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)

Noma Neonatorum is characterized by a gangrenous process involving mucocutaneous junctions of oral, nasal and anal area and occasionally, the eyelids and scrotum. It is seen during the first few weeks of neonatal life in premature and low birth weight babies. Noma Neonatorum is commonly described with pseudomonas aeruginosa septicemia. A case of Noma Neonatorum associated with E.coli sepsis is described for the first time.
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PMID:Noma neonatorum. 1674 34

In this report, we describe a brother and sister who presented at birth with short-limb skeletal dysplasia, polyhydramnios, prematurity, and generalized edema. Dysmorphic features included broad nose, thick ears, thin lips, micrognathia, inverted nipples, ulnar deviation at the wrists, spatulate fingers, fifth finger camptodactyly, nail hypoplasia, and talipes equinovarus. Other features included short stature, microcephaly, psychomotor retardation, B-cell lymphopenic hypogammaglobulinemia, sensorineural deafness, retinal detachment and blindness, intestinal malrotation with poor gastrointestinal motility, persistent hyponatremia, intermittent hypoglycemia, and thrombocytopenia. Cardiac anomalies included PDA, VSD, hypertrophic cardiomyopathy, and arrhythmias. The brother had a small penis with hypospadias, hypoplastic scrotum, and non-palpable testes. Skeletal findings included absent ossification of cervical vertebral bodies, pubic bones, knee epiphyses, and tali. Both sibs died before age 2 years, one of overwhelming sepsis and the other of cardiorespiratory failure associated with her cardiomyopathy. Metabolic studies showed a type 1 pattern of abnormal serum transferrin glycosylation. Fibroblasts synthesized truncated LLOs, primarily Man(7)GlcNAc(2), suggestive of CDG-Ig. Both sibs were compound heterozygotes for a novel 301 G > A (G101R) mutation and a previously described 437 G > A (R146Q) mutation in ALG12. Congenital disorders of glycosylation should be considered for children with undiagnosed multi-system disease including neurodevelopmental delay, skeletal dysplasia, immune deficiency, male genital hypoplasia, and cardiomyopathy.
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PMID:Expanding spectrum of congenital disorder of glycosylation Ig (CDG-Ig): sibs with a unique skeletal dysplasia, hypogammaglobulinemia, cardiomyopathy, genital malformations, and early lethality. 1750 7

A 53-year-old male was admitted with a two-day history of abdominal pain, anal bleeding, fever, diarrhea, vomiting, and mental confusion. A diagnosis of thrombosis of very large hemorrhoids (Grade 4) was made. On the day of admission, he underwent an exploring laparotomy followed by abdominoperineal resection. The peritoneal cavity was filled with pus and blood clots. Because rectal necrosis was involved, sigmoid colostomy was imperative. Twenty-eight hours after surgery, the patient demonstrated signs of soft-tissue perineal necrosis associated with progressive pain and fever. He developed a rapidly progressive gangrene of the lower limbs and scrotum followed by acute renal and respiratory failure, and he died of sepsis. At autopsy, the cadaver showed jaundice and a large gangrene of the perineum and lower limbs. The internal organs showed features secondary to sepsis complications. To the best of our knowledge, this is the first autopsy study of a patient who died because of complications of hemorrhoids.
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PMID:Death resulting from fournier gangrene secondary to thrombosis of very large hemorrhoids: report of a case. 1784 38

Fournier's gangrene is a life-threatening necrotising infection of the perineal and genital regions. The case presented here refers to an HIV-positive 42-year-old man, admitted in emergency to our department with clinical signs and symptoms of sepsis related to gangrene of the perineum and scrotum. An early wide surgical necrosectomy was performed under epidural anaesthesia. Treatment was completed by intensive care, broad-spectrum antibiotics and hyperbaric oxygen therapy. The wound was managed with advanced dressing (AQUACEL Hydrofiber) until complete healing was obtained, and the scrotum was reconstructed with skin flaps. The disease did not involve the testes, spermatic cord or anorectal canal. The satisfactory aesthetic and functional outcome prompts the authors to stress a number of features of the therapeutic approach adopted: (i) the advantages of epidural anaesthesia with an indwelling catheter that allows further necrosectomy and wound dressing to be performed totally painlessly; (ii) the possibility of avoiding faecal diversion by means of synthetic opioid drugs which are useful to reduce the frequency of defecation; and (iii) the positive impact of advanced dressing on the wound healing process in relation to patient satisfaction and cost management.
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PMID:[Fournier's gangrene in an HIV-positive patient. Therapeutic options]. 1883 66

