firecrack feb 1 Flashcards
(297 cards)
1
Q
goal FiO2
A
less than 60% (need to minimze oxygen radicals)
2
Q
tidal volume =
A
8-10ml/kg of ideal body wt
3
Q
how to change patient’s arterial pco2
A
by modifying TV or RR
4
Q
high resp rates use to
A
blow off excess pco2
ex severe metabolic acidosis
5
Q
low resp rates use to
A
increase pco2
resp alkalosis
6
Q
how to change pts arterial po2
A
modify fio2 or peep
7
Q
most common complication of mechanical ventilation
A
barotrauma
risk increase with PEEP
8
Q
PEEP
A
alveolar pressure above atomspheric pressure found in lung at end of expiration
keeps alveoli from collpasing on expiration
9
Q
complications of peep
A
increased intracranial pressure
inrecased risk of barotrauma
hypotension from diminished venous return
10
Q
classic presentation of pheo
A
htn, tachycardia
diaphoresis, headaches, palpitations
11
Q
long standing undiganosed pheo causes
A
catecholamine cardiomyopathy
12
Q
radiology for pheo
A
CT scans - adrenal adenoma
MIBG scans, pheo not on adrenal gland
13
Q
pheo crisis
A
adrenergic hypertensive crisis leads to multiple system organ faliure
14
Q
conditions that can induce pheo crisis
A
anesthesia induction agents
emotional stress
iv urographic contrast
drugs
15
Q
pheo treatment - first choice
A
surgical resection
16
Q
pheo medical management
A
alpha blocker: phenoxybenzamine
17
Q
CML mutation
A
constiutively active tyrosine kinase
t9:22
bcr-ab1
18
Q
median age of onset for CML
A
50
19
Q
CML presents with
A
nonspecific - fatigue, fever, wt loss
early satiety (enlarged spleen)
LUQ pain - spleen infarction or spleenomegaly
20
Q
CML & LAP
A
low leukocyte alkaline phosphatase
21
Q
CML DOC
A
tyrosine kinase inhibitors - imatinib
22
Q
CML with blast crisis
A
need hematopoietic stem cell transplant
23
Q
CML can conver to
A
ALL or AML in a blast crisis
myeloid/lymphoid blasts proliferate
usually fatal
24
Q
How is genetic sideroblastic anemia treated?
A
iron levels managed by transfusions, chelation, phlebotomy
25
What metabolic defect causes sideroblastic anemia?
defective heme synthesis
iron can't combine with heme
unused iron accumulates in mitochondria and around nucleus
26
hat is a potential long-term complication of acquired, irreversible sideroblastic anemia?
myelodysplastic syndrome
| acute leukemia
27
What vitamin can treat sideroblastic anemia?
vit b6 (pyridoxine)
28
What stain is used to diagnose sideroblastic anemia?
Prussian Blue of bone marrow
| basophilic inclusions
29
what is a ringed sideroblast?
unused iron encircling nucleus
30
How is molluscum contagiosum diagnosed?
clinical
31
What histological findings are associated with molluscum contagiosum?
giemsa or wright stains - inclusion bodies
32
What treatment options are available for patients with molluscum contagiosum?
cryotherapy
curettage
cantharidin
topical trichloroacetic acid
33
What are five reversible causes of sideroblastic anemia?
```
lead, zinc, alcohol tox
copper def
isonizaid
chloramphenicol
hypothermia
```
34
diagnosis of CMV infection
urinarylsis
culture buffy coat WBC
PCR or serology
35
What effect can CMV infection have on the adrenal glands?
addison's dz (primary adrenal insuff)
36
What is the classic description of microscopy findings in CMV infection?
large intranuclear basophilic inclusion surrounded by a halo and small intracytoplasmic basophilic inclusion
37
What are the most significant adverse effects of ganciclovir?
hematologic - neutropenia, etc
38
What are the signs/symptoms of congenital CMV?
```
microcephaly
MR
Deafness
Intracranial Califications
Seizures
(MRDICS)
```
39
What s/sx of CMV may be present at birth, but resolve within the first few weeks of life?
thrombocytopenia purpura - blueberry muffin rash (also in rubella)
HSM, jaundice
40
What medication is used to treat ganciclovir-resistant CMV?
foscarnet
41
Why is congenital CMV important?
