bluuebunny/crossref_metadata_2025_split huggingface dataset:
FRI359 Postprandial Hypoglycemia With Elevated Pro-insulin As A Rare Presentation Of Insulinoma
Authors: Kalik Salina , Dehghani Shabnam , Zaidan Julie 
Published: October 2023

Abstract
Disclosure: S. Kalik: None. S. Dehghani: None. J. Zaidan: None.
Background: Insulinoma, a neuroendocrine neoplasm, typically presents with fasting hypoglycemia with abnormally elevated insulin levels but can infrequently present with post-prandial hypoglycemia with elevated pro-insulin levels. Clinical Case: A 49-year-old non-diabetic male, presented with a 3-year history of episodes of blurry vision, sweating, and palpitations associated with low finger stick glucose (45-70mg/dL). Episodes primarily occurred 2-3 hours post-meals particularly after eating a high carbohydrate meal. Symptoms improved after taking glucose tabs consistent with Whipple’s triad. He denied fasting hypoglycemia symptoms. Patient had symptoms at the office 2 hours after eating breakfast and Lab work done at that time showed glucose &lt;35 of mg/dL (70-99 mg/dL), inappropriately high-normal c-peptide level of 3.57 ng/mL (0.81-3.85 ng/mL), elevated pro-insulin level of 12.8 pmol/L (&lt;8.0 pmol/L), and a high insulin level of 29.8 uIU/mL (3-25.0 uIU/mL). Insulin antibodies and Sulfonylurea screen were negative. Review of old imaging revealed unremarkable pancreas on Ct scan done three years ago and a month prior to presentation. Given persistent symptoms and lab results Patient underwent an endoscopic ultrasonography with advanced gastroenterology which showed a 14.2 x 11mm hypoechoic, well circumscribed, regularly shaped and homogenous mass at the pancreatic head that was sampled using fine needle biopsy. Pathology confirmed pancreatic neuroendocrine neoplasm, low grade, consistent with insulinoma. Patient went for MRI of abdomen and pelvis which confirmed a 1.4 x 1.2cm slightly T2 hyperintense, hyper-enhancing lesion in the uncinate process suspicious for islet cell tumor. In addition, a 0.6cm liver lesion was identified although not clearly characterized but small metastatic lesion could not be excluded. PET/CT scan did not reveal any metastatic lesions except the focal uptake at the pancreatic uncinate process. Patient was evaluated by surgery and underwent a successful Whipple procedure for resection of the insulinoma. Discussion: 20% of all insulinomas are misdiagnosed with a seizure disorder or psychiatric disorder before an insulinoma is recognized. This case highlights the fact that insulinoma can be present as post prandial hypoglycemia and the superior imaging modality of EUS and MRI for localizing and identifying smaller functional insulinomas. Conclusion: Insulinoma can present as postprandial hypoglycemia with elevated pro-insulin levels. If high clinical and biochemical suspicion for insulinoma, patient should be evaluated with EUS or MRI even in the setting of a negative CT due to higher diagnostic specificity for insulinoma.
Presentation: Friday, June 16, 2023
THU167 Trends Of Recombinant Human Growth Hormone Treatment In Korea: Data From The Health Insurance Review And Assessment Service 2015-2020
Authors: Kim Jaehyun , Rhie Young-Jun , Kim Yoo-Mi , Lee Hae Sang , Lee Young Ah , Chae Hyun Wook , Choi Jin-Ho , Chung Sochung , Kim Hyoungseok , Son Yunjeong , Han Seungju , Hwang Il-Tae 
Published: October 2023

Abstract
Disclosure: J. Kim: None. Y. Rhie: None. Y. Kim: None. H. Lee: None. Y. Lee: None. H. Chae: None. J. Choi: None. S. Chung: None. H. Kim: None. Y. Son: None. S. Han: None. I. Hwang: None.
