PubMed Health⌕ Search

Biomedical subjects

E W Nielsen

Publications and source records attributed to E W Nielsen.

16 recordsLinked to original sources
PubMed1996

[Reduced blood utilization in hip arthroplasty. Introduction of a blood preservation program].

In spring 1992 a blood conservation programme was established at Nordland Central Hospital with emphasis on indications for blood transfusion and intraoperative blood salvage (cell-saver). Medical records from all patients who underwent hip arthroplasty during the period 1 June 1991 to 28 February 1994 were examined. Mean transfusion of homologous SAGMAN-blood (bank blood) was substantially reduced during the period: for total prosthesis (n = 192) from 2.6 to 1.1 units per operation (p < 0.0001) and for hemiprosthesis (n = 66) from 3.1 to 0.9 units per operation (p = 0.0202). The percentage of patients in each of these two groups who did not receive blood transfusion at all increased from 18 to 61 in the first group (p < 0.0001) and from 24 to 65 (p = 0.0202) in the second. The substantial reduction of bank blood transfusion in this material conforms with current international transfusion guidelines. A particular benefit, considering the risk of transfusing contagious blood, is the marked increase in the number of patients who did not receive any blood product at all.

Aged↗
PubMed1996

Hereditary angio-oedema: new clinical observations and autoimmune screening, complement and kallikrein-kinin analyses.

OBJECTIVES: To study clinical and laboratory manifestations of hereditary angio-oedema (HAE). SUBJECTS: Thirty-three affected members of a kindred of 63. RESULTS: Oedematous attacks in the skin, mucous membranes and gastrointestinal tract with fluid displacement were elicited by mental and physical stress, minor traumas, dental and surgical procedures, eruption of teeth, tonsillitis, pregnancies, and use of oestrogen-containing pills including menopausal substitution. Every adult woman with symptomatic HAE (n = 11) showed symptoms of urinary tract infections in conjunction with the attacks (P = 0.010), and also experienced more spontaneous abortions or premature labours (P = 0.037) than healthy relatives. Patients with HAE of both sexes more frequently reported heartburn or peptic ulcers (P = 0.002). Rheumatic complaints were reported by 53% of HAE patients and 12% of their unaffected relatives (P = 0.013), but biochemical screening for 18 autoantibodies and quantitation of immunoglobulins did not reveal statistically significant differences between the two groups. C3, prekallikrein, total kininogen, high molecular weight kininogen (HK), alpha-2-macroglobulin and factor XII were not significantly different in HAE patients. In contrast, levels of C1-INH and C4 were depressed and cleaved HK increased in patients compared to unaffected relatives. CONCLUSIONS: HAE manifests in a variety of ways, and may influence risk of spontaneous abortions and premature labour.

Adolescent↗
PubMed1996

Activation of the complement, coagulation, fibrinolytic and kallikrein-kinin systems during attacks of hereditary angioedema.

Five patients with hereditary angioedema (HAE) were studied during attacks and remission as were healthy controls. The high levels of C1/C1-INH complexes, low C4 and high ratio C4 activation products (C4bc)/C4 also differed significantly during remission compared to controls. During attacks C4bc/C4 increased (922-2007; P = 0.022, remission versus attacks, median values throughout), C2 and CH50 dropped (111-31%; P = 0.043 and 110-36%; P = 0.016, respectively), TCC (C5b-9) increased (0.88-1.23 AU/ml; P = 0.028). Cleavage of HK increased to be almost complete during attacks (20-90%; P = 0.009). While factor XIa/serpin-complexes did not increase, a more than twofold rise in thrombin/antithrombin-complexes (0.20-0.50 microgram/l; P = 0.009) and in plasmin/alpha-2-antiplasmin-complexes (7.3-17 nmol/l; P = 0.028) was observed. For the first time cascade activation in HAE was studied simultaneously, and corroborates that attacks lead to activation of the kallikrein-kinin system, fibrinolysis and early part of the classical complement pathway. In addition, the authors present novel data of terminal complement and coagulation activation, the latter apparently not via FXIa.

Angioedema↗
PubMed1995

[A fatal case of hereditary angioedema].

A 27 year old woman suffered from recurrent attacks of laryngeal oedema due to C1-inhibitor deficiency, and was treated with danazol and tranexamic acid. The trachea was intubated with great difficulty, twice on one occasion. Two and a half years later she was admitted to the Intensive Care Unit with dyspnoea and dysphagia. Tranexamic acid, corticosteroids, adrenaline (also inhalated), were administered intravenously, but dyspnoea progressed. During preparation for tracheostomy the patient suffered from sudden airway collapse. Attempts to ventilate by mask, puncture of the cricothyroid membrane and intubation were unsuccessful. A small tube was eventually inserted into the trachea after four minutes. The patient was then severely cyanotic with a pulse of thirty, and had dilated pupils. The next morning convulsions ensued and a CT scan showed cerebral oedema. In spite of treatment with pentothal, mannitol and hyperventilation she died. The authors advocate the use of intravenous infusion of C1-inhibitor concentrate, since traditional treatment is inadequate. Persons with hereditary angio-oedema should have a personal supply of C1-inhibitor at hand.

