scholarly journals Pediatric Adrenal Insufficiency: Diagnosis, Management, and New Therapies

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Sasigarn A. Bowden ◽  
Rohan Henry
Keyword(s):  
Adrenal Insufficiency ◽  
Adrenal Cortex ◽  
Clinical Management ◽  
Current Treatment ◽  
Treatment Recommendations ◽  
Morbidity And Mortality ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
New Therapies ◽  
Timely Diagnosis

Adrenal insufficiency may result from a wide variety of congenital or acquired disorders of hypothalamus, pituitary, or adrenal cortex. Destruction or dysfunction of the adrenal cortex is the cause of primary adrenal insufficiency, while secondary adrenal insufficiency is a result of pituitary or hypothalamic disease. Timely diagnosis and clinical management of adrenal insufficiency are critical to prevent morbidity and mortality. This review summarizes the etiologies, presentation, and diagnosis of adrenal insufficiency utilizing different dynamic hormone testing and describes current treatment recommendations and new therapies.

Pazopanib-associated secondary adrenal insufficiency in a patient with malignant solitary fibrous tumor

2021 ◽  
pp. 107815522110160
Author(s):  
Muhammed Muhiddin Er ◽  
Murat Araz ◽  
Meryem Karabacak ◽  
Muzaffer Uğraklı ◽  
Melek Karakurt Eryılmaz ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Solitary Fibrous Tumor ◽  
Single Case ◽  
Soft Tissue Sarcomas ◽  
Pituitary Hormones ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Hypoglycemic Attack ◽  
Fibrous Tumor

Introduction Pazopanib is an agent that is being successfully used in soft tissue sarcomas. Some endocrine side effects may develop during pazopanib treatment. Here, we presented a case diagnosed with secondary adrenal insufficiency while being investigated for etiology of hypoglycemia which developed after pazopanib. Case report A 69-year-old male patient was operated in June 2019 due to a lung mass 26 × 18 × 10 cm in size. Pathological diagnosis revealed a solitary fibrous tumor with malignant behavior. The patient received three lines of chemotherapy. After pazopanib treatment, a hypoglycemic attack was reported. Management and outcome: Blood cortisol and ACTH (Adrenocorticotropic hormone) levels were not increased at the time of the hypoglycemic attack, and levels of other pituitary hormones were found to be normal. Electrolyte levels were in normal range. Since the counteracting hormone did not reach a sufficient level, it was considered secondary adrenal insufficiency. Hypoglycemic attacks did not occur during follow-up while taking steroid therapy and pazopanib. Discussion A single case of primary adrenal insufficiency has been reported in the literature. We here present a case who developed hypoglycemia after pazopanib and was diagnosed with drug-associated secondary adrenal insufficiency. When hypoglycemia develops during pazopanib treatment, we must be aware of adrenal insufficiency.

Corticosteroid replacement: getting it right

1990 ◽  
Vol 28 (18) ◽  
pp. 71-72
Keyword(s):  
Adrenal Insufficiency ◽  
Cortisone Acetate ◽  
Adrenal Failure ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Acth Deficiency ◽  
Mineralocorticoid Activity

Glucocorticoid replacement is needed in primary and secondary adrenal insufficiency. Cortisol (hydrocortisone) is the most physiologically appropriate drug; prednisolone has little mineralocorticoid activity, and cortisone acetate suffers variable absorption and hepatic conversion to cortisol.1 Patients with primary adrenal insufficiency usually need additional mineralocorticoid replacement with fludrocortisone once a day. Patients with secondary adrenal failure (ACTH deficiency due to pituitary/hypothalamic disease) still produce aldosterone and so do not need fludrocortisone.

scholarly journals Primary adrenal insufficiency replacement therapy

2000 ◽  
Vol 46 (3) ◽  
pp. 31-45
Author(s):  
V. V. Fadeev ◽  
G. A. Melnichenko
Keyword(s):  
Heart Failure ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Adrenal Cortex ◽  
Adrenal Glands ◽  
Pathological Process ◽  
Clinical Syndrome ◽  
Primary Adrenal Insufficiency ◽  
Adrenal System ◽  
System 2

Adrenal insufficiency is a clinical syndrome caused by insufficient secretion of hormones by the adrenal cortex, which is the result of a malfunction of one or more parts of the hypothalamic-pituitary-adrenal system [2]. Primary chronic adrenal insufficiency (1-CNI) develops as a result of the destruction of more than 90% of the cortex of both adrenal glands by a pathological process. The main causes of 1-CNN are currently autoimmune (80–85%) and tuberculosis (5–10%) destruction of the adrenal cortex [2, 3]. 1-CNN of the indicated etiology is better known as "Addison's disease." 1-CNN is a relatively rare disease (40-110 new cases per 1 million people per year) [2, 3], but it is of considerable importance in the practice of endocrinology. Without exception, all patients with a diagnosis of 1-chronic heart failure need lifelong replacement therapy with corticosteroids (CS), which will be discussed in this work.

