Stress Doses Of Hydrocortisone Research Articles

Circulatory collapse requiring mechanical circulatory support in a child with autoimmune adrenal insufficiency: a case report

BackgroundWe report a rare case of new onset autoimmune adrenal insufficiency in childhood, presenting with severe shock requiring mechanical circulatory support. In the current era of readily available imaging, laboratory and other diagnostic investigations, medical history and careful physical exam can often provide valuable diagnostic information for timely therapy.Case presentationA 7-year-old boy with a history of mild intermittent asthma, presented with severe cardiogenic shock requiring extracorporeal membrane oxygenation (ECMO). Bronzing of his entire body was noted on physical exam. Stress dose hydrocortisone was given for suspected adrenal insufficiency. After weaning from ECMO and extensive rehabilitation, the patient recovered and was discharged home.ConclusionPrimary adrenal insufficiency (PAI) should be considered in the context of physical exam and laboratory findings, even in the presence of circulatory shock.

6937 A Rare Case of Pembrolizumab-induced Multiple Endocrinopathies Presenting with Acute Adrenal Crisis Secondary to Isolated ACTH Deficiency - A Case Report

Abstract Disclosure: R. Abdelmasih: None. W. Pan: None. Introduction: Pembrolizumab is an immune checkpoint inhibitor (ICI) that blocks programmed cell death receptor 1 (PD-1) which took a massive leap in the oncology world. However, we are still understanding more about its immune-related adverse events (irAEs). Here we report a case of adrenal crisis due to pembrolizumab-induced hypophysitis and adrenalitis. Case Description: A 74-year-old male with a history of left renal cell carcinoma post left nephrectomy 5 years ago, complicated with lung metastasis for which Pembrolizumab was started. 5 months after, patient developed hypothyroidism and was started on Levothyroxine. 3 months later, Patient presented with lethargy and hypotension. Physical exam revealed hypotension (MAP of 55), fever, and acute encephalopathy. AM cortisol 4.2 ug/dL, Patient was started on IV pressors and a stress dose of Hydrocortisone. Hemodynamics improved, and Hydrocortisone was tapered down. ACTH stimulation test revealed a subnormal response of cortisol 0.2, 1.7, and 2.7 ug/dL at 0, 30, and 60 minutes respectively in the setting of very low ACTH of < 1.5 pg/ml, other pituitary hormones were checked, revealing normal insulin-like growth factor was 116 ng/mL, LH 15.29 mIU/mL, and FSH 27.45 mIU/mL. TSH was high 15 mIU/mL and free T4 was 1.4 ng/dl, levothyroxine dose was increased, and hydrocortisone was maintained at 10 in am and 5 in pm with excellent response. Discussion:ICIs inhibit the immunological pathways that control T-cell activation, leading to multiple immune-related endocrinopathies. Thyroiditis is the most reported one (30%), though anti-PD1-induced hypophysitis is exceptionally rare (< 1%). To date, the pathophysiology of pembrolizumab-induced central AI is not clearly understood, it’s presumed that it causes lymphocytic hypophysitis often presenting with isolated ACTH deficiency. Also, Pembrolizumab-induced primary adrenal insufficiency has seldom been reported and not yet understood, interestingly, anti-steroidogenic enzyme antibodies were absent in those few most reported cases which suggests a different mechanism compared to traditional primary AI. We suggest that our patient had combined primary and secondary adrenal insufficiency based on ACTH stim test result with very low cortisol responses in the setting of very low ACTH, which we believe is what led to such severe presentation of adrenal crisis. Diagnosis of PD1-induced AI can be challenging due to the overlapping of advanced malignancy and AI presentation. A higher level of suspicion is prompted especially in patients who experienced other endocrine irAEs (as our patient who had PDL-1-induced hypothyroidism). We present this case to shed light on this rare yet potentially fatal endocrine adverse event that can be reversed by prompt diagnosis and timely management due to the increasing use of pembrolizumab. More cases are needed for a better understanding of such a rare adverse event. Presentation: 6/2/2024

12391 When Pressure Drops, Consequences Rise: A Case Of Pituitary Apoplexy And Watershed Infarcts

Abstract Disclosure: D. Moreno Watashi: None. H.R. Alkaissi: None. M.B. Sharma: None. Introduction: Pituitary apoplexy (PA) results from sudden hemorrhaging and infarction of the pituitary gland, typically within a pituitary adenoma. The primary symptom is a sudden, severe headache, often followed by acute endocrine dysfunction, ophthalmoplegia and visual or neurological disturbances. Case Description: A 68-year-old male with a past medical history of hypertension presented to the emergency department with acute chest and upper back pain, beginning one day prior. No history of smoking, alcohol intake or drug use. Blood pressure (BP) on arrival was 180/95 mmHg. Initial labs revealed mild electrolyte abnormalities. Troponins were normal, but ECG showed diffuse T wave inversion and ST depression. CT angiography revealed a type B aortic dissection. He was managed in the critical care unit with continuous esmolol intravenous (IV) infusion and planned for endovascular repair. During his stay, he became disoriented and hypotensive. MRI from the brain incidentally discovered a pituitary macroadenoma measuring 2.9x2.2x3cm and recent infarcts, with a distribution concerning for hypoperfusion. Fundoscopy concerning for optic chiasm compression. Labs indicated secondary hypothyroidism and hypogonadism (TSH 0.3 uUI/ml, free T4 0.88 ng/dL, total testosterone 56.3 ng/dL). Neurosurgical intervention was deferred until after aortic repair. Endovascular aortic repair was performed with episodes of hypotension. Post-procedure, the patient developed fever (39.5°C) and worsening mental status. Head CT revealed an increased suprasellar extension of the pituitary macroadenoma and vasogenic edema, consistent with hemorrhage of the lesion. He was started on stress doses of hydrocortisone, levothyroxine 125mcg and managed conservatively due to a high surgical risk. Discussion: PA's extrinsic risk factors include angiographic procedures, endocrinological dynamic tests, anticoagulation therapy, exogenous estrogen administration, orthopedic and cardiac surgeries (due to BP fluctuations). The intrinsic risk factors are non-functioning adenomas, macroadenomas and suprasellar extension. Treatment is focused on immediate correction of endocrine derangements and relieving local compression. Stress dosage of corticosteroids should be administered IV as soon as the diagnosis is confirmed to prevent life-threatening corticotropic deficiency. Neurosurgical decompression via a transsphenoidal approach is the definitive therapy to relieve local compression with severe visual loss, obtundation or rapidly progressive course. The potential outcomes are based on the extent of hemorrhage, necrosis, and edema. PA should be treated as a medical emergency in the setting of hemodynamic changes from acute corticotropic deficiency and irreversible neurological damage from acute intracranial compression. The risk factors for PA should be known to assist in early diagnosis. Presentation: 6/1/2024

