Acute febrile neutrophilic dermatosis (Sweet's syndrome) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia.
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Biomedical subjects
Publications and source records attributed to A Shende.
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A 12-year-old girl with acute lymphoblastic leukemia (ALL) had two types of acquired cytogenetic abnormalities in her pretreatment peripheral blood and bone marrow: hyperdiploidy due to tetrasomy 8, 10, and 21; and, in the hyperdiploid cells, a shift from heterozygosity to homozygosity for a polymorphic variant on chromosome 15. Both abnormalities disappeared after chemotherapy, when the patient entered clinical remission. It has recently been found that shifts to homozygosity occur in retinoblastoma and Wilms' tumor. Our observation extends this finding to leukemia and indicates that such shifts may have general importance in tumorigenesis.
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Chronic hemolytic anemia in a 7-year-old Chinese boy was found to be associated with decreased activity of erythrocyte hexokinase (approximately 20% of appropriate control values). Alterations in kinetics, stability, pH optimum, or electrophoretic patterns could not be demonstrated with proband hexokinase, but subtle differences between maternal and paternal hexokinase characteristics suggested that two separate mutant isozymes were involved and that the proband was heterozygous for each. Comparisons with previously reported cases of hexokinase deficiency demonstrate the broad genetic heterogeneity that characterizes this disorder.
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The authors describe a case of histiocytosis X of the parietal lobe presenting as a space-occupying lesion on a computerized tomography scan of the brain. The clinical, radiographic, and therapeutic aspects of the case are discussed. A brief review of the literature is presented.
Two children who presented initially with a lymphoid malignancy were noted to develop recurrences with myeloid features late in the course of their disease. In both cases, evidence of lymphoid differentiation was present in the myelogenous cells that were Ph1 chromosome negative. The first patient had acute myelogenous leukemia and developed a recurrence with morphologic features of acute myelogenous leukemia. Terminal transferase was present in the myelogenous blasts. The second patient initially had a diffuse lymphoblastic non-Hodgkin lymphoma. During the course of her illness she developed a myeloproliferative disorder characterized by basophilic meningitis, splenomegaly, and hypereosinophilia. Lymphocyte T-cell (E-rosette) markers were present on the eosinophils. These observations lend further support to the hypothesis of varying lymphoid and myeloid differentiation in certain cases of leukemia.
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Seventeen children with sickle cell anemia received 40 partial exchange transfusions for serious complications of sickle cell anemia, and preoperatively to reduce the risk of anesthesia. Each patient received two partial exchange transfusions at 24-hour intervals and all patients tolerated the exchange transfusions well. The mean hematocrit level rose from 22.9% before the exchange transfusion to 40.6% at the end of the second exchange. The mean sickle hemoglobin level decreased from 93.8% before the exchange to 28.0% after the second exchange. Patients with acute lung syndrome had remarkable improvement in clinical symptoms and PaO2 levels, and those with acute liver crisis had substantial reduction in serum bilirubin levels. All the other patients showed marked clinical improvement following the exchange transfusions.
Pulmonary blastoma is a rare tumor of the lung. Although it has been stated that this is predominantly a tumor of adults, a review of the 42 cases published to date reveals that it is a disease which occurs with some frequency in children. The tumor is relatively benign compared to pulmonary carcinosarcoma. Of the eight cases previously described in children, three are known to have survived and to be free of disease 10 months, 3 and 8 years after surgical resection. We report two additional cases of pulmonary blastoma in children followed for 34 and 26 months without any evidence of recurrence or metastases.
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Torsion of the pedicle of a visceroptosed spleen, a rare condition, was diagnosed preoperatively in a 4-year-old girl, with the aid of history, physical examination, blood smear findings, splenic scans with technetium Tc 99m sulfur colloid, and selective angiography. A splenectomy was performed, and the child made an uneventful recovery. This case report illustrates some of the diagnostic and therapeutic considerations pertaining to torsion of the spleen.
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Twenty-five cases of hodgkin's Disease (15 males and 10 females) aged 5 to 17 years were studied from April 1970 to July 1976 (75 month period). Histology revealed that 2 had lymphocytic predominance, 12 had nodular sclerosis, and 11 had mixed cellularity. Pathologic staging revealed that 3 were IA, 1 IB, 5 IIA, 4IIB, 6IIIA, and 6 IIIB. Laparotomy altered the staging in 12 patients (9 were staging up and 3 down). All but 2 patients received extended field radiation, and 5 had recurrence of disease and were treated with combination chemotherapy. Twenty-three are alive without evidence of disease (21-75 months), and the 2 deaths were not due to Hodgkin's Disease but to hemobilia (postliver biopsy) and penumococcal septicemia, purpura fulminans, and disseminated intravascular coagulation (14 months postsplenectomy). Other complications included 2 patients with intestinal obstruction, 1 with postoperative subphrenic abscess, and 1 with streptococcal septicemia and polyarthritis. Nineteen patients received continuous penicillin prophylaxis postoperatively and the 2 with serious infections were amongst the 6 who had not received penicillin or whose penicillin had been discontinued at the time of infection. It is concluded that laparotomy and splenectomy in children is essential for accurate staging but carries significant risk, and continuous penicillin prophylaxis is recommended.
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