17 systemic dz Flashcards
(268 cards)
1
Q
MPS underlying process and two basic syndromes
A
lack of enzymes to process glycosaminoglycans eg heparin/dermatan/keratin/chondroitin sulfate
hurler, hunter
2
Q
oral/facisl MPS
A
heavy brow ridges, impaected teeth w pronounced follicles, macroglossia
3
Q
lipid reticuloendothelioses fka, inheritance, underlying
A
AR, “lipid storage dzz”
no enzymes to process certain lipids
4
Q
Gaucher what’s lacking, what’s accumulating
A
lacks glucocerebrosidase
accum glucosylceramide
5
Q
Niemann-Pick what’s lacking, what’s accumulating
A
lacks acid sphyngomielinase
accum sphyngomyelin
6
Q
Tay-Sachs what’s lacking, what’s accumulating
A
lacks beta-hexosaminidase A
accum ganglioside
7
Q
Gaucher bones
A
Erlenmeyer glask deformity of femus
8
Q
Niemann-Pick type with identified gene
A
type C – NPC-1/2 gene mutation
9
Q
gaucher cells
A
lipid laden macrophages with bluish cytoplasm, wrinkled silk
10
Q
NPC-1/2 gene mutation
A
Niemann-Pick type C
11
Q
accum glucosylceramide
A
Gaucher
lacks glucocerebrosidase
accum glucosylceramide
12
Q
lacks glucocerebrosidase
A
Gaucher
lacks glucocerebrosidase
accum glucosylceramide
13
Q
lacks acid sphyngomielinase
A
Niemann-Pick
lacks acid sphyngomielinase
accum sphyngomyelin
14
Q
accum sphyngomyelin
A
Niemann-Pick
lacks acid sphyngomielinase
accum sphyngomyelin
15
Q
accum ganglioside
A
Tay-Sachs
lacks beta-hexosaminidase A
accum ganglioside
16
Q
lacks beta-hexosaminidase A
A
Tay-Sachs
lacks beta-hexosaminidase A
accum ganglioside
17
Q
lipid laden macrophages with bluish cytoplasm, wrinkled silk
A
gaucher cells
18
Q
niemann pick histo
A
bone marrow aspirate: sea blue histiocytes
19
Q
sea blue histiocytes
A
niemann pick bone marrow aspirate
20
Q
lipoid proteinosis aka, inheritance, clinical process, mutation
A
aka Urbach-Wiethe syndrome; hyalinosis cutis and mucosae
AR, deposition of waxy material in dermis and submucosa
ECM1 gene mutation
21
Q
ECM1 gene mutation
A
lipoid proteinosis
22
Q
deposition of waxy material in dermis and submucosa
A
lipoid proteinosis
23
Q
first sign of lipoid proteinosis
A
infant hoarse crying or unable to cry
24
Q
jaundice molecular reason and causes
A
exces Bb in blood and tissue
hemolytic anemia, liver malfunction, Gilbert syndrome (unconjugated Bb)
25
types of amyloid and where seen
AL -- amyloid IG light chain (organ limited, primary, and myeloma assoc)
AA -- acute phase proterin (secondary)
Abeta2 microglobulin -- hemodyalisis assoc
26
hemodialysis amyloid
Abeta2 microglobulin
27
acute phase amyloid
secondary -- AA
28
myeloma amyloid
AL -- amyloid light chain
29
primary amyloid
AL -- amyloid light chain
30
retinol aka
vit A
31
thiamin aka
B1
32
B1 aja
thiamin
33
vit A aka
retinol
34
B2 aka
riboflavin
35
riboflavin aka
B2
36
B3 aka
niacin
37
niacin aka
B3
38
pyridoxine aka
B6
39
B6 aka
pyridoxine
40
biotin aka
B7
41
B7 aka
biotin
42
folic acid aka
B9
43
B9 aka
folic acid
44
B12 aka
cobalamin
45
cobalamin aka
B12
46
B3 defcy
pellagra (3Ds)
47
pellagra
B3 defcy (3Ds)
48
vit D defcy
rickets/osteomalacia
49
Wernicke's encaphalopathy
B1 def
| vomiting, nystagmus, mental retardation
50
plummer vinson components
Fe def anemia, glossitis, dysphagia
