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1.
Arch Pediatr ; 31(3): 165-171, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38538470

RESUMO

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.


Assuntos
Hormônio do Crescimento Humano , Hipopituitarismo , Adulto , Criança , Recém-Nascido , Humanos , Hibridização Genômica Comparativa , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Hipopituitarismo/terapia , Hipófise/patologia , Hormônio Adrenocorticotrópico
2.
BMC Endocr Disord ; 24(1): 20, 2024 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-38326790

RESUMO

BACKGROUND: Lymphocytic hypophysitis is a rare autoimmune condition that usually presents during pregnancy and causes inflammation of the pituitary gland. Although the pathophysiology is not well understood, it often presents with headaches, visual disturbances, and symptoms of hypopituitarism. However, not all cases may present with hypopituitarism which can make this rare disease with an incidence of ~ 1 in 9 million much more difficult to diagnose. CASE PRESENTATION: We present a 35-year-old G4P4 woman with progressive vision loss and intermittent frontal headaches during her first trimester through 2 months postpartum. She presented with no symptoms of hypopituitarism and her hormone panel only showed elevated prolactin, possibly due to her breastfeeding. She was treated with a right pterional craniotomy with decompression of both optic nerves, partial resection of the suprasellar mass, and glucocorticoid therapy for headaches and visual disturbances. CONCLUSION: This case is notable for a presentation of lymphocytic hypophysitis without symptoms of hypopituitarism. This is important for outpatient providers to be aware of, especially those that care for pregnant patients so that unfavorable outcomes can be avoided.


Assuntos
Hipofisite Autoimune , Hipopituitarismo , Doenças da Hipófise , Neoplasias Hipofisárias , Humanos , Gravidez , Feminino , Adulto , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/cirurgia , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/complicações , Doenças da Hipófise/diagnóstico , Doenças da Hipófise/complicações , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Hormônios Hipofisários , Cefaleia/etiologia , Cefaleia/complicações , Imageamento por Ressonância Magnética
3.
Probl Endokrinol (Mosk) ; 69(6): 54-62, 2024 Jan 24.
Artigo em Russo | MEDLINE | ID: mdl-38311995

RESUMO

In recent years, there has been a significant increase in the prevalence of autoimmune endocrinopathies, which are known to affect various levels of the endocrine system, including the pituitary gland. Hypophysitis is a general term used to describe any form of sellar and suprasellar inflammation that leads to structural changes in the hypothalamic-pituitary region and manifests itself in varying degrees of hormonal deficiency of the anterior and posterior pituitary glands. To date, there is a primary form of hypophysitis, which occurs as a result of an autoimmune lesion directly to the pituitary gland, and a secondary form of hypophysitis, which occurs as a result of the presence of a systemic autoimmune disease. Regardless of the etiology, patients with hypophysitis show various signs and symptoms caused by an inflammatory process in the pituitary gland, which can lead to the development of hypopituitarism, compression of the sellar and parasellar structures. MRI is currently the best non-invasive diagnostic tool for diagnosing hypopituitarism, however, the diagnosis can be made with certainty only by histological examination of the pituitary tissue, which requires an invasive approach, which greatly reduces the feasibility of this procedure. In this article, we present a patient with MRI showing signs of hypophysitis in the absence of clear clinical symptoms.


Assuntos
Hipofisite , Hipopituitarismo , Doenças da Hipófise , Humanos , Diagnóstico Diferencial , Hipofisite/complicações , Hipofisite/diagnóstico , Doenças da Hipófise/complicações , Doenças da Hipófise/diagnóstico , Doenças da Hipófise/terapia , Hipófise/diagnóstico por imagem , Hipófise/patologia , Hipopituitarismo/diagnóstico
4.
Praxis (Bern 1994) ; 113(1): 23-26, 2024 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-38381107

RESUMO

INTRODUCTION: A 27-year-old man presented due to unilateral leg pain. He had a history of diabetes insipidus and panhypopituitarism. Laboratory analysis revealed hormonal undersupply. MRI showed a large contrast medium-absorbing mass in the pituitary gland extending into the hypothalamus. FDG-PET/CT examination revealed a hypermetabolic soft tissue lesion around the left femoral shaft. After biopsy of the lesion, a diagnosis of multisystemic Langerhans cell histiocytosis was made.


