Repairing a "broken heart" with hormone replacement therapy: case report of cardiogenic shock due to undiagnosed pituitary insufficiency

ObjectivesTo indicate cardiogenic shock as a very rare but serious clinical consequence of untreated panhypopituitarism attributable to Sheehan syndrome; to emphasize the importance of eliciting a detailed endocrine and obstetric history in women presenting with idiopathic heart failure; to highlight the diagnostic shortcomings of screening for thyroid dysfunction solely with thyroidstimulating hormone determinations; and to report the reversibility of severe heart failure induced by longterm pituitary insufficiency.MethodsDescribed is a case report of a 35yearold woman who presented with severe congestive heart failure, hypotension, and confusion. Her 2dimensional echocardiogram revealed appreciable systolic and diastolic dysfunction. In screening for possible endocrine causes of heart failure, a normal thyroidstimulating hormone level of 0.72 mIU/L (reference range, 0.35 to 5.5) was unremarkable; however, a profoundly low free thyroxine level of 0.12 ng/dL (reference range, 0.9 to 1.8) led clinicians to pursue a workup of central hypothyroidism.ResultsEndocrine testing confirmed the presence of panhypopituitarism and adrenal insufficiency. Magnetic resonance imaging of the brain revealed empty sella syndrome. Further questioning of the patient revealed a history of extensive postpartum bleeding 15 years earlier, failure to lactate, and secondary amenorrhea—all consistent with undiagnosed Sheehan syndrome. In the hospital, the patient was treated with intravenously administered corticosteroids and levothyroxine. Her mental status and symptomatic heart failure improved dramatically. After 9 months of oral levothyroxine and glucocorticoid therapy, the patient remained asymptomatic, and repeated echocardiography indicated completely normalized cardiac function.ConclusionSevere heart failure and cardiogenic shock can be a very rare (but fortunately reversible) complication of longstanding panhypopituitarism resulting from undiagnosed Sheehan syndrome. (Endocr Pract. 2012;18:e26e31)     

Long-Standing Undiagnosed Sheehan Syndrome Presenting as Polymorphic and Monomorphic Ventricular Tachycardia: A Case Series of 2 Patients

ObjectiveTo describe 2 cases of Sheehan syndrome presenting with ventricular tachycardia.MethodsIn this case series, we present 2 cases of Sheehan syndrome presenting with ventricular tachycardia, which is an extremely rare complication of Sheehan syndrome. We review the literature for cases of panhypopituitarism presenting with ventricular tachycardia and also review the pathophysiologic mechanisms underlying development of ventricular tachycardia in these patients.ResultsTwo female patients presented with ventricular tachycardia. One patient had monomorphic and the other had polymorphic ventricular tachycardia. On further workup, both patients were found to have panhypopituitarism. Due to past history of postpartum hemorrhage, both patients were suspected of having Sheehan syndrome as the cause of panhypopituitarism. Electrocardiogram revealed prolonged QT interval. Both patients were started on hormone replacement therapy. Both patients responded well initially and were discharged home. One of the patients is alive and healthy at the time of this report. However, the other patient was readmitted with seizures a few days after discharge and unfortunately died of sudden cardiac arrest.ConclusionUntreated cases of Sheehan syndrome can present with fatal ventricular tachycardia. Hormone replacement in these patients can treat and prevent fatal arrhythmias. (Endocr Pract. 2014;20:e211-e214)     

Should We Use Prolactin Adjustment in Bilateral Inferior Petrosal Sinus Sampling to Diagnose Cushing Disease? A Joint Meta-Analysis of Head-to-Head Diagnostic Tests Accuracy Studies