51
Fe def anemia, glossitis, dysphagia
plummer vinson
52
spoon shaped nails
koilonychia, plummer vinson
53
koilonychia
spoon shaped nails, plummer vinson
54
pernicious anemia
autoimmune vit b12 defcy
| vit b12 defcy can be seen in other conditions
55
test to compare absorption and excretion of b12
Schirling test
56
Schirling test
test to compare absorption and excretion of b12
57
pituitary dwarfism whats up
low grwoth hormone production or tissues nt responding to growth hormone
58
gigantism whats going on and when seen
increased growth hormone production (usually 2/2 pituitary adenooma) before epiphyseal plates close
20% cases 2/2 McCune-Albright syndrome
59
increased growth hormone possibel scenarios
if before epiphyseal plates close --> gigantism
| if after --> acromegaly
60
hypothyroid in infancy
cretinism
61
hypothyroid --> gags
myxedema
62
hyperthyroid aka
thyrotoxicosis and Grave's dz
63
thyroid storm
release of large amts of T4 (eg after infx, stress)
64
hypoparathyroid conditions
DiGeroge and APECED
65
twicthing of upper lip when facial nerve is tapped below zygoma
Chvostek sign -- hypoCaemia
66
Chvostek sign
twicthing of upper lip when facial nerve is tapped below zygoma - hypoCaemia
67
primary and secondary hyperparathyroid
primary -- gland adenoma
| secondary -- chronic low Ca
68
classic triad of hyperparathyroid
```
stones (kidney)
bones (variety of changes)
abdominal groans (duodenal ulcers)
```
69
osteitis fibrosa cystica
central degeneration and fibrosis of long standing brown tumors
70
central degeneration and fibrosis of long standing brown tumors
osteitis fibrosa cystica
71
renal osteodystrophy
brown tumors in ESRD
72
brown tumors in ESRD
renal osteodystrophy
73
Cushing syndrome
hypercortisolism (called Cushing dz if endogenous)
| buffalo hump, moon face, HTN, DM
74
moon face
Cushing -- hypercortisolism
75
Addison's dz primary and secondary
primary hypoadrenocorticism (eg autoimmune, infx, tumors) or secondary (low ACTH)
76
addisonian crisis
acite episode of hypoadrenocorticism 2/2 ACTH suppression by corticoids
77
acite episode of hypoadrenocorticism 2/2 ACTH suppression by corticoids
addisonian crisis
78
oral consequences of diabetes
delayed healing, SG enlargement, gingival enlargement, fungal infx (Candida, Mucor), dry mouth, erythema migrans
79
buffalo hump
Cushing
80
hypophosphatasia mechanism and mouth
defcy of alkaline phosphatase
| premature loss of teeth 2/2 lacks cementum
81
defcy of alkaline phosphatase
hypophosphatasia
82
premature loss of teeth 2/2 lacks cementum
hypophosphatasia
83
vit D resistant rickets mutation
PHEX
84
PHEX
vit D resistant rickets
85
pyostomatitis vegetans manifestation of --
Crohn's or UC
86
snailk track ulcerations
pyostomatitis vegetans manifestation of Crohn's or UC
87
uremic stomatitis whats up
increased urea levels in chronic renal failure
88
increased urea levels in chronic renal failure
uremic stomatitis
89
face of mucopolysaccharidosis
cloudy cornea, heavy brow ridges, stiff joints, mental retardation
90
oral MPS
gingival hyperplasia, impacted teeth with pronounced follicle, macroglossia
91
examples of mucopolysaccharides (and aka?)
