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Sepsis caused by dialysis-related amyloidosis on the buttocks
Journal of The European Academy of Dermatology and Venereology
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Extracellular deposition of altered autologous protein (amyloid protein) within the dermis is the hallmark of cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement. Amyloidoses may be acquired or hereditary in nature and subclassification differentiates between primary amyloidosis (no obvious predisposing disease) and secondary amyloidosis (specific underlying disease). More than 26 different proteins and peptides have been identified as amyloid precursors and these proteins are used to subclassify this heterogeneous group of diseases. The amyloid proteins show an anti-parallel beta-sheet conformation and form non-branching linear filaments of variable lengths and diameters of approximately 7.5 to 10 nm. However, the exact etiopathogenesis of amyloid formation still remains unclear. Depending on histoanatomical distribution and amount, amyloid may cause progressive and life-threatening organ dysfunction. Clinical presentation, histology, electron microscopy, and biochemical-immunological differentiation represent decisive tools for an accurate diagnosis.
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Thirteen patients with hemodialysis-related shoulder arthropathy were treated either with arthroscopic synovectomy or with open surgery. Arthroscopic synovectomy was performed in eight patients who had shoulder pain, shoulder immobility or both but did not have cystic bone lesions. The therapy was effective for pain relief and improvement of shoulder function for six months but in 12 months the shoulder pain reappeared in most of the patients. Open surgery was done in 5 patients who, in addition to shoulder pain and immobility, had humeral head bone cysts. Resection of the deposited mass on the biceps tendon sheath, of hypertrophied synovium and bursa as well as curettage of cysts and calcium hydroxyapatite ceramic implantation were performed. The therapy was effective for pain relief throughout the follow-up period (12 months). No adverse effects were noted for either procedure. Resected specimens of the synovia contained amyloid as indicated by a positive Congo-red stain by light microscopy and the presence of amyloid fibrils by electron microscopy. Deposition of amyloid in the biceps tendon sheath, synovium and bursa and invasion of the humeral head by amyloid were observed upon open surgery. The results suggest that the resection of deposited material induces the improvement of the shoulder arthropathy.
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Deposition of beta 2-microglobulin amyloid in the joints of dialysis patients is common and begins early in the course of treatment, but its pathogenic significance in the production of dialysis arthropathy is uncertain. The joints (hip, knee, shoulder, elbow, wrist, cervical and lumbar spine, sacroiliac joint) and systemic tissues of 19 autopsied patients who had undergone haemodialysis for between 6 and 231 months were examined histopathologically for the presence of beta 2-microglobulin amyloid; it was present in all joints examined, including those unassociated with radiological changes and those of patients who had been on haemodialysis alone for only 24 months. Osteoarticular beta 2-microglobulin amyloid deposits were also found in patients who had been treated mainly by continuous ambulatory peritoneal dialysis. Systemic amyloid deposition was only seen in patients who had been haemodialysed for more than 13 years and consisted of sparse tiny deposits in blood vessel walls.
Carpal tunnel syndrome (CTS) has been associated with amyloid deposits and is now regarded as a major complication in chronic hemodialysis patients. While this new syndrome has been receiving increasing attention, its etiology has not been clarified. We have isolated amyloid fibrils from amyloid laden tissues inside the carpal tunnel in four different hemodialysis patients with CTS. After solubilization in guanidine HCl, a significant amount of the protein was located in a homogeneous, low molecular weight fraction. Each protein was found to be identical to beta 2-microglobulin with regard to its molecular weight of 11,000 on SDS-PAGE, amino acid composition and N-terminal amino acids: Ile-Gln-Arg-Thr-Pro-Lys-Ile-Gln-Val-Tyr-Ser-Arg-His-Pro-Ala-Glu. In direct immunofluorescent study, anti-beta 2-microglobulin did react positively with amyloid deposits. These results demonstrate that the amyloid associated with chronic hemodialysis contains as major component a new form of amyloid fibril protein that is homologous to beta 2-microglobulin. It is postulated that beta 2-microglobulin cannot be removed from the blood by conventional hemodialysis, and accumulates in tissues causing the formation of amyloid fibrils, which, having a relatively high affinity to the carpal tunnel area, thus causes CTS.
While beta 2-microglobulin amyloidosis occurring in patients undergoing long-term dialysis is frequently associated with joint involvement, skin lesions have rarely been encountered. We report a 57-year-old man with extensive subcutaneous amyloid deposition forming large nodules on the buttocks; the patient had been on maintenance dialysis for 28 years. Although this condition is rare, a review of the literature indicates that the majority of such lesions occur around the buttock region.
Nine patients with severely destructive spondyloarthropathy and marked neurologic deficits associated with dialysis-related amyloidosis underwent posterior decompression and fusion by means of instrumentation at our institute. All patients showed segmental kyphosis, six patients vertebral ankylosis, and eight patients spondylolisthesis. Spondylolisthesis at two levels was noted in three patients. Of the 11 levels of spondylolisthesis in all, 9 were proximally adjacent and 2 were distally adjacent to vertebral ankylosis. All patients underwent posterior decompression and multisegment fusion with autogenous iliac bone. From three to five spinal segments were fixed. Seven patients underwent posterior fusion by means of a pedicle or lateral mass screw between levels C3 and C7, one patient between C3 and C6, and one between C3 and T1. The clinical rate of improvement at the final follow-up was 74.3%. Though complete stability could not be achieved in three patients, the results were rated as good. No postoperative neurologic deterioration has been observed in this series, nor did any patients die immediately after surgery or during the postoperative follow-up period. As anterior long-span surgery might be too invasive for hemodialysis patients, we think that posterior decompression and fusion may well be a reasonable and effective strategy for severe hemodialysis-associated cervical spondyloarthropathy with neurologic deficits. To achieve complete stability, 360 degrees fusion with both anterior and posterior fixation with instrumentation may be required for these patients.
Dialysis-related amyloidosis (DRA) is a frequent and serious complication in patients on long-term dialysis. The amyloid has a marked affinity for joint tissues, and carpal tunnel syndrome, polyarthralgia, destructive spondyloarthropathy, and bone cysts are the major clinical manifestations of DRA. beta(2)-Microglobulin (beta(2)-m) was identified as the major protein constituent of the amyloid fibrils. Risk factors for the development of DRA include age, duration of dialysis treatment, use of low-flux dialysis membrane, use of low purity dialysate, monocyte chemoattractant protein-1 GG genotype, and apolipoprotein E4 allele, although the retention of beta(2)-m in the plasma appears to be prerequisite. Clinical therapeutic strategies for DRA include dialysis, medical or surgical therapy, and renal transplantation. Preventive measures have attempted to remove beta(2)-m from the serum by using high-flux membranes and a beta(2)-m adsorption column in hemodialysis. Renal transplantation is a radical approach to treating the arthralgias attributed to the amyloid deposits while the regression of dialysi-related amyloid deposits is not identified after successful renal transplantation in many studies. It is necessary to elucidate the pathogenesis of DRA and to establish more effective prevention and therapy in the future.