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1.
BACKGROUND: Inhaled nitric oxide (NO) has been shown to selectively lower pulmonary vascular resistance and is applied in patients with pulmonary hypertension (PHT). However, application and monitoring is complex and not always successful ("non-responders"). We evaluated the effect of aerolized prostacyclin (aePGI2) as a therapeutic alternate to NO. PATIENTS AND METHODS: aePGI2 and NO were applied to patients with different causes of pulmonary hypertension (Group 1a: preoperative patients with intracardiac shunting defects and Eisenmenger's disease, n = 30; Group 1b: patients with primary or postoperative PHT, n = 13; Group 2: PHT immediately following surgery for congenital heart disease, n = 6). RESULTS: Pulmonary vascular resistance could be lowered significantly (Group 1a: from 91% of systemic vascular resistance to 58% with NO and 53% with aePGI2; Group 1b: from 20.2 Wood Units*m2 to 13.4 and 11.3; Group 2: from 24.9 Wood Units*m2 to 9.5 and 10.5); cardiac index increased (Group 1b: from 2.96 to 3.55 and 3.96 l/min*m2, Group 2: from 1.57 to 1.89 and 2.00 l/min*m2). CONCLUSIONS: The short-term application of aePGI2 shows a selective pulmonary vasodilation similar to NO. Given adequate monitoring, aePGI2 appears to be useful for the acute treatment of PHT.  相似文献   

2.
Pulmonary hemorrhage is a rare and often fatal complication of systemic lupus erythematosus (SLE). Treatment with high-dose steroids and cyclophosphamide has been of only modest value, with a reported mortality of up to 92%. We have recently seen three patients with active SLE who developed acute life-threatening pulmonary hemorrhage. Diagnostic evaluation of these patients showed negative sputum and blood cultures, negative glomerular basement membrane antibodies, and negative antineutrophilic cytoplasmic antibodies. In two patients, an open-lung biopsy was performed, and histological examination showed granular alveolar immunofluorescence staining for immunoglobulin and complement components. Treatment with plasmapheresis was initiated with prompt resolution of symptoms and clearing of chest radiograph. Two patients had recurrent bleeds despite treatment with cyclophosphamide and high-dose steroids and required repeated intubation. Plasmapheresis resulted in rapid radiographic and clinical improvement on each occasion. Two patients survived long-term and are presently without pulmonary problems; one patient died of sepsis after initial response to plasmapheresis. The dramatic improvement of the pulmonary disease in these patients leads us to conclude that rapid initiation of plasmapheresis should be strongly considered in SLE patients with severe, life-threatening pulmonary hemorrhage.  相似文献   

3.
Mizoribine, a novel immunosuppressive agent developed in Japan, was administered as a monotherapy to a systemic lupus erythematosus (SLE) patient with the clinical symptoms and immunological abnormalities accompanying SLE showing marked improvement. The result of prolonged administration over 22 months in this case showed neither relapse nor side-effects. Reports have been made about mizoribine used concomitantly with steroids in the treatment of SLE; however, there have not been any reports of mizoribine as a monotherapy for SLE being effective. In this case, mizoribine (150 mg/day) was administered without steroids as a monotherapy on a outpatient basis since the patient's condition overall was relatively good and the serious complications of the heart, kidneys, and lungs that accompany SLE were not observed. The results of this treatment showed improvements in alopecia, arthritis, and systemic malaise from about the 4th week after the start of administration, and the clinical symptoms that accompany SLE had completely disappeared in the 8th week. Also, the immunological tests markedly improved. Four months after the start of administration the immunological abnormalities in the anti-DNA antibody, rheumatoid factor, and immune complex were completely corrected. This case showed dramatic improvement in the SLE clinical symptoms and immunological abnormalities with the mizoribine monotherapy as well as the potential for mizoribine monotherapy to maintain a state of remission over the long term.  相似文献   

