scholarly journals Mifepristone Treatment in Four Cases of Primary Bilateral Macronodular Adrenal Hyperplasia (BMAH)

2019 ◽  
Vol 104 (12) ◽  
pp. 6279-6290 ◽  
Author(s):  
Pejman Cohan ◽  
Honey E East ◽  
Sandi-Jo Galati ◽  
Jennifer U Mercado ◽  
Precious J Lim ◽  
...  
Keyword(s):  
Treatment Duration ◽  
Cushing Syndrome ◽  
Signs And Symptoms ◽  
Response Assessment ◽  
Common Adverse Event ◽  
Adrenal Hyperplasia ◽  
Significant Weight Loss ◽  
Rare Form ◽  
Unilateral Adrenalectomy ◽  
Medical Practices

ContextPrimary bilateral macronodular adrenal hyperplasia (BMAH) is a rare form of adrenal Cushing syndrome conventionally treated with adrenalectomy. Medical treatment is often reserved for patients not eligible for surgery. However, to date there have been few studies about the efficacy of mifepristone for the treatment of BMAH associated with hypercortisolism.ObjectiveTo describe a series of patients with hypercortisolism due to BMAH treated with mifepristone from multiple medical practices.DesignWe retrospectively assessed four patients treated with mifepristone for hypercortisolism due to BMAH who had either failed unilateral adrenalectomy, declined surgery, or were poor surgical candidates.ResultsMifepristone induced clinical improvement and remission of the signs and symptoms of hypercortisolism in all described patients with BMAH. The median treatment duration at the time of efficacy response assessment was 5 months (range: 3 to 18 months). Improvement in cardiometabolic parameters was observed as early as 2 weeks after treatment was started. All patients achieved improvements in glycemic control and hypertension and had significant weight loss. The most common adverse event observed with mifepristone therapy was fatigue. Increases in TSH level occurred in two patients.ConclusionMifepristone can be an effective medical alternative to surgery in patients with hypercortisolism due to BMAH.

Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

2017 ◽  
Vol 88 (3-4) ◽  
pp. 285-290 ◽  
Author(s):  
Tomoyo Itonaga ◽  
Hironori Goto ◽  
Manabu Toujigamori ◽  
Yasuharu Ohno ◽  
Seigo Korematsu ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Adrenal Crisis ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Mccune Albright Syndrome ◽  
Total Adrenalectomy ◽  
Bilateral Adrenal Hyperplasia ◽  
New Treatment ◽  
Albright Syndrome ◽  
Mccune Albright

Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.

scholarly journals SAT-184 Silent Pheochromocytoma Complicated by Adrenal Insufficiency After Unilateral Adrenalectomy

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Hassaan B Aftab ◽  
Parvathy Madhavan ◽  
Latha Dulipsingh
Keyword(s):  
Adrenal Insufficiency ◽  
Cushing Syndrome ◽  
Case Reports ◽  
Hypertensive Crisis ◽  
Signs And Symptoms ◽  
Caffeine Intake ◽  
Vanillylmandelic Acid ◽  
Unilateral Adrenalectomy ◽  
Ct Angiogram ◽  
Free Cortisol

Abstract Background: Adrenal insufficiency (AI) following unilateral adrenalectomy for pheochromocytoma (PCC) is an exceedingly rare occurrence described previously in 1 study in only 4 out of 13 patients with unilateral PCC. We discuss an unusual case of a patient with incidentally discovered PCC who underwent unilateral adrenalectomy and subsequently developed AI. Clinical Case: 61-year-old male in overall good health taking no medications presented with left flank and groin pain after a handlebar injury in a bicycle crash. CT angiogram Abdomen & Pelvis revealed a complex enhancing left adrenal mass with internal necrosis measuring 13 x 12 cm. He denied history of headaches, diaphoresis, chest pain or hypertensive crisis. He did endorse palpitations which had resolved with reduced caffeine intake. Labs showed plasma free metanephrine 3,295 pg/mL (ref range <57) and normetanephrine 68,472 pg/mL (<148), 24hr urine metanephrine 192,677 ug (52-341), 24hr urine normetanephrine 171,880 ug (88-444), 24hr urine vanillylmandelic acid (VMA) 182 mg (1.8-6.7), serum aldosterone 8.1 ng/dL (<39.2), serum free cortisol 0.47 ug/dL (0.07-0.93), 24hr urine cortisol 41.6 ug (<60), DHEA sulfate 37 ug/dL (42-290). He was started on phenoxybenzamine and underwent left adrenalectomy. Of note patient remained only minimally hypertensive with tumor manipulation intraoperatively. Pathology was consistent with PCC with no evidence of regional or distant metastases. Postop-labs showed plasma free metanephrine <25 pg/mL and normetanephrine 122 pg/mL. Calcitonin and parathyroid hormone levels were normal. Patient was evaluated in endocrinology clinic 4 weeks after surgery for complaint of severe fatigue, weight loss, anorexia and myalgias. Adrenocorticotropic hormone (ACTH) level was 31 pg/mL (7.2-63) with AM cortisol of 2.1 ug/dL (5-23) which increased to 7.4 ug/dL 1 hour after Cosyntoprin 250 mcg stimulation. He was started on prednisone 10 mg once daily with significant improvement in his symptoms. Conclusion: PCCs typically manifest as sustained or paroxysmal hypertension, episodic headaches, palpitations or diaphoresis. Our patient was found to have a PCC with strikingly elevated levels of catecholamines without typical signs and symptoms of PCC. To the best of our knowledge this is the 5th reported case where a patient developed AI after unilateral adrenalectomy for PCC. There are case reports describing PCCs which secrete both catecholamines and ACTH. However, our patient lacked clinical or biochemical evidence of hypercortisolism preoperatively and his tumor cells in path sample stained negative for ACTH. Other possible pathophysiologic mechanisms include ectopic corticotropin releasing factor production leading to subclinical Cushing syndrome (SCS). Clinicians should have a high suspicion for SCS in the setting of PCC to promptly diagnose and treat AI after unilateral adrenalectomy.

