Effects of growth hormone replacement therapy on glucose and lipid metabolism in patients with hypopituitarism / 中华内分泌代谢杂志

Objective:To investigate the effect of growth hormone replacement therapy(GHRT) on glucose and lipid metabolism in patients with hypopituitarism.Methods:Clinical data of patients with hypopituitarism who received GHRT in Department of Endocrine and Metabolic Diseases, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from December 2016 to February 2020 were retrospectively analyzed. The patients were divided into normal glucose regulation(NGR) group and impaired glucose regulation(IGR) group according to their glucose metabolism status before GHRT. The changes of the characteristics of glucose metabolism before and after GHRT were analyzed.Results:A total of 30 patients aged(23.0±5.2) years were included, 23 patients in NGR group and 7 patients in IGR group. After 12 months of GHRT, there were no significant changes in fasting plasma glucose(FPG), 2-hour postprandial plasma glucose(2hPG), and insulin sensitivity index(ISI) in both groups(all P>0.05), while homeostasis model assessment insulin resistance(HOMA-IR) in IGR group was significantly decreased compared with that before GHRT( P<0.05). None of the patients in NGR group progressed to IGR or diabetes mellitus, and none of the 7 patients in the IGR group progressed to diabetes mellitus, while 4 of them recovered from impaired glucose tolerance(IGT) to NGR. Triglyceride, total cholesterol, and low density lipoprotein-cholesterol levels were all significantly decreased in two groups(all P<0.05). Multivariate linear regression analysis showed that the increase of body mass index was an independent risk factor for the increase of FPG and 2hPG( P<0.05). Conclusion:12-month GHRT significantly improved their blood lipid profiles in patients with hypopituitarism without adversely affecting glucose homeostasis.

Acute glucagon-like peptide-1 infusion has no effects on sex hormones in healthy men / 中华内分泌代谢杂志

The main functions of glucagon-like peptide-1(GLP-1) includes diminishing food intake and enhancing glucose-stimulated insulin secretion. Evidence from basic research suggests that reproduction system is also a target of GLP-1. GLP-1 receptor agonists are now widely used in managing patients with obesity and type 2 diabetes, thus, there is a need to explore the effects of GLP-1 on the human reproductive system. This paper is a Chinese translation of "Effects of Glucagon-like Peptide-1 on the Reproductive Axis in Healthy Men" , published on J Clin Endocrinol Metab[Izzi-Engbeaya C, Jones S, Crustna Y, et al. J Clin Endocrinol Metab, 2020, 105(4). DOI: 10.1210/clinem/dgaa072], with the permission from the original journal. This research studied the effects of acute GLP-1 infusion on sex hormones in healthy men through a randomized, single-blind, placebo-controlled study. The results showed that during GLP-1 infusion, the mean levels of serum luteinizing hormone(LH), follicle stimulating hormone(FSH), and testosterone, as well as the pulsatility of LH and testosterone were similar with vehicle infusion. The data from this study indicates that acute GLP-1 administration has no impact on reproductive hormone secretion in healthy men.

Clinical characteristics and molecular diagnosis in 2 cases of P450 oxidoreductase deficiency / 中华内分泌代谢杂志

Objective To analyze clinical characteristics and gene mutation of two patients diagnosed with P450 oxidoreductase deficiency(PORD). Methods Clinical data of 2 patients with PORD was collected from Ruijin hospital. POR gene mutation was analyzed by PCR-Sanger sequencing. A retrospective analysis of literatures concerning PORD was performed. Results Patient 1, female, 16 years old, with 46,XX karyotype, presented with anorectal anomalies, clitoral hypertrophy at birth and irregular menstruation; Patient 2, female, 32 years old, with 46,XX karyotype, showed irregular menstruation and infertility, both without obvious skeletal deformity. Genetic test of POR gene mutation revealed that patient 1 carried a homozygous missense mutation (R457H) and patient 2 carried a heterozygous mutation (R223X/ Y607C). The two mutations (R223X and Y607C) are reported for the first time in China. Conclusion P450 oxidoreductase deficiency which caused by mutations in POR gene has a variety of clinical manifestations, including abnormal steroid hormone synthesis with or without Antley-Bixler syndrome. The affirmative diagnosis should rely on steroid hormone measurement and POR gene analysis.

