Endocrine: Pathoma, BRS, FA Flashcards
1
Q
Benign tumor of anterior pituitary cells
A
Pituitary Adenoma
2
Q
May be functional (Hormone-producing) or non-functional
A
Pituitary Adenoma
3
Q
The nonfunctional version of this presents with mass effect.
A
Pituitary Adenoma
4
Q
Presents with bitemporal hemianopsia due to compression of optic chiasm
A
Non-functional Pituitary Adenoma
5
Q
Presents with hypopituitarism due to compression
A
Non-functional Pituitary Adenoma
6
Q
Presents as galactorrhea and amenorrhea in females
A
Prolactinoma
7
Q
Presents as decreased libido and headache in males
A
Prolactinoma
8
Q
Most common pituitary adenoma type
A
Prolactinoma
9
Q
Treatment is dopamine agonists
A
Prolactinoma
10
Q
Stains chromophobic
A
Prolactinoma
11
Q
Tumor with hypersecretion of growth hormone
A
Somatotropic adenoma
12
Q
Presents as gigantism in children with increased linear bone growth
A
Somatotropic adenoma
13
Q
Presents as acromegaly in adults (enlarged bones of hands, feet and jaw) and growth of visceral organs
A
Somatotropic adenoma
14
Q
Cardiac failure most common cause of death
A
Somatotropic adenoma
15
Q
Presents with enlarged tongue
A
Somatotropic adenoma
16
Q
Staining is acidophilic
A
Somatotropic adenoma
17
Q
Results in increased IGF-1 and somatomedin C
A
Somatotropic adenoma
18
Q
Secondary diabetes mellitus is often seen
A
Somatotropic adenoma
19
Q
Diagnosed by high GH and IGF-1 levels along with lack of GH suppression by oral glucose
A
Somatotropic adenoma
20
Q
Treatment is octreotide, GH receptor antagonists or surgery
A
Somatotropic adenoma
21
Q
Tumor secreting ACTH leading to Cushing syndrome
A
ACTH cell adenoma
22
Q
Insufficient production of hormones by the anterior pituitary gland
A
Hypopituitarism
23
Q
Symptoms arise with 75% of parenchyma is lost
A
Hypopituitarism
24
Q
Generalized pan-hypopituitarism
A
Pituitary Cachexia - Simmonds Disease
25
Caused by pituitary tumors (adults - adenomas and children - craniopharyngioma) and Sheehan Syndrome
Pituitary Cachexia - Simmonds Disease
26
Pregnancy related infarction of pituitary gland
Sheehan Syndrome
27
Gland doubles in size during pregnancy but blooo supply does not increase.
Sheehan Syndrome
28
Presents as poor lactation, loss of pubic hair, and fatigue
Sheehan Syndrome
29
Congenital defect of the sella
Empty Sella Syndrome
30
Herniation of the arachnoid and CSF into the sella compresses pituitary gland
Empty Sella Syndrome
31
Results in growth retardation (dwarfish) in children
Growth Hormone Deficiency
32
Results in increased insulin sensitivity (hypoglycemia), weakness, and anemia in adults
Growth Hormone Deficiency
33
Results in retarded sexual maturation in children
Gonadotropin Deficiency
34
Results in loss of libido, impotence, loss of muscle & facial hair in men, and amenorrhea and vaginal atrophy in women
Gonadotropin Deficiency
35
Results in secondary hypothyroidism
TSH Deficiency
36
Results in secondary adrenal failure
ACTH Deficiency
37
ADH deficiency
Central Diabetes Insipidus
38
Due to hypothalamic or posterior pituitary pathology
Central Diabetes Insipidus
39
Signs and symptoms all due to loss of free water
Central Diabetes Insipidus
40
Presents with polyuria, polydipsia, hypernatremia, and low urine osmolality
Central Diabetes Insipidus
41
