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Course: Pediatric Nursing

Topic: Nursing Care of Child with

Endocrine Disorders Part I

The Nurses International Community

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Module Goals

Learners will be able to:

Describe anatomy and physiology of the endocrine system in children
Identify common endocrine disorders in children including clinical manifestations
Describe related diagnostic procedures for these disorders
Explain evidence-based management for these disorders
Discuss nursing management of a child with an endocrine disorder

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Endocrine System in Children: Anatomy

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Endocrine System in Children: Physiology

Hypothalamus secretes:

Growth hormone-releasing hormone
Gonadotropin-releasing hormone
Thyrotrophin-releasing hormones
Corticotropin-releasing hormone
Dopamine
Somatostatin

Betts, et al., 2022a

Anterior pituitary gland secretes:

Growth hormone
Prolactin
Thyroid stimulating hormone
Adrenocorticotropic hormone
Follicle-stimulating hormone
Luteinizing hormone
Melanocyte stimulating hormone

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Endocrine System in Children: Physiology

Pineal body produces melatonin hormone that controls sleep cycle.
Thyroid glands produces thyroxine or tetraiodothyronine (T4) and triiodothyronine (T3) hormones regulate metabolism.
Parathyroid glands produces parathyroid hormones that regulate calcium and phosphorus balance.
Thymus produces hormone thymosin that helps in production and development of T-lymphocytes.
Pancreas produces hormones glucagon, insulin, somatostatin, and pancreatic polypeptide (PP), that regulate blood sugar level and appetite.

Betts, et al., 2022b; Betts, et al., 2022c

Betts, et al., 2022d; Betts, et al., 2022e

MacMillan Cancer Support, 2018

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Endocrine System in Children: Physiology

Adrenal glands produce:

Glucocorticoids: Controls use of fats, proteins, carbohydrates; suppresses inflammation; regulates blood pressure; increases blood sugar; may decrease bone formation
Aldosterone hormone: Regulates sodium, potassium level
Epinephrine and Norepinephrine: Causes ‘fight or flight’ response to stress
Androgens: Precursors for estrogen and testosterone hormones

Ovaries make estrogen and progesterone
Testes produces testosterone

Betts, et al., 2022f

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Endocrine System Disorders in Children

Growth hormone disorders
Hypothyroidism and Hyperthyroidism
Diabetes mellitus
Syndrome of inappropriate antidiuretic hormone (SIADH)
Diabetes insipidus
Congenital adrenal hyperplasia
Adrenal Insufficiency
Cushing’s syndrome
Precocious puberty and delayed puberty

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Growth Hormone (GH) Disorders in Children

GH is responsible for growth of bone structures and muscle mass
Growth Hormone Deficiency:

Inadequate amount of GH in the body
Causes abnormal slow growth, and short stature

Growth Hormone Insensitivity (GHI):

Genetic mutations that disrupt body’s ability to use GH.

Gigantism:

Excessive secretion of GH causing body tissues to increase to abnormal size.

Calabria, 2020

Brinkman, 2021

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Critical Thinking Question

Which of the following hormones are secreted by the anterior pituitary gland? (Select all that apply)

Growth hormone-releasing hormone
Gonadotropin-releasing hormone
Follicle stimulating hormone
Prolactin
Thyroid stimulating hormone
Adrenocorticotropic hormone

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GH Deficiency in Children

Causes

Congenital: Genetic or structural abnormalities of pituitary gland.
Acquired: Head trauma, infection, tumor, or radiation.
Can be idiopathic.

Signs and Symptoms

Short stature, slowed growth in height
Small penis at birth
Low blood glucose levels in infants and toddlers
Chubby body build
Prominent forehead
Underdeveloped nose bridge
Absence of puberty during teenage years

Brinkman, 2021; American Academy of Pediatrics and Pediatric Endocrine Society, n.d.

National Organization for Rare Disorders, 2016a; Rogol, et al., 2022

Boston Children’s Hospital, n.d.

