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

In an effort to attain earlier diagnoses in children with hemophagocytic lymphohistiocytosis (HLH), the International Histiocyte Society has now broadened their diagnostic criteria to no longer differentiate primary (HLH) and secondary hemophagocytic lymphohistiocytosis (SHLH). Five of the following eight diagnostic criteria needed to be met: 1) fever, 2) cytopenia of two lines, 3) hypertriglyceridemia and/or hypofibrinogenemia, 4) hyperferritinemia (>500 microg/L), 5) hemophagocytosis, 6) elevated soluble interleukin-2 receptor (CD25), 7) decreased natural killer-cell activity, and 8) splenomegaly can also commonly be found in patients with sepsis, systemic inflammatory response syndrome (SIRS), multiorgan dysfunction syndrome (MODS), and macrophage activation syndrome (MAS). Nevertheless, the therapeutic options for these are radically different. Chemotherapy and bone marrow transplant have been used for treatment of HLH/SHLH, whereas antibiotics and supportive treatment are used in severe sepsis/SIRS and MODS. MAS is treated with limited immune suppression. Outcomes are also different, SHLH has a mortality rate around 50%, whereas pediatric septic shock and MODS have a mortality of 10.3% and 18%, respectively, and severe sepsis in previously healthy children has a mortality rate of 2%. MAS has a mortality rate between 8% and 22%. Because SHLH and severe sepsis/SIRS/MODS/MAS share clinical and laboratory inflammatory phenotypes, we recommend extreme caution when considering applying HLH therapies to children with sepsis/SIRS/MODS/MAS. HLH therapies are clearly warranted for children with HLH; however, a quantitative functional estimate of cytotoxic lymphocyte function may be a more precise approach to define the overlap of these conditions, better identify these processes, and develop novel therapeutic protocols that may lead to improved treatments and outcomes.
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PMID:Secondary hemophagocytic lymphohistiocytosis and severe sepsis/ systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation. 1932 10

Marginal zone macrophages in the murine spleen play an important role in the capture of blood-borne pathogens and are viewed as an essential component of host defense against the development of pneumococcal sepsis. However, we and others have previously described the loss of marginal zone macrophages associated with the splenomegaly that follows a variety of viral and protozoal infections; this finding raises the question of whether these infected mice would become more susceptible to secondary pneumococcal infection. Contrary to expectations, we found that mice lacking marginal zone macrophages resulting from Leishmania donovani infection have increased resistance to Streptococcus pneumoniae type 3 and do not develop sepsis. Using biophotonic imaging, we observed that pneumococci are rapidly trapped in the spleens of L. donovani-infected mice. By selective depletion studies using clodronate liposomes, depleting monoclonal antibodies specific for Ly6C/G and Ly6G, and CD11c-DTR mice, we show that the enhanced early resistance in L. donovani-infected mice is entirely due to the activity of SIGNR1(-) red pulp macrophages. Our data demonstrate, therefore, that the normal requirement for SIGNR1(+) marginal zone macrophages to protect against a primary pneumococcal infection can, under conditions of splenomegaly, be readily compensated for by activated red pulp macrophages.
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PMID:SIGNR1-negative red pulp macrophages protect against acute streptococcal sepsis after Leishmania donovani-induced loss of marginal zone macrophages. 1964 16

Recent studies suggest that patients with common variable immunodeficiency (CVID) and low numbers of switched memory B cells have lower IgG levels and higher rates of autoimmune disease, splenomegaly, and granulomatous disease; however, no prior literature has focused exclusively on pediatric cases. We examined the relationship between switched memory B cells and clinical and immunologic manifestations of CVID in a pediatric population. Forty-five patients were evaluated. Patients were categorized as Group I (<5 switched memory B cells/ml, n = 24) or Group II (> or =5 switched memory B cells/mL, n = 21). CD3(+) T-cell counts and CD19(+) B-cell levels were lower among Group I patients. Only those in Group I had meningitis, sepsis, bronchiectasis, granulomatous lung disease, autoimmune cytopenias, or hematologic malignancies. Segregation of pediatric patients into high risk (Group I) and average risk (Group II) may assist in targeting surveillance appropriately.
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PMID:Pediatric common variable immunodeficiency: immunologic and phenotypic associations with switched memory B cells. 2145 41

Vertical transmission of Plasmodium falciparum is under-recognized and usually associated with asymptomatic low-level parasitemia at birth. We report symptomatic congenital malaria presenting as a neonatal sepsis syndrome. The presence at birth of a high asexual parasitemia, gametocytemia, and splenomegaly indicated in utero rather than intrapartum transmission. The neonate was successfully treated with intravenous artesunate followed by oral dihydroartemisinin-piperaquine, without apparent adverse effects.
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PMID:Severe congenital malaria acquired in utero. 2034 99

Thrombocytopenia is usually acquired. The laboratory artefact of pseudothrombocytopenia should always be excluded. Bone marrow insufficiency with impaired platelet production results from infiltrating tumor cells or from a myelodsplastic syndrome. In patients with splenomegaly, platelets are trapped by the spleen. An increased platelet turnover is caused by activation of the clotting cascade, e.g. due to sepsis or malignancy. Platelet binding antibodies cause thrombocytopenia by increased platelet clearance. Important differential diagnoses in patients with severe thrombocytopenia are: acute leukemia, thrombotic thrombocytopenic purpura, autoimmune thrombocytopenia and drug-dependent thrombocytopenia. Multifactorial causes are thrombocytopenia associated with pregnancy, chronic alcohol abuse, and liver cirrhosis. Treatment should focus on the underlying disease. In regard to low platelet counts only clinical bleeding and not platelet count numbers should be treated.
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PMID:[Differential diagnosis and treatment of thrombocytopenia]. 2094 73

