AACE Clinical Case Reports最新文献_第8页

Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease 先天性心脏病患者副神经节瘤多年的临床进展

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-11-01 DOI: 10.1016/j.aace.2023.09.003

Megan M. McConnell MD , Maralee R. Kanin MD , Martin S. Auerbach MD , Run Yu MD, PhD

Background

Documented symptomatic progression of a paraganglioma (PGL) over many years is unusual. Our objective is to report a young man with such an occurrence.

Case Report

A 27-year-old male presented with headache, sweating, and palpitation. He had a history of cyanotic congenital heart disease. Five years before presentation, he had 24-hour urine metanephrines 43 mcg/d (25-222), vanillylmandelic acid 3 mg/d (<6), and homovanillic acid 2.4 mg/d (1.6-7.5) levels and a 3.13 cm mass in the upper aortocaval space. Subsequent imaging showed slow growth of the mass. On admission, his blood pressure was 197/134 mm Hg, heart rate was 163 beats per minute, respiratory rate was 25 per minute, and oxygen saturation was 76% on room air. His 24-hour urine normetanephrine level was 2644 mcg/d (81-667) while metanephrine was 405 mcg/d (55-320). Plasma free metanephrine level was 0.92 nmol/L (0-0.49) and normetanephrine was 11.85 nmol/L (0-0.89). DOTATATE positron emission tomography–computed tomography revealed a 4.3 × 3.1 × 4.9 cm mass with activity in the right upper aortocaval space. He was treated with Prazosin. Two months later, he underwent resection of the mass. Pathology diagnosed a 4.9 cm PGL. He had improvement in metanephrine levels.

Discussion

PGL is diagnosed by documenting excess catecholamines and identifying a lesion on imaging. False negative laboratory testing is rare but can occur. Patients with cyanotic congenital heart disease have a greater risk of developing PGL.

Conclusion

It is crucial to evaluate a patient for PGL if clinical conditions suggest catecholamine excess, especially if a retroperitoneal tumor has grown or the patient has risk factors.

背景:文献记载的副神经节瘤(PGL)的症状进展超过多年是不寻常的。我们的目标是报告一个年轻人发生了这样的事情。病例报告一名27岁男性，表现为头痛、出汗和心悸。他有紫绀型先天性心脏病的病史。发病前5年，患者24小时尿肾上腺素43毫克/天(25-222)，香草酸(VMA) 3毫克/天(<6)，纯香草酸(HVA) 2.4毫克/天(1.6-7.5)，主动脉上腔间隙3.13 cm肿块。随后的影像学显示肿块生长缓慢。入院时，患者血压为197/134 mmHg，心率163次/分钟，呼吸频率25次/分钟，室内空气氧饱和度76%。24小时尿去甲肾上腺素为2644 McG /d(81 ~ 667)，肾上腺素为405 McG /d(55 ~ 320)。血浆游离肾上腺素0.92 nmol/L(0 ~ 0.49)，去甲肾上腺素11.85 nmol/L(0 ~ 0.89)。DOTATATE PET/CT显示一个4.3 x 3.1 x 4.9 cm的肿块，位于右上腹主动脉间隙。他接受了哌唑嗪治疗。两个月后，他接受了肿块切除术。病理诊断为4.9 cm PGL。他的肾上腺素水平有所改善。pgl的诊断是通过记录过量的儿茶酚胺和在影像学上确定病变。假阴性实验室检测是罕见的，但可能发生。CCHD患者发生PGL的风险更大。结论当临床情况提示儿茶酚胺过量时，尤其是腹膜后肿瘤生长或患者有危险因素时，评估PGL是至关重要的。

{"title":"Clinical Progression of a Paraganglioma Over Many Years in a Man With Congenital Heart Disease","authors":"Megan M. McConnell MD , Maralee R. Kanin MD , Martin S. Auerbach MD , Run Yu MD, PhD","doi":"10.1016/j.aace.2023.09.003","DOIUrl":"10.1016/j.aace.2023.09.003","url":null,"abstract":"<div><h3>Background</h3><p>Documented symptomatic progression of a paraganglioma (PGL) over many years is unusual. Our objective is to report a young man with such an occurrence.</p></div><div><h3>Case Report</h3><p>A 27-year-old male presented with headache, sweating, and palpitation. He had a history of cyanotic congenital heart disease. Five years before presentation, he had 24-hour urine metanephrines 43 mcg/d (25-222), vanillylmandelic acid 3 mg/d (<6), and homovanillic acid 2.4 mg/d (1.6-7.5) levels and a 3.13 cm mass in the upper aortocaval space. Subsequent imaging showed slow growth of the mass. On admission, his blood pressure was 197/134 mm Hg, heart rate was 163 beats per minute, respiratory rate was 25 per minute, and oxygen saturation was 76% on room air. His 24-hour urine normetanephrine level was 2644 mcg/d (81-667) while metanephrine was 405 mcg/d (55-320). Plasma free metanephrine level was 0.92 nmol/L (0-0.49) and normetanephrine was 11.85 nmol/L (0-0.89). DOTATATE positron emission tomography–computed tomography revealed a 4.3 × 3.1 × 4.9 cm mass with activity in the right upper aortocaval space. He was treated with Prazosin. Two months later, he underwent resection of the mass. Pathology diagnosed a 4.9 cm PGL. He had improvement in metanephrine levels.</p></div><div><h3>Discussion</h3><p>PGL is diagnosed by documenting excess catecholamines and identifying a lesion on imaging. False negative laboratory testing is rare but can occur. Patients with cyanotic congenital heart disease have a greater risk of developing PGL.</p></div><div><h3>Conclusion</h3><p>It is crucial to evaluate a patient for PGL if clinical conditions suggest catecholamine excess, especially if a retroperitoneal tumor has grown or the patient has risk factors.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 6","pages":"Pages 193-196"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2376060523001396/pdfft?md5=99d1f515acd38b218938f26164164633&pid=1-s2.0-S2376060523001396-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134917287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Primary Adrenal Insufficiency due to Cryptococcus With Persistent Adrenal Enlargement and Insufficiency 隐球菌所致原发性肾上腺功能不全伴持续肾上腺肿大和功能不全

