1. RENAL CYSTIC DISEASE-
(A) AUTOSOMAL RECESSIVE RENAL POLYCISTIC DISEASE- A BILATERAL CYSTIC DISEASE OF THE KIDNEY INHERITED AS AN AUTOSOMAL RECESSIVE TRAIT.
The condition most frequently present at birth, with severe nephromegaly, dilatation and elongation of collecting tubules, sponginess of the cortex and small cysts in the medulla, often associated with ductal plate malformation, pulmonary, hypoplasia, the potter facies, uremia and sometimes skeletal abnormalities, in such cases death occurs with in a few days.
AUTOSOMAL DOMINANT RENAL POLYCYSTIC DISEASE (ADULT POLYCYSTIC KIDNEY DISEASE)-
A bilateral cystic disease of the kidney inherited as an autosomal dominant trait. Onset may occur at any time of life, but most frequently occurs in the fourth to sixth decades. Cases in newborn infants are being recognized with increasing frequency. Cysts may develop anywhere in the nephron and the collection system, and their enlargement leads to extremely severe nephromegaly. Hypertension almost occurs. Symptomatic cystic liver disease and hepatic fibrosis occur occasionally. The term GLOMERUELOCYSTIC KIDNEY DISEASE is applied to a heterogeneous group of disorders, which is characterized by the presence of numerous glomerular cysts in variable combinations with tubular and ductal cysts.
MEDULLARY SPONGE KIDNEY:-
This is a sporadic abnormality characterized by wide cysticdilation of distal collecting ducts in the medulla. Calculi containing calcium phosphate are frequently found in the cysts and stone disease is a recognized complication. It is asymptomatic with no impairment of renal function. Urinary tract infection appears to be common.
A cysts that is usually either lymphatic in origin or the result of subcapsular extravasation of urine which may be due to trauma or to urinary tract obstruction.
RENAL MEDULLARY CYSTIC DISEASE (ADULT TYPE) –
The kidneys are reduced in size as a result of cortical atrophy. This is a hereditary tubulo interstitial nephropathy of autosomal dominant. Cystic changes appear to be a necessary part of the disease. Onset most commonly occurs at the age of 20-40 years with polyuria polydipsia, a severe concentration defect and anemia progression to renal failure is rapid.
FAMILIAL JUVENILE NEPHRONOPHTHISIS-
A hereditary tubulointerstitial nephropathy of autosomal recessive inheritance resembling renal medullary cystic disease but differs from the later by its onset in childhood with severe retardation of growth and by its mode of inheritance.
This disorder may be associated with hereditary atrophy of the optic nerve and retina and retinitis pigmentosa, the combination inherited as an autosomal recessive trait, has been referred to as renal-retinal dysplasia. It may also be associated with congenital hepatic fibrosis. A syndrome inherited as an autosomal recessive trait characterized by gross proteinuria, oedema and inanition presenting in the first few weeks after birth, most commonly in infants.
The condition is rapidly progressive and death usually occurs in the first year of life in the absence of dialysis or renal transplantation. Affected infants are usually premature and of low weight, the placenta is unusually large. Tubular dilation and small cysts are commonly found in the cortex.
INFANTILE NEPHROTIC SYNDROME WITH DIFFUSE MESANGIAL SCLEROSIS –
The disorder is manifested by severe proteinuria and oedema present during the first year of life. The condition progresses to renal insufficiency and uremia, death usually occurs before 3 yr. of age.
A syndrome believed to be inherited as an autosomal recessive trait characterized by diffuse mesangial sclerosis of the glomeruli with atrophy of the tubules and interstitial fibrosis.
2. GLOMERULAR DISEASE :-
(A) MINIMAL-CHANGE NEPHROTIC SYNDROME:-
A glomerular disease with selective protinuria (albumin, oedema and usually hypercholesterolaemia)
(B) FOCAL/SEGMENTAL GLOMERULAR HYALINOSIS AND SCLEROSIS:
A glomerular disease in which accumulation of eosinophilic material, some of which has the appearance of mesangial matrix and some of which is amorphous and capillary collapse gives rise to an area of glomerular abnormality sometimes affects predominantly hextamedullary glomeruli. It may occur in association with several other conditions notably reflux nephropathy, diabetic nephropathy and the acquired immunodeficiency syndrome and in drug addicts.
