The diagnosis of Fanconi syndrome is made based on tests that document the excessive loss of substances in the urine (eg, amino acids, glucose, phosphate, bicarbonate) in the absence of high plasma concentrations. (medscape.com)
More elaborate tests are designed to determine the renal threshold for these substances (ie, the concentration in the blood at which these substances appear in the urine) or their fractional reabsorption (ie, the percentage of the filtered load that is reabsorbed by the renal tubule). (medscape.com)
Urine color varies based on the urine concentration and chemical composition. (medscape.com)
Conversely, uric acid and cystine calculi are associated with acidic urine (although pH is more important for treatment than formation of cystine stones). (medscape.com)
Acidic urine is associated with uric acid calculi and cystine calculi. (medscape.com)
Specific gravity is a measurement of urine concentration and is representative of the kidney's ability to concentrate urine. (medscape.com)
The result is a decrease in serum and urine uric acid concentrations. (merckvetmanual.com)
Urine was cultured on a cystine-lactose-electrolyte-deficient agar plate and incubated at 37°C in an atmosphere containing 6% CO 2 for 1 day. (cdc.gov)
Patients with cystinuria excrete high levels of cystine in their urine and are at risk for kidney stone formation. (drugbank.com)
Kidney stones form when the solubility limit is exceeded and urine becomes supersaturated with endogenous cystine. (drugbank.com)
Youre not drinking enough water to dilute the concentration of minerals in your urine. (healthykidneyclub.com)
This causes a higher concentration of minerals and salts in your urine and can lead to kidney stones. (osu.edu)
Metabolic disorders - If you have a condition or disorder that prevents your body from properly breaking down food, it can lead to high levels of oxalate and cystine in your urine, which leads to stone formation. (osu.edu)
Cystinuria is an inherited disorder of renal transport characterised by high urine concentrations of cystine and other similar amino acids (ornithine, lysine, arginine). (eurovetgene.com)
An increased concentration of lysine in the urine. (nih.gov)
Urease hydrolyzes (or splits) urea into ammonium and hydroxyl ions resulting in an increase in ammonium and phosphate concentrations leading to an alkaline urine. (web.app)
Cystinuria is an inherited autosomal recessive disorder of the kidney that is the result of an inability to reabsorb cystine from the urine. (ucbraid.org)
Supersaturation of cystine in the urine produces crystals that precipitate and form stones in the kidney, which can be a cause of obstruction, infection, and chronic kidney disease. (ucbraid.org)
A mainstay of therapy is breaking or preventing the cystine bond on the molecular level such that cystine (which is formed from the joining of two cysteineamino acids and their corresponding sulfur atoms) cannot precipitate in the urine. (ucbraid.org)
This effectively increases the concentration of glucose in the urine. (ucbraid.org)
Our hypothesis suggests that administration of this drug to patients with cystine will introduce sufficient glucose into the urine to prevent the formation of cystine stones. (ucbraid.org)
Cystine stones typically form when a substance called "cystine" leaks into the urine. (englewoodhealthphysicians.org)
Glutamate5
Cystine increased glutamate efflux and decreased miniature EPSC (mEPSC) and spontaneous EPSC (sEPSC) frequency as well as evoked EPSC amplitude. (jneurosci.org)
Alcohol consumption is largely associated with alterations in the extracellular glutamate concentrations in several brain reward regions. (chapman.edu)
Cystine glutamate antiporter (xCT) is also downregulated after chronic ethanol exposure in P rats, and its upregulation could be valuable in attenuating ethanol drinking. (chapman.edu)
Along these lines, our present goal was to evaluate the effect(s) of the fumarate ester and antipsoriatic agent monomethylfumarate (MMF) on the expression and functional activity of the cystine/glutamate exchanger SLC7A11 (system x c - ), a transport system critical to potentiation of antioxidant signaling in retina. (elsevierpure.com)
Extracellular glutamate concentration is strictly maintained through several glutamate transporters, such as glutamate transporter 1 (GLT-1), cystine/glutamate transporter (xCT) and glutamate aspartate transporter (GLAST). (utoledo.edu)