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-11-01 DOI: 10.1016/j.aace.2023.09.002

Catherine E. Price MD, Cynthia Burns MD, Joseph A. Aloi MD

Background/Objective

Infiltrative fungal infections are an unusual cause of primary adrenal insufficiency (AI). Our objective is to present a long-term follow-up of a patient with AI due to cryptococcal adrenalitis.

Case Report

A 47-year-old woman presented in January 2004, with 50-lb weight loss, nausea, emesis, and headache with diplopia. During the 6 months prior to her presentation the patient had multiple admissions for evaluation of recurrent nausea and emesis. Prior to the most recent of these admissions, the patient developed a headache; evaluation of her cerebrospinal fluid revealed the presence of Cryptococcus, and she was treated with a 2-week course of amphotericin B. Physical examination demonstrated a temperature of 101.1 °F, heart rate of 110 bpm, and blood pressure of 94/65 mm Hg. She appeared ill and was underweight with dry mucous membranes and photophobia. Laboratory tests revealed random cortisol of 0.5 μg per dL. CT imaging showed bilateral adrenal gland enlargement and fine needle aspiration of the adrenal gland revealed encapsulated budding yeast. Stress dose intravenous glucocorticoids were administered and switched to oral hydrocortisone and fludrocortisone because the patient clinically improved with a second course of amphotericin B. Further evaluation in 2017 revealed persistently enlarged adrenal glands, positive cryptococcus antigen, and low IgG levels.

Discussion

Our literature review noted few publications of AI caused by disseminated cryptococcus with no long-term follow-up of these cases beyond a 1- to 4-year time frame.

Conclusion

Patients with AI due to disseminated fungal infection need long-term follow-up to assess for resolution of adrenal enlargement and evaluation of immunocompromised status.

背景/目的浸润性真菌感染是原发性肾上腺功能不全(AI)的罕见病因。我们的目的是对一例隐球菌性肾上腺炎所致AI患者进行长期随访。病例报告:一名47岁女性，2004年1月出现体重减轻50磅，恶心，呕吐，头痛伴复视。在她就诊前的6个月，患者多次入院评估复发性恶心和呕吐。在最近一次入院之前，患者出现头痛;脑脊液检查发现隐球菌，并给予两周两性霉素b疗程。体格检查显示体温101.1°F，心率110 bpm，血压94/65 mmHg，体重过轻，黏膜干燥，畏光。实验室检测随机皮质醇0.5 ug/dL。CT示双侧肾上腺肿大，肾上腺FNA示包被芽孢酵母。患者给予应激剂量静脉注射糖皮质激素后，经第二疗程两性霉素b治疗后临床好转，改为口服氢化可的松和氟化可的松。2017年进一步评估发现肾上腺持续增大，隐球菌抗原阳性，IgG水平低。我们的文献综述注意到很少有关于播散性隐球菌引起的AI的出版物，这些病例没有超过1-4年的长期随访。结论弥散性真菌感染所致AI患者需要长期随访，以评估肾上腺肿大的消退情况和免疫功能低下状况。

