Persistant pre-eclampsia post partum with elevated liver enzymes and hemolytic uremic syndrome.

The spectrum of complications with pre-eclampsia, which may include AFLP (acute fatty liver of pregnancy) as well as the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), is resolved by early delivery. However, the ravages of HUS/TTP (hemolytic uremic syndrome/thrombotic thrombocytopenic purpura) require therapy usually by plasma exchange. Overlap between these two groups of syndromes has occurred on rare occasions and usually requires the therapy of the predominant or more dangerous or threatening form. Such overlap can be appreciated and then treated successfully without residual morbidity. The index case is presented and an extensive review of the two groups of syndromes is provided.

Predictable recovery from myasthenia gravis crisis with plasma exchange: thirty-six cases and review of current management.

Adult, acquired, idiopathic, autoimmune myasthenia gravis has a well-characterized IgG anti-acetylcholine striated-muscle receptor antibody. Removal by plasma exchange is effective, established therapy to augment anti-cholinesterase and immunosuppressive therapy and is the treatment of choice for myasthenia gravis crisis. We report 36 consecutive patients referred and accepted for plasma exchange, 32 of whom were in or entering myasthenia crisis, over a 10 year period. An average of 7.8 (range 1 to 16) plasma exchange procedures were done, with uniform, significant improvement, including extubation of 13 in myasthenic crisis and discharge from hospital in all. We conclude that this is the best treatment for myasthenia gravis crisis in hospital. From recent cases, most, if not all, crises can be prevented by IVIgG or plasma exchange as out-patients with use of corticosteroid and or azathioprine.

Characteristics of 73 patients, 1984-1993, treated by plasma exchange for Guillain-Barre syndrome.

Acute Guillain-Barre syndrome (GBS) is a demyelinating polyneuropathy which responds readily to plasma exchange (PEX). According to the North American Acute GBS PEX study there is a 50% or more reduction in the recovery time if PEX is initiated early in the course of the disease. Demyelinating antibodies are usually IgM. IgA antibodies require prolonged PEX. Patients with predominant IgG antibodies have chronic inflammatory demyelinating polyneuropathy (CIDP), which requires an even longer course of PEX, over weeks to months or years. We reviewed records of 73 patients with the initial diagnosis of GBS treated with PEX. Among these patients, 55 had classic GBS, three had the Miller-Fisher variant, two had CIDP, and 13 had demyelinating-like polyneuropathies associated with other conditions including malignancy, vaccine-related myelitis, steroid-induced myopathy, polymyositis, botulism, gram-negative sepsis, Sjogren's, and AIDS. Hughes grading system was used. Patients were graded 3 to 5, with grade 3 patients being unable to walk 5 m without support, grade 4 patients being bed or chair bound, and grade 5 patients being ventilator dependent. Of 60 unassociated (GBS) demyelinating cases receiving a mean of 6.5 PEX procedures, 13 (21%) were intubated early in the treatment, with four (6%) remaining ventilator dependent post-PEX. Of 51 non-intubated patients, 15 became ambulatory post-PEX. Patients with the Miller-Fisher variant showed improvement within 6 hours of PEX initiation. We did not investigate correlation of GBS with infection; however, we did observe a rise in CMV titer among 15% of the 58 patients with acute GBS. Considering our results we believe that intensive PEX on a daily basis for a few days is necessary for severely affected individuals. We advise five to nine procedures at consultation unless early, rapid recovery occurs.

Durable remissions following prolonged plasma exchange in thrombotic thrombocytopenic purpura.

We evaluated the efficacy of prolonged plasma exchange (PEX) for attaining durable remissions in thrombotic thrombocytopenic purpura (TTP). A recent review using steroids or PEX in initial management showed an 80% response rate but produced a relapse rate of 67-84%. Records of 50 patients starting PEX treatment for TTP/HUS were reviewed to identify and select those whose course of treatment had ended over 1 year earlier, whether or not the result was satisfactory. Records were evaluated for outcome, especially remission associated with treatment by "prolonged" plasma exchange. "Prolonged" was defined as continuing PEX beyond the stage where a normal platelet count was attained and until evidence of hemolysis was "minimal or at least compensated." If disease activity as judged by the criteria of hemolysis became accelerated or resumed, PEX was increased by volume of FFP (e.g., from 3 to 4 L) or rate (from less than daily to daily). Of 50 consecutive patients treated by PEX for TTP/HUS there were 40 cases after which at least one year had passed since the end of treatment. These 40 patients were evaluated for the results of treatment by PEX. Eight failed to achieve remission, dying in hospital within 1 month of admission. Twenty-eight achieved remission, sustained for 1 year or more in all. These are the reasons for our enthusiasm about this report. Four achieved remission lasting less than 1 year. Splenectomy was performed to obtain a sustained remission in one patient following administration of three 2 mg doses of vincristine and two relapses.(ABSTRACT TRUNCATED AT 250 WORDS)

Hemapheresis in pregnancy.

