Presentation
The first patient, a ten-year-old with XX genotype, reared as a male, presented with resistant hypokalaemia and hypertension. The second patient, a 23-year-old with XY genotype, presented with bilateral adrenal masses and resistant hypertension. [ncbi.nlm.nih.gov]
Entire Body System
- Resistant Hypertension
The first patient, a ten-year-old with XX genotype, reared as a male, presented with resistant hypokalaemia and hypertension. The second patient, a 23-year-old with XY genotype, presented with bilateral adrenal masses and resistant hypertension. [ncbi.nlm.nih.gov]
Cardiovascular
- Hypotension
After hypotensive pretreatment, a left adrenalectomy was performed, resulting in normalization of corticoadrenal function, blood pressure, Cushing features and growth rate. Histopathology confirmed PPNAD. [ncbi.nlm.nih.gov]
Post-operative concerns included acute adrenal insufficiency, hypoglycaemia, hypotension and hyperkalemia, which were successfully managed in ICU. Patient was then given oral corticosteroids. [ijaweb.org]
His hospital course was complicated with tachycardia and hypotension, which was successfully treated with aggressive fluid resuscitation and ileus treated with bowel rest. His hydrocortisone was tapered during the stay based on his clinical status. [edmcasereports.com]
Face, Head & Neck
- Neck Swelling
RESULTS: A 59-year-old man presented with nasal congestion and neck swelling and was noted to have hypokalemia, hypertension, and hyperglycemia. A nasal biopsy demonstrated a poorly differentiated carcinoma with neuroendocrine features. [ncbi.nlm.nih.gov]
Neurologic
- Cranial Neuropathy
Two patients developed new pituitary dysfunction and no patient developed cranial neuropathy or visual deficit after GK SRS. [ncbi.nlm.nih.gov]
Urogenital
- Secondary Amenorrhea
A 21-year-old woman was referred to Hamamatsu University School of Medicine University Hospital complaining of a deepening voice, hirsutism and secondary amenorrhea. [ncbi.nlm.nih.gov]
Workup
Tumor marker CA-125 was elevated and additional ovarian cancer staging workup confirmed stage IIIC due to one lymph node positive for carcinoma. [ncbi.nlm.nih.gov]
Preoperative workup revealed hypokalemia, anaemia, hypertension and hyperglycemia. She was posted for laparoscopic bilateral adrenalectomy. [ijaweb.org]
No hormonal workup was performed by the surgical service evaluating the patient on initial presentation. Treatment The differential diagnosis of these masses included benign lesions such as lipoma, teratoma, or myelolipoma. [edmcasereports.com]
Biopsy
- Liver Biopsy
One patient underwent a simultaneous wedge liver biopsy for a right lobar lesion. There were no complications. Each patient resumed a regular diet on the first postoperative day. [ncbi.nlm.nih.gov]
Treatment
Abstract Up to one-third of subjects who have had bilateral adrenalectomy for treatment of Cushing's disease will develop Nelson's syndrome. [ncbi.nlm.nih.gov]
Prognosis
Prognosis after Bilateral Adrenalectomy: The prognosis for a positive end result following bilateral adrenalectomy is good. Recovery from Bilateral Adrenalectomy: The total recovery time for bilateral adrenalectomy is three to six weeks. [orangecountysurgeons.org]
Metastatic non-small-cell lung cancer is a common condition with a dismal prognosis. Although palliative chemotherapy improves survival and quality of life, nearly all patients die of progressive disease. [ncbi.nlm.nih.gov]
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]
Prognosis The prognosis is good, providing there is early recognition. Co-ordination between surgeons and radiotherapists is important. Postoperative aftercare, with adequate long-term monitoring and replacement of hormones, is vital. [patient.info]
Etiology
The patient records were analyzed to obtain patient demographics, disease etiology, surgical approach, operating room information, postoperative complications (30 days), hospital length of stay (LOS), and follow-up information. [ncbi.nlm.nih.gov]
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]
Some authors suggested that CS should be included in the differential diagnoses of certain high-risk patient populations, including patients who present with diabetes mellitus, hypertension, and early-onset osteoporosis. 85 Quality of Life CS of any etiology [endocrinefacts.org]
Epidemiology
Summary Epidemiology Prevalence of endogenous CS is estimated at 1/26,000 and AIMAH represents less than 1% of the cases. [orpha.net]
Epidemiology [ 1 ] Nelson's syndrome is rare. [patient.info]
In patients with a benign adrenal adenoma, SMR=1.90. * Graversen et al. 2012 64 Spain, N=49, 1975-1992 Epidemiological study Overall, SMR=3.8. [endocrinefacts.org]
Pathophysiology
This Second Edition has been thoroughly updated with the latest information on pathophysiology, treatment, and drug dosing. New chapters cover obesity, vitamin D deficiency, autoimmune polyendocrine syndromes, and endocrine disorders in HIV/AIDS. [books.google.com]
The pathophysiology of Nelson's syndrome and the factors leading to its development are poorly understood. [patient.info]
Prevention
After bilateral adrenalectomy, adrenal insufficiency must be prevented by hormonal substitution, please see section adrenal insufficiency. [urology-textbook.com]
Laparoscopic bilateral adrenalectomy was offered to both patients to prevent accumulation of adrenal steroid precursors; this would likely restore regular 4-week menstrual cycles, decrease virilization, and decrease dosage of replacement therapy. [pediatrics.aappublications.org]
Prevention Bilateral adrenalectomy is said to carry an acceptable risk but follow-up should include awareness of Nelson's syndrome. [ 14 ] Monitoring of ACTH level and pituitary MRI are recommended 3-6 months after surgery and regularly thereafter. [ [patient.info]
Prevention of venous thromboembolism: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition) Chest. 2008; 133 (6 Suppl):381S–453S. [ PubMed ] [ Google Scholar ] 38. [ncbi.nlm.nih.gov]
The present studies reinforce the necessity for preventing sustained high levels of ACTH. This is particularly important because many patients have adrenal remnants that are capable of responding to ACTH. [caresfoundation.org]