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GH Deficiency in Children: Diagnostics

Medical history, including mother’s pregnancy, labor, delivery
Growth chart, monitoring height over a period of time
Low levels of Insulin-like growth factor 1 (IGF-1) in older children
Low levels of IGF binding protein type 3 (IGFBP-3) in infancy, early childhood
Lower levels of GH in two or more GH stimulation test

Gold standard test: Insulin tolerance test
Less reliable tests: Arginine, levodopa, clonidine, glucagon

(Calabria, 2020; Rogol, et al., 2022; Boston Children’s Hospital, n.d.; Kim, et al., 2020)

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GH Deficiency in Children: Diagnostics

Screening laboratory tests to rule out other possible causes of poor growth:

Hypothyroidism (eg, thyroid-stimulating hormone, thyroxine)
Renal disorders (eg, electrolytes, creatinine levels)
Inflammatory and immune conditions (eg, tissue transglutaminase antibodies, C-reactive protein)
Hematologic disorders (eg, complete blood count with differential)

Imaging

Delayed bone-age X-ray
Magnetic resonance image (MRI) for problems in pituitary or brain

Calabria, 2020; Rogol, et al., 2022

Boston Children’s Hospital, n.d.

Kim, et al., 2020

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GH Deficiency in Children: Management

Administration of Recombinant Human Growth Hormone (rHGH), also called somatotropin,

Initiated soon after diagnosis of GH deficiency
Injected once daily
Initial dose based on child’s weight, and condition being treated
Later doses based on dose effect, IGF-1 test results, pubertal stage

Length of treatment depends on response to GH injections, and usually until growth is complete
Parents are trained to administer injection at home

Calabria, 2020; Rogol, et al., 2022

Boston Children’s Hospital, n.d.

Kim, et al., 2020

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Special Nursing Considerations for

Children with GH Deficiency

Assess for signs/symptoms of GH deficiency for early detection
Monitor child’s growth with appropriate growth chart
Quick referral to specialist in presence of short stature or signs of GH deficiency
Close monitoring of child during insulin tolerance tests
Child/Parent education:

Signs/symptoms of GH deficiency
Safe administration of rHGH injection daily at home
Importance of routine follow-up

Assist child/family access to community resources for peer-support

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Critical Thinking Question

Which of the following interventions would a nurse anticipate to undertake while caring for a child with growth hormone deficiency? (Select all that apply)

Collecting throat swab for culture
Collecting blood sample for complete blood count
Assisting child to get chest X-ray
Assisting child to get MRI of brain
Teach parent how to inject growth hormone at home

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Growth Hormone Insensitivity (GHI) in Children

Genetic mutations make cells unresponsive to growth hormone
Acquired GHI

Antibodies from rHGH treatment block GH binding to its receptors

Can be hereditary, higher risks in consanguineous family
Signs/symptoms:

Intrauterine growth retardation
Congenital abnormalities, disproportionate skull/facial structures
Severe postnatal growth failure
Delayed puberty

Diagnosis:Presence of growth failure with typical facial appearance, central chubbiness, with normal to elevated serum GH level

Genetic and Rare Diseases Information Center, 2021

Goswami, Ghosh, & Chowdhury, 2012

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GHI in Children (Continued)

Laboratory tests may include: Serum GH, IGF-1, IGFBP-3, Growth hormone binding protein, STAT5b deficiency and primary acid-labile subunit (ALS) deficiency
Treatment: Injections of IGF-1 (like orphan drug mecasermin rinfabate)
Special nursing considerations:

Assess for signs of abnormal slow growth, refer appropriately
Assist in referral to genetic counselling
Safe administration of Injection IGF-1 with adequate diet to prevent hypoglycemia
Child/parent education about disease, it’s management, resources available

National Organization of Rare Disorders, 2016b

Boston Children’s Hospital, n.d.

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Gigantism in Children

Hypersecretion of GH, during childhood before the closure of long-bone epiphysis, that leads to excessive growth in height
Pituitary adenoma is the most common cause
Other causes:

Carney complex
McCune-Albright syndrome
Multiple endocrine neoplasia type 1 or type 4
Neurofibromatosis
Genetic disease that forms pituitary tumors

ADAM Health Soluntions, n.d.

Eugster, 2018

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Gigantism in Children (Continued)

Signs/Symptoms:

Excessive linear growth with macrocephaly and/or obesity
Frontal bossing
Broad nasal bridge
Prognathism
Excessive sweating
Voracious appetite
Coarse facial features
Enlargement of hands, feet

ADAM Health Soluntions, n.d.

