Acute idiopathic thrombocytopenic purpura--management in childhood.
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Adrenal Cortex Hormones↗
Biomedical subjects
G R Buchanan
Publications and source records attributed to G R Buchanan.
Safety of early hospital discharge of selected febrile children and adolescents with cancer with prolonged neutropenia.
PROBLEM: The safety of early hospital discharge (i.e., before the absolute neutrophil count [ANC] exceeds 500 cell/mm3) of febrile neutropenic children and adolescents with cancer who had experienced prolonged neutropenia (i.e., for more than 7 days) following admission has not been studied. METHOD OF STUDY: Three hundred and thirty nine consecutive admissions of children and adolescents with cancer for management of febrile neutropenia were reviewed. Early discharge criteria included absence of fever for 24 hours prior to discharge, sterile blood cultures for 24 hours, evidence of bone marrow recovery defined as a sustained increase in platelet count and ANC or absolute phagocyte count (APC), and control of local infection if present. Children hospitalized with febrile neutropenia who remained neutropenic for more than 7 days were analyzed to assess their outcomes following discharge it they had met criteria for early hospital discharge. RESULTS: Thirty-three patients in whom neutropenia had persisted for more than 7 days were discharged before attaining an ANC greater than 500/mm3 when they met the early discharge criteria. Only two children (6%) required readmission for recurrent fever, a rate which was not different from that of patients discharged after a more transient episode of neutropenia (2 of 33 vs. 3 of 121, P = 0.3). Both patients who were readmitted had a source of local infection which worsened despite oral antibiotics. Both patients appeared clinically well at the time of readmission and had sterile cultures during their second hospitalization with resolution of local infection. CONCLUSION: This study confirms that low-risk criteria used to select children with cancer for discharge before complete resolution of neutropenia can be safely applied to those patients whose neutropenia lasted more than 7 days following admission.
Adolescent↗
Comparison of laparoscopic and open splenectomy in children with hematologic disorders.
OBJECTIVE: To compare laparoscopic and traditional open splenectomy in children with nonmalignant hematologic disorders. STUDY DESIGN: Retrospective review of 36 consecutive nonrandomized splenectomies (16 laparoscopic and 20 open) performed for hematologic disorders at a single pediatric institution during the past 3 years. The two-sided Mann-Whitney U test for non-parametric variables was used for statistical analysis. RESULTS: An open procedure was performed on 20 patients (mean age, 9.7 years), five of whom had a concomitant cholecystectomy. A laparoscopic splenectomy was performed on 16 children (mean age, 10.3 years), seven of whom had a concomitant cholecystectomy. The mean anesthesia and operative times were longer in the laparoscopic than in the open group (p < 0.001). However, the mean number of hours of postoperative analgesia was less in the laparoscopic group (p < 0.005). Patients who had laparoscopic splenectomy were also discharged home earlier (p < 0.01) and resumed a regular diet sooner. Mean operating room charges were higher in the laparoscopic group (p < 0.001), but total hospitalization costs were not significantly different. Postoperative complication rates were similar. The hematologic response was comparable. CONCLUSIONS: laparoscopic splenectomy is feasible and safe in children with hematologic disorders. Although it currently requires more operative time than the open approach, it is superior with regard to duration of postoperative analgesia, duration of hospital stay, and recovery of bowel function.
Adolescent↗
Acute splenic complications in children with sickle cell-hemoglobin C disease.
OBJECTIVE: To determine the frequency and severity of acute splenic complications in children and adolescents with sickle cell (SC) hemoglobin C disease. METHODS: The medical records of 271 patients with SC disease seen at our center were reviewed to evaluate the incidence and severity of acute complications involving the spleen. RESULTS: Sixteen (6%) children had acute splenic complications. Thirteen (5%) had 16 episodes of acute splenic sequestration (ASSC), with the initial event occurring at a mean age of 8.9 years (range, 2 to 17 years). Splenomegaly had been noted before the initial event in 6 (46%) of the 13 cases, and 3 (23%) had a history of painful splenic infarction. Two young children (aged 4 and 6 years) had a hemoglobin value less than 2 gm/dl, one without history of splenic enlargement. Three (23%) children had a second episode of ASSC. Three additional patients had a history of acute painful splenic infarction, two of whom also had splenic hemorrhage. Eight (3%) of the 271 children required splenectomy (1 after the initial episode of ASSC, 3 after a second episode of ASSC, 2 as a result of pain accompanying chronic infarction and ASSC, and 2 because of splenic hemorrhage). No deaths resulted from ASSC. CONCLUSIONS: We conclude that (1) acute splenic complications in children and adolescents with SC disease are relatively uncommon, (2) most episodes of ASSC occur in preadolescents, (3) ASSC can be life threatening, even in younger children, and (4) prior splenomegaly is not a good predictor of ASSC. Thus it is vital that the parents of all children with SC disease be instructed to palpate their child's spleen regularly.
