Brain stem encephalitis with central neurogenic hyperventilation.
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Biomedical subjects
Publications and source records attributed to E W Nielsen.
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BACKGROUND: Up to seven in every 1000 patients experience angioedema from angiotensin-converting enzyme (ACE) inhibitors, even after many years of use. In 2003, every 20th Norwegian used an ACE inhibitor. CASE REPORT: A 61-year-old woman with chronic obstructive pulmonary disease and a past acute myocardial infarction had used 7.5 mg of ramipril daily for the past 7 years. She also used acetylsalicylic acid, simvastatin, theophylline and salmeterol. One night she woke up with edema of the tongue. On hospital arrival, 250 mg of hydrocortisone and 5 mg of dexchlorpheniramine were given intravenously (i.v.) and 0.3 mg of epinephrine was given subcutaneously (s.c.). The edema of the tongue progressed over the next 8 h and made the tongue protrude. Fiberscopy revealed glassy edema of the arytenoids. Inspiratory stridor was heard and the patient could not speak. She became increasingly uneasy and restless. Berinert complement 1 (C1) inhibitor concentrate (1500 units) was administered i.v. Over the following 20 min, stridor gradually subsided, the patient calmed and she was able to talk. DISCUSSION: ACE inhibitor-provoked angioedema shares many clinical features with hereditary angioedema (HAE), including a limited effect of steroids, antihistamines and epinephrine. HAE, caused by excess bradykinin formation as a result of C1 inhibitor deficiency, usually has its laryngeal edema effectively reversed by C1 inhibitor in less than 0.5 h. Although patients experiencing ACE inhibitor-provoked angioedema have normal C1 inhibitor values, as in our patient, excess bradykinin is probably important as ACE breaks down bradykinin. It is unknown why ACE inhibitor-provoked angioedema appears in some and sometimes after many years of use. CONCLUSION: We believe that C1 inhibitor was effective in reversing the ACE inhibitor-induced angioedema in our patient.
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In adverse reactions with shock, tripled tryptase values can support a diagnosis of anaphylaxis. A 51-year old physically fit woman experienced angio-oedema and hypotensive shock after irbesartan ingestion requiring noradrenaline infusion. Serum tryptase rose to three times the normal value. Total immunoglobulin E and skin prick tests were normal, however. As nonallergic increases in tryptase have been observed, e.g. during angio-oedema from angiotensin-converting enzyme inhibitors, and bradykinin itself can degranulate mast cells acutely, we interpret the reaction as a class effect. To our knowledge, our report is one of the first on shock and angio-oedema from irbesartan.
A schizophrenic patient on long-time neuroleptic medication was admitted with ileus. Secondarily, a high fever, rigidity, mental confusion, tachycardia and hypotension developed. After bromocriptine was given, the temperature dropped by 2 degrees C and the patient improved markedly. A diagnosis of neuroleptic malignant syndrome was made. Five years later she was re-admitted with similar symptoms and also severe liver failure. Meanwhile the discontinued neuroleptic medication had been reinstituted. Again bromocriptine reduced the temperature of approximately 2 degrees C, and was paralleled by a normalization of liver function. To our knowledge this is the second report on severe liver failure in conjunction with neuroleptic malignant syndrome. The efficacy of bromocriptine in the treatment of this syndrome is underlined.
BACKGROUND: A national air ambulance service, including helicopters and airplanes, was implemented in Norway in 1988. The main intention was to offer advanced medical services when needed. All helicopters are manned by anesthesiologists. Catchment areas for the 11 helicopters span from cities to scarcely populated areas, particularly in the north. Our aim was to assess what proportion of ambulance missions carried out by the rescue helicopter in Bodø, northern Norway, delivered advanced medical treatment needing the skills of an anesthesiologist. METHODS: Flight and ambulance records (n = 2078) from 1988 and 1990-98 (10 years) were analyzed retrospectively. Inter-hospital transfers (n = 147) and search- and rescue missions (n = 332) were not included. According to the level of medical treatment given missions were categorized into three groups (A, B and C). Treatment in groups A and B would not require an anesthesiologist. RESULTS: Two thousand and seventy-eight ambulance missions carried 2166 patients (114 per 100 000 per year). Median take-off and on-scene times were 29 and 55 min, respectively. Seven hundred and fifty-five patients (35%) suffered from cardiovascular disease, 495 (23%) were injured and 250 (12%) were parturients. One hundred and seven patients (5.0%) received advanced prehospital emergency treatment requiring an anesthesiologist. Forty-five of the 107 patients survived to discharge from hospital, amongst whom 28 had received intravenous nitroglycerin for angina or suspected myocardial infarction. CONCLUSION: In our rural area, with a widely scattered population, 95% of patients received medical treatment not requiring an anesthesiologist. A selective use of the anesthesiologist seems indicated.
