Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino-acid mixture.

BACKGROUND: Acute decompensation of maple syrup urine disease (MSUD) is usually treated by enteral feeding with an amino-acid mixture without leucine (Leu), valine or isoleucine. However, its administration is ineffective in cases of gastric intolerance and some adult patients refuse enteral feeding via a nasogastric tube. We developed a new parenteral amino-acid mixture for patients with MSUD. METHODS: Seventeen decompensation episodes in four adult patients with MSUD treated with a parenteral amino-acid mixture (group P) were compared to 18 previous episodes in the same patients treated by enteral feeding (group E). RESULTS: The mean Leu concentration at presentation was similar in the groups P and E (1196.9 µmol/L and 1212.2 µmol/L, respectively). The mean decrease in the Leu concentration during the first 3 days of hospitalisation was significantly higher in group P than group E (p = 0.0026); there were no side effects. The mean duration of hospitalisation was similar (4 vs. 4.5 days, p = NS). No patient in group P deteriorated whereas one patient in group E required dialysis. CONCLUSION: This new parenteral amino-acid mixture is safe and allows efficient Leu concentration decrease during acute MSUD decompensation episodes in adults. Its use avoids the need for nasogastric tube insertion.

[Theriaque: independent-drug database for good use of drugs by health practitioners]. / Thériaque : base de données indépendante sur le médicament, outil de bon usage pour les professionnels de santé.

Thériaque is a French-drug database, which was created 25 years ago by the centre national hospitalier d'information sur le médicament (CNHIM). The aim was to collect and diffuse independent information to healthcare professionals. From the beginning, the database had two objectives: information access and data input into prescription/dispensary software. Initially accessible via Minitel, then on CD-ROM, Thériaque has been integrated into hospital software. Since 1998, Thériaque is available on the Internet (www.theriaque.org). Today, the CNHIM develops Thériaque in a totally independent way. The eight main qualities of Thériaque are: firstly, independence from pharmaceutical companies and the national healthcare insurance fund; secondly, exhaustive data collection: Thériaque gathers official information and information from reference books; thirdly, reliability: validation of the work carried out by pharmacists, references of all information sources; fourthly, accessibility: free Internet site for all health professionals, prescription analyses; fifthly, originality: integration of an international thesaurus with input from WHO or the European Pharmacopeia and scientific validated information; sixthly, interactivity with users: letterbox, user groups, identification of the needs of health professionals; seventhly, collaboration with other databases or health authorities, that is, EMEA, INCa, HAS or AFSSAPS, which made it possible to develop the concept of infovigilance; eighthly, interoperability: undoubted identification of a drug allowing secure communication between different software. The number of users increased regularly: 41,000 monthly users of the Internet site at the end of 2006 and 216 hospitals using software-integrating Thériaque. Thériaque participates in ensuring the safety of the drug circuit and helps prevent iatrogenic events as it has been defined in France in the decree of 24th August 2005.

[The Theriaque database and information on side effects of drugs]. / La base de données Thériaque et les informations sur les effets indésirables des médicaments.

The new module of Theriaque data base deals with side effects of drugs. It is composed of 3,300 monographs. One or more nature of side effect appears on each monograph, to correspond to a defined entity; organ or apparatus, syndrome or special type of pharmacological effect. Side effects are attached to either a whole pharmacological or chemical class, either one or several drugs. Side effects of drugs are described at usual dose, or/and in case of overdose. Data are collected from specialized books and publications. Some difficulties were encountered with this work, particularly side effects frequency. Side effects module content is validated with experts in pharmacology or clinicians, their opinion being a determining factor in case of any doubt, especially on causality assessment or frequency.

[Value of C-reactive protein in the cardiac postoperative period]. / Intérêt de la CRP en postopératoire cardiaque.

Twenty patients underwent special clinical and biological monitoring during their period of hospitalisation in the Department of cardiovascular surgery. The clinical monitoring concentrated on the patient's temperature and the search for postoperative complications; the biological part of the study was concerned with monitoring the levels of serum C-Reactive protein (CRP) as assessed by an immuno-nephelometric method. In 10 patients with a normal postoperative course the levels of this protein, an indicator of an inflammatory or infective process, were similar, giving an identical graph in all patients. On the other hand, in the 10 patients with inflammatory or infective postoperative complications, the levels of CRP were abnormal, parallel with the clinical state, sometimes rising even before the complication manifested itself clinically. It therefore seems useful and justified to measure CRP systematically, once before surgery and at least once daily in the postoperative period. The frequency of this investigation could be increased in patients with difficult or complex postoperative courses. A high CRP, even with a normal temperature, should alert the surgeon to a complication or to the inefficacy of anti-inflammatory or anti-infective therapy. These results confirm those published by other surgical teams, both in cardiovascular surgery and traumatology.