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Biomedical subjects

J Lieberman

Publications and source records attributed to J Lieberman.

At least 199 records · Page 11Linked to original sources

Presence of a serum hemagglutinin (lectinlike factor) in cystic fibrosis homozygotes and heterozygotes.

A lectinlike activity was discovered in serum from 25 patients with CF, 70 obligate heterozygotes (parents), and 18 of 27 siblings (67%) of 675 controls, 4.2% were found to have a positive test for the CF-lectin, approximating the 5% estimated prevalence of CF heterozygotes. The CF-lectin can be detected by dextran-enhanced agglutination of mouse RBCs and confirmed by agglutination inhibition with D-fructose. This simple and inexpensive assay has been found useful to detect carriers of the CF gene, but variability of commercial dextran and some unusual characteristics of the lectin itself have interfered with the successful completion of blind studies. All sugar moieties present in mucus inhibit the agglutination of mouse RBCs by CF serum, implying that the CF-lectin can bind the glycoproteins of mucus. The factor may play a role in the pathogenesis of Cf by stimulating mucus secretion and reacting with mucous glycoproteins to cause their precipitation. Most antibiotics remove or inhibit the CF-lectin activity, suggesting a therapeutic effect of antibiotics unrelated to their antibacterial action. This discovery of a lectinlike factor in the blood of patients with CF and their parents opens a new area for investigation of this devastating disease.

Animals↗

Goodwill--what is it worth in the market?

This paper discusses the intangible asset that dentists refer to as practice goodwill. How is the market value of practice goodwill estimated? After defining goodwill in economic terms, a formula is given that dentists can use to estimate and negotiate the price of goodwill. Guidelines are suggested that indicate how the formula can best be used. Some general market conditions that can affect the price of goodwill in a particular area are discussed.

Financial Management↗

Serum angiotensin-converting enzyme: elevations in diabetes mellitus.

Elevated levels of serum angiotensin-converting enzyme were detected in 24% of 265 patients with diabetes mellitus. The elevations correlated strongly with the presence of severe retinopathy. Diabetes mellitus must be considered when an assay of serum angiotensin-converting enzyme is used to confirm a diagnosis of sarcoidosis.

Cardiovascular Diseases↗

alpha1-Antitrypsin deficiency in twins and parents-of-twins.

Serum-trypsin-inhibitory-capacity (STIC) and alpha1-antitrypsin (AAT) genotypes were evaluated in 83 twins and 112 paired parents-of-twins. An increased prevalence (17.0--21.9%) of intermediate AAT deficiency (STIC less than 0.95 units/ml) was detected in both of these groups as compared to a prevalence of 4.1% in 1,841 healthy controls. PiS and PiZ molecular variants of AAT were also found more frequently in the twin and parent groups, but this was not statistically significant. Low levels of protease inhibition may enhance fertility and a tendency towards twinning, since proteolytic enzymes are involved in fertilization of ova by sperm and in gametogenesis. Increased fertility and twinning may be heterozygous advantages for AAT deficiency.

Female↗

Angiotensin-I-converting enzyme and gallium scan in noninvasive evaluation of sarcoidosis.

Angiotensin-converting enzyme assays and gallium-scan results were obtained from 27 patients with biopsy-proven, clinically active sarcoidosis. Twenty-three of these patients had elevated converting enzyme levels, and 22 had positive gallium-scan results. Three of four patients with normal or borderline-elevated levels of angiotensin-converting enzyme also had positive gallium-scan results. Of 156 nonsarcoid patients (pulmonary and other diseases), 27 were found to have elevated serum converting enzyme levels, and 25 of these had negative gallium-scan results. These results indicate that the combination of an assay of angiotensin-converting enzyme and gallium scan increases diagnostic specificity from 83% to 99% without sacrificing sensitivity. We conclude that the concurrent use of angiotensin-converting enzyme assay and gallium scan is of value in the diagnosis of sarcoidosis.

Clinical Enzyme Tests↗

Pulmonary function in nonsmoking subjects with alpha1 antitrypsin deficiency (MZ phenotype).

We measured pulmonary functions in 10 nonsmoking asymptomatic subjects, ages 40.5 years +/- 9.2 years, with alpha1 antitrypsin heterozygous deficiency (phenotype MZ). The subjects were longstanding residents of the greater Los Angeles area. The range of physiologic studies and per cent of normal predicted values were forced vital capacity (FVC), 2.8 to 7.0 liters (86 to 124 per cent predicted); ratio of the forced expiratory volume in 1 second to the FVC, 70 to 86 per cent (86 to 104 per cent predicted); the ratio of the residual volume to total lung capacity, 28 to 44 per cent (94 to 119 per cent predicted); total lung capacity, 4.8 to 9.8 liters (80 to 119 per cent predicted); flow at 50 per cent FVC, 3.1 to 7.8 liters per second (69 to 140 per cent); and volume of isoflow, 7.3 to 26 per cent of forced vital capacity (38 to 137 per cent predicted). In eight patients studied, static deflation pressure volume curves were normal, and at respiratory rate of 60 breaths/min the ratio of dynamic compliance to static compliance did not fall below 84 per cent. We have found that these nonsmoking heterozygotes with alpha1 antitrypsin deficiency have normal pulmonary functions (within 1.67 SD of predicted mean).

Adult↗

Abnormal response of cultured lymphocytes to phytohemagglutinin and autologous serum in cystic fibrosis.

Lymphocytes from adults homozygous or heterozygous for cystic fibrosis show biochemical abnormalities when cultured for 48 hours in the presence of phytohemagglutinin and autologous serum. In contrast to the 45 per cent increase in total protein and beta-glucuronidase concentrations seen in healthy control subjects when measured per 10(10) cells, both concentrations decreased by 1 per cent in adults heterozygous for cystic fibrosis and by 18 per cent in adults homozygous for cystic fibrosis. The abnormal response of the lymphocytes from persons with cystic fibrosis was due to a serum factor and not to any intrinsic abnormality of the lymphocytes. An abnormal response to hytohemagglutinin occurred in only 14 per cent of 44 healthy control subjects, but in 100 per cent of 14 adults homozygous for cystic fibrosis and in 85 per cent of 26 adults presumed to be heterozygous for cystic fibrosis. As a result of this phenomenon, lymphocytic beta-glucuronidase concentrations were significantly lower than normal in patients with cystic fibrosis when the cells were cultured with phytohemagglutinin and autologous serum. The demonstration of this phenomenon in both homozygotes and presumed heterozygotes (parents) suggests a relationship to the genetic defect in cystic fibrosis.

Adolescent↗

Serum angiotensin-converting enzyme in leprosy and coccidioidomycosis.

Serum angiotensin-converting enzyme levels were found to be elevated in 71.4% of 42 leprosy patients, both treated and untreated, but in only one of 13 patients with disseminated coccidioidomycosis. The elevations with leprosy were present in association with each of the three major categories: lepromatous, borderline, or tuberculoid. Sulfone therapy had no immediate effect on the elevated serum levels, although long-term sulfone therapy appeared to result in lowering of the level. Corticosteroid therapy had a more immediate and dramatic effect on reducing the elevated angiotensin-converting enzyme level in leprosy. This assay cannot distinguish between sarcoidosis and leprosy or between the various categories of leprosy, but it can help differentiate sarcoidosis from fungal or tuberculous disease. Elevated levels of serum angiotensin-converting enzyme have now been associated with three diseases states: sarcoidosis, Gaucher's disease, and leprosy.

Coccidioidomycosis↗