. HEHEDITARY DISORDERS - (a) Airport's syndrome -Progressive nesphritis and sensorlneural hearing loss.

glomerular diseasesr whether occurring. In or as pan of multisystem diseases are associated with demonstrabie immunologial abnormalities. There are two possible mechani sms. 1. Antigan In kidney - (in situ complex formailon) - Antigen may be an Intrinsic pan of the glomerulus. or derived from other parts of the body and deposited in the glomerulus. Thus formation of antigen-antibody complex can occur if an appropriate antibody is generated 2 Antigen remote from the kidiay - (Antigien either antbgenole or exogenous from other sources than the kidney) - When the antigen-antibody complex gains access to. or produced in the circulation* it can be deposited in the kidneys, where it activates mediators of inflammation The production of glomerular injury is complex and probably depends on tanked celIular and non-cellular mechanisms. Irrnxinohlsilpolhologcal study of renal tissue from renal tiiopsies reveals antibody (lgG. IgM or IgA) and complement compoments. Also tha commonn finding of monocytea. macrophages (producedrs of TNF-alpha and interleukin-1) and T helper lymphocyies (producers of intareuk-2) suggests a rola'or these cells and cytoknies In pathogeneste CAUSES of glorrerular dsease: 1. HEHEDITARY DISORDERS - (a) Airport's syndrome -Progressive nesphritis and sensorlneural hearing loss. Characteristic basket-weave1 appearance of glomarular basemenl membrane. (b) Thin membrane basement disease • presents as racurrant hamaturia (c) Familial clusters- Some conditions like minimal change nenphroapathy. local and sugmental glomerulosclerosis hava been reported in famiiai clusters. 2. SECONDARY AND SVSTEMlC DISORDERS - Glomerulopathy occurs as pan of systemic disease (e.g SLE) or secondary to a disorder