Search

Background A walking blood bank (WBB) refers to the use of fellow combatants for battlefield blood donation. This requires pretesting combatants for infectious diseases and blood type. A fundamental prerequisite for this technique is that the donating soldier will suffer minimal physiological and mental impact. The purpose of the current study is to assess the effect of blood shedding on battlefield performance. Methods This is a double-blind randomized control trial. Forty Israel Defense Forces combatants volunteered for the study. Participants underwent baseline evaluation, including repeated measurement of vital signs, cognitive evaluation, physical evaluation, and a strenuous shooting test. Three weeks after the baseline evaluation, subjects were randomized to either blood donation or the control group. For blinding purposes, all subjects underwent venous catheterization for the duration of a blood donation. Repeated vital signs and function evaluation were then performed. Results Thirty-six patients were available for randomization. Baseline measurements were similar for both groups. Mean strenuous shooting score was 80.5 ± 9.5 for the control group and 82 ± 6.6 for the test group (p = 0.58). No clinically or statistically significant differences were found in tests designed to evaluate cognitive performance or physical functions. Vital signs taken multiple times were also similar between the test and control groups. Conclusions Executive, cognitive, and physical functions were well preserved after blood donation. This study supports the hypothesis that a WBB does not decrease donor combat performance. The categorical prohibition of physical exercise following blood donation might need to be reconsidered in both military and civilian populations.

Background The ambient exposure does not always reflect the internal levels of pollution absorbed in the body. While human biomonitoring (HBM) could provide a valid estimate of exposure extent, it is usually an expensive and a heavily manpowered enterprise. Using samples collected during blood donations for HMB may provide a more efficient platform for a routine biomonitoring. Methods The current study is aimed to explore the feasibility of using the national blood banking system for the purposes of HBM, to compare between residents of a suspected polluted area in northern Israel (Haifa Bay) to the rest of the country. Specifically, we will assemble a geographically representative sample of blood donors residing in the study area and of the general population, to test for four industry and traffic-related metals: lead (Pb), cadmium (Cd), arsenic (As) and chromium (Cr). Samples of whole blood from donors will be tested in the Laboratory of Public Health Services managed by the Ministry of Health. The information on donors’ biomarkers levels will be further linked with the air pollution and meteorological data assessed at the location of the blood collection sites (short-term exposure) and donors’ permanent address (long-term exposure), as recorded by the monitoring stations spread throughout Israel and the satellite-based exposure models. The association between biomarkers and ambient environmental exposures will be assessed. The samples’ collection is planned for 2 years of 2020–2021. Discussion The information collected in this study could lead to environmental regulations within Haifa Bay area aimed to prevent exposure to high levels of hazardous chemicals.

Erythrocyte sedimentation rate (ESR) is a simple test that is not without limitations but is still widely used. Dysfunction is used as a diagnostic test on the one hand and as a follow-up test on the other, where many physicians attribute normal results of this test to the absence of disease. Therefore, the question remains: Is there still room for continued ESR in the 21st century? And are there other tests with higher sensitivity and specificity that can replace the ESR? The purpose of the current review is to acquaint the reader with this test, its advantages and limitations, and to expand the scope of alternative tests and their effectiveness in comparison to ESR. It seems to us that the development of technology and research has strongly discouraged the use of ESR, but there are still quite a few instances where this test is still cost-effective. In most cases, especially during the diagnosis of life-threatening diseases, other tests are needed, or there is a need for a combination of the ESR with additional tests.

Due to trends of population movements, Israeli family physicians are treating increasing numbers of African immigrants from Ethiopia. These immigrants were found to have complete blood counts (CBC) that are different from other ethnic groups, with a higher prevalence of eosinophilia and neutropenia.To evaluate haematological findings in an attempt to define whether they behave as familial (genetic) or environmental.Retrospective chart review of 300 patients from a primary care clinic: 100 individuals of Ethiopian heritage born in Ethiopia (EE); 100 individuals of Ethiopian heritage born in Israel, whose parents were born in Ethiopia (EI), and a control group of 100 patients who were not of Ethiopian heritage (C).Absolute eosinophilia (greater than 500/dl) was found in 13% of the EE study group significantly higher than the two other groups (P 0.05), with no difference between EI and C. neutropenia (defined as less than 1500/dl) was found in 32% of EE group, 20% of EI, and 1% of C (P 0.01).On the one hand, findings point to a marked environmental influence on the eosinophilic response (most probably due to intestinal parasites present in immigrants from Ethiopia). On the other hand, a familial-genetic nature is probably the reason for the higher prevalence of neutropenia in this population, although some environmental influence may play a role. The knowledge of these findings may be useful for physicians treating people migrating from Africa.

