HCG对青春期前低促性腺激素性腺发育不良型小阴茎皮肤组织T、DHT含量的影响

人绒毛膜促性腺激素(HCG)治疗可提高患儿血清睾酮(T)水平,临床上常用其治疗低促性腺激素性腺发育不良型小阴茎,且有较好疗效[1],但其促进阴茎发育具体机制尚不太清楚.本文将临床确诊的10例青春期前低促性腺激素性腺发育不良型小阴茎患儿予以HCG治疗,观察患儿阴茎长度的变化,及其对患儿阴茎皮肤组织T和双氢睾酮(DHT)含量的影响.     

HCG对青春期前低促性腺激素性腺发育不良型小阴茎皮肤组织T、DHT含量的影响

人绒毛膜促性腺激素(HCG)治疗可提高患儿血清睾酮(T)水平,临床上常用其治疗低促性腺激素性腺发育不良型小阴茎,且有较好疗效[1],但其促进阴茎发育具体机制尚不太清楚.本文将临床确诊的10例青春期前低促性腺激素性腺发育不良型小阴茎患儿予以HCG治疗,观察患儿阴茎长度的变化,及其对患儿阴茎皮肤组织T和双氢睾酮(DHT)含量的影响.     

HCG对青春期前低促性腺激素性腺发育不良型小阴茎皮肤组织T、DHT含量的影响

人绒毛膜促性腺激素(HCG)治疗可提高患儿血清睾酮(T)水平,临床上常用其治疗低促性腺激素性腺发育不良型小阴茎,且有较好疗效[1],但其促进阴茎发育具体机制尚不太清楚.本文将临床确诊的10例青春期前低促性腺激素性腺发育不良型小阴茎患儿予以HCG治疗,观察患儿阴茎长度的变化,及其对患儿阴茎皮肤组织T和双氢睾酮(DHT)含量的影响.     

男性单纯性肥胖患儿性发育的变化

目的 探讨男性单纯性肥胖患儿性发育的影响。方法 检测42例8-14岁男性单纯性肥胖儿童睾丸容积、阴茎横径长度、血清卵泡刺激素（FSH）、黄体生成素（LH）、雌二醇（E2）、睾酮（TTT）、泌乳素（PRL）、皮质醇（Cortisol）并与正常对照组比较。结果 患儿睾丸容积、阴茎大小及长度，血清FSH、LH、TTT均低于正常对照组，并与其肥胖程度呈负相关。PRL、E2、Cortisol均高于正常对照组，且与其肥胖程度呈正相关。结论 男性单纯性肥胖儿童具有性发育不良倾向。     

简易化黄体生成素释放激素激发试验诊断性早熟

目的通过对性早熟患儿黄体生成素释放激素(LHRH)激发试验中各时间段黄体生成素(LH)、卵泡刺激素(FSH)、雌二醇(E2)、睾酮(T)水平分析,探求激发试验的简易化。方法运用全自动化学发光免疫分析系统(ACS:180)检测60例性早熟患儿基础血清E2、T、FSH、LH水平及LHRH激发试验后血清FSH、LH水平,并进行比较。结果中枢性性早熟(CPP)组39例LHRH注射30 min后FSH和LH明显升高(Pa<0.01),LH/FSH>1,LH峰值>12 IU/L。周围性性早熟(PPP)组21例注射LHRH前及注射后30、60、90 min后,血LH、FSH无显著变化(Pa>0.05)。E2和T水平于注射前和注射后60 min无差异显著性(P>0.05)。二组LH、FSH高峰均集中在30、60 min。结论LHRH激发试验主要依据LH峰值和LH/FSH值来判断;激发试验可简化为LHRH激发前和激发后30、60 min分别抽血检测LH、FSH,即能为临床提供诊断依据。     

GnRHR 基因突变的低促性腺激素性性功能减退症一家系报告及文献复习

目的探讨孤立性低促性腺激素性性功能减退症(IHH)的临床特点、基因突变及治疗。方法回顾分析1例IHH患儿及其家系的临床资料,并复习相关文献。结果先证者为7岁男性患儿,因阴茎和睾丸小而就诊;其弟5岁,也有阴茎小并曾有隐睾。两兄弟睾酮、黄体生成素(LH)、卵泡刺激素(FSH)水平均低下,且GnRH激发试验无反应。患儿父母非近亲结婚。Illumina测序发现兄弟俩均存在GnRHR受体(GnRHR)基因C.806CT纯合突变,父母为C.806CT杂合突变。明确诊断后予绒毛膜促性腺激素(hCG)治疗,6周后睾酮及双氢睾酮水平明显升高。结论结合临床表型、生化特征分析,以及基因检测,有助于早期诊断IHH。     

