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Renal and Osteodystrophy

Yi-Chou Hou, Chien-Lin Lu, Kuo-Cheng Lu
As the GFR loss aggravates, the disturbed mineral metabolism worsens the bone microstructure and remodelling - scenario, which is known as CKD-mineral bone disease (MBD). CKD-MBD is characterized by : (i) abnormal metabolism of calcium, phosphorus, parathyroid hormone (PTH), or vitamin D; (ii) abnormalities in bone turnover, mineralization, volume linear growth or strength; (iii) soft-tissue calcifications, either vascular or extra-osseous. Uremic vascular calcification and osteoporosis are the most common complications related to CKD-MBD...
October 2018: Nephrology
Yogendranath Purrunsing, Jingjing Zhang, Ying Cui, Wei Liu, Yi Xu, Xunning Hong, Changying Xing, Xiaoming Zha, Ningning Wang
Secondary hyperparathyroidism (SHPT) is a long-term complication of chronic kidney disease-mineral and bone disorder (CKD-MBD). SHPT is characterized by hyperplasia of the parathyroid glands and abnormal secretion of parathyroid hormones (PTH), calcium and phosphorous metabolic disorders, renal osteodystrophy, vascular and soft tissue calcification, malnutrition, and other multiple system complications, which can seriously affect the quality of life of the patient and increase the risk of cardiovascular disease and mortality rate...
July 2018: JBMR Plus
Mehwish Gilani, Asif Ali Memon, Naveed Asif, Nida Basharat
Pseudohypoparathyroidism (PHP) is an extremely rare group of disorders. It is a spectrum of disorders caused by end organ resistance to parathyroid hormone (PTH) and is represented by impaired signalling that activates cAMP dependent pathways via alpha subunit of G-protein (GS). It is characterised by hypocalcemia, hyperphosphatemia, raised PTH levels due to insensitivity to biological activity of PTH, and normal renal function tests. We describe a case of 10-year girl who presented with fear of falling down from heights...
September 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Rakesh John, Prasoon Kumar, Sameer Aggarwal, Rajesh Kumar Rajnish, Saurabh Agarwal, Kuldeep Vatsyan
Introduction: Bilateral, simultaneous, non-traumatic, pathological femur neck fractures due to renal osteodystrophy (RO) in chronic renal failure cases are uncommon but are increasingly being reported in the literature. Seizure episodes due to uremic encephalopathy could lead to such fractures. Case Reports: We describe three cases of patients with end-stage renal disease and RO, who presented with bilateral, non-traumatic femur neck fractures after episodes of grand mal con-vulsions...
March 2018: Journal of Orthopaedic Case Reports
Wouter A M Broos, Maurits Wondergem, Friso M van der Zant, Remco J J Knol
A 27-year-old man, with a history of chronic renal failure due to obstructive uropathy, treated with hemodialysis and awaiting kidney transplantation, presented with tertiary hyperparathyroidism. Despite regulation of phosphate and calcium and treatment with cinacalcet, the serum parathyroid hormone levels remained elevated. The patient was referred for parathyroidectomy, and a F-fluorocholine PET/CT was acquired for preoperative planning. Besides visualization of the hyperfunctioning parathyroid glands, the scan also showed a typical picture of renal osteodystrophy, a finding on F-fluorocholine PET/CT that has not been reported before...
October 2018: Clinical Nuclear Medicine
Toshifumi Sugatani
The high cardiovascular mortality associated with chronic kidney disease (CKD) is caused in part by the CKD-mineral bone disorder (CKD-MBD) syndrome. The CKD-MBD consists of skeletal, vascular and cardiac pathology caused by metabolic derangements produced by kidney disease. The prevalence of osteopenia/osteoporosis resulting from the skeletal component of the CKD-MBD, renal osteodystrophy (ROD), in patients with CKD exceeds that of the general population and is a major public health concern. That CKD is associated with compromised bone health is widely accepted, yet the mechanisms underlying impaired bone metabolism in CKD are not fully understood...
August 23, 2018: International Journal of Molecular Sciences
Melani R Custodio, Rosilene M Elias, Wagner D Velasquez, Luciene M Dos Reis, Ivone B Oliveira, Rosa M A Moysés, Aluizio B Carvalho, Vanda Jorgetti
PURPOSE: Bone biopsy defines classical diseases that constitute the renal osteodystrophy. There is a recent concern regarding other histological findings that are not appreciated by using the turnover, mineralization, and volume (TMV) classification. Iron (Fe) overload has been considered a new challenge and the real significance of the presence of this metal in bones is not completely elucidated. Therefore, the main goal of the current study was to not only to identify bone Fe, but also correlate its presence with demographic, and biochemical characteristics...
