Board Review 1 Flashcards
1
Q
Mutation in MEFV gene implies… consequences and management?
A
diagnosis of familial Mediteranean fever; untreated –> inflammation, amyloidosis, renal failure. Start colchicine, 0.6 mg po bid.
1
Q
Pulmonary presentation of FMF?
A
recurrent chest or abdominal pain lasting 24-72 hours, responds to colchicine
2
Q
For patients with FMF who do not respond to colchicine, try…
A
etanercept, thalidomide, infliximab
3
Q
Discuss nodular tumorous sarcoidosis.
A
Lower extremity involvement is common. Often multiple nodules. Often weakness and tenderness. Commonly with extramuscular sarcoid (LAD, cuteneous, etc.). Response to steroids is inconsistent.
4
Q
Pulmonary complication of a double aortic arch?
A
Encircles the trachea, can cause compression. Most often associated with tracheomalacia.
5
Q
What is napkin ring trachea?
A
incomplete separations of the cartilage rings; associated with anomalous origin of the left pulmonary artery
6
Q
What is the lab diagnosis of carcinoid syndrome?
A
24-h urinary 5-hydroxyindoleacetic acid
7
Q
Biopsy and management of endobronchial sarcoid?
A
Large series showed that endobronchial biopsy is safe despite high vascularity. Resection is primary management.
8
Q
What are the only two agents that have improved cough in the common cold?
A
Dexbrompheniramine and naproxen.
9
Q
What lung malignancy is the most common cause of SVC syndrome?
A
small cell lung cancer; up to 20% of patients with small cell develop SVC syndrome
10
Q
Diabetes insipidus in a young person who smokes… think:
A
Langerhans cell histiocytosis
11
Q
Most important treatment for LCH:
A
cessation of smoking
12
Q
Bony involvement in LCH?
A
Common: 20% have bone involement, often flat bones (e.g. ribs)
13
Q
What airway diseases are seen in IBD? (3)
A
follicular bronchiolitis: extensive lymphoid follicle formation in the respiratory bronchiolar walls; also organizing pneumonia and bronchiectasis
14
Q
In patients with HIT, which agent do you use in which population?
A
argatroban (cleared hepatically) in patients with renal failure, lepirudin (cleared renally) in patients with liver injury
15
Q
What is the word for applying a powedered slurry tot he pleural space?
A
poudrage
16
Q
Discuss lupus pernio.
A
indurated plaques with discoloration of nose, cheeks, lips, ears; often associated with bone cysts and pulmonary fibrosis; infrequent remission; African-american women; nontender; do not ulcerate
17
Q
What is Lofgren syndrome? What is the prognosis?
A
EN, bilateral hilar adenopathy, arthralgias; remission is common
18
Q
Of testicular cancers, which is the more aggressive?
A
Nonseminomas (embryonal cell carcinoma, choriocarcinoma, yolk sac tumor and teratoma) are more aggressive than seminomas.
19
Q
Unusual presentation of choriocarcinoma?
A
Often metastasizes hematogenously, with the testicular primary burning out. Rarely originates in the lung and is associated with a DAH-like syndrome.
20
Q
Standard chemotherapy for testicular cancer?
A
bleomycin, etoposide and cisplatin
21
Q
What are the relevant SUV ranges in PET’s?
A
Malignant lesions have an SUV of 2.5 to 15.
22
Q
How does diabetes complicate the use of PET?
A
May cause false negatives because of increased dilution of the radiolabeled tracer in nonlabeled serum glucose.
23
Q
How are the modified Light criteria different from the orignal ones?
A
LDHp/LDHs greater than .45 ULN rather than .66.
24
New metaanalysis-derived criteria for exudate?
Protein ratio greater than .5, LDH ratio greater than .6, LDH greater than .45 ULN, PF protein greater than 2.9 and PF cholesterol greater than 45.
25
Discuss granular cell tumors.
Histology: submucosal infiltration by aggregate of ovoid-to-polygonal cells of moderate size, lots of granular eosinophilic cytoplasm, small hyperchromatic nuclei. Likely Schwann cell-derived. Can occur all over (tongue, skin, breast), rarely in larynx, trachea and bronchial tree, lung parenchyma. Middle-aged adults, can be multicentric. Can see lots of endobronchial lesions. Growth is slow, can resolve spontaneously. Treatment is symptomatic: endobronchial or sleeve resection if needed.
26
How common is pleuropulmonary disease in patients with rheumatoid arthritis? What is the usual sequence of presentation?
50-70%, though most are asymptomatic. 80% of the time joint complaints come before pulmonary manifestations.
27
What is the male:female breakdown of RA-associated lung disease?
