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Biomedical subjects

G S Jones

Publications and source records attributed to G S Jones.

At least 19 recordsLinked to original sources

Synthesis and binding to beta-adrenergic receptors of p-aminobenzyl analogues of practolol and atenolol.

The p-aminobenzyl analogues (8a and 8b, respectively) of the cardioselective beta-adrenergic receptor antagonists practolol and atenolol were prepared from the corresponding phenoxymethyloxiranes in 30 and 13% yields, respectively. The dissociation constants for the beta-adrenergic receptor were measured in membrane preparations of rat heart and lung. In membranes from the heart (which contain mostly beta 1-adrenergic receptors), the affinities of the derivatives and parent compounds were similar. By contrast, in membranes from the lung (which contain mostly beta 2-adrenergic receptors), the derivatives were more potent than the parent compounds. Thus, the cardioselectivities of the p-aminobenzyl analogues 8a and 8b were about one-sixth those of the respective parents.

Adrenergic beta-Antagonists

Aromatic trivalent arsenicals: covalent yet reversible reagents for the agonist binding site of nicotinic receptors.

The agonist binding site of nicotinic acetylcholine receptors (AChRs) includes a disulfide bond that is easily reduced with dithiothreitol to a pair of thiols, and can be then either reoxidized with dithiobis(nitrobenzoic acid) (DTNB) or irreversibly alkylated with bromoacetylcholine (BAC). Aromatic trivalent arsenicals form stable complexes with pairs of appropriately-spaced thiols, but not single thiols. Furthermore, once complexed in proteins, trivalent arsenicals can be removed with dimercaptans, such as 2,3-dimercaptopropanesulfonic acid (DMPS). In an effort to develop reagents that will covalently, yet reversibly label AChRs, we investigated the effects of two model arsenicals, p-aminophenyldichloroarsine (APA) and 4-bromoacetyl-aminophenylarsenoxide (BAPA) on two types of nicotinic receptors: AChRs from Torpedo electroplax and neuronal receptors from chick retina. APA and BAPA significantly decrease the number of 125I-alpha-bungarotoxin binding sites in reduced Torpedo AChRs. Furthermore, arsenylation of neuronal and Torpedo receptors with APA or BAPA (1) prevents reoxidation with DTNB, (2) is reversible with DMPS, and (3) protects against irreversible alkylation by BAC. In Torpedo receptors, the EC50 of protection against BAC alkylation with APA or BAPA is approximately 30 nM. APA arsenylation of Torpedo receptors persists up to 20 h, but can be reversed at any time with DMPS. These results suggest that heterobifunctional arsenicals could anchor labeling groups in the agonist binding site in order to map the agonist binding site, quantitate receptors, or purify and reconstitute functional receptors.

Acetylcholinesterase

Effects of oxidizing and reducing analogs of acetylcholine on neuronal nicotinic receptors.

The synthesis and pharmacological characterization of dithiobisacetylcholine and dithiobis-N,N-dimethyl-4-acetylpiperazinium (two oxidizing analogs of acetylcholine), as well as those of their reduced counterparts, are described. Both the oxidizing and reducing analogs stimulate nicotinic receptors in the chick retina and block the binding of 125I-labeled neuronal bungarotoxin to retinal homogenates (IC50 values of 2 x 10(-6) to 6 x 10(-5) M). Both oxidizing compounds reverse the physiological effects of reduction by dithiothreitol on nicotinic function in intact chick retina, when applied for 2 sec (EC50 values of about 10(-5) M). This effect is selective, insofar as neither agent alters the effects of dithiothreitol treatment on receptors for N-methyl-D-aspartate. Reoxidation takes place at the disulfide located near the nicotinic receptor agonist binding site, inasmuch as reoxidation by these agents prevents affinity alkylation by bromoacetylcholine, and occupation by the competitive antagonist d-tubocurarine prevents reoxidation. Unlike thiocholine, a weak agonist with a free sulfhydryl that, paradoxically, is reported to oxidize nicotinic receptors in electroplax, the reduced forms, mercaptoacetylcholine and N,N-dimethylamino-4-mercaptoacetylpiperazinium, have no direct redox effects on retinal receptors, but they do protect the receptors against reduction by dithiothreitol.