Eugster, 2018

UCLA Health, n.d.

Complications due to untreated excess GH include hypertension, diabetes mellitus, heart disease/failure due to heart enlargement

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Image from Endotext [Internet] https://www.ncbi.nlm.nih.gov/books/NBK279155/

Twelve-year-old boy with pituitary gigantism measuring 6’5” with his mother. Note the coarse facial features and prominent jaw

Enlarged hand of the same boy in comparison with the hand of an adult male with a height of 6’1”

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Gigantism in Children: Diagnostics

Medical history and physical exam
Test for elevated blood IGF-1 level
Oral glucose tolerance test (OGTT)

Inability to suppress serum GH to an appropriate level following an oral glucose load

Magnetic resonance imaging (MRI) of hypothalamus and pituitary region for tumor.

ADAM Health Solutions, n.d.

Eugster, 2018

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Gigantism in Children: Management

Surgical removal of tumor
Pharmacological treatment:

Somatostatin analog (Octreotide, Lanreotide) injections to suppress GH production

Lifelong treatment required
Monthly basis

Daily injection Pegvisomant, a genetically engineered human GH analogue that normalize IGF-1 levels
Dopamine agonists, most effective as adjunctive agents

Most effective in cases of hyperprolactinemia

Radiation, as last resort due to risk of causing learning disabilities

Eugster, 2018

UCLA Health, n.d.

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Special Nursing Considerations for

Children with Gigantism

Assessment for signs/symptoms of gigantism for early identification and management
Provide child/parent education:

About disease condition, possible complications
Need for early management
Prescribed medication or treatment management

Signs of complications, and when to seek medical care

Assist in referral to appropriate specialist
Provide information on community resources for treatment management of the child’s condition, and peer support

Eugster, 2018

UCLA Health, n.d.

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Critical Thinking Question

What should the nurse do next after the assessment of the child shows signs/symptoms of gigantism?

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Cultural Considerations

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Health beliefs: In some cultures talking about a possible poor health outcome will cause that outcome to occur.
Health customs: In some cultures family members play a large role in health care decision-making.
Ethnic customs: Differing gender roles may determine who makes decisions about accepting & following treatment recommendations.

AHRQ, 2020

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Cultural Considerations

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Religious beliefs: Faith and spiritual beliefs may effect health seeking behavior and willingness to accept treatment.
Dietary customs: Dietary advice may be difficult to follow if it does not fit the foods or cooking methods of the family.
Interpersonal customs: Eye contact or physical touch may be ok in some cultures but inappropriate or offensive in others.

AHRQ, 2020

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References:

ADAM Health Solutions. (n.d.). Gigantism. MedlinePlus, National Library of Medicine. https://medlineplus.gov/ency/article/001174.htm

American Academy of Pediatrics and Pediatric Endocrine Society. (n.d.). Growth Hormone Deficiency. Pediatric Endocrine Society. https://pedsendo.org/patient-resource/growth-hormone-deficiency/

Betts, J.G, Young, K.A., Wise, J.A., Johnson, E., Poe, B., Kruse, D.H., Korol, O., Johnson, J.E., Womble, M., & DeSaix, P. (2022a, April 20). 17.3 The Pituitary Gland and Hypothalamus. OpenStax. https://openstax.org/books/anatomy-and-physiology-2e/pages/17-3-the-pituitary-gland-and-hypothalamus

Betts, J.G, Young, K.A., Wise, J.A., Johnson, E., Poe, B., Kruse, D.H., Korol, O., Johnson, J.E., Womble, M., & DeSaix, P. (2022b, April 20). 17.4 The Thyroid Gland. OpenStax. https://openstax.org/books/anatomy-and-physiology-2e/pages/17-4-the-thyroid-gland

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References:

Betts, J.G, Young, K.A., Wise, J.A., Johnson, E., Poe, B., Kruse, D.H., Korol, O., Johnson, J.E., Womble, M., & DeSaix, P. (2022c, April 20). 17.5 The Parathyroid Glands. OpenStax. https://openstax.org/books/anatomy-and-physiology-2e/pages/17-5-the-parathyroid-glands