促性腺激素治疗对多种垂体激素缺乏症男性患者睾丸形态和功能影响的时效性

目的研究人绒毛膜促性腺激素(hCG)和人绝经期促性腺激素(hMG)起始治疗年龄对多种垂体激素缺乏症男性患者的睾丸形态和功能改善情况的影响。方法以多种垂体激素缺乏症男性患者为研究对象,给予hCG和hMG治疗,同时维持其他激素正常,观察睾丸、阴茎形态,促性激素水平,身高,骨龄等变化值与开始治疗年龄的相关性。结果入组的54例患者经hCG和hMG治疗6个月后,阴茎长度由治疗前的(2.58±0.69)cm增长至(4.19±0.77)cm,阴茎周长由治疗前的(3.71±1.36)cm增长至(5.95±1.26)cm,睾丸容积从治疗前的(1.76±1.49)ml增加至(5.20±2.30)ml,身高从治疗前的(147.01±12.29)cm增加至(151.98±11.52)cm,骨龄从治疗前的(11.22±2.71)岁增长至(11.64±2.72)岁,差异均有统计学意义(P0.01)。睾丸容积及睾酮水平在治疗前、后的增长值随年龄增长均呈降低趋势,在各年龄组间的差异均有统计学意义(P均0.05)。用药前后睾丸容积增长值、血清促卵泡激素(FSH)和黄体生成素(LH)水平增加值以及用药前后身高增长值均与开始治疗年龄呈负相关(r=-0.517~-0.334,P0.05)。结论在正常青春期时期适当早期给予多种垂体激素缺乏症男性患者hCG和hMG治疗可更好改善其第二性征和睾丸功能。     

HCG与外用睾酮霜治疗小阴茎的临床疗效观察

目的 探寻临床治疗小阴茎的有效治疗办法及其选择用药的依据.方法 按照小阴茎诊断标准选择病例,分为HCG组(肌注HCG,每次1000IU,每周2次,连用6周)和T组(每日外用睾酮霜2次),并于治疗前后测定阴茎长度、睾丸大小及性激素水平等以观察其疗效及影响.结果 阴茎长度测量:用药前两组阴茎长度比较无显著差异(P>0.05),而用药后两组均有显著增长(P<0.05),但两组用药后增加值比较无显著差异(P>0.05).睾丸体积测量:用药前两组睾丸体积比较无显著差异(P>0.05),但HCG组用药后有显著增长(P<0.05),而睾酮组用药后睾丸体积仪稍有增长(P>0.05).激素水平测定:除HCG组用药前后睾酮水平比较有显著性差异(P<0.05)外,其他各检测指标均无明显变化.疗效:HCG组中7例显效、5例有效、4例无效;T组中6例显效、7例有效、2例无效.两组比较疗效无显著性差异(P>0.05).结论 肌注HCG和外用睾酮霜治疗儿童小阴茎均可使阴茎明显的增长,且对性激素水平无明显干扰,并与患儿年龄大小无关.但外用睾酮霜更为方便实用,在为使睾丸得到同步增长时也可首选HCG治疗.西药治疗无效时应想到雄激素抵抗综合症可能.     

青春期前小阴茎患儿的临床治疗探讨

目的观察小阴茎患儿使用激素及辅助治疗后阴茎变化规律,探讨青春期前小阴茎的治疗方案。方法采取自身治疗前后对照,共收集5～14岁不同年龄组单纯性小阴茎患儿246例,给予综合治疗(HCG1000U,肌注隔日一次,共10次。同时给予短小阴茎仪负压治疗,每日一次,每次20min及中药治疗)熏20d一疗程。治疗前后进行阴茎测量。结果患儿治疗后阴茎明显增大,有显著性差异(P<0.001)。结论对于青春期前小阴茎患儿应用HCG辅以短小阴茎仪负压治疗及中药治疗,能得到较满意的效果。     

儿童阴茎发育不良治疗前后血睾酮检测的临床意义

目的　探讨血睾酮含量在儿童阴茎发育不良中的临床价值。方法　应用放免法对 137例阴茎发育不良患儿及 30例正常儿童血睾酮含量进行检测 ,并测阴茎长度 ,同时观察治疗后血睾酮水平及阴茎长度的变化。结果　 137例阴茎发育不良患儿血血睾酮含量显著低于正常组 ,绒毛膜促性腺激素 (HCG)治疗后血睾酮明显升高 ,阴茎长度明显增加。结论　儿童外阴茎发育不良与HCG、血血睾酮含量有关 ,经HCG治疗后可增加机体血睾酮含量 ,促进阴茎发育     

Comparison of growth hormone releasing hormone therapy and growth hormone therapy in growth hormone deficiency