- #1 congenital infection in the developed world
- #1 viral cause of mental retardation in the U.S.
- #1 cause of sensorineural hearing loss
42
How does CMV infection manifest in immunocompetent vs immunocompromised hosts?
asymptomatic, mono vs
| GI,pulm,renal,adrenal dz
43
What are the GI sequelae of CMV infection?
esophagitis (AIDs, painful swallowing)
hepatitis (granulmatous0
colitis
44
What infection does CMV cause in the lung?
interstitial/atypical pneumonia
45
How does CMV affect the kidneys?
progressive renal failure
| will see intranuclear inclusions
46
Why does presence of HbS result in vaso-occlusive phenomena?
HbS - poorly soluble when deoxygenated
| polymerize within RBCs, impaired RBC deformability - vaso occlusions
47
Sickle Cell Disease Lab Findings
elevated reticulocyte index 3-15%
indirect hyperbilirubenimia
elevated LDH, decrease haptoglobin
sickle cells, polychormasis, howell-jolly bodies
48
Why do children with sickle cell disease experience delayed growth and development?
primary hypogonadism, hypopituitarism, hypothalamic insuff
49
sickle cell bacteremia
s pneumo
50
sickle cell meningitis
s pneumo
51
sickle cell pneumonia
mycoplasma, chlamydia, legionella
52
What screening test regimens should sickle cell patients begin?
Transcranial Doppler ultrasound for evaluation of cerebral blood flow, starting at age 2 and repeated every 1-2 years until age 16
Retinal evaluation should start at age 10 and repeated routinely
53
How is sickle cell disease diagnosed in the prenatal period? How is it diagnosed after birth?
prenatal: dna pcr testing
newborn: electrophoresis (HbF, HbS but no HbA)
54
What antimicrobial prophylaxis is recommended for sickle cell patients?
vaccianations: s pneumo/h flu/mengitis
hep b
3 months - 5 years: penicillin or erythromycin
55
What medication & supplements can be provided for patients with symptomatic sickle cell disease?
hydroxyurea
| folic acid supplementation
56
What are 6 common neurological complications of sickle cell disease?
```
TIA, ischemic stroke
intracerebral hemorrhage
vestibular dysfxn
sensory hearing loss
retinopathy
```
57
What are 3 common skeletal complications of sickle cell disease?
osteonecrosis
pancytopenia
osteoporosis
58
What cardiac complication are sickle cell patients at risk for?
increased cardiac output to compensate for anemia
| MI
59
What type of gallstones are typically seen in patients with sickle cell disease?
pigmented
60
acute chest syndrome
appearance of new infiltrate on CXR along with pulm symptoms
| can be due to infarction or infection
61
What are the most common causes of malignant solitary pulmonary nodules?
primary lung cancer
metastasis
carcinoid tumor
62
Following an episode of pharyngitis, a 7-year-old child develops periorbital edema and hematuria. Explain the pathophysiology of this patient’s condition.
type III hypersensitivity
63
Infections of what organ systems can result in PSGN (post streptococcal glomerulonephritis)?
skin or URI
64
What is the confirmatory test for PSGN?
kidney biopsy
| igg and c3 positive bumpy deposits on renal basement membrane
65
What are symptoms of PSGN (post streptococcal glomerulonephritis) aside from the classic triad?
brown urine, renal failure
66
What lab findings are common in PSGN (post streptococcal glomerulonephritis) other than hematuria and proteinuria
aso titer
increase bun,cr
postivie anti-dnase b titer
67
What is the classical clinical presentation of post-strep glomerulonephritis?
edema, esp periorbital
hematuria
hypertension
68
What levels of hematuria and proteinuria are found in PSGN (post streptococcal glomerulonephritis)?
4+ blood on urine dipstick
| <3.5g protein/day
69
treatment for psgn
self limited
| only for complications (diuretics, acei)
70
What is the most common anatomic location for a carcinoid tumor?
appendix
bronchopulm tree
ileum
rectum
71
In addition to octreotide, what therapies are indicated for the treatment of metastatic carcinoid disease?
