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Biomedical subjects

J Adams

Publications and source records attributed to J Adams.

At least 361 records · Page 20Linked to original sources

Familial polycystic ovaries: a genetic disease?

High resolution ultrasonography was used to establish the presence of polycystic ovaries (PCO) in 50 women with symptoms of polycystic ovary syndrome and in 17 women with congenital adrenal hyperplasia. One hundred and thirty-seven post-menarcheal, premenopausal female members of the families of these patients were scanned to assess the heredity of the condition. Familial PCO was found in 56 of the 61 pedigrees (92%) in which sufficient members were available for study. The frequency of PCO in the relatives of the patients with congenital adrenal hyperplasia was no different from that found in the main group. Twenty-four out of thirty-six (67%) mothers of probands and 45 out of 52 (87%) sisters of probands were affected. The segregation ratio (fraction of females affected) for all sibships was 107 out of 133 (80.5%). The volumes of the polycystic ovaries (mean 9.97 ml, 95% confidence limits (CL) +/- 0.75) were significantly different from those of the normal ovaries (mean 5.38 ml, 95% CL +/- 0.26) (P less than 0.0001), although there was no significant difference between the volumes of the ovaries of the probands and those of their affected relatives. Even after allowing for a high frequency of PCO in the general population (22%), the observed segregation ratios were significantly different from those predicted for autosomal dominant (P less than 10(-4)) and X-linked dominant (P = 0.0002) modes of inheritance. A number of mechanisms which might account for the observed segregation ratio are considered. These include meiotic drive due to a genetic segregation distorted, vertical transmission of an infective agent, and environmental factors, such as the effect of maternal androgen on gonadal development.

Female↗

Reduced frequency of luteinizing hormone pulses in women with weight loss-related amenorrhoea and multifollicular ovaries.

We have studied pulsatile secretion of LH in 10 women with secondary amenorrhoea and multifollicular ovaries (MFO). This group of patients have a history of mild to moderate, or partially recovered weight loss. They have normal basal LH concentrations but evidence of oestrogen deficiency suggesting a hypothalamic abnormality of gonadotrophin regulation. The results of gonadotrophin pulse analysis were compared with those in normal women during the early follicular phase of the cycle. The mean LH concentration during the 8 h study (5.0 +/- 0.9 [SD] U/l) was not significantly different from that in normal women (5.7 +/- 2.5). There was no difference between the groups in mean LH pulse amplitude (2.1 +/- 0.5 in MFO; 2.2 +/- 1.3 in normal women). The frequency of LH pulses was, however, significantly lower in women with MFO (2.8 +/- 1.6 vs 4.8 +/- 1.5, P less than 0.05). Two women with MFO had LH pulses of normal frequency. One subsequently developed a normal pattern of ovarian follicles. The other showed a sleep-related rise in LH concentrations during a 24 h profile which was similar to the pattern of gonadotrophin secretion normally observed during late puberty. These results show that women with MFO have a hypothalamic disturbance of gonadotrophin regulation with slowing of LH pulses without a diminution of pulse amplitude.

Amenorrhea↗

Synergism of pulsatile LHRH therapy with oral clomiphene treatment.

Eighteen anovulatory patients who were resistant to induction of ovulation with clomiphene and with subcutaneous pulsatile LHRH were treated with these two agents given simultaneously. Twelve of the 14 patients with polycystic ovary syndrome, 1 patient with weight-related amenorrhea and 1 of 3 patients with intrinsic pituitary disease responded to the combined treatment. Serial determinations of serum gonadotropin concentrations showed that these remained unchanged by clomiphene treatment, suggesting a direct action on the ovary. For induction of ovulation for in vivo fertilization, the combination of oral clomiphene with subcutaneous pulsatile LHRH is worth trying before proceeding to intravenous LHRH or hMG therapy.

Administration, Oral↗

Evolution of polycystic ovaries in a girl with delayed menarche. A case report.

A 17-year-old girl had delayed growth secondary to low birth weight. Although she entered puberty at 12.5 years, she had delayed menarche. She underwent serial ovarian ultrasound scans, and a change in ovarian morphology was observed, from a normal multicystic appearance to one typical of polycystic ovaries (PCOs). We hypothesize that normal ovarian morphology may change to PCOs during the peripubertal period because of a change in the endocrine milieu.

Adolescent↗

Induction of puberty by pulsatile gonadotropin releasing hormone.