scholarly journals Clinical and genetic heterogeneity of micronodular adrenal hyperplasia

2021 ◽  
Vol 15 (1) ◽  
pp. 27-35
Author(s):  
A. Chevais ◽  
D. G. Beltsevich ◽  
A. Yu. Abrosimov ◽  
D. A. Derkatch ◽  
A. A. Lazareva
Keyword(s):  
Cushing Syndrome ◽  
Gene Mutations ◽  
Carney Complex ◽  
Clinical Aspects ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Pathogenic Variants ◽  
Familial Form ◽  
Genes Encoding ◽  
Genetically Determined

Micronodular adrenal hyperplasia is a rare cause of ACTH-independent Cushing syndrome. It can be divided into two entities: primary pigmented nodular adrenocortical disease (PPNAD) and non-pigmented micronodular adrenocortical disease, among which familial and sporadic forms are distinguished. The most common is the genetically determined familial form PPNAD, as one of the components of Carney complex. The vast majority of patients have identifiable pathogenic variants in the PRKAR1A gene. In addition to the PRKAR1A gene mutations, inactivating mutations in the genes encoding phosphodiesterases (PDE11A4 and PDE8B), as well as PRKACA gene amplification, have been described in individuals with isolated forms. Despite the relative antiquity of the description of micronodular adrenal hyperplasia and the Carney comlex, a detailed study of pathophysiological mechanisms, genetic and clinical aspects of this pathology, nowadays, clinicians continue to face «atypical» cases. Thus, the nature of this disease is not well understood and requires further research. This review presents the accumulated data on micronodular adrenal hyperplasia, genetics aspects, and also describes 2 unique clinical cases of isolated PPNAD with unilateral adrenalectomy results.

scholarly journals Rare form of adrenal hyperplasia

2014 ◽  
Vol 0 (3-4(48)) ◽  
pp. 53-57
Author(s):  
М. С. Черенько ◽  
Т. Ю. Юзвенко ◽  
О. А. Товкай
Keyword(s):  
Adrenal Hyperplasia ◽  
Rare Form

scholarly journals Late-onset Congenital Adrenal Hyperplasia with Cushing Syndrome

2014 ◽  
Vol 53 (17) ◽  
pp. 1955-1959 ◽  
Author(s):  
Gurbuz Erdogan ◽  
Recai Pabuccu ◽  
Sibel Ertek ◽  
Shoshana Israel ◽  
Banu Yilmaz ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing Syndrome ◽  
Late Onset ◽  
Adrenal Hyperplasia

Case 121: Familial Adrenocorticotropin-independent Macronodular Adrenal Hyperplasia Causing Cushing Syndrome

Radiology ◽  
2007 ◽  
Vol 244 (3) ◽  
pp. 923-926 ◽  
Author(s):  
Timothy D. Watson ◽  
Shital J. Patel ◽  
Peter M. Nardi
Keyword(s):  
Cushing Syndrome ◽  
Adrenal Hyperplasia

scholarly journals Paraneoplastic endocrine-metabolic syndromes

2013 ◽  
pp. 19-29
Author(s):  
Marco Grandi ◽  
Carla Sacchetti ◽  
Stefano Pederzoli ◽  
Cinzia Torri ◽  
Ilaria Sgarbi ◽  
...  
Keyword(s):  
Antidiuretic Hormone ◽  
Cushing Syndrome ◽  
Signs And Symptoms ◽  
Paraneoplastic Syndromes ◽  
Therapeutic Management ◽  
Biochemical Alterations ◽  
Cancer Relapse ◽  
Inappropriate Secretion ◽  
Paraneoplastic Hypercalcemia ◽  
Metabolic Syndromes