A study of congenital lipoid adrenal hyperplasia in two pedigrees / 中华内分泌代谢杂志

The clinical features and laboratory data of two patients with congenital lipoid adrenal hyperplasia (CLAH)were collected. The genomic DNA was extracted from the peripheral blood white cells in the two patients and their family members and the STAR gene was screened for mutations by PCR and Sanger sequencing. Patient 1 was a girl aged 2 years and 8 months,and she visited us because of continual cyanosis for more than two years. Physical examination showed no obvious pigmentation or clitoral hypertrophy,and Tanner stage was B1P1. Clinical examination revealed serum ACTH 1 284.1 pg/ml and 17α-hydoxyprogesterone(17-OHP)0.54 ng/ml, with Karyotype 46, XX. Genetic analysis showed compound heterozygous mutations of c.201_202delCT and c.229C>T in the STAR gene. Her father carried heterozygous c.201_202delCT mutation, and her mother showed heterozygous c.229C>T mutation. Patient 2 was a girl aged 22 years and referred to us because of dark skin for more than 21 years. Physical examination revealed generalized hyperpigmentation,with Tanner stage B5P2. Hormone examination showed ACTH>2 000 pg/ml and serum cortisol 0.77μg/dl. Karyotype analysis revealed 46,XX. Genetic analysis found compound heterozygous mutations of c.64+1G>C and c.707_708delinsCTT in the STAR gene,which descended from her father and mother respectively. Of note,c.64+1G>C is a novel splicing mutation of STAR gene.

A case of 21-hydroxylase deficiency in Turner's syndrome and literature review / 中华内分泌代谢杂志

To improve the recognition and treatment of the combination of classical congenital adrenal hyperplasia (CAH) and Turner's syndrome. A case of 21-hydroxylase deficiency (21-OHD) in 45,X[3] / 46,XX [47] was reported,and the related literatures were reviewed. A 29-year-old woman with 45,X[3] / 46,XX[47] was referred with clitorimegaly and primary amenorrhea. Her height was 150 cm with a weight of 56 kg. Physical examination revealed a Tanner stage Ⅵ for both breast development and pubic hair development. She showed a little signs of Turner' s syndrome, such as cubitus valgus. Lab findings: sex hormones are significantly increased, including progesterone, testosterone, dehydroepiandrosterone, 17 hydroxyprogesterone, dihydrotestosterone, and androstendione. Enhanced CT scan showed bilateral adrenal hyperplasia. Gynecological ultrasound showed that the size of the uterus and ovary were near normal and the endometrium was not clear. By gene mutation screening, two mutation sites were found in CYP21A2 gene, such as IVS2-13C/ A→G and p. Ile173Asn( c. 518T→A). Taken together, the patient was diagnosed as a combination of 21-OHD and Turner syndrome. A total of ten patients associated with CAH in Turner's syndrome have been reported so far. The findings showed that routine karyotyping during investigations of patients presenting with ambiguous genitalia or with a diagnosis of CAH may reveal the concomitant presence of Turner's syndrome. We should make a definite diagnosis and give early treatment as soon as possible.

A case of 21-hydroxylase deficiency in Turner's syndrome and literature review / 中华内分泌代谢杂志

To improve the recognition and treatment of the combination of classical congenital adrenal hyperplasia (CAH) and Turner's syndrome. A case of 21-hydroxylase deficiency (21-OHD) in 45,X[3] / 46,XX [47] was reported,and the related literatures were reviewed. A 29-year-old woman with 45,X[3] / 46,XX[47] was referred with clitorimegaly and primary amenorrhea. Her height was 150 cm with a weight of 56 kg. Physical examination revealed a Tanner stage Ⅵ for both breast development and pubic hair development. She showed a little signs of Turner' s syndrome, such as cubitus valgus. Lab findings: sex hormones are significantly increased, including progesterone, testosterone, dehydroepiandrosterone, 17 hydroxyprogesterone, dihydrotestosterone, and androstendione. Enhanced CT scan showed bilateral adrenal hyperplasia. Gynecological ultrasound showed that the size of the uterus and ovary were near normal and the endometrium was not clear. By gene mutation screening, two mutation sites were found in CYP21A2 gene, such as IVS2-13C/ A→G and p. Ile173Asn( c. 518T→A). Taken together, the patient was diagnosed as a combination of 21-OHD and Turner syndrome. A total of ten patients associated with CAH in Turner's syndrome have been reported so far. The findings showed that routine karyotyping during investigations of patients presenting with ambiguous genitalia or with a diagnosis of CAH may reveal the concomitant presence of Turner's syndrome. We should make a definite diagnosis and give early treatment as soon as possible.

