Adrenal insufficiency is caused by either primary adrenal failure or by hypothalamic-pituitary impairment of the corticotropic axis which is predominantly due to long-term glucocorticoid treatment or by pituitary disease. Primary adrenal failure is a rare disease that is life-threatening when overlooked whle adrenal failure secondary to hypothalamo-pituitary disease is a common clinical problem that has serious impact on the quality of life. Recent data suggests that the health-related quality of life in many patients with adrenal insufficiency is more severely impaired than previously thought and that current hormone replacement strategies are insufficient to prevent increased morbidity and mortality due to the disease. Therefore, the optimization and careful monitoring of glucocorticoid and mineralocorticoid replacement therapy remains one of the most challenging tasks in endocrinology. Leaving treatment aside, diagnosis of adrenal insufficiency is also challenging, because the main presenting symptoms such as fatigue, anorexia, and weight loss are non-specifically associated with many other common ailments, delaying a correct diagnosis. Some pitfalls in diagnostic work-up, particularly in the identification of secondary adrenal insufficiency, make the diagnosis even more difficult. Despite the difficulties, the complications associated with adrenal insufficiency make the rapid diagnosis and proper management of adrenal failure are important tasks for all physicians. We review here current standards for clinical assessment, diagnosis and treatment of adrenal insufficiency in light of recent research findings.
Addison Disease ; Adrenal Cortex Function Tests ; Adrenal Insufficiency ; Anorexia ; Endocrinology ; Fatigue ; Glucocorticoids ; Humans ; Light ; Pituitary Diseases ; Quality of Life ; Rare Diseases ; Weight Loss ; Axis, Cervical Vertebra

A 39-year-old woman presented with a 20 day history of recurrent hypoosmolar hyponatremia. Because her volume status seemed to be normal, the most suspected causes of her hyponatremia were adrenal insufficiency and hypothyroidism. Endocrinologic examination, including a combined pituitary function test, showed TSH and ACTH deficiency without GH deficiency, and hyperprolactinemia was also present. Sella MRI showed a pituitary mass, stalk thickening and loss of the normal neurohypophysial hyperintense signal on the T1 weighted image. Pathologic exam demonstrated granulomatous lesions and Langhans' multinucleated giant cells with inflammatory cell infiltration. After high dose methylprednisolone pulse therapy (1 g/day for 3 days) with subsequent prednisolone and levothyoxine replacement, there was no more recurrence of the hyponatremia. The sella MRI on the 6th month showed decreased mass size, narrowed stalk thickening and the reappearance of the normal neurohyphophysial hyperintense signal. She is currently in a good general condition and is receiving hormone replacement therapy.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Female ; Giant Cells ; Hormone Replacement Therapy ; Humans ; Hyperprolactinemia ; Hyponatremia ; Hypothyroidism ; Methylprednisolone ; Pituitary Function Tests ; Prednisolone ; Recurrence

Most of the patients with hemorrhagic fever with renal syndrome (HFRS) by Hantaviruses recover completely. However, the prevalence of hypopituitarism as sequel of HFRS may be approximately 10%. However, it is rare for hyponatremia to present as a manifestation of hypopituitarism due to HFRS. A 42-year-old Asian woman presented with anorexia, nausea, and vomiting. She had a history of HFRS 7 years ago. During her first hospital admission, her serum sodium was 103 mmol/L. The rapid adrenocorticotropic hormone (ACTH) stimulation test showed no abnormal findings suggesting the possibility of adrenal insufficiency. After she underwent fluid therapy for 5 days, her serum sodium level was slightly increased to 131 mmol/L, and her clinical symptoms were improving. The next day after she was discharged, she suffered from the same symptoms and was admitted to our hospital again. On the day of her second admission, her serum sodium was 117 mmol/L. A combined pituitary function test showed diminished function of the anterior pituitary gland, which led to secondary adrenal insufficiency. Magnetic resonance imaging findings of her sella were compatible with empty sella turcica. After the replacement of prednisolone, her serum sodium levels returned to normal. Pituitary dysfunction should be included in the differential diagnosis of severe hyponatremia, especially in patients who suffered HFRS. This case also suggests that the rapid ACTH stimulation test has limitations with regard to the differential diagnosis of primary or secondary adrenal insufficiency.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Anorexia ; Asian Continental Ancestry Group ; Diagnosis, Differential ; Empty Sella Syndrome ; Female ; Fluid Therapy ; Hantavirus ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Hyponatremia ; Hypopituitarism ; Magnetic Resonance Imaging ; Nausea ; Pituitary Function Tests ; Pituitary Gland, Anterior ; Prednisolone ; Prevalence ; Sodium ; Vomiting

OBJECTIVETo study the pathology, diagnostic and therapeutic method of primary pigmented nodular adrenocortical disease (PPNAD).

METHODSThe data of 5 cases of PPNAD were analyzed retrospectively. Among the 5 cases, 2 were male and 3 were female. The range of age was from 12 to 53 years. All the 5 cases had symptoms of Cushing syndrome. The diagnose depended on the results of endocrine exams, ultrasound, CT, MR and pathological reports. All patients received operation of unilateral adrenalectomy. The therapeutic effects were determined by post-operative results, which concluded clinical symptoms and endocrine exams.

RESULTSThe follow-up time was from 4 months to 3 years. All patients' symptoms of Cushing syndrome were relieved in 6 months after operation. The endocrine exam was normal in one case and obvious improved in the other four cases. Up to now, one patient drop out of the follow-up, the other 4 cases had no evidence of recurrence.

CONCLUSIONPPNAD is a rare type of Cushing syndrome. Diagnose depends on endocrine exams and pathological results. Operation is the effective method for the disease.

Adolescent ; Adrenalectomy ; Adult ; Child ; Cushing Syndrome ; diagnosis ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pituitary-Adrenal Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed