484:
initial ACR analysis, suggested their potential usefulness for diagnostic purposes as well. Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminative abilities on the prevalence of the various vasculitides in the analyzed populations. Recently, an original study, combining the analysis of more than 100 items used to describe patients' characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively discriminating items to be used as a screening tool for diagnosis in patients suspected of systemic vasculitis.
94:
364:
66:
204:, which is the most common neurologic sign of PAN. Mononeuritis multiplex develops in more than 70% of patients with polyarteritis nodosa because of damage to arteries supplying large peripheral nerves. Most cases are marked by asymmetric polyneuropathy, but progressive disease can lead to symmetric nerve involvement. Central nervous system involvement may cause
476:
367:
Microscopic findings in polyarteritis nodosa: nodular thickened and branched arteries from small bowel mucosa (Fig. 1), flexor digitorum superficialis artery with early diffuse nuclear proliferation (X155; Fig. 2), nodular thickened and aneurysmal expanded artery: (a) tunica intima, (b) tunica media,
528:
is used before the leflunomide treatment is begun. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal
163:
PAN may affect nearly every organ system and thus can present with a broad array of signs and symptoms. These manifestations result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system. Constitutional symptoms are seen in up to 90% of affected individuals and
483:
In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making this "rosary sign" an important diagnostic feature of the vasculitis. The 1990 ACR criteria were designed for classification purposes only, but their good discriminatory performances, indicated by the
347:. The cause remains unknown in the remaining cases; there may be causal and clinical distinctions between classic idiopathic PAN, the cutaneous forms of PAN, and PAN associated with chronic hepatitis. In children, cutaneous PAN is frequently associated with
1239:
1224:
417:
A patient is said to have polyarteritis nodosa if he or she has three of the 10 signs known as the 1990 American
College of Rheumatology (ACR) criteria, when a radiographic or pathological diagnosis of vasculitis is made:
540:
The condition affects adults more frequently than children and males more frequently than females. Most cases occur between the ages of 40 and 60. Polyarteritis nodosa is more common in people with hepatitis B infection.
981:
Henegar, Corneliu; Pagnoux, Christian; Puéchal, Xavier; Zucker, Jean-Daniel; Bar-Hen, Avner; Guern, Véronique Le; Saba, Mona; Bagnères, Denis; Meyer, Olivier; Guillevin, Loïc (1 May 2008).
492:
Polyarteritis nodosa rarely affects the blood vessels of the lungs and this feature can help to differentiate it from other vasculitides that may have similar signs and symptoms (e.g.,
372:
No specific lab tests exist for diagnosing polyarteritis nodosa. Diagnosis is generally based on the physical examination and a few laboratory studies that help confirm the diagnosis:
1551:
1566:
1561:
1556:
1434:
1328:
1062:
Giannini, AJ; Black, HR. Psychiatric, Psychogenic and
Somatopsychic Disorders Handbook. Garden City, NY. Medical Examination Publishing, 1978. Pp. 219–220.
532:
In case of remission, about 60% experience relapse within five years. In cases caused by hepatitis B virus, however, recurrence rate is only around 6%.
479:
In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" a diagnostic feature of the vasculitis.
238:
in the urine may also be seen. Almost all patients with PAN have renal insufficiency caused by renal artery narrowing, thrombosis, and infarctions.
143:
PAN is a rare disease. With treatment, five-year survival is 80%; without treatment, five-year survival is 13%. Death is often a consequence of
186:
The skin may show rashes, swelling, necrotic ulcers, and subcutaneous nodules (lumps). Skin manifestations of PAN include palpable purpura and
706:
658:
613:
316:
132:, therefore making this "rosary sign" an important diagnostic feature of the vasculitis. PAN is sometimes associated with infection by the
394:) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as
1480:
262:
Damage to mesenteric arteries can cause abdominal pain, mesenteric ischemia, and bowel perforation. Abdominal pain may also be seen.
1495:
1459:
1321:
1067:
1420:
493:
1464:
1490:
828:
Russell
Goodman; Paul F. Dellaripa; Amy Leigh Miller; Joseph Loscalzo (January 2, 2014). "An Unusual Case of Abdominal Pain".
