TITLE

Marked reduction of Tamm-Horsfall protein synthesis in hyperprostaglandin E-syndrome

AUTHOR(S)
Schröter, Jens; Timmermans, Gundi; Seyberth, Hansjörg W.; Greven, Jürgen; Bachmann, Sebastian
PUB. DATE
August 1993
SOURCE
Kidney International;Aug1993, Vol. 44 Issue 2, p401
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Hyperprostaglandin E-syndrome (HPS), a recently described variant of Bartter's syndrome (BS), resembles BS in a number of symptoms but is distinct from BS in others. Similar to BS, HPS is characterized by congenital hypokalemic alkalosis, hypertrophy of the juxtaglomerular apparatus, hyperreninemia, secondary hyperaldosteronism, normal blood pressure and renal diabetes insipidus. Other than BS, HPS is constantly associated with chronic hypercalciuria and nephrocalcinosis as well as both renal and systemic PGE2 overproduction. Correction of most of the symptoms in HPS is achieved by permanent inhibition of prostaglandin synthesis with indomethacin. Among the causes leading to HPS, a selective damage of the distal tubule in HPS has been suggested. Therefore, synthesis of Tamm-Horsfall protein (THP), a glycoprotein exclusively produced in the thick ascending limb of the loop of Henle, was measured by ELISA in the urine of seven infant HPS patients (aged 3 to 8 years). Patients were investigated both under constant indomethacin treatment and after a one week period without indomethacin. Nine healthy children (aged 5 months to 10 years) served as controls. In controls mean daily THP excretion was 54.2 ± 13.9 (median 46.0) mg/24 hr/1.73 m² whereas in HPS, THP levels were strongly diminished. During withdrawal of indomethacin treatment, mean THP level was 12.7 ± 10.1 (median 7.2) mg/24 hr/1.73 m² and 10.3 ± 10.1 (median 3.5) mg/24 hr/1.73 m² under indomethacin treatment, respectively. THP excretion values both without indomethacin and under indomethacin treatment were significantly different from controls (P ≤ 0.005); however, there was no significant difference between the THP levels during or after cessation of indomethacin treatment. Creatinine clearance in HPS patients was 75.1 ± 159 (median 76.2) ml/min/1.73 m² without indomethacin and 81.9 ± 151, (median 83.0) ml/min/1.73 m² under indomethacin treatment. Control values were not obtained. Comparative measurements of THP excretion in six classical BS-patients (aged 3 months to 17 years) revealed normal THP values in two individuals and intermediate levels in the others: the mean level of six BS patients was 30.8 ± 13.5 (median 25.0) mg/24 hr/1.73 m² and was thus significantly higher than in HPS both with and without indomethacin treatment (P ≤ 0.05). Immunohistochemistry in renal biopsies of three of the HPS patients showed a strong reduction of cortical tubular THP immunoreactivity in two cases and a less pronounced reduction in the third. In situ hybridization using a THP-riboprobe in these three biopsies revealed significantly reduced or absent THP-mRNA levels. The severely decreased THP-production supports previous assumptions that one of the major causes underlying HPS is a selective congenital defect of the distal tubule. Differences in THP excretion between HPS and BS support a concept that defines HPS and BS as separate entities with a number of similarities.
ACCESSION #
17967234

 

Related Articles

  • Primary Hyperaldosteronism in the Hypertensive Disease. Fardella, Carlos E.; Carvajal, Cristian A.; Mosso, Lorena M. // Current Hypertension Reviews;2006, Vol. 2 Issue 1, p33 

    Primary aldosteronism (PA) is one of the potentially curable forms of hypertension. The hypertension is secondary to aldosterone action in the kidney, reabsorbing sodium and water that increases the intravascular volume and blood pressure. PA was previously believed to account for less than 1%...

  • Hyperaldosteronism and Hypergranularity of the Juxtaglomerular Cells in Renal Hypertension. Goldberg, Martin; McCurdy, Donna K. // Annals of Internal Medicine;Jul63 Part 1, Vol. 59 Issue 1, p24 

    Focuses on the hyperaldosteronism and hypergranularity of the juxtaglomerular cells in renal hypertension. Data indicating that the hypokalemia and renal potassium wastage manifested as a function of a renal cation exchange mechanism probably influenced by aldosterone; Suggested procedures...

