Correction to Journal of the Endocrine Society, Volume 6, Issue Supplement 1, 2022

Introduction

Included here is a list of the corrections, submitted by the authors, for the November-December 2022 Journal of Endocrine Society Supplement issue's publication of the abstracts from the ENDO 2022 Annual Meeting of the Endocrine Society. Most of the corrections concern the incorrect ordering of author names in the byline, missing author information, and abstracts that were included in the Meeting program but omitted from the Supplement. The Endocrine Society acknowledges the errors.

Corrections for each abstract

Abawi et al. “Age of Onset of Obesity and Childhood BMI Trajectories in Rare Genetic Obesity Disorders.” doi: 10.1210/jendso/bvac150.1272

• The correct order of authors is “O. Abawi, R.J. Wahab, L. Kleinendorst, A.E. Brandsma, E.F.C. van Rossum, B. van der Voorn, M.M. van Haelst, R. Gaillard, E.L.T. van den Akker.”

Alzenaidi and Alkhalidi. “Empagliflozin Induced Bladder Cancer in an Adult Patient With Latent Autoimmune Diabetes.” doi: 10.1210/jendso/bvac150.815

• The following affiliation should be included for Ahlam Alzenaidi: “Department of Internal Medicine, College of Medicine, Qassim University.”

• The following affiliation should be included for Hisham Alkhalidi: “Department of Histopathology, Medical Diagnostic Labs, Dr. Sulaiman Alhabib Medical Group.”

Campos Haedo et al. “Thyroid Hormones membrane receptor, Integrin αvβ3, as a potential prognostic marker of chemotherapy response in breast cancer.” doi: 10.1210/jendso/bvac150.1813

• The author name “M. Celeste Díaz Albuja” should be “M. Celeste Díaz Flaqué.”

Chugh and Harkens. “Plasmapheresis and Extracorporeal Membrane Oxygenation (ECMO) for Treatment of Thyroid Storm with Multiorgan Failure.” doi: 10.1210/jendso/bvac150.1775

• The correct order of authors is “Wiley Harkens, DO, Radhika Chugh, MD.”

Dashevsky et al. “An EMR And Educational Intervention To Avoid Excessive Fingerstick Blood Glucose Testing In Low-risk Hospitalized Patients With Type Ii Diabetes.” doi: 10.1210/jendso/bvac150.711

• The correct full abstract is:

Fingerstick blood glucose (FSBG) testing in hospital settings allows for real-time insulin adjustment, protecting patients from significant hypo/hyperglycemia. Admitted patients with Type II diabetes mellitus (T2DM) are often placed on sliding scale insulin with four FSBG tests per day, regardless of home insulin use or baseline control of diabetes. FSBG monitoring of patients at low risk for inadequate glycemic control may add to nursing workload, cause patient discomfort, and waste resources without changing clinical care. We piloted a multifaceted, student-led intervention to avoid FSBG testing in low-risk T2DM patients on all teaching and non-teaching Hospital Medicine services at an urban, academic, tertiary care center. Inclusion criteria for “low-risk” hospitalized patients with T2DM included: 1) not on home insulin, 2) 72-hour blood glucose less than 250 mg/dl, 3) total daily dose of insulin less than five units, 4) not on high-risk medications such as steroids. Our opportunity assessment to avoid FSBG tests involved a pre-intervention point prevalence study (PPS), a provider awareness study (PAS), and a nurse perception survey (NPS). Our PPS and PAS found that FSBG testing is common, but provider awareness of FSBG monitoring is low. Additionally, our NPS found that administering the FSBG test requires ∼5 minutes of nursing time, with 61% of nurses estimating that fewer than 50% of patients undergoing FSBG testing are actually given insulin in response to test results. Each FSBG test, including all supplies, costs approximately $9. Our exploratory studies suggested opportunities to avoid excess FSBG monitoring. The first intervention included adding a new, twice daily before meals (BID AC) order option for point-of-care FSBG monitoring in the electronic medical record (EMR). The second intervention included educating hospitalists, residents, physician assistants, nurse practitioners, and nurses using monthly educational lectures, didactics, and individual education about which patients with T2DM qualify as “low-risk” and how to execute the BID FSBG order in the EMR. Our outcome measures included the number of FSBG order changes per month in the EMR, the proportion of patients with T2DM receiving BID FSBG or none, and the number of fingersticks avoided. Our post-intervention analysis found a significant 208% increase in order changes per month from 4.60 to 14.71 (p<0.01). The proportion of qualifying patients whose FSBG order changed increased 115% during the post-intervention period from .0290 to .0493, avoiding 1,541 fingersticks and saving $14,000. These results suggest that educational interventions and changes in EMR ordering options were sufficient to avoid unnecessary FSBG testing, but additional interventions may be required to sustain the impact.