384:
73:
Polyarteritis nodosa: Macroscopic specimen of the heart with abundant adipose tissue and nodular thickened coronary vessels
1541:
1314:
340:
553:
Kussmaul disease or
Kussmaul-Maier disease reflect the seminal description of the disease in the medical literature by
128:
and other internal organs but generally sparing the lungs' circulation. Small aneurysms are strung like the beads of a
1485:
983:"A paradigm of diagnostic criteria for polyarteritis nodosa: Analysis of a series of 949 patients with vasculitides"
1386:
1128:"Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients"
328:
1451:
324:
196:
Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy).
1439:
1254:
497:
395:
1126:
Guillevin, L.; Lhote, F.; Cohen, P.; Sauvaget, F.; Jarrousse, B.; Lortholary, O.; Noël, L.; Trépo, C. (1995).
1353:
348:
201:
1546:
245:
197:
148:
1520:
1081:"Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients"
580:
is diagnosed with polyarteritis nodosa after experiencing excruciating chest pain and is treated with
1515:
1358:
516:. When present, underlying hepatitis B virus infection should be immediately treated. In some cases,
378:
864:
343:
are occasionally discovered in people affected by PAN. PAN has also been associated with underlying
1337:
1243:
558:
344:
93:
1157:
427:
409:
187:
1294:
1265:
1169:
1149:
1114:
1102:
1063:
1045:
1004:
969:
933:
892:
884:
845:
783:
702:
654:
609:
336:
169:
78:
1381:
1139:
1092:
1035:
994:
923:
876:
837:
775:
695:
Greenson, Joel K.; Montgomery, Elizabeth A.; Polydorides, Alexandros D. (1 September 2009).
571:
513:
177:
111:
554:
550:
524:
may be helpful. Some patients have entered a remission phase when a four-dose infusion of
951:
802:
1181:
634:
332:
293:
144:
1248:
1535:
1144:
1127:
289:
249:
1161:
1111:
982:
363:
65:
1405:
1166:
517:
297:
253:
227:
223:
125:
87:
1097:
1080:
1259:
696:
1289:
863:
Ebert, Ellen C.; Hagspiel, Klaus D.; Nagar, Michael; Schlesinger, Naomi (2008).
577:
521:
466:
455:
368:(c) tunica adventitia, (d) newly formed connective tissue and fat (Fig. 3; X155)
320:
231:
137:
133:
1270:
1040:
1023:
1396:
1368:
1345:
1233:
880:
779:
629:
509:
446:
272:
219:
117:
83:
888:
17:
928:
581:
525:
459:
402:
235:
1106:
1049:
1008:
937:
896:
849:
841:
787:
433:
Testicular pain or tenderness (occasionally, a site biopsied for diagnosis)
1153:
475:
465:
Biopsy of tissue showing the arteritis (typically inflamed arteries): The
452:
Hepatitis B (not C) virus tests positive (for surface antigen or antibody)
226:, which supplies the kidneys with highly oxygenated blood, often leads to
352:
303:
173:
114:
508:
Treatment involves medications to suppress the immune system, including
268:
209:
1306:
1216:
1024:"Low-Dose Methotrexate Controls a Severe Form of Polyarteritis Nodosa"
999:
911:
1428:
1414:
1228:
912:"Evolution of paediatric-specific vasculitis classification criteria"
430:(a mottled purplish skin discoloration over the extremities or torso)
391:
284:
205:
152:
129:
121:
529:
bleeding, infection, myocardial infarction, and/or kidney failure.
442:
Diastolic blood pressure greater than 90 mmHg (high blood pressure)
27:
Systemic necrotizing inflammation of medium-sized muscular arteries
474:
165:
604:
Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007).
1310:
401:
Tissue biopsy (reveals inflammation in small arteries, called
390:
Perinuclear pattern of antineutrophil cytoplasmic antibodies (
1079:
Selga, D.; Mohammad, A.; Sturfelt, G.; Segelmark, M. (2006).
698:
Diagnostic
Pathology: Gastrointestinal: Published by Amirsys
970:
http://www.medicinenet.com/polyarteritis_nodosa/article.htm
445:
Elevated kidney blood tests (BUN greater than 40 mg/dL or
766:
Forbess, L; Bannykh, S (2015). "Polyarteritis Nodosa".
200:
are often affected, and this most commonly presents as
865:"Gastrointestinal Involvement in Polyarteritis Nodosa"
690:
688:
244:
Involvement of the arteries of the heart may cause a
1206:
649:
Kumar, Vinay; K. Abbas, Abul; C. Aster, Jon (2015).
