outpatient week 7 Flashcards
(151 cards)
1
Q
common causes of acute bilatearl cervical lymphadenitis
A
Viral URI
GAS
2
Q
common cause of chornic bilateral cervical lymphadenitis
A
EBV, CMV
3
Q
common cause of acute unilateral cervical lymphadenitis
A
S aureus
GAS
4
Q
noninfectious causes of cervical lymphadenitis
A
CTD, leukemia, lymphoma, kawasakia
meds
5
Q
common cause of chronic unilateral cervical lymphadenitis
A
nonTB myobacterium
bartonella henselae
6
Q
What is the most common congenital long QT syndrome?
A
Romano-Ward syndrome
AD
7
Q
What is the mainstay of treatment for patients with congenital long QT syndrome?
A
non-cardioselective beta blockers: propanolol, nadolol
8
Q
What is congenital long QT syndrome?
A
affects myocardial repolarization
9
Q
What congenital neurological disorder only affecting females is associated with QT prolongation?
A
Rett syndrome
10
Q
What 6 common pharmacological agents are known to cause acquired long QT syndrome?
A
quinidine, amiodarone
haloperiodal
eryhtomycin
11
Q
A pediatric patient with impaired hearing and palpitations should be suspected of having what congenital condition?
A
Jervell and Lange-Nielsen syndrome
sensoineural deafness
12
Q
What arrhythmias are associated with congenital long QT syndrome?
A
ventricular tachyarrythmias
torsades de pointes
13
Q
Juvenile Angiofibroma
A
nasal obstruction, nasal mass, frequent nosebleeds
14
Q
Fanconi Anemia
A
abnormal thumbs, hyper/hypo pigmentation
15
Q
pilocarpine inotrophoresis =
A
sweat test
16
Q
galactosemia
A
FTT
bilateral catarcts
jaundice, hypoglycemia
17
Q
Potter Sequence
A
UT anomaly –> anuria in utero –> oligohydroamings –>
pulm hypoplasia, flat facies, limb deformation
18
Q
neonatal polycythemia
A
hct more than 65%
19
Q
leesch nyan
A
x linked
def HPRT
self harm
20
Q
most common predisposing factor to orbital cellulitis
A
bacterial sinusitis
21
Q
tinea capitis treatment
A
griseofulvin
22
Q
fulminant meningococemia
A
purpura on flank due to adrenal hemorrhage
23
Q
hyper igm
A
decrease igg, iga, increased igm
x linked
cd40 igand
24
Q
HUS smear
A
schistocytes
large platelts
25
minimal change disease histopath
normal
26
most common causes of osteomyelitis in SCD
salmonella and staph
27
anemia of prematurity
normocytic, normochronmic
low retics
nml everything else
28
umbilicated vesicles
herpec eczema
29
stroke after foreign body injury
ICA dissection
30
retinal vascularization
16 weeks
| center outward
31
ROP=
vasoprolifererative disorder of the eye
32
incidence of ROP in infants weight less than 1250g
50-70%
33
resp failure contributes to ROP by
hypoxemia - upregulate VEGF
hyperoxemia - downregulate VEGF
BV constrict
34
screening for ROP
infants less than 1500g or GA of 31 weeks or less
35
mump complications
orchitis
| meningitis
36
mumps neuro complications
encephalitis, deafness, GBS, transverse myelitis
37
mumps hallmark
swollen, tender parotid glands
38
mumps sterility
if bilateral
39
mumps type of virus
ss RNA, paramyxovirus
40
mumps affects what tissues
parotid glands
testis, ovaries
meningies
41
mumps meningitis prognosis
self limited
42
mumps incubation period
18-21d
43
antibody titer rise in mumps
fourfold rise
44
ASD physical exam
wide fixed split s2
| systolic ejection murmur at ULSB
45
What additional test is performed during echocardiography to better visualize a patient’s atrial septal defect?
agitated saline contrast can illustrate the defect
| multiple bubbles
46
What are some complications that may be seen in patients with an uncorrected atrial septal defect?
```
eisenmenger's syndrome
pulmonary htn
RV dysfxn -- RHF
a fib
stroke, paradoxical embolus
```
47
ASD CXR
right heart enlargement
| increased pulmonary vascular markings
48
How does excessive urinary loss of protein lead to prothrombotic state that would predispose patients to renal vein thrombosis?
decreased antithrombin III
49
Renal vein thrombosis is most commonly seen in patients with what underlying disorder?
nephrotic syndrome
- 3g/d protein loss
- hypoalbumin
- hyperchol
- edema
50
What are four general signs and symptoms associated with renal vein thrombosis?
hematuria, pain
| flank masses, oliguria
51
What signs of nephrotic syndrome should patients with renal vein thrombosis be observed for?
edema, anasarca
52
What findings may signal bilateral renal vein thrombosis in the pediatric patient?
gross hematuria
| bilateral flank masses
53
What risk factors are associated with renal vein thrombosis of infancy?