• Wang et al. have been incorrectly listed as authors. The correct authors are: “Jared Dashevsky, ME, Samir Kamat, BA, Alyssa Gontzes, BS, Madeline Floodstrand, BS, Caroline Liu, MHS, Grenye O’Malley, MD, Anne Linker, MD.”

Faruqi et al. “Over-Expression of IGF-2 Causing Hypoglycemia from a Gastrointestinal Stromal Tumor.”

• This abstract was included in the ENDO 2022 program but was not published in the JES supplement. The full abstract is:

Introduction: Tumors arising from mesenchymal or epithelial cells can produce IGF-2 as part of tumor proliferation pathways. This overproduction of IGF-2 can cause non-islet-cell-mediated hypoglycemia. We present a case of new-onset, refractory hypoglycemia in a patient diagnosed with a gastrointestinal stromal tumor.

Case: A 57-year-old female with stage IV gastrointestinal stromal tumor (GIST) with peritoneal carcinomatosis and tumor progression presented to the hospital with worsening abdominal distention, pain, nausea, and new-onset hypoglycemia meeting the criteria for Whipple's triad. She had recurrent episodes of hypoglycemia as low as 29 mg/dL despite continuous dextrose infusion and frequent oral intake. The patient denied a personal history of diabetes or use of hypoglycemic agents. Infectious work up was unremarkable. Critical labs showed: negative sulfonylurea screen; point-of-care glucose 29 mg/dL; plasma glucose 61 mg/dL; beta hydroxybutyrate less than 0.1 mmol/L; insulin level less than 0.4 uIU/mL; c-peptide level 0.07 (reference range 1.10–1.40 ng/mL); proinsulin level 2.1 pmol/L (reference range 3.6–22 pmol/L); glucagon 30 pg/mL (reference range less than or equal to 80 pg/mL); cortisol level 10 mcg/dL; IGF-1 11ng/mL (reference range 37–208 ng/mL); and IGF-2 of 702 ng/mL (reference range 333–967 ng/mL), with IGF-2 to IGF-1 ratio of 63:1. Administration of glucagon showed adequate response, indicating good glycogen reserve. The patient's fluids were changed to D10W and she was started on dexamethasone. In addition, cornstarch with applesauce before bedtime was incorporated to provide delayed absorption of carbohydrates with the intent to prevent nighttime hypoglycemia. We were able to stabilize her glucose and wean off the dextrose drip for a brief period of time. However, her disease had rapid progression and with declining clinical status she was transitioned to palliative care.

Discussion: IGF2-mediated hypoglycemia is being recognized as a cause of profound fasting hypoglycemia in oncological patients. Certain tumors may overproduce IGF-2 for autocrine-paracrine stimulation of tumor cells. This excess production of mature IGF-2 and big IGF-2 (incompletely processed IGF-2) results in high levels of circulating IGF-2. While hypoglycemia itself does not predict the size or malignant potential of tumors, IGF-2 mediated hypoglycemia is clinically challenging to treat due to suppression of counterregulatory hormones. IGF-2 binds to insulin receptors, acting functionally like insulin. IGF-2 mediated hypoglycemia leads to active suppression of GH, glucagon, IGF-1 and insulin. Since IGF-2 acts on insulin receptors, there is also a blunted rise in ketones, suppression of free fatty acid release, glycogenolysis, gluconeogenesis, and ketogenesis in the liver. Ultimate treatment is removal of the causative tumor; unfortunately, our patient's disease continued to progress despite several lines of chemotherapy and tumor resection. We present this case to emphasize that IGF-2 should be routinely considered in cancer patients who present with new onset hypoglycemia.

