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International Journal of Pediatric Endocrinology最新文献

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Growth and descent of the testes in infants with hypogonadotropic hypogonadism receiving subcutaneous gonadotropin infusion. 皮下注射促性腺激素治疗促性腺功能减退症婴儿睾丸的生长和下降。
International Journal of Pediatric Endocrinology
Pub Date : 2016-01-01 Epub Date: 2016-07-04 DOI: 10.1186/s13633-016-0031-9
Anne-Sophie Lambert, Pierre Bougneres

Background: One third of infants with congenital hypogonadotropic hypogonadism (CHH) are said to have micropenis and/or bilateral or unilateral cryptorchidism leading many of them to orchiopexy. Our previous study in two patients suggests that prolonged subcutaneous infusion of large doses of gonadotropins might normalize testicular function and growth.

Case presentation: To confirm the effects of early and prolonged subcutaneous infusion of large doses of gonadotropins on growth and descent of the testes. Eight boys with CHH, aged 0.25-11 months. Testes were non-palpable in 5 or in high scrotal position in 3. CHH was isolated in 5 infants and part of a syndrome of combined pituitary hormonal deficits in the 3 others. In response to gonadotropin infusion, mean levels of testicular hormones were normalized. Complete testis descent occurred in 6 patients. Partial descent occurred in 2. Testes re-ascended in 1 patient. Testes and penis gained normal dimensions in all cases.

Conclusion: Subcutaneous gonadotropin infusion seems able to induce testis descent in a large proportion of infants with CHH. If confirmed, this may allow patients to avoid testes surgery but studies in larger series are needed to evaluate the benefits of this treatment versus traditional orchiopexy.

背景:三分之一的先天性促性腺功能低下(CHH)婴儿据说有小阴茎和/或双侧或单侧隐睾，导致他们中的许多人进行了睾丸切除术。我们之前对两例患者的研究表明，长时间皮下注射大剂量促性腺激素可能使睾丸功能和生长正常化。病例介绍:确认早期和长期皮下注射大剂量促性腺激素对睾丸生长和下降的影响。8名CHH男孩，年龄0.25-11个月。5例未触及睾丸，3例位于阴囊高位。5例婴儿分离出CHH，另外3例为合并垂体激素缺陷综合征的一部分。作为对促性腺激素输注的反应，睾丸激素的平均水平正常化。6例患者出现完全睾丸下降。部分下降发生在2。1例患者睾丸再次上升。所有病例的睾丸和阴茎尺寸均恢复正常。结论:皮下注射促性腺激素似乎能够引起很大比例CHH婴儿睾丸下降。如果得到证实，这可能使患者避免睾丸手术，但需要更大规模的研究来评估这种治疗与传统睾丸切除术的益处。
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引用次数: 38
Body mass index in girls with idiopathic central precocious puberty during and after treatment with GnRH analogues. 在GnRH类似物治疗期间和之后特发性中枢性性早熟女孩的体重指数。
International Journal of Pediatric Endocrinology
Pub Date : 2016-01-01 Epub Date: 2016-08-05 DOI: 10.1186/s13633-016-0033-7
A J Arcari, M G Gryngarten, A V Freire, M G Ballerini, M G Ropelato, I Bergadá, M E Escobar

Background: In girls with Idiopathic Central Precocious Puberty (ICPP) concern has been raised by the potential impact of GnRH-analogues (GnRHa) treatment on body weight. We evaluated the effect of GnRHa on Body Mass Index (BMI) in girls with ICPP according to weight status at diagnosis.

Methods: One hundred seventeen ICPP girls were divided according to pretreatment weight status in: normal weight (NW), overweight (OW) and obese (OB). BMI at one and two years of treatment was assessed. BMI-SDS of 60 patients who reached adult height (AH) was compared to that of 33 ICPP untreated girls.

Results: NW girls significantly increased their baseline BMI-SDS at 1 and 2 years of treatment. OW girls only had a significant increment at one year of treatment while OB girls showed no BMI-SDS change. Patients evaluated at AH (at least four years after GnRHa withdrawal) showed a significant decrease on BMI compared to baseline and a significantly lower BMI than the untreated group.