Plasma exchange in pregnancy has benefited those diseases that occur and are treated in the nonpregnant patient. In addition, special emphasis was given to diseases unique to pregnancy, such as hemolytic disease of the newborn, which is significantly modified by plasma exchange therapy. Fluid volumes, replacement solution, and techniques were discussed.

An anti-i biphasic hemolysin in chronic paroxysmal cold hemoglobinuria.

Paroxysmal cold hemoglobinuria (PCH) was diagnosed in an elderly patient with a history of chronic lymphocytic leukemia. In this case, the Donath-Landsteiner antibody demonstrated anti-i specificity rather than the more commonly reported anti-P specificity. This unusual case of a biphasic hemolysin with anti-i specificity emphasizes the importance of performing a Donath-Landsteiner test when presented with serological or clinical findings that are suggestive of PCH.

Dihydroxyacetone, pyruvate, and phosphate effects on 2,3 DPG and ATP in citrate-phosphate-dextrose-adenine blood preservation.

Blood was stored with various combinations of 60 mM dihydroxyacetone (DHA), 80 mM pyruvate and 10 mM phosphate in citrate-phosphate-dextrose-adenine-one (CPDA-1) preservative for 42 days. During that time, DHA was found to have a beneficial effect on 2,3 diphosphoglycerate (2,3 DPG), although a deleterious effect on adenosine triphosphate (ATP). Preservatives containing pyruvate gave similar results. However, DHA and phosphate together in CPDA-1 improved both 2,3 DPG and ATP. The adverse effect on 2,3 DPG levels that phosphate normally shows was reversed by the addition of DHA. These results indicate that the addition of phosphate alone, rather than pyruvate, or pyruvate and phosphate, to DHA in CPDA-1 would be preferable to the presently licensed CPDA-1 preservative.

Efficacy of hetastarch in the resuscitation of patients with multisystem trauma and shock.

A prospective trial of 6% hetastarch (HES) v 5% plasma protein fraction (PPF) as the colloid component of intravenous (IV) fluid resuscitation was conducted in 32 patients with multisystem trauma and/or hemorrhagic shock. Patient age, mechanism and pattern of injury, and IV fluid requirements were similar in both groups. No intergroup differences were noted in indexes of hepatic, pulmonary, or renal function or in the incidence of infection. The frequency of other complications, including bleeding diatheses, and mortality were identical in the two groups. Although this investigation should be viewed as a pilot study, our results suggest that, compared with PPF, HES in large volumes is a safe, effective colloid solution in the resuscitation of patients with multisystem trauma and/or hemorrhagic shock. Further study of HES in a larger number of patients is warranted by these findings.

Preservation of red blood cells for transfusion.

Development of red blood cell preservative solutions began in the early 1900s. During the 1930s, definition of the glycolytic pathway of red blood cell metabolism provided a rational basis for these studies and contributed to the establishment of standard criteria for satisfactory red blood cell preservation. Recently, the use of adenine in the preservative solution has allowed a 14-day increase in red blood cell storage time. Additional approaches to red blood cell preservation are the use of optional additive solutions and rejuvenation. One major controversial issue is the clinical significance of transfusion of stored red blood cells that are depleted of 2,3-diphosphoglycerate and have a high oxygen affinity.

Laboratory evidence of unsuspected parental consanguinity among cases of disputed paternity.

A search was conducted to find evidence of possible incestuous unions between the biologic parents of children involved in 2500 paternity cases. Suspicion was raised when either (1) a mother and her child possessed identical HLA phenotypes, or (2) the child appeared to be possibly homozygous for one maternal haplotype (i.e., one of the child's HLA haplotypes was a blank). These mother-child HLA-haplotype dualisms (MHDs) occurred in 5% of all cases. Frequency of exclusion of the accused men in cases demonstrating MHD, was compared with the remaining paternity cases. No significant difference was found in overall exclusion rates between MHD cases and controls when exclusion produced by HLA and red cell antigen systems were observed. However, there was a greater rate of exclusion in MHD cases when comparing exclusions produced by red cell antigen systems regardless of whether HLA tests excluded paternity (p less than 0.025). MHD cases involving teenaged mothers differed from control cases in frequency of exclusion of paternity only on the basis of red cell antigen phenotyping (p less than 0.005). The HLA system's usefulness in paternity testing is diminished when there is MHD; multiple, independently-inherited systems are relatively more useful in these circumstances. The search method detects only half of potential incest cases; proof of incest requires more extensive testing for homozygosity among other polymorphisms. Since calculations of likelihood of paternity are inappropriate in cases involving close consanguinity, detection and follow up studies are important. Data suggest that one-fifth of MHD cases may involve first degree consanguinity and that the incest rate among paternity cases may be as high as 2%.