Zehavit Gilboa-Negari,1 Sarah Abu-Kaf,2 Ephrat Huss,1 Gavriel Hain,3 Asher Moser4 1Spitzer Department of Social Work, Ben-Gurion University of the Negev, Beer-Sheva, Israel; 2Conflict Management and Resolution Program, Ben-Gurion University of the Negev, Beer-Sheva, Israel; 3Department of Pediatrics, Soroka University Medical Center, Beer-Sheva, Israel; 4Department of Pediatric Oncology, Soroka University Medical Center, Beer-Sheva, Israel Purpose: Medical clowning has proven effective for reducing pain, anxiety, and stress, however, its differential effects on children from different cultures have not yet been researched. This study evaluated the effects of medical-clowning intervention on anxiety and pain among Jewish and Bedouin children, and anxiety among their parents, in southern Israel. Patients and methods: The study was conducted in hospital pediatric departments and employed a pre–post design involving quantitative and qualitative methods. The study included 89 children whose ages ranged from 7.5 to 12 years (39 Jewish and 50 Bedouin) and 69 parents (19 Jewish and 50 Bedouin). Questionnaires assessing pain, anxiety, and demographics were used at the pre-intervention stage and pain, anxiety, and enjoyment of different aspects of the intervention were evaluated following the intervention. The intervention stage lasted for 8–10 minutes and included the use of word play, body language, and making faces, as well as the use of props brought by the clown. Semi-structured interviews were also conducted at the post-intervention stage. Results: The intervention reduced pain and anxiety among both groups of children and reduced anxiety among both groups of parents. However, anxiety levels were reduced more significantly among Bedouin children. The nonverbal components of the clowns’ humor were most central, but it was the verbal components that mediated the reduction in anxiety among the Bedouin children. Conclusion: This study underscored the effectiveness and importance of medical clowning in reducing pain and anxiety among children in different cultural contexts. Moreover, the issue of culturally appropriate humor was underscored and implications for intercultural clown training are discussed. Keywords: humor, cultural contexts, intervention, pre–post design, parents, pediatric departments, nonverbal component

This study examines whether structured writing about receiving a diagnosis and treatment for pediatric cancer reduces distress among highly distressed parents of children with cancer (PCWC). Eight PCWC completed measures of posttraumatic stress symptoms (PTSS) and depressive symptoms at two baselines, and again after writing, with 1-month gaps between assessments. Using a guided disclosure protocol (GDP), parents were asked to write about receiving the diagnosis first in a chronological manner, then to explicitly label their emotions at the time of diagnosis and explain the impact of the child's illness on their life. Finally, they were asked to reflect on current feelings, future coping ability, and personal growth. Although symptoms of distress did not change between baselines, significant reductions were found in PTSS from the first baseline to postwriting, but not in depression. This preliminary study suggests that the GDP may reduce PTSS in distressed PCWC.

Hypocholesterolemia of unknown etiology has been previously described in various chronic anemias. Few small studies also suggested that those patients have a lower incidence of atherosclerotic events. The aim of our study was to determine the extent of hypocholesterolemia in various types of anemias. We studied 59 patients with chronic anemias associated with high-erythropoietic activity (thalassemia intermedia, congenital dyserythropoietic anemia type I, congenital spherocytosis), 8 patients with low-erythropoietic activity anemias (acquired aplastic anemia, Fanconi anemia, and Diamond Blackfan anemia), and 20 healthy controls. Mean serum cholesterol, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, triglycerides, hemoglobin, serum ferritin, soluble transferrin receptor (STR), and serum erythropoietin levels were determined in each patient. All patients with chronic anemia and increased erythropoietic activity had hypocholesterolemia, whereas none of those with low erythropoietic activity was hypocholesterolemic. Mean serum cholesterol, HDL cholesterol, and LDL cholesterol levels were found to be significantly lower in the high-erythropoietic activity group (80+/-19 mg/dl; 31+/-10 mg/dl; 35+/-14 mg/dl, respectively) compared with the control group (P

T-cell prolymphocytic leukemia is a very rare neoplasm, peaking in the seventh decade. An extensive search failed to find any report of this malignancy in the pediatric population. The malignant cell is morphologically characterized by a high nucleocytopasmic ratio, condensed chromatin, a single nucleolus, and nongranular basophilic cytoplasm. Cells are usually positive for the α/β and only rarely to the γ/δ T-cell receptors. Most patients follow an aggressive clinical course, only some respond to anti-CD52. We present a 6-year-old boy with T-cell prolymphocytic leukemia. The malignant cells expressed a postthymic immunophenotype (CD4/CD8) and positivity for the γ/δ T-cell receptors. The child died after 8 months despite aggressive chemotherapy, anti-CD52, and an allogeneic bone marrow transplant.

Here we describe a cytogenetic and flow-cytometric study of a case in which a conversion of childhood acute lymphocytic leukemia (ALL) into juvenile myelomonocytic leukemia (JMML) occurred. A 3-year-old boy diagnosed CALLA+, pre-B-ALL with double t(12;21) (by fluorescence in situ hybridization analysis), was treated as per the BFM protocol. A cytogenetic analysis performed at 17 months into treatment showed no t(12;21) in bone marrow (BM) cells; however, a novel translocation, namely, t(4;11), involving the p12 locus on chromosome 4 and the MLL gene at 11q23 was detected in monocytes. No cytogenetic abnormalities were found either in Epstein-Barr virus-transformed B cells or in phytohemagglutinin-stimulated T-lymphoid cells. Flow-cytometric analysis demonstrated an asynchronous expression of the antigenic determinants in populations of granulocytic and monocytoid cells: 60% of monocytes expressed low levels of CD14, an unusually high level of CD15, and no CD13 or HLA-DR antigens; 74% of myeloid cells expressed no CD13. Our results indicate that the transformation from B-cell ALL to JMML in this case occurred most probably in the granulocyte-erythroid-macrophage-megakaryocyte progenitor cells without involving the lymphoid cell line. To date, the child is 10 months off therapy and asymptomatic, with t(4;11) in only 3% of the cells.

