Anomalies du métabolisme du cuivre chez l’adulte

Copper is essential for many enzymatic reactions and in neurotransmitter biosynthesis. Its deficiency or its excess has consequences on many organs, especially the liver and the brain. The biochemical tests performed in case of suspicion of copper metabolism disorder are difficult to analyse. They include the measurement of serum ceruloplasmin, serum copper and 24 h urinary copper excretion. The interpretation must take into account the clinical features. We distinguish mainly: (1) copper deficiency, acquired in malabsorption or in copper diet deficiency, or related to a genetic disease (Menkes disease); (2) copper overload, acquired or from a genetic origin (Wilson disease); (3) aceruloplasminemia, reducing ferroxidase activity leading to iron overload. It is important to diagnose these diseases as some of them have an effective treatment if it is started early enough.     

Le vidéolaryngoscope permet une intubation avec un tube à double lumière plus rapide que le laryngoscope standard

Purpose

This study was designed to compare videolaryngoscopy with direct laryngoscopy with respect to ease of intubation when inserting a double lumen tube (DLT).

Methods

In this prospective randomized study 68 patients American Society of Anesthesiologists (ASA) physical status I and II were included. Patients with criteria indicating possible difficult intubation were excluded. The patients were randomized into two groups, depending on the tool used to facilitate intubation: videolaryngoscope (VL group) or direct laryngoscopy (DL group). The time required for intubation was the primary endpoint. Cormack and Lehane glottic visualization (CL) scores, the need for external laryngeal maneuvers and the number of attempts were measured.

Results

Glottic visualization was better in the VL group than in the DL group. The CL scores were I, II and III in 24, eight and two patients, respectively, in the VL group compared with 13, 11 and eight in the DL group (P = 0.025). Patients in the VL group required fewer attempts than the DL group (P = 0.019). Intubation time was 39.9 ± 4.4 sec in the VL group and 47.9 ± 5.4 sec in the DL group (P < 0.001). No intubation failure was noted in group VL compared with two in the DL group (not significant).

Conclusion

The use of a videolaryngoscope reduces the time required for intubation with a DLT compared with the direct laryngoscopy in elective thoracic surgery.     

The hypolipidaemic activity of aqueous Erica multiflora flowers extract in Triton WR-1339 induced hyperlipidaemic rats: a comparison with fenofibrate

In the present study, an aqueous extract from Erica multiflora L. (Ericaceae) flowers was evaluated for its hypocholesterolaemic and hypotriglyceridaemic activities using Triton WR-1339 induced hyperlipemic rats as experimental model. Hyperlipideamia was developed by intraperitonial injection of Triton (200mg/kg body weight). The animals were divided into control (CG), hyperlipidaemic (HG), hyperlipidaemic plus herb extract (HG+EmE) and hyperlipidaemic plus fenofibrate (HG+FF) treated groups. Intragastric administration of Erica multiflora extract (0.25 g/100g body weight) to the rats caused a significant decrease on their plasma lipid levels (quantified using enzymatic kits). At 7h after treatment, plasma total cholesterol, triglycerides and LDL-cholesterol were decreased by 47%, 95% and 67%, respectively, but the change of HDL-cholesterol level was not significant. However, the hypolipidaemic effect of fenofibrate was limited to triglycerides and LDL-cholesterol, which were lowered by about 92% and 41%, respectively. At 24h after treatment, Erica multiflora extract reduced plasma total cholesterol by 68.5% and triglycerides by 91%. HDL-cholesterol was not significantly increased and LDL-cholesterol was lowered by 80.5%. In fenofibrate treated rats, only plasma triglyceride concentrations were lowered by 82%, while the other lipid parameters were not significantly changed indicating that this aqueous herb extract may contain products that lower plasma lipid concentrations and might be beneficial in treatment of hyperlipideamia.     

