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)

Acute liver failure is a rare disease that can cause death in the majority of untreated cases. Sudden loss of liver function in the absence of a preexisting liver disease is considered the true form and has to be distinguished from impaired function following exacerbation of an underlying liver disease (acute or chronic failure). Common causes include acute viral hepatitis, drug induced liver injury (DILI) and toxins. The loss of the excretory and synthetic function of the liver marks the clinical presentation and results in icterus, coagulopathy and encephalopathy. Additionally impairment of renal function and sepsis occur and contribute to the high mortality of this disease. The activation of cell death mechanisms (apoptosis) leading to a reductio of viable, functional liver tissue is considered to be an important pathophysiologic mechanism. Curative therapy of this disease includes liver transplantation that has been performed in Germany for the first time in 1969. In the year 2004 a total of 91 liver transplantation were performed for acute liver failure (10.3% of all transplants) in German transplant centers.
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PMID:[Acute liver failure--medical viewpoints]. 1717 26

Wandering spleen, which is defined as a spleen without peritoneal attachments, is a rare disease and a delay in the clinical and/or radiological diagnosis may lead to splenic torsion, infarction, and necrosis. Owing to the physiologic importance of the spleen, especially in children, and the risk of postsplenectomy sepsis, early diagnosis and splenopexy are recommended. In the present article, we describe the results of our management of this rare problem on six patients, and we review all available literature from 1895 to 2005. Briefly, our technique includes flap creation from parietal peritoneum and settlement of spleen in the fossa splenica. Free edges of this flap are stitched to the stomach and the left end of transverse colon and the beginning of the descending colon. The body of the stomach was stitched to the abdominal wall to prevent gastric volvulus, while the fundus region was fixed to the diaphragm to support the spleen. Finally, an omental patch was stitched to the intact abdominal wall above the flap. In conclusion, the procedure of splenopexy without using mesh is considered to be a safe and curative modality for wandering spleen without imposing any undue risk of infection or foreign material reaction.
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PMID:Surgical treatment of patients with wandering spleen: report of six cases with a review of the literature. 1734 72

A 55 years old man presented with long standing voiding obstructive symptoms like poor stream, frequency of micturition, straining at micturition and a sense of incomplete evaluation since 2002. He was clinically diagnosed a case of stricture urethra. Later he developed a swelling in the penoscrotal region with retention of urine in March 2005 and was treated by dilatation. But on removal of the catheter he developed retention again for several times and ultimately for gross periurethral sepsis, urinary diversion was required in May 2005. In October 2005, biopsy from the scrotal abscess wall established the diagnosis of urethral carcinoma. In November 2005 extensive penoscrotectomy was done followed by chemo-radiotherapy. But the prognosis was so bad that the patient died in November 2006. Urethral carcinoma is a rare disease diagnosed in the department of urology, Mymensingh Medical College Hospital, the prognosis of which is also poor even after extensive operations.
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PMID:Male urethral cancer. 1734 91

Concomitant herpetic and candidal esophagitis is a very rare disease that had not been reported in uremic patients. A 57-year-old woman receiving continuous ambulatory peritoneal dialysis (CAPD) therapy for 3 years was admitted due to CAPD-related peritonitis. Endoscopic examination was performed due to severe epigastralgia and upper gastrointestinal bleeding, and combined herpetic and candidal esophagitis was diagnosed. Intravenous acyclovir and fluconazole were prescribed and symptoms improved. The patient subsequently died due to progressive sepsis and respiratory failure. This is the first report of a dual infectious esophagitis in a uremic patient. Since infectious esophagitis may cause severe complications, early diagnosis and aggressive treatment are important.
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PMID:Combined herpes viral and candidal esophagitis in a CAPD patient: case report and review of literature. 1749 41

Approximately 60 cases of biliary papillomatosis have been reported in the world literature, while only 6 cases have been reported to be treated with liver transplantation. This rare disease, which is characterized by relapsing episodes of obstructive jaundice and cholangitis that lead to secondary cirrhosis and death from sepsis or liver failure, it is also considered premalignant because of its frequent malignant transformation (25-50%). We present a case of a 43-year-old white man with papillomatosis of intra- and extrahepatic biliary tree who sought care for repeated episodes of obstructive jaundice and cholangitis. The diagnosis was suspected after endoscopic retrograde cholangiopancreatography and confirmed by liver and common bile duct biopsies. The patient underwent orthotopic liver transplantation with Roux-en-Y hepatico-jejunostomy to treat end-stage liver cirrhosis. Fifteen months' follow-up revealed a patient with normal graft function and with no clinically or laboratory findings of disease recurrence or cancer development.
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PMID:Papillomatosis of intra- and extrahepatic biliary tree: Successful treatment with liver transplantation. 1760 Mar 52