7658 A Case of Recurrent Cardiac Tamponade as Initial Presentation of Primary Adrenal Insufficiency

Abstract Disclosure: A. Farooq: None. A. Ghumatkar: None. J. Snitzer: None. Introduction:Cardiac tamponade, a life-threatening emergency, can be associated with primary adrenal insufficiency (Addison Disease), and accurate diagnosis requires a high degree of clinical suspicion in patients with uncertain etiology and nonspecific signs and symptoms. Case Presentation:A 58-year-old female with a history of Hashimoto thyroiditis was evaluated in the hospital for two episodes of cardiac tamponade requiring emergent pericardiocentesis within one month. The patient had been experiencing symptoms of fatigue, nausea, lightheadedness, dizziness, hiccups, and tanned skin for the past year. She also had a recent flu-like illness with nausea and vomiting four months before her current presentation. Her vitals were notable for hypotension during these episodes. Her lab work showed hyponatremia (128 mEq/L, reference range: 136-145 mEq/L) with normal potassium and glucose levels. Her thyroid function tests were within normal limits on her home levothyroxine dosage (TSH 4.588 IU/ml, reference range: 0.5-8.9 IU/ml). An ACTH stimulation test was performed based on the patient's clinical presentation, consistent with adrenal insufficiency. Her baseline cortisol level was 5.9 mcg/dL (reference range: 6.7-22 mcg/dL), a post-ACTH stimulation level of 6.7 mcg/dL after 30 minutes, and 6.0 mcg/dL after 60 minutes. Her 21 alpha-hydroxylase antibody titer was also positive for Addison Disease (231U/mL, reference range <1 IU/mL). A CT scan of the abdomen with intravenous contrast was done, which revealed no abnormality of the adrenal glands. She was treated with stress doses of hydrocortisone during hospitalization and discharged with maintenance dosage and follow-up with endocrinology. She had another episode of acute adrenal crisis about a month later but had no pericardial effusion this time and responded well to the treatment with stress doses of steroids. She has remained healthy at 14 years of follow-up. Conclusion: This case emphasizes the need to consider adrenal insufficiency in the evaluation of cardiac tamponade or pericardial effusions, especially in patients with existing autoimmune glandular disorders. This patient may have autoimmune polyglandular syndrome type 2 (APS-2), which is a cluster of two or more endocrine glandular disorders primarily diagnosed in adulthood and predominant in females. The risk of adrenal crisis is more than twofold in Addison disease due to APS-2. Lastly, this case highlights that hiccups can be related to adrenal insufficiency, with pathophysiology possibly involving diaphragmatic irritation due to adrenal inflammation that resolves with treatment. Presentation: 6/3/2024

7761 Unmasking The Uncommon: A Case Of Primary Adrenal Insufficiency Presenting As Severe Hypercalcemia

Abstract Disclosure: A. Misbah: None. Z. Al-Hiti: None. A. Saleh: None. M. Joda: None. K. Rajamani: None. Adrenal insufficiency is a recognized but uncommon cause of hypercalcemia. The exact mechanism remains unknown; however, it is thought to be a combination of the hypovolemic state in adrenal insufficiency leading to decreased urinary clearance, as well as increased bone resorption. We present a case of primary adrenal insufficiency presenting with severe hypercalcemia. A 41 year old female with a past medical history of osteopenia, recently diagnosed postural orthostatic tachycardia syndrome (POTS), and hyponatremia attributed to SIADH presented with complaints of weakness and poor oral intake. She also reported a history of nausea, vomiting, weight loss, fatigue, and dizziness, for the last few months. She reported feeling well prior to a COVID-19 infection a few months back. At presentation, she was hypotensive with a blood pressure of 70/50 mmHg, while her other vital signs were stable. She appeared ill, and was noted to have dry oral mucous membranes. There was no increased pigmentation noted on exam. Initial laboratory evaluation revealed sodium of 130 (135-145 mmol/L), potassium of 5.3 (3.5-5.1 mmol/L), and creatinine of 1.7 (0.5-0.8 mg/dL). She had severe hypercalcemia with a calcium level of 15.5 (8.3-10.6 mg/dL) and had a blood glucose level of 53 (70-100 mg/dL). Venous blood gas indicated metabolic acidosis. Morning cortisol was low at 1 (3-23 ug/dL). Serum ACTH was elevated at 775 (0-46 pg/mL). Cosyntropin stimulation test confirmed primary adrenal insufficiency. She underwent work-up for hypercalcemia, which revealed a suppressed PTH, normal levels of PTHrP, vitamin D, and vitamin A. Serum and urine protein electrophoresis was normal. She was initially managed in the ICU and received aggressive fluid resuscitation. She was started on stress dose of hydrocortisone, which was then tapered to physiological doses of oral hydrocortisone and fludrocortisone. Subsequent improvements were observed in blood pressure, laboratory abnormalities, and normalized serum calcium levels. She was discharged home on hydrocortisone and fludrocortisone. On follow-up, she was doing well with the resolution of her symptoms. This case highlights the importance of recognizing adrenal insufficiency as one of the causes of hypercalcemia. Although hypercalcemia as a consequence of adrenal insufficiency is well-documented, the exact prevalence is not known but is estimated to be around 6%. After excluding more common diseases such as primary hyperparathyroidism and malignancy, the possibility of adrenal insufficiency should be considered as a cause of hypercalcemia. Treatment involves hormone replacement as well as hydration, bringing resolution to symptoms. Her presentation was unique, also due to the fact that her initial symptoms were diagnosed as POTS as well as SIADH. Given the onset of symptoms after a COVID-19 infection a few months ago, there could also be a potential link between the two. Presentation: 6/3/2024