High-molecular-weight hyaluronan – a possible new treatment for sepsis-induced lung injury: a preclinical study in mechanically ventilated rats

Introduction

Mechanical ventilation with even moderate-sized tidal volumes synergistically increases lung injury in sepsis and has been associated with proinflammatory low-molecular-weight hyaluronan production. High-molecular-weight hyaluronan (HMW HA), in contrast, has been found to be anti-inflammatory. We hypothesized that HMW HA would inhibit lung injury associated with sepsis and mechanical ventilation.

Methods

Sprague–Dawley rats were randomly divided into four groups: nonventilated control rats; mechanical ventilation plus lipopolysaccharide (LPS) infusion as a model of sepsis; mechanical ventilation plus LPS with HMW HA (1,600 kDa) pretreatment; and mechanical ventilation plus LPS with low-molecular-weight hyaluronan (35 kDa) pretreatment. Rats were mechanically ventilated with low (7 ml/kg) tidal volumes. LPS (1 or 3 mg/kg) or normal saline was infused 1 hour prior to mechanical ventilation. Animals received HMW HA or low-molecular-weight hyaluronan via the intraperitoneal route 18 hours prior to the study or received HMW HA (0.025%, 0.05% or 0.1%) intravenously 1 hour after injection of LPS. After 4 hours of ventilation, animals were sacrificed and the lung neutrophil and monocyte infiltration, the cytokine production, and the lung pathology score were measured.

Results

LPS induced lung neutrophil infiltration, macrophage inflammatory protein-2 and TNFα mRNA and protein, which were decreased in the presence of both 1,600 kDa and 35 kDa hyaluronan pretreatment. Only 1,600 kDa hyaluronan completely blocked both monocyte and neutrophil infiltration and decreased the lung injury. When infused intravenously 1 hour after LPS, 1,600 kDa hyaluronan inhibited lung neutrophil infiltration, macrophage inflammatory protein-2 mRNA expression and lung injury in a dose-dependent manner. The beneficial effects of hyaluronan were partially dependent on the positive charge of the compound.

Conclusions

HMW HA may prove to be an effective treatment strategy for sepsis-induced lung injury with mechanical ventilation.     

Arrhythmogenic right ventricular dysplasia/cardiomyopathy

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiac disease characterized by fibrofatty replacement of the cardiac myocytes. Patients with ARVD/C frequently present with ventricular tachycardia and many are thought to have sudden cardiac death as the initial manifestation of the disease. Over the past decade, our understanding of the disease has increased dramatically along with delineation of the genetic basis of ARVD/C and characteristic features on diagnostic imaging. The management of patients with ARVD/C remains a challenge, especially in the light of incomplete genotype-phenotype characterization, and poor predictors of sudden cardiac death. In this article, we review the pathologic and genetic basis of ARVD/C, focusing on the diagnostic features and therapeutic challenges emerging with our enhanced knowledge of this rare disease.     

Neutrophil secretory defect in the gray platelet syndrome: a new case

We report the case of a 60-year-old woman who was newly diagnosed for the gray platelet syndrome (GPS). This patient had long-term thrombocytopenia which had been initially misdiagnosed as idiopathic thrombocytopenic purpura (ITP). Blood smear displayed characteristic gray platelets, allowing the diagnosis to be made, which was confirmed by electron microscopy (EM). Polymorphonuclear neutrophils (PMN) appeared poorly granulated on the May-Grunwald-Giemsa-stained blood smear. Flow cytometry analysis of PMN demonstrated increased expression of CD35, CD11b and CD18 at resting PMN surface, without any changes after fMLP stimulation. Ultrastructural study retrieved a decreased number of myeloperoxidase (MPO)-negative secondary granules in PMN. Immunolabeling confirmed the presence of membrane proteins and the absence of soluble content in platelet and megakaryocyte (MK) alpha-granules, and the decrease of secondary granules and secretory vesicles in PMN. This new observation demonstrates that the impairment of the secretory compartment of PMN is definitely a hallmark of GPS, and that the detection of these subtle abnormalities should be searched with adequate and up-to-date technical approaches.