scholarly journals Corticosteroids Replacement in a Patient With Panhypopituitarism and COVID-19 Infection

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A578-A579
Author(s):  
Gowri Karuppasamy ◽  
Zaina Abdelhalim Alamer ◽  
Samman Rose ◽  
Ibrahim Abdulla Al-Janahi
Keyword(s):  
Pituitary Adenoma ◽  
Adrenal Insufficiency ◽  
Hormone Replacement ◽  
Hypogonadotropic Hypogonadism ◽  
Adrenal Crisis ◽  
High Dose ◽  
Pituitary Hormone ◽  
Secondary Hypothyroidism ◽  
Stress Dose

Abstract Background: Hypopituitarism refers to complete or partial insufficiency of pituitary hormone secretion and patients require lifelong hormone replacement. Those with ACTH deficiency rely on exogenous glucocorticoids and at times of intercurrent illness require stress doses to prevent an adrenal crisis. The benefits and adverse effects of corticosteroids for treatment of COVID-19 pneumonia are currently under investigation. We report our experience in a patient with COVID-19 pneumonia who received high dose corticosteroids for panhypopituitarism. Clinical Case: A 51-year-old man presented with one-week history of fever and generalized weakness. He had been diagnosed with a non-functional pituitary macro-adenoma causing panhypopituitarism 1 year ago when he developed generalized tonic-clonic seizures. He underwent trans-sphenoidal resection of the pituitary adenoma. However, he then discontinued his hormonal therapy and was lost to follow up. He had postural hypotension but was not tachypneic or hypoxemic. He tested positive for COVID-19 and chest x-ray showed prominent bilateral broncho-vascular markings; he was hospitalized as mild COVID-19 pneumonia. Laboratory investigations revealed secondary adrenal insufficiency, secondary hypothyroidism and hypogonadotropic hypogonadism. MRI of the pituitary now showed persistence of the pituitary macroadenoma, measuring 3.5 x 3.7 x 2.4 cm in dimensions, causing sellar obliteration and left cavernous sinus invasion. Treatment with stress dose steroids, Hydrocortisone 50 mg 4 times daily was initiated, followed by thyroid hormone replacement with Levothyroxine 125 mcg daily. He also received antivirals and supportive care for COVID-19, guided by local hospital protocol. After significant clinical improvement, steroids were tapered down and he was discharged on a maintenance dose of 20 mg hydrocortisone per day in divided doses. The patient was stable at outpatient follow up after one month. He was started on testosterone replacement for erectile dysfunction due to hypogonadotropic hypogonadism. He was offered surgery for complete resection of the residual pituitary adenoma, but he declined and preferred to continue medical therapy. Conclusion: Hypopituitarism is associated with significant morbidity and premature mortality, a key risk factor being cortisol deficiency. Adrenal crisis is a life-threatening medical emergency and remains an important cause of death in patients with adrenal insufficiency. These patients are also vulnerable to develop severe complications from COVID-19 infection due to the absence of normal cortisol responses to stress. Despite receiving stress dose corticosteroids, this high-risk patient recovered from COVID-19 pneumonia without complications. These findings support the use of corticosteroids when necessary for treatment of coexisting conditions in patients with COVID-19.

scholarly journals MON-261 Pituitary Abscess Presenting as Pituitary Macroadenoma Causing Hypopituitarism and Acute Meningitis; A Wolf in Sheep’s Clothing

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Fatima Jalil ◽  
Sidra Azim ◽  
Vitaly Kantorovich
Keyword(s):  
Hormone Replacement ◽  
Hypogonadotropic Hypogonadism ◽  
High Dose ◽  
Pituitary Macroadenoma ◽  
Pituitary Abscess ◽  
Free T4 ◽  
High Grade Fever ◽  
Mri Brain ◽  
Cystic Component ◽  
Secondary Hypothyroidism

