Νον-Islet Cell Tumor Hypoglycemia Associated With Uterine Leiomyomata

ObjectiveWe report a case of non-islet cell tumor hypoglycemia (NICTH) in a patient with large leiomyomata.MethodsWe present the clinical, laboratory, and pathologic findings of a diabetic patient who presented with recurrent hypoglycemia later linked to uterine leiomyomata.ResultsAn 80-year-old woman with diabetes was admitted after falling at home. She reported dizziness and had recorded low capillary blood glucose despite discontinuing her diabetic medication prior to admission. Her physical examination was remarkable for nonorthostatic vital signs, normal cardiovascular and lung examination, and a pelvi-abdominal mass the size of a gravid uterus at 28 weeks of gestation. After receiving a 50% dextrose infusion, she became alert with no focal neurological deficit. Capillary blood glucose rose from 31 mg/ dL to 110 mg/dL. A pelvic sonogram confirmed fibromyomata. She was initially treated with steroids after ahormonal profile suggested NICTH (normal fasting insulin, C-peptide, cosyntropin and glucagon stimulation tests, and negative insulin antibodies). Insulinlike growth factor (IGF) levels were IGF-1, 69 ng/mL and IGF-2, 782 ng/mL, and the IGF-2/IGF-1 ratio was 10.8. The patient underwent a total abdominal hysterectomy. Pathology reported a 3-kg uterus with multiple, large cellular fibromyomas. After steroids were discontinued, she became hyperglycemic requiring insulin and oral diabetic agents. Repeat IGF-2 and IGF-1 measurements were 261 ng/mL and 36 ng/mL, respectively. She was discharged 2 weeks after surgery.ConclusionNICTH is a rare complication associated with large neoplasms. Leiomyomata should be included in the differential diagnoses of NICTH. Surgery is curative in such cases. (Endocr Pract. 2011;17:e109-e112)     

Use of A Continuous Glucose Monitor in the Management of Inoperable Meta Static Insulinoma: A Case Report

ObjectiveTo describe the successful use of a continuous glucose monitor in the management of a patient with inoperable metastatic insulinoma.MethodsWe present a case of inoperable recurrent metastatic insulinoma in which medical therapy failed to relieve symptoms of dangerous hypoglycemia. We describe how the use of a continuous glucose monitor has assisted in avoiding hypoglycemia and improving her quality of life.ResultsA 70-year-old woman with a history of recurrent surgically treated insulinoma presented with recurrent hypoglycemia secondary to multiple metastases in the liver. Diazoxide therapy decreased the frequency of symptoms, but she continued to have hypoglycemic episodes resulting in frequent visits to the emergency department. Since starting to use a continuous glucose monitor, she has been able to avoid hypoglycemia with associated neuroglycopenic symptoms. While the accuracy of the device was poor when compared with conventional fingerstick monitors, the sensor tended to read higher than the meter in the hypoglycemic range. Although this led to more frequent false-positive hypoglycemic alarms, true episodes of severe hypoglycemia were rare.ConclusionsMalignant insulinomas are rare tumors. Many affected patients have disease that is unresectable, and medical therapy is limited in its ability to prevent hypoglycemic episodes. We have demonstrated that a continuous glucose monitor can be a useful adjunct to therapy to reduce hypoglycemic episodes by alerting the patient to low glucose concentrations before the development of neuroglycopenic symptoms. (Endocr Pract. 2008;14:880-883)     

