James J. Castles

Acute Fulminant Aortic and Mitral Insufficiency in Ankylosing Spondylitis

EXTRASKELETAL manifestations of ankylosing spondylitis include aortitis with aortic1 , 2 and rarely mitral3 , 4 valvular insufficiency. Cardiac dysfunction usually develops late and progresses slowly.1 The acute case of aortitis presented below required emergency bivalvular replacement antedating spondylitis. Case Report A 16-year-old boy was admitted to Sacramento Medical Center, on August 10, 1971, with a three-week history of exertional dyspnea, constitutional symptoms and a 4-kg weight loss. The history was negative for previous murmurs. The blood pressure was 120/50 mm Hg, the pulse 90, and the temperature 37.0°C. The precordium was hyperactive, with a Grade IV of VI harsh systolic murmur at the .xa0.xa0.

The activity of purine salvage pathway enzymes in murine and horse models of congenital and acquired dysimmunity.

Previous studies of human congenital immunodeficiency states and in vitro observations of lymphocyte response to mitogens have implicated two purine salvage pathway enzymes, andenosine deaminase (ADA) and nucleoside phosphorylase (NP), as critical in the normal maturation and/or function of the immune system. Based on this information, ADA and NP activities were examined in a variety of congenital and acquired animal models of dysimmunity. The animals studied herein included: congenitally athymic (nude) mice; congenitally asplenic mice; congenitally athymic-asplenic mice; motheaten mice; New Zealand mice; and Arabian foals with severe combined immunodeficiency. No significant differences in the activities of ADA and NP were observed in any of these animals when compared with either normal littermates or animals with intact immune function. Major species differences were apparent when erythrocyte ADA acitivty was compared between mice and horses. In contrast, only minor strain alterations in ADA or NP activity were noted between several inbred groups of mice.

The behavior of fetal canine cardiac cells in culture: synthesis and phosphorylation of myosin.

Abstract This study describes the first in vitro culturing of canine cardiac cells. Canine cardiac myosin which was synthesized in a 14-day tissue culture, based on l -[3H]leucine incorporation, was precipitated with goat γG antimyosin (cardiac-specific) and analyzed on dodecylsulfate gels; the specific activity of the highly purified myosin chains was determined. Incorporation of 32PO4 was similarly analyzed. The comparative degree of synthesis and phosphorylation of myosin chains, occurring in culture, was the same as that obtained in vivo. Both l -[3H]leucine and 32PO4 incorporation were inhibited by addition of cycloheximide to the culture medium. Removal of 32PO4 from myosin heavy chains with base treatment indicated the presence of phosphoserine and/or phosphothreonine in canine cardiac myosin heavy chains. Myosins from fetal and adult canine cardiac tissue were immunologically identical with each other and with the cultured fetal tissue; all had similar myosin ATPase activity and the degree of heavy chain phosphorylation was similar. The tissue and techniques used here gave a high yield of cardiac myocytes based principally on synthesis of cardiac-specific myosin.

Standard Gibbs energies of formation of ZnC2O4 · 2H2O(s), CdC2O4 · 3H2O(s), Hg2C2O4(s), and PbC2O4(s) at 298 K and 1 bar

Abstract We test the hypothesis that cycling of some heavy metals in soils and aquifers is affected by equilibrium with oxalate solids. The hypothesis was tested using electrochemical cells that allow us to determine Δ G f ∘[PbC 2 O 4 (s)], Δ G f ∘[CdC 2 O 4 · 3H 2 O(s)], Δ G f ∘[ZnC 2 O 4 · 2H 2 O(s)], and Δ G f ∘[Hg 2 C 2 O 4 (s)] and hence, the solubilities in soil solutions. The approach was stepwise. First, reversible equilibria was achieved using an electrochemical cell: Pb(Hg),2-phase ¦PbC 2 O 4 (s), CaC 2 O 4 · H 2 O(s)¦CaCl 2 (aq,m)¦Hg 2 Cl 2 (s)¦ Hg(1), in order to calculate Δ G f ∘[PbC 2 O 4 (s)] from the relatively well-known value of G f ∘[CaC 2 O 4 · H 2 O(s)]. This value of Δ G f ∘[PbC 2 O 4 (s)] then allowed us to obtain values of Δ G f ∘[CdC 2 O 4 · 3H 2 O(s)], Δ G f ∘[ZnC 2 O 4 · 2H 2 O(s)], and Δ G f ∘[Hg 2 C 2 O 4 (s)] from suitable electrochemical cells. When these values of Δ G f ∘ are incorporated into multicomponent speciation calculations, we find that CdC 2 O 4 · 3H 2 O(s), Hg 2 C 2 O 4 (s), and ZnC 2 O 4 · 2H 2 O(s) are unlikely to form except in highly contaminated soils, or in intercellular environments where the concentration of dissolved oxalate is very high. Of these heavy-metal-oxalate minerals, it is conceivable that the PbC 2 O 4 (s) may reach equilibrium in soil solutions. Although the metal-oxalate solids may precipitate locally in the rhizosphere, these solids would not be in equilibrium with the adjacent soil solution.

