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1.
Clin Endocrinol (Oxf) ; 100(6): 558-564, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38652736

RESUMO

CONTEXT: Epidemiological studies involving patients with acromegaly have yielded conflicting results regarding cancer incidence and causes of mortality in relation to control of growth hormone (GH) excess. OBJECTIVE: The objective of this retrospective cohort study is to clarify these questions and identify goals for treatment and monitoring patients. METHODS: We studied 1845 subjects from the UK Acromegaly Register (1970-2016), obtaining cancer standardised incidence rates (SIR) and all causes standardised mortality rates (SMR) from UK Office for National Statistics, to determine the relationship between causes of mortality-age at diagnosis, duration of disease, post-treatment and mean GH levels. RESULTS: We found an increased incidence of all cancers (SIR, 1.38; 95% CI: 1.06-1.33, p < .001), but no increase in incidence of female breast, thyroid, colon cancer or any measure of cancer mortality. All-cause mortality rates were increased (SMR, 1.35; 95% CI: 1.24-1.46, p < .001), as were those due to vascular and respiratory diseases. All-cause, all cancer and cardiovascular deaths were highest in the first 5 years following diagnosis. We found a positive association between post-treatment and mean treatment GH levels and all-cause mortality (p < .001 and p < .001), which normalised with posttreatment GH levels of <1.0 µg/L or meantreatment GH levels of <2.5 µg/L. CONCLUSION: Acromegaly is associated with increased incidence of all cancers but not thyroid or colon cancer and no increase in cancer mortality. Excess mortality is due to vascular and respiratory disease. The risk is highest in the first 5 years following diagnosis and is mitigated by normalising GH levels.


Assuntos
Acromegalia , Hormônio do Crescimento Humano , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Acromegalia/sangue , Acromegalia/complicações , Acromegalia/terapia , Doenças Cardiovasculares/sangue , Doenças Cardiovasculares/complicações , Hormônio do Crescimento Humano/sangue , Hormônio do Crescimento Humano/metabolismo , Incidência , Neoplasias/complicações , Sistema de Registros , Doenças Respiratórias/complicações , Estudos Retrospectivos , Reino Unido , Doenças Vasculares/complicações
2.
Br J Neurosurg ; : 1-3, 2022 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-35531871

RESUMO

Lithium is a psychotropic drug used primarily in the treatment of bipolar disorder. It is renally excreted and characteristically causes nephrogenic diabetes insipidus as an adverse drug reaction. Lithium also requires serum level monitoring as there is a narrow therapeutic window and untreated toxicity can result in neurological sequelae including drowsiness, coma, seizures, and ultimately death. We present the case of a 65-year old man admitted for pituitary surgery complicated by post-operative difficult fluid management and subsequent lithium toxicity. We highlight this rare situation and the need to be vigilant in the peri-operative period with any patients on lithium who undergo pituitary surgery.

3.
Clin Endocrinol (Oxf) ; 95(1): 143-152, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33749903

RESUMO

CONTEXT: A lack of consensus remains about the relative importance of insulin-like growth factor-1 (IGF-1) and growth hormone (GH) in predicting adverse outcomes in patients with acromegaly. OBJECTIVE: To describe the differing association between IGF-1 and GH and major disease outcomes in acromegaly. DESIGN: Retrospective cohort study. PATIENTS: United Kingdom National Health Service patients with acromegaly who had an IGF-1 and/or a GH measurement recorded following diagnosis, prior to December 2019. MEASUREMENTS: A composite endpoint including all-cause mortality (ACM), type 2 diabetes (DM), major adverse cardiovascular events (MACE) or cancer was the primary outcome. These outcomes were also analysed individually. Follow-up period was capped at 5 years. RESULTS: A maximum of 417 cases and 332 cases were eligible for the IGF-1 and GH analyses, respectively, comprising 1041.5 and 938.9 years of follow-up. There was a direct association between increased IGF-1 concentration and adjusted event risk for the composite endpoint (hazard ratio [HR] = 1.2; 95% confidence interval [CI] = 1.02-1.5); in GH, the HR was 1.1 (1.0-1.2). For the individual endpoints in relation to IGF-1 level, the HRs were ACM (1.2; 0.93-1.5), MACE (1.2; 0.64-2.1), DM (1.53; 1.09-2.2) and cancer (1.3; 0.95-1.7). For GH, the HRs were ACM (1.1; 0.97-1.2), MACE (0.99; 0.73-1.3), DM (1.1; 0.99-1.2) and cancer (0.90; 0.66-1.2). CONCLUSIONS: In this contemporary data set with extended follow-up, IGF-1 and GH concentrations showed an association with major adverse outcomes from acromegaly.