Adolescent↗
Autosomal dominantly inherited Diamond-Blackfan anemia resulting in nonimmune hydrops.
BACKGROUND: Autosomal dominant inheritance of Diamond-Blackfan syndrome has been considered an uncommon occurrence. The onset of anemia is characteristically within the first year of life, with 10% of cases presenting at birth. Hydrops fetalis has been reported rarely. CASES: Two women with Diamond-Blackfan anemia had uncomplicated pregnancies without important exacerbation of their anemia. Each delivered an edematous infant affected with Diamond-Blackfan anemia, both of whom required immediate transfusions. One infant is currently 7 years old with transfusion-dependent Diamond-Blackfan anemia. The second infant died 2 days after birth; autopsy showed severe erythroid hypoplasia with absence of extramedullary hematopoiesis and placental villous edema. CONCLUSION: Diamond-Blackfan anemia may result in severe fetal anemia requiring transfusion. Among women with this disorder, a greater percentage than previously suspected are at risk for having an infant with substantial anemia in both the fetal and perinatal periods. Because the penetrance of the disorder is variable, pregnant women with a history of Diamond-Blackfan anemia should be considered at risk.
Adult↗
Early discharge of low-risk febrile neutropenic children and adolescents with cancer.
During the past decade, a relatively lower-risk patient population of febrile neutropenic children with cancer (over one-half of all these patients) has been identified. These patients can be safely discharged from the hospital before their absolute neutrophil count (ANC) exceeds 500/mm3. To evaluate the practice of early discharge of these patients, 580 consecutive episodes of chemotherapy-induced febrile neutropenia in 253 children and adolescents with cancer between June 1992 and May 1995 were reviewed. Three hundred thirty episodes ended in discharge before the patient's ANC was > 500/mm3. Patients were characterized as being at relatively lower risk if they had sterile blood cultures, were afebrile for > 24 hours, appeared well, and were thought to have evidence of marrow recovery. Of the 330 episodes, only 21 (6%) were associated with admission for recurrent fever during the subsequent 7 days. In retrospect, in only six of these 21 cases of readmission (or 2% of 330 episodes) was there evidence of bone marrow recovery, and none of the blood cultures were positive during the subsequent hospitalization. All patients who met low-risk criteria fared well during their second hospitalization. This early discharge strategy was safe and resulted in substantial cost savings.
Adolescent↗
Low risk of hepatitis C transmission by blood products.
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Blood Transfusion↗
Serum aminotransferase elevation during and following treatment of childhood acute lymphoblastic leukemia.
PURPOSE: The clinical significance of methotrexate (MTX)-induced hepatic toxicity in children with acute lymphoblastic leukemia (ALL) is poorly defined. Therefore, we conducted a study to determine whether intensive MTX therapy could be safely delivered despite isolated serum ALT elevations in children with ALL. PATIENTS AND METHODS: A total of 243 children with B-precursor ALL were treated with extended pulses of oral divided-dose MTX (dMTX). Serum ALT levels were measured approximately every 7 weeks during therapy, as well as after its cessation. By protocol design, treatment was continued without modification in the presence of ALT elevations if there was no other evidence of liver dysfunction. RESULTS: Of 239 assessable patients, 159 (66.5%) had an ALT level > or = 180 IU/L during therapy and 28 patients (17.6%) had one or more values > or = 720 IU/L. After the completion of therapy, only 17 of 104 assessable patients have had one or more elevated ALT value. Eight of these 17 patients (47%) are hepatitis C virus (HCV)-seropositive. The remaining nine children had subsequent normal or near normal ALT values, and none have clinical evidence of liver disease. CONCLUSION: Our data show that MTX can be safely delivered without dose modification in patients with isolated ALT elevations and that continued therapy does not lead to clinically apparent liver disease. ALT elevations are not a reliable predictor of the presence or extent of hepatic injury, and persistently increased ALT values following the completion of ALL therapy are rare in the absence of HCV infection. Continued MTX therapy allows for increased dose-intensity and may improve outcome in children with ALL.