(C) FOCAL GLOMERULONEPHRITIS:-
A general term for glomreulonephritis in which the glomeruli show proliferative or narcotizing changes in one or two lobules; the changes are therefore both focal and segmental. The disorder may occur as a primary glomerular disease, but is usually the result of renal involvement in certain systemic diseases. It is often self-limiting, although there may be segmental scar formation after healing. The clinical manifestations are variable.
(D) MEMBRANOUS GLOMERULONEPHRITIS:-
Forms of glomerulonephritis characterized by thickening of the glomerular walls resulting from the formation of subepithelical at protein deposits around which the basement membrane forms projections which later coalese with consequent thickening of the membrane The mesangium is usually unaffected. Renal vein thrombosis is an occasional complication.
(E) MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS:-
Forms of GLOMERULONEPHRITIS characterized by fairly uniform or irregular mesangial hypercellularity of varying degree in almost all glomeruli, with no involvement of the leumina, in the late stages, the major lesion is frequently mesangial sclerosis Crescentic proliferation may occur.
(F) ENDOCAPILLARY PROLIFERATIVE GLOMERULONEPHRITIS:-
A form of glomerulonephritis characterized by fairly uniform hypercellularity of tufts in almost all glomeruli, the leumina being narrowed or occluded; hypercellularity results from a combination of leukocyte infiltration and mesangial cell proliferation. Crescents may complicate the course occasionally. The manifestations vary widely, but the acute nephritic syndrome is characteristic; the nephrotic syndrome may occur occasionally.
(G) MESANGIOCAPILLARY GLOMERULONEPHRITIS:-
Glomerular deposits of immunoflobulins are often found and c3 is almost always present. The manifestations vary widely. Nephrotic syndrome occurs at the time of onset in about half of the cases and eventually develop in most. Serum complement is usually depressed. Most cases end in renal failure, with few patients surviving more than 15 years. Focal and segmental forms of the disorder occur usually with milder clinical expression. Most of cases are idionathic. The disease is usually manifested by proteinuria, haematuria and the nephrotic syndrome often with renal insufficiency and hypertension. Renal failure is usually more rapidly progressive and the prognosis poorer, than in mesangiocapillary glomerulonephritis.
(H) CRESCENTIC GLOMERULONEPHRITIS:-
Rapidly progressive forms of glomerulonephritis characterized by the formation of cellular, fibrocellular or fibrous crescents in most of the glomeruli,. Deposits of immunogloubulins are generally scanty or absent. The diseases is usually of insidious onset often following an illness of the upper respiratory tract, with hacmaturia, moderate proteinuria and azotaemia.
(I) SCLEROSING GLOMERULONEPHRITIS:-
A condition that may arise as a result of numerous glomerular diseases, characterized by a reduction in the size of the kidney and in the number of glomeruli, all of which show severe sclerosis. The onset is usually insidious and the condition slowly progressive over a period of many years with haematuria, proteinuria renal insufficiency and hypertension leading ultimately to renal failure.
3. RENAL DISEASE INDUCED BY CHEMICALS:-
Renal disease may be caused by many chemical substances, including drugs used therapeutically and toxins other than drugs, exposure to such substances may occur by inhalation, ingestion, parental administration or absorption through the skin. In general, two different types of chemically induced renal diseases are recognized that due to an idiosyncratic (hypersensitivity) reaction which is unpredictable and not dose related. Some renal disease of latter type may arise as the result of effects on other organ systems.
(A) TOXIC NEPHROPATHY:-
A general term for nephropathy induced by the action of a chemical substance as the result of either direct toxicity or an idiosyncratic reaction, the toxic agent may be a drug or some other chemical substance that is inhaled, ingested, administered parentally or absorbed through the skin. The disorder may be either acute or chronic.