Though RA is more common in women, pulmonary disease is more common in men (3:1).
28
What is the most common pulmonary manifestation of rheumatoid arthritis?
ILD (up to 58% of patients when using HRCT)
29
Threshold for treatment with PAP in OSA?
All patients with an AHI of 15/h or greater or for symptomatic patients (e.g. excessive daytime sleepiness) with an AHI of 5-30/h.
30
How do the delivered pressures differ in autotitrating PAP and conventional CPAP?
Mean delivered pressure is generally lower during APAP compared with CPAP, but peak pressures may be higher.
31
In whom is APAP (autotitrating PAP) contraindicated?
Patients with other reasons for desastting: CHF, COPD, daytime hypoxemia, etc..
32
Why is "superficial femoral vein" something of a misnomer?
It is part of the deep venous system of the thigh.
33
Duration of therapy in a pregnant woman with DVT?
Throughout pregnancy and at least 6 weeks postpartum to complete three months.
34
How, histologically, does one distinguish between mesothelioma and adenocarcinoma?
MPM stains for epithelial membrane antigens as opposed to the cytoplasmic pattern seen in adenocarcinomas. CEA and CD15 are negative in MPM, wheras positive staining for cytokeratin 5, cytokeratin 6, calretinin and vimentin are specific for MPM.
35
How is pleural fluid analysis in mesothelioma?
Unreliabe. Reactive cells from other malignancies such as sarcomas and adenocarcinomas are difficult to distinguish from MPM. Pleural fluid cytology yield: 25-33%.
36
Discuss pleural biopsy in mesothelioma.
Yield is ~90%, but seeding of tumor track is common (40% of cases) and may be prevented by local radiation.
37
Chemotherapy in mesothelioma?
Absolutely no improvement in survival from anthracycline or platinum based regimens. Some newer generation folate inhibitors combined with cisplatin are showing promise.
38
Bottom line on PCP in patients without HIV:
more fulminant, though PCP burden is much lower
39
Utility of BAL vs induced sputum in PCP?
Induced sputum is reasonable in patients with HIV given higher fungal burdern. But low yield in non-HIV patients.
40
Discuss sirolimus lung toxicity.
Most common among solid organ transplant recipients (kidney mostly). 11% in one series. Most common: interstitial pneumonitis with COP. DAH and vasculitis are also reported. Patchy alveolar and interstitial infiltrates with GGO and consolidation, often peribronchial. BAL: lymphocytes. Histology: lymphocytic pneumonitis, organizing pneumonia, epitheliod granulomas. Stop sirolimus, try steroids.
41
Two most common scenarios for lipoid pneumonia:
old people taking mineral oil for constipation at night, and people using petroleum nasal gels for dry nasal passages
42
technical description of crazy paving:
GGO with a mesh-work of reticular lines representing thickened intralobular septa
43
How do you stain for lipoid pneumonia?
BAL stained before fixation with oil-red-O or Sudan red will show alveolar macrophages with vacuolated cytoplasms
44
How does hypertrophic osteoarthropathy affect lung cancer staging?
It doesn't.
45
What is the most sensitive imaging modality for hypertrophic osteoarthropathy?
99mTc bone scan.
46
Which patients seemed to derive benefit from LVRS?
marked exercise impairment (under 25W) and upper lobe disease
47
Summarize methotrexate lung injury.
Generally within first year of treatment. Dyspnea, cough, fever. Patchy airspace disease with thickened septa, scattered GGO. Crazy paving. Generally high CD4/CD8.
48
BAL findings in organizing pneumonia?
Similar to HP: lymphocytosis, decreased CD4/CD8.
49
Initial treatment of organizing pneumonia?
corticosteroids 0.75 - 1.5 mg/kg/day with slow taper over 3 to 12 months.
50
Explain most definitive treatment for aspirin-exacerbated respiratory disease.
desensitization followed by daily ASA administration; up to 87% experience improvements after a year: better sinus symptoms, need for steroids, sense of smell, asthma symptoms; ASA must be continued indefinitely, as sensitivity will recur within 7 days if stopped
51
Discuss treatment of carcinoid tumors.
Treatment is surgical resection with systematic sampling of mediastinal and hilar nodes.
52
What is the most common source of ectopic ACTH production?
Pulmonary carcinoid tumors. They can cause Cushing syndrome years before they are detected radiographically.
53
What fraction of all carcinoid tumors are pulmonary?
25%. Most are in the gut.
54
What is the classification system for neuroendocrine tumors?
1) typical carcinoid, 2) atypical carcinoid, 3) large cell and small cell carcinomas
55
Medication options for thoracic endometriosis?
