Thursday, October 30, 2014

Article Reviews (1996 and 2007) - Early summary, and AFib in Italy

Hello again, loyal and patient blog readers. It is time for another monthly post, and this one will include not one, but two article reviews. As a bonus, at the end of the post you will find the always exciting monthly blog stats.

First, a quick Mom update. There really is not much to update, other than the fact that she has not been hospitalized again and things are more or less stable. It turns out this month was a good month for her to stay out of the hospital because the hospital she was in during her September visit, which is also the hospital closest to her internist and gastroenterologist, is none other than Texas Health Presbyterian in Dallas. You may recall that is the hospital where Thomas Eric Duncan, the first person diagnosed with ebola in the US, was treated. In fact, his first visit to that hospital was the same day Mom was released, which was Thursday, September 25. So yes, for awhile we were wondering what we would do if Mom's bleeding issues came back and she needed to go to the hospital. It was also a little weird watching Anderson Cooper live on CNN broadcasting from in front of that hospital, knowing I could drive to that spot myself in about 20 minutes. Fortunately we did not have to make any hospital decisions and the local ebola drama seems to be fading away.

Today I am going out of sequence with the article reviews to catch up on some older ones I acquired after starting to review articles. Both of these reviews will be rather short.

Title: Five Kindreds With Variant Fibrinogen Presenting Renal Amyloidosis (1)

Authors: T. Uemichi, JJ Liepnieks, MT Hull, MA Gertz, MD Benson (Indiana University, Indianapolis, IN; RLR VA Medical Center, Indianapolis, IN; Mayo Clinic, Rochester, MN)

Journal: Neuromuscular Disorders (1996)

This article appeared in a supplement to the journal "Neuromuscular Disorders" and is just one paragraph (ten sentences.)  It briefly describes the three mutations known at that time to cause fibrinogen amyloidosis. Those mutations were:

  • Arg554Leu (one kindred with three affected members)
  • Glu526Val (three kindreds with a total of 11 affected members)
  • 4904DelG (one kindred with two affected members)

So that is where things stood in 1996, three years after the first article about a fibrinogen amyloidosis mutation was published. Three mutations, five kindreds, and 15 people known to be affected. Although the Glu526Val mutation was not the first one discovered, it did take the lead rather quickly. The next article will give us a sense of how prevalent AFib was in Italy about ten years later.

Title: Renal involvement in systemic amyloidosis  --  an Italian retrospective study on epidemiological and clinical data at diagnosis (2)

Authors: Bergesio, Ciciana, Santostefano, Brugnana, Manganaro, Paladini, Di Palma, Gallo, Tosi, Salvadori (Italy)

Journal: Nephrology Dialysis Transplantation (2007)

This is a much longer article (eight and a half pages, plus one page of references). But my review, which is actually just a summary of the part relevant to us AFibbers, will be shorter than the abstract. This article reports on a study of 373 patients diagnosed with some form of systemic amyloidosis (with renal involvement) in Italy between January 1995 and December 2000. Of those 373 patients, only six were diagnosed with a hereditary form of amyloidosis, and only one of those six patients had a fibrinogen mutation (Glu526Val). There really isn't enough data there to draw any conclusions regarding the prevalence of fibrinogen amyloidosis in Italy, other than to say it was rarely diagnosed before 2007. See, I told you that would be a short review.  ;-)

No, there was not much in this month's blog post but hopefully I can do some more cleanup of the article reviews and get those closer to completion in the next few months. The next blog post should be a report on the Dallas Support Group meeting in November.

onthly Blog Status Update===== 

As of September 30, 2014:

Total posts: 142 (1 in September)

Total pageviews: 20,100 (~1000 in September)

Email subscribers: 10 (no change)

Total number of countries that have viewed the blog: 101

1 new country viewed the blog in September:

British Indian Ocean Territory**

**The British Indian Ocean Territory is a group of very small islands in the Indian Ocean. However, since the two-letter country code is io (as in input/output), that code is often used by technology companies.


(1) Uemichi T, Liepnieks JJ, Hull MT, Gertz MA, Benson MD. Five kindreds with variant fibrinogen presenting renal amyloidosis. Neuromuscular Disorders. 1996;6, Supplement 1(0):S35.

(2) Bergesio F, Ciciani AM, Santostefano M, et al. Renal involvement in systemic amyloidosis--an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant. 2007;22(6):1608-1618.