals with <2000 births per year, the rate of EAs was markedly lower: 4% in 2005, 5.5% in 2006 and 7.8% in 2007. In both cases, the increase was statistically significant (P<0.0001). At the three-year time-point, the rate of Cesarean sections did not change. CONCLUSIONS: The continuous increase of EA for labor after regional financings suggests that the low rate of pain relief procedures in Lombardia was mainly due to economic and organizational issues, rather than to cultural and psychological factors.

Caudal anesthesia in pediatrics: an update.

AIM: Caudal anesthesia is one of the most used-popular regional blocks in children. This technique is a useful adjunct during general anesthesia and for providing postoperative analgesia after infraumbilical operations. The quality and level of the caudal blockade is dependent on the dose, volume, and concentration of the injected drug. Although it is a versatile block, one of the major limitations of the single-injection technique is the relatively short duration of postoperative analgesia. The most frequently used method to further prolong postoperative analgesia following caudal block is to add different adjunct drugs to the local anesthetics solution. Only few studies evaluated quality and duration of caudal block against the volume of the local anaesthetic applied. After reviewing recent scientific literature, the authors compare the duration of postoperative analgesia in children scheduled for hypospadia repair when 2two different volumes and concentrations of a fixed dose of ropivacaine are used. METHODS: After informed parental consent, 30 children (ASA I, 1-5 years old) were enrolled in a multicentre, perspective, not randomized, observational study conducted in two 2 children hospitals. After premedication with midazolam, anesthesia was induced with thiopental and maintained with sevoflurane in oxygen/air. After induction, patients received a caudal blockade either with ropivacaine 0.375% at 0.5 mL/kg (Low Volume High Concentration Group, LVHC; n = 15), or ropivacaine 0.1% at 1.8 mLl/kg (High Volume Low Concentration Group, HVLC; n = 15). Surgery was allowed to begin 10ten minutes after performing the block. MAC-hour was calculated. In the recovery room, pain was assessed using the Children's Hospital of Eastern Ontario Pain Scale (CHEOPS). In addition, the motor block was scored. After transferral to the ward, the patients were observed for 24 hours for signs of postoperative pain. The time period to first supplemental analgesic demand, i.e., from establishment of the block until the first registration of a CHEOPS score = or > 9, was considered the primary endpoint of the study. The time periods were compared using analysis of variance adjusted for age, weight and duration of surgical procedure as covariates. RESULTS: All patients were judged to have sufficient intraoperative analgesia, and none of them received additional analgesics intraoperatively. Patients' characteristics were similar, besides the age (32+/-10 vs 24 +/- 9 months; P < 0.05) and weigh (15.13 +/- 3.92 vs 11.93 +/- 1.83; P = 0.08). Analgesics were needed after 520 +/- 480 min in the LVHC and 952 +/- 506 min in the HVLC group (P < 0.05). Motor block was less in the HVLC group. CONCLUSIONS: In children undergoing hypospadia repair, caudal block with a ''high volume, low concentration'' regimen produces prolonged analgesia and less motor block, compared to a ''low volume, high concentration'' regimen.

[Sedation with magnetic resonance imaging in pediatric patients]. / Tracheostomia in età pediatrica.

219 children (ASA I-II, age 30 days-12 yrs), underwent deep sedation with intravenous thiopental for magnetic resonance imaging in a hospital setting. Sedation strategies and monitoring are described. The procedure showed to be safe for the patients and cause low artefacts by movements.

[Cost of ICU in Italy. Results from an empirical study on a sample of 12 hospitals]. / Il costo del reparto di terapia intensiva in Italia. Risultati da un'indagine empirica su un campione di 12 centri.

BACKGROUND: The Italian hospital payment system based on DRG doesn t properly include Intensive Care Units (ICU) costs. Since great emphasis has been recently given to rationing health care resources, assessing ICU costs seems to be dramatically relevant. Aim of the study was to assess the average yearly cost and the cost per diem of a sample of Italian multispecialistic ICU wards. METHODS: In September 1995, a questionnaire concerning data on variable and fixed cost was sent to 25 Italian ICU wards, 11 NHS hospital-based (Northern Italy: 5; Central Italy: 4; Southern Italy: 2) and 14 school of medicine-based (Northern Italy: 7; Central Italy: 5; Southern Italy: 2). Variable cost data included: disposable, drugs, blood and blood-derived products, physical tests, chemical and microbiological routines, instrumental diagnostic procedures and physiotherapy. Concerning fixed costs, data on personnel and equipment were requested. In addition, some hospital overheads data (utilities; power; heating; maintenance; cleaning; laundry; accounting; waste disposal; cafeteria) were collected. RESULTS: On the basis of the 12 questionnaires returned (Northern Italy: 9; Central Italy: 3; Southern Italy: 0), the yearly cost of an ICU ward is Liras 4,580,032,000 (range 2,739,277,000-7,704,292,000), whereas the average cost per diem is Liras 1,802,000 (range 1,234,000-3,179,000). Cost of personnel is about 61% of the above mentioned costs. CONCLUSIONS: Despite the lack of questionnaires from Southern Italy and the unavailability of some data concerning both the cost of equipment and the overheads, the remarkable average cost values obtained could support further research.

Anaesthesia with desflurane-nitrous oxide in elderly patients. Comparison with isoflurane-nitrous oxide.

