scholarly journals Spontaneous resolution of bilateral avascular necrosis of the femoral head following cure of Cushing's syndrome secondary to primary pigmented micronodular adrenal disease

2015 ◽  
Author(s):  
Agnieszka Pazderska ◽  
Stephen Crowther ◽  
Pradeep Govender ◽  
Kevin C Conlon ◽  
James Gibney ◽  
...  
Keyword(s):  
Femoral Head ◽  
Avascular Necrosis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Spontaneous Resolution ◽  
Adrenal Disease

scholarly journals Spontaneous resolution of avascular necrosis of femoral heads following cure of Cushing’s syndrome

2016 ◽  
Vol 2016 ◽  
Author(s):  
A Pazderska ◽  
S Crowther ◽  
P Govender ◽  
K C Conlon ◽  
M Sherlock ◽  
...  
Keyword(s):  
Femoral Head ◽  
Avascular Necrosis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Leg Pain ◽  
List Type ◽  
Rapid Weight Gain ◽  
Free Cortisol ◽  
Femoral Heads ◽  
Adrenal Disease

Summary Avascular necrosis (AVN) is a rare presenting feature of endogenous hypercortisolism. If left untreated, complete collapse of the femoral head may ensue, necessitating hip replacement in up to 70% of patients. The majority of the described patients with AVN due to endogenous hypercortisolaemia required surgical intervention. A 36-year-old female, investigated for right leg pain, reported rapid weight gain, bruising and secondary amenorrhoea. She had abdominal adiposity with violaceous striae, facial plethora and hirsutism, atrophic skin, ecchymosis and proximal myopathy. Investigations confirmed cortisol excess (cortisol following low-dose 48h dexamethasone suppression test 807nmol/L; 24h urinary free cortisol 1443nmol (normal<290nmol)). Adrenocorticotrophic hormone (ACTH) was <5.0pg/mL. CT demonstrated subtle left adrenal gland hypertrophy. Hypercortisolaemia persisted after left adrenalectomy. Histology revealed primary pigmented micronodular adrenal disease. Post-operatively, right leg pain worsened and left leg pain developed, affecting mobility. MRI showed bilateral femoral head AVN. She underwent right adrenalectomy and steroid replacement was commenced. Four months after surgery, leg pain had resolved and mobility was normal. Repeat MRI showed marked improvement of radiological abnormalities in both femoral heads, consistent with spontaneous healing of AVN. We report a case of Cushing’s syndrome due to primary pigmented nodular adrenocortical disease, presenting with symptomatic AVN of both hips. This was managed conservatively from an orthopaedic perspective. Following cure of hypercortisolaemia, the patient experienced excellent recovery and remains symptom free 4 years after adrenalectomy. This is the first report of a favourable outcome over long-term follow-up of a patient with bilateral AVN of the hip, which reversed with treatment of endogenous hypercortisolaemia. Learning points AVN of femoral head can be a presenting feature of hypercortisolism, both endogenous and exogenous. Rarely, treatment of hypercortisolaemia can reverse AVN without the need for orthopaedic intervention. Primary pigmented nodular adrenal disease is a rare cause of ACTH-independent Cushing’s syndrome.

scholarly journals Thymus hyperplasia after resolution of hypercortisolism in ACTH-dependent Cushing’s syndrome: the importance of thymic vein catheterization

2006 ◽  
Vol 154 (6) ◽  
pp. 807-811 ◽  
Author(s):  
Malebranche Berardo C Cunha Neto ◽  
Márcio Carlos Machado ◽  
Flávia Mesquita ◽  
Nina Rosa de Castro Musolino ◽  
Andrea Cecília Toscanini ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cushing’S Syndrome ◽  
Transsphenoidal Surgery ◽  
Cushing's Syndrome ◽  
Chest Ct ◽  
Spontaneous Resolution ◽  
Tumor Visualization ◽  
Histopathological Studies ◽  
Thymic Lobe

Thymic hyperplasia has been described after the resolution of hypercortisolism from several etiologies, causing great diagnostic dilemmas. We describe a case where the catheterization of the thymic vein was essential for the differential diagnosis of a thymic enlargement in an adrenalectomized patient with ACTH-dependent Cushing’s syndrome. The patient was a 48-year-old female with clinical and laboratorial data suggesting Cushing’s disease. She underwent a transsphenoidal surgery with no tumor visualization and no remission of the syndrome. Histopathological studies disclosed a normal pituitary. She underwent a bilateral adrenalectomy and 8 months later a chest CT showed an increase of left thymic lobe, which was previously non-existent. After a negative 111In-pentetreotide scintigraphy, the patient underwent simultaneous and bilateral catheterism of the petrosus sinuses and catheterization of the thymic and inominate veins and no ACTH gradient was shown among the sites of collection. She did not undergo thoracotomy and a follow-up was established. During the evolution, there was a spontaneous regression of the thymic lesion 38 months after the diagnosis. The ACTH gradient during the catheterization of thymic vein was essential for the differential diagnosis of the thymic enlargement tumor after hypercortisolism resolution in ACTH-dependent Cushing’s syndrome, especially in this case, where the ACTH source was occult, thus avoiding an invasive surgical procedure for a benign entity with spontaneous resolution.

