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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The newborn infant, particularly when premature, has a haemostatic mechanism which may not be entirely capable of withstanding the onslaughts of trauma, infection, asphyxia or other complications of the neonatal period. He is at risk of local or diffuse haemorrhage, which may at times be serious or even life-threatening. The cause of haemorrhage during the newborn period can generally be ascertained by a careful history and brief physical examination directed toward recognition of any predisposing factors or underlying diseases. Screening laboratory tests can usually be correctly interpreted as long as certain laboratory artifacts and physiological peculiarities of the neonatal coagulation mechanism are kept in mind. Diagnosis of and therapy for vitamin K deficiency and haemophilia in the healthy-appearing neonate is generally carried out with little difficulty. The seriously ill neonate with bacterial sepsis, respiratory distress syndrome, or extreme immaturity presents greater problems, for laboratory tests may be more difficult to obtain and interpret and underlying conditions may be untreatable. DIC occurs commonly in such neonates, and transfusion therapy, with or without heparin, is often unsuccessful. A persistent dilemma are those neonates with fatal intravascular haemorrhage, in whom definable haemostatic abnormalities are few and transfusion therapy is futile.
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PMID:Neonatal coagulation: normal physiology and pathophysiology. 35 Apr 67

The iliac hemophilic pseudotumor is a rare complication of hemophilia occurring in 1-2% of patients with Factor VIII or Factor IX deficiency. It is frequently disabling and life threatening. This report presents a comparative study of postoperative results of two cases of hemophilic pseudotumor of ilium. One patient undergoing partial resection showed a favorable postoperative course, whereas the patient with complete resection of the pseudotumor died of postoperative bleeding and sepsis. Studies on the postoperative results of these two cases indicate that careful preoperative consideration of tumor size and degree of infiltration is of the utmost importance in operative management. Early excision of tumors eliminates the possibility of endogenous infection. Even partial resection of huge tumors, leaving the lateral wall intact for compression, can promote recovery of functions.
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PMID:Surgical excision of hemophilic pseudotumor of the ilium. 139 99

Over an average span of one year, we performed a prospective clinical and immunologic evaluation of 30 patients with hemophilia. No patient developed life-threatening opportunistic infection or malignancy; however, the immunologic abnormalities and lymphadenopathy initially present in nine patients (lymphadenopathy group) persisted. In addition, five patients, representing 24% of the initial group without lymphadenopathy, developed generalized lymphadenopathy (converter group). One episode of idiopathic thrombocytopenia (ITP) and one episode of staphylococcal sepsis occurred in this "converter" group; one episode of ITP also occurred in the lymphadenopathy group. Sixteen patients remained asymptomatic. At the time of the follow-up evaluation, those differences in mononuclear cell (MNC) percentages and numbers noted initially among the three hemophiliac groups were no longer present. Natural killer cell function alone or in the presence of biologic response modifiers was not different among hemophiliac and control groups. Before developing lymphadenopathy, the converter group of patients had significantly better lymphocyte mitogenic function than did the other two groups of patients with hemophilia. However, lymphocyte mitogenic responses of all groups of patients with hemophilia significantly deteriorated over the course of the study. The abnormal mitogenic responses noted in these patients was explained in part by higher levels of spontaneous suppressor cell activity in mononuclear cell preparations from patients with hemophilia. We conclude that long-term immunologic studies of this patient population requires both quantitative and qualitative evaluations. Our data show that patients with hemophilia have progressive dysfunction of cell-mediated immunity.
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PMID:A longitudinal immunologic evaluation of hemophiliac patients. 293 Nov 44

Concentric joint space narrowing of the hip is an expected radiographic finding in cases of inflammatory arthritis such as rheumatoid arthritis or sepsis. However, similar joint space narrowing is associated with chronic hemorrhagic conditions that produce hemosiderotic synovitis. Hemosiderotic synovitis results from chronic intra-articular bleeding such as occurs in pigmented villonodular synovitis, generalized bleeding diathesis, synovial hemangioma, and chronic trauma. Five hips in five patients with concentric joint space narrowing not associated with inflammatory arthritis or with hemophilia were reviewed clinically, radiographically, and pathologically. All patients had a hemosiderotic synovitis. The definitive diagnosis of pigmented villonodular synovitis was made pathologically in two cases that demonstrated nodular areas of giant cell proliferation, collagen production, and lipid-laden histiocytes on histologic samples.
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PMID:Concentric joint space narrowing of the hip associated with hemosiderotic synovitis (HS) including pigmented villonodular synovitis (PVNS). 335 35

We report here nine children with AIDS. The risk factors of these patients were hemophilia in one, blood transfusions in four, maternal intravenous drug use in three and paternal AIDS in one. One baby was also of Haitian parentage. The major clinical symptoms included failure to thrive, hepatomegaly, lymphadenopathy, interstitial pneumonia, recurrent bacterial and viral infections and persistent oral thrush. Three infants had chronic recurrent parotitis. Five infants developed opportunistic infections primarily Pneumocystis carinii pneumonia and all five died of bacterial sepsis. None of the infants were lymphopenic but all had reversed T4/T8 ratios and poor in vitro lymphocyte responses to pokeweed mitogens. Although many of the clinical and laboratory features of pediatric and adult AIDS are similar, there are some unique features for pediatric AIDS such as the absence of lymphopenia and the high prevalence of recurrent bacterial infections and sepsis.
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PMID:Acquired immunodeficiency syndrome (AIDS) in infants and children: report of nine cases. 383 Feb 64

