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Dissolution of cystine stones by irrigated tiopronin solution.
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A new method involving the introduction of tiopronin solution directly into the renal pelvis or bladder has been developed for the treatment of cystine stones. Therapeutic irrigation by tiopronin solution was done in 4 cases of residual cystine kidney stones using a nephrostomy tube, and in an
Folate pro-drug of cystamine as an enhanced treatment for nephropathic cystinosis.
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Nephropathic cystinosis is a rare autosomal recessive disease characterised by raised intracellular levels of the amino acid, cystine. If untreated, the disease, progressively deteriorates towards end stage renal disease (ESRD) at the end of the first decade. The disease is caused by a defect in the
Intravenous cysteamine therapy for nephropathic cystinosis.
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A 4-y-old boy with nephropathic cystinosis and gastrointestinal dysmotility of unknown etiology was treated with i.v. cysteamine over a period of 10 mo. Thirty minutes after a dose of 10 mg/kg cysteamine free base, the leukocyte cystine value had fallen from 11.9 to 4.9 nmol of half-cystine/mg of
The in vivo use of dithiothreitol in cystinosis.
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Two male patients with late stage (uremic) infantile nephropathic cystinosis (INC) (Table 1) were treated by mouth with the reducing agent dithiothreitol (DTT), at doses not exceeding 25 mg-kg-1 body weight three times per day. Three sequential periods of observation were obtained in both patients:
[Cystinuria therapy by ascorbic acid (author's transl)].
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At the beginning of the four chapters on phenomena, analysis, pathophysiology and therapy of cystinuria the essentials of the published literature are summarized. The frequency of cystinuria is in the order of 1:10,000. Besides the cystine lithiasis occurring in nine tenths of all cystinuria
Gastrointestinal manifestations of nephropathic cystinosis in children.
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BACKGROUND
Cystinosis is an autosomal recessive disorder which is characterized by both renal and extrarenal symptoms. Gastrointestinal dysfunction has been reported in adolescent with cystinosis, and it is rarely considered in the infants. The present case series reviewed gastrointestinal
Phase I trial of imexon in patients with advanced malignancy.
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OBJECTIVE
Imexon, a pro-oxidant small molecule, has antitumor activity in preclinical models. The drug induces apoptosis through accumulation of reactive oxygen species. The purpose of this trial was to define the maximum-tolerated dose (MTD), toxicities, pharmacokinetics, and pharmacodynamics of
Use of N-acetylcysteine in clinical toxicology.
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The major use of N-acetylcysteine in clinical toxicology is in the treatment of acetaminophen (paracetamol) overdosage. The hepatorenal toxicity of acetaminophen is mediated by a reactive metabolite normally detoxified by reduced glutathione. If glutathione is depleted, covalent binding to
Adverse gastrointestinal effects of arginine and related amino acids.
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Oral supplements of arginine and citrulline increase local nitric oxide (NO) production in the small intestine and this may be harmful under certain circumstances. Gastrointestinal toxicity was therefore reviewed with respect to the intestinal physiology of arginine, citrulline, ornithine, and
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