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C Mueller-Eckhardt

Publications and source records attributed to C Mueller-Eckhardt.

At least 235 records · Page 13Linked to original sources

Treatment of idiopathic thrombocytic purpura in pregnancy by high-dose intravenous immunoglobulin.

ITP in pregnancy may lead to fetal thrombocytopenia caused by the transplacental passage of maternal antiplatelet antibody. The most hazardous complication in the infant is intracranial hemorrhage. In addition ITP in pregnancy is reported to be associated with an increased abortion rate and an elevated fetal morbidity and mortality. Therefore obstetric management must aim at increasing maternal and fetal platelets. Several therapeutic approaches to the treatment of ITP in pregnancy are evaluated. Two cases of ITP in pregnancy are reported. Administration of high-dose intravenous immunoglobulin is introduced as a new therapy for ITP in pregnancy. The rapid reversal of thrombocytopenia following immunoglobulin G administration suggests that it is useful especially as emergency treatment for ITP in pregnancy.

Adult↗

Improved monocyte cytotoxicity test.

A micro-modification of the two-colour monocyte cytotoxicity test (MCTT) is described. It employs exclusively adherent monocytes for staining, sensitizing and labeling instead of monocyte suspensions. Thereby unspecific cytotoxicity is decreased while sensitivity and specificity of the test are improved. As a micro-technique, this modification of the MCTT is useful for antibody screening as well as antigen definition.

Cell Adhesion↗

Deposition of the terminal C5b-9 complement complex on erythrocytes by human red cell autoantibodies.

A radioassay for the detection of complement activating autoantibodies (autohaemolysins) in patients with autoimmune haemolytic anaemia (AIHA) is described. The method is based on immunoradiometric quantitation of the cytolytic C5b-9 complement complex following its antibody-dependent deposition on red blood cells (RBC). The use of affinity-purified, radiolabelled antibodies directed against the neoantigens of the C5b-9 complex ensured specificity of the test which proved more sensitive than conventional haemolytic assays and was not subject to disturbances by haemolytic sera. The results obtained in 70 patients with various forms of AIHA (warm type N = 45); cold type (N = 22); Donath-Landsteiner type (N = 3] support the prevailing assumption that autohaemolysins can trigger the complement cascade to completion. Since intact RBC from patients with detectable autohaemolysins carried C3/C4 components but never C5b-9, it is inferred that regulatory mechanisms operate in vivo at the level of C4b/C3b inactivation to arrest the cascade and rescue the autologous cells.

Adolescent↗

Platelet-associated complement C3 in thrombocytopenic states.

Platelet-associated complement components C3, C3d and C4 (PAC3, PAC3d, PAC4) were quantitated by a modification of the platelet radioactive anti-IgG test using highly purified C3 antibodies in 74 patients with immune thrombocytopenia (ITP), 26 patients with presumed nonimmune thrombocytopenia (NTP), and 114 normal individuals. Elevated PAC3 levels were found in 26 out of 74 patients with ITP (35%) and in 10 out of 26 patients with NTP. Although the percentage of elevated PAC3 values was higher in thrombocytopenic patients than in nonthrombocytopenic patients, no statistically significant correlation existed between platelet counts and PAC3 levels, neither for ITP nor for NTP. However, such a relationship was demonstrable between PAC3 and platelet-associated IgG, both for ITP (P less than 0.05) as well as NTP patients (P less than 0.001). We conclude that elevated PAC3 values are not restricted to immune thrombocytopenias. Quantitative differences of PAC3 between ITP and NTP patients suggest that part of the PAC3 is immunologically mediated and has a role in the pathogenesis of autoimmune thrombocytopenias.

Adult↗

New evidence for the existence of HLA-B14 subtypes. Linkage with different DR antigens.