Friedman et al. “Fructose Consumption and NAFLD in US Adult Population of NHANES 17-18.” doi: 10.1210/jendso/bvac150.035

• The correct order of authors is “Theodore C. Friedman, MD, PhD¹, Magda Shaheen, MD, PhD, MPH, MS¹, Katrina Schrode, PhD¹, Dulcie Kermah, MS, EdD¹, Zarrinpar,Ali, PhD², Sonia Michael Najjar, MS, PhD³.”

• The following affiliations should be added: “¹Charles R. Drew University, Los Angeles, CA, USA, ²University of Florida College of Medicine, Gainesville, Florida, USA, ³Ohio University-Heritage College of Medicine, Athens, OH, USA.”

Friedman et al. “Hepatic Fibrosis and Race/Ethnicity in Adult US Population: Data from NHANES 2017-18.” doi: 10.1210/jendso/bvac150.048

• The correct order of authors is “Theodore C. Friedman, MD, PhD¹, Magda Shaheen, MD, PhD, MPH, MS¹, Katrina Schrode, PhD¹, Dulcie Kermah, MS, EdD¹, Zarrinpar,Ali, PhD², Sonia Michael Najjar, MS, PhD³.”

• The following affiliations should be added: “¹Charles R. Drew University, Los Angeles, CA, USA, ²University of Florida College of Medicine, Gainesville, Florida, USA, ³Ohio University-Heritage College of Medicine, Athens, OH, USA.”

Friedman et al. “Nonalcoholic Steatohepatitis (NASH), Race/Ethnicity, and Gender in Adult US Population of NHANES 17-18.” doi: 10.1210/jendso/bvac150.049

• The correct order of authors is “Theodore C. Friedman, MD, PhD¹, Magda Shaheen, MD, PhD, MPH, MS¹, Katrina Schrode, PhD¹, Dulcie Kermah, MS, EdD¹, Zarrinpar,Ali, PhD², Sonia Michael Najjar, MS, PhD³.”

• The following affiliations should be added: “¹Charles R. Drew University, Los Angeles, CA, USA, ²University of Florida College of Medicine, Gainesville, Florida, USA, ³Ohio University-Heritage College of Medicine, Athens, OH, USA.”

Grabowski et al. “Design of an Ultra-Stable Insulin/Glucagon Fusion Protein.” doi: 10.1210/jendso/bvac150.870

• “Nicolas Varas” is the first author.

Hall et al. “Cloning, sequencing, and characterization of aromatase interacting partner in breast (AIPB). A new protein regulating estradiol synthesis.” doi: 10.1210/jendso/bvac150.1824

• The correct order of authors is “Neal M. Hall, Randy M. Whittal, William E. Burak, and Himangshu S. Bose.”

Hallman et al. “The Effects of Perfluorooctanesulfonic Acid (PFOS) on the Expression of Estrogen Receptor Alpha (ERα) and Tumor Suppressor Gene BRCA1 in Breast Cancer Cells.” doi: 10.1210/jendso/bvac150.915

• The author listed as “Samantha Pfiffner, MD” should be changed to “Samantha Pfiffner, BS.”

Iwamoto et al. “Research Priorities of Gender Diverse Adults: What is Most Important to the Community?” doi: 10.1210/jendso/bvac150.1455

• The correct order of authors is “Sean J. Iwamoto, MD, Lindsey Warner, MD, Micol S. Rothman, MD, Danielle Loeb, MD, MPH, Lisa M. Schilling, MD, MSPH, Danielle M. Kline, MS, Mary P. Mancuso, MA, Natalie Nokoff, MD, MSCS.”

