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    A Decision Support Tool for Healthcare Professionals in the Management of Hyperphosphatemia in Hemodialysis.
    Stud Health Technol Inform 2018 ;247:810-814
    Dept. of Health Science and Technology, Aalborg University, Aalborg, Denmark.
    Hyperphosphatemia is known as one of the more challenging conditions in end-stage renal disease patients. This study set out to present and evaluate a healthcare-oriented decision support tool in the management of hyperphosphatemia within hemodialysis therapy. A prototype module was designed to fit into the interface of a modern dialysis machine (Fresenius 5008). Read More

    Nutritional status and survival of maintenance hemodialysis patients receiving lanthanum carbonate.
    Nephrol Dial Transplant 2018 Apr 16. Epub 2018 Apr 16.
    Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan.
    Background: Hyperphosphatemia and poor nutritional status are associated with increased mortality. Lanthanum carbonate is an effective, calcium-free phosphate binder, but little is known about the long-term impact on mineral metabolism, nutritional status and survival.

    Methods: We extended the follow-up period of a historical cohort of 2292 maintenance hemodialysis patients that was formed in late 2008. Read More

    Should phosphate management be limited to the KDIGO/ KDOQI guidelines?
    Semin Dial 2018 Apr 19. Epub 2018 Apr 19.
    NorthShore University HealthSystem and University of Chicago-Pritzker School of Medicine, Chicago, IL, USA.
    Hyperphosphatemia is a common complication of CKD. Prior to development of overt hyperphosphatemia, there are several adaptive mechanisms that occur to maintain normal phosphorus equilibrium in patients with CKD. These include an early and progressive rise in fibroblast growth factor 23 (FGF 23), followed by an increase in parathyroid hormone (PTH) with a decrease in 1,25-dihydroxyvitamin D (1,25 Vit D). Read More

    Ubiquitin C-terminal hydrolase L1 deletion is associated with urinary α-klotho deficiency and perturbed phosphate homeostasis.
    Am J Physiol Renal Physiol 2018 Apr 18. Epub 2018 Apr 18.
    Medicine, University of Ottawa / Ottawa Hospital Research Institute, Canada.
    Loss of ubiquitin C-terminal hydrolase L1 (UCHL1), a deubiquitinating enzyme required for neuronal function, led to hyperphosphatemia accompanied by phosphaturia in mice, while calcium homeostasis remained intact. We therefore investigated the mechanisms underlying the phosphate imbalance in Uchl1-/- mice. Interestingly, phosphaturia was not a result of lower renal brush border membrane sodium-phosphate cotransporter expression as sodium-phosphate cotransporter 2a and 2c expression levels were similar to wild type levels. Read More

    Long-term efficacy and safety of sucroferric oxyhydroxide in African American dialysis patients.
    Hemodial Int 2018 Apr 15. Epub 2018 Apr 15.
    University of California, Los Angeles, California, USA.
    Introduction: Sucroferric oxyhydroxide (SFOH) is a non-calcium, iron-based phosphate binder that demonstrated sustained serum phosphorus (sP) control, good tolerability, and lower pill burden, vs. sevelamer carbonate ("sevelamer"), in a Phase 3 study conducted in dialysis patients with hyperphosphatemia. This analysis evaluates the efficacy and safety of SFOH and sevelamer among African American (AA) patients participating in the trial. Read More

    Zinc Inhibits Phosphate-Induced Vascular Calcification through TNFAIP3-Mediated Suppression of NF-B.
    J Am Soc Nephrol 2018 Apr 13. Epub 2018 Apr 13.
    Department of Internal Medicine and Cardiology, Charité- Universitätsmedizin Berlin, Berlin, Germany.
    The high cardiovascular morbidity and mortality of patients with CKD may result in large part from medial vascular calcification, a process promoted by hyperphosphatemia and involving osteo-/chondrogenic transdifferentiation of vascular smooth muscle cells (VSMCs). Reduced serum zinc levels have frequently been observed in patients with CKD, but the functional relevance of this remains unclear. We performed experiments in primary human aortic VSMCs; klotho-hypomorphic (), subtotal nephrectomy, and cholecalciferol-overload mouse calcification models; and serum samples from patients with CKD. Read More