Utility of HLA and six erythrocyte antigen systems in excluding paternity among 500 disputed cases.

Six erythrocyte antigen systems and the HLA system were evaluated to establish their practical value in 500 cases of disputed paternity. The actual results were very close to predicted values. HLA testing is expected to detect 92% and red cell testing is expected to detect 67% of men falsely accused in paternity suits. The findings of this study show that HLA detected 94% and red cell testing detected 69% of 107 men falsely accused in 500 paternity cases. In order of sensitivity, Rh, MNSs, and ABO were the most useful erythrocyte marker systems. There were six out of 107 cases in which exclusions would have been undetected if red cell typing had not been performed. Five of the six cases involved "common" HLA haplotypes.

Blood preservation 42: improvement of ascorbate's ability to maintain 2,3-DPG with inosine.

Inosine and ascorbate have been shown to maintain normal 2,3-DPG levels during three to four weeks of blood storage. With the introduction of CPD-adenine, which allows five weeks of storage, the desire for 2,3-DPG maintenance may receive new emphasis. Red blood cell 2,3-DPG remained at normal or higher levels for six weeks whenever 10 or 15 mM inosine and 10 mM vitamin C (L-ascorbate) or D-ascorbate were present in the CPD-adenine preservative. Provision by inosine of a five-carbon sugar for 2,3-DPG synthesis, bypassing the rate-limiting phosphofructokinase reaction, may allow NADH oxidation by ascorbate to provide an increased supply of substrate for the Rappoport-Luebering shunt, thus affecting the net increase and maintenance of 2,3-DPG.

Blood preservation 33. Phosphate enhancement of ribose maintenance of 2,3-DPG and ATP.

Blood storage in CPD-adenine supplemented with 25 mM inosine and 10 mM phosphate gave 2,3-DPG levels as high as 140 per cent of normal for six weeks of blood storage at 4 C. Lower but normal 2,3-DPG levels were maintained throughout six weeks with inosine or inosine plus ribose. Ribose alone provided marginally increased DPG maintenance over the control, but ribose with phosphate maintained 2,3-DPG levels above 70 per cent of normal for five weeks of storage and two weeks longer than the control preservative. ATP levels were maintained at normal or above for six weeks with phosphate plus ribose or inosine. 2,3-DPG maintenance has previously been shown to be impaired by phosphate, unless inosine is also present. The ribose and inosine effects on 2,3-DPG maintenance are not additive. Phosphate also has an enhancement effect on ATP maintenance in the presence of either ribose or inosine.

Blood preservation 35. Red cell 2,3-DPG and ATP maintained by DHA-ascorbate-phosphate.

DHA (dihydroxyacetone, 60 mM) with ascorbic acid (d-ascorbate, 10 mM) kept 2,3-DPG concentrations above normal for six weeks. Levels of 2,3-DPG were below normal after four weeks with DHA alone and after two weeks with DHA-ascorbate-phosphate. As in previous studies, high phosphate concentrations decreased 2,3-DPG maintenance. ATP maintenance was best achieved with the following (in order of performance): DHA-phosphate (20 mM); DHA-phosphate (10 mM); the control, CPD-adenine preservative; Phosphate 20 mM; and DHA. DHA with ascorbate provides normal 2,3-DPG for six weeks. The adverse effects of DHA and DHA with ascorbate on ATP levels are modified by 10 mM phosphate.

Acute renal failure associated with the thrombocytopenia of septicemia.

Gram-negative sepsis is a recognized cause of thrombocytopenia and has recently been associated with acute renal failure from acute glomerulonephritis. We report here a patient in which the development of and treatment for trauma, gram-negative sepsis, was followed one day later by rapidly rising WBC (20,000-65,000), creatinine, BUN, and thrombocytopenia (300,000-25,000). Peak creatinine (6.5 mg%) and BUN (160 mg%) levels occurred on the third day of septicemia, when dialysis was begun. During the profound thrombocytopenia (less than 20,000 on days three through five of the septicemia) platelet transfusions did not raise the platelet count. The platelets returned to above 100,000 by day seven of the septicemia and the WBC was under 30,000 by this time. We propose that septicemia caused by E. coli (and staph) was responsible for the concomitant appearance of both thrombocytopenia and acute renal failure in a 20-year-old man.