ObjectivePrevious studies have shown inconsistent results regarding the usefulness of prolactin adjustment in bilateral inferior petrosal sinus sampling (BIPSS) in the differential diagnosis of adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome (CS). This meta-analysis compared the diagnostic accuracy of prolactin adjustment versus no adjustment in BIPSS.MethodsThis study searched the PubMed, Embase, Web of Science, Cochrane library, and WanFang databases for published data as of March 2022 on the use of prolactin adjustment in BIPSS to differentially diagnose ACTH-dependent CS. A Bayesian joint bivariate model was used in the head-to-head comparison of the diagnostic accuracy.ResultsThis meta-analysis included a total of 10 studies with 300 patients. The combined sensitivity and specificity for BIPSS without prolactin adjustment were 94.47% (95% CI, 88.67%-98.44%) and 91.14% (95% CI, 57.17%-99.80%), respectively. The combined sensitivity and specificity after prolactin adjustment were 99.97% (95% CI, 99.03%-100.00%) and 80.69% (95% CI, 50.27%-97.82%), respectively. After the prolactin adjustment, the sensitivity of BIPSS to diagnose Cushing disease was improved with a statistically significant difference (difference in sensitivity, 5.39%; 95% CI, 1.37%-11.26%), whereas the decrease in specificity was without a statistically significant difference (difference in specificity, −9.20%; 95% CI, −42.02% to 26.61%).ConclusionThis meta-analysis indicated that prolactin adjustment in BIPSS might help to recognize Cushing disease among patient with ACTH-dependent CS. However, prolactin adjustment may not help identify ectopic ACTH syndrome in BIPSS.     

Presentation of 493 Consecutive Girls with Idiopathic Central Precocious Puberty: A Single-Center Study

Background

Despite the number of reported data concerning idiopathic central precocious puberty (CPP) in girls, major questions remain including its diagnosis, factors, and indications of gonadotropin releasing hormone (GnRH) analog treatment.

Methods

A retrospective, single-center study was carried out on 493 girls with CPP.

Results

Eleven girls (2.2%) were aged less than 3 years. Breast development was either isolated (Group 0, n = 99), or associated with one sign, pubic hair development, growth rate greater than 2 standard deviation score (SDS) or bone age (BA) >2 years above chronological age, (Group 1, n = 187), two signs (Group 2, n = 142) or three signs (Group 3, n = 65). The interval between onset of puberty and evaluation, body mass index (BMI) SDS, plasma luteinising hormone (LH) concentrations (basal and peak) and LH/ follicle-stimulating hormone (FSH) peak ratio after GnRH test, plasma estradiol and uterus length were significantly greater in Groups 2 and 3 than in Groups 0 and 1 respectively. 211 (42.8%) patients were obese and/or had excessive weight gain during the year before puberty. Obese girls more often had BA advance of >2 years (p = 0.0004) and pubic hair development (p = 0.003) than the others. BMI did not correlate with LH or with LH/FSH peak ratio. Girls with familial history of early puberty (41.4%) had greater frequencies of pubertal LH/FSH peak ratios (p = 0.02) than the others. During the 31 years of the study, there was no increase in the frequency of CPP or variation in its characteristics.

Conclusion

Obesity is associated with a higher BA advance and higher frequency of pubic or axillary hair development but not with LH secretion, suggesting that obesity accelerates adrenarche but not the maturation of the hypothalamic-pituitary-ovarian axis. The LH/FSH peak ratio was more frequently pubertal in girls with a familial history of early puberty, suggesting that this maturation depends on genetic factors.     

Some Clues and Pitfalls in the Diagnosis of Acromegaly

ObjectiveTo describe a case of acromegaly in a young woman using oral contraceptives who had nonspecific symptoms and persistent hyperphosphatemia and to analyze the reasons for delay in diagnosis.MethodsA 31-year-old woman underwent evaluation for failure of menstrual flow to resume after discontinued use of oral contraceptives. Clinical and laboratory findings are summarized, and atypical manifestations of acromegaly are discussed.ResultsThe patient complained of weight gain, acne, and increased facial hair growth, and laboratory tests showed a low estradiol level and hyperinsulinemia. “Postpill amenorrhea” and polycystic ovary syndrome were considered possible diagnoses. During subsequent endocrinology consultation, hyperphosphatemia was present on three occasions during a 7-month period. Hormonal studies for evaluation of pituitary function revealed increased insulin-like growth factor-I and growth hormone (GH) levels. Acromegaly was suspected, and the increased GH level failed to suppress after a glucose load. Magnetic resonance imaging revealed a pituitary macroadenoma with suprasellar extension. Visual field testing showed bitemporal abnormalities. Transsphenoidal resection successfully removed the GH-producing tumor, and hormone replacement therapy was initiated postoperatively.ConclusionThe textbook presentation of GH excess is a late feature of acromegaly. The diagnosis in a young woman can be easily missed when clinicopathologic findings are not classic and the picture is further clouded by factors such as oral contraceptive use. Features may also resemble polycystic ovary syndrome, a more common condition in women of childbearing age. This case also illustrates that hyperphosphatemia, overlooked in this patient, can precede the full-blown clinical manifestations of acromegaly and serve as a subtle clue to the underlying disease. Awareness of these situations and screening for high GH levels in patients with unexplained, persistent hyperphosphatemia are advisable for making an early correct diagnosis and providing appropriate therapy. (Endocr Pract. 2004;10:348-352)     

