Home About us Contact
|
Home About us Contact | ||
|
|
||
Deposition Disease (deposition + disease)
Selected AbstractsPhosphoglyceride (PG) crystal deposition disease: A novel acquired disease in which PG crystals are deposited in injured soft tissue and bone with phospholipid metabolism disturbancePATHOLOGY INTERNATIONAL, Issue 12 2004Katsutoshi Miura The clinical history and crystal characteristics of three published cases and three new cases of phosphoglyceride (PG) crystal deposition disease of soft tissues and bones were compared. All patients (age range, 51,64 years) were generally healthy without a genetic background of congenital immunodeficiency or lipidosis. Foreign body granulomas grew slowly, predominantly at postoperative or repeat injection lesions. In two cases, crystals were deposited in multiple locations, and in one case, lipophage accumulations were found in the bone marrow. The crystals characteristically dissolved in acetic acid with oxygen gas formation, easily dissolved in alkalis and showed positive staining for PG by the gold hydroxamic acid method. All crystals examined by infrared microscopy, mass spectrometry and X-ray microanalysis showed similar results, supporting the theory that the crystals were PG. Phosphoglyceride deposition disease is a lipid metabolic disorder in which PG crystals are slowly deposited, predominantly in injured soft tissues, forming foreign body granulomas. The diagnosis can be based on histological characteristics. The prognosis is favorable, although some cases showed systemic depositions with repetitions. Lysosomal phosphoglyceride metabolism in macrophages might be affected. [source] Phosphoglyceride crystal deposition diseasePATHOLOGY INTERNATIONAL, Issue 12 2000Katsutoshi Miura An extremely rare phosphoglyceride deposition disease is reported. A healthy 62-year-old Japanese woman suffered from tumors that repeatedly appeared in injured soft tissues for more than 20 years. No immunologic disorders or abnormal laboratory data were found. Histology showed foreign body granulomas consisting of macrophages surrounding yellowish-white crystals. The crystals were weakly positive by von Kossa's method, were dissolved in 30% acetic acid with gas, and were easily dissolved in 0.1 N NaOH or potassium hydroxide, losing their crystal structure. Using a scanning electron microscopy X-ray microanalyzer, phosphorus and calcium peaks were detected. Phosphoglycerides were detected by microscopic infrared spectrophotometry and microsampling mass spectrometry. The gold hydroxamic acid method for detecting phosphoglyceride showed strong positive staining in the crystals. Based on the above analyses, the deposited crystals were regarded as phosphoglyceride, which bound calcium as a counter ion. The crystals tended to be deposited at sites of injury, where macrophages had accumulated. The patient had received many injections of a medicine made from alcohol extract from bovine liver. We suspect that this medicine was related to the cause of the deposition as the deposition repeatedly appeared at the site of the injections. [source] Pulmonary manifestations of light chain deposition diseaseRESPIROLOGY, Issue 5 2009Lisa RHO ABSTRACT Light chain deposition disease (LCDD) is a rare condition characterized by extracellular light chain deposition in tissues. Patients commonly have an underlying plasma cell dyscrasia, and produce excess levels of monoclonal light chains. Renal involvement is the most common clinical manifestation. Rarely, light chains are deposited in the lung. We present the pathologic and radiographic findings of three patients with biopsy-proven pulmonary light chain disease and a review of the literature. [source] Myeloma kidney with isolated tubulointerstitial light chain deposition in a renal allograftCLINICAL TRANSPLANTATION, Issue 6 2009S. Balamuthusamy Abstract:, Myeloma kidney and myeloma-associated renal disorders including light chain deposition disease can occur as recurrent or de novo disease in renal allografts. These kidney disorders usually manifest with worsening allograft function and proteinuria. Identification of the precise cause of kidney disorder often requires kidney biopsy and demonstration of monoclonal light chains in the kidney. Here, we present an unusual case of light chain nephropathy in a living-related kidney transplant recipient involving light chain crystallization in the proximal tubule occurring within less than three months after transplant. The etiology of renal failure prior to transplant in our patient is not clear. To the best of our knowledge, the ultrastructural changes seen in our patient have not been described in literature previously. Our patient was treated with steroids, which resulted in short-term improvement in allograft dysfunction. [source] | ||||||||||