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Direct antiglobulin test positivity had been reported in the course of some lymphoproliferative neoplasms. However, there are a few case reports describing direct antiglobulin test (DAT) positivity in children with acute lymphoblastic leukemia (ALL). We herein report 8 patients who had positive DAT among 95 newly diagnosed children with ALL. None of these patients had evidence of hemolysis during the follow-up. An antibody was detected in 2 of 8 patients with positive DAT. These 2 children also had positive indirect antiglobulin test (IAT); an autoantibody that was reactive at 4°C, and an alloantibody (anti E) that was reactive at 37°C was detected. We believe DAT positivity in ALL without significant hemolysis is not a rare disorder, and a need for further prospective studies is apparent.

Purpose: In this study, it is aimed to compare the serum leptin and PAI-1 levels and evaluate their relationship in children on hemodialysis (HD) and peritoneal dialysis (PD)., Method: Thirty-six patients on HD (mean age: 15.0 ± 2.8 years), 19 patients on PD (mean age: 13.0 ± 3.5 years) and 15 healthy subjects (mean age: 14.5 ± 2.7 years) were included in the study. Laboratory investigations included blood count, biochemical parameters, serum iron, iron binding capacity, parathormone, erythrocyte sedimentation rate, C-reactive protein (CRP), prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, serum leptin and PAI-1 levels., Results: Serum leptin levels were significantly higher in HD group than in control group when the effects of BMI and sex were controlled, while PD and control groups had similar leptin levels. PAI-1 levels were also significantly higher in HD group than in control group, while there was no statistically significant difference in PAI-1 levels of PD and control group. PAI-1 levels and leptin levels were significantly correlated, which was independent of the effect of BMI in both HD and PD groups when they are evaluated separately., Conclusion: Results of our study showed that HD patients had higher leptin and PAI-1 levels and leptin and PAI-1 levels were correlated significantly in both patient groups. The effect of elevated serum leptin and PAI-1 levels on the cardiovascular complications remains to be established.

A slurry sampling method is proposed for the determination of iron and zinc in baby food by flame atomic absorption spectrometry without sample digestion prior to analysis. The effect of slurry concentration (the ratio of solid sample to total slurry volume), different acids at various concentrations as diluent and the addition of dispersant on accuracy and precision were investigated. The samples were dried at 105 °C overnight and ground using an agate mortar. To obtain quantitative recoveries, 500 mg of sample was slurried in 20 ml of 0.05% Triton X-114 containing 0.1 M HNO(3), homogenised using a high-performance overhead disperser at 15,000 rpm for 5 min and directly aspirated into the flame. The accuracy of the method was tested by determination of analytes in various certified reference materials. The limits of detection of the method (N = 10; 3σ) for iron and zinc were 5.5 and 3.4 µg g(-1), respectively, using a very dilute slurry of baby food, which gave a very low background signal. Finally, the proposed method was applied to the determination of iron and zinc in different baby food samples obtained from markets in Turkey. The range of iron and zinc content for the samples were 33-76 and 15-73 µg g(-1), respectively.

Aim: To understand the effect of prenatal chronic hypoxia on prohepcidin levels in term newborns., Method: We determined prohepcidin (Pro-Hep) levels in both term appropriate-for-gestational age (AGA) and term small-for-gestational-age (SGA) infants. Uteroplacental insufficiency had exposed all SGA infants to chronic hypoxia. Serum samples were collected from nine full-term SGA infants. Samples were analyzed for complete blood count, serum iron and ferritin concentrations, iron-binding capacity, and prohepcidin levels., Results: The mean serum Pro-Hep level was 156.4 ? 46.7 ng/ml for SGA infants and 482 ? 371.9 ng/ml for 16 healthy term AGA infants (historical controls); this difference was statistically significant. Statistical analyses revealed significant between-group differences for hemoglobin, hematocrit, mean corpuscular volume, red blood cell distribution width, and serum ferritin and Pro-Hep levels., Conclusion: This study showed that compared with AGA infants, Pro-Hep levels were lower in term SGA infants, suggesting that prenatal chronic hypoxia decreases Pro-Hep synthesis.

Cardiac complications of the pediatric patients with acute leukemia are common. Most of the cardiac complications may be due to chemotherapeutics such as antracyclins, besides anemia, infections, or direct leukemic infiltrations of the heart. It is reported that leukemic infiltration is frequent in the postmortem examination of the myocardium and pericardium. However, at the antemortem examination, pericardial involvement is rare and there is no myocardial involvement reported at the time of diagnosis in patients with acute leukemia in the English literature. Here, the authors report an adolescent with acute lymphoblastic leukemia who had myocardial infiltration at the time of diagnosis.

