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https://www.readbyqxmd.com/read/28732801/bicarbonate-in-cystic-fibrosis
#1
REVIEW
Karl Kunzelmann, Rainer Schreiber, Hans Beat Hadorn
BACKGROUND: Cystic fibrosis (CF, mucoviscidosis) is caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), which is a chloride and bicarbonate channel necessary for fluid secretion and extracellular alkalization. For a long time, research concentrated on abnormal Cl(-) and Na(+) transport, but neglected bicarbonate as a crucial factor in CF. METHODS: The present short review reports early findings as well as recent insights into the role of CFTR for bicarbonate transport and its defects in CF...
July 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28725949/tubulointerstitial-nephritis-induced-hypophosphatemic-osteomalacia-in-sj%C3%A3-gren-s-syndrome-a-case-report-and-review-of-the-literature
#2
REVIEW
Yan Geng, Youlu Zhao, Zhuoli Zhang
Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disease that typically affects the salivary and lacrimal glands. Renal involvement is relatively uncommon and may precede other complaints. Tubulointestitial nephritis (TIN) is the most common renal involvement in SS. Osteomalacia occurring as the first manifestation of renal tubular disorder due to SS is very rare. We report a 39-year-old male who presented with polydipsia, polyuria, and multiple bone pain. Bone density test showed severe osteoporosis, and laboratory findings suggested hypokalemia, hypophosphatemia, and vitamin D deficiency, which supported the diagnosis of hypophosphatemic osteomalacia...
July 20, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28708304/hyperpolarized-13-c-magnetic-resonance-evaluation-of-renal-ischemia-reperfusion-injury-in-a-murine-model
#3
Celine Baligand, Hecong Qin, Aisha True-Yasaki, Jeremy W Gordon, Cornelius von Morze, Justin Delos Santos, David M Wilson, Robert Raffai, Patrick M Cowley, Anthony J Baker, John Kurhanewicz, David H Lovett, Zhen Jane Wang
Acute kidney injury (AKI) is a major risk factor for the development of chronic kidney disease (CKD). Persistent oxidative stress and mitochondrial dysfunction are implicated across diverse forms of AKI and in the transition to CKD. In this study, we applied hyperpolarized (HP) (13) C dehydroascorbate (DHA) and (13) C pyruvate magnetic resonance spectroscopy (MRS) to investigate the renal redox capacity and mitochondrial pyruvate dehydrogenase (PDH) activity, respectively, in a murine model of AKI at baseline and 7 days after unilateral ischemia reperfusion injury (IRI)...
July 14, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28658721/-prevalence-of-allergic-diseases-in-children-with-secondary-renal-tubular-acidosis-attending-a-tertiary-care-pediatric-center
#4
Blanca María Morfín-Maciel, Silvestre García-De la Puente, Aurora Bojórquez-Ochoa, Alfonso Huante-Anaya, Socorro Orozco-Martínez, Samuel Zaltzman-Girshevich, Elizabeth Guzmán-Vázquez
BACKGROUND: It has been suggested a high prevalence of allergic disease in children with RTA. OBJECTIVE: To describe the prevalence of allergic diseases in children with secondary RTA (renal tubular acidosis) in the nephrology department of the National Institute of Pediatrics (NIP), México. METHODS: An observational, prospective, cross-sectional, descriptive study. Children with secondary RTA < 18 years who attended the outpatient nephrology service in the NIP for 24 months, were included...
April 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28655387/hemodialysis-treatment-of-cardiorenal-syndrome
#5
Boštjan Leskovar, Tjaša Furlan, Simona Poznič, Maja Potisek, Anton Adamlje
AIMS: We evaluated the impact of hemodialysis on mortality and hospital readmission in patients with cardiorenal syndrome. METHODS: All patients were NYHA IV functional class and underwent laboratory testing, echocardiography, and cardiac functional testing. Hemodialysis was indicated in patients with progressive decline of kidney function and consequent failure to titrate heart failure medication as well as in patients with hypervolemia that was resistant to conservative treatment with more than 4 annual hospitalizations due to heart failure and/or concomitant chronic kidney disease stage III - IV...
