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Biomedical subjects

J Lieberman

Publications and source records attributed to J Lieberman.

At least 127 records · Page 7Linked to original sources
PubMed1991

A CF-lectin factor in amniotic fluid from pregnancies at risk for cystic fibrosis.

A method was developed for the assay of the CF-lectin factor in amniotic fluid using samples from fetuses identified by other means as having or not having cystic fibrosis (CF). The method involves protein removal from the amniotic fluid by filtration through Centricon-10 Amicon filters, admixture of filtrate with normal serum as a source of IgM, and incubation in 5% Dextran-Sulfate with mouse red blood cells to detect specific hemagglutination. Blind samples submitted from four different laboratories were used to evaluate the ability of the assay to differentiate CF fetuses from fetuses without CF. Storage of samples from one laboratory had been quite prolonged and was associated with poor correlations. Freezing and thawing of samples were shown to deactivate the CF-lectin factor. In three of the four studies, CF lectin was detected in 77% of pregnancies identified as CF at birth, prenatally through microvillar enzyme assay or through DNA analysis of terminated pregnancies. Eighty-three percent of samples identified as normal were confirmed as such by a lack of CF-lectin activity. These data suggest that CF fetuses produce, and are exposed to, the CF-lectin factor at least as early as the second trimester of gestation.

Amniotic Fluid↗
PubMed1991

Alpha-1 antitrypsin deficiency in a patient with widespread prurigo nodularis.

Skin lesions associated with alpha 1-antitrypsin deficiency are becoming better defined and understood. Deficiency in this major antiproteinase, which neutralizes multiple proteolytic enzymes ranging from collagenases and elastases to trypsin and chymotrypsin, thus results in significant tissue autodigestion. This anti-proteinase is secreted by activated lymphocytes and macrophages, suggesting the existence of homeostasis which titrates the release of proteolytic enzymes by these cells, and the adequate neutralization of these proteases in order to prevent excessive tissue autodigestion each time these inflammatory cells are activated. We report a patient with alpha 1-antitrypsin deficiency who, following insect bites and cellulitis developed widespread itching and scratching, leading to widespread lesions of prurigo nodularis. The colonization of his multiple skin lesions with Staphylococcus aureus and the release of potent T cell mitogens, such as Protein A and enterotoxin A from the bacterial cell membrane may have resulted in the release of additional proteolytic enzymes by the activated lymphocytes and macrophages, without the concomitant secretion of alpha 1-antitrypsin with subsequent aggravation of his pruritus. These concepts are supported by electron microscopic evidence of excessive tissue autodigestion, and by immunocytochemical data identifying the presence of T helper and T cytotoxic/suppressor lymphocytes as well as macrophages within the upper dermis.

Humans↗
PubMed1991

Intermediate alpha 1-antitrypsin deficiency with apical lung bullae and spontaneous pneumothorax. Presence of a Z variant in an American black.

A 43-year-old black man had an 18-year history of apical lung cystic-bullous disease. Following two episodes of spontaneous pneumothorax and two instances of thoracotomy for bullectomy and pleural abrasion, he was found to have an intermediate AAT deficiency with an MZ phenotype. It is believed that this is the first case of localized bullous lung disease to be reported in association with any degree of AAT deficiency. There is evidence that the cystic lesions progressed to form upper lobe bullae. It is postulated that the AAT deficiency may have played a role in this progression, as did the patient's cigarette smoking. Following two instances of surgery, CT scans of the lungs, compliance studies and complete pulmonary function tests show no further evidence of lung bullae or emphysema. The rarity of the Z variant of AAT in blacks is discussed.

Adult↗
PubMed1990

Age-related decrease in serum angiotensin converting enzyme activity: the role of thyroidal status and food intake.

