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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Our experience at the Ramathibodi Hospital with 20 infants and children who had Aeromonas septicemia is reviewed. Their ages were from 1 day to 14 years. Eighteen patients had underlying diseases: leukemia, 5; aplastic anemia, 4; cirrhosis, 2; thalassemia/hemoglobinopathy, 3; renal failure, 1; ileal perforation, 1; marasmus, 1; and cavernous hemangioma with thrombocytopenia, 1. Blood cultures yielded Aeromonas hydrophila in all patients, and four patients had polymicrobial bacteremia. Fifteen episodes of septicemia were community-acquired and five were hospital-acquired. The clinical manifestations of these patients were similar to septicemia due to other Gram-negative enteric bacilli. Two patients each had ecthyma gangrenosum, necrotizing fasciitis and meningitis. Antibiotic treatment included penicillins, cephalosporins, aminoglycosides and sulfamethoxazole-trimethoprim. The overall case fatality rate was 50%; eight of the nine patients with acute leukemia or aplastic anemia died. With the exception of one child the blood cultures were sterile in all patients before death. Aeromonas septicemia is an uncommon but severe infection which occurs predominantly in compromised hosts.
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PMID:Aeromonas septicemia in infants and children. 672 2

The Authors describe a rare case of Yersinia Enterocolitica (Y.E.) infection in a child affected by thalassemia. The onset of the disease was that of an acute enteritis with diarrhea, fever, vomiting and abdominal pain which subsequently evolved in a picture consistent with an acute appendicitis. Laparotomy was then performed and showed a marked suppurative mesenterial lymphadenitis with mild appendicular inflammation and Y.E. infection was suspected. Culture from lymphonodes confirmed the presence of Y.E. sensitive to tobramicin and CTM. The use of these chemiotherapic agents has been followed by a rapid clinical improvement. Our recent experience could suggest some practical considerations: 1) Culture of Y.E. should be routinely performed in all children affected by acute gastroenteritis and particularly in those, above 5 years of age, in which the infection can simulate acute appendicitis. 2) Special attention should be carried out in children affected by thalassemia who can easily present more serious disease often complicated by septicemia. 3) Therapy depends on the form and severity of the disease and should be always guided by in vitro sensitivity test because of the possibility of resistence of Y.E. against the most frequently used antibiotics in septicemia.
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PMID:[Yersinia enterocolitica infection in thalassemia. Report of one case (author's transl)]. 697 42

Because of the risk of overwhelming sepsis following splenectomy, and since it is possible to reduce the splenic function while preserving the spleen, the treatment of various hematologic disorders by splenic artery ligation has been attempted in 11 children, among whom 3 under 5 years of age. 3 patients had thalassemia, 4 had hereditary spherocytosis and 4 others suffered from secondary hypersplenism. One patient died of overlooked splenic necrosis. The other patients are well, with an average follow-up of 19 mo. The aim of this study is to find out whether the desarterization produces the expected results and is well tolerated and whether the desarterialized spleen still preserves its ability to protect the host against infection. The satisfactory treatment of hereditary spherocytosis and secondary hypersplenism would indicate that splenic artery ligation offers an alternative to splenectomy, especially in children under 5 year old.
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PMID:[Treatment of hematologic disorders in children by splenic artery ligation (author's transl)]. 707 16

In clinical studies, frequent hepatic dysfunction associated with crises in sickle cell disease has been noted, but whether irreversible morphologic changes arise from these transient episodes is uncertain. We studied 70 patients with sickle cell disease (57 SS, 12 SC and one S-thalassemia (S-thal) hemoglobin) autopsied at The Johns Hopkins Hospital. They ranged in age from five months to 75 years (average 21 years) and 35 (50 percent) were female, In 64 patients (91 percent), livers were enlarged and had distention of Kupffer cells with phagocytized sickled red cells; this was massive in 10. In 19 patients (27 percent) the sinusoids were markedly distended with sickled red cells and appeared obstructed. Focal parenchymal necroses were present in 24 patients (34 percent) and were explained in 12, eight by cardiac dysfunction and four by sepsis. Reparative changes, portal fibrosis and regenerative nodules were each found in 14 patients (20 percent), only one of whom had a known history of viral hepatitis despite the frequency of transfusions. Cirrhosis of unknown cause was present in seven patients and cardiac cirrhosis in one. Cirrhosis with hemochromatosis was present in three patients and 30 others had parenchymal iron accumulation. Thus, unexplained hepatic necroses, portal fibrosis, regenerative nodules and cirrhosis were frequently encountered in these patients. This spectrum of liver disease appears to be best understood as a consequence of recurrent vascular obstruction, necrosis and repair arising as a component of sickle cell disease.
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PMID:The liver in sickle cell disease. A clinicopathologic study of 70 patients. 744 49