Stones5
After cystine stones have been dissolved, a prevention protocol can be instituted. (merckvetmanual.com)
Cystine is an amino acid that forms stones when in high concentration, due to a rare inherited condition. (healthykidneyclub.com)
Excess cystine leads to formation of cystine crystals and stones in the urinary tract, which can result in urinary obstruction, haematuria and renal failure. (eurovetgene.com)
Cystine stones constitute a major health challenge for affected individuals with cystinuria because of the frequent recurrence of painful symptoms and the current absence of effective, patient-accepting treatment. (ucbraid.org)
For the second study, it was hypothesized that dogs fed pulse-based, grain-free diets for 28 days would cause decreased apparent total tract digestibility of macronutrients, increased fecal bile acid excretion, and decreased plasma levels of cystine, cysteine, methionine, and taurine, resulting in sub-clinical cardiac or blood changes indicative of early stages of DCM. (usask.ca)
This finding was specific in that four other amino acids, which have been shown to have significant plasma concentration alterations during HIV infection (i.e., cystine, methionine, taurine, and lysine), showed no significant change with nicotinamide therapy. (qxmd.com)
Lysine1
Cystinuria is an autosomal disorder characterized by impaired epithelial cell transport of cystine and dibasic amino acids (lysine, ornithine, and arginine) in the proximal renal tubule and gastrointestinal tract. (nih.gov)
Amino Acids5
The aim of the study was to compare plasma concentrations of 24 amino acids in umbilical cord blood in newborns from normoglycaemic pregnancies versus those complicated by gestational diabetes. (jpccr.eu)
The relationship between birth weight and length of newborns and placental concentrations of individual amino acids were also assessed. (jpccr.eu)
The placental plasma concentrations of 24 amino acids were determined using ion-exchange chromatography with an automated amino acid analyser. (jpccr.eu)
This suggests that research on the effects of amino acids concentration in umbilical cord blood on the foetal development in gestational diabetes-complicated pregnancies should be continued. (jpccr.eu)
The principal factor responsible for the observed immune effect does not appear to be the availability or concentration of single essential amino acids but rather the composite effect of the specific amino acid distribution in the protein. (immunehealthscience.com)
Cysteine2
At intervals for 24 hr, samples of blood and other tissues were obtained, processed, and analyzed for reduced GSH, glutathione disulfide (GSSG), cysteine, and cystine using HPLC with dual electrochemical detection. (aspetjournals.org)
In the uninjected controls, the GSH concentration decreased about 30% in all tissues of the aging mouse, but the GSSG cysteine, and cystine levels were unchanged during the life span. (aspetjournals.org)
Renal2
Cystinuria, with subsequent cystine urolith formation, results from a breed-related inherited disorder of renal tubular transport in dogs. (merckvetmanual.com)
The impaired renal reabsorption of cystine and its low solubility causes the formation of calculi in the urinary tract, resulting in obstructive uropathy, pyelonephritis, and, rarely, renal failure (summary by Barbosa et al. (nih.gov)
Prefrontal cortex1
Physiological levels of cystine (100-300 n m ) were restored to acute tissue slices from the nucleus accumbens or prefrontal cortex. (jneurosci.org)
Glutamic1
Anti Mouse Cystine/Glutamic acid transp. (caslab.com)
Crystals2
in 1903, he found cystine crystals in the liver and spleen of a 21-month-old infant and called the disease "a familial cystine diathesis. (medscape.com)
When concentration levels reach the point at which the salts no longer dissolve, they precipitate out and form crystals . (curezone.org)
Excretion2
Tiopronin is used as a second-line therapy to control the rate of cystine precipitation and excretion, and prevent kidney stone formation. (drugbank.com)
General risk factors include disorders that increase urinary salt concentration, either by increased excretion of calcium or uric acid salts, or by decreased excretion of urinary citrate. (msdmanuals.com)
Solubility1
By reducing urinary cystine concentrations below the solubility limit, tiopronin helps reduce cystine stone formation. (drugbank.com)
