European Journal of Pediatrics

, Volume 177, Issue 5, pp 775–779 | Cite as

Hyperthyrotropinemia in newly diagnosed cystic fibrosis patients with pancreatic insufficiency reversed by enzyme therapy

  • Aris GiannakopoulosEmail author
  • Anni Katelaris
  • Maria Noni
  • Theodore Karakonstantakis
  • Christina Kanaka-Gantenbein
  • Stavros Doudounakis
Original Article


Patients with cystic fibrosis (CF) commonly present with an elevated TSH concentration, suggesting subclinical hypothyroidism. Its relation to concomitant pancreatic insufficiency and its natural course upon initiation of enzyme replacement have not been adequately studied. Herein, we investigated the thyroid function in newly diagnosed infants with CF and monitored the course of thyroid function response to pancreatic enzyme substitution treatment. Fourteen, newly diagnosed infants with CF and pancreatic insufficiency, were followed every 6–8 weeks for 6 months ensuing onset of pancreatic enzyme substitution therapy. All infants had normal TSH values on neonatal screening. Ten out of 14 (71%) had hyperthyrotropinemia and normal freeT4 values at presentation. No patient received thyroxine. Upon follow-up, after 6 months, TSH values normalized in 90% of infants with CF and hyperthyrotropinemia. Serum selenium levels were negatively correlated with TSH levels.

Conclusion: Mild TSH elevation is a frequent finding in newly diagnosed cystic fibrosis patients with pancreatic insufficiency during infancy. TSH elevation resolves in most cases after initiation of enzyme substitution and improvement of nutritional status without any substitutive therapy with thyroxine.

What is Known:

Newly diagnosed infants with cystic fibrosis often present with a state of hyperthyrotropinemia suggesting subclinical hypothyroidism.

What is New:

Pancreatic enzyme substitution and improvement of nutrition restores normal TSH levels without the need of thyroxine therapy.


Subclinical hypothyroidism Pancreatic enzyme therapy Cystic fibrosis Hyperthyrotropinemia Thyroid 



Cystic fibrosis


Subclinical hypothyroidism


Thyroid-stimulating hormone


Authors’ contributions

AG wrote the manuscript and processed the data; AK and MN retrieved the data; TK did lab work, and CK and SD designed the study.

Compliance with ethical standards

Informed written consent was obtained from the parents of the participating individuals and the study wasapproved from the local Ethical Committee.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Aris Giannakopoulos
    • 1
    Email author
  • Anni Katelaris
    • 2
  • Maria Noni
    • 2
  • Theodore Karakonstantakis
    • 3
  • Christina Kanaka-Gantenbein
    • 4
  • Stavros Doudounakis
    • 2
  1. 1.Department of PediatricsUniversity Hospital of PatrasPatrasGreece
  2. 2.Cystic Fibrosis Unit, Department of Pediatrics Cystic Fibrosis Unit, Department of Pediatrics“Aghia Sophia” Children’s HospitalAthensGreece
  3. 3.Department of Clinical Biochemistry“Aghia Sophia” Children’s HospitalAthensGreece
  4. 4.Division of Endocrinology, Metabolism and Diabetes, First Department of Pediatrics, Medical School, “Aghia Sophia” Children’s HospitalNational and Kapodistrian University of AthensAthensGreece

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