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1.
Rev Endocr Metab Disord ; 25(3): 453-455, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38801648

RESUMEN

Hypopituitarism is a rare endocrine disorder characterized by insufficient hormone secretion from the pituitary gland. This condition leads to deficient production of one or more pituitary hormones, including growth hormone (GH), thyroid-stimulating hormone (TSH), luteinizing hormone (LH), follicle-stimulating hormone (FSH), adrenocorticotropic hormone (ACTH), and antidiuretic hormone (ADH), also called arginine vasopressin (AVP). Symptoms vary widely and are often not, late recognized.Diagnosis typically involves a thorough clinical evaluation, hormone level assessments, and neuroimaging studies to identify underlying causes. Treatment aims to replace deficient hormones and address the underlying cause and related complications when possible. In this special issue we address diagnosis, comorbidities, and management of hypopituitarism. We hope that it will help healthcare professionals to manage their patients.


Asunto(s)
Hipopituitarismo , Humanos , Hipopituitarismo/terapia , Hipopituitarismo/diagnóstico
2.
Rev Endocr Metab Disord ; 25(3): 513-539, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38565758

RESUMEN

The results of many studies in recent years indicate a significant impact of pituitary function on bone health. The proper function of the pituitary gland has a significant impact on the growth of the skeleton and the appearance of sexual dimorphism. It is also responsible for achieving peak bone mass, which protects against the development of osteoporosis and fractures later in life. It is also liable for the proper remodeling of the skeleton, which is a physiological mechanism managing the proper mechanical resistance of bones and the possibility of its regeneration after injuries. Pituitary diseases causing hypofunction and deficiency of tropic hormones, and thus deficiency of key hormones of effector organs, have a negative impact on the skeleton, resulting in reduced bone mass and susceptibility to pathological fractures. The early appearance of pituitary dysfunction, i.e. in the pre-pubertal period, is responsible for failure to achieve peak bone mass, and thus the risk of developing osteoporosis in later years. This argues for the need for a thorough assessment of patients with hypopituitarism, not only in terms of metabolic disorders, but also in terms of bone disorders. Early and properly performed treatment may prevent patients from developing the bone complications that are so common in this pathology. The aim of this review is to discuss the physiological, pathophysiological, and clinical insights of bone involvement in pituitary disease.


Asunto(s)
Hipopituitarismo , Humanos , Hipopituitarismo/terapia , Hipopituitarismo/fisiopatología , Hipopituitarismo/etiología , Hipopituitarismo/diagnóstico , Osteoporosis/terapia , Osteoporosis/etiología , Osteoporosis/diagnóstico , Huesos/metabolismo , Densidad Ósea/fisiología
3.
Ann Endocrinol (Paris) ; 85(4): 327-339, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38452869

RESUMEN

Pituitary deficiency, or hypopituitarism, is a rare chronic disease. It is defined by insufficient synthesis of one or more pituitary hormones (growth hormone, TSH, ACTH, LH-FSH, prolactin), whether or not associated with arginine vasopressin deficiency (formerly known as diabetes insipidus). In adult patients, it is usually acquired (notably during childhood), but can also be congenital, due to abnormal pituitary development. The present study focuses on congenital pituitary deficiency in adults, from diagnosis to follow-up, including special situations such as pregnancy or the elderly. The clinical presentation is highly variable, ranging from isolated deficit to multiple deficits, which may be part of a syndromic form or not. Diagnosis is based on a combination of clinical, biological (assessment of all hormonal axes), radiological (brain and hypothalamic-pituitary MRI) and genetic factors. Treatment consists in hormonal replacement therapy, adapted according to the period of life and the deficits, which may be progressive. Comorbidities, risk of complications and acute decompensation, and the impact on fertility and quality of life all require adaptative multidisciplinary care and long-term monitoring.


