The Journal of pediatric endocrinology最新文献

Long-term therapy with a single daily subcutaneous dose of growth hormone releasing hormone (1-29) in prepubertal growth hormone deficient children. Venezuelan Collaborative Study Group. 青春期前生长激素缺乏儿童每日皮下单剂量生长激素释放激素(1-29)的长期治疗。委内瑞拉合作研究小组。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.303

R Lanes, E Carrillo

{"title":"Long-term therapy with a single daily subcutaneous dose of growth hormone releasing hormone (1-29) in prepubertal growth hormone deficient children. Venezuelan Collaborative Study Group.","authors":"R Lanes, E Carrillo","doi":"10.1515/jpem.1994.7.4.303","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.303","url":null,"abstract":"As part of a multicenter study to evaluate the efficacy and safety of one daily subcutaneous dose of 30 micrograms/kg of GHRH, 16 prepubertal GH-deficient children with a mean chronological age of 9.0 +/- 2.3 years were treated for 12 to 24 months. After six months of therapy 11 children (68.7%) were considered good responders in that their growth velocity increased by greater than 2 cm/yr over baseline and were continued on GHRH, while five subjects (31.3%) were regarded as poor responders and switched to recombinant hGH for the following six months. Growth velocity increased significantly in responders from a baseline of 3.4 +/- 0.7 cm/yr (mean +/- SD) to 6.8 +/- 0.1 cm/yr, 6.2 +/- 0.9 cm/yr, 6.6 +/- 1.0 cm/yr and 6.5 +/- 0.7 cm/yr at 6, 12, 18 and 24 months respectively. Bone ages advanced by an amount equivalent to the months of treatment. GHRH antibodies were detected in 4/11 and 6/11 responders at six and 12 months of treatment and in 2/5 non-responders at six months, but seemed not to interfere with growth. No side effects or changes in glucose and lipid levels were noted during therapy. These results suggest that GHRH (1-29) at the dose and schedule used is generally effective in the treatment of GH deficiency.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"303-8"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.303","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737797","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 7

A randomized trial of a somatostatin analog for preserving beta cell function in children with insulin dependent diabetes mellitus. 一项生长抑素类似物在胰岛素依赖型糖尿病儿童中保护β细胞功能的随机试验。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.331

J A Grunt, H al-Hakim, L Willoughby, C P Howard

引用次数: 12

HLA haplotypes and hormonal studies in 25 Italian families of patients with classical and non-classical 21-OH deficiency. 25个意大利经典和非经典21-OH缺乏症患者家庭的HLA单倍型和激素研究

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.349

S Einaudi, I Borelli, R Lala, L Praticŏ, E S Curtoni, C De Sanctis

{"title":"HLA haplotypes and hormonal studies in 25 Italian families of patients with classical and non-classical 21-OH deficiency.","authors":"S Einaudi, I Borelli, R Lala, L Praticŏ, E S Curtoni, C De Sanctis","doi":"10.1515/jpem.1994.7.4.349","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.349","url":null,"abstract":"To investigate the genetic polymorphisms of the HLA region and the molecular defect of the P450c21B gene in congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, we studied 89 individuals from 25 families of CAH patients (14 classical forms, 11 non-classical forms). The following immunogenetic and hormonal investigations were performed: HLA-A and B typing, restriction fragment length polymorphism (RFLP) analysis of 21-hydroxylase A and B genes, and serum 17-OH-progesterone values determined basally and 60 min after ACTH stimulation. In the patients affected by the classical form, RFLP analysis revealed 5 deletions and 1 gene conversion in 6 haplotypes and no molecular defect in the others, who probably carry point mutations. In the patients with non-classical form we found P450c21A duplication in 11/18 haplotypes; 9 of the 11 patients shared the HLA-B14 allele. Utilizing both hormonal and genetic data we identified two cryptic forms; hormonal data alone failed to differentiate heterozygous from normal individuals.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"349-55"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.349","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The role of TRH-stimulated prolactin responses in distinguishing gonadotropin deficiency from constitutional delayed puberty. trh刺激的催乳素反应在区分促性腺激素缺乏症和体质性青春期延迟中的作用。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.325

A Büyükgebiz, S Oktay

{"title":"The role of TRH-stimulated prolactin responses in distinguishing gonadotropin deficiency from constitutional delayed puberty.","authors":"A Büyükgebiz, S Oktay","doi":"10.1515/jpem.1994.7.4.325","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.325","url":null,"abstract":"LHRH and TRH tests were performed in 16 boys age 14.5-18.5 years with constitutional delayed puberty (CDP); 9 idiopathic hypogonadotropic hypogonadism (IHH) males, age 15.0-22.0 years; and 7 control subjects age 14.5-19.5 years. The responses of FSH and LH to LHRH stimulation overlapped so that it was difficult to differentiate IHH from CDP. Some patients with IHH had normal gonadotropin responses. Basal PRL levels were in the normal range in control, IHH, and CDP patients. We found a 6 to 25 times increment or a response of more than 22 ng/ml (normal response) with respect to basal levels in the control group. In the CDP group, we found 2 to 19 fold increments in basal PRL levels after TRH stimulation and the maximum response was more than 22 ng/ml in all the patients. In the IHH group, the increment in basal PRL levels was 2-9 times more after the TRH test and the maximum PRL response was more than 22 ng/ml in 6 of the 9 patients. Although the mean peak responses of PRL to TRH were significantly lower in the IHH group compared to CDP and controls (p < 0.001), the mean peak individual responses in all groups were in the normal range (minimum 2-fold increment) and the responses were more than 22 ng/ml in all cases except 3 of 9 patients in the IHH group. We conclude that PRL response to TRH may help in differentiating CDP from IHH if a \"cut off\" response level of 22 ng/ml is used.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"325-30"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.325","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Hirschsprung's disease associated with isolated familial medullary carcinoma of the thyroid. 巨结肠病与孤立的家族性甲状腺髓样癌相关。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.373