8800 A Case of Hypopituitarism Due to Sheehan Syndrome

Abstract Disclosure: N. Akabogu: None. S. Dejhansathit: None. F. Marium: None. B. Stanley: None. E.M. Bazuaye: None. L.S. Krusniak: None. L.G. Kurukulasuriya: None. U.Z. Khan: None. Background: Sheehan syndrome, which is also known as postpartum pituitary infarction, refers to the necrosis of the anterior pituitary gland due to postpartum hypovolemia, hemorrhage, shock, resulting in various degrees of hypopituitarism. The pituitary gland enlarges during pregnancy due to estrogen stimulation and becomes hyper-vascular, making it prone to infarction with postpartum hemorrhage. The initial symptoms include hyponatremia, weakness, weight loss, but the most prominent specific symptoms are inability to nurse and postpartum amenorrhea. Depending on the severity of hypopituitarism, symptoms may develop within the first few days to weeks after delivery or may take months to years to develop. Case presentation: 35year-old Caucasian female with a medical history of gestational diabetes, hyperlipidemia, and hypertension, who presented to the hospital with flu-like symptoms, leg pain, abdominal pain, and proximal muscle weakness. The evaluating hospitalist observed that patient appeared sluggish and spoke with a deep coarse voice, which concerned him for hypothyroidism. Thyroid function test revealed undetectable free T4 with inappropriately normal TSH of 1.38 – consistent with secondary hypothyroidism. Additional history from patient uncovered 9 months history of constant fatigue, weight gain, dry skin, hair loss, cold intolerance, and intermittent ankle swelling. There is no history of brain irradiation or head trauma. She has 4 biological children, and her last childbirth 3years ago was complicated by severe bleeding from her incision site, requiring transfusion of 6 units of blood. She has been amenorrheic since and is neither on birth control nor had hysterectomy. Physical exam showed mildly enlarged tongue, diffuse pallor, dry scaly skin on upper arms, scanty eyebrows, and slowed speech. Further work-up for pituitary insufficiency showed secondary adrenal insufficiency, secondary hypogonadism, low prolactin, and growth hormone deficiency. There was no evidence of AVP deficiency. Other lab and clinical findings were hypoglycemia, hyperlipidemia, elevated CK, elevated Cr, pericardial effusion, and diastolic hypertension. Pituitary MRI showed markedly attenuated and flattened pituitary gland with no focal mass. Patient was given stress dose hydrocortisone and thyroid hormone replacement, initially IV then oral. Following hydrocortisone and thyroid hormone replacement, her diastolic BP, hypoglycemia, CK, and Cr improved. Further management plans include initiating estrogen (plus progesterone) for bone health. Conclusion: Although Sheehan syndrome is rare in developed countries, it does occur. This case emphasizes the importance of detailed history taking, to discover the root of patients’ presentation. Even though TSH is the screening test for thyroid disease, when pituitary etiology is suspected, it is important to also check a free T4. Presentation: 6/1/2024

12393 Myxedema Presenting With Impending Cardiac Tamponade : A Case Report

Abstract Disclosure: A. Hoskote: None. V. Gupta: None. J. Seidenberg: None. The widespread availability of thyroid stimulating hormone (TSH) assays, routine screening and affordable replacement therapy has resulted in decreased prevalence of complications of untreated hypothyroidism. “Myxedema heart” is a rare syndrome that results in bradycardia, cardiomyopathy and pericardial effusion. We present a rare case of cardiac tamponade due to myxedema in a patient with unidentified hypothyroidism. A 49-year-old male was brought to the ER by his family for several weeks of cognitive slowing, weakness and inappropriate behavior involving defecating on himself eating old food and barricading in his room. The patient denied past medical or psychiatric history, medication use or recent physician evaluation. On exam he had macroglossia, was disheveled, forgetful but alert with slow speech, ataxia and muscle wasting. He was afebrile and hemodynamically stable. EKG showed sinus bradycardia. Labs were notable for sodium 133, hemoglobin 8.8g/dL and TSH was incidentally >100U. Levothyroxine was begun. The next day, he was noted to have increased lethargy, bradycardia and hypoglycemia and was transferred to the intensive care unit with concern for myxedema coma. Free T4 was <0.3 ng/dL (0.6-1.6) and free T3 was 1.6pg/mL (2.5-3.9). An echocardiogram revealed a large circumferential pericardial effusion with right atrial and ventricular collapse with tamponade. Treatment with 5 mcg liothyronine, intravenous levothyroxine and stress dose hydrocortisone was begun. A pericardial drain was placed, draining 980mL of sanguineous fluid. After clamping trials, the drain was removed 48 hours later. Labs revealed thyroglobulin <0.1ng/mL (1.6-50), thyroid peroxidase (TPO) antibody 574 IU/mL (<9), thyroglobulin antibody >2500 (<4) and thyroid ultrasound showed a normal sized thyroid gland with heterogenous echogenicity confirming a diagnosis of Hashimoto’s thyroiditis. Serial echocardiograms during the admission and a follow-up two months later showed a stable posterior effusion with no recurrence of the anterior pericardial effusion. Gradually mentation, hemodynamics and electrolyte derangements improved, and he was discharged on oral levothyroxine. TSH values showed sequential improvement to 48U in 15 days and to 11U in 2 months. Myxedema is a manifestation of severe hypothyroidism. The pericardium, a collagen-rich structure, is susceptible to myxedematous infiltration, leading to pericardial effusion. While small pericardial effusions are common with chronic hypothyroidism, a large pericardial effusion with tamponade is a rare life-threatening complication that requires prompt recognition and management. Clinicians must promptly identify myxedema and replace thyroid hormone to prevent progression of pericardial effusion to tamponade, maintaining especially high suspicion in those at risk of poor access to healthcare or inadequate health literacy. Presentation: 6/3/2024

7648 Bilateral Adrenal Hemorrhage After Laminectomy: A Rare Complication

Abstract Disclosure: M. Rizk: None. S. Sharma: None. H. Qadeer: None. R. Paudel: None. C. Sherpa: None. Introduction: Bilateral Adrenal Hemorrhage (BAH) is an exceedingly uncommon complication of laminectomy, with few cases reported in the literature. In patients with history of any surgical procedure who present with unexplained symptoms of abdominal pain, back pain, hypotension, fever, confusion, or electrolyte abnormalities especially hyponatremia, acute adrenal insufficiency (AI) should be highly suspected. We herein describe a case of BAH following laminectomy. Clinical Case: 63-year-old male with past medical history of hypertriglyceridemia was admitted for post traumatic L1 burst fracture and was treated with T11-L3 fusion and T12-L3 laminectomy. The patient had normal bilateral adrenal glands after the fall on CT thoracic/lumbar spine imaging. The course was complicated by thrombocytopenia initially thought to be induced by therapeutic heparin since heparin antibody was positive. Serotonin-release assay returned negative weeks later ruling out heparin induced thrombocytopenia. Two weeks later, he developed unexplained tachycardia and CT angiogram was done which showed segmental pulmonary embolism and bilateral adrenal nodules measuring at least 3.9 cm on right side and 3.3 cm on left side. He was treated with Eliquis and was discharged home. Two weeks later, he was readmitted with weight loss of 30 lbs, weakness and altered mental status. His vital signs were significant for hypotension and tachycardia. Initial work up showed profound hyponatremia (sodium 121 mEq/L), hyperkalemia (potassium 6.1 mEq/L), hypoglycemia (Glucose 58 mg/dL), hypercalcemia (calcium 11.7 mg/dL) and acute kidney injury (creatinine 2.58 mg/dL). Repeat CT scan of chest/abdomen/pelvis without contrast showed bilateral hyperdense 4 cm adrenal mass/hematoma. Since initial CT of thorax/spine done after the fall showed normal adrenal glands, the new bilateral hyperdense adrenal masses noted post procedure is suggestive of adrenal hemorrhage/hematoma. Undetectable cortisol and high ACTH confirmed AI. Patient was treated with stress dose hydrocortisone followed by slow taper. MRI of pituitary gland showed partial empty Sella however since ACTH was high, empty sella was only an incidental finding and not the cause of AI. He is maintained on hydrocortisone 15 mg in the morning and 5 mg in the evening plus fludrocortisone 0.1 mg daily. His fatigue has improved, he is gaining weight and electrolytes are normal. Conclusions: This case report emphasizes the significance of timely diagnosis and management of BAH, which leads to primary AI. BAH is a rare complication following laminectomy. Clinicians should be aware of this possibility and consider it in the differential diagnosis of postoperative abdominal pain and electrolyte abnormalities, especially hyponatremia. Timely recognition and high index of suspicion should prompt empiric glucocorticoid replacement to decrease mortality. Presentation: 6/1/2024