scholarly journals Type 4 Renal Tubular Acidosis (RTA) Induced by Spironolactone Use in Secondary Adrenal Insufficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A162-A163
Author(s):  
Claudia Villatoro Santos ◽  
Lisa De Rose ◽  
Nikoletta Proudan
Keyword(s):  
Adrenal Insufficiency ◽  
Renal Tubular Acidosis ◽  
The Elderly ◽  
Altered Mental Status ◽  
Secondary Adrenal Insufficiency ◽  
Acth Stimulation ◽  
Primary Adrenal Insufficiency ◽  
Inhaled Steroids ◽  
Baseline Cortisol ◽  
Renal Tubular

Abstract Type 4 RTA is caused by either decreased aldosterone production or resistance. Primary adrenal insufficiency results in decreased aldosterone whereas spironolactone can cause aldosterone resistance1. We present a case of spironolactone-induced type 4 RTA in a patient with suspected primary adrenal insufficiency. A 70-year-old female with liver cirrhosis on spironolactone and chronic bronchitis on inhaled steroids (ICS) presented for altered mental status. Laboratories showed hyponatremia 131 mmol/L (135–145 mmol/L) and hyperammonemia 113 µmol/L (11–51 µmol/L). She was successfully treated with oral lactulose for hepatic encephalopathy. However, on day 3, she developed worsening hyponatremia (126 mmol/L) and hyperkalemia 5.8 mmol/L (3.5–5.4 mmol/L). Spironolactone was discontinued, and hyperkalemia improved after medical treatment. Nonetheless, hyperkalemia recurred with worsening hyponatremia (125 mmol/L), hypoglycemia (57 mg/dL), and mild non-anion gap metabolic acidosis without other signs or symptoms of adrenal insufficiency. On day 5, her morning cortisol was 1.5 µg/dL (5–20 µg/dL), with ACTH 11 pg/mL (6–70 pg/mL). Her hyperkalemia persisted (6.3–6.8 mmol/L), and she was started on oral patiromer. Due to suspected adrenal insufficiency, she received dexamethasone 10 mg daily, and endocrinology was consulted. On day 7, an ACTH stimulation test (250 µg IV) showed a baseline ACTH <3 pg/mL, baseline cortisol 0.7 µg/dL (3–15 µg/dL), cortisol 30 minutes 9.9 µg/dL, and 60 minutes 12.2 µg/dL, consistent with incomplete response attributed to the supraphysiologic dexamethasone versus chronic ICS. On day 8, endocrinology discontinued dexamethasone and enoxaparin, and started hydrocortisone 10 mg orally in AM and 5 mg in PM. Aldosterone (measured at day 6) was 7.2 ng/dL (<= 31.0 ng/dL), renin activity 3.1 ng/mL/hr (0.5–4.0 ng/mL/hr), and aldosterone/renin ratio 2.3 (<= 25) consistent with hyporeninemic hypoaldosteronism since aldosterone and renin were inappropriately normal for the hyperkalemia. Repeat cosyntropin test on day 11 showed low ACTH (3 pg/mL), low baseline cortisol 1.1 µg/dL, cortisol 30 minutes 7.9 µg/dL, and 60 minutes 11.2 µg/dL, consistent with secondary adrenal insufficiency, ascribed to chronic ICS. Potassium level normalized seven days after spironolactone discontinuation, related to its approximate duration of action1. The patient was discharged with hydrocortisone 10 mg daily, and spironolactone was permanently discontinued. Spironolactone use can result in type 4 RTA due to aldosterone resistance and mimic mineralocorticoid deficits characteristic of primary adrenal insufficiency. 1. O’Connell JE, Colledge NR. Type IV renal tubular acidosis and spironolactone therapy in the elderly. Postgrad Med J. 1993;69(817):887–889.

scholarly journals Careful Evaluation of Cosyntropin Dose in the Diagnosis of Adrenal Insufficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A122-A123
Author(s):  
Clio Musurakis ◽  
Solab Chitrakar ◽  
Randa Eldin Sharag ◽  
Ekta Shrestha ◽  
Gauri Pethe ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Gold Standard ◽  
Low Dose ◽  
Treatment Plan ◽  
Stimulation Test ◽  
Diagnostic Process ◽  
High Dose ◽  
Secondary Adrenal Insufficiency ◽  
Acth Stimulation ◽  
Primary Adrenal Insufficiency