Adult↗
PubMed1995

C3 is activated in hereditary angioedema, and C1/C1-inhibitor complexes rise during physical stress in untreated patients.

Seven patients with hereditary angioedema (HAE) were studied to understand further how physical exercise may induce attacks. The most pronounced differences between patients and controls, however, were independent of the controlled bicycle run (mean values in patients/ controls); C4(g/L): 0.12/0.28 (P = 0.0122); C4bc (AU/ml): 137.0/18.0 (P = 0.0002); C4d (mg/mL): 5.03/2.35 (P = 0.0004); C3bc (AU/ml): 8.4/6.3 (P = 0.0049); C3a (AU/ml): 11.1/5.6 (P = 0.0102). The ratio C4bc to C4 was 1141 versus 64. Consequently, a substantial part of the low amount of C4 left in HAE patients consists of activation products, and the authors show for the first time that a mild but significant activation of C3 occurs in HAE. The two HAE patients treated with danazol had values of C1-INH function and antigen, C4, and C2 in-between those of normal and untreated patients, and lower levels of split products from C4 and high molecular weight kininogen than untreated patients. As a result of the exercise, fibrinolysis increased significantly in both patients and controls, while C1/C1-INH complexes rose significantly only in the five HAE patients without treatment when compared to the seven controls (P = 0.0089). This study thus suggests that complement activation is enhanced in untreated HAE patients following physical stress.

Angioedema↗
PubMed1995

Factor VIIa in patients with C1-inhibitor deficiency.

In hereditary angioedema (HAE), normal C1-inhibitor (C1-INH) is low and the contact system activated. Recently, the findings of a tissue factor mutant selectively deficient in promoting the conversion of FVII to FVIIa, but with retained cofactor for FVIIa, made it possible to examine reliably the pre-existing content of FVIIa in HAE patients. This was of interest as FXIIa (mainly inhibited by C1-INH) is able to activate FVII directly. FVIIa in 21 remission HAE patients were within normal limits but nearly doubled as compared to their 23 normal siblings (p = 0.0017). Cold promoted activation of FVII (CPA) (common clot assay) was displayed in plasma of all 5 untreated patients (C1-INH function < 35%), but not in plasma of 2 patients treated prophylactically with danazol (C1-INH function about 40%). These results suggest that there is a minute, yet significant activation of FVII in patients with C1-INH deficiency.

Angioedema↗
PubMed1994

Effect of time, temperature and additives on a functional assay of C1 inhibitor.

There are different recommendations for the handling of blood samples for analyses of the kallikrein-kinin or complement system, respectively. C1 inhibitor (C1-INH) takes a crucial part in both systems. In order to establish recommendations for blood specimen collection and transport for making the diagnosis of hereditary angioedema (HAE), the effect of time, temperature and different additives on C1-INH function and antigen was determined. We used blood samples from normals and patients suffering from HAE type I. Plasma containing EDTA, heparin, sodium citrate or polybrene-EDTA, and serum were assayed after incubations at 4 degrees C or 37 degrees C for 6 or 24 h. In addition, pooled serum was incubated for up to 5 days at room temperature. A modest decrease in C1-INH function was observed as an effect of storage-time in samples from normals (p = 0.039) and a substantial decrease was seen for the HAE patients (p = 0.0002). No significant effect of temperature (4 degrees C or 37 degrees C) was found. Clotting did not reduce C1-INH activity. Plasma containing heparin or polybrene interfered with the functional assay, yielding falsely high and low values, respectively. C1-INH functional assay performed within 24 h in serum, EDTA-treated or citrated plasma discriminated well between HAE patients and normals. This was also the case for serum kept at room temperature for up to 5 days, although a modest fall in C1-INH function was seen in the incubation period. For practical purposes we recommend serum as the sample of choice, preferably received within 48 h.

Angioedema↗
PubMed1994

[Angioedema associated with ACE inhibitors].

Angio-oedema as an adverse effect of angiotensin converting enzyme-inhibitors (ACE-inhibitors) is reported to occur in one to two of 1,000 treated patients per year. This estimate may be too low, since the swelling of face, lips, tongue and throat is very often attributed to food allergy. Furthermore, attacks of oedema may occur after months or even years of ACE-inhibitor treatment, thereby obscuring the link to the medication. This adverse effect is non-immunogenic. The oedema is explained by local enhancement of bradykinin, which under normal circumstances is broken down by angiotensin converting enzyme. The question of what makes some people vulnerable is still unsolved. The oedema can develop quickly and may lead to suffocation. In many cases, conventional antiallergic treatment in the form of steroids, antihistamines and epinephrine has inadequate effect. Intubation of the larynx may be necessary, but can be extremely difficult in the case of massive glassy oedema. Involvement of the neck can sometimes hinder tracheotomy, making early intervention essential. The authors describe two patients with moderate swellings, and discuss a potential, yet experimental, use of C1-inhibitor concentrate in the treatment of ACE-inhibitor provoked angioedema.

Angioedema↗
PubMed1994

C1 inhibitor and diagnosis of hereditary angioedema in newborns.

