Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Pathology > Endocrinology > Flashcards

Endocrinology Flashcards

(238 cards)

Start Studying
1
Q

What hormones are produced by Somatotrophs?

A

GH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What hormones are produced by Lactotrophs?

A

Prolactin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What hormones are produced by Corticotrophs?

A

ACTH

POMC

MSH

Endorphins

Lipotropin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What hormones are produced by Thyrotrophs?

A

TSH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What hormones are produced by Gonadotrophs?

A

LH

FSH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What molecule inhibits prolactin secretion?

A

Dopamine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What molecule inhibits GH secretion?

A

Somatostatin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is pituitary apoplexy?

A

Rapid enlargement of pituitary due to acute hemorrhage

***surgical emergency***

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the most common cause of hyperpituitarism?

A

Anterior lobe adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the causes of hyperpituitarism?

A
  • adenoma
  • Pituitary CA
  • Hyperplasia
  • Hypothalamic disorders
  • Ectopic tumors
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How can non-functioning adenomas cause hypopituitarism?

A

Pressure effects on normal pituitary tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the size difference between a microadenoma and a macroadenoma?

A
  • Microadenoma: 1 cm or less
  • Macroadenoma: more than 1 cm
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the major mutation seen in GH somatotroph adenomas?

A

GNAS

Lack of GTPase

(also in ACTH corticotroph adenomas)

G = GH adenomas, GNAS, GTPase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the most common cause of pituitary adenoma?

A

Sporatic

5% genetic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What gene mutations are associated with Pituitary adenomas?

A
  • MEN1
  • CDKN1B
  • PRKAR1A
  • AIP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What gene mutation is associated with GH adenomas in patients under 35 y/o?

A

AIP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What mutations are found in Pituitary Carcinomas?

A

HRAS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the characteristics of Invasive Adenomas?

A
  • Not encapsulated
  • Infiltrate adjacent bone, dura, sometimes brain
  • Do not metastasize
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What disease process is pictured below?

A

Pituitary adenoma

monomorphism, lack of reticulin

This side: normal pituitary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What disease process is pictured below?

A

Pituitary adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What distinguishes adenomas from non-neoplastic anterior pituitary histologically?

A

Cellular monomorphism (uniformity)

Lack of reticulin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the most common hyperfunctioning pituitary adenoma?

A

Lactotroph adenoma

(Prolactinoma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the difference between Chromophobic and Acidophil Prolactinomas?

A
  • Chromophobic
    • sparsely granulated
    • juxtanuclear transciption factor PIT-1
  • Acidophil
    • dnesely granular
    • cytoplasmic transciption factor PIT-1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are the symptoms of Prolactinoma?