Sheehan Syndrome Presenting as Central Diabetes Insipidus: A Rare Presentation of an Uncommon Disorder

ObjectiveTo add to the current scant literature on rare clinical presentations of Sheehan syndrome.MethodsWe describe the study patient’s clinical, laboratory, and imaging findings and review the literature for publications regarding varied clinical presentations of Sheehan syndrome.ResultsA 36-year-old multigravida woman developed severe postpartum hemorrhage and disseminated intravascular coagulation followed by Sheehan syndrome, with central diabetes insipidus as the primary presenting feature. This was diagnosed when, postoperatively, she developed polyuria with a urine output of 11 L in 24 hours with an accompanying rise in creatinine. She had laboratory evidence of diabetes insipidus, with serum osmolality greater than urine osmolality. Her clinical status improved significantly with intranasal desmopressin supplementation, thus confirming the diagnosis of Sheehan syndrome. Although Sheehan syndrome is a known complication of postpartum hemorrhage, central diabetes insipidus is seldom considered or suspected. Hypovolemia is usually presumed to be secondary to blood loss and polyuria resulting from a diuretic phase of acute renal failure.ConclusionsIt is important to consider posterior pituitary ischemia resulting from Sheehan syndrome presenting as central diabetes insipidus as a cause of polyuria because appropriate hormonal replacement initiated early can possibly improve clinical status and patient outcomes. (Endocr Pract. 2011;17:108-114)     

46,XX SRY-Positive Male Syndrome Presenting with Primary Hypogonadism in the Setting of Scleroderma

ObjectiveTo describe a case of SRY gene translocation in a man with scleroderma presenting with primary hypogonadism.MethodsWe present the clinical, physical, laboratory, and pathologic findings of the study patient and discuss the cytogenetic analysis and the cause of the sexual dysfunction. Relevant literature is reviewed.ResultsA 35-year-old man with a recent diagnosis of diffuse cutaneous sclerosis was referred by his rheumatologist because of a low testosterone level. His medical history was notable for right cryptorchidism corrected after birth. He had no history of sexual activity, but reported normal erectile function before his current presentation. Physical examination findings were remarkable for a height of 157.5 cm; weight of 72.7 kg; extensive, diffuse thickening of the skin; mild gynecomastia; little axillary and pubic hair; and soft testes (1-2 mL bilaterally). Initial laboratory testing revealed the following values: follicle-stimulating hormone, 22.1 mIU/mL (reference range, 1.4-18.1 mIU/mL); luteinizing hormone, 19.7 mIU/mL (reference range, 1.5-9.3 mIU/mL); total testosterone, 25 ng/dL (reference range, 241-827 ng/dL); and free direct testosterone, 0.8 pg/mL (reference range, 8.7-25.1 pg/mL). Laboratory test results were consistent with primary hypogonadism. A urologist performed testicular biopsy, which showed severe testicular atrophy with absent spermatogenesis. Primary hypogonadism due to Klinefelter syndrome or testicular fibrosis secondary to scleroderma was suspected. Karyotype analysis showed a 46,XX karyotype, and fluorescence in situ hybridization was consistent with a 46,XX,Xp22.3(SRY +) gene translocation. After a normal prostate-specific antigen level was documented, testosterone replacement therapy was initiated, and he was referred for genetic counseling.ConclusionsThe 46,XX SRY-positive male syndrome is rare. Adult diagnosis can be challenging because of normal sexual development. Scleroderma, which rarely can occur in Klinefelter-type syndromes, further complicated the diagnosis in this case. (Endocr Pract. 2011;17:95-98)     

Endocrine Dysfunction in A Patient with Phace Syndrome,Including Port-Wine Stain of the Right Periorbital Area

