Creutzfeldt--Jakob Disease in Recipients of Human Growth Hormone in the United Kingdom: A Clinical and Radiographic Study

In the past 3 years there have been five further cases, in additionto one case reported in 1985, of Creutzfeldt-Jakob disease inrecipients of human growth hormone in the United Kingdom. Theclinical findings of two of these cases are described, demonstratinga typical presentation with a predominantly cerebellar syndromeat onset which is not commonly a presenting feature of sporadicCreutzfeldt-Jakob disease. In one case a ^99mTc hexamethylpropylenaminesingle photon emission tomographic scan showed marked impairmentof tracer uptake in the basal ganglia and cerebral cortex ata time when the clinical picture was predominantly cerebellar.This technique may be useful in early diagnosis. In the othercase post mortem examination of the brain showed prominent amyloiddeposition in the cerebellum, which has not been described previouslyin pituitary-hormone related Creutzfeldt-Jakob disease. Thepreviously published cases of growth hormone-related Creutzfeldt-Jakobdisease are reviewed and reasons for the particular clinicalpattern seen are discussed.     

JMH variants: serologic, clinical, and biochemical analyses in two cases

BACKGROUND: JMH is a high-frequency red cell blood group antigen that resides on a 76- to 80-kDa glycosylphosphatidylinositol-linked protein also known as CDw108. Antibodies with JMH specificity are often autoimmune and are usually, if not always, clinically benign. Some individuals with JMH-variant antigen produce alloantibodies to JMH, but little evidence concerning their clinical significance is available. This article reports on two patients who express a JMH-variant antigen and produced alloanti-JMH. STUDY DESIGN AND METHODS: Murine monoclonal antibodies and human antibodies to JMH were used in hemagglutination, radioimmunoassay, and Western blot testing of red cells from two JMH- variant patients; antiserum from one of these patients was also used in biochemical studies. In addition, in vivo survival of JMH-positive red cells was studied in the same patient. RESULTS: Biochemically, both examples of red cells with the JMH-variant phenotype expressed a JMH protein with a molecular weight similar to that of the normal JMH protein. For both patients, family studies suggested an autosomal recessive pattern of inheritance. Survival study demonstrated reduced in vivo red cell survival in one patient. CONCLUSION: JMH-variant phenotypes express a protein of normal molecular weight and are inherited in an autosomal recessive pattern. Furthermore, individuals with this phenotype can produce clinically significant antibodies.     

Detection of Ovarian Tumors in Chicken by Sonography

OBJECTIVE: Animal models of spontaneous ovarian cancer are important for understanding early tumor development. Ovarian imaging may play an important role in following changes in tumor development. Laying hens are the only animals that develop spontaneous ovarian cancer similar to humans. The aim of this study was to determine the feasibility of detecting ovarian tumors in laying hens using sonography. METHODS: Ovaries of commercial strains of White Leghorn laying hens (n = 29, 2.5-3.0 years old) were examined by transvaginal 2-dimensional gray scale and color Doppler sonography. Sonographic evaluations were compared with ovarian anatomy and histologic features. RESULTS: Results of in vivo sonography and ovarian anatomic and histologic examinations were consistent. The presence of gross ovarian tumors was correctly detected in all hens by sonography. The resistive and pulsatility index values associated with ovarian tumors were lower than for normal ovaries (P < .001) suggesting that blood flow velocity was increased in ovarian tumors. Values associated with abnormal ovarian histologic findings but no gross tumors were intermediate. CONCLUSIONS: Transvaginal sonography can be used to determine ovarian status in hens. It offers the ability to make repeated examinations on the same hen to monitor early changes in the ovary associated with ovarian cancer.     