4.
Dexfenfluramine and fenfluramine greatly increase the risk of developing pulmonary hypertension (PHT). The mechanism of anorexigen-associated PHT (AA-PHT) and the reason PHT occurs in a minority of people exposed are unknown. Anorexigens are weak pulmonary vasoconstrictors, but they become potent when synthesis of the endogenous vasodilator nitric oxide (NO) is suppressed. We hypothesized NO deficiency predisposes affected individuals to develop AA-PHT. A prospective, case-control, study was performed on consecutive patients with AA-PHT (n = 9). Two sex-matched control groups were selected: patients with primary PHT (P-PHT, n = 8) and normal volunteers (n = 12). Lung NO production (VNO) and systemic plasma oxidation products of NO (NOx) were measured at rest and during exercise. AA-PHT developed 17 +/- 6 mo after a short course of anorexigen (6 +/- 2 mo) and was irreversible. VNO was lower in AA-PHT than in P-PHT and correlated inversely with PVR (p < 0.05). The apparent VNO deficiency may have resulted from increased oxidative inactivation of NO in patients with AA-PHT, as their NOx levels were elevated (p < 0.05) in inverse proportion to VNO (r2 = 0. 55; p < 0.02). In susceptible persons, anorexigens can cause an irreversible syndrome of PHT, hypoxemia, and systemic vascular complications after brief exposures. These patients have a relative NO deficiency years after discontinuing the anorexigen, perhaps explaining their original susceptibility.  相似文献   

5.
Anticardiolipin antibodies (aCL) were studied in relation to pulmonary hypertension (PH) in 22 patients with mixed connective tissue disease (MCTD) or systemic lupus erythematosus (SLE). The mean pulmonary arterial pressure (mPAP) values were similar in the 12 MCTD and 10 SLE patients: 26 +/- 11 and 25 +/- 11 mm Hg, respectively. However, the frequency of PH was higher in SLE (60%) than in MCTD patients (33%). The titers of aCL were significantly higher in SLE (38 +/- 27 IU/ml) than in MCTD (17 +/- 7 IU/ml; p < 0.02). Two SLE patients with high titers of aCL had multiple cerebral infarction and transverse myelitis, and deep vein thrombosis, respectively. A significant correlation between the titers of aCL and mPAP was observed in patients with MCTD (p < 0.05), but not in patients with SLE.  相似文献   

6.
A 47 year-old Japanese female who showed transverse myelopathy (TM) due to spinal epidural hematoma diagnosed by MRI in the course of systemic lupus erythematosus (SLE) was reported. She was admitted to Keio University Hospital due to paraplegia, anesthesia of lower extremity, urinary disturbance. Neurological examination revealed transverse disturbance of Th 10. Lumbar spinal cord MRI showed irregular mass that located at epidural region of 9th-11th thoracic vertebrae. When the laminectomy of 9th-11th thoracic vertebrae was performed, hematoma (4.5 cm x 1.5 cm in size) was confirmed and removed completely. Post operative condition was stable and symptoms had been improving gradually. It has been reported that TM associated with SLE was closely related to myelitis. In this case, epidural hematoma was a major cause of TM and MRI was very useful for her diagnosis and treatment. This is the rare case of SLE associated with spinal epidural hematoma and was thought as a important case to consider the cause of neurological complication of SLE.  相似文献   

7.
Although the association between transverse myelitis (TM) and systemic lupus erythematosus (SLE) has been reported previously, it remains a rare clinical condition. Our experience treating three women with lupus TM within a few months is presented. In each patient, spinal cord dysfunction was accompanied by laboratory or clinical evidence of SLE. Further neurologic manifestations, such as optic neuritis, developed in all patients, suggesting other diagnoses, including multiple sclerosis (MS), Devic's syndrome, and lupoid sclerosis. The outcomes for these three women were severe disability, death, and moderate disability, respectively. The severity of outcome did not seem to correlate with the timing or intensity of treatment. Physiatrists should be aware of the potential for neurologic progression in lupus TM, because patients with this condition invariably require rehabilitation.  相似文献   

