Xuriden Drug Information
Generic name: URIDINE TRIACETATE
Uses of Xuriden
XURIDEN ® is indicated in adult and pediatric patients for the treatment of hereditary orotic aciduria.
Dosage & Administration of Xuriden
Recommended Dosage
The recommended starting dosage of oral XURIDEN is 60 mg/kg once daily. Increase the dosage of XURIDEN to 120 mg/kg (not to exceed 8 grams) once daily for insufficient efficacy, such as occurrence of one of the following: Levels of orotic acid in urine remain above normal or increase above the usual or expected range for the patient Laboratory values (e.g., red blood cell or white blood cell indices) affected by hereditary orotic aciduria show evidence of worsening Worsening of other signs or symptoms of the disease The XURIDEN dose to be administered at the 60 mg/kg and 120 mg/kg dose levels by body-weight is presented in Tables 1 and 2. A 2 gram packet of XURIDEN contains approximately ¾ teaspoon of XURIDEN.
Therefore, in the tables below for patients requiring doses in multiples of 2 grams (¾ teaspoon) an entire packet(s) may be administered without weighing or measuring. XURIDEN Daily Dose Based on Body Weight (kg) 8
Preparation and Administration Preparation Measure the dose using either a scale accurate to at least 0.1 gram, or a graduated teaspoon, accurate to the fraction of the dose to be administered. Once the measured dose has been removed from the XURIDEN packet, discard the unused portion of granules. Do not use any granules left in the open packet.
Administration with Food Place 3 to 4 ounces of applesauce, pudding or yogurt in a small clean container. Mix the measured amount of granules in the applesauce, pudding or yogurt Swallow applesauce/pudding/yogurt immediately. Do not chew the granules.
Do not save the applesauce/pudding/yogurt for later use. Drink at least 4 ounces of water. Administration in Milk or Infant Formula XURIDEN can be mixed with milk or infant formula instead of the soft foods described above for patients receiving up to 3/4 teaspoon (2 grams) of XURIDEN.
After weighing the dose of XURIDEN: Pour 5 mL of milk or infant formula into a 30 mL medicine cup. Insert the tip of the oral syringe into the medicine cup and draw up 5 mL of milk/infant formula into the syringe. Hold the syringe with the tip pointing upward.
Pull down on the plunger until the plunger reaches 10 mL. This will add air to the syringe. Place the cap over the tip of the syringe.
Then invert the syringe so the syringe tip is pointing down, and remove the plunger. Pour the measured amount of XURIDEN granules into the syringe barrel and reinsert the syringe plunger. Do not push up on the plunger.
Gently swirl the syringe to mix the XURIDEN granules with the liquid. Turn the syringe so the syringe tip is pointing up. Then remove the syringe cap and push up on the plunger until the plunger reaches the 5 mL mark.
This will remove air from the syringe. Place the tip of the syringe in the patient's mouth between the cheek and gum at the back of the mouth. Gently push the plunger all the way down.
Refill the syringe with another 5 mL of milk/infant formula. Gently swirl the syringe to rinse any remaining XURIDEN granules from the syringe barrel. Follow with a bottle of milk or infant formula, if desired.
