[Head and neck paragangliomas: An interdisciplinary challenge].

PubMed

Künzel, J; Bahr, K; Hainz, M; Rossmann, H; Matthias, C

2015-12-01

Current treatment strategies for head and neck paragangliomas are moving away from radical resection and toward surgical tumor reduction, in order to preserve function and reduce morbidity. Radiotherapy modalities are alternative primary treatment options. A PubMed search of the relevant literature on genetics and treatment of head and neck paragangliomas was conducted. The rapid progress made in genetic research was mainly triggered by two factors: firstly, the establishment of central registries for paraganglioma patients and secondly, the availability of next-generation sequencing methods. Exome sequencing and a gene-panel sequencing approach have already been successfully applied to paraganglioma syndromes. The latter method in particular is rapid and cost-effective, and may soon replace complex genotyping algorithms. The literature provides good evidence that diversified modern treatment options are available to realize individual treatment concepts for almost all paraganglioma manifestations. Generally, small and symptomatic tumors should be completely resected, particularly in younger patients. Considering the patient's age, symptoms, morbidity risk, and comorbidities, larger tumors should be surgically treated in a function-preserving manner. In these cases, primary radiotherapy is an equivalent alternative option. A "wait and scan" strategy is possible in selected cases. The potential morbidity of surgical treatment must be weighed against the expectable quality of life. Comprehensive consultation with the patient about possible treatment modalities is mandatory. Treatment decision making should involve a multidisciplinary team of experts.

68Ga-DOTA-TATE PET/CT for Molecular Imaging of Somatostatin Receptor Expression in Extra-adrenal Paraganglioma in a Case of Complete Carney Triad.

PubMed

Derlin, Thorsten; Hartung, Dagmar; Hueper, Katja

2017-12-01

Carney triad is a very rare syndrome characterized by the synchronous or metachronous occurrence of gastrointestinal stromal tumors, pulmonary chondroma, and extra-adrenal paraganglioma. We present the case of a 36-year-old woman with complete Carney triad who underwent a Ga-DOTA-TATE PET/CT scan for restaging of metastasizing extra-adrenal paraganglioma and for evaluation of targeted radionuclide therapy potential. On the Ga-DOTA-TATE PET scan, increased tracer accumulation was observed in paraganglioma metastases. This case highlights the usefulness of Ga-DOTA-TATE PET/CT for restaging of metastasizing paraganglioma in Carney triad and the option of targeted radionuclide therapy in this entity.

A patient with a painless neck tumour revealed as a carotid paraganglioma: a case report.

PubMed

Peric, Barbara; Marinsek, Ziva Pohar; Skrbinc, Breda; Music, Maja; Zagar, Ivana; Hocevar, Marko

2014-08-20

Carotid paragangliomas are usually slowly enlarging and painless lateral neck masses. These mostly benign lesions are recognized due to their typical location, vessel displacement and specific blood supply, features that are usually seen on different imaging modalities. Surgery for carotid paraganglioma can be associated with immediate cerebrovascular complications or delayed neurological impairment.We are reporting the case of a 36-year-old man who presented with a painless mass on the right side of his neck 11 months after being treated for testicular cancer. After a fine-needle aspiration biopsy, he was diagnosed with a testicular cancer lymph node metastasis. Neck US and fluorine [F-18]-fluorodeoxy-D-glucose (FDG) PET-CT showed no signs of hypervascularity or vessel displacement. The patient underwent a level II to V functional neck dissection. During the procedure, suspicion of a carotid paraganglioma was raised and the tumour was carefully dissected from the walls of the carotid arteries with minimal blood loss and no cranial nerve dysfunction.The histology report revealed carotid paraganglioma with no metastasis in the rest of the lymph nodes. The patient's history of testicular germ cell tumour led to a functional neck dissection during which a previously unrecognized carotid paraganglioma was removed.Surgery for carotid PG can be associated with complications that have major impact on quality of life. A thorough assessment of the patient and neck mass must therefore be performed preoperatively in order to perform the surgical procedure under optimal conditions.

Molecular markers of paragangliomas/pheochromocytomas

PubMed Central

Zaretsky, Andrew R; Alekseev, Boris Y; Pokrovsky, Anatoly V; Golovyuk, Alexander L; Melnikova, Nataliya V; Stepanov, Oleg A; Kalinin, Dmitry V; Moskalev, Alexey A; Krasnov, George S; Dmitriev, Alexey A; Kudryavtseva, Anna V

2017-01-01

Paragangliomas/pheochromocytomas comprise rare tumors that arise from the extra-adrenal paraganglia, with an incidence of about 2 to 8 per million people each year. Approximately 40% of cases are due to genetic mutations in at least one out of more than 30 causative genes. About 2530% of pheochromocytomas/paragangliomas develop under the conditions of a hereditary tumor syndrome a third of which are caused by mutations in the VHL gene. Together, the gene mutations in this disorder have implicated multiple processes including signaling pathways, translation initiation, hypoxia regulation, protein synthesis, differentiation, survival, proliferation, and cell growth. The present review contemplates the mutations associated with the development of pheochromocytomas/paragangliomas and their potential to serve as specific markers of these tumors and their progression. These data will improve our understanding of the pathogenesis of these tumors and likely reveal certain features that may be useful for early diagnostics, malignancy prognostics, and the determination of new targets for disease therapeutics. PMID:28187001

[Periampullary gangliocytic paraganglioma].

PubMed

Sankot, J; Svarcová, I

2008-01-01

We present the case of very rare periampullary gangliocytic paraganglioma in which we preferred proximal duodenopancreatectomy to local resection of the ampula of Vater because of not clear preoperative pathohistology and biologic behavior of the tumor. Although the definitive examination of the specimen proved its benign character we consider our procedure reasonable.

Treatment of Head and Neck Paragangliomas With External Beam Radiation Therapy

DOE Office of Scientific and Technical Information (OSTI.GOV)

Dupin, Charles, E-mail: c.dupin@bordeaux.unicancer.fr; Lang, Philippe; Dessard-Diana, Bernadette

2014-06-01

Purpose: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. Methods and Materials: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm{sup 3} (range, 0.9-243 cm{sup 3}) and 116 cm{sup 3} (range, 24-731 cm{sup 3}), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories ofmore » paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). Results: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. Conclusion: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas.« less

Devascularization of Head and Neck Paragangliomas by Direct Percutaneous Embolization

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ozyer, Umut, E-mail: umut_ozyer@yahoo.com; Harman, Ali; Yildirim, Erkan

2010-10-15

Preoperative transarterial embolization of head and neck paragangliomas using particulate agents has proven beneficial for decreasing intraoperative blood loss. However, the procedure is often incomplete owing to extensive vascular structure and arteriovenous shunts. We report our experience with embolization of these lesions by means of direct puncture and intratumoral injection of n-butyl cyanoacrylate (NBCA) or Onyx. Ten patients aged 32-82 years who were referred for preoperative embolization of seven carotid body tumors and three jugular paragangliomas were retrospectively analyzed. Intratumoral injections were primarily performed in four cases with multiple small-caliber arterial feeders and adjunctive to transarterial embolization in six casesmore » with incomplete devascularization. Punctures were performed under ultrasound and injections were performed under roadmap fluoroscopic guidance. Detailed angiographies were performed before and after embolization procedures. Control angiograms showed complete or near-complete devascularization in all tumors. Three tumors with multiple small-caliber arterial feeders were treated with primary NBCA injections. One tumor necessitated transarterial embolization after primary injection of Onyx. Six tumors showed regional vascularization from the vasa vasorum or small-caliber branches of the external carotid artery following the transarterial approach. These regions were embolized with NBCA injections. No technical or clinical complications related to embolization procedures occurred. All except one of the tumors were surgically removed following embolization. In conclusion, preoperative devascularization with percutaneous direct injection of NBCA or Onyx is feasible, safe, and effective in head and neck paragangliomas with multiple small-caliber arterial feeders and in cases of incomplete devascularization following transarterial embolization.« less

Paragangliomas arise through an autonomous vasculo-angio-neurogenic program inhibited by imatinib.

PubMed

Verginelli, Fabio; Perconti, Silvia; Vespa, Simone; Schiavi, Francesca; Prasad, Sampath Chandra; Lanuti, Paola; Cama, Alessandro; Tramontana, Lorenzo; Esposito, Diana Liberata; Guarnieri, Simone; Sheu, Artenca; Pantalone, Mattia Russel; Florio, Rosalba; Morgano, Annalisa; Rossi, Cosmo; Bologna, Giuseppina; Marchisio, Marco; D'Argenio, Andrea; Taschin, Elisa; Visone, Rosa; Opocher, Giuseppe; Veronese, Angelo; Paties, Carlo T; Rajasekhar, Vinagolu K; Söderberg-Nauclér, Cecilia; Sanna, Mario; Lotti, Lavinia Vittoria; Mariani-Costantini, Renato

2018-05-01

Tumours can be viewed as aberrant tissues or organs sustained by tumorigenic stem-like cells that engage into dysregulated histo/organogenetic processes. Paragangliomas, prototypical organoid tumours constituted by dysmorphic variants of the vascular and neural tissues found in normal paraganglia, provide a model to test this hypothesis. To understand the origin of paragangliomas, we built a biobank comprising 77 cases, 18 primary cultures, 4 derived cell lines, 80 patient-derived xenografts and 11 cell-derived xenografts. We comparatively investigated these unique complementary materials using morphofunctional, ultrastructural and flow cytometric assays accompanied by microRNA studies. We found that paragangliomas contain stem-like cells with hybrid mesenchymal/vasculoneural phenotype, stabilized and expanded in the derived cultures. The viability and growth of such cultures depended on the downregulation of the miR-200 and miR-34 families, which allowed high PDGFRA and ZEB1 protein expression levels. Both tumour tissue- and cell culture-derived xenografts recapitulated the vasculoneural paraganglioma structure and arose from mesenchymal-like cells through a fixed developmental sequence. First, vasculoangiogenesis organized the microenvironment, building a perivascular niche which in turn supported neurogenesis. Neuroepithelial differentiation was associated with severe mitochondrial dysfunction, not present in cultured paraganglioma cells, but acquired in vivo during xenograft formation. Vasculogenesis was the Achilles' heel of xenograft development. In fact, imatinib, that targets endothelial-mural signalling, blocked paraganglioma xenograft formation (11 xenografts from 12 cell transplants in the control group versus 2 out of 10 in the treated group, P = 0.0015). Overall our key results were unaffected by the SDHx gene carrier status of the patient, characterized for 70 out of 77 cases. In conclusion, we explain the biphasic vasculoneural structure of

Temporal bone paragangliomas: 15 years experience.

PubMed

Düzlü, Mehmet; Tutar, Hakan; Karamert, Recep; Karaloğlu, Furkan; Şahin, Muammer Melih; Göcek, Mehmet; Uğur, Mehmet Birol; Göksu, Nebil

2016-12-08

Temporal bone paragangliomas (TBPs) are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. In this paper we aim to present our clinical experience with TBPs and to review literature data. The patients who were operated for tympanomastoid paraganglioma (TMP) or tympanojugular paraganglioma (TJP) in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. There were 18 (52.9%) cases with TMPs and 16 (47.1%) cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3± 11.7 (range 25-71 years). The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4%) and 10 (62.5%) cases for TMPs and TJPs, respectively. Five patients (31.2%) with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months). In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Clinical and Molecular Features of Renal and Pheochromocytoma/Paraganglioma Tumor Association Syndrome (RAPTAS): Case Series and Literature Review.

PubMed

Casey, Ruth T; Warren, Anne Y; Martin, Jose Ezequiel; Challis, Benjamin G; Rattenberry, Eleanor; Whitworth, James; Andrews, Katrina A; Roberts, Thomas; Clark, Graeme R; West, Hannah; Smith, Philip S; Docquier, France M; Rodger, Fay; Murray, Vicki; Simpson, Helen L; Wallis, Yvonne; Giger, Olivier; Tran, Maxine; Tomkins, Susan; Stewart, Grant D; Park, Soo-Mi; Woodward, Emma R; Maher, Eamonn R

2017-11-01

The co-occurrence of pheochromocytoma (PC) and renal tumors was linked to the inherited familial cancer syndrome von Hippel-Lindau (VHL) disease more than six decades ago. Subsequently, other shared genetic causes of predisposition to renal tumors and to PC, paraganglioma (PGL), or head and neck paraganglioma (HNPGL) have been described, but case series of non-VHL-related cases of renal tumor and pheochromocytoma/paraganglioma tumor association syndrome (RAPTAS) are rare. To determine the clinical and molecular features of non-VHL RAPTAS by literature review and characterization of a case series. A review of the literature was performed and a retrospective study of referrals for investigation of genetic causes of RAPTAS. Literature review revealed evidence of an association, in addition to VHL disease, between germline mutations in SDHB, SDHC, SDHD, TMEM127, and MAX genes and RAPTAS [defined here as the co-occurrence of tumors from both classes (PC/PGL/HNPGL and renal tumors) in the same individual or in first-degree relatives]. In both the literature review and our case series of 22 probands with non-VHL RAPTAS, SDHB mutations were the most frequent cause of non-VHL RAPTAS. A genetic cause was identified in 36.3% (8/22) of kindreds. Renal tumors and PC/PGL/HNPGL tumors share common molecular features and their co-occurrence in an individual or family should prompt genetic investigations. We report a case of MAX-associated renal cell carcinoma and confirm the role of TMEM127 mutations with renal cell carcinoma predisposition. Copyright © 2017 Endocrine Society

[Paragangliomas of the head and neck. Our experience].

PubMed

Alvarez Zapico, M J; Llorente Pendás, J L; Suárez Nieto, C; Carreño Villarreal, M; Moro Melón, M

1998-04-01

Paragangliomas of the head and neck are rare tumors of neuroectodermal origin. However, jugulotympanic paragangliomas are the second most common tumor of the temporal bone. Paragangliomas are recognized as benign tumors, but some show a biological behavior similar to that of malignant lesions, such as the development of recurrence or invasive growth patterns. We report 31 patients with a total of 35 paragangliomas who were seen in our department. Multiple paragangliomas were found in 2 patients. Twenty-two patients had jugulotympanic paragangliomas, 11 carotid and 2 vagal paragangliomas. None of the patients had prior treatment and the minimum follow-up was 1 year. Surgery was performed in 28 (90%), primary irradiation in 1 (3%), and 2 (7%) refused treatment. We describe our results, the clinical course, diagnostic methods and treatment options.

Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up: a case report.

PubMed

Barret, Maximilien; Rahmi, Gabriel; Duong van Huyen, Jean-Paul; Landi, Bruno; Cellier, Christophe; Berger, Anne

2012-01-01

Gangliocytic paraganglioma (GP) is a rare tumor, usually located in the second part of the duodenum. On pathological examination, GP is characterized by the association of the histological features of paragangliomas, ganglioneuromas, and carcinoid tumors. Classical clinical presentations are upper gastrointestinal bleeding and abdominal pain. Preoperative diagnosis is difficult because of the submucosal site of the tumor, with usually negative mucosal biopsies. Endoscopic ultrasound helps establish the diagnosis and allows lymph node staging, which will guide the choice of the treatment. If GPs usually follow a benign course, metastatic spread to regional lymph nodes treated by surgical resection alone has been reported. We report a case of GP with lymph node metastases treated by duodenopancreatectomy with long-term disease-free survival, suggesting that surgical resection is a reasonable approach for metastatic GPs.

Management of sizeable carotid body tumor: Case report and review of literature.

PubMed

Elsharawy, Mohamed A; Alsaif, Hind; Elsaid, Aymen; Kredees, Ali

2013-10-01

Carotid body tumor is a paraganglioma derived from the neural crest. It arises from the carotid body which acts as a vascular chemoreceptors and is usually located at the carotid bifurcation. Sizeable (Shamblin III, >5 cm size) tumors are large and typically encase the carotid artery requiring vessel resection and replacement. Management of such tumors carries a high risk of postoperative mortality and morbidity rates specially with regards to neurovascular complications. We report a case of sizeable tumor which was surgically removed with minimal complications.

Management of sizeable carotid body tumor: Case report and review of literature

PubMed Central

Elsharawy, Mohamed A; Alsaif, Hind; Elsaid, Aymen; Kredees, Ali

2013-01-01

Carotid body tumor is a paraganglioma derived from the neural crest. It arises from the carotid body which acts as a vascular chemoreceptors and is usually located at the carotid bifurcation. Sizeable (Shamblin III, >5 cm size) tumors are large and typically encase the carotid artery requiring vessel resection and replacement. Management of such tumors carries a high risk of postoperative mortality and morbidity rates specially with regards to neurovascular complications. We report a case of sizeable tumor which was surgically removed with minimal complications. PMID:24327970

Tracheal paraganglioma: an unusual neoplasm of the upper airway.

PubMed

Metzdorff, Mark T; Seaman, Joseph C; Opperman, David A; Goates, Jeffrey J; Musani, Ali I

2012-05-01

Paraganglioma of the trachea is a rare neoplasm, with fewer than 15 cases reported. A 40-year-old man presented with stridor and hemoptysis. Bronchoscopy demonstrated a tumor of the posterior trachea and biopsy initially suggested typical carcinoid. The patient underwent surgical resection uneventfully and made a good recovery. Final pathology disclosed the tumor to be a paraganglioma based on immunohistology. The pathophysiology and treatment of this tumor are discussed. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Thyroid paraganglioma. Report of 3 cases and description of an immunohistochemical profile useful in the differential diagnosis with medullary thyroid carcinoma, based on complementary DNA array results.

PubMed

Castelblanco, Esmeralda; Gallel, Pilar; Ros, Susana; Gatius, Sonia; Valls, Joan; De-Cubas, Aguirre A; Maliszewska, Agnieszka; Yebra-Pimentel, M Teresa; Menarguez, Javier; Gamallo, Carlos; Opocher, Giuseppe; Robledo, Mercedes; Matias-Guiu, Xavier

2012-07-01

Thyroid paraganglioma is a rare disorder that sometimes poses problems in differential diagnosis with medullary thyroid carcinoma. So far, differential diagnosis is solved with the help of some markers that are frequently expressed in medullary thyroid carcinoma (thyroid transcription factor 1, calcitonin, and carcinoembryonic antigen). However, some of these markers are not absolutely specific of medullary thyroid carcinoma and may be expressed in other tumors. Here we report 3 new cases of thyroid paraganglioma and describe our strategy to design a diagnostic immunohistochemical battery. First, we performed a comparative analysis of the expression profile of head and neck paragangliomas and medullary thyroid carcinoma, obtained after complementary DNA array analysis of 2 series of fresh-frozen samples of paragangliomas and medullary thyroid carcinoma, respectively. Seven biomarkers showing differential expression were selected (nicotinamide adenine dinucleotide dehydrogenase 1 alpha subcomplex, 4-like 2, NDUFA4L2; cytochrome c oxidase subunit IV isoform 2; vesicular monoamine transporter 2; calcitonin gene-related protein/calcitonin; carcinoembryonic antigen; and thyroid transcription factor 1) for immunohistochemical analysis. Two tissue microarrays were constructed from 2 different series of paraffin-embedded samples of paragangliomas and medullary thyroid carcinoma. We provide a classifying rule for differential diagnosis that combines negativity or low staining for calcitonin gene-related protein (histologic score, <10) or calcitonin (histologic score, <50) together with positivity of any of NADH dehydrogenase 1 alpha subcomplex, 4-like 2; cytochrome c oxidase subunit IV isoform 2; or vesicular monoamine transporter 2 to predict paragangliomas, showing a prediction error of 0%. Finally, the immunohistochemical battery was checked in paraffin-embedded blocks from 4 examples of thyroid paraganglioma (1 previously reported case and 3 new cases), showing also a

[Pheochromocytoma and paraganglioma in childhood: a report of 2 cases report].

PubMed

Mosquera Gorostidi, A; Justo Ranera, A; Zakirian Denis, S E; González Temprano, N; Sagaseta de Ilúrdoz Uranga, M; Molina Garicano, J

2015-01-01

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors in children and most of them are sporadic. However, they represent the most common endocrine tumor in childhood, and hereditary tumor syndromes are most relevant in these age. Advances in genetic, biochemistry and imaging techniques have revised the management of these tumors; thus A biochemical study should be always initiated once the clinical diagnosis is suspected, followed by imaging and molecular studies, particularly in the context of known familial disease. The diagnostic and therapeutic features are reviewed after the presentation of two clinical cases, where the second one is a patient with type 1 Neurofibromatosis. Copyright © 2014. Published by Elsevier Espana.

Paraganglioma of the larynx. A case report and clinical review.

PubMed

Shipton, E A; van der Linde, J C

1984-02-04

An 8-year-old Black boy recently underwent surgery for the excision of a non-chromaffin laryngeal paraganglioma. The peri-operative management of this patient is presented. The specific problems involved and their importance to the anaesthesiologist and surgeon alike are discussed.

Diagnosis and Management of Hereditary Phaeochromocytoma and Paraganglioma.

PubMed

Lalloo, Fiona

2016-01-01

About 30% of phaeochromocytomas or paragangliomas are genetic. Whilst some individuals will have clinical features or a family history of inherited cancer syndrome such as neurofibromatosis type 1 (NF1) or multiple endocrine neoplasia 2 (MEN2), the majority will present as an isolated case. To date, 14 genes have been described in which pathogenic mutations have been demonstrated to cause paraganglioma or phaeochromocytoma . Many cases with a pathogenic mutation may be at risk of developing further tumours. Therefore, identification of genetic cases is important in the long-term management of these individuals, ensuring that they are entered into a surveillance programme. Mutation testing also facilitates cascade testing within the family, allowing identification of other at-risk individuals. Many algorithms have been described to facilitate cost-effective genetic testing sequentially of these genes, with phenotypically driven pathways. New genetic technologies including next-generation sequencing and whole-exome sequencing will allow much quicker, cheaper and extensive testing of individuals in whom a genetic aetiology is suspected.

Large gangliocytic paraganglioma of the duodenum: A rare entity.

PubMed

Hernández, Alejandra Gordillo; Lanuza, Eduardo Dominguez-Adame; Matias, Auxiliadora Cano; Huertas, Rosario Perez; Rodriguez, Katherine Maria Gallardo; Perez, Purificacion Gallinato; Mompean, Fernando Oliva

2015-08-27

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.

Large gangliocytic paraganglioma of the duodenum: A rare entity

PubMed Central

Hernández, Alejandra Gordillo; Lanuza, Eduardo Dominguez-Adame; Matias, Auxiliadora Cano; Huertas, Rosario Perez; Rodriguez, Katherine Maria Gallardo; Perez, Purificacion Gallinato; Mompean, Fernando Oliva

2015-01-01

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence. PMID:26328037

Unusual Presentation of Bladder Paraganglioma: Comparison of 131I MIBG SPECT/CT and 68Ga DOTANOC PET/CT

PubMed Central

Jain, Tarun Kumar; Basher, Rajender Kumar; Gupta, Nitin; Shukla, Jaya; Singh, Shrawan Kumar; Mittal, Bhagwant Rai

2016-01-01

Extraadrenal chromaffin cell-related tumors or paragangliomas are rare, especially in the bladder, accounting for less than 1% of cases. We report a 16-year-old boy who presented with hematuria and paroxysmal headache and was found to have a prostatic growth infiltrating the urinary bladder on anatomical imaging. Iodine-131 (131I) metaiodobenzylguanidine (MIBG) whole-body scanning and subsequently gallium-68 (68Ga) DOTANOC positron emission tomography/computed tomography (PET/CT) were performed. The MIBG scan revealed a non-tracer-avid soft-tissue mass, while DOTANOC PET/CT revealed a tracer-avid primary soft-tissue mass involving the urinary bladder and prostate with metastasis to the iliac lymph nodes. He underwent surgical management; histopathology of the surgical specimen revealed a bladder paraganglioma, whereas the prostate was found to be free of tumor. PMID:26912984

Unusual Presentation of Bladder Paraganglioma: Comparison of (131)I MIBG SPECT/CT and (68)Ga DOTANOC PET/CT.

PubMed

Jain, Tarun Kumar; Basher, Rajender Kumar; Gupta, Nitin; Shukla, Jaya; Singh, Shrawan Kumar; Mittal, Bhagwant Rai

2016-01-01

Extraadrenal chromaffin cell-related tumors or paragangliomas are rare, especially in the bladder, accounting for less than 1% of cases. We report a 16-year-old boy who presented with hematuria and paroxysmal headache and was found to have a prostatic growth infiltrating the urinary bladder on anatomical imaging. Iodine-131 ((131)I) metaiodobenzylguanidine (MIBG) whole-body scanning and subsequently gallium-68 ((68)Ga) DOTANOC positron emission tomography/computed tomography (PET/CT) were performed. The MIBG scan revealed a non-tracer-avid soft-tissue mass, while DOTANOC PET/CT revealed a tracer-avid primary soft-tissue mass involving the urinary bladder and prostate with metastasis to the iliac lymph nodes. He underwent surgical management; histopathology of the surgical specimen revealed a bladder paraganglioma, whereas the prostate was found to be free of tumor.

Malignant Jugular Paraganglioma: Unusual Presentation on 68Ga DOTANOC PET/CT.

PubMed

Jain, Tarun Kumar; Basher, Rajender Kumar; Shukla, Jaya; Mittal, Bhagwant Rai; Panda, Naresh K

2016-02-01

Metastatic jugular paraganglioma are rare tumors and account for less than 1% of the cases of head and neck tumors. We report a 40-year-old woman of jugular paraganglioma, presenting with right-sided neck swelling, hearing loss, and pulsatile tinnitus. Contrast-enhanced CT temporal bone revealed a mass in the right jugular foramina and extending inferiorly to internal jugular vein. Ga DOTANOC PET/CT was performed, which revealed somatostatin receptor expressing lesion in the right internal jugular vein and extension into sigmoid sinus and additional metastatic focus in the sacrum.

A large deletion in the succinate dehydrogenase B gene (SDHB) in a Japanese patient with abdominal paraganglioma and concomitant metastasis.

PubMed

Kodama, Hitomi; Iihara, Masatoshi; Nissato, Sumiko; Isobe, Kazumasa; Kawakami, Yasushi; Okamoto, Takahiro; Takekoshi, Kazuhiro

2010-01-01

Recently, mutations in nuclear genes encoding two mitochondrial complex II subunit proteins, Succinate dehydrogenase D (SDHD) and SDHB, have been found to be associated with the development of familial pheochromocytomas and paragangliomas (hereditary pheochromocytoma/paraganglioma syndrome: HPPS). Growing evidence suggests that the mutation of SDHB is highly associated with abdominal paraganglioma and the following distant metastasis (malignant paraganglioma). In the present study, we used multiplex ligation dependent probe amplification (MLPA) analysis to identify a large heterozygous SDHB gene deletion encompassing sequences corresponding to the promoter region, in addition to exon 1 and exon 2 malignant paraganglioma patient in whom previously characterized SDHB mutations were undetectable. This is the first Japanese case report of malignant paraganglioma, with a large SDHB deletions. Our present findings strongly support the notion that large deletions in the SDHB gene should be considered in patients lacking characterized SDHB mutations.

MRI screening of kindred at risk of developing paragangliomas: support for genomic imprinting in hereditary glomus tumours.

PubMed Central

van Gils, A. P.; van der Mey, A. G.; Hoogma, R. P.; Sandkuijl, L. A.; Maaswinkel-Mooy, P. D.; Falke, T. H.; Pauwels, E. K.

1992-01-01

Paragangliomas of the head and neck (glomus tumours) can occur in a hereditary pattern and may be hormonally active as well as being associated with paragangliomas elsewhere. A number of these tumours may be present without symptoms. To detect the presence of subclinical paragangliomas we screened 83 members of a family at risk of developing hereditary paragangliomas using whole body MRI and urinary catecholamine testing. In eight previously diagnosed members, eight known glomus tumours of which one functioning, and two unknown glomus tumours and one unknown pheochromocytoma were present. Six unsuspected members showed ten glomus tumours and one pheochromocytoma. It has been suggested that the manifestation of hereditary glomus tumours is determined by the sex of the transmitting parent. There were no tumours in the descendants of female gene carriers. Comparing the likelihood of inheritance with genomic imprinting versus inheritance without genomic imprinting we found an odds ratio of 23375 in favour of genomic imprinting. PMID:1616861

Facial Nerve Paralysis after Onyx Embolization of a Jugular Paraganglioma: A Case Report with a Long-Term Follow Up

PubMed Central

Odat, Haitham; Alawneh, Khaled; Al-Qudah, Mohannad

2018-01-01

Jugular paragangliomas are slow growing highly vascular tumors arising from jugular paraganglia. The gold standard of treatment is complete surgical resection. Pre-operative embolization of these highly vascular tumors is essential to reduce intra-operative bleeding, allow safe dissection, and decrease operative time and post-operative complications. Onyx (ethylene-vinyl alcohol copolymer) has been widely used as permanent occluding material for vascular tumors of skull base because of its unique physical properties. We present the case of a 33-year-old woman who had left-sided facial nerve paralysis after Onyx embolization of jugular paraganglioma. The tumor was resected on the next day of embolization. The patient was followed up for 30 months with serial imaging studies and facial nerve assessment. The facial verve function improved from House–Brackmann grade V to grade II at the last visit. PMID:29518926

Facial Nerve Paralysis after Onyx Embolization of a Jugular Paraganglioma: A Case Report with a Long-Term Follow Up.

PubMed

Odat, Haitham; Alawneh, Khaled; Al-Qudah, Mohannad

2018-03-07

Jugular paragangliomas are slow growing highly vascular tumors arising from jugular paraganglia. The gold standard of treatment is complete surgical resection. Pre-operative embolization of these highly vascular tumors is essential to reduce intra-operative bleeding, allow safe dissection, and decrease operative time and post-operative complications. Onyx (ethylene-vinyl alcohol copolymer) has been widely used as permanent occluding material for vascular tumors of skull base because of its unique physical properties. We present the case of a 33-year-old woman who had left-sided facial nerve paralysis after Onyx embolization of jugular paraganglioma. The tumor was resected on the next day of embolization. The patient was followed up for 30 months with serial imaging studies and facial nerve assessment. The facial verve function improved from House-Brackmann grade V to grade II at the last visit.

Metastatic Renal Cell Carcinoma Masquerading as Jugular Foramen Paraganglioma: A Role for Novel Magnetic Resonance Imaging.

PubMed

Thomas, Andrew J; Wiggins, Richard H; Gurgel, Richard K

2017-08-01

To describe a case of metastatic renal cell carcinoma (RCC) masquerading as a jugular foramen paraganglioma (JP). To compare imaging findings between skull base metastatic RCC and histologically proven paraganglioma. A case of unexpected metastatic skull base RCC is reviewed. Computed tomography (CT) and magnetic resonance imaging (MRI) were compared between 3 confirmed cases of JP and our case of metastatic RCC. Diffusion-weighted MRI (DW-MRI) sequences and computed apparent diffusion coefficient (ADC) values were compared between these entities. A 55-year-old man presents with what appears clinically and radiographically to be JP. The tumor was resected, then discovered on postoperative pathology to be metastatic RCC. Imaging was retrospectively compared between 3 histologically confirmed cases of JP and our case of skull base RCC. The RCC metastasis was indistinguishable from JP on CT and traditional MRI but distinct by ADC values calculated from DW-MRI. Metastatic RCC at the skull base may mimic the clinical presentation and radiographic appearance of JP. The MRI finding of flow voids is seen in both paraganglioma and metastatic RCC. Diffusion-weighted MRI is able to distinguish these entities, highlighting its potential utility in distinguishing skull base lesions.

Synchronous adrenocortical neoplasms, paragangliomas, and pheochromocytomas: syndromic considerations regarding an unusual constellation of endocrine tumors.

PubMed

LeBlanc, Melissa; Tabrizi, Mohsen; Kapsner, Patricia; Hanson, Joshua Anspach

2014-12-01

The most common clinical syndromes presenting with paragangliomas and/or pheochromocytomas as their endocrine components are multiple endocrine neoplasia type 2, neurofibromatosis, Von Hippel-Lindau syndrome, Carney-Stratakis syndrome, Carney triad, and the recently described hereditary paraganglioma syndrome. Only Carney triad is known to also present with adrenocortical adenomas, currently representing the only described syndrome in which all 3 of the aforementioned tumors are found together. In most cases, prototypical lesions of the triad such as gastrointestinal stromal tumor and pulmonary chondromas are also seen. We present a case of a young woman with synchronous paragangliomas, adrenal/extra-adrenal cortical neoplasms, and pheochromocytoma without genetic mutations for multiple endocrine neoplasia 2, Von Hippel-Lindau syndrome, neurofibromatosis, and succinate dehydrogenase. We speculate that this represents a previously undescribed presentation of Carney triad and, at the very least, indicates the need for monitoring for the development of other tumors of the triad. Copyright © 2014 Elsevier Inc. All rights reserved.

Hypertensive crisis during wide excision of gastrointestinal stromal cell tumor (GIST): Undiagnosed paraganglioma -A case report-.

PubMed

Shinn, Helen Ki; Jung, Jong Kwon; Park, Jay Kim; Kim, Jong Hoon; Jung, In Young; Lee, Hong Sik

2012-03-01

Although paraganglioma (PGL), an extra-adrenal retroperitoneal pheochromocytoma (PHEO), is a rare catecholamine-secreting neuroendocrine tumor, it can cause severe hypertensive crisis during anesthesia or surgery if undiagnosed preoperatively. Extraluminal perigastric masses may be presumed to be gastrointestinal stromal tumors (GISTs) or soft tissue sarcomas even when histologic confirmation is not possible. Therefore, without a histologic diagnosis or symptoms of excessive catecholamine secretion, PGL may be mistaken for GIST. We report a case of preoperatively undiagnosed PGL which caused hypertensive crisis during anesthesia for retroperitoneal mass excision.

Contemporary management of jugular paragangliomas.

PubMed

Wanna, George B; Sweeney, Alex D; Haynes, David S; Carlson, Matthew L

2015-04-01

Jugular paragangliomas are generally benign slow-growing tumors that can cause pulsatile tinnitus, hearing loss, and cranial nerves neuropathy. Progressive growth can also lead to intracranial extension. Historically, the treatment of choice for these lesions has been gross total resection. However, over the last 15 years, many groups have adopted less invasive management strategies including stereotactic radiation therapy, subtotal resection, and primary observation in order to reduce treatment-associated morbidity. The focus of this article is to review the modern management of jugular paraganglioma, highlighting the evolving treatment paradigm at the Otology Group of Vanderbilt. Copyright © 2015 Elsevier Inc. All rights reserved.

Hypertensive crisis during wide excision of gastrointestinal stromal cell tumor (GIST): Undiagnosed paraganglioma -A case report-

PubMed Central

Shinn, Helen Ki; Jung, Jong Kwon; Park, Jay Kim; Kim, Jong Hoon; Jung, In Young

2012-01-01

Although paraganglioma (PGL), an extra-adrenal retroperitoneal pheochromocytoma (PHEO), is a rare catecholamine-secreting neuroendocrine tumor, it can cause severe hypertensive crisis during anesthesia or surgery if undiagnosed preoperatively. Extraluminal perigastric masses may be presumed to be gastrointestinal stromal tumors (GISTs) or soft tissue sarcomas even when histologic confirmation is not possible. Therefore, without a histologic diagnosis or symptoms of excessive catecholamine secretion, PGL may be mistaken for GIST. We report a case of preoperatively undiagnosed PGL which caused hypertensive crisis during anesthesia for retroperitoneal mass excision. PMID:22474560

CT AND MRI FEATURES OF CAROTID BODY PARAGANGLIOMAS IN 16 DOGS.

PubMed

Mai, Wilfried; Seiler, Gabriela S; Lindl-Bylicki, Britany J; Zwingenberger, Allison L

2015-01-01

Carotid body tumors (paragangliomas) arise from chemoreceptors located at the carotid bifurcation. In imaging studies, this neoplasm may be confused with other neck neoplasms such as thyroid carcinoma. The purpose of this retrospective, cross-sectional study was to describe computed tomographic (CT) and magnetic resonance imaging (MRI) characteristics of confirmed carotid body tumors in a multi-institutional sample of dogs. A total of 16 dogs met inclusion criteria (14 examined using CT and two with MRI). The most common reason for imaging was a palpable cervical mass or respiratory signs (i.e., dyspnea or increased respiratory noises). The most commonly affected breed was Boston terrier (n = 5). Dogs were predominantly male castrated (n = 10) and the median age was 9 years [range 3-14.5]. Most tumors appeared as a large mass centered at the carotid bifurcation, with poor margination in six dogs and discrete margins in ten dogs. Masses were iso- to hypoattenuating to adjacent muscles in CT images and hyperintense to muscles in T1- and T2-weighted MRI. For both CT and MRI, masses typically showed strong and heterogeneous contrast enhancement. There was invasion into the adjacent structures in 9/16 dogs. In six of these nine dogs, the basilar portion of the skull was affected. The external carotid artery was entrapped in seven dogs. There was invasion into the internal jugular vein in three dogs, and into the external jugular, maxillary, and linguo-facial veins in one dog. Imaging characteristics helped explain some clinical presentations such as breathing difficulties, Horner's syndrome, head tilt, or facial nerve paralysis. © 2015 American College of Veterinary Radiology.

Paraganglioma of the Urinary Bladder: A Rare Cause of Hypertension and Urinary Tract Infections.

PubMed

Chaaya, Gerard; Morales, Jorge; Castiglioni, Analia; Subhani, Noman; Asmar, Abdo

2018-02-01

Pheochromocytoma is a neoplasm, which develops from cells of the chromaffin tissues that are derived from the ectodermic neural system and mostly situated within the adrenal medulla. Approximately 15% of pheochromocytoma cases arise from extra-adrenal chromaffin tissue. Pheochromocytoma of the bladder is rare and accounts for less than 0.06% of all bladder neoplasms and less than 1% of all pheochromocytomas. We report a case of a young woman who presented with uncontrolled hypertension, recurrent urinary tract infections and micturition attacks and was found to have a metastatic bladder paraganglioma. In addition, we provide a summary table of the clinical manifestations of paragangliomas based on anatomic locations. Published by Elsevier Inc.

The Dutch founder mutation SDHD.D92Y shows a reduced penetrance for the development of paragangliomas in a large multigenerational family.

PubMed

Hensen, Erik F; Jansen, Jeroen C; Siemers, Maaike D; Oosterwijk, Jan C; Vriends, Annette Hjt; Corssmit, Eleonora Pm; Bayley, Jean-Pierre; van der Mey, Andel Gl; Cornelisse, Cees J; Devilee, Peter

2010-01-01

Germline mutations in SDHD predispose to the development of head and neck paragangliomas, and phaeochromocytomas. The risk of developing a tumor depends on the sex of the parent who transmits the mutation: paragangliomas only arise upon paternal transmission. In this study, both the risk of paraganglioma and phaeochromocytoma formation, and the risk of developing associated symptoms were investigated in 243 family members with the SDHD.D92Y founder mutation. By using the Kaplan-Meier method, age-specific penetrance was calculated separately for paraganglioma formation as defined by magnetic resonance imaging (MRI) and for paraganglioma-related signs and symptoms. Evaluating clinical signs and symptoms alone, the penetrance reached a maximum of 57% by the age of 47 years. When MRI detection of occult paragangliomas was included, penetrance was estimated to be 54% by the age of 40 years, 68% by the age of 60 years and 87% by the age of 70 years. Multiple tumors were found in 65% and phaeochromocytomas were diagnosed in 8% of paraganglioma patients. Malignant paraganglioma was diagnosed in one patient (3%). Although the majority of carriers of a paternally inherited SDHD mutation will eventually develop head and neck paragangliomas, we find a lower penetrance than previous estimates from studies based on predominantly index cases. The family-based study described here emphasizes the importance of the identification and inclusion of clinically unaffected mutation carriers in all estimates of penetrance. This finding will allow a more accurate genetic counseling and warrants a 'wait and scan' policy for asymptomatic paragangliomas, combined with biochemical screening for catecholamine excess in SDHD-linked patients.

Paraganglioma presenting as cholesterol granuloma of the petrous apex.

PubMed

Heman-Ackah, Selena E; Huang, Tina C

2013-09-01

We report the unique finding of a petrous apex cholesterol granuloma associated with a paraganglioma, also known as a glomus jugulare tumor, in a 52-year-old woman who presented to our department with pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. She had been treated for a petrous apex cholesterol granuloma 20 years earlier, at which time she had undergone drainage of the granuloma via subtotal petrous apicectomy. When she came to our facility approximately 20 years later, she had signs and symptoms consistent with a jugular paraganglioma, which was likely to have been present at the time of her initial presentation for the cholesterol granuloma. In fact, microscopic bleeding from the paraganglioma might have led to the formation of the cholesterol granuloma. The metachronous presentation of these two entities, which to our knowledge has not been reported previously in the literature, indicates the potential association of paragangliomas with the formation of cholesterol granulomas of the petrous apex.

Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome.

PubMed

Buffet, Alexandre; Smati, Sarra; Mansuy, Ludovic; Ménara, Mélanie; Lebras, Maëlle; Heymann, Marie-Françoise; Simian, Christophe; Favier, Judith; Murat, Arnaud; Cariou, Bertrand; Gimenez-Roqueplo, Anne-Paule

2014-02-01

HIF2A germline mutations were known to cause congenital polycythemia. Recently, HIF2A somatic mutations were found in several patients with polycythemia and paraganglioma, pheochromocytoma, or somatostatinoma, suggesting the occurrence of a de novo postzygotic HIF2A mutation that has not been demonstrated clearly. Patient 1 is a woman suffering from polycythemia diagnosed at the age of 16 years. She was operated on for a pheochromocytoma at 45 years and for two abdominal paragangliomas at 59 years. She was also diagnosed with somatostatinoma. Patient 2 is a young boy who suffered from polycythemia since infancy. He underwent surgery for a nonfunctional adrenal paraganglioma at the age of 9 years. We sequenced by Sanger and next-generation sequencing the HIF2A gene in DNA extracted from tumors, leukocytes, and buccal cells. In patient 1, we identified a somatic HIF2A mutation (c.1586T>C; p.Leu529Pro) in DNA extracted from both paragangliomas. The mutation was detected as a somatic mosaic in DNA extracted from somatostatinoma and was absent from germline DNA. In patient 2, we found an HIF2A heterozygous mutation (c.1625T>C; p.Leu542Pro) in the paraganglioma, but the mutation was also present as a mosaic in leukocyte DNA and in DNA extracted from buccal cells (3.3 and 8.96% of sequencing reads, respectively). Both mutations disrupt the hydroxylation domain of the HIF2α protein. Our study shows that HIF2A-related tumors are caused by postzygotic mutations occurring in early developmental stages. Potential germline mosaicism should be considered during the familial genetic counseling when an individual has been diagnosed with HIF2A-related polycythemia-paraganglioma syndrome.

Phaeochromocytoma and Paraganglioma.

PubMed

Gunawardane, P T Kavinga; Grossman, Ashley

2017-01-01

Phaeochromocytomas and paragangliomas are relatively uncommon tumours which may be manifest in many ways, specifically as sustained or paroxysmal hypertension, episodes of palpitations, sweating, headache and anxiety, or increasingly as an incidental finding. Recent studies have shown that an increasing number are due to germline mutations. This review concentrates on the diagnosis, biochemistry and treatment of these fascinating tumours.

Resection of a large carotid paraganglioma in Carney-Stratakis syndrome: a multidisciplinary feat.

PubMed

Nicholas, Rebecca Spenser; Quddus, Ayyaz; Topham, Charlotte; Baker, Daryll

2015-04-16

A 39-year-old man was referred to the vascular surgeons with a right-sided cervical mass, palpitations, headaches and sweating. He had presented with abdominal discomfort 12 months earlier. Investigations had revealed a gastrointestinal stromal tumour (GIST) and left adrenalectomy. CT of the neck with contrast demonstrated a large right carotid paraganglioma, extending superiorly from below the carotid bifurcation to encase the internal carotid artery. Genetic screening confirmed the diagnosis of Carney-Stratakis syndrome, an SDH-D germline mutation leading to GIST and multifocal paragangliomas. Successful surgical excision required considerable multidisciplinary teamwork between neuroendocrinologists, anaesthetists and surgeons. The tumour was highly vascular and involved the right carotid body, hypoglossal and vagus nerves. Access was challenging and maxillofacial surgical expertise were required for division of the mandible. The patient made a good recovery following speech and swallowing rehabilitation. 2015 BMJ Publishing Group Ltd.

Resection of a large carotid paraganglioma in Carney-Stratakis syndrome: a multidisciplinary feat

PubMed Central

Nicholas, Rebecca Spenser; Quddus, Ayyaz; Topham, Charlotte; Baker, Daryll

2015-01-01

A 39-year-old man was referred to the vascular surgeons with a right-sided cervical mass, palpitations, headaches and sweating. He had presented with abdominal discomfort 12 months earlier. Investigations had revealed a gastrointestinal stromal tumour (GIST) and left adrenalectomy. CT of the neck with contrast demonstrated a large right carotid paraganglioma, extending superiorly from below the carotid bifurcation to encase the internal carotid artery. Genetic screening confirmed the diagnosis of Carney-Stratakis syndrome, an SDH-D germline mutation leading to GIST and multifocal paragangliomas. Successful surgical excision required considerable multidisciplinary teamwork between neuroendocrinologists, anaesthetists and surgeons. The tumour was highly vascular and involved the right carotid body, hypoglossal and vagus nerves. Access was challenging and maxillofacial surgical expertise were required for division of the mandible. The patient made a good recovery following speech and swallowing rehabilitation. PMID:25883251

Stages of Pheochromocytoma and Paraganglioma

MedlinePlus

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

Pitfalls in genetic analysis of pheochromocytomas/paragangliomas-case report.

PubMed

Canu, Letizia; Rapizzi, Elena; Zampetti, Benedetta; Fucci, Rossella; Nesi, Gabriella; Richter, Susan; Qin, Nan; Giachè, Valentino; Bergamini, Carlo; Parenti, Gabriele; Valeri, Andrea; Ercolino, Tonino; Eisenhofer, Graeme; Mannelli, Massimo

2014-07-01

About 35% of patients with pheochromocytoma/paraganglioma carry a germline mutation in one of the 10 main susceptibility genes. The recent introduction of next-generation sequencing will allow the analysis of all these genes in one run. When positive, the analysis is generally unequivocal due to the association between a germline mutation and a concordant clinical presentation or positive family history. When genetic analysis reveals a novel mutation with no clinical correlates, particularly in the presence of a missense variant, the question arises whether the mutation is pathogenic or a rare polymorphism. We report the case of a 35-year-old patient operated for a pheochromocytoma who turned out to be a carrier of a novel SDHD (succinate dehydrogenase subunit D) missense mutation. With no positive family history or clinical correlates, we decided to perform additional analyses to test the clinical significance of the mutation. We performed in silico analysis, tissue loss of heterozygosity analysis, immunohistochemistry, Western blot analysis, SDH enzymatic assay, and measurement of the succinate/fumarate concentration ratio in the tumor tissue by tandem mass spectrometry. Although the in silico analysis gave contradictory results according to the different methods, all the other tests demonstrated that the SDH complex was conserved and normally active. We therefore came to the conclusion that the variant was a nonpathogenic polymorphism. Advancements in technology facilitate genetic analysis of patients with pheochromocytoma but also offer new challenges to the clinician who, in some cases, needs clinical correlates and/or functional tests to give significance to the results of the genetic assay.

Genetics Home Reference: nonsyndromic paraganglioma

MedlinePlus

... people with the nonsyndromic form of these conditions. Gene mutations increase the risk of developing paraganglioma or pheochromocytoma ... Information What is a gene? What is a gene mutation and how do mutations occur? How can gene ...

Posterior Retroperitoneoscopic Resection of Extra-adrenal Paraganglioma Located in the Aorto-caval Space.

PubMed

Kang, Sang-Wook; Kandil, Emad; Kim, Min Jhi; Kim, Kwang Soon; Lee, Cho Rok; Jeong, Jong Ju; Nam, Kee-Hyun; Chung, Woong Youn; Park, Cheong Soo

2018-04-01

The posterior retroperitoneoscopic adrenalec tomy has several advantages compared with the transperitoneal approach such as a shorter and more direct route to the target organ, no breach of the intraperitoneal space, and no required retraction of the adjacent organs. It also is a safe procedure with a short learning curve.1 - 5 This report presents a challenging case of an extra-adrenal paraganglioma located in the aorto-caval space and managed using the retroperitoneal approach. A 39-year-old man was placed in the prone jackknife position, and three incisions were made in the right posterior abdominal wall for placement of the laparoscopic ports. The retroperitoneal space was entered with diathermy and blunt finger dissection, and retropneumoperitoneum was achieved with carbon dioxide insufflation pressure up to 18 mmHg. After identification of the right kidney and vessels, the tumor was meticulously dissected and excised with an energy device. The specimen was removed using a laparoscopic specimen retrieval bag, and the port sites were closed in layers. The operative time was 130 min, and the total blood loss was 30 ml. The tumor was diagnosed as a moderately differentiated extra-adrenal paraganglioma. The Von Hippel-Lindau gene mutation was detected using next-generation sequencing. The posterior retroperitoneoscopic approach is a safe, feasible, and effective method for excising an extra-adrenal paraganglioma even in the aorto-caval space. The authors suggest that this procedure is a useful surgical option for treatment of an aorto-caval paraganglioma for selected patients and by experienced surgeons.

Bilateral pheochromocytoma associated with paraganglioma and papillary thyroid carcinoma: report of an unusual case.

PubMed

Yang, Jeong Hoon; Bae, Sung Jin; Park, Sanghui; Park, Hyun-Kyung; Jung, Hye Seung; Chung, Jae Hoon; Min, Yong-Ki; Lee, Myung-Shik; Kim, Kwang-Won; Lee, Moon-Kyu

2007-04-01

A 42-year old woman presented with headache, palpitation and facial flushing. Ultrasonograms and computed tomograms revealed tumors in both of the adrenal glands, anterior aspect of the inferior vena cava, and the right lobe of the thyroid gland. Fine needle aspiration biopsy of the thyroid nodule revealed papillary thyroid carcinoma. Serum calcitonin, CEA, intact PTH and calcium levels were within normal limits. Markedly elevated levels of urinary normetanephrine and vanillylmandelic acid, and the result of 131I-metaiodobenzylguanidine (131I-MIBG) scintigraphy indicated that both adrenal masses were pheochromocytoma. Bilateral adrenalectomy, paracaval mass removal and total thyroidectomy together with central lymph node dissection were performed. The final pathological diagnosis was bilateral adrenal pheochromocytoma, paraganglioma, papillary thyroid carcinoma and either parathyroid adenoma or hyperplasia. Analysis of the RET proto-oncogene mutation, von Hippel Lindau mutation, succinate dehydrogenase subunit B mutation, and succinate dehydrogenase subunit D mutation yielded negative results. The relationship of these lesions could not be determined. This is the first report of a combination of bilateral pheochromocytoma, abdominal paraganglioma, papillary thyroid carcinoma and either parathyroid adenoma or hyperplasia without hyperparathyroidism.

Treatment Option Overview (Pheochromocytoma and Paraganglioma)

MedlinePlus

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma.

PubMed

Bayley, Jean-Pierre; Kunst, Henricus P M; Cascon, Alberto; Sampietro, Maria Lourdes; Gaal, José; Korpershoek, Esther; Hinojar-Gutierrez, Adolfo; Timmers, Henri J L M; Hoefsloot, Lies H; Hermsen, Mario A; Suárez, Carlos; Hussain, A Karim; Vriends, Annette H J T; Hes, Frederik J; Jansen, Jeroen C; Tops, Carli M; Corssmit, Eleonora P; de Knijff, Peter; Lenders, Jacques W M; Cremers, Cor W R J; Devilee, Peter; Dinjens, Winand N M; de Krijger, Ronald R; Robledo, Mercedes

2010-04-01

Paragangliomas and phaeochromocytomas are neuroendocrine tumours associated frequently with germline mutations of SDHD, SDHC, and SDHB. Previous studies have shown the imprinted SDHAF2 gene to be mutated in a large Dutch kindred with paragangliomas. We aimed to identify SDHAF2 mutation carriers, assess the clinical genetic significance of SDHAF2, and describe the associated clinical phenotype. We undertook a multicentre study in Spain and The Netherlands in 443 apparently sporadic patients with paragangliomas and phaeochromocytomas who did not have mutations in SDHD, SDHC, or SDHB. We analysed DNA of 315 patients for germline mutations of SDHAF2; a subset (n=200) was investigated for gross gene deletions. DNA from a group of 128 tumours was studied for somatic mutations. We also examined a Spanish family with head and neck paragangliomas with a young age of onset for the presence of SDHAF2 mutations, undertook haplotype analysis in this kindred, and assessed their clinical phenotype. We did not identify any germline or somatic mutations of SDHAF2, and no gross gene deletions were noted in the subset of apparently sporadic patients analysed. Investigation of the Spanish family identified a pathogenic germline DNA mutation of SDHAF2, 232G-->A (Gly78Arg), identical to the Dutch kindred. SDHAF2 mutations do not have an important role in phaeochromocytoma and are rare in head and neck paraganglioma. Identification of a second family with the Gly78Arg mutation suggests that this is a crucial residue for the function of SDHAF2. We conclude that SDHAF2 mutation analysis is justified in very young patients with isolated head and neck paraganglioma without mutations in SDHD, SDHC, or SDHB, and in individuals with familial antecedents who are negative for mutations in all other risk genes. Dutch Cancer Society, European Union 6th Framework Program, Fondo Investigaciones Sanitarias, Fundación Mutua Madrileña, and Red Temática de Investigación Cooperativa en Cáncer. 2010

Evaluation of somatostatin, CXCR4 chemokine and endothelin A receptor expression in a large set of paragangliomas.

PubMed

Kaemmerer, Daniel; Sänger, Jörg; Arsenic, Ruza; D'Haese, Jan G; Neumann, Jens; Schmitt-Graeff, Annette; Wirtz, Ralph Markus; Schulz, Stefan; Lupp, Amelie

2017-10-27

Paragangliomas are predominantly benign tumors, but in some cases invasive growth and also metastasis are observed. Given the limited number of nonsurgical treatment options, novel target structures for diagnostics and therapy of this tumor entity are urgently needed. In the present study, expression of all five somatostatin receptor (SST) subtypes, chemokine receptor CXCR4 and endothelin receptor type A (ETA) was assessed by means of immunohistochemistry in a total of 66 paraffin-embedded paraganglioma samples from 55 patients. The stainings were rated by means of the Immunoreactive Score and correlated to clinical data and to succinate dehydrogenase subunit B (SDHB) expression. SST2A was by far the most prominent receptor in the paragangliomas investigated. It was present in 89% of the tumors at a high intensity, followed by SST5, SST3, SST1 and SST4, which were detected in 47%, 35%, 35% and 13% of the samples, respectively. SDHB positive tumors exhibited significantly higher SST2A and SST3 expression as compared to SDHB negative cases. There was no correlation between SST and Ki-67 expression or grading of the tumors and no difference in SST expression between primary tumors and metastases. Cell surface expression of CXCR4 and ETA was detected only in few samples. On tumor capillaries, however, exceptionally strong staining for these two receptors was noticed in the vast majority of the tumors. In conclusion, paragangliomas are well suited for SST2A-based diagnostics and treatment modalities. An indirect targeting of these highly vascularized tumors via CXCR4 or ETA may also represent a promising future strategy.

Evaluation of somatostatin, CXCR4 chemokine and endothelin A receptor expression in a large set of paragangliomas

PubMed Central

Kaemmerer, Daniel; Sänger, Jörg; Arsenic, Ruza; D’Haese, Jan G.; Neumann, Jens; Schmitt-Graeff, Annette; Wirtz, Ralph Markus; Schulz, Stefan; Lupp, Amelie

2017-01-01

Paragangliomas are predominantly benign tumors, but in some cases invasive growth and also metastasis are observed. Given the limited number of nonsurgical treatment options, novel target structures for diagnostics and therapy of this tumor entity are urgently needed. In the present study, expression of all five somatostatin receptor (SST) subtypes, chemokine receptor CXCR4 and endothelin receptor type A (ETA) was assessed by means of immunohistochemistry in a total of 66 paraffin-embedded paraganglioma samples from 55 patients. The stainings were rated by means of the Immunoreactive Score and correlated to clinical data and to succinate dehydrogenase subunit B (SDHB) expression. SST2A was by far the most prominent receptor in the paragangliomas investigated. It was present in 89% of the tumors at a high intensity, followed by SST5, SST3, SST1 and SST4, which were detected in 47%, 35%, 35% and 13% of the samples, respectively. SDHB positive tumors exhibited significantly higher SST2A and SST3 expression as compared to SDHB negative cases. There was no correlation between SST and Ki-67 expression or grading of the tumors and no difference in SST expression between primary tumors and metastases. Cell surface expression of CXCR4 and ETA was detected only in few samples. On tumor capillaries, however, exceptionally strong staining for these two receptors was noticed in the vast majority of the tumors. In conclusion, paragangliomas are well suited for SST2A-based diagnostics and treatment modalities. An indirect targeting of these highly vascularized tumors via CXCR4 or ETA may also represent a promising future strategy. PMID:29163802

Old, New, and Emerging Immunohistochemical Markers in Pheochromocytoma and Paraganglioma.

PubMed

Cheung, Veronica K Y; Gill, Anthony J; Chou, Angela

2018-05-19

The evolution of genetic research over the past two decades has greatly improved the understanding of pheochromocytomas and paragangliomas. It is now accepted that more than one third of pheochromocytoma and paragangliomas arise in the context of syndromic disease, usually hereditary. The genetic profile of these tumors also has important prognostic implications which may help guide treatment. Accompanying the changing molecular landscape is the development of new immunohistochemical markers. Initially used in assisting with diagnosis, immunohistochemical markers have now become an important adjunct to screening programs for inherited conditions and subsequently as prognostic markers. The accessibility and efficiency of immunohistochemistry bring pathologists to the forefront in triaging patients based on tumor genotype-phenotype. In this review, we provide an update on the role of immunohistochemistry in the diagnosis of pheochromocytomas and paragangliomas, as an adjunct to assessment for hereditary disease and finally as a potential tool to assist risk stratification.

Single-centre study of the diagnostic performance of plasma metanephrines with seated sampling for the diagnosis of phaeochromocytoma/paraganglioma.

PubMed

Boot, Christopher; Toole, Barry; Johnson, Sarah J; Ball, Stephen; Neely, Dermot

2017-01-01

Background Measurement of plasma metanephrines is regarded as one of the best screening tests for phaeochromocytoma/paraganglioma. Current guidelines recommend that samples are ideally collected in the supine position after 30 min rest and interpreted using supine reference ranges, in order to optimize the diagnostic performance of the test. Current practice in our centre is to collect samples for plasma metanephrines from seated patients. The aim of the study was to determine, if seated sampling for plasma metanephrines provides acceptable diagnostic performance in our centre. Methods Clinical and laboratory data of 113 patients, gathered over a four-year period 2010-2014, were reviewed. All had undergone preoperative plasma metanephrines measurement and had postoperative histopathology confirmation or exclusion of phaeochromocytoma/paraganglioma. Results Of 113 patients included in the study, 40 had a histological diagnosis of phaeochromocytoma/paraganglioma. The remaining 73 patients had an alternative adrenal pathology. The diagnostic sensitivity of normetanephrine or metanephrine above the upper limit of our in-house seated reference range was 93%. However, excluding three cases of paraganglioma determined clinically and biochemically to be non-functional raised the sensitivity to 100%. Diagnostic specificity was 90%. Applying published supine reference ranges made no difference to diagnostic sensitivity in this group of patients but decreased diagnostic specificity to 75%. Conclusions While these data are derived from a relatively small study population, they demonstrate acceptable diagnostic performance for seated plasma metanephrines as a screening test for phaeochromocytoma/paraganglioma. These data highlight a high diagnostic sensitivity for plasma metanephrines with seated sampling in our centre.

Current Approaches and Recent Developments in the Management of Head and Neck Paragangliomas

PubMed Central

Kaliski, Alexandre; Boedeker, Carsten C.; Martucci, Victoria; Fojo, Tito; Adler, John R.

2014-01-01

Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors belonging to the family of pheochromocytoma/paraganglioma neoplasms. Despite advances in understanding the pathogenesis of these tumors, the growth potential and clinical outcome of individual cases remains largely unpredictable. Over several decades, surgical resection has long been the treatment of choice for HNPGLs. However, increasing experience in various forms of radiosurgery has been reported to result in curative-like outcomes, even for tumors localized in the most inaccessible anatomical areas. The emergence of such new therapies challenges the traditional paradigm for the management of HNPGLs. This review will assist and guide physicians who encounter patients with such tumors, either from a diagnostic or therapeutic standpoint. This review will also particularly emphasize current and emerging knowledge in genetics, imaging, and therapeutic options as well as the health-related quality of life for patients with HNPGLs. PMID:25033281

Dynamic Contrast-Enhanced MRI in the Evaluation of Carotid Space Paraganglioma versus Schwannoma.

PubMed

Gaddikeri, Santhosh; Hippe, Daniel S; Anzai, Yoshimi

2016-11-01

To describe the potential role of dynamic contrast-enhanced (DCE) MRI in differentiating carotid space (CS) paraganglioma from schwannoma in the head and neck. We retrospectively reviewed records of 126 patients who had undergone DCE-MRI between June 2008 and July 2014 and found six patients with histologically verified benign CS tumors. The images were evaluated for tumor T1 and T2 signal characteristics, flow voids, and enhancement pattern. The dynamic data were analyzed for quantitative parameters using extended Toft's model (K trans , K ep , V e , and V p ) and semiquantitative parameters based on time-intensity curve (area under curve, peak enhancement, wash-in, wash-out, signal-enhancement ratio [SER], and time for maximum enhancement [TME]). Due to the small sample size, groups were compared qualitatively. Patients with CS paraganglioma (P group, n = 2) and schwannoma (S group, n = 4) were included. All tumors were hypointense on T1W imaging, hyperintense on T2W imaging, and show avid enhancement. One patient with paraganglioma had subtle flow voids. The conventional MR images were insufficient to confidently diagnose tumor type. Both paragangliomas had high peak enhancement and SER, and a short TME, while the schwannomas had relatively low peak enhancement and SER with a longer TME. K trans , K ep , and V e were relatively low in the paragangliomas than in the schwannomas. DCE-MRI could potentially be used to assist differentiating paraganglioma from schwannoma, when diagnosis is difficult on the conventional MR imaging sequences. Simple assessment of semiquantitative parameters suffices to provide supportive information. Copyright © 2016 by the American Society of Neuroimaging.

An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis

PubMed Central

van Nederveen, Francien H; Gaal, José; Favier, Judith; Korpershoek, Esther; Oldenburg, Rogier A; de Bruyn, Elly M C A; Sleddens, Hein F B M; Derkx, Pieter; Rivière, Julie; Dannenberg, Hilde; Petri, Bart-Jeroen; Komminoth, Paul; Pacak, Karel; Hop, Wim C J; Pollard, Patrick J; Mannelli, Massimo; Bayley, Jean-Pierre; Perren, Aurel; Niemann, Stephan; Verhofstad, Albert A; de Bruïne, Adriaan P; Maher, Eamonn R; Tissier, Frédérique; Méatchi, Tchao; Badoual, Cécile; Bertherat, Jérôme; Amar, Laurence; Alataki, Despoina; Van Marck, Eric; Ferrau, Francesco; François, Jerney; de Herder, Wouter W; Peeters, Mark-Paul F M Vrancken; van Linge, Anne; Lenders, Jacques W M; Gimenez-Roqueplo, Anne-Paule; de Krijger, Ronald R; Dinjens, Winand N M

2016-01-01

Summary Background Phaeochromocytomas and paragangliomas are neuro-endocrine tumours that occur sporadically and in several hereditary tumour syndromes, including the phaeochromocytoma–paraganglioma syndrome. This syndrome is caused by germline mutations in succinate dehydrogenase B (SDHB), C (SDHC), or D (SDHD) genes. Clinically, the phaeochromocytoma–paraganglioma syndrome is often unrecognised, although 10–30% of apparently sporadic phaeochromocytomas and paragangliomas harbour germline SDH-gene mutations. Despite these figures, the screening of phaeochromocytomas and paragangliomas for mutations in the SDH genes to detect phaeochromocytoma–paraganglioma syndrome is rarely done because of time and financial constraints. We investigated whether SDHB immunohistochemistry could effectively discriminate between SDH-related and non-SDH-related phaeochromocytomas and paragangliomas in large retrospective and prospective tumour series. Methods Immunohistochemistry for SDHB was done on 220 tumours. Two retrospective series of 175 phaeochromocytomas and paragangliomas with known germline mutation status for phaeochromocytoma-susceptibility or paraganglioma-susceptibility genes were investigated. Additionally, a prospective series of 45 phaeochromocytomas and paragangliomas was investigated for SDHB immunostaining followed by SDHB, SDHC, and SDHD mutation testing. Findings SDHB protein expression was absent in all 102 phaeochromocytomas and paragangliomas with an SDHB, SDHC, or SDHD mutation, but was present in all 65 paraganglionic tumours related to multiple endocrine neoplasia type 2, von Hippel–Lindau disease, and neurofibromatosis type 1. 47 (89%) of the 53 phaeochromocytomas and paragangliomas with no syndromic germline mutation showed SDHB expression. The sensitivity and specificity of the SDHB immunohistochemistry to detect the presence of an SDH mutation in the prospective series were 100% (95% CI 87–100) and 84% (60–97), respectively

Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.

PubMed

Bausch, Birke; Schiavi, Francesca; Ni, Ying; Welander, Jenny; Patocs, Attila; Ngeow, Joanne; Wellner, Ulrich; Malinoc, Angelica; Taschin, Elisa; Barbon, Giovanni; Lanza, Virginia; Söderkvist, Peter; Stenman, Adam; Larsson, Catharina; Svahn, Fredrika; Chen, Jin-Lian; Marquard, Jessica; Fraenkel, Merav; Walter, Martin A; Peczkowska, Mariola; Prejbisz, Aleksander; Jarzab, Barbara; Hasse-Lazar, Kornelia; Petersenn, Stephan; Moeller, Lars C; Meyer, Almuth; Reisch, Nicole; Trupka, Arnold; Brase, Christoph; Galiano, Matthias; Preuss, Simon F; Kwok, Pingling; Lendvai, Nikoletta; Berisha, Gani; Makay, Özer; Boedeker, Carsten C; Weryha, Georges; Racz, Karoly; Januszewicz, Andrzej; Walz, Martin K; Gimm, Oliver; Opocher, Giuseppe; Eng, Charis; Neumann, Hartmut P H

2017-09-01

and neck paraganglioma. MAX disease occurred almost exclusively in the adrenal glands with frequently bilateral tumors. Penetrance in the largest subset, SDHA carriers, was 39% at 40 years of age and is statistically different in index patients (45%) vs mutation-carrying relatives (13%; P < .001). The SDHA, TMEM127, MAX, and SDHAF2 genes may contribute to hereditary pheochromocytoma and paraganglioma. Genetic testing is recommended in patients at clinically high risk if the classic genes are mutation negative. Gene-specific prevention and/or early detection requires regular, systematic whole-body investigation.

Clinically guided genetic screening in a large cohort of italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas.

