+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Gitelman syndrome: when will it turn into Gitelman disease?

Gitelman syndrome: when will it turn into Gitelman disease?

Pediatric Nephrology 18(7): 613-616

Chronic renal failure (CRF) disrupts the time-dependent secretion of multiple hormones. The present review focuses on altered pulsatile release of peptide hormones. CRF is marked by impaired tissue actions, disorderly release patterns, and relative [growth hormone (GH)] or absolute [luteinizing hormone (LH)] deficiency of secretion. At the hypothalamo-pituitary level, experimental evidence suggests that CRF reduces the synthesis and/or release of the cognate hypothalamic releasing factors, GHRH and LHRH, and enforces excessive inhibition by somatostatin. Parathyroid hormone (PTH) and insulin are secreted in both basal and pulsatile modes, wherein the latter is putatively coordinated by autonomic innervation. Amplitude and frequency-dependent adaptations of PTH and insulin outflow fail in CRF, as assessed under steady-state conditions and during metabolic drive (i.e., calcium for PTH and glucose for insulin). A common feature in CRF is a diminished mass of hormone released per burst, due in principle to attenuation of feedforward signals and/or accentuation of (unknown) feedback signals. Damping of neuronal control and/or prolonged network response times may contribute to aberrant pulse frequency, disproportionate basal (nonpulsatile) hormone release, and consistent erosion of secretory process regularity in the uremic state. The homeostatic consequences of distorted secretory dynamics, tissue resistance, impaired hormone clearance, and altered mean agonist concentrations are evident in certain therapeutic interventions, such as GH supplementation in CRF.

(PDF emailed within 0-6 h: $19.90)

Accession: 010716724

Download citation: RISBibTeXText

PMID: 12728368

DOI: 10.1007/s00467-003-1171-8

Related references

Mutations in SLC12A3 and CLCNKB and Their Correlation with Clinical Phenotype in Patients with Gitelman and Gitelman-like Syndrome. Journal of Korean Medical Science 31(1): 47-54, 2016

Is Gitelman's syndrome an acquired disease?. British Journal of Rheumatology 37(7): 794, 1998

Graves' disease and Gitelman syndrome. Clinical Endocrinology 84(1): 149-150, 2016

New SLC12A3 disease causative mutation of Gitelman's syndrome. World Journal of Nephrology 5(6): 551-555, 2016

Magnesium, cardiovascular-renal disease and the Gitelman's syndrome paradox. Journal of Hypertension 35(5): 1122-1124, 2018

Management of uncommon disorders in pregnancy: Von Hippel-Lindau disease, Gitelman syndrome, and Nutcracker syndrome. Obstetric Medicine 10(3): 138-141, 2017

Gitelman syndrome, calcium pyrophosphate dihydrate deposition disease and crowned dens syndrome. A new association?. Rheumatology 49(3): 610-613, 2010

Gitelman syndrome. An overlooked disease with chronic hypomagnesemia and hypokalemia in adults. Ugeskrift for Laeger 165(11): 1123-1127, 2003

Coexistence of Graves' Disease in a 14-year-old young girl with Gitelman Syndrome. Clinical Endocrinology 83(6): 995-997, 2016

The Importance of Chronic Magnesium Deficiency in human disease and the Gitelman's syndrome paradox. Qjm 2018, 2018

A Case of Gitelman's Syndrome that was difficult to distinguish from Hypokalemic Periodic Paralysis due to Graves' Disease. Journal of Nippon Medical School 2019, 2019

Gitelman syndrome disclosed by calcium pyrophosphate deposition disease: early diagnosis by ultrasonographic study. Reumatismo 68(1): 53-55, 2017

Familiar association of Gitelman's syndrome and calcium pyrophosphate dihydrate crystal deposition disease--a case report. Rheumatology 46(9): 1506-1508, 2007

Gitelman syndrome: consensus and guidance from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney International 91(1): 24-33, 2017

Gitelman disease associated with growth hormone deficiency and empty sella A new hereditary renal tubular pituitary syndrome?. Pediatric Nephrology 12(7): C67, Sept, 1998