BACKGROUND: Both C1-inhibitor (C1-INH) and antibodies against the CD18 adhesion molecule have been shown to reduce ischaemia-reperfusion injuries. The objective of this study was to investigate the effect of increased ischaemia times and to determine whether inhibiting C1 or blocking the CD18 function was protective in skeletal muscle ischaemia-reperfusion injury after aortic cross-clamping. MATERIALS AND METHODS: BALB/c mice were subjected to aortic cross-clamping below the renal artery for 60, 75 or 105 min, followed by 3 h of reperfusion. Two-thirds of a total dose of anti-CD18 antibody (40 mg/kg) or human C1-INH (1,000 IU/kg) was given by intraperitoneal injection before ischaemia and one-third immediately after the clamping. Creatine kinase (CK) in the plasma was used as an indicator of muscle injury severity. RESULTS: There was a consistent rise in the plasma CK concentration proportional to the length of ischaemia (P < 0.0005). C1-INH treatment significantly (P = 0.012) reduced the plasma CK for the ischaemia times of 75 and 105 min. The anti-CD18 antibody did not have any effect, as demonstrated by the CK values that were similar to controls (P = 0.836). CONCLUSION: The data support a beneficial role for C1-INH in the treatment of ischaemia-reperfusion injuries of skeletal muscles.
Human C1 inhibitor is a highly glycosylated serine protease inhibitor of the serpin family. The protein contains two disulfide bonds. In this study, an N-terminally truncated form of recombinant C1 inhibitor was overexpressed in Escherichia coli strains BL21(DE3) and AD494(DE3), the latter enabling the formation of disulfide bonds within the cytoplasm. With both strains, a major fraction of the recombinant protein produced appeared to be insoluble. However, the soluble fraction of lysates from strain AD494(DE3) inhibited the C1s target protease in functional assays. Recombinant C1 inhibitor produced in this strain also displayed the ability to complex with C1s in vitro. In contrast, lysates from strain BL21(DE3) displayed no C1 inhibitor activity. These data support the notion that glycosylation is not important, whereas disulfide bond formation appears to be essential for the production of an active recombinant C1 inhibitor. Thus, bacterial strains that permit the formation of disulfide bonds may represent a reliable system for the production of recombinant C1 inhibitor. However, a major obstacle to large-scale production will be to produce the protein in a soluble form. Attempts to increase the yield of soluble protein by coexpression of the GroEL/ES chaperonins resulted in an increase in solubility.
BACKGROUND: At a county hospital in northern Norway, coronary angiographies have been performed for more than 25 years. We wanted to compare our complication rates with results from larger hospitals. MATERIAL AND METHODS: Data concerning indication for angiography, findings and complications were collected retrospectively from the files of 837 patients who underwent coronary angiography at our hospital during the last ten years. The number of procedures per year has varied from 30 to 125. RESULTS: 50% of the patients had three-vessel and/or left main coronary artery stenosis. Less than 10% of the patients had no significant coronary stenosis or valvular disease at angiography. The total complication rate was 4.8%. Nine patients (1.1%) had myocardial infarction or stroke with sequelae; three patients died (0.4%). INTERPRETATION: Our complication rates are higher than those from other larger hospitals in Norway and internationally, but they have decreased over time. The high percentage of serious coronary pathology among our patients may have contributed significantly to the complication rate. In addition, the annual number of exams may have been too low to give sufficient operator training. The results require a continuing evaluation of our practice.
BACKGROUND: At a county hospital in northern Norway, coronary angiographies have been performed for more than 25 years. We wanted to compare our complication rates with results from larger hospitals. MATERIAL AND METHODS: Data concerning indication for angiography, findings and complications were collected retrospectively from the files of 837 patients who underwent coronary angiography at our hospital during the last ten years. The number of procedures per year has varied from 30 to 125. RESULTS: 50% of the patients had three-vessel and/or left main coronary artery stenosis. Less than 10% of the patients had no significant coronary stenosis or valvular disease at angiography. The total complication rate was 4.8%. Nine patients (1.1%) had myocardial infarction or stroke with sequelae; three patients died (0.4%). INTERPRETATION: Our complication rates are higher than those from other larger hospitals in Norway and internationally, but they have decreased over time. The high percentage of serious coronary pathology among our patients may have contributed significantly to the complication rate. In addition, the annual number of exams may have been too low to give sufficient operator training. The results require a continuing evaluation of our practice.
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MATERIALS AND METHODS: Data on all patients with acute myocardial infarction who were treated in Nordland County Hospital in a six-month period in 1996 were analyzed retrospectively (137 patients). After the introduction of checklists for the treatment of such patients, we did a prospective six-month registration in 1997 (111 patients) in order to find out whether treatment and delay times had improved. RESULTS: The proportion of patients who received thrombolytic treatment did not change (28% in 1996 as compared to 25% in 1997). The in-hospital delay time before treatment did not differ before and after the introduction of a check-list (approximately 40 minutes in both periods). There was an increase in the use of intravenous beta blockers and aspirin. INTERPRETATION: The percentage of patients with acute myocardial infarction receiving thrombolytics in our hospital does not differ substantially from that of other hospitals in Norway.
