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OP - Yield > 17 systemic dz > Flashcards

17 systemic dz Flashcards

1
Q

MPS underlying process and two basic syndromes

A

lack of enzymes to process glycosaminoglycans eg heparin/dermatan/keratin/chondroitin sulfate

hurler, hunter

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2
Q

oral/facisl MPS

A

heavy brow ridges, impaected teeth w pronounced follicles, macroglossia

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3
Q

lipid reticuloendothelioses fka, inheritance, underlying

A

AR, “lipid storage dzz”

no enzymes to process certain lipids

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4
Q

Gaucher what’s lacking, what’s accumulating

A

lacks glucocerebrosidase

accum glucosylceramide

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5
Q

Niemann-Pick what’s lacking, what’s accumulating

A

lacks acid sphyngomielinase

accum sphyngomyelin

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6
Q

Tay-Sachs what’s lacking, what’s accumulating

A

lacks beta-hexosaminidase A

accum ganglioside

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7
Q

Gaucher bones

A

Erlenmeyer glask deformity of femus

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8
Q

Niemann-Pick type with identified gene

A

type C – NPC-1/2 gene mutation

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9
Q

gaucher cells

A

lipid laden macrophages with bluish cytoplasm, wrinkled silk

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10
Q

NPC-1/2 gene mutation

A

Niemann-Pick type C

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11
Q

accum glucosylceramide

A

Gaucher
lacks glucocerebrosidase
accum glucosylceramide

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12
Q

lacks glucocerebrosidase

A

Gaucher
lacks glucocerebrosidase
accum glucosylceramide

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13
Q

lacks acid sphyngomielinase

A

Niemann-Pick
lacks acid sphyngomielinase
accum sphyngomyelin

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14
Q

accum sphyngomyelin

A

Niemann-Pick
lacks acid sphyngomielinase
accum sphyngomyelin

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15
Q

accum ganglioside

A

Tay-Sachs
lacks beta-hexosaminidase A
accum ganglioside

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16
Q

lacks beta-hexosaminidase A

A

Tay-Sachs
lacks beta-hexosaminidase A
accum ganglioside

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17
Q

lipid laden macrophages with bluish cytoplasm, wrinkled silk

A

gaucher cells

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18
Q

niemann pick histo

A

bone marrow aspirate: sea blue histiocytes

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19
Q

sea blue histiocytes

A

niemann pick bone marrow aspirate

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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

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21
Q

ECM1 gene mutation

A

lipoid proteinosis

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22
Q

deposition of waxy material in dermis and submucosa

A

lipoid proteinosis

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23
Q

first sign of lipoid proteinosis

A

infant hoarse crying or unable to cry

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24
Q

jaundice molecular reason and causes

A

exces Bb in blood and tissue

hemolytic anemia, liver malfunction, Gilbert syndrome (unconjugated Bb)

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25
Q

types of amyloid and where seen

A

AL – amyloid IG light chain (organ limited, primary, and myeloma assoc)

AA – acute phase proterin (secondary)
Abeta2 microglobulin – hemodyalisis assoc

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26
Q

hemodialysis amyloid

A

Abeta2 microglobulin

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27
Q

acute phase amyloid

A

secondary – AA

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28
Q

myeloma amyloid

A

AL – amyloid light chain

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29
Q

primary amyloid

A

AL – amyloid light chain

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30
Q

retinol aka

A

vit A

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31
Q

thiamin aka

A

B1

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32
Q

B1 aja

A

thiamin

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33
Q

vit A aka

A

retinol

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34
Q

B2 aka

A

riboflavin

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35
Q

riboflavin aka

A

B2

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36
Q

B3 aka

A

niacin

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37
Q

niacin aka

A

B3

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38
Q

pyridoxine aka

A

B6

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39
Q

B6 aka

A

pyridoxine

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40
Q

biotin aka

A

B7

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41
Q

B7 aka

A

biotin

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42
Q

folic acid aka

A

B9

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43
Q

B9 aka

A

folic acid

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44
Q

B12 aka

A

cobalamin

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45
Q

cobalamin aka

A

B12

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46
Q

B3 defcy

A

pellagra (3Ds)