Optimizing the method in calculating the insulin dosages in the insulin hypoglycemia-growth hormone stimulation test:preliminary exploration / 中华内分泌代谢杂志

Objective To explore more suitable calculation method of the insulin dosage in insulin hypoglycemia-growth hormone stimulation test(insulin tolerance test, ITT). Methods Fifty-six subjects suspected of growth hormone deficiency were divided into primary and secondary onset groups. All the patients took oral glucose tolerance test and ITT. Homeostasis model of assessment for insulin resistance index ( HOMA-IR) and insulin sensitivity index ( ISI), area under insulin curve ( AUCINS ) and the area under glucose curve ( AUCPG ) were calculated. The insulin dosages during ITT between two groups were compared and the main factors influencing the insulin dosage were analyzed. Results There was no difference in the insulin dosage during ITT between primary and secondary groups. The actual dosage of insulin in this cohort study revealed a significant difference from the initial insulin dosage recommended by the guideline. Multiple linear regression analysis found that AUCINS and body mass index were the independent factors affecting the insulin dosage. Then the optimized coefficient of ITT ( γ) were found. Conclusion The insulin dosage used in our study was inconsistent with the guidelines-recommended ones. In order to make ITT more efficient and safer, a more optimized calculation method to improve the successful rate of insulin-induced hypoglycemia in ITT is proposed.

Management of pituitary endocrine function in craniopharyngioma / 中华内分泌代谢杂志

Craniopharyngioma is a kind of benign tumor of the relict squamous epithelial cells originated from the pituitary gland of cranial cheek pouch in the process of embryonic development. With an overall incidence of 0.13 cases per 100 000 person-years, they may represent up to 5%-10% of intracranial tumors in children. There is always debate regarding the appropriate treatment for craniopharyngiomas which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. And the complications caused by the treatment such as diabetes insipidus, hypothalamic obesity, and hypopituitarism, that throw out a challenge to the postoperative management and the quality of the patients. Craniopharyngioma should be recognized as a chronic disease requiring constant monitoring along with the clinical and multidisciplinary treatment in order to provide optimal care of surviving patients.

Extremely mild cholesterol side chain cleavage enzyme deficiency with infertility as the only presenting complaint / 中华内分泌代谢杂志

[Summary] All patients with cholesterol side-chain cleavage enzyme ( P450scc) deficiency that have been reported presented with early adrenal failure. Here we described a 35-year-old male presented with infertility as the only initial presenting complaint. He had received two separate surgeries to remove bilateral testicular masses. We reevaluated the resected tumors and found testicular adrenal rest tumor ( TART) pathology in the resected tumor. We profiled steroid hormones and found significantly elevated ACTH. CT scan revealed bilateral adrenal hyperplasia. Mutation screening identified compound heterozygous mutations (R353W and P432L) in the P450scc encoding gene (CYP11A1). The patient was finally diagnosed as congenital adrenal hyperplasia.

Clinical characteristics and genetic analysis in two patients with 3β-hydroxysteroid dehydrogenase deficiency / 中华内分泌代谢杂志

Objective To analyze the clinical characteristics of two patients with 3β-hydroxysteroid dehydrogenase deficiency and to explore their molecular genetic defects.Methods The clinical features and laboratory data of two patients were collected.The exons of HSD3B2 gene were amplified by PCR and sequenced by Sanger sequencing.Results Patient 1, aged 5 yrs old, was raised as a girl with 46, XY karyotype, presented with hyperpigmentation, female infant vulva, clitoral hypertrophy, and bilateral cryptorchidism;Patient 2, aged 11 yrs old, was raised as a girl at birth but as a boy after 1 yr old for known 46, XY karyotype, presented with hyperpigmentation, micropenis and severe hypospadias.Both patients had markedly elevated adrenocorticotropin and decreased cortisol.Two homozygous missense mutations in HSD3B2 gene were identified:conversions of codon Pro155 toLeu(p.P155L)inpatient1,andcodonAla82toThr(p.A82T)inpatient2,bothofwhichwerereportedforthe first time in China.Conclusion The patients with 3β-hydroxysteroid dehydrogenase deficiency in 46,XY karyotype mainly present with male pseudohermaphroditism and adrenocortical deficiency, and the diagnosis should rely on the steroids detection and HSD3B2 gene screening.

