Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
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Five fatal cases of Japanese patients with type 1 Gaucher disease were studied. The causes of death included hemorrhage secondary to esophageal varices (two cases), respiratory distress (one case), hepatic failure (one case) and postoperative sepsis (one case). All of the patients had previous splenectomies, four patients had bone involvement and hepatic cirrhosis. The identified Gaucher genotypes were 1448C/1213G, 1603T/1603T, 1448C/1390G, and 1213G/1213G. The prognosis of type 1 Gaucher disease is generally good. We propose that patients with a similar clinical course and genotype to those presented in the present study should receive prompt comprehensive treatment. Patients with the 1213G mutation, pulmonary and liver involvement and a previous splenectomy should be considered as candidates for early vigorous treatment.
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PMID:Clinical and genetic studies of five fatal cases of Japanese Gaucher disease type 1. 874 12

Portal hypertension hemorrhage (PHH) due to esophageal varices (EV) rupture in nearly 80% of cases, or gastric varices (GV) in the remaining 20%, account for one-fifth of the GI tract bleedings in a general hospital. Its frequency, but more importantly, its magnitude, that causes up to one-third of the cirrhotic casualties, deserves constant update in its management. Diverse inherent patient factors influence the course of any PHH, i.e., a) remaining liver function, which is determinant; b) variceal size; c) sepsis, and d) alcohol intake. Mortality due to PHH is 27% during the first week, 41% within 6 weeks and 75% by one year of follow-up after the index hemorrhage. Time of intervention is then of utmost importance. All these key circumstances determine the ultimate course of the bleeding event, in many cases to a greater degree than the opportunity and quality of the specific treatment itself. This diversity of influential factors also jeopardizes adequate patient randomization in trials designed to compare treatment modalities. During the last decade, EV sclerosis, when compared to conventional medical treatment (non-beta blockers), has proved useful to stop active bleeding in 71 vs. 31% of cases, decreasing early and late recurrence from 70 to 40%, and direct bleeding-related mortality from 24 to 9%, even when global mortality remains around 14% per year. Disappointing as it seems, remaining liver function is the determinant issue, but a biased underestimation factor may also play a role, due to greater surgical rescue of patients in the medical branch compared to EV sclerosis, 6 vs. 28%. Minor morbidity in 14% of sclerosis treatment has given way to endoscopic ligation with similar results and less morbidity. Prophylactic EV sclerosis was prohibited by prospective controlled trials, which demonstrated significant increase in bleeding and mortality, even though there might be a subgroup of patients with large varices or endoscopic prognostic signs of bleeding that decrease by 10% their incidence expected 35%/year bleeding. GV bleeding remains a challenge; where cyanoacrylate may be needed to improve immediate control and prevent recurrence of PHH. These patients, as well as those failures to endoscopic treatment are candidates for intrahepatic portosystemic shunt (TIPS), although long-lasting control is achieved, in most cases, by liver transplant.
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PMID:[Therapeutic endoscopy in portal hypertension. When, how and how far in 1998]. 1006 21

The prognosis of GI bleeding depends upon many factors. Patients should be evaluated carefully for risk factors. To avoid complications from GI bleeding, triage should be performed promptly after patient presentation. The history and physical examination should emphasize analysis of risk factors for severe GI bleeding and mortality. Factors that increase the morbidity and mortality include: age greater than 60 years; underlying comorbidity such as pulmonary diseases, liver diseases, renal diseases, encephalopathy, or cancer; physiologic stress from major surgery, trauma, or sepsis; coexisting disease in three organ systems; low hematocrit; melena or hematochezia; and prolonged prothrombin time. Hospitalized patients who require more than five units of packed erythrocytes transfusion or who develop hypotension or hypovolemic shock are more likely to need surgery. Patients with a high APACHE II score, the presence of esophageal varices, active bleeding, or other endoscopic stigmata of recent hemorrhage are more likely to rebleed and undergo surgery. The proliferation of multivariable prognostic scales, as described herein, provides ample evidence that the goal of developing a single comprehensive multivariable scale to accurately assess severity of disease and to determine prognosis of GI bleeding is still not achieved. Yet significant progress has occurred in this field, leading to the hope of developing a universally applicable multivariable scale.
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PMID:Clinical scoring systems for determining the prognosis of gastrointestinal bleeding. 1083 89

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.
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PMID:Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. 1132 44

