1. Arterial pH <7.3 2. Serum bicarbonate <15 meq/L 3. Ketones are elevated in serum or urine

Endo Flashcards by Hazel Baconga

Endocrine disorders manifest by

Excess hormone
Deficient hormone
Abnormal Response to end organ
Gland enlargement

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What are stimulated by the FF
GHRH
CRH
GNRH
TRH

In the hypothala

GH
ACTH
FSH,LH
TSH

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Dopamine inhibits

Prolactin

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Hypothalamic deficiency leads to decrease in most pituitary hormones expect

Prolactin

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Diagnosis of diabetes

Fasting glucose >126mg/dL
Random glucose >200
Abnormal ogtt >200 at 2 hours
Hba1c >6.5

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When ___% islet cells are destroyed the remaining ones are unable to sustain euglycemia

80-90%

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Renal threshold for glucose absorption

160-190mg/dl

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Polydypsia occurs because

Glucose causes osmotic diuresis

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DKA considered if

Arterial pH <7.3
Serum bicarbonate <15 meq/L
Ketones are elevated in serum or urine

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Very short acting insulin

Lispo
Aspart
glulisine

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Short acting insulin

Regular

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Intermediate acting

Neutral protamine hagedorn

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Long acting insulin

Glargine

Detemir

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Hba1c targets

<6 years old
7.5-8.5

6-13 years old
<8

13-18 years old
<7.5

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Severe hypoglycemia
Massive hepatomegaly
Growth retardation
Lactic acidosis

Glucose 6 phosphatase deficiency

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During hypoglycemic episode

Blood sample obtained for

Glucose
Insulin
GH
Cortisol
FFAs
Beta hydroxybutyrate
acetoacetate
Lactate

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Hypoglycemia without ketonuria means

Hyperinsulinism

Or defect in fatty acid oxidation

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Normal values upper to lower segment ratio

Infant 1.7:1
1 year old 1.4:1
10 year old 1:1

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Retinitis pigmentosa
Hypogonadism
Developmental delay
Autosomal dominant
Obesity
Polydactyly
Growth delay

Bardet Biedl syndrome

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Retinitis pigmentosa
Hypogonadism
Developmental delay
Autosomal dominant
Growth delay
Spastic paraplegia

Laurence Moon syndrome

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Short stature
Developmental delay
Short 4th and 5th digits
Resistance to parathyroid hormone
High Phos
Low Ca

Pseudohypoparathyroidism

Albright hereditary osteodystrophy phenotype

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Autosomal recessive
Prominent forehead
Hypoplastic nasal bridge
Delayed dentition
Sparse hair
Blue sclerae
Delayed bone maturation
Osteoporosis
Progressive adiposity
Hypercholesterolemia
Low blood glucose
Elevated GH
Low IGF-1

Laron syndrome

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FSH

In females stimulation of estrogen production and formation and support of corpus luteum

In males stimulation of devt of seminiferous tubules

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In males stimulation of testosterone from leydig cells

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Delayed puberty if

13 yrs in females 14 in males No sign of puberty

activity of GnRH hormone failure Anosmia or hyposmia Delayed puberty Hypogonadotropic hypogonadism

Kallman syndrome

Congenital absence of uterus

Mayer-Rokitansky-Kuster-Hauser syndrome

Precocious puberty

8 years in girls | 9 years in boys

autosomal dominant genetic disorder characterized by the development of benign hamartomatous polyps in the gastrointestinal tractand hyperpigmented macules on the lips and oral mucosa (melanosis) Precocious puberty

Peutz–Jeghers syndrome

suspected when two or more of the following features are present: Fibrous dysplasia Café au lait macules following lines of Blaschko Hyperfunctioning endocrine disease (Precious puberty, hyperthyroidism, testicular abnormalities, growth hormone excess, Cushing's syndrome)

McCune–Albright syndrome

Treatment for central precocious puberty

GnRH analog Leuprolide Histrelin

Tx for boys with GnRH independent precocious puberty

Inhibit testosterone synthesis (Ketoconazole) Antiandrogen (Spironolactone) Aromatase inhibitor (testolactone, letrozole)

Tx for McCune Albright precocious puberty

Testolactone | Tamoxifen

Thyroid metabolism of fetus occurs

Third trimester

___ exerts metabolic effect and negative feedback on TSH release

Ft3

Ft4 is test of choice because

It eliminates effects of variation in protein binding

Most common cause of acquired hypothyroidism in usa

Hashimoto thyroiditis | Lymphocytic autoimmune thyroiditis

Most common cause of acquired hypothyroidism in developing countries

Iodine deficiency | Endemic cretinism

Diagnosis for hashimoto thyroiditis

Serum antithyroid peroxidase

Side effect of methimazole

Granulocytopenia

___ can precipitate hypoCa by lowering ionized calcium without changing total serum Ca

Alkalosis

Most common cause of female ambiguous genitals

Congenital virilizing adrenal hyperplasia

Test to diagnose CAH with 21 hydroxylase deficiency

High levels 17 hydroxyprogesterone and androstenedione

Adrenal gland outer cortex for

Synthesis of steroids

Adrenal gland inner medulla

Synthesizes catecholamines

End product in adrenal cortex A. Outer glomerulosa B. Middle fasciculata C. Inner reticularis

