Abstract:
:Congenital chloride diarrhea is due to mutations in the intestinal Cl(-)/HCO(3)(-) exchange (SLC26A3) which results in sodium chloride and fluid depletion leading to hypochloremic and hypokalemic metabolic alkalosis. Although treatment with sodium and potassium chloride offers protection from renal involvement in childhood, the long-term renal outcome remains unclear. Here we describe two cases of congenital chloride diarrhea-associated end-stage renal disease with transplantation. Further, we show that there is a high incidence of mild chronic kidney disease in 35 other patients with congenital chloride diarrhea. The main feature of the renal injury was nephrocalcinosis, without hypercalciuria or nephrolithiasis with small sized kidneys and commensurately reduced glomerular filtration rates. This suggests that diarrhea-related sodium chloride and volume depletion, the first signs of non-optimal salt substitution, promote urine supersaturation and crystal precipitation. The poor compliance with salt substitution along with long-lasting hypochloremic and hypokalemic metabolic alkalosis is likely to induce progressive calcification and renal failure. Both our patients developed nephrocalcinosis in the transplanted kidneys suggesting that this complication is a consequence of intestinal SLC26A3 deficiency. Interestingly, the transporter is expressed in the distal nephron but the recurrence of nephrocalcinosis in the transplanted kidney suggests that it does not play a significant renal role in this syndrome.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Wedenoja S,Ormälä T,Berg UB,Halling SF,Jalanko H,Karikoski R,Kere J,Holmberg C,Höglund Pdoi
10.1038/ki.2008.401subject
Has Abstractpub_date
2008-10-01 00:00:00pages
1085-93issue
8eissn
0085-2538issn
1523-1755pii
S0085-2538(15)53478-9journal_volume
74pub_type
杂志文章abstract::Our genetic hypercalciuric (GH) rats have been selected and inbred for 29 generations to maximize urine calcium (UCa) excretion compared to identical gender controls (Ctl). To determine the effect of the increased UCa on urinary supersaturation and stone formation, we pair fed 15 GH and 15 Ctl rats a standard 1.2% cal...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1995.468
更新日期:1995-12-01 00:00:00
abstract::In early clinical testing, acute addition of alanyl-glutamine (AlaGln) to glucose-based peritoneal dialysis (PD) fluids restored peritoneal cellular stress responses and leukocyte function. This study was designed to test the effect of extended treatment with AlaGln-supplemented PD fluid on biomarkers of peritoneal he...
journal_title:Kidney international
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1016/j.kint.2018.08.031
更新日期:2018-12-01 00:00:00
abstract::From August 1987 through February 1995 we performed 42 surgical procedures in 29 patients with occluded or stenotic radiocephalic arteriovenous fistulae. Operations were designed to preserve native veins for cannulation (Group I) or to preserve access in the same forearm, bypassing the failed fistula (Group II). For 2...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00769.x
更新日期:1998-02-01 00:00:00
abstract::Nephronophthisis is an autosomal recessive chronic tubulointerstitial disease that progresses to end-stage renal disease (ESRD) in about 10% of cases during infancy. Mutations in the INVS (NPHP2) gene were found in a few patients with infantile nephronophthisis. Mutations of NPHP3, known to be associated with adolesce...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2008.662
更新日期:2009-04-01 00:00:00
abstract::Chronic kidney disease (CKD) accelerates muscle protein degradation by stimulating the ubiquitin proteasome system through activation of the E3 ligases, Atrogin-1/MAFbx and MuRF-1. Forkhead transcription factors (FoxOs) can control the expression of these E3 ligases, but the contribution of individual FoxOs to muscle ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2012.84
更新日期:2012-08-01 00:00:00
abstract::Gap junctions are present in the juxtaglomerular apparatus enabling intercellular communication. Our study determined the location of different connexin subtypes within the juxtaglomerular apparatus of the rat, and the role of these subtypes in renal hemodynamics through the use of specific mimetic peptides. Immunohis...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002673
