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Most staghorn calculi are infection stones composed of struvite and/or carbonate apatite. Sometimes, cystine, uric acid, whewellite and brushite stones also assume a staghorn configuration when located in the kidney. It is very important in stone crushing to know the composition and architecture of the stones. Struvite stones show a concentric laminal structure and are fragile because of wide interstices of crystals and rich organic matrix. These stones usually contain many bacterial colonies in the interstices of crystals and bacteria break out of the stones when they are crushed. Therefore, perioperative administration of antibiotics is necessary for prevention of bacteremia and sepsis. Whewellite stones and uric acid stones have a smooth surface and reveal compact radial and laminal structure especially in the peripheral layer. They are very hard and are refractory to crushing, and the fragments are large. Cystine stones show a compact radial monomineral texture and are very hard. The fragments made by crushing are large. Therefore, combination therapy of stone crushing and irrigation of alkali solution may be useful for treatment of cystine stones as well as uric acid stones. Calcium phosphate stones, hydroxyapatite or brushite stones, are rare and are formed in hyperparathyroidism, Cushing syndrome and renal tubular acidosis. Hydroxyapatite stones are rich in matrix and fragile. Brushite stones reveal radiate structure and are hard. There is no general method of treatment for staghorn calculi but we should select the most reasonable method including open surgery for each case taking into consideration the stone composition, predisposing factors and possibility of stone residue and recurrence.
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PMID:[Treatment of staghorn calculi on the basis of composition and structure]. 826 80

Calciphylaxis, a syndrome of disseminated calcification found in chronic renal failure patients with secondary hyperparathyroidism, results in soft tissue calcification and vascular medial calcinosis leading to subsequent ischemic tissue necrosis. It is a rarely occurring condition in which patients present with painful, violaceous, mottled lesions of the extremities and/or trunk that progress to skin and subcutaneous tissue necrosis, non-healing ulcers, and gangrene. We reviewed the clinical course of seven patients (aged 24-69) with calciphylaxis treated at our institution over a 4-year period (October 1988-June 1992). All seven patients underwent parathyroidectomy, with a mean time of 8 weeks (range 3-20 weeks) between the onset of calciphylactic symptoms and parathyroidectomy. Four patients died, three secondary to wound-related sepsis. Of the three survivors, two healed soft tissue lesions primarily. The other required extremity amputation and wound excision before healing. Neither anatomical location of the soft tissue lesions nor post-parathyroidectomy serum calcium and phosphorus levels had any bearing on wound healing or mortality. Lesion severity at the time of parathyroidectomy appeared to best correlate with clinical course. Although treatment with phosphate-binding antacids, total or subtotal parathyroidectomy, and avoidance of challengers such as Vitamin D or local tissue trauma remain the mainstays of therapy, the uniform cure for calciphylaxis remains elusive. Prognosis for patients with calciphylaxis is dismal, even following late surgical intervention. Earlier recognition of the signs and symptoms of calciphylaxis should lead to timely parathyroidectomy in the hopes of ameliorating the symptoms and preventing or retarding its progressive sequelae.
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PMID:Calciphylaxis: early recognition and management. 830 50

After a near total small bowel resection for an acute thrombosis of the mesenterial artery, a 61 year-old man was treated with total parenteral nutrition at home for five years. The treatment was complicated by episodes of sepsis, anaemia and uremia. After four years he developed pain in long bones and the back and grave hypercalcuria. Roentgenogram showed demineralisation. There was no hyperparathyroidism and serum phosphate and serum calcium were normal. His chronic metabolic acidosis was treated continuously with enteral acetate. He received basal amounts of vitamin D and amino acids. By administering calcitonin we were able to cure his progressive bone pains and normalize his calcium urinary output. No side effects were observed. Therefore, calcitonin may contribute to the treatment of bone disease associated with total parenteral nutrition.
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PMID:[Calcitonin treatment of metabolic bone disease induced by parenteral nutrition]. 832 48

The articular complications observed in dialysed chronic renal failure failures, whose incidence increases with the duration of dialysis, are closely correlated with the development of beta 2-microglobulin amyloidosis, responsible for nerve tunnel syndromes, arthralgia and chronic joint swelling with frequently multiple subchondral cysts on x-rays. Microcrystalline pathology is dominated by apatite deposits, which may also be involved in the pathogenesis of destructive arthropathy. Articular complications with destruction of the large joints or involvement of the first carpometacarpal joint interfere with the functional prognosis. Sepsis must be excluded in cases of destructive cervical spondyloarthropathies. The pathogenesis of destructive arthropathies is probably multifactorial, consisting of apatite and amyloid deposits, secondary hyperparathyroidism and aluminium poisoning.
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PMID:[Rheumatological complications of dialysis]. 833 5

