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

HIV is efficiently transmitted through transfusion with HIV-infected blood. Accordingly, 203 multitransfused children with thalassemia attending the thalassemia clinic of the Charak Palika Hospital in New Delhi were screened for antibodies to HIV using ELISA and Western blot tests. 8.37% of the sample tested HIV-seropositive (HIV+). These 17 children were joined by 3 others referred from a neighboring state to constitute a group to be matched against 20 HIV-children for the purpose of comparing psychosocial aspects. The control group was matched for age, sex, educational level, and socioeconomic status with mean age 10.8 years ranging over 1-16 years. 4 members of the HIV+ sample were diagnoses as having clinical AIDS according to WHO criteria. The remaining 14 boys and 2 girls were HIV+, but asymptomatic. 25% were of lower class, 63.5% middle class, and 12.5% upper class. Of those with AIDS, 50% were diagnosed in their first year of life and 82% were diagnosed by year 3. Symptoms generally developed after 4-6 months of life. Lymphadenopathy and hepatomegaly tend to be visible at birth, while chronic diarrhea, prolonged fever, oral thrush, recurrent bacterial infections, and hepatosplenomegaly may also be presented. 7.1% of cases aged 2-3 years exhibited rocking and head banging problems worse than did control subjects. Furthermore, 28.5% had temper tantrums and 21.5% ground teeth. These children may have delayed developmental milestones as well as behavioral problems. The small sample size, however, precludes concluding that psychosocial differences exist between those with HIV/AIDS and those with thalassemia major. In fact, behavioral problems in these children were due to child illness and not of HIV-positivity, for children tend to be unaware of HIV/AIDS infections and its consequences. The author recommends that HIV+ children continue to attend school unless they can not control bodily secretions, have uncoverable oozing lesions, have unacceptable behaviors, or if there is extreme possibility of contracting infectious diseases at school. The author also stresses parents' and families' need for long-term medical and psychological care.
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PMID:Psycho-social aspects of HIV infection and AIDS in multiple transfused thalassemic children. 145 60

The case of a young woman from Guadeloupe developing severe megaloblastic anemia is reported. She presented with fever and hemolysis (she had heterozygotic beta-thalassemia) after a six month history of diarrhea. She was shown to have folate deficiency, steatorrhea, vitamin K and B12 malabsorption. She responded well to intravenous folinic acid and tetracyclines. Diagnosis was based on the following criteria: a) no other cause of malabsorption was found despite a comprehensive inquiry, b) the pattern of the illness corresponded to the definition of disease. Three particular aspects are outlined: the acute onset, malabsorption of vitamin B12 requiring prolonged and combined treatment, and the unusual place in which the disease was contracted.
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PMID:[Tropical sprue revealed by severe anemia in a woman from Guadeloupe]. 158 43

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

From 1977 to 1984, Salmonella typhi was isolated from 85% and Salmonella paratyphl A was isolated from 15% of 192 Thai children with enteric fever at Children's Hospital, Bangkok. Children with enteric fever presented with sudden onset of fever and gastrointestinal symptoms. of fever presented with sudden onset Diarrhea occurred in 62% of children with paratyphoid fever and 36% of children with typhoid fever. Rose spots were seen in 15% of patients with typhoid and 7% of patients with paratyphoid fever. There were no deaths. Bronchitis and pneumonia occurring in 11% of patients were the most common complications. Acute hemolysis occurred in 3% of the patients with typhoid fever who had thalassemia or glucose-6-phosphate dehydrogenase deficiency. Chloramphenicol-resistant S typhi, which accounted for 70% of the isolates in 1977, has since 1982 accounted for less than 2% of isolates.
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PMID:Typhoid and paratyphoid fever in 192 hospitalized children in Thailand. 311 88

Dactylitis commonly occurs in patients with homozygous hemoglobin S disease (sickle cell anemia), sickle cell-hemoglobin C disease or sickle cell-beta-thalassemia. A case is reported of dactylitis associated with sickle cell trait, a very rare occurrence. It may be that in this patient the disorder was secondary to severe diarrhea and dehydration.
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PMID:Dactylitis in a child with sickle cell trait. 397 4

