Growth without growth hormone (GH): a case report

Growth without growth hormone (GH) has occasionally been described in patients with organic pituitary pathology, and even more rarely in patients with idiopathic pituitary hormone deficiency. The mechanism of growth without GH remains a mystery. We describe a 17-year old male who grew 38.5 cm in height over a 7-year period, despite the fact that he had established panhypopituitarism. The hypopituitarism was initially attributed to a presumptive hypothalamic hamartoma which was not, however, confirmed on subsequent and prolonged follow-up. Regular endocrine evaluation confirmed persistent anterior and posterior pituitary hormonal deficiencies with severe concomitant hyperinsulinemia as shown by an exaggerated insulin response to a standard oral glucose tolerance test. In our patient, a postulated mechanism could be the severe hyperinsulinemia, acting either through the insulin and/or IGF receptors and thus potentiating the mitogenic effect. This case illustrates that final height attainment within or above target height may occur in patients with idiopathic pituitary hormonal deficiency despite persistent, severe GH insufficiency.     

Aspergillosis of the sphenoid sinus: presentation as a pituitary mass

A rare manifestation of aspergillosis in the central nervous system is its invasion through the sphenoidal wall into the sella turcica representing itself as a pituitary mass. The symptoms may be headache, visual defect caused by compression of the chiasma, hypopituitarism and diabetes insipidus. In the majority of cases only the postoperative histology leads to the correct diagnosis. A case of invasive aspergillosis was reported here with the clinical picture of a pituitary tumor and without underlying immunodeficiency.     

A prismatic case: A 31-year old man who did not miss his pituitary

Combined pituitary hormone deficiency (CPHD) is a rare disorder resulting from impaired production of several pituitary hormones. This report describes the 30-year history of a patient who has apparently lived without a pituitary. The patient, born in 1976, experienced recurrent episodes of hypoglycaemia (as low as 2.9 mg/dl) in early childhood and showed elevated liver enzymes up to the age of two years without specific diagnosis. At the age of approximately 13 years, he first presented at our Department of Paediatric Endocrinology (University Hospital for Children and Adolescents, Leipzig) with signs of hypopituitarism and a height SDS of - 5.4, a high pitched voice and hypogenitalism. Endocrine testing confirmed panhypopituitarism (GH 1.3 ng/mL, LH 0.8 mU/mL, FSH 0.1 mU/ml, ACTH <1.0 pmol/l, TSH 1.0 mU/l) and substitution therapy was initiated. Magnetic resonance imaging (MRI) of the sella turcica showed a planar, hypoplastic and empty sella with pituitary stalk aplasia and ectopic neurohypophysis. Currently nearly 31 years of age, though taking his medication irregularly or not at all, he claims to be in good mental and physical condition irrespective of compliance or non compliance with substitution therapy. This case illustrates the importance of continued follow-up in patients with hypopituitarism and a systemic transferral of adolescents with CPHD to the care of adult endocrinologists.     

Pituitary apoplexy: an endocrinologic emergency

A 32-year-old woman, a 73-year-old man and a 26-year-old pregnant woman presented with headache, vomiting, and variable presence of visual disturbances, impaired consciousness, and circulatory shock. All three had pituitary apoplexy. In the first patient lymphocytic hypophysitis was diagnosed, the second had a nonfunctional adenoma and the last patient probably also. All three patients were treated conservatively with full recovery of signs and symptoms. However pituitary insufficiency remained in all three. Pituitary apoplexy is an acute event with significant morbidity and mortality. The cause is a rapid expansion of, usually, a pre-existing, often not yet known, adenoma by massive haemorrhage or infarction. Pressure, among other things, causes hypopituitarism, meningism, compression of the chiasma opticum and visual disturbances. Although many predisposing factors have been identified, pituitary apoplexy is often an unpredictable event. Diagnosis is made by the typical clinical presentation, eye examination, MRI and by measuring pituitary hormones. Treatment is with suppletion of the deficient hormones and in selected patients by transsphenoidal decompression surgery.     