Management of infertility in patients with non-obese polycystic ovary syndrome by micro-pulse infusion of GnRH / 中华内分泌代谢杂志

[Summary] Patients with infertility and non-obese polycystic ovary syndrome ( PCOS) were treated with continuous subcutaneous pulse infusion of GnRH. After the treatment, a 32-year old female had regular menstrual cramps. Dominant follicle occurred after 2 months of treatment. The patient was pregnant and now has a healthy baby boy. It shows that the pulse infusion of GnRH could induce spontaneous ovulation and natural fertilization of the patients with non-obese PCOS.

Autoimmune hypophysitis / 中华内分泌代谢杂志

Autoimmune hypophysitis is an inflammation of the pituitary gland resulting from autoimmune response, which can be classified as primary hypophysitis and secondary hypophysitis according to the etiology.The clinical presentation includes hypopituitarism and a pituitary mass.The diagnosis is based on clinical findings and radiology, while the pathology serves as the golden standard.The treatment is aimed at reducing the size of the pituitary mass, replacing the defective hormones and decreasing hyperprolactinemia.This review is about the epidemiology, pathogenesis, diagnosis, and treatment of lymphocytic hypophysitis, hypophysitis secondary to cytotoxic T-4 blockade, and IgG4-related hypophysitis.

Clinical research of impulse GnRH infusion treatment to a patient with central secondary amenorrhea / 中华内分泌代谢杂志

Objective To apply continuous subcutaneous pulse infusion of gonadotropin-releasing hormone (GnRH) to treat patients with central secondary amenorrhea,and to observe the induction of spontaneous ovulation and natural fertilization.Methods Using micro-pulse infusion pumps with each 90-minute infusion of LHRH 10 μg daily,the serum LH,FSH,estrogen,and progesterone levels ; and change in endometrium and ovarian size before and after treatment were monitored.The end point is natural pregnancy.Results The levels of serum LH,FSH,and estrogen were obviously increased after 4 weeks of treatment.The natural menstrual cycle was rebuilt after 8 weeks of treatment.The patient was pregnant and the treatment was stopped by 16 weeks.The experience of pregnancy was favourable.40 weeks later,the patient delivered a healthy female infant via caesarean section.Conclusions It was the first time in China that the technology of micro GnRH pump was applied in patients with secondary central amenorrhea.The result demonstrates that this technology can perfectly simulate the physiology of hypothalamic GnRH secretion.At present,more patients are included in this research to confirm the effectiveness.

Neurokinin B and its receptor in idiopathic hypogonadotropic hypogonadism / 中华内分泌代谢杂志

Idiopathic hypogonadotropic hypogonadism (IHH) is characterized by delayed or absent puberty and lowered sexual function as a result of impaired pulsatile gonadotropin-realeasing hormone (GnRH) secretion.Identification of TAC3/TACR3 mutations as the culprits of IHH revealed that neurokinin B (NKB) signaling pathway was involved in the regulation of pulsatile GnRH secretion.This review focuses on the involvement of NKB signaling in pulsatile GnRH release,the discovery of TAC3/TACR3 mutations and the phenotypes,and treatment of patients who carry TAC3 or/and TACR3 mutations.

Effect of glucocorticoid replacement therapy on glucose-lipid metabolism in patients with 21-hydroxylase deficiency / 中华内分泌代谢杂志

