614:
well as internists, commonly misdiagnose Fabry disease. All immediate and extended family members in the same family have the same family mutation, so if one member of a family has a DNA sequence analysis performed, other members of the family can be diagnosed by performing a targeted sequence analysis instead of testing the entire gene. Targeted sequencing is quicker and less expensive to perform. One study reported that for every first diagnosis in a family, on average five more family members (immediate and extended) are also diagnosed.
279:
829:, but due to its rarity, determining an accurate disease frequency is difficult. Reported incidences, ranging from one in 476,000 to one in 117,000 in the general population, may largely underestimate the true prevalence. Newborn screening initiatives have found an unexpectedly high prevalence of the disease, as high as one in about 3,100 newborns in Italy and have identified a surprisingly high frequency of newborn males around one in 1,500 in Taiwan.
53:
662:), is a recombinant form of alpha-galactosidase A It received approval in the EU in 2001. FDA approval was applied for the United States. However, Shire withdrew their application for approval in the United States in 2012, citing that the agency will require additional clinical trials before approval. As of March 2022, Replagal has not received FDA approval.
287:
2921:
593:(GL-3) within lysosomes, that is believed to trigger a cascade of cellular events. The demonstration of marked alpha-galactosidase deficiency is the conclusive method for the diagnosis in homozygous males. It may be detected in heterozygotous females, but it is often inconclusive due to random X-chromosomal inactivation, so molecular testing (
816:
with Fabry disease for males was 58.2 years, compared with 74.7 years in the general population, and for females 75.4 years compared with 80.0 years in the general population, according to registry data from 2001 to 2008. The most common cause of death was cardiovascular disease, and most of those
613:
gene is the most accurate method of diagnosis in females, particularly if the mutations have already been identified in male family members. Many disease-causing mutations have been noted. Kidney biopsy may also be suggestive of Fabry disease if excessive lipid buildup is noted. Pediatricians, as
634:
The treatments available for Fabry disease can be divided into therapies that aim to correct the underlying problem of decreased activity of the alpha galactosidase A enzyme and thereby reduce the risk of organ damage, and therapies to improve symptoms and life expectancy once organ damage has
647:
is designed to provide the enzyme the patient is missing as a result of a genetic malfunction. This treatment is not a cure, but can partially prevent disease progression, and potentially reverse some symptoms. As of March 2022, three medical drugs based on enzyme replacement therapy are
461:(also known as vortex keratopathy), i.e. clouding of the corneas. Keratopathy may be the presenting feature in asymptomatic patients, and must be differentiated from other causes of vortex keratopathy (e.g. drug deposition in the cornea). This clouding does not affect vision.
294:
Symptoms are typically first experienced in early childhood and can be very difficult to diagnose; the rarity of Fabry disease to many clinicians sometimes leads to misdiagnoses. Manifestations of the disease usually increase in number and severity as an individual ages.
715:, is a pharmacological chaperone that can stabilize many mutant forms of alpha-galactosidase. It is taken by mouth. In a randomized trial comparing Migalastat with enzyme replacement therapy, the efficacy and safety of both treatments were similar. The US
870:
independently in 1898. It was recognised to be due to abnormal storage of lipids in 1952. In the 1960s, the inheritance pattern was established as being X-linked, as well as the molecular defect responsible for causing the accumulation of glycolipids.
625:
can show low T1 signal due to sphingolipid storage in the heart even without ventricular hypertrophy in 40% of those affected by the disease. Thus, MRI is a useful way of diagnosing the disease early. T2 signal is increased in inflammation and oedema.
2786:
2766:
1154:
Hoffmann B, Beck M, Sunder-Plassmann G, Borsini W, Ricci R, Mehta A (July 2007). "Nature and prevalence of pain in Fabry disease and its response to enzyme replacement therapy--a retrospective analysis from the Fabry
Outcome Survey".
2228:
311:
is believed to be related to the damage of peripheral nerve fibers that transmit pain. GI-tract pain is likely caused by accumulation of lipids in the small vasculature of the GI tract, which obstructs blood flow and causes pain.
332:(which causes foamy urine) is often the first sign of kidney involvement. End-stage kidney failure in those with Fabry disease typically occurs in the third decade of life, and is a common cause of death due to the disease.
520:
At least 443 disease-causing mutations in the GLA gene have been discovered. The DNA mutations that cause the disease are X-linked recessive with incomplete penetrance in heterozygous females. The condition affects
609:) to measure the level of alpha-galactosidase activity. An enzyme assay is not reliable for the diagnosis of disease in females due to the random nature of X-inactivation. Molecular genetic analysis of the
2383:"UHN Start-up AVROBIO, Inc. Announces $ 60 Million Series B Financing to Advance Gene Therapy Pipeline for Lysosomal Storage Disorders and Apply Lentiviral Platform to Other Genetic Diseases | TDC"
1859:
762:
A nucleoside modified RNA treatment that has shown efficacy in a mouse model of Fabry disease and in cardiomyocytes derived from induced pluripotent stem cells from individuals with Fabry disease.
2956:
672:, is another recombinant form of alpha-galactosidase. Like replagal, it received approval in the EU in 2001. In 2003, it was the first treatment for Fabry disease to be approved by the FDA.
