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Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation.
Eur J Pediatr 1989; 149(2):90-3EJ

Abstract

We describe a girl who presented at the age of 6 weeks with cardiogenic shock due to congenital hypothyroidism (serum thyroxine (T4) less than 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.

Authors+Show Affiliations

Department of Paediatrics, Sophia Children's Hospital, Rotterdam, The Netherlands.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

2512164

Citation

Drop, S L., et al. "Congenital Hypothyroidism and Partial Thyroid Hormone Unresponsiveness of the Pituitary in a Patient With Congenital Thyroxine Binding Albumin Elevation." European Journal of Pediatrics, vol. 149, no. 2, 1989, pp. 90-3.
Drop SL, Krenning EP, Docter R, et al. Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation. Eur J Pediatr. 1989;149(2):90-3.
Drop, S. L., Krenning, E. P., Docter, R., de Muinck Keizer-Schrama, S. M., Visser, T. J., & Hennemann, G. (1989). Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation. European Journal of Pediatrics, 149(2), pp. 90-3.
Drop SL, et al. Congenital Hypothyroidism and Partial Thyroid Hormone Unresponsiveness of the Pituitary in a Patient With Congenital Thyroxine Binding Albumin Elevation. Eur J Pediatr. 1989;149(2):90-3. PubMed PMID: 2512164.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation. AU - Drop,S L, AU - Krenning,E P, AU - Docter,R, AU - de Muinck Keizer-Schrama,S M, AU - Visser,T J, AU - Hennemann,G, PY - 1989/11/1/pubmed PY - 1989/11/1/medline PY - 1989/11/1/entrez SP - 90 EP - 3 JF - European journal of pediatrics JO - Eur. J. Pediatr. VL - 149 IS - 2 N2 - We describe a girl who presented at the age of 6 weeks with cardiogenic shock due to congenital hypothyroidism (serum thyroxine (T4) less than 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. SN - 0340-6199 UR - https://www.unboundmedicine.com/medline/citation/2512164/Congenital_hypothyroidism_and_partial_thyroid_hormone_unresponsiveness_of_the_pituitary_in_a_patient_with_congenital_thyroxine_binding_albumin_elevation_ L2 - http://www.diseaseinfosearch.org/result/1842 DB - PRIME DP - Unbound Medicine ER -