Abstract Background: Pituitary abscess (PA) is a rare but life-threatening cause of suprasellar (SS) lesion and pituitary hormonal deficiencies with an incidence of 0.2% - 1.1%. Clinical Case: A 65-year woman with history of transient ischemic attacks, presented with double vision for 3 months (mon) and sudden onset, severe headache. She was diagnosed with cranial nerve (CN) VI palsy. MRI brain showed 1.7 cm SS mass suggestive of pituitary macroadenoma abutting optic chiasm. Visual field testing was unreliable due to underlying visual defects. She had progressive improvement in her diplopia. She was referred to Endocrinology for worsening fatigue. Physical exam was unremarkable. Pituitary hormonal work up showed secondary hypothyroidism with TSH 1.17 mIU/L (0.4-4.5), free T4 0.6 ng/dL (0.8-1.8); hypogonadotropic hypogonadism with FSH 12 mIU/ml (23-116.3), LH 1.7 mIU/ml (10-54.7), estradiol <15 (<31); elevated prolactin due to stalk affect with prolactin level of 83.3 ng/mL (3-30). She had normal IGF-1 of 154 ng/mL (41-279), ACTH 12 pg/mL (6-50), cortisol 14.1 mcg/dL (4-22) and mildly low sodium 134 mmol/L (135-146). Levothyroxine 50 mcg daily was started. The MRI brain at 2 mon and 6 mon follow up showed stable 1.8 cm peripherally enhancing SS mass. She was planned for elective pituitary adenoma resection but prior to that that was emergently admitted to ICU with high grade fever, confusion, seizures, severe hyponatremia with sodium of 122 mmol/L (135-146) and a concern for meningitis. She had a dental crown placed 3 weeks ago. MRI brain showed increase in size of the cystic component of SS mass. She was started on empiric IV antibiotics and high dose steroids. She underwent trans-sphenoidal surgery (TSS), and actually found to have a pituitary abscess. Gram stain of purulent material was positive for neutrophils. Pathology showed pituitary gland with focal infarct and surrounding acute on chronic inflammation and fibrosis. The intra-operative abscess cultures grew Cutibacterium (Proprionibacterium) acnes. She is planned to receive 6 weeks of IV antibiotics. Conclusion: We present a case of pituitary abscess presenting as a SS mass causing hypopituitarism. It was presumed pituitary macroadenoma due to the sub-acute onset and lack of progression. She developed acute deterioration in sensorium leading to concern for meningitis and PA requiring timely diagnosis and management with trans-sphenoidal resection and IV antibiotics. Definitive diagnosis of PA is usually made post-operatively. 60% of patients with PA and new onset hypopituitarism may require long term hormone replacement. References: 1. Agyei JO, Lipinski LJ, Leonardo J. Case Report of a Primary Pituitary Abscess and Systematic Literature Review of Pituitary Abscess with a Focus on Patient Outcomes. World Neurosurg. 2017 May;101:76-92

scholarly journals Adrenal Crisis in Long Term Use of Exogenous Steroid With Inappropriate Tapering off Process

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A108-A109
Author(s):  
Nani Oktavia ◽  
Chici Pratiwi ◽  
Jerry Nasaruddin ◽  
Muhammad Ikhsan Mokoagow ◽  
Marina Epriliawati ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Blood Glucose ◽  
Adrenal Insufficiency ◽  
Clinical Manifestations ◽  
Adrenal Crisis ◽  
High Dose ◽  
Symptomatic Therapy ◽  
Bowel Sound ◽  
Primary Adrenal Insufficiency