{"title":"Primary Adrenal Insufficiency due to Cryptococcus With Persistent Adrenal Enlargement and Insufficiency","authors":"Catherine E. Price MD, Cynthia Burns MD, Joseph A. Aloi MD","doi":"10.1016/j.aace.2023.09.002","DOIUrl":"10.1016/j.aace.2023.09.002","url":null,"abstract":"<div><h3>Background/Objective</h3><p>Infiltrative fungal infections are an unusual cause of primary adrenal insufficiency (AI). Our objective is to present a long-term follow-up of a patient with AI due to cryptococcal adrenalitis.</p></div><div><h3>Case Report</h3><p>A 47-year-old woman presented in January 2004, with 50-lb weight loss, nausea, emesis, and headache with diplopia. During the 6 months prior to her presentation the patient had multiple admissions for evaluation of recurrent nausea and emesis. Prior to the most recent of these admissions, the patient developed a headache; evaluation of her cerebrospinal fluid revealed the presence of <em>Cryptococcus</em>, and she was treated with a 2-week course of amphotericin B. Physical examination demonstrated a temperature of 101.1 °F, heart rate of 110 bpm, and blood pressure of 94/65 mm Hg. She appeared ill and was underweight with dry mucous membranes and photophobia. Laboratory tests revealed random cortisol of 0.5 μg per dL. CT imaging showed bilateral adrenal gland enlargement and fine needle aspiration of the adrenal gland revealed encapsulated budding yeast. Stress dose intravenous glucocorticoids were administered and switched to oral hydrocortisone and fludrocortisone because the patient clinically improved with a second course of amphotericin B. Further evaluation in 2017 revealed persistently enlarged adrenal glands, positive cryptococcus antigen, and low IgG levels.</p></div><div><h3>Discussion</h3><p>Our literature review noted few publications of AI caused by disseminated cryptococcus with no long-term follow-up of these cases beyond a 1- to 4-year time frame.</p></div><div><h3>Conclusion</h3><p>Patients with AI due to disseminated fungal infection need long-term follow-up to assess for resolution of adrenal enlargement and evaluation of immunocompromised status.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 6","pages":"Pages 189-192"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2376060523001384/pdfft?md5=acdf28154fce382b5bdc781979bb5c2a&pid=1-s2.0-S2376060523001384-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135434338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Sellar Mass in 2 Patients With Acute-Onset Headache and Visual Symptoms: Not Your Usual Pituitary Adenoma 鞍区肿块2例伴有急性头痛和视觉症状:不是常见的垂体腺瘤

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-11-01 DOI: 10.1016/j.aace.2023.09.004

Run Yu MD, PhD

Background/Objective

Clinical diagnosis of rare aggressive sellar malignancies requires a high index of suspicion. The objective was to report 2 patients with primary sellar atypical teratoid (AT)/rhabdoid tumor (RT) who presented with acute-onset headache and visual symptoms.

Case Report

Patient 1 was a 45-year-old woman who presented with 3 weeks of headache and 1 week of eye pain and diplopia. Magnetic resonance imaging (MRI) identified a 2.2-cm sellar mass. Pituitary hormone testing showed elevated prolactin and suppressed luteinizing hormone, follicle-stimulating hormone, and estradiol levels. Patient 2 was a 32-year-old woman who presented with 1 month of headache and 1 week of diplopia. MRI showed a 2.1-cm sellar mass. Hormonal test results were reportedly unremarkable. Both patients did not have a significant medical history. They each underwent transsphenoidal resection. Surgical histology and molecular studies were consistent with primary sellar AT/RT. After surgery, patient 1 developed bilateral blindness and was lost to follow-up. Patient 2 developed hypopituitarism; her visual symptoms improved temporarily but recurred 2 weeks later. Pituitary MRI showed sellar recurrence. She underwent further debulking, but the tumor recurred promptly again. Despite radiation therapy, she died 4 months after the original presentation.

Discussion

AT/RT appears to be the most aggressive sellar malignancy.

Conclusion

Based on the 2 cases presented and the literature, I conclude that rapidly progressive headache with subsequent visual impairment in women with large sellar masses is almost pathognomonic of sellar AT/RT.

背景/目的临床诊断罕见的侵袭性鞍区恶性肿瘤需要高度的怀疑。目的是报告2例原发性鞍不典型畸胎瘤/横纹肌样瘤(AT/RT)患者，他们表现为急性头痛和视觉症状。病例报告:患者1是一名45岁女性，表现为头痛3周，眼痛和复视1周。MRI示2.2 cm鞍区肿块。垂体激素测试显示，催乳素升高，黄体生成素、卵泡刺激素和雌二醇水平下降。患者2为32岁女性，头痛1个月，复视1周。MRI示2.1 cm鞍区肿块。据报道，激素测试结果并不显著。两例患者均无明显既往病史。他们都接受了经蝶窦切除术。手术组织学和分子研究与原发性鞍区AT/RT一致。术后患者1出现双侧失明，失访。患者2出现垂体功能减退;她的视力症状暂时改善，但2周后复发。垂体MRI显示鞍区复发。她接受了进一步的切除手术，但肿瘤很快又复发了。尽管接受了放射治疗，她还是在最初的症状出现4个月后去世了。at /RT似乎是最具侵袭性的鞍恶性肿瘤。结论基于这两个病例和文献，我得出结论:大鞍区肿块妇女的快速进展性头痛并随后的视力损害几乎是鞍区AT/RT的典型病理特征。

