Name
MALABSORPTION SYNDROME
DESCRIPTION
DETAIL
CAUSED BY EXCESSIVE PRODUCTION OF IMMUNOGLOBULIN LIGHT CHAIN ( AL ). TYPES OF AMYLOIDOSIS : - PRIMARY - SECONDARY ( INFLAMMATORY ) - FAMILIAL - SENILEOTHER TESTS - * CONGO RED STAINING OF BIOPSY PRODUCES APPLE-GREEN BIREFRINGENCE UNDER POLARIZED LIGHT * ELECTRON MICROSCOPY - CHARACTERISTIC FIBRILLAR PATTERN OF AMYLOID DEPOSITS * PROTEIN OR DNA STUDIES - MUTANT PROTEIN IDENTIFICATION , IMMUNOCYTOCHEMISTRY, IMMUNOFLUORESCENT OR IMMUNOPEROXIDASE STAINS WITH SPECIFIC ANTISERA. * PROTEINURIA * ECG SHOWS LOW VOLTAGE COMPLEXES WITH ATRIOVENTRICULAR OR INTRAVENTRICULAR CONDUCTION ABNORMALITIES. * ECHOCARDIOGRAPHY - DIFFUSE THICKENING OF VENTRICULAR WALLS WITH HYPOKINESIA & REDUCED CAVITY SIZE & A DIFFUSE HYPERREFRACTIVE GRANULAR SPARKLING APPEARANCE OF WALLS. * CARDIAC CATHETERIZATION WITH ENDOMYOCARDIAL BIOPSY IN CASES OF CARDIAC DISEASE IS GOLD STANDARD * AVOID IVP - BECAUSE DYE EXPOSURE MAY LEAD TO ACUTE RENAL FAILURE * RADIOLABELLED P- COMPONENT GAMMA SCANNING - TO DEMONSTRATE THE TOTAL BODY BURDEN OF AMYLOID & ITS DISAPPEARANCE AFTE SUCCESSFUL TREATMENT IS DIAGNOSTIC * X-RAY CHEST - MAY SHOW CARDIOMEGALY, PLEURAL EFFUSION & PULMONARY CONGESTION
TYPENOTES
TREATMENT FOR CARDIAC CONDITION : - TT. OF UNDERLYING DISEASE PROCESS LIKE MULTIPLE MYELOMA MAY HELP THE HEART. - SALT RESTRICTION - DIGITALIS TOXICITY CAN OCCUR AT MUCH LOWER DOSES BECAUSE IT IS BOUND EXTRACELLULARLY BY AMYLOID FIBRILS. - DIURETICS CAN HELP. CARE FOR HYPOTENSION - CALCIUM CHANNEL BLOCKERS BY BINDING TO AMYLOID FIBRILS CAN LEAD TO IMPAIRED CARDIAC CONTRACTILITY & HYPOTENSION - ACE INHIBITORS CAN BE USEFUL, CARE FOR ORTHOSTATIC HYPOTENSION - INTRAVENOUS MELPHALAN IS USED TO TREAT PRIMARY AMYLOIDOSIS AS WELL AS THE ASSOCIATED CARDIOMYOPATHY
RELATED DISEASE
Not Available Disease
Disease
Remarks
AMYLOIDOSIS, PRIMARY
MAY BE SEEN
COMMON PATHOLOGY
QUITE COMMON PATHOLOGY
MAY BE SEEN ALONG WITH SYSTEMIC EMBOLI
DUE TO AUTONOMIC NEUROPATHY
DISEASE
INVESTIGATION
URINE ROUTINE, X-RAY CHEST P.A. VIEW( NORMAL ), COMPLETE BLOOD COUNT, ECHOCARDIOGRAPHY, ECG, BIOPSY