A
  • amenorrhea
  • galactorrhea
  • loss of libido
  • infertility
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What is the most likely presentation of Prolacinomas in older men and women?
Macroadenoma with mass effect
26
Prolactinomas cause what percentage of cases of amenorrhea?
25%
27
What are the treatments for Prolactinoma?
Surgery Dopamine receptor agonists: Bromocriptine
28
Calcifications around the the intracranial cavity are indicative of what?
Prolactinoma
29
What is the second most common functioning adenoma?
Growth Hormone (Somatotroph) Adenoma
30
GH adenoma causes secretion of what hormone?
IGF-1 from liver
31
What diseases are caused by GH Adenomas?
* Gigantism * if before closure of epiphyseal plate * Acromegaly * if in adults
32
What complications can arise from GH adenomas?
* Diabetes mellitus (GH increases blood sugar levels) * HTN * Arthritis * CHF * GI cancer * Increased GH =\> increased polyp formation
33
How is GH Adenoma diagnosed?
1. Failure to suppress GH in response to an oral load of glucose 2. Elevated basal levels of GH and IGF-1
34
What is the treatment for GH Adenomas?
Surgury Somatostatin analogs GH receptor antagonists
35
What is the difference between Cushing Disease and Cushing Syndrome?
* Disease * An ACTH secreting adenoma * Syndrome * Any hypercortisol state
36
Do ACTH secreting adenomas stain with PAS? Why or why not?
Yes, due to carbs in POMC, a precursor to ACTH
37
What is Nelson Syndrome?
Large ACTH adenoma secondary to removal of adrenal glands. Presents with mass effect and hyperpigmentation.
38
What is the common presentation in Gonadotroph Adenomas?
Non-functional Symptoms due to mass effect: impaired vision, headaches, diplopia, pituitary apoplexy May result in hormone deficiencies from mass effect
39
Which hormone is most affected by mass effect in Gonadotroph Adenomas? What are the side effects?
LH secretion is impaired, while FSH is the predominately secreted hormone Symptoms: Decreased libido, amenorrhea
40
What transcription factors are secreted by Gonadotroph Adenomas?
SF-1 GATA-2
41
What percent of pituitary tumors are non-functioning adenomas?
25-30%
42
What hormones are most commonly secreted in Pituitary CA?
Prolactin ACTH
43
What are the most common causes of pituitary hypofunction?
1. Traumatic brain injury 2. Subarachnoid hemorrhage
44
Patient presents with a sudden, excruciating headache with diplopia and hypopituitarism. What is the likely diagnosis?
Pituitary apoplexy | (Sudden hemorrhage into the pituitary)
45
What is the most common cause of pituitary necrosis?
Sheehan Syndrome
46
What is Sheehan Syndrome? How does this occur?
Postpartum necrosis of the pituitary Pituitary doubles its size during pregnancy, but does not have increased vasculature. Therefore, if there is hemorrhage during or following birth, the pituitary can become ischemic and necrotic.
47
A fluid-filled cyst is found near the pituitary. It is lined by ciliated cuboidal epithelium, occasional goblet cells, and anterior pituitary cells. What is the diagnosis?
Rathke cleft cyst
48
What is the common cause of empty sella syndrome?
Defect in diaphram sella allows arachnoid mater and CSF to herniate into the sella, damaging the pituitary tissue
49
What population is most effected by empty sella syndrome?
Obese women with multiple pregnancies
50
What are the symptoms of empty sella syndrome?
Visual field defects Endocrine abnormalities: Hyperprolactinemia, sometimes hypopituitarism
51
What are the causes of secondary empty sella syndrome?
Surgical removal Infarction of pituitary adenoma
52
What genetic defect is most commonly associated with hypopituitarism?
PIT-1 Causes decreased: GH, Prolactin, TSH
53
What inflammatory disorders/infxns can cause Ant. pituitary hormone deficiencies?
Sarcoidosis TB meningitis
54
What results from Congenital TSH deficiency?
Hypothyroidism Cretinism with mental retardation and retarded growth
55
What results from LH and FSH deficiency?
Hypogonadism * Females * Amenorrhea * Infertility * Atrophy of ovaries * Males * Testicular atrophy * Sterility * Impotence * Loss of axillary and pubic hair
56
What is the Hand-Schuller-Christian triad?
Diabetes insipidus Calvarial bone defects Exophthalmos
57
What causes Diabetes Insipidus? How is diagnosis made?
* Cause * ADH deficiency * Unable to reabsorb water * serum osm \> urine osm * Diagnosis * Water deprivation test
58
What causes SIADH? What are the symptoms?
