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This was part of RSNA 2017 virtual meeting. I am writing down the salient points from this excellent lecture:
Congenital immunodeficiency syndromes:
Chronic Granulomatous Disease:
- Inability to kill catalase positive organisms like staphylococcus and aspergillus.
- Early manifestations: Miliary nodules, abscess, pneumonia, empyema.
- Late manifestations: bronchiectasis and pulmonary fibrosis.
Leukocyte adhesion deficiency type I.
- Presents in infants as bacterial infection.
- Affects mucosa and skin.
- No pus formation.
- May present with infections similar that of CGD.
- Haemolytic anaemia on consumption of fava beans or medications.
- Typically occurs in patients with diabetes.
- Most common infection is Candida.
- Paediatric chest x-ray showing cardiomegaly.
- The most common primary cardiac tumour in children.
- It is associated with the ventricular myocardium and appears homogeneously hyperintense on T2-weighted images.
- 50 to 70% are associated with tuberous sclerosis.
- Spontaneous regression is common.
- Tuberous sclerosis can have pulmonary manifestations in the form of
Case courtesy of Dr Effendi Mansoor, <a href=”https://radiopaedia.org/”>Radiopaedia.org</a>. From the case <a href=”https://radiopaedia.org/cases/17302″>rID: 17302</a>
- 2nd MC
- Free wall of ventricle
- Associated with Gorlin Syndrome.
- 3rd MC
- Pedunculated. Interatrial septum.
- Asso with Carney complex: Hyperpigmentation and endocrine overactivity
- Within pericardium
- Pericardial effusion.
- Heterogenous with fat/calcification.
Sickle Cell disease:
- Soft tissue swelling.
- Periosteal reaction.
- Cortical disruption.
- Spontaneous improvement.
- New opacity.
- Increased pulmonary vasculature.
Langerhans Cell Histiocytosis:
- Early: Nodules with small cysts. Spontaenous pneumothorax.
- Advanced: Large cysts with pulmonary fibrosis.
- Can involve bones as well.
- Trachea: Subglottic stenosis. Image from RSNA article: Wegners uncommon and common manifestations.
- Pulmonary nodules with cavitation.
Part 2 here: