Literature DB >> 32869320

Central hypothyroidism improves with age in very young children with Prader-Willi syndrome.

Ayako Konishi1,2, Shinobu Ida1,3, Yasuko Shoji1, Yuri Etani1, Masanobu Kawai1,4.   

Abstract

OBJECTIVE: Abnormalities in the hypothalamic-pituitary-thyroid (HPT) axis have been implicated in Prader-Willi syndrome (PWS); however, limited information is currently available on age-dependent alterations in the HPT axis. We herein investigated age-dependent differences in thyroid hormone levels in PWS children. DESIGN/PATIENTS/MEASUREMENTS: Free T4 (FT4), free T3 (FT3) and thyroid-stimulating hormone (TSH) concentrations were retrospectively compared between genetically confirmed PWS children (N = 43, median age: 11.2 months) and controls (N = 85, median age: 14.5 months) matched for age, sex, body weight-SD score (SDS), height-SDS, body mass index-SDS and serum albumin level, a marker of the nutritional status. Subjects were subdivided into two groups based on their age: an infant group aged between 1 and 11 months (PWS: N = 22, controls: N = 30) and a toddler group aged between 12 and 47 months (PWS: N = 21, controls: N = 55). None of the subjects had ever been treated with growth hormone or levothyroxine.
RESULTS: After adjustments for confounding variables, in the infant group, FT4 levels (pmol/L) were significantly lower in PWS (11.24 in PWS vs 14.32 in controls, P = .0002), whereas no significant differences were observed in FT3 or TSH levels. In the toddler group, no significant differences were noted in FT4 (12.23 in PWS vs 15.31 in controls, P = .10), FT3 or TSH levels. The FT3/FT4 ratio was significantly increased in PWS in both groups. FT4 levels were positively correlated with age in PWS.
CONCLUSIONS: Infants with PWS had lower FT4 levels, but FT3 levels were normal, indicating that the levothyroxine replacement therapy may not need to be routinely performed.
© 2020 John Wiley & Sons Ltd.

Entities:  

Keywords:  Prader-Willi syndrome; central hypothyroidism; hypothalamic-pituitary-thyroid axis

Mesh:

Substances:

Year:  2020        PMID: 32869320     DOI: 10.1111/cen.14323

Source DB:  PubMed          Journal:  Clin Endocrinol (Oxf)        ISSN: 0300-0664            Impact factor:   3.478


  3 in total

1.  Thyroid function in children with Prader-Willi syndrome in Southern China: a single-center retrospective case series.

Authors:  Xiaojian Mao; Li Liu; Xinjiang Huang; Xi Yin; Dongyan Wu; Yanna Cai; Xiuzhen Li; Wen Zhang; Chunhua Zeng
Journal:  BMC Pediatr       Date:  2022-04-29       Impact factor: 2.567

2.  Genetic subtypes and phenotypic characteristics of 110 patients with Prader-Willi syndrome.

Authors:  Lu Zhang; Xiaoliang Liu; Yunjing Zhao; Qingyi Wang; Yuanyuan Zhang; Haiming Gao; Bijun Zhang; Wanting Cui; Yanyan Zhao
Journal:  Ital J Pediatr       Date:  2022-07-23       Impact factor: 3.288

3.  Epilepsia Partialis Continua a Clinical Feature of a Missense Variant in the ADCK3 Gene and Poor Response to Therapy.

Authors:  Mahmoud Reza Ashrafi; Roya Haghighi; Reza Shervin Badv; Homa Ghabeli; Ali Reza Tavasoli; Elham Pourbakhtyaran; Zahra Rezaei; Nejat Mahdieh; Pouria Mohammadi; Morteza Heidari
Journal:  J Mol Neurosci       Date:  2022-03-11       Impact factor: 2.866

  3 in total

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