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