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47
Q

pellagra

A

B3 defcy (3Ds)

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48
Q

vit D defcy

A

rickets/osteomalacia

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49
Q

Wernicke’s encaphalopathy

A

B1 def

vomiting, nystagmus, mental retardation

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50
Q

plummer vinson components

A

Fe def anemia, glossitis, dysphagia

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51
Q

Fe def anemia, glossitis, dysphagia

A

plummer vinson

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52
Q

spoon shaped nails

A

koilonychia, plummer vinson

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53
Q

koilonychia

A

spoon shaped nails, plummer vinson

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54
Q

pernicious anemia

A

autoimmune vit b12 defcy

vit b12 defcy can be seen in other conditions

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55
Q

test to compare absorption and excretion of b12

A

Schirling test

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56
Q

Schirling test

A

test to compare absorption and excretion of b12

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57
Q

pituitary dwarfism whats up

A

low grwoth hormone production or tissues nt responding to growth hormone

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58
Q

gigantism whats going on and when seen

A

increased growth hormone production (usually 2/2 pituitary adenooma) before epiphyseal plates close
20% cases 2/2 McCune-Albright syndrome

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59
Q

increased growth hormone possibel scenarios

A

if before epiphyseal plates close –> gigantism

if after –> acromegaly

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60
Q

hypothyroid in infancy

A

cretinism

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61
Q

hypothyroid –> gags

A

myxedema

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62
Q

hyperthyroid aka

A

thyrotoxicosis and Grave’s dz

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63
Q

thyroid storm

A

release of large amts of T4 (eg after infx, stress)

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64
Q

hypoparathyroid conditions

A

DiGeroge and APECED

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65
Q

twicthing of upper lip when facial nerve is tapped below zygoma

A

Chvostek sign – hypoCaemia

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66
Q

Chvostek sign

A

twicthing of upper lip when facial nerve is tapped below zygoma - hypoCaemia

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67
Q

primary and secondary hyperparathyroid

A

primary – gland adenoma

secondary – chronic low Ca

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68
Q

classic triad of hyperparathyroid

A 
stones (kidney)
bones (variety of changes)
abdominal groans (duodenal ulcers)
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69
Q

osteitis fibrosa cystica

A

central degeneration and fibrosis of long standing brown tumors

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70
Q

central degeneration and fibrosis of long standing brown tumors

A

osteitis fibrosa cystica

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71
Q

renal osteodystrophy

A

brown tumors in ESRD

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72
Q

brown tumors in ESRD

A

renal osteodystrophy

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73
Q

Cushing syndrome

A

hypercortisolism (called Cushing dz if endogenous)

buffalo hump, moon face, HTN, DM

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74
Q

moon face

A

Cushing – hypercortisolism

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75
Q

Addison’s dz primary and secondary

A

primary hypoadrenocorticism (eg autoimmune, infx, tumors) or secondary (low ACTH)

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76
Q

addisonian crisis

A

acite episode of hypoadrenocorticism 2/2 ACTH suppression by corticoids

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77
Q

acite episode of hypoadrenocorticism 2/2 ACTH suppression by corticoids

A

addisonian crisis

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78
Q

oral consequences of diabetes

A

delayed healing, SG enlargement, gingival enlargement, fungal infx (Candida, Mucor), dry mouth, erythema migrans