更新日期:2008-02-01 00:00:00
abstract::Iron is critical for cellular, organismal, and possibly universal existence. Use of iron complexes to treat human diseases is ancient and is described in detail in Ayurveda/Siddha systems of medicine. Old aphorisms from Siddha medicine ("Alavukku Minjinal Amirdhamum Nanjagum," an elixir turns poisonous when taken in e...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2016.02.035
更新日期:2016-07-01 00:00:00
abstract:BACKGROUND:Hyperhomocysteinemia is a risk factor for atherosclerosis that is common in chronic renal failure (CRF), but its cause is unknown. Homocysteine metabolism is linked to betaine-homocysteine methyl transferase (BHMT), a zinc metalloenzyme that converts glycine betaine (GB) to N,N dimethylglycine (DMG). DMG is ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2001.00743.x
更新日期:2001-06-01 00:00:00
abstract:BACKGROUND:Inhibition of the renin-angiotensin system (RAS) prevents development of chronic allograft dysfunction in experimental animals. Whether this therapeutic approach is effective even if started when signs of allograft nephropathy are already manifested has not been investigated. METHODS:To address this issue, ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2003.00318.x
更新日期:2003-12-01 00:00:00
abstract::The hormonal responsiveness profile of the cortical collecting duct varies from one species to another. To identify the hormones and agonists that modulate the functions of this tubule segment in the human species, we generated a cell line (HCD) immortalized by SV40 virus. The tubular origin of this cell line was asse...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1995.186
更新日期:1995-05-01 00:00:00
abstract:BACKGROUND:Human immunodeficiency virus-associated nephropathy (HIVAN) has become the third leading cause of end-stage renal disease (ESRD) in African Americans, and is expected to grow exponentially. Highly active antiretroviral therapy (HAART) has significantly prolonged the survival of patients with HIV infection. D...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2005.00245.x
更新日期:2005-04-01 00:00:00
abstract::31P NMR as a descriptive technique is of interest to nephrologists. Particular contributions of 31P NMR to our understanding of renal function may be enumerated.: "Free" metabolite levels are different from those classically accepted; in particular, ADP and Pi are low with implications for the control of renal metabol...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1038/ki.1986.15
更新日期:1986-01-01 00:00:00
abstract::Spleen tyrosine kinase (SYK) is an important component of the intracellular signaling pathway for various immunoreceptors. Inhibition of SYK has shown promise in preclinical models of autoimmune and glomerular disease. However, the description of SYK expression in human renal tissue, which would be desirable ahead of ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2015.29
更新日期:2015-07-01 00:00:00
abstract::Podocyte effacement and the reformation of foot processes and slit diaphragms can be induced within minutes experimentally. Therefore, it seems likely that the slit diaphragm proteins underlie orchestrated recycling mechanisms under the control of posttranslational modifiers. One of these modifiers, SUMO (small ubiqui...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2014.198
更新日期:2014-12-01 00:00:00
abstract::Kidney transplant patients receiving phenytoin or phenobarbital may have decreased graft survival. These drugs have been shown to enhance the metabolism of glucocorticoids. We determined the disposition of total and unbound prednisolone in six stable kidney transplant patients receiving prednisone for immunosuppressio...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1984.17
更新日期:1984-01-01 00:00:00
abstract:BACKGROUND:The fluid filling renal cysts in human polycystic kidneys is secreted chiefly by the tubular epithelium lining the cysts via secondary chloride transport. Inhibiting this process by somatostatin therapy should induce shrinking of renal cysts. METHODS:In this randomized, cross-over, placebo-controlled trial ...
