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https://www.readbyqxmd.com/read/29455549/multiple-endocrine-neoplasia-2a-men-2a-syndrome
#1
J Breza, J Breza
INTRODUCTION: In the MEN 2A syndrome, which is the most common of the three types of MEN, three endocrine systems are affected simultaneously or subsequently by the development of tumours manifested by medullary thyroid gland carcinoma, pheochromocytoma (often bilateral) and hyperparathyroidism. MATERIAL AND METHODS: 27 patients from 3 families affected by MEN 2A syndrome were examined clinically (by detecting the effects of catecholamine overproduction), biochemically (screening for metanephrine and normetanephrine in the serum), visualization (CT, MRI, MIBG, PET CT) and some of them also genetically (DNA fragment analysis obtained by PCR amplification)...
2018: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/29453922/a-short-review-of-primary-aldosteronism-in-a-question-and-answer-fashion
#2
Frederick-Anthony Farrugia, Nicolaos Zavras, Georgios Martikos, Panagiotis Tzanetis, Anestis Charalampopoulos, Evangelos P Misiakos, Dimitrios Sotiropoulos, Nikolaos Koliakos
OBJECTIVES: The aim of this study was to present up to date information concerning the diagnosis and treatment of primary aldosteronism (PA). PA is the most common cause of endocrine hypertension. It has been reported up to 24% of selective referred hypertensive patients. METHODS: We did a search in Pub-Med and Google Scholar using the terms: PA, hyperaldosteronism, idiopathic adrenal hyperplasia, diagnosis of PA, mineralocorticoid receptor antagonists, adrenalectomy, and surgery...
January 1, 2018: Endocrine Regulations
https://www.readbyqxmd.com/read/29448302/retroperitoneal-laparoscopic-adrenalectomy-with-transient-renal-artery-occlusion-for-large-adrenal-tumors-%C3%A2-8-cm
#3
Binbin Gong, Ming Ma, Wenjie Xie, Xiaorong Yang, Ting Sun
OBJECTIVES: To analyze our experience in retroperitoneal laparoscopic adrenalectomy (RLA) with transient renal artery occlusion for large adrenal tumors (≥8 cm) and to explore the safety and feasibility of this surgical procedure. METHODS: A retrospective cohort study was conducted with a surgical data review of 18 patients with large adrenal tumors who underwent RLA with transient renal artery occlusion in our hospital. RESULTS: Eighteen patients were treated by RLA with transient occlusion of the renal artery, and none were converted to open adrenalectomy...
February 15, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29440130/cardiac-magnetic-resonance-imaging-of-myocardial-mass-and-fibrosis-in-primary-aldosteronism
#4
Marianne Aardal Grytaas, Kjersti Sellevåg, Hrafnkell Baldur Thordarson, Eystein Sverre Husebye, Kristian Løvås, Terje Hjalmar Larsen
BACKGROUND: Primary aldosteronism (PA) is associated with increased cardiovascular morbidity, presumably due to left ventricular (LV) hypertrophy and fibrosis. However, the degree of fibrosis has not been extensively studied. Cardiac magnetic resonance imaging (CMR) contrast enhancement and novel sensitive T1 mapping to estimate increased extracellular volume (ECV) are available to measure the extent of fibrosis. OBJECTIVES: To assess LV mass and fibrosis before and after treatment of PA using CMR with contrast enhancement and T1 mapping...
February 12, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29426273/testosterone-and-cortisol-secreting-oncocytic-adrenocortical-adenoma-in-the-pediatric-age-group
#5
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29412551/laparoscopic-approach-to-pheochromocytoma-in-pregnancy-case-report
#6
Felipe de Almeida E Paula, Ravisio Israel Dos Santos, Odivaldo Antonio Ferruzzi, Rafael Osti Melo, Mariana Takaku
A 32-year-old 22-week pregnant hypertensive woman with sporadic episodes of headaches, sweating, and facial flushing was diagnosed with pheochromocytoma through biochemical and imaging tests. Perioperative management included a multidisciplinary approach, symptom stabilization with ɑ blockade followed by β blockade, and tumor resection by laparoscopic adrenalectomy at 24 weeks gestation. The diagnosis was confirmed by histopathological examination and immunohistochemistry tests. The decision for surgical removal of the tumor was based on maternal symptoms, tumor size, gestational age, the possibility of doing a laparoscopy, and the expertise of the surgical team...
