Fanconi

Fanconi has afflicted many Basenjis in America.   A couple of Australian exports have developed Fanconi overseas.  Recently in 2005 a Basenji Rescue dog was diagnosed with Fanconi in Victoria and then we heard of a Basenji which had died from Fanconi in Queensland.  This is very sad news as prior to this there were no reported cases in Australia.  The following information supplies basic details of the disease and most importantly explains how to test a Basenji for glucose in urine which may indicate early warning of the disease.    

Fanconi Syndrome is a kidney disease.  It is a renal disorder caused by a defect in the proximal renal tubule.  This defect causes a reduction in the reabsorption of sodium, glucose, calcium, phosphate and amino acid from the proximal tubular cells of the kidney back into the bloodstream.  This can lead to fatal disturbances in Acid balance.  The acidic condition of the body can cause calcium to dissolve from the bones.  There is potential for Kidney and bladder infections due to the glucose and low pH situation.

Symptoms are excessive drinking, excessive urination, muscle wasting and weight loss.  Fanconi Syndrome is often mistaken for Diabetes or Cushing Syndrome so Fanconi should be suspected when there is excessive urination, normal blood sugar levels and sugar in urine.  Early diagnosis is essential as chemical levels can be balanced medically to prevent further damage to organs.  If left untreated the condition will deteriorate leading to eventual death.  

Fanconi is believed to be genetic so research is being conducted in order to find the gene responsible so that a genetic test can be developed.  Meanwhile owners of Basenjis can check their dogs for Fanconi by regularly testing urine.  This is inexpensive, easy and can be done at home.

How to test your Basenji.

It is important to test your Basenji for Fanconi from 3 years of age.  Although onset of the disease is usually between 5 and 7 years of age it has been reported as young as 3 years and as old as 11 years in Basenjis.  Test your Basenji's urine once a month for glucose in their urine.  To do this purchase a product called Clinistix or similar from diabetics section of the Chemist.  There are usually 100 Reagent Strips in a container.  They are used to test for Glucose in urine.  When your Basenji does a morning wee catch some of the urine in a clean container.  I recommend something with a handle so a ladle, saucepan, frying pan is ideal.  Then dip the test area of the strip into the fresh urine.  Remove immediately.  Tap edge of test strip against container to remove excess urine.  10 seconds after wetting compare the colour of the test area closely with the colour chart on the Clinistix container.  If you get positive results for the presence of glucose contact your vet.  It is important to give your vet information from the links below so that a positive diagnosis can be made.  A positive test strip does not necessarily mean your Basenji has Fanconi but can be an early indicator to the disease.  Do not delay in taking your Basenji to the vet as early diagnosis can limit damage to organs by commencing the Fanconi Protocol.  With the help of supplementation your Basenji can then lead a better quality of life.  Without treatment your Basenji will decline in health rapidly.

To find out what Fanconi is click here

To find out about research into Fanconi and how you can assist click here

To find out the Fanconi Disease Management Protocol for Veterinarians click here

Breaking News - Exciting News !!
Research is about to get under way in the USA to find the gene or marker for FANCONI
Below is information on this research project as posted on the Basenji List by Lisa Auerbach
All email sent through BASENJI-L is Copyright 2005 by its original author but permission has been granted to cross post.

Date: Sun, 10 Apr 2005
From: Lisa Auerbach <itzyu@PRODIGY.NET>
Subject: Fanconi affecteds and relatives needed for research study

Steve Gonto, Laurie Stargell, and I were recently contacted by Dr. Robert Kleta, a researcher at the
National Institutes of Health - a researcher who works with human renal Fanconi patients.

Dr. Kleta was interested in using Basenjis as a model for human renal Fanconi, and searching for the gene in
Basenjis. Basenjis were of interest because we have a genetic form of the disease, and because there are
more Basenjis with renal Fanconi than there are humans with inherited renal Fanconi. Thus, it might be
easier to find the gene in Basenjis than in people - but once found, the gene might well be the same in
both humans and Basenjis. 

After some discussion as to if and how our banked Basenji blood would be used, the three groups
interested in studying this are going to work together. Some details, from a note from Jon Curby,
are below. 

The researchers involved are Dr. Robert Kleta with National Institutes of Health, Dr. Gary Johnson of the
University of Missouri, and Dr. Elaine Ostrander of the National Human Genome Research Institute. 

The human researchers are interested in seeing if Basenji Fanconi and human Fanconi are genetically
related. They will be doing a full genome scan of Basenji affecteds and normals, to attempt to detect
the gene or a marker for the gene.

This work will be primarily funded by the human folks - some CHF funding may also be used - at this time, no
one is asking us for money.