* Cause * excess ADH causes excessive resorption of water * Most common cause: ADH secreting oat cell CA of lung * Symptoms * hyponatremia * cerebral edema * neurologic dysfuntion * Urine osm \> serum osm
59
What disease process is pictured below?
Craniopharyngioma
60
What suprasellar tumors are most common?
1. Gliomas 2. Craniopharyngiomas
61
What is the origin of Craniopharyngiomas?
Reminants of Rathke pouch
62
What populations are most affected by craniopharyngiomas? What type of tumor is found in each? What are the symptoms?
* Children 5-15 y/o * type: * Adamantinomatous: SS epithelium, lamellar keratin, cholesterol-rich fluid, calcifications * Symptoms: * endocrine deficiencies (growth retardation) * Adults 65 years or older * type: * Papillary: solid sheets and papilla; lacks keratin, calcifications, cysts * Symptoms: * Visual disturbances
63
What is the prognosis for craniopharyngiomas?
Excellent prognosis Malignant transformation very rare
64
What are goitrogens?
Inhibitors of thyroid gland * Suppress production of T2 and T4 * Increase TSH
65
What effect does Propylthiouracil have on the thyroid?
Inhibits oxidation of iodine to T3 or T4
66
What function does large doses of iodine have on the thyroid?
Inhibits proteolysis of thyroglobulin to form thyroid hormones
67
What is the difference between Thyrotoxicosis and Hyperthyroidism?
* Thyrotoxicosis * Elevated T3 and T4 * Hypermetabolic state * Hyperthyroidism * Increased TSH * Hyperfunctioning
68
What are the most common causes of Thyrotoxicosis?
1. Diffuse hyperplasia (Graves) 2. Hyperfunctional multinodular goiter 3. Hyperfunctional adenoma
69
What happens to the neuromuscular system with excess thyroid hormone?
Sympathetic overactivity
70
What happens to the skeletal system with excess thyroid hormone?
Osteoporosis
71
What is thyroid storm? What are the symptoms?
* Def * Severe hyperthyroidism * usually in background of Graves * Cause * set off by infxn, surgery, stress * Symptoms * Fever * Pronounced tachycardia
72
What is Apathetic Hyperthyroidism? How does it present?
* Def * thyperthyroidism in elderly * masked by aging and co-morbidities * Presentation * Weight loss * Worsening cardiac disease
73
What are the lab values for primary and secondary Apathetic Hyperthyroidism?
* Primary * Decreased TSH * Elevated Free T4 or Free T3 * Secondary * Increased TSH (or normal) * Elevated FT4 and FT3
74
What is the difference in results of radioactive iodine scan in Graves and Thyrotoxicosis?
* Graves * Uptake of radioactive iodine * Thyrotoxicosis * No uptake
75
What is the most common cause of Hypothyroidism?
Autoimmune, usually Hashimoto's
76
What is Dyshormonogenetic hypothyroidism?
Genetic inability to produce thyroid hormone
77
What is Cretinism? How does it present?
* Cretinism * hypothyroidism * develops in infancy / early childhood * Presentation * Impaired CNS and Skeletal development * "floppy baby" * Protruding tongue * Umbilical hernia
78
What is Myxedema? How does it present? What do labs show for primary and secondary disease?
* Myxedema * Hypothyroidism developing in an older child or adult * Presentation * Slowed mental and physical activity * Increased LDL or creatine kinase * Primary * Increased TSH * Decreased FT4 * Secondary * Decreased TSH * Decreased FT4
79
What does increased cholesterol / LDL in a person who has "just been feeling crappy" indicate?
TSH testing should be done | (Possible myxedema)
80
Patient presents with fever, chills, and neck pain. They are found to have thyroiditis. What type of thyroiditis do they have? What is the effect on thyroid function?
Infectious thyroiditis No effect on thyroid function
81
What is the most common cause of hypothyroidism in iodine sufficienct areas?
Hashimoto's
82
What Abs are found in Hashimoto Thyroiditis?
Thyroid peroxidase (thyroblobulin)
83
What is the histologic appearance of Hashimoto thyroiditis?
* mononuclear infiltrate with germinal centers * Hurthle cells * atrophic, enlarged eosinophilic cells of follicles * Fibrosis
84
What are the labs present in Hashimoto's thyroiditis?
* Increased TSH * Decreased FT4 * TPO abs
85
What is the presentation of Hashimoto's?
Painless enlargement Normally in middle-aged women
86
Hashimoto's Thyroiditis has an increased predisposition for what malignancies?
* B-cell lymphomas, esp. marginal zone type * already has germinal centers present * Papillary carcinoma
87
What is the cause of Subacute DeQuervain Granulomatous Thyroiditis?
Viral infection (esp UTI) This activates cytotoxic T-cells, damaging follicles This causes a granulomatous infiltrate