February 8, 2018: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29409357/macrolides-for-kcnj5-mutated-aldosterone-producing-adenoma-mapa-design-of-a-study-for-personalized-diagnosis-of-primary-aldosteronism
#7
Giuseppe Maiolino, Giulio Ceolotto, Michele Battistel, Giulio Barbiero, Maurizio Cesari, Laurence Amar, Brasilina Caroccia, Roberto Padrini, Michel Azizi, Gian Paolo Rossi
PURPOSE: Aldosterone-producing adenoma (APA) is the main curable cause of endocrine hypertension cause of primary aldosteronism (PA) and it is in up to 66% of all cases investigated with adrenal vein sampling (AVS). Mutations in the KCNJ5 potassium channel involve up to 70% of APA and cause the most florid PA phenotypes. The recent finding that macrolide antibiotics specifically inhibit in vitro the altered function of mutated KCNJ5 channels has opened new horizons for the diagnosis and treatment of APA with KCNJ5 mutations in that it can allow identification and target treatment of PA patients harbouring a mutated APA...
February 6, 2018: Blood Pressure
https://www.readbyqxmd.com/read/29408964/a-homozygous-ret-k666n-genotype-with-an-men2a-phenotype
#8
Tania Jaber, Samuel M Hyde, Gilbert J Cote, Elizabeth G Grubbs, Wesley H Giles, Cathy A Stevens, Ramona Dadu
Context: Germline RET K666N mutation has been described as a pathogenic mutation with low disease penetrance for medullary thyroid cancer (MTC) without other features of MEN2A. We describe a patient with homozygous RET K666N mutation with MTC and bilateral pheochromocytoma (PHEO). Case Description: A 59-year-old female was diagnosed with MTC after biopsy of two thyroid nodules. Coincident biochemical and radiologic testing was suspicious for bilateral PHEO, confirmed after bilateral adrenalectomy...
February 2, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29404245/pediatric-pituitary-adenoma-case-series-review-of-the-literature-and-a-skull-base-treatment-paradigm
#9
REVIEW
Avital Perry, Christopher Salvatore Graffeo, Christopher Marcellino, Bruce E Pollock, Nicholas M Wetjen, Fredric B Meyer
Background  Pediatric pituitary adenoma is a rare skull base neoplasm, accounting for 3% of all intracranial neoplasms in children and 5% of pituitary adenomas. Compared with pituitary tumors in adults, secreting tumors predominate and longer disease trajectories are expected due to the patient age resulting in a natural history and treatment paradigm that is complex and controversial. Objectives  The aims of this study were to describe a large, single-institution series of pediatric pituitary adenomas with extensive long-term follow-up and to conduct a systematic review examining outcomes after pituitary adenoma surgery in the pediatric population...
February 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29396817/renal-medullary-carcinoma-a-nearly-fatal-malignancy-specifically-affecting-patients-with-a-so-called-benign-condition
#10
Yamila Goenaga-Vázquez, Gloria Colón, Nilka Barrios, María Correa
Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found almost exclusively in young patients with sickle cell trait (SCT). Metastatic disease is commonly present at diagnosis. There is very limited experience treating disseminated disease and the prognosis is dismal. We report the case of a young 9-year-old boy with SCT, who presented with 4 months' progression of abdominal pain, nausea and vomiting associated with cough spells, dysphagia, and weight loss. Upon evaluation, he was underweight, pale, and in mild respiratory distress...
February 2, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29396110/operative-technique-transperitoneal-robotic-adrenalectomy
#11
C Nomine-Criqui, S Moog, L Bresler, L Brunaud
No abstract text is available yet for this article.