Intestinal injury following abdominal radiation therapy or accidental exposure remains a significant clinical problem that can result in varying degrees of mucosal destruction such as ulceration, vascular sclerosis, intestinal wall fibrosis, loss of barrier function, and even lethal gut-derived sepsis. We determined the ability of a high-molecular-weight polyethylene glycol-based copolymer, PEG 15-20, to protect the intestine against the early and late effects of radiation in mice and rats and to determine its mechanism of action by examining cultured rat intestinal epithelia. Rats were exposed to fractionated radiation in an established model of intestinal injury, whereby an intestinal segment is surgically placed into the scrotum and radiated daily. Radiation injury score was decreased in a dose-dependent manner in rats gavaged with 0.5 or 2.0 g/kg per day of PEG 15-20 (n = 9-13/group, P < 0.005). Complementary studies were performed in a novel mouse model of abdominal radiation followed by intestinal inoculation with Pseudomonas aeruginosa (P. aeruginosa), a common pathogen that causes lethal gut-derived sepsis following radiation. Mice mortality was decreased by 40% in mice drinking 1% PEG 15-20 (n = 10/group, P < 0.001). Parallel studies were performed in cultured rat intestinal epithelial cells treated with PEG 15-20 before radiation. Results demonstrated that PEG 15-20 prevented radiation-induced intestinal injury in rats, prevented apoptosis and lethal sepsis attributable to P. aeruginosa in mice, and protected cultured intestinal epithelial cells from apoptosis and microbial adherence and possible invasion. PEG 15-20 appeared to exert its protective effect via its binding to lipid rafts by preventing their coalescence, a hallmark feature in intestinal epithelial cells exposed to radiation.
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PMID:Oral PEG 15-20 protects the intestine against radiation: role of lipid rafts. 1983 62

A minute, pleomorphic, motile, Gram-negative bacterium has been isolated from two specimens of nodular tissue from human verruga. In films and sections of the original tissues the organism in question is difficult to distinguish from Bartonella bacilliformis, with which it was associated, and even in pure culture it has a number of properties in common with that parasite. No sugars are fermented by it, it is an obligate aerobe, the optimum temperature for its growth is 25 degrees C., and it has two to four spiral flagella attached to one end of the body. It is, however, readily cultivated on any ordinary culture medium. Broth cultures contain much mucin, but no hydrogen sulfide is formed. Coagulated serum is liquefied by its growth, and the red corpuscles in a blood agar plate are hemolyzed. Rabbits, guinea pigs, rats, and mice develop acute, fatal septicemia as a result of intravenous or intratesticular inoculation of young cultures. The liver is characteristically affected and shows a general parenchymatous degeneration and necrosis; the entire gastrointestinal tract is intensely congested, and numerous hemorrhagic areas are present; the spleen, dark and soft, is rarely much enlarged; the kidneys are swollen and congested; the adrenals are much swollen and intensely red; the lungs are sometimes congested but otherwise normal. In the case of intratesticular inoculation the scrotum and testicle both undergo rapid gangrene. In monkeys no septicemia has been observed, but a violent local reaction-swelling, congestion, sometimes necrosis-follows intradermal inoculation. Since no microorganism corresponding in character with this one has previously been described, it is regarded as a new species, and because of its presence in material obtained from Peru it has been given the name Bacterium peruvianum. The significance of the association of B. peruvianum with Bartonella bacilliformis deserves further investigation; it is not impossible that the two organisms are introduced into the human body by the same blood-sucking insect.
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PMID:ETIOLOGY OF OROYA FEVER : IX. BACTERIUM PERUVIANUM, N. SP., A SECONDARY INVADER OF THE LESIONS OF VERRUGA PERUANA. 1986 95

A 62-year-old patient diagnosed with pulmonary blastoma with submandibular, scrotum and adrenal metastases was admitted to Sotiria General Hospital in Athens. No other such case has been published to date. The patient started receiving chemotherapy, but the scrotum metastasis grew rapidly and erupted. This led to sepsis despite surgical excision of infected and necrotic tissues and intravenous antibiotics. Treatment strategy in pulmonary blastoma should be defined by a multidisciplinary team, and surgical treatment should be considered as quickly as possible when such a tumor is suspected.
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PMID:Pulmonary blastoma with submandibular, scrotum and adrenal metastases: case report. 2144 35