88
A patient presents with a painful enlargement in the neck. The pain radiates and causes pain when swallowing. Labs show low TSH and increased FT3 and FT4. The hyperthyroidism lasts a couple weeks, followed by full recovery. What is the diagnosis?
Subacute Granulomatous DeQuervain Thyroiditis
89
What is the morphology of Subacute DeQuervain Granulomatous Thyroiditis?
Aggregates of inflammatory cells (granulomatous inflammation) Multinucleated giant cells surrounding pools of colloid
90
What is the prognosis for Subacute DeQuervain Granulomatous Thyroiditis?
Complete recovery
91
A patient presents with a stony hard, painless thyroid with an asymmetric nodularity. It was found to be fibrotic with extention into the surrounding neck structures. What is the diagnosis?
Riedel's Thyroiditis
92
What is the etiology of Riedel's Thyroiditis?
Autoimmune Plasma cell IgG causes fibrosis
93
What is the most common causeof endogenous hyperthyroidism?
Graves Disease
94
What antibodies are found in Graves disease?
1. TSH receptor Ab 2. Thyroid peroxidase Ab 3. Thyroglobulin Ab
95
What HLA polymorphism is associated with Graves?
HLA-D3
96
What is the histologic appearance of Graves Disease?
Increased height and number of follicular cells Decreased colloid with sawtooth edges
97
What are the major clinical findings of Graves?
1. Thyrotoxicosis 2. Diffuse thyroid hyperplasia 3. Ophthalmopathy (exophthalmos) * increased glycosaminoglycan and adiposite deposition behind eye 4. Dermopathy (myxedema) * scaly thickening and induration * most common in shins
98
What are the lab findings for Graves?
* Decreased TSH * Elevated FT3 and FT4 (stimulated by binding of Ab to TSH receptor)
99
What can increased intake of thiocynates cause?
Endemic goiter (Diffuse Non-toxic/Simple Goiter) Thiocynates block iodine transport Found in cabbage, cassava, cauliflower
100
What is the appearance of diffuse non-toxic goiter?
Diffusely enlarged w/o nodularity
101
What are the characteristics of the hyperplastic phase and the colloid involutional phase of Diffuse Non-toxic Goiter?
* Hyperplastic​ * Occurs first * crowded follicular cells * Reduced colloid in follicles * Colloid Involutional Phase * accumulation of colloid w/i enlarged follicles * Follicular flattening and atrophy
102
What is a plunging goiter?
Extends behind sternum, trachea, esophagus aka intrathoracic goiter
103
What is Plummer's disease?
Development of autonomous nodules in longstanding multinodular goiter Results in hyperthyroidism
104
Which is more likely to be more neoplastic: solitary or multiple nodules?
Solitary
105
Which is more likely to be more neoplastic: nodules in a younger patient or an older patient?
Younger patient
106
Which is more likely to be more neoplastic: Nodules in a male or female patient?
Male
107
Which is more likely to be more neoplastic: Hot nodules or cold nodules?
Cold
108
What mutation is found most often in Follicluar Thyroid adenoma?
GNAS RAS, PIK3CA, and PAX8-PPARG fusion gene can also be found in some, but are also found in thyroid malignancies
109
What differentiates Follicular adenoma from Follicular CA?
* Follicular adenoma * well demarcated (w/capsule) * Follicular CA * invades through capsule
110
What are the clinical features of Follicular Adenoma?
* Painless * Unilateral * Cold nodule
111
RET mutation is found in which types of Thyroid CA?
1. Papillary CA 2. Medullary Thyroid CA
112
NTRK1 gain of function mutation is found in which types of Thyroid CA?
1. Papillary CA
113
BRAF mutation is found in which types of Thyroid CA?
1. Papillary CA * assoc. w/ metastatic disease and extrathyroid extension
114
RAS and PIK3CA mutation is found in which types of Thyroid CA?
1. Follicular CA 2. Anaplastic CA
115
PTEN mutation is found in which types of Thyroid CA?
1. Follicular
116
PAX8-PPARG fusion gene is found in which types of Thyroid CA?
1. Follicular CA 2. (Papillary adenomas)
117
TP53 and beta-catenin mutation is found in which types of Thyroid CA?
1. Anaplastic CA * probably relates to aggressive behavior
118
What is the most common form of thyroid cancer?
Papillary CA
119
What is the most common thyroid CA associated with radiation exposure?
Papillary CA
120
What are the microscopic features of Papillary CA?
* Papillae * "Orphan Annie Eye" nuclei * Psammoma bodies * Intranuclear inclusions / invaginations
121
What is the clinical course of Papillary CA?
* Present as asymptomatic nodule * May spread to cervical nodes * Overall good prognosis