Assuntos
Acromegalia , Diabetes Mellitus Tipo 2 , Hormônio do Crescimento Humano , Hormônio do Crescimento , Humanos , Fator de Crescimento Insulin-Like I , Estudos Retrospectivos , Medicina Estatal
4.
BMC Med Educ ; 20(1): 274, 2020 Aug 18.
Artigo em Inglês | MEDLINE | ID: mdl-32811488

RESUMO

BACKGROUND: Simulation-based learning (SBL) has been increasingly used in both undergraduate and postgraduate medical training curricula. The aim of Simulation via Instant Messaging-Birmingham Advance (SIMBA) is to create a simple virtual learning environment to improve trainees' self-reported confidence in diabetes and Endocrinology. METHODS: This study was done as part of the continuous professional development for Health Education England West Midlands speciality trainees in diabetes and Endocrinology. Standardized transcripts of anonymized real-life endocrinology (endocrine session) and diabetes cases (diabetes session) were used in the simulation model. Trainees interacted with moderators through WhatsApp® in this model. All cases were then discussed in detail by a consultant endocrinologist with reference to local, national and international guidelines. Trainee acceptance rate and improvement in their self-reported confidence levels post-simulation were assessed. RESULTS: 70.8% (n = 17/24) and 75% (n = 18/24) strongly agreed the simulation session accommodated their personal learning style and the session was engaging. 66.7% (n = 16/24) strongly felt that the simulation was worth their time. In the endocrine session, there was a significant improvement in trainees' confidence in the management of craniopharyngioma (p = 0.0179) and acromegaly (p = 0.0025). There was a trend towards improved confidence levels to manage Cushing's disease and macroprolactinoma. In diabetes session, there was a significant improvement in trainees' confidence to interpret continuous glucose monitor readings (p = 0.01). There was a trend towards improvement for managing monogenic diabetes, hypoglycaemic unawareness and interpreting Libre readings. Overall, there was a significant improvement in trainees' confidence in managing cases that were discussed post-simulation. CONCLUSION: SIMBA is an effective learning model to improve trainees' confidence to manage various diabetes and endocrine case scenarios. More sessions with a variety of other speciality case scenarios are needed to further assess SIMBA's effectiveness and application in other areas of medical training.


Assuntos
Diabetes Mellitus , Endocrinologia , Competência Clínica , Currículo , Diabetes Mellitus/terapia , Endocrinologia/educação , Inglaterra , Humanos
5.
Clin Endocrinol (Oxf) ; 89(3): 346-353, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29894000

RESUMO

OBJECTIVE: Discontinuation of dopamine agonist (DA) treatment in women with prolactinoma after menopause is a potential approach; studies systematically assessing long-term outcomes are lacking. Our aim was to investigate the natural history of prolactinoma in this group. DESIGN/PATIENTS: Retrospective cohort study of women with prolactinoma diagnosed before menopause and who after menopause were not on DA. RESULTS: Thirty women were included. Twenty-eight received DA (median duration 18 years, median age at DA withdrawal 52 years). At last assessment (median follow-up 3 years) and compared with values 6-12 months after stopping DA, Prolactin (PRL) increased in 15%, decreased but not normalized in 33% and was normal in 52%; PRL levels or visible adenoma on imaging before DA withdrawal, treatment duration and presence of macro-/microadenoma at diagnosis were not predictors of normoprolactinaemia at last review, whereas PRL values 6-12 months after stopping DA were. Adenoma regrowth was detected in 2/27 patients (7%), who showed gradual increase in PRL. Comparison with 28 women who had DA withdrawal before their menopause revealed lower risk of hyperprolactinaemia recurrence in the postmenopausal group (HR:0.316, 95% CI: 0.101-0.985, P < .05). Two women with microprolactinoma diagnosed in perimenopausal period had not been offered DA; PRL decreased (but not normalized) during observation of 1 and 8 years. CONCLUSIONS: Prolactin normalized over time in nearly half of the women and serum PRL 6-12 months after DA withdrawal is useful predictor. Nonetheless, 7% of the patients demonstrated adenoma regrowth which, given the life expectancy postmenopause, necessitate regular monitoring of the cases with persistent hyperprolactinaemia.