Danger of Hypoglycemia Due to Acute Tramadol Poisoning

ObjectiveTo report a case of prolonged hypoglycemia after acute tramadol poisoning.MethodsWe describe a patient’s clinical presentation and outcome with prolonged hypoglycemia attributable to acute tramadol poisoning. In addition, the possible mechanism for the hypoglycemia is discussed, and a brief review of the pertinent literature is presented.ResultsA 54-year-old woman had previously under- gone a partial hepatectomy because of involvement of her liver by a gastrointestinal stromal tumor. After ingestion of 3,000 mg of tramadol with suicidal intent, she developed prolonged hypoglycemia that necessitated treatment with continuous intravenous glucose infusion for 24 hours. Reports in the literature have described central nervous system depression, nausea, vomiting, tachycardia, seizures, and even death from tramadol overdoses.ConclusionThis report alerts clinicians to the potential danger of severe hypoglycemia in tramadol poisoning. (Endocr Pract. 2012;18:e151-e152)     

Successful Treatment of Persistent Hyperinsulinemic Hypoglycemia with Nifedipine in an Adult Patient

ObjectiveTo describe the successful treatment of severe noninsulinoma hyperinsulinemic hypoglycemia with use of a calcium channel blocking agent in an adult patient who had previously undergone a gastric bypass surgical procedure.MethodsA 65-year-old woman who had undergone a gastric bypass surgical procedure 26 years earlier was hospitalized because of severe postprandial hypoglycemia. During and after hospitalization, the patient underwent assessment with conventional measurements of glucose, insulin, proinsulin, and C-peptide; toxicologic studies; magnetic resonance imaging studies of the pancreas; and determination of hepatic vein insulin concentrations after selective splanchnic artery calcium infusion.ResultsMetabolic variables were consistent with the diagnosis of hyperinsulinemic hypoglycemia. Magnetic resonance imaging revealed the presence of a side branch intraductal papillary mucinous tumor that had been stable for more than 1 year. The results of the calcium-stimulated insulin release study were consistent with nonlocalized hypersecretion of insulin. A trial of frequent small feedings failed to prevent hypoglycemia. On the basis of reports of successful treatment of childhood nesidioblastosis, the patient was then prescribed nifedipine, 30 mg daily. She has subsequently remained free of symptomatic hypoglycemia for 20 months.ConclusionA calcium channel blocking agent may be efficacious and a potential alternative to partial pancreatectomy in cases of noninsulinoma hyperinsulinemic hypoglycemia in adults. (Endocr Pract. 2010;16:107-111)     

Transformation of a Pituitary Macroadenoma into to a Corticotropin-Secreting Carcinoma Over 16 Years

ObjectiveTo describe a case of a pituitary macroadenoma that differentiated into a corticotropin (ACTH)-secreting carcinoma with metastasis to the thigh.MethodsWe present a case report with a 16-year follow-up that includes anatomic and endocrine documentation of the history of an ACTH-secreting carcinoma.ResultsA 32-year-old woman presented for evaluation in 1989 because of visual feld defects. Magnetic resonance imaging revealed a locally invasive 3-cm macroadenoma. She had no clinical signs of cortisol excess. The patient underwent surgical debulking followed by a course of radiation directed to the pituitary. Results from retrospective immunohistochemical staining with antibodies against ACTH, prolactin, and MIB-1 were negative. Postoperatively, she could not be weaned from exogenous steroids without developing symptoms of adrenal insuffciency. In 1995, she developed left facial palsy and diplopia caused by tumor growth. In 1997, the patient developed progressive symptoms of cortisol excess, which continued after exogenous steroid replacement was discontinued. The patient’s clinical status continued to deteriorate because of local mass effect from tumor growth and uncontrolled hypercortisolism. She underwent bilateral adrenalectomy in 2003. The patient remained debilitated in a long-term care facility for 2 years when she was found to have a mass on her left hip. Biopsy results of the obturator muscle revealed metastatic tumor of neuroendocrine origin with strong reactivity to ACTH antibodies and MIB-1 labeling in 8% of tumor cell nuclei.ConclusionA pituitary tumor can transform into an ACTH-secreting carcinoma in an indolent manner. Patients with invasive pituitary adenomas require long-term surveillance to monitor for differentiation into pituitary carcinoma. (Endocr Pract. 2007;13:463-471)     