glycosaminoglycans
| heparin, dermatan, keratan, chondroitin sulfate
92
examples and inheritance of MPSs
AR except Hunter -- XLR
Hurler, Scheie, Hunter, Sanfillippo (A/B), Morquio (A/B), Maroteux-Lamy
93
lipid storage diseases better group name, examples, inheritance
AR, lipid reticuloendothelioses
lipids accumulate in macrophages
Gaucher, Niemann-Pick, Tay-Sachs
94
most common lipid storage disease
Gaucher
95
clinical types of gaucher
type 1 - non-neuronopathic, most in jewish
| types 2 and 3 - neuronopathic
96
niemann pick with known mutation
NPC-1 type C
97
lipoid proteinosis inheritance and mutation
AR - ECM1
98
face/head/mouth of lipoid proteinosis
face: thick yellow papules in lips and eyelids
thick furrowed skin
symmterical intrcranial calcifications
oral -- enlargement of tongue, lips, cheek;
smooth tongue can attach to floor of mouth
99
PAS quality in lipod proteinosis
PAS+
100
hemoglobin breakdown cascade
Hb--> Bb --> unconj in blood --> conj in liver --> excretion in bile
101
unconjugated bilirubin excess
Gilbert syndome
102
possible causes of excess Bb
hemolytic anemia, sickle cell anemia
liver malfunction
Gilbert syndome
reduced excretion in bile
103
ddx for jaundice
hypercarotenemia -- skin is yellow but sclera is not
104
protein structure of amyloid
beta sheets
105
amyloidosis by involvement
organ limited and systemic
106
types of systemic amyloid
primary; myeloma-assoc; secondary (TB, sarcoid, osteomyelitis); hemodyalisi assoc; heredofamilial
107
amyloid death
2/2 kidney or heart failure
108
molecular types of amyloid
AL -- organ limited, primary, myeloma assoc (Ig light chain)
AA - secondary (acute phase)
b2 microglobulin -- hemodialysis
109
scurvy underlying mechanisms
vit c reqd for post-translational proline hydroxylation of collagen chains --> weak vascular walls
bad healing
scorbutic gingivitis
110
rachitic rosary
prominence of costochondral junctions
111
prominence of costochondral junctions
rachitic rosary
112
symptoms of osteomalacia
bone fragility, fractures, pain
113
vit e defcy who's at risk
children w chronic cholestatic liver disease and malabsorption
--> CNS issues
114
risk for vit k defcy
malabsorption syndromes, abx, anticoagulants
115
most common anemia in US
fe def
116
most common form of anemia in world
fe def
117
reasons for fe def anemia
blood loss (menorrhagia, GI dusease)
need for RBC (pregnancy)
low iron intake (children, elderly)
low Fe absoprtion (celiac)
118
oral signs of fe def anemia
angular cheilitis and atrophic glossitis or generalized oral mucosa atrophy
painful tongue
119
achlorhydria
absence of gastric acid --> fe def anemia
120
howell jolly bodies
clusters of DNA in circulating RBCs -- Fe def anemia
121
plummer vinson aka
patterson kelly or sideropenic dysphagia
122
components of plummer vinson
fe def anemia, glossitis, dysphagia, angular cheilitis, esophageal wevs (abnormal bands of tissue)
increased risk for oral and esophagela SCC
spoon nails
123
spoon nails
koilonychia: plummer vinson
124
pernicious anemia aka and whats up
megaloblastic enamia
vut b 12 deficiency (cobalamin)
autoimmune destruction of intrinsic factor (needed for b12 absoprtion)
125
pernicious anemia also concurrent with
autoimmune gastritis
126
oral pernicious anemia
burning mouth, erythema/atrophy of tongue (may present as focal patchy areas)
127
compares absorption and excretion rates of vit b 12
schirling test
128
pernicious anemia diagnosis
serum AB against intrinsic factor
129
causes of pituitrary dwarfism
low GH production by anterior pituitary or tissues not responding to growth hormone