Butyrate analogues have been shown to increase fetal hemoglobin (HbF) production in vitro and in vivo. Sodium phenylbutyrate (SPB), an oral agent used to treat individuals with urea-cycle disorders, has been shown to increase HbF in nonanemic individuals and in individuals with sickle cell disease. We have treated eleven patients with homozygous beta thalassemia (three transfusion dependent) and one sickle-beta-thalassemia patient with 20 g/d (forty 500-mg tablets) of SPB for 41 to 460 days. All patients showed an increase in the percent of F reticulocytes associated with treatment, but only four patients responded by increasing their Hb levels by greater than 1 g/dL (mean increase, 2.1 g/dL; range, 1.2 to 2.8 g/dL). None of the transfusion-dependent thalassemia subjects responded. Increase in Hb was associated with an increase in red blood cell number (mean increase, 0.62 x 10(12)/L), and mean corpuscular volume (mean increase, 6 fL). Changes in percent HbF, absolute HbF levels, or alpha- to non-alpha-globin ratios as measured by levels of mRNA and globin protein in peripheral blood did not correlate with response to treatment. Response to treatment was not associated with the type of beta-globin mutation, but baseline erythropoietin levels of greater than 120 mU/mL was seen in all responders and only two of eight nonresponders to SPB. Compliance with treatment was greater than 90% as measured by pill counts. Side effects of the drug included weight gain and/or edema caused by increase salt load in 2/12, transient epigastric discomfort in 7/12, and abnormal body odor in 3/12 subjects. Two splenectomized patients who were not on prophylactic antibiotics developed sepsis while on treatment. We conclude that SPB increases Hb in some patients with thalassemia, but the precise mechanism of action is unknown.
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PMID:Oral sodium phenylbutyrate therapy in homozygous beta thalassemia: a clinical trial. 752 72

We determined prospectively during a 12-month period the incidence, clinical characteristics and outcome of Yersinia enterocolitica infection in 144 thalassemic patients (mean age, 12.8 years) and compared them with 100 controls (mean age, 12.1 years). Symptomatic Y. enterocolitica infection occurred in 14 (10%) of the thalassemic patients and in 2 (2%) controls (P = 0.017). Of the 14 thalassemic patients 5 (36%) had septicemia and 9 (64%) had focal infection (enteritis in 8 and tonsillitis in 1). One control patient had acute enteritis and the other had tonsillitis. All isolates from these patients belonged to pathogenic phenotypes of Y. enterocolitica. Pending culture results symptomatic thalassemic patients discontinued treatment with deferoxamine and were treated with intravenous antibiotic therapy. Patients with the ultimate diagnosis of focal Y. enterocolitica infection continued treatment with intramuscular ceftriaxone or intravenous trimethoprim/sulfamethoxazole (TMP/SMX) for 7 days, whereas those with septicemia continued treatment with intravenous TMP/SMX for 14 days. The outcome was favorable in all 14 thalassemic patients. We conclude that Y. enterocolitica is a significant cause of morbidity in our patients with thalassemia and that prompt antibiotic therapy might prevent life-threatening conditions as well as a complicated course with long term sequelae.
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PMID:Prospective study of Yersinia enterocolitica infection in thalassemic patients. 756 85

Vibrio vulnificus infection, which is a rare and fatal disease, can be categorized clinically as either primary septicemia or wound infection. The clinical presentation of patients with primary septicemia can vary from fever alone to a more severe illness including high-grade bullous lesions, hypotension, and shock. Wound infection typically results from either injury to the skin in a marine environment or contact of a preexisting wound with sea water. We reported eight cases with Vibrio vulnificus infection in Chang gung Memorial Hospital and reviewed ten other cases previously reported with details in Taiwan. Fourteen patients presented with primary septicemia, and four with wound infection. Thirteen patients had alcoholism or chronic liver disease, two had peptic ulcer disease, one was steroids abuser, and one patient had thalassemia and chronic liver disease. Overall mortality was 55.6% (ten patients). Patients with hypotension within 48 hours of admission had higher mortality than normotensive patients (77% vs. 0%, P = 0.007). Patients with chronic liver disease or liver cirrhosis also had tendency to a higher mortality than not (64% vs. 25%, P = 0.274). Chronic liver diseases and liver cirrhosis are common disease in Taiwan. They take a high risk for Vibrio vulnificus infection. Clinician should keep in mind of this potentially fatal infection in these patients reporting a history of recent raw oyster consumption and presented with sepsis and characterized skin lesions. Prompt empirical antibiotics treatment and aggressive surgical treatment may be lifesaving for this acute and fatal disease.
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PMID:Vibrio vulnificus infection--report of 8 cases and review of cases in Taiwan. 785 Jun 49