Water deprivation test fails to increase urine osmolality
Central Diabetes Insipidus
42
Treatment is desmopressin
Central Diabetes Insipidus
43
Impaired renal response to ADH
Nephrogenic Diabetes Insipidus
44
Due to inherited mutation to ADH receptor or drugs such as lithium
Nephrogenic Diabetes Insipidus
45
Presents with polyuria, polydipsia, hypernatremia, and low urine osmolality
Nephrogenic Diabetes Insipidus
46
No response to desmopressin
Nephrogenic Diabetes Insipidus
47
Normal ADH levels
Nephrogenic Diabetes Insipidus
48
Water deprivation test fails to increase urine osmolality
Nephrogenic Diabetes Insipidus
49
Due to excessive ADH secretion
Syndrome of Inappropriate ADH Secretion
50
Most often due to ectopic production especially small cell carcinoma of the lung
Syndrome of Inappropriate ADH Secretion
51
Clinical features are based on retention of free water
Syndrome of Inappropriate ADH Secretion
52
Presents with hyponatremia and low serum osmolality
Syndrome of Inappropriate ADH Secretion
53
Presents with mental status changes, seizures and cerebral edema
Syndrome of Inappropriate ADH Secretion
54
Treatment is free water restriction, demeclocycline, conivaptan, tolvaptan
Syndrome of Inappropriate ADH Secretion
55
Serum aldosterone is low
Syndrome of Inappropriate ADH Secretion
56
Development of large pituitary adenomas following bilateral adrenalectomy due to loss of feeback inhibition on growth of pre-existing pituitary microadenomas
Nelson Syndrome
57
Cystic dilatation of thyroglossal duct remnant
Thyroglossal Duct Cyst
58
Presents as anterior neck mass
Thyroglossal Duct Cyst
59
Most common thyroid congenital anomaly
Thyroglossal Duct Cyst
60
Persistence of thyroid tissue at base of tongue
Lingual Thyroid
61
Presents as base of tongue mass
Lingual Thyroid
62
Increased level of circulating thyroid hormone
Hyperthyroidism
63
Presents with increase in basal metabolic rate (increased ATPase synthesis)
Hyperthyroidism
64
Presents with increase symapthetic nervous system activity (Increased Beta1-receptors)
Hyperthyroidism
65
Autoantibody IgG stimulates TSH receptor (Type II HS)
Graves Disease
66
Most common cause of hyperthyroidism
Graves Disease
67
Presents most commonly in women of childbearing age (20-40)
Graves Disease
68
Increased incidence in HLA-DR3 and HLA-B8 positive individuals
Graves Disease
69
Presents with a diffuse goiter
Graves Disease
70
Presents with exophthalmos and pretibial myxedema due to TSH receptors in orbit and shin that produce glycosaminoglycan
Graves Disease
71
Irregular follicles with scalloped colloid seen on histology
Graves Disease
72
Lab findings: Increased total and free T4 with Hypocholesterolemia, Increased serum glucose
decreased TSH
Graves Disease
73
Fatal complication of Grave's Disease
Thyroid Storm
74
Due to elevated catecholamines and massive hormone excess in response to stress.
Thyroid Storm
75
Presents as arrhythmia, hyperthermia, and vomiting with hypovolemic shock
Thyroid Storm
76
Treat with 3 P's: Propranolol, Propylthiouracil, and Prednisolone
Thyroid Storm
77
Combination of hyperthryoidism, nodular goiter, and absence of exophthalmos
Plummer Disease
78
Teratoma made up of thyroid tissue
Struma ovarii
79
Due to relative iodine deficiency
Multinodular Goiter
80
Nontoxic, but can become TSH-independent over time.