PubMed

Mannelli, Massimo; Castellano, Maurizio; Schiavi, Francesca; Filetti, Sebastiano; Giacchè, Mara; Mori, Luigi; Pignataro, Viviana; Bernini, Gianpaolo; Giachè, Valentino; Bacca, Alessandra; Biondi, Bernadette; Corona, Giovanni; Di Trapani, Giuseppe; Grossrubatscher, Erika; Reimondo, Giuseppe; Arnaldi, Giorgio; Giacchetti, Gilberta; Veglio, Franco; Loli, Paola; Colao, Annamaria; Ambrosio, Maria Rosaria; Terzolo, Massimo; Letizia, Claudio; Ercolino, Tonino; Opocher, Giuseppe

2009-05-01

The aim of the study was to define the frequency of hereditary forms and the genotype/phenotype correlations in a large cohort of Italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas. We examined 501 consecutive patients with pheochromocytomas and/or paragangliomas (secreting or nonsecreting). Complete medical and family histories, as well as the results of clinical, laboratory, and imaging studies, were recorded in a database. Patients were divided into different groups according to their family history, the presence of lesions outside adrenals/paraganglia considered syndromic for VHL disease, MEN2, and NF1, and the number and types of pheochromocytomas and/or paragangliomas. Germ-line mutations in known susceptibility genes were investigated by gene sequencing (VHL, RET, SDHB, SDHC, SDHD) or diagnosed according to phenotype (NF1). In 160 patients younger than 50 yr with a wild-type profile, multiplex ligation-dependent probe amplification assays were performed to detect genomic rearrangements. Germline mutations were detected in 32.1% of cases, but frequencies varied widely depending on the classification criteria and ranged from 100% in patients with associated syndromic lesions to 11.6% in patients with a single tumor and a negative family history. The types and number of pheochromocytomas/paragangliomas as well as age at presentation and malignancy suggest which gene should be screened first. Genomic rearrangements were found in two of 160 patients (1.2%). The frequency of the hereditary forms of pheochromocytoma/paraganglioma varies depending on the family history and the clinical presentation. A positive family history and an accurate clinical evaluation of patients are strong indicators of which genes should be screened first.

Subtotal resection for management of large jugular paragangliomas with functional lower cranial nerves.

PubMed

Wanna, George B; Sweeney, Alex D; Carlson, Matthew L; Latuska, Richard F; Rivas, Alejandro; Bennett, Marc L; Netterville, James L; Haynes, David S

2014-12-01

To evaluate tumor control following subtotal resection of advanced jugular paragangliomas in patients with functional lower cranial nerves and to investigate the utility of salvage radiotherapy for residual progressive disease. Case series with planned chart review. Tertiary academic referral center. Patients who presented with advanced jugular paragangliomas and functional lower cranial nerves were analyzed. Primary outcome measures included extent of resection, long-term tumor control, need for additional treatment, and postoperative lower cranial nerve function. Twelve patients (mean age, 46.2 years; 7 women, 58.3%) who met inclusion criteria were evaluated between 1999 and 2013. The mean postoperative residual tumor volume was 27.7% (range, 3.5%-75.0%) of the preoperative volume. When the residual tumor volume was less than 20% of the preoperative volume, no tumor growth occurred over an average of 44.6 months of follow-up (P < .01). Four tumors (33.3%) demonstrated serial growth at a mean of 23.5 months following resection, 2 of which were treated with salvage stereotactic radiotherapy providing control through the last recorded follow-up. No patient experienced permanent postoperative lower cranial neuropathy as a result of surgery. Subtotal resection of jugular paragangliomas with preservation of the lower cranial nerves is a viable management strategy. If more than 80% of the preoperative tumor volume is resected, the residual tumor seems less likely to grow. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis.

PubMed

van Nederveen, Francien H; Gaal, José; Favier, Judith; Korpershoek, Esther; Oldenburg, Rogier A; de Bruyn, Elly M C A; Sleddens, Hein F B M; Derkx, Pieter; Rivière, Julie; Dannenberg, Hilde; Petri, Bart-Jeroen; Komminoth, Paul; Pacak, Karel; Hop, Wim C J; Pollard, Patrick J; Mannelli, Massimo; Bayley, Jean-Pierre; Perren, Aurel; Niemann, Stephan; Verhofstad, Albert A; de Bruïne, Adriaan P; Maher, Eamonn R; Tissier, Frédérique; Méatchi, Tchao; Badoual, Cécile; Bertherat, Jérôme; Amar, Laurence; Alataki, Despoina; Van Marck, Eric; Ferrau, Francesco; François, Jerney; de Herder, Wouter W; Peeters, Mark-Paul F M Vrancken; van Linge, Anne; Lenders, Jacques W M; Gimenez-Roqueplo, Anne-Paule; de Krijger, Ronald R; Dinjens, Winand N M

2009-08-01

Phaeochromocytomas and paragangliomas are neuro-endocrine tumours that occur sporadically and in several hereditary tumour syndromes, including the phaeochromocytoma-paraganglioma syndrome. This syndrome is caused by germline mutations in succinate dehydrogenase B (SDHB), C (SDHC), or D (SDHD) genes. Clinically, the phaeochromocytoma-paraganglioma syndrome is often unrecognised, although 10-30% of apparently sporadic phaeochromocytomas and paragangliomas harbour germline SDH-gene mutations. Despite these figures, the screening of phaeochromocytomas and paragangliomas for mutations in the SDH genes to detect phaeochromocytoma-paraganglioma syndrome is rarely done because of time and financial constraints. We investigated whether SDHB immunohistochemistry could effectively discriminate between SDH-related and non-SDH-related phaeochromocytomas and paragangliomas in large retrospective and prospective tumour series. Immunohistochemistry for SDHB was done on 220 tumours. Two retrospective series of 175 phaeochromocytomas and paragangliomas with known germline mutation status for phaeochromocytoma-susceptibility or paraganglioma-susceptibility genes were investigated. Additionally, a prospective series of 45 phaeochromocytomas and paragangliomas was investigated for SDHB immunostaining followed by SDHB, SDHC, and SDHD mutation testing. SDHB protein expression was absent in all 102 phaeochromocytomas and paragangliomas with an SDHB, SDHC, or SDHD mutation, but was present in all 65 paraganglionic tumours related to multiple endocrine neoplasia type 2, von Hippel-Lindau disease, and neurofibromatosis type 1. 47 (89%) of the 53 phaeochromocytomas and paragangliomas with no syndromic germline mutation showed SDHB expression. The sensitivity and specificity of the SDHB immunohistochemistry to detect the presence of an SDH mutation in the prospective series were 100% (95% CI 87-100) and 84% (60-97), respectively. Phaeochromocytoma-paraganglioma syndrome can be diagnosed

The importance of pheochromocytoma case detection in patients with neurofibromatosis type 1: A case report and review of literature.

PubMed

Tate, Joshua M; Gyorffy, Janelle B; Colburn, Jeffrey A

2017-01-01

Neurofibromatosis type 1 is a complex, multi-system genetic disorder that is associated with an increased prevalence of pheochromocytoma and paraganglioma compared to the general population, 1.0%-5.7% versus 0.2%-0.6%, respectively. A delay in pheochromocytoma and paraganglioma diagnosis or undiagnosed pheochromocytoma and paraganglioma, as seen in normotensive and asymptomatic patients, may portend a significant morbidity and mortality risk due to excess catecholamine secretion. Currently, there are no generally accepted guidelines of screening for pheochromocytoma and paragangliomas in asymptomatic individuals of this population with approaches and practices varying considerably between physicians. Emerging data suggest benefit in routine pheochromocytoma and paraganglioma screening of all individuals with neurofibromatosis type 1. Herein, we present a case to highlight how routine case detection screening would have identified pheochromocytoma earlier in an active duty military member.

Spectrum and prevalence of FP/TMEM127 gene mutations in pheochromocytomas and paragangliomas.

PubMed

Yao, Li; Schiavi, Francesca; Cascon, Alberto; Qin, Yuejuan; Inglada-Pérez, Lucia; King, Elizabeth E; Toledo, Rodrigo A; Ercolino, Tonino; Rapizzi, Elena; Ricketts, Christopher J; Mori, Luigi; Giacchè, Mara; Mendola, Antonella; Taschin, Elisa; Boaretto, Francesca; Loli, Paola; Iacobone, Maurizio; Rossi, Gian-Paolo; Biondi, Bernadette; Lima-Junior, José Viana; Kater, Claudio E; Bex, Marie; Vikkula, Miikka; Grossman, Ashley B; Gruber, Stephen B; Barontini, Marta; Persu, Alexandre; Castellano, Maurizio; Toledo, Sergio P A; Maher, Eamonn R; Mannelli, Massimo; Opocher, Giuseppe; Robledo, Mercedes; Dahia, Patricia L M

2010-12-15

Pheochromocytomas and paragangliomas are genetically heterogeneous neural crest-derived neoplasms. We recently identified germline mutations of the novel transmembrane-encoding gene FP/TMEM127 in familial and sporadic pheochromocytomas consistent with a tumor suppressor effect. To examine the prevalence and spectrum of FP/TMEM127 mutations in pheochromocytomas and paragangliomas and to test the effect of mutations in vitro. We sequenced the FP/TMEM127 gene in 990 individuals with pheochromocytomas and/or paragangliomas, including 898 previously unreported cases without mutations in other susceptibility genes from 8 independent worldwide referral centers between January 2009 and June 2010. A multiplex polymerase chain reaction-based method was developed to screen for large gene deletions in 545 of these samples. Confocal microscopy of 5 transfected mutant proteins was used to determine their subcellular localization. The frequency and type of FP/TMEM127 mutation or deletion was assessed and correlated with clinical variables; the subcellular localization of 5 overexpressed mutants was compared with wild-type FP/TMEM127 protein. We identified 19 potentially pathogenic FP/TMEM127 germline mutations in 20 independent families, but no large deletions were detected. All mutation carriers had adrenal tumors, including 7 bilateral (P = 2.7 × 10(-4)) and/or with familial disease (5 of 20 samples; P = .005). The median age at disease onset in the FP/TMEM127 mutation group was similar to that of patients without a mutation (41.5 vs 45 years, respectively; P = .54). The most common presentation was that of a single benign adrenal tumor in patients older than 40 years. Malignancy was seen in 1 mutation carrier (5%). Expression of 5 novel FP/TMEM127 mutations in cell lines revealed diffuse localization of the mutant proteins in contrast with the discrete multiorganelle distribution of wild-type TMEM127. Germline mutations of FP/TMEM127 were associated with pheochromocytoma but

MAX mutations status in Swedish patients with pheochromocytoma and paraganglioma tumours.

PubMed

Crona, Joakim; Maharjan, Rajani; Delgado Verdugo, Alberto; Stålberg, Peter; Granberg, Dan; Hellman, Per; Björklund, Peyman

2014-03-01

Pheochromocytoma (PCC) and Paraganglioma are rare tumours originating from neuroendocrine cells. Up to 60% of cases have either germline or somatic mutation in one of eleven described susceptibility loci, SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, EPAS1, RET, NF1, TMEM127 and MYC associated factor-X (MAX). Recently, germline mutations in MAX were found to confer susceptibility to PCC and paraganglioma (PGL). A subsequent multicentre study found about 1% of PCCs and PGLs to have germline or somatic mutations in MAX. However, there has been no study investigating the frequency of MAX mutations in a Scandinavian cohort. We analysed tumour specimens from 63 patients with PCC and PGL treated at Uppsala University hospital, Sweden, for re-sequencing of MAX using automated Sanger sequencing. Our results show that 0% (0/63) of tumours had mutations in MAX. Allele frequencies of known single nucleotide polymorphisms rs4902359, rs45440292, rs1957948 and rs1957949 corresponded to those available in the Single Nucleotide Polymorphism Database. We conclude that MAX mutations remain unusual events and targeted genetic screening should be considered after more common genetic events have been excluded.

Hereditary paraganglioma due to the SDHD M1I mutation in a second Chinese family: a founder effect?

PubMed

Lee, Soo-Chin; Chionh, Siok-Bee; Chong, Siew-Meng; Taschner, Peter E M

2003-06-01

Hereditary paraganglioma is a rare condition that is inherited in an autosomal-dominant fashion. Four distinct loci have been associated with hereditary paraganglioma, including the SDHD, SDHC, and SDHB genes and a locus at 11q13. The SDHD, SDHC, and SDHB genes code for subunits of succinate dehydrogenase, which forms part of the mitochondrial respiratory chain. SDHD mutations are widely distributed along the gene with no apparent hot spots, although a founder effect has been described in the Dutch population. Following a prior report of the SDHD M1I mutation in an Australian Chinese family, a second Chinese family with the same mutation is reported. The proband developed bilateral head and neck paragangliomas at age 34 years and a functioning adrenal pheochromocytoma and two extra-adrenal abdominal paragangliomas 7 years later. His brother had unilateral head and neck paraganglioma at age 39 years. Given the multicentricity of the proband's tumor and the familial clustering of paragangliomas, a clinical diagnosis of hereditary paraganglioma was made, and the proband was tested for a mutation in the SDHD gene. The proband was found to be heterozygous for the SDHD MII mutation that removes the start codon, and his brother subsequently tested positive for the same mutation. The family is not related to the Australian Chinese family. The finding suggests the possibility of a founder effect in the Chinese population and warrants further investigation.

Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention

PubMed Central

Schiavi, Francesca; Ni, Ying; Welander, Jenny; Patocs, Attila; Ngeow, Joanne; Wellner, Ulrich; Malinoc, Angelica; Taschin, Elisa; Barbon, Giovanni; Lanza, Virginia; Söderkvist, Peter; Stenman, Adam; Larsson, Catharina; Svahn, Fredrika; Chen, Jin-Lian; Marquard, Jessica; Fraenkel, Merav; Walter, Martin A.; Peczkowska, Mariola; Prejbisz, Aleksander; Jarzab, Barbara; Hasse-Lazar, Kornelia; Petersenn, Stephan; Moeller, Lars C.; Meyer, Almuth; Reisch, Nicole; Trupka, Arnold; Brase, Christoph; Galiano, Matthias; Preuss, Simon F.; Kwok, Pingling; Lendvai, Nikoletta; Berisha, Gani; Makay, Özer; Boedeker, Carsten C.; Weryha, Georges; Racz, Karoly; Januszewicz, Andrzej; Walz, Martin K.; Gimm, Oliver; Opocher, Giuseppe; Eng, Charis; Neumann, Hartmut P. H.

2017-01-01

carriers (48%) and in 23 of 29 SDHA mutation carriers (79%), particularly with head and neck paraganglioma. MAX disease occurred almost exclusively in the adrenal glands with frequently bilateral tumors. Penetrance in the largest subset, SDHA carriers, was 39% at 40 years of age and is statistically different in index patients (45%) vs mutation-carrying relatives (13%; P < .001). Conclusions and Relevance The SDHA, TMEM127, MAX, and SDHAF2 genes may contribute to hereditary pheochromocytoma and paraganglioma. Genetic testing is recommended in patients at clinically high risk if the classic genes are mutation negative. Gene-specific prevention and/or early detection requires regular, systematic whole-body investigation. PMID:28384794

Pheochromocytoma and Paraganglioma Treatment (PDQ®)—Patient Version

Cancer.gov

Pheochromocytoma and paraganglioma treatment is usually surgery and drug therapy. Chemotherapy, radiation therapy, targeted therapy, ablation, and embolization can be used for disease that has spread or come back. Learn more in this expert-reviewed summary.

Soluble Endoglin (CD105) Serum Level as a Potential Marker in the Management of Head and Neck Paragangliomas.

PubMed

Litwiniuk, Małgorzata; Niemczyk, Kazimierz; Niderla-Bielińska, Justyna; Łukawska-Popieluch, Izabela; Grzela, Tomasz

2017-10-01

To assess the expression of endoglin in head and neck paragangliomas and the soluble endoglin level in serum of paraganglioma patients. Seven tumor samples of patients operated for cervical paraganglioma were assessed, as well as serum samples collected preoperatively, on days 4 and 28 postoperation. Serum level of endoglin in healthy controls was also determined. Tumor samples were subjected to immunofluorescent staining and examined with confocal microscope. The level of soluble endoglin in serum samples was examined using the immunoenzymatic assay (ELISA). Endoglin was highly expressed in all tumor samples. The level of soluble endoglin was significantly higher in paraganglioma patients compared to healthy controls and correlated with the tumor size. The serum level of s-endoglin was reduced after surgical excision of the tumor and remained stable after 4 weeks in all patients with complete resection of the tumor. Endoglin is an important factor in the pathophysiology of head and neck paragangliomas and may be a potential diagnostic and prognostic marker in these types of tumors.

[Head and neck paragangliomas. Embryological origin and anatomical characteristics: topographic distribution and vascularization pattern].

PubMed

Carretero González, José; Blanco Pérez, Pedro; Vázquez Osorio, María Teresa; Benito González, Fernando; Sañudo Tejedo, José Ramón

2009-02-01

Paragangliomas are tumors that arise in the extraadrenal paraganglia and result from migration of neural crest cells during embryonic development. Based on their anatomical distribution, innervation and microscopic structure, these tumors can be classified into interrelated families: branchiomeric paraganglia (related to the branchial clefts and arches), intravagal, aortic-sympathetic and visceral-autonomic. Head and neck paragangliomas belong mainly to the first two of these families. The present article is divided into two parts. The first part reviews the embryological origin of these tumors. Special emphasis is placed on the process of neurulation or neural tube formation, neurosegmentation (with a summary of the mechanisms involved in the initial segmentation of the neural tube and of the hindbrain and spinal medulla), and the development of the sensory placodes and secondary inductions in the cranial region. Subsequently, the neural crest is analyzed, with special attention paid to the cranial neural crest. The embryonogenesis of paragangliomas is also described. The second part describes the topographical distribution of head and neck paragangliomas according to their localization: jugulotympanic, orbit, intercarotid, subclavian and laryngeal. The embryonogenesis and most important anatomical characteristics are described for each type.

Duodenal gangliocytic paraganglioma with lymph node metastases: A case report and comparative review of 31 cases

PubMed Central

Cathcart, Sahara J; Sasson, Aaron R; Kozel, Jessica A; Oliveto, Jennifer M; Ly, Quan P

2017-01-01

Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published

Duodenal gangliocytic paraganglioma with lymph node metastases: A case report and comparative review of 31 cases.

PubMed

Cathcart, Sahara J; Sasson, Aaron R; Kozel, Jessica A; Oliveto, Jennifer M; Ly, Quan P

2017-06-16

Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published

Pheochromocytoma and Paraganglioma Treatment (PDQ®)—Health Professional Version

Cancer.gov

Pheochromocytoma and paraganglioma treatment is usually surgery, however, preoperative medical preparation is critical. Palliative care for metastatic disease may include chemotherapy, radiation therapy, targeted therapy, and other modalities. Get detailed information in this clinician summary.

Concurrent bilateral pheochromocytoma and thoracic paraganglioma during pregnancy.

PubMed

Snabboon, Thiti; Plengpanich, Wanee; Houngngam, Natnicha; Buranasupkajorn, Patinut; Plengvidhya, Nattachet; Sereepapong, Wisan; Sunthornyothin, Sarat; Shotelersuk, Vorasuk

2010-04-01

Although hypertension occurring during pregnancies is not uncommon and its prognosis is generally excellent, some of its unusual causes can lead to catastrophic consequences, especially in undiagnosed cases. Here, we report a pregnant woman who presented with hypertension in her early pregnancy. It was subsequently found to be caused by bilateral pheochromocytoma. After removal of both tumors, catecholamine levels unexpectedly and unexplainably remained elevated. At 23 weeks of gestation, the fetus was found dead in utero. After the fetal death, additional studies were performed and revealed a thoracic paraganglioma. To our knowledge, this is the first report of a case of three catecholamine-producing tumors occurring concurrently during a pregnancy. Genetic analysis helped identify this unprecedented condition; the patient harbored a heterozygous missense mutation c.482G>A in exon 3 of the VHL gene, indicating von Hippel-Lindau syndrome. Physicians who care for hypertensive pregnant patients should be aware of this condition as its diagnosis would probably lead to a better outcome.

The Genetic Basis of Pheochromocytoma and Paraganglioma: Implications for Management

PubMed Central

Shuch, Brian; Ricketts, Christopher J.; Pacak, Karol; Linehan, W. Marston

2015-01-01

Chromaffin cells are catecholamine-producing cells derived from neural crest tissue. Chromaffin tumors (ChT) are rare tumors arising from these cells and are divided into pheochromocytoma (PCC) arising from adrenal tissue and paraganglioma (PGL) arising from extra-adrenal ganglia. Previously, ∼10% were believed to be hereditary, but advances in genome sequencing has shown roughly 35% of apparently sporadic tumors have a hereditary component. In this review we describe both classic and newly discovered hereditary ChT syndromes and provide recommendations for genetic testing. In many cases the genes associated with these conditions are linked to common kidney cancer pathways familiar to urologic oncologists. PMID:24642075

Initial experience with dual-lumen balloon catheter injection for preoperative Onyx embolization of skull base paragangliomas.

PubMed

Ladner, Travis R; He, Lucy; Davis, Brandon J; Yang, George L; Wanna, George B; Mocco, J

2016-06-01

OBJECT Paragangliomas are highly vascular head and neck tumors for which preoperative embolization is often considered to facilitate resection. The authors evaluated their initial experience using a dual-lumen balloon to facilitate preoperative embolization in 5 consecutive patients who underwent preoperative transarterial Onyx embolization assisted by the Scepter dual-lumen balloon catheter between 2012 and 2014. OBJECT The authors reviewed the demographic and clinical records of 5 patients who underwent Scepter-assisted Onyx embolization of a paraganglioma followed by resection between 2012 and 2014. Descriptive statistics of clinical outcomes were assessed. RESULTS Five patients (4 with a jugular and 1 with a vagal paraganglioma) were identified. Three paragangliomas were embolized in a single session, and each of the other 2 were completed in 3 staged sessions. The mean volume of Onyx used was 14.3 ml (range 6-30 ml). Twenty-seven vessels were selectively catheterized for embolization. All patients required selective embolization via multiple vessels. Two patients required sacrifice of parent vessels (1 petrocavernous internal carotid artery and 1 vertebral artery) after successful balloon test occlusion. One patient underwent embolization with Onyx-18 alone, 2 with Onyx-34 alone, and 1 with Onyx-18 and -34. In each case, migration of Onyx was achieved within the tumor parenchyma. The mean time between embolization and resection was 3.8 days (range 1-8 days). Gross-total resection was achieved in 3 (60%) patients, and the other 2 patients had minimal residual tumor. The mean estimated blood loss during the resections was 556 ml (range 200-850 ml). The mean postoperative hematocrit level change was -17.3%. Two patients required blood transfusions. One patient, who underwent extensive tumor penetration with Onyx, developed a temporary partial cranial nerve VII palsy that resolved to House-Brackmann Grade I (out of VI) at the 6-month follow-up. One patient

Pheochromocytoma/Paraganglioma: Review of perioperative management of blood pressure and update on genetic mutations associated with pheochromocytoma

PubMed Central

Fishbein, Lauren; Orlowski, Robert; Cohen, Debbie

2015-01-01

Pheochromocytomas and paragangliomas are rare tumors with high morbidity, due to excessive catecholamine secretion, even though the majority of tumors are benign. The use of perioperative blockade regimens, together with improved surgical techniques, has greatly impacted the perioperative morbidity associated with these tumors. The old dogma of the “tumor of tens” no longer holds true. For example, at least one-third of all pheochromocytomas and paragangliomas are hereditary with mutations in one of ten well characterized susceptibility genes, and one-quarter of all tumors are malignant. This review will focus on the perioperative management of pheochromocytoma and paragangliomas and the clinical implications of the associated genetic mutations. PMID:23730992

Exercise-induced nausea and vomiting: another sign and symptom of pheochromocytoma and paraganglioma.

PubMed

King, Kathryn S; Darmani, Nissar A; Hughes, Marybeth S; Adams, Karen T; Pacak, Karel

2010-06-01

A cohort of nine patients, mostly young adults, presented with a new sign/symptom of pheochromocytoma/paraganglioma: exercise-induced nausea and vomiting. The aims of this article are to introduce this sign/symptom and offer a possible hypothesis for the observation. Following a 2000 report from a paraganglioma patient experiencing exercise-induced nausea and vomiting, we began asking patients about instances of nausea and vomiting with exercise. A total of nine patients, 4.4% of our pheochromocytoma/paraganglioma population, presented with reports of exercise-induced nausea and vomiting, initially with moderate-to-intense levels of exercise, at the first presentation of their disease. All of these patients reported a cessation of exercise-induced nausea and vomiting following the removal of their primary tumor. Two patients with metastatic disease to the lungs reported a recurrence of exercise-induced nausea and vomiting. The majority of patients studied were young adults with mean onset age of 19.4 years (range of 9-51 years) and the mean age of diagnosis being 24.1 years (range of 11-53 years). Exercise-induced nausea and vomiting should be considered a sign/symptom of pheochromocytoma/paraganglioma and should be addressed in the clinical evaluation of these patients, especially in young adults. Whether exercise-induced elevated catecholamine levels could account for the induced nausea and vomiting via activation of adrenergic receptors in the area postrema remains to be established.

Rapid-sequence MRI for long-term surveillance for paraganglioma and phaeochromocytoma in patients with succinate dehydrogenase mutations.

PubMed

Daniel, Eleni; Jones, Robert; Bull, Matthew; Newell-Price, John

2016-12-01

Patients with SDHx mutations need long-term radiological surveillance for the development of paragangliomas and phaeochromocytomas, but no longitudinal data exist. The aim of the study was to assess the performance of rapid-sequence non-contrast magnetic resonance imaging (MRI) in the long-term monitoring of patients with SDHx mutations. Retrospective study between 2005 and 2015 at a University Hospital and regional endocrine genetics referral centre. Clinical and imaging data of 47 patients with SDHx mutations (SDHB (36), SDHC (6) and SDHD (5)) who had surveillance for detection of paragangliomas by rapid-sequence non-contrast MRI (base of skull to pubic symphysis) were collected. Twelve index cases (nine SDHB, one SDHC and two SDHD) and 35 mutation-positive relatives were monitored for a mean of 6.4 years (range 3.1-10.0 years). Mean age at the end of the study: SDHB 46.9 ± 17.6 years; SDHC 42.3 ± 24.4 years; SDHD 54.9 ± 10.6 years. On excluding imaging at initial diagnosis of index cases, 42 patients underwent 116 rapid-sequence MRI scans: 83 scans were negative and 31 scans were positive for sPGL/HNPGL in 13 patients. Most patients had multiple scans (n = number of patients (number of rapid-sequence MRI scans during screening)): n = 9 (2), n = 20 (3), n = 6 (4), n = 1 (6). Nine patients (three index) were diagnosed with new paragangliomas during surveillance and non-operated tumour size was monitored in nine patients. There were two false-positive scans (1.6%). Scans were repeated every 27 ± 9 months. Biannual rapid-sequence non-contrast MRI is effective to monitor patients with SDHx mutations for detection of new tumours and monitoring of known tumours. © 2016 European Society of Endocrinology.

(131)I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: systematic review and meta-analysis.

PubMed

van Hulsteijn, L T; Niemeijer, N D; Dekkers, O M; Corssmit, E P M

2014-04-01

(131)I-MIBG therapy can be used for palliative treatment of malignant paraganglioma and phaeochromocytoma. The main objective of this study was to perform a systematic review and meta-analysis assessing the effect of (131)I-MIBG therapy on tumour volume in patients with malignant paraganglioma/phaeochromocytoma. A literature search was performed in December 2012 to identify potentially relevant studies. Main outcomes were the pooled proportions of complete response, partial response and stable disease after radionuclide therapy. A meta-analysis was performed with an exact likelihood approach using a logistic regression with a random effect at the study level. Pooled proportions with 95% confidence intervals (CI) were reported. Seventeen studies concerning a total of 243 patients with malignant paraganglioma/phaeochromocytoma were treated with (131)I-MIBG therapy. The mean follow-up ranged from 24 to 62 months. A meta-analysis of the effect of (131)I-MIBG therapy on tumour volume showed pooled proportions of complete response, partial response and stable disease of, respectively, 0·03 (95% CI: 0·06-0·15), 0·27 (95% CI: 0·19-0·37) and 0·52 (95% CI: 0·41-0·62) and for hormonal response 0·11 (95% CI: 0·05-0·22), 0·40 (95% CI: 0·28-0·53) and 0·21 (95% CI: 0·10-0·40), respectively. Separate analyses resulted in better results in hormonal response for patients with paraganglioma than for patients with phaeochromocytoma. Data on the effects of (131)I-MIBG therapy on malignant paraganglioma/phaeochromocytoma suggest that stable disease concerning tumour volume and a partial hormonal response can be achieved in over 50% and 40% of patients, respectively, treated with (131)I-MIBG therapy. It cannot be ruled out that stable disease reflects not only the effect of MIBG therapy, but also (partly) the natural course of the disease. © 2013 John Wiley & Sons Ltd.

Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline.

PubMed

Lenders, Jacques W M; Duh, Quan-Yang; Eisenhofer, Graeme; Gimenez-Roqueplo, Anne-Paule; Grebe, Stefan K G; Murad, Mohammad Hassan; Naruse, Mitsuhide; Pacak, Karel; Young, William F

2014-06-01

The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL). The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration. This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines. The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Consideration should be given to preanalytical factors leading to false-positive or false-negative results. All positive results require follow-up. Computed tomography is suggested for initial imaging, but magnetic resonance is a better option in patients with metastatic disease or when radiation exposure must be limited. (123)I-metaiodobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. We recommend consideration of genetic testing in all patients, with testing by accredited laboratories. Patients with paraganglioma should be tested for SDHx mutations, and those with metastatic disease for SDHB mutations. All patients with functional PPGLs should undergo preoperative blockade to prevent perioperative complications. Preparation should include a high-sodium diet and fluid intake to prevent postoperative hypotension. We recommend minimally invasive adrenalectomy for most pheochromocytomas with open resection for most

[Hypertension, catecholamine hypersecretion and potential for metastasis: recent progress in the pathophysiology and genetics of pheochromocytoma and paraganglioma].

PubMed

Plouin, Pierre-François; Amar, Laurence; Gimenez-Roqueplo, Anne-paule

2015-01-01

Pheochromocytomas and paragangliomas are catecholamine-secreting tumors usually associated with arterial hypertension. They can contribute to acute cardiovascular events. Ten to 15 percent of tumors are metastatic. Autosomal dominant gene alterations are present in more than a third of cases. The secretory phenotype and the risk of malignancy are driven by the presence of gene mutations, specifically in the subunits of succinate dehydrogenase. Recent advances in genomics have clinical implications for family screening, biological follow-up, prediction of the risk of recurrence, and therapeutic options in cases with malignant recurrence.

Pheochromocytoma-paraganglioma: Biochemical and genetic diagnosis.

PubMed

Cano Megías, Marta; Rodriguez Puyol, Diego; Fernández Rodríguez, Loreto; Sención Martinez, Gloria Lisette; Martínez Miguel, Patricia

Pheochromocytomas and paragangliomas are tumours derived from neural crest cells, which can be diagnosed by biochemical measurement of metanephrine and methoxytyramine. Advances in genetic research have identified many genes involved in the pathogenesis of these tumours, suggesting that up to 35-45% may have an underlying germline mutation. These genes have a singular transcriptional signature and can be grouped into 2 clusters (or groups): cluster 1 (VHL and SHDx), involved in angiogenesis and hypoxia pathways; and cluster 2 (MEN2 and NF1), linked to the kinase signalling pathway. In turn, these genes are associated with a characteristic biochemical phenotype (noradrenergic and adrenergic), and clinical features (location, biological behaviour, age of presentation, etc.) in a large number of cases. Early diagnosis of these tumours, accompanied by a correct genetic diagnosis, should eventually become a priority to enable better treatment, early detection of complications, proper screening of family members and related tumours, as well as an improvement in the overall prognosis of these patients. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

In Vivo Detection of Succinate by Magnetic Resonance Spectroscopy as a Hallmark of SDHx Mutations in Paraganglioma.

PubMed

Lussey-Lepoutre, Charlotte; Bellucci, Alexandre; Morin, Aurélie; Buffet, Alexandre; Amar, Laurence; Janin, Maxime; Ottolenghi, Chris; Zinzindohoué, Franck; Autret, Gwennhael; Burnichon, Nelly; Robidel, Estelle; Banting, Benjamin; Fontaine, Sébastien; Cuenod, Charles-André; Benit, Paule; Rustin, Pierre; Halimi, Philippe; Fournier, Laure; Gimenez-Roqueplo, Anne-Paule; Favier, Judith; Tavitian, Bertrand

2016-03-01

Germline mutations in genes encoding mitochondrial succinate dehydrogenase (SDH) are found in patients with paragangliomas, pheochromocytomas, gastrointestinal stromal tumors, and renal cancers. SDH inactivation leads to a massive accumulation of succinate, acting as an oncometabolite and which levels, assessed on surgically resected tissue are a highly specific biomarker of SDHx-mutated tumors. The aim of this study was to address the feasibility of detecting succinate in vivo by magnetic resonance spectroscopy. A pulsed proton magnetic resonance spectroscopy ((1)H-MRS) sequence was developed, optimized, and applied to image nude mice grafted with Sdhb(-/-) or wild-type chromaffin cells. The method was then applied to patients with paraganglioma carrying (n = 5) or not (n = 4) an SDHx gene mutation. Following surgery, succinate was measured using gas chromatography/mass spectrometry, and SDH protein expression was assessed by immunohistochemistry in resected tumors. A succinate peak was observed at 2.44 ppm by (1)H-MRS in all Sdhb(-/-)-derived tumors in mice and in all paragangliomas of patients carrying an SDHx gene mutation, but neither in wild-type mouse tumors nor in patients exempt of SDHx mutation. In one patient, (1)H-MRS results led to the identification of an unsuspected SDHA gene mutation. In another case, it helped define the pathogenicity of a variant of unknown significance in the SDHB gene. Detection of succinate by (1)H-MRS is a highly specific and sensitive hallmark of SDHx mutations. This noninvasive approach is a simple and robust method allowing in vivo detection of the major biomarker of SDHx-mutated tumors. ©2015 American Association for Cancer Research.

Targeted Exome Sequencing of Krebs Cycle Genes Reveals Candidate Cancer-Predisposing Mutations in Pheochromocytomas and Paragangliomas.

PubMed

Remacha, Laura; Comino-Méndez, Iñaki; Richter, Susan; Contreras, Laura; Currás-Freixes, María; Pita, Guillermo; Letón, Rocío; Galarreta, Antonio; Torres-Pérez, Rafael; Honrado, Emiliano; Jiménez, Scherezade; Maestre, Lorena; Moran, Sebastian; Esteller, Manel; Satrústegui, Jorgina; Eisenhofer, Graeme; Robledo, Mercedes; Cascón, Alberto

2017-10-15

Purpose: Mutations in Krebs cycle genes are frequently found in patients with pheochromocytomas/paragangliomas. Disruption of SDH, FH or MDH2 enzymatic activities lead to accumulation of specific metabolites, which give rise to epigenetic changes in the genome that cause a characteristic hypermethylated phenotype. Tumors showing this phenotype, but no alterations in the known predisposing genes, could harbor mutations in other Krebs cycle genes. Experimental Design: We used downregulation and methylation of RBP1, as a marker of a hypermethylation phenotype, to select eleven pheochromocytomas and paragangliomas for targeted exome sequencing of a panel of Krebs cycle-related genes. Methylation profiling, metabolite assessment and additional analyses were also performed in selected cases. Results: One of the 11 tumors was found to carry a known cancer-predisposing somatic mutation in IDH1 A variant in GOT2 , c.357A>T, found in a patient with multiple tumors, was associated with higher tumor mRNA and protein expression levels, increased GOT2 enzymatic activity in lymphoblastic cells, and altered metabolite ratios both in tumors and in GOT2 knockdown HeLa cells transfected with the variant. Array methylation-based analysis uncovered a somatic epigenetic mutation in SDHC in a patient with multiple pheochromocytomas and a gastrointestinal stromal tumor. Finally, a truncating germline IDH3B mutation was found in a patient with a single paraganglioma showing an altered α-ketoglutarate/isocitrate ratio. Conclusions: This study further attests to the relevance of the Krebs cycle in the development of PCC and PGL, and points to a potential role of other metabolic enzymes involved in metabolite exchange between mitochondria and cytosol. Clin Cancer Res; 23(20); 6315-24. ©2017 AACR . ©2017 American Association for Cancer Research.

Long Distance Endovascular Growth of Jugulotympanic Paraganglioma Evident in 68Ga-DOTATATE PET but Concealed on CT.

PubMed

Avramovic, Nemanja; Weckesser, Matthias; Velasco, Aglaé; Stenner, Markus; Noto, Benjamin

2017-02-01

A 60-year-old woman was referred to contrast-enhanced CT for evaluation of jugular vein thrombosis incidentally detected by ultrasound. Contrast-enhanced CT showed an enhanced tumor of the right skull base highly suspicious of jugulotympanic paraganglioma. However, the jugular veins showed a nearly symmetric contrast enhancement without clear evidence of thrombosis. Consecutive Ga-DOTATATE PET/CT depicted high tumor uptake, which comprised the entire internal jugular vein. Endovascular growth of paraganglioma might be missed on contrast-enhanced CT because of high vascularization of the lesion. Ga-DOTATATE PET is suited for accurate determination of tumor extent.

Update on Modern Management of Pheochromocytoma and Paraganglioma.

PubMed

Lenders, Jacques W M; Eisenhofer, Graeme

2017-06-01

Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as ⁶⁸Ga-DOTATATE (⁶⁸Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace ¹²³I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong. Copyright © 2017 Korean Endocrine Society.

Metastatic Pheochromocytoma/Paraganglioma Related to Primary Tumor Development in Childhood or Adolescence: Significant Link to SDHB Mutations

PubMed Central

King, Kathryn S.; Prodanov, Tamara; Kantorovich, Vitaly; Fojo, Tito; Hewitt, Jacqueline K.; Zacharin, Margaret; Wesley, Robert; Lodish, Maya; Raygada, Margarita; Gimenez-Roqueplo, Anne-Paule; McCormack, Shana; Eisenhofer, Graeme; Milosevic, Dragana; Kebebew, Electron; Stratakis, Constantine A.; Pacak, Karel

2011-01-01

Purpose To present data on the high rate of SDHB mutations in patients with metastatic pheochromocytoma/paraganglioma whose initial tumor presentation began in childhood or adolescence. Patients and Methods From 2000 to 2010, 263 patients with pheochromocytoma/paraganglioma were evaluated through the National Institutes of Health (NIH), Bethesda, MD. Of the 263 patients, 125 patients were found to have metastatic disease; of these 125 patients, 32 patients presented with a tumor before 20 years of age. An additional 17 patients presented with a tumor before 20 years of age but demonstrated no development of metastatic disease. Genetic testing for mutations in the VHL, MEN, and SDHB/C/D genes was performed on patients without previously identified genetic mutations. Results Of the 32 patients who presented with metastatic disease and had their primary tumor in childhood or adolescence, sequence analysis of germline DNA showed SDHB mutations in 23 patients (71.9%), SDHD mutations in three patients (9.4%), VHL mutations in two patients (6.3%), and an absence of a known mutation in four patients (12.5%). The majority of these 32 patients (78.1%) presented with primary tumors in an extra-adrenal location. Conclusion The majority of patients with metastatic pheochromocytoma/paraganglioma who presented with a primary tumor in childhood/adolescence had primary extra-adrenal tumors and harbored SDHB mutations. Except for primary tumors located in the head and neck where SDHD genetic testing is advised, we recommend that patients who present with metastatic pheochromocytoma/paraganglioma with primary tumor development in childhood or adolescence undergo SDHB genetic testing before they undergo testing for other gene mutations, unless clinical presentation or family history suggests a different mutation. PMID:21969497

Retroperitoneal composite pheochromocytoma-ganglioneuroma : a case report and review of literature

PubMed Central

2013-01-01

Abstract Composite pheochromocytoma/paraganglioma is a rare tumor with elements of pheochromocytoma/paraganglioma and neurogenic tumor. Most were located in the adrenal glands, and extra-adrenal composite pheochromocytoma is extremely rare. Only 4 cases in the retroperitoneum have been described in the online database PUBMED. Here, we report a case of retroperitoneal extra-adrenal composite pheochromocytoma and review the related literature. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1700539911908679 PMID:23587063

Unusual cardiac paraganglioma mimicking an atypical carcinoid tumor of the lung

PubMed Central

Evans, Mark; Wang, Beverly; Delrosario, J. Lawrence; Cheng, Timmy; Milliken, Jeffrey

2018-01-01

We present a case of unusual cardiac paraganglioma (PG) initially misdiagnosed as atypical carcinoid tumor of the lung and discuss key clinical and pathologic characteristics that guide surgical management of these rare chromaffin cell tumors. A 64-year-old female with persistent cough and back pain was found to have a 4 cm × 3 cm mass abutting multiple cardiopulmonary structures. A biopsy was performed at an outside institution and pathology reported “atypical neuroendocrine carcinoma, consistent with carcinoid”. The patient was transferred to our institution and pericardial resection with right pneumonectomy was performed to excise the tumor. Histology of the mass was that of PG with multiple ethanol embolizations. Immunohistochemical examination revealed that type I (chief) cells were positive for neuroendocrine markers (chromogranin A and synaptophysin), while type II (sustentacular) cells were positive for S100. There was no evidence of atypical carcinoid tumor in the lung. PG is an entity of chromaffin cell tumors that often affects the adrenal glands and carotid body. PG rarely occurs in the thoracic region, accounting for just 1–2% of all PG. Proper diagnosis of cardiac PG is challenging owing to its rare prevalence, subtle symptoms of presentation, and the neuroendocrine histopathological features it shares with atypical carcinoids. These tumors are typically benign and are best treated by surgical resection. Our report examines the approach to appropriate diagnosis of cardiac PG vs. atypical carcinoid, preoperative management, and surgical treatment by describing successful resection through thoracotomy without the use of cardiopulmonary bypass. PMID:29600100

A novel succinate dehydrogenase subunit B germline variant associated with head and neck paraganglioma in a Dutch kindred: A family-based study.

PubMed

de Vos, B; Rijken, J A; Adank, M A; Hoksbergen, A W J; Bayley, J P; Leemans, C R; Hensen, E F

2018-06-01

In the Netherlands, the majority of hereditary head and neck paragangliomas (HNPGL) are caused by germline variants in the succinate dehydrogenase genes (SDHD, SDHB, SDHAF2). Here, we evaluate a four-generation family linked to a novel SDHB gene variant with the manifestation of a HNPGL. A family-based study. The VU University Medical Center (VUmc) Amsterdam, a tertiary clinic for Otolaryngology and Head and Neck Surgery. The index patients presented with an embryonic rhabdomyosarcoma and a non-Hodgkin lymphoma. Array-based comparative genomic hybridisation (aCGH) analysis and multiplex ligation-dependent probe amplification (MLPA) revealed a novel deletion of exon 1-3 in the SDHB gene, suspected to predispose to paraganglioma (PGL)/pheochromocytoma (PHEO) syndrome type 4. Subsequently, genetic counselling and DNA testing were offered to all family members at risk. Individuals that tested positive for this novel SDHB gene variant were counselled and additional clinical evaluation was offered for the identification of HNPGL and/or PHEO. The DNA of 18 family members was tested, resulting in the identification of 10 carriers of the exon 1-3 deletion in the SDHB gene. One carrier was diagnosed with a carotid body PGL and serum catecholamine excess, which was surgically excised. Negative SDHB immunostaining of the carotid body tumour confirmed that it was caused by the SDHB variant. The remaining 9 carriers showed no evidence of PGL/PHEO. Deletion of exon 1-3 in the SDHB gene is a novel germline variant associated with the formation of hereditary HNPGL. © 2018 The Authors. Clinical Otolaryngology Published by John Wiley & Sons Ltd.

Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma.

PubMed

Giavarini, Alessandra; Chedid, Antoine; Bobrie, Guillaume; Plouin, Pierre-François; Hagège, Albert; Amar, Laurence

2013-10-01

Phaeochromocytomas and paragangliomas (PPGL) can cause acute catecholamine cardiomyopathy (ACC). We assessed the prevalence of ACC and compared the presentation of cases with and without ACC in a large series of PPGL. Single centre retrospective study. Hypertension Unit, University Hospital, Paris. 140 consecutive patients with PPGL, referred from January 2003 to September 2012. Left ventricular ejection fraction (LVEF), perioperative mortality. Fifteen patients (11%) had suffered an ACC, occurring in 14 cases before the diagnosis of PPGL. Precipitating factors were identified in 11 cases. Twelve patients presented with acute pulmonary oedema, including 10 with cardiogenic shock, requiring life support in eight cases. Seven patients (five with pulmonary oedema) presented with acute chest pain and cardiac dysfunction. Electrocardiographic abnormalities were present in 14 cases: ST segment elevation or pathological Q waves, ST segment depression, and/or diffuse T wave inversion. Six patients displayed classical (apical ballooning) or inverted (basal/mid ventricular stunning) takotsubo-like cardiomyopathy. Coronary arteries were always normal on angiography. In patients with ACC, median LVEF rose from 30% (IQR 23-33%) during ACC to 71% (50-72%) before surgery (n=11, p<0.001). Median LVEF before PPGL surgery was 65% (51-72%) and 65% (60-70%) in patients with and without a history of ACC, respectively (not significant). PPGL may present as ACC in 11% of cases, excluding patients dying from undiagnosed tumours. Left ventricular dysfunction is usually reversible before surgery. PPGL should be suspected in patients with acute heart failure without evidence of valvular or coronary artery disease.

Sympathetic Chain Schwannoma Resembling Carotid Body Tumour.

PubMed

Najeeb, Tallat; Khan, Musaddiq

2016-06-01

Schwannomas are rare, benign nerve sheath tumours of parapharyngeal space. Differential diagnosis should include salivary gland tumours, paragangliomas, neurofibromas, and metastatic lymph nodes. The tumours may arise from vagus nerve and cervical sympathetic chain (CSC). Diagnosis is usually made by imaging techniques: contrast CT, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Fine needle aspiration cytology (FNAC) is useful diagnostic procedure but poor results are seen in neurogenic tumours. Rarely, a vascular CSC schwannoma at the level of carotid arteries bifurcation may mimic carotid body tumour (CBT) on imaging techniques, especially if they are vascular, causing splaying of internal and external carotid arteries. Clinically patient was asymptomatic except for a pulsatile swelling in neck for 5 years. The presented case resembled CBTclinically, on ultrasound and on imaging techniques causing splaying of carotid arteries. FNAC was inconclusive and was always hemorrhagic. During operation, it was found to be CSC schwannoma just posterior to carotid body. CSC was sacrificed and patient developed Horner syndrome postoperatively.

A Rare Case of Periampullary Tumor

DTIC Science & Technology

2017-10-13

portion of the duodenum. They commonly present with abdominal pain, gastrointestinal bleeding, or rarely with obstructive jaundice. This is a case of an asymptomatic periampullary gangliocytic paraganglioma.

Polycythemia and paraganglioma with a novel somatic HIF2A mutation in a male.

PubMed

Toyoda, Hidemi; Hirayama, Jyunya; Sugimoto, Yuka; Uchida, Keiichi; Ohishi, Kohshi; Hirayama, Masahiro; Komada, Yoshihiro

2014-06-01

Recently, a new syndrome of paraganglioma, somatostatinoma, and polycythemia has been discovered (known as Pacak-Zhuang syndrome). This new syndrome, with somatic HIF2A gain-of-function mutations, has never been reported in male patients. We describe a male patient with Pacak-Zhuang syndrome who carries a newly discovered HIF2A mutation. Congenital polycythemias have diverse etiologies, including germline mutations in the oxygen-sensing pathway. These include von Hippel-Lindau (Chuvash polycythemia), prolyl hydroxylase domain-containing protein-2, and hypoxia-inducible factor-2α (HIF-2α). Somatic gain-of-function mutations in the gene encoding HIF-2α were reported in patients with paraganglioma and polycythemia and have been found exclusively in female patients. Through sequencing of the HIF2A using DNA from paraganglioma in 15-year-old male patient, we identified a novel mutation of HIF2A: a heterozygous C to A substitution at base 1589 in exon 12 of HIF2A. The mutation was not found in germline DNA from leukocytes. The C1589A mutations resulted in substitution of alanine 530 in the HIF-2α protein with glutamic acid. This mutation is undoubtedly associated with increased HIF-2α activity and increased protein half-life, because it affects the vicinity of the prolyl hydroxylase target residue, proline 531. To our knowledge, this is the first report describing Pacak-Zhuang syndrome with somatic gain-of-function mutation in HIF2A in a male patient. Congenital polycythemia of unknown origin should raise suspicion for the novel disorder Pacak-Zhuang syndrome, even in male patients. Copyright © 2014 by the American Academy of Pediatrics.

Genetics of pheochromocytoma and paraganglioma: new developments.

PubMed

Pigny, P; Cardot-Bauters, C

2010-03-01

Since 2000, several new susceptibility genes for hereditary pheochromocytoma or paraganglioma have been discovered. The aim of this review is to highlight how these discoveries have improved our knowledge on the mode of inheritance of these tumors and also on their molecular pathogenesis. Concerning this specific point, we will show that the different key players of tumorigenesis can converge on two pathways, the first being the hypoxia/angiogenesis pathway and the second being the control of neural crest cell development pathway. Finally, practical issues are considered; for us, it would be preferable to apply easy-to-identify clinical predictors to preselect patients eligible for molecular testing in order to improve the efficiency of these high-cost tests. Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.

Silent genetic alterations identified by targeted next-generation sequencing in pheochromocytoma/paraganglioma: A clinicopathological correlations.

PubMed

Pillai, Suja; Gopalan, Vinod; Lo, Chung Y; Liew, Victor; Smith, Robert A; Lam, Alfred King Y

2017-02-01

The goal of this pilot study was to develop a customized, cost-effective amplicon panel (Ampliseq) for target sequencing in a cohort of patients with sporadic phaeochromocytoma/paraganglioma. Phaeochromocytoma/paragangliomas from 25 patients were analysed by targeted next-generation sequencing approach using an Ion Torrent PGM instrument. Primers for 15 target genes (NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2, TMEM127, MAX, MEN1, KIF1Bβ, EPAS1, CDKN2 & PHD2) were designed using ion ampliseq designer. Ion Reporter software and Ingenuity® Variant Analysis™ software (www.ingenuity.com/variants) from Ingenuity Systems were used to analysis these results. Overall, 713 variants were identified. The variants identified from the Ion Reporter ranged from 64 to 161 per patient. Single nucleotide variants (SNV) were the most common. Further annotation with the help of Ingenuity variant analysis revealed 29 of these 713variants were deletions. Of these, six variants were non-pathogenic and four were likely to be pathogenic. The remaining 19 variants were of uncertain significance. The most frequently altered gene in the cohort was KIF1B followed by NF1. Novel KIF1B pathogenic variant c.3375+1G>A was identified. The mutation was noted in a patient with clinically confirmed neurofibromatosis. Chromosome 1 showed the presence of maximum number of variants. Use of targeted next-generation sequencing is a sensitive method for the detecting genetic changes in patients with phaeochromocytoma/paraganglioma. The precise detection of these genetic changes helps in understanding the pathogenesis of these tumours. Copyright © 2016 Elsevier Inc. All rights reserved.

Treatment for Patients With Malignant Pheochromocytomas and Paragangliomas: A Perspective From the Hallmarks of Cancer

PubMed Central

Jimenez, Camilo

2018-01-01

Malignant pheochromocytomas and paragangliomas affect a very small percentage of the general population. A substantial number of these patients have a hereditary predisposition for the disease and consequently, bear the risk of developing these tumors throughout their entire lives. It is, however, unclear why some patients with no hereditary predisposition develop these tumors, which frequently share a similar molecular phenotype with their hereditary counterparts. Both hereditary and sporadic tumors usually appear at an early age, and affected people often die before reaching their expected lifespans. Unfortunately, there is currently no systemic therapy approved for patients with this orphan disease. Therefore, pheochromocytomas and paragangliomas are very challenging malignancies. The recognition of genetic and molecular abnormalities responsible for the development of these tumors as well as the identification of effective therapies for other malignancies that share a similar pathogenesis is leading to the development of exciting clinical trials. Tyrosine kinase inhibitors, radiopharmaceutical agents, and immunotherapy are currently under evaluation in prospective clinical trials. A phase 2 clinical trial of the highly specific metaiodobenzylguanidine, iobenguane 131I, has provided impressive results; this radiopharmaceutical agent may become the first approved systemic therapy for patients with malignant pheochromocytoma and paraganglioma by the United States Food and Drug Administration. Nevertheless, systemic therapies are still not able to cure the disease. This review will discuss the development of systemic therapeutic approaches using the hallmarks of cancer as a framework. This approach will help the reader to understand where research efforts currently stand and what the future for this difficult field may be.

Diagnosing phaeochromocytoma/paraganglioma in a patient presenting with critical illness: biochemistry versus imaging.

PubMed

Amar, Laurence; Eisenhofer, Graeme

2015-09-01

Phaeochromocytomas and paragangliomas (PPGLs) are revealed by acute cardiovascular complications involving end-organ damage in up to 20% of cases, a presentation associated with particularly high risk for mortality. Among such cases, PPGLs should be considered in patients with unexplained left ventricular failure, multi-organ failure, hypertensive crises or shock. The diagnosis of PPGL commonly relies on measurements of metanephrines in plasma or urine. However, acute critical illness is usually associated with sympathoadrenal activation. Thus, levels of metanephrines in patients in an acute emergency or intensive care setting, whether treated or not with vasoactive drugs, usually cannot be used to reliably diagnose PPGL. Delays in provision of diagnostic test results, particularly when these require 24-h urine collections, may also be incompatible for any need for rapid decisions on patient management or therapeutic interventions. The acute emergency situation therefore represents one exception to the rule where imaging studies to search for a PPGL may be undertaken without biochemical evidence of a catecholamine-producing tumour. © 2015 John Wiley & Sons Ltd.

A Somatic HIF2α Mutation-Induced Multiple and Recurrent Pheochromocytoma/Paraganglioma with Polycythemia: Clinical Study with Literature Review.

PubMed

Liu, Qiuli; Wang, Yan; Tong, Dali; Liu, Gaolei; Yuan, Wenqiang; Zhang, Jun; Ye, Jin; Zhang, Yao; Yuan, Gang; Feng, Qingxing; Zhang, Dianzheng; Jiang, Jun

2017-03-01

A syndrome known as pheochromocytomas (PCC)/paragangliomas (PGL) and polycythemia resulted from gain-of-function mutation of hypoxia-inducible factor 2α (HIF2α) has been reported recently. However, clinical features of this syndrome vary from patient to patient. In our study, we described the clinical features of the patient within 15-year follow-up with a literature review. The patient presented with "red face" since childhood and was diagnosed with polycythemia and pheochromocytoma in 2000, and then, tumor was removed at his age of 27 (year 2000). However, 13 years later (2013), he was diagnosed with multiple paragangliomas. Moreover, 2 years later (2015), another two paragangaliomas were also confirmed. Genetic analysis of hereditary PCC/PGL-related genes was conducted. A somatic heterozygous missense mutation of HIF2α (c.1589C>T) was identified at exon 12, which is responsible for the elevated levels of HIF2α and erythropoietin (EPO) and subsequent development of paragangaliomas. However, this mutation was only found in the tumors from three different areas, not in the blood. So far, 13 cases of PCC/PGL with polycythemia have been reported. Among them, somatic mutations of HIF2α at exon 12 are responsible for 12 cases, and only 1 case was caused by germline mutation of HIF2α at exon 9. The HIF2α mutation-induced polycythemia with PCC/PGL is a rare syndrome with no treatment for cure. Comprehensive therapies for this disease include removal of the tumors and intermittent phlebotomies; administration of medications to control blood pressure and to prevent complications or death resulted from high concentration of red blood cell (RBC). Genetic test is strongly recommended for patients with early onset of polycythemia and multiple/recurrent PCC/PGL.

[Significance of 3-methoxytyramine urine measurement in the diagnosis of pheochromocytomas and paragangliomas: about 28 patients].

PubMed

Le Jacques, Aurélien; Abalain, Jean-Hervé; Le Saos, Fabienne; Carré, Jean-Luc

2011-01-01

Measurement of catecholamines derivatives is used to diagnose tumors such as pheochromocytomas and paragangliomas. Despite the low incidence of these diseases, their diagnosis is essential because of potentially lethal episodes of malignant hypertension related to an inappropriate secretion of catecholamines by these tumors. The catecholamines derivatives include 3-methoxytyramine, normetanephrine and metanephrine, assayed in urine or plasma. The significance of the measurement of urinary 3-methoxytyramine was addressed by analysing the records of 28 patients aged 25 to 84 years with isolated elevation of this derivative, with non-pathological urinary rates of metanephrine and normetanephrine, that might help suspect a catecholamine inappropriate secretion. In these situations, no pheochromocytoma or paraganglioma was diagnosed. This study, after discussing possible biases in the clinical care and diagnosis approach of these patients, raises the question of the relevance of this assay in the diagnostic management of these diseases.

Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma.

PubMed

Kimura, Noriko; Takayanagi, Ryoichi; Takizawa, Nae; Itagaki, Eiji; Katabami, Takayuki; Kakoi, Narihiko; Rakugi, Hiromi; Ikeda, Yukihiro; Tanabe, Akiyo; Nigawara, Takeshi; Ito, Sadayoshi; Kimura, Itaru; Naruse, Mitsuhide

2014-06-01

Phaeochromocytomas (PHEO) and paragangliomas are rare catecholamine-producing tumours. Although 10-30% of these tumours metastasise, histopathological criteria to discriminate malignant from benign tumours have not been established; therefore, reliable histopathological markers predicting metastasis are urgently required. A total of 163 tumours, including 40 metastatic tumours, collected by the Phaeochromocytoma Study Group in Japan (PHEO-J) were analysed using a system called grading system for adrenal phaeochromocytoma and paraganglioma (GAPP). The tumours were scored based on GAPP criteria as follows: histological pattern, cellularity, comedo-type necrosis, capsular/vascular invasion, Ki67 labelling index and catecholamine type. All tumours were scored from 0 to 10 points and were graded as one of the three types: well-differentiated (WD, 0-2 points), moderately differentiated (MD, 3-6 points) and poorly differentiated (PD, 7-10 points). GAPP scores of the non-metastatic and metastatic groups were 2.08±0.17 and 5.33±0.43 (mean±s.e.m., P<0.001) respectively. There was a significant negative correlation between the GAPP score and the interval until metastasis (r=-0.438, P<0.01). The mean number of years until metastasis after the initial operation was 5.5±2.6 years. The study included 111 WD, 35 MD and 17 PD types. The five-year survival of these groups was 100, 66.8 and 22.4% respectively. In addition, negative immunoreactivity for succinate dehydrogenase gene subunit B (SDHB) was observed in 13 (8%) MD or PD tumours and ten of the 13 (77%) had metastases. Our data indicate that a combination of GAPP classification and SDHB immunohistochemistry might be useful for the prediction of metastasis in these tumours.

Validation of pathological grading systems for predicting metastatic potential in pheochromocytoma and paraganglioma

PubMed Central

Koh, Jung-Min; Ahn, Seong Hee; Kim, Hyeonmok; Kim, Beom-Jun; Sung, Tae-Yon; Kim, Young Hoon; Hong, Suck Joon; Song, Dong Eun

2017-01-01

Purpose The Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP) was proposed for predicting the metastatic potential of pheochromocytoma and paraganglioma to overcome the limitations of the Pheochromocytoma of the Adrenal Scaled Score (PASS). However, to date, no study validating the GAPP has been conducted, and previous studies did not include mutations in the succinate dehydrogenase type B (SDHB) gene in the score calculation. In this retrospective cohort study, we validated the prediction ability of GAPP and assessed whether it would be improved by inclusion of the loss of SDHB immunohistochemical staining. Methods We divided the tumors into non-metastatic and metastatic groups based on the presence of synchronous or metachronous metastases. The GAPP score and PASS at the initial operation were measured. Moreover, we combined some GAPP parameters with the immunohistochemical staining of SDHB to obtain a modified GAPP (M-GAPP) score. Results Metastasis occurred in 15/72 (20.8%) patients, with a mean follow-up of 43.5 months. Loss of SDHB staining was more frequent (P = 0.044) in the metastatic group. The GAPP score (P = 0.006), PASS (P = 0.003), and M-GAPP score (P<0.001) were all higher in the metastatic group. Twelve of 40 (30.0%) moderately or poorly differentiated tumors, as defined by the GAPP score, and 12/34 (35.3%) tumors with a PASS ≥4 were metastatic. Conversely, 10/19 (52.6%) tumors with an M-GAPP score ≥3 were metastatic. The area under the curve of the M-GAPP score (0.822) was significantly higher than that of the GAPP (0.728) (P = 0.012), but similar to that of the PASS (0.753) (P = 0.411). The GAPP (P = 0.032) and M-GAPP scores (P = 0.040), but not PASS (P = 0.200), negatively correlated with metastasis-free survival. Conclusion The GAPP was validated, and M-GAPP, a combination of some GAPP parameters and loss of SDHB staining, might be useful for the prediction of the metastatic potential of pheochromocytoma and paraganglioma

Recurrence of Phaeochromocytoma and Abdominal Paraganglioma After Initial Surgical Intervention.

PubMed

Johnston, Philip C; Mullan, Karen R; Atkinson, A Brew; Eatock, Fiona C; Wallace, Helen; Gray, Moyra; Hunter, Steven J

2015-05-01

Clinical and biochemical follow up after surgery for phaeochromocytoma is essential with long term studies demonstrating recurrence frequencies between 6% and 23%. To examine the characteristics and frequency of tumour recurrence in a regional endocrine referral centre, in patients with surgical resection of phaeochromocytoma (P) and abdominal paraganglioma (AP). We identified a cohort of 52 consecutive patients who attended our Regional Endocrinology & Diabetes Centre and retrospectively reviewed their clinical, biochemical and radiological data (between 2002 and 2013). After confirmation of early post-operative remission by negative biochemical testing, tumour recurrence was defined by demonstration of catecholamine excess with confirmatory imaging. Phaeochromocytoma was confirmed histologically in all cases (43:P, 9:AP, mean-age:53 years). Open adrenalectomy was performed in 20 cases and laparoscopically in 32. Hereditary phaeochromocytoma was confirmed by genetic analysis in 12 (23%) patients. Median follow up time from initial surgery was 47 months, (range: 12 - 296 months), 49 patients had no evidence of tumour recurrence at latest follow-up. Three patients (6%) demonstrated tumour development, one in a patient with VHL which occurred in a contralateral adrenal gland, one sporadic case had local recurrence, and an adrenal tumour occurred in a patient with a SDHB gene mutation who had a previous bladder tumour. After initial surgery, the tumours occurred at 8.6, 12.0 and 17.7 years respectively. In this study tumour development occurred in 6% of patients. Although tumour rates were low, careful and sustained clinical and biochemical follow up is advocated, as new tumour development or recurrence may occur long after the initial surgery is performed.

Pheochromocytoma and Paraganglioma in Cyanotic Congenital Heart Disease

PubMed Central

Moko, Lilamarie E.; Ginns, Jonathan; Rosenbaum, Marlon; Greutmann, Matthias; Aboulhosn, Jamil; Hageman, Abbie; Kim, Yuli; Deng, Lisa X.; Grewal, Jasmine; Zaidi, Ali N.; Almansoori, Ghadeera; Oechslin, Erwin; Earing, Michael; Landzberg, Michael J.; Singh, Michael N.; Wu, Fred

2015-01-01

Context: Aberrant cellular oxygen sensing is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). Objective: The objective of the study was to test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD) increases the risk for PHEO-PGL. Design/Setting/Participants: We investigated the association between CCHD and PHEO-PGL with two complementary studies: study 1) an international consortium was established to identify congenital heart disease (CHD) patients with a PHEO-PGL diagnosis confirmed by pathology or biochemistry and imaging; study 2) the 2000–2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with noncyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. Results: In study 1, we identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20 years (range 1–57 y). Cases were young at diagnosis (median 31.5 y, range 15–57 y) and 7 of 18 had multiple tumors (two bilateral PHEO; six multifocal or recurrent PGL), whereas 11 had single tumors (seven PHEO; four PGL). PGLs were abdominal (13 of 17) or head/neck (4 of 17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes but without clinical signs of such syndromes. In study 2, hospitalized CCHD patients had an increased likelihood of PHEO-PGL (adjusted odds ratio 6.0, 95% confidence interval 2.6–13.7, P < .0001) compared with those without CHD; patients with noncyanotic CHD had no increased risk (odds ratio 0.9, P = .48). Conclusions: There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases coassociate due to hypoxic stress, common

Is the c.3G>C mutation in the succinate dehydrogenase subunit D (SDHD) gene due to a founder effect in Chinese head and neck paraganglioma patients?

PubMed

Zha, Yang; Chen, Xing-ming; Lam, Ching-wan; Lee, Soo-chin; Tong, Sui-fan; Gao, Zhi-qiang

2011-08-01

Three Chinese patients with head and neck paragangliomas have been reported to carry the c.3G>C mutation in the succinate dehydrogenase subunit D (SDHD) gene. In addition, in our hospital, two further patients were identified who have the same mutation. It is unclear whether the c.3G>C mutation in Chinese patients is a recurrent mutation or if it is due to a founder effect. We conducted haplotype analysis on these patients to answer this question. Individual case-control study. Germ-line mutations were confirmed in the patients and their families examined in this study using direct sequencing. We also constructed and analyzed haplotypes in four Chinese families. Genotype frequencies were compared to the control group. Three of four families shared the same haplotype, which rarely occurred in the control group. The last family shared a very short area on the physical map with the other three families. There is a founder effect in Chinese head and neck paraganglioma patients carrying the SDHD c.3G>C mutation. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.

Novel germline SDHD deletion associated with an unusual sympathetic head and neck paraganglioma.

PubMed

Cadiñanos, Juan; Llorente, José L; de la Rosa, Jorge; Villameytide, José A; Illán, Rafael; Durán, Noelia S; Murias, Eduardo; Cabanillas, Rubén

2011-08-01

Paragangliomas (PGLs) are rare tumors arising either from sympathetic or parasympathetic-associated chromaffin tissue. PGLs can occur either sporadically or as part of a hereditary syndrome. Sympathetic head and neck PGLs are extremely rare tumors and only a few cases have been reported to date. We report the pedigree of a patient with a head and neck PGL arising from the right sympathetic trunk. SDHD mutation analysis was performed using standard sequencing, multiplex ligation-dependent probe amplification, chromosome 11-specific comparative genome hybridization, and long-range/short-range polymerase chain reaction (PCR) approaches. A previously unreported chromosome 11q deletion encompassing 5 annotated genes (SDHD, DLAT, PIH1D2, C11Orf57, and TIMM8B) was detected in the proband. PGL families considered "mutation-negative" may be attributable to large gene deletions not detectable by standard sequencing methods. Therefore, deletion analysis should be offered to families or individuals at risk for hereditary PGLs. Copyright © 2010 Wiley Periodicals, Inc.

The clinical genetics of phaeochromocytoma and paraganglioma.

PubMed

Kavinga Gunawardane, P T; Grossman, Ashley

2017-10-01

Phaeochromocytoma and paraganglioma are rare catecholamine-producing tumours, recognised to have one of the richest hereditary backgrounds of all neoplasms, with germline mutations seen in approximately 30% of patients. They can be a part of genetic syndromes such as MEN 2 or Neurofibromatosis type 1, or can be found as apparently sporadic tumours. Germline mutations are almost always found in syndromic patients. Nonetheless, apparently sporadic phaeochromocytoma too show high germline mutation rates. Early detection of a genetic mutation can lead to early diagnosis of further tumours via surveillance, early treatment and better prognosis. Apart from this, the genetic profile has important relevance for tumour location and biochemical profile, and can be a useful predictor of future tumour behaviour. It also enables family screening and surveillance. Moreover, recent studies have demonstrated significant driver somatic mutations in up to 75% of all tumours. Arch Endocrinol Metab. 2017;61(5):490-500.