Objectives: Recombinant human growth hormone (rhGH) has been used for treating diseases associated with short stature for several decades worldwide and in Korea. The trends in prescribing rhGH in children and adolescents in Korea were evaluated using the nationwide claim data. Methods: Data from the Health Insurance Review and Assessment Service (HIRA) between 2015 and 2020 were used. The nationwide claim data on the prescription of rhGH for approved indications were extracted from the HIRA database and analyzed. Off-label use of rhGH was not investigated. Results: The number of all patients treated with rhGH increased from 5,812 in 2015 to 16,248 in 2020. The number of newly treated patients with rhGH was from 1,763 in 2015 to 5,177 in 2020. Among children and adolescents with short stature, those who were prescribed rhGH increased from 1.9% in 2016 to 5.7% in 2020. The overall proportion of the indications for rhGH treatment was 55% for growth hormone deficiency (GHD), 27% for idiopathic short stature (ISS), 13% for small for gestational age (SGA), and 5% for Turner syndrome. From 2015 to 2020, the increment in newly treated patients was highest in GHD, followed by ISS and SGA. In all indications, male to female ratio decreased during the period: 1.52 in 2016 and 1.30 in 2020 for GHD; 1.18 in 2016 and 0.91 in 2020 for ISS; and 1.13 in 2016 and 1.03 in 2020 for SGA. Expenditure for claims to rhGH also increased from 25.9 billion KRW in 2015 to 62.5 billion KRW in 2020. The mean age at the start of rhGH and the mean discontinuation age was 7.8 years (boys 8.0 years and girls 7.7 years) and 12.0 years (boys 12.2 years and girls 11.8 years), respectively. The mean treatment duration was 3.2 years. Conclusion: In Korea, the prescription of rhGH in the pediatric population showed a 2.8-fold increase in number and a 2.4-fold increase in expenditure over 6 years according to nationwide HIRA data. Boys were treated more with rhGH than girls, whereas the discrepancy decreased. Long-term follow-up for prescription patterns, effectiveness and safety in rhGH treatment is required.
Presentation: Thursday, June 15, 2023
FRI598 Evolving Growth Hormone Deficiency: A Proof Of Concept
Authors: Chimatapu Sri Nikhita , Sethuram Swathi , Samuels Julie , Savage Martin , Rapaport Robert 
Published: October 2023

Abstract
Disclosure: S. Chimatapu: None. S. Sethuram: None. J. Samuels: None. M. Savage: None. R. Rapaport: None.
Introduction: Most cases of growth hormone deficiency (GHD) in pediatric patients are idiopathic, isolated and acquired. All acquired conditions commence with a period during which the diagnosis maybe unapparent. We document here evidence for evolution of GHD in a group of adolescent males who tested sufficient on initial GH stimulation test (GST) but tested deficient upon reevaluation. The rationale for repeat GST was ongoing growth failure. Hypothesis: We hypothesize that GHD in some individuals evolves with time and can be diagnosed by repeat testing (EGHD). We also postulate that their response to GH therapy is similar to other patients with GHD. Methods: We performed an IRB-approved retrospective chart review of children who presented for short stature (height less &lt; 2SD for mean or &lt; 2SD for mid-parental height) to pediatric endocrinology at Kravis Children’s Hospital at Mount Sinai, New York and who had 2 GST. GST were performed using two different stimuli (arginine and glucagon) and highly sensitive assay serum GH measurements were processed by the same laboratory, Esoterix. No patient had obesity, chronic illnesses, or any genetic conditions affecting growth. Data were collected from electronic medical records and analyzed using SPSS v28.0 once at least half the population studied reached adult height. We defined adult height as bone age ≥ 16 years and growth velocity ≤ 2 cm/year. Results: Of 42 charts analyzed, all male, 25 had EGHD. Their average GH peak on initial GST was 15.48 ± 4.92 ng/ml, mean age 10.07 ± 2.65 years, mean height -1.68 ± 0.56 SD , IGF-1 -1.00 ± 0.88 SD. After 2.23 ± 1.22 years, at 12.04 ± 2.41years, height decreased to -1.82 ± 0.63 SD and IGF-1 was -1.08 ± 0.84 SD. At repeat GST, GH peak was 7.59 ± 2.12 ng/mL and 28% were in puberty. All patients had MRI before starting GH therapy; 40% had small anterior pituitary glands. 12 males who reached adult height had a height of -1.88 ± 0.64 SD, IGF-1 of -1.15 ± 0.81 SD, with 42% pubertal and peak GH of 7.33 ± 2.47 ng/mL at repeat GST. They had a mean height gain of 1.83 ± 0.56 SD(p&lt;0.005), with an adult height of 0.09 ± 0.70 SD after 4.64 ± 1.4years of treatment. They were 2.10 ± 4.20 cm taller than their mid-parental target heights. Discussion/Conclusions: We provide proof of concept for the diagnosis of EGHD. The diagnosis could be established by repeat GST in children with ongoing growth deceleration despite pubertal progression and a non-diagnostic initial GST. Our patients who reached adult height had a height gain consistent with that reported by KIGS for patients with growth hormone deficiency (1.7SD; Cutfield,1999) and indeed better than patients with ISS (0.7SD; Maghnie,2022).