    Soluble Klotho causes hypomineralization in Klotho-deficient mice.
    J Endocrinol 2018 Apr 9. Epub 2018 Apr 9.
    Y Yoshiko, Oral Grwoth and Developmental Biology, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima, 734-8553, Japan
    The type I transmembrane protein αKlotho (Klotho) serves as a coreceptor for the phosphaturic hormone fibroblast growth factor 23 (FGF23) in kidney, while a truncated form of Klotho (soluble Klotho, sKL) is thought to exhibit multiple activities, including acting as a hormone, but whose mode(s) of action in different organ systems remains to be fully elucidated. FGF23 is expressed primarily in osteoblasts/osteocytes and aberrantly high levels in the circulation acting via signaling through an FGF receptor (FGFR)-Klotho coreceptor complex cause renal phosphate wasting and osteomalacia. We assessed the effects of exogenously added sKL on osteoblasts and bone using Klotho-deficient () mice and cell and organ cultures. Read More

    Effects of education on low-phosphate diet and phosphate binder intake to control serum phosphate among maintenance hemodialysis patients: A randomized controlled trial.
    Kidney Res Clin Pract 2018 Mar 31;37(1):69-76. Epub 2018 Mar 31.
    Department of Nephrology, Ajou University School of Medicine, Suwon, Korea.
    Background: For phosphate control, patient education is essential due to the limited clearance of phosphate by dialysis. However, well-designed randomized controlled trials about dietary and phosphate binder education have been scarce.

    Methods: We enrolled maintenance hemodialysis patients and randomized them into an education group (n = 48) or a control group (n = 22). Read More

    Immune complex glomerulonephritis of suspected iatrogenic origin in five Japanese Black calves.
    J Vet Med Sci 2018 Apr 6. Epub 2018 Apr 6.
    Department of Veterinary Medicine and Surgery, University of Missouri, College of Veterinary Medicine.
    Five Japanese Black embryo transfer calves from a single embryo flush, 30 to 45-days-old, including 4 live animals for clinical examination and 1 dead for necropsy, were presented with a history of decreased milk intake and hypoproteinemia. Consistent clinicopathological abnormalities in the 4 calves presented for clinical evaluation included hyperkalemia, hyperphosphatemia, hypoproteinemia, hypoalbuminemia, hyperbilirubinemia, increased creatine phosphokinase activity, and proteinuria. Four calves ultimately were necropsied and all had histologic evidence of immune complex glomerulonephritis. Read More

    Effect of Lanthanum Carbonate on All-Cause Mortality in Patients Receiving Maintenance Hemodialysis: a Meta-Analysis of Randomized Controlled Trials.
    Kidney Blood Press Res 2018 Mar 29;43(2):536-544. Epub 2018 Mar 29.
    Department of Blood Purification, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
    Background/aims: Hyperphosphatemia is common in patients on hemodialysis. The efficacy of lanthanum carbonate (LC) in the treatment of hyperphosphatemia in these patients remains controversial. The objective of this meta-analysis was to evaluate the effect of LC on all-cause mortality in patients on maintenance hemodialysis. Read More

    Esophageal Mucosal Calcinosis: A Rare Site of Gastrointestinal Mucosal Calcinosis.
    Am J Case Rep 2018 Apr 6;19:406-409. Epub 2018 Apr 6.
    Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA.
    BACKGROUND Gastrointestinal tract mucosal calcinosis (MC) tends to affect the gastric mucosa, while esophageal involvement is rare. Gastric MC may be seen with solid organ transplantation, use of aluminum-containing antacids or sucralfate, malignancy, and chronic renal failure. While the incidence of gastric MC in renal transplant patients undergoing gastric biopsy is common (between 15-29%), to our knowledge esophageal MC has only been previously reported 3 times. Read More

    Hepatic production of fibroblast growth factor 23 in autosomal dominant polycystic kidney disease.
    J Clin Endocrinol Metab 2018 Mar 29. Epub 2018 Mar 29.
    Université Paris Descartes, Faculté de médecine, Paris, France.
    Context: the bone-derived hormone fibroblast growth factor 23 (FGF23) controls phosphate homeostasis and urinary phosphate excretion. FGF23 plasma levels increase at the early step of renal insufficiency to prevent hyperphosphatemia. Recent evidence suggests that this increase has off-target effects on cardiac and immune cells that compromises patients' health. Read More