8.
Pulmonary hemorrhage (PH) is a rare but potentially life-threatening manifestation of systemic lupus erythematosus (SLE). In this report we describe a 13 year old girl with PH as the sole presenting clinical manifestation of her SLE. Her serology was diagnostic of SLE and one year after presentation she developed arthritis. She had a rapid serologic but delayed clinical response to combination therapy of intravenous pulse methylprednisolone, pulse cyclophosphamide and daily prednisone. Awareness of the possibility of pulmonary hemorrhage as a presentation of SLE may aid in the diagnosis and early, aggressive management of this condition.  相似文献   

9.
The effect of preliminary heat treatment (PHT) for hydrated cellulose fibers impregnated with boric acid on the composition and structure of nitrided products is studied. Subsequent nitriding after PHT in helium leads to formation of carbon fibers coated with boron nitride, but after PHT in air there is formation of hexagonal and rhombohedral boron nitride powder. Data obtained are compared with results reported previously.  相似文献   

10.

The effect of preliminary heat treatment (PHT) for hydrated cellulose fibers impregnated with boric acid on the composition and structure of nitrided products is studied. Subsequent nitriding after PHT in helium leads to formation of carbon fibers coated with boron nitride, but after PHT in air there is formation of hexagonal and rhombohedral boron nitride powder. Data obtained are compared with results reported previously.

  相似文献   

11.
OBJECTIVE: To determine whether pulmonary hypertension developed in a coronary artery-ligated rabbit model of left ventricular dysfunction (LVD) and to examine the effects of i.v. 5-hydroxytryptamine (5-HT) and endothelin-1 (ET-1) on pulmonary arterial pressure (PAP). METHODS: Eight weeks after experimental coronary artery ligation or sham operation, ejection fractions were assessed by echocardiography. The rabbits were later anaesthetised and pulmonary arterial pressure was measured via a catheter inserted into the pulmonary artery via the right external jugular vein. 5-HT (1-400 micrograms/kg) and ET-1 (0.001-4 nmol/kg) were administered i.v. RESULTS: Ejection fraction was significantly decreased from 76.6 +/- 1.4% in sham-operated to 42.2 +/- 1.3% in coronary artery-ligated rabbits (n = 9 in each group; P < 0.001), consistent with LVD. Baseline mean pulmonary arterial pressure was significantly increased in the coronary artery-ligated group compared to the shams, (16.5 +/- 0.5 vs. 11.5 +/- 0.8 mmHg; P < 0.001). A significant degree of right ventricular hypertrophy was found in the coronary artery-ligated rabbits (0.70 +/- 0.04 g/kg final body weight (f.b.wt.), n = 8 cf. 0.48 +/- 0.02 g/kg f.b.wt. in sham-operated controls, n = 8; P < 0.001). There was a significant increase in the percentage of muscularised pulmonary vessels adjacent to alveolar ducts and alveoli < 60 microns i.d. in the LVD rabbits compared with their sham-operated controls (8.5 +/- 0.4 cf. 20 +/- 0.5%; P < 0.0005). 5-HT produced a greater response in the coronary artery-ligated rabbits (a maximum increase of 8.7 +/- 1.0 mmHg in mean pulmonary artery pressure vs. 4.6 +/- 1.5 mmHg for sham-operated controls; P < 0.05). ET-1 did not have any effect on pulmonary arterial pressure in either group. CONCLUSION: In the rabbit, LVD secondary to coronary artery ligation, causes right ventricular hypertrophy, pulmonary vascular remodelling, and an increased PAP consistent with the onset of pulmonary hypertension (PHT). The greater PAP response to i.v. 5-HT in the PHT group supports the hypothesis that this substance could be involved in the development of PHT. A role for ET-1 cannot be excluded, despite its lack of effect on PAP when intravenously administered in either group.  相似文献   