Acute idiopathic (immune) thrombocytopenic purpura (ITP) in the pediatric population is a disease in which autoimmune features are mainly self-limited, with a reported mortality of 0.1-0.5%. Major treatment requires intravenous gammaglobulins (i.v. IgG) and corticosteroids. Recently a new globulin, anti-D, has been introduced. The authors have treated 25 children suffering from acute idiopathic thrombocytopenic purpura, with an i.v. anti-D dose of 75 microg/kg as the first treatment. Eligibility criteria included a platelet count15,000 and Rh+. Post-treatment response was 76%20,000 platelets at 6-10 h and 80%50,000 platelets at 48 h; three patients developed chronic idiopathic thrombocytopenic purpura. There were 5/25 patients who did not respond to the initial dose and received i.v. IgG and corticosteroids, 2/5 with a positive response (platelets20,000). Side effects consisted of chills (9/25), fever38 degrees C (6/25), headache and vomiting (1/25), hemolysis (20/25) from 0.9-6.9 g%, and decrease in hemoglobin levels. One patient needed a blood transfusion after his Hbg decreased from 12.4 to 5.5 g%. The results indicate that anti-D is an effective treatment in acute ITP, but with side effects. Administration of steroids and antipyretics prior to anti-D treatment may prevent the side effects.

Post-transplantation lymphoproliferative disorders (PTLDs) and Kaposi's sarcoma (KS) are immunosuppression-related tumours developing in solid organ transplant patients. Although the Epstein-Barr virus (EBV) is detected in the majority of the PTLDs during the first year after transplantation, the proportion of EBV-negative PTLDs has increased in recent years. We report a case of a 17-year-old man who developed severe immune haemolytic anaemia, KS and human herpesvirus 8 (HHV-8)-associated, polymorphic-type PTLD 9 months after allogeneic renal transplantation from his HHV-8-seropositive father. It is suggested that: (i) HHV-8 may be associated with EBV-negative, polymorphous-type PTLD occurring less than 1 year after transplantation, and (ii) PTLD may be listed among other tumours, including KS, Castleman's disease and primary effusion lymphoma (PEL), that are related to HHV-8 infection.

Autosomal recessive malignant osteopetrosis (MOP) is a lethal disease, unless bone marrow is successfully transplanted. Yet a donor may not always be available, and even when there is one transplantation results are far from optimal. The difficulty in obtaining conclusive results by sonographic and X-ray evaluation of the fetus makes prenatal molecular diagnosis highly desirable. Subsequent to the chromosomal localization of the MOP gene in Arab-Bedouin families from the Negev region in Israel, linkage analysis was used for the prenatal diagnosis of this disease in Bedouin families at risk. Twelve cases were diagnosed, three fetuses were found to be affected, and one of the pregnancies was terminated. The other two pregnancies continued to term and the diagnosis of osteopetrosis was confirmed by X-ray immediately after birth. This is the first report on prenatal diagnosis of autosomal recessive osteopetrosis by linkage analysis. Copyright © 2001 John Wiley & Sons, Ltd.

BACKGROUND Identifying markers associated with blood stream infection (BSI) in children with fever and neutropenia (FN) could lead to a substantial reduction in unnecessary treatment. STUDY OBJECTIVE The aim of this study was to determine the association between clinical/laboratory parameters and BSI in pediatric oncology patients with FN. METHODS This prospective study was conducted between 2007 and 2010 at the Pediatric oncology unit. Clinical and laboratory parameters were obtained from all hospitalized FN patients. Linear regression and trends were calculated to determine the association between clinical/laboratory parameters and BSI. RESULTS Of the 195 FN episodes in 73 children, BSIs were identified in 38 (19%) episodes. Gram-positive bacteria, gram-negative bacteria, and fungi caused 47%, 43%, and 10% of all BSIs, respectively. Mean fever duration was longer in the BSI group (5 d) compared with the non-BSI group (2 d, P=0.01). Mean (±SD) monocyte count at admission was lower in the BSI group compared with the non-BSI group (0.06±0.1 vs. 0.14±0.33 cells/mm, respectively, P=0.05). Mean C-reactive protein (CRP) levels at hospitalization days 5 to 8 were higher in children with BSI (P

Structured lipid emulsion, an innovative approach in which both medium-chain and long-chain fatty acids are esterified to the same glycerol backbone, has been recently shown to be a safe and efficient way of providing energy to patients requiring parenteral nutrition. As yet, no assessment has been made of its safety and effect on liver functions during long-term treatment. Twenty-two home parenteral nutrition patients with Crohn's disease or short bowel syndrome were enrolled in a double-blind randomized, cross-over study. Twenty patients who completed the study were treated for 4 wk with a structured lipid emulsion and for 4 wk with long-chain triacylglycerol emulsion. Determined every 1 or 2 wk were blood pressure, body weight, respiratory rate, blood count, liver functions, albumin, transferrin, plasma lipids, free fatty acids (FFAs), and, at the end of each treatment period (weeks 4 and 8), plasma dicarboxylic acids and 3-OH-fatty acids. No differences were observed between the groups or within the groups between the two treatments with respect to either clinical safety and adverse event occurrence or laboratory assessments. Plasma dicarboxylic acids and 3-OH-fatty acids were similar and within normal range. No alteration of liver function occurred in any of the patients treated with the structured lipid emulsion, whereas two of the patients receiving long-chain triaclyglycerol emulsion developed abnormal liver function, which resolved after switching to the structured lipid emulsion. In conclusion, structured triacylyglycerols containing both medium- and long-chain fatty acids appear to be safe and well tolerated on a long-term basis in patients on home parenteral nutrition, and it may be associated with possible reduction in liver dysfunction.