Assuntos
Diabetes Insípido , Diabetes Mellitus , Hipopituitarismo , Masculino , Humanos , Adulto , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Diabetes Insípido/diagnóstico , Diabetes Insípido/etiologia , Dor/etiologia
5.
Zhonghua Yi Xue Za Zhi ; 104(4): 241-246, 2024 Jan 23.
Artigo em Chinês | MEDLINE | ID: mdl-38246768

RESUMO

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.


Assuntos
Hipopituitarismo , Doenças da Hipófise , Feminino , Gravidez , Humanos , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Hipopituitarismo/terapia , Cirrose Hepática/diagnóstico , Cirrose Hepática/terapia , Hipófise
6.
Growth Horm IGF Res ; 74: 101571, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38280246

RESUMO

PURPOSE: Growth hormone (GH) has been recognized to play a regulatory role in female reproduction. It has been reported that infertile GH deficient patients regained fertility after GH replacement. The frequency of GH deficiency is not established in patients diagnosed with unexplained infertility. Here, we aim to present the prevalence of GH deficieny in this patient group. METHODS: We included patients diagnosed with unexplained infertility throughout 18 months. Insulin tolerance test (ITT) and glucagon stimulation tests (GST) were performed and insufficient response to both tests was required for the diagnosis of GH deficiency. RESULTS: Twenty-five patients were included in the study, the mean age was 27.4 ± 4.5 years and the median duration of infertility was 60 months (min:14, max:120). Two patients were GH deficient according to GST and 14 to ITT. Two patients (8%) showed lack of response on both tests and were diagnosed with GH deficiency. CONCLUSION: The rate of GH deficiency among women with unexplained infertility was 8% in this preliminary study. There is need for further studies with larger patient groups to verify the results.


Assuntos
Nanismo Hipofisário , Hormônio do Crescimento Humano , Hipopituitarismo , Infertilidade , Humanos , Feminino , Adulto Jovem , Adulto , Hipopituitarismo/diagnóstico , Hormônio do Crescimento , Insulina
7.
J Obstet Gynaecol Res ; 50(2): 205-211, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37986644

RESUMO

AIM: To identify the symptoms and relevant factors associated with acute adrenal insufficiency of early-onset Sheehan syndrome. METHODS: We retrospectively reviewed the charts of 125 women admitted to our intensive care unit because of postpartum hemorrhage between January 2011 and December 2021. Three women developed acute adrenal insufficiency. We investigated the total blood loss, shock status, consciousness level upon arrival, and intensive care provided to the women. We also analyzed the symptoms and laboratory data that led to the diagnosis of acute adrenal insufficiency. Continuous variables were presented by median (minimum-maximum). RESULTS: The medians and ranges of age, total blood loss, and shock index [heart rate/systolic blood pressure] on admission were 33.1 (17.2-45.3) years, 3351 (595-20 260) g, and 0.94 (0.55-2.94), respectively. Seven women were older than 40 years, 28 experienced >5000 g blood loss, 17 had shock index >1.5, 27 had impaired consciousness upon arrival, and 15 underwent hysterectomy. Women who developed acute adrenal insufficiency were <40 years old and had a bleeding volume of over 5000 g, impaired consciousness upon arrival, and had undergone hysterectomy. They had experienced lactation failure, presented with hyponatremia-related symptoms on postpartum days 8-9, experienced general malaise, headache, and impaired consciousness, and showed severe hyponatremia. CONCLUSIONS: Massive postpartum hemorrhage over 5000 g, impaired consciousness upon arrival, and hysterectomy as a hemostatic measure were relevant factors associated with acute adrenal insufficiency of early-onset Sheehan syndrome. Hyponatremia-related symptoms occurring after lactation failure are indicative of the onset of acute adrenal insufficiency.