12.
Changes in sex hormone metabolism seem to play a role in the expression of systemic lupus erythematosus (SLE). Epidemiological studies have demonstrated an increased risk of developing SLE in women using oestrogens for more than ten years. Onset or aggravation of symptoms have been described in case reports and small retrospective series of SLE patients. Oestrogen replacement therapy is generally well tolerated whereas oral contraceptives containing oestrogen can induce flares in a small proportion of SLE patients. A generally negative attitude towards oestrogens seems inadequate since controlled prospective studies are lacking. Patients with stable disease may use oestrogen provided there is close follow-up, particularly during the first six months of treatment. Treatment with oestrogen is contraindicated in SLE patients with a history of thromboembolism, positivity for phospholipid antibodies, and in the presence of severe organ involvement.  相似文献   

13.
Objective: Working memory (WM) deficits have been reported previously in systemic lupus erythematosus (SLE), but the relationship between information processing speed (PS) and WM deficits in SLE is unknown. This study examined whether or not PS slowing could account for the WM deficits observed in SLE. Method: A visual n-back task was used to measure simple and complex PS and WM in 40 SLE patients and 36 healthy controls. Simple PS was defined as reaction time (RT) to correct responses under a very low WM load condition (0-back), while complex PS was defined as RT to correct responses under moderate and high WM load conditions (1 and 2-back). Results: The results showed that SLE patients performed as well as the controls at the lower WM load conditions but had fewer correct responses than controls under the highest WM load condition (2-back). SLE patients had slower RTs than controls under all conditions, but they had relatively greater RT slowing than controls under the higher WM load conditions. Further, when RT for simple PS was subtracted from complex PS, SLE patients still showed slower complex PS for the 1- and 2-back compared with controls. Both simple and complex PS slowing were related to poorer accuracy scores on the 2-back condition, only for the SLE group. Conclusions: The n-back task provides a sensitive measure of PS and WM. The results suggest that PS deficits alone could not account for the WM deficits in SLE. Disease duration, disease activity, and depression did not appear to account for the observed PS and WM deficits. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

14.
Anti-phospholipid antibodies (aPL) are concerned with many central nervous system diseases in systemic lupus erythematosus (SLE). However, no report has described the relationship between aseptic meningitis and aPL in SLE. We report a case of SLE with aPL, presenting cerebral infarction and aseptic meningitis. A 14 year old female with SLE with aPL experienced cerebral infarction and recurrent aseptic meningitis. Combination therapy with steroids and aspirin improved the condition and prevented relapses. The aPL are associated with cerebral infarction, even in young patients with SLE. In addition, aPL may induce aseptic meningitis in SLE.  相似文献   

15.
OBJECTIVE: To investigate how sera from 37 patients with systemic lupus erythematosus (SLE) stimulate interleukin (IL) 6 release from IL-1beta pretreated endothelial cells and compare these effects to those of sera from 16 normal controls. METHODS: Endothelial cells pretreated 18 h with IL-1beta (5 U/ml) were incubated 2 h with sera diluted 10-fold with phosphate buffered saline (PBS). IL-6 concentrations in endothelial culture supernatants collected after incubation were measured by ELISA. RESULTS: Compared with PBS, sera from controls and 24 patients with SLE suppressed IL-6 release from IL-1beta pretreated cells. However, sera from 13 patients with SLE augmented IL-6 release. Of note, sera from 5 patients with pulmonary hypertension induced the highest level of IL-6 release. IgG from control sera suppressed IL-6 release, whereas F(ab')2 did not. Both IgG and F(ab')2 from the sera of patients with SLE with pulmonary hypertension augmented IL-6 release from IL-1beta pretreated cells. CONCLUSION: IgG antiendothelial cell antibodies from patients with SLE may be associated with the pathogenesis of SLE and pulmonary hypertension.  相似文献   

16.
Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects young women. The respiratory system is more commonly involved in SLE than in any other collagen vascular disease. SLE may affect virtually all components of the respiratory system, including the upper airway, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles. Respiratory system involvement ranges from symptomatic to fulminant and life threatening. This article reviews the pulmonary manifestations of SLE, including drug-induced SLE.  相似文献   