Autoimmune neutropenia, caused by neutrophil-specific autoantibodies is a common phenomenon in autoimmune disorders such as Felty's syndrome and systemic lupus erythematosus. Felty's syndrome is associated with neutropenia and splenomegaly in seropositive rheumatoid arthritis which can be severe and with recurrent bacterial infections. Neutropenia is also common in systemic lupus erythematosus and it is included in the current systemic lupus classification criteria. The pathobiology of the autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus is complex, and it could be a major cause of morbidity and mortality due to increased risk of sepsis. Treatment should be individualized on the basis of patient's clinical situation, and prevention or treatment of the infection. Recombinant human granulocyte colony-stimulating factor is a safe and effective therapeutic modality in management of autoimmune neutropenia associated with Felty's syndrome and systemic lupus erythematosus, which stimulates neutrophil production. There is a slight increased risk of exacerbation of the underlying autoimmune disorder, and recombinant human granulocyte colony-stimulating factor dose and frequency should be adjusted at the lowest effective dose.
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PMID:Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus. 2125 89

We present the case of a patient who presented with evidence of pneumonia, sepsis and anaemia but no significant abdominal signs. A routine abdominal ultrasound scan revealed evidence of spontaneous splenic rupture. He underwent splenectomy but passed away subsequently from respiratory complications. The many associations of spontaneous splenic rupture are discussed. The diagnosis should be considered in any patient presenting with shock and non-specific abdominal signs and in those with pre-existing conditions known to cause splenomegaly.
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PMID:Spontaneous splenic rupture in a patient with pneumonia and sepsis. 2165 3

Familial hemophagocytic lymphohistiocytosis is a rare and inherited disease that affects both males and females equally and is most often fatal if not treated. Diagnosis is challenging because it mimics severe sepsis by demonstrating hepatomegaly, splenomegaly, persistent fever, central nervous system involvement, and cytopenias. The etiologies of hemophagocytic lymphohistiocytosis have been well established through the identification of 3 causative genetic mutations. Therapies for hemophagocytic lymphohistiocytosis focus on restoring health by diminishing the disease sequelae with a goal of hematopoietic stem cell transplant, the only known curative therapy for hemophagocytic lymphohistiocytosis. Current research is being conducted to identify other causative genetic mutations and newer, more effective treatment modalities.
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PMID:Familial hemophagocytic lymphohistiocytosis in the neonate. 2173 Aug 97

There are few studies regarding the clinical presentation of visceral leishmaniasis (VL) in children. The aim of this study was to investigate the clinical manifestations, major complications and causes of death in children with VL. A retrospective study was performed with pediatric patients (< or = 14 years old) with a diagnosis of VL in Fortaleza, state of Ceara, in Northeast Brazil. A total of 120 patients were included. The mean age was 5 +/- 3.9 years, and 53.4% were male. The main clinical manifestations at admission were: fever (94.2%), splenomegaly (94.2%), hepatomegaly (82.5%), anorexia (55%), malaise (47.5%), cough (41.6%), abdominal pain (27.5%), vomiting (25.5%), and diarrhea (16.6%). Acute kidney injury was found in 25% of the patients. The main complication during hospital stay was pulmonary infection, found in 27.5% (n = 33), leading to sepsis in 3 cases. Glucantime was the drug of choice in 90% (n = 108) of the cases, amphotericin B in 7.5% (n = 9) and AmBisome in 2.5% (n = 3). Death occurred in 4 cases (3.3%) due to sepsis (3 cases) and hemorrhagic complications (1 case). Visceral leishmaniasis is a frequent infection among children in our region. The main complications were pulmonary infection and acute kidney injury related to antiparasitic therapy, along with sepsis and hemorrhage.
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PMID:Visceral leishmaniasis in children: a cohort of 120 patients in a metropolitan city of Brazil. 2185 52

A 42-year-old white woman, who was a general practitioner referral to the medical team, presented with a 3-day history of left upper quadrant pain; an urgent private ultrasound scan had showed splenomegaly. She was initially admitted with sepsis without an obvious cause but with a differential diagnosis of a haematological malignancy. Her admission blood tests showed a mildly reduced white cell count and low platelets. Her symptoms progressed and she developed right upper quadrant pain. Her blood counts deteriorated showing a disseminated intravascular coagulation (DIC) picture and mildly deranged liver function tests. Blood films were non-diagnostic. A CT scan of the abdomen/pelvis showed splenomegaly and also hepatomegaly and ascites, not seen in her initial ultrasound scan. Multiple cultures of blood/urine/ascites and infective serology were unremarkable.She was transferred to a larger tertiary centre under the care of the surgeons with presumed abdominal sepsis and underwent an open laparotomy, which showed a big firm liver and spleen but no obvious cause for sepsis. The infectious disease team were unable to find a cause, and haematology became involved to investigate the possibility of a haematological malignancy. The patient underwent two bone marrow biopsies, a percutaneous liver biopsy and had flow cytometry of her ascitic fluid, which revealed the diagnosis of a natural killer cell leukaemia. After some slight improvement on steroids, the patient was given cyclophosphamide, doxorubicin, vincristine, prednisone, rituximab (CHOP-R) chemotherapy. The patient had an initial response to chemotherapy, with reduction in ascitic volume and hepatosplenomegaly, and normalisation of her coagulation. This was accompanied by an overall improvement in her physical condition. She had a second cycle of CHOP-R, but unfortunately approximately 2 weeks after that, she deteriorated rapidly. She was too weak for salvage chemotherapy, so she was put on comfort care. She died peacefully.
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PMID:Natural killer cell leukaemia. 2188 53


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