Radiotherapy of pituitary tumours

In this article, the role of conventional radiotherapy and radiosurgery in the management of pituitary tumors is discussed. After a brief review about the mechanism of action and different techniques of irradiation therapy, the therapeutic effectiveness and side effects are analysed in the various types of pituitary tumors. Conventional fractionated radiotherapy has long been used to control growth and/or hormonal secretion of residual or recurrent pituitary tumors. Nevertheless, there is still a controversy concerning patient selection for radiotherapy, because several potentially significant side effects including hypopituitarism may develop. Stereotactic radiosurgical methods may have several advantages over conventional radiotherapy; they can be applied, for example, in patients with residual or recurrent pituitary tumors who had previously received conventional radiotherapy. However, long-term follow-up data with these relatively new techniques are still limited.     

垂体脓肿的诊断与治疗

目的 探讨垂体脓肿的临床表现及治疗方法.方法 回顾性分析6例垂体脓肿的诊治资料,并复习相关文献.结果 6例患者中,头痛5例、垂体功能低下4例、视力下降和/或颞侧偏盲4例、发热1例.MRI或cT检查5例有病灶周边环形强化.术前诊断为垂体腺瘤3例,颅咽管瘤1例,垂体脓肿2例.1例经颅手术,5例经蝶手术.术后均静脉滴注抗生素治疗3周.所有病例术后症状缓解,随访8个月至10年,1例经颅手术者2年后复发改行经蝶手术而治愈,5例经蝶手术者无复发.结论 垂体脓肿易误诊,对鞍区囊性病变应怀疑有垂体脓肿的可能.及早经蝶手术及围手术期合理使用抗生素是垂体脓肿治疗的关键.     

The Bragg-Peak Proton Beam in Noninvasive Hypophysectomy

The ionization and penetration characteristics of the proton beam permit an ablative dose of radiation to be delivered to the pituitary with minimal effects on other intracranial structures. This approach to hypophysectomy is especially appropriate in conditions like acromegaly, where delay in achieving hypopituitarism is not critical (as in terminal breast cancer). The steps in the procedure are outlined.     

Non-secretory adenomas of the pituitary treated by trans-ethmoidal sellotomy.

A consecutive series of 35 clinically non-secretory pituitary adenomas treated surgically by the transethmodial approach is presented. Mean age at presentation was 60 years, with a male-to-female ratio of 2:1. These tumours present later than hormone secreting tumours: 90% were over 2 cm in diameter at operation. Seventy-one per cent of patients had impaired vision and 89% had hypopituitarism. Surgical treatment relieved or significantly improved visual fields in 79% of patients with impaired vision. Ninety-one per cent required permanent hormone replacement. Mean follow-up was for 4.4 years. Five tumours recurred, causing recurrent visual symptoms. No tumours recurred in those patients treated with postoperative radiotherapy, but follow-up in this group was only for a mean of 2.7 years.     

颅外垂体移植术供体细胞的培养研究

目的 本实验通过对人垂体腺瘤细胞体外培养实验，证实培养技术的可靠性和可重复性；为颅外移植术应用垂体腺瘤细胞作为移植物供体细胞的实施，以及为腺垂体移植细胞库的建立提供有用的实验依据。方法 利用手术切除的人垂体腺瘤组织做体外培养，动态观察培养细胞的生长状态，通过放免测定和免疫化学染色技术，评价体外培养的垂体细胞的功能状态。结果 人垂体腺瘤细胞经体外培养仍具有分泌激素的功能，6例GH腺瘤均有大量GH分泌，5例PRL腺瘤分泌大量PRL，3例无功能腺瘤细胞培养液中分别有不同程度的FSH及LH、PRL分泌。结论 培养成功的14例人垂体腺瘤细胞在体外均具有激素分泌功能。为应用垂体腺瘤细胞为移植物供体，进行颅外移植的研究提供了有用的实验基础。     

