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Biomedical subjects

J Herrera

Publications and source records attributed to J Herrera.

At least 127 records · Page 7Linked to original sources

Normal ovarian function in a mild form of late-onset 3 beta-hydroxysteroid dehydrogenase deficiency.

Late-onset 3 beta-hydroxysteroid dehydrogenase (HSD) deficiency was diagnosed in a 30-year-old woman with hirsutism and normal menstrual cycles. No genital abnormalities were present. Elevated basal serum levels of delta 5-3 beta-hydroxysteroids were demonstrated. Serum pregnenolone (P5) was 3.0 ng/ml, and dehydroepiandrosterone sulphate (DHEA-S) 3245 ng/ml. Basal serum levels of delta 4 steroids were low or within normal limits. Serum progesterone (P) was 0.5 ng/ml, 17 alpha-hydroxyprogesterone (17-OHP) 0.2 ng/ml, androstenedione (delta 4A) 0.4 ng/ml, and testosterone (T) 0.1 ng/ml. All delta 5/delta 4 steroid ratios were elevated. Dexamethasone (DEX) administration normalized the elevated levels of delta 5-3 beta-hydroxysteroids, whereas delta 4-3-ketometabolites exhibited only minor modifications. The DHEA-S/delta 4A ratio increased more than five times over the basal ratio, and P5/P and 17 alpha-hydroxypregnenolone (17-OHP5)/17-OHP ratios did not increase after adrenocorticotrophic hormone ACTH stimulation. Studies of basal ovarian function revealed 17 beta-estradiol (E2) and gonadotropins within normal limits according to the menstrual cycle. In the follicular phase, follicle-stimulating hormone (FSH) was 101.3 ng/ml, luteinizing hormone (LH) 46.0 ng/ml, and E2 49.7 pg/ml; in the luteal phase, FSH was 180.0 ng/ml, LH 69.3 ng/ml, and E2 50.1 pg/ml. The presence of ovulatory cycles was documented on the basis of the biphasic pattern of the basal body temperature cycles and the increment in P levels. This case demonstrates the existence of normal ovulatory function in a woman with late-onset of a mild form of HSD.

3-Hydroxysteroid Dehydrogenases↗

Effect of molindone on weight change in hospitalized schizophrenic patients.

A retrospective review was conducted to assess the effect of molindone on weight. Monthly weights and neuroleptic dosages during the first three months of psychiatric hospitalization were compared between matched groups of patients receiving molindone, a combination of molindone and other neuroleptics, or other neuroleptic drugs. We found no significant differences in weight gain among the three groups. Our results challenge the common opinion that molindone can be associated with a lack of weight gain compared to other antipsychotics.

Adult↗

A new inherited variant of the 3 beta-hydroxysteroid dehydrogenase-isomerase deficiency syndrome: evidence for the existence of two isoenzymes.

The clinical and endocrine features of a unique form of adrenal insufficiency secondary to an inherited deficiency of 3 beta-hydroxysteroid dehydrogenase-isomerase (3-HSD) were studied. The propositus was a 19-yr-old man with a history of repeated episodes of acute adrenal crisis. Family study disclosed that a 6-yr-old female sibling also was affected, and a third sibling had died during the course of an adrenal crisis. The diagnosis of adrenal insufficiency was established on the basis of extremely low serum cortisol levels and urinary 17-hydroxycorticosteroid excretion with concomitantly elevated serum ACTH levels and lack of cortisol response to ACTH administration. Impairment of C-21 steroid 3-HSD activity was strongly suggested by persistency elevated serum 17-hydroxypregnenolone to 17-hydroxyprogesterone and pregnenolone to progesterone ratios, their significant increase after ACTH administration, and their return to normal during cortisol therapy in both patients. Nevertheless, the serum dehydroepiandrosterone to androstenedione ratio, both basally and after ACTH and/or hCG stimulation, was normal. These findings coupled with the normal phenotypic development and onset of puberty in the two patients indicated intact C-19 steroid 3-HSD activity. The overall results indicate an inherited impairment of 3-HSD activity confined only to C-21 steroid substrates and, thus, suggest the existence of at least two 3-HSD isoenzymes under independent genetic regulation.

