Abstract:
:Congenital generalized lipoatrophy (CGL) is a syndrome with multiple clinical manifestations and complete atrophy of adipose tissue. The exact mechanism of this disease remains unknown. One hypothesis presupposes an abnormal development of adipocytes. Leptin, the adipocyte-specific product of the ob gene, acts as a regulatory factor of body weight. In children, as in adults, leptin levels are correlated with body mass index (BMI) and body fat mass. Some authors have demonstrated that adults with congenital or acquired generalized lipoatrophy have decreased leptin concentrations. In order to study serum leptin profile during childhood in this disease, we measured serum leptin concentrations in six children aged 5.5-11 years suffering from CGL, and investigated the relationship between metabolic parameters and the variations in leptin levels. Serum leptin concentrations (1.19+/-0.32 ng/ml (+/- S.D.)) were extremely low compared with those observed in normal children. No significant correlation was found with BMI, which is known to be one of the major determinants of serum leptin. Serum leptin values were significantly correlated with fasting insulin levels (r=0.83, P=0.024). In conclusion, extremely low leptin values measured in children with CGL could be regarded as one among other diagnostic parameters. However, the detectable levels observed in all of these children support the evidence that a small amount of body fat is likely to be present in these patients, despite complete subcutaneous lipoatrophy. Our data suggest that this small amount of adipose tissue could be metabolically active and, at least in part, sensitive to insulin. Further investigations are required to uncover the pathophysiological mechanisms of this syndrome, known to be commonly associated with insulin resistance.
journal_name
Eur J Endocrinoljournal_title
European journal of endocrinologyauthors
Jaquet D,Khallouf E,Lévy-Marchal C,Czernichow Pdoi
10.1530/eje.0.1400107subject
Has Abstractpub_date
1999-01-01 00:00:00pages
107-9issue
1eissn
0804-4643issn
1479-683Xjournal_volume
140pub_type
杂志文章abstract::The p16INK4A and p15INK4B genes on chromosome 9p21 encode the p16 and p15 inhibitors of cyclin D/cyclin-dependent kinase 4 complexes respectively. Mutations and deletions of the p16INK4A gene have been found in melanomas and many other types of tumors. To assess the role of the p16INK4A and p15NK4B genes in tumorigene...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1360074
更新日期:1997-01-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-08-0866
更新日期:2009-07-01 00:00:00
abstract:OBJECTIVE:Vascular endothelial growth factor (VEGF) exists in three main splice variants, characterized by 121, 165 and 189 amino acids (VEGF 121, VEGF 165 and VEGF 189) and acts via two specific receptors: VEGF-R1 or Flt-1 and VEGF-R2 or KDR. VEGF plays an important role in the pathogenesis of diabetic retinopathy. Th...
journal_title:European journal of endocrinology
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doi:10.1530/eje.0.1500799
更新日期:2004-06-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/eje.0.1450155
更新日期:2001-08-01 00:00:00
abstract:OBJECTIVE:Local 5'-deiodination of l-thyroxine (T(4)) to active thyroid hormone 3,3',5-tri-iodothyronine (T(3)) catalyzed by the two 5'-deiodinase enzymes (D1 and D2) regulates various T(3)-dependent functions in the anterior pituitary and has been well studied in rodents. Only limited information about deiodinase expr...
journal_title:European journal of endocrinology
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doi:10.1530/eje.0.1470263
更新日期:2002-08-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-12-0191
更新日期:2012-12-31 00:00:00
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journal_title:European journal of endocrinology
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doi:10.1530/eje.0.1370410
更新日期:1997-10-01 00:00:00
abstract:Objective:Hypertension and symptoms of catecholamine excess are features of pheochromocytomas and paragangliomas (PPGLs). This prospective observational cohort study assessed whether differences in presenting features in patients tested for PPGLs might assist establishing likelihood of disease. Design and methods:Pati...
journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:2019-10-01 00:00:00
abstract:OBJECTIVE:Cases of men with estrogen resistance and aromatase deficiency have highlighted the effects of estrogens on bone metabolism, the cardiovascular system and biochemical variables of the metabolic syndrome. In eugonadal men, administration of an aromatase inhibitor induces a substantial elevation of LH and testo...
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pub_type: 杂志文章,随机对照试验
doi:10.1530/eje.1.02126
更新日期:2006-04-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/EJE-19-0234
更新日期:2019-09-01 00:00:00
abstract::Evidence suggests that insulin resistance and hyperinsulinaemia are associated with ovarian hyperandrogenism and menstrual irregularities in polycystic ovary syndrome (PCOS). Sixteen obese women with PCOS on a weight-maintaining diet were studied before and after 6 months of therapy with the insulin-sensitizing antidi...
journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章
doi:10.1530/eje.0.1380269
更新日期:1998-03-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-12-0879
更新日期:2013-03-15 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-12-0160
更新日期:2012-07-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1330166
更新日期:1995-08-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-11-0545
更新日期:2011-10-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-11-0849
更新日期:2012-05-01 00:00:00
abstract::Sex hormone-binding globulin (SHBG) and corticosteroid-binding globulin (CBG) levels were evaluated in euthyroid (N = 111), hyper- (N = 58) and hypothyroid (N = 38) men, in pre- and postmenopausal women (study 1) and in hyper- (N = 24) and hypothyroid (N = 15) patients before and after treatment with carbimazole or le...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1320594
更新日期:1995-05-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章,随机对照试验
doi:10.1530/EJE-07-0857
更新日期:2008-06-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1530/eje.0.1460339
更新日期:2002-03-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1530/eje.1.01971
更新日期:2005-09-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-07-0762
更新日期:2008-05-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1410160
更新日期:1999-08-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-08-0519
更新日期:2009-02-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-19-0844
更新日期:2020-02-01 00:00:00
abstract::Corticotrophin-releasing hormone (CRH) causes vasodilatation in the human fetal-placental circulation and has paracrine actions in placental tissue, suggesting that CRH receptors may be present in the human placenta. We have now identified and characterized placental CRH binding sites and compared them to those descri...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1330591
更新日期:1995-11-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章
doi:10.1530/eje.0.1470059
更新日期:2002-07-01 00:00:00
abstract::The GH/IGF1 axis may play an important role in cognitive function. This theory is supported by the finding that both GH and IGF1 receptors are located in several brain areas such as the hippocampus, a brain area that is known to play an essential role in cognitive processes, especially memory and learning. However, th...
journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/EJE-08-0279
更新日期:2008-12-01 00:00:00
abstract::Objective To explore whether gestational prolactin and breast increase are markers of metabolic health in pregnancy and on long-term, in PCOS. Design Follow-up study. Women with PCOS, according to the Rotterdam criteria (n = 239), former participants of the randomized controlled trial (RCT) PregMet were invited, 131 p...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-19-0002
更新日期:2019-06-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.1.02031
更新日期:2005-12-01 00:00:00
abstract::In addition to the classical hormones, the production of numerous polypeptide growth factors, cytokines, vasogenic substances and neuropeptides by pituitary cells has been demonstrated. Expression of the corresponding receptors on pituitary cells enables these factors to influence growth and function of the pituitary ...
journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/eje.0.1350515
更新日期:1996-11-01 00:00:00