Case Report: Eosinophilic Fasciitis: A Distinct Clinical Entity?

Abstract Eosinophilic Fasciitis is a syndrome characterized by exertion related scleroderma-like skin changes, peripheral eosinophilia, hypergam-maglobulinemia and diffuse faciitis. Controversy exists as to the precise classification of the syndrome, i.e., whether it is a distinct entity or a variant of scleroderma . We describe a patient with eosinophilic faciitis but with several unique features: 1) progressive skin changes unresponsive to corticosteroid therapy; 2) elevated anti-DNA antibodies; 3) hypocomplementemia; and 4) a followup biopsy showing sclerodermatoid skin changes. These features and others relating to the controversial aspects of classification of eosinophilic fasciitis are discussed.

Alpha-fetoprotein-associated alterations of New Zealand mouse immunopathology.

The influence(s) of chronic injections of alpha-fetoprotein (AFP), derived from murine amniotic fluid, on the natural history of immunopathology and autoantibody production in New Zealand mice was studied and compared to analagous treatment with albumin, transferrin, or phosphate-buffered saline. Treatment of young New Zealand Black (NZB) mice with AFP, at sera levels of 60-210 micrograms/ml, significantly reduces the titer of IgG1, IgG2, and IgA antierythrocyte antibodies. Similarly, such treated mice have relatively normal levels of splenic Thy-1.2-bearing cells and sera immunoglobulins, at older ages, compared to control groups. In contrast, AFP has no apparent effect on the appearance of either naturally occurring thymocytotoxic antibodies (NTa) or lymphoma. Moreover, the positive features of AFP on disease were only noted for mice treated early in life; AFP had no effect when injected into older animals. Similar, but not as dramatic, changes were observed in NZB x NZW F1 hybrids. It is concluded that in pharmacologic doses, mouse aminotic fluid enriched with AFP may alter the appearance of thymic-dependent autoantibodies.

Immunologic characteristics of hereditarily asplenic (Dh/+) New Zealand Black x White F1 mice

Abstract The natural history of spontaneous immunopathology and autoimmune phenomena was studied in hereditarily asplenic New Zealand Black x White F 1 (NZB/W) hybrid mice. NZB/W mice, independent of sex, and including both Dh/+ and +/+ animals, had major age dependent reductions in immune function, including reduction in responsiveness to mitogens and sheep red blood cells and an inability to be tolerized by bovine gamma globulin. In contrast, there were major differences between male and female Dh/+ and +/+ mice with respect to survival and expression of renal disease. Dh/+ female mice had a lower median survival than +/+ mice; in contrast, male Dh/+ mice live longer than +/+ controls. These alterations in survival were reflected in renal and choroid plexus histology and proteinuria. The differences between female NZB/W Dh/+ and +/+ mice appeared to be accounted for by reductions in serum IgM, but enhanced IgG anti-DNA antibodies. The differences between female NZB/W Dh/+ and +/+ mice appeared secondary to the negligible titers of anti-nucleic acid antibodies in male Dh/+ animals. Hereditary absence of the spleen is significantly different from either neonatal or adult splenectomy, and its study, in parallel with other New Zealand immunologic mutants, suggests the existence of multiple independent developmental defects in the pathogenesis and clinical expression of autoimmune disease.