Asunto(s)
Hipopituitarismo , Humanos , Hipopituitarismo/diagnóstico , Hipopituitarismo/terapia , Francia/epidemiología , Adulto , Femenino , Embarazo , Terapia de Reemplazo de Hormonas/métodos , Masculino , Anciano , Hipófisis/anomalías
4.
Arch Pediatr ; 31(3): 165-171, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38538470

RESUMEN

Hypopituitarism (or pituitary deficiency) is a rare disease with an estimated prevalence of between 1/16,000 and 1/26,000 individuals, defined by insufficient production of one or several anterior pituitary hormones (growth hormone [GH], thyroid-stimulating hormone [TSH], adrenocorticotropic hormone [ACTH], luteinizing hormone [LH], follicle-stimulating hormone [FSH], prolactin), in association or not with diabetes insipidus (antidiuretic hormone [ADH] deficiency). While in adults hypopituitarism is mostly an acquired disease (tumors, irradiation), in children it is most often a congenital condition, due to abnormal pituitary development. Clinical symptoms vary considerably from isolated to combined deficiencies and between syndromic and non-syndromic forms. Early signs are non-specific but should not be overlooked. Diagnosis is based on a combination of clinical, laboratory (testing of all hormonal axes), imaging (brain magnetic resonance imaging [MRI] with thin slices centered on the hypothalamic-pituitary region), and genetic (next-generation sequencing of genes involved in pituitary development, array-based comparative genomic hybridization, and/or genomic analysis) findings. Early brain MRI is crucial in neonates or in cases of severe hormone deficiency for differential diagnosis and to inform syndrome workup. This article presents recommendations for hormone replacement therapy for each of the respective deficient axes. Lifelong follow-up with an endocrinologist is required, including in adulthood, with multidisciplinary management for patients with syndromic forms or comorbidities. Treatment objectives include alleviating symptoms, preventing comorbidities and acute complications, and optimal social and educational integration.


Asunto(s)
Hormona de Crecimiento Humana , Hipopituitarismo , Adulto , Niño , Recién Nacido , Humanos , Hibridación Genómica Comparativa , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Hipófisis/patología , Hormona Adrenocorticotrópica
5.
Zhonghua Yi Xue Za Zhi ; 104(4): 241-246, 2024 Jan 23.
Artículo en Chino | MEDLINE | ID: mdl-38246768

RESUMEN

More and more clinical evidence confirms that hypopituitary dysfunction can lead to liver cirrhosis. The causes of hypopituitary dysfunction include sellarneoplastic diseases, hereditary diseases, perinatal adverse events, etc. Clinically, growth hormone deficiency (GHD) often the first cause in hypopituitary dysfunction.Hypopituitary dysfunction, especially GHD, can lead to liver cirrhosis, which in turn can result in damage to multiple organs such as the secondary lungs, spleen, and heart.Therefore, its clinical manifestations are complex, and the patients are first diagnosed in various departments.Reliable clinical observations have shown that GH replacement therapy at the early stage of the disease can effectively prevent and reverse the progression of cirrhosis, or even avoid inappropriate or unnecessary combined transplantation of liver or liver-related organs.Multidisciplinary collaboration and attention to the new findings that hypopituitary dysfunction can lead to liver cirrhosis will help to improve the accurate diagnosis and treatment of liver cirrhosis.


Asunto(s)
Hipopituitarismo , Enfermedades de la Hipófisis , Femenino , Embarazo , Humanos , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/terapia , Hipófisis
6.
Rev Endocr Metab Disord ; 25(3): 555-573, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38112850

RESUMEN

Hypopituitarism, or the failure to secrete hormones produced by the anterior pituitary (adenohypophysis) and/or to release hormones from the posterior pituitary (neurohypophysis), can be congenital or acquired. When more than one pituitary hormone axis is impaired, the condition is known as combined pituitary hormone deficiency (CPHD). The deficiency may be primarily due to a hypothalamic or to a pituitary disorder, or concomitantly both, and has a negative impact on target organ function. This review focuses on the pathophysiology, diagnosis and management of anterior pituitary hormone deficiency in the pediatric age. Congenital hypopituitarism is generally due to genetic disorders and requires early medical attention. Exposure to toxicants or intrauterine infections should also be considered as potential etiologies. The molecular mechanisms underlying the fetal development of the hypothalamus and the pituitary are well characterized, and variants in the genes involved therein may explain the pathophysiology of congenital hypopituitarism: mutations in the genes expressed in the earliest stages are usually associated with syndromic forms whereas variants in genes involved in later stages of pituitary development result in non-syndromic forms with more specific hormone deficiencies. Tumors or lesions of the (peri)sellar region, cranial radiation therapy, traumatic brain injury and, more rarely, other inflammatory or infectious lesions represent the etiologies of acquired hypopituitarism. Hormone replacement is the general strategy, with critical periods of postnatal life requiring specific attention.