Assuntos
Insuficiência Adrenal , Hiponatremia , Hipopituitarismo , Hemorragia Pós-Parto , Gravidez , Feminino , Humanos , Adulto , Hemorragia Pós-Parto/epidemiologia , Hemorragia Pós-Parto/etiologia , Hemorragia Pós-Parto/terapia , Hiponatremia/complicações , Estudos Retrospectivos , Insuficiência Adrenal/complicações , Insuficiência Adrenal/diagnóstico , Insuficiência Adrenal/epidemiologia , Hipopituitarismo/complicações , Hipopituitarismo/diagnóstico , Período Pós-Parto , Doença Aguda
9.
Hormones (Athens) ; 23(1): 69-79, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38147295

RESUMO

The most commonly identified genetic cause of combined pituitary hormone deficiency (CPHD) is PROP1 gene mutations. The aim of the study was to compare selected clinical features of patients with CPHD caused by variants of the PROP1 gene (CPHD-PROP1) and patients with inborn CPHD of other etiology (CPHD-nonPROP1). MATERIAL AND METHODS: The retrospective analysis included childhood medical records of 74 patients (32 female) with CPHD, including 43 patients (23 female) with the mutation in the PROP1 gene. RESULTS: Patients with CPHD-PROP1 compared to the CPHD-nonPROP1 presented with the following: significantly higher median birth weight (0.21 vs. - 0.29 SDS, p = 0.019), lower growth velocity within 3 years preceding growth hormone administration (- 2.7 vs. - 0.8 SDS, p < 0.001), higher mean maximal blood concentration of growth hormone within the stimulation process (1.2 vs. 1.08 ng/mL, p = 0.003), lower TSH (1.8 vs. 2.4 µIU/mL, p < 0.001), significantly lower prolactin concentrations (128 vs. 416.3 µIU/mL, p < 0.001), and less frequent typical signs of hypogonadism at birth in boys (n = 6; 30% vs. n = 12, 54%, p < 0.001). Secondary adrenal insufficiency was less frequent in CPHD-PROP1 (20 vs. 25 cases, p = 0.006) and occurred at a later age (13.4 vs. 10.4 years). MRI of the pituitary gland in CPHD-PROP1 revealed a small pituitary gland (21 cases), pituitary gland enlargement (eight cases), and one pituitary stalk interruption and posterior lobe ectopy, while it was normal in nine cases. CONCLUSION: Patients with the PROP1 mutations present a clinical picture significantly different from that of other forms of congenital hypopituitarism. Certain specific clinical results may lead to the successful identification of children requiring diagnostics for the PROP1 gene mutation.


Assuntos
Proteínas de Homeodomínio , Hipopituitarismo , Criança , Feminino , Humanos , Recém-Nascido , Masculino , Hormônio do Crescimento/genética , Proteínas de Homeodomínio/genética , Hipopituitarismo/diagnóstico , Mutação , Estudos Retrospectivos
10.
Arch Endocrinol Metab ; 68: e220254, 2023 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-37948564

RESUMO

Objective: Congenital hypopituitarism (CH) is a rare disease characterized by one or more hormone deficiencies of the pituitary gland. To date, many genes have been associated with CH. In this study, we identified the allelic variant spectrum of 11 causative genes in Turkish patients with CH. Materials and methods: This study included 47 patients [21 girls (44.6%) and 26 boys (55.4%)] from 45 families. To identify the genetic etiology, we screened 11 candidate genes associated with CH using next-generation sequencing. To confirm and detect the status of the specific familial variant in relatives, Sanger sequencing was also performed. Results: We identified 12 possible pathogenic variants in GHRHR, GH1, GLI2, PROP-1, POU1F1, and LHX4 in 11 patients (23.4%), of which six were novel variants: two in GHRHR, two in POU1F1, one in GLI2, and one in LHX4. In all patients, these variants were most frequently found in GLI2, followed by PROP-1 and GHRHR. Conclusion: Genetic causes were determined in only 23.4% of all patients with CH and 63% of molecularly diagnosed patients (7/11) from consanguineous families. Despite advances in genetics, we were unable to identify the genetic etiology of most patients with CH, suggesting the effect of unknown genes or environmental factors. More genetic studies are necessary to understand the etiology of CH.