ObjectiveTo report a case of PHACE syndrome— Posterior fossa brain abnormalities, Hemangioma (usually facial), Arterial anomalies, Coarctation of the aorta along with cardiac defects, and Eye abnormalities—in a 16-yearold female patient with a port-wine stain of the right periorbital area present since birth in conjunction with hypoplasia of the contralateral internal carotid artery.MethodsThyroid-stimulating hormone, free thyroxine, and growth hormone (GH) levels were measured, and insulin-induced hypoglycemia and arginine infusion tests were done. Radiologic investigations included sagittal enhanced T1-weighted magnetic resonance imaging of the brain and the pituitary gland as well as computed tomography and magnetic resonance angiography of the head and neck.ResultsThe patient had a normal karyotype. Her height and weight were below the 5th percentile for her chronologic age, and she had amenorrhea. Laboratory investigations revealed both thyroid and GH deficiencies and confirmed the diagnosis of hypogonadotropic hypogonadism. The imaging studies showed a right intraorbital hemangioma as well as an enhancing mass in the right internal auditory canal at the cerebellopontine angle, consistent with a posterior fossa hemangioma. Initiation of both thyroid and GH replacement therapy improved her growth rate and yielded a good clinical outcome.ConclusionIn patients with facial or neck hemangiomas, PHACE syndrome should be suspected, and brain imaging and cardiac, ophthalmologic, and endocrinologic evaluations are recommended to screen for other potential PHACE abnormalities. (Endocr Pract. 2010;16:255-259)     

化疗对乳腺癌患者激素水平及月经状态的影响

目的:讨论乳腺癌患者术后辅助化疗对患者激素水平及月经状况的影响。方法:收集我院2014年1月-2015年8月初诊绝经前乳腺癌患者78例,绝经后乳腺癌患者50例,检测化疗前及化疗结束后的雌二醇(E2)、黄体生成素(LH)、卵泡刺激素(FSH)水平,随访绝经前乳腺癌患者化疗期间及化疗后月经变化情况。结果:绝经前乳腺癌患者化疗后E2水平明显下降,FSH、LH水平明显升高,差异具有统计学意义(P0.05),绝经后乳腺癌患者化疗后E2水平无明显变化(P0.05),FSH、LH水平均下降,差异具有统计学意义(P0.05)。绝经前乳腺癌患者三个不同年龄段化疗后E2水平降低,而FSH、LH水平升高,差异具有统计学意义(P0.05),但三个不同年龄段患者化疗前后性激素水平组间比较均无统计学差异(P0.05)。绝经前乳腺癌患者三个不同年龄段化疗后闭经率比较差异具有统计学意义(P0.05)。绝经前乳腺癌患者化疗后闭经患者E2水平明显低于未闭经患者,FSH、LH水平明显高于未闭经患者,差异具有统计学意义(P0.05)。结论:化疗可影响乳腺癌患者E2、FSH、LH水平,导致患者闭经,闭经情况与患者年龄有关。     

Assessment the Correlations of Hormones,Lipid Profiles,Oxidative Stress,and Zinc Concentration in Iraqi Women with Polycystic Ovary Syndrome

Background: Polycystic ovary syndrome (PCOS) is a hormonal disorder in women with unknown causes and is the leading cause of infertility in women of reproductive age, presenting a wide range of clinical manifestations worldwide. The objective of study is to compare the correlation between hormones, lipid profile, oxidative stress and Zinc concentration in PCOS patients. Methods:The present study examined hormone levels (progesterone, prolactin, luteinizing and follicle stimulation hormones (LH and FSH, respectively), antioxidant factors (catalase, glutathione-s- transferase), lipid profiles and zinc concentration of 50 Iraqi women patients’ diagnosis with PCOS and 40 healthy women, divided in two age groups of 15-29 and 30-45 years. Body mass index was estimated for two age groups.Results:The results showed decreasing of catalase, glutathione, and Zn concentrations with an increase in age. A slightly significant increase in LH and prolactin and decrease in high-density lipoprotein (HDL-C) with an increase in age in the patient group compared to the control group was noted. Conclusion:Our study demonstrated that some factors (such as family history, genetics, environmental, etc…) could play a role in altering hormone levels, lipid profiles, and antioxidant. Controlling these factors may be useful for reducing the PCOS-associated problems in women’s health. Needed extensive studies to assess the correlation with insulin resistant and obesity.Key Words: Hormones disorders, Lipid profile, Polycystic ovary syndrome, Zinc element      

