Elemental Milk Formula as a Possible Cause of Hypophosphatemic Rickets in Wiedemann-Steiner Syndrome

Autor: Al-Juraibah F; King Abdullah Specialist Children’s Hospital, Clinic of Pediatrics, Riyadh, Saudi Arabia; King Saud bin Abdul-Aziz University for Health Sciences, College of Medicine, Riyadh, Saudi Arabia; King Abdullah International Medical Research Center, Riyadh, Saudi Arabia, Melha M; King Abdullah Specialist Children’s Hospital, Clinic of Pediatrics, Riyadh, Saudi Arabia, Alromaih A; King Saud bin Abdul-Aziz University for Health Sciences, College of Medicine, Riyadh, Saudi Arabia, Al-Sunaid A; King Abdullah Specialist Children’s Hospital, Clinic of Pediatrics, Riyadh, Saudi Arabia; King Saud bin Abdul-Aziz University for Health Sciences, College of Medicine, Riyadh, Saudi Arabia; King Abdullah International Medical Research Center, Riyadh, Saudi Arabia, Alkhalaf HA; King Abdullah Specialist Children’s Hospital, Clinic of Pediatrics, Riyadh, Saudi Arabia; King Saud bin Abdul-Aziz University for Health Sciences, College of Medicine, Riyadh, Saudi Arabia; King Abdullah International Medical Research Center, Riyadh, Saudi Arabia
Jazyk: angličtina
Zdroj: Journal of clinical research in pediatric endocrinology [J Clin Res Pediatr Endocrinol] 2024 Sep 05; Vol. 16 (3), pp. 355-360. Date of Electronic Publication: 2023 Feb 16.
DOI: 10.4274/jcrpe.galenos.2022.2022-8-23
Abstrakt: Phosphate has a fundamental role in bone mineralization, and its chronic deficiency has multiple negative consequences in the body, including defects in bone mineralization that will manifest in children as rickets and osteomalacia. Here we present a young boy known to have Wiedemann-Steiner syndrome with multiple co-morbidities that necessitated gastric tube feeding. The child at 22 months was found to have hypophosphatemia and a high alkaline phosphatase level associated with rachitic skeletal manifestations that were attributed to low phosphate intake and/or gastrointestinal absorption, as there was no evidence of excessive phosphate wasting based on appropriate tubular renal re-absorption of phosphate. The primary nutritional source was an elemental amino acid-based milk formula (Neocate ® ) from 12 months of age. After switching from Neocate ® to another elemental amino-acid based milk formula, all biochemical and radiological abnormalities returned to normal, indicating that the Neocate ® formula was the possible cause of the patient’s low phosphate intake. However, in the literature, this formula-associated effect was only described in a limited number of patients. Whether or not some patient-related factors, such as the very rare syndrome described in our patient, could influence this effect warrants further exploration.
Competing Interests: Conflict of interest: None declared.
(©Copyright 2024 by Turkish Society for Pediatric Endocrinology and Diabetes / The Journal of Clinical Research in Pediatric Endocrinology published by Galenos Publishing House.)
Databáze: MEDLINE
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