Assuntos
Hipopituitarismo , Feminino , Humanos , Masculino , Alelos , Hipopituitarismo/diagnóstico , Hipopituitarismo/genética , Mutação , Proteínas Nucleares/genética , Fator de Transcrição Pit-1/genética , Fatores de Transcrição/genética , Proteína Gli2 com Dedos de Zinco/genética
11.
Endocrinol Diabetes Nutr (Engl Ed) ; 70(9): 584-591, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37977921

RESUMO

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.


Assuntos
Lesões Encefálicas Traumáticas , Lesões Encefálicas , Hipopituitarismo , Humanos , Adulto , Neuroendocrinologia , Qualidade de Vida , Lesões Encefálicas/complicações , Lesões Encefálicas/epidemiologia , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Hipopituitarismo/terapia , Lesões Encefálicas Traumáticas/complicações , Lesões Encefálicas Traumáticas/terapia , Lesões Encefálicas Traumáticas/metabolismo , Hormônios/uso terapêutico
12.
BMC Med Imaging ; 23(1): 188, 2023 11 17.
Artigo em Inglês | MEDLINE | ID: mdl-37978440

RESUMO

BACKGROUND: Although it is generally thought that disturbance of perfusion in the anterior lobe of the pituitary gland leads to complete or partial hypopituitarism, the gadolinium (Gd) enhancement findings on Magnetic Resonance Imaging (MRI) of patients with growth hormone deficiency (GHD) remain unknown. The purpose of this study was to compare Gd enhancement of the pituitary gland on MRI of patients with GHD to that of healthy subjects. METHODS: In this retrospective study, we analyzed the data of 10 patients with clinically diagnosed GHD who underwent Gd-enhanced MRI of their pituitaries (age 8.3[Formula: see text]3.5 year, female 1, males 9), together with data of 5 patients with clinically normal growth hormone (GH) dynamics who also underwent Gd-enhanced pituitary MRI (age 6.2[Formula: see text]3.4 year, female 4, males 1). In each subject, a maximum-diameter region of interest (ROI) was drawn on the anterior pituitary gland of post Gd-enhanced coronal T1-weighted images, and the signal intensity ratio of the anterior pituitary gland to the white matter on the right temporal lobe of the same cross section was assessed. RESULTS: The mean area of the ROI in the anterior pituitary gland and white matter of temporal lobe on the same cross section showed no significant differences between patients with GHD and those with normal GH (pituitary, 17.43 mm2[Formula: see text]8.24 vs. 21.08 mm2[Formula: see text]10.40, p = 1.00; white matter, 74.47mm2[Formula: see text]24.19 and 62.50 mm2[Formula: see text]17.90, p = 0.37), suggesting that the sizes of the pituitary glands were comparable. The ratios of Gd enhancement in the anterior pituitary gland showed significant differences between GHD and normal-GH subjects ([Formula: see text][Formula: see text]0.68[Formula: see text]0.26 vs.[Formula: see text]0.16, p= 0.04). CONCLUSIONS: These results suggested that the contrast effect on Gd-enhanced MRI is attenuated in the pituitary glands of patients with GHD compared to those with normal GH. These new clinical findings regarding Gd-enhanced MRI can assist the diagnosis of pediatric GHD.


Assuntos
Hormônio do Crescimento Humano , Hipopituitarismo , Masculino , Criança , Humanos , Feminino , Meios de Contraste , Gadolínio , Estudos Retrospectivos , Hipófise/diagnóstico por imagem , Hipopituitarismo/diagnóstico , Imageamento por Ressonância Magnética , Hormônio do Crescimento
13.
BMJ Case Rep ; 16(11)2023 Nov 23.
Artigo em Inglês | MEDLINE | ID: mdl-37996133

RESUMO

Sheehan's syndrome (SS) is characterised by pituitary necrosis resulting from postpartum haemorrhage. While SS is uncommon in developed nations, it remains a prevalent cause of hypopituitarism in women, particularly in low/middle-income countries. Clinically, SS is characterised by a deficiency in anterior pituitary hormones; involvement of the posterior pituitary is less common. SS presenting as cardiac tamponade is rare, with only a few reported cases in the literature. In this report, we present the case of a patient with SS who arrived at the emergency department with symptoms of light-headedness, palpitations and dyspnoea. Echocardiography revealed a massive pericardial effusion with cardiac tamponade, and during treatment, the patient experienced ventricular tachycardia and circulatory collapse. The collaboration between various medical specialties, including emergency medicine, cardiology, critical care, endocrinology and radiology, played a crucial role in successful patient management. The multidisciplinary approach allowed for comprehensive care addressing acute cardiac complications and underlying hormonal deficiencies.