Mucocolpos In A Toddler: Central Precocious Puberty With Vaginal Atresia

ObjectiveTo report the first case of imperforate hymen and vaginal atresia in a patient with mucocolpos during toddlerhood who was found to have central preco cious puberty.MethodsWe review the details of assessment of an 18-month-old girl who had the presence of pubic hair and breast enlargement. She underwent biochemical evaluation with serum follicle-stimulating hormone, luteinizing hor mone, and estradiol and radiologic evaluation with ultra sonography of the abdomen and pelvis as well as magnetic resonance imaging of the pelvis and brain.ResultsThis young female patient had clinical and imaging findings suggestive of idiopathic central preco cious puberty. Imaging also revealed imperforate hymen, vaginal atresia, and mucocolpos. She underwent surgical treatment to ensure an unobstructed vaginal opening before initiation of gonadotropin-releasing hormone agonist ther apy, since the latter may precipitate uterine bleeding and might have converted mucocolpos to a combination of mucocolpos and hematocolpos.ConclusionThis case highlights the need to suspect the presence of precocious puberty in all female patients in whom mucocolpos is detected beyond infancy but before adolescence. (Endocr Pract. 2012;18:e144-e146)     

Positive Prolactin Response to Bromocriptine in 2 Patients with Cabergoline-Resistant Prolactinomas

ObjectiveTo describe a positive prolactin response to bromocriptine treatment in 2 patients with cabergolineresistant prolactinomas.MethodsWe report the patients’ clinical presentations, laboratory test results, imaging findings, and clinical courses.ResultsPatient 1 had a 5-mm pituitary microadenoma that was initially diagnosed at age 30 years. After initial diagnosis, she was treated with transvaginal bromocriptine for 9 years and then subsequently went untreated for 2 years. After developing symptoms of amenorrhea, decreased libido, and hyperprolactinemia, oral cabergoline, 0.5 mg twice weekly, was initiated. Her prolactin concentration remained elevated at 80 ng/mL while taking cabergoline. Her prolactin concentration decreased to 13 ng/mL after her regimen was switched to bromocriptine, 5 mg daily. Patient 2 had a 17-mm pituitary macroadenoma that was initially diagnosed at age 15 years. Oral cabergoline was started at 0.5 mg twice weekly and increased to 1 mg 3 times weekly when prolactin levels continued to rise to 340 ng/mL over 18 months. After visual field defects developed, transsphenoidal surgery was performed. One year after surgery, magnetic resonance imaging showed a 6-to 7-mm pituitary adenoma, and there was a gradual rise in serum prolactin. Her serum prolactin concentration continued to rise to 212 ng/mL with increasing tumor size over 3 years. Cabergoline was discontinued and oral bromocriptine was initiated at a dosage of 10 mg daily. After 4.5 months of bromocriptine therapy, her serum prolactin concentration decreased to 133 ng/mL. However, after 2 months, the macroadenoma continued to increase in size and a visual field defect developed, so another transsphenoidal operation was performed.ConclusionsAlthough cabergoline is generally preferred to bromocriptine for the treatment of patients with prolactinomas because of its better tolerance profile and greater effectiveness, in patients with cabergoline-resistant prolactinomas, a bromocriptine trial should be considered a safe, relatively inexpensive, and well-tolerated alternative. (Endocr Pract. 2011;17:e55-e58)     

Revisiting Cushing Syndrome,Milder Forms Are Now a Common Occurrence: A Single-Center Cohort of 76 Subjects

ObjectiveGuidelines recommend initiating testing for Cushing syndrome (CS) based on strong clinical suspicion. Our recent experience suggests the absence of classical stigmata in the majority of patients with CS. We aimed to confirm this premise by revisiting the clinical features of this syndrome in a modern series of patients from a single center.MethodsComputerized records of subjects with CS diagnosed at Tel Aviv Sourasky Medical Center between 2000 and 2018 were reviewed. A Cushing inventory score, including all clinical components of the syndrome, was computed for each subject. Data were compared between the subtypes and evaluated in light of those in the literature.ResultsOf the 76 subjects with CS (60 women/16 men), 49 (64.5%) had Cushing disease; 16 (21.1%), adrenal adenoma; 7 (9.2%), adrenocortical carcinoma; and 4 (5.3%), ectopic adrenocorticotropic hormone secretion. In only 15 of 74 cases (20.3%), clinical suspicion of CS led to testing. Catabolic signs of CS were present in less than 30% of cases. The most common symptom was weight gain (52/67, 77.6%), and the most common comorbidity was hypertension (47/76, 61.8%). There were no differences in the Cushing inventory score between the subtypes. Signs, symptoms, and comorbidities were all significantly less common than in the classical syndrome.ConclusionsModern-day CS presents with subtler features than in the past. Initiating a testing cascade solely based on a strong clinical suspicion may lead to underdiagnosis of milder cases. A concerted effort to devise cost-efficient testing for CS in the current era is needed.     

