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1.
BMJ Open ; 14(4): e078595, 2024 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-38569705

RESUMO

INTRODUCTION: When children with head and neck cancer receive radiation therapy as part of their treatment, a considerable frequency of hypopituitarism has been recognised. However, in adults, it has been little studied and it is possible that patients may be inadvertently affected. The objective is to estimate the incidence of anterior pituitary dysfunction in adults undergoing radiotherapy for head and neck cancer. METHODS AND ANALYSIS: A total of five databases will be used to perform the document search: PubMed, Scopus, Web of Science (Core Collection), Ovid-MEDLINE and Embase. Cohort studies will be included without restriction by language or date. The main outcome will be the incidence of adenohypophyseal dysfunction for each axis: prolactin, growth hormone, thyroid-stimulating hormone, adrenocorticotropic hormone, luteinising hormone and follicle-stimulating hormone. Incidence meta-analysis will be performed using the Freeman-Tukey double arcsine method. In addition, a random-effects model will be used along with a 95% CI. Subgroup analyses will be performed according to tumour location, radiation dose and endocrine assessment time. Meta-regression will be applied according to patient's age and time elapsed until diagnosis. ETHICS AND DISCLOSURE: Since this will be a systematic review of published data, no ethics committee approval is required. The results will be presented at conferences and finally published in a peer-reviewed journal. PROSPERO REGISTRATION NUMBER: CRD42021235163.


Assuntos
Neoplasias de Cabeça e Pescoço , Hipopituitarismo , Neoplasias Pancreáticas , Adulto , Criança , Humanos , Incidência , Revisões Sistemáticas como Assunto , Metanálise como Assunto , Neoplasias de Cabeça e Pescoço/radioterapia , Hipopituitarismo/epidemiologia , Hipopituitarismo/etiologia
2.
Turk Neurosurg ; 34(2): 331-342, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38497187

RESUMO

AIM: To share the surgical outcomes of 31 patients who underwent endoscopic endonasal transsphenoidal surgery (EETS) at a single center. MATERIAL AND METHODS: This retrospective analysis of 31 craniopharyngioma cases (2013-2022) with a minimum 6-month follow-up included demographic data, preoperative findings, postoperative resection volumes, recurrence rates, pathological diagnoses, and complications. RESULTS: Herein, 34 EETS surgeries were performed on 31 patients (12 males, 19 females). The presenting symptoms included visual loss (58%), hypopituitarism (54.8%), and diabetes insipidus (25.8%). Gross total resection was achieved in 87% of the patients, with 64.5% total and 22.5% near-total resection. Total resection prevented recurrences, contrasting with 75% recurrence in the subtotal resection patients (p=0.000). The primary patients showed 73.1% total resection, while only 20% of the recurrent patients achieved it (p=0.049). When comparing the first 16 cases with the last 15 cases in terms of surgical experience, the rates of resection (p=0.040) and recurrence-free survival (p=0.020) in the last 15 cases were statistically significant. Patients with preoperative visual loss demonstrated 94.4% improvement or stability postoperatively. Postoperative complications included hypopituitarism (71.4%), permanent diabetes insipidus (60.8%), worsening vision (6.5%), cerebrospinal fluid leakage (9.7%), meningitis (6.5%), and a 3.2% perioperative mortality rate. CONCLUSION: This study underscores the role of surgical resection in craniopharyngiomas, emphasizing the impact of surgical experience on recurrence-free survival. Primary surgery, with minimal complications and maximal resection, is crucial in managing recurrence challenges. Endoscopic endonasal transsphenoidal surgery, particularly in experienced centers, offers advantages such as panoramic vision and access to the third ventricle base, facilitating total and near-total resection and extending recurrence-free survival.


