Um caso de hipopituitarismo adquirido durante a vida do indivíduo.
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Um caso de hipopituitarismo adquirido durante a vida do indivíduo.
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Publicações mais relevantes
Management of infertility in women with hypothalamic hypogonadotropic hypogonadism: an expert opinion.
Hypothalamic gonadotropin-releasing hormone (GnRH) plays a central role in regulating the pituitary-gonadal axis. The pulsatility of GnRH release is critical for maintaining the function of GnRH receptors and the secretion pattern of gonadotropins, namely follicle-stimulating hormone (FSH) and luteinizing hormone (LH), which regulate endocrine function and follicular growth and maturation. During the luteal phase, LH is crucial for supporting a functional corpus luteum and stimulating it to produce progesterone, estradiol and relaxin.Hypothalamic hypogonadotropic hypogonadism originates from a deficiency in GnRH secretion. Low circulating gonadotropin levels subsequently lead to reduced ovarian function and anovulation. This condition may be congenital or acquired, for example through functional hypothalamic amenorrhoea (FHA) or FHA combined with polycystic ovarian morphology (PCOM). Pulsatile GnRH therapy plays a pivotal role in restoring the physiological menstrual cycle and selecting a dominant follicle in these women, thereby inducing ovulation and achieving fertility. There is extensive literature accounting for a high ovulation rate and consequently high pregnancy and birth rates per cycle, with a lower risk of adverse outcomes. In this review, based on clinical evidence and published studies, we provide recommendations for the alternative treatment of infertility in women with congenital hypothalamic hypogonadotropic hypogonadism (CHH) and FHA (with or without PCOM), until pulsatile GnRH therapy becomes available again or in countries where this device is not marketed. Starting doses and adjustments should be made according to the aetiology of hypothalamic hypogonadotropic hypogonadism and other patient parameters. In all cases, luteal phase support is imperative and should ideally be provided by hCG injections to optimize corpus luteum functions. When pulsatile GnRH therapy is not available, and to ensure the effective treatment of female infertility due to FHA (with or without PCOS) or hypothalamic CHH, we advise physicians to optimise stimulation with exogenous gonadotropins according to the cause of hypothalamic hypogonadotropic hypogonadism. In all cases, providing luteal phase support by optimising corpus luteum function is mandatory.
Acquired transient vasopressin deficiency by cannabinoids and other substances.
Water movement across cell membranes through aquaporin water channels creates osmotic equilibrium between extracellular and intracellular fluid compartments. Plasma osmolality is tightly regulated by the kidneys and brain through the process of osmoregulation. The antidiuretic hormone, arginine vasopressin (AVP), is normally released from the posterior pituitary in response to increased osmolality or decreased intravascular volume. Defects in the synthesis or release of AVP result in AVP deficiency (AVP-D) and the syndrome of central diabetes insipidus, characterized by inappropriate aquaresis leading to hyperosmolality and insatiable thirst. While most cases of AVP-D are due to local mechanical, infiltrative, compressive, infectious, or inflammatory processes, some recreational and pharmacological substances can cause AVP-D. In this review, we discuss the history and current knowledge about these substances, including cannabinoids, ethanol, κ opioid receptor agonists, phenytoin, and anesthetic agents.
GH Response to Glucagon in Transition: Role of BMI and Etiology in Childhood-Onset GH Deficiency.
