Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study

Introduction: Acquired demyelinating syndromes (ADS) are immune-mediated demyelinating disorders of the central nervous system in children. A nationwide, multicentre and prospective cohort study was initiated in the Netherlands in 2006, with a reported ADS incidence of 0.66/100,000 per year and MS incidence of 0.15/100,000 per year in the period between 2007 and 2010. In this study, we provide an update on the incidence and the long-term follow-up of ADS in the Netherlands. Methods: Children < 18 years with a first attack of demyelination were included consecutively from January 2006 to December 2016. Diagnoses were based on the International Paediatric MS study group consensus criteria. Outcome data were collected by neurological and neuropsychological assessments, and telephone call assessments. Results: Between 2011 and 2016, 55/165 of the ADS patients were diagnosed with MS (33%). This resulted in an increased ADS and MS incidence of 0.80/100,000 per year and 0.26/100,000 per year, respectively. Since 2006 a total of 243 ADS patients have been included. During follow-up (median 55 months, IQR 28–84), 137 patients were diagnosed with monophasic disease (56%), 89 with MS (37%) and 17 with multiphasic disease other than MS (7%). At least one form of residual deficit including cognitive impairment was observed in 69% of all ADS patients, even in monophasic ADS. An Expanded Disability Status Scale score of ≥ 5.5 was reached in 3/89 MS patients (3%). Conclusion: The reported incidence of ADS in Dutch children has increased since 2010. Residual deficits are common in this group, even in monophasic patients. Therefore, long-term follow-up in ADS patients is warranted

RegaDB: Community-driven data management and analysis for infectious diseases

Summary: RegaDB is a free and open source data management and analysis environment for infectious diseases. RegaDB allows clinicians to store, manage and analyse patient data, including viral genetic sequences. Moreover, RegaDB pr

Susceptibility to Malignant Hyperthermia.

Contains fulltext : 19527.pdf (publisher's version ) (Open Access)In this thesis the author studied the diagnostic procedures for susceptibility to malignant hyperthermia (MH), with special emphasis upon refining the biological diagnostic test and improving protocols and guidelines for investigation of MH susceptibility. MH is a pharmacogenetic disease of skeletal muscle which is mainly of concern during and following anaesthesia. Abnormalities in the calcium release channel of skeletal muscle sarcoplasmic reticulum, the ryanodine receptor, have been implicated as the central factor in the cause of the MH syndrome. The in vitro muscle contracture test (IVCT) is the standard test to establish an individual's risk of susceptibility to MH. The diagnostic sensitivity of 99.0% and the accompanying specificity of 93.6% of the standard halothane-caffeine IVCT have been determined in a joint European study. Sevoflurane, a potent inhalational anaesthetic agent that is structurally similar to halothane, has been able to trigger an abnormal contracture in human muscle in vitro. The IVCT is invasive, time consuming and it is a technically demanding test that requires expertise. Cultured skeletal muscle cells, obtained by percutaneous needle biopsies may well be applied in diagnostic tests for MH susceptibility since the dose-dependant Ca2+ response in cultured muscle cells of MH-susceptible individuals is significantly different from that of control individuals after exposure to halothane. Because MH displays a high level of locus heterogeneity, it is not feasible to diagnose MH susceptibility, and, more specifically, to exclude MH risk, on the basis of a simple genetic test alone. Clinical practitioners and geneticists of the European MH Group have agreed on guidelines for the detection of MH susceptibility using molecular genetic techniques. Evaluation of the diagnostic procedure in 4 European MH investigation units helped to gain an insight into the need for further standardisation and quality assurance for the diagnosis of MH.KUN Katholieke Universiteit Nijmegen, 11 november 2004Promotor : Sengers, R.C.A. Co-promotor : Gielen, M.J.M.136 p

Continue hoog-thoracale toediening van bupivacaïne-clonidine versus bupivacaine-sufentanil voor (post)operatieve pijnbestrijding na thoracotomie

Contains fulltext : 23590___.PDF (publisher's version ) (Open Access

Maligne hyperthermie als complicatie van anesthesie: aanleg is erfelijk

Contains fulltext : 24356___.PDF (publisher's version ) (Open Access

Steady state bupivacaine plasma concentrations and safety of a femoral "3-in-1" nerve block with bupivacaine in patients over 80 years of age.

OBJECTIVES: Fracture of the upper femur is a common injury in the elderly. Several anesthetic techniques exist for surgery of traumatic hip fracture. The aim of this investigation was to study plasma concentrations and safety of 2 mg/kg bupivacaine in a femoral "3-in-1" nerve block in patients older than 80 years of age. SUBJECTS AND METHODS: A 3-in-1 femoral nerve block, combined with a general anesthetic was used in 10 elderly patients aged over 80 years. They were undergoing emergency surgery for stabilization of their fractured femur. Bupivacaine plasma concentrations of radial artery blood samples were assessed over a 6-hour period after a femoral 3-in-1 injection of 2 mg/kg bupivacaine 0.375% with epinephrine (1:400,000). RESULTS: No toxic reactions to bupivacaine were seen. In 8 of the 10 patients per- and postoperative analgesia were adequate as a result of the nerve block. Patients experienced loss of sensation and analgesia for 26.6 +/- 4.6 hours (mean +/- SD). This was inversely related to the apparent steady state concentration of bupivacaine. The mean of the individual peak plasma concentrations of bupivacaine (C(max) was 0.74+/- 0.64 microg/ml. The highest plasma concentration was 1.83 microg/ml. Large variations in plasma concentrations were detected in these patients. Bupivacaine metabolites were not detected. CONCLUSIONS: A femoral 3-in-1 nerve block, using 2 mg/kg bupivacaine with epinephrine, provides prolonged pain reliefwithout local anesthetic toxicity in elderly patients. It is a satisfactory supplementary analgesic technique for hip and knee surgery in the elderly

Steady state bupivacaine plasma concentrations and safety of a femoral "3-in-1" nerve block with bupivacaine in patients over 80 years of age.

Item does not contain fulltextOBJECTIVES: Fracture of the upper femur is a common injury in the elderly. Several anesthetic techniques exist for surgery of traumatic hip fracture. The aim of this investigation was to study plasma concentrations and safety of 2 mg/kg bupivacaine in a femoral "3-in-1" nerve block in patients older than 80 years of age. SUBJECTS AND METHODS: A 3-in-1 femoral nerve block, combined with a general anesthetic was used in 10 elderly patients aged over 80 years. They were undergoing emergency surgery for stabilization of their fractured femur. Bupivacaine plasma concentrations of radial artery blood samples were assessed over a 6-hour period after a femoral 3-in-1 injection of 2 mg/kg bupivacaine 0.375% with epinephrine (1:400,000). RESULTS: No toxic reactions to bupivacaine were seen. In 8 of the 10 patients per- and postoperative analgesia were adequate as a result of the nerve block. Patients experienced loss of sensation and analgesia for 26.6 +/- 4.6 hours (mean +/- SD). This was inversely related to the apparent steady state concentration of bupivacaine. The mean of the individual peak plasma concentrations of bupivacaine (C(max) was 0.74+/- 0.64 microg/ml. The highest plasma concentration was 1.83 microg/ml. Large variations in plasma concentrations were detected in these patients. Bupivacaine metabolites were not detected. CONCLUSIONS: A femoral 3-in-1 nerve block, using 2 mg/kg bupivacaine with epinephrine, provides prolonged pain reliefwithout local anesthetic toxicity in elderly patients. It is a satisfactory supplementary analgesic technique for hip and knee surgery in the elderly

Long duration of pain relief after 3-in-1 nerve block in geriatric patients

Item does not contain fulltex