Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a patient with progressive systemic sclerosis (PSS), osteolysis of the posterior portion of the rib cage developed in an insidious fashion, without symptoms or preceding trauma. Six previous examples of rib resorption in PSS are reviewed. The destructive mechanism is unknown but may be related to endarteritis and ischemia.
 ...
PMID:Osteolysis of the ribs in progressive systemic sclerosis. 48 4

The treatment of digital ischemia in systemic sclerosis remains inadequate. We report a double blind, placebo controlled trial of recombinant tissue plasminogen activator (rtPA), a potent thrombolytic agent. Ten patients received rtPA. A potent, acute fibrinolytic effect was observed. During the infusion of rtPA, improvements in skin blood flow were seen. These improvements were shortlived.
 ...
PMID:A double blind placebo controlled trial of recombinant tissue plasminogen activator in the treatment of digital ischemia in systemic sclerosis. 161

Treatment of severe hand ischemia a associated to progressive systemic sclerosis and other disorders is controversial. We studied prospectively 45 patients, 41 female and 4 males, over an 11 year period. Age ranged from 16 to 73 years, mean 46. Underlying disease was systemic sclerosis in 30, CREST in 8, overlapping syndrome in 4, systemic lupus in 1 and non rheumatic vasculitis in 2 patients. Treatment consisted of intravenous injection of reserpine, 1 mg, at a superficial arm vein after controlled local circulatory block for 15 min. Regional anesthesia was required in 38 patients. Adequate follow up was obtained in 32 females and 2 males, receiving a mean of 3.1 therapy sessions (range 1 to 13). Morphologic improvement from 3.09 +/- 0.16 to 1.57 +/- 0.13 and functional improvement from 3.6 +/- 0.12 to 1.75 +/- 0.14 (5 grade scoring system), was observed (p < 0.001). These results correlate with adequate rehabilitation confirmed clinically.
 ...
PMID:[Severe ischemia of the hand. Treatment with regional intravenous sympathicolysis with reserpine]. 184 85

Myocardial thallium-201 (Tl-201) imaging performed in conjunction with exercise stress has enhanced the accuracy of detecting coronary artery disease among patients with chest pain. Sensitivity and specificity of qualitative visual Tl-201 scintigraphy for detection of coronary artery disease average 84% and 87%, respectively. Quantitative analysis of planar Tl-201 scintigrams has yielded sensitivity and specificity in the 90% range. Single photon emission computed tomographic imaging is associated with even higher sensitivity but with specificity in the 82-85% range. Perfusion defects representing ischemia can now be distinguished from scar by demonstration of delayed Tl-201 redistribution or enhanced uptake after reinjection of a second dose of Tl-201. Stenoses of the left circumflex coronary artery are less easily detected than lesions of the right and left anterior descending coronary arteries. False-positive Tl-201 perfusion defects may occur as a result of attenuation artifacts, most often caused by overlying breast tissue or by a high left hemidiaphragm. Patient motion during acquisition of single photon emission computed tomographic images results in artifactual defects on reconstruction. Abnormal Tl-201 uptake has been noted in patients with 1) left bundle branch block and normal coronary arteries, 2) hypertrophic cardiomyopathy, and 3) progressive systemic sclerosis.
 ...
PMID:Diagnostic accuracy of thallium-201 myocardial perfusion imaging. 188 75

Microcirculatory and haemorheological parameters were investigated before and after plasmapheresis in eighteen patients with secondary Raynaud's phenomenon based on progressive systemic sclerosis. After 4 plasmaphereses, once a week, all patients claimed explicit improvement of their complaints. Raynaud's phenomenon and especially the reaction upon cold provocation had disappeared and skin ulcers healed. Red blood cell (RBC) velocity increased significantly (p less than 0.001) after 4 weeks plasmapheresis. RBC aggregation and plasma viscosity were significantly lower (p less than 0.001) after the last plasmapheresis than before treatment. After 3 years four patients were still free of complaints, but in 14 patients the symptoms of Raynaud's phenomenon had reappeared after 6 to 9 months. The skin ulcers, however, did not return in these patients. RBC aggregation and plasma viscosity returned to the initial values after 9 months, while skin capillary blood flow remained significantly enhanced for 24 months. The finding that restoration of RBC aggregation and plasma viscosity to normal level is associated with enhanced skin capillary blood flow, indicates that disturbed haemorheology plays a role in the diminished skin blood flow, as observed in patients with secondary Raynaud's phenomenon. In these patients, plasmapheresis can be considered to treat severe ischemia of the digits.
 ...
PMID:Plasmapheresis in Raynaud's phenomenon in systemic sclerosis: a microcirculatory study. 201 79

The susceptibility of lung tissue to ischemia-reperfusion injury has made distant procurement of heart-lung allografts difficult. The effects of hypothermia, ventilation without perfusion, and various reperfusion solutions (PSS/Ficoll or whole blood) on the development of ischemia-reperfusion lung injury were investigated. Use of an ex vivo rat lung model in which the above variables were individually varied permitted a direct approach for these studies. Normothermic ischemia for 1 hour caused significant damage, documented by increased iodine 125 bovine serum albumin (125I-BSA) in alveolar lavage fluid and lung parenchyma compared with nonischemic controls. Hypothermic (4 degrees C) ischemia for 4 hours in lungs reperfused with salt solution and for as many as 12 hours in lungs reperfused with whole blood caused no significant increase in 125I-BSA in alveolar lavage fluid and lung parenchyma compared with nonischemic controls. Lungs ventilated without perfusion showed no increase in 125I-BSA leakage compared with controls. The ex vivo rat lung model is excellent for studying ischemia-reperfusion injury. It is reproducible, allows for variance of reperfusion solutions, and permits change in temperature and ventilation easily.
 ...
PMID:Hypothermia or continuous ventilation decreases ischemia-reperfusion injury in an ex vivo rat lung model. 265 79

