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The Journal of Pediatrics Apr 2014
Topics: Acidosis, Renal Tubular; Child; Humans
PubMed: 24345454
DOI: 10.1016/j.jpeds.2013.10.085 -
Lancet (London, England) Sep 1983
Topics: Acidosis, Renal Tubular; Connective Tissue Diseases; Humans
PubMed: 6136753
DOI: No ID Found -
Endocrinology and Metabolism Clinics of... Dec 1990Renal tubular acidosis refers to a group of disorders that result from pure tubular damage without concomitant glomerular damage. They could be hereditary (primary) or... (Review)
Review
Renal tubular acidosis refers to a group of disorders that result from pure tubular damage without concomitant glomerular damage. They could be hereditary (primary) or acquired (secondary to various disease states like sickle cell disease, obstructive uropathy, postrenal transplant, autoimmune disease, or drugs). The hallmark of the disorder is the presence of hyperchloremic metabolic acidosis with, or without, associated defects in potassium homeostasis, a UpH greater than 5.5 in the presence of systemic acidemia, and absence of an easily identifiable cause of the acidemia. There are three physiologic types whose basic defects are impairment of or a decrease in acid excretion, i.e., type 1 (dRTA); a failure in bicarbonate reabsorption, i.e., type 2 (pRTA); and deficiency of buffer or impaired generation of NH4+, i.e., type 4 RTA. Several pathophysiologic mechanisms have been postulated for these various types. pRTA is the least common of all in the adult population. It rarely occurs as an isolated defect. It is frequently accompanied by diffuse proximal tubule transport defects with aminoaciduria, glycosuria, hyperphosphaturia, and so forth (Fanconi syndrome). dRTA is associated with a high incidence of nephrolithiasis, nephrocalcinosis, osteodystrophy, and growth retardation (in children). Osteodystrophy also occurs in pRTA to a lesser degree and is believed to be secondary to hypophosphatemia. Patients with type 4 RTA usually have mild renal insufficiency from either diabetes mellitus or interstitial nephritis. Acute bicarbonate loading will result in a high fractional excretion of bicarbonate greater than 15% (FEHCO3- greater than 15%) in patients with pRTA, but FEHCO3- less than 3% in patients with dRTA. Type I patients will also have a low (U - B) PCO2 with bicarbonate loading. They are also unable to lower their urine pH to less than 5.5 with NH4Cl loading. The treatment of these patients involves avoidance of precipitating factors when possible, treatment of underlying disease, correction of electrolyte imbalance, particularly hypokalemia and hyperkalemia, and most importantly, the use of alkali. This will prevent or reduce all the various complications.
Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Bone Diseases; Humans; Kidney; Nephrocalcinosis
PubMed: 2081516
DOI: No ID Found -
Nephrology, Dialysis, Transplantation :... May 2022
Topics: Acidosis; Acidosis, Renal Tubular; Humans; Kidney Diseases
PubMed: 33313681
DOI: 10.1093/ndt/gfaa309 -
Advances in Chronic Kidney Disease Jul 2018Renal tubular acidosis (RTA) represents a group of diseases characterized by (1) a normal anion gap metabolic acidosis; (2) abnormalities in renal HCO absorption or new... (Review)
Review
Renal tubular acidosis (RTA) represents a group of diseases characterized by (1) a normal anion gap metabolic acidosis; (2) abnormalities in renal HCO absorption or new renal HCO generation; (3) changes in renal NH, Ca, K, and HO homeostasis; and (4) extrarenal manifestations that provide etiologic diagnostic clues. The focus of this review is to give a general overview of the pathogenesis of the various clinical syndromes causing RTA with a particular emphasis on type I (hypokalemic distal RTA) and type II (proximal) RTA while reviewing their pathogenesis from a physiological "bottom-up" approach. In addition, the factors involved in the generation of metabolic acidosis in both type I and II RTA are reviewed highlighting the importance of altered renal ammonia production/partitioning and new HCO generation. Our understanding of the underlying tubular transport and extrarenal abnormalities has significantly improved since the first recognition of RTA as a clinical entity because of significant advances in clinical acid-base chemistry, whole tubule and single-cell H/base transport, and the molecular characterization of the various transporters and channels that are functionally affected in patients with RTA. Despite these advances, additional studies are needed to address the underlying mechanisms involved in hypokalemia, altered ammonia production/partitioning, hypercalciuria, nephrocalcinosis, cystic abnormalities, and CKD progression in these patients.
Topics: Acid-Base Imbalance; Acidosis, Renal Tubular; Ammonia; Ammonium Compounds; Animals; Bicarbonates; Biological Transport; Calcium; Citric Acid; Humans; Hypercalciuria; Hypokalemia; Ketoglutaric Acids; Kidney Tubules, Distal; Kidney Tubules, Proximal; Sodium-Bicarbonate Symporters
PubMed: 30139460
DOI: 10.1053/j.ackd.2018.05.005 -
NeoReviews Feb 2024See Bonus NeoBriefs videos and downloadable teaching slides Metabolic acidosis can manifest in the neonatal period and cause significant morbidity and mortality in... (Review)
Review
See Bonus NeoBriefs videos and downloadable teaching slides Metabolic acidosis can manifest in the neonatal period and cause significant morbidity and mortality in neonates. Preterm infants are at an even higher risk of developing metabolic acidosis. If the acidosis results from a dysfunction of acid-base homeostasis by the renal system, the disorder is known as renal tubular acidosis (RTA). In this review, we will describe renal development and normal acid-base homeostasis by the renal system. We will also discuss the pathophysiology of the different types of RTA, laboratory findings to aid in diagnosis, and treatment considerations. Understanding RTA will help neonatal clinicians recognize and diagnose an infant affected by RTA and initiate treatment in a timely manner.