Acetylcholine

Late-onset congenital adrenal hyperplasia in a group of hyperandrogenic women.

The aim of this study was to determine the prevalence of late-onset congenital adrenal hyperplasia (LOCAH) in a group of hyperandrogenic women presenting with menstrual disturbances and/or infertility. Thirty-five women were evaluated by basal hormonal profiles and underwent ACTH stimulation testing. In this study, 17.1% of women showed evidence of partial 21-OH deficiency (21-OHD), and 5.7% 3 beta-HSD deficiency. Neither basal hormonal levels nor clinical characteristics distinguished women with LOCAH from other hyperandrogenic women. And although the mean basal 17-OH progesterone (17-OHP) level in women with 21-OHD (152 +/- 66 ng/dl) was significantly higher than levels in other hirsute women, 4 of 6 (67%) women with 21-OHD had normal 17-OHP levels. Thus, to identify all affected individuals with partial 21-OHD, our data suggest that hyperandrogenic women with basal unsuppressed 17-OHP levels greater than 100 ng/dl should undergo dynamic testing. With regard to partial 3 beta-HSD deficiency, basal DHEA-S levels greater than the 95th percentile of other hirsute women may be used to screen for this deficiency. In conclusion, LOCAH due to partial steroid enzyme deficiencies are a frequent occurrence in women who present with symptoms of hyperandrogenism and ACTH stimulation remains an important tool in making the diagnosis of enzyme deficiencies.

Adrenal Hyperplasia, Congenital

Computer analysis of etiology and pregnancy rate in 636 cases of primary infertility.

Since the rate of pregnancy is a function of time, conventional pregnancy rates (number of patients achieving pregnancy per number of patients treated) are inadequate for counseling unless the follow-up period is specified. To overcome this problem, the expectancy of pregnancy for 636 cases of primary infertility was calculated with the assumption that the patients were followed up indefinitely. The overall "conventional" pregnancy rate was 38%, whereas the overall expectancy of pregnancy was 64%. Endometriosis was found to be the most common factor, comprising 25% of the cases, with a pregnancy rate of 31% and an expectancy of 52%. The expectancy of future pregnancy in a patient who has not achieved pregnancy by a given time is presented for each etiologic factor. This paper also presents a comparison of expectancies of pregnancy by different treatments, which may be helpful in selecting appropriate therapy.

Adult

Hormonal considerations in early normal pregnancy and blighted ovum syndrome.

The hormonal profiles of six pregnancies which terminated in miscarriage with the blighted ovum syndrome have been studied and compared with those of a group of patients similarly studied who had clinically normal pregnancies terminating in live birth at term. The serum chorionic gonadotropin (HCG) values were below normal or at the lowest limit of normal in five of six patients. Three patients had progesterone values within 1 SD of the normal, with normal serum estradiol values. It was concluded that the hormonal profile of early pregnancy is characterized by rising serum HCG and estradiol levels and a declining serum progesterone level from the 5th to the 8th week. The theoretical explanation for the dichotomy seems to be that the fetal adrenal anlagen, even at this early embryonic stage, can produce steroid precursors which are aromatized to estradiol. The production of progesterone, however, does not seem to be possible. Abnormal serum estradiol levels strongly suggest the absence of fetal development and a blighted ovum. However, no single hormonal level will distinguish between blighted ovum and potentially salvagable threatened abortion.

Abortion, Spontaneous

Gestational outcome of clomiphene-related conceptions.

The experience of the gynecologic endocrinology and infertility clinic at The Johns Hopkins Hospital has been subjected to a nonconcurrent prospective analysis in an attempt to evaluate the gestational fate of clomiphene-related conceptions (study series, n = 86). This latter series was contrasted with a series of pregnancies following bilateral ovarian wedge resection (BOWR) (n = 51) in a comparative analysis of gestational outcome event rates. Post-therapy follow-up was available for varying time spans of up to 15 years. A 12.8% twinning rate constituted the single most important complication of clomiphene therapy, resulting in measurable increments in perinatal morbidity and mortality rates. The observation of a 26.5% spontaneous abortion rate would seem to suggest that clomiphene-related conceptions are at little or more risk for spontaneous abortion than would have been expected from the infertile population under discussion. A 3.1% incidence of post-clomiphene birth defects was not increased as compared with commonly quoted rates for the population at large. The corresponding incidence rates of twinning, spontaneous abortion, and birth defects for the BOWR series were 0%, 21.6%, and 0%, respectively.

