The Journal of pediatric endocrinology最新文献_第5页

Anthropometry in skeletal dysplasia. 骨骼发育不良的人体测量。

The Journal of pediatric endocrinology Pub Date : 1994-04-01 DOI: 10.1515/jpem.1994.7.2.155

N T Hertel, J Müller

{"title":"Anthropometry in skeletal dysplasia.","authors":"N T Hertel, J Müller","doi":"10.1515/jpem.1994.7.2.155","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.2.155","url":null,"abstract":"Skeletal dysplasia or osteochondrodysplasia is the designation of more than 200 different disorders, that are characterized by abnormalities of the skeleton, disproportional short stature, and a variety of other problems. The underlying biochemical defect is unknown in the vast majority of skeletal dysplasias, and the diagnosis is based on radiological findings and anthropometric measurements. Despite this fact, the information on body proportions in even the more common forms of skeletal dysplasia is scarce. Patients with achondroplasia are often diagnosed shortly after birth. Linear growth is severely compromised with relatively short extremities. Head circumference is above normal and final height ranges from 115 to 145 cm in males and 112 to 137 cm in females. Individuals with hypochondroplasia may go unnoticed until puberty, at which time the growth problem becomes obvious. Sitting height to height ratio is increased, but the body disproportion may not be apparent until puberty. Final height has been reported between 118 and 165 cm. Spondyloepiphyseal and spondylometaepiphyseal dysplasias are characterized by severe impairment of growth both in trunk and extremities, and therefore the sitting height to height ratio may be normal. Final height is severely reduced and ranges from 94 to 132 cm. It is concluded that anthropometric studies of patients with skeletal dysplasia are needed. More quantitative information on body proportions may assist in the diagnostic procedure and ensure that growth promoting therapy, e.g. growth hormone, does not worsen the disproportion.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 2","pages":"155-61"},"PeriodicalIF":0.0,"publicationDate":"1994-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.2.155","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19055627","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: 5

The growth hormone/insulin-like growth factor axis in the kidney: aspects in relation to chronic renal failure. 肾中的生长激素/胰岛素样生长因子轴:与慢性肾衰竭有关的方面。

The Journal of pediatric endocrinology Pub Date : 1994-04-01 DOI: 10.1515/jpem.1994.7.2.85

A Flyvbjerg

{"title":"The growth hormone/insulin-like growth factor axis in the kidney: aspects in relation to chronic renal failure.","authors":"A Flyvbjerg","doi":"10.1515/jpem.1994.7.2.85","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.2.85","url":null,"abstract":"Chronic renal failure (CRF) is characterized by a series of compensatory adaptations in the surviving nephrons of the diseased kidney aimed at maintaining glomerular filtration rate and tubular resorptive functions. Several lines of evidence indicate that in normal kidney growth hormone (GH) and insulin-like growth factors (IGFs) modulate the nephron, both in respect to function and size. Virtually all members of the GH/IGF axis are present in the kidney, comprising: 1) GH-receptors; 2) IGF-1 and IGF-2 mRNA; 3) distinct receptors for IGFs: the IGF-1 receptor and the IGF-2/mannose-6-phosphate receptor, and 4) specific binding proteins (IGFBPs), indicating that GH and IGFs may affect the kidney in both an endocrine and autocrine/paracrine fashion. GH and IGFs modulate renal metabolism and the kidney plays an important role in the metabolism and degradation of circulating GH and IGFs. The action of GH to enhance kidney function and size is mediated through IGF-1, and IGF-1 infusion in animals and man stimulates renal function and volume. In addition, renal growth following various pathophysiological conditions (e.g. reduction in renal mass, diabetes mellitus) is preceded by an increase in endogenous renal IGF-1. In CRF circulating levels of GH are elevated, serum IGF-1 is normal and circulating IGFBP-1, -2, and -3 are elevated. Given the ability of GH and IGF-1 to stimulate various functions of the kidney, the potential use of GH or IGF-1 in the setting of CRF has been suggested.(ABSTRACT TRUNCATED AT 250 WORDS)","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 2","pages":"85-92"},"PeriodicalIF":0.0,"publicationDate":"1994-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.2.85","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19055628","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: 12