Presentation: Friday, June 16, 2023
FRI388 Follicle-Stimulating Hormone And Salt-inducible Kinases Crosstalk On The Regulation Of Genome-wide Gene Expression In Human Granulosa Cumulus Cells
Authors: Hayes Emily , Filzen Rachael , Esquivel Miriam Rodriguez , Winston Nichola , Fierro Michelle , Sana Salih , Stocco Carlos Oscar 
Published: October 2023

Abstract
Disclosure: E. Hayes: None. R. Filzen: None. M. Rodriguez Esquivel: None. N. Winston: None. M. Fierro: None. S. Sana: None. C.O. Stocco: Grant Recipient; Self; NIH R01HD097202.
Infertility affects approximately 15% of couples, 40% are due to ovulatory defects. Successful ovulation requires the timely and tight regulation of folliculogenesis by gonadotropins, more crucially, follicle-stimulating hormone (FSH), which is needed to form preovulatory follicles. However, the mechanisms governing folliculogenesis are incompletely understood. We previously showed that salt inducible kinases (SIKs) are critical regulators of female fertility. Specifically, knockout of SIK2 enhances ovulation, whereas deletion of SIK3 results in anovulation and premature ovarian failure. At the molecular level, SIK2, but not SIK3, attenuates the expression of FSH-induced steroidogenic genes. This report aimed to outline the genome-wide effect of SIK inhibition on gene expression in the presence or absence of FSH. Here, we treated human cumulus cells with a SIK inhibitor, HG-9-91-01 (HG), FSH, or their combination. We then performed RNA sequencing and identified 3,472 differentially expressed genes (DEGs) across treatment groups. Interestingly, 498/646 (77%) of DEGs in FSH-treated cells were also identified as DEGs in HG-treated cells. Further, HG + FSH resulted in 865 DEGs not differentially regulated by FSH or HG alone. Analysis of DEGs by Gene Set Enrichment Analysis (GSEA) revealed top enriched gene ontology terms including processes related to the extracellular matrix, steroid hormone biosynthesis, PI3K-Akt signaling, and cAMP signaling. The data suggest that SIK activity profoundly affects gene expression in human granulosa cells, likely via regulation of FSH signaling and other mechanisms. These findings provide new insights and directions for investigating the mechanisms by which SIKs regulate ovarian function and fertility.
Presentation: Friday, June 16, 2023
FRI398 A Case Of Secondary Amenorrhea: Is It Only PCOS To Blame?
Authors: Panjawatanan Panadeekarn , Riahi Shayan , Chaidarun Sushela S 
Published: October 2023

Abstract
Disclosure: P. Panjawatanan: None. S. Riahi: None. S.S. Chaidarun: None.
Background: One of the most common causes of secondary amenorrhea is PCOS (polycystic ovary syndrome) which accounts for 30% of the cases. Though not as common, hyperprolactinemia similarly contributes to the condition. Here we describe a patient who presented with secondary amenorrhea from medication-induced hyperprolactinemia with underlying PCOS.
Clinical Case: A 31-year-old female with a past medical history of bipolar disorder, hypothyroidism, and PCOS, presented with secondary amenorrhea. The patient started menarche at 11 years old and was diagnosed with PCOS a year later due to irregular menses and hirsutism. The estrogen-containing pill was prescribed, but she developed a blood clot due to Factor V Leiden thrombophilia. She was taking atomoxetine, lithium, mirtazapine, and olanzapine for her psychiatric issues and recently started on paliperidone last year. She reported no period in the past two years after stopping oral contraception. Pregnancy was ruled out. She tried progesterone challenge test but did not have any flow. Transvaginal ultrasound showed thin endometrium. She had IUD placed for contraception. Laboratory testing four months and one year after starting paliperidone showed prolactin of 79.2 ng/mL and 110 ng/mL consecutively. The patient reported headaches but denied galactorrhea or visual loss. There are no signs or symptoms of Cushing’s disease, acromegaly, or hypothyroidism. Further workup showed TSH 1.52 mcIU/mL, estradiol 16 pg/mL, FSH 5.6 mIU/mL, 17-OH progesterone &lt;40 ng/dL, total testosterone 14 ng/dL, ACTH 11 pg/mL, IGF-1 z-score -0.67 SD. MRI brain was ordered, which ruled out pituitary adenoma. Repeat prolactin level remained elevated at 87.7 ng/mL without treatment. We concluded that the patient has secondary amenorrhea from PCOS and antipsychotic-induced hyperprolactinemia. Patient was referred to a psychiatrist for antipsychotic medication changes.
