PedAM
Pediatric Disease Annotations & Medicines
| hyperammonemia | ||||
| Disease ID | 1664 |
|---|---|
| Disease | hyperammonemia |
| Definition | Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA. |
| Synonym | elevated ammonia level high blood ammonia levels hyperammonaemia hyperammonaemia, nos hyperammonemia (disorder) hyperammonemia [disease/finding] hyperammonemia [dup] (disorder) hyperammonemia, nos increased blood ammonia |
| UMLS | C0220994 |
| MeSH | |
| SNOMED-CT | |
| Comorbidity | UMLS | Disease | Sentences' Count(Total Sentences:30) C0019151 | hepatic encephalopathy | 9 C0023890 | cirrhosis | 3 C0026764 | multiple myeloma | 2 C0006112 | metabolic encephalopathy | 2 C0023890 | liver cirrhosis | 2 C0154246 | urea cycle disorders | 2 C0268575 | isovaleric acidemia | 2 C0026764 | myeloma | 2 C0020459 | hyperinsulinism | 1 C0268583 | methylmalonic acidemia | 1 C0014544 | epileptic seizure | 1 C0020456 | hyperglycemia | 1 C0014544 | epilepsy | 1 C0268579 | propionic acidemia | 1 C0020598 | hypoglycemia | 1 C1527311 | brain edema | 1 C0024623 | gastric cancer | 1 C0268542 | ornithine transcarbamylase deficiency | 1 C0019204 | hepatocellular carcinoma | 1 C0023449 | acute lymphoblastic leukemia | 1 C0023418 | leukemia | 1 C0158981 | neonatal diabetes mellitus | 1 C0039445 | rendu-osler-weber syndrome | 1 C0023895 | liver disease | 1 C0001126 | renal tubular acidosis | 1 C0334287 | fibrolamellar hepatocellular carcinoma | 1 C0023448 | lymphoblastic leukemia | 1 C0014544 | epileptic seizures | 1 C0003857 | arteriovenous malformation | 1 C0206754 | neuroendocrine tumor | 1 |
| Curated Gene | Entrez_id | Symbol | Resource(Total Genes:5) |
| Inferring Gene | (Waiting for update.) |
| Text Mined Gene | (Waiting for update.) |
| Locus | (Waiting for update.) |
| Disease ID | 1664 |
|---|---|
| Disease | hyperammonemia |
| Manually Symptom | (Waiting for update.) |
| Text Mined Symptom | (Waiting for update.) |
Manually Genotype(Total Text Mining Genotypes:0) |
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| (Waiting for update.) |
All Snps(Total Genotypes:1) | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| snpId | pubmedId | geneId | geneSymbol | diseaseId | sourceId | sentence | score | Year | geneSymbol_dbSNP | CHROMOSOME | POS | REF | ALT |
| rs72554356 | NA | 5009 | OTC | umls:C0220994 | CLINVAR | NA | 0.24 | NA | OTC | X | 38381417 | C | T |
GWASdb Annotation(Total Genotypes:0) | |
|---|---|
| (Waiting for update.) | |
GWASdb Snp Trait(Total Genotypes:0) | |
|---|---|
| (Waiting for update.) | |
Mapped by lexical matching(Total Items:0) |
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| (Waiting for update.) |
Mapped by homologous gene(Total Items:0) |
|---|
| (Waiting for update.) |
Chemical(Total Drugs:11) | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| CUI | ChemicalName | ChemicalID | CasRN | DiseaseName | DiseaseID | DirectEvidence | PubMedIDs | ||
| C0220994 | acetaminophen | D000082 | 103-90-2 | hyperammonemia | MESH:D022124 | marker/mechanism | 10447915 | ||
| C0220994 | carbamazepine | D002220 | 298-46-4 | hyperammonemia | MESH:D022124 | marker/mechanism | 3341477 | ||
| C0220994 | fluorouracil | D005472 | 51-21-8 | hyperammonemia | MESH:D022124 | marker/mechanism | 10327032 | ||
| C0220994 | leucovorin | D002955 | 1958/5/9 | hyperammonemia | MESH:D022124 | therapeutic | 18173932 | ||
| C0220994 | lactulose | D007792 | 4618-18-2 | hyperammonemia | MESH:D022124 | therapeutic | 11430560 | ||
| C0220994 | methotrexate | D008727 | 1959/5/2 | hyperammonemia | MESH:D022124 | marker/mechanism | 18173932 | ||
| C0220994 | spironolactone | D013148 | 1952/1/7 | hyperammonemia | MESH:D022124 | marker/mechanism | 600713 | ||
| C0220994 | thiopental | D013874 | 76-75-5 | hyperammonemia | MESH:D022124 | marker/mechanism | 16704891 | ||
| C0220994 | topiramate | C052342 | 97240-79-4 | hyperammonemia | MESH:D022124 | marker/mechanism | 10636156 | ||
| C0220994 | valproic acid | D014635 | 99-66-1 | hyperammonemia | MESH:D022124 | marker/mechanism | 10636156 | ||
| C0220994 | valproic acid | D014635 | 99-66-1 | hyperammonemia | MESH:D022124 | therapeutic | 17412645 | ||
FDA approved drug and dosage information(Total Drugs:12) | ||||||||
|---|---|---|---|---|---|---|---|---|
| DiseaseID | Drug_name | active_ingredients | strength | Dosage Form/Route | Marketing Status | TE code | RLD | RS |
