firecrack feb 1 Flashcards
goal FiO2
less than 60% (need to minimze oxygen radicals)
tidal volume =
8-10ml/kg of ideal body wt
how to change patient’s arterial pco2
by modifying TV or RR
high resp rates use to
blow off excess pco2
ex severe metabolic acidosis
low resp rates use to
increase pco2
resp alkalosis
how to change pts arterial po2
modify fio2 or peep
most common complication of mechanical ventilation
barotrauma
risk increase with PEEP
PEEP
alveolar pressure above atomspheric pressure found in lung at end of expiration
keeps alveoli from collpasing on expiration
complications of peep
increased intracranial pressure
inrecased risk of barotrauma
hypotension from diminished venous return
classic presentation of pheo
htn, tachycardia
diaphoresis, headaches, palpitations
long standing undiganosed pheo causes
catecholamine cardiomyopathy
radiology for pheo
CT scans - adrenal adenoma
MIBG scans, pheo not on adrenal gland
pheo crisis
adrenergic hypertensive crisis leads to multiple system organ faliure
conditions that can induce pheo crisis
anesthesia induction agents
emotional stress
iv urographic contrast
drugs
pheo treatment - first choice
surgical resection
pheo medical management
alpha blocker: phenoxybenzamine
CML mutation
constiutively active tyrosine kinase
t9:22
bcr-ab1
median age of onset for CML
50
CML presents with
nonspecific - fatigue, fever, wt loss
early satiety (enlarged spleen)
LUQ pain - spleen infarction or spleenomegaly
CML & LAP
low leukocyte alkaline phosphatase
CML DOC
tyrosine kinase inhibitors - imatinib
CML with blast crisis
need hematopoietic stem cell transplant
CML can conver to
ALL or AML in a blast crisis
myeloid/lymphoid blasts proliferate
usually fatal
How is genetic sideroblastic anemia treated?
iron levels managed by transfusions, chelation, phlebotomy
What metabolic defect causes sideroblastic anemia?
defective heme synthesis
iron can’t combine with heme
unused iron accumulates in mitochondria and around nucleus
hat is a potential long-term complication of acquired, irreversible sideroblastic anemia?
myelodysplastic syndrome
acute leukemia
What vitamin can treat sideroblastic anemia?
vit b6 (pyridoxine)
What stain is used to diagnose sideroblastic anemia?
Prussian Blue of bone marrow
basophilic inclusions
what is a ringed sideroblast?
unused iron encircling nucleus
How is molluscum contagiosum diagnosed?
clinical
What histological findings are associated with molluscum contagiosum?
giemsa or wright stains - inclusion bodies
What treatment options are available for patients with molluscum contagiosum?
cryotherapy
curettage
cantharidin
topical trichloroacetic acid
What are five reversible causes of sideroblastic anemia?
lead, zinc, alcohol tox copper def isonizaid chloramphenicol hypothermia
diagnosis of CMV infection
urinarylsis
culture buffy coat WBC
PCR or serology
What effect can CMV infection have on the adrenal glands?
addison’s dz (primary adrenal insuff)
What is the classic description of microscopy findings in CMV infection?
large intranuclear basophilic inclusion surrounded by a halo and small intracytoplasmic basophilic inclusion
What are the most significant adverse effects of ganciclovir?
hematologic - neutropenia, etc
What are the signs/symptoms of congenital CMV?
microcephaly MR Deafness Intracranial Califications Seizures (MRDICS)
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
What medication is used to treat ganciclovir-resistant CMV?
foscarnet
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
How does CMV infection manifest in immunocompetent vs immunocompromised hosts?
asymptomatic, mono vs
GI,pulm,renal,adrenal dz
What are the GI sequelae of CMV infection?
esophagitis (AIDs, painful swallowing)
hepatitis (granulmatous0
colitis
What infection does CMV cause in the lung?
interstitial/atypical pneumonia
How does CMV affect the kidneys?
progressive renal failure
will see intranuclear inclusions
Why does presence of HbS result in vaso-occlusive phenomena?