BACKGROUND The paraneoplastic syndromes (PS) are characterized by the presence of biochemical alterations, signs and symptoms expressive of cancer distance action into the patient’s organism. Sometimes these syndromes can precede the evidence of malignancy even of some years or can correspond to cancer relapse. PS, even being characterized by general symptoms (fever, anorexia, cachexia), may occur with neurological, rheumathological, osteoarticular, vascular, haematological, nephrological and endocrinological/metabolic symptoms; the latter ones are discussed in this article. AIM OF THE STUDY Here we will focus on the most common PS: paraneoplastic hypercalcemia, inappropriate secretion of antidiuretic hormone (SIADH) and paraneoplastic Cushing syndrome. CONCLUSIONS Our work can be useful in the diagnosis and therapeutic management of paraneoplastic syndromes.

Unilateral Adrenalectomy for Primary Bilateral Macronodular Adrenal Hyperplasia: Analysis of 71 Cases

2019 ◽  
Vol 128 (12) ◽  
pp. 827-834
Author(s):  
Mohammad Sheikh-Ahmad ◽  
Gabriel Dickstein ◽  
Ibrahim Matter ◽  
Carmela Shechner ◽  
Jacob Bejar ◽  
...  
Keyword(s):  
Medical Center ◽  
Urinary Free Cortisol ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Long Period ◽  
Pubmed Database ◽  
Free Cortisol ◽  
Adrenal Masses ◽  
Current Article

Abstract Objective Primary bilateral macronodular adrenal hyperplasia (PBMAH) is characterized by benign bilateral enlarged adrenal masses, causing Cushing’s syndrome (CS). The aim of the current article is to define the role of unilateral adrenalectomy (UA) in treating patients with CS related to PBMAH. Methods A PubMed database search was conducted to identify articles reporting UA to treat PBMAH. We also report cases of PBMAH from our medical center treated by UA. Results A total number of 71 cases of PBMAH (62 cases reported in the literature and 9 cases from our center) are presented. Most patients were women (73.2%) and most UA involved the left side (64.3%). In most cases, the resected gland was the larger one. Following UA, 94.4% of cases had remission of hypercortisolism. Recurrence rate of CS was 19.4% and hypoadrenalism occurred in 29.6%. After UA, when the size of the remained adrenal gland was equal or greater than 3.5 cm, CS persisted in 21.4% of cases, and recurrence occurred in 27.3% of cases (after 20±9.2 months). However, when the size of the remained gland was less than 3.5 cm, CS resolved in all cases and recurrence occurred in 21.2% of cases after a long period (65.6±52.1 months). High levels of urinary free cortisol (UFC) were not correlated with post-surgical CS recurrence or persistence. Conclusions UA leads to beneficial outcomes in patients with CS related to PBMAH, also in cases with pre-surgical elevated UFC or contralateral large gland.

scholarly journals Knowledge Sharing of Medical Practices—Reducing the Negative Socio-Economic Effects of Lead Exposure due to Unhealthy Consumption Habits

2020 ◽  
Vol 12 (4) ◽  
pp. 1553
Author(s):  
Radu Ciprian Țincu ◽  
Cătălina Radu ◽  
Alecxandrina Deaconu ◽  
Corina Frăsineanu ◽  
Mihai Vrîncuț ◽  
...  
Keyword(s):  
Knowledge Sharing ◽  
Lead Exposure ◽  
Quantitative Methods ◽  
Interdisciplinary Approach ◽  
Signs And Symptoms ◽  
Economic Effects ◽  
Medical Practices ◽  
Qualitative And Quantitative Methods ◽  
Socio Economic Impact ◽  
Consumption Habits

The emergence of knowledge economy has created the necessary conditions for an interdisciplinary approach of medical practices. This paper explores, in an interdisciplinary manner, the case of lead exposure in the Romanian context. We consider it crucial to be aware of the socio-economic impact of lead exposure in Romania and prevent unhealthy consumption habits that generate economic and social costs. The purpose of the research is to evaluate the social, economic, and medical effects of lead poisoning, and, on this basis, to identify prevention recommendations. The research was conducted while using a descriptive survey based on qualitative and quantitative methods, by analyzing a group of 115 participants that were admitted in the Toxicology Department of the Clinical Emergency Hospital Bucharest with the diagnosis of lead exposure/intoxication. The study followed an Observational Retrospective Protocol that was based on the investigations of the participants registered in the Toxicology Department with signs and symptoms suggestive of lead exposure/intoxication. The data was statistically processed by while using Microsoft Excel 2013 and SPSS Statistics 22. Based on our research results, we facilitated the knowledge sharing process of medical practices and formulated a series of recommendations in an interdisciplinary manner, in order to diminish the negative socio-economic effects of unhealthy consumption habits.

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