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Biomedical subjects

H J Zeitz

Publications and source records attributed to H J Zeitz.

31 records · Page 2Linked to original sources

Fatal panniculitis.

The Weber-Christian syndrome (relapsing nodular panniculitis) displays a clinical spectrum varying from short, self-limited, or intermittent disease episodes to persistent disease with fatal outcome. Inflamed adipose tissue is exclusively subcutaneous in some patients and is both subcutaneous and perivisceral in others. Inflammation of fat may induce a focal cutaneous or a systemic extracutaneous histiocytic proliferative response in which hemophagocytosis may be a frequent characteristic. Major causes of death--sepsis, hepatic failure, hemorrhage, and thrombosis--are identical in the patients with and without the systemic histiocytic proliferation. Inflammation in fat, of and by itself, may be associated with significant morbidity and mortality, regardless of specific histopathology or inciting factors.

Adolescent↗

Panniculitis associated with cutaneous T-cell lymphoma and cytophagocytic histiocytosis.

A 36-year-old woman had a 6-year history of recurrent panniculitis with development of an angiocentric and angiodestructive cutaneous T-cell lymphoma (CTCL) of the helper cell phenotype. She subsequently developed a rapidly progressive fatal syndrome characterized by cytophagocytic histiocytosis and hyperlipidaemia. Cytophagocytic histiocytosis has previously been reported in association with panniculitis, malignancy and infection, but not with CTCL and the precise relationship between panniculitis, CTCL, cytophagocytic histiocytosis and hyperlipidemia is unclear.

Adult↗

Decreased C5b67-inhibitor activity in two families with hereditary functional deficiency of the eighth component of complement.

In addition to its role in hemolysis and host defense against Neisseria infection, the eighth component of human complement (C8) is one of several plasma proteins that are C5b67-inhibitors (C5b67-INH). The recent identification in our laboratory of two new families with hereditary deficiency of C8 provided an opportunity to study further the role of C8 as a C5b67-INH. Based on mixing and reconstitution experiments, the deficiency of C8 seemed to be due to a selective lack of the C8 beta-chain in one family and the C8 alpha-gamma subunit in the other family. Sera from individuals homozygous for the C8 abnormality were substantially deficient in C5b67-INH activity as well as totally deficient in hemolytic activity. Sera from control individuals possessed approximately 2500 C5b67-INH U/ml, whereas sera from the C8-deficient individuals had markedly depressed C5b67-INH activity, with a mean of only 428 U/ml. C5b67-INH activity was completely reconstituted in C8-deficient serum by the addition of purified human C8. We conclude that C8 constitutes the substantial majority of the C5b67-INH activity of normal human serum.

Adolescent↗

Frequency of anaphylactoid reactions during intravenous urography with radiographic contrast media at two different temperatures.

One hundred randomly selected patients undergoing routine excretory urography were assigned in double-blind fashion by alternate entry pairs to receive radiographic contrast media at 37 degrees C (Group I) or room temperature (Group II). There were three anaphylactoid reactions and five nonanaphylactoid reactions in Group I, and five anaphylactoid and eight nonanaphylactoid reactions in Group II, differences that were not statistically significant. No differences were detected between reactors and nonreactors. Thus, in this study, infusion temperature had no effect on the frequency of adverse reactions to radiographic contrast media during excretory urography.

Adolescent↗

Renal transplantation in a patient with hereditary deficiency of the second component of complement.

The HLA haplotype A 10,B18 has been associated with hereditary deficiency of the second component of complement(C2). In an effort to detect individuals homozygous for C2 deficiency, a thorough audit of HLA serotyping results in 3,100 individuals was performed, and a single patient homozygous for the A10, B18 haplotype was identified. Detailed complement studies in this patient's serum and plasma revealed previously undetected selective absence of C2 antigen and haemolytic activity, and a hereditary basis for this deficiency was indicated by half-normal levels of C2 haemolytic activity in both of his children. The patient was of special interest in that he had previously developed renal failure which was treated by cadaver kidney transplantation. C2 antigen was undetectable in serum and plasma samples taken prior to and up to 9 months following transplantation. This experience suggests that HLA serotyping can be a valuable screening technique for the detection of individuals with C2 deficiency, and that renal transplantation does not reconstitute normal levels of C2.

Adult↗

Inherited deficiency of the ninth component of complement in man.