| MESH:D022124 | topamax | topiramate | 100MG | TABLET;ORAL | Prescription | AB | Yes | Yes |
| MESH:D022124 | topamax | topiramate | 15MG | CAPSULE;ORAL | Prescription | AB | Yes | No |
| MESH:D022124 | topamax | topiramate | 100MG | TABLET;ORAL | Prescription | AB | Yes | Yes |
| MESH:D022124 | topamax | topiramate | 15MG | CAPSULE;ORAL | Prescription | AB | Yes | No |
| MESH:D022124 | topamax | topiramate | 100MG | TABLET;ORAL | Prescription | AB | Yes | Yes |
| MESH:D022124 | topamax | topiramate | 15MG | CAPSULE;ORAL | Prescription | AB | Yes | No |
| MESH:D022124 | topamax | topiramate | 100MG | TABLET;ORAL | Prescription | AB | Yes | Yes |
| MESH:D022124 | topamax | topiramate | 15MG | CAPSULE;ORAL | Prescription | AB | Yes | No |
| MESH:D022124 | ofirmev | acetaminophen | 1GM/100ML (10MG/ML) | SOLUTION;IV (INFUSION) | Prescription | AP | Yes | Yes |
| MESH:D022124 | ofirmev | acetaminophen | 1GM/100ML (10MG/ML) | SOLUTION;IV (INFUSION) | Prescription | AP | Yes | Yes |
| MESH:D022124 | acetaminophen | acetaminophen | 650MG | SUPPOSITORY;RECTAL | Over-the-counter | None | Yes | Yes |
| MESH:D022124 | acetaminophen | acetaminophen | 650MG | SUPPOSITORY;RECTAL | Over-the-counter | None | Yes | Yes |
FDA labeling changes(Total Drugs:12) | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| DiseaseID | Pediatric_Labeling_Date | Trade_Name | Generic_Name_or_Proper_Name | Indications Studied | Label Changes Summary | Product Labeling | BPCA(B) | PREA(P) | BPCA(B) and PREA(P) | Pediatric Rule (R) | Sponsor | Pediatric Exclusivity Granted Date | NNPS |
| MESH:D022124 | 12/22/2009 | topamax | topiramate | Migraine Prophylaxis | Safety and effectiveness for migraine prevention in pediatric patients have not been established Dose-related increased shift in serum creatinine in adolescent patients occurred in a clinical study Information added to Warnings and Precautions and Pediatric Use | Labeling | - | P | - | - | Ortho-McNeil-Janssen | - | FALSE' |
| MESH:D022124 | 12/22/2009 | topamax | topiramate | Migraine Prophylaxis | Safety and effectiveness for migraine prevention in pediatric patients have not been established Dose-related increased shift in serum creatinine in adolescent patients occurred in a clinical study Information added to Warnings and Precautions and Pediatric Use | Labeling | - | P | - | - | Ortho-McNeil-Janssen | - | FALSE' |
| MESH:D022124 | 12/22/2009 | topamax | topiramate | Adjunctive Treatment for Partial Onset Epilepsy in Infants and Toddlers 1 to 24 months | Effectiveness was not demonstrated as adjunctive therapy in a randomized, double-blind trial in infants/toddlers 1 to 24 months of age with refractory partial onset seizures Trials in infants/toddlers 1 to 24 months suggested some adverse reactions/toxicities not previously observed in older pediatric patients and adults; i.e, growth/length retardation, certain clinical laboratory abnormalities, and other adverse reactions/toxicities that occurred with a greater frequency and/or greater severity than had been recognized previously from studies in older pediatric patients or adults for various indications. Information added to Warnings and Precautions and Pediatric Use | Labeling | B | - | - | - | Ortho-McNeil-Janssen | 07/24/2008 | FALSE' |
| MESH:D022124 | 12/22/2009 | topamax | topiramate | Adjunctive Treatment for Partial Onset Epilepsy in Infants and Toddlers 1 to 24 months | Effectiveness was not demonstrated as adjunctive therapy in a randomized, double-blind trial in infants/toddlers 1 to 24 months of age with refractory partial onset seizures Trials in infants/toddlers 1 to 24 months suggested some adverse reactions/toxicities not previously observed in older pediatric patients and adults; i.e, growth/length retardation, certain clinical laboratory abnormalities, and other adverse reactions/toxicities that occurred with a greater frequency and/or greater severity than had been recognized previously from studies in older pediatric patients or adults for various indications. Information added to Warnings and Precautions and Pediatric Use | Labeling | B | - | - | - | Ortho-McNeil-Janssen | 07/24/2008 | FALSE' |