OCP's, danazol, GRH analogues
56
What effect has BiPAP been shown to have on patients with Cheyne-Stokes due to CHF?
increase in incidence of central apneic events (both compared to baseline and to CPAP)
57
Four treatments for Cheyne-Stokes in CHF. What outcome do they improve?
acetazolamide, nocturnal O2, theophylline, CPAP; all improve frequency/duration of central apneas, but not survival
58
One-liner on Idiopathic Pneumonia Syndrome.
In BMT patients, diagnosis of exclusion in 1-6 month posttransplant period with interstitial disease and restriction on PFT's.
59
What is unusual about busulfan lung toxicity?
Can occur long after drug administration, often more than 4 years later, usually with basilar interstitial disease an restrictive dysfunction.
60
Three flavors of cyclophosphamide lung injury.
Acute pneumonitis, organizing pneumonia, or chronic fibrosis.
61
How do you stain for LAM? How about for LCH?
LAM: cells stain positive for human melanin black-45 (HMB-45), a monoclonal antibody against melanoma extract. LCH: S-100 protein.
62
Key clinical differences between LAM and LCH:
LAM: women of childbearing age. LCH: 90% are current smokers. LAM: just cysts, few nodules. LCH: nodules and cysts, and cysts are both thin- and thick-walled. LCH: sparing of the bases.
63
Recommendations for discharging a patient with active TB?
Abdolutely not if someone at home has impaired T-cell immunity or is under 4. Wait 2 weeks after starting treatment, test those at home for latent TB and follow them closely.
64
Discuss 'sarcoid' in a patient at high risk for malignancy.
A sarcoid-like reaction can be seen in patients with history of malignancy. Non-Hodgkin, Hodgkin lymphomas, but also primary or metastatic carcinomas. Probably an immunologic reaction to antigens releasted by tumor cells via lymphatic channels inducing a T-cell mediated response ? activate macrophages, create epithelioid granulomas.
65
How dramatic were the advantages in length of mechanical ventilation and ICU stay in the FACTT trial?
MV: 2.5 days; LOS: 2.2 days
66
How useful is a serum anti-GBM IgG in diagnosing Goodpasture's?
Diagnostic if positive, but only 60% sensitive.
67
Expalin the confusing crossover in diagnosing pulmonary-renal syndrome.
Up to 38% of patients with anti-GBM antibodies will also have positive p-ANCA levels in settings of a systemic vasculitis. And plasmapheresis would be indicated.
68
What is the main complication of an aspergilloma?
Massive hemoptysis. But in a large series, the incidence of bleeding was no higher than in simple healed TB cavities.
69
Invasive diagnosis of aspergilloma?
When you've got a mobile mass in a cavity and a past history of TB, the diagnosis is made (over 95%) and there's no need for further diagnostics.
70
One-liner on benign fibrous tumors of the pleura.
Formally called benign mesotheliomas. No relationship to asbestos nor tobacco use. May be associated with paraneoplastic syndromes like hypoglycemia.
71
With regard to CPET's, what is the significance of periodic breathing?
Oscillatory patterns in VO2, VCO2, and Ve/VO2. Cyclic hyperpnea and hypopnea in exertion and sleep reflect cardiomyopathy and are presumably due to variations in cardiac output and altered cardiopulmonary reflex control.
72
Discuss vocal cord papillomatosis.
Caused by HPV. Tend to recur and often require recurrent therapy. Also called RRP (recurrent respiratory papillomatosis). Use respiratory precautions when ablating with laser because of risk of aerosolized virus. Alternate therapies for recurrence: IFN-alpha, methotrexate, cruciferous vegetables (brocolli and cabbage), cidofovir.
73
Significant risk factors for iatrogenic pneumothorax in the ICU?
ARDS on admission, AIDS, low weight (under 80 kg), cardiogenic edema, inotropes within first 24 hours, central line in first 24 hours.
74
What PFT values predict high perioperative mortality and pulmonary disability in lung cancer resection?
FEV1 under 30%, FEV1 x DLCO under 1,650%.
75
Discuss lymphangiomatosis.
Not LAM. Increased number of complex anastamosing lymphatic channels (multiple and diffuse lymphangiomas) in the liver, spleen, bone, soft tissues, mediastinum or lung. Often fatal in childhood, but can be delayed in onset and manifest in adolescence or early adulthood. Progressive, poor prognosis. Chylothorax, chylopericardium, lymphedema. No positive treatment trials. Various CT findings (effusions, GGO, thickened intralobular septae).
76
What is lymphangiectasis?
Dilation of existing lymphatic channels without an increase in their number or complexity.
77
What are lymphangiomas?