• The following information should be added for the authors:

Primary Author: Sean J. Iwamoto, MD (he/him)

Assistant Professor of Medicine

Division of Endocrinology, Metabolism & Diabetes

University of Colorado School of Medicine

Rocky Mountain Regional VA Medical Center

UCHealth Integrated Transgender Program

Co-Author: Lindsey Warner, MD (he/him)

Department of Pediatrics

University of Colorado Anschutz Medical Campus

Micol S. Rothman, MD (she/her)

Division of Endocrinology, Metabolism & Diabetes

University of Colorado School of Medicine

UCHealth Integrated Transgender Program

Danielle Loeb, MD, MPH (they/them)

Division of General Internal Medicine

University of Colorado School of Medicine

UCHealth Integrated Transgender Program

Lisa M. Schilling, MD, MSPH (she/her)

Division of General Internal Medicine

University of Colorado School of Medicine

Danielle M. Kline, MS (she/her)

Division of General Internal Medicine

University of Colorado School of Medicine

Mary P. Mancuso, MA (she/her)

Division of General Internal Medicine

University of Colorado School of Medicine

Natalie Nokoff, MD, MSCS (she/her)

Department of Pediatrics

University of Colorado School of Medicine

Children's Hospital Colorado TRUE Center for Gender Diversity

Johnson et al. “Fezolinetant for Treatment of Moderate-to-severe Vasomotor Symptoms Associated with Menopause: Results from a 52-week Study (Skylight 2).” doi: 10.1210/jendso/bvac150.1403

• The correct order of authors is “Kimball Johnson, Rossella E. Nappi, Genevieve Neal-Perry, Marla Shapiro, Petra Stute, Rebecca C Thurston, Wendy Wolfman, Marci English, Catherine Franklin, Misun Lee, Faith Ottery, Nanette Santoro.”

Kim et al. “Polymorphisms of ITM2A rs1751094 on X Chromosome Is Associated with intractable Graves’ disease in Korean Children.” doi: 10.1210/jendso/bvac150.1605

• The order of authors should have the author “Sung Eun Kim” listed first.

Lalani and Lalani. “Management of Adrenal Insufficiency with Continuous Subcutaneous Hydrocortisone Infusion: Long-Term Experience in 27 Patients.” doi: 10.1210/jendso/bvac150.268

• The correct order of authors is “Benjamin Lalani and Atul Lalani.”

Lazaretti-Castro et al. “Weekly Supplementation with Vitamin D3 reduces 1,25(OH)2 vitamin D3 Measured by Mass Spectrometry, and increases Fibroblast-like growth factor 23 (FGF23) in Individuals with and without Primary Hyperparathyroidism (PHPT).” doi: 10.1210/jendso/bvac150.412

• Sidana et al. have been incorrectly listed as the authors. The correct authors are Marise Lazaretti-Castro, MD, PhD, Sthefanie Pallone, MD, Livia Maria Santos, PhD, Monique Ohe, PhD, Isabella Nacaguna, academic, Ilda Kunii, PhD, Renata Silva, Master, Karina Cardozo, PhD, Jose Gilberto Vieira, PhD.

Mora et al. “Late Versus Early Administration of Radioiodine Therapy for Patients with Differentiated Thyroid Cancer: A Systematic Review and Meta-analysis.” doi: 10.1210/jendso/bvac150.1665

• The primary author should be “Samantha Mora” instead of “Eddy P. Lincango Naranjo.”

• The following information should be included for the authors, and they should be listed in the following order in the byline:

  • Samantha Mora, MS. Maestría en Biomedicina, Facultad de Ciencias de la Salud, Universidad Internacional SEK, Quito, Ecuador.

  • Eddy P. Lincango Naranjo, MD. Knowledge and Evaluation Research Unit Mayo Clinic.

  • Ivelin Leal-Medina, Medical Student. Facultad de Medicina, Universidad Central del Ecuador, Quito, Ecuador.

  • Sarahí Dueñas-Andrade, Medical Student. Facultad de Medicina, Universidad Central del Ecuador, Quito, Ecuador.

  • Tannya Ledesma-León, Medical Student. Facultad de Medicina, Universidad Central del Ecuador, Quito, Ecuador.

  • Camila Valle, Medical Student. Facultad de Medicina, Universidad Central del Ecuador, Quito, Ecuador.

  • Carla M. Dominguez, Medical Student. Facultad de Medicina, Universidad Central del Ecuador, Quito, Ecuador.

  • Andres Ayala, MD. Médicos Asociados, Cayambe, Ecuador.

  • Oscar J. Ponce, MD. Knowledge and Evaluation Research Unit, Mayo Clinic, Rochester, Minnesota, USA.

