These diseases are characterized by urinary hyperexcretion of insoluble mineral salts, leading to recurrent kidney stones or increased renal calcium deposition (Edvardsson et al., 2013).
Cystinuria is an autosomal recessive disorder in which the kidney, due to a genetic defect in the cystine transporter, is unable to reabsorb cystine in the proximal tubule, resulting in urinary hyperexcretion of amino acids cystine, ornithine, lysine, and arginine (COLA).
[1,2] Primary abnormality in CEP is due to decreased uroporphyrinogen III cosynthase activity resulting in accumulation and hyperexcretion
of biologically inactive type I porphyrins.
can be blocked by using cobalt chloride 370 meg per drop, 3 drops per day.
Cystinuria was confirmed by urine amino acid chromatography (half-cystine, ornithine, lysine and arginine concentrations >10 times the upper limit of the reference interval), thus excluding transient hyperexcretion
of cystine in the first months of life as an explanation (5).
Estrogen Treatment 'Failure': Hyperexcretion of Estrogen
Estrogen hyperexcretion can be confirmed (or not) with a finding of higher than anticipated estrogens in a 24-hour urine specimen, while serum estrogens remain low normal or low.
The importance of the hyperexcretion
of these metabolites for evaluation of the metabolic status of patients with type 1 diabetes is unknown, but lactate and acetate were higher in those patients with [HbA.sub.1c] >8.0%.
In this circumstance, much higher than anticipated levels of all estrogens are frequently found in the urine, a situation termed "hyperexcretion
" or "failure of hormone retention." Treatment with physiologic-dose (300-600 micrograms) of cobalt chloride almost always corrects this situation, gradually reducing urinary estrogen excretion towards normal, while menopausal symptoms gradually fade.
Pharmacokinetics of tin-mesoporphyrin in man and the effects of tin-chelated porphyrins on hyperexcretion
of heme pathway precursors in patients with acute inducible porphyria.
In the absence of such overt proteinuria however, measurement of specific urinary proteins such as [[beta].sub.2] microglobulin or myeloma proteins may be useful in the diagnosis of tubulopathies or overflow proteinuria, whereas subclinical albumin hyperexcretion
may serve as useful early predictor of diabetic nephropathy (1-5).
Patients were all 5 years of age or older and were classified by: (a) prior history of renal dysfunction, (b) hyperexcretion
of oxalate/glycerate or oxalate/glycolate (determined by standard organic acid analysis at reference laboratories), (c) liver AGT activity in liver biopsy material (when performed), and (d) response to pyridoxine.