3-Hydroxysteroid Dehydrogenases↗

Pursed lips breathing training using ear oximetry.

Pursed lips breathing (PLB) training is often used in the management of patients with chronic obstructive lung disease (COLD). Previous clinical studies have demonstrated that PLB improves arterial oxygen saturation (SaO2) and CO2 removal as well as relieving dyspnea. Twelve hypoxemic subjects with stable COLD were randomly assigned to either the pursed lips (P) or control group consisting of general relaxation (R). The SaO2 was monitored via ear oximetry, and respiratory rate and tidal volume were monitored using a strain gage transducer and the minute volume was calculated. The PLB was taught by an experienced instructor using the ear oximeter as a monitoring display with a goal toward increasing SaO2. The subject was taught general relaxation (Rlx) with the aid of pleasant music. We compared PLB and Rlx treatments using an A-B-A crossover study design. In both groups, PLB significantly improved SaO2 over baseline (p less than 0.001) whereas Rlx did not. We conclude that patients can learn to increase their SaO2 by PLB using ear oximetry adjunctively.

Aged↗

Low plasma zinc and androgen in insulin-dependent diabetes mellitus.

Plasma zinc and pituitary and testicular hormone concentrations were measured in two groups of male adolescents. One group comprised insulin-dependent diabetes mellitus patients, aged 14-19 years; the other, as control, included 12 healthy youngsters aged 13-19 years. Plasma concentration of zinc, prolactin, testosterone, and dihydrotestosterone were lower in diabetics than in controls, whereas the ratios of androstenedione and androstenedione to testosterone + dihydrotestosterone were higher. Plasma FSH and LH were normal. These results suggest a diminished conversion of androstenedione to testosterone and relate zinc with the 17-beta-hydroxysteroid dehydrogenase enzyme activity.

Adolescent↗

Response to acute protein load in kidney donors and in apparently normal postacute glomerulonephritis patients: evidence for glomerular hyperfiltration.

25 kidney donors who had undergone nephrectomy 1-11 years ago, 35 patients followed up for 13 years after poststreptococcal glomerulonephritis, and 44 controls were investigated for their capacity to increase their glomerular filtration rate after an acute oral load of 100-150 g of protein. Their mean baseline creatinine clearances (Ccrl, ml/min +/- SEM) were similar (controls 108.5 +/- 6.1; kidney donors 115.4 +/- 8.54; postacute nephritis 82.0 +/- 6.45), but the postmeal filtration rates (Ccr2) were significantly (p less than 0.05) lower in the two patient groups (kidney donors 137.4 +/- 11.60; postacute nephritis 90.3 +/- 5.30) than in the controls (161.5 +/- 9.39), as was the Ccr2/Ccr1 ratio (p less than 0.01, controls 1.58 +/- SEM 0.10; kidney donors 1.20 +/- 0.07; postacute nephritis 1.18 +/- 0.08). In normal individuals the degree of change was inversely related to the initial creatinine clearance and varied from 135.6% +/- 43.0 when Ccrl was less than 70 ml/min to 32.7% +/- 9.50 when Ccrl was 130 ml/min or higher. This relative response was decreased in kidney donors and postacute nephritis patients. Kidney donors and apparently normal postacute nephritis patients thus have diminished renal reserve capacity.

Acute Disease↗

Gastrointestinal aluminium absorption: is it modulated by the iron-absorptive mechanism?

Gastrointestinal aluminium (A1) absorption has been proved but its mechanism is still unknown. This study investigates the pattern of A1 absorption in patients with different degrees of iron stores. We studied 29 haemodialysis patients forming three groups according to their serum ferritin values. Over seven days all patients received the same dose of aluminium hydroxide after which patients with 'low-normal' and normal serum ferritin increased their serum A1 proportionally with the increased aluminium hydroxide intake. By contrast patients with high serum ferritin did not show any change in their serum A1 values. Our results therefore suggest that a 'common pathway' of metal absorption could be implicated in A1 absorption. Serum ferritin might be a valuable predictor of different behaviour.

Adult↗

Assessing the benefit of changing aluminium hydroxide schedule on anaemia and serum phosphorus control.