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79
Q

buffalo hump

A

Cushing

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80
Q

hypophosphatasia mechanism and mouth

A

defcy of alkaline phosphatase

premature loss of teeth 2/2 lacks cementum

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81
Q

defcy of alkaline phosphatase

A

hypophosphatasia

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82
Q

premature loss of teeth 2/2 lacks cementum

A

hypophosphatasia

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83
Q

vit D resistant rickets mutation

A

PHEX

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84
Q

PHEX

A

vit D resistant rickets

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85
Q

pyostomatitis vegetans manifestation of –

A

Crohn’s or UC

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86
Q

snailk track ulcerations

A

pyostomatitis vegetans manifestation of Crohn’s or UC

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87
Q

uremic stomatitis whats up

A

increased urea levels in chronic renal failure

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88
Q

increased urea levels in chronic renal failure

A

uremic stomatitis

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89
Q

face of mucopolysaccharidosis

A

cloudy cornea, heavy brow ridges, stiff joints, mental retardation

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90
Q

oral MPS

A

gingival hyperplasia, impacted teeth with pronounced follicle, macroglossia

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91
Q

examples of mucopolysaccharides (and aka?)

A

glycosaminoglycans

heparin, dermatan, keratan, chondroitin sulfate

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92
Q

examples and inheritance of MPSs

A

AR except Hunter – XLR

Hurler, Scheie, Hunter, Sanfillippo (A/B), Morquio (A/B), Maroteux-Lamy

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93
Q

lipid storage diseases better group name, examples, inheritance

A

AR, lipid reticuloendothelioses
lipids accumulate in macrophages
Gaucher, Niemann-Pick, Tay-Sachs

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94
Q

most common lipid storage disease

A

Gaucher

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95
Q

clinical types of gaucher

A

type 1 - non-neuronopathic, most in jewish

types 2 and 3 - neuronopathic

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96
Q

niemann pick with known mutation

A

NPC-1 type C

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97
Q

lipoid proteinosis inheritance and mutation

A

AR - ECM1

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98
Q

face/head/mouth of lipoid proteinosis

A

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

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99
Q

PAS quality in lipod proteinosis

A

PAS+

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100
Q

hemoglobin breakdown cascade

A

Hb–> Bb –> unconj in blood –> conj in liver –> excretion in bile

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101
Q

unconjugated bilirubin excess

A

Gilbert syndome

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102
Q

possible causes of excess Bb

A

hemolytic anemia, sickle cell anemia
liver malfunction
Gilbert syndome
reduced excretion in bile

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103
Q

ddx for jaundice

A

hypercarotenemia – skin is yellow but sclera is not

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104
Q

protein structure of amyloid

A

beta sheets

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105
Q

amyloidosis by involvement

A

organ limited and systemic

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106
Q

types of systemic amyloid

A

primary; myeloma-assoc; secondary (TB, sarcoid, osteomyelitis); hemodyalisi assoc; heredofamilial

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107
Q

amyloid death

A

2/2 kidney or heart failure

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108
Q

molecular types of amyloid

A

AL – organ limited, primary, myeloma assoc (Ig light chain)
AA - secondary (acute phase)
b2 microglobulin – hemodialysis

109
Q

scurvy underlying mechanisms

A

vit c reqd for post-translational proline hydroxylation of collagen chains –> weak vascular walls
bad healing
scorbutic gingivitis

110
Q

rachitic rosary

A

prominence of costochondral junctions

111
Q

prominence of costochondral junctions

A

rachitic rosary

112
Q

symptoms of osteomalacia

A

bone fragility, fractures, pain

113
Q

vit e defcy who’s at risk

A

children w chronic cholestatic liver disease and malabsorption
–> CNS issues

114
Q

risk for vit k defcy

A

malabsorption syndromes, abx, anticoagulants

115
Q

most common anemia in US

116
Q

most common form of anemia in world

117
Q

reasons for fe def anemia

A

blood loss (menorrhagia, GI dusease)
need for RBC (pregnancy)
low iron intake (children, elderly)
low Fe absoprtion (celiac)

118
Q

oral signs of fe def anemia

A

angular cheilitis and atrophic glossitis or generalized oral mucosa atrophy
painful tongue