Topics: Infant; Humans; Infant, Newborn; Acidosis, Renal Tubular; Infant, Premature; Kidney; Homeostasis
PubMed: 38296789
DOI: 10.1542/neo.25-2-e99 -
World Journal of Pediatrics : WJP Oct 2019Distal renal tubular acidosis (dRTA) is a kidney tubulopathy that causes a state of normal anion gap metabolic acidosis due to impairment of urine acidification. This... (Review)
Review
BACKGROUND
Distal renal tubular acidosis (dRTA) is a kidney tubulopathy that causes a state of normal anion gap metabolic acidosis due to impairment of urine acidification. This review aims to summarize the etiology, pathophysiology, clinical findings, diagnosis and therapeutic approach of dRTA, with emphasis on genetic causes of dRTA.
DATA SOURCES
Literature reviews and original research articles from databases, including PubMed and Google Scholar. Manual searching was performed to identify additional studies about dRTA.
RESULTS
dRTA is characterized as the dysfunction of the distal urinary acidification, leading to metabolic acidosis. In pediatric patients, the most frequent etiology of dRTA is the genetic alteration of genes responsible for the codification of distal tubule channels, whereas, in adult patients, dRTA is more commonly secondary to autoimmune diseases, use of medications and uropathies. Patients with dRTA exhibit failure to thrive and important laboratory alterations, which are used to define the diagnosis. The oral alkali and potassium supplementation can correct the biochemical defects, improve clinical manifestations and avoid nephrolithiasis and nephrocalcinosis.
CONCLUSIONS
dRTA is a multifactorial disease leading to several clinical manifestations. Clinical and laboratory alterations can be corrected by alkali replacement therapy.
Topics: Acidosis, Renal Tubular; Adolescent; Anion Exchange Protein 1, Erythrocyte; Child; Humans; Mutation; Vacuolar Proton-Translocating ATPases
PubMed: 31079338
DOI: 10.1007/s12519-019-00260-4 -
Dimensions of Critical Care Nursing :... 2010Renal tubular acidosis is a relatively uncommon clinical syndrome characterized by the inability of the kidney to adequately excrete hydrogen ions, retain adequate...
Renal tubular acidosis is a relatively uncommon clinical syndrome characterized by the inability of the kidney to adequately excrete hydrogen ions, retain adequate bicarbonate, or both. This syndrome can be categorized into 3 separate disorders, each with unique clinical characteristics. Although an uncommon finding, prompt and inexpensive tests can lead to early intervention and subsequently reduce complications from persistent renal dysfunction. The purpose of this article was to bring awareness of the clinical manifestations, diagnosis, and treatments of renal tubular acidosis to critical care nurses.
Topics: Acidosis; Acidosis, Renal Tubular; Aged; Critical Care; Diagnosis, Differential; Early Diagnosis; Female; Humans; Hyperaldosteronism; Kidney Tubules; Middle Aged; Nursing Assessment
PubMed: 20395726
DOI: 10.1097/DCC.0b013e3181d24b62 -
The Journal of International Medical... Mar 2021We report the case of a family in which two sisters have distal renal tubular acidosis (dRTA). Familial dRTA is a rare disorder, with both autosomal dominant and...
We report the case of a family in which two sisters have distal renal tubular acidosis (dRTA). Familial dRTA is a rare disorder, with both autosomal dominant and recessive transmission. This is a report of familial dRTA from China.
Topics: Acidosis, Renal Tubular; China; Humans; Mutation
PubMed: 33726529
DOI: 10.1177/03000605211000533 -
La Revue de Medecine Interne Jan 2014Renal tubular acidosis (RTAs) are a group of metabolic disorders characterized by metabolic acidosis with normal plasma anion gap. There are three main forms of RTA: a... (Review)
Review
Renal tubular acidosis (RTAs) are a group of metabolic disorders characterized by metabolic acidosis with normal plasma anion gap. There are three main forms of RTA: a proximal RTA called type II and a distal RTA (type I and IV). The RTA type II is a consequence of the inability of the proximal tubule to reabsorb bicarbonate. The distal RTA is associated with the inability to excrete the daily acid load and may be associated with hyperkalaemia (type IV) or hypokalemia (type I). The most common etiology of RTA type IV is the hypoaldosteronism. The RTAs can be complicated by nephrocalcinosis and obstructive nephrolithiasis. Alkalinization is the cornerstone of treatment.
Topics: Acidosis; Acidosis, Renal Tubular; Adult; Child; Diagnosis, Differential; Female; Humans; Hypokalemia; Male; Middle Aged
PubMed: 24070792
DOI: 10.1016/j.revmed.2013.08.012