    The effects of tenapanor on serum fibroblast growth factor 23 in patients receiving hemodialysis with hyperphosphatemia.
    Nephrol Dial Transplant 2018 Apr 3. Epub 2018 Apr 3.
    Duke University School of Medicine and Duke Clinical Research Institute, Durham, NC, USA.
    Background: Elevated serum fibroblast growth factor 23 (FGF23) is strongly associated with cardiovascular risk and mortality. Tenapanor, an inhibitor of gastrointestinal sodium/hydrogen exchanger isoform 3, decreased serum phosphate in a randomized, double-blind, placebo-controlled Phase 2 trial (ClinicalTrials.gov identifier NCT02081534) of patients receiving hemodialysis with hyperphosphatemia. Read More

    Aortic stenosis is a risk factor for all-cause mortality in patients on dialysis: a multicenter prospective cohort analysis.
    BMC Nephrol 2018 Apr 3;19(1):80. Epub 2018 Apr 3.
    Department of Nephrology, Fujita Health University, Toyoake, Aichi, Japan.
    Background: Aortic stenosis (AS) is common in patients on dialysis as well as in the general population. AS leads to difficulty with dialysis therapy because of unstable conditions such as intradialytic hypotension due to low cardiac output. However, the precise morbidity rates and risk factors of AS in patients on dialysis are unknown. Read More

    A novel calcimimetic agent, evocalcet (MT-4580/KHK7580), suppresses the parathyroid cell function with little effect on the gastrointestinal tract or CYP isozymes in vivo and in vitro.
    PLoS One 2018 3;13(4):e0195316. Epub 2018 Apr 3.
    Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Kanagawa, Japan.
    Cinacalcet hydrochloride (cinacalcet), an oral calcimimetic agent has been widely used for the management of secondary hyperparathyroidism (SHPT) in chronic kidney disease (CKD). In sharp contrast to vitamin D receptor activators, cinacalcet suppresses SHPT without inducing hypercalcemia or hyperphosphatemia. Nevertheless, some patients remain refractory to SHPT with this agent, as the dose cannot be sufficiently increased due to gastrointestinal symptoms. Read More

    Hyperphosphatemic tumoral calcinosis caused by FGF23 compound heterozygous mutations: what are the therapeutic options for a better control of phosphatemia?
    Pediatr Nephrol 2018 Mar 28. Epub 2018 Mar 28.
    Centre de Référence des Maladies Rénales Rares, Centre de Référence des Maladies Rares du Calcium et du Phosphate, Hospices Civils de Lyon, Hôpital Femme Mère Enfant, 59 boulevard Pinel, 69677, Bron cedex, France.
    Background: Hyperphosphatemic familial tumoral calcinosis (HFTC) is a rare autosomal recessive disease caused by mutations in genes encoding FGF23 or its regulators, and leading to functional deficiency or resistance to fibroblast growth factor 23 (FGF23). Subsequent biochemical features include hyperphosphatemia due to increased renal phosphate reabsorption, and increased or inappropriately normal 1,25-dihydroxyvitamin D (1,25-D) levels.

    Case-diagnosis/treatment: A 15-year-old girl was referred for a 1. Read More

    [CKD-MBD in Peritoneal Dialysis].
    G Ital Nefrol 2018 Mar;35(2)
    Dipartimento di Scienze Cardiovascolari Respiratorie Nefrologiche Anestetiche e Geriatriche. Sapienza Università di Roma.
    CKD-MBD is a systemic disorder of the mineral and bone metabolism as a result of CKD. The clinical relevance of this syndrome has led to the identification of the biochemical targets to be achieved in order to improve the outcome of the patient. However, in hemodialysis (HD) and peritoneal dialysis (DP) patients, these targets are not reached. Read More

    Prevention and treatment of hyperphosphatemia in chronic kidney disease.
    Kidney Int 2018 May 23;93(5):1060-1072. Epub 2018 Mar 23.
    Department of Nephrology, Amsterdam Cardiovascular Sciences, VU University Medical Center, Amsterdam, the Netherlands; Department of Epidemiology and Biostatistics, Amsterdam Public Health Institute, VU University Medical Center, Amsterdam, the Netherlands.
    Hyperphosphatemia has consistently been shown to be associated with dismal outcome in a wide variety of populations, particularly in chronic kidney disease (CKD). Compelling evidence from basic and animal studies elucidated a range of mechanisms by which phosphate may exert its pathological effects and motivated interventions to treat hyperphosphatemia. These interventions consisted of dietary modifications and phosphate binders. Read More

    Cost-Effectiveness of First-Line Sevelamer and Lanthanum versus Calcium-Based Binders for Hyperphosphatemia of Chronic Kidney Disease.
    Value Health 2018 03 18;21(3):318-325. Epub 2017 Oct 18.
    Department of Epidemiology and Biostatistics, Western University, London, Ontario, Canada.
    Background: Phosphate binders are used to treat hyperphosphatemia among patients with chronic kidney disease (CKD).