Assuntos
Tamponamento Cardíaco , Diabetes Insípido , Diabetes Mellitus , Hipopituitarismo , Derrame Pericárdico , Taquicardia Ventricular , Feminino , Humanos , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/etiologia , Tamponamento Cardíaco/complicações , Hipopituitarismo/complicações , Hipopituitarismo/diagnóstico , Diabetes Insípido/complicações , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/terapia
14.
Medicina (B Aires) ; 83(5): 744-752, 2023.
Artigo em Espanhol | MEDLINE | ID: mdl-37870332

RESUMO

INTRODUCTION: Primary hypophysitis (PH) is a rare disease that represents a challenge among differential diagnosis and management. Our aim was to describe clinical characteristics, diagnostic criteria and different treatment outcomes in patients with PH. Multicentric, retrospective study. Clinical presentation, endocrine function, magnetic resonance imaging findings, visual field defects at diagnosis and treatment outcomes were recorded. METHODS: Twenty-eight patients (23 women), with PH were included. Median age: 37. RESULTS: The most frequent symptoms: headache: 68%, polyuria-polydipsia: 50% and visual disturbances: 48%. At diagnosis, anterior pituitary deficiency was present in 71%, being hypogonadotrophic hypogonadism the most frequent manifestation. The radiological findings: symmetric lesion: 78.5%, homogeneous enhancement: 78.5% and pituitary stalk thickening: 70%. Association with pregnancy or puerperium was found in 4/23 women (17%). Fourteen patients did not receive any treatment ("wait and see" group), 8 underwent surgery for mass reduction or resection and 6 were treated with immunosuppression therapy. Among 15 patients with histopathological diagnosis, 9 were lymphocytic hypophysitis, 5 IgG4 related hypophysitis and 1 xanthomatous hypophysitis. Thirteen were diagnosed by established clinical criteria. Mass reduction was observed in 43% of "wait and see group" patients, 62.5% of operated patients and 50% with immunosuppression therapy. Compressive symptoms showed improvement in the 3 groups, with modest effect on anterior pituitary function, diabetes insipidus did not resolve in any patients. DISCUSSION: In patients without severe compressive symptoms, we adopted a "wait and see" approach. In patients with uncertain diagnosis of PH or severe compressive symptoms, transsphenoidal surgery was the best option.


Introducción: La hipofisitis es una enfermedad infrecuente que plantea un desafío en el diagnóstico y tratamiento. El objetivo de este estudio multicéntrico y retrospectivo fue describir: a) características de pacientes con hipofisitis primaria (HP), b) métodos diagnósticos, y c) tratamientos realizados. Además, evaluar: a) presentación clínica, b) bioquímica, c) radiológica, d) oftalmológica al diagnóstico y evolución según el tratamiento recibido. Métodos: Estudio retrospectivo donde se estudiaron 28 pacientes (23 mujeres/5 varones) con HP, edad promedio de 38±11.1 años. Resultados. Los síntomas fueron: cefalea: 68%, poliuria- polidipsia: 50% y alteraciones visuales: 48%. El examen de laboratorio inicial informó disfunción adenohipofisaria en 71% de los pacientes, siendo el eje gonadal el más afectado. Los hallazgos radiológicos más frecuentes fueron: lesión simétrica:78.5%, aumento homogéneo post contraste: 78.5% y engrosamiento de tallo:70%. En 4/23 mujeres (17%) se relacionó con embarazos o puerperio La conducta inicial fue expectante (CE) en 14 pacientes, cirugía de resección/descompresión en 8 y tratamiento con inmunosupresores en 6. Quince pacientes tuvieron confirmación histológica, 9 resultaron hipofisitis linfocitaria, 5 hipofisitis por IgG4 y una xantomatosa. Trece fueron diagnosticados por criterios clínicos establecidos. El tamaño de la lesión disminuyó en 43% de pacientes con CE, 62.5% con cirugía y 50% con inmunosupresores. Los síntomas compresivos mejoraron en los 3 grupos, con escaso efecto sobre la función adenohipofisaria, sin resolución de la diabetes insípida. Discusión: En pacientes sin síntomas compresivos adoptamos CE. En aquellos pacientes sin certeza diagnóstica o con síntomas compresivos graves, se optó por cirugía transesfenoidal.