Slow-growing lesions with proliferating lymphatic channels. Come from sequestered lymphatics that don't communicate with lymphatic system. Grow cystic. Also called cystic hygromas. Often neck/skin/soft tissue of infants and children. Benign.
78
Acuity of presentation of lupus pneumonitis?
There is both an acute and a chronic presentation.
79
What happens to residual volume when there is severe neuromuscular weakness?
It gets increased.
80
In a patient with ASA-exacerbated respiratory disease (AERD) and refractory nasal symptoms, what is the optimal treatment?
Nasal polypectomy, aspirin desensitization and treatment: this is the only intervention (other than chronic steroids) that has delayed need for recurrent polypectomies.
81
Mechanism of action of varenicline? Side effects?
Selective alpha4beta2 partial agonist at nicotinic acetylcholine receptor. Releases dopamine that counteracts common nicotine withdrawal symptoms, also acts a partial antagonist by blocking further nicotine binding to the receptors. Side effects are nausea and insomnia.
82
If instead of FEV1/FVC under 70 we use the 95% confidence interval, what will happen to our diagnosis of obstructive lung disease?
We will overdiagnose OLD in older patients and underdiagnose it in younger ones. Studies of patients in this gap show no difference in long-term mortality but increased hospitalizations.
83
Histological appearance of mucor?
Nonseptated, wode-angle branching hyphae.
84
How might our prophylaxis in neutropenic patients be giving rise to a serious infection?
Increasing mucor rates may be due to prophylaxis with voriconazole.
85
Voriconazole is better than amphotericin for _____ but worse (ineffective) against \_\_\_\_\_. What newer agent is different?
aspergillus, mucor. Posaconazole has activity against mucor.
86
Discuss DIPNECH.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Growth of NE cells within walls and lumens of small airways resulting in an obstructive lung disease that is clinically similar to obliterative bronchiolitis. Can see carcinoid tumorlets, scattered nodules. NE cells stain positive with chromogranin. Always nonsmoking women between 50 and 60. Octreotide may help. Prognosis 10-15 years.
87
Constellation that should make you think of follicular bronchiolitis?
bronchiectasis, CTD and inflammatory bowel disease
88
Clinical mimic of idiopathic OB?
DIPNECH (diffuse idiopathic pulmonary neuroendocrine cell hyperplasia)
89
Best way to exclude occupational asthma?
Negative methacholine challenge test within two weeks of the exposure.
90
Discuss hard metal disease.
AKA cobalt-related interstitial lung disease. Key: 1) cobalt exposure and 2) giant cell interstitial pneumonia (GIP) or finding multinucleated "cannibalistic" cells in BAL fluid. Treatment is avoidance. Maybe steroids.
91
Occupations involving cobalt exposure:
maintenance of hard metal tools, production of hard metals, diamond tooling, oil well drilling sites (using tungsten carbide), armored plate production, manufacture of cutting tools and loops for fishing poles.
92
Giant cell interstitial pneumonia is nearly pathognomonic for:
hard metal disease.
93
Occupational exposures to berrylium:
nuclear weapons, ceramics, computer manufacture, aerospace electronics employment
94
Diagnosis and treatment of solitary fibrous tumors of the pleura (SFT)?
Surgical resection with 1- to 2- cm margins. Failure to have clear margins is associated with local recurrence, metastasis. Most (80%) are benign but 20% have malignant components and risk for metastasizing. Diagnosis should include CD-34 staining of biopsy.
95
Interesting paraneoplastic phenomenon in solitary fibrous tumors of the pleura?
In large (greater than 10 cm) tumors, hypoglycemia is reported, due to increased insulin-like growth factor.
96
What relationship is there between solitary fibrous tumors of the pleura and asbestos exposure?
There is none.
97
Dose of methylene blue in methemoglobinemia?
1 to 2 mg/kg of body weight over 5 minutes
98
Predisposing factors for HAPE?
agenesis of pulmonary artery; patent foramen ovale; prior episodes of HAPE; human leukocyte antigen-DR6 phenotype
99
What meds may help prevent HAPE?
albuterol, nifedipine
100
aspergillus morphology
fungal hyphae of uniform diameter showing acute angle branching and septae
101
Why did they switch the propellant in MDI's?
CFC's were changed because chlorine catabolizes the stratospheric ozone. They now uses hydrofluoroalkanes. The new propellants made the inhalers 'branded' again and more expensive.
102
Most common two proteins in amyloidosis and their derivation? Difference in clinical course?
AL - primary amyloid (monoclonal, associated with MM and plasma cell dyscrasias); AA - secondary amyloid (chronic inflammatory disorders); AL is more aggressive
103
Discuss pulmonary involvement in amyloidosis.