January 27, 2018: Journal of Visceral Surgery
https://www.readbyqxmd.com/read/29395236/randomized-trial-of-low-versus-high-carbon-dioxide-insufflation-pressures-in-posterior-retroperitoneoscopic-adrenalectomy
#12
Sheila Fraser, Olov Norlén, Kyle Bender, Joanne Davidson, Sonya Bajenov, David Fahey, Shawn Li, Stan Sidhu, Mark Sywak
BACKGROUND: Posterior retroperitoneoscopic adrenalectomy has gained widespread acceptance for the removal of benign adrenal tumors. Higher insufflation pressures using carbon dioxide (CO2) are required, although the ideal starting pressure is unclear. This prospective, randomized, single-blinded, study aims to compare physiologic differences with 2 different CO2 insufflation pressures during posterior retroperitoneoscopic adrenalectomy. METHODS: Participants were randomly assigned to a starting insufflation pressure of 20 mm Hg (low pressure) or 25 mm Hg (high pressure)...
January 30, 2018: Surgery
https://www.readbyqxmd.com/read/29394778/-a-case-of-metachronous-resection-for-advanced-esophageal-cancer-with-pheochromocytoma
#13
Kohei Murakami, Atsushi Takeno, Toru Masuzawa, Shigeyuki Tamura, Kenji Kawai, Takuya Sakamoto, Junichi Inatome, Atsushi Naito, Yoshiteru Katsura, Yoshiaki Ohmura, Yoshinori Kagawa, Chiyomi Egawa, Yutaka Takeda, Kohei Murata
A 66-year-old man reporting chest stenosis was examined. Upper gastrointestinal endoscopy showed type 2 esophageal cancer. An enhanced CT scan showed several swollen lymph nodes in the lesser curvature of the esophagus. We diagnosed the patient as having advanced esophageal cancer with multiple lymph node metastases(cT3N2M0, Stage III ). After 2 courses of chemotherapy(DCF), a significant reduction was observed in the esophageal tumor; however, the tumor at the lesser curvature was almost unchanged. MIBG scintigraphy showed high uptake in the left adrenal gland...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29394662/-esophagogastric-junction-cancer-resection-with-bilateral-adrenal-metastasis-a-case-report
#14
Sho Toyoda, Atsuo Imagawa, Kouichi Demura, Kurumi Tsuchihashi, Satoshi Okumura, Hiroshi Yasuda, Kansuke Yamamoto, Aya Ito, Naoto Mizumura, Ken Yuu, Hiroshi Tsuchihashi, Masao Ogawa, Masayasu Kawasaki, Masao Kameyama
The patient was a 64-year-old man with esophagogastric junction cancer. We performed right thoracotomy-laparotomy for lower esophageal and cardiac gastric resection, D2 lymphadenectomy, and reconstruction of a gastric tube in October 2011. Histopathology confirmed T4aN1M1(LYM), Stage IV cancer(Japanese Classification of Gastric Carcinoma, 14th edition) with R0 resection. Because of preexisting alcoholic cirrhosis, postoperative chemotherapy was not an option. In March 2014, we performed left adrenalectomy for left adrenal metastasis, and in December 2014, we performed right adrenalectomy for metastasis to the right adrenal gland...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29394637/-a-case-of-recurrent-rectal-cancer-with-adrenal-and-lung-metastasis-with-more-than-ten-year-survival-after-surgery
#15
Kiyoshi Uchiyama, Yu Katarao, Yu Kimura, Ryoichi Tanaka, Takashi Fuse, Manabu Takemura, Yoshiki Yamamoto, Yoshihiro Shimizu
A 60-year-old man underwent low anterior resection for rectal cancer. Histological findings indicated well-differentiated adenocarcinoma(T3[SS]N1M0, ly3, v2, Stage III a). Two years and 1 month later, right adrenalectomy was performed for solitary adrenal metastasis. Three months thereafter, left partial pulmonary resection was performed for a metastatic lung tumor. All resected specimens showed metastatic adenocarcinoma derived from the rectal cancer. The patient is alive and well without recurrence for more than 10years after lung resection...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29394636/-an-enhanced-multimodality-treatment-strategy-in-pancreatic-ductal-carcinoma-requiring-heminephrectomy-for-radical-resection-a-case-report
#16
Akihiro Kitagawa, Yoshifumi Iwagami, Hidetoshi Eguchi, Daisaku Yamada, Tadafumi Asaoka, Takehiro Noda, Hiroshi Wada, Koichi Kawamoto, Kunihito Gotoh, Shogo Kobayashi, Masaki Mori, Yuichiro Doki