569:, and hypohydrosis. In severe cases there is renal, cerebrovascular, and cardiac involvement and it is predominately responsible for premature mortality in Fabry patients. Fabry disease is
2242:
705:
of alpha-galactosidase despite the mutations that cause Fabry disease. As of March 2022, one medical drug based on pharmacological chaperone therapy is available for Fabry disease:
1882:
479:(in particular, burning extremity pain, red hands and feet on and off), cerebrovascular effects leading to an increased risk of stroke - early strokes, mostly vertebrobasilar system
464:
Other ocular findings can include conjunctival and retinal vascular abnormalities and anterior/posterior spoke-like cataract. Visual reduction from these manifestations is uncommon.
1203:
Putko BN, Wen K, Thompson RB, Mullen J, Shanks M, Yogasundaram H, et al. (March 2015). "Anderson-Fabry cardiomyopathy: prevalence, pathophysiology, diagnosis and treatment".
495:
Fabry disease is caused by a DNA sequence (gene) that is not functioning as it should. A person who inherits this gene does not have enough of a functioning enzyme known as
256:
The treatment for Fabry disease varies depending on the organs affected by the condition, and the underlying cause can be addressed by replacing the enzyme that is lacking.
1027:
557:
Fabry disease is an inherited lysosomal storage disorder that is caused by a deficiency of alpha-galactosidase A. This enzyme deficiency is a result of an accumulation of
2613:
2105:"Oral pharmacological chaperone migalastat compared with enzyme replacement therapy in Fabry disease: 18-month results from the randomised phase III ATTRACT study"
724:
2382:
1851:
773:
Pain associated with Fabry disease may be partially alleviated by enzyme replacement therapy in some patients, but pain management regimens may also include
844:
Chaperone therapy: Uses small-molecule drugs that bind to the defective enzyme and stabilize it to increase enzyme activity and increase cellular function
2903:
933:
685:
Clinically, agalsidase alfa and agalsidase beta are generally similar in effectiveness and safety, however they have never been compared directly in a
2971:
2358:"Open-Label, Study Of Efficacy and Safety Of AVR-RD-01 for Treatment -Naive Subjects With Classic Fabry Disease - Full Text View - ClinicalTrials.gov"
368:
589:, conduction abnormalities, and valvular abnormalities. Deficient activity of lysosomal alpha-galactosidase results in progressive accumulation of
841:
Substrate synthesis inhibition, also called substrate reduction therapy: Inhibits the production of the lipid (GL-3) that accumulates in the cells
1481:
Perrot A, Osterziel KJ, Beck M, Dietz R, Kampmann C (November 2002). "Fabry disease: focus on cardiac manifestations and molecular mechanisms".
785:, though the latter are usually best avoided in kidney disease. The kidney failure seen in some of those with Fabry disease sometimes requires
433:
that can appear on any region of the body, but are predominant on the thighs, around the navel, buttocks, lower abdomen, and groin) are common.
2253:
798:
1988:
Text was copied from this source which is copyright
European Medicines Agency. Reproduction is authorized provided the source is acknowledged.
621:
shows increased signal of the midwall at the inferolateral wall of the base of the left ventricle, usually in the non-hypertrophic ventricle.
2941:
2655:
2553:
1886:
565:
and most cell types and tissues, which leads it to be considered a multisystem disease. Indications include painful crisis, angiokeratomas,
2961:
2801:
782:
376:
2229:"Amicus Therapeutics Announces European Commission Approval for Galafold (Migalastat) in Patients with Fabry Disease in European Union"
2582:
618:
605:
Fabry disease is suspected based on the individual's clinical presentation, and can be diagnosed by an enzyme assay (usually done on
2087:
1105:
500:
2886:
2069:
2966:
2951:
2157:
for "Study to
Compare the Efficacy and Safety of Oral AT1001 and Enzyme Replacement Therapy in Patients With Fabry Disease" at
945:
2170:
920:(Korean drama): "Achiara's Secret" features daughters of a serial rapist who find each other because they share Fabry disease.
1632:
1584:
Marchesoni CL, Roa N, Pardal AM, Neumann P, Cáceres G, Martínez P, et al. (May 2010). "Misdiagnosis in Fabry disease".
992:[A contribution to the knowledge of the purpura haemorrhagica nodularis (purpura papulosa haemorrhagica Hebrae)].
655:
2911:
1409:"Refinement of evolutionary medicine predictions based on clinical evidence for the manifestations of Mendelian diseases"
693:
every two weeks. They are available in Europe and in many other parts of the world, but treatment costs remain very high.
2176:
867:
716:
267:
582:
499:. The lack of alpha-galactosidase leads to Fabry disease. A deficiency of alpha galactosidase A (a-GAL A, encoded by
357:
167:
2417:
2047:
644:
398:, thickening the valves and affecting the way they open and close. If severe, this can cause the valves to leak (
307:) or gastrointestinal (GI) tract is common in patients with Fabry disease. This pain can increase over time. This
2614:"Shire Submits Biologics License Application (BLA) for Replagal with the U.S. Food and Drug Administration (FDA)"
2307:
2283:
2249:
2204:
698:
211:
727:(CHMP) granted the drug a marketing approval under the name Galafold in May 2016. FDA approval followed in 2018.
2812:
2503:"Life expectancy and cause of death in males and females with Fabry disease: findings from the Fabry Registry"
2444:"GLA-modified RNA treatment lowers GB3 levels in iPSC-derived cardiomyocytes from Fabry-affected individuals"
2199:
924:
380:
356:. This hypertrophy can cause the heart muscle to become abnormally stiff and unable to relax, leading to a
120:
2946:
2000:"Agalsidase alfa versus agalsidase beta for the treatment of Fabry disease: an international cohort study"
321:
162:
46:
Fabry's disease, Anderson–Fabry disease, angiokeratoma corporis diffusum, alpha-galactosidase A deficiency
1946:
735:
A gene therapy treatment that is in early-phase clinical trials, with the technology licensed to AvroBio.
