Corticotropin-Independent Macronodular Adrenal Hyperplasia

Swain, James; Grant, Clive S.; Schlinkert, Richard T.; Thompson, Geoffrey B.; vanHeerden, Jon A.; Lloyd, Ricardo V.; Young, William F.

doi:10.1001/archsurg.133.5.541

Cited by 63 publications

(49 citation statements)

References 14 publications

(24 reference statements)

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“…The mean age of our patients (53 years) at presentation is another clinical feature in favor of AIMAH (25). Primary pigmented nodular adrenocortical disease, another form of bilateral ACTH-independent CS, occurs at an earlier age.…”

Section: Discussionmentioning

confidence: 85%

Aberrant cortisol regulations in bilateral macronodular adrenal hyperplasia: a frequent finding in a prospective study of 32 patients with overt or subclinical Cushing's syndrome

Libé¹,

Coste²,

Guignat³

et al. 2010

European Journal of Endocrinology

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Context: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and heterogeneous condition characterized by abnormal steroid production. Cortisol secretion can be regulated by aberrant hormone receptors. Objective: A large series of patients with AIMAH were evaluated to provide information on the prevalence and profile of aberrant regulations, in relation with the functional status. Design and patients: Thirty-two consecutive patients with AIMAH were prospectively studied: 10 had a Cushing's syndrome (CS), and 22 had a subclinical CS (SCS). Methods: A baseline endocrine evaluation was followed by an in vivo protocol in search of aberrant cortisol responses (seven provocative tests). An acute inhibition test with the somatostatin analog octreotide was also performed. Results: At least one aberrant cortisol response was identified in 28 of 32 (87%) patients. The overall prevalence of aberrant responses was independent of the functional status. Responses to the upright posture and to metoclopramide were frequently observed (67 and 56% respectively). A glucagon response was frequently observed in the SCS group (58%). A cortisol inhibition by octreotide was specifically found in the three CS patients who positively responded to the mixed meal, and was observed also in 12 of 13 (92%) patients with SCS. Conclusions: Cortisol responses indicative of aberrant receptor expression were highly prevalent in AIMAH. Thorough phenotyping of AIMAH may help uncover the underlying pathophysiology.

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Section: Discussionmentioning

confidence: 85%

Aberrant cortisol regulations in bilateral macronodular adrenal hyperplasia: a frequent finding in a prospective study of 32 patients with overt or subclinical Cushing's syndrome

Libé¹,

Coste²,

Guignat³

et al. 2010

European Journal of Endocrinology

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“…However such treatments have so far been reported in only very few patients and the reference treatment of aiMah remains bilateral adrenalectomy [39], with ensuing postoperative adrenal insufficiency, which carries a risk for fatal adrenal crisis in cases of poor compliance or inadequate adaptation of the lifelong substitutive therapy. Therefore one must better define the clinical characteristics of patients with AIMAH that may benefit from an alternative to bilateral adrenalectomy.…”

Section: Subject and Methodsmentioning

confidence: 99%

Adrenal Cushing's Syndrome Due to Bilateral Macronodular Adrenal Hyperplasia: Prediction of the Efficacy of .BETA.-blockade Therapy and Interest of Unilateral Adrenalectomy

et al. 2009

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Abstract. Bilateral adrenalectomy is the standard treatment for Cushing's syndrome (CS) related to aCTh-independent bilateral macronodular hyperplasia (AIMAH), although it imposes life-long adrenal insufficiency. This study reports a clinical case in order to discuss the clinical interest of pharmacological β-blockade of illegitimate membrane receptors and unilateral adrenalectomy as alternatives to bilateral adrenalectomy for treatment of CS due to aiMah. evidence for cortisol stimulation by upright posture and insulin-induced hypoglycemia in a patient with CS related to aiMah led us to try β-blockers as a therapeutic test and then as a first line treatment. Thus, a 3-day β-blocker test (320mg/d propranolol) induced normalization of cortisol secretion, with return of hypercortisolism at the end of the test. a long term treatment with 320mg/d propranolol allowed sustained normalization of cortisol secretion and progressive disappearance of Cushingoid features but after 8 months the patient complained of Raynaud's syndrome and fatigue. Lowering propranolol dosage or switching to atenolol was less efficient to reduce cortisol levels. Unilateral adrenalectomy was then performed as a second line treatment, leading to normalisation of the 24h urinary cortisol without adrenal insufficiency. Long term control of blood pressure and glycemia were observed during a 7-year follow-up without β-blocker. In conclusion, a 3-day propranolol test may identify patients with AIMAH who can benefit from a long term β-blocker treatment. In case of intolerance to β-blocking agents, unilateral adrenalectomy may allow for long term control of Cushing's syndrome related to AIMAH without adrenal insufficiency.