Abstract Background: Adrenal crisis is an emergency condition in endocrinology that commonly found in primary adrenal insufficiency but also occur in chronic adrenal insufficiency triggered by various conditions such as sepsis, infection, trauma, burns, surgery, and myocardial infarction. In chronic adrenal insufficiency, adrenal crisis can be induced by excessive reductions or inadequate discontinuation of steroid treatment. Case Illustration: A 40-year old-man admitted with chief complaint abdominal pain since seven days before admission. He felt pain in the umbilical area and slowly radiated to all the part of abdomen. Other than that, he also felt nausea, had vomitus, fever, and constipation for five days. He was immobilized for four months, with muscles weakness and atrophy. He was diagnosed with Focal Segmented Glomerulosclerosis and had a high dose of methylprednisolone (48 mg) for 6 months, 40 mg for the next 2 months and methylprednisolone 12 mg for the last 2 months. The last two month, he began to have severe general weakness and hypotension. On physical examination we found hypotension, fever (38.1o C), pale conjunctiva, moon face, buffalo hump, slightly distended and tenderness of abdomen, normal bowel sound, and purple striae all over the abdomen and extremities. On laboratory examination, Hb was 8.2 (n 11.7 – 15.5 g/dl), leukocytes 10,400 (5.00 – 10.00 x 103/μL), Na 123 (n 135 – 147 mmol/L), random blood glucose 74 (n 70 – 140 mg/dL). On abdominal X ray, there was prominent faecal material and no signs of ileus. No sign of infection found in urinalysis. He had sodium correction, packed red cell transfusion, symptomatic therapy including laxative, methylprednisolone 12 mg, but no improvement of signs and symptoms beside be able to defecate. The abdominal ultrasound gave a normal result. The morning cortisol level was then examined, with the result 14.4 (n 3.7–19.4). The patient was then diagnosed with adrenal crisis based on the clinical manifestations and had hydrocortisone therapy 100 mg a day for 2 consecutive days. After hydrocortisone administration, the symptoms improved, no fever and abdominal pain, he had normotension, increased sodium level 132 (n 135 – 147 mmol/L) and blood glucose level 118 (n 70 – 140 mg/dL). On the third day the patient discharged with oral hydrocortisone 15 mg in the morning and 10 mg in the afternoon. Conclusion: Adrenal crisis was generally found in primary adrenal insufficiency but could also occur in secondary adrenal insufficiency due to inappropriate tapering off process of long term glucocorticoid use.

scholarly journals Reduced epinephrine reserve in response to insulin-induced hypoglycemia in patients with pituitary adenoma

2007 ◽  
Vol 157 (3) ◽  
pp. 265-270 ◽  
Author(s):  
Shinya Morita ◽  
Michio Otsuki ◽  
Maki Izumi ◽  
Nobuyuki Asanuma ◽  
Shuichi Izumoto ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Adrenal Insufficiency ◽  
Serum Cortisol ◽  
Tolerance Test ◽  
Secondary Adrenal Insufficiency ◽  
Counterregulatory Hormones ◽  
Insulin Tolerance ◽  
Secondary Hypothyroidism ◽  
Severe Impairment ◽  
Plasma Gh

Objective: Hypoglycemia induces rapid secretion of counterregulatory hormones such as catecholamine, glucagon, cortisol, and GH. Insulin-induced hypoglycemia is used for evaluating GH–IGF-I and ACTH–adrenal axes in patients with pituitary disorders. The aim of this study was to determine whether the response of catecholamine secretion to hypoglycemia is disrupted in patients with pituitary adenoma. Methods: The study population comprised 23 patients with pituitary adenoma (non-functioning adenoma or prolactinoma). An insulin tolerance test was performed and serum catecholamines as well as plasma GH and serum cortisol were measured. Results: The study patients showed diminished response of plasma epinephrine to insulin-induced hypoglycemia. With the cutoff level of peak epinephrine for defining severe impairment set at 400 pg/ml, more patients with secondary adrenal insufficiency showed severe impairment of the epinephrine response than did those without it. Peak epinephrine levels to insulin-induced hypoglycemia were significantly correlated with peak cortisol levels. In patients with secondary hypothyroidism, secondary hypogonadism, GH deficiency, or diabetes insipidus, the prevalence of severe impairment of the epinephrine response was similar to that in patients without these deficiencies. Conclusions: Impaired epinephrine secretion in response to insulin-induced hypoglycemia was frequently observed in patients with pituitary adenoma. This disorder was especially severe in patients with secondary adrenal insufficiency.

scholarly journals PC3 - 142 Gamma Knife Radiosurgery in Patients with Persistent Acromegaly or Cushing’s Disease: Long-Term Risk of Hypopituitarism

2016 ◽  
Vol 43 (S4) ◽  
pp. S22-S22
Author(s):  
O. Cohen-Inbar
Keyword(s):  
Gamma Knife ◽  
Cavernous Sinus ◽  
Cushing’S Disease ◽  
Hormone Replacement ◽  
Gamma Knife Radiosurgery ◽  
Common Adverse Effect ◽  
Cushing's Disease ◽  
Pituitary Hormone