1021:
955:(Italian drama): Series two features an episode with a tennis player who is diagnosed with Fabry disease
732:
Experimental therapies that are not approved for treatment as of March 2022 include the following:
590:
510:
278:
990:"Ein Beitrag zur Kenntniss der Purpura haemorrhagica nodularis (Purpura papulosa haemorrhagica Hebrae)"
914:("There's No Place Like Home", season two, episode one) features a patient who died from Fabry disease.
838:
Enzyme replacement therapy: Replacement of the missing enzyme to clear the lipids (GL-3) from the cells
537:
to having severe symptoms. Research suggests many women experience severe symptoms ranging from early
1791:
Keating GM (October 2012). "Agalsidase alfa: a review of its use in the management of Fabry disease".
1420:
794:
756:
690:
675:
447:
399:
207:
2103:
Hughes DA, Nicholls K, Shankar SP, Sunder-Plassmann G, Koeller D, Nedd K, et al. (April 2017).
2790:
2332:
712:
496:
458:
361:
345:
246:
60:
2068:
Turner NN, Turner NN, Lameire N, Goldsmith DJ, Winearls CG, Himmelfarb J, Remuzzi G, eds. (2015).
2442:
Ter Huurne M, Parker BL, Liu NQ, Qian EL, Vivien C, Karavendzas K, et al. (September 2023).
2158:
1773:
1506:
1228:
1180:
1065:
1009:
902:
517:, other tissues, and organs. This accumulation leads to an impairment of their proper functions.
487:, nausea, inability to gain weight, chemical imbalances, and diarrhea are other common symptoms.
129:
1129:
739:
17:
2823:
2731:
2688:
2651:
2588:
2578:
2549:
2543:
2524:
2483:
2134:
2083:
2029:
1924:
1808:
1765:
1744:
Wanner C, Arad M, Baron R, Burlina A, Elliott PM, Feldt-Rasmussen U, et al. (July 2018).
1721:
1680:
1601:
1566:
1498:
1446:
1389:
1354:
1282:
1220:
1172:
1111:
1101:
895:
890:
723:
status in 2004, and the
European Commission followed in 2006. The European Medicines Agency's
566:
558:
545:
left ventricular heart problems and kidney failure. This variability is thought to be due to
484:
472:
152:
110:
2278:
1971:
1829:
282:
A bilateral, whorl-like corneal pattern of cream-colored lines in a person with Fabry disease
2721:
2713:
2678:
2514:
2473:
2463:
2455:
2124:
2116:
2075:
2019:
2011:
1800:
1757:
1711:
1670:
1593:
1556:
1546:
1490:
1436:
1428:
1381:
1372:
Karen JK, Hale EK, Ma L (December 2005). "Angiokeratoma corporis diffusum (Fabry disease)".
1322:
1312:
1272:
1264:
1212:
1164:
1093:
1057:
1001:
686:
372:
215:
76:
2700:
Wilcox WR, Banikazemi M, Guffon N, Waldek S, Lee P, Linthorst GE, et al. (July 2004).
1657:
Hagège A, Réant P, Habib G, Damy T, Barone-Rochette G, Soulat G, et al. (April 2019).
877:
published his classic paper on his electron microscopic findings in Fabry disease in 1965.
2925:
2890:
1998:
Arends M, Biegstraaten M, Wanner C, Sirrs S, Mehta A, Elliott PM, et al. (May 2018).
910:
813:
702:
665:
651:
415:
403:
308:
304:
250:
191:
2665:
Schiffmann R, Kopp JB, Austin HA, Sabnis S, Moore DF, Weibel T, et al. (June 2001).
2079:
701:
therapy is another strategy to maintain enzyme activity. It does so by assisting correct
2478:
2443:
1698:
Baig S, Vijapurapu R, Alharbi F, Nordin S, Kozor R, Moon J, et al. (January 2019).
1424:
1130:"Fabry disease | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
2883:
2726:
2701:
2129:
2104:
2024:
1999:
1561:
1534:
1441:
1408:
1327:
1300:
1277:
1252:
1097:
1061:
863:
778:
546:
388:
349:
325:
263:
2795:
2396:
533:
females. While males typically experience severe symptoms, women can range from being
52:
2935:
1804:
1301:"Proceedings of the 24th Paediatric Rheumatology European Society Congress: Part two"
874:
786:
581:
females. Cardiac involvement is recurrent in Fabry patients. Patients have developed
440:
426:
227:
125:
2852:
2048:"Search for interventional trials with Replagal and Fabrazyme on clinicaltrials.gov"
1777:
1510:
1345:
Chew E, Ghosh M, McCulloch C (June 1982). "Amiodarone-induced cornea verticillata".
1232:
1184:
1069:
1013:
850:
Gene therapy: Genetically modifies the affected cells to produce the missing enzyme.
826:
752:
622:
578:
534:
514:
411:
407:
384:
231:
2357:
2153:
2120:
2015:
1745:
1659:"Fabry disease in cardiology practice: Literature review and expert point of view"
617:
MRI is accurate in accessing left ventricular mass and thickness and hypertrophy.