122
Follicular variant Papillary thyroid CA * Histology * Mutation * Prognosis
* Histology * No papillae * Follicular pattern * Orphan Annie Eye nuclear change * Mutation * RAS mutations * BRAF * Prognosis * Encapsulated form has best prognosis * Infiltrative form more aggressive
123
Tall Cell Variant of PTC * Population * Histology * Mutation * Prognosis
* Population * older individuals * Histology * Tall columnar cells * Mutation * BRAF * RET/PTC translocation * Prognosis * Higher incidence of extension, vascular invasion, and metastasis * More aggressive
124
Diffuse Sclerosing Variant of PTC * Population * Histology * Mutation * Prognosis
* Population * children and young adults * Histology * papillae mixed with squamous cell nests * fibrosis * Mutation * RET/PTC mutation * Prognosis * 1/2 nodal metastasis
125
Are papillary microcarcinomas malignant?
They are precursors to standard PTC
126
Which Thyroid Carcinoma has increased incidence in areas of endemic iodine deficiency?
Follicular Carcinoma
127
What is the gross and microscopic morphology of Follicular Carcinoma?
* Gross * Slowly growing painless nodule * Single nodule * May be well circumscribed or widely infiltrative * Micro * Follicular cell pattern w/ nest or sheet of cells * No Orphan Annie Eye nuclei, papillae, or psammoma bodies
128
How do metastasis travel in papillary vs follicular thyroid CA?
* Papillary * via lymphatics * Follicular * via blood
129
Levels of what molecule are followed for recurrence of Follicular TC?
Thyroglobulin
130
Anaplastic Thyroid CA * Morphology * Clinical Course
* Morphology * Giant cells * Spindle cells * Clinical Course * Rapidly growing bulky mass of neck * Can metastasize * Death in less han a year
131
Medullary Carcinoma * Cells of origin * Mutations * Morphology * Clinical course
* Cells of origin * Parafollicular C cells * secrete calcitonin * Mutations * RET * MEN 2A and 2B * Familial forms * Morphology * Neoplastic cells in amyloid stroma * Clinical course * May have paraneoplastic syndromes * Diarrhea (VIP) * Cushing's syndrome (ACTH)
132
What is the most aggressive form of medullary TC?
MEN 2B
133
What lines Thyroglossal duct cyst?
Squamous or respiratory epithelium Thyroid tissue
134
What disease process is pictured below?
Follicular adenoma
135
What disease process is pictured below?
Follicular CA of Thyroid
136
What disease process is pictured below?
L: Follicular Adenoma R: Follicular CA of Thyroid
137
What disease process is pictured below?
Subacute De Quervain Granulomatous Thyroiditis
138
What disease process is pictured below?
Graves
139
What disease process is pictured below?
Hashimoto Thyroiditis
140
What disease process is pictured below?
Medullary CA of Thyroid
141
What disease process is pictured below?
Multinodular goiter
142
What disease process is pictured below?
Papillary CA of Thyroid
143
What is the most common cause of primary hyperparathyroidism?
Adenoma
144
What genetic mutations are associated with sporadic adenoma?
Cyclin D1 gene overexpression MEN 1 gene mutation even in absence of MEN 1
145
What is the function of the MEN 1 gene? What manifestations does it cause in primary hyperparathyroidism?
* Function * Tumor suppressor * Manifestation * adenoma * hyperplasia
146
What is the function of the MEN 2A gene? What manifestation does it cause in primary hyperparathyroidism?
* Function * mutation in RET gene * activates tyrosine kinase receptor * Manifestation * Parathyroid hyperplasia
147
What is the morphology of Parathyroid adenomas?
Loss of fat Predominantly composed of chief cells 0.5 - 5.0 gm
148
Familial Hypocaliuric Hypercalcemia * Inheritance * Mutation * Morphology * Course
* Inheritance * AD * Mutation * CASR LOS mutation (Ca sensing gene) * Increases PTH * Hypercalcemia and hypocalciuria * Morphology * mild hyperplasia * Course * benign
149
What is the morphology of Primary Parathyroid Hyperplasia?
* All glands are involved * Weight of all glands less than 1.0 gm * Hyperplasia * Chief cell hyperplasia * Oxyphil / water clear cell hyperplasia * Decreased stromal fat
150
What is the morphology of Parathyroid carcinomas?
One gland involved up to 10 gm in size
151
What determines malignancy in Parathyroid CA?
Invasion or metastasis | (Cell histology is unreliable)
152
What are the labs for Primary Hyperparathyroidism? Hypercalcemia of cancer?
* Primary Hyperparathyroidism * Increased PTH * Increased Ca * Decreased PO4 * Hypercalcemia of Cancer * increased PTH-related protein * Low - Normal PTH * Increased Ca * Normal - Increased PO4
