Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epidermolytic hyperkeratosis is an unusual type of ichthyosis. This inherited keratinization disorder is characterized clinically by erythema, blistering, and peeling shortly after birth. It may resolve and be replaced with thick scaling. It can lead to life-threatening complications, such as sepsis. Histologically, there is a hyperkeratosis and vacuolar degeneration. Genetically, this is an autosomal dominant disease with complete penetrance; however, 50% are spontaneous mutations. The clinical phenotype is a result of alterations in the gene(s) for keratin 1 and/or 10. We review this disorder and its therapy, which is mainly symptomatic with emollients and retinoids.
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PMID:Epidermolytic hyperkeratosis: a keratin 1 or 10 mutational event. 1566 49

A 38-year-old male farm worker with relapsing acute lymphoblastic leukemia spontaneously developed an ulcerating ulcer on his anterior thigh which was surrounded by a non-tender area of erythema. Bacillus cereus was isolated from the ulcer and blood, and the patient received intravenous penicillin and vancomycin for one week. When sensitivity studies were returned he was treated with gatifloxacin orally. After two weeks of combined antimicrobial therapy and negative blood cultures, the patient received combination chemotherapy with vincristine, prednisone, doxorubicin and cyclophosphamide. He was hospitalized a day after completing chemotherapy with neutropenic sepsis due to B. cereus. He received similar antimicrobial therapy as previously, but died three days later. At autopsy, the patient was found to have acute mitral valve endocarditis and bilateral brain abscesses. This was the first case of B. cereus endocarditis reported in a patient with acute lymphoblastic leukemia.
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PMID:Fatal Bacillus cereus endocarditis masquerading as an anthrax-like infection in a patient with acute lymphoblastic leukemia: case report. 1570 Apr 34

Glucagonoma is a very rare endocrine pancreatic tumor. At diagnosis, most glucagonomas are malignant and often metastatic. Suspicion of glucagonoma is based on characteristic presentations known as "glucagonoma syndrome". Glucagonoma is often found in the pancreatic body and/or tail and is usually large enough to be localized by computed tomography. We report a case of diffuse glucagonoma necrolytic migratory erythema (NME) in a 45-year-old man with mild diabetes mellitus, mild anemia, and weight loss over 1.5 years. Diffused enlarged pancreas was noted on abdominal ultrasonography incidentally during a routine health check-up. The levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were within normal limits. No enlarged lymph node or extrapancreatic tumor mass was found by several imaging studies. Total pancreatectomy was performed, and the pathology revealed glucagon-producing islet cells and intrapancreatic vascular emboli of tumor cells. He died due to internal bleeding and sepsis after surgery. Presentation of diffuse malignant glucagonoma with tumor emboli but no metastasis or NME is unusual.
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PMID:Rare presentation of endocrine pancreatic tumor: a case of diffuse glucagonoma without metastasis and necrolytic migratory erythema. 1595 5

Calciphylaxis is a potentially life-threatening disease that occurs up to 4% of patients with chronic terminal renal failure and secondary hyperparathyroidism. Clinical symptoms are painful skin erythema and ischemic necrosis as a result of calcification of the small and medium-sized arteries of the subcutaneous tissues. The mortality of the disease is as high as 87% mainly due to sepsis from superinfection. We report on a 59-year-old patient with chronic renal failure and normal calcium-phosphate-product with leg ulcers as a rare manifestation of a calciphylaxis. In spite of an early parathyroidectomy and a temporary complete wound-healing the death because of calciphylaxis could not be prevented.
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PMID:[Calciphylaxis in terminal renal failure as a rare manifestation of leg ulcers]. 1618 43

A 4-year-old Japanese boy, the youngest of three brothers, presented with ichthyosiform hyperkeratosis over his whole body, eczematous erythema with partial desquamation and erosion on the flexor side of the joints of extremities, the fossa axillaries, and the genital and buttock regions, and total hair loss on the scalp and the absence of eyebrows and eyelashes. In addition to the ichthyotic eruptions and hair abnormalities, he also had a ventricular septal defect, mental retardation, growth retardation, characteristic facial features such as a depressed nasal bridge, low-set ears, and ocular hypertelorism; therefore, he was diagnosed with cardio-facio-cutaneous (CFC) syndrome. The patient's family did not have a history of consanguineous marriage. The parents and the eldest son were healthy. However, the second son, also born with ichthyosiform hyperkeratosis over his whole body, total hair loss on the scalp, myocardial deficiency, mental retardation, growth retardation, and characteristic facial features, had died of pneumonia and sepsis at the age of 1.5 years. Because the middle brother had the same disease, the present case is considered to be a rare case of CFC syndrome with in a single generation.
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PMID:Cardio-facio-cutaneous syndrome: two cases in the same generation. 1636 53

