Hypopituitary

OBJECTIVE: Little is known of the effects of a fixed very low dose of growth hormone (GH) replacement on cardiovascular risk factors, bone mass, muscle strength and quality of life (QoL) in hypopituitary patients. DESIGN/PATIENTS/METHODS: This was an open-label randomized study performed at a single center. Consecutive hypopituitary patients with adult onset GH deficiency (GHD) and BMI ≥ 27 kg/m² were randomized to receive a very low fixed dose of GH (LG, n = 9) or a standard dose of GH (SG, n = 9)...

BACKGROUND: Shock is among the most common conditions that clinicians face in intensive care unit (ICU), of which hypovolemic shock is encountered most frequently; some patients instead suffer from neurogenic, cardiogenic, or infectious forms of shock. However, there are additional types of shock from unusual causes that are often undiagnosed. Here, we report the case of a patient who was initially misdiagnosed with hypovolemic shock, but exhibited persistent hypotension because of continuous fluid replacement and vasoactive drug administration, and was eventually diagnosed with hypopituitarism with crisis...

PURPOSE: We aimed to investigate the molecular basis underlying a novel phenotype including hypopituitarism associated with primary ovarian insufficiency. METHODS: We used next-generation sequencing to identify variants in all pedigrees. Expression of Rnpc3/RNPC3 was analyzed by in situ hybridization on murine/human embryonic sections. CRISPR/Cas9 was used to generate mice carrying the p.Leu483Phe pathogenic variant in the conserved murine Rnpc3 RRM2 domain. RESULTS: We described 15 patients from 9 pedigrees with biallelic pathogenic variants in RNPC3, encoding a specific protein component of the minor spliceosome, which is associated with a hypopituitary phenotype, including severe growth hormone (GH) deficiency, hypoprolactinemia, variable thyrotropin (also known as thyroid-stimulating hormone) deficiency, and anterior pituitary hypoplasia...

Introduction: Hypopituitarism is a rare, often underdiagnosed, complex hormonal disease caused by the decreased secretion of one or more hormones in the pituitary gland. The present study was designed to assess left ventricular (LV) rotational mechanics in patients with treated hypopituitarism. It was also aimed to find possible differences in these parameters according to the origin of hypopituitarism (congenital vs. acquired). Methods: The present prospective study involved 35 treated patients with hypopituitarism; however, 4 patients had to be excluded due to inferior image quality...

Growth hormone (GH) deficiency develops early in patients with hypothalamic-pituitary disorders and is therefore common among these patients. GH deficiency in adults is associated with increased morbidity, increased body fat mass, abdominal obesity, dyslipidaemia, reduced exercise capacity, impaired cardiac function as well as reduced self-reported well-being and impaired quality of life. Since recombinant human GH became available as replacement therapy more than 25 years ago, randomised controlled trials and long-term studies, together with meta-analyses, have shown improved outcomes in adult patients with hypopituitarism receiving GH...

Objective: To investigate the clinical features and risk factors of metabolic syndrome (MS) in adult hypopituitary patients (HP). Methods: Patients diagnosed with HP in the outpatient or inpatient department of the endocrinology department in West China Hospital from January,2012 to December,2019 were selected as the experimental group (HP group), and patients with normal pituitary function treated for saddle lesions were selected as the control group. HP patients with or without MS were divided into MS group and non-MS group HP patients were divided into four groups according to the level of growth hormone by the quartile method (GH>0...

OBJECTIVE: To analyze the clinical characteristics and MRI imaging of the patients with pituitary stalk interruption syndrome (PSIS) in the First Affiliated Hospital of Anhui Medical University in the past four years, and to achieve better comprehension of this disease. METHODS: Ten patients with PSIS (9 males, 1 female) in our hospital were retrospectively analyzed, regarding clinical manifestation, laboratory data and MRI imaging. RESULTS: The clinical features of 10 cases of PSIS were as follows: growth retardation, 55% of patients with hypogonadism, 45% of patients with short stature; the dystocia rate at birth is as high as 90%, of which 61% are breech presentation and 27% are foot presentation; 10 patients with PSIS, the height was between 135 cm and 180 cm, the weight was between 31 kg and 93 kg, the lower part was larger than the upper part, and the finger distance was smaller than the height; bone age is behind 3~7 years old; normal intelligence; 10 patients have clinical manifestations of hypopituitary hypofunction; no manifestations of diabetes insipidus; MRI imaging of pituitary showed that the pituitary stalks were not shown, atrophy or aplasia of anterior pituitary, posterior pituitary ectopic...

OBJECTIVE: To investigate the prevalence of NAFLD in adolescents and young adults with hypopituitarism, and to examine associations for GH deficiency with occurrence of NAFLD. METHODS: A cross-sectional study for determination of NAFLD prevalence included 76 patients with childhood-onset hypopituitarism and 74 controls matched by age and body mass index (BMI). We investigated the prevalence of NAFLD in adolescents and young adults with hypopituitarism and in the age- and BMI-matched controls...

PURPOSE: Many patients who undergo bone marrow transplantation (BMT) in adulthood experience unexplained chronic fatigue which can have a major impact on their health-related quality of life (QoL). Pre-BMT treatment regimens increase the risk of developing acquired growth hormone deficiency (GHD), which results in a clinical syndrome with decreased energy and has additionally been linked to metabolic syndrome. METHODS: Using the gold-standard insulin hypoglycemic test (IHT), we evaluated the prevalence of GHD in 18 post-BMT adult patients with unexplained chronic fatigue, as well as the correlation between peak serum GH response and QoL scores, the metabolic syndrome, and insulin resistance...