2/2 gland aplasia/hypoplasia
or destruction of pituitary/hypothalamus (RT, tumor)
130
pituitary dwarfism body morphology and oral
short stature but normal proportions
| smaller mx/md; delayed eruption, smaller teeth
131
gigantism timing
increased GH (usually from adenoma) before closure of epiphyseal plates
132
gigantism association
20% cases with McCune-Albright
133
gigantism head
enlarged sella bc adenoma
| oral -- generalized macrodontia
134
enlarged sella in skull
pituitary adenoma -- gigantism
135
closure of epiphyseal plates interferences
growth hormone:
- before closure --> gigantism
after closure --> acromegaly
136
common cause of acromegaly
increased GH (usually pituitary adenoma) after closure of epiphyseal plates
137
other problems in acromegaly
HTN, CHD, hyperhidrosis, arthritis, peripheral neuropathy
bones of hands and feet grow --> gloves and hats become small
138
orofacial acromegally
MD prognathism, anterior open bite, diastema, macroglossia, sleep apnea
coarse face
139
two types of hypothyroidism
cretinism -- in infancy (teeth fail to erupt)
| myxedema -- long standind --> spotigion of glycosaminoglycans
140
pimrary vs secondary hypothyroidism
primary -- most cases; problem in thyroid
| secondary -- pituitary not procuding TSH
141
example of destruction of thyroid
hashimoto
142
myxedema systemic and oral
decreased metabolism -- bradycardia, hypothermia
| facial, lip, tongue swelling
143
labs in hypothyroid
```
low T4
TSH elevated (primary) or normal (secondary)
```
144
most common cause of hyporthyroid
90% - graves dz
| auto AB to TSH receptors stimulate thyroid cells
145
graves dz general presentation
warm moist skin, fine tremor, sensitivity to heat, diffuse thryoid enlargement, exophthalmos
146
non graves causes of hyperthyroid
thyroid tumors (produce hormones) and pituitary adenoma (produces TSH)
147
hyperthyroid labs
high T4, low TSH
148
hyperthyroid treatment
radioactive iodine (risk of hypothyroid)
149
thyroid storm
release of large amounts of T4 at one time -- eg after infx, trauma, stress
delirium, convulsion, fever tachycardia
150
PTH function
takes Ca from bone to blood, in conjunction with Vit D
151
causes of hypoPTH
surgical removal of parathyroid or autoimmune
152
2 syndromes with hypoPTH
diGeorge and APECED
153
Ca levels in hypoPTH
hypoCaemia but asymptomatic; may lead to tetany tho
154
chvostek sign for what and how
hypoCaemia
| twitching of upper lip when facial nerve is tapped below zygoma
155
twitching of upper lip when facial nerve is tapped below zygoma
chvostek sign
| hypoCaemia
156
test for hypoCaemia
chvostek sign
| twitching of upper lip when facial nerve is tapped below zygoma
157
teeth in hypoPTH
if young -- enamel pitting and failure of teeth to eript
158
persistent oral candidiasis in a young patient
APECED
159
labs and tx for hypoPTH
low PTH and Ca
| tx - vit D precursors
160
pseudohypoPTH aka and what is
Albright hereditary osteodystrophy; acrodysostosis
| normal levels of PTH but dysfx pathway -- symptoms of hypoPTH
161
types of pseudohypoPTH
1a -- Gsa defect, cAMP defcy; most common. obesity, short neck, short/thick fingers, round face, osteoma cutis, hypogonadism, hypoTHY
1b - PTH receptors, pt normal but w hypoCaemia
Ic - adenylate cyclase of Gsa, same as Ia
II - cAMP induced but cells don't respond. pt normal but hypoCaemia
162
pseudohypoPTH when pt normal but hypoCaemia
Ib -- PTH recepetors
| and II - cAMP induced but cells don't respond. pt normal but hypoCaemia
163
pseudohypoPTH and obesity
