• Share
  • Email
  • Embed
  • Like
  • Save
  • Private Content
A Case of Renal Amyloidosis
 

A Case of Renal Amyloidosis

on

  • 2,613 views

 

Statistics

Views

Total Views
2,613
Views on SlideShare
2,349
Embed Views
264

Actions

Likes
2
Downloads
47
Comments
0

7 Embeds 264

http://smcphysiciansmeet.blogspot.in 194
http://smcphysiciansmeet.blogspot.com 61
http://smcphysiciansmeet.blogspot.com.es 3
http://smcphysiciansmeet.blogspot.co.uk 2
https://www.google.co.in 2
http://www.smcphysiciansmeet.blogspot.com 1
http://www.smcphysiciansmeet.blogspot.in 1
More...

Accessibility

Categories

Upload Details

Uploaded via as Microsoft PowerPoint

Usage Rights

© All Rights Reserved

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Processing…
Post Comment
Edit your comment

    A Case of Renal Amyloidosis A Case of Renal Amyloidosis Presentation Transcript

    • A Case of Anasarca for Evaluation PROF P.VIJAYARHAGAVAN’ S UNIT C. R. RAJ KUMAR
      • 60 yrs male admitted with
      • c/o
      • generalized swelling all over the body - 20days
      • H/O present illness:
      • 20 days back pt was apparently normal then pt
      • C/O Swelling all over the body – 20 days
      • -associated with abdominal distension
      • c/o malaise, easy fatigability +
      • No h/o palpitation , syncope , giddiness
      • No h/o difficulty in breathing, PND, orthopnea
      • No h/o abdominal pain ,
      • No h/o jaundice
      • No h/o hemetemesis or melena
      • No h/o decreased urine output, heamaturia,or dysuria
      • No h/o cough with expectoration
      • No h/o hemoptysis
      • No h/o fever
      • No h/o altered sensorium
      • PAST H/O;
      • No h/o similar illness in the past
      • not a known case of T2DM , SHT, BA ,TB
      • No h/o surgery in the past
      • FAMILY H/O;
      • No h/o similar illness in his family
      • PERSONNEL H/O ;
      • Mixed diet
      • Known alcoholic, smoker for past 20 yrs.
      • He stopped alcohol for 1 ½ yrs.
      • GENERAL EXAMINATION;
      • Pt conscious,
      • Oriented
      • A febrile
      • No pallor
      • No cyanosis,
      • No clubbing
      • Not icteric
      • Bilateral pitting pedal edema ++
      • No generalized lymphadenopathy
      • No signs of liver cell failure
      • VITALS:
      • Pulse – 82/min
      • BP - 110/ 80 mm/Hg
      • JVP – normal
      • RR -18/min
      • CVS – S1S2 heard
      • no murmurs
      • RS - NVBS
      • no added sounds
      • P/A - Distended,
      • Free fluid +
      • no organomegaly
      • CNS - NFND
      • ANASARCA FOR EVALUTION
      • INVESTIGATIONS;
      • CBC;
      • Hb -11.9
      • TC – 21OOO
      • DC – P85 , L -15
      • ESR -5/12
      • PLATELETS- 1.7 lakhs
      • PCV – 36
      • RBC – 3.87
      • RFT:
      • Blood sugar – 98mg
      • Urea -28 mg
      • Creatinine – 0.9mg
      • ECG:
      • Low voltage complexes, T inversion V3 – V6
    •  
    • TOTAL COUNT 8500cells/cumm DC POLYMORPHS 68% LYMPHOCYTES 36% EOSINOPHILS 6% PCV 34.7 MCV 90.8fl MCH 29.3pg MCHC 32.3g HB 11.2gm ESR 38mm/ hr PROTHROMBIN TIME TEST -14 APTT 26 PLATELETS 79000 RBC 3.8 million
    • RFT SUGAR 90mg UREA 18mg CREATININE 0.7mg LFT TOTAL BILIRUBIN 0.42mg DIRECT 0.28mg AST 33u ALT 17 ALK PHOSPATASE 159 GGT 23 TOTAL PROTEIN 4mg ALBUMIN 1.2mg GLOBULINS 2.8mg
    • ELECTROLYTES SODIUM 131.3meq POTTOSIUM 4.33meq CHLORIDE 94.3meq SEROLOGY HIV NEGATIVE ANTIHCV NEGATIVE HbsAg POSITIVE URINE SUGAR NIL ALBUMIN + DEPOSITS 4-6 pus cells, no RBC,no casts