RCC, antiphospholipid
umbilical venous cather placement
behecents
moms with DM
54
What is the method of choice for noninvasively diagnosing renal vein thrombosis?
CT with contrast
55
What ultrasound findings are associated with renal vein thrombosis?
swollen, echogenic kidneys
56
What pharmacologic agents can be used to reduce proteinuria associated with renal vein thrombosis?
ACEI, ARB
57
What prothrombotic complication may result from renal vein thrombosis? What prophylaxis should be administered?
pulmonary embolism
| warfarin
58
When is surgical intervention indicated in the management of renal vein thrombosis?
when associated with RCC
59
horseshoe kidney and malignancy
wilm's
60
pathophys of horseshoe kidney
midline fusion of lower kidney poles
61
horseshoe kidney complications
compressing ureters --> UTI, nephrolithiasis
62
What symptoms should prompt a physician to suspect the presence of an osteoid osteoma?
neck, back pain
painful scoliosis
radicular or referred pain to lower limb or shoulder
63
What is the role of biopsy in the workup of an osteoid osteoma?
rarely needed
64
What is the typical presentation of a patient with an osteoid osteoma?
pain that is worse at night
| relieved by aspirin
65
What is the most common location for osteoid osteomas to develop?
shafts of long bones
66
How is an osteoid osteoma diagnosed?
plain radiographs
67
What malignant changes are associated with an osteoid osteoma?
none!
68
What is an osteoid osteoma?
benign osteoblastic neoplasm in young men
69
What makes osteoid osteomas difficult to diagnose during the initial presentation?
pain precedes their apperance on imaging
70
What is the appearance of an osteoid osteoma on plain radiograph?
small central nidus wiht surrounding bony sclerosis
71
What treatment options are available for patients with an osteoid osteoma?
medical
radiofrequency ablation
surgical
72
osteoid osteoma medical treatment
aspirin, nsaids
73
What are the physical exam findings of ventricular septal defect? What are the characteristics of the murmur?
pansystolic murmur at LLSB
systolic thrill
loud s2
74
What are the clinical manifestations of ventricular septal defect?
resp infections, FTT
dyspnea, SOA
heart failure
75
In patients with a large ventricular septal defect, what is seen on the electrocardiogram?
LVhypertrophy w/or without RVH
76
What is the management of a small ventricular septal defect?
monitered
| medically managed
77
What is seen on chest X-ray in patients with symptomatic ventricular septal defects?
cardiomegaly
marked pulmonary vasculature
pulmonary edema
78
VSD medical management
diuretics
| ACEIs
79
In what scenario is surgical closure of a ventricular septal defect contraindicated?
irreversible pulmonary htn
80
pathogenesis of beta-thalassemia major
impaired production of beta chains
| excess of alpha chains
81
what thalassemia is usually fatal before birth
hemoglobin barts - 4 gamma globin chains
82
pathogenesis of alpha-thalassemias
defective alpha-globin production
| excess beta globin chains
83
difference btwen HbA and Hb2
```
HbA = alpha + beta
HbA2 = alpha + delta
```
84
types of alpha thalassemis
minima
minor
HbH disease
hydrops fetalis with Hb Barts
85
pathogenesis of beta thal minor
mild decrease in beta chain production
86
age which beta-thal major presents
after 6 months of life
87
anemia associated with beta thal
microcytic, hypochromic
88
management for beta thal major
hyperransfuation, iron chelation therapy
stem cell transplant
splenectomy, vitamins
89
target cells
b thal major
90
HbH disease
neonatal jaundice and anemia
| HbH = 4 beta chains
91
What two cardiac anomalies must be present to support the initial survival of a patient with hypoplastic left heart syndrome?
PDA
| ASD
92
What are the physical exam findings of hypoplastic left heart syndrome.
cyanosis
| no murmur
93
What are the diagnostic features of hypoplastic left heart syndrome on echo?
small LV, abnormal left sided valves, hypoplastic ascending aorta
94
What is the typical symptomatic presentation of an infant with hypoplastic left heart syndrome.
cyanosis, resp distress
95
What are the two main elements of the INITIAL management of hypoplastic left heart syndrome?
prostaglandin to maintain PDA
| if no ASH - transcather or surgical atrial septostomy
96
What are the later in life complications of treated hypoplastic left heart syndrome
thrombotic complications
neurodevelopmental impairment
decreased exercise tolerance
97
How does hypoplastic left heart syndrome progress if left untreated at birth?
heart failure, cardiogenic shock, death
98
What is the path that oxygenated blood takes through the circulation of an infant with untreated hypoplastic left heart syndrome.
lungs - pulmonary veins - LA - ASH - RA - RV - pulm arteries - PDA - aorta - body
99
inheritance of hypoplastic left heart syndrome
sporadic
100
What genetic syndromes has hypoplastic left heart syndrome been associated with?
trisomy 13,18
turners
jacobsen's
101
hypoplastic left heart syndrome diagnosed prenatally
1/2 of time
102
infants with Chediak-Higashi present...
absence of skin pigmentation
103
What is the typical prognosis of Chediak-Higashi syndrome?
fatal in childhood
104
What follow-up diagnostic studies are required once the diagnosis of Noonan syndrome is made?
