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Judith Sebestyen VanSickle, Tarak Srivastava, Uri S Alon
The calcium-sensing receptor (CaSR) plays an important role in the homeostasis of serum ionized calcium by regulating parathyroid hormone (PTH) secretion and tubular calcium handling. Calcimimetics, which act by allosteric modulation of the CaSR, mimic hypercalcemia resulting in suppression of PTH release and increase in calciuria. Mostly used in children to treat secondary hyperparathyroidism associated with advanced renal failure, we have shown that calcimimetics can also be successfully used in children with bone and mineral disorders in which elevated PTH plays a detrimental role in skeletal pathophysiology in the face of normal kidney function...
March 19, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Kenji Yorita, Kazuya Tsuji, Yoko Takano, Naoto Kuroda, Kei Sakamoto, Kaoru Arii, Yukio Yoshimoto, Kimiko Nakatani, Satoshi Ito
BACKGROUND: Acantholytic squamous cell carcinoma (ASQCC), histologically characterized by intercellular bridge loosening, is recognized as a rare variant of squamous cell carcinoma (SQCC). ASQCC may demonstrate a worse prognosis than conventional SQCC. Pulmonary ASQCC is particularly rare; its biological behavior and prognostic data have not been reported. CASE PRESENTATION: We report the clinical and autopsy findings of a 71-year-old Japanese man with pulmonary ASQCC...
March 16, 2018: BMC Cancer
Luis Malpica, Agustin Pimentel, Isildinha M Reis, Eduardo Gotuzzo, Lazaros Lekakis, Krishna Komanduri, Thomas Harrington, Glen N Barber, Juan C Ramos
Adult T-cell leukemia/lymphoma (ATLL) is a fatal disease caused by human T-cell leukemia virus type 1 (HTLV-1). We retrospectively analyzed 195 patients with ATLL (lymphomatous n = 96, acute n = 80, unfavorable chronic n = 7, chronic n = 5, smoldering n = 3, and unclassified n = 4) diagnosed between 1987 and 2016 (median age 52 years, 77% Afro-Caribbean). Hypercalcemia was associated with acute ATLL (65%, vs 23% lymphomatous) ( P = .012). The median survival for patients treated with modern therapies between 2000 and 2016 was 4...
March 27, 2018: Blood Advances
Mirza Sultan Ahmad, Danial Ahmad, Naila Medhat, Syed Aizaz Hussain Zaidi, Hadia Farooq, Sair Ahmad Tabraiz
OBJECTIVE: To ascertain the frequency of electrolyte abnormalities in patients of probable and culture-proven sepsis on admission and assess any association of electrolyte disorders with mortality. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: NICU, Fazle-Omar Hospital, Rabwah, from October 2015 to September 2016. METHODOLOGY: All neonates with the diagnosis of probable and culture-proven sepsis during the study period admitted in NICU, Fazle-Omar Hospital, were included in the study...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Muhammad Shuja, Ayman A Elghazaly, Asif Iqbal, Reham Mohamed, Amal Marie, Mutahir A Tunio, Moamen M Aly, Ali Balbaid, Mushabbab Asiri
Introduction Bone metastasis (BM) is a major complication of many solid tumors like breast, prostate, lung and renal cancers. BM leads to serious sequelae of pain, fractures, spinal cord compression and hypercalcemia. Radiotherapy has an established role in relieving pain caused by BM. Worldwide different radiotherapy schedules are being used for BM. The aim of this study is to determine the efficacy of single fraction palliative radiotherapy for painful bone metastases. Methods Between April 2014 and April 2017, single fraction radiotherapy was used to treat 73 patients in our institution...
January 8, 2018: Curēus
Elvira Larqué, Eva Morales, Rosaura Leis, José E Blanco-Carnero
BACKGROUND: To what extent does the circulating 25-hydroxyvitamin D (25[OH]D) concentration help to meet the physiological needs of humans is an ongoing subject of debate. Remaining unexposed to the sun to reduce melanoma cancer risk, current lifestyle with less out door activities, and increasing obesity rates, which in turn increases the storage of vitamin D in the adipose tissue, are presumably factors that contribute to the substantial upsurge in the prevalence of vitamin D deficiency in humans...
March 13, 2018: Annals of Nutrition & Metabolism
Satoshi Kondo, Hiroyuki Kakihata, Yosuke Nishida, Yuko Furuno, Yumiko Kobayashi, Hidehiro Tabata, Makoto Nomura
We conducted a post-marketing observational study to investigate the safety and effectiveness of eldecalcitol for the treatment of osteoporosis in a Japanese clinical setting. The observation period was 12 months for women and 36 months for men. The final results for the female patients have already been published. In this article, the final results for the male patients are reported. A total of 470 male osteoporosis patients were enrolled. The safety analysis set included 431 patients (mean age, 76.8 years; mean ± SD follow-up period, 631...