Assuntos
Hipofisite Autoimune , Hipofisite , Hipopituitarismo , Gravidez , Humanos , Feminino , Adulto , Estudos Retrospectivos , Hipofisite/diagnóstico , Hipofisite/terapia , Hipofisite/patologia , Hipófise/patologia , Hipopituitarismo/diagnóstico , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/terapia , Hipofisite Autoimune/patologia , Imageamento por Ressonância Magnética
15.
Front Endocrinol (Lausanne) ; 14: 1270845, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37881494

RESUMO

Objective: Isolated childhood growth hormone deficiency (GHD) can persist into adulthood, and re-testing at the transition period is needed to determine whether continued growth hormone therapy is indicated. Here, our objective was to identify predictors of permanent GHD. Design: Retrospective single-centre study of patients with childhood-onset GHD who were re-tested after adult height attainment. Methods: Auxological, clinical, laboratory, and MRI data throughout follow-up were collected. Results: We included 101 patients. At GH treatment initiation, age was 8.1 ± 0.4 years, height -2.25 ± 0.8, and BMI -0.27 ± 0.1 SDS. The 29 (28.7%) patients with persistent GHD had lower height SDS (-2.57 ± 0.1 vs. -2.11 ± 0.1, p<0.001) and mean GH peaks (8.4 ± 1.0 vs.13.2 ± 0.5 mIU/L, p<0.001) at GHD diagnosis; at adult height, they had lower IGF1 (232 ± 19.9 vs. 331 ± 9.1 ng/mL, p<0.001) and higher BMI SDS (-0.15 ± 0.27 vs. -0.73 ± 0.13, p<0.005). By multivariate analysis, the best predictive model included height and BMI SDS, both GH peaks, and MRI findings at diagnosis. Patients with height at diagnosis <-3 SDS had a 7.7 (95% IC 1.4-43.1, p=0.02) fold higher risk of persistent GHD after adjustment on BMI SDS. An abnormal pituitary region by MRI was the strongest single predictor (7.2 times, 95% CI 2.7-19.8) and after multivariate analysis adjustment for GH peaks and height SDS at diagnosis, the risk increased to 10.6 (1.8 - 61.3) times. Conclusions: Height <-3 SDS at GHD diagnosis and pituitary MRI abnormalities should lead to a high index of suspicion for persistent GHD.


Assuntos
Nanismo Hipofisário , Hormônio do Crescimento Humano , Hipopituitarismo , Adulto , Criança , Humanos , Nanismo Hipofisário/diagnóstico , Nanismo Hipofisário/tratamento farmacológico , Hormônio do Crescimento Humano/deficiência , Hipopituitarismo/diagnóstico , Hipopituitarismo/tratamento farmacológico , Estudos Retrospectivos
17.
Endocrinol Diabetes Metab ; 6(6): e445, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37697708