Assuntos
Craniofaringioma , Diabetes Insípido , Hipopituitarismo , Neoplasias Hipofisárias , Masculino , Feminino , Humanos , Craniofaringioma/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Neoplasias Hipofisárias/cirurgia , Neoplasias Hipofisárias/patologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Hipopituitarismo/etiologia , Diabetes Insípido/etiologia , Diabetes Insípido/complicações , Transtornos da Visão/etiologia
4.
Clin Endocrinol (Oxf) ; 100(5): 441-446, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38463009

RESUMO

OBJECTIVES: Various biases pertaining to stature account for a male sex predominance in growth hormone deficiency (GHD) cases diagnosed by endocrinology clinics. This manuscript will assess the sex distribution when biases are minimised. METHODS: Retrospective chart review was conducted on patients diagnosed with GHD between 3 and 16 years of age. The sex distribution of cases was ascertained according to: (1) peak GH (pGH) by groups; based on growth hormone provocative testing, (2) pituitary gland imaging results, and (3) isolated GHD (IGHD) versus multiple pituitary hormone deficiencies (MPHD). The relative frequency of each sex was compared according to these subgroups with significance evaluated at α = .05 level. RESULTS: Of the 5880 clinic referrals for short stature, there were 3709 boys (63%) and 2171 girls (37%). Of these, 20% of boys (n = 745) and 15.3% of girls (n = 332) underwent provocative testing for GHD. Of those tested, 39.2% of boys (n = 292) and 32.2% of girls (n = 107) were diagnosed with GHD, all p < .001. There was a male predominance in GHD cases based on pGH or GHD severity. Though not significant, girls were more likely than boys to have MPHD (p = .056), even across pGH groups (p = .06). Both boys and girls had a similar distribution of imaging abnormalities. CONCLUSION: Stratifying by sex, we found similar percentages of pituitary imaging abnormalities (including tumours) and the number of pituitary hormone deficiencies in boys and girls as the cause of GHD. For these classifications, we did not find the historically reported male sex predominance.


Assuntos
Nanismo Hipofisário , Hormônio do Crescimento Humano , Hipopituitarismo , Feminino , Humanos , Criança , Masculino , Estudos Retrospectivos , Hipopituitarismo/epidemiologia , Nanismo Hipofisário/epidemiologia , Hormônio do Crescimento , Distribuição por Sexo
5.
Acta Neurochir (Wien) ; 166(1): 120, 2024 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-38430312

RESUMO

BACKGROUND: The management of craniopharyngiomas is challenging due to their high rate of recurrence following resection. Excision of recurrent tumors poses further surgical challenges due to loss of arachnoidal planes and adherence to anatomical structures. The endoscopic endonasal approach (EEA) offers a favorable alternative to transcranial approaches for primary craniopharyngiomas. However, the safety and efficacy of EEA for recurrent tumors, specifically after a prior transcranial approach, needs further investigation. METHODS: We performed a systematic review using PubMed to develop a database of cases of recurrent craniopharyngiomas previously treated with a transcranial approach. RESULTS: Fifteen articles were included in this review with a total of 75 cases. There were 50 males and 25 females with a mean age of 38 years (range 2-80). One prior transcranial surgery was done in 80.0% of cases, while 8.0% had two and 12.0% had more than two prior surgeries. Radiotherapy after transcranial resection was given in 18 cases (24.0%). Following EEA, vision improved in 60.0% of cases, and vision worsened in 8.6% of the cases. Of cases, 64.4% had pre-existing anterior hypopituitarism, and 43.8% had diabetes insipidus prior to EEA. New anterior hypopituitarism and diabetes insipidus developed in 24.6% and 21.9% of cases, respectively following EEA. Gross total resection (GTR) was achieved in 64.0%, subtotal resection in 32.0%, and partial resection in 4.0% revision EEA cases. GTR rate was higher in cases with no prior radiotherapy compared to cases with prior radiotherapy (72.0% vs 39.0%, p = 0.0372). The recurrence rate was 17.5% overall but was significantly lower at 10.0% following GTR (p = 0.0019). The average follow-up length was 41.2 months (range, 1-182 months). CONCLUSION: The EEA can be utilized for resection of recurrent or residual craniopharyngiomas previously managed by a transcranial approach.