{"title":"Sellar Mass in 2 Patients With Acute-Onset Headache and Visual Symptoms: Not Your Usual Pituitary Adenoma","authors":"Run Yu MD, PhD","doi":"10.1016/j.aace.2023.09.004","DOIUrl":"10.1016/j.aace.2023.09.004","url":null,"abstract":"<div><h3>Background/Objective</h3><p>Clinical diagnosis of rare aggressive sellar malignancies requires a high index of suspicion. The objective was to report 2 patients with primary sellar atypical teratoid (AT)/rhabdoid tumor (RT) who presented with acute-onset headache and visual symptoms.</p></div><div><h3>Case Report</h3><p>Patient 1 was a 45-year-old woman who presented with 3 weeks of headache and 1 week of eye pain and diplopia. Magnetic resonance imaging (MRI) identified a 2.2-cm sellar mass. Pituitary hormone testing showed elevated prolactin and suppressed luteinizing hormone, follicle-stimulating hormone, and estradiol levels. Patient 2 was a 32-year-old woman who presented with 1 month of headache and 1 week of diplopia. MRI showed a 2.1-cm sellar mass. Hormonal test results were reportedly unremarkable. Both patients did not have a significant medical history. They each underwent transsphenoidal resection. Surgical histology and molecular studies were consistent with primary sellar AT/RT. After surgery, patient 1 developed bilateral blindness and was lost to follow-up. Patient 2 developed hypopituitarism; her visual symptoms improved temporarily but recurred 2 weeks later. Pituitary MRI showed sellar recurrence. She underwent further debulking, but the tumor recurred promptly again. Despite radiation therapy, she died 4 months after the original presentation.</p></div><div><h3>Discussion</h3><p>AT/RT appears to be the most aggressive sellar malignancy.</p></div><div><h3>Conclusion</h3><p>Based on the 2 cases presented and the literature, I conclude that rapidly progressive headache with subsequent visual impairment in women with large sellar masses is almost pathognomonic of sellar AT/RT.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 6","pages":"Pages 197-200"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2376060523001402/pdfft?md5=b69ea4bbf02f6fd6326e6652726e77dd&pid=1-s2.0-S2376060523001402-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135588619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The Use of Ovarian Vein Sampling to Lateralize a Virilizing Leydig Cell Ovarian Tumor 卵巢静脉取样在卵巢间质细胞瘤病毒化中的应用

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-11-01 DOI: 10.1016/j.aace.2023.07.003

Kirun Baweja MbChB , Shirley Shuster MD , Sara Awad MBBS, MHPE

Background/Objective

Leydig cell tumors are a rare androgen-secreting ovarian tumor. We present a patient with virilization symptoms secondary to a Leydig cell tumor, with nonrevealing imaging studies, that was localized using ovarian vein sampling (OVS).

Case Report

A 56-year-old postmenopausal woman was referred by her gynecologist to the endocrinology clinic for voice-deepening, clitoral enlargement, scalp hair loss, and excessive body hair growth. Her total testosterone was 11.5 (0.3-1.3 nmol/L), bioavailable testosterone was 7.19 (0.1-0.6 nmol/L), and dehydroepiandrosterone sulfate was 4.0 (0.8-4.9 μmol/L). Transvaginal ultrasound and abdominal magnetic resonance imaging showed no adrenal or ovarian masses bilaterally. On adrenal vein sampling (AVS) and OVS, total testosterone from the left gonadal vein was 780.0 (0.3-1.3 nmol/L) and right gonadal vein was 18.6 (0.3-1.3 nmol/L), with a left-to-right ovarian testosterone ratio of 41.94. A bilateral salpingo-oophorectomy was performed, and a 1.0 cm Leydig cell tumor in the left ovary was noted on histopathology. One month after surgery, her total and bioavailable testosterone were <0.4 (0.3-1.3 nmol/L and 0.1-0.6 nmol/L, respectively). At 6 months, she had normalization of her voice to baseline, decreased clitoral size, decreased hair growth on her back, and improvement in her male-pattern baldness.

Discussion

OVS and AVS are useful diagnostic investigation tools in cases of virilization, in which imaging is nonrevealing. Our case supports previously suggested left-to-right ovarian vein testosterone ratio of ≥15 being associated with a left-sided tumor.

Conclusion

Few cases have been published on the interpretation of AVS and OVS in the setting of virilization. Previously suggested ratios for lateralization were valid for this patient.

背景/目的卵巢上皮细胞瘤是一种罕见的雄激素分泌性卵巢肿瘤。我们报告了一个继发于间质细胞瘤的男性化症状的患者，使用卵巢静脉取样(OVS)进行了定位。病例报告一名56岁绝经后妇女因声音加深、阴蒂增大、头皮脱发和体毛过多被妇科医生转介到内分泌科诊所。总睾酮为11.5 (0.3 ~ 1.3 nmol/L)，生物可利用睾酮为7.19 (0.1 ~ 0.6 nmol/L)，硫酸脱氢表雄酮为4.0 (0.8 ~ 4.9 μmol/L)。经阴道超声及腹部磁共振显示双侧未见肾上腺或卵巢肿物。肾上腺静脉取样(AVS)和OVS结果显示，左生殖腺静脉总睾酮为780.0 (0.3 ~ 1.3 nmol/L)，右生殖腺静脉总睾酮为18.6 (0.3 ~ 1.3 nmol/L)，左右卵巢睾酮比为41.94。行双侧输卵管卵巢切除术，病理检查发现左侧卵巢有一1.0 cm的间质细胞瘤。术后1个月，总睾酮和生物可利用睾酮为0.4(分别为0.3 ~ 1.3 nmol/L和0.1 ~ 0.6 nmol/L)。6个月时，患者的声音恢复到基线水平，阴蒂尺寸减小，背部毛发生长减少，男性型秃发有所改善。讨论ovs和AVS是男性化病例中有用的诊断调查工具，其中成像不明显。我们的病例支持先前提出的左侧卵巢静脉睾丸激素比≥15与左侧肿瘤相关的建议。结论关于AVS和OVS在男性化背景下的解释，文献报道较少。先前建议的偏侧比例对该患者有效。