    Objectives: To conduct an economic evaluation comparing calcium-free binders sevelamer and lanthanum with calcium-based binders for patients with CKD.

    Methods: Effectiveness data were obtained from a recent meta-analysis of randomized trials. Read More

    Nicotinic acid and related compounds: A meta-analysis of their use for hyperphosphatemia in dialysis patients.
    Medicine (Baltimore) 2018 Mar;97(12):e0117
    Hemodialysis Room, Liaocheng People's Hospital of Shandong Province, China.
    Background: Studies indicate that nicotinic acid and related compounds may decrease phosphorus concentrations effectively by reducing the absorption in the gastrointestinal tract. However, the efficacy and safety of oral niacin treatments have only been investigated in a limited number of small-scale studies.

    Methods: We performed this meta-analysis by pooling 12 qualified relevant preclinical and clinical trials to evaluate the association of nicotinic acid (and its related compounds) treatment and hyperphosphatemia among dialysis patients. Read More

    Dialysis as a Treatment Option for a Patient With Normal Kidney Function and Familial Tumoral Calcinosis Due to a Compound Heterozygous FGF23 Mutation.
    Am J Kidney Dis 2018 Mar 14. Epub 2018 Mar 14.
    Nephrology Division, University of São Paulo School of Medicine, São Paulo-SP, Brazil; Universidade Nove de Julho, São Paulo-SP, Brazil. Electronic address:
    Primary tumoral calcinosis is a rare autosomal recessive disorder characterized by ectopic calcified tumoral masses. Mutations in 3 genes (GALNT3, FGF23, and KL) have been linked to this human disorder. We describe a case of a 28-year-old man with a history of painful firm masses over his right and left gluteal region, right clavicle region, knees, and left elbow. Read More

    Inhibition of enzymes involved in collagen cross-linking reduces vascular smooth muscle cell calcification.
    FASEB J 2018 Mar 16:fj201700653R. Epub 2018 Mar 16.
    Hospital Clínico Universitario Virgen de la Arrixaca, Universidad de Murcia, Instituto Murciano de Investigación Biosanitaria (IMIB)-Arrixaca, Murcia, Spain.
    Vascular smooth muscle cells (VSMCs) transdifferentiate into osteoblast-like cells during vascular calcification, inducing active remodeling and calcification of the extracellular matrix (ECM). Intracellular and extracellular enzymes, such as lysyl hydroxylase 1 (PLOD1) and lysyl oxidase (LOX), contribute to ECM maturation and stabilization. We assessed the contribution of these enzymes to hyperphosphatemia (HPM)-induced calcification. Read More

    Ingestion of Fireworks: Rare Cause of Poisoning in Children.
    Pediatr Emerg Care 2018 Mar 12. Epub 2018 Mar 12.
    Background: Mistaken ingestion of all manner of toxic matter is common in childhood, but poisoning with fireworks and matchsticks is rare. Fireworks usually contain 10% yellow phosphorus and 50% potassium chlorate. Potassium chlorate is an extremely reactive and toxic agent that is used in fireworks and matchstick heads. Read More

    Rationale, design, and characteristics of a trial to evaluate the new phosphate iron-based binder sucroferric oxyhydroxide in dialysis patients with the goal of advancing the practice of E.B.M. (EPISODE).
    Clin Exp Nephrol 2018 Mar 13. Epub 2018 Mar 13.
    Department of Comprehensive Kidney Disease Research, Osaka University Graduate School of Medicine, Suita, Japan.
    Background: In dialysis patients, mortality risk due to cardiovascular diseases is remarkably high and prognosis is poor; coronary artery calcification is considered one of the major contributing factors. It is known that hyperphosphatemia is associated with coronary artery calcification. Therefore, controlling serum phosphate level and thereby mitigating vascular calcification could improve the poor prognosis of dialysis patients. Read More