Glucose-6-Phosphate Dehydrogenase Deficiency and Physical and Mental Health until Adolescence

BackgroundTo examine the association of glucose-6-phosphate dehydrogenase (G6PD) deficiency with adolescent physical and mental health, as effects of G6PD deficiency on health are rarely reported.MethodsIn a population-representative Chinese birth cohort: “Children of 1997” (n = 8,327), we estimated the adjusted associations of G6PD deficiency with growth using generalized estimating equations, with pubertal onset using interval censored regression, with hospitalization using Cox proportional hazards regression and with size, blood pressure, pubertal maturation and mental health using linear regression with multiple imputation and inverse probability weighting.ResultsAmong 5,520 screened adolescents (66% follow-up), 4.8% boys and 0.5% girls had G6PD deficiency. G6PD-deficiency was not associated with birth weight-for-gestational age or length/height gain into adolescence, but was associated with lower childhood body mass index (BMI) gain (-0.38 z-score, 95% confidence interval (CI) -0.57, -0.20), adjusted for sex and parental education, and later onset of pubic hair development (time ratio = 1.029, 95% CI 1.007, 1.050). G6PD deficiency was not associated with blood pressure, height, BMI or mental health in adolescence, nor with serious infectious morbidity until adolescence.ConclusionsG6PD deficient adolescents had broadly similar physical and mental health indicators, but transiently lower BMI gain and later pubic hair development, whose long-term implications warrant investigation.     

A Model Combining Testosterone,Androstenedione and Free Testosterone Index Improved the Diagnostic Efficiency of Polycystic Ovary Syndrome

ObjectiveHyperandrogenism is frequently observed in patients with polycystic ovary (PCO). The purpose of this study was to develop an easy-to-use tool for predicting polycystic ovary syndrome (PCOS) and to evaluate and compare the value of androstenedione (Andro) and other hormone indicators in the diagnosis of patients with hyperandrogenic PCOS.MethodsThis study included 139 women diagnosed with hyperandrogenic PCOS according to the Rotterdam criteria and 74 healthy control women from Shanghai Tenth People's Hospital. The serum hormone levels of the patients and controls were measured using a chemiluminescence immunoassay and incorporated for further analysis.ResultsTotal testosterone (TT), Andro, dehydroepiandrosterone sulfate (DHEAS), and free androgen index (FAI) were significantly higher in the PCOS group than the control group. Further, Andro, follicle-stimulating hormone (FSH), luteinizing hormone (LH), TT, FAI, and LH/FSH in the hyperandrostenedione group were higher than the normal Andro group. The Youden index was the highest for Andro (0.65), with 81.82% sensitivity and 83.16% specificity. Correlation analysis showed that FSH, LH, TT, FAI, insulin sensitivity index, and LH/FSH were positively correlated with Andro, while fasting blood glucose and 2-hour postprandial blood glucose were negatively correlated with Andro.ConclusionsThe model using Andro, TT, and FAI may help to identifying women with undiagnosed PCOS. Serum Andro is a meaningful biomarker for hyperandrogenism in PCOS patients and may further aid disease diagnosis.     

