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1.
Baseline somatomedin activity in seven of eight patients with Turner's syndrome was found to be within normal limits. Somatomedin activity readily suppressed with oestrogen administration. The overall mean serum somatomedin activity during oestrogen therapy (0.87 U/ml, SD 0.15) was significantly lower (P less than 0.005) than the mean of the control serum somatomedin activities (1.09 U/ml, SD 0.24). During oestrogen therapy, suggestive elevations of fasting growth hormone levels were noted in five of eight patients. The data indicate that oestrogenic suppression of serum somatomedin was not due to decreased growth hormone secretion and suggest the existence of a negative feedback link between somatomedin and growth hormone.  相似文献   

2.
Three children with hypothalamic mass lesions and continued linear growth in the absence of circulating growth hormone are presented. Serum growth hormone levels were either undetectable or very low both basally and following insulin-induced hypoglycaemia, whereas serum somatomedin levels were normal. Two subjects had elevated serum levels of prolactin, and in one of these prolactin suppression with bromoergocryptine failed to influence either somatomedin levels or urinary excretion of nitrogen, hydroxyproline and calcium. Although only one case was studied in detail these data suggest that maintenance of serum somatomedin and of normal growth in these children was not dependent upon prolactin secretion.  相似文献   

3.
Plasma protein C (PC) activity, prothrombin time (PT), partial thromboplastin time (PTT) and platelet count were studied in 23 children with nephrotic syndrome (NS) and AT III activity was determined in 15 children in the same group. All parameters were compared with those obtained in a group of 16 age matched healthy controls. Mean plasma AT III activity was significantly reduced in patients with NS (P < 0.001) correspondingly, plasma AT III levels were found to be directly correlated with serum albumin and inversely correlated with proteinuria. In contrast, mean plasma PC activity, as well as PT, PTT and platelet count were similar in the NS group when compared with the control group and in addition no remarkable difference was found in the mean plasma PC activity between the steroid sensitive and resistant NS groups. In conclusion, this study demonstrated acquired AT III deficiency and normal PC levels in childhood NS. These data suggest that although plasma AT III activity depends on the severity of NS, neither the severity of NS nor the underlying renal disease is an important factor determining the changes of PC activity in childhood NS.  相似文献   

4.
In 4 children with celiac disease, aged 7 months to 11 years, serum somatomedin activities (SMA) were consistently low: less than 0.40 U/ml (N greater than 0.50 U/ml). Basal plasma growth hormone (GH) levels were not elevated and increased normally under arginine-insulin stimulation in 3 patients. Human GH administration at a dosage which usually determines an increase of serum SMA in children with GH deficiency (4 mg/day/2 days) did not modify significantly the low serum SMA. However, in 1 child a clear-cut increase of serum SMA (0.22-0.82 U/ml) was noted under a higher dosage of human GH (8 mg/day/2 days). In 3 patients serum SMA was studied 3 weeks to 4 months after starting the gluten-free diet and was found to be normal. A limitation of the somatomedin generation unrelated to a deficit in GH secretion and probably resistant to GH appears therefore to be present in celiac disease. The rapid normalization of serum SMA under gluten-free diet suggests that the low serum SMA is induced through some unknown hormonal or metabolic signal by the protein malabsorption and/or the nutritional deficiency present in celiac disease.  相似文献   

