Procoagulant activity of lymphocyte-macrophage populations in rabbits: selective increases in marrow, blood, and spleen cells during Shwartzman reactions

The present experiments examine leukocyte procoagulant activity using mononuclear cell populations purified or enriched from rabbit bone marrow, blood, spleen, lymph node, thymus, and pulmonary alveoli. Cells from these six sites, obtained from control and endotoxemic animals and assayed without an intermediate culture step, were found to have procoagulant activity identified as tissue factor. Under control conditions, tissue factor activity was found to be at low levels in marrow and blood populations compared to median activities 3- and 11- fold higher in populations from spleen and lymph node, and 33- and 45- fold higher in thymus and alveolar populations. By contrast to respective controls, significantly increased amounts of tissue factor (35-, 15-, and 12-fold at median levels) were found in marrow, blood, and spleen populations from endotoxemic animals. The types of leukocytes in these latter three populations were morphologically and histochemically indistinguishable from respective controls, indicating that endotoxin induced increases of activity in cells with relatively low amounts under control conditions. Activity did not change significantly in lymph node, thymus, or alveolar populations after endotoxemia. These studies show that tissue factor is present in a range of leukocyte populations not previously reported to have procoagulant activity. In addition, the finding of widespread gains of tissue factor in the marrow-blood-spleen pool due to endotoxemia provides new evidence supporting the importance of leukocyte procoagulants in Shwartzman-like reactions.     

Zinc ion dependent B-cell epitope, associated with primary Sjogren's syndrome, resides within the putative zinc finger domain of Ro60kD autoantigen: physical and immunologic properties

The Ro/La ribonucleoprotein (RNP) complex is composed of the proteins Ro60kD, Ro52kD, and La48kD that are in association with one small cytoplasmic RNA (YRNA). Specific protein-RNA and protein-protein interactions are thought to occur through the RNP and zinc-finger secondary structure elements of the Ro60kD protein. The aim of our study was to investigate the antigenic properties of the zinc finger domain of the Ro60KD autoantigen and its contribution to the formation of Ro/La RNP complex. It was found that the peptide VSLVCEKLCNEKLLKKARIHPFHILIA (Zif-1), which corresponds to the natural sequence of the zinc finger domain (301-327), and the peptide C(Acm)NEKLLKKARIC(Acm), analogous to the intermediate loop 310-319 (Zif-3) of the same domain of Ro60KD, are recognized by the majority of anti-Ro/SSA and anti-La/SSB positive sera (82.6% and 77.1%, respectively) in the absence of zinc ions. The same sera failed to react with Zif-1 peptide in the presence of Zn2+. In contrast, the addition of zinc ions was necessary for the binding of Zif-1 to recombinant Ro52KD as shown by direct binding experiments of the recombinant protein with synthetic peptides. Our data suggest the zinc finger domain of Ro60kD contains a B-cell epitope with high specificity for primary Sjogren's syndrome. Furthermore, depending on the presence of zinc ions, the zinc finger domain of the Ro60KD protein can exist in two different conformational states favoring either an interaction with the Ro52KD protein or binding with autoantibodies.     

Confluent hepatic fibrosis as the presenting imaging sign in nonadvanced alcoholic cirrhosis

Confluent hepatic fibrosis (CHF) is usually a feature of advanced cirrhosis, while occurrence in early stage compensated cirrhosis is uncommon. We report the imaging findings of masslike CHF in a patient with previously unsuspected compensated alcoholic cirrhosis. The combination of partially wedge shape, minimal capsular retraction, and homogeneous delayed enhancement on multiphase contrast-enhanced spiral CT led to the characterization of the lesions and establishment of the diagnosis of cirrhosis.     

