Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 15-year-old boy with thalassaemia major treated with desferrioxamine, experienced fever associated with digestive disorders. These symptoms led to the discovery of multiple liver abscesses. Therapy included antibiotics (third generation cephalosporin and aminoglycoside) and desferrioxamine discontinuation. Recovery occurred within one month approximately. Systemic yersiniosis was further confirmed by the finding of antibodies to Yersinia enterocolitica O5. The systemic diffusion of Yersinia is favoured by iron-overload and iron-chelating agents.
 ...
PMID:[Hepatic abscess due to Yersinia in a child with thalassemia]. 839 82

Over 15 years, 14 patients with yersiniosis in two North American comprehensive thalassemia clinics (0.6 cases per 100 patient-years) presented with fever (100%), diarrhea (86%), right-lower-quadrant abdominal pain (71%), bacteremia (57%), a palpable abdominal mass (36%), and pharyngitis (28%). Clinically apparent infection occurred within 10 days of blood transfusion in 57% of patients. Nine patients (64%) had only a modest elevation in serum level of ferritin (< 2,000 micrograms/L). Patients with focal abdominal findings had a higher body iron burden, as estimated by the serum ferritin level, and significant intraabdominal suppurative complications. Two patients were not receiving iron-chelating therapy with deferoxamine; one patient was receiving the experimental chelator deferiprone (L1). Iron-loaded patients with beta-thalassemia are at greatly increased risk for severe yersiniosis, even when their body iron burden (as indicated by the serum ferritin level) is only moderately elevated and they are not receiving iron-chelating therapy with deferoxamine.
 ...
PMID:Infection due to Yersinia enterocolitica in a series of patients with beta-thalassemia: incidence and predisposing factors. 986 43

We report a case of Yersinia enterocolitica sepsis syndrome and the acute respiratory distress syndrome in a chronically transfused adolescent with beta-thalassemia. This manifestation of serious Y. enterocolitica infection has not previously been reported. Dyspnea, hypoxia, and fever were the principal features of the clinical presentation. The acute onset of respiratory symptoms occurred after appendectomy. Chest radiographs revealed frontal bilateral infiltrates and alveolar consolidation to three quadrants. Y. enterocolitica was identified from blood and intraoperative appendix cultures. Although there was no need for mechanical ventilation, a remarkable persistence of clinical and X-ray findings was noted. Therapy with high levels of oxygen, and intravenous amikacin and piperacillin/tazobactam led to a favorable outcome.
 ...
PMID:ARDS in a patient with homozygous beta-thalassemia due to yersiniosis. 1065 28

Patients who are homozygous for thalassemia major are at risk for Yersinia enterocolitica infections. We present a case of a 4-year-old child with intussusception of the terminal ileum whose past medical history was significant for beta-thalassemia. His monthly blood transfusions for this condition may have put him at risk for Y enterocolitica enterocolitis. The pathogenesis of this disease relates to the role of iron as an essential growth factor for Yersinia, and this patient's transfusions left him in an iron-overloaded state, despite treatment with Desferal. Our patient's unusual presentation of intussusception was secondary to the mass effect caused by lymphoid hyperplasia, specifically hypertrophied Peyer's patches in the ileum caused by Y enterocolitica infection. To our knowledge, this is the first such case of intussusception caused by Yersinia to be reported.
 ...
PMID:Intussusception due to Yersinia enterocolitica enterocolitis in a patient with beta-thalassemia. 1169 9

We report a case of transfusion-mediated Yersinia enterocolitica septicemia in a 43-y-old woman with homozygous beta-thalassemia. Two h after transfusion of 3 units of red blood cells the patient suffered high-grade fever and shaking chills. Y. enterocolitica serotype O3 grew in blood cultures. Prolonged treatment with i.v. ceftriaxone plus ciprofloxacin led to a favorable outcome. Transfusion-associated Y. enterocolitica septicemia has not previously been reported in an adult beta-thalassemic patient from the Mediterranean area. Our report is particularly important, because of the high incidence of chronically transfused thalassemic patients in Mediterranean countries.
 ...
PMID:Transfusion-mediated Yersinia enterocolitica septicemia in an adult patient with beta-thalassemia. 1176 Jan 71