The glucagon stimulation test (GST) is increasingly used as an alternative to the insulin tolerance test for diagnosing persistent growth hormone (GH) deficiency during transition, though its accuracy and appropriate cutoff values are still uncertain. To investigate the GH response to GST in transitional-age patients with childhood-onset GHD (CO-GHD), with a focus on the influence of BMI and underlying etiology. A total of 180 patients with CO-GHD (median age 17.39 years) underwent GST. They were grouped based on the number of pituitary deficiencies and MRI findings into I-GHD (isolated, n=80), OM-GHD (1-2 deficiencies with congenital or acquired anomalies, n=63), and OS-GHD (≥3 deficiencies with complex CNS abnormalities, n=37). Additionally, patients were classified by BMI as normal weight, overweight, or obesity, according to age-appropriate BMI criteria. Childhood cancer survivors (CCS) accounted for 42% of the cohort. Peak GH response to GST showed a significant inverse association with the severity of pituitary dysfunction (p < 0.001) and an inverse correlation with BMI SDS (ρ = -0.46, p < 0.001). However, adjusting by disease group strongly reduced the impact of BMI on the GST response. When stratified by etiology or CCS status, GH peaks were primarily influenced by hypothalamic-pituitary damage with BMI showing a minimal effect. The GST provides valuable insights into GH deficiency in transitional-age patients with CO-GHD. GH response is primarily influenced by the severity of pituitary dysfunction, with BMI playing a minimal role once adjusted for etiology.
Hypoprolactinemia: Biology, Clinical Relevance, and Diagnostic Challenges.
Prolactin (PRL) is a pleiotropic hormone, traditionally associated with lactation, but now recognised for its role in reproduction, metabolism, and neuroendocrine and immune regulation. While the clinical features and consequences of hyperprolactinemia are well known, hypoprolactinemia is poorly understood. The causes of PRL deficiency range from genetic mutation to acquired pituitary diseases and rare isolated disorders. Its diagnostic evaluation is often challenging, due to the lack of standardised lower reference limits. Nonetheless, growing evidence links hypoprolactinemia not only to postpartum agalactia, but also to reproductive and sexual dysfunction, and to metabolic alterations and increased cardiometabolic risk. Data from animal models and rare human cases further suggest that PRL exerts unique biological functions that are not fully compensated by other pituitary hormones. PRL measurement, when interpreted alongside gonadotropin levels and clinical context, may help in the differential diagnosis of secondary amenorrhoea. Markedly low PRL levels-especially in association with hypogonadotropic profiles-may support a diagnosis of functional hypothalamic amenorrhoea. PRL should be recognised as a hormone whose deficiency may cause broader systemic disturbances. A comprehensive understanding of hypoprolactinemia is essential to better define its diagnostic criteria and clinical significance.
Impact of traumatic brain injury severity on anterior pituitary function: A prospective study.
India experiences the highest number of road traffic fatalities globally. Acquired hypopituitarism is a common sequela in patients who sustain traumatic brain injury (TBI). This study aimed to investigate the prevalence and imaging characteristics of hypopituitarism in patients with TBI at a tertiary care centre in North India. Our prospective study included 76 patients with TBI (mild, moderate, or severe), whom we followed for 24 weeks at a tertiary care centre in North India. All included subjects underwent assessments of anterior pituitary hormones (LH, FSH, TSH, T4, cortisol, testosterone, estrogen) at baseline and again at 24 weeks, as well as an MRI. Those who had low cortisol level were subjected to glucagon stimulation test and cortisol and growth hormone was measured after stimulation in these subjects. We recorded the severity of traumatic brain injury, findings from CT scans such as skull fractures, and imaging characteristics of pituitary gland in all the patients by magnetic resonance imaging (MRI). Appropriate statistical analyses, including logistic regression, were utilized to determine the determinants of hypopituitarism. Among the 76 patients, the prevalence of hypopituitarism was 11.84 % in the acute stage and 2.63 % at 24 weeks. Hypopituitarism significantly correlated with injury severity (p < 0.001) and imaging abnormalities observed on MRI. The main imaging findings on MRI were heterogeneous signal intensity, subacute haemorrhage in the anterior pituitary, and reduced pituitary height. A statistically significant decrease was observed in LH (p = 0.009) and FSH levels (p = 0.039) from baseline to 24 weeks. The severity of the injury and the presence of base skull fractures were significantly associated with hypopituitarism (p < 0.001). Our results highlight the importance of checking pituitary function in TBI patients, particularly those with moderate to severe injuries and skull base fractures, to quickly find and treat hormonal deficiencies, which can improve long-term results. Future studies should concentrate on longer follow-up periods and more sophisticated imaging methods to gain a more profound understanding of the mechanisms underlying post-traumatic hypopituitarism.
