Renal cysts and nephrocalcinosis in a patient with Bartter syndrome type III

Watanabe, Toru; Tajima, Toshihiro
May 2005
Pediatric Nephrology;May2005, Vol. 20 Issue 5, p676
Academic Journal
Chronic hypokalemia is known to induce renal cyst formation in some diseases including primary aldosteronism, distal renal tubular acidosis, Liddle disease and apparent mineralocorticoid excess syndrome. Although chronic hypokalemia is the main clinical feature of Bartter syndrome, renal cyst formation in this disease has never been reported. We describe a patient with classic Bartter syndrome who exhibited renal cysts and nephrocalcinosis. Direct sequencing analysis of the chloride channel CLC-Kb gene identified a heterozygous nonsense mutation (W610X) in exon 16 indicating a diagnosis of Bartter syndrome type III. Although the precise mechanism underlying the development of renal cysts in our patient remains unclear, chronic hypokalemia and nephrocalcinosis may contribute to cyst development.


Related Articles

  • Gastroileostomy Leading to Severe Potassium Depletion and Renal Tubular Acidosis. Kovnat, Paul J.; Tin, Aurora Dee // American Journal of Gastroenterology;Apr1973, Vol. 59 Issue 4, p340 

    Presents a case of a patient with renal tubular acidosis (RTA) without renal potassium wasting which reversed with partial treatment of hypokalemia. Result of physical examination of the patient; Information on renal tubular acidosis; Characteristics of proximal or type II RTA.

  • Functional up-regulation of basolateral Na+-dependent HCO3- transporter NBCn1 in medullary thick ascending limb of K+-depleted rats. Jakobsen, Jakob K.; Odgaard, Elvin; Wang, Weidong; Elkjaer, Marie Louise; Nielsen, Søren; Aalkjaer, Christian; Leipziger, Jens // Pflugers Archiv European Journal of Physiology;Sep2004, Vol. 448 Issue 6, p571 

    The electroneutral, Na+-coupled HCO3- co-transporter (NBCn1) is expressed in the basolateral membrane of rat medullary thick ascending limb (mTAL) segments and is up-regulated strongly during metabolic acidosis. We have suggested previously that NBCn1-mediated HCO3- uptake across the basolateral...

  • Adaptive change in ammonia excretion in renal insufficiency. MacLean, Alan J.; Hayslett, John P. // Kidney International;May1980, Vol. 17 Issue 5, p595 

    Experiments were performed to study the mechanism of the compensatory increase in the excretion of metabolic acid by residual nephrons after reduction in renal mass. Despite a decrease in nephron population to 20% of control, total excretion of acid remained similar to pair-fed controls due to...

  • Potassium-related inherited tubulopathies. Landau, D. // Cellular & Molecular Life Sciences;Sep2006, Vol. 63 Issue 17, p1962 

    Hyper- and hypokalemia may carry severe clinical consequences. Different regulatory mechanisms, including the kidney, exert a tight regulation of plasma potassium levels. The renal pathway of potassium handling begins in the proximal tubule followed by the fine-tuning of its secretion or...

  • A New Syndrome: Angiotensin-Converting Enzyme Dysfunction Syndrome: Differential Diagnosis and Pathogenesis--Case Reports. Umeki, Shigenobu; Terao, Akira; Sawayama, Toshitami // Angiology;Jan1988 Part 1, Vol. 39 Issue 1, p58 

    The authors previously reported a new syndrome, angiotensin-converting enzyme dysfunction syndrome (ACEDS), which is clinically characterized by mild systemic hypertension, a hypokalemic alkalosis, and hyperreninism with a high concentration of angiotensin-I (ANG-I), a normal angiotensin-II...

  • Renal Tubular Acidosis: Practical Guides to Diagnosis and Treatment. Hirschman, Gladys H.; Rao, David D.; Oyemade, Olusola; Chan, James C. M. // Clinical Pediatrics;Jul1976, Vol. 15 Issue 7, p645 

    The syndrome of renal tubular acidosis in some one of its various forms should be suspected when an infant or child has failure to thrive, metabolic acidosis, constipation, diarrhea, vomiting, anorexia, polyuria, or dehydration in infancy. Confirmatory biochemical findings include an...

  • Glutamine transport in the rabbit proximal straight tubule: Effect of acute acid pH. Kuwahara, michio; Sasaki, Sei; Shiigai, Tatsuo; Takeuchi, Jugoro // Kidney International;Sep1986, Vol. 30 Issue 3, p340 

    Proximal straight tubules (PST) has been shown to be an important nephron segment of renal ammonia production. To clarify the nature of glutamine (substrate of ammonia production) transport in PST, both luminal resorption and peritubular uptake of gluatamine were measured in isolated rabbit PST....

  • Renal tubular acidosis induced by dietary chloride. Toto, Robert D.; Hulte, Henry N.; Mackie, Sandra; Sebastian, Anthony // Kidney International;Jan1984, Vol. 25 Issue 1, p26 

    Previous studies have demonstrated that dietary intake of anions with high renal reabsorbability (Cl- > SO4=) can result in either exacerbation of chronic metabolic acidosis or correction of chronic metabolic alkalosis. These results, however, fail to predict the renal acid-base response to Cl-...

  • Chronic adaptations in proximal tubular H/HCO[SUB3] transporters. Alpern, Robert J.; Horie, Shigeo; Moe, Orson; Tejedor, Alberto; Miller, R. Tyler; Preisig, Patricia A. // Kidney International Supplement;Jul1991, Issue 33, pS-29 

    Chronic acidosis, chronic K deficiency, and chronic hyper- filtration all lead to similar parallel increases in Na/H antiporter and Na/3HCO3 cotransporter activities. These effects persist when the transporters are removed from the inducing environment, and thus represent memory effects. The...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics