U06-20274 #324676110 Creat 250 Nephritic urine ? Crescentic GN.

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U # Creat 250 Nephritic urine ? Crescentic GN

54-year old Caucasian male CC: Leg Edema, Skin Rash, Leg Pain and hands numbness. HPI: 8 Wks prior to admission he noticed leg edema and rash. Treated with Cephazolin and cephalexin for few days for cellulitis. No URI, No Fever, No gross hematuria, No Sore throat. PMH: Asthma (using combivent PRN)

Labs: (on admission) Hgb: 132 Cr 247 (Baseline Cr: 110) U/A: SG of >1.030 Pro/Crea: ALT, AST and Alk.Pho: NL dsDNA: Neg, RF(Sep2006): 22 ANA: NEG p-ANCA and c-ANCA: NEG X 2 C3 and C4: 1.33 and.91

Kidney Biopsy on 25-OCT-06 ?

IF IgG- Negative. IgA- Moderate irregular peripheral lobular finely granular staining. IgM- Negative. C3- Moderate irregular peripheral lobular staining. Moderate vascular staining. C1q- Negative. Kappa- Negative. Lambda- Negative. Fibrin- Mild interstitial staining. Albumin- Negative.

IgA

C3

Fibrin

EM Will be ready in the coming weeks.

Diagnosis Renal Biopsy: Focal proliferative GN with peripheral lobular granular staining for IgA and possible arteriolitis with necrotizing changes. Rule out Henoch-Schönlein purpura. Comment: the IF pattern is unusual but has been described in Henoch-Schönlein purpura.

Series of 10 paediatric cases A/a focal and segmental hypercellularity with # monocytes IgA deposits at periphery of lobules, not in mesangium Early phase of disease ? 3/6 subsequent biopsy was normal (with no IgA deposition) 3/6 subsequent biopsy with mesangial IgA