In an attempt to understand the metabolism by the liver of fatty acids (FA) of different chain length, we have studied the incorporation of [1(-14)C]-labeled C2, C8, C10, C12, and C16 into cellular lipids by HepG-2 cells. Over 90% of the radiolabeled FA were detected in phospholipids (PL) and triacylglycerols (TAG). The incorporation of C12 and C16 was three to four times higher than that of C8 and C10 (and reached 35 nmoles per mg protein after 1.5 h). The radioactivity of C2, C8, and C10 was recovered mainly in PL. C12 and C16 were incorporated at approximately equal amounts into PL and TAG. The radioactivity of both C2 and C8 was recovered exclusively in long-chain FA, suggesting oxidation of C8 into C2 units prior to FA synthesis. C10 likewise yielded mainly long-chain FA. However 10% of unchanged C10 was found in PL and up to 30% in TAG. 14C-C12 was largely incorporated unchanged. Under these conditions, the presence of C10 and C12 in PL and TAG was shown also by gas-liquid chromatography. In the presence of either C2, C8, or C10, up to 30% of 14C-monounsaturated FA were detected in PL and TAG. With C12 and C16, the fraction of 14C-monounsaturated FA was much smaller suggesting that extensive desaturation occurred during de novo synthesis.

Congenital dyserythropoietic anemia (CDA) type I is an inherited disorder characterized by macrocytic anemia with pathognomonic morphologic ultrastructural features of the erythroid precursors. The authors recently cloned the CDAN1 gene and identified one founder missense mutation in all of their Bedouin patients. In a previous study, the authors found that the majority of their 31 Bedouin patients had anemia and jaundice during the first month of life and required blood transfusions; some had persistent pulmonary hypertension. In the present retrospective evaluation of 70 Bedouin patients with CDA type I, the authors more than doubled the number. Forty-five (64%) patients were symptomatic in the neonatal period, 29 (65%) had hepatomegaly, 24 (53%) had early jaundice, 11 (27%) were born small for gestational age, 7 (15%) had persistent pulmonary hypertension, and 6 (13%) had direct hyperbilirubinemia and another 6 (13%) had transient thrombocytopenia. Thirty-six of the symptomatic neonates (80%) required at least one blood transfusion. These results confirm the authors' previous findings and add neonatal manifestations not previously described, particularly hyperbilirubinemia and thrombocytopenia. Early diagnosis of CDA type I may be beneficial in light of the potential efficacy of alpha-interferon in avoiding transfusions in some patients.

The effect of a lipid emulsion containing long-chain triglycerides (LCT) and supplemented with L-carnitine on plasma lipids and bilirubin in premature neonates on total parenteral nutrition was compared to that of lipid emulsions containing either LCT or a mixture of LCT and medium-chain triglycerides (MCT). In a double-blind randomized study 49 premature neonates received one of the three fat emulsions, given intravenously, over 16-20 h daily for 6 days. Plasma carnitine levels increased significantly in the supplemented group only; the addition of carnitine did not seem to affect any of the parameters studied. Mean plasma triglycerides rose by 193 and 199% in the carnitine-supplemented and the LCT groups, respectively, and by 314% in the MCT/LCT group. On the sixth day of the study free fatty acids were significantly higher in the MCT/LCT group than in the other two groups. Plasma phospholipids and free cholesterol increased (p < 0.05) progressively in all groups and were correlated (r = 0.74, p < 0.001). At the end of the 6-day study all groups showed a similar decline in free and total bilirubin levels despite the significant increase in plasma lipids and free fatty acids resulting from the stepwise increase in lipid load. No correlation was found between free fatty acids and free bilirubin. Since hyperbilirubinemia and hypertriglyceridemia appear to be clinically independent factors, the infusion of lipids should not be withheld from jaundiced infants on total parenteral nutrition.

MOTIVATION: It has been proposed that clustering clinical markers, such as blood test results, can be used to stratify patients. However, the robustness of clusters formed with this approach to data pre-processing and clustering algorithm choices has not been evaluated, nor has clustering reproducibility. Here, we made use of the NHANES survey to compare clusters generated with various combinations of pre-processing and clustering algorithms, and tested their reproducibility in two separate samples. METHOD: Values of 44 biomarkers and 19 health/life style traits were extracted from the National Health and Nutrition Examination Survey (NHANES). The 1999-2002 survey was used for training, while data from the 2003-2006 survey was tested as a validation set. Twelve combinations of pre-processing and clustering algorithms were applied to the training set. The quality of the resulting clusters was evaluated both by considering their properties and by comparative enrichment analysis. Cluster assignments were projected to the validation set (using an artificial neural network) and enrichment in health/life style traits in the resulting clusters was compared to the clusters generated from the original training set. RESULTS: The clusters obtained with different pre-processing and clustering combinations differed both in terms of cluster quality measures and in terms of reproducibility of enrichment with health/life style properties. Z-score normalization, for example, dramatically improved cluster quality and enrichments, as compared to unprocessed data, regardless of the clustering algorithm used. Clustering diabetes patients revealed a group of patients enriched with retinopathies. This could indicate that routine laboratory tests can be used to detect patients suffering from complications of diabetes, although other explanations for this observation should also be considered. CONCLUSIONS: Clustering according to classical clinical biomarkers is a robust process, which may help in patient stratification. However, optimization of the pre-processing and clustering process may be still required.

