bluuebunny/crossref_metadata_2025_split huggingface dataset:
6600 Hyperkalemia Shortly After Surgical Treatment For Primary Hyperaldosteronism
Authors: Hernandez Hernandez M , Colon Castellano J 
Published: October 2024

Abstract
Disclosure: M. Hernandez Hernandez: None. J. Colon Castellano: None.
Primary hyperaldosteronism was thought to be a rare cause of secondary hypertension in the United States. However, recent epidemiologic data estimates that approximately 5-15% of patients with a diagnosis of hypertension are due to hyperaldosteronism. This number may be higher due to patients being widely underdiagnosed. Most of these patients can be cured with a surgical approach. Patients who undergo adrenalectomy for primary hyperaldosteronism need to be closely monitored for electrolyte disturbances. Post-adrenalectomy hyperkalemia in hyperaldosteronism is thought to be secondary to resultant hypoaldosteronism caused by suppression of the juxtaglomerular apparatus on the contralateral adrenal gland secondary to the volume-expanded state before unilateral adrenalectomy. It has been reported in a subset of patients and it is a known cause of increased morbidity and mortality as patients may present with severe hyperkalemia. We present the case of a 63-year-old male patient with medical history of hypertension who was diagnosed with primary hyperaldosteronism when he presented with hypokalemia in routine laboratories performed after an elective laparoscopic cholecystectomy. The patient’s laboratories were remarkable for potassium levels at &lt;2mEq/L, normal sodium levels, and further workup obtained which was remarkable for plasma aldosterone concentration: plasma renin activity (PAC/PRA) at 216. The medication regimen at home included Lisinopril 40mg p.o. daily, Nifedipine 60mg p.o. daily, and oral potassium replacement. The patient was consulted to the Endocrinology service which recommended further imaging studies. Abdomino-pelvic CT scan was remarkable for a right adrenal adenoma measuring 1.5 cm with 24 Hounsfield units. The patient was initially managed with aldosterone antagonists and then a right adrenalectomy was performed successfully after adrenal vein sampling. Approximately two weeks after the adrenalectomy, the patient was called back to the Emergency Room (ER) due to hyperkalemia 6.5mEq/L. Upon arrival at the ER, there were no EKG changes associated with hyperkalemia, and he was given appropriate treatment for potassium levels. The patient was started on Fludrocortisone with excellent clinical response and is currently being followed at Endocrinology clinics. The etiology of this patient’s hyperkalemia soon after adrenalectomy demonstrates the effect of relative hypoaldosteronism from chronic suppression of the left adrenal gland due to aldosterone excess produced by the right adrenal adenoma. Fludrocortisone is a synthetic mineralocorticoid and it has potent effects in reducing potassium levels mediated by sodium reabsorption and potassium excretion at the distal renal tubules. Early treatment in these patients is crucial to prevent the development of electrolyte disturbances that may ultimately lead to life-threatening arrhythmias.
Presentation: 6/2/2024
6462 A Case of Presumed IgG4 Related Hypopituitarism
Authors: Lai William , Turtz Alan , Traisak Pamela , Morgan Farah Hena 
Published: October 2024

Abstract
Disclosure: W. Lai: None. A. Turtz: None. P. Traisak: None. F.H. Morgan: None.
