Stone dissolution with D-penicillamine in cystinuria.

Lotz, Myron; Bartter, Frederic C.

doi:10.1136/bmj.2.5475.1408

Cited by 27 publications

(7 citation statements)

References 5 publications

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“…It is of note that the large crystals have lost their typical hexagonal shape, also suggesting the beginning of a dissolution process. Such dissolution of cystine stones has already been reported (suggested) in the literature (Lotz & Bartter, 1965;Lindell et al, 1995). Similar alterations of cystine crystals as seen with tiopronine were observed for patients (subjects 20-22, Fig.…”

Section: Discussionsupporting

confidence: 88%

Therapy modifies cystine kidney stones at the macroscopic scale. Do such alterations exist at the mesoscopic and nanometre scale?

et al. 2014

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International audienceWith an incidence of 1:7000 births, cystinuria, the most frequent cause of stone formation among genetic diseases, represents a major medical problem. Twentyfive cystine stones randomly selected from cystinuric patients were investigated. From a crystallographic point of view, cystine stones are composed of micrometre size crystallites, which are made up of an aggregation of nanocrystals. Through scanning electron microscopy, the morphology and size of the crystallites have been described, while the size of the nanocrystals was investigated by means of powder neutron diffraction. Powder neutron diffraction analysis and/or scanning electron microscopy examination of cystine stones provide evidence that usual alkalinization by sodium bicarbonate associated with high diuresis significantly reduces the size of both nanocrystals and crystallites, while for other treatments, including alkalinizing drugs and thiol derivatives, the data suggest mainly changes in the topology of crystallites. Alkalinization with sodium bicarbonate affects cystine kidney stones at the mesoscopic and nanoscopic scales, while other medical treatments only alter their surface. Such an approach may help to assess the interaction between drugs and cystine stones in cystinuric patients

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Section: Discussionsupporting

confidence: 88%

Therapy modifies cystine kidney stones at the macroscopic scale. Do such alterations exist at the mesoscopic and nanometre scale?

et al. 2014

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“…The biochemical results of penicillamine in cases 1 and 2, i.e., a marked decrease in the 24-hour excretion of cystine (table I, fig. 1) are in accordance with the results of other workers (2,8,17). In view of the short duration of treatment, X-ray survey of the kidneys showed no significant difference in the size of the concretion in case 1.…”

Section: Discussionsupporting

confidence: 90%

“…The definite biochemical improvement, as well as the less decisive signs of clinical improvement, in cases 1 and 2 in addition to the good results reported by other workers (2,8,17) suggest that the therapeutic aims in these two cases might be achieved by prolonged penicillamine therapy. I n case 1 the aim was to avoid further operations, which seemed hazardous because of the existing coronary disease, and in case 2 to avoid a possible bilateral kidney operation.…”

Section: Discussionsupporting

confidence: 60%

Excretion of Trace Elements in Two Penicillamine‐treated Cases of Cystinuria

Boström

Wester

1967

Journal of Internal Medicine

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“…Cystine is a homodimer of two cysteine molecules HSCH 2 CH(NH 2 )COOH connected by a disulfide bond: COOH(NH 2 )CHCH 2 S-SCH 2 CH(NH 2 )COOH. CBTDs work by chemically reducing the disulfide bond of cystine and forming drug-bound cysteine complexes, which are up to 50 times more soluble than cystine [31]. The potential for CBTDs to dissolve cystine molecules was demonstrated in a study of seven patients whose urine had higher cystine capacity while taking CBTDs [27].…”

Section: Current Medical Treatments Of Cystinuriamentioning

confidence: 99%

Cystine Growth Inhibition Through Molecular Mimicry: a New Paradigm for the Prevention of Crystal Diseases

et al. 2015

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Cystinuria is a genetic disease marked by recurrent kidney stone formation, usually at a young age. It frequently leads to chronic kidney disease. Treatment options for cystinuria have been limited despite comprehensive understanding of its genetic pathophysiology. Currently available therapies suffer from either poor clinical adherence to the regimen or potentially serious adverse effects. Recently, we employed atomic force miscopy (AFM) to identify l-cystine dimethylester (CDME) as an effective molecular imposter of l-cystine, capable of inhibiting crystal growth in vitro. More recently, we demonstrated CDME’s efficacy in inhibiting l-cystine crystal growth in vivo utilizing a murine model of cystinuria. The application of AFM to discover inhibitors of crystal growth through structural mimicry suggests a novel approach to preventing and treating crystal diseases.

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Stone dissolution with D-penicillamine in cystinuria.

Abstract: A man aged 49 was admitted to hospital on 15 October 1964 with a two-day history of increasingly severe abdominal pain, vomiting, and diarrhoea. He had been admitted to hospital five years, six months, and two months previously on account of abdominal pain,

Cited by 27 publications

References 5 publications

Therapy modifies cystine kidney stones at the macroscopic scale. Do such alterations exist at the mesoscopic and nanometre scale?

Therapy modifies cystine kidney stones at the macroscopic scale. Do such alterations exist at the mesoscopic and nanometre scale?

Excretion of Trace Elements in Two Penicillamine‐treated Cases of Cystinuria

Cystine Growth Inhibition Through Molecular Mimicry: a New Paradigm for the Prevention of Crystal Diseases

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