The Journal of pediatric endocrinology最新文献_第2页

Thyroid function and serum IGF-1 in children before and after liver transplantation. 肝移植前后儿童甲状腺功能及血清IGF-1的变化。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.343

T Pasqualini, P Fainstein-Day, R Gutman, M Balzaretti, D D'Agostino

{"title":"Thyroid function and serum IGF-1 in children before and after liver transplantation.","authors":"T Pasqualini, P Fainstein-Day, R Gutman, M Balzaretti, D D'Agostino","doi":"10.1515/jpem.1994.7.4.343","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.343","url":null,"abstract":"We report results of serum thyroid hormone and IGF-1 concentrations in 20 children, 1.2 to 13.6 years old, with various degrees of chronic liver dysfunction (CLD), before and after successful orthotopic liver transplantation (OLT). Ten children presented with moderate chronic liver disease (CLD-M) with prothrombin time (PT) > 50% and serum albumin concentration > 3 g/dl; 7 children had severe chronic liver disease (CLD-S) with PT < 50% and serum albumin concentration < 3 g/dl; and 7 children who had received an OLT, who had normal liver function at the time of the study. Four of the latter group were also studied before OLT. Patients with CLD-M had normal mean +/- SD serum levels of total T3 (2.0 +/- 0.7 nmol/l), total T4 (125 +/- 25.9 nmol/l) and fT4 concentrations (16 +/- 2.8 pmol/l). In contrast, children with CLD-S showed a significant decrease in thyroid hormones together with normal basal TSH values (T3 0.8 +/- 0.0 nmol/l; T4 45.6 +/- 19.5 nmol/l; fT4 7.4 +/- 1.1 pmol/l; TSH 3.8 +/- 0.9 mU/l). Patients who received a successful OLT showed mean peripheral thyroid hormone concentrations significantly higher than CLD-S patients (T3 1.7 +/- 0.7 nmol/l, p < 0.005; T4 92.8 +/- 18.2 nmol/l, p < 0.001; fT4 14.5 +/- 3.1 pmol/l, p < 0.001). A significant correlation was found between thyroid hormone levels and PT or serum albumin. In the nine patients with CLD-M and CLD-S in whom serum IGF-1 concentration was measured, values found (mean +/- SD 0.08 +/- 0.05 U/ml) were below the 95% confidence limit of matched controls.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"343-8"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.343","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737031","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

A case of Laron syndrome diagnosed in Slovenia. 斯洛文尼亚诊断出一例拉伦综合征。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.365

C Krzisnik, A Silbergeld, Z Laron

引用次数: 4

Hypothalamic dysfunction at ideal body weight simulating anorexia nervosa. 理想体重下丘脑功能障碍模拟神经性厌食症。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.357

A H Levine, J C Pomeroy, T A Wilson

引用次数: 3

Steroid hormone receptors: activators of gene transcription. 类固醇激素受体:基因转录的激活因子。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.275

A O Brinkmann

{"title":"Steroid hormone receptors: activators of gene transcription.","authors":"A O Brinkmann","doi":"10.1515/jpem.1994.7.4.275","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.4.275","url":null,"abstract":"Over the past three decades, a great deal of evidence has accumulated in favor of the hypothesis that steroid hormones act via regulation of gene expression. The action is mediated by specific nuclear receptor proteins, which belong to a superfamily of ligand-modulated transcription factors that regulate homeostasis, reproduction, development and differentiation. This family includes receptors for steroid hormones, thyroid hormones, hormonal forms of vitamin A and D, peroxisomal activators, and ecdysone. Molecular cloning and structure/function analyses have revealed that all members of the steroid/thyroid hormone/retinoic acid receptor family have a similar functional domain structure: a variable N-terminal region, which is involved in modulation of gene expression; a short well-conserved DNA-binding domain, which is crucial for recognition of specific DNA sequences and for receptor dimerization; and a partially conserved C-terminal ligand-binding domain, which is important for hormone binding and also for receptor dimerization and transactivation. In contrast to other members of the receptor superfamily steroid hormone receptors form transient complexes with several heat shock proteins. This interaction promotes proper folding and stability of the receptor molecule. Hormone binding induces a conformational change in the receptor molecule and simultaneously a dissociation of all heat shock proteins, which results in DNA-binding of the hormone-receptor complex.(ABSTRACT TRUNCATED AT 250 WORDS)","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 4","pages":"275-82"},"PeriodicalIF":0.0,"publicationDate":"1994-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.4.275","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18737794","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: 26

