Board Review 1 Flashcards
Mutation in MEFV gene implies… consequences and management?
diagnosis of familial Mediteranean fever; untreated –> inflammation, amyloidosis, renal failure. Start colchicine, 0.6 mg po bid.
Pulmonary presentation of FMF?
recurrent chest or abdominal pain lasting 24-72 hours, responds to colchicine
For patients with FMF who do not respond to colchicine, try…
etanercept, thalidomide, infliximab
Discuss nodular tumorous sarcoidosis.
Lower extremity involvement is common. Often multiple nodules. Often weakness and tenderness. Commonly with extramuscular sarcoid (LAD, cuteneous, etc.). Response to steroids is inconsistent.
Pulmonary complication of a double aortic arch?
Encircles the trachea, can cause compression. Most often associated with tracheomalacia.
What is napkin ring trachea?
incomplete separations of the cartilage rings; associated with anomalous origin of the left pulmonary artery
What is the lab diagnosis of carcinoid syndrome?
24-h urinary 5-hydroxyindoleacetic acid
Biopsy and management of endobronchial sarcoid?
Large series showed that endobronchial biopsy is safe despite high vascularity. Resection is primary management.
What are the only two agents that have improved cough in the common cold?
Dexbrompheniramine and naproxen.
What lung malignancy is the most common cause of SVC syndrome?
small cell lung cancer; up to 20% of patients with small cell develop SVC syndrome
Diabetes insipidus in a young person who smokes… think:
Langerhans cell histiocytosis
Most important treatment for LCH:
cessation of smoking
Bony involvement in LCH?
Common: 20% have bone involement, often flat bones (e.g. ribs)
What airway diseases are seen in IBD? (3)
follicular bronchiolitis: extensive lymphoid follicle formation in the respiratory bronchiolar walls; also organizing pneumonia and bronchiectasis
In patients with HIT, which agent do you use in which population?
argatroban (cleared hepatically) in patients with renal failure, lepirudin (cleared renally) in patients with liver injury
What is the word for applying a powedered slurry tot he pleural space?
poudrage
Discuss lupus pernio.
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
What is Lofgren syndrome? What is the prognosis?
EN, bilateral hilar adenopathy, arthralgias; remission is common
Of testicular cancers, which is the more aggressive?
Nonseminomas (embryonal cell carcinoma, choriocarcinoma, yolk sac tumor and teratoma) are more aggressive than seminomas.
Unusual presentation of choriocarcinoma?
Often metastasizes hematogenously, with the testicular primary burning out. Rarely originates in the lung and is associated with a DAH-like syndrome.
Standard chemotherapy for testicular cancer?
bleomycin, etoposide and cisplatin
What are the relevant SUV ranges in PET’s?
Malignant lesions have an SUV of 2.5 to 15.
How does diabetes complicate the use of PET?
May cause false negatives because of increased dilution of the radiolabeled tracer in nonlabeled serum glucose.
How are the modified Light criteria different from the orignal ones?
LDHp/LDHs greater than .45 ULN rather than .66.
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.
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.
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.
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).
What is the most common pulmonary manifestation of rheumatoid arthritis?
ILD (up to 58% of patients when using HRCT)
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.
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.
In whom is APAP (autotitrating PAP) contraindicated?
Patients with other reasons for desastting: CHF, COPD, daytime hypoxemia, etc..
Why is “superficial femoral vein” something of a misnomer?
It is part of the deep venous system of the thigh.
Duration of therapy in a pregnant woman with DVT?
Throughout pregnancy and at least 6 weeks postpartum to complete three months.
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.
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%.
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.
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.
Bottom line on PCP in patients without HIV:
more fulminant, though PCP burden is much lower
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.
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.
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
technical description of crazy paving:
GGO with a mesh-work of reticular lines representing thickened intralobular septa
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
How does hypertrophic osteoarthropathy affect lung cancer staging?
It doesn’t.
What is the most sensitive imaging modality for hypertrophic osteoarthropathy?
99mTc bone scan.
Which patients seemed to derive benefit from LVRS?
marked exercise impairment (under 25W) and upper lobe disease
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.
BAL findings in organizing pneumonia?
Similar to HP: lymphocytosis, decreased CD4/CD8.
Initial treatment of organizing pneumonia?
corticosteroids 0.75 - 1.5 mg/kg/day with slow taper over 3 to 12 months.
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
Discuss treatment of carcinoid tumors.
Treatment is surgical resection with systematic sampling of mediastinal and hilar nodes.
What is the most common source of ectopic ACTH production?
Pulmonary carcinoid tumors. They can cause Cushing syndrome years before they are detected radiographically.
What fraction of all carcinoid tumors are pulmonary?
25%. Most are in the gut.
What is the classification system for neuroendocrine tumors?
1) typical carcinoid, 2) atypical carcinoid, 3) large cell and small cell carcinomas
Medication options for thoracic endometriosis?
OCP’s, danazol, GRH analogues
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)
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
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.
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.
Three flavors of cyclophosphamide lung injury.
Acute pneumonitis, organizing pneumonia, or chronic fibrosis.
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.
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.
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.
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.
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
How useful is a serum anti-GBM IgG in diagnosing Goodpasture’s?
Diagnostic if positive, but only 60% sensitive.
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.
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.
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.
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.
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.
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.
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.
What PFT values predict high perioperative mortality and pulmonary disability in lung cancer resection?
FEV1 under 30%, FEV1 x DLCO under 1,650%.
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).
What is lymphangiectasis?
Dilation of existing lymphatic channels without an increase in their number or complexity.
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.
Acuity of presentation of lupus pneumonitis?
There is both an acute and a chronic presentation.
What happens to residual volume when there is severe neuromuscular weakness?
It gets increased.