Conclusion: In ICPP girls the BMI increase under GnRHa was inversely related to the pretreatment weight status. In the long term follow-up, no detrimental effect of GnRHa on body weight was observed. BMI-SDS was lower in treated than in untreated girls.

背景:在患有特发性中枢性性早熟(ICPP)的女孩中，gnrh类似物(GnRHa)治疗对体重的潜在影响引起了人们的关注。我们根据诊断时的体重状况评估GnRHa对ICPP女孩体重指数(BMI)的影响。方法:117例ICPP女生按预处理体重分为正常体重(NW)、超重(OW)和肥胖(OB)。对治疗1年和2年的BMI进行评估。将60例达到成人身高(AH)的患者的BMI-SDS与33例未接受ICPP治疗的女孩进行比较。结果:NW女孩在治疗1年和2年的基线BMI-SDS显著增加。在治疗一年后，OW女孩的BMI-SDS仅显著增加，而OB女孩的BMI-SDS没有变化。经AH评估的患者(GnRHa停药后至少4年)BMI与基线相比显著降低，BMI明显低于未治疗组。结论:在ICPP女孩中，GnRHa下BMI的增加与预处理体重状况呈负相关。在长期随访中，未观察到GnRHa对体重的有害影响。治疗组的BMI-SDS低于未治疗组。
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引用次数: 21
Fat free mass index and fat mass index tracing on body composition chart in diabetes adolescent girls 糖尿病少女身体成分图的无脂质量指数和脂肪质量指数追踪
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P20
Sochung Chung, H. Park, B. Kwak, Kyo Sun Kim
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引用次数: 1
Growth effect of tki treatment in childhood CML tki治疗对儿童期CML生长的影响
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P32
Y. J. Jeon, In Ah Jung, W. Cho, Jaewook Lee, N. Chung, M. Jung, B. Cho, Byung-Kyu Suh
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引用次数: 0
An infant with Cushing syndrome 一个患有库欣综合症的婴儿
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P41
A. Sng, K. Loke, Y. Lim, C. Ho
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引用次数: 0
ISPAD and its role in the management of diabetes in the young in the Indian subcontinent and the Far East ISPAD及其在印度次大陆和远东年轻人糖尿病管理中的作用
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-O15
S. Greene
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引用次数: 0
Novel mutation in the hepatocyte nuclear factor 1B/maturity – onset diabetes of the young type 5 gene – unreported Vietnamese case 新突变的肝细胞核因子1B/成熟型糖尿病的年轻5型基因-未报道的越南病例
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P25
V. C. Dũng, Bùi Phương Thảo, C. T. B. Ngọc, Nguyen Ngoc Khanh, Nguyen Phu Dat, S. Ellard, M. Craig
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引用次数: 0
Novel heterozygous deletion in the HNF1β gene - adolescent with antibody negative diabetes, longstanding hyperglycaemia without ketosis, cataracts, small echogenic kidneys with a cortical cyst, pancreatic atrophy, exocrine pancreatic insufficiency and a uterine anomaly 新发现的HNF1β基因杂合缺失——患有抗体阴性糖尿病、长期高血糖但无酮症、白内障、伴有皮质囊肿的小回声肾、胰腺萎缩、外分泌胰腺功能不全和子宫异常的青少年
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P1
L. Conwell, I. McGown
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引用次数: 0
Sirolimus therapy following subtotal pancreatectomy in neonatal hyperinsulinemic hypoglycaemia: a case report 西罗莫司治疗次全胰切除术后新生儿高胰岛素性低血糖:1例报告
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P81
M. Abraham, S. Flanagan, V. Shetty, Glynis Price, M. deBock, A. Siafarikas, S. Ellard, S. Resnick, E. Whan, E. Davis, T. Jones, K. Hussain, C. Choong
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引用次数: 0
Insulin-like Growth Factor 1 (IGF-1) measurements recorded in the OZGROW database and their relation to growth response in children currently being treated with growth hormone OZGROW数据库中记录的胰岛素样生长因子1 (IGF-1)测量及其与目前接受生长激素治疗的儿童生长反应的关系
International Journal of Pediatric Endocrinology
Pub Date : 2015-04-28 DOI: 10.1186/1687-9856-2015-S1-P38
I. Hughes, M. Harris, A. Cotterill
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引用次数: 0
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