Peripheral neuropathy in late-onset Krabbe disease: report of three cases.

Late-onset Krabbe disease may have variable misleading clinical manifestations and be a puzzling problem for physicians. We report clinical and peripheral nerve studies of three patients with adult-onset Krabbe disease. Two cases had a predominantly spastic paraparesis; in one case, the symptoms mimicked a cerebrovascular disorder. Predominantly, demyelinating neuropathy was observed in one case and axonal neuropathy in two cases. In all cases, no typical intracytoplasmic inclusions were found. These observations suggest that peripheral neuropathy in adult-onset Krabbe disease has variable clinical and pathological characteristics, different from those described in the classic form.

[Anesthesia in severely burnt patients: a 10-year experience]. / Anesthésie du grand brûlé: 10 années d'expérience.

A retrospective study of our anaesthesiologic activity during the decade 1982-1991 concerning 314 severely burned patients (53.4% of all the hospitalizations) submitted to escharrectomy with cutaneous grafts showed a greatest incidence in 1990 (19.5% of all the interventions) mostly for 12 to 60 year-old patients. In 51% of all cases, patients had burns over less than 30% of the body surface. 76% got a combined general anaesthesia mainly by isoflurane (68%) and 24%, especially 0 to 12 year-old patients (63.1%), a monopharmacological anaesthesia by ketamine.

[Severely burned patients and atracurium: the train-of-four response]. / Grands brûlés et atracurium: réponse au train de quatre.

Severely burned patients show a relative hyposensitivity to atracurium. The neuromuscular block obtained in 20 patients with major thermal injury after 0.75 mg.kg-1 i.v. of atracurium, by muscular activity recording (train of four-TOF), was studied. The results confirmed the resistance to atracurium, which mechanism is unknown. Even the doses administered, 50% higher than clinical dose (0.3-0.5 mg.kg-1), proved insufficient in order to obtain a complete neuromuscular block in severely burned patients.

Occupational therapy for advanced HIV patients at a home care facility: a pilot study.

Occupational therapy holds promise to increase quality of life and social functioning in patients with HIV infection. Since 2000 through 2005, we experimented a complex structured intervention including directly administered HAART, psychiatric support and occupational therapy for 14 patients with advanced HIV infection and multiple disabilities, cared for at an Italian home care facility. Social and occupational abilities were evaluated using the Axis V of DMS-IV, as assessed by the Global Assessment of Functioning Scale. Patients' abilities in coping with stressful situations were examined using the Social Dysfunction Rating Scale. Both outcomes were evaluated in interviews at study entrance and yearly thereafter. As compared to baseline, social function mean score significantly increased by 42% after one year of follow-up, and social stress mean score was significantly reduced by 11%. Both outcomes continued to improve constantly during the entire follow-up. Acceptance of the intervention was high, and three patients got outdoor job positions. The findings from this pilot study suggest that occupational therapy could be integrated with success in the treatment of severely disabled patients with advanced HIV infection. Confirmation from further research is required.

[Isoflurane: clinical experience in plastic surgery]. / Isoflurane: esperienza clinica in chirurgia plastica.

The authors' experience with Isoflurane, a volatile anesthetic agent recently introduced in Italy, is reported. A non-selected group of twenty patients, who underwent plastic surgery operations, represents the subject of this study. It is concluded that the clinical properties of Isoflurance are fully satisfactory with respect to the anesthestiological needs of plastic surgery.

[Intraoperative changes in blood cortisol and prolactin during surface surgery: totally intravenous anesthesia with propofol vs balanced anesthesia]. / Variazione perioperatorie della cortisolemia e prolattinemia in corso di chirurgia di superficie: anestesia totalmente endovenosa con propofol vs anestesia bilanciata.

Forty patients ASA class 1-2 admitted to elective plastic-reconstructive surgery were randomized in two groups of 20 patients to receive either total intravenous anaesthesia (TIVA) with propofol (group P) or balanced-anaesthesia with sodium thiopental + isoflurane + N2O (group M). 24 hours before surgery (t0) blood was drawn for cortisol and prolactin; this was repeated 30 minutes after skin incision (t1), 30 minutes after extubation (t2) and 24 hours after surgery (t3). Serum cortisol level rose, but not significantly, at t2 in group M. Serum prolactin level rose significantly at t1 and t2 in group M, at t1 in group P. By 24 hours after surgery, the cortisol and prolactin concentration in both groups was not significantly different from the baseline value.

[Control of the side effects of ketamine: chlordemethyldiazepam vs diazepam in pre-anesthesia]. / Controllo degli effetti collaterali della ketamina: clordemetildiazepam vs diazepam in preanestesia.