Boerhaave syndrome is a rare disease with a mortality rate that varies from 10 to 40%. The typical clinical presentation (vomiting, pain, subcutaneuous emphysema) is relatively infrequent. In the case of atypical clinical presentation CT scan with contrast medium administered per os is fundamental for diagnosis. Though there is no general consensus on therapeutic strategies, prognosis is dependent on time interval between onset and diagnosis. We observed four patients with Boerhaave syndrome with an atypical presentation. The time lapse between acute event and diagnosis was less than 6 hours in two cases, 24 hours in one case and 72 hours in the last. All patients presented abdominal pain at admission, preceeded by vomiting in two cases. In all cases diagnosis was carried out by CT scan. All patients were treated surgically: in one case raffia alone was performed, in two cases raffia was associated with temporal bipolar oesophageal exclusion, one case went through oesophageal resection with delayed reconstruction of digestive continuity. One patient with severe COBP died from post-surgical sepsis. One fistula after cervical recanalisation and another after raffia of the oesophageal lesion were successfully treated with endoscopy. We suggest that an aggressive surgical approach is the best treatment for this rare and often severe disease.
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PMID:[The Boerhaave syndrome. Personal experience]. 1772 95

In 1958 Caroli described a rare disease with multifocal, segmental and saccular dilation of the large intrahepatic bile ducts which causes stagnation of bile and formation of bile sludge and stones. This results in recurrent abdominal pain, cholangitis and hepatic abscesses. The diagnosis is confirmed with endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) and the purpose of treatment is to restore normal bile flow. Partial resection of the liver has given good results in patient with localized disease. The prognosis is poor despite drainage of bile and 46% of patients die from sepsis, hepatic abscesses, hepatic failure or portal hypertension. There is more than a hundred fold risk of cholangiocarcinoma. We report a case where a male who had a history of recurrent bouts of abdominal pain and pancreatitis was diagnosed with Caroli's disease. He later developed cholangiocarcinoma. Caroli's disease has not, to our knowledge, been reported in Iceland before.
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PMID:[Caroli's disease, case report and review of the literature]. 1782

Angio-immunoblastic T-cell lymphoma (AITL), a rare disease that constitutes 1% to 2% of non-Hodgkin's lymphomas, presents in middle-aged and elderly individuals. This report describes a patient with high-grade fever and lymphadenopathy. An extensive in-hospital work-up that included lymph node biopsy yielded negative results. The patient became asymptomatic for 4 wk, but then symptoms recurred with more pronounced lymphadenopathy. A computed tomography scan documented recurrent diffuse lymphadenopathy. Another lymph node biopsy revealed florid changes that strongly suggested AITL. Flow cytometry confirmed this diagnosis. The patient was treated with hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and achieved complete remission after the first cycle. The patient developed sepsis during the second cycle and expired within 4 mo of diagnosis. The prognosis and natural course of AITL are poor when the classic chemotherapy protocol is administered. When infection has been ruled out, physicians should be wary of aggressive lymphomas that present with diffuse lymphadenopathy and fever.
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PMID:Angio-immunoblastic T-cell lymphoma in evolution: a case report. 1790 Oct 30

Pyomyositis is an acute bacterial infection manifesting as pyemic abscess formation in the skeletal muscles. We examined 8 autopsy cases (seven males, one female; age range 21-75 years) of fatal nontropical pyomyositis to better describe individual case characteristics and pathologic features of this rare disease. The pathogen most frequently involved was Staphylococcus aureus. In most cases, there were several abscesses and multiple sites involved. The trunk, shoulder girdle, and thigh muscles were most frequently affected and involvement of multiple sites was a common finding. In 6 cases, a recent trauma had occurred to the anatomic location where the pyemic abscesses were found. Three deceased were known as intravenous drug abusers. Except for the presence of pyomyositis, liver diseases such as cirrhosis in 3 cases, and a fatty liver in 2 cases were the most frequent autopsy findings. Death was due to sepsis in all cases. Because pyomyositis may develop in association with intravenous catheterization in the clinical setting, the question whether pyomyositis was caused by an infected or improperly placed indwelling intravenous catheter may be of forensic importance in the light of alleged medical malpractice. According to our observations, severe underlying illnesses seem not always necessary for fatal outcome of pyomyositis. Because a detailed dissection of superficial as well as deep skeletal muscles during autopsy is a prerequisite for the diagnosis, the disease may be overlooked when this essential step is not performed.
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PMID:Fatal pyomyositis: a report of 8 autopsy cases. 1852 Apr 79

Acid sphingomyelinase (ASM; E.C. 3.1.4.12) is best known for its involvement in the lysosomal storage disorder Niemann-Pick disease (NPD). Through studies that began by investigating this rare disease, recent findings have uncovered the important role of this enzyme in the initiation of ceramide-mediated signal transduction. This unique function involves translocation of the enzyme from intracellular compartments to the outer leaflet of the cell membrane, where hydrolysis of sphingomyelin into ceramide initiates membrane reorganization and facilitates the formation and coalescence of lipid microdomains. These microdomains are sites of protein-protein interactions that lead to downstream signaling, and perturbation of microdomain formation influences the pathophysiology of many common diseases. The initial observations implicating ASM in this process have come from studies using cells from patients with NPD or from ASM knockout (ASMKO) mice, where the genetic deficiency of this enzymatic activity has been shown to protect these cells and animals from stress-induced and developmental apoptosis. This review will discuss the complex biology of this enzyme in the context of these new findings and its recently reported importance in common human diseases, including cancer, sepsis, cardiovascular, pulmonary, liver, and neurological diseases as well as the potential for using ASM (or ASM inhibitors) as therapeutic agents.
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PMID:The unexpected role of acid sphingomyelinase in cell death and the pathophysiology of common diseases. 1856 38


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