Introduction: Hypopituitarism is a rare disorder of decreased hormone secretion with a prevalence of 0.05% [1]. It is exceedingly rare to be caused by pituitary inflammation such as IgG4 mediated plasmacytic cell invasion hypophysitis which accounts for 4% of all hypopituitarism cases [2]. We present a challenging case of presumed IgG4 hypophysitis using Leporti criteria. Case: A 79 year old male with a medical history of hypertension, hypothyroidism, and mitral valve repair presented to the hospital for syncope, fatigue, and hypotension. Blood work done was significant for a low cortisol of 0.8 µg/dL (5-25 µg/dL), ACTH of 5 pg/mL (10-60 pg/mL), TSH of 0.120IU/mL (0.5-5IU/mL), and FT4 of 0.89ng/dL(0.7-1.9ng/dL). Prolactin and IGF1 were unremarkable. He was started on hydrocortisone 10 mg in the morning and 5 mg in the evening with improvement in symptoms. On follow up with Endocrinology, an MRI revealed an intrasellar/ suprasellar mass measuring 18 mm craniocaudal, sellar component of 11x9 mm, and suprasellar component 13x9mm with effacement and superior displacement of the optic chiasm raising the question of hypophysitis, and an incidental finding of a parotid mass. IgG4 testing was sent with results elevated at 377 mg/dL. PET scan showed uptake in the pituitary gland, right parotid gland, as well as mediastinal and hilar lymphadenopathy. Biopsy of the right parotid lesion showed pleomorphic adenoma. Patient deferred biopsy of hilar lymph nodes.The patient’s steroids were changed to prednisone to treat likely IgG4 mediated hypophysitis and repeat MRI after 4 weeks of treatment showed a reduction in the size of the pituitary lesion. He was subsequently referred to rheumatology for further evaluation of systemic disease. Additional work up including SPEP, Cryoglobulins, ANA IFA, ANCA, C3, C4, SSA, SSB, ACE, and immunoglobulin levels were negative. Conclusion: IgG4 related hypopituitarism is a rare diagnosis that is difficult to diagnose. In this case a definitive diagnosis with histological sampling was not obtained but a working diagnosis of IgG4 related hypophysitis was made based on clinical presentation, MRI findings, an elevated IgG4 &gt; 140, and response to treatment. He ultimately fulfilled the diagnostic criteria proposed from Leporti et al [3]: MRI with sellar mass, IgG4 &gt; 140mg, and steroid responsive decrease in size of the pituitary lesion, with concomitant improvement of symptoms. This case highlights the difficulty in diagnosis of IgG4 hypophysitis and the benefit of using non histological diagnostic criteria.
Presentation: 6/1/2024
12352 A Case Of Sellar Pituitary Cyst With Elevated Prolactin In A 74-Year-Old Woman
Authors: Tkach Marko S , Pinero-Pilona Antonio 
Published: October 2024

Abstract
Disclosure: M.S. Tkach: None. A. Pinero-Pilona: None.
A 74-year-old woman diagnosed with a pituitary mass, described as a prolactinoma, presented for follow-up care with elevated prolactin (“PL”). Initial diagnosis of a prolactinoma was 46 years prior to presentation. Prior imaging was not available. She received bromocriptine for 31 years; as PL increased to 90 on a drug holiday, bromocriptine was resumed for over a decade. After that time, PL decreased to 23, 11 years later. She was switched to cabergoline after reporting a “stuffy nose”. With PL up to 37.8 two years later, cabergoline was increased to 0.5 mg one tablet twice a week. PL subsequently dropped to 8.2 over the next two years. Upon presentation, she reported no brain imaging for “over 30 years”. She denied galactorrhea, headache, sweating, nausea, weight change, severe hypertension, hypotension, diabetes, hirsutism, fatigue, or muscle weakness. She denied visual field changes, or any formal visual field testing for over 20 years. Exam on initial presentation was unremarkable except for a diffuse goiter. Initially ordered labs were unremarkable, though A1c was 6.2%. Undiluted PL was 6.0 (confirmed on 1:100 dilution). Another PL was measured at 4.6 total and 3.8 monomeric. TSH was 1.47. Thyroglobulin antibodies and TPO were undetectable. On pituitary MRI, "the pituitary gland shows a partially empty sella turcica which is asymmetrically slightly outwardly convex on the right, possibly representing an occult arachnoid cyst versus cystic adenoma. There is a midline pituitary stalk. No parasellar abnormalities.” Scalloping of the right sellar portion was noted. Cystic changes in the pituitary in this case may be related to cystic prolactinoma, arachnoid cysts, or cystic degeneration of a prolactinoma after long-term treatment with a dopamine agonist. Cystic prolactinomas have been termed “transitional cell tumors of the pituitary” as they possess both Rathke cleft cyst and prolactinoma features. Reduced efficacy of response to medical as compared to surgical therapy in these patients has been observed. They also tend to lead to lower PL levels than solid prolactinomas, complicating diagnosis. Arachnoid cysts (“AC”) are collections of CSF within the arachnoid membrane: present in about 1.7% of the population, they account for about 1% of intracranial masses. Although an uncommon location, sellar AC have been previously described and are estimated to comprise about 3% of AC. Nonfunctioning pituitary masses or AC with stalk compression can cause elevated PL; these respond to dopamine agonist therapy, though it is not considered definitive therapy in the case of a nonfunctioning adenoma. This case represents the difficulties in management and transition of long-term care and treatment of prolactinomas as well as assessing pituitary radiological findings.
