bluuebunny/crossref_metadata_2025_split huggingface dataset:
12516 Bilateral Adrenalectomy And Bariatric Surgery For Hormonal And Metabolic Control Of Classic Congenital Adrenal Hyperplasia
Authors: Palú Gabriele , Maria Rodrigues Adriane 
Published: October 2024

Abstract
Disclosure: G. Palú: None. A.M. Rodrigues: None.
Background: Hyperandrogenism is sometimes difficult to control in female patients with classic congenital adrenal hyperplasia (CAH), requiring supra-physiologic glucocorticoid dose with its metabolic consequences. Clinical Case: A 25-year woman with salt-losing CAH has been followed since she was born, requiring high glucocorticoid dose (hydrocortisone dose equivalent 50-75 mg), in addition to fludrocortisone (0.1-0.15 mg/day), without control of hyperandrogenism (testosterone and androstenedione levels more than 3 times ULN). She progressively gained weight, reaching 116 kg (256 pounds) and BMI of 46 kg/m2 when she was 17 years old. Bilateral adrenalectomy was performed in 2016 for disease control and to lower glucocorticoid dose. Androgen levels were supressed, glucocorticoid dose was reduced to 5mg prednisone (hydrocortisone dose equivalent 20mg) and she lost 10 kg of weight, but she remained in class III Obesity (BMI 42.5 kg/m2). Serious adrenal crises were not reported after bilateral adrenalectomy. Pharmacologic obesity treatment was done (sibutramine plus topiramate) and she lost 16 kg of weight, reaching 90 kg and a BMI of 36 from April 2017 to February 2020. She stopped treatment during the COVID- 19 pandemic and regained weight, reaching 106 kg again. She was lost to follow-up and returned in November 2023. In May 2022, she was submitted to a gastric bypass in a country town. She achieved 71 kg with a BMI of 28. She was feeling very well, taking post-bariatric vitamins and minerals, combined oral contraceptive, prednisone 5 mg/day (hydrocortisone dose equivalent 20 mg/day) and fludrocortisone 0.1 mg/day. Until now, she did not have any adrenal crises requiring parenteral glucocorticoid use. Conclusion: Even though treatment was aggressive with two surgeries that could lead to increased risk of adrenal crises, the patient had a benign course. New medicines that block CRH or ACTH action in CAH patients could prevent excessive glucocorticoid doses and weight gain and would be a better alternative to bilateral adrenalectomy in cases such as this one.
Presentation: 6/1/2024
7178 Human loss-of-function variants in the TRPC5 associated with Postpartum Depression
Authors: Li Yongxiang , Farooqi Ismaa Sadaf , Xu Yong 
Published: October 2024

Abstract
Disclosure: Y. Li: None. I.S. Farooqi: None. Y. Xu: None.
Postpartum depression is a serious psychiatric disorder that is understudied and underdiagnosed. Postpartum depression, the most common complication of childbirth negatively impacts the mother, with suicide accounting for approximately 20% of postpartum deaths. Further, postpartum depression also has adverse effects on infant behavioral, emotional, and cognitive development. However, the neurobiological mechanisms underlying postpartum depression remain to be comprehensively elucidated. Transient receptor potential channel 5 (TRPC5/Trpc5), an ion channel that conducts calcium inward currents, as a critical regulator of many behaviors. Here, we found that microdeletions on chromosome Xq23 disrupting TRPC5 gene in humans and identified seven rare TRPC5 variants in mothers who exhibited postpartum depression and reduced bonding with their infants. Heterozygous females with TRPC5 deletions had postpartum depression and anxiety after delivery. We then generated a knock-in mouse model of a human severe loss-of-function mutation, K34del (Trpc5K34del). Our study showed that Trpc5K34del/+ heterozygous dams abandoned their pups after delivery. The Trpc5K34del/+ dams also showed inefficient retrieval and increased the time to retrieve pups, which is associated with abnormal pup care. Importantly, Trpc5K34del/+ dams exhibited severe depression-like and anxiety-like behaviors during the postpartum period. Oxytocin (OXT), a polypeptide comprised of 9 amino acids, is primarily synthesized by neurons