BACKGROUND: Acromegaly, which may be present in patients with McCune-Albright syndrome (MCAS), in association with café-au-lait spots, precocious puberty, and fibrous dysplasia, is often difficult to treat surgically because skull base bone dysplasia prevents the removal of the pituitary adenoma. Somatostatin analogs (SAs) generally give only partial responses. The use ...

OBJECTIVE: ALS is the most common motor neurone disorder in human adults. Scanty data on endocrine abnormalities have been reported. The aim of the present study was to investigate the GH-IGF-I axis in ALS patients. PATIENTS: Twenty-two ALS patients (12 men, 10 women), mean age 61 years, and 25 normal ...

OBJECTIVE: To explore adrenal function in severe West Nile virus (WNV) infection. DESIGN AND SETTING: Prospective interventional cohort study in a medical ICU of a teaching hospital. PATIENTS: Ten consecutive patients (seven men, mean age 64+/-12years, mean SAPS II 26+/-6) with definite diagnosis of WNV related meningoencephalitis and variable proportion ...

OBJECTIVE: Some patients with hyperandrogenemia had no identifiable cause, which was named as idiopathic hyperandrogenemia (IHA). The role of the adrenal glands in these patients was investigated. DESIGN: Clinical study in patients with IHA at the Endocrinology Department of a University Hospital. PATIENT(S): In this study, 26 pre-menopausal women with ...

CONTEXT: A variant of the human GH receptor (GHR) lacks a 22-amino-acid sequence derived from exon 3 (d3-GHR). It was reported that pediatric patients, born small for gestational age or with idiopathic short stature who were homozygous or heterozygous for this variant responded better to GH treatment than those homozygous ...

BACKGROUND: Retroperitoneal schwannomas are rare, benign tumors. The aim of this study is to present our surgical experience with 7 such tumors. METHODS: Between 1989 and 2004, 7 patients with pathologically proven retroperitoneal schwannomas were reviewed retrospectively. RESULTS: There were 6 male patients and 1 female patient, with a mean ...

Among 297 women with nonclassic adrenal hyperplasia (NCAH), premature pubarche was the most common complaint in girls (87%), and the frequency of hirsutism increased progressively with age from adolescence (50%) to adulthood (70%). The frequency of spontaneous miscarriages was high in NCAH patients (20%), but it decreased significantly after treatment.

The overnight one-mg dexamethasone suppression test has been used for many years to screen for Cushing's syndrome. This test has usually been evaluated in controls versus patients with severe hypercortisolism. Under these conditions, the overnight dexamethasone suppression test has been reported to have high sensitivity and specificity. The objective of ...

OBJECTIVE: The aim of this study was to evaluate the role of transsphenoidal selective adenomectomy alone or in combination with adjuvant therapy in treatment of recurrent Cushing's disease. METHODS: A total of 16 patients with recurrent Cushing's disease underwent reoperation, 15 via a transsphenoidal approach and one via a combined ...

HYPOTHESIS: Adrenal vein sampling is superior to computed tomography for subtype differentiation of primary hyperaldosteronism. DESIGN: Retrospective review. SETTING: University medical center. PATIENTS: Forty-eight patients (32 men and 16 women) with biochemically confirmed primary hyperaldosteronism. MAIN OUTCOME MEASURES: We compared demographic factors, results of biochemical and imaging studies (computed tomography ...

We carried out an audit of management of patients with 21-Hydroxylase deficiency CAH who presented to the Department of Endocrinology OPD from 1999 till 2004 and had a minimum follow up of 6 months. Of the 30 patients analysed 24 were girls and 6 were boys. The majority belonged to ...

Inactivating PROP1 gene alterations are responsible for over 50% of familial combined pituitary hormone deficiency cases. Pituitary enlargement followed by regression and subnormal pituitary size has been documented in a number of PROP1 deficient patients. Data derived from PROP1 deficient mice (Ames dwarfs) have revealed some of the underlying cellular ...

