Growth Hormone (GH)

What is the GOLD STANDARD to diagnose GH deficiency?

*GH stimulation test* ~Insulin induced hypoglycemia ~Arginine Quiz: *GH measurement following insulin admin.*

Radiographic imaging for pituitary gigantism include:

*MRI* hypothalamus and pituitary gland

What are the 3 phases of post-natal growth?

1) Infantile phase 2)Childhood phase 3) Pubertal phase

Serum IGF-1 levels may be low in conditions other than GH deficiency: what are they?

A) Hypothyroidism B) Diabetes C) Renal Failure

GH beings in ____a____ life and GH secretion is ____b____

A) early b) pulsatile

A) GH releasing hormone B) GH Inhibibiting hormone

A)Stimulates GH secretion B) Inhibits GH secretion (Somatostatin)

Excess Growth Hormone AFTER puberty

Acromegaly

______ phase is a relatively constant growth (5-7cm per year) ~Slightly slower in late childhood "prepubertal phase"

Childhood Phase

Most common cause of Acromegaly is:

GH secreting adenoma in the anterior pituitary.

what is the GOLD STANDARD test for Acromegaly?

GH suppression test *OGTT*

Which test is considered GOLD standard in diagnosing pituitary gigantism? (If IGF-1 suggests GH EXCESS)

GH suppression test- *OGTT*

What is the most abundant pituitary hormone?

Growth Hormone (GH) (Somatotrophs)

______ is produced in the liver after stimulation by Growth Hormone (GH)

IGF-1

What is the: ~MC cause of congenital hypogonadism ~Eunochoidal proportions (long arms & legs) ~Learning disability mainly expressive language ~Small testes & gynecomastia

Klinefelters syndrome (XXY syndrome- male is born with extra copy of X chromosome)

GH stimulates ______ in kiddos

Linear Growth (epiphyseal plates of long bones)

Poor nutrition can ______ IGF-1 despite normal levels of GH (lower or increase)

Lower

radiograph exam on acromegaly?

MRI on the pituitary

_________ is an autosomal dominant disorder-connective tissue disease. ~tall stature ~Long, thin fingers &hyper extensibility of the joints.

Marfans Syndrome

Do patients who are diagnosed with acromegaly get taller?

NO

If a child is growing with in parental target range (Tall/Short) this growth velocity is typically ______

NORMAL ~genetic (Tall/Short) stature

Diagnosis of ACROMEGALY: what is the best single test for acromegaly?

SERUM IGF-1 concentration

What is the treatment of GH deficiency in Kiddos?

SubQ Somatotropin (multiple dose pen device) GH therapy: ~Continue until GH completion ~May develop normal GH secretion

Whether the patient has growth hormone excess or deficiency. when a growth hormone abnormality is noted. which of the following must be ruled out in both cases?

Thyroid hormone abnormalities

Why would we do an x-ray on hand and wrist for short stature?

To compare bone development to height and chronological age.

What is the *treatment of choice* of pituitary gigantism? ~Curative for pituitary microadenomas and macroadenomas.

Transphenoidal Surgery

True or False: Acromegaly is a Insidious onset/slow progression

True

What are the pathological causes of *SHORT STATURE*? (7)

1) Gh deficiency 2)Intrauterine growth retardation 3) Infection (Rubella, CMV) 4) Maternal Drug use/Alcohol usage 5) Genetic Syndromes (Turners Syndrome) 6) Chronic Systemic Disease (Cancer, Cystic Fibrosis, GI, Renal insufficiency) 7) Psychosocial Dwarfism (Nutritional and emotional deprivation)

Causes of TALL stature include:

Constitutional Tall stature ~Taller then peers ~Have accelerate growth velocity ~Moderately advanced bone age

Absence of IGF-1 produces _____

Dwarfism (Laron Type Dwarfism)

We measure IGF-1 levels if H&P suggests _____

GH deficiency (measure plasma IGF-1 levels)

________ phase: is a rapid growth ~First 2 years of life (~30-35cm)

Infantile Phase

14 year old male patient with pituitary gigantism due to a pituitary adenoma is not a surgical candidate. What is a good treatment option for this patient?

Octreotide

A 32 year old male presents with complaints of headaches, increasing shoe size, he can no longer wear his wedding ring and he thinks his face looks like it is changing. On testing you find bitemporal hemianopsia. What is the most likely diagnosis?

Pituitary Growth Hormone (Macroadenoma)

_____ phase: growth spurt (8-14cm per year) Effects of increased gonadal steroids and GH secretion.

Pubertal phase

Long term management of acromegaly include:

~Clinical evaluation & assessment every 3-4 months. (IGF-1 & OGTT) ~Anterior Pituitary Function (monitor for S&S of pituitary hormone deficiency annually) ~Adenoma Size (MRI-repeat yearly for several years after surgery) (visual assess.) ~Systemic Eval (Colonoscopy & Annual CV eval)

What is the treatment for pituitary gigantism? Radiation

~Cranial radiation- adjunctive ~can cause problems with children.

Acromegaly growth (acral growth) include:

~Enlarged jaw, hands and feet ~Enlargement of nose and frontal bones ~Teeth become spread apart

Acromegaly: adenoma may cause local symptoms such as:

~Headaches ~Visual field defects (bitemporal hemianopsia) ~Cranial nerve palsies

Lab abnormalities in Acromegaly include:

~Hyperglycemia ~Hyperprolactinemia Mortality rate 2-3x expected rate (mostly from CV disease and cancer)

What are the ENDOCRINE causes of TALL stature? (3)

~Hyperthyroidism ~Precocious puberty ~pituitary gigantism (Rare) -- Most kiddos with tall stature DO NOT have pituitary gigantism

What is the treatment for pituitary gigantism? PHARMACOLOGIC

~OCTREOTIDE (somatostatin analog) (MC agent) ~Bromocriptine- was mc used till recently ~GH receptor antagonist ~combotherapy (bromo + octreotide in kiddos)

Summary: IF suspicious of pathologic SHORT stature:

~Obtain bone age with wrist radiograph ~IGF-1 ~Confirm with GH stimulation test ~Refer to pediatric Endocrinologist

What are the three treatment options for pituitary gigantism?

~Transphenoidal surgery (Tx of choice) ~Radiation ~Pharmacologic