12212 A Rare Case Of Central Precocious Puberty In A Male Infant With Adrenal Hypoplasia Congenita

Abstract Disclosure: A. Mastoropoulou: None. A.H. Lane: None. Introduction: We describe a male with Adrenal Hypoplasia Congenita (AHC) caused by a novel mutation in NR0B1, who was noted at 9 months of age to have central precocious puberty (CPP). Clinical case: A 3 week-old full-term male presented with hypothermia and lethargy, and a 0.3 kg weight loss since birth. Labs were consistent with adrenal crisis: Na 109 mmol/L, K 10.4 mmol/L, Ca 12.2 mg/dL, glucose 43 mg/dL. He was stabilized with stress dose hydrocortisone (HC), insulin and antibiotics, and was admitted. Subsequent labs revealed cortisol 3.5 ug/dL (4.6-23), ACTH 1,123 pg/mL (7.2-63), 17OHP 181 ng/dL (<199), and a renin of 180 ng/mL/h (1.7-11.2) with aldosterone 14.9 ng/dL (6-179). Abdominal ultrasound was remarkable for non-visualization of the adrenal glands. The patient was discharged with HC, fludrocortisone and sodium supplementation with good tolerance and interval weight gain and normal electrolytes. Genetic testing identified a novel pathogenic c.707G>A [p.Trp236Ter] nonsense variant in the DNA-binding domain of NR0B1 (DAX-1). At 9 months of age, penile enlargement with prepubertal testes was noted. Testosterone was 318 ng/dL (Tanner stage I: 0-18), LH 3.2 mIU/mL (prepubertal: 0-0.3), DHEA-S 2 ug/dL (0-33), FSH 1.1 mIU/mL (prepubertal: 0-1), consistent with hypothalamic pituitary gonadal (HPG) axis activation. Given previous reports of prolonged but self-resolving “mini-puberty of infancy” in AHC, observation was initially chosen. At 12 months of age the bone age was consistent with the Greulich and Pyle 17 months standard. At 13 months of age testosterone remained elevated, therefore based on others’ reported experiences we increased HC from 8 to 15 mg/m2/day and noted a decrease in testosterone to 104 ng/dL within 5 days. HC was further increased to 18 mg/m2/day for 15 days, however testosterone increased to 270 ng/dL, therefore decision was made to treat with Lupron Depot. Conclusions: Historically, hypogonadotropic hypogonadism has been observed in 76% of adolescent patients with AHC who have alterations in NR0B1. CPP has been infrequently described in AHC, and the natural history and management of CPP in this setting is not established. CPP in AHC does not seem to correlate with any particular functional domain of DAX1, because previously reported NR0B1 variants associated with CPP, and this case’s, are dispersed across the gene. The course of our patient suggests multiple underlying mechanisms, including early HPG axis activation as well as the possibility of ACTH dependent precocious puberty. Our observations may contribute to the understanding of factors influencing normal and abnormal puberty in infants. Increased awareness of the possibility of CPP in AHC will aid clinicians in the earlier clinical and laboratory detection of this complication. Presentation: 6/1/2024

6419 Cushing’s Syndrome due to Metastatic Pancreatic Neuroendocrine Neoplasm With Incidental Pituitary Microadenoma

Abstract Disclosure: A. Ibrahim: None. L. Esper: None. R. Jain: None. N. El Asmar: None. Introduction: Pancreatic neuroendocrine neoplasms (PNENs) are rare with a reported incidence of 1-2/100,000. While the majority are non-functional, insulinomas and gastrinomas are the most common functional PNENs. Our case of ACTH-producing PNENs is extremely rare. Case presentation: A previously healthy 47-year-old female was hospitalized for worsening dyspnea. Her symptoms included rapid weight gain, skin hyperpigmentation, and lower extremity edema. Her exam was notable for elevated blood pressure of 181/120 mmHg, moon facies, purple abdominal striae, and proximal myopathy. Her lab analysis showed a cortisol level of 42 mcg/dL (6.2-19.4), ACTH of 254 pg/ml (7.2-63), DHEA sulfate of 171 mcg/dl (41-243), HbA1C 7%, K of 3 mmol/l and 24H urine cortisol >2100 mcg/24hrs. A post low-dose DST cortisol level was 42.7 mcg/dl. CT chest demonstrated an ill-defined hypodense lesion in the region of the pancreatic head. MRI abdomen revealed a 3 x 2.3 x 2.3 cm mass in the pancreatic head and many small enhancing lesions within the right lobe of the liver. Adrenal glands appeared normal. Subsequent PET CT scan with GA 68 DOTOTATE showed increased uptake in the pancreatic head mass and in the right hepatic lobe. Her pituitary MRI revealed a right pituitary gland 6 mm hypointense lesion, possibly a microadenoma. A high dose DST resulted in a cortisol level of 80 mcg/dl mostly consistent with ectopic ACTH secretion. EUS-guided biopsy of the pancreatic mass showed a well-differentiated neuroendocrine tumor that stained positive for ACTH, chromogranin, synaptophysin, and CD56. Treatment with Ketoconazole 600mg q6h and Octreotide 50mg q6h was started 3 days before surgery to control acute hypercortisolism as she developed acute psychosis. The patient subsequently underwent a Whipple procedure with successful resection of the pancreatic mass. Post-op, her ACTH levels dropped to 19 pg/ml with a cortisol level of 6 mcg/dl. Final pathology showed a well-differentiated grade 3 neuroendocrine tumor with a KI-67 labeling index greater than 20%. After surgery, the patient received a short course of stress dose hydrocortisone which was tapered off over the following 2 months. The patient is being followed for planning further intervention given the aggressive grade of the tumor and hepatic metastases. Conclusion: Ectopic ACTH-producing PNENs are rare. An accurate and timely diagnosis, while challenging, is key. Patients usually present with rapidly progressing symptoms that could cause life-threatening complications such as thrombosis and sepsis. We demonstrated the importance of utilizing a multidisciplinary team approach and starting cortisol-lowering medications early given the complexity of the disease, requiring rapid intervention and lifelong follow-up. Presentation: 6/3/2024

