Cystine: A covalently linked dimeric nonessential amino acid formed by the oxidation of CYSTEINE. Two molecules of cysteine are joined together by a disulfide bridge to form cystine.Cystinosis: A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosin. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. In the KIDNEY, nephropathic cystinosis is a common cause of RENAL FANCONI SYNDROME.Kidney Calculi: Stones in the KIDNEY, usually formed in the urine-collecting area of the kidney (KIDNEY PELVIS). Their sizes vary and most contains CALCIUM OXALATE.Cystine Knot Motifs: Amino acid sequence in which two disulfide bonds (DISULFIDES) and their connecting backbone form a ring that is penetrated by a third disulfide bond. Members include CYCLOTIDES and agouti-related protein.Urinary Calculi: Low-density crystals or stones in any part of the URINARY TRACT. Their chemical compositions often include CALCIUM OXALATE, magnesium ammonium phosphate (struvite), CYSTINE, or URIC ACID.Lithotripsy: The destruction of a calculus of the kidney, ureter, bladder, or gallbladder by physical forces, including crushing with a lithotriptor through a catheter. Focused percutaneous ultrasound and focused hydraulic shock waves may be used without surgery. Lithotripsy does not include the dissolving of stones by acids or litholysis. Lithotripsy by laser is LITHOTRIPSY, LASER.Amino Acid Transport System y+Gallstones: Solid crystalline precipitates in the BILIARY TRACT, usually formed in the GALLBLADDER, resulting in the condition of CHOLELITHIASIS. Gallstones, derived from the BILE, consist mainly of calcium, cholesterol, or bilirubin.Cystinuria: An inherited disorder due to defective reabsorption of CYSTINE and other BASIC AMINO ACIDS by the PROXIMAL RENAL TUBULES. This form of aminoaciduria is characterized by the abnormally high urinary levels of cystine; LYSINE; ARGININE; and ORNITHINE. Mutations involve the amino acid transport protein gene SLC3A1.Cholelithiasis: Presence or formation of GALLSTONES in the BILIARY TRACT, usually in the gallbladder (CHOLECYSTOLITHIASIS) or the common bile duct (CHOLEDOCHOLITHIASIS).Ureteral Calculi: Stones in the URETER that are formed in the KIDNEY. They are rarely more than 5 mm in diameter for larger renal stones cannot enter ureters. They are often lodged at the ureteral narrowing and can cause excruciating renal colic.Calcium Oxalate: The calcium salt of oxalic acid, occurring in the urine as crystals and in certain calculi.Cysteamine: A mercaptoethylamine compound that is endogenously derived from the COENZYME A degradative pathway. The fact that cysteamine is readily transported into LYSOSOMES where it reacts with CYSTINE to form cysteine-cysteamine disulfide and CYSTEINE has led to its use in CYSTINE DEPLETING AGENTS for the treatment of CYSTINOSIS.Calculi: An abnormal concretion occurring mostly in the urinary and biliary tracts, usually composed of mineral salts. Also called stones.Urolithiasis: Formation of stones in any part of the URINARY TRACT, usually in the KIDNEY; URINARY BLADDER; or the URETER.Urinary Bladder Calculi: Stones in the URINARY BLADDER; also known as vesical calculi, bladder stones, or cystoliths.Ureteroscopy: Endoscopic examination, therapy or surgery of the ureter.Cysteine: A thiol-containing non-essential amino acid that is oxidized to form CYSTINE.Nephrolithiasis: Formation of stones in the KIDNEY.Magnesium Compounds: Inorganic compounds that contain magnesium as an integral part of the molecule.Amino Acids, SulfurFanconi Syndrome: A hereditary or acquired form of generalized dysfunction of the PROXIMAL KIDNEY TUBULE without primary involvement of the KIDNEY GLOMERULUS. It is usually characterized by the tubular wasting of nutrients and salts (GLUCOSE; AMINO ACIDS; PHOSPHATES; and BICARBONATES) resulting in HYPOKALEMIA; ACIDOSIS; HYPERCALCIURIA; and PROTEINURIA.Cyclotides: A continuous circle of peptide bonds, typically of 2-3 dozen AMINO ACIDS, so there is no free N- or C-terminus. They are further characterized by six conserved CYSTEINE residues that form CYSTINE KNOT MOTIFS.Nephrostomy, Percutaneous: The insertion of a catheter through the skin and body wall into the kidney pelvis, mainly to provide urine drainage where the ureter is not functional. It is used also to remove or dissolve renal calculi and to diagnose ureteral obstruction.Cholangiopancreatography, Endoscopic Retrograde: Fiberoptic endoscopy designed for duodenal observation and cannulation of VATER'S AMPULLA, in order to visualize the pancreatic and biliary duct system by retrograde injection of contrast media. Endoscopic (Vater) papillotomy (SPHINCTEROTOMY, ENDOSCOPIC) may be performed during this procedure.Sphincterotomy, Endoscopic: Incision of Oddi's sphincter or Vater's ampulla performed by inserting a sphincterotome through an endoscope (DUODENOSCOPE) often following retrograde cholangiography (CHOLANGIOPANCREATOGRAPHY, ENDOSCOPIC RETROGRADE). Endoscopic treatment by sphincterotomy is the preferred method of treatment for patients with retained or recurrent bile duct stones post-cholecystectomy, and for poor-surgical-risk patients that have the gallbladder still present.Glutathione: A tripeptide with many roles in cells. It conjugates to drugs to make them more soluble for excretion, is a cofactor for some enzymes, is involved in protein disulfide bond rearrangement and reduces peroxides.Cholecystectomy: Surgical removal of the GALLBLADDER.Choledocholithiasis: Presence or formation of GALLSTONES in the COMMON BILE DUCT.Disulfides: Chemical groups containing the covalent disulfide bonds -S-S-. The sulfur atoms can be bound to inorganic or organic moieties.Cystamine: A radiation-protective agent that interferes with sulfhydryl enzymes. It may also protect against carbon tetrachloride liver damage.Amino Acid Transport Systems, Neutral: Amino acid transporter systems capable of transporting neutral amino acids (AMINO ACIDS, NEUTRAL).Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.Methionine: A sulfur-containing essential L-amino acid that is important in many body functions.Amino Acid Transport Systems, Basic: Amino acid transporter systems capable of transporting basic amino acids (AMINO ACIDS, BASIC).Cholangiography: An imaging test of the BILIARY TRACT in which a contrast dye (RADIOPAQUE MEDIA) is injected into the BILE DUCT and x-ray pictures are taken.Cholecystography: Radiography of the gallbladder after ingestion of a contrast medium.Bile Duct Diseases: Diseases in any part of the ductal system of the BILIARY TRACT from the smallest BILE CANALICULI to the largest COMMON BILE DUCT.Oldenlandia: A plant genus of the family RUBIACEAE. Some species are used as an ingredient in Chinese and African traditional medicines. Members contain kalata B1, a macrocyclic peptide.Common Bile Duct: The largest bile duct. It is formed by the junction of the CYSTIC DUCT and the COMMON HEPATIC DUCT.Gallbladder: A storage reservoir for BILE secretion. Gallbladder allows the delivery of bile acids at a high concentration and in a controlled manner, via the CYSTIC DUCT to the DUODENUM, for degradation of dietary lipid.Cystine-Knot Miniproteins: A structurally-related family of small proteins that form a stable tertiary fold pattern which is supported by a series of disulfide bonds. The arrangement of disulfide bonds between the CYSTEINE moieties results in a knotted structure which is unique to this family of proteins.Calcium Phosphates: Calcium salts of phosphoric acid. These compounds are frequently used as calcium supplements.Oxalates: Derivatives of OXALIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that are derived from the ethanedioic acid structure.Coprophagia: Eating of excrement by animal species.Sulfhydryl Compounds: Compounds containing the -SH radical.Hypercalciuria: Excretion of abnormally high level of CALCIUM in the URINE, greater than 4 mg/kg/day.Archaeology: The scientific study of past societies through artifacts, fossils, etc.MaleatesCholecystectomy, Laparoscopic: Excision of the gallbladder through an abdominal incision using a laparoscope.Sphincterotomy, Transhepatic: Surgery of the smooth muscle sphincter of the hepatopancreatic ampulla to relieve blocked biliary or pancreatic ducts.Hyperoxaluria: Excretion of an excessive amount of OXALATES in the urine.Potassium Citrate: A powder that dissolves in water, which is administered orally, and is used as a diuretic, expectorant, systemic alkalizer, and electrolyte replenisher.
  • For several weeks after treatment, those small stone fragments are passed out of the body in the urine. (bidmc.org)
  • New onset pain or pain that is getting worse instead of better may indicate that either there is bleeding around the kidney as a result of ESWL or that the kidney stone fragments have all fallen into the ureter and are blocking the drainage of urine from the kidney. (bidmc.org)
  • It is a disease involving the defective transepithelial transport of cystine and dibasic amino acids in the kidney and intestine, and is one of many causes of kidney stones. (wikipedia.org)
  • The high affinity and sodium-independent transport of cystine and neutral and dibasic amino acids is the role of the protein [b(0,+)-type amino acid transporter that is encoded by SLC7A9 ( Figure 1 ). (frontiersin.org)
  • The investigators confirmed this is a likely mechanism by demonstrating that the urine from α-lipoic acid-treated animals formed significantly less cystine precipitate (solid deposits that form from the compound in solution) during a 3-day period in the laboratory. (nih.gov)
  • D-penicillamine has been used the longest in cystine stone prevention but is the least well-tolerated. (medscape.com)
  • Penicillamine combines chemically with cystine (cysteine-cysteine disulfide) to form penicillamine-cysteine disulfide, which is more soluble than cystine and is readily excreted. (medscape.com)
  • These are active reducing agents that undergo thiol-disulfide exchange with cystine (cysteine-cysteine disulfide) to form tiopronin-cystine disulfide, which is more water-soluble than cystine and is readily excreted. (medscape.com)
  • The median stone burden and mean urine pH before each surgery were 6.5 cm2 and 6.5+/-0.9, respectively. (bvsalud.org)
  • Raise the urine pH level to 7-7.5 to make cystine more soluble. (medscape.com)
  • I came across a 1930s case in which a doctor treated a woman suffering from carbonate stones with the above-mentioned brew of dilute aqua regia and whatnot plus an acid ash diet, reducing her urine pH to 4.5 for several months. (straightdope.com)