Assuntos
Agonistas de Dopamina/uso terapêutico , Menopausa/fisiologia , Prolactinoma/tratamento farmacológico , Adolescente , Adulto , Bromocriptina/administração & dosagem , Bromocriptina/uso terapêutico , Cabergolina/administração & dosagem , Cabergolina/uso terapêutico , Agonistas de Dopamina/administração & dosagem , Ergolinas/administração & dosagem , Ergolinas/uso terapêutico , Feminino , Humanos , Pós-Menopausa , Prolactina/sangue , Prolactinoma/sangue , Estudos Retrospectivos , Suspensão de Tratamento , Adulto Jovem
6.
Hepatobiliary Pancreat Dis Int ; 17(2): 169-175, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29576279

RESUMO

BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) display wide heterogeneity with highly variable prognosis. This study aimed to identify variables related to survival after surgical resection of PNET. METHODS: A total of 143 patients were identified from a prospectively maintained database. Patient characteristics were analyzed and prognostic factors for overall survival and progression-free survival were evaluated. The WHO, ENETS and AJCC scoring systems were applied to the cohort, and their ability to predict patient outcomes were compared. RESULTS: Multivariate analysis found that female gender, lymph node metastases and increasing WHO 2010 grade to be independently associated with reduced overall survival (P < 0.05). Patients requiring multi-visceral resection or debulking surgery found to be associated with shortest survival. ROC analysis found the ENETS and AJCC scoring systems to be similarly predictive of 5-year overall survival. Modified Ki67 significantly improved its accuracy in predicting 5-year overall survival (AUROC: 0.699 vs 0.605; P < 0.01). CONCLUSIONS: Multi-visceral or debulking surgery is associated with poor outcomes. There seems to be no significant difference between enucleation and anatomical segmental resection. Available scoring systems have reasonable accuracy in stratifying disease severity, with no system identified as being superior. Prognostic stratification with modified grading systems needs further validation before applied in clinical practice.


Assuntos
Estadiamento de Neoplasias/métodos , Tumores Neuroendócrinos/secundário , Tumores Neuroendócrinos/cirurgia , Pancreatectomia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Adulto , Idoso , Área Sob a Curva , Bases de Dados Factuais , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Antígeno Ki-67/análise , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Gradação de Tumores , Tumores Neuroendócrinos/química , Tumores Neuroendócrinos/mortalidade , Pancreatectomia/efeitos adversos , Pancreatectomia/mortalidade , Neoplasias Pancreáticas/química , Neoplasias Pancreáticas/mortalidade , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Curva ROC , Reprodutibilidade dos Testes , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Resultado do Tratamento
7.
Clin Endocrinol (Oxf) ; 87(5): 508-514, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28626987

RESUMO

OBJECTIVE: Most prolactinomas in females are diagnosed during the reproductive age, and the majority are microadenomas. Prolactinomas detected in the postmenopausal period are less common with limited published data on their presentation and prognosis. Our objective was to assess the presenting clinical, biochemical and imaging findings, as well as the outcomes of women diagnosed with a prolactinoma in the postmenopausal period. DESIGN AND METHODS: We undertook a retrospective cohort study of women diagnosed with prolactinoma after menopause and followed up in a large UK pituitary centre. Information on presentation, management and outcomes was collected. RESULTS: Seventeen women with a median age at diagnosis of 63 years (range 52-78) were identified. Headaches and/or visual deterioration were the most commonly reported complaints at detection of the adenoma (47%). Acute pituitary apoplexy was diagnosed at presentation or during follow-up in 18% of the cases. The median serum prolactin was 12 364 mU/L (range 2533-238 479). Macroprolactinomas comprised 94% of the tumours, and 88% of them had supra/parasellar extension. All patients with macroprolactinoma were offered dopamine agonist, and normal prolactin was achieved in 94% of them (median follow-up 91.5 months). Adenoma shrinkage was observed in all women. Improvement or resolution of visual disturbances documented at presentation was observed in 86% of cases. CONCLUSIONS: The clinical phenotype of prolactinomas diagnosed in the postmenopausal period is characterized by dominance of macroadenomas, with frequent supra/parasellar extension and a relative high rate of acute pituitary apoplexy. In this group of patients, the response of the macroadenomas to dopamine agonists is good.


Assuntos
Pós-Menopausa , Prolactinoma/diagnóstico , Idoso , Agonistas de Dopamina/uso terapêutico , Feminino , Humanos , Pessoa de Meia-Idade , Fenótipo , Apoplexia Hipofisária , Prolactinoma/diagnóstico por imagem , Prolactinoma/tratamento farmacológico , Estudos Retrospectivos , Resultado do Tratamento , Transtornos da Visão
8.
Clin Endocrinol (Oxf) ; 85(5): 748-756, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27327840