Objective21 -hydroxylase deficiency ( 21-OHD) patients are at high risk of developing metabolic syndrome.Low dose of glucocorticoid is crucial in the treatment.This study is to investigate the effect of glucocorticoid therapy on potential metabolic disorders.Methods Thirty-two treated and 31 untreated 21-OHD patients were recruited.The components of metabolic syndrome were investigated in both groups.Results Serum testosterone [ (0.61 ±0.12 vs 4.10±0.66) ng/ml,P＜0.01 ],17-(OH) progesterone[ 17-OHP,( 14.83±3.48 vs 48.52±4.72 )ng/ml,P＜0.01 ],dehydroepiandrosterone sulfate[ DHEAS,(55.7±23.6 vs 405.2±65.7 ) μg/dl,P＜0.01 ],and ACTH[ ( 105.8±44.7 vs 617.4± 163.3 ) pg/ml,P＜0.01 ] levels were significantly reduced,whereas body mass index [ ( 23.2±0.9 vs 21.1 ±0.5 ) kg/mz,P＜0.05 ],systolic blood pressure [ ( 120.5 ± 1.3 vs 115.5 ± 1.8 ) mm Hg,P＜0.05,1 mm Hg =0.133 kPa ],serum triglyceride [ ( 1.8±0.2 vs 1.1 ±0.1 ) mmol/L,P＜0.05 ],and homeostasis model assessment for insulin resistance [ HOMA-IR,( 2.07 ± 0.27 vs 1.16 ± 0.12 ),P ＜ 0.01 ] were markedly increased in glucocorticoid treated group.Multivariates regression analysis showed that body mass index was the most important risk factor for HOMA-IR.The correlation of glucocorticoid replacement and HOMA-IR was not observed after adjustment of age and body mass index.ConclusionGlucocorticoid treatment increases body weights,which leads to insulin resistance and metabolic disorders for 21-OHD patients.More attention should be paid to control BMI and metabolic disturbances in 21-OHD patients.

Perioperative management of high risk ACTH-independent macronodular adrenal hyperplasia: Report of one case / 中华内分泌代谢杂志

Objective To improve the diagnosis and treatment of ACTH-independent macronodular adrenal hyperplasia(AIMAH).Methods A 51-year-old female patient with Cushing's syndrome caused by AIMAH was reported.Elevated early morning plasma cortisol levels,increased 24 h urinary free cortisol excretion,and loss of the normal circadian rhythm in cortisol secretion were presented.There was no suppression of cortisol secretion by administration of low-and high-dose overnight dexamethasone suppression test.Cardio-pulmonary function was very bad with the highest blood pressure reaching 300/120 mm Hg( 1 mm Hg=0.133 kPa).Initially,she was treated with mitotane(60 mg/d),but was not effective.After taking ketoconazole (800 mg/d)for 5 days,cardio-pulmonary function was not effectively improved with blood pressure only descending to 180/120 mm Hg.Orthopnoea appeared and Spo2fell once to 75％.The patient had to undergo right total adrenalectomy immediately.ResultsThe mass resected was 10 cm× 10 cm in size and weighted 67.5 g.Histological examination of the removed adrenal revealed nonpigmented macronodular cortical hyperplasia.The patient continued to take ketoconazole (400-800 mg/d)from the 6th day of the operation without steroid replacement therapy in that period.With normal cortisol levels( plasma cortisol at 8:00 was 18.65 μg/dl,24 h urinary free cortisol was 78.75 μmol),she left hospital after the general condition had been improved.During the following updated 10 months follow up,the indexes of her laboratory examination were maintained normal.ConclusionIndividualized therapy should be adopted for the patient with AIMAH.The medication is useful to suppress the adrenal gland cortisol production for those with progression of symptoms,very high blood pressure,hypokalemia and hypoproteinemia.Once the cardio-pulmonary function improves,the target organ should be resected as soon as possible.The supplement of cortisol is not appropriate during the perioperative period.The unilateral adrenalectomy is an effective treatment for AIMAH.

A randomized, double blind, placebo-controlled, parallel and multicenter study to evaluate the safety and efficacy of pioglitazone with sulphonyurea in type 2 diabetic patients / 中华内科杂志

ObjectiveTo evaluate the safety and efficacy of 30 mg pioglitazone hydrochloride combined with sulphonyurea in the treatment of type 2 diabetic patients.MethodsA randomized, double blind, placebo-controlled, parallel group, multicenter study was performed.A total of 236 patients, who had fasting plasma glucose(FPG) 7.5-13.0 mmol/L and glycosylated hemoglobin A1c(HbA1 c) 7.0％ -12.0％,treated with stable dosage of a sulphonyurea for at least 30 days previously, were randomized to receive placebo or pioglitazone 30 mg once daily for 16 weeks.The sulphonyurea and dosage remained unchanged.ResultsThe patients who had been treated with pioglitazone 30 mg showed significant decrease than that in the placebo group on the average from baseline in FPG [(1.48 ±2.08) mmol/L vs (-0.17 ± 1.92)mmol/L, P＜0.05], and in HbAlc [(0.92 ±0.10)％ vs (0.28 ±0.11)％, P＜0.05].Since fasting plasma insulin (Flns) levels decreased (0.24 ±0.04) mU/L and (0.09 ±0.04) mU/L in the two groups.The homeostatic model assessment insulin resistant (HOMA-IR) decreased 1.42 ± 2.90 and 0.46 ± 3.53 in two groups.The triglyceride level was decreased 0.36 mmol/L and 0.14 mmol/L, and the HDL-C level increased 0.17 mmol/L and 0.05 mmol/L in two groups.There were significant differences in two groups (all P ＜ 0.05).ConclusionsThe 16-week clinical study demonstrated that pioglitazone hydrochloride with a dosage of 30mg daily, could significantly improve the blood glucose control and enhance the insulin sensitivity, lower triglyceride and raise HDL-C level as an additional therapy to a stable-dose sulphonyurea in Chinese type 2 diabetic patients previously poorly controlled by single sulphonyurea therapy, and furthermore had good safety and compliance.