For patient with a recurrent or residual acromegaly or Cushing’s disease (CD) after resection, Gamma knife radiosurgery (GKRS) is often used. Hypopituitarism is the most common adverse effect after GKRS treatment. The paucity of studies with long-term follow up has hampered understanding of the latent risks of hypopituitarism in patients with a Acromegaly or CD. We report the long-term risks of hypopituitarism for patients treated with GKRS for Acromegaly or CD. Methods: From a prospectively created, IRB approved database, we identified all patients with a Acromegaly or CD treated with GKRS at the University of Virginia from 1989 to 2008. Only patients with a minimum endocrine follow up of 60 months were included. The median follow-up is 159.5 months (60.1-278). Thorough radiological and endocrine assessments were performed immediately before GKRS and at regular follow-up intervals. New onset of hypopituitarism was defined as pituitary hormone deficits after GKRS requiring corresponding hormone replacement. Results: 60 patients with either Acromegaly or CD were included. Median tumor volume at time of GKRS was 1.3 cm3 (0.3-13.4), median margin dose was 25 Gy (6-30). GKRS induced new pituitary deficiency occurred in 58.3% (n=35) of patients. Growth Hormone deficiency was most common (28.3%, n=17). The actuarial overall rates of hypopituitarism at 3, 5, and 10 years were 10%, 21.7%, and 53.3%, respectively. The median time to hypopituitarism was 61 months after GKRS (range, 12-160). Cavernous sinus invasion of the tumor was found to correlate with the occurrence of a new or progressive hypopituitarism after GKRS (p=0.018). Conclusions: Delayed hypopituitarism increases as a function of time after radiosurgery. Hormone axes appear to vary in terms of radiosensitivity. Patients with adenoma in the cavernous sinus are more prone to develop loss of pituitary function after GKRS.

scholarly journals MON-096 Endocrine Complications Following Cancer Treatment in Survivors of Pediatric Solid Tumors: 18 Years’ Experience of a Single Academic Center

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Yena Lee ◽  
Arum Oh ◽  
Jin-Ho Choi ◽  
Han-Wook Yoo
Keyword(s):  
Cancer Treatment ◽  
Solid Tumors ◽  
High Dose ◽  
Pituitary Hormone ◽  
Hypergonadotropic Hypogonadism ◽  
Hematopoietic Stem ◽  
Pediatric Solid Tumors ◽  
The Mean ◽  
Endocrine Complications

Abstract Background: Survival rates of pediatric cancer have been significantly improved over recent decades because of advances in chemotherapy and radiotherapy. The endocrine consequences of cancer treatment have become the major medical issues in the childhood cancer survivors. This study was performed to investigate the long-term endocrine complications in survivors of pediatric solid tumors. Methods: From 2000 to 2018, 402 patients were diagnosed with solid tumors including hepatoblastoma (n = 72), neuroblastoma (n = 117), Wilms tumor (n = 57), Ewing sarcoma (n = 40), osteosarcoma (n = 65), and rhabdomyosarcoma (n = 51) in our institute. Among them, 96 patients (24%) were expired during the follow-up period. Growth profiles and endocrinologic findings were analyzed by retrospective chart review in 306 survivors of solid tumors. Results: The median age at diagnosis of primary cancer was 3 years (range, 0 month to 18 years). The mean treatment duration was 11.7 ± 12.6 months, and the mean follow-up duration after cancer treatment was 7.1 ± 4.8 years. Short stature,which was defined by height-SDS below -2.0, was found in 39 patients (12.7%) with the mean height-SDS of -2.59 ± 0.45. Primary hypothyroidism was detected in 19 patients (6.2%), and 15 of them were treated with radiotherapy or 131I-MIBG therapy due to the metastatic neuroblastoma. Sixteen patients (5.2%) developed hypergonadotropic hypogonadism, whereas three patients (1%) were diagnosed with central precocious puberty. Vitamin D deficiency and osteoporosis were found in 4 patients (1.3%) and 3 patients (1%), respectively. Primary adrenal insufficiency was found in one patient who underwent bilateral adrenalectomy because of bilateral neuroblastoma. One patient with rhabdomyosarcoma in the nasal cavity underwent high dose radiotherapy (50.4 Gy) around the tumor site, eventually leading to multiple pituitary hormone deficiency. In multivariable analysis, longer duration of treatment (≥24 months) was associated with the endocrine complications (OR = 3.94; CI 1.41-11.06) and hematopoietic stem cell transplantation was a major risk factor for endocrine complications (OR = 4.70; CI 2.14-10.29). Conclusions: Various endocrine complications can occur in survivors of solid tumors in children and adolescents caused by treatment modalities including surgery, chemotherapy, and radiotherapy, rather than the tumor itself. Lifetime monitoring is necessary to detect endocrine consequences such as growth retardation, hypergonadotropic hypogonadism, and thyroid dysfunctions.