Assuntos
Craniofaringioma , Diabetes Insípido , Hipopituitarismo , Neoplasias Hipofisárias , Humanos , Craniofaringioma/diagnóstico por imagem , Craniofaringioma/cirurgia , Craniofaringioma/patologia , Endoscopia , Neoplasias Hipofisárias/cirurgia , Neoplasias Hipofisárias/patologia
6.
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
7.
Front Endocrinol (Lausanne) ; 15: 1338781, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38464967

RESUMO

Background: Pituitary stalk interruption syndrome (PSIS) is a complex clinical syndrome characterized by varied pituitary hormone deficiencies, leading to severe manifestations across multiple systems. These include lifelong infertility, short stature, mental retardation, and potentially life-threatening pituitary crises if not promptly diagnosed and treated. Despite extensive research, the precise pathogenesis of PSIS remains unclear. Currently, there are two proposed theories regarding the pathogenic mechanisms: the genetic defect theory and the perinatal injury theory. Methods: We systematically searched English databases (PubMed, Web of Science, Embase) and Chinese databases (CNKI, WanFang Med Online, Sinomed) up to February 24, 2023, to summarize studies on gene sequencing in PSIS patients. Enrichment analyses of reported mutated genes were subsequently performed using the Metascape platform. Results: Our study included 37 articles. KEGG enrichment analysis revealed mutated genes were enriched in the Notch signaling pathway, Wnt signaling pathway, and Hedgehog signaling pathway. GO enrichment analysis demonstrated mutated genes were enriched in biological processes such as embryonic development, brain development, axon development and guidance, and development of other organs. Conclusion: Based on our summary and analyses, we propose a new hypothesis: disruptions in normal embryonic development, partially stemming from the genetic background and/or specific gene mutations in individuals, may increase the likelihood of abnormal fetal deliveries, where different degrees of traction during delivery may lead to different levels of pituitary stalk interruption and posterior lobe ectopia. The clinical diversity observed in PSIS patients may result from a combination of genetic background, specific mutations, and variable degrees of traction during delivery.


Assuntos
Hipopituitarismo , Doenças da Hipófise , Humanos , Proteínas Hedgehog , Doenças da Hipófise/patologia , Hipófise/patologia , Hipopituitarismo/genética , Hipopituitarismo/patologia , Mutação , Síndrome
9.
Front Endocrinol (Lausanne) ; 15: 1348972, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38449845

RESUMO

Introduction: The mortality ratio in patients with acromegaly has improved over the last few decades. We aimed to determine the mortality rate and correlated factors in patients with acromegaly before and after the introduction of national protocols for treatment. In addition, we determined whether there are sex-related differences in mortality of patients with acromegaly. Methods: This observational retrospective study included 399 consecutive patients with acromegaly between January 2001-December 2022. Paraclinical data included random growth hormone (GH) and insulin-like growth factor-I (IGF1) levels, maximal pituitary tumor diameter at diagnosis, first visit, and last evaluation. Standardized mortality ratio (SMR) was calculated by dividing the observed and expected mortality rates. Cox regression analysis revealed the independent factors associated with mortality. Results: At the last visit, 31.07% (124) of patients were cured, 22.05% (88) had controlled acromegaly with medication, and 45.31% (181) had not controlled acromegaly. During follow-up (13.03 ± 5.65 years, 5216.62 person-years), 89 patients died (0.017%), resulting in an SMR of 1.18 [95% CI 0.95-1.45]. The independent factors associated with mortality were the last IGF1 level/last random GH level, absence of surgery, gonadotropin deficiency, and age. Patients with normal IGF1 after treatment showed an SMR of 0.71, whereas patients with IGF1 ratio > 1 showed SMR=1.51. Patients diagnosed between 1975-2007 and 2008-2022 had SMR = 1.25 [95% CI 0.97-1.58] and SMR = 1.09 [95% CI 0.68-1.65], respectively. In females with acromegaly, SMR was 1.63 [95% CI 1.24-2.11]; 1.76 [95% CI 1.30-2.34] in women diagnosed before 2008 and 1.33 [95% CI 0.69-2.33] in those diagnosed after 2008. Males with acromegaly had a mortality ratio similar to males from the general population (SMR = 0.99, [95% CI 0.66-1.41]). Conclusion: Patients diagnosed with acromegaly in the last 15 years had lower mortality rates than those diagnosed before 2008, due to the availability of new medications, primarily somatostatin receptor analogs and to a higher proportion of patients undergoing surgery. Females still have a high mortality ratio owing to older age at diagnosis and higher risk of metabolic complications. Therefore, efforts should be made for early diagnosis of acromegaly in women.


