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Biomedical subjects

H Englert

Publications and source records attributed to H Englert.

35 records · Page 2Linked to original sources
PubMed1995

Immunogenetic analysis of 5 families with multicase occurrence of scleroderma and/or related variants.

OBJECTIVE: To investigate the relative contribution of the major histocompatibility (MHC) gene complex in the etiopathogenesis of familial scleroderma and/or its variants, in 5 Australian families, each with 2 affected members. METHODS: Affected individuals and consenting first degree relatives were examined and had blood collected for histocompatibility leukocyte (HLA) class I, class II antigen, and complement C4 typing. An antinuclear (ANA) profile screen on each family member was performed. RESULTS: Family 1, had 2 affected siblings (scleroderma, CREST), each anticentromere positive (> 1/640 titer), with identical HLA haplotypes. A brother, who was HLA identical to his affected sisters was clinically normal and ANA negative. In Family 2, there were no HLA haplotype similarities between the 2 sisters affected with diffuse scleroderma. Both were ANA positive (> 1/640). A 3rd sister was HLA identical to the proband but was clinically normal and ANA negative. In Family 3, affected siblings (CREST, morphea) had identical HLA haplotypes. In Family 4, both mother (CREST) and one of her 2 daughters (scleroderma) had anticentromere antibodies (1/2560). The unaffected daughter, not sharing either of her sister's haplotypes, was normal and ANA negative. In Family 5, 2 sisters (CREST, CREST) were HLA identical. CONCLUSION: A female predominance in familial scleroderma was observed. There was no common HLA haplotype between different families affected with scleroderma or its disease variants. Within families (except in one case, where the possibility of crossover exists or a question of paternity) affected siblings shared both HLA haplotypes. The development of disease was not totally accounted for by HLA genes, since family members with the same HLA haplotypes as the proband, were not affected. It appears that genes within the MHC complex are required but are not sufficient for the development of systemic sclerosis.

Adolescent↗
PubMed1995

Limited scleroderma is associated with increased prevalence of macrovascular disease.

Microvascular involvement in scleroderma is well recognized. Macrovascular disease is not. OBJECTIVE. To test the hypothesis that the prevalence of macrovascular disease is increased in patients with limited scleroderma (systemic sclerosis, lSSc). METHODS. A retrospective cohort study design was employed in which the prevalence of macrovascular disease in all female patients from specified hospitals (1974-90) with lSSc of at least 5 years' duration was compared and contrasted with that in a comparable group of controls. Each control was matched to one lSSc case by sex; age (+/- 5 yrs); number of inpatient admissions (+/- 2); year of last hospital admission (+/- 2 yrs), history of hypertension, cigarette smoking and diabetes mellitus, and medical record number most closely approximating the case. The distribution of vascular disease was assessed in the peripheral, coronary, and cerebral arterial territories. RESULTS. Peripheral macrovascular disease (PVD) occurred in 18 (58%) of the lSSC patients and only 3 (9.6%) of the controls (RR = 6.0; 95% CI 2.0-18). Of the 18 lSSc cases, 8 had PVD documented angiographically, 4 by arterial Doppler ultrasound, and 6 had absent peripheral pulses. Five of these required subsequent partial limb amputation. Two of the 3 controls with PVD had absent peripheral pulses, and 1 had an angiographically documented abdominal aortic aneurysm. No control required limb amputation. There was no significant difference in the prevalence of coronary artery or cerebrovascular disease between the groups. CONCLUSION. The prevalence of peripheral large vessel occlusive disease is increased in lSSC and associated with severe morbidity.

Adult↗
PubMed1993

Large vessel occlusive disease associated with CREST syndrome and scleroderma.

OBJECTIVES: To report the cases of three patients with CREST syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors. METHODS: The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients. RESULTS: These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with CREST syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma. CONCLUSIONS: These cases suggest an association of both the CREST syndrome and scleroderma with macrovascular disease.

Aged↗
PubMed1992

Reproductive function prior to disease onset in women with scleroderma.