Multinodular Goiter
81
Follicular cells working independent of TSH due to mutation in TSH receptor
Toxic Multinodular Goiter
82
Increased release of T3 and T4
Toxic Multinodular Goiter
83
Thyrotoxicosis if a patient with iodine deficiency goiter is made iodine replete
Jod-Basedow Phenomenon
84
Hypothyroidism in neonates and infants
Cretinism
85
Presents as 6 P's: Pot-bellied, Pale, Puffy face, Protruding umbilicus, Protuberant tongue, and Poor Brain development
Cretinism
86
Causes: Maternal hypothyroidsm during pregnancy, thyroid agenesis, dyshormonogenetic goiter, and iodine deficiency
Cretinism
87
Most common cause in US is thyroid dysgenesis
Cretinism
88
Congenital defect in thyroid hormone production, usually with thyroid peroxidase
Dyshormonogenetic goiter
89
Hypothyroidism in older children or adults
Myxedema
90
Presents with myxedema, weight gain, cold intolerance, slow mentally
Myxedema
91
Common causes: Iodine deficiency, Hashimoto Thyroiditis, therapy for hyperthyroidism, and primary idiopathic
Myxedema
92
Decreased serum free T4 & T3. Increased serum TSH. Hypercholesterolemia
Hypothyroidism
93
Most common cause of hypothyroidism in regions with normal iodine nutrition
Hashimoto Thyroiditis
94
Autoimmune destruction of thyroid gland associated with HLA-DR5
Hashimoto Thyroiditis
95
Initially presents as hyperthyroidism due to follicle damage
Hashimoto Thyroiditis
96
Anti-TSH-R, Anti-thyroglobulin and antithyroid peroxidase antibodies are often present
Hashimoto Thyroiditis
97
Chronic inflammation with germinal centers and Hurthle cells are seen
Hashimoto Thyroiditis
98
Increased risk for B-Cell (Marginal zone) Lymphoma
Hashimoto Thyroiditis
99
Finding: Nontender but enlarged thyroid
Hashimoto Thyroiditis
100
Follows a viral infection
Subacute Granulomatous Thyroiditis (De Quervain)
101
Presents as tender thyroid with transient hyperthyroidism
Subacute Granulomatous Thyroiditis (De Quervain)
102
Self-limited hypothyroidism is seen
Subacute Granulomatous Thyroiditis (De Quervain)
103
More common in women
Subacute Granulomatous Thyroiditis (De Quervain)
104
Chronic inflammation with extensive fibrosis of thyroid gland
Reidel Fibrosing Thyroiditis
105
Presents as hypothyroidism with 'hard as wood' non tender thyroid gland
Reidel Fibrosing Thyroiditis
106
Fibrosis may extend to local structures such as an airway
Reidel Fibrosing Thyroiditis
107
Manifestation of IgG4-related systemic disease
Reidel Fibrosing Thyroiditis
108
Clinically mimics anaplastic carcinoma, but patients are younger with this and malignant cells are absent.
Reidel Fibrosing Thyroiditis
109
Benign proliferation of follicles surrounded by a fibrous capsule
Follicular Adenoma
110
Nonfunctional usually but can secrete thyroid hormone
Follicular Adenoma
111
Most common type of thyroid carcinoma
Papillary Carcinoma
112
Exposure to ionizing radiation in childhood is a major risk factor
Papillary Carcinoma
113
Comprised of papillae lined by cells with clear "Orphan-Annie eye" or "Ground-glass" nuclei and nuclear grooves.
Papillary Carcinoma
114
Often associated with psammoma bodies
Papillary Carcinoma
115
Often spreads to cervical lymph nodes, but prognosis is excellent
Papillary Carcinoma
116
Associated with a change in chromosome 10 forming a RET-PTC fusion gene that acts as tyrosine kinase
Papillary Carcinoma
117
Associated with mutations in B-type Ras Kinase (BRAF)
Papillary Carcinoma
118
Malignant proliferation of follicles (uniformly shaped) surrounded by a fibrous capsule with invasion through the capsule
Follicular Carcinoma
119
Cannot be distinguished from follicular adenoma by FNA
Follicular Carcinoma
120
Metastasis through the blood
Follicular Carcinoma
121
Prognosis is good but not as good as papillary carcinoma
Follicular Carcinoma
122
Malignant proliferation of parafollicular C Cells
Medullary Carcinoma
123
Results in high levels of Calcitonin being produced causing hypocalcemia
Medullary Carcinoma
124
Biopsy reveals sheets of malignant cells in an amyloid stroma
Medullary Carcinoma
125
Familial cases are due to multiple endocrine neoplasia 2A and 2B
Medullary Carcinoma
126
Associated with medullary carcinoma, pheochromocytoma and parathyroid adenomas
Multiple Endocrine Neoplasia 2A
127
Associated with medullary carcinoma, pheochromocytoma, and ganglioneuromas of oral mucosa
Multiple Endocrine Neoplasia 2B
128
Sporadic and familial forms are both associated with RET oncogene mutation
Medullary Carcinoma
129
Undifferentiated malignant tumor thyroid in elderly
Anaplastic Carcinoma
130
Often invades structures leading to dysphagia or respiratory compromise
Anaplastic Carcinoma
131
Poor prognosis.