1508:
1473:
1450:
1404:
1395:
1367:
1344:
1280:
1210:
458:(angiogram) showing the arteries that are dilated (
335:-mediated cause in that subset. Infection with the
331:complexes in affected blood vessels, indicating an
77:
40:
35:
462:) or constricted by the blood vessel inflammation
319:, but about 30% of people with PAN have chronic
252:, and inflammation of the sac around the heart (
218:Kidney involvement is common and often leads to
140:virus. The condition may be present in infants.
1170:Entry in Polyarteritis Nodosa Follow-up article
1115:Entry in Polyarteritis Nodosa Follow-up article
651:Robbins and Cotran: Pathologic Basis of Disease
381:(may demonstrate an elevated white blood count)
910:Sarah Ringold; Carol A Wallace (May 1, 2010).
761:
759:
757:
755:
753:
751:
749:
747:
745:
1435:Eosinophilic granulomatosis with polyangiitis
1322:
743:
741:
739:
737:
735:
733:
731:
729:
727:
725:
8:
1022:Boehm, Ingrid; Bauer, R (1 February 2000).
1552:Steroid-responsive inflammatory conditions
1401:
1329:
1315:
1307:
1207:
768:Rheumatic Disease Clinics of North America
672:
670:
644:
642:
599:
597:
118:inflammation of blood vessels (vasculitis)
92:
64:
32:
1143:
1096:
1039:
998:
927:
439:Nerve disease (either single or multiple)
869:Clinical Gastroenterology and Hepatology
436:Muscle pain, weakness, or leg tenderness
424:Weight loss greater than/equal to 4.5 kg
362:
355:is included in the diagnostic criteria.
222:parts of the kidney. Involvement of the
653:(9th ed.). Elsevier. p. 509.
593:
469:is a frequent location for the biopsy.
317:anti-neutrophil cytoplasmic antibodies
1567:Hepatitis C virus-associated diseases
1562:Vascular-related cutaneous conditions
701:. Lippincott Williams & Wilkins.
7:
1557:Systemic connective tissue disorders
576:the protagonist character played by
1481:Acute hemorrhagic edema of infancy
25:
1496:Cutaneous small-vessel vasculitis
1460:Cutaneous small-vessel vasculitis
952:"Log in | BMJ Best Practice"
180:, and unintentional weight loss.
1421:Granulomatosis with polyangiitis
1145:10.1097/00005792-199509000-00002
916:Annals of the Rheumatic Diseases
803:"Infantile Polyarteritis Nodosa"
801:Person, A, Donald (2006-06-15).
494:granulomatosis with polyangiitis
120:affecting medium-sized muscular
1491:Bullous small vessel vasculitis
681:. W. B. Saunders. p. 243.
1:
351:, and positive streptococcal
230:in about one-third of cases.
398:or leukocytoclastic angiitis
315:PAN has no association with
1486:Cryoglobulinemic vasculitis
1098:10.1093/rheumatology/kel091
1583:
1387:Thromboangiitis obliterans
1041:10.1001/archderm.136.2.167
987:Arthritis & Rheumatism
569:In the 1956 American film
124:, typically involving the
1452:Type III hypersensitivity
881:10.1016/j.cgh.2008.04.004
780:10.1016/j.rdc.2014.09.005
606:Dermatology: 2-Volume Set
72:
63:
1440:Microscopic polyangiitis
1186:The Criterion Collection
498:microscopic polyangiitis
396:microscopic polyangiitis
349:streptococcal infections
323:and deposits containing
260:Gastrointestinal system:
1028:Archives of Dermatology
968:Shiel, Jr., William C,
929:10.1136/ard.2009.127886
449:greater than 1.5 mg/dL)
266:Musculoskeletal system:
126:arteries of the kidneys
842:10.1056/NEJMcps1215559
488:Differential diagnosis
480:
369:
242:Cardiovascular system:
202:mononeuritis multiplex
57:Kussmaul-Maier disease
478:
366:
232:deposition of protein
190:in some individuals.
149:myocardial infarction
1516:Goodpasture syndrome
1377:Polyarteritis nodosa
1359:Giant cell arteritis
1354:Takayasu's arteritis
956:bestpractice.bmj.com
608:. St. Louis: Mosby.