| Patient Weight | Table 1: XURIDEN 60 mg/kg total daily dose by weight category in the tables was rounded to achieve the approximate dose level Dose Level | |
|---|---|---|
| Kilograms | Dose to be Administered Using a Scale (grams) | Dose in Teaspoons |
| up to 5 | 0.4 | ⅛ |
| 6-10 | 0.4 to 0.6 | ¼ |
| 11-15 | 0.7 to 0.9 | ½ |
| 16-20 | 1 to 1.2 | |
| 21-25 | 1.3 to 1.5 | |
| 26-30 | 1.6 to 1.8 | ¾ |
| 31-35 | 1.9 to 2.1 may use 1 entire 2 gram packet without weighing or measuring | |
| 36-40 | 2.2 to 2.4 | 1 |
| 41-45 | 2.5 to 2.7 | |
| 46-50 | 2.8 to 3 | |
| 51-55 | 3.1 to 3.3 | 1 ¼ |
| 56-60 | 3.4 to 3.6 | |
| 61-65 | 3.7 to 3.9 may use 2 entire 2 gram packets without weighing or measuring | 1 ½ |
| 66-70 | 4 to 4.2 | |
| 71-75 | 4.3 to 4.5 | |
| Above 75 | 6 may use 3 entire 2 gram packets without weighing or measuring | 2 |
| Patient Weight | Table 2: XURIDEN 120 mg/kg total daily dose by weight category in the tables was rounded to achieve the approximate dose level Dose Level | |
|---|---|---|
| Kilograms | Dose to be Administered Using a Scale (grams) | Dose in Teaspoons |
| up to 5 | 0.8 | ¼ |
| 6-10 | 0.8 to 1.2 | ½ |
| 11-15 | 1.4 to 1.8 | ¾ |
| 16-20 | 2 to 2.4 | 1 |
| 21-25 | 2.6 to 3 | |
| 26-30 | 3.2 to 3.6 | 1 ¼ |
| 31-35 | 3.8 to 4.2 may use 2 entire 2 gram packets without weighing or measuring | 1 ½ |
| 36-40 | 4.4 to 4.8 | 1 ¾ |
| 41-45 | 5 to 5.4 | 2 may use 3 entire 2 gram packets without weighing or measuring |
| 46-50 | 5.6 to 6 | |
| 51-55 | 6.2 to 6.6 | 2 ¼ |
| 56-60 | 6.8 to 7.2 | 2 ½ |
| 61-65 | 7.4 to 7.8 | |
| 66-70 | 8 | 2 ¾ may use 4 entire 2 gram packets without weighing or measuring |
| 71-75 | 8 | |
| Above 75 | 8 | |
Side Effects of Xuriden
Clinical Trials Experience
Because clinical trials are conducted under widely varying conditions and using a wide range of doses, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice. All patients continued to receive XURIDEN for at least 24 months at dosages of up to 120 mg/kg once daily. No adverse reactions were reported with XURIDEN.
Pregnancy Safety for Xuriden
Pregnancy Risk Summary There are no available data on XURIDEN use in pregnant women to inform a drug-associated risk. When administered orally to pregnant rats during the period of organogenesis, uridine triacetate at doses similar to the maximum recommended human dose (MRHD) of 120 mg/kg per day was not teratogenic and did not produce adverse effects on embryo-fetal development. The background risk of major birth defects and miscarriage for the indicated population are unknown.
In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is respectively. Data Animal Data In an embryo-fetal development study, uridine triacetate was administered orally to pregnant rats during the period of organogenesis at doses up to 2000 mg/kg per day (about 2.7 times the maximum recommended human dose (MRHD) of 120 mg/kg per day on a body surface area basis). There was no evidence of teratogenicity or harm to the fetus and no effect on maternal body weight and overall health.
Pediatric Use of Xuriden
Pediatric Use The safety and effectiveness of XURIDEN have been established in pediatric patients. Use of XURIDEN is supported by a single open-label clinical trial of uridine triacetate in 4 patients and a retrospective review of the clinical course of 18 patients with hereditary orotic aciduria who were treated with uridine beginning at ages 2 months to 12 years. There are no apparent differences in clinical response between adults and pediatric patients with hereditary orotic aciduria treated with uridine, however, data are limited.
Clinical Studies of Xuriden
Three patients were previously treated with uridine and were switched at study entry to XURIDEN. All patients were administered XURIDEN orally at a daily dosage of 60 mg/kg once daily. The study assessed changes in the patients' pre-specified hematologic parameters during the initial 6-week period and the extension phase.