A 76-year-old male visited a clinic and the images incidentally revealed a tumor in the tail of pancreas. Pancreatic ductal adenocarcinoma was diagnosed by the following examination, indicating that the tumor invasion to spleen, left adrenal gland and left kidney(cT3N0M0, cStage II A), requiring heminephrectomy for radical resection. Enhanced preoperative treatments were performed while taking into account a possibility of any restrictions for the age and the standard adjuvant chemotherapies after heminephrectomy...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29392429/minimally-invasive-surgery-mis-in-children-and-adolescents-with-pheochromocytomas-and-retroperitoneal-paragangliomas-experiences-in-42-patients
#17
Martin K Walz, Laura D Iova, Judith Deimel, Hartmut P H Neumann, Birke Bausch, Stefan Zschiedrich, Harald Groeben, Pier F Alesina
BACKGROUND: Pheochromocytomas (PH) and paragangliomas (PGL) are rare tumours in children accounting for about 1% of the paediatric hypertension. While minimally invasive surgical techniques are well established in adult patients with PH, the experience in children is extremely limited. To the best of our knowledge, we herewith present the largest series of young patients operated on chromaffin tumours by minimally invasive access. MATERIALS: In the setting of a prospective study (1/2001-12/2016), 42 consecutive children and adolescents (33 m, 9 f) were operated on...
February 1, 2018: World Journal of Surgery
https://www.readbyqxmd.com/read/29390591/robot-assisted-laparoendoscopic-single-site-adrenalectomy-a-comparison-of-3-different-port-platforms-with-3-case-reports
#18
Hung-Cheng Kan, See-Tong Pang, Chun-Te Wu, Ying-Hsu Chang, Chung-Yi Liu, Cheng-Keng Chuang, Po-Hung Lin
RATIONALE: Laparoscopic adrenalectomy is currently the standard of care for adrenal lesion. Minimal invasive laparoscopic surgery such as laparoendoscopic single site surgery (LESS) and natural orifice transluminal endoscopic surgery (NOTES) have been developed to improve cosmetic outcomes and reduce postoperative pain. However, there are still some problems related to instruments and port limitation during LESS surgery. Robot-assisted laparoscopic surgery may help to overcome these problems, and port platforms selection is an important issue...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29390437/chronic-primary-adrenal-insufficiency-after-unilateral-adrenonephrectomy-a-case-report
#19
Satoshi Yoshiji, Kimitaka Shibue, Toshihito Fujii, Takeshi Usui, Keisho Hirota, Daisuke Taura, Mayumi Inoue, Masakatsu Sone, Akihiro Yasoda, Nobuya Inagaki
RATIONALE: Unilateral adrenalectomy as part of surgical resection of renal cell carcinoma (RCC) is not thought to increase the risk of chronic adrenal insufficiency, as the contralateral adrenal gland is assumed to be capable of compensating for the lost function of the resected gland. However, recent studies have indicated that adrenalectomy might cause irreversible impairment of the adrenocortical reserve. We describe a case of chronic primary adrenal insufficiency in a 68-year-old man who previously underwent unilateral adrenonephrectomy, which was complicated by severe postoperative adrenal stress that involved cardiopulmonary disturbance and systemic infection...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29390296/carney-complex-with-prkar1a-gene-mutation-a-case-report-and-literature-review
#20
Qiuli Liu, Dali Tong, Gaolei Liu, Yuting Yi, Dianzheng Zhang, Jun Zhang, Yao Zhang, Zaoming Huang, Yaoming Li, Rongrong Chen, Yanfang Guan, Xin Yi, Jun Jiang
RATIONALE: Carney complex (CNC) is a multiple neoplasia syndrome with autosomal dominant inheritance. CNC is characterized by the presence of myxomas, spotty skin pigmentation, and endocrine overactivity. No direct correlation has been established between disease-causing mutations and phenotype. PATIENT CONCERNS: A 16-year-old boy was admitted because of excessive weight gain over 3 years and purple striae for 1 year. Physical examination revealed Cushingoid features and spotty skin pigmentation on his face, lip, and sclera...
December 2017: Medicine (Baltimore)
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