104:Polyarteritis nodosa
49:Periarteritis nodosa
36:Polyarteritis nodosa
1542:Disorders of fascia
1338:Systemic vasculitis
559:Rudolf Robert Maier
345:hairy cell leukemia
228:high blood pressure
45:Panarteritis nodosa
1521:Sneddon's syndrome
1281:External resources
1182:"Bigger Than Life"
481:
428:Livedo reticularis
410:C-reactive protein
370:
194:Neurologic system:
188:livedo reticularis
159:Signs and symptoms
1529:
1528:
1504:
1503:
1304:
1303:
1091:(10): 1276–1281.
1000:10.1002/art.23470
774:(1): 33–46, vii.
708:978-1-931884-26-6
679:Surgery, Volume 5
660:978-1-4557-2613-4
615:978-1-4160-2999-1
337:hepatitis C virus
198:Peripheral nerves
101:
100:
30:Medical condition
16:(Redirected from
1574:
1402:
1382:Kawasaki disease
1331:
1324:
1317:
1308:
1208:
1196:
1195:
1193:
1192:
1178:
1172:
1165:
1147:
1123:
1117:
1110:
1100:
1076:
1070:
1060:
1054:
1053:
1043:
1019:
1013:
1012:
1002:
993:(5): 1528–1538.
978:
972:
966:
960:
959:
948:
942:
941:
931:
907:
901:
900:
860:
854:
853:
825:
819:
818:
816:
814:
798:
792:
791:
763:
720:
719:
717:
715:
692:
683:
682:
674:
665:
664:
646:
637:
626:
620:
619:
601:
572:Bigger Than Life
514:cyclophosphamide
178:loss of appetite
97:
96:
68:
53:Kussmaul disease
33:
21:
1582:
1581:
1577:
1576:
1575:
1573:
1572:
1571:
1532:
1531:
1530:
1525:
1500:
1469:
1446:
1391:
1363:
1340:
1335:
1305:
1300:
1299:
1276:
1275:
1219:
1205:
1200:
1199:
1190:
1188:
1180:
1179:
1175:
1125:
1124:
1120:
1078:
1077:
1073:
1061:
1057:
1021:
1020:
1016:
980:
979:
975:
967:
963:
950:
949:
945:
909:
908:
904:
862:
861:
857:
827:
826:
822:
812:
810:
800:
799:
795:
765:
764:
723:
713:
711:
709:
694:
693:
686:
677:Keen, William.
676:
675:
668:
661:
648:
647:
640:
627:
623:
616:
603:
602:
595:
590:
567:
555:Adolph Kussmaul
551:medical eponyms
547:
538:
506:
490:
361:
313:
306:and perforation
292:resulting from
281:
161:
91:
31:
28:
23:
22:
15:
12:
11:
5:
1580:
1578:
1570:
1569:
1564:
1559:
1554:
1549:
1544:
1534:
1533:
1527:
1526:
1524:
1523:
1518:
1512:
1510:
1506:
1505:
1502:
1501:
1499:
1498:
1493:
1488:
1483:
1477:
1475:
1471:
1470:
1468:
1467:
1465:IgA vasculitis
1462:
1456:
1454:
1448:
1447:
1445:
1444:
1443:
1442:
1437:
1425:
1424:
1423:
1410:
1408:
1399:
1393:
1392:
1390:
1389:
1384:
1379:
1373:
1371:
1365:
1364:
1362:
1361:
1356:
1350:
1348:
1342:
1341:
1336:
1334:
1333:
1326:
1319:
1311:
1302:
1301:
1298:
1297:
1285:
1284:
1282:
1278:
1277:
1274:
1273:
1262:
1251:
1236:
1220:
1215:
1214:
1212:
1211:Classification
1204:
1203:External links
1201:
1198:
1197:
1173:
1138:(5): 238–253.
1118:
1071:
1055:
1014:
973:
961:
943:
902:
875:(9): 960–966.
