151:
107:
194:
PPD is an extremely rare disease. In the United States the disease is estimated to affect less than 5,000 people and approximately 1 per million people in the United
Kingdom however it is believed to be more common in Turkey and the Middle East.
142:
Treatment for PPD is supportive. Anti-inflammatory drugs may be used for pain due to secondary osteoarthritis. More severe joint pain may be treated with surgery. Physical therapy, occupational therapy, may help with joint stiffness.
46:
gene, which encodes a signalling factor involved in cartilage homeostasis. Genomic changes to the gene result in a non-functional protein, which disrupts cartilage aintenance and bone growth. The disorder is inherited in an
351:
Bhavani, Gandham SriLakshmi; Shah, Hitesh; Shukla, Anju; Dalal, Ashwin; Girisha, Katta Mohan (1993), Adam, Margaret P.; Everman, David B.; Mirzaa, Ghayda M.; Pagon, Roberta A. (eds.),
22:(PPD, PPRD), also known as progressive pseudorheumatoid arthropathy of childhood (PPAD), is a disorder of bone and cartilage that affects many joints. The disorder leads to
264:
Torreggiani, Sofia; Torcoletti, Marta; Campos-Xavier, Belinda; Baldo, Francesco; Agostoni, Carlo; Superti-Furga, Andrea; Filocamo, Giovanni (March 2019).
39:
162:
118:
240:
324:
212:
385:"Progressive pseudorheumatoid dysplasia with new-found gene mutation of Wntl inducible signaling pathway protein 3"
99:
Since symptoms of PPD are similar to juvenile rhematoid arthritis, people with PPD may be initially misdiagnosed.
241:"Progressive pseudorheumatoid dysplasia - About the Disease - Genetic and Rare Diseases Information Center"
383:
Chen, Wenji; Mo, Shiyan; Luo, Gui; Wang, Yanyan; Deng, Xiaohu; Zhu, Jian; Zhao, Wei (2018-09-10).
448:
453:
8:
48:
419:
384:
301:
30:
and widening of the ends of the finger and toe bones as well as other tubular bones.
424:
406:
360:
293:
285:
305:
414:
396:
277:
93:
352:
401:
281:
442:
410:
289:
27:
265:
428:
364:
297:
23:
150:
106:
263:
266:"Progressive pseudorheumatoid dysplasia: a rare childhood disease"
59:
Symptoms are present typically between ages three and six years.
96:
or through radiography with characteristic skeletal features.
325:"Progressive pseudorheumatoid dysplasia: MedlinePlus Genetics"
350:
359:, Seattle (WA): University of Washington, Seattle,
440:
69:Stiffness in the joints of the fingers and knees
40:Wnt1-inducible signalling protein 3 (WISP3) gene
217:NORD (National Organization for Rare Disorders)
382:
73:Symptoms that may develop over time include:
418:
400:
353:"Progressive Pseudorheumatoid Dysplasia"
213:"Progressive pseudorheumatoid dysplasia"
186:PPD has no severe effect on life span.
20:Progressive pseudorheumatoid disyplasia
441:
346:
344:
319:
317:
315:
235:
233:
145:
101:
13:
14:
465:
341:
312:
230:
149:
105:
38:PPD is caused by changes in the
189:
80:enlarged finger and knee joints
376:
257:
205:
92:PPD is diagnosed by molecular
1:
198:
181:
137:
87:
7:
54:
16:Bone and cartilage disorder
10:
470:
270:Rheumatology International
402:10.1186/s12969-018-0272-7
282:10.1007/s00296-018-4170-6
245:rarediseases.info.nih.gov
77:permanently bent fingers
63:Abnormal walking pattern
33:
389:Pediatric Rheumatology
42:, also known as the
49:autosomal recessive
161:. You can help by
117:. You can help by
179:
178:
135:
134:
461:
433:
432:
422:
404:
380:
374:
373:
372:
371:
348:
339:
338:
336:
335:
321:
310:
309:
261:
255:
254:
252:
251:
237:
228:
227:
225:
224:
209:
174:
171:
153:
146:
130:
127:
109:
102:
66:Weakness/fatigue
469:
468:
464:
463:
462:
460:
459:
458:
439:
438:
437:
436:
381:
377:
369:
367:
349:
342:
333:
331:
329:medlineplus.gov
323:
322:
313:
262:
258:
249:
247:
239:
238:
231:
222:
220:
211:
210:
206:
201:
192:
184:
175:
169:
166:
159:needs expansion
140:
131:
125:
122:
115:needs expansion
94:genetic testing
90:
57:
36:
17:
12:
11:
5:
467:
457:
456:
451:
435:
434:
375:
340:
311:
276:(3): 441–452.
256:
229:
219:. 16 June 2022
203:
202:
200:
197:
191:
188:
183:
180:
177:
176:
170:September 2022
156:
154:
139:
136:
133:
132:
126:September 2022
112:
110:
89:
86:
85:
84:
81:
78:
71:
70:
67:
64:
56:
53:
35:
32:
15:
9:
6:
4:
3:
2:
466:
455:
452:
450:
449:Rare diseases
447:
446:
444:
430:
426:
421:
416:
412:
408:
403:
398:
394:
390:
386:
379:
366:
362:
358:
354:
347:
345:
330:
326:
320:
318:
316:
307:
303:
299:
295:
291:
287:
283:
279:
275:
271:
267:
260:
246:
242:
236:
234:
218:
214:
208:
204:
196:
187:
173:
164:
160:
157:This section
155:
152:
148:
147:
144:
129:
120:
116:
113:This section
111:
108:
104:
103:
100:
97:
95:
82:
79:
76:
75:
74:
68:
65:
62:
61:
60:
52:
50:
45:
41:
31:
29:
28:short stature
25:
21:
454:Osteopathies
392:
388:
378:
368:, retrieved
357:GeneReviews®
356:
332:. Retrieved
328:
273:
269:
259:
248:. Retrieved
244:
221:. Retrieved
216:
207:
193:
190:Epidemiology
185:
167:
163:adding to it
158:
141:
123:
119:adding to it
114:
98:
91:
72:
58:
43:
37:
24:stiff joints
19:
18:
51:pattern.
443:Categories
370:2022-09-07
334:2022-09-07
250:2022-09-07
223:2022-09-07
199:References
411:1546-0096
395:(1): 55.
290:1437-160X
182:Prognosis
138:Treatment
88:Diagnosis
429:30200995
365:26610319
306:53528751
298:30327864
83:hip pain
55:Symptoms
420:6131911
427:
417:
409:
363:
304:
296:
288:
302:S2CID
34:Cause
425:PMID
407:ISSN
361:PMID
294:PMID
286:ISSN
44:CCN6
415:PMC
397:doi
278:doi
165:.
121:.
445::
423:.
413:.
405:.
393:16
391:.
387:.
355:,
343:^
327:.
314:^
300:.
292:.
284:.
274:39
272:.
268:.
243:.
232:^
215:.
26:,
431:.
399::
337:.
308:.
280::
253:.
226:.
172:)
168:(
128:)
124:(
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
