Flippin’ ‘eck… What happen to FFFF last week?
Your guess is as good as ours…
Not to worry, FFFF is back with a fwack….
Q1. Patients with which disorder, with a very strong HLA association, should not be startled during dinner time?
- Narcolepsy with cataplexy — Over 90% of patients with narcolepsy-cataplexy carry HLA-DQB1*0602
[see Stanford Center for Narcolepsy FAQ].
- HLA stands for Human Leukocyte Antigen and refers to group of genes on chromosome 6 primarily involved in immune function (known as the major histocompatibility complex (MHC) in non-human species).
- Narcolepsy is a disorder categorised by excessive daytime sleepiness, despite adequate night-time sleep. It may be associated with cataplexy — a sudden loss of muscular tone that may lead to paroxysmal collapse, often brought on by excitement…. As this poodle demonstrates:
Q2. What previously incurable condition was successfully treated in 1926 by eating 1/2 a pound of raw liver daily?
- Pernicious anemia — this easily treatable condition was once as dreaded as leukemia. Pernicious anemia refers to intrinsic factor deficiency, resulting from autoimmune-mediated atrophic gastritis with loss of the intrinsic factor secreting parietal cells. Intrinsic factor is required for the absorption of vitamin B12 in the terminal ileum. Vitamin B12 deficiency may have many manifestations including glossitis, megaloblastic anemia and neurological consequences such as dementia and subacute combined degeneration of the spinal cord.
- Pernicious anemia was first described in 1849 by Thomas Addison, and was originally known as Addison’s anemia. A somewhat unsustainable treatment was discovered by William Bosworth Castle when he fed regurgitated hamburger meat to affected patients. The control patients, who received non-regurgitated hamburger meat, did not respond to treatment. This work suggested the existence of ‘intrinsic factor’ in gastric juice.
- George Whipple suggested raw liver as a treatment in 1920. George Minot and William Murphy subsequently showed that it was raw liver juice that mediated the cure. These three men shared the 1934 Nobel Prize in Medicine.
- It was not until 1928 that Karl A. Folkers and Alexander R. Todd identifiedvitamin B12 in ‘liver juice’ as the so-called ‘extrinsic factor’.
Q3. A resident presented a patient with a past history of abdomino-perineal resection to his consultant. Among his examination findings he stated ‘PR NAD’. The consultant was mystified… what did the resident mean by NAD?
- “No anus detected!”
[From Montgomery H, et al, My First MRCP Book, 2003]
- Be careful how you use your TLAs!… and your FFLAs too for that matter.
Q4. How did Q fever get its name?
- Q for ‘query‘, as in ? fever.
- In 1937, Edward Holbrook Derrick first described Q fever after investigating a 1935 outbreak of a febrile illness among abattoir workers in Brisbane, Australia. The illness was named Q fever because the etiology of the new malady was so elusive, and to avoid negative connotations associated with the cattle industry or Queensland…
- Frank Macfarlane Burnet and Mavis Freeman isolated the organism in blood samples from one of Derrick’s patients, and identified it as a Rickettsial species in 1937. Although primarily disseminated as an aerosol via inhalation or ingestion, Herald Rea Cox and Gordon Davis identified vector transmission when the organism was isolated from ticks at Nine Mile Creek in Montana in 1938. As a result of this discovery, the causative organism subsequently became known as Coxiella burnetii.
- Now that that mystery is solved, we have Post Q Fever Fatigue Syndrome to think about….
Q5. You are treating a Peruvian pig farmer who has just has a seizure. What is the likely cause?
- Cysticercosis is a parasitic infection that results from ingestion of eggs from the adult tapeworm, Taenia solium. When cysticercosis involves the central nervous system, it is called neurocysticercosis.
- Neurocysticercosis is the most common parasitic infection of the brain and a leading cause of epilepsy in the developing world, especially Latin America, India, Africa, and China.
A Final Parting Piece of Funtabulous Wisdom…
The LITFL team aren’t convinced that this is quite what Peter Safar had in mind with the 9th of his Laws for the Navigation of Life:
“Law 9. If you can’t win, change the rules.”