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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital lymphedema is a relatively rare disease caused by congenital abnormality of the lymphatic system. Although bacterial infection frequently causes complications with lymphedema, severe sepsis in congenital lymphedema of the genitalia has not yet been reported. We describe a patient with congenital penoscrotal lymphedema complicated by cellulitis, lymphangitis, and severe sepsis associated with a streptococcal infection. This case represents the importance of obtaining a detailed clinical history and physical findings.
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PMID:Congenital penoscrotal lymphedema complicated by sepsis associated with a streptococcal infection. 1113 88

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

Idiopathic thrombocytopenic purpura (ITP) is a common acquired bleeding disorder in infancy and childhood. Most children rapidly improve, exhibiting a rise in platelet count to hemostatically normal levels within weeks to several months. Traditionally, chronic ITP is defined as persistence of thrombocytopenia (platelet count < 150 x 10(9)/L) for greater than 6 months. The Authors retrospectively evaluated 16 patients with chronic ITP, identified during a 12-year period of time in their Department of Pediatrics. The most important clinical and hematological parameters of patients were analyzed, including age at diagnosis, type and response to the initial treatment, number of multiple treatments, and duration of follow-up. At the last evaluation (december 1999) one patient was lost to the follow-up; one died of overwhelming postsplenectomy sepsis; four still require intermittent or chronic infusions of intravenous gamma-globulin; seven are in stable partial remission (PLT > 50 x 10(9)/L < 150 x 10(9)/L) and do not require any treatment; three are in complete remission (PLT > 150 x 10(9)/L). Finally, the Authors discuss of the natural history and management of this rare disease. Presently there are insufficient trial data to support evidence-based treatment guidelines in childhood chronic ITP and therefore it is reasonable to encourage future multicentre collaboration.
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PMID:[Long-term follow-up of chronic idiopathic thrombocytopenic purpura in children]. 1138 65

Calciphylaxis is a rare disease that is caused by calcium deposition in medium and small sized vessels of the skin causing ischemic necrosis. These lesions often become infected and cause septicemia. We report a unique case of penile and scrotal calciphylaxis that rapidly progressed to Fournier's gangrene. We review the etiology of calciphylaxis and also its management.
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PMID:Calciphylaxis of the penis: a unique cause of Fournier's gangrene. 1171 34

Non-occlusive mesenteric ischaemia is characterized by gastrointestinal ischaemia with normal vessels. In gastroenterology it is recognized as rare disease occasionally causing acute bowel infarction or ischaemic colitis. From intensive care literature this disorder is recognized as an early phenomenon during circulatory stress. This early mucosal ischaemia then leads to increased permeability, bacterial translocation, and further mucosal hypoperfusion. The damage is produced mainly during reperfusion following ischaemia with fresh inflow of oxygen and outflow of waste products into the systemic circulation. The mechanisms underlying non-occlusive mesenteric ischaemia include macrovascular vasoconstriction, hypoperfusion of the tips of the villi and shunting. It is very common in critically ill and perioperative patients, but also occurs in pancreatitis, renal failure and sepsis. Treatment options include aggressive fluid resuscitation and careful choice of vasoactive drugs. Control of reperfusion damage and new endothelin-antagonists are potentially useful new treatment options.
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PMID:Non-occlusive mesenteric ischaemia: a common disorder in gastroenterology and intensive care. 1276 7

Transmission of donor infections to immunosuppressed recipients may produce serious complications. Here, we report two cases of ruptured renal artery pseudoaneurysm within a few months after renal transplantation from a donor with Gorham's syndrome, a rare disease characterized by proliferation of vascular and lymphatic channels associated with extensive bony destruction. The donor had died of respiratory failure, sepsis, and anoxic brain death due to difficult airway control secondary to a maxillofacial deformity.
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PMID:Arterial anastomosis disrupton in two kidney recipients of contaminated grafts from a donor with Gorham's syndrome. 1525 40