5.
OBJECTIVE: To investigate the effect of a therapeutic diet on serum biotin levels and to explain the seborrheic dermatitis in phenylketonuric (PKU) patients on a "loose" diet. DESIGN: Forty-seven patients were divided into two groups: group A (n=21) demonstrated good compliance to a special diet and group B (n=26) were on a "loose" diet. Most of the patients in group B (20/26), who suffered from mild seborrheic dermatitis, were requested to return to phenylalanine (Phe)-restricted diet for at least 15 days. Seventy-nine healthy children of comparable age were used as controls. Biotin serum levels and plasma biotinidase activity were measured in patients as well as controls. In addition, biotinidase activity was evaluated in vitro after incubation with various concentrations of Phe. RESULTS: Biotin levels in group A patients (636+/-118 ng/L) were statistically significantly elevated (P < 0.01) compared with those of group B patients before (412+/-184 ng/L) and after (501+/-160 ng/L) 15 days on a Phe-restricted diet, as well as with those of controls (337+/-290 ng/L). Furthermore, biotinidase activities were decreased in group B patients (4.2+/-1.68 nmol/min/L) compared with those of group A patients (6.4+/-0.7 nmol/min/L) and controls (6.10+/-0.8 nmol/min/L). Additionally, biotinidase activities in the patients of group B were restored to normal (5.78+/-0.81 nmol/min/L), with a simultaneous remission of their skin lesions, after 15 days on a Phe-restricted diet. Moreover, the in vitro findings showed a 51% inhibition of biotinidase activity when incubated with Phe (20 mg/dL). CONCLUSIONS: It is suggested that the high biotin levels in group A patients reflect the intake of water-soluble biotin of vegetable origin. In contrast, the low biotinidase activity in group B patients may be attributed to their high Phe plasma levels, which acts as an enzyme inhibitor, as shown by the in vivo and in vitro results. Consequently, the observed seborrheic dermatitis in PKU children (group B) is associated with an impairment of biotin recycling.  相似文献   

6.
INTRODUCTION: Recently it has been revealed that Obstructive Sleep Apnea Syndrome has an influence on the endocrine system, especially to the secretion of growth hormone (GH). It was previously reported that secretion of GH has a circadian rhythm along with a correlation with slow wave sleep. Normally in a pediatric patient, after 90 min of sleep the brain wave shows patterns of deep sleep, and a matching peak in GH secretion is observed. We have studied the effect of GH secretion in sleep breathing disorder of pediatric patients by observing the pre- and post-operative difference in GH secretion in children with tonsillar hypertrophy who had tonsillectomies. MATERIAL AND METHODS: Ten pediatric patients who complained of sleep apnea or hypopnea, and were highly suspected of having sleep breathing disorders due to tonsil hypertrophy and underwent surgery were chosen. Pre and post-operative blood somatomedin C and urinary growth hormone levels were measured. In 6 of the 10 patients blood GH levels were measured after overnight collection of blood every 30 min through a catheter placed in the vein. METHOD OF ANALYSIS: Integral blood GH calculated from GH levels of the blood samples taken every 30 min was used to express the efficacy of GH secretion during sleep, and was compared with blood somatomedin C and urinary GH levels. Pre- and post-operative somatomedin C and urinary GH measurements were compared. RESULT: 1) In 6 of the 10 patients, there was a positive correlation with integral GH and urinary GH, and no correlation with integral GH or somatomedin C. 2) Post-operatively, 7 of the 10 patients had increased urinary GH levels, 1 showed no change and 2 had decreased levels. There was a significant increase in the mean urinary GH levels from 18.6 +/- 16.1 pg/ml pre-operatively to 35.8 +/- 19.6 pg/ml post-operatively (P < 0.05). 3) Eight of the 10 patients had a post-operative increase in somatomedin C levels, 1 showed no change and the other patient had a decreased level. There was a significant increase in mean somatomedin C levels from 175.9 +/- 124.8 ng/ml pre-operatively to 226.6 +/- 134.3 ng/ml post-operatively (P < 0.01). CASE: An 8-year-old-boy was diagnosed as having habitual tonsillitis and suspected sleep breathing disorder, and was admitted to our hospital for a tonsillectomy. Apnea and hypopnea were observed during sleep in the hospital. After receiving his parents' consent, GH secretion was evaluated before and after the tonsillectomy. Integrated GH measurements, somatomedin C and urinary GH increased significantly after the operation. CONCLUSION: In this study, the improvement of sleep breathing disorder by surgery was associated with a positive effect to the GH secretion. GH secretion is important for growth of a child. Wen the cause of sleep breathing disorder is evident in the upper airway system, active treatment and cure by surgery is advised. Measurement of urinary GH in the morning is useful and simple for evaluating the ability to secrete GH.  相似文献   