  • “Bartter-like” phenotype in Kearns—Sayre syndrome. Emma, Francesco; Pizzini, Carla; Tessa, Alessandra; di Giandomenico, Silvia; Onetti-Muda, Andrea; Santorelli, Filippo M.; Bertini, Enrico; Rizzoni, Gianfranco // Pediatric Nephrology;Mar2006, Vol. 21 Issue 3, p355 

    Kearns–Sayre syndrome (KSS) is a mitochondrial disease caused by large deletions in mitochondrial DNA (mtDNA). In most patients the disease is characterized by mtDNA heteroplasmy, where a mixture of wild-type and mutated mtDNA co-exist within cells in variable proportion, modulating the...

  • 22-Year-old patient with malignant hypertension associated with primary aldosteronism. Prejbisz, A; Klisiewicz, A; Januszewicz, A; Lenders, J W; Pręgowska-Chwała, B; Jóźwik-Plebanek, K; Michałowska, I; Januszewicz, M; Andziak, P; Hoffman, P; Guzik, T J // Journal of Human Hypertension;Feb2013, Vol. 27 Issue 2, p138 

    The article presents the study on the malignant hypertension with primary aldosteronism (PA). It states that PA can seldom associated with malignant hypertension in which excessive amounts of aldosterone and elevated blood pressure (BP) may have damaging effects on heart and vasculature. It...

  • Regression of left ventricular hypertrophy in patients with primary aldosteronism/low-renin hypertension on low-dose spironolactone. Ori, Yaacov; Chagnac, Avry; Korzets, Asher; Zingerman, Boris; Herman-Edelstein, Michal; Bergman, Michael; Gafter, Uzi; Salman, Hertzel // Nephrology Dialysis Transplantation;Jul2013, Vol. 28 Issue 7, p1787 

    In a world with an elevated salt intake, we may need to redefine both normal aldosterone levels and what in fact constitutes primary aldosteronismBackground The incidence of left ventricular hypertrophy (LVH) in primary aldosteronism (PA) is higher than in essential hypertension. LVH is an...

  • Severe Hypertension with Segmental Renal Infarction Following Surgical Removal of a Retroperitoneal Malignant Hemangiopericytoma: A Case Report. Matsumura, Kiyoshi; Muratani, Hiromi; Kinjou, Kunihiko; Kimura, Yorio; Eto, Tanenao; Fukiyama, Koshiro; Kusaba, Akira // Angiology;Apr1991, Vol. 42 Issue 4, p331 

    Severe hypertension developed in a fifty-five year-old woman after surgical removal of a retroperitoneal tumor, when the renal artery was Injured. Renal arteriography after the surgery demonstrated a segmental infarction of the right kidney. A close relationship between activation of the...

  • El eje renina-angiotensina-aldosterona. Velázquez, Jordi Fernández // AMF: Actualización en Medicina de Familia;2012, Vol. 8 Issue 4, p230 

    No abstract available.

  • Recruited renin-containing renal microvascular cells demonstrate the calcium paradox regulatory phenotype. MacGriff, Spencer; Woo, Richard E.; Ortiz-Capisano, M. Cecilia; Atchison, Douglas K.; Beierwaltes, William H. // Integrated Blood Pressure Control;2014, Vol. 7, p9 

    Renin is the critical regulatory enzyme for production of angiotensin (Ang)-II, a potent vasoconstrictor involved in regulating blood pressure and in the pathogenesis of hypertension. Chronic sodium deprivation enhances renin secretion from the kidney, due to recruitment of additional cells from...

  • The roles of V1a vasopressin receptors in blood pressure homeostasis: a review of studies on V1a receptor knockout mice. Fujiwara, Yoko; Tanoue, Akito; Tsujimoto, Gozoh; Koshimizu, Taka-aki // Clinical & Experimental Nephrology;Feb2012, Vol. 16 Issue 1, p30 

    A prompt rise in blood pressure occurs when arginine-vasopressin is administered in quantities adequate to activate vascular V1a subtype vasopressin receptors. However, it has been controversial whether the endogenous vasopressin-V1a system contributes to the maintenance of basal blood pressure...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

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

Try another library?
Sign out of this library

Other Topics