Anaplastic Carcinoma
132
Excess PTH due to a disorder of a parthyroid gland
Primary Hyperparathyroidism
133
Most common cause is a parathyroid adenoma
Primary Hyperparathyroidism
134
SPoradic parathyroid hyperplasia and parathyroid carcinoma are less common causes
Primary Hyperparathyroidism
135
MOst often presents as asymptomatic hypercalcemia
Primary Hyperparathyroidism
136
Can also present with groans (constipation), stones (hypercalciuria), bones (osteitis fibrosa cystica), and psychiatric overtones (depression)
Primary Hyperparathyroidism
137
Lab findings: Increased PTH, increased serum calcium, decrease serum phosphate, increase urinary cAMP, and increase serum alkaline phosphatase
Primary Hyperparathyroidism
138
Also will present with ab or flank pain due to kidney stones or acute pancreatitis and also peptic ulcers
Primary Hyperparathyroidism
139
Cystic bone spaces filled with brown fibrous tissue
Osteitis fibrosa cystica
140
Also known as Von Recklinghausen disease of bone
Osteitis fibrosa cystica
141
Excess production of PTH due to a disease process extrinci to parathyroid gland
Secondary Hyperparathyroidism
142
Most common cause is chronic renal failure
Secondary Hyperparathyroidism
143
Lab findings: Increased PTH, decreased serum calcium, increased serum phosphate, and increase alkaline phosphatase.
Secondary Hyperparathyroidism
144
This occurs in spite of correction of hypocalcemia and pre-existing secondary hyperparathyroidism from chronic renal disease
Tertiary Hyperparathyroidism
145
Lab findings: Extremely high PTH and increased Calcium
Tertiary Hyperparathyroidism
146
Cause is development of an adenoma in a previously hyperplastic gland
Tertiary Hyperparathyroidism
147
Impaired hydroxylation of 25-hydroxycholecalciferol
Vitamin D-dependent rickets
148
Decreased absorption of calcium and increased PTH secretion
Vitamin D-dependent rickets
149
Causes include autoimmune damage, surgical excision, and DiGeorge Syndrome
Hypoparathyroidism
150
Findings: Muscle spasms when using blood pressure cuff (Trousseau sign) or tapping on facial nerve (Chvostek sign)
Hypoparathyroidism
151
Labs reveal decreased PTH and decrease serum calcium
Hypoparathyroidism
152
Also known as Albright Hereditary Osteodystrophy
Pseudohypoparathyroidism
153
Autosomal dominant unresponsiveness of kidney to PTH
Pseudohypoparathyroidism
154
Findings: Shortened fourth and fifth metacarpals and metatarsals, short stature, and hypocalcemia
Pseudohypoparathyroidism
155
Due to a mutation in GNAS1 that encodes G proteins that respond to hormones
Pseudohypoparathyroidism
156
Selective paternal imprinting results in a mother passing on a mutated GNAS1 to her offspring
Pseudohypoparathyroidism
157
Skeletal abnormalities without hormone dysfunction due to transmission of mutant paternal GNAS1 allele
Pseudopseudohypoparathyroidism
158
Excess Cortisol
Cushing Syndrome
159
Findings: Muscle weakness, moon facies, buffalo hump, and truncal obesity, abdominal striae, osteoporosis, hypertension, hyperglycemia
Cushing Syndrome
160
Causes: Exogenous corticosteroids (bilateral adrenal atrophy), primary adrenal tumor, ACTH-secreting tumor (bilateral adrenal hyperplasia), and paraneo-plastic ACTH secretion (bilateral adrenal hyperplasia)
Cushing Syndrome
161
When caused by a adrenal tumor, this can't be suppressed by exogenous adrenal steroids
Cushing Syndrome
162
When caused by a pituitary tumor, this can be suppressed by exogenous adrenal steroids
Cushing Syndrome
163
Hypercorticism due to a corticotropic adenoma of pituitary or multiple very small pituitary adenomas.
Cushing Disease
164
Excess Aldosterone
Hyperaldosteronism
165
Presents as hypertension due to sodium retention, hypkalemia,a nd metabolic alkalosis
Hyperaldosteronism
166
Aldosterone Secreting Adrenal Adenoma
Conn Syndrome
167
Most commonly due to sporadic adrenal hyperplasia. But also can be caused by adrenal adenoma (aldosteronoma) and adrenal carcinoma
Primary Hyperaldosteronism
168
Characterized by high aldosterone and low renin
Primary Hyperaldosteronism
169
Seen in activation of RAS due to CHF or renovascular hypertension
Secondary Hyperaldosteronism
170
Characterized by high aldosterone and high renin
Secondary Hyperaldosteronism
171
Excess specific adrenal hormones with bilateral adrenal hyperplasia
Congenital Adrenal Hyperplasia
172
Deficiency in cortisol and mineralcorticoids with an increase in sex hormones.
