Uncommon Connective Tissue Disorders in Childhood

1974 ◽  
Vol 19 (2) ◽  
pp. 65-75 ◽  
Author(s):  
K. M. Goel ◽  
R. A. Shanks

Nineteen cases of the rarer connective tissue disorders diagnosed between 1948 and 1972 have been reviewed. Of these 6 were of systemic lupus erythematosus (SLE), 3 of dermatomyositis and 5 each of progressive systemic sclerosis (PSS) and polyarteritis nodosa (PAN). Of the 6 cases of SLE, 4 were of true SLE, one of ethosuximide induced lupus syndrome and one newborn with placental transfer of lupus erythematosus factor. Three cases of true SLE died of renal failure. None of the patients with dermatomyositis or PSS died. Of the 5 cases of PAN, 2 died and one could not be traced. The disease is quiescent in the other two.

Author(s):  
Colin Berry

This chapter describes the anaesthetic management of the patient with those musculoskeletal disorders which are relevant to anaesthetic practice. Topics covered include rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, systemic sclerosis, scoliosis, and achondroplasia. For each topic, preoperative investigation and optimization, treatment, and anaesthetic management are described.


1992 ◽  
Vol 68 (05) ◽  
pp. 516-520 ◽  
Author(s):  
M Jurado ◽  
J A Páramo ◽  
M Gutierrez-Pimentel ◽  
E Rocha

SummaryWe studied the fibrinolytic response before and after venous occlusion (VO) in 30 patients with systemic lupus erythematosus (SLE), 25 with rheumatoid arthritis (RA) and 25 with different connective tissue disorders. Results were compared in patients with and without antiphospholipid antibodies (APA) and a history of either thrombosis or abortions. Before occlusion plasma levels of tissue-type plasminogen activator (t-PA) antigen and its inhibitor (PAI-1) were significantly higher in the patient group (p <0.001). After occlusion, a low fibrinolytic activity on fibrin plates (p <0.005) was observed in the same group. t-PA capacity and t-PA release were similar in relation to controls. The plasma PAI-1 activity was significantly elevated in each group of patients (p <0.005) as compared to the control group. No significant differences with respect to t-PA and PAI-1 were observed in patients as to the presence or absence of thrombosis. There was also no correlation between the fibrinolytic changes and the presence of APA. It is concluded that an impairment of the fibrinolytic system, mainly related to increased PAI-1 levels, is present in most patients with connective tissue disorders, although these changes did not correlate with the presence of APA or the incidence of thrombosis.


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