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Glomerular subepithelial immune-complex deposits

MedGen UID:
1782712
Concept ID:
C5539713
Finding
HPO: HP:0033601

Definition

A type of glomerular subepithelial deposit characterized by finely granular material deposited between the outer (epithelial) aspect of the glomerular basement membrane (GBM) and the visceral epithelial cell, with varying degrees of incorporation into the GBM and corresponding to immunoglobulin and/or complement by immunofluorescence/immunohistochemistry. This feature is associated with a prominent GBM reflecting an diffuse and relatively uniform increase in thickness (subjective estimate). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGlomerular subepithelial immune-complex deposits

Conditions with this feature

Nephronophthisis 13
MedGen UID:
482242
Concept ID:
C3280612
Disease or Syndrome
The nephronophthisis (NPH) phenotype is characterized by reduced renal concentrating ability, chronic tubulointerstitial nephritis, cystic renal disease, and progression to end-stage renal disease (ESRD) before age 30 years. Three age-based clinical subtypes are recognized: infantile, juvenile, and adolescent/adult. Infantile NPH can present in utero with oligohydramnios sequence (limb contractures, pulmonary hypoplasia, and facial dysmorphisms) or postnatally with renal manifestations that progress to ESRD before age 3 years. Juvenile NPH, the most prevalent subtype, typically presents with polydipsia and polyuria, growth retardation, chronic iron-resistant anemia, or other findings related to chronic kidney disease (CKD). Hypertension is typically absent due to salt wasting. ESRD develops at a median age of 13 years. Ultrasound findings are increased echogenicity, reduced corticomedullary differentiation, and renal cysts (in 50% of affected individuals). Histologic findings include tubulointerstitial fibrosis, thickened and disrupted tubular basement membrane, sporadic corticomedullary cysts, and normal or reduced kidney size. Adolescent/adult NPH is clinically similar to juvenile NPH, but ESRD develops at a median age of 19 years. Within a subtype, inter- and intrafamilial variability in rate of progression to ESRD is considerable. Approximately 80%-90% of individuals with the NPH phenotype have no extrarenal features (i.e., they have isolated NPH); ~10%-20% have extrarenal manifestations that constitute a recognizable syndrome (e.g., Joubert syndrome, Bardet-Biedl syndrome, Jeune syndrome and related skeletal disorders, Meckel-Gruber syndrome, Senior-Løken syndrome, Leber congenital amaurosis, COACH syndrome, and oculomotor apraxia, Cogan type).
Senior-Loken syndrome 8
MedGen UID:
905171
Concept ID:
C4225376
Disease or Syndrome
Any Senior-Loken syndrome in which the cause of the disease is a mutation in the WDR19 gene.

Recent clinical studies

Therapy

Hirose O, Itabashi M, Takei T, Honda K, Nitta K
Clin Exp Nephrol 2017 Aug;21(4):643-650. Epub 2016 Nov 9 doi: 10.1007/s10157-016-1341-1. PMID: 27830351
Pohl M
Pediatr Nephrol 2015 Feb;30(2):245-52. Epub 2014 Apr 15 doi: 10.1007/s00467-014-2815-6. PMID: 24733586
Olaru F, Luo W, Suleiman H, St John PL, Ge L, Mezo AR, Shaw AS, Abrahamson DR, Miner JH, Borza DB
J Am Soc Nephrol 2014 May;25(5):918-25. Epub 2013 Dec 19 doi: 10.1681/ASN.2013050498. PMID: 24357670Free PMC Article
Haas M, Kerjaschki D, Mayer G
Kidney Int Suppl 1999 Jul;71:S110-2. doi: 10.1046/j.1523-1755.1999.07127.x. PMID: 10412751
Davies DJ, Dowling J, Xipell JM
Pathology 1977 Oct;9(4):281-8. doi: 10.3109/00313027709094448. PMID: 201907

Prognosis

Huang Z, Chen B, Zhou Y, Liang Y, Qiu W, Lv Y, Ding X, Zhang J, Chen C
BMC Nephrol 2021 Jul 5;22(1):248. doi: 10.1186/s12882-021-02462-3. PMID: 34225678Free PMC Article
Almaani S, Parikh SV
Adv Chronic Kidney Dis 2019 Sep;26(5):393-403. doi: 10.1053/j.ackd.2019.08.009. PMID: 31733724
Couser WG, Nangaku M
J Nephrol 2006 Nov-Dec;19(6):699-705. PMID: 17173240
Haas M, Kerjaschki D, Mayer G
Kidney Int Suppl 1999 Jul;71:S110-2. doi: 10.1046/j.1523-1755.1999.07127.x. PMID: 10412751
Helin H, Pasternack A, Hakala T, Penttinen K, Wager O
Clin Nephrol 1980 Jul;14(1):23-30. PMID: 7408252

Clinical prediction guides

Huang Z, Chen B, Zhou Y, Liang Y, Qiu W, Lv Y, Ding X, Zhang J, Chen C
BMC Nephrol 2021 Jul 5;22(1):248. doi: 10.1186/s12882-021-02462-3. PMID: 34225678Free PMC Article
Andeen NK, Jefferson JA, Akilesh S, Alpers CE, Bissonnette ML, Finn LS, Higgins J, Houghton DC, Kambham N, Magil A, Najafian B, Nicosia RF, Troxell ML, Smith KD
Hum Pathol 2018 Nov;81:272-280. Epub 2018 Jul 3 doi: 10.1016/j.humpath.2018.06.031. PMID: 30420049
Hirose O, Itabashi M, Takei T, Honda K, Nitta K
Clin Exp Nephrol 2017 Aug;21(4):643-650. Epub 2016 Nov 9 doi: 10.1007/s10157-016-1341-1. PMID: 27830351
Couser WG, Nangaku M
J Nephrol 2006 Nov-Dec;19(6):699-705. PMID: 17173240
Haas M, Kerjaschki D, Mayer G
Kidney Int Suppl 1999 Jul;71:S110-2. doi: 10.1046/j.1523-1755.1999.07127.x. PMID: 10412751

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