In a joint study 109 unrelated HLA-B14 positive individuals were subtyped for the provisional splits 14.1 and 14.2 respectively, based on sera of the 7th and 8th Histocompatibility Workshops. Sixty-eight of these donors as well as 23 families were also typed for DR antigens. Earlier findings concerning a linkage disequilibrium B14.1/Aw32 and B14.2/Aw33 were confirmed. In addition a highly significant association B14.1/DR7 as well as a less striking one for B14.2/DR1 were observed. These findings contribute new arguments for the partition of the B14 antigen.

Genes, MHC Class II↗

The value of platelet-associated IgG in predicting the efficacy of splenectomy in autoimmune thrombocytopenia.

In a prospective study, the hypothesis of whether the quantitative determination of platelet-associated IgG (PAIgG) in patients with chronic autoimmune thrombocytopenic purpura (ATP) can predict the efficacy of splenectomy, was investigated. PAIgG levels were repeatedly determined in 16 patients with definite ATP pre- and postsplenectomy, and related to platelet counts and platelet mean life span. It was found that patients with an immediate remission after splenectomy tended to have lower PAIgG levels (less than 6%) than failures, but this difference was not statistically significant. We conclude that PAIgG is of limited value for the prediction of the efficacy of splenectomy in ATP.

Adolescent↗

[Immunothrombocytopenia of the newborn caused by platelet-specific antibodies (anti-P1A1) (author's transl)].

For the first time in the German speaking area 8 cases of neonatal immunothrombocytopenia caused by platelet-specific P1A1 antibodies could be ascertained. The disease is caused by fetomaternal incompatibility against platelet antigens. All mothers were healthy. The children showed a postnatal tendency for petechial haemorrhages. Cerebral haemorrhages occurred in two cases. Optimal treatment consists of administration of P1A1-negative platelets (perhaps of the mother). Demonstration of platelet antibodies is possible nowadays by adequate serological methods.

Adult↗

Apolipoprotein AIMarburg: studies on two kindreds with a mutant of human apolipoprotein AI.

Three probands heterozygous for a mutant of apolipoprotein AI (apo AIMarburg, Utermann et al. 1982a) were detected by screening of 2282 unrelated individuals resulting an a frequency estimate of about 1/750 in the German population. All three probands with apo AIMarburg had hypertriglyceridemia (triglyceride above 250 mg/dl) and subnormal HDL-cholesterol (below 30 mg/dl), but no other lipoprotein abnormalities. The kindreds of two probands with AIMarburg were studied. The family data are consistent with an autosomal codominant inheritance of the trait. A total of 16 heterozygous blood relatives with the mutant AIMarburg were detected in these kindreds. Analysis of the plasma lipid and lipoprotein levels in relation to the apo AI phenotype was complicated by the high prevalence of diabetes mellitus and thyroid disease in one kindred and of hyperlipidemia in both kindreds. No consistent relationship between plasma lipid and lipoprotein levels, and the mutant apo AI could be demonstrated. Instead the mutant apo AI and the dyslipoproteinemia seem to co-exist independently in these kindreds. Three sibs with the homozygous apo E-2/2 phenotype were detected in one kindred, and all three sibs had subnormal LDL-cholesterol and beta-VLDL, e.g., the lipoprotein abnormality characterizing primary dysbetalipoproteinemia. Genetic apo E phenotypes and the apo AI mutant segregated independently, indicating that the structural gene loci for apo E and apo AI are not closely linked.

Adult↗

Immunogenetic studies of the platelet-specific antigen P1A1 (Zw(a)).

The phenotype frequency of the platelet-specific antigens P1A1/A2 ( = Zw(a,b)) in the German population (N = 711) is 97.75% and 2.25% respectively. The corresponding gene frequencies are P1A1 0.85; P1A2 0.15. The P1A1 determinant is codominantly inherited (19 families; 151 family members). There is a strong gene dosage effect between P1A1 homo and heterozygous individuals. No definite linkage of the locus P1A1 versus the loci HLA-ABC, ABO, or Rh was found.