7.
We measured morning plasma concentrations of delta sleep-inducing-peptide-like-immunoreactivity (DSIP-LI) in 9 sleep apnea patients, 10 narcolepsy patients, and 11 normal controls. Comparisons between the three groups showed no significant differences, although there was a trend toward association with low levels of DSIP-LI in the narcoleptic group, particularly in patients not using medications. No differences were found in the morning or evening plasma DSIP-LI levels in a second group of 11 normal controls and 8 sleep apneics. Our findings do not appear to support a biological marker role of disease activity for single measures of plasma DSIP in sleep apnea.  相似文献   

8.
A study was initiated to determine whether hypersecretion of growth hormone (HGH) was involved in the inhibition of normal glucose metabolism in pregnant insulin-independent diabetic (IID). A total of 25 patients was studied, of whom 13 had normal glucose tolerance and 12 were recently diagnosed IIDs. Hgh secretion was stimulated by insulin-induced hypoglycemia. The mean basal HGH levels and the plasma HGH pattern following the insulin stimulus were similar in the study group and the normal controls. Although it is unlikely that HGH participates to any significant degree in the diabetogenicity of normal and diabetic pregnancy, final proof depends on the assessment of the biologically active inhibitory polypeptide fraction of HGH and the growth-promoting protein, somatomedin.  相似文献   

9.
Plasma somatomedin activity was low in rats fed a vitamin B6 deficient diet for 4 weeks. The reduction appears to be in the fraction which is not growth hormone dependent. Plasma growth hormone levels were unaltered while plasma insulin levels were significantly reduced.  相似文献   

10.
The Netherlands has well-organized school health services, and children are assessed on a regular basis for scoliosis among other disturbances and pathologies. The purpose of this study was to assess the benefits of an annual screening programme for scoliosis in the Netherlands. Three cohorts of 10,000 children sampled at 10, 12 and 14 years of age, respectively, were followed for 3 years. Children with a positive bending sign were referred to a second screening stage, in which external asymmetry was quantified. Children diagnosed via the programme (group 1) were compared with those children who had been referred for treatment independently of the screening (group 2). The total number of children in these groups combined was then compared with the number that would have been expected on the basis of accepted prevalence figures for idiopathic scoliosis given in current literature. Over 30,000 children were screened. Although the programme established a total of 57 cases of definite scoliosis (0.18%), the 34 cases (0.11%) already known, mainly detected by previous school health checks, were more severe regarding the risk of progression and treatment. The annual screening programme did not detect a single case that needed surgery. These figures provide the basis on which to decide for or against adopting an annual screening programme for scoliosis; the decision is a socio-political one. Based on this study, we expect all scoliotic patients needing treatment should be detected in time if periodic health checks will be maintained biennially. On medical grounds, it is our view, that screening for scoliosis should not be performed in the Netherlands annually.  相似文献   

11.
Human growth hormone (HGH) and somatomedin (Sm) concentrations have been studied in a group of newborns. Plasma HGH values were 41.17 +/- 24.26 (SD) ng/ml (14.00-90.00 ng/ml) and the Sm value was 0.59 +/- 0.43 (SD) U/ml (0.18-1.8 U/ml); the difference between these values and the ones observed in normal adults (2.45 +/- 2.53 (SD) ng/ml and 1.16 +/- 0.28 (SD) U/ml respectively) were statistically significant. While growth hormone values were higher than in normal adult controls, somatomedin was significantly decreased. It is possible that the dissociation between human growth hormone and somatomedin in newborn could reflect a reduced biosynthesis of the somatomedin-generating system and consequently a lack of a feed-back control on GH exerted by somatomedin.  相似文献   

12.
Estimates of prekallikrein levels in plasma specimens from patients with migraine and from healthy individuals were obtained by determining the benzoyl-arginine ethyl ester (BAEe) esterase activities developed on activation with kaolin, as suggested by Costerase level- 'n the patients and in the control material, and kinetic data provided no evidence of a difference in inhibitor levels. Only very low BAEe esterase activity was registered in samples of cerebrospinal fluid obtained from the patients and no significant difference between attacks and free intervals was detected. When citrated EDTA-treated plasma was activated with acetone-incubated normal plasma containing prekallikrein activator (factor XIIf), no significant difference in BAEe esterase activity was noticed between plasma from the patients and that from the control persons. When, however, citrated plasma without EDTA was used, a significantly higher peak level of esterase activity was registered in the patient plasma. This observation might suggest the presence of a factor positioned between active factor XII and prekallikrein, and present in higher amounts in plasma from patients with migraine than in healthy individuals.  相似文献   

