Random Flashcards
How is EIB diagnosed?
spirometry showing a reduction in FEV1 after a suitable challenge test.
gold-standard is indirect bronchoprovocation
preventative measures in learning disabilities- Patients with NMD who have a VC <60% predicted- what should happen next?
overnight ‘breathing’ assessment
when do you use Mechanical insufflation–exsufflation?
treatment of choice in patients with NMD to enhance cough efficacy
Who needs yearly sleep studies in learning disability groups?
Downs - oximetry
prader willi- oximetry and capnography
What is the main cause of PAVMs?
HHT (hered haem teleng)
autosomal dom
HHT1: ENG encoding endoglin
HHT2: ACVRL1 encoding ALK-1
SMAD4
Which type of HHT is more likley to have PAVMs?
HHT1 and SMAD4
rare complications of juvenile polyposis and aortopathy are associated with SMAD4
incrase of PAH in HHT2
When do you get 2ary PAVMs?
following surgical treatment of complex cyanotic congenital heart disease
if a lung receives no or minimal hepatic venous return
rarely as part of other inherited vasculopathies
in which patient group do you give prophylactic antibiotics for dental procedures?
PAVMs- highly associated with brain abscess formation
taken 1-2hr pre and a dose post
When do you give prophylactic abx in PAVMs?
dental procedures, endoscopy, surgery, embolisation, and obstetric delivery
which iron preparation should you use in PAVM patients?
Fe gluconate- lower amounts of elemental Fe- prevent overload (drive HHT complications/bleeding)
When do you see PAH in PAVM?
rarely caused by PAVMs- normally caused by hepatic AVMs due to high pulmonary blood flow
What are Curaçao criteria?
HHT diagnosis (need 3/4):
(1) spontaneous recurrent epistaxis
(2) mucocutaneous telangiectasia at characteristic sites
(3) a visceral manifestation (AVMs/gastric telangiectasia
(4) an affected first-degree relative
What is T2MR blood test?
look for invasive fungal disease (candida)
What is galactomannan?
Specific to aspergillus
What is beta d glycan test?
Funghi
What is a positive skin test?
> 3mm, assuming control is negative
30yr old male with a 30 pk year Hx presents with cough and sweats. HRCT shows nodules some of which have cavitated to form irregular cysts in the upper lobes.
What is the diagnosis?
pulmonary Langerhans cell histocytosis
What are the features of LCH on HRCT?
upper lobe predominance
nodules which cavitate to forn irrgular cysts.
active disease- thicker walled cysts, later the cysts become thin walled
GGO due to co-existent DIP/RBILD
What is the histology of LCH?
loose granulomas CD1a and largerin pos cells with eosinophilic cyto
associated: hyperpigmented macro, emphysema
Which mutations (that are also targetable) are featured in LCH?
MAPK/ERK
MAP2K1- 80% patients
BRAF- can use vemutafenib
What is the lung cancer risk in LCH?
x17 excess of lung cancer in LCH compared to non LCH pop
What is the inheriteance pattern of BHD?
auto dom with equal sex distribution
What are the non pulmonary features of BHD?
Lung features?
skin hamartomas/fibrofolliculomas
increased risk of renal ca
Lung cysts in 70-80%
- thin walled
- cigar or lentiform or round
- pre-dom in basilar/medial regions often against mediastinal surfaces
how should BHD be managed?
prevention of recurrent PTX (consider pleurodesis after initial episode)
genetic counselling
annual/biannual PFTs- FEV1 FVC and DLCO
life long renal surveillance (MRI)