Recurrence of Phaeochromocytoma and Abdominal Paraganglioma After Initial Surgical Intervention

PubMed Central

Mullan, Karen R; Atkinson, A Brew; Eatock, Fiona C; Wallace, Helen; Gray, Moyra; Hunter, Steven J

2015-01-01

Background Clinical and biochemical follow up after surgery for phaeochromocytoma is essential with long term studies demonstrating recurrence frequencies between 6% and 23%. Aim To examine the characteristics and frequency of tumour recurrence in a regional endocrine referral centre, in patients with surgical resection of phaeochromocytoma (P) and abdominal paraganglioma (AP). Methods We identified a cohort of 52 consecutive patients who attended our Regional Endocrinology & Diabetes Centre and retrospectively reviewed their clinical, biochemical and radiological data (between 2002 and 2013). After confirmation of early post-operative remission by negative biochemical testing, tumour recurrence was defined by demonstration of catecholamine excess with confirmatory imaging. Results Phaeochromocytoma was confirmed histologically in all cases (43:P, 9:AP, mean-age:53years). Open adrenalectomy was performed in 20 cases and laparoscopically in 32. Hereditary phaeochromocytoma was confirmed by genetic analysis in 12 (23%) patients. Median follow up time from initial surgery was 47 months, (range: 12 - 296 months), 49 patients had no evidence of tumour recurrence at latest follow-up. Three patients (6%) demonstrated tumour development, one in a patient with VHL which occurred in a contralateral adrenal gland, one sporadic case had local recurrence, and an adrenal tumour occurred in a patient with a SDHB gene mutation who had a previous bladder tumour. After initial surgery, the tumours occurred at 8.6, 12.0 and 17.7 years respectively. Conclusion In this study tumour development occurred in 6% of patients. Although tumour rates were low, careful and sustained clinical and biochemical follow up is advocated, as new tumour development or recurrence may occur long after the initial surgery is performed. PMID:26170485

[Interpretation of ultrasound findings in otorhinolaryngology. Salivary glands, paraganglioma, angioma, esophagus, hypopharynx, extra cranial vessels and temporomandibular joint].

PubMed

Bozzato, A

2015-06-01

The second part of this continuing medical education article focuses on sonographic assessment of the salivary glands, cervical paraganglioma, angioma, esophagus, extra cranial blood vessels and the temporomandibular joint. The currently available minimally invasive therapeutic options (e. g. sialendoscopy, lithotripsy, therapeutic duct lavage and extracapsular dissection) for salivary gland disease presuppose a precise imaging modality. Modern ultrasound is able to meet this challenge, making additional imaging a rare necessity. Regions of the neck with a difficult topography (esophagus and hypopharynx) can often be successfully portrayed sonographically. Furthermore, ultrasound enables functional evaluation of swallowing in the cervical parts of the esophagus in dysphagia patients. In addition to the branchial cleft anomalies and lymph nodes discussed in part 1, paraganglioma, angiomatosis and neurogenic tumors are important differential diagnoses of solid lesions of the neck. Finally, venous and arterial alterations of the extracranial vessels of the neck relevant to clinical routine are depicted, as are pathological conditions of the temporomandibular joint relevant to the otorhinolaryngologist.

[Analysis of laboratory data of 155 patients with pheochromocytoma-paraganglioma syndrome diagnosed during the past 20 years].

PubMed

Balog, Beatrice; Tőke, Judit; Róna, Kálmán; Szücs, Nikolette; Igaz, Péter; Pusztai, Péter; Sármán, Beatrix; Gláz, Edit; Kiss, Róbert; Patócs, Attila; Rácz, Károly; Tóth, Miklós

2015-04-19

Laboratory diagnosis of pheochromocytoma-paraganglioma syndrome has been markedly improved during the past two decades. Retrospective assessment of diagnostic utility of urinary catecholamines and their metabolites as well as serum chromogranin A in 155 patients diagnosed at the 2nd Department of Medicine, Semmelweis University. Urinary catecholamines and metabolites were measured using high-performance liquid chromatography with electrochemical detection in 155 patients with pheochromocytoma-paraganglioma (of whom 28.4% had hereditary background) and in 170 non-pheochromocytoma patients used as controls. Serum chromogranin A was measured by immunoradiometry. Sensitivity (93.2%) and specificity (87.0%) of urinary fractionated metanephrines were higher than those of urinary catecholamines (90.9% vs. 85.7%, respectively) and serum chromogranin A (88.7% and 77.5%, respectively). Urinary normetanephrine and serum chromogranin A correlated positively with tumor size (r = 0.552, p<0.0001 and r = 0.618, p<0.0001, respectively). These data confirm the diagnostic utility of urinary catecholamines and their metabolites. Urinary normetanephrine and serum chromogranin A may help to estimate tumour mass and probably tumour progression.

Paragangliomas: presentation and management by radiotherapy at the Prince of Wales Hospital.

PubMed

Smee, Robert I; Jayasekara, Jayana; Williams, Janet R; Hanna, Claire

2015-04-01

Paragangliomas are commonly treated with surgery, while radiotherapy is reserved for those that are inoperable or have relapsed. However, this retrospective study aims to determine whether radiotherapy is a viable initial treatment for paragangliomas. Of 73 tumours researched, 44 were diagnosed and treated from January 1967 to December 2012 at the Radiation Oncology Department at the Prince of Wales Hospital and thus were eligible for analysis. Median follow-up time was 3.5 years with a range of 1 to 40 years. Thirty-four tumours were treated with radiotherapy only, and 10 tumours were treated with both surgical resection and radiotherapy. Local control and cause-specific survival were the primary end points measured. Five-year local control rate for the population of 44 lesions was 89%; it was 100% in the group treated by radiotherapy alone, but only 50% in the group treated by surgery followed by radiotherapy, with radiation used for salvage. The difference in control rates between these two subset groups was found to be statistically significant (P < 0.001). Cause-specific survival rates for this eligible population at 5 and 10 years were 98% and 90%, respectively. After initial radiotherapy, 4 patients had improved cranial nerve function, there was clinical improvement in tinnitus, and one new cranial nerve deficit developed where a high dose was used. Radiotherapy has high local control rates and few complications. The local control and complication rates compare favourably to surgery. © 2014 The Royal Australian and New Zealand College of Radiologists.

Body packing and intra-vaginal body pushing of cocaine: A case report.

PubMed

Wankhade, Vishwajit Kishor; Chikhalkar, B G

2018-03-01

Drug trafficking is an international problem. The prevalence of drug trafficking and newer concealing methods has been ever increasing. Body packing is described as using the abdominal or pelvic cavity for concealing illegal drugs. Body pushers smuggle illicit drugs by inserting them into rectum or vagina. These cases are either presented to the emergency departments as Body Packer Syndrome or as asymptomatic cases for observation, detained for alleged possession of contraband substances. We report a unique case of an asymptomatic white female who was detained at Mumbai International Airport under suspicion and brought to hospital for observation. X ray and CT scan examination revealed 7 wrapped packets in gastrointestinal track and 1 large packet in vagina. A case of female body packer using multiple modalities of concealment especially in vagina is rare in India so it is becomes imperative to present this case in the light of body packing and body pushing of contraband substances. Copyright © 2017 Elsevier B.V. All rights reserved.

Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome.

PubMed

Därr, Roland; Nambuba, Joan; Del Rivero, Jaydira; Janssen, Ingo; Merino, Maria; Todorovic, Milena; Balint, Bela; Jochmanova, Ivana; Prchal, Josef T; Lechan, Ronald M; Tischler, Arthur S; Popovic, Vera; Miljic, Dragana; Adams, Karen T; Prall, F Ryan; Ling, Alexander; Golomb, Meredith R; Ferguson, Michael; Nilubol, Naris; Chen, Clara C; Chew, Emily; Taïeb, David; Stratakis, Constantine A; Fojo, Tito; Yang, Chunzhang; Kebebew, Electron; Zhuang, Zhengping; Pacak, Karel

2016-12-01

Worldwide, the syndromes of paraganglioma (PGL), somatostatinoma (SOM) and early childhood polycythemia are described in only a few patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A). This study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings into perspective these experiences with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11-46). Two were found to have HIF2A somatic mosaicism. No relatives were affected. All patients were diagnosed with polycythemia before age 8 and before PGL/SOM developed. PGLs were found at a median age of 17 years (range 8-38) and SOMs at 29 years (range 22-38). PGLs were multiple, recurrent and metastatic in 100, 100 and 29% of all cases, and SOMs in 40, 40 and 60%, respectively. All PGLs were primarily norepinephrine-producing. All patients had abnormal ophthalmologic findings and those with SOMs had gallbladder disease. Computed tomography (CT) and magnetic resonance imaging revealed cystic lesions at multiple sites and hemangiomas in 4 patients (57%), previously thought to be pathognomonic for von Hippel-Lindau disease. The most accurate radiopharmaceutical to detect PGL appeared to be [ 18 F]-fluorodihydroxyphenylalanine ([ 18 F]-FDOPA). Therefore, [ 18 F]-FDOPA PET/CT, not [ 68 Ga]-(DOTA)-[Tyr3]-octreotate ([ 68 Ga]-DOTATATE) PET/CT is recommended for tumor localization and aftercare in this syndrome. The long-term prognosis of the syndrome is unknown. However, to date no deaths occurred after 6 years follow-up. Physicians should be aware of this unique syndrome and its diagnostic and therapeutic challenges. © 2016 Society for Endocrinology.

Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features.

PubMed

Mazza, Alberto; Armigliato, Michela; Marzola, Maria Cristina; Schiavon, Laura; Montemurro, Domenico; Vescovo, Giorgio; Zuin, Marco; Chondrogiannis, Sotirios; Ravenni, Roberta; Opocher, Giuseppe; Colletti, Patrick M; Rubello, Domenico

2014-04-01

Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. Although are unusual cause of hypertension (HT) accounting for at most 0.1-0.2 % of cases, they may lead to severe and potentially lethal hypertensive crisis due to the effects of the released catecholamines. However, both PH and PG may be asymptomatic as ~30 % of subjects are normotensive or have orthostatic hypotension and in these cases the 24 h ambulatory blood pressure (BP) monitoring is an important toll to diagnose and treat HT. HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective α1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a β-adrenergic blockade (i.e., propranolol, atenolol). This latter should never be started first because blockade of vasodilatory peripheral β-adrenergic receptors with unopposed α-adrenergic receptor stimulation can lead to a further elevation of BP. Although labetalol is traditionally considered the ideal agent due to its α- and β-adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.

Preoperative alpha-blockade in phaeochromocytoma and paraganglioma: is it always necessary?

PubMed

Isaacs, Michelle; Lee, Paul

2017-03-01

Resection of phaeochromocytoma and paraganglioma (PPGL) is traditionally preceded by alpha-blockade to prevent complications of haemodynamic instability intraoperatively. While there is general agreement on preoperative alpha-blockade for classic PPGLs presenting with hypertension, it is less clear whether alpha-blockade is necessary in predominantly dopamine-secreting tumours, normotensive PPGLs, as well as tumours that appear to be biochemically 'silent'. Preoperative management of these 'atypical' PPGLs is challenging and the treatment approach must be individualized, carefully weighing the risk of intraoperative hypertension against the possibility of orthostatic and prolonged postoperative hypotension. Consideration of antihypertensive medication pharmacology in the light of catecholamine physiology and PPGL secretory profile will facilitate the formulation of individualized preoperative preparatory strategies. © 2016 John Wiley & Sons Ltd.

Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome

PubMed Central

Därr, Roland; Nambuba, Joan; Del Rivero, Jaydira; Janssen, Ingo; Merino, Maria; Todorovic, Milena; Balint, Bela; Jochmanova, Ivana; Prchal, Josef T.; Lechan, Ronald M.; Tischler, Arthur S.; Popovic, Vera; Miljic, Dragana; Adams, Karen T.; Prall, F. Ryan; Ling, Alexander; Golomb, Meredith R.; Ferguson, Michael; Nilubol, Naris; Chen, Clara C.; Chew, Emily; Taïeb, David; Stratakis, Constantine A.; Fojo, Tito; Yang, Chunzhang; Kebebew, Electron; Zhuang, Zhengping; Pacak, Karel

2016-01-01

The syndrome of paraganglioma (PGL), somatostatinoma (SOM), and early childhood polycythemia in patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A) gene is described in only a few patients worldwide. The present study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings these experiences into perspective with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11–46). Two were found to have HIF2A somatic mosaicism. No relatives were affected. All patients were diagnosed with secondary polycythemia before age 8 and before PGL/SOM developed. PGLs were found at a median age of 17 years (range 8–38) and SOMs at 29 years (range 22–38). PGLs were multiple, recurrent, and metastatic in 100%, 100%, and 29% of all cases, and SOMs in 40%, 40%, and 60%, respectively. All PGLs were primarily norepinephrine producing. All patients had abnormal ophthalmologic findings and those with SOMs had gallbladder disease. Computed tomography (CT) and magnetic resonance imaging revealed cystic lesions at multiple sites and hemangiomas in 4 patients (57%), previously thought to be pathognomonic for von Hippel-Lindau disease. The most accurate radiopharmaceutical to detect PGL appeared to be [18F]-fluorodihydroxyphenylalanine ([18F]-FDOPA). Therefore, [18F]-FDOPA PET/CT, not [68Ga]-(DOTA)-[Tyr3]-octreotate ([68Ga]-DOTATATE) PET/CT is recommended for tumor localization and aftercare in this syndrome. The long-term prognosis of the syndrome is unknown. However, to date no deaths occurred after 6 years follow-up. Physicians should be aware of this unique syndrome and its diagnostic and therapeutic challenges. PMID:27679736

68Ga-PSMA-HBED-CC-Avid Synchronous Urinary Bladder Paraganglioma in a Patient With Metastatic Prostate Carcinoma.

PubMed

Parihar, Ashwin Singh; Vadi, Shelvin Kumar; Mittal, Bhagwant Rai; Kumar, Rajender; Bal, Amanjit; Singh, Shrawan Kumar

2018-06-19

Ga-PSMA-HBED-CC PET/CT has proven to be a useful modality in patients with prostate carcinoma, especially in those with suspected recurrence and in detection of locoregional and distant metastases. However, with expanding use of this tracer, several recent reports of in vivo expression of PSMA in nonprostatic benign and malignant entities have been published. We report a patient with PSMA-avid synchronous urinary bladder paraganglioma and metastatic prostate carcinoma.

Germ-line PHD1 and PHD2 mutations detected in patients with pheochromocytoma/paraganglioma-polycythemia.

PubMed

Yang, Chunzhang; Zhuang, Zhengping; Fliedner, Stephanie M J; Shankavaram, Uma; Sun, Michael G; Bullova, Petra; Zhu, Roland; Elkahloun, Abdel G; Kourlas, Peter J; Merino, Maria; Kebebew, Electron; Pacak, Karel

2015-01-01

We have investigated genetic/pathogenetic factors associated with a new clinical entity in patients presenting with pheochromocytoma/paraganglioma (PHEO/PGL) and polycythemia. Two patients without hypoxia-inducible factor 2α (HIF2A) mutations, who presented with similar clinical manifestations, were analyzed for other gene mutations, including prolyl hydroxylase (PHD) mutations. We have found for the first time a germ-line mutation in PHD1 in one patient and a novel germ-line PHD2 mutation in a second patient. Both mutants exhibited reduced protein stability with substantial quantitative protein loss and thus compromised catalytic activities. Due to the unique association of patients' polycythemia with borderline or mildly elevated erythropoietin (EPO) levels, we also performed an in vitro sensitivity assay of erythroid progenitors to EPO and for EPO receptor (EPOR) expression. The results show inappropriate hypersensitivity of erythroid progenitors to EPO in these patients, indicating increased EPOR expression/activity. In addition, the present study indicates that HIF dysregulation due to PHD mutations plays an important role in the pathogenesis of these tumors and associated polycythemia. The PHD1 mutation appears to be a new member contributing to the genetic landscape of this novel clinical entity. Our results support the existence of a specific PHD1- and PHD2-associated PHEO/PGL-polycythemia disorder. • A novel germ-l i n e PHD1 mutation causing heochromocytoma/paraganglioma and polycythemia. • Increased EPOR activity and inappropriate hypersensitivity of erythroid progenitors to EPO.

Imaging work-up for screening of paraganglioma and pheochromocytoma in SDHx mutation carriers: a multicenter prospective study from the PGL.EVA Investigators.

PubMed

Gimenez-Roqueplo, Anne-Paule; Caumont-Prim, Aurore; Houzard, Claire; Hignette, Chantal; Hernigou, Anne; Halimi, Philippe; Niccoli, Patricia; Leboulleux, Sophie; Amar, Laurence; Borson-Chazot, Françoise; Cardot-Bauters, Catherine; Delemer, Brigitte; Chabolle, Frédéric; Coupier, Isabelle; Libé, Rossella; Peitzsch, Mirko; Peyrard, Séverine; Tenenbaum, Florence; Plouin, Pierre-François; Chatellier, Gilles; Rohmer, Vincent

2013-01-01

Recommendations have not been established concerning imaging to screen SDHx mutation carriers for paraganglioma and pheochromocytoma. Our objective was to compare the performance of gadolinium-enhanced magnetic resonance angiography, contrast-enhanced computed tomography, and [(123)I]metaiodo-benzylguanidine and somatostatin receptor scintigraphies for detecting head and neck and thoracic-abdominal-pelvic paragangliomas in SDHx mutation carriers. We conducted a prospective, multicenter study from June 2005 to December 2009 at 23 French medical centers. A total of 238 index cases or relatives carrying mutations in SDHD, SDHB, or SDHC genes were included. Images obtained by each technique were analyzed blind, without knowledge of results from other tests, first in each local center and then centrally. We evaluated sensitivity, specificity, and likelihood ratios for individual and combinations of tests, the gold standard being the consensus of an expert committee. Two hundred two tumors were diagnosed in 96 subjects. At local assessment, the sensitivity of anatomical imaging for detecting all tumors was higher (85.7%) than that of both scintigraphic techniques (42.7% for [(123)I]metaiodo-benzylguanidine and 69.5% for somatostatin receptor scintigraphy), except for thoracic localizations where somatostatin receptor scintigraphy was more sensitive (61.5 vs. 46.2% for anatomical imaging and 30.8% for [(123)I]metaiodo-benzylguanidine scintigraphy). The best diagnostic performance during local assessment was obtained by combining anatomical imaging tests and somatostatin receptor scintigraphy (sensitivity 91.7%). Central assessment significantly increased the sensitivity (98.6%) of tests in combination. In routine practice, the imaging work-up for screening SDHx mutation carriers should include thoraco-abdomino-pelvic computed tomography, head and neck magnetic angiography, and somatostatin receptor scintigraphy. Expert centralized image assessment is recommended.

Pheochromocytoma and Paraganglioma—Health Professional Version

Cancer.gov

Pheochromocytomas and extra-adrenal paragangliomas are rare tumors arising from neural crest tissue that develops into sympathetic and parasympathetic paraganglia throughout the body. Find evidence-based information on pheochromocytoma and paraganglioma treatment, research, and genetics.

Clinical progression and metachronous paragangliomas in a large cohort of SDHD germline variant carriers.

PubMed

Heesterman, Berdine L; de Pont, Lisa M H; van der Mey, Andel Gl; Bayley, Jean-Pierre; Corssmit, Eleonora Pm; Hes, Frederik J; Verbist, Berit M; van Benthem, Peter Paul G; Jansen, Jeroen C

2018-05-18

Although it is well established that paternally transmitted germline variants in SDHD are associated with multifocal paragangliomas and lifelong follow-up is generally advised, the risk of metachronous lesions is presently unknown. In a large Dutch cohort of SDHD variant carriers, we studied the development of new paragangliomas, and the evolution of symptoms and cranial nerve impairment. Recurrent event analysis and the Kaplan-Meier product limit estimator were used to study the risk of new lesions. The relation between several predictors and development of new symptoms was assessed using logistic regression. Of the 222 SDHD variant carriers included, 65% presented with symptoms and 11% with cranial nerve dysfunction. Over a median period of 8 years, 42% reported new symptoms, and new cranial nerve impairment was observed in 11% of subjects. The estimated fraction of subjects that developed new HNPGL increased to 73% (95% CI: 52-85%) after 22 years of follow-up. Males were more likely to develop new HNPGL compared to females (HR: 1.63, 95% CI: 1.10-2.40), as were subjects that presented with symptoms, compared to subjects that were asymptomatic at baseline (HR: 1.61, 95% CI: 1.01-2.55). In addition, the risk of new lesions decreased with number of HNPGL present at first diagnosis (HR: 0.68 and 95% CI: 0.56-0.82). Carriers of a paternally inherited SDHD variant face a considerable risk for new HNPGL. In addition, nearly 50% of subjects reported new symptoms. However, new cranial nerve deficits were observed in only 11%, which is less than reported in surgical series. These risks should be taken into account when considering treatment strategies and counseling.

Pheochromocytoma/Paraganglioma: A Poster Child for Cancer Metabolism.

PubMed

Tevosian, Sergei G; Ghayee, Hans K

2018-05-01

Pheochromocytomas (PCCs) are tumors that are derived from the chromaffin cells of the adrenal medulla. Extra-adrenal PCCs called paragangliomas (PGLs) are derived from the sympathetic and parasympathetic chain ganglia. PCCs secrete catecholamines, which cause hypertension and have adverse cardiovascular consequences as a result of catecholamine excess. PGLs may or may not produce catecholamines depending on their genetic type and anatomical location. The most worrisome aspect of these tumors is their ability to become aggressive and metastasize; there are no known cures for metastasized PGLs. Original articles and reviews indexed in PubMed were identified by querying with specific PCC/PGL- and Krebs cycle pathway-related terms. Additional references were selected through the in-depth analysis of the relevant publications. We primarily discuss Krebs cycle mutations that can be instrumental in helping investigators identify key biological pathways and molecules that may serve as biomarkers of or treatment targets for PCC/PGL. The mainstay of treatment of patients with PCC/PGLs is surgical. However, the tide may be turning with the discovery of new genes associated with PCC/PGLs that may shed light on oncometabolites used by these tumors.

Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas.

PubMed

Bacciu, Andrea; Medina, Marimar; Ait Mimoune, Hassen; D'Orazio, Flavia; Pasanisi, Enrico; Peretti, Giorgio; Sanna, Mario

2015-02-01

The aim of this study was to report the postoperative lower cranial nerves (LCNs) function in patients undergoing surgery for tympanojugular paraganglioma (TJP) and to evaluate risk factors for postoperative LCN dysfunction. A retrospective case review of 122 patients having Fisch class C or D TJP, surgically treated from 1988 to 2012, was performed. The follow-up of the series ranged from 12 to 156 months (mean, 39.4 ± 32.6 months). The infratemporal type A approach was the most common surgical procedure. Gross total tumor removal was achieved in 86% of cases. Seventy-two percent of the 54 patients with preoperative LCN deficit had intracranial tumor extension. Intraoperatively, LCNs had to be sacrificed in 63 cases (51.6%) due to tumor infiltration. Sixty-six patients (54.09%) developed a new deficit of one or more of the LCNs. Of those patients who developed new LCN deficits, 23 of them had intradural extension. Postoperative follow-up of at least 1 year showed that the LCN most commonly affected was the CN IX (50%). Logistic regression analysis showed that intracranial transdural tumor extension was correlated with the higher risk of LCN sacrifice (p < 0.05). Despite the advances in skull base surgery, new postoperative LCN deficits still represent a challenge. The morbidity associated with resection of the LCNs is dependent on the tumor's size and intradural tumor extension. Though no recovery of LCN deficits may be expected, on long-term follow-up, patients usually compensate well for their LCNs loss.

EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma

PubMed Central

Timmers, Henri J.; Hindié, Elif; Guillet, Benjamin A.; Neumann, Hartmut P.; Walz, Martin K.; Opocher, Giuseppe; de Herder, Wouter W.; Boedeker, Carsten C.; de Krijger, Ronald R.; Chiti, Arturo; Al-Nahhas, Adil; Pacak, Karel

2016-01-01

Purpose Radionuclide imaging of phaeochromocytomas (PCCs) and paragangliomas (PGLs) involves various functional imaging techniques and approaches for accurate diagnosis, staging and tumour characterization. The purpose of the present guidelines is to assist nuclear medicine practitioners in performing, interpreting and reporting the results of the currently available SPECT and PET imaging approaches. These guidelines are intended to present information specifically adapted to European practice. Methods Guidelines from related fields, issued by the European Association of Nuclear Medicine and the Society of Nuclear Medicine, were taken into consideration and are partially integrated within this text. The same was applied to the relevant literature, and the final result was discussed with leading experts involved in the management of patients with PCC/PGL. The information provided should be viewed in the context of local conditions, laws and regulations. Conclusion Although several radionuclide imaging modalities are considered herein, considerable focus is given to PET imaging which offers high sensitivity targeted molecular imaging approaches. PMID:22926712

Immunohistochemical profile of neurotrophins and MIB-1 in jugulotympanic paragangliomas: prognostic value and review of the literature.

PubMed

Artico, M; De Vincentiis, M; Ionta, B; Bianchi, E; Bosco, S; Onteleone, M; Fumagalli, L; Magliulo, G

2012-01-01

Jugulo-tympanic paragangliomas are the most common primary neoplasm of the middle ear, but little is still known about the histological features differentiating the benign and malignant forms. We investigated, with an immunohistochemical procedure, the expression of neurotrophins with their receptors, in fifteen samples of paragangliomas, and MIB-1 in order to consider them as prognostic factors of malignancy. We observed a general positivity for NGF - TrKA - NT4 - TrKC in the cytoplasm, and a strong expression for BDNF in the extracellular space. MIB-1 was moderate in the nucleus of neoplastic cells, weak in the cytoplasm and totally absent in the extracellular space. The comparison between the clinical recurrences and the rate of cytoplasmatic neurotrophins showed strong immunoreactivity in recurrent patients. It should be emphasized that 2 of the 3 recurrences had a wider distribution of the neutrophins, leading to hypothesize the involvement of these substances in the cell proliferation of glomus tumors. Malignant forms of these rare glomus tumors cannot be clearly identified using MIB-1 as a prognostic marker, although we can affirm that neurotrophins and their receptors can be considered as a panel of potential diagnostic markers to monitor the development of such malignancies. Although the small number of patients does not allow definitive conclusions to be made, our findings showed a possible trend towards significance which requires a more powerful study to evaluate this further.

Functional imaging of SDHx-related head and neck paragangliomas: comparison of 18F-fluorodihydroxyphenylalanine, 18F-fluorodopamine, 18F-fluoro-2-deoxy-D-glucose PET, 123I-metaiodobenzylguanidine scintigraphy, and 111In-pentetreotide scintigraphy.

PubMed

King, Kathryn S; Chen, Clara C; Alexopoulos, Dimitrios K; Whatley, Millie A; Reynolds, James C; Patronas, Nicholas; Ling, Alexander; Adams, Karen T; Xekouki, Paraskevi; Lando, Howard; Stratakis, Constantine A; Pacak, Karel

2011-09-01

Accurate diagnosis of head and neck paragangliomas is often complicated by biochemical silence and lack of catecholamine-associated symptoms, making accurate anatomical and functional imaging techniques essential to the diagnostic process. Ten patients (seven SDHD, three SDHB), with a total of 26 head and neck paragangliomas, were evaluated with anatomical and functional imaging. This study compares five different functional imaging techniques [(18)F-fluorodihydroxyphenylalanine ((18)F-FDOPA) positron emission tomography (PET), (18)F-fluorodopamine ((18)F-FDA) PET/computed tomography (CT), (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG) PET/CT, (123)I-metaiodobenzylguanidine ((123)I-MIBG) scintigraphy, and (111)In-pentetreotide scintigraphy] in the localization of head and neck paragangliomas. Prospectively (18)F-FDOPA PET localized 26 of 26 lesions in the 10 patients, CT/magnetic resonance imaging localized 21 of 26 lesions, (18)F-FDG PET/CT localized 20 of 26 lesions, (111)In-pentetreotide scintigraphy localized 16 of 25 lesions, (18)F-FDA PET/CT localized 12 of 26 lesions, and (123)I-MIBG scintigraphy localized eight of 26 lesions. Differences in imaging efficacy related to genetic phenotype, even in the present small sample size, included the negativity of (18)F-FDA PET/CT and (123)I-MIBG scintigraphy in patients with SDHB mutations and the accuracy of (18)F-FDG PET/CT in all patients with SDHD mutations, as compared with the accuracy of (18)F-FDG PET/CT in only one patient with an SDHB mutation. Overall, (18)F-FDOPA PET proved to be the most efficacious functional imaging modality in the localization of SDHx-related head and neck paragangliomas and may be a potential first-line functional imaging agent for the localization of these tumors.

Surgery without Blood Transfusion for Giant Paraganglioma in a Jehovah's Witness Patient

PubMed Central

Narita, Takuya; Hamano, Itsuto; Kusaka, Ayumu; Murasawa, Hiromi; Tokui, Noriko; Imanishi, Kengo; Okamoto, Teppei; Yamamoto, Hayato; Imai, Atsushi; Hatakeyama, Shingo; Yoneyama, Takahiro; Hashimoto, Yasuhiro; Koie, Takuya; Ohyama, Chikara; Hakamada, Kenichi

2014-01-01

A retroperitoneal tumor was identified in a 57-year-old female belonging to Jehovah's Witnesses during a health check. Subsequent examination led to the suspicion of a right pheochromocytoma. The patient wished to be treated by bloodless surgery and consulted our hospital after being refused surgery by several hospitals. She signed a liability waiver for blood transfusion refusal. After obtaining consent for diluted autotransfusion and preoperative administration of erythropoietin, the surgery was scheduled. The tumor was attached to the inferior vena cava and left renal vein and engulfed the right renal artery and vein. The tumor and right kidney were removed en bloc. Operative time was 8 h and 18 min, with 1,770 ml of blood loss. The histopathological diagnosis was paraganglioma with the normal adrenal gland within the border of the tumor. The patient was discharged from the hospital with no postoperative complications. PMID:24803901

Biochemically Silent Abdominal Paragangliomas in Patients with Mutations in the Succinate Dehydrogenase Subunit B Gene

PubMed Central

Timmers, Henri J. L. M.; Pacak, Karel; Huynh, Thanh T.; Abu-Asab, Mones; Tsokos, Maria; Merino, Maria J.; Baysal, Bora E.; Adams, Karen T.; Eisenhofer, Graeme

2008-01-01

Context: Patients with adrenal and extra-adrenal abdominal paraganglioma (PGL) almost invariably have increased plasma and urine concentrations of metanephrines, the O-methylated metabolites of catecholamines. We report four cases of biochemically silent abdominal PGL, in which metanephrines were normal despite extensive disease. Objective: Our objective was to identify the mechanism underlying the lack of catecholamine hypersecretion and metabolism to metanephrines in biochemically silent PGL. Design: This is a descriptive study. Setting: The study was performed at a referral center. Patients: One index case and three additional patients with large abdominal PGL and metastases but with the lack of evidence of catecholamine production, six patients with metastatic catecholamine-producing PGL and a mutation of the succinate dehydrogenase subunit B (SDHB) gene, and 136 random patients with catecholamine-producing PGL were included in the study. Main Outcome Measures: Plasma, urine, and tumor tissue concentrations of catecholamines and metabolites were calculated with electron microscopy and tyrosine hydroxylase immunohistochemistry. Results: All four patients with biochemically silent PGL had an underlying SDHB mutation. In the index case, the tumor tissue concentration of catecholamines (1.8 nmol/g) was less than 0.01% that of the median (20,410 nmol/g) for the 136 patients with catecholamine-producing tumors. Electron microscopy showed the presence of normal secretory granules in all four biochemically silent PGLs. Tyrosine hydroxylase immunoreactivity was negligible in the four biochemically silent PGLs but abundant in catecholamine-producing PGLs. Conclusions: Patients with SDHB mutations may present with biochemically silent abdominal PGLs due to defective catecholamine synthesis resulting from the absence of tyrosine hydroxylase. Screening for tumors in patients with SDHB mutations should not be limited to biochemical tests of catecholamine excess. PMID

The missing "Bratz" doll: a case of vaginal foreign body.

PubMed

Someshwar, Jean; Lutfi, Riad; Nield, Linda S

2007-12-01

Young female patients with gynecologic complaints, including vaginal discharge, commonly seek care in the emergency department. Prepubertal vaginal discharges have various potential etiologies including nonspecific vulvovaginitis, infections, inflammatory or dermatologic processes, precocious puberty, malignancy, anatomical anomaly, trauma, sexual abuse, and foreign body. We present the case of a 4-year-old girl with vaginal odor and discharge secondary to a foreign body. The foreign body was part of a toy, the lower half of the patient's missing "Bratz" (MGA Entertainment) doll. Although it is well known that a vaginal foreign body can cause a vaginal discharge, the unusual nature of the foreign body in this case and the revealing radiograph made this a case of particular interest.

External beam radiation therapy for advanced/unresectable malignant paraganglioma and pheochromocytoma.

PubMed

Breen, William; Bancos, Irina; Young, William F; Bible, Keith C; Laack, Nadia N; Foote, Robert L; Hallemeier, Christopher L

2018-01-01

To evaluate the role of external beam radiation therapy (EBRT) for treatment of malignant paraganglioma (PGL) and pheochromocytoma (PCC). A retrospective review was performed of all patients with malignant PGL/PCC treated with EBRT at our institution between 1973 and 2015. Local control (LC) per treated lesion and overall survival were estimated using the Kaplan-Meier method. Toxicities were scored using the Common Toxicity Criteria for Adverse Events (AE), version 4. The cohort included 41 patients with 107 sites treated. Median (range) age at EBRT was 33 (11-80) years. Treatment intention was curative in 20 patients (30 lesions) and palliative in 21 patients (77 lesions). The primary tumor was PGL (63%) and PCC (37%). Previous local therapies were surgical resection (90%) and percutaneous ablation (19%). Indications for EBRT were local control (66%), pain (22%), or spinal cord compression (12%). Treatment site included bone (69%), soft tissue (30%), and liver (1%). Median (range) EBRT dose was 40 (6.5-70) Gy. Median biologic effective dose using α/β = 10 (BED 10 ) was 53 (9-132). Median follow-up was 3.8 years (0.04-41.5), and mean follow-up was 9.7 years. Overall survival at 5 years was 65%: 79% for curative- and 50% for palliative-intention patients ( P  = .028). LC at 5 years was 81% for all lesions; 91% for lesions receiving BED 10 ≥53, and 62% for lesions receiving BED 10 <53 ( P  = .001). All 11 lesions treated with stereotactic body RT or radiosurgery had LC at a median of 3.0 (0.2-5.4) years. For the symptomatic lesions, symptoms improved in 94%. There were no acute grade ≥3 treatment-related AEs, including no hypertensive crises. Two patients developed a late grade ≥3 AE. EBRT is a useful treatment modality for malignant PGL and PCC. Higher RT dose was associated with improved LC.

Pituitary Adenoma With Paraganglioma/Pheochromocytoma (3PAs) and Succinate Dehydrogenase Defects in Humans and Mice

PubMed Central

Xekouki, Paraskevi; Szarek, Eva; Bullova, Petra; Giubellino, Alessio; Quezado, Martha; Mastroyannis, Spyridon A.; Mastorakos, Panagiotis; Wassif, Christopher A.; Raygada, Margarita; Rentia, Nadia; Dye, Louis; Cougnoux, Antony; Koziol, Deloris; Sierra, Maria de La Luz; Lyssikatos, Charalampos; Belyavskaya, Elena; Malchoff, Carl; Moline, Jessica; Eng, Charis; Maher, Louis James; Pacak, Karel; Lodish, Maya

2015-01-01

Context: Germline mutations in genes coding succinate dehydrogenase (SDH) subunits A, B, C, and D have been identified in familial paragangliomas (PGLs)/pheochromocytomas (PHEOs) and other tumors. We described a GH-secreting pituitary adenoma (PA) caused by SDHD mutation in a patient with familial PGLs. Additional patients with PAs and SDHx defects have since been reported. Design: We studied 168 patients with unselected sporadic PA and with the association of PAs, PGLs, and/or pheochromocytomas, a condition we named the 3P association (3PAs) for SDHx germline mutations. We also studied the pituitary gland and hormonal profile of Sdhb+/− mice and their wild-type littermates at different ages. Results: No SDHx mutations were detected among sporadic PA, whereas three of four familial cases were positive for a mutation (75%). Most of the SDHx-deficient PAs were either prolactinomas or somatotropinomas. Pituitaries of Sdhb+/− mice older than 12 months had an increased number mainly of prolactin-secreting cells and several ultrastructural abnormalities such as intranuclear inclusions, altered chromatin nuclear pattern, and abnormal mitochondria. Igf-1 levels of mutant mice tended to be higher across age groups, whereas Prl and Gh levels varied according to age and sex. Conclusion: The present study confirms the existence of a new association that we termed 3PAs. It is due mostly to germline SDHx defects, although sporadic cases of 3PAs without SDHx defects also exist. Using Sdhb+/− mice, we provide evidence that pituitary hyperplasia in SDHx-deficient cells may be the initial abnormality in the cascade of events leading to PA formation. PMID:25695889

Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review.

PubMed

Därr, Roland; Kuhn, Matthias; Bode, Christoph; Bornstein, Stefan R; Pacak, Karel; Lenders, Jacques W M; Eisenhofer, Graeme

2017-06-01

To determine the accuracy of biochemical tests for the diagnosis of pheochromocytoma and paraganglioma. A search of the PubMed database was conducted for English-language articles published between October 1958 and December 2016 on the biochemical diagnosis of pheochromocytoma and paraganglioma using immunoassay methods or high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection for measurement of fractionated metanephrines in 24-h urine collections or plasma-free metanephrines obtained under seated or supine blood sampling conditions. Application of the Standards for Reporting of Diagnostic Studies Accuracy Group criteria yielded 23 suitable articles. Summary receiver operating characteristic analysis revealed sensitivities/specificities of 94/93% and 91/93% for measurement of plasma-free metanephrines and urinary fractionated metanephrines using high-performance liquid chromatography or immunoassay methods, respectively. Partial areas under the curve were 0.947 vs. 0.911. Irrespective of the analytical method, sensitivity was significantly higher for supine compared with seated sampling, 95 vs. 89% (p < 0.02), while specificity was significantly higher for supine sampling compared with 24-h urine, 95 vs. 90% (p < 0.03). Partial areas under the curve were 0.942, 0.913, and 0.932 for supine sampling, seated sampling, and urine. Test accuracy increased linearly from 90 to 93% for 24-h urine at prevalence rates of 0.0-1.0, decreased linearly from 94 to 89% for seated sampling and was constant at 95% for supine conditions. Current tests for the biochemical diagnosis of pheochromocytoma and paraganglioma show excellent diagnostic accuracy. Supine sampling conditions and measurement of plasma-free metanephrines using high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection provides the highest accuracy at all prevalence rates.

Carotid body tumor imitator: An interesting case of Castleman's disease.

PubMed

Shakir, Hakeem J; Diletti, Sara M; Hart, Alexandra M; Meyers, Joshua E; Dumont, Travis M; Siddiqui, Adnan H

2015-01-01

There are very few reports in the literature of Castleman's disease affecting the carotid artery and a single previous report of a case of Castleman's disease of the neck originally mistaken as a carotid body tumor. We describe a rare case of Castleman's disease, manifesting with classic radiographic hallmarks of a carotid body tumor. The postoperative pathologic examination identified the resected mass as Castleman's lymphadenopathy. The management of this particular case is discussed, and the findings are highlighted. We present a unique case of a tumor initially and incorrectly diagnosed as a carotid body tumor. However, after comprehensive treatment with endovascular and surgical modalities and subsequent pathologic examination, the diagnosis of this rare entity was made.

Foreign bodies retained in the vagina: a case report.

PubMed

Nanda, Smiti; Singhal, Savita Rani; Marya, Ashima

2006-04-01

Foreign bodies have been found in the vagina in a variety of forms. A 3-year-old girl had a variety of foreign bodies in her vagina. One should always think of foreign bodies in the vagina in cases of vaginal discharge in childhood.

[Three cases of an intracranial wooden foreign body].

PubMed

Fujimoto, S; Onuma, T; Amagasa, M; Okudaira, Y

1987-07-01

Three cases of intracranial wooden foreign body are reported discussing the diagnostic and therapeutic problems. First case is a 50-year-old man. After drinking, he drove a bike and fell to the ground. On admission the wooden foreign body could not been detected in appearance. CT scan showed low density area similar to air in bilateral anterior horn of lateral ventricle. The patient was treated for traumatic pneumocephalus at first. Later, it proved that he was stabbed with a foreign body penetrating into the contralateral frontal lobe through the left nasal cavity. It was extracted by endonasal approach by otolaryngologist, fortunately without trouble. The foreign body was a branch of tree. The second case is an 18-year-old man. He was driving a car, and suffered injury. He was stabbed with a wooden stake penetrating into his left eye. Immediately, bifrontal craniotomy was performed and the stake was withdrawn carefully. Moreover bone fragments were removed. The third case is a 61-year-old man. When he cut the timber by chain saw, a piece of wood hit and stabbed his right eye directly. Immediately right front temporal craniotomy was performed. The piece of wood was withdrawn from the right eye, and pieces of glass, wood and bone fragments were evacuated. It is difficult to confirm intracranial foreign body accurately by means of only plain skull film and usual CT scans. It is necessary to utilize various function of CT scanner. For example, it is useful to know CT values or select measure mode with window width and level or make reconstruction image to sagittal or coronal section, and so on.(ABSTRACT TRUNCATED AT 250 WORDS)

Case report: vertebral foreign body granuloma mimicking a skeletal metastasis.

PubMed

Vossen, Josephina A; Bathaii, Seyed M; Hatfield, Bryce; Hayes, Curtis W

2018-06-01

Intraosseous foreign body granuloma formation related to migrated surgical material is a rarely reported condition with variable imaging appearance. In this case report, we describe a foreign body granuloma that occurred in a lumbar vertebral body one level above a prior surgical fusion. The lytic appearance mimicked a skeletal metastasis in a 65-year-old patient with recently diagnosed renal cell carcinoma. To the best of our knowledge, this is the first reported case of a lumbar vertebral foreign body granuloma occurring distant from the site of surgery, indistinguishable from skeletal metastasis on radiologic examination.

Is there an optimal preoperative management strategy for phaeochromocytoma/paraganglioma?

PubMed

Challis, B G; Casey, R T; Simpson, H L; Gurnell, M

2017-02-01

Phaeochromocytomas and paragangliomas (PPGLs) are catecholamine secreting neuroendocrine tumours that predispose to haemodynamic instability. Currently, surgery is the only available curative treatment, but carries potential risks including hypertensive and hypotensive crises, cardiac arrhythmias, myocardial infarction and stroke, due to tumoral release of catecholamines during anaesthetic induction and tumour manipulation. The mortality associated with surgical resection of PPGL has significantly improved from 20-45% in the early 20th century (Apgar & Papper, AMA Archives of Surgery, 1951, 62, 634) to 0-2·9% in the early 21st century (Kinney et al. Journal of Cardiothoracic and Vascular Anesthesia, 2002, 16, 359), largely due to availability of effective pharmacological agents and advances in surgical and anaesthetic practice. However, surgical resection of PPGL still poses significant clinical management challenges. Preoperatively, alpha-adrenoceptor blockade is the mainstay of management, although various pharmacological strategies have been proposed, based largely on reports derived from retrospective data sets. To date, no consensus has been reached regarding the 'ideal' preoperative strategy due, in part, to a paucity of data from high-quality evidence-based studies comparing different treatment regimens. Here, based on the available literature, we address the Clinical Question: Is there an optimal preoperative management strategy for PPGL? © 2016 John Wiley & Sons Ltd.

Acquired hypermethylation of the P16INK4A promoter in abdominal paraganglioma: relation to adverse tumor phenotype and predisposing mutation

PubMed Central

Kiss, Nimrod B; Muth, Andreas; Andreasson, Adam; Juhlin, C Christofer; Geli, Janos; Bäckdahl, Martin; Höög, Anders; Wängberg, Bo; Nilsson, Ola; Ahlman, Håkan; Larsson, Catharina

2013-01-01

Recurrent alterations in promoter methylation of tumor suppressor genes (TSGs) and LINE1 (L1RE1) repeat elements were previously reported in pheochromocytoma and abdominal paraganglioma. This study was undertaken to explore CpG methylation abnormalities in an extended tumor panel and assess possible relationships between metastatic disease and mutation status. CpG methylation was quantified by bisulfite pyrosequencing for selected TSG promoters and LINE1 repeats. Methylation indices above normal reference were observed for DCR2 (TNFRSF10D), CDH1, P16 (CDKN2A), RARB, and RASSF1A. Z-scores for overall TSG, and individual TSG methylation levels, but not LINE1, were significantly correlated with metastatic disease, paraganglioma, disease predisposition, or outcome. Most strikingly, P16 hypermethylation was strongly associated with SDHB mutation as opposed to RET/MEN2, VHL/VHL, or NF1-related disease. Parallel analyses of constitutional, tumor, and metastasis DNA implicate an order of events where constitutional SDHB mutations are followed by TSG hypermethylation and 1p loss in primary tumors, later transferred to metastatic tissue. In the combined material, P16 hypermethylation was prevalent in SDHB-mutated samples and was associated with short disease-related survival. The findings verify the previously reported importance of P16 and other TSG hypermethylation in an independent tumor series. Furthermore, a constitutional SDHB mutation is proposed to predispose for an epigenetic tumor phenotype occurring before the emanation of clinically recognized malignancy. PMID:23154831

Stereotactic Body Radiotherapy for Head and Neck Tumors

ClinicalTrials.gov

2016-04-18

Squamous Cell Carcinoma of the Head and Neck; Nasopharyngeal Carcinoma; Salivary Gland Cancer; Head and Neck Sarcoma; Paraganglioma of Head and Neck; Chordoma of Head and Neck; Chondrosarcoma of Head and Neck; Angiofibroma of Head and Neck

Double osteotomy of mandibula in the treatment of carotid body tumors with skull base extension.

PubMed

Prouse, Giorgio; Mazzaccaro, Daniela; Settembrini, Fernanda; Carmo, Michele; Biglioli, Federico; Settembrini, Piergiorgio G

2013-08-01

We report two patients with a carotid body paraganglioma that extended to the skull base, a position that is surgically inaccessible by means of a traditional lateral cervical approach. In both patients we were able to remove the lesion by performing a double mandibular osteotomy. Both patients underwent preoperative embolization to reduce the mass. In our experience, this approach has allowed a safe radical excision of exceptionally high lesions with only minor permanent nerve damage. In our opinion this advantage definitely outweighs the consequences of the increased invasiveness of this technique. Copyright © 2013 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.

Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false "reverse" discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy.

PubMed

Basu, S; Nair, N

2006-08-01

The incidence of paraganglioma in the paediatric population is exceedingly rare, accounting for < 0.1% of childhood cancers. We report here the response and toxicity profile in a case of malignant paraganglioma which was treated with what is currently perceived as an unconventional and non-standard approach, using three consecutive low doses of 131I-MIBG (a cumulative dose of 11 647.6 MBq). The patient had a stable disease at the end of 42 months follow-up following the first treatment with 131I-MIBG. Excellent symptomatic and hormonal responses were observed. The only adverse effect was mild nausea in the first 24 h after therapy. In addition to the potentially primary end point of radiological and biochemical response measurement, we, in this paper, endeavoured to look for a quality of life evaluation for this form of treatment. Given the rarity of this condition, the experience gained by this therapeutic approach is intriguing from response and toxicity standpoints and may be extrapolated to malignant pheochromocytoma as well. An apparent "reverse discordance" between 131I-MIBG scintigraphy and 99Tc(m)-MDP bone scan encountered during pre-therapy work up is also described with possible explanations. This draws attention to an important clinical issue in selecting patients for high dose targeted therapy.

Metabologenomics of Phaeochromocytoma and Paraganglioma: An Integrated Approach for Personalised Biochemical and Genetic Testing

PubMed Central

Eisenhofer, Graeme; Klink, Barbara; Richter, Susan; Lenders, Jacques WM; Robledo, Mercedes

2017-01-01

The tremendous advances over the past two decades in both clinical genetics and biochemical testing of chromaffin cell tumours have led to new considerations about how these aspects of laboratory medicine can be integrated to improve diagnosis and management of affected patients. With germline mutations in 15 genes now identified to be responsible for over a third of all cases of phaeochromocytomas and paragangliomas, these tumours are recognised to have one of the richest hereditary backgrounds among all neoplasms. Depending on the mutation, tumours show distinct differences in metabolic pathways that relate to or even directly impact clinical presentation. At the same time, there has been improved understanding about how catecholamines are synthesised, stored, secreted and metabolised by chromaffin cell tumours. Although the tumours may not always secrete catecholamines it has become clear that almost all continuously produce and metabolise catecholamines. This has not only fuelled changes in laboratory medicine, but has also assisted in recognition of genotype-biochemical phenotype relationships important for diagnostics and clinical care. In particular, differences in catecholamine and energy pathway metabolomes can guide genetic testing, assist with test interpretation and provide predictions about the nature, behaviour and imaging characteristics of the tumours. Conversely, results of genetic testing are important for guiding how routine biochemical testing should be employed and interpreted in surveillance programmes for at-risk patients. In these ways there are emerging needs for modern laboratory medicine to seamlessly integrate biochemical and genetic testing into the diagnosis and management of patients with chromaffin cell tumours. PMID:29332973

Integrated whole-body PET/MR imaging with 18F-FDG, 18F-FDOPA, and 18F-fluorodopamine in paragangliomas, in comparison to PET/CT: NIH first clinical experience with a single-injection, dual-modality imaging protocol

PubMed Central

Blanchet, Elise M.; Millo, Corina; Martucci, Victoria; Maass-Moreno, Roberto; Bluemke, David A.; Pacak, Karel

2017-01-01

Purpose Paragangliomas (PGLs) are tumors that can metastasize and recur; therefore, lifelong imaging follow-up is required. Hybrid positron emission tomography (PET)/computed tomography (/CT) is an essential tool to image PGLs. Novel hybrid PET/magnetic resonance (/MR) scanners are currently being studied in clinical oncology. We studied the feasibility of simultaneous whole-body PET/MR imaging to evaluate patients with PGLs. Methods Fifty-three PGLs or PGL-related lesions from eight patients were evaluated. All patients underwent a single-injection, dual-modality imaging protocol consisting of a PET/CT and subsequent PET/MR scan. Four patients were evaluated with 18F-fluorodeoxyglucose (18F-FDG), two with 18F-fluorodihydroxyphenylalanine (18F-FDOPA), and two with 18F-fluorodopamine (18F-FDA). PET/MR data were acquired using a hybrid whole-body 3-Tesla integrated PET/MR scanner. PET and MR data (DIXON images for attenuation correction and T2-weighted sequences for anatomic allocation) were acquired simultaneously. Imaging workflow and imaging times were documented. PET/MR and PET/CT data were visually assessed (blindly) in regards to image quality, lesion detection, and anatomic allocation and delineation of the PET findings. Results With hybrid PET/MR, we obtained high quality images in an acceptable acquisition time (median: 31 min, range: 25–40 min) with good patient compliance. A total of 53 lesions, located in the head-and-neck area (6), mediastinum (2), abdomen and pelvis (13), lungs (2), liver (4), and bone (26) were evaluated. 51 lesions were detected with PET/MR and confirmed by PET/CT. Two bone lesions (L4 body (8 mm) and sacrum (6 mm)) were not detectable on an 18F-FDA scan PET/MR, likely due to washout of the 18F-FDA. Co-registered MR tended to be superior to co-registered CT for head-and-neck, abdomen, pelvis, and liver lesions for anatomic allocation and delineation. Conclusions Clinical PGL evaluation with hybrid PET/MR is feasible with high image

Intraorbital foreign bodies--5 own cases and review of literature.

PubMed

Markowski, Jarosław; Dziubdziela, Włodzimierz; Gierek, Tatiana; Witkowska, Małgorzata; Mrukwa-Kominek, Ewa; Niedzielska, Iwona; Paluch, Jarosław

2012-01-01

Five patients were surgically treated for intraorbital foreign body: a 14-year-old girl had a door glass splinter, a 23-year-old man a metallic foreign body--gunshot pellet, a 55-year-old man a splinter from a metallic bar, a 48-year-old patient the splinters of circular saw and 61-year-old man with shot. Two foreign bodies were removed using the Krönlein-Reese-Berk lateral orbitotomy, two others by Sewell medial orbitotomy and one with superior orbitotomy of Dandy-Naffziger. Radiographs and CT scans were used to identify and localize intraorbital foreign bodies. In one case we found coexistence foreign body (shot) and tumor--inflammation pseudotumor of the orbita. It is possible, that in this case long-time occupy foreign body in the orbita was a cause of that tumor. All foreign bodies were successfully removed, and postoperative course was uneventful. The Krönlein-Reese-Berk orbitotomy provides a satisfactory access to the lateral and posterior orbit, which is of particular importance in the case of a deeply penetrating foreign body (metallic or glass). Surgical removal of intraorbital foreign bodies is a classic example of an interdisciplinary therapeutic approach. Best outcome is usually a result of a team of an ophtalmologist, ENT surgeon, maxillary surgeon and possibly also neurosurgeon performing the operation. Copyright © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.

Update on the management of unusual neuroendocrine tumors: pheochromocytoma and paraganglioma, medullary thyroid cancer and adrenocortical carcinoma.

PubMed

Strosberg, Jonathan R

2013-02-01

Pheochromocytomas, paragangliomas, and medullary thyroid carcinomas (MTCs) originate in cells that share a common neuroectodermal origin. Like other neuroendocrine neoplasms, they are characterized by a propensity to secrete amines (epinephrine and norepinephrine) and peptide hormones (calcitonin). Improved understanding of underlying molecular pathways, such as mutations of the RET (rearranged during transfection) proto-oncogene, has led to new rational targeted therapies. Adrenocortical carcinomas (ACCs) originate in the steroid hormone-producing adrenal cortex. While tumors of the adrenal cortex are not, strictly speaking, part the "diffuse neuroendocrine system," they are often included in neuroendocrine tumor guidelines due to their orphan status. In this update on management of unusual neuroendocrine tumors, we review the biology and treatment of these rare neoplasms. Copyright © 2013 Elsevier Inc. All rights reserved.

Risk assessment of maternally inherited SDHD paraganglioma and phaeochromocytoma.

PubMed

Burnichon, Nelly; Mazzella, Jean-Michaël; Drui, Delphine; Amar, Laurence; Bertherat, Jérôme; Coupier, Isabelle; Delemer, Brigitte; Guilhem, Isabelle; Herman, Philippe; Kerlan, Véronique; Tabarin, Antoine; Wion, Nelly; Lahlou-Laforet, Khadija; Favier, Judith; Gimenez-Roqueplo, Anne-Paule

2017-02-01

Germline mutations in the SDHD tumour suppressor gene (11q23.1) predispose to phaeochromocytomas and paragangliomas (PPGL) mainly on a paternal transmission. However, PPGL have been recently reported in three carriers of a maternally inherited SDHD mutation. To assess the risk of PPGL occurrence on maternal transmission of SDHD mutation. Pedigrees of 80 SDHD-related families have been reviewed. 35 asymptomatic subjects carrying a maternally transmitted SDHD mutation were identified. 20 of them accepted to benefit from a PPGL imaging screening. A unique histologically proven biochemically negative phaeochromocytoma has been diagnosed in a 35-year-old woman. Molecular investigations carried out on tumour tissue revealed that the loss of heterozygosity encompassed the paternally derived q arm and the maternally derived p arm of chromosome 11. This study demonstrates that the risk of developing PPGL for a subject carrying a germline SDHD mutation on the maternal allele remains a rare scenario but does exist. Our data suggest an adjustment of current genetic counselling and clinical care recommendations for at-risk subjects. A targeted familial genetic test should be proposed from the age of 18 years to every subject having a mother carrying a germline SDHD mutation and a first medical workup, including imaging, should be recommended to SDHD-positive mutation carriers. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Hypertensive emergency masquerading as phaeochromocytoma: a report of two cases.

PubMed

Hulkower, Raphael; Gubbi, Sriram; Meholli, Mimoza; Schubart, Ulrich

2017-08-07

We report two cases of patients who were hospitalised for hypertensive emergency. In both cases, laboratory testing during admission revealed elevated normetanephrines in the range of 4-5 times the upper limit of normal using liquid chromatography with mass spectrometry. Imaging studies did not localise any phaeochromocytoma or paraganglioma (PPGL). Repeat biochemical testing after discharge was within normal limits in both cases. These observations suggest that hypertensive emergency should be recognised as a potential cause of 'false-positive' laboratory findings in the diagnostic assessment for PPGL. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Diffuse Lewy body disease: clinical features in 15 cases.

PubMed Central

Byrne, E J; Lennox, G; Lowe, J; Godwin-Austen, R B

1989-01-01

Fifteen cases of diffuse Lewy body disease were diagnosed on pathological grounds during a single year in one health district. The range and frequency of clinical features contrast strikingly with previous reports. The majority of cases presented with classical levodopa-responsive Parkinson's disease either alone (6 cases) or with mild cognitive impairment (3 cases); the remaining 6 cases presented with cognitive impairment alone. In time almost all patients developed both dementia and Parkinsonism. The dementia was cortical in type, but unusual in that most (12 cases) showed day-to-day fluctuation in severity at some point in their illness. These findings suggest that diffuse Lewy body disease is not rare, and that it presents in a range of ways from dementia with subsequent Parkinsonism to Parkinson's disease with subsequent dementia. The latter mode of presentation suggests that it should be considered as a significant pathological substrate of dementia in Parkinson's disease. Images PMID:2545827

Ingested foreign bodies in children: a report of two cases.

PubMed

Singh, Gurjit; Sharma, Surendra; Khurade, Shrikant; Gooptu, Somnath

2014-01-01

Accidental foreign body ingestion and impaction of food bolus present as an emergency. Pediatric population is the most vulnerable. Since the act may go unnoticed, the child may present late. However, most foreign bodies pass spontaneously in the stools. Two children were brought with history of battery ingestion. In one case, an emergency gastro intestinal endoscopy had to be done for the foreign body removal which was made up of corroded battery. In the other case, no interventation was undertaken & an uncorroded battery passed per anum along with stools after 15 days of ingestion. Decision regarding immediate intervention or conservative approach will require consideration of the level of lodgement & type of foreign body. Prevention is possible if parents/guardians exercise control on their wards & are aware of implications of such an event.

First report of bilateral pheochromocytoma in the clinical spectrum of HIF2A-related polycythemia-paraganglioma syndrome.

PubMed

Taïeb, David; Yang, Chunzhang; Delenne, Blandine; Zhuang, Zhengping; Barlier, Anne; Sebag, Fréderic; Pacak, Karel

2013-05-01

Molecular genetic research has so far resulted in the identification of 10 well-characterized susceptibility genes for hereditary pheochromocytoma (PHEO) or paraganglioma (PGL). Recently, a new syndrome characterized by multiple PGLs and somatostatinomas associated with congenital polycythemia due to somatic mutations in HIF2A has been reported. The aim of the study was to define the genetic defect in a new case of bilateral PHEO and multiple PGLs associated with congenital polycythemia. A female patient presented with neonatal polycythemia (treated by phlebotomies, 1 session approximately every 4 mo), mildly enlarged cerebral ventricles, and bilateral PHEO and multiple PGLs. There was no family history of any neuroendocrine tumor or polycythemia. Surgical removal of the tumors only temporarily normalized plasma erythropoietin (Epo) levels and discontinued phlebotomies. No germline mutations were initially detected in the SDHB, SDHC, SDHD, VHL, and PHD2 genes, known to be associated with polycythemia. The PHEOs presented with a typical noradrenergic biochemical phenotype. A heterozygous missense mutation (c.1589C>T) was identified in exon 12 of HIF2A, resulting in an alanine 530 substitution in the HIF-2α protein with valine (A530V). This somatic mutation was detected in the tissue from 1 PHEO and 1 PGL, with no HIF2A germline mutation found. This mutation led to stabilization of HIF-2α and hence a gain-of-function phenotype, as in previously published studies. This case represents the first association of a somatic HIF2A gain-of-function mutation with PHEO and congenital polycythemia, and it alerts physicians to perform proper genetic screening in patients presenting with multiple norepinephrine-producing PHEOs and polycythemia. This report also extends the previous findings of a new syndrome of only multiple PGLs, somatostatinomas, and polycythemia to multiple PHEOs.

Precision medicine in pheochromocytoma and paraganglioma: current and future concepts.

PubMed

Björklund, P; Pacak, K; Crona, J

2016-12-01

Pheochromocytoma and paraganglioma (PPGL) are rare diseases but are also amongst the most characterized tumour types. Hence, patients with PPGL have greatly benefited from precision medicine for more than two decades. According to current molecular biology and genetics-based taxonomy, PPGL can be divided into three different clusters characterized by: Krebs cycle reprogramming with oncometabolite accumulation or depletion (group 1a); activation of the (pseudo)hypoxia signalling pathway with increased tumour cell proliferation, invasiveness and migration (group 1b); and aberrant kinase signalling causing a pro-mitogenic and anti-apoptotic state (group 2). Categorization into these clusters is highly dependent on mutation subtypes. At least 12 different syndromes with distinct genetic causes, phenotypes and outcomes have been described. Genetic screening tests have a documented benefit, as different PPGL syndromes require specific approaches for optimal diagnosis and localization of various syndrome-related tumours. Genotype-tailored treatment options, follow-up and preventive care are being investigated. Future new developments in precision medicine for PPGL will mainly focus on further identification of driver mechanisms behind both disease initiation and malignant progression. Identification of novel druggable targets and prospective validation of treatment options are eagerly awaited. To achieve these goals, we predict that collaborative large-scale studies will be needed: Pheochromocytoma may provide an example for developing precision medicine in orphan diseases that could ultimately aid in similar efforts for other rare conditions. © 2016 The Association for the Publication of the Journal of Internal Medicine.

Phase II trial of pazopanib in advanced/progressive malignant pheochromocytoma and paraganglioma.

PubMed

Jasim, Sina; Suman, Vera J; Jimenez, Camilo; Harris, Pamela; Sideras, Kostandinos; Burton, Jill K; Worden, Francis Paul; Auchus, Richard J; Bible, Keith C

2017-08-01

Pheochromocytomas and paragangliomas (Pheo/PGL) are rare, vascular, sometimes malignant endocrine tumors. Case reports indicate the activity of vascular endothelium growth factor receptor-targeted kinase inhibitors in these cancers. To assess the antitumor activity and tolerability of pazopanib in progressive malignant Pheo/PGL. This multicenter Phase II trial (MC107C) enrolled individuals  ≥18 years old with disease progression ≤ 6 months prior to registration, Eastern Cooperative Oncology Group PS 0-2, and measurable disease (response evaluation criteria in solid tumors 1.0). Pazopanib was administered in 28-day cycles, with the regimen ultimately being as follows: cycle 1: 400 mg daily on days 1-14, cycle 2: 800 mg daily on days 1-14, and then cycle 2 + : 800 mg daily on all days. The study was halted due to poor accrual. Seven patients were enrolled (05/2011-11/2014). One patient withdrew consent prior to treatment, leaving six evaluable patients. Treatment was discontinued, due to the following reasons: disease progression (4); withdrawal (1); and grade 4 (Takotsubo) cardiomyopathy (1). The median number of cycles administered was 4 (range: 2-29, total: 49). Four patients had >1 dose reduction due to the following reasons: fatigue (1), abnormal liver tests (2), hypertension and (Takotsubo) cardiomyopathy (1), and headaches (1). Common severe (Common Terminology Criteria for Adverse Events v3.0 grades 3-5) toxicities were as follows: hypertension (3/6), (Takotsubo) cardiomyopathy (2/6), diarrhea (1/6), fatigue (1/6), headache (1/6), and hematuria (1/6). One confirmed partial response was observed in PGL (17%, duration 2.4 years); median progression-free survival and overall survival were 6.5 and 14.8 months, respectively. Pazopanib has activity in Pheo/PGL requiring more study; optimal alpha- and beta-blockade are imperative pre-therapy in patients with secretory tumors, as risk of hypertension and cardiomyopathy are potentially life

Succinate dehydrogenase subunit D and succinate dehydrogenase subunit B mutation analysis in canine phaeochromocytoma and paraganglioma.

PubMed

Holt, D E; Henthorn, P; Howell, V M; Robinson, B G; Benn, D E

2014-07-01

Phaeochromocytomas (PCs) are tumours of the adrenal medulla chromaffin cells. Paragangliomas (PGLs) arise in sympathetic ganglia (previously called extra-adrenal PCs) or in non-chromaffin parasympathetic ganglia cells that are usually non-secretory. Parenchymal cells from these tumours have a common embryological origin from neural crest ectoderm. Several case series of canine PCs and PGLs have been published and a link between the increased incidence of chemoreceptor neoplasia in brachycephalic dog breeds and chronic hypoxia has been postulated. A similar link to hypoxia in man led to the identification of germline heterozygous mutations in the gene encoding succinate dehydrogenase subunit D (SDHD) and subsequently SDHA, SDHB and SDHC in similar tumours. We investigated canine PCs (n = 6) and PGLs (n = 2) for SDHD and SDHB mutations and in one PGL found a somatic SDHD mutation c.365A>G (p.Lys122Arg) in exon 4, which was not present in normal tissue from this brachycephalic dog. Two PCs were heterozygous for both c.365A>G (p.Lys122Arg) mutation and an exon 3 silent variant c.291G>A. We also identified the heterozygous SDHB exon 2 mutation c.113G>A (p.Arg38Gln) in a PC. These results illustrate that genetic mutations may underlie tumourigenesis in canine PCs and PGLs. The spontaneous nature of these canine diseases and possible association of PGLs with hypoxia in brachycephalic breeds may make them an attractive model for studying the corresponding human tumours. Copyright © 2014 Elsevier Ltd. All rights reserved.

ACCURACY OF PLASMA FREE METANEPHRINES IN THE DIAGNOSIS OF PHEOCHROMOCYTOMA AND PARAGANGLIOMA: A SYSTEMATIC REVIEW AND META-ANALYSIS.

PubMed

Chen, Yan; Xiao, Huangmeng; Zhou, Xieda; Huang, Xiaoyu; Li, Yanbing; Xiao, Haipeng; Cao, Xiaopei

2017-10-01

Various studies have validated plasma free metanephrines (MNs) as biomarkers for pheochromocytoma and paraganglioma (PPGL). This meta-analysis aimed to estimate the overall diagnostic accuracy of this biochemical test for PPGL. We searched the PubMed, the Cochrane Library, Web of Science, Embase, Scopus, OvidSP, and ProQuest Dissertations & Theses databases from January 1, 1995 to December 2, 2016 and selected studies written in English that assessed plasma free MNs in the diagnosis of PPGL. Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) was used to evaluate the quality of the included studies. We calculated pooled sensitivities, specificities, positive and negative likelihood ratios, diagnostic odds ratios (DORs) and areas under curve (AUCs) with their 95% confidence intervals (95% CIs). Heterogeneity was assessed by I 2 . To identify the source of heterogeneity, we evaluated the threshold effect and performed a meta-regression. Deeks' funnel plot was selected for investigating any potential publication bias. Although the combination of metanephrine (MN) and normetanephrine (NMN) carried lower specificity (0.94, 95% CI 0.90-0.97) than NMN (0.97, 95% CI 0.92-0.99), NMN was generally more accurate than individual tests, with the highest AUC (0.99, 95% CI 0.97-0.99), DOR (443.35, 95% CI 216.9-906.23), and pooled sensitivity (0.97, 95% CI 0.94-0.98) values. Threshold effect and meta-regression analyses showed that different cut-offs, blood sampling positions, study types and test methods contributed to heterogeneity. This meta-analysis suggested an effective value for combined plasma free MNs for the diagnosis of PPGL, but testing for MNs requires more standardization using tightly regulated studies. AUC = area under curve; CI = confidence interval; DOR = diagnostic odds ratio; EIA = enzyme immunoassay; LC-ECD = liquid chromatography-electrochemical detection; LC-MS/MS = liquid chromatography-tandem mass spectrometry; MN = metanephrine; NMN

Review of sequencing platforms and their applications in phaeochromocytoma and paragangliomas.