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“…Trata-se de uma patologia rara, descrita pela primeira vez em 1964 (10) e a seguir em relatos isolados. Em 1998, estudo publicado no Archives of Surgery relata a experiência da Mayo Clinic com 9 pacientes acompanhados nos últi-mos 18 anos (11). Os autores chamam a atenção para a raridade da doença já que, até esta data, só haviam sido relatados cerca de 40 casos na literatura, e definem as características clínicas, bioquímicas, radiológicas e patológicas da doença.…”

Section: Possíveis Mecanismos Patogenéticos Da Possíveis Mecanismos Punclassified

Síndrome de Cushing associada à hiperplasia macronodular das adrenais: apresentação de um caso e revisão da literatura

Pereira

Araújo

Bisi

2001

Arq Bras Endocrinol Metab

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RESUMO RESUMOO objetivo dessa apresentação é discutir a Síndrome de Cushing secundária à hiperplasia macronodular primária das adrenais. Inicialmente, será apresentado um caso clínico e, em seguida, serão discutidos a apresentação clínica, laboratorial, radiológica e histológica, e os possíveis mecanismos patogenéticos envolvidos no desenvolvimento dessa patologia. APRESENTAÇÃO DO CASO APRESENTAÇÃO DO CASODra. Maria Adelaide Albergaria Pereira Dra. Maria Adelaide Albergaria Pereira Paciente de 37 anos, sexo feminino, com história de ganho de peso (13kg) e aparecimento de hirsutismo e acne no rosto há 5 anos. Há 2 anos, espaniomenorréia; há 8 meses amenorréia e diminuição da libido e há 6 meses desenvolvimento de hipertensão arterial e dispepsia.Exame físico: PA= 130X90, P= 80bpm, peso= 65kg, altura= 153cm, IMC= 28kg/cm 2 , fácies cushingóide, pletora facial, obesidade de distribuição centrípeta, atrofia muscular nos membros, hirsutismo facial discreto (Ferriman = 12) Avaliação laboratorial: Cortisol urinário = 507-773µg/24h (normal = 30 a 300µg/24h); cortisol sérico 8hs = 20µg/dl (normal = 5 a 35µg/dl); ACTH = < 20pg/ml (normal < 60pg/ml); cortisol sérico após 1mg VO de dexametasona = 16,7µg/dl; cortisol sérico após 8mg VO de dexametasona (dose única noturna) = 19,2µg/dl. A injeção de 10µg de desmopressina (DDAVP) não provocou aumento do ACTH e cortisol, que permaneceram em níveis semelhantes aos basais: < 20pg/ml e 19,7µg/dl, respectivamente.Avaliação radiológica: Ressonância magnética (RM) de hipófise = normal; Tomografia computadorizada (TC) de tórax = normal e TC de abdome: hiperplasia macronodular bilateral das adrenais (figura 1).Com a hipótese diagnóstica de hiperplasia macronodular primária das adrenais a paciente foi submetida a outros testes funcionais:Teste da Cortrosina (250µg IV): cortisol basal = 22,3µg/dl e 60min após = 39,2µg/dl. 2.Determinação do cortisol no período pós alimentar (1 e 2 horas após cada refeição principal): não houve alteração do cortisol no período pós-prandial.

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Corticotropin-Independent Macronodular Adrenal Hyperplasia

Cited by 63 publications

References 14 publications

Aberrant cortisol regulations in bilateral macronodular adrenal hyperplasia: a frequent finding in a prospective study of 32 patients with overt or subclinical Cushing's syndrome

Aberrant cortisol regulations in bilateral macronodular adrenal hyperplasia: a frequent finding in a prospective study of 32 patients with overt or subclinical Cushing's syndrome

Adrenal Cushing's Syndrome Due to Bilateral Macronodular Adrenal Hyperplasia: Prediction of the Efficacy of .BETA.-blockade Therapy and Interest of Unilateral Adrenalectomy

Síndrome de Cushing associada à hiperplasia macronodular das adrenais: apresentação de um caso e revisão da literatura

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