Asunto(s)
Hipopituitarismo , Humanos , Hipopituitarismo/diagnóstico , Hipopituitarismo/terapia , Niño , Hormonas Adenohipofisarias/deficiencia , Hormonas Adenohipofisarias/metabolismo
7.
Endocrinol Diabetes Nutr (Engl Ed) ; 70(9): 584-591, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37977921

RESUMEN

Traumatic brain injury (TBI) is associated with hypopituitarism with a variable incidence, depending on the time and methods used to diagnosis, and on factors related to the trauma, such as its severity, its anatomical location and the drugs used in the acute phase. The pituitary gland can be damaged directly by the impact or secondary to factors such as ischemia, inflammation, excitotoxicity or immunity. In acute phases ACTH deficiency is the most relevant, since failure to detect and treat it can compromise the patient's life. Clinical manifestations are typical of each hormone deficient axes, although the combination hypopituitarism-trauma has been associated with cognitive deterioration, worse metabolic profile and greater impairment of quality of life. One of the clinical challenges is to determine which patients benefit from a systematic hormonal evaluation, and therefore from hormone replacement, and what is the appropriate time to do so and the most suitable diagnostic methods.


Asunto(s)
Lesiones Traumáticas del Encéfalo , Lesiones Encefálicas , Hipopituitarismo , Humanos , Adulto , Neuroendocrinología , Calidad de Vida , Lesiones Encefálicas/complicaciones , Lesiones Encefálicas/epidemiología , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Lesiones Traumáticas del Encéfalo/complicaciones , Lesiones Traumáticas del Encéfalo/terapia , Lesiones Traumáticas del Encéfalo/metabolismo , Hormonas/uso terapéutico
8.
J Clin Endocrinol Metab ; 108(11): 3009-3021, 2023 10 18.
Artículo en Inglés | MEDLINE | ID: mdl-37246615

RESUMEN

CONTEXT: Pathologies attributed to perturbations of the GH/IGF-I axis are among the most common referrals received by pediatric endocrinologists. AIM: In this article, distinctive cased-based presentations are used to provide a practical and pragmatic approach to the management of pediatric growth hormone deficiency (GHD). CASES: We present 4 case vignettes based on actual patients that illustrate (1) congenital GHD, (2) childhood GHD presenting as failure to thrive, (3) childhood GHD presenting in adolescence as growth deceleration, and (4) childhood-onset GHD manifesting as metabolic complications in adolescence. We review patient presentation and a management approach that aims to highlight diagnostic considerations for treatment based on current clinical guidelines, with mention of new therapeutic and diagnostic modalities being used in the field. CONCLUSION: Pediatric GHD is diverse in etiology and clinical presentation. Timely management has the potential not only to improve growth but can also ameliorate or even mitigate adverse metabolic outcomes, which can be directly attributed to a GH deficient state.


Asunto(s)
Enanismo Hipofisario , Hormona de Crecimiento Humana , Hipopituitarismo , Adolescente , Niño , Humanos , Enanismo Hipofisario/terapia , Enanismo Hipofisario/tratamiento farmacológico , Hormona del Crecimiento/metabolismo , Hormona de Crecimiento Humana/uso terapéutico , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Factor I del Crecimiento Similar a la Insulina/metabolismo
9.
JAMA ; 329(16): 1386-1398, 2023 04 25.
Artículo en Inglés | MEDLINE | ID: mdl-37097352

RESUMEN

Importance: Pituitary adenomas are neoplasms of the pituitary adenohypophyseal cell lineage and include functioning tumors, characterized by the secretion of pituitary hormones, and nonfunctioning tumors. Clinically evident pituitary adenomas occur in approximately 1 in 1100 persons. Observations: Pituitary adenomas are classified as either macroadenomas (≥10 mm) (48% of tumors) or microadenomas (<10 mm). Macroadenomas may cause mass effect, such as visual field defects, headache, and/or hypopituitarism, which occur in about 18% to 78%, 17% to 75%, and 34% to 89% of patients, respectively. Thirty percent of pituitary adenomas are nonfunctioning adenomas, which do not produce hormones. Functioning tumors are those that produce an excess of normally produced hormones and include prolactinomas, somatotropinomas, corticotropinomas, and thyrotropinomas, which produce prolactin, growth hormone, corticotropin, and thyrotropin, respectively. Approximately 53% of pituitary adenomas are prolactinomas, which can cause hypogonadism, infertility, and/or galactorrhea. Twelve percent are somatotropinomas, which cause acromegaly in adults and gigantism in children, and 4% are corticotropinomas, which secrete corticotropin autonomously, resulting in hypercortisolemia and Cushing disease. All patients with pituitary tumors require endocrine evaluation for hormone hypersecretion. Patients with macroadenomas additionally require evaluation for hypopituitarism, and patients with tumors compressing the optic chiasm should be referred to an ophthalmologist for formal visual field testing. For those requiring treatment, first-line therapy is usually transsphenoidal pituitary surgery, except for prolactinomas, for which medical therapy, either bromocriptine or cabergoline, is usually first line. Conclusions and Relevance: Clinically manifest pituitary adenomas affect approximately 1 in 1100 people and can be complicated by syndromes of hormone excess as well as visual field defects and hypopituitarism from mass effect in larger tumors. First-line therapy for prolactinomas consists of bromocriptine or cabergoline, and transsphenoidal pituitary surgery is first-line therapy for other pituitary adenomas requiring treatment.