ECG, echo
105
How is Alagille syndrome managed?
maybe liver transplant
106
What is the therapy of choice for Chediak-Higashi syndrome?
bone marrow transfer
107
pulmonic valve issues or stenosis
noonan
108
How is common variable immunodeficiency diagnosed?
low ig levels
poor vaccine response
decreased cd4/cd8 ratio
109
What can result from recurrent pulmonary infections associated with common variable immunodeficiency?
bronchiectasis
110
Generally speaking, where do physical exam findings of common variable immunodeficiency manifest?
lungs
skin
autoimmune
111
What dermatologic manifestations are associated with common variable immunodeficiency?
alopecia
| polyarteritis nodosa
112
alagille syndrome diagnosis
clinical
| JAG1 or NOTCH2 mutations
113
symptoms of cyclic neutropenia
fever, pharyngitis, givngivitis, stomatitis, bacterial infections
114
CVID treatment
IVIG
115
When may splenectomy be associated with common variable immunodeficiency?
severe autoimmune thrombocytopenia or hemolytic anemia
116
What children with Langerhans cell histiocytosis require chemotherapy?
lesions involving orbit, mastoid, temporal bones
117
What is the “accelerated phase” of Chediak-Higashi syndrome?
lymphoma like infiltation of multiple organs
| precipitated by viral infection like EBV
118
CVID=
AD disorder affecting B cells
| low igs
119
Dubin-Johnson PE findings
HSM
120
treatments for Noonan syndrome
somtattropin
121
Noonan mutation
PTPN11, SHP-2 protein
122
cyclic neutropenia pathophys
disorder of bone marrow stem cells
| arrest of myelocyte maturation
123
Chediak-Higashi pathophys
LYST or CHST1 gene
| dysfunction of neutrophils
124
Dubin JOhnson syndrome abnormal transport
bilirubin-glucuronide transport across membrane separting hepatocyte from bile canaliculi
125
cyclic neutropenia treatment
recombinant G-CSF
126
Dubin-Johnson gene mutation
MRP2
127
Hand-Schüller-Christian triad
lytic bone lesions
exopthalamos
central DI
128
langerhans cell histiocytosis symptoms
```
lytic bone lesions
skin lesions (cradle crap)
```
129
Chediak-Higashi syndrome diagnose
peripheral smear finding of giant granules in neutrophils, eosinophils, and granulocytes
130
cause of adult form of cylic neutropenia
cytotoxic cd56+ large granular lymphocytes
131
Alagille syndrome presentation
cholestaiss -- jaundice
| butterfly vertebrae
132
How is Langerhans cell histiocytosis diagnosed in children?
biopsy, cd1a staining
133
cyclic neutropenia diagnosis
neutropenia of
134
What clinical features besides skin rash and lytic bone lesions are associated with Letterer-Siwe Disease?
LAD, HSM, ANemia, thrombocytopenia
135
What laboratory feature is characteristic of Langerhans cells?
birbeck granules
136
What laboratory findings are associated with Alagille syndrome?
prolonged PT, PTT
HLD
elevated GGT, bilirubin
137
CVID autoimmue diseases
RA, vitilgo, hemolytic anemia
138
death in CVID
lymphoma
139
What results from the liver dysfunction of Dubin-Johnson syndrome?
chronic conjugated hyperbilirubinemia
140
What is the inheritance pattern of Alagille syndrome?
AD
141
What is the pathophysiology of Rotor syndrome?
reduced ability of liver to uptake bilirubin from blood
142
hallmark of PRader Willi during neonatal period
neonatal hypotonia
143
Noonan syndrome cardiac
dysplastic/stenotic pulmonic valve
HCM
ASD, VSD
144
The mutated genes in Alagille syndrome normally regulate what developmental processes?
3-dimensional intrahepatic biliary architecture.
145
Dubin-Johnson associated conditions
chronic hep b
hx of tubercular lymphadenitits
chronic cholestasis
CoronaryHD
146
treatment of fanconi anemia
G-CSF
androgens
hematopoietic cell transplantation
147
dubin johnson diagnosis
increase in ration or coproporphyin 1 to 2
148
dubin johnson treatment
no treatment
149
dubin johnson scintigraphy findings
extended period of liver visualization after dye injection
150
Chediak-Higashi Syndrome
AR
hypopigmentation
neutrophil dysfunction
151
cyclic neutropenia genetic mutation
elastase gene