A double-blind, randomized, dose-finding, phase II mefloquine trial was carried out in 147 adult male patients suffering from acute, uncomplicated, falciparum malaria and admitted to the Hospital for Tropical Diseases, Bangkok, between January 1980 and April 1981. Mefloquine was administered as a single oral dose of 500, 750, or 1000 mg (base) in the form of the hydrochloride. The clinical and parasitological responses were satisfactory with all three dosage regimens. The cure rates for the 1000-, 750-, and 500-mg doses were 100%, 92.5%, and 95% respectively, over an observation period of 63 days.The side-effects, which were transient and generally mild, included nausea, vomiting, and diarrhoea. No significant changes were noted in haematological or biochemical parameters in any of the three groups. Sinus bradycardia, which started 4-7 days after drug administration and lasted for a few weeks, was seen in 10 patients. It was symptomless and needed no treatment.Acute brain syndrome was observed in one patient on day 21 after receiving a 1000-mg dose of mefloquine.Mefloquine was well tolerated in one case of acute renal failure, in 10 cases of moderately severe malaria with jaundice, in 13 cases with glucose-6-phosphate dehydrogenase deficiency, and in one case of thalassaemia.Mefloquine showed no effect on either gametocytes of Plasmodium falciparum or tissue forms of P. vivax.Mefloquine hydrochloride was found to be an effective drug for the treatment of falciparum malaria and tended to produce a more rapid clinical and parasitological response at the highest tested dose of 1000 mg (base).
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PMID:A phase II clinical trial of mefloquine in patients with chloroquine-resistant falciparum malaria in Thailand. 634 13

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

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.
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PMID:Infection due to Yersinia enterocolitica in a series of patients with beta-thalassemia: incidence and predisposing factors. 986 43

We report three children with tubulointerstitial renal failure following leptospirosis. All had acute nonoliguric renal failure with mild hypocalemia and mild metabolic acidosis. Maximum blood urea nitrogen (BUN) and creatinine were 217 and 7.1 mg/dl, respectively, on the 6th day of disease, and no patient required dialysis. They presented with acute febrile illness and dehydration, and required intravenous fluid supplements. Myalgia, vomiting, and bleeding were found in two children. Abdominal pain, arthralgia, diarrhea, and conjunctival suffusion were found in one child. Only one child, who had an underlying disease of beta-thalassemia/Hb E, had jaundice, hepatosplenomegaly, anemia, and thrombocytopenia. Penicillin treatment was given in one case. All recovered, with normal renal function. The leptospirosis complement fixation test was used to confirm diagnosis. L. batavia was considered the etiologic agent in two of the children.
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PMID:Tubulointerstitial renal failure in childhood leptospirosis. 1053 63

Portal vein thrombosis (PVT) following splenectomy is a potentially life-threatening complication, and the true incidence of PVT in splenectomized patients is unknown. The objective of this study was to determine the incidence of symptomatic PVT after splenectomy. The hospital database was searched to identify cases of PVT associated with splenectomy from January 1990 to May 2002. Six hundred eighty-eight patients underwent splenectomy during this period, 321 of them for hematologic diseases. Eleven of the 688 patients had PVT associated with splenectomy, and the charts of these patients were reviewed. Six patients developed PVT after splenectomy. Five had hematologic diseases. Symptoms were abdominal pain (6), ileus (5), fever (3), or diarrhea (2). Diagnosis was confirmed by computed tomography (CT) (4), duplex ultrasonography (1), and magnetic resonance imaging (1). The indications for splenectomy included hemolytic anemia (3), thalassemia (1), and myelofibrosis (1). One patient had an incidental splenectomy during gastrectomy. There were four laparoscopic and two open splenectomies. The median interval between splenectomy and diagnosis of PVT was 40 days (range, 13-741). One patient died of pulmonary embolism. Five of six patients with postsplenectomy PVT had splenomegaly and hemolysis. We conclude that the risk of PVT is higher in patients with hematologic conditions associated with splenomegaly and hemolysis.
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PMID:Portal vein thrombosis following splenectomy: identification of risk factors. 1462 54


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