      • LIPID PROFILE:
      • Serum total cholesterol – 204mg
      • Total triglyceride - 230 mg
      • LDL- 110
      • VLDL- 180
      • HDL - 40
      • USG ABDOMEN:
      • Normal sized kidneys with mild increase in cortical echogenicity
      • Minimal ascities +
      • Normal liver echo texture
      • GB, CBD, Portal vein, pancreas, spleen, bladder and prostate
      • Pt was treated with symtomatically with
      • diuretics
      • Inj frusemide and conservative management
      • Meanwhile 24 hrs urinary protein was send
      • Blood culture , urine culture was send
      • Meanwhile pt
      • c/o decreased urine output
      • -increasing swelling all over the body with facial puffiness
      • Repeat RFT was taken
      • It shows increased renal parameters
      • 1.9mg
      • 2.9mg
      • 3.8mg
      • SUSPECTED :
      • AKI – Mutifactorial- diuretics (?pre-renal/?cast nephropathy)/ sepsis
      • Nephrology opinion obtained
      • ?Nephrotic syndrome
      • Suggested
      • -Viral markers
      • 24 hrs urinary protein:
      • -1250mg /day
      • Urine Bence Jones protein
      • - negative
      • Serum calcium - 9.2mg
      • Thyroid function test – normal
      • Peripheral smear study - normal
      • CARDIAC EVALUATION:
      • concentric LVH
      • No RWMA
      • Normal LV systolic function
      • MR mild
    • SERUM IMMUNO ELECTROPHORESIS
    •  
      • Serum immuno electrophoresis:
      • IgA - 447.1mgmg/dl [70 – 400mg/dl]
      • IgM - 381.55mg/dl [700 – 1600mg/ml]
      • IgG - 67.25mg/dl [40-230mg/dl]
      • IMPRESSSION:
      • IgA monoclonal gammapathy
      • Bone marrow:
      • normal study
    • RENAL BIOPSY
    •  
      • Renal Biopsy (Microscopic description):
      • -blood vessels are thickened and show minimal a cellular material which are CONGO RED positive
      • -There are multiple foci of tubular atrophy. apple green bifringence seen under polarized light which is resistant to pre treatment by potassium permanganate
      • -Immmunoflurecence stain shows minimal mesangial deposits of IgM
      • FINAL REPORT OF RENAL BIOPSY:
      • Renal biopsy showing features of AMYLOIDOSIS [NON –AA]
      • After 10 days
      • pt RFT was normal after stopping diuretics and coverage with broad spectrum antibiotics
      • -serum creatinine - 0.8mg
      • -24 hrs urinary protein – 950 mg
      • -pt symptomatically improved
      • FINAL DIAGNOSIS:
      • IgA monoclonal gammapathy
      • Primary amyloidosis AL [non AA]
      • AKI recovered
      • HbsAg positive (possibly explaining IgM deposition in renal biopsy)
      • Journal of Transplantation Volume 2009 (2009), Article ID 103784, 3 pages doi:10.1155/2009/103784Case Report
      • Hepatitis B Associated Monoclonal Gammopathy That Resolved after Successful Liver Transplant
      • P. Sreenivasan and S. Nair
      • The role of hepatitis B surface antigen in Nigerian children with nephrotic syndrome.
      • Abdurrahman MB, Fakunle YM, Whittle HC.
      • Abstract
      • Hepatitis B surface antigen was detected by radioimmunoassay in the sera of 18 out of 50 (36%) children with nephrotic syndrome and in 28 of 61 (45.9%) controls . Immunofluorescent studies of kidney biopsies showed HBsAg, IgG, IgM and C3 deposits in a granular pattern in the biopsies of 12 children with nephrotic syndrome and in none of the control kidney biopsies, even though there was no significant difference between the frequencies of HBsAg in the sera of these two groups. We conclude that these findings are indicative of an aetiologic role for HBsAg in these patients