HbS - poorly soluble when deoxygenated
polymerize within RBCs, impaired RBC deformability - vaso occlusions
Sickle Cell Disease Lab Findings
elevated reticulocyte index 3-15%
indirect hyperbilirubenimia
elevated LDH, decrease haptoglobin
sickle cells, polychormasis, howell-jolly bodies
Why do children with sickle cell disease experience delayed growth and development?
primary hypogonadism, hypopituitarism, hypothalamic insuff
sickle cell bacteremia
s pneumo
sickle cell meningitis
s pneumo
sickle cell pneumonia
mycoplasma, chlamydia, legionella
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
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)
What antimicrobial prophylaxis is recommended for sickle cell patients?
vaccianations: s pneumo/h flu/mengitis
hep b
3 months - 5 years: penicillin or erythromycin
What medication & supplements can be provided for patients with symptomatic sickle cell disease?
hydroxyurea
folic acid supplementation
What are 6 common neurological complications of sickle cell disease?
TIA, ischemic stroke intracerebral hemorrhage vestibular dysfxn sensory hearing loss retinopathy
What are 3 common skeletal complications of sickle cell disease?
osteonecrosis
pancytopenia
osteoporosis
What cardiac complication are sickle cell patients at risk for?
increased cardiac output to compensate for anemia
MI
What type of gallstones are typically seen in patients with sickle cell disease?
pigmented
acute chest syndrome
appearance of new infiltrate on CXR along with pulm symptoms
can be due to infarction or infection
What are the most common causes of malignant solitary pulmonary nodules?
primary lung cancer
metastasis
carcinoid tumor
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
Infections of what organ systems can result in PSGN (post streptococcal glomerulonephritis)?
skin or URI
What is the confirmatory test for PSGN?
kidney biopsy
igg and c3 positive bumpy deposits on renal basement membrane
What are symptoms of PSGN (post streptococcal glomerulonephritis) aside from the classic triad?
brown urine, renal failure
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
What is the classical clinical presentation of post-strep glomerulonephritis?
edema, esp periorbital
hematuria
hypertension
What levels of hematuria and proteinuria are found in PSGN (post streptococcal glomerulonephritis)?
4+ blood on urine dipstick
<3.5g protein/day
treatment for psgn
self limited
only for complications (diuretics, acei)
What is the most common anatomic location for a carcinoid tumor?
appendix
bronchopulm tree
ileum
rectum
In addition to octreotide, what therapies are indicated for the treatment of metastatic carcinoid disease?
IFN-alpha
chemoembolization of hepatic artery
What is a carcinoid tumor?
neuroendocrine origin
derived from primitive stem cells in gut wall
What are some potential complications of a carcinoid tumor?
carcinoid syndrome and carcinoid crisis
bowel obstruction
carcinoid heart disease
metastasis, recurrence
Describe the constellation of symptoms seen in a patient with carcinoid syndrome.
flushing
diarrhea
bronchoonstriction
tricuspid/pulm valvular dz
What is the best screening method for carcinoid tumor?
urine 5 HIAA
serum serotonin
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
What substance secreted by carcinoid tumors is responsible for the symptoms seen in carcinoid syndrome?
serotonin
Carcinoid tumors are most likely to be found in what patient population?
adults
rare in kids
Under what conditions can a carcinoid tumor cause carcinoid syndrome?
GI tumor metastasizes to liver
or
primary tumor outside of GI system
In a patient with carcinoid syndrome, what abnormalities may be present on cardiopulmonary exam?
wheezing
rs heart murmur
What is the standard treatment for a localized carcinoid tumor?
resection
What pharmacologic agent may be administered for symptomatic relief in a patient with carcinoid syndrome?
octreotide
kidney biopsy for iga nephropathy
increased prolif of mesangial cells
iga nephropathy classic presentation
hematuria
flank pain
low grade fever
What is the basic pathophysiology of IgA nephropathy?
igA immune complexes deposit in mesangial cells of kidneys, cause damage to glomeruli
diseases iga nephropathy is associated with
HSP
cirrhosis
celiac disease
inflamm disorders - sarcoidosis, IBD
What is the most common cause of glomerular hematuria world-wide?
iga nephropathy
What will the urine dipstick show for IgA nephropathy?
pos for blood and protein
RBC and RBC casts can be present
What is seen on light microscopy in IgA nephropathy?
normal-appearing glomeruli or mesangial widening due to mesangial proliferation
What are the medical treatment options for IgA nephropathy?