Convincing evidence exists concerning aluminium hydroxide (A1 (OH)3) absorption and risk of toxicity. Over recent years our aim has been to reduce exposure to this risk. In this study we evaluated the effect of changing our A1 (OH)3 prescription policy, reducing its intake by stopping the breakfast dose, separating the iron intake from the binder's influence, and tailoring the A1 (OH)3 dose according to the protein intake patterns. The change was done gradually, initially in a pilot group and then in the whole unit. The results from the pilot group, who completed two years follow-up and from the whole unit, when more patients adhered to the new scheme, were similar. After the A1 (OH)3 reduction serum phosphorus did not change, haemoglobin increased and the blood transfusion requirements decreased. These results support our preliminary findings that A1 (OH)3 might interfere with erythropoiesis and stress the necessity of reassessing the prescription of binders thoroughly aiming to give adequate individual doses according to the different protein intake patterns.

Aluminum Hydroxide↗

Modifications in the testis steroidogenic pathways in rats fed with cottonseed flour.

Suppression of the testicular T production by effect of the CSF has been previously reported. Changes in the testicular content of intermediates and precursors of T biosynthesis were determined in rats fed during 28 days with diets containing 25,35 and 50% of CSF and compared with a defated cottonseed flour diet group and a control Purina-chow diet group. Also described were modifications during a six wk post CSF diet period. The results showed a diminution in pregnenolone and progesterone testicular content to a third and a half of the control values (21.5 and 19.3 pg/mg of protein respectively). Other compounds in the delta 5 pathway, 17 hidroxypregnenolone and dehydroepiandrosterone were undetectable since the 35% CSF diet, the 17 hidroxyprogesterone diminished to undetectable values only with the maximal (50%) CSF content diet. During the post CSF diet period all the steroids showed a tendency toward the control values. The 25% CSF content diet group reached the initial contents; other groups presented different recovery degrees. The most affected was the 50% diet group with a direct effect of the CSF on the testicular androgen steroidogenesis affecting both the delta 4 and the delta 5 pathways.

17-alpha-Hydroxypregnenolone↗

Fungal endocarditis caused by Arnium leporinum following cardiac surgery.

A case of fungal endocarditis caused by the coprophilous ascomycete Arnium leporinum is reported in a 19-year-old male living in Colombia. The fungus was seen in tissue and recovered in pure culture from peripheral emboli, a mitral valve, pus from an osteolytic lesion, and kidney. The patient died 21 months after cardiac surgery. The range of A. leporinum in the Americas is expanded to include Colombia.

Adult↗

Hepatic injury during doxorubicin therapy.

Six patients with acute lymphoblastic leukemia manifested liver dysfunction related to doxorubicin hydrochloride therapy. Other causes, eg, infection, hepatitis, posttransfusion reaction, and leukemic infiltration were ruled out. There was close correlation between the administration of doxorubicin and the appearance of hepatic dysfunction. Doxorubicin may produce an idiosyncratic reaction and must be considered a drug with potential liver toxicity.

Adolescent↗

Echocardiographic changes after successful renal transplantation in young nondiabetic patients.

Eighteen young nondiabetic patients with chronic renal failure were studied by M-mode echocardiography before and three to 67 weeks after a successful renal transplant. Left ventricular mass (LVM), cardiac output (CO), and stroke work, which were increased before the operation, decreased afterward, in some cases to normal values. Both regression of the LVM and normalization of CO were detected as early as three weeks postoperatively and probably resulted from changes in the end-diastolic volume, mean systemic blood pressure, and hematocrit as a consequence of normal renal function. Because all the patients had normal left ventricular function and only moderate dilatation of the left ventricle, it is not known whether these striking beneficial changes after SRT also will occur in patients with significant dilatation or dysfunction of the left ventricle.

Adult↗

Hypergonadotrophic hypogonadism in an XX female subject due to 17,20 steroid desmolase deficiency.