ACTH-independent massive bilateral adrenal disease (AIMBAD): A subtype of Cushing's syndrome with major diagnostic and therapeutic implications

1994 ◽  
Vol 131 (1) ◽  
pp. 67-73 ◽  
Author(s):  
Steven A Lieberman ◽  
T Ross Eccleshall ◽  
David Feldman
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Glands ◽  
Medical Center ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Bilateral Adrenalectomy ◽  
Preoperative Treatment ◽  
Therapeutic Implications ◽  
Adrenal Imaging ◽  
Adrenal Disease

Lieberman SA, Eccleshall TR, Feldman D. ACTH-independent massive bilateral adrenal disease (AIMBAD): a subtype of Cushing's syndrome with major diagnostic and therapeutic implications. Eur J Endocrinol 1994:131:67–73. ISSN 0804–4643 A 49-year-old man with classic manifestations of Cushing's syndrome had undetectable levels of ACTH, lack of suppression of hypercortisolism with dexamethasone in doses of 2, 8, or 16 mg per day, bilaterally enlarged adrenal glands on MRI, and bilateral adrenal uptake of iodocholesterol. Preoperative treatment with ketoconazole lowered blood pressure and serum cortisol and produced symptoms of steroid withdrawal. Bilateral adrenalectomy revealed massively enlarged adrenal glands (left: 199 g, right: 93 g). Sequencing of the gene encoding the stimulatory G protein, Gsα, did not show either of two activating mutations previously reported in patients with McCune–Albright syndrome or acromegaly. Twenty-three previous cases of Cushing's syndrome due to ACTH-independent massive bilateral adrenal disease (AIMBAD) have been reported. AIMBAD may cause confusion in the differential diagnosis of Cushing's syndrome as endocrine testing suggests a unilateral, ACTHindependent process while adrenal imaging demonstrates bilateral abnormalities. Bilateral adrenalectomy is curative and appears to carry little risk of Nelson's syndrome. The pathogenesis of AIMBAD appears to be heterogeneous, as recent reports have demonstrated GIP-mediated hypercortisolism and familial AIMBAD. Transition from Cushing's disease to ACTH-independence is not supported by the available data. Future cases of AIMBAD should be investigated carefully to further elucidate the pathogenesis of this disorder. David Feldman, Division of Endocrinology, Dept. of Medicine, Room S-005, Stanford University Medical Center, Stanford, CA 94305, USA

Avascular Necrosis of Bone in Cushing's Syndrome

Radiology ◽  
1964 ◽  
Vol 83 (6) ◽  
pp. 1068-1070 ◽  
Author(s):  
Samuel H. Madell ◽  
Leonard M. Freeman
Keyword(s):  
Avascular Necrosis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Avascular Necrosis Of Bone

scholarly journals Is diabetes in Cushing's syndrome only a consequence of hypercortisolism?

2014 ◽  
Vol 170 (2) ◽  
pp. 311-319 ◽  
Author(s):  
Carla Giordano ◽  
Valentina Guarnotta ◽  
Rosario Pivonello ◽  
Marco Calogero Amato ◽  
Chiara Simeoli ◽  
...  
Keyword(s):  
Insulin Sensitivity ◽  
Glucose Tolerance ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Visceral Adiposity Index ◽  
Metabolic Complications ◽  
Family History Of Diabetes ◽  
Pituitary Disease ◽  
History Of ◽  
Adrenal Disease

ObjectiveDiabetes mellitus (DM) is one of the most frequent complications of Cushing's syndrome (CS). The aim of this study was to define the changes in insulin sensitivity and/or secretion in relation to glucose tolerance categories in newly diagnosed CS patients.DesignCross-sectional study on 140 patients with CS.MethodsA total of 113 women (80 with pituitary disease and 33 with adrenal disease, aged 41.7±15.7 years) and 27 men (19 with pituitary disease and eight with adrenal disease, aged 38.1±20.01 years) at diagnosis were divided according to glucose tolerance into normal glucose tolerance (CS/NGT), impaired fasting glucose and/or impaired glucose tolerance (CS/prediabetes), and diabetes (CS/DM) groups.ResultsSeventy-one patients had CS/NGT (49.3%), 26 (18.5%) had CS/prediabetes and 43 (30.8%) had CS/DM. Significant increasing trends in the prevalence of family history of diabetes (P<0.001), metabolic syndrome (P<0.001), age (P<0.001) and waist circumference (P=0.043) and decreasing trends in HOMA-β (P<0.001) and oral disposition index (DIo) (P<0.002) were observed among the groups. No significant trends in fasting insulin levels, area under the curve for insulin (AUCINS), Matsuda index of insulin sensitivity (ISI-Matsuda) and visceral adiposity index were detected.ConclusionsImpairment of glucose tolerance is characterized by the inability of β-cells to adequately compensate for insulin resistance through increased insulin secretion. Age, genetic predisposition and lifestyle, in combination with the duration and degree of hypercortisolism, strongly contribute to the impairment of glucose tolerance in patients with a natural history of CS. A careful phenotypic evaluation of glucose tolerance defects in patients with CS proves useful for the identification of those at a high risk of metabolic complications.

scholarly journals Cushing’s syndrome due to primary pigmented nodular adrenal disease in two brothers with Carney complex

2020 ◽  
Vol 26 (3) ◽  
pp. 155-158
Author(s):  
Bhawna Attri ◽  
Anshita Aggarwal ◽  
Sahil Mattoo ◽  
Bindu Kulshreshtha
Keyword(s):  
Cushing’S Syndrome ◽  
Carney Complex ◽  
Cushing's Syndrome ◽  
Adrenal Disease
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