With AA (secondary): low. With primary (AL): common, over 85%. Can be isolated to lungs or part of systemic disease. Endobronchial amyloid tumors, pulmonary nodules, diffuse interstitial or alveolar-septal amyloid infiltration (most common). Can have severe pulmonary hypertension.
104
Treatment options for amyloidosis?
AA amyloid: only colchicine has slowed protein formation. AL: increased survival with melphalan and prednisone. 4-iodo-4-deoxydoxorubicin had a positive trial.
105
Discuss the two forms of central sleep apnea.
Hypercapnic: high waking PaCO2, diminished response to CO2, due to neuromuscular diseases and diminished chemoresponsiveness. Nonhypercapnic: normal or low waking PaCO2, increased ventilatory response to CO2; idiopathic CSA, central apnea secondary to CHF.
106
Do patients with CSA due to CHF benefit from PAP?
Yes. Some may require mechanical ventilation during sleep.
107
Three causes of melanoptysis:
crack lung, coal miners pneumoconiosis, silicosis with submassive fibrosis
108
Of patients diagnosed with CTEPH, what percentage have positive LE duplex studies?
Only 35-40%. Lots of in situ thrombosis?
109
Bottom line on clopidogrel in transbronchial biopsies? ASA?
High bleeding risk (93% vs 3.4% controls). No difference with ASA.
110
Society guidelines on bronchoscopy after MI?
Wait 6 weeks. Not based on any data.
111
What chemo agents have a slight mortality benefit in mesothelioma?
pemetrexed with cisplatin has a small (3-month) survival advantage when compared to cisplatin (12 vs 9 mos).
112
What are amphiboles? What are they associated with? Which is the most potent?
fibers that are straight, long and needle-shaped (cocidolite, amosite, anthophyllite, tremolite and actinolit); strongly associated with mesothelioma; crocidolite (maily mined in South Africa and Western Australia) is the most potent inducer of mesothelioma
113
Alternative treatment options for nocardia?
1) TMP-SMX; alternatives: moxifloxacin, amikacin plus a beta-lactam; linezolid as substitute for amikacin or beta-lactam
114
Recommended treatment for mycobacterium kansasii:
rifampin, ethambutol and isoniazid for at least 18 months, and 12 months after negative culture results (REI in Kansas)
115
Discuss DPB.
Diffuse Panbronchiolitis. Described in Japan. Familial cases reported. More common in men. Only rarely seen in Asians abroad. Most are never-smokers, 85% have chronic sinusitis. Chronic relapsing bronchiolitis, progressive airflow limitation; 'pan' denotes spread through all layers of respiratory bronchiole and surrounding tissues. Sinus, then cough with purulent sputum, dyspnea, wheeze. Adults (30's - 60's). Positive cold agglutinins, negative mycoplasma titers. Responds to chronic daily macrolide therapy.
116
Indications for transplant: COPD
BODE over 7, FEV1 under 20, DLCO under 20, PaCO2 over 50, cor pulmonale, O2-dependent hypercapnic patients
117
Indications for transplant: IPF
DLCO under 39, decrease of FVC of greater than 10% in 6 months of followup, decrease of pulse oximetry below 88% on 6-min walk, honeycombing
118
Indications for transplant: CF
FEV1 under 30%, PaCO2 over 50, PaO2 under 55 on RA, young female patient, pHTN
119
Indications for transplant: PPH
NYHA III-IV despite vasodilator treatment, including epoprostenol agent; CI under 2; RA pressures greater than 15; low (under 350 M) or declining 6-min walk
120
In a patient on APRV with a set inspiratory pressure of 25 cm H2O and spontaneous inspiratory pressures of -10 cm H2O, what is the total end inspiratory transpulmonary pressure?
35 cm H2O
121
How is exhaled NO potentially useful in pulmonary diagnostics?
increased in uncontrolled asthma and some forms of bronchiectasis, normal in cystic fibrosis; in PCD, abnormally low
122
Important theophylline/antibiotic interaction?
If starting a macrolide, decrease theophylline dose by half.
123
What medications decrease theophylline clearance?
macrolides, quinolones, cimetidine, allopurinol
124
Up to \_\_% of patients with DIP are non-smokers.
20%
125
Differences between CIDP and GBS?
CIDP is more chronic, months to years. No infectious prodrome or clear association with immunization. Much worse prognosis
126
What GI med has been associated with eosinophilic pneumonia?
mesalamine
127
Top two causes of empyema necessitans today?
1) TB; 2) actinomycosis
128
Treatment of choice for actinomycosis?
penicillin
129
Discuss Erdheim-Chester disease.
