fanconi syndrome and trt

[tmckenzie]

Hey all, I found out friday my kidneys are in bad shape. I have fanconi syndrome. Basically, I piss all of my minerals out. I have not had a chance to speak to my trt dr. Today is shot day, injust wonder how this effects trt. With adrenal insufficiency, diabetic, this cannot be good.

 

[Nelson Vergel]

Tmckensie

I am so sorry to hear. You actually got a diagnosis?

I did a google scholar search and found nothing about testosterone replacement in patients with Fanconi syndrome.

I found this good reference:

The treatment of a child with Fanconi syndrome mainly consists of the replacement of substances lost in the urine. Prominent among these substances are fluids and electrolytes.
Dehydration due to polyuria must be prevented by allowing free access to water; treat dehydration with either oral or parenteral solutions.

Metabolic acidosis due to the loss of bicarbonate is corrected by the administration of alkali, usually 3-10 mg/kg/d of sodium bicarbonate in divided doses.

Addition of a diuretic, such as 1-3 mg/kg/d of hydrochlorothiazide, may be necessary to avoid volume expansion, which magnifies the excretion of bicarbonate by lowering the renal threshold. Unfortunately, the diuretic increases potassium wasting and thus the need to augment potassium supplementation in the form of potassium bicarbonate, citrate, or acetate.

Correction of metabolic acidosis is beneficial but is not sufficient for the treatment of bone disease. Phosphate and vitamin D supplementation are also necessary.

Normalization of serum phosphate levels may be achieved by administering 1-3 g/d of supplemental phosphate. Administration should start at the lower level and be slowly increased over several weeks to minimize GI symptoms.
Vitamin D, administered as 1,25-dihydroxyvitamin D3 or 1a-hydroxyvitamin D3, is preferred because liver and/or renal hydroxylation may be impaired in patients with Fanconi syndrome.
The losses of glucose, amino acids, and uric acid are not usually symptomatic and do not require replacement. Recently, carnitine supplementation has been tried in an attempt to increase muscle strength; however, results have been mixed.
http://emedicine.medscape.com/article/981774-treatment

 

[tmckenzie]

Thanks Nelson, yes, that was the diagnoses. But the dr thinks there is something underlying causing all of this. Started with low t, then empty sella, adrenal insufficiency, diabetes, now this. It is like my body is attacking itself. I have more appts next week.

 

[tmckenzie]

Just now looking at my blood work, seems thyroid off too. Tsh was 1.42, free t4 was. .76 range is.61 -1.12. t4 marked low at 3.96 range was 6.09-12.23.