in the paraventricular nucleus of the hypothalamus (PVH) and the supraoptic nucleus (SON). We found that endogenous Trpc5 protein is abundantly expressed by OXT neurons in PVH but not by SON. Further, the Trpc5K34del/+ dams reduced OXT expression in the PVH but does not affect OXT expression in the SON. Deletion of Trpc5 in OXTPVH neurons recapitulated phenotypes in Trpc5K34del/+ dams, including inefficient retrieval, abnormal pup care, depression-like and anxiety-like behaviors during the postpartum period. We then bilaterally injected AAV-DIO-Trpc5 into the PVH of virgin female Trpc5K34del/+/OXT-Cre mice to result in restoration of Trpc5 only in OXTPVH neurons in these mutant mice. Restoration of Trpc5 in OXTPVH neurons in Trpc5K34del/+/OXT-Cre mutant mice significantly improved pup retrieval behavior and indices of maternal care. After weaning, Trpc5K34del/+/OXT-Cre dams with Trpc5 restoration in OXTPVH neurons showed reduced depression-like and anxiety-like behaviors. Our study indicates that the postpartum depression and impaired maternal behavior of Trpc5 deficient female mice are mediated by impaired function of OXTPVH neurons. This revelation not only offers crucial insights into the pathogenesis of postpartum depression but also provides a foundation for potential therapeutic interventions.
Presentation: 6/2/2024
8231 Hyperprolactinemia Post-Brain Radiation
Authors: Kalas R , Manohar V , Bapat N S , Kim Y 
Published: October 2024

Abstract
Disclosure: R. Kalas: None. V. Manohar: None. N.S. Bapat: None. Y. Kim: None.
Hormonal dysfunction in adults is common after brain radiotherapy, especially when the hypothalamus or the pituitary gland is within the field of radiation. Following cranial radiotherapy, it is crucial to routinely monitor the emergence of hormonal imbalances, with particular attention to the somatotroph-GH axis, which is most frequently affected. Notably, hyperprolactinemia may manifest in 5%-50% of individuals following radiation. The degree and extent of the hormonal disorder can depend on radiation dose and technique, and the length of time after radiation. We report a case of hyperprolactinemia occurring five years after radiotherapy for cavernous sinus schwannoma. A 44-year-old woman with a history of cavernous sinus schwannoma status post right pterional craniotomy and radiation therapy 5 years prior presented with a 4-month-history of secondary amenorrhea. She underwent pencil beam scanning proton radiation to the right orbital apex and cavernous sinus for a dose of 5040 cGy in 28 fractions. Review of systems was negative for hot flashes, breast tenderness, or galactorrhea. Labs revealed: diluted prolactin 118 (4.8-23.3 ng/mL), monomeric prolactin 99.6 (4.79-23.3 ng/ml), estradiol 184 pg/ml, LH 10.5 mIU/mL, FSH 2.9 mIU/mL, serum beta-HCG &lt;5 mIU/mL, TSH 2.630 (0.450-4.500 mIU/L), free T4 0.85 (0.82-1.77 ng/dL), IGF-1 104 ( 74-239 ng/mL), and ACTH 17.8 (7.2-63.3 pg/mL) with morning cortisol 14.0 (6.2-19.4 ug/dL). A brain MRI with and without contrast revealed stable mass with postsurgical changes in the right cavernous sinus extending to the orbital apex and no abnormalities of the pituitary gland when compared to 2 years ago. The patient was started on cabergoline 0.5 mg twice a week. Her menses resumed four weeks after starting treatment, and the prolactin level repeated one month after was &lt;1.0 ng/mL. The proposed mechanism for radiation-induced hyperprolactinemia is damage to the hypothalamus resulting in reduction of dopamine, an inhibitory neurotransmitter. It is typically dose dependent, and for the majority, it is subclinical occurring years after radiation. It can develop in both sexes and all ages but most commonly amongst young women. Hyperprolactinemia can sometime self-resolve, potentially due to evolving damages to lactotrophs. We emphasize that while brain radiotherapy can frequently lead to hormonal deficiencies, hyperprolactinemia can occur and eventually lead to dysfunction in the LH/FSH axis. Pituitary function should be routinely checked for many years post-radiation, as the likelihood of developing hormonal dysfunction tends to increase with time.