Abortion, Incomplete

Endocrine features of an adrenal-like tumor of the ovary.

Detailed in vivo endocrine studies of a virilizing adrenal-like tumor of the ovary are reported. A nonspecific steroidogenic baseline profile was observed in the face of clearcut histological adrenal-like features. Dynamic testing disclosed incomplete dexamethasone-related steroidogenic suppression suggestive of partial tumoral autonomy. At the same time, the observation of sizable dexamethasone-related steroidogenic decrements, unaccounted for by normal adrenal contribution, may be interpreted to suggest a direct, pharmacological, inhibitory effect of dexamethasone on tumoral and/or ovarian stromal steroidogenesis. These observations confirm the limited role of baseline steroid profiling and dynamic testing in the preoperative localization of androgen-secreting tumors. Reversible dysfunction of hypothalamic gonadotropic feedback mechanisms was suggested by combined single dose LRH-clomiphene testing. Complete resolution followed tumor removal.

17-Hydroxycorticosteroids

An attenuated form of congenital virilizing adrenal hyperplasia.

The families of two patients with hirsutism and oligomenorrhea were studied with iv ACTH (Cortrosyn) stimulation. The parents responded with a combined incremental rise of progesterone and 17-hydroxyprogesterone greater than 6.5 ng/dl.min, a response seen in the heterozygote parents of patients with congenital virilizing adrenal hyperplasia (CVAH). One sibling in one family responded as do heterozygous subjects, while two other siblings responded normally. The abnormal response of the two pairs of parents and the sibling along with the pattern of steroid secretion observed in the propositi suggest that the so-called adult-onset CVAH is a mild form of the homozygous state of CVAH.

Adolescent

Endometrial pathology and estrogens.

Endometrial biopsies were obtained from 46 hypogonadal patients (44 with gonadal dysgenesis; 2 panhypopituitary) who were receiving estrogen-progestogen therapy. Endometrial abnormalities occurred only in patients receiving a total lifetime conjugated estrogen dose of greater than or equal to 2500 mg and who had received estrogen treatment for a period longer than 4.2 years. The biopsy outcome was significantly related (P less than 0.01) to the estrogen dose at time of biopsy and to the total lifetime dose (P less than 0.05). The progestational drugs administered did not protect against development of endometrial abnormalities. None of the abnormal endometrial patterns were associated with abnormal vaginal bleeding.

Adolescent

Early detection of human chorionic gonadotropin in urine by simple immunoassays.

Data are presented to show that human chorionic gonadotropin (hCG) in urine can be detected early in pregnancy by simple immunoassays performed prior to or around the time of the expected but missed menstrual period. Differences between the use of simple immunoassays with urine and radioimmunoassays and radioreceptor assays with serum are discussed.

Chorionic Gonadotropin

Phosphorus-nitrogen compounds. 22. Synthesis and antitumor activity of arylsulfonylhydrazone analogues.

A series of pyridine-2-carboxaldehyde N-oxide and pyridine-2-carboxaldehyde (thio)phosphoric hydrazones and two cupric chelates was synthesized. The hydrazones, chelates, and combinations of hydrazones and cupric chloride were tested against mice bearing P388 lymphocytic leukemia, Sarcoma 180, or Ehrlich carcinoma ascites cells. The effects of various structural modifications of the hydrazones on antineoplastic activity for this latter system were determined. In general, the pyridine-2-carboxaldehyde thiophosphoric monohydrazones containing P-phenyl or P-phenoxy substituents possessed the highest activity when concurrently administered with cupric ion, whereas the ligands themselves were inactive. Two of the compounds were prepared with P-hydroxyl groups to permit increased hydrophilicity. The ability of the hydrazones to chelate cupric, ferrous, and cobaltous salts was investigated, and discrepancies between determined and calculated log P values for three compounds are discussed.

