PedAM
Pediatric Disease Annotations & Medicines
| pulmonary fibrosis | ||||
| Disease ID | 390 |
|---|---|
| Disease | pulmonary fibrosis |
| Manually Symptom | (Waiting for update.) |
| Text Mined Symptom | UMLS | Name | Sentences' Count(Total Symptoms:12) C0242379 | lung cancer | 24 C0020542 | pulmonary hypertension | 23 C1145670 | respiratory failure | 13 C0035229 | respiratory insufficiency | 4 C0264490 | acute respiratory failure | 3 C0013404 | dyspnea | 3 C0034642 | crackles | 1 C0034072 | cor pulmonale | 1 C0032326 | pneumothorax | 1 C0032273 | pneumoconiosis | 1 C0011633 | dermatomyositis | 1 C1619734 | pulmonary arterial hypertension | 1 |
Manually Genotype(Total Text Mining Genotypes:0) |
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| (Waiting for update.) |
All Snps(Total Genotypes:8) | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| snpId | pubmedId | geneId | geneSymbol | diseaseId | sourceId | sentence | score | Year | geneSymbol_dbSNP | CHROMOSOME | POS | REF | ALT |
| rs11575937 | 19859838 | 4000 | LMNA | umls:C0034069 | BeFree | A clinical picture related to the LMNA Arg482Gln mutation may be more diversified than it was previously considered and include low BMI and pulmonary fibrosis. | 0.000271442 | 2009 | LMNA | 1 | 156136985 | G | A,T |
| rs2004640 | 19116937 | 3663 | IRF5 | umls:C0034069 | BeFree | Association between the IRF5 rs2004640 functional polymorphism and systemic sclerosis: a new perspective for pulmonary fibrosis. | 0.005276948 | 2009 | IRF5 | 7 | 128938247 | T | G |
| rs2004640 | 19116937 | 3663 | IRF5 | umls:C0034069 | GAD | [Association between the IRF5 rs2004640 functional polymorphism and systemic sclerosis: a new perspective for pulmonary fibrosis.] | 0.005276948 | 2009 | IRF5 | 7 | 128938247 | T | G |
| rs2004640 | 25572744 | 472 | ATM | umls:C0034069 | BeFree | SSc patients of Han Chinese showed increased homozygous TT genotype of the rs2004640 (p = 0.027, odds ratio (OR) = 1.4, CI =1.03-1.93), which was significantly associated with pulmonary fibrosis of SSc and ATA-positive SSc of Han Chinese. | 0.000271442 | 2015 | IRF5 | 7 | 128938247 | T | G |
| rs2034650 | 25275363 | 3712 | IVD | umls:C0034069 | BeFree | The SNP in IVD (chromosome15, rs2034650) was significantly associated with pulmonary fibrosis in both the Mexican (OR = 0.40, P = .01) and Korean (OR = 0.13, P = .0008) cohorts. | 0.000271442 | 2015 | IVD | 15 | 40425103 | G | A |
| rs2276109 | 20595276 | 4321 | MMP12 | umls:C0034069 | BeFree | The MMP-12 rs2276109 gene polymorphism may contribute to susceptibility to SSc, and in particular to dcSSc and pulmonary fibrosis. | 0.000542884 | 2010 | MMP12 | 11 | 102875061 | T | C |
| rs35705950 | 23695349 | 727897 | MUC5B | umls:C0034069 | BeFree | To determine whether the MUC5B promoter polymorphism (rs35705950), previously reported to be associated with the development of pulmonary fibrosis, is associated with survival in IPF. | 0.001900093 | 2013 | NA | 11 | 1219991 | G | A,T |
| rs3917200 | 21148227 | 7043 | TGFB3 | umls:C0034069 | BeFree | Moreover, we could demonstrate a borderline significance between TGF-β3 (rs3917200) and lung fibrosis (p = 0.050). | 0.000542884 | 2011 | TGFB3 | 14 | 75963525 | A | G |
GWASdb Annotation(Total Genotypes:0) | |
|---|---|
| (Waiting for update.) | |
GWASdb Snp Trait(Total Genotypes:7) | |||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| CHR | POS | SNPID | REF | ALT | ORI_SNPID | PMID | P_VALUE | P_VALUE_TEXT | OR/BETA | CI95_TEXT | GWAS_INITIAL_SAMPLE_SIZE | SUB_POPULATION | SUPER_POPULATION | GWAS_TRAIT | HPO_ID | HPO_TERM | DO_ID | DO_TERM | MESH_ID | MESH_TERM | EFO_ID | EFO_TERM | DOLITE_TERM | RISK_ALLELE | PUBLICATION_TYPE | AA | GENE_SYMBOL | TYPE | REFGENE |
| 5 | 1286516 | rs2736100 | C | A | rs2736100 | 18835860 | 3.00E-08 | 2.11 | [1.61-2.78] | 159 Japanese ancestry cases; 934 Japanese ancestry controls | Japanese(1093) | ALL(1093) | ASN(1093) | ALL(1093) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs2736100-A | NA | T | TERT | |
| 11 | 1241221 | rs35705950 | G | A,T | rs35705950 | 24429156 | 2.00E-50 | NA | 2.43 | [2.13-2.77] | 542 European ancestry cases; 542 European ancestry controls | European(1084) | ALL(1084) | EUR(1084) | ALL(1084) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs35705950-T | Multicenter Study | Research Support, N.I.H., Extramural | Research Support, Non-U.S. Gov't |
