Universität zu Köln

Semler, Oliver, Partsch, Carl-Joachim, Das, Anibh Martin, Prechtl, Andreas and Grasemann, Corinna ORCID: 0000-0003-1793-4603 (2021). Cross-sectional analysis: clinical presentation of children with persistently low ALP levels. J. Pediatr. Endocrinol. Metab., 34 (12). S. 1559 - 1567. BERLIN: WALTER DE GRUYTER GMBH. ISSN 2191-0251

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Abstract

Objectives: Low activity of serum alkaline phosphatase (ALP) is a hallmark of hypophosphatasia (HPP), but low readings of ALP are not always recognized in clinical routine. Understanding the clinical presentations associ-ated with low ALP may contribute to a timelier diagnosis of HPP. Methods: Data from paediatric patients with low ALP, excluding patients in intensive care and with oncological/ haematological disorders, were analysed. Most recent ALP values, previous diagnoses, medication and relevant symptoms were extracted from patient records at nine specialised centres and analysed descriptively. A rela-tionship between body height and ALP values was scruti-nised by linear regression. Results: Of 370 children, 15 (4.1%) had a diagnosis of HPP. In the subgroup without a diagnosis of HPP, 241(67.9%) out of 355 patients had one or more medical conditions known to be associated with low serum ALP. Of those, hypothyroid-ism, malnutrition and steroid administration were most frequent. Characteristic symptoms, particularly, short stat-ure, muscle weakness and delay of motor development were more frequent and ALP values were lower in patients with documented HPP diagnosis compared to patients without diagnosis of HPP (Oz-scores: -2.52) (interquartile range [IQR] = 0.20) vs. -1.96 (IQR = 0.87). A weak positive linear relationship between z-scores of ALP and body height was identified (p<0.001). Conclusions: This analysis of paediatric patient records elucidates a wide range of disorders associated with low ALP activity. In case of additional specific symptoms, HPP should always be considered as a differential diagnosis.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Semler, Oliver UNSPECIFIED UNSPECIFIED UNSPECIFIED Partsch, Carl-Joachim UNSPECIFIED UNSPECIFIED UNSPECIFIED Das, Anibh Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Prechtl, Andreas UNSPECIFIED UNSPECIFIED UNSPECIFIED Grasemann, Corinna UNSPECIFIED orcid.org/0000-0003-1793-4603 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-586921
DOI:	10.1515/jpem-2021-0330
Journal or Publication Title:	J. Pediatr. Endocrinol. Metab.
Volume:	34
Number:	12
Page Range:	S. 1559 - 1567
Date:	2021
Publisher:	WALTER DE GRUYTER GMBH
Place of Publication:	BERLIN
ISSN:	2191-0251
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ALKALINE-PHOSPHATASE ACTIVITY; REFERENCE INTERVALS; HYPOPHOSPHATASIA; POPULATION; DIAGNOSIS Multiple languages Endocrinology & Metabolism; Pediatrics Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/58692