119
Q

achlorhydria

A

absence of gastric acid –> fe def anemia

120
Q

howell jolly bodies

A

clusters of DNA in circulating RBCs – Fe def anemia

121
Q

plummer vinson aka

A

patterson kelly or sideropenic dysphagia

122
Q

components of plummer vinson

A

fe def anemia, glossitis, dysphagia, angular cheilitis, esophageal wevs (abnormal bands of tissue)

increased risk for oral and esophagela SCC

spoon nails

123
Q

spoon nails

A

koilonychia: plummer vinson

124
Q

pernicious anemia aka and whats up

A

megaloblastic enamia
vut b 12 deficiency (cobalamin)
autoimmune destruction of intrinsic factor (needed for b12 absoprtion)

125
Q

pernicious anemia also concurrent with

A

autoimmune gastritis

126
Q

oral pernicious anemia

A

burning mouth, erythema/atrophy of tongue (may present as focal patchy areas)

127
Q

compares absorption and excretion rates of vit b 12

A

schirling test

128
Q

pernicious anemia diagnosis

A

serum AB against intrinsic factor

129
Q

causes of pituitrary dwarfism

A

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
Q

pituitary dwarfism body morphology and oral

A

short stature but normal proportions

smaller mx/md; delayed eruption, smaller teeth

131
Q

gigantism timing

A

increased GH (usually from adenoma) before closure of epiphyseal plates

132
Q

gigantism association

A

20% cases with McCune-Albright

133
Q

gigantism head

A

enlarged sella bc adenoma

oral – generalized macrodontia

134
Q

enlarged sella in skull

A

pituitary adenoma – gigantism

135
Q

closure of epiphyseal plates interferences

A

growth hormone:
- before closure –> gigantism
after closure –> acromegaly

136
Q

common cause of acromegaly

A

increased GH (usually pituitary adenoma) after closure of epiphyseal plates

137
Q

other problems in acromegaly

A

HTN, CHD, hyperhidrosis, arthritis, peripheral neuropathy

bones of hands and feet grow –> gloves and hats become small

138
Q

orofacial acromegally

A

MD prognathism, anterior open bite, diastema, macroglossia, sleep apnea

coarse face

139
Q

two types of hypothyroidism

A

cretinism – in infancy (teeth fail to erupt)

myxedema – long standind –> spotigion of glycosaminoglycans

140
Q

pimrary vs secondary hypothyroidism

A

primary – most cases; problem in thyroid

secondary – pituitary not procuding TSH

141
Q

example of destruction of thyroid

142
Q

myxedema systemic and oral

A

decreased metabolism – bradycardia, hypothermia

facial, lip, tongue swelling

143
Q

labs in hypothyroid

A 
low T4
TSH elevated (primary) or normal (secondary)
144
Q

most common cause of hyporthyroid

A

90% - graves dz

auto AB to TSH receptors stimulate thyroid cells

145
Q

graves dz general presentation

A

warm moist skin, fine tremor, sensitivity to heat, diffuse thryoid enlargement, exophthalmos

146
Q

non graves causes of hyperthyroid

A

thyroid tumors (produce hormones) and pituitary adenoma (produces TSH)

147
Q

hyperthyroid labs

A

high T4, low TSH

148
Q

hyperthyroid treatment

A

radioactive iodine (risk of hypothyroid)