RESUMO

BACKGROUND: Nonfunctioning pituitary adenomas (NFPAs) constitute one of the most common tumours in the sellar region and are often discovered only when associated with compressive symptoms. With the frequent use of brain imaging, there has been an increase in the prevalence of incidentally discovered NFPAs. AIM: We aim to determine the prevalence of incidental diagnosis with NPAs observed over a decade and compare the analytical, clinical and treatment differences between those who were diagnosed either incidentally or symptomatically. We also intend to evaluate the pathology differences between both groups. METHODS: We retrospectively analysed patients aged ≥18 years with an apparent NFPA, defined as a pituitary lesion compatible with pituitary adenoma which is not associated with the clinical or biochemical evidence of a hormone-secreting tumour. Inclusion criteria included normal prolactin level for lesions <9 mm or a prolactin level <100 ng/mL for lesions ≥10 mm in maximal tumour diameter. RESULTS: We included 119 patients [53.8% males; mean age: 56.8 years (SD = 16.7)]. Diagnosis was incidental in 47.1% of patients, and many patients had unappreciated signs and symptoms of pituitary disease. In the symptomatic and incidental groups, 66.7% and 41.1% of patients had hypopituitarism, respectively (p = .005). Only 20.4% of patients incidentally diagnosed had microadenoma (p = .060). Hypopituitarism was present in 18.8% of those patients with microadenomas. Most tumours were macroadenomas (87.4%). Half of those patients diagnosed incidentally were submitted to surgery, compared with 75.8% of those who were diagnosed symptomatically (p = .004). CONCLUSIONS: Nonfunctioning pituitary adenomas are commonly diagnosed incidentally, with many manifesting symptoms on examination. NFPAs incidentally diagnosed are more commonly macroadenomas and less frequently associated with hypopituitarism than symptomatic. Accordingly, if there was a greater level of knowledge and more suspicion about these pathologies, it might be possible to discover them earlier.


Assuntos
Adenoma , Hipopituitarismo , Neoplasias Hipofisárias , Masculino , Humanos , Adolescente , Adulto , Pessoa de Meia-Idade , Feminino , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/complicações , Prolactina/uso terapêutico , Estudos Retrospectivos , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/patologia , Hipopituitarismo/diagnóstico , Hipopituitarismo/epidemiologia , Hipopituitarismo/etiologia
18.
Best Pract Res Clin Endocrinol Metab ; 37(6): 101820, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37704550

RESUMO

Growth hormone is among the most common hormones to be deficient in pituitary insult. It can occur either in isolation or combined with other hormone deficiencies. Growth hormone deficiency in adults (AGHD) can be due to causes acquired in adulthood or have a childhood-onset etiology, but the former is about three times more common. Usual causes of AGHD include mass effects due to a pituitary tumour, and/or its treatment (surgery, medical therapy, or radiotherapy), or radiotherapy to the head and neck region for non-pituitary lesions. The unusual or lesser-known causes of AGHD, are usually due to non-tumoral etiology and range from vascular and infective to inflammatory and miscellaneous causes. These not only expand the spectrum of AGHD but may also contribute to increased morbidity, adverse metabolic consequences, and mortality due to the primary condition, if unrecognised. The review features these lesser-known and rare causes of AGHD and highlights their clinical and diagnostic implications.


Assuntos
Nanismo Hipofisário , Hormônio do Crescimento Humano , Hipopituitarismo , Adulto , Humanos , Criança , Nanismo Hipofisário/complicações , Nanismo Hipofisário/tratamento farmacológico , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Hormônio do Crescimento , Hipófise
19.
J Med Case Rep ; 17(1): 388, 2023 Sep 12.
Artigo em Inglês | MEDLINE | ID: mdl-37697403

RESUMO

INTRODUCTION: Rathke's cleft cysts are thought to have a benign clinical outcome apart from associated hypopituitarism and visual defects. Synchronous central nervous system lesions, including pituitary adenoma and intracerebral aneurysms, are rarely reported. Diagnosis of Rathke's cleft cyst after presenting with a subarachnoid hemorrhage due to a ruptured arterial aneurysm is reported only once before. CASE PRESENTATION: A 33-year-old Sri Lankan female presented with a subarachnoid hemorrhage due to a ruptured anterior communication artery aneurysm. She underwent pterional craniotomy and aneurysm clipping. She was found to have partial cranial diabetes insipidus and hypogonadotropic hypogonadism. She had a cystic lesion occupying enlarged sella turcica with characteristics of a Rathke's cleft cyst. Subsequently, she underwent trans-sphenoidal excision of the sellar lesion. Histology confirmed the diagnosis of Rathke's cleft cyst. CONCLUSIONS: Rare co-occurrence of a Rathke's cleft cyst and an anterior communicating artery aneurysm would have been missed if subtle manifestations atypical for subarachnoid hemorrhage were not further pursued. This could have led to progressive visual deterioration and hypopituitarism.


