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Adrenal Cortex Disease

Disease of Adrenal Cortex


Presentation

  • Clinical description The disease presents a bimodal age distribution with a rare subset presenting in the first years of life, particularly associated to McCune-Albright syndrome (MAS; see this term).[orpha.net]
  • Depends on the course of the disease: acute stage: the patient presents with fever, back pain, hypotension, weakness chronic stage : progressive lethargy, weakness, cutaneous pigmentation, weight loss Laboratory data chemistry: hyponatremia, hyperkalemia[radiopaedia.org]
  • These two case reports highlight sudden death among patients with differing presentations of Cushing’s disease and syndrome.[intechopen.com]
  • These different diseases present in different forms and vary widely with no two patients presenting with the same symptoms.[drlam.com]
  • Dominant Symptoms and Signs Even though a majority of adrenal adenomas do not present with any symptoms, their growth may press other organs and result in abdominal pain and weight loss.[news-medical.net]
High Fever
  • The patient was discharged, but she persisted in having a high fever (40 C) and vomiting. She returned to the same hospital and was admitted to the Department of Medicine.[intechopen.com]
Cyanosis
  • A few hours later, she developed cyanosis and dyspnea and died suddenly. At forensic autopsy, performed 7 days after death, macroscopic examination revealed pulmonary edema and bilateral adrenal hemorrhage ( Figures 3 – 5 ). Figure 3.[intechopen.com]
Purpura
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized[nejm.org]
Withdrawn
  • Recruiting NCT02637531 Phase 1 IPI-549;Nivolumab 43 Possible New Therapy for Advanced Cancer Terminated NCT01832974 Phase 1 IL-13-PE 44 Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor Withdrawn[malacards.org]
Encephalopathy
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized[nejm.org]

Workup

  • Anytime anyone presents with fatigue and low energy state, a complete cardiac, hormonal, hematological, oncological and metabolic workup is needed. When the result is normal and out of the box a different diagnosis needs to be considered.[drlam.com]
  • Those with these symptoms deserve compassion, a thorough workup looking for recognized (but often overlooked) medical causes, and a thoughtful treatment plan.[verywell.com]
  • Pediatric Adrenal Insufficiency (Addison Disease) Workup. Medscape Reference. Available online at . Accessed February 2017. Johannsson G, et al. Adrenal Insufficiency: Review of Clinical Outcomes With Current Glucocorticoid Replacement Therapy.[labtestsonline.org]

Treatment

  • Treatment of Cushing's Syndrome Obviously, the treatment of this disease depends on the cause. Pituitary tumors are usually removed surgically and often treated with radiation therapy. Neurosurgeons and some ENT surgeons specialize in these tumors.[endocrineweb.com]
  • (FIRM-ACT) Completed NCT00094497 Phase 3 Etoposide;Doxorubicin;Cisplatin;Streptozotocin;Mitotane 4 Efficacy of Adjuvant Mitotane Treatment (ADIUVO) Recruiting NCT00777244 Phase 3 MITOTANE 5 Mitotane With or Without Cisplatin and Etoposide After Surgery[malacards.org]
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized[nejm.org]
  • What are the treatments for adrenal gland disorders? The treatment for adrenal gland disorders depends on the specific disorder or the specific cause of the disorder. For example: The treatment for Cushing’s Syndrome depends on the cause.[nursinglink.monster.com]
  • Management and treatment Treatment can be medical if aberrant adrenal receptors are identified and can be blocked (i.e. with propranolol, somatostatin or GnRH analogs).[orpha.net]

Prognosis

  • Prognosis Prognosis after treatment is good but quality of life may be affected due to the long-term effects of hypercortisolemia. Without treatment the disease is life-threatening as in the case of manifest hypercortisolism.[orpha.net]
  • […] adrenalitis): enlargement of both adrenal glands, with necrotic centers and peripheral enhancing rims chronic : both adrenal glands appear small and atrophic, associated with calcifications ( adrenal calcification ) in granulomatous adrenalitis Treatment and prognosis[radiopaedia.org]
  • In spite of treatment with sulphonamides, antibiotics, and steroids, the prognosis is still extremely poor.[intechopen.com]

Etiology

  • Etiology The exact etiology is unknown but the adrenal overgrowth seen in AIMAH may be due to the expression of aberrant membrane receptors found in the adrenal cortex that regulate cortisol secretion and which are stimulated by gastric inhibitory polypeptide[orpha.net]
  • Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common etiology overall.[radiopaedia.org]
  • ETIOLOGY AND RISK FACTORS• Tuberculosis (TB) :TB is an infection which usually affects the lungs.[slideshare.net]
  • Depending on the etiologies of adrenal insufficiency, clinical manifestations may be severe or mild, have gradual or sudden onset, begin in infancy or childhood/adolescence.[raysahelian.com]
  • Tuberculosis and other infections involving adrenal glands About 10% of Addison’s disease cases have an infectious etiology [ 8 ].[intechopen.com]

Epidemiology

  • Summary Epidemiology Prevalence of endogenous CS is estimated at 1/26,000 and AIMAH represents less than 1% of the cases.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • The main causes of sudden death due to adrenal gland pathology will be examined, paying particular attention to the pathophysiology of sudden death, macroscopic and microscopic characteristics and their correlation with clinical features.[intechopen.com]

Prevention

  • […] the immune response, especially cell-mediated immune responses . [ Discussion of mechanism ] For this reason glucocorticoids are widely used in therapy: to reduce the inflammatory destruction of rheumatoid arthritis and other autoimmune diseases to prevent[biology-pages.info]
  • In the same year, Evans was able to prevent this by pituitary extraction.[endocrinesurgeon.co.uk]
  • Destruction or removal of the pituitary has the opposite effect, stopping ACTH secretion, preventing stimulation of the adrenal cortex, and thus leading to adrenal failure.[daviddarling.info]
  • To sum up, it is easy to prevent the disorders of the suprarenal glands. Firstly, you should try to avoid the excessive use of steroid drugs. Moreover, go for a frequent examination of the adrenal glands.[organsofthebody.com]
  • There is no evidence that treatment can prevent this autoimmune attack, which may also affect other organs in the same person.[netdoctor.co.uk]

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