The serum ACE activity was determined in male Fischer 344 rats at 2, 6, 13, and 25 months of age to determine whether serum angiotensin converting enzyme (ACE) activity is a potential biomarker of tissue hypothyroidism in aged rats. Since rodent serum contains an ACE activity inhibitor, the measurements were done in both undiluted and 1:8 diluted sera. The highest serum inhibitor activity was found in the 2-month-old animals. The serum ACE activity measured in the diluted serum of the aged rats (77.6 +/- 2.9 units/ml) was significantly reduced compared to 2-month-old (178.2 +/- 6.4 units/ml), 6-month-old (101.5 +/- 6.1 units/ml) and 13-month-old rats (84.9 +/- 8.6 units/ml); (p less than 0.001). Hyperthyroidism induced by injecting L-triiodothyronine (T3) 15 micrograms/100 gm body weight intraperitoneally for 10 days increased the serum ACE activity in the older rats, but reduced the levels in 2-month-old rats. There was no significant change in 6-month-old rats. The levels of serum ACE activity in hypothyroid 6-month-old rats (95.5 +/- 3.5 units/ml) and in 2-month-old-rats (94.2 +/- 4.0 units/ml) were similar to the level seen in hypothyroid old rats (88.9 +/- 5.8 units/ml). Pair feeding of young rats (8 months old) with old did not alter the baseline ACE level (117.4 +/- 3.7 units/ml) or the T3 stimulated (105.2 +/- 10.2 units/ml) serum ACE activity. It is concluded that the reduced serum ACE activity in aged rats cannot be accounted for by the reduced caloric intake or reduced serum thyroid hormone levels.(ABSTRACT TRUNCATED AT 250 WORDS)

Aging↗
PubMed1990

Assay of a urinary CF-lectin factor as a second diagnostic test for cystic fibrosis.

Analysis of urine samples for a lectin-like factor in patients with a diagnosis of cystic fibrosis (CF) revealed that the low molecular weight (MW) factor is present in urine only from such patients and not from heterozygous carriers of the CF gene or from normal controls. The urine fraction containing the factor must be separated from whole urine by a gel filtration column (TSK-20) for the activity to appear. The assay requires addition of an aliquot of normal serum to provide the necessary IgM to which the CF-factor binds, resulting in the lectin-like activity that causes agglutination of mouse erythrocytes. All 22 CF patients tested, who were not receiving intravenous (IV) antibiotics had positive CF-lectin urinary activity, whereas five others receiving IV aminoglycosides were negative. Four patients with a clinical diagnosis of CF, but with normal sweat tests (35-54 mEq/L), all had positive urinary CF-lectin tests. A blind study in which urine samples were shipped from Miami, FL to Sepulveda, CA was completely successful in correctly identifying 11 samples from CF patients as compared with ten from non-CF patients. It was concluded that an assay for urinary CF-lectin factor is specific and reliable for confirming a diagnosis of CF when the sweat test is indeterminate, and when patients have not received recent IV aminoglycosides.

Adolescent↗
PubMed1990

Profile of bronchospastic disease in Puerto Rican patients in New York City. A possible relationship to alpha 1-antitrypsin variants.

A high prevalence of asthmalike symptoms was noted among patients of Puerto Rican descent attending Beth Israel and North Central Bronx Medical Centers in New York City, as compared with other ethnic groups. An evaluation of family and medical histories, pulmonary function data, and alpha 1-antitrypsin phenotypes was undertaken in such Puerto Rican patients and control subjects without asthma. The patients showed a higher proportion of MS and MV phenotypes. All the patients in both MM and variant phenotype groups, with the exception of four MM patients, had features indicative of asthma, with labile airway obstruction, and elevated serum immunoglobulin E and eosinophil levels. The latter was significantly higher in the patients with variant phenotypes than in MM patients. Patients with alpha 1-antitrypsin variants also had much shorter smoking histories as compared with the MM group, and all reported histories of asthma in first-degree relatives, as compared with 66% among the MM patients. We conclude that there is an increased incidence of asthma among Puerto Ricans in New York City, and that the antitrypsin variant phenotypes (specifically S and V) play a role in this incidence and its expression.

Asthma↗
PubMed1989

Clozapine pharmacology and tardive dyskinesia.