多囊卵巢综合征患者血清抗苗勒管激素与肥胖、胰岛素抵抗程度的相关性分析

目的:探讨多囊卵巢综合征(polycystic ovary syndrome,PCOS)患者血清抗苗勒管激素(anti-Müllerian hormone,AMH)水平与肥胖、胰岛素抵抗(insulin resistance,IR)程度的相关性。方法:选择在我院生殖中心就诊的139名PCOS患者为研究组,并以月经周期正常、因输卵管因素或男性因素导致不孕者48名作为对照组。检测和比较PCOS患者的血清AMH、性激素水平及代谢指标,分析血清AMH水平与PCOS患者肥胖、胰岛素抵抗程度的关系。结果:PCOS组患者体质量指数(body mass index,BMI)、黄体生成素(luteinizing hormone,LH)、睾酮(testosterone,T)、垂体泌乳素(pituitary prolactin PRL)、空腹血糖(fasting plasma glucose,FPG)、空腹胰岛素(fasting insulin,FINS)、稳态模型胰岛素抵抗指数(homenostasis models assessment-insulin resistance index,HOMA-IR)的水平均显著高于对照组(P0.05),PCOS组和对照组年龄、卵泡刺激素(follicle stimulating hormone,FSH)比较差异无统计学意义(P0.05)。PCOS各表型组的血清AMH浓度、LH/FSH比值均明显高于对照组(P0.05)。肥胖组患者的AMH浓度低于正常体重组,BMI、FPG、FINS、HOMA-IR、甘油三脂(triglycerides,TG)水平均高于正常体重组,LH、LH/FSH、高密度脂蛋白(high density lipoprotein,HDL-C)水平均低于正常体重组(P0.05)。高HOMA-IR组患者的血清AMH浓度、LH、LH/FSH水平均明显低于低HOMA-IR组,BMI、T、FPG、FINS、TG、低密度脂蛋白(low density lipoprotein,LDL-C)水平均高于低HOMA-IR组(P0.05)。PCOS患者血清AMH浓度和BMI及HOMA-IR均存在显著负相关。结论:PCOS患者血清的AMH水平较对照组明显升高,与其肥胖、胰岛素抵抗(IR)程度呈显著负相关。     

Effect of diethylstilbesterol and prolactin on the induction of follicle stimulating hormone receptors in immature and cycling rats

Induction of follicle stimulating hormone receptor in the granulosa cells of intact immature rat ovary by diethylstilbesterol, an estrogen, has been studied. A single injection of 4 mg of diethylstilbesterol produced 72 h later a 3-fold increase in follicle stimulating hormone receptor concentration as monitored by [¹²⁵I]-_oFSH binding to isolated cells. The newly induced receptors were kinetically indistinguishable from the preexisting ones, as determined by Lineweaver-Burk plot of the binding data. The induced receptors were functional as evidenced by increased ability of the granulosa cells to incorporate [³H]-leucine into cellular proteins. Neutralization of endogenous follicle stimulating hormone and luteinizing hormone by administering specific antisera had no effect on the ability of diethylstilbesterol to induce follicle stimulating hormone receptors, whereas blockade of endogenous prolactin secretion by ergobromocryptin administration significantly inhibited (∼ 30 %) the response to diethylstilbesterol; this inhibition could be completely relieved by ovine prolactin treatment. However, ovine prolactin at the dose tried did not by itself enhance follicle stimulating hormone receptor level. Administration of ergobromocryptin to adult cycling rats at noon of proestrus brought about as measured on diestrusII, (a) a reduction of both follicle stimulating hormone (∼ 30 %) and luteinizing hormone (∼ 45 %) receptor concentration in granulosa cells, (b) a drastic reduction in the ovarian tissue estradiol with no change in tissue progesterone and (c) reduction in the ability of isolated granulosa cells to convert testosterone to estradiol in response to follicle stimulating hormone. Ergobromocryptin treatment affected only prolactin and not follicle stimulating hormone or luteinizing hormone surges on the proestrus evening. Treatment of rats with ergobromocryptin at proestrus noon followed by an injection of ovine prolactin (1 mg) at 1700 h of the same day completely reversed the ergobromocryptin induced reduction in ovarian tissue estradiol as well as the aromatase activity of the granulosa cells on diestrus II, thus suggesting a role for proestrus prolactin surge in the follicular maturation process     

Associations of Birth Order with Early Adolescent Growth,Pubertal Onset,Blood Pressure and Size: Evidence from Hong Kong’s “Children of 1997” Birth Cohort