A 76-year-old man was found to have low (33% normal) serum complement (C) hemolytic activity, although C3 and C4 protein levels were normal. Further evaluation of his serum and plasma indicated that all C components were present in normal or elevated amounts except for C9, which was undetectable by both antigenic and functional assays. Addition of purified human C9 led to full restoration of the hemolytic activity. Family studies demonstrated that the deficiency was inherited as an autosomal codominant trait and was not linked with alleles at the HLA-A or HLA-B loci. The patient had no history of recurrent or unusual infections and no evidence of autoimmune disease. The availability of serum totally lacking in C9 permitted an investigation of the lytic capacity of the C5b-8 segment of the C attack mechanism, which was pursued in kinetic studies on the hemolysis of erythrocyte intermediates. These studies indicated that hemolysis occurred approximately 100 times slower in patient than in normal serum, using either EA or EAC1-7 intermediates as target cells. Serum bactericidal activity also was slower in patient serum, occurring at a rate about 1/35 that observed in normal serum. These studies provide direct independent evidence that cytolysis of erythrocytes and bacteria can be mediated by C5b-8, and allow a quantitative estimation of the increment in the rates of these reactions provided by normal serum levels of C9. The presence of readily detectable though slow hemolytic activity of C9-deficient serum may account for the difficulty in identifying individuals with this defect.

Aged↗

Inherited deficiency of second component of complement and HLA haplotype A10,B18 associated with inflammatory bowel disease.

A patient with inflammatory bowel disease and sacroiliitis had haplotypes A10,B18 and Aw32,b18 at the major histocompatibility locus. Serum total complement and C2 hemolytic complement activities were undetectable; levels of the remaining C1-C9 components were normal. The parents, both siblings, and a child each had half-normal levels of C2 and either the A10,B18 or the Aw32,b18 hla haplotype. In a second unrelated family, an only child and both parents developed inflammatory bowel disease. The father and child had HLA haplotype A10,B18, but, along with the mother, each had normal serum levels of hemolytic C and C2. Homozygous C2 deficiency, often in association with the A10,B18 haplotype, has previously been linked with various autoimmune diseases and with propensity to infection. Our findings suggest that C2 deficiency or this haplotype also may predispose to inflammatory diseases of the intestine.

Adult↗

Efficacy and safety of nedocromil sodium 2% ophthalmic solution b.i.d. in the treatment of ragweed seasonal allergic conjunctivitis.

The efficacy and safety of twice-daily nedocromil sodium 2% ophthalmic solution and vehicle were compared in the treatment of ragweed seasonal allergic conjunctivitis. Two separate multicenter, randomized, double-masked, placebo-controlled studies were subjected to a combined analysis. Following a one-week baseline period during the beginning of the ragweed pollen season, 189 patients with seasonal allergic conjunctivitis received either nedocromil sodium or vehicle b.i.d. for eight weeks. Efficacy was evaluated by patient diary cards and clinical eye examinations. Safety was assessed by reports of adverse events. Compared with vehicle, nedocromil sodium produced significantly greater decreases in summary symptom score (p = 0.005), itch (p = 0.005), tearing (p = 0.004), overall eye condition (p = 0.001), and clinician-evaluated conjunctival edema (p = 0.018), and significantly better (p = 0.001), and patient (p = 0.001) opinions of treatment effectiveness at the peak pollen period. Additionally, the superiority of nedocromil sodium compared to vehicle approached statistical significance in redness reduction (p = 0.087) and clinician-evaluated conjunctival injection (p = 0.087). There were no serious treatment-related adverse events in either treatment group. In summary, nedocromil sodium 2% ophthalmic solution b.i.d. was found to be effective and to have a favorable safety profile in the treatment of seasonal allergic conjunctivitis.

Adolescent↗

Problem based learning: development of a new strategy for effective continuing medical education.

The major format for Continuing Medical Education (CME) in the United States is the large group lecture. To test the hypothesis that the problem based learning (PBL) model for medical student education could be successfully adapted for use in CME with practicing primary care physicians (PCPs), PBL adaptations were developed for experiments with both small (5-10 participants) and large (10-40 participants) groups of learners. Approximately 90% of participants in PBL for CME meetings enjoyed and enthusiastically accepted these education models. Physicians who participated in these programs developed the motivation to change their clinical practice behavior; the frequency of this motivation (30%, 65%, and 85% of participants) was directly linked to the length of the program (1, 2, or 3 hours, respectively). This end-of-program motivation to change practice behavior transferred into actual change as measured both by self-report and by direct measurement of prescription writing. In a completely separate experiment, PBL-based CME programs were provided to PCPs participating in a pediatric asthma disease state management project in a Midwestern health maintenance organization; in the very first year of implementation, the project resulted in a significant decrease (P < 0.03) in utilization of urgent care, with a concomitant 75% decrease in the average cost of urgent care services. Finally, 300 CME educators learned how to teach using the PBL for CME format; outcomes such as acceptance of the model, and end-of-program motivation to change practice behavior in programs given by these teachers (beta results) matched those observed in the initial experiments (alpha results). Taken together, these results suggest that for CME, the large group lecture format can be replaced by the small and large group PBL formats.

Allergy and Immunology↗