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We have studied a large Swedish family with a mutation in the nerve growth factor beta (NGFB) gene causing insensitivity to deep pain without anhidrosis (hereditary sensory and autonomic neuropathy, type V; HSAN V). Painfree joint destruction and fractures were common. Peripheral nerve conduction was normal, but temperature thresholds were increased. Sural(More)
60 consecutive patients (age 15-77 years) with the Guillain-Barré syndrome were studied. 37 subjects had an antecedent infection. Onset occurred with motor and/or sensory limb symptoms in 56 cases; 4 subjects experienced onsets with pain, diplopia or bladder disturbances. The motor symptoms reached a maximum within 42 days in all cases, 87% within less than(More)
PURPOSE We wished to evaluate the prognostic usefulness of various EEG parameters with respect to remission rates after discontinuation of antiepileptic drug (AED) therapy in children treated for epileptic seizures. METHODS Two hundred forty-four children with uncomplicated epileptic seizures were randomized to either 1 or 3 years of treatment with AEDs.(More)
PURPOSE The main purpose of this prospective study was to analyze whether 1 year of treatment was as effective as 3 years with respect to remission rate in children with idiopathic epilepsy. METHODS Treatment for epileptic seizures was started in 207 children aged 2-16 years. They were randomized to treatment for 1 or 3 years. At the end of the(More)
Extrinsic coagulation pathway inhibitor may be an important regulator of haemostasis to prevent thrombosis after tissue damage. The functional activity of this inhibitor was determined using a chromogenic substrate assay, and compared to the activities of antithrombin, heparin cofactor II and protein C during the perioperative period of elective hip(More)
During a general population follow-up study in Health Region I in Norway, 162 patients with multiple myeloma (MM) were diagnosed. 71 of these (44%) were asymptomatic, and were observed without chemotherapy. The great majority (90%) were in stage I, and there were only 2 with light chain disease. 45 of the 71 asymptomatic patients developed progressive(More)
The coagulation inhibitor protein C was measured in 151 patients with various liver diseases. The protein C level was significantly decreased in patients with alcoholic cirrhosis (n = 73) compared to patients with steatosis (n = 24) (40 +/- 2%) vs. 88 +/- 4%, mean S.E., p less than 0.001). It was also decreased in cases of acute liver damage (n = 8) and in(More)
In metachromatic leukodystrophy (MLD), the deficiency of the lysosomal enzyme arylsulfatase A (ARSA) leads to demyelination in the central and peripheral nervous system and ultimately to death. Allogeneic hematopoietic SCT (HSCT) is currently the only treatment for adult and late-onset juvenile MLD, although it is still in question because of insufficient(More)
Serial determinations of plasma coagulation inhibitor levels were performed with chromogenic substrate activity assays in 7 patients with cancer. At time of diagnosis normal median activities of Antithrombin, Protein C, Heparin Cofactor II and Extrinsic Pathway Inhibitor were found. The inhibitor activities changed significantly with the progress of(More)
The coagulation inhibitors heparin cofactor II (HC II), protein C (PC) and antithrombin (AT) were measured in 14 patients undergoing uncomplicated abdominal, orthopedic or vascular surgery. Marked transient decrease of all the inhibitors was found after all surgical procedures, most prominently in vascular surgery. The greatest mean reduction was in HC II.(More)