Assuntos
Aneurisma Roto , Cistos do Sistema Nervoso Central , Hipopituitarismo , Aneurisma Intracraniano , Hemorragia Subaracnóidea , Adulto , Feminino , Humanos , Povo Asiático , Craniotomia , Hipopituitarismo/diagnóstico , Hipopituitarismo/etiologia , Aneurisma Intracraniano/complicações , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Hemorragia Subaracnóidea/diagnóstico por imagem , Hemorragia Subaracnóidea/etiologia , Hemorragia Subaracnóidea/cirurgia , Cistos do Sistema Nervoso Central/complicações , Cistos do Sistema Nervoso Central/diagnóstico
20.
Medicina (B Aires) ; 83(4): 514-521, 2023.
Artigo em Espanhol | MEDLINE | ID: mdl-37582125

RESUMO

INTRODUCTION: Pituitary incidentalomas (PIs) are lesions found incidentally in the pituitary on imaging performed for reasons unrelated to pituitary disease. METHODS: A cross-sectional, retrospective and descriptive study was carried out with the aim of analyzing the clinical and evolutionary characteristics of a population of patients with PIs in the city of Córdoba. RESULTS: A total of 67 patients were included, 67% female, with a median age at diagnosis of 44 years. Among the reasons that led to requesting the first image, chronic or recurrent headache was the most prevalent (34%). The median tumor size was 12 mm. Fifty-eight percent were macroincidentalomas. Men had significantly larger lesions (p = 0.04). At diagnosis, including both sexes, 30% showed extrasellar extension and 45% invasion of the cavernous sinuses. Neuro-ophthalmological compromise was detected in 21%. A positive correlation was found between age at diagnosis and tumor size (r= +0.31, p = 0.001). Ninety-one percent were non-functioning tumors and at presentation, 21% of patients had one or more hormonal deficiencies. Of the total, 26% required surgery. Most of those who continued without treatment showed no change in tumor size at the end of follow-up (median 42 months). CONCLUSION: We highlight the high frequency of macroincidentalomas in our series, with visual field defects and hypopituitarism being frequent at diagnosis. Although most non-operated PIs remained stable, there was a high frequency of clinically significant lesions.


Introducción: Los incidentalomas hipofisarios (IH) son lesiones halladas fortuitamente en la hipófisis mediante imágenes realizadas por motivos no relacionados con enfermedad hipofisaria. Métodos: Se realizó un estudio transversal, retrospectivo y descriptivo con el objetivo de analizar las características clínicas y evolutivas de una población de pacientes portadores de IH en la ciudad de Córdoba. Resultados: Se incluyeron 67 pacientes, 67% del sexo femenino, con una mediana de edad al diagnóstico de 44 años. Entre los motivos que llevaron a solicitar la primera imagen, la cefalea crónica o recurrente fue el más prevalente (34%). La mediana del tamaño tumoral fue de 12 mm. El 58% fueron macroincidentalomas. Los hombres tuvieron lesiones significativamente más grandes (p = 0.04). Al diagnóstico, considerando ambos sexos, el 30% evidenció extensión extraselar y el 45% invasión a senos cavernosos. Se detectó compromiso neurooftalmológico en el 21%. Se halló correlación positiva entre la edad al momento del diagnóstico y el tamaño tumoral (r = +0.31, p = 0.001). El 91% fueron tumores no funcionantes y en su presentación, el 21% de los pacientes presentaron una o más deficiencias hormonales. El 26% del total requirió cirugía. La mayoría de aquellos que continuaron sin tratamiento no evidenciaron cambios en el tamaño tumoral al final del seguimiento (mediana 42 meses). Conclusión: Destacamos la elevada frecuencia de macroincidentalomas en nuestra serie, siendo los defectos del campo visual y el hipopituitarismo frecuentes al diagnóstico. Si bien la mayoría de los IH no operados permanecieron estables, existió una alta frecuencia de lesiones clínicamente significativas.


Assuntos
Adenoma , Hipopituitarismo , Neoplasias Hipofisárias , Masculino , Humanos , Feminino , Adulto , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/epidemiologia , Estudos Retrospectivos , Estudos Transversais , Adenoma/diagnóstico , Hipopituitarismo/diagnóstico
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