  • Juan P. Brito, MD. Knowledge and Evaluation Research Unit, Mayo Clinic, Rochester, Minnesota, USA.

  • William Acosta, MD. Pontificia Universidad Católica del Ecuador, Quito, Ecuador.

  • Dámaris P. Intriago-Baldeón, MS. Grupo de Investigación en Biomedicina Experimental y Aplicada, Facultad de Ciencias de la Salud, Universidad Internacional SEK, Quito, Ecuador.

• The following information should be included after the byline: “Corresponding authors. Dámaris P. Intriago-Baldeón - [email protected]. William Acosta, MD - [email protected].”

Nathni et al. “Evaluation Of Factors Associated With The Development Of Diabetic Retinopathy Based On A Case-control Study.” doi: 10.1210/jendso/bvac150.568

• The sentence “Adherence to diabetes medications was measured using four items in vernacular language adapted from the MMAS-4 (Morisky Medication Adherence Scale).” should be removed.

• The word “dyslipidemia” should be “dyslipidaemia.”

• “Conclusion: Diabetics under insulin therapy and with hypertension have a seriously increased risk of developing DR. Smoking, alcohol abuse, sedentary lifestyle, failure to take diabetes medications, and failure to follow dietary advice…” should be “Conclusion: Diabetics that fail to take medications regularly and with hypertension have a seriously increased risk of developing DR. Smoking, alcohol, sedentary lifestyle, insulin, and failure to follow dietary advice…”

• The following information should be at the end of the abstract: “Author Disclosure Information: P.S. Nathani: None. P.R. Kharat: None. S. Wadde: None. S. Kamdar: None.”

Olajide et al. “An Unusual Case of Refractory Hypocalcemia associated with Osteoblastic Metastases and Hypoparathyroidism.”

• This abstract was included in the ENDO 2022 program but was not published in the JES supplement. The full abstract is as follows:

Case Presentation: 71-year-old female with past medical history of Right Breast cancer Stage IIB (T2 N1M0) grade 1 invasive lobular carcinoma s/p lumpectomy and radiation presented to the hospital with complaints of nausea, vomiting and abdominal cramps that started 3 days prior. This was associated with constipation, numbness and tingling sensation on her hands and face. She also had a history of thyroid cancer s/p thyroidectomy, surgical hypoparathyroidism. She was on calcitriol 3mcg daily, calcium 600mg TID daily. Her calcium had been stable for 24 years on the current regimen. A recent bone scan done showed widespread osteoblastic metastatic disease. Physical examination was significant for Chvostek sign. Vital signs were normal. Lab studies were remarkable for calcium 5.6mg/dl, albumin 2.5mg/dl, PTH 14 pg/ml, alkaline phosphatase was 445U/L. 25 OH vitamin D, magnesium, phosphorus and creatinine were all normal. She was admitted and started on IV calcium and calcitriol 1mcg BID. Her calcium remained persistently low despite high doses of both IV and oral calcium and calcitriol. Oncology was consulted and she received one dose of fulvestrant. Her oral calcium was titrated to a total dose of 8g of elemental calcium/day, calcitriol 2 mcg Q12hr, vitamin D3 2000 IU daily and was discharged with a serum calcium of 8.6 mg/dl after she was hospitalized for 22 days. She was readmitted a month later with severe hypocalcemia and was aggressively treated with oral and IV calcium along with high doses of calcitriol. She was discharged 7 days later.

Discussion: Hypocalcaemia is not commonly seen in-patients with metastatic cancer. Prostate and Breast cancer are the malignancies most associated with Hypocalcemia due to Osteoblastic Lesions. One of the proposed mechanisms is an increase in calcium uptake by the osteoblastic bone metastases. This is expected to elicit a compensatory increase in PTH to restore normal calcium levels. Our patient had a history of surgical hypoparathyroidism and so there was an inadequate PTH response and a persistently low level despite severe hypocalcemia. She was notably stable on her home regimen of calcium and calcitriol for many years before she developed osteoblastic metastases. Treatment includes high doses of oral and IV calcium and calcitriol. In patients with refractory hypocalcemia to standard therapy, radionucleotide therapy can be considered.

