Prevention of Recurrent Calcium Stone Formation with Potassium Citrate Therapy in Patients with Distal Renal Tubular Acidosis

AbstractTen years ago, a 73 year-old patient presented at our unit with a right nephritic colic and elevated serum creatinine (2.1 mg/dl). This was the first time that the patient had consulted for a urology workup. An abdominal X-ray was performed in which we observed a severe bilateral nephrocalcinosis with right ureteral lithiasis. One of the causes of nephrocalcinosis is distal renal tubular acidosis (dRTA), in that sense the patient presented metabolic acidosis (pH 7.25) together with normopotassaemia (4.4 meq/L) and normochlorine (105 mEq/L). A 24-hour urine test detected citrate (55 mg/dl), calcium (12 mg/dl) and pH of 6.5. A diuretic renogram showed the right relative renal function as 91.2% and left relative renal function as 8.8%. A test with bicarbonate and acetazolamide was performed, confirming a diagnosis of dRTA because the urinary CO2 pressure was 32 mmHg (normal, greater than 70 mmHg). Treatment with potassium citrate and increased intake of liquids was prescribed. Consequently, the patient’s serum creatinine normalized, her blood pH rose to 7.35 and urinary citrate increased to 154 mg/dl. After 10 years of treatment with potassium citrate the patient remains stable. We believe that in these patients it is crucial to carry out an alkalizing treatment: patients with dRTA cannot acidify the urine because a defect in the permeability of the tubule membrane prevents secretion of H+. It is important to administer potassium citrate continuously to improve blood pH, increase urinary citrate and reduce the risk of calcium phosphate crystallization.

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THE EFFECT OF LONG TERM ALKALI THERAPY FOR RECURRENT CALCIUM STONE PATIENTS WITH DISTAL RENAL TUBULAR ACIDOSIS

The Japanese Journal of Urology ◽

10.5980/jpnjurol1989.84.674 ◽

1993 ◽

Vol 84 (4) ◽

pp. 674-679

Author(s):

Seiji Yamaguchi ◽

Takuo Koide ◽

Masato Utsunomiya ◽

Toshiaki Yoshioka ◽

Akihiko Okuyama

Keyword(s):

Renal Tubular Acidosis ◽

Distal Renal Tubular Acidosis ◽

Calcium Stone ◽

Alkali Therapy ◽

Renal Tubular

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Prospective long-term evaluation of incomplete distal renal tubular acidosis in idiopathic calcium nephrolithiasis diagnosed (treated) by low-dose NH4CL loading – gender prevalences and impact of alkali treatment

Journal of Nephrology ◽

10.1007/s40620-021-01207-7 ◽

2022 ◽

Author(s):

Juri Sromicki ◽

Georg Kacl ◽

Malin Föhl ◽

Bernhard Hess

Keyword(s):

Calcium Phosphate ◽

Renal Tubular Acidosis ◽

Alkali Treatment ◽

Distal Renal Tubular Acidosis ◽

Urine Ph ◽

Calcium Stone ◽

Stone Formers ◽

Alkali Therapy ◽

Renal Tubular

Abstract Purpose Prospective evaluation of the prevalence of incomplete distal renal tubular acidosis (idRTA) in idiopathic calcium stone formers (ICSF) diagnosed by half-dose ammonium chloride loading (NH4Cl, 0.05 g/kg body weight/day) and impact of alkali treatment of idRTA. Methods Evaluation of 386 consecutive idiopathic calcium stone formers (ICSF) (280 males, 106 females) for idRTA. If screening fasting urine pH was > 5.80, 1-day NH4Cl loading was performed without severe adverse effects. Normally, urine pH falls below 5.45. Results Sixty-four idiopathic calcium stone formers exhibited idRTA, one complete dRTA. Prevalence was higher in women (25.4%) than in men (13.6%). Thus, for more equilibrated comparisons, we formed pairs of 62 idiopathic calcium stone formers (ICSF) with and 62 without idRTA, matched for gender, age, BMI and serum creatinine. Idiopathic calcium stone formers with idRTA more often had hypercalciuria (p < 0.025) and urine citrate < 2 mmol/d (p < 0.05), formed calcium phosphate stones more frequently, exhibited higher numbers of stones/year (1.4 ± 1.5 vs. 0.9 ± 0.8, p = 0.034) and 2.5 times more intrarenal calcifications (4.6 ± 5.9 vs. 1.8 ± 3.6, p = 0.002). All idiopathic calcium stone formers with idRTA were recommended chronic alkali therapy. After 4–15 years of follow-up, stone events /years follow-up (stone passage or urologic intervention) were higher in patients non-adherent to alkali therapy (0.61 ± 0.92) than in patients adherent to treatment (0.11 ± 0.21, p = 0.006). Conclusion Incomplete distal renal tubular acidosis is 1.8-fold more prevalent among female idiopathic calcium stone formers, predicts more stone recurrences, predisposes to calcium phosphate stones and is associated with 2.5 times more intrarenal calcifications vs. non-idRTA patients. Chronic alkali treatment reduces clinical stone recurrences by 5.5 times. Graphical abstract

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Gut It Out: Laxative Abuse Mimicking Distal Renal Tubular Acidosis