Adolescent↗
Serotype-specific immunoglobulin G antibody responses to pneumococcal polysaccharide vaccine in children with sickle cell anemia: effects of continued penicillin prophylaxis.
OBJECTIVES: (1) To determine serotype-specific IgG antibody responses to reimmunization with pneumococcal polysaccharide vaccine at age 5 years in children with sickle cell anemia and (2) to determine whether continued penicillin prophylaxis had any adverse effects on these responses. STUDY DESIGN: Children with sickle cell anemia, who had been treated with prophylactic penicillin for at least 2 years before their fifth birthday, were randomly selected at age 5 years to continue penicillin prophylaxis or to receive placebo treatment. These children had been immunized once or twice in early childhood with pneumococcal polysaccharide vaccine and were reimmunized at the time of randomization. RESULTS: Serotype-specific IgG antibody responses to reimmunization varied according to pneumococcal serotype but in general were mediocre or poor; the poorest response was to serotype 6B. The antibody responses were similar in subjects with continued penicillin prophylaxis or placebo treatment, and in subjects who received one or two pneumococcal vaccinations before reimmunization. The occurrence of pneumococcal bacteremia was associated with low IgG antibody concentrations to the infecting serotype. CONCLUSIONS: Reimmunization of children with sickle cell anemia who received pneumococcal polysaccharide vaccine at age 5 years induces limited production of serotype-specific IgG antibodies, regardless of previous pneumococcal vaccine history. Continued penicillin prophylaxis does not interfere with serotype-specific IgG antibody responses to reimmunization.
Adult↗
Low-dose recombinant human granulocyte colony-stimulating factor therapy in children with symptomatic chronic idiopathic neutropenia.
OBJECTIVES: To prospectively define the lowest possible doses of recombinant human granulocyte colony-stimulating factor (rhG-CSF) that would benefit selected children with chronic idiopathic neutropenia whose disease was severe enough to interfere appreciably with quality of life. STUDY DESIGN: The efficacy of low-dose rhG-CSF therapy was investigated in six children with symptomatic chronic idiopathic neutropenia. All patients received rhG-CSF, 5 micrograms/kg subcutaneously, as a single daily dose until an absolute neutrophil count (ANC) above 1.5 x 10(9)/L was observed. The rhG-CSF dosage interval and amount were then increased and decreased, respectively, in an alternating fashion until the lowest rhG-CSF dose that would maintain the ANC above 1.0 x 10(9)/L (1000/mm3) was reached. RESULTS: Although the minimal dose requirements varied, all patients were able to maintain a mean ANC > 1.0 x 10(9)/L during a mean follow-up period of 14 months at doses ranging from 1.0 microgram/kg once weekly to 5.0 micrograms/kg every other day. Administration of rhG-CSF resulted in resolution of all preexisting chronic infections, reduction in the frequency of new infectious episodes, and discontinuation of prophylactic antibiotics. In all patients the ANC decreased to pretreatment values when further reduction or discontinuation of rhG-CSF therapy was attempted. By identifying the minimal effective dose in each patient, we were able to reduce the treatment cost by a mean of 81% compared with daily dosage at 5 micrograms/kg. CONCLUSIONS: Recombinant human granulocyte colony-stimulating factor therapy at low doses (< or = 5 micrograms/kg) every 2 to 7 days to symptomatic children with chronic idiopathic neutropenia is effective and no more costly than supportive treatment with antibiotics.
Child↗
Relationship between Fc receptor IIA polymorphism and infection in children with sickle cell disease.