acei/arb with statins
When are steroids indicated for treating IgA nephropathy?
nephrotic range proteinuria
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
cell that accumulates in CLL
functionally incompetent, but mature appearing lymphocytes
Rai staging system
stage 0 - lymphocytosis
I, II, LAD, organomegaly
III, IV: anemia, thrombocytopenia
median survival for CLL pts
10 years
very variable
scabies
mite sarcoptes scabiei
pruritic lesions
populations affected by CLL
caucasions, male, elderly (70)
next step in treatment of localized CLL
radiation
two staging systems for CLL
Rai and Binet
most common PE findings for CLL
LAD, HSM
CLL complications
infection, anemia, thrombocytopenia
tumor lysis syndrome
Binet staging system
a: fewer than 3 LN
b: 3 or more LN
c: anemia or thrombocytopenia
CLL pts that should receive chemo
advanced, symptomatic
scabies presents with
itching
worse at night and after a hot bath
presenting symptoms of CLL
asympatomtic
b symptoms
leukemia cutis
plum-colored purpur
manifestaton of leukemia
scabies treatment
topical permethrin cream or oral ivermectin
graves dz highest risk
women, age 20-40
RA spares
DIP joints
Graves disease cellular infiltrate
lymphocytic infiltrate, germinal centers
RA first line DMARDS
MTX
sulfasalazine
hydroxychloroquine
leflunomide
NSAIDS/glucticorticoids in RA
adjuntive antiinflamm agents
RA clinical diagnosis, next step
x ray imaging:
joint erosion
joint space narrowing
subluxation
drugs for prinzmetals
dihydropyridine CCB
amlodipine, nifedipine
joint space aspiration in RA
decreased complement
increased leukocyte count
RA lab findings
increase ESR, CRP
+ RF
+ ANA
Anti=CCP
prinzmetal complication that occurs with coronary relaxation
severe life threatening arrhythmias (v fib, vtach, etc)
prinzmetal contraindicated drug
aspirin
RA - systemic manifestations
pericarditis scleritis felty's syndrome still's disease sjogren's syndrome
management of hyperthyroidism during pregnancy
PTU in first trimester
methimazole after that
meth - teratogenic, PTU - liver injury
gold standard test for prinzmetal’s angina
coronary angiography with provocative test for vasospasm (acetylcholine infusion directly into the vessel)
RA - classic hand deformitites
ulnar deviation
swan-neck deform, boutonnier deform
z-thumb
mcp joint hypertrophy
graves dz antibodies
TSH receptor
can have anti-TPO and anti-thyroglobulin
RA - joints affected first
hands and feet
rheumatoid factor is..
IgM against the constant region of patient’s own IgG antibodies
diverticulitis complications
obstruction
perforation
abscess formation
diverticulitis symptoms
LLQ or diffuse abd pain
fever
leukocytosis
diverticulitis with abscess greater than 4cm
CT guided percutaneous drainage
fecal materal or perforation –> surgery
diverticulitis imaging
CT with oral and IV contrast
treatment of diverticulitis
NPO
BS atb
IV fluids
pain control
exposure to lead in children, adults
lead paint in kids
occupational in adults
bone lead level indicative of
total lifetime lead exposure
lead poisoning treatment
dimercaprol, EDTA, succimer
use of x-ray in children with lead poisoning
looking for foreign body containing lead
dense metaphyses
hemochromatosis - HLA ?
