Analysis Of Clinical And Pathological Characteristics Of24Patients With Renal Amyloidosis

Background: With the change of our spectrum of disease, the incidence of therenal amyloidosis tended to increase. These diseases mainly occur in the elderly, therapid clinical progression and poor prognosis and high mortality. The clinicalmanifestation lack of specificity， resulting in a higher rate of misdiagnosis, whichseriously affected the prognosis of the disease.Objective: To investigate the characteristics of renal amyloidosis, to deepen theunderstanding of these diseases and improve the level of diagnosis and treatment.Methods: A retrospective analysis from September2004to March2012, UnionHospital, Huazhong University of Science and Technology Department of Nephrology,hospitalization, passing through percutaneous renal biopsy diagnosis of renalamyloidosis in24patients. To records the the the age of of the all patients, Gender,the first symptom, and other accompanying symptoms, the time from fall ill todefinite diagnosis, past history, family history, causes of death and from thediagnosed to the the time of death, laboratory examination indicators in the afteradmission and the auxiliary information, carry out comparison and statistical analysis.Results: The male to female ratio1.18:1of24patients, average age53.9±8.4yearsold,54.1%between the ages of51-60. The most common first symptom is edema oflower limbs or facial edema (54.1%), followed by chest tightness, palpitations(16.7%). From onset of symptoms to renal biopsy confirmation;renal amyloidosis, themedian duration of6months (0.5—60months).42.8%represent kidney nephroticsyndrome.14cases of renal biopsy was observed only1case (7.1%) large perirenal hematoma.16cases of renal B-ultrasound, only1case (6.3%) renal volume increases.Renal pathological changes:(1) under the light microscope: HE staining extracellularuniform, amorphous, acellular, weakly eosinophilic deposits;(2) Congo red staining;(3) under the electron microscope, ultrastructural branch diameter8~10nm, there isno particular direction, arranged above the fibrils can be distributed in glomerularmesangial glomerular basement membrane, the renal interstitium renal blood vessels.12cases of blood the light chain of examination,11cases (91.7%) of the chain K isreduced.9routine urine protein electrophoresis,4(44.4%) were positive.11patientsof Blood immune protein electrophoresis,7patients (63.6%) of the M protein.9casesof bone marrow biopsy, of which4cases (44.4%) were diagnosed with multiplemyeloma or plasma cell ratio increased.14cases of ECG,7cases (50%) showarrhythmia.90.5%of case the total cholesterol raised up.3patients of6cases (50%)the liver and spleen are enlargement.Conclusion:1.Renal amyloidosis considerable rate of prevalence of sexually transmitted diseases,men and women, more than half of patients aged between51-60years of age. Thedisease high rate of misdiagnosis， symptoms to diagnosis bit longer course6months(0.5—60months).2. This disease lack of specific symptoms, the most common first symptom of lowerextremity edema or facial edema.3. This is a systemic disease, chest tightness, palpitations, hypotension, syncope,arrhythmias, pericardial effusion; gastrointestinal bleeding, dyslipidemia, elevatedliver enzymes, the more common complications such as liver and spleen enlargement.4. Renal involvement with nephrotic syndrome is the most common, can also bemanifested as chronic nephritis syndrome and chronic renal failure; urinary proteinlevels may occur from a small to a large number; performance urinary protein spectrum, largely non-selective proteinuria;ultrasound majority of patients with renalvolume does not increase.5．A large number of amyloid deposition in the mesangial are relate to a large numberof proteinuria; primary or limitations amyloid deposition in the tubular basementmembrane, tubulointerstitial and blood vessels relate toLow levels of proteinuria.6．The blood immunoglobulin light chain level detection, hematuria immune proteinelectrophoresis, bone marrow biopsy and other tests have high positive rate, theseamyloidosis better non-invasive screening tool, in clinical popularity will help toimproveamyloidosis diagnosis and treatment of sexually transmitted diseases.7．Diagnosis depends mainly on renal pathology. Genomics and proteomics to identifyamyloid typing and individualized treatment is the future direction of development.