Betts, J.G, Young, K.A., Wise, J.A., Johnson, E., Poe, B., Kruse, D.H., Korol, O., Johnson, J.E., Womble, M., & DeSaix, P. (2022d, April 20). 17.7 The Pineal Gland. OpenStax. https://openstax.org/books/anatomy-and-physiology-2e/pages/17-7-the-pineal-gland

Betts, J.G, Young, K.A., Wise, J.A., Johnson, E., Poe, B., Kruse, D.H., Korol, O., Johnson, J.E., Womble, M., & DeSaix, P. (2022e, April 20). 17.9 The Endocrine Pancreas. OpenStax. https://openstax.org/books/anatomy-and-physiology-2e/pages/17-9-the-endocrine-pancreas

Betts, J.G, Young, K.A., Wise, J.A., Johnson, E., Poe, B., Kruse, D.H., Korol, O., Johnson, J.E., Womble, M., & DeSaix, P. (2022f, April 20). 17.6 The Adrenal Glands. OpenStax. https://openstax.org/books/anatomy-and-physiology-2e/pages/17-6-the-adrenal-glands

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References:

Boston Children’s Hospital. (n.d.). Growth Hormone Deficiency. https://www.childrenshospital.org/conditions/growth-hormone-deficiency

Brinkman, J.E., Tariq, M.A., Leavitt, L., et al. (2021, May 7). Physiology, Growth Hormone. In: StatPearls [Internet]. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK482141/

Calabria, A. (2020, July). Growth Hormone Deficiency in Children. MSD Manual Professional Version. https://www.msdmanuals.com/professional/pediatrics/endocrine-disorders-in-children/growth-hormone-deficiency-in-children

Eugster, E. (2018, April 17). Gigantism. In: Feingold, K.R., Anawalt, B., Boyce, A., et al., (Eds). Endotext [Internet]. MDText.com, Inc. https://www.ncbi.nlm.nih.gov/books/NBK279155/

Genetic and Rare Diseases Information Center. (2021, November 8). Laron syndrome. National Center for Advancing Translational Sciences. https://rarediseases.info.nih.gov/diseases/6859/laron-syndrome

Contact info: info@nursesinternational.org

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References:

Goswami, S., Ghosh, S., & Chowdhury, S. (2012). Growth hormone insensitivity syndrome: A sensitive approach. Indian journal of endocrinology and metabolism, 16(5), 817–818. https://doi.org/10.4103/2230-8210.100636

Kim, J. H., Chae, H. W., Chin, S. O., Ku, C. R., Park, K. H., Lim, D. J., Kim, K. J., Lim, J. S., Kim, G., Choi, Y. M., Ahn, S. H., Jeon, M. J., Hwangbo, Y., Lee, J. H., Kim, B. K., Choi, Y. J., Lee, K. A., Moon, S. S., Ahn, H. Y., Choi, H. S., … Lee, E. J. (2020). Diagnosis and Treatment of Growth Hormone Deficiency: A Position Statement from Korean Endocrine Society and Korean Society of Pediatric Endocrinology. Endocrinology and metabolism (Seoul, Korea), 35(2), 272–287. https://doi.org/10.3803/EnM.2020.35.2.272

MacMillan Cancer Support. (2018, September 13). THE THYMUS GLAND. https://www.macmillan.org.uk/cancer-information-and-support/thymus-cancer/the-thymus-gland

National Organization for Rare Disorders. (2016a). Growth Hormone Deficiency. https://rarediseases.org/rare-diseases/growth-hormone-deficiency/

Contact info: info@nursesinternational.org

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References:

National Organization for Rare Disorders. (2016b). Growth Hormone Insensitivity. https://rarediseases.org/rare-diseases/growth-hormone-insensitivity/

Orphanet. (2022, May 9). Growth hormone insensitivity syndrome. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=181393

Rogol, A., Anhalt, H., Haines, M.S., & Vogiatzi, M.G. (2022, January 23). Growth Hormone Deficiency. Endocrine Society. https://www.endocrine.org/patient-engagement/endocrine-library/growth-hormone-deficiency

UCLA Health. (n.d.). Gigantism. http://pituitary.ucla.edu/resources

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