Random Resp

Question 1

What is a vascular ring?

Answer 1

It is a congenital abnormality in which the aorta forms a double arch which forms a ring around the trachea and oesophagus.

This can cause problems with breathing and rarely digestive symptoms.

Note may be asymptomatic

Question 2

How may a vascular ring present?

Answer 2

Respiratory distress
High-pitched cough
Stridor
Repeated LRTI
Wheezing

Question 3

What is Congenital Cystic Adenomatoid Malformation?

Answer 3

It is a rare abnormality of lung growth in which there is adenomatous overgrowth of the terminal bronchioles with subsequent reduction in alveolar growth.

Question 4

How may a congenital cystic adenomatoid malformation (CCAM) present?

Answer 4

Often picked up in antenatal USS screening.

Presents with respiratory distress in the newborn (ranging from grunting to fulminant respiratory failure)
May develop spontaneous pneumothorces

Also causes recurrent respiratory infection.

Question 5

What are some of the associations of CCAM?

Answer 5

Polyhydraminos
Fetal hydrops

Renal Abnormalities

Question 6

What is primary ciliary dyskinesia?

Answer 6

It is an autosomal recessive condition characterised by:

• Neonatal respiratory distress
• Chronic rhinorrhoea
• Recurrent infection causing bronchiectasis
• Male infertility

As well as Situs invertus (organs on opposite side)

It is also called Kartagener’s syndrome

Also associated with conductive deafness.

Question 7

What is bronchiolitis obliterans?

Answer 7

An extended version of bronchiolitis which can last for several months and results in fibrosis of the lung tissue and permanent damage.

Commonly caused by adenovirus.

Question 8

What is idiopathic juvenile dermatomyositis and what are the classical symptoms?

Answer 8

It is an idiopathic inflammatory myopathy

Classical symptoms:

• Red heliotrope rash affecting the face, eyelids, hands, and sometimes the skin above joints.
• Muscle weakness

Question 9

What is tracheobronchomalacia?

Answer 9

Tracheobronchomalacia (TBM) is a rare condition that occurs when the walls of the airway (specifically the trachea and bronchi) are weak.

Question 10

How does tracheobronchomalacia present?

Answer 10

Can  cause:
SOB 
Cough
Wheeze 
Stridor
Sputum retention

Persistent wheeze which worsens after administration of bronchodilators. (bronchodilators further relaxes the muscle)

Question 11

What is SCID?

Answer 11

Severe combined immunodeficiency.

It is an x linked recessive disorder in which there is a deficiency in B and T cells causing there to be recurrent infections.

Note: other forms of inheritance but X linked is the most common