149
Q

thyroid storm

A

release of large amounts of T4 at one time – eg after infx, trauma, stress

delirium, convulsion, fever tachycardia

150
Q

PTH function

A

takes Ca from bone to blood, in conjunction with Vit D

151
Q

causes of hypoPTH

A

surgical removal of parathyroid or autoimmune

152
Q

2 syndromes with hypoPTH

A

diGeorge and APECED

153
Q

Ca levels in hypoPTH

A

hypoCaemia but asymptomatic; may lead to tetany tho

154
Q

chvostek sign for what and how

A

hypoCaemia

twitching of upper lip when facial nerve is tapped below zygoma

155
Q

twitching of upper lip when facial nerve is tapped below zygoma

A

chvostek sign

hypoCaemia

156
Q

test for hypoCaemia

A

chvostek sign

twitching of upper lip when facial nerve is tapped below zygoma

157
Q

teeth in hypoPTH

A

if young – enamel pitting and failure of teeth to eript

158
Q

persistent oral candidiasis in a young patient

159
Q

labs and tx for hypoPTH

A

low PTH and Ca

tx - vit D precursors

160
Q

pseudohypoPTH aka and what is

A

Albright hereditary osteodystrophy; acrodysostosis

normal levels of PTH but dysfx pathway – symptoms of hypoPTH

161
Q

types of pseudohypoPTH

A

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
Q

pseudohypoPTH when pt normal but hypoCaemia

A

Ib – PTH recepetors

and II - cAMP induced but cells don’t respond. pt normal but hypoCaemia

163
Q

pseudohypoPTH and obesity

A

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
Q

pseudohypoPTH when no PTH receptors

A

1b - PTH receptors, pt normal but w hypoCaemia

165
Q

Gsa defect and cAMP

A

cAMP deficient – pseudohypoPTH type Ia

cAMP induced – type Ic

166
Q

dental pseudohypoPTH

A

enamel hypoplasia, oligodontia, dagger-shaped pulp stones, blunt apices

167
Q

dagger-shaped pulp stones and blunt apices

A

pseudohypoPTH

168
Q

labs and tx for pseudohypoPTH

A

dx: elevated PTH but low Ca
tx: vit D and Ca

169
Q

primary vs secondary hyperPTH

A

primary – 90% gland adenoma

secondary – chronic low Ca from chronic renal dz; vit D not activated and Ca not absorbed

170
Q

function of PTH

A

mobilization of Ca from bone
increases renal tubular reabsorption of Ca
promotes renal prodiction of ,25 dihydroxyvitD
increases serum Ca

171
Q

hyperPTH syndromes

A

MEN1 and MEN2A and hyperPTH-jaw tumor syndrome

172
Q

hyperPTH triad

A

kidney stones (elevated Ca), bones (various), abdominal groans (duodenal ulcers)

173
Q

central degeneration and fibrosis of long standing brown tumor

A

osteitis fibrosa cystica – hyperPTH

174
Q

brown tumor

175
Q

renal osteodystrophy and PTH

A

secondary hyperPTH

striking jaw enlargement

176
Q

bones of hyperPTH

A

brown tumor, subperiosteial bone resopriton (esp phalanges), generalized osteopenia, loss of lamina dura, blurring of trabeculae (ground glass)

177
Q

hyperPTH relevant histo

A

CGCG –

trabeculare of woven bone + giant cells in fibrous background

178
Q

adrenal hormonal pathway

A

pituitary: ACTH –> adrenal: corticoids

179
Q

cushing syndrome vs cushing dz

A

exogenous corticouds – syndrome

endogenous (adrenal/pituitary adenoma) Cushing dz (rare, most in young F)

180
Q

cushing aka

A

hypercortisolism

181
Q

hypercortisolism signs

A

central weight gain, buffalo hump, moon facies, HTN< diabetes, osteoporosis

182
Q

dexamethasone test

A

normal pts – cortisol and ACTH decrease

tumors don’t respond to feedback loop

183
Q

addisonian crisis

A

acute episode of hypoadrenocorticism

after withdrawal of corticoids (drugs suppress pituitary – can’t produce ACTH)

184
Q

nevi, atrial muxoma, myxoid NF, ephelides, hypercortisolism

A

subest of Carney complex

185
Q

hypoadrenocorticism aka

A

addison’s dz

low corticosteroids 2/2 adrenal dysfx

186
Q

causes of addison

A

autoimmune, infections (TB, deep fungal (histo, PCM), mets, sarcoid, amyloid

187
Q

fungi that can destroy adrenals

A

histoplasma and paracoccidio

188
Q

secondary hypoadrenocorticism

A

low corticoids 2/2 low ACTH from pituitary dysfx

189
Q

how soon does hypoadrenocorticism manifest

A

only when 90% of gland destroyed

190
Q

skin in addison

A

bronzing

increased beta-lipotropin and ACTH stimulate melanocytes

191
Q

addison + hypoparatHY + mucocutaneous candidiasis

192
Q

oral addison

A 
diffuse pigmentation of oral mucosa
sudden onset (vs physiologic)
193
Q

addison labs

A 
low serum cortisol (<20 ug/dL)
high ACTH (primary) or low ACTH (secondary)
194
Q