13.
R LeBlanc  H Labelle  F Forest  B Poitras 《Canadian Metallurgical Quarterly》1998,23(10):1109-15; discussion 1115-6
STUDY DESIGN: A prospective and controlled comparative study. OBJECTIVES: To identify variables that would allow discrimination among patients with progressive adolescent idiopathic scoliosis, patients with nonprogressive adolescent idiopathic scoliosis, and control subjects. SUMMARY OF BACKGROUND DATA: In a previous study, the correlation was demonstrated between morphologic somatotypes and adolescent idiopathic scoliosis. METHODS: One hundred forty-six subjects were evaluated anthropometrically and were classified according to their morphologic somatotype. Of these subjects, 52 were adolescent girls with progressive idiopathic scoliosis, whereas 32 girls had nonprogressive idiopathic scoliosis. The control group was composed of 62 healthy adolescent girls. Somatotype values for ectomorphism, mesomorphism, and endomorphism were obtained according to a technique based on Sheldon's method, and 77 anthropometric measurements of segments of the thorax, head, and limbs were taken. RESULTS: The discriminant analysis realized on a subset of 18 variables allowed the correct identification of each subject's group in 84% of the cases. CONCLUSIONS: It is possible to differentiate healthy adolescent subjects, patients with nonprogressive adolescent idiopathic scoliosis, and patients with progressive idiopathic scoliosis by using anthropometric measurements and morphologic classification. These findings may be useful in the early detection of children at risk for progression of scoliosis and may allow earlier application of treatment methods without waiting for a significant increase in the curve.  相似文献   

14.
STUDY DESIGN: A study was done to evaluate the use of voluntary supine side bending radiographs and Risser table traction radiographs in adolescent patients undergoing posterior spinal fusion for idiopathic scoliosis. OBJECTIVES: To compare the usefulness of supine side bending and traction radiographs in assessing curve flexibility and determining fusion levels in patients undergoing posterior spinal fusion for adolescent idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: Supine side bending radiographs have been used in the preoperative evaluation of idiopathic scoliosis to determine curve flexibility and fusion area. Traction films have been used to determine the flexibility of large curves and neuromuscular curves where active side bending is not possible. No study to date has compared the use of these films in patients with adolescent idiopathic scoliosis undergoing surgery. METHODS: Seventy-five patients with more than a 2-year follow-up period after surgery were included in this study. Preoperative radiographs included a standing posteroanterior and lateral film and both supine maximal voluntary side bending films and a traction film done on a Risser table. A preoperative review of these radiographs was done to determine curve flexibility and fusion levels. At follow-up evaluation, the patients were examined for any evidence of decompensation or "adding-on" of levels. RESULTS: For curves less than 60 degrees, side bending radiographs showed greater curve correction than traction radiographs, whereas the opposite was true for curves greater than 60 degrees. For King I and II curves, side bending radiographs were superior for determination of lumbar curve flexibility and for distinguishing these two types of curves. On traction radiographs, the stable vertebra was 1.4 vertebral levels higher than on the standing film. When the fusion level was moved proximally because of the traction radiograph, decompensation or "adding-on" commonly occurred. CONCLUSIONS: Supine bending radiographs are superior to traction radiographs for assessing curve flexibility except for curves more than 60 degrees. The selection of the distal extent of fusion based on the traction radiograph gave a large number of poor results. The selection of fusion levels in adolescent Idiopathic scoliosis is best determined by a combination of standing posteroanterior and lateral radiographs and the supine maximum voluntary bend films.  相似文献   

