Abstract:
:Twenty years after the first description of combined hypopituitarism (CPHD) caused by PROP1 mutations, the phenotype of affected subjects is still challenging for clinicians. These patients suffer from pituitary hormone deficits ranging from IGHD to panhypopituitarism. ACTH deficiency usually develops later in life. Pituitary size is variable. PROP1 mutation is the most frequent in familial congenital hypopituitarism (CH). Reports on initiation of hormonal replacement including growth hormone (GH) in adults with CH are scarce. We identified 5 adult siblings with CPHD due to PROP1 mutation (301-302delAG), aged 36-51 years (4 females), never treated for hormone deficiencies. They presented with short stature (SD from - 3.7 to - 4.7), infantile sexual characteristic, moderate abdominal obesity and low bone mineral density in 3 of them. Complete hypopituituitarism was confirmed in three siblings, while two remaining demonstrated GH, TSH, FSH and LH deficiencies. Required hormonal replacement including rhGH was initiated in all patients. After several months necessity for hydrocortisone replacement developed in all patients. After 2 years of continual replacement therapy, BMD and body composition (measured by DXA-dual X-ray absorptiometry) improved in all subjects, most prominently in two younger females and the male sibling. Besides rhGH therapy, these three patients have received sex hormones contributing to the favorable effect. The male sibling was diagnosed with brain glioblastoma two years following complete hormonal replacement. This report provides important experience regarding hormonal replacement, particularly rhGH treatment, in adults with long-term untreated CH. Beneficial effect of such therapy are widely acknowledged, yet these subjects could be susceptible to certain risks of hormonal treatment initiated in adulthood. Careful and continual clinical follow-up is thus strongly advised.
journal_name
Pituitaryjournal_title
Pituitaryauthors
Doknic M,Gasic V,Stojanovic M,Pavlovic S,Marinkovic S,Miljic D,Pekic S,Manojlovic-Gacic E,Damjanovic D,Soldatovic I,Petakov Mdoi
10.1007/s11102-020-01049-9subject
Has Abstractpub_date
2020-08-01 00:00:00pages
400-408issue
4eissn
1386-341Xissn
1573-7403pii
10.1007/s11102-020-01049-9journal_volume
23pub_type
杂志文章相关文献
Pituitary文献大全abstract::A 57-year-old woman presented with 2-year history of polyuria and polydipsia. Hormonal studies revealed almost normal anterior pituitary function and central diabetes insipidus. Magnetic resonance imaging showed thickening of the pituitary stalk and enlargement of the neurohypophysis without high intensity of the post...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1023/a:1009914711287
更新日期:1999-05-01 00:00:00
abstract::Pituitary cysts are usually craniopharyngiomas or remnants of Rathke's cleft. Histologically craniopharyngiomas are lined by stratified squamous epithelium whereas Rathke's cleft cysts are lined by cuboidal or columnar epithelium. The management of pituitary cysts remains difficult because of the risks associated with...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1023/a:1025373715860
更新日期:2002-01-01 00:00:00
abstract:PURPOSE:Endogenous Cushing's syndrome (CS) is a rare disease that results from exposure to high levels of cortisol; Cushing's disease (CD) is the most frequent form of CS. Patients with CS suffer from a variety of comorbidities that increase the risk of mortality. Surgical resection of the disease-causing lesion is gen...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-016-0742-1
更新日期:2016-12-01 00:00:00
abstract::Silent growth hormone adenomas (SGHA) are a rare entity of non-functioning pituitary neuroendocrine tumors. Diagnosis is invariably made post-operatively of a tumor immunopositive for GH (and Pit-1 in selected cases) but without clinical acromegaly. Mainly young females are affected, and tumors are often uncovered by ...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-017-0858-y
更新日期:2018-04-01 00:00:00
abstract::We aimed to study the occurrence of acute-onset symptoms at initial presentation in a national Danish cohort of patients with childhood- or adult-onset craniopharyngioma, and to investigate potential risk factors for acute presentation. Medical records of 189 consecutive patients (39 children, 150 adults) presenting w...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0451-3
更新日期:2013-12-01 00:00:00
abstract:CONTEXT:Somatostatin (SST) and dopamine (DA) inhibit growth hormone (GH) secretion and proliferation of GH-secreting pituitary adenomas (GHomas) through binding to SSTR2 and D2R receptors. Chimeric SST-DA compounds (Dopastatins) display increased potency in inhibiting GH secretion, as compared with individual SST or DA...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-020-01113-4
更新日期:2021-01-12 00:00:00