sheehan syndrome

A

acute adrenal cortical insufficiency caused by meningococci

195
Q

acute adrenal cortical insufficiency caused by meningococci

A

waterhouse friedrichsen

196
Q

diabetes: type 1 vs type 2

A

type 1 - insulin dependent (lack insulin; AI destruction of pancreas islet cells)
type 2 - non insulin dependent – insulin resistance; genetic + environmental

197
Q

ketoacidosis

A

more common in DM type 1

no glucose –> fat and protein used for energy –> ketones as byproduct

198
Q

blood vessels and diabetes

A

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
Q

diabetic nephropathy

A

2/2 microangiopathy

aka kimmelstein-wilson dz

200
Q

kimmelstein-wilson dz

A

diabetic nephropathy

201
Q

oral DM

A

perio dz, delayed healing, sialadenosis, gingival enlargement, fungal infx (candida and mucor), xerostomia

202
Q

metformin action

A

increases glucose use, reduces insulin resistance

203
Q

insulin shock

A

type 1 pt:

if no carbs right after insulin injection, glucose levels may fall dramatically (below 40mg/dL)

204
Q

hypophosphatasia what’s the defect

A

deficiency of alkaline phosphatase

  • reduced alk phos in bone, liver, kidney
    increased blood and urinary phosphoethanolamine
  • bone abnormalities similar to rickets
205
Q

hypophosphatasia forms and features

A

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
Q

hypophosphatasia labs

A

clinical + low serum alk phos + high serum phosphoethanolamine

207
Q

vit d resistant rickets what is

A

clinical features of rickets but no response to vit D tx

208
Q

vit d resistant rickets genetics and inheritance

A

PHEX
X-linked dominant
F affected less severely 2/2 lyonization

209
Q

labs and looks of vit d resistant rickets

A

rachitic changes + hypophosphatemia + decreased reabs of P in kiney
short stature, bowed lower limbs

210
Q

teeth of vit d resistant rickets

A

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
Q

enlarged pulps, elongated pulp horns extend into incisal edge just below DEJ
lage calcospherites of globular dentin

A

vit d resistant rickets

212
Q

tx for vit d resistant rickets

A

calcitriol and phosphate

213
Q

vit d dependent rickets inheritance and defect

A

AR, lacks 1 alpha hydroxylase (activates vit D)

214
Q

vit d dependent rickets teeth

A

hypocalcification of teeth

215
Q

hypocalcification of teeth which rickets

A

vit d dependent

216
Q

elongated pulp horns extend into incisal edge just below DEJ which rickets

A

vit d resistant

217
Q

vit d dependent vs resistane rickets teeth

A

dependent – hypocalcification; resistant – elongated pulp horns extend into incisal edge just below DEJ

218
Q

crohn’s site of action

A

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
Q

skip lesions which affliction

220
Q

how common is oral crohn’s and what are the varieties

A

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
Q

pyostomatitis vegetans look and reason

A

inflammatory bowel dz - Crohn’s and ulcerative colitis

yellowish linear serpentine pustules over erythematous mucosa (snail track ulcerations)

222
Q

(snail track ulcerations)

A

pyostomatitis vegetans

223
Q

pyostomatitis vegetans locations

A

buccal/labial, soft palate, ventral tongueu

224
Q

histo pyostomatitis vegetans

A

acantholytic with intra or subepithelial eosinophilic abscesses

225
Q

acantholytic with intra or subepithelial eosinophilic abscesses

A

pyostomatitis vegetans

226
Q

uremic stomatitis reason

A

urease degrades urea in saliva and releases ammonia –> damages oral mucosa

227
Q

uremic stomatitis locations

A

painful white plaques, cheek, tongue, FOM

228
Q

uremic stomatitis ddx and how

A

oral hairy leukoplakia

ammonia/urine odor

229
Q

uremic stomatitis tx

A

clears with dialysis in up to 3 weeks

also helps to dilute H2O2 as rinse

230
Q

gaucher, niemann-pick, tay-sachs

what’s lacking
what accumulates
where

A

Gaucher: glucocerebrosidase; glucosylceramide in lysosomes of macrophages
NP: sphingomyelinase: sphingomeyline in lysosomes of macrophages
Tay-Sachs: beta-hexosaminidase - gandlioside in lysosomes of neurons