Xenotransplantation may be a future alternative due to increased shortage of organs. Classical complement activation is central in hyperacute rejection in pig-to-human combinations. We investigated the effects of C1-inhibitor (C1-INH), a regulator of the complement and contact systems, on hyperacute rejection. Pig kidneys were perfused with fresh human blood to which either C1-INH (n = 6) or human serum albumin (n = 6) was added. The survival of the C1-INH perfused kidneys (mean 327 min) was significantly longer (p < 0.00001) than the controls (79 min). C1-INH substantially inhibited complement activation (C1rs-C1-INH complexes, C4bc, C3bc and terminal complement complex) (p < 0.001 for all) compared with the marked complement activation in the controls. No contact activation was found. Leukocytes and platelets were substantially activated (counts, myeloperoxidase, beta-thromboglobulin, thrombospondin, soluble P-selectin) in the control group, and this activation was markedly reduced by C1-INH (p < 0.02 for all). Immunohistochemistry showed less C1q, C3, TCC, IgG and fibrin deposition in the C1-INH group. C1-INH may be useful to attenuate hyperacute rejection, probably through inhibition of complement. The reduced activation of neutrophils and platelets may mainly be secondary to inhibition of complement.
OBJECTIVE: To study the association between disease activity and complement activation prospectively in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: Twenty-one SLE patients were examined monthly for 1 yr. Disease activity, autoantibodies, conventional complement tests and the following complement activation products were investigated: C1rs-C1inh complexes, C4bc, Bb, C3a, C3bc, C5a and the terminal SC5b-9 complement complex (TCC). RESULTS: Modest variation in disease activity was noted. None of the patients had nephritis. Flare was observed at 27 visits. Four patients had anti-C1q antibodies in conjunction with modestly low C1q concentrations. The complement parameters were rather constant during the observation period. Slightly to moderately decreased C4 (0.05-0.10 g/l) was found in 10 patients and severely decreased C4 (<0.05 g/l) in seven patients. Decreased C4 was not associated with increased complement activation. Complement activation products were either normal or slightly elevated. TCC was the only activation product correlating significantly with score for disease activity at flare. None of the variables tested predicted flares. CONCLUSION: Complement tests are of limited importance in routine examination of SLE without nephritis, although TCC is suggested to be one of the most sensitive markers for disease activity.
In this article we present a Norwegian family with hereditary multiple exostoses and a review of relevant literature. 21 family members were examined; ten males and six females had multiple exostoses, the youngest from six months of age. The exostoses had led to compression of nerves, disturbance of bone growth with shortening and bowing of the forearm bones, and valgus deformity of the knee and ankle. Physical function and quality of life was generally well preserved. Contrary to most reports, nearly half of the patients had allegedly noticed growth or debut of exostoses in adulthood. Almost half of the patients had had the exostoses removed surgically twice, (median value). Malignant transformation of exostoses has not been observed in the family.
The use of blood pressure cuffs containing bladders of appropriate size for the arm is a known prerequisite for correct blood pressure measurements. The Tricuff, containing three inflatable bladders of varying dimensions, one of which is automatically selected to fit the arm circumference, was compared to a standard cuff (12 x 35 cm) in 137 persons. In persons with large arms (circumference > or = 32 cm) measurement with the Tricuff showed on average 19.2 mm Hg lower systolic pressure and 12.9 mm Hg lower diastolic pressure than with the standard cuff. In persons with small arms (< 32 cm) the corresponding differences were 9.0 mm Hg and 7.5 mm Hg. Compared to the Tricuff, the standard cuff overestimates blood pressure in people with a large arm circumference. Overestimation may also be caused by the rubber bladder bulging more from below the standard soft nylon cuff than what the firmer texture of the Tricuff allows, resulting in falsely high values even in persons with normal arm circumference.
We report on a patient who suffered an acute, extensive intracerebral haemorrhage, leading to symptoms of cerebral herniation within a few hours. The clinical diagnosis of brain death was made based on a neurological examination, and an apnoea test eight hours after the haemorrhage. A few hours later the diagnosis was changed, as several reflexes reappeared. After six days mechanical ventilation was withdrawn, as the brain damage was considered so serious as to render further therapy futile. It was considered unethical to sustain therapy for a possible organ donation at a later date. A review of relevant the literature, however, shows that brain-dead patients may exhibit such varying degrees of autonomic and spinal reflexes as to cause confusion, thus delaying the physician in making a diagnosis. Often, an opportunity for organ donation is lost. Based on this review, we believe that our patient was indeed brain dead when the first diagnosis was made, and that a cerebral angiography should have been performed. Because organ donation is an important issue, the diagnosis of brain death must be definitive.
Over a four-year period total serum cholesterol values were obtained from all 7th grade lower secondary school students (age 13) in a municipality in Norway. The 139 girls included in the survey had a mean value of 4.24 mmol/l (95% CI 4.12-4.37), and the 160 boys 4.11 mmol/l (95% CI 3.98-4.23), which is slightly lower than other Nordic surveys. Some students from one year were also measured in the 9th and 11th grades. One student was found to have familial hyper-cholesterolemia, and this value was excluded from the reference material. Screening for this disease among students is not recommended.