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Biomedical subjects

C S Johnson

Publications and source records attributed to C S Johnson.

At least 145 records · Page 8Linked to original sources

Profiles of black and Latin-American patients having pernicious anemia. HLA antigens, lymphocytotoxic antibody, anti-parietal cell antibody serum gastrin levels, and ABO blood groups.

Because of the unusual presentation of pernicious anemia in many black women, a further characterization of this disease among the different racial groups was undertaken. The following were found. (1) Black women under the age of 50 years had a significantly lower prevalence of anti-parietal cell antibody than did "European" and Latin-American patients or even older black women. The disease in the younger black women resembles juvenile pernicious anemia in this respect. Blacks in general had a lower prevalence of anti-parietal cell antibody than did "Europeans" and Latin Americans, though the difference was not statistically significant. A particularly striking finding was that anti-parietal cell antibody occurred much less often than anti-intrinsic factor antibody among black patients. (2) An analysis of all published HLA data for patients of European origin indicated a weak association of pernicious anemia with HLA-B7. However, no antigen association among the black and Latin-American patients studied was found. HLA-DR typing, not previously reported even for whites, also showed no association. (3) Lymphocytotoxic antibody was equally increased in frequency in all racial groups. (4) Serum gastrin levels did not follow a racial pattern; however, women with pernicious anemia had much higher levels than did men. (5) Pernicious anemia was not associated with blood group A.

ABO Blood-Group System↗

Arterial blood pressure in adults with sickle cell disease.

Arterial blood pressures (BPs) in 187 adult patients with sickle cell disease, casually recorded during hospitalization or clinic visits, were compared with BPs from age- and sex-matched populations of black Americans. The BPs in those with sickle cell disease were significantly lower than those in the control populations in all ages and did not demonstrate the expected rise with advancing age. In these patients, there was no difference between BP and sex, degree of anemia, or hemoglobin genotype. Four patients had diastolic and two had systolic hypertension. The prevalence of hypertension was significantly less than that in the block population. These BP findings in sickle cell disease may be due to the renal tubular defect responsible for increased sodium and water excretion, which may blunt the plasma volume expansion necessary for sustained hypertension and thus promote lower arterial pressures, similar to that situation observed in patients with salt-losing nephritis.

Adolescent↗

Hemoglobin Pasadena,alpha 2 beta 275(E19)Leu leads to Arg. Identification by high performance liquid chromatography of a new unstable variant with increased oxygen affinity.

This report describes structural and functional characteristics of a new hemoglobin variant, Hb Pasadena [beta 75(E19)Leu leads to Arg] and illustrates the use of high performance liquid chromatography in the analysis of the substitution. As a consequence of this substitution, there is compensated hemolysis, instability, increased oxygen affinity, and reduced cooperativily. However, the health of the individual is hardly affected.

Arginine↗

Release of soluble "blocking" and "suppressor" factors from normal lymphocytes treated with RNA from spleens of tumour-bearing mice.

RNA extracted from the spleens of tumour-bearing (TLRNA) and tumour-immune (ILRNA) mice was shown to transfer to normal lymphocytes (NL) the ability to produce factors that blocked specific tumour-cell cytotoxicity and mediated specific antibody-dependent cell cytotoxicity (ADCC). Aliquots of normal C3H mouse lymphocytes were treated with TLRNA or ILRNA and cultured in vitro in the absence of tumour antigen. Supernatants were collected at 24h intervals and tested in a microcytotoxicity assay for blocking and ADCC activities. Factors that inhibited tumour destruction by specifically sensitized lymphocytes at the level of both the tumour cells and effector cells were demonstrable in culture supernatants of NL pretreated with TLRNA (50 or 100 microgram/4 X 10(6) cells) but not ILRNA. However, treatment of NL with either RNA resulted in the production factors that mediated tumour-specific ADCC. Cytotoxicity testing and absorption studies of the tumour cell and a control cell (LM) indicated that factors mediating ADCC and blocking at the target-cell level were specific for the tumour. Suppressor activity at the effector-cell level was not absorbed by tumour cells and represents a separate and distinct mechanism of immunosuppression. These data indicate that RNA faithfully transfers "suppressive" as well as "positive" types of immune responses that have been reported previously for lymphocytes obtained directly from tumour-bearing and tumour-immune animals.