Corticotropin-Independent Cushing Syndrome in a Child With an Ovarian Tumor Misdiagnosed as Nonclassic Congenital Adrenal Hyperplasia

ObjectiveTo describe a patient with corticotropinindependent Cushing syndrome previously diagnosed and treated as congenital adrenal hyperplasia (CAH).MethodsWe describe the initial manifestations, clinical investigations, and postoperative follow-up of the patient and review similar cases in the literature.ResultsA 5 and 9/12-year-old girl who was initially diagnosed and treated as having CAH and was noncompliant with glucocorticoid therapy presented with weight gain, hypertension, and a mass in the lower abdomen. On physical examination, she was a cushingoid-appearing girl with proximal muscle weakness and notable facial acne. Laboratory findings included elevated serum testosterone, 17-hydroxyprogesterone, dehydroepiandrosterone sulfate, androstenedione, estradiol, and cortisol, as well as elevated urinary cortisol and cortisone. Serum corticotropin was undetectable. She had normal serum electrolytes and plasma renin activity. Computed tomography scan of the abdomen and pelvis showed a cystic mass with a focal enhancing solid component arising from the right ovary, which was subsequently determined to be a steroid cell tumor not otherwise specified.ConclusionAlthough ovarian steroid cell tumors typically secrete gonadal steroids, the rare steroid cell tumors not otherwise specified can secrete both glucocorticoids and gonadal steroids and are an unusual cause of Cushing syndrome. (Endocr Pract. 2008;14:875-879)     

Pheochromocytoma With Histologic Transformation to Composite Type,Complicated By Watery Diarrhea,Hypokalemia, and Achlorhydria Syndrome

ObjectiveTo describe the rare occurrence of histologic transformation of a pheochromocytoma to a composite type of tumor during a long-term follow-up, which was complicated by watery diarrhea, hypokalemia, and achlorhydria syndrome.MethodsWe report the case of a 12-year-old girl who presented with headache, hypertension, and elevated catecholamine levels in the blood and urine. A tumor was found in the right adrenal gland and resected. When she was 15 years of age, multiple metastatic nodules were found in the lung and liver. Intensive chemotherapy was ineffective, and she underwent follow-up with conservative therapy. At 25 years of age, she complained of diarrhea. Laboratory studies revealed hypokalemia and an increase in the level of serum vasoactive intestinal polypeptide (VIP). A year later, she died of extensive metastatic disease. The primary and recurrent tumors at autopsy were histologically examined.ResultsThe primary tumor was pure pheochromocytoma, and the tumors at autopsy were a composite type of pheochromocytoma and ganglioneuroma. Only a few VIP-positive cells were found in the primary tumor, whereas both pheochromocytoma and ganglioneuroma cells of composite tumors were frequently positive for VIP.ConclusionOur case showed histologic transformation from pheochromocytoma to a composite type of tumor during a 14-year clinical course, which was associated with additional hormone production and a change in symptoms. Careful attention should be paid to the alteration of endocrine symptoms and hormone levels during prolonged follow-up of pheochromocytoma in young patients. (Endocr Pract. 2012;18:e91-e96)     

Unexpected Clinical Course During Treatment of a TSH-Secreting Pituitary Adenoma

ObjectiveTo report the rare occurrence of a patient with thyrotropinoma that transitioned into a secretory thyro-somatotroph adenoma during medical treatment with somatostatin analogue.MethodsWe report the case of a patient with a thyrotroph pituitary adenoma who developed de novo evidence of growth hormone cosecretion following one year of successful medical treatment.ResultsA 78-year-old woman was diagnosed with a thyroid stimulating hormone (TSH) secreting pituitary macroadenoma (TSHoma) based on classical clinical and biochemical features. There was no clinical or biochemical evidence of growth hormone (GH) cosecretion. She declined surgical resection and was treated with primary medical therapy, octreotide long acting repeatable (LAR), to which she had an antitumor and antisecretory response; however, following 12 months of successful medical treatment she developed de novo hypersecretion of growth hormone despite involution of the tumor mass. TSH-secreting pituitary adenomas may rarely become plurihormonal during apparently successful medical treatment. This may represent an unusual form of secondary resistance to somatostatin analogue or the rarer phenomenon of tumor transformation into a secretory thyro-somatotroph adenoma.ConclusionThe unexpected clinical course of this case highlights the need for careful long-term surveillance in patients with TSH secreting pituitary adenomas. (Endocr. Pract. 2013;19:e88-e91)     