Presentation: 6/1/2024
8144 Hypertension in Sheehan Syndrome: A Diagnostic Challenge
Authors: Ahsan E , Foo F F , Lesniak C , Akula M , Hu K , Ong R , Chalasani K , Cheng J , Holland S 
Published: October 2024

Abstract
Disclosure: E. Ahsan: None. F.F. Foo: None. C. Lesniak: None. M. Akula: None. K. Hu: None. R. Ong: None. K. Chalasani: None. J. Cheng: None. S. Holland: None.
Introduction: Postpartum pituitary necrosis, commonly known as Sheehan syndrome (SS), is the term used to describe the necrosis of anterior pituitary gland cells that occurs after significant postpartum bleeding, hypovolemia, and shock. This typically occurs after delivery, during which the mother suffers significant blood loss. The pituitary gland fails to produce hormones due to this blood loss. Depending on the degree of tissue damage, SS can have an acute or chronic course, and patients may present with a wide range of clinical symptoms at diagnosis. Acute SS is associated with postpartum pituitary infarction, which can result in hypotension, shock, hypoglycemia, hyponatremia, headache, vision disturbances, unconsciousness, and an inability to lactate. We present a rare case of SS where the patient experienced hypertension instead of classic hypotension due to postpartum preeclampsia. Clinical Case: A 40-year-old female with no significant medical history presented to the hospital for scheduled induction of labor for polyhydramnios and underwent vacuum-assisted vaginal delivery and postpartum hysterectomy due to severe postpartum hemorrhage secondary to DIC. The postpartum course was also complicated by preeclampsia with severe features. Patients developed features like excessive fatigue, hypoglycemia, hyponatremia, hypothyroidism, polyuria, and failure of lactation, which were consistent with panhypopituitarism secondary to Sheehan syndrome. However, the patient was hypertensive to 145/91 before steroids were given. Labs were significant for serum sodium 116 mmol/L (135-145 mmol/L), FSH &lt;0.7 mIU/mL (Follicular Phase: 2.5-10.2, Mid-cycle Peak: 3.1-17.7, Luteal Phase: 1.5-9.1, Postmenopausal: 23.0-116.3), LH &lt;0.2 mIU/m (Follicular Phase: 1.9-12.5, Mid-Cycle Peak: 8.7-76.3, Luteal Phase: 0.5-16.9, Postmenopausal: 10.0-54.7), Prolactin: 2.4 ng/mL (Non-pregnant: 3.0-30.0, Pregnant: 10.0-209.0, Postmenopausal: 2.0-20.0), PM Cortisol on admission: 1.10 ug/dL (3.44-16.76 ug/dL), plasma ACTH 7 pg/mL( 6 - 50 pg/mL), TSH 0.430 uIU/mL (0.550 - 4.780 uIU/mL), FT4 0.59 ng/dL - 1.76 ng/dL). MRI of the brain pituitary showed- A 1.5 cm region of hypoenhancement and an additional 2 mm focus of hypoenhancement in the sella, findings represent evolving pituitary necrosis. The patient received 24 units of blood transfusion and hormone replacement therapy, followed by outpatient endocrinology follow-up. Clinical lessons: Although considerable blood loss causes hypotension in patients with SS, additional postpartum medical issues can sometimes result in atypical presentations. In our patient, postpartum preeclampsia caused hypertension. Identifying the atypical symptoms of SS is just as vital as identifying its typical features to prevent potentially fatal consequences.