IFN-alpha
| chemoembolization of hepatic artery
72
What is a carcinoid tumor?
neuroendocrine origin
| derived from primitive stem cells in gut wall
73
What are some potential complications of a carcinoid tumor?
carcinoid syndrome and carcinoid crisis
bowel obstruction
carcinoid heart disease
metastasis, recurrence
74
Describe the constellation of symptoms seen in a patient with carcinoid syndrome.
flushing
diarrhea
bronchoonstriction
tricuspid/pulm valvular dz
75
What is the best screening method for carcinoid tumor?
urine 5 HIAA
| serum serotonin
76
What parameters are associated with a poor prognosis in a patient with a carcinoid tumor?
mets
tumors greater than 2 cm in size
positive lymph nodes
77
What substance secreted by carcinoid tumors is responsible for the symptoms seen in carcinoid syndrome?
serotonin
78
Carcinoid tumors are most likely to be found in what patient population?
adults
| rare in kids
79
Under what conditions can a carcinoid tumor cause carcinoid syndrome?
GI tumor metastasizes to liver
or
primary tumor outside of GI system
80
In a patient with carcinoid syndrome, what abnormalities may be present on cardiopulmonary exam?
wheezing
| rs heart murmur
81
What is the standard treatment for a localized carcinoid tumor?
resection
82
What pharmacologic agent may be administered for symptomatic relief in a patient with carcinoid syndrome?
octreotide
83
kidney biopsy for iga nephropathy
increased prolif of mesangial cells
84
iga nephropathy classic presentation
hematuria
flank pain
low grade fever
85
What is the basic pathophysiology of IgA nephropathy?
igA immune complexes deposit in mesangial cells of kidneys, cause damage to glomeruli
86
diseases iga nephropathy is associated with
HSP
cirrhosis
celiac disease
inflamm disorders - sarcoidosis, IBD
87
What is the most common cause of glomerular hematuria world-wide?
iga nephropathy
88
What will the urine dipstick show for IgA nephropathy?
pos for blood and protein
| RBC and RBC casts can be present
89
What is seen on light microscopy in IgA nephropathy?
normal-appearing glomeruli or mesangial widening due to mesangial proliferation
90
What are the medical treatment options for IgA nephropathy?
acei/arb with statins
91
When are steroids indicated for treating IgA nephropathy?
nephrotic range proteinuria
92
What are some features that distinguish IgA nephropathy from Post-Streptococcal Glomerulonephritis?
iga within one week of URI, psgn several weeks latera
normal complement in iga nephropathy
positive throat culture in PSGN
93
cell that accumulates in CLL
functionally incompetent, but mature appearing lymphocytes
94
Rai staging system
stage 0 - lymphocytosis
I, II, LAD, organomegaly
III, IV: anemia, thrombocytopenia
95
median survival for CLL pts
10 years
| very variable
96
scabies
mite sarcoptes scabiei
| pruritic lesions
97
populations affected by CLL
caucasions, male, elderly (70)
98
next step in treatment of localized CLL
radiation
99
two staging systems for CLL
Rai and Binet
100
most common PE findings for CLL
LAD, HSM
101
CLL complications
infection, anemia, thrombocytopenia
| tumor lysis syndrome
102
Binet staging system
a: fewer than 3 LN
b: 3 or more LN
c: anemia or thrombocytopenia
103
CLL pts that should receive chemo
advanced, symptomatic
104
scabies presents with
itching
| worse at night and after a hot bath
105
presenting symptoms of CLL
asympatomtic
| b symptoms
106
leukemia cutis
plum-colored purpur
| manifestaton of leukemia
107
scabies treatment
topical permethrin cream or oral ivermectin
108
graves dz highest risk
women, age 20-40
109
RA spares
DIP joints
110
Graves disease cellular infiltrate
lymphocytic infiltrate, germinal centers
111
RA first line DMARDS
MTX
sulfasalazine
hydroxychloroquine
leflunomide
112
NSAIDS/glucticorticoids in RA
adjuntive antiinflamm agents
113
RA clinical diagnosis, next step
x ray imaging:
joint erosion
joint space narrowing
subluxation
114
drugs for prinzmetals
dihydropyridine CCB
| amlodipine, nifedipine
115
joint space aspiration in RA
decreased complement
| increased leukocyte count
116
RA lab findings
increase ESR, CRP
+ RF
+ ANA
Anti=CCP
117
prinzmetal complication that occurs with coronary relaxation
severe life threatening arrhythmias (v fib, vtach, etc)
118
prinzmetal contraindicated drug
aspirin
119
RA - systemic manifestations
```
pericarditis
scleritis
felty's syndrome
still's disease
sjogren's syndrome
```
120
management of hyperthyroidism during pregnancy
PTU in first trimester
methimazole after that
meth - teratogenic, PTU - liver injury
121
gold standard test for prinzmetal's angina
coronary angiography with provocative test for vasospasm (acetylcholine infusion directly into the vessel)
122
RA - classic hand deformitites
ulnar deviation
swan-neck deform, boutonnier deform
z-thumb
mcp joint hypertrophy
123
graves dz antibodies
TSH receptor
| can have anti-TPO and anti-thyroglobulin
124
RA - joints affected first
hands and feet
125
rheumatoid factor is..