2817:
2519:
2502:
1168:
847:
Gene editing: Technology that can potentially cut and fix a broken gene in a cell
2899:
2864:
1761:
1597:
860:
720:
542:
395:
353:
329:
260:
223:
2858:
2855:
2702:"Long-term safety and efficacy of enzyme replacement therapy for Fabry disease"
2570:
2459:
1675:
1658:
1432:
2869:
2828:
2775:
1900:
1494:
1317:
1268:
1216:
790:
708:
606:
594:
586:
574:
506:
476:
451:
436:
242:
2683:
2666:
1700:"Diagnosis and treatment of the cardiovascular consequences of Fabry disease"
906:("My Catalyst", season three, episode 12) features a Fabry disease diagnosis.
513:(abbreviated as Gb3, GL-3, or ceramide trihexoside) to accumulate within the
2847:
2667:"Enzyme replacement therapy in Fabry disease: a randomized controlled trial"
802:
774:
659:
2735:
2692:
2592:
2528:
2487:
2138:
2033:
1812:
1769:
1746:"European expert consensus statement on therapeutic goals in Fabry disease"
1725:
1716:
1699:
1684:
1605:
1570:
1551:
1502:
1450:
1393:
1286:
1224:
1176:
1115:
928:(Korean drama): In episode two, a prisoner is diagnosed with Fabry disease.
286:
1852:"With A Life-Saving Medicine In Short Supply, Patients Want Patent Broken"
1358:
678:(Elfabrio) was approved for medical use in the European Union in May 2023.
938:
570:
562:
538:
530:
526:
522:
480:
235:
745:
Bio-better ERT (CDX-6311) under pre-clinical development by the company
2468:
1385:
1005:
746:
430:
2758:
937:, a copycat of the titular Bone Collector has Fabry disease and takes
898:", season six, episode three) centers on a patient with Fabry disease.
2770:
880:
The first specific treatment for Fabry disease was approved in 2001.
669:
259:
The first descriptions of the condition were made simultaneously by
219:
195:
1045:
989:
320:
Kidney complications are common and serious effects of the disease;
2717:
2627:
1347:
Canadian
Journal of Ophthalmology. Journal Canadien d'Ophtalmologie
1092:. Handbook of Clinical Neurology. Vol. 132. pp. 231–248.
2894:
2501:
Waldek S, Patel MR, Banikazemi M, Lemay R, Lee P (November 2009).
341:
285:
277:
203:
199:
2569:
Mehta A, Beck M, Linhart A, Sunder-Plassmann G, Widmer U (2006).
2806:
2779:
2542:
John Thorne
Crissey, Lawrence C. Parish, Karl Holubar (2013).
801:, while the restrictive cardiomyopathy often seen may require
218:
that causes Fabry disease interferes with the function of an
85:
2333:"Canada launches first gene therapy trial for Fabry disease"
344:
in several ways. The accumulation of sphingolipids within
290:
Angiokeratoma, a common skin manifestation in Fabry disease
94:
1883:"Shire withdraws Replagal in USA as FDA wants more trials"
439:(lack of sweating) is a common symptom, and less commonly
230:, leading to these substances building up in the walls of
210:. Fabry disease is one of a group of conditions known as
949:(Korean drama), features Doctor K, who had Fabry disease.
367:
Fabry disease can also affect the way in which the heart
303:
Full-body or localized pain to the extremities (known as
245:
that measures the activity of the affected enzyme called
194:
that can affect many parts of the body, including the
2909:
789:. The cardiac complications of Fabry disease include
668:, sold under the brand name Fabrazyme by the company
549:
patterns during embryonic development of the female.
383:. These abnormal heart rhythms can cause blackouts,
82:
2748:
2571:"History of lysosomal storage diseases: an overview"
1947:"Drug Approval Package: Fabrazyme (agalsidase beta)"
941:, which allows the detectives to learn his identity.
711:, sold under the brand name Galafold by the company
654:, sold under the brand name Replagal by the company
91:
88:
2957:
Skin conditions resulting from errors in metabolism
2838:
2752:
2648:
2171:"Migalastat Orphan Drug Designations and Approvals"
173:
161:
151:
143:
135:
119:
109:
79:
67:
42:
37:
2545:Historical Atlas of Dermatology and Dermatologists
2573:. In Mehta A, Beck M, Sunder-Plassmann G (eds.).
2243:"Summary of Product Characteristics for Galafold"
1919:
1917:
1463:
972:
253:is also sometimes used, particularly in females.
1824:
1822:
742:(Ibiglustat) under development by Sanofi-Genzyme
525:males (i.e. all non-intersex males), as well as
2575:Fabry Disease: Perspectives from 5 Years of FOS
2308:"Drug Approval Package: Galafold (migalastat)"
1340:
1338:
1026:: CS1 maint: DOI inactive as of August 2024 (
725:Committee for Medicinal Products for Human Use
379:, and abnormally rapid heart rhythms such as
241:Fabry disease is sometimes diagnosed using a
27:Rare human genetic lysosomal storage disorder
8:
402:) or to restrict the forward flow of blood (
328:may worsen throughout life. The presence of
394:Sphingolipids can also build up within the
2749:
934:Lincoln Rhyme: Hunt for the Bone Collector
755:(ST-920) under development by the company
457:Ocular involvement may be present showing
234:and other organs. It is inherited in an
51:
34:
2725:
2682:
2518:
2477:
2467:
2128:
2023:
1715:
1674:
1560:
1550:
1440:
1407:Šimčíková D, Heneberg P (December 2019).
1326:
1316:
1276:
1253:"Anderson-Fabry disease in heart failure"
454:(in particular, burning extremity pain).