HLA-A3
hemochromatosis when i s surgery indicated
end stage liver dz (transplant) severe arthropathy (arthroplasty)
presentation of acute lead poisoning
abdominal and neurological symptoms
secondary hemochromatosis
ineffective erythropoiesis (thalassemia, sideroblastic anemia)
multiple transfusions
chronic liver disease
lead toxicity pts peripheral smear
basophilic stippling
microcytic, sideroblastic, hypochromic anemia
triad of adult onset hemochromatosis
micronodular cirrhosis
diabetes mellitus
bronze skin pigmentation
hemochromatosis other symptoms
arthropathy/chondrocalcinosis
dilated cm
amenorrhea, impotence, hypogonadism
lead toxicity effects on fetus
miscarriages, stillbirths, low birth wt
HFE
chromosome 6
AR
liver biopsy in hemochromatosis
high iron content seen with prussian blue staining
most accurate test for lead toxicity
blood lead level
hemochromatosis treatment
phlebotomy
deferoxamine
chronic lead exposure symptoms
nonspecific GI
behavorial
peripheral neuropathies
lead-induced peripheral neuropathy
wrist or foot drop in adults
hand-eye coordination in children
hemochromatosis labs
serum iron increased
transferrin sat increase
increased ferritin
decreased TIBC
screening text for hemochromatosis
transferrin sat
lead poisoning anemia - age group
children
under 6, BBB not fully formed
areas of intrinsic weakness inside colon –
vasa recta enters muscularis externa
treatment of trichinella
self limiting
severe -bendazoles
how do humans acuqire trichinellosis
undercooked infected meat
antibiotic for cellulitis
clindamycin, linezolid
acne vulgaris is inflamm of
hair follicules, sebaceous glands
common preseentation of trichinellosis
muscle pain, tenderness, swelling, weakness
cutaneous structures affected by cellulitis
deeper dermis
subc fat
acne complications
permanent scarring
vit a - birth defects, hepatotoxicity
where does trichinella encyst
brain
heart
skeletal muscle
antibiotic for cellulitis, febrile pts
IV ceftriaxone or cefazolin
trichinella diagnosis
clinical, confirm with serology
severe acne treatment
oral isotretinoin
trichinella neuro symptom
ha worse with movement
erysipelas affects what cutaneous structures
upper dermis
superficial lymphatics
trichenella - cardiac
eosinophilic myocarditis
erysipelas specific symptoms
acute onset
systemic - fever and chills
cellulitis with systemic toxicity, suspect
necrotizing fasciitis
toxic shock syndrome
gas gangrene
AML - pt population
elderly (65), female
AML complications
anemia infection, bleeding
leukostasis, metabolic abn, CNS involvement
neutropenic enterocolitis
DIC
pathophysiology of AML
failure of bone marrow stem cells to mature
suppression of all cell lines
rapid prolif of immature myeloid cells
cholangiocarcinoma age group
elderly
AML presenting symptoms
pancytopenia
infections, fatigue, pallor, bone pain, bleeding
AML bone marrow biopsy
greater than 20% blasts
symptoms of cholangiocarcinoma
jaundice, pruritis, wt loss
cholangiocarcinoma =
cancer of bile ducts
adenocarcinoma
AML lab tests
myeloperoxidase
auer rods
myeloid markers
risk factor for cholangiocarcinoma
PSC
auer rods - what subtype
M1, M3
cholangiocarcinoma most common location
proximal common bile duct
aeur rods =
AML
myeloblasts
peroxidase positive
AML treatment
chemotherapy
treatmetn for M3 subtype of AML or APL
all trans retinoic acid (vit A)
AML supportive therapy
platelet, RBC transfusions
antibiotics
uric acid lowering agents
AML genetics
t 15 17 translocation
ITP associated with what conditions
HIV
Hep C
SLE
CLL
ITP =
acquire thrombocytopenia mediated by antibodies
ITP diagnosed by
exclusion
ITP: when to transfuse platelets
severe bleeding (GI or intracranial)
ITP treatment guided by
platelet counts
presence of bleeding
mild ITP vs severe ITP
mild - clinically silent/mild drop in platelets
severe - bleeding
ITP First line treatment
corticosteroids and/or IVIG
ITP level above to not treat
30,000
ITP second line treatment
splenectomy
rituximab
polymyalgia rheumatica common in what populations
elderly
women
african americans
what is polymyalgia rheumatica
autoimmune dz with several sites of muscle and joint pain
associated with temporal arteritis
5 PE findings in polymyalgia rheumatica
- fatigue
- edema of extremities
- tenderness to palpitation
- movement limited by pain
- normal muscle strength
polymyalgia rheumatica MRI
increased signal at tendon sheaths and tissue outside of joints
polymyalgia rheumatica lab findings
decreased hct
increased ESR
clinical presentation of polymyalgia rheumatica
morning muscle stiffness
pain in neck, shoulders, hips
treatment for polymyalgia rheumatica
low dose corticosteroids
alport’s underlying defect
type IV collagen
alport’s triad
hematuria
sensorineural hearing loss
ocural abnormalities
alports urinalysis
hematuria, proteinuria,
RBC casts
pyuria
alports treatment
ACEI/ARBs
alports electron microscopy
basketweave apperance
thickeninning/thinning of GBM
alports mode of inheritance
x-linked
who is most commonly affected by polymyositis and dermatomyositis?