Clozapine, an atypical neuroleptic, does not cause extrapyramidal symptoms of Parkinsonism and dystonia and appears to have a reduced or absent capacity to produce tardive dyskinesia. 37 subjects, most with chronic schizophrenia, were treated with clozapine and TD outcome was analyzed. A subset of these subjects underwent plasma and CSF studies. TD response was heterogenous, but a proportion of patients improved with clozapine treatment. Neurochemical data differed from published reports of classical neuroleptics with the most robust effect produced by clozapine seen in CSF norepinephrine levels. Other neurochemical data and implications for the mechanism of clozapine in TD are reviewed.

Adolescent↗
PubMed1989

3,5,3'-triiodothyroacetic acid therapy for thyroid hormone resistance.

3,5,3'-Triiodothyroacetic Acid (Triac) is reputed to suppress pituitary secretion of TSH with minimal metabolic effects. Triac has been used successfully to treat eight patients with thyroid hormone resistance. We gave Triac to a woman with selective pituitary resistance for treatment of hyperthyroidism (patient 1) and to a man with generalized resistance and chronic schizophrenia to determine whether it would improve his schizophrenia (patient 2). Patient 1 was given 0.35-3.5 mg Triac/day; patient 2 was given 0.7-4.2 mg/day. Dosages were increased by 0.7 mg/day every 2 weeks. Serum T3, T4, free T4, TSH, TSH response to TRH, systolic time intervals (STI), angiotensin-converting enzyme (ACE), and lipids were monitored bimonthly. In both patients, there was no change in symptoms, weight, lipids, or STI. In patient 1, basal TSH suppressed from 16.3 to 1.5 mU/L; in patient 2, from 2.0 to 0.5 mU/L. The peak TSH response to TRH stimulation decreased from 144 to 12.5 mU/L in patient 1 and from 14.2 to 2.8 mU/L in patient 2. Serum T4 decreased from 160 to 109 nmol/L in patient 1 and from 270 to 192 nmol/L in patient 2. ACE levels were persistently elevated in both patients. Resting energy expenditure, measured by oxygen consumption, was increased by Triac in both patients (12% in patient 1 and 9% in patient 2). Although Triac suppressed TSH and T4 secretion in both patients, it did not reduce peripheral action of thyroid hormone as expressed in STI, resting energy expenditure, and ACE. We conclude that in these two patients with resistance to thyroid hormone, at the doses used to suppress TSH and T4 secretion, the intrinsic peripheral action of Triac offset whatever decrease in thyroid hormone secretion it produced.

Adult↗
PubMed1989

Effect of menses, estrogens and hemolysis on a serum-lectin-like factor in cystic fibrosis.

Twelve CF heterozygous and two CF homozygous women who were tested serially for the CF-lectin activities during one to two months of their menstrual cycles, were found consistently to have negative tests during menses. The specific hormonal alteration during menses that affects the assay is unknown. However, estrogenic medication administered without progesterone to postmenopausal heterozygous women also caused false-negative tests, suggesting that a balance between progesterone and estrogen is critical for the CF-lectin activity; mere depletion of these hormones alone does not interfere. The addition of estrogen in vitro also inhibited the CF-lectin activity at a physiologic concentration of 10(-6) M, and addition of an equivalent concentration of progesterone blocked this inhibitory effect. Future studies of the CF-lectin or its assay as a potential CF-carrier test must be limited to those women not menstruating at the time of blood drawing and not receiving estrogens. Hemolysis or contamination of the serum with RBC should invalidate that sample for testing, although the effect of RBC contamination can be circumvented with the addition of mannose. A more objective, simplified means of performing the CF-lectin assay is reported. A preliminary blind study taking these issues into consideration was 100 percent correct in detecting 16 CF patients and 17 obligate heterozygotes, and revealed 4 percent of 64 control subjects to have positive tests. The presence of this lectin-like factor may reflect the underlying biochemical defect responsible for this disease.

Agglutination Tests↗