Random Flashcards

1
Q

How is EIB diagnosed?

A

spirometry showing a reduction in FEV1 after a suitable challenge test.

gold-standard is indirect bronchoprovocation

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2
Q

preventative measures in learning disabilities- Patients with NMD who have a VC <60% predicted- what should happen next?

A

overnight ‘breathing’ assessment

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3
Q

when do you use Mechanical insufflation–exsufflation?

A

treatment of choice in patients with NMD to enhance cough efficacy

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4
Q

Who needs yearly sleep studies in learning disability groups?

A

Downs - oximetry
prader willi- oximetry and capnography

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5
Q

What is the main cause of PAVMs?

A

HHT (hered haem teleng)
autosomal dom
HHT1: ENG encoding endoglin
HHT2: ACVRL1 encoding ALK-1
SMAD4

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6
Q

Which type of HHT is more likley to have PAVMs?

A

HHT1 and SMAD4

rare complications of juvenile polyposis and aortopathy are associated with SMAD4
incrase of PAH in HHT2

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7
Q

When do you get 2ary PAVMs?

A

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

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8
Q

in which patient group do you give prophylactic antibiotics for dental procedures?

A

PAVMs- highly associated with brain abscess formation
taken 1-2hr pre and a dose post

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9
Q

When do you give prophylactic abx in PAVMs?

A

dental procedures, endoscopy, surgery, embolisation, and obstetric delivery

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10
Q

which iron preparation should you use in PAVM patients?

A

Fe gluconate- lower amounts of elemental Fe- prevent overload (drive HHT complications/bleeding)

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11
Q

When do you see PAH in PAVM?

A

rarely caused by PAVMs- normally caused by hepatic AVMs due to high pulmonary blood flow

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12
Q

What are Curaçao criteria?

A

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

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13
Q

What is T2MR blood test?

A

look for invasive fungal disease (candida)

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14
Q

What is galactomannan?

A

Specific to aspergillus

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15
Q

What is beta d glycan test?

A

Funghi

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16
Q

What is a positive skin test?

A

> 3mm, assuming control is negative

17
Q
A
18
Q

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?

A

pulmonary Langerhans cell histocytosis

19
Q

What are the features of LCH on HRCT?

A

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

20
Q

What is the histology of LCH?

A

loose granulomas CD1a and largerin pos cells with eosinophilic cyto

associated: hyperpigmented macro, emphysema

21
Q

Which mutations (that are also targetable) are featured in LCH?

A

MAPK/ERK
MAP2K1- 80% patients
BRAF- can use vemutafenib

22
Q

What is the lung cancer risk in LCH?

A

x17 excess of lung cancer in LCH compared to non LCH pop

23
Q

What is the inheriteance pattern of BHD?

A

auto dom with equal sex distribution

24
Q

What are the non pulmonary features of BHD?

Lung features?

A

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

25
Q

how should BHD be managed?

A

prevention of recurrent PTX (consider pleurodesis after initial episode)
genetic counselling
annual/biannual PFTs- FEV1 FVC and DLCO
life long renal surveillance (MRI)