BackgroundBirth order has been proposed as a cardiovascular risk factor, because the lower birth weight and greater infant weight gain typical of firstborns could programme metabolism detrimentally.MethodsWe examined the associations of birth order (firstborn or laterborn) with birth weight-for-gestational age, length/height and body mass index (BMI) z-scores during infancy, childhood, and puberty using generalized estimating equations, with age at pubertal onset using interval-censored regression and with age-, sex- and height-standardized blood pressure, height and BMI z-scores at 13 years using linear regression in a population-representative Chinese birth cohort: “Children of 1997” (n = 8,327).ResultsCompared with laterborns, firstborns had lower birth weight-for-gestational age (mean difference = -0.18 z-score, 95% confidence interval (CI) -0.23, -0.14), lower infant BMI (-0.09 z-score, 95% CI -0.14, -0.04), greater childhood height (0.10 z-score, 95% CI 0.05, 0.14) and BMI (0.08 z-score, 95% CI 0.03, 0.14), but not greater pubertal BMI (0.05 z-score, 95% CI -0.02, 0.11), adjusted for sex, parental age, birthplace, education and income. Firstborns had earlier onset of pubic hair (time ratio = 0.988, 95% CI 0.980, 0.996), but not breast or genitalia, development. Firstborns had greater BMI (0.07 z-score, 95% CI 0.002, 0.15), but not height (0.05 z-score, 95% CI -0.01, 0.11), at 13 years, but similar blood pressure.ConclusionsDifferences by birth order continue into early adolescence with firstborns being heavier with earlier pubic hair development, which could indicate long-term cardiovascular risk.     

Experience of Ectopic Adrenocorticotropin Syndrome: 88 Cases With Identified Causes

ObjectiveEctopic adrenocorticotropic hormone syndrome (EAS) is a rare cause of Cushing's syndrome and diagnosis and management remain challenging. The aim of this study was to present the clinical spectrum of a group of EAS cases in a single center to explore better management strategies.MethodsA retrospective study was conducted to identify 88 confirmed EAS cases at our hospital from 1984 to 2019. The clinical, biochemical, imaging, and pathological features were analyzed.ResultsOf the 88 eligible patients with EAS, 38 (43.2%) cases of pulmonary neuroendocrine tumors (NETs) and a larger number of thymic/mediastinal NETs (29 cases, 33%) were identified. The clinical and biological features of EAS and Cushing's disease overlapped but were more severe in EAS. Inferior petrosal sinus sampling (97.4%) and computed tomography (85.4%) provided the highest positive diagnostic accuracy. Computed tomography is also a useful tool to identify tumors in chest cavity compared with nonchest lesions (91.2% vs 57.1%). Although a greater tumor size (4.54 cm vs 1.44 cm) and higher rate of insuppressible high-dose dexamethasone suppression test (83.3% vs 51.5%) were found in thymic/mediastinum NETs than in pulmonary NETs, the level of hormone production had no difference.ConclusionEAS had more common and severe clinical presentations than Cushing's disease, and multiple imaging approaches are required for reliable diagnosis. A higher proportion of thymic/mediastinal NETs was found in our study. For patients without a certain tumor source, long-term follow-up and further evaluations are needed.     

Eventos adversos perinatales y anomalías neuroanatómicas en pacientes con hipopituitarismo idiopático

ObjectiveTo analyze the possible causes of growth hormone (GH) deficiency, whether isolated (GHD) or in combination with other pituitary deficiencies classified as idiopathic.Patients and methodsWe studied patients with idiopathic GHD included in a protocol of recombinant GH treatment in adults attending the outpatient clinic of the Endocrinology and Nutrition Service of the San Cecilio University Hospital. Perinatal history, findings on magnetic resonance imaging (MRI) of the hypothalamic-pituitary axis and diagnosis of GHD and other deficiencies were retrospectively evaluated.ResultsA total of 17 patients were included: 14 men and 3 women with a mean age at diagnosis of 8.4±7.3 years. Perinatal adverse events occurred in 12 patients (69.2%). MRI showed empty sella (2 patients), pituitary hypoplasia or absence of the pituitary stalk (7 patients) and pituitary hypoplasia with ectopic posterior pituitary gland (6 patients); in the remaining 2 patients these data were not available. All had an established diagnosis of GHD: 15 with (88.2%) gonadotropin deficiency, 9 (52.9%) with adrenocorticotropic hormone (ACTH) deficiency and 8 (47.1%) with thyroid-stimulating hormone (TSH) deficiency.ConclusionsIn our patients, adverse events during pregnancy or the perinatal period and the presence of anatomical abnormalities identified by MRI are a marker of pituitary dysfunction and may be important in the pathogenesis of this entity. The clinical spectrum of disease varies from isolated GH deficiency to multiple pituitary hormone deficiencies.