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- Gkaniatsa, Eleftheria, et al.
(author)
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Adrenal venous sampling in young patients with primary aldosteronism. Extravagance or irreplaceable?
- 2021
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In: The Journal of clinical endocrinology and metabolism. - : The Endocrine Society. - 1945-7197 .- 0021-972X. ; 106:5
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Journal article (peer-reviewed)abstract
- Current clinical guidelines suggest that adrenal venous sampling (AVS) may not be mandatory in young patients with primary aldosteronism (PA) and a solitary adrenal adenoma on imaging.The aim of this study was to further elucidate whether conventional imaging alone is sufficient to distinguish unilateral from bilateral PA among patients aged 40 years or younger.This was a retrospective study where data from 45 patients with PA, aged between 26 and 40 years, who underwent successful AVS between 2005 and 2019, were analyzed. Results concerning laterality on imaging studies and AVS were recorded. Outcome in surgically treated patients was assessed according to the Primary Aldosteronism Surgical Outcomes (PASO) criteria.In four of 25 patients with unilateral aldosterone production according to AVS, CT inaccurately suggested bilateral disease. Following unilateral adrenalectomy, all four patients showed complete clinical success. Five of 20 patients with bilateral aldosterone production according to AVS had a solitary adrenal nodule (8-19 mm) on imaging. Two of these five patients were treated with unilateral adrenalectomy, neither having complete biochemical and/or clinical success postoperatively. Two of 16 patients younger than 35 years had discordant results, one with unilateral, and one with bilateral aldosterone production, according to AVS.Imaging studies inaccurately predicted laterality in a significant number of young patients with PA. In contrast to current clinical guidelines, our results support AVS for subtype evaluation in young adults with PA, including patients 35 years or younger.
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| 2. |
- Gkaniatsa, Eleftheria, et al.
(author)
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Increasing Incidence of Primary Aldosteronism in Western Sweden During 3 Decades -Yet An Underdiagnosed Disorder
- 2021
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In: Journal of Clinical Endocrinology & Metabolism. - : The Endocrine Society. - 0021-972X .- 1945-7197. ; 106:9, s. E3603-E3610
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Journal article (peer-reviewed)abstract
- Context: Primary aldosteronism (PA) is the most common cause of secondary hypertension.Yet, the incidence of PA in the general population has not been studied. Objective: To estimate the incidence of PA in the general population. Design and methods: Patients who had received a diagnostic code for PA between 1987 and 2016 were identified in the Swedish National Patient Registry. Assessment of clinical and biochemical data was used to validate the diagnosis. The annual incidence of PA was calculated by using the number of inhabitants in the Vastra Gotaland County as a reference. Results: Of 570 identified patients, 473 (83%) had confirmed PA. Eligible for the incidence analysis were 416 patients, 248 (60%) men and 168 (40%) women, diagnosed with PA between 1987 and 2016. The mean (+/- standard deviation) age at diagnosis was 56 +/- 12 years. The median (interquartile range) annual incidence was 2 (1-2) cases per million between 1987 and 1996, 6 (4-9) cases per million between 1997 and 2006 and 17 (12-24) cases per million between 2007 and 2016. At the end of the study (December 31, 2016), 386 patients with confirmed PA were alive and living in the Vastra Gotaland County, giving a prevalence of 231 cases per million (0.022%). Conclusions: Despite increasing incidence, the proportion of patients identified with PA is lower than expected. Given the serious consequences of untreated PA, the noticeably low prevalence at the end of the study stresses the need to increase the awareness of PA among health care providers.
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| 3. |
- Gkaniatsa, Eleftheria, et al.
(author)
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Mortality in Patients With Primary Aldosteronism: A Swedish Nationwide Study.
