Diagnosis, treatment and follow-up in a patient with Nephrocalcinosis due to distal renal tubular acidosis

Open Medicine ◽  
2011 ◽  
Vol 6 (6) ◽  
pp. 804-806
Author(s):  
Miguel Arrabal-Polo ◽  
Miguel Arrabal-Martin ◽  
Antonio Jiménez-Pacheco ◽  
Sergio Merino-Salas

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.

Urolithiasis ◽  
1989 ◽  
pp. 509-509
Author(s):  
P. Malasit ◽  
S. Nilwarangkur ◽  
S. Ong-Aj-Yooth ◽  
W. Susaengrat ◽  
S. Vasuvattakul ◽  
...  

1992 ◽  
Vol 262 (3) ◽  
pp. F449-F453 ◽  
Author(s):  
E. Dafnis ◽  
M. Spohn ◽  
B. Lonis ◽  
N. A. Kurtzman ◽  
S. Sabatini

Considerable evidence supports the presence of an H(+)-K(+)-ATPase along the mammalian nephron. Inhibition of this enzyme might be expected to reduce acid excretion while increasing potassium excretion, thus causing hypokalemic distal renal tubular acidosis (RTA). In this study we administered vanadate at a dose of 5 mg/kg ip for 10 days to rats. These animals developed hypokalemic distal RTA with a blood pH of 7.22 +/- 0.01, a plasma bicarbonate of 15.2 +/- 0.6 meq/l, and a plasma potassium of 3.28 +/- 0.06 meq/l. The vanadate-treated animals had a urine pH of 6.70 +/- 0.09, a value significantly higher than NH4Cl-treated animals with the same degree of acidemia (urine pH = 5.25 +/- 0.04). When cortical collecting tubules (CCT) from these animals were microdissected and H(+)-K(+)-ATPase was measured, it was decreased by approximately 75% (P less than 0.001); but H(+)-ATPase was no different from control. In medullary collecting tubule, H(+)-K(+)-ATPase was also decreased but less than in CCT. Muscle potassium concentration in the vanadate-treated animals was significantly lower than in controls. These results demonstrate that vanadate causes hypokalemic distal RTA in association with inhibition of collecting tubule H(+)-K(+)-ATPase activity.


1978 ◽  
Vol 92 (3) ◽  
pp. 394-400 ◽  
Author(s):  
Michael E. Norman ◽  
Nathan I. Feldman ◽  
Robert M. Cohn ◽  
Karl S. Roth ◽  
Donna K. McCurdy

2019 ◽  
Vol 44 (5) ◽  
pp. 1294-1299 ◽  
Author(s):  
Marius Sidler ◽  
Nilufar Mohebbi ◽  
Ewout J. Hoorn ◽  
Carsten A. Wagner

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.


2002 ◽  
Vol 39 (2) ◽  
pp. 383-391 ◽  
Author(s):  
Somnuek Domrongkitchaiporn ◽  
Sookkasem Khositseth ◽  
Wasana Stitchantrakul ◽  
Wiwat Tapaneya-olarn ◽  
Piyanuch Radinahamed

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Aurélia Bertholet-Thomas ◽  
Catherine Guittet ◽  
Maria Asunción Manso-Silván ◽  
Victor Navas Serrano ◽  
Luc André Granier ◽  
...  

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.


PEDIATRICS ◽  
2003 ◽  
Vol 112 (6) ◽  
pp. 1361-1367 ◽  
Author(s):  
L. Cheidde ◽  
T. C. Vieira ◽  
P. R. M. Lima ◽  
S. T. O. Saad ◽  
I. P. Heilberg

2001 ◽  
Vol 59 (3) ◽  
pp. 1086-1093 ◽  
Author(s):  
Somnuek Domrongkitchaiporn ◽  
Chonlatrip Pongsakul ◽  
Wasana Stitchantrakul ◽  
Vorachai Sirikulchayanonta ◽  
Boonsong Ongphiphadhanakul ◽  
...  

2021 ◽  
Author(s):  
Wencong Guo ◽  
Qijing Song ◽  
Ruixiao Zhang ◽  
Qing Xin ◽  
Zhiying Liu ◽  
...  

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