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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with homozygous beta-thalassemia are at increased risk of serious infections. Yersinia enterocolitica is an organism with a predilection for these and other iron-overloaded patients. Three young adult patients with beta-thalassemia who were chronically transfused and developed yersiniosis are reported. Iron overload and desferrioxamine use are predisposing factors, as supported by clinical, animal, and in vitro data. Iron excess both immunologically compromises the host and greatly enhances yersinial growth. Desferrioxamine may make host iron even more bioavailable to Yersinia. Recognition of this association and unusual manifestations in these patients such as an appendicitis-like syndrome may direct clinicians to earlier antiyersinial therapy and temporary cessation of chelation.
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PMID:Yersinia infections in patients with homozygous beta-thalassemia associated with iron overload and its treatment. 152 3

Despite the frequency of gastrointestinal disease caused by Yersinia enterocolitica, intestinal perforation is a rare complication of this infection and to date only eight cases have been reported in the English literature. We describe a case of this unusual condition in a 17-year-old male patient with thalassemia intermedia requiring transfusion, who was also taking deferoxamine. The severity of the clinical picture is probably due to the increased virulence of Yersinia enterocolitica in the presence of deferoxamine and iron.
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PMID:Yersinia enterocolitica infection with ileal perforation associated with iron overload and deferoxamine therapy. 186 9

We report two cases of severe Yersinia enterocolitica infection in children with homozygous thalassemia. One patient had septicemia and the other had mesenteric adenitis. Two factors can enhance the infectivity of Yersinia enterocolitica in children with thalassemia: iron overload and deferoxamine therapy. Laparotomy and cefotaxime-netilmicin therapy were successful in the patient with mesenteric adenitis. In the patient with septicemia, cefotaxime-netilmicin, then doxycycline-netilmicin failed, and recovery was finally achieved under rifampicin-netilmicin. Because of the possibility of septicemic dissemination secondary to digestive Yersinia enterocolitica infection in children with thalassemia, we advocate immediate discontinuation of deferoxamine and prescription of oral antimicrobial therapy (trimethoprim-sulfamethoxazole for instance) in every thalassemic patient with febrile diarrhea.
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PMID:[Yersinia enterocolitica infections and thalassemia major in children]. 266 80

A study of 3 personal and 32 published cases showed that Yersinia septicaemia in subjects under 21 presents as digestive disorders with fever and occurs in one half of the cases in thalassaemia patients. Among the factors which may be blamed for this predisposition the most important are excessive tissue iron levels and treatment with chelating agents. Iron overload is present in 70% of patients with Yersinia septicaemia. The risk of infection in such subjects requires appropriate therapeutic measures.
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PMID:[Roles of iron overload and chelating treatment in Yersinia infectious complicated by major thalassemia]. 295 32

Yersinia enterocolitis with peritonitis and septicaemia developed in a 4-year-old child with thalassemia intermedia and iron overload. It is likely that the illness was exacerbated by continued administration of desferrioxamine, a bacterial siderophore, which facilitated systemic spread of Yersinia. It is recommended that Yersinia enterocolitis be considered in children with iron overload and that desferrioxamine be discontinued and prophylactic antibiotics be administered while Yersinia is sought.
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PMID:Yersinia enterocolitis in iron overload. 343 Feb 72

Antibodies against Yersinia were found in 12 of 50 patients with hemosiderosis, in 11 of 47 patients with thalassemia major and in one of three patients with Blackfan-Diamond anemia. All patients were treated with subcutaneous continuous deferoxamin-infusions. A systemic yersiniosis occurred in seven patients, all with homozygous beta-thalassemia, in five during and in two before treatment with deferoxamin. Hemosiderosis and infusions with deferoxamin seem to increase the risk of yersinia septicemia.
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PMID:[Frequent occurrence of Yersinia infection in hemosiderosis]. 408 85

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

A septicaemic course of infection due to Yersinia enterocolitica developed in three children, two Italian girls with thalassemia (serotype 03) and a previously healthy boy (serotype 09). There were marked gastrointestinal symptoms. The boy required two transfusions for acute haemolysis. Because it may take a serious course, the possibility of this organism as the causative agent should be considered in the differential diagnosis of severe infections, especially in patients with chronic debilitating disease. The death rate is about 30%.
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PMID:[Infection due to Yersinia enterocolitica in healthy and chronically ill children (author's transl)]. 743 64

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

The characteristic spectrum of infections in patients with aplastic anemia, chronic neutropenic diseases, sickle cell disease, thalassemia, and other hemoglobinopathies are described. The major risk factor for infection in patients with bone marrow failure is the degree of neutropenia and monocytopenia. In patients with aplastic anemia, invasive fungal infections emerge as the major causes of mortality. Life-threatening infections are rare in patients with chronic neutropenic diseases; however, necrotizing enterocolitis due to Clostridium species may be an exception. Bacterial infections, predominantly with encapsulated bacteria, are the most common cause of death in patients with sickle cell disease, especially those who are younger than 5 years of age. Patients with thalassemia and other hemoglobinopathies are particularly susceptible to life-threatening infections with Yersinia enterocolitica as a result of iron overload or of the chelating therapy with desferrioxamine.
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PMID:Approach to management of fever and infection in patients with primary bone marrow failure and hemoglobinopathies. 835 59


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