Sp026quantification of Dimethylsulfide Associated With Cysteamine Bitartate-Induced Halitosis Using Breath Analysis in Cystinosis Patients Treated With Delayed-Release and Immediate-Release Cysteamine Bitartrate

Greenbaum, Larry A.; Deschênes, Georges; Levtchenko, Elena; Besouw, Martine; Fosser, Cecilia; Confer, Nils; Zheng, Shuo; Checani, Gregg

doi:10.1093/ndt/gfx138.sp026

Cited by 3 publications

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“…A halitosis sub-study of a Phase III clinical trial with DR cysteamine bitartrate associated that formulation with a 26% reduction in exhaled dimethyl sulfide in comparison with immediate-release cysteamine bitartrate. 7 Neither this study nor the halitosis sub-study assessed halitosis as a factor in therapy compliance.…”

Section: Introductionmentioning

confidence: 95%

“…These beads are enteric coated with Eudragit L 30 D-55, an acid-resistant polymer that allows the bead to bypass dissolution in the acidic environment of the stomach while allowing dissolution in the more alkaline environment of the small intestine (pH >5.5). 7 This results in a predictable and reproducible pharmacokinetic profile that allows for more convenient dosing every 12 hours. 1 , 6 This coating has been used on a number of other medications, including esomeprazole magnesium trihydrate, tamsulosin hydrochloride, ranitidine hydrochloride, and diclofenac sodium.…”

Section: Introductionmentioning

confidence: 99%

“…1 , 6 This coating has been used on a number of other medications, including esomeprazole magnesium trihydrate, tamsulosin hydrochloride, ranitidine hydrochloride, and diclofenac sodium. 7 …”

Section: Introductionmentioning

confidence: 99%

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The effect of food and liquid pH on the integrity of enteric-coated beads from cysteamine bitartrate delayed-release capsules

Pavloff¹,

Hauser²,

Williams³

et al. 2018

DDDT

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BackgroundCysteamine bitartrate delayed-release (DR) capsule (Procysbi®) is approved for treatment of nephropathic cystinosis in the USA, Canada, and the EU. The capsules contain cysteamine bitartrate beads that are enteric coated with acid-resistant Eudragit L 30 D-55, preventing drug release in the acidic stomach environment while allowing dissolution of the beads in the more alkaline environment of the small intestine. Patients who have difficulty swallowing capsules can open capsules, sprinkle beads onto 4 ounces of a suitable food or liquid, gently mix, and consume the entire content within 30 minutes. Foods found to be suitable for administration, and described in the Procysbi US labeling, include fruit juices (except grapefruit juice), applesauce, and berry jelly; there are minor variations in the foods and liquids recommended by regulatory authorities in other countries. This study aimed to assess the stability of enteric-coated beads exposed to additional foods at different conditions to expand the list of suitable foods for drug administration.MethodsFor each test condition, beads from eight opened 75 mg cysteamine bitartrate DR capsules were gently mixed with test food and maintained at a prespecified temperature and duration; remaining undissolved beads were then recovered from the food. The recovered beads were split into two portions: one assayed for remaining drug content and the other subjected to dissolution testing to assess the effect on the drug-release profile.ResultsThe results show that bead integrity was maintained when mixed with foods at pH values <5.5 at all time points when refrigerated (2°C–8°C) and at room (20°C–22°C) and lukewarm (37°C–41°C) temperatures. Bead integrity was not maintained when mixed with foods at pH values of ≥5.5 at room temperature.ConclusionThe results from this in vitro dissolution study help in identifying additional foods that may be used for the administration of cysteamine bitartrate DR beads from opened capsules using the sprinkle method.