Conclusion: Severe hypocalcemia can be associated with metastatic Breast cancer and worsened by the presence of Hypoparathyroidism. An increased awareness of this condition is key to ensure proper management and prevent life threatening complications.

Pesantez et al. “The Missing Link Between Obesity and Depression in Transgender Patients: A Retrospective Review at the Border of the United States.” doi: 10.1210/jendso/bvac150.1459

• “Maria Pesantez, MD” should be listed as the first author in the byline for this abstract.

Pfiffner et al. “The Effects of Tris(2-Chloroethyl) phosphate (TCEP) on the Expression of Estrogen Receptor Alpha and Tumor Suppressor Gene BRCA-1 in Breast Cancer Cell Lines.” doi: 10.1210/jendso/bvac150.904

• Author listed as “Samantha Pfiffner, MD” should be “Samantha Pfiffner, BS.”

Pollé et al. “Identification of Four New Glucotypes during the First Year after Type 1 Diabetes Onset Using Continuous Glucose Monitoring Metrics.” doi: 10.1210/jendso/bvac150.726

• The correct order of authors is: Olivier Pollé, Antoine Delfosse, Manon Martin, Thierry Mouraux, Inge Gies, Marieke den Brinker, Jacques Louis, Nicole Seret, Marie-Christine Lebrethon, Laurent Gatto, and Philippe Lysy.

Ramachandran et al. “Hyperinsulinism-Hyperammonaemia syndrome: a dive into the rare presentation and diagnostic challenges encountered in adults.” doi: 10.1210/jendso/bvac150.938

• The correct order of authors is “Akshaya Ramachandran, MD, Aishwarya Ramachandran, MD, Sarah Ghaith, MD.”

Rusalenko et al. “Significance Of Insulin Resistance And Development Of Type 2 Diabetes Mellitus.” doi: 10.1210/jendso/bvac150.563

• The lead author name “Maria Rusalinka” should be “Maria G. Rusalenko.”

• The byline should be “Maria G. Rusalenko¹, Irina G. Savasteeva¹, Tamara M. Sharshakova², Anastasia V. Sachkovskaya², Dmitriy V. Kovalevskiy², Mohamed Wisham¹.”

• In both affiliations, “Belarus” should be changed to “Republic of Belarus, 246000.”

• Section headings should be added.

  • “Aim” should be inserted before the sentence beginning “A study was conducted via Clinical and laboratory parameters…”

  • “Data Analysis & Methods” should be inserted before the sentence beginning “The results of the analysis demonstrated…”

  • “Conclusion” should be inserted before the sentence beginning “Thus, individuals over 37.74 years…”

• There should be paragraph breaks inserted before each section heading, opening sentences of each section, and the following sentences within the Data Analysis & Methods section:

  • “It was also noted that the risk of developing…”

  • “When assessing the risk of developing…”

  • “In addition, the risk of developing…”

Sood et al. “A Rare Case of Pembrolizumab Induced Diabetic Ketoacidosis.” doi: 10.1210/jendso/bvac150.614

• The correct order of authors is “Aayushi Sood, MD, Brihant Sharma, MD, Khyati Khattar, MD, Ajinkya Buradkar, MD, Jawahar Durrani, MD.”

Sweis et al. “Empagliflozin for Treatment of Neutropenia in a Patient with Glycogen Storage Disease Type 1b: An Emerging Use for SGLT2i.”

• This abstract was included in the ENDO 2022 program but was not published in the JES supplement. The full abstract is as follows:

Introduction: Glycogen storage disease type 1b (GSD 1b) is a rare inherited disorder of defective glycogen metabolism due to a mutation in the glucose-6-phosphate transporter gene, resulting in severe hypoglycemia, hepatomegaly, and growth retardation. Patients with GSD 1b develop impaired neutrophil function, in addition to neutropenia, which has been treated with granulocyte colony stimulating factor (G-CSF). We report a case of GSD 1b with neutropenia refractory to G-CSF treatment who was subsequently treated with empagliflozin.