March 12, 2018: Journal of Bone and Mineral Metabolism
Wei Ling Lau, Yoshitsugu Obi, Kamyar Kalantar-Zadeh
Secondary hyperparathyroidism develops in CKD due to a combination of vitamin D deficiency, hypocalcemia, and hyperphosphatemia, and it exists in nearly all patients at the time of dialysis initiation. There is insufficient data on whether to prefer vitamin D analogs compared with calcimimetics, but the available evidence suggests advantages with combination therapy. Calcium derangements, patient adherence, side effects, and cost limit the use of these agents. When parathyroid hormone level persists >800 pg/ml for >6 months, despite exhaustive medical interventions, monoclonal proliferation with nodular hyperplasia is likely present along with decreased expression of vitamin D and calcium-sensing receptors...
March 9, 2018: Clinical Journal of the American Society of Nephrology: CJASN
Kadie M Anderson, Albert Lewandowski, Patricia M Dennis
An 8 yr, intact male red-rumped agouti ( Dasyprocta leporina) was evaluated for weight loss. Examination revealed poor body condition, hypercalcemia, elevated serum 25-hydroxyvitamin D, metastatic calcification of soft tissues, and hyperechoic kidneys. The diet, formulated for laboratory rodents, contained elevated levels of vitamin D3 . Histopathology from a female conspecific that died 5 mo prior identified dystrophic mineralization and nephrosclerosis, suggestive of a vitamin D3 toxicity. The male agouti responded well to a dietary reduction in vitamin D3 and calcium...
March 2018: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Harrison T Supnick, Rodica P Bunaciu, Andrew Yen
Differentiation therapy is used in cancer treatment. Epidemiologic studies showed that higher vitamin D levels are associated with reduced cancer risks. However, the therapeutic doses needed for differentiation are accompanied by hypercalcemia and intolerable pathological sequelae. In the present work we evaluated if RRD-251, a small-molecule, can enhance vitamin D3-induced differentiation of leukemic cells, in the hope of decreasing the needed vitamin D3-dose. We demonstrate that RRD-251 enhances vitamin D3-induced differentiation of leukemic cells, the enrichment of the c-Raf kinase in the nucleus, the binding of nuclear c-Raf to GSK-3, increased phosphorylation of GSK-3 ser 21/9 inhibitory sites, and the binding of GSK-3 to VDR, where GSK-3 inhibition is known to enhance transcriptional activation by VDR...
February 9, 2018: Oncotarget
Masood Umer, Yasir Mohib, Muhammed Atif, Muhammad Nazim
Background: Renal cell carcinoma account for 3% of all cancers, with peak incidence between 60 and 70 years of age predominantly affecting male population. Renal carcinoma is the most common malignancy of kidney constitutes for 80-90% of renal neoplasm with an overall 45% five years survival rate. Majority are diagnosed incidentally during investigation for other disease process of abdomen. Classical triad of gross hematuria, pain and palpable mass in abdomen is rare accounting to only 6-10%...
March 2018: Annals of Medicine and Surgery
Kohzo Takebayashi, Atsushi Ujiie, Mio Kubo, Sho Furukawa, Mototaka Yamauchi, Hiroyuki Shinozaki, Tatsuhiko Suzuki, Rika Naruse, Kenji Hara, Takafumi Tsuchiya, Toshihiko Inukai
We describe a 58-year-old man with a malignant melanoma metastasis to the liver. After initiation of nivolumab therapy, he developed destructive thyroiditis and subsequently simultaneous isolated adrenocorticotropic hormone (ACTH) deficiency and severe hypercalcemia. Although isolated ACTH deficiency and hypercalcemia due to nivolumab therapy are both rare occurrences, these conditions can often cause a severe clinical course accompanied by a disturbance of consciousness. Therefore, clinicians should pay attention to these possible side effects of nivolumab if the patients have clinical symptoms, such as fatigue and a disturbance of consciousness...
April 2018: Journal of Clinical Medicine Research
J Anoun, F Ben Fredj Ismail, A Mzabi, S Dadaa, I Ben Hassine, M Karmani, B Mrad, H Mhiri, A Rezgui, C Laouani Kechrid
Arteriosclerosis is an alteration of the blood vessels whose walls calcify, lose their elasticity and thicken. The result is a decrease in circulating blood flow and ischemic manifestations. Calcification of the arteries is a physiological phenomenon in the elderly but young subjects may also be affected. Indeed, certain risk factors can favor the formation of plaques at the level of the vessels. These include classic cardiovascular risk factors, as well as systemic inflammatory diseases, connective tissue diseases, chronic hypercalcemia… We report the case of a 26-year-old female patient with diffuse arteriosclerosis discovered accidentally, and whose exhaustive etiological investigation remained negative...
