A Case of Primary Systemic Amyloidosis Predominantly Presenting Nephrotic Syndrome and Peripheral Neuropathy.
- Author:
Yun Sang BAE
1
;
Eun Ah LEE
;
Ji Hyock RHEE
;
Sang Ho LEE
;
Man Jong CHO
;
Seon Hee CHOI
;
Young Jae KO
;
Byoung Jun KIM
;
Sung Joo LEE
;
Yoon Shig YANG
Author Information
1. Department of Internal Medicine, Pusan Medical Center, Pusan, Korea.
- Publication Type:Case Report
- Keywords:
Primary systemic amyloidosis;
Nephrotic syndrome;
Perpheral neuropathy
- MeSH:
Aged;
Amyloidosis*;
Biopsy;
Capillaries;
Diagnosis;
Edema;
Extremities;
Female;
Heart;
Heart Failure;
Humans;
Hypotension, Orthostatic;
Kidney;
Kidney Failure, Chronic;
Leg;
Methenamine;
Nephrotic Syndrome*;
Paresthesia;
Peripheral Nervous System Diseases*;
Prognosis;
Renal Insufficiency;
Survival Rate
- From:Korean Journal of Nephrology
1997;16(3):596-602
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Primary systemic amyloidosis is a progressive disease that is frequently fatal. Nephrotic syndrome is present in almost one-third, congestive heart failure in one-quarter, and peripheral neuropathy in one-sixth of patients at the time of diagnosis. If heart or renal failure are presented, survival rate is poor. We experienced a case of a 66 year-old female patient who had complained lower leg edema and paresthesia of extremities for about 5 months. The laboratory findings were consistent with nephrotic syndrome, but the lower leg edema was non-pitting and the cause of paresthesia was unknown. We performed kidney and nerve biopsy and confirmed a case of primary systemic amyloidosis. In this case, presence of postural hypotension, probable cardiac involvement and relatively long spikes along the outside of the glomerular capillary loops on methenamine silver stain is suggestive of poor prognosis. We can predict chronic renal failure and congestive heart failure in the course of this case. We report a case of primary systemic amyloidosis predominantly presenting nephrotic syndrome and peripheral neuropathy with review of related literatures.