The pre-operative circulatory and psychotomimetic side-effects are studied in one hundred patients undergoing elective plastic-reconstructive surgery and anesthetized by ketamine 5-8 mg/kg i.m. Fifty patients were premedicated by atropine 0.01 mg/kg + CDDz 0.0285 mg/kg i.m. (group C), fifty by atropine 0.01 mg/kg + Dz 0.14 mg/kg i.m. (group D). All patients breathed spontaneously. Statistical analysis was performed with X 2 test. Not statistical difference was observed for the circulatory side-effects, whereas the frequency of emergence phenomena fell significantly from 31% to 14% (p less than 0.05) with CDDZ in greater than 16 years old patients. These finding seem confirm that CDDZ is more effective than DZ in reducing the psychotomimetic side-effects of ketamine, though it may be recommended a its wider experiment in this connection.

[Recovery time after general anesthesia with propofol administered in continuous infusion or with isoflurane]. / Tempi di risveglio dopo anestesia generale con propofol in infusione continua o con isoflurano.

The recovery time after Propofol-Tiva (P) or Isoflurane-anesthesia (I) for elective plastic surgery was studied by the Authors in two series of patients: 60 in group P, 60 in group I. All patients received a standard premedication and induction-anesthesia (premedication: Atropine 0.005 mg/kg i.m.; induction: Fentanyl 2.1 micrograms/kg+Propofol 2.5 mg/kg i.m. and Succinylcholine 1 mg/kg i.v. for tracheal intubation) and a different maintenance (group P received an infusion of 12 mg/kg/h for 10 min, 9 mg/kg/h for the next 10 min and 6 mg/kg/h thereafter+Fentanyl 1.4 micrograms/kg/h, group I clinically appropriate inspired concentration of Isoflurane and both Fentanyl-bolus at demand). They were ventilated mechanically with air and oxygen mixture (FiO2 = 0.3) to maintain normal blood-oxygen tension and normocarbia. The results showed that the recovery time was significantly shorter in the group P than in the group I and that Propofol-Tiva is accompanied by a more rapid recovery than Isoflurane-anesthesia.

[Chlordesmethyldiazepam and prolactinemia during surgical stress]. / Clordemetildiazepam e prolattinemia in corso di stress chirurgico.

The per-operative plasma prolactin levels in 16 male patients undergoing plastic-reconstructive surgery were studied. 8 patients received chlordesmethyldiazepam 0.1 mg/kg-1 i.v. (group C), 8 sodium thiopental 5 mg/kg-1 i.v. (Group T) in anaesthesia induction. All patients were premedicated by diazepam 0.15 mg/kg-1 by mouth 90 min. before anaesthesia induction and atropine 0.007 mg/kg-1 i.v. just before induction, and received succinylcholine 1 mg/kg-1 i.v. and an endotracheal tube for mechanically controlled ventilation. Anaesthesia maintenance was assured by isoflurane 0.5-2.5% in a N2/O2 (2/1) gas mixture. Blood samples were collected from each patient at the following times; 24 h before surgery (t0); 30 min after skin incision (t1); 30 min after extubation (t2). Significant variation of blood prolactin levels are not been shown.

[Effects of chlordemethyldiazepam on blood cortisol during surgical stress]. / Effetti del clordemetildiazepam sulla cortisolemia in corso di stress chirurgico.

The pre-operative plasma cortisol levels in 16 male patients undergoing plastic-reconstructive surgery were studied. 8 patients received chlordesmethyldiazepam 0.1 mg/kg-1 i.v. (group C), 8 sodium thiopental 5 mg/kg-1 i.v. (group T) in anaesthesia induction. All patients were premedicated by diazepam 0.15 mg/kg-1 by mouth 90 min before anaesthesia induction and atropine 0.007 mg/kg-1 i.v. just before induction, and received succinylcholine 1 mg/kg-1 i.v. and an endotracheal tube for mechanically controlled ventilation. Anaesthesia maintenance was assured by isoflurane 0.5-2.5% in a N2O/O2 (2/1) gas mixture. Blood samples were collected from each patient at the following times: 24 h before surgery (t0); 30 min after skin incision (t1); 30 min after extubation (t2). Significant variations of blood cortisol levels have not been shown.

[Hepatic injury and halogenated anesthetics: preliminary clinical experience]. / Danno epatico ed anestetici alogenati: esperienza clinica preliminare.

The potential hepatotoxic activity of isoflurane, a volatile anesthetic agent recently introduced in Italy, has been investigated by the Authors in the present study. Hepatic markers blood level (GPT, gamma GT, alkaline phosphatase and albumin) have been checked preoperatively and at day 1 and 4 postoperatively in a group of 35 patients who underwent general anesthesia for plastic surgery operations by means of isoflurane. As control group 32 patients were tested, treated with general anesthesia for the same type of surgery by means of halotane. The Authors conclude that isoflurane, according to the data obtained from present study, cannot be, at the moment, considered hepatotoxic.

[Clinical evaluation of atracurium besylate in patients at risk: major burns]. / Valutazione clinica dell'atracurium besilato nei pazienti a rischio: il grande ustionato.