    Comparison of Renal Function and Other Predictors in Lacto-Ovo Vegetarians and Omnivores With Chronic Kidney Disease.
    J Am Coll Nutr 2018 Mar 13:1-6. Epub 2018 Mar 13.
    a Department of Nutrition , Chung Shan Medical University Hospital , Taichung , Taiwan.
    Objective Vegetarian diets have been shown to increase the risk of certain nutritional deficiencies, such as iron. As a number of patients with chronic kidney disease (CKD) in Taiwan are lacto-ovo vegetarians, the aim of this study was to investigate the effects of different proportions and sources of protein in lacto-ovo vegetarian and omnivorous diets, as well as the influence of adequate dietary protein intake, on renal function and nutritional status of Taiwanese patients with stage 3 to stage 5 CKD. Methods This is a cross-sectional study. Read More

    Parathyroidectomy in the Management of Secondary Hyperparathyroidism.
    Clin J Am Soc Nephrol 2018 Mar 9. Epub 2018 Mar 9.
    Harold Simmons Center for Kidney Disease Research and Epidemiology, University of California, Irvine, California
    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. Read More

    Etidronate for Prevention of Ectopic Mineralization in Patients With Pseudoxanthoma Elasticum.
    J Am Coll Cardiol 2018 Mar;71(10):1117-1126
    Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. Electronic address:
    Background: In pseudoxanthoma elasticum (PXE), low pyrophosphate levels may cause ectopic mineralization, leading to skin changes, visual impairment, and peripheral arterial disease.

    Objectives: The authors hypothesized that etidronate, a pyrophosphate analog, might reduce ectopic mineralization in PXE.

    Methods: In the Treatment of Ectopic Mineralization in Pseudoxanthoma Elasticum trial, adults with PXE and leg arterial calcifications (n = 74) were randomly assigned to etidronate or placebo (cyclical 20 mg/kg for 2 weeks every 12 weeks). Read More

    Impact of Serum Phosphate in Mechanically Ventilated Patients With Severe Sepsis and Septic Shock.
    J Intensive Care Med 2018 Jan 1:885066618762753. Epub 2018 Jan 1.
    2 Department of Pharmacy, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
    Background: Hypo- and hyperphosphatemia are common in severe sepsis and septic shock. Published outcome data in patients with phosphate derangements primarily focus on hypophosphatemia and the general critically ill population. This study aimed to determine the impact of serum phosphate on clinical outcomes in patients with severe sepsis and septic shock. Read More

    Sevelamer-Induced Gastrointestinal Injury Presenting as Gastroenteritis.
    Case Rep Gastroenterol 2018 Jan-Apr;12(1):41-45. Epub 2018 Feb 1.
    Madigan Army Medical Center, Tacoma, Washington, USA.
    End-stage renal disease (ESRD) is a common disease encountered in clinical practice and is associated with increasing metabolic derangements through disease progression. Phosphate retention is one of the most common derangements and is associated with increased mortality. Hyperphosphatemia becomes increasingly prevalent as glomerular filtration rate decreases. Read More

    Sanjad-Sakati syndrome with macrocytic anemia and failure to thrive: a case from South Jordan.
    J Pediatr Endocrinol Metab 2018 Mar 1. Epub 2018 Mar 1.
    Pediatric Department, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
    Backgorund: Sanjad-Sakati syndrome (SSS) is a rare autosomal recessive disease caused by a deletion mutation (155-166del) in exon 3 of the TBCE gene on chromosome 1q42-43. The syndrome is characterized by primary hypoparathyroidism, typical dysmorphic features and severe growth retardation.

    Case Presentation: We encountered a 2-year-old boy with hypocalcemia, failure to thrive and macrocytic anemia. Read More

    Misdiagnosis of idiopathic hypoparathyroidism: A case report and literature review.
    Medicine (Baltimore) 2018 Mar;97(9):e9884
    Department of Neurology, PLA 44 Hospital.
    Rationale: Idiopathic hypoparathyroidism (IHP) is a rare endocrine condition, which is frequently represented by neuropsychiatric disorders. Hence, the misdiagnosis rate of the disease is rather high, especially for neurologists.