Conclusion: Antipsychotics, typically first-generation, are known to cause hyperprolactinemia due to their dopamine-antagonist effect. Prolactin level ranging from 25-100 ng/mL is seen in medication-induced hyperprolactinemia, while particular medication like risperidone and phenothiazines can raise the level to more than 200 ng/mL. Newer agent paliperidone has been reported to induce hyperprolactinemia similarly to risperidone which explains significant prolactin elevation in this patient. Hyperprolactinemia poses a risk for hypogonadism due to suppressed pituitary-gonadal axis, resulting in anovulation and bone loss. Estrogen therapy is considered an adjunct to help with hypogonadism. However, it is contraindicated in patients with thrombophilia. The guideline recommends switching to a low-risk antipsychotic agent if feasible. Despite having an established diagnosis of PCOS, it is important to consider other secondary causes of amenorrhea which may occur concomitantly.
Presentation: Friday, June 16, 2023
SAT422 Gender Affirming Hormone Therapy in a Non-Binary Individual with Swyer Syndrome
Authors: Yohannan Jiby M , Reisman Tamar 
Published: October 2023

Abstract
Disclosure: J.M. Yohannan: None. T. Reisman: None.
Background: Swyer syndrome is a rare genetic disorder of sexual development. Patients with Swyer syndrome require physiologic hormone replacement congruent with their gender identity in order to prevent bone loss. It is also important to screen for complications of this condition including osteopenia and dysgerminomas. Clinical Case: A 25 year old individual with a history of Swyer syndrome (46 XY gonadal dysgenesis) presents to clinic to discuss initiation of gender affirming hormone therapy. The patient identifies as non-binary and is interested in starting testosterone for masculinization.
The patient was initially diagnosed with Swyer syndrome at age 23 during an evaluation for primary amenorrhea. At that time the patient had a laparoscopic gonadectomy and was found to have a dysgerminoma. Upon diagnosis the patient was started on hormone replacement therapy with norethindrone acetate-ethinyl estradiol 1 - 0.01 mg for bone loss prevention. They had a bone mineral density scan two years prior to presentation that was reportedly normal. To avoid the deleterious effects of hypogonadism they were started on estradiol-norethindrone 0.05 - 0.25 mg patch to provide bone protection. They were also provided testosterone gel for masculinization as part of their gender affirming care. Swyer syndrome is a disorder of sex development in which individuals with XY chromosomes develop phenotypically female Mullerian structures (uterus, fallopian tubes, and vaginal canal). Individuals with Swyer syndrome typically have non-functional “streak gonads” and are diagnosed during evaluation for primary amenorrhea. While some cases may be from a novel mutation, there are case reports showing that the condition can have an autosomal dominant, autosomal recessive, or even X-linked pattern of inheritance depending on the mutated gene. Patients with Swyer syndrome require physiologic doses of hormone replacement for maturation of secondary sex characteristics and bone health. For this patient in particular it is also important to provide appropriate hormone replacement congruent with their gender identity. Additionally, approximately 30% of patients with this condition may develop a gonadal tumor, including dysgerminomas. Because of this, patients frequently have their gonads removed upon diagnosis. Conclusion: Patient’s with Swyer syndrome should be on appropriate hormone replacement as they do not make endogenous sex steroids. Additional consideration of masculinizing hormone replacement should be made for patients that have a gender identity other than cisgender female. It is also important to screen patients for gonadal tumors as there is an increased incidence in this population.
References: King, Thomas F.J.; Conway, Gerard S. Swyer syndrome. Current Opinion in Endocrinology & Diabetes and Obesity: December 2014 - Volume 21 - Issue 6 - p 504-510, doi: 10.1097/MED.0000000000000113
Presentation Date: Saturday, June 17, 2023
SAT009 Investigating The Role Of Hepatic Small Heterodimer Partner In Regulating Gallstone Formation
Authors: Dang Nathanlown Mun , Anakk Sayeepriyadarshini 
Published: October 2023

Abstract
Disclosure: N.M. Dang: None. S. Anakk: None.
Nuclear receptor, Small Heterodimer partner (Shp) regulates bile acid homeostasis by inhibiting transcription of Cholesterol 7 alpha-hydroxylase (Cyp7a1), the rate-limiting enzyme involved in converting cholesterol to bile acids, within the liver. Bile acid regulation prevents the formation of gallstones as cholesterol is excreted into bile along with bile acids and phospholipids. How hepatic Shp affects the development of gallstones and the mechanisms underlying these effects remain unclear. Here we used a liver-specific knockout mouse model and a lithogenic diet model to uncover potential liver-to-gallbladder crosstalk that contributes to gallstone development. Previously, we had shown that the knockout of Shp alters bile acid composition, resulting in higher percentages of secondary bile acid, deoxycholic acid. This increased the hydrophobic index of hepatic bile acids and also implicated a trend for a subsequent increase in the hydrophobicity of biliary bile. Higher hydrophobicity is sufficient to increase susceptibility to developing gallstones by promoting cholesterol nucleation. Of note, the expression of transporter genes Abcg5 and Aqp1 were altered in the gallbladder of hepatic Shp knockout mice, indicating a potential change in their bile composition. The enterokine, Fgf15 is known to stimulate gallbladder filling through its interaction with its receptor, Fgfr4. Recently, the Fgf15-Fgfr4 axis was shown to prevent degradation of the SHP protein such that impeding this axis led to higher Cyp7a1 transcript, increased bile acid levels, and altered gallbladder filling. Taken together, these findings suggest that hepatic Shp acts as a central node that regulates bile acid transport, homeostasis, and composition within the gall bladder and thus can prevent gallstone development. Elucidating Shp-mediated liver-to-gallbladder crosstalk is important for understanding the contribution of liver dysfunction towards gallbladder diseases and gallstones.
Presentation: Saturday, June 17, 2023
FRI497 Thyroid Storm And Recurrent Myopericarditis
Authors: Gomez D' Aza Danelly J , Zahedi Tooraj , Zhang Fan 
Published: October 2023

Abstract
Disclosure: D.J. Gomez D' Aza: None. T. Zahedi: None. F. Zhang: None.
Introduction: Thyroid storm is an acute life-threatening complication of hyperthyroidism with high mortality rate. Plasmapheresis is often used in patients who are unresponsiveness to conventional treatment for thyroid storm. About 5% of thyroid storm patients present with heart failure while other cardiovascular dysfunctions include tachycardia, arrhythmias, hypertension, and pericardial disease. Myopericarditis is characterized by pericarditis associated with myocardial inflammation evident by elevated cardiac biomarkers. Thyrotoxicosis associated with pericarditis and myocarditis is rare. Here, we report a case of recurrent myopericarditis and thyroid storm in Grave’s disease treated with plasmapheresis. Case Report: A 47 years male with a history of Schizophrenia and Hyperthyroidism due to Grave’s disease was presented with poor oral intake, generalized weakness, and tachycardia with HR 180 bpm. Labs showed elevated troponin 0.505 ng/mL, Free T4 &gt; 6.99 mg/dL, T3 &gt; 651 ng/dL, and low TSH &lt; 0.015 mg/dL. Burch-Warsofsky scale was 50 points which suggested thyroid storm. EKG revealed atrial fibrillation. Patient was managed with propranolol, propylthiouracil (PTU), iodine solution, hydrocortisone, and Diltiazem. However, his mental status deteriorated requiring intubation and patient developed cardiac shock with BP 77/53 mmHg. Troponin I trended up to 23.60 ng/mL and EKG showed ST-segment elevation in the anterior and inferior leads. Echocardiogram revealed severely reduced ejection fraction of 15 % and diffuse hypokinesis. Cardiac catheterization was unremarkable. PTU was discontinued due to liver dysfunction with AST and ALT levels above 1500 U/L and 2500 U/L. A session of plasmapheresis was initiated and the post labs showed an improvement of thyroid and liver function to Free T4 2.46 mg/dL, TSH 0.022 mg/dL, and ALT/AST 641/682 U/L. Patient was hemodynamically stable with normal sinus rhythm. Cardiac magnetic resonance images revealed left ventricular dilation with high T2 signal, late gadolinium enhancement in the pericardial region of basal inferolateral, inferior, and mid segments, consistent with pericarditis. Previous record showed patient had a similar admission 4 months ago with a diagnosis of myopericarditis. Discussion: The association of recurrent myopericarditis and hyperthyroidism may be related with the autoimmune dysfunction and underlying viral infection that precipitated inflammation of the thyroid gland, triggered thyroid storm, and caused cardiac event. Rapid correction of the hyperthyroid state is warranted in thyroid storm in addition to aggressive treatments for heart failure, arrhythmias, and myopericarditis. Our patient’s cardiac function improved rapidly after achieving euthyroidism by plasmapheresis. The treatment of hyperthyroidism is critical in preventing the recurrence of myopericarditis.
Presentation: Friday, June 16, 2023