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Biomedical subjects

P Clark

Publications and source records attributed to P Clark.

At least 253 records · Page 14Linked to original sources
PubMed1983

Familial occurrence of alpha 1-antitrypsin deficiency and Weber-Christian disease.

Severe panniculitis of the Weber-Christian type occurred in two brothers, both with marked alpha 1-antitrypsin (alpha 1-AT) deficiency and phenotype PiZZ. Studies of inflammatory and immunologic function were undertaken in these two patients as well as in a third brother with severe alpha 1-AT deficiency but without Weber-Christian disease. The findings of these investigations were suggestive of exaggerated immunologic and inflammatory function with enhanced lymphocyte responsiveness to phytohemagglutinin, enhanced activation of neutrophils and monocytes, and accelerated delayed hypersensitivity responses in all three subjects. This hyperreactivity may explain the apparent association of alpha 1-AT deficiency with Weber-Christian disease.

Adult↗
PubMed1982

Genetic study of a family with two members with Weber Christian disease (panniculitis) and alpha 1 antitrypsin deficiency.

Alpha 1 antitrypsin phenotypes and serum levels are presented for a family in which two brothers have Weber Christian disease and alpha 1 antitrypsin (PI) Z phenotypes. Clinical histories are described for these two men. A younger brother has the PI Z phenotype but does not have the disease, indicating that additional genetic and/or environmental factors contribute to the pathogenesis of Weber Christian disease. However, the two cases described provide additional evidence for a relationship between alpha 1 antitrypsin deficiency and the development of symptoms.

Adult↗
PubMed1982

Immunogenetic factors in inflammatory eye disease. Influence of HLA-B27 and alpha 1-antitrypsin phenotypes on disease expression.

The relationship between the nature and severity of inflammatory eye disease was analyzed with respect to HLA antigens and alpha 1-antitrypsin phenotypes. Using standard ophthalmologic criteria, we divided patients with anterior uveitis into acute, chronic (greater than 3-month duration), bilateral, or recurrent disease. There was a significantly increased incidence of alpha 1-antitrypsin-deficient phenotypes in anterior uveitis, especially in those patients with severe (chronic, bilateral, or recurrent) disease. HLA-B27 acts as an independent predisposing factor: it was present in 22% of patients with their first attack of acute uveitis compared with 51% of patients with recurrent disease. Together, these genetic factors are present in 63% of patients with severe anterior uveitis and represent the most significant predisposing and prognostic factors so far detected.

HLA Antigens↗
PubMed1982

An excess of the Pi S allele in dizygotic twins and their mothers.

A significant excess of the alpha 1-antitrypsin (protease inhibitor) PiS allele has been found in 147 pairs of dizygotic (DZ) twins, but frequencies in 170 pairs of monozygotic (MZ) twins do not differ from those in a sample of 1007 blood donors. In 51 mothers of DZ twins the frequency of the PiS allele was double that in the same sample of donors, but there was no corresponding increase in the fathers of DZ twins nor in the parents of MZ twins. In an independent sample of 66 mothers of twins of unknown zygosity, there was also a significant excess of PiMPiS and PiMPiZ phenotypes, and this was particularly marked in the subsample of mothers of opposite-sex twin pairs. We speculate that lowered protease inhibitor levels in women carrying the PiS allele may enhance sperm migration, increase the probability of multiple ovulation, or both.

Alleles↗
PubMed1982

Acute abdomen masquerading as acute retention.

Urologists should remain constantly alert for patients with acute abdominal emergencies who may be admitted under their care with the erroneous diagnosis of acute retention. These cases are not uncommon. The patients may be suffering from suppression of urine, or their inability to pass urine may be the predominant symptom of their intra-abdominal catastrophe. When catheterization has yielded only a small amount of concentrated urine and has failed to relieve the patient's discomfort, think of an acute abdomen, investigate this possibility and treat the patient accordingly.

Abdomen, Acute↗
PubMed1982

Alpha-1-protease inhibitor phenotypes in Australia.

Alpha-1-protease inhibitor (alpha-1-antitrypsin) phenotypes of 1,007 white Australian blood donors, 597 Aboriginals and 63 Vietnamese immigrants were examined by isoelectric focussing in a pH range of 3.5--5.0. The infrequency of the PIM2 allele was high in the Aboriginal populations and low in the blood donors and Vietnamese. The S allele was higher in one Aboriginal group than in the blood donors but very low or absent in the rest. The Z allele was also absent in the northern Aboriginals. Other alleles found in low frequency were PII in the blood donors and the PIF in the Aborigines, with PIX in Aborigines, Vietnamese and blood donors.

Alleles↗
PubMed1982

Immunoregulation by alpha 1 antitrypsin.

alpha 1 antitrypsin (alpha 1 AT) deficiency is a common genetic disorder seen in about 10% of the population. It predisposes to the development of a large number of inflammatory and immunologic disorders including rheumatoid arthritis, systemic lupus erythematosus, juvenile chronic arthritis, anterior uveitis, ankylosing spondylitis, fibrosing alveolitis and emphysema. We have investigated immunologic function in subjects with severe alpha 1 AT deficiency and demonstrated serum mediated enhancement of lymphocyte response to PHA and increased zymosan activation of mononuclear cells and neutrophils as measured by their chemiluminescence. These patients also have accelerated delayed hypersensitivity responses and increased levels of factor B, C3 and C5 but normal levels of immunoglobulin and other complement components. Such abnormalities in immunoregulation demonstrate a tendency to hyperreactivity that may contribute to disease predisposition.