abstract::Anemia and other hematological abnormalities are common in patients with Sheehan's syndrome. The response of these abnormalities to replacement of thyroxine and glucocorticoids is not clear. The aim of the present study was to document the profile of hematological abnormalities and response to treatment in patients wi...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-010-0255-2
更新日期:2011-03-01 00:00:00
abstract::Ideal surgical treatment of craniopharyngiomas remains a major challenge for neurosurgeons. Craniopharyngiomas grow in the deep-seated hypothalamic area that is paramount for vegetative, emotional and endocrine function, and for maintaining worthwhile life. The benign histological nature of craniopharyngiomas belies t...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-008-0137-z
更新日期:2008-01-01 00:00:00
abstract:INTRODUCTION:There has been growing interest on medical therapy for the management of Cushing's disease (CD), particularly in cases of persistent or recurrent hypercortisolism. Ketoconazole, an inhibitor of adrenal steroidogenesis, is the most widely used drug, whereas cabergoline and pasireotide are the most promising...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-015-0641-x
更新日期:2015-04-01 00:00:00
abstract::The purpose of this study is to verify whether acute pre-treatment with alprazolam (ALP), a benzodiazepine that inhibits HPA secretion in normal subjects, could better characterize patients with subclinical Cushing's syndrome (SCS) than the 1-mg dexamethasone test (DST). In 22 patients with SCS, 10 with overt Cushing'...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0433-5
更新日期:2013-09-01 00:00:00
abstract::Double pituitary adenomas are rare in surgical specimens and the most common clinical feature in reported patients has been acromegaly. We report 3 cases of double pituitary lesions in patients who presented with Cushing's disease. In a 22-year-old man (case 1) with delayed puberty and low testosterone levels, mild hy...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1023/a:1011499609096
更新日期:2000-11-01 00:00:00
abstract:PURPOSE:Severe fluctuations in plasma sodium concentration and plasma osmolarity, including central diabetes insipidus (CDI), may have significant influence on postoperative morbidity and mortality after pediatric brain tumor surgery.The aim of this study was to describe the frequency, severity and neurological consequ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-018-0886-2
更新日期:2018-08-01 00:00:00
abstract::We evaluated the efficacy of Gamma knife stereotactic radiosurgery (GKSR) as an adjunctive management modality for patients with drug resistant or intolerant cavernous sinus invasive prolactinomas. Twenty-two patients with cavernous sinus invasive prolactinoma underwent GKSR between 1994 and 2009. Thirteen patients we...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0376-x
更新日期:2013-03-01 00:00:00
abstract::Inferior petrosal sinus sampling (IPSS) of ACTH with CRH stimulation helps distinguish pituitary ACTH-dependent Cushing's syndrome from the ectopic ACTH syndrome (EAS). The usefulness of the paradoxical response of other pituitary hormones including prolactin to CRH remains controversial. Data from 33 IPSS procedures ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-009-0200-4
更新日期:2010-06-01 00:00:00
abstract::Medications commonly cause hyperprolactinemia and their use must be differentiated from pathologic causes. The most common medications to cause hyperprolactinemia are the antipsychotic agents, although some of the newer atypical antipsychotics do not do so. Other medications causing hyperprolactinemia include antidepr...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-008-0106-6
更新日期:2008-01-01 00:00:00
abstract::Meningiomas account for about 1% of sellar masses. Although they can mimic pituitary adenomas, they are more vascularized and invasive. To gain insights that would enhance our ability to establish a pre-surgical diagnosis of meningioma, we performed a retrospective study of these tumors. Query of the surgical patholog...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-013-0507-z
更新日期:2014-08-01 00:00:00
abstract::Apart from the radiologic features regarding size and invasiveness, we had noticed some differences in morphology among types of pituitary adenomas. We conducted this study to verify the differences in radiologic morphology between growth hormone producing pituitary adenomas (GHoma) and nonfunctioning pituitary adenom...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-013-0479-z
更新日期:2014-04-01 00:00:00
abstract::Non-functioning pituitary neuroendocrine tumors do not cause endocrine symptoms related to hypersecretion of adenohypophyseal hormones and are clinically characterized by symptoms due to growing sellar tumor mass. Histopathological classification of this tumor group has always been challenging due to their heterogenei...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-017-0855-1
更新日期:2018-04-01 00:00:00