OBJECTIVE: Despite penicillin prophylaxis and vaccination, infection with encapsulated organisms remains a leading cause of morbidity and death in children with sickle cell disease. The role of Fc receptors in the clearance of encapsulated organisms is well documented. The His(H)-Arg(R) polymorphism at amino acid 131 of the Fc gamma RIIA receptor alters binding affinity for human IgG2 and influences infection with encapsulated organisms in children without sickle cell disease. We hypothesized that the genotype for high-affinity human IgG2 binding (H/H131) is underrepresented in children with sickle cell disease who had encapsulated organism infection. DESIGN: We studied 60 black children with sickle cell disease from four participating centers who had a history of encapsulated organism infection. Genomic DNA from peripheral blood was subjected to amplification by polymerase chain reaction and to sequence analysis for identification of the Fc gamma RIIA genotype, and the genotype distribution was then compared with our data from ethnically matched control subjects. RESULTS: Contrary to our hypothesis, the H/H131 genotype was overrepresented in all individuals (p = 0.046) and in particular in the 11 individuals with a history of Haemophilus influenzae type b infection (64% H/H131, 27% H/R131, 9% R/R131; p = 0.002), in comparison with ethnically matched control subjects (14% H/H131, 60% H/R131, 26% R/R131). In the 51 individuals with a history of Streptococcus pneumoniae infection, the genotype distribution was not statistically significantly different from that of the control population. CONCLUSIONS: The H/H131 Fc gamma RIIA genotype is overrepresented in black children with sickle cell disease and a history of H. influenzae type b infection but not in those with S. pneumoniae infection.
Adolescent↗
A pilot study of outpatient management of febrile neutropenic children with cancer at low risk of bacteremia.
Febrile neutropenic children with cancer were eligible for outpatient management with intravenous ceftriaxone therapy if they displayed selected low-risk criteria. Nineteen children were enrolled. All patients had sterile blood cultures, and only one of them was hospitalized because of persistent fever. This pilot study suggests that selected children with febrile neutropenia might be successfully managed without hospitalization.
Adolescent↗
Current controversies in the management of idiopathic thrombocytopenic purpura during childhood.
Both acute and chronic ITP in children are generally benign conditions. Few patients develop serious complications or long-term sequelae. Therefore, most patients require little or no specific therapy. IVIG or high-dose steroids may benefit some patients who have evidence of clinical bleeding, and splenectomy may be of value in patients with chronic ITP whose lives are altered by low platelet counts or bleeding. It is difficult to predict which patients are at risk for the development of ICH, and severe hemorrhage is not always curtailed by prior or concomitant therapy. The decision to treat a child with ITP should be based on the entire clinical picture rather than on the platelet count alone.
Adolescent↗
Interferon-alpha-2b treatment of chronic hepatitis C in children with hemophilia.
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Adolescent↗
AIDS-associated non-Hodgkin's lymphomas as primary and secondary AIDS diagnoses in hemophiliacs. Hemophilia Malignancy Study Group.
We studied the characteristics and temporal trends of AIDS- associated non-Hodgkin's lymphoma (AIDS-NHL) in individuals with hemophilia. Prospective data were collected on 33 HIV-positive hemophiliacs with AIDS-NHL enrolled in the Hemophilia Malignancy Study (HMS), of whom 21 had primary and 12 had secondary or subsequent AIDS-defining illnesses, and analyzed for frequency and temporal trends. As compared with primary AIDS- NHL, secondary AIDS-NHL occurred at an older mean age, 37 versus 29 years (p = 0.12); at a lower mean CD4 count, 46 versus 154 (p = 0.07); after a longer period of immunosuppression (CD4 < 200/microl), 41 versus 16 months (p = 0.03); and with shorter median survival, 2 versus 7 months (p = 0.09). The presence of EBV in tumor tissue was associated with shorter survival, 1 versus 7 months (p = 0.17). Between 1981 and 1988 and 1989 and 1994, the proportion of primary AIDS diagnoses that were AIDS-NHL changed minimally, 4.6 versus 6.1%, whereas there were significant decreases in Pneumocystis carinii pneumonia (PCP, p = 0.02) and wasting (p = 0.07), and an increase in Candida (p = 0.004). These findings confirm that an increasing proportion of AIDS-NHL in hemophiliacs are occurring as secondary or later AIDS diagnoses, and they are associated with prolonged duration of immunosuppression.
AIDS-Related Opportunistic Infections↗
Antibiotic-resistant pneumococcal infection in children with sickle cell disease in the United States.