855:
820:
793:
721:
707:
684:
666:
659:
638:
621:
614:
592:
591:
589:
586:
566:
563:
546:
543:
537:
534:
505:
502:
489:
486:
473:
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471:
470:
463:
453:
450:
443:
440:
437:
434:
431:
425:
415:
414:
413:
412:
406:
399:
388:
382:
360:
357:
333:immune complex
312:
309:
308:
307:
300:
294:cardiomyopathy
287:
280:
277:
160:
157:
145:kidney failure
99:
98:
81:
75:
74:
70:
69:
61:
60:
42:
38:
37:
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
1579:
1568:
1565:
1563:
1560:
1558:
1555:
1553:
1550:
1548:
1545:
1543:
1540:
1539:
1537:
1522:
1519:
1517:
1514:
1513:
1511:
1507:
1497:
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1492:
1489:
1487:
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1482:
1479:
1478:
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1472:
1466:
1463:
1461:
1458:
1457:
1455:
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1449:
1441:
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1436:
1433:
1432:
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1430:
1426:
1422:
1419:
1418:
1417:
1416:
1412:
1411:
1409:
1407:
1403:
1400:
1398:
1394:
1388:
1385:
1383:
1380:
1378:
1375:
1374:
1372:
1370:
1369:Medium vessel
1366:
1360:
1357:
1355:
1352:
1351:
1349:
1347:
1343:
1339:
1332:
1327:
1325:
1320:
1318:
1313:
1312:
1309:
1296:
1292:
1291:
1287:
1286:
1283:
1279:
1272:
1268:
1267:
1263:
1261:
1257:
1256:
1252:
1250:
1246:
1245:
1241:
1237:
1235:
1231:
1230:
1226:
1222:
1221:
1218:
1213:
1209:
1202:
1187:
1183:
1177:
1174:
1171:
1167:
1163:
1159:
1155:
1151:
1146:
1141:
1137:
1133:
1129:
1122:
1119:
1116:
1112:
1108:
1104:
1099:
1094:
1090:
1086:
1082:
1075:
1072:
1069:
1068:0-87488-596-5
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996:
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988:
984:
977:
974:
971:
965:
962:
957:
953:
947:
944:
939:
935:
930:
925:
922:(5): 785–86.
921:
917:
913:
906:
903:
898:
894:
890:
886:
882:
878:
874:
870:
866:
859:
856:
851:
847:
843:
839:
835:
831:
824:
821:
808:
804:
797:
794:
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781:
777:
773:
769:
762:
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758:
756:
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750:
748:
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742:
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738:
736:
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710:
704:
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699:
691:
689:
685:
680:
673:
671:
667:
662:
656:
652:
645:
643:
639:
636:
635:Who Named It?
632:
631:
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617:
611:
607:
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598:
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583:
579:
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365:
358:
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354:
350:
346:
342:
338:
334:
330:
326:
322:
318:
310:
305:
301:
299:
295:
291:
290:Heart failure
288:
286:
283:
282:
279:Complications
278:
276:
274:
270:
267:
263:
261:
257:
255:
251:
250:heart failure
247:
243:
239:
237:
233:
229:
225:
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1397:Small vessel
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1346:Large vessel
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275:are common.
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246:heart attack
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1290:MedlinePlus
813:24 December
578:James Mason
522:leflunomide
467:sural nerve
456:Arteriogram
321:hepatitis B
302:Intestinal
273:joint aches
138:hepatitis C
134:hepatitis B
115:necrotizing
41:Other names
1536:Categories
1266:DiseasesDB
1191:2021-07-16
588:References
510:prednisone
447:creatinine
387:(elevated)
84:Immunology
1474:Ungrouped
889:1542-3565
807:eMedicine
714:19 August
582:cortisone
526:rituximab
504:Treatment
460:aneurysms
408:Elevated
403:arteritis
359:Diagnosis
79:Specialty
1162:38111848
1132:Medicine
1107:16595516
1050:10677090
1009:18438816
938:20388739
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850:24382068
788:25399938
630:synd/764
353:serology
304:necrosis
220:death of
210:seizures
174:weakness
164:include
122:arteries
112:systemic
1260:D010488
1154:7565065
809:. WebMD
565:Culture
545:History
206:strokes
170:fatigue
110:) is a
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285:Stroke
269:Muscle
153:stroke
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1271:10220
1249:446.0
1234:M30.0
1158:S2CID
329:HBsAb
325:HBsAg
236:blood
184:Skin:
166:fever
151:, or
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1255:MeSH
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1150:PMID
1103:PMID
1064:ISBN
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893:PMID
885:ISSN
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815:2009
784:PMID
716:2013
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