IgM against the constant region of patient's own IgG antibodies
126
diverticulitis complications
obstruction
perforation
abscess formation
127
diverticulitis symptoms
LLQ or diffuse abd pain
fever
leukocytosis
128
diverticulitis with abscess greater than 4cm
CT guided percutaneous drainage
| fecal materal or perforation --> surgery
129
diverticulitis imaging
CT with oral and IV contrast
130
treatment of diverticulitis
NPO
BS atb
IV fluids
pain control
131
exposure to lead in children, adults
lead paint in kids
| occupational in adults
132
bone lead level indicative of
total lifetime lead exposure
133
lead poisoning treatment
dimercaprol, EDTA, succimer
134
use of x-ray in children with lead poisoning
looking for foreign body containing lead
| dense metaphyses
135
hemochromatosis - HLA ?
HLA-A3
136
hemochromatosis when i s surgery indicated
```
end stage liver dz (transplant)
severe arthropathy (arthroplasty)
```
137
presentation of acute lead poisoning
abdominal and neurological symptoms
138
secondary hemochromatosis
ineffective erythropoiesis (thalassemia, sideroblastic anemia)
multiple transfusions
chronic liver disease
139
lead toxicity pts peripheral smear
basophilic stippling
| microcytic, sideroblastic, hypochromic anemia
140
triad of adult onset hemochromatosis
micronodular cirrhosis
diabetes mellitus
bronze skin pigmentation
141
hemochromatosis other symptoms
arthropathy/chondrocalcinosis
dilated cm
amenorrhea, impotence, hypogonadism
142
lead toxicity effects on fetus
miscarriages, stillbirths, low birth wt
143
HFE
chromosome 6
| AR
144
liver biopsy in hemochromatosis
high iron content seen with prussian blue staining
145
most accurate test for lead toxicity
blood lead level
146
hemochromatosis treatment
phlebotomy
| deferoxamine
147
chronic lead exposure symptoms
nonspecific GI
behavorial
peripheral neuropathies
148
lead-induced peripheral neuropathy
wrist or foot drop in adults
| hand-eye coordination in children
149
hemochromatosis labs
serum iron increased
transferrin sat increase
increased ferritin
decreased TIBC
150
screening text for hemochromatosis
transferrin sat
151
lead poisoning anemia - age group
children
| under 6, BBB not fully formed
152
areas of intrinsic weakness inside colon --
vasa recta enters muscularis externa
153
treatment of trichinella
self limiting
| severe -bendazoles
154
how do humans acuqire trichinellosis
undercooked infected meat
155
antibiotic for cellulitis
clindamycin, linezolid
156
acne vulgaris is inflamm of
hair follicules, sebaceous glands
157
common preseentation of trichinellosis
muscle pain, tenderness, swelling, weakness
158
cutaneous structures affected by cellulitis
deeper dermis
| subc fat
159
acne complications
permanent scarring
| vit a - birth defects, hepatotoxicity
160
where does trichinella encyst
brain
heart
skeletal muscle
161
antibiotic for cellulitis, febrile pts
IV ceftriaxone or cefazolin
162
trichinella diagnosis
clinical, confirm with serology
163
severe acne treatment
oral isotretinoin
164
trichinella neuro symptom
ha worse with movement
165
erysipelas affects what cutaneous structures
upper dermis
| superficial lymphatics
166
trichenella - cardiac
eosinophilic myocarditis
167
erysipelas specific symptoms
acute onset
| systemic - fever and chills
168
cellulitis with systemic toxicity, suspect
necrotizing fasciitis
toxic shock syndrome
gas gangrene
169
AML - pt population
elderly (65), female
170
AML complications
anemia infection, bleeding
leukostasis, metabolic abn, CNS involvement
neutropenic enterocolitis
