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

Splenic abscess is an unusual disease and may be presented either as a localized area of infection in the spleen or as a part of generalized sepsis. Population-based autopsy studies have established the incidence of splenic abscess at between 0.2-0.7 per cent. An eleven-year retrospective study of cases of splenic abscess treated at Siriraj hospital, a total of 9 cases, is presented. Pseudomonas pseudomallei is the most frequent causative agent, found in one-third of the cases, especially if the patient is thalassemic or a resident in the Northeastern part of the country. Thalassemia is also the leading predisposing condition of this malady with the incidence of 33 per cent. There are some differences in the presenting clinical features in Thai patients compared with those reported in the literature. Splenectomy was performed in all but one who died of leukemia preoperatively. The mortality rate of this disease in this series is 11 per cent and we recommend splenectomy under antibiotic coverage as soon as the diagnosis of splenic abscess has been confirmed.
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PMID:Splenic abscess at Siriraj Hospital, Thailand. 280 52

Splenic dysfunction measured by pitted red cells (pit) was studied in hemoglobinopathies (SS-, SC-, and S beta-type thalassemias and CC-type hemoglobinopathy) in relation to age, in steady state, and during certain significant events. Our experience revealed that the pit count rose with age during steady state in most children with SS disease. A marked increase in pit count was noted in patients with CC disease. The pit count in four patients with S beta+ thalassemia remained normal (i.e., less than 3.5%) at all ages. In children with homozygous SS disease tested at the time of pneumococcal sepsis, the pit count was universally elevated. The pit count was in the normal range in one child with SS disease and osteomyelitis but was elevated in all others. All children had normal pit counts (less than 3.5%) at the onset of acute splenic sequestration crisis, and the counts remained normal during transfusion therapy. No correlation was detected between the pit count and the size of the spleen in patients under 1 year of age.
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PMID:Spleen dysfunction in hemoglobinopathies determined by pitted red cells. 323 12

Newborn screening for sickle cell disease has been recommended as a method of decreasing patient mortality. However, its effectiveness in accomplishing this has not been reliably measured. To help determine the effectiveness, 10 years of experience in newborn screening have been summarized. The effects of early patient enrollment in a comprehensive treatment program on long-term morbidity and mortality are reported. From 1975 to 1985, 84,663 newborns were screened regardless of race or ethnic background. Bart's hemoglobin was present in 5%, hemoglobin AS in 2.6%, and hemoglobin AC in 0.75%. Excluding Bart's, approximately 3.6% of all newborns were carriers for hemoglobinopathy. Sickle cell disease occurred in 1:951 births (58 hemoglobin SS, 25 hemoglobin FSC, three hemoglobin S-beta +-thalassemia, and three hemoglobin S-beta O-thalassemia). In addition, one in every 4,233 newborns had a clinically significant thalassemia syndrome (eight hemoglobin FE, ten hemoglobin F only, two hemoglobin H). Compared with other newborn screening programs in California, (congenital hypothyroidism, 1:3,849; phenylketonuria 1:22,474, galactosemia 1:74,103), hemoglobinopathies are the most prevalent congenital disease. Eighty-one newborns with sickle cell disease were followed for 7.2 years. Patients experienced 513 hospitalizations, including 13 episodes of sepsis with or without meningitis and ten acute sequestration crises. The overall mortality rate for patients with sickle cell anemia diagnosed in the newborn period was 1.8%. In comparison, the clinical course of 64 patients with sickle cell anemia diagnosed after 3 months of age and followed for an average of 9.4 years was analyzed. Five of these patients died. In two of these, sickle cell anemia was diagnosed at the time of the death.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Newborn screening for sickle cell disease: effect on mortality. 336 74

To determine the advantages and complications of splenectomy in the treatment of beta thalassemia, 221 splenectomies for thalassemia performed upon pediatric patients from 1971 to 1982 are evaluated. There were 125 boys and 96 girls with a mean age of 8.2 +/- 2.5 years at the time of the operation. Sixty-one other patients who underwent splenectomy for other diseases served as the controls. Early and late complications after splenectomy are considered with a follow-up study of ten years. Hemoglobin (Hb) value, transfusional quotients and mean of transfused blood previous to surgical treatment are matched with the same parameters evaluated during the follow-up period. The effect of treatment with salicylates and dipyridamole upon the incidence of early complications after operation is analyzed. The postoperative complications in patients with thalassemia were 43.4 versus 3.2 per cent (p less than 0.01) registered in control patients. Late complications occurred with an incidence of 10.7 per cent and were due principally to sepsis. Six patients died of sepsis during the follow-up period, but the mortality rate for sepsis in the patients we studied was significantly lower than that reported by others in 73 instances of splenectomy for beta thalassemia. Blood consumption dropped from 270 +/- 99 to 155 +/- 31 milliliters per kilogram per year postoperatively (p less than 0.01) and Hb levels rose from 9.7 +/- 1.3 to 11.2 +/- 0.7 grams per milliliter. These results suggest that, even though splenectomy for beta thalassemia causes a relevant incidence of complications and fatalities, surgical treatment permits an improvement in the quality of the lives of patients with beta thalassemia and significantly reduces blood consumption. Prophylactic antibiotic therapy can reduce the incidence of sepsis, as was observed in the patients we studied.
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PMID:Indications and results for splenectomy for beta thalassemia in two hundred and twenty-one pediatric patients. 340 27

