Measurement of urinary porphobilinogen and porphyrins: preliminary data from a pilot QA scheme.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to J H Barth.
Explore the source record for details and available documents.
No standards exist for the evaluation of hair in hirsute women. This study compared the semi-quantitative visual scoring of body hair, using the Ferriman & Gallwey scale, in 88 hirsute women with direct objective measurements of hair shaft diameter and daily linear growth rates of hair growing on the pre-auricular area of the face, the forearm, the anterior abdominal wall and the anterior thigh. There was a significant correlation between the semi-quantitative score and diameter measurements on the forearm, abdominal wall and thigh. There was no relationship between linear growth rates at any of the four sites and the semi-quantitative score. The conclusion of this report is that suitably standardised and controlled semi-quantitative measurement of hair in hirsute women with visual analogue scores would appear to offer information similar to that obtained by direct measurement of hair diameter.
Most hirsute women have polycystic ovaries. The few women who have a more sinister cause for their hirsutism can probably be identified by clinical symptoms and signs. Therefore, the purpose of investigation is to identify those women, and this can probably be best achieved by a first-line measurement of serum testosterone. If this is normal, subsequent investigations are only required to guide the choice and use of any systemic therapy. It is probably unnecessary to measure any endocrine parameters once therapy has been initiated, since there is no relationship between these variables and hair growth.
Explore the source record for details and available documents.
OBJECTIVES: We wished to compare peak and incremental rise in plasma cortisol in response to insulin induced hypoglycaemia (IIH) stress test, i.m. glucagon stimulation test (IMGST) and short Synacthen test (SST) in patients with pituitary disease, using a modern radioimmunoassay for cortisol. We compared the three stimulants using receiver operator characteristic (ROC) plots, assuming a cortisol threshold of 500 nmol/l or 580 nmol/l for the IIH stress test which we used as the standard from which to evaluate the SST and the IMGST. PATIENTS AND DESIGN: We prospectively studied 16 patients (8F, 8M mean age 43.69 +/- 3.72 years) admitted to the investigation ward for IIH stress test and who were asked to undergo two additional tests (IMGST and SST) on consecutive days. MEASUREMENTS: We measured serum cortisol at baseline, 30, 45, 60, 90 and 120 minutes during the IIH stress test; baseline, 150 and 180 minutes during GST, and baseline and 30 minutes during the SST. RESULTS: There was a significant rise in cortisol from baseline in all tests (P < 0.001). There was no significant difference among the peak plasma cortisol responses or the incremental rises in plasma cortisol following IMGST, SST and IIH stress test (repeated measures ANOVA F = 0.704, P = 0.503; F = 0.238, P = 0.79). The ROC plots clearly showed that the SST has poor diagnostic utility at both IIH thresholds, compared with the IMGST. CONCLUSION: The peaks and incremental rises in cortisol following all three tests are comparable. Using the insulin induced hypoglycaemia stress test as a reference and peak cortisol thresholds of 500 and 580 nmol/l as discriminating variables, the short Synacthen displayed poor diagnostic utility when compared to the i.m. glucagon stimulation test. The short Synacthen may be misleading if used as a screening test as advocated by a number of authors.
It has been reported that the relationship between serum total calcium and albumin concentrations in hospital patients deviates from linearity at low albumin concentrations. We searched a large laboratory computer data base for adult patients with discretionary requests for serum calcium analysis but with no other data suggestive of disturbances of calcium homeostasis, and collected a minimum of 100 calcium values at each of a wide range of albumin concentrations. We confirmed the earlier observation, but found that the use of a single regression to derive an adjustment of total calcium for albumin gave only small differences of no clinical significance. To investigate whether equations to adjust total calcium for albumin can be transferred between laboratories, three laboratory computers were searched for calcium requests from patients likely to have a low prevalence of calcium disturbances. The regressions of total calcium on albumin differed significantly between laboratories, but within each laboratory gave adjusted calcium values identical with those in health. Although the errors resulting from applying an equation from one laboratory to another were small and unlikely to be of major clinical significance, we recommend that where possible laboratories should derive adjustment equations from their own data.
A total of 669 women and 609 men were selected from a laboratory computer data base in such a way that they would be expected to have a low prevalence of disturbances of calcium homeostasis but a wide range of serum albumin concentrations. The least squares regression coefficients of total calcium on albumin did not differ between men and women, nor did they differ at different ages. Mean serum albumin-adjusted calcium concentrations did not change with age in men from 1 to 90 years, and values were similar in women aged 1-20 years. However, adjusted calcium concentrations were significantly lower in women aged 21-50 and higher in women aged 61-90 than in younger women and all men. The differences were small and are unlikely to affect clinical interpretation.
