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@ARTICLE{Yldz:277745,
author = {Y. Yıldız and O. Kuseyri Hübschmann and A. Akgöz
Karaosmanoğlu and F. Manti and M. Karaca and I. V. D.
Schwartz and R. Pons and E. López-Laso and N. A. J.
Palacios and F. Porta and I. Kavecan and M. C. Balcı and M.
E. Dy-Hollins and S.-N. Wong and M. Oppebøen and L. S.
Medeiros and L. C. P. de Paula and A. García-Cazorla and G.
F. Hoffmann and K. Jeltsch and V. Leuzzi and G. Gökçay and
D. Hübschmann$^*$ and I. Harting and Z. A. Özön and S.
Sivri and T. Opladen},
title = {{L}evodopa-refractory hyperprolactinemia and pituitary
findings in inherited disorders of biogenic amine
metabolism.},
journal = {Journal of inherited metabolic disease},
volume = {47},
number = {3},
issn = {0141-8955},
address = {Hoboken, NJ},
publisher = {Wiley},
reportid = {DKFZ-2023-01462},
pages = {431-446},
year = {2024},
note = {2024 May;47(3):431-446},
abstract = {Elevated serum prolactin concentrations occur in inherited
disorders of biogenic amine metabolism because dopamine
deficiency leads to insufficient inhibition of prolactin
secretion. This work from the International Working Group on
Neurotransmitter Related Disorders (iNTD) presents the
results of the first standardized study on
levodopa-refractory hyperprolactinemia (LRHP; >1000 mU/L)
and pituitary magnetic resonance imaging (MRI) abnormalities
in patients with inherited disorders of biogenic amine
metabolism. Twenty-six individuals had LRHP or abnormal
pituitary findings on MRI. Tetrahydrobiopterin deficiencies
were the most common diagnoses (n = 22). The median age at
diagnosis of LRHP was 16 years (range: 2.5-30, 1st-3rd
quartiles: 12.25-17 years). Twelve individuals (nine
females) had symptoms attributed to hyperprolactinemia:
menstruation-related abnormalities (n = 7), pubertal delay
or arrest (n = 5), galactorrhea (n = 3), and decreased
sexual functions (n = 2). MRI of the pituitary gland was
obtained in 21 individuals; six had
heterogeneity/hyperplasia of the gland, five had adenoma,
and 10 had normal findings. Eleven individuals were treated
with the dopamine agonist cabergoline, ameliorating the
hyperprolactinemia-related symptoms in all those assessed.
Routine monitoring of these symptoms together with prolactin
concentrations, especially after the first decade of life,
should be taken into consideration during follow-up
evaluations. The potential of slow-release levodopa
formulations and low-dose dopamine agonists as part of
first-line therapy in the prevention and treatment of
hyperprolactinemia should be investigated further in animal
studies and human trials. This work adds
hyperprolactinemia-related findings to the current knowledge
of the phenotypic spectrum of inherited disorders of
biogenic amine metabolism.},
keywords = {biogenic amines (Other) / hyperprolactinemia (Other) /
hypogonadism (Other) / neurotransmitter disorders (Other) /
prolactinoma (Other) / tetrahydrobiopterin (BH4) deficiency
(Other)},
cin = {HD01},
ddc = {610},
cid = {I:(DE-He78)HD01-20160331},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37452721},
doi = {10.1002/jimd.12658},
url = {https://inrepo02.dkfz.de/record/277745},
}