1/31. Production of recombinant human beta2-microglobulin for scintigraphic diagnosis of amyloidosis in uremia and hemodialysis.Amyloid of beta2-microglobulin (beta2m) origin can be diagnosed using 131I-radiolabelled-beta2m scintigraphy in patients with uremia and hemodialysis treatment. As the tracer beta2m is isolated from another patient, it carries the common risks, including viral infections such as hepatitis b, C and hiv, which are associated with human plasma products. In order to exclude these risks we have produced recombinant human beta2m (rhbeta2m) in escherichia coli. The expression vector pASK40DeltaLbeta2m(His)5 contains a C-terminal (His)5-tag for purification via immobilized metal ion affinity chromatography (IMAC). Size exclusion chromatography on a Superose 12 column represents the second step of purification. The isolated rhbeta2mH5 reacted in an immunochemically identical manner to native human beta2m, and showed a single band of approximately 11.8 kDa in Western blot analysis and revealed a single spot in two-dimensional gel electrophoresis. mass spectrometry analysis revealed a single peak at the expected molecular mass of 12 415.8 Da. Uniformity was further proven by crystallization and N-terminal amino-acid sequence analysis. The rhbeta2mH5 protein was then produced under conditions that allow the intravenous use in humans. Intraveneously applied indium-111-labelled rhbeta2mH5 was monitored in hemodialysed patients with and without known beta2m-amyloidosis. The tracer was localized specifically to particular areas known to contain amyloid. Thus, this rhbeta2mH5 preparation is suitable for detecting amyloid-containing organs of the beta2m-class in vivo and fulfils the requirements of a tracer for common use. Finally, the use of indium-111 instead of iodine-131 has reduced the radioactive load and resulted in higher resolution.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/31. Compression of the ulnar nerve in Guyon's canal by uremic tumoral calcinosis.We describe the case of a 70-year-old woman with chronic renal failure on haemodialysis presenting with an ulnar nerve compression in Guyon's canal secondary to uremic tumoral calcinosis. Excision of calcium deposits and external neurolysis of the ulnar nerve were successfully performed. Simultaneously, the hyperphosphatemia and hypercalcemia were corrected. The pathogenesis of this condition is different from primary tumoral calcinosis. Clinical and radiological features and therapy are discussed. Uremic tumoral calcinosis is an unusual etiology of ulnar nerve compression in Guyon's canal not previously reported in dialysis patients.- - - - - - - - - - ranking = 72.20204561529keywords = nerve (Clic here for more details about this article) |
3/31. Intestinal necrosis following calcium Resonium-sorbitol administration in a premature uraemic infant.sodium polystyrene sulphonate (Resonium A) in sorbitol given as an enema or orally to treat hyperkalaemia has been described to induce intestinal necrosis in uraemic patients. We report a case of a premature infant with acute renal insufficiency who developed focal transmural necrosis and perforation of the small intestine after 10 days of administration of calcium polystyrene sulphonatum (calcium Resonium) in sorbitol by enema and by nasogastric tube. On histological examination of the resected part of the small intestine, numerous strongly basophilic angular crystals of resonium were found in the lumen, in the necrotic wall, as well as in the organized exudate on the peritoneal surface. The crystals showed a strong direct Schiff positivity without preoxidation. They were also stained using PAS, Giemsa, Ziehl-Neelsen, Schmorl, and Gram method. In contrast, the crystals were congo red and alcian blue (pH 2.5) negative and non-birefringent. The direct Schiff positivity without preoxidation is virtually pathognomonic for resin crystals in routinely processed tissues. The same crystals were observed in the lumen of the small intestine and in peritoneal adhesions at autopsy. Thus our case provides additional evidence that Resonium A/calcium Resonium in sorbitol administered as an enema or orally can lead to intestinal necrosis in uraemic patients.