Maternal zinc deficiency raises plasma prolactin levels in lactating rats

There is an inverse relation between zinc (Zn) intake and plasma prolactin in men and nonpregnant women. Whether a relation exists in lactating women is unknown, despite the potential consequences of perturbations in prolactin regulation on lactation performance. We examined the effects of low Zn intake on prolactin concentration, the prolactin regulatory pathway in the pituitary gland, and lactation performance in lactating rats. Female rats were fed diets containing 7 (zinc deficient; ZD), 10 (marginally zinc deficient; MZD) or 25 mg Zn/kg (control) from 70 d preconception to lactation d 11. Rats were killed, pituitary glands dissected, and tissues and plasma collected and analyzed for prolactin concentration. Pituitary gland pituitary factor 1 (Pit-1), dopamine 2 receptor (D2R), and prolactin receptor mRNA expression were measured in the pituitary gland. Liver, mammary gland, plasma, and milk Zn were measured. Milk intake of the pups was also recorded. Plasma prolactin concentration was higher in rats fed the ZD (125.9 microg/L) diet compared with control rats (21.7 microg/L). Pituitary gland prolactin concentration was higher in rats fed the ZD diet (69.8 mg/g total protein) compared with controls (29.0 mg/g). Plasma Zn concentration was lower in rats fed the MZD and ZD diets, and mammary gland and milk Zn concentrations were lower in rats fed the ZD diet compared with control rats. Rats fed the ZD diet had lower D2R, prolactin receptor, and Pit-1 mRNA levels, whereas rats fed the MZD diet had lower prolactin receptor and Pit-1 mRNA levels compared with control rats. Milk intake was lower in pups of rats fed the MZD and ZD diets. Our results suggest that marginal Zn nutriture may compromise milk production despite increased prolactin levels. In addition, increased circulating prolactin concentration is not due to altered nursing behavior, but may be due to alterations in the prolactin regulatory pathway in the pituitary gland.     

Cholesterol granuloma at the sella region: a new method of the differential diagnosis of craniopharyngioma

Cholesterol-granuloma is a pseudotumoral mass that is believed to enlarge by a self-perpetuating sequence of repeated hemorrhages and reparative tissue reaction. Albeit an almost ubiquitous phenomenon throughout the body, cholesterol-granuloma has recently been appreciated as a distinctive lesion mimicking or associated with craniopharyngiomas. Upon review of a surgical series of 15 purported craniopharyngiomas, the authors identified 3 such occurrences. All were characterized by a predominance of slit-like cholesterol clefts with multi-nucleated giant cells embedded in a fibrotic stroma permeated with lipid laden macrophages, lymphocytes, as well as organizing hemorrhage. Non-craniopharyngioma specific cuboidal epithelium was present in one case. The mean age of patients--all males--with cholesterol-granuloma was 26 years, and all but one had an intrasellar tumor component. Clinical symptoms referrable to hypopituitarism predominated. At variance with the above, patients with adamantinomatous or papillary craniopharyngiomas were 23.5 and 46 years old, respectively, and presented with neurological deficits or ones due to hypothalamic involvement by their tumors. With marginal central nervous tissue present in 53 percent of the specimens, 75 percent of adamantinomatous craniopharyngiomas, but only 12 percent of cholesterol-granulomas showed invasive growth. At present cholesterol-granulomas are conceived as a clinicopathologically distinctive lesion of uncertain origin. They most probably represent a clinically relevant entity in the ontogenesis of adamantinomatous craniopharyngiomas with predisposing factors yet to be elucidated.     

