Characteristics of large granular lymphocyte leukemia associated with variable common immunodeficiency disorders: A study of 12 cases.

OBJECTIVES: Common Variable Immunodeficiency Disorders (CVID) and Large Granular Lymphocytes leukemia (LGLL) exhibit diverse clinical manifestations including infections, dysimmunity, and lymphoproliferation. Recent decades have seen the discovery of new genes in the lymphopoiesis pathway, such as JAK-STAT. This case series supplemented by a literature review aims to describe clinical and biological characteristics of patients with both CIVD and LGLL. METHODOLOGY: Patients were included through a call for comments to French and Belgian centers and through a literature review via PubMed. Clinical characteristics were compared to two large French cohort involving CVID and LGLL patients. RESULTS: Twelve patients were included. In all cases, CVID precedes LLGL (median diagnosis delay for LLGL was 7 years). Most cases presented with splenomegaly and autoimmune cytopenia. Ten out of 12 patients underwent splenectomy during follow up. CONCLUSIONS: Patients with LGLL and CVID differ from patients without immune deficiency in term of clinical presentation and prognosis. We suggest CVID may act as a trigger of LGL lymphocytosis, due to endogenous and exogenous antigenic pressure leading to the selection of a dominant LGL clone and stimulation of the JAK-STAT pathway. The role of splenomegaly and splenectomy in LGLL onset warrant further investigation in future studies.

Behavior of atrial natriuretic factor in an experimental model of Trypanosoma cruzi infection in rats.

Enhanced atrial natriuretic factor (ANF) production by the heart is related to hemodynamic overload, cardiac growth, and hypertrophy. The heart is one of the most affected organs during Trypanosoma cruzi infection. We tested the hypothesis that myocarditis produced by parasite infection alters the natriuretic peptide system by investigating the behavior of plasma ANF during the acute and chronic stages of T. cruzi infection in rats. Sprague-Dawley rats were infected with T. cruzi clone Sylvio-X10/7. Cardiac morphology showed damage to myocardial cells and lymphocyte infiltration in the acute phase; and fibrosis and cell atrophy in the chronic period. Plasma ANF levels (radioimmunoassay) were significantly higher in acute (348 +/- 40 vs. 195 +/- 36 pg/ml, P < 0.05, n = 17) and chronic T. cruzi myocarditis (545 +/- 81 vs. 229 +/- 38 pg/ml, P < 0.001, n = 11) than in their respective controls (n = 10 and 14). Rats in the chronic phase also showed higher levels than rats in the acute phase (P < 0.01). The damage of myocardial cells produced by the parasite and the subsequent inflammatory response could be responsible for the elevation of plasma ANF during the acute period of T. cruzi infection. The highest plasma ANF levels found in chronically infected rats could be derived from the progressive failure of cardiac function.

Rubella screening and vaccination program for US Air Force trainees: an analysis of findings.

A preventive medicine program of rubella control for trainees at the Air Force Military Training Center, Lackland AFB, Texas, was begun in October 1977. Incoming trainees were screened for rubella susceptibility, and female trainees were additionally screened for pregnancy. During the period October 1977 to December 1978, an overall rubella susceptibility rate of 17.3 per cent was determined for 71,387 trainees entering basic training. Flights (50 persons each) to which these trainees are assigned varied widely in susceptibility from 0.0-47.7 per cent. Comparisons of susceptibility rates for trainees for for geographic areas and states indicated the highest overall percentage of susceptibles were from the Pacific geographic area with California showing the highest susceptibility (24.5 per cent). Race specific susceptibility rates were found to differ significantly between Whites and Blacks, 17.6 vs 14.8 per cent, respectively. The immunization program was effective as judged by a dampening of the incidence of clinical rubella at the basic training center and at other secondary training centers.

Rubella immunity: comparison of hemagglutination inhibition and radioimmunoassay antibody methods.

A threshold (1:10) rubella antibody hemagglutination inhibition (HAI) titer was obtained for 288 of 6537 (4.4%) obstetric patients. Random sera from 84 of these patients were compared for rubella antibody by both HAI and a very sensitive radioimmunoassay (RIA) technique. By RIA, 17% of the sera had no detectable rubella antibody, suggesting that those patients were truly susceptible to rubella. In addition, 8 of 55 paired prevaccine and postvaccine sera with titer increases demonstrated by HAI were analyzed by RIA. Seven of the 8 prevaccine sera were shown to have no rubella virus antibody by RIA, and RIA showed seroconversion in all 8 postvaccine sera.

