Often, the college asks the candidates to discuss diseases with a significant community prevalence which have some sort of serious impact on the ICU management of affected patients. it makes sense to expect final-stage CICM trainees to be able to discuss SLE or scleorderma, and the attention directed at these diseases is understandable.
On the other hand, there are occasionally questions in the Fellowship Exam which cannot be explained by this framework. There is a group of rare diseases which have only ever appeared once and which can reasonably be expected to never appear again. These are grouped here in no particular order, basically because there is nowehere else to put them.
Question 30.1 from the first paper of 2017 asked for six clinical features of myotonic dystrophy, a congenital disorder which results in an abnormal skeletal muscle myosin kinase protein. The college answer lists 11 features, but in fact there may be many more. An excellent review by Turner et al (2010) adds a few extra features. Remixed with the college answer, the full list looks like this:
Neuromuscular phenomena
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Other features
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What ICU relevance might we wring from this? Well. These people do end up in ICU a lot. Not the least of their presentations are from theatre recovery, as they are referred to the ICU by concerned anaesthetists as elective post-operative admissions. An excellent (though somewhat dated) article by Mudge et al (1980) discusses some of these concerns, which remain relevant in the contemporary anaesthetic setting.
In short, the following anaesthetic problems usually arise, which are worth knowing about
This also came up in Question 30.1 from the first paper of 2017, where the college wanted candidates to identify acromegaly from Wikipedia's copyright-free images (originally from Chanson et al, 2008). Additionally, some of the clinical features were asked for (specifically, biochemical abnormalities).
Pituitary tumour effect |
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Skeletal effects |
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Muscular effects |
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Cutaenous manifestations |
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Airway consequences |
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Respiratory effects |
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Cardiovascular effects |
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Electrolyte, endocrine and metabolic abnormalities |
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Renal problems |
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Haematological problems |
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This has come up in Question 2 from the first paper of 2019. The examiners asked for characteristic plain Xray findings in the C-spine series, which is baffling because nobody has ordered one since 2008. However, in case they ask it again, here they are, from Radiopedia and Østergaard (2012):
This appeared in Question 14.4 from the second paper of 2019, as a tiny shred of a question worth 25% of the marks (i.e. 2.5 marks out of the total 300 for the paper). The college wanted to know what problems might arise in a patient who has severe Parkinson’s disease and who must miss out on their medications because of bowel rest. There are a couple of excellent articles which appears almost designed to answer this sort of question (Freeman et al, 2007, and Katus et al, 2014). The contents of these was distilled into what follows:
Problems of routine housekeeping:
Question 22 from the second paper of 2020 asked for the important features of Down syndrome which could impact on the management of a six-year-old boy. There's an article by Donoso from 2017 which seems like it was tailor-made for this SAQ, but it is in Spanish. Still, it contained gold. Armed with nothing but highschool-level Spanish and Google Translate, the author embarked on an expedition to extract its wisdom:
Airway features:
Respiratory function:
Cardiovascular features:
Neurological features:
Endocrine associations
Nutritional issues
Immune features
Mudge, Barbara J., Peter B. Taylor, and Abraham FL Vanderspek. "Perioperative hazards in myotonic dystrophy." Anaesthesia 35.5 (1980): 492-495.
Turner, Chris, and David Hilton-Jones. "The myotonic dystrophies: diagnosis and management." Journal of Neurology, Neurosurgery & Psychiatry 81.4 (2010): 358-367.
Philippe Chanson and Sylvie Salenave - Acromegaly. Orphanet Journal of Rare Diseases 2008, 3:17. doi:10.1186/1750-1172-3-17
Molitch, M. E. "Clinical manifestations of acromegaly." Endocrinology and metabolism clinics of North America 21.3 (1992): 597-614.
Melmed, Shlomo. "Acromegaly." New England Journal of Medicine 355.24 (2006): 2558-2573.
Østergaard, Mikkel. "Imaging of ankylosing spondylitis." Arthritis Research & Therapy. Vol. 14. No. 2. BioMed Central, 2012.
Freeman, William D., et al. "ICU management of patients with Parkinson's disease or Parkinsonism." Current Anaesthesia & Critical Care 18.5-6 (2007): 227-236.
Vincken, Walter G., et al. "Involvement of upper-airway muscles in extrapyramidal disorders: a cause of airflow limitation." New England Journal of Medicine 311.7 (1984): 438-442.
Katus, Linn, and Alexander Shtilbans. "Perioperative management of patients with Parkinson's disease." The American journal of medicine 127.4 (2014): 275-280.
Donoso, F. A., et al. "Down Syndrome child in the Intensive care unit." Revista chilena de pediatria 88.5 (2017): 668-676.
Benhaourech, Sanaa, Abdenasser Drighil, and Ayoub El Hammiri. "Congenital heart disease and Down syndrome: various aspects of a confirmed association." Cardiovascular journal of Africa 27.5 (2016): 287-290.