Case Description: A 22-year-old woman with a history of GSD 1b, chronic neutropenia, and a history of frequent hospitalizations for infections, was admitted with complaints of nausea, vomiting, diarrhea, and difficulty tolerating oral intake and was found to have hypoglycemia, metabolic acidosis. She was being treated with G-CSF thrice a week and corn starch six times a day at home. In the last few years, she had multiple hospital admissions for lip abscess, tonsillar abscess, gastrointestinal infections, COVID 19.

On admission, she was noted to have a WBC count of 4.2 (RR: 3.9-12 K/UL), absolute neutrophils count (ANC) of 1.1 K/UL (RR: 1.3 -7.5 K/UL), serum blood glucose of 14mg/dl, lactate of 4.8 (RR: 0.5-2 mmol/L). Dextrose containing IV fluids, G-CSF, tube feeds and cornstarch were initiated. WBC count and ANC further dropped to 2.4 K/UL and 0.6 K/UL respectively, despite receiving treatment with G-CSF. 1,5 Anhydroglucitol (1,5-AG) was elevated at 36.5 ug/ml (RR: 6.8-29.3 ug/ml). Stool PCR test for C. Difficile was positive. This was patient's second C. Difficile infection in the last year. Patient was started on vancomycin therapy. Given persistent neutropenia refractory to G-CSF, she was started on empagliflozin 10mg daily. Four weeks later, ANC had improved to 4.2 K/UL, and patient denied any new infections since discharge.

Discussion: For many years, G-CSF has been the standard treatment for neutropenia in GSD 1b patients, despite its serious potential side effects of myelodysplastic syndrome and acute myeloid leukemia. Empagliflozin, an SGLT-2 inhibitor that stimulates glucosuria, was found to also stimulate the excretion of 1,5-AG, a glucose analog implicated in the dysfunction of neutrophils in GSD 1b. In comparison to G-CSF, empagliflozin not only improves neutrophil count, but is also associated with improved quality of neutrophils. In all cases which have been reported, all patients have had improvement in ANC, reduction in number of infections and no episodes of hypoglycemia. Also, ANC remained normal despite discontinuation of G-CSF in 50% of these patients.

References

1. Kishnani PS, Austin SL, Abdenur JE, et al. Diagnosis and management of glycogen storage disease type I: A practice guideline of the American College of Medical Genetics and Genomics. Genetics in Medicine. 2014;16(11):1-29. doi:10.1038/gim.2014.128

2. Wortmann SB, van Hove JLK, Derks TGJ, et al. Treating neutropenia and neutrophil dysfunction in glycogen storage disease type Ib with an SGLT2 inhibitor. Blood. 2020;136(9):1033-1043. doi:10.1182/blood.2019004465

3. Grünert SC, Elling R, Maag B, et al. Improved inflammatory bowel disease, wound healing and normal oxidative burst under treatment with empagliflozin in glycogen storage disease type Ib. Orphanet Journal of Rare Diseases. 2020;15(1):8-15. doi:10.1186/s13023-020-01503-8

4. Veiga-da-Cunha M, Chevalier N, Stephenne X, et al. Failure to eliminate a phosphorylated glucose analog leads to neutropenia in patients with G6PT and G6PC3 deficiency. Proceedings of the National Academy of Sciences. 2019;116(4):1241 LP - 1250. doi:10.1073/pnas.1816143116

5. Takao MMV, Sandy NS, Riccetto AGL, De Tommaso AMA Long term management of glycogen storage disease type 1b: a Brazilian tertiary center experience

Sweis et al. “Pheochromocytoma in Pregnancy: A Case Report.”

• This abstract was included in the ENDO 2022 program but was not published in the JES supplement. The full abstract is as follows:

Introduction: Pheochromocytoma in pregnancy is extremely rare, with an estimated incidence of <2 per 100,000 pregnancies. Despite its rarity, this tumor has the potential to be disastrous for both the mother and the fetus, with mortality rates as high as 50% when left untreated.