RESUMO

CONTEXT AND OBJECTIVE: Nonfunctioning pituitary adenomas (NFPAs) are the most common subtype of pituitary tumour. Hypopituitarism is observed in NFPAs due to tumour- or treatment-related factors and may increase mortality risk. Here, we analysed the associations of hypopituitarism, hormone replacement and mortality in a large NFPA cohort derived from two large European centres. DESIGN, SETTING AND PARTICIPANTS: Case note review of all patients treated for NFPA in University Hospitals Birmingham and Beaumont Hospital Dublin between 1999 and 2014 was performed. MAIN OUTCOME MEASURES: Clinical presentation, treatment strategies, pituitary function and vitality status were recorded in each patient. A multivariate Cox regression model was used to examine the association between hypopituitarism, hormone replacement and premature mortality. RESULTS: A total of 519 patients were included in the analysis. Median duration of follow-up was 7·0 years (0·5-43). A total of 81 deaths were recorded (15·6%). On multivariate analysis, adrenocorticotropic hormone (ACTH) and gonadotropin (Gn) deficiencies were associated with an increased relative risk of death (OR 2·26, 95% CI 1·15-4·47, P = 0·01 and OR 2·56, 95% CI 1·10-5·96, P = 0·01, respectively). Increased hydrocortisone (HC) (P-trend = 0·02) and lower levothyroxine (LT4) doses (P-trend = 0·03) were associated with increased risk of death. Mortality increased with the degree of pituitary failure observed (P-trend = 0·04). CONCLUSION: ACTH and gonadotropin-deficient patients have higher mortality rates compared to those with intact hormonal axes. Excessive HC and suboptimal LT4 replacement may also increase risk of death. Complex associations between hormone deficiency and replacement underpin the increased mortality risk in NFPA patients.


Assuntos
Adenoma , Hormônio Adrenocorticotrópico/deficiência , Gonadotropinas/deficiência , Neoplasias Hipofisárias/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Europa (Continente) , Feminino , Seguimentos , Terapia de Reposição Hormonal , Humanos , Hidrocortisona/administração & dosagem , Hipopituitarismo , Masculino , Pessoa de Meia-Idade , Mortalidade , Prognóstico , Tiroxina/administração & dosagem , Adulto Jovem
9.
Neuroendocrinology ; 103(1): 66-74, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-25661647

RESUMO

Acromegaly is a rare condition characterized by excessive secretion of growth hormone (GH), which is almost always due to a pituitary adenoma. Acromegaly is associated with significant morbidity such as hypertension, type 2 diabetes, cardiomyopathy, obstructive sleep apnoea, malignancy and musculoskeletal abnormalities. Acromegaly has also been associated with increased mortality in several retrospective studies. This review will focus on the epidemiological data relating to mortality rates in acromegaly, the relationship between acromegaly and malignancy, the role of GH and insulin-like growth factor-I in assessing the risk of future mortality, and the impact of radiotherapy and hypopituitarism on mortality.


Assuntos
Acromegalia , Adenoma/tratamento farmacológico , Adenoma/mortalidade , Hormônio do Crescimento Humano/metabolismo , Neoplasias Hipofisárias/tratamento farmacológico , Neoplasias Hipofisárias/mortalidade , Hormônio do Crescimento/metabolismo , Humanos , Hipopituitarismo/complicações , Hipopituitarismo/tratamento farmacológico
10.
Eur J Clin Invest ; 45(8): 888-94, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25995119

RESUMO

BACKGROUND: Hyponatraemia is a very common medical condition that is associated with multiple poor clinical outcomes and is often managed suboptimally because of inadequate assessment and investigation. Previously published guidelines for its management are often complex and impractical to follow in a hospital environment, where patients may present to divergent specialists, as well as to generalists. DESIGN: A group of senior, experienced UK clinicians, met to develop a practical algorithm for the assessment and management of hyponatraemia in a hospital setting. The latest evidence was discussed and reviewed in the light of current clinical practicalities to ensure an up-to-date perspective. An algorithm was largely developed following consensus opinion, followed up with subsequent additions and amendments that were agreed by all authors during several rounds of review. RESULTS: We present a practical algorithm which includes a breakdown of the best methods to evaluate volume status, simple assessments for the diagnosis of the various causes and a straightforward approach to treatment to minimise complexity and maximise patient safety. CONCLUSION: The algorithm we have developed reflects the best available evidence and extensive clinical experience and provides practical, useable guidance to improve patient care.