(B) DIRECT TOXIC NEPHROPATHY:-
A general term for nephropathy (tubular necrosis) due to direct toxic action of chemical substances, characterized by degeneration and franc necrosis or tubular epithelial cells especially in the pronominal convoluted tubules, it frequently leads to acute renal failure. In many cases the nephropathy takes both acute and chronic forms. The toxins most often implicated are organic solvents, heavy metal and certain drugs.
(C) CISPLATIN NEPHROPATHY: –
A form of nephropathy caused by cisplatin an antineoplastic agent resembling nephropathy caused by other renal failure may occur. Chronic nephropathy is characterized by tubular atrophy and by interstitial oedema and fibrosis.
(D) LEAD NEPHROPATHY:-
Renal involvement in acute or chronic lead intoxication. The acute form is characterized by the presence of nuclear inclusion bodies, which have the appearance of a fibrillar network with a solid center.
(E) MERCURY NEPHROPATHY:-
Renal involvement in acute or chronic mercury intoxication. The signs depend on the degree and duration of exposure, there is proteinuria, the nephrotic syndrome may occur and there is often renal insufficiency.
(F) GOLD NEPHROPATHY:-
A form of nephropathy that sometimes occurs as the result of prolonged exposure to gold, silver, copper, iron, bismuth, uranium, carbon tetrachloride or ethylene glycol compounds characterized by membranous glomerulonephritis accompanied by protienuria which may lead to the nephrotic syndrome and chronic renal failure.
(G) PENCILLAMINE NEPHROPATHY:-
Nephropathy that develops in up to 30% of patients undergoing pencillamine therapy manifested by proteinuria often leading to the nephrotic syndrome.
(H) AMINOGLY CONSIDE NEPHROPATHY:-
A general term for nephropathy caused by aminogly consides tubular necrosis, may occur and there may be proteinuria elevated serum creatinine and decreased creatinine durance. The disorder most frequently occurs in-patients with renal impairment, those who are dehydrated, the elderly, or incase of high dosages or prolonged administration of aminoglycosides.
(I) A form of nephropathy caused by tetracycline or ciclosporin, occurring in variable proportion of patients to whom this drug is administered to prolong allograft survival.
(J) SULFONAMIDE NEPHROPATHY:-
This disorder is now rare however, a hypersensitivity reaction to sulfonamides may give rise to acute tubulo interstitial nephritis.
(K) ANALGESIC NEPHROPATHY:-
A general term for a chronic tubulo interstitial nephritis due to excessive use of certain analgesics especially phenacetin. There is a risk of transitional cell carcinoma in the renal pelvis and the ureter. In addition to phenacetin, aspirin and paracetamol have been implicated.
(L) LITHIUM NEPHROPATHY:-
Renal damage caused by lithium compounds, it may be acute or chronic. In chronic lithium nephropathy, which occurs as a rare complication of long term lithium treatment of certain psychiatric disorders, there is a chronic tubulo-interstitial nephritis.
(M) HYPERSENSITIVITY NEPHROPATHY:-
A general term for nephropathy arising as a result of an idiosyncratic reaction to a chemical substance; renal histology and clinical manifestations are variable.
4. MISCELLANEOUS RENAL DISEASES :-
(A) DIABETIC GLOMERULOSCLEROSIS
(B) OXALATE NEPHROPATHY
(C) URIC ACID NEPHROPATHY
(D ) HYPERCALCAEMIC NEPHROPATHY
(E) HYPOLICALAEMIC NEPHROPATHY
(F) THIN GLOMERULAR BASEMENT MEMBRANE SYNDROME
(G) ALPORT SYNDROME
(H) ANTIGLOMERULAR BASEMENT MEMBRANE GLOMERULONEPHRITIS
(I) GOODPASTURE SYNDROME
(J) LUPUS NEPHRITIS
(K) RADIATION NEPHRITIS
(L) PRE-ECLAMPTIC NEPHROPATHY
(M) BALKAN ENDEMIC NEPHROPATHY
(N) RENAL INVOLVEMENT IN DYSPROTEINAEMIAS
(O) CRYOGLOBULINAEMIC NEPHROPATHY
(P) LIGHT-CHAIN DEPOSIT NEPHROPATHY