Presentation: 6/2/2024
12623 Hypopituitarism Secondary To Empty Sella Syndrome Post Motor Vehicle Accident
Authors: Fawcett D , Ceballos S , Livasky C 
Published: October 2024

Abstract
Disclosure: D. Fawcett: None. S. Ceballos: None. C. Livasky: None.
Introduction: Empty sella is a pituitary disorder characterized by the herniation of the subarachnoid space within the sella turcica. It can be further classified as primary empty sella (PES), considered an idiopathic disease or secondary empty sella (SES) which may result after pituitary tumor treatment, spontaneous necrosis, infection, autoimmune disease or brain trauma. Case Description: 47-year-old female with no medical history, who presented to emergency due to a 2-week history of fatigue associated with persistent heart pounding sensation and intermittent holocranial headache. On admission, the patient had sinus tachycardia with a normal blood pressure, and a non-focal neurologic exam. Her initial labs were significant for elevated lactic acid and low TSH. Initially a hyperthyroid etiology was considered, however, thyroid panel obtained on admission showed low T4 level. On further interrogation patient recalled a recent motor vehicle accident (MVA) a month prior to symptoms developing, without medical evaluation at the time. Brain MRI was completed as part of a central etiology work up demonstrating an empty sella with enlargement of the sella turcica measuring 1.6 cm. No contrast enhancing lesions were seen. Endocrinologic work up was consistent with a secondary adrenal insufficiency. Patient was discharged with hormonal thyroid and hydrocortisone supplementation. Discussion: Empty sella is a pituitary disorder in which a herniation of the subarachnoid space within the sella turcica occurs. There are two types: Primary empty sella (PES) which is considered idiopathic and is seen in patients with no history of previous pituitary pathology, and Secondary empty sella (SES) which may result after pituitary tumor treatment such as surgery or radiation, spontaneous necrosis, infection, autoimmune disease or brain trauma. The patient we presented did not have medical history consistent with autoimmune diseases or prior procedures. However, she did report an MVA with head trauma, that did not receive medical attention with subsequent development of headache and rest of previously described symptoms, highly concerning for a possible traumatic etiology. A high level of suspicion is required for diagnosis, particularly in patients with unspecific symptoms in order to improve patient’s prognosis.
Presentation: 6/1/2024
8043 IgG4-related disease with isolated involvement of the pituitary gland: case report
Authors: Ramos Jayme Mendonça 
Published: October 2024

Abstract
Disclosure: J.M. Ramos: None.
Female patient, 36 years old, presented to our service complaining of a headache lasting five weeks, mainly in the frontal and left periorbital region, radiating to the occipital region. The pain was intense and intermittent, lasting about 40 minutes. No nausea or vomiting was reported, but the patient experienced dizziness and double vision. During the symptomatic interview, the patient mentioned increased thirst and urinary volume. In her medical history, she denied comorbidities, with only frequent episodes of tension-type headaches. She had no continuous medication use and no known drug allergies. The patient had undergone two cesarean sections, with the last one in December 2021. On physical examination, she had horizontal binocular double vision in all three gazes to the left, accompanied by pain during eye movement. Increased facial sensitivity on the left in branches V1 and V2 was observed. The rest of the physical examination showed no abnormalities. Initial general laboratory tests were normal. Evaluation of pituitary function was mostly normal, except for slightly elevated prolactin. A cranial MRI revealed an expansive lesion with sellar and suprasellar components measuring 1.2x1.1x1.2cm. Additional imaging of the sella turcica showed an intra- and suprasellar expansive lesion, predominantly isointense on T1, hyperintense on T2, with discreetly heterogeneous enhancement with gadolinium. In consultation with the neuroradiology team, a hypointensity on T2 at the left margin of the pituitary was noted, suggesting an inflammatory etiology. Based on this, laboratory tests were conducted, and treatment with methylprednisolone 1g per day for 7 days was initiated. After treatment completion, the IgG4 level was found to be 146.7 mg/dL (upper limit of reference: 86 mg/dL). A follow-up MRI, five days after the steroid pulse therapy, showed a reduction in lesion size to 0.8 cm in the cranio-caudal dimension, with a preserved appearance of the optic chiasm. The patient experienced considerable symptom improvement and was discharged with oral corticosteroids and a scheduled initiation of rituximab. The patient meets criteria confirming the diagnosis of IgG4-related hypophysitis, a rare condition representing 30% of hypophysitis cases. Isolated pituitary involvement is even rarer, and its association with diabetes insipidus has been described. Treatment involves corticosteroids, and rituximab can be considered, especially in recurrent cases.
