Antiphospholipid antibodies syndrome

OBJECTIVE: To measure the prognostic utility of helper T-cell (CD4) counts in human immunodeficiency virus (HIV)-infected patients undergoing major abdominal surgery. DESIGN: Retrospective case series. SETTING: Three university-affiliated hospitals. PATIENTS: Forty-three HIV-infected patients undergoing major abdominal surgery. MAIN OUTCOME MEASURES: Morbidity and mortality rates with respect to CD4 cell counts. RESULTS: Nineteen of 32 patients who had CD4 cell counts less than 0.20 X 10(9)/L (200 cells/microL) suffered major complications compared with 2 of 11 patients who had CD4 cell counts greater than 0.20 x 10(9)/L (200 cells/microL) (P=.03). Perioperative mortality was 38% for patients with CD4 cell counts less than 0.20 x 10(9)/L, and was 9% for those with CD4 cell counts greater than 0.20 x 10(9)/L (P=.13). Six months postoperatively, mortality rates were 47% and 9%, respectively (P=.03). Of patients with septic processes perioperatively (n=12), mortality was 75%, and was 19% (P=.009) for those with nonseptic processes (n=31). Nine patients had HIV-related intra-abdominal pathologic conditions at laparotomy. Mortality was 56% perioperatively (P=.13) and 88% after 6 months (P=.001). Sixty-eight percent of patients who received blood product transfusions developed complications, whereas only 7% of those who did not receive transfusions developed complications (P<.001). Overall mortality and morbidity rates were 37% and 49%, respectively. Patients with morbidity had lower CD4 cell counts (median, 0.034 x 10(9)/L) than those without complications (median, 0.102 x 10(9)/L) (P=.02). Similarly, patients who died had lower CD4 cell counts (median, 0.031 x 10(9)/L vs 0.088 x 10(9)/L) (P=.05). CONCLUSIONS: Patients with acquired immunodeficiency syndrome-defining CD4 cell counts undergoing major abdominal surgery developed more complications and had poorer outcomes at 6-month follow-up compared with HIV-infected patients whose CD4 cell counts were greater than 0.20 x 10(9)/L (200 cells/microL). A perioperative septic process and HIV-related pathologic conditions seen at laparotomy are also associated with worse outcomes.     

Antiphospholipid antibody syndrome: current concepts

Antiphospholipid antibody syndrome is a well-recognized cause of hypercoagulability and has multiple manifestations. Patients with an unexplained thrombotic event should be evaluated for the disorder, which can be primary or secondary to another autoimmune disease. The mechanisms of thrombosis are still being elucidated, and the optimal therapeutic regimens remain controversial.     

Antiphospholipid syndrome associated with progressive systemic sclerosis

Hemifacial spasm is a neurological disorder due to abnormal hyperactivity of the facial nerve. The most common cause of hemifacial spasm is a neuro-vascular conflict in the cerebellopontine angle between a vascular loop and the root of the facial nerve (96% of cases). Tumors are the cause of hemifacial spasm in only 1% of cases). The authors present their results in 100 patients who underwent microvascular decompression for essential hemifacial spasm between 1990 and 1995. They used microsurgical and endoscopic procedures by a minimal retrosigmoid approach in all cases. The most common offending vessels were the posterior inferior cerebellar artery (70%), the vertebral artery (41%) and the anterior inferior cerebellar artery (28%). An aberrant vein was found in 2 cases. There were 38% of multiple artery-nerve conflicts. Physiopathology of hemifacial spasm is explained by two principal theories: in the ephaptic theory, hyperactivity and an abnormal nervous impulse pathway are due to a short demyelinated area on the nerve trunk caused by the offending vessel, inducing short circuiting between adjacent nerve fibers. In the nuclear theory, hyperactivity of the facial nerve is due to an abnormal and automatic activity of the facial nerve nucleus itself, induced by the vessel. The authors used pre and postoperative electromyographic tests and intraoperative electromyographic tests. Their results tend to prove the nuclear theory. Ninety per cent of the patients had a good result, with a mean follow-up time of 30 months in 60 cases. In 82% of the cases, there was a total recovery after a single procedure. There was no mortality and no facial palsy. Hearing loss occurred in less than 5%.     

Antiphospholipid antibodies: an epiphenomenon in Tourette syndrome

Antiphospholipids (aPLAs) have been previously identified in children with Tourette syndrome (TS), which has led to the speculation that these antibodies might have a pathophysiologic role in this disorder. Therefore, 21 healthy children and adolescents with TS, whose ages ranged from 7 to 17 years, underwent laboratory studies designed to diagnose the lupus anticoagulant, anticardiolipin (aCL) antibodies [immunoglobulin (Ig) G, IgA, and IgM], and antinuclear antibodies. Although five subjects had at least one value that differed from accepted laboratory standards, the changes were marginal in four of them. Lupus anticoagulant was identified in one patient, based on a minimal requirement of a prolonged dilute Russell viper venom time, clotting studies that did not correct after mixture with normal plasma, and an abnormal platelet neutralization procedure. A prolonged (but correctable) activated partial thromboplastin time was found in one individual, and aCL IgG was marginally increased in three subjects. Two (10%) of a control population of 20 same-age children also had low positive aCL IgG levels. There were no differences in tics (onset, type, frequency, severity, and family history) or comorbid features between children with normal or "abnormal" laboratory study results. Our data suggest that the presence of aPLAs in TS represents an epiphenomenon rather than a pathophysiologic mechanism.     