Sheehan's syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. The aim of the present study was to determine the clinical characteristics of Sheehan's syndrome in 20 patients (mean age 60.15 +/- 3.41 years) with typical obstetric history. The mean duration between time of diagnosis ...

With two study protocols, one retrospective and the other prospective, we evaluated hypothalamo-hypophysial dysfunction (HHD) in paediatric patients treated for traumatic brain injury (TBI) in the neurosurgical or intensive care department at our hospital. The retrospective group comprised 22 patients who had experienced TBI 0.7-7.25 years before the study. The ...

Acute adrenal insufficiency has been demonstrated in a number of patients with shock. This study was designed to evaluate the rate of occult adrenal insufficiency in the critically ill trauma population and to determine the impact of hypoproteinemia on the use of random cortisol levels as a marker for adrenal ...

CONTEXT: Some variants of the glucocorticoid receptor (GR) gene have been found to alter glucocorticoid sensitivity and have been associated with altered metabolic profiles. OBJECTIVE: The objective of the study was to examine whether N363S and ER22/23K variants of the GR gene may be associated with the development of adrenal ...

Current therapies for X-linked adrenoleukodystrophy (X-ALD) include replacement therapy with adrenal steroids, which is mandatory for all patients with impaired adrenal function but does not alter neurological progression significantly; dietary therapy with "Lorenzo's Oil," which appears to have a preventive effect in asymptomatic boys whose brain MRI is normal; and ...

The diagnosis of non-organic growth hormone (GH) deficiency (GHD) remains difficult. OBJECTIVE: To evaluate the value of measuring plasma insulin-like growth factor (IGF)-I and -II, IGF binding protein-3 (IGFBP-3) and acid-labile subunit (ALS) as criteria for diagnosing GHD. PATIENTS: 120 prepubertal patients having at least one of the main auxological ...

CONTEXT: Recent data have demonstrated that traumatic brain injury (TBI)-mediated hypopituitarism could be more frequent than previously known. However, most previous data were obtained from retrospective studies. OBJECTIVES: The aim of this study was to determine 1) the prevalence of anterior pituitary hormone deficiencies in the acute phase of TBI ...

PURPOSE: To assess patterns of practice in our institution specifically regarding corticosteroid deficiency diagnosis in patients with septic shock. METHODS: Consecutive adult patients with vasopressor-dependent septic shock admitted to the medical intensive care unit between January 2002 and September 2003 were studied. Relative adrenal insufficiency (RAI) was diagnosed by a ...

BACKGROUND AND OBJECTIVE: There is agreement in the literature that pituitary apoplexy is a rare disorder. As our experience differs from this view, we analysed the incidence in patients operated on for a nonfunctioning pituitary adenoma. PATIENTS AND DESIGN: One hundred ninety-two consecutive patients with a suprasellar, clinically inactive adenoma ...

OBJECTIVES: Very little is still known about ghrelin in cases of GH hypersecretion. We decided to evaluate whether ghrelin concentrations in acromegalic patients differ according to the administered therapy (surgical and/or with long acting octreotide). We wanted to assess the correlation between serum ghrelin levels and 1) the treatment applied ...

BACKGROUND & AIMS: Irritable bowel syndrome (IBS) is a functional disorder with an etiology that has been linked to both psychological stress and infection. The primary aim of this study was to examine the hypothalamic-pituitary-adrenal axis in patients with IBS and to relate such response to plasma cytokine profiles. METHODS: ...

OBJECTIVE: Pituitary tumours occurring after bilateral adrenalectomy for Cushing's disease (Nelson's syndrome) are frequently aggressive, so an early diagnosis and careful management are of prime importance. For a new insight into this entity it is necessary to analyse the factors predisposing to its development and the course of the disease, ...

CONTEXT: Anorexia nervosa (AN) is an eating disorder characterized by self-induced starvation. Gastric hormone ghrelin, potent orexigen, and natural GH secretagogue are increased in AN. Although exogenous ghrelin stimulates appetite, GH, prolactin, and cortisol release in humans, its effects have not been studied, during infusions, in AN patients. OBJECTIVE: The ...