abstract:PURPOSE:Hypofractionated stereotactic radiotherapy (HSRT) for refractory Cushing's disease may offer a condensed treatment schedule for patients with large tumors abutting the optic chiasm unsuitable for stereotactic radiosurgery (SRS). To-date only four patients have been treated by HSRT in the published literature. W...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-019-00992-6
更新日期:2019-12-01 00:00:00
abstract::Leptin is a key mediator in the maintenance of neuroendocrine homeostasis. The aim of this study was to determine the changes in serum leptin, tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6), nitric oxide (NO) levels in patients with hyperprolactinemia. The study consists of 16 consecutive patients with ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-008-0140-4
更新日期:2009-01-01 00:00:00
abstract:PURPOSE:Approximately 25 % of cases of clinically active acromegaly cases treated in our academic center between 1996 and 2000, were diagnosed in patients who had elevated plasma IGF-1 levels, but apparently "normal" 24-h mean plasma GH levels. The current study served to update the data for patients with acromegaly re...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-016-0735-0
更新日期:2016-12-01 00:00:00
abstract:INTRODUCTION:Cushing's syndrome is associated with a number of clinical manifestations and co-morbidities which may not resolve even after long-term remission leading to excessive mortality. MATERIALS AND METHODS:This review summarizes the main manifestations of Cushing's syndrome (active or in remission) with particu...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-014-0631-4
更新日期:2015-04-01 00:00:00
abstract::Acromegaly is a consequence of chronic growth hormone (GH) excess, due in the majority of cases to a GH-secreting pituitary adenoma, and occurring with a population prevalence of 60 per million and an incidence of 3-4 per million per year. Males and females appear to be equally affected with an average age of presenta...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1023/a:1009965803750
更新日期:1999-06-01 00:00:00
abstract::In western countries, the process of "ageing of the population" is increasingly forcing clinical medicine to find answers for pathologies affecting the elder segments of our community. In this respect, pituitary adenomas often raise difficult questions on surgical indications, since little is known about postoperative...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0390-z
更新日期:2013-06-01 00:00:00
abstract::Arterial bleeding during transsphenoidal surgery for pituitary adenoma is known complication. This usually happens due to rupture of intracavernous carotid or delayed hemorrhage due to the carotico-cavernous fistula and/or pseudoaneurysm. There is also evidence that cavernous carotid aneurysms may occur with pituitary...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-009-0198-7
更新日期:2010-06-01 00:00:00
abstract:PURPOSE:To provide an overview of fundamental concepts in machine learning (ML), review the literature on ML applications in imaging analysis of pituitary tumors for the last 10 years, and highlight the future directions on potential applications of ML for pituitary tumor patients. METHOD:We presented an overview of t...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-019-01026-x
更新日期:2020-06-01 00:00:00
abstract:CONTEXT:Octreotide causes significant tumour shrinkage in patients with acromegaly but the exact mechanism of action is unclear in vivo. OBJECTIVE:To determine the mechanism of action of octreotide in vivo using dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). DESIGN:Five patients with acromegaly were ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-007-0044-8
更新日期:2007-01-01 00:00:00
abstract::Acute lymphoblastic leukaemia (ALL) is the most common childhood malignancy. The survival rate in the Scandinavian countries is now around 85 %. ALL patients treated with cranial radiotherapy (CRT) are at risk for growth hormone deficiency (GHD), but little is known about other pituitary insufficiencies, e.g. ACTH. Ad...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0459-8
更新日期:2014-02-01 00:00:00
abstract:PURPOSE:Doses of growth hormone in adults with growth hormone deficiency are now lower than previously. However, it is not clear they are as effective as higher doses. The objective of this meta-analysis was to assess efficacy of low to moderate dose (LD) GH replacement on standard endpoints of GH compared to higher do...
journal_title:Pituitary
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s11102-014-0571-z
更新日期:2015-06-01 00:00:00
abstract:INTRODUCTION:Autoimmune lymphocytic hypophysitis associates predominantly with other autoimmune endocrinopathies and is most commonly treated with glucocorticoids and/or decompressive pituitary surgery. Here we report a new association and treatment modality for lymphocytic hypophysitis. METHODS:A 52-year-old woman pr...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-014-0592-7
更新日期:2015-08-01 00:00:00