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Query: UMLS:C0036690 (
sepsis
)
59,461
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The 6-year follow-up of a patient affected by Wolfram's syndrome, a
rare disease
characterized by diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract and other abnormalities (DIDMOAD or Wolfram's syndrome), is described. Our patient has diabetes insipidus, diabetes mellitus, abnormal audiograms, without subjective evidence of hearing loss, and dilatation of the urinary tract. Diagnosis was suspected at the age of 8 years. Diabetes mellitus was the first manifestation and treatment with insulin was necessary. Desmopressin therapy decreased dramatically the daily urinary output. In view of the significant morbidity and mortality from renal failure associated with recurrent urinary infections, we have drawn special attention to the urological manifestations of the syndrome. During the follow-up, the patients underwent some investigations, such as renal ultrasound and echotomography and cystourethroscopy. Outstanding results of these studies are severe bilateral hydronephrosis with dilatated ureters and loss of renal tissue. The particular finding is the presence of posterior urethral valves with obstructed bladder. The anatomical outlet obstruction are variable and may be disastrous. There may be failure to thrive,
sepsis
, anemia be disanal failure. In such instances corrective surgery could improve bladder and ureteral functions.
...
PMID:[Wolfram syndrome. Peculiar urologic aspects]. 779 16
Fournier's gangrene is a necrotising soft-tissue infection of the scrotum and perineal region caused by gram-negative and gram-positive Enterobacteriaceae. The disease is characterised by its unique appearance, its speed of onset, and its high mortality. CASE REPORT. A 26-year-old male presented to the emergency room complaining of a painful, tremendously swollen scrotum and penis (Fig. 1) that had developed within the past 24 h. Later, slurred speech, pallor, and hypotension were recognised, leading to the patient's admission to the intensive care unit. Suspecting a severe internal haemorrhage, vigorous volume therapy was started using crystalloids and colloids until blood and fresh frozen plasma were available. One hour later, septic shock was presumed and therapy augmented by IV antibiotics, tracheal intubation, and mechanical ventilation. Despite all efforts, the patients condition deteriorated rapidly and he died a few hours later due to multiple organ failure in septic shock. Postmortem, a perforated external hemorrhoidal node was found to be the primary focus of
sepsis
. Microbiologic cultures revealed Escherichia coli in blood and tissue samples. DISCUSSION. Fournier's gangrene is a
rare disease
; nevertheless, its clinical picture has to be recognised immediately in order to provide appropriate treatment in time. It occurs predominantly in males after minor trauma, colorectal or urological disease, and perineal or abdominal surgery. Fournier's gangrene usually begins with itching and pain in the scrotal region followed by swelling and dark-blueish discolouration of the scrotum and penis, occasionally including the lower abdominal wall. Fever and chills are usually present. The illness progresses to severe prostation and septic shock with a mortality of 20%-50%. Tissue cultures mostly reveal E. coli, gram-positive enterococci, Pseudomonas, Proteus, and various anaerobes. The treatment should include immediate radical surgical debridement, i.v. administration of broad-spectrum antibiotics, and cardiopulmonary support. CONCLUSION. The dramatic course of Fournier's gangrene requires early recognition, extensive surgical debridement, as well as intensive care treatment in order to prevent irreversible septic shock.
...
PMID:[Fulminating E. coli sepsis in Fournier's gangrene]. 814 38
Enteritis necroticans is a necrotizing process manifesting as segmental gangrene of the bowel, triggered by Clostridium perfringens toxins under specific dietary conditions. It is a
rare disease
in developed countries and is probably underdiagnosed. A case of enteritis necroticans presenting with midgut necrosis with
sepsis
and hemolysis is reported herein. Bacteriologic culture of blood and peritoneal content revealed C perfringens. Dietary history, including the ingestion of meat together with sweet potatoes, should increase clinical suspicion of enteritis necroticans. Early recognition and timely surgical intervention are required for successful treatment. Clinicians are encouraged to be aware of this clinically fulminant yet rarely recognized surgical entity.
...
PMID:Enteritis necroticans with midgut necrosis caused by Clostridium perfringens. 818 79
Purulent pericarditis is diagnosed when pus is drained from the pericardial space or when bacteria are cultured from the pericardial fluid. This
rare disease
is often diagnosed late, when severe hemodynamic compromise develops due to pericardial tamponade. It is usually a complication of pneumonia, especially if there is empyema as well, and often follows chest surgery or chest wall infections. It sometimes appears in patients with
septicemia
, especially when they are debilitated or immuno-compromised. Diagnosis is aided by echocardiography. Pericardiocentesis and drainage of the pus, as well as prolonged antibiotic treatment, are mandatory. Delay in diagnosis and treatment often results in death. Some surviving patients may develop constrictive pericarditis and require pericardiectomy. We report a 73-year-old man with pulmonary lymphoma who suffered from purulent pericarditis secondary to
sepsis
with methicillin-resistant Staphylococcus aureus. Pericardial drainage and appropriate antibiotic treatment eventually resulted in complete recovery.
...