Presentation: 6/1/2024
7229 A Rare Case of Igg4 Hypophysitis Presenting as Hypogonadotropic Hypogonadism
Authors: Dimech Christina , Jaiswal Gayatri 
Published: October 2024

Abstract
Disclosure: C. Dimech: None. G. Jaiswal: None.
Hypophysitis is a rare pituitary disorder including a broad range of inflammatory processes which affect the pituitary gland and infundibulum. Previously reported as 1 per 9 million individuals, the true incidence of hypophysitis per year is largely unknown due to the increase of novel etiologies such as immunoglobulin G4 (IgG4)- related disease. IgG4-related hypophysitis (IgG4-RH) was first clinically described in 2004 and later biopsy proven in 2007. The main clinical features are elevated serum IgG4 level and IgG4-positive lymphocyte infiltration of tissues on histopathology. IgG4-RH is reported to account for 1.3% of primary hypophysitis with 3.2:1 incidence ratio of men to women, in the 6th decade of life. Here we present a case of a 68-year-old Caucasian male who presented for evaluation of hypogonadotropic hypogonadism. As fasting morning testosterone levels were significantly low, MRI pituitary was completed which revealed a homogenously enhancing pituitary gland with thickened, nodular enhancement of the pituitary infundibulum. Subsequent lab work-up revealed normal function of remaining anterior pituitary without evidence of central vasopressin deficiency. Evaluation of underlying etiologies was unremarkable except for significantly elevated IgG4 levels (472mg/dL). Based on the significantly elevated IgG-4 levels (&gt;135 mg/dl) and the radiographic findings of the pituitary infundibulum, the diagnosis of possible IgG4-RH was made. The patient was initiated on treatment with rituximab and glucocorticoid therapy with prednisone. Following 3 months of medical therapy, MRI pituitary showed resolution of stalk inflammation, confirming diagnosis of IgG4- RH.
Presentation: 6/1/2024
6591 Effects of Levoketoconazole on 24-hour Mean UFC (mUFC) in the SONICS Study: Relation to Baseline mUFC in Adults with Cushing’s Syndrome: a Post-hoc Analysis
Authors: Huang Robbie , Harper Dawn , Meyer James , Cohen Fredric M 
Published: October 2024

Abstract
Disclosure: R. Huang: Employee; Self; Xeris Pharmaceuticals. D. Harper: Employee; Self; Xeris Pharmaceuticals. J. Meyer: Employee; Self; Xeris Pharmaceuticals. F.M. Cohen: Consulting Fee; Self; Xeris Pharmaceuticals.
Background: Prior exploratory analyses of the open-label, non-randomized, single-arm Phase 3 SONICS study of levoketoconazole suggested that the mUFC response rate at 6 months varied by baseline mUFC stratum. That exploration focused on the subset of the study population that had completed Dose-Titration phase and advanced into the 6-month Maintenance phase. We now expand this exploration to include all enrolled patients in SONICS who were treated and had a postbaseline mUFC, aiming to further elucidate relationships between baseline biochemical disease severity, drug dose, and intermediate-term mUFC response. Methods: 94 adults with confirmed Cushing’s syndrome were enrolled to receive levoketoconazole treatment at a starting dose of 150 mg BID, titrated as needed at 150 mg intervals until a maximum dose of 600 mg BID or an adequate response was achieved in the investigator’s judgment. Following Dose-Titration phase, eligible patients immediately entered a Maintenance phase of approximately 6 months, during which the dose could be changed only to regain biochemical control or for safety reasons. The primary endpoint was the proportion of patients in the ITT population with mUFC normalization at end of the 6-month Maintenance phase, without a dose increase during maintenance. For the current analyses, 92 patients treated with levoketoconazole and with baseline mUFC measurement (modified ITT) were stratified into 3 baseline mUFC subgroups—Group 1: 1.5x to ≤2.5x upper limit of normal (ULN); Group 2: &gt;2.5x to ≤5x ULN; Group 3: &gt;5x ULN and analyzed with respect to mUFC response, average daily dose, and adverse events following 6 months of Maintenance therapy. Results: Group 2 had the highest apparent mUFC response rate (12/33 [36.4%]); 95% CI 0.20, 0.54) as compared with Group 1 (12/38 [31.6%]; 95% CI 0.16, 0.47) or Group 3 (5/21 [23.8%]; 95% CI 0.01, 0.55); Group 3 having a notably lower response. Daily doses of levoketoconazole were related to baseline mUFC. Thus, Group 3 used a nominally higher average daily dose during Maintenance therapy and at the end of the 6-month Maintenance phase (680 mg and 741 mg) than Group 1 (508 mg and 566 mg) or Group 2 (617 mg and 611 mg). Group 3 had more liver-related adverse events of special interest than Group 1 or 2 (14% vs 7.9% or 3.0%) and more AEs leading to discontinuation (24% vs 12% or 16%). Conclusion: In this post-hoc analysis, lower mUFC at baseline predicted the likelihood of achieving mUFC normalization following 6 months of levoketoconazole Maintenance therapy and was associated with lower Maintenance dose requirements and lower rates of potentially clinically important liver-related AEs.