| 11 | 1312706 | rs111521887 | C | G | rs111521887 | 24429156 | 2.20E-12 | NA | 1.48 | [1.32-1.66] | 542 European ancestry cases; 542 European ancestry controls | European(1084) | ALL(1084) | EUR(1084) | ALL(1084) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs111521887-G | Multicenter Study | Research Support, N.I.H., Extramural | Research Support, Non-U.S. Gov't |
| 11 | 1324772 | rs5743894 | T | C | rs5743894 | 24429156 | 1.00E-12 | NA | 1.49 | [1.33-1.68] | 542 European ancestry cases; 542 European ancestry controls | European(1084) | ALL(1084) | EUR(1084) | ALL(1084) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs5743894-G | Multicenter Study | Research Support, N.I.H., Extramural | Research Support, Non-U.S. Gov't |
| 11 | 1325829 | rs5743890 | T | C | rs5743890 | 24429156 | 3.00E-11 | NA | 1.64 | [1.41-1.92] | 542 European ancestry cases; 542 European ancestry controls | European(1084) | ALL(1084) | EUR(1084) | ALL(1084) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs5743890-A | Multicenter Study | Research Support, N.I.H., Extramural | Research Support, Non-U.S. Gov't |
| 14 | 48040375 | rs7144383 | G | A | rs7144383 | 24429156 | 4.00E-06 | NA | 1.44 | [1.23-1.69] | 542 European ancestry cases; 542 European ancestry controls | European(1084) | ALL(1084) | EUR(1084) | ALL(1084) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs7144383-G | Multicenter Study | Research Support, N.I.H., Extramural | Research Support, Non-U.S. Gov't |
| 17 | 43925297 | rs17690703 | C | T | rs17690703 | 24429156 | 6.00E-09 | NA | 1.43 | [1.27-1.61] | 542 European ancestry cases; 542 European ancestry controls | European(1084) | ALL(1084) | EUR(1084) | ALL(1084) | Idiopathic pulmonary fibrosis | HPOID:0002206 | Pulmonary fibrosis | DOID:0050156 | idiopathic pulmonary fibrosis | D054990 | Idiopathic Pulmonary Fibrosis | EFOID:0000768 | idiopathic pulmonary fibrosis | Pulmonary fibrosis | rs17690703-C | Multicenter Study | Research Support, N.I.H., Extramural | Research Support, Non-U.S. Gov't |
Mapped by lexical matching(Total Items:0) |
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| (Waiting for update.) |
Mapped by homologous gene(Total Items:0) |
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| (Waiting for update.) |
Chemical(Total Drugs:19) | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| CUI | ChemicalName | ChemicalID | CasRN | DiseaseName | DiseaseID | DirectEvidence | PubMedIDs | ||
| C0034069 | acetylcysteine | D000111 | 616-91-1 | pulmonary fibrosis | MESH:D011658 | therapeutic | 23900960 | ||
| C0034069 | beractant | C072197 | 108778-82-1 | pulmonary fibrosis | MESH:D011658 | therapeutic | 12607909 | ||
| C0034069 | bleomycin | D001761 | 11056-06-7 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 11554783 | ||
| C0034069 | busulfan | D002066 | 55-98-1 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 4301103 | ||
| C0034069 | cabergoline | C047047 | 81409-90-7 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 19170199 | ||
| C0034069 | carmustine | D002330 | 154-93-8 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 15286697 | ||
| C0034069 | cyclophosphamide | D003520 | 50-18-0 | pulmonary fibrosis | MESH:D011658 | therapeutic | 16636934 | ||
| C0034069 | enalapril | D004656 | 75847-73-3 | pulmonary fibrosis | MESH:D011658 | therapeutic | 17265423 | ||
| C0034069 | gefitinib | C419708 | 184475-35-2 | pulmonary fibrosis | MESH:D011658 | therapeutic | 18424623 | ||
| C0034069 | imatinib mesylate | D000068877 | - | pulmonary fibrosis | MESH:D011658 | therapeutic | 17849262 | ||
| C0034069 | melphalan | D008558 | 148-82-3 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 21129433 | ||
| C0034069 | metformin | D008687 | 657-24-9 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 3091170 | ||
| C0034069 | methysergide | D008784 | 361-37-5 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 1196890 | ||
| C0034069 | paclitaxel | D017239 | - | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 20025538 | ||
| C0034069 | pirfenidone | C093844 | 53179-13-8 | pulmonary fibrosis | MESH:D011658 | therapeutic | 12832656 | ||
| C0034069 | troglitazone | C057693 | 97322-87-7 | pulmonary fibrosis | MESH:D011658 | therapeutic | 18162602 | ||