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/31. Reappraisal of long-term renal replacement therapy. Renal transplantation: medical versus statistical approach.The validity of the numerous statistical methods used for the assessment of the results achieved with renal transplantation performed for terminally uremic patients may be challenged by a medicalized approach of the patient's post transplantation course and outcome. This assumption has been applied to a population of 700 patients who received a grafted kidney in our unit between November 1972 and December 1990. Among 77 patients who had been followed up for 10-15 years after grafting, 75% had not suffered any serious related complication during the first decade after transplantation. In contrast, among the whole transplant population, transplantation entailed either a rapidly fatal issue due to an unpredictable and irreversible complication in 45 patients (6.4%), or a state of chronic illness with severely disabling complications in 150 others (21.5%). At present, the main cause of concern remains, however, the persistent long-term graft loss due to chronic rejection. Nevertheless, the advances and results achieved in the field of organ transplantation in recent years justifies the policy which privileges renal transplantation as the first-choice method of renal replacement therapy for every terminally uremic patient aged less than 60-65 years in whom careful workup does not reveal any clinical/psychological contraindication.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/31. Hemorrhagic cardiac tamponade in critically ill patients with acute renal failure.PURPOSE: The purpose of this study was to report the development, management, and follow up of tamponading uremic pericardial effusion in critically ill patients with acute renal failure. SETTING: The setting for this study was an adult, 24-bed tertiary multidisciplinary intensive care unit (ICU) of a university hospital. patients: The subjects were 5 critically ill patients with multiple organ failure including acute renal failure (ARF) that was slow to resolve. RESULTS: Renal involvement was attributed to renal hypoperfusion, sepsis and myoglobinuria. Continuous veno-venous hemofiltration (CVVH) was instituted early during hospitalization in 4 cases and lasted for 35 to 48 days; renal replacement therapy was not used in 1 case. Tamponade developed late in the course of ARF, after CVVH was discontinued in the 4 cases and was effectively managed with percutaneous pericardiocentesis under echocardiography and continuous catheter drainage of the pericardial sac for 48 to 72 hours. Hemorrhagic fluid (Hb 2.2-5.9 g/dL) with lymphocyte predominance was detected. Transient constrictive-like pericarditis findings were present in all patients after the procedure. All patients were discharged from the hospital in a good condition with normal serum and creatinine levels; 1-year follow up showed a normal echocardiogram. CONCLUSION: awareness for the possibility of hemorrhagic pericarditis and cardiac tamponade is needed in ICU patients with ARF slow to resolve. Transient constrictive-like pericarditis may present after pericardiocentesis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/31. Diffuse calcinosis and intradermal tophi in a uremic pateint: effect of low-calcium hemodialysis and mechanism of hypercalcemia.Soft tissue calcification is a frequent complication in end-stage renal disease (ESRD) patients with a high serum calcium-phosphate product, but systemic involvement of both the visceral organs and skin is rarely seen. We report on a newly diagnosed ESRD patient with gouty nephropathy who had initial presentations of extensive intradermal tophi, diffuse calcinosis, and hypercalcemia. He received maintenance hemodialysis (HD) with low-calcium dialysate (1.25 mEq/l) for 11 months. Although the above complications diminished, serum calcium remained elevated. Thereafter, unexpected cervical lymphadenitis from a mycobacterium tuberculosis (TB) infection with high extra-renal production of calcitriol was found. serum calcium levels normalized only after anti-TB treatment for 2 months. We thought that this patient might have had occult TB infection before the start of HD, which resulted in calcitriol production and hypercalcemia. In addition, concomitant hyperphosphatemia in chronic renal failure contributed to severe diffuse calcinosis. After the initiation of HD therapy, both the elevated serum calcitriol levels and accelerated resolution and mobilization of diffuse calcinosis from low-calcium HD contributed to persistent hypercalcemia.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/31. Ischemic optic neuropathy in dialyzed patients: a previously unrecognized manifestation of calcific uremic arteriolopathy.Two patients with end-stage renal disease and on long-term hemodialysis presented with hypotension and an acute unilateral loss of vision. A diagnosis of anterior ischemic optic neuropathy (AION) was made quickly, but despite high-dose steroid therapy, significant vision was not recovered. Temporal artery biopsy results showed extensive medial calcification. The possibility that hypotension, when coupled with calcific uremic arteriolopathy in arteries supplying the optic nerve head, will lead to AION in dialyzed patients is discussed. A short review of AION in the dialysis population is given.- - - - - - - - - - ranking = 10.314577945041keywords = nerve (Clic here for more details about this article) |