    Patient Concerns: We reported a case of misdiagnosed, atypical IHP. Read More

    Phosphate stimulates myotube atrophy through autophagy activation: evidence of hyperphosphatemia contributing to skeletal muscle wasting in chronic kidney disease.
    BMC Nephrol 2018 Feb 27;19(1):45. Epub 2018 Feb 27.
    Department of Nephrology, Shanghai General Hospital, Shanghai Jiaotong University School of Medcine, Haining Road 100rd, Hongkou Distrinct, Shanghai, 200080, China.
    Background: Accelerated muscle atrophy is associated with a three-fold increase in mortality in chronic kidney disease (CKD) patients. It is suggested that hyperphosphatemia might contribute to muscle wasting, but the underlying mechanisms remain unclear. Although evidence indicates that autophagy is involved in the maintenance of muscle homeostasis, it is not known if high phosphate levels can result in activation of autophagy, leading to muscle protein loss. Read More

    Nitrite-induced acute kidney injury with secondary hyperparathyroidism: Case report and literature review.
    Medicine (Baltimore) 2018 Feb;97(8):e9889
    Department of Nephrology, Shandong University Qilu Hospital, Jinan City, China.
    Rationale: Acute kidney injury (AKI) with hyperparathyroidism caused by nitrite was rare, and renal function and parathyroid hormone (PTH) decreased to normal range after therapy.

    Patient Concerns: Acute kidney injury was diagnosed in a 40-year-old male with hyperparathyroidism and cyanosis of his hands and both forearms.

    Diagnoses: The patient ate some recently pickled vegetables, and he experienced nausea, vomiting and diarrhoea without oliguria or anuria; Additionally, his hands and both forearms had a typical blue ash appearance. Read More

    Efficacy of sucroferric oxyhydroxide treatment in Japanese hemodialysis patients and its effect on gastrointestinal symptoms.
    Pharmazie 2017 Feb;72(2):118-122
    Sucroferric oxyhydroxide (SFOH) is a non-calcium, iron-based phosphate binder indicated for the treatment of hyperphosphatemia in adult dialysis patients. Studies in Japan about the side effects of SFOH treatment indicate that the incidence of diarrhea (25%) is greater while that of constipation (2.9%) is lesser in comparison to that observed upon treatment with an existing phosphate binder. Read More

    Managing hyperparathyroidism in hemodialysis: role of etelcalcetide.
    Int J Nephrol Renovasc Dis 2018 5;11:69-80. Epub 2018 Feb 5.
    Division of Nephrology, Hennepin County Medical Center, University of Minnesota, Minneapolis, MN.
    Secondary hyperparathyroidism (SHPT) is common in patients receiving maintenance hemodialysis and is associated with adverse outcomes. Currently, SHPT is managed by reducing circulating levels of phosphate with oral binders and parathyroid hormone (PTH) with vitamin D analogs and/or the calcimimetic cinacalcet. Etelcalcetide, a novel calcimimetic administered intravenously (IV) at the end of a hemodialysis treatment session, effectively reduces PTH in clinical trials when given thrice weekly. Read More

    Rapid whole genome sequencing identifies a novel AIRE variant associated with Autoimmune Polyendocrine Syndrome Type 1.
    Cold Spring Harb Mol Case Stud 2018 Feb 1. Epub 2018 Feb 1.
    Rady Children's Institute for Genomic Medicine.
    Autoimmune polyendocrine syndrome type 1 (APS1; OMIM #240300), also referred to as autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), is a rare monogenic disorder caused by mutations in the autoimmune regulator (AIRE) gene. APS1 is classically characterized by a triad of chronic mucocutaneous candidiasis, autoimmune hypoparathyroidism, and autoimmune adrenocortical insufficiency. We report a five-year-old female who presented with symptoms of tetany due to hypocalcemia and was subsequently found to be secondary to hypoparathyroidism. Read More

    Pseudohypoparathyroidism type 1B in a patient conceived by in vitro fertilization: another imprinting disorder reported with assisted reproductive technology.
    J Assist Reprod Genet 2018 Feb 7. Epub 2018 Feb 7.
    Pediatric Endocrinology, Mercy Hospital St. Louis, St. Louis, MO, USA.
    Pseudohypoparathyroidism type 1B (PHP1B) is characterized by renal tubular resistance to parathyroid hormone (PTH) leading to hyperphosphatemia, hypocalcemia, elevated PTH, and hyperparathyroid bone changes. PHP1B is an imprinting disorder that results from loss of methylation at the maternal GNAS gene, which suppresses transcription of the alpha subunit of the stimulatory G protein of the PTH receptor. Emerging evidence supports an association between assisted reproductive technologies (ART) and imprinting disorders; however, there is currently little evidence linking PHP1B and ART. Read More

