firecrack feb 1 Flashcards Preview

Internal Med > firecrack feb 1 > Flashcards

Flashcards in firecrack feb 1 Deck (297):
1

goal FiO2

less than 60% (need to minimze oxygen radicals)

2

tidal volume =

8-10ml/kg of ideal body wt

3

how to change patient's arterial pco2

by modifying TV or RR

4

high resp rates use to

blow off excess pco2
ex severe metabolic acidosis

5

low resp rates use to

increase pco2
resp alkalosis

6

how to change pts arterial po2

modify fio2 or peep

7

most common complication of mechanical ventilation

barotrauma
risk increase with PEEP

8

PEEP

alveolar pressure above atomspheric pressure found in lung at end of expiration
keeps alveoli from collpasing on expiration

9

complications of peep

increased intracranial pressure
inrecased risk of barotrauma
hypotension from diminished venous return

10

classic presentation of pheo

htn, tachycardia
diaphoresis, headaches, palpitations

11

long standing undiganosed pheo causes

catecholamine cardiomyopathy

12

radiology for pheo

CT scans - adrenal adenoma
MIBG scans, pheo not on adrenal gland

13

pheo crisis

adrenergic hypertensive crisis leads to multiple system organ faliure

14

conditions that can induce pheo crisis

anesthesia induction agents
emotional stress
iv urographic contrast
drugs

15

pheo treatment - first choice

surgical resection

16

pheo medical management

alpha blocker: phenoxybenzamine

17

CML mutation

constiutively active tyrosine kinase
t9:22
bcr-ab1

18

median age of onset for CML

50

19

CML presents with

nonspecific - fatigue, fever, wt loss
early satiety (enlarged spleen)
LUQ pain - spleen infarction or spleenomegaly

20

CML & LAP

low leukocyte alkaline phosphatase

21

CML DOC

tyrosine kinase inhibitors - imatinib

22

CML with blast crisis

need hematopoietic stem cell transplant

23

CML can conver to

ALL or AML in a blast crisis
myeloid/lymphoid blasts proliferate
usually fatal

24

How is genetic sideroblastic anemia treated?

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