垂体肿瘤转化基因对垂体腺瘤发生和发展作用机制的研究

目的通过定量分析垂体肿瘤转化基因(PTTG)在乙烯雌酚诱发的大鼠催乳素(PRL)瘤组织以及不同类型的人垂体腺瘤中的表达量,探讨PTTG在垂体腺瘤发生发展中的作用。方法动物实验:用皮下植入乙烯雌酚的方法诱发大鼠垂体PRL瘤模型,用RT-PCR方法分析PRL瘤组织中rPTTGmRNA的水平。临床研究:收集32例垂体腺瘤标本。用RT-PCR方法检测hPTTGmRNA和碱性成纤维细胞生长因子(bFGFmRNA)在各组垂体腺瘤组织中的表达量。结果rPTTG基因在大鼠1PRL瘤中的表达量明显高于对照组(P<0.01);rPTTGmRNA水平与大鼠垂体重量呈明显正相关(γ=0.9920,P<0.01);hPTTGmRNA和bFGFmRNA在所有垂体腺瘤中均有表达。hPTTGmRNA和bFGFmRNA水平随着肿瘤的分级和分期的增高而升高,组间比较具有统计学差别(P<0.01)。结论PTTG表达与大鼠PRL瘤以及人垂体腺瘤的侵袭性相关,PTTG在垂体肿瘤发生和发展中起重要作用。     

Live birth after assisted reproductive technology treatment in a case with lumboperitoneal shunt following intracranial pathology

Lumbar peritoneal shunt is a technique of cerebrospinal fluid diversion from the lumbar thecal sac to the peritoneal cavity. By diverting, a second compartment of the body; abdomen starts to accompany the case as a new problem source. 32 years old woman who had been considered to be primary infertile for fifteen years applied to our infertility clinic. She had a transsphenoidal surgery due to macroadenoma of pituitary gland fourteen years ago. By trans-vaginal ultrasonography we revealed 4 antral follicles and diffuse ascites in pouch of douglas due to the shunt. There were spicular projections fom uterus and bowel serosa to inner abdominal wall, that we thought about severe intraabdominal adhesions. Controlled ovarian hyperstimulation was started with 300 IU menotropin on third day of the cycle. We yielded 4 oocyte. One grade-1 embryo transferred at day two. After two weeks hCG positivity was determined. At 39th week 3 900 g female baby was born vaginaly with 1st minute apgar score as 9 and 5th minute apgar score as 10.     

垂体瘤切除术围手术期的护理体会——神经内镜下经鼻蝶入路

目的:探讨神经内镜下经鼻蝶入路垂体瘤切除术围手术期的护理要点。方法:对我院2008年02月-2011年02月收治的90例全麻下行神经内镜下经鼻蝶入路垂体瘤切除术患者的围手术期护理资料进行回顾性分析。结果:患者术后出现并发症共8例,其余均取得良好效果。随访12-24个月,原垂体瘤症状均不同程度减轻,并发症痊愈。结论:加强神经内镜下经鼻蝶入路切除垂体瘤患者的围手术期护理,针对术后易出现的并发症及早采取相应的护理措施是患者手术顺利进行、术后早日康复的关键因素之一。     

垂体卒中诱发因素的临床意义

目的探讨有诱发因素垂体卒中和无诱发因素垂体卒中患者在临床特征、治疗结果方面的区别及其临床意义．方法42例急性垂体卒中患者中有诱发因素(12例)与无诱发因素患者(30例)在临床特征及预后方面进行比较、结果有诱发因素的患者与无诱发因素的患者：前者更多地出现精神状态的改变(P=0．032)、视觉功能障碍(P=0．020)和视野缺损(P=0．046)，诊断比后者更加延迟(P=0．015)。结论垂体卒中患者常因诱发因素加重其病情，混杂其临床表现而使诊断和治疗延迟，使视觉功能恢复不佳。对头痛患者中突然出现视力衰退者应考虑到垂体卒中的可能性。     