We tested in a retrospective study the hypothesis that women with scleroderma have an increased risk, before disease presentation, of problems in pregnancy. Reproductive history, as reported from a validated postal questionnaire, was obtained from 204 women with scleroderma [mean year of birth (yob) 1942] and compared to that reported by 233 women with primary Raynaud's phenomenon (mean yob 1948) and 189 healthy women from a population register (mean yob 1950). Only pregnancies occurring before symptom onset in the scleroderma group were considered for analysis. All analyses were adjusted for job, social class, age at first pregnancy, smoking habit, and where relevant, parity. The results are expressed as odds ratios (OR) [with 95% confidence limits (CL)] giving the risk of developing scleroderma, with previous exposure to each of the specific reproductive variables analyzed, relative to the 2 comparison groups. The women with scleroderma were more likely than the population women to have had a delay in conception (> or = 12 months): OR 2.6 (1.1, 5.7) or be infertile: OR 2.3 (0.7, 7.2). These differences were not apparent when the scleroderma group was compared to the women with Raynaud's: OR's 0.7 and 1.1, respectively. Similarly there was a greater self report of maternal ill health in pregnancy in the group with scleroderma compared to the population women: OR 2.1 (1.2, 3.5) but not compared to the women with Raynaud's: OR 1.0 (0.7, 1.6).(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗
PubMed1991

A double blind, randomised, multicentre comparison of two doses of intravenous iloprost in the treatment of Raynaud's phenomenon secondary to connective tissue diseases.

OBJECTIVE: To compare low (0.5 ng/kg/min) and standard dose (2 ng/kg/min) iloprost (a stable carbacyclin analogue of prostacyclin) in patients with Raynaud's phenomenon secondary to connective tissue disorders. DESIGN: Double blind, random allocation, three six hour infusions on consecutive days. Follow up period eight weeks. SETTING: Rheumatology units, five teaching hospitals. PATIENTS: 55 Patients with Raynaud's phenomenon (greater than seven attacks per week), 32 secondary to well documented classical progressive systemic sclerosis (American Rheumatism Association criteria), 11 CREST syndrome, 5 mixed connective tissue disease, 1 rheumatoid arthritis, 1 Sjögren's syndrome, 1 childhood dermatomyositis, and 4 abnormal nailfold capillaroscopy and antibody profiles but no definite diagnosis. INTERVENTIONS: All other treatment for Raynaud's phenomenon was discontinued two weeks before entry. 28 Patients were randomly allocated to receive the low dose, 27 the standard dose. Differing dilutions allowed infusion rates to be started at 10 ml/h with increments of 10 ml/h every 15 minutes until infusion rates reached 0.5 ng/kg/min and 2 ng/kg/min respectively. MAIN OUTCOME MEASURE(s)--Reduction in frequency, duration, and severity of attacks of Raynaud's phenomenon. Assessment of ulcer and ischaemic lesion healing. RESULTS: Both dosage regimens were equally effective in reducing severity, frequency, and duration of Raynaud's attacks. Ulcer healing occurred to similar degree in both treatment groups (standard dose 44%, low dose 39%). Low dose was associated with significantly fewer side effects. CONCLUSIONS: Both dosage regimens reduce severity of Raynaud's phenomenon and encourage ulcer healing. Low dose was associated with fewer side effects and was better tolerated by the patients.

Adult↗
PubMed1988

Binding of loop diuretics to their renal receptors: use as a screening model for potential diuretic activity.

Loop diuretics of the benzoic acid and aryloxyacetic acid families inhibit Na+K+Cl- cotransport. The ranking order of potencies measured in the thick ascending limb of Henle's loop and the ranking order of affinities for [3H]piretanide receptors on renal plasma membranes are the same. Potencies and affinities correlate well (correlation coefficient r = 0.959 for the medulla and r = 0.951 for the cortex). Therefore, measurement of [3H]piretanide binding is proposed to facilitate screening for loop diuretic action.

Animals↗
PubMed1986

Incomplete Reiter's syndrome following chemotherapy of acute myeloid leukaemia.

Reiter's syndrome and other reactive arthritides have been described following infection with various organisms although they can occur in unusual circumstances without an obvious infectious precipitant. We have recently witnessed two attacks of reactive arthritis and keratoderma blenorrhagica occurring in an HLA B27 adult male following chemotherapy on two separate occasions with the same drugs for acute myeloid leukaemia. No attacks occurred before or following the cessation of these drugs. This supports the view that in Reiter's syndrome a common pathogenic pathway is triggered by an 'arthritogenic factor' which in this case appears to have been chemical.