2074:. Vol. 1. Oxford University Press.
63:- the deficient protein in Fabry disease
2916:
965:
2646:James WD, Berger TG, Elston D (2006).
1862:from the original on 14 September 2010
1088:Schiffmann R (2015). "Fabry disease".
1019:
859:Fabry disease was first described by
799:implantable cardioverter-defibrillator
658:(since its acquisition of the company
157:Enzyme activity assay, genetic testing
2071:Fabry disease: Management and outcome
1739:
1737:
1735:
1627:
1625:
1623:
1621:
1619:
1617:
1615:
1528:
1526:
1524:
1522:
1520:
1476:
1474:
1472:
1305:Pediatric Rheumatology Online Journal
1251:Akhtar MM, Elliott PM (August 2018).
416:valves on the right side of the heart
7:
1246:
1244:
1242:
1198:
1196:
1194:
1083:
1081:
1079:
1039:
1037:
994:Archiv für Dermatologie und Syphilis
983:
981:
783:nonsteroidal anti-inflammatory drugs
414:are more commonly affected than the
1663:Archives of Cardiovascular Diseases
639:Therapies targeting enzyme activity
446:Additionally, patients can exhibit
2706:American Journal of Human Genetics
2616:. FierceBiotech. 22 December 2009.
2448:American Journal of Human Genetics
2080:10.1093/med/9780199592548.003.0338
1098:10.1016/B978-0-444-62702-5.00017-2
1062:10.1111/j.1365-2133.1898.tb16317.x
817:had received kidney replacements.
348:causes abnormal thickening of the
25:
1750:Molecular Genetics and Metabolism
1539:Orphanet Journal of Rare Diseases
1311:(Suppl 2): 65. 1 September 2017.
2972:Diseases named after discoverers
2919:
1805:10.2165/11209690-000000000-00000
738:The substrate reduction therapy
75:
2628:"The Village: Achiara's Secret"
1464:James, Berger & Elston 2006
973:James, Berger & Elston 2006
18:Angiokeratoma corporis diffusum
2884:Fabry Disease Information Page
1901:"Replagal FDA Approval Status"
1050:British Journal of Dermatology
1:
2231:. GlobeNewswire. 31 May 2016.
2121:10.1136/jmedgenet-2016-104178
2016:10.1136/jmedgenet-2017-104863
1885:. PharmaTimes. Archived from
1046:"A Case of 'Angeio-Keratoma'"
648:available for Fabry disease:
373:abnormally slow heart rhythms
340:Fabry disease can affect the
2942:X-linked recessive disorders
2520:10.1097/GIM.0b013e3181bb05bb
2177:Food and Drug Administration
1533:Germain DP (November 2010).
1169:10.1097/AJP.0b013e318074c986
1157:The Clinical Journal of Pain
717:Food and Drug Administration
369:conducts electrical impulses
2252:. June 2016. Archived from
2109:Journal of Medical Genetics
2004:Journal of Medical Genetics
1762:10.1016/j.ymgme.2018.06.004
1598:10.1016/j.jpeds.2010.02.012
1008:(inactive 13 August 2024).
619:Late gadolinium enhancement
597:) of females is mandatory.
583:hypertrophic cardiomyopathy
505:) due to mutation causes a
358:hypertrophic cardiomyopathy
168:Hypertrophic cardiomyopathy
2988:
2962:Lysosomal storage diseases
2548:. CRC Press. p. 179.
2460:10.1016/j.ajhg.2023.07.013
1881:Grogan K (15 March 2012).
1676:10.1016/j.acvd.2019.01.002
1433:10.1038/s41598-019-54976-4
1374:Dermatology Online Journal
645:Enzyme replacement therapy
212:lysosomal storage diseases
2418:"Fabry Disease Treatment"
2397:"Venglustat (Ibiglustat)"
2284:European Medicines Agency
2250:European Medicines Agency
2205:European Medicines Agency
1976:European Medicines Agency
1633:"Diagnosis & Testing"
1586:The Journal of Pediatrics
1495:10.1007/s00059-002-2429-9
1318:10.1186/s12969-017-0186-9
1269:10.1007/s12551-018-0432-5
1217:10.1007/s10741-014-9452-9
1134:rarediseases.info.nih.gov
1044:Anderson W (April 1898).
988:Fabry J (December 1898).
699:Pharmacological chaperone
59:
50:
2684:10.1001/jama.285.21.2743
2577:. Oxford PharmaGenesis.
1090:Neurocutaneous Syndromes
769:Organ-specific treatment
573:and manifests mostly in
2967:Cardiogenetic disorders
2952:Lipid storage disorders
2906:Genetics Home Reference
2889:2 December 2016 at the
719:(FDA) granted Galafold
483:(ringing in the ears),
381:ventricular tachycardia
2151:Clinical trial number
1552:10.1186/1750-1172-5-30
946:Partners for Justice 2
793:, which may require a
791:abnormal heart rhythms
443:(excessive sweating).
322:chronic kidney disease
291:
283:
188:Anderson–Fabry disease
163:Differential diagnosis
130:abnormal heart rhythms
2650:. Saunders Elsevier.