    [Pharmacological, pharmaceutical and clinical profiles of sucroferric oxyhydroxide (P-TOL Chewable Tab. 250 mg, 500 mg), a therapeutic agent for hyperphosphatemia].
    Nihon Yakurigaku Zasshi 2018;151(2):75-86
    Clinical Administration, Clinical Research Department, Kissei Pharmaceutical Co., Ltd.
    Sucroferric oxyhydroxide (P-TOL chewable tablets, 250 and 500 mg) is a phosphate binder for oral use; it is composed of polynuclear iron (III)-oxyhydroxide, sucrose, and starches, and is currently indicated for alleviating hyperphosphatemia in patients with chronic kidney disease (CKD) on dialysis. The results of non-clinical pharmacological studies have suggested that P-TOL consistently decreases serum phosphorus levels in the aqueous environment at pH levels similar to those in the gastrointestinal tract, thereby suppressing the progression of secondary hyperparathyroidism, aberrant calcification, and abnormal bone metabolism associated with hyperphosphatemia. Since the diameter of the P-TOL tablet exceeds 15 mm, it is manufactured with a doughnut-shape to minimize choking hazards. Read More

    Phosphate is a potential biomarker of disease severity and predicts adverse outcomes in acute kidney injury patients undergoing continuous renal replacement therapy.
    PLoS One 2018 7;13(2):e0191290. Epub 2018 Feb 7.
    Department of Internal Medicine, College of Medicine, Institute of Kidney Disease Research, Yonsei University, Seoul, Korea.
    Hyperphosphatemia is associated with mortality in patients with chronic kidney disease, and is common in critically ill patients with acute kidney injury (AKI); however, its clinical implication in these patients is unknown. We conducted an observational study in 1144 patients (mean age, 63.2 years; male, 705 [61. Read More

    Phosphate Removal During Conventional Hemodialysis: a Decades-Old Misconception.
    Kidney Blood Press Res 2018 31;43(1):110-114. Epub 2018 Jan 31.
    Department of Medicine, Renal Division, Universidade de São Paulo, São Paulo, Brazil.
    Background/aims: Hyperphosphatemia is associated with high mortality rate in patients on dialysis. Conventional hemodialysis (HD) is a limit technique in removing phosphate (P). There is a widespread belief that P is removed mainly in the first hour of HD. Read More

    [Chronic Kidney Disease - Update 2018].
    Dtsch Med Wochenschr 2018 Feb 6;143(3):169-173. Epub 2018 Feb 6.
    Medizinische Klink III, Nephrologische Abteilung, Klinikum Coburg.
    SGLT2-INHIBITION IN DIABETIC AND NON-DIABETIC KIDNEY DISEASE:  The CANVAS Program Collaborative Group study confirmed nephroprotective actions by canagliflocin comparable to empagliflozin as published in the EMPA-REG Outcome study. Treatment with Liraglutide (LEADER study) also suggests nephroprotection via albuminuria reduction a decreased eGFR decline in subgroups and depending on stages of diabetic nephropathy. KDIGO CKD-MBD GUIDELINE UPDATE 2017:  In July 2017, an update of the KDIGO (Kidney Disease: Improving Global Outcomes) 2009 guideline on diagnostic and treatment chronic kidney disease - mineral and bone disorders (CKD-MBD) was published. Read More

    Hypophosphatasia: the contribution of imaging.
    Arch Pediatr 2017 May;24(5S2):5S74-5S79
    Centre de référence pour les maladies rares du métabolisme du calcium et du phosphore, filière OSCAR; unité d'endocrinologie, maladies osseuses, génétique et gynécologie, hôpital des enfants, CHU de Toulouse, TSA 70034, 31059 Toulouse Cedex 09, France; Centre de physiopathologie de Toulouse-Purpan, CPTP, INSERM UMR 1043, université de Toulouse-Paul-Sabatier, 31059 Toulouse, France. Electronic address:
    Radiography and imaging are necessary for the diagnosis of hypophosphatasia (HPP) at all stages of life, from the antenatal period to the complications of adulthood, and in the forms of variable severity. The consequences of alkaline phosphatase activity deficiency, namely defective mineralization and bone fragility, may be detected by radiological tools and share features that distinguish them from other diseases responsible for mineralization defects. Radiography and imaging are also fundamental for the screening and diagnosis of the complications of HPP, some of which are related to the episodes of hypercalcemia and hyperphosphatemia (nephrocalcinosis). Read More