PubMed

Pillai, Suja; Gopalan, Vinod; Lam, Alfred King-Yin

2017-08-01

Genetic testing is recommended for patients with phaeochromocytoma (PCC) and paraganglioma (PGL) because of their genetic heterogeneity and heritability. Due to the large number of susceptibility genes associated with PCC/PGL, next-generation sequencing (NGS) technology is ideally suited for carrying out genetic screening of these individuals. New generations of DNA sequencing technologies facilitate the development of comprehensive genetic testing in PCC/PGL at a lower cost. Whole-exome sequencing and targeted NGS are the preferred methods for screening of PCC/PGL, both having precise mutation detection methods and low costs. RNA sequencing and DNA methylation studies using NGS technology in PCC/PGL can be adopted to act as diagnostic or prognostic biomarkers as well as in planning targeted epigenetic treatment of patients with PCC/PGL. The designs of NGS having a high depth of coverage and robust analytical pipelines can lead to the successful detection of a wide range of genomic defects in PCC/PGL. Nevertheless, the major challenges of this technology must be addressed before it has practical applications in the clinical diagnostics to fulfill the goal of personalized medicine in PCC/PGL. In future, novel approaches of sequencing, such as third and fourth generation sequencing can alter the workflow, cost, analysis, and interpretation of genomics associated with PCC/PGL. Copyright © 2017 Elsevier B.V. All rights reserved.

HIF-2alpha: Achilles' heel of pseudohypoxic subtype paraganglioma and other related conditions.

PubMed

Tella, Sri Harsha; Taïeb, David; Pacak, Karel

2017-11-01

Paragangliomas (PGLs) belong to the most hereditary endocrine tumours. The existence of mutated HIF2A in these tumours, the role of oncometabolites on HIFs stabilisation and a recent concept proposing how hereditary PGLs converge on the hypoxia-signalling pathway, brought solid evidence of the existence of PGL hypoxiom. Hypoxia-inducible factor 2alpha (HIF-2α) antagonists -PT2385, and PT2399 have been shown to have promising results in the management of clear cell renal cell carcinoma by targeting the HIF-2α pathway in recent and ongoing clinical trials (PT2799). The main aim of this perspective is to address the possibility of HIF-2α antagonists in the management of tumours, beyond clear cell renal cell carcinoma, where the dysfunctional hypoxia-signalling pathway, especially HIF-2α, referred here as the Achilles' heel, plays a unique role in tumorigenesis and other disorders. These tumours or disorders include PGLs, somatostatinomas, hemangioblastomas, gastrointestinal stromal tumours, pituitary tumours, leiomyomas/leiomyosarcomas, polycythaemia and retinal abnormalities. We hope that HIF-2α antagonists are likely to emerge as a potential effective treatment of choice for HIF-2α-related tumours and disorders. Copyright © 2017. Published by Elsevier Ltd.

Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients.

PubMed

Walz, Martin K; Iova, Laura D; Deimel, Judith; Neumann, Hartmut P H; Bausch, Birke; Zschiedrich, Stefan; Groeben, Harald; Alesina, Pier F

2018-04-01

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. In the setting of a prospective study (1/2001-12/2016), 42 consecutive children and adolescents (33 m, 9 f) were operated on. Thirty-seven patients (88%) suffered from inherited diseases. Twenty-six patients had PH, 11 presented retroperitoneal PGL, and five patients suffered from both. Altogether, 70 tumours (mean size 2.7 cm) were removed (45 PH, 25 PGL). All operations were performed by a minimally invasive access (retroperitoneoscopic, laparoscopic, extraperitoneal). Partial adrenalectomy was the preferred procedure for PH (31 out of 39 patients). Twenty patients received α-receptor blockade preoperatively. One patient died after induction of anaesthesia due to cardiac arrest. All other complications were minor. Conversion to open surgery was necessary in two cases with PGL. Median operating time for unilateral PH was 55 min, in bilateral cases 125, 143 min in PGs, and 180 min in combined cases. Median blood loss was 20 ml (range 0-1000). Blood transfusion was necessary in two cases. Intraoperative, systolic peak pressure was 170 ± 39 mmHg with α-receptor blockade and 191 ± 33 mmHg without α-receptor blockade (p = 0.41). The median post-operative hospital stay was 3 days. After a mean follow-up of 8.5 years, two patients presented ipsilateral recurrence (after partial adrenalectomy). All patients with bilateral PH (n = 13) are steroid independent post-operatively. PH and PGL in children and adolescents should preferably be removed by minimally invasive surgery. Partial adrenalectomy provides long-term steroid independence

European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma.

PubMed

Plouin, P F; Amar, L; Dekkers, O M; Fassnacht, M; Gimenez-Roqueplo, A P; Lenders, J W M; Lussey-Lepoutre, C; Steichen, O

2016-05-01

Phaeochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours. Standard treatment is surgical resection. Following complete resection of the primary tumour, patients with PPGL are at risk of developing new tumoural events. The present guideline aims to propose standardised clinical care of long-term follow-up in patients operated on for a PPGL. The guideline has been developed by The European Society of Endocrinology and based on the Grading of Recommendations Assessment, Development and Evaluation (GRADE) principles. We performed a systematic review of the literature and analysed the European Network for the Study of Adrenal Tumours (ENS@T) database. The risk of new events persisted in the long term and was higher for patients with genetic or syndromic diseases. Follow-up in the published cohorts and in the ENS@T database was neither standardised nor exhaustive, resulting in a risk of follow-up bias and in low statistical power beyond 10 years after complete surgery. To inform patients and care providers in this context of low-quality evidence, the Guideline Working Group therefore prepared recommendations on the basis of expert consensus. Key recommendations are the following: we recommend that all patients with PPGL be considered for genetic testing; we recommend assaying plasma or urinary metanephrines every year to screen for local or metastatic recurrences or new tumours; and we suggest follow-up for at least 10 years in all patients operated on for a PPGL. High-risk patients (young patients and those with a genetic disease, a large tumour and/or a paraganglioma) should be offered lifelong annual follow-up. © 2016 European Society of Endocrinology.

Additional value of hybrid SPECT/CT systems in neuroendocrine tumors, adrenal tumors, pheochromocytomas and paragangliomas.

PubMed

Wong, K K; Chondrogiannis, S; Fuster, D; Ruiz, C; Marzola, M C; Giammarile, F; Colletti, P M; Rubello, D

The aim of this review was to evaluate the potential advantages of SPECT/CT hybrid imaging in the management of neuroendocrine tumors, adrenal tumors, pheochromocytomas and paragangliomas. From the collected data, the superiority of fused images was observed as providing both functional/molecular and morphological imaging compared to planar imaging. This provided an improvement in diagnostic imaging, with significant advantages as regards: (1) precise locating of the lesions; (2) an improvement in characterization of the findings, resulting higher specificity, improved sensitivity, and overall greater accuracy, (3) additional anatomical information derived from the CT component; (4) CT-based attenuation correction and potential for volumetric dosimetry calculations, and (5) improvement on the impact on patient management (e.g. in better defining treatment plans, in shortening surgical operating times). It can be concluded that SPECT/CT hybrid imaging provides the nuclear medicine physician with a powerful imaging modality in comparison to planar imaging, providing essential information about the location of lesions, and high quality homogeneous images. Copyright © 2016 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

['Barbie Doll Syndrome'. A case report of body dysmorphic disorder].

PubMed

Gruber, Maria; Jahn, Rebecca; Stolba, Karin; Ossege, Michael

2018-03-01

This case report aims to present a 37-year-old women striving to shape her body like a Barbie doll of which she has been fascinated since childhood. She could hardly tolerate any deviation from this beauty ideal. She has been admitted to the psychosomatic ward due to an eating disorder. The ICD-10 and DSM-5 criteria were established for axis I disorders and the German version of the SCID II interview (for DSM-4) was applied for axis II disorders. Additionally, the "modified Yale-Brown Obsessive Compulsive Scale for body dysmorphic disorder" was carried out. The diagnosis of dysmorphophobia (ICD-10: F45.21) or body dysmorphic disorder (DSM-5: 300.7) and bulimia nervosa (ICD-10: F50.2; DSM-5: 307.51) was confirmed. The patient fulfilled criteria of an avoidant, depressive and histrionic personality disorder. Psychopharmacological treatment with Fluoxetine was started and the patient participated in an intensive inpatient psychosomatic program. The body image, self-concept and the sense of shame were therapeutic key topics. The present case report focuses on body dysmorphic disorder as a distinctive entity with high prevalence. Diagnostic criteria of different classification systems were contrasted and comorbidity with eating disorders was discussed. In clinical praxis, body dysmorphic disorder remains underdiagnosed, especially when cooccurring with an eating disorder. However, the correct diagnosis could be relevant for therapy planning.

Isolated displaced non-union of a triquetral body fracture: a case report

PubMed Central

2012-01-01

Introduction Fractures of the body of the triquetral bone are the second most common carpal fractures, and these fractures can be missed on plain X-ray. Although non-union of triquetral body fractures is very rare, such cases are associated with considerable morbidity and reduction in functional activity. Case presentation We report the case of a 29-year-old Caucasian British man who sustained an isolated displaced triquetral body fracture that resulted in non-union, who was treated surgically. We describe an original operative management for this debilitating injury. An open reduction and internal fixation using double headed compression screws was performed, without bone grafting, and with early immobilization of the wrist. Conclusions We propose this novel approach and advocate early clinical suspicion of triquetral body fractures in patients with a history of fall on an outstretched hand and ulnar sided wrist pain. We recommend evaluation using computed tomography or magnetic resonance imaging scanning. PMID:22325450

Complete body-neck torsion of the gallbladder: a case report.

PubMed

Pu, Ta-Wei; Fu, Chun-Yu; Lu, Huai-En; Cheng, Wei-Tung

2014-10-14

Gallbladder torsion is a rare, acute abdominal disease. It was first reported by Wendell in 1898. Since then, only 500 cases have been reported. Gallbladder torsion occurs in all age groups, although it usually appears in the latter stages of life. The occurrence ratio between women and men is 3:1. Most cases are diagnosed during surgery. The main treatment is surgical detorsion and cholecystectomy. Despite progress in radiologic imaging diagnosis, it is not easy to obtain a precise preoperative diagnosis of gallbladder torsion. In previous reports, only 9.8% of all gallbladder torsion cases were diagnosed preoperatively. We present a case of acute body-neck gallbladder torsion in an elderly man, and we review the radiologic findings of magnetic resonance imaging, computed tomography, and ultrasonography. The radiologic findings in the present case were helpful in obtaining a preoperative diagnosis of gallbladder torsion. The diagnosis was confirmed by T2-weighted magnetic resonance images, which showed an intra-gallbladder segment located between the body and neck of the gallbladder, with a notable crease within this segment.

An unusual autopsy case of lethal hypothermia exacerbated by body lice-induced severe anemia.

PubMed

Nara, Akina; Nagai, Hisashi; Yamaguchi, Rutsuko; Makino, Yohsuke; Chiba, Fumiko; Yoshida, Ken-ichi; Yajima, Daisuke; Iwase, Hirotaro

2016-05-01

Pediculus humanus humanus (known as body lice) are commonly found in the folds of clothes, and can cause skin disorders when they feed on human blood, resulting in an itching sensation. Body lice are known as vectors of infectious diseases, including typhus, recurrent fever, and trench fever. An infestation with blood-sucking body lice induces severe cutaneous pruritus, and this skin disorder is known as "vagabond's disease." A body lice infestation is sometimes complicated with iron deficiency anemia. In the present case, a man in his late 70s died of lethal hypothermia in the outdoors during the winter season. The case history and autopsy findings revealed that the cause of the lethal hypothermia was iron deficiency anemia, which was associated with a prolonged infestation of blood-sucking body lice. Also, he had vagabond's disease because the skin on his body was abnormal and highly pigmented. This is an unusual autopsy case since the body lice contributed to the cause of the death.

Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma.

PubMed

Thosani, Sonali; Ayala-Ramirez, Montserrat; Román-González, Alejandro; Zhou, Shouhao; Thosani, Nirav; Bisanz, Annette; Jimenez, Camilo

2015-09-01

Pheochromocytomas (PHs) and sympathetic paragangliomas (PGs) are tumors that produce catecholamines, predisposing patients to cardiovascular disease and gastrointestinal effects such as constipation. i) determine the prevalence of constipation, its risk factors, and its impact on survival; ii) identify whether a systematic combination of fiber, water, and laxatives was effective for treatment of constipation. We retrospectively studied 396 patients with PH/PG diagnosed in 2005-2014. The study population was patients with constipation as a presenting symptom; the control group was patients without constipation as a presenting symptom. The MD Anderson Symptom Inventory was used to assess constipation and quality of life. Twenty-three patients (6%) had constipation. Constipation was associated with headaches, palpitations, diaphoresis, weight loss, and excessive noradrenaline production (P<0.0001). Eighteen of these patients had non-metastatic primary tumors larger than 5 cm and/or extensive metastases. No statistically significant differences in age, sex, and genotype were noted between the study and control groups. In patients without metastases, resection of the primary tumor led to symptom disappearance. A systematic combination of fiber, water, and laxatives was associated with symptom improvement. Two patients who presented unmanaged constipation died because of sepsis from toxic megacolon. Constipation is a rare and potentially lethal complication in patients with PH/PGs. Severe constipation can be prevented by recognizing and treating mild symptoms. © 2015 European Society of Endocrinology.

Cranial injury caused by penetrating non-missile foreign body: an autopsy case.

PubMed

Eren, B; Türkmen, N; Toprak Ergönen, A; Gündogmus, U N

2012-10-01

Presented case was 32-year-old male marble worker, who underwent industrial accident at workplace. On gross physical examination; on forehead region round skin wound in 0.9 cm diameter was detected, radiological examination showed the image of metallic object. in the skull cavity. Brain dissection showed obvious brain injury, haemorrahge explaining the pattern of injury caused by the metallic bodys path, from left frontal lobe to the left cerebellar hemisphere was identified. We presented rare case of penetrating injury of the cranial region caused by non-missile foreign body. cranial injury - non-missile foreign body - autopsy.

SDHB-related pheochromocytoma and paraganglioma penetrance and genotype-phenotype correlations.

PubMed

Jochmanova, Ivana; Wolf, Katherine I; King, Kathryn S; Nambuba, Joan; Wesley, Robert; Martucci, Victoria; Raygada, Margarita; Adams, Karen T; Prodanov, Tamara; Fojo, Antonio Tito; Lazurova, Ivica; Pacak, Karel

2017-08-01

Succinate dehydrogenase subunit B (SDHB) gene mutations are associated with an aggressive clinical disease course of pheochromocytoma/paraganglioma (PHEO/PGL). Limited information is available concerning PHEO/PGL penetrance among SDHB mutation carriers with regards to primary tumor location, specific mutation type, and gender. We assessed PHEO/PGL penetrance in SDHB mutation carriers and described the clinical presentation and disease course. Asymptomatic relatives (N = 611) of 103 index patients were tested for SDHB mutations. Mutation carriers (N = 328) were offered PHEO/PGL screening, of which 241 participated and were included in penetrance analysis. For additional disease outcome analysis, the 103 index patients and 40 screened individuals who developed PHEO/PGL were included. Clinical data were collected between October 2004 and June 2016. Forty (16.60%) of the 241 screened individuals developed PHEO/PGL during the study. The penetrance estimate in this population was 49.80% (95% CI 29-74.9) at 85 years. A significantly higher age-related penetrance of disease was observed in males compared to females, with 50% penetrance achieved at age 74 vs. not reached. Age-related penetrance analysis demonstrated 4 mutations (Ile127Ser, IVS1+1G>T, Exon 1 deletion, Arg90X) presenting with a slower rate of disease development (50% penetrance ages, respectively: not achieved, 70, 63, 61 years) compared to Arg46X and Val140Phe mutations (50% penetrance at 38 years). Here, we found a higher estimated penetrance compared to several other studies, and a striking difference in age-related penetrance between male and female SDHB mutation carriers with no association between mutation and gender or tumor location.

Alopecia with foreign body granulomas induced by Radiesse injection: A case report.

PubMed

Liu, Ren-Feng; Kuo, Tseng-Tong; Chao, Yen-Yu; Huang, Yu-Huei

2018-02-26

Radiesse is a soft tissue filler which has been widely used for cosmetic enhancement. The safety of Radiesse has been thoroughly investigated via numerous studies. A late-onset complication of Radiesse injection consists of foreign body granulomas, with only three case reports in over 10 years of clinical use. Herein, we describe the case of a patient who experienced alopecia with foreign body granulomas at the injection region one month after receiving a Radiesse injection. To our knowledge, this is the first report in the English literature of alopecia as an adverse event associated with Radiesse injection. The present case reminded physicians to evaluate more cautiously the necessity of injecting filler into hair-bearing area for lifting purpose. This procedure may cause foreign body granulomatous reaction, which may result in hair loss at the injection region.

Surgical intervention for gastrointestinal foreign bodies in adults: a case series.

PubMed

Syrakos, Theodoros; Zacharakis, Emmanouil; Antonitsis, Polichronis; Zacharakis, Evangelos; Spanos, Constantinos; Georgantis, G; Kiskinis, Dimitrios

2008-01-01

The aim of our study was to demonstrate our experience regarding the surgical treatment of complications after foreign body ingestion. From 1997 to 2005, we treated 16 adult patients (mean age 44.8 years, range 21-77), who presented with complications after foreign body ingestion. The complications treated were perforation-peritonitis: n = 7 (44%), intra-abdominal abscess formation: n = 5 (31%), upper gastrointestinal bleeding: n = 3 (19%) and inflammatory mass formation: n = 1 (6%) patient. The diagnosis was made intra-operatively in 13 (81.3%) of the cases. The commonest anatomical position of the perforation was the large bowel, in 7 (43%) of the cases. Bony food parts were the commonest foreign bodies accidentally swallowed, in 9 (56%) patients. The median hospital stay was 7 days (2-18), while no death occurred in the postoperative period among the patients of the study. The postoperative morbidity rate was 22.2%. The most common complication after foreign body ingestion was the perforation of the gastrointestinal tract. The risk of perforation was higher when sharp foreign bodies were ingested. The pre-operative diagnosis was difficult, and it was usually achieved intra-operatively. (c) 2008 S. Karger AG, Basel

Acute coronary syndrome: a rare case of multiple endocrine neoplasia syndromes with pheochromocytoma and medullary thyroid carcinoma

PubMed Central

Maloberti, Alessadro; Meani, Paolo; Pirola, Roberto; Varrenti, Marisa; Boniardi, Marco; De Biase, Anna Maria; Vallerio, Paola; Bonacina, Edgardo; Mancia, Giuseppe; Loli, Paola; Giannattasio, Cristina

2015-01-01

Pheochromocytoma is a tumor arising from neuroectodermal chromaffin tissues in the adrenal gland or extra-adrenal paraganglia (paragangliomas). The prevalence of the tumor is 0.1%-0.6% in the hypertensive population, of which 10%-20% are malignant. Pheochromocytoma produces, stores, and secretes catecholamines, as well as leads to hypertensive crisis, arrhythmia, angina, and acute myocardial infarction without coronary artery diseases. We report a case of acute coronary syndrome (ACS) with a final diagnosis of multiple endocrine neoplasia with pheochromocytoma and medullary thyroid carcinoma (MTC). PMID:26487970

Acute coronary syndrome: a rare case of multiple endocrine neoplasia syndromes with pheochromocytoma and medullary thyroid carcinoma.

PubMed

Maloberti, Alessadro; Meani, Paolo; Pirola, Roberto; Varrenti, Marisa; Boniardi, Marco; De Biase, Anna Maria; Vallerio, Paola; Bonacina, Edgardo; Mancia, Giuseppe; Loli, Paola; Giannattasio, Cristina

2015-09-01

Pheochromocytoma is a tumor arising from neuroectodermal chromaffin tissues in the adrenal gland or extra-adrenal paraganglia (paragangliomas). The prevalence of the tumor is 0.1%-0.6% in the hypertensive population, of which 10%-20% are malignant. Pheochromocytoma produces, stores, and secretes catecholamines, as well as leads to hypertensive crisis, arrhythmia, angina, and acute myocardial infarction without coronary artery diseases. We report a case of acute coronary syndrome (ACS) with a final diagnosis of multiple endocrine neoplasia with pheochromocytoma and medullary thyroid carcinoma (MTC).

Topological classification of the Goryachev integrable case in rigid body dynamics

DOE Office of Scientific and Technical Information (OSTI.GOV)

Nikolaenko, S S

2016-01-31

A topological analysis of the Goryachev integrable case in rigid body dynamics is made on the basis of the Fomenko-Zieschang theory. The invariants (marked molecules) which are obtained give a complete description, from the standpoint of Liouville classification, of the systems of Goryachev type on various level sets of the energy. It turns out that on appropriate energy levels the Goryachev case is Liouville equivalent to many classical integrable systems and, in particular, the Joukowski, Clebsch, Sokolov and Kovalevskaya-Yehia cases in rigid body dynamics, as well as to some integrable billiards in plane domains bounded by confocal quadrics -- in othermore » words, the foliations given by the closures of generic solutions of these systems have the same structure. Bibliography: 15 titles.« less

Isolated displaced non-union of a triquetral body fracture: a case report.

PubMed

Rasoli, Sonia; Ricks, Matthew; Packer, Greg

2012-02-10

Fractures of the body of the triquetral bone are the second most common carpal fractures, and these fractures can be missed on plain X-ray. Although non-union of triquetral body fractures is very rare, such cases are associated with considerable morbidity and reduction in functional activity. We report the case of a 29-year-old Caucasian British man who sustained an isolated displaced triquetral body fracture that resulted in non-union, who was treated surgically. We describe an original operative management for this debilitating injury. An open reduction and internal fixation using double headed compression screws was performed, without bone grafting, and with early immobilization of the wrist. We propose this novel approach and advocate early clinical suspicion of triquetral body fractures in patients with a history of fall on an outstretched hand and ulnar sided wrist pain. We recommend evaluation using computed tomography or magnetic resonance imaging scanning.

Effects of PPARα inhibition in head and neck paraganglioma cells

PubMed Central

Florio, Rosalba; di Giacomo, Viviana; Di Marcantonio, Maria Carmela; Cristiano, Loredana; Basile, Mariangela; Verginelli, Fabio; Verzilli, Delfina; Ammazzalorso, Alessandra; Prasad, Sampath Chandra; Cataldi, Amelia; Sanna, Mario; Cimini, Annamaria; Mariani-Costantini, Renato; Mincione, Gabriella; Cama, Alessandro

2017-01-01

Head and neck paragangliomas (HNPGLs) are rare tumors that may cause important morbidity, because of their tendency to infiltrate the skull base. At present, surgery is the only therapeutic option, but radical removal may be difficult or impossible. Thus, effective targets and molecules for HNPGL treatment need to be identified. However, the lack of cellular models for this rare tumor hampers this task. PPARα receptor activation was reported in several tumors and this receptor appears to be a promising therapeutic target in different malignancies. Considering that the role of PPARα in HNPGLs was never studied before, we analyzed the potential of modulating PPARα in a unique model of HNPGL cells. We observed an intense immunoreactivity for PPARα in HNPGL tumors, suggesting that this receptor has an important role in HNPGL. A pronounced nuclear expression of PPARα was also confirmed in HNPGL-derived cells. The specific PPARα agonist WY14643 had no effect on HNPGL cell viability, whereas the specific PPARα antagonist GW6471 reduced HNPGL cell viability and growth by inducing cell cycle arrest and caspase-dependent apoptosis. GW6471 treatment was associated with a marked decrease of CDK4, cyclin D3 and cyclin B1 protein expression, along with an increased expression of p21 in HNPGL cells. Moreover, GW6471 drastically impaired clonogenic activity of HNPGL cells, with a less marked effect on cell migration. Notably, the effects of GW6471 on HNPGL cells were associated with the inhibition of the PI3K/GSK3β/β-catenin signaling pathway. In conclusion, the PPARα antagonist GW6471 reduces HNPGL cell viability, interfering with cell cycle and inducing apoptosis. The mechanisms affecting HNPGL cell viability involve repression of the PI3K/GSK3β/β-catenin pathway. Therefore, PPARα could represent a novel therapeutic target for HNPGL. PMID:28594934

Effects of PPARα inhibition in head and neck paraganglioma cells.

PubMed

Florio, Rosalba; De Lellis, Laura; di Giacomo, Viviana; Di Marcantonio, Maria Carmela; Cristiano, Loredana; Basile, Mariangela; Verginelli, Fabio; Verzilli, Delfina; Ammazzalorso, Alessandra; Prasad, Sampath Chandra; Cataldi, Amelia; Sanna, Mario; Cimini, Annamaria; Mariani-Costantini, Renato; Mincione, Gabriella; Cama, Alessandro

2017-01-01

Head and neck paragangliomas (HNPGLs) are rare tumors that may cause important morbidity, because of their tendency to infiltrate the skull base. At present, surgery is the only therapeutic option, but radical removal may be difficult or impossible. Thus, effective targets and molecules for HNPGL treatment need to be identified. However, the lack of cellular models for this rare tumor hampers this task. PPARα receptor activation was reported in several tumors and this receptor appears to be a promising therapeutic target in different malignancies. Considering that the role of PPARα in HNPGLs was never studied before, we analyzed the potential of modulating PPARα in a unique model of HNPGL cells. We observed an intense immunoreactivity for PPARα in HNPGL tumors, suggesting that this receptor has an important role in HNPGL. A pronounced nuclear expression of PPARα was also confirmed in HNPGL-derived cells. The specific PPARα agonist WY14643 had no effect on HNPGL cell viability, whereas the specific PPARα antagonist GW6471 reduced HNPGL cell viability and growth by inducing cell cycle arrest and caspase-dependent apoptosis. GW6471 treatment was associated with a marked decrease of CDK4, cyclin D3 and cyclin B1 protein expression, along with an increased expression of p21 in HNPGL cells. Moreover, GW6471 drastically impaired clonogenic activity of HNPGL cells, with a less marked effect on cell migration. Notably, the effects of GW6471 on HNPGL cells were associated with the inhibition of the PI3K/GSK3β/β-catenin signaling pathway. In conclusion, the PPARα antagonist GW6471 reduces HNPGL cell viability, interfering with cell cycle and inducing apoptosis. The mechanisms affecting HNPGL cell viability involve repression of the PI3K/GSK3β/β-catenin pathway. Therefore, PPARα could represent a novel therapeutic target for HNPGL.

Apotemnophilia or body integrity identity disorder: a case report review.

PubMed

Bou Khalil, Rami; Richa, Sami

2012-12-01

Apotemnophilia or body integrity identity disorder (BIID) denotes a syndrome in which a person is preoccupied with the desire to amputate a healthy limb. In this report, we review the available case reports in the literature in order to enhance psychiatrists' and physicians' comprehension of this disorder. A search for the case reports available via MEDLINE was done since the first case report published by Money et al in 1977 till May 2011, using the following terms: apotemnophilia, self-demand amputation, body integrity identity disorder, and BIID. In all, 14 case reports were found relevant to our search. The desire to amputate one's healthy limb seems to be related to a major disturbance in the person's perception of one's own identity, where limb amputation can relieve temporarily the patient's feeling of distress without necessarily and uniformly adjusting the patient's own identity misperception. More investigations are needed in this domain in order to develop noninvasive treatment strategies that approach this aspect of the patient's distress within a globalist perspective. In addition, the health professionals' awareness regarding this disorder is required to ensure professional management of patients' suffering.

Button battery intake as foreign body in Chinese children: review of case reports and the literature.

PubMed

Liao, Wenjing; Wen, Guangyi; Zhang, Xiaowen

2015-06-01

Button batteries have been recognized as one of the dangerous foreign bodies to children for more than 30 years, but few related studies have been published to give more concern in China. We reported 6 cases of button battery intake as foreign body in children. The Chinese literature on button battery as foreign body in children was reviewed. The interval between the accidental ingestion and battery removal ranged from 6 hours to 3 days. Two patients had no sequela, 3 patients had tracheoesophageal fistulas, and 1 patient had nasal septal perforation. Twenty-eight articles about button battery as foreign body in children were obtained by Chinese-language literature searches including 25 case reports, 2 health education articles, and 1 imaging article. In total, 172 cases of button battery intake as foreign body in children were identified, 23 and 10 of the 159 cases involving nasal button battery lodgment developed nasal septal perforation and nasal adhesion, respectively. Tracheoesophageal fistula was identified in 4 of the 12 ingestion cases. One case of button battery intake was in external auditory canal. A small number of children with button battery as foreign body were reported in China, which is 1 of the biggest countries with large population of children.

Silicone impression material foreign body in the middle ear: Two case reports and literature review.

PubMed

Suzuki, Nobuyoshi; Okamura, Koji; Yano, Takuya; Moteki, Hideaki; Kitoh, Ryosuke; Takumi, Yutaka; Usami, Shin-ichi

2015-10-01

We report two cases of impression material foreign body in the middle ear. The first case had been affected with chronic otitis media. The silicone flowed into the middle ear through a tympanic membrane perforation during the process of making an ear mold. About 4 years and 8 months after, the patient had severe vertigo and deafness. We found bone erosion of the prominence of the lateral semicircular canal and diagnosed labyrinthitis caused by silicone impression material. In the second case silicone flowed into the canal wall down mastoid cavity. Both cases required surgery to remove the foreign body. The clinical courses in such cases are variable and timing of surgery is sometimes difficult. In addition to reporting these two cases, we present here a review of the literature regarding impression material foreign bodies. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

18F-FDG avidity of pheochromocytomas and paragangliomas: a new molecular imaging signature?

PubMed

Taïeb, David; Sebag, Frederic; Barlier, Anne; Tessonnier, Laurent; Palazzo, Fausto F; Morange, Isabelle; Niccoli-Sire, Patricia; Fakhry, Nicolas; De Micco, Catherine; Cammilleri, Serge; Enjalbert, Alain; Henry, Jean-François; Mundler, Olivier

2009-05-01

Our objective was to evaluate (18)F-FDG PET uptake in patients with nonmetastatic and metastatic chromaffin-derived tumors. Twenty-eight consecutive unrelated patients with chromaffin tumors, including 9 patients with genetically determined disease, were studied. A combination of preoperative imaging work-up, surgical findings, and pathologic analyses was used to classify the patients into 2 groups: those with nonmetastatic disease (presumed benign, n = 18) and those with metastatic tumors (n = 10). (18)F-FDG PET was performed in all cases. Visual and quantitative analyses were individually graded for each tumor. Somatic mutations of the succinate dehydrogenase subunits B and D and Von-Hippel Lindau genes were also evaluated in 6 benign sporadic tumor samples. All but 2 patients showed significantly increased (18)F-FDG uptake on visual analysis. The maximum standardized uptake value (SUVmax) ranged from 1.9 to 42 (mean +/- SD, 8.2 +/- 9.7; median, 4.6) in nonmetastatic tumors and 2.3 to 29.3 (mean +/- SD, 9.7 +/- 8.4; median, 7.4) in metastatic tumors. No statistical difference was observed between the groups (P = 0.44), but succinate dehydrogenase-related tumors were notable in being the most (18)F-FDG-avid tumors (SUVmax, 42, 29.3, 21, 17, and 5.3). Succinate dehydrogenase and Von-Hippel Lindau-related tumors had a significantly higher SUVmax than did neurofibromatosis type 1 and multiple endocrine neoplasia type 2A syndrome-related tumors (P = 0.02). (18)F-FDG PET was superior to (131)I-metaiodobenzylguanidine in all metastatic patients but one. By contrast, (18)F-FDG PET underestimated the extent of the disease, compared with 6-(18)F-fluorodopa PET, in 5 patients with metastatic pheochromocytoma. However, succinate dehydrogenase mutations (germline and somatic) and functional dedifferentiation do not adequately explain (18)F-FDG uptake since most tumors were highly avid for (18)F-FDG. (18)F-FDG PET positivity is almost a constant feature of pheochromocytomas

All the wrong places: an unusual case of foreign body ingestion and inhalation.

PubMed

Tammana, V S; Valluru, N; Sanderson, A

2012-09-01

Intentional ingestion of foreign bodies is common in psychiatric patients and prison inmates. Timing of endoscopy for ingested foreign bodies varies and depends on the type and location of the foreign body in the gastrointestinal tract. We present the case of a 26-year-old man who was brought from a correctional facility after confessing to have swallowed a few shower curtain hooks. Abdominal X-ray done in the emergency room revealed multiple foreign bodies in the stomach. An upper endoscopy was done in the emergency room with the use of an overtube. The first metal piece was caught by a snare and removed with the endoscope. All other foreign bodies which were present on the abdominal X-ray could not be visualized initially as there was retained food in the stomach. After multiple attempts, four other foreign bodies were found and each one was caught by the forceps and then the scope was removed with the forceps holding the foreign body. There was an additional foreign body in the right mainstem bronchus. The patient had coughed up the foreign body and swallowed it into the gastrointestinal tract. A computed tomography scan of chest and abdomen was done for evaluation, which showed the foreign body in the cecum. To our knowledge, this is the first case report of a patient intentionally transferring a foreign body from one organ system to another. Colonoscopy was done and the foreign body was removed rectally with a snare without any complications.

World Trade Center human identification project: experiences with individual body identification cases.

PubMed

Budimlija, Zoran M; Prinz, Mechthild K; Zelson-Mundorff, Amy; Wiersema, Jason; Bartelink, Eric; MacKinnon, Gaille; Nazzaruolo, Bianca L; Estacio, Sheila M; Hennessey, Michael J; Shaler, Robert C

2003-06-01

To present individual body identification efforts, as part of the World Trade Center (WTC) mass disaster identification project. More than 500 samples were tested by using polymerase chain reaction (PCR) amplification and short tandem repeat (STR) typing. The extent to which the remains were fragmented and affected by taphonomic factors complicated the identification project. Anthropologists reviewed 19,000 samples, and detected inconsistencies in 69, which were further split into 239 new cases and re-sampled by DNA specialists. The severity and nature of the disaster required an interdisciplinary effort. DNA profiling of 500 samples was successful in 75% of the cases. All discrepancies, which occurred between bone and tissue samples taken from the same body part, were resolved by re-sampling and re-testing of preferably bone tissue. Anthropologists detected inconsistencies in 69 cases, which were then split into 239 new cases. Out of 125 "split" cases, 65 were excluded from their original case. Of these 65 cases, 37 did not match any profiles in M-FISys, probably because profiles were incomplete or no exemplar for the victim was available. Out of the 60 remains not excluded from their original case, 30 were partial profiles and did not reach the statistical requirement to match their original case, because the population frequency of the DNA profile had to be body parts. Especially in disasters with a high potential for commingling, the described anthropological review process should be part of the investigation.

[A Case of Uterine Body Metastasis from Sigmoid Colon Adenocarcinoma].

PubMed

Mayumi, Katsuyuki; Terakura, Masanobu; Hori, Takaaki; Takemura, Masashi

2017-11-01

We report a case of metastatic carcinoma to the uterine body from a colorectal adenocarcinoma. A 73-year-old woman underwent laparoscopic sigmoidectomy for sigmoid colon carcinoma 2 years before. In the following study, her serum carcinoembryonic antigen level was elevated, and a uterine body tumor invading the rectal wall was detected via enhanced computed tomography. Colonoscopic examination revealed an elevated lesion at the rectum, which was diagnosed as an adenocarcinoma. Based on these results, we diagnosed the uterine tumor as metastatic tumor from the colon carcinoma. Immunostaining was negative for CK7, but positive for CK20. Thus, we confirmed metastasis of the sigmoid colon cancer to the uterus. Metastasis to the female genital tract from extragenital malignancies are rare, and the prognosis is extremely poor. However, some patients attain long-term survival by surgical intervention even in such cases.

Updates on the genetics and the clinical impacts on phaeochromocytoma and paraganglioma in the new era.

PubMed

Pillai, Suja; Gopalan, Vinod; Smith, Robert A; Lam, Alfred K-Y

2016-04-01

Genetic mutations of phaeochromocytoma (PCC) and paraganglioma (PGL) are mainly classified into two major clusters. Cluster 1 mutations are involved with the pseudo hypoxic pathway and comprised of PHD2, VHL, SDHx, IDH, HIF2A, MDH2 and FH mutated PCC/PGL. Cluster 2 mutations are associated with abnormal activation of kinase signalling pathways and included mutations of RET, NF1, KIF1Bβ, MAX and TMEM127. In addition, VHL, SDHx (cluster 1 genes) and RET, NF1 (cluster 2 genes) germline mutations are involved in the neuronal precursor cell pathway in the pathogeneses of PCC/PGL. Also, GDNF, H-ras, K-ras, GNAS, CDKN2A (p16), p53, BAP1, BRCA1&2, ATRX and KMT2D mutations have roles in the development of PCC/PGLs. Overall, known genetic mutations account for the pathogenesis of approximately 60% of PCC/PGLs. Genetic mutations, pathological parameters and biochemical markers are used for better prediction of the outcome of patients with this group of tumours. Immunohistochemistry and gene sequencing can ensure a more effective detection, prediction of malignant potential and treatment of PCC/PCLs. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Molecular and Therapeutic Advances in the Diagnosis and Management of Malignant Pheochromocytomas and Paragangliomas

PubMed Central

Lowery, Aoife J.; Walsh, Siun; McDermott, Enda W.

2013-01-01

Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies. PMID:23576482

Migrating pharyngeal foreign bodies: a series of four cases of saw-toothed fish bones.

PubMed

Chung, Sung Min; Kim, Han Su; Park, Eun Hee

2008-09-01

Pharyngeal foreign bodies are common problems seen at emergency rooms or ENT outpatient clinics, and fish bones are the most common foreign bodies encountered in East Asia and in Korea. One of the rare complications of a swallowed sharp fish bone is its migration from the site of entry into the subcutaneous tissues of the neck. We present four unusual cases of ingested fish bones that migrated out of the upper digestive tract to the neck. In the first case, this caused a recurrent deep neck infection for 2 years; in the second case, there was penetration of the facial artery; in the third case, there was a hematoma of the floor of the mouth; in the fourth case, there was a retropharyngeal abscess.

Heterogeneous Genetic Background of the Association of Pheochromocytoma/Paraganglioma and Pituitary Adenoma: Results From a Large Patient Cohort

PubMed Central

Dénes, Judit; Swords, Francesca; Rattenberry, Eleanor; Stals, Karen; Owens, Martina; Cranston, Treena; Xekouki, Paraskevi; Moran, Linda; Kumar, Ajith; Wassif, Christopher; Fersht, Naomi; Baldeweg, Stephanie E.; Morris, Damian; Lightman, Stafford; Agha, Amar; Rees, Aled; Grieve, Joan; Powell, Michael; Boguszewski, Cesar Luiz; Dutta, Pinaki; Thakker, Rajesh V.; Srirangalingam, Umasuthan; Thompson, Chris J.; Druce, Maralyn; Higham, Claire; Davis, Julian; Eeles, Rosalind; Stevenson, Mark; O'Sullivan, Brendan; Taniere, Phillipe; Skordilis, Kassiani; Gabrovska, Plamena; Barlier, Anne; Webb, Susan M.; Aulinas, Anna; Drake, William M.; Bevan, John S.; Preda, Cristina; Dalantaeva, Nadezhda; Ribeiro-Oliveira, Antônio; Garcia, Isabel Tena; Yordanova, Galina; Iotova, Violeta; Evanson, Jane; Grossman, Ashley B.; Trouillas, Jacqueline; Ellard, Sian; Stratakis, Constantine A.; Maher, Eamonn R.; Roncaroli, Federico

2015-01-01

Context: Pituitary adenomas and pheochromocytomas/paragangliomas (pheo/PGL) can occur in the same patient or in the same family. Coexistence of the two diseases could be due to either a common pathogenic mechanism or a coincidence. Objective: The objective of the investigation was to study the possible coexistence of pituitary adenoma and pheo/PGL. Design: Thirty-nine cases of sporadic or familial pheo/PGL and pituitary adenomas were investigated. Known pheo/PGL genes (SDHA-D, SDHAF2, RET, VHL, TMEM127, MAX, FH) and pituitary adenoma genes (MEN1, AIP, CDKN1B) were sequenced using next generation or Sanger sequencing. Loss of heterozygosity study and pathological studies were performed on the available tumor samples. Setting: The study was conducted at university hospitals. Patients: Thirty-nine patients with sporadic of familial pituitary adenoma and pheo/PGL participated in the study. Outcome: Outcomes included genetic screening and clinical characteristics. Results: Eleven germline mutations (five SDHB, one SDHC, one SDHD, two VHL, and two MEN1) and four variants of unknown significance (two SDHA, one SDHB, and one SDHAF2) were identified in the studied genes in our patient cohort. Tumor tissue analysis identified LOH at the SDHB locus in three pituitary adenomas and loss of heterozygosity at the MEN1 locus in two pheochromocytomas. All the pituitary adenomas of patients affected by SDHX alterations have a unique histological feature not previously described in this context. Conclusions: Mutations in the genes known to cause pheo/PGL can rarely be associated with pituitary adenomas, whereas mutation in a gene predisposing to pituitary adenomas (MEN1) can be associated with pheo/PGL. Our findings suggest that genetic testing should be considered in all patients or families with the constellation of pheo/PGL and a pituitary adenoma. PMID:25494863

Predictors of malignancy in patients with pheochromocytomas/paragangliomas: Asian Indian experience

PubMed Central

Sarathi, Vijaya; Kasaliwal, Rajeev; Pandit, Reshma; Goroshi, Manjunath; Malhotra, Gaurav; Dalvi, Abhay; Bakshi, Ganesh; Bhansali, Anil; Rajput, Rajesh; Shivane, Vyankatesh; Lila, Anurag; Bandgar, Tushar; Shah, Nalini S

2016-01-01

Background and aims Malignant transformation of pheochromocytomas/paragangliomas (PCC/PGL) is a rare occurrence, and predictive factors for the same are not well understood. This study aims to identify the predictors of malignancy in patients with PCC/PGL. Materials and methods We performed a retrospective analysis of 142 patients with either PCC or PGL registered at our institute between 2000 and 2015. Records were evaluated for clinical parameters like age, gender, familial/syndromic presentation, symptomatic presentation, biochemistry, size, number and location of tumours and presence of metastases and mode of its diagnosis. Results Twenty patients were found to have metastases; 13 had metastases at diagnosis and seven during follow-up. Metastases were detected by radiology (CT-neck to pelvis) in 11/20 patients (5/13 synchronous and 6/7 metachronous), 131I-metaiodobenzylguanidine in five (2/12 synchronous and 3/6 metachronous) patients and 18F-flurodeoxyglucose PET/CT in 15 (12/12 synchronous and 3/3 metachronous) patients. Malignant tumours were significantly larger than benign tumours (8.3 ± 4.1 cm, range: 3–22 cm vs 5.7 ± 2.3 cm, range: 2–14 cm, P = 0.0001) and less frequently metanephrine secreting. On linear regression analysis, tumour size and lack of metanephrine secretion were the independent predictors of malignancy. Conclusions Patients with primary tumour size >5.7 cm and lack of metanephrine secretory status should be evaluated for possible malignancy not only at diagnosis but also in the postoperative period. As compared to CT and 131I-MIBG scan, 18F-flurodeoxyglucose PET/CT analyses are better (sensitivity: 100%) for the diagnosis of metastases in our study. PMID:27852633

Surgical treatment of late-diagnosed bronchial foreign body aspiration: a report of 23 cases.

PubMed

Duan, Liang; Chen, Xiaofeng; Wang, Hao; Hu, Xuefei; Jiang, Gening

2014-07-01

Late-diagnosed bronchial foreign bodies can lead to irreversible changes in the bronchi and the lungs. To date, few reports are available concerning surgical treatment for this condition. The present report summarizes clinicopathological features and surgical treatment options for late-diagnosed bronchial foreign body aspiration. Clinical data of 23 patients who underwent surgical treatment for late-diagnosed bronchial foreign body aspiration in our hospital from 1980 to 2010 were reviewed. The patients included 17 male and six female patients, aged 6 to 66 years. To remove the foreign body, bronchotomy was performed in six patients and pulmonary resection in 17 patients. Foreign bodies were located on the right side in 17 cases and on the left side in six cases. The majority of the foreign bodies were of food origin. Postoperative bronchopleural fistula occurred in two patients. One patient died of renal failure subsequent to lung infections. Detailed history, awareness of potential foreign body aspiration and thorough bronchoscopic examination are helpful to avoid pulmonary resection. However, the latter is necessary in patients with the associated substantial lung lesions to prevent these from progressing into serious lung infections such as pulmonary suppuration or empyema. © 2013 John Wiley & Sons Ltd.

An unusual case of intraorbital foreign body and its management

PubMed Central

Mukherjee, Bipasha; Goel, Shubhra; Subramanian, Nirmala

2011-01-01

Intraorbital foreign bodies are usually the result of accidental trauma and can lead to considerable morbidity. We report an unusual case of an industrial injury in a plastic manufacuring unit wherein hot molten plastic splashed and solidified inside the orbit. The resultant increased intraorbital pressure led to loss of vision in that eye. The extreme temperature of the foreign body caused extensive thermal damage to the surrounding adnexal structures. Staged reconstructive surgery was undertaken to repair the damage, with an acceptable final cosmetic outcome. Employment of protective eye wear to prevent such accidents in high-risk occupations should be made mandatory. PMID:21157077

Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes.

PubMed

Janssen, Ingo; Chen, Clara C; Zhuang, Zhenping; Millo, Corina M; Wolf, Katherine I; Ling, Alexander; Lin, Frank I; Adams, Karen T; Herscovitch, Peter; Feelders, Richard A; Fojo, Antonio T; Taieb, David; Kebebew, Electron; Pacak, Karel

2017-08-01

Pheochromocytoma/paraganglioma (PPGL) syndromes associated with polycythemia have previously been described in association with mutations in the von Hippel-Lindau gene. Recently, mutations in the prolyl hydroxylase gene ( PHD ) 1 and 2 and in the hypoxia-inducible factor 2 α ( HIF2A ) were also found to be associated with multiple and recurrent PPGL. Such patients also presented with PPGL and polycythemia, and later on, some presented with duodenal somatostatinoma. In additional patients presenting with PPGL and polycythemia, no further mutations have been discovered. Because the functional imaging signature of patients with PPGL-polycythemia syndromes is still unknown, and because these tumors (in most patients) are multiple, recurrent, and metastatic, the goal of our study was to assess the optimal imaging approach using 4 different PET radiopharmaceuticals and CT/MRI in these patients. Methods: Fourteen patients (10 women, 4 men) with confirmed PPGL and polycythemia prospectively underwent 68 Ga-DOTATATE (13 patients), 18 F-FDG (13 patients), 18 F-fluorodihydroxyphenylalanine ( 18 F-FDOPA) (14 patients), 18 F-fluorodopamine ( 18 F-FDA) (11 patients), and CT/MRI (14 patients). Detection rates of PPGL lesions were compared between all imaging studies and stratified between the underlying mutations. Results: 18 F-FDOPA and 18 F-FDA PET/CT showed similar combined lesion-based detection rates of 98.7% (95% confidence interval [CI], 92.7%-99.8%) and 98.3% (95% CI, 90.9%-99.7%), respectively. The detection rates for 68 Ga-DOTATATE (35.3%; 95% CI, 25.0%-47.2%), 18 F-FDG (42.3; 95% CI, 29.9%-55.8%), and CT/MRI (60.3%; 95% CI, 48.8%-70.7%) were significantly lower ( P < 0.01), irrespective of the mutation status. Conclusion: 18 F-FDOPA and 18 F-FDA are superior to 18 F-FDG, 68 Ga-DOTATATE, and CT/MRI and should be the radiopharmaceuticals of choice in this rare group of patients. © 2017 by the Society of Nuclear Medicine and Molecular Imaging.

PubMed Central

BOSCARINO, G.; CREA, M.A.; MINELLI, F.; SNIDER, F.

2015-01-01

SUMMARY Paragangliomas (PGL) are rare lesions of the neuroendocrine system; in the neck, they usually affect the carotid glomus (carotid body tumours-CBT). This retrospective analysis reports our experience in management of these lesions in patients treated by surgical resection. Between 2000 and 2014, 33 patients were surgically treated at our institution, obtaining a series of 44 cervical PGLs. Tumour characteristics, family history, diagnostic procedures, surgical treatment, short- and long-term outcomes were reviewed. A female prevalence was found (76% of cases). Familial cases occurred in 9 patients (20%); 6 presented with bilateral lesions and 1 had multiple paragangliomas. Lymph node metastasis was not found in any patient. All lesions were classified into three groups according to the latero-lateral diameter. Complete resection of the PGL was performed in all patients. Mortality was null; transitory cranial nerve deficit occurred in 20% of cases with permanent palsy in 6.7%. No perioperative stroke/TIA were observed. Surgical resection of PGL should be considered as the only therapeutic option because it can ensure complete removal of the disease. Patients with bilateral lesions and positive family history should be referred for genetic analysis. Preoperative planning of the surgical procedure by integrated diagnostic imaging and a full mastery of vascular surgery techniques are mandatory to minimise the risk of the most common postoperative complications. Long-term follow-up is recommended, particularly in patients with familial disease or sporadic lesions treated in an advanced stage. PMID:26019391

Body Weight and Breast Cancer: Nested Case-Control Study in Southern Brazil.

PubMed

Kops, Natália Luiza; Bessel, Marina; Caleffi, Maira; Ribeiro, Rodrigo Antonini; Wendland, Eliana Marcia

2018-04-28

Current studies have shown that fast weight gain may be more important than body mass index on the incidence of breast cancer. The aim of this study was to evaluate the association between body weight and breast cancer. This was a case-control study nested in a cohort of a breast cancer mammography screening program in Southern Brazil. A trained investigator administered a standardized interview to collect sociodemographic and clinical data, and body weight history (weight at menarche, at marriage, at first and last pregnancy, and at menopause). Current anthropometric measurements were also made. Fifty-seven women with cancer (66.7% postmenopausal) and 159 controls were included. Current age (60.3 ± 10.4 vs. 55.8 ± 8.4 years, P < .01), marital status (49.1% vs. 64.8% with a partner, P = .03), and physical activity (48.2% vs. 32.3% sedentary, P = .01) were significantly different between cases and controls, respectively. Odds ratio showed that age and current waist circumference were associated with postmenopausal cancer. No difference was found in relation to body weight at different stages of life. Women with social vulnerability recruited at a mammography screening program in Southern Brazil showed a large weight gain during life, but no significant differences were found in body weight between women with or without breast cancer. Copyright © 2018 Elsevier Inc. All rights reserved.

Voices of donors: case reports of body donation in Hong Kong.

PubMed

Chiu, Hei Yeung; Ng, Kwok Sing; Ma, Sin Kwan; Chan, Chi Hung; Ng, Sheung Wah; Tipoe, George L; Chan, Lap Ki

2012-01-01

Body donation is important for medical education and academic research. However, it is relatively rare in Hong Kong when compared with many Western countries. Comprehensive research has been performed on the motivation for body donation in Western countries; however, there is still insufficient research on body donation in Hong Kong to provide information on how to increase the body-donation rate. To understand the factors involved in the decision to donate one's body, the authors interviewed a registered donor and the daughter of another donor in Hong Kong. The authors interpreted the information collected in light of the available published reports, which mostly focus on body donation in Western countries. Despite the consistency of some demographic factors and motivations between the participants in our study and those investigated in the published reports from Western countries, there are differences in education level and socioeconomic status between the donors in our study and those from Western studies. The authors also suggest that Confucianism and Buddhism in Chinese culture may motivate potential body donors in Hong Kong. Other important factors that influence the body-donation decision may include family members' body donation, registration as organ donors, and good doctor-patient relationships. Although case report studies have their limitations, this study allows us to explore the complexity of events and establish the interconnectivity of factors involved in body donation, which could not be achieved in previous survey-based studies. Copyright © 2012 American Association of Anatomists.

A case of retained wooden foreign body in orbit.

PubMed

Lee, Jin A; Lee, Hae Young

2002-12-01

A 41-year-old man visited our clinic complaining of esodeviation of the right eye. He had been operated on for corneal laceration 3 years before. One month later, exodeviation of the right eye had developed. The result of computed tomography (CT) was reported as orbital abscess and cellulitis. Although antibiotic treatment was administered for 2 weeks, the exodeviation didn't improve. On ocular examinations performed in our hospital in November-2001, his right eye was esotropic and had a relative afferent pupillary defect. Vision of the right eye was decreased to 0.02. Fundus examination showed optic atrophy. A new CT scan disclosed a foreign body introduced into the right medial orbital wall, nasal cavity and ethmoidal sinus. Although foreign body was surgically removed, vision and eye movement were not improved. In the case of a patient who has undergone orbital trauma, complete history taking and physical examinations must be performed. On suspicion of a foreign body, imaging study such as CT or MRI must be performed. However, because CT findings can be variable, careful follow-up is needed.

A Case of Retained Graphite Anterior Chamber Foreign Body Masquerading as Stromal Keratitis

PubMed Central

Han, Eun Ryung; Wee, Won Ryang; Lee, Jin Hak

2011-01-01

We report a case of a retained graphite anterior chamber foreign body that was masquerading as stromal keratitis. A 28-year-old male visited with complaints of visual disturbance and hyperemia in his right eye for four weeks. On initial examination, he presented with a stromal edema involving the inferior half of the cornea, epithelial microcysts, and moderate chamber inflammation. Suspecting herpetic stromal keratitis, he was treated with anti-viral and anti-inflammatory agents. One month after the initial visit, anterior chamber inflammation was improved and his visual acuity recovered to 20/20, but subtle corneal edema still remained. On tapering the medication, after three months, a foreign body was incidentally identified in the inferior chamber angle and was surgically removed resulting in complete resolution of corneal edema. The removed foreign body was a fragment of graphite and he subsequently disclosed a trauma with mechanical pencil 12 years earlier. This case showed that the presence of an anterior chamber foreign body should always be considered in the differential diagnosis of idiopathic localized corneal edema. PMID:21461226

Treatment with Sunitinib for Patients with Progressive Metastatic Pheochromocytomas and Sympathetic Paragangliomas

PubMed Central

Ayala-Ramirez, Montserrat; Chougnet, Cecile N.; Habra, Mouhammed Amir; Palmer, J. Lynn; Leboulleux, Sophie; Cabanillas, Maria E.; Caramella, Caroline; Anderson, Pete; Al Ghuzlan, Abir; Waguespack, Steven G.; Deandreis, Desirée

2012-01-01

Context: Patients with progressive metastatic pheochromocytomas (PHEOs) or sympathetic paragangliomas (SPGLs) face a dismal prognosis. Current systemic therapies are limited. Objectives: The primary end point was progression-free survival determined by RECIST 1.1 criteria or positron emission tomography with [18F]fluorodeoxyglucose/computed tomography ([18F]FDG-PET/CT), in the absence of measurable soft tissue targets. Secondary endpoints were tumor response according to RECIST criteria version 1.1 or FDG uptake, blood pressure control, and safety. Design: We conducted a retrospective review of medical records of patients with metastatic PHEO/SPGL treated with sunitinib from December 2007 through December 2011. An intention-to-treat analysis was performed. Patients and Setting: Seventeen patients with progressive metastatic PHEO/SPGLs treated at the Institut Gustave-Roussy and MD Anderson Cancer Center. Interventions: Patients treated with sunitinib. Results: According to RECIST 1.1, eight patients experienced clinical benefit; three experienced partial response, and five had stable disease, including four with predominant skeletal metastases that showed a 30% or greater reduction in glucose uptake on [18F]FDG-PET/CT. Of 14 patients who had hypertension, six became normotensive and two discontinued antihypertensives. One patient treated with sunitinib and rapamycin experienced a durable benefit beyond 36 months. The median overall survival from the time sunitinib was initiated was 26.7 months with a progression-free survival of 4.1 months (95% confidence interval = 1.4–11.0). Most patients who experienced a clinical benefit were carriers of SDHB mutations. Conclusion: Sunitinib is associated with tumor size reduction, decreased [18F]FDG-PET/CT uptake, disease stabilization, and hypertension improvement in some patients with progressive metastatic PHEO/PGL. Prospective multi-institutional clinical trials are needed to determine the true benefits of sunitinib. PMID

Thermoplasticity of coupled bodies in the case of stress-dependent heat transfer

NASA Technical Reports Server (NTRS)

Kilikovskaya, O. A.

1987-01-01

The problem of the thermal stresses in coupled deformable bodies is formulated for the case where the heat-transfer coefficient at the common boundary depends on the stress-strain state of the bodies (e.g., is a function of the normal pressure at the common boundary). Several one-dimensional problems are solved in this formulation. Among these problems is the determination of the thermal stresses in an n-layer plate and in a two-layer cylinder.

Hashish Body Packing: A Case Report

PubMed Central

Soriano-Perez, Manuel Jesus; Serrano-Carrillo, Jose Luis; Marin-Montin, Inmaculada; Cruz-Caballero, Alfonso

2009-01-01

A 42-year-old African male was brought by the police to the emergency department under suspicion of drug smuggling by body-packing. Plain abdominal radiograph showed multiple foreign bodies within the gastrointestinal tract. Contrast-enhanced abdominal CT confirmed the findings, and the patient admitted to have swallowed “balls” of hashish. Body-packing is a recognized method of smuggling drugs across international borders. Body packers may present to the emergency department because of drug toxicity, intestinal obstruction, or more commonly, requested by law-enforcement officers for medical confirmation or exclusion of suspected body packing. PMID:19724651

A Rare Case of Foreign Body Causing Recurrent Vaginal Discharge in Prepubertal Child

PubMed Central

Gobbur, Raghavendra.H.; Patil, Ashwini.G; Endigeri, Preetish

2015-01-01

Vaginal discharge in prepubertal children is mainly due to hypoestrogenic state of vaginal mucosa making it thin and alkaline leading to mucosal invasion by pathogen. In a paediatric case with persistent foul smelling , blood stained vaginal discharge not responding to medical therapy, vaginal foreign body should always be ruled out. Here, we report a 3 -year -old girl with complaint of recurrent vaginal discharge occasionally blood stained not relieved despite few antibiotics courses. On X -ray pelvis, a radioopaque foreign body hair clip was seen. Under sedation foreign body was removed by forceps following which child became asymptomatic. PMID:25738041

A rare case of foreign body causing recurrent vaginal discharge in prepubertal child.

PubMed

Gobbur, Vijayalakshmi R; Gobbur, Raghavendra H; Patil, Ashwini G; Endigeri, Preetish

2015-01-01

Vaginal discharge in prepubertal children is mainly due to hypoestrogenic state of vaginal mucosa making it thin and alkaline leading to mucosal invasion by pathogen. In a paediatric case with persistent foul smelling , blood stained vaginal discharge not responding to medical therapy, vaginal foreign body should always be ruled out. Here, we report a 3 -year -old girl with complaint of recurrent vaginal discharge occasionally blood stained not relieved despite few antibiotics courses. On X -ray pelvis, a radioopaque foreign body hair clip was seen. Under sedation foreign body was removed by forceps following which child became asymptomatic.

Overtube-Assisted Foreign Body Removal: A Review of Endoscopic Management and Case Illustration.

PubMed

Ofosu, Andrew; Ramai, Daryl; Reddy, Madhavi

2017-09-29

The ingestion of foreign bodies is a common medical emergency seen in both adults and children. In children, the most commonly ingested foreign bodies include coins, toys, magnets, and batteries. In adults, food bolus impaction represents the most common cause of foreign body ingestion. The majority of foreign bodies pass spontaneously. Sharp or pointed objects increase the risk of perforation. Emergent endoscopic intervention is indicated in cases of esophageal obstruction, ingestion of disk batteries, and sharp pointed objects in the esophagus. Flexible endoscopy is the therapeutic method of choice for removing foreign bodies. It is preferred due to its high success rate and low risk for complications. Additionally, the use of an overtube provides gastric and esophageal protection from mucosal laceration. We present a 27-year-old male who ingested six razor blades and a curtain hook and review endoscopic management.

Etonogestrel implant migration to the vasculature, chest wall, and distant body sites: cases from a pharmacovigilance database.

PubMed

Kang, Sarah; Niak, Ali; Gada, Neha; Brinker, Allen; Jones, S Christopher

2017-12-01

To describe clinical outcomes of etonogestrel implant patients with migration to the vasculature, chest wall and other distant body sites spontaneously reported to the US Food and Drug Administration Adverse Event Reporting System (FAERS) database. We performed a standardized Medical Dictionary for Regulatory Activities (MedDRA) query in the FAERS database (through November 15, 2015), with reports coded with one or more MedDRA preferred terms that indicate complications with device placement or migration of the device from the original site of insertion to the vasculature, chest wall and other distant body sites. We excluded any cases previously described in the medical literature. We identified 38 cases of pronounced etonogestrel implant migration. Migration locations included the lung/pulmonary artery (n=9), chest wall (n=1), vasculature at locations other than the lung/pulmonary artery (n=14) and extravascular migrations (n=14) to other body sites (e.g., the axilla and clavicle/neck line/shoulder). The majority of cases were asymptomatic and detected when the patient desired implant removal; however, seven cases reported symptoms such as pain, discomfort and dyspnea in association with implant migration. Three cases also describe pulmonary fibrosis and skin reactions as a result of implant migration to the vasculature, chest wall and other distant body sites. Sixteen cases reported surgical removal in an operating room setting. Our FAERS case series demonstrates etonogestrel implant migration to the vasculature, chest wall and other body sites distant from the site of original insertion. As noted by the sponsor in current prescribing information, a key determinant in the risk for etonogestrel contraceptive implant migration appears to be improper insertion technique. Although migration of etonogestrel implants to the vasculature is rare, awareness of migration and education on proper insertion technique may reduce the risk. Published by Elsevier Inc.

Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre.

PubMed

Rutherford, M A; Rankin, A J; Yates, T M; Mark, P B; Perry, C G; Reed, N S; Freel, E M

2015-05-01

Phaeochromocytoma (phaeo) and paraganglioma (PGL) are rare conditions, which are malignant in up to 30%. Optimal treatment is controversial, but in patients with metastatic iodine-131-meta-iodobenzylguanidine ((123)I-MIBG) avid tumours, we offer (131)I-MIBG therapy. We summarize response rates, survival and safety in a cohort of such patients treated with (131)I-MIBG in our centre from 1986 to 2012. Retrospective analysis of the case notes of patients with metastatic phaeo/PGL who received (131)I-MIBG was undertaken; patients underwent clinical, biochemical and radiological evaluation within 6 months of each course of (131)I-MIBG therapy. Twenty-two patients (9 males) were identified, 12 with metastatic PGL and 10 with phaeo. Overall median follow-up time after first dose of (131)I-MIBG was 53 months. In total, 68 doses of (131)I-MIBG were administered; average dose was 9967 MBq (269.4 mCi). After the first dose, >50% of patients demonstrated disease stability or partial response; progressive disease was seen in 9%. A subset of patients underwent repeated treatment with the majority demonstrating partial response or stable disease. No life-threatening adverse events were reported, but three patients developed hypothyroidism and two developed ovarian failure after repeated dosing. Five-year survival after original diagnosis was 68% and median (+inter quartile range) survival from date of diagnosis was 17 years (7.6-26.4) with no difference in survival according to diagnosis (P < 0.1). (131)I-MIBG is well tolerated and associates with disease stabilization or improvement in the majority of patients with metastatic phaeo/PGL. However, stronger conclusions on treatment effectiveness are limited by lack of a directly comparable 'control group' as well as an alternative 'gold standard' treatment. © The Author 2014. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Two cases of contact dermatitis resulting from use of body wash as a skin moisturizer.

PubMed

Miller, Michael A; Borys, Doug; Riggins, Michele; Masneri, David C; Levsky, Marc E

2008-02-01

The use of liquid skin cleanser or body wash has become common in the United States. We report 2 cases of contact dermatitis secondary to the application of Dove Body Wash (Unilever US, Inc., Englewood Cliffs, NJ) with the consumer misconception that the product was a skin moisturizing cream.

Pheochromocytoma of the Organ Zuckerkandl.

PubMed

Lee, C; Chang, E; Gimenez, J; McCarron, R

2017-01-01

Pheochromocytomas (PCCs);, or intra-adrenal paragangliomas (PGLs);, are neuroendocrine tumors arising within the adrenal medulla. Extra-adrenal paragangliomas may arise in the sympathetic or parasympathetic paraganglia and more rarely in other organs. One of the most common extra-adrenal sites is in the organ of Zuckerkandl, a collection of chromaffin cells near the origin of the inferior mesenteric artery or near the aortic bifurcation. The following is a case of a patient with resistant hypertension secondary to an extra-adrenal paraganglioma in the organ of Zuckerkandl. The patient is a 43 year old man with a history of depression, type 2 diabetes mellitus, and hypertension who was sent to the emergency department by his primary care physician for severely elevated blood pressures. Patient also had diaphoresis, tachycardia, and a new, fine tremor of his left hand. Upon presentation, the patient's blood pressure was 260/120 mmHg with a heart rate of 140 beats per minute. Plasma fractionated metanephrines sent on admission revealed significantly elevated levels of total plasma metanephrines (2558 pg/mL);, free metanephrine (74 pg/ml); and free normetanephrine (2484pg/mL);. An I-123 metaiodobenzylguanidine (MIBG); scan showed abnormal uptake in the lower abdomen at the level of the aortic bifurcation. Patient was started on alpha-blockade, with subsequent addition of a beta-blocker prior to surgery. Patient underwent surgical removal of the tumor with pathology consistent with a paraganglioma. Pheochromocytomas and paragangliomas are responsible for approximately 0.5 percent of cases of secondary hypertension. Many different biochemical markers have been used to aid in the diagnosis of PCC/PGL including plasma catecholamines, plasma metanephrines, urine fractionated metanephrines, urine catecholamines, total metanephrines and vanillymandellic acid. Definitive management of a PCC and PGL involves surgical removal of the tumor. Finally, there should be a discussion

An unusual case of foreign body pulmonary embolus: case report and review of penetrating trauma at a pediatric trauma center.

PubMed

Boomer, Laura A; Watkins, Daniel J; O'Donovan, Julie; Kenney, Brian D; Yates, Andrew R; Besner, Gail E

2015-03-01

Penetrating thoracic trauma is relatively rare in the pediatric population. Embolization of foreign bodies from penetrating trauma is very uncommon. We present a case of a 6-year-old boy with a penetrating foreign body from a projectile dislodged from a lawn mower. Imaging demonstrated a foreign body that embolized to the left pulmonary artery, which was successfully treated non-operatively. We reviewed the penetrating thoracic trauma patients in the trauma registry at our institution between 1/1/03 and 12/31/12. Data collected included demographic data, procedures performed, complications and outcome. Sixty-five patients were identified with a diagnosis of penetrating thoracic trauma. Fourteen of the patients had low velocity penetrating trauma and 51 had high velocity injuries. Patients with high velocity injuries were more likely to be older and less likely to be Caucasian. There were no statistically significant differences between patients with low vs. high velocity injuries regarding severity scores or length of stay. There were no statistically significant differences in procedures required between patients with low and high velocity injuries. Penetrating thoracic trauma is rare in children. The case presented here represents the only report of cardiac foreign body embolus we could identify in a pediatric patient.

Screening for phaeochromocytoma and paraganglioma: impact of using supine reference intervals for plasma metanephrines with samples collected from fasted/seated patients.