Kidney & Blood Pressure Research ◽

10.1159/000501855 ◽

2019 ◽

Vol 44 (5) ◽

pp. 1294-1299 ◽

Cited By ~ 2

Author(s):

Marius Sidler ◽

Nilufar Mohebbi ◽

Ewout J. Hoorn ◽

Carsten A. Wagner

Keyword(s):

Metabolic Acidosis ◽

Renal Tubular Acidosis ◽

Kidney Stones ◽

Relative Number ◽

Potassium Citrate ◽

Distal Renal Tubular Acidosis ◽

Anion Gap ◽

Intercalated Cells ◽

Laxative Abuse ◽

Renal Tubular

Background: Distal renal tubular acidosis (dRTA) can be inherited or acquired. Case Presentation: Here, we describe the case of a 45-year-old female patient with non-anion gap metabolic acidosis, hypokalemia, and alkaline urine. She had a history of rheumatoid arthritis and kidney stones and failed to acidify urine upon the fludrocortisone and furosemide test. Therefore, the diagnosis of dRTA secondary to an autoimmune disease was made. A kidney biopsy was examined for markers of acid-secretory intercalated cells. Surprisingly, no obvious difference in the relative number of acid-secretory intercalated cells or in the distribution of major proteins involved in acid secretion was found. Furthermore, increasing doses of potassium citrate failed to correct the hypokalemia and acidosis. Since these findings were rather atypical for autoimmune dRTA, alternative causes of her hypokalemia and metabolic acidosis were sought. The patient was found to chronically consume laxatives, which can also cause kidney stones and may result in a false-positive urinary acidification test. Conclusion: Chronic laxative abuse may mimic dRTA and should therefore be considered in unexplained hypokalemia with non-anion gap metabolic acidosis.

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Dosage of potassium citrate in the correction of urinary abnormalities in pediatric distal renal tubular acidosis patients

American Journal of Kidney Diseases ◽

10.1053/ajkd.2002.30560 ◽

2002 ◽

Vol 39 (2) ◽

pp. 383-391 ◽

Cited By ~ 33

Author(s):

Somnuek Domrongkitchaiporn ◽

Sookkasem Khositseth ◽

Wasana Stitchantrakul ◽

Wiwat Tapaneya-olarn ◽

Piyanuch Radinahamed

Keyword(s):

Renal Tubular Acidosis ◽

Potassium Citrate ◽

Distal Renal Tubular Acidosis ◽

Renal Tubular ◽

Urinary Abnormalities

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P0003ADHERENCE BENEFITS OF ADV7103, AN INNOVATIVE PROLONGED-RELEASE ORAL COMBINATION PRODUCT, IN PATIENTS WITH DISTAL RENAL TUBULAR ACIDOSIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0003 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Aurélia Bertholet-Thomas ◽

Catherine Guittet ◽

Maria Asunción Manso-Silván ◽

Victor Navas Serrano ◽

Luc André Granier ◽

...

Keyword(s):

Quality Of Life ◽

Renal Tubular Acidosis ◽

Potassium Citrate ◽

Distal Renal Tubular Acidosis ◽

Prolonged Release ◽

Open Label ◽

Advanced Therapy ◽

Renal Tubular

Abstract Background and Aims Distal renal tubular acidosis (dRTA) is a rare disorder leading to impaired net acid excretion by the kidney inducing hyperchloremic metabolic acidosis and hypokalemia. The therapeutic effect of the standard of care is of short duration and requires multiple day and night administrations; it is also often accompanied by gastrointestinal discomfort and poor palatability impacting medication adherence. ADV7103, the first advanced therapy for dRTA, consists of a combination of prolonged-release potassium citrate and prolonged-release potassium bicarbonate granules providing round-the-clock alkali and potassium coverage with twice daily administration. Long-term adherence with ADV7103 is reported, along with acceptability of the product. Method B22CS is a multicentre, open-label long-term extension study, evaluating safety, tolerability, acceptability and efficacy of ADV7103 in adult and paediatric patients with dRTA. Adherence was assessed at each study visit up to 24 months, based on accountability of study drug retrieval, laboratory results, and interview of the patients in a diary and expressed as the proportion of patients that presented adherence lower than 50%, between 50% and 74%, between 75 and 90%, and higher than to 90%. Treatment acceptability as well as quality of life of the patients and their parents were assessed using a 100-mm visual analogue scales (VAS). Results Table 1 shows the evolution of compliance between Month 6 and Month 24. Overall, of the 29 patients remaining in the study after 24 months, 18 (62%) had adherence rates >90%, 5 (17%) had adherence rates of 75-90%, 6 (21%) had adherence rates of 50-74%, and there were no patients with adherence <50%. Adherence was good in all age groups, with rates of ≥75% in 100% of adults, 63% of adolescents 85% of children, and 67% of infants and toddlers. Compared to the alkalising treatments they had before the study, more than 80% of the patients perceived both the improvement of the formulation and of the number of daily doses at scores ≥ 75 mm. The overall improvement of quality of life reported by the patients was of 89 ± 19 mm and that reported by their parents was of 90 ± 14 mm after 24 months of treatment. Conclusion Adherence to treatment was maintained at a high level throughout the 24 months of the study confirming the good acceptance of ADV7103 therapy.

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