For patients switched from oral uridine to oral XURIDEN (Patients 1, 2, and 3), the primary endpoint was stability of the hematologic parameter; for the treatment-naïve patient (Patient 4), the primary endpoint was improvement of the hematologic parameter. Secondary endpoints were urine orotic acid and orotidine levels, and growth (height and weight) for all patients. Table 4 summarizes the changes in the patients' pre-specified hematologic parameters at Week 6 and 24 Months compared to baseline.
After 6 weeks of treatment, Patients 1 and 3 met the pre-specified criteria for stability of the hematologic parameter. When Patient 2 was switched from uridine to XURIDEN treatment, the pre-specified parameter of white blood cell count remained stable; however, documentation of a low white blood cell count prior to uridine initiation was not available. Patient 4 did not meet the pre-specified endpoint of improvement of the hematologic parameter.
By 24 months of treatment with XURIDEN, the pre-specified hematologic parameter for Patient 1 worsened, but the assessment was confounded by Cohen syndrome with associated neutropenia, which was diagnosed after the patient completed the study. The pre-specified hematologic parameters for Patients 2, 3, and 4 remained essentially unchanged. All four patients had normal urine orotidine levels at baseline which remained stable after 6 weeks of treatment with XURIDEN.
After 24 months of treatment, urine orotic acid and orotidine levels remained stable in all four patients. The treatment effect of XURIDEN on growth was assessed in the three pediatric patients (Patients 1, 3, and 4). At baseline, weight and height were at or below the lower limit of normal for age for all three patients.
After 24 months of treatment, each patient's growth parameters remained essentially unchanged. Case reports Nineteen case reports of patients with hereditary orotic aciduria have been documented in published literature. Eighteen patients were diagnosed as infants or children between the ages of 2 months and 12 years and were treated with exogenous sources of uridine.
One patient, diagnosed at age 28, was not treated with exogenous uridine. All 19 patients presented with significantly elevated levels of urinary orotic acid. Oral administration of exogenous sources of uridine was reported to significantly improve hematologic abnormalities (megaloblastic anemia, leukopenia and neutropenia) within 2 to 3 weeks in almost all documented cases when administered in sufficient amounts.
Concentrations of urinary orotic acid were significantly reduced within 1 to 2 weeks of initiating uridine replacement therapy. Some fluctuation in levels of urinary orotic acid were observed, but always at much lower levels than those reported prior to treatment. Improvements in body weight were also documented over time with continued uridine replacement therapy.
The effects of exogenous uridine were maintained over months and years, as long as treatment continued at sufficient doses (with appropriate dose increases based on body weight increases). Most hematologic abnormalities and orotic aciduria reappeared within days up to 2 or 3 weeks when administration of uridine was stopped or the dose was reduced. If treatment was interrupted for longer periods, body weight growth receded.
If absolute dosages were not adjusted adequately to compensate for body weight gains, signs and symptoms of hereditary orotic aciduria recurred.
| Patient | Pre-specified Hematologic Parameter (Age-specific reference range) | Baseline (Day 0) | 6 Weeks (% Change from Baseline) | 24 Months (% Change from Baseline) |
|---|---|---|---|---|
| Patient 1 | Neutrophil Count (1.5 to 8.0 x10 3 /mm 3 ) | 0.95 | 0.81 (-15%) | 0.61 (-36%) |
| Neutrophil % (26 to 48%) | 21 | 23 (10%) | 13 (-38%) | |
| Patient 2 | White Blood Cell Count (3.8 to 10.6 x10 9 /L) | 7.8 | 7.4 (-5%) | 8.6 (10%) |
| Patient 3 | Mean Corpuscular Volume (75 to 91 fL) | 109.9 | 108.5 (-1%) | 108.8 (-1%) |
| Patient 4 | Mean Corpuscular Volume (72 to 90 fL) | 114.6 | 113.4 (-1%) | 112.9 (-1.5%) |
Drug information sourced from the FDA. This content is for informational purposes only and does not constitute medical advice. Consult a healthcare professional before making any medication decisions.
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