Below are some notes from Jon -

Hi Lisa, I just talked to Gary and they have worked out an agreement that involves the three organizations. MU is going to send DNA to Dr Ostrander's lab and NIH is going to pay for a student technician to perform the work there. They hope to have the DNA to Elaine within three weeks and results within 2 to 4 months. 
... 

What they need now is for us to try to verify the status on as many of the dogs in the sample pool as
possible. This needs to be done within the next three weeks and is critical. I can send the list and what
status information we have to everyone on the health committee, or whoever needs to see it. I think the
only thing we can do is divide it up and start calling people.

And, from a subsequent note,

As far as additional samples are concerned. We would need to get them to MU quickly so they could extract
the DNA and forward it to Ostrander's lab. I will get the latest list from Liz Monday. After I clean it up, add missing pedigree information and send it to the health committee it needs to be divided such that each committee member takes charge of those dogs where they have some logical connection, geographically, pedigree or personal. The owners or breeders need to be contacted to establish the latest Fanconi status. 

If you are willing and able to donate blood from an affected or a parent of an affected, and can do so in
the next couple of weeks, please let me know. If you have already told me you were willing to donate, you
don't need to send another email unless the timeframe makes it impossible.

If you are contacted by the Health Committee about a study dog, please cooperate in getting us information about that dog's Fanconi status. Accurate identification of Fanconi status is very important to
this study.

Needless to say, this is extremely good news.

Lisa

Please feel free to forward in its entirety.


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Date: Sun, 10 Apr 2005
From: Lisa Auerbach <itzyu@PRODIGY.NET>
Subject:  Fanconi affecteds and relatives needed for research study

Here are the sample submission instructions.

They are interested in dogs that are either

1. Affecteds, or 
2. Normals age 9 or older that are parents,
offspring, or full siblings of affecteds. This is to
avoid identifying a dog as normal when it simply
hasn't onset yet.

Affecteds under age 9 are wanted. 


jon curby <jcurby@offa.org> wrote:

To: <BasenjiBreederRoundtable@yahoogroups.com>
From: "jon curby" <jcurby@offa.org>
Date: Sun, 10 Apr 2005
Subject: Fanconi affecteds and relatives needed for research study

I have pasted the submission form and instructions below.
Jon Curby

 SAMPLE HANDLING

For Canine DNA Research at the University of Missouri

Blood Sample - The ideal sample for DNA extraction is 5-10cc's of whole blood, in purple-topped (EDTA)
tubes. For very small dogs or puppies, 3ccs should be sufficient. The blood sample needs only to be put in
the tubes and rocked gently a few times to distribute the anticoagulant - do not spin, extract serum, or
anything further.  Refrigerate if the sample is being held for any time before shipping. 

Frozen Semen - If there is frozen semen stored from sires or affected dogs, DNA can be extracted from it.
Please send 2 straws. They do not need to be shipped frozen, but do pack them in a crush-proof container.

Tissue Sample - Upon death of the dog, donating an organ will provide an endless supply of DNA for
research. Please discuss this with your vet ahead of time if you intend to do this. (If the dog is to be
euthanized, have a blood sample pulled first, if possible, and send both samples.)  First choice is spleen, second choice kidney, and third choice is liver (only a portion is needed, not the entire liver).  One tissue sample is sufficient. Have the organ removed as soon as possible following death, place into a labelled freezer bag, place that into a second bag, freeze, and ship.

Label sample with the following;

call name - owner's last name 

(If samples from several dogs are sent together, number samples and forms)

An Individual Dog Information form should be completed, and a pedigree copy must be included with
the sample to tie it in with the correct family. If the dog is not affected but is a relative of an affected, please indicate the relationship.

Shipping - Ideally the sample should be shipped immediately (with a tissue sample make certain it is
completely frozen first). If samples are held for a day or over a weekend, blood must be refrigerated, and
tissue samples must be kept frozen. Ship via overnight delivery (US Mail, UPS, or FedEx). Do not send on a
Friday - there will not be anyone to accept the delivery on a Saturday, and the sample could be unusable by Monday. Pack in a small insulated container (most vets have these for shipping samples to labs), with one or more cool packs - it is important that blood samples be kept cool but not frozen, and tissue samples be kept as frozen as possible. 

The delivery address is;

Dr. Gary Johnson - Basenji DNA Research
320 Connaway Hall
University of Missouri
Columbia, MO 65211

If you need clarification, or have any questions about any of these procedures, please contact Liz Hansen by phone (573-884-3712), email (HansenL@missouri.edu), or regular mail (321 Connaway Hall, University of
Missouri, Columbia, MO 65211). Liz is Dr. Johnson's Coordinator of Veterinary Information, and can help
with any questions you may have.

Thank you for your cooperation and participation!

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Copyright © Davina Hopkins 2005