From Saskatchewan, Canada:
We received a brief medical history for the 5 year old Russian girl we are adopting. It mentions a phosphatic diabetes -- may refer to elevation of serum alkaline phosphatase levels due to obstructive hepatic jaundice -- residual effects of previous Hepatitis A. What is this? (They also mention "secondary pyelonephritis due to urinary reflux." She was born prematurely.)
I do not think that the 'phosphatic diabetes' is related to an increased serum alkaline phosphatase that occurred during an episode of Hepatitis A. It is a term that refers to one of a group of phosphate-losing renal tubular syndromes that may be both congenital or acquired. The proximal renal tubule normally reabsorbs most of the phosphate, glucose, bicarbonate and amino acids that are filtered through the glomerulus - you may have to look up these anatomical terms to understand all this. Sometimes though, either as a result of a congenital condition or of another metabolic disease such as cystinosis or Wilson's disease, the tubule fails to reabsorb one or more of these products. When the reabsorption of all of them is impaired it is called Fanconi Syndrome, when it is bicarbonate alone it is called Renal Tubular Acidosis. When it is phosphate only, phosphatic diabetes would be an appropriate name, though in Western Europe and North America the term Renal Tubular Rickets would be most commonly used.
The main clinical problem is the rickets and its effect on stature. However, determined medication with buffered phosphates that keeps the serum phosphorus greater than 3mg/dl can prevent this to a significant degree; but the solution is not very pleasant to take.
Urinary reflux means that when a radio-opaque dye is introduced into the bladder, it can be seen to move back up into the ureter -- the tube between the kidney and the bladder. This may be due to a bladder outlet obstruction which can often be easily remedied or it may be secondary to repeated urinary tract infections that also involve the pelvis of the kidney and may long term lead to renal failure. Surgery may be required.
I am sorry to be a messenger of this rather upsetting news. You are going to need support from a pediatric nephrology unit and what I would suggest is that you get as much additional information about this little girl as you can, especially X rays and laboratory work including serum phosphorus levels and a blood urea. This may not be easy from a Russian unit at this time; but with or without this information I think you should get help from your doctor to establish contact with a pediatric nephrologist and then go and talk this situation over.
Original posting 12 Jul 97
Last Updated: Tuesday April 06, 2010 15:08:54
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