Introduction Osseous involvement of Hodgkin's lymphoma is uncommon. When osteolytic lesions are seen on imaging it is important to evaluate potential other causes. Case presentation We report the case of a 10-year-old Caucasian boy who presented to our facility with a bony lesion of the right clavicle and enlarged cervical lymph nodes. A simultaneous biopsy of the lymph node and of the osteolytic process of his right proximal clavicle was performed and revealed two different kinds of lesions: a mixed cellularity Hodgkin's lymphoma and an osteochondroma. Conclusions Since the latter is a common benign bone tumor, which should not interfere with the staging of the lymphoma, we emphasize the importance of ensuring that all efforts are made to acquire a diagnostic biopsy of all atypical lesions.

BACKGROUND Iron deficiency anemia (IDA) is the most common hematologic disorder worldwide. Measures to prevent IDA in infants have been successful with questionable sustainability. AIM To evaluate the incidence of developing IDA in the second year of life, in infants who were nonanemic at the age of 1 year on routine blood test. METHODS Blood samples were obtained from 193, 24-month-old toddlers, from 2 large clinics of both main sectors in Southern Israel, comparable for lower economic status. IDA was defined as hemoglobin < 11 gr% and microcytosis as mean corpuscular volume < 70 fL. RESULTS IDA was detected in 8 of 118 Bedouins (5 males) and in 10 of 75 Jewish (6 males) infants (P < 0.01). The probability of a nonanemic child to develop IDA in the second year of life for the whole study population was 9.3% (18 of 193 infants) and significantly higher in the Jewish population (13.3.0% vs. 6.8%, P < 0.01). CONCLUSIONS Given the difficulty of toddlers to maintain a non-IDA status, and the very low probability of iron overload, our results clearly support the need to continue iron supplementation into the second year.

Iron deficiency is the most common single cause of anemia worldwide. Treatment consists of improved nutrition along with oral, intramuscular or intravenous iron administration.To describe the efficacy and adverse effects of intravenous iron sucrose therapy in a group of children with iron deficiency anemia who did not respond to oral iron therapy.We conducted a prospective investigation of 45 children, aged 11 months to 16 years, whose oral iron therapy had failed. The children attended the Pediatric Day Care Unit where they received intravenous iron sucrose infusion.Forty-four of the 45 patients were non-compliant. Nine had Helicobacter pylori gastritis and 16 patients suffered from intestinal malabsorption from different causes. Before treatment, the blood mean hemoglobin concentration was 7.43 g/dl (range 5-10.1 g/dl). Fourteen days after treatment it increased to 9.27 g/dl (SD 1.23) and 6 months later to 12.40 g/dl (SD 1.28). One patient demonstrated a severe side effect with temporary and reversible reduced blood pressure during treatment.These preliminary data suggest that administration of intravenous iron in pediatric patients is well tolerated and has a good clinical result, with minimal adverse reactions.

Constitutional symptoms and pancytopenia are occasionally the initial presentation of pediatric brucellosis. Therefore, in endemic areas, in children with pancytopenia, both brucellosis and malignancy should be included in the deferential diagnosis. We report here a child with pancytopenia and hepatosplenomegaly as manifestations of brucellosis in whom bone marrow morphology and flow cytometry data revealed hemophagocytosis, left shift in myeloid cells and activation changes in antigenic properties of T and B lymphocytes and monocytes. The patient had an uneventful and complete recovery after appropriate antibiotic therapy. Our report demonstrates that bone marrow and flow cytometry findings in children with brucellosis may include significant reactive changes in hematopoiesis.

To explore how enzyme affinities and enzyme activities regulate hydrolysis of water-insoluble substrates, we compared hydrolysis of phospholipid-stabilized emulsions of medium-chain (MCT) versus long-chain triacylglycerols (LCT). Because substrate solubility at the emulsion surface might modulate rates of hydrolysis, the ability of egg yolk phosphatidylcholine to solubilize MCT was examined by NMR spectroscopy. Chemical shift measurements showed that 11 mol % of [13C]carbonyl enriched trioctanoin was incorporated into phospholipid vesicles as a surface component. Similar methods with [13C]triolein showed a maximum solubility in phospholipid bilayers of 3 mol % (Hamilton & Small, 1981). Line widths of trioctanoin surface peaks were half that of LCT, and relaxation times, T1, were also shorter for trioctanoin, showing greater mobility for MCT in phospholipid. In assessing the effects of these differences in solubility on lipolysis, we found that both purified bovine milk lipoprotein lipase and human hepatic lipase hydrolyzed MCT at rates at least 2-fold higher than for LCT. With increasing concentrations of MCT, saturation was not reached, indicating low affinities of lipase for MCT emulsions, but with LCT emulsion incubated with lipoprotein lipase, saturation was reached at relatively low concentration, demonstrating higher affinity of lipase for LCT emulsions. Differences in affinity were also demonstrated in mixed incubations where increasing amounts of LCT emulsion resulted in decreased hydrolysis of MCT emulsions. Increasing MCT emulsion amounts had little or no effect on LCT emulsion hydrolysis.(ABSTRACT TRUNCATED AT 250 WORDS)

The case of an 11-year-old child with adult-type chronic myeloid leukemia, Philadelphia ( BCR-ABL ) positive, reverse transcription–polymerase chain reaction negative for the major, minor, and micro breakpoints is presented. In the course of 3 years, the child failed to respond to treatment with hydroxyurea, refused all therapy for 6 months, was intolerant to α-interferon and progressed, while on imatinib, to acute basophilic leukemia. Subsequently he underwent successful bone marrow transplantation. A secondary cytogenetic clonal evolution, i(17q), developed during hydroxyurea treatment and a tertiary clonal evolution, +8, was detected during imatinib treatment. It is not clear to what extent the several factors (undefined BCR-ABL breakpoint, treatment avoidance, and initial treatment choices, alone or in combination) played a role in the imatinib relapse and resistance and in the disease progression. We conclude that close follow-up with frequent bone marrow sampling is crucial in order to monitor such patients for early relapse and prompt referral for bone marrow transplant.