Ia ( Gsa defect, cAMP defcy; most common. obesity, short neck, short/thick fingers, round face, osteoma cutis, hypogonadism, hypoTHY)
and Ic - adenylate cyclase of Gsa, same as Ia
164
pseudohypoPTH when no PTH receptors
1b - PTH receptors, pt normal but w hypoCaemia
165
Gsa defect and cAMP
cAMP deficient -- pseudohypoPTH type Ia
cAMP induced -- type Ic
166
dental pseudohypoPTH
enamel hypoplasia, oligodontia, dagger-shaped pulp stones, blunt apices
167
dagger-shaped pulp stones and blunt apices
pseudohypoPTH
168
labs and tx for pseudohypoPTH
dx: elevated PTH but low Ca
tx: vit D and Ca
169
primary vs secondary hyperPTH
primary -- 90% gland adenoma
| secondary -- chronic low Ca from chronic renal dz; vit D not activated and Ca not absorbed
170
function of PTH
mobilization of Ca from bone
increases renal tubular reabsorption of Ca
promotes renal prodiction of ,25 dihydroxyvitD
increases serum Ca
171
hyperPTH syndromes
MEN1 and MEN2A and hyperPTH-jaw tumor syndrome
172
hyperPTH triad
kidney stones (elevated Ca), bones (various), abdominal groans (duodenal ulcers)
173
central degeneration and fibrosis of long standing brown tumor
osteitis fibrosa cystica -- hyperPTH
174
brown tumor
hyperPTH
175
renal osteodystrophy and PTH
secondary hyperPTH
| striking jaw enlargement
176
bones of hyperPTH
brown tumor, subperiosteial bone resopriton (esp phalanges), generalized osteopenia, loss of lamina dura, blurring of trabeculae (ground glass)
177
hyperPTH relevant histo
CGCG --
| trabeculare of woven bone + giant cells in fibrous background
178
adrenal hormonal pathway
pituitary: ACTH --> adrenal: corticoids
179
cushing syndrome vs cushing dz
exogenous corticouds -- syndrome
| endogenous (adrenal/pituitary adenoma) Cushing dz (rare, most in young F)
180
cushing aka
hypercortisolism
181
hypercortisolism signs
central weight gain, buffalo hump, moon facies, HTN< diabetes, osteoporosis
182
dexamethasone test
normal pts -- cortisol and ACTH decrease
| tumors don't respond to feedback loop
183
addisonian crisis
acute episode of hypoadrenocorticism
| after withdrawal of corticoids (drugs suppress pituitary -- can't produce ACTH)
184
nevi, atrial muxoma, myxoid NF, ephelides, hypercortisolism
subest of Carney complex
185
hypoadrenocorticism aka
addison's dz
| low corticosteroids 2/2 adrenal dysfx
186
causes of addison
autoimmune, infections (TB, deep fungal (histo, PCM), mets, sarcoid, amyloid
187
fungi that can destroy adrenals
histoplasma and paracoccidio
188
secondary hypoadrenocorticism
low corticoids 2/2 low ACTH from pituitary dysfx
189
how soon does hypoadrenocorticism manifest
only when 90% of gland destroyed
190
skin in addison
bronzing
| increased beta-lipotropin and ACTH stimulate melanocytes
191
addison + hypoparatHY + mucocutaneous candidiasis
APECED
192
oral addison
```
diffuse pigmentation of oral mucosa
sudden onset (vs physiologic)
```
193
addison labs
```
low serum cortisol (<20 ug/dL)
high ACTH (primary) or low ACTH (secondary)
```
194
sheehan syndrome
acute adrenal cortical insufficiency caused by meningococci
195
acute adrenal cortical insufficiency caused by meningococci
waterhouse friedrichsen
196
diabetes: type 1 vs type 2
type 1 - insulin dependent (lack insulin; AI destruction of pancreas islet cells)
type 2 - non insulin dependent -- insulin resistance; genetic + environmental
197
ketoacidosis
more common in DM type 1
| no glucose --> fat and protein used for energy --> ketones as byproduct
198
blood vessels and diabetes
microangiopathy: occlusion of small vessels 2/2 uptake of glucose by EC