Assuntos
Algoritmos , Antibacterianos/uso terapêutico , Antagonistas dos Receptores de Hormônios Antidiuréticos/uso terapêutico , Benzazepinas/uso terapêutico , Demeclociclina/uso terapêutico , Hidratação , Hiponatremia/terapia , Síndrome de Secreção Inadequada de HAD/terapia , Hospitalização , Humanos , Hiponatremia/diagnóstico , Síndrome de Secreção Inadequada de HAD/diagnóstico , Guias de Prática Clínica como Assunto , Tolvaptan , Desequilíbrio Hidroeletrolítico/diagnóstico , Desequilíbrio Hidroeletrolítico/terapia
11.
Am J Kidney Dis ; 63(4): 691-5, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24100128

RESUMO

Serum magnesium concentration is determined by the interplay of intestinal absorption and renal excretion. Hypomagnesemia can occur as a result of insufficient magnesium intake, increased gastrointestinal or renal loss, or redistribution from extracellular to intracellular compartments. A number of drugs are known to cause hypomagnesemia, including proton pump inhibitors (PPIs). We report the case of a patient with symptomatic hypomagnesemia due to short bowel syndrome and PPI therapy. Investigations revealed low 24-hour urinary magnesium excretion and secondary hypocalcemia. PPI treatment was withdrawn and the patient was managed with intravenous and oral magnesium and calcium replacement. This teaching case provides an evidence-based discussion of the treatment of hypomagnesemia.


Assuntos
Compostos de Magnésio/administração & dosagem , Deficiência de Magnésio/tratamento farmacológico , Doenças Assintomáticas , Feminino , Humanos , Absorção Intestinal/fisiologia , Magnésio/sangue , Deficiência de Magnésio/etiologia , Deficiência de Magnésio/fisiopatologia , Pessoa de Meia-Idade , Inibidores da Bomba de Prótons/uso terapêutico , Síndrome do Intestino Curto/complicações
12.
Artigo em Inglês | MEDLINE | ID: mdl-38655872

RESUMO

BACKGROUND: Pheochromocytoma is associated with systemic inflammation, but the underlying mechanisms are unclear. Therefore, we investigated the relationship between plasma metanephrine levels and haematological parameters - as a surrogate of inflammation - in patients with pheochromocytoma and the influence of preoperative α-blockade treatment. DESIGN AND METHODS: We retrospectively studied 68 patients with pheochromocytoma who underwent adrenalectomy (median age 53 years, 64.7% females) and two control groups matched for age, sex, and body mass index (BMI): 68 patients with non-functioning adrenocortical tumors (NFAT) and 53 with essential hypertension (EAH). The complete blood count (CBC) and several inflammation-based scores [Neutrophil-to-Lymphocyte Ratio (NLR), Platelet-to-Lymphocyte Ratio (PLR), Lymphocyte-to-Monocyte Ratio (LMR), Systemic-Immune-Inflammation Index (SII), Prognostic-Nutrition Index (PNI)] were assessed in all patients and, in a subset of pheochromocytomas, after adrenalectomy (n=26) and before and after preoperative α-blockade treatment (n=29). RESULTS: A higher inflammatory state, as indicated by both CBC and inflammation-based scores, was observed in patients with pheochromocytoma compared to NFAT and EAH. Plasma metanephrine levels showed a positive correlation with NLR (r=0.4631), PLR (r=0.3174), SII (r=0.3709), and a negative correlation with LMR (r=0.4368) and PNI (r=0.3741), even after adjustment for age, sex, ethnicity, BMI and tumor size (except for PLR). After adrenalectomy, we observed a reduction in NLR (p=0.001), PLR (p=0.003), SII (p=0.004) and a concomitant increase in LMR (p=0.0002). Similarly, α-blockade treatment led to a reduction in NLR (p=0.007) and SII (p=0.03). CONCLUSIONS: Inflammation-based scores in patients with pheochromocytoma showed pro-inflammatory changes that correlated with plasma metanephrine levels and are ameliorated by adrenalectomy and α-blockade.

13.
Nat Rev Endocrinol ; 20(5): 278-289, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38336897

RESUMO

Tumours of the anterior part of the pituitary gland represent just 1% of all childhood (aged <15 years) intracranial neoplasms, yet they can confer high morbidity and little evidence and guidance is in place for their management. Between 2014 and 2022, a multidisciplinary expert group systematically developed the first comprehensive clinical practice consensus guideline for children and young people under the age 19 years (hereafter referred to as CYP) presenting with a suspected pituitary adenoma to inform specialist care and improve health outcomes. Through robust literature searches and a Delphi consensus exercise with an international Delphi consensus panel of experts, the available scientific evidence and expert opinions were consolidated into 74 recommendations. Part 1 of this consensus guideline includes 17 pragmatic management recommendations related to clinical care, neuroimaging, visual assessment, histopathology, genetics, pituitary surgery and radiotherapy. While in many aspects the care for CYP is similar to that of adults, key differences exist, particularly in aetiology and presentation. CYP with suspected pituitary adenomas require careful clinical examination, appropriate hormonal work-up, dedicated pituitary imaging and visual assessment. Consideration should be given to the potential for syndromic disease and genetic assessment. Multidisciplinary discussion at both the local and national levels can be key for management. Surgery should be performed in specialist centres. The collection of outcome data on novel modalities of medical treatment, surgical intervention and radiotherapy is essential for optimal future treatment.