OBJECTIVE: To evaluate the prevalence of class-specific antibodies (G, A, M) to Yersinia enterocolitica plasmid-encoded outer proteins (Yops), in a closely followed multitransfused population of patients with thalassemia. METHODS: Sera from 408 beta-thalassemic patients and 386 healthy blood donors used as controls were analyzed with the enzyme-linked immunosorbent assay (ELISA) for IgG, IgA and IgM antibodies to yersinia outer proteins. The Yop antigen for the ELISA was prepared using a plasmid-bearing wild-type strain of Y. enterocolitica of serotype O:8. RESULTS: Anti-Yop IgG antibodies were detected in 84 out of 408 beta-thalassemic patients (20.6%) compared with only eight out of 386 (2.1%) healthy blood donors. None of the sera of either group was positive for anti-Yop IgA or IgM antibodies. On evaluating patients with registered clinical and laboratory signs of a previous yersinia infection in the period from 1978 to 1996, we found that those with a positive agglutination test for Y. enterocolitica infection at the time of manifestation showed a higher rate of persisting IgG seropositivity to Yops than those with positive culture and clinical signs only. A significant percentage (9.49%) of the seropositive patients had no registered data of a past Y. enterocolitica infection. There was remarkable persistence of anti-Yop IgG antibodies in the thalassemic population, even in patients infected during the early years of our study period (1978--80). CONCLUSIONS: The results suggest that the determination of class-specific antibodies to Yops, which are specific antigens for the pathogenic yersiniae (Y. enterocolitica, Y. pseudotuberculosis and Y. pestis), in addition to its usefulness in the diagnosis of infection, will be a very sensitive and specific index for epidemiologic studies.
 ...
PMID:Prevalence of yersinia plasmid-encoded outer protein (Yop) class-specific antibodies in multitransfused Greek patients with thalassemic syndromes. 1185 20

Infections are major complications and constitute the second most common cause of mortality and a main cause of morbidity in patients with thalassaemia, a group of genetic disorders of haemoglobin synthesis characterised by a disturbance of globin chain production. Thalassaemias are among the most common genetic disorders in the world. Predisposing factors for infections in thalassaemic patients include severe anaemia, iron overload, splenectomy, and a range of immune abnormalities. Major causative organisms of bacterial infections in thalassaemic patients are Klebsiella spp in Asia and Yersinia enterocolitica in western countries. Transfusion-associated viral infections (especially hepatitis C) can lead to liver cirrhosis and hepatocellular carcinoma. A unique and challenging infection detected in Asian patients is pythiosis, caused by a fungus-like organism, the mortality rate of which is very high. Because the prognosis for thalassaemia has much improved, with many patients surviving to the fifth decade of life in developed countries, it is mandatory to reduce mortality by recognising and presumptively treating infections in these patients as quickly as possible.
 ...
PMID:Infections and thalassaemia. 1655 47

A 49 years old Vietnamese male with a history of thalassemia, presented with gastrointestinal symptoms and signs of hemolysis. He was diagnosed with yersinia enterocolitis. Yersinia is a gram-negative rod that most frequently occurs in children especially during the winter months. In the current case, the bone marrow biopsy showed hemophagocytosis along with positive cultures for Yersinia. The microorganism likely triggered hemophagocytosis. This syndrome, also known as, hemophagocytic lymphohistiocytosis, is defined by fever for more than 7 d, cytopenia of two or more cell lines, hemophagocytosis, hepatitis, serum ferritin greater than 500, jaundice, lymphadenopathy, and hepatosplenomegaly. This disorder can be either familial or secondary to a strong immunologic activation. Both have an overwhelming activation of T-cells and macrophages.
...
PMID:An ironic case of liver infections: Yersinia enterocolitis in the setting of thalassemia. 2411 30


<< Previous 1 2