- -> peripheral vascular dz, ischemia --> renal failure, gangrene of limbs, neuropathy, inability to figh infx, infarct, stroke, blindness
199
diabetic nephropathy
2/2 microangiopathy
| aka kimmelstein-wilson dz
200
kimmelstein-wilson dz
diabetic nephropathy
201
oral DM
perio dz, delayed healing, sialadenosis, gingival enlargement, fungal infx (candida and mucor), xerostomia
202
metformin action
increases glucose use, reduces insulin resistance
203
insulin shock
type 1 pt:
| if no carbs right after insulin injection, glucose levels may fall dramatically (below 40mg/dL)
204
hypophosphatasia what's the defect
deficiency of alkaline phosphatase
- reduced alk phos in bone, liver, kidney
increased blood and urinary phosphoethanolamine
- bone abnormalities similar to rickets
205
hypophosphatasia forms and features
perinatal -- death in a few hours 2/2 respir failure
infantile -- short bowed limbs (6months and older), similar to rickets
premature shedding of deciduous teeth (teeth lack cement)
childhood: loss of primary teeth (mostly incisors), bowed legs, short stature; beaten copper skull (uniformly spaced, poorly defined radiolucency) 2/2 thinning of cortical plate
adult: mild disease; history of loss of primary or permanent dentition
206
hypophosphatasia labs
clinical + low serum alk phos + high serum phosphoethanolamine
207
vit d resistant rickets what is
clinical features of rickets but no response to vit D tx
208
vit d resistant rickets genetics and inheritance
PHEX
X-linked dominant
F affected less severely 2/2 lyonization
209
labs and looks of vit d resistant rickets
rachitic changes + hypophosphatemia + decreased reabs of P in kiney
short stature, bowed lower limbs
210
teeth of vit d resistant rickets
enlarged pulps, elongated pulp horns extend into incisal edge just below DEJ
large calcospherites of globular dentin
small exposures cau cause necrosis in healthy teeth --> multiple abscesses in primary dentition
211
enlarged pulps, elongated pulp horns extend into incisal edge just below DEJ
lage calcospherites of globular dentin
vit d resistant rickets
212
tx for vit d resistant rickets
calcitriol and phosphate
213
vit d dependent rickets inheritance and defect
AR, lacks 1 alpha hydroxylase (activates vit D)
214
vit d dependent rickets teeth
hypocalcification of teeth
215
hypocalcification of teeth which rickets
vit d dependent
216
elongated pulp horns extend into incisal edge just below DEJ which rickets
vit d resistant
217
vit d dependent vs resistane rickets teeth
dependent -- hypocalcification; resistant -- elongated pulp horns extend into incisal edge just below DEJ
218
crohn's site of action
affects distal portion of small bowel and proximal colon (skip lesions)
can affect any region from mouth to anus
also extra-GI like eyes, skin
219
skip lesions which affliction
crohn's
220
how common is oral crohn's and what are the varieties
first sign of dz in 30% of cases
swelling of oral tissues, cobblestone, deep granulomatous ulcers (linear and in buccal vestibule)
also Inflammatory fibrous hyperplasia like or aphthous like
mucognigivitis (patchy erythematous macules and plaques)
221
pyostomatitis vegetans look and reason
inflammatory bowel dz - Crohn's and ulcerative colitis
| yellowish linear serpentine pustules over erythematous mucosa (snail track ulcerations)
222
(snail track ulcerations)
pyostomatitis vegetans
223
pyostomatitis vegetans locations
buccal/labial, soft palate, ventral tongueu
224
histo pyostomatitis vegetans
acantholytic with intra or subepithelial eosinophilic abscesses
225