Assuntos
Adenoma , Neoplasias Hipofisárias , Adulto , Criança , Humanos , Adolescente , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/terapia , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/terapia , Hipófise , Consenso , Neuroimagem
14.
Nat Rev Endocrinol ; 20(5): 290-309, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38336898

RESUMO

Pituitary adenomas are rare in children and young people under the age of 19 (hereafter referred to as CYP) but they pose some different diagnostic and management challenges in this age group than in adults. These rare neoplasms can disrupt maturational, visual, intellectual and developmental processes and, in CYP, they tend to have more occult presentation, aggressive behaviour and are more likely to have a genetic basis than in adults. Through standardized AGREE II methodology, literature review and Delphi consensus, a multidisciplinary expert group developed 74 pragmatic management recommendations aimed at optimizing care for CYP in the first-ever comprehensive consensus guideline to cover the care of CYP with pituitary adenoma. Part 2 of this consensus guideline details 57 recommendations for paediatric patients with prolactinomas, Cushing disease, growth hormone excess causing gigantism and acromegaly, clinically non-functioning adenomas, and the rare TSHomas. Compared with adult patients with pituitary adenomas, we highlight that, in the CYP group, there is a greater proportion of functioning tumours, including macroprolactinomas, greater likelihood of underlying genetic disease, more corticotrophinomas in boys aged under 10 years than in girls and difficulty of peri-pubertal diagnosis of growth hormone excess. Collaboration with pituitary specialists caring for adult patients, as part of commissioned and centralized multidisciplinary teams, is key for optimizing management, transition and lifelong care and facilitates the collection of health-related quality of survival outcomes of novel medical, surgical and radiotherapeutic treatments, which are currently largely missing.


Assuntos
Acromegalia , Adenoma , Neoplasias Hipofisárias , Prolactinoma , Adulto , Masculino , Feminino , Humanos , Adolescente , Criança , Idoso , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/terapia , Neoplasias Hipofisárias/patologia , Adenoma/diagnóstico , Adenoma/terapia , Prolactinoma/diagnóstico , Prolactinoma/cirurgia
15.
J Clin Endocrinol Metab ; 109(1): e389-e396, 2023 Dec 21.
Artigo em Inglês | MEDLINE | ID: mdl-37417693

RESUMO

CONTEXT: Pheochromocytomas are increasingly diagnosed in incidentally detected adrenal masses. However, the characteristics of incidental pheochromocytomas are unclear. OBJECTIVE: We aimed to assess the proportion and clinical, biochemical, radiological, genetic, histopathological, and follow-up characteristics of incidental pheochromocytomas. METHODS: A retrospective review was conducted of patients with pheochromocytoma seen between January 2010 and October 2022 at a large UK tertiary care center. The diagnosis was confirmed histologically or by the combined presence of increased plasma and/or urinary metanephrines (MN), indeterminate adrenal mass on cross-sectional imaging, and metaiodobenzylguanidine avidity. RESULTS: We identified 167 patients with pheochromocytoma; 144 (86.2%) underwent adrenalectomy, for 23 (13.8%) surgery was either awaited, deemed unsuitable due to frailty or other metastatic malignancy, or declined by the patients. Excluding pheochromocytomas diagnosed via screening genetically predisposed individuals (N = 20), 37 of 132 (28.0%) presented with adrenergic symptoms and/or uncontrolled hypertension, while 91 of 132 (69.0%) patients presented with an incidentally detected adrenal mass. Incidentally detected patients were older (median age 62 years) than those detected due to clinical suspicion (aged 42 years) or after genetic screening (aged 33 years) (all P < .05). Incidentally detected pheochromocytomas were smaller (median 42 mm) than tumors detected due to adrenergic symptoms/uncontrolled hypertension (60 mm), but larger than tumors identified by genetic screening (30 mm) (all P < .05). Increased MN excretion showed a similar pattern (symptomatic/uncontrolled hypertension > incidental > genetic screening) (all P < .05). Hereditary predisposition was detected in 20.4% of patients (incidental, 15.3%; symptomatic/uncontrolled hypertension, 42.9%). CONCLUSION: The majority of pheochromocytomas are diagnosed incidentally and have distinct clinical, radiological, biochemical, and genetic features. Their detection at older age but smaller size may point to a different underlying tumor biology.