Xanthogranulomatous pyelonephritis is a rare disease in childhood. Because the symptoms and signs are chronic and non-specific, preoperative diagnosis is usually difficult. We report an 8-year-old boy who had an abdominal mass and anemia for more than 6 months. Fever and dyspnea occurred 4 days prior to admission. Ultrasonography revealed an enlarged right kidney with multiple parenchymal hypoechogenic areas, absence of normal parenchymal structures, and perinephric thickening with multiple calcifications. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas consistent with hydronephrosis and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. Poor right kidney function was demonstrated by Tc99m-diethylenetriamine penta-acetic acid split renal function examination. We diagnosed xanthogranulomatous pyelonephritis preoperatively based on the clinical and radiological features. The child first had drainage of an extrarenal abscess and antibiotic therapy, followed by definitive nephrectomy. The hospital course was complicated with pleural effusion, peritonitis, pelvic abscess, and sepsis. A two-stage nephrectomy requiring less radical resection and decreasing the surgical complications would have been preferable.
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PMID:Diffuse xanthogranulomatous pyelonephritis in a child with severe complications. 1537 22

Descending necrotizing mediastinitis is a rare disease that is usually caused by a spreading, diffuse inflammatory reaction (phlegmon) to an odontogenic infection or peritonsillar abscess. Reported mortality rates range from 25 to 40%. The use of antibiotics and advances in resuscitation procedures and critical care techniques have not essentially improved survival, and an effective treatment has not been clearly established. We report the findings of our 10-year study of 21 patients affected by phlegmon and/or fasciitis of the neck. The aim of our contribution is to help define the clinical criteria and diagnostic procedures that will improve the early diagnosis of mediastinal sepsis secondary to neck fasciitis and to suggest optimal treatment approaches. Our experience indicates that (1) cervical drainage alone is sufficient for cases of cervical phlegmon or mediastinal involvement that are limited to a single superior mediastinal space and (2) thoracotomy and drainage of mediastinal collections is necessary when mediastinal sepsis is more extensive.
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PMID:Descending necrotizing mediastinitis: ten years' experience. 1562 36

Lemierre syndrome is characterized by pharyngitis followed by Fusobacterium necrobacillosis sepsis complicated by internal jugular vein thrombosis and infectious metastatic abscesses. It has been considered to be a rare disease until the last decade when a larger number of cases have been reported. We discuss a case of Lemierre syndrome in a 17-year-old girl and review the pathophysiology of Fusobacterium necrobacillosis and diagnosis and treatment of Lemierre syndrome.
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PMID:Lemierre syndrome. 1616 Jun 63

Adrenal insufficiency is a rare disease, but its prevalence is increasing. The most frequent cause of primary adrenal insufficiency in western countries is autoimmune adrenalitis, whereas secondary adrenal insufficiency is most often caused by pituitary tumours and their treatment (e.g., surgery). Chronic glucocorticoid replacement consists of hydrocortisone 15-25 mg/day in divided doses and dose monitoring is largely based on clinical judgement. Fludrocortisone 0.05-0.2 mg/day is given for substitution in mineralocorticoid deficiency aiming at normotension, normokalaemia and a plasma renin activity in the upper normal range. It has recently been shown that, despite adequate glucocorticoid and mineralocorticoid replacement well being in patients with adrenal insufficiency is still impaired. Several studies have demonstrated that dehydroepiandosterone 25-50 mg/day p.o. may improve mood, fatigue, well-being and, in women, also sexuality, suggesting that dehydroepiandosterone should become part of the standard treatment regime. However, large Phase III trials of dehydroepiandosterone for adrenal insufficiency are still lacking and it has not yet been approved for the treatment of this disease. Patients with adrenal insufficiency are at risk of adrenal crisis, usually precipitated by major stress, such as severe infection or surgery. Early dose adjustments are required to cover the increased glucocorticoid demand in stress. Careful and repeated education of patients and their partners is the best strategy to avoid this life-threatening emergency. Some recent studies suggest that during sepsis some patients with intact adrenal function may develop transient relative adrenal insufficiency and benefit from administration of hydrocortisone plus fludrocortisone. However, the pathophysiology and diagnosis criteria of relative adrenal insufficiency and its treatment remain unsettled issues.
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PMID:Management of adrenal insufficiency in different clinical settings. 1625 72


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