This study was devised to look at trends in the microbiological spectrum and susceptibility patterns of pathogens causing bacteraemia in paediatric febrile oncology patients. The retrospective study compared various microbiological aspects recorded for febrile oncology neutropenic patients treated with two different empirical antibiotic regimens (ceftazidime plus gentamicin during 1998-1999 and piperacillin/tazobactam plus amikacin during 2000-2002). Eighty-one bacteraemic episodes occurred in 41 patients. Overall, 132 (34 during 1998-1999 and 98 during 2000-2002) organisms were isolated: 84 (65%) Gram-negative bacteria, 39 (30%) Gram-positive bacteria and 7 (5%) fungi. Enterobacter spp. incidence decreased from 18 to 6% (P=0.07) while the recovery rates of Gram-positive organisms increased from 24 to 32% (P=0.4) during 2000-2002 compared with 1998-1999. MRSA were not isolated from any episode of bacteraemia. Five (18%) of the 28 Escherichia coli and Klebsiella spp. isolates were beta-lactamase producers (80% [4/5] isolated during 2000-2002). Twenty-seven of 28, 27/27, 23/28, 20/25 and 27/28 of these isolates were susceptible to imipenem, piperacillin/tazobactam, gentamicin, ceftazidime and ciprofloxacin, respectively. Thirty-two of 34 (94%) and 60/74 (81%) of the Gram-negative organisms isolated during 2000-2002 were susceptible to piperacillin/tazobactam and ceftazidime, respectively (P=0.076). No major differences in the microbial spectrum and antibiotic susceptibilities were recorded between the two consecutive study periods. An increase in the number of extended beta-lactamase producing E. coli and Klebsiella spp. occurred during 2000-2002. All beta-lactamase producing organisms were susceptible to piperacillin/tazobactam and initial empirical therapy with piperacillin/tazobactam was more appropriate than ceftazidime to cover most of the pathogens causing bacteraemia.

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children, but remarkable progress in methods of chemotherapy has increased the cure rate to 80%. The leukemic cells called blasts are eliminated within 7 days of chemotherapy. Clinically, the blast count is monitored directly with the use of blood smears on the basis of specific genetic markers and immunophenotyping methods such as flow cytometry. In this article, we present preliminary results, obtained with the use of Fourier-transform infrared microspectroscopy and cluster analysis, of an approach to monitoring the progress made with chemotherapy in 1 B-cell and 2 T-cell pediatric ALL patients. Our results indicated that the biological marker derived from the spectra did not provide accurate prediction of the progress made with chemotherapy. However, cluster analysis of FTIR-MSP spectra provided good classification of the samples with and without blasts, which correlate satisfactorily with clinical data. Extensive studies are required to substantiate our findings statistically which may have potential application of FTIM in the diagnosis and follow-up of various types of malignancies.

Obstructive sleep apnea can be caused by hypertrophy of tonsils and adenoids or neuromuscular diseases. The authors describe a child with thalassemia intermedia in whom severe obstructive sleep apnea syndrome developed. Computed tomography scanning revealed an obstruction of the nasopharynx resulting from extramedullary hematopoiesis. The child was treated with hydroxyurea and blood transfusions. Relief of symptoms was noted 1.5 months after initial treatment. Extramedullary hematopoiesis causes sleep apnea syndrome in thalassemic patients, and the treatment of hydroxyurea and blood transfusion for extramedullary hematopoiesis should be further studied.

Acute Lymphoblastic Leukemia (ALL) accounts for majority of the childhood leukemia. Outcome of children with ALL treatment has improved dramatically. Sensitive techniques are available today for detection of minimal residual disease in children with ALL, which provide insight into the effective cytotoxic treatment. Here, we present a case study, where lymphocytes isolated from two children before and after the treatment were characterized using microscopic Fourier Transform Infrared spectroscopy. Significant changes in the absorbance and spectral pattern in the wavenumber region between 800-1800 cm-1 were found after the treatment. Preliminary analysis of the spectra revealed that the protein content decreased in the T-lymphoma patient before the treatment in comparison to the age matched controls. The chemotherapy treatment resulted in decreased nucleic acids, total carbohydrates and cholesterol contents to a remarkable extent in both B and T lymphoma patients.© (2001) COPYRIGHT SPIE--The International Society for Optical Engineering. Downloading of the abstract is permitted for personal use only.

Congenital dyserythropoietic anemia (CDA) is a rare group of inherited bone marrow disorders characterized by anemia with ineffective erythropoiesis. We report 3 siblings from a family known to have CDA type I who presented with persistent pulmonary hypertension of the newborn (PPHN). We suggest that the diagnosis of CDA type I should be considered in any neonate with PPHN and anemia.