Everolimu Resolving Hypoglycemia,Producing Hyperglycemia,and Necessitating Insulin Use in A Patient With Di Etes and Nonresectable Malignant Insulinoma

ObjectiveTo present a case of management of refractory hypoglycemia due to malignant insulinoma with use of everolimusresulting in recurrent insulin-requiring diabetes.MethodsThis report describes a case of a nonresectable malignant insulinoma in a 78-year-old patient with long-standing type 2 diabetes mellitus. Endogenous hyperinsulinism was confirmed by a fasting test, which revealed a glucose level of 35 mg/dL and an insulin value of 23.7 μIU/mL. Endoscopic ultrasonography, magnetic resonance imaging, and computed tomography identified a pancreatic mass, infiltration of the superior mesenteric vein, and metastatic lesions in the liver.ResultsAfter chemoembolization of the metastatic lesions, hypoglycemia recurred, despite combined treatment with somatostatin analogues, dexamethasone, and diazoxide. Everolimus, an orally administered mammalian target of rapamycin, was used at a daily dose of 5 mg. After 6 months, the hypoglycemia was controlled, and the patient presented with a C-peptide level of 0.2 ng/mL and secondary hyperglycemia that necessitated insulin treatment.ConclusionThe orally administered drug everolimus controlled hypoglycemia due to a malignant insulinoma in a patient with prior insulinrequiring diabetes. Secondary hyperglycemia was an acceptable drug effect (to the patient and managing physicians), in light of the complex and often poorly tolerated treatments available for this rare condition. (Endocr Pract. 2011;17:e17-e20)     

Medullary Thyroid Carcinoma Manifesting as an Ovarian Mass: Case Report and Review of Literature

ObjectiveTo report the uncommon case of a woman with abdominal pain and a complex adnexal mass, who was subsequently found to have medullary thyroid carcinoma (MTC) metastatic to the ovary.MethodsWe present the clinical history, physical findings, laboratory and imaging studies, and pathologic findings in a woman with metastatic MTC and locally aggressive disease. The genetic associations, variable clinical course, and histopathologic findings in MTC are reviewed.ResultsA 38-year-old woman with abdominal and pelvic pain underwent a computed tomographic scan of the abdomen, which showed a complex left adnexal mass. After laparoscopic left oophorectomy, histopathologic analysis of the resected ovary suggested the presence of a metastatic neuroendocrine tumor. The patient recovered but was referred to the surgery clinic 3 months later because of hoarseness, a left neck mass, and left-sided vocal cord paralysis. Further work-up was suggestive of MTC, which prompted RET testing for multiple endocrine neoplasia. The patient underwent left thyroid lobectomy and selective lymph node dissection but later required tracheostomy because of tumoral invasion of the trachea, laser debulking of the tumor, and external beam radiation therapy. One year postoperatively, development of a metastatic lesion in her right ovary necessitated a second oophorectomy.ConclusionMTC usually manifests as a solitary thyroid nodule but should be considered in patients with metastatic lesions characterized by neuroendocrine features. This unusual case highlights the biologic and clinical variability of this often aggressive thyroid cancer, which necessitates an attentive work-up, a rigorous operative strategy, and a periodic postoperative surveillance program. (Endocr Pract. 2008;14:351-357)     