poly - adults
derm - children
women
Raynaud + myositisi
1/3 will develop it
myosititis immune cells
poly - CD8 T cells
derm - B and T cells
how do polymyositis and dermatomyositis present?
symmetrical proximal limb and neck weakness over 3 to 6 months
life threatening complications associated with myositis
resp failure
myoglobinuric renal failure
cardiomyopathy
myositis - HLA subtypes
BA
DR3
DRW52
non-pharmacologic treatment may be required if there is severe inflammation associated with polymyositis and dermatomyositis?
bed rest with PT to prevent contractures
What are the first line treatments for polymyositis and dermatomyositis?
oral glucocorticoids
topical steroids for derm
What is the age distribution of patients commonly affected by polymyositis and dermatomyositis?
bimodal
10-15
45-60
What is the second line therapy for polymyositis and dermatomyositis?
MTX,aza, other immunosuppressants
In addition to symmetrical proximal limb weakness, what symptoms are associated with polymyositis and dermatomyositis?
myalgia
dysphagia/arthralgia
raynaud
What limitations associated with polymyositis and dermatomyositis may cause patients to first visit a physicians office?
trouble combing hair or climbing stairs
How are polymyositis and dermatomysitis diagnosed?
- symmetric proximal muscle weakness
- elevated skeletal muscle enzymes
- myopathic changes on EMG
- inflamm on muscle biopsy
Gottron sign
lacy purple rash around MCP or PIP joints
pathophysiology of polycythemia vera
JAK 2 mutation
stimulation of EPO receptor
polycythemia vera imaging
look for tumor that produce EPO (pheo, RCC, hemangioblastoma, HCC)
what is polycythemia vera
myeloproliferative disorder
increased RBC volume
hyperviscosity syndrome
What are some complications of polycythemia vera NOT related to hyperviscosity syndrome?
post-PV myelofibrosis
AML
What lab findings are required to make the diagnosis of polycythemia vera?
elevated hgb, hct
jak2 mutation
What are some complications of polycythemia vera related to hyperviscosity syndrome?
MI
ischemic stroke
budd-chiarir syndrome
What is the life expectancy of treated & untreated polycythemia vera patients?
10 or more years vs 6-18 months
What are the 5 aspects of medical treatment of polycythemia vera?
phlebotomy myelosuppresive agents - hydroxyurea aspirin antihistaminies uric acid lowering agents
What are the 5 chief presenting symptoms of polycythemia vera?
aquagenic pururitis burning pain in hands & feet fatigue easy bleeding and bruising headache
most common causes of death in polycythemia vera?
thrombosis
hematologic malginancies
hemorrhage
post pv-myelofibrosis
polycytehmia vera oxygen sat
> 92%
otherwise hypoxia-induced erythrocytosis
EPO in polycythemia vera
should eb low (negative feedback)
polycythemia vera bone marrow biopsy
generalized hypercellularity
polycythemia vera PE
HSM
TdT & CD10
ALL
ALL symptoms
nonspecific
ALL treatment
chemotherapy
prophylatic intrathecal chemo with MTX
T cell ALL symptoms
SOB due to mediastinal mass
effect of ALL on other cell lines
decreases other cell lines
thrombocytopenia, anemia, neutropenia
ALL peripheral smear
lymphoblasts
ALL prognosis
cured in children
worse in adults
ALL cell of origin
immature lymphoblasts
B cells > T cells
ALL population
children age 2-5
boys, caucasians
aplastic anemia - 2 broad mechanisms
injury to pluripotent stem cells
hereditary
aplastic anemia =
diminshed or abscent hematopoietic precursors in bone marrow
aplastic anemia drugs/toxins
radiation
anti-epilpetics
nsaids
sulfonamides
aplastic anemia symptoms
anemia – fatigue
leukopenia - recurrent infections
thrombocytopneia - mucosal hemorrhage
aplastic anemia caused by what viruses
parvovirus b19
hepatitis
HIV
aplastic anemia hereditary cause
fanconi anemia (impaired dna repair mechanisms)
aplastic anemia bone marrow biopsy
hypocellulairty
no fibrotic infiltration or malignant cells