15.
OBJECTIVE: To assess nucleosome plasma levels in patients with systemic lupus erythematosus (SLE) and to study the correlations with serum antinucleosome, anti-double-stranded DNA (anti-dsDNA), and antihistone antibody activities, as well as with disease activity (by the SLE Disease Activity Index [SLEDAI]). METHODS: In a cross-sectional study, we assessed 58 SLE patients for their plasma nucleosome levels. Plasma nucleosome levels as well as serum antinucleosome, anti-double-stranded DNA, and antihistone antibody activities were assessed by enzyme-linked immunosorbent assay. SLE activity was evaluated using the SLEDAI: RESULTS: The mean (+/-SD) plasma nucleosome concentration in SLE patients was 52 +/- 159 ng/ml (range 5-1,180), and was significantly higher than that of the controls (16 +/- 8.8 ng/ml, range 8-52; P = 0.03). Thirteen of the 58 lupus patients had levels over the range of normal (defined as the control mean + 3 SD, or 42 ng/ml). An inverse correlation was found between nucleosome plasma levels and serum antinucleosome antibody activity in the entire group of SLE patients, those with active disease, and those with inactive disease, respectively. No correlation was found between the SLEDAI and nucleosome plasma concentrations. CONCLUSION: Nucleosome plasma levels may be normal or increased in SLE, and found in patients with active or inactive SLE. Longitudinal studies are needed to further establish whether high levels of circulating nucleosomes may predict the occurrence of an SLE flare.  相似文献   

16.
AIMS/BACKGROUND: Alpha-glutathione S-transferases (alpha-GST) are the cytoplasmatic class of enzymes responsible for cellular detoxifying processes. We evaluated the plasma alpha-GST activity in relation to chronic infection caused by hepatitis C virus (HCV). METHODS: Eighteen anti-HCV-negative healthy subjects (controls), 32 anti-HCV-positive subjects with a constant normality of alanine aminotransferases (ALT) and gamma-glutamyl transpeptidase (gamma-GT) levels ("apparently healthy carriers"), and 85 patients with HCV-related chronic liver disease (40 chronic hepatitis, 27 cirrhosis, and 18 with hepatocellular carcinoma) were studied. We assayed plasma alpha-GST in all subjects upon their entry into the study; and every 6 months for 3 years in the control group and in anti-HCV apparently healthy carriers. RESULTS: Alpha-GST values were significantly higher than normal values in 57% of the 21 HCV-RNA-positive apparently healthy carriers and in none of 11 persistently HCV-RNA-negative subjects; the highest increment of alpha-GST was documented in patients with chronic hepatitis. We did not observe correlation among HCV-RNA, histological activity, gamma-GT and ALT or alpha-GST values. CONCLUSIONS: Therefore, the increment of plasma alpha-GST indicates a liver involvement even when ALT levels are normal. This may be clinically relevant to "apparently healthy carriers" whose plasma alpha-GST values, when increased, might need further evaluation.  相似文献   

17.
Based on a previous report of a zinc deficiency syndrome in children characterized by low hair zinc, anorexia, poor growth, and hypogeusia, 12 children attending a pediatric endocrinology clinic for growth retardation and judged as having short stature as a variant of normal (SVN) were evaluated as to their zinc nutriture to learn whether zinc deficiency was a contributory factor. None was found to have the above syndrome, although one child did have a hair zinc concentration below 70 mug/g. The mean hair zinc of the SVN subjects was lower than the hair zinc of 40 apparently normal adolescents, 131+/-37 mug/g vs. 168+/-44 mug/g (P less than 0.02), but there was no difference found in plasma levels of zinc or in taste acuity. In contrast, five patients with total growth arrest secondary to juvenile Crohn's disease (CD) were found to have multiple findings consistent with zinc deficiency including low plasma zinc, low hair zinc, or hypogeusia.  相似文献   

