Fibrosing colonopathy associated with cysteamine bitartrate delayed-release capsules in cystinosis patients.

BACKGROUND: The objective of this report is to identify and characterize cases of fibrosing colonopathy, a rare and underrecognized adverse event, associated with cysteamine delayed-release (DR) in patients with nephropathic cystinosis. METHODS: We searched the U.S. Food and Drug Administration Adverse Event Reporting System (FAERS) and the medical literature for postmarketing reports of fibrosing colonopathy associated with cysteamine through August 2, 2023. RESULTS: We identified four cases of fibrosing colonopathy reported with the use of cysteamine DR. The time to onset ranged from 12 to 31 months. In one case, the patient required surgery to have a resection of a section of the strictured colon and a diverting ileostomy. Fibrosing colonopathy was diagnosed by histopathology in two of the cases. CONCLUSIONS: Our case series identified the risk of fibrosing colonopathy in patients taking cysteamine DR and prompted regulatory action by the FDA. As outlined in changes to the U.S. prescribing information for cysteamine DR, healthcare professionals should be aware of the potential risk of fibrosing colonopathy with cysteamine DR, especially as symptoms can be non-specific leading to misdiagnosis or delayed diagnosis. If the diagnosis of fibrosing colonopathy is confirmed, consideration should be given to permanently discontinuing cysteamine DR and switching to cysteamine immediate-release treatment.

Post-operative non-steroidal anti-inflammatory drug use for pain in infant and paediatric cardiac surgery patients.

BACKGROUND: Pain control is an important element of care for patients after surgery, leading to better outcomes, quicker transitions to recovery, and improvement in quality of life. The purpose of this study was to evaluate the safety and efficacy of non-steroidal anti-inflammatory drugs in children after cardiac surgery. MATERIALS AND METHODS: Patients between the ages of 1 month and 18 years of age, who received intravenous or oral non-steroidal anti-inflammataory drugs after cardiac surgery, from November 2015 until September 2017 were included in this study. The primary endpoints were non-steroidal anti-inflammataory drug-associated renal dysfunction and post-operative bleeding. Secondary endpoints examined the effect of non-steroidal anti-inflammataory drug use on total daily dose of narcotics, number of intravenous PRN narcotic doses received, and pain assessment score. Data were analysed using descriptive statistics for frequencies and ranges. Multivariate analysis was performed to measure the association of all predictors and outcomes. Wilcoxon singed-rank test was performed for secondary outcomes. RESULTS: There was no association between the incidence of renal dysfunction and the use of or duration of non-steroidal anti-inflammataory drugs; in addition no association was found with increased chest tube output. There was a statistically significant reduction of patients' median Face, Legs, Activity, Cry, Consolability (FLACC) scores (2-0; p = 0.003), seen within first 24 hours after initiation of ketorolac, and a significant reduction of morphine requirements seen from day 1 to day 2 (0.3 mg/kg versus 0.1 mg/kg; p < 0.001) and number of as-needed doses. CONCLUSION: Non-steroidal anti-inflammataory drugs in paediatric cardiac surgery patients are safe and effective for post-operative pain management.

Effect of fish oil supplement administration method on tolerability and adherence: a randomized pilot clinical trial.

OBJECTIVES: Anecdotally, several strategies have been suggested in order to improve tolerability of fish oil supplements, but there is little evidence supporting any of these strategies. The aim of this study was to determine if there is a difference among four methods of oral administration of fish oil supplementation in terms of tolerability and adherence. METHODS: A randomized, prospective, open-label, four-arm pilot study was conducted on 60 healthy adult subjects randomized to different fish oil supplement administration methods with (1) milk, (2) food, (3) an empty stomach, and (4) frozen capsules prior to ingestion. Each subject was instructed to take two capsules three times daily for 30 consecutive days. Adherence was assessed by pill counts. Adverse effects were assessed by survey and patient exit interview. RESULTS: No apparent differences were demonstrated among the four administration groups in terms of adherence, reasons for non-adherence, or self-reported adverse effects. CONCLUSIONS: Method of administration did not affect rates of adherence or incidence of adverse effects in a small cohort of healthy adults taking fish oil supplement capsules for 30 days. TRIAL REGISTRATION: ClinicalTrials.gov NCT01471366. Registered November 16, 2011.