Four cases of extraintestinal salmonellosis caused by Salmonella enteritidis were described. Underlying diseases in the three patients were haematological neoplasms (2 splenectomized and 1 with massive leukemic infiltrations of the spleen) and in the fourth haemophilia B: only that patient had a prior symptomatic intestinal infection. Blood cultures for S.ent. were positive in all patients and additionally in that suffering from Hodgkin's disease urine, lymph node and stool cultures also showed S.ent. In the haemophiliac patient culture of suppurated hematoma was positive. All patients recovered from S. sepsis but three then died of their neoplastic diseases. The haemophiliac patient is in a good condition. Various disturbances in the immunological tests were observed.
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PMID:[Extraintestinal salmonellosis in patients with blood diseases caused by Salmonella enteritidis]. 747 35

The risk of acquiring diseases from transfusion of blood and blood products is well recognized and the issue of parvovirus in haemophiliacs is not a new one. We report two patients with haemophilia acquiring iatrogenic parvovirus B19 infection, resulting in life-threatening sepsis in one, and immunocompetent adult. Over the last 10 years there has been great progress in manufacturing safer products with regard to enveloped viruses such as HIV, hepatitis B and C. A recent outbreak across Europe of hepatitis A in haemophiliacs treated with plasma-derived factor VII concentrates has made haemophilic treaters concerned about the known (parvovirus B19 and hepatitis A) and the unknown non-lipid enveloped viruses that may be contained in the clotting factor concentrates, because these are resistant to the existing viral inactivating techniques. The possibility of HIV itself mutating into a non-lipid enveloped virus emphasizes the need to seek and use safer products.
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PMID:Transmission of symptomatic parvovirus B19 infection by clotting factor concentrate. 863 48

Before 1983, septic arthritis was rare in patients with hemophilia. With the advent of human immunodeficiency virus infection in the hemophilia population, many centers noted an increasing incidence of patients with septic arthritis. Fifteen septic joints in 10 patients with severe hemophilia were documented. Eight patients were human immunodeficiency virus positive, 1 was human immunodeficiency virus negative, and 1 was not tested. The diagnosis was delayed in 5 patients because the symptoms are similar to an acute hemarthrosis. An elevated temperature was common. The white blood cell count was elevated in only 1/3 of the infections, being modified by human immunodeficiency virus infection. Associated risk factors included infected angioaccess catheters (2), pneumonia (2), and generalized sepsis (1). All but 1 joint responded to appropriate antibiotics and either repeated aspiration or arthrotomy. However, 6 patients died of acquired immunodeficiency syndrome from 2 to 109 months after infection. Three patients are alive 29, 86, and 96 months, respectively, after infection.
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PMID:Long term evaluation of septic arthritis in hemophilic patients. 865 78

Twenty-five central venous lines (two external 23 subcutaneous ports) were placed in 19 boys with haemophilia A (n = 17) or B (n = 2). The mean age of the boys was 4.9 years (range 0.2-15.3 years). The haemophilia was severe (factor level < 1%) in 18 boys and moderate (factor level 3%) in one. Three boys had circulating inhibitors and three were positive for human immunodeficiency virus (HIV)-1 antibody. Central venous lines were placed to facilitate intermittent factor replacement therapy (n = 6), long-term factor prophylaxis (n = 9), induction of an immune tolerance protocol (n = 2) or therapy for acquired immunodeficiency syndrome (AIDS)-related complications (n = 2). The ports remained in place for 15795 days (mean 687 days, range 11-2059 days). The frequency of port-related sepsis was 48% (11/23 ports in eight boys) or 0.7 port infections per 1000 patient days. Ports were removed from five boys with an unresolved infection (four with Staphylococcus aureus sepsis and one with Pseudomonas sp. sepsis). Other complications requiring port removal included a catheter tip placed too high in the venous system (n = 1), severe persistent pain associated with needle access of the port (n = 1) and a subclavian vein thrombosis (n = 1). Both the benefits and risks of a subcutaneous port should be considered when deciding whether to place this device in a very young child with haemophilia.
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PMID:Central venous access catheters in children with haemophilia. 873 96

The most frequent indication for placement of a central venous access device in hemophiliacs is in very young boys (ages 1-2 years) with severe hemophilia who are started on a program of long-term factor prophylaxis designed to eliminate target joint bleeding and the development of chronic musculoskeletal disease. Although expensive, this strategy is extremely successful. It involves intravenous infusion of 25-40 factor units per kg on alternate days (minimum 3 times a week) for boys with severe hemophilia A, and twice a week for boys with severe hemophilia B. To facilitate this prophylaxis regimen some hemophilia clinics routinely recommend placement of a central venous access device; others, more concerned about associated complications such as sepsis, stress the importance of using peripheral veins wherever possible, with central access devices reserved for occasional, selected cases only. A decision to use such a device should only be made after discussion of the risks/benefits with parents (or guardians) and with patients if of an appropriate age. If such a system is to be used, we recommend that a totally implantable device (Port-A-Cath) be placed because of the lower risk of infection, and because totally implantable devices allow children to take part in activities such as swimming. Important complications include catheter-related sepsis, which may occur in 25% or more of devices over time and, much less frequently, catheter-related deep vein thrombosis.
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PMID:Central venous access devices in children with hemophilia: an update. 935 30


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