153
What symptoms are associated with Primary Hyperparathyroidism?
"Painful bones, renal stones, abdominal groans, and psychic moans"
154
What is the most common cause of Secondary Hyperparathyroidism?
Renal Failure
155
What is the mechanism of Secondary Hyperparathyroidism?
Chronic renal failure * Decreased PO4 excretion and increased serum lvls * Phosphorus binds free calcium * Decreased free Ca * Stimulation of PTH * Increased Ca * (Also low levels of Vit D)
156
What is the morphology,clinical course, and treatment for Secondary Hyperparathyroidism?
* Morphology * Hyperplasia * Course * Renal osteodystrophy * bone probs * Calciphylaxis * blood vessel calcification leading to damage to other orgnas * Treatment * Vit D supplementation * PO4 binders
157
What is tertiary hyperparathyroidism?
_Autonomy of PTH activity_ in those with secondary hyperparathyroidism, resulting in hypercalcemia
158
What are the components of DiGeorge syndrome?
* Absence of parathyroid glands * Thymic aplasia * CV defects
159
Mutation in the AIRE gene results in what?
Autoimmune Hypoparathyroidism | (AutoImmune REgulator = AIRE)
160
Mutation in CASR results in what? What is its function?
AD Hypoparathyroidism Function: Ca sensing, mutation causes hightened sensing
161
What is Chvostek sign? What diagnosis does it indicate?
* Sign * Tapping along course of f_acial nerve_ * If it induces _contraction_ of the muscles of the eye, mouth, or nose, it may be due to subclinical hypoparathyroidism
162
What is Trousseau sign? What diagnosis does it indicate?
* Sign * inflate a bp cuff for several minutes * induces carpal spasm * Diagnosis * hypoparathyroidism
163
What are the clinical manifestations of hypoparathyroidism?
* Tetany * Circumoral numbness * Parasthesias * Mental status changes * Cataracts * Calcification of the lenses * Prolonged QT * Conduction abnormalities
164
Hypoparathyroidism causes calcification in what area of the brain?
Basal ganglia
165
What is the cause of Pseudohypoparathyroidism?
end-organ resistance to PTH
166
Albright hereditary osteodystrophy * Inheritance * Type of disease * Symptoms * Labs
* Inheritance * AD * Type of disease * Pseudohypoparathyroidism * Cause * end-organ resistance to: * PTH * TSH * LH/FSH * Symptoms * Short stature * obesity * Short metacarpal and metatarsal bones * dimples replace 3rd and 4th knuckles * Labs * Elevated PTH * Decreased Ca * Increased PO4
167
What is pseudopseudohypoparathyroidism?
Same physical findings as AHO but w/o PTH, Ca, and PO4 abnormalities
168
What are the criteria of malignancy for Pancreatic Neuroendocrine Tumors (PanNETs)?
* Local invasion * Vascular invastion * Metastasis NOT histology
169
Are most PanNETs benign or malignant?
malignant Except insulinomas (90% benign)
170
What genetic alterations are associated with Sporadic PanNETs?
* MEN1 * PTEN / TSC2 * activate oncogenic TOR signaling pathway * ATRX / DAXX * activating mutations
171
A Patient presents with confusion after exercising heavily. Blood sugar is below 50. Labs show high levels of insulin. The patient is given glucose and promptly recovers. What is the diagnosis?
Insulinoma
172
What is the histology of Insulinoma?
* Solitary tumor * Deposition of amyloid in extracellular tissues
173
What is the treatment of Insulinoma?
Surgical removal
174
What is the "Gastrinoma Triangle"?
Duodenum, peripancreatic soft tissue, and pancreas Areas where Gastrinomas may occur
175
Patient presents multiple gastric and duodenal ulcers. They are unresponsive to therapy. What is the diagnosis?
Gastrinoma | (aka Zollinger Ellison Syndrome)
176
What is the presentation in Alpha-cell PanNETs? What population is most affected?
* Presentation * High glucagon levels * Mild diabetes * Rash * Necrolytic migrating erythema * Anemia * Population * Peri and post menopausal women
177
What is the presentation in Delta cell PanNETs?
* Increased somatostatin * Diabetes mellitus * Cholelithiasis * Steatorrhea * Hypochlorhydria
178
What is the presentation in VIPoma?
WDHA Syndrome * Watery Diarrhea * Hypokalemia * Achlorhydria
179
What disease process is pictured below?
Solitary Chief Cell Parathyroid Adenoma
180
What disease process is pictured below?
Insulinoma
181
What disease process is pictured below?
Dissecting Osteitis
182
What disease process is pictured below?
Craniopharyngeoma
183
What cells are pictured below?
beta cells of the pancreas
184
What cells are pictured below?
L: alpha cells R: delta cells of pancreas