Y Rakover, M Dharan, R Luboshitsky

引用次数: 7

Priming with GHRH (1-29) NH2: an aid in differential diagnosis between hypothalamic and pituitary deficiencies. 启动与GHRH (1-29) NH2:在下丘脑和垂体缺陷的鉴别诊断的帮助。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.309

G Bueno, M Bueno, J M Garagorri, G Juste, J Rejas, I Alvarez

{"title":"Priming with GHRH (1-29) NH2: an aid in differential diagnosis between hypothalamic and pituitary deficiencies.","authors":"G Bueno, M Bueno, J M Garagorri, G Juste, J Rejas, I Alvarez","doi":"10.1515/jpem.1994.7.4.309","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.309","url":null,"abstract":"More than 80% of children with growth hormone deficiency (GHD) respond with a rise in growth hormone levels when given 1 microgram/kg body weight of growth hormone-releasing hormone (GHRH) in an i.v. bolus. We conducted a study to determine whether the failure of the remaining 20% to respond to GHRH is due to a pituitary deficiency or a secondary effect associated with chronically understimulated somatotrophs. We administered GHRH to \"prime\" 16 short-statured children (> 2 SD) presenting delayed growth (< 4 cm/year), who had not responded initially when given a single dose of GHRH. Priming consisted of administering GHRH (1-29) NH2 (5 micrograms/kg body weight, s.c.) for six consecutive days. Plasma GH response was studied again after an i.v. injection of 1 microgram/kg body weight of GHRH (1-29) NH2 on the seventh morning. On the basis of these results we were able to separate our patients into two groups: a) responders to priming (n = 8), whose GH responses to pharmacological and acute GHRH tests were < 10 ng/ml, with a 12-hour sleep secretion < 3 ng/ml/min. Priming increased the plasma GH response to acute GHRH in all the children in this group (6.0 +/- 2.1 ng/ml to 18.0 +/- 5.4 ng/ml; p < 0.001); b) non-responders to priming (n = 8), whose GH responses to pharmacological and acute GHRH tests were also < 10 ng/ml, with 12-hour sleep secretion < 3 ng/ml/min, but in whom priming with GH did not increase the plasma GH response (5.5 +/- 2.8 ng/ml to 6.2 +/- 2.9 ng/ml; p = NS).(ABSTRACT TRUNCATED AT 250 WORDS)","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"309-16"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.309","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737026","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Growth hormone (GH)-deficiency in adults: clinical features and effects of GH substitution. 成人生长激素(GH)缺乏症:临床特征和GH替代的效果。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.283

J O Jørgensen, J Møller, T Wolthers, N Vahl, A Juul, N E Skakkebaek, J S Christiansen

引用次数: 27

Epidermal growth factor receptor and human fetoplacental development. 表皮生长因子受体与人胎胎盘发育。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.295

D Evain-Brion, E Alsat

引用次数: 24

True precocious puberty associated with phenylketonuria. 真性性早熟与苯丙酮尿症有关。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.361

B Büyükgebiz, Y Eroğlu, A Büy ukgebiz

引用次数: 5

The new highly sensitive adrenocorticotropin assay improves detection of patients with partial adrenocorticotropin deficiency in a short-term metyrapone test. 新的高灵敏度促肾上腺皮质激素试验提高了短期美替拉酮试验中部分促肾上腺皮质激素缺乏症患者的检测。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.317

P S Thornton, C A Alter, L E Katz, D A Gruccio, P J Winyard, T Moshang

{"title":"The new highly sensitive adrenocorticotropin assay improves detection of patients with partial adrenocorticotropin deficiency in a short-term metyrapone test.","authors":"P S Thornton, C A Alter, L E Katz, D A Gruccio, P J Winyard, T Moshang","doi":"10.1515/jpem.1994.7.4.317","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.317","url":null,"abstract":"This study was designed to evaluate a short term metyrapone test using a highly sensitive (HS) IRMA ACTH assay and to evaluate the usefulness of a morning ACTH level as a screening test for partial ACTH deficiency. ACTH, 11-deoxycortisol and cortisol levels were evaluated over four hours in the morning after a single 40 mg/kg oral dose of metyrapone was administered at 0800 hours. 26 control children and 32 possibly pituitary deficient patients were evaluated. Based on 11-deoxycortisol levels alone, 17 of the patients passed the test, 11 patients failed the test and the result was inconclusive in four patients (12.5%). Evaluation of the increase in ACTH levels (delta ACTH) following metyrapone identified three of the above four with partial ACTH deficiency. The delta ACTH was consistent with the 11-deoxycortisol results in the remainder of patients. There was no difference in morning ACTH levels between controls and patients with partial ACTH deficiency. The measurement of ACTH using the HS IRMA assay, increases the sensitivity of the metyrapone test in detecting patients with partial ACTH deficiency. This test may be used safely in pediatric patients on a repetitive basis, especially in those children who may have progressive ACTH failure following hypothalamic-pituitary irradiation.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"317-24"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.317","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6