supplier [30:30 days - 11 months 9 (39%) =1 - 13 years 5 (22%) NAGS gene mutations by DNA testing homozygous 14 (61%) heterozygous 4 (17%) Not available 5 (22%) Patients current treatment status On-going 18 (78%) Discontinued 5 (22%) The clinical observations in the 23 patient case series were retrospective, unblinded and uncontrolled and preclude any meaningful formal statistical analyses of the data. However, short-term efficacy was evaluated using mean and median change in plasma ammonia levels from baseline to days 1 to 3. Persistence of efficacy was evaluated using long-term mean and median change in plasma ammonia level. Table 3 summarizes the plasma ammonia levels at baseline, days 1 to 3 post-Carbaglu treatment, and long-term Carbaglu treatment for 13 evaluable patients. Of the 23 NAGS deficiency patients who received treatment with Carbaglu , a subset of 13 patients who had both well documented plasma ammonia levels prior to Carbaglu treatment and after long-term treatment with Carbaglu were selected for analysis. All 13 patients had abnormal ammonia levels at baseline. The overall mean baseline plasma ammonia level was 271 mol/L. By day 3, normal plasma ammonia levels were attained in patients for whom data were available. Long-term efficacy was measured using the last reported plasma ammonia level for each of the 13 patients analyzed (median length of treatment was 6 years; range 1 to 16 years). The mean and median ammonia levels were 23 mol/L and 24 mol/L, respectively, after a mean treatment duration of 8 years. Table 3: Plasma ammonia levels at baseline and after treatment with Carbaglu Timepoint Statistics (N = 13*) Ammonia** (mol/L) Baseline (prior to first treatment with Carbaglu) N 13 Mean (SD) 271 (359) Median 157 Range 72-1428 Missing Data 0 Day 1 N 10 Mean (SD) 181 (358) Median 65 Range 25-1190 Missing Data 3 Day 2 N 8 Mean (SD) 69 (78) Median 44 Range 11-255 Missing Data 5 Day 3 N 5 Mean (SD) 27 (11) Median 25 Range 12-42 Missing Data 8 Long-term Mean: 8 years Median: 6 years 1 to 16 years (last available value on Carbaglu treatment) N 13 Mean (SD) 23 (7) Median 24 Range 9-34 Missing Data 0 *13/23 patients with complete short-term and long-term plasma ammonia documentation **Mean ammonia normal range: 5 to 50 mol/L The mean plasma ammonia level at baseline and the decline that is observed after treatment with Carbaglu in 13 evaluable patients with NAGS deficiency is illustrated in Figure 1. Figure 1: Ammonia response for 13 evaluable NAGS deficiency patients at baseline and after treatment with Carbaglu How Supplied/Storage and Handling How Supplied Carbaglu is a white and elongated tablet, scored and coded "C" on one side. Each tablet contains 200 mg of carglumic acid. Carbaglu is available in 5 or 60 tablets in a polypropylene bottle with polyethylene cap and desiccant unit. NDC 52276-312-05 Bottle of 5 tablets NDC 52276-312-60 Bottle of 60 tablets Storage Before opening, store refrigerated at 2-8 C (36-46 F). After first opening of the container: Do not refrigerate, do not store above 30 C (86 F). Keep the container tightly closed in order to protect from moisture. Write the date of opening on the tablet container. Do not use after the expiration date stated on the tablet container. Discard one month after first opening. Patient Counseling Information Physicians should inform patients and caregivers about the following instructions for safe use of Carbaglu : Carbaglu tablets should not be swallowed whole or crushed. Each tablet should be dispersed in a minimum of 2.5 mL of water. Carbaglu tablets do not dissolve completely in water and undissolved particles of the tablet may remain in the mixing container. The mixing container should be rinsed with additional volumes of water and the contents swallowed immediately. Before opening, store in a refrigerator 2-8 C (36-46 F). Keep the container tightly closed in order to protect from moisture. After first opening of the container: do not refrigerate, do not store above 30 C (86 F). Write the date of opening on the tablet container. Discard one month after first opening. Do not use after the expiration date stated on the tablet container. Physicians should also advise patients and caregivers that: When plasma ammonia levels have normalized, dietary protein intake can usually be increased with the goal of unrestricted protein intake. Human milk-feeding is not recommended. The most common adverse reactions are vomiting, abdominal pain, pyrexia, tonsilitis, anemia, ear infection, diarrhea, nasopharyngitis, and headache. Supplied by: Orphan Europe SARL Paris, France Distributed by: Recordati Rare Diseases, Inc. Lebanon, NJ 08833 For drug or ordering information please call Accredo Health Group Inc., Customer Service at 1-888-454-8860. Licensed to: Recordati Rare Diseases, Inc. Lebanon, NJ 08833 Carbaglu is a registered Trademark of RRD PACKAGING AND LABELING Carbaglu bottle label - 60 Tablets in Bottle Carbaglu carton label - 60 Tablets in Bottle Carbaglu bottle label - 5 Tablets in Bottle Carbaglu carton label - 5 Tablets in Bottle Carbaglu carglumic acid tablet Product Information Product Type HUMAN PRESCRIPTION DRUG LABEL Item Code (Source) NDC:52276-312 Route of Administration ORAL DEA Schedule Active Ingredient/Active Moiety Ingredient Name Basis of Strength Strength Carglumic acid (Carglumic acid) Carglumic acid 200 mg Inactive Ingredients Ingredient Name Strength Cellulose, Microcrystalline 270 mg Sodium Lauryl Sulfate 0.50 mg HYPROMELLOSES 4.0 mg Croscarmellose sodium 19.0 mg Silicon Dioxide 1.5 mg SODIUM STEARYL FUMARATE 5.0 mg Product Characteristics Color white (Bar-shaped) Score 3 pieces Shape RECTANGLE (Bar-shaped) Size 18mm Flavor Imprint Code Contains Packaging # Item Code Package Description 1 NDC:52276-312-05 5 TABLET in 1 BOTTLE 2 NDC:52276-312-60 60 TABLET in 1 BOTTLE Marketing Information Marketing Category Application Number or Monograph Citation Marketing Start Date Marketing End Date NDA NDA022562 03/18/2010 Labeler - Orphan Europe, SARL (767598352) Registrant - Orphan Europe, SARL (767598352) Establishment Name Address ID/FEI Operations Aesica Pharmaceuticals GmbH 341737465 manufacture(52276-312) Revised: 11/2015 Orphan Europe, SARL Next Pregnancy Warnings Print this page Add to My Med List More about Carbaglu (carglumic acid) Side Effects During Pregnancy Dosage Information Support Group Pricing & Coupons En Español 0 Reviews Add your own review/rating Drug class: miscellaneous uncategorized agents Consumer resources Carbaglu Carbaglu (Advanced Reading) Professional resources Carbaglu (AHFS Monograph) Related treatment guides Hyperammonemia] FEATURED: CAR-T Cell Therapy Overview Mechanism of Action KTE-C19 Studies KTE-C19 Cancer Targets Adverse Events Manufacturing Drug Status Rx Availability Prescription only C Pregnancy Category Risk cannot be ruled out N/A CSA Schedule Not a controlled drug 7 years Approval History FDA approved 2010 Drug Class Miscellaneous uncategorized agents Related Drugs miscellaneous uncategorized agents Accutane , isotretinoin , anagrelide , Esbriet , Claravis Hyperammonemia sodium benzoate / sodium phenylacetate , carglumic acid , More... 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