      • PMID: 6191643 [PubMed - indexed for MEDLINE]
      • Publication Types, MeSH Terms, Substances
      • Monoclonal gammopathy of undetermined significance (MGUS) has been most commonly associated with diseases like
      • -multiple myeloma,
      • -Waldenstrom's macroglobulinemia,
      • -primary systemic amyloidosis,
      • and other lymphoproliferative disorders.
      • There has been an isolated report of MGUS in patients coinfected with HIV and Hepatitis B, as the work by Amara et al. in 2006. Here , we report a case of IgA-kappa light chain gammopathy secondary to Hepatitis B infection, which resolved after liver transplantation.
      t
    • MONOCLONAL GAMMOPATHY
      • M protein in the serum without symptoms or signs of multiple myeloma, macroglobulinemia, amyloidosis, or lymphoma. Less than 10 % plasma cells in the bone marrow .
      • The incidence of monoclonal gammopathy of uncertain significance (Monoclonal Gammopathy of Uncertain Significance ) increases with age and may approach 3% in persons 70 years of age or older
      • Lymphoid malignancies, amyloidosis, or multiple myeloma will develop in as many as one-third of patients with apparently benign monoclonal gammopathies.
      • Two major types of MGUS :
      • -IgM MGUS and
      • -non- IgM MGUS [mostly comprised of IgG and IgA]
      • Patients with non-IgM MGUS progress to
      • multiple myeloma at a rate of 1% per year
      • No specific therapy is necessary, but close observation is required.
      • MGUS patients should be periodically monitored for changes in serum M proteins, urinary Bence-Jones proteins, evidence of renal failure, anemia, hypercalcemia, lytic bone lesions, or bone marrow plasmacytoses.
      • Risk of developing a malignant disorder is 12% at 10 years, 25% at 20 years, and 30% at 25 years.
      • favorable prognosis
      • concentrations of homogeneous immunoglobulin less than 2 g/dL,
      • no increase in concentration of the immunoglobulin from the time of diagnosis,
      • no decrease in the concentration of normal immunoglobulins,
      • absence of a homogeneous light chain in the urine, and
      • normal hematocrit and serum albumin
    • *see below Disease M-protein type Associated clinical features Monoclonal Gammopathy of Uncertain Significance (MGUS) Any in small quantity None Multiple Myeloma IgG, IgA, light chain only, IgD CRAB* Lymphoplasmacytoid-Cell Lymphoma (Waldenström's Macroglobulinemia) IgM OVA* amyloidosis Any of the above Protein deposition in multiple organ systems
      • AL AMYLOIDOSIS
      • Definition
      • Clinical manifestations (examples only – there are lots more)
      Underlying disorder Effects MGUS, MM, WM Light chains metabolized to form amyloid which accumulates in organs Organ infiltrated Effects Heart Diastolic dysfunction - CHF Liver/spleen Enlargement Kidney Nephrotic syndrome, renal failure Nerves Neuropathy Skin Bleeding, bruising Gut Large tongue. Dysmotility Treatment Chemotherapy Prognosi Poor: average survival about 1 year ( Kyle 1997 ) Treatment Chemotherapy Prognosis Poor: average survival about 1 year ( Kyle 1997 )
      • THANK YOU