An Unusual Case of Recurrent Hyperparathyroidism and Papillary Thyroid Cancer

ObjectiveTo report an unusual occurrence of recurrent hyperparathyroidism due to papillary thyroid carcinoma.MethodsWe describe the clinical history, physical examination findings, laboratory values, imaging findings, and pathologic findings of a woman who developed recurrent hyperparathyroidism 13 years after successful parathyroidectomy.ResultsA 59-year-old woman presented to our clinic with recurrent primary hyperparathyroidism. In 1994, she presented with nephrolithiasis and underwent resection of a right superior parathyroid adenoma that resulted in clinical and biochemical cure. Her clinical course had been followed at periodic intervals, and she had been symptom-free and normocalcemic. In 2007, she again developed nephrolithiasis and was documented to have recurrent hyperparathyroidism. Imaging studies suggested a parathyroid adenoma near the right inferior pole of the thyroid. The patient had reoperative neck exploration. No obvious parathyroid adenoma was found and a right thyroid lobectomy was performed, which resulted in normalization of intraoperative intact parathyroid hormone levels, and the incision was closed. Final pathology demonstrated no parathyroid adenoma, but instead, a 1-cm papillary thyroid carcinoma that stained positive for parathyroid hormone. More than 6 months after surgery, she remains clinically and biochemically cured.ConclusionsRecurrent hyperparathyroidism occurs secondary to multiple causes. This case demonstrates the challenge a surgeon faces in managing recurrent disease and highlights a rare phenomenon of papillary thyroid cancer causing recurrent hyperparathyroidism. (Endocr Pract. 2009;15:349-352)     

Octreotide Therapy for Recurrent Refractory Hypoglycemia Due to Sulfonylurea In Diabetes-Related Kidney Failure

ObjectiveTo describe a patient with kidney insufficiency from diabetes treated with glyburide, who presented with prolonged and recurrent hypoglycemia unresponsive to large intravenous doses of glucose, which was treated successfully with intravenously administered octreotide, and to review the therapeutic options for hypoglycemia.MethodsWe present a case report of a 66-year-old man with diabetes causing chronic kidney disease, who was treated with orally administered glyburide, 7.5 mg twice a day. He initially presented to another hospital because of hypoglycemia and was treated with intravenously administered glucose and discharged. The next day, his family brought him to our emergency department because of recurring low blood glucose levels and symptoms of sweating, fever, and nightmares. Laboratory tests revealed a blood glucose level of 33 mg/dL and a creatinine concentration of 6.2 mg/dL.ResultsThe patient was treated with a 5% dextrose and, subsequently, a 10% dextrose infusion without any sustained improvement. The blood glucose level remained low despite the additional administration of 3 ampules of 50% dextrose in water. The patient was given a bolus of octreotide (50 μg subcutaneously) 14 hours after his second presentation. He received another 50-μg dose of octreotide 6 hours later. After this bolus, the hypoglycemia resolved, and he no longer required intravenous administration of glucose to maintain euglycemia.ConclusionPatients with diabetes and kidney disease frequently have persistent and difficult-to-treat hypoglycemia, unresponsive to conventional therapy. Octreotide is an effective and safe treatment for patients with refractory hypoglycemia attributable to sulfonylureas. (Endocr Pract. 2007;13:417-423)     