Electronmicroscopic examination of white cell reduction by four white cell-reduction filters

The mechanisms of white cell (WBC) reduction in 16-hour-old CPDA-1 red cell (RBC) concentrates by filtration on a column filter and on three different flatbed filters were studied by electron microscopy, with special emphasis on cell-to-cell interaction, cell damage, and interaction of blood cells with the material. Generally, lymphocytes were removed by mechanical sieving and monocytes by adherence and mechanical sieving. Granulocyte depletion occurred by mechanical sieving, direct adhesion to the fibers, and indirect adhesion to activated and spread platelets. In the column filter, most granulocytes were captured by adhesion. In the coarse layers of two of the flatbed filters, indirect adhesion was most prominent, whereas direct adhesion was most prominent in the other flatbed filter. For the most part, granulocytes were captured by direct adhesion in the fine layers, but in one flatbed filter, capture apparently occurred by mechanical sieving. The results of this study suggest that the efficiency and the mechanism of WBC reduction depend on the physicochemical characteristics of the non-woven materials in the filters as well as the cellular composition of the RBC concentrates.     

Donor levels of serum alanine aminotransferase activity and antibody to hepatitis B core antigen associated with recipient hepatitis C and non- B, non-C outcomes

BACKGROUND: Hepatitis virus(es) that are neither hepatitis B (HBV) nor hepatitis C (HCV) (non-B, non-C [NBNC]) may be transmitted by transfusion. The present study assessed donor values for alanine aminotransferase (ALT) and antibody to hepatitis B core antigen (anti- HBc) for their association with HCV and NBNC hepatitis outcomes among allogeneic blood recipients. STUDY DESIGN AND METHODS: Data on blood donors and recipients enrolled in the Transfusion- Transmitted Viruses Study in four United States cities from 1974 through 1980 were supplemented by anti-HBc testing of donors and anti-HCV evaluation of recipients. Two statistical approaches estimated the value of these indirect tests in detecting donors associated with HCV seroconversion and NBNC hepatitis in recipients. RESULTS: For HCV cases, donor ALT alone (at > or = 60 IU/L) had a sensitivity and a specificity of 30 and 96 percent, respectively, and anti-HBc alone (at > or = 60% inhibition) had a sensitivity and specificity of 53 and 86 percent, respectively. The two markers combined had a sensitivity and a specificity of 69 and 83 percent. For NBNC hepatitis cases, each measure had low sensitivity (20%) that was not improved by using both (28%) [corrected]. CONCLUSION: The indirect tests proved to be equal in sensitivity to the first-generation anti-HCV tests. The positive predictive power of these indirect tests in the 1980s was sufficient to affect HCV incidence in studies during that period. Improved anti-HCV assays, however, replaced the need for indirect tests. The sensitivity of indirect tests for NBNC hepatitis contributed little.     

Hepatic expression of A disintegrin and metalloproteinase (ADAM) and ADAMs with thrombospondin motives (ADAM-TS) enzymes in patients with chronic liver diseases

BACKGROUND/AIMS: ADAMs (A Disintegrin And Metalloprotease) are multifunctional, membrane-bound and soluble cell surface glycoproteins with numerous functions in cell physiology. We assessed the expression of ADAMs in fibrotic liver disease of different aetiologies and clarified whether the expression of ADAMs is related to histological staging of fibrosis. In addition, the expression of ADAMs was determined in different cell types of liver. METHODS: Seventy-one biopsy samples from patients with chronic liver diseases were analyzed for mRNA expression of ADAM-8, -9, -12, -28, -TS1, -TS2, matrix metalloprotease (MMP)-2, -9 and tissue inhibitor of metalloproteinases-1 and -2 by quantitative real-time RT-PCR. RESULTS: The ADAM expression in liver injury is independent of aetiology. A strong correlation between ADAM -9, -28, -TS1 versus MMP-2 and SMA was identified. Activated hepatic stellate cells (HSC) showed increased mRNA expression of ADAM-8, -9, -12, -28, -TS2 compared to quiescent HSC. Significant differences between histological stages of fibrosis were found for ADAM-28, MMP-2 and MMP-9. CONCLUSIONS: The results suggest that ADAMs are differentially expressed in the liver. We assume that ADAM-9, -TS1 and -TS2 play a crucial role in extracellular matrix remodeling during fibrotic processes in the liver.     

Nonpeptidic Lysosomal Modulators Derived from Z-Phe-Ala-Diazomethylketone for Treating Protein Accumulation Diseases

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Evaluation der Ergebnisqualität des besonderen Heilverfahrens der Gesetzlichen Unfallversicherung

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