Animals↗

Acute lymphocytic leukemia manifesting as splenic rupture.

Pathologic rupture of the spleen is a rare phenomenon in acute lymphocytic leukemia, and its mechanism is unknown. This complication has been seen almost exclusively in men, and at ages much older than those of the majority of patients who have this leukemia; these unusual features are also unexplained. A case of acute lymphoctyic leukemia with pathologic rupture of the spleen in which the pathologic findings suggest that the splenic rupture was due to infiltration and destruction of the splenic capsule by leukemic cells is reported.

Adult↗

The radiologic significance of the left pulmonary ligament. Experience with 26 patients.

Pathologic processes confined to or by the left pulmonary ligament present a confusing radiographic appearance. Such processes may simulate pleural scarring, parenchymal scarring, or even left lower lobe collapse. Radiologic awareness of this structure is limited because in the normal state, it is not visualized on either posteroanterior or lateral chest x-ray films. The absence of secondary signs of left lower lobe collapse, together with a process which may extend above the level of the left hilum, is valuable in confirming that such a triangular density seen behind the left cardiac border is disease in or confined by the pulmonary ligament, rather than left lower lobe collapse.

Adult↗

Intravenous iron-dextran in the treatment of iron deficient anemia.

Administration of oral ferrous salts is the preferred method of treatment for anemia due to iron deficiency. However, in certain clinical situations, the response to oral therapy may be suboptimal. Parenteral iron therapy is effective in these instances and may produce a faster response than the oral route.Of 30 patients treated by total dose intravenous infusion of iron-dextran, a prompt reticulocytosis occurred in all patients except one case associated with systemic lupus erythematosus. Hematologic improvement in this case followed remission of the systemic lupus erythematosus. Hematologic response was complete in 18 patients in five to nine weeks, but could not be evaluated in 11 cases because of recurrent bleeding. There were two adverse reactions: generalized pruritus after injection in one patient, and superficial thrombophlebitis at the injection site of another.The response to therapy in iron deficient anemia is dependent on bone marrow capacity, the severity of the anemia, and the availability of iron. Response was fastest in those who had been severely anemic for prolonged periods of time. Total dose infusion with iron-dextran is a safe and effective treatment for iron deficient anemia in selected cases. Initial response appears to be faster than that on oral therapy with the exception of those with a mild degree of anemia.

Adult↗

Racial patterns in pernicious anemia. Early age at onset and increased frequency of intrinsic-factor antibody in black women.

Pernicious anemia affects primarily elderly northern Europeans, but may affect others more often than previously thought. Therefore, we analyzed the data from 156 documented cases: there were 73 patients of "European" origin, 52 black patients and 31 Latin-American patients. The mean age (+/- 1 S.D.) at presentation among black women, 53 +/- 16 years, was lower than that of all the others (P less than 0.001 in most comparisons), and seven of the 33 black women were less than 40 years old. In addition, 23 of the 24 black women tested had circulating antibody to intrinsic factor. A similar though less striking antibody prevalence (85 per cent) and age pattern (60 +/- 13 years) in Latin-American women did not reach statistical significance. No other group exceeded the usual 55 to 70 per cent prevalence of antibody. These finding suggest a different form of or a different response to the disease in black women and perhaps in Latin-American women.

Adult↗

Bilirubin-protein interactions monitored by difference spectroscopy.

Difference spectroscopy is used to monitor bilirubin-protein interactions, to assess the residual binding capacity of proteins for bilirubin. A change in the difference spectra monitored at 482 nm is directly proportional to bound bilirubin up to a molar ratio of bilirubin to albumin of approximately 1; increasing bilirubin beyond the 1:1 molar ratio does not further change the difference spectra. After excess free bilirubin is added, the change in the difference spectrum is proportional to the residual binding capacity of the serum for bilirubin. The risk of kernicterus among neonates may be assessed by monitoring the residual bilirubin binding capacity of serum. This report summarizes our research effort leading to an assay method which requires only 40 microliter of serum and can be completed in less than 10 min.

Adult↗

Pregnancy and delivery in a woman with hemophilia B.

Hemophilia B (Factor IX deficiency, Christmas disease) may cause excessive bleeding in women. The obstetric and hematologic management of the pregnancy and delivery of a woman with a Factor IX level of 4% is presented and discussed.

Adult↗