Table 4.

Organ-specific consensus recommendations.

Recommendations		Strength
Rec. 18	A formal dermatologic assessment should be considered at diagnosis.	strong
Rec. 19	Surgical intervention should usually be performed when the largest renal tumour reaches 3 cm in diameter.	strong
Rec. 20	Nephron-sparing surgery should ideally be performed whenever possible, with percutaneous thermal ablation being an alternative.	strong
Rec. 21	Routine Lung Function Testing is not usually required in the follow-up of asymptomatic patients with BHD syndrome.	moderate
Rec. 22	Risk of pneumothoraces in flying/diving should be assessed and counselled on an individual basis with specific advice from respiratory medicine based on results of high-resolution computed tomography and previous history of pneumothoraces.	strong
Rec. 23	Flights on commercial airlines are generally safe but for activities that may pose a risk for pneumothorax, such as working as a pilot, flying in unpressurised aircraft or diving, expert advice should be sought so that individuals can be advised appropriately.	strong
Rec. 24	Surgical interventions should be considered for the treatment of recurrent pneumothorax.	strong
Rec. 25	Ablative procedures (e.g. electrosurgery, laser therapy) to manage fibrofolliculomas and trichodiscomas (especially facial) should be considered and discussed in patients requesting intervention, particularly if a patient states their skin lesions are affecting their quality of life.	strong