Necrotizing fasciitis (NF) is an uncommon soft tissue infection, usually caused by toxin-producing virulent bacteria. It is characterized by widespread fascial necrosis primarily caused by Streptococcus hemolyticus. Shortly after the onset of the disease, patients become colonized with their own aerobic and anaerobic microflora from the gastrointestinal and/or urogenital tracts. Early diagnosis with aggressive multidisciplinary treatment is mandatory. We describe three clinical cases with NF. The first is a 69 years old man with diabetes mellitus type II, who presented with NF on the posterior chest wall, shoulder and arm. He was admitted to the intensive care unit (ICU) with a clinical picture of severe sepsis. Outpatient treatment and early surgical debridement of the affected zones (inside 3 hours after admittance) and critical care therapy were performed. The second case is of a 63 years old paraplegic man with diabetes mellitus type I. Pressure sores and perineal abscesses progressed to Fournier's gangrene of the perineum and scrotum. He had NF of the anterior abdominal wall and the right thigh. Outpatient treatment and early surgical debridement of the affected zones (inside 6 hour after admittance) and critical care therapy were performed. The third patient was a 56 year old man who had NF of the anterior abdominal wall, flank and retroperitoneal space. He had an operation of the direct inguinal hernia, which was complicated with a bowel perforation and secondary peritonitis. After establishing the diagnosis of NF of the abdominal wall and retroperitoneal space (RS), he was transferred to the ICU. There he first received intensive care therapy, after which emergency surgical debridement of the abdominal wall, left colectomy, and extensive debridement of the RS were done (72 hours after operation of inquinal hernia). On average, 4 serial debridements were performed in each patient. The median of serial debridement in all three cases was four times. Other intensive care therapy with a combination of antibiotics and adjuvant hyperbaric oxygen therapy (HBOT) was applied during the treatment. After stabilization of soft tissue wounds and the formation of fresh granulation tissue, soft tissue defect were reconstructed using simple to complex reconstructive methods.
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PMID:Necrotizing fasciitis: literature review of contemporary strategies for diagnosing and management with three case reports: torso, abdominal wall, upper and lower limbs. 2306 46

Fournier gangrene represents a rare but progressive perineal infection that may result in rapid death. A 70-year-old man with poorly controlled diabetes mellitus and alcohol abuse is reported who was found unexpectedly dead. He had last been contacted the night before his death. At autopsy, the most striking finding was deep necrotic ulceration of the scrotum with exposure of underlying deep muscles and testicles, with blood cultures positive for Escherichia coli. Death was, therefore, attributed to necrotic ulceration/gangrene of the perineum (Fournier gangrene) that was due to E. coli sepsis with underlying contributing factors of diabetes mellitus and alcoholism. In addition there was morbid obesity (body mass index 46.9), cirrhosis of the liver, and marked focal coronary artery atherosclerosis with significant cardiomegaly. Fournier gangrene may be an extremely aggressive condition that can result in rapid death, as was demonstrated by the rapid progression in the reported case.
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PMID:Fournier gangrene and unexpected death. 2247 25

Fournier's gangrene is a rare process which affects soft tissue in the genital and perirectal area. It can also progress to all different stages of sepsis, and abdominal compartment syndrome can be one of its complications. Two patients in septic shock due to Fournier gangrene were admitted to the Intensive Care Unit of Emergency Department. In both cases, infection started from the scrotum and the necrosis quickly involved genitals, perineal, and inguinal regions. Patients were treated with surgical debridement, protective colostomy, hyperbaric oxygen therapy, and broad-spectrum antibacterial chemotherapy. Vacuum-assisted closure (VAC) therapy was applied to the wound with the aim to clean, decontaminate, and avoid abdominal compartmental syndrome development. Both patients survived and were discharged from Intensive Care Unit after hyperbaric oxygen therapy cycles and abdominal closure.
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PMID:Treatment of Fournier's Gangrene with Combination of Vacuum-Assisted Closure Therapy, Hyperbaric Oxygen Therapy, and Protective Colostomy. 2260 89


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