OBJECTIVE: The purpose of this report is to examine the increasing problem of antibiotic-resistant pneumococcal infection in children with sickle cell disease in the United States. PATIENTS AND METHODS: In this retrospective review, 16 children with sickle cell disease and penicillin-resistant pneumococcal invasive infection were identified. They had a median age of 2 years (range 1-15) and were treated in Memphis, Dallas, Los Angeles, and five other cities between 1987 and early 1995. RESULTS: At presentation, patients frequently had high fever (> or 40.0 degrees C in 75%) and a toxic appearance (44%). Meningitis was present initially in two and diagnosed on days 4 and 5 in two. All were treated with an intravenous cephalosporin and nine received vancomycin. The clinical course was variable: two died within 36 h of presentation. In 20-86% of cases the organisms were resistant to cephalosporins, chloramphenicol, trimethoprim/sulfamethoxazole, erythromycin, and clindamycin; none were resistant to vancomycin. CONCLUSIONS: (a) The increasing prevalence of antibiotic-resistant Streptococcus pneumoniae infection in the United States poses special problems for patients with sickle cell disease. (b) Prompt antibiotic susceptibility testing of pneumococcal isolates should be performed. (c) Initial antibiotic management for patients suspected of sepsis/meningitis should include intravenous cephalosporin and vancomycin. (d) No alternative to penicillin prophylaxis is currently available. (e) An effective conjugated pneumococcal vaccine is needed.
Adolescent↗
Osteogenic sarcoma in a child with transfusion-dependent Diamond-Blackfan anemia.
PURPOSE: Diamond-Blackfan anemia or congenital pure red blood cell aplasia is one of the best defined congenital hematopoietic disorders. Although it has been suggested that affected patients are predisposed to developing acute leukemia, there have been no reports of sarcoma in patients with Diamond-Blackfan anemia. We describe a child who had Diamond-Blackfan anemia and who developed an osteogenic sarcoma. PATIENT AND METHODS: A case is presented of a 5-year-old girl who had transfusion-dependent Diamond-Blackfan anemia and hemochromatosis and who developed an osteogenic sarcoma of the tibia. RESULTS: The patient had an initial response with an ifosfamide-containing chemotherapy regimen but died of multiple pulmonary metastases. CONCLUSION: Our patient is the 10th reported case of malignancy in Diamond-Blackfan anemia but the first instance of sarcoma. Given the increased rate of leukemia in Diamond-Blackfan anemia patients, we speculate there may also exist an association between osteogenic sarcoma occurring at a particularly young age and this uncommon hematologic disorder.
Adolescent↗
Intensive oral methotrexate protects against lymphoid marrow relapse in childhood B-precursor acute lymphoblastic leukemia.
PURPOSE: To describe the use of combination chemotherapy, including divided-dose oral methotrexate (dMTX), for children with B-precursor acute lymphoblastic leukemia (ALL). dMTX produced prolonged MTX exposure on an outpatient basis. PATIENTS AND METHODS: Two hundred forty-three patients were treated from January 1986 to May 1992. dMTX was given weekly during consolidation and biweekly for the first 16 months of continuation therapy with mercaptopurine (6-MP) and asparaginase (L-ASP). Initially, etoposide (VP-16) and cytarabine (Ara-C) pulses were included. Treatment continued for 30 months with single-dose weekly MTX replacing dMTX during continuation, part 2. Unexpected acute neurotoxicity was eliminated by the addition of leucovorin. VP-16 and Ara-C were omitted in the face of acute myelogenous leukemia (AML). RESULTS: Two hundred thirty-nine patients entered remission: 16 had a lymphoid marrow relapse, two each with testicular or CNS relapse; 19 a CNS relapse; 16 secondary AML; three other second malignancies; two withdrew for transplant; three died in remission; 16 withdrew because of noncompliance, and nine withdrew with toxicity. Event-free survival (EFS) at 4 years was 73 +/- 4%; 81 +/- 4% for 150 patients with better risk features and 60 +/- 7% for 93 with high-risk features. Lymphoid marrow relapse-free survival in the standard- and high-risk patients was 94 +/- 3% and 86% +/- 6%, respectively. The most common adverse event was secondary AML in the standard-risk group and isolated CNS relapse in the high-risk group. CONCLUSION: This therapy produced an overall EFS similar to other published regimens, but the pattern of failures is very different, with few patients having a lymphoid marrow relapse. These data suggest that highly effective therapy for children with ALL can be delivered on an outpatient basis using a regimen featuring repetitive dMTX.
Administration, Oral↗