Antiphospholipid antibody syndrome: an acquired cause of venous and arterial hypercoagulability

Antiphospholipid antibody syndrome is a major acquired cause of both venous and arterial hypercoagulability. Recent advances in the understanding of antiphospholipid antibodies, their detection, clinical presentations, and management are reviewed.     

Mitral valve prolapse syndrome. Nonpharmacologic management

As an alternative to drug therapy, patients with MVPS must be informed of their many options. As nurses, you can do an excellent job through education, through organization of support groups, and by developing written materials for these patients. In this field, a tremendous challenge with rewarding outcomes awaits you.     

Immunology in medical practice. II. Antiphospholipid antibodies in pregnancy

Antibodies against phospholipids are a risk factor for thrombotic disorders, but also for foetal death, pre-eclampsia, foetal distress and dysmaturity. This group of antibodies (aPLab) includes lupus anticoagulant (LAC) and anticardiolipin antibodies (aCL). These antibodies are encountered in patients with systemic lupus erythematosus (SLE), but also in patients with lupus-like disease and in women with (a history of) symptoms compatible with the antiphospholipid syndrome. Screening for a aPLab is advisable in these patients when they want to conceive and in women with recurrent foetal death after the 12th week of pregnancy. It is not clear if the antibodies exert a direct noxious action or are an accompanying phenomenon. Secondary prevention is possible with acetylsalicylic acid (80 mg/day), if desired in combination with subcutaneous heparin (5000-12,000 units twice daily). The thrombosis prophylaxis should be continued for 6 weeks after delivery.     

Antiphospholipid antibodies and atherosclerosis

The family of antiphospholipid antibodies includes antibodies binding to cardiolipin in serological test for syphilis, antibodies prolonging the clotting time in lupus anticoagulant test, antibodies reacting with plasma phospholipid-binding proteins, such as beta 2-glycoprotein I and prothrombin, and antibodies binding to oxidized low-density lipoprotein (LDL). Antiphospholipid antibodies are traditionally associated with arterial and venous thrombosis in patients with primary or secondary antiphospholipid syndrome. The recent studies, especially those on patients with myocardial infarction, extend the concept of antiphospholipid antibodies, and suggest that they play a role also in atherosclerosis. Based on the clinical studies and immunological findings, it seems that the differences in the specificity of antiphospholipid antibodies may reflect to their pathogenetic mechanisms and, finally, to their clinical consequences. The present review suggests that antibodies to oxidized LDL may not interfere directly with blood coagulation, but seem to have importance in the inflammation of the vessel wall in atherosclerosis and in vasculitis. Instead, antibodies to beta 2-glycoprotein I and to prothrombin show a closer association with thrombosis. It is possible that in the atherosclerotic plaque, the plasma proteins, such as beta 2-glycoprotein I or prothrombin, are bound to the endothelial surface and antibodies to cryptic epitopes revealed in these proteins are induced. These antibodies may contribute to the formation of atherosclerotic thrombosis by changing the balance of haemostasis toward hypercoagulative state.     

Antiphospholipid antibody syndrome in a case with redo coronary artery bypass grafting under cardiopulmonary bypass

Until now 'c-series' polysialogangliosides were known to exist in human brain only during development and in some pathological conditions like Alzheimer's disease. Using thin-layer chromatography (TLC) and immunostaining with Q211 antibody (TLC-overlay technique) we have analysed 'c-series' gangliosides in four human cerebella (age 20, 47, 52 and 54 years). Four distinct ganglioside bands, most probably corresponding to GT1c, GQ1c, GP1c and GH1c were found to exist in the analysed brains, which is convincing demonstration of the existence of 'c-series' gangliosides in normal adult human brain. Immunohistochemical analysis was performed to locate polysialogangliosides in the analysed tissue. Q211 antibody was found to bind specifically to a single subpopulation of neurons in the molecular layer of adult cerebellum. According to their position and morphology these cells correspond to stellate neurons.     

Antiphospholipid syndrome complicated by thrombosis of the superior mesenteric artery, co-existence of smooth muscle hyperplasia

A 37-year-old woman underwent an emergency operation at our hospital because of severe abdominal pain and ileus. Most of her small intestine and ascending colon were observed to have become necrotic due to occlusion of her superior mesenteric artery (SMA). Pathological findings of the resected intestine revealed that her SMA was completely thrombosed 2 cm distal from its origin with smooth muscle proliferation. Post-surgical blood analysis of her pre-operative serum was positive for lupus anticoagulant and antinuclear antibodies. She noticed vaginal bleeding due to missed abortion on the 31st day after the operation. We diagnosed her acute abdominal pain to be that of antiphospholipid syndrome associated with her pregnancy.