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3.
Pediatrics ; 101(5): E10, 1998 May.
Article in English | MEDLINE | ID: mdl-9565443

ABSTRACT

An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, approximately 140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy.


Subject(s)
Hamartoma/complications , Splenic Diseases/complications , Adult , Child , Diagnosis, Differential , Female , Hamartoma/pathology , Humans , Spleen/pathology , Splenic Diseases/pathology , Splenomegaly/etiology , Thrombocytopenia/etiology
5.
Hum Hered ; 38(4): 228-32, 1988.
Article in English | MEDLINE | ID: mdl-3169797

ABSTRACT

Six individuals in three families with a history of suxamethonium sensitivity have been found to have genotype E1kE1s. The biochemical data for the recognition of this genotype have been analysed and the mean values compared with similar parameters for the usual phenotype. Individuals with genotype E1kE1s will be sensitive to suxamethonium.


Subject(s)
Alleles , Cholinesterases/genetics , Genotype , Adult , Aged , Child, Preschool , Cholinesterases/blood , Female , Genetic Variation , Humans , Male , Pedigree , Phenotype , Succinylcholine/adverse effects
6.
Anaesthesia ; 41(8): 841-3, 1986 Aug.
Article in English | MEDLINE | ID: mdl-3752466

ABSTRACT

The plasma cholinesterase status of 22 people resident in the same small town was investigated. A high incidence of the atypical cholinesterase gene was found.


Subject(s)
Cholinesterases/genetics , Aged , Drug Hypersensitivity/genetics , Female , Genotype , Humans , Male , Northern Ireland , Space-Time Clustering , Succinylcholine/adverse effects
7.
Vox Sang ; 51(2): 96-101, 1986.
Article in English | MEDLINE | ID: mdl-2430366

ABSTRACT

A case of intravascular hemolytic transfusion reaction without detectable antibodies occurring in a 55-year-old male is reported. Specificity for the C antigen in the Rh system was demonstrated by technetium-99m red cell survival studies. A cell-mediated mechanism of hemolysis was suspected and investigated. Previously reported cases are reviewed and discussed. The entity of intravascular hemolytic transfusion reaction associated with minimal symptoms and no detectable antibodies deserves further investigation.


Subject(s)
Erythrocyte Aggregation/etiology , Erythrocyte Transfusion , Transfusion Reaction , Antigens, Surface , Epitopes , Erythrocyte Aging , Erythrocytes/immunology , Hemoglobinuria/blood , Humans , Immunity, Cellular , Isoantibodies/analysis , Lymphocyte Activation , Male , Middle Aged , Rh-Hr Blood-Group System/immunology , Technetium
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