185
What is the normal blood glucose and HbA1c measurements?
Blood glucose: 70-120 HbA1c: 4-6%
186
What are the diagnostic values of the following for Diabetes Mellitus? * Random glucose * Fasting glucose * OGTT * HbA1c
* Random glucose * \>200 * Fasting glucose * \>126 * OGTT * \>200 * HbA1c * \>6.5%
187
What are the diagnositc values of the following for pre-diabetes? * Fasting glucose * OGTT * HbA1c
* Fasting glucose * 100-125 * OGTT * 140-199 * HbA1c * 6 - 6.5%
188
Which type of diabetes is increased by adiposity?
Type 2 It increases insulin resistance by downregulating receptors
189
What are the differences between Type 1 and Type 2 DM histologically?
* Islet size * T1: decreased number and size * T2: Subtle reduction in mass * Other * T1: Leukocytic infiltrate in islets (insulinitis) * T2: Amyloid deposition in islets; may have fibrosis
190
What type of arteriolosclerosis is present in diabetic macrovascular disease?
Hyaline arteriolosclerosis: diffuse thickening of membranes Leaky capillaries
191
What do the afferent and efferent glomerular hilar arterioles show in diabetic nephropathy?
Hyalnie thickening Involvement of both is characteristic of DM
192
What are the gross findings of chronic pyelonephritis in diabetics?
Shurnken kidneys Deep U-shaped scars
193
What causes Diabetic Retinopathy?
Hypoxia-induced overexpression of VEGF in the retina
194
How does Diabetic neuropathy present in the upper extremity?
Glove and stocking pattern
195
What causes the increased susceptibility to infection in DM?
Defects in: * Chemotaxis * Endothelial adherance * Microbicidal activity * Cytokine production And vascular compromise
196
What disease process is pictured below?
Amyloidosis in Type 2 DM - induced Diabetic nephropathy
197
What disease process is pictured below?
Diabetic nephropathy
198
What disease process is pictured below?
Insulinitis: leukocytic infiltrate in islet Type 1 DM
199
What disease process is pictured below?
Nephrosclerosis with long-standing DM
200
What is the most common cause of Cushing Syndrome?
Iatrogenic
201
What is Cushing disease?
Pituitary hypersecretion of ACTH
202
hat is the most common cause of CUshing Disease?
ACTH producing microadenoma
203
What is the most common cause of ectopic ACTH secretion in Cushing Syndrome?
Bronchogenic (Oat Cell) CA
204
What is Massive Macronodular Adrenocortical Disease?
Primary adrenal hyperplasia Over-expression of various hormone receptors causes hyperplasia in response to ADH, GIP, LH
205
What is Microdulular - Primary Pigmented Nodular Adrenal Disease?
Primary Adrenal Hyperplasia Bilateral pigmented nodules May be assoc. w/ Carney's complex (PRKAR1A gene mutation)
206
What are the lab results for the following tests in Pituitary Cushing? * ACTH * Suppression of Cortisol w/ high dose Dexamethasone * ACTH level w/ CRH stimulation
* ACTH * Increased * Suppression of Cortisol w/ high dose Dexamethasone * \>50% * ACTH level w/ CRH stimulation * Increased
207
What are the lab results for the following tests in Adrenal Cushing? * ACTH * Suppression of Cortisol w/ high dose Dexamethasone * ACTH level w/ CRH stimulation
* ACTH * Decreased * Suppression of Cortisol w/ high dose Dexamethasone * \<50% * ACTH level w/ CRH stimulation * No change
208
What are the lab results for the following tests in Ectopic Cushing? * ACTH * Suppression of Cortisol w/ high dose Dexamethasone * ACTH level w/ CRH stimulation
* ACTH * Increased * Suppression of Cortisol w/ high dose Dexamethasone * \<50% * ACTH level w/ CRH stimulation * No change
209
What are the following lab values for Primary vs Secondary Hyperaldosteronism? * Aldosterone * Renin * Na * K
* Aldosterone * Primary = Increased * Secondary = Increased * Renin * Primary = Low * Secondary = High * Na * Primary = Increased * Secondary = Increased * K * Primary = Decreased * Secondary = Decreased
210
What mutation may be present in those with Bilateral Idiopathic Hyperaldosteronism?
KCNJ5 Encodes a Potassium Channel
211
What is Conn Syndrome? What population does it affect?
* Conn Syndrome * Aldosterone - secreting adenoma * Population * Men more than women * (Only endocrine disorder studies so far!)
212
What genetic mutation causes Glucocorticoid Remediable Hyperaldosteronism?
Rearrangement on chromosome 8 Puts aldosterone synthase under control of ACTH promoter Aldosterone production is under the control of the ACTH feedback loop
213
In what syndrome is aldosterone inhibited by dexamethasone?
Glucocorticoid remediable hyperaldosteronism?