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Section: Introductionmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 99%

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The effect of food and liquid pH on the integrity of enteric-coated beads from cysteamine bitartrate delayed-release capsules

Pavloff¹,

Hauser²,

Williams³

et al. 2018

DDDT

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“…[ 23 ] and in a substudy conducted by Greenbaum et al. [ 24 ], where treatment with twice-daily ER cysteamine caused less halitosis than treatment with IR cysteamine. This finding is key, given that halitosis makes it difficult for patients to comply with treatment and greatly negatively affects their self-perception and social relationships, especially in adolescents and adults, where it has been demonstrated that adherence decreases compared with paediatric patients [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Switching from immediate- to extended-release cysteamine in patients with nephropathic cystinosis: from clinical trials to clinical practice

Ariceta,

Santos,

López Muñiz

et al. 2024

Clinical Kidney Journal

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Background The purpose of this study is to evaluate the effectiveness and safety of switching from immediate-release (IR) to extended-release (ER) cysteamine in patients with nephropathic cystinosis (NC) in Spain. Methods observational, retrospective, multicentre study in NC patients that received IR cysteamine for at least 12 months, switched to ER cysteamine, and received it for at least 6 months before inclusion. Results Data was collected from 9 patients (4 children, 5 adults) 36 months before and after the switch. Despite the highly selected population, an improvement in growth, particularly in children (P < 0.001), and a significant reduction in hospitalization days (P < 0,0001) was observed. A decrease in halitosis, body odor, and gastrointestinal effects was reported in most of the patients who suffered before the switch, and the use of Proton pump inhibitors (PPI) decreased in some patients. The estimated glomerular filtration rate (eGFR) remained stable in patients with preserved kidney function. No significant changes in white blood cell (WBC) cystine levels were observed after the switch. There was no significant difference in the cysteamine dose received. However, some patients were receiving < 50% of recommended doses of cysteamine before and after the switch and showed elevated levels of WBC cystine. Conclusions Switching from IR to ER cysteamine in clinical practice reduces hospital stays, improves nutritional status and growth in paediatric patients, and could help to enhance treatment tolerability by reducing side effects. Furthermore, the dosing of ER cysteamine could promote therapeutic compliance and positively affect the quality of life of the NC population.

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Gastrointestinal challenges in nephropathic cystinosis: clinical perspectives

Joseph,

Stein,

Stein

2024

Pediatr Nephrol

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Gastrointestinal (GI) sequelae, such as vomiting, hyperacidity, dysphagia, dysmotility, and diarrhea, are nearly universal among patients with nephropathic cystinosis. These complications result from disease processes (e.g., kidney disease, cystine crystal accumulation in the GI tract) and side effects of treatments (e.g., cysteamine, immunosuppressive therapy). GI involvement can negatively impact patient well-being and jeopardize disease outcomes by compromising drug absorption and patient adherence to the strict treatment regimen required to manage cystinosis. Given improved life expectancy due to advances in kidney transplantation and the transformative impact of cystine-depleting therapy, nephrologists are increasingly focused on addressing extra-renal complications and quality of life in patients with cystinosis. However, there is a lack of clinical data and guidance to inform GI-related monitoring, interventions, and referrals by nephrologists. Various publications have examined the prevalence and pathophysiology of selected GI complications in cystinosis, but none have summarized the full picture or provided guidance based on the literature and expert experience. We aim to comprehensively review GI sequelae associated with cystinosis and its treatments and to discuss approaches for monitoring and managing these complications, including the involvement of gastroenterology and other disciplines.

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Sp026quantification of Dimethylsulfide Associated With Cysteamine Bitartate-Induced Halitosis Using Breath Analysis in Cystinosis Patients Treated With Delayed-Release and Immediate-Release Cysteamine Bitartrate

Cited by 3 publications

References 0 publications

The effect of food and liquid pH on the integrity of enteric-coated beads from cysteamine bitartrate delayed-release capsules

The effect of food and liquid pH on the integrity of enteric-coated beads from cysteamine bitartrate delayed-release capsules

Switching from immediate- to extended-release cysteamine in patients with nephropathic cystinosis: from clinical trials to clinical practice

Gastrointestinal challenges in nephropathic cystinosis: clinical perspectives

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