Adem Yasin Köksoy, Orhan Görükmez
No abstract text is available yet for this article.
February 23, 2021: Pediatric Nephrology
Adem Yasin Köksoy, Orhan Görükmez
No abstract text is available yet for this article.
February 23, 2021: Pediatric Nephrology
Renjithkumar Kalikkot Thekkeveedu, Sumana Ramarao, Nilesh Dankhara, Pradeep Alur
Diuretic therapy, commonly used in the newborn intensive care unit, is associated with a variety of electrolyte abnormalities such as hyponatremia, hypokalemia, and hypochloremia. Hypochloremia, often ignored, is associated with significant morbidities and increased mortality in infants and adults. Clinicians respond in a reflex manner to hyponatremia than to hypochloremia. Hypochloremia is associated with nephrocalcinosis, hypochloremic alkalosis, and poor growth. Besides, the diuretic resistance associated with hypochloremia makes maintaining chloride levels in the physiological range even more logical...
2021: Global Pediatric Health
Muthuvel Jayachandran, Stanislav V Yuzhakov, Sanjay Kumar, Nicholas B Larson, Felicity T Enders, Dawn S Milliner, Andrew D Rule, John C Lieske
No abstract text is available yet for this article.
February 18, 2021: Orphanet Journal of Rare Diseases
Elvira O Gosmanova, Pascal Houillier, Lars Rejnmark, Claudio Marelli, John P Bilezikian
A systematic literature review was performed to summarize the frequency and nature of renal complications in patients with chronic hypoparathyroidism managed with conventional therapy. Methodology was consistent with the recommendations outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Peer-reviewed journal articles with specified medical subject heading terms were identified using the PubMed, EMBASE, and Cochrane databases. Data were extracted from eligible articles based on prespecified parameters for clinical outcomes of renal calcifications and disease...
February 18, 2021: Reviews in Endocrine & Metabolic Disorders
Mònica Vall-Palomar, Leire Madariaga, Gema Ariceta
Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC; OMIM 248250) is a rare autosomal recessive kidney disease caused by mutations in the CLDN16 or CLDN19 genes encoding the proteins claudin-16 and claudin-19, respectively. These are involved in paracellular magnesium and calcium transport in the thick ascending limb of Henle's loop and account for most of the magnesium reabsorption in the tubules. FHHNC is characterized by hypomagnesaemia, hypercalciuria, and nephrocalcinosis, and progresses to kidney failure, requiring dialysis and kidney transplantation mainly during the second to third decades of life...
February 17, 2021: Pediatric Nephrology
Chiddarwar Dr Poonam, Dillibabu Ethiraj, Niranjan Dhanaji Kanase, Venkatraman Indiran
Primary hyperoxaluria (PH) is a rare metabolic disorder with autosomal recessive inheritance pattern which is due to deficiency of alanine-glyoxylate aminotransferase enzyme. It causes defective glyoxylate metabolism in liver which in turn leads to excessive oxalate production and deposition. Supersaturation of oxalic acid in urine (>45 mg/day) is known as hyperoxaluria which causes nephrolithiasis, cortical nephrocalcinosis and renal insufficiency. Secondary hyperoxaluria is due to over ingestion of oxalic acids or its precursors or can be due to its reduced excretion...
February 8, 2021: QJM: Monthly Journal of the Association of Physicians
Yoshitaka Iwazu, Makoto Kuro-O, Yutaka Miura, Shin-Ichi Takeda, Toshiyuki Yamada, Daisuke Nagata
In patients with sarcoidosis, dysregulated calcium metabolism is one of the frequently observed complications. However, little attention has been paid to abnormal phosphate metabolism. Herein we present the case of a 42-year-old Japanese man with renal sarcoidosis who developed acute kidney injury due to hypercalcemia and nephrolithiasis. Laboratory data showed hypercalcemia with a normal serum phosphate level and high serum 1,25-hydroxyvitamin D3 , fibroblast growth factor 23 (FGF23) and calciprotein particle (CPP) levels...
January 2021: Clinical Kidney Journal
Fengyuan Wu, Ying Zhang, Yunpeng Cheng, Yan Lu, Yinong Jiang, Wei Song
RATIONALE: Medullary sponge kidney (MSK) is a congenital renal disorder characterized by recurrent nephrolithiasis or nephrocalcinosis. Recently, it has been found that MSK can be also combined with other diseases, such as primary aldosteronism and Beckwith-Wiedemann, but whether it is associated with secondary hypertension remains unknown. PATIENT CONCERNS: A 22-year-old hypertensive female presented to our hospital characterized by hypokalemia and hypertension...
January 22, 2021: Medicine (Baltimore)
Prince Singh, Jason K Viehman, Ramila A Mehta, Andrea G Cogal, Linda Hasadsri, Devin Oglesbee, Julie B Olson, Barbara M Seide, David J Sas, Peter C Harris, John C Lieske, Dawn S Milliner
BACKGROUND: Primary hyperoxaluria type 3 (PH3) is caused by mutations in the HOGA1 gene. PH3 patients often present with recurrent urinary stone disease (USD) in first decade of life, but prior reports suggested PH3 may have a milder phenotype in adults. The current study characterized clinical manifestations of PH3 across the decades of life in comparison to PH1 and PH2. METHODS: Clinical information was obtained from the Rare Kidney Stone Consortium Primary Hyperoxaluria Registry (PH1 n = 384; PH2 n = 51; PH3 n = 62)...