Tall-Cell Variant Papillary Thyroid Carcinoma Arising from Struma Ovarll

ObjectiveTo present a case of tall-cell variant (TCV) papillary thyroid carcinoma (PTC) arising from Struma ovarii (SO) and to discuss special considerations in the management of this patient.MethodsThe clinical presentation and relevant pathologic features of a patient with PTC-TCV developing from SO are described, and a concise review of literature regarding this topic is also presented.ResultsA 36-year-old woman with a history of stable right ovarian dermoid cyst presented with amenorrhea and was found to have a significantly enlarged right ovary with multiple cysts. Following laparoscopic cystectomy, pathology revealed mature cystic teratoma (SO) with associated PTC-TCV. Based on this finding, she underwent right salpingo-oophorectomy, right pelvic lymph node dissection, and partial omentectomy. Pathology was negative for extra-ovarian disease, and her tumor was staged as pT1pN0M0. Total thyroidectomy was performed in preparation for radioactive iodine (RAI) therapy. A diagnostic iodine-131 (I-131) scan showed residual uptake in the neck with faint uptake in the lower left quadrant of the abdomen and was followed by therapy with 90 mCi of I-131. The patient had an unremarkable course with no clinical or biochemical evidence of disease recurrence to date.ConclusionsThis is to our knowledge the first reported case of TCV-PTC arising from SO. The presence of this aggressive variant of PTC factored into our decision to proceed with thyroidectomy and I-131 ablation, despite the lack of conclusive evidence in the literature. Recent discoveries on the natural history of thyroid-derived TCV-PTC were critical in choosing the appropriate management for this patient’s disease. (Endocr Pract. 2014;20:e24-e27)     

Confirmation of Hypoglycemia in the “Dead-In-Bed” Syndrome,as Captured by a Retrospective Continuous Glucose Monitoring System

ObjectiveTo report a case that substantiates the presence of hypoglycemia at the time of death of a young man with type 1 diabetes, who was found unresponsive in his undisturbed bed in the morning.MethodsWe describe a 23-year-old man with a history of type 1 diabetes treated with an insulin pump, who had recurrent severe hypoglycemia. In an effort to understand these episodes better and attempt to eliminate them, a retrospective (non-real-time) continuous subcutaneous glucose monitoring system (CGMS) was attached to the patient. He was found dead in his undisturbed bed 20 hours later. The insulin pump and CGMS were both downloaded for postmortem study.ResultsPostmortem download of the data in the CGMS demonstrated glucose levels below 30 mg/dL around the time of his death, with only a minimal counterregulatory response. This finding corresponded to a postmortem vitreous humor glucose of 25 mg/dL. An autopsy showed no major anatomic abnormalities that could have contributed to his death.ConclusionTo our knowledge, this is the first documentation of hypoglycemia at the time of death in a patient with the “dead-in-bed” syndrome. This report should raise the awareness of physicians to the potentially lethal effectsof hypoglycemia and provide justification for efforts directed at avoiding nocturnal hypoglycemia. (Endocr Pract. 2010;16:244-248)     

Recurrent Takotsubo Cardiomyopathy Associated with Pheochromocytoma

ObjectiveTo describe a case of recurrent takotsubo cardiomyopathy in a patient with pheochromocytoma.MethodsWe present a case report, including clinical and laboratory data. In addition, the current relevant literature pertaining to pheochromocytoma and takotsubo syndrome is reviewed and summarized.ResultsIn 2004, an 81-year-old woman with no history of cardiac disease presented with chest discomfort, and takotsubo syndrome was diagnosed. No emotional or physical stressors were identified at that time. Her left ventricular systolic function normalized during that hospitalization. In 2007, the patient was readmitted to the hospital with chest discomfort and ST-segment elevation. Cardiac catheterization demonstrated only minor nonobstructive coronary artery disease. She was again found to have takotsubo syndrome with a classic apical hypokinetic segment. Treatment with a heart failure regimen was initiated, and she was screened for pheochromocytoma as the precipitant for her recurrent takotsubo cardiomyopathy. A 24-hour urine collection showed minimally elevated normetanephrine excretion of 719 μg (reference range, 148 to 560) and vanillylmandelic acid of 8.3 mg (reference range, < 8.0). The plasma normetanephrine level was 1.57pg/mL (reference range, < 0.9). Subsequent magnetic resonance imaging revealed a left adrenal mass (2 cm by 1 cm). Ultimately, the patient underwent left adrenalectomy, and the pathology report was consistent with pheochromocytoma. She has been asymptomatic since then, and a repeated echocardiogram demonstrated normal left ventricular systolic function.ConclusionIn patients presenting with takotsubo cardiomyopathy, a precipitating factor, such as emotional or physical stress, can often be identified. In some patients (such as our current case), however, pheochromocytoma may be the underlying disease and should be considered. (Endocr Pract. 2009;15:560-562)     