Assuntos
Neoplasias das Glândulas Suprarrenais , Hipertensão , Feocromocitoma , Humanos , Pessoa de Meia-Idade , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/genética , Feocromocitoma/diagnóstico , Feocromocitoma/epidemiologia , Feocromocitoma/genética , Hipertensão/epidemiologia , Adrenérgicos , Predisposição Genética para Doença
16.
Eur J Surg Oncol ; 49(2): 497-504, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36602554

RESUMO

BACKGROUND: Due to the risk of postoperative hypotension (PH), invasive monitoring is recommended for patients who undergo adrenalectomy for phaeochromocytoma. Due to high costs and limited availability of intensive care, our aim was to identify patients at low risk of PH who may not require invasive monitoring. METHODS: Data for patients who underwent adrenalectomy for phaeochromocytoma between 2012 and 2020 were retrospectively collected by nine UK centres, including patient demographics, intraoperative and postoperative haemodynamic parameters. Independent risk factors for PH were analysed and used to develop a clinical risk score. RESULTS: PH developed in 118 of 430 (27.4%) patients. On univariable analysis, female sex (p = 0.007), tumour size (p < 0.001), preoperative catecholamine level (p < 0.001), open surgery (p < 0.001) and epidural analgesia (p = 0.006) were identified as risk factors for PH. On multivariable analysis, female sex (OR 1.85, CI95%, 1.09-3.13, p = 0.02), preoperative catecholamine level (OR: 3.11, CI95%, 1.74-5.55, p < 0.001), open surgery (OR: 3.31, CI95%, 1.57-6.97, p = 0.002) and preoperative mean arterial blood pressure (OR: 0.59, CI95%, 0.48-1.02, p = 0.08) were independently associated with PH, and were incorporated into a clinical risk score (AUROC 0.69, C-statistic 0.69). The risk of PH was 25% and 68% in low and high risk patients, respectively. CONCLUSION: The derived risk score allows stratification of patients at risk of postoperative hypotension after adrenalectomy for phaeochromocytoma. Postoperatively, low risk patients may be managed on a surgical ward, whilst high risk patients should undergo invasive monitoring.


Assuntos
Neoplasias das Glândulas Suprarrenais , Hipotensão , Laparoscopia , Feocromocitoma , Humanos , Feminino , Feocromocitoma/cirurgia , Estudos Retrospectivos , Adrenalectomia , Neoplasias das Glândulas Suprarrenais/cirurgia , Fatores de Risco , Catecolaminas
17.
BJS Open ; 7(5)2023 09 05.
Artigo em Inglês | MEDLINE | ID: mdl-37757753

RESUMO

BACKGROUND: To determine the incidence and risk factors for postoperative complications and prolonged hospital stay after adrenalectomy for phaeochromocytoma. METHODS: Demographics, perioperative outcomes and complications were evaluated for consecutive patients who underwent adrenalectomy for phaeochromocytoma from 2012 to 2020 in nine high-volume UK centres. Odds ratios were calculated using multivariable models. The primary outcome was postoperative complications according to the Clavien---Dindo classification and secondary outcome was duration of hospital stay. RESULTS: Data were available for 406 patients (female n = 221, 54.4 per cent). Two patients (0.5 per cent) had perioperative death, whilst 148 complications were recorded in 109 (26.8 per cent) patients. On adjusted analysis, the age-adjusted Charlson Co-morbidity Index ≥3 (OR 8.09, 95 per cent c.i. 2.31 to 29.63, P = 0.001), laparoscopic converted to open (OR 10.34, 95 per cent c.i. 3.24 to 36.23, P <0.001), and open surgery (OR 11.69, 95 per cent c.i. 4.52 to 32.55, P <0.001) were independently associated with postoperative complications. Overall, 97 of 430 (22.5 per cent) had a duration of stay ≥5 days and this was associated with an age-adjusted Charlson Co-morbidity Index ≥3 (OR 4.31, 95 per cent c.i. 1.08 to 18.26, P = 0.042), tumour size (OR 1.15, 95 per cent c.i. 1.05 to 1.28, P = 0.006), laparoscopic converted to open (OR 32.11, 95 per cent c.i. 9.2 to 137.77, P <0.001), and open surgery (OR 28.01, 95 per cent c.i. 10.52 to 83.97, P <0.001). CONCLUSION: Adrenalectomy for phaeochromocytoma is associated with a very low mortality rate, whilst postoperative complications are common. Several risk factors, including co-morbidities and operative approach, are independently associated with postoperative complications and/or prolonged hospitalization, and should be considered when counselling patients.