The liver plays a central role in the clotting process, and acute and chronic liver diseases are invariably associated with coagulation disorders due to multiple causes: decreased synthesis of clotting and inhibitor factors, decreased clearance of activated factors, quantitative and qualitative platelet defects, hyperfibrinolysis, and accelerated intravascular coagulation. The bleeding tendency accounts for increased risk of morbidity and mortality in patients with liver disease undergoing diagnostic or therapeutic invasive procedures. Peculiar coagulation disorders are prevalent in patients with acute fatty liver of pregnancy or undergoing liver transplantation. Emerging evidence shows that sepsis further impairs hemostasis in patients with liver cirrhosis bleeding from esophageal varices. Thrombotic events, even if rare in cirrhotic patients, occur mainly in the portal and mesenteric veins. The therapeutic approach to coagulative disorders is also discussed.
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PMID:Coagulation disorders in liver disease. 1192 81

We report a case of late perforation of the thoracic esophagus with an esophagopleural fistula after endoscopic sclerotherapy for esophageal varices in a Child-Pugh B9 cirrhotic patient. The existence of a thoracic empyema without diffuse mediastinitis allowed management of the fistula by percutaneous drainage-lavage and antibiotic therapy with subsequent closure of the esophageal wall defect and recovery from sepsis. This observation indicates that minimally invasive management of an esophageal perforation complicated by an esophago-pleural fistula is possible in highly selected patients.
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PMID:[Drainage-lavage and closure of a late esophageal perforation with esophagopleural fistula and encysted pleural effusion after endoscopic injection sclerotherapy for varices]. 1473 50

This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3-5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.
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PMID:Extrahepatic portal hypertension in children: observations on three surgical procedures. 1535 94

We report that haemostasis was obtained by the use of SEMS in two patients with bleeding oesophageal varices which failed conventional therapy (vasoactive drugs, antibiotics, endoscopy, and Sengstaken balloon tamponade). One patient subsequently died of sepsis; the other was treated with TIPS and was discharged.
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PMID:[Therapy of continuously bleeding oesophageal varices by self expanding metal stents]. 1787 18

Portal vein thrombosis is a rare but well-known complication after abdominal surgery. To our knowledge, there have been no reports about this complication after cardiopulmonary bypass surgery. This can probably be explained by the variety of clinical pictures and difficulties in the establishment of a diagnosis of portal vein thrombosis. Among the possible trigger factors, bacteremia, that is usually caused by Bacteroides fragilis or by Escherichia coli, has been assessed. In this case, several blood culture specimens and fluid from abdominal paracentesis consistently grew coagulase-negative staphylococci which have been proved to be one of the most common pathogens in postoperative infection after cardiac surgery. The patient received clopidogrel before and after coronary artery bypass grafting. We speculate that in this clinical situation associated with coagulase-negative staphylococcal bacteremia, clopidogrel resistance may play an important role. The natural history of portal vein thrombosis in this case is complicated by massive fatal gastrointestinal bleeding from rupture of the esophageal varices. Emergency endoscopic therapy with esophageal variceal ligation, injection of a sclerosing solution and using of vasoconstrictive agents helped only in the first episode of bleeding. Portal vein thrombosis after coronary artery bypass grafting is a serious complication. The clinical picture of portal vein thrombosis may vary greatly and the presence of this condition should be suspected when faced with abdominal pain with gastrointestinal bleeding of unknown origin and sepsis.
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PMID:Portal vein thrombosis associated with coronary artery bypass surgery. 1821 99

Patients with impaired hepatic functional reserve when submitted to surgeries may have high rates of morbidity and mortality. Pre-existing liver disease should be detected without need for invasive methods. Clinical history and physical examination provide important clues. Laboratory liver function is not necessary unless there are changes in history or physical examination. Liver disease has many effects on surgery and anesthesia. A decrease in oxygenation and increased risk of liver dysfunction can be caused by anesthesia, hemorrhage, hypoxemia, hypotension, vasoactive drugs or the patient's position on the operating table during and after surgery. Emergency surgery is a major predictor of poor prognosis as well as sepsis and reoperations. The nature of liver disease, severity and type of surgery to be performed should take into account for a correct preoperative preparation. Some actions must be taken at preoperative to decrease chances of complications in patients with liver disease undergoing surgical procedures. Very close attention should be given to coagulopathy, encephalopathy, ascites, renal and pulmonary dysfunction, spontaneous bacterial peritonitis and esophageal varices. Patients with Child-Pugh score C and MELD>15 should not undergo elective surgery. Patients with Child-Pugh score B and MELD 10 to 15 may undergo minor surgical procedures with care in cases of extreme necessity. Patients with Child-Pugh score A and MELD<10 may be submitted to elective surgery.
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PMID:[Pre-operative care for liver disease patients]. 2049 99


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