Seven children with growth hormone deficiency of hypothalamic origin responded to an i.v. bolus of growth hormone releasing hormone (GHRH) (1–29)-NH2 with a mean serum increase of 10.7 ng/ml growth hormone (GH) (range 2.5–29.3 ng/ml). Continuous s.c. administration of GHRH of 4–6 g/kg twice daily for at least 6 months did not improve the growth rate in five of the patients. One patient increased his growth rate from 1.9 to 3.8 cm/year and another from 3.5 to 8.2 cm/year; however, the growth rate of the latter patient then decreased to 5.4 cm/year. When treatment was changed to recombinant human growth hormone (rhGH) in a dose of 2 U/m² daily, given s.c. at bedtime, the growth rate improved in all patients to a mean of 8.5 cm/year (range: 6.2 to 14.6). Presently GHRH cannot be recommended for the routine therapy of children with growth hormone deficiency since a single daily dose of rhGH produced catch-up growth which GHRH therapy did not.Abbreviations GH growth hormone - GHD growth hormone deficiency - GHRH growth hormone releasing hormone - hGH human growth hormone - rhGH recombinant human growth hormone - SM C/IGF I somatomedin C/insulin-like growth factor I On the occasion of the 85th birthday of Prof. Dr.Dr.h.c. mult. Adolf Butenandt     

Effect of two consecutive administrations of GHRH in children with constitutional growth delay

It has been suggested that children with constitutional growth delay might have a transient immaturity of the neurotransmitter pathways necessary for the control of growth hormone releasing hormone (GHRH) secretion. In this study we evaluated the effects of two consecutive GHRH boluses (1 g/kg, i.v.) in nine prepubertal boys with constitutional growth delay. Growth hormone (GH) responses to GHRH administration were similar to that observed in normal children (first GHRH bolus, GH net incremental area under the curve (nAUC) ±SE: 788±244 vs 984±242 ng/ml per hour; second bolus, GHnAUC: 657±122 vs 541±129 ng/ml per hour, respectively). These data suggest that no relevant abnormalities in the mechanisms determining the somatotroph sensitivity to GHRH are present in children with constitutional growth delay.     

Oral administration of arginine enhances the growth hormone response to growth hormone releasing hormone in short children

We have evaluated the effect of oral administration of arginine chlorhydrate on the growth hormone response to growth hormone releasing hormone in a group of nine short prepubertal children (six boys and four girls). Arginine chlorhydrate 10 g, administered orally 60 min before an iv bolus injection of growth hormone releasing hormone 1–29, 1 μg/kg, significantly enhanced the growth hormone response to the neuropeptidc, confirming the results of previous studies which used the iv route. Furthermore, our data strengthen the view that the effects of arginine chlorhydrate on growth hormone secretion are mediated by inhibition of endogenous somatostatin release.     

The Dosage Dependency of Growth and Maturity in Growth Hormone Deficiency Treated with Human Growth Hormone

ABSTRACT. A group of 11 pre-pubertal growth hormone deficient patients were treated with human growth hormone over a period of 4 years. In 6 of the patients the dosage was 4 IU 3 times a week and in 5, 8 IU 3 times a week. Changes in height demonstrated that the "catch up" was significantly greater and of longer duration in the second group. In spite of a more rapid increase of bone age in the second group, the prognosis of final height had improved significantly at the end of the study period. A comparative study of the plasma concentrations of T4, T SH, gonadotrophins and steroids, to see if the greater velocity of bone maturity in the second group could be due to contamination of the preparation by other could be due to contamination of the preparation by other hypophysary hormones, did not demonstrate significant differences between the groups.     

Rapid effect of intravenous growth hormone (GH)-releasing hormone 1–44 on plasma GH levels in children

Growth hormone releasing hormone (GHRH)-testing was performed in 24 short normal children (16 male, 8 female). Before and after administration of GHRH_1–44 (1g/kg body weight i.v.) blood samples for growth hormone (GH) determination were drawn at-30, 0, 1, 2, 3, 4, 6, 8, 10, 15, 30, 45, 60, and 90 min. Plasma GH increase was apparent 1 min after injection and in 12 patients (7 female) peak plasma GH values were reached within 15 min. In all patients plasma GH levels were greater than 10 ng/ml within the first 8 min following GHRH injection, but in 4 patients this level was not attained when considering only GH values obtained after 15 min. These results demonstrate the capability of the pituitary to rapidly secerete GH in response to GHRH_1–44 in children. Therefore, in this age group blood samples for GH determination should be taken earlier when testing with GHRH_1–44.Abbreviations GHRH growth hormone releasing hormone - GH growth hormone - SD standard deviation     

Oral administration of arginine enhances the growth hormone response to growth hormone releasing hormone in short children

We have evaluated the effect of oral administration of arginine chlorhydrate on the growth hormone response to growth hormone releasing hormone in a group of nine short prepubertal children (six boys and four girls). Arginine chlorhydrate 10 g, administered orally 60 min before an iv bolus injection of growth hormone releasing hormone 1–29, 1 μg/kg, significantly enhanced the growth hormone response to the neuropeptidc, confirming the results of previous studies which used the iv route. Furthermore, our data strengthen the view that the effects of arginine chlorhydrate on growth hormone secretion are mediated by inhibition of endogenous somatostatin release.     