AKA non-langerhans cell histiocytosis. Rare, noninherited. Tissue infiltration with large foamy histiocytes, surrounded by variable fibrosis. Negative S-100 labeling. No Birbeck granules. Often starts with bone involvement: symmetric osteosclerosis of long bones. Heterogeneous speckled appearance on plain films. Reticular infiltrates, variable fibrosis, pleural involvement and thickening. "Coated aorta" on imaging. Treatment unclear, steroids vs IFN-alpha.
130
Coated aorta ?
Erdheim-Chester (non-langerhans cell histiocytosis)
131
Discuss treatment of RLS.
1) carbidopa-levodopa; leads to augmentation, worsening early in day after evening dose w/ spread to UE's; 2) dopamine agonists (less augmentation than levodopa); 3) benzos (very effective, block augmentation); 3) iron supplementation if ferritin is less than 45
132
Most specific finding for VCD?
inspiratory and expiratory vocal cord adduction with a posterior glottic chink; can also occur with just inspiratory phase
133
Discuss vocal cord adduction during expiration.
Seen when healthy volunteers undergo histamine bronchoprovocation challenges and in patients with severe airflow obstruction and during mid-exhalation in asthmatics with experimentally-induced bronchosconstriction. This latter effect was reversed with CPAP, suggesting it's a way of unloading inspiratory muscle activity.
134
Points on CEP: 1) smoking? 2) M:F 3) frequency of relapse?
1) 90% among nonsmokers; 2) female twice as common as male (unlike AEP); 3) relapse common, may need chronic steroids
135
A negative d-dimer in a patient with a ______ pre-test probability for PE is enough to exclude the diagnosis.
low to moderate
136
According to ACCP, a "small" pneumothorax is less than...
3 cm
137
Two forms of pulmonary calcification:
metastatic calcification (calcium deposits in previously normal lungs: hyperparathyroidism, malignancy, milk-alkali) and dystrophic calcification (TB, histo, sarcoid, s/p PCP, s/p varicella, silicosis, coal worker's)
138
In Lambert-Eaton, the antibodies are directed against...
the P/Q voltage gated presynaptic calcium channel receptor
139
Difference in potentiation between myesthenia gravis and Lambert-Eaton?
LE gets better with repetition, improves throughout day.
140
Recommendations for severe malaria in low transmission areas or outside endemic areas?
1) artesunate (not available); 2) quinidine. Chloroquine is ok for prophylaxis but inadequate for treatment of severe malaria.
141
Diet modification that can help chylothorax?
Low-fat with medium chain triglycerides (absorbed directly into blood, not via thoracic duct)
142
What is the most determinant factor in prognosis of thymomas?
invasiveness (not histology or cytology)
143
Treatment of thymomas?
All get resected. All but completely encapsulated Stage I tumors should get postoperative adjuvant radiation therapy.
144
Clinical significance of myasthenia gravis in thymomas?
May actually have survival advantage (catch more early stage tumors). Other associated autoimmune pathologies (polymyositis, AHA) portent worse survival.
145
Which variants of asthma all have latency periods?
All immune-mediated variants. But irritant nonimmune-mediated forms (like RADS) do not have latency periods.
146
What is hypocretin-1?
neurotransmitter associated with wakefulness; CNS deficiency is associated with wakefulness
147
General strategy for treating portopulmonary hypertension?
Rarely responsive to vasodilators; calcium channel blockers are rarely used. Bosentan causes liver toxicity, generally avoided. OLT may improve hemodynamics, but patients with sPAP greater than 60 generally do poorly. Epoprostenol is a good choice, as is sildenafil.
148
What is now acceptable on commercial flights for oxygen-dependent patients?
They can bring their POC's on board with them if they notify the terminal counter and keep it under the seat in front of them.
149
What malignancy population generally has a contraindication to anticoagulation, and an indication for IVC filter?
metastatic renal cell, melanoma and thyroid cancer with mets to brain
150
How does CNS involvement change the management of cryptococcal pneumonia?
Normal LP: 6 weeks of oral fluconazole; CNS involvement: 2 weeks of amphotericin before being switched to 6 weeks of oral fluconazole
151
What patients are most vulnerable to cavitation of VTE's?
patients with conditions that impair the dual circulation of the lung: COPD, LV dysfunction
152
Which population with a risk factor for progressing from latent to active TB has the highest progression rate?
HIV
153
List the populations in the under 5mm (highest risk) group for LTBI PPD interpretation.
HIV positive, recent contacts of active TB patients, fibrotic changes on radiograph consistent with prior TB, organ transplant recipients, other immunosuppressed patients (at least 15 mg/d prednisone for at least one month)
154
Pearls on DAH in SCT:
1) 5-10% of SCT recipients; 2) rates are identical for allogenic and autologous transplants; 3) risk factors: pretransplant intensive chemo, total body irradiation, thoracic irradiation, advanced age (not thrombocytopenia); 4) hemoptysis is uncommon; 5) occurs within 30 days of engraftment; 6) high dose (pulse) steroids, though no RCT's
155
Vent intervention when someone is hypoperfusing from abdominal compartment syndrome?