{"title":"The Use of Ovarian Vein Sampling to Lateralize a Virilizing Leydig Cell Ovarian Tumor","authors":"Kirun Baweja MbChB , Shirley Shuster MD , Sara Awad MBBS, MHPE","doi":"10.1016/j.aace.2023.07.003","DOIUrl":"10.1016/j.aace.2023.07.003","url":null,"abstract":"<div><h3>Background/Objective</h3><p>Leydig cell tumors are a rare androgen-secreting ovarian tumor. We present a patient with virilization symptoms secondary to a Leydig cell tumor, with nonrevealing imaging studies, that was localized using ovarian vein sampling (OVS).</p></div><div><h3>Case Report</h3><p>A 56-year-old postmenopausal woman was referred by her gynecologist to the endocrinology clinic for voice-deepening, clitoral enlargement, scalp hair loss, and excessive body hair growth. Her total testosterone was 11.5 (0.3-1.3 nmol/L), bioavailable testosterone was 7.19 (0.1-0.6 nmol/L), and dehydroepiandrosterone sulfate was 4.0 (0.8-4.9 μmol/L). Transvaginal ultrasound and abdominal magnetic resonance imaging showed no adrenal or ovarian masses bilaterally. On adrenal vein sampling (AVS) and OVS, total testosterone from the left gonadal vein was 780.0 (0.3-1.3 nmol/L) and right gonadal vein was 18.6 (0.3-1.3 nmol/L), with a left-to-right ovarian testosterone ratio of 41.94. A bilateral salpingo-oophorectomy was performed, and a 1.0 cm Leydig cell tumor in the left ovary was noted on histopathology. One month after surgery, her total and bioavailable testosterone were <0.4 (0.3-1.3 nmol/L and 0.1-0.6 nmol/L, respectively). At 6 months, she had normalization of her voice to baseline, decreased clitoral size, decreased hair growth on her back, and improvement in her male-pattern baldness.</p></div><div><h3>Discussion</h3><p>OVS and AVS are useful diagnostic investigation tools in cases of virilization, in which imaging is nonrevealing. Our case supports previously suggested left-to-right ovarian vein testosterone ratio of ≥15 being associated with a left-sided tumor.</p></div><div><h3>Conclusion</h3><p>Few cases have been published on the interpretation of AVS and OVS in the setting of virilization. Previously suggested ratios for lateralization were valid for this patient.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 6","pages":"Pages 182-185"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2376060523001281/pdfft?md5=545505b0885f52fc704f17c24f420151&pid=1-s2.0-S2376060523001281-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48949072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Editorial for November/December Issue of AACE Clinical Case Reports AACE临床病例报告十一月/十二月号社论

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-11-01 DOI: 10.1016/j.aace.2023.10.007

Sina Jasim MD, MPH (Editor in Chief)

引用次数: 0

Rare Neurologic Presentation of Thyrotoxicosis 甲状腺毒性疾病的罕见神经系统表现。

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-09-01 DOI: 10.1016/j.aace.2023.06.002

Kalkidan Alachew, Keberte Tsegaye

引用次数: 1

Hypertensive Crisis in a Patient With a Functioning Mesenteric Paraganglioma: Dramatic Response to Octreotide Treatment 功能性肠系膜副神经节瘤患者的高血压危象：奥曲肽治疗的显著反应。

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-09-01 DOI: 10.1016/j.aace.2023.05.003

Omayma T. Elshafie MBBS, MD , Abir C. Bou Khalil MD , Maha A. Alshaibi MD , Boris L. Itkin MD, MSc , Babikir M. Ismail MBBS, MD , Nicholas J. Woodhouse OBE

Background/Objective

To report a dramatic and immediate clinical and biochemical response during treatment with octreotide in a patient with a functioning mesenteric paraganglioma (PGL).

Case Report

A 44-year-old woman was admitted with a severe hypertensive crisis and a blood pressure reaching 260/150 mm Hg. She was 2 months postpartum and had been previously diagnosed with pre-eclampsia. Secondary hypertension was suspected. This was confirmed by finding a 6 × 5-cm² retroperitoneal mass located using 68-Gallium DOTA–octreotate positron emission tomography/computed tomography and a grossly elevated plasma catecholamine level of 93 000 pmol/L (normal reference range: 650-2433 pmol/L). Treatment was immediately started with high doses of long- and short-acting octreotide. After 6 weeks and before surgery, the patient was normotensive, with a blood pressure of 120/70 mm Hg and a norepinephrine level of 6000 pmol/L. The tumor resection was uneventful, and histology confirmed the diagnosis. Following the surgery, the patient remained normotensive without any medications.

Discussion

PGLs and pheochromocytomas are neuroendocrine tumors, and most have receptors for octreotide. This case and another patient previously reported responded dramatically to treatment with a high dose of octreotide. Earlier reports of patients failing to respond are likely to have been the result of using a smaller octreotide dose.