morphologically normal
aplastic anemia treatment
removal of offending agent
supportive care
transplant or immunosuppressive thearpy
hodgkin lymphoma cell type
B cell
hodgkin lymphoma histo findings
Reed-Sternberg cells in inflamm background
4 types of hodgkin lymphoma
lymphocytic predom
nodular sclerosis
mixed cellularity
lymphocyte depleted
hodgkin lymphoma pt risk factors
age 20 & 65
EBV virus
family hx
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
Reed-sternberg cells
CD 15 and CD 30
presenting symptoms of hodgkin lymphoma
cervical LAD
nonspecific b symptoms
early vs late hodgkin lymphoma
early - cure (chemo + rad)
late - prognosis by IPS (chemo only)
hodgkin lymphoma treatment
early - chemo and rad
late - chemo only
cause of vit D def
poor intake increased loss impared 25-hydrox impaired 1-alpha hydroxy target organ resistance
treatment of osteomalacia due to poor diet
50,000 IU weekly for 3-12 weeks
800IU daily
osteomalacia and rickets
impaired calcification of newly formed bone matrix
treatment of osteomalacia due to increased metabolism or impaired hydroxylation
50,000 IU weekly
impaired 25-hydroxylation due to
liver disease
isoniazid
treatment for osteomalacia due to imapired 1alpha hydroxylation
give active form (1,25)oh2 vit d
What causes of vitamin D deficiency are associated with impaired 1alpha-hydroxylation?
renal osteodystrophy
hypoparathyroidism
What changes to bone structure occur in rickets?
changes in growth plate (hypertrophic cartilage) and bone bowing/bone cortical thinning
What is meant when osteomalacia and rickets are called a “qualitative” defect?
process of bone mineralization is abnormal
How much calcium should patients with vitamin D deficiency supplement per day?
1.5-2g
How is iatrogenic osteomalacia managed?
withhold offending med
What causes of vitamin D deficiency are associated with increased loss of vitamin D?
increased metabolism
imapired enterohepatic circulation
nephrotic syndrome
rickets lab values
decreased 25 vit d
decreased ca, phosphate
increased alk phos, PTH
rickets x ray findings
Widening of physes Bowing of long bones Translucent lines in bones Flattening of the skull Enlarged costal cartilages
What results from the hypocalcemic state seen in osteomalacia and rickets?
hypocalcemia tetany
What causes of vitamin D deficiency are associated target organ resistance?
vit d receptor mutation
phenytoin
internal organs affected by scleroderma
GI tract
lungs
MSK
organ system most often involved with systemic sclerosis
skin
thick, tight, shiny skin
populations affected by scleroderma
women
black
pulmonary complications in systemic sclerosis
interstitial fibrosis
how to treat esophageal conditions of scleroderma
PPI, H2
strictures may need surgical correction
what causes the GI symptoms of scleroderma
decreased motlitly
overgrwoth of bacteria and malabsorption
scleroderma age range
20-50
CREST
anti-centromere Ab, skin calcification, raynaud phenomenon, esophageal dysmotility, sclerodactly, telangiectasia
esophageal conditions associated with scleroderma
esophageal reflux
esophageal candida
scleroderma - treatment of joint contractures
physical therapy
what three processes contribute to scleroderma pathogenesis
overproduction of ECM components
microvascular dysfxn
dysfxn cell/humoarl immunity
other diseases associated with scleroderma
PBC
malignacies
spontaneaous PTX
scleroderma & the heart
myocardial fibrosis –> CHF, arrhythmias
systemic sclerosis antibodiy
anti-topoisomerase-1 (anti-scl-70)
scleroderma GI symptoms
diarrhea, constipation, esophageal disease
GI symptoms treatment in scleroderma
promotility agents
metronidazole
treatment for scleroderma renal crisis
ACEI
treatment for sclerotic vessels in scleroderma
vasodilators
scleroderma renal crisis
sudden onset of hypertension and renal insuff
treatment of rapidly progressive scleroderma
cyclophophamide, steroids