March 2, 2018: Annales de Cardiologie et D'angéiologie
Michael J Licata, Murali Janakiram, Stephanie Tan, Yanan Fang, Urvi A Shah, Amit K Verma, Yanhua Wang
Adult T-cell leukemia-lymphoma (ATLL) is caused by human T-cell lymphotropic virus type 1 (HTLV-1) and little is known about ATLL endemic to the Caribbean basin and Latin America, designated as western ATLL (W-ATLL). Due to extensive systemic involvement and nonspecific clinical presentation, the initial diagnosis in this cohort can be very challenging. We have diagnosed 60 patients with W-ATLL over a 14-year period. ATLL involves the peripheral blood, bone marrow, and cerebrospinal fluid (CSF) in 98, 87, and 52% cases, respectively; while lymphadenopathy, pulmonary infiltrates, splenomegaly, and hepatomegaly was present in 90, 82, 48, and 45% patients, respectively...
May 2018: Leukemia & Lymphoma
Pamela Trejo, Frank Rauch, Leanne Ward
Children with osteogenesis imperfecta (OI) type VI often have high fracture rates despite the current standard treatment with bisphosphonates. Subcutaneous injections of denosumab have been proposed as an alternative treatment approach, but safety data on denosumab in children are limited. Here we describe fluctuations in bone and mineral metabolism during denosumab treatment in four children with OI type VI who started denosumab (basic protocol: 1 mg per kg body mass every 3 months) between 1.9 and 9.0 years of age, after having received intravenous bisphosphonates previously...
March 1, 2018: Journal of Musculoskeletal & Neuronal Interactions
Adriana Handra-Luca, Frederique Tissier
Parathyroid cysts are rare, more frequently functional. Here we report 2 cases of infracentimetric parathyroid cysts identified in parathyroid adenomas resected for hyperparathyroidemia and hypercalcemia. Chronic parathyroiditis was associated. Chromogranin and E-cadherin/uvomorulin were expressed heterogeneously in the cyst lining and in parathyroid cells. In one of the cases, CD10 and CD56 were expressed by the luminal membrane of parathyroid cells. CD31 was expressed in interparathyroid cell monocyte/macrophage membrane as well as CD68, several cells showing also CD10 and/or CD56 expression...
September 28, 2017: Pathology, Research and Practice
Idanna Innocenti, Davide Rossi, Giulio Trapè, Francesco Autore, Luigi Maria Larocca, Vincenzo Gomes, Michaela Cerri, Paolo Falcucci, Simona Sica, Gianluca Gaidano, Luca Laurenti
Richter syndrome, a transformation of chronic lymphocytic leukemia (CLL) into a diffuse large B-cell lymphoma, is a rare complication of patients treated with chemo-immunotherapy. Richter syndrome might be both clonally related or unrelated to the underlying CLL and often showed mutations of the TP53 and NOTCH1 genes. Recently, ibrutinib was approved for patients with relapsed/refractory CLL or for untreated CLL patients with del 17p or TP53 mutation. The clinical picture, pathology, and genetics of Richter transformation after IBR treatment are largely unknown...
February 27, 2018: Hematological Oncology
Agazi Gebreselassie, Alem Mehari, Rahel Dagne, Firehiwet Berhane, Angesom Kibreab
RATIONALE: The usual presentation of sarcoidosis is hilar adenopathy, pulmonary reticular opacities, skin, joint, or eye lesions. Pancreatic involvement is unusual and hypercalcemic pancreatitis as initial manifestation is very rare. PATIENT CONCERNS: We present a case that presented with 1-day history of vomiting, diffuse abdominal pain, and altered mental status. DIAGNOSES: Initial investigations showed highly elevated calcium levels, acute pancreatitis, and kidney failure...
January 2018: Medicine (Baltimore)
Caroline M Gorvin, Angela Rogers, Michelle Stewart, Anju Paudyal, Tertius A Hough, Lydia Teboul, Sara Wells, Steve Dm Brown, Roger D Cox, Rajesh V Thakker
The adaptor protein-2 sigma subunit (AP2σ), encoded by AP2S1 , forms a heterotetrameric complex, with AP2α, AP2β, and AP2μ subunits, that is pivotal for clathrin-mediated endocytosis, and AP2σ loss-of-function mutations impair internalization of the calcium-sensing receptor (CaSR), a G-protein-coupled receptor, and cause familial hypocalciuric hypercalcemia type-3 (FHH3). Mice with AP2σ mutations that would facilitate investigations of the in vivo role of AP2σ, are not available, and we therefore embarked on establishing such mice...
May 2, 2017: JBMR Plus
Fredrick Venter, Arash Heidari, Macsen Viehweg, Mark Rivera, Piruthiviraj Natarajan, Everardo Cobos
Low-risk human papillomavirus types 6 and 11 can manifest as giant condylomata acuminata (GCA) of Buschke-Lowenstein. Up to 50% of GCA can slowly progress over years to fungating, invasive tumors. The malignant potential is attributed to unique immune evading abilities of the human papillomavirus. A 42-year-old male presented with pain and foul-smelling discharge from his genital warts. The histopathological examination of the mass showed invasive squamous cell carcinoma, and it was associated with paraneoplastic hypercalcemia...
January 2018: Journal of Investigative Medicine High Impact Case Reports
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