Conclusion

We conclude that high doses of short- and long-acting octreotide are valuable in severely hypertensive patients. Our experience suggests that octreotide is of value in other patients with PGLs and pheochromocytomas. The response is rapid, sustained, effective, and with minimal reported side effects. To the best of our knowledge, this is the first report of a hypertensive crisis in a functional mesenteric PGL.

背景/目的：报告一例功能性肠系膜副神经节瘤（PGL）患者在奥曲肽治疗过程中出现的显著且即时的临床和生化反应。病例报告：一名44岁女性因严重高血压危象入院，血压达到260/150mm Hg。她产后2个月，之前被诊断为先兆子痫。怀疑为继发性高血压。这一点通过使用68镓DOTA奥曲酸正电子发射断层扫描/计算机断层扫描定位的6×5 cm2腹膜后肿块和93 000 pmol/L的血浆儿茶酚胺水平显著升高（正常参考范围：650-2433 pmol/L）得到了证实。立即开始高剂量长效和短效奥曲肽的治疗。6周后和手术前，患者血压正常，血压为120/70mm Hg，去甲肾上腺素水平为6000pmol/L。肿瘤切除顺利，组织学证实了诊断。手术后，患者在没有任何药物的情况下保持血压正常。讨论：PGL和嗜铬细胞瘤是神经内分泌肿瘤，大多数都有奥曲肽受体。该病例和之前报道的另一名患者对高剂量奥曲肽的治疗反应显著。早期关于患者没有反应的报道可能是使用较小剂量的奥曲肽的结果。结论：我们得出结论，高剂量的短效和长效奥曲肽对严重高血压患者是有价值的。我们的经验表明，奥曲肽对其他PGL和嗜铬细胞瘤患者有价值。反应迅速、持续、有效，副作用最小。据我们所知，这是第一例功能性肠系膜PGL出现高血压危象的报告。

{"title":"Hypertensive Crisis in a Patient With a Functioning Mesenteric Paraganglioma: Dramatic Response to Octreotide Treatment","authors":"Omayma T. Elshafie MBBS, MD , Abir C. Bou Khalil MD , Maha A. Alshaibi MD , Boris L. Itkin MD, MSc , Babikir M. Ismail MBBS, MD , Nicholas J. Woodhouse OBE","doi":"10.1016/j.aace.2023.05.003","DOIUrl":"10.1016/j.aace.2023.05.003","url":null,"abstract":"<div><h3>Background/Objective</h3><p>To report a dramatic and immediate clinical and biochemical response during treatment with octreotide in a patient with a functioning mesenteric paraganglioma (PGL).</p></div><div><h3>Case Report</h3><p>A 44-year-old woman was admitted with a severe hypertensive crisis and a blood pressure reaching 260/150 mm Hg. She was 2 months postpartum and had been previously diagnosed with pre-eclampsia. Secondary hypertension was suspected. This was confirmed by finding a 6 × 5-cm<sup>2</sup> retroperitoneal mass located using 68-Gallium DOTA–octreotate positron emission tomography/computed tomography and a grossly elevated plasma catecholamine level of 93 000 pmol/L (normal reference range: 650-2433 pmol/L). Treatment was immediately started with high doses of long- and short-acting octreotide. After 6 weeks and before surgery, the patient was normotensive, with a blood pressure of 120/70 mm Hg and a norepinephrine level of 6000 pmol/L. The tumor resection was uneventful, and histology confirmed the diagnosis. Following the surgery, the patient remained normotensive without any medications.</p></div><div><h3>Discussion</h3><p>PGLs and pheochromocytomas are neuroendocrine tumors, and most have receptors for octreotide. This case and another patient previously reported responded dramatically to treatment with a high dose of octreotide. Earlier reports of patients failing to respond are likely to have been the result of using a smaller octreotide dose.</p></div><div><h3>Conclusion</h3><p>We conclude that high doses of short- and long-acting octreotide are valuable in severely hypertensive patients. Our experience suggests that octreotide is of value in other patients with PGLs and pheochromocytomas. The response is rapid, sustained, effective, and with minimal reported side effects. To the best of our knowledge, this is the first report of a hypertensive crisis in a functional mesenteric PGL.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 5","pages":"Pages 149-152"},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10509379/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41100376","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Rash in a Patient With New-Onset Type 1 Diabetes Mellitus 新发1型糖尿病患者皮疹。

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-09-01 DOI: 10.1016/j.aace.2023.07.004

Amr Wahba MD, Amie Ogunsakin MD

引用次数: 1

Amelioration of Paget Disease of Bone After Denosumab for Osteopenia Denosumab治疗骨质疏松症后骨Paget病的改善。

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-09-01 DOI: 10.1016/j.aace.2023.05.007

Vijayvardhan Kamalumpundi BS , Elham Shams MD , Maisoon Torfah MD , Marcelo L. Correia MD, MSc, PhD

Background/Objective

Denosumab is a monoclonal antibody that inhibits bone resorption and is indicated for the treatment of osteoporosis, bone metastases, and giant cell tumor of bone. We describe a woman with symptomatic Paget disease of the skull whose headaches and monostotic disease of the skull improved after receiving denosumab for concomitant low bone density.

