446:
of corticosteroid therapy and most other patients responding by the 4th week. Few do not respond to corticosteroids and have to rely on an alternative therapy. However, despite positive response to corticosteroids, relapses are common, requiring repeat treatment with corticosteroids. About 25% never relapse, another 25% relapse infrequently (one relapse within 6 months of initial response or 1–3 relapses in 12 months), and 50% relapse frequently (>2 relapses within 6 months of initial response or >4 relapses in 12 months). The relapse rate is the reason behind a discussion on continuing prednisone treatment to even beyond 12 weeks to possibly decrease relapse rate; several studies trying this have failed to show significant improvement. A majority of relapses seem to be triggered by respiratory infections. Long term, children can relapse several years after having no symptoms; though after 2 years, the risk is significantly lower.
32:
71:
366:) and if they fail to respond to corticosteroid therapy. These would suggest that it may not be minimal change disease. In adults, a renal biopsy is required as there is a much wider differential for nephrotic syndrome. As the name suggests, the renal biopsy of a patient with minimal change disease would show minimal or no evidence of disease in
317:
reduces relative to the interstitial tissue. The subsequent movement of fluid from the vascular compartment to the interstitial compartment manifests as the soft tissue swelling referred to as edema. This fluid collects most commonly in the feet and legs, in response to gravity, particularly in those
304:
There has been discussion of B cell involvement in nephrotic syndrome, especially minimal change disease due to the success of immunotherapy that target both B and T cells, increased markers for B cell activation during a relapse of minimal change disease, and alterations in B cell sub-classes during
410:
though studies on their effectiveness is fairly limited. There is no consensus on how long the corticosteroid therapy should be, with treatment length ranging from 4–12 weeks. Along with corticosteroid therapy, acute symptomatic management involves salt and fluid restriction to control the swelling.
498:
It is by far the most common cause of nephrotic syndrome in children, accounting for 70–90% of children >1 year of age. After puberty, it is caused by minimal change disease about half the time. Among young children, boys seem to be more likely to develop minimal change disease than girls (about
445:
Minimal change disease usually responds well to initial treatment with the first-line therapy: corticosteroids, with 95% responding. Younger children, who are more likely to develop minimal change disease, usually respond faster than adults with 50% of children having complete remission with 8 days
462:
While most adults diagnosed with minimal change disease respond to corticosteroids, 25% fail to respond after 3–4 months of corticosteroid therapy; it is possible that these patients were incorrectly diagnosed, and do not have minimal change disease. Adults with MCD tend to respond more slowly to
419:
Treatment guidelines for adults are fairly limited, and are largely based on studies done on children. The mainline therapy is also corticosteroid therapy prednisone 1 mg/kg/day with other immunosuppressants as possible alternatives, though there is very little data on these alternatives'
300:
having been most studied in this regard, yet never conclusively implicated. Data from a longitudinal study (Nephrotic
Syndrome Study Network – NEPTUNE) published in 2022 suggested that up to 29% of biopsy-confirmed, mixed pediatric and adult minimal change disease cases exhibited serum
449:
In most children with minimal change disease, particularly among those who respond typically, there is minimal to no permanent damage observed in their kidneys. Complications primarily arise from the side effects of therapy. Prolonged use of corticosteroids can lead to
510:
Minimal change disease has been called by many other names in the medical literature, including minimal change nephropathy, minimal change nephrosis, minimal change nephrotic syndrome, minimal change glomerulopathy, foot process disease (referring to the
167:
993:
973:
463:
corticosteroid treatment, taking up to 3 or 4 months, than children do. Data in adults is less complete than for children, but relapses are fairly frequent with 56–76% of patients relapsing and needing further treatment with
334:
As minimal change disease is a subset of nephrotic syndrome, diagnosis involves looking for a combination of edema, high amounts of protein in urine, low albumin and high serum cholesterol. Initial workup can include a
354:
As MCD is the most common type of nephrotic syndrome in children, renal biopsy is not usually done in children under the age of 10 unless there are concerning features that are unusual for the disease (
911:
Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, Wigfall D, Miles P, Powell L, Lin JJ, Trachtman H, Greenbaum LA (August 2009). "Management of childhood onset nephrotic syndrome".
391:, 60 mg/sq.m/day or 2 mg/kg/day. For those children who are unable to tolerate corticosteroid treatment, or are unresponsive (usually after a trial of 8 weeks), another
127:
at 2 to 6 years of age. MCD is responsible for 10–25% of nephrotic syndrome cases in adults. It is also the most common cause of nephrotic syndrome of unclear cause (
326:. As a result of the excess fluid, individuals with minimal change disease often gain weight, as they are excreting less water in the urine, and experience fatigue.