Twenty-four patients with homozygous beta-thalassaemia who had been splenectomised and currently on treatment were studied retrospectively. They were divided into two groups. Group A: who had splenectomy prior to commencement of any regular blood transfusion. The mean haemoglobin for this group rose from 5.5 gm/dl pre-splenectomy to 7.7 gm/dl post splenectomy (p < 0.001). Group B: who were on regular blood transfusion when they had their splenectomy and the mean blood transfusion requirement dropped from 317 ml/kg/yr to 230 ml/kg/yr of packed red cells following splenectomy (p < 0.001). Three patients who were on regular blood transfusion and desferrioxamine developed Yersinia enterocolitica infection. They presented with fever and signs of an acute abdomen. At laparotomy, 2 of the patients had acute appendicitis. All 3 appendices grew Yersinia enterocolitica and one patient also had a Yersinia enterocolitica septicaemia. If a patient develops fever and enteritis, desferrioxamine should be stopped temporarily and cotrimoxazole started as prophylaxis against systemic Yersiniosis. No cases of pneumoccocal sepsis was reported.
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PMID:Homozygous beta-thalassaemia: a review of patients who had splenectomy at the Royal Alexandra Hospital for Children, Sydney. 800 82

Hydroxyurea (HU) is one of several agents that have been shown to enhance hemoglobin (Hb) F levels in patients with sickle cell disease and may be useful as a therapy for beta-globinopathies. However, limited information exists on the effects of HU in patients with thalassemia. Accordingly, we examined the hematologic effects of orally administered HU in 13 patients with beta-thalassemia/Hb E, including four patients who had been splenectomized. These patients were treated with escalating doses (final range, 10 to 20 mg/kg/d) for 5 months and were observed in the outpatient hematology clinic every 2 to 4 weeks. Complete blood counts including reticulocyte counts, amounts of Hb E and Hb F, G gamma:A gamma and alpha:non-alpha globin biosynthetic ratios were evaluated before and during treatment. Almost all patients responded with an average increase of 33% in Hb F levels, from a mean (+/- SD) of 42% +/- 11% to 56% +/- 8% (P < .0001), and a reciprocal decline in the percentage of Hb E from 59% +/- 9% to 49% +/- 8% (P < .001). Reticulocytosis was decreased from a mean (+/- SD) of 18.0% +/- 15.6% to 11.7% +/- 9.1% (P < .05); there was also a slight (10%) but statistically significant increase in hemoglobin levels and an improved balance in alpha:non-alpha globin chains ratios. The side effects were minimal in most patients, although these patients tended to tolerate a lower dose of HU before significant myelosuppression than has been our previous experience in sickle cell disease. One splenectomized patient died of sepsis during the trial. We conclude that increased Hb F production in beta-thalassemia/Hb E patients, with an improvement in the alpha:non-alpha globin ratios and, probably, the effectiveness of erythropoiesis, can be achieved using HU. Longer trials of HU in this population, including at other doses and in combination with other agents, appear warranted.
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PMID:Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease. 856 58

Long term effects of BMT in thalassemia were monitored in 33 patients transplanted between 1987 and 1995 and compared with 155 patients matched for age and treated during the same period with conventional therapy (CT). The incidence of fulminant sepsis and growth impairment was significantly higher in transplanted patients, whereas the occurrence of hypothyroidism, hypogonadism, and cardiopathy was higher in CT patients. For diabetes, liver disease, and severe infections, the differences were not statistically significant. After BMT we performed monthly erythrocytaferesis for iron removal in 23 (70%) patients, obtaining a complete normalization of iron stores in 91% of cases; among untreated patients, 60% had evidence of iron up to 8.3 years after BMT. Protection against poliovirus, tetanus, diphtheria, and hepatitis B has been lost in 74%, 47%, 78%, and 44%, respectively. After BMT a careful follow-up is needed to monitor and treat late transplant-related and thalassemia-related complications.
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PMID:Late effects of bone marrow transplantation for thalassemia. 966 51


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