In a study of the outcome of marrow transplantation in patients with advanced thalassemia, 40 patients with homozygous beta-thalassemia who were 8 to 15 years of age (median, 10) received HLA-identical allogeneic marrow after treatment with busulfan and cyclophosphamide. Twenty-eight of the 40 patients were alive and free of disease 260 to 939 days after transplantation, and 2 patients were alive with thalassemia 372 and 1133 days after transplantation. The actuarial probabilities of survival and of disease-free survival at two years were 75 percent and 69 percent, respectively. Ten patients (25 percent) died. Three died of cardiac failure, interstitial pneumonitis, or septicemia within 14 days of transplantation. Three died of infectious complications associated with acute graft-versus-host disease at 46 to 97 days, and two died of infectious complications of chronic graft-versus-host disease at 249 and 290 days. Two patients had transplant rejection and died with marrow aplasia 115 and 192 days after transplantation. One patient had rejection after four months and while the marrow was aplastic underwent a successful second transplantation; the patient was alive without thalassemia 624 days after the first transplantation. The actuarial probability of grade 2 or higher acute graft-versus-host disease in the 32 patients with initial sustained engraftment was 35 percent. Three patients had chronic graft-versus-host disease, which was fatal in two and still active on day 710 in the third. We conclude that bone marrow transplantation can potentially save patients with advanced thalassemia from an otherwise inexorable progression to death from the complications of blood transfusions. The ultimate outcome in this group of patients must await a longer follow-up.
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PMID:Marrow transplantation in patients with advanced thalassemia. 355 Apr 63

Early symptoms were observed in a representative sample of 166 children with sickle cell-hemoglobin C disease diagnosed at birth. Symptoms were uncommon in the first year of life; in approximately 50% specific symptoms had developed by 5 years, but 22% remained without specific symptoms to 10 years. The age at presentation was significantly earlier in patients with low hemoglobin F levels, but was not influenced by heterozygous alpha-thalassemia-2. Painful crisis was the initial manifestation in 77% of the children; other symptoms included dactylitis (14%) and pneumococcal septicemia and acute splenic sequestration (4% each). The commonest nonspecific symptom was acute chest syndrome. The relatively mild early clinical course of sickle cell-hemoglobin C disease indicates that neonatal diagnosis does not have the same urgency as for homozygous sickle cell disease.
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PMID:Clinical presentation of sickle cell-hemoglobin C disease. 376 Oct 71

Among 1882 splenectomized children with a mean follow-up period of more than 5 years the incidence of sepsis was 3.2% with a mortality of 1.4%. As expected the lowest risk (1.6%) was found in case of trauma. Susceptibility to sepsis increases after splenectomy caused by severe disease. It was highest (9.8%) in case of M. Hodgkin, portal hypertension and thalassemia. The younger the patient the higher the risk of PSI. 55% of the affected patients suffered from sepsis within the first two years, 35% after three up to six years. Vaccination and antibiotic prophylaxis are recommended including information about the risk of sepsis. Preservation of orthotopic splenic tissue, if indicated, should be the surgical consequence in childhood.
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PMID:[Risk of infection and surgical consequences of splenic loss in childhood]. 380 31

From 1981-1982 we performed partial splenectomy in 6 children with beta-thalassaemia major as an alternative to splenectomy to reduce transfusion and to preserve some splenic immune function. In two of our young patients with transfusion requirements of 150 ml/kg/year, where more than 2/3 of the spleen was removed, the number of transfusions was significantly reduced with prolonged intervals, haemoglobin rose with a marked improvement in the general condition, nutrition and growth. There were no major post-operative complications. 2 years' follow-up revealed no infection without sepsis prophylaxis.
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PMID:Partial splenectomy in the treatment of thalassaemia major. 406 Aug 88

Classification of platelet disorders has been based on the platelet count. Addition of a second variable, mean platelet volume (MPV), to the routine blood count allows classification of patients into 9 categories: high, low, or normal MPV, and high, low or normal platelet count. We studied 1,244 adult inpatients. 1,134 had both platelet values normal. 11 patients had high MPV and low platelet count: all had hyperdestructive causes. 15 patients had high MPV and normal platelet count: 12 had heterozygous thalassemia, and three had iron deficiency. Seven patients had high MPV and high platelet count: causes included myeloproliferative disorders, inflammation, iron deficiency, and splenectomy, 25 patients had high platelet counts and normal MPV: the causes were inflammation, infection, sickle cell anemia, iron deficiency, or chronic myelogenous leukemia. 52 patients had an MPV that was inappropriately low for the platelet count (high, normal, or low). All had sepsis, splenomegaly, aplastic anemia, chronic renal failure, or a disease being treated with myelosuppressive drugs. High MPV thus appears correlated with myeloproliferative disease or thalassemia; and low MPV, with cytotoxic drugs or marrow hypoplasia. Addition of MPV to the platelet count allows subtler disorders to be detected (when the platelet count is normal), and allows distinction of the cause of thrombocytopenia.
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PMID:Use of mean platelet volume improves detection of platelet disorders. 407 87

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


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