scholarly journals The incidence of adrenal crisis in the postoperative period of HPA axis insufficiency after surgical treatment for Cushing’s syndrome

2019 ◽  
Vol 181 (2) ◽  
pp. 201-210 ◽  
Author(s):  
Leonie H A Broersen ◽  
Femke M van Haalen ◽  
Tina Kienitz ◽  
Olaf M Dekkers ◽  
Christian J Strasburger ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Crisis ◽  
Pituitary Hormone ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Life Threatening ◽  
Glucocorticoid Deficiency

Background Adrenal crisis, the most feared complication of adrenal insufficiency, is a potentially life-threatening state of acute glucocorticoid deficiency. After successful surgery for Cushing’s syndrome, many patients develop (transient) adrenal insufficiency. The incidence of adrenal crisis in patients treated for hypercortisolism is unknown. Methods Cohort study included consecutive patients with Cushing’s syndrome with adrenal insufficiency after surgery from Leiden and Berlin from 2000 to 2015. We summarized the incidence of adrenal crisis, compared patients with and without adrenal crisis regarding potential risk factors for its occurrence and assessed the effect of better education in time on incidence of adrenal crisis. Results We included 106 patients, of whom 19 patients had a total of 41 adrenal crises. There were 9.0 crises per 100 patient-years at risk (95% confidence interval (CI): 6.7–12.0). All crises occurred while on hydrocortisone replacement. The risk ratio for a recurrent crisis was 2.3 (95% CI: 1.2–4.6). No clear change in incidence of adrenal crisis due to better education in time was observed. There was no difference in recurrence rate between patients with, and without any crisis, but patients with adrenal crisis had more often pituitary deficiencies. Conclusions The incidence of adrenal crises after treatment for Cushing’s syndrome is substantial, and patients who suffered from an adrenal crisis have higher risk for recurrent crisis. Adrenal crisis tends to present early after remission of Cushing’s syndrome, which is probably the period of severest HPA axis suppression, despite in general higher hydrocortisone replacement doses for withdrawal complaints in this period. Additional pituitary hormone deficiencies may be a risk marker for increased risk of adrenal crisis. However, further risk factor analysis is needed to identify risks for a first crisis. Effective education methods to prevent adrenal crises should be identified and implemented, including stress instructions by trained nursing staff before hospital discharge.

scholarly journals Idiopathic Hypophysitis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A588-A588
Author(s):  
Hafsa Amjed ◽  
Sobia Sadiq
Keyword(s):  
Thyroid Hormone ◽  
Hormone Replacement ◽  
Hormone Deficiency ◽  
Bright Spot ◽  
High Dose ◽  
Mri Findings ◽  
Central Hypothyroidism ◽  
Thyroid Hormone Replacement ◽  
Suprasellar Region ◽  
Secondary Hypothyroidism

