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European Journal of Pediatrics

, Volume 149, Issue 2, pp 90–93 | Cite as

Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation

  • S. L. S. Drop
  • E. P. Krenning
  • R. Docter
  • S. M. P. E. de Muinck Keizer-Schrama
  • T. J. Visser
  • G. Hennemann
Endocrinology Original Papers
  • 46 Downloads

Abstract

We describe a girl who presented at the age of 6 weeks with cardiogenic shock due to congenital hypothyroidism (serum thyroxine (T4) <12 nmol/l). Thyroxine replacement therapy was instituted. In spite of high total serum T4 levels, thyroid stimulating hormone (TSH) serum values remained elevated. The raised serum T4 levels were the result of congenital elevation of thyroid binding albumin (TBA). Toxic doses of both T4 and triiodothyronine (T3) normalized the elevated TSH levels indicating that the pituitary is responsive to thyroid hormone, albeit at a higher threshold. In patients with congenital TBA elevation and an altered T4 pituitary response requiring thyroid replacement therapy, the measurement of serum free T4 levels is the parameter of choice to monitor treatment.

Key words

Thyroxine-binding-albumin Congenital hypothyroidism Hyperthyroxinaemia Thyroid hormones Resistance of the pituitary 

Abbreviations

CHT

congenital hypothyroidism

T3

triiodothyronine

T4

thyroxine

TBA

thyroxine binding albumin

TRH

thyrotropin releasing hormone

TSH

thyroid stimulating hormone

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Copyright information

© Springer-Verlag 1989

Authors and Affiliations

  • S. L. S. Drop
    • 1
  • E. P. Krenning
    • 2
    • 3
  • R. Docter
    • 2
  • S. M. P. E. de Muinck Keizer-Schrama
    • 1
  • T. J. Visser
    • 2
  • G. Hennemann
    • 2
  1. 1.Division of Paediatric Endocrinology, Department of PaediatricsSophia Children's HospitalRotterdamThe Netherlands
  2. 2.Department of Internal Medicine (III)University Hospital, Erasmus University RotterdamThe Netherlands
  3. 3.Department of Nuclear MedicineUniversity Hospital, Erasmus University RotterdamThe Netherlands

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