Assuntos
Neoplasias das Glândulas Suprarrenais , Feocromocitoma , Humanos , Feminino , Masculino , Feocromocitoma/cirurgia , Adrenalectomia/efeitos adversos , Neoplasias das Glândulas Suprarrenais/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fatores de Risco , Estudos de Coortes
18.
Eur J Endocrinol ; 189(1): 87-95, 2023 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-37345849

RESUMO

OBJECTIVE: The optimal approach to the surveillance of non-functioning pituitary microadenomas (micro-NFPAs) is not clearly established. Our aim was to generate evidence on the natural history of micro-NFPAs to support patient care. DESIGN: Multi-centre, retrospective, cohort study involving 23 endocrine departments (UK NFPA consortium). METHODS: Clinical, imaging, and hormonal data of micro-NFPA cases between January, 1, 2008 and December, 21, 2021 were analysed. RESULTS: Data for 459 patients were retrieved [median age at detection 44 years (IQR 31-57)-152 males/307 females]. Four hundred and nineteen patients had more than two magnetic resonance imagings (MRIs) [median imaging monitoring 3.5 years (IQR 1.71-6.1)]. One case developed apoplexy. Cumulative probability of micro-NFPA growth was 7.8% (95% CI, 4.9%-8.1%) and 14.5% (95% CI, 10.2%-18.8%) at 3 and 5 years, respectively, and of reduction 14.1% (95% CI, 10.4%-17.8%) and 21.3% (95% CI, 16.4%-26.2%) at 3 and 5 years, respectively. Median tumour enlargement was 2 mm (IQR 1-3) and 49% of micro-NFPAs that grew became macroadenomas (nearly all >5 mm at detection). Eight (1.9%) patients received surgery (only one had visual compromise with surgery required >3 years after micro-NFPA detection). Sex, age, and size at baseline were not predictors of enlargement/reduction. At the time of detection, 7.2%, 1.7%, and 1.5% patients had secondary hypogonadism, hypothyroidism, and hypoadrenalism, respectively. Two (0.6%) developed hypopituitarism during follow-up (after progression to macroadenoma). CONCLUSIONS: Probability of micro-NFPA growth is low, and the development of new hypopituitarism is rare. Delaying the first follow-up MRI to 3 years and avoiding hormonal re-evaluation in the absence of tumour growth or clinical manifestations is a safe approach for micro-NFPA surveillance.


Assuntos
Adenoma , Hipopituitarismo , Neoplasias Hipofisárias , Masculino , Feminino , Humanos , Adulto , Pessoa de Meia-Idade , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/complicações , Estudos Retrospectivos , Estudos de Coortes , Adenoma/diagnóstico por imagem , Adenoma/epidemiologia , Hipopituitarismo/complicações , Reino Unido/epidemiologia
19.
Clin Endocrinol (Oxf) ; 76(3): 328-31, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22107606

RESUMO

Radiotherapy for pituitary adenomas is a well-established and effective treatment, but it has been linked to a number of complications, including an increased risk of cerebrovascular disease and mortality. Possible underlying mechanisms include the direct effects of radiation on cerebral vasculature and deficiencies in specific pituitary hormones. Higher administered dose of radiotherapy is one of the main predictors of stroke. Although treatment strategies for stroke prevention in patients treated with pituitary radiotherapy have not been studied, using the lowest effective radiation dose and ensuring hormone deficiencies are adequately treated may be beneficial in reducing the risk of stroke.


Assuntos
Adenoma/radioterapia , Hipopituitarismo/prevenção & controle , Neoplasias Hipofisárias/radioterapia , Acidente Vascular Cerebral/prevenção & controle , Relação Dose-Resposta à Radiação , Terapia de Reposição Hormonal/métodos , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Hipopituitarismo/etiologia , Radioterapia/efeitos adversos , Dosagem Radioterapêutica/normas , Fatores de Risco , Acidente Vascular Cerebral/etiologia
20.
Asian J Neurosurg ; 17(3): 527-531, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36398168

RESUMO

Infected Rathke's cleft cysts (RCC) are extremely rare with only a few published cases. We report the case of a 31-year-old man who presented with headaches, visual disturbance, and hypopituitarism secondary to an infected RCC with extension of abscesses along the optic tract. Magnetic resonance imaging showed ring enhancing cystic lesions within an expanded sella with suprasellar and intraparenchymal extension. The radiological appearance suggested a high-grade optic glioma, but an endoscopic transsphenoidal biopsy revealed frank pus in the pituitary fossa, which subsequently grew Staphylococcus aureus . Pathological examination of the cyst wall showed an inflamed RCC. Following a prolonged course of intravenous antibiotics, the infection resolved and vision improved. RCC abscesses are rare and the intracranial extension of the infection in our case makes it unique.

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