499:
2:1). Minimal change disease is seen in about 16 in every 100,000 children, being more common in South Asians and Native
Americans, but rarer in African Americans.
1162:
667:
620:
428:
to decrease high levels of cholesterol seen with nephrotic syndrome are generally unnecessary. ACE inhibitors may be considered in people with MCD who also have
1301:
1093:
1157:
860:
1167:
251:(i.e., podocyte effacement), vacuolation, and growth of microvilli on the visceral epithelial cells, allowing for excess protein loss in the urine.
284:, specifically the podocytes, increasing permeability. This allows the leakage of albumin and other serum proteins into the urine. Also, the exact
305:
minimal change disease remission. This hypothesis is supported by recent findings of anti-nephrin antibodies isolated in minimal change disease.
1279:
1252:
1240:
1218:
1209:
1134:
515:, nil disease (referring to the lack of pathologic findings on light microscopy), nil lesions, lipid nephrosis, and lipoid nephrosis.
643:
596:
181:
with an associated increase in weight. The swelling may be mild but patients can present with edema in the lower half of the body,
1291:
1086:
1262:
816:; Lusco, Mark A.; Najafian, Behzad; Alpers, Charles E. (Aug 2015). "AJKD Atlas of Renal Pathology: Minimal Change Disease".
1139:
1347:
281:
1079:
1188:
1244:
272:
or immune complex deposition. Rather, an altered T cell-mediated immunologic response with abnormal secretion of
1296:
1222:
1008:
124:
31:
861:"Discovery of Autoantibodies Targeting Nephrin in Minimal Change Disease Supports a Novel Autoimmune Etiology"
638:. Kumar, Vinay, 1944–, Abbas, Abul K.,, Aster, Jon C.,, Perkins, James A. (Ninth ed.). Philadelphia, PA.
512:
313:
When albumin is excreted in the urine, its serum (blood) concentration decreases. Consequently, the plasma
495:
Minimal change disease is most common in very young children but can occur in older children and adults.
403:
399:
348:
340:
1230:
1183:
269:
123:
commonly experienced by those affected by the disease. It is most common in children and has a peak
70:
997:
355:
243:, the changes now known as the hallmarks of the disease were discovered. These are diffuse loss of
240:
222:
194:
190:
1326:
1321:
1204:
1149:
1121:
1112:
936:
661:
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229:
160:
104:
1048:
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719:
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639:
602:
592:
451:
392:
363:
319:
182:
170:
59:
301:
autoantibodies against nephrin, a structural protein located in the podocyte slit diaphragm.
39:
The three hallmarks of minimal change disease (seen on electron microscopy): diffuse loss of
920:
880:
872:
825:
785:
769:
709:
367:
314:
210:
756:
Vivarelli, Marina; Massella, Laura; Ruggiero, Barbara; Emma, Francesco (February 7, 2017).
1269:
1257:
233:
156:
1102:
885:
790:
757:
388:
297:
293:
289:
197:. Due to the disease process, patients with minimal change disease are also at risk of
120:
100:
1002:
502:
In adults, it accounts for less than 15% of adults diagnosed with nephrotic syndrome.
264:
The cause and pathogenesis of the pathology is unclear and it is currently considered
1341:
476:
421:
218:
140:
697:
209:
For years, pathologists found no changes when viewing kidney biopsy specimens under
1283:
1071:
1059:
940:
484:
429:
359:
248:
43:
155:(swelling of soft tissues as a consequence of water retention), weight gain, and
1013:
1043:
468:
395:
344:
198:
144:
112:
47:
829:
1024:
813:
606:
472:
384:
336:
273:
265:
128:
64:
51:
837:
781:
653:
1054:
986:
982:
480:
407:
214:
166:
932:
924:
894:
876:
845:
799:
723:
714:
398:
is an alternative; other immunosuppressants have also been used such as a
318:
with poorly functioning valves. In severe cases, fluid can shift into the
773:
487:
for MCD. Complications primarily arise from the side effects of therapy.
285:
244:
186:
40:
965:
323:
148:
108:
189:
in more severe cases. In older adults, patients may also present with
977:
425:
277:
587:
Johnson, Richard J.; Feehally, John; Floege, Jürgen (2018-06-26).
178:
165:
152:
116:
159:(low serum albumin). These signs are referred to collectively as
107:. Nephrotic syndrome leads to the loss of significant amounts of
1075:
177:
The first clinical sign of minimal change disease is usually
454:(leading to infection), growth complications, weight gain.
370:, which is unique among the causes of nephrotic syndrome.
217:
expansion may be seen in some cases, there are no other
762:
Clinical
Journal of the American Society of Nephrology
383:
The first line therapy for minimal change disease is
955:
634:
Kumar, Vinay; Abbas, Abul K.; Aster, Jon C. (2014).