Asunto(s)
Adenoma , Neoplasias Hipofisarias , Adulto , Niño , Femenino , Humanos , Embarazo , Adenoma/complicaciones , Adenoma/diagnóstico , Adenoma/metabolismo , Adenoma/terapia , Hormona Adrenocorticotrópica/biosíntesis , Bromocriptina/uso terapéutico , Cabergolina/uso terapéutico , Hormona de Crecimiento Humana/biosíntesis , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/metabolismo , Hipopituitarismo/terapia , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/metabolismo , Neoplasias Hipofisarias/terapia , Prolactinoma/diagnóstico , Prolactinoma/etiología , Prolactinoma/metabolismo , Prolactinoma/terapia
10.
Dtsch Med Wochenschr ; 148(7): 386-394, 2023 03.
Artículo en Alemán | MEDLINE | ID: mdl-36940689

RESUMEN

Pituitary insufficiency is a partial or complete failure of secretion of one or more hormones from the pituitary gland. The pituitary gland is located in the hypophysial fossa of the sella turcica of the os sphenoidale and produces ACTH, LH, FSH, GH, TSH, and prolactin. Pituitary insufficiency can be caused by acute damage, such as secondary to traumatic brain injury. It can also be a result of chronic alterations, such as increasing tumor expansion.Pituitary insufficiency often presents with nonspecific symptoms (e.g. fatigue, listlessness, decreased performance, sleep disturbances, weight change) that leads to a challenging and sometimes delayed diagnosis. The symptoms correspond to the failure of the corresponding endorgans. Occasionally, symptoms such as a loss of libido, secondary amenorrhea or nausea in stressful situations are diagnostically indicative.Further clarification includes a clinical examination with endocrinological testing of the pituitary function. Alteration of pituitary hormone secretion can also occur physiologically as in pregnancy, depression or obesity. Substitution therapy of the failed corticotropic, thyrotropic and gonadotropic axis is corresponding to the therapy of a primary endorgan insufficiency. Adequate diagnosis and treatment of pituitary insufficiency is important, as this may prevent life-threatening crises such as an adrenal crisis.


Asunto(s)
Hipopituitarismo , Prolactina , Femenino , Embarazo , Humanos , Hipófisis , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Hormonas
11.
Hormones (Athens) ; 22(2): 305-309, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-36905572

RESUMEN

PURPOSE: Hypopituitarism and tumor growth are rare in patients with non-functioning pituitary microadenomas (NFPmA). However, patients often present with non-specific symptoms. The aim of this brief report is to examine presenting symptomatology in patients with NFPmA compared to patients with non-functioning pituitary macroadenomas (NFPMA). METHODS: We performed a retrospective review of 400 patients (347 NFPmA and 53 NFPMA) who were conservatively managed; no patients had indications for urgent surgical intervention. RESULTS: Average tumor size was 4.5 ± 1.9 and 15.5 ± 5.5 mm for NFPmA and NFPMA, respectively (p < 0.001). At least one pituitary deficiency was present in 7.5% of patients with NFPmA and 25% of patients with NFPMA. Patients with NFPmA were younger (41.6 ± 15.3 vs. 54.4 ± 22.3 years, p < 0.001) and more commonly female (64.6 vs. 49.1%, p = 0.028). There was no significant difference reported for similarly high rates of fatigue (78.4% and 73.6%), headache (70% and 67.9%), and blurry vision (46.7% and 39.6%). There were no significant differences in comorbidities. CONCLUSION: Despite smaller size and lower rate of hypopituitarism, patients with NFPmA presented with a high prevalence of headache, fatigue, and visual symptoms. This was not significantly different from patients with NFPMA who were conservatively managed. We conclude that symptoms of NFPmA cannot fully be attributed to pituitary dysfunction or mass effect.