June 28, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28648509/pendred-syndrome
#6
REVIEW
Jean-Louis Wémeau, Peter Kopp
Pendred syndrome is an autosomal recessive disorder that is classically defined by the combination of sensorineural deafness/hearing impairment, goiter, and an abnormal organification of iodide with or without hypothyroidism. The hallmark of the syndrome is the impaired hearing, which is associated with inner ear malformations such as an enlarged vestibular aqueduct (EVA). The thyroid phenotype is variable and may be modified by the nutritional iodine intake. Pendred syndrome is caused by biallelic mutations in the SLC26A4/PDS gene, which encodes the multifunctional anion exchanger pendrin...
March 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28646128/%C3%AE-copi-mediates-the-retention-of-kae1-g701d-protein-in-golgi-apparatus-a-mechanistic-explanation-of-drta-associated-g701d-mutation
#7
Natapol Duangtum, Mutita Junking, Suratchanee Phadngam, Nunghathai Sawasdee, Andrea Castiglioni, Komgrid Charngkaew, Thawornchai Limjindaporn, Ciro Isidoro, Pa-Thai Yenchitsomanus
Mutations of solute carrier family 4 member 1 ( SLC4A1 ) gene encoding kidney anion (chloride/bicarbonate ion) exchanger 1 (kAE1) can cause genetic distal renal tubular acidosis (dRTA). Different SLC4A1 mutations give rise to mutant kAE1 proteins with distinct defects in protein trafficking. The mutant kAE1 protein may be retained in endoplasmic reticulum (ER), Golgi apparatus, or mis-targeted to the apical membrane, failing to display its function at the baso-lateral membrane. The ER-retained mutant kAE1 interacts with calnexin chaperone protein; disruption of this interaction permits the mutant kAE1 to reach the cell surface and display anion exchange activity...
June 23, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28645705/acid-load-and-phosphorus-homeostasis-in-ckd
#8
Pascale Khairallah, Tamara Isakova, John Asplin, Lee Hamm, Mirela Dobre, Mahboob Rahman, Kumar Sharma, Mary Leonard, Edgar Miller, Bernard Jaar, Carolyn Brecklin, Wei Yang, Xue Wang, Harold Feldman, Myles Wolf, Julia J Scialla
BACKGROUND: The kidneys maintain acid-base homeostasis through excretion of acid as either ammonium or as titratable acids that primarily use phosphate as a buffer. In chronic kidney disease (CKD), ammoniagenesis is impaired, promoting metabolic acidosis. Metabolic acidosis stimulates phosphaturic hormones, parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF-23) in vitro, possibly to increase urine titratable acid buffers, but this has not been confirmed in humans. We hypothesized that higher acid load and acidosis would associate with altered phosphorus homeostasis, including higher urinary phosphorus excretion and serum PTH and FGF-23...
June 21, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28642116/contrast-induced-nephropathy-basic-concepts-pathophysiological-implications-and-prevention-strategies
#9
REVIEW
Charalampos Mamoulakis, Konstantinos Tsarouhas, Irini Fragkiadoulaki, Ioannis Heretis, Martin F Wilks, Demetrios A Spandidos, Christina Tsitsimpikou, Aristides Tsatsakis
Contrast-induced nephropathy (CIN) is reversible acute renal failure observed following administration of iodinated contrast media (CM) during angiographic or other medical procedures such as urography. There are various mechanisms through which CM develop their nephrotoxic effects, including oxidative stress and apoptosis. CIN is a real-life, albeit not very rare, entity. Exact pathophysiology remains obscure and no standard diagnostic criteria apply. The Acute Kidney Injury Network criteria was recently employed but its incidence/clinical significance warrants further clarification based on recent methodological advancements, because most published studies to date were contaminated by bias...
June 19, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28596649/use-of-surrogate-samples-to-monitor-ph-during-high-dose-methotrexate-therapy
#10
Uday Yanamandra, Pooja Chauhan, Deepesh Lad, Alka Khadwal, Gaurav Prakash, Subhash Varma, Pankaj Malhotra
High dose methotrexate (Mtx) therapy is commonly used in hemato-oncological practice. Alkalization of urine is a part of high dose methotrexate therapy for preventing crystallization in urine to avert renal insufficiency. Alkalization is monitored by urine pH at regular intervals. Oral pH has occasionally been used as a surrogate for oral mucositis. To compare and correlate pH of various body secretions (venous blood, oral salivary, lacrimal and urine) among patients undergoing alkalization with intravenous sodium bicarbonate during high dose methotrexate therapy...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28560615/effect-of-continuous-dialysis-on-blood-ph-in-acidemic-hypercapnic-animals-with-severe-acute-kidney-injury-a-randomized-experimental-study-comparing-high-vs-low-bicarbonate-affluent
#11
Thiago Gomes Romano, Luciano Cesar Pontes Azevedo, Pedro Vitale Mendes, Eduardo Leite Vieira Costa, Marcelo Park
BACKGROUND: Controlling blood pH during acute ventilatory failure and hypercapnia in individuals suffering from severe acute kidney injury (AKI) and undergoing continuous renal replacement therapy (CRRT) is of paramount importance in critical care settings. In this situation, the optimal concentration of sodium bicarbonate in the dialysate is still an unsolved question in critical care since high concentrations may worsen carbon dioxide levels and low concentrations may not be as effective in controlling pH...