21-hydroxylase Congenital Adrenal Hyperplasia
173
Presents with hypotension and hyperkalemia and clitoral enlargement or precocious puberty
21-hydroxylase Congenital Adrenal Hyperplasia
174
Presents with increased mineralocorticoids and decreased cortisol and decreased sex hormones
17-alpha-hydroxylase Congenital Adrenal Hyperplasia
175
Presents with hypertension and Hypokalemia
17-alpha-hydroxylase Congenital Adrenal Hyperplasia
176
Presents with low-renin hypertension
11-Beta-hydroxylase Congenital Adrenal Hyperplasia
177
Presents with decreased aldosterone and cortisol, but an increase in aldosterone pre-cursors causing hypertension
11-Beta-hydroxylase Congenital Adrenal Hyperplasia
178
Functional and are components of MEN1, Carney Complex, and McCune-Albright Syndrome
Adrenocortical Adenoma
179
Functional and associated with Li Fraumeni and Beckwith-Wiedemann syndrome
Adrenocortical Carcinomas
180
Lack of adrenal hormones
Adrenal Insufficiency
181
Acute insufficiency of adrenal hormones
Waterhouse-Friderichsen Syndrome
182
Hemorrhagic necrosis of adrenal glands due to DIC in young children with N meningitidis infection (meningococcemia)
Waterhouse-Friderichsen Syndrome
183
Lack of cortisol exacerbates hypotension leading to death
Waterhouse-Friderichsen Syndrome
184
Progressive destruction of adrenal glands resulting in chronic insufficiency of adrenal hormones
Addison disease
185
Caused by autoimmune destruction most commonly in the West
Addison disease
186
Caused by TB most commonly in the developing world
Addison disease
187
Can be caused by metastatic carcinoma or infections
Addison disease
188
Clinical features: hypotension, hyponatremia, hypovolemia, hyperkalemia, weakness, and hyperpigmentation
Addison disease
189
Increased ACTH and MSH
Addison disease
190
Most common tumor adrenal medulla in children
Neuroblastoma
191
Originates from neural crest cells
Neuroblastoma
192
Presents as abdominal distention with firm, irregular mass that can cross the midline.
Neuroblastoma
193
Presents with Homovanillic acid in the urine
Neuroblastoma
194
Bombesin positive
Neuroblastoma
195
Associated with overexpression of M-Myc oncogene
Neuroblastoma
196
Tumor of chromaffin cells
Pheochromocytoma
197
Clinical features due to increased catecholamines: Hypertension, headache, palpitations, tachycardia and sweating
Pheochromocytoma
198
Diagnosed by increased serum metanphrines and increased 24-hour urine metanephrines and vanillylmandelic acid.
Pheochromocytoma
199
Rule of 10%'s: 10% malignant, 10% bilateral, 10% extra-adrenal, 10% calcify, 10% kids
Pheochromocytoma
200
Most common tumor of renal medulla in adults
Pheochromocytoma
201
Phenoxybenzamine is administered perioperatively to prevent hypertensive crisis
Pheochromocytoma
202
Insulin deficiency leading to a metabolic disorder characterized by hyperglycemia
Type 1 Diabetes Mellitus
203
Due to autoimmune destruction of beta cells by T lymphocytes
Type 1 Diabetes Mellitus
204
Characterized by inflammation of islets
Type 1 Diabetes Mellitus
205
Associated with HLA-DR3 and HLA-DR4
Type 1 Diabetes Mellitus
206
Manifests in childhood
Type 1 Diabetes Mellitus
207
Signs: High serum glucose, weight loss, low muscle mass, polyphagia, polyuria, polydipsia, glycosuria
Type 1 Diabetes Mellitus
208
Risk for ketoacidosis characterized by excessive serum ketones
Type 1 Diabetes Mellitus
209
Arises with stress in which glucagon release causes increased lipolysis to increase FFA's which are turned into ketone bodies
Diabetic Ketoacidosis
210
Results in hypoglycemia, anion gap metabolic acidosis, and hyperkalemia
Diabetic Ketoacidosis
211
Presents with Kussmaul respirations, dehydration, flu-like, and fruity smelling breath.