Presentation: 6/3/2024
8565 Impact of Mild Blast Traumatic Brain Injury on Pituitary Hormone Secretion Trajectory in Male and Female Mice in Response to Restraint
Authors: Donaldson H , Wu T Y J 
Published: October 2024

Abstract
Disclosure: H. Donaldson: None. T.J. Wu: None.
Over 10 million people suffer from traumatic brain injury (TBI) annually. Mild blast TBI (mbTBI) account for about 80% of TBIs in service members. One third of people exposed to mbTBIs have prolonged neuroendocrine defects, particularly anterior pituitary dysfunction. Previous studies in our laboratory have shown that male and female mice demonstrate different neuroendocrine defects in response to mbTBI. Specifically, male mice with mbTBI have an increase (p&lt;0.05) in corticosterone (CORT) response at 30 min after the initiation of restraint. Conversely, female mice show attenuated (p&lt;0.05) levels of CORT at the same timepoint after the initiation of restraint. It is not known, however, what the trajectory of anterior pituitary hormone changes is in response to mbTBI. Our project aims to characterize the temporal relationship of peripheral anterior pituitary hormones in male and female mice in response to mbTBI and restraint. Male and female mice were randomly assigned to undergo sham or mbTBI injury using the Advanced Blast System (ABS). At 1 week after mbTBI, mice within each group were randomly assigned to be killed at 0-, 5-, 10-, 20-, 30-, or 90-min after the initiation of a 20-min restraint. At the appropriate timepoint, tissue was collected after the mice were euthanized. Prolactin (PRL) and adrenocorticotropin hormone (ACTH). were measured using commercially available ELISA. Analysis of the data show that the peak response to restraint was altered in mice exposed to the ABS. PRL increased (p&lt;0.05) starting at 5 min in both males and females after the initiation of restraint and returned to baseline by 90 min. ACTH increased (p&lt;0.05) starting at 10 min and 20 min after the initiation of restraint and returned to baseline by 90 min in males and females, respectively. Peak PRL was delayed in both males (20 min vs 5 min) and females (10 min vs 5 min) whereas peak ACTH advanced in both males (10 min vs 30 min) and females (20 min vs 30 min). Area under the curve analysis show that ABS decreased overall PRL in both males and females whereas ACTH was lower in males and higher in females. The results of the current study showed that mice of both sexes have different serum PRL and ACTH trajectories in response to restraint when exposed to mbTBI.
Presentation: 6/2/2024
7940 Insulinoma as a Rare Manifestation of MEN4
Authors: Han Hye Jeong , Moalem Jacob , Shih Angela R , Gigliotti Benjamin J 
Published: October 2024

Abstract
Disclosure: H. Han: None. J. Moalem: None. A.R. Shih: None. B.J. Gigliotti: None.
