immunoglobinopathies Dr Sara Amjad immunoglobinopathies
Clinical significance of albumin Prevention from crossing BBB Hypoalbuminemia Malnutrition Nephrotic syndrome Cirrhosis of liver
Colloid osmotic pressure Low albumin, causing edema.
Albuminuria Microalbuminuria Therapeutical use of albumin
Clinical significance of α antitrypsin α antitrypsin deficiency has been implicated in two diseases Emphysema α1 AT deficiency liver disease
Emphysema Abnormal distension of lungs by air 5 percent of emphysema cases are due to deficiency of α1 AT Associated with lung infections Increase in activity of macrophages to release elastase
α1 AT deficiency liver disease Accumulation of a mutant α1 AT which aggregates to form polymers Polymer cause liver damage Accumulation of collagen resulting in fibrosis
α1 Fetoglobulin Presence of α1 Fetoglobulin is usually diagnostically in determining presence of hepatocelluar carcinoma
Clinical significance of Haptoglobin Anemia is associated with decrease plasma concentration of haptoglobin
Transferrin Deficiency PCM Cirrhosis of liver
Clinical significance of Ceruloplasmin: Wilson,s disease Failure to synthesize ceruloplasmin or an impairment in the binding capacity of copper Copper is free in the plasma which easily enters tissue
Manifestations Copper deposition in abnormal amounts in liver and brain Low level of ceruloplasmin in plasma with increase excretion Copper deposition in kidney causes renal damage Intestinal absorption of copper is very high
Multiple myeloma Plasma cell cancer Abnormal Ig production malignancy of a single cone of plasma cell Over production of abnormal immunoglobulin's Decreased immunity Recurrent infections
Electrophoretic pattern of multiple myeloma Sharp distant band M band between beta and gamma globulin Diminished synthesis of gamma globulin
Bence Jones proteins Light chain of immunoglobulin are in excess Renal tubes damage Amyloidosis Deposition of light chains in tissues
Genetic deficiency of plasma proteins Analbuminaemia Complete absence Defect in synthesis Bruton,s agammaglobulinaemia Inherited, X linked Differentiation of beta lymphocytes to plasma cells is defective
Afibrinogenaemia Inherited disorder Defect in fibrinogen formation Uncontrollable hemorrhages
Plasma Proteins