351:
are present in the urine of 10-30% adults with MCD.
1314:
1278:
1239:
1217:
1201:
1176:
1148:
1120:
1109:
1034:
959:
698:"The treatment of minimal change disease in adults"
58:
24:
483:. There is little evidence to support the use of
213:, hence the name "minimal change disease." While
751:
749:
747:
745:
743:
741:
739:
737:
735:
733:
424:to reduce the amount of protein in the urine or
582:
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572:
570:
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566:
564:
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560:
558:
556:
554:
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236:or complement deposits bound to kidney tissue.
636:Robbins and Cotran pathologic basis of disease
546:
544:
542:
540:
538:
536:
534:
532:
530:
528:
1302:Eosinophilic granulomatosis with polyangiitis
1087:
906:
904:
865:Journal of the American Society of Nephrology
702:Journal of the American Society of Nephrology
8:
691:
689:
687:
685:
683:
681:
679:
677:
288:responsible has yet to be elucidated, with
1214:
1117:
1094:
1080:
1072:
956:
666:: CS1 maint: location missing publisher (
619:: CS1 maint: location missing publisher (
185:, swelling in the scrotal/labial area and
173:that can be seen in minimal change disease
69:
30:
21:
884:
789:
713:
268:. However, it does not appear to involve
16:Kidney disease causing nephrotic syndrome
147:(abnormal excretion of proteins, mainly
1168:Membranoproliferative/mesangiocapillary
696:Hogan J, Radhakrishnan J (April 2013).
524:
659:
612:
7:
420:efficacy. Other medications such as
818:American Journal of Kidney Diseases
14:
589:Comprehensive clinical nephrology
1292:Granulomatosis with polyangiitis
1103:Disease of the kidney glomerules
193:(20–25% of affected adults) and
115:), which causes the widespread
859:Watts, Andrew (January 2022).
143:of minimal change disease are
96:
1:
591:(Sixth ed.). Edinburgh.
101:disease affecting the kidneys
349:Microscopic amounts of blood
343:, serum albumin level and a
282:glomerular basement membrane
1163:Endocapillary proliferative
119:(soft tissue swelling) and
1364:
830:10.1053/j.ajkd.2015.04.006
1245:Type III hypersensitivity
280:is thought to modify the
245:visceral epithelial cells
50:, and the appearance of
38:
29:
1297:Microscopic polyangiitis
1223:Type II hypersensitivity
758:"Minimal Change Disease"
121:impaired kidney function
1158:Mesangial proliferative
513:podocyte foot processes
925:10.1542/peds.2008-1559
877:10.1681/ASN.2021060794
715:10.1681/ASN.2012070734
174:
81:Minimal change disease
25:Minimal change disease
1263:diffuse proliferative
404:mycophenolate mofetil
400:calcineurin inhibitor
341:kidney function tests
169:
1231:Goodpasture syndrome
774:10.2215/CJN.05000516
322:(abdomen) and cause
1348:Glomerular diseases
430:high blood pressure
356:high blood pressure
241:electron microscopy
239:With the advent of
195:high blood pressure
191:acute kidney injury
151:, into the urine),
1327:glomerulonephrosis
1322:glomerulonephritis
1253:Post-streptococcal
1035:External resources
465:immunosuppressants
230:immunofluorescence
175:
161:nephrotic syndrome
135:Signs and symptoms
105:nephrotic syndrome
1335:
1334:
1310:
1309:
1197:
1196:
1122:Non-proliferative
1069:
1068:
452:immunosuppression
393:immunosuppressant
364:renal dysfunction
320:peritoneal cavity
183:periorbital edema
171:Periorbital edema
87:), also known as
78:
77:
19:Medical condition
1355:
1215:
1118:
1096:
1089:
1082:
1073:
957:
945:
944:
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856:
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849:
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728:
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717:
693:
672:
671:
665:
657:
631:
625:
624:
618:
610:
584:
368:light microscopy
315:oncotic pressure
221:injuries to the
211:light microscopy
201:and infections.
89:lipoid nephrosis
74:
73:
34:
22:
1363:
1362:
1358:
1357:
1356:
1354:
1353:
1352:
1338:
1337:
1336:
1331:
1306:
1274:
1235:
1208:
1203:
1193:
1172:
1144:
1135:Focal segmental
1111:
1105:
1100:
1070:
1065:
1064:
1030:
1029:
968:
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949:
948:
910:
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902:
858:
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731:
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526:
521:
508:
493:
460:
443:
438:
417:
381:
376:
332:
311:
262:
257:
255:Pathophysiology
234:immunoglobulins
232:, there are no
207:
157:hypoalbuminemia
137:
131:) in children.