Animals

Stimulation of follicular growth with "pure" FSH in patients with anovulation and elevated LH levels.

Ovarian follicular growth was stimulated in 4 patients with clomiphene resistant polycystic ovary (PCO)-like disease, with "pure" human pituitary FSH during 10 cycles. In 8 cycles additional hCG was given to induce ovulation. Serum LH and FSH and plasma estradiol and progesterone were determined daily. From the response patterns of steroidogenesis, four functional stages of follicular development can be distinguished, each subsequent stage being characterized by an increasing spontaneous estradiol production and a decreasing capacity to produce extra estradiol in response to stimulation with additional hCG. It is concluded that the unpredictable response to gonadotropin stimulation of patients with PCO-like disease is due to the varying state of development of the follicles at the start of the stimulation. The suggestion is made, based on the different responses in 3 cycles of the same patient (EW), that the lack of positive estrogen feedback in patients with PCO-like disease may be a hyposensitivity to estrogens rather than an absolute insensitivity.

Anovulation

Prognosis in primary amenorrhea.

A retrospective review of 62 patients with primary amenorrhea indicates that thoughtful clinical evaluation combined with dynamic hypothalamic-pituitary testing can usually determine the etiology and prognosis. A careful history, consideration of chronologic age/bone age/height relationships, and assay of serum gonadotropins distinguished all patients with medical diseases, ovarian failure, hypogonadotropic hypogonadism, and polycystic ovaries. Luteinizing hormone-releasing hormone (LH-RH) stimulation, sometimes combined with clomiphene therapy and estrogen provocation tests, assisted with prognostic and pathophysiologic evaluation, although patient management rarely changed. An age-inappropriate LH-RH test result was helpful in identifying patients with abnormal maturational patterns.

Adolescent

The use of clomiphene citrate.

Two hundred and fifty-five women received clomiphene citrate during a 10-year period. Fifty-six were treated diagnostically and one hundred and ninty-nine therapeutically for infertility. It is concluded that clomiphene may aid in confirming the diagnosis and establishing a prognosis in patients with primary or secondary amenorrhea. The success of clomiphene therapy in patients with anovulatory infertility is related to the etiologic factor responsible for the anovulation, but results can be improved by adjustment of the therapeutic regimen used. In the present series, 48% of the patients who ovulated became pregnant, and 25.3% of the pregnancies were miscarried. Those patients who ovulated spontaneously and became pregnant after having discontinued clomiphene therapy showed only a 10% abortion rate. A review of the results indicates that both the ovulation rate and the pregnancy rate can be improved if adjustments are made in therapy to ensure normal follicular maturation and corpus luteum function.

Abortion, Spontaneous

Studies of pathophysiology in primary amenorrhea.

Patients with primary amenorrhea underwent stimulation with luteinizing hormone releasing hormone (LRH) before and after clomiphene administration to determine the maturational status of the hypothalamic-pituitary-gonadal axis, and to assess the development of feedback control mechanisms. Four patients with clinically recognizable conditions served as models. One patient had panhypopituitarism with undetectable baseline FSH and LH values, no response to LRH or clomiphene, and no detectable feedback control. One patient with a "specific" hypothalamic hypogonadotropism had low baseline gonadotropins with a normal response to LRH and no positive or negative feedback mechanisms reflected by no change in LRH response after clomiphene. A patient with delayed puberty due to low bodyweight had low FSH and LH baseline values and an "immature" response to clomiphene, characterized by lowered baseline gonadotropins and an increased peak LH following LRH stimulation. The fourth patient, with an adolescent polycystic ovarian syndrome, had a high baseline LH, normal FSH, hyperresponse to LRH, and a "mature feedback control" as evidenced by an increase in baseline FSH and LH, and a decrease in the LRH response while on clomiphene. The remaining patients showed inappropriate responses. They are regarded as having defects rather than dysfunctions, and the possible abnormalities are discussed in relation to the clinical findings.

Amenorrhea