1205:Heart Failure Reviews
591:globotriaosylceramide
511:globotriaosylceramide
497:alpha-galactosidase A
289:
281:
2507:Genetics in Medicine
2422:www.fabrydisease.org
1717:10.1093/qjmed/hcy120
691:intravenous infusion
689:. Both are given by
676:Pegunigalsidase alfa
529:, and in many cases
468:Other manifestations
450:-like symptoms with
389:sudden cardiac death
377:complete heart block
330:protein in the urine
2288:. 17 September 2018
2209:. 17 September 2018
1927:. 17 September 2018
1832:. 17 September 2018
1425:2019NatSR...918577S
1257:Biophysical Reviews
884:Society and culture
713:Amicus Therapeutics
623:T1-weighted imaging
459:cornea verticillata
362:shortness of breath
247:alpha-galactosidase
61:Alpha galactosidase
2839:External resources
2362:clinicaltrials.gov
2159:ClinicalTrials.gov
1889:on 19 August 2014.
1413:Scientific Reports
1386:10.5070/D30TT696NJ
1006:10.1007/bf01986897
635:already occurred.
577:males but also in
559:glycosphingolipids
371:, leading to both
346:heart muscle cells
292:
284:
274:Signs and symptoms
177:Enzyme replacement
2879:
2878:
2677:(21): 2743–2749.
2657:978-0-7216-2921-6
2555:978-1-84214-100-7
1858:. 4 August 2010.
825:Fabry disease is
567:corneal dystrophy
448:Raynaud's disease
181:
180:
153:Diagnostic method
32:Medical condition
16:(Redirected from
2979:
2924:
2923:
2922:
2915:
2750:
2739:
2729:
2696:
2686:
2661:
2632:
2631:
2624:
2618:
2617:
2610:
2604:
2603:
2601:
2599:
2566:
2560:
2559:
2539:
2533:
2532:
2522:
2498:
2492:
2491:
2481:
2471:
2454:(9): 1600–1605.
2439:
2433:
2432:
2430:
2428:
2414:
2408:
2407:
2405:
2403:
2393:
2387:
2386:
2379:
2373:
2372:
2370:
2368:
2354:
2348:
2347:
2345:
2343:
2329:
2323:
2322:
2320:
2318:
2304:
2298:
2297:
2295:
2293:
2275:
2269:
2268:
2266:
2264:
2259:on 22 April 2018
2258:
2247:
2239:
2233:
2232:
2225:
2219:
2218:
2216:
2214:
2196:
2190:
2189:
2187:
2185:
2167:
2161:
2149:
2143:
2142:
2132:
2100:
2094:
2093:
2065:
2059:
2058:
2056:
2054:
2044:
2038:
2037:
2027:
1995:
1989:
1987:
1985:
1983:
1968:
1962:
1961:
1959:
1957:
1943:
1937:
1936:
1934:
1932:
1925:"Fabrazyme EPAR"
1921:
1912:
1911:
1909:
1907:
1897:
1891:
1890:
1878:
1872:
1871:
1869:
1867:
1848:
1842:
1841:
1839:
1837:
1826:
1817:
1816:
1788:
1782:
1781:
1741:
1730:
1729:
1719:
1695:
1689:
1688:
1678:
1654:
1648:
1647:
1645:
1643:
1637:FabryDisease.org
1629:
1610:
1609:
1581:
1575:
1574:
1564:
1554:
1530:
1515:
1514:
1478:
1467:
1461:
1455:
1454:
1444:
1404:
1398:
1397:
1369:
1363:
1362:
1342:
1333:
1332:
1330:
1320:
1297:
1291:
1290:
1280:
1263:(4): 1107–1119.
1248:
1237:
1236:
1200:
1189:
1188:
1151:
1145:
1144:
1142:
1140:
1126:
1120:
1119:
1085:
1074:
1073:
1041:
1032:
1031:
1025:
1017:
985:
976:
970:
868:William Anderson
687:randomized trial
429:(tiny, painless
268:William Anderson
266:and the surgeon
216:genetic mutation
186:, also known as
115:Medical Genetics
101:
100:
97:
96:
93:
90:
87:
84:
81:
55:
35:
21:
2987:
2986:
2982:
2981:
2980:
2978:
2977:
2976:
2932:
2931:
2930:
2920:
2918:
2910:
2891:Wayback Machine
2880:
2875:
2874:
2834:
2833:
2761:
2747:
2742:
2699:
2664:
2658:
2645:
2641:
2639:Further reading
2636:
2635:
2626:
2625:
2621:
2612:
2611:
2607:
2597:
2595:
2585:
2568:
2567:
2563:
2556:
2541:
2540:
2536:
2513:(11): 790–796.