Complement System Proteins↗
PubMed1981

Thyroid function after mantle irradiation in Hodgkin's disease.

We studied the thyroid function of 64 patients with Hodgkin's disease who received mantle irradiation during the period 1966 to 1976. More than two thirds (44 of 64) had some thyroid dysfunction. Twenty had mild dysfunction manifested by an abnormal thyroid-stimulating hormone response to thyrotropin-releasing hormone. Twenty had what could be termed compensated hypothyroidism while four were overtly hypothyroid. The severity of dysfunction was not related to age, sex, or chemotherapy. We found, however, that decreased thyroid function was inversely proportional to the length of time between a diagnostic lymphangiogram and the radiation therapy. These results are consistent with the hypothesis that the iodine load of the lymphangiogram renders the thyroid gland more radiosensitive. Thyroxine suppression of the thyroid gland during the period from the lymphangiogram through the termination of radiation therapy is suggested as a means of avoiding thyroid injury.

Ethiodized Oil↗
PubMed1981

Autistic children's responses to structure and to interpersonal demands.

Ten autistic children were exposed to four different styles of approach by an adult, in which the common context was the child's involvement in the completion of a model-building task. These styles varied in the extent to which they made interpersonal demands of the child and in the amount of task-directed structure that was imposed. Measures based upon observation of the adult's and the children's behavior indicated that the styles applied were reliably discriminable, and that the children's responses, both social and task-directed, were positively related to the interpersonal and task-oriented demands that were made of them. Within the group of children tested, some individual differences in the responses to the styles were suggested that may be relevant to the diagnosis of autism.

Adolescent↗
PubMed1981

Incidence of adverse effects following metrizamide myelography in nonambulatory and ambulatory patients.

A study was done to determine whether cerebrospinal fluid loss through a lumbar puncture tear or metrizamide contact with central nervous system tissue is the more important contributor to postmyelographic headache. To do this, the influence of postmyelographic positioning was recorded in 43 ambulatory and 43 nonambulatory (confined to bed for 24 hours after the procedure) patients. The incidence of headache was 10.5% in the ambulatory group and 43.6% in the nonambulatory group (p less than 0.01). There were no significant differences between the two groups with respect to leg and back pain or nausea and vomiting. It is concluded that irritation of the central nervous system by metrizamide is more important as a causative factor of postmyelographic headache than is cerebrospinal fluid leakage. Following metrizamide myelography, patients can be encouraged to be ambulatory without any increase in the incidence of adverse effects.

Adult↗
PubMed1981

Directional dominance for low IgM and IgA levels.

A biometrical genetical analysis of IgG, IgM, and IgA levels in 134 sets of twins is reported. High heritabilities, around .8, are found for all three immunoglobulin levels, and possible reasons for lower heritabilities found in family studies are discussed. There is evidence for genetical dominance tending to decrease IgM and IgA levels, but there is no evidence for the importance of family environment although the presence of dominance may make its detection difficult. The causes of covariation in the three measurements are unclear in males but in females appear to be mainly environmental in correlations with IgA and equally genetical and environmental in the IgG-IgM correlation.

Adolescent↗
PubMed1980

A prospective randomized trial of tocainide in patients following myocardial infarction.

One hundred forty-six patients with recent acute myocardial infarction were grouped at random into those treated with tocainide, an oral analogue of lignocaine, or placebo and followed up for 6 months. In addition to standard investigations, a 24-hour ambulatory taped ECG recording was obtained prior to randomization and thereafter at 2, 8, 16, and 24 hours after discharge. The ECGs were analyzed by means of an automated, computerized reporting system. Forty-two patients had significant ventricular arrhythmias, 10 of whom had effective plasma levels of tocainide compared with 27 patients on placebo (P < 0.005). In the placebo patients with increasing mobilization there was a consistent rise in the number of ventricular ectopic beats per day. There was no such increase in the tocainide patients (P < 0.01). Side effects were few and the incidence of central nervous system side effects was similar in both the tocainide and placebo groups. There was no conclusive evidence of myocardial depression, heart rate and blood pressure being unchanged over the 6-month period. Although ventricular arrhythmias were suppressed, the number of patients in the study was too small to draw conclusions regarding the mortality rate.

Anilides↗
PubMed1980

In vivo stability and distribution of [131I]iodomethyl trimethylammonium chloride: concise communication.

[131I]iodomethyl trimethylammonium chloride (I-131 TMA) was prepared by the isotope-exchange reaction of diiodomethane with Na131I, followed by reaction with trimethylamine. A specific activity of 20 microCi/mg was obtained. Tissue distributions following intravenous injection of I-131 TMA in mice showed that between 10 min and 2 hr, the highest accumulations of radioactivity were in the urine, kidney, and heart. The uptake of this compound in the heart was very rapid, and the levels of radioactivity remained fairly high up to 2 hr. At 10, 30, 60, and 120 min the heart-to-blood ratios were, respectively, 10.2, 7.7, 8.3, and 9.6. Thyroid uptake of this compound in the rats and analysis of urine samples of the mouse indicated no extensive deiodination of the compound in vivo. These results show that this compound does have potential for use in myocardial imaging. In addition, its stability in vivo makes it very useful for the radiolabeling of compounds containing quaternary ammonium moieties.

Animals↗