A 22 year old XX female patient with primary amenorrhoea and sexual infantilism was studied. Persistently elevated serum LH and FSH concentrations and exaggerated LRH pituitary responsiveness indicated deficient ovarian hormonal production. Serum levels of C21 and C19 steroids measured by specific radioimmunoassays before and after appropriate stimulations demonstrated an impairment of adrenal and ovarian steroid biosynthesis. Baseline levels of androstenedione (delta 4-A), testosterone (T), and oestradiol-17 beta (E2) were persistently below the normal range for healthy women at early follicular phase, whereas progesterone (P) and 17 alpha-OH-progesterone (17-OH-P) serum levels were significantly higher than those observed for normal women. Adrenal and gonadal stimulation with ATCH and hCG, respectively, resulted in a considerable rise in serum P and 17-OH-P without any significant change in circulating levels of delta 4-A, T, and E2. These findings were consistent with the diagnosis of 17,20 steroid desmolase deficiency at both adrenal and ovarian levels. This is the first report of a 17,20 desmolase deficiency in an XX individual, and is in line with previous suggestions that familial occurrence of the disorder would fit an autosomal recessive pattern of inheritance.

Adrenal Cortex Hormones↗

Cottonseed flour effects on androgen testicular content and serum levels in rats.

Natural gossypol present in the whole cottonseed flour (CSF) has clear antifertility properties, decreasing the number and motility of spermatozoa. In this study the endocrine function of the testes was evaluated, measuring by specific radioimmunoassays the testicular content and the serum levels of androstenedione (A), testosterone (T), and 5 alpha-dihydrotestosterone in male rats fed with different and nontoxic concentrations of CSF along with Purina chow for 4 weeks, followed by 6 weeks of a recovery period. The testicular content of A, T, and DHT decreased 63%, 90%, and 38%, respectively, in comparison with their control. The serum levels of the three androgens diminished as follows: A 74%, T 95%, and DHT 60%. All the steroids measured in the recovery period showed a tendency towards the control values in both testicular content and serum. The most affected steroid was testosterone, which, after the recovery period, did not reach the control values. A also decreased, suggesting a blockade in testicular steroidogenesis caused by the initial diet.

Androgens↗

Influence of aluminium hydroxide intake on haemoglobin concentrations and blood transfusion requirements in haemodialysis patients.

To investigate the likely influence of aluminium hydroxide intake (Al(OH)3) on haemoglobin concentrations and blood transfusion requirements we studied 27 long-term haemodialysis patients for 24 months divided in two equal periods: (P I and P II). All patients received oral iron as a fasting single dose, intravenous iron being used only occasionally. During P I Al(OH)3 was given thrice daily, during P II Al(OH)3 was reduced significantly by stopping the breakfast dose, thereby separating the oral iron from the influence of the binder. After Al(OH)3 reduction haemoglobin increased, the requirement for blood transfusion decreased, and the need for intravenous iron also decreased. Serum phosphorous did not change. This suggests that Al(OH)3 might interfere with erythropoiesis and we consider it advisable to avoid the morning dose of Al(OH)3 which in many cases is not necessary.

Aluminum Hydroxide↗

Morphophysiological correlation of boar Leydig cell development during postnatal stage.

To analyze the possible correlation between steroidogenic activity of the Leydig cells and their morphology and ultrastructure in the boar testes at different ages, testes from six Yorkshire boars were obtained by orchidectomy at 8,14,28,40,70,78,86,94 and 100 days of age. Using representative sections of testicular parenchyma, light as well as transmission electron microscopic studies were performed. Homogenized, albuginea-free tissue was further used for quantitative estimation of total proteins and metabolic pools of androstenedione (A), testosterone (T) and 5 alpha-dihydrotestosterone (DHT). Under light microscopy, abundant hyperplastic Leydig cells were observed from 14 to 40 days, thereafter was a decrease in their size and number. Spermatogenesis remained in the spermatogonia stage while the tubules increased in diameter until age 80 days; from 90 days on, pachytene primary spermatocytes could be found. A well developed smooth endoplasmic reticulum was observed in Leydig cells from the 8th postnatal day. Metabolic pools from age 8 to 28 days showed a decrease for T and A from 4.33 to 1.78 and 2.51 to 0.48 microgram/mg protein respectively, maintaining thereafter the same levels throughout the age-span studied. However, DHT was found at low levels from the beginning of neonatal period (from 0.07 to 0.38 microgram/mg protein).

Aging↗