A 54-year-old man with hepatitis C fell into shock with symptoms similar to enterocolitis after ingesting an undercooked barbecued mackerel. Most of his eruptions developed into annular erythema with small vesicles. He had taken high dose corticosteroids with intravenous cefotiam. His eruptions improved, but his shock state was exacerbated on Day 2. Treatment for endotoxin shock was initiated using piperacillin, intravenous immunoglobulin (IVIg), and hemoperfusion with Polymyxin B immobilized fiber (PMX-F), which resulted in shock reversal. The serum IL-6 value was 118,000 pg/mL on admission, and decreased to 2040 pg/mL on Day 3. On Day 6, the results from the culture of skin biopsy specimens showed the diagnosis as Vibrio vulnificus septic shock. Debridement was not needed, which is thought to be essential to Vibrio vulnificus sepsis. The changes in the serum IL-6 levels demonstrated that hemoperfusion with PMX-F and IVIg therapy was practical for Vibrio vulnificus septic shock.
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PMID:Endotoxin shock due to Vibrio vulnificus infection. 1693 3

A phase I/II clinical study was performed to evaluate the safety and potential efficacy of topical recombinant human basic fibroblast growth factor on the healing of partial-thickness skin graft donor sites in burned children. Each child served as his or her own control. In a blinded and random fashion, one donor site was sprayed with basic fibroblast growth factor (5 microg/cm(2)) on days 0 to 4 after harvest, whereas the other site was treated with vehicle. Twelve patients were entered in the study but one patient died of sepsis that was unrelated to growth factor treatment. Of the remaining 11 patients, no adverse events related to basic fibroblast growth factor occurred. Serum basic fibroblast growth factor levels were never detected and antibody levels remained unchanged. No differences in the rate of epithelialization or days until complete closure were noted (basic fibroblast growth factor = 12.9 +/- 3.9 days, placebo = 12.2 +/- 5.5 days; mean +/- standard error of the mean). No differences in pain, itching, wound fragility, erythema, scarring, or pigmentation were noted. All of the scars matured within 1 year with good to excellent results. Investigators, patients, or families could not distinguish between the two wounds. Although basic fibroblast growth factor proved safe, no enhancement of donor site healing was seen in this small study. Because the time for donor site healing limits subsequent autograft use in patients with sizeable burns, studies should focus on accelerating healing in patients with larger burns where donor site healing is delayed and reharvest is required.
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PMID:Effects of basic fibroblast growth factor on the healing of partial-thickness donor sites. A prospective, randomized, double-blind trial. 1713 80

Flushing of the skin of an infant may be a sign of the child's first allergic reaction to food, insect envenomation, or other allergens, a sign of sepsis, or due to dilation of cutaneous vessels caused by a vasodilator substance or neural mechanisms. A rare cause of this condition results in the release of mast cell mediators such as histamine, prostaglandin D2, tryptase, chymase, and leukotrienes. We present a case of a 6-month-old with severe total body flushing and a yellow-tan, raised, well-demarcated lesion on the thigh consistent with a solitary mastocytoma. Erythema was most pronounced adjacent to the lesion, suggesting a positive Darier sign. Subsequent evaluation by a dermatologist confirmed the diagnosis, and the patient underwent no further therapy; however, the family was appropriately counseled on management if the symptoms were to reappear. Appropriate diagnosis and management of this patient and other forms of mastocytosis in children are discussed.
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PMID:A 6-month old with total body flushing and a macular-papular lesion. 1750 76

Granuloma annulare is a benign inflammatory skin lesion of unknown etiology that is usually seen in adults and children and subtypes of it includes localized granuloma annulare, generalized granuloma annulare, subcutaneous granuloma annulare and arcuate dermal erythema. Etiology and pathogenesis of granuloma annulare are obscure, although there is much evidence for an immunologic mechanism. Precipitating factors are insect bites, sunburn, photochemotherapy, drugs, physical trauma, acute phlebitis and sepsis after surgery. Some investigators were suggested a relationship of granuloma annulare to a latent or clinically manifest diabetes or rheumatoid arthritis. In contrast, an association of subcutaneous granuloma annulare with these diseases in childhood has not been reported in the literature. Subcutaneous granuloma annulare of the scalp is rare lesion in childhood and nodules on the scalp are usually non-, or slightly mobile, whereas lesions on the extremities are freely mobile. For definitive diagnosis, a biopsy should be performed but wide surgical intervention or medical treatment is not indicated. In case of recurrence, no additional diagnostic studies are necessary.
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PMID:Subcutaneous granuloma annulare of the scalp in childhood: a case report and review of the literature. 1791 73

An 85-year-old man patient was admitted to the hospital complaining of fever and bilateral leg pain with swelling and erythema. A laboratory investigation revealed leukocytopenia, thus suggesting sepsis. Gram negative rods were detected in the specimen from the affected skin and empiric antibacterial therapy was initiated. The following day, his symptoms worsened and Pseudomonas aeruginosa was isolated from the blood culture and the skin specimen. Magnetic resonance imaging (MRI) did not show the typical characteristics of necrotizing fasciitis. In spite of intensive medical treatment, the patient's condition became critical, and on day 10 after admission, he died of multiple organ failure. An autopsy revealed necrotizing fasciitis due to P. aeruginosa. This is a rare case and its clinical presentation was atypical. The clinical diagnosis of this infection may be difficult, and therefore such cases warrant the careful attention of physicians.
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PMID:Necrotizing fasciitis caused by Pseudomonas aeruginosa. 1834 45


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