PubMed

Casey, R; Griffin, T P; Wall, D; Dennedy, M C; Bell, M; O'Shea, P M

2017-01-01

Background The Endocrine Society Clinical Practice Guideline on Phaeochomocytoma and Paraganglioma recommends phlebotomy for plasma-free metanephrines with patients fasted and supine using appropriately defined reference intervals. Studies have shown higher diagnostic sensitivities using these criteria. Further, with seated-sampling protocols, for result interpretation, reference intervals that do not compromise diagnostic sensitivity should be employed. Objective To determine the impact on diagnostic performance and financial cost of using supine reference intervals for result interpretation with our current plasma-free metanephrines fasted/seated-sampling protocol. Methods We conducted a retrospective cohort study of patients who underwent screening for PPGL using plasma-free metanephrines from 2009 to 2014 at Galway University Hospitals. Plasma-free metanephrines were measured using liquid chromatography-tandem mass spectrometry. Supine thresholds for plasma normetanephrine and metanephrine set at 610 pmol/L and 310 pmol/L, respectively, were used. Results A total of 183 patients were evaluated. Mean age of participants was 53.4 (±16.3) years. Five of 183 (2.7%) patients had histologically confirmed PPGL (males, n=4). Using seated reference intervals for plasma-free metanephrines, diagnostic sensitivity and specificity were 100% and 98.9%, respectively, with two false-positive cases. Application of reference intervals established in subjects supine and fasted to this cohort gave diagnostic sensitivity of 100% with specificity of 74.7%. Financial analysis of each pretesting strategy demonstrated cost-equivalence (€147.27/patient). Conclusion Our cost analysis, together with the evidence that fasted/supine-sampling for plasma-free metanephrines, offers more reliable exclusion of PPGL mandates changing our current practice. This study highlights the important advantages of standardized diagnostic protocols for plasma-free metanephrines to ensure the highest

Fatal heroin intoxication in body packers in northern Thailand during the last decade: two case reports.

PubMed

Sribanditmongkol, Pongruk; Supasingsiripreecha, Wiroon; Thampitak, Subharat; Junkuy, Anongphan

2006-01-01

A body packer is an important means of drug trafficking. While drug packets are inside the body, they can leak or rupture causing acute substance toxicity. Most of the reports of body packer syndrome have come from Europe and North America, which are destination targets. In the present study, the authors reported two cases of fatal heroin body packers from the northern part of Thailand. Both cases were foreign tourists who came to Chiang Mai and stayed in a hotel or a guesthouse room in which the deaths occurred. The autopsy findings revealed rupturing of heroin packages in the stomach. The packaging used in both cases was not sophisticated. The powder was packed inside condoms without extra covering, as observed in some other professional packers. The amount of heroin transported was about 30-50 gm. The purity of heroin in this powder was about 50-90%. Their destinations were their home countries and not directly to Europe or North America. Deaths occurred just prior to their return. The cause of death was a heroin overdose. A significant level of heroin metabolites, 6-MAM and morphine were detected in the blood and urine.

Migrating foreign body in the tracheobronchial tree: an unusual case of firework penetrating neck injury.

PubMed

Khan, M Sarwar; Kirkland, P M; Kumar, R

2002-02-01

Firework injuries can manifest themselves in many different ways; usually as an explosive or burn injury. This case describes an unusual presentation of a firework penetrating injury resulting in a sharp coiled metal foreign body travelling through a small entry wound in the neck and subsequently lodging itself in the tracheobronchial tree. A foreign body such as this can potentially travel a considerable distance through the soft tissues and end up in an unsuspecting distant site. There must, therefore, be a high index of suspicion with the appropriate radiological investigations for appropriate management of such cases.

Russell body apical periodontitis: an unusual case report.

PubMed

Dos Santos, Jean Nunes; Ramos, Eduardo Antônio Gonçalves; Gurgel, Clarissa Araújo Silva; Barros, Adna Conceição; de Freitas, André Carlos; Crusoé-Rebello, Iêda Maria

2008-12-01

Russell bodies (RBs) changes in chronic apical lesions have rarely been reported in the literature. We describe a case of a periapical lesion abundantly and extensively composed of RB. Microscopic examination showed accumulation of plasma cells containing globular, spherical, polygonal, and eosinophilic structures against fibrous connective tissue. Initial diagnostic considerations based on a smaller magnification included hypersecretory plasmocytoma, although there was no evidence of infiltrative growth, mitotic activity, nuclear atypia, or cellular pleomorphism. Then, a panel of immunohistochemical markers was applied and the cells showed positivity with both kappa and lambda chains demonstrating their polyclonal origin. The extensive accumulation of RBs involving the periapical region represents an unreported and significant histologic change, as it was mimicking a malignant neoplasm.

Endovascular Approach to Glomus Jugulare Tumors

PubMed Central

Kocur, Damian; Ślusarczyk, Wojciech; Przybyłko, Nikodem; Hofman, Mariusz; Jamróz, Tomasz; Suszyński, Krzysztof; Baron, Jan; Kwiek, Stanisław

2017-01-01

Summary Background Paragangliomas are benign neuroendocrine tumors derived from the glomus cells of the vegetative nervous system. Typically, they are located in the region of the jugular bulb and middle ear. The optimal management is controversial and can include surgical excision, stereotactic radiosurgery and embolization. Case Report We report the endovascular approach to three patients harboring glomus jugulare paragangliomas. In all cases incomplete occlusion of the lesions was achieved and recanalization in the follow-up period was revealed. Two patients presented no clinical improvement and the remaining one experienced a transient withdrawal of tinnitus. Conclusions It is technically difficult to achieve complete obliteration of glomus jugulare tumors with the use of embolization and the subtotal occlusion poses a high risk of revascularization and is not beneficial in terms of alleviating clinical symptoms. PMID:28685005

Pheochromocytoma and Paraganglioma—Patient Version

Cancer.gov

Pheochromocytoma and paraganglioma are rare tumors that can be benign (not cancer) or malignant. Pheochromocytomas form in the adrenal glands, and paragangliomas usually along nerve pathways in the head, neck, and spine. Start here to find information on pheochromocytoma and paraganglioma treatment, and research.

Protrusion of the tongue in bodies burned after death: Two cases of arson to cover homicide.

PubMed

Nikolić, Slobodan; Živković, Vladimir

2015-10-01

In the forensic assessment of burned bodies, the question of whether the victim was exposed to fire before or after death is of crucial importance. Many authors consider tongue protrusion in cases of burned bodies to be a post-mortem phenomenon. Deep-heating effects of fire are sufficient to cook muscle. The muscle becomes shortened by dehydration and protein denaturation. Exposure to heat causes flexion of the extremities on the contraction of muscles and tendons - heat rigour. The flexors, being bulkier than the extensors, contract more and force the limbs into the position of general flexion. The genioglossus is the major muscle of the tongue and is responsible for protruding or sticking out the tongue: by means of its inferior fibres, it draws the root of the tongue forward and protrudes the apex from the mouth. Similar to the action of limb flexors exposed to heat and the appearance of post-mortem general flexion of a burned body due to heat rigour, perhaps the geniglossus could be shortened by heat, causing post-mortem tongue protrusion to appear as heat rigour of the tongue. In this paper, we present two such cases of protrusion of the tongue in bodies burned after death - cases of arson to cover homicide. © The Author(s) 2014.

A rare case of a sharp foreign body on the vocal cord.

PubMed

Nor Hisyam, C I; Misron, K; Mohamad, I

2017-01-01

A foreign body (FB) in the upper aerodigestive tract is a common clinical problem that presents as as acute emergency. Sharp FB, such as fish bone or chicken bone, commonly lodges in the tonsil, base of tongue, vallecula or pyriform fossa. Dislodgement of a FB into the laryngopharynx is very rare and specifically onto the vocal cord is extremely uncommon. This case report illustrates a rare case of a sharp FB that was dislodged into the airway and stuck on to the right vocal cord, which was removed under local anaesthesia.

Hypoinsulinemic hypoglycemia triggered by liver injury in elderly subjects with low body weight: case reports.

PubMed

Anno, Takatoshi; Kaneto, Hideaki; Shigemoto, Ryo; Kawasaki, Fumiko; Kawai, Yasuhiro; Urata, Noriyo; Kawamoto, Hirofumi; Kaku, Kohei; Okimoto, Niro

2018-01-01

Hypoglycemia is induced by many causes, especially over-dose of insulin or oral hypoglycemic agents in diabetic subjects. In such a case, hyperinsulinemic hypoglycemia is usually observed. On the other hand, it is important to classify secondary hypoglycemia and hypoinsulinemic hypoglycemia. Liver injury-induced hypoglycemia is one of the causes of hypoinsulinemic hypoglycemia but rarely observed in clinical practice. Herein, we experienced similar 2 cases of non-diabetic hypoinsulinemic hypoglycemia. Both of them were elderly subjects with low body weight. Furthermore, it is likely that hypoinsulinemic hypoglycemia in both subjects was triggered by severe liver injury, at least in part, due to possible limited liver glycogen store. In elderly subjects with low body weight and/or malnutrition, metabolism in the liver is reduced and glycogen accumulation is decreased. Such alteration brings out acute and marked liver injury, which finally leads to the onset of severe hypoglycemia. It is known that not only liver injury but also multiple organ failure could be induced due to extreme emaciation in subjects. It is likely that in elderly subjects with low body weight and/or malnutrition, multiple organ failure including liver failure could be induced due to the similar reason. Therefore, we should be very careful of such subjects in order to avoid the development of multiple organ failure which leads to life-threatening situations. In conclusion, we should keep in mind the possibility of hypoinsulinemic hypoglycemia when we examine severe liver injury, especially in elderly or starving subjects with low body weight and limited liver glycogen stores. It is important to classify secondary hypoglycemia and hypoinsulinemic hypoglycemia.Liver injury-induced hypoglycemia is one of the causes of hypoinsulinemic hypoglycemia but rarely observed in everyday clinical practice.Herein, we reported similar 2 cases of hypoinsulinemic hypoglycemia without diabetes presumably triggered

Self-amputation of a healthy hand: a case of body integrity identity disorder.

PubMed

Sorene, E D; Heras-Palou, C; Burke, F D

2006-12-01

A case report is presented of self-amputation of a healthy hand. We have reviewed the literature and seek to broaden the scope of understanding of Body Integrity Identity Disorder. This rare condition can constitute a pitfall for the unsuspecting hand surgeon.

Live births after polar body biopsy and frozen-thawed cleavage stage embryo transfer: case report

PubMed Central

Guimarães, Fernando; Roque, Matheus; Valle, Marcello; Kostolias, Alessandra; de Azevedo, Rodrigo A; Martinhago, Ciro D; Sampaio, Marcos; Geber, Selmo

2016-01-01

Pre-implantation genetic diagnosis (PGD) or screening (PGS) technology, has emerged and developed in the past few years, benefiting couples as it allows the selection and transfer of healthy embryos during IVF treatments. These techniques can be performed in oocytes (polar-body biopsy) or embryos (blastomere or trophectoderm biopsy). In this case report, we describe the first two live births to be published in Brazil after a polar-body (PB) biopsy. In case 1, a 42-year-old was submitted to PB biopsy with PGS due to advanced maternal age and poor ovarian reserve. Five MII oocytes underwent first and second polar body biopsy and four cleavage embryos were cryopreserved. The PGS analysis resulted in two euploid embryos (next generation sequence). A frozen-thawed embryo transfer (FET) was performed after endometrial priming and a healthy baby was delivered after a cesarean section (37 weeks, female, 3390g, 47.5 cm). In case 2, a 40-year old patient with balanced translocation and poor ovarian response was submitted to PB biopsy. Two MII oocytes underwent first and second polar body biopsy and two embryos were cryopreserved in cleavage stage. The analysis resulted in one euploid embryo that was transferred after endometrial priming. A preterm healthy baby (34 weeks, female, 2100g, 40 cm) was delivered via cesarean section. In conclusion, although the blastocyst biopsy is the norm when performing PGS/PGD during IVF treatments, other alternatives (as PB biopsy) should be considered in some specific situations. PMID:28050963

Live births after polar body biopsy and frozen-thawed cleavage stage embryo transfer: case report.

PubMed

Guimarães, Fernando; Roque, Matheus; Valle, Marcello; Kostolias, Alessandra; Azevedo, Rodrigo A de; Martinhago, Ciro D; Sampaio, Marcos; Geber, Selmo

2016-12-01

Pre-implantation genetic diagnosis (PGD) or screening (PGS) technology, has emerged and developed in the past few years, benefiting couples as it allows the selection and transfer of healthy embryos during IVF treatments. These techniques can be performed in oocytes (polar-body biopsy) or embryos (blastomere or trophectoderm biopsy). In this case report, we describe the first two live births to be published in Brazil after a polar-body (PB) biopsy. In case 1, a 42-year-old was submitted to PB biopsy with PGS due to advanced maternal age and poor ovarian reserve. Five MII oocytes underwent first and second polar body biopsy and four cleavage embryos were cryopreserved. The PGS analysis resulted in two euploid embryos (next generation sequence). A frozen-thawed embryo transfer (FET) was performed after endometrial priming and a healthy baby was delivered after a cesarean section (37 weeks, female, 3390g, 47.5 cm). In case 2, a 40-year old patient with balanced translocation and poor ovarian response was submitted to PB biopsy. Two MII oocytes underwent first and second polar body biopsy and two embryos were cryopreserved in cleavage stage. The analysis resulted in one euploid embryo that was transferred after endometrial priming. A preterm healthy baby (34 weeks, female, 2100g, 40 cm) was delivered via cesarean section. In conclusion, although the blastocyst biopsy is the norm when performing PGS/PGD during IVF treatments, other alternatives (as PB biopsy) should be considered in some specific situations.

Lamotrigine Induced Whole Body Tics: A Case Report and Literature Review.

PubMed

Centorino, Michael B; Catalano, Glenn; Catalano, Maria C

2016-01-01

Lamotrigine is an anticonvulsant medication that also has utility in the treatment of bipolar disorder. It has been associated with many side effects, including rashes that can progress to Stevens-Johnson syndrome or toxic epidermal necrolysis. It has also been associated with the development of motor tics, most commonly in the head, neck, and shoulders. We will now present the case of a 45-year-old woman who developed tics that involved the entire left side of her body after her dose of lamotrigine was increased from 200 mg daily (2.0 mg/kg/day) to 225 mg daily (2.3 mg/kg/day). We will review the prior cases of lamotrigine induced tics, and compare them to the circumstances surrounding our patient. We will also discuss the neurobiology of tics and make suggestions to improve the tics, based on the reported cases.

Postmortem changes of human bodies on the bathyal sea floor--two cases of aircraft accidents above the open sea.

PubMed

Dumser, Thomas K; Türkay, Michael

2008-09-01

Forensic taphonomy in the marine context recently received growing attention. However, only limited information is available about the fate of human bodies at greater sea depth. Following two fatal aircraft accidents (west of Namibia, south of Sicily) human remains were recovered from a depth of 540-580 m (both cases) after 3 months (Namibia)/34 days (Sicily). In the Namibia case fully skeletonized bones were lifted. In the Sicily case a complete, dressed body was found exhibiting a partially skeletonized skull, starting adipocere formation and pink teeth. The rate and mode of decomposition of human bodies in the deep sea varies considerably and is mainly influenced by the local faunal composition. Of special relevance for the understanding of both cases was the oceanographic observation that the highly efficient necrophageous lyssianassids are abundant off Namibia but are rare in the Mediterranean, emphasizing the importance of collaboration of forensic and marine scientists in such case work.

New HIF2α inhibitors: potential implications as therapeutics for advanced pheochromocytomas and paragangliomas.

PubMed

Toledo, Rodrigo Almeida

2017-09-01

Two recent independent studies published in Nature show robust responses of clear cell renal cell carcinoma (ccRCC) cell lines, preclinical ccRCC xenograft models and, remarkably, a patient with progressive ccRCC despite receiving multiple lines of treatment, to the long-awaited, recently developed inhibitors of hypoxia-inducible factor 2-alpha (HIF2α). This commentary published in Endocrine-Related Cancer is based on the recognition of similar molecular drivers in ccRCC and the endocrine neoplasias pheochromocytomas and paragangliomas (PPGLs), ultimately leading to stabilization of HIFs. HIF-stabilizing mutations have been detected in the von Hippel-Lindau (VHL) gene, as well as in other genes, such as succinate dehydrogenase (SDHx), fumarate hydratase (FH) and transcription elongation factor B subunit 1 (TCEB1), as well as the gene that encodes HIF2α itself: EPAS1 HIF2α Importantly, the recent discovery of EPAS1 mutations in PPGLs and the results of comprehensive in vitro and in vivo studies revealing their oncogenic roles characterized a hitherto unknown direct mechanism of HIF2α activation in human cancer. The now available therapeutic opportunity to successfully inhibit HIF2α pharmacologically with PT2385 and PT2399 will certainly spearhead a series of investigations in several types of cancers, including patients with SDHB -related metastatic PPGL for whom limited therapeutic options are currently available. Future studies will determine the efficacy of these promising drugs against the hotspot EPAS1 mutations affecting HIF2α amino acids 529-532 (in PPGLs) and amino acids 533-540 (in erythrocytosis type 4), as well as against HIF2α protein activated by VHL , SDHx and FH mutations in PPGL-derived chromatin cells. © 2017 Society for Endocrinology.

Vaginal foreign body mimicking cervical cancer in postmenopausal woman - case study.

PubMed

Ciebiera, Michał; Słabuszewska-Jóźwiak, Aneta; Ledowicz, Witold; Jakiel, Grzegorz

2015-09-01

We present a case report of a 73-year-old, postmenopausal woman with detailed history of breast cancer and oncology treatment including tamoxifen therapy. She presented at the clinic of gynecology and obstetrics with recurrent inflammation of the urinary and genital tract and suspicion of a cervical mass. She also presented occasional abdominal complaints and malodorous vaginal discharge. These symptoms were observed in the patient for several years. Before hospitalization she received many kinds of empirical, antimicrobial treatment such as chlorquinaldol, metronidazole, nifuratel, and nystatin. She did not receive further guidance from doctors about the causes of ailments and further diagnostic and treatment capabilities. In our clinic a detailed diagnostic process including ultrasound transvaginal examination and a minisurgical procedure revealed the presence of a vaginal foreign body (which turned out to be a plastic, shampoo bottle cap) surrounded by a mass of inflamed tissue mimicking a cervical tumor. All symptoms and complaints subsided after surgical removal of the foreign body and antibacterial therapy with metronidazole and cefuroxime. Our study draws attention to the need of thorough gynecological care including prophylaxis, especially in the case of complaints of an intimate nature. Even trivial, frequently occurring disorders can be dangerous and require proper and responsible doctor's supervision and management through the healing process.

Metallic foreign body in the sphenoid sinus after ballistic injury: a case report.

PubMed

Akhaddar, A; Abouchadi, A; Jidal, M; Gazzaz, M; Elmostarchid, B; Naama, O; Rzin, A; Boucetta, M

2008-05-01

Paranasal sinus injuries by foreign bodies have a lower incidence compared with facial injuries. Among them, penetrating maxillofacial injuries to the sphenoid sinus and skull base remain rare. We report the case of a 41-year-old man who presented with, after a missile-related maxillofacial injury, a metallic foreign body enclosed within the sphenoid sinus with carotid-canal fracture. Angiographic evaluation showed a mass in the right internal carotid artery. The foreign object was successfully extracted through a transmaxillary sublabial approach with a good outcome. We discuss the extensive preoperative evaluation and interdisciplinary management of this unusual injury.

A review of the management of positive biochemical screening for phaeochromocytoma and paraganglioma: a salutary tale.

PubMed

Garrahy, A; Casey, R; Wall, D; Bell, M; O'Shea, P M

2015-07-01

Phaeochromocytomas (PC) and paragangliomas (PGL) are rare neuroendocrine tumours of chromaffin cells. Diagnosis depends on biochemical evidence of excessive production of catecholamines. This is straightforward when test results are orders of magnitude above the concentrations expected in healthy individuals and those with essential hypertension. Equivocal results pose a management dilemma. We reviewed biochemical screens that were positive and the ensuing management for PC/PGL at our institution. The objective was to inform the development of a standardised approach to investigation and clinical follow-up. All records of positive biochemical screening for PC/PGL were extracted from the laboratory information system between January 2004 and June 2012. Clinical notes of patients with positive results were reviewed. A total of 2749 biochemical screens were performed during the evaluation period. Of these, 106 (3.9%) performed on 82 patients were positive. Chart review determined that 12/82 patients had histologically confirmed PC/PG. Of the 70 patients remaining, the most common indication for biochemical screening was hypertension and the medical subspecialty most frequently requesting the test was Endocrinology. The primary team carried out repeat testing on 35/70 (50%) patients and in 29 results normalised. Notably, 35/70 (50%) patients did not have any follow-up of positive test results. This study highlights the necessity for a standardised diagnostic protocol for PC/PGL. We suggest that appropriate follow-up of borderline-elevated results should first include repeat biochemical testing. This should be performed under standardised pre-analytical conditions and where possible off all potentially interfering medications, measuring plasma free metadrenalines. © 2015 John Wiley & Sons Ltd.

Virtual reality in the treatment of body image disturbances after bariatric surgery: a clinical case.

PubMed

Riva, Giuseppe; Cárdenas-López, Georgina; Duran, Ximena; Torres-Villalobos, Gonzalo M; Gaggioli, Andrea

2012-01-01

Bariatric surgery is an operation on the stomach and/or intestines that helps patients with extreme obesity to lose weight. Even if bariatric surgery, compared with traditional obesity treatment, is more effective in reducing BMI, this approach does not achieve equal results in every patient. More, following bariatric surgery common problems are body image dissatisfaction and body disparagement: there is a significant difference between the weight loss clinicians consider successful (50% of excess weight) and the weight loss potential patients expect to achieve (at least 67% of the excess weight). The paper discusses the possible role of virtual reality (VR) in addressing this problem within an integrated treatment approach. More, the clinical case of a female bariatric patient who experienced body dissatisfaction even after a 30% body weight loss and a 62% excess body weight loss, is presented and discussed.

The impact of shallow burial on differential decomposition to the body: a temperate case study.

PubMed

Schotsmans, Eline M J; Van de Voorde, Wim; De Winne, Joan; Wilson, Andrew S

2011-03-20

Extant literature contains a number of specific case studies on differential decomposition involving adipocere formation or desiccation, but few describe the co-occurrence of these features within a temperate climate. The case of a 65-year-old male, partially buried in a shallow grave for 7 months, is presented in which the soft tissues of the body were outwardly well preserved. The right leg was desiccated, some parts of the body were covered with adipocere (head, neck, right shoulder, upper torso and left leg) and other parts could be classified as in the early stages of decomposition. In this study the taphonomic variables resulting in differential decomposition with desiccation and adipocere formation are discussed. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

Foreign body aspiration in a child detected through emergency department radiology reporting: a case report.

PubMed

Crawford, Nigel W

2007-08-01

Foreign-body aspiration remains a leading cause of mortality in children under 3 years despite child-safety initiatives. This case report describes a classic history of peanut aspiration in a young child. Unfortunately, the diagnosis was delayed and only detected the next day through radiology review. The clinical history is paramount and this case highlights how emergency radiology reporting can minimize morbidity.

Use of letter writing as a means of integrating an altered body image: a case study.

PubMed

Rancour, Patricia; Brauer, Kathryn

2003-01-01

To describe the use of letter writing as a technique to assist patients in adjusting to an altered body image after dramatic cancer treatment. Published articles and books. Gestalt therapy, psychosynthesis, and journaling techniques evolve into a technique that can assist patients who are challenged to accept altered body parts. Described in a case study presentation, letter writing was found to assist female patients with recurrent breast cancer in adjusting to reconstruction of lost breasts. Nurses can use letter writing as a means of assisting patients through the grief process associated with body image alterations.

Hybrid-Wing-Body Vehicle Composite Fuselage Analysis and Case Study

NASA Technical Reports Server (NTRS)

Mukhopadhyay, Vivek

2014-01-01

Recent progress in the structural analysis of a Hybrid Wing-Body (HWB) fuselage concept is presented with the objective of structural weight reduction under a set of critical design loads. This pressurized efficient HWB fuselage design is presently being investigated by the NASA Environmentally Responsible Aviation (ERA) project in collaboration with the Boeing Company, Huntington Beach. The Pultruded Rod-Stiffened Efficient Unitized Structure (PRSEUS) composite concept, developed at the Boeing Company, is approximately modeled for an analytical study and finite element analysis. Stiffened plate linear theories are employed for a parametric case study. Maximum deflection and stress levels are obtained with appropriate assumptions for a set of feasible stiffened panel configurations. An analytical parametric case study is presented to examine the effects of discrete stiffener spacing and skin thickness on structural weight, deflection and stress. A finite-element model (FEM) of an integrated fuselage section with bulkhead is developed for an independent assessment. Stress analysis and scenario based case studies are conducted for design improvement. The FEM model specific weight of the improved fuselage concept is computed and compared to previous studies, in order to assess the relative weight/strength advantages of this advanced composite airframe technology

Management of Gynecomastia in Patients With Different Body Types: Considerations on 312 Consecutive Treated Cases.

PubMed

Innocenti, Alessandro; Melita, Dario; Mori, Francesco; Ciancio, Francesco; Innocenti, Marco

2017-05-01

Gynecomastia is a common finding in male subjects which incidence varies widely in the world population. In adolescents, it is frequently temporary but, if it becomes persistent, it generates considerable embarrassment, inducing the patients to seek surgical consultation. Even in patients with good body contour, gynecomastia creates even greater distress considering the special attention given by these subjects to their physical appearance. The authors present their experience in the treatment of gynecomastia comparing different body types of patients with the aim to investigate dissimilar expectations, needs and surgical outcomes thus optimizing the management of the pathological condition, achieving high levels of agreement and reducing unsatisfied patients arising from cosmetic surgery. Between January 2007 and January 2015, 312 selected patients have been treated surgically for gynecomastia. Patients were grouped according to their physical aspect: 97 were classified as high muscle mass body type (group A), 106 as normal (group B) and 109 as overweight patients (group C). All of them were adults ranging in age between 18 and 52 years. Follow-up ranged from 12 to 60 months. In all cases, an excision of the gland in the form of a subcutaneous mastectomy was performed; the most common surgical access was in the inferior part of the areola. No breast cancers were found at the histological examinations. Also, no skin or areola necrosis have been referred, and no recurrence of gynecomastia disorder has been reported. Six cases of seroma (limited to the fatty gynecomastia) and 3 cases of hematomas (requiring immediate surgical revision) were found. Although the patients in group B resulted more distressed by the disorder, higher levels of postoperative satisfaction were recorded in this group. The study demonstrates the importance of the different management of the same disorder according to the different patients' expectations, related to the different body type. Our

Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

PubMed

Jansen, T T G; Timmers, H J L M; Marres, H A M; Kaanders, J H A M; Kunst, H P M

2018-04-01

Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. Local control, cranial nerve damage, complications, function recovery. Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was <1%. For class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P < .05). There was no difference in treatment outcomes between tumours of different C class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P < .05) and cranial nerve damage/complication rates were 67%-100% (3% post-radiotherapy; P < .05). C1-4DeDi tumours showed lesser local control and cranial nerve damage rates when compared to C1-4De tumours. An individual risk is constituted for surgery and radiotherapy, stratified per Fisch class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group. © 2017 John Wiley & Sons Ltd.

[Case report of live threatening complications due to self insertion of foreign body into the vagina for masturbation purpose].

PubMed

Kuzaka, Bolesław; Kobryń, Andrzej; Niemierko, Maciej; Czaplicki, Maciej

2009-01-01

Authors have reported a case report of life threatening complications due to insertion of foreign bodies into the vagina, because of masturbation purpose. In this case subsequently came to perforation of the urinary bladder by the huge calculus that developed over the foreign body, and next to the peritoneum with development peritonitis and acute renal insufficiency with the need of dialysotherapy. After a number of surgical operations, the patient with a definitive percutaneous nephrostomy was discharged.

Vaginal foreign body mimicking cervical cancer in postmenopausal woman – case study

PubMed Central

Słabuszewska-Jóźwiak, Aneta; Ledowicz, Witold; Jakiel, Grzegorz

2015-01-01

We present a case report of a 73-year-old, postmenopausal woman with detailed history of breast cancer and oncology treatment including tamoxifen therapy. She presented at the clinic of gynecology and obstetrics with recurrent inflammation of the urinary and genital tract and suspicion of a cervical mass. She also presented occasional abdominal complaints and malodorous vaginal discharge. These symptoms were observed in the patient for several years. Before hospitalization she received many kinds of empirical, antimicrobial treatment such as chlorquinaldol, metronidazole, nifuratel, and nystatin. She did not receive further guidance from doctors about the causes of ailments and further diagnostic and treatment capabilities. In our clinic a detailed diagnostic process including ultrasound transvaginal examination and a minisurgical procedure revealed the presence of a vaginal foreign body (which turned out to be a plastic, shampoo bottle cap) surrounded by a mass of inflamed tissue mimicking a cervical tumor. All symptoms and complaints subsided after surgical removal of the foreign body and antibacterial therapy with metronidazole and cefuroxime. Our study draws attention to the need of thorough gynecological care including prophylaxis, especially in the case of complaints of an intimate nature. Even trivial, frequently occurring disorders can be dangerous and require proper and responsible doctor's supervision and management through the healing process. PMID:26528112

Pheochromocytoma and paraganglioma

PubMed Central

Kantorovich, Vitaly; Pacak, Karel

2016-01-01

Pheochromocytoma is a very special kind of tumor full of duplicity. On the one hand it represents its own microworld with unique clinical, biochemical and pathological features, while on the other it constitutes a tremendously significant part of whole body system, playing a vital role for practically every organ system. It has a very special character – sometimes like a child it can be sweet and predictable, while at times it can behave like a deadly wild beast, crashing and tearing everything on its path in a fierce rage. It also consists of the amazingly intelligent neuroendocrine cells that possess a magical ability to make miraculous substances of many kinds. But most of all, it is a system that is able to drive our curiosity and the itch of “Cogito, ergo sum” to limitless depths and year by year it still amazes us with new and unexpected discoveries that move our understanding of multiple pathways and metabolic events closer to the ultimate truth. Recent discoveries of succinate dehydrogenase (SHD) and prolyl hydroxylase (PHD) mutations, for example, propelled our understanding of neuroendocrine tumorigenesis as a whole, as well as physiology of mitochondrial respiratory chain and phenomenon of pseudohypoxia in particular. Good old discoveries make their way from dusty repositories to shine with new meaning, appropriate for the current level of knowledge. This acquired wisdom makes us better physicians – knowing the specific expression makeup of catecholamine transporters, GLUTs and SRIFs allows for better tailored imaging and therapeutic manipulations. There are still long ways to go, keeping in mind that pheochromocytoma is but so very special, and we are optimistic and expect many great things to come. PMID:20541673

Compliant mechanism road bicycle brake: a rigid-body replacement case study

DOE Office of Scientific and Technical Information (OSTI.GOV)

Olsen, Brian M; Howell, Larry L; Magleby, Spencer P

2011-01-19

The design of high-performance bicycle brakes is complicated by the competing design objectives of increased performance and low weight. But this challenge also provides a good case study to demonstrate the design of compliant mechanisms to replace current rigid-link mechanisms. This paper briefly reviews current road brake designs, demonstrates the use of rigid-body replacement synthesis to design a compliant mechanism, and illustrates the combination of compliant mechanism design tools. The resulting concept was generated from the modified dual-pivot brake design and is a partially compliant mechanism where one pin has the dual role of a joint and a mounting pin.more » The pseudo-rigid-body model, finite element analysis, and optimization algorithms are used to generate design dimensions, and designs are considered for both titanium and E-glass flexures. The resulting design has the potential of reducing the part count and overall weight while maintaining a performance similar to the benchmark.« less

Case-control study of low-back pain referred for magnetic resonance imaging, with special focus on whole-body vibration.

PubMed

Palmer, Keith T; Harris, Clare E; Harris, E Claire; Griffin, Michael J; Bennett, James; Reading, Isabel; Sampson, Madelaine; Coggon, David

2008-10-01

This study investigated risk factors for low-back pain among patients referred for magnetic resonance imaging (MRI), with special focus on whole-body vibration. A case-control approach was used. The study population comprised working-aged persons from a catchment area for radiology services. The cases were those in a consecutive series referred for a lumbar MRI because of low-back pain. The controls were age- and gender-matched persons X-rayed for other reasons. Altogether, 252 cases and 820 controls were studied, including 185 professional drivers. The participants were questioned about physical factors loading the spine, psychosocial factors, driving, personal characteristics, mental health, and certain beliefs about low-back pain. Exposure to whole-body vibration was assessed by six measures, including weekly duration of professional driving, hours driven in one period, and current root mean square A(8). Associations with whole-body vibration were examined with adjustment for age, gender, and other potential confounders. Strong associations were found with poor mental health and belief in work as a causal factor for low-back pain, and with occupational sitting for > or =3 hours while not driving. Associations were also found for taller stature, consulting propensity, body mass index, smoking history, fear-avoidance beliefs, frequent twisting, low decision latitude, and low support at work. However, the associations with the six metrics of whole-body vibration were weak and not statistically significant, and no exposure-response relationships were found. Little evidence of a risk from professional driving or whole-body vibration was found. Drivers were substantially less heavily exposed to whole-body vibration than in some earlier surveys. Nonetheless, it seems that, at the population level, whole-body vibration is not an important cause of low-back pain among those referred for MRI.

Current surgical management of carotid body tumors.

PubMed

Davila, Victor J; Chang, James M; Stone, William M; Fowl, Richard J; Bower, Thomas C; Hinni, Michael L; Money, Samuel R

2016-12-01

Carotid body tumors (CBTs) are rare. Management guidelines may include genetic testing for succinate dehydrogenase (SDH) mutations. We performed an institutional review of the surgical management of CBT. A retrospective analysis (1994-2015) of CBT excisions at our institution was performed. Data obtained included demographics, genetic testing (if performed), intraoperative details, postoperative morbidity, and long-term outcomes. Data from the first CBT excision were included in patients with bilateral tumors. Genetic testing was routinely offered in patients with a family history of CBT or multiple paragangliomas. A total of 183 CBTs (124 female [67.7%]) were excised. A neck mass was present in 106 patients (57.9%), 24 patients (12.1%) presented with tenderness or neck pain, and 3 (1.6%) presented with cranial nerve dysfunction. Computed tomography (57.9%) or magnetic resonance imaging (51.3%) were the most commonly used imaging modalities. Preoperative angiography was performed in 73 patients (39.8%), and 62 of them (84.5%) underwent embolization or internal carotid balloon occlusion testing, or both. Mean tumor diameter was 3.2 cm (range, 0.6-7.2 cm). There were 71 (38.8%), 75 (41%), and 37 (20.2%) Shamblin type 1, 2, and 3 tumors, respectively. Average operating time was 224 minutes (range, 52-696 minutes). Average blood loss was 143.9 mL (range, 10-2000 mL). Arterial reconstruction with an interposition graft was required in 10, and patch angioplasty was performed in four. Cranial nerve injury was permanent in 10 (5.5%), and the rate of stroke was 1% (n = 2). A total of 382 lymph nodes were excised, and all were benign. There were no deaths ≤30 days. Only one patient presented with malignant disease 2 years after CBT excision, and this patient did not undergo genetic testing. Thirty-four (18.6%) had a family history of CBT. SDH testing was performed in 18 patients, and 17 tested positive. Positive genetic testing had a correlation with earlier age

Body integrity identity disorder crosses culture: case reports in the Japanese and Chinese literature.

PubMed

Blom, Rianne M; Vulink, Nienke C; van der Wal, Sija J; Nakamae, Takashi; Tan, Zhonglin; Derks, Eske M; Denys, Damiaan

2016-01-01

Body integrity identity disorder (BIID) is a condition in which people do not perceive a part of their body as their own, which results in a strong desire for amputation or paralyzation. The disorder is likely to be congenital due to its very early onset. The English literature describes only Western patients with BIID, suggesting that the disorder might be merely prevalent in the West. To scrutinize this assumption, and to extend our knowledge of the etiology of BIID, it is important to trace cases with BIID in non-Western populations. Our objective was to review Chinese and Japanese literature on BIID to learn about its presence in populations with a different genetic background. A systematic literature search was performed in databases containing Japanese and Chinese research, published in the respective languages. Five Japanese articles of BIID were identified which described two cases of BIID, whereas in the Chinese databases only BIID-related conditions were found. This article reports some preliminary evidence that BIID is also present in non-Western countries. However, making general statements about the biological background of the disorder is hampered by the extremely low number of cases found. This low number possibly resulted from the extreme secrecy associated with the disorder, perhaps even more so in Asian countries.

Endovascular Approach to Glomus Jugulare Tumors.

PubMed

Kocur, Damian; Ślusarczyk, Wojciech; Przybyłko, Nikodem; Hofman, Mariusz; Jamróz, Tomasz; Suszyński, Krzysztof; Baron, Jan; Kwiek, Stanisław

2017-01-01

Paragangliomas are benign neuroendocrine tumors derived from the glomus cells of the vegetative nervous system. Typically, they are located in the region of the jugular bulb and middle ear. The optimal management is controversial and can include surgical excision, stereotactic radiosurgery and embolization. We report the endovascular approach to three patients harboring glomus jugulare paragangliomas. In all cases incomplete occlusion of the lesions was achieved and recanalization in the follow-up period was revealed. Two patients presented no clinical improvement and the remaining one experienced a transient withdrawal of tinnitus. It is technically difficult to achieve complete obliteration of glomus jugulare tumors with the use of embolization and the subtotal occlusion poses a high risk of revascularization and is not beneficial in terms of alleviating clinical symptoms.

A Case-Cohort Study of Cadmium Body Burden and Gestational Diabetes Mellitus in American Women.

PubMed

Romano, Megan E; Enquobahrie, Daniel A; Simpson, Christopher D; Checkoway, Harvey; Williams, Michelle A

2015-10-01

Environmental cadmium (Cd) exposure is associated with type 2 diabetes. However, the association of Cd and gestational diabetes mellitus (GDM) is unknown. We examined the association between body burden of Cd and GDM risk. We used 140 GDM cases and 481 randomly selected noncase subcohort members from the Omega Study to conduct a case-cohort study. Creatinine (Cr)-corrected Cd in early pregnancy urine (U-Cd) was measured by inductively coupled plasma mass spectrometry. Tertiles (< 0.29; 0.29-0.42; ≥ 0.43 μg/g Cr) were defined using the subcohort's U-Cd distribution. GDM was diagnosed using the 2004 American Diabetes Association guidelines. Adjusted odds ratios (ORs) and 95% confidence intervals (CIs) were estimated using logistic regression. GDM cases had higher geometric mean U-Cd (0.39 μg/g Cr; 95% CI: 0.37, 0.41) than noncases (0.31 μg/g Cr; 95% CI: 0.29, 0.33). Odds ratios for GDM increased with increasing U-Cd tertile (OR = 1.64; 95% CI: 0.88, 3.05 for middle vs. low tertile; OR = 2.07; 95% CI: 1.15, 3.73 for high vs. low tertile; p-trend = 0.015). Overweight/obesity (body mass index ≥ 25 kg/m(2)) did not modify the association between U-Cd and GDM (p = 0.26). Our findings suggest that body burden of Cd increases risk of GDM in a dose-dependent manner. Improved understanding of environmental factors influencing GDM may facilitate early identification of women at high risk of GDM.

Foreign bodies in the larynx and tracheobronchial tree in children. A review of 225 cases.

PubMed

Rothmann, B F; Boeckman, C R

1980-01-01

In a study of 225 patients with foreign bodies in the larynx, trachea and bronchi, 77% were 36 months of age or less. The male-female ratio was 2:1. Food or food derivatives were the causative agent in 70% of the cases, with 38% due to a portion of nut. The foreign body involved the right and left bronchus with equal frequency. A choking episode followed by an audible wheeze (55%) was the most common presenting complaint. Obstructive emphysema was demonstrated in 60% and was best demonstrated by inspiration-expiration chest roentgenograms or fluoroscopy. A radio-opaque object was seen in 13%. Two hundred ten foreign objects (93%) were removed by endoscopy. Four patients required pulmonary resection for bronchiectasis and in three patients bronchotomy was performed. Five patients expelled the foreign body spontaneously, two patients were transferred to another hospital, and one foreign body was not recovered. There was no mortality.

Decomposed bodies--still an unrewarding autopsy?

PubMed

Ambade, Vipul Namdeorao; Keoliya, Ajay Narmadaprasad; Deokar, Ravindra Baliram; Dixit, Pradip Gangadhar

2011-04-01

One of the classic mistakes in forensic pathology is to regard the autopsy of decomposed body as unrewarding. The present study was undertaken with a view to debunk this myth and to determine the characteristic pattern in decomposed bodies brought for medicolegal autopsy. From a total of 4997 medicolegal deaths reported at an Apex Medical Centre, Yeotmal, a rural district of Maharashtra over seven year study period, only 180 cases were decomposed, representing 3.6% of the total medicolegal autopsies with the rate of 1.5 decomposed body/100,000 population per year. Male (79.4%) predominance was seen in decomposed bodies with male female ratio of 3.9:1. Most of the victims were between the ages of 31 and 60 years with peak at 31-40 years (26.7%) followed by 41-50 years (19.4%). Older age above 60 years was found in 8.6% cases. Married (64.4%) outnumbered unmarried ones in decomposition. Most of the decomposed bodies were complete (83.9%) and identified (75%). But when the body was incomplete/mutilated or skeletonised then 57.7% of the deceased remains unidentified. The cause and manner of death was ascertained in 85.6% and 81.1% cases respectively. Drowning (35.6%) was the commonest cause of death in decomposed bodies with suicide (52.8%) as the commonest manner of death. Decomposed bodies were commonly recovered from open places (43.9%), followed by water sources (43.3%) and enclosed place (12.2%). Most of the decomposed bodies were retrieved from well (49 cases) followed by barren land (27 cases) and forest (17 cases). 83.8% of the decomposed bodies were recovered before 72 h and only in 16.2% cases the time since death was more than 72 h, mostly recovered from barren land, forest and river. Most of the decomposed bodies were found in summer season (42.8%) with peak in the month of May. Despite technical difficulties in handling the body and artefactual alteration of the tissue, the decomposed body may still reveal cause and manner of death in significant number

A Case of Treatment- resistant Depression and Body Dysmorphic Disorder: The Role of Electroconvulsive Therapy Revisited.

PubMed

Mahato, Ram S; San Gabriel, Maria Chona P; Longshore, Carrol T; Schnur, David B

2016-01-01

Body dysmorphic disorder is a common, often disabling condition, and is frequently comorbid with major depressive disorder. Selective serotonin reuptake inhibitors constitute first line set of somatic interventions but the management of refractory patients remains challenging. Electroconvulsive therapy, an often highly beneficial treatment for medication resistant-depression, is not considered an effective therapeutic alternative for treatment refractory body dysmorphic disorder. Here we present a 50-year-old woman with body dysmorphic disorder and comorbid major depressive disorder who remained incapacitated and suicidal despite several trials with selective serotonin reuptake inhibitors and antipsychotic medication. Depressive and dysmorphic symptoms appeared to resolve with electroconvulsive therapy, and remission was sustained for two months. Electroconvulsive therapy has an important place in the management of treatment- resistant depression associated with body dysmorphic disorder, and, in select cases, may be effective for dysmorphic symptoms as well.

Management of mandibular body fractures in pediatric patients: a case report with review of literature.

PubMed

John, Baby; John, Reena R; Stalin, A; Elango, Indumathi

2010-10-01

Mandibular fractures are relatively less frequent in children when compared to adults, which may be due to the child's protected anatomic features and infrequent exposure of children to alcohol related traffic accidents. Treatment principles of mandibular fractures differ from that of adults due to concerns regarding mandibular growth and development of dentition. A case of a 4.5-year-old boy with fractured body of mandible managed by closed reduction using open occlusal acrylic splint and circum mandibular wiring is presented. This article also provides a review of literature regarding the management of mandibular body fracture in young children.

Management of mandibular body fractures in pediatric patients: A case report with review of literature

PubMed Central

John, Baby; John, Reena R.; Stalin, A.; Elango, Indumathi

2010-01-01

Mandibular fractures are relatively less frequent in children when compared to adults, which may be due to the child's protected anatomic features and infrequent exposure of children to alcohol related traffic accidents. Treatment principles of mandibular fractures differ from that of adults due to concerns regarding mandibular growth and development of dentition. A case of a 4.5-year-old boy with fractured body of mandible managed by closed reduction using open occlusal acrylic splint and circum mandibular wiring is presented. This article also provides a review of literature regarding the management of mandibular body fracture in young children. PMID:22114443

Russell body gastritis with Dutcher bodies evaluated using magnification endoscopy

PubMed Central

Yorita, Kenji; Iwasaki, Takehiro; Uchita, Kunihisa; Kuroda, Naoto; Kojima, Koji; Iwamura, Shinichi; Tsutsumi, Yutaka; Ohno, Akinobu; Kataoka, Hiroaki

2017-01-01

Russell body gastritis (RBG) is an unusual type of chronic gastritis characterized by marked infiltration of Mott cells, which are plasma cells filled with spherical eosinophilic bodies referred to as Russell bodies. It was initially thought that Helicobacter pylori (H. pylori) infection was a major cause of RBG and that the infiltrating Mott cells were polyphenotypic; however, a number of cases of RBG without H. pylori infection or with monoclonal Mott cells have been reported. Thus, diagnostic difficulty exists in distinguishing RBG with monoclonal Mott cells from malignant lymphoma. Here, we report an unusual case of an 86-year-old-Japanese man with H. pylori-positive RBG. During the examination of melena, endoscopic evaluation confirmed a 13-mm whitish, flat lesion in the gastric antrum. Magnification endoscopy with narrow-band imaging suggested that the lesion was most likely a poorly differentiated adenocarcinoma. Biopsy findings were consistent with chronic gastritis with many Mott cells with intranuclear inclusions referred to as Dutcher bodies. Endoscopic submucosal dissection confirmed the diagnosis of RBG with kappa-restricted monoclonal Mott cells. Malignant lymphoma was unlikely given the paucity of cytological atypia and Ki-67 immunoreactivity of monoclonal Mott cells. This is the first reported case of RBG with endoscopic diagnosis of malignant tumor and the presence of Dutcher bodies. PMID:28874963

Time since death and decomposition of the human body: variables and observations in case and experimental field studies.

PubMed

Mann, R W; Bass, W M; Meadows, L

1990-01-01

Much of the difficulty in determining the time since death stems from the lack of systematic observation and research on the decomposition rate of the human body. Continuing studies conducted at the University of Tennessee, Knoxville, provide useful information on the impact of carrion insect activity, ambient temperature, rainfall, clothing, burial and depth, carnivores, bodily trauma, body weight, and the surface with which the body is in contact. This paper reports findings and observations accumulated during eight years of research and case studies that may clarify some of the questions concerning bodily decay.

Esophageal foreign bodies: 177 cases.

PubMed

Nadir, A; Sahin, E; Nadir, I; Karadayi, S; Kaptanoglu, M

2011-01-01

The aim of this study is to assess patients treated for esophageal foreign bodies. The charts of patients (n=177), between January 1994 and April 2009, were investigated retrospectively. Patients' age and gender, locations and types of foreign bodies (FBs) and interventions were taken into consideration. Fifty-seven percent of the patients were male. The youngest patient was 6 months, whereas the oldest was 83 years old. The median age was 9 years. Half of the patients were in their first decade. Treatment took place 11 h (ranging from 1 to 120 h) after impaction of the FB. One hundred fifty-two FBs were removed in 177 patients. Our negative esophagoscopy (n=25) rate was 14%. The FBs were radiopaque in 75% (n=114) and were commonly (71%; n=109) located in the cervical esophagus. Metallic coins (n=81-53%) were the most commonly observed inorganic FB while bones and/or meat impaction (n=54-35%) were the most frequent organic FB. A total of 182 endoscopic interventions were performed on these patients. One hundred eleven of them were esophagoscopy and the remaining 71 were direct laryngoscopy. The FB was pushed into the stomach in 11 patients. Our morbidity rate was 1.6% (n=3). Iatrogenic perforation occurred in two patients. There was no mortality. Esophageal FBs may vary in type: sharp or round objects, metallic, plastic or organic material. FBs are commonly found at pharyngoesophageal junction and usually removed by McGill forceps. Rigid esophagoscopy is used for children and adults because of its large working channel. Rigid instruments are considered reliable and safe for extracting foreign bodies. © 2010 Copyright the Authors. Journal compilation © 2010, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

The Canadian Legal System, the Robert Latimer Case, and the Rhetorical Construction of (Dis)ability: "Bodies that Matter?"

ERIC Educational Resources Information Center

Hayward, Sally

2009-01-01

This paper considers Judge Ted Noble's 1997 ruling of the Latimer case in terms of how it rhetorically constructs and privileges the normal, able-bodied status quo, while, at the same time, deconstructs and positions as inferior the "abnormal," dis-abled minority. In this case, Noble not only took the unprecedented step of granting…

Fluoroscopy-Guided Endoscopic Removal of Foreign Bodies.

PubMed

Kim, Junhwan; Ahn, Ji Yong; So, Seol; Lee, Mingee; Oh, Kyunghwan; Jung, Hwoon-Yong

2017-03-01

In most cases of ingested foreign bodies, endoscopy is the first treatment of choice. Moreover, emergency endoscopic removal is required for sharp and pointed foreign bodies such as animal or fish bones, food boluses, and button batteries due to the increased risks of perforation, obstruction, and bleeding. Here, we presented two cases that needed emergency endoscopic removal of foreign bodies without sufficient fasting time. Foreign bodies could not be visualized by endoscopy due to food residue; therefore, fluoroscopic imaging was utilized for endoscopic removal of foreign bodies in both cases.

Near-death experience and out of body phenomenon during torture--a case report.

PubMed

Cooper, Maxwell J F

2011-01-01

A case of a near death experience (NDE) associated with an "Out of body" phenomenon in an African man as a result of torture is presented. Although NDEs occur in approximately ten per cent of survivors of cardiac arrest, case reports emerging from the medical examination of torture victims are lacking. This may be due to cultural/linguistic barriers and fear of disbelief. Low NDE incidence during torture would suggest that torture techniques rarely induce the critical brain ischaemia considered necessary to provoke an NDE. Alternatively psychological or physical characteristics of torture may render NDE harder to recall. Proof of low incidence during torture would counter the theory that NDEs are a psychological response to perceived threat of death. NDEs often induce transformational benefits in patients' lives and for this reason the author urges physicians to consider the possibility of NDE amongst torture victims under their care. A request for information about similar cases is made.

The phase topology of a special case of Goryachev integrability in rigid body dynamics

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ryabov, P. E., E-mail: orelryabov@mail.ru

2014-07-31

The phase topology of a special case of Goryachev integrability in the problem of motion of a rigid body in a fluid is investigated using the method of Boolean functions, which was developed by Kharlamov for algebraically separated systems. The bifurcation diagram of the moment map is found and the Fomenko invariant, which classifies the systems up to rough Liouville equivalence, is specified. Bibliography: 15 titles. (paper)

Orbitocranial wooden foreign body: a pre-, intra-, and postoperative chronicle: case report.

PubMed

Dunn, Ian F; Kim, Dong H; Rubin, Peter A; Blinder, Russell; Gates, Jonathan; Golby, Alexandra J

2009-08-01

Intraorbital wooden foreign bodies--usually from a low-velocity puncture--are elusive and demand a low threshold for further imaging. In patients with traumatic injuries, orbital and intracranial air from fractures may be present, and it is particularly easy to overlook a wooden fragment A 53-year-old equestrian was kicked in the face by the rear hoof of a horse. The event was captured on video by her husband. Although no obvious entry point in and around the eye was observed, her ocular examination was notable for superior orbital fissure syndrome and increasing intraocular pressure in the left eye. Closer inspection revealed a 5-mm laceration above her superior lid margin; imaging revealed a foreign body at the orbital apex with apparent communication with the cranial vault. We proceeded with cranio-orbital exploration because of the risk of continued ocular damage in the setting of increasing intraocular pressure and the potential for infection of both the eye and the intracranial space from a suspected foreign body. A 3.0 cm x 0.5 cm fragment was found lodged in the orbital apex and removed. The patient recovered well after surgery and a course of antibiotics and has returned to riding. This case report presents an algorithm for approaching cranio-orbital foreign objects of unclear identity and the favorable outcomes that may be achieved.

Prognosis of cocaine body-packers.

PubMed

de Prost, Nicolas; Lefebvre, Aurélie; Questel, Frank; Roche, Nicolas; Pourriat, Jean-Louis; Huchon, Gérard; Rabbat, Antoine

2005-07-01

To study the prognosis and complications of cocaine body-packing (concealment of cocaine in the body for transportation between countries). We retrospectively reviewed the files of all cocaine body-packers hospitalized during a 4-year period in a medico-judiciary emergency unit. Subjects included in the survey were identified from the hospital databases using ICD-10 codes. The Medico-Judiciary Emergency Unit of Hôtel-Dieu university hospital in Paris is a unique medical and surgical emergency unit receiving all patients in legal custody arrested at the two Paris international airports and suspected of body-packing. All the cases of cocaine body-packers (n=581) hospitalized between January 1999 and December 2002 were studied. They had been arrested at Paris airports while arriving from drug-producing countries. The mean number of carried packets was 70.0+/-20.4 (range 18-150). The mean duration of hospitalization was 5.0+/-1.6 days (range 1-18). No complication occurred in 573 body-packers cases. Eight subjects developed a complication requiring admission to an intensive care unit: six acute cocaine intoxications due to packet rupture and two intestinal occlusions. No one died. Surgical treatment was necessary in six cases. Good prognosis observed in these body-packers cases is due to the careful monitoring of asymptomatic patients, allowing early detection and treatment of complications. Surgical removal of the packets when complication occurs is warranted.

Localization and prediction of malignant potential in recurrent pheochromocytoma/paraganglioma (PCC/PGL) using 18F-FDG PET/CT.

PubMed

Fikri, Ahmad Saad Fathinul; Kroiss, A; Ahmad, A Z F; Zanariah, H; Lau, W F E; Uprimny, C; Donnemiller, E; Kendler, D; Nordin, A J; Virgolini, I J

2014-06-01

To our knowledge, data are lacking on the role of 18F-FDG PET/CT in the localization and prediction of neuroendocrine tumors, in particular the pheochromocytoma/paraganglioma (PCC/PGL) group. To evaluate the role of 18F-FDG PET/CT in localizing and predicting the malignant potential of PCC/PGL. Twenty-three consecutive patients with a history of PCC/PGL, presenting with symptoms related to catecholamine excess, underwent 18F-FDG PET/CT. Final confirmation of the diagnosis was made using the composite references. PET/CT findings were analyzed on a per-lesion basis and a per-patient basis. Tumor SUVmax was analyzed to predict the dichotomization of patient endpoints for the local disease and metastatic groups. We investigated 23 patients (10 men, 13 women) with a mean age of 46.43 ± 3.70 years. Serum catecholamine levels were elevated in 82.60% of these patients. There were 136 sites (mean SUVmax: 16.39 ± 3.47) of validated disease recurrence. The overall sensitivities for diagnostic CT, FDG PET, and FDG PET/CT were 86.02%, 87.50%, and 98.59%, respectively. Based on the composite references, 39.10% of patients had local disease. There were significant differences in the SUVmax distribution between the local disease and metastatic groups; a significant correlation was noted when a SUVmax cut-off was set at 9.2 (P<0.05). In recurrent PCC/PGL, diagnostic 18F-FDG PET/CT is a superior tool in the localization of recurrent tumors. Tumor SUVmax is a potentially useful predictor of malignant tumor potential. © The Foundation Acta Radiologica 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

The case of GWAS of obesity: does body weight control play by the rules?

PubMed

Müller, Manfred J; Geisler, Corinna; Blundell, John; Dulloo, Abdul; Schutz, Yves; Krawczak, Michael; Bosy-Westphal, Anja; Enderle, Janna; Heymsfield, Steven B

2018-05-24

As yet, genome-wide association studies (GWAS) have not added much to our understanding of the mechanisms of body weight control and of the etiology of obesity. This shortcoming is widely attributed to the complexity of the issues. The appeal of this explanation notwithstanding, we surmise that (i) an oversimplification of the phenotype (namely by the use of crude anthropometric traits) and (ii) a lack of sound concepts of body weight control and, thus, a lack of a clear research focus have impeded better insights most. The idea of searching for polygenetic mechanisms underlying common forms of obesity was born out of the impressive findings made for monogenetic forms of extreme obesity. In the case of common obesity, however, observational studies on normal weight and overweight subjects never provided any strong evidence for a tight internal control of body weight. In addition, empirical studies of weight changes in normal weight and overweight subjects revealed an intra-individual variance that was similar to inter-individual variance suggesting the absence of tight control of body weight. Not least, this lack of coerciveness is reflected by the present obesity epidemic. Finally, data on detailed body composition highlight that body weight is too heterogeneous a phenotype to be controlled as a single entity. In summary GWAS of obesity using crude anthropometric traits have likely been misled by popular heritability estimates that may have been inflated in the first place. To facilitate more robust and useful insights into the mechanisms of internal control of human body weight and, consequently, the genetic basis of obesity, we argue in favor of a broad discussion between scientists from the areas of integrative physiologic and of genomics. This discussion should aim at better conceived studies employing biologically more meaningful phenotypes based on in depth body composition analysis. To advance the scientific community-including the editors of our top

Habit Reversal Therapy for Body-Focused Repetitive Behaviors in Williams Syndrome: A Case Study

PubMed Central

Klein-Tasman, Bonita P.

2013-01-01

Williams syndrome (WS) is genetic neurodevelopmental disorder with a well-characterized cognitive and behavioral phenotype. Research has consistently demonstrated high rates of psychopathology in this population; however, little research has examined the use of empirically-supported psychosocial interventions in those with WS. The current case study reports on the use of Habit Reversal Therapy (HRT) to treat multiple body-focused repetitive behaviors in a child with WS. Although HRT is a well-established cognitive-behavioral intervention for body-focused repetitive behaviors, it has been infrequently used in populations with developmental disabilities. An etiologically-informed approach was used to adapt HRT to fit the known behavioral and cognitive phenotype of WS. Results suggest that HRT may be beneficial for this population. Modified treatment elements are described and future research areas highlighted. PMID:24357918

Osteoblastoma of body of the talus--Report of a rare case with atypical radiological features.

PubMed

Mir, Naseer Ahmed; Baba, Asif Nazir; Maajid, Saheel; Badoo, Abdul Rashid; Mir, Ghulam Rasool

2010-06-01

Osteoblastoma is a benign bone tumour found commonly in the spine and long tubular bones. Involvement of the talus is uncommon, and when present, is found in the neck of the talus. Osteoblastoma of the body of talus is a very rare entity. We report a young male, presenting as chronic ankle pain, with a radiolucent lesion with a thick periosteal shell in the body of the talus. Analysis of clinical, radiological and histological findings confirmed the diagnosis of osteoblastoma. The case is reported for the rarity of the site and atypical radiological features that osteoblastoma can present with. Copyright 2009 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.

Follicular adenoma in ectopic thyroid. A case-report.

PubMed

Consalvo, Vincenzo; Barbieri, Gerarda; Rossetti, Amalia Rosaria Rita; Romano, Mafalda; Contieri, Rosaria; Tramontano, Salvatore; Rescigno, Carmela; Infranzi, Massimo; Lombardi, Domenico

2017-01-01

The term ectopic thyroid refers to the presence of thyroid tissue located far from its usual anatomic placement and with no vascular connection to the main gland. The presence of swelling in atypical locations is diagnostically differentiated from other pathologies like pleomorphic adenoma or carcinoma, inflammatory lesions like sialadenitis, neurogenic tumors, paraganglioma, fibrolipoma and lymphadenopaties of diverse etiologies. Here we present the case of a submandibular ectopic thyroid in a 67year old woman. She came to our attention for a left submandibular swelling. The anamnesis did not show related pathologies, as well as blood tests. Diagnostic image studies and a FNAC were performed. The mass was surgically removed and histopatology showed a follicular adenoma in the contest of the capsulated lesion. It is important to not underestimate these types of lesions and procede with hematochemical, instrumental tests and above all surgery that can eliminate any diagnostic uncertainty and on the whole be therapeutic. It should not be forgotten that ectopic thyroid tissue can be a site for adenoma or papillary carcinoma and thus any watch and wait strategy should be avoided. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

[Double homicide with a damning bite mark on the offender´s body - case report].

PubMed

Kulvajtová, Markéta; Hladík, Jiří; Zemančíková, Lucie

2017-01-01

The authors deal with a case of a double homicide, the parental murder made by their older son, which was committed by a combination of stabbing and cutting wounds. The perpetra-tor tried to hide the bodies, so he doused them with gasoline and set fire to them on a lonely forest road. Then he ran over the remains by his car. The cause of death was in both cases open wound of the neck - autopsy findings of the woman´s body testified for a blood aspi-ration, on the man´s body it was not clear for a partial charring, so the possibilities were blood aspiration, air embolism or external bleeding with haemorrhagic shock. There were also many other injuries on the bodies, especially of the post-mortem origin. During the combat, one of the victims - the mother - bit the assailant´s left forearm. So a few days after creation the injury on murderer´s left upper extremity, the police technicians took photos and during the forensic autopsies of both bodies, authors created the impressions of the teeth. The utilities were loaned from Department of Stomatology of University Hospital Královské Vinohrady in Prague and they were designed for the general adult population. The work was more difficult for the state of mortal remains, which were damaged by combination of fire and blunt violence. However finally the impressions of each arch were made, and they were given to the experts from Institute of Criminalistic Prague, who compared them with pictures of the bite mark. The results of this analysis were used in a court as evidence against the perpetrator.

Case Study: Body Composition Periodization in an Olympic-Level Female Middle-Distance Runner Over a 9-Year Career.

PubMed

Stellingwerff, Trent

2018-05-25

This case study features an Olympic-level female middle-distance runner implementing a science-based approach to body composition periodization. Data are emerging to suggest that it is not sustainable from a health and/or performance perspective to be at peak body composition year-round, so body composition needs to be strategically periodized. Anthropometric (n = 44), hematological, other health measures, and 1,500-m race performances (n = 83) were periodically assessed throughout a 9-year career. General preparation phase (September to April) featured the athlete at ∼2-4% over ideal competition phase body weight (BW) and body fat (%), with optimal energy availability being prioritized. The competition body composition optimization phase (May to August) included creating an individualized time frame and caloric deficit with various feedback metrics (BW, performance, and hunger) to guide the process. There were significant seasonal fluctuations in anthropometric outcomes between phases (47.3 ± 0.8 vs. 48.3 ± 0.9 kg BW; 53.6 ± 7.8 vs. 61.6 ± 9.7 mm International Society for the Advancement of Kinanthropometry sum of 8 [So8] skinfolds; p < .01), and a significant correlation of decreasing So8 during the peak competition period over her career (r = -.838; p = .018). The range of body composition during the competition period was 46.0-48.0 kg BW and a So8 range was 42.0-55.9 mm. There were also significant positive correlations between slower 1,500-m race times and increasing So8 (r = .437; p < .01), estimated fat mass (r = .445; p < .01), and BW (r = .511; p < .0001). The athlete only had two career injuries. This case study demonstrates a body composition periodization approach that allowed for targeted peak yearly performances, which improved throughout her career, while maximizing training adaptation and long-term athlete health through optimal energy availability.

Surgical body modification and altruistic individualism: a case for cyborg ethics and methods.

PubMed

Frank, Arthur W

2003-12-01

Three cases of pediatric surgical body modification--limb lengthening, normalization of genitalia, and craniofacial surgery--are considered through the moral language used by those who experience these surgeries. This language has been described as altruistic individualism. Decision making remains individualist, but it also shows considerable concern for others; egoism is complementary with altruism. The altruistic individualist is one of many incompatible identities that are predicted and described by the figure of the cyborg. Cyborgs suggest both ethics and qualitative methods appropriate to surgically shaped children.

Impaired Integration of Emotional Faces and Affective Body Context in a Rare Case of Developmental Visual Agnosia

PubMed Central

Aviezer, Hillel; Hassin, Ran. R.; Bentin, Shlomo

2011-01-01

In the current study we examined the recognition of facial expressions embedded in emotionally expressive bodies in case LG, an individual with a rare form of developmental visual agnosia who suffers from severe prosopagnosia. Neuropsychological testing demonstrated that LG‘s agnosia is characterized by profoundly impaired visual integration. Unlike individuals with typical developmental prosopagnosia who display specific difficulties with face identity (but typically not expression) recognition, LG was also impaired at recognizing isolated facial expressions. By contrast, he successfully recognized the expressions portrayed by faceless emotional bodies handling affective paraphernalia. When presented with contextualized faces in emotional bodies his ability to detect the emotion expressed by a face did not improve even if it was embedded in an emotionally-congruent body context. Furthermore, in contrast to controls, LG displayed an abnormal pattern of contextual influence from emotionally-incongruent bodies. The results are interpreted in the context of a general integration deficit in developmental visual agnosia, suggesting that impaired integration may extend from the level of the face to the level of the full person. PMID:21482423

Migrating foreign body in the tracheobronchial tree of childen: report of two cases.

PubMed

Oviawe, O; Abhulimhen-Iyoha, B I; Obaseki, D E

2011-06-01

Tracheobronchial foreign bodies (FBs) especially in infants and children can cause respiratory obstruction and can lead to death if intervention is delayed. Two cases of migrating FBs in the tracheobronchial (T-b) tree with fatal outcome are reported: Case 1 was an 11-year-old girl with a ten-week history of episodes of fever, cough, breathlessness and progressive weight loss. No positive history of FB aspiration. She was cachetic and febrile with short attention span, dyspnoeic, a flattened left upper hemithorax, mediastinal shift and evidence of atelectasis. Case 2, a 5-year-old girl with a three-month history of aspiration of a tiny toy. She developed fever, cough and marked weight loss, developed severe respiratory distress three days prior to presentation. She was febrile, had grade II finger clubbing, markedly dyspnoeic, left mediastinal shift, and evidence of left sided atelectasis. X-ray films in Case 1 showed evidence of FB at various locations in the T-b tree, and complete opacification and atelectasis of left hemithorax in Case 2. Planned bronchoscopy could not be done as both patients were adjudged to be too severely ill for the procedure. Both patients died after 9 days and 8 hours of hospital stay, respectively. Postmortem revealed FB in right main bronchus in both patients (tooth and toy, respectively) and evidence of tuberculosis in Case 2. Fatal outcome is attributed to inappropriate healthcare seeking behaviour, lack of medical expertise and material resources in our health facilities.

Ignition of a human body by a modest external source: a case report.

PubMed

Palmiere, Cristian; Staub, Christian; La Harpe, Romano; Mangin, Patrice

2009-07-01

A case of sustained combustion of a human body that occurred in 2006 in Geneva, Switzerland, is presented. The body of a man was discovered at home and found to have been almost completely incinerated between the knees and the mid-chest, with less damage to the head, arms, lower legs and feet. His dog was also found dead just behind the house door. The external source of ignition was most likely a cigarette or a cigar. The chair in which the man had been sitting was largely consumed while other objects in the room exhibited only a brown oily or greasy coating and were virtually undamaged. Toxicological analyses carried out on the blood from the lower legs showed low levels of desalkylflurazepam. Alcohol concentration was 1.10 per thousand, carboxyhaemoglobin levels were 12% and cyanide concentration was 0.05 mg/L. Toxicological analyses carried out on the dog's blood showed carboxyhaemoglobin levels at 65%.

Death by heroin intoxication in a body pusher with an innovative packaging technique: case report and review of the literature.