We present clinical details of a patient with a 20-yr history of amennorhea, a pituitary tumor, elevated PRL levels, and initially undetectable GH. Bromocriptine failed to fully suppress PRL, and there was no tumor shrinkage. Within 7 months of starting bromocriptine treatment, the patient developed clinical and biochemical signs of acromegaly. At surgery, a stem cell adenoma was excised. The mechanisms by which bromocriptine may have resulted in the development of acromegaly in this patient are discussed.
The relationship between hidradenitis suppurativa (HS) and hyperandrogenism is largely based on the finding of an increased free androgen index due to a low sex hormone binding globulin (SHBG). As SHBG is now believed to be regulated by factors that influence body weight, and previous studies were not controlled for body weight, we have re-evaluated the androgen status of female patients with HS. We have studied the endocrine status of 66 women with HS. Twenty-three had acne, and 23 were significantly obese (body mass index: BMI > 30). There was no relationship between obesity and disease duration. Nineteen of 56 women were hirsute. A premenstrual flare in disease activity was reported by 32 women, but this was not related to menstrual disturbances. No consistent relationship was reported with pregnancy. Eight women with HS were menopausal at presentation, and one developed her disease 6 years after the menopause. Plasma androgens in women with HS were compared with controls matched for BMI and hirsuties. There was no difference between HS and controls. Testosterone and dehydroepiandrosterone sulphate were normal in all subjects with HS. In obese subjects, SHBG was reduced, consistent with BMI-matched controls. We have found no supporting evidence for biochemical hyperandrogenism in women with HS when compared with age-, weight- and hirsuties-matched controls. We report the continuation and primary development of HS in postmenopausal women.
A comparison of three commercially available assays for growth hormone (Pharmacia hGH RIA, IDS Gamma-BCT GH and Delfia 22 kDa hGH) were used to measure growth hormone (GH) secretion in 26 short children after pharmacological stimulation. The IDS Gamma-BCT assay was compared with Pharmacia assay in 15 provocative tests (13 children; n = 94). The Pharmacia assay showed a strong positive proportional bias and this was particularly marked at GH > 20 mU/L; the mean difference between the assays at GH < 10 mU/L was 12%. The IDS Gamma-BCT assay was also compared with the Delfia 22 kDa assay in 18 provocative tests (13 children; n = 100). Results from the IDS assay was higher throughout the range 0-90 mU/L with a mean difference of 11.4% in the range 10-90 mU/L; the mean difference between the assays at GH < 10 mU/L was -39%.
Explore the source record for details and available documents.
Sixty-nine pre-menopausal hirsute women were studied to determine the prevalence of psychological morbidity, and to identify factors associated with psychiatric symptoms. Measures used included: GHQ and POMS to assess psychological morbidity; self-esteem; personality (EPQ); and social adjustment, amongst other. About a third of subjects were found to be GHQ cases, one third had never discussed their hirsutism before, and two thirds avoided some social situations. Psychological morbidity was associated with poor social adjustment, higher levels of neuroticism and introversion, and avoidance of some social situations, but not with the severity of hirsutism or testosterone levels. The results suggest that a proportion of women with hirsutism experiences psychological and social difficulties, and that factors other than their dermatological status play an important part in their psychological difficulties.
A retrospective analysis of patients receiving isotretinoin for acne was performed, in order to determine the necessity for routine testing of lipid profiles and liver function tests during therapy. Data were analysed from 209 individuals, 113 (69 males, 44 females) of whom had been treated with 1 mg/kg/day, and 96 (67 males, 29 females) with 0.5 mg/kg/day. There were no significant changes in any of the tests of liver function. There were significant elevations in both plasma cholesterol and triglycerides at 8 and 16 weeks (P < 0.01) for both dose schedules, which were significant in both male and female subjects (P < 0.001). All the individuals with elevated cholesterol (> 6.5 mmol/l) at 16 weeks had elevated cholesterol at the onset of therapy. Triglyceride concentrations were elevated at 8 weeks, but there was no further increase thereafter. It was not possible to predict which subjects would become hypertriglyceridaemic from pretreatment lipid estimations. In conclusion, there appears to be little evidence to support the previously recommended regular biochemical monitoring of liver function and lipid profiles in patients who are treated with isotretinoin for 16 weeks. It would appear prudent to ensure that there is neither liver disease nor hyperlipidaemia prior to the onset of therapy, and to determine the triglyceride response to therapy on one occasion after 4 weeks' treatment. This change in patient management should result in considerable savings both in patient time and in blood collection and analysis.