    Perinatal and infantile hypophosphatasia: clinical features and treatment.
    Arch Pediatr 2017 May;24(5S2):5S61-5S65
    Centre de référence maladies osseuses constitutionnelles, Institut Imagine, université Paris-Descartes-Sorbonne-Paris Cité, hôpital Necker-Enfants malades, 149, rue de Sèvres, 75015 Paris, France.
    Hypophosphatasia (HPP) is a rare hereditary disease characterized by defective skeletal mineralization, and with a broad severity spectrum. The perinatal forms, lethal and non-lethal, are associated with severe neonatal respiratory distress, potential seizures, hypotrophy and marked hypotonia. The diagnosis is rapidly suggested by a combination of typical radiological signs, hypercalcemia, hyperphosphatemia and low alkaline phosphatase (ALP) activity. Read More

    Relation of the Brugada Phenocopy to Hyperkalemia (from the International Registry on Brugada Phenocopy).
    Am J Cardiol 2018 Mar 29;121(6):715-717. Epub 2017 Dec 29.
    Department of Medicine, Queen's University, Kingston, Ontario, Canada. Electronic address:
    Brugada phenocopies (BrPs) are clinical entities that differ in etiology from true congenital Brugada syndrome but have identical electrocardiographic (ECG) patterns. Hyperkalemia is known to be one of the causes of BrP. The aim of this study was to determine the clinical characteristics and evolution of hyperkalemia-induced BrP. Read More

    Pulse versus daily oral Alfacalcidol treatment of secondary hyperparathyroidism in hemodialysis patients: a randomized controlled trial.
    Int J Nephrol Renovasc Dis 2018 15;11:25-32. Epub 2018 Jan 15.
    Public Health Department, College of Health Sciences, Qatar University, Doha, Qatar.
    Background: Secondary hyperparathyroidism is a common complication of chronic kidney disease and is managed using vitamin D replacement therapy. Very few studies have examined the effectiveness of pulse alfacalcidol therapy in comparison to daily oral alfacalcidol therapy in suppressing serum parathyroid hormone (PTH) levels in hemodialysis patients. The aim of this randomized controlled trial was to replicate the findings of prior studies comparing effectiveness of pulse oral alfacalcidol therapy versus daily oral alfacalcidol therapy in suppressing PTH after 13 weeks of therapy using a Palestinian sample of hemodialysis patients, and to identify demographic and biomedical characteristics of patients that are independently associated with PTH levels. Read More

    Classic and Non-Classic Features in Pseudohypoparathyroidism: Case Study and Brief Literature Review.
    Cureus 2017 Nov 26;9(11):e1878. Epub 2017 Nov 26.
    Assistant Clinical Professor of Internal Medicine, West Virginia University School of Medicine.
    Pseudohypoparathyroidism is a rare condition that is due to a defect in the stimulatory G-protein coupled receptor, resulting in end-organ resistance to parathyroid hormone. Hereditary forms of pseudohypoparathyroidism present with certain classic features such as obesity, short stature, brachydactyly, and intellectual disability. Constellation of these physical features is known as Albright's hereditary osteodystrophy. Read More

    (Epi)genotype-Phenotype Analysis in 69 Japanese Patients With Pseudohypoparathyroidism Type I.
    J Endocr Soc 2018 Jan 21;2(1):9-23. Epub 2017 Nov 21.
    Department of Molecular Endocrinology, National Research Institute for Child Health and Development, Tokyo 157-8535, Japan.
    Context: Pseudohypoparathyroidism type I (PHP-I) is divided into PHP-Ia with Albright hereditary osteodystrophy and PHP-Ib, which usually shows no Albright hereditary osteodystrophy features. Although PHP-Ia and PHP-Ib are typically caused by genetic defects involving subunit of the stimulatory G protein (Gs)-coding exons and methylation defects of the differentially methylated regions (DMRs) on the maternal allele, respectively, detailed phenotypic characteristics still remains to be examined.

    Objective: To clarify phenotypic characteristics according to underlying (epi)genetic causes. Read More

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