Case Description: A 29-year-old woman at 22 weeks of gestation, with no known ho HTN, was admitted for evaluation of a recently diagnosed supra-renal mass. She was initially diagnosed with a UTI at 11w of gestation. At that time renal ultrasound showed a 3.6cm left suprarenal mass. Later, at 16w of gestation she was admitted for HTN emergency and underwent cardiac catheterization which showed no CAD. During that hospitalization, she was found to have elevated urine metanephrine levels of 4102mcg/24h (RR: 36 - 229 ug/d), suspicious for pheochromocytoma. She had a history of preeclampsia in her last pregnancy but no history of chronic hypertension and was not on any antihypertensive medications. She denied any spells of palpitations, headaches, pallor, or diaphoresis. Family history was unremarkable.

In this admission, her blood pressure was normal at 124/76mm Hg and pulse was 88/min. CBC and metabolic panel was unremarkable. She was noted to have elevated serum metanephrines of 7.66 nmol/L (RR: 0.00 - 0.49 nmol/L), normetanephrines 1.52 nmol/L (RR: 0.00 - 0.89 nmol/L) and 24h urinary metanephrines of 8929 mcg/24h. MRI of the abdomen showed a left adrenal mass measuring 4.3×2.9×3.8 cm which was slightly hyperintense to the normal adrenal tissue on T2 and isointense on T1, without any signal dropout on out of phase imaging, concerning for pheochromocytoma. She was adequately blocked with Doxazosin 6mg bid and Metoprolol 100mg bid preoperatively and underwent laparoscopic left adrenalectomy at 24w of gestation. Alpha and beta blockers were discontinued postoperatively. Surgical pathology confirmed a pheochromocytoma of 4.3cm, with negative margins and absent capsular invasion. PASS Score (Pheochromocytoma of the Adrenal gland Scaled Score) was 2. Postoperative serum metanephrines and normetanephrine levels returned at <0.10 nmol/L and 0.51 nmol/L respectively.

Discussion: The diagnosis of pheochromocytoma in pregnancy continues to be challenging for physicians as it may mimic other pregnancy-related conditions. Early detection and treatment are imperative for a favorable outcome. Preoperative blockade is done with the same medications used in non-pregnant individuals. Orthostatic vital signs may not be positive in pregnant patients even with adequate blockade, as the gravid uterus pressing on IVC in a supine position leads to decreased venous return, fall in cardiac output and stroke volume. Within the first 24 weeks of gestation, a pheochromocytoma may be removed by laparoscopic adrenalectomy. If the tumor is discovered in the third trimester, surgical intervention is deferred until delivery, and the tumor may be removed after a cesarean section.

References

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  Ahlawat SK, Jain S, Kumari S, Varma S, Sharma BK. Pheochromocytoma associated with pregnancy: case report and review of the literature. Obstet Gynecol Surv. 1999;54(11):728-737. doi:10.1097/00006254-199911000-00025

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  Lenders JW. Pheochromocytoma and pregnancy: a deceptive connection. Eur J Endocrinol. 2012;166(2):143-150. doi:10.1530/EJE-11-0528

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  Oliva R, Angelos P, Kaplan E, Bakris G. Pheochromocytoma in pregnancy: a case series and review. Hypertension. 2010;55(3):600-606. doi:10.1161/HYPERTENSIONAHA.109.147579

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  Schenker JG, Granat M. Phaeochromocytoma and pregnancy–an updated appraisal. Aust N Z J Obstet Gynaecol. 1982;22(1):1-10. doi:10.1111/j.1479-828x.1982.tb01388.x

Wu et al. “Obesity and Weight Loss Apps in COVID-19 Era: Do They Help Both the Doctor and the Patient?” doi: 10.1210/jendso/bvac150.027

• Lawrence et al. have been wrongly listed as the authors. The correct authors are Gloria Wu, MD, Katherine Tien, BS, Weichen Zhao, BS.

Yuan et al. “The Association between Life's Simple 7 Score and Three Biomarkers of Cardiovascular Disease: Aldosterone, CRP, and IL-6.” doi: 10.1210/jendso/bvac150.517

• The correct order of authors is “Yuan, Yan Emily; Adler, Gail K.; Haas, Andrea; Seely, Ellen W.; Williams, Gordon.”

Yuksel et al. “Hypertriglyceridemia-Induced Acute Pancreatitis in Second Trimester of Pregnancy.” doi: 10.1210/jendso/bvac150.499

• “Simge Yuksel” should be listed first in the byline.