Soluble4
Tiopronin is an active reducing agent which undergoes a thiol-disulfide exchange with cystine to form a water-soluble mixed disulfide complex. (drugbank.com)
Consequently, the cystine amino acid undergoes a conversion into a water-soluble derivative known as cystic acid. (ecoslay.com)
The stored cystine is poorly soluble and crystallizes within the lysosomes of many cell types, leading to widespread tissue and organ damage. (medscape.com)
Alpha lipo1
This study evaluates how daily alpha lipoic acid supplementation affects cystine kidney stone recurrence. (ucbraid.org)
Like BMPs, BMP antagonists contain cystine knots and typically form homo- and heterodimers. (thermofisher.com)
Tiopronin2
Tiopronin and d -penicillamine are cystine-binding agents. (merckvetmanual.com)
Tiopronin is dosed on an individual basis using close monitoring of urinary cystine concentrations and urinary output. (drugbank.com)
Minerals2
Its concentrations of minerals such as magnesium and potassium make avocado oil particularly good for the hair with hair follicle needing magnesium to hold its strength and formation and potassium to enable the follicle to absorb vital nutrients to stay healthy. (loreoriginals.com)
Certain blood tests help show whether you have higher than normal concentrations of certain minerals in your blood as well as checking for kidney function. (englewoodhealthphysicians.org)
Levels1
Regulations regarding constraints, permitted concentration levels and availability vary by country and region. (paulaschoice.co.uk)
Nephropathic cystinosis is an inherited (autosomal recessive) lysosomal storage disorder caused by defective transport of the amino acid cystine out of lysosomes. (medscape.com)
Disulfide1
Cystine plays a crucial role in the structure of hair, as it forms disulfide linkages within the helical structure of keratin. (ecoslay.com)
Legionella2
L-cystine is an amino acid that is required for the growth of Legionella. (stemcelldaily.com)
L-cystine is an essential amino acid that Legionella species cannot synthesize. (stemcelldaily.com)
Patients1
PROCYSBI (cysteamine bitartrate) delayed-release capsules and delayed-release oral granules is a cystine-depleting agent indicated for the treatment of nephropathic cystinosis in adults and pediatric patients 1 year of age and older. (horizonbyyourside.com)
Fatty1
The high concentration of fatty acids in cotton seed oil goes hand in hand with much sought after natural Ceramides which are lipids naturally present in the hair cuticle. (loreoriginals.com)
Growth3
Increased atmospheric CO 2 concentrations promote the growth of fastidious microorganisms. (cdc.gov)
Nitrite supported the growth of many strains providing that the optimum concentration was used, together with a carbon source which was available under those conditions. (gla.ac.uk)
Growth factor concentrations and their placental mRNA expression are modulated in gestational diabetes mellitus: possible interactions with macrosomia. (jpccr.eu)
Absence1
ARPE-19 and primary mouse RPE cells were cultured in the presence or absence of varying concentrations of MMF (0-5000 μM) for 0 to 24 hours. (elsevierpure.com)
Protein1
The above-described immune effect of all tested proteins was obtained at 20 g/100 g concentration with no further increments with 30- and 40 g/100 g protein in the diet. (immunehealthscience.com)
Significantly2
Combinations of Imatinib or Dasatinib with different concentrations of Inecalcitol increased significantly the antiproliferative activities and potencies of both drugs (****p (bvsalud.org)
This conversion reduces the amount of cystine present in the hair while significantly increasing the concentration of cystic acid. (ecoslay.com)
Tissues1
The choice of tissues and the factors influencing measured cyanide concentrations are also important (Ballantyne 1983c, 1987a). (cdc.gov)
Conditions2
Penicillamine or N-acetylcysteine yielded similar results except that under aerobic conditions, reaction between Ni(II) and N-acetylcysteine without hydroperoxide did not generate a significant concentration of free radicals. (cdc.gov)
Under the same experimental conditions, cystine did not generate any detectable free radicals, suggesting an important role of the -SH group in Ni(II)-mediated free radical generation. (cdc.gov)
Helps1
L-cystine also helps to maintain the optimal pH of the medium. (stemcelldaily.com)