Effects of long-term growth hormone releasing hormone 1 -29 in significantly short children

Seven children with significant idiopathic short stature (SISS) whose heights were significantly below the third percentile (SD score for height —2.5 to —3.5) and who had normal levels of growth hormone (GH) were treated with growth hormone releasing hormone (GH-RH) in a dose of 30 /μg/kg/day. Therapy was discontinued if patients failed to increase their rates of growth by more than 2.0 cm/year over their pre-therapy growth rate. Treatment was discontinued in two of the patients after 12 months but was continued in the other five for 24 months. These data demonstrate that some patients with SISS grow well during the first 2 years of treatment with GH-RH.     

Diagnostic value of growth hormone-releasing hormone test in children and adolescents with idiopathic growth hormone deficiency

Average growth hormone (GH) peaks following an i.v. growth hormone releasing hormone (GHRH) 1–29 stimulation test were significantly lower in 48 children and adolescents with GH deficiency (GHD) than in 20 age-matched controls (15.2+12.7 vs 37.5+28.1 ng/ml, 2P<0.001). Twelve patients exhibited a low GH peak (<5 ng/ml), 27 demonstrated a normal response (>10 ng/ml) and 9 showed an intermediate rise in plasma GH (5–10 ng/ml). Six of the 12 patients with low GH response to the first GHRH stimulation failed to respond to two other tests immediately before and after a 1 week priming with s.c. GHRH. These subjects with subnormal GH increase at repeat testing had total GHD (TGHD) and multiple pituitary hormone deficiency (MPHD) and had suffered from perinatal distress. On the contrary, 26 of 27 patients with normal GH response to the first test had isolated GHD and only a minority (8/27) had signs of perinatal distress. It is concluded that perinatal injuries primarily damage pituitary structures and that a pituitary defect more probably underlies more severe forms (TGHD and MPHD) of GHD.Presented in part at the 7th Meeting of the Italian Society for Paediatric Endocrinology (Milan, 20–21 October 1989)     

Evaluation of growth hormone secretion after completion of therapy

BACKGROUND: Growth hormone (GH) reserve in young adults previously diagnosed as having GH insufficiency, who were treated with human (h)GH replacement in childhood needs confirmation in adulthood. METHODS: Nine patients (seven males, two females; two empty cella, one hypoplasia of the hypophysis and six with idiopathic GH deficiency) diagnosed as having GH insufficiency by the insulin tolerance test (ITT) and dopamine stimulation test in childhood (mean age 12.8+/-2.6 years) were retested at completion of linear growth (mean age 21.0+/-3.0 years), 4.6+/-1.6 years after discontinuation of hGH therapy. RESULTS: At the initial diagnosis, seven had complete and two had partial GH deficiency. At diagnosis, the mean peak GH response to ITT and dopamine was 4.8+/-4.08 and 3.4+/-2.9 mU/L, respectively. At retesting, the mean GH response to ITT and dopamine stimulation was 3.5+/-2.5 and 3.3+/-3.1 mU/L, respectively (P=0.91 and 0.96, respectively). During hGH therapy, mean height velocity increased from 3.5+/-1.9 cm/year at diagnosis to 9.9+/-3.64 cm/year during the first year (P=0.002). One of nine children diagnosed as having GH insufficiency who was treated with hGH replacement had normal growth hormone secretion at completion of linear growth. CONCLUSIONS: All GH-insufficient children should be retested after completion of their hGH treatment and linear growth to identify those who are truly GH insufficient and who may benefit from GH therapy in adulthood.     

Synchronization of Growth Hormone Therapy

Based on the hypothesis of periodical refractoriness of the growing tissue to exogenous administration of GH, an experimental approach is presented in which the dosage of GH was adjusted to the natural occurrence of periodical changes of the lower leg growth velocity in five children with short stature. Two 6-month periods were compared, during which the children received an identical cumulative dose of GH of 224 IU/m². During the first period, the children received a constant GH dose of 14 IU/m²/week by daily subcutaneous injection, whereas a low/high dose alternation was administered at approximately 3-week intervals synchronously with the occurrence of mini growth spurts during the second 6-month period. In all the children, mean linear growth of the lower leg accelerated during the period of synchronization by 595% compared to the previous growth rate during constant GH administration.