ENDOCRINE chap 556-589 Flashcards by Karen Morales

Major regulator

Master gland

Pituitary gland

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Persistent remnants of the connection between Rathke pouch and oral cavity can develop into

Craniopharyngioma

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Under production of Growth hormone

Hypopituitarism

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Most common cause of Acquired hypopituitarism

Pituitary tumors

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Most common cause of Acquired hypopituitarism

Pituitary tumors

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T/F: Diabetes Insipidus is more frequent in acquired hypopituitarism

True

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Most common non pituitary tumor causing hypopituitarism

Craniopharyngioma

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Presents with neonatal emergeny (apnea, cyanosis, hypoglycemia
On pe: birth weight 1 SD below the mean, frontal bossing, depressed nasal bridge, eyes are somewhat bulging, blue sclerae, short neck, micropenis, high pitched voice

Congenital hypopituitarism

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Initially normal child
But came in due to weight loss, lack of energy, sensitivity to cold & absence of sweating. There was noted amenorrhea & loss of pubic and axillary hair.
Noted to be not growing (slowed growth)

Acquired hypopituitarism

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Principal regulator of osmolality (tonicity)

Vasopressin/ADH

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Hyperosmolality (>300 mOsm/kg)
Polydipsia
Polyuria
Serum osmolality (>300bmOsm/kg) > usine osmolality (<300 mOsm/kg)

Diabetes Insipidus

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Diabetes Insipidus (DI)
Diabetes Mellitus
Optic atrophy
Deafness

Wolfram syndrome

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Most common primary brain tumors associated with Central DI

Germinomas & Pinealomas

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Treatment for central DI

Fluid therapy (give fluids)
Desmopressin

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Treatment for Nephrogenic DI (vasopressin insensitivity)

Eliminate underlying disorder (hypercalcemia, hypokalemia, or ureteral obstruction)
Thiazide diuretics (increase sodium excretion at the expense of water causing mild volume depletion resulting to sodium and water reabsorption)

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Hyponatremia
inappropriately concentrates urine (>100 mOsm/kg)
Normal or slightly elevated plasma volume
Norma to high urine sodium
Low serum uric acid

Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH)

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Hyponatremia (Na<140 meq/L)
High urine sodium (>150meq/L)
High urine output
Hypovolemia
Normal or high uric acid

Cerebra Salt Wasting

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Lethargy
Psychosis
Coma 
Generalized seizure
Serum sodium <120meq/L

Acute hyponatremia

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Rapid correction of hyponatremia

Hypertonic 3% sodium chloride

0.5meq/L/hr or 12 meq/L/24 hr

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Most commonly diagnosed adenoma in childhood

Prolactinoma

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MCC of infants LGA

Maternal DM

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Macrosomia
Macroglossia
Hepatomegaly
Nehromegaly 
Omphalocoele
Hypoglycemia 
Predisposed to childhood neoplasm

Beckwith-Widemann Syndrome

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Most common cause of tall stature

Normal variant, familia or constitutional tall stature

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Male
Tall stature
Gynecomastia
Learning disabilities
Decreased upper body:lower body segment ratio
Hypertelorism (wide spaced eyes), hypotonia, clinodactyly (bending of finger towards adjacent finger)
Small penis & testes
Infertile

Kleinfelter syndrome (XXY)

MCC pituitary tumor in adolescents

Prolactinoma

Female | Headace, primary or secondary amenorrhea, galactorrhea but with normal puberty

Prolactinoma

First sign of puberty in females

Thelarche (breast bud) - 10 to 11 years old

Chronoligical features of puberty in females

1. Thelarche (breast bud) 2. Pubarche (pubic hair) 3. Menarche

First sign of puberty in males

Testicular enlargement

Onset of secondary sexual characteristics before 8yrs old in girls & 9 years old in boys

Precocious puberty

Thelarche at 7 years old Irregular menses Height and weight advanced for age Osseous maturation (bone age) is advanced for age

Precocious puberty

Treatment for central precocious puberty

GnRH agonist (Leuprolide 0.25-0.35 mg/kg)

Most common brain lesion causing central precocious puberty

Hypothalamic hamartomas

``` Precocious puberty Gelastic seizure (uncontrolled laughing or crying) ```

Hypothalamic Hamartomas

Precocious puberty Patchy cutaneous pigmentation Fibrous dysplasia

McCune-Albright Syndrome

Most common extraglandular manifestation of precocious puberty

Phosphaturia

2 most common forms of partial (incomplete) precocious development

1. Breast development in girls | 2. Sexual hain growth in both

MCC of permanent congenital hypothyroidism

Thyroid Dysgenesis

MCC of syndromic deafness Autosomal recessive Mutation in the chloride-iodide transport protein

Pendred syndrome

Patient came in with sensorineural hearing loss and goiter. Upon thyroiodide test, there was noted positive percholate discharge. (Diagnosis?)

Pendrin syndrome

MCC of congenital hypothyroidism world wide

Goiter or Iodine deficiency

6 mos female came due to poor feeding Noted to have little crying and sleeping a lot, appears sluggish, she is also CONSTIPATED with UMBILICAL HERNIA NO head control Pe: large ant & POST fontanel, large TONGUE, SMALL for age, eyes are WIDE APART, DEPRESSED nose bridge, NO TEETH (delayed dentition), SHORT & THICK neck, DRY SCALY skin, COARSE SCANTY BRITTLE hair Labs: Low T4, high TSH, macrocytic anemia Radio: ABSENCE of DISTAL femoral & PROXIMAL tibial EPIPHYSES, WORMIAN bones in the skull

Congenital hypothyroidism

Treatment of choice for congenital hypothyroidism

Levothyroxine (10-15 umcg/kg/day)

MCC of acquired hypothyroidism

Hashimoto thyroiditis (Chronic lymphocytic thyroiditis)

Girls More common after 6 yrs old With goiter (asymmetric, enlarged, firm, nontender) and growth retardation May have pressure symptoms (difficulty swallowing, shortness of breath) Clinically euthyroid but may jave symptoms of hypothyroidism

Hashimoto Thyroiditis

Lynphoid follicle formation with germinal centers

Hashimoto Thyroiditis

MCC of thyroid disease in children and adolescents

Hashimoto Thyroiditis

Normal FT4, slightly elevated TSH

Subclinical hypothyroidism

Antiarrythmic drug associated with congenital goiter with hypothyroidism

Amiodarone

Most serious consequence of iodine deficiency

Endemic cretinism

Excessive secretion of thyroid hormone

Hyperthyroidism

``` Female predominance Peak at 11 to 12 years old Emotional lability Tremors Voracious appettite without weigjt gain Excessive sweating, palpitations ``` Pe: exopthalmos, upper eyelid lag, tachycardia, apical systolic murmur (Mitral regurgitation), hyperreflexia Lab: low TSH, elevated T3, T4, FT3 and FT4

Graves disease

Preferred treatment for hyperthyroidism in pregnancy

Propylthiouracil (PTU)

Preferred treatment for hyperthyroidism in children

Methimazole

Unwanted adverse effect of PTU

Severe liver disease

MCC liver disease associated with Methimazole

Cholestatic jaundice

Treatment for Neonatal Graves disease or Congenital Hyperthyroidism

``` Propanolol (1-2mg/kg/24h orally in 3 divided doses) Methimazole (0.25-1mkday q12) Potassium iodide (1 drop per day) ```

MC thyroid carcinoma in childhood

Papillary carcinoma

Adolescent female Presented with painless nodule, fast growing, large in size, firm, non-movable, with noted hoarseness, dysphagia and presence of neck lymphadenopathy

Thyroid carcinoma