This study was conducted to analyze the incidence of and risk for thrombosis in thrombotic children monitored in the Department of Pediatric Hematology of our hospital at the time of diagnosis, in addition to the clinical characteristics of those patients. The clinical and laboratory findings of 122 patients diagnosed with thrombosis from 1997 to 2006 were retrospectively analyzed. The incidence of thrombosis was 88.6/10,000 hospital admissions. The authors found that 31.1% of the patients studied had a thrombosis in more than 1 region. The incidence of thrombosis by anatomic site was as follows: 42 thromboses in the peripheral arterial system, 39 in an intracardiac region, 38 in the abdominal venous system, 36 in the deep peripheral venous system, and 28 in the cerebral vascular system. The mean age of the patients at the time of diagnosis was 4.9 years. Of the patients studied, 10.7% were neonates, 35.3% were infants younger than 1 year, and 48.4% were younger than 2 years. Most of the patients had a congenital cardiac disease and spontaneous thrombosis, and 66.1% had at least 1 acquired risk factor, the most common of which were having undergone surgery (42%) or wearing a central venous catheter (39%). A hereditary factor for the development of thrombosis was present in 54% of the patients. The most frequently observed hereditary risk factor was the MTHFR 677C-T mutation, and the second most common was the factor V Leiden mutation. Thrombosis should be considered a systemic disorder, and thrombotic patients should be evaluated with appropriate methods. Acquired and hereditary risk factors should be analyzed systematically in thrombotic patients.

Some clinical manifestations of acute leukemia in children can mimic orthopedic conditions, and t is variable presentation often makes diagnosis difficult. Bone changes in leukemia are well documented, but there are only a few accounts of children with acute leukemia who present with bone fractures. This report describes a case of this rare combination in a very young boy who presented with fractures of both proximal humerus and left proximal femur and massive periosteal reactions of both humerus and femur and also cystic lesions of proximal femur and iliac bone accompanying aggressive acute megakaryoblastic leukemia.

Invasive fungal infection is one of the major causes of morbidity and mortality in immunocompromised patients. The occurrence of two invasive fungal infections in one patient at the same time is quite rare. Here the authors report on two adolescent patients with acute lymphoblastic leukemia who developed combined invasive pulmonary aspergillosis and hepatosplenic candidiasis during chemotherapy. They were treated with liposomal amphotericin B, but one of them died due to massive pulmonary hemorrhage during recovery from neutropenia.

Transient myeloproliferative disorder is a self limiting disorder characterized by leukocytosis with the presence of megakaryoblasts in the peripheral blood and bone marrow, anemia, thrombocytopenia, and organomegaly. It occurs in approximately 10% of newborn infants with Down syndrome. Hepatic fibrosis is seen in the severe form of transient myeloproliferative disorder with Down syndrome that is characterized by diffuse intralobular sinusoidal fibrosis and extramedullary hematopoesis. We describe a patient with hydrops fetalis, Down syndrome, and transient myeloproliferative disorder. We suggest that patients with the severe form of transient myeloproliferative disorder should be examined for hepatic fibrosis.

Congenital leukemia is rare, and is frequently diagnosed as a form of acute myeloid leukemia at, or immediately after birth. Some infections, viral in particular, can mimic clinical signs and/or laboratory findings of congenital leukemia. This is the first documented case of candidemia resembling leukemia.

Aspergillosis is one of the most common invasive fungal infections in patients with leukemia. In this patient group, this form of Aspergillus infection is a life-threatening condition with a mortality of 50-100%. The lungs are most often affected, but the esophagus can also be involved.The authors report the case of a child with leukemia who developed invasive esophageal aspergillosis. The condition was diagnosed by microscopic examination of endoscopic biopsy specimens. The patient was already receiving empirical liposomal amphotericin B when the diagnosis was made, so a second antifungal (caspofungin) was added to the regimen. This combination was successful. This case to demonstrates a case of successful treatment of invasive esophageal aspergillosis using combination therapy of liposomal amphotericin B and caspofungin.

A 4-year-old girl with juvenile myelomonocytic leukemia presented to the emergency room with dyspnea. Echocardiography was performed due to cardiomegaly and prominent main pulmonary artery on a chest X-ray film. On echocardiography the right ventricular pressure calculated from the velocity of tricuspid regurgitation jet was 55 mmHg with no pulmonary stenosis. Despite treatment for pulmonary hypertension and provision of respiratory support, the patient died. A postmortem lung biopsy specimen showed infiltration by tumor cells, which suggested that the pulmonary hypertension had been caused by leukemic infiltration. In conclusion, the findings suggest that leukemic infiltration into the lungs may occur in children with juvenile myelomonocytic leukemia. It should be recognized as a potentially treatable cause of pulmonary hypertension in patients with juvenile myelomonocytic leukemia.