acantholytic with intra or subepithelial eosinophilic abscesses
pyostomatitis vegetans
226
uremic stomatitis reason
urease degrades urea in saliva and releases ammonia --> damages oral mucosa
227
uremic stomatitis locations
painful white plaques, cheek, tongue, FOM
228
uremic stomatitis ddx and how
oral hairy leukoplakia
| ammonia/urine odor
229
uremic stomatitis tx
clears with dialysis in up to 3 weeks
| also helps to dilute H2O2 as rinse
230
gaucher, niemann-pick, tay-sachs
what's lacking
what accumulates
where
Gaucher: glucocerebrosidase; glucosylceramide in lysosomes of macrophages
NP: sphingomyelinase: sphingomeyline in lysosomes of macrophages
Tay-Sachs: beta-hexosaminidase - gandlioside in lysosomes of neurons
231
neuronopathic niemann pick types
A and C
232
visceral involvement niemann pick
type B - liver, spleen, lung
233
molecular/cellular source of lipoid proteinosis
collagen 4, 5, laminin
| from basement membrane
234
lipoid proteinosis brain
medial temporal lobe calcifications in 70% --> seizures
235
three stains of amyloid
1-congo red
2-Crystal violet
3-thioflavineT
236
blindness vitamin
A
237
beri beri vitamin
B1 (thiamin)
238
pellagra vitamin
B3 (niacin)
239
rachitic rosary
prominence of costachondral junction in Vit D resistant Rickets (looks like
beads / nodules)
240
dagger pulp
pseudohypoPTH
241
high PTH, low serum Ca, high serum P, normal renal dx
pseudohypoPTH
242
common cause of hypoTHY
hashimoto
243
labs in thyroid
primary hypo: low T4, high TSH
secondary hypo: low T4, normal TSH
hyperTHY: high T4, low TSH
244
common cause of hyperTHY
graves (autoAB to TSH receptors --> make T4)
245
low PTH, low serum Ca, , high P, Normal
| Kidney
hypoPTH
246
chromosome digeorge and aka
22
| velo-cardiofacial
247
ground glass
fibrous dysplasia and hyperPTH
248
synromes w primary hyperPTH
MEN1, MEN2A
249
high PTH, high Ca
pimary hyperPTH
250
high PTH, low Ca
secondary hyperPTH
251
resorption of middle and index finger
hyperPTH
252
addison labs
Hyponatremia = low soium / crave salt
Low cortisol : 18μg
Primary: High ACTH ( over 100ng/L )
253
salt craving
addison
254
Hypoparathyroidism
Hypoaldosteronism
Candida
Polyendocrinopathy –candida-ectodermal
| dystrophy syndrome:
255
(20mg of
prednisone on a daily basis for several
months)
cushing
256
cushing face, body
```
Buffalo hump / moon face / weight gain
Red / purple abdominal striae
Hirsutism
Osteoporosis
Depression
Hyperglycemia with thirst and polyuria
Poor healing
```
257
Suppress production of
ACTH in Pituitary gland and cannot respond to
stress due to exogenous corticosteroids
Addisonian crisis:
258
biggest killer in diabetes
peripheral vascular disease
259
insulin shock
below 40mg/dl
260
sugar concentration at which kidney can't absorb
over 300 mg.dL
261
head and neck diabetes
```
Microangiopathy
Diabetic sialadenosis
Oral canida
Mucormycosis
Goegraphic tongue
xerostomia
```
262
types of hypophosphatasia
1. Perinatal letha: death within hours
2. Perinatal benign
3. Infanile nomal upo 6m hen failure to grow
4. Childhood: Pemature closue of cranial sutures /beaten copper
5. Adult: Metatarsal bones of feet
6. Odonohypophosphatasia: Premature loss of anterior teeth is the only clinical sign
263
labs in hypophosphatasia
low alk phos, high serum and urine phosphotheanolamine
264
rickets inheritance
vit d resistant -- XLD
| vit d dependent -- AR
265
PHEX
vit d resistant rickets
266
1α-hydroxylase
vit d dependent rickets
267
snail track
Pyostomatitis vegetans:
| associated with crohn or ulcerative colitis
268
uremic stomatitis ddx
oral hairy leukoplakia