Case Report

A 75-year-old woman presented with unremitting headache of 1 month. She had a medical history of polymyalgia rheumatica, osteopenia, hypothyroidism, and gastroesophageal reflux disease. She reported taking prednisone 1 to 20 mg daily for polymyalgia rheumatica for 1 year and received a dose of denosumab 60 mg for osteopenia 1 month before presentation. The calcium, alkaline phosphatase, and bone-specific alkaline phosphatase levels were 8.2 mg/dL (reference range [RR], 8.5-10.5 mg/dL), 132 U/L (RR, 40-129 U/L), and 17.8 μg/L (RR, 7-22.4 μg/L), respectively. Skull radiography revealed sclerosis/hyperostosis, lytic lesions, and expansion of bone, consistent with Paget disease of bone (PDB). Five months after the initial presentation, her headache resolved, and her calcium and alkaline phosphatase levels were 9.7 U/L and 96 U/L, respectively.

Discussion

Denosumab neutralizes the receptor activator of nuclear factor-kappa B ligand. To date, there have been 2 case reports reported in the English literature of denosumab used successfully in patients with PDB who could not tolerate or were not eligible for bisphosphonates. This case report describes a patient with PDB treated with denosumab for osteopenia who experienced improvement in PDB-related symptoms.

Conclusion

Although denosumab was originally approved for the treatment of osteoporosis, the inhibition of bone resorption via inhibition of the receptor activator of nuclear factor-kappa B ligand may be potentially effective in the treatment of PDB.

背景/目的：Denosumab是一种抑制骨吸收的单克隆抗体，适用于治疗骨质疏松、骨转移和骨巨细胞瘤。我们描述了一名患有症状性颅骨Paget病的女性，其头痛和颅骨单发性疾病在接受替诺沙单抗治疗伴发的低骨密度后有所改善。病例报告：一位75岁的女性，持续头痛1个月。她有风湿病、骨质减少、甲状腺功能减退和胃食管反流病病史。据报道，她每天服用泼尼松1至20 mg治疗风湿性多肌痛，持续1年，并在出现症状前1个月服用剂量为60 mg的替诺沙单抗治疗骨质减少症。钙、碱性磷酸酶和骨特异性碱性磷酸酶水平分别为8.2 mg/dL（参考范围[RR]，8.5-10.5 mg/dL）、132 U/L（RR，40-129 U/L）和17.8μg/L（RR，7-22.4μg/L）。颅骨X线片显示骨硬化/骨质增生、溶解性病变和骨膨胀，与骨Paget病（PDB）一致。初次就诊五个月后，她的头痛症状缓解，钙和碱性磷酸酶水平分别为9.7U/L和96U/L。讨论：Denosumab中和核因子κB配体的受体激活剂。到目前为止，英国文献中已经报道了2例狄诺沙单抗成功用于不能耐受或不符合双磷酸盐条件的PDB患者的病例报告。本病例报告描述了一名PDB患者，该患者在PDB相关症状方面有所改善。结论：尽管狄诺沙单抗最初被批准用于治疗骨质疏松症，但通过抑制核因子κB配体的受体激活剂来抑制骨吸收可能对PDB的治疗具有潜在的有效性。

{"title":"Amelioration of Paget Disease of Bone After Denosumab for Osteopenia","authors":"Vijayvardhan Kamalumpundi BS , Elham Shams MD , Maisoon Torfah MD , Marcelo L. Correia MD, MSc, PhD","doi":"10.1016/j.aace.2023.05.007","DOIUrl":"10.1016/j.aace.2023.05.007","url":null,"abstract":"<div><h3>Background/Objective</h3><p>Denosumab is a monoclonal antibody that inhibits bone resorption and is indicated for the treatment of osteoporosis, bone metastases, and giant cell tumor of bone. We describe a woman with symptomatic Paget disease of the skull whose headaches and monostotic disease of the skull improved after receiving denosumab for concomitant low bone density.</p></div><div><h3>Case Report</h3><p>A 75-year-old woman presented with unremitting headache of 1 month. She had a medical history of polymyalgia rheumatica, osteopenia, hypothyroidism, and gastroesophageal reflux disease. She reported taking prednisone 1 to 20 mg daily for polymyalgia rheumatica for 1 year and received a dose of denosumab 60 mg for osteopenia 1 month before presentation. The calcium, alkaline phosphatase, and bone-specific alkaline phosphatase levels were 8.2 mg/dL (reference range [RR], 8.5-10.5 mg/dL), 132 U/L (RR, 40-129 U/L), and 17.8 μg/L (RR, 7-22.4 μg/L), respectively. Skull radiography revealed sclerosis/hyperostosis, lytic lesions, and expansion of bone, consistent with Paget disease of bone (PDB). Five months after the initial presentation, her headache resolved, and her calcium and alkaline phosphatase levels were 9.7 U/L and 96 U/L, respectively.</p></div><div><h3>Discussion</h3><p>Denosumab neutralizes the receptor activator of nuclear factor-kappa B ligand. To date, there have been 2 case reports reported in the English literature of denosumab used successfully in patients with PDB who could not tolerate or were not eligible for bisphosphonates. This case report describes a patient with PDB treated with denosumab for osteopenia who experienced improvement in PDB-related symptoms.</p></div><div><h3>Conclusion</h3><p>Although denosumab was originally approved for the treatment of osteoporosis, the inhibition of bone resorption via inhibition of the receptor activator of nuclear factor-kappa B ligand may be potentially effective in the treatment of PDB.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 5","pages":"Pages 158-161"},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10509370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41104343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Autoimmune Hypoglycemia With Anti-Insulin Autoantibodies in an Eighty-One-Year-Old Woman Without Apparent Risk Factors 无明显危险因素的81岁女性抗胰岛素自身抗体自身免疫性低血糖。