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Investigation of the Effects of Hypothyroidism Duration on Hearing Function: An Experimental Animal Study.
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Primary adrenocortical insufficiency in patients with AIDS in Wuhan, China: 3 cases report and literature review.
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📚 EuropePMCmostrando 166
Management of infertility in women with hypothalamic hypogonadotropic hypogonadism: an expert opinion.
Reproductive biology and endocrinology : RB&EAcquired transient vasopressin deficiency by cannabinoids and other substances.
EndocrinologyGH Response to Glucagon in Transition: Role of BMI and Etiology in Childhood-Onset GH Deficiency.
The Journal of clinical endocrinology and metabolismHypoprolactinemia: Biology, Clinical Relevance, and Diagnostic Challenges.
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Nutrition journalA Rare SPRY4 Gene Mutation Is Associated With Anosmia and Adult-Onset Isolated Hypogonadotropic Hypogonadism.
Frontiers in endocrinologyPathogenesis of Anti-PIT-1 Antibody Syndrome: PIT-1 Presentation by HLA Class I on Anterior Pituitary Cells.
Journal of the Endocrine SocietyAutoimmune Pituitary Disease: New Concepts With Clinical Implications.
Endocrine reviewsSystematic profiling of clinical missence mutation effects on the intermolecular interaction between human growth hormone and its receptor in isolated growth hormone deficiency.
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Endocrine connectionsTranscriptomics and machine learning predict diagnosis and severity of growth hormone deficiency.
JCI insightSemen quality in patients with pituitary disease and adult-onset hypogonadotropic hypogonadism.
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European journal of endocrinologyLong-Standing Isolated Autoimmune Hypothalamitis Diagnosed with Endoscopic Transventricular Biopsy.
World neurosurgeryHypopituitarism is associated with lower oxytocin concentrations and reduced empathic ability.
EndocrineOpioid-induced hyponatremia in a patient with central diabetes insipidus: independence from ADH.
Journal of pediatric endocrinology & metabolism : JPEMCentral hypothyroidism - a neglected thyroid disorder.
Nature reviews. EndocrinologyStem cell therapy and its potential role in pituitary disorders.
Current opinion in endocrinology, diabetes, and obesityGrowth hormone deficiency and human immunodeficiency virus.
Best practice & research. Clinical endocrinology & metabolismClassical and non-classical causes of GH deficiency in adults.
Best practice & research. Clinical endocrinology & metabolismPattern of Thyroid Disorders in Children and Adolescents Seen at the Lagos University Teaching Hospital, Nigeria, Over a 10-year Period.
Nigerian medical journal : journal of the Nigeria Medical AssociationCholestasis caused by panhypopituitarism and acquired cytomegalovirus infection in a 2-month-old male infant: A case report.
MedicineA Novel Clinical Entity of Autoimmune Endocrinopathy: Anti-PIT-1 Antibody Syndrome.
Frontiers of hormone researchCENTRAL DIABETES INSIPIDUS: CLINICAL CHARACTERISTICS AND LONG-TERM COURSE IN A LARGE COHORT OF ADULTS.
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical EndocrinologistsA novel thymoma-associated autoimmune disease: Anti-PIT-1 antibody syndrome.
Scientific reportsUntangling the Gordian knot of HIV, stress, and cognitive impairment.
Neurobiology of stressBaseline Characteristics and Gender Differences in Prepubertal Children Treated with Growth Hormone in Europe, USA, and Japan: 25 Years' KIGS® Experience (1987-2012) and Review.
Hormone research in paediatricsUK Iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches.