复发卵巢成人型颗粒细胞瘤的临床特点及复发的影响因素分析

摘要 目的：探讨复发卵巢成人型颗粒细胞瘤（AGCT）的临床特点及复发的影响因素。方法：回顾性分析我院收治的24例复发AGCT患者的临床资料。结果：2014年1月-2021年12月在四川大学华西第二医院共收治卵巢成人型颗粒细胞瘤的患者97例,复发AGCT患者24例,复发率24.7 %。初次复发距离初次治疗的中位间隔时间为69个月（24月-144月）。24例复发AGCT初发平均年龄42.8岁（24岁-60岁）。初诊时肿瘤最大直径<10 cm 15例,肿瘤最大直径≥10 cm 9例。初次FIGO分期：I期14例,占58.3 %,II期3例,占12.5 %,III期7例,占29.1 %。I期患者中肿瘤破裂8例。所有病例初次治疗时均接受手术治疗。保留生育功能手术8例,接受非保留生育功能的手术16例。初次手术后14例患者接受了化疗,其中I期患者14例,有4例接受辅助化疗,有10例术后未接受辅助化疗。复发后有7例患者发生多次复发。Cox回归模型分析显示FIGO分期、I期患者肿瘤破裂为导致复发卵巢成人型颗粒细胞瘤复发的危险因素（P<0.05）。结论：AGCT为低度恶性肿瘤,有远期复发和多次复发的风险,FIGO分期是影响复发的因素,晚期的患者更易复发。对于部分临床早期患者,肿瘤破裂也会增加复发的风险。AGCT患者需长期随访。     

Risks and Benefits of Estrogen Therapy for a Male-to-Female Transsexual with a Prothrombin Gene Mutation

ObjectiveWe present the case of a male-to-female transsexual person in her 20s requesting hormone therapy in the setting of a history of a deep venous thrombosis and pulmonary embolus and carrying the prothrombin G20210A gene mutation.MethodsWe interviewed the patient and reviewed her medical records. We carefully weighed the risks and benefits of hormone therapy and took into account two important ethical principles: beneficence (to act in the patient’s best interest) and nonmaleficence (to avoid harm).ResultsOur patient presented to an outside facility with weight loss, generalized weakness, right lower extremity swelling, and chest pain. She was diagnosed with a pulmonary embolus and extensive deep venous thrombus by computed tomography (CT) scan and Doppler ultrasound, respectively. She was found to carry the pro-thrombin G20210A gene mutation. She was treated with anticoagulation therapy for 12 months, which was restarted prior to beginning therapy with transdermal estrogen.ConclusionWhile the exact risk of recurrent deep venous thrombosis and pulmonary embolus in our patient is unknown, we recommended that hormone therapy should only be given in conjunction with anticoagulation. We speculate that this strategy would allow the patient to experience the benefits to her overall well-being with hormone therapy while reducing the risks of venous thrombosis to acceptable levels. Prospective long-term follow-up of this patient is needed to verify the benefits and risk of the intervention chosen. (Endocr. Pract. 2013;19:e150-e153)     

Case report of acute necrotizing pancreatitis associated with combination treatment of sitagliptin and exenatide