8/31. Severe proximal myopathy in advanced renal failure. diagnosis and management.Myopathies encountered in uremic patients may have different pathogenetic mechanisms and treatment. Secondary hyperparathyroidism may cause uremic myopathy responding to specific treatment. This study aimed at presenting a case illustrative of the clinical features, diagnosis and management of uremic parathyroid myopathy. A 66-year old man with renal failure from membranous nephropathy developed sensory signs of uremic neuropathy and progressive painless weakness of the pelvic girdle muscles bilaterally. Motor nerve conduction velocity was normal, electromyogram was consistent with a myopathic pattern, while muscle biopsy showed a pattern of atrophy more consistent with a neuropathic pattern. Serological tests for collagen vascular diseases and hyperthyroidism were negative, while serum muscle enzymes were not elevated and serum phosphate levels were not low. serum parathyroid hormone level was grossly elevated, while serum calcium was mildly elevated in a small fraction of the measurements, serum alkaline phosphatase showed a progressive rise and skeletal bone survey did not disclose osteopenia or signs of parathyroid bone disease. A course of calcitriol failed to improve the myopathy, which responded promptly and dramatically to parathyroidectomy. Uremic parathyroid myopathy, which has a characteristic clinical picture, must be differentiated from other neuropathic or myopathic conditions that require specific treatments. Progressive parathyroid myopathy is, by itself, an indication for parathyroidectomy, which is curative in this case.- - - - - - - - - - ranking = 10.314577945041keywords = nerve (Clic here for more details about this article) |
9/31. Acute hyperkalemic paralysis in a uremic patient.hyperkalemia is a less-recognized life-threatening cause of paralysis. We describe a 38-year-old uremic man on regular hemodialysis (HD) without interruption who suffered from muscle weakness progressing to ascending symmetric paralysis, and inability to masticate. physical examination revealed flaccid paralysis with areflexia of the four limbs. Computed tomography of the brain and cervical spine did not demonstrate any organic lesions. Laboratory investigations revealed serum K 8.1 mmol/L, urea nitrogen 32 mmol/L, creatinine 919 micromol/L. Of note, electrocardiography (ECG) did not show typical features of hyperkalemia. Emergent HD with low K dialysate (1.0 mmol/L) rapidly normalized serum K (4.7 mmol/L) and reversed all neuromuscular symptoms within one hour. Upon reviewing his food and medication history, he admitted drinking 750 ml of raw coconut juice per day (K concentration 44.3 mmol/L) to quench his thirst for three consecutive days. hyperkalemia should be born in mind in the differential diagnosis of acute paralysis despite no ECG changes. Hidden sources of K intake, such as coconut juice, should not be overlooked, especially in patients with impaired renal function.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/31. Fulminant pulmonary calciphylaxis and metastatic calcification causing acute respiratory failure in a uremic patient.calciphylaxis is a rare and life-threatening disorder characterized by small-vessel mural calcification with intimal proliferation, fibrosis, and thrombosis, resulting in tissue ischemic necrosis. Although it has been viewed as a systemic disease involving mainly the dermis, subcutaneous fat, or muscle, calciphylaxis of other organs rarely is reported. We describe the case of a 25-year-old uremic woman who rapidly developed massive pulmonary calcification that led to acute respiratory failure after the initiation of hemodialysis therapy. Chest radiography and high-resolution computed tomography showed typical pulmonary calcification. Pulmonary calciphylaxis and metastatic calcification were confirmed further by lung tissue biopsy. No skin or muscle calciphylaxis was discovered. Despite multiple factors precipitating pulmonary calciphylaxis in this patient, we speculate that hemodialysis was the main culprit in accelerating the development of fulminant pulmonary calciphylaxis and metastatic calcification. Alteration in the local environment from an acid to an alkaline condition and a relatively high dialysate calcium level in the presence of systemic hyperphosphatemia are believed to have facilitated the deposition of calcification. This case highlights the importance of "visceral calciphylaxis" and early identification of its causes.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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