Acta neuropathologicaLong-term safety of growth hormone replacement therapy after childhood medulloblastoma and PNET: it is time to set aside old concerns.
Journal of neuro-oncologyClinical Applications of Gonadotropins in the Male.
Progress in molecular biology and translational scienceHypogonadotropic Hypogonadism and Short Stature in Patients with Diabetes Due to Neurogenin 3 Deficiency.
The Journal of clinical endocrinology and metabolismHypopituitarism patterns among adult males with prolactinomas.
Clinical neurology and neurosurgeryThe physiology of functional hypothalamic amenorrhea associated with energy deficiency in exercising women and in women with anorexia nervosa.
Hormone molecular biology and clinical investigationBurden of Growth Hormone Deficiency and Excess in Children.
Progress in molecular biology and translational scienceCompensated reduction in Leydig cell function is associated with lower semen quality variables: a study of 8182 European young men.
Human reproduction (Oxford, England)Reversible growth failure and complete GH deficiency in a 4-year-old girl with very early Hashimoto's thyroiditis and subsequent hyperplasia of pituitary thyrotroph cells.
European journal of pediatricsCauses of hypogonadotropic hypogonadism predict response to gonadotropin substitution in adults.
Andrology[Endocrine consequences in young adult survivors of childhood cancer treatment].
Annales d'endocrinologieAcquired Hypogonadotropic Hypogonadism (AHH) in Thalassaemia Major Patients: An Underdiagnosed Condition?
Mediterranean journal of hematology and infectious diseasesAtypical defects resulting in growth hormone insensitivity.
Growth hormone & IGF research : official journal of the Growth Hormone Research Society and the International IGF Research SocietyHypotestosteronaemia in the aging male: should we treat it?
Swiss medical weekly[A Novel Clinical Entity "Anti-PIT-1 Antibody Syndrome"--Autoimmunity against a Transcription Factor].
Rinsho byori. The Japanese journal of clinical pathologyThe practical management of testosterone deficiency in men.
Nature reviews. UrologyAutosomal Dominant Growth Hormone Deficiency (Type II).
Pediatric endocrinology reviews : PERSubjects with isolated GH deficiency due to a null GHRHR mutation eat proportionally more, but healthier than controls.
EndocrineAge-related testosterone decline is due to waning of both testicular and hypothalamic-pituitary function.
The aging male : the official journal of the International Society for the Study of the Aging MaleTreatment situation of male hypogonadotropic hypogonadism in pediatrics and proposal of testosterone and gonadotropins replacement therapy protocols.
Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric EndocrinologyAnti-PIT-1 antibody syndrome; a novel clinical entity leading to hypopituitarism.
Pediatric endocrinology reviews : PERNatural history of growth hormone deficiency in a pediatric cohort.
Hormone research in paediatricsSecondary IGF-I deficiency as a prognostic factor of growth hormone (GH) therapy effectiveness in children with isolated, non-acquired GH deficiency.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationReversal of idiopathic hypogonadotropic hypogonadism: a cohort study in Chinese patients.
Asian journal of andrologyAssociações
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Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Management of infertility in women with hypothalamic hypogonadotropic hypogonadism: an expert opinion.
- Acquired transient vasopressin deficiency by cannabinoids and other substances.
- GH Response to Glucagon in Transition: Role of BMI and Etiology in Childhood-Onset GH Deficiency.
- Hypoprolactinemia: Biology, Clinical Relevance, and Diagnostic Challenges.
- Impact of traumatic brain injury severity on anterior pituitary function: A prospective study.
- Investigation of the Effects of Hypothyroidism Duration on Hearing Function: An Experimental Animal Study.
- Primary adrenocortical insufficiency in patients with AIDS in Wuhan, China: 3 cases report and literature review.
- First in class monoclonal antibody potentiating human follicle stimulating hormone activity improves spermatogenesis in azoospermic rodent models.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:95502(Orphanet)
- MONDO:0019832(MONDO)
- GARD:19278(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55788906(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