214
What is the primary symptoms in hyperaldosteronism?
HTN
215
What are the symptoms of late-onset adrenal virilism in 21-OH deficiency?
* Appear in late childhood or adolescence * May be asymptomatic * Symptoms: * hirsuitism * Acne * Menstrual irregularities
216
What should be suspected in any neonate with ambiguous genitalia?
Congenital Adrenal Hyperplasia (CAH)
217
How does androgen excess present in males?
Enlargement of external genitalia Precocious puberty Oligospermia
218
How does androgen excess present in females?
* Masculinization * Infants * Clitoral hypertrophy * Psuedohermaphroditism * Post-pubertal * Oligomenorrhea * Hirsuitism * Acne
219
Increased plasma 17-hydroxyprogesterone is diagnostic of what syndrome?
21-OH deficiency
220
Waterhouse-Friderichsen Syndrome * Population * Cause * Signs / Symptoms
* Population * Most common in children * Cause: infectious * Neisseria meningitidis most common * Pseudomonas * Staph * H. influenza * Signs / Symptoms * Massive bilat adrenal hemorrhage * Adrenocortical insufficiency * Rapidly progressive hypotension * Shock * DIC
221
Autoimmune Polyendocrine Syndrome Type 1 * Cause * Symptoms
* APST1 * Form of Addison's * Cause * Mutation in AIRE (autoimmune regulator gene) * Ab to IL-17 and IL-22 * crucial to fungal immunity * Chronic Mucocutaneous Candidiasis * Symptoms * Ectodermay dystrophy * Assoc. w/ combo of autoimmune disorders
222
Autoimmune Polyendocrine Syndrome Type 2 * Population * Cause * Symptoms
* APST2 * Form of Addison's * Population * Early adulthood * Cause * Genetic HLA linkage * Symptoms * Autoimmune adrenalitis * w/ Autoimmune thyroiditis or Type I DM
223
Addison's Disease induced by carcinoma is most likely from mets from which organs?
* Lungs * Breast
224
What is the clinical course of Primary Adrenocortical Insufficiency?
* Hyperpigmentation * sun-exposed areas * pressure points * Hyponatrema * Volume depletion * HypoTN * Hyperkalemia * Possible Hypoglycemia
225
What is the screening test for Primary and Secondary Adrenocortical Insufficiency?
Low response to Cosyntropin Stimulation Test
226
What are the values of the following lab tests in Primary vs Secondary Adrenocortical Insufficiency? * Cosyntropin Stimulation Test * ACTH * ACTH test response
* Cosyntropin Stimulation Test * Primary: Low response * Secondary: Low response * ACTH * Primary: Increased * Secondary: Low * ACTH test response * Primary: None * Secondary: Increased cortisol
227
Are adrenal adenomas or carcinomas more common in children?
Carcinoma
228
Are carcinomas or adenomas more likely to be virilizing? Have Cushing Syndrome? Hyperaldosteronism?
* Virilizing = CA * Cushing = adenoma * Hyperaldosteronism = adenoma
229
What is the Rule of 10% in Pheochromocytoma?
* 10% extra-adrenal (paragangliomas) * 10% sporatic are bilateral (50% in familial) * 10% adrenal are malignant * 10% associated with familial (actually 25%) * 10% not associated with HTN
230
What is the histology of Pheochromocytoma?
Zellballen pattern (trabeculae or nests of cells) Prominent vascularity
231
How is malignancy determined?
Exclusively by metastasis
232
What labs are used to diagnose Pheochromocytoma?
* Metanephrines * VMA * (either in urine or plasma)
233
What are the abnormalities present in MEN-1? What mutation causes it?
* Abnormalities: the 3 P's * Pituitary adenomas * Most common: Prolactinoma * Parathyroid hyperplasia or adenoma * Pancreatic Neuroendocrine tumors * Mutation * MEN-1 gene on 11q13
234
What abnormalities are present in MEN-2A? What is the associated mutation?
* Abnormalities * Medullary Thyroid CA * Pheochromocytoma * Parathyroid hyperplasia * Mutation * RET gene * chromosome 10
235
What abnormalities are present in MEN-2B? What is the associated mutation?
* Abnormalities * Medullary Thyroid CA * Pheochromocytoma * Mucosal neuromas * Marfanous habitis * Mutation * RET gene
236
Is Medullary Thyroid CA more aggressive in MEN-2A or MEN-2B?
2B
237
What is the composition of Pineoblastomas? How does it spread? What population is most commonly affected?
* Composition * Primitive neuroectodermal cells * Spread * * Spread along CSF pathways * Population * Pediatrics
238
What is the composition of Pineocytomas? What symptoms result? What population is most commonly affected?
* Composition * more differentiated cells (glial, neuronal, retinal) * Symptoms * Headache * Visual disturbance * Mental deterioration * Population * Adults

Pathology (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions