Universität zu Köln

Kolbuc, Marcin, Lessmeier, Lennart, Salamon-Slowinska, Dorota, Malecka, Ilona, Pawlaczyk, Krzysztof, Walkowiak, Jaroslaw, Wysocki, Jacek, Beck, Bodo B. and Zaniew, Marcin (2020). Hypomagnesemia is underestimated in children with HNF1B mutations. Pediatr. Nephrol., 35 (10). S. 1877 - 1887. NEW YORK: SPRINGER. ISSN 1432-198X

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Abstract

Background Hypomagnesemia in patients with congenital anomalies of the kidneys and urinary tract or autosomal dominant tubulointerstitial kidney disease is highly suggestive of HNF1B-associated disease. Intriguingly, the frequency of low serum Mg2+ (sMg) level varies and is lower in children than in adults with HNF1B mutations that could be partially due to application of inaccurate normal limit of sMg, irrespective of age and gender. We aimed to re-assess cross-sectionally and longitudinally the frequency of hypomagnesemia in HNF1B disease by using locally derived reference values of sMg. Methods Fourteen children with HNF1B-associated kidney disease were included. Control group comprising 110 subjects served to generate 2.5th percentiles of sMg as the lower limits of normal. Results In both controls and patients, sMg correlated with age, gender, and fractional excretion of Mg2+. In girls, sMg concentration was higher than in boys when analyzed in the entire age spectrum (p < 0.05). In HNF1B patients, mean sMg was lower than in controls as compared with respective gender- and age-specific interval (p < 0.001). Low sMg levels (< 0.7 mmol/l) were found in 21.4% of patients at diagnosis and 36.4% at last visit, which rose to 85.7% and 72.7% respectively when using the age- and gender-adjusted reference data. Similarly, in the longitudinal observation, 23% of sMg measurements were < 0.7 mmol/l versus 79.7% when applying respective references. Conclusions Hypomagnesemia is underdiagnosed in children with HNF1B disease. sMg levels are age- and gender-dependent; thus, the use of appropriate reference data is crucial to hypomagnesemia in children.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Kolbuc, Marcin UNSPECIFIED UNSPECIFIED UNSPECIFIED Lessmeier, Lennart UNSPECIFIED UNSPECIFIED UNSPECIFIED Salamon-Slowinska, Dorota UNSPECIFIED UNSPECIFIED UNSPECIFIED Malecka, Ilona UNSPECIFIED UNSPECIFIED UNSPECIFIED Pawlaczyk, Krzysztof UNSPECIFIED UNSPECIFIED UNSPECIFIED Walkowiak, Jaroslaw UNSPECIFIED UNSPECIFIED UNSPECIFIED Wysocki, Jacek UNSPECIFIED UNSPECIFIED UNSPECIFIED Beck, Bodo B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Zaniew, Marcin UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-334092
DOI:	10.1007/s00467-020-04576-6
Journal or Publication Title:	Pediatr. Nephrol.
Volume:	35
Number:	10
Page Range:	S. 1877 - 1887
Date:	2020
Publisher:	SPRINGER
Place of Publication:	NEW YORK
ISSN:	1432-198X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MAGNESIUM; KIDNEY; NEPHROPATHY; COHORT Multiple languages Pediatrics; Urology & Nephrology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/33409