68:
20:
17:
12:
11:
5:
1361:
1359:
1351:
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1255:
1249:
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1234:
1233:
1227:
1225:
1212:
1199:
1198:
1195:
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1191:
1186:
1180:
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1174:
1173:
1171:
1170:
1165:
1160:
1154:
1152:
1146:
1145:
1143:
1142:
1137:
1132:
1130:Minimal change
1126:
1124:
1115:
1107:
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1101:
1099:
1098:
1091:
1084:
1076:
1067:
1066:
1063:
1062:
1051:
1039:
1038:
1036:
1032:
1031:
1028:
1027:
1016:
1005:
990:
989:with .0 suffix
969:
964:
963:
961:
960:Classification
953:
952:External links
950:
947:
946:
900:
871:(1): 238–252.
851:
824:(2): 376–377.
814:Fogo, Agnes B.
805:
768:(2): 332–345.
729:
673:
644:
626:
597:
523:
522:
520:
517:
507:
504:
492:
489:
459:
456:
442:
439:
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422:ACE inhibitors
416:
413:
389:corticosteroid
380:
377:
375:
372:
331:
328:
310:
307:
261:
258:
256:
253:
249:foot processes
206:
203:
141:clinical signs
136:
133:
111:to the urine (
76:
75:
62:
56:
55:
44:foot processes
36:
35:
27:
26:
18:
15:
13:
10:
9:
6:
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3:
2:
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1150:Proliferative
1147:
1141:
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1133:
1131:
1128:
1127:
1125:
1123:
1119:
1116:
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1097:
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984:
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975:
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962:
958:
951:
942:
938:
934:
930:
926:
922:
919:(2): 747–57.
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914:
907:
905:
901:
896:
892:
887:
882:
878:
874:
870:
866:
862:
855:
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831:
827:
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748:
746:
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742:
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738:
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734:
730:
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721:
716:
711:
708:(5): 702–11.
707:
703:
699:
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690:
688:
686:
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682:
680:
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674:
669:
663:
655:
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645:9781455726134
641:
637:
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608:
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598:9780323547192
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579:
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541:
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531:
529:
525:
518:
516:
514:
505:
503:
500:
496:
490:
488:
486:
482:
478:
477:mycophenolate
474:
470:
466:
457:
455:
453:
447:
440:
435:
433:
431:
427:
423:
414:
412:
409:
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271:
267:
259:
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226:
224:
220:
219:pathognomonic
216:
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204:
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162:
158:
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134:
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126:
122:
118:
114:
110:
106:
103:which causes
102:
98:
94:
90:
86:
82:
72:
66:
63:
61:
57:
53:
49:
45:
42:
37:
33:
28:
23:
1284:Pauci-immune
1280:Type III RPG
1177:By condition
1129:
1053:
1042:
1018:
1007:
992:
972:
916:
912:
868:
864:
854:
821:
817:
808:
765:
761:
705:
701:
635:
629:
588:
509:
501:
497:
494:
491:Epidemiology
485:azathioprine
461:
448:
444:
418:
382:
360:bloody urine
353:
333:
312:
303:
263:
238:
227:
208:
176:
138:
97:among others
92:
88:
84:
80:
79:
1241:Type II RPG
1189:Amyloidosis
1044:MedlinePlus
469:ciclosporin
396:cyclosporin
345:lipid panel
274:lymphokines
260:Proteinuria
199:blood clots
145:proteinuria
113:proteinuria
93:nil disease
48:vacuolation
1219:Type I RPG
1140:Membranous
1020:DiseasesDB
913:Pediatrics
607:1047958109
519:References
473:tacrolimus
385:prednisone
337:urinalysis
270:complement
266:idiopathic
223:glomerulus
129:idiopathic
65:Nephrology
52:microvilli
1205:nephritic
1202:Primarily
1113:nephrotic
1110:Primarily
1055:eMedicine
838:1523-6838
782:1555-905X
662:cite book
654:879416939
615:cite book
506:Etymology
481:rituximab
436:Prognosis
408:rituximab
374:Treatment
330:Diagnosis
215:mesangial
205:Pathology
125:incidence
60:Specialty
1342:Category
1184:Diabetic
1060:med/1483
933:19651590
895:34732507
846:26210726
800:27940460
724:23431071
467:such as
441:Children
379:Children
286:cytokine
225:itself.
187:anasarca
41:podocyte
1315:General
1014:D009402
941:8226984
886:8763186
791:5293332
426:statins
406:, and
324:ascites
278:T cells
149:albumin
109:protein
99:, is a
1049:000496
939:
931:
893:
883:
844:
836:
798:
788:
780:
722:
652:
642:
605:
595:
479:, and
458:Adults
415:Adults
228:Under
67:
1258:Lupus
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290:IL-12
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1025:8230
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