12417 Atypical Patterns Of Hypoglycemia Post-bariatric Surgery Due To Pancreatic Insulinoma

Abstract Disclosure: K.A. Chopra: None. Accurately diagnosing an insulinoma in patients who have had bariatric surgery can be challenging because the autonomic symptoms of palpitations, tremors, anxiety and neuroglycopenic hypoglycemia symptoms of confusion and loss of consciousness may overlap with dumping syndrome or be attributed to non-insulinoma pancreatogenous hypoglycemia. When evaluating hypoglycemia, it is crucial to obtain a detailed history of the pattern and timing of hypoglycemia episodes and collect a critical sample of insulin, proinsulin, c-peptide levels during an episode of low glucose to prevent misdiagnosis. 58-year-old woman with hypertension, obesity, and history of laparoscopic sleeve gastrectomy presented for evaluation of hypoglycemia. The patient lost 100 pounds after her sleeve gastrectomy. Within the past year, the patient had two hospitalizations for severe symptomatic hypoglycemia with associated loss of consciousness. Imaging showed atrophic pancreas and she was discharged with a prescription for daily prednisone without critical sample testing for insulin or c-peptide levels. She sought a second opinion for her hypoglycemia at UCM. Since she was taking prednisone for 10 months prior to initial evaluation at UCM, evaluation for adrenal insufficiency could not be completed initially. She started continuous glucose monitoring which showed multiple daily episodes of hypoglycemia, fasting and at other various timepoints. Fasting labs showed low glucose of 20 mg/dL (RR 60-99 mg/dL), elevated proinsulin 120 pmol/L (RR 3.6- 22 pmol/L), inappropriately normal insulin 16.4 uIU/mL (RR <28.5 uIU/mL), and inappropriately normal c-peptide 0.82 pmol/mL (RR 0.30-2.35 pmol/mL), consistent with insulinoma. CT abdomen showed 1.4 cm arterially enhancing round lesion in the pancreatic body. Endoscopic ultrasound showed 13 mm mass in the pancreatic body with fine needle aspiration with pathology showing a well-differentiated neuroendocrine tumor. She received stress dose hydrocortisone preceding surgery and underwent successful enucleation of 1.6 cm pancreatic neuroendocrine tumor. A few weeks after her surgery, her HPA axis was re-evaluated, and steroids were discontinued. She has had no episodes of hypoglycemia since. Although hypoglycemia can be associated post-bariatric surgery, most patients develop postprandial hypoglycemia due to surgical alterations in alimentary flow leading to increased incretin and insulin secretion (Mulla et al 2016). Therefore, it is important to consider insulinoma in the differential diagnosis of post-bariatric surgery patients presenting without typical postprandial patterns of hypoglycemia. Reference: Mulla CM, Storino A, Yee EU, Lautz D, Sawnhey MS, Moser AJ, Patti ME, Insulinoma After Bariatric Surgery: Diagnostic Dilemma and Therapeutic Approaches. Obes Surg. 2016 Apr;26(4):874-81. Presentation: 6/1/2024

Association of Stress Dose Hydrocortisone with the Prevention of Bronchopulmonary Dysplasia in Extremely Preterm Neonates

Background: Steroids are a key component of the management of bronchopulmonary dysplasia (BPD) in preterm neonates, but certain steroids are associated with an increased risk of neurodevelopmental impairment. Hydrocortisone may be a viable option due to milder side effects, but optimal dosing and timing of use have not been established. We aimed to determine whether early administration of stress dose hydrocortisone to preterm babies during acute illness is associated with decreased incidence of BPD at 36 weeks of gestation. Methods: We retrospectively identified extremely premature neonates admitted to our neonatal intensive care unit before 24 hours of age. The primary exposure was receipt of a course of stress dose hydrocortisone (at least 3 mg/kg/day for 3 days or more) in the first 14 days of life. Our control group comprised of neonates who did not receive stress dose hydrocortisone in the first 14 days of life. Results: We analyzed data for 375 neonates, 21 of whom received stress dose hydrocortisone in the first 14 days of life. About 90% of intervention group developed BPD vs. 64% in the control group ( P = .016). Incidence of severe BPD was significantly higher in the stress steroid group—62% vs. 23% in the control group ( P < .001). Conclusion: We noted that stress dose hydrocortisone administered during acute illness in the first 14 days of life was not associated with decreased incidence of BPD at 36 weeks among extremely preterm neonates. More studies are needed to determine the optimal dose and timing of steroid therapy.

FRI400 A Rare Case Of Acute Sheehan's Syndrome In An Antepartum Patient

Abstract Disclosure: E. Jalal: None. C. Baldwin: None. Sheehan’s syndrome (SS) is a known complication of postpartum hemorrhage. It is typically diagnosed several years after birth given slow symptom onset related to hypopituitarism. We report a rare case of life-threatening antepartum pituitary ischemia secondary to hypotension, representing an acute presentation of SS. A 19-year-old primigravid woman at 31 weeks of gestation was admitted to the intensive care unit for septic shock secondary to Klebsiella pneumoniae pyelonephritis. Despite appropriate treatment, persistent hypotension prevented weaning of vasopressor support. Due to concurrent mild hyponatremia, the following studies were obtained: morning cortisol (2.46 mcg/dL [4.5-23 mcg/dL]), thyroid stimulating hormone (TSH) (0.379 IU/L [0.47-4.7 IU/L]), free triiodothyronine (2.1 pg/mL [2.8-5.3 pg/mL]), and free thyroxine (0.77 ng/dL [0.7-1.8 ng/dL]). Endocrinology was consulted for evaluation of adrenal insufficiency and abnormal thyroid function tests. Her prolactin was measured at 10.3 ng/mL, far below the expected range in a 3rd trimester pregnancy (35-600 ng/mL). Her remaining anterior pituitary hormone panel was unremarkable. MRI brain without contrast showed no evidence of an acute stroke or hemorrhage and commented that the prominence of the pituitary gland was likely related to current pregnancy. Stress dose hydrocortisone 100mg thrice daily and levothyroxine 50 mcg daily were initiated, allowing for weaning of vasopressor support. By discharge, she was titrated to levothyroxine 75mcg daily and hydrocortisone 20mg in the morning and 5mg in the afternoon. Repeat outpatient pituitary function testing found increase of prolactin to 397 ng/mL (35-600 ng/mL) and of IGF-1 to 486 ng/mL (113-408 ng/mL) as well as normalization of TSH to 3.210 (0.45-4.5IU/L), indicating likely recovery of pituitary axis. When readmitted at 38 weeks gestation for scheduled induction, she was given stress dose hydrocortisone 100mcg for labor. On discharge, she was tapered to prednisone 10mg daily and levothyroxine 50mcg daily. Appropriate hormone replacement and stress dose hydrocortisone resulted in an uncomplicated delivery and signs of clinical pituitary recovery postpartum as she was able to breastfeed without need for supplementation and maintain normotension after self-discontinuing prednisone and levothyroxine. The leading diagnosis to explain this patient’s hypopituitarism is acute SS, which is defined as symptom onset within 6 weeks postpartum. In our case, septic shock caused an abrupt onset of persistent hypotension leading to hormone levels suggestive of panhypopituitarism while the patient was pregnant at 31 weeks of gestation, which has not been reported in prior literature. While known as a chronic diagnosis in the postpartum population, further research should explore possible stunning effect of acute hypotension manifesting as acute SS in peripartum patients. Presentation: Friday, June 16, 2023