A novel mutation of SRY gene identified in a 46,XY complete gonadal dysgenesis patient / 中华内分泌代谢杂志

The clinical and genetic characteristics in a patient with 46,XY complete gonadal dysgenesis was investigated. Clinical features and laboratory data were collected from the patient and the family. The exon of SRY gene was amplified by PCR and sequenced. The patient presented with primary amenorrhea, nonambiguous female external genitalia, slight breast development, and no axillary hair or pubic hair. The female internal reproductive organs consisted of uterus and streaks of ovarian tissue. Howerver, the chromosome karyotype was 46,XY. A missense mutation of A66T in SRY gene was identified, which was not previously reported. The novel SRY mutation caused the sex reversal in this 46,XY female patient.

A case with 21-hydroxylase deficiency combined with adrenal adenoma and testicular adrenal rest tumors / 中华内分泌代谢杂志

Objective To investigate the clinical and genetic characteristics in a male patient with 21hydroxylase deficiency combined with adrenal and testicular tumors.Methods Clinical features and laboratory data were collected from the patient.Testicular biopsy was performed.The CYP21 gene was sequenced for mutations.Results The patient presented left adrenal and testicular enlargements.The laboratory examinations showed that plasma ACTH,androstenedione,testosterone,progesterone,and 17-hydroxyprogesterone were markedly elevated.CT scan revealed that the right adrenal gland being resected and the left adrenal with nodular enlargement.Furthermore,testicular biopsy showed a prominent peritubular fibrosis with increased number of peritubular fibroblasts,tubular hyalinisation,and calcification.Sequencing analysis showed a A>G homozygous mutation at intron 2.Conclusion Patients with untreated 21-hydroxylage deficiency may.have adrenal adenomas and(or)testicular adrenal rest tumor simultaneously.

Treatment of idiopathic hypogonadotropic hypogonadism with pulse infusion of gonadorelin via micro pump / 中华内分泌代谢杂志

Objective To explore the efficacy of pulse infusion of gonadorelin (LHRH) on the patients with idiopathic hypogonadotropic hypogonadism (IHH) via a micro infusion pump. Methods The protocol was designed as an open, self-controlled prospective study. 31 patients were enrolled and assigned to 3 groups: 23 males without gonadotropin-releasing hormone ( GnRH ) pulse ( group A), 2 males with GnRH pulse frequency insufficiency ( group B), and 6 females ( group C). All the subjects were admitted LHRH every 90 min via the micro infusion pump for 24 weeks. Sex hormones and related characteristics were compared before and after the treatment. Results After 24-weeks treatment, LH peak value reached ( 6. 92 ±5.66 ), ( 9. 55 ±0. 98 ), and ( 6. 93 ±4. 52 ) IU/L; and FSH peak value reached ( 7.44 ± 3. 80 ), ( 12. 85 ± 12. 80 ), and ( 7.38 ±4. 98 ) IU/L among 3 groups, respectively. The testosterone also reached ( 3.18± 1.81 ) and ( 5.78±4. 65 ) ng/ml in groups A and B ( all P＜0. 01 ). In groups A and B, the testis volumes were increased, seminal fluid production was found in 7 patients and spermatogenesis in 6 patients. In group C, uterus was enlarged 85.4%, as well as the ovaries of both sides. Menarche was reported in 5 patients. 19. 4% of the studied patients complained uncomfortable at the injection sites, all the symptoms were mild. Conclusion Pulse infusion of LHRH in IHH patients via a micro infusion pump is effective, while the medication system needs improving.