Abstract Introduction: The use of the 250μg cosyntropin dose or otherwise called high-dose ACTH test is the gold standard test for diagnosis of primary adrenal insufficiency. The 1μg dose test or the low-dose test is mostly reserved for diagnosis of secondary adrenal insufficiency. Careful consideration of the results produced during the diagnostic process is imperative to avoid mislabeling of patients with a disease that requires lifelong treatment. Case Report: This is the case of a 45-year-old female with a history of asthma and psoriasis who presented with emesis. Home medications included monthly TNF-alpha inhibitor injections for psoriasis, triamcinolone acetonide topical spray and budesonide-formoterol inhaler. On admission, she also had nausea, chills and diaphoresis, as well as palpitations, lightheadedness, and shortness of breath. When she arrived at the ER, vitals were remarkable for low blood pressure. Labs were unremarkable except for CMP concerning for anion gap metabolic acidosis, hyponatremia, and hypokalemia. A random serum cortisol was 6.4 mcg/dL, which was relatively low. ACTH was within normal range. Due to concern for adrenal insufficiency, a 1μg cosyntropin test was performed which showed a peak cortisol concentration of less than 18 mcg/dL. As the response was assessed as suboptimal, endocrinology was consulted to offer a treatment plan for steroids. However, the test was repeated using the gold standard 250μg cosyntropin dose and the patient then showed an adequate response and she was not started on steroids. Conclusions: This is a case that demonstrates how the 250 μg ACTH or high-dose stimulation test should be used for diagnosis of primary adrenal insufficiency (AI), as it is the gold standard. The 1 μg ACTH or low-dose stimulation test can be used for diagnosis of primary AI but only when the high dose test is not available. On the other hand, the 1 μg ACTH stimulation test has been shown to be more sensitive than the 250 μg test in diagnosing secondary adrenal insufficiency. When using the most appropriate test correctly, the clinician can only then offer the patient the best treatment strategies. Our patient did not require chronic replacement therapy. The steroids in this case could have harmed the patient as chronic administration could cause adrenal gland suppression.

scholarly journals Challenging Diagnosis of Addison’s Disease Presenting with Adrenal Crisis

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Ni Wayan Wina Dharmesti ◽  
Made Ratna Saraswati ◽  
Ketut Suastika ◽  
Wira Gotera ◽  
I Made Pande Dwipayana
Keyword(s):  
Adrenal Insufficiency ◽  
Adrenal Cortex ◽  
Addison’S Disease ◽  
Adrenal Crisis ◽  
Addison's Disease ◽  
Rapid Progression ◽  
Circulatory Collapse ◽  
Emergency Ward ◽  
Primary Adrenal Insufficiency ◽  
Life Threatening

Primary adrenal insufficiency, also known as Addison’s disease, is a rare but potentially fatal condition resulting from the failure of the adrenal cortex to produce glucocorticoid and/or mineralocorticoid hormones. Unfortunately, the clinical manifestation of primary adrenal insufficiency is not specific and often progresses insidiously, resulting in late diagnosis, or in severe cases, life-threatening circulatory collapse. Adrenal insufficiency should be considered in patients with unexplained vascular collapse. We report the case of a woman who presented to the emergency ward with unexplainable shock that was later diagnosed as adrenal crisis due to Addison’s disease. The presence of hyperpigmentation in patients with rapid progression of adrenal insufficiency suggests the diagnosis of Addison’s disease presenting with adrenal crisis.

scholarly journals Difficulties in diagnosis of addisonian crisis in primary adrenal insufficiency

2016 ◽  
Vol 97 (5) ◽  
pp. 797-800
Author(s):  
V N Oslopov ◽  
J V Oslopova ◽  
M A Makarov ◽  
E N Khasanov
Keyword(s):  
Adrenal Insufficiency ◽  
Adrenal Cortex ◽  
Correct Diagnosis ◽  
Hemodynamic Instability ◽  
Left Ventricular ◽  
Infusion Therapy ◽  
Stressful Situation ◽  
Primary Adrenal Insufficiency ◽  
Recovery From Anesthesia ◽  
High Doses