15 girls and 17 boys with delayed or arrested puberty were treated with gonadotropin releasing hormone (GnRH) for a mean of 1.04 years. GnRH was administered subcutaneously in a pulsatile fashion at 90 min intervals, and the dose was increased as required to maintain progression of puberty, initially only at night and subsequently over 24 h. Initial GnRH dose was 1-2 micrograms per pulse in the girls and 2-4 micrograms per pulse in the boys. The effect of treatment was monitored by serial overnight gonadotropin profiles in all patients and with pelvic ultrasound in the girls. The clinical features, growth acceleration, endocrinology, and ovarian ultrasound morphology of puberty were those seen in normal children. Measurement of spontaneous gonadotropin pulsatility after treatment had been discontinued allowed the distinction between 20 patients with hypogonadotropic hypogonadism and 12 who had constitutional delay of growth and puberty. 2 girls and 6 boys did not respond to the treatment regimen. These findings indicate that normal puberty is GnRH dependent.

Adolescent↗

Correction of hypoprothrombinemia by immunosuppressive treatment of the lupus anticoagulant-hypoprothrombinemia syndrome.

A 66-year-old man with the lupus anticoagulant-hypoprothrombinemia syndrome was treated with cyclophosphamide and prednisone to correct a factor II deficiency prior to elective major surgery. Whereas the lupus anticoagulant activity persisted, functional factor II levels normalized and he underwent surgery without a bleeding diathesis. Immunosuppressive therapy may temporarily normalize factor II levels in patients with the lupus anticoagulant-hypoprothrombinemia syndrome and reduce the risk of excessive hemorrhage. The disparate response of the lupus anticoagulant and hypoprothrombinemia to immunosuppression suggests that the lupus anticoagulant did not directly cause the hypoprothrombinemia.

Aged↗

Hypergonadotropic amenorrhea--etiology and outcome in 93 young women.

Ninety-three cases of amenorrheic women aged 40 years or less in whom serum FSH concentrations were found to be greater than 20 units/l have been reviewed. The etiology and diagnosis of this uncommon but serious condition and the potential for spontaneous recovery of ovarian follicular activity (17.1%) are discussed. Management is reviewed in the light of ultrasonic, karyotypic and immunological findings.

Adult↗

The evolution of polycystic ovaries in a girl with hypogonadotropic hypogonadism before puberty and during puberty induced with pulsatile gonadotropin-releasing hormone.

We describe a girl of 12.7 years who had GnRH deficiency after surgery and radiotherapy for a craniopharyngioma at age 5 years. She had large cystic ovaries with a small uterus in the absence of spontaneous gonadotropin pulsatility. Puberty was induced by low-dose pulsatile GnRH treatment over a 1.5-year period. In the perimenarcheal period, ovarian morphology became typical of PCO with abnormal diurnal pulsatile gonadotropin secretion. These data suggest that PCO is primarily a disorder of the ovary and that the observed gonadotropin abnormalities are secondary to the ovarian lesion.

Child↗

Evolution of Escherichia coli during growth in a constant environment.

Populations of Escherichia coli, initiated with a single clone and maintained for long periods in glucose-limited continuous culture, developed extensive polymorphisms. In one population, examined after 765 generations, two majority and two minority types were identified. Stable mixed populations were reestablished from the isolated strains. Factors involved in the development of this polymorphism included differences in the maximum specific growth rate and in the transport of glucose, and excretion of metabolites by some clones which were utilized by minority clones.

Adaptation, Physiological↗

Amenorrhoea in cystic fibrosis.

Menstrual history in relation to nutritional status and lung function was assessed in 45 adolescent and adult patients with cystic fibrosis (CF). Twenty patients had regular menstrual cycles (of whom six had been pregnant), 12 had primary or secondary amenorrhoea, 11 had irregular cycles and two were premenarcheal. Patients with regular menstruation had a significantly higher body mass index and percentage body fat (estimated from skinfold thickness measurements) than those with amenorrhoea (P less than 0.001), and also better lung function (P less than 0.01). A linear discriminant analysis indicated that of the four indices studied percentage body fat was the most important predictor of menstrual function. Ovarian and uterine morphology were studied by ultrasonography in 28 patients and 17 healthy controls. In 10 patients multicystic ovaries were noted. Even in patients with regular cycles uterine size was reduced (P less than 0.01) and in those with amenorrhoea appeared to be related to thinness. Amenorrhoea in CF is an appropriate adaptive response in preventing pregnancy, but the resultant oestrogen deficiency and psychological effects must be considered.