December 2017: Intensive Care Medicine Experimental
https://www.readbyqxmd.com/read/28550912/does-metabolic-alkalosis-influence-cerebral-oxygenation-in-infantile-hypertrophic-pyloric-stenosis
#12
Matthias Nissen, Grigore Cernaianu, Rene Thränhardt, Mohammad R Vahdad, Karin Barenberg, Ralf-Bodo Tröbs
BACKGROUND: This pilot study focuses on regional tissue oxygenation (rSO2) in patients with infantile hypertrophic pyloric stenosis in a perioperative setting. To investigate the influence of enhanced metabolic alkalosis (MA) on cerebral (c-rSO2) and renal (r-rSO2) tissue oxygenation, two-site near-infrared spectroscopy (NIRS) technology was applied. MATERIALS AND METHODS: Perioperative c-rSO2, r-rSO2, capillary blood gases, and electrolytes from 12 infants were retrospectively compared before and after correction of MA at admission (T1), before surgery (T2), and after surgery (T3)...
May 15, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28550472/salt-induced-na-k-atpase-%C3%AE-%C3%AE-expression-involves-soluble-adenylyl-cyclase-in-endothelial-cells
#13
Mirja Mewes, Johanna Nedele, Katrin Schelleckes, Olga Bondareva, Malte Lenders, Kristina Kusche-Vihrog, Hans-Joachim Schnittler, Stefan-Martin Brand, Boris Schmitz, Eva Brand
High dietary salt intake may lead to vascular stiffness, which predicts cardiovascular diseases such as heart failure, and myocardial and cerebral infarctions as well as renal impairment. The vascular endothelium is a primary target for deleterious salt effects leading to dysfunction and endothelial stiffness. We hypothesize that the Ca(2+)- and bicarbonate-activated soluble adenylyl cyclase (sAC) contributes to Na(+)/K(+)-ATPase expression regulation in vascular endothelial cells and is an important regulator of endothelial stiffness...
May 26, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28542241/signification-of-distal-urinary-acidification-defects-in-hypocitraturic-patients
#14
Valentina Forni Ogna, Anne Blanchard, Rosa Vargas-Poussou, Adam Ogna, Stéphanie Baron, Jean-Philippe Bertocchio, Caroline Prot-Bertoye, Jérôme Nevoux, Julie Dubourg, Gérard Maruani, Margarida Mendes, Alejandro Garcia-Castaño, Cyrielle Treard, Nelly Lepottier, Pascal Houillier, Marie Courbebaisse
BACKGROUND AND OBJECTIVES: Hypocitraturia has been associated with metabolic acidosis and mineral disorders. The aim of this study was to investigate the occurrence of urinary acidification defects underlying hypocitraturia. MATERIALS AND METHODS: This retrospective observational study included 67 patients (32 men), aged 40.7±15.1 years with hypocitraturia (<1.67 mmol/24-h) and nephrolithiasis, nephrocalcinosis, and/or bone demineralization, referred to our center from 2000 to 2015...
2017: PloS One
https://www.readbyqxmd.com/read/28540602/late-conversion-from-tacrolimus-to-a-belatacept-based-immuno-suppression-regime-in-kidney-transplant-recipients-improves-renal-function-acid-base-derangement-and-mineral-bone-metabolism
#15
Kevin Schulte, Clara Vollmer, Vera Klasen, Jan Hinrich Bräsen, Jodok Püchel, Christoph Borzikowsky, Ulrich Kunzendorf, Thorsten Feldkamp
BACKGROUND: Calcineurin inhibitor (CNI)-induced nephrotoxicity and chronic graft dysfunction with deteriorating glomerular filtration rate (GFR) are common problems of kidney transplant recipients. The aim of this study was to analyze the role of belatacept as a rescue therapy in these patients. METHODS: In this retrospective, observational study we investigated 20 patients (10 females, 10 males) who were switched from a CNI (tacrolimus) to a belatacept-based immunosuppression because of CNI intolerance or marginal transplant function...