Assuntos
Acromegalia , Hormônio do Crescimento Humano , Hipopituitarismo , Masculino , Humanos , Feminino , Estudos Retrospectivos , Hormônio do Crescimento
10.
Growth Horm IGF Res ; 74: 101573, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38368660

RESUMO

OBJECTIVE: Children with growth hormone deficiency (GHD) face multiple challenges that can negatively impact the transition from pediatric to adult endocrinology care. For children with GHD resulting from brain cancer or its treatment, the involvement of oncology care providers and possible disease-related comorbidities add further complexity to this transition. DESIGN: An advisory board of pediatric and adult endocrinologists was convened to help better understand the unique challenges faced by childhood cancer survivors with GHD, and discuss recommendations to optimize continuity of care as these patients proceed to adulthood. Topics included the benefits and risks of growth hormone (GH) therapy in cancer survivors, the importance of initiating GH replacement therapy early in the patient's journey and continuing into adulthood, and the obstacles that can limit an effective transition to adult care for these patients. RESULTS/CONCLUSIONS: Some identified obstacles included the need to prioritize cancer treatment over treatment for GHD, a lack of patient and oncologist knowledge about the full range of benefits provided by long-term GH administration, concerns about tumor recurrence risk in cancer survivors receiving GH treatment, and suboptimal communication and coordination (e.g., referrals) between care providers, all of which could potentially result in treatment gaps or even complete loss of follow-up during the care transition. Advisors provided recommendations for increasing education for patients and care providers and improving coordination between treatment team members, both of which are intended to help improve continuity of care to maximize the health benefits of GH administration during the critical period when childhood cancer survivors transition into adulthood.


Assuntos
Neoplasias Encefálicas , Sobreviventes de Câncer , Nanismo Hipofisário , Hormônio do Crescimento Humano , Hipopituitarismo , Adulto , Criança , Humanos , Encéfalo , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/terapia , Hormônio do Crescimento/uso terapêutico , Hormônio do Crescimento Humano/deficiência , Transferência de Pacientes
11.
Int J Mol Sci ; 25(4)2024 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-38396741

RESUMO

Oxytocin (Oxt) regulates thermogenesis, and altered thermoregulation results in Prader-Willi syndrome (PWS), Schaaf-Yang syndrome (SYS), and Autism spectrum disorder (ASD). PWS is a genetic disorder caused by the deletion of the paternal allele of 15q11-q13, the maternal uniparental disomy of chromosome 15, or defects in the imprinting center of chromosome 15. PWS is characterized by hyperphagia, obesity, low skeletal muscle tone, and autism spectrum disorder (ASD). Oxt also increases muscle tonicity and decreases proteolysis while PWS infants are hypotonic and require assisted feeding in early infancy. This evidence inspired us to merge the results of almost 20 years of studies and formulate a new hypothesis according to which the disruption of Oxt's mechanism of thermoregulation manifests in PWS, SYS, and ASD through thermosensory abnormalities and skeletal muscle tone. This review will integrate the current literature with new updates on PWS, SYS, and ASD and the recent discoveries on Oxt's regulation of thermogenesis to advance the knowledge on these diseases.