231
Q

neuronopathic niemann pick types

232
Q

visceral involvement niemann pick

A

type B - liver, spleen, lung

233
Q

molecular/cellular source of lipoid proteinosis

A

collagen 4, 5, laminin

from basement membrane

234
Q

lipoid proteinosis brain

A

medial temporal lobe calcifications in 70% –> seizures

235
Q

three stains of amyloid

A

1-congo red
2-Crystal violet
3-thioflavineT

236
Q

blindness vitamin

237
Q

beri beri vitamin

A

B1 (thiamin)

238
Q

pellagra vitamin

A

B3 (niacin)

239
Q

rachitic rosary

A

prominence of costachondral junction in Vit D resistant Rickets (looks like
beads / nodules)

240
Q

dagger pulp

A

pseudohypoPTH

241
Q

high PTH, low serum Ca, high serum P, normal renal dx

A

pseudohypoPTH

242
Q

common cause of hypoTHY

243
Q

labs in thyroid

A

primary hypo: low T4, high TSH
secondary hypo: low T4, normal TSH

hyperTHY: high T4, low TSH

244
Q

common cause of hyperTHY

A

graves (autoAB to TSH receptors –> make T4)

245
Q

low PTH, low serum Ca, , high P, Normal

Kidney

246
Q

chromosome digeorge and aka

A

22

velo-cardiofacial

247
Q

ground glass

A

fibrous dysplasia and hyperPTH

248
Q

synromes w primary hyperPTH

A

MEN1, MEN2A

249
Q

high PTH, high Ca

A

pimary hyperPTH

250
Q

high PTH, low Ca

A

secondary hyperPTH

251
Q

resorption of middle and index finger

252
Q

addison labs

A

Hyponatremia = low soium / crave salt
Low cortisol : 18μg
Primary: High ACTH ( over 100ng/L )

253
Q

salt craving

254
Q

 Hypoparathyroidism
 Hypoaldosteronism
 Candida

A

Polyendocrinopathy –candida-ectodermal

dystrophy syndrome:

255
Q

(20mg of
prednisone on a daily basis for several
months)

256
Q

cushing face, body

A 
Buffalo hump / moon face / weight gain
Red / purple abdominal striae
Hirsutism
Osteoporosis
Depression
Hyperglycemia with thirst and polyuria
Poor healing
257
Q

Suppress production of
ACTH in Pituitary gland and cannot respond to
stress due to exogenous corticosteroids

A

Addisonian crisis:

258
Q

biggest killer in diabetes

A

peripheral vascular disease

259
Q

insulin shock

A

below 40mg/dl

260
Q

sugar concentration at which kidney can’t absorb

A

over 300 mg.dL

261
Q

head and neck diabetes

A 
 Microangiopathy
 Diabetic sialadenosis
 Oral canida
 Mucormycosis
 Goegraphic tongue
 xerostomia
262
Q

types of hypophosphatasia

A
  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
Q

labs in hypophosphatasia

A

low alk phos, high serum and urine phosphotheanolamine

264
Q

rickets inheritance

A

vit d resistant – XLD

vit d dependent – AR

265
Q

PHEX

A

vit d resistant rickets

266
Q

1α-hydroxylase

A

vit d dependent rickets

267
Q

snail track

A

Pyostomatitis vegetans:

associated with crohn or ulcerative colitis

268
Q

uremic stomatitis ddx

A

oral hairy leukoplakia

OP - Yield (19 decks)

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