BACKGROUND: Desflurane is a new volatile anaesthetic, very little soluble. We wished to compare efficacy, safety, and emergence and recovers; profiles of desflurane-N2O versus isoflurane-N2O anaesthesia in elderly patients. EXPERIMENTAL DESIGN: single blind, prospective randomised study. SETTING: operating rooms of two major teaching hospitals affiliated with the University of Milan. PATIENTS: fifty-seven patients ASA physical status II or III, aged 65 or older, undergoing urological (non-endoscopic), orthopaedic or gynaecological (non-laparoscopic) surgery of at least one hour duration, were randomly assigned to receive general anaesthesia with either desflurane or isoflurane in 60% N2O- 40% O2, after standardised premedication and induction. MEASUREMENTS: vital signs, end-tidal agent, narcotic requirement, and adverse event appearance were monitored throughout the study. RESULTS: Twenty-eight patients received desflurane and 29 isoflurane. Demographics, anaesthesia duration and exposure, and intraoperative fentanyl requirement were comparable in the two groups. Immediate emergence from anaesthesia (time to extubation and hand grip on command) was faster in desflurane group, albeit not significantly (8.4 +/- 6.4 vs 11.0 +/- 6.5 min and 8.6 +/- 6.0 vs 11.8 +/- 6.0); on the contrary, early recovery (time to state the name and date of birth) was significantly shorter in patients receiving desflurane (11.1 +/- 6.2 vs 17.3 +/- 7.8 min and 13.1 +/- 6.0 vs 20.9 +/- 10.9 min). Only 24 patients (12 in desflurane and 12 in isoflurane group) did need postoperative fentanyl administration; among them, requirement was significantly higher in desflurane patients (3.4 +/- 1.1 vs 2.4 +/- 1.3 micrograms.kg-1. Total time in recovery room was not different between anaesthetics, as well as adverse event prevalence and severity. CONCLUSIONS: Early recovery in elderly patients is faster after desflurane than isoflurane anaesthesia; this might contribute to increased requirement of postoperative analgesia. Occurrence of adverse event is comparable between the two anaesthetics.

[One day anesthesia in pediatrics]. / One day anestesia in età pediatrica.

The paper includes a short review on paediatric anaesthesia in day surgery. It discussed on why day surgery in children is so popular since the beginning of the century; the Italian laws on this item and the guidelines of the Italian Society of Anaesthesiology on "day surgery" and "preoperatives of children".

Arterial to end-tidal carbon dioxide gradient and physiological dead space monitoring during general anaesthesia: effects of patients' position.

METHODS: One hundred and five ASA I-II patients, scheduled for elective surgical procedures were studied in order to evaluate the effect of different surgical postures on physiological pulmonary dead space (VDphys/ VT) and arterial to end-tidal carbon dioxide gradient [P(a-Et)CO2]. Patients were divided into four groups according to their position on the operating table: supine position (acting as control group, n = 33), 20 degree Trendelenburg position (n = 24), lateral position (n = 24) and prone position with convex saddle frame (n = 24). Physiologic dead space was measured using Enghoff modification of Bohr equation. Arterial CO2 partial pressure was measured by blood gas analysis and end tidal CO2 was measured by means of an infrared CO2 analyser. All measurements were performed 20 minutes after general anaesthesia induction, with patients mechanically ventilated by a constant inspiratory flow (TV = 8 ml kg-1, RR = 10-14, EIP = 10%) in order to reach a steady state end tidal CO2 ranging between 32 and 36 mmHg; afterwards surgery started. RESULTS: Arterial blood pressure showed a mean decrease of about 5-10% compared to baseline values, but no significant differences in arterial pressure decrease were found between the four groups. A significant VDphys/VT increase in postures other than supine was observed, unless it was statistically significant in lateral and prone position only; while P(a-Et)CO2 was higher in all postures compared to supine. Changes of intrapulmonary gas and blood distribution due to patients' posture are probably responsible for the observed physiologic dead space and CO2 gradient differences. CONCLUSIONS: In conclusion, the clinical practice of predicting PaCO2 from EtCO2 must be tempered by recognition of the potential magnitude of P(a-Et)CO2 gradient, which is higher than normal during general anaesthesia and further increased when positioning the patient other than supine.

The Italian SEPSIS study: preliminary results on the incidence and evolution of SIRS, sepsis, severe sepsis and septic shock.

This prospective, multicenter, epidemiological study was carried out in 99 Italian ICUs, distributed throughout the country, from April 1993 to March 1994. In the study, we applied the new ACCP/SCCM classification system for sepsis (SIRS, sepsis, severe sepsis and septic shock) and determined the prevalence, incidence, evolution and outcome of these categories in critically ill patients. The preliminary analysis of 1101 patients showed that on admission SIRS accounted for about half of the diagnoses (52%) with sepsis, severe sepsis and septic shock accounting for 4.5%, 2.1% and 3% of patients, respectively. Patients with severe sepsis or septic shock more frequently had high SAPS scores than patients without sepsis. Mortality rates were similar in patients with SIRS (26.5%) and without SIRS or infection (24%), but rose to 36% in patients with sepsis, to 52% in those with severe sepsis and to 81.8% in those with septic shock. Sepsis, severe sepsis and septic shock were more common in patients with medical diagnoses, and neither severe sepsis nor septic shock was observed in trauma patients. With respect to evolution, the incidence of septic shock was progressively higher in patients admitted with more severe "sepsis-related" diagnoses, while only a trivial difference in rates of incidence was observed between SIRS patients and those admitted without SIRS or any septic disorder (nil). The breakdown of the various ACCP/SCCM "sepsis-related" diagnoses at any time during the study was: SIRS in 58% of the population, sepsis in 16.3%, severe sepsis in 5.5% and septic shock in 6.1%. It seems reasonable to expect from the final evaluation of our study answers to the questions raised by the ACCP/SCCM Consensus Conference about the correlations between "sepsis-related" diagnosis, severity score, organ dysfunction score and outcome.