Abstract Introduction: Hypophysitis is a heterogeneous condition that leads to inflammation of the sella / suprasellar region, potentially resulting in hormonal deficiencies or mass effects. Prevalence of hypophysitis ranges from 0.2% to 0.88%. Annual incidence of hypophysitis is 1 case per 9 million individuals. We present an interesting case of idiopathic hypophysitis. Clinical Case: A 52-year-old female was evaluated for secondary hypothyroidism. Patient complained of excess fatigue, weight gain, headaches, polyuria with nocturia and vision changes. She was seen by ophthalmology for formal visual field testing revealing right superior quadrantanopia. Labs significant for TSH 4 uIU/mL (ref range 0.27-4.2uIU/mL), FT4 0.72ng/dL (ref range 0.93-1.7 ng/dL), Anti TPO ab negative, AM cortisol 8.9UG/dL, ACTH 18pg/mL, IGF1 127ng/mL, prolactin 7ng/mL, FSH 102MIU/mL, LH 41 MIU/mL. MRI sella revealed homogenous enhancement of the pituitary with convex superior margin - 0.8*1*0.9cm concerning for hyperplasia. Due to the concern for hypophysitis, an extensive inflammatory workup was pursued which was negative. She was started on thyroid hormone replacement and DDAVP. An empiric trial of high dose steroids failed to provide any relief. During workup, patient complained of transient right sided face and arm tingling, diagnosed with TIA and started on aspirin. Due to worsening headaches, a pituitary biopsy was pursued. Pathology significant for glandular tissue ruling out inflammatory, neoplastic, and infectious etiologies. Neurology diagnosed the patient with hemicrania continua. Patient is currently maintained on thyroid hormone replacement and DDAVP with close follow-up. Discussion: The incidence of hypophysitis has recently increased due to increased awareness and also due to the use of medications like ICI. Primary hypophysitis is mostly due to autoimmune etiology. Secondary hypophysitis could result from infections, neoplastic conditions or an adverse effect of medications. Clinical presentations ranges from being asymptomatic to having features of hypopituitarism. Usually presents with mass effect, visual symptoms due to the upward expansion of the pituitary gland impinging the dura mater and optic chiasm. This is followed by symptoms of hormone deficiency, central DI and hyperprolactinemia. Central AI has been reported in 20%-75%, central hypogonadism in 15%-60%, central hypothyroidism in 25%-58%, GH deficiency in 5%-41%, and prolactin deficiency in 13%-25%. Typical MRI findings include, homogeneous pituitary contrast enhancement, pituitary stalk thickening and loss of posterior pituitary bright spot, ‘figure of 8’ appearance, ‘dural tail’. Treatment consists of surgery, anti-inflammatory medications, and radiotherapy. Glucocorticoid treatment is the cornerstone for medical management; however, the overall recurrence rate is high.

scholarly journals Increased mortality risk in patients with primary and secondary adrenal insufficiency

2021 ◽  
Author(s):  
Kanchana Ngaosuwan ◽  
Desmond G Johnston ◽  
Ian F Godsland ◽  
Jeremy Cox ◽  
Azeem Majeed ◽  
...  
Keyword(s):  
Cardiovascular Disease ◽  
Adrenal Insufficiency ◽  
Mortality Risk ◽  
Reference Population ◽  
Mortality Data ◽  
Adrenal Crisis ◽  
Malignant Neoplasms ◽  
Geographical Differences ◽  
All Cause Mortality

Abstract Context Mortality data in patients with adrenal insufficiency are inconsistent, possibly due to temporal and geographical differences between patients and their reference populations. Objective To compare mortality risk and causes of death in adrenal insufficiency with an individually-matched reference population. Design Retrospective cohort study. Setting UK general practitioner database (CPRD). Participants 6821 patients with adrenal insufficiency (primary, 2052; secondary, 3948) and 67564 individually-matched controls (primary, 20366; secondary, 39134). Main outcome measures All-cause and cause-specific mortality; hospital admission from adrenal crisis. Results With follow-up of 40799 and 406899 person-years for patients and controls respectively, the hazard ratio (HR; [95%CI]) for all-cause mortality was 1.68 [1.58 - 1.77]. HRs were greater in primary (1.83 [1.66 - 2.02]) than in secondary (1.52 [1.40 - 1.64]) disease; (HR; primary versus secondary disease, 1.16 [1.03 - 1.30]). The leading cause of death was cardiovascular disease (HR 1.54 [1.32-1.80]), along with malignant neoplasms and respiratory disease. Deaths from infection were also relatively high (HR 4.00 [2.15 - 7.46]). Adrenal crisis contributed to 10% of all deaths. In the first two years following diagnosis, the patients’ mortality rate and hospitalisation from adrenal crisis were higher than in later years. Conclusion Mortality was increased in adrenal insufficiency, especially primary, even with individual matching and was observed early in the disease course. Cardiovascular disease was the major cause but mortality from infection was also high. Adrenal crisis was a common contributor. Early education for prompt treatment of infections and avoidance of adrenal crisis hold potential to reduce mortality.

scholarly journals Immune checkpoint inhibitor related hypophysitis: diagnostic criteria and recovery patterns

