Here’s a mnemonic and acronym for the USMLE I thought up earlier today for remembering the clinical features of Beckwith-Wiedemann Syndrome. First, do you remember WAGR Syndrome?
WAGR Syndrome includes:
Aniridia (absence of the iris)
Retardation (motor & mental)
How many letters are in WAGR Syndrome? There are just 12 letters, not including the space. So, due to the smaller number of letters we can remember that this involves the WT1 gene (on 11p13) and not the WT2 (11p15.5, assoc. with IGF-2). Whereas, Beckwith-Wiedemann Syndrome has 25 letters (that’s over 2x the number of letters) and therefore we can remember that this involves the WT2 gene (for 2x the number of letters).
Here’s the mnemonic: “Beck is with Wide WOMMEN.” The first part of the eponym sounds like the guitarist Jeff Beck, who is married to Sandra Cash. Let’s just imagine Beck taking a ‘selfie’ with a group of female fans that are “wide” hypothetically for the sake of passing examinations. If you can picture that in your mind, then you can picture this sentence:
“Beck is with Wide WOMEN.” This phonetically sounds very much like the eponym “Beckwith-Wiedemaan.” Now let’s use the phonetic analogue “WOMEN” as our mnemonic acronym:
Organomegaly (including “macroglossia” – enlargement of tongue)
Midline abdominal wall defects (omphalocele/exomphalos, umbilical hernia, diastasis recti)
Neonatal Hypoglycemia & Macrosomia
Remember that this will have a WT2 mutation (a tumor suppressor gene) and that there are 2 M’s in the mnemonic acronym.
WT1 gene is on 11p13 and the WT2 is on 11p15.5, assoc. with IGF-2. Both WT1 and WT2 are tumor suppressor genes, so they follow the Knudson’s two-hit hypothesis, require both of the 2 alleles to be knocked out by the mutation and acquire a loss of function [decreased protection/repair allowing for further mutations leading to cancer], and not oncogenes which require only 1 allele and acquire a gain of function [tumor growth from increased mutated gene expression].
Credit: First Aid for the USMLE Step 1 2014, Fundamentals of Pathology/Pathoma, and Wikipedia.