Pediatric Hypopituitarism Treatment & Management

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

Approach Considerations

Treatment for hypopituitarism primarily involves appropriate hormone replacement.[27] The presence of 1 or more hormone deficiencies determines medication choice. Conduct appropriate stress dosing of corticosteroid replacement. Surgical intervention can be employed in tumor-associated hypopituitarism, with the tumor location and type dictating the choice of surgical procedure. Diet and activity are unrestricted in patients with hypopituitarism.

Next

Consultations

Consultations are dependent on the etiology of hypopituitarism. Some of the consultants that may be involved in the care of patients with hypopituitarism come from the following specialties:

  • Ophthalmology - Optic nerve hypoplasia, septo-optic dysplasia, pituitary tumors
  • Neurology - Septo-optic dysplasia, holoprosencephaly, traumatic brain injury, pituitary tumors or other CNS tumors
  • Genetics - Congenital hypopituitarism, septo-optic dysplasia, holoprosencephaly
  • Oncology - CNS tumors (including pituitary tumors), other malignancies
  • Rehabilitation medicine
Previous
Next

Long-Term Monitoring

Routinely monitor growth and development at 3-month intervals in patients with hypopituitarism. If a patient is receiving recombinant human growth hormone (rhGH) therapy, monitor for adverse effects and monitor insulinlike growth factor (IGF)-I and insulinlike growth factor binding protein-3 (IGFBP3) levels at least annually. Also, consider monitoring for impaired glucose tolerance with a fasting morning blood sugar or hemoglobin A1c (HgbA1c), particularly in the patient with risk factors for diabetes mellitus (eg, family history, obesity).

Monitor thyroid functions routinely in hypopituitarism (FT4) or as part of scheduled monitoring in isolated GHD, when appropriate. Consider repeat low-dose ACTH stimulation testing in high-risk patients or if clinical symptoms of cortisol deficiency are apparent.

Home blood glucose monitoring to screen for hypoglycemia in very young patients and/or patients with central adrenal insufficiency should be strongly considered. In those patients with hypopituitarism that includes adrenal insufficiency, a medical alert bracelet should be worn, alerting first-responders of the patient’s need for stress hydrocortisone therapy.

Previous
Proceed to Medication
 
 
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.