Diabetic Ketoacidosis
212
Insulin treatment is always necessary
Type 1 Diabetes Mellitus
213
Severe glucose intolerance but high insulin sensitivity
Type 1 Diabetes Mellitus
214
End-organ insulin resistance leading to a metabolic disorder characterized by hyperglycemia
Type 2 Diabetes Mellitus
215
Most common type of diabetes
Type 2 Diabetes Mellitus
216
Arises in middle-aged obese adults (obesity decreases receptors)
Type 2 Diabetes Mellitus
217
Strong genetic pre-disposition exists
Type 2 Diabetes Mellitus
218
Insulin levels are increased early, but later due to beta-islet cell exhaustion, will be decreased
Type 2 Diabetes Mellitus
219
Amyloid (amylin) deposition in islets.
Type 2 Diabetes Mellitus
220
Low glucose intolerance and low insulin sensitivity
Type 2 Diabetes Mellitus
221
Risk for hyperosmolar non-ketotic coma (super high glucose)
Type 2 Diabetes Mellitus
222
Autosomal dominant syndrome characterized by mild hypglycemia and hyposecretion of insulin bu no loss of beta cells
Maturity-Onset Diabetes Mellitus of the Young
223
Insulitis marked by lymphocytic infiltration
Type 1 Diabetes Mellitus
224
Nonenzymatic glycosylation of large vessel vascular basement membranes leads to atherosclerosis
Diabetes
225
Nonenzymatic glycosylation of small vessel vascular basement membranes leads to hyaline arteriolosclerosis
Diabetes
226
Preferential involvement of efferent arterioles leads to glomerular hyperfiltration injury that progresses to nephrotic syndrome (Kimmelstiel-Wilson nodules)
Diabetes
227
Cataract formation, peripheral neuropathy, xanthomas, and abscesses are common
Diabetes
228
Component of MEN1 along with parathyroid hyperplasia and pituitary adenomas
Islet Cell Tumor
229
Present as episodic hypoglycemia with mental status changes that are relieved by administration of glucose (Whipple triad)
Insulinoma
230
Diagnosed by decreased serum glucose levels, increased insulin, and increased C-peptide
Insulinoma
231
Present as treatment resistant peptic ulcers that extend to duodenum and jejunum.
Gastrinoma
232
Known as Zollinger-Ellison syndrome
Gastrinoma
233
Known as an alpha cell tumor
Glucagonoma
234
Results in secondary diabetes mellitus
Glucagonoma
235
Causes skin lesion called necrolytic migratory erythema
Glucagonoma
236
Endocrine tumor marked by secretion of vasoactiv eintestinal peptide
VIPoma
237
Associated with "Watery diarrhea, Hypokalemia, and Achlorhydria (WDHA)"
VIPoma
238
Also known Verner-Morrison syndrome or pancreatic cholera
VIPoma
239
Also known as Wermer Syndrome
Multiple Endocrine Neoplasia 1
240
Hyperplasias or tumors of pituitary, parathyroid, or pancreatic islets (3P's)
Multiple Endocrine Neoplasia 1
241
Pancreatic portion presents as Zollinger-Ellison, hyperinsulinism, or pancreatic cholera
Multiple Endocrine Neoplasia 1
242
Linked to mutations in MEN I gene
Multiple Endocrine Neoplasia 1
243
Commonly presents with kidney stones and stomach ulcers
Multiple Endocrine Neoplasia 1
244
Known as Sipple Syndrome
Multiple Endocrine Neoplasia 2A
245
Presents with Parathyroid hyperplasia and Pheochromocytoma (2P's) Also includes medullary thyroid carcinoma
Multiple Endocrine Neoplasia 2A
246
Linked to mutations in ret oncogene
Multiple Endocrine Neoplasia 2A
247
Pheochromocytoma (1P) and also medullary thyroid carcinoma and mucosal neuromas.
Multiple Endocrine Neoplasia 2B
248
Linked to mutations in ret oncogene
Multiple Endocrine Neoplasia 2B