A. Aldosterone B. Cortisol C. Sex steroids

Feature of congenital adrenal mineralcorticoid deficiency

HypoNa | HyperK

autoimmune destruction of the adrenal cortex hyperpigmentation, salt craving, postural hypotension, fasting hypoglycemia, anorexia, weakness, and episodes of shock during severe illness hyponatremia, hyperkalemia, and elevated plasma renin activity ACTH subnormal

Addison disease

Low dose dexamethasone suppression test Normal result? Abnormal result?

Normal - Decrease in cortisol | Cushing's disease - no change in cortisol

High dose dexamethasone suppression test | Cushing's disease result

Cortisol low in high dose

bone age? growth rate? adult height? Androgen excess

Advanced Increased Diminished

bone age? growth rate? adult height? Androgen deficiency

Normal or delayed Normal or decreased Increased slightly or normal

bone age? growth rate? adult height? Thyroxine excess

Advanced Increased Normal or diminished

bone age? growth rate? adult height? Thyroxine deficiency

Retarded Decreased Diminished

bone age? growth rate? adult height? Growth hormone excess

Normal or advanced Increased Excessive

bone age? growth rate? adult height? Growth hormone deficiency

Retarded Decreased Diminished

bone age? growth rate? adult height? Cortisol excess

Retarded Decreased Diminished

bone age? growth rate? adult height? Cortisol deficiency

Normal Normal Normal

provocative test for GH

insulin arginine clonidine glucagon

``` diabetes insipidus diabetes mellitus optic atrophy deafness vasopressin deficiency ```

wolfram syndrome

triphasic response after brain surgery

initial transient DI 12-48hours then SIADH up to 10 days then permanent DI

drugs that inhibit vasopressin

``` ethanol phenytoin opiate antagonists halothane alpha adrenergic ```

acquired nephrogenic DI can occur with

``` lithium demeclocycline foscarnet clozapine amphotericin methicilln rifampin ```

drugs that mimic vasopressin

``` vincristine vinblastine carbamazepine tricycle antidepressants oxcarbazepine ```

relationship between sodium and glucose

for every 100mg/dl increase in GLUCOSE (above 100mg/dl), | SODIUM decreases 1.6 meq/L

bone age sexual development HYPOPITUITARISM GH DEFICIENCY

Delayed | Delayed

bone age sexual development CONSTITUTIONAL DELAY

Delayed | Delayed

bone age sexual development FAMILIAL SHORT STATURE

normal | normal

bone age sexual development DEPRIVATIONAL DWARFISM

Usually delayed; growth arrest lines present | May be delayed

bone age sexual development TURNER SYNDROME

delayed | Female prepubertal

bone age sexual development HYPOTHYROIDISM

delayed | Usually delayed, may be precocious if hypothyroidism is severe

bone age sexual development CHRONIC DISEASE

delayed | Delayed

CALCIUM PHOSPHATE PARATHYROID HORMONE 25(OH)D Primary hypoparathyroidism

↓ ↑ ↓ N

CALCIUM PHOSPHATE PARATHYROID HORMONE 25(OH)D Pseudohypoparathyroidism

↓ ↑ ↑ N

CALCIUM PHOSPHATE PARATHYROID HORMONE 25(OH)D Vitamin D de ciency

Nl(↓) ↓ ↑ ↓

CALCIUM PHOSPHATE PARATHYROID HORMONE 25(OH)D Familial hypophosphatemic rickets

Nl ↓ Nl (sl↑) Nl

CALCIUM PHOSPHATE PARATHYROID HORMONE 25(OH)D Hyperparathyroidism

↑ ↓ ↑ Nl

CALCIUM PHOSPHATE PARATHYROID HORMONE 25(OH)D Immobilization

↑ ↑ ↓ Nl

TOTAL T4 FREE T4 SERUM TSH SERUM TBG Primary hypothyroidism

↓ ↓ ↑ N

TOTAL T4 FREE T4 SERUM TSH SERUM TBG Hypothalamic (TRH) tertiary hypothyroidism

↓ ↓ ↓ N

TOTAL T4 FREE T4 SERUM TSH SERUM TBG Pituitary (TSH) secondary hypothyroidism

↓ ↓ ↓ N

TOTAL T4 FREE T4 SERUM TSH SERUM TBG TBG de ciency

↓ N N ↓

TOTAL T4 FREE T4 SERUM TSH SERUM TBG TBG excess

↑ N N ↑

tx mccune albright syndrome

letrozole | tamoxifen

thyroxine synthesis occurs at __ wks aog

iodine trapping occurs at __ wks aog

8-10

most common cause of congenital hypothyroidisim

thyroid dysgenesis

autosomal recessive deafness goiter impaired iodide organification

pendred syndrome

muscular pseudohypertrophy in congenital hypothyroidism

kocher debre semelaigne syndrome

thyroxine tablets should not be mixed to __ because these bind t4 and inhibit absorption

soy calcium concentrated iron

most common cause of acquired hypothyroidisim

chronic lymphocytic thyroiditis

drugs that cause hypothyroidism

``` amiodarone methimazole propylthiouracil lithium interferon alpha thalidome valproate ```

pathogens in acute suppurative thyroiditis

anaerobe strep viridans staph pneumococcus

how are cassava and cruciferous veggies like cabbage, kale goitrogens?

compete with iodine for uptake in thyroid

reduction in thyroid hormone levels caused by ingestion of a large amount of iodine

Wolff–Chaikoff effect

retraction of eyelid in graves

dalrymple sign

``` neonate with: advanced bone age iugr infant is restless,irritable exophthalmic eyes weight loss severe hypertension cardiac decompression hepatomegaly frontal bossing craniosynostosis triangular facies ```

congenital hyperthyroidism

most common type of thyroid carcinoma in kids

papillary

most common site of mets for thyroid carcinoma

lungs

marker for tumor recurrence in thyroid carcinoma

serum thyroglobulin

thyroid nodules producing hyperthyroidism

plummer disease

hypocalcemia is common ___ hrs in newborns

12 to 72 hours

autoimmune hypoparathyroidism associated with

addison disease | chronic mucocutaneous candidiasis

``` medullary stenosis of long bones short stature delayed fontanel closure delayed bone age abnormal PTH episodic hypocalcemia ```

kenny-caffey syndrome

``` Oliguria Azotemia Stupor Coma HyperCa ```

Parathyroid crisis

Ectopic source of vit D in granulomatous disease

Activated macrophages

Prolonged immobilization can lead to

Hypercalcemia

Short limbed dwarfism Severe hyperCa Undetectable PTH levels

Jansen type metaphyseal chondrodysplasia

Rate limiting step in adrenal steroidogenesis

Importation of cholesterol across mitochondrial outer and inner membrane

Schedule of ACTH pulses

every 30-120 min, are highest at about the time of waking, are low in late afternoon and evening, and reach their lowest point 1 or 2 hr after sleep begins

Achalasia Alacrima Adrenal insufficiency

Allgrove syndrome

Adrenocortical deficiency | Associated with demyelination of cns

Adrenoleukodystrophy

Chronic mucocutaneous candidiasis as first manifestation Hypoparathyroidism Addison disease Autosomal recessive

Type 1 autoimmune polyendocrinopathy OR autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED)

Most common cause of corticotropin deficiency

Craniopharyngioma | Germinoma

Most common cause of Addison disease

Autoimmune destruction of glands

Prominent labs in adrenal insufficiency

HyperK HypoNa Hypoglycemia

Test when CAH suspected

17 hydroxyprogesterone | Along with cortisol in acth stimulation test

required for conversion of Δ5 steroids (pregnenolone, 17-hydroxypregnenolone, dehydroepiandrosterone [DHEA])

3β-hydroxysteroid dehydrogenase

Acth dependent Cushing syndrome Small adrenal gland Multiple small pigmented black nodules with large cells with lipofuscin

Primary pigmented nodular adrenocortical disease

autosomal dominant disorder also consisting of centrofacial lentigines and blue nevi; cardiac and cutaneous myxomas; pituitary, thyroid, and testicular tumors; and pigmented melanotic schwannomas With Primary pigmented nodular adrenocortical disease

Carney complex

Centrally acting serotonin antagonist that blocks acth release Treatment

Cyproheptadine

``` Hypertension HypoK Autosomal dominant Aldosterone and renin levels suppressed Mineralcorticoid excess ```

Liddle syndrome

``` Short stature Webbing of neck Pectus carinatum Cubitus valgus Right sided CHD Facies ```

Turner

nephropathy with ambiguous genitalia and bilateral Wilms tumor Progress to nephrotic syndrome and end stage renal failure

Denys drash syndrome

Most common form of male DSD

Androgen insensitivity syndrome

Wilms tumor Aniridia GU malformation Retardation

WAGR syndrome

Testes absent but male phenotype complete

Bilateral anorchia Or Vanishing testes syndrome

Dose of insulin with long standing DM and no insulin reserve

Prepubertal 0.7 u/kg/day Midpuberty 1.0 u/kg/day End of puberty 1.2 u/kg/day

Sodium should increase by _ for every _ of glucose

1.6mmol 100mg/dl

In DKA when should glucose be added

When serum glucose 250mg/dL

hormones that promote glycogenolysis

epinephrine | glucagon

hormones that promote gluconeogenesis

glucagon | cortisol

hormones that inhibit muscle uptake of glucose

epinephrine growth hormone cortisol

hormone that mobilizes amino acid for gluconeogenesis

cortisol

hormones for activating lipolysis

epinephrine cortisol growth hormone glucagon

hormone that inhibits insulin release and promotes growth factor and glucagon secretion

epinephrine