PubMed

Visentin, Sindi; Bevilacqua, Greta; Giraudo, Chiara; Dengo, Caterina; Nalesso, Alessandro; Montisci, Massimo

2017-11-01

Death due to mechanical or chemical intoxication of heroin body packers, thanks to the continuous improvement in packaging techniques, are increasingly rare, and almost all the cases reported in the literature refer to drug swallowers. A case of fatal acute heroin intoxication in a body pusher with an unreported packaging technique is presented, and previous deaths due to heroin body packing are reviewed, taking into consideration imaging techniques performed, cause of death, toxicological analysis on biological and non-biological samples, as well as number, position and type of drug packages identified at the dissection of the body. The innovative packaging technique found in the present case, constituted by an external multilayer cellophane casing containing 16 smaller packages of hardened heroin powder, each one covered with cigarette paper and multiple layers of heat-sealed cellophane, was probably used to avoid both chemical complications of package rupture and to create a package with morphological and radiological features different from those reported by previous studies. Drug dealers, in fact, are continually looking for packaging methods that, besides being safer, minimize the risk of detection at the radiological examinations performed, thus increasing the number of false negative findings. The identification of new types of package is therefore important, in order to identify packages that do not have the typical radiological signs, both in order to protect the patient's health and to avoid the non-recognition of a drug carrier. Despite the presence of multilayer composition of both the smaller and the bigger external coverage, these new types of package did not guarantee the greater safety of the drug dealer. Copyright © 2017 Elsevier B.V. All rights reserved.

Haloperidol for severe anorexia nervosa restricting type with delusional body image disturbance: a nine-case chart review.

PubMed

Mauri, Mauro; Miniati, Mario; Mariani, Michela Giorgi; Ciberti, Agnese; Dell'Osso, Liliana

2013-09-01

Here we report on a case series chart review conducted on nine severe and treatment-resistant patients with anorexia nervosa, body mass index < 13 kg/m(2), and a delusional body image disturbance. Patients received low doses of haloperidol during hospitalization. Haloperidol was well tolerated. The delusional body image disturbance and the drive for thinness were subjectively perceived as less intense. BMI increased from the initial 12.2 ± 0.5 to 16.0 ± 1.5 kg/m(2). Mean pulse rate and blood pressure did not change significantly from admission to discharge (66 ± 11.6 bpm; 91/56 mmHg vs 77 ± 12.0 bpm; 102/66 mmHg). Mean of QTc, available from electrocardiograms performed during hospitalization, was 413 ± 38.5 ms (range 342-469 ms). Further investigations are warranted to elucidate clinical usefulness and safety of low doses of haloperidol for patients with treatment-resistant anorexia and delusional body image disturbance.

Inclusion body disease in snakes: a review and description of three cases in boa constrictors in Belgium.

PubMed

Vancraeynest, D; Pasmans, F; Martel, A; Chiers, K; Meulemans, G; Mast, J; Zwart, P; Ducatelle, R

2006-06-03

Inclusion body disease, a fatal disorder in Boidae, is reviewed, and three cases in boa constrictors, the first reported cases in Belgium, are described. The snakes showed nervous signs, and numerous eosinophilic intracytoplasmic inclusions, which are considered to be characteristic of the disease, were found in the liver and pancreas. The disease is suspected to be caused by a retrovirus, but transmission electron microscopic examinations of several tissues from one of the snakes did not reveal particles with a typical retroviral morphology.

Are patients with hormonally functional phaeochromocytoma and paraganglioma initially receiving a proper adrenoceptor blockade? A retrospective cohort study.

PubMed

Luiz, Henrique Vara; Tanchee, Mary Jane; Pavlatou, Maria G; Yu, Run; Nambuba, Joan; Wolf, Katherine; Prodanov, Tamara; Wesley, Robert; Adams, Karen; Fojo, Tito; Pacak, Karel

2016-07-01

Pharmacological treatment is mandatory in patients with hormonally functional phaeochromocytoma and paraganglioma (PHAEO/PGL). We evaluated if patients initially diagnosed with hormonally functional PHAEO/PGL by various medical subspecialties received proper adrenoceptor blockade, and analysed factors predicting the prescription of adequate treatment. In a retrospective cohort study, we reviewed data from patients initially diagnosed with hormonally functional PHAEO/PGL outside the National Institutes of Health and Cedars-Sinai Medical Center, who were referred to these institutions between January 2001 and April 2015. Logistic regression was used to assess factors associated with proper adrenoceptor blockade. A total of 381 patients were included. Adequate pharmacological treatment was prescribed to 69·3%, of which 93·1% received α-adrenoceptor blockers. Regarding patients who were inappropriately treated, 53% did not receive any medication. Independent predictors of the prescription of a proper blockade were the diagnosis by endocrinologists [odds ratio (OR) 4·14; 95% confidence interval (CI), 2·51-6·85; P < 0·001], the presence of high blood pressure (OR 5·94; 95% CI, 3·11-11·33; P < 0·001) and the evidence of metastasis (OR 5·96; 95% CI, 1·93-18·46; P = 0·002). Although most patients received adequate pharmacological treatment, almost one-third were either not treated or received inappropriate medications. The diagnosis by endocrinologists, the presence of high blood pressure and the evidence of metastatic disease were identified as independent predictors of a proper blockade. These results highlight the need to educate physicians about the importance of starting adequate adrenoceptor blockade in all patients with hormonally functional PHAEO/PGL. © 2016 John Wiley & Sons Ltd.

Age Estimation Based on Appearance of Gray Hair in Different Body Sites of Sri Lankan Autopsy Cases.

PubMed

Senanayake, Harshana Mahendra Kumara; Wickramasinghe, Nuwan Darshana

2017-07-01

Owing to the scanty evidence on usefulness of information of appearance of gray hair for age estimation, this study was conducted to estimate age based on the appearance of gray hair on different body sites in a sample of autopsy cases in Sri Lanka. A descriptive cross-sectional study was conducted in Teaching Hospital-Kurunegala during 2011 to 2013. Based on the pattern of the presence of gray hair in different body sites, six stages of gray hair were computed. The analysis 1155 autopsy cases revealed strong, positive correlations between age and appearance of gray hair in head, mustache, beard, and pubic area among males and strong, positive correlations between age and the appearance of gray hair in head and pubic area among females (p < 0.01). Our findings demonstrate the value of information of appearance of gray hair for age estimation in the field of forensic science. © 2016 American Academy of Forensic Sciences.

The effect of body shape on weight-for-height and mid-upper arm circumference based case definitions of acute malnutrition in Ethiopian children.

PubMed

Myatt, Mark; Duffield, Arabella; Seal, Andrew; Pasteur, Frances

2009-01-01

Nutritional anthropometry surveys from Somalia and Ethiopia have reported that standard weight-for-height z-score (WHZ) and mid-upper arm circumference (MUAC) case definitions return different estimates of the prevalence of acute malnutrition in pastoralist livelihood zones but similar estimates of the prevalence of acute malnutrition in the agrarian livelihood zones. A study undertaken in Somalia to investigate this finding reported that children from pastoralist livelihood zones tended to have longer limbs and lower SSRs than children from agrarian livelihood zones. The present study investigated the relationship between weight-for-height and body shape and the relationship between MUAC and body shape in different populations of Ethiopian children. Six cross-sectional nutritional anthropometry surveys were undertaken. The combined survey datasets form the study sample. Data sources were grouped according to the livelihood zone from which data originated (either settled agrarian or semi-nomadic pastoralist). Case definitions of acute malnutrition using WHZ calculated using the NCHS and WHO reference populations and MUAC uncorrected for age or height were used. The SSR was used as an index of body shape. The association between body shape and the different case definitions of acute malnutrition were investigated using standard statistical techniques. Weight-for-height and MUAC case definitions yielded similar estimates of the prevalence of acute malnutrition in agrarian children but different estimates of the prevalence of acute malnutrition in pastoralist children. These populations also exhibit different SSRs. The SSR is an important predictor of weight-for-height. The SSR is a poor predictor of MUAC. WHZ and WHZ case status in children are associated with body shape and may overestimate the prevalence of acute malnutrition in some populations. Consideration should be given as to whether WHZ should be replaced by MUAC for the purposes of estimating the prevalence

[Case with probable dementia with Lewy bodies, who shows reduplicative paramnesia and Capgras syndrome].

PubMed

Ohara, Kazuyuki; Morita, Yoshio

2006-01-01

We report a case of probable dementia with Lewy bodies (DLB), showing reduplicative paramnesia (RP) and Capgras syndrome (CS). The patient, a right-handed 60 year-old male, began to show progressive dementia. At the age of 65, he showed fluctuating cognitive impairment and recurrent visual hallucinations. His SPECT demonstrated hypoperfusion not in the medial temporal cortices, but in the parieto-occipital lobes, where the right hemisphere was dominantly hypoperfused. He was diagnosed with probable DLB. In addition to recurrent visual hallucinations, he showed a sense of self- (or others) transfiguration, consciousness of something non-existent (Leibhaftige Bewusstheit; Jaspers, K.), and fluctuating visuo-spacial impairment. At the age of 67, he gradually complained of his duplicative wives "sosie". Finally he went so far as to talk about a nameless phantom boarder. We considered that RP and CS of this case comprised a sense of self-(or others) transfiguration, misidentification of important persons and places, and productive symptoms such as consciousness of something non-existent (Leibhaftige Bewusstheit) and visual hallucinations. The above mentioned symptoms might be originated not only from the disturbance of visuospacial recognition, which involves the limbic system (especially amygdala), medial frontal cortex, and right hemisphere of the brain, but also from the disturbance of recursive consciousness, due to diffusely damaged brain regions with Lewy body pathology. (Authors' abstract)

The body's story: a case report of hypnosis and physiological narration of trauma.

PubMed

Pantesco, Victor F

2005-01-01

Adult Posttraumatic Stress Disorder secondary to childhood sexual abuse is clinically complicated by its increasingly noted deficient linguistic recording of the abuse, perhaps partially explaining consequent difficulties with verbalizing in therapy. A single case illustrates that hypnotically utilizing the body-emotion register of encrypted sexual abuse trauma may not only afford more naturalistic retrieval and purgation of the experience, but may also provide the very medium for the healing narrative required for recovery. The patient's original and continuing therapist was also present as support and observer for all but 1 of 25 hypnosis sessions. Treatment gains were robust at 3-year follow up. This case suggests that effective treatment for sexual abuse PTSD may in some instances reside in more nonverbally sensitive interventions not aiming to prove, probe, or process linguistic reconstructions of memory. This is the first published report of such a bodily narrative in hypnosis.

18F-FLT PET/CT in the Evaluation of Pheochromocytomas and Paragangliomas: A Pilot Study.

PubMed

Blanchet, Elise M; Taieb, David; Millo, Corina; Martucci, Victoria; Chen, Clara C; Merino, Maria; Herscovitch, Peter; Pacak, Karel

2015-12-01

(18)F-FDG PET/CT has been proven to be a highly sensitive method for pheochromocytomas/paragangliomas (PHEOs/PGLs) associated with succinate dehydrogenase (SDH) mutations. This finding has been attributed to altered tumor cell metabolism resulting from these mutations and does not provide additional prognostic information to genotype. Therefore, identification of new biomarkers for aggressiveness is needed. A high Ki-67 index was proposed to be an additional prognostic factor. This pilot study aimed to evaluate 3'-deoxy-3'-(18)F-fluorothymidine ((18)F-FLT) PET/CT, a PET proliferation tracer, as a potential imaging agent in a series of 12 PHEO/PGL patients with different genetic backgrounds, to compare (18)F-FLT uptake with (18)F-FDG PET/CT, and to evaluate classic factors of aggressiveness. Twelve patients (7 metastatic and 5 nonmetastatic) were prospectively evaluated with (18)F-FDG and (18)F-FLT and followed for at least 2 y after the initial imaging work-up. Uptake was assessed at a lesion level, visually and quantitatively by maximum standardized uptake values (SUVmax) for both tracers. (18)F-FLT uptake was compared with risk factors known to be linked with a poor prognosis in PGLs (SDHB-mutated status, lesion size, dopaminergic phenotype) and with (18)F-FDG uptake. In 12 patients, 77 lesions were assessed. All lesions had low (18)F-FLT uptake (median SUVmax, 2.25; range, 0.7-4.5). There was no apparent superiority of (18)F-FLT uptake in progressive lesions, and most of the lesions showed a mismatch, with high (18)F-FDG uptake (median SUVmax, 10.8; range, 1.1-79.0) contrasting with low (18)F-FLT uptake. This study suggests that PHEOs/PGLs-even those that progress-do not exhibit intense (18)F-FLT uptake. It provides the first in vivo demonstration that proliferation may not be a major determinant of (18)F-FDG uptake in these tumors. These findings provide new insight into the biologic behavior of PGL and suggest that antiproliferative agents may be suboptimal

Pooled analysis of two case-control studies in New Caledonia and French Polynesia of body mass index and differentiated thyroid cancer: the importance of body surface area.

PubMed

Cléro, Enora; Leux, Christophe; Brindel, Pauline; Truong, Thérèse; Anger, Antoinette; Teinturier, Cécile; Diallo, Ibrahima; Doyon, Françoise; Guénel, Pascal; de Vathaire, Florent

2010-11-01

New Caledonia and French Polynesia have among of the world highest thyroid cancer incidence rates. Studies have demonstrated a relationship between anthropometric parameters and the prevalence of cancer. In this study we evaluated further the relationship between body mass index (BMI) and other anthropometric parameters on the incidence of thyroid cancer in the New Caledonia and French Polynesia populations. We performed a pooled analysis of two case-control studies in New Caledonia and French Polynesia. We included a total of 554 cases (65 men and 489 women) of differentiated thyroid cancers and 776 population control subjects matched on sex, age, and study. Anthropometric factors (height, weight, BMI, body fat percentage [BF%], and body surface area [BSA]), at age 18 and before diagnosis, were analyzed by conditional logistic regression, adjusting for other independent risk factors. A high proportion of cases (73%) were overweight (25-29.9 kg/m(2)) or obese (≥30 kg/m(2)) before diagnosis of thyroid cancer (against 57% of control subjects). An increased risk of thyroid cancer was observed with greater height, weight, BMI, BF%, and BSA. The association of thyroid cancer risk with height, weight, BMI, and BF% did not remain when adjustment was made for BSA. By comparison, the odds ratios for the highest versus the lowest quartile of BSA at age 18 were 3.97 (95% confidence interval, 2.57-6.15; p < 0.001) for women and 4.06 (95% confidence interval, 1.03-16.06; p = 0.04) for men. The association between thyroid cancer risk and each of anthropometric factors did not depend on tumor size or menopausal status before diagnosis. Among anthropometric factors, BSA plays a dominant role in thyroid cancer risk and explains the apparent role of BMI.

Endoscopic management of foreign bodies in the upper gastrointestinal tract: An analysis of 846 cases in China

PubMed Central

Yuan, Fangfang; Tang, Xiaowei; Gong, Wei; Su, Lei; Zhang, Yali

2018-01-01

Foreign body ingestion is a relatively common occurrence, which may lead to morbidity and mortality. The aim of the present study was to report the experience of management of upper gastrointestinal foreign bodies by endoscopy in a large center. All patients who presented at the Department of Gastroenterology at Nanfang Hospital (Guangzhou, China) with complaints regarding upper gastrointestinal (GI) foreign body ingestion from December 1987 to December 2013. Hospital medical charts and endoscopic records were examined to evaluate etiology, treatment, and outcomes for these patients. A total of 846 patients were enrolled in the present study, from which foreign bodies were detected in 737 (87.1%) patients via X-ray or endoscopy. The objects most frequently ingested were bones (n=395, 53.6%). The detected foreign bodies were predominantly located in the cervical esophagus (n=325, 44.1%). Endoscopic foreign body extraction was successful in 92.5% of cases, whereas surgery was required in 6 patients. The most frequently used endoscopic accessory devices were retrieval forceps (n=480, 65.1%). The complication rate was 6.9%, including mucosal laceration (n=10) and others, all of which were managed conservatively. Associated GI diseases were reported in 74 (10.0%) patients, including postesophagectomy (n=34) and others. In conclusion, the endoscopic procedure was safe and effective for the removal of foreign bodies from the upper gastrointestinal tract, with a high success rate and low complication rate. PMID:29434711

Postmortem whole-body computed tomography angiography visualizing vascular rupture in a case of fatal car crash.

PubMed

Flach, Patricia M; Ross, Steffen G; Bolliger, Stephan A; Preiss, Ulrich S; Thali, Michael J; Spendlove, Danny

2010-01-01

In addition to the increasingly significant role of multislice computed tomography in forensic pathology, the performance of whole-body computed tomography angiography provides outstanding results. In this case, we were able to detect multiple injuries of the parenchymal organs in the upper abdomen as well as lesions of the brain parenchyma and vasculature of the neck. The radiologic findings showed complete concordance with the autopsy and even supplemented the autopsy findings in areas that are difficult to access via a manual dissection (such as the vasculature of the neck). This case shows how minimally invasive computed tomography angiography can serve as an invaluable adjunct to the classic autopsy procedure.

Case study on rehabilitation of a polluted urban water body in Yangtze River Basin.

PubMed

Wu, Juan; Cheng, Shuiping; Li, Zhu; Guo, Weijie; Zhong, Fei; Yin, Daqiang

2013-10-01

In the past three decades, the fast development of economy and urbanization has caused increasingly severe pollutions of urban water bodies in China. Consequently, eutrophication and deterioration of aquatic ecosystem, which is especially significant for aquatic vegetation, inevitably became a pervasive problem across the Yangtze River Basin. To rehabilitate the degraded urban water bodies, vegetation replanting is an important issue to improve water quality and to rehabilitate ecosystem. As a case study, a representative polluted urban river, Nanfeihe River, in Hefei City, Anhui Province, was chosen to be a rehabilitation target. In October 2009 and May 2010, 13 species of indigenous and prevalent macrophytes, including seven species emergent, one species floating leaved, and five species submersed macrophytes, were planted along the bank slopes and in the river. Through 1.5 years' replanting practice, the water quality and biodiversity of the river had been improved. The concentrations of total nitrogen (TN), total phosphorus (TP), and ammonia nitrogen (NH4 (+)-N) declined by 46.0, 39.5, and 60.4 %, respectively. The species of macrophytes increased from 14 to 60, and the biodiversity of phytoplankton rose significantly in the river (p<0.05). The biomasses of zooplankton and benthos were also improved after the vegetation replanting. The study confirmed that vegetation replanting could alleviate the increasing water pollution and rehabilitate the degraded aquatic ecosystem. The case study would be an example for polluted urban waters restoration in the middle-downstream area of Yangtze River Base.

Body Weight Gain and Hyperphagia After Administration of SGLT-2 Inhibitor: A Case Report

PubMed Central

Hamamoto, Hiromi; Noda, Mitsuhiko

2015-01-01

Patient: Male, 44 Final Diagnosis: Type 2 diabetes Symptoms: Hunger • increased appetite Medication: GLP-1 receptor agonist • SGLT-2 inhibitor Clinical Procedure: — Specialty: Internal Medicine/Diabetology Objective: Unusual or unexpected effect of treatment Background: A detailed description is given of a case we encountered in which unexpectedly marked weight gain occurred following a treatment switch from a GLP-1 receptor agonist to an SGLT-2 inhibitor. Case Report: The patient, a 44-year-old man with type 2 diabetes mellitus, had gained about 10 kg in weight in the previous year. Therefore, metformin was replaced with liraglutide to obtain reduction of body weight. Although the patient lost about 8 kg (7%), during the 18-month period on the medication, the weight loss stabilized; therefore, the treatment was again switched to tofogliflozin to obtain further reduction of body weight. However, the patient reported increasing hunger and an exaggerated appetite from week 3 onward after the start of tofogliflozin, and gained about 9 kg in weight within 2 weeks, associated with a tendency towards increased HbA1c; therefore, tofogliflozin was discontinued. Immediate reinstitution of liraglutide resulted in reduction of the increased appetite, weight, and HbA1c level. Conclusions: Caution should be exercised against hyperphagia and weight gain due to hunger that may occur following discontinuation of a GLP-1 receptor agonist and/or initiation of an SGLT-2 inhibitor. PMID:26638727

Engaging stakeholder communities as body image intervention partners: The Body Project as a case example.

PubMed

Becker, Carolyn Black; Perez, Marisol; Kilpela, Lisa Smith; Diedrichs, Phillippa C; Trujillo, Eva; Stice, Eric

2017-04-01

Despite recent advances in developing evidence-based psychological interventions, substantial changes are needed in the current system of intervention delivery to impact mental health on a global scale (Kazdin & Blase, 2011). Prevention offers one avenue for reaching large populations because prevention interventions often are amenable to scaling-up strategies, such as task-shifting to lay providers, which further facilitate community stakeholder partnerships. This paper discusses the dissemination and implementation of the Body Project, an evidence-based body image prevention program, across 6 diverse stakeholder partnerships that span academic, non-profit and business sectors at national and international levels. The paper details key elements of the Body Project that facilitated partnership development, dissemination and implementation, including use of community-based participatory research methods and a blended train-the-trainer and task-shifting approach. We observed consistent themes across partnerships, including: sharing decision making with community partners, engaging of community leaders as gatekeepers, emphasizing strengths of community partners, working within the community's structure, optimizing non-traditional and/or private financial resources, placing value on cost-effectiveness and sustainability, marketing the program, and supporting flexibility and creativity in developing strategies for evolution within the community and in research. Ideally, lessons learned with the Body Project can be generalized to implementation of other body image and eating disorder prevention programs. Copyright © 2015 Elsevier Ltd. All rights reserved.

Endoscopic techniques and management of foreign body ingestion and food bolus impaction in the upper gastrointestinal tract: a retrospective analysis of 139 cases.

PubMed

Katsinelos, Panagiotis; Kountouras, Jannis; Paroutoglou, George; Zavos, Christos; Mimidis, Kostas; Chatzimavroudis, Grigoris

2006-10-01

Ingested foreign bodies and food bolus impaction are frequently seen in endoscopic practice. Successful foreign body and food bolus removal may depend on the method used, the choice of device, and the experience level of the endoscopist, although few papers report experience and outcome of tertiary centers. To investigate the effectiveness of our protocol designed for removal of ingested foreign bodies and food boluses. We retrospectively reviewed all patients with a diagnosis of foreign body ingestion and food bolus impaction from 1994 to 2005 identified by computer search. Patients were excluded if medical record was incomplete. The analysis included 171 patients. Foreign bodies and impacted food boluses were found in 77 and 62 patients, respectively. In 32 cases (23%), the foreign bodies passed spontaneously through the gastrointestinal tract. The overall success rate for endoscopic management was obtained in 137 patients (98.6%). Surgical removal of a foreign body was required in only 2 cases (1.4%). According to the type and location of the foreign object and food bolus we used Dormia baskets, retrieval forceps, polypectomy snares, and all sizes of Roth net. No complications relating to the endoscopic procedure were observed; 50 patients (35.2%) had an underlying esophageal disease. Endoscopic removal of upper gastrointestinal tract foreign bodies and food bolus impaction is efficacious and safe. Especially the Roth net is the best device for safe retrieval of food boluses and button disc batteries.

Body to Scalp: Evolving Trends in Body Hair Transplantation

PubMed Central

Saxena, Kuldeep; Savant, Sandeep S.

2017-01-01

Follicular unit extraction (FUE) is becoming an increasingly popular method for hair restoration. As FUE leaves behind no linear scars, it is more suitable to harvest from various body areas including beard, chest, and extremities in hirsute individuals. Body hair characteristics such as thickness, length, and hair cycle may not completely match to that of the scalp hair. The techniques of harvesting body hairs are more time consuming, requiring higher degree of skill than regular scalp FUE. Body hair transplantation can be successfully used either alone or in combination with scalp hair in advanced grades of baldness, for improving the cosmetic appearance of hairlines and in scarring alopecia when there is paucity of donor scalp hair. Harvesting of body hairs opens up a new viable donor source for hair restoration surgeons, especially in cases of advanced Norwood grades five and above of androgenetic alopecia. PMID:28584752

A supersonic three-dimensional code for flow over blunt bodies: Program documentation and test cases

NASA Technical Reports Server (NTRS)

Chaussee, D. S.; Mcmillan, O. J.

1980-01-01

The use of a computer code for the calculation of steady, supersonic, three dimensional, inviscid flow over blunt bodies is illustrated. Input and output are given and explained for two cases: a pointed code of 20 deg half angle at 15 deg angle of attack in a free stream with M sub infinite = 7, and a cone-ogive-cylinder at 10 deg angle of attack with M sub infinite = 2.86. A source listing of the computer code is provided.

On the Coplanar Integrable Case of the Twice-Averaged Hill Problem with Central Body Oblateness

NASA Astrophysics Data System (ADS)

Vashkov'yak, M. A.

2018-01-01

The twice-averaged Hill problem with the oblateness of the central planet is considered in the case where its equatorial plane coincides with the plane of its orbital motion relative to the perturbing body. A qualitative study of this so-called coplanar integrable case was begun by Y. Kozai in 1963 and continued by M.L. Lidov and M.V. Yarskaya in 1974. However, no rigorous analytical solution of the problem can be obtained due to the complexity of the integrals. In this paper we obtain some quantitative evolution characteristics and propose an approximate constructive-analytical solution of the evolution system in the form of explicit time dependences of satellite orbit elements. The methodical accuracy has been estimated for several orbits of artificial lunar satellites by comparison with the numerical solution of the evolution system.

Body piercing with fatal consequences.

PubMed

Ranga, N; Jeffery, A J

2011-01-25

Body modifications such as piercings, tattoos and surgery have increased in popularity in recent times and have become more socially acceptable. The common complications of piercing different parts of the human anatomy are well-documented, including sepsis, allergic reactions and, more rarely, endocarditis and ischaemia. Deaths related to piercing complications are primarily septic in origin. In this case, a man in his 50s died due to complications of his multiple umbilical piercings. The cause of death was unusually linked to body modification; the umbilical piercings had ultimately led to a mesenteric infarction. Cases such as these are forensically important due to potential manslaughter charges that could be brought against a piercing establishment. More importantly, this case highlights another extreme complication of body modification. Fashion statements are always changing and impact upon many lives. It is important to highlight to people the potentially life-threatening complications of common piercing practices.

Open reduction-internal fixation of a navicular body fracture with dorsal displacement of the first and second cuneiforms: a case report.

PubMed

Andersen, Robert C; Neiderer, Katherine; Martin, Billy; Dancho, James

2013-01-01

Body fractures of the tarsal navicular are relatively uncommon. To date, there is little literature discussing a navicular body fracture with dorsal subluxation of the first and second cuneiforms over the navicular. This case study presents a 30-year-old patient with this injury. He underwent open reduction internal fixation of the navicular body fracture successfully but failed adequate reduction of the navicular cuneiform joint after ligamentous reconstruction. After revisional surgery, he also failed 6 weeks of percutanous pinning with Kirschner-wire fixation. When comparing the literature of a similar injury, the Lisfranc fracture disclocation, the same principles may apply. One should consider rigid open reduction internal fixation or even primary fusion to treat disclocation of the naviculocuneiform joint following a navicular body fracture.

Death by 'ice': fatal methamphetamine intoxication of a body packer case detected by postmortem computed tomography (PMCT) and validated by autopsy.

PubMed

Bin Abdul Rashid, Saiful Nizam; Rahim, Amir Saad Abdul; Thali, Michael J; Flach, Patricia M

2013-03-01

Fatal acute methamphetamine (MA) poisoning in cases of internal drug trafficking is rarely described in the literature. This case study reports an MA 'body packer' who died from fatal methamphetamine intoxication due to leaking drug packages in the alimentary tract. The deceased was examined by postmortem computed tomography (PMCT), and the results were correlated to subsequent autopsy and toxicological findings. The deceased was arrested by the police when he was found disoriented in the city of Kuala Lumpur. He was transferred to the emergency department on suspicion of drug abuse. The initial drug screening was reactive for amphetamines. Shortly after admission to the hospital, he died despite rigorous resuscitation attempts. The postmortem plain chest and abdominal radiographs revealed multiple suspicious opacities in the gastrointestinal tract attributable to body packages. An unenhanced whole body PMCT revealed twenty-five drug packages, twenty-four in the stomach and one in the transverse colon. At least two were disintegrating, and therefore leaking. The autopsy findings were consistent with the PMCT results. Toxicology confirmed the diagnosis of fatal methamphetamine intoxication.

Controlling Tumor Progression with Cyclophosphamide, Vincristine, and Dacarbazine Treatment Improves Survival in Patients with Metastatic and Unresectable Malignant Pheochromocytomas/Paragangliomas.

PubMed

Asai, Shiko; Katabami, Takuyuki; Tsuiki, Mika; Tanaka, Yasushi; Naruse, Mitsuhide

2017-04-01

Evidence has not been established to support that combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) improves survival in patients with malignant pheochromocytoma and paraganglioma (M-PPGL). To investigate the efficacy of CVD for this disease, we retrospectively analyzed data of 23 patients with metastatic and unresectable M-PPGL (mean age, 41.7 ± 15.4 years) who received at least 2 cycles of this regimen. The follow-up period after initiation of CVD ranged from 0.3 to 13.7 years, with a median of 3.3 years. CVD therapy achieved a complete tumor response (CR) in 1 patient (4%), a partial response (PR) in 5 (22%), stable disease (SD) in 5 (22%), and progressive disease (PD) in 13 (52%), respectively. All of the responders (CR and PR) but 6% of the non-responders (SD and PD) showed substantial biochemical improvement. The progression-free survival period in the responders was significantly longer than in the non-responders (p < 0.01). Although the overall survival and survival after the diagnosis of M-PPGL were longer in the responders than the non-responders, the difference was not statistically significant (p = 0.08). The progression-free and overall survival period were significantly longer in the non-progression group (CR, PR, and SD) than in the progression group (PD) (1.7 ± 3.3 vs. 0.3 ± 0.3 years, p < 0.01, and 4.6 ± 3.6 vs. 2.0 ± 3.7 years, p = 0.01, respectively). It is therefore suggested that CVD chemotherapy could be useful in controlling tumor progression and improving survival in patients with metastatic and progressive M-PPGL.

High specificity of spot urinary free metanephrines in diagnosis and prognosis of pheochromocytomas and paragangliomas by HPLC with electrochemical detection.

PubMed

Zuo, Ming; Zhen, Qianna; Zhang, Xiaoqing; Zou, Wenbi; Yang, Xiangchun; Tian, Gang; Shi, Zhenghu; Li, Qifu; Ding, Min

2018-03-01

The metanephrines (MNs) in plasma and urine were proposed as biomarkers for the diagnosis of pheochromocytomas and paragangliomas (PPGLs). However, plasma free MNs and 24h urinary fractionated MNs were not satisfactory enough in specificity for the diagnosis of PPGLs. Moreover, the collection of 24h urine was inconvenient. This work examined the diagnostic and prognostic efficiency of free MNs in spot urine for PPGLs. We measured free MNs concentration in spot urine and plasma of 28 PPGLs patients and 155 control subjects by HPLC with electrochemical detection. Postoperative free MNs levels in spot urine and plasma of 14 PPGLs patients were also determined. Creatinine (Cr) concentration was used for the correction of urine volume. The specificity of spot urinary free MNs/Cr in the diagnosis of PPGLs was significantly higher than that of plasma free MNs [normetanephrine (NMN), 98.7% (95.4%-99.8%) vs 93.0% (87.4%-96.6%); metanephrine (MN), 93.6% (88.5%-96.9%) vs 84.5% (77.5%-90.0%)]. Meanwhile, the positive likelihood ratios for spot urinary free NMN/Cr and MN/Cr were 69.21 and 13.29, compared with 12.68 and 5.30 for plasma free NMN and MN, respectively. For the PPGLs patients underwent surgery, the plasma free MNs level appeared an abnormal elevation and yielded false-positive results for some patients. Our findings were validated in an independent cohort, resulting in the specificity of 100% for both urinary free NMN/Cr and MN/Cr, and 97.3% and 83.8% for plasma free NMN and MN, respectively. Spot urinary free MNs/Cr, superior to plasma free MNs, presented a promising biomarker for the diagnosis and prognosis of PPGLs. Copyright © 2017 Elsevier B.V. All rights reserved.

A case of generalized bromhidrosis following whole-body depilatory laser.

PubMed

Helou, Josiane; Haber, Roger; Kechichian, Elio; Tomb, Roland

2015-01-01

Laser treatment is a widespread method for hair removal. Despite its very common use, side effects remain relatively rare and transient. Axillary hyperhidrosis and bromhidrosis have already been reported in the literature after depilatory lasers. We report here a novel side effect of total body bromhidrosis following hair removal laser. A 27-year-old man, phototype 3 underwent four sessions of total body depilatory laser, combining pulsed alexandrite and pulsed diode lasers. A few days afterwards, a generalized foul odor was noted and was resistant to regular deodorants and Aluminum chloride based antiperspirants. Possible mechanisms include the activation of dormant bacteria in the skin flora, sweat gland dysfunction, altered skin flora, sweat gland hormone receptor disturbances, and genetic factors. Total body bromhidrosis and hyperhidrosis are potential complications of total body laser hair removal.

Neighbourhood effects on body constitution-A case study of Hong Kong.

PubMed

Low, Chien Tat; Lai, Poh Chin; Li, Han Dong; Ho, Wai Kit; Wong, Paulina; Chen, Si; Wong, Wing Cheung

2016-06-01

Traditional Chinese Medicine (TCM) has long perceived environment as an integral part of the development of body constitution, which is a personal state of health closely related to disease presence. Despite of the ever-growing studies on the clinical effectiveness of TCM and the scientific linking between body constitution and diseases, the geographical influence on body constitution has yet remained an unexplored territory. This study sought to investigate whether the neighbourhood environment is relevant to the composition of body type of a population through statistical multilevel and Geographic Information Systems modelling. The analysis comprised 3277 participants who had completed their body type assessment between 2009 and 2012 inclusive. The multilevel analysis also took simultaneous accounts of both individual-level (gender, age, BMI, type of housing) and area-level (percent greenery, percent road surface, total road intersection, sky view factor, temperature, relative humidity, rainfall and social deprivation index) characteristics to explain geographical variation by body types. Significant random or place effects (p < 0.001) were identified in the multilevel models. The spatial variation of body constitution involved the dynamic interplay between individual and environmental factors. The findings amassed the first scientific indications to back the common belief that place does play a role in the development of body constitution and is worthy of further investigation. By considering spatial and personal attributes simultaneously, the study can yield valuable insights into the patterning of area variation in body constitution and disease presence. Copyright © 2016 Elsevier Ltd. All rights reserved.

PENETRATING OCULAR INJURY WITH RETAINED INTRAOCULAR FOREIGN BODY FROM DRYWALL.

PubMed

Syed, Reema; Kim, Sung-Hye; Palacio, Agustina; Nunery, William R; Schaal, Shlomit

2018-03-23

To present a case of open globe injury and retained intraocular foreign body secondary to drywall. Interventional case report. A 21-year-old man presented with corneal laceration, iris defect, and vitreous hemorrhage after hammering drywall. Computed tomography scan was negative for intraocular foreign body, but a drywall intraretinal foreign body was found on 25-gauge vitrectomy. Intraoperative findings and 6-month follow-up are presented. Intraocular foreign body must always be suspected in all cases of penetrating ocular trauma. Although magnetic resonance imaging is ideal in diagnosing nonmetallic foreign bodies, computed tomography scan with Hounsfield units should be used in an emergency setting.

Hypochondriasis as an early manifestation of dementia with Lewy bodies: an autopsied case report.

PubMed

Fujishiro, Hiroshige; Iritani, Shuji; Sekiguchi, Hirotaka; Habuchi, Chikako; Torii, Youta; Matsunaga, Shinji; Ozaki, Norio; Yoshida, Mari; Fujita, Kiyoshi

2016-03-01

Discrepancies between clinical and pathological diagnoses of dementia with Lewy bodies (DLB) may occur because the full disease progression remains unclear, especially during the early stage. Herein, we report the case of a 78-year-old Japanese man with hypochondriasis who had autopsy-confirmed limbic-type DLB pathology. He exhibited no core clinical features of DLB. We attempted to identify the clinicopathological correlations in the early stages of DLB. At the age of 77, he became hypochondriacal and exhibited progressive cognitive decline after the death of his wife. He was concerned about his poor physical condition, but hospital examinations did not identify any overtly abnormal findings. At 78 years of age, he consulted a neurologist with complaints of facial numbness and irritability. Neurological examination revealed no overt abnormality, and he scored 21 points on the Mini-Mental State Examination. Magnetic resonance imaging of the brain showed mild bilateral ventricular enlargement. The patient was clinically diagnosed as having possible Alzheimer's disease. Approximately 1 month after his consult, he died of acute pneumonia in a psychiatric hospital to which he had been admitted for severe aggressive behaviour. He exhibited no core clinical features pointing towards a clinical diagnosis of DLB. Neuropathological investigation revealed limbic-type Lewy body disease with concurrent minimum Alzheimer-type pathology, which corresponds to high-likelihood DLB pathology based on the Third Consortium DLB pathological criteria. The patient had minimum nigral degeneration, which is consistent with the absence of parkinsonism. This autopsied case suggests that some DLB patients exhibit hypochondriasis in the early stage of the disease, even if they lack the core clinical features of DLB. © 2015 The Authors. Psychogeriatrics © 2015 Japanese Psychogeriatric Society.

A cast metal core for a deformed implant body: case report.

PubMed

Soeno, Kohyoh; Jimbo, Ryo; Sawase, Takashi; Taira, Yohsuke

2006-12-01

The present report consists of a clinical evaluation of an osseointegrated implant using a cast metal core instead of abutment for a deformed implant body. The intramobile connector insert for the implant in the mandibular left first premolar region broke 7 years after the superstructure was attached to the implant system. The intramobile connector insert was replaced, and the prosthesis was reattached, but the new intramobile connector insert broke again 2 weeks later. A thorough examination confirmed deformation of the upper section of the implant body in the mandibular left first molar region. Breakage of the new intramobile connector insert in the mandibular left first premolar region was believed to be due to deformation of the implant body in the mandibular left first molar region. Therefore, a cast metal core was used to deal with the deformation of the implant body in this region. Although slight bone resorption was observed around the implant body after 5 years, no major problems were found in the implant body itself.

[Neuroanatomy of Isolated Body Lateropulsion].

PubMed

Nakazato, Yoshihiko; Tamura, Naotoshi; Ikeda, Kei; Tanaka, Ai; Yamamoto, Toshimasa

2016-03-01

Axial body lateropulsion, a phenomenon where the body is pulled toward the side of the lesion, with tendency of falling down, is the well-known transient feature of lateral medullary syndrome. In some cases, axial body lateropulsion occurs without vestibular and cerebellar symptoms (isolated body lateropulsion:[iBL]). Patients with iBL have a lesion located in the spinocerebellar tract, descending lateral vestibulospinal tract, vestibulo-thalamic pathway, dentatorubrothalamic pathway, or thalamocortical fascicle. This review deals with the anatomic basis and clinical significance of iBL.

The Sharma-Parthasarathy stochastic two-body problem

NASA Astrophysics Data System (ADS)

Cresson, J.; Pierret, F.; Puig, B.

2015-03-01

We study the Sharma-Parthasarathy stochastic two-body problem introduced by Sharma and Parthasarathy in ["Dynamics of a stochastically perturbed two-body problem," Proc. R. Soc. A 463, 979-1003 (2007)]. In particular, we focus on the preservation of some fundamental features of the classical two-body problem like the Hamiltonian structure and first integrals in the stochastic case. Numerical simulations are performed which illustrate the dynamical behaviour of the osculating elements as the semi-major axis, the eccentricity, and the pericenter. We also derive a stochastic version of Gauss's equations in the planar case.

Voices of Donors: Case Reports of Body Donation in Hong Kong

ERIC Educational Resources Information Center

Chiu, Hei Yeung; Ng, Kwok Sing; Ma, Sin Kwan; Chan, Chi Hung; Ng, Sheung Wah; Tipoe, George L.; Chan, Lap Ki

2012-01-01

Body donation is important for medical education and academic research. However, it is relatively rare in Hong Kong when compared with many Western countries. Comprehensive research has been performed on the motivation for body donation in Western countries; however, there is still insufficient research on body donation in Hong Kong to provide…

Body machine interfaces for neuromotor rehabilitation: a case study.

PubMed

Pierella, Camilla; Abdollahi, Farnaz; Farshchiansadegh, Ali; Pedersen, Jessica; Chen, David; Mussa-Ivaldi, Ferdinando A; Casadio, Maura

2014-01-01

High-level spinal cord injury (SCI) survivors face every day two related problems: recovering motor skills and regaining functional independence. Body machine interfaces (BoMIs) empower people with sever motor disabilities with the ability to control an external device, but they also offer the opportunity to focus concurrently on achieving rehabilitative goals. In this study we developed a portable, and low-cost BoMI that addresses both problems. The BoMI remaps the user's residual upper body mobility to the two coordinates of a cursor on a computer monitor. By controlling the cursor, the user can perform functional tasks, such as entering text and playing games. This framework also allows the mapping between the body and the cursor space to be modified, gradually challenging the user to exercise more impaired movements. With this approach, we were able to change the behavior of our SCI subject, who initially used almost exclusively his less impaired degrees of freedom - on the left side - for controlling the BoMI. At the end of the few practice sessions he had restored symmetry between left and right side of the body, with an increase of mobility and strength of all the degrees of freedom involved in the control of the interface. This is the first proof of concept that our BoMI can be used to control assistive devices and reach specific rehabilitative goals simultaneously.

Muscular development and lean body weight in body builders and weight lifters.

PubMed

Katch, V L; Katch, F I; Moffatt, R; Gittleson, M

1980-01-01

The extent of extreme muscular development in 39 males identified as body builders (N = 18), power weight lifters (N = 13), and Olympic weight lifters (N = 8) were studied. Body composition and anthropometric data, including calculations of pre-excess muscle body weight (scale weight minus excess muscle) were obtained. The lean body weight and percent fats of the subjects were: body builders = 74.6 kg, 9.3%; power weight lifters = 73.3 kg, 9.1%; and Olympic weight lifters = 68.2 kg, 10.8%. No group differences were present in frame size, percent fat, lean body weight, skinfolds, and diameter measurements. The only group differences were for the shoulders, chest, biceps relaxed and flexed, and forearm girths. In each case the body builders were larger. Calculations of excess muscle by the Behnke method revealed that the body builders had 15.6 kg excess muscle, power weight lifters 14.8 kg, and Olympic weight lifters 13.1 kg. Somatographic comparisons revealed only slight differences between the groups, while differences with reference man were substantial.

Body Weight Gain and Hyperphagia After Administration of SGLT-2 Inhibitor: A Case Report.

PubMed

Hamamoto, Hiromi; Noda, Mitsuhiko

2015-12-07

A detailed description is given of a case we encountered in which unexpectedly marked weight gain occurred following a treatment switch from a GLP-1 receptor agonist to an SGLT-2 inhibitor The patient, a 44-year-old man with type 2 diabetes mellitus, had gained about 10 kg in weight in the previous year. Therefore, metformin was replaced with liraglutide to obtain reduction of body weight. Although the patient lost about 8 kg (7%), during the 18-month period on the medication, the weight loss stabilized; therefore, the treatment was again switched to tofogliflozin to obtain further reduction of body weight. However, the patient reported increasing hunger and an exaggerated appetite from week 3 onward after the start of tofogliflozin, and gained about 9 kg in weight within 2 weeks, associated with a tendency towards increased HbA1c; therefore, tofogliflozin was discontinued. Immediate reinstitution of liraglutide resulted in reduction of the increased appetite, weight, and HbA1c level. Caution should be exercised against hyperphagia and weight gain due to hunger that may occur following discontinuation of a GLP-1 receptor agonist and/or initiation of an SGLT-2 inhibitor.

[A morbidity study of intraocular foreign bodies].

PubMed

Mocanu, C; Bădescu, S

1993-01-01

190 patients presenting intraocular foreign bodies, hospitalized between 1981-1991, are examined. The study of accidents: cases showed a clear prevalence of work accidents--127 cases (66.84%); 86 of these cases occurred in industry (45.26%), the rest of 41 occurred in agriculture (21.58%). The professions most exposed to such accidents are those of locksmith, mechanic in agriculture or automobile industry. The postsurgical evolution and the incidence of complications show that intraocular foreign bodies are wosse-making factors of ocular plagues, because of anatomical structures disorganization, transparency modifications and infectious complications implied, which determined the diminishing of visual acuity in 88.08% of cases. The big percentage of work accidents in the etiology of intraocular foreign bodies emphasize the great importance of foreign the work-protection rules (regarding a especially the wearing of protection glasses) and of introducing of automation in technological processes involving great risks.

Novel mutation in the TMEM127 gene associated with phaeochromocytoma.

PubMed

Elston, M S; Meyer-Rochow, G Y; Prosser, D; Love, D R; Conaglen, J V

2013-04-01

Phaeochromocytomas and paragangliomas are rare neuroendocrine tumours that arise from the adrenal glands or paraganglia (paragangliomas) within the abdomen, thorax and neck. Although it was originally suggested that approximately 10% of these tumours were inherited, it is now recognised that up to approximately 30% of these tumours are associated with a germline mutation in one of the phaeochromocytoma/paraganglioma susceptibility genes. Of the 12 currently known genes predisposing to these tumours, the TMEM127 gene is one of the more recently identified and appears to be present in approximately 2% of apparently sporadic phaeochromocytomas. We report a 33-year-old man who presented with an apparently sporadic adrenal phaeochromocytoma and was identified as carrying a novel TMEM127 germline mutation, p.Gln139X. Patients harbouring a germline TMEM127 mutation most commonly present with an apparently sporadic solitary adrenal phaeochromocytoma. Testing patients who present with a phaeochromocytoma or paraganglioma for an underlying germline mutation needs to be considered in all patients due to implications for family members, but a strategy based on clinical and immunohistochemical findings would be prudent to limit costs. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

Fatal aspiration of foreign bodies in infants and children.

PubMed

Bamber, Andrew R; Pryce, Jeremy; Ashworth, Michael; Sebire, Neil J

2014-02-01

To investigate the frequency, circumstances, demographics and autopsy findings of infants and children dying as a result of foreign body aspiration. Retrospective review of autopsy cases in children aged between seven days and 18 years, at one specialist centre over a 16-year period, in which death was the result of aspiration of a foreign body. Ten cases were identified out of a total autopsy population of 2165. Only one individual had an underlying diagnosis potentially contributing to aspiration. All but one case involved aspiration of food, with grapes being a feature of four cases. In cases with a prolonged survival interval, autopsy demonstrated bronchopneumonia and hypoxic-ischaemic encephalopathy. In the remaining cases autopsy findings were non-specific. Fatal aspiration of a foreign body is rare in this population. The cases involve normal children who aspirate food, particularly grapes. There are typically minimal, non-specific findings at autopsy.

Rectorrhagia and vaginal discharge caused by a vaginal foreign body--a case report and review of literature.

PubMed

Shiryazdi, S M; Heiranizadeh, N; Soltani, H R

2013-06-01

An 8-year-old girl was taken to an outpatient clinic of surgery suffering from rectorrhagia and purulent, smelly vaginal discharge. Colonoscopy and biopsy were done before referring and sulfasalazine regime was administrated for probable colitis. The surgeon performed a rigid rectosigmoidoscopy under general anesthesia and no positive evidence was found. Having examined the hymen, it was found to be intact but a purulent discharge was observed. After drying the discharge, a black foreign body was seen in the bottom of the vagina and brought out by a fine forceps. It was a toy's wheel. The patient and her mother both denied foreign body abusing history. It was recommended she go for a psychological consultation. Generally speaking, all young patients suffering from vaginal or rectal bleeding with or without discharge should have their vagina checked for a foreign body. What is recommended in such cases is to undergo sonography or plain radiography before considering invasive or non-invasive procedures for evaluating anal and sigmoid abnormalities. Last but not least, a vaginal irrigation, precise examination of hymen, and posterior fossa can prove very useful for detecting simple vaginal bodies that can lead to various vaginal, rectal and abdominal complications. Copyright © 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Impact of Surgical Resection of the Primary Tumor on Overall Survival in Patients With Metastatic Pheochromocytoma or Sympathetic Paraganglioma.

PubMed

Roman-Gonzalez, Alejandro; Zhou, Shouhao; Ayala-Ramirez, Montserrat; Shen, Chan; Waguespack, Steven G; Habra, Mouhammed A; Karam, Jose A; Perrier, Nancy; Wood, Christopher G; Jimenez, Camilo

2018-07-01

To determine whether primary tumor resection in patients with metastatic pheochromocytoma or paraganglioma (PPG) is associated with longer overall survival (OS). Patients with metastatic PPG have poor survival outcomes. The impact of surgical resection of the primary tumor on OS is not known. We retrospectively studied patients with metastatic PPG treated at the University of Texas, MD Anderson Cancer Center from January 2000 through January 2015. Kaplan-Meier analysis with log-rank tests was used to compare OS among patients undergoing primary tumor resection and patients not treated surgically. Propensity score method was applied to adjust for selection bias using demographic, clinical, biochemical, genetic, imaging, and pathologic information. A total of 113 patients with metastatic PPG were identified. Eighty-nine (79%) patients had surgery and 24 (21%) patients did not. Median OS was longer in patients who had surgery than in patients who did not [148 months, 95% confidence interval (CI) 112.8-183.2 months vs 36 months, 95% CI 27.2-44.8 months; P < 0.001].Fifty-three (46%) patients had synchronous metastases; of these patients, those who had surgery had longer OS than those who did not (85 months, 95% CI 64.5-105.4 months vs 36 months, 95% CI 29.7-42.3 months; P < 0.001). Patients who had surgery had a similar ECOG performance status to the ones who did not (P = 0.1798, two sample t test; P = 0.2449, Wilcoxon rank sum test). Univariate and propensity score analysis confirmed that patients treated with surgery had longer OS than those not treated surgically irrespective of age, race, primary tumor size and location, number of metastatic sites, and genetic background (log-rank P < 0.001).In patients with hormonally active tumors (70.8%), the symptoms of catecholamine excess improved after surgery. However, the tumor burden was a more important determinant of OS than hormonal secretion. Primary tumor resection in patients with metastatic PPG appeared to be

Long-term effects of a ketogenic diet on body composition and bone mineralization in GLUT-1 deficiency syndrome: a case series.

PubMed

Bertoli, Simona; Trentani, Claudia; Ferraris, Cinzia; De Giorgis, Valentina; Veggiotti, Pierangelo; Tagliabue, Anna

2014-06-01

The only known treatment of glucose transporter 1 deficiency syndrome (GLUT-1 DS) is a ketogenic diet (KD), which provides the brain with an alternative fuel. Studies in children with intractable epilepsy have shown that a prolonged KD can induce a progressive loss of bone mineral content associated with poor bone health status, probably as a consequence of a chronic acidic environment. The aim of this study is to determine the long-term effects of a KD on body composition and bone mineral status of patients with GLUT-1 DS, is currently unknown. In this case series, we report the changes in body composition and bone mineral status observed in three adult patients with GLUT-1 DS who have been treated with a KD for more than 5 y. A long-term KD did not produce appreciable changes in weight and body composition of adults with GLUT-1 DS. Moreover, we found no evidence of potential adverse effects of a KD on bone health. In summary, this case series contributes to a small but growing body of literature that investigated the potential long-term effects of a KD on bone health. Our data suggest that maintaining a KD for more than 5 y does not pose any major negative effects on body composition, bone mineral content, and bone mineral density in adults with GLUT-1 DS, a finding that is at variance with previous reports focusing on children with intractable epilepsy. Further studies with larger sizes are needed to confirm and expand our findings. Copyright © 2014 Elsevier Inc. All rights reserved.

Foreign body in ear, nose and oropharynx: experience from a tertiary hospital.

PubMed

Tiago, Romualdo Suzano Louzeiro; Salgado, Daniel Cauduro; Corrêa, Juliano Piotto; Pio, Márcio Ricardo Barros; Lambert, Ernani Edney

2006-01-01

The occurrence of foreign bodies in otorhinolaryngology is reason of constant searches for emergency services. To value the incidence of patients with foreign body, to analyze the clinical situation and the treatment in these cases. The prospective study was realized in 81 patients with diagnosis of foreign body of nose, ear or oropharynx in the otorhinolaryngology service of the Hospital do Servidor Público Municipal de São Paulo between april/2003 and march/2005. 57 cases of foreign body of ear, 13 cases of nose and 11 of oropharynx. These patients, 51.85% were men and 48.15% were women. The age average was 23 years old. The average of the evolution time was 18.36 days, being that 38.27% these cases were taken care in less 24 hours of evolution. Inside the total of patients, 83.95% received initial attendance in the otorhinolaryngology clinic, and 16.05% came of another service after some previous removal attempt. The most common symptom of the foreign bodies cases of oropharynx it was odinofagia present in 90.91% of the cases; in the foreign bodies of nose, the unilateral rhinorrhea and cacosmia were present in 46.15 of the cases; in the foreign bodies of ear, 38.60% evolved without symptoms and 28.07 with hipoacusia. The most frequent foreign body of oropharynx it was the fish spine (54.55%); in the nose it was the paper (30.77%); and in the ear it was the cotton (31.58%). The complications resulting of the presence of foreign body or about the manipulation of these had been found in 13 cases (16.05%). Most cases of foreign body conditions, in which a non-specialist professional or a non-professional person previously handles its removal, have a bad evolution with emerging complications. Such outcomes strengthen the fact that an otorhinolaryngologist using the proper equipment must treat patients with foreign body.

[Delayed recurrence of a phaechromocytoma with metastasis as a cause of severe hypertension in a woman with a surgically intervened retroperitoneal tumour].

PubMed

Martín Varas, C; García, P A de la Torre; Heras Benito, M; Urzola Rodríguez, G; Castro Ramos, J C

We present the case of a woman with a previous history of hypertension and retroperitoneal tumour. The histology was reported as a paraganglioma. There was no clinical follow-up. Years later, there was a recurrence of the phaeochromocytoma after presenting with a difficult to control hypertension, along with elevation of urinary catecholamines. The case aims to highlight the importance of performing a clinical follow-up in these patients, due to the risk of recurrence. Copyright © 2017 SEH-LELHA. Publicado por Elsevier España, S.L.U. All rights reserved.

Increase in pediatric magnet-related foreign bodies requiring emergency care.

PubMed

Silverman, Jonathan A; Brown, Julie C; Willis, Margaret M; Ebel, Beth E

2013-12-01

We describe magnetic foreign body injuries among children and obtain national estimates of magnetic foreign body injury incidence over time. We searched the National Electronic Injury Surveillance System for cases of magnetic foreign bodies in children younger than 21 years in the United States, from 2002 to 2011. Cases were analyzed by location: alimentary or respiratory tract, nasal cavity, ear canal, or genital area. We identified 893 cases of magnetic foreign bodies, corresponding to 22,581 magnetic foreign body cases during a 10-year period (95% confidence interval [CI] 17,694 to 27,469). Most magnetic foreign bodies were ingested (74%) or intranasal (21%). Mean age was 5.2 years for ingested magnetic foreign bodies and 10.1 years for nasal magnetic foreign bodies (difference 4.9; 95% CI 4.1 to 5.6), suggesting different circumstances of injury. The incidence of pediatric magnet ingestions increased from 2002 to 2003 from 0.57 cases per 100,000 children per year (95% CI 0.22 to 0.92) to a peak in 2010 to 2011 of 3.06 cases per 100,000 children per year (95% CI 2.16 to 3.96). Most ingested magnetic foreign bodies (73%) and multiple magnet ingestions (91%) occurred in 2007 or later. Patients were admitted in 15.7% of multiple magnet ingestions versus 2.3% of single magnet ingestions (difference 13.4%; 95% CI 2.8% to 24.0%). Magnet-related injuries are an increasing public health problem for young children, as well for older children who may use magnets for play or to imitate piercings. Education and improved magnet safety standards may decrease the risk small magnets pose to children. Copyright © 2013 American College of Emergency Physicians. Published by Mosby, Inc. All rights reserved.

Localization of activities in the human body with a whole-body counter.

PubMed

Fischer, H; Schlagbauer, M

2007-01-01

The whole-body counter of the Radiation Protection Unit at the ARC Seibersdorf research GmbH has two HP Ge-detectors for measuring radionuclides, which are internally deposited in the human body. The detector system has a scanning geometry, where one detector is placed below the bed and the other detector above the bed. The body counter is placed in a massive shielded chamber. This device is especially used for measuring radioactive exposed workers with the possibility of intake by inhalation and ingestion. In the most cases whole-body counters are calibrated with anthropomorphic phantoms where activity is homogenously distributed. However, in some cases radioactivity can be located as a 'Hot Spot' in an organ. The localisation of 'Hot spots' at least in one dimension was the topic of this work. Experiments were done by means of a water-filled bottle phantom where three point sources (137Cs, 133Ba and 60Co) were placed at different positions. Measurements show that these radionuclides can be located within 1.5 cm along the longitudinal axis of the phantom with activities for 137Cs of at least 240 Bq, 133Ba of at least 670 Bq and 60Co of at least 140 Bq.

"I've got a UFO stuck in my throat!"--an interesting case of foreign body impaction in the oesophagus.

PubMed

Yip, L W; Goh, F S; Sim, R S

1998-03-01

This is a case report of an elderly lady with odynophagia because she accidentally swallowed a tablet which was still wrapped in its blister pack. A discussion of foreign body ingestion, particularly in the elderly, is included. To the authors' knowledge, this is the first paper that includes a lateral cervical radiograph of an ingested blister pack.

The Sharma-Parthasarathy stochastic two-body problem

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cresson, J.; SYRTE/Observatoire de Paris, 75014 Paris; Pierret, F.

2015-03-15

We study the Sharma-Parthasarathy stochastic two-body problem introduced by Sharma and Parthasarathy in [“Dynamics of a stochastically perturbed two-body problem,” Proc. R. Soc. A 463, 979-1003 (2007)]. In particular, we focus on the preservation of some fundamental features of the classical two-body problem like the Hamiltonian structure and first integrals in the stochastic case. Numerical simulations are performed which illustrate the dynamical behaviour of the osculating elements as the semi-major axis, the eccentricity, and the pericenter. We also derive a stochastic version of Gauss’s equations in the planar case.

Genetic testing and private insurance--a case of "selling one's body"?

PubMed

Hübner, D

2006-01-01

Arguments against the possible use of genetic test results in private health and life insurance predominantly refer to the problem of certain gene carriers failing to obtain affordable insurance cover. However, some moral intuitions speaking against this practice seem to be more fundamental than mere concerns about adverse distributional effects. In their perspective, the central ethical problem is not that some people might fail to get insurance cover because of their 'bad genes', but rather that some people would manage to get insurance cover because of their 'good genes'. This paper tries to highlight the ethical background of these intuitions. Their guiding idea appears to be that, by pointing to his favourable test results, a customer might make an attempt to 'sell his body'. The rationale of this concept is developed and its applicability to the case at issue is critically investigated. The aim is to clarify an essential objection against the use of genetic information in private insurance which has not yet been openly addressed in the academic debate of the topic.

Abdominal Aortic Dissection and Cold-Intolerance After Whole-Body Cryotherapy: A Case Report.

PubMed

Cámara-Lemarroy, Carlos R; Azpiri-López, José R; Vázquez-Díaz, Luis A; Galarza-Delgado, Dionicio A

2017-09-01

Whole-body cryotherapy (WBC) involves short exposures to air temperatures below -100°C and is purported to enhance recovery after exercise and accelerate rehabilitation after injury. It is generally considered a procedure with few side effects, but there are no large studies that have established its safety profile. We present the case of a 56-year-old patient who developed an abdominal aortic dissection after receiving 15 sessions of WBC. The patient had no other strong risk factors for aortic dissection. Exposure to cold temperatures, including WBC, has multiple hemodynamic effects, including increases in blood pressure, heart rate, and an adrenergic response. We suggest that these changes could act as a trigger for the onset of aortic dissections. This could be the first reported cardiovascular complication associated with WBC.

Body composition in males with adolescent idiopathic scoliosis: a case-control study with dual-energy X-ray absorptiometry.

PubMed

Wang, Weijun; Wang, Zhiwei; Zhu, Zezhang; Zhu, Feng; Qiu, Yong

2016-02-29

In contrast to the well-characterized body growth and development of females with adolescent idiopathic scoliosis (AIS), the pubertal growth pattern of male patients has not been well-documented. Recently, significantly lower body weight (BW) and body mass index (BMI) were reported in males with AIS, and were thought to be related to curve progression. A case-control study was carried out to characterize the body composition and bone status of males with AIS, with the aim of gaining a better understanding of lower BW among these patients. Forty-seven males with AIS and forty age- and gender-matched healthy controls were recruited. Standing height (SH) and BW were measured. The SH of the males who had AIS was corrected using Bjure's equation, and then the BMI was calculated. Body composition, including subcranial fat mass (FM), lean mass (LM), and bone mineral content (BMC), and bone mineral density (BMD) were analyzed using dual-energy X-ray absorptiometry. The LM index (LMi) and the FM index (FMi) were calculated by dividing the FM and LM by the square of the SH. Logistic regression analysis was employed for comparison between AIS and controls. The AIS patients had comparable age and Tanner staging for pubic hair as the controls. After adjustment for age, the AIS patients showed comparable SH but significantly lower BW and BMI than that of the controls. The LM, LMi, BMC and BMD were also significantly lower in the AIS patients than in the controls. However, the difference in BMC between two groups was not significant by adjusting for age, FM and LM. The male AIS patients showed abnormal body composition, presenting as significantly lower LM than the controls. The lower BMC observed in the patients might due to the abnormal body composition.

Fluoroscopy-Guided Percutaneous Vertebral Body Biopsy Using a Novel Drill-Powered Device: Technical Case Series

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wallace, Adam N., E-mail: wallacea@mir.wustl.edu; Pacheco, Rafael A., E-mail: pachecor@mir.wustl.edu; Tomasian, Anderanik, E-mail: tomasiana@mir.wustl.edu

2016-02-15

BackgroundA novel coaxial biopsy system powered by a handheld drill has recently been introduced for percutaneous bone biopsy. This technical note describes our initial experience performing fluoroscopy-guided vertebral body biopsies with this system, compares the yield of drill-assisted biopsy specimens with those obtained using a manual technique, and assesses the histologic adequacy of specimens obtained with drill assistance.MethodsMedical records of all single-level, fluoroscopy-guided vertebral body biopsies were reviewed. Procedural complications were documented according to the Society of Interventional Radiology classification. The total length of bone core obtained from drill-assisted biopsies was compared with that of matched manual biopsies. Pathology reportsmore » were reviewed to determine the histologic adequacy of specimens obtained with drill assistance.ResultsTwenty eight drill-assisted percutaneous vertebral body biopsies met study inclusion criteria. No acute complications were reported. Of the 86 % (24/28) of patients with clinical follow-up, no delayed complications were reported (median follow-up, 28 weeks; range 5–115 weeks). The median total length of bone core obtained from drill-assisted biopsies was 28 mm (range 8–120 mm). This was longer than that obtained from manual biopsies (median, 20 mm; range 5–45 mm; P = 0.03). Crush artifact was present in 11 % (3/28) of drill-assisted biopsy specimens, which in one case (3.6 %; 1/28) precluded definitive diagnosis.ConclusionsA drill-assisted, coaxial biopsy system can be used to safely obtain vertebral body core specimens under fluoroscopic guidance. The higher bone core yield obtained with drill assistance may be offset by the presence of crush artifact.« less

Creating Inclusive Physical Activity Spaces: The Case of Body-Positive Yoga.

PubMed

Pickett, Andrew C; Cunningham, George B

2017-09-01

Within the modern cultural climate, those in larger bodies face high levels of weight stigma, particularly in sport and physical activity spaces, which serves as a strong barrier to their participation. However, given the strong link between physical activity and general health and well-being for participants, it is important to explore strategies that encourage participation of these individuals. Thus, the current research examined strategies that physical activity instructors use to develop inclusive exercise spaces for all body sizes. This study employed a series of semistructured qualitative interviews (n = 9) with instructors of body-inclusive yoga classes to explore the ways in which they encourage participation for those in larger bodies. Emergent themes from the current study suggested support for 6 factors for creating body-inclusive physical activity spaces: authentic leadership, a culture of inclusion, a focus on health, inclusive language, leader social activism, and a sense of community. This study revealed that leaders must intentionally cultivate inclusion in their spaces to encourage those in nonconforming bodies to participate. These findings have important health and management implications for the sport and physical activity context and provide a basic outline of practical strategies that practitioners can use to foster inclusion in their spaces.

Foreign body aspiration in dentistry: a review.

PubMed

Cameron, S M; Whitlock, W L; Tabor, M S

1996-08-01

This article reviews the dangers of aspirating foreign bodies of dental origin. Two illustrative cases are presented, including an unusual case involving aspiration of an elastomeric impression material. The authors describe the techniques used to identify the foreign body. A radiodensimetric study of four impression materials demonstrates the difficulty of identifying most impression materials. The authors also present some strategies for reducing the risk of aspiration during dental procedures.

Cognitive-Behavioral Body Image Therapy for Body Dysmorphic Disorder.

ERIC Educational Resources Information Center

Rosen, James C.; And Others

1995-01-01

Randomly assigned 54 body dysmorphic disorder (BDD) subjects to cognitive behavior therapy or no treatment. BDD symptoms were significantly decreased in therapy subjects and the disorder was eliminated in 82 percent of cases at posttreatment and 77 percent at follow-up. Subjects' overall psychological symptoms and self-esteem also improved. (RJM)

Delayed vertebral body collapse after stereotactic radiosurgery and radiofrequency ablation: Case report with histopathologic-MRI correlation.

PubMed

Wallace, Adam N; Vyhmeister, Ross; Hsi, Andy C; Robinson, Clifford G; Chang, Randy O; Jennings, Jack W

2015-12-01

Stereotactic radiosurgery and percutaneous radiofrequency ablation are emerging therapies for pain palliation and local control of spinal metastases. However, the post-treatment imaging findings are not well characterized and the risk of long-term complications is unknown. We present the case of a 46-year-old woman with delayed vertebral body collapse after stereotactic radiosurgery and radiofrequency ablation of a painful lumbar metastasis. Histopathologic-MRI correlation confirmed osteonecrosis as the underlying etiology and demonstrated that treatment-induced vascular fibrosis and tumor progression can have identical imaging appearances. © The Author(s) 2015.

Comparison of absorption properties of colored dissolved organic matter in six different case 2 water bodies

NASA Astrophysics Data System (ADS)

Nima, Ciren; Frette, Øyvind; Hamre, Børge; Erga, Svein Rune; Chen, Yi-Chun; Zhao, Lu; Muyimbwa, Dennis; Ssenyonga, Taddeo; Ssebiyonga, Nicolausi; Okullo, Willy; Stamnes, Knut; Stamnes, Jakob J.

2017-02-01

Colored Dissolved Organic Matter (CDOM) is one of the main factors controlling the penetration of solar radiation in Case 2 water and affecting satellite-based estimation of ocean color. We present absorption properties of CDOM sampled in 6 water bodies including three in Norway (Røst coastal water, Samnangerfjord, Lysefjord), two in China (Bohai Sea, Lake Namtso), and one in Africa (Lake Victoria). These locations, which range from near the equator to subarctic regions, include water types from oligotrophic to eutrophic, and altitudes from sea level to 4,700 m above sea level.

Impacted thoracic foreign bodies after penetrating chest trauma.