Q3 Medicine

AACE Clinical Case Reports

Pub Date : 2023-09-01 DOI: 10.1016/j.aace.2023.04.012

Vikash Kumar MD , Dhir Gala BS , Ibnul Rafi BS , Mili Shah BS , Sabrin Marowa MD , Diksha Kaul MD , Simon Lukose MD , Saka Kazeem MD

Background/Objective

Insulin autoimmune syndrome (IAS) is a very rare cause of hypoglycemia presenting with recurrent fasting or postprandial hypoglycemia episodes with elevated serum insulin levels and insulin autoantibodies. The objective of this case is to highlight the importance of considering IAS in patients with hypoglycemia.

Case Report

We present a case of an 81-year-old female who presented with symptoms of hypoglycemia. She was found to have hyperinsulinemic hypoglycemic episodes without any apparent risk factors for IAS. She had positive–insulin autoantibodies in her serum leading to the diagnosis of IAS. Acutely, hypoglycemia was managed with D50 pushes, oral glucose, and glucagon injection.

Discussion

Patients who present with hypoglycemia due to endogenous hyperinsulinemia should have IAS considered as a possible differential diagnosis. Insulin autoantibodies are measured as the gold standard diagnostic test for IAS. Foods with a low glycemic index are the primary treatment for IAS.

Conclusion

This case presentation highlights the importance of considering IAS as a differential diagnosis in patients presenting with hypoglycemia secondary to hyperinsulinemia, even in the absence of apparent risk factors.

背景/目的：胰岛素自身免疫综合征（IAS）是一种非常罕见的低血糖病因，表现为反复出现的空腹或餐后低血糖发作，伴有血清胰岛素水平升高和胰岛素自身抗体。本病例的目的是强调在低血糖患者中考虑IAS的重要性。病例报告：我们报告了一例81岁的女性，她出现低血糖症状。她被发现有高胰岛素血症低血糖发作，没有任何明显的IAS危险因素。她的血清中有阳性的胰岛素自身抗体，从而被诊断为IAS。急性低血糖症采用D50推压、口服葡萄糖和胰高血糖素注射治疗。讨论：内源性高胰岛素血症引起低血糖的患者应将IAS作为可能的鉴别诊断。胰岛素自身抗体作为IAS的金标准诊断测试进行测量。低血糖指数的食物是IAS的主要治疗方法。结论：本病例报告强调了将IAS作为高胰岛素血症继发低血糖患者的鉴别诊断的重要性，即使在没有明显危险因素的情况下也是如此。

{"title":"Autoimmune Hypoglycemia With Anti-Insulin Autoantibodies in an Eighty-One-Year-Old Woman Without Apparent Risk Factors","authors":"Vikash Kumar MD , Dhir Gala BS , Ibnul Rafi BS , Mili Shah BS , Sabrin Marowa MD , Diksha Kaul MD , Simon Lukose MD , Saka Kazeem MD","doi":"10.1016/j.aace.2023.04.012","DOIUrl":"10.1016/j.aace.2023.04.012","url":null,"abstract":"<div><h3>Background/Objective</h3><p>Insulin autoimmune syndrome (IAS) is a very rare cause of hypoglycemia presenting with recurrent fasting or postprandial hypoglycemia episodes with elevated serum insulin levels and insulin autoantibodies. The objective of this case is to highlight the importance of considering IAS in patients with hypoglycemia.</p></div><div><h3>Case Report</h3><p>We present a case of an 81-year-old female who presented with symptoms of hypoglycemia. She was found to have hyperinsulinemic hypoglycemic episodes without any apparent risk factors for IAS. She had positive–insulin autoantibodies in her serum leading to the diagnosis of IAS. Acutely, hypoglycemia was managed with D50 pushes, oral glucose, and glucagon injection.</p></div><div><h3>Discussion</h3><p>Patients who present with hypoglycemia due to endogenous hyperinsulinemia should have IAS considered as a possible differential diagnosis. Insulin autoantibodies are measured as the gold standard diagnostic test for IAS. Foods with a low glycemic index are the primary treatment for IAS.</p></div><div><h3>Conclusion</h3><p>This case presentation highlights the importance of considering IAS as a differential diagnosis in patients presenting with hypoglycemia secondary to hyperinsulinemia, even in the absence of apparent risk factors.</p></div>","PeriodicalId":7051,"journal":{"name":"AACE Clinical Case Reports","volume":"9 5","pages":"Pages 146-148"},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/91/1b/main.PMC10509373.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41106971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1