| MESH:D022124 | 07/15/2011 | topamax | topiramate | Monotherapy for partial onset or primary generalized tonic-clonic seizures | Expanded age range down to 2 years; previously approved for monotherapy for partial onset or primary generalized tonic-clonic seizures in patients10 years and older Information on weight based dosing in 2 to < 10 yearsPostmarketing study | Labeling | - | P | - | - | Janssen | - | FALSE' |
| MESH:D022124 | 07/15/2011 | topamax | topiramate | Monotherapy for partial onset or primary generalized tonic-clonic seizures | Expanded age range down to 2 years; previously approved for monotherapy for partial onset or primary generalized tonic-clonic seizures in patients10 years and older Information on weight based dosing in 2 to < 10 yearsPostmarketing study | Labeling | - | P | - | - | Janssen | - | FALSE' |
| MESH:D022124 | 03/28/2014 | topamax | topiramate | Prophylaxis of migraine headache | Approved for use in pediatric patients 12 years and older Safety and effectiveness in pediatric patients less than12 years have not been established for the prophylaxis treatment of migraine headache In the adolescent migraine trials (12 to 17 years), the most commonly observed adverse reactions were: paresthesia, upper respiratory tract infection, anorexia, and abdominal pain The most common cognitive adverse reaction in pooled double-blind studies in adolescent patients 12 to 17 years was difficulty with concentration/attention Markedly abnormally low serum bicarbonate values indicative of metabolic acidosis were reported in topiramate-treated adolescent migraine patients In topiramate-treated patients 12 to 17 years compared to placebo-treated patients, abnormally increased results were more frequent for creatinine, BUN, uric acid, chloride, ammonia, total protein, and platelets. Abnormally decreased results were observed with topiramate vs placebo treatment for phosphorus and bicarbonate Notable changes (increases and decreases) from baseline in systolic blood pressure, diastolic blood pressure, and pulse were observed more commonly in adolescents treated with topiramate compared to adolescents treated with placebo Information on dosing, adverse reactions, laboratory abnormalities, and clinical trials Postmarketing study | Labeling | - | P | - | - | Janssen | - | FALSE' |
| MESH:D022124 | 03/28/2014 | topamax | topiramate | Prophylaxis of migraine headache | Approved for use in pediatric patients 12 years and older Safety and effectiveness in pediatric patients less than12 years have not been established for the prophylaxis treatment of migraine headache In the adolescent migraine trials (12 to 17 years), the most commonly observed adverse reactions were: paresthesia, upper respiratory tract infection, anorexia, and abdominal pain The most common cognitive adverse reaction in pooled double-blind studies in adolescent patients 12 to 17 years was difficulty with concentration/attention Markedly abnormally low serum bicarbonate values indicative of metabolic acidosis were reported in topiramate-treated adolescent migraine patients In topiramate-treated patients 12 to 17 years compared to placebo-treated patients, abnormally increased results were more frequent for creatinine, BUN, uric acid, chloride, ammonia, total protein, and platelets. Abnormally decreased results were observed with topiramate vs placebo treatment for phosphorus and bicarbonate Notable changes (increases and decreases) from baseline in systolic blood pressure, diastolic blood pressure, and pulse were observed more commonly in adolescents treated with topiramate compared to adolescents treated with placebo Information on dosing, adverse reactions, laboratory abnormalities, and clinical trials Postmarketing study | Labeling | - | P | - | - | Janssen | - | FALSE' |
| MESH:D022124 | 2/11/2010 | ofirmev | acetaminophen | Management of mild-to-moderate pain, for the management of moderate-to-severe pain with adjunctive opioid analgesics, and for the reduction of fever | The safety and effectiveness of Ofirmev for the treatment of acute pain and fever in pediatric patients ages 2 years and older is supported by evidence from adequate and well-controlled studies of Ofirmev in adults. Additional safety and PK data was collected in 355 from premature neonates to adolescents. The effectiveness of Ofirmev for the treatment of acute pain and fever has not been studied in pediatric patients < 2 years of age.The PK exposure of Ofirmev observed in children and adolescents is similar to adults, but higher in neonates and infants. Dosing simulations from PK data in infants and neonates suggest that dose reductions of 33% in infants 1 month to < 2 years of age, and 50% in neonates up to 28 days, with a minimum dosing interval of 6 hours, will produce a PK exposure similar to that observed in children age 2 years and olderMost common adverse reactions in pediatric patients were nausea, vomiting, constipation, pruritus, agitation, and atelectasis.Information on dosing, clinical studies, adverse reactions and PK parametersNew dosage form and route of administration | Labeling | - | P | - | - | Cadence | - | FALSE' |