Presentation: 6/1/2024
7900 Growth Hormone Secretion Disruption Leads to Sex Dimorphic Effects on Mices Reproduction
Authors: Alves Guilherme Andrade , Frazao Renata 
Published: October 2024

Abstract
Disclosure: G.A. Alves: None. R. Frazao: None.
Growth hormone (GH) is required for sexual maturation and sex steroid mediation of the ovulatory cycle. In humans and animal models, late puberty onset and infertility can be seen in cases of GH secretion disruption. Hypothalamic nuclei containing either kisspeptin or leptin receptor-expressing neurons, required for puberty onset and fertility, are GH-responsive. The selectively induced ablation of GH receptors from Kiss1-expressing cells in female mice is not required for puberty onset despite the downregulation of Kiss1 and Gnrh1 hypothalamic expression in pubertal animals. In contrast, the lack of GH signaling in leptin receptor-expressing cells leads to late sexual maturation but no modulation of Kiss1 or Gnrh1 hypothalamic levels in female mice. The impact of disrupted GH secretion throughout the development on hypothalamic areas associated with reproductive function remains unclear. To determine whether GH secretion disruption differentially affects female and male mice reproduction, we selected mice that hypersecrete GH (here denominate as bGH) or with spontaneous GH deficiency (Ghrhrlit/lit), as well as their littermates (control) for the experiments. We recorded mice’s body weight gain and sexual maturation during development, as well as body length and hypothalamic mRNA expression at the adult stage. We further assess the estrous cycle length of female mice and the fertility rate of male and female animals. As predicted, male and female bGH mice showed increased body weight gain during development and increased nasoanal length compared to control mice. The Ghrhrlit/lit male and female mice showed decreased body weight gain and lower nasoanal length than control mice. bGH and Ghrhrlit/lit female mice showed similar puberty timing to control mice, whereas bGH and Ghrhrlit/lit male mice exhibited a late puberty onset. Interestingly, bGH female mice exhibited disrupted estrous cycles and were infertile, whereas Ghrhrlit/lit female mice showed no differences in estrous cycle length compared to control animals and were fertile. While it is known that Ghrhrlit/lit male mice are subfertile, bGH male mice exhibited no fertility deficit. In contrast to control mice, bGH mice showed upregulated AgRP mRNA expression levels, whereas Pomc expression levels were downregulated, despite no differences in Kiss1, Gnrh1, Esr1, and Tac3 mRNA expression. AgRP and Pomc levels were downregulated in Ghrhrlit/lit females, whereas Tac3 and Ghrh mRNA expression were upregulated compared to control animals. Our findings suggest a GH-induced sexually dimorphic modulation of the HPG axis. Adequate GH signaling seems required for puberty transition in male mice, while GH hypersecretion impairs female mice's fertility despite not affecting puberty onset. Whether changes in metabolism or a specific class of neurons contribute to the observed effects needs further investigation.