Factors II, VII, IX· and X, and proteins C and S are synthesized in the liver by a process that is dependent on vitamin K. When vitamin K is deficient, hemorrhagic disturbances can occur. Hyphema has not been previously described in patients with vitamin K deficiency and consequent low levels of factors VII, IX and X. We describe a case of traumatic subto­ tal hyphema in a child in whom further investigation revealed deficiency of vitamin K-dependent factors. CASE REPORT

The effects of an intravenously administered lipid emulsion supplemented with γ-linolenic acid on the fatty acid profile of premature infants were compared with those of two conventional lipid emulsions. Fifty-nine premature neonates receiving total parenteral nutrition were randomly assigned to receive either fat emulsion containing γ-linolenic acid and long-chain triglycerides (LCT), an LCT emulsion, or a 50% (wt/wt) mixture of medium-chain triglycerides and LCT emulsion. Forty-nine infants completed the study. During the 6-day study there was a significant tenfold increase in the plasma levels of γ-linoleic acid in the supplemented group versus the other two groups. A significant threefold to fivefold increase in the ω6 long-chain polyunsaturated fatty acids was observed in all groups. These changes seemed to be attributable mostly to linoleic acid from the lipid emulsion, despite the 50% lower dose in the medium- and long-chain triglycerides group. The increase in the ω3 long-chain polyunsaturated fatty acids also was mainly caused by a similar increase in the level of α-linolenic acid. No differences were recorded in the linoleic/α-linolenic acid ratio among the groups. Plasma levels of some of the semiessential fatty acids were significantly higher in the medium- and long-chain triglycerides group than in the LCT group. This may be related to slower elimination of LCT, to the difference between emulsions, or to less substrate inhibition on Δ-6-desaturase, which seems to be less of a rate-limiting enzyme than previously considered. Further intravenous feeding trials are needed to identify the optimal balance of fatty acids for nutrition of these premature infants. (J P EDIATR 1994;125:596-602)

Abstract 4533 Background Identifying and treating iron deficiency anemia (IDA) is recommended by the American Academy of Pediatrics, the CDC and a U.S. Preventive Services Task Force to prevent various long-term squeals such as delayed mental and psychomotor development. As the prevalence of IDA and Thalassemia is high in Israel, the ministry of health has instituted that all infants of 4-12 months of age be supplemented with an iron preparation at 3 mg/kg/day. Iron deficient infants are treated with 6mg/kg/day iron, and rechecked at 3 months. Aim of Study To determine the prevalence of IDA in infants in the second year of life Methods Study population consists of 1818 toddlers between 12 to 24 months, from eight clinics in the southern (Negev) district of Israel; four clinics of the Bedouin sector (1167 toddlers) and four of the Jewish sector (651 toddlers) Samples were collected during 2006 and 2007 from otherwise healthy individuals, as recommended by the ministry of health. Toddlers were eligible unless previously known to be suffering from a congenital anemia disorder. Anemia was defined as MCV < 70. Iron deficiency was defined by an increment in MCV at a repeat test within 6 months or a ferritin level below norm. Results Of the 968 males and 850 females (Mean age 16.9±3.4 months), 505 (27.8%) toddlers were found to be anemic. The prevalence of anemia was significantly higher amongst Bedouin toddlers, (37.5% vs. 10.3% in Jews, p=0.001), and Male gender (30.5% vs. 24.7% in females, p=0.006), highly significant for Jewish males (14.6% vs. 5.5% in Jewish females p One hundred thirteen (22.3%) anemic infants had a follow-up test (average time 4.4 months), 81 of them had an improvement in MCV and 5 had a low ferritin level. The group of toddlers with IDA was determined at 76.1% of the anemic children. We found no significant difference in the execution of a follow-up test between sectors and genders. However, toddlers that underwent a second test were significantly younger, by an average of 3 months (p No differences in demographics gender, age or blood indices were found between the groups of infants who repeated the test and those without further data. Of the total sample population 20.1% were defined as IDA. Multivariate analysis of the anemia model revealed a significantly higher risk for developing IDA in the second year of life for Bedouin toddlers (27%) compared to Jewish toddlers (7.4%; OR: 3.7; 95% CI: 2.482-5.468), and for male toddlers compared to female. Conclusions A cost-effective screening program to prevent and treat IDA and other anemias in a high-risk population is cost effective and easily executed. Physicians need to be aware of IDA in order to implement an effective screen. With an overall prevalence of more than 1 in 5 infants suffering from IDA, and the very low probability of iron overload, our results clearly support the need to continue iron supplementation in the second year of life. Disclosures: No relevant conflicts of interest to declare.

The possible beneficial effects of infusing a lipid emulsion containing 50% by weight of medium-chain triglycerides (MCT) compared with a standard long-chain triglyceride (LCT) emulsion were studied in 18 premature neonates (gestational age less than 34 weeks) requiring parenteral nutrition. The infants were assigned in a double-blind manner to receive one of the two lipid emulsions over 17 hours a day as a supplemental regimen for total parenteral nutrition. A lipid load of 1 g/kg per day was initiated on the third day of life and was increased at the rate of 1 g/kg per day until a maximal dose of 3 g/kg per day was obtained on the fifth day of life and maintained thereafter. Both bound and unbound bilirubin decreased with both infusion regimens during the study period. Despite a marked increase in plasma free fatty acid levels (260% in the MCT/LCT group compared with 210% in the LCT group), the fraction of unbound (free) bilirubin was significantly lower in the MCT/LCT group (34% vs 13%). Free fatty acid levels, corrected to albumin, were positively correlated to the percentage of free bilirubin only for the LCT lipid infusion. The finding of a significant elevation of plasma cholesterol levels only in the MCT/LCT group is now under investigation. Use of the MCT-containing emulsion was not associated with a higher frequency of adverse effects than the commonly used LCT-containing emulsion.(ABSTRACT TRUNCATED AT 250 WORDS)

