There is now strong evidence that DDD is caused by uncontrolled AP activation.Spontaneous remissions of MPGN II are rare; approximately half of those affected with MPGN II will progress to end stage renal disease within ten years.In many cases, people with MPGN II can develop drusen caused by deposits within Bruchs membrane beneath the retinal pigment epithelium of the eye. Over time, vision can deteriorate, and subretinal neovascular membranes, macular detachment, and central serous retinopathy can develop. Type III
Type III is very rare, it is characterized by a mixture of subepithelial and subendothelial immune and/or complement deposits. These deposits elicit an immune response, causing damage to cells and structures within their vicinity. Has similar pathological findings of Type I disease.A candidate gene has been identified on chromosome 1.Complement component 3 is seen under immunofluorescence. it is associated with complement receptor 6 deficiency. Pathology
Membranoproliferative glomerulonephritis involves deposits at the intraglomerular mesangium. It is also the main hepatitis C associated nephropathy. It also is related to a number of autoimmune diseases, prominently systemic lupus erythematosus (SLE), Class IV. Also found with Sjögren syndrome, rheumatoid arthritis, inherited complement deficiencies (esp C3 deficiency), scleroderma, Celiac disease.The histomorphologic differential diagnosis includes transplant glomerulopathy and thrombotic microangiopathies. Diagnosis
The GBM is rebuilt on top of the deposits, causing a "tram tracking" appearance under the microscope. Mesangial cellularity is increased. Treatment
Primary MPGN is treated with steroids, plasma exchange and other immunosuppressive drugs. Secondary MPGN is treated by treating the associated infection, autoimmune disease or neoplasms.
The term "testicular feminization" was coined to reflect Morris observation that the testicles in these patients produced a hormone that had a feminizing effect on the body, a phenomenon now understood to be due to the inaction of androgens, and subsequent aromatization of testosterone into estrogen. A few years before Morris published his landmark paper, Lawson Wilkins had shown through experiment that unresponsiveness of the target cell to the action of androgenic hormones was a cause of "male pseudohermaphroditism". Wilkins work, which clearly demonstrated the lack of a therapeutic effect when 46,XY patients were treated with androgens, caused a gradual shift in nomenclature from "testicular feminization" to "androgen resistance". Other names
A distinct name has been given to many of the various presentations of AIS, such as Reifenstein syndrome (1947), Goldberg-Maxwell syndrome (1948), Morris syndrome (1953), Gilbert-Dreyfus syndrome (1957), Lubs syndrome (1959), "incomplete testicular feminization" (1963), Rosewater syndrome (1965), and Aimans syndrome (1979).