Adolescent↗

The distribution of oestradiol in plasma in relation to uterine cross-sectional area in women with polycystic or multifollicular ovaries.

The uterine cross-sectional area (UXA) of women with polycystic (PCO) or multifollicular ovaries (MFO) is significantly larger and smaller, respectively, than those of normal women during the early-mid-follicular phase of the menstrual cycle. In the present study the distribution of oestradiol in plasma from normal women and women with PCO or MFO was measured to determine if differences in the available fractions of oestradiol could account for the differences in UXA of women with PCO or MFO. No differences in plasma levels of oestradiol were detected and the concentrations of oestradiol present in a free state or bound to albumin were similar in normal women and women with PCO or MFO. The concentration of oestrone was significantly higher in plasma from women with PCO (516 +/- 120 pmol/l, mean +/- SD) than in plasma from women with MFO (389 +/- 91 pmol/l) or normal women (376 +/- 89 pmol/l). Differences in UXA for women with PCO or MFO as compared with normal women cannot therefore be attributed to differences in available oestradiol concentrations. It is possible that abnormalities in oestrogen metabolism within uterine or other tissues may account for the UXA of women with PCO or MFO. Increased plasma oestrone levels in women with PCO may provide more substrate for conversion to oestradiol within the uterus whilst the smaller UXA of women with MFO may reflect both lack of normal cyclical increases of oestradiol and formation of biologically inactive oestradiol metabolites.

Body Constitution↗

Role of extracellular iron in the action of the quinone antibiotic streptonigrin: mechanisms of killing and resistance of Neisseria gonorrhoeae.

The quinone antibiotic streptonigrin is believed to kill bacteria by promoting formation of oxygen radicals. This antibiotic has also been used to select resistant bacterial mutants, some of which vary in iron utilization. We examined the effects of streptonigrin on Neisseria gonorrhoeae and several types of gonococcal mutants. Streptonigrin (0.025 microgram/ml) efficiently killed gonococcal strain FA1090, and this effect depended on iron. Streptonigrin-resistant mutant FA6271 had normal iron uptake but was moderately deficient in total iron. Resistance most likely resulted from failure of FA6271 to divert electrons to streptonigrin, as demonstrated by a reduction in KCN-insensitive respiration (a hallmark of the action of quinones) and superoxide formation. Other mutants selected for inability to use human iron-binding proteins (strains FA6273 and FA6275) had no increase in streptonigrin MIC and no decrease in KCN-insensitive respiration. Mutants did not demonstrate an increase in superoxide dismutase or catalase. Streptonigrin killing of gonococci depended on a reaction(s) in which extracellular iron was important, presumably because iron was required for catalysis of hydroxyl radical. The results suggest that a membrane component may be a target for the actions of streptonigrin.

Catalase↗

Association of adverse perinatal events with an empty sella turcica in children with growth hormone deficiency.

High-resolution computed tomography (HR-CT) of the hypothalamo-pituitary region was performed in 26 consecutive children presenting with growth hormone deficiency (GHD) at one clinic. 58% had an empty sella turcica (ES) and 42% a full sella turcica (FS). There was no difference between the ES and FS groups for mean (+/- 95% confidence limits) presentation age (ES 6.7 (+/- 1.8) years, FS 5.6 (+/- 2.2) years), height standard deviation score (SDS) (ES -3.9 (+/- 0.8), FS -3.3 (+/- 0.5] nor head circumference SDS (ES -1.9 (+/- 1.1), FS -0.7 (+/- 1.1]. There were significant associations between the ES group and a history of adverse perinatal events (p less than 0.001) and multiple pituitary deficiency (p = 0.014). Growth hormone response to an acute growth hormone releasing factor test showed no association with HR-CT diagnosis. Sella turcica volumes were calculated from the HR-CT scans. All sella volumes were small; mean SDS for height was -2.6 (+/- 0.2). There was no difference in sella volume SDS between the ES and FS groups (ES -2.9 (+/- 0.3), FS -2.5 (+/- 0.4]. Adverse perinatal events may cause an ES and GHD by compromising the blood supply to the pituitary gland or infundibulum.

Birth Injuries↗