ObjectiveTo report the first postmarketing case of necrotizing pancreatitis in a patient on combination therapy of sitagliptin and exenatide.MethodsWe describe the patient’s clinical presentation, laboratory test results, imaging, and autopsy findings.ResultsA 76-year-old woman with a history of type 2 diabetes mellitus presented with severe abdominal pain, vomiting, and fever requiring hospital admission. She had been treated with exenatide for 3 years to manage her diabetes mellitus. A few weeks before presentation, sitagliptin was added, presumably to further optimize her glycemic control. Acute pancreatitis was diagnosed during hospital admission. At initial presentation, her serum amylase concentration was 1136 U/L (reference range, 10-130 U/L) and her lipase concentration was greater than 3500 U/L (reference range, 0-75 U/L). In addition, computed tomography of the abdomen and pelvis demonstrated extensive pancreatic parenchymal necrosis. She had undergone previous cholecystectomy, reported no alcohol consumption, and had a normal lipid profile. Although she had a long-standing history of diabetes mellitus, she had no history of pancreatitis or other risk factors that would have caused her to develop the underlying condition. After initial brief improvement, her symptoms worsened, and despite aggressive care, her clinical state deteriorated and she died. Autopsy findings demonstrated acute necrotizing pancreatitis with complete digestion of the pancreas.ConclusionsConsidering the temporal relationship of her symptoms to the addition of sitagliptin to her existing exenatide regimen, this case strongly suggests a possible causal link between exenatide or sitagliptin (or the combination of the 2 drugs) and the etiology of pancreatitis in this patient. (Endocr Pract. 2012;18:e10-e13)     

Metformin-Responsive Classic Salt-Losing Congenita L Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency: a Case Report

ObjectiveTo study the effect of adding metformin to standard steroid replacement therapy in a patient with classic salt-losing congenital adrenal hyperplasia due to 21- hydroxylase deficiency with suboptimal biochemical and clinical control.MethodsWe present the clinical and laboratory findings before and after the addition of metformin to the therapeutic regimen of the study patient.ResultsA 17-year-old girl had been diagnosed as a neonate with classic salt-losing congenital adrenal hyperplasia caused by 21-hydroxylase deficiency (CYP21A2 deficiency). She was treated with hydrocortisone, 20 mg in the morning and 10 mg at bedtime, and fludrocortisone, 50 mcg daily. While on steroid replacement, she maintained normal serum electrolytes, glucose, blood pressure, and external genitalia, but she continued to express clinical features of obesity, hirsutism, amenorrhea, and acanthosis nigricans. Elevated laboratory measurements included the following: fasting 17-hydroxyprogesterone, 3410 ng/dL; total testosterone, 326 ng/dL; and androstenedione, 390 ng/dL. She was initiated on metformin, 500 mg twice daily after meals. After 3 months, the patient lost 2 kg, amenorrhea resolved, 17-hydroxyprogesterone decreased to 1539 ng/dL, total testosterone decreased to 163 ng/dL, and androstenedione levels remained unchanged.ConclusionsMetformin, an agent known to reduce insulin resistance, further suppressed the 17-hydroxyprogesterone concentration in a patient with classic congenital adrenal hyperplasia on steroid replacement therapy. Metformin may improve clinical and biochemical outcomes in classic congenital adrenal hyperplasia without the risk of iatrogenic Cushing syndrome. (Endocr Pract. 2008;14:889-891)     

Insulinoma in a Young Female Patient With Systemic Lupus Erythematosus: A Case Report

ObjectiveFasting hypoglycemia may occur in subjects with systemic lupus erythematosus (SLE) when accompanied with insulin-binding antibodies or insulin-receptor antibodies. However, insulinoma has not been reported in SLE subjects with hypoglycemia.MethodsWe present a case report and review the relevant literature.ResultsA 26-year-old female with underlying SLE experienced several episodes of neuropsychiatric symptoms in a fasting state. The steroid dosage was titrated up, but in vain. Timely imaging studies showed a pancreatic tumor, and insulinoma was proven by pathology. Hypoglycemia did not recur after surgery.ConclusionPhysicians should distinguish insulinoma from autoimmunity-mediated hypoglycemia in SLE patients with fasting hypoglycemia. (Endocr Pract. 2014; 20:e256-e259)