October 2018: International Urology and Nephrology
Harjit Pal Bhattoa
With an aging population, there is a marked increase in prevalence of metabolic bone diseases, especially osteoporosis. Perhaps the most dreaded complication of metabolic bone disease, fractures typically impose a huge burden on the ailing body and are associated with high co-morbidity and mortality. The consequent public health and socioeconomic burden warrant timely diagnosis, treatment and follow-up of these disorders. Knowing the limitations of radiological techniques, biochemical markers of bone turnover measurements come handy since the changes in their levels readily reflect bone physiology...
July 2018: EJIFCC
Radhika R Narla, Susan M Ott
Sex hormones act in multiple ways to maintain a strong skeleton. In men, estrogen regulates cortical bone turnover, but testosterone maintains trabecular turnover. In normal men, sex hormone-binding protein is an independent risk factor for fractures. This led Aleksova and colleagues to measure the sex hormones and their binding protein in men receiving dialysis. Both higher sex hormone-binding globulin and higher total testosterone were associated with prevalent nonvertebral fractures, adding another layer of complexity to renal osteodystrophy...
August 2018: Kidney International
Sara Shakeri, Soroush Zarehparvar Moghadam, Ramin Sadeghi, Narjess Ayati
Sagliker syndrome is a rare form of renal osteodystrophy resulted from untreated secondary hyperparathyroidism. It is described by severe skeletal deformities, high level of PTH in patients with chronic renal failure, and deformed face. This paper reports a 44-year-old male patient with the mentioned characteristics. In addition to the unique clinical features, high levels of ALP and PTH hormones encouraged us to search for syndrome-like a disease, which clinically and paraclinically matched the Sagliker syndrome...
2018: Asia Oceania Journal of Nuclear Medicine & Biology
Magdalena Stârcea, Cristina Gavrilovici, Andra Elsayed, Mihaela Munteanu, Vasile Valeriu Lupu, Elena Cojocaru, Ingrith Miron, Lucian Miron
RATIONALE: Though to be rare, calcific uremic arteriolophathy (CUA) is an ectopic calcification entity causing pain and disabilities in patients with chronic renal insufficiency, thus increasing the morbidity and mortality. PATIENT CONCERN: We report a case of four years old boy admitted with acute respiratory failure. Physical examination revealed: irritability, purple subcutaneous hard nodules, tachypnea, dry spasmodic cough, respiratory rate 45/min, heart rate 110/min, blood pressure 100/60 mmHg, with normal heart sounds, no murmurs, hepatomegaly with hepato-jugular reflux...
July 2018: Medicine (Baltimore)
Arti Bhan, Shijing Qiu, Sudhaker D Rao
With the widespread use of measurement of bone mineral density to detect, diagnose, and monitor therapy in the management of osteoporosis, bone histomorphometry has largely been relegated to research settings and academic pursuits. However, bone density measurement cannot distinguish between osteoporosis and other metabolic bone disorders such as different types of osteomalacia, osteitis fibrosa, renal osteodystrophy, hypophosphatasia, and Paget's disease of bone. Furthermore, bone density test cannot tell us anything about microarchitecture of bone, tissue level dynamics, bone cellular activity, bone mineralization and bone remodeling, understanding of which is essential to make a specific diagnosis of a suspected metabolic bone disease, to evaluate beneficial (or adverse) effects of various therapies, treatment (medical or surgical) decisions in hyperparathyroid states...
June 2018: Bone Reports
Soichi Hirashima, Akihiko Miyawaki, Kotaro Oho, Yawaka Shitozawa, Motohiro Arai, Norihiko Furuta, Ryoichi Oya
Peritoneal dialysis can be performed at home, and the transfer of solutes in the blood and other body fluids is slow compared to hemodialysis, reducing the load on the circulatory organs and lessening the frequency of hospital visits. We encountered a male patient in his 70s on peritoneal dialysis for end-stage renal failure who developed obsolete mandibular fracture-associated pseudarthrosis accompanied by osteomyelitis, which was treated with noninvasive reduction and fixation using circumferential wiring after the resolution of inflammation...
2018: Journal of UOEH
Claudia Friedl, Emanuel Zitt
Secondary hyperparathyroidism (sHPT) is a frequently occurring severe complication of advanced kidney disease. Its clinical consequences include extraskeletal vascular and valvular calcifications, changes in bone metabolism resulting in renal osteodystrophy, and an increased risk of cardiovascular morbidity and mortality. Calcimimetics are a cornerstone of parathyroid hormone (PTH)-lowering therapy, as confirmed by the recently updated 2017 Kidney Disease: Improving Global Outcomes chronic kidney disease - mineral and bone disorder clinical practice guidelines...
