Renal Tubular Acidosis

Soriano, Juan Rodríguez

doi:10.1097/01.asn.0000023430.92674.e5

Cited by 396 publications

(171 citation statements)

References 51 publications

(63 reference statements)

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“…Isolated type 2 proximal RTA, caused by a decrease in the renal threshold for HCO 3 − reabsorption in the absence of alterations in the transport of other solutes, is extremely rare. The vast majority of genetic forms of type 2 proximal RTA are found as a component of Fanconi syndrome caused by inborn metabolic diseases (e.g., cystinosis) rather than isolated proximal RTA.…”

Section: Clinical Approach To Diagnosismentioning

confidence: 99%

“…Type 3 RTA has proximal (type 2 RTA) and distal (type 1 RTA) components. In addition to type 3 RTA caused by loss of function of carbonic anhydrase (CA) 2, as mentioned in Table 1, cases of permanent distal type 1 RTA with transiently impaired proximal reabsorption of HCO 3 − can be found in infants; this form of type 3 RTA should not be considered as a separate entity from distal type 1 RTA. Type 4 hyperkalemic RTA of hereditary origin is most frequently observed in children with resistance to the action of aldosterone, mainly primary pseudohypoaldosteronism (PHA) type 1.…”

Section: Clinical Approach To Diagnosismentioning

confidence: 99%

“…Although in adults RTA is frequently diagnosed in the context of systemic diseases or exposure to drugs or toxins, most pediatric cases correspond to primary disorders resulting from specific genetic defects in a protein involved in the processes of HCO 3 − reabsorption, HCO 3 − regeneration and H + secretion [1].…”

Section: Introductionmentioning

confidence: 99%

“…According to their pathophysiological basis, the following types of RTA are distinguished: type 1 RTA is caused by the inability of the distal convoluted tubule and the collecting tubule to maximally increase the urinary elimination of H + in the presence of metabolic acidosis; type 2 RTA results from impaired HCO 3 − reabsorption in the proximal tubule; type 3 RTA is a mixed form of type 1 and type 2 RTA; type 4 RTA is caused mainly by defective production of ammonium (NH 4 + ) resulting from either aldosterone deficiency or aldosterone resistance [3]. Table 1 summarizes the genetic and molecular basis, as well as the clinical, biochemical, and radiological findings useful to identify the subtype of RTA [1][2][3][4][5][6][7][8][9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

“…Table 1 summarizes the genetic and molecular basis, as well as the clinical, biochemical, and radiological findings useful to identify the subtype of RTA [1][2][3][4][5][6][7][8][9][10][11][12]. Information on acquired forms of RTA secondary to drugs and toxins or associated to systemic diseases is not included because this review mostly deals with congenital primary types of RTA, which are more frequently found in pediatric patients.…”

Section: Introductionmentioning

confidence: 99%

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Clinical and laboratory approaches in the diagnosis of renal tubular acidosis

et al. 2015

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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. Functional tests to explore the proximal wasting of bicarbonate and the urinary acidification capacity are also useful diagnostic tools. Although currently the molecular basis of the disease can frequently be discovered by gene analysis, patients with RTA must undergo a detailed clinical study and laboratory work-up in order to understand the pathophysiology of the disease and to warrant a correct and accurate diagnosis.

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Section: Clinical Approach To Diagnosismentioning

confidence: 99%

Section: Clinical Approach To Diagnosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical and laboratory approaches in the diagnosis of renal tubular acidosis

et al. 2015

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Renal Tubular Acidosis, Sjögren Syndrome, and Bone Disease

Fulop

Mackay²

2004

Arch Intern Med

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Acid–Base Balance Disorders

Hawfield¹,

DuBose²

2010

Encyclopedia of Life Sciences

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Abnormalities in maintaining the pH of the blood in a range that is incompatible with sustaining metabolic processes are known as acid–base balance disorders. Here, we define normal acid–base balance and describe the four basic acid–base balance disorders including metabolic acidosis, metabolic alkalosis, respiratory acidosis and respiratory alkalosis that occur. We suggest a diagnostic approach in defining the disorders and a differential diagnosis to their causes. Routine management of these disorders is also discussed. Key Concepts: Normal Physiologic function depends on the maintenance of a systemic arterial pH between 7.35 and 7.45. The coordination of renal and respiratory regulatory mechanisms is responsible for maintaining physiologic pH. The use of simple equations can differentiate between different acid–base disorders. The causes of metabolic acidosis can be divided among those that lead to a high anion gap and those that present with a normal anion gap. An approach to differentiating the causes of metabolic alkalosis is based on assessing volume status. Respiratory acidosis develops in the setting of hypoventilation. Respiratory alkalosis occurs when carbon dioxide output in the lungs exceeds its metabolic production in the tissues. The ultimate treatment of acid–base disorders is focused on identifying and managing their underlying cause.

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Renal Tubular Acidosis

Cited by 396 publications

References 51 publications

Clinical and laboratory approaches in the diagnosis of renal tubular acidosis

Clinical and laboratory approaches in the diagnosis of renal tubular acidosis

Renal Tubular Acidosis, Sjögren Syndrome, and Bone Disease

Acid–Base Balance Disorders

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