The use of recombinant human growth hormone in short children with chronic renal failure. 重组人生长激素在矮个子儿童慢性肾功能衰竭中的应用。

The Journal of pediatric endocrinology Pub Date : 1994-04-01 DOI: 10.1515/jpem.1994.7.2.107

O Mehls, B Tönshoff, D Haffner, E Wühl, F Schaefer

{"title":"The use of recombinant human growth hormone in short children with chronic renal failure.","authors":"O Mehls, B Tönshoff, D Haffner, E Wühl, F Schaefer","doi":"10.1515/jpem.1994.7.2.107","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.2.107","url":null,"abstract":"Regulation of the somatotropic axis is altered in chronic renal failure (CRF) resulting in a secondary syndrome of growth hormone (GH) insensitivity. Secretion of growth hormone estimated by deconvolution analysis is low normal in prepubertal patients and reduced in late pubertal children with CRF. Basal and integrated GH serum concentration measured by RIA is increased due to reduced renal metabolic clearance, whereas the fractional urinary excretion is increased due to damage of renal tubular cells. GH receptor mRNA is decreased (rat) and the serum concentration of GH binding protein (BP) activity is low (man). Insulin-like growth factor (IGF)-1 production rate is reduced, whereas serum concentrations of IGFBPs are increased secondary to reduced renal metabolic clearance. This results in a reduction of free, active IGF-1. Treatment with GH induces a rise in serum IGF-1 concentration and normalizes IGF bioactivity. Clinical studies in prepubertal children demonstrated a dramatic rise in height velocity during the first treatment year and to a lesser extent during the following years. In children on conservative treatment prior to dialysis, mean height SDS improved by 1.5 within two years and by 2.0 within four years. Patients with renal allografts responded in a similar way. Age and pretreatment height velocity SDS are confounding variables for the response to GH. Renal function seems not be altered by recombinant human (rh) GH in patients with CRF, and the number of renal allograft rejection crises seems not to be substantially increased under rhGH treatment in allograft recipients.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 2","pages":"107-13"},"PeriodicalIF":0.0,"publicationDate":"1994-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.2.107","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19055720","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: 16

Celiac disease as a cause of transient hypocalcemia and hypovitaminosis D in a 13 year-old girl. 乳糜泻是13岁女孩短暂性低钙血症和维生素D缺乏症的病因。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.53

Y Rakover, H Hager, E Nussinson, R Luboshitzky

引用次数: 14

Five years experience with recombinant human growth hormone treatment of children with chronic renal failure. 5年重组人生长激素治疗儿童慢性肾功能衰竭的经验。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.1

R N Fine, O Yadin, L Moulton, P A Nelson, M I Boechat, B M Lippe

{"title":"Five years experience with recombinant human growth hormone treatment of children with chronic renal failure.","authors":"R N Fine, O Yadin, L Moulton, P A Nelson, M I Boechat, B M Lippe","doi":"10.1515/jpem.1994.7.1.1","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.1.1","url":null,"abstract":"11 males, aged 2.5-16.3 years (6.8 +/- 4.1) with growth retardation (Standard Deviation Score--SDS > -2.00) consequent to chronic renal failure (CRF) received recombinant human growth hormone (rhGH) for 18 to 60 mo (40.9 +/- 15.4). Growth velocity (GV) increased from 5.4 +/- 2.2 for the year prior to rhGH to 8.9 +/- 1.6 (p = 0.00001), 7.4 +/- 1.7 (p < 0.03), 7.6 +/- 1.6 (p < 0.006), 6.5 +/- 1.0 (p < 0.05) and 7.5 +/- 1.3 (p = NS) cm/yr following 12, 24, 36, 48 and 60 mo respectively of treatment. The mean SDS for height decreased from -3.21 at baseline to -0.85 at 60 mo (p = 0.0004); 7 of 8 pts treated for > 36 mo had a SDS more positive than -2.00; 3 reached the 50th percentile on the growth curve. In 2 patients the dosage was doubled to achieve the increase in GV; in one patient it took 5 yrs to reach a SDS more positive than -2.00. A significant increase in weight gain and mid-arm muscle circumference over baseline values were indicative of the anabolic effect of rhGH. The mean increase in bone age was similar to the increase in chronologic age; the delta bone age-delta height age was not significant indicating no loss of growth potential following rhGH. Although 3 patients required the initiation of dialysis following rhGH treatment, the mean calculated creatinine clearance did not decrease significantly. No significant adverse effects were noted. These data indicate that long-term rhGH treatment is effective in improving the GV of children with CRF and facilitating catch-up growth without loss of growth potential.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 1","pages":"1-12"},"PeriodicalIF":0.0,"publicationDate":"1994-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.1.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19178891","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: 53