SAT317 Adrenal Insufficiency Diagnosed During Pregnancy

Abstract Disclosure: A. Rosado-Burgos: None. L.N. Madera Marin: None. M.A. Ortiz-Rivera: None. L. El Musa Penna: None. W. Medina-Torres: None. L.R. Sepulveda-Garcia: None. M. Alvarado: None. L.A. Gonzalez-Rodriguez: None. M. Ramirez: None. Introduction: Primary adrenal insufficiency (AI) that is a potentially life-threatening condition and commonly associated to destruction of adrenal cortex by autoantibodies. AI predominantly affects women of reproductive age and can affect the outcome and course of pregnancy, causing reduced parity, risk of preterm birth, and low birth weight. Symptoms of AI are nonspecific and can result in a challenging diagnosis. Clinical case: 25 year-old pregnant female G1P0A0 at 12 weeks gestational age (WGA) with long standing history of Type 1 Diabetes Mellitus (DMT1) and Hypothyroidism. She was evaluated while hospitalized for metabolic control during pregnancy. The patient had been previously admitted at 7 WGA with hyperglycemic episodes requiring insulin dosing adjustments. Now, 5 weeks later, was found with numerous hypoglycemic episodes even after omitting pre-prandial insulins. Additionally, she had persistent fatigue, general malaise, nausea, vomits, anorexia, and difficulty concentrating. Laboratory workup showed a TSH of 22.96 uIU/mL (nl 0.4-4.0), FreeT4 0.91 (nl 0.89-1.74), that had been within normal limits 6 weeks prior. She reported taking pre-natal vitamins only one hour after LT4, therefore malabsorption was suspected. Insulin requirements were decreased by 50%. Vitals signs without evidence of hemodynamic instability and laboratory workup without electrolyte nor acid base disturbances. However, due to clinical presentation and history of other autoimmune diseases, AI was suspected. Severe hypothyroidism, AI and hyperemesis gravidarum can mimic several features of each other’s clinical presentation. Midmorning cortisol level was found at 7.8 ug/dl (nl for pregnancy >19) but no ACTH levels taken prior to replacement. Considering acuteness and severity of symptoms it was decided to administer IV LT4 equivalent dose which can increase cortisol metabolism precipitating adrenal crisis. For this reason, stress dose hydrocortisone (HC) was empirically administered. Rapid clinical improvement was observed after LT4 and corticosteroid replacement therapy with resolution of nausea, vomits, anorexia as well as hypoglycemic episodes and was discharge home with her usual LT4 PO dose and corticosteroid replacement therapy. Twenty-one-alpha hydroxylase antibodies were positive, making the diagnosis of primary adrenal insufficiency highly suspected. Conclusion: Presentation was consistent with Polyglandular Autoimmune Syndrome Type 2. Diagnosis of AI during pregnancy is rare as it is considered a hypercortisolemic state. Changes in cortisol levels during pregnancy make the diagnosis of AI even more challenging. Dynamic testing can be considered, however, most of these studies have not been validated in pregnancy. In this case patient continues with HC replacement therapy while in her 3rd trimester of pregnancy without any maternal of fetal complications. Presentation: Saturday, June 17, 2023

SAT155 Insulin Receptor Autoantibody Syndrome (Iras): A Rare Occurrence In Lymphoproliferative Disease

Abstract Disclosure: C. Villatoro Santos: None. E.A. Oral: None. Insulin receptor antibody syndrome (IRAS) may present as fasting hypoglycemia, high serum insulin, and serum autoantibodies to insulin receptor at low titers. We present a case of a patient with multiple myeloma (MM) who developed hypoglycemia suspected to be related to insulin receptor antibodies. The patient is a 60-year-old male with history of prediabetes, MM, anemia, and thrombocytopenia treated with carfilzomib, dexamethasone 20 mg weekly, and prior radiation to the right pelvis, who presented to the hospital for increased right hip pain and cough. Patient reported 4 days of markedly decreased appetite with poor intake. He noted increased chronic right hip and low back pain. On exam, he was cachectic, had hyperpigmentation under bilateral eyes, and muscle wasting. Hypoglycemia as low as 38 mg/dL (70-180 mg/dL) was noted. MM studies on hospital day 4 showed increased kappa-M protein and kappa-free light chains with worsening anemia and thrombocytopenia, concerning for progression of his disease. He later developed recurrent hypoglycemia; endocrinology was consulted on hospital day 6. He also had hyponatremia and borderline low blood pressure. Cortisol on hospital day 7 at 9:00 am was 15.4 μg/dL (5.3-22.5 μg/dL) in the setting of normoglycemia. On hospital day 9, hematology started dexamethasone 40 mg daily for 3 days for MM relapse, which resulted in marked improvement in his mental status, appetite, and resolution of hypoglycemia. On hospital day 11, he started weekly cyclophosphamide, bortezomib, plus dexamethasone for 3 weeks. Results for hypoglycemia workup returned on hospital day 12 from samples of Day 7: glucose 53 mg/dL (70-180 mg/dL), C-peptide 0.2 ng/mL (1-5.2 ng/mL), insulin 14.6 μU/mL (1-21 μU/mL), insulin antibodies undetectable, negative serum screen for oral hypoglycemic agents. His course was complicated by sepsis with gram-negative bacteremia treated with antibiotics and stress dose hydrocortisone. He was discharged to rehab on hospital day 34 on dexamethasone 4 mg daily. Glucose, c-peptide and insulin levels could be consistent with exogenous hyperinsulinemic hypoglycemia but may also represent delayed insulin clearance due to insulin receptor antibodies and autocrine suppression of pancreatic insulin production in a patient with no access to insulin. Therefore, the most likely diagnosis was IRAS. The insulin antibody radioactive essay was negative for native IgG and IgM antibodies; testing for insulin receptor antibodies is in progress as a clinical test is unavailable. Treatment is common with high doses of steroids. IRAs can be seen in patients with lymphoproliferative disorders and would respond to steroid treatment for hospital management. Definitive treatment should be tailored with oncology guidance. There is a need for an accessible clinical test for IRAS in the US. Presentation: Saturday, June 17, 2023