OBJECTIVES: To evaluate cortisol levels and prevalence of adrenal insufficiency in patients with severe community-acquired pneumonia (CAP). DESIGN AND SETTING: Retrospective cohort study in a 24-bed medical-surgical intensive care unit (ICU). PATIENTS: Forty patients with severe CAP admitted to the ICU from March 2003 and May 2005. MEASUREMENTS AND RESULTS: ...

OBJECTIVE: Adrenal failure is emerging as an important cause of excess morbidity and mortality in critically ill patients. We have recently reported the high incidence of adrenal failure in patients with liver disease, the so called "hepato-adrenal syndrome." It has been noted that patients who on initial testing may have ...

Transient diabetes insipidus is a well-known complication after transsphenoidal surgery (TSS). On the other hand, transient hyponatremia has been reported as being a delayed complication of TSS. Transient hyponatremia has been attributed to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but the details of hyponatremia have not been ...

OBJECTIVE: To assess the pressor response to phenylephrine infusion before and after hydrocortisone in severe trauma patients and to correlate this response with their adrenal reserve. DESIGN: Prospective clinical study. SETTING: Surgical intensive care unit in a university teaching hospital. PATIENTS: Twenty-three young trauma patients (Injury Severity Score, 38 +/- ...

AIM: To evaluate anti-Müllerian hormone (AMH) levels in patients with clinical and molecular diagnosis of 5alpha-reductase 2 deficiency. PATIENTS AND METHODS: Data from 14 patients whose age ranged from 21 days to 29 years were analyzed according to age and pubertal stage. Sexual ambiguity was rated as Prader III in ...

PURPOSE: Hypopituitarism has been reported in up to half of long-term survivors of traumatic brain injury. We attempted to define the natural history of post-traumatic hypopituitarism to devise guidelines for the optimal timing of patients' assessment and hormone replacement. SUBJECTS AND METHODS: Fifty consecutive patients with severe or moderate head ...

Adrenoleukodystrophy (ALD) and its adult variant adrenomyeloneuropathy (AMN) are X-linked diseases associated with a deficiency in the peroxisomal degradation of saturated very long chain fatty acids (VLCFA) resulting in an accumulation of VLCFA in the central and peripheral myelin, the adrenal cortex and the testis. Adrenal insufficiency with clinical hypocortisolism ...

BACKGROUND: Adrenal insufficiency impacts on the haemodynamic management of patients in intensive care. Very little is known about the incidence of adrenal insufficiency in the first 10 days after traumatic brain injury. METHODS: We retrospectively reviewed the charts of 113 traumatic brain injury patients within 10 days of their injury. ...

Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients ...

CONTEXT: Half of the patients with Noonan syndrome (NS) carry mutation of the PTPN11 gene, which plays a role in many hormonal signaling pathways. The mechanism of stunted growth in NS is not clear. OBJECTIVE: The objective of the study was to compare growth and hormonal growth factors before and ...

Adrenal insufficiency is a rare disease, but its prevalence is increasing. The most frequent cause of primary adrenal insufficiency in western countries is autoimmune adrenalitis, whereas secondary adrenal insufficiency is most often caused by pituitary tumours and their treatment (e.g., surgery). Chronic glucocorticoid replacement consists of hydrocortisone 15-25 mg/day in ...

BACKGROUND: The integrity of the hypothalamic-pituitary-adrenal (HPA) axis is a major determinant of the host response to stress. Recent studies have proven that low doses of hydrocortisone and fludrocortisone can significantly reduce mortality in patients with relative adrenal insufficiency (RAI) without increasing adverse events. STUDY DESIGN: This study was a ...

CONTEXT: The high-dose short Synacthen (corticotropin) test (SST) is widely used to investigate suspected secondary adrenal insufficiency, but concern remains about falsely reassuring results. OBJECTIVE: Our objective was to evaluate the long-term safety of the SST. METHOD: We retrospectively evaluated the clinical outcome in 178 patients who achieved 30-min cortisol ...

Pegvisomant is a pegylated analog of growth that functions as a growth hormone receptor antagonist. The drug is capable of normalizing serum IGF-I concentrations (the chief mediator of disease activity in acromegaly) in 97% of patients, and therapy is associated with significant improvements in the symptoms and signs of GH ...

Subjects at their first psychotic episode show an enlarged volume of the pituitary gland, but whether this is due to hypothalamic-pituitary-adrenal (HPA) axis hyperactivity, or to stimulation of the prolactin-secreting cells by antipsychotic treatment, is unclear. We measured pituitary volume, using 1.5-mm, coronal, 1.5 T, high-resolution MRI images, in 78 ...

OBJECTIVE: To investigate the effect of Rosiglitazone in three patients treated with bilateral adrenalectomy followed by hyperpigmentation and hypersecretion of ACTH. PATIENTS AND METHODS: One patient had increasing ACTH after previous transsphenoidal surgery for Nelson's syndrome, and two patients without pituitary adenomas had recurrence of Cushing's disease after primary and ...

OBJECTIVE: To investigate the incidence of endocrinopathies in holoprosencephaly (HPE) and correlate the severity of the endocrinopathies with the neuroanatomic abnormalities. STUDY DESIGN: We reviewed the histories and medical records of 117 children with HPE for endocrinopathies and related treatments. Neuroimaging studies were graded for severity of HPE, hypothalamus non-separation, ...

BACKGROUND: Isolated growth hormone deficiency (IGHD) provides the ideal model to characterize GHD without interference from other pituitary deficiencies or their treatment. No study has addressed the question whether adult patients with IGHD differ in clinical presentation or in responsiveness to GH replacement from adult patients with multiple pituitary hormone ...

Acute adrenal failure is a potentially fatal condition if overlooked. Occasionally, acute adrenal insufficiency may ensue from bilateral adrenal haemorrhage in patients with known antiphospholipid syndrome (APS). APS is characterized by recurrent arterial and venous thrombosis, pregnancy complications and detection of autoantibodies to phospholipids. This syndrome may be associated with ...

There appears to be an allostatic shift in the hypothalamic-pituitary-adrenal (HPA) axis of animals exposed to chronic stress, such that vasopressinergic regulation of the HPA axis becomes critical for sustaining corticotroph responsiveness in the presence of high circulating glucocorticoid levels. It has been hypothesised that this is also a feature ...

Chronic growth hormone (GH)/insulin-like growth factor I (IGF-I) excess is associated with considerable mortality in acromegaly, but no data are available in pituitary gigantism. The aim of the study was to evaluate the long-term effects of early exposure to GH and IGF-I excess on cardiovascular and metabolic parameters in adult ...

AIM: The aim of this study was three-fold: 1) to quantify [131I]-6beta-iodomethyl-norcholesterol ([131I]-NP-59) adrenal uptake trend in patients with incidentalomas, 2) to identify a specific uptake trend (TREND) capable of characterising pre-clinical Cushing syndrome (PC-CS) patients, 3) to assess the clinical availability of TREND as a prognostic factor of late ...

Patients with hypopituitarism have an increased mortality from cardiovascular events. Reduced nocturnal blood pressure decline (non-dipping) and impaired glucose tolerance are considered as cardiovascular risk factors. To evaluate the role of these risk factors in patients with hypopituitarism we determined the 24-hour blood pressure regulation and glucose tolerance status in ...

OBJECTIVE: Optic nerve hypoplasia (ONH) is a heterogeneous disease with variable findings of pituitary insufficiency, CNS and neurodevelopmental abnormalities. We characterized the spectrum of endocrinopathy in a cohort of children with ONH and attempted to correlate the presence of different midline CNS findings with the degree of hypopituitarism. The correlation ...

OBJECTIVES: To determine the etiologies and associated endocrine disorders in children with central diabetes insipidus (DI). MATERIAL AND METHOD: The authors retrospectively reviewed the medical records of children with central DI, who were admitted at department of Pediatrics, King Chulalongkorn Memorial Hospital, between 2000 and 2004. Aims of this study ...