[Treatment and metaphylaxis of cystine urolithiasis in children]. 1989

R de Temple, and G Brien, and C Hellmann, and U Otting, and W Schöpke, and B Schönberger

Basic ideas about the pathogenesis of cystinuria and calculi formation are shown in this case report. Aspects of a present and effective cystine stone therapy are discussed after that. We present the therapeutic procedure in two patients with recurrent cystine urinary stone formation who were operated on cystine stones in the past. Our experiences with a combination of chemolitholysis with N-acetyl-cystein through percutaneous nephrostomy and an oral drug therapy with alpha-mercaptopropionyl-glycin are reported. This form of treatment was successful in our two cases. A metaphylactic therapy is necessary to prevent recurrent cystine stone formation.

UI MeSH Term Description Entries
D007507 Therapeutic Irrigation The washing of a body cavity or surface by flowing water or solution for therapy or diagnosis. Douching,Lavage,Douchings,Irrigation, Therapeutic,Irrigations, Therapeutic,Lavages,Therapeutic Irrigations
D007669 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. Kidney Stones,Renal Calculi,Nephrolith,Renal Calculus,Calculi, Kidney,Calculi, Renal,Calculus, Kidney,Calculus, Renal,Kidney Calculus,Kidney Stone,Stone, Kidney,Stones, Kidney
D008625 Tiopronin Sulfhydryl acylated derivative of GLYCINE. Mercaptopropionylglycine,Thiopronine,2-Mercaptopropionylglycine,2-Thiol-propionamido-acetic Acid,2-Thiolpropionamidoacetic Acid,Acadione,Captimer,Meprin,Thiola,Tiopronine,alpha-Mercaptopropionylglycine,2 Mercaptopropionylglycine,2 Thiol propionamido acetic Acid,2 Thiolpropionamidoacetic Acid,Acid, 2-Thiol-propionamido-acetic,Acid, 2-Thiolpropionamidoacetic,alpha Mercaptopropionylglycine
D009403 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. Percutaneous Nephrostomy,Nephrostomies, Percutaneous,Percutaneous Nephrostomies
D012008 Recurrence The return of a sign, symptom, or disease after a remission. Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D003555 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. Cystinurias
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000111 Acetylcysteine The N-acetyl derivative of CYSTEINE. It is used as a mucolytic agent to reduce the viscosity of mucous secretions. It has also been shown to have antiviral effects in patients with HIV due to inhibition of viral stimulation by reactive oxygen intermediates. Mercapturic Acid,Acemuc,Acetabs,Acetylcystein AL,Acetylcystein Atid,Acetylcystein Heumann,Acetylcystein Trom,Acetylcysteine Hydrochloride,Acetylcysteine Sodium,Acetylcysteine Zinc,Acetylcysteine, (D)-Isomer,Acetylcysteine, (DL)-Isomer,Acetylcysteine, Monoammonium Salt,Acetylcysteine, Monosodium Salt,Acetylin,Acetyst,Acétylcystéine GNR,Airbron,Alveolex,Azubronchin,Bisolvon NAC,Bromuc,Broncho-Fips,Broncholysin,Broncoclar,Codotussyl,Cystamucil,Dampo Mucopect,Eurespiran,Exomuc,Fabrol,Fluimucil,Fluprowit,Frekatuss,Genac,Hoestil,Ilube,Jenacystein,Jenapharm,Lantamed,Larylin NAC,Lindocetyl,M-Pectil,Muciteran,Muco Sanigen,Mucomyst,Mucosil,Mucosol,Mucosolvin,N-Acetyl-L-cysteine,N-Acetylcysteine,NAC AL,NAC Zambon,Optipect Hustengetränk,Siccoral,Siran,Solmucol,acebraus,durabronchal,mentopin Acetylcystein,Acetylcystein, mentopin,Acid, Mercapturic,Broncho Fips,BronchoFips,Hustengetränk, Optipect,Hydrochloride, Acetylcysteine,M Pectil,MPectil,Monoammonium Salt Acetylcysteine,Monosodium Salt Acetylcysteine,Mucopect, Dampo,N Acetyl L cysteine,N Acetylcysteine,NAC, Bisolvon,Sanigen, Muco,Sodium, Acetylcysteine,Zambon, NAC,Zinc, Acetylcysteine
D000284 Administration, Oral The giving of drugs, chemicals, or other substances by mouth. Drug Administration, Oral,Administration, Oral Drug,Oral Administration,Oral Drug Administration,Administrations, Oral,Administrations, Oral Drug,Drug Administrations, Oral,Oral Administrations,Oral Drug Administrations

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