Background/Objective: Multiple endocrine neoplasia 4 (MEN4) is a rare syndrome that is caused by germline mutations in CKDN1B. Its phenotypic expression is similar to multiple endocrine neoplasia 1 (MEN1). Manifestations include primary hyperparathyroidism (PHPT), pituitary adenomas, and pancreatic neuroendocrine tumors (NET). Prevalence of MEN4 is less than one per million. Case Report: A 55-year-old female with history of hypercalcemia presented with symptomatic hypoglycemia. She had a fasting plasma glucose of 41mg/dL, proinsulin of 84.3 pmol/L, insulin level of 24 uIU/mL, c-peptide of 5.2 ng/mL, and beta hydroxybutyrate level of 0.34 mmol/L, consistent with endogenous hyperinsulinism. She was incidentally noted to have PHPT. CT abdomen showed a 1.5 x 1.1 x 1.0 cm pancreatic lesion consistent with an insulinoma. Pathology from subsequent Whipple surgery showed that it was a well-differentiated neuroendocrine tumor with no metastasis. She became normoglycemic after surgery. Germline testing revealed a variant of unknown significance in CDKN1B (p.R93W). Discussion: Increasing evidence suggests that MEN4 exhibits subtle differences in penetrance and natural history compared to MEN1. A recent systemic review of MEN4 cases showed that most gastrointestinal NETs in MEN4 are non-functional NETs and gastrinomas; to date, no insulinomas have been reported. PHPT in MEN4 may exhibit a lower risk of recurrence after parathyroidectomy. This case highlights the importance of germline genetic testing when a patient presents with clinical manifestations of MEN1. Conclusion: This is the first reported case of insulinoma in MEN4.
Presentation: 6/3/2024
7044 It Is Back, Three Decades Later! When To Stop Glucagonoma surveillance?
Authors: Wahba A T , Dillon J S 
Published: October 2024

Abstract
Disclosure: A.T. Wahba: None. J.S. Dillon: Advisory Board Member; Self; Crinetics Pharmaceuticals, CA.
A 76-year-old woman presented to the Neuroendocrine Tumor (NET) Clinic for evaluation of sore mouth, perianal area and itchy ankles. She had a medical history notable for glucagonoma, status post distal pancreatectomy and splenectomy in 1988, 32 years prior to her current presentation. She recalled that she had CT imaging post operatively for a few years, but no more recent imaging. She was diagnosed with diabetes mellitus in approximately 2002, with highly variable glucose control. She developed a left leg DVT with pulmonary emboli one year prior to presentation. Shortly after her thromboembolic events, she noted her current presenting complaints. A CT abdomen scan with contrast was reported as normal. Three months later, an abdomen ultrasound for evaluation of right upper quadrant pain and intractable vomiting, showed a liver mass. MRI revealed a contrast enhancing 3.1x2.1x1.8 cm lesion in liver segment 6 and 7. Her plasma Glucagon concentration, which had been undetectable when last checked in 2007, was 382 (N: &lt; 80 pg/ml). CT-guided needle biopsy of the liver lesion confirmed a well differentiated neuroendocrine tumor, immunohistochemically positive for somatostatin receptor 2 (SSTR2A), Islet-1 and PAX6, with low mitotic count and Ki-67 proliferation index of 0.45%. Gallium-68 DOTATATE-PET scan showed a single avid hepatic metastasis, with no other evidence of somatostatin receptor expressing tumor. She was initially started on a combination of short-acting Octreotide 100 mcg TID and monthly 20 mg Sandostatin LAR injections for symptomatic relief. Zinc copper 100 mg capsules were prescribed with significant improvement of skin rash and glossitis within 2-4 months. Repeat MRI abdomen at 3 months and 6 months showed a stable single liver lesion without evidence of additional metastasis. She has continued annual clinic visits and abdomen MRI follow up with stable imaging findings at 3 years. Percutaneous ablation, trans hepatic arterial embolization and surgical resection were discussed, but the patient preferred a more conservative approach of monthly octreotide LAR, which she tolerated well, and which resolved her symptoms. Glucagonoma syndrome presents classically with 5D’s: Dermatitis (Necrolytic Migratory Erythema), DVTs, Diabetes mellitus, Depression, Diarrhea. The National Comprehensive Cancer Network guidelines recommend monitoring glucagonoma patients post-resection for up to 10 years with clinical evaluation, glucagon levels every 6-12 months and imaging studies as clinically indicated. Our case illustrates that recurrence can occur after 30 years from initial resection and that longer surveillance should be considered.
Presentation: 6/3/2024