Amyloid goiter in a child with familial Mediterranean fever. 家族性地中海热患儿的淀粉样甲状腺肿。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.371

C J Mache, J Schwingshandl, E Ring, A Pfleger, M H Borkenstein

引用次数: 11

Both glucagon excess and insulin deficiency characterize maturity-onset diabetes mellitus of youth (MODY). 胰高血糖素过量和胰岛素缺乏是青年型糖尿病(MODY)的特征。

The Journal of pediatric endocrinology Pub Date : 1994-10-01 DOI: 10.1515/jpem.1994.7.4.335

T W AvRuskin, V Obilessetty, M Jabbar, V Prasad, E Greenfield, F Greig, C S Juan

引用次数: 4

Decreased T3 and T4 levels following topical application of povidone-iodine in premature neonates. 早产儿局部应用聚维酮碘后T3和T4水平降低。

The Journal of pediatric endocrinology Pub Date : 1994-07-01 DOI: 10.1515/jpem.1994.7.3.205

T W AvRuskin, E Greenfield, V Prasad, F Greig, C S Juan

引用次数: 14

Clinical and/or biochemical pancreatitis in diabetic ketoacidosis. 糖尿病酮症酸中毒的临床和/或生化胰腺炎。

The Journal of pediatric endocrinology Pub Date : 1994-07-01 DOI: 10.1515/jpem.1994.7.3.261

A H Slyper, D T Wyatt, C W Brown

引用次数: 17

Prenatal diagnosis and treatment of congenital adrenal hyperplasia. 先天性肾上腺增生症的产前诊断与治疗。

The Journal of pediatric endocrinology Pub Date : 1994-07-01 DOI: 10.1515/jpem.1994.7.3.193

L S Levine, S Pang

{"title":"Prenatal diagnosis and treatment of congenital adrenal hyperplasia.","authors":"L S Levine, S Pang","doi":"10.1515/jpem.1994.7.3.193","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.3.193","url":null,"abstract":"Advances in technology have made possible the prenatal diagnosis and treatment of female fetuses with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hormonal measurement of 17-hydroxyprogesterone, androstenedione, testosterone and 21-deoxycortisol and HLA typing and DNA analysis for 21-OH/C4/HLA class I and II genes in chorionic villus cells and amniocytes are utilized for prenatal diagnosis. Maternal dexamethasone administration begun in the first trimester has prevented or ameliorated virilization in approximately three-fourths of infants. Maternal estriol levels appear to be the most accurate measure of fetal adrenal suppression. Maternal side effects are not infrequent and include excess weight gain, edema, glucose intolerance, hypertension and gastrointestinal problems. Severe permanent striae have been reported. Although no complications of prenatal treatment in the treated fetus or child have been reported long-term follow-up with careful neuropsychologic evaluation is not yet available and is necessary to fully evaluate possible long-term side-effects of prenatal dexamethasone treatment.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 3","pages":"193-200"},"PeriodicalIF":0.0,"publicationDate":"1994-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.3.193","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18820616","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: 17

Gonadotrophin-independent precocious puberty and dyschondrosteosis. 性腺激素非依赖性性性早熟和软骨发育障碍。

The Journal of pediatric endocrinology Pub Date : 1994-07-01 DOI: 10.1515/jpem.1994.7.3.265

L Patel, P E Clayton, M Super, D A Price

引用次数: 2