PubMed

Sersar, Sameh I; Albohiri, Khalid A; Abdelmohty, Hysam

2016-10-01

Retained foreign bodies in the chest may include shell fragments, bullets, shrapnel, pieces of clothing, bones, and rib fragments. The risks of removal of foreign bodies must be weighed against the complications of leaving them inside the chest. We treated 90 cases of retained intrathoracic foreign bodies in patients admitted to 3 tertiary centers in Saudi Arabia between March 2015 and March 2016. Sixty patients were injured by shrapnel, 26 had one or more bullets, 3 had broken rib fragments, and one had a metal screw. The chest wall was site of impaction in 48 cases, the lungs in 24, pleura in 14, and mediastinum in 4. Removal of the retained foreign body was carried out in 12 patients only: bullets in 9 cases, bone fragments in 2, and a metal screw in one. The predictors for removal were bullets, female sex, and mediastinal position with bilateral chest injury, especially with fracture ribs. Retained intrathoracic foreign bodies due to penetrating chest trauma are treated mainly conservatively unless there is another indication for chest exploration. © The Author(s) 2016.

[Uncommon foreign body in a nose].

PubMed

Alagić-Smailbegović, Jasminka; Hadzić, Edina; Sutalo, Kamenko; Resić, Mudzahid

2007-01-01

Foreign body in the nose most frequently occurs in childhood. It could be of various origin, pieces of toys, paper and uncommon metal body. Consequences include one- sided nasal breathing problem, nasal secretion and in some cases pain and secretion become purulent. The aim of this paper is to present uncommon foreign body in the nose. RTG diagnosis and extraction are the methods of choice.

Body Imaging

NASA Technical Reports Server (NTRS)

1990-01-01

Magnetic Resonance Imaging (MRI) and Computer-aided Tomography (CT) images are often complementary. In most cases, MRI is good for viewing soft tissue but not bone, while CT images are good for bone but not always good for soft tissue discrimination. Physicians and engineers in the Department of Radiology at the University of Michigan Hospitals are developing a technique for combining the best features of MRI and CT scans to increase the accuracy of discriminating one type of body tissue from another. One of their research tools is a computer program called HICAP. The program can be used to distinguish between healthy and diseased tissue in body images.

NASA/MSFC's Calculation for Test Case 1a of ATAC-FSDC Workshop on After-body and Nozzle Flows

NASA Technical Reports Server (NTRS)

Ruf, Joseph H.

2006-01-01

Mr. Ruf of NASA/MSFC executed the CHEM computational fluid dynamics (CFD) code to provide a prediction of the test case 1 a for the ATAC-FSDC Workshop on After-body and Nozzle Flows. CHEM is used extensively at MSFC for a wide variety of fluid dynamic problems. These problems include; injector element flows, nozzle flows, feed line flows, turbomachinery flows, solid rocket motor internal flows, plume vehicle flow interactions, etc.

Asbestos bodies and the diagnosis of asbestosis in chrysotile workers

DOE Office of Scientific and Technical Information (OSTI.GOV)

Holden, J.; Churg, A.

1986-01-01

It has been suggested that because chrysotile asbestos forms asbestos bodies poorly, use of the traditional histologic requirements (diffuse interstitial fibrosis plus asbestos bodies) for the diagnosis of asbestosis, may lead to an underdiagnosis of this condition in workers exposed only to chrysotile. Lungs from 25 chrysotile miners with diffuse interstitial fibrosis were examined. Asbestos bodies were found easily in histologic section using hematoxylin and eosin stains in all cases. Mineralogic analysis of four cases showed that 46 of 72 (64%) bodies isolated and examined contained chrysotile cores, and 21 of 72 (29%) bodies contained cores of the amphiboles tremolitemore » and actinolite. By contrast, tremolite and actinolite constituted the majority of uncoated fibers in these cases. The mean length for bodies formed on chrysotile was 35 ..mu..m, and for bodies formed on tremolite or actinolite, 36 ..mu..m. The authors conclude that (1) the usual histologic criteria for the diagnosis of asbestos are applicable to chrysotile-exposed workers; (2) in workers with occupational chrysotile exposure, bodies form readily on this mineral; and (3) asbestos bodies in these lungs reflect the presence of long asbestos fibers.« less

A Case for the Demedicalization of Queer Bodies

PubMed Central

Eckhert, Erik

2016-01-01

The medicalization of queer bodies in the clinic and the lab is inexorably linked to the history of LBGTQ politics. Increasingly, activists and scholars are recognizing that while the natural origins of queer sexualities carry a certain political weight, invoking the naturalness of being “born this way” fails to articulate a more substantive challenge to the effects of unexamined cis- and heteronormativity on our social institutions. With this in mind, it is crucial to understand the way these biases operate in scientific research and healthcare so their impact on what we know and how we care can be addressed. It what follows, it will be shown that the medicalization of queer bodies not only fails to diminish these deep-seated biases from sexuality research and clinical practice, but that it also impedes care providers from addressing the healthcare disparities facing queer patients today. PMID:27354849

Retinal vasculitis and cystoid macular edema after body tattooing: a case report.

PubMed

Moschos, M M; Guex-Crosier, Y

2004-05-01

To report a case of retinal vasculitis occurring after the placement of permanent tattoos. A 21-year-old male was referred to our department with impairment of visual acuity. Permanent tattoos covered the head, body, arms and legs. The patient was examined with ophthalmoscopy, fluorescein angiography, indocyanine green angiography and optical coherence tomography. Systemic medical and laboratory work-up were performed in order to exclude an infectious agent or an inflammatory disease. He had no history of intravenous drug abuse. Our patient presented severe posterior uveitis associated with retinal vasculitis and cystoid macular edema. Laboratory tests ruled out all diseases causing vasculitis. HIV and B, C hepatitis tests were negative. Cystoid macular edema and vasculitis were resolved after immunosuppressive therapy. This is the first description of a retinal vasculitis associated with cystoid macular edema in a completely healthy individual after the placement of permanent tattoos. A phagocytosis of tattoo pigments leading to their lysis is described in the literature as a mechanism causing vasculitis.

Applied Sports Nutrition Support, Dietary Intake and Body Composition Changes of a Female Athlete Completing 26 Marathons in 26 Days: A Case Study.

PubMed

McManus, Chris J; Murray, Kelly A; Parry, David A

2017-03-01

The aim of this case study is to describe the nutrition practices of a female recreational runner (VO 2 max 48.9 ml · kg -1 · min -1 ) who completed 26 marathons (42.195 km) in 26 consecutive days. Information relating to the nutritional intake of female runners during multi-day endurance events is extremely limited, yet the number of people participating year-on-year continues to increase. This case study reports the nutrition intervention, dietary intake, body composition changes and performance in the lead-up and during the 26 days. Prior to undertaking the 26 marathon challenge, three consultations were held between the athlete and a sports nutrition advisor; planning and tailoring the general diet and race-specific strategies to the endurance challenge. During the marathons, the mean energy and fluid intake was 1039.7 ± 207.9 kcal (607.1 - 1453.2) and 2.39 ± 0.35 L (1.98 - 3.19). Mean hourly carbohydrate intake was 38.9 g·hr -1 . 11 days following the completion of the 26 marathons, body mass had reduced by 4.6 kg and lean body mass increasing by 0.53 kg when compared with 20 days prior. This case study highlights the importance of providing general and event-specific nutrition education when training for such an event. This is particularly prudent for multi-day endurance running events.

Mitochondrial Structure and Reactive Oxygen Species in Mammary Oncogenesis

DTIC Science & Technology

2007-04-01

for the subunits of this Complex responsible for the hereditary paraganglioma and pheochromocytoma (2- 4,12,13,30,31,32) suggest that Complex II...familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet. 2001, 69:49-54. 3. Baysal BE, Ferrell RE, Willett-Brozick JE, Lawrence EC...Eng C. Somatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in nonfamilial pheochromocytoma . Cancer Res. 2000, 60

Use of postmortem computed tomography to retrieve small metal fragments derived from a weapon in the bodies of victims in two homicide cases.

PubMed

Sano, Rie; Takahashi, Yoichiro; Hayakawa, Akira; Murayama, Masayuki; Kubo, Rieko; Hirasawa, Satoshi; Tokue, Hiroyuki; Shimada, Takehiro; Awata, Sachiko; Takei, Hiroyuki; Yuasa, Masahiro; Uetake, Shinji; Akuzawa, Hisashi; Kominato, Yoshihiko

2018-05-01

Postmortem computed tomography (PMCT) is becoming a commonly used modality in routine forensic investigation. Mechanical injuries including lacerations, incisions, stab wounds and gunshot wounds frequently contain foreign bodies that may have significant value as clues in criminal investigations. CT is a sensitive modality for detection of metal foreign bodies that may be associated with injuries to the victim in cases of homicide or traffic accidents. Here we report two cases in which PMCT was able to act as a guide to forensic pathologists for retrieval of metal fragments in the corpses of the victims, the retrieved fragments then being used to validate the confessions of the assailants through comparison with the knife and the crowbar, respectively, that had been used in the crimes. In these cases, the small metal fragments retrieved from the corpses of the victims with the aid of PMCT were decisive pieces of evidence confirming the circumstances of the crimes. These cases illustrate how PMCT can be used to complement the findings of classical autopsy for integrative investigation of corpses with injury. Copyright © 2018 Elsevier B.V. All rights reserved.

PubMed Central

POLAT, B.; UNSALER, S.; ULUSAN, M.; ASLAN, I.; HAFIZ, G.

2014-01-01

SUMMARY The aim of this study was to describe our experience with benign parapharyngeal space tumours resected via a transcervical route without mandibulotomy and to investigate associated postoperative sequelae and complications. The study investigated and analysed the retrospective charts of 44 patients who underwent surgery for benign parapharyngeal space tumours over a 10-year period. The diagnosis was reached in all patients with clinical and radiologic findings; preoperative fine-needle aspiration biopsy was not performed in any case. The preferred means of accessing the parapharyngeal space in all patients was a transcervical route. In 5 of these patients, transparotid extension was performed due to the position of the tumour. Tumours were classified radiologically as poststyloid in 27 cases and prestyloid in 17 cases. The final histopathologic diagnosis was vagal paraganglioma in 16 cases, pleomorphic adenoma in 13 cases, schwannoma in 10 cases and comparatively rarer tumours in the remaining 5 cases. In three patients, cranial nerve paralysis was observed during preoperative evaluation. Permanent cranial nerve paralysis occurred in 19 cases (43.2%) in the postoperative period, the majority of which were neurogenic tumours such as vagal paraganglioma (n = 16) and schwannoma (n = 2), and one case of non-neurogenic parapharyngeal tumour. The median duration of follow-up was 61 ± 33 months. There was no local recurrence in any patient during the follow-up period. A transcervical approach should be the first choice for excision of parapharyngeal space tumours, except for recurrent or malignant tumours, considering its advantages of providing direct access to the neoplasm, adequate control of neurovascular structures from the neck and optimal aesthetic outcomes due to preservation of mandibular continuity with minimal morbidity and hospitalisation time. PMID:25709146

Predictors of body satisfaction: differences between older men and women's perceptions of their body functioning and appearance.

PubMed

de Souto Barreto, Philipe; Ferrandez, Anne-Marie; Guihard-Costa, Anne-Marie

2011-04-01

This article examines the predictors of body satisfaction among older men and women. A self-reported questionnaire on body satisfaction (focused on body appearance and functioning), health, physical activity, and sociodemographic characteristics was completed by 384 older adults twice, at a year's interval. Women (n = 243) were on average 70.3 ± 7.9 years old, and men 70.9 ± 7.5 years old. Body mass index was found to be the strongest predictor of satisfaction with body appearance for both genders, along with body functioning in the case of men. Regarding satisfaction with body functioning, functional limitations were women's most important predictor of satisfaction, whereas for men body appearance was more important. Men seemed to link satisfaction with body appearance more closely to body functioning than did women. Gender differences suggest that interventions to improve body satisfaction must be gender specific, particularly those relating to body functioning.

Molecular identification of fungi found on decomposed human bodies in forensic autopsy cases.

PubMed

Schwarz, Patrick; Dannaoui, Eric; Gehl, Axel; Felske-Zech, Heike; Birngruber, Christoph G; Dettmeyer, Reinhard B; Verhoff, Marcel A

2015-07-01

To investigate which fungi can be found during forensic autopsies, a PubMed literature review was done in regard to fungal growth on decomposed human bodies. Unfortunately, the existing data is limited and not all fungi were identified to the species level. We, therefore, collected skin samples with macroscopically visible fungal growth from 23 autopsy cases in Germany and identified the fungi to the species level by molecular methods. The identified species included Aspergillus fumigatus and Candida albicans, which pose an allergenic risk, especially to persons with underlying lung diseases. Because safety standards are lacking, we recommend the use of respiratory protection during exhumations and forensic autopsies, when fungal growth is noted. With regard to the future, a database was set up which could possibly be used as a forensic tool to determine the time of death.

Body Image and Body Experience Disturbances in Schizophrenia: an Attempt to Introduce the Concept of Body Self as a Conceptual Framework.

PubMed

Sakson-Obada, Olga; Chudzikiewicz, Paulina; Pankowski, Daniel; Jarema, Marek

2018-01-01

Disturbances in body experience are described as key schizophrenia symptoms and early disease predictors. In case studies, different disorders relating to body experience are presented, but only a few empirical studies have aimed to distinguish the characteristics of body experience in schizophrenia, and these have been selected arbitrarily and without reference to cohesive theoretical model. To integrate this fragmentary approach, we propose a body self (BS) model, composed of: functions; representations (e.g., body image); and sense of body identity. The aim of the study was to determine whether the BS differentiates schizophrenic patients from healthy controls, and to investigate the relations between aspects of BS and a history of illness and clinical characteristics. The Body Self Questionnaire and the Positive and Negative Syndrome Scale were administered to 63 schizophrenic patients and 63 healthy subjects. The difference was found in the functions of the body-self (perceiving, interpreting, and regulating body experience), in the sense of body identity, and in one of three aspects of body image explored (e.g., acceptance of biological sex). Disturbances in BS were related to positive symptoms and to the number of hospitalizations for other diseases. Together, the results demonstrate that schizophrenia is more body experience than body image disorder, since the negative emotional attitude towards the body and acceptance of fitness were not distinctive for schizophrenia. The link between the disturbances in BS and the number of nonpsychiatric hospitalizations suggests that misinterpretation of body experiences in schizophrenia can promote a search for medical attention.

Intralenticular foreign body: a D-Day reminder.

PubMed

Dhawahir-Scala, Felipe E; Kamal, A

2005-12-01

Intralenticular foreign bodies are not uncommon after penetrating eye injuries. This is an observational case report where a patient was found to have an embedded lenticular foreign body for more than 60 years. Following such a long period of time the patient has not experienced any significant cataract formation or compromised ocular function due to the foreign body.

Ketone bodies as signaling metabolites

PubMed Central

Newman, John C.; Verdin, Eric

2014-01-01

Traditionally, the ketone body β-hydroxybutyrate (βOHB) has been looked upon as a carrier of energy from liver to peripheral tissues during fasting or exercise. However, βOHB also signals via extracellular receptors and acts as an endogenous inhibitor of histone deacetylases (HDACs). These recent findings support a model in which βOHB functions to link the environment, in this case the diet, and gene expression via chromatin modifications. Here, we review the regulation and functions of ketone bodies, the relationship between ketone bodies and calorie restriction, and the implications of HDAC inhibition by the ketone body βOHB in the modulation of metabolism, and diseases of aging. PMID:24140022

Power optimization in body sensor networks: the case of an autonomous wireless EMG sensor powered by PV-cells.

PubMed

Penders, J; Pop, V; Caballero, L; van de Molengraft, J; van Schaijk, R; Vullers, R; Van Hoof, C

2010-01-01

Recent advances in ultra-low-power circuits and energy harvesters are making self-powered body sensor nodes a reality. Power optimization at the system and application level is crucial in achieving ultra-low-power consumption for the entire system. This paper reviews system-level power optimization techniques, and illustrates their impact on the case of autonomous wireless EMG monitoring. The resulting prototype, an Autonomous wireless EMG sensor power by PV-cells, is presented.

Swallowed foreign bodies in adults.

PubMed

Ambe, Peter; Weber, Sebastian A; Schauer, Mathias; Knoefel, Wolfram T

2012-12-01

Foreign-body ingestion is a common event most often seen in children from 6 months to 6 years of age. In adults, foreign bodies are usually ingested accidentally together with food. This happens more commonly in persons with certain pathological changes of the gastrointestinal tract. We present a selective review of pertinent literature retrieved by a search in the PubMed database. The foreign bodies most commonly ingested by adults are fish bones and chicken bones. The clinical approach to the problem depends on the type of material ingested and on the patient's symptoms and physical findings. In about 80% of cases, the ingested material passes uneventfully through the gastrointestinal tract; endoscopy is performed in about 20% of cases, and surgery in less than 1%. Emergency esophagogastroduodenoscopy (EGD) is recommended when the esophagus is completely occluded (because of the risk of aspiration and/or pressure necrosis), when the ingested object has a sharp point or edge (because of the risk of perforation, with ensuing mediastinitis or peritonitis), and when a battery has been ingested (because of the risk of necrosis and fistula formation). For non-occluding esophageal foreign bodies, including magnets, an urgent but non-emergency EGD within 12 to 24 hours is recommended. Most patients can be treated conservatively by observation alone, but there should be a low threshold for deciding to proceed to endoscopic retrieval. Surgery is reserved for complicated cases.

Foreign bodies in the aerodigestive tract in pediatric patients.

PubMed

Higo, Ryuzaburo; Matsumoto, Yu; Ichimura, Keiichi; Kaga, Kimitaka

2003-12-01

To investigate pediatric foreign body cases in the aerodigestive tract, and to elucidate the characteristic problems in Japan. A total of 310 pediatric patients (age 15 or below), gathered from two medical university hospitals (University of Tokyo and Jichi Medical School), were included in this study. Data were collected by retrospective chart review and were statistically analyzed. Two-year-olds were the most common patients, and the range from age 1 to age 4 included 67.7% of all the patients. The most involved sites were the nose (39.4%) and the pharynx (38.4%), followed by the esophagus (12.9%) and the trachea-bronchi (6.5%). Fish bones and toys were the representative foreign bodies (30.7 and 13.6%, respectively). Other foreign bodies often encountered included coins, food, candy, peanuts and nuts, and batteries. The type of foreign body was closely related to the site in which foreign bodies were lodged: 77.3% of foreign bodies in the pharynx were fish bones, and toys were the most common impacted foreign body in the nose. In the esophagus, representative impacted foreign bodies were coins (35.7%), but disk-type battery ingestion has been increasing in recent years. Although most foreign bodies in the esophagus were safely removed, one case of a disk-type battery had a serious sequela. In the trachea-bronchi, peanuts, food, cotton, and coins were impacted. A rigid bronchoscope was basically used to remove foreign bodies, but in some cases, a fine flexible endoscope, with a channel for fiber forceps, was useful, because it could be inserted into narrow bronchi. Advance of a flexible endoscope will make it an excellent tool for diagnosis and management of the trachea-bronchial foreign body. Fish bones in the pharynx, which were closely related to Japanese eating habits, and toys in the nose, were the typical foreign bodies encountered in this study. In the esophagus, an increased incidence of disk-type battery ingestion has become a serious problem in recent

Nurse migration in an increasingly interconnected world: the case for internationalization of regulation of nurses and nursing regulatory bodies.

PubMed

Cutcliffe, John R; Bajkay, Renay; Forster, Stu; Small, Rudy; Travale, Rodger

2011-10-01

Psychiatric/Mental Health nursing has a long history of professional self-regulation; nevertheless, interest in how governments protect consumers of health care from poor or dangerous practice(s) is on the increase. Correspondingly, there have been calls, in several parts of the world, for greater watchfulness and due diligence from regulatory bodies. Mindful of the concept of "globalization" and the unequivocal data regarding the significant increase in the migration of nurses, it is difficult to ignore/deny the reality of an increasingly mobile and connected international nursing workforce. However, the extant literature also indicates the existence of significant disparities between countries and even states/provinces within countries as to the enforcement of professional regulation. What this means is that decisions made by one regulatory body can have a direct impact on the standard(s) of nursing quality and practice in a country on the opposite side of the world. As a result, the authors attempt to advance the debate that there is a clear need to reconcile these positions, and they introduce the argument for the creation of an international oversight body. Using case study material, the relevant theoretical and policy literature in this area (such as it is), and by drawing on examples of analogous oversight bodies from other areas, we draw attention to the need to create a genuinely international body for the oversight of nurse regulation. Copyright © 2011 Elsevier Inc. All rights reserved.

Epidemiological study of a von Hippel-Lindau family in northwest China.

PubMed

Zhang, Jingyao; Wu, Dapeng; Ai, Hong; Bai, Jigang; Dong, Shunbin; Yang, Qinling; Qu, Kai; Zhou, Lei; Xu, Xinsen; Liu, Chang

2013-09-01

von Hippel-Lindau (VHL) disease is a rare, inherited neoplastic disease characterized by hemangioblastomas (HBL) of the central nervous system (CNS), retinal angiomas, renal cell carcinomas (RCC), pancreatic endocrine tumors (PETs), pheochromocytomas, paragangliomas, and visceral cysts. We encountered a large VHL family in northwest China and conducted a systematic screening of the family members based on their epidemiological and clinical characteristics. A self-designed questionnaire was used to collect the general sociodemographic and health information of the family members. For the preliminary family screening, physical examination and abdomen B ultrasonography were performed. The suspected patients were subjected to cranial computerized tomography and fundus examination. The clinical data of the patients with confirmed VHL disease were collected from hospital records. A total of 63 lineal descendants in six generations were observed in the family (generations O, A, B, C, D, E), including 9 dead suspected cases (6 males, 3 females) and 10 living cases (2 males, 8 females). Among the 10 living cases, 4, 2, 1, 3, 4, 8, and 2 manifested HBLs of the CNS, PETs, RCC, pancreatic cysts, renal cysts, pheochromocytomas (4 hemi and 4 bilateral), and paragangliomas, respectively. Data showed that the morbidity of VHL disease in generation C was lower than that in generation B, but the age of onset was younger. This study is the first to report VHL disease in northwest China and VHL-associated PET cases in Chinese. Therefore, follow-up checkups of the family should be focused on younger generations. Proper family screening protocols should be followed for the treatment of patients with VHL disease.

Intralaminar nuclei of the thalamus in Lewy body diseases.

PubMed

Brooks, Daniel; Halliday, Glenda M

2009-02-16

Although the intralaminar thalamus is a target of alpha-synuclein pathology in Parkinson's disease, the degree of neuronal loss in Lewy body diseases has not been assessed. We have used unbiased stereological techniques to quantify neuronal loss in intralaminar thalamic nuclei concentrating alpha-synuclein pathology (the anterodorsal, cucullar, parataenial, paraventricular, central medial, central lateral and centre-median/parafascicular complex) in different clinical forms of Lewy body disease (Parkinson's disease with and without dementia, and dementia with Lewy bodies, N=21) compared with controls (N=5). Associations were performed in the Lewy body cases between intralaminar cell loss and the main diagnostic clinical (parkinsonism, dementia, fluctuation in consciousness, and visual hallucinations) and pathological (Braak stage of Parkinson's disease) features of these diseases, as well as between cell loss and the scaled severity of the alpha-synuclein deposition within the intralaminar thalamus. As expected, significant alpha-synuclein accumulation occurred in the intralaminar thalamus in the cases with Lewy body disease. Pathology concentrated anteriorly and in the central lateral and paraventricular nuclei was related to the Braak stage of Parkinson's disease, ageing, and the presence of dementia. Across all types of Lewy body cases there was substantial atrophy and neuronal loss in the central lateral, cucullar and parataenial nuclei, and neuronal loss without atrophy in the centre-median/parafascicular complex. Cases with visual hallucinations showed a greater degree of atrophy of the cucullar nucleus, possibly due to amygdala denervation. The significant degeneration demonstrated in the intralaminar thalamus is likely to contribute to the movement and cognitive dysfunction observed in Lewy body disorders.

Ustur whole body case 0269: demonstrating effectiveness of i.v. CA-DTPA for Pu.

PubMed

James, A C; Sasser, L B; Stuit, D B; Glover, S E; Carbaugh, E H

2007-01-01

This whole body donation case (USTUR Registrant) involved a single acute inhalation of an acidic Pu(NO3)4 solution in the form of an aerosol 'mist'. Chelation treatment with intravenously (i.v.) Ca-EDTA was initiated on the day of the intake, and continued intermittently over 6 months. After 2.5 y with no further treatment, a course of i.v. Ca-DTPA was administered. A total of 400 measurements of 239+240Pu excreted in urine were recorded; starting on the first day (both before and during the initial Ca-EDTA chelation) and continuing for 37 y. This sampling included all intervals of chelation. In addition, 91 measurements of 239+240Pu-in-feces were recorded over this whole period. The Registrant died about 38 y after the intake, at age 79 y, with extensive carcinomatosis secondary to adenocarcinoma of the prostate gland. At autopsy, all major soft tissue organs were harvested for radiochemical analyses of their 238Pu, 239+240Pu and 241Am content. Also, all types of bone (comprising about half the skeleton) were harvested for radiochemical analyses, as well as samples of skin, subcutaneous fat and muscle. This comprehensive data set has been applied to derive 'chelation-enhanced' transfer rates in the ICRP Publication 67 plutonium biokinetic model, representing the behaviour of blood-borne and tissue-incorporated plutonium during intervals of therapy. The resulting model of the separate effects of i.v. Ca-EDTA and Ca-DTPA chelation shows that the therapy administered in this case succeeded in reducing substantially the long-term burden of plutonium in all body organs, except for the lungs. The calculated reductions in organ content at the time of death are approximately 40% for the liver, 60% for other soft tissues (muscle, skin, glands, etc.), 50% for the kidneys and 50% for the skeleton. Essentially, all of the substantial reduction in skeletal burden occurred in trabecular bone. This modelling exercise demonstrated that 3-y-delayed Ca-DTPA therapy was as effective

USTUR WHOLE BODY CASE 0269: DEMONSTRATING EFFECTIVENESS OF I.V. CA-DTPA FOR PU

DOE Office of Scientific and Technical Information (OSTI.GOV)

James, Anthony C.; Sasser , Lyle B.; Stuit, Dorothy B.

2008-01-28

This whole body donation case (USTUR Registrant) involved a single acute inhalation of an acidic Pu(NO3)4 solution in the form of an aerosol ‘mist.’ Chelation treatment with i.v. Ca-EDTA was initiated on the day of the intake, and continued intermittently over 6 months. After 2½ years with no further treatment, a course of i.v. Ca-DTPA was administered. A total of 400 measurements of 239+240Pu excreted in urine were recorded; starting on the first day (both before and during the initial Ca-EDTA chelation), and continuing for 37 years. This sampling included all intervals of chelation. In addition, 91 measurements of 239+240Pu-in-fecesmore » were recorded over this whole period. The Registrant died about 38 years after the intake, at age 79 y, with extensive carcinomatosis secondary to adenocarcinoma of the prostate gland. At autopsy, all major soft tissue organs were harvested for radiochemical analyses of their 238Pu, 239+240Pu and 241Am content. Also, all types of bone (comprising about half the skeleton) were harvested for radiochemical analyses, as well as samples of skin, subcutaneous fat and muscle. This comprehensive dataset has been applied to derive ‘chelation-enhanced’ transfer rates in the ICRP Publication 67 plutonium biokinetic model, representing the behaviour of blood-borne and tissue-incorporated plutonium during intervals of therapy. The resulting model of the separate effects of i.v. Ca-EDTA and Ca-DTPA chelation shows that the therapy administered in this case succeeded in reducing substantially the long-term burden of plutonium in all body organs, except for the lungs. The calculated reductions in organ content at the time of death are approximately 40% for the liver, 60% for other soft tissues (muscle, skin, glands, etc.), 50% for the kidneys, and 50% for the skeleton. Essentially all of the substantial reduction in skeletal burden occurred in trabecular bone. This modeling exercise demonstrated that 3-y-delayed Ca-DTPA therapy was

Alveolar soft part sarcoma presenting as a breast metastasis in a patient with a history of thyroid cancer: a case report

PubMed Central

Bychkov, Andrey; Sampatanukul, Pichet

2015-01-01

Metastases to the breast are uncommon, accounting for 0.5% of breast tumors, and most of them are originated from lymphoma, melanoma and carcinomas of various organs. Alveolar soft part sarcoma (ASPS) is a very rare neoplasm that is usually found in the lower extremities. Lungs are the common site of dissemination and may represent initial manifestation of disease. We report a clinically unsuspected case of ASPS presenting as a breast metastasis in a 25-year-old woman. The patient’s medical history was notable for a thyroid cancer treated by surgery and radioiodine ablation 2 years ago. Core needle biopsy of slowly growing breast mass yielded polygonal cells with abundant eosinophilic cytoplasm arranged into solid pattern. Differential diagnosis between apocrine cell carcinoma, paraganglioma, granular cell tumor, neuroendocrine carcinoma, ASPS and metastatic hepatocellular and renal cell carcinoma was rendered by immunohistochemistry. Strong nuclear TFE3 immunoreactivity confirmed a diagnosis of ASPS. Retrospectively a primary tumor was found in the thigh. Most likely, ASPS and thyroid cancer in the patient were growing synchronously and independently. PMID:26464747

Pediatric foreign bodies and their management.

PubMed

Kay, Marsha; Wyllie, Robert

2005-06-01

Ingestion of foreign bodies is a common pediatric problem, with more than 100,000 cases occurring each year. The vast majority of pediatric ingestions are accidental; increasing incidence of intentional ingestions starts in the adolescent age group. In the United States, the most common pediatric foreign bodies ingested are coins, followed by a variety of other objects, including toys, toy parts, sharp objects, batteries, bones, and food. In adolescents and adults, meat or food impactions are the most common accidental foreign body ingestion. Esophageal pathology underlies most cases of food impaction. Management of foreign body ingestions varies based on the object ingested, its location, and the patient's age and size. Esophageal foreign bodies as a group require early intervention because of their potential to cause respiratory symptoms and complications, esophageal erosions, or even an aortoesophageal fistula. Ingested batteries that lodge in the esophagus require urgent endoscopic removal even in the asymptomatic patient due to the high risk of complications. Sharp foreign bodies increase the foreign body complication rate from less than 1% to 15% to 35%, except for straight pins, which usually follow a relatively benign course unless multiple pins are ingested. Magnets are increasingly ingested, due to their ubiquitous nature and the perception that they do not pose a risk. Ingestion of multiple magnets creates a significant risk of obstruction, perforation, and fistula development. Methods to deal with foreign bodies include the suture technique, the double snare technique, and the combined forceps/snare technique for long, large, and sharp foreign bodies, along with newer equipment, such as retrieval nets and a variety of specialized forceps.

Review of ear, nose and throat foreign bodies in Sarawak General Hospital. A five year experience.

PubMed

Chiun, Kian Chai; Tang, Ing Ping; Tan, Tee Yong; Jong, Doris Evelyn Yah Hui

2012-02-01

Ear, nose and throat foreign bodies are common in ENT clinical practice. This study was designed to establish the local data of otorhinolaryngeal foreign bodies in term of prevalence among paediatric and adult groups, the clinical features, types of foreign body at different sites, and laterality of foreign bodies. This study was carried out at ENT department, Sarawak General Hospital, Malaysia, from 1st January 2005 to 31st December 2009. A total of 1084 cases were included and statistically analyzed. Ear foreign bodies showed the highest incidence which was consisted of 480 (44.3%) cases, followed by nose in 270 (24.9%) cases, pharynx in 251 (23.2%) cases, esophagus in 57 (5.3%) cases and laryngo-tracheobronchial tree in 26 (2.4%) cases. Otorhinolaryngeal foreign bodies occurred more frequently in 0-10 year old age group which constituted 651 (60.1%) cases. The descending order of frequency for foreign body sites in adult was pharynx (17.2%), ear (12.8%), esophagus (3.1%), nose (1.7%) and laryngo-tracheobronchial tree (1.1%). The type of foreign bodies varies with age group and site of foreign body lodgement. In general, common foreign bodies in both adult and children were food related, with the additional of small objects such as plastic toy in paediatric group. Otorhinolaryngeal foreign bodies were found more frequently in children. The types of foreign body were different from age group and sites of foreign body lodgement. The local food constituted the highest incidence of ear, nose, and throat foreign bodies with additional of plastic toys in paediatric group.

Rheological investigation of body cream and body lotion in actual application conditions

NASA Astrophysics Data System (ADS)

Kwak, Min-Sun; Ahn, Hye-Jin; Song, Ki-Won

2015-08-01

The objective of the present study is to systematically evaluate and compare the rheological behaviors of body cream and body lotion in actual usage situations. Using a strain-controlled rheometer, the steady shear flow properties of commercially available body cream and body lotion were measured over a wide range of shear rates, and the linear viscoelastic properties of these two materials in small amplitude oscillatory shear flow fields were measured over a broad range of angular frequencies. The temperature dependency of the linear viscoelastic behaviors was additionally investigated over a temperature range most relevant to usual human life. The main findings obtained from this study are summarized as follows: (1) Body cream and body lotion exhibit a finite magnitude of yield stress. This feature is directly related to the primary (initial) skin feel that consumers usually experience during actual usage. (2) Body cream and body lotion exhibit a pronounced shear-thinning behavior. This feature is closely connected with the spreadability when cosmetics are applied onto the human skin. (3) The linear viscoelastic behaviors of body cream and body lotion are dominated by an elastic nature. These solid-like properties become a criterion to assess the selfstorage stability of cosmetic products. (4) A modified form of the Cox-Merz rule provides a good ability to predict the relationship between steady shear flow and dynamic viscoelastic properties for body cream and body lotion. (5) The storage modulus and loss modulus of body cream show a qualitatively similar tendency to gradually decrease with an increase in temperature. In the case of body lotion, with an increase in temperature, the storage modulus is progressively decreased while the loss modulus is slightly increased and then decreased. This information gives us a criterion to judge how the characteristics of cosmetic products are changed by the usual human environments.

Pharmacological Treatment Of Body Dysmorphic Disorder.

PubMed

Hong, Kevin; Nezgovorova, Vera; Uzunova, Genoveva; Schlussel, Danya; Hollander, Eric

2018-04-26

Body dysmorphic disorder is a challenging disorder that manifests as erroneously perceived flaws in one's physical appearance and repetitive behaviors in response to appearance concerns. This disorder is also frequently comorbid with other psychiatric disorders, including major depressive disorder and autism spectrum disorder. It is currently understood to arise from a combination of biological, psychological, and environmental factors. Treatment of body dysmorphic disorder typically consists of a combination of pharmacotherapy and cognitive behavioral therapy. However, not all patients respond to treatment, and BDD symptoms remain even in those who do respond. This review outlines current pharmacological and neuromodulation treatments for body dysmorphic disorder, and suggests directions for future studies of novel treatments such as augmentation with atypical antipsychotics and the use of intranasal oxytocin in cases of body dysmorphic disorder that show residual symptomatology even with tailored monotherapy. There is emerging evidence suggesting that non-invasive neurostimulatory techniques, such as repetitive transcranial magnetic stimulation, may be of value in treatment-resistant cases. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Laminar Flow About a Rotating Body of Revolution in an Axial Airstream

NASA Technical Reports Server (NTRS)

Schlichting, H.

1956-01-01

We have set ourselves the problem of calculating the laminar flow on a body of revolution in an axial flow which simultaneously rotates about its axis. The problem mentioned above, the flow about a rotating disk in a flow, which we solved some time ago, represents the first step in the calculation of the flow on the rotating body of revolution in a flow insofar as, in the case of a round nose, a small region about the front stagnation point of the body of revolution may be replaced by its tangential plane. In our problem regarding the rotating body of revolution in a flow, for laminar flow, one of the limiting cases is known: that of the body which is in an axial approach flow but does not rotate. The other limiting case, namely the flow in the neighborhood of a body which rotates but is not subjected to a flow is known only for the rotating circular cylinder, aside from the rotating disk. In the case of the cylinder one deals with a distribution of the circumferential velocity according to the law v = omega R(exp 2)/r where R signifies the cylinder radius, r the distance from the center, and omega the angular velocity of the rotation. The velocity distribution as it is produced here by the friction effect is therefore the same as in the neighborhood of a potential vortex. When we treat, in what follows, the general case of the rotating body of revolution in a flow according to the calculation methods of Prandtl's boundary-layer theory, we must keep in mind that this solution cannot contain the limiting case of the body of revolution which only rotates but is not subjected to a flow. However, this is no essential limitation since this case is not of particular importance for practical purposes.

Full body action remapping of peripersonal space: the case of walking.

PubMed

Noel, Jean-Paul; Grivaz, Petr; Marmaroli, Patrick; Lissek, Herve; Blanke, Olaf; Serino, Andrea

2015-04-01

The space immediately surrounding the body, i.e. peripersonal space (PPS), is represented by populations of multisensory neurons, from a network of premotor and parietal areas, which integrate tactile stimuli from the body's surface with visual or auditory stimuli presented within a limited distance from the body. Here we show that PPS boundaries extend while walking. We used an audio-tactile interaction task to identify the location in space where looming sounds affect reaction time to tactile stimuli on the chest, taken as a proxy of the PPS boundary. The task was administered while participants either stood still or walked on a treadmill. In addition, in two separate experiments, subjects either received or not additional visual inputs, i.e. optic flow, implying a translation congruent with the direction of their walking. Results revealed that when participants were standing still, sounds boosted tactile processing when located within 65-100 cm from the participants' body, but not at farther distances. Instead, when participants were walking PPS expands as reflected in boosted tactile processing at ~1.66 m. This was found despite the fact the spatial relationship between the participant's body and the sound's source did not vary between the Standing and the Walking condition. This expansion effect on PPS boundaries due to walking was the same with or without optic flow, suggesting that kinematics and proprioceptive cues, rather than visual cues, are critical in triggering the effect. These results are the first to demonstrate an adaptation of the chest's PPS representation due to whole body motion and are compatible with the view that PPS constitutes a dynamic sensory-motor interface between the individual and the environment. Copyright © 2014 Elsevier Ltd. All rights reserved.

The periodic dynamics of the irregular heterogeneous celestial bodies

NASA Astrophysics Data System (ADS)

Lan, Lei; Yang, Mo; Baoyin, Hexi; Li, Junfeng

2017-02-01

In this paper, we develop a methodology to study the periodic dynamics of irregular heterogeneous celestial bodies. Heterogeneous bodies are not scarce in space. It has been found that bodies, such as 4 Vesta, 624 Hektor, 87 Sylvia, 16 Psyche and 25143 Itokawa, may all have varied internal structures. They can be divided into large-scale and small-scale cases. The varied internal structures of large-scale bodies always result from gradient pressure inside, which leads to compactness differences of the inner material. However, the heterogeneity of a small-scale body is always reflected by the different densities of different areas, which may originate from collision formation from multiple objects. We propose a modeling procedure for the heterogeneous bodies derived from the conventional polyhedral method and then compare its dynamical characteristics with those of the homogeneous case. It is found that zero-velocity curves, positions of equilibrium points, types of bifurcations in the continuation of the orbital family and the stabilities of periodic orbits near the heterogeneous body are different from those in the homogeneous case. The suborbicular orbits near the equatorial plane are potential parking orbits for a future mission, so we discuss the switching of the orbital stability of the family because it has fundamental significance to orbit maintenance and operations around actual asteroids.

Failure of MIBG scan to detect metastases in SDHB-mutated pediatric metastatic pheochromocytoma.

PubMed

Sait, Sameer; Pandit-Taskar, Neeta; Modak, Shakeel

2017-11-01

123 I-meta-iodo benzyl guanidine (MIBG) scans are considered the gold standard imaging in neuroblastoma; however, flouro deoxy glucose positron emission tomography (FDG-PET) scans have increased sensitivity in adults with pheochromocytoma/paraganglioma. We describe a pediatric patient initially considered to have localized neuroblastoma based on anatomical imaging and 123 I-MIBG scan, but subsequent investigations revealed germline succinate dehydrogenase complex iron sulfur subunit B (SDHB) mutation-associated pheochromocytoma with multiple FDG-avid skeletal metastases. We then compared 123 I-MIBG and FDG-PET scans in children with metastatic pheochromocytoma/paraganglioma. FDG-PET was superior to 123 I-MIBG scan for the detection of skeletal metastases (median number of skeletal lesions detected 10 [range 1-30] vs. 2 [range 1-26], respectively; P = 0.005 by t-test). FDG-PET should be considered the functional scan of choice in children with pheochromocytoma/paraganglioma. © 2017 Wiley Periodicals, Inc.

Magnetic spheres as foreign body into the bladder.

PubMed

Graziottin, Tulio M; de Freitas G Soares, Daniel; Da Ros, Carlos T; Sogari, Paulo R; Telöken, Cláudio; Laste, Paulo Roberto

2013-10-01

A great variety of foreign bodies in the lower urinary tract have been described; many of them are self-inflicted by the patient with masturbatory purposes. Depending on the nature of the foreign body the diagnostic and management might be challenging. We report a case of an unusual magnetic self-inserted foreign body into the bladder for autoerotism and briefly discuss the diagnostic and therapeutic implications in this challenging situation. We describe all the steps we have used to adequately diagnose the problem, describe the foreign body and treatments for the patient. Related articles were found by utilizing the PubMed database and are summarized in this study.   The management approach must be planned according to the nature of the foreign body and should minimize bladder and urethral trauma. However, most of cases can be managed endoscopically. Removal of magnetic foreign body may be quite challenging, requiring high-level surgical skills and minimally invasive techniques resulting in fast recovery and low complication rate. © 2012 International Society for Sexual Medicine.

Biophoton emission of human body.

PubMed

Cohen, S; Popp, F A

2003-05-01

For the first time systematic measurements of the "ultraweak" photon emission of the human body (biophotons) have been performed by means of a photon detector device set up in darkness. About 200 persons have been investigated. In a particular case one person has been examined daily over several months. It turned out that this biophoton emission reflects, (i) the left-right symmetry of the human body; (ii) biological rhythms such as 14 days, 1 month, 3 months and 9 months; (iii) disease in terms of broken symmetry between left and right side; and (iv) light channels in the body, which regulate energy and information transfer between different parts. The results show that besides a deeper understanding of health, disease and body field, this method provides a new powerful tool of non-invasive medical diagnosis in terms of basic regulatory functions of the body.

Detecting Foreign Bodies in a Head Laceration

PubMed Central

Fowler, Thomas R.; Crellin, Steven J.; Greenberg, Marna Rayl

2015-01-01

Open wounds represent a potential area of medicolegal risk if foreign bodies are not identified prior to wound closure. The importance of imaging of lacerations was underscored by a recent case where a 20-year-old male collided with a friend's mouth on a trampoline sustaining a simple, superficial scalp laceration. The wound was evaluated in typical fashion including irrigation and local exploration and was prepared for closure. The friend was then evaluated and noted to have multiple extensive dental fractures. An increased index of suspicion generated further evaluation of the first patient's wound. Plain radiography obtained of the first patient's skull was noted to have bony foreign bodies consistent with teeth, which were then removed after further exploration. Superficial wounds are common and complications arising from retained foreign bodies are a potential source of substantial morbidity and consequently medical litigation. This case serves as a reminder to be vigilant and maintain a high index of suspicion regarding the potential for foreign body. PMID:25802770

Detecting foreign bodies in a head laceration.

PubMed

Fowler, Thomas R; Crellin, Steven J; Greenberg, Marna Rayl

2015-01-01

Open wounds represent a potential area of medicolegal risk if foreign bodies are not identified prior to wound closure. The importance of imaging of lacerations was underscored by a recent case where a 20-year-old male collided with a friend's mouth on a trampoline sustaining a simple, superficial scalp laceration. The wound was evaluated in typical fashion including irrigation and local exploration and was prepared for closure. The friend was then evaluated and noted to have multiple extensive dental fractures. An increased index of suspicion generated further evaluation of the first patient's wound. Plain radiography obtained of the first patient's skull was noted to have bony foreign bodies consistent with teeth, which were then removed after further exploration. Superficial wounds are common and complications arising from retained foreign bodies are a potential source of substantial morbidity and consequently medical litigation. This case serves as a reminder to be vigilant and maintain a high index of suspicion regarding the potential for foreign body.

Localization in a random XY model with long-range interactions: Intermediate case between single-particle and many-body problems

NASA Astrophysics Data System (ADS)

Burin, Alexander L.

2015-09-01

Many-body localization in an XY model with a long-range interaction is investigated. We show that in the regime of a high strength of disordering compared to the interaction an off-resonant flip-flop spin-spin interaction (hopping) generates the effective Ising interactions of spins in the third order of perturbation theory in a hopping. The combination of hopping and induced Ising interactions for the power-law distance dependent hopping V (R ) ∝R-α always leads to the localization breakdown in a thermodynamic limit of an infinite system at α <3 d /2 where d is a system dimension. The delocalization takes place due to the induced Ising interactions U (R ) ∝R-2 α of "extended" resonant pairs. This prediction is consistent with the numerical finite size scaling in one-dimensional systems. Many-body localization in an XY model is more stable with respect to the long-range interaction compared to a many-body problem with similar Ising and Heisenberg interactions requiring α ≥2 d which makes the practical implementations of this model more attractive for quantum information applications. The full summary of dimension constraints and localization threshold size dependencies for many-body localization in the case of combined Ising and hopping interactions is obtained using this and previous work and it is the subject for the future experimental verification using cold atomic systems.

Oesophageal foreign body and a double aortic arch: rare dual pathology.

PubMed

O'Connor, T E; Cooney, T

2009-12-01

We report the rare case of an oesophageal foreign body which lodged above the site of oesophageal compression by a double aortic arch. Case report and a review of the literature surrounding the classification, embryology, diagnosis and management of vascular rings and slings. An eight-month-old male infant presented with symptoms of tracheal compression following ingestion of an oesophageal foreign body. Following removal of the oesophageal foreign body, the infant's symptoms improved initially. However, subsequent recurrence of respiratory symptoms lead to a repeat bronchoscopy and the diagnosis of a coexisting double aortic arch, causing tracheal and oesophageal compression. To our knowledge, this is only the second reported case of a double aortic arch being diagnosed in a patient following removal of an oesophageal foreign body.

Chaotic Dynamics in the Planar Gravitational Many-Body Problem with Rigid Body Rotations

NASA Astrophysics Data System (ADS)

Kwiecinski, James A.; Kovacs, Attila; Krause, Andrew L.; Planella, Ferran Brosa; van Gorder, Robert A.

The discovery of Pluto’s small moons in the last decade has brought attention to the dynamics of the dwarf planet’s satellites. With such systems in mind, we study a planar N-body system in which all the bodies are point masses, except for a single rigid body. We then present a reduced model consisting of a planar N-body problem with the rigid body treated as a 1D continuum (i.e. the body is treated as a rod with an arbitrary mass distribution). Such a model provides a good approximation to highly asymmetric geometries, such as the recently observed interstellar asteroid ‘Oumuamua, but is also amenable to analysis. We analytically demonstrate the existence of homoclinic chaos in the case where one of the orbits is nearly circular by way of the Melnikov method, and give numerical evidence for chaos when the orbits are more complicated. We show that the extent of chaos in parameter space is strongly tied to the deviations from a purely circular orbit. These results suggest that chaos is ubiquitous in many-body problems when one or more of the rigid bodies exhibits nonspherical and highly asymmetric geometries. The excitation of chaotic rotations does not appear to require tidal dissipation, obliquity variation, or orbital resonance. Such dynamics give a possible explanation for routes to chaotic dynamics observed in N-body systems such as the Pluto system where some of the bodies are highly nonspherical.

Is Radiologic Evaluation Necessary to Find out Foreign Bodies in Nasal Cavity?

PubMed

Oh, Hoon; Min, Hyun Jin; Yang, Hoon Shik; Kim, Kyung Soo

2016-01-01

Although there were previous studies on the clinical aspects such as etiology, treatment modalities, studies regarding the necessity of radiologic evaluation for nasal foreign body were limited. The aim of this study is to evaluate the necessity and indication of radiologic evaluation for nasal foreign bodies. There are consecutive patients aged less than 10 years who presented with suspected foreign bodies in nasal cavity. We reviewed the patient's age and sex, including the methods of evaluation, management tools, and types of foreign bodies. There were 35 cases (11.4%) on whom radiographs were performed in the 24 uncooperative patients and 11 cooperative patients who were not identified with any foreign bodies via nasal endoscopy. Among them, only 4 cases had positive reports of foreign body and the others were normal radiologic findings. We suggest that the radiologic evaluation is always not necessary to find the location of nasal foreign bodies. It, however, should be performed in cases of negative findings of physical examination with anterior rhinoscopy or sinus endoscopy and unwitnessed foreign bodies to rule out metallic contents, especially button type battery.

Complications in children from foreign bodies in the airway.

PubMed

Rodríguez, Hugo; Cuestas, Giselle; Botto, Hugo; Nieto, Mary; Cocciaglia, Alejandro; Passali, Desiderio; Gregori, Dario

2016-01-01

Foreign body aspiration in childhood is a common and potentially serious problem. Complications may be the result of the aspiration episode itself, delayed diagnosis or treatment. We describe our experience in a paediatric hospital in Argentina. We retrospectively evaluated 56 patients with complications due to foreign body aspiration recorded in the Susy Safe Project between January 2010 and November 2013. The clinical variables analysed were sex, age at time of aspiration, foreign body location and type, time elapsed from the event until object removal, extraction technique, complications, need for hospitalisation and circumstances of the event. 58.9% of the cases described occurred in males, with high presence of adults (76.8%) at the time of aspiration. The incidence was slightly higher in children older than 3 years. In 37 cases (66.1%), the foreign body was located in bronchus; sunflower seeds and ballpoint caps were the most common foreign objects. Only in 10 cases (17.9%) was the object extracted within 24h of the event. The most common complications were pneumonia (18 cases), granuloma (15 cases) and mucosal erosion (9 cases). Hospitalisation was necessary for 41 patients. Early diagnosis and immediate control through specialised teams are essential to ensure proper treatment, usually endoscopic, without risk of complications. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

A Report of a Case Involving Body Lateropulsion with Numbness of the Ipsilesional Fingers Caused by a Small Infarction in the Dorsal Part of the Middle Medulla.

PubMed

Yamaoka, Yumiko; Kishishita, Sadahiro; Takayama, Yohei; Okubo, Seiji

2018-01-01

Based on the complexity of functional anatomy, a small infarction in the medulla can produce various types of clinical symptoms or signs depending on the location of this infarction. We describe the case of a 46-year-old man who presented with sudden onset of body lateropulsion to the left side and numbness of the ipsilateral fingers. 3-tesla diffusion-weighted magnetic resonance imaging with a section thickness of 2 mm revealed a small infarction in the dorsal part of the left middle medulla. To our knowledge, this is the first case report describing vestibular dysfunction apparent upon otoelectrophysiological examination but without vestibular symptoms or signs except for body lateropulsion.

Connection between three-body configuration and four-body configuration of the Sitnikov problem when one of the masses approaches zero: circular case

NASA Astrophysics Data System (ADS)

Shahbaz Ullah, M.; Hassan, M. R.

2014-09-01

In this manuscript we have established averaged equation of motion of the Sitnikov restricted three- body and four-body problem when all the primaries are point masses, by applying the Van der Pol transformation and averaging technique of J. Guckenheimer and P. Holmes (in Nonlinear Oscillations, Dynamical System Bifurcations of Vector Fields, Springer, Berlin, 1983). In addition to the resonance criterion at the 3/2 commensurability we have chosen ω=2 n/3, n=4, ω is the angular velocity of the coordinate system. Further we established the Series solution of the three-body and four-body problem in the sense of Sitnikov. Lastly the periodicities of the solutions have been examined by the Poincare section and four-body and three-body problem have been compared by different comparative graphs and surfaces.

Case report: Time of death estimation of a buried body by modeling a decomposition matrix for a pig carcass.

PubMed

Niederegger, Senta; Schermer, Julia; Höfig, Juliane; Mall, Gita

2015-01-01

Estimating time of death of buried human bodies is a very difficult task. Casper's rule from 1860 is still widely used which illustrates the lack of suitable methods. In this case study excavations in an arbor revealed the crouching body of a human being, dressed only in boxer shorts and socks. Witnesses were not able to generate a concise answer as to when the person in question was last seen alive; the pieces of information opened a window of 2-6 weeks for the possible time of death. To determine the post mortem interval (PMI) an experiment using a pig carcass was conducted to set up a decomposition matrix. Fitting the autopsy findings of the victim into the decomposition matrix yielded a time of death estimation of 2-3 weeks. This time frame was later confirmed by a new witness. The authors feel confident that a widespread conduction of decomposition matrices using pig carcasses can lead to a great increase of experience and knowledge in PMI estimation of buried bodies and will eventually lead to applicable new methods. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

[Occult bronchial foreign bodies - analysis of own material].

PubMed

Szafrański, Wojciech; Dobielski, Jarosław; Papiewski, Wojciech; Czechowska, Urszula

2013-01-01

The aspiration of a foreign body is usually combined with acute clinical symptoms requiring immediate medical intervention. Nevertheless, in approximately one third of patients the symptoms of aspiration are less prominent; such a clinical condition is called occult bronchial foreign body (OBFB). The aim of our study was to assess the frequency of OBFB in the pulmonary unit of a district hospital and to evaluate the diagnostic difficulties and treatment modalities in such patients. The examined group consisted of patients hospitalized in the Department of Lung Diseases in Radom District Hospital. A retrospective analysis of medical records was preformed. In the period 1978-2008 - 12 patients (10 males, 2 females) were hospitalized due to OBFB. The foreign bodies occluded the bronchi over 2 months (3 to 7) in 4 patients. The moment of aspiration was not remembered by 8 patients. Cases of OBFB were rare. In the presented material the frequency was 4 per 10,000 hospitalizations and 8 per 10,000 bronchoscopies. In our region of 600,000 population the index of hospitalization due to OBFB in adults (〉 14 years of age) was 0.07 per 100,000 inhabitants/year. Foreign bodies mainly included bone fragments (5 cases), vegetal remnants - clove of garlic, ear of corn (3 patients), and other food remnants (2 patients). Occasionally other aspirates were found, such as a wooden peg or a piece of plastic. The aspiration took place mostly during meals. The patients developed one or more of the following symptoms: purulent pneumonia (3 cases), pleural empyema (1 case), atelectasis (5 cases), and recurrent bronchitis and pneumonia (2 cases). The foreign body (fragments of plants) was mimicking a bronchial tumour in 4 patients. Fibre optic or rigid bronchoscopy was applied successfully in 11 patients. Only one patient needed surgical intervention. OBFB is a rare condition, but has to be taken into consideration as a cause of chest radiological pathology and in patients with chronic

Body checking and body avoidance in eating disorders: Systematic review and meta-analysis.

PubMed

Nikodijevic, Alexandra; Buck, Kimberly; Fuller-Tyszkiewicz, Matthew; de Paoli, Tara; Krug, Isabel

2018-05-01

This review sought to systematically review and quantify the evidence related to body checking and body avoidance in eating disorders (EDs) to gauge the size of effects, as well as examine potential differences between clinical and nonclinical populations, and between different ED subtypes. PsycINFO, PsycARTICLES, PsycEXTRA, Cochrane Library, and MEDLINE databases were searched for academic literature published until October 2017. A grey literature search was also conducted. Fifty-two studies were identified for the systematic review, of which 34 were eligible for meta-analysis. Only female samples were included in the meta-analysis. ED cases experienced significantly higher body checking (d = 1.26, p < .001) and body avoidance (d = 1.88, p < .001) overall relative to healthy controls, but neither behaviour varied by ED subtype. In nonclinical samples, body checking (r = .60) and body avoidance (r = .56) were significantly correlated with ED pathology (p < .001). These findings support transdiagnostic theoretical models and approaches to ED treatment and early intervention programmes. Copyright © 2018 John Wiley & Sons, Ltd and Eating Disorders Association.

Plastic body, permanent body: Czech representations of corporeality in the early twentieth century.

PubMed

Sleigh, Charlotte

2009-12-01

In the early twentieth century, the body was seen as both an ontogenetic and a phylogenetic entity. In the former case, its individual development, it was manifestly changeable, developing from embryo to maturity and thence to a state of decay. But in the latter case, concerning its development as a species, the question was an open one. Was its phylogenetic nature a stationary snapshot of the slow process of evolution, or was this too mutable? Historians have emphasised that the question of acquired inheritance remained open into the twentieth century; this paper explores how various constructions of the individual as a phylogenetic episode--a stage in the race's evolution--related to representations of the body in the same period. A discussion of the work of the brothers Josef and Karel Capek offers a contextualised answer to the question of bodily representation. Karel Capek (1890-1938) explored the nature of the 'average man' through two different organisms, the robot and the amphibian, epitomes respectively of corporeal permanence and plasticity. Josef Capek (1887-1945), along with other members of the Group of Plastic Artists, explored visual representations of the body that challenged cubist Bergsonian norms. In so doing, he affirmed what his brother also held: that despite the constrictions imposed by the oppressive political conditions in which the Czechs found themselves, the individual body was a fragile but fluid entity, capable of effecting change upon the future evolution of humankind.

Case study - human body relay race.

PubMed

Lewis, Heather

2017-04-01

This case presentation introduces the work of Heather Lewis, Graphic Designer from Birmingham Community Healthcare Foundation Trust, Clinical Illustration department. The graphic design team offer professional design solutions in a variety of formats such as scientific posters, banners, patient information booklets and promotional items. This particular project was requested by the Combined Community Dental Service, a Specialist Division in Birmingham.

Advances in Turbulent Combustion Dynamics Simulations in Bluff-Body Stabilized Flames-Body Stabilized Flames

DTIC Science & Technology

2015-11-30

Master’s Thesis 3. DATES COVERED (From - To) 01 Nov 2015 – 30 Nov 2015 4. TITLE AND SUBTITLE Advances in Turbulent Combustion Dynamics Simulations...the three main aspects of bluff-body stabilized flames: stationary combustion , lean blow-out, and thermo-acoustic instabilities. For the cases of...stationary combustion and lean blow-out, an improved version of the Linear Eddy Model approach is used, while in the case of thermo-acoustic

[Foreign body in the tricuspid valve with valvular insufficiency and right-left shunt].

PubMed

Delebarre, P; Augustin-Normand, C; Capronier, C; Cramer, J; Godeau, P; Letac, B; Forman, J; Maurice, P; Ourbak, P

1987-05-01

We present the case of a 50-year old man who progressively developed tricuspid valve insufficiency with opening of a patent foramen ovale responsible for right-to-left shunt with polycythaemia. The tricuspid valve insufficiency was due to a foreign body, probably of surgical origin as suggested by its radiological image and by the patient's previous history. It would have been introduced, far away from the tricuspid valve (compound fracture of the wrist), several years previously. At surgery, we found the foreign body embedded in the valve system. As a possible mechanism for the mutilation, an undiagnosed endocarditis was suspected but could not be confirmed. Three cases tricuspid endocarditis (with foreign bodies in the right ventricle) and 3 cases of asymptomatic tricuspid valve foreign bodies have been published. Fifty-five cases of foreign bodies introduced peripherally and migrated into the heart, the pericardium and the pulmonary artery are reviewed.

Removal of impacted esophageal foreign bodies with a dual-channel endoscope: 19 cases

PubMed Central

WANG, CHANGXIONG; CHEN, PING

2013-01-01

There have been few reports concerning the endoscopic removal of impacted esophageal foreign bodies from patients. The objective of this study was to evaluate the effectiveness of dual-channel endoscopy in managing foreign-body ingestions in patients. A total of 19 patients with foreign-body ingestions between September 2008 and July 2011 were selected from the Digestive Endoscope Center in Lishui, a typical middle-sized city in China. The patients underwent endoscopy following admission. The impacted foreign bodies were successfully removed from 18 patients without complications using a dual-channel endoscope. One patient underwent surgery for an ingested denture following the failure of the endoscopic removal method. This study demonstrates that dual-channel endoscopic management may be a useful option for removing ingested foreign bodies from the esophagus. PMID:23935752

BMI, body fat and waist-to-height ratio of stunted v. non-stunted Indian children: a case-control study.

PubMed

Savanur, Mitravinda S; Ghugre, Padmini S

2016-06-01

To compare the BMI, body fat and waist-to-height ratio (WHtR) of stunted and non-stunted children following different growth trajectories from low socio-economic strata in Mumbai, India. Cross-sectional, case-control study. Weight, height, skinfold thicknesses and waist circumference were measured. Information regarding the duration of breast-feeding, age at initiation of complementary feeding and income was obtained. Birth weight was obtained from records. BMI, body fat, WHtR and change in weight sd were calculated. Children who were beneficiaries of anganwadis, Mumbai city, India. Three hundred and thirty children aged 2-4 years were selected in each of the stunted and non-stunted groups after matching for age and sex. After adjusting for birth weight, change in weight sd, duration of breast-feeding, age at complementary feeding initiation and income, stunted children had significantly higher body fat, WHtR and BMI than the non-stunted (P<0·01). The stunted and non-stunted children were classified based on their change in weight sd. Stunted children with no change in weight sd had higher mean body fat, BMI (P<0·01) and WHtR (P<0·05) than their non-stunted counterparts. In the catch-up growth group, stunted children had higher BMI and WHtR than the non-stunted (both P<0·001). In the catch-down growth group, stunted children had higher BMI than the non-stunted (P<0·001). Stunting was seen to increase the tendency of conserving body fat in young children. Such a tendency, if continued during later childhood and adolescence, can increase the risk of obesity and non-communicable diseases.

Vaginal foreign body presenting as bleeding with defecation in a child.

PubMed

Abdessamad, Hasan M; Greenfield, Marjorie

2009-04-01

Symptoms secondary to a vaginal foreign body are responsible for approximately 4% of pediatric gynecologic outpatient visits.(1) The classic symptom is vaginal bleeding, but vaginal discharge, foul odor, irritation, abdominal pain, and hematuria have been described. We are reporting a case of a microscopic vaginal foreign body presenting as hematochezia in a preadolescent girl. This case is unique in that the patient presented with bleeding with defecation, without vaginal bleeding. Vaginal foreign bodies can present with diverse symptomatology. Physicians should consider the diagnosis of vaginal foreign body when presented with a young female patient with unexplained hematochezia.

Abdominal foreign body: late presentation as a rectus sheath abscess.

PubMed

Noushif, M; Sivaprasad, S; Prashanth, A

2011-05-01

Intra-abdominal ingested foreign bodies are usually an incidental finding, typically encountered in mentally challenged patients. We present the case of a 65-year-old mentally sound woman who presented with recurrent abdominal pain and a lump in the hypogastrium. Evaluation revealed a rectus sheath abscess extending to the peritoneum, with a foreign body in situ. On enquiry, the patient revealed that she had accidentally ingested a tailoring needle 17 years ago. This case illustrates an unusual presentation of an ingested foreign body as a rectus sheath abscess after a long duration.

Dietary Intake, Body Composition, and Menstrual Cycle Changes during Competition Preparation and Recovery in a Drug-Free Figure Competitor: A Case Study

PubMed Central

Halliday, Tanya M.; Loenneke, Jeremy P.; Davy, Brenda M.

2016-01-01

Physique competitions are events in which competitors are judged on muscular appearance and symmetry. The purpose of this retrospective case study was to describe changes in dietary intake, body mass/composition, and the menstrual cycle during the 20-week competition preparation (PREP) and 20-week post competition recovery (REC) periods of a drug-free amateur female figure competitor (age = 26–27, BMI = 19.5 kg/m2). Dietary intake (via weighed food records) and body mass were assessed daily and averaged weekly. Body composition was estimated via Dual-energy X-ray absorptiometry (DXA) and 7-site skinfold measurements. Energy intake, body mass and composition, and energy availability decreased during the 20-week PREP period (changes of ~298 kcals, 5.1 kg, 6.5% body fat, and 5.4 kcal/kg fat free mass, respectively) and returned to baseline values by end of the 20-week REC period. Menstrual cycle irregularity was reported within the first month of PREP and the last menstruation was reported at week 11 of PREP. Given the potentially adverse health outcomes associated with caloric restriction, future, prospective cohort studies on the physiological response to PREP and REC are warranted in drug-free, female physique competitors. PMID:27879627

Nasal Foreign Bodies: A Sweet Experiment.

PubMed

Leopard, D C; Williams, R G

2015-10-01

It is generally accepted that paediatric intranasal foreign bodies should be removed in the emergency setting. In the case of a difficult to access dissolvable foreign body in an uncooperative child, the question must be raised regarding whether or not a watch and wait strategy is more appropriate. We ask: How long does it take for popular sweets (candy) to dissolve in the human nose? Five popular UK sweets were placed in the right nasal cavity of a 29-year-old male (the author) with no sino-nasal disease. Time taken to dissolve was recorded. All five sweets were completely dissolved in under one hour. A watch and wait strategy in favour of examination under anaesthetic may be a viable option in some cases. Limitations of the study include the age of the participant and size of the sweets. It is also important in practice that the clinician is able to elicit an accurate history regarding the exact nature of the foreign body. It remains prudent to perform an examination under anaesthetic of an uncooperative child with a solid or unknown nasal foreign body. However, if the clinician can be certain the foreign body is a small sugar or chocolate based sweet only, a watch and wait strategy may be a reasonable choice. © 2015 John Wiley & Sons Ltd.

Bone edema of the whole vertebral body: an unusual case of spondyloarthritis.

PubMed

Ortolan, Augusta; Lazzarin, Paolo; Lorenzin, Mariagrazia; Rampin, Lucia; Ramonda, Roberta

2017-01-01

Spondyloarthritis (SpA) is usually characterized by early inflammatory involvement of the sacroiliac joints (SI), which constitutes one of the most important classification criteria according to the SpondyloArthritis International Society (ASAS). These criteria do not include inflammatory spine lesions which can be detected on MRI, although spine involvement is very common in axial SpA. This is because spine MRI lesion often retrieved in SpA are not very specific, and can be found in many other diseases such as malignancy and osteoarthritis. Here we present the case of a 33-year old woman who presented a worsening low back pain, with a thoracic spine MRI showing bone marrow edema (BME) of the whole T8 vertebral body. Owing to this peculiar presentation, together with the unresponsiveness of the pain to nonsteroidal anti inflammatory drugs (NSAIDs) and a slight increase of the biomarker CA19-9, a malignancy was suspected. Therefore, the patient underwent bone scintigraphy, Single positron emission computed tomography (SPET/TC), positron emission tomography and repeated MRI without reaching a diagnosis. Finally, when SI joints MRI was performed, BME of the SI joints emerged: this was fundamental to formulate the diagnosis of axSpA.

Evaluating Cumulative OP Pesticide Body Burden of Children: A National Case Study

PubMed Central

Payne-Sturges, Devon; Cohen, Jonathan; Castorina, Rosemary; Axelrad, Daniel A.; Woodruff, Tracey J.

2009-01-01

Biomonitoring is a valuable tool for identifying exposures to chemicals that pose potential harm to human health. However, to date there has been little published on ways to evaluate the relative public health significance of biomonitoring data for different chemicals, and even less on cumulative assessment of multiple chemicals. The objectives of our study are to develop a methodology for a health risk interpretation of biomonitoring data, and to apply it using NHANES 1999–2002 body burden data for organophosphorus (OP) pesticides. OP pesticides present a particularly challenging case given the non-specificity of many metabolites monitored through NHANES. We back-calculate OP pesticide exposures from urinary metabolite data, and compare cumulative dose estimates with available toxicity information for a common mechanism of action (brain cholinesterase inhibition) using data from U.S. EPA. Our results suggest that approximately 40% of children in the United States may have had insufficient margins of exposure (MOEs) for neurological impacts from cumulative exposures to OP pesticides (MOE less than 1,000). Limitations include uncertainty related to assumptions about likely precursor pesticide compounds of the urinary metabolites, sources of exposure, and intra-individual and temporal variability. PMID:19921915

Risk factors for granuloma formation in children induced by tracheobronchial foreign bodies.