Atracurium besylate 0.5 mg/kg-1, an intermediate-duration non-depolarizing neuromuscular relaxant, was administered slowly (over 75 sec) in anesthesia induction of 61 patients with major thermal injury undergoing surgical excision and immediate skin-grafting procedures. Patients' mean +/- SD age was 40 +/- 9, body weight 64 +/- 2, burn size ranging from 20% to 90% of body surface area (BSA), postburn day of surgery 5th and more. Induction of anesthesia was carried out with sodium thiopental 2-5 mg/kg-1 plus fentanyl 2.8 micrograms/kg-1 e.v. and after few minutes atracurium 0.5 mg/kg-1 e.v. Anesthesia was maintained with N2O/O2 (70%/30%), isoflurane and small amounts of fentanyl. The mean arterial pressure and heart rate were recorded at I, II, III, IV, V min post atracurium administration. The endotracheal intubation conditions were assessed by a "IOT score". Results are expressed as mean value +/- standard deviation. The significance of the difference in mean values was analysed by t-test. Little haemodynamic changes occurred; intubating conditions showed a relative hyposensitivity of burn patients to atracurium, more severely burned patients (greater than 50-60%) exhibiting greater resistance.

[Clinical use of isoflurane in otorhinolaryngoiatric surgery]. / Impiego clinico dell'isoflurane in chirurgia otorinolaringoiatrica.

Authors employ Isoflurane, a volatile anesthetic agent recently introduced in Italy, in a 111 patients group undergoing ORL surgery. Results show that clinical properties of Isoflurane well satisfy ORL surgery needs during and after operation.

[Anesthesiologic management in scleroderma patients. Presentation of a clinical case]. / Condotta anestesiologica nel paziente sclerodermico. Presentazione di un caso clinico.

The scleroderma (literally, hard skin), or progressive systemic sclerosis (PSS), is a multisystem disease and can present various anaesthetic problems. The anaesthesist should be aware of the difficulty in opening mouth wide enough for laryngoscopy and intubation, the possibility that cardiopulmonary changes may be present and the probability of lesions in oesophagus, bowel, kidneys, skin and joints. In this article the authors describe the anesthetic management of a patient with Thibierge-Weissenbach syndrome (scleroderma with calcinosis cutis) and explore potential problems that should be anticipated by the anesthesiologist.

[The technic of sub-vigil anesthesia in plastic-reconstructive surgery]. / La tecnica di anestesia sub-vigile in chirurgia plastico-ricostruttiva.

The personal experience in plastic-reconstructive surgery with 395 cases of sub-vigil anaesthesia (neuroleptanalgesia + benzodiazepine in 124 patients (group A); ketamine + benzodiazepine in 138 patients (group B); neuroleptanalgesia + ketamine + benzodiazepine in 133 patients (group C) is described by the authors in the present study. They conclude that the sub-vigil anaesthesia is usually free from complications and a very safe, tolerable, expedient technique for plastic surgical procedures; furthermore, the result is good patient acceptance, a stable blood pressure and heart rate.

[Chlordesmethyldiazepam as an anesthesia-inducing agent: time of loss of consciousness. Statistical analysis in man]. / Il clordemetildiazepam come farmaco ipnoinducente: tempo di perdita di coscienza. Analisi statistica nell'uomo.

.1 mg/kg e.v. of chlordesmethyldiazepam, a 1.4 benzodiazepine derivative, were given in induction of anaesthesia of 60 plastic-reconstructive surgery patients respectively in 30 (Group 1.30 patients) and in 60 sec (Group 2.30 patients) to assess the times to spontaneous closing of the eyes and to disappearance of the palpebral reflex. It is concluded that the reflex times are not significantly different in both groups.

[Cerebral perfusion pressure in endocranial hypertension in comatose head-injured patients]. / Pressione di perfusione cerebrale ed ipertensione endocranica nei traumi cranici in coma.

The aim of the intensive care of the injured is the coupling of the availability and the requirement of the cerebral metabolic substates. The measurement of the cerebral blood flow is not currently available at the bedside and less direct monitoring is required. The cerebral perfusion can be estimated looking at the cerebral perfusion pressure (CPP), that can be easily measured using intracranial pressure (ICP) and the systemic arterial pressure (MAP) monitoring. Hundred-twenty-one consecutive head injured admitted to an Intensive Care Unit were studied assessing the severity of the neurological injury, the CT-Scan diagnosis of the intracranial lesion, the Trauma Score and the behavior of the ICP and MAP. The outcome was classified according to a modified version of the Glasgow Outcome Scale. More than 77% of the patients suffered raised intracranial pressure above 20 mmHg and 16 of them had a CPP less than 60 mmHg for more than 5 minutes. The outcome was directly related to the degree of intracranial hypertension and to the severity of insufficient CPP. The treatment of the severe head injured must be aimed at maintaining a good CPP, because of the close relationships between this value and the prognostic result. The monitoring of the ICP is a reliable and relatively safe procedure in this series, where the rate of infections complicating the intracranial recording is less than 3%.