Silicone-induced splenomegaly: treatment of pancytopenia by splenectomy in a patient on hemodialysis.
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Biomedical subjects
Publications and source records attributed to J Bommer.
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Accumulation of a foreign material in grotesque quantities was observed in the macrophages of lung, liver and spleen of a patient on maintenance haemodialysis. The material appeared in macrophages which were found either in groups or singly, without causing epitheloid cell reaction, necrosis or fibrosis. The material was non-isotropic, non-crystalline and did not stain with routine staining procedures. Transmission electron microscopy showed its presence within lysosomal membranes. The nature of the material and the mechanism of its incorporation into the patient remain unclear, but it is conceivable that incorporation is a consequence of longterm interaction of blood and foreign material during haemodialysis. The clinical consequences of such incorporation have to established.
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In seven long-term dialysis patients, autopsy material showed varying amount of refractile foreign material in macrophages and giant cells of lung, liver, spleen, bone marrow, skin, thoracic and abdominal lymph nodes, but not in brain, heart, kidney or endocrine organs. Electron microscopy showed its presence within lysosomal membranes of macrophages. Such material was also demonstrable in liver biopsies of patients dialysed for only several months. Possible clinical consequences were hepatosplenomegaly, elevation of transaminases, hypersplenism with pancytopenia and possibly bile duct carcinoma. SEM with X-ray fluorescence showed particles within spleen and liver cells with a characteristic Si K alpha 2.09 kev peak. Identical material was found as fillings on silicone tubing exposed to roller pumps.
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In 113 hemodialysed patients, 167 hospitalized patients, and 143 outpatients the frequency of HAV and HBV markers were studied by testing HBsAg, anti-HBs, anti-HBc, HBeAg, anti-HBe, and anti-HAV. The hemodialysis patients in a dialysis-center had significantly more often HBV markers (85.7%) than those maintained on home-dialysis (46.5%). 29.9% of the hospitalized patients and 32.1% of the outpatients had HBV markers. By the anti-HBc test up to 41% of additional HBV infections could be detected.--The prevalence of anti-HAV was very high in all groups. Significant differences between the hemodialysis patients and the control groups existed only in the age groups up to 39 years.--The frequencies of HAV and HBV markers were related to age, duration of dialysis treatment, transfusional frequency, and transaminases. The HBV appeared as the clinically important hepatitis agent in dialysis.
Repeated episodes of acute renal flank pain were observed in a uremic patient on maintenance hemodialysis. Computer tomography during the acute attack repeatedly showed unilateral hydronephrosis which always subsided after passage of stones. X-ray failed to visualize the non-radioopaque stones and sonography was non-contributory. The stones consisted of organic matrix and contained protein material. The present observation documents that computer tomography is valuable in establishing the diagnosis of acute ureteral obstruction resulting from non-radioopaque renal stones in dialysed patients with contracted kidneys.
The development of renal cysts appears to be a common feature of terminal renal failure in patients with diffuse renal parenchymal disease. In the present investigation, the kidneys of 13 patients with terminal renal failure but not receiving dialysis, of 14 patients on maintenance hemodialysis and of 4 patients after renal transplantation (patients' own kidneys) were studied by computed tomography. Cystic lesions in the contracted renal parenchyma could be demonstrated by computed tomography in 7/13 non-dialyzed patients with terminal renal failure, in 11/14 patients on maintenance hemodialysis as well as in 3/4 transplanted patients (patients' own kidneys). Both solitary cysts (10/21 patients) and multiple cysts (11/21 patients) were observed. The size varied from 0.5 cm (barely detectable) to 3 cm in diameter. Such cysts could also be demonstrated at autopsy. Possible clinical complications include spontaneous retroperitoneal hemorrhage, macrohematuria, matrix stone formation and formation of benign or malignant papilloma. The present study shows that computed tomography allows the detection of acquired renal cysts in uremic patients in vivo. The cysts appear prior to dialysis, seem to increase in frequency during dialysis and do not disappear after transplantation. The lesions can be distinguished from multicystic or polycystic disease.
