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Renal tubular acidosis, type 4

Autumn N Harris, P Richard Grimm, Hyun-Wook Lee, Eric Delpire, Lijuan Fang, Jill W Verlander, Paul A Welling, I David Weiner
Background Hyperkalemia in association with metabolic acidosis that are out of proportion to changes in glomerular filtration rate defines type 4 renal tubular acidosis (RTA), the most common RTA observed, but the molecular mechanisms underlying the associated metabolic acidosis are incompletely understood. We sought to determine whether hyperkalemia directly causes metabolic acidosis and, if so, the mechanisms through which this occurs. Methods We studied a genetic model of hyperkalemia that results from early distal convoluted tubule (DCT)-specific overexpression of constitutively active Ste20/SPS1-related proline-alanine-rich kinase (DCT-CA-SPAK)...
May 2018: Journal of the American Society of Nephrology: JASN
Kenrick Berend
Background: Normal anion gap metabolic acidosis is a common but often misdiagnosed clinical condition associated with diarrhea and renal tubular acidosis (RTA). Early identification of RTA remains challenging for inexperienced physicians, and diagnosis and treatment are often delayed. Summary: The presence of RTA should be considered in any patient with a high chloride level when the CL- /Na+ ratio is above 0.79, if the patient does not have diarrhea. In patients with significant hyperkalemia one should evaluate for RTA type 4, especially in diabetic patients, with a relatively conserved renal function...
December 2017: Kidney Diseases
Yin Ping Liew, Timothy A Rogers, Jane L Garb, Holley F Allen, Edward O Reiter, Thomas J Campfield, Vikas R Dharnidharka, Gregory L Braden
BACKGROUND: We identified two boys with type 3 renal tubular acidosis (RTA) and growth hormone deficiency and we sought to differentiate them from children with classic type 1 distal RTA. METHODS: We reviewed all children <6 years of age with RTA referred over a 13-year period and compared the growth response to alkali therapy in these two boys and in 28 children with only type 1 distal RTA. RESULTS: All children with type 1 RTA reached the 5th percentile or higher on CDC growth charts within 2 years of alkali therapy...
October 26, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
Ganesh Pathare, Nasser Dhayat, Nilufar Mohebbi, Carsten A Wagner, Lydie Cheval, Thomas J Neuhaus, Daniel G Fuster
It is well known that pendrin, an apical Cl- /HCO3 - exchanger in type B intercalated cells, is modulated by chronic acid-base disturbances and electrolyte intake. To study this adaptation further at the acute level, we analyzed urinary exosomes from individuals subjected to oral acute acid, alkali, and NaCl loading. Acute oral NH4 Cl loading (n = 8) elicited systemic acidemia with a drop in urinary pH and an increase in urinary NH4 excretion. Nadir urinary pH was achieved 5 h after NH4 Cl loading. Exosomal pendrin abundance was dramatically decreased at 3 h after acid loading...
February 2018: Pflügers Archiv: European Journal of Physiology
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
Thomas McDonnell, Chukwuma Chukwu, Christopher Wong
This case report details a 51-year-old man with Alport's syndrome resulting in chronic nephritis with stable renal function and sensorineural deafness. The patient was being investigated for persistently raised potassium refractory to dietary and pharmacological modification. Subsequently, the patient was found to have type 4 renal tubular acidosis, and potassium normalised with the addition of fludrocortisone.
May 31, 2017: BMJ Case Reports
S J H Dobbin, J R Petrie, M E J Lean, G A McKay
BACKGROUND: Type 4 renal tubular acidosis causes hyperkalaemia, for which diabetes and medications commonly used in this patient group are aetiological factors. Here we describe the novel use of fludrocortisone in this difficult condition. CASE REPORT: A 55-year-old woman with complex co-morbidities, including Type 2 diabetes (HbA1c 37 mmol/mol 5.5%), was admitted with renal failure. Bloods on admission: eGFR 25 ml/min, creatinine 184 ?mol/L, urea 35.9 mmol/L, sodium 128 mmol/L, potassium 5...
July 2017: Diabetic Medicine: a Journal of the British Diabetic Association
Ranga M Weerakkody, Pushpa N Lokuliyana, Ruchika D Lanerolle
Hump-nosed viper (Hypnale hypnale; HNV) is one of the six major snake species in Sri Lanka that cause envenomation. Nephrotoxicity, coagulopathy, and neurotoxicity are wellrecognized features of its envenomation. Type 4 renal tubular acidosis (RTA4) has only once been described previously in this condition, and we report two further cases. Two patients aged 53 and 51 presented following HNV bites with acute kidney injury and microangiopathic hemolytic anemia. Both underwent multiple cycles of hemodialysis until the polyuric phase was reached...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
Ira Shah, Forum Shah
We present four children with tyrosinemia and their response to NTBC [2-[2-nitro-4-trifluoromethylbenzoyl]-1, 3-cyclohexanedione]. One child received NTBC for only 3 months after which he was diagnosed to have hepatocellular carcinoma and underwent a living-related liver transplantation. The child is doing well post-transplant. Remaining three children have been on NTBC for almost 3 years, have normal liver functions, undetectable urine succinylacetone, and no portal hypertension or renal tubular acidosis...