Identification of lipoxygenase-linoleate decomposition products by direct gas chromatography-mass spectrometry.

Lipoxygenase, prepared from Virginia-type peanuts, was used to catalyze the oxidation of linoleic acid and methyl linoleate to form the C-9 and C-13 hydroperoxides. These reactions were monitored by rapid unconventional direct gas chromatography-mass spectroscopy. An aliquot of the enzymatic reaction mixture, without prior extraction or chemical modification, was secured directly into the heated (40-70 C) or nonheated (room temperature) injection system. When the reaction mixture was analyzed at room temperature, only hexanal was found. At elevated temperatures, five major and several minor components were identified. The predominant compounds identified were pentane, hexanal, 2-pentylfuran, trans-2,cis-4-decadienal, and trans-2,trans-4-decadienal. These products originate from decomposition of either the C-9 or C-13 hydroperoxides generated by peanut lipoxygenase.

Storage Quality of Brown Rice as Affected by Packaging with and without Carbon Dioxide.

Shelled brown rice (100 g/bag) was packed in regular plastic bags in air, in laminated film (nylon-EVA) bags in air, and in laminated bags plus CO2. Samples of each were stored in the dark at 4 C and at 24 C, and samples were removed after 1, 3, 5 and 7 months for analysis of odor changes, free fatty acids, total microbial counts, total lipolytic fungi and bacteria, lipid peroxides and gas chromatographic volatiles profiles. Brown rice in laminated bags plus CO2 was more stable under refrigerated conditions than at ambient temperatures. However, at 24-C storage there was no consistent significant decrease in free fatty acids, lipid peroxides and volatile compounds in these bags compared to the other types of packing. Laminated bags seem to have had an adverse effect on total microbial populations at both 4 and 24 C. Selection of either type of package for brown rice would be governed by end use, storage time and conditions.

An endemic focus of Trypanosoma cruzi infection in a subhuman primate research colony.

Leishmanial forms of Trypanosoma cruzi with multifocal pseudocysts were identified in a deceased adult female rhesus monkey (Macaca mulatta) that had been maintained in an outdoor primate colony in southern Texas. Subsequent clinical investigation of the remainder of the colony identified 20 additional monkeys seropositive for T cruzi. Concurrent epizootiologic surveys confirmed the presence of sylvatic reservoirs and triatomine vectors of T cruzi infection and identified a natural T cruzi transmission cycle. Veterinarians associated with the research colony were informed of the potential for further T cruzi transmission and appropriate insect vector and reservoir animal control programs were implemented..

The rationale, development, and standardization of a basic word vocabulary test.

The results of the studies to date indicate that the Basic Word Vocabulary Test provides a range of items in terms of item difficulty levels useful in printed form from about the third grade to the highest educational levels. Since pictorial and orally given vocabulary tests are used from about ages 2 to 8 years, further work should be done to extend the scale downward so that a single comprehensive vocabulary scale ranging from age 2 years to the highest level of verbal development is available for general use. Validation studies should also be conducted with other well-known intelligence tests so that scores can be compared. Alternate forms need to be developed to allow for longitudinal studies of growth and development. The use of a single standard of measurement of vocabulary development, suitable over a wide range of age and ability levels, by different investigators should materially aid in comparing results across studies and samples and lead to more consistent findings, advances in knowledge, and wider application of findings in practical circumstances, The findings presented in this report indicate that the Basic Word Vocabulary Test adequately measures basic word knowledge acquisition and development. The BWVT is suitable for evaluation of individuals and for use in making group comparisons in levels of basic word knowledge attainment, growth, and development.

Evaluation of agar-gel double diffusion for the diagnosis of adenovirus infection.

Agar-gel double diffusion was compared to the Laboratory Branch complement fixation test. The efficacy of the agar-gel double diffusion test for the semiquantitation of group-specific adenovirus antibody and its usefulness as a serodiagnostic aid for the evaluation of adenovirus infection was determined.

Apparent spontaneous induction of Staphylococcus aureus isolated from clinical sources.

Apparent spontaneous induction in staphylococcal strains from two clinical specimens was described. One of the two phages associated with these strains was found useful in typing otherwise untypable strains.