Asunto(s)
Adenoma , Hipopituitarismo , Neoplasias Hipofisarias , Humanos , Femenino , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/terapia , Neoplasias Hipofisarias/diagnóstico , Adenoma/complicaciones , Adenoma/terapia , Adenoma/diagnóstico , Estudios Retrospectivos , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Hipopituitarismo/diagnóstico , Cefalea , Fatiga/etiología
12.
Front Endocrinol (Lausanne) ; 14: 1100007, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36967769

RESUMEN

Hypopituitarism is defined as a complete or partial deficiency in one or more pituitary hormones. Anterior hypopituitarism includes secondary adrenal insufficiency, central hypothyroidism, hypogonadotropic hypogonadism, growth hormone deficiency and prolactin deficiency. Patients with hypopituitarism suffer from an increased disability and sick days, resulting in lower health status, higher cost of care and an increased mortality. In particular during adulthood, isolated pituitary deficits are not an uncommon finding; their clinical picture is represented by vague symptoms and unclear signs, which can be difficult to properly diagnose. This often becomes a challenge for the physician. Aim of this narrative review is to analyse, for each anterior pituitary deficit, the main related etiologies, the characteristic signs and symptoms, how to properly diagnose them (suggesting an easy and reproducible step-based approach), and eventually the treatment. In adulthood, the vast majority of isolated pituitary deficits are due to pituitary tumours, head trauma, pituitary surgery and brain radiotherapy. Immune-related dysfunctions represent a growing cause of isolated pituitary deficiencies, above all secondary to use of oncological drugs such as immune checkpoint inhibitors. The diagnosis of isolated pituitary deficiencies should be based on baseline hormonal assessments and/or dynamic tests. Establishing a proper diagnosis can be quite challenging: in fact, even if the diagnostic methods are becoming increasingly refined, a considerable proportion of isolated pituitary deficits still remains without a certain cause. While isolated ACTH and TSH deficiencies always require a prompt replacement treatment, gonadal replacement therapy requires a benefit-risk evaluation based on the presence of comorbidities, age and gender of the patient; finally, the need of growth hormone replacement therapies is still a matter of debate. On the other side, prolactin replacement therapy is still not available. In conclusion, our purpose is to offer a broad evaluation from causes to therapies of isolated anterior pituitary deficits in adulthood. This review will also include the evaluation of uncommon symptoms and main etiologies, the elements of suspicion of a genetic cause and protocols for diagnosis, follow-up and treatment.


Asunto(s)
Hipopituitarismo , Enfermedades Hipotalámicas , Hipotiroidismo , Hormonas Adenohipofisarias , Humanos , Prolactina , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Hormonas Hipofisarias , Hipófisis/patología , Enfermedades Hipotalámicas/complicaciones , Hipotiroidismo/etiología
13.
Neurocrit Care ; 39(3): 714-723, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36788181

RESUMEN

Traumatic brain injury (TBI) incurs substantial health and economic burden, as it is the leading reason for death and disability globally. Endocrine abnormalities are no longer considered a rare complication of TBI. The reported prevalence is variable across studies, depending on the time frame of injury, time and type of testing, and variability in hormonal values considered normal across different studies. The present review reports evidence on the endocrine dysfunction that can occur after TBI. Several aspects, including the pathophysiological mechanisms, clinical consequences/challenges (in the acute and chronic phases), screening and diagnostic workup, principles of therapeutic management, and insights on future directions/research agenda, are presented. The management of hypopituitarism following TBI involves hormonal replacement therapy. It is essential for health care providers to be aware of this complication because at times, symptoms may be subtle and may be mistaken to be caused by brain injury itself. There is a need for stronger evidence for establishing recommendations for optimum management so that they can be incorporated as standard of care in TBI management.