2021 ◽  
Author(s):  
Ha Nguyen ◽  
Komal Shah ◽  
Steven G Waguespack ◽  
Mimi I Hu ◽  
Mouhammed Amir Habra ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Diagnostic Criteria ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitor ◽  
Checkpoint Inhibitor ◽  
High Dose ◽  
Central Hypothyroidism ◽  
Central Adrenal Insufficiency ◽  
Mri Changes

Data on the diagnosis, natural course and management of immune checkpoint inhibitor (ICI) related hypophysitis (irH) are limited. We propose this study to validate the diagnostic criteria, describe characteristics and hormonal recovery and investigate factors associated with occurrence and recovery of irH. A retrospective study including patients with suspected irH at the University of Texas MD Anderson Cancer Center from 5/2003 to 8/2017 was conducted. IrH was defined as: (1) ACTH or TSH deficiency plus MRI changes or (2) ACTH and TSH deficiencies plus headache/fatigue in the absence of MRI findings. We found that of 83 patients followed for a median of 1.75 years (range 0.6-3), the proposed criteria used at initial evaluation accurately identified 61/62 (98%) irH cases. In the irH group (n=62), the most common presentation were headache (60%), fatigue (66%), central hypothyroidism (94%), central adrenal insufficiency (69%) and MRI changes (77%). Compared with non-Ipilimumab (Ipi) regimens, Ipi has a stronger association with irH occurrence (p=0.004) and a shorter time to irH development (p<0.01). Thyroid, gonadal and adrenal axis recovery occurred in 24%, 58% and 0% patients, respectively. High dose steroids (HDS) or ICI discontinuation were not associated with hormonal recovery. In the non-irH group (n=19), one patient had isolated central hypothyroidism and 6 had isolated central adrenal insufficiency. All remained on hormone therapy at last follow up. We propose a strict definition of irH that identifies the vast majority of patients. HDS and ICI discontinuation is not always beneficial. Long term follow up to assess recovery is needed.

scholarly journals Central Adrenal Insufficiency Is Rare in Adults With Prader–Willi Syndrome

2020 ◽  
Vol 105 (7) ◽  
pp. e2563-e2571 ◽  
Author(s):  
Anna G W Rosenberg ◽  
Karlijn Pellikaan ◽  
Christine Poitou ◽  
Anthony P Goldstone ◽  
Charlotte Høybye ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Outcome Measure ◽  
Tolerance Test ◽  
Pituitary Hormone ◽  
Prader Willi Syndrome ◽  
Insulin Tolerance ◽  
Synacthen Test ◽  
Central Adrenal Insufficiency ◽  
Stress Dose ◽  
Hydrocortisone Treatment

Abstract Context Prader–Willi syndrome (PWS) is associated with several hypothalamic-pituitary hormone deficiencies. There is no agreement on the prevalence of central adrenal insufficiency (CAI) in adults with PWS. In some countries, it is general practice to prescribe stress-dose hydrocortisone during physical or psychological stress in patients with PWS. Side effects of frequent hydrocortisone use are weight gain, osteoporosis, diabetes mellitus, and hypertension—already major problems in adults with PWS. However, undertreatment of CAI can cause significant morbidity—or even mortality. Objective To prevent both over- and undertreatment with hydrocortisone, we assessed the prevalence of CAI in a large international cohort of adults with PWS. As the synacthen test shows variable results in PWS, we only use the metyrapone test (MTP) and insulin tolerance test (ITT). Design Metyrapone test or ITT in adults with PWS (N = 82) and review of medical files for symptoms of hypocortisolism related to surgery (N = 645). Setting Outpatient clinic. Patients or Other Participants Eighty-two adults with genetically confirmed PWS. Main Outcome Measure For MTP, 11-deoxycortisol &gt; 230 nmol/L was considered sufficient. For ITT, cortisol &gt; 500 nmol/L (Dutch, French, and Swedish patients) or &gt; 450 nmol/L (British patients) was considered sufficient. Results Central adrenal insufficiency was excluded in 81 of 82 patients. Among the 645 patients whose medical files were reviewed, 200 had undergone surgery without perioperative hydrocortisone treatment. None of them had displayed any features of hypocortisolism. Conclusions Central adrenal insufficiency is rare (1.2%) in adults with PWS. Based on these results, we recommend against routinely prescribing hydrocortisone stress-doses in adults with PWS.

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