OBJECTIVES: Nonfunctionning pituitary macroadenomas (NFPMA) are benign tumors that cause symptoms of mass effects including hypopituitarism. Their primary treatment is transsphenoidal surgery. We aimed to determine the outcome of pituitary hormone deficits after surgical treatment of NFPMA and to identify factors predicting hormonal recovery. DESIGN: We retrospectively included 246 patients with NFPMA diagnosed and operated in one of the two participating centers...

BACKGROUND: Hypopituitarism in the elderly population is an underdiagnosed condition and may increase comorbidities related to glucose metabolism, dyslipidemia, and cardiovascular risk factors. Optimization of hormone replacement that considers alterations in clearance rates of hormones, interaction with other medications, and evaluation of the risk-benefit ratio of treatment is a big challenge for clinical practice. OBJECTIVES: This study aimed to evaluate classic cardiovascular risk factors in hypopituitary septuagenarians and octagenarians by diagnosis and after long-term hormone replacement...

The hypothalamus and pituitary have been identified to play essential roles in maintaining homeostasis. Various diseases can disrupt the functions of these systems, which can often result in serious lifelong symptoms. The current treatment for hypopituitarism involves hormone replacement therapy. However, exogenous drug administration cannot mimic the physiological changes that are a result of hormone requirements. Therefore, patients are at a high risk of severe hormone deficiency, including adrenal crisis...

SUMMARY: Wilson's disease (WD) is a rare disorder of copper metabolism usually presenting with variable liver damage and neuropsychiatric symptoms. Here we report a 39-year-old Taiwanese female with late manifestation of WD presenting with gonadotroph, thyreotroph and corticotroph hypopituitarism. Molecular genetic testing revealed compound heterozygosity for two mutations in exons 12 and 14 (c.2828G>A and c.3140A>T). Copper-chelating therapy with D-penicillamine and zinc was initiated along with supplementation of hydrocortisone and L-thyroxine...

Context: Pharmacokinetic properties of cortisone acetate (CA) and hydrocortisone (HC) differ because CA needs to be converted into cortisol to become active. Objective: This work analyzed the metabolic consequences of switching CA to an equivalent daily dose of HC in patients with secondary adrenal insufficiency (SAI). Design: This was a post hoc analysis from a prospective study including individuals with hypopituitarism receiving growth hormone replacement...

In a seminal paper from 1990, Rosen and Bengtsson suggested that hypopituitary patients with a presumed growth hormone (GH) deficiency (GHD) have an excess mortality. Later studies have confirmed this finding but have also shown that the cause of the increased risk of death in these patients is multifactorial, including unreplaced GHD as well as non-physiological replacement therapy of other deficiencies, the etiology of hypopituitarism, and the side effects of tumor treatment. Only a few studies have investigated mortality in hypopituitary patients with GHD receiving GH replacement therapy (GHRT): these studies are retrospective observational studies with a wide range of underlying diseases but most of them show a mortality that is not different from the general population...

BACKGROUND: Glucocorticoid therapy is the most common cause of iatrogenic osteoporosis. Less is known regarding the effect of glucocorticoids when used as replacement therapy on bone remodelling in patients with adrenal insufficiency. Enhanced intracellular conversion of inactive cortisone to active cortisol, by 11 beta-hydroxysteroid dehydrogenase type 1(11β-HSD1) and other enzymes leading to alterations in glucocorticoid metabolism, may contribute to a deleterious effect on bone health in this patient group...

CONTEXT: Acylated ghrelin increases growth hormone (GH) and adrenocorticotrophic hormone (ACTH) secretion from the anterior pituitary gland. Additionally, it increases free fatty acid levels independently of GH and ACTH, but the impact of ghrelin on fatty acid turnover has not been determined. This study was designed to test whether acylated ghrelin directly increases the turnover rate of fatty acids. DESIGN: Eight hypopituitary patients on stable replacement with GH and hydrocortisone were included in a randomized, double-blinded, placebo-controlled crossover study including two study days: (a) infusion of acylated ghrelin and (b) infusion of saline...

PURPOSE: To evaluate the prevalence of metabolic syndrome (MetS) and its components in adult hypopituitary patients. PATIENTS AND METHODS: Retrospective, cross-sectional analysis of a cohort of hypopituitary adult patients followed in a single reference center for pituitary diseases. MetS was defined by the National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATP III) criteria. Patients with 18 years or older, presenting two or more anterior pituitary deficiencies associated or not with diabetes insipidus (DI), were included, while patients with hypopituitarism due to Acromegaly or Cushing's disease were excluded...

CONTEXT: Clinically nonfunctioning pituitary adenomas (CNFPAs) typically remain undetected until mass effect symptoms develop. However, currently, head imaging is performed commonly for many other indications, which may increase incidental discovery of CNFPAs. Since current presentation and outcome data are based on older, retrospective series, a prospective characterization of a contemporary CNFPA cohort was needed. OBJECTIVE: To determine the prevalence of incidental presentation and hypopituitarism and its predictors in a CNFPA cohort that spanned 6 to 9 mm micro- to macroadenoma included observational and surgical therapy...

When initiating growth hormone replacement therapy, it is important to consider a patient's other pituitary hormone replacement medications, as adjustments are often necessary. Growth hormone therapy can increase the metabolism of hydrocortisone or endogenous cortisol, unmasking borderline ACTH deficiency and leading to the development of adrenal insufficiency and adrenal crisis. In addition, growth hormone can enhance the metabolism of thyroxine to triiodothyronine, uncovering borderline TSH deficiency. In many patients, thyroid hormone replacement therapy must be started, or the dose of levothyroxine must be increased...