A recently isolated peptide hormone, hepcidin, is thought to be the principal regulator of iron homeostasis. Hepcidin acts by limiting intestinal iron absorption and promoting iron retention in reticuloendothelial cells. Its precursor peptide form is called pro-hepcidin. The aims of this study were to determine serum pro-hepcidin levels in healthy preterm and term newborns, and to assess possible relationships between pro-hepcidin and serum iron, serum ferritin, and transferrin. A serum sample was collected from each of 26 healthy preterm (gestational age < 37 weeks) and 16 healthy, full-term, appropriate-for-gestational age babies. The preterm babies were also divided into 2 subgroups based on gestational age. Samples were analyzed for complete blood count, serum iron and ferritin concentrations, iron-binding capacity, and transferrin and pro-hepcidin levels. Group findings were compared and correlations between pro-hepcidin and the iron parameters were tested. The respective serum pro-hepcidin levels (mean +/- SD) in the 16 healthy term and 26 healthy preterm newborns were 482 +/- 371.9 ng/mL and 496.7 +/- 443.5 ng/mL. Analysis revealed no significant correlations between serum pro-hepcidin level and serum iron, serum ferritin, or transferrin in the preterm or term newborns. Pro-hepcidin levels were not correlated with gestational age in the preterm group. The results indicate that healthy preterm and term newborns have high pro-hepcidin levels.

The diagnosis and management of a surgical abdomen in patients with acute leukemia is quite difficult because of the complications and treatment of disease itself. A 13-year-old boy with acute myelogenous leukemia developed 2 episodes of febrile neutropenia during induction therapy. The second one was treated with a 5-day course of parenteral antimicrobial therapy, but the patient then presented with right lower quadrant abdominal tenderness, guarding, and rebound tenderness. Abdominal ultrasonography and computed tomography revealed appendicitis. Conservative medical management was unsuccessful, and appendectomy was performed 5 days after appendicitis was diagnosed. The patient's clinical manifestations resolved 5 days later. The case illustrates that fever may be the first manifestation of appendicitis in a child with acute myelogenous leukaemia who is neutropenic. Surgery is acceptable as first-line treatment in such cases.

Leishmania-associated hemophagocytic lymphohistiocytosis is a rare clinicopathological entity. This condition is often difficult to diagnose, so treatment is often delayed. This report describes the case of a 5-year-old boy who was admitted with fever of 1 month's duration, hepatosplenomegaly, and pancytopenia. Serum testing showed elevated transaminase levels, hypertriglyceridemia, hyperferritinemia, and normal fibrinogen level. Hemophagocytic lymphohistiocytosis was diagnosed on bone marrow examination. The patient was tested for various infectious agents. He was negative for all except Leishmania, which was detected by indirect fluorescent antibody testing. Treatment with amphotericin B resulted in a dramatic resolution of all signs and symptoms within 1 week.

Although human parvovirus B19 (PVB19) is mainly known to be the causative agent of fifth disease, it is also important in the etiopathogenesis of various hematologic disorders. Recently, its role in the occurrence of chronic anemia and pancytopenia in immunocompromised patients has been revealed. In this study, the authors searched for the presence of PVB19 in a group of patients with idiopathic thrombocytopenic purpura (ITP). The PVB19 DNA was determined by nested PCR, and serologic responses to the virus were evaluated by ELISA. Viral genome was detected by PCR in the sera of 9 of the 19 patients. The IgM and IgG positivities were 57 and 73% in the patients, respectively. These results suggest that PVB19 is associated with ITP nearly in half of the patients and its pathogenetic role in the cases of ITP needs further consideration.

Treatment of neutropenia with corticosteroids and hemopoietic growth factors may cause alterations in the immune system. This study investigated and compared the effects of various regimens (HDMP, G-CSF, and G-CSF + HDMP) on total lymphocyte counts and subtypes in neutropenic patients with acute lymphoblastic leukemia. Lymphocyte subtypes were studied just prior to treatment and 1 week later. In the HDMP group, total lymphocyte counts and the numbers of CD3+, CD4+, CD8+, and CD19+ lymphocytes and monocytes increased, while the helper/suppressor lymphocyte ratio decreased. No significant changes were seen in lymphocyte subtypes in the G-CSF and G-CSF + HDMP groups, apart from an increase in total lymphocyte numbers. In the control group there was an increase in the number of CD3+ and CD8+ lymphocytes and a decrease in the helper/suppressor lymphocyte ratio. These results suggest that HDMP has an inducing, rather than a suppressive effect on all lymphocyte subtypes studied.