Assuntos
Transtorno do Espectro Autista , Regulação da Temperatura Corporal , Transtornos Cromossômicos , Deficiências do Desenvolvimento , Facies , Hipopituitarismo , Ocitocina , Síndrome de Prader-Willi , Humanos , Lactente , Transtorno do Espectro Autista/genética , Transtorno do Espectro Autista/metabolismo , Deficiências do Desenvolvimento/genética , Deficiências do Desenvolvimento/metabolismo , Hipotonia Muscular , Síndrome de Prader-Willi/genética , Síndrome de Prader-Willi/metabolismo
12.
Acta Neurochir (Wien) ; 166(1): 84, 2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38355813

RESUMO

PURPOSE: Pituitary adenomas (PAs) usually have a soft consistency, facilitating gross total resection. However, 5-13% of PAs with fibrous consistency are challenging to remove entirely and are accompanied by greater morbimortality. This study aims to identify the clinical and radiological characteristics that correlate with PA fibrous consistency preoperatively. A simple scoring system has been proposed to predict incidence of fibrous PAs. MATERIALS AND METHODS: Consecutive interventions (226) were analyzed, all performed through an endoscopic endonasal transsphenoidal approach. Univariable and multivariable logistic regression analysis was performed. Hosmer-Lemeshow test and receiver operating characteristic (ROC) curves were assessed to evaluate the model. A point scoring system (PiTCon) was derived based on the multivariable regression model. Our study aimed to identify the clinical and radiological characteristics that correlate with fibrous tumor consistency preoperatively. RESULTS: The best diagnostic accuracy for predicting PA consistency consisted of five predictive factors: age, compressive symptoms, panhypopituitarism, craniocaudal extension of the PA in mm, and prior surgery. The multivariable model achieved good discrimination with an area under the curve (AUC) of the ROC curve being 0.82 and the 95% CI 0.76 to 0.88. Internal validation yielded an optimism-adjusted C-statistic of 0.80 (95% CI 0.74 to 0.86). A point scoring system (PiTCon score) was designed using the best predictive model. CONCLUSIONS: PA consistency can be estimated preoperatively regarding clinical and radiological characteristics. We propose a point-based scoring system (PiTCon score) that can better guide neurosurgeons in clinical decision-making and surgical risk assessment and help establish and describe patient prognosis.


Assuntos
Adenoma , Hipopituitarismo , Neoplasias Hipofisárias , Humanos , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/cirurgia , Endoscopia , Adenoma/diagnóstico por imagem , Adenoma/cirurgia , Curva ROC , Estudos Retrospectivos
13.
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
14.
Zhonghua Yi Xue Za Zhi ; 104(6): 450-452, 2024 Feb 06.
Artigo em Chinês | MEDLINE | ID: mdl-38326058

RESUMO

To analyze the clinical features of patients with anterior hypopituitarism (HP) complicated with cirrhosis, and to explore the effects of growth hormone supplementation on liver and lung function. A total of 11 patients with HP complicated with cirrhosis admitted to Peking Union Medical College Hospital from January 2016 to December 2022 were included in the study, including 8 males and 3 females, aged [M(Q1, Q3)]31 (20, 37) years. There were 6 patients with pituitary stalk interruption syndrome, 4 patients after craniopharyngioma resection, and 1 patient after germinal cell tumor chemoradiotherapy. Cirrhosis appeared at [M(Q1, Q3)]7 (1, 16) years after the diagnosis of HP. There were 7 cases complicated with hepatopulmonary syndrome (HPS). The liver and lung function of 5 patients were improved significantly after the addition of growth hormone, and the arterial partial pressure of oxygen increased from (47±11) mmHg(1 mmHg=0.133 kPa) to (84±12) mmHg. Timely supplementation of growth hormone can improve the symptoms of fatty liver, cirrhosis and HPS, and postpone or even avoid the transplantation of liver and other organs.