May 2016: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Adam Daniel Jakes, Kevin Baynes, Catherine Nelson-Piercy
We describe the clinical course of renal tubular acidosis (RTA) type 4 in pregnancy, which has not been previously published. Renal tubular acidosis type 4 is a condition associated with increased urinary ammonia secondary to hypoaldosteronism or pseudohypoaldosteronism. Pregnancy may worsen the hyperkalaemia and acidosis of renal tubular acidosis type 4, possibly through an antialdosterone effect. We advise regular monitoring of potassium and pH throughout pregnancy to ensure safe levels are maintained.
March 17, 2016: BMJ Case Reports
Sumit Mohan
No abstract text is available yet for this article.
December 17, 2015: New England Journal of Medicine
Martijn C G J Brouwers
No abstract text is available yet for this article.
December 17, 2015: New England Journal of Medicine
Angela M Righi, Michael J Peeters, Bryan Dotson
No abstract text is available yet for this article.
December 17, 2015: New England Journal of Medicine
Mark L Graber
No abstract text is available yet for this article.
December 17, 2015: New England Journal of Medicine
Biff F Palmer, Deborah J Clegg
No abstract text is available yet for this article.
December 17, 2015: New England Journal of Medicine
Nasser A Dhayat, Andre Schaller, Giuseppe Albano, John Poindexter, Carolyn Griffith, Andreas Pasch, Sabina Gallati, Bruno Vogt, Orson W Moe, Daniel G Fuster
Mutations in the vacuolar-type H(+)-ATPase B1 subunit gene ATP6V1B1 cause autosomal-recessive distal renal tubular acidosis (dRTA). We previously identified a single-nucleotide polymorphism (SNP) in the human B1 subunit (c.481G>A; p.E161K) that causes greatly diminished pump function in vitro To investigate the effect of this SNP on urinary acidification, we conducted a genotype-phenotype analysis of recurrent stone formers in the Dallas and Bern kidney stone registries. Of 555 patients examined, 32 (5.8%) were heterozygous for the p...
May 2016: Journal of the American Society of Nephrology: JASN
Shatha Hussain Ali, Fadhil Shanan Hussien, Haider Abd Al-Amer
Renal disease in hospitalized children can be difficult to diagnose early as it may exhibit few symptoms, unlike in adults. This study reports the epidemiological data, percentages and types of renal disorders in children seen at the pediatric nephrology center of the AlKadhymia Teaching Hospital, Baghdad, Iraq. A retrospective review of the charts of all patients, aged between one month and 14 years, who were admitted and followed-up for a period of three years from January 2009 till January 2012 were studied...
May 2015: Saudi Journal of Kidney Diseases and Transplantation
Amel Ben Chehida, Takoua Bensmaïl, Faten Ben Rehouma, Rim Ben Abdelaziz, Hatem Azzouz, Hela Boudabbous, Mohamed Slim Abdelmoula, Sonia Abdelhak, Naziha Kaabachi, Hadhami Ben Turkia, Néji Tebib
AIM: To investigate risk factors of renal complications in glycogen storage disease type I, in order to identify practical implications for renal preservation. METHODS: A retrospective study of 38 patients with glycogen storage disease type I. RESULTS: The patients studied were 8.6 years old in average (1.5 to 22 years) and were followed during 7.4 ± 4.5 years. Hypercalciuria was detected in 23 patients and was related to acidosis (P=0.028), higher lactate levels (5...
July 2015: Néphrologie & Thérapeutique
Fernando Santos, Flor A Ordóñez, Débora Claramunt-Taberner, Helena Gil-Peña
In the absence of a gastrointestinal origin, a maintained hyperchloremic metabolic acidosis must raise the diagnostic suspicion of renal tubular acidosis (RTA). Unlike adults, in whom RTA is usually secondary to acquired causes, children most often have primary forms of RTA resulting from an inherited genetic defect in the tubular proteins involved in the renal regulation of acid-base homeostasis. According to their pathophysiological basis, four types of RTA are distinguished. Distal type 1 RTA, proximal type 2 RTA, mixed-type 3 RTA, and type 4 RTA can be differentiated based on the family history, the presenting manifestations, the biochemical profile, and the radiological findings...
December 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Y-W Fang, S-S Yang, T Chau, M Nakamura, O Yamazaki, G Seki, H Yamada, H-M Hsu, C-J Cheng, S-H Lin
We created Na(+)/HCO3(-) cotransporter 1 (NBCe1) p.W516* knock-in mice as a model of isolated proximal renal tubular acidosis showing early lethality associated with severe metabolic acidosis to investigate the therapeutic effects of prenatal alkalization or posttranscriptional control 124 (PTC124). NBCe1(W516*/W516*) mice were treated with non-alkalization (control, n=12), prenatal alkalization postcoitus (prenatal group, n=7) and postnatal alkalization from postnatal day 6 (postnatal group, n=12). Mutation-specific therapy, PTC124 (60 mg kg(-1)) or gentamicin (30 mg kg(-1)), was administered intraperitoneally from postnatal day 6...
May 2015: Gene Therapy
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