February 5, 2021: Nephrology, Dialysis, Transplantation
Alexander D Chesover, Jennifer Harrington, Farid H Mahmud
Background: Subcutaneous fat necrosis (SCFN) can be complicated by severe hypercalcemia, which is frequently asymptomatic. Nephrocalcinosis is associated with hypercalcemia and, in other clinical settings, has been linked to furosemide and glucocorticoid use. First-line bisphosphonate therapy treating hypercalcemia in neonatal SCFN is not well described. Objectives: To describe the biochemical changes and risk of nephrocalcinosis in infants with hypercalcemia, secondary to neonatal SCFN, treated with initial pamidronate...
February 2021: Paediatrics & Child Health
Adem Yasin Köksoy, Derya Bako, Servet Yel
No abstract text is available yet for this article.
February 2, 2021: Pediatric Nephrology
Adem Yasin Köksoy, Derya Bako, Servet Yel
No abstract text is available yet for this article.
February 2, 2021: Pediatric Nephrology
Mahmut Gok, Omer Ayten, Ozkan Onur, Hakki Cetinkaya, Gulistan Gumrukcu, Gulizar Sahin
Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Renal involvement in sarcoidosis patients is occurred, but the incidence and prevalence is uncertain. The most common renal involvement of systemic sarcoidosis is nephrocalcinosis and interstitial nephritis. After sarcoidosis was diagnosed in a 31-year-old male patient, we performed a renal biopsy because of nephrotic range proteinuria and renal dysfunction. The collapsing variant of focal segmental glomerulosclerosis (FSGS) secondary to sarcoidosis was diagnosed by kidney biopsy...
January 2021: Iranian Journal of Kidney Diseases
A Çağlar, H Tuğçe Çağlar
INTRODUCTION: Vitamin D intoxication (VDI) is a well-known cause of hypercalcemia in children and leads to serious kidney, heart, and neurological problems. In the treatment of VDI, the goal is to correct hypercalcemia. Our aim was to evaluate the clinical features of patients with VDI, identify the causes of VDI in our region, and help guide precautions and treatment of VDI. MATERIALS AND METHODS: The medical records of patients with VDI presenting between January 2015 and December 2019 were retrospectively analyzed...
January 19, 2021: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Stan Benjamens, Ines F Antunes, Jan-Luuk Hillebrands, Melanie Reijrink, Marian L C Bulthuis, Stefan P Berger, Cyril Moers, Martin H de Borst, Riemer H J A Slart, Robert A Pol
Nephrocalcinosis is present in up to 43% of kidney allograft biopsies at one-year after transplantation and is associated with inferior graft function and poor graft survival. We studied [18 F]-sodium fluoride ([18 F]-NaF) imaging of microcalcifications in donor kidneys (n = 7) and explanted kidney allografts (n = 13). Three µm paraffin-embedded serial sections were used for histological evaluation of calcification (Alizarin Red; Von Kossa staining) and ex-vivo [18 F]-NaF autoradiography. The images were fused to evaluate if microcalcification areas corresponded with [18 F]-NaF uptake areas...
January 19, 2021: Scientific Reports
Carlos R Ferreira, Dillon Kavanagh, Ralf Oheim, Kristin Zimmerman, Julian Stürznickel, Xiaofeng Li, Paul Stabach, R Luke Rettig, Logan Calderone, Colin MacKichan, Aaron Wang, Hunter A Hutchinson, Tracy Nelson, Steven M Tommassini, Simon von Kroge, Imke A K Fiedler, Ethan R Lester, Gilbert W Moeckel, Björn Busse, Thorsten Schinke, Thomas O Carpenter, Michael A Levine, Mark C Horwowitz, Demetrios T Braddock
Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and Autosomal Recessive Hypophosphatemic Rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc...
January 19, 2021: Journal of Bone and Mineral Research
Mitsuru Muto, Tatsuru Kaji, Shun Onishi, Keisuke Yano, Waka Yamada, Satoshi Ieiri
Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients...
January 19, 2021: Surgery Today
Catherine Monet-Didailler, Jean-François Chateil, Lise Allard, Astrid Godron-Dubrasquet, Jérôme Harambat
Nephrocalcinosis is defined by calcium phosphate or calcium oxalate deposits in the kidney parenchyma, particularly in tubular epithelial cells and interstitial tissue. It should be differentiated from urolithiasis where calcium salts deposits are located in the kidney and urinary tract. The epidemiology of nephrocalcinosis in children is unknown but the condition is not so rare, with an increased incidence in preterm infants. Often detected as an incidental finding, nephrocalcinosis may be classified according to the radiological type: medullary, cortical or diffuse...
February 2021: Néphrologie & Thérapeutique
Giulia Magni, Robert J Unwin, Shabbir H Moochhala
Renal tubular acidosis (RTA) is a set of raredis orders in which the renal tubule is unable to excreteacid normally and there by maintain normal acid-basebalance, resulting in a complete or incomplete metabolicacidosis. In distal RTA (dRTA, also known as classicalor type 1 RTA), there is a defect in excreting H+ ionsalong the distal nephron (distal tubule and collectingduct), leading to an alkaline urinary pH with calcium phosphate precipitation and stones. Causes of dRTAinclude genetic mutations, autoimmune disease, and some drugs...
January 2021: Archivos Españoles de Urología
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