磁共振鉴别女童特发性性早熟与单纯乳房早发育的探讨

目的分析特发性性早熟(idiopathic central precocious puberty,ICPP)和单纯乳房早发育(premature thelarche,PT)女童的磁共振(magnetic resonance imaging,MRI)影像学特点,探讨MRI在两者鉴别诊断中的价值。方法以在河南某省级医院确诊的ICPP女童和PT女童各35例作为研究对象,均行头颅鞍区磁共振扫描,测量MRI脑垂体高度,Elster法划分脑垂体发育等级。结果垂体高度:ICPP组的均值为(5.18±1.01)mm,PT组(4.30±0.38)mm,差异有统计学意义(P0.05);垂体形态:ICPP组Ⅳ级及以上的占82.86%,PT组Ⅳ级及以上占11.43%(P0.05)。结论 ICPP组患儿的垂体偏高,上缘饱满;PT组患儿垂体偏低,以上缘扁平或低凹为主。磁共振脑垂体检查对ICPP与PT鉴别诊断有重要参考价值,无创无痛,可作为性早熟初诊的辅助检查。     

Neuroendocrine dysfunctions and their consequences following traumatic brain injury

Posttraumatic hypopituitarism is of major public health importance because it is more prevalent than previously thought. The prevalence of hypopituitarism in children with traumatic brain injury is unknown. Most cases of posttraumatic hypopituitarism remain undiagnosed and untreated in the clinical practice, and it may contribute to the severe morbidity seen in patients with traumatic brain injury. In the acute phase of brain injury, the diagnosis of adrenal insufficiency should not be missed. Determination of morning serum cortisol concentration is mandatory, because adrenal insufficiency can be life threatening. Morning serum cortisol lower than 200 nmol/L strongly suggests adrenal insufficiency. A complete hormonal investigation should be performed after one year of the trauma. Isolated growth hormone deficiency is the most common deficiency after traumatic brain injury. Sports-related chronic repetitive head trauma (because of boxing, kickboxing, football and ice hockey) may also result in hypopituitarism. Close co-operation between neurosurgeons, endocrinologists, rehabilitation physicians and representatives of other disciplines is important to provide better care for these patients.     

低血糖症状的病因研究

目的探讨导致低血糖症状的常见病因.方法回顾性分析本院1994年1月至2001年11月临床诊治的119例患者出现低血糖症状的病因.结果糖尿病患者使用过量降血糖药物或胰岛素82例(69%),胰岛细胞瘤9例(7.6%),垂体、肾上腺功能不全9例(7.6%),肝脏病3例(2.5%),败血症3例(2.5%),甲亢误诊为糖尿病2例(1.6%),盆腔肿瘤1例(0.8%).结论低血糖可由多种原因引起,虽然最常见的原因还是糖尿病治疗过程中用药不当,但脑垂体、肾上腺、甲状腺疾病亦可造成,甚至败血症、肿瘤也可引起.     

Combined dietary and pharmacological weight management in obese hypopituitary patients

OBJECTIVE: The high prevalence of obesity and cardiovascular risk factors in hypopituitarism affirms the need for effective weight loss intervention. In this study, we investigated the combined effect of sibutramine, diet, and exercise in obese hypopituitary patients (HPs). RESEARCH METHODS AND PROCEDURES: In an open-label prospective intervention trial, 14 obese well-substituted nondiabetic HPs and 14 matched simple obese controls were allocated to 11-month treatment with sibutramine (10 to 15 mg), diet (600 kcal/d deficit), and exercise. Anthropometric indices and body composition (obtained from DXA scan) were assessed monthly for the first 5 months and thereafter every second month for the next 6 months. RESULTS: Mean (+/-SD) weight loss at 11 months was 11.3 +/- 4.8 kg in patients vs. 10.7 +/- 4.7 kg in controls. The HPs exhibited the same improvements in body composition, waist circumference, blood lipids, and fasting glucose as the simple obese. In a multivariate model, baseline weight, duration of growth hormone replacement therapy, and duration of pituitary disease explained 79% (p = 0.001) of the variation in weight loss at 4 months in the HPs. Only baseline weight and waist circumference could predict weight loss at 11 months. DISCUSSION: HPs are not resistant to weight loss therapy. Almost all will achieve at least 5% weight loss, and 60% can lose >10% weight within 11 months. However, the long-term effect on risk factors associated with type 2 diabetes and cardiovascular disease as well as on mortality needs to be established.