Asunto(s)
Lesiones Traumáticas del Encéfalo , Lesiones Encefálicas , Hipopituitarismo , Humanos , Lesiones Traumáticas del Encéfalo/complicaciones , Lesiones Traumáticas del Encéfalo/terapia , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Lesiones Encefálicas/complicaciones , Lesiones Encefálicas/terapia , Lesiones Encefálicas/epidemiología , Síndrome
14.
Eur J Endocrinol ; 188(2)2023 Feb 14.
Artículo en Inglés | MEDLINE | ID: mdl-36655394

RESUMEN

OBJECTIVE: The aim of the study is to assess the distinguishing features of pregnancy-related hypophysitis (PR-Hy) compared to non-pregnancy autoimmune hypophysitis and to evaluate the changing therapeutic approaches and outcomes in PR-Hy over time. DESIGN: Retrospective analysis of all published cases with PR-Hy and 6 own cases. METHODS: A PubMed search was performed and abstracts screened for publications with information on cases with PR-Hy from which full-text review was performed. Clinical features, diagnostic findings, and outcome in relation to treatment modalities in PR-Hy were assessed. RESULTS: One hundred and forty-eight cases with PR-Hy were identified. PR-Hy was significantly delimited from non-PR-Hy by the frequent occurrence of the chiasmal syndrome (50% vs 13%, P < .0001), higher rate of intrasellar origin (94% vs 74%, P = .0005), lower rate of pituitary stalk involvement (39% vs 86%, P < .0001), and low rate of diabetes insipidus (12% vs 54%, P < .0001). The role of surgery in PR-Hy decreased over time while noninvasive treatment modalities increased. The recurrence rate after high-dose glucocorticoid therapy (33%) was high and exceeded that of surgery (2%) and conservative management (2%). In contrast to initial reports on PR-Hy, recent literature regarding outcome of mother's and child's health was positive. The frequency of spontaneous preterm delivery was not increased. Recurrent PR-Hy in a subsequent pregnancy was reported in only two females. CONCLUSION: PR-Hy has distinct features that delineate the disorder from non-PR-Hy. With increasing experience in diagnosis, availability of adequate replacement therapy, and improved treatment modalities, PR-Hy has lost its threat and the outcome is encouraging.


Asunto(s)
Hipofisitis Autoinmune , Diabetes Insípida , Hipofisitis , Hipopituitarismo , Enfermedades de la Hipófisis , Femenino , Niño , Recién Nacido , Humanos , Enfermedades de la Hipófisis/diagnóstico , Enfermedades de la Hipófisis/epidemiología , Enfermedades de la Hipófisis/terapia , Estudios Retrospectivos , Hipófisis , Diabetes Insípida/diagnóstico , Hipofisitis/diagnóstico , Hipofisitis/epidemiología , Hipofisitis/terapia , Hipofisitis Autoinmune/diagnóstico , Hipofisitis Autoinmune/terapia , Hipopituitarismo/diagnóstico , Hipopituitarismo/epidemiología , Hipopituitarismo/terapia , Imagen por Resonancia Magnética
15.
Growth Horm IGF Res ; 66: 101495, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35933894

RESUMEN

OBJECTIVE/DESIGN: Approximately 2.9 million children and adults in the US experience traumatic brain injuries (TBIs) annually, most of which are considered mild. TBI can induce varying consequences on pituitary function, with growth hormone deficiency (GHD) among the more commonly reported conditions. Panels of pediatric and adult endocrinologists, neurologists, physical medicine and rehabilitation specialists, and neuropsychologists convened in February and October 2020 to discuss ongoing challenges and provide strategies for detection and optimal management of patients with mild TBI and GHD. RESULTS: Difficulties include a low rate of seeking medical attention in the population, suboptimal screening tools, cost and complexity of GHD testing, and a lack of consensus regarding when to test or retest for GHD. Additionally, referrals to endocrinologists from other specialists are uncommon. Recommendations from the panels for managing such patients included multidisciplinary guidelines on the diagnosis and management of post-TBI GHD and additional education on long-term metabolic and probable cognitive benefits of GH replacement therapy. CONCLUSION: As patients of all ages with mild TBI may develop GHD and/or other pituitary deficiencies, a multidisciplinary approach to provide education to endocrinologists, neurologists, neurosurgeons, traumatologists, and other providers and guidelines for the early identification and management of persistent mild TBI-related GHD are urgently needed.


