Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 46-year-old male patient underwent long-term hemodialysis treatment had suffered from calciphylaxis (defined by Selye), such symptoms as advanced systemic vascular calcification, rapid progression of gangrene on both fingers and toes, disturbance of consciousness, and sclerosis and obstruction of the superficial vein after venipuncture during 11.5 years of dialysis. Furthermore, he had a long history (30 years) of heavy smoking. He died as a result of sepsis due to pneumonia after 12.5 years of dialysis. He had received dialysis treatment using a small amount of dialysate (50 liters on a recirculating system) for 8.5 years and had been dialysed 2 and 2 or 3 times a week for 10 years. As a result of this insufficient dialysis treatment, his characteristic laboratory data showed hypocalcemia, hyperphosphatemia, elevated calcium-phosphorus product, advanced metabolic acidosis, hyperalkaliphosphatemia and elevated serum parathyroid hormone. Autopsy revealed the following: 1) enlargement parathyroid gland enlarged in two (4.0 g and 2.0 g, respectively) showing adenomatous hyperplasia presenting cord-like arrangement of chief cells and water-clear cells, 2) systemic medial calcification in radial, ulnar, renal, mesenteric and brain arteries, and 3) Berline-blue positive iron deposit in calcified arteries in mesenteric and parathyroid tissue. From these results, we concluded that factors (challengers) related to the appearance of calciphylaxis might be as follows: 1) advanced secondary hyperparathyroidism, 2) long-term uremic state, 3) administration of VD2 and VD3, 4) iron salt injection, and 5) a long history of heavy smoking. We speculated that these challengers might act synergistically to cause calciphylaxis.
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PMID:[A long-term hemodialysis patient complicated with systemic calciphylaxis]. 823 Aug 23

Calciphylaxis is a rare and life-threatening complication that is estimated to occur in 1% of patients with ESRD each year. Typically, extensive microvascular calcification and occlusion/thrombosis leads to violaceous skin lesions, which progress to nonhealing ulcers and sepsis. Secondary infection of skin lesions is common, often leading to sepsis and death. The lower extremities are predominantly involved (roughly 90% of patients). Patients with skin involvement over the trunk or proximal extremities have a poorer prognosis. Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis remains unclear. Recently, functional protein C deficiency has been hypothesized to cause a hypercoagulable state that could induce thrombosis in small vessels, with resulting skin ischemia, necrosis, and gangrene. The lack of understanding of the pathophysiology of the disease results in treatments that are equally unsatisfactory. Patients who undergo parathyroidectomy have a tendency to improve, but the prognosis for the disease is poor and mortality remains high.
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PMID:Calciphylaxis in chronic renal failure. 882 11

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

We report an unusual case of congenital leukemia with leukemia cutis (LC) and diffuse calcinosis cutis. A newborn girl presented with widespread dusky red and yellowish cutaneous nodules and papules. Bone marrow morphology was consistent with the diagnosis of acute monocytic leukemia of the FAB M5 type. Skin biopsy specimens confirmed the presence of a leukemic infiltrate and revealed calcium salt deposition in the papillary and reticular dermis. Calcinosis was diffuse in the whole skin but spared other organs. Vascular calcification was not present. Serum calcium levels oscillated between 2.5 and 2.86 mmol/l, and phosphorus, parathyroid hormone and 25-hydroxyvitamin D(3) levels were normal. There were diffuse osteoporosis and spontaneous fractures of small tubular bones. The patient responded to chemotherapy but, following consolidation treatment, developed sepsis and died at 120 days of age. Congenital leukemia is rare and LC is uncommon. Hypercalcemia may be a complication of leukemia, which leads to multiorgan metastatic calcification. Despite the absence of frank hypercalcemia, the presence of bone lesions suggests that the patient's calcinosis cutis was of the metastatic type. However, the cutaneous leukemic infiltrate may also represent a triggering factor for calcium deposition in the skin.
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PMID:Diffuse calcinosis cutis in a patient with congenital leukemia and leukemia cutis. 1077 6

A 69-year-old female having a large left adrenal mass and a nodular lesion in thyroid gland was admitted to the hospital. Endocrinologically she showed high levels of plasma ACTH and cortisol, which were not suppressed by high dose dexamethasone, elevated serum parathyroid hormone and markedly increased daily urinary excretion of catecholamines. She died of sepsis, and postmortem examination was performed. A left adrenal pheochromocytoma, which was immunohistochemically proven to be ACTH producing, a papillary thyroid carcinoma and parathyroid adenomas were found. To our knowledge this is the first report of coexistence of these three endocrine tumors in a single patient.
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PMID:[Multiple endocrine neoplasia]. 1514 27