Assuntos
Síndrome Hepatopulmonar , Hormônio do Crescimento Humano , Hipopituitarismo , Neoplasias Hipofisárias , Humanos , Masculino , Feminino , Idoso , Hormônio do Crescimento , Cirrose Hepática , Hipopituitarismo/complicações , Hipopituitarismo/patologia , Síndrome Hepatopulmonar/complicações , Síndrome Hepatopulmonar/diagnóstico , Pulmão/patologia , Suplementos Nutricionais
15.
Indian Pediatr ; 61(2): 154-157, 2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38321728

RESUMO

OBJECTIVE: To analyse the clinical and radiological characteristics of pituitary stalk interruption syndrome (PSIS). METHODS: A retrospective analysis of confirmed cases of PSIS was performed. The development of new pituitary hormonal deficiencies and response to recombinant human growth hormone (rhGH) therapy were assessed during follow-up. RESULTS: This study included 14 children (10 boys) of PSIS with median (range) age of 12.15 years (2 months - 18 years). Short stature was the most common presentation (n = 13), and micropenis (n = 4), cleft lip (n = 1) and single central incisor (n = 1) were other midline defects. Growth hormone (GH) deficiency was present in 14 children and 7 of them also had multiple pituitary hormone deficiencies at baseline. Central hypothyroidism (n = 5), secondary adrenal deficiency (n = 4) and gonadotropin deficiencies (n = 2) were also seen. All children received rhGH. The mean height gain on follow-up was 12.78 cm in first year (n = 14), 6.5 cm in second year (n = 8) and 4.07 cm in third year (n = 7) of rhGH therapy. Four children developed additional pituitary hormone deficiency on follow-up. CONCLUSION: Short stature with isolated GH deficiency was the most common presentation of PSIS that showed good response to rhGH therapy.


Assuntos
Nanismo Hipofisário , Hormônio do Crescimento Humano , Hipopituitarismo , Criança , Humanos , Masculino , Hormônio do Crescimento/uso terapêutico , Hipófise , Estudos Retrospectivos , Feminino , Lactente , Pré-Escolar , Adolescente
16.
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
17.
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
18.
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
19.
Childs Nerv Syst ; 40(4): 1259-1270, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38276973

RESUMO

BACKGROUND: Intracranial teratoma represents a rare neoplasm, occurring predominantly during childhood. Characteristic symptoms depend on the location but are mainly hydrocephalus, visual disturbances, hypopituitarism, and diabetes insipidus. Initial diagnosis can be challenging due to similar radiological features in both teratomas and other lesions such as craniopharyngiomas. Gross total resection is recommended if feasible and associated with a good prognosis. CASE DESCRIPTION: A 10-year-old girl presented with newly diagnosed growth retardation, fatigue, cephalgia and bilateral hemianopia. Further laboratory analysis confirmed central hypothyroidism and hypercortisolism. Cranial magnetic resonance imaging showed a cystic space-occupying lesion in the sellar and suprasellar compartment with compression of the optic chiasm without hydrocephalus present, suspicious of craniopharyngioma. Subsequently, an endonasal endoscopic transsphenoidal near-total tumor resection with decompression of the optic chiasm was performed. During postoperative recovery the patient developed transient diabetes insipidus, the bilateral hemianopia remained unchanged. The patient could be discharged in a stable condition, while hormone replacement for multiple pituitary hormone deficiency was required. Surprisingly, histopathology revealed conspicuous areas of skin with formation of hairs and squamous epithelia, compatible with a mature teratoma. CONCLUSIONS: We present an extremely rare case of pediatric sellar teratoma originating from the pituitary gland and a review of literature focusing on the variation in presentation and treatment. Sellar teratomas are often mistaken for craniopharyngioma due to their similar radiographic appearances. However, the primary goal of treatment for both pathologies is to decompress eloquent surrounding structures such as the optic tract, and if applicable, resolution of hydrocephalus while avoiding damage to the pituitary stalk and especially the hypothalamic structures. If feasible, the aim of surgery should be gross total resection.


Assuntos
Neoplasias do Sistema Nervoso Central , Craniofaringioma , Diabetes Insípido , Hidrocefalia , Hipopituitarismo , Neoplasias Hipofisárias , Teratoma , Feminino , Humanos , Criança , Craniofaringioma/cirurgia , Hemianopsia , Neoplasias Hipofisárias/cirurgia , Neoplasias do Sistema Nervoso Central/complicações , Teratoma/cirurgia , Hidrocefalia/complicações
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