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Membranoproliferative glomerulonephritis, X-linked

MedGen UID:
336706
Concept ID:
C1844501
Disease or Syndrome
Synonym: MESANGIOCAPILLARY GLOMERULONEPHRITIS, X-LINKED
 
Monarch Initiative: MONDO:0010596
OMIM®: 305800

Definition

Habib et al. (1973) recognized 2 morphologic classes for the glomerular changes seen in patients with mesangiocapillary (membranoproliferative) glomerulonephritis (MPGN). Type I is characterized by double contour appearance of the capillary walls due to mesangial cell interposition, with nonargyrophilic subendothelial deposits which are finely granular on electron microscopy. Type II is characterized by linear dense deposits within the basement membrane and only rare double contours. These 2 types appear to be distinct with no conversion of one type to another on serial biopsy. Strife et al. (1977) described a third variety in which there are not only subendothelial deposits but also numerous subepithelial and intramembranous deposits, associated with replication of the lamina densa and frequently disruption of the whole basement membrane. [from OMIM]

Clinical features

From HPO
Cardiac shunt
MedGen UID:
488795
Concept ID:
C0232180
Finding
Pattern of blood flow in the heart that deviates from the normal circuit of the circulatory system.
Lipodystrophy
MedGen UID:
6111
Concept ID:
C0023787
Disease or Syndrome
Degenerative changes of the fat tissue.
Mesangiocapillary glomerulonephritis
MedGen UID:
9033
Concept ID:
C0017662
Disease or Syndrome
A type of glomerulonephritis characterized by diffuse mesangial cell proliferation and the thickening of capillary walls due to subendothelial extension of the mesangium. The term membranoproliferative glomerulonephritis is often employed to denote a general pattern of glomerular injury seen in a variety of disease processes that share a common pathogenetic mechanism, rather than to describe a single disease entity
Abnormality of metabolism/homeostasis
MedGen UID:
867398
Concept ID:
C4021768
Finding

Recent clinical studies

Etiology

Deltas C, Gale D, Cook T, Voskarides K, Athanasiou Y, Pierides A
Adv Exp Med Biol 2013;735:189-96. doi: 10.1007/978-1-4614-4118-2_12. PMID: 23402027

Diagnosis

Hussein MRA, Babiker M, Asim S, Elsamwal M
Saudi J Kidney Dis Transpl 2023 Nov 1;34(6):660-665. Epub 2024 May 9 doi: 10.4103/sjkdt.sjkdt_133_23. PMID: 38725215
Tan L, An Y, Yang Q, Yang H, Zhang G, Li Q, Wang M
Mol Genet Genomic Med 2022 Jun;10(6):e1945. Epub 2022 Apr 18 doi: 10.1002/mgg3.1945. PMID: 35434975Free PMC Article
Deltas C, Gale D, Cook T, Voskarides K, Athanasiou Y, Pierides A
Adv Exp Med Biol 2013;735:189-96. doi: 10.1007/978-1-4614-4118-2_12. PMID: 23402027
Yoshino A, Honda M, Kanegane H, Obata K, Matsukura H, Sakazume S, Katada Y, Miyawaki T, Ueda Y, Nagai T
Pediatr Nephrol 2006 Jan;21(1):36-8. Epub 2005 Nov 11 doi: 10.1007/s00467-005-2029-z. PMID: 16550363
Bogdanović RM, Dimitrijević JZ, Nikolić VN, Ognjanović MV, Rodić BD, Slavković BV
Pediatr Nephrol 2000 May;14(5):400-5. doi: 10.1007/s004670050782. PMID: 10805468

Therapy

Hussein MRA, Babiker M, Asim S, Elsamwal M
Saudi J Kidney Dis Transpl 2023 Nov 1;34(6):660-665. Epub 2024 May 9 doi: 10.4103/sjkdt.sjkdt_133_23. PMID: 38725215
Yoshino A, Honda M, Kanegane H, Obata K, Matsukura H, Sakazume S, Katada Y, Miyawaki T, Ueda Y, Nagai T
Pediatr Nephrol 2006 Jan;21(1):36-8. Epub 2005 Nov 11 doi: 10.1007/s00467-005-2029-z. PMID: 16550363

Prognosis

Deltas C, Gale D, Cook T, Voskarides K, Athanasiou Y, Pierides A
Adv Exp Med Biol 2013;735:189-96. doi: 10.1007/978-1-4614-4118-2_12. PMID: 23402027
Foster K, Markowitz GS, D'Agati VD
Semin Nephrol 2005 May;25(3):149-58. doi: 10.1016/j.semnephrol.2005.01.006. PMID: 15880325
Bogdanović RM, Dimitrijević JZ, Nikolić VN, Ognjanović MV, Rodić BD, Slavković BV
Pediatr Nephrol 2000 May;14(5):400-5. doi: 10.1007/s004670050782. PMID: 10805468

Clinical prediction guides

Deltas C, Gale D, Cook T, Voskarides K, Athanasiou Y, Pierides A
Adv Exp Med Biol 2013;735:189-96. doi: 10.1007/978-1-4614-4118-2_12. PMID: 23402027

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