Hypocalcemia and hypomagnesemia are common in horses with sepsis and endotoxemia. We hypothesize that endotoxemia triggers a systemic inflammatory response that results in hypocalcemia and hypomagnesemia. The goal of this study was to determine the effect of endotoxin (lipopolysaccharide [LPS]) administration to healthy horses on serum parathyroid hormone (PTH), ionized calcium (Ca2+) and total calcium (tCa), ionized magnesium (Mg2+) and total magnesium (tMg), phosphate (Pi), potassium (K+), sodium (Na+), chloride (Cl-), and insulin concentrations, and on the urinary excretion of these electrolytes. Twelve mares were infused with Escherichia coli LPS (30 ng/kg/h i.v.) for 1 hour. Six mares were infused with saline (controls). In LPS-infused horses, heart rate increased significantly from (mean +/- SD) 40.0 +/- 1.3 to 70.0 +/- 9.0 beats/min, respiratory rate from 12.7 +/- 1.0 to 21.1 +/- 3.0 breaths/min, body temperature from 37.4 +/- 0.3 to 38.9 +/- 0.6 degrees C, and tumor necrosis factor-alpha concentrations from 6.6 +/- 3.5 to 507 +/- 260 pg/mL (P < .05). White blood cell count decreased significantly from 7570 +/- 600 to 1960 +/- 560 cells/ microL. Serum concentrations of Ca2+ decreased from 6.5 +/- 0.3 to 6.0 +/- 0.3 mg/dL, of Mg2+ from 0.53 +/- 0.06 to 0.43 +/- 0.04 mM, of tMg from 0.78 +/- 0.05 to 0.62 +/- 0.08 mM, of K+ from 4.3 +/- 0.4 to 3.0 +/- 0.5 mEq/L, and of Pi from 3.4 +/- 0.5 to 1.7 +/- 0.5 mg/dL (all P < .05). PTH increased significantly from 1.3 +/- 0.4 to 6.0 +/- 5.2 pM; however, in some horses (n=2), PTH did not increase despite hypocalcemia. Insulin increased significantly from 9.4 +/- 3.6 to 50.5 +/- 9.6 microIU/mL (n=3). Urinary fractional excretion of Ca2+ decreased significantly from 4.7 +/- 1.4 to 1.7 +/- 1.2%, of Mg2+ from 36.6 +/- 6.5 to 11.7 +/- 7.3%, and of K+ from 37.9 +/- 11.3 to 17.7 +/- 6.2%. Fractional excretion of Pi increased from 0.02 +/- 0.02 to 0.14 +/- 0.07% and of Na+ from 0.26 +/- 0.13% to 1.2 +/- 0.5%. No changes were found in serum tCa, Na+, and Cl- concentrations. In conclusion, endotoxemia in horses resulted in electrolyte abnormalities that included hypocalcemia, hypomagnesemia, hypokalemia, hypophosphatemia, and increased serum PTH and insulin concentrations.
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PMID:Alterations in serum parathyroid hormone and electrolyte concentrations and urinary excretion of electrolytes in horses with induced endotoxemia. 1582 68

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.
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PMID:Calciphylaxis--a topical overview. 1668 74

A 52-year-old woman was admitted with complaints of nonhealing skin ulcers. The laboratory assessment showed a calcium level of 13.2 mg/dL (normal 8.4-10.2 mg/dL), albumin 2 g/dL (normal 3.5-4.8 g/dL), corrected calcium 14.8 mg/dL, phosphorus 1.4 mg/dL (normal 2.4-4.7 mg/dL), creatinine 0.7 mg/dL (normal 0.5-1.2 mg/dL), parathyroid hormone 893.3 pg/mL (normal 11.1-79.5 pg/mL). A technetium-sestamibi scan showed a left inferior parathyroid adenoma. The patient underwent a parathyroidectomy. Pathology showed a parathyroid adenoma. Despite treatment of her skin wounds with broad spectrum antibiotics and local wound care, the patient died of overwhelming sepsis. Calciphylaxis is a rare but serious disorder that leads to skin and soft tissue necrosis and requires prompt diagnosis and management.
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PMID:Calciphylaxis in primary hyperparathyroidism: a case report and brief review. 1920 21

Calciphylaxis is a rare complication that occurs in 1% of patients with end-stage renal disease (ESRD) each year. Extensive microvascular calcification and occlusion/thrombosis lead to violaceous skin lesions, which progress to nonhealing ulcers with secondary infection, often leading to sepsis and death. The lower extremities are predominantly involved (roughly 90% of patients). Although most calciphylaxis patients have abnormalities of the calcium-phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder. We report on a case of histologically proven calciphylaxis in a 54-year-old woman with normal renal function and normal calcium-parathyroid homeostasis. She had a history of alcoholic cardiomyopathy, and was treated with warfarin anticoagulation. She has been successfully treated with antibiotics, i.v. biophosphonates and intensive local wound care. We recorded a complete wound healing in contrast to what is reported in other series.
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PMID:Calciphylaxis in a cardiac patient without renal disease. 1931 4

Calciphylaxis is a rare disease associated with thrombotic cutaneous ischemia and necrosis. Lesions are usually located on the lower extremities, buttocks and the abdomen. Calciphylaxis is recognized by medial calcification, inflammation and subintimal fibrosis of cutaneous arterioles. Calcification, thrombus formation and occlusion occur sequential before tissue necrosis. The disease is usually observed in patients with end-stage renal disease and hyperparathyroidism. In end-stage renal disease, an elevated parathyroid hormone level, hypercalcemia and hyperphosphatemia direct to vascular mineralization. Calciphylaxis affects about 4% of hemodialysis patients. The clinical syndrome is characterized by a high mortality rate. The most important measure is an active multidisciplinary management approach, with intention to wound care and prevention of sepsis.
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PMID:[Calciphylaxis. Pathogenesis and therapy]. 2168 45


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