The American journal of pediatric hematology/oncology
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Am J Pediatr Hematol Oncol · Jan 1989
Growth in children following irradiation for bone marrow transplantation.
Longitudinal height data from 46 pediatric bone marrow transplant (BMT) patients, including 18 with aplastic anemia (AA), 19 with acute nonlymphoblastic leukemia (ANLL), and 9 with acute lymphoblastic leukemia (ALL), were analyzed to assess growth posttransplantation. Patients were prepared for BMT with high-dose cyclophosphamide followed by 7.5 Gy single-dose irradiation; AA patients received total lymphoid irradiation (TLI), and leukemia patients received total body irradiation (TBI). AA patients demonstrated reduced height posttransplant as reflected in a negative mean standard deviation score. ⋯ Mean growth velocities in the leukemia patients were significantly below median for the 3 years following transplant. With a median follow-up of 4 years, antithymocyte globulin plus steroids in combination with methotrexate as graft vs. host prophylaxis was associated with less severe growth suppression than methotrexate alone, while there were no significant associations between growth during the first 2 years following transplant and prepubertal status at transplant (as defined by age), graft vs. host disease, thyroid or gonadal function, or previous therapies received by the leukemia patients. Children undergoing marrow transplantation, particularly those receiving TBI, are at significant risk of subsequent growth suppression.
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Cerebral mucormycosis is a rare disorder caused by several genera of the family Mucoraceae. The genera Rhizopus, Absidia, and Mucor are the predominant pathogenic groups. Disease caused by these organisms usually complicates an underlying chronic illness, such as diabetes mellitus or malignancy. ⋯ The pulmonary system is the most common site of infection in patients with leukemia. Isolated cerebral mucormycosis not associated with head trauma or intravenous drug abuse is a rare disorder. We report what we believe to be the first successfully treated case of isolated cerebral mucormycosis in a patient with acute lymphocytic leukemia in remission.
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Three cases of right-sided Wilms' tumor with direct intracardiac extension are described. Wilms' tumor is one of the most common intraabdominal tumors of childhood. Primary cardiac tumors that arise from myocardium are extremely rare. ⋯ A thorough cardiovascular examination and a metastatic workup should be done for abdominal masses presumptive of Wilms' tumor. Two-dimensional echocardiography is a noninvasive diagnostic tool, and the information obtained by its use not only allows planning of surgical intervention, chemotherapy, or local radiation therapy, but also is beneficial for long-term follow-up. Aggressive surgery, chemotherapy, and radiation therapy has increased the survival figures of these children.
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Am J Pediatr Hematol Oncol · Jan 1988
Case ReportsIntracranial hemorrhage in congenital deficiency of factor XIII.
We describe a male infant with congenital deficiency of coagulation Factor XIII who presented in the immediate postnatal period with umbilical stump bleeding and suffered a severe intracranial hemorrhage at 2 months of age. Factor XIII, also known as "fibrin-stabilizing factor," is a transpeptidase that produces strong covalent bonds between soluble fibrin monomers formed during coagulation. ⋯ Factor XIII deficiency is associated with a high incidence of life-threatening complications, notably intracranial hemorrhage. In light of the long half-life of this factor and the relatively low risk associated with new Factor XIII concentrates, such as Fibrogammin, prophylactic life-long replacement therapy should be considered for patients with severe Factor XIII deficiency.