Diabetic nephropathy in a prepubertal diabetic female. 青春期前糖尿病女性的糖尿病肾病。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.43

T J DeClue, A Campos

引用次数: 11

Prolactinoma associated with transient growth hormone deficiency but persistent growth retardation. 催乳素瘤与短暂性生长激素缺乏和持续生长迟缓有关。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.69

I D Schwartz, L M Hussey, J A Grunt, C P Howard

{"title":"Prolactinoma associated with transient growth hormone deficiency but persistent growth retardation.","authors":"I D Schwartz, L M Hussey, J A Grunt, C P Howard","doi":"10.1515/jpem.1994.7.1.69","DOIUrl":"https://doi.org/10.1515/jpem.1994.7.1.69","url":null,"abstract":"A 14.8 year old boy was evaluated for galactorrhea of two months duration and growth deceleration for greater than three years. He was 3.7 standard deviations (SD) below the mean for age in height and euthyroid with uncompromised vision, bilateral galactorrhea, and pubertal arrest. MRI demonstrated a 10 x 8 mm left pituitary mass. Bone age was 11.5 years. Serum prolactin (PRL) decreased by more than 85% after 5 weeks of treatment with bromocriptine (Br). After five months, the prolactinoma (PRLoma) measured 5 x 4 mm. Hypothalamic-pituitary function indicated growth hormone (GH) deficiency and hypogonadotropic hypogonadism as assessed by ITT-TRH-GnRH-clonidine. After nine months of Br, despite return of adequate gonadotropin and GH secretion as assessed by repeat ITT-TRH-GnRH-clonidine, pooled 12 hour nocturnal spontaneous GH secretion, and clinical progression of puberty, there was no linear \"catch-up growth\" (growth rate = 4.4 cm/yr and height 4.2 SD below the mean for age). Growth rate increased following supplemental GH administration without untoward effect. We conclude that there may be discordance/lag between reduction in secretion and size of PRLomas and growth despite resolution of other anterior pituitary dysfunction. Other possibilities are discussed.","PeriodicalId":79383,"journal":{"name":"The Journal of pediatric endocrinology","volume":"7 1","pages":"69-73"},"PeriodicalIF":0.0,"publicationDate":"1994-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/jpem.1994.7.1.69","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19176906","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: 3

Non-classical 3 beta-hydroxysteroid dehydrogenase deficiency in children in central Iowa. Difficulties in differentiating this entity from cases of precocious adrenarche without an adrenal enzyme defect. 爱荷华州中部儿童非经典3 -羟基类固醇脱氢酶缺乏症与无肾上腺酶缺陷的性早熟相鉴别的困难。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.19

D M Cathro, S G Golombek

引用次数: 4

Two interesting cases of transient neonatal diabetes mellitus. 两例有趣的新生儿短暂性糖尿病。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.47

M Salerno, N Gasparini, M L Sandomenico, A Franzese, A Tenore

引用次数: 19

Use of a two-site immunoradiometric assay to resolve a factitious elevation of ACTH in primary pigmented nodular adrenocortical disease. 使用双位点免疫放射测定法解决原发性色素结节性肾上腺皮质疾病中ACTH的人为升高。

The Journal of pediatric endocrinology Pub Date : 1994-01-01 DOI: 10.1515/jpem.1994.7.1.61

A H Slyper, J W Findling

引用次数: 4