PMID:[Purulent pericarditis]. 879 38
Mesenteric panniculitis is a
rare disease
involving the adipose tissue of the mesentery. We report a case of a 27-year-old woman with mesenteric panniculitis, who presented clinical and radiological features mimicking Crohn's disease. In the outcome, she presented a small bowel perforation, unusual in this pathology, and an annexial involvement. This case reminds us of the role of
sepsis
and repeated abdominal surgery in relation to the pathogenesis of mesenteric panniculitis. We report the first case of mesenteric panniculitis mimicking Crohn's disease.
...
PMID:[Mesenteric panniculitis simulating Crohn disease]. 899 Nov 52
The evaluation of tissue copper concentration is essential for the diagnosis of Wilson's disease. It is characterized by symptoms of the damages to parenchymatous organs, primarily liver and brain, due to chronic copper intoxication. The paper presents the autopsy tissue analysis of a 39-year-old patient diagnosed to suffer from the Wilson's disease while still alive. The patient died from
sepsis
due to burns caused by direct flame exposure. The standard histochemical staining of tissue samples failed to demonstrate the presence of copper but it was qualitatively proved by line spectrum-based mass spectrography. The copper concentrations in the liver, brain, and kidney (240, 73.8, and 30 micrograms/g wet tissue weight, respectively), measured by flame-atomic absorption spectrometry, were significantly elevated. In this study, the Wilson's disease was verified by a postmortem determination of increased copper concentration in the tissues. The results obtained contribute to the understanding of this
rare disease
.
...
PMID:[Copper levels in the tissues of patients with Wilson's disease]. 947 69
Polycythemia--characterized by an excessive number of erythrocytes--is a
rare disease
in the dog with a chronic progressive course and unspecific symptoms. There are several forms: a primary, a secondary adequate or a secondary inadequate polycythemia. The clinical workup is done step by step and after stabilization of critical patients, the remaining therapy must address the primary cause. We report on a five year old male Leonberger dog suffering from secondary, inadequate polycythemia. He was presented with apathy, gait disturbances and disorientation. On the basis of the diagnostic workup a pathological process in the kidneys was postulated. Initially focal seizures became generalized later, most probably because of formation of a forebrain thrombus with secondary hypoxia. Even after emergency treatment the general state deteriorated. The course indicated possible
sepsis
. Because of the critical picture with secondary complications and the poor prognosis, the dog was euthanised. The histopathological results showed T-cell renal lymphoma and secondary injury to the forebrain.
...
PMID:[Convulsions in relation to polycythemia: literature review and case description]. 952 46
The problems of terminology are discussed, as well as classification and definition of
sepsis
. Comparative analysis of traditional for our country and up-to-date foreign classifications is carried out. The criteria for diagnosis of
sepsis
are discussed. On the basis of 20-year experience in examination and treatment of patients with
sepsis
, the authors set forth their own stand, suggesting two varieties of
sepsis
for consideration: common complication of surgical infection, a
rare disease
.
...
PMID:[Surgical sepsis-definition of the notion. Problems of terminology]. 1054 May 44
Acute necrotizing esophagitis is a
rare disease
. Its pathogenesis is influenced by situations of low systemic perfusion, such as hypertension, heart failure or
sepsis
, in which other factors, such as the application of a nasal tube, infections or drugs also play a role. We present a case of acute necrotizing esophagitis in a patient with copious vomiting, renal failure, gastric hemorrhage due to Mallory-Weiss syndrome and esophageal infection due to Actinomyces. The patient was undergoing coadjuvant chemotherapy for a surgically-treated colonic neoplasia. Maintenance therapy produced favorable evolution with restoration of esophageal epithelium and no stenotic complications.
...
PMID:[Acute necrotizing esophagitis]. 1072 88
Intrahepatic lithiasis (IHL) is a very
rare disease
in Europe and no gold-standard treatment has yet been codified. The aim of our study is to report our personal experience of IHL detected in 20 patients (6 males [30%] and 14 females [70%]) between January 1982 and December 1991. Eleven (55%) of these cases presented with only IHL, 7 cases (35%) had IHL associated with gallbladder or common bile duct stones, while 2 (10%) were affected by secondary IHL caused by previous biliodigestive anastomosis-induced stricture. The diagnosis was determined by cholangiography in all patients, by ultrasound in 94.7%, and by computed tomography in 70%. The surgical procedures used were as follows: liver resection in 12 patients (60%); various types of biliodigestive anastomosis in 6 cases (30%); choledocholithotomy with a simultaneous removal of stones from the hepatic duct in 2 patients (10%). The intraoperative mortality rate was nil. Complications consisted of 2 subphrenic abscesses, 1
septicemia
, and 1 bowel occlusion. Two patients (10%) dropped out of the follow-up, 2 (10%) died 2 years after surgery for reasons not connected with the disease, 1 (5%) died from suppurative cholangitis 6 months after surgery, 2 (10%) had recurrent stones and were treated by extracorporeal lithotripsy and endoscopic stone removal, while the remaining 13 patients (65%) are symptom-free at follow-up which varied between 6 months and 11 years. Liver resection is the treatment of choice when IHL is confined to one lobe of the liver. When IHL affects the entire liver, a resection of the main involved area should be performed whenever possible. Other options include hepatoduodenal anastomosis using the interposed jejunal loop to enable endoscopic or combined treatment of recurrent stones.
...
PMID:Intrahepatic lithiasis: a Western experience. 1079 62
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