Asunto(s)
Conmoción Encefálica , Lesiones Encefálicas , Enanismo Hipofisario , Hormona de Crecimiento Humana , Hipopituitarismo , Adulto , Humanos , Niño , Conmoción Encefálica/complicaciones , Conmoción Encefálica/diagnóstico , Conmoción Encefálica/terapia , Consenso , Lesiones Encefálicas/metabolismo , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Hormona del Crecimiento
16.
Neoreviews ; 23(5): e300-e310, 2022 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-35490188

RESUMEN

Congenital hypopituitarism is the deficiency in 1 or more hormones produced by the anterior pituitary or released by the posterior pituitary and has an estimated incidence of 1 in 4,000 to 10,000. Due to the critical role the pituitary plays in growth, metabolic, and reproductive processes, early diagnosis is essential to prevent devastating and often preventable outcomes. However, in neonates with congenital hypopituitarism, symptoms are often nonspecific and tend to overlap with other disease processes, making diagnosis extremely challenging in the neonatal period. This review highlights the embryology and organogenesis of the pituitary gland, genetic causes of hypopituitarism, clinical presentations in the neonatal period, and methods to diagnose and treat select deficiencies with a focus on anterior pituitary hormones.


Asunto(s)
Hipopituitarismo , Neonatólogos , Humanos , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Recién Nacido
17.
Artículo en Español | LILACS | ID: biblio-1388711

RESUMEN

Resumen La embolia de líquido amniótico es una condición catastrófica propia del embarazo que ocurre típicamente durante el parto o justo posterior a este, cuyo sustrato fisiopatológico no ha sido aclarado por completo. Se ha estimado, según cifras de los Estados Unidos, que su incidencia rondaría 1 por cada 12.953 partos, y en el Reino Unido 1 por cada 50.000 partos; sin embargo, estas cifras pueden ser imprecisas debido a que no existen una referencia ni un consenso respecto a los criterios diagnósticos, además de que el cuadro clínico se puede confundir con otras emergencias obstétricas. Se presenta el caso de una paciente sin antecedentes mórbidos que presenta un cuadro de embolia de líquido amniótico no fatal, caracterizado por un estado fetal no tranquilizador durante la inducción del trabajo de parto, seguido de un paro cardiorrespiratorio durante la cesárea de urgencia y la rápida y catastrófica aparición de signos clínicos de una coagulopatía de consumo grave. Se describen además las complicaciones posoperatorias y su manejo, entre ellas un síndrome de Sheehan y la aparición de convulsiones tónico-clónicas generalizadas con alteración de neuroimágenes.


Abstract Amniotic fluid embolism is a catastrophic pregnancy condition that typically occurs during or inmediately after delivery, and whose pathophysiological background has not been fully clarified. According to US records the incidence of amniotic fluid embolism could been around 1 for every 12,953 births and in the United Kingdom 1 for every 50,000 births, however these numbers may be imprecise because there is no gold standard as well as no consensus regarding the diagnostic criteria, in addition that the clinical presentation can be misdiagnosis with other obstetric emergencies. We present the clinical case of a patient without a morbid history who presents with a non-fatal amniotic fluid embolism, characterized by an non-reassuring fetal status during labor induction, followed by cardiorespiratory arrest during emergency cesarean section and the rapid and catastrophic appearance of clinical signs of a severe consumptive coagulopathy. Postoperative complications and their management are also described, including Sheehans syndrome and the appearance of generalized tonic-clonic seizures with impaired neuroimaging.


Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Embolia de Líquido Amniótico/cirugía , Paro Cardíaco/etiología , Hipopituitarismo/etiología , Cesárea , Reanimación Cardiopulmonar , Coagulación Intravascular Diseminada , Urgencias Médicas , Paro Cardíaco/terapia , Hipopituitarismo/terapia
18.
J Clin Endocrinol Metab ; 107(3): e1020-e1031, 2022 02 17.
Artículo en Inglés | MEDLINE | ID: mdl-34718649

RESUMEN

CONTEXT: Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy. OBJECTIVE: This work aimed to identify patients' characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits, and number of patients starting growth hormone (GH) treatment. METHODS: A retrospective multicenter collection took place of 145 patients aged 0 to 18 years who underwent surgery for CP between 2000 and 2018, and followed up in 17 Italian centers of pediatric endocrinology. RESULTS: Age at diagnosis was 8.4 ± 4.1 years. Duration of symptoms was 10.8 ± 12.5 months and headache was most frequent (54%), followed by impaired growth (48%) and visual disturbances (44%). Most lesions were suprasellar (85%), and histology was adamantinomatous in all cases but two. Surgical approach was transcranial (TC) in 67.5% of cases and transsphenoidal (TS) in 31.%. The TC approach was prevalent in all age groups. Postsurgery complications occurred in 53% of cases, with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented with at least one hormone pituitary deficiency, with thyrotropin deficiency most frequent (98.3%), followed by adrenocorticotropin (96.8%), arginine vasopressin (91.1%), and GH (77.4%). Body mass index (BMI) significantly increased over time. A hypothalamic disturbance was present in 55% of cases. GH therapy was started during follow-up in 112 patients at a mean age of 10.6 years, and 54 developed a recurrence or regrowth of the residual lesion. CONCLUSION: CP is often diagnosed late in Italy, with TC more frequent than the TS surgical approach. Postsurgery complications were not rare, and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.