Symptoms of hereditary angioedema may present during the child's first years. Attacks may be a particular threat to the narrower airway of the child. An early diagnosis is most valuable because effective C1 inhibitor (C1 INH) concentrate is available. We present a reference area for the antigenic and functional determination of C1 INH by using uncontaminated umbilical cord blood from 80 normal newborns collected by puncturing vessels in the newly delivered placenta. We examined two full-term babies (1 and 2) from mothers with hereditary angioedema type I the same way. The concentration of C1 INH antigen was determined by radial immunodiffusion. The C1 INH functional assay was based on the addition of a known quantity of C1s, which enzymatically splits a chromogenic substrate. The test was performed in the presence of methylamine and heparin in a kinetic microtiter plate assay. Citrated plasma was used in both assays. The data obtained in the 80 cord blood samples (2.5-97.5 percentile) were 0.11-0.22 g/L for C1 INH antigen (adults, 0.15-0.33 g/L) and 47.2-85.9% for C1 INH function (percentage of adults). In cord blood, baby 1 had an antigenic value of 0.12 g/L (7.5 percentile) and C1 INH function of 61.8% (42 percentile). The corresponding values for baby 2 in cord blood were less than 0.05 g/L (0.106 g/L < 2.5 percentile) and 34.3% (12.9% < 2.5 percentile). Baby 2 had markedly lower C4 values yet much higher C4 activation products than baby 1. At 4 mo, baby 1 had an antigenic C1 INH value of 0.24 g/L.(ABSTRACT TRUNCATED AT 250 WORDS)

Angioedema↗
PubMed1993

[Complement analysis tests].

Although routine analysis of the complement system is rarely demanded, it is important from a diagnostical and therapeutical point of view to include the relevant tests when clinically required. Complement analysis should be performed when hereditary angioedema (C1 inhibitor deficiency) is suspected, and as part of investigation of patients with immunodeficiency (recurrent infections in childhood). Furthermore, complement analysis may be required in cases of recurrent systemic neisserial infections at any age, and to follow up certain autoimmune diseases. Only a few tests are available for routine purposes. These are reviewed briefly, with emphasis on collection and storage of samples prior to analysis. It is important that these guidelines be followed, to avoid misinterpretation of the results.

Angioedema↗
PubMed1992

The justifications and controversies of panendoscopy--a review.

The triple endoscopies of direct laryngoscopy, bronchoscopy, and esophagoscopy, commonly known as panendoscopy, are frequent surgical procedures in otorhinolaryngology. Their purpose is to diagnose additional primary malignancies. The incidence of multiple synchronous primary malignancies has been reported between 3% and 13%. This unusually high rate is attributed to the process of field cancerization in which an anatomical region is exposed to a surface carcinogen and multifocal areas undergo irreversible change to multifocal malignancies. The majority of additional malignancies are diagnosed within 2 years of the index tumor's diagnosis, and are most consistently diagnosed within routine panendoscopy. Nevertheless, a significant percentage (40%) of additional primary malignancies present after this time and consequently, long-term follow-up is essential to the timely diagnosis of metachronous lesions. The predominant controversies surrounding panendoscopy involve the comparable diagnostic efficacies between endoscopic procedures and various radiologic studies. Although the operative complications reported for panendoscopy have been minimal, the expense is considerably greater than that of the respective radiologic alternatives. After a critical review of the literature, the use of endoscopy has been found to be superior to CXRs and barium swallows as the principal means of diagnosis of upper aerodigestive tract cancers. Specifically, bronchoscopy with bronchial washings should be obtained via passage of the bronchoscope through the endotracheal tube in order to decrease contamination. Positive washings are significant and demand further evaluation. Negative washings, on the other hand, yield little information.(ABSTRACT TRUNCATED AT 250 WORDS)

Bronchoscopy↗
PubMed1991

[Hereditary angioedema].

Hereditary angioedema is a rare disease, transmitted as an autosomal dominant trait. The disease usually manifests as acute subcutaneous or submucosal swellings and abdominal pain of two to five days duration. When localised to head or throat life-threatening edema of the larynx may occur. The disease is caused by reduced amount of active C1 inhibitor. The edema is not due to an allergic reaction and medication for such is of little or no value. Profylactic medication may be given when the attacks are frequent and ought to be given before dental extractions, intubation of the larynx, major surgery especially of the oropharynx, and births. The acute attack is best treated by intravenous injection of C1 inhibitor concentrate.

Angioedema↗
PubMed1989

[Hereditary angioedema].

Hereditary angioedema is a rare disease, transmitted as an autosomal dominant trait. The disease usually manifests as acute subcutaneous or submucosal swellings and abdominal pain of two to five days duration. When localized to head or throat, life-threatening edema of the larynx may occur. The disease is caused by reduced amount of active C1 inhibitor. The edema is not due to an allergic reaction and medication for such is of little or no value. Prophylactic medication may be given when the attacks are frequent and ought to be given before dental extractions, intubation of the larynx, major surgery especially of the oro-pharynx, and birth. An acute attack is best treated by intravenous injection of C1 inhibitor concentrate.

Angioedema↗