2018: Drug Design, Development and Therapy
Jiemei Gu, Chun Wang, Hao Zhang, Hua Yue, Weiwei Hu, Jinwei He, Wenzhen Fu, Zhenlin Zhang
Hypophosphatemic rickets/osteomalacia is characterized by defective renal phosphate reabsorption and abnormal bone mineralization. Hypophosphatemic rickets/osteomalacia consists of inherited and acquired forms, many of which have unknown aetiology. In the present study, next‑generation sequencing‑based resequencing was used on samples from Chinese subjects with hypophosphatemic rickets/osteomalacia, aiming to detect the spectrum of pathogenic genes in these patients. A total of 86 hypophosphatemic rickets/osteomalacia patients (ranging from 3 to 70 years old) were recruited...
September 2018: International Journal of Molecular Medicine
Vincent M Brandenburg, Anja Verhulst, Anne Babler, Patrick C D'Haese, Pieter Evenepoel, Nadine Kaesler
Canonical Wnt signalling activity is a major player in physiological and adaptive bone metabolism. Wnt signalling is regulated by soluble inhibitors, with sclerostin being the most widely studied. Sclerostin's main origin is the osteocyte and its major function is blockade of osteoblast differentiation and function. Therefore, sclerostin is a potent inhibitor of bone formation and mineralization. Consequently, blocking sclerostin via human monoclonal antibodies (such as romosozumab) represents a promising perspective for the treatment of (postmenopausal) osteoporosis...
May 24, 2018: Nephrology, Dialysis, Transplantation
Sarah-Kim Bisson, Roth-Visal Ung, Fabrice Mac-Way
Vascular calcification and bone fragility are common and interrelated health problems that affect chronic kidney disease (CKD) patients. Bone fragility, which leads to higher risk of fracture and mortality, arises from the abnormal bone remodeling and mineralization that are seen in chronic kidney disease. Recently, sclerostin and Dickkopf-related protein 1 were suggested to play a significant role in CKD-related bone disease as they are known inhibitors of the Wnt pathway, thus preventing bone formation. This review focuses on new knowledge about the Wnt pathway in bone, how its function is affected by chronic kidney disease and how this affects bone structure...
2018: International Journal of Endocrinology
Ashish K Sharma, Nigel D Toussaint, Rosemary Masterson, Stephen G Holt, Chamith S Rajapakse, Peter R Ebeling, Sindhu T Mohanty, Paul Baldock, Grahame J Elder
BACKGROUND: Cortical bone is a significant determinant of bone strength and its deterioration contributes to bone fragility. Thin cortices and increased cortical porosity have been noted in patients with chronic kidney disease (CKD), but the "Turnover Mineralization Volume" classification of renal osteodystrophy does not emphasize cortical bone as a key parameter. We aimed to assess trabecular and cortical bone microarchitecture by histomorphometry and micro-CT in patients with CKD G5 and 5D (dialysis)...
2018: American Journal of Nephrology
Xiaoliang Sun, Xiaoqing Zhang, Yao Lu, Ling Zhang, Meng Yang
Severe hypocalcemia (SH) is a common and serious complication in dialysis patients with secondary hyperparathyroidism (SHPT) after parathyroidectomy (PTX). The aim is to explore the risk predictors of SH in post-PTX dialysis ESRD patients with SHPT. 129 consecutive dialysis patients with SHPT underwent PTX were retrospectively reviewed. A total of 22 clinical parameters were included in the study. SH was defined as the minimum values of serum calcium lower than 1.875 mmol/L (7.5 mg/dL) after surgery. Univariate analysis showed that pruritus, lumbar X-ray changes of renal osteodystrophy, pre- and post-operative intact parathyroid hormone (iPTH), Calcium, alkaline phosphatase, and gland mass were significantly different between SH and non-SH groups...
May 17, 2018: Scientific Reports
Sandro Mazzaferro, Giuseppe Cianciolo, Antonio De Pascalis, Chiara Guglielmo, Pablo A Urena Torres, Jordi Bover, Lida Tartaglione, Marzia Pasquali, Gaetano La Manna
Recent improvements in our understanding of physiology have altered the way in which bone is perceived: no longer is it considered as simply the repository of divalent ions, but rather as a sophisticated endocrine organ with potential extraskeletal effects. Indeed, a number of pathologic conditions involving bone in different ways can now be reconsidered from a bone-centred perspective. For example, in metabolic bone diseases like osteoporosis (OP) and renal osteodystrophy (ROD), the association with a worse cardiovascular outcome can be tentatively explained by the possible derangements of three recently discovered bone hormones (osteocalcin, fibroblast growth factor 23 and sclerostin) and a bone-specific enzyme (alkaline phosphatase)...
May 4, 2018: Nephrology, Dialysis, Transplantation
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