The observation of a rare case of Addisonian crisis, which developed in 28-year old patient with asymptomatic hypocorticoidism in a stressful situation and was fatal, is described. In patient with left-sided interrupted tubal pregnancy after recovery from anesthesia after a successful laparoscopic tubectomy, shock blood pressure lowering happened, an acute left ventricular heart failure occurred. Addisonian crisis developed under stress conditions (pregnancy, rupture of the fallopian tube, culdocentesis, anesthesia, performed surgery) amid the previously undiagnosed primary adrenal insufficiency due to congenital hypoplasia of the adrenal cortex. During the autopsy there was found hypoplasia of fasciculate and reticular zones of adrenal cortex, which formed only about 40%, while normally form 85% of the adrenal cortex. Under stress (in the presence of normal adrenal glands) fasciculate zone must significantly increase in size (and account for more than 75%), while in patient who was under the severe stress they accounted for only 10-20% of the adrenal cortex. Adrenal insufficiency was not recognized. The patient has no hyperpigmentation and hyperkalemia, which confirms the conclusion of primary adrenal insufficiency. Therapeutic measures were reduced to the administration of low-dose prednisone, sympathomimetic drugs and liquid. Due to the possible existence of forms of the adrenal insufficiency with mild manifestations, there should be an awareness in terms of possible hypocorticoidism in case of development of hemodynamic instability in response to stress, despite intensive infusion therapy and administration of adrenergic drugs. In such cases, it is necessary to administer, perhaps ex juvantibus, high doses of glucocorticoids, which at the correct diagnosis match «as the key to the lock», and at the same time are safe for use. In the treatment of shock of various etiologies, there is a need for wider use of glucocorticoid therapy using high doses of hormones.

scholarly journals Perioperative Severe Hypotension in a Patient with Multiple Endocrine Neoplasia Type IIb and Bilateral Adrenalectomies: Time to Review the Evidence for Stress Dose Steroids

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Jens Tan ◽  
Acsa Zavala ◽  
Katherine B. Hagan ◽  
Antoinette Van Meter ◽  
Uduak Ursula Williams ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Multiple Endocrine Neoplasia ◽  
Recent Literature ◽  
Multiple Endocrine Neoplasia Type ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Severe Hypotension ◽  
Stress Dose

Multiple endocrine neoplasia type IIb (MEN IIb) is an endocrine disorder which can manifest with tumors such as pheochromocytomas and neuromas. We present the case of a patient with MEN IIb, after bilateral adrenalectomies, on maintenance steroid replacement, who underwent a neuroma resection and developed severe hypotension. There is persistent controversy regarding the general administration of perioperative “stress dose” steroids for patients with adrenal insufficiency. While the most recent literature suggests that stress dose steroids are unnecessary for secondary adrenal insufficiency, the rarer form of primary adrenal insufficiency always requires supplemental steroids, specifically hydrocortisone, when undergoing surgical procedures.

scholarly journals Adrenal Insufficiency

2016 ◽  
pp. 43-48
Author(s):  
SM Ashrafuzzaman ◽  
Muhammad Abdur Rahim
Keyword(s):  
Weight Loss ◽  
Adrenal Insufficiency ◽  
Adrenal Cortex ◽  
High Index ◽  
Electrolyte Imbalance ◽  
Adrenal Crisis ◽  
Adrenal Androgen ◽  
Primary Adrenal Insufficiency ◽  
Short Acting ◽  
High Index Of Suspicion

Adrenal insufficiency means hypo-function of the adrenal cortex, usually glucocorticoid, may be associated with mineralocorticoid and rarely adrenal androgen. It can be categorized into two types: Primary adrenal insufficiency, or Addison’s disease (cortisol low and ACTH high) or secondary/ tertiary adrenal insufficiency due to pituitary or hypothalamic disorders (both cortisol and ACTH are low). Identification of etiology is very important for plan of treatment. The reported incidence of adrenal insufficiency varies greatly depending on the population studied, the test and cut off levels used, and the cause. Many patients may present with adrenal crisis, some of them have subtle symptoms, hyperpigmentation, hypotension, electrolyte imbalance, shock etc. Many of them have iatrogenic adrenal insufficiency with features of Cushing’s syndrome. So, the manifestations of adrenal insufficiency are numerous and nonspecific, and clinicians should have a high index of suspicion and be alert to see important diagnostic clues, such as hyponatremia, hyperkalemia, and hypotension (that are refractory to fluids and vasopressors), diarrhea, weight loss, vomiting, fever etc. without any clear causation. Treatment of adrenal insufficiency is usually life-long, of course based on etiology. Short acting glucocorticoid is better, like hydrocortisone. Patients may be treated with prednisolone. Patients should be provided a “steroid card”. Sometimes it is essential to take patients under “withdrawal protocol”.Birdem Med J 2015; 5(1) Supplement: 43-48

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