OBJECTIVE: The interpretation of dynamic endocrine investigations is dependent on the timing of blood sampled after a stimulus. We have studied the accuracy with which doctors adhered to a prescribed protocol. DESIGN: A new request form for dynamic tests was designed to include both the planned and the actual time of blood sampling in order to allow assessment of the variance of sample timing. MEASUREMENTS: We followed 152 investigation procedures comprising 791 planned specimens. A total number of 627 timing intervals could be analysed of which 366 timing intervals did not differ from the planned interval. Protocols were divided into long and short (greater or less than 6 hours) and into those governed by clock times or 'stopwatch', i.e. minutes elapsed, intervals. RESULTS: A total of 22% specimens were taken more than 10 minutes earlier or later than planned. Six per cent of specimens were taken more than 30 minutes earlier or later than planned. CONCLUSIONS: The data presented in this study suggest that in order to improve the interpretative accuracy of dynamic tests, it would appear prudent to pay more attention to the accuracy of sample timing. New dynamic test publications should include time tolerance for the normal response.
Should hirsute women be investigated? Most only need careful clinical evaluation. First, they need to be examined to determine whether they are hirsute or hypertrichotic, and for the degree of hair growth to assess the most appropriate form of treatment. Second, they need to be clinically evaluated for signs and symptoms of virilism to determine the extent of investigation needed. If virilism is absent laboratory investigation need only be minimal. As most hirsute women will have mild ovarian hyperandrogenism they will only require the appropriate tests for polycystic ovaries, and only those women who are virilised will need intensive investigation. The approach described is considered minimalist by some; but unless a tumour is diagnosed, anti-androgen treatment will only be offered to those with severe hirsutism who want treatment. Anti-androgens will be prescribed because (i) current medical treatment is insufficiently specific to require accurate localisation of the source of excess androgen and (ii) because anti-androgens are more effective at reducing hair growth than hydrocortisone, even in late onset congenital adrenal hyperplasia.
Androgen metabolism was investigated in normal human apocrine glands and in those isolated from age-matched patients with hidradenitis suppurativa. Axillary glands were isolated by shearing and androgen interconverting enzyme activities were measured in cell-free homogenates by incubation with [3H] dehydroepiandrosterone, [3H] androstenedione and [3H] testosterone. The activities (pmol/mg protein/min: mean + SEM) of 3 beta-hydroxysteroid dehydrogenase delta 4-5-isomerase (10.0 +/- 1.2 vs. 5.3 +/- 0.5: n = 5) and 17 beta-hydroxysteroid dehydrogenase (58.1 +/- 4.5 vs. 35.7 +/- 5.2: n = 5) were significantly lower (P less than 0.005) in hidradenitis suppurativa, whereas 5 alpha-reductase activity (12.5 +/- 2.3 vs. 12.5 +/- 2.0: n = 5) was similar. This report suggests that hidradenitis suppurativa cannot be attributed to exaggerated activities of end-organ androgen interconverting enzymes.
OBJECTIVE: The objective was to determine if cyproterone acetate (CPA) therapy for hirsute women demonstrated a dose response. DESIGN: A double-blind dose-ranging study of the effect of 3 doses of cyproterone acetate for a period of 12 months. PATIENTS: Twenty-one hirsute women received the Dianette contraceptive pill (35 micrograms ethinyl oestradiol + 2 mg CPA, Schering Healthcare, UK), 20 received Dianette plus 20 mg CPA and 19 received Dianette plus 100 mg CPA: supplementary CPA was administered on days 1-10 of the birth control pill cycle as described by Hammerstein. MEASUREMENTS: Hair growth was measured using the clinical scale of Ferriman and Gallwey and by direct measurement of hair shaft diameter and linear growth of hair on the face, forearm, abdomen and thigh. RESULTS: Thirty-eight women completed 12 months therapy, eight withdrew due to side-effects and 14 were lost to follow-up. All three dose schedules produced significant reductions in clinical hair growth scores. This reduction was seen after 6 months with Dianette alone (P less than 0.005) and after 3 months with both the higher doses (P less than 0.01). There were no significant differences between the effect of different doses at any of the three-monthly time points. Hair diameter measurements were reduced by all doses after 12 months: face by 27*, 37, 37%*, forearm 5, 10, 8%*, abdomen 22*, 39*, 33%*, thigh 12, 24*, 30%* (median reductions (*P less than 0.01) for Dianette, D + 20 mg CPA and D + 100 mg CPA respectively). There were no significant reductions in daily linear growth rates. A comparison of the percentage reduction in hair shaft diameter at each site demonstrated no significant difference between doses, although the reductions by the three doses on the forearm, abdomen and thigh suggested a trend towards a dose response. CONCLUSIONS: We conclude that cyproterone acetate 2 mg daily appears to be as effective as higher doses in the therapy of hirsute women.