18.
In a 4 month study, a group of 16 patients with stable renal graft function receiving triple immunosuppressive therapy including cyclosporin A (Cy A) were investigated for the levels of calcium, magnesium and zinc in erythrocytes. The patients were randomized to be converted to the new microemulsion formulation (Sandimmun Neoral) in a 1:1 fashion (n = 8) or to continue with the classical formulation (Sandimmun) (n = 8). The concentrations of creatinine, phosphate, alkaline phosphatase activity, calcium, magnesium and zinc were measured twice a month in blood plasma. The concentration of calcium, magnesium and zinc in erythrocytes was also measured. The concentration of magnesium in blood plasma and erythrocytes during the study showed no deviation from normal values. The level of zinc in erythrocytes was almost twice as high as in normal healthy controls and was not dependent on Cy A formulation. Calcium content in erythrocytes of patients receiving Sandimmun was 27.6% higher than in healthy persons. Conversion of the patients to Sandimmun Neoral normalized the calcium concentration in erythrocytes and caused a transient increase of calcium levels in blood plasma.  相似文献   

19.
Oxidant stress secondary to dopamine metabolism has been proposed as a pathogenic factor in the development of Parkinson's disease. Biochemical abnormalities extending beyond the central nervous system have been identified in patients with this condition. Previous investigators have found abnormally elevated concentrations of the lipid peroxidation product, malondialdehyde, in the plasma and serum of patients with Parkinson's disease. We attempted to replicate these findings but controlled for other factors that could influence malondialdehyde levels. We detected no significant elevations in mean serum malondialdehyde concentrations in either levodopa-treated or untreated patients with Parkinson's disease, compared to normal controls; similarly, no elevation was found in a group of patients with dementia of Alzheimer's type. On the other hand, a group of subjects with diabetes mellitus but no neurodegenerative disease had significantly elevated mean serum malondialdehyde levels, consistent with previous studies of diabetic patients. Autoxidation is one of the two major routes by which dopamine and dopa metabolism may generate oxygen free radicals. We analyzed the autoxidation product of dopa, 5-S-cysteinyl-dopa, in the plasma of these same groups of patients with neurodegenerative disease and normal controls; no significant differences were identified. Serum concentrations of two other antioxidant substances, alpha-tocopherol and uric acid, were also statistically similar in these groups. In conclusion, analysis of several blood products relevant to oxidant stress, including malondialdehyde, 5-S-cysteinyl-dopa, alpha-tocopherol, and uric acid, failed to distinguish patients with Parkinson's disease or dementia of Alzheimer's type from controls.  相似文献   

20.
INTRODUCTION: Valproic acid (VPA) is an antiepileptic drug widely used in paediatrics. In spite of being a safe and effective anticonvulsant, VPA has been involved in the onset of changes in the metabolism of ammonia and carnitine, although few prospective studies have been made of this. OBJECTIVES: To evaluate the effect of long-term VPA administration, particularly on the metabolism of carnitine, ammonia and plasma amino-acids and the possible clinical repercussions of this in a group of epileptic patients studied prospectively and retrospectively. MATERIAL AND METHODS: A study was made of 102 epileptic children on long term anticonvulsant treatment mainly with VPA. These patients were divided into two groups: group I (n = 25) were studied prospectively (basal sample, after one, six and twelve months of treatment) and group II (n = 77) or long term treatment group (a single sample extraction). In each epileptic patient and in 56 children from a control group (group III) studies were made of free plasma carnitine, ammonia and amino-acids related to the urea cycle and the plasma levels of each anticonvulsant drug. RESULTS: It was observed that in group I there was a fall in plasma carnitine concentrations with time and a progressive rise which was statistically significant (p = 0.001) in plasma levels, mainly of ammonia, glutamine, glycine and ornithine, from the basal levels to those after a year of treatment in practically 100% of the children studied. In group II children on antiepileptic drugs, mainly VPA, were seen to have lower plasma carnitine levels than those in the control group and higher serum ammonia, glutamine and glycine levels than the healthy population not treated with anticonvulsants. These differences were statistically significant (p = 0.001). No relationship was found between the parameters studied and the plasma levels of the drug, type of epilepsy or presence of side effects. CONCLUSIONS: These changes show the negative effects of VPA on the metabolism of carnitine and ammonia. It would therefore seem advisable to monitor these parameters in epileptic children on long term antiepileptic treatment.  相似文献   

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