The vitamin metabolite 25(OH)D is transformed into the active secosterole 1.25(OH)2D3 in the proximal tubular epithelium of the kidney. This transformation is disturbed in patients with renal insufficiency. However, this review shows that presumably not all vitamin D dependent disturbances in patients with renal insufficiency are explicable merely as the consequence of reduced renal synthesis of 1.25(OH)2D3 secondary to nephronal loss. In incipient renal failure, vitamin D dependent functions (calcemic action of PTH, intestinal absorption of Ca) are disturbed. Yet, circulating 1.25(OH)2D3 levels are slightly elevated. This finding is compatible with an inadequate response of the renal 1-alpha-hydroxylase system to activating stimuli (hyperparathyroidism, hypocalcemia, fasting hypophosphatemia) and/or end-organ resistance to the action of 1.25(OH)2D3. Osteomalacia in renal insufficiency cannot entirely be explained as the consequence of a reduction of the serum-concentration of any of the known vitamin D metabolites [25(OH)D3; 1.25(OH)2D3; 24.25(OH)2D3]. The relatively poor response of osteomalacia of uremic patients to the administration of 1.25(OH)2D3 leads to the question of whether other vitamin D metabolites or non-vitamin D related factors are important in its genesis. Critical information is lacking with respect to 1.25(OH)2D3 receptors, post receptor events and interaction between vitamin D metabolites and PTH in bone cells of such patients. A specific action of 1.25(OH)2D3 on longitudinal growth of uremic children has been described. However, several clinical and experimental studies failed to provide evidence of normalization of growth by 1.25(OH)2D3 and failed to show differences in this respect between vitamin D and 1.25(OH)2D3. Currently, it remains undecided whether vitamin D metabolites affect PTH secretion, and if so which vitamin D metabolite is involved. Clarification of this problem is of paramount importance for the therapeutic suppression of the parathyroids of uremic patients. Vitamin D metabolites play an important role in some organ functions unrelated to homeostasis of Ca-Pi-metabolism (e.g. muscle, testis, pancreas, etc). The loss of such function is of potential importance in the genesis of the uremic syndrome and its imcomplete reversal by hemodialysis.
The effect of bromocriptine on sexual activity was studied in male haemodialysis patients in a single-blind placebo controlled trial with random cross-over. At a dose of 2.5 mg bromocriptine twice day, plasma-prolactin concentrations were consistently reduced, while sexual function as assessed by a questionnaire was markedly improved. At the doses used, side-effects, particularly hypotension, were common, but bromocriptine helped to restore sexual function in dialysed patients.
In seven patients on maintenance hemodialysis, de novo recurrent renal stone formation was observed. In all patients, the underlying disease was glomerulonephritis, with or without the nephrotic syndrome. All patients had considerable persistent proteinuria. The stones consisted predominantly of protein, as revealed by amino acid analysis, and had a negligible carbohydrate and lipid content. Only in some specimens, X-ray diffraction and scanning electron microscopy revealed the presence of small amounts of whewellit (calcium oxalate monohydrate) and/or uric acid. In semithin sections, the stones had a laminated texture and exhibited structural anisotropy under polarized light. With transmission electron microscopy, they were found to consist of peculair microfibrils. The proteinaceous material differed from fibrin or Tamm-Horsfall-protein, as indicated by ultrastructure, carbohydrate analysis, and amino acid analysis. Symptomatic de novo matrix stone formation constitutes another complication of dialyzed patients which has not been reported so far.
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X-ray films of the hand skeleton (mammography technique), serum chemistry, and quantitative bone histology (micromorphometry of undecalcified sections, iliac crest spongiosa) were compared in 25 patients on maintenance hemodialysis. The X-ray findings correlated better with serum PTH levels than with bone histology. Of all radiological signs of renal osteodystrophy, pronounced subperiosteal resorption (radial aspect, second finger, middle phalanx) and periosteal new bone formation (middle phalanx) correlated best with histological indicators of osteitis fibrosa. These signs were never seen in control patients. Acroosteolysis (endphalanx) and intracortical or endosteal resorption (middle phalanx) were less specific (i.e., seen even in the absence of metabolic bone disease) and correlated less with bone histology. Osteosclerosis in iliac cancellous bone was paralleled by abnormal texture of spongy bone in the proximal metaphysis of the middle phalanx (second finger).