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.

Activities of a medical emergency team twenty years after its introduction.

INTRODUCTION: We describe and quantify the wide range of activities that a mature Medical Emergency Team can progressively perform. METHODS: The activities performed by a Medical Emergency Team 20 years after its introduction were prospectively collected during 105 consecutive days. RESULTS: The main activity was focused on the follow-up visits to previously treated critically ill patients (mean 7.5 visits/die in working days, 5.1 in the others). A large amount of other scheduled or unscheduled activities (like sedation or analgesia for diagnostic procedures, central venous line placement, phone consultation regarding critical care aspects of treatments) were performed: on average, 7.3 side-activities/die in working days and 5.2 in the others. First consultations in patients not previously seen were on average 3.1/die on working days, 2.4 in the others. Cardiac arrest accounted for 27 (9%) of first time visits. CONCLUSIONS: A Medical Emergency Team can progressively perform many kinds of activities. An evaluation limited to the reduction of in-hospital cardiac arrests or a too early assessment may underestimate its beneficial effects on the Hospital complexity.

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.

Use of protein C concentrate in pediatric patients with sepsis.

AIM: Protein C (PC) is a plasma glycoprotein implicated in modulating coagulation and inflammation. Its levels decrease in sepsis and related diseases, where it has also proved to be a prognostic indicator of outcome. Infusion of exogenous PC, although not able to decrease mortality in severe sepsis and septic shock, can safely resolve the coagulation imbalances related to these pathological states. METHODS: A retrospective study was performed about utilisation of PC in severe sepsis and septic shock patients in three italian PICUs during a one-year period. Data from 29 patients were analysed. Age, PIM 2, mortality and length of stay were compared between treated and non treated patients. Treated patients were also analysed for PC dosage received, length of treatment, and modification of hemocoagulation parameters, before PC infusion and every 24 hours. RESULTS: In treated patients, the activity of PC, PT and PTT activity and fibrinogen improved significantly from basal to day 5 (p<0.05). Diminution of d-dimer was not quite significant (p=0.0514). Rise in platelets count and antithrombin III activity was not significant. No adverse reactions related to Protein C concentrate were observed. No difference in mortality was observed between the two groups. CONCLUSIONS: Although PC is included in guidelines for management of severe sepsis and septic shock, only 38%, of observed patients received PC treatment. Even in the treated group, patients received a lower dosage of PC, and for a shorter period, than recommended. In accordance to previous studies, we did not observe differences in mortality between treated and untreated patients. Our results showed a significant increase in plasma PC activity, following infusion of PC concentrate. This increase in PC appeared sufficient to restore some, but not all, of the abnormalities in the coagulation system. A large randomized, phase 3, placebo-controlled trial in children with severe sepsis and septic shock is advisable to establish effective role of therapy with PC in reducing mortality of these patients.

[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.

Emergency endovascular treatment of a life-threatening hemorrhage from traumatic rupture of the left extracranial vertebral artery.

Hemorrhagic complications from transection of cervical arteries in blunt traumas are rare. We report a case of potentially fatal hemorrhage from rupture of the left vertebral artery in a closed trauma, successfully treated by endovascular injection of glue. Endovascular embolization may be considered as an alternative to surgical exploration in the treatment of traumatic lesions of vertebral arteries.

[Streptococcus pyogenes toxic shock. A clinical case]. / Shock tossiemico da Streptococcus pyogenes. Caso clinico.

Toxic shock-like syndrome (TSLS) due to Streptococcus pyogenes has been recently reported in both children and adults. This syndrome is characterized by hypotension or shock, fever, multiorgan system involvement and death in 30 to 60% of patients. This syndrome closely resembles the more frequent staphylococcal TSLS. Only one case of TSLS caused by streptococcus has been reported, up to now, in our Country. We describe a second case of fatal streptococcus pyogenes TSLS in a 64-year-old man, in which the site of infection was in the soft tissues. The illness was characterized by rapid progression of shock, erythematous rash, multisystem organ involvement and finally death. Clinicians must be aware of the presentation of this disease as its incidence appears to be increasing.

[Conventional radiology and computerized axial tomography in the diagnosis of pneumothorax in intensive therapy. Retrospective study of 2 years of activity]. / Radiologia convenzionale e tomografia assiale computerizzata nella diagnosi di pneumotorace in terapia intensiva. Analisi retrospettiva di un biennio di attività.

The role played by chest radiograph and CT scan in diagnosis of pneumothorax is analyzed in a retrospective study based on two years of activity in Intensive Care Unit. The sensibility of traditional chest radiography was 75% while the sensibility of CT scan resulted higher (87.5%). In the authors opinion, when pneumothorax is suspected, particularly in patients with polytrauma, CT scan of the chest is mandatory unless patient's conditions or logistic difficulties related with transport of the patient contraindicate it.

[Claude Bernard-Horner syndrome following percutaneous catheterization of the jugular vein in an infant]. / Syndrome de Claude Bernard-Horner après cathétérisme percutané de la veine jugulaire interne chez un nourrisson.

A case of Horner's syndrome is reported in a 8 month-infant after ultrasound guided, percutaneous internal jugular cannulation. This syndrome receded spontaneously after resolution of a laterocervical hematoma caused by reiterated venipunctures performed during the cannulation. Ultrasound imaging, by providing vizualization of the internal jugular vein and surrounding structures, may help to localize the vein and decrease the incidence of complications. Considering this case report and the cases previously reported in literature, the authors conclude that paediatric patients are at a higher risk compared to adults to develop Horner's syndrome following percutaneous internal jugular cannulation.