2500:
2499:
2495:
2441:
2440:
2436:
2426:
2424:
2416:
2415:
2411:
2401:
2399:
2395:
2394:
2390:
2381:
2380:
2376:
2366:
2364:
2356:
2355:
2351:
2341:
2339:
2331:
2330:
2326:
2316:
2314:
2306:
2305:
2301:
2291:
2289:
2279:"Galafold EPAR"
2277:
2276:
2272:
2262:
2260:
2256:
2245:
2241:
2240:
2236:
2227:
2226:
2222:
2212:
2210:
2198:
2197:
2193:
2183:
2181:
2169:
2168:
2164:
2150:
2146:
2102:
2101:
2097:
2090:
2067:
2066:
2062:
2052:
2050:
2046:
2045:
2041:
1997:
1996:
1992:
1981:
1979:
1972:"Elfabrio EPAR"
1970:
1969:
1965:
1955:
1953:
1945:
1944:
1940:
1930:
1928:
1923:
1922:
1915:
1905:
1903:
1899:
1898:
1894:
1880:
1879:
1875:
1865:
1863:
1850:
1849:
1845:
1835:
1833:
1830:"Replagal EPAR"
1828:
1827:
1820:
1790:
1789:
1785:
1743:
1742:
1733:
1697:
1696:
1692:
1656:
1655:
1651:
1641:
1639:
1631:
1630:
1613:
1583:
1582:
1578:
1535:"Fabry disease"
1532:
1531:
1518:
1480:
1479:
1470:
1462:
1458:
1406:
1405:
1401:
1371:
1370:
1366:
1344:
1343:
1336:
1299:
1298:
1294:
1250:
1249:
1240:
1202:
1201:
1192:
1153:
1152:
1148:
1138:
1136:
1128:
1127:
1123:
1108:
1087:
1086:
1077:
1043:
1042:
1035:
1018:
987:
986:
979:
971:
967:
962:
911:Crossing Jordan
886:
857:
835:
823:
814:Life expectancy
811:
779:anticonvulsants
771:
666:Agalsidase beta
652:Agalsidase alfa
641:
632:
603:
555:
493:
470:
424:
338:
318:
309:acroparesthesia
305:acroparesthesia
301:
276:
251:genetic testing
222:that processes
192:genetic disease
105:
78:
74:
33:
28:
23:
22:
15:
12:
11:
5:
2985:
2983:
2975:
2974:
2969:
2964:
2959:
2954:
2949:
2944:
2934:
2933:
2929:
2928:
2908:
2907:
2897:
2877:
2876:
2873:
2872:
2861:
2843:
2842:
2840:
2836:
2835:
2832:
2831:
2820:
2809:
2798:
2783:
2762:
2757:
2756:
2754:
2753:Classification
2746:
2745:External links
2743:
2741:
2740:
2718:10.1086/422366
2697:
2662:
2656:
2642:
2640:
2637:
2634:
2633:
2619:
2605:
2584:978-1903539033
2583:
2561:
2554:
2534:
2493:
2434:
2409:
2388:
2374:
2349:
2324:
2299:
2270:
2234:
2220:
2191:
2162:
2144:
2115:(4): 288–296.
2095:
2088:
2060:
2039:
2010:(5): 351–358.
1990:
1963:
1938:
1913:
1892:
1873:
1843:
1818:
1799:(5): 335–354.
1783:
1756:(3): 189–203.
1731:
1690:
1669:(4): 278–287.
1649:
1611:
1592:(5): 828–831.
1576:
1516:
1489:(7): 699–702.
1468:
1456:
1399:
1364:
1334:
1292:
1238:
1211:(2): 179–191.
1190:
1163:(6): 535–542.
1146:
1121:
1106:
1075:
1056:(4): 113–117.
1033:
1000:(1): 187–200.
977:
964:
963:
961:
958:
957:
956:
950:
942:
929:
921:
915:
907:
899:
885:
882:
864:Johannes Fabry
856:
853:
852:
851:
848:
845:
842:
839:
834:
831:
822:
819:
810:
807:
770:
767:
766:
765:
764:
763:
760:
749:
743:
736:
730:
729:
728:
695:
694:
682:
681:
680:
679:
673:
663:
640:
637:
631:
628:
602:
599:
554:
551:
547:X-inactivation
541:or strokes to
492:
489:
469:
466:
427:Angiokeratomas
423:
420:
337:
334:
326:kidney failure
317:
314:
300:
297:
275:
272:
264:Johannes Fabry
179:
178:
175:
171:
170:
165:
159:
158:
155:
149:
148:
145:
141:
140:
137:
133:
132:
123:
117:
116:
113:
107:
106:
104:
103:
71:
69:
65:
64:
57:
56:
48:
47:
44:
40:
39:
31:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2984:
2973:
2970:
2968:
2965:
2963:
2960:
2958:
2955:
2953:
2950:
2948:
2947:Rare diseases
2945:
2943:
2940:
2939:
2937:
2927:
2917:
2913:
2905:
2901:
2900:Fabry disease
2898:
2896:
2892:
2888:
2885:
2882:
2881:
2871:
2870:Fabry disease
2867:
2866:
2862:
2860:
2857:
2854:
2850:
2849:
2845:
2844:
2841:
2837:
2830:
2826:
2825:
2821:
2819:
2815:
2814:
2810:
2808:
2804:
2803:
2799:
2797:
2793:
2792:
2788:
2784:
2781:
2777:
2773:
2772:
2768:
2764:
2763:
2760:
2755:
2751:
2744:
2737:
2733:
2728:
2723:
2719:
2715:
2711:
2707:
2703:
2698:
2694:
2690:
2685:
2680:
2676:
2672:
2668:
2663:
2659:
2653:
2649:
2644:
2643:
2638:
2629:
2623:
2620:
2615:
2609:
2606:
2594:
2590:
2586:
2580:
2576:
2572:
2565:
2562:
2557:
2551:
2547:
2546:
2538:
2535:
2530:
2526:
2521:
2516:
2512:
2508:
2504:
2497:
2494:
