Pediatric Hypopituitarism Medication

  • Author: Joel W Steelman, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Jul 14, 2011
 

Medication Summary

Agents used to treat hypopituitarism simply replace the deficient hormone or hormones. When appropriately administered, dosing is determined in a physiologic manner, and adverse effects are rare. Careful titration is critical. Consistent and accurate compliance with appropriately prescribed regimens is mandatory to avoid hormone deficiency or excess.

Next

Endocrine hormones

Class Summary

These hormones are designed to replace absent hormones in patients with a pituitary deficiency.

Somatropin (Genotropin, Humatrope, Norditropin, Nutropin, Omnitrope, Saizen, Tev-Tropin)

 

Somatropin is an rhGH used to treat growth failure and metabolic abnormalities that accompany GHD. It is a purified polypeptide hormone of recombinant deoxyribonucleic acid (DNA) origin. The amino acid sequence of somatropin is identical to that of pituitary-derived human GH. The growth response of infants and children with severe GHD secondary to congenital hypopituitarism often is remarkable.

Levothyroxine (Synthroid, Levoxyl, Tirosint, Unithroid)

 

In active form, levothyroxine influences the growth and maturation of tissues. Sufficient thyroid hormone is mandatory for normal growth, metabolism, and neurologic development. For central hypothyroidism, the goal is normal FT4.

Hydrocortisone (Cortef, Solu-Cortef, A-Hydrocort)

 

Hydrocortisone is used for cortisol replacement therapy; it has mineralocorticoid activity and glucocorticoid effects.

Vasopressin (Pitressin)

 

Vasopressin is used for ADH replacement therapy mainly in the intensive care unit (ICU) or inpatient setting. It may be given as a continuous intravenous (IV) drip or as intermittent injections. The dose widely varies and is titrated depending on serum and/or urine sodium osmolality, fluid balance, and urine output.

Desmopressin (DDAVP, Stimate)

 

This agent increases the cellular permeability of collecting ducts, resulting in the reabsorption of water by the kidneys; it is used for ADH replacement.

Previous
Next

Androgen

Class Summary

These hormones are designed to replace testosterone absent secondary to gonadotropin deficiency.

Testosterone (Delatestryl, AndroGel, Testim)

 

Testosterone is an anabolic steroid that promotes and maintains secondary sex characteristics in androgen-deficient males. Dosing routes include intramuscular and transdermal. Oral forms of testosterone are very rarely used in the United States.

Previous
Next

Estrogen Derivative

Class Summary

These hormones are designed to replace estrogen absent secondary to gonadotropin deficiency.

Estradiol (Alora, Climara, Estrace, Estraderm)

 

Estradiol restores estrogen levels in girls with hypogonadotropism to concentrations that induce negative feedback at gonadotrophic regulatory centers, which in turn reduces the release of gonadotropins from the pituitary. Multiple studies have shown that estradiol will prevent bone loss at the spine and hip when started within 10 years of menopause.

Estradiol is used for hormone replacement and the induction of puberty. It acts by regulating the transcription of a limited number of genes. Estrogens diffuse through cell membranes, distribute themselves throughout the cell, and bind to and activate the nuclear estrogen receptor, a DNA-binding protein found in estrogen-responsive tissues. The activated estrogen receptor binds to specific DNA sequences or hormone-response elements, which enhances transcription of adjacent genes and, in turn, leads to the observed effects.

Previous
 
Contributor Information and Disclosures
Author

Joel W Steelman, MD  Endocrine Consultant, Division of Endocrinology and Diabetes, Cook Children's Medical Center

Joel W Steelman, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Society for Bone and Mineral Research, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Pfizer Honoraria Speaking and teaching

Specialty Editor Board

Phyllis W Speiser, MD  Chief, Division of Pediatric Endocrinology, Steven and Alexandra Cohen Children's Medical Center of New York; Professor of Pediatrics, Hofstra-North Shore LIJ School of Medicine at Hofstra University

Phyllis W Speiser, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London)  Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgments

I want to thank Simon Rhodes, PhD and Stephen Shalet, MD for kindly allowing me to republish illustrations from their work.

References
  1. Mehta A, Hindmarsh PC, Mehta H, Turton JP, Russell-Eggitt I, Taylor D, et al. Congenital hypopituitarism: clinical, molecular and neuroradiological correlates. Clin Endocrinol (Oxf). Mar 6 2009;[Medline].

  2. Parks JS, Kinoshita EI, Pfaffle RW. Pit-1 and hypopituitarism. Trends Endocrinol Metab. 1993;4:81-5.

  3. Rosenbloom AL, Almonte AS, Brown MR, et al. Clinical and biochemical phenotype of familial anterior hypopituitarism from mutation of the PROP1 gene. J Clin Endocrinol Metab. Jan 1999;84(1):50-7. [Medline]. [Full Text].

  4. Lebl J, Vosahlo J, Pfaeffle RW, et al. Auxological and endocrine phenotype in a population-based cohort of patients with PROP1 gene defects. Eur J Endocrinol. Sep 2005;153(3):389-96. [Medline].

  5. Bottner A, Keller E, Kratzsch J, et al. PROP1 mutations cause progressive deterioration of anterior pituitary function including adrenal insufficiency: a longitudinal analysis. J Clin Endocrinol Metab. Oct 2004;89(10):5256-65. [Medline]. [Full Text].

  6. Fluck C, Deladoey J, Rutishauser K, et al. Phenotypic variability in familial combined pituitary hormone deficiency caused by a PROP1 gene mutation resulting in the substitution of Arg-->Cys at codon 120 (R120C). J Clin Endocrinol Metab. Oct 1998;83(10):3727-34. [Medline]. [Full Text].