DIC
171
pathophysiology of AML
failure of bone marrow stem cells to mature
suppression of all cell lines
rapid prolif of immature myeloid cells
172
cholangiocarcinoma age group
elderly
173
AML presenting symptoms
pancytopenia
| infections, fatigue, pallor, bone pain, bleeding
174
AML bone marrow biopsy
greater than 20% blasts
175
symptoms of cholangiocarcinoma
jaundice, pruritis, wt loss
176
cholangiocarcinoma =
cancer of bile ducts
| adenocarcinoma
177
AML lab tests
myeloperoxidase
auer rods
myeloid markers
178
risk factor for cholangiocarcinoma
PSC
179
auer rods - what subtype
M1, M3
180
cholangiocarcinoma most common location
proximal common bile duct
181
aeur rods =
AML
myeloblasts
peroxidase positive
182
AML treatment
chemotherapy
183
treatmetn for M3 subtype of AML or APL
all trans retinoic acid (vit A)
184
AML supportive therapy
platelet, RBC transfusions
antibiotics
uric acid lowering agents
185
AML genetics
t 15 17 translocation
186
ITP associated with what conditions
HIV
Hep C
SLE
CLL
187
ITP =
acquire thrombocytopenia mediated by antibodies
188
ITP diagnosed by
exclusion
189
ITP: when to transfuse platelets
severe bleeding (GI or intracranial)
190
ITP treatment guided by
platelet counts
| presence of bleeding
191
mild ITP vs severe ITP
mild - clinically silent/mild drop in platelets
| severe - bleeding
192
ITP First line treatment
corticosteroids and/or IVIG
193
ITP level above to not treat
30,000
194
ITP second line treatment
splenectomy
| rituximab
195
polymyalgia rheumatica common in what populations
elderly
women
african americans
196
what is polymyalgia rheumatica
autoimmune dz with several sites of muscle and joint pain
| associated with temporal arteritis
197
5 PE findings in polymyalgia rheumatica
- fatigue
- edema of extremities
- tenderness to palpitation
- movement limited by pain
- normal muscle strength
198
polymyalgia rheumatica MRI
increased signal at tendon sheaths and tissue outside of joints
199
polymyalgia rheumatica lab findings
decreased hct
| increased ESR
200
clinical presentation of polymyalgia rheumatica
morning muscle stiffness
| pain in neck, shoulders, hips
201
treatment for polymyalgia rheumatica
low dose corticosteroids
202
alport's underlying defect
type IV collagen
203
alport's triad
hematuria
sensorineural hearing loss
ocural abnormalities
204
alports urinalysis
hematuria, proteinuria,
RBC casts
pyuria
205
alports treatment
ACEI/ARBs
206
alports electron microscopy
basketweave apperance
| thickeninning/thinning of GBM
207
alports mode of inheritance
x-linked
208
who is most commonly affected by polymyositis and dermatomyositis?
poly - adults
derm - children
women
209
Raynaud + myositisi
1/3 will develop it
210
myosititis immune cells
poly - CD8 T cells
| derm - B and T cells
211
how do polymyositis and dermatomyositis present?
symmetrical proximal limb and neck weakness over 3 to 6 months
212
life threatening complications associated with myositis
resp failure
myoglobinuric renal failure
cardiomyopathy
213
myositis - HLA subtypes
BA
DR3
DRW52
214
non-pharmacologic treatment may be required if there is severe inflammation associated with polymyositis and dermatomyositis?
bed rest with PT to prevent contractures
215
What are the first line treatments for polymyositis and dermatomyositis?
oral glucocorticoids
| topical steroids for derm
216
What is the age distribution of patients commonly affected by polymyositis and dermatomyositis?
bimodal
10-15
45-60
217
What is the second line therapy for polymyositis and dermatomyositis?