17.
Hypocomplementaemic urticarial vasculitis syndrome is a leukocytoclastic vasculitis characterized by urticarial lesions, associated with fever, arthralgias, arthritis and abdominal pain. Other systemic manifestations include glomerulonephritis, uveitis, episcleritis, chronic obstructive pulmonary disease and neurological abnormalities. Some case associated with systemic lupus erythematosus have been described and SLE diagnosis was made by previous or concomitant diagnostic criteria before onset of urticarial vasculitis. Urticarial vasculitis prior to SLE diagnosis is rare. The development of anti-Ro/SS-A antibody for the diagnosis of SLE is emphasized. The authors alert to the importance of periodically searching for this marker in patients with urticarial vasculitis.  相似文献   

18.
OBJECTIVE: To describe the relative risk for venous thrombosis (VT) associated with antiphospholipid antibodies (aPL) in systemic lupus erythematosus (SLE). DESIGN: Systematic review and meta-analysis of 26 articles that examined the association between aPL and VT in SLE. SETTING: Mostly secondary and tertiary referral centres. PATIENTS: 2249 patients with SLE, 1120 tested for LA (lupus anticoagulant) and 1563 tested for aCL (anticardiolipin antibodies). MAIN OUTCOME MEASURES: A summary of study characteristics and a critical appraisal of study quality were done. Two statistical combinations of 18 primary studies that examined the association of VT and LA and of 14 studies that examined the association of VT and aCL were performed to estimate the risk for VT associated with aPL. RESULTS: The odds ratios of the risk of VT related to the LA summarized from 18 studies were 5.61 [95% CI; 3.80-8.27] overall, 6.32 [CI; 3.71-10.78] for deep venous thrombosis and pulmonary embolism, 11.6 [3.65-36.91] for recurrent venous thrombosis after the first event. The odds ratios of the risk of VT related to aCL summarized from 14 studies were 2.17 [95% CI; 1.51-3.11] overall, 2.50 [CI; 1.51-4.14] for deep venous thrombosis and pulmonary embolism, 3.91 [1.14-13.38] for recurrent venous thrombosis after the first event. CONCLUSIONS: Patients with SLE and LA are at approximately six times greater risk for VT than patients without LA, whereas patients with SLE and aCL are approximately two times greater risk for VT than patients without aCL. We have identified important methodologic limitations and differences in study characteristics. Other risk factors for VT have not been thoroughly evaluated in these studies. Further studies are needed that provide an accurate estimate of the absolute risk for aPL related VT.  相似文献   

19.
The effect of preliminary heat treatment (PHT) of hydrated cellulose fibers impregnated with an ammonium tetraborate solution on the composition and structure of nitrided products was studied. The PHT conditions which upon subsequent nitriding led to the formation of C—BN fibers with a coating of BN, and also hollow and solid BN fibers, were determined Institute for Problems of Materials Science, Ukraine National Academy of Sciences, Kiev. Translated from Poroshkovaya Metallurgiya, Nos. 5–6(407), pp. 30–36, May–June, 1999.  相似文献   

20.
174 alcoholics (mean age 41.37 yrs) were randomly assigned to partial hospital treatment (PHT) or extended inpatient (EIP) rehabilitation after inpatient evaluation and/or detoxification. 12-mo follow-up results for the 115 Ss who consented to continue in the study show few differences in clinical outcomes between the PHT and EIP groups. Both reported more than 80% abstinent days during follow-up, and over 70% had a full-time occupational role, although almost a third experienced job losses during the year. Ss showed significant improvements in psychological well-being and social behavior. One-third were rehospitalized during the follow-up year. Costs for the PHT group were significantly lower than the EIP group, leading to an overall conclusion that PHT provides a cost-effective alternative to EIP treatment for many alcoholics. Implications for health care planning are addressed. (30 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

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