  7. Mullen, R.D., Colvin, S.C., Hunter, C.H., et al. Roles of the LHX3 and LHX4 LIM-homeodomain factors in pituitary development. Mol. Cell. Endocrinol. 2007;265:190-195.

  8. Kim SS, Kim Y, Shin YL, et al. Clinical characteristics and molecular analysis of PIT1, PROP1, LHX3, and HESX1 in combined pituitary hormone deficiency patients with abnormal pituitary MR imaging. Horm Res. 2003;60(6):277-83. [Medline].

  9. Pinto G, Netchine I, Sobrier ML, et al. Pituitary stalk interruption syndrome: a clinical-biological-genetic assessment of its pathogenesis. J Clin Endocrinol Metab. Oct 1997;82(10):3450-4. [Medline]. [Full Text].

  10. Darzy KH, Shalet SM. Hypopituitarism following Radiotherapy. In: Wallace WHB, Kelnar CJH. Endocrinopathy after Childhood Cancer Treatment. 15. Basel: Karger; 2009:1-24.

  11. Ghigo E, Masel B, Aimaretti G, et al. Consensus guidelines on screening for hypopituitarism following traumatic brain injury. Brain Inj. Aug 20 2005;19(9):711-24. [Medline].

  12. Acerini CL, Tasker RC. Endocrine Sequelae of Traumatic Brain Injury in Childhood. Horm Res. 2007;68(suppl 5):14-17.

  13. Sklar CA. Craniopharyngioma: endocrine abnormalities at presentation. Pediatr Neurosurg. 1994;21 Suppl 1:18-20. [Medline].

  14. Mootha SL, Barkovich AJ, Grumbach MM, et al. Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. J Clin Endocrinol Metab. May 1997;82(5):1362-7. [Medline]. [Full Text].

  15. Lindsay R, Feldkamp M, Harris D, et al. Utah Growth Study: growth standards and the prevalence of growth hormone deficiency. J Pediatr. Jul 1994;125(1):29-35. [Medline].

  16. Regal M, Parmo C, Sierra SM, Garcia-Mayor RV. Prevaence and Incidence of Hypopituitarism in an Adult Caucasian Population in Northwestern Spain. Clin Endocrinol. 2001;55:735-740.

  17. Stochholm K, Laursen T, Green A, Laurberg P, Andersen M, Kristensen LØ, et al. Morbidity and GH deficiency: a nationwide study. Eur J Endocrinol. Apr 2008;158(4):447-57. [Medline].

  18. White SM, Campbell DJ. Primary hypopituitarism and peri-operative steroid supplementation. Anaesthesia. Mar 2009;64(3):336-7. [Medline].

  19. Choo-Kang LR, Sun CC, Counts DR. Cholestasis and hypoglycemia: manifestations of congenital anterior hypopituitarism. J Clin Endocrinol Metab. Aug 1996;81(8):2786-9. [Medline].

  20. Sane K, Pescovitz OH. The clitoral index: a determination of clitoral size in normal girls and in girls with abnormal sexual development. J Pediatr. Feb 1992;120(2 Pt 1):264-6. [Medline].

  21. Garcia-Filion P, Epport K, Nelson M, Azen C, et al. Neuroradiographic, Endocrinologic, and Ophthalmic Correlates of Adverse Developmental Outcomes in Children With Optic Nerve Hypoplasia: A Prospective Study. Pediatrics. 2008;121:e653-e659.

  22. Borchert M, Garcia-Filion P. The Syndrome of Optic Nerve Hypoplasia. Current Neurology and Neuroscience Reports. 2008;8:395 - 403.

  23. Argyropoulou MI, Kiortsis DN. MRI of the hypothalamic-pituitary axis in children. Pediatr Radiol. Nov 2005;35(11):1045-55. [Medline].

  24. Rosenfeld RG. Disorders of growth hormone and insulin-like growth factor secretion and action. Pediatric Endocrinology. 1996;117-169.

  25. Weinzimer SA, Homan SA, Ferry RJ, Moshang T. Serum IGF-I and IGFBP-3 concentrations do not accurately predict growth hormone deficiency in children with brain tumours. Clin Endocrinol. 1999;51(3):339-45.

  26. Jostel A, Ryder WD, Shalet SM. The use of thyroid function tests in the diagnosis of hypopituitarism: definition and evaluation of the TSH Index. Clin Endocrinol (Oxf). Feb 18 2009;[Medline].

  27. Toogood AA, Stewart PM. Hypopituitarism: Clinical Featues, Diagnosis, and Management. In: Barkan AL. Endocrinology and Metabolism Clinics of North America. 37. 1. Philadelphia: Elsevier Saunders; 2008:235-261.

Previous
Next
 
The left photograph shows an untreated 21-month-old girl with congenital hypopituitarism. The right panel depicts the same child aged 29 months, following 8 months of growth hormone therapy.
Regulation of the development of the mammalian anterior pituitary gland by transcription factors. Following, inductive signals between the developing diencephalon and the oral ectoderm, early transcription factors guide the formation of rudimentary Rathke's Pouch (rRP) and then subsequent gene regulatory pathways control the determination, proliferation, and differentiation events that establish the specialized hormone-secreting cells. AP = anterior pituitary, IP = intermediate pituitary, PP = posterior pituitary. Modified by S. Rhodes from Mullen, R.D., Colvin, S.C., Hunter, C.H., Savage, J.J., Walvoord, E.C., Bhangoo, A.P.S., Ten, T., Weigel, J., Pfäffle, R.W., and Rhodes, S.J. (2007). Roles of the LHX3 and LHX4 LIM-homeodomain factors in pituitary development. Mol. Cell. Endocrinol., 265-266: 190-195.
Summary of Neuroendocrine Dysfunction following radiotherapy (courtesy of Stephen M Shalet, MD)
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.