MTX,aza, other immunosuppressants
218
In addition to symmetrical proximal limb weakness, what symptoms are associated with polymyositis and dermatomyositis?
myalgia
dysphagia/arthralgia
raynaud
219
What limitations associated with polymyositis and dermatomyositis may cause patients to first visit a physicians office?
trouble combing hair or climbing stairs
220
How are polymyositis and dermatomysitis diagnosed?
- symmetric proximal muscle weakness
- elevated skeletal muscle enzymes
- myopathic changes on EMG
- inflamm on muscle biopsy
221
Gottron sign
lacy purple rash around MCP or PIP joints
222
pathophysiology of polycythemia vera
JAK 2 mutation
| stimulation of EPO receptor
223
polycythemia vera imaging
look for tumor that produce EPO (pheo, RCC, hemangioblastoma, HCC)
224
what is polycythemia vera
myeloproliferative disorder
increased RBC volume
hyperviscosity syndrome
225
What are some complications of polycythemia vera NOT related to hyperviscosity syndrome?
post-PV myelofibrosis
| AML
226
What lab findings are required to make the diagnosis of polycythemia vera?
elevated hgb, hct
| jak2 mutation
227
What are some complications of polycythemia vera related to hyperviscosity syndrome?
MI
ischemic stroke
budd-chiarir syndrome
228
What is the life expectancy of treated & untreated polycythemia vera patients?
10 or more years vs 6-18 months
229
What are the 5 aspects of medical treatment of polycythemia vera?
```
phlebotomy
myelosuppresive agents - hydroxyurea
aspirin
antihistaminies
uric acid lowering agents
```
230
What are the 5 chief presenting symptoms of polycythemia vera?
```
aquagenic pururitis
burning pain in hands & feet
fatigue
easy bleeding and bruising
headache
```
231
most common causes of death in polycythemia vera?
thrombosis
hematologic malginancies
hemorrhage
post pv-myelofibrosis
232
polycytehmia vera oxygen sat
>92%
| otherwise hypoxia-induced erythrocytosis
233
EPO in polycythemia vera
should eb low (negative feedback)
234
polycythemia vera bone marrow biopsy
generalized hypercellularity
235
polycythemia vera PE
HSM
236
TdT & CD10
ALL
237
ALL symptoms
nonspecific
238
ALL treatment
chemotherapy
| prophylatic intrathecal chemo with MTX
239
T cell ALL symptoms
SOB due to mediastinal mass
240
effect of ALL on other cell lines
decreases other cell lines
| thrombocytopenia, anemia, neutropenia
241
ALL peripheral smear
lymphoblasts
242
ALL prognosis
cured in children
| worse in adults
243
ALL cell of origin
immature lymphoblasts
| B cells > T cells
244
ALL population
children age 2-5
| boys, caucasians
245
aplastic anemia - 2 broad mechanisms
injury to pluripotent stem cells
| hereditary
246
aplastic anemia =
diminshed or abscent hematopoietic precursors in bone marrow
247
aplastic anemia drugs/toxins
radiation
anti-epilpetics
nsaids
sulfonamides
248
aplastic anemia symptoms
anemia -- fatigue
leukopenia - recurrent infections
thrombocytopneia - mucosal hemorrhage
249
aplastic anemia caused by what viruses
parvovirus b19
hepatitis
HIV
250
aplastic anemia hereditary cause
fanconi anemia (impaired dna repair mechanisms)
251
aplastic anemia bone marrow biopsy
hypocellulairty
no fibrotic infiltration or malignant cells
morphologically normal
252
aplastic anemia treatment
removal of offending agent
supportive care
transplant or immunosuppressive thearpy
253
hodgkin lymphoma cell type
B cell
254
hodgkin lymphoma histo findings
Reed-Sternberg cells in inflamm background
255
4 types of hodgkin lymphoma
lymphocytic predom
nodular sclerosis
mixed cellularity
lymphocyte depleted
256
hodgkin lymphoma pt risk factors
age 20 & 65
EBV virus
family hx
257
stages I-IV of hodgkin lymphoma
1 - single LN or site