The development of myocardial scintigraphy (MS) reflects the clinical success of a representative procedure in nuclear medicine. Radiopharmaceuticals for visualizing vital and damaged myocardium and techniques (planar-qualitative, planar-quantitative, SPECT-qualitative-quantitative with comparative sensitivities) are briefly reviewed with the main focus on their clinical application in coronary (CHD) and noncoronary heart disease, where recent literature from the United States and Europe is considered. The limited value of MS for screening of CHD is outlined and its present and future role in detecting asymptomatic (silent) ischemia/infarction and symptomatic patients at professional risk is stressed. The present state of MS in coronary heart disease is discussed for single and multivessel disease, previous infarction, and risk stratification (myocardial washout, pulmonary uptake, ischemic dilation, absent heart sign), reflecting the importance of the procedure in exercise-induced ischemia as well as in ischemia at rest for prognostication of the natural and therapeutic course, i.e., therapy control (angioplasty, bypass, lysis, cardiac drugs). More marginal but upcoming clinical indications are mentioned, such as progressive systemic sclerosis, cardiac transplantation, pediatric cardiology, and problems of nephrology/urology. The "normal" values and the impact of digital radiology and of contrast cardiography are touched upon. Preliminary cases with 111In-antimyosin and 99mTc-Isonitriles are presented including correlative results between global ejection fraction determination according to gated 99mTc-isonitrile and conventional 99mTc-erythrocyte ventriculogram (r = 0.75; n = 10).
 ...
PMID:Myocardial scintigraphy--25 years after start. 328 85

Periarteritis Nodosa (P.A.N.) is a systemic connective tissue disease with a variety of manifestations that includes ocular involvement in 20% of cases. The diagnosis of this condition is difficult due to the absence of any specific clinical signs or laboratory findings. However, histologic studies have demonstrated a segmental vasculitis that is often necrotic. Ocular findings frequently include choroidal involvement that is characteristic. Nevertheless, angiographic studies of this disease are extremely rare. The findings in three patients suspected of having P.A.N. are presented. Fluorescein angiography established the diagnosis of P.A.N. in two cases and ruled-out its presence in the third case. In the first case angiography demonstrated a retinal vasculitis with multiple arteriolar and capillary occlusions. There was also ischemic involvement of the choriocapillaris and a mild anterior optic nerve vasculitis. All findings resolved, leaving numerous Elschnig spots. In the second case the angiogram showed acute multifocal ischemia of the choriocapillaris. The ocular examination and fluorescein angiogram in the third case were entirely normal, thereby ruling-out P.A.N. on the basis of insufficient criteria. Acute multifocal choroidal ischemia is present in a variety of rare conditions: Toxemia of pregnancy, Disseminated Intravascular coagulopathy, Moskowitz Disease (T.T.P.), Leukemia and Malignant Hypertension. However, the presence of multifocal choroidal ischemia in the presence of a systemic connective tissue disorder strongly favors the diagnosis of P.A.N. The relative contributions of co-existent Malignant Hypertension and P.A.N. in producing choroidal ischemia are discussed. The spectrum of clinical manifestations and laboratory findings in P.A.N. as well as hypotheses concerning pathogenesis (immune-complex deposition) are described. Among all systemic vasculitis , only P.A.N., and rarely Scleroderma, feature choroidal involvement. This is possibly due to the fact that the degree of vasculitis in P.A.N. is sufficiently severe to cause clinically significant choroidal involvement.
 ...
PMID:[Fluorescein angiography in the diagnosis of periarteritis nodosa]. 614 75

Although membranocystic changes of adipose tissue, characteristic of membranous lipodystrophy, can be found in some cases of limb ischemic necrosis caused by arteriosclerotic obstruction, the incidence of the lesions in several forms of limb ischemia is not known. Subcutaneous fat of 53 patients with limb ischemic necrosis was studied histologically and ultrastructurally for the presence of membranocystic changes. The lesions were found in 11 (38%) of 29 cases of thromboangiitis obliterans, 15 (75%) of 20 cases of arteriosclerotic obstruction, and two (50%) of four cases of progressive systemic sclerosis. The membranous lipodystrophy-like changes can be produced by several forms of chronic circulatory disturbance and they are one of the nonspecific changes of adipose tissue.
 ...
PMID:Incidence of membranous lipodystrophy-like change among patients with limb necrosis caused by chronic arterial obstruction. 654 23

To treat severe painful digital ulcers on progressive systemic sclerosis (PSS) patients, we developed a new combination therapy which included neural blockade, intravenous urokinase, and prostaglandin E1 infusion. All of these are already recognized treatments for circulatory disturbances in PSS. Although each of them alone has a limited effect on the painful ischemic attack in PSS; in stepwise combination, neural blockade for release of vascular spasm and pain, prostaglandin E1 for further vasodilatation, and urokinase for thrombolysis were effective in the treatment of digital ischemia in two PSS patients. This therapy reduced the necrotic areas predicted before therapy and saved fingers from amputation. It also relieved the intolerable digital pain and effected the recovery of digital function.
 ...
PMID:Neural blockade, urokinase and prostaglandin E1 combination therapy for acute digital ischemia of progressive systemic sclerosis. 779 34


1 2 3 4 5 6 7 8 9 10 Next >>