Decrease tidal volume as much as pH allows.
156
PFT's: decreased VC and FEV1 with a normal ratio, reduced TLC, normal-to-increased RV strongly suggests:
neuromuscular disease
157
DDx for malignant causes of SVC syndrome:
lung cancer (67%), mediastinal tumors (20%), metastatic solid tumors (5%); small cell is most common lung cancer cause
158
What modalities of NPPV have been used in acute cardiogenic pulmonary edema?
both CPAP and BPAP have been shown to be effective
159
What tumors are most likely to metastasize to lung?
melanomas, GU, colorectal, breast, head and neck, soft-tissue and osteogeneic sarcomas, germ cell
160
Immunohistochemical marker: TTF-1:
thyroid transcription factor-1, neoplastic epithelial cells of both lungs and thyroid; very specific for lung cancer, more common in adenocarcinomas than squamous cell
161
Immunohistochemical marker: CK-20:
cytokeratin 20, low molecular weight cytokeratin, specific to adenocarcinomas of GI tract and transitional cell carcinomas of urinary tract
162
Immunohistochemical marker: CK-7:
cytokeratin 7, adenocarcinomas of lung and breast and occasionally in tumors of ovary or endometrium
163
Immunohistochemical marker: vimentin and S-100
have affinity for mesenchymal cells, so specific for melanoma and sarcoma
164
Tumor marker pattern: TTF-1 positive, CK-7 positive, CK-20 negative
primary adenocarcinoma of the lung
165
Tumor marker pattern: TTF-1 negative, CK-20 positive and CK-7 negative
GI (probably colorectal) in 80% of cases
166
Stop TB meds if LFT's:; how to rechallenge?
transaminases rise more than 5x ULN, or 3x ULN with jaundice or hepatitis symptoms; restart rifampin, then after 3 to 7 days restart INH if ALT is less than twice ULN
167
Optimal treatment for benign subglottic stenosis?
neodymium:yttrium-aluminum-garnet (Nd:YAG) laser therapy vs CO2 laser vs electrocautery
168
What can bronchoscopy diagnose in chronic cough with normal imaging?
endobronchial lesions (masses, lipomas), broncholithiasis, tracheobronchopathia osteochondroplastica, endobronchial TB, central carcinoid
169
What causes primary effusion lymphoma? What is its prognosis?
HHV-8, generally in patients with HIV. Prognosis is worse than other lymphomas.
170
Which pulmonary AVM's can be coiled?
Over 3 cm. Probably not present in hepatopulmonary syndrome (probably HHT or idiopathic).
171
What size lesions are strongly associated with PTX risk in transthoracic needle biopsy?
Under or equal to 2 cm. Oddly, # of needle passes and size needle aren't correlated.
172
What is the relationship between IFN and sarcoid?
Well-described relationship between the two: IFN has worsened preexisting sarcoid, provoked new onset sarcoid-syndrome. Causes CD4 cells to differentiate into Th1 cells? Only IFN-alpha.
173
Discuss OHSS.
Ovarian hypersensitization syndrome. Hormonal stimulation of ovaries in preparation for fertalization procedures. Follicular cysts, abdominal discomfort. Fluid accumulations: ascites, pleural effusions, pericardial effusions. Pleural effusions: exudative, right-sided.
174
Discuss post-CABG pleural effusion.
Small, L-sided: 85% of patients. Related to PCIS (post-cardiac injury syndrome, Dressler). Effusion is mildly hemorrhagic, initially neutrophilic and then lymphocytic. Often resolve spontaneously, but can require NSAID's or corticosteroids.
175
Discuss nasogastric tube syndrome.
1) throat pain; 2) hoarseness; 3) bilateral vocal cord paralysis due to NGT irritation. ~50% of patients with it need tracheostomy.
176
Five factors that increase perioperative pulmonary risk?
1) Age; 2) ASA class II or greater; 3) functional dependence; 4) COPD; 5) CHF. No convincing evidence regarding asthma, obesity, cigarette use, OSA.
177
For DAH refractory to steroids and cyclophosphamide...
recombinant factor VIIa
178
Flow-volume loop that has initially high peak followed by rapid decrease to a plateau of flow followed by rapid decline in flow to end of breath implies...
unilateral lung transplant for COPD. Development of a collapsible 'choke point' in compliant native bronchus just proximal to anastamosis site, coupled with slow flow from the native lung
179
What is the only outcome in CF for which chest physiotherapy has had demonstrated benefit?
volume of sputum production
180
What exactly are ANCA antibodies?