THU147 Peritoneal Dialysis To Improve Hyperglycemic Hyperosmolar State In Child With Renal Failure

Abstract Disclosure: P. Choudhari: None. S. Mootha: None. Background: Hyperglycemic hyperosmolar state (HHS) without ketosis is rare in children. While the crux of management of HHS is extensive hydration followed by insulin, this management can be difficult for children with kidney failure. Clinical Case: A 6-year-old female with complex medical history including Jeune syndrome (asphyxiating thoracic dystrophy), end stage renal disease status post kidney transplant, currently with rejection, now on peritoneal dialysis (PD), and history of steroid induced hyperglycemia was admitted for altered mental status. Several days prior to the current admission, she was admitted for an upper respiratory infection and found to have hyponatremia, which improved with fluid restriction and sodium supplementation. Subsequent worsening of hyponatremia led to adjustments in her PD to include higher amounts of dextrose in the dialysate (1.5% to 2.5%). At the current admission, she was found to have a blood glucose of 2096 mg/dL, uncorrected sodium of 121 mg/dL, corrected to 153 mg/dL, serum osmolality of 406 mOsm/kg, and hemoglobin A1c of 11.3%. She had mild acidemia and beta-hydroxybutyrate of 0.5 mmol/L. She was transferred to the Pediatric Intensive Care Unit (PICU) and intubated given her waxing and waning mental status and complex history. Since she was anuric, fluid resuscitation was carefully managed. She received a total of 14 mL/kg normal saline boluses, which lowered blood glucose by about 240 mg/dL. Multidisciplinary conversations between PICU, Endocrinology, and Nephrology culminated with a plan to attempt to slowly reduce blood glucose levels via PD prior to using regular insulin infusion. 24-hour PD was restarted with lower dextrose in the dialysate (1.5%). Due to persistent hypotension, she required norepinephrine infusion and stress dose hydrocortisone. With PD, blood glucose levels decreased by 20-30 mL/kg/hr in the first day. Regular insulin infusion was started, while continuing 24-hour PD, after blood glucoses stagnated at 1400-1500 mg/dL. After 4-5 days of PD and insulin infusion, the child’s blood glucoses decreased to 300-400 mg/dL. After tolerating her g-tube feeds, she was transitioned to an insulin pump as she was requiring high doses of insulin (1.8 units/kg/hr). Conclusion: We present a child with complex medical history, including history of steroid induced hyperglycemia, end stage renal disease status post renal transplant, anuria, currently on PD, who developed hyperglycemic hyperosmolar state (HHS). PD helped to lower her significant hyperglycemia, initially without insulin. There are few case reports on the use of PD or hemodialysis to improve hyperglycemia or hypernatremia in children with renal failure. This case demonstrates dialysis can be an effective strategy to attempt for children in a hyperosmolar state in whom aggressive fluid management can be detrimental. Presentation: Thursday, June 15, 2023

FRI344 Is It Possible To Have Two Endocrinopathies From Checkpoint Inhibitor Therapy At The Same Time?

Abstract Disclosure: A.N. Davis: None. G. Elshimy: None. Introduction: Checkpoint inhibitor (CPI) therapy with ipilimumab and nivolumab has become commonly used in patients with metastatic melanoma. Endocrinopathies, including hypophysitis and thyroiditis, have been reported as adverse effects. These endocrinopathies may occur with ipilimumab alone, but the incidence increases in combination therapy. Most of the cases present with one endocrinopathy at a time. We present a case highlighting the possibility of two endocrinopathies presenting simultaneously in patients treated with ipilimumab and nivolumab. Case: A 69-year-old female with stage IV choroidal melanoma presented to the ED on 7/12/22 with a sudden new onset headache for one week. CT head without contrast was unremarkable, and she was discharged home. She had been started on combination CPI therapy with ipilimumab and nivolumab on 5/26/2022, and her last dose was on 7/30/2022. An MRI brain with and without contrast was obtained by her Hematology-Oncology provider for worsening headaches on 8/15/2022 which revealed an eccentric left sellar hypo-enhancing mass that was not seen on the previous MRI brain performed on 5/24/2022. On 8/31/2022, the patient returned back to the ED with complaints of fever, weakness, altered mentation, and diarrhea. She was noted to be febrile and tachycardic with hypotension refractory to intravenous fluids. She was transferred to the medical ICU and started on a norepinephrine drip. Prior to the initiation of norepinephrine, her cortisol level was 2.40 mcg/dL with ACTH level of <5.0 pg/mL. The remainder of labs were significant for TSH <0.010 mcIU/mL, free T4 3.26 (0.58-1.76ng/dL), free T3 4.59 (2.30-4.20 pg/mL), LH 1.27 (15.9-54.0 mIU/mL), FSH 12.76 (23.0-116.3 mIU/mL), prolactin 0.56 (1.8-20.3 ng/mL), TSI <1.0 (<1.3), TRAb <1.10 (<1.75 IU/L), TPO Ab 0.5 (<9.0 IU/mL). Labs were consistent with hypopituitarism and thyrotoxicosis. She was started on stress dose hydrocortisone which was weaned to a PO maintenance dose prior to discharge. An MRI brain pituitary protocol was obtained on 9/5/2022 which revealed the same hypoattenuating sellar lesion which was slightly smaller than on the previous MRI. The patient’s presentation was attributed to pituitary apoplexy in the setting of preceding hypophysitis as well as thyroiditis. Her hypophysitis and thyroiditis were attributed to her treatment with ipilimumab and nivolumab. On 11/22/2022, free T3 was 1.83 pg/mL, free T4 was <0.25 ng/dL, and TSH was 2.538 mcIU/mL, consistent with resolved thyroiditis but developing central hypothyroidism. Discussion: CPI therapy is associated with the development of endocrinopathies including thyroiditis and hypophysitis. Our case demonstrates that it is possible for multiple endocrinopathies to present simultaneously in the setting of CPI therapy and that delay in diagnosis can precipitate adrenal crises. Given the frequency with which endocrinopathies can occur with CPI therapy, appropriate screening and follow-up are recommended in order to provide prompt diagnosis and management of these patients. Presentation: Friday, June 16, 2023