Asunto(s)
Craneofaringioma/terapia , Hormona de Crecimiento Humana/uso terapéutico , Hipopituitarismo/terapia , Neoplasias Hipofisarias/terapia , Complicaciones Posoperatorias/epidemiología , Edad de Inicio , Niño , Preescolar , Craneofaringioma/complicaciones , Craneofaringioma/diagnóstico , Craneofaringioma/patología , Femenino , Estudios de Seguimiento , Humanos , Hipofisectomía/efectos adversos , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Italia/epidemiología , Masculino , Neoplasia Residual , Hipófisis/patología , Hipófisis/cirugía , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/patología , Complicaciones Posoperatorias/etiología , Recurrencia , Estudios Retrospectivos , Resultado del Tratamiento
19.
Nat Rev Endocrinol ; 17(10): 608-624, 2021 10.
Artículo en Inglés | MEDLINE | ID: mdl-34417587

RESUMEN

Growth hormone (GH) deficiency (GHD) in children is defined as impaired production of GH by the pituitary gland that results in growth failure. This disease might be congenital or acquired, and occurs in isolation or in the setting of multiple pituitary hormone deficiency. Isolated GHD has an estimated prevalence of 1 patient per 4000-10,000 live births and can be due to multiple causes, some of which are yet to be determined. Establishing the correct diagnosis remains key in children with short stature, as initiating treatment with recombinant human GH can help them attain their genetically determined adult height. During the past two decades, our understanding of the benefits of continuing GH therapy throughout the transition period from childhood to adulthood has increased. Improvements in transitional care will help alleviate the consequent physical and psychological problems that can arise from adult GHD, although the consequences of a lack of hormone replacement are less severe in adults than in children. In this Review, we discuss the differential diagnosis in children with GHD, including details of clinical presentation, neuroimaging and genetic testing. Furthermore, we highlight advances and issues in the management of GHD, including details of transitional care.


Asunto(s)
Hormona de Crecimiento Humana/deficiencia , Hipopituitarismo/diagnóstico , Hipopituitarismo/terapia , Estatura/genética , Niño , Diagnóstico Diferencial , Humanos , Hipopituitarismo/metabolismo , Hipófisis/metabolismo , Transición a la Atención de Adultos
20.
Handb Clin Neurol ; 181: 149-159, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34238454

RESUMEN

Hypothalamitis is a rare inflammatory disorder involving the hypothalamus and classified as primary, or isolated, and secondary hypothalamitis. Secondary hypothalamitis although very rare is more common than the primary one and may occur in patients affected by autoimmune diseases such as autoimmune hypophysitis, systemic autoimmune diseases, infective diseases in patients affected by immune-deficit, paraneoplastic encephalitis, or in patients treated with immune checkpoint inhibitors. In accordance with the rarity of this disease, diagnosis and management of hypothalamitis prove to be challenging. The diagnosis requires a high index of clinical suspicion. The main symptoms may be: various degrees of hypopituitarism, neuropsychiatric and behavioral disorders, and disturbances of autonomic and metabolic regulation. Magnetic resonance images play a crucial role in the diagnosis of hypothalamitis and in the exclusion of a neoplastic lesion. Therapeutic management should be oriented according to the disease etiology. In most cases, after ruling out infective hypothalamitis, the mainstay of therapy consists of immunosuppressive treatment. Great attention should be paid to hormonal replacement therapy, if partial or total hypopituitarism is present, in particular in patients affected by diabetes insipidus, central hypoadrenalism and hypothyroidism. According to the complexity of this disease, a multidisciplinary approach is strongly advocated to reach an early diagnosis and an integrated therapy.


Asunto(s)
Hipofisitis Autoinmune , Hipopituitarismo , Enfermedades de la Hipófisis , Atrofia , Humanos , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiología , Hipopituitarismo/terapia , Imagen por Resonancia Magnética , Enfermedades de la Hipófisis/complicaciones , Enfermedades de la Hipófisis/diagnóstico , Enfermedades de la Hipófisis/terapia , Hipófisis
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