GRAVES' DISEASE WITH ORGANIC MOOD SYNDROME (A Case Report)

A case of Graves'' disease with organic mood syndrome in a 3G year old man is reported. Patient had thyrotoxicosis and developed features of mania while in the hospital which necessitated antipsychotic drug therapy.KEY WORDS: Graves'' disease, ManiaFrank psychotic decompensation occurring in the background of Graves'' disease is an explosive clinical situation as manifestations range from severe manic excitement to total apathy. Although rare, the gravity of such situation warrants energetic intervention on both fronts. One such instance of organic mood syndrome with Graves'' disease is being presented, highlighting the problems encountered in the management.     

MR imaging of the scrotum: pathologic conditions

The utility of high-resolution magnetic resonance (MR) imaging in studying a variety of intratesticular and extratesticular pathologic conditions was assessed. The high magnetic signal intensity of the testis provided an excellent background for visualization of intratesticular abnormalities. Except for old blood, all intratesticular processes were less intense than testis, especially on T2-weighted images. The visualization of the tunica albuginea is a distinct advantage, allowing its assessment in cases of trauma or testicular tumors. Epididymal and spermatic cord abnormalities were easily recognized. All pathologic conditions were best seen on T2-weighted images acquired in the coronal plane. Balanced images allowed for tissue characterization.     

Brain MRI abnormalities in muscular dystrophy due to FKRP mutations

INTRODUCTION: FKRP mutations cause a muscular dystrophy which may present in the neonatal period (MDC1C) or later in life (LGMD2I). Intelligence and brain imaging have been previously reported as being normal in FKRP-associated muscular dystrophy, except in rare cases presenting with mental retardation associated with structural brain abnormalities. PATIENTS AND METHODS: We studied cerebral MRIs in twelve patients with FKRP-associated muscular dystrophy presenting in infancy or early childhood, at ages between 14 months and 43 years. Two patients had severe cognitive deficits, four had mild-moderate mental retardation and the rest were considered to have normal intelligence. All, but one were wheelchair-bound and 7 were mechanically ventilated. RESULTS: Brain MRI was abnormal in 9 of 12 patients. Brain atrophy was seen in 8 patients. One child had isolated ventricular enlargement at 4 years. Cortical atrophy involved predominantly temporal and frontal lobes and was most important at later ages. In two cases with serial images this atrophy seemed progressive. Three patients, two with severe and one with moderate mental retardation, showed structural abnormalities of the posterior fossa with hypoplasia of the vermis and pons, and cerebellar hemispheric cysts. These abnormalities were stable with time. Two of these three patients also showed diffuse white matter abnormalities in early childhood, which regressed with time. CONCLUSIONS: MRI abnormalities are common in patients with FKRP-associated muscular dystrophy presenting at birth or in early childhood. Progressive brain atrophy is the most frequent finding. Posterior fossa malformations and transient white matter changes may be seen in patients with associated mental retardation.     

Peripheral blood mononuclear cells from patients with rheumatoid arthritis suppress erythropoiesis in vitro via the production of tumor necrosis factor alpha

Abstract: Anemia is a frequent manifestation of rheumatoid arthritis, with a probably multifactorial etiology. We investigated the effect of peripheral blood mononuclear cell supernatants (PBMCS) from rheumatoid arthritis (RA) patients on hematopoietic colony formation in vitro, by using a methylcellulose assay. PBMCS from patients suppress in vitro erythroid (BFU-E), mixed-lineage (CFU-GEMM) and to a lesser degree granulocyte-macrophage (CFU-GM) progenitors. PBMCS from anemic RA patients were more suppressive for BFU-E than those from non-anemic patients. Addition of antibodies to tumor-necrosis factor alpha (TNFα) almost completely reversed the inhibition of BFU-E and CFU-GEMM, but had little effect on the CFU-GM colony formation. Antibodies to interferon-gamma (IFNγ) and interleukin-1 (IL-1) were not effective. The above data suggest that PBMCS from RA patients suppress in vitro erythropoiesis via the production of TNFα; a pathogenetic role for TNFα in the anemia of RA can be implied.