2489:
2485:
2480:
2475:
2470:
2465:
2461:
2457:
2453:
2449:
2445:
2438:
2435:
2423:
2419:
2413:
2410:
2398:
2392:
2389:
2384:
2378:
2375:
2363:
2359:
2353:
2350:
2338:
2334:
2328:
2325:
2313:
2309:
2303:
2300:
2287:
2285:
2280:
2274:
2271:
2255:
2251:
2244:
2238:
2235:
2230:
2224:
2221:
2208:
2206:
2201:
2200:"EU/3/06/368"
2195:
2192:
2180:
2178:
2172:
2166:
2163:
2160:
2156:
2155:
2148:
2145:
2140:
2136:
2131:
2126:
2122:
2118:
2114:
2110:
2106:
2099:
2096:
2091:
2089:9780199592548
2085:
2081:
2077:
2073:
2072:
2064:
2061:
2049:
2043:
2040:
2035:
2031:
2026:
2021:
2017:
2013:
2009:
2005:
2001:
1994:
1991:
1977:
1973:
1967:
1964:
1952:
1948:
1942:
1939:
1926:
1920:
1918:
1914:
1902:
1896:
1893:
1888:
1884:
1877:
1874:
1861:
1857:
1853:
1847:
1844:
1831:
1825:
1823:
1819:
1814:
1810:
1806:
1802:
1798:
1794:
1787:
1784:
1779:
1775:
1771:
1767:
1763:
1759:
1755:
1751:
1747:
1740:
1738:
1736:
1732:
1727:
1723:
1718:
1713:
1709:
1705:
1701:
1694:
1691:
1686:
1682:
1677:
1672:
1668:
1664:
1660:
1653:
1650:
1638:
1634:
1628:
1626:
1624:
1622:
1620:
1618:
1616:
1612:
1607:
1603:
1599:
1595:
1591:
1587:
1580:
1577:
1572:
1568:
1563:
1558:
1553:
1548:
1544:
1540:
1536:
1529:
1527:
1525:
1523:
1521:
1517:
1512:
1508:
1504:
1500:
1496:
1492:
1488:
1484:
1477:
1475:
1473:
1469:
1465:
1460:
1457:
1452:
1448:
1443:
1438:
1434:
1430:
1426:
1422:
1418:
1414:
1410:
1403:
1400:
1395:
1391:
1387:
1383:
1379:
1375:
1368:
1365:
1360:
1356:
1352:
1348:
1341:
1339:
1335:
1329:
1324:
1319:
1314:
1310:
1306:
1302:
1296:
1293:
1288:
1284:
1279:
1274:
1270:
1266:
1262:
1258:
1254:
1247:
1245:
1243:
1239:
1234:
1230:
1226:
1222:
1218:
1214:
1210:
1206:
1199:
1197:
1195:
1191:
1186:
1182:
1178:
1174:
1170:
1166:
1162:
1158:
1150:
1147:
1135:
1131:
1125:
1122:
1117:
1113:
1109:
1107:9780444627025
1103:
1099:
1095:
1091:
1084:
1082:
1080:
1076:
1071:
1067:
1063:
1059:
1055:
1051:
1047:
1040:
1038:
1034:
1029:
1023:
1015:
1011:
1007:
1003:
999:
996:(in German).
995:
991:
984:
982:
978:
975:, p. 538
974:
969:
966:
959:
954:
951:
948:
947:
943:
940:
936:
935:
930:
927:
926:
922:
919:
916:
913:
912:
908:
905:
904:
900:
897:
893:
892:
888:
887:
883:
881:
878:
876:
875:Ken Hashimoto
872:
869:
866:and surgeon
865:
862:
861:dermatologist
854:
849:
846:
843:
840:
837:
836:
832:
830:
828:
820:
818:
815:
808:
806:
804:
800:
796:
792:
788:
787:haemodialysis
784:
780:
776:
768:
761:
758:
754:
750:
748:
744:
741:
737:
734:
733:
731:
726:
722:
718:
714:
710:
707:
706:
704:
700:
697:
696:
692:
688:
684:
683:
677:
674:
671:
667:
664:
661:
657:
653:
650:
649:
646:
643:
642:
638:
636:
629:
627:
624:
620:
615:
612:
608:
600:
598:
596:
592:
588:
584:
580:
576:
572:
568:
564:
561:found in the
560:
552:
550:
548:
544:
540:
536:
532:
528:
524:
518:
516:
515:blood vessels
512:
508:
504:
503:
498:
490:
488:
486:
482:
478:
474:
467:
465:
462:
460:
455:
453:
449:
444:
442:
441:hyperhidrosis
438:
434:
432:
428:
421:
419:
417:
413:
412:mitral valves
409:
405:
401:
400:regurgitation
397:
392:
390:
386:
382:
378:
374:
370:
365:
363:
359:
355:
351:
347:
343:
335:
333:
331:
327:
323:
315:
313:
310:
306:
298:
296:
288:
280:
273:
271:
269:
265:
262:
261:dermatologist
257:
254:
252:
248:
244:
239:
237:
233:
232:blood vessels
229:
228:sphingolipids
225:
221:
217:
213:
209:
205:
201:
197:
193:
189:
185:
184:Fabry disease
176:
172:
169:
166:
164:
160:
156:
154:
150:
146:
142:
138:
134:
131:
127:
126:Heart failure
124:
122:
121:Complications
118:
114:
112:
108:
99:
73:
72:
70:
68:Pronunciation
66:
62:
58:
54:
49:
45:
41:
38:Fabry disease
36:
30:
19:
2863:
2846:
2822:
2811:
2800:
2785:
2765:
2712:(1): 65–74.
2709:
2705:
2674:
2670:
2647:
2622:
2608:
2596:. Retrieved
2574:
2564:
2544:
2537:
2510:
2506:
2496:
2451:
2447:
2437:
2425:. Retrieved
2421:
2412:
2400:. Retrieved
2391:
2377:
2365:. Retrieved
2361:
2352:
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