Author/Editor		Kaplan-Pavlovčič, S; Kveder, R; Vizjak, A; Bren, AF
Title		Clinical characteristics of patients with light chain deposition disease
Type		članek
Source		In: Buturović-Ponikvar J, Bren AF, editors. Zbornik 2. slovenski nefrološki kongres z mednarodno udeležbo ob 30. letnici dialize in transplantacije ledvic v Sloveniji; 2000 sep 27-30; Brdo pri Kranju. Ljubljana: Klinični center, Klinični oddelek za nefrologijo,
Publication year		2000
Volume		str. 317-21
Language		eng
Abstract		In retrospective study we evaluated clinical characteristics of 15 patients with light chain deposition disease (LCDD). Patients were aged from 39 to 75 years there were 11 men and 4 women. On renal biopsy deposition of monoclonal light chains (kappa 11, lambda 4) in the glomerular mesangium and basement membrane, as well in the tubular basement membrane a pattern consistent with light chain deposition disease was seen. Diffuse plasmocytoma was diagnosed in 7 patients simultaneous with nephropathy. The mode of onset of nephropathy was acute or rapidly progressive renal failure in 5 (30 %) patients, chronic renal failure with the concentration of serum creatinine from 200 to 533 in 10 patients. All patients had proteinuria (from 0,9 to 10 g/24 hours), nephrotic syndrome was present in 3 patients. Monoclonal component was not detected in serum in 3 (20 %) patients, in urine in 5 (30 %) patients. The monoclonal imunoglobulines detected in serum and/or urine were IgM kappa (1), kappa (8), IgG lambda (2), IgG kappa (1), IgA kappa (1). Arterial hypertension was presented in 8 pts (60 %). Nine patients were treated by melphalan and methylprednisolone, 4 patients with vincristine, adriamycin and dexamethasone, two patients with plasma exchange too. One patient recovered renal function after the treatment and stopped dialysis treatment. Five patients were treated by hemodialysis immiadetly after the presentation of the disease. Conclusion: The most common clinical findings in LCDD were proteinuria from mild to nephrotic range and renal insufficiency mostly rapidly worsening. In most cases of LCDD a monoclonal protein was be detected in serum and/or urine, mostly in minute quantities.
Descriptors		IMMUNOGLOBULINS, LIGHT-CHAIN KIDNEY FAILURE