August 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28535516/citrate-anticoagulation-during-continuous-renal-replacement-therapy
#16
Davide Ricci, Laura Panicali, Maria Grazia Facchini, Elena Mancini
During extracorporeal dialysis, some anticoagulation strategy is necessary to prevent the coagulation of blood. Heparin has historically been used as an anticoagulant because of its efficacy combined with low cost. However, a variable incidence of hemorrhagic complications (5-30%) has been documented in patients undergoing continuous renal replacement therapy (CRRT) with heparin as an anticoagulant. Citrate has anticoagulation properties secondary to its ability to chelate calcium, which is necessary for the coagulation cascade...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28526688/loss-of-the-anion-exchanger-dra-slc26a3-or-pat1-slc26a6-alters-sulfate-transport-by-the-distal-ileum-and-overall-sulfate-homeostasis
#17
Jonathan M Whittamore, Marguerite Hatch
The ileum is considered the primary site of inorganic sulfate (SO4(2-)) absorption. In the present study, we explored the contributions of the apical chloride/bicarbonate (Cl(-)/HCO3(-)) exchangers DRA (Slc26a3), and PAT1 (Slc26a6), to the underlying transport mechanism. Transepithelial (35)SO4(2-) and (36)Cl(-) fluxes were determined across isolated, short-circuited segments of the distal ileum from wild-type (WT), DRA-knockout (KO) and PAT1-KO mice, together with measurements of urine and plasma sulfate. The WT distal ileum supported net sulfate absorption (197...
May 19, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28516409/survival-after-severe-rhabdomyolysis-following-monensin-ingestion
#18
Michela Blain, Alexander Garrard, Robert Poppenga, Betty Chen, Matthew Valento, Melissa Halliday Gittinger
INTRODUCTION: Monensin is a veterinary antibiotic with a narrow therapeutic window that has led to lethal intoxication in many animal species. Only two prior cases of human toxicity have been reported, both fatal. We present the first case of survival from severe toxicity following monensin ingestion. CASE: A 58-year-old man presented with 8 days of vomiting and abdominal pain. Due to delusions of central nervous system toxoplasmosis, he ingested 300 mg of monensin...
May 17, 2017: Journal of Medical Toxicology: Official Journal of the American College of Medical Toxicology
https://www.readbyqxmd.com/read/28493372/glycosuria-and-hyperglycemia-in-the-neonatal-period-as-the-first-clinical-sign-of-fanconi-bickel-syndrome
#19
María Pilar Bahíllo-Curieses, Rebeca Garrote-Molpeceres, María Miñambres-Rodríguez, M Rosa Del Real-Llorente, Cristina Tobar-Mideros, Sara Rellán-Rodríguez
Fanconi-Bickel syndrome is a rare inherited disease characterized by the combination of hepatorenal glycogen accumulation, proximal renal tubular dysfunction and impaired utilization of glucose and galactose. The first symptoms of the disorder are recognized in late infancy as clinical characteristics appear. Therapeutic approach is mainly conservative with supplements of calcium, phosphate and vitamin D and small frequent feedings to avoid hypoglycemia. We report 1 clinical case of very early diagnosis, a 19 days old baby girl, in which the first clinical sign of the disease was the detection of glycosuria and vomits...
May 11, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28492128/validating-the-utilisation-of-venous-bicarbonate-as-a-predictor-of-acute-kidney-injury-in-crush-syndrome-from-sjambok-injuries
#20
David Lee Skinner, Grant L Laing, John Bruce, Bruce Biccard, David J J Muckart
BACKGROUND: Crush injury secondary to sjambok beatings is a well-described phenomenon in southern Africa. Owing to a number of factors, it can result in acute kidney injury (AKI). In 1992, Muckart et al. described a risk stratification system using venous bicarbonate (VB) that can be used in the management of these patients. OBJECTIVE: To validate this score in the modern era of AKI risk stratification. METHODS: A retrospective study was performed on a local trauma database from June 2010 to December 2012...
April 25, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
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