2 - 2 or more LN, site on same side of diaphargm
3 - both sides of diaphragm
4 - outside of LN
258
Reed-sternberg cells
CD 15 and CD 30
259
presenting symptoms of hodgkin lymphoma
cervical LAD
| nonspecific b symptoms
260
early vs late hodgkin lymphoma
early - cure (chemo + rad)
| late - prognosis by IPS (chemo only)
261
hodgkin lymphoma treatment
early - chemo and rad
| late - chemo only
262
cause of vit D def
```
poor intake
increased loss
impared 25-hydrox
impaired 1-alpha hydroxy
target organ resistance
```
263
treatment of osteomalacia due to poor diet
50,000 IU weekly for 3-12 weeks
| 800IU daily
264
osteomalacia and rickets
impaired calcification of newly formed bone matrix
265
treatment of osteomalacia due to increased metabolism or impaired hydroxylation
50,000 IU weekly
266
impaired 25-hydroxylation due to
liver disease
| isoniazid
267
treatment for osteomalacia due to imapired 1alpha hydroxylation
give active form (1,25)oh2 vit d
268
What causes of vitamin D deficiency are associated with impaired 1alpha-hydroxylation?
renal osteodystrophy
| hypoparathyroidism
269
What changes to bone structure occur in rickets?
changes in growth plate (hypertrophic cartilage) and bone bowing/bone cortical thinning
270
What is meant when osteomalacia and rickets are called a “qualitative” defect?
process of bone mineralization is abnormal
271
How much calcium should patients with vitamin D deficiency supplement per day?
1.5-2g
272
How is iatrogenic osteomalacia managed?
withhold offending med
273
What causes of vitamin D deficiency are associated with increased loss of vitamin D?
increased metabolism
imapired enterohepatic circulation
nephrotic syndrome
274
rickets lab values
decreased 25 vit d
decreased ca, phosphate
increased alk phos, PTH
275
rickets x ray findings
```
Widening of physes
Bowing of long bones
Translucent lines in bones
Flattening of the skull
Enlarged costal cartilages
```
276
What results from the hypocalcemic state seen in osteomalacia and rickets?
hypocalcemia tetany
277
What causes of vitamin D deficiency are associated target organ resistance?
vit d receptor mutation
| phenytoin
278
internal organs affected by scleroderma
GI tract
lungs
MSK
279
organ system most often involved with systemic sclerosis
skin
| thick, tight, shiny skin
280
populations affected by scleroderma
women
| black
281
pulmonary complications in systemic sclerosis
interstitial fibrosis
282
how to treat esophageal conditions of scleroderma
PPI, H2
| strictures may need surgical correction
283
what causes the GI symptoms of scleroderma
decreased motlitly
| overgrwoth of bacteria and malabsorption
284
scleroderma age range
20-50
285
CREST
anti-centromere Ab, skin calcification, raynaud phenomenon, esophageal dysmotility, sclerodactly, telangiectasia
286
esophageal conditions associated with scleroderma
esophageal reflux
| esophageal candida
287
scleroderma - treatment of joint contractures
physical therapy
288
what three processes contribute to scleroderma pathogenesis
overproduction of ECM components
microvascular dysfxn
dysfxn cell/humoarl immunity
289
other diseases associated with scleroderma
PBC
malignacies
spontaneaous PTX
290
scleroderma & the heart
myocardial fibrosis --> CHF, arrhythmias
291
systemic sclerosis antibodiy
anti-topoisomerase-1 (anti-scl-70)
292
scleroderma GI symptoms
diarrhea, constipation, esophageal disease
293
GI symptoms treatment in scleroderma
promotility agents
| metronidazole
294
treatment for scleroderma renal crisis
ACEI
295
treatment for sclerotic vessels in scleroderma
vasodilators
296
scleroderma renal crisis
sudden onset of hypertension and renal insuff
297
treatment of rapidly progressive scleroderma
cyclophophamide, steroids