Antilymphocyte Serum↗

Platelet associated IgG, platelet survival, and platelet sequestration in thrombocytopenic states.

Platelet-associated IgG (PAIgG), platelet mean life span (MLS), and platelet sequestration sites were studied in 69 patients with immune (ITP) and presumed nonimmune thrombocytopenias (NTP). A shortened MLS was associated with elevated PAIgG (N = 46), and with normal PAIgG (N = 15). Four patients had a normal MLS, but elevated PAIgG, four patients were normal for both parameters. The highest PAIgG values occurred in ITP patients with a very short MLS. Nine NTP patients had also elevated PAIgG, but a normal or slightly shortened MLS. There was a significant double log correlation between PAIgG and MLS for ITP, but not for NTP patients. Judged from the coefficient of determination, only 10% of PAIgG were directly related to a shortened MLS. 70% of patients (N = 63) had exclusively splenic and 30% hepatosplenic sequestration. PAIgG was elevated in 29/44 patients with splenic (66%) and in 16/19 patients with hepatosplenic sequestration (84%). In ITP, PAIgG-positive cases were observed in 69% of splenic v 82% of hepatosplenic sequestration, while in NTP the corresponding figures were 6/11 and 2/2. No significant correlation between PAIgG and either sequestration type was demonstrable. We conclude that in immunologically mediated thrombocytopenia only a small portion of PAIgG accounts for a decreased MLS, and that the concentration of PAIgG per se does not determine the platelet sequestration type.

Adolescent↗

HLA-B7 on human red blood cells. Improved detection by a radioactive anti-IgG test.

By means of a sensitive radioimmune anti-IgG test (RIAT) HLA-B7 was demonstrated on RBC of all B7 positive individuals (N = 52). Most antigens of the B7 cross-reacting group (B8, 13, w22, 27, 40; N = 36) were also detected. Eluates from B7 positive RBC exhibited clear B7 specificity against RBC as well as lymphocytes.

Blood Platelets↗

Improved assay for detection of platelet-specific PlA1 antibodies in neonatal alloimmune thrombocytopenia.

A technique is described which permits the detection of platelet-specific, noncomplement-fixing ("blocking') PlA1 antibodies in serologically negative sera of mothers from children suffering from neonatal alloimmune thrombocytopenia. The technique consists of (1) antibody enrichment in eluates prepared from maternal serum and PlA1-positive platelets, and (2) quantitation of antibodies in eluates by the platelet radioactive anti-IgG test. With this assay, PlA1 antibodies were demonstrated in 6 out of 7 maternal sera, and anti-A antibodies in one. It proved valuable also for longitudinal studies for periods up to 30 months after delivery.

ABO Blood-Group System↗

[Indications and clinical aspects of granulocyte transfusions (author's transl)].

Granulocyte transfusions (n = 222) were administered to 41 patients with granulocytopenia (less than 500 X 10(6)/1) and antibiotics-resistant infections. The average granulocyte content per transfusion was 2.1 X 10(10), 212 transfusions had been obtained by filter leucapheresis. Infections improved in 25 episodes, and 18 patients survived the 30th day. There was no influence in 18 episodes and only 2 patients survived beyond the 30th day. Extent and duration of bone marrow insufficiency, and type and grade of infection, clearly influenced prognosis. Further prognostically relevant risk factors were age (greater than 60 years) and platelet deficiency (less than 20 X 10(9)/1), as well as liver and renal insufficiency. Adherence to precise guidelines may prevent pointless transfusions.

Adolescent↗

Immunological studies in a case of Evans' syndrome.

Immunological studies in a case of Evans' syndrome (autoimmune hemolytic anemia of the "warm type" associated with idiopathic thrombocytopenic purpura) are presented in whom the autoimmune nature of both conditions was verified by the demonstration of cell-bound and free serum autoantibodies against red blood cells as well as platelets by a radioimmune technique.

Aged↗