Presentation: 6/3/2024
8793 Bilateral Pheochromocytoma Related To Multiple Endocrine Neoplasia Syndrome 2
Authors: Compas Frantzcess , Samir Desai Neesha , Moalem Jacob , Harbuz-Miller Inga 
Published: October 2024

Abstract
Disclosure: F. Compas: None.
Background: Pheochromocytomas are rare catecholamine secreting neuroendocrine tumors of the adrenal medulla that overproduce metanephrines. These tumors may occur sporadically or arise from several genetic disorders including Multiple Endocrine Neoplasia Type 2 (MEN 2). Symptoms of a pheochromocytoma are hypertension, palpitations, anxiety, headache, diaphoresis, and tremors. Bilateral pheochromocytomas are exceedingly rarer compared to unilateral cases. Clinical Case: A 30-year-old male without significant past medical history initially presented to his primary care physician at the age of 18 with episodic palpitations that occurred at least once a week during exercise. Over the years the palpitations progressed and were accompanied by intermittent chest pain. Family history included a mother with thyroid nodules who died from multiple organ failure. Extensive cardiac evaluation revealed hypertension, leading to beta-blocker initiation. He underwent further testing for causes of secondary hypertension. Shortly after he presented to the emergency room with sudden onset aphasia, confusion, and combativeness. CT scan was performed which revealed posterior reversible encephalopathy syndrome. Abdominal imaging from secondary hypertension workup revealed bilateral adrenal masses. Urine metanephrines revealed a metanephrine of 67,950 mcg/24 hr and urine normetanephrine of 27,150 mcg/24 hr. He was admitted to the hospital. In preparation for bilateral adrenalectomy alpha blockade was initiated followed by beta blockade. He underwent a bilateral adrenalectomy. Pathology from adrenalectomy revealed that bilateral masses consistent with a bilateral pheochromocytoma. Shortly afterwards he was diagnosed with medullary thyroid cancer. Genetic testing revealed that this patient had MEN 2a syndrome. Discussion Bilateral pheochromocytomas from MEN 2a syndrome are exceptionally rare, accounting for a small percentage of all cases. They present more severe and cause fluctuating hypertension due to the simultaneous release of catecholamines from both adrenal glands. This case highlights the importance of considering rare causes of hypertension and the implications of delayed diagnosis and treatment. Conclusion: This is an exceedingly rare case of a bilateral pheochromocytoma implicated by MEN 2a that went undetected for years and presented with life threatening complications.
Presentation: 6/2/2024
7411 Hypothalamic Estrogenic Signal Regulates Prolactin Homeostasis and Reproduction in Female
Authors: Yu Meng , Wang Chunmei , Xu Yong 
Published: October 2024

Abstract
Disclosure: M. Yu: None. C. Wang: None.
Both 17β-estradiol (E2) and prolactin (PRL) are important reproductive hormones. It has been well established that E2 can act on estrogen receptor-α (ERα) in the lactotroph cells in the pituitary to upregulate PRL level. This mechanism may explain the high PRL and high E2 during proestrus phase or pregnancy, however, it could not explain high PRL but low E2 singling during lactation. We found that central supplement of E2 reduced PRL level in lactating dams, suggesting a novel inhibitory role of E2 on lactational hyperprolactinemia. To explore the mechanism and the relationship with reproductive regulation, firstly we compared the activity of ERα-expressing neurons in the hypothalamus during virgin and lactation. We found that ERα neurons in the ventrolateral subdivision of the ventromedial hypothalamus (ERαvlVMH) were inhibited during lactation. Further, we specifically deleted ERα from vlVMH in virgin females. These mice with deletion of ERαvlVMH showed dramatic changes: 1) lactation-like hyperprolactinemia and prolactinoma, 2) developed mammary gland and amenorrhea, and 3) disrupted reproduction including enlarged uterus and reduced fertility. Consistently, we specifically activated ERαvlVMH neurons in lactating mice and found that these lactating mice showed attenuated lactational hyperprolactinemia and impaired maternal cares. Together, we revealed a novel neuroendocrine mechanism that hypothalamic E2-ERα signaling regulate PRL homeostasis, which is important to maintain reproduction in females. Our study also supports that lactational suppression of hypothalamic E2-ERα signaling is required to maintain normal lactation which may contribute to reduced fertility during lactation.
Presentation: 6/1/2024