SAT325 Recurrent Pericardial Effusion As Initial Presentation Of Primary Adrenal Insufficiency

Abstract Disclosure: S. Ward: None. A.L. Champion: None. A. Mrkaic: None. Recurrent Pericardial Effusion as Initial Presentation of Primary Adrenal Insufficiency: Background: Primary adrenal insufficiency (PAI) is a rare but serious endocrinopathy. High clinical suspicion is important to avoid delay in diagnosis and treatment. We present a rare case of recurrent pericardial effusion as initial presentation. Clinical Case: 63 year old male with hypertension and diabetes presented to ED complaining of SOB and chest pain. Workup revealed pericardial effusion causing cardiac tamponade treated with emergent pericardiocentesis. He was admitted to ICU for hypotension. He was also diagnosed with COVID-19 and acute kidney injury. Labs showed hyperkalemia ranging from 5.3 to 7.2 mmol/l (n= 3.5-5.1 mmol/l) and hyponatremia 132 mmol/L (n= 136-145 mmol/L). He was started on dexamethasone for COVID-19 infection. Etiology of pericardial effusion was unclear; infectious disease workup was unremarkable, cytology showed no malignant cells, autoimmune panel was negative. Initial echo showed EF >55%. After initiation of steroids, symptoms improved with normalization of electrolytes and improvement of hypotension, however was discharged on midodrine 2.5 mg TID. Seven days after discharge patient called 911 complaining of worsening shortness of breath. He was found to be severely hypotensive with BP 67/40. Echo showed moderate to large pericardial effusion causing tamponade. Emergent pericardial window was performed. Labs showed hyponatremia 123 mmol/L and potassium ranging from 4.9-5.4 mmol/L. Patient remained hypotensive requiring pressors despite pericardial effusion drainage. With persistent hypotension, hyperkalemia, and hyponatremia there was strong suspicion for adrenal insufficiency and workup was consistent with PAI. Labs showed 8:00 am cortisol 2.7 ug/dl (n= 3.7-19.4 ug/dl), ACTH 256.0 pg/ml (n=7.2-63.3pg/ml), aldosterone <1.0 ng/dL, renin 4.216 ng/mL/hr (n= 0.167-5.380 ng/mL/hr). Due to his critical illness patient was started on stress dose hydrocortisone 50 mg Q6 hr. With initiation of steroids, hyponatremia, hypokalemia, and hypotension resolved. Patient was weaned to physiological doses of hydrocortisone and started on fludrocortisone at time of discharge. Conclusion: Timely diagnosis of PAI is essential and can be lifesaving, if untreated it can lead to adrenal crisis with high mortality. Having a high clinical suspicion is crucial to a prompt diagnosis. Pericardial effusion should prompt clinical suspicion for PAI as multiple cases have now been reported in the literature. Our patient has multiple potential causes of pericardial effusion including PAI, acute kidney injury, and COVID-19 infection. Although we cannot show definitive causation from PAI, it most certainly contributed and prompt diagnosis and treatment may have prevented recurrence. It is important to raise awareness of PAI as a differential in pericardial effusion. Presentation: Saturday, June 17, 2023

The Hypothalamus-pituitary-adrenocortical Response to Critical Illness: A Concept in Need of Revision.

Based on insights obtained during the past decade, the classical concept of an activated hypothalamus-pituitary-adrenocortical axis in response to critical illness is in need of revision. After a brief central hypothalamus-pituitary-adrenocortical axis activation, the vital maintenance of increased systemic cortisol availability and action in response to critical illness is predominantly driven by peripheral adaptations rather than by an ongoing centrally activated several-fold increased production and secretion of cortisol. Besides the known reduction of cortisol-binding proteins that increases free cortisol, these peripheral responses comprise suppressed cortisol metabolism in liver and kidney, prolonging cortisol half-life, and local alterations in expression of 11βHSD1, glucocorticoid receptor-α (GRα), and FK506 binding protein 5 (FKBP51) that appear to titrate increased GRα action in vital organs and tissues while reducing GRα action in neutrophils, possibly preventing immune-suppressive off-target effects of increased systemic cortisol availability. Peripherally increased cortisol exerts negative feed-back inhibition at the pituitary level impairing processing of pro-opiomelanocortin into ACTH, thereby reducing ACTH-driven cortisol secretion, whereas ongoing central activation results in increased circulating pro-opiomelanocortin. These alterations seem adaptive and beneficial for the host in the short term. However, as a consequence, patients with prolonged critical illness who require intensive care for weeks or longer may develop a form of central adrenal insufficiency. The new findings supersede earlier concepts such as "relative," as opposed to "absolute," adrenal insufficiency and generalized systemic glucocorticoid resistance in the critically ill. The findings also question the scientific basis for broad implementation of stress dose hydrocortisone treatment of patients suffering from acute septic shock solely based on assumption of cortisol insufficiency.

Electrolyte abnormalities and stress dosing predict illness-related hospitalizations among infants and toddlers with congenital adrenal hyperplasia.

Infants and toddlers with classical congenital adrenal hyperplasia (CAH) are at high risk for morbidity/mortality arising from life-threatening adrenal crisis. Management of acute illnesses in CAH requires an understanding of factors leading to emergency department (ED) visits and hospitalizations in the first few years of life. We, therefore, examined adrenal crisis at prehospital and ED stages of illness in young children with CAH as they related to medical outcomes. Retrospective cohort study of 39 children with CAH due to 21-hydroxylase deficiency (0-4 years of age) and 27 age-matched controls. ED visit, acute illness symptoms (fever, vomiting, diarrhoea) and other characteristics (hospitalizations, administration of stress-dose hydrocortisone, electrolyte abnormalities). CAH infants and toddlers had significantly higher rates of ED visits (0.50 [0.25-0.88] per person-year) than controls (0 [0-0] per person-year; p < .001). Moreover, CAH children under 6 months old had significantly higher rates of ED visits compared with older ages. Only 50% (51/102) of illness-related ED visits in CAH children were preceded by the administration of either oral (46/51) or intramuscular (11/51) stress dosing by parents. A total of 10.8% of ED visits resulted in hospital admission. Controlling for age and 17-hydroxyprogesterone at diagnosis, electrolyte abnormalities and administration of parenteral hydrocortisone in the ED significantly predicted hospital admission. Receiving a hydrocortisone injection before the ED was a significant predictor of having electrolyte abnormalities. Infants and toddlers with classical CAH are at high risk for acute illness and hospitalizations and often do not receive adequate stress dosing before the ED.