Nephropathologic Characteristics of a Woman with Bartter’s Syndrome after Prolonged Treatment with Spironolactone

Abstract

A study was made of two renal biopsy specimens obtained from a 22-year-old woman with Bartter’s syndrome, the first to substantiate the diagnosis, the second 2 years later after the treatment with spironolactone. The first renal biopsy revealed remarkable hyperplasia and hypertrophy of the juxtaglomerular apparatus. The numerous proliferating cells were characterized by abundant lysosomal granules and dilated endoplasmic reticulum. The macula densa revealed slight proliferation, with narrow intercellular space. The ultrastructural picture of the second biopsy specimen showed increased lipofuscin-like granules in the juxtaglomerular cells, with epitheloid cells of the macula densa showing degeneration and irregular dilatation of the intercellular spaces. However, the light microscopic finding was compatible with that of the first. These findings suggest that the treatment was inadequate although clinical features or biochemical laboratory data were improved.

Characteristic Changes of the Juxtaglomerular Cells before and after Treatment of Pseudo-Bartter’s Syndrome due to Furosemide Abuse

“…There were significant differences between the lightmicroscopic appearance of the renal specimens obtained in the first and the second biopsies which is inconsistent with a previous report about a patient with Bartter's syndrome [6].…”

The pathophysiological and molecular basis of Bartter's and Gitelman's syndromes

“…Potassium supplementation usually fails to normalise serum K + concentrations. Addition of potassium-sparing diuretics, eg, amiloride, triamterene or spironolactone, improves serum K + concentrations19 20 but normalisation remains elusive. In a proportion of those patients with associated hypomagnesaemia, magnesium (Mg ++ ) supplementation augments the partial K + correction (box FB4).…”

Effective medical treatment strategies to help cessation of purging behaviors

“…This condition is incurable, but treatment is aimed at correcting the electrolyte abnormalities that lead to discomfort and may possibly result in death. It is focused on a combination of four agents: Potassium supplements, and spironolactone . Thus, treatment with nonsteroidal anti‐inflammatory agents, and by the use of angiotensin‐converting enzyme (ACE) inhibitors such as lisinopril …”

“…It is focused on a combination of four agents: Potassium supplements, 11 and spironolactone. 12 Thus, treatment with nonsteroidal anti-inflammatory agents, 13,14 and by the use of angiotensin-converting enzyme (ACE) inhibitors such as lisinopril. 15,16 Relevant to patients with bulimia nervosa, as well as to patients with any eating disorder with purging behaviors, over the past five decades, since the original description by Bartter, there have been reported cases of hypokalemia with biochemical and histological markers exactly similar to Bartter syndrome, but specifically caused by chronic volume depleted states provoked by extrarenal factors such as self-induced vomiting, laxative abuse, and diuretic abuse.…”