- 2023
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In: Hypertension (Dallas, Tex. : 1979). - 1524-4563. ; 80:12, s. 2601-2610
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Journal article (peer-reviewed)abstract
- Primary aldosteronism (PA) is associated with increased mortality. The extent to which this phenomenon is affected by sex, age, comorbidities at diagnosis, and different treatment modalities is largely unknown. The objective was to determine all-cause and cause-specific mortality in a population-based cohort of patients with PA and the impact of age at diagnosis, sex, comorbidities, and treatment modalities.We used national registers to identify patients diagnosed with PA between 1997 and 2019 (n=2419) and controls (n=24 187) from the general population, matched for sex, age, and county of residence. We obtained mortality data from the Cause-of-Death Register. We used Cox regression models, adjusted for socioeconomic factors and diabetes, to estimate adjusted hazard ratios (HRs [95% CI]).Overall, 346 (14.3%) patients with PA and 2736 (11.3%) controls died during a median follow-up time of 8.1 years. PA was associated with increased risk from all-cause mortality (HR, 1.23 [95% CI, 1.10-1.38]), death from cardiovascular disease (HR, 1.57 [95% CI, 1.30-1.89]), and stroke (HR, 1.85 [95% CI, 1.16-2.93]). Patients with cardiovascular disease at diagnosis (HR, 1.53 [1.26-1.85]), age >56 years (HR, 1.28 [95% CI, 1.13-1.45]), patients treated with a low dose of a mineralocorticoid receptor antagonist (HR, 1.30 [95% CI, 1.02-1.66]), and untreated patients (HR, 2.51 [95% CI, 1.72-3.67]) had excess mortality.Mortality, mainly due to cardiovascular disease, is increased in patients with PA compared with controls from the general population, particularly in patients aged >56 years, patients with preexisting cardiovascular comorbidities, and patients receiving low dose of a mineralocorticoid receptor antagonist.
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| 5. |
- Gunnesson, Lisa, 1982, et al.
(author)
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Maternal pheochromocytoma and childbirth in Sweden 1973-2015: a population-based study on short and long-term outcome
- 2024
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In: ENDOCRINE. - 1355-008X .- 1559-0100.
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Journal article (peer-reviewed)abstract
- Purpose Data guiding management of pheochromocytoma and paraganglioma (PPGL) in pregnant women is limited, and long-term effects on the child are unknown. The aim of this retrospective registry-based case-cohort study was to assess how maternal PPGL and treatment impacts maternal and fetal outcome, including long-term outcome for the child. The main outcomes were maternal and fetal mortality and morbidity at delivery and relative healthcare consumption in children born by mothers with PPGL during pregnancy. Methods The National Birth Register identified 4,390,869 pregnancies between 1973-2015. Data was crosslinked with three Swedish national registers to identify women diagnosed with pheochromocytoma or paraganglioma within one year before or after childbirth. Hospital records were reviewed and register data was collected for five age-matched controls for each child until age 18. Results 21 women and 23 children were identified (incidence 4.8/1.000.000 births/year), all women with adrenal pheochromocytomas (Pc). The majority (71%) were diagnosed post-partum. Nine women (43%) were hypertensive during pregnancy. Preterm delivery was more common in Pc patients compared to controls (30% vs 6%, p < 0.001). There was no maternal or fetal mortality. Timing of tumor removal did not affect gestational weight or APGAR scores. There was no observed difference in hospital admissions between children affected by maternal Pc and controls. Conclusion Pc was commonly diagnosed after delivery and raised the risk of pre-term delivery, suggesting a need for an increased awareness of this diagnosis. However, reassuringly, there was no fetal or maternal mortality or any observed long-term impact on the children.
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| 7. |
- Jabarkhel, Fatema, et al.
(author)
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Primary Adrenal Leiomyosarcoma: Clinical, Radiological, and Histopathological Characteristics
- 2020
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In: Journal of the Endocrine Society. - : The Endocrine Society. - 2472-1972. ; 4:6
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Journal article (peer-reviewed)abstract
- Primary adrenal leiomyosarcoma (PAL) is a rare, high-grade proliferating mesenchymal tumor with a considerable risk of metastasis, deriving from the smooth muscle wall of a central adrenal vein, or its tributaries. Roughly 40 patients with PAL have been reported in the literature. Herein, we present 3 patients with incidentally discovered PAL, along with an overview of the current knowledge on the clinical, radiological, and histopathological characteristics of PAL. (C) Endocrine Society 2020.
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| 8. |
- Muth, Andreas, 1974, et al.
(author)
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Systematic review of surgery and outcomes in patients with primary aldosteronism.
- 2015
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In: The British journal of surgery. - : Oxford University Press (OUP). - 1365-2168 .- 0007-1323. ; 102:4, s. 307-17
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Journal article (peer-reviewed)abstract
- Primary aldosteronism (PA) is the most common cause of secondary hypertension. The main aims of this paper were to review outcome after surgical versus medical treatment of PA and partial versus total adrenalectomy in patients with PA.
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| 9. |
- Papakokkinou, Eleni, et al.
(author)
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Adrenal venous sampling in patients with ACTH-independent hypercortisolism
- 2019
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In: Endocrine. - : Springer Science and Business Media LLC. - 1355-008X .- 1559-0100. ; 66:2
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Journal article (peer-reviewed)abstract
- © 2019, The Author(s). Purpose: To study the usefulness of adrenal venous sampling (AVS) in distinguishing unilateral from bilateral cortisol production in patients with ACTH-independent hypercortisolism and bilateral adrenal lesions, or morphologically normal adrenal glands. Methods: A retrospective analysis of ten consecutive patients with ACTH-independent hypercortisolism who underwent AVS at our institution between 2009 and 2017. Unilateral dominant cortisol production was defined as a side-to-side cortisol/aldosterone lateralization ratio >2. Results: Four of ten patients had overt Cushing’s syndrome. Of these, two had bilateral adrenal lesions on computed tomography and two had normal adrenal glands. One of the two patients with bilateral adrenal lesions had, based on the AVS, a unilateral dominant cortisol production. Following unilateral adrenalectomy the patient developed adrenal insufficiency. The other three patients were considered to have bilateral cortisol production and underwent bilateral adrenalectomy. Six patients had a mild autonomous cortisol secretion and bilateral adrenal lesions. Based on AVS, one patient was considered to have unilateral dominant cortisol production, underwent unilateral adrenalectomy and developed transient adrenal insufficiency postoperatively. Conclusions: AVS may contribute to appropriate treatment in patients with ACTH-independent hypercortisolism and bilateral adrenal lesions. In our series, AVS was helpful in the decision-making of two out of ten patients, avoiding chronic treatment with steroidogenesis inhibitors, or inappropriate bilateral adrenalectomy.
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| 10. |
- Ragnarsson, Oskar, 1971, et al.
(author)
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Illness perspectives in patients with primary aldosteronism.
- 2022
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In: PloS one. - : Public Library of Science (PLoS). - 1932-6203. ; 17:11
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Journal article (peer-reviewed)abstract
- The burden of symptoms and treatment in patients with primary aldosteronism (PA), as well as the patients' experience of the health care is sparsely studied. The objectives of this study were to describe symptoms considered to be the most troublesome by patients with PA, and to explore health related worries and expectations following treatment.This was an explorative qualitative study where 25 patients with PA, diagnosed between 2017 and 2019, were included; 13 patients who had undergone adrenalectomy and 12 who were receiving medical treatment. Data was collected during six group interviews and analyzed using a thematic approach.Three main themes were identified: 1) Distress of the past, where the most important issues were struggle to receive a correct diagnosis, impaired well-being and the consumption of a large number of tablets, 2) Satisfaction after receiving a correct diagnosis, both in patients with unilateral and bilateral disease, but also dissatisfaction with lack of information about the disease, and 3) Future concerns, where worries about the long-term effects of PA on health in general dominated.Our findings illustrate several important issues related to PA where improvements in patient care are needed, including actions aiming at shortening the long diagnostic delay, a thorough information to the patients about the disease is of great importance, and that all patients with PA, regardless of treatment, would benefit from a structured long-term follow-up.
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