Adult↗
PubMed1985

Diphenylamine-2-carboxylate, a blocker of the Cl(-)-conductive pathway in Cl(-)-transporting epithelia.

The present study examines the effects of diphenylamine-2-carboxylate (DPC) in Cl(-)-transporting epithelia. This substance blocks reversibly the Cl(-)-conductance present under normal circumstances in the basolateral membrane of the thick ascending limb of the loop of Henle (TAL) and in the apical membrane of shark rectal gland tubules (RGT). This leads to a reduction in active NaCl reabsorption (TAL) and NaCl secretion (RGT) respectively, as measured by the equivalent short circuit current. The cells hyperpolarize as the membrane voltage drifts from the control value (some compromise between the chemical potential of Cl- and K+) towards the chemical potential of K+. The resistance of the basolateral (TAL) or apical membrane (RGT) increases and this leads to a moderate increase in transepithelial resistance. In addition, the Cl(-)-concentration step induced membrane voltage changes, which can be produced under control conditions, disappear in the presence of the blocker. Finally, experiments in excised membrane patches indicate that this substance inhibits the single current events of individual Cl(-)-channels.

Animals↗
PubMed1985

Anticardiolipin antibodies in autoimmune thrombocytopenic purpura.

Using a recently devised solid phase radioimmunoassay to detect anticardiolipin antibodies, we report the presence of these antibodies in 30 of 96 patients with chronic autoimmune thrombocytopenic purpura (AITP). IgG anticardiolipin antibody levels were elevated in 14 patients and IgM anticardiolipin antibody levels were elevated in 27 patients. We suggest that these antibodies may mediate peripheral platelet destruction by binding to phospholipids in the platelet membrane. It is also conceivable that the presence of anticardiolipin antibodies may select a subpopulation of patients with chronic AITP who may go on to develop other autoimmune disorders, such as systemic lupus erythematosus.

Antibodies↗
PubMed1985

Neurology and the lupus anticoagulant.

The lupus anticoagulant, an immunoglobulin of the IgG or IgM class, is one of a group of antiphospholipid antibodies. Although an anticoagulant in vitro, its action in vivo is that of a procoagulant. This procoagulant activity may involve many organ systems including the nervous system. Thus far cerebral thrombosis, spinal thrombosis, chorea and Guillain-Barré syndrome have been described in association with the lupus anticoagulant. Although the lupus anticoagulant is an uncommon cause of neurological disease, it must be considered, especially in a setting of a prolongation of the common pathway of coagulation, thrombosis and other autoimmune phenomena.

Blood Coagulation↗
PubMed1985

Affinity purified anti-cardiolipin and anti-DNA antibodies.

Recent studies have raised questions concerning the specificity of anticardiolipin antibodies and their relationship to anti-DNA antibodies, the lupus anticoagulant, the biological false positive test for syphilis, and reagin, the antibody detected in syphilis. In an attempt to answer some of these questions, 3 IgG and 2 IgM affinity purified anticardiolipin antibodies, as well as 3 affinity purified anti-DNA antibodies were studied. Affinity purified anti-cardiolipin antibodies showed high binding to negatively charged phospholipids but not to ssDNA by solid phase radioimmunoassay. On the other hand, affinity purified anti-DNA antibodies did not bind cardiolipin. Inhibition experiments showed that negatively charged phospholipids and VDRL liposomes inhibited the binding of anticardiolipin antibodies to phosphatidylserine, but ssDNA, alpha-glycerol phosphate and hyaluronic acid did not. Similar studies of sera from patients with high anticardiolipin antibody levels supported the results obtained with affinity purified anticardiolipin antibodies. These results suggest that anticardiolipin antibodies bind negatively charged phospholipids and there appears to be little crossreactivity with DNA or unrelated negatively charged polymers such as hyaluronic acid. Both the negatively charged phosphodiester group and glyceride portions of the phospholipid molecules appear important for their antigenicity. Four of the 5 affinity purified anti-cardiolipin antibodies had lupus anticoagulant activity providing further evidence to suggest that these 2 groups of antibodies have the same or very similar specificities. Studies of sera from 3 patients with syphilis showed that VDRL liposomes inhibited reagin activity to a greater extent than did cardiolipin. On the other hand, in patients with autoimmune disorders, cardiolipin inhibited anticardiolipin antibody activity to a greater extent than did VDRL liposomes.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies↗