Chronic (CRF) and acute renal failure (ARF) are accompanied by cardiac dysfunction, particularly if ARF is complicated by sepsis. Intermyocardiocytic fibrosis is described in CRF, but there is also evidence for functional cardiomyopathy. Acetate ion (present in the dialysate) and secondary hyperparathyroidism do not appear to be clinically relevant myocardial depressant factors in uremia. The role of carnitine deficiency is not clarified, because most of the data are evaluated in poorly controlled study trials. Multiple effects of serum fractions and ultrafiltrates obtained from CRF and ARF patients during dialysis suggest the existence of myocardial depressant factor(s). Beneficial effects of continuous hemofiltration in multiorgan failure give evidence for the pathogenetic role of this substance(s). One group of experiments suggests a molecular weight between 500 and 5,000 d; other experiments suggest activity at > 10,000 d. It is currently believed that myocardial depressant substance is a water-soluble molecule weighing 10,000-30,000 d. The data confirm the existence of "specific cardiomyopathy" caused by a functional defect related to filterable toxins. There are different myocardial depressant factors in CRF, ARF, and sepsis.
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PMID:Cardiac depressant factors in renal disease. 838 5

Patients with end-stage renal disease commonly develop secondary hyperparathyroidism. Calcitriol may be administered to such patients to decrease the synthesis and secretion of parathyroid hormone (PTH) and to help maintain calcium and phosphorus homeostasis. However, the doses of calcitriol required to suppress serum PTH concentrations can lead to hypercalcemia or hyperphosphatemia in many patients undergoing hemodialysis. Paricalcitol is a new vitamin D analogue that is safe and effective in suppressing elevated concentrations of PTH in patients with established hyperparathyroidism who are maintained on chronic hemodialysis. As with vitamin D, the biologic action of paricalcitol is mediated through activation of the vitamin D receptor (VDR). The VDR functions as a ligand-induced transcription factor regulating the rate of expression of genes that are involved in controlling not only calcium homeostasis and bone remodeling but also hormone secretion, inhibition of cell growth, and induction of cell differentiation. In vitro studies have shown that paricalcitol inhibits PTH secretion from bovine parathyroid cells in a dose-dependent manner. Studies in renally insufficient rats demonstrated that paricalcitol caused approximately 10 times less elevation of serum calcium concentrations than calcitriol. In clinical studies, paricalcitol effectively decreased PTH by about 60% over a 12-week period. Mean serum concentrations of calcium were significantly increased but remained within the normal range. There were occasional (5/414 determinations) transient elevations in serum calcium above the upper limit of normal in some (5/401) patients. Serum phosphorus values did not change significantly compared with baseline, although they tended to be slightly higher in the paricalcitol-treated group than in the group receiving placebo. Elevations of the calcium-times-phosphorus product were relatively few but occurred more often in the paricalcitol than in the placebo group. The terminal half-life of paricalcitol was 5 to 7 hours in healthy subjects; in patients undergoing hemodialysis, it was 14 hours. Adverse events associated with paricalcitol use included, among others, chills, feeling unwell, fever, sepsis, palpitations, dry mouth, gastrointestinal bleeding, nausea, vomiting, edema, light-headedness, and pneumonia. Paricalcitol should be considered as an alternative to calcitriol in the treatment of patients who are undergoing maintenance hemodialysis for end-stage renal disease, as it has a decreased potential to induce hypercalcemia and hyperphosphatemia. Additional studies are required to determine the long-term effects of therapy.
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PMID:Paricalcitol, a new agent for the management of secondary hyperparathyroidism in patients undergoing chronic renal dialysis. 1032 13

Calciphylaxis (uraemic gangrene syndrome) is a rare complication of chronic renal failure and secondary hyperparathyroidism. Patients present with painful purple skin lesions which undergo necrosis and ulceration. The histology is specific. There is medial calcification with intimal hyperplasia and thrombosis of the lumen of small sized arteries in the underlying subcutaneous tissue. Death frequently arises from overwhelming sepsis. Early recognition of this condition and prompt parathyroidectomy can lead to rapid relief of symptoms and ulcer healing and may be life saving.
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PMID:Calciphylaxis: a rare limb and life threatening cause of ischaemic skin necrosis and ulceration. 1073 37

We report a patient with severe bilateral leg ulceration that was resistant to treatment. A biopsy confirmed the cause as calciphylaxis. Calciphylaxis refers to a syndrome of calcium deposition in the small and intermediate dermal vasculature which can lead to epidermal ischaemia, ulceration and necrosis. Most cases occur in those with chronic renal failure and secondary hyperparathyroidism. We describe the rare presentation of calciphylaxis in a patient with normal renal function and primary hyperparathyroidism who had many classical features. Unfortunately she developed gangrene, sepsis and died.
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PMID:Calciphylaxis in the absence of renal failure. 1101 92

A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.
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PMID:Fulminant and relentless cutaneous necrosis with excruciating pain caused by calciphylaxis developing in a patient undergoing peritoneal dialysis. 1128 Apr 61

Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification. It is seen almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. We experienced a case of 67-year-old man with calciphylaxis that manifested with characteristic skin lesions, pathologic findings, and laboratory changes. His skin lesions began as painful erythematous patches and subsequently progressed to necrotic ulcers with eschars on the distal aspect of the extremities. Pathologically, calcification was found in small and medium-sized blood vessels in the deep dermis and subcutaneous tissue. His serum calcium was 9.5 mg/dL, phosphorus was 7.8 mg/dL, and nPTH was 99.9 pg/mL. The patient had been treated with surgical debridement and other supportive treatment. However, he eventually underwent an amputation below the right knee and died from sepsis.
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PMID:Calciphylaxis in a patient with end-stage renal disease. 1143 66


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