IgG autoantibodies against granules of neutrophils and lysosomes; most important are against proteinase 3 and myeloperoxidase
181
What are the four types of pulmonary parenchymal calcification?
1) nodular calcification (multiple calcified foci, often in healed disseminated infections - TB, histo, varicella; also talkosis and silicosis, mitral stenosis; 2) tiny punctate calcifications - alveolar microlithiasis; 3) dendriform pulmonary ossification (rare, branching and tubular foci of bone in lower lobes, old people at autopsy); 4) interstitial (metastatic) calcificatin - CKD, hypercalcemia, poorly defined nodular opacities
182
First-line therapy for patients with severe kyphoscoliosis and chronic respiratory failure?
NIV at night; better outcomes than O2 alone
183
Discuss RBD.
Rapid eye movement sleep behavior disorder. Abnormal behaviors develop during REM sleep. Loss of REM-related muscle atonia, hypotonia. Disruption of pathways responsible for tone suppression. Diagnose with PSG. Or can try brief period with clonazepam. More often in second half of sleep (more REM). End with rapid awakening, alertness, good dream recall. Can be associated w/ Parkinsons, Lewy body dementia, multiple system atrophy. Tx is clonazepam.
184
DDx for GPR's (7):
rhodococcus (formerly corynebacterium equi, can be coccobacilli), corynebacterium (dipthroids), lactobacilli, bacillus, listeria, clostridium, erysipelothrix
185
Discuss rhodococcal infections. Presentation, micro, treatment.
Predominantly pulmonary in immunocompromised hosts. Often first identified as diptheroids. Consolidation, cavitation. Two-drug treatment: vanc plus macrolide, or imipenem plus aminoglycoside or rifampin. Treat for at least two months.
186
Ideal candidate for outpatient management of new PE?
young, female, normal vital signs, no chronic medical conditions
187
Recurrent pneumonia and a monoclonal IgG spike?
Probably myeloma or CLL. Overproduction of one immunoglobulin results in underproduction of others ? recurrent infection by encapsulated organisms.
188
Malignancy association in dermatomyositis/polymyositis?
Strong association with adenocarcinoma of the lung or GI tract. Up to 15% of DM/PM cases have malignancy.
189
What is the antibody association in DM/PM-ILD?
Strong association with anti-Jo-1, anti-PL 7 and anti-PL 12 antibodies.
190
Describe 'auto-triggering' on a ventilator.
Rhythmic frequent (often greater than 1/second) breaths that is caused by a drop in airway pressure (e.g. a leak in the circuit) below the threshold for initiating a breath. Can be caused by too low trigger sensitivity, a leak in the circuit, water in the circuit, even cardiogenic oscillations.
191
Describe 'flow asynchrony.'
The ventilator flow does not match the patient flow, and is recognized by a 'scooped' contour to the inspiratory phase of the pressure-time curve. This is corrected by increasing ventilator flow delivery to meet the patient demand.
192
When does radiation pneumonitis typically occur?
Within 3 months of treatment. Late changes of fibrosis typically arise 6-9 months after treatment and are stable by 2 years.
193
Does osteoporosis occur more with heparin or LMWH?
heparin (unfractionated)
194
Discuss anterior mediastinal lipomatosis.
Benign condition of overabundant histologically normal unencapsulated fat within the mediastinum. Diagnosed radiographically, no treatment. Associated with obesity, glucocorticoid treatment, Cushing's syndrome.
195
Discuss Swyer-James syndrome.
Unilateral hyperlucent lung. Secondary to childhood airway infection. Bronchiolitis prevents normal development of peripheral airways and leads to reduced pulmonary perfusion accounting for decreased left-sided pulmonary vasculature, hyperlucency.
196
PFT pattern in RB-ILD?
Mild restriction, unless there's some obstruction from concurrent emphysema.
197
Hemoptysis in a patient who has undergone ablation for atrial tachyarrhyhmias?
Pulmonary vein stenosis. Up to 15% in one series. 2-6 months after procedure with cough, hemoptysis, DOE, pleuritic chest pain. Easy to miss on CTA.
198
Discuss Charcot-Leyden crystals and NAEB.
Bipyramidal, seen in asthma and nonasthmatic eosinophilic bronchitis. Rather than mast cells invading the airway smooth muscle (as in asthma), they invade the epithelium. Out-of-the US diagnosis (13-33% of chronic cough overseas). Responds well to inhaled corticosteroids.
199
What lung disease has 20% recurrence after transplant?
Langerhans cell histiocytosis