| C0034069 | valsartan | D000068756 | - | pulmonary fibrosis | MESH:D011658 | therapeutic | 17241526 | ||
| C0034069 | vinblastine | D014747 | 865-21-4 | pulmonary fibrosis | MESH:D011658 | marker/mechanism | 4688040 | ||
| C0034069 | vitamin e | D014810 | 1406-18-4 | pulmonary fibrosis | MESH:D011658 | therapeutic | 18759078 | ||
FDA approved drug and dosage information(Total Drugs:5) | ||||||||
|---|---|---|---|---|---|---|---|---|
| DiseaseID | Drug_name | active_ingredients | strength | Dosage Form/Route | Marketing Status | TE code | RLD | RS |
| MESH:D011658 | busulfex | busulfan | 6MG/ML | INJECTABLE;INJECTION | Prescription | AP | Yes | Yes |
| MESH:D011658 | gleevec | imatinib mesylate | EQ 50MG BASE Federal Register determination that product was not discontinued or withdrawn for safety or efficacy reasons | CAPSULE;ORAL | Discontinued | None | Yes | No |
| MESH:D011658 | gleevec | imatinib mesylate | EQ 100MG BASE | TABLET;ORAL | Prescription | AB | Yes | No |
| MESH:D011658 | diovan | valsartan | 80MG | CAPSULE;ORAL | Discontinued | None | No | No |
| MESH:D011658 | diovan | valsartan | 80MG | TABLET;ORAL | Prescription | AB | Yes | No |
FDA labeling changes(Total Drugs:5) | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 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:D011658 | 01/13/2003 | busulfex | busulfan | Part of a conditioning regimen administered prior to hematopoietic progenitor cell transplantation for a variety of malignant hematologic or non-malignant diseases | The population pharmacokinetic estimates of busulfan for clearance and volume of distribution were determined in an open-label, uncontrolled PK study in 24 pediatric patients 5 months to 16 years who received busulfan as part of a conditioning regimen administered prior to hematopoietic progenitor cell transplantation for a variety of malignant hematologic or non-malignant diseases Suggested dosing regimen | Labeling | B | - | - | - | Orphan Medical | 12/3/2002 | FALSE' |
| MESH:D011658 | 09/27/2006 | gleevec | imatinib mesylate | Treatment of newly diagnosed pediatric patients with Philadelphia chromosome positive (Ph+) chronic myeloid leukemia (CML) in chronic phase | Extended age range for the treatment of newly diagnosed CML down to pediatric patients There are no data in children < 2 years of age Follow-up in children with newly diagnosed Ph+ chronic phase CML is limited Information on hematologic toxicities, AE profile, clinical studies and dosing guidelines new for newly diagnosed pediatric patients | Labeling | - | - | B, P | - | Novartis | 9/6/2006 | FALSE' |
| MESH:D011658 | 09/27/2006 | gleevec | imatinib mesylate | Treatment of newly diagnosed pediatric patients with Philadelphia chromosome positive (Ph+) chronic myeloid leukemia (CML) in chronic phase | Extended age range for the treatment of newly diagnosed CML down to pediatric patients There are no data in children < 2 years of age Follow-up in children with newly diagnosed Ph+ chronic phase CML is limited Information on hematologic toxicities, AE profile, clinical studies and dosing guidelines new for newly diagnosed pediatric patients | Labeling | - | - | B, P | - | Novartis | 9/6/2006 | FALSE' |
| MESH:D011658 | 11/29/2007 | diovan | valsartan | Hypertension | Labeling for 6-16 years of age Not recommended for pediatric patients less than 6 years due to safety findings possibly related to treatment or with glomerular filtration rate < 30mL/min/1.73m2 Information on dose, clinical studies in 1-16 years and pharmacokinetics No relevant differences were identified between adverse experience profile for pediatric patients and that previously reported for adult patients Information on preparation of a suspension | Labeling | B | - | - | - | Novartis | 8/8/2007 | FALSE' |
| MESH:D011658 | 11/29/2007 | diovan | valsartan | Hypertension | Labeling for 6-16 years of age Not recommended for pediatric patients less than 6 years due to safety findings possibly related to treatment or with glomerular filtration rate < 30mL/min/1.73m2 Information on dose, clinical studies in 1-16 years and pharmacokinetics No relevant differences were identified between adverse experience profile for pediatric patients and that previously reported for adult patients Information on preparation of a suspension | Labeling | B | - | - | - | Novartis | 8/8/2007 | FALSE' |