PubMed

Huang, Zhenghua; Zhou, Ai; Zhang, Jianya; Xie, Lisheng; Li, Qi

2015-12-01

The aim of this study was to analyze the risk factors for granuloma formation caused by plant-based tracheobronchial foreign bodies in children, and investigate the underlying pathogenesis. In this retrospective analysis of 153 cases with tracheobronchial foreign bodies (peanuts and watermelon seeds), 35 cases of granuloma formation as granulation group (G), and 118 cases of no granuloma formation as non-granulation group (NG) were studied. Clinical data pertaining to sex (S), age (A), foreign body surface smoothness (SF), foreign body shape (SH), foreign body oil release state (O), the location of foreign bodies (L), and foreign body retention time (T) were collected for statistical analysis. Univariate analysis showed no significant difference between the two groups (G and NG) with respect to S, A, SH and L. Significant factors based on univariate analysis included SF, O and T. Multivariate logistic regression analysis revealed that SF and T were independent risk factors associated with development of granuloma. SF, O and T had relationship with the granuloma formation. Local trauma caused by an irregular and sharp foreign body, and extended period of time represent the main factors causing granuloma formation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Failure of surgical treatment in methamphetamine body-stuffers.

PubMed

Bahrami-Motlagh, Hooman; Hassanian-Moghaddam, Hossein; Behnam, Behdad; Arab-Ahmadi, Mehran

2015-05-01

Body stuffing is defined as ingestion of unpackaged or packaged illicit drugs in a quick process. The drugs have usually been wrapped loosely in cellophane, plastic bags, paper, or aluminum foil. Methamphetamine toxicity is a dangerous state that occurs during methamphetamine leakage from the ingested packages in the gastrointestinal tract. This is usually occurring with cocaine and heroin, but methamphetamine body stuffing may less commonly happen, as well. Accordingly, management of methamphetamine body-stuffers is an important subject that has remained a controversy in clinical and legal aspects. We have reported two body-stuffer cases who underwent exploratory laparotomy. Although surgery was done, it was not useful to exit packs and even led to severe methamphetamine toxicity. These cases show that surgical treatment may be ineffective and even harmful in body-stuffers. On the other hand, this report suggests that pre and post-operation abdominal CT-scan is necessary for evaluating surgical treatment in patients who are still symptomatic. Copyright © 2015 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

Railway vehicle body structures

DOE Office of Scientific and Technical Information (OSTI.GOV)

Not Available

1985-01-01

The strength and durability of railway vehicle structures is a major topic of engineering research and design. To reflect this importance the Railway Division of the Institution of Mechanical Engineers organised a conference to discuss all matters relating to railway vehicle design. This book presents the papers discussed in that conference. The contents include: Vehicle body design and the UIC's international contribution; LUL prototype 1986 stock - body structure; vehicle structure for the intermediate capacity transmit system vehicles; car body technology of advanced light rapid transit vehicles; concepts, techniques and experience in the idealization of car body structures for finitemore » element analysis; Calcutta metropolitan railway; design for a lightweight diesel multiple unit body; the design of lightweight inter-city coal structures; the BREL international coach body shell structure; new concepts and design techniques versus material standards; structures of BR diesel electric freight locomotives; structural design philosophy for electric locomotives; suspension design for a locomotive with low structural frequencies; freight wagon structures; a finite element study of coal bodyside panels including the effects of joint flexibility; a fresh approach to the problem of car body design strength; energy absorption in automatic couplings and draw gear; passenger vehicle design loads and structural crashworthiness; design of the front part of railway vehicles (in case of frontal impact); the development of a theoretical technique for rail vehicle structural crashworthiness.« less

Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001).

PubMed

Druce, M R; Kaltsas, G A; Fraenkel, M; Gross, D J; Grossman, A B

2009-09-01

Phaeochromocytoma and paraganglioma are rare neuroendocrine tumours (NETS). They may be benign or malignant but the pathological distinction is mainly made when metastases are present. Available treatments in the form of surgery, chemotherapy, and radionuclide therapy may improve symptoms and biochemical markers, but the results for the control of tumour bulk are less favourable. Furthermore, responses to treatment are frequently short-lived. This short review outlines the main molecular and histological features of malignant phaeochromocytoma and the difficulties in differentiating between benign and malignant disease. We list current therapies used for malignant pheochromocytoma; however, these generally achieve relatively low success rates. Hence, there is a need for new and more effective therapies. In vitro studies have implicated the PI3/Akt/mTOR pathway in the pathogenesis of malignant NETS, including phaeochromocytoma. Everolimus (RAD001, Novartis UK) is a compound that inhibits mTOR (mammalian Target Of Rapamycin) signalling. We have used RAD001 in four patients with progressive malignant paraganglioma/phaeochromocytoma in addition to other therapies (with institutional approval for compassionate use), and evaluated the effects of this treatment. We outline these four cases and review the theoretical background for this therapy, although the outcomes were relatively disappointing.

Increasing trend in retained rectal foreign bodies

PubMed Central

Ayantunde, Abraham A; Unluer, Zynep

2016-01-01

AIM To highlight the rising trend in hospital presentation of foreign bodies retained in the rectum over a 5-year period. METHODS Retrospective review of the cases of retained rectal foreign bodies between 2008 and 2012 was performed. Patients’ clinical data and yearly case presentation with data relating to hospital episodes were collected. Data analysis was by SPSS Inc. Chicago, IL, United States. RESULTS Twenty-five patients presented over a 5-year period with a mean age of 39 (17-62) years and M: F ratio of 2:1. A progressive rise in cases was noted from 2008 to 2012 with 3, 4, 4, 6, 8 recorded patients per year respectively. The majority of the impacted rectal objects were used for self-/partner-eroticism. The commonest retained foreign bodies were sex vibrators and dildos. Ninty-six percent of the patients required extraction while one passed spontaneously. Two and three patients had retrieval in the Emergency Department and on the ward respectively while 19 patients needed examination under anaesthesia for extraction. The mean hospital stay was 19 (2-38) h. Associated psychosocial issues included depression, deliberate self-harm, illicit drug abuse, anxiety and alcoholism. There were no psychosocial problems identified in 15 patients. CONCLUSION There is a progressive rise in hospital presentation of impacted rectal foreign bodies with increasing use of different objects for sexual arousal. PMID:27830039

Increasing trend in retained rectal foreign bodies.

PubMed

Ayantunde, Abraham A; Unluer, Zynep

2016-10-27

To highlight the rising trend in hospital presentation of foreign bodies retained in the rectum over a 5-year period. Retrospective review of the cases of retained rectal foreign bodies between 2008 and 2012 was performed. Patients' clinical data and yearly case presentation with data relating to hospital episodes were collected. Data analysis was by SPSS Inc. Chicago, IL, United States. Twenty-five patients presented over a 5-year period with a mean age of 39 (17-62) years and M: F ratio of 2:1. A progressive rise in cases was noted from 2008 to 2012 with 3, 4, 4, 6, 8 recorded patients per year respectively. The majority of the impacted rectal objects were used for self-/partner-eroticism. The commonest retained foreign bodies were sex vibrators and dildos. Ninty-six percent of the patients required extraction while one passed spontaneously. Two and three patients had retrieval in the Emergency Department and on the ward respectively while 19 patients needed examination under anaesthesia for extraction. The mean hospital stay was 19 (2-38) h. Associated psychosocial issues included depression, deliberate self-harm, illicit drug abuse, anxiety and alcoholism. There were no psychosocial problems identified in 15 patients. There is a progressive rise in hospital presentation of impacted rectal foreign bodies with increasing use of different objects for sexual arousal.

Significance of fibrotic bands in utero--Amniotic band sequence with limb body wall complex: A rare case of fetal autopsy.

PubMed

Devi, P Lekshmi; Cicy, P J; Thambi, Renu; Poothiode, Usha

2015-01-01

Amniotic band sequence (ABS) includes a wide spectrum of abnormalities resulting from entrapment of various fetal parts from a disrupted amnion, ranging from a mere constriction ring affecting a finger to a fatal form called limb body wall complex (LBWC). Reported cases of ABS with LBWC are very few. The spectrum of anomalies depends on which part gets entrapped and at what point of gestation. Hence, the clinical presentation can be extremely variable. Early detection of such cases using sonology is really challenging due to the small size of the fibrotic bands. Here, we present a case of amniotic band syndrome with LBWC in a fetus at 24 weeks of gestation, which was referred for an autopsy. The fetus also showed scoliosis, gastroschisis, lumbosacral meningocele, congenital talipes equinovarus, and cleft palate, thus having features of placenta cranial and placenta abdominal phenotype which is very rare.

Flight dynamics of axisymmetric rotating bodies in an air medium

NASA Astrophysics Data System (ADS)

Borisenok, I. T.; Lokshin, B. Ia.; Privalov, V. A.

1984-04-01

The free flight motion of a rigid axisymmetric body due to the action of its own weight, aerodynamic effects (autorotation), and possible reactive forces is examined. It is assumed that the central ellipsoid of inertia of the body is an ellipsoid of rotation about the axis of symmetry, and that the center of gravity is at the geometric center of the body. The region of stability of vertical descent is approximated by dividing a system of characteristic equations into fast and slow parts. It is shown that, for given gyroscopic forces, the presence of the nonconservative Magnus moment may lead to a loss of stability of this type of motion. The stability of the case of planar motion, where the Magnus force and weight form an equilibrium force system, and of the case of spiral motion is considered. Stability is also studied for the case of the center of mass at an arbitrary point on the axis of symmetry, and for the case of an axisymmetric body not having an equatorial plane of symmetry. Conditions for the equilibrium and precession stability of a rotating parachute in a wind tunnel are identified.

Paraganglioma Anatomy

MedlinePlus

... exact term or phrase, no variations. Use multiple keywords separated by spaces (e.g. kidney renal ) for ... higher those with all or most of the keywords. Plurals and other variations are automatically included. To ...

Four-body decays of B meson with lepton number violation

NASA Astrophysics Data System (ADS)

Yuan, Han; Wang, Tianhong; Jiang, Yue; Li, Qiang; Wang, Guo-Li

2018-06-01

The existence of heavy meson lepton number violating (LNV) processes shows the Majorana nature of the neutrino. Much of this theoretical and experimental researche focuses on this type of decay. Four-body epton-number violation (LNV) processes of the B meson may have sizable branching ratios as they share the same vertex and mixing parameters with the three-body case. Mixing parameters between the heavy Majorana neutrino and charged leptons extracted from the three-body case can be used to constrain the branching ratios of four-body decays of the B meson. So we can update the upper limits of these mixing parameters with new experimental data of the three-body LNV decays. We also analyze {B}0\\to {D}* -{{\\ell }}1+{{\\ell }}2+{M}2- using the updated parameters and estimate some channels’ reconstruction events using current experimental data from Belle.

[Body experience and motion in a biographical context: about the necessity of a body- and motion-related biographical view in gerontology].

PubMed

Abraham, Anke

2008-06-01

According to the special view of natural sciences, ageing processes are connected with measurable changes in the body. At the same time we know little about how bodily change is experienced and the subjective acceptance of the body during aging. Therefore a perspective with respect to the body has to be systematically embraced in gerontology. Knowledge perspectives and the view of the body are exemplified in theory and by analysing a case. The knowledge of experience and sense of body and motion in a person's life allows the creation of stimulating offers of growth development and health in age.

Experimental investigation of biodynamic human body models subjected to whole-body vibration during a vehicle ride.

PubMed

Taskin, Yener; Hacioglu, Yuksel; Ortes, Faruk; Karabulut, Derya; Arslan, Yunus Ziya

2018-02-06

In this study, responses of biodynamic human body models to whole-body vibration during a vehicle ride were investigated. Accelerations were acquired from three different body parts, such as the head, upper torso and lower torso, of 10 seated passengers during a car ride while two different road conditions were considered. The same multipurpose vehicle was used during all experiments. Additionally, by two widely used biodynamic models in the literature, a set of simulations were run to obtain theoretical accelerations of the models and were compared with those obtained experimentally. To sustain a quantified comparison between experimental and theoretical approaches, the root mean square acceleration and acceleration spectral density were calculated. Time and frequency responses of the models demonstrated that neither of the models showed the best prediction performance of the human body behaviour in all cases, indicating that further models are required for better prediction of the human body responses.

The First Confirmed Case of Down Syndrome with Dementia with Lewy Bodies

ERIC Educational Resources Information Center

Prasher, V. P.; Airuehia, E.; Carey, M.

2010-01-01

Dementia with Lewy bodies (DLB) is the second commonest cause of dementia in the general population. Several researches have established an association between Down syndrome (DS) and Alzheimer's disease. Very few studies have however showed such an association between dementia with Lewy bodies and Down syndrome. The occurrence of DLB in persons…

Glomus Tumor of the Neck Detected With 99mTc EDDA HYNIC-TOC.

PubMed

Girotto, Neva; Bogović-Crnčić, Tatjana; Grbac-Ivanković, Svjetlana; Valković-Zujić, Petra

2017-10-01

A 54-year-old woman was referred to thyroid evaluation because of a lump on the left side of the neck. Ultrasound exam did not show any thyroid abnormality, but highly perfused nodule at the left common carotid artery bifurcation was found. Because of the specific location, somatostatin receptor scintigraphy with Tc EDDA HYNIC-TOC was performed, starting with perfusion images and followed with SPECT/CT imaging at 2 and 4 hours. Well-perfused nodule with intensive accumulation and no other visible pathology in the body raised suspicion of a glomus tumor, consistent with MR exam performed later. Subsequent surgical removal confirmed carotid paraganglioma.

Body-packing as cause of unexpected sudden death.

PubMed

Heinemann, A; Miyaishi, S; Iwersen, S; Schmoldt, A; Püschel, K

1998-03-02

Four fatalities related to smuggling of drugs by body-packing were investigated. The victims were examined at the Institute of Forensic Medicine of Hamburg University between 1983 and 1995, two of them due to "sudden" unknown cause of death. All victims were male. Two of them were found already dead in a backyard and in a hotel, two other were emergency cases and died at a hospital. Smuggled substances included cocaine (two cases), heroin and amphetamine/caffeine. In all cases, the cause of death was intoxication caused by torn packages which were detected at autopsy. The maximum weight of the packet's contents was 630 g divided in 90 packages. Only one victim was apparently an intravenous drug-abuser. Hair analysis was performed in three cases and revealed in one case a difference between a concealed and a habitually consumed drug. Toxicological analysis revealed that the substances were quite pure and provided evidence that rather long survival was possible following intoxication in three cases, in two cases supported by hospital treatment in the final stage. The procedural regimen in cases of suspected body-packing is discussed.

Incidental Lewy Body Disease: Clinical Comparison to a Control Cohort

PubMed Central

Adler, Charles H.; Connor, Donald J.; Hentz, Joseph G.; Sabbagh, Marwan N.; Caviness, John N.; Shill, Holly A.; Noble, Brie; Beach, Thomas G.

2010-01-01

Limited clinical information has been published on cases pathologically diagnosed with incidental Lewy body disease (ILBD). Standardized, longitudinal movement and cognitive data was collected on a cohort of subjects enrolled in the Sun Health Research Institute Brain and Body Donation Program. Of 277 autopsied subjects who had antemortem clinical evaluations within the previous 3 years, 76 did not have Parkinson’s disease, a related disorder, or dementia of which 15 (20%) had ILBD. Minor extrapyramidal signs were common in subjects with and without ILBD. Cognitive testing revealed an abnormality in the ILBD group in the Trails B test only. ILBD cases had olfactory dysfunction; however, sample size was very small. This preliminary report revealed ILBD cases have movement and cognitive findings that for the most part were not out of proportion to similarly assessed and age-similar cases without Lewy bodies. Larger sample size is needed to have the power to better assess group differences. PMID:20175211

[Foreign bodies in the esophagus not removable endoscopically--the surgical procedure and treatment].

PubMed

Markov, G; Kondarev, G

1999-01-01

During 1991-1997 period 37 patients with foreign bodies in the esophagus were treated in our clinic. In 36 cases the foreign body was "starlet". That is a construction made for this special purpose. It lodges mainly in the upper digestive tract. The most usual localization is the middle third of the esophagus. Unlike the foreign bodies that impact there by accident the operative activity is very high in these cases. Out of the total number of 37 patients 22 were operated, in 7 cases an endoscopic extraction was performed and in one case the foreign body eliminated spontaneously. Three patients died before we performed any manipulations whatsoever and four of them refused any treatment. We offer methods for treating such patients which in our opinion are the most appropriate ones for this type of pathology.

The correlation between the amplitude of Osborn wave and core body temperature.

PubMed

Omar, Hesham R; Camporesi, Enrico M

2015-08-01

Several reports illustrate an inverse correlation between the Osborn wave (J wave) amplitude and core body temperature. We attempted to study the strength of this correlation. We reviewed all articles reporting hypothermic J waves from 1950-2014 for patient demographics, core body temperature in Celsius (°C), amplitude of the J wave in millimeters (mm), lead with the highest amplitude of J wave, presence of acidosis, PO2, electrolytes and outcome. In cases with more than one electrocardiogram (ECG), the respective core body temperature and J wave amplitude of each ECG were recorded. The main study outcome is to evaluate the correlation between the J wave amplitude and core body temperature in the admission ECG. We have also examined the strength of this relationship in cases with more than one ECG. We attempted to find the most frequent lead that recorded the highest amplitude of the J wave in addition to the correlation between the amplitude of J wave and pH. We found 64 articles comprising a total of 68 cases. When analyzing only cases with more than one reported ECG, there was a strong inverse correlation (r = - 0.682, p<0.001) between J wave amplitude and body temperature: however, when analyzing admission ECG of all cases, the correlation was only moderate (r = - 0.410, p<0.001). The lead with the highest amplitude of the J wave was V4 (44% of the cases, p<0.001) followed by V3 (23.7% of the cases, p<0.001). The amplitude of the J wave in the admission ECG of hypothermic patients may not accurately predict the core body temperature. © The European Society of Cardiology 2014.

A Diagnostic Dilemma for the Pediatrician: Radiolucent Tracheobronchial Foreign Body.

PubMed

Taşkınlar, Hakan; Bahadır, Gökhan Berktuğ; Erdoğan, Cankat; Yiğit, Doğakan; Avlan, Dinçer; Naycı, Ali

2017-06-01

The purpose of this study is to determine the role of clinical history, physical examinations, and radiological findings in the evaluation of patients with suspected radiolucent foreign body aspiration. The medical records of 236 children (under the age of 18 years), on whom a rigid bronchoscopy was performed between 1999 and 2015 because of suspected radiolucent foreign body aspiration, were analyzed retrospectively. Sensitivity, specificity, positive and negative predictive values of clinical history, physical examinations, and radiological findings were evaluated. In 71.1% of all cases, the children were under the age of 3 years. The bronchoscopy showed the presence of a foreign body in 52.9% of cases, with the locations of the foreign bodies being as follows: (1) right main bronchus, 47.2%; (2) left main bronchus, 36.0%; (3) trachea, 11.2%; (4) both bronchi, 5.6%. Organic foreign bodies were found in 78% of the patients, whereas inorganic foreign bodies were detected in 22% of the patients. The sensitivity and specificity of clinical history, physical examinations, and radiological findings were 98.4% and 54.9%, 47.2% and 74.7%, and 35.2% and 92.7%, respectively. Tracheobronchial foreign body aspirations usually occur prior to the age of 3 years, with the most frequently aspirated foreign bodies being food or items of a radiolucent nature. Clinical history, physical examinations, and radiological findings are not able to detect the presence of a radiolucent foreign body aspiration in children. Therefore, a bronchoscopy should be performed on children in whom a choking event has been witnessed, even in cases of normal radiological and clinical findings. Copyright © 2016. Published by Elsevier B.V.

Attitude dynamics simulation subroutines for systems of hinge-connected rigid bodies

NASA Technical Reports Server (NTRS)

Fleischer, G. E.; Likins, P. W.

1974-01-01

Several computer subroutines are designed to provide the solution to minimum-dimension sets of discrete-coordinate equations of motion for systems consisting of an arbitrary number of hinge-connected rigid bodies assembled in a tree topology. In particular, these routines may be applied to: (1) the case of completely unrestricted hinge rotations, (2) the totally linearized case (all system rotations are small), and (3) the mixed, or partially linearized, case. The use of the programs in each case is demonstrated using a five-body spacecraft and attitude control system configuration. The ability of the subroutines to accommodate prescribed motions of system bodies is also demonstrated. Complete listings and user instructions are included for these routines (written in FORTRAN V) which are intended as multi- and general-purpose tools in the simulation of spacecraft and other complex electromechanical systems.

[Foreign bodies aspiration in pediatrics. 15-year experience. Analysis of 337 cases].

PubMed

Aguirre Vázquez, I R; Blanco Rodríguez, G; Penchyna Grub, J; Teyssier Morales, G; Serrano Salas, A L

2013-01-01

Foreign body aspiration is a pediatric urgency that occurs primarily between 1 and 3 years old. The aim of this paper is to present the symptomatology, clinical and radiological findings, and the therapeutic used in our hospital with patients with foreign body aspiration diagnosis from 1995 until 2011. Patient's age was between 0 and 16 years old. We included 337 patients; where males predominate (205). The medium age was 23 months. The most frequent foreign bodies founded were seeds (192) in 57% and metallic objects in 18.3%. Cough (74.5%), dyspnea (70%), cyanosis (42.4%) and stridor (26%), were the main symptoms. Right bronchus was the most frequent localization (37.7%). In 21% of the patients the reference diagnostic was wrong due to treating it as a respiratory infection. All foreign bodies were extracted by rigid bronchoscopy. No deaths were reported in any proceeding. In our experience, infants are the most affected group, and the primordial symptoms are cough and dyspnea. Organic objects had the highest prevalence of aspiration.

Neglected foreign body aspiration mimicking bronchial carcinoma.

PubMed

Afghani, Reza; Khandashpour Ghomi, Mahmoud; Khandoozi, Seyed Reza; Yari, Behrouz

2016-07-01

Foreign body aspiration can occur in any age group, but it is more commonly seen in children. In adults, there is usually a predisposing condition that poses a risk of aspiration. If aspiration occurs, prompt diagnosis and extraction of the foreign body is needed to prevent early and late complications. We report a rare case of neglected foreign body aspiration in a 45-year-old schizophrenic opium addicted patient, which resulted in an occlusive lesion in the bronchus, mimicking bronchial carcinoma. © The Author(s) 2016.

Body Dysmorphic Disorder in Patients Seeking Abdominoplasty, Rhinoplasty, and Rhytidectomy.

PubMed

de Brito, Maria José Azevedo; Nahas, Fábio Xerfan; Cordás, Táki Athanássios; Tavares, Hermano; Ferreira, Lydia Masako

2016-02-01

Body dysmorphic disorder may negatively affect self-perception of body shape and lead patients to seek cosmetic surgery. This study estimates the level of body dissatisfaction and prevalence of body dysmorphic disorder symptoms in candidates for three plastic surgical procedures. Three hundred patients of both sexes divided into three groups (abdominoplasty, n = 90; rhinoplasty, n =151; and rhytidectomy, n =59) were classified as having (n =51, n =79, and n =25, respectively) or not having (n =39, n =72, and n =34, respectively) body dysmorphic disorder symptoms, based on the Body Dysmorphic Disorder Examination, which was administered preoperatively. Prevalence rates of body dysmorphic disorder symptoms in the abdominoplasty, rhinoplasty, and rhytidectomy groups were 57, 52, and 42 percent, respectively. Significant between-group differences were observed regarding age (p < 0.001), body mass index (p = 0.001), and onset of body dysmorphic disorder symptoms (p < 0.001). Within-group differences in body dysmorphic disorder severity were observed in the abdominoplasty (p < 0.001), rhinoplasty (p < 0.001), and rhytidectomy (p = 0.005) groups. Body dysmorphic disorder severity was significantly associated with degree of body dissatisfaction (mean Body Dysmorphic Disorder Examination total scores; p < 0.001), avoidance behaviors (p< 0.001), sexual abuse (p = 0.026), suicidal ideation (p < 0.001), and suicide attempt (p = 0.012). Abdominoplasty candidates showed the highest prevalence; rhytidectomy candidates exhibited the highest percentage of severe cases, and rhinoplasty candidates had the lowest percentage of severe cases.

Tracheo-bronchial foreign bodies: a retrospective study and review of literature.

PubMed

Jaswal, Abhishek; Jana, Utpal; Maiti, Pradip Kumar

2014-01-01

Tracheobronchial foreign body aspiration is a common emergency in childhood constituting major cause of mortality. Although ample studies regarding airway foreign bodies are present in western literature, studies in Indian context are however lacking. The aim of the study is to present an epidemiological data regarding airway foreign bodies in Indian context thereby helping to analyze the situation with regard to our socio-economic condition. Retrospective file review of all case (n = 82) that underwent rigid bronchoscopy for suspected tracheo-bronchial foreign body over a period of 7 years (2001-2008) in the department of otolaryngology of a tertiary care centre of eastern India. Patient characteristics, history, clinical, radiographic and bronchoscopic findings were noted in an attempt to define the epidemiology, clinical presentation, management and associated morbidity. Most common age of presentation was between 1 and 3 years (56.4%). Most common symptom in our study was Cough, wheezing and respiratory distress (63.4%). Most common clinical signs at presentation were diminished breath sound in unilateral lung field seen in 36.6% cases. Most common radiological finding on chest radiograph was collapse seen in 41.65% cases. Most common type of foreign body below 3 years of age was food material (seeds, beans) removed in 48.78%. Complications were encountered in 14.6% cases of which most common complication was bronchospasm and acute respiratory distress seen in 41.6% cases.

Aerodynamic Analysis of Body-Strake Configurations

DTIC Science & Technology

2006-06-01

combination by Macha . Adjustment for finite body was done based on the empirical correlation for circular bodies by Jorgensen. Additional adjustment...these cases were based on the results obtained with plain cylinders. Schematic from Macha . Fig. 2 also compares Macha’s results for circular...is apparent that Macha included in his charts plain cylinder data at s/r=1.0 and that his curves consider these data. In Section 5.5 of Ref. 6 he

Body ownership promotes visual awareness.

PubMed

van der Hoort, Björn; Reingardt, Maria; Ehrsson, H Henrik

2017-08-17

The sense of ownership of one's body is important for survival, e.g., in defending the body against a threat. However, in addition to affecting behavior, it also affects perception of the world. In the case of visuospatial perception, it has been shown that the sense of ownership causes external space to be perceptually scaled according to the size of the body. Here, we investigated the effect of ownership on another fundamental aspect of visual perception: visual awareness. In two binocular rivalry experiments, we manipulated the sense of ownership of a stranger's hand through visuotactile stimulation while that hand was one of the rival stimuli. The results show that ownership, but not mere visuotactile stimulation, increases the dominance of the hand percept. This effect is due to a combination of longer perceptual dominance durations and shorter suppression durations. Together, these results suggest that the sense of body ownership promotes visual awareness.

Foreign bodies ingestion: what responsibility?

PubMed

Ricci, Serafino; Massoni, Francesco; Schiffino, Luigi; Pelosi, Marcello; Salesi, Marialucia

2014-03-01

The ingestion of foreign bodies is one of the most important and difficult emergencies for a physician to diagnose. Accidental ingestion is more common in children, in patients with dental implants, in individuals with mental disability and in drug users. Voluntary ingestion is found in patients who are psychologically unstable, in prisoners or those who attempt suicide. Foreign bodies may be divided into food as fish bones, chicken bones, food bolus, meat, etc. or real foreign bodies such as orthodontic implants, needles, pins, glass, coins, etc. The authors present a case of management, from the medicolegal point of view, of a female patient age 80, who complained, for some weeks of modest pain in the left iliac fossa, and afterwards the endoscopy showed a toothpick into the wall of the sigmoid colon. Assessed of the clinical status of the patient presented severe cardiac comorbidities so that before processing the patient to a second resolutive endoscopy, it was necessary to obtain the hemodynamic stability. However the management of cases of accidental ingestion of foreign bodies is particularly difficult. Medical errors can arise from the very first contact with the patient resulting in delays in appropriate treatment. The doctor to avoid compromising its position on medical liability, must use all the knowledge and diligence known by the art and science of medicine. Copyright © 2013 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

Phantom Sensations, Supernumerary Phantom Limbs and Apotemnophilia: Three Body Representation Disorders.

PubMed

Tatu, Laurent; Bogousslavsky, Julien

2018-01-01

Body representation disorders continue to be mysterious and involve the anatomical substrate that underlies the mental representation of the body. These disorders sit on the boundaries of neurological and psychiatric diseases. We present the main characteristics of 3 examples of body representation disorders: phantom sensations, supernumerary phantom limb, and apotemnophilia. The dysfunction of anatomical circuits that regulate body representation can sometimes have paradoxical features. In the case of phantom sensations, the patient feels the painful subjective sensation of the existence of the lost part of the body after amputation, surgery or trauma. In case of apotemnophilia, now named body integrity identity disorder, the subject wishes for the disappearance of the existing and normal limb, which can occasionally lead to self-amputation. More rarely, a brain-damaged patient with 4 existing limbs can report the existence of a supernumerary phantom limb. © 2018 S. Karger AG, Basel.

Quasi-cylindrical theory of wing-body interference at supersonic speeds and comparison with experiment

NASA Technical Reports Server (NTRS)

Nielsen, Jack N

1955-01-01

A theoretical method is presented for calculating the flow field about wing-body combinations employing bodies deviating only slightly in shape from a circular cylinder. The method is applied to the calculation of the pressure field acting between a circular cylindrical body and a rectangular wing. The case of zero body angle of attack and variable wing incidence is considered as well as the case of zero wing incidence and variable body angle of attack. An experiment was performed especially for the purpose of checking the calculative examples.

Dynamic Parameter Identification of Subject-Specific Body Segment Parameters Using Robotics Formalism: Case Study Head Complex.

PubMed

Díaz-Rodríguez, Miguel; Valera, Angel; Page, Alvaro; Besa, Antonio; Mata, Vicente

2016-05-01

Accurate knowledge of body segment inertia parameters (BSIP) improves the assessment of dynamic analysis based on biomechanical models, which is of paramount importance in fields such as sport activities or impact crash test. Early approaches for BSIP identification rely on the experiments conducted on cadavers or through imaging techniques conducted on living subjects. Recent approaches for BSIP identification rely on inverse dynamic modeling. However, most of the approaches are focused on the entire body, and verification of BSIP for dynamic analysis for distal segment or chain of segments, which has proven to be of significant importance in impact test studies, is rarely established. Previous studies have suggested that BSIP should be obtained by using subject-specific identification techniques. To this end, our paper develops a novel approach for estimating subject-specific BSIP based on static and dynamics identification models (SIM, DIM). We test the validity of SIM and DIM by comparing the results using parameters obtained from a regression model proposed by De Leva (1996, "Adjustments to Zatsiorsky-Seluyanov's Segment Inertia Parameters," J. Biomech., 29(9), pp. 1223-1230). Both SIM and DIM are developed considering robotics formalism. First, the static model allows the mass and center of gravity (COG) to be estimated. Second, the results from the static model are included in the dynamics equation allowing us to estimate the moment of inertia (MOI). As a case study, we applied the approach to evaluate the dynamics modeling of the head complex. Findings provide some insight into the validity not only of the proposed method but also of the application proposed by De Leva (1996, "Adjustments to Zatsiorsky-Seluyanov's Segment Inertia Parameters," J. Biomech., 29(9), pp. 1223-1230) for dynamic modeling of body segments.

A case of adult-onset reducing body myopathy presenting a novel clinical feature, asymmetrical involvement of the sternocleidomastoid and trapezius muscles.

PubMed

Fujii, Takayuki; Hayashi, Shintaro; Kawamura, Nobutoshi; Higuchi, Masa-Aki; Tsugawa, Jun; Ohyagi, Yasumasa; Hayashi, Yukiko K; Nishino, Ichizo; Kira, Jun-Ichi

2014-08-15

We herein report a 32-year-old woman with adult-onset reducing body myopathy (RBM) who had a mutation in the four-and-a-half LIM domain 1 gene (FHL1) and showed a marked asymmetrical involvement of sternocleidomastoid and trapezius muscles. At 30 years of age she noticed bilateral foot drop, and over the next two years developed difficulty raising her right arm. At 32 years of age she was admitted to our hospital for a diagnostic evaluation. Neurological examination showed moderate weakness and atrophy of her right sternocleidomastoid muscle, right trapezius muscle, and bilateral upper proximal muscles. There were severe weakness and atrophy of her bilateral tibialis anterior muscles. Her deep tendon reflexes were hypoactive in her upper extremities. Her serum creatine kinase level was mildly increased. Muscle biopsy specimens from the left tibialis anterior muscle revealed marked variation in fiber size, some necrotic or regenerating fibers, and reducing bodies. Gene analysis of FHL1 demonstrated a mutation: a heterozygous missense mutation of c.377G>A (p. C126T) in FHL1. Compared with previous adult-onset RBM cases harboring mutations in FHL1, our case was characterized by asymmetrical atrophy of the sternocleidomastoid and trapezius muscles. Copyright © 2014 Elsevier B.V. All rights reserved.

Ingested and Aspirated Foreign Bodies.

PubMed

Green, S Sarah

2015-10-01

Esophageal and aspirated foreign bodies have important clinical significance, and both should be considered carefully when the history or physical examination findings raise sufficient suspicion. The published evidence regarding the diagnosis and management of foreign body ingestion or aspiration is weighted disproportionately with observational studies, case controls, expert opinion, and systematic reviews. Most of the publications would receive a categorization of C (observational studies including case-control and cohort design) and D (expert opinion, case reports, and clinical reasoning). One of the few prospective studies examining the diagnostic evaluation of foreign body aspiration in children could be considered level B evidence (randomized clinical trials, systematic reviews, or diagnostic studies with minor limitations). This study found that the medical history is the most important predictive part of the evaluation. There is evidence for considering bronchoscopy if there is significant history suggestive of foreign body aspiration, even in the setting of normal physical examination findings. (28). Most ingested foreign bodies spontaneously pass without incident. However, special attention should be paid to objects in the esophagus as well as to batteries and magnets. Based on a systematic review of the literature (level B evidence) and the potential for rapid and life-threatening damage, batteries in the esophagus should be removed immediately. (10) Other objects, such as coins, may be observed for passage in an asymptomatic patient. In addition, given the high risk of significant complications, ingestion of high-powered magnets should be quickly and carefully evaluated. Although single magnets are likely to pass without complication, multiple magnets or magnets ingested with other metal objects can cause significant damage and should be removed if there is any concern for mural entrapment, bowel perforation, or failure to progress. (10

Aristotle on Like-partedness and the Like-parted Bodies.

PubMed

Berman, Brad

2015-01-01

This paper offers an interpretation of Aristotle's treatment of the homoeomerous, or like-parted, bodies. I argue that they are liable to be far more complexly structured than is commonly supposed. While Aristotelian homoeomers have no intrinsic macrostructural properties, they are, in an important class of cases, essentially marked by the presence and absence of microstructural ones. As I show, these microstructural properties allow Aristotle to neatly demarcate the non-elemental homoeomers from the elements. That demarcation, in turn, helps to clarify Aristotle's conceptions of both homoeomery and what it is to be a bodily element. On Aristotle's account, I argue, a homoeomerous body, as such, is divisible into at least one part that is the same specific kind as the whole. Elemental bodies are the limiting case. For Aristotle, an elemental body is only divisible into parts that are of the same specific kind as the whole.

Late sequelae of retained foreign bodies after world war II missile injuries.

PubMed

Surov, Alexey; Thermann, Florian; Behrmann, Curd; Spielmann, Rolf-Peter; Kornhuber, Malte

2012-09-01

A number of people injured during the second world war harbour foreign bodies such as grenade splinters or bullets in some part of the body. Most of these metal fragments remain clinically silent. Some of them, however, may cause delayed complications. The purpose of this study was to determine the characteristics of delayed complications associated with foreign bodies after world war II injuries. 159 patients with retained foreign bodies after world war II injuries were retrospectively identified radiologically in our data bases in the time interval from 1997 to 2009. Diverse delayed complications secondary to the metal objects were diagnosed in 3 cases (2%): one patient with grenade splinter migration into the choledochal duct, one case with pseudotumoural tissue reaction, and one patient with late osteomyelitis. The time from injury to clinical presentation varied from 56 to 61 years. PubMed and Medline were screened for additional cases with delayed sequelae after foreign body acquisition during the 2nd world war. A 30 year search period from 1980 up to date was selected. 15 cases were identified here. Our study demonstrates that health consequences of the 2nd world war extend into the present time, and therefore physicians should be aware of the presence of hidden foreign bodies and their different possible late reactions. Crown Copyright © 2011. Published by Elsevier Ltd. All rights reserved.

Adolescent build plotting on body composition chart and the type of diabetes mellitus.

PubMed

Park, Hye Won; Kim, Yong Hyuk; Cho, Myunghyun; Kwak, Byung Ok; Kim, Kyo Sun; Chung, Sochung

2012-11-01

Although the prevalence of type 2 diabetes is increasing, there are cases difficult to categorize into certain type in pediatric diabetic patients. The aims of this study were to detect and choose a proper treatment modality for atypical cases of diabetes mellitus, using the body composition chart. We conducted a retrospective study from August 2005 to 2012 with patients who visited Konkuk University Medical Center, and were diagnosed with diabetes mellitus. The medical records were reviewed for the anthropometric data and indices of body composition. The subjects were grouped by the type of diabetes and gender. We constructed a body composition chart plotting fat free mass index and fat mass index (FMI). Body mass index and all body composition indices were higher in type 2 diabetes, in each gender in analysis with Mann-Whitney test. Significant determinant of diabetes type was revealed as FMI and contributing factors on FMI were analyzed with regression analysis. Six atypical cases were identified by a body composition chart including non-obese type 2 diabetes showing suboptimal growth with lower BMI related to relatively lower insulin secretion and type 1 diabetes with insulin resistance resulted from obesity. Body composition chart analysis might be useful in characterization of diabetes type and detection of atypical cases and early adjustment of diabetes management strategy.

Near Wake Depletion of Non-Magnetized Bodies Immersed in Mesosonic Plasma Flow

NASA Technical Reports Server (NTRS)

Wright, K. H.; Stone, N. H.; Samir, U.; Sorensen, J.; Winningham, J. D.

1997-01-01

During the recent TSS-1R mission, measurements of ion depletion in the near wake were obtained at a downstream distance of two body radii from the satellite center. The ratio of satellite radius to Debye length is approximately 150. Similar measurements were also obtained at the same downstream location in the wake of the shuttle during the Spacelab 2 mission of August 1985. In the case of the shuttle, the ratio of body radius to Debye length is greater than 1000. The wake depletion observed in the these two cases, together with data obtained from previous ionospheric satellites and from applicable laboratory experiments involving small bodies, will be compared in order to determine the influence of body size on wake filling. Extrapolation of these results to the case of the moon in the solar wind will be noted.

Circumferential Ciliary Body Cysts Presenting as Acute Pigment Dispersion and Ocular Hypertension.

PubMed

Sarıgül Sezenöz, Almila; Güngör, Sirel Gür; Kıratlı, Hayyam; Akman, Ahmet

2017-09-15

To report a case of circumferential neuroepithelial cyst of the ciliary body presenting with pigment dispersion (PD) and ocular hypertension. 48-year-old female patient presented with a complaint of pain in the left eye. On examination, visual acuity of the left eye was 0.9, and the intraocular pressure was 48 mmHg. Biomicroscopic anterior segment examination of the left eye revealed 4+ pigmented cells in the anterior chamber. Active PD from the pupillary region at 11 o'clock was noticed at the time of the examination. Ultrasound biomicroscopy demonstrated 360º cystic lesions of the ciliary body in the left eye. The patient was diagnosed as neuroepithelial cyst of the ciliary body. Our case is unique as it is the first case of circumferential neuroepithelial ciliary body cyst presenting with acute PD and ocular hypertension.

Attitude dynamics simulation subroutines for systems of hinge-connected rigid bodies with nonrigid appendages

NASA Technical Reports Server (NTRS)

Fleischer, G. E.; Likins, P. W.

1975-01-01

Three computer subroutines designed to solve the vector-dyadic differential equations of rotational motion for systems that may be idealized as a collection of hinge-connected rigid bodies assembled in a tree topology, with an optional flexible appendage attached to each body are reported. Deformations of the appendages are mathematically represented by modal coordinates and are assumed small. Within these constraints, the subroutines provide equation solutions for (1) the most general case of unrestricted hinge rotations, with appendage base bodies nominally rotating at a constant speed, (2) the case of unrestricted hinge rotations between rigid bodies, with the restriction that those rigid bodies carrying appendages are nominally nonspinning, and (3) the case of small hinge rotations and nominally nonrotating appendages. Sample problems and their solutions are presented to illustrate the utility of the computer programs.

Genetics Informs Precision Practice in the Diagnosis and Management of Pheochromocytoma.

PubMed

Neumann, Hartmut P H; Young, William; Krauss, Tobias; Bayley, Jean-Pierre; Schiavi, Francesca; Opocher, Giuseppe; Boedeker, Carsten; Tirosh, Amit; Castinetti, Frederic; Ruf, Juri; Beltsevich, Dmitry; Walz, Martin K; Groeben, Harald; Von Dobschuetz, Ernst; Gimm, Oliver; Wohllk, Nelson; Pfeifer, Marija; Lourenço, Delmar Muniz; Peczkowska, Mariola; Patocs, Attila; Ngeow, Joanne; Makay, Ozer; Shah, Nalini; Tischler, Arthur S; Leijon, Helena; Pennelli, Gianmaria; Villar Gómez de Las Heras, Karina; Links, Thera P; Bausch, Birke; Eng, Charis

2018-05-24

Although the authors of the present review have contributed to genetic discoveries in the field of pheochromocytoma research, we can legitimately ask whether these advances have led to improvements in the diagnosis and management of patients with pheochromocytoma. The answer to this question is an emphatic Yes! In the field of molecular genetics, the well-established axiom that familial (genetic) pheochromocytoma represents 10% of all cases has been overturned, with >35% of cases now attributable to germline disease-causing mutations. Furthermore, genetic pheochromocytoma can now be grouped into five different clinical presentation types in the context of the 10 known susceptibility genes for pheochromocytoma-associated syndromes. We now have the tools to diagnose patients with genetic pheochromocytoma, identify germline mutation carriers, and to offer gene-informed medical management including enhanced surveillance and prevention. Clinically, we now treat an entire family of tumors of the paraganglia, with the exact phenotype varying by specific gene. In terms of detection and classification, simultaneous advances in biochemical detection and imaging localization have taken place, and the histopathology of the paraganglioma tumor family has been revised by immunohistochemical-genetic classification by gene-specific antibody immunohistochemistry. Treatment options have also been substantially enriched by the application of minimally-invasive and adrenal-sparing surgery. Finally and most importantly, it is now widely recognized that patients with genetic pheochromocytoma/paraganglioma syndromes should be treated in specialized centers dedicated to the diagnosis, treatment, and surveillance of this rare neoplasm.

Knife blade as a facial foreign body.

PubMed

Gardner, P A; Righi, P; Shahbahrami, P B

1997-08-01

This case demonstrates the unpredictability of foreign bodies in the face. The retained knife blade eluded detection on two separate examinations. The essential components to making a correct diagnosis of a foreign body following a stabbing to the face include a thorough review of the mechanism of injury, a complete head and neck examination, a high index of suspicion, and plain radiographs of the face.

Phenotypic variability within the inclusion body spectrum of basophilic inclusion body disease and neuronal intermediate filament inclusion disease in frontotemporal lobar degenerations with FUS-positive inclusions.

PubMed

Gelpi, Ellen; Lladó, Albert; Clarimón, Jordi; Rey, Maria Jesús; Rivera, Rosa Maria; Ezquerra, Mario; Antonell, Anna; Navarro-Otano, Judith; Ribalta, Teresa; Piñol-Ripoll, Gerard; Pérez, Anna; Valldeoriola, Francesc; Ferrer, Isidre

2012-09-01

Basophilic inclusion body disease and neuronal intermediate filament inclusion disease (NIFID) are rare diseases included among frontotemporal lobar degenerations with FUS-positive inclusions (FTLD-FUS). We report clinical and pathologic features of 2 new patients and reevaluate neuropathologic characteristics of 2 previously described cases, including an early-onset case of basophilic inclusion body disease (aged 38 years) with a 5-year disease course and abundant FUS-positive inclusion bodies and 3 NIFID cases. One NIFID case (aged 37 years) presented with early-onset psychiatric disturbances and rapidly progressive cognitive decline. Two NIFID cases had later onset (aged 64 years and 70 years) and complex neurologic deficits. Postmortem neuropathologic studies in late-onset NIFID cases disclosed α-internexin-positive "hyaline conglomerate"-type inclusions that were positive with 1 commercial anti-FUS antibody directed to residues 200 and 250, but these were negative to amino acids 90 and 220 of human FUS. Early-onset NIFID had similar inclusions that were positive with both commercial anti-FUS antibodies. Genetic testing performed on all cases revealed no FUS gene mutations. These findings indicate that phenotypic variability in NIFID, including clinical manifestations and particular neuropathologic findings, may be related to the age at onset and individual differences in the evolution of lesions.

Is surgical decontamination definitive treatment of "body-packers"?

PubMed

Olmedo, R; Nelson, L; Chu, J; Hoffman, R S

2001-11-01

The current recommendations for body-packers are based on packet content, the presence of drug toxicity, or of bowel obstruction. Asymptomatic patients are usually treated with activated charcoal and whole bowel irrigation (WBI). Surgical removal of packets is advocated in symptomatic cocaine body-packers and in those with bowel obstruction. Currently, surgery is regarded as definitive. However, we report 2 body-packers who show the limitations of this technique. These cases show the importance of confirming the absence of drug packets in the gastrointestinal (GI) tract as the definitive end-point in the treatment of body-packers.

Serological levels of apoptotic bodies, sFAS and TNF in lupus erythematosus.

PubMed

Alecu, M; Coman, G; Alecu, S

In our study we have investigated the presence of apoptotic bodies, soluble FAS receptor and TNF (tumor necrosis factor) in three clinical forms of lupus erythematosus. Determinations were performed in attack period of: systemic lupus erythematosus (SLE) for 20 patients, 20 patients with subacute cutaneous lupus erythematosus (SCLE), 20 patients with chronic discoid lupus erythematosus (DLE). Determinations were performed by ELISA (for apoptotic bodies, kit Boehringer, normal values 400-800 mU), (for sFAS, kit R&D Systems, normal values 4500-17000 pg/ml) (for TNF, ELISA kit R&D Systems, normal values 0.4-3.6 pg/ml). Results in SLE: apoptotic bodies were increased in 16 cases (980-1030); sFAS in 18 cases (17000-24000 pg/ml) TNF was increased in all 20 cases (40-140 pg/ml). In SCLE with multiple cutaneous lesions and without internal organs disturbance the apoptotic bodies were increased in 10 cases (960-1030 pg/ml), sFAS in 9 cases (17000-22000 pg/ml), and TNF alpha in 9 cases. In DLE, apoptotic bodies were increased in 2 patients (980-1010 pg/ml), sFAS in 3 patients (17000-20000 pg/ml) and TNF in 2 patients (20-40 pg/mil). Investigated values were slightly correlated with immune parameters (anti dsDNA antibodies), but they were correlated with the presence of renal disturbances or extension of cutaneous lesions. We consider that the presence of increased apoptotic bodies as a result of peripheral mononuclear cells apoptosis appear as a nauto-limiting mechanism in a pathological immune response. The increase of sFAS in lupus patients serum might be interpreted as an alteration of apoptosis respectively a deficit in apoptosis which has as a first consequence the persistence of B and T lymphocytes, activated, in the pathogen immune response.

Body ownership promotes visual awareness

PubMed Central

Reingardt, Maria; Ehrsson, H Henrik

2017-01-01

The sense of ownership of one’s body is important for survival, e.g., in defending the body against a threat. However, in addition to affecting behavior, it also affects perception of the world. In the case of visuospatial perception, it has been shown that the sense of ownership causes external space to be perceptually scaled according to the size of the body. Here, we investigated the effect of ownership on another fundamental aspect of visual perception: visual awareness. In two binocular rivalry experiments, we manipulated the sense of ownership of a stranger’s hand through visuotactile stimulation while that hand was one of the rival stimuli. The results show that ownership, but not mere visuotactile stimulation, increases the dominance of the hand percept. This effect is due to a combination of longer perceptual dominance durations and shorter suppression durations. Together, these results suggest that the sense of body ownership promotes visual awareness. PMID:28826500

Height, waist circumference, body mass index, and body somatotype across the life course and risk of glioma.

PubMed

Cote, David J; Downer, Mary K; Smith, Timothy R; Smith-Warner, Stephanie A; Egan, Kathleen M; Stampfer, Meir J

2018-06-26

Recent studies have suggested height as a risk factor for glioma, but less is known regarding body mass index (BMI) or other anthropomorphic measures. We evaluated the association between body habitus and risk of glioma. We evaluated the association of measures of height, BMI, waist circumference, and somatotypes with risk of glioma in two prospective cohorts, the Nurses' Health Study and the Health Professionals Follow-Up Study. We documented 508 incident cases of glioma (321 glioblastoma [GBM]). In both cohorts, we found no significant association between adult BMI or waist circumference and risk of glioma, with pooled HR for BMI of 1.08 (95% CI 0.85-1.38 comparing ≥ 30 to < 25 kg/m 2 ) and for waist circumference of 1.05 (95% CI 0.80-1.37 highest vs. lowest quintile). Higher young adult BMI (at age 18 in NHS and 21 in HPFS) was associated with modestly increased risk of glioma in the pooled cohorts (pooled HR 1.35, 95% CI 1.06-1.72 comparing ≥ 25 kg/m 2 vs. less; HR 1.34 for women and 1.37 for men). Analysis of body somatotypes suggested reduced risk of glioma among women with heavier body types at all ages this measure was assessed (HRs ranging from 0.52 to 0.65 comparing highest tertile to lowest tertile), but no significant association among men. Height was associated with increased risk of glioma among women (HR 1.09, 95% CI 1.04-1.14 per inch), but not significantly among men. Within the 8 years prior to diagnosis, cases had no material weight loss compared to non-cases. All results were similar when limited to GBM. Adult BMI and waist circumference were not associated with glioma. Higher BMI at age 21 for men and at age 18 for women was modestly associated with risk in the pooled cohort. Based on body somatotypes, however, women with heavier body types during childhood and young adulthood may be at lower risk of glioma, although this association was not observed later in life with measurements of BMI. Greater height was associated with

A rare cause of hypertension in children: intrathoracic pheochromocytoma.

PubMed

Singh, Jaswinder; Rana, Sandeep Singh; Sharma, Rajeshwar; Ghai, Babita; Puri, G D

2008-07-01

Mediastinal pheochromocytomas account for only a small fraction of mediastinal tumors. Most commonly, these tumors are located in posterior mediastinum. Increasing number of cases of pheochromocytomas is being reported from middle mediastinum. Excision of mediastinal paraganglioma is often hazardous because of its rich blood supply and tendency to involve surrounding structures. It can be a big challenge to manage asymptomatic cases of pheochromocytoma intraoperatively. It is imperative that these patients receive adequate alpha adrenergic and if necessary beta adrenergic blockade. Adequate preoperative preparation with alpha and beta blockers may not prevent serious intraoperative hypertension. We report a case of posterior mediastinal pheochromocytoma which was biochemically active preoperatively. We review the presentation, diagnosis and management of intrathoracic pheochromocytomas including cardiac pheochromocytomas.

Correction of gynecomastia in body builders and patients with good physique.

PubMed

Blau, Mordcai; Hazani, Ron

2015-02-01

Temporary gynecomastia in the form of breast buds is a common finding in young male subjects. In adults, permanent gynecomastia is an aesthetic impairment that may result in interest in surgical correction. Gynecomastia in body builders creates an even greater distress for patients seeking surgical treatment because of the demands of professional competition. The authors present their experience with gynecomastia in body builders as the largest study of such a group in the literature. Between the years 1980 and 2013, 1574 body builders were treated surgically for gynecomastia. Of those, 1073 were followed up for a period of 1 to 5 years. Ages ranged from 18 to 51 years. Subtotal excision in the form of subcutaneous mastectomy with removal of at least 95 percent of the glandular tissue was used in virtually all cases. In cases where body fat was extremely low, liposuction was performed in fewer than 2 percent of the cases. Aesthetically pleasing results were achieved in 98 percent of the cases based on the authors' patient satisfaction survey. The overall rate of hematomas was 9 percent in the first 15 years of the series and 3 percent in the final 15 years. There were no infections, contour deformities, or recurrences. This study demonstrates the importance of direct excision of the glandular tissue over any other surgical technique when correcting gynecomastia deformities in body builders. The novice surgeon is advised to proceed with cases that are less challenging, primarily with patients that require excision of small to medium glandular tissue. Therapeutic, IV.

Supersonic full-potential methods for missile body analysis

NASA Technical Reports Server (NTRS)

Pittman, James L.

1992-01-01

Accounts are presented of representative applications to missile bodies of arbitrary shape of methods based on the steady form of the full potential equation. The NCOREL and SIMP full-potential codes are compared, and their results are evaluated for the cases of an arrow wing and a wing-body configuration. Attention is given to the effect of cross-sectional and longitudinal geometries. Comparisons of surface pressure and longitudinal force and moment data for circular and elliptic bodies have shown that the full-potential methods yielded excellent results in attached-flow conditions. Results are presented for a conical star body, waveriders, the Shuttle Orbiter, and a highly swept wing-body cruising at Mach 4.

[Body hair and advertising in French magazines].

PubMed

Héas, S; Bodin, D; Robène, L; Misery, L

2007-10-01

Sociological analysis of advertising reveals the currently operative body codes within a society, and more fundamentally, depicts the (ideal) relationships between men and women, between generations, and so on. To demonstrate that sports advertising based largely on trichological stereotypes. The idealised portrayal of human relations in advertisements (N=700) taken from French magazines was analysed by means of systematic coding and use of analytical software. This approach allowed characterisation of each advertisement in relation to the entire sample, with identification of significant elements (dominant colour, stature of models, setting, etc.), and determination of frequency of appearance and occurrence as well as testing of dependency relations. There were significant differences in the portrayal of men and women in advertisements. In the 700 advertisements in the series we examined, male subjects very often had short hair (231 cases, 33%) or shaved heads (33 cases, 4.7%) while women were shown with long hair, either free (80 cases) or tied (73 cases). Women with short hair were rarely portrayed (4.3%), as were men with long hair (42 cases, 6%). Above all, with the exception of eyebrows and eyelashes, no other body hair was seen in male and female athletic figures in 238 advertisements, being visible in only 60 cases (8.6%). Facial stubble, and more particularly beards and moustaches, was fairly infrequent, despite the omnipresence of male models. The majority of advertising situations involving sporting figures show clear stereotyping. Body hair is a pertinent pointer to understanding of contemporary sports models. A clear overall male/female distinction was present throughout. Men were presented in these adverts as active figures, leaders, etc. while women tended to be passive, spectators, and in some cases, admiring onlookers. A degree of confusion between genders was noticeable as a result of the shaving and depilation trends currently in vogue in the

Hand and body radiation exposure with the use of mini C-arm fluoroscopy.

PubMed

Tuohy, Christopher J; Weikert, Douglas R; Watson, Jeffry T; Lee, Donald H

2011-04-01

To determine whole body and hand radiation exposure to the hand surgeon wearing a lead apron during routine intraoperative use of the mini C-arm fluoroscope. Four surgeons (3 hand attending surgeons and 1 hand fellow) monitored their radiation exposure for a total of 200 consecutive cases (50 cases per surgeon) requiring mini C-arm fluoroscopy. Each surgeon measured radiation exposure with a badge dosimeter placed on the outside breast pocket of the lead apron (external whole body exposure), a second badge dosimeter under the lead apron (shielded whole body exposure), and a ring dosimeter (hand exposure). Completed records were noted in 198 cases, with an average fluoroscopy time of 133.52 seconds and average cumulative dose of 19,260 rem-cm(2) per case. The total measured radiation exposures for the (1) external whole body exposure dosimeters were 16 mrem (for shallow depth), 7 mrem (for eye depth), and less than 1 mrem (for deep depth); (2) shielded whole body badge dosimeters recorded less than 1 mrem; and (3) ring dosimeters totaled 170 mrem. The total radial exposure for 4 ring dosimeters that had registered a threshold of 30 mrem or more of radiation exposure was 170 mrem at the skin level, for an average of 42.5 mrem per dosimeter ring or 6.3 mrem per case. This study of whole body and hand radiation exposure from the mini C-arm includes the largest number of surgical cases in the published literature. The measured whole body and hand radiation exposure received by the hand surgeon from the mini C-arm represents a minimal risk of radiation, based on the current National Council on Radiation Protection and Management standards of annual dose limits (5,000 mrem per year for whole body and 50,000 mrem per year to the extremities). Copyright © 2011 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

[The elderly subject and social violence: the body as a factor in communication].

PubMed

Tamburro, G A; Di Sciascio, G; De Giglio, F

1992-01-01

The social inferiority of an elderly person stems from his physical inferiority--actual or alleged but always possible. And yet this "inferior" body is paradoxically hypertrophic: at first it masks the person, then takes up its space until it negates it. Hence, an elderly person is not only a body but a lonely body. In his relations with other people, his body becomes a receiver, a receptacle and a source of communication. Social violence underlies relations with elderly people: such violence may be deceptive, widespread and continuous or, on the other hand, manifest, episodic and conspicuous. In the first case it may be a way of assigning subalternate roles to them in relation to the efficiency expected of them or a way of mythologizing their condition as one of pseudo-happiness. In the second case is generally relates to assaults, thefts, bag-snatching, etc. In any case, however, communication with them entails violence: their body perceives this and reacts to it. This is why their body's language is violent: their body cries out, it is stunned and it is acted upon (even--and unavoidably--in relations with a therapist).

Between My Body and My "Dead Body": Narratives of Coma.

PubMed

Meoded Danon, Limor

2016-01-01

This article is based on narrative research that focuses on corporeal experience during coma and during the rehabilitation process. Seventeen participants from different areas of Israel who had been in various kinds of coma states reveal what the corporeal experience of coma is. The participants are divided into three types of narrative protagonists--"dead-alive," "rational," and "emissaries." Each of the participants redefined the boundaries of the body, especially in cases when they spoke of experiences they did not understand as corporeal, for example, out-of-body experiences, near-death experiences, or experiences of being between the earthly and unearthly. Their struggle to find suitable words to tell their coma stories emphasizes these boundaries between experiencing and telling, which crossed the normative discursive border of the medical establishment and illustrates the ambiguous nature of human existence. © The Author(s) 2015.

Imagery Rescripting for Body Dysmorphic Disorder: A Multiple-Baseline Single-Case Experimental Design.

PubMed

Willson, Rob; Veale, David; Freeston, Mark

2016-03-01

Individuals with body dysmorphic disorder (BDD) often experience negative distorted images of their appearance, and research suggests these may be linked to memories of adverse events such as bullying or teasing. This study evaluates imagery rescripting (ImR) as an intervention for BDD. In this article, we present a multiple-baseline single-case experimental design testing imagery rescripting as a brief, stand-alone intervention, with six individuals with BDD that related to aversive memories. The impact of the intervention was assessed by self-reported daily measures of symptom severity (preoccupation with appearance, appearance-related checking behaviors, appearance-related distress, and strength of belief that their main problem is their appearance) and standardized clinician ratings of BDD severity (Yale-Brown Obsessive Compulsive Scale modified for BDD). Four out of six of the participants responded positively to the intervention, with clinically meaningful improvement in symptomatology. Overall response was rapid; improvements began within the first week post-ImR intervention. From a small sample it is cautiously concluded that imagery rescripting may show promise as a module in cognitive-behavioral therapy for BDD, and is worthy of further investigation. Copyright © 2015. Published by Elsevier Ltd.

Foreign bodies in a pediatric emergency department in South Africa.

PubMed

Timmers, Maarten; Snoek, Kitty G; Gregori, Dario; Felix, Janine F; van Dijk, Monique; van As, Sebastian A B

2012-12-01

Foreign body-related pediatric trauma has a high incidence, but studies with large data sets are rare and typically stem from Western settings. The aim of this study was to identify characteristics of foreign body-related trauma in children treated at our trauma unit in South Africa. In this retrospective study, we analyzed all foreign body-related trauma admissions from 1991 to 2009. We collected detailed data including age, sex, type of foreign body, injury severity, and anatomical location of the foreign body. We analysed 8149 cases. Marginally more boys (54.9%) than girls were involved. The overall median age was 3 years (interquartile range, 2-6 years); 78.8% were younger than 7 years. The predominant anatomical sites were the respiratory tract and the gastrointestinal tract (39.1%); ears (23.9%); nose (19.4%); and extremities (8.8%). The commonest objects were coins (20.8 %), (parts of) jewelry (9.5%), and food (8.7%). Three quarters (74.5%) of patients presented between 1 and 2 hours after the injury (median, 1 hour). A total of 164 cases (2.0%) were marked as possible child abuse; 17 cases were filed as confirmed child abuse. Preventive parent education programs targeting foreign body-related injury should mainly focus on both sexes younger than 7 years. Parents should be taught to keep small objects out of reach of young children, especially coins, because these most often result in a trauma unit visit.

Non-verbal Full Body Emotional and Social Interaction: A Case Study on Multimedia Systems for Active Music Listening

NASA Astrophysics Data System (ADS)

Camurri, Antonio

Research on HCI and multimedia systems for art and entertainment based on non-verbal, full-body, emotional and social interaction is the main topic of this paper. A short review of previous research projects in this area at our centre are presented, to introduce the main issues discussed in the paper. In particular, a case study based on novel paradigms of social active music listening is presented. Active music listening experience enables users to dynamically mould expressive performance of music and of audiovisual content. This research is partially supported by the 7FP EU-ICT Project SAME (Sound and Music for Everyone, Everyday, Everywhere, Every Way, www.sameproject.eu).

Management of ingested foreign bodies in childhood and review of the literature.

PubMed

Arana, A; Hauser, B; Hachimi-Idrissi, S; Vandenplas, Y

2001-08-01

The management of ingested foreign bodies in children is not standardised. During a 15-year period, we recorded 325 consecutive paediatric cases of accidental ingestion of foreign bodies or with symptoms suggesting oesophageal obstruction presented at the emergency department or the paediatric gastroenterology unit. The foreign bodies that had to be removed were, in decreasing order of frequency: coins, toy parts, jewels, batteries, sharp materials such as needles and pins, fish and chicken bones, and "large" amounts of food. Only 54% of the patients had transient symptoms at the moment of ingestion, such as retrosternal pain, cyanosis and dysphagia. A minority (28, 9%) of foreign bodies could be removed with a McGill forceps; 65 (20%) were removed with a magnet probe. Endoscopic removal was performed in 82 cases (25%). In the majority of cases (150, 46%) natural elimination occurred. The outcome of all patients was uneventful. Recommendations for management of children presenting with a history of suspected accidental ingestion of a foreign body for the community paediatrician are proposed.

Association between cytochrome P450 2D6 polymorphisms and body fluid methamphetamine concentrations in Japanese forensic autopsy cases.

PubMed

Matsusue, Aya; Ikeda, Tomoya; Tani, Naoto; Waters, Brian; Hara, Kenji; Kashiwagi, Masayuki; Takayama, Mio; Ikematsu, Natsuki; Kubo, Shin-Ichi; Ishikawa, Takaki

2018-05-18

Methamphetamine (MA) is an illicit stimulant that affects the central nervous system. Cytochrome P450 2D6 (CYP2D6) plays an important role in MA metabolism. Numerous allelic variants confer substantial variation in CYP2D6 activity among individuals. In the present study, we examined the frequencies of CYP2D6 alleles, including CYP2D6*1, *2, *4, *5, *10, *14A, *14B, *18, and *36, and multiplication, in 82 forensic autopsy cases of MA abusers and 567 autopsy cases in which MA was not detected (controls). Ultrarapid metabolizer (UM), extensive metabolizer (EM), intermediate metabolizer (IM), and poor metabolizer (PM) phenotypes were predicted from CYP2D6 genotypes. Of MA abusers, 64 subjects were predicted to be EM, 17 were IM, and 1 was UM. No MA abuser had the predicted PM phenotype. No significant differences in CYP2D6 phenotype frequencies were found between MA abusers and controls. MA and amphetamine (AMP) concentrations were measured in the right heart blood, left heart blood, peripheral external iliac blood, urine, pericardial fluid, and bone marrow of MA abusers. MA concentrations in urine and bone marrow were significantly higher in IM than in EM. AMP concentration was not associated with CYP2D6 phenotype in any body fluid. These results suggest that the MA concentration in body fluids is influenced by CYP2D6 phenotypes in the Japanese population. Copyright © 2018 Elsevier B.V. All rights reserved.

Tracheobronchial foreign body aspiration in children.

PubMed

Burton, E M; Brick, W G; Hall, J D; Riggs, W; Houston, C S

1996-02-01

In this retrospective study, we reviewed the demographic and radiographic findings of 155 children with bronchoscopy-proven tracheobronchial foreign body aspiration (FBA). Two thirds of the patients were male, and most were children between 1 and 2 years of age. An aspirated peanut accounted for one third of all cases. Foreign body location was distributed nearly evenly to the right and left primary bronchi; tracheal foreign body was noted in 16 patients. The most frequent symptoms of FBA were cough (85 patients) and wheezing (60 patients). Although most patients were seen within 1 day of aspiration, 30 patients had symptoms that lasted at least 1 week before diagnosis. The most common radiographic findings were unilateral or segmental hyperlucency (59) or atelectasis (38). The trachea was the site of the foreign body in one half of children with a normal chest radiograph and FBA.

[Smuggling of illegal drugs by body suffers].

PubMed

Aksnes, Tonje Amb; Jacobsen, Dag

2004-07-01

Body packers are persons who smuggle illegal drugs by swallowing condoms or plastic cylinders containing such substances. Body stuffers are drug dealers or drug abusers who swallow illegal drugs in an effort to conceal evidence during an arrest or in fear of being arrested. We report four cases and discuss management. Asymptomatic body packers may be managed conservatively with laxatives and water-soluble contrast medium. This method allows an accurate follow-up with abdominal radiography. If patients develop abdominal pain or signs and symptoms of intoxication, surgical intervention should be considered. Both types of patients should be monitored carefully.

Calculation of flexoelectric deformations of finite-size bodies

NASA Astrophysics Data System (ADS)

Yurkov, A. S.

2015-03-01

The previously developed approximate theory of flexoelectric deformations of finite-size bodies has been considered as applied to three special cases: a uniformly polarized ball, a uniformly polarized circular rod, and a uniformly polarized thin circular plate of an isotropic material. For these cases simple algebraic formulas have been derived. In the case of the ball, the solution is compared with the previously obtained exact solution.

Investigating the Relation between Women's Body Image and Unconsummated Marriage.

PubMed

Hosseini, Sara; Noroozi, Mahnaz; Montazery, Gita

2017-01-01

Unconsummated marriage is considered to be one of the complicated sexual issues that lead to multiple complications and problems for couples as well as the society. It is thought that this disorder is more common in traditional cultures and some religions such as Islam, Hinduism, and Judaism. The aim of this study was to determine the relation between women's body image and unconsummated marriage. This was a case-control study which was conducted among 50 women who had an unconsummated marriage (case group) and 100 women who had a consummated marriage (control group) in Isfahan, Iran during 2015-2016. Data were collected using the Multidimensional Body-Self Relations Questionnaire (MBSRQ). The data were analyzed using descriptive and inferential statistical tests. The total score of body image and all its components had no significant difference between both the groups of the case and the control ( P > 0.05). Considering that no relation was found between body image and unconsummated marriage and the religious culture of the Iranian society with conservative sexual norms, investigating unconsummated marriage with emphasis on cultural factors is recommended. Hence, such sexual disorders would be avoided and the number of affected people and challenges can be decreased.

Food foreign body injuries.

PubMed

Sebastian van As, Arjan B; Yusof, Abdullah M; Millar, Alastair J W