journal_title:Kidney international
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1111/j.1523-1755.2005.00395.x
更新日期:2005-07-01 00:00:00
abstract::A major factor contributing to failure of arteriovenous fistulas (AVFs) is migration of smooth muscle cells into the forming neointima. To identify the source of smooth muscle cells in neointima, we created end-to-end AVFs by anastomosing the common carotid artery to the jugular vein and studied neural crest-derived s...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2015.73
更新日期:2015-09-01 00:00:00
abstract::Using in situ hybridization and immunocytochemistry we describe the renal localization of the PDGF alpha-receptor. PDGF alpha-receptor mRNA was uniformly present in human metanephric kidney in interstitial cells and vascular arcades that course through the blastema. PDGF alpha-receptor mRNA was present in some mesangi...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1997.157
更新日期:1997-04-01 00:00:00
abstract::Quartan malarial infection causes an immune complex nephritis in some individuals, which, once established, is sustained by mechanisms not yet fully explained, but which probably involve autoimmune processes. The presenting clinical and biochemical findings of the quartan malarial nephrotic syndrome are similar to tho...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1979.103
更新日期:1979-07-01 00:00:00
abstract:BACKGROUND:Chronic oversecretion of parathyroid hormone (PTH) is associated with parathyroid hyperplasia, reflecting a disturbed balance between cell proliferation and apoptosis. This study addressed the unsolved issue of apoptosis in hyperparathyroidism. METHODS:Parathyroid glands from 19 patients with primary (1 deg...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2000.00863.x
更新日期:2000-02-01 00:00:00
abstract::Genetic variation at the MYH9 locus is linked to the high incidence of focal segmental glomerulosclerosis (FSGS) and non-diabetic end-stage renal disease among African Americans. To further define risk alleles with FSGS we performed a genome-wide association analysis using more than one million single-nucleotide polym...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2010.251
更新日期:2010-10-01 00:00:00
abstract:BACKGROUND:The aim of this study was to determine the glucose-dependent regulation of the sodium-proton-antiporter (Na+/H+ antiporter) in patients with mild chronic renal failure (CRF). METHODS:We measured plasma glucose concentrations, plasma insulin concentrations, plasma C peptide concentrations, arterial blood pre...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1999.00510.x
更新日期:1999-07-01 00:00:00
abstract::The biological action of calcitriol is mediated through a hormone-receptor complex interacting with nuclear chromatin. Interaction of the calcitriol receptor (VDR) with VDR response elements produces bioactive proteins which carry out the physiological actions of calcitriol. Since biological response to calcitriol app...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1994.252
更新日期:1994-07-01 00:00:00
abstract::Although angiotensin-converting enzyme (ACE) inhibitors are well-established drugs in the treatment of hypertension, they are not supposed to be sufficient in the inhibition of aldosterone formation. The present study analyzes the effect of aldosterone receptor antagonist, spironolactone and ACE inhibitor, captopril o...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5001513
更新日期:2006-07-01 00:00:00
abstract:BACKGROUND:In X-linked Alport syndrome, mutations in the COL4A5 gene encoding the alpha 5 chain of type IV collagen result in progressive renal failure. This nephropathy appears to relate to the arrest of a switch from an alpha 1/alpha 2 to an alpha 3/alpha 4/alpha 5 network of type IV collagen in the developing glomer...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00188.x
更新日期:1998-12-01 00:00:00
abstract::The impact of dialysis on cognitive trends is uncertain. Kurella Tamura et al. evaluated cognitive trajectories in 212 participants from the Chronic Renal Insufficiency Cohort study and found that dialysis initiation was associated with a decline in executive function. This is consistent with a cerebrovascular basis f...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2017.01.022
更新日期:2017-04-01 00:00:00
abstract::Acute kidney injury (AKI), previously termed acute renal failure, is associated with increased mortality, prolonged hospital stay, and accelerated chronic kidney disease (CKD). Over the past 2 decades, dramatic rises in the incidences of AKI have been reported, particularly within the United States. The question arise...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1038/ki.2014.293
更新日期:2015-01-01 00:00:00
abstract::Using a combination of intra-operative digital photography and micro-biopsy we measured renal cortical and papillary changes in five patients with primary hyperparathyroidism and abundant calcium phosphate kidney stones. Major tissue changes were variable papillary flattening and retraction, dilation of the ducts of B...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2008.161
更新日期:2008-07-01 00:00:00
abstract::Bovine serum albumin (BSA, pI 4.9) was maleylated to yield highly anionic MBSA (pI 3.0). Maleylation of BSA lead to an expansion of molecular size of native BSA from an effective molecular radius (EMR) of 37 A to 57 A for MBSA as assessed by gel filtration chromatography. MBSA, but not BSA, bound to the peripheral cap...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1988.161
更新日期:1988-08-01 00:00:00
abstract:BACKGROUND:Recent advances enable the direct measurement of small molecule clearance, Kecn, during each dialysis. Average Kecn and treatment length, t, are multiplied giving total clearance, Kt. The body surface area (BSA) is a fixed transformation of height and weight and is a well recognized measure of body size. Thi...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1111/j.1523-1755.2005.00533.x
更新日期:2005-09-01 00:00:00