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DIC is excluded by normal coagulation results Other causes of microangiopathic hemolytic anemia (Table 13 18) should be entertained Occasionally, vasculitis or acute glomerulonephritis is considered, and in these cases renal biopsy may be necessary to establish the diagnosis if the platelet count will allow it HUS is arbitrarily distinguished from TTP by the consistent presence of renal failure and the lack of neurologic findings

Microangiopathic hemolytic anemia Thrombocytopenia and renal failure Elevated serum LDH Normal coagulation tests Absence of neurologic abnormalities



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In children, HUS is almost always self-limited and requires only conservative management of acute renal failure In adults, however, without treatment, there is a high rate of permanent renal insufficiency and death The treatment of choice (as in TTP) is large-volume plasmapheresis with fresh-frozen replacement (exchange of up to 80 mL/kg), repeated daily until remission is achieved





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Hemolytic-uremic syndrome (HUS) is an uncommon disorder consisting of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure due to microangiopathy (with decreased glomerular filtration, proteinuria, and hematuria) The cause is unclear The disease is similar to TTP except that different vascular beds are involved In fact, the two diseases are probably best considered as part of a spectrum of HUS TTP disorders The pathogenesis of the two disorders is probably similar, and a platelet-agglutinating factor found in plasma may be involved In children, HUS frequently occurs after a diarrheal illness secondary to infections with Shigella, Salmonella, E coli strain O157:H7, or viral agents The mortality rate of this form is low (< 5%) In adults, this syndrome is often precipitated by estrogen use or by the postpartum state HUS may be seen as a delayed complication of high-dose corticosteroid therapy and autologous bone marrow or stem cell transplantation, or of the use of cyclosporine or tacrolimus as immunosuppression in allogeneic transplantation A familial (hereditary) type has been identified in which members of a family have recurrent episodes over several years

Figure 14-33

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The prognosis of HUS in adults remains unclear Without effective therapy, up to 40% of patients have died, and 80% have had chronic renal insufficiency Early institution of aggressive therapy with plasmapheresis promises to be beneficial Survival and correction of hematologic abnormalities are the rule, but restoration of renal function requires that treatment be initiated early

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Garg AX et al Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression JAMA 2003 Sep 10;290(10):1360 70 [PMID: 12966129] Vesely SK et al ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients Blood 2003 Jul 1;102(1):60 8 [PMID: 12637323]

Patients present with anemia, bleeding, or renal failure The renal failure may or may not be oliguric In contrast to TTP, there are no neurologic manifestations other than those due to the uremic state

As in TTP, there is microangiopathic hemolytic anemia and thrombocytopenia, but the thrombocytopenia is often less severe The peripheral blood smear should show striking red blood cell fragmentation, and the diagnosis is

Prolonged bleeding time, either at baseline or after challenge with aspirin Reduced levels of factor VIII antigen or ristocetin cofactor Reduced levels of factor VIII coagulant activity in some patients

von Willebrand s disease is rarely as severe as hemophilia, and spontaneous hemarthroses do not occur (except in the rare type III) The bleeding tendency is exacerbated by aspirin Characteristically, bleeding decreases during pregnancy or estrogen use

Each end of the T1 line goes into a router (After going through a CSU/DSU, of course!) Note that this connection does not use the Internet in any way it is not a VPN connection Dedicated connections of this type are expensive and are only used by organizations that need the high bandwidth and high security these connections provide These connections are invisible to the individual computers on each network There is no special remote connection configuration of the individual systems, although there may be some configuration of DHCP, DNS, and WINS servers to insure that the network runs optimally

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How to Use JavaScript to Print a PDF | Techwalla.com
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