| MESH:D022124 | 01/27/2017 | ofirmev | acetaminophen | Treatmeny of pain and fever in pediatric patients birth to 2 years | Treatment of pain Efficacy was not demonstrated in pediatric patients younger than 2 years in a double-blind, placebo-controlled study of 198 pediatric patients younger than 2 years. Pediatric patients less than 2 years of age, including neonates from 28 to 40 weeks gestational age at birth, were randomized to receive opioid plus acetaminophen or opioid plus placebo. No difference in analgesic effect of intravenous acetaminophen, measured by assessment of reduced need for additional opioid treatment for pain control, was observed. Treatment of fever The safety and effectiveness for the treatment of fever in pediatric patients, including premature neonates born at 32 weeks or greater gestation is supported by adequate and well-controlled studies of Ofirmev in adults, clinical studies in 244 pediatric patients 2 years and older, and safety and pharmacokinetic data from 239 patients younger than 2 years including neonates 32 weeks or greater gestational age. Information on dosing, clinical trials. Postmarketing study. | Labeling | - | - | B,P | - | Mallinckrodt | 11/7/2016 | FALSE |
| MESH:D022124 | 2/11/2010 | ofirmev | acetaminophen | Management of mild-to-moderate pain, for the management of moderate-to-severe pain with adjunctive opioid analgesics, and for the reduction of fever | The safety and effectiveness of Ofirmev for the treatment of acute pain and fever in pediatric patients ages 2 years and older is supported by evidence from adequate and well-controlled studies of Ofirmev in adults. Additional safety and PK data was collected in 355 from premature neonates to adolescents. The effectiveness of Ofirmev for the treatment of acute pain and fever has not been studied in pediatric patients < 2 years of age.The PK exposure of Ofirmev observed in children and adolescents is similar to adults, but higher in neonates and infants. Dosing simulations from PK data in infants and neonates suggest that dose reductions of 33% in infants 1 month to < 2 years of age, and 50% in neonates up to 28 days, with a minimum dosing interval of 6 hours, will produce a PK exposure similar to that observed in children age 2 years and olderMost common adverse reactions in pediatric patients were nausea, vomiting, constipation, pruritus, agitation, and atelectasis.Information on dosing, clinical studies, adverse reactions and PK parametersNew dosage form and route of administration | Labeling | - | P | - | - | Cadence | - | FALSE' |
| MESH:D022124 | 01/27/2017 | ofirmev | acetaminophen | Treatmeny of pain and fever in pediatric patients birth to 2 years | Treatment of pain Efficacy was not demonstrated in pediatric patients younger than 2 years in a double-blind, placebo-controlled study of 198 pediatric patients younger than 2 years. Pediatric patients less than 2 years of age, including neonates from 28 to 40 weeks gestational age at birth, were randomized to receive opioid plus acetaminophen or opioid plus placebo. No difference in analgesic effect of intravenous acetaminophen, measured by assessment of reduced need for additional opioid treatment for pain control, was observed. Treatment of fever The safety and effectiveness for the treatment of fever in pediatric patients, including premature neonates born at 32 weeks or greater gestation is supported by adequate and well-controlled studies of Ofirmev in adults, clinical studies in 244 pediatric patients 2 years and older, and safety and pharmacokinetic data from 239 patients younger than 2 years including neonates 32 weeks or greater gestational age. Information on dosing, clinical trials. Postmarketing study. | Labeling | - | - | B,P | - | Mallinckrodt | 11/7/2016 | FALSE |