Asparaginase (ASP) is an effective antileukemic agent. However, hypersensitivity and/or allergic reactions (HA), estimated to occur in 2.5%–45% of patients are major limitations for its clinical use. The risk of developing hypersensitivity and/or an allergic reaction to ASP increases with higher doses, IV (vs. IM), continuous infusions and repeat treatment after prolonged abstinence. We surveyed the rate of HA in pediatric ALL in Israel, and identified additional factors of clinical significance. Methods: Data was gathered from the medical records of 245 children treated for ALL during the years 2000–2006 from 4 Pediatric Hematology-Oncology wards. All children were enrolled on the Israel National Leukemia Study 1998 or 2003, modified from BFM protocols. In Brief, patient received 8 doses of ASP in the induction and 4 additional doses in each reinduction, starting after a two-month period free of ASP. The uniform treatment used an E coli L-ASP preparation and was replaced with a pegylatedE coli ASP preparation in case of HA. The definition and severity of the HA was defined by the professional opinion of the treating medical team. Results: Forty-Eight of the 245 patients (19.6%) developed an HA reaction. 25 of the 48 were defined as severe. A wide range of HA was noticed between the treating centers (6.4%–29.4%). 16/48 of the children with HA were treated on a high-risk protocol (33% vs. 14% of the entire group, p Conclusions: The overall HA rate (with the noticed wide variations) and the high incidence at the beginning of the reinduction protocol are within the described literature, We believe that the lack of steroid treatment that precedes ASP in the reinduction contributes to the high rate of HA at this point. The findings regarding the PEG were unexpected. We found a relationship between the higher risk group and the development of HA as well as the severity. We believe that the differences observed between treating wards are due to unclear definitions of reaction and of severity .We have noticed a wide variability in executing the supporting treatment. We recommend a revision of the reinduction protocol to initiate steroids at the beginning of all treatment cycles and to consider the use of a different than E. coli source after a hypersensitivity or allergic reaction.

Flow cytomerty analysis of bone marrow (BM) is performed routinely in children with acute leukemia in different phases of chemotherapy. The aim of flow cytometric monitoring is to measure the amount of residual blasts and to evaluate the efficacy of chemotherapy. Multiparametric flow cytometry may be also used to evaluate normal hematopoisis in BM of patients with acute leukemia in different phases of chemotherapy. The aim of this study was to study normal lymphopoiesis in patients with relapsed disease. Twenty-two pediatric patients with ALL were enrolled in the protocol of study. Patients were treated in accordance with BFM-98 protocol. Eighteen children were in complete remission during follow-up period, while 4 children relapsed. Three of the relapsed children had B-lineage ALL, and one had T-cell ALL. Using multiparamertic flow cytometry we measured normal pro-B cells (CD19+CD34+), immature B (CD19+CD10+CD20+ and CD19+CD10−CD20+) and mature B (CD19+CD10−CD20+) cells in BM of relapsed patients in different phases of chemotherapy. Amounts or different subsets of B cells were compared with reference values determined in patients with relapse-free disease. Reference values were expressed as mean ± confidence interval for each subset of B cells and for each phase of chemotherapy. Elevation of immature normal B-cells was determined in all 4 patients who relapsed.Three during chemotherapy. Increased amount of mature B cells was determined 3 mo before the diagnosis of relapse in the patient who relapsed after the end of chemotherapy.This event was not observed in patient who did not relapse We conclude that elevation of normal immature B cells in BM of patient with ALL can be a predictive marker for eventual relapse.

Sleep fragmentation and periodic leg movement syndrome (PLMS) have been reported in adults with iron deficiency anemia. Little is known about sleep function and daytime sleepiness in children with chronic anemia such as beta-thalassemia or congenital dyserythropoietic anemia type 1 (CDA-1).To investigate if children and adolescents who have beta-thalassemia (major or intermedia) or CDA-1 experience sleep fragmentation and objective daytime sleepiness and also to investigate if children and adolescents with beta-thalassemia have obstructive sleep apnea.Ten patients (7 males and 3 females) with beta-thalassemia (mean [SD] age, 10.4 [7.3] years), 10 patients (7 males and 3 females) with CDA-1 (mean [SD] age, 13.5 [5.1] years), and 13 healthy volunteer control children(7 males and 6 females) (mean [SD] age, 10 [4] years) underwent nocturnal polysomnographic studies. A multiple sleep latency test was performed for 6 patients who had beta-thalassemia and 8 patients who had CDA-1.Both patient groups, that is, those who had beta-thalassemia and those who had CDA-1, had multiple arousals during sleep (mean [SD], 27.8 [11.4] events per hour and 23.8 [11.8] events per hour, respectively) compared with the control subjects (12.1 [6.6] events per hour) (P.002). Thirty-eight percent (10.6 events per hour) of the arousals in patients with beta-thalassemia and 25% (6.0 events per hour) of the arousals in patients with CDA-1 were induced by periodic limb movements during sleep. In the control group, most (98%) arousals were spontaneous and unrelated to any definable event. The multiple sleep latency test average was 7.8 minutes for patients with beta-thalassemia (n = 6) and 10.7 minutes for patients with CDA-1 (n = 8). Five patients with beta-thalassemia and 4 patients with CDA-1 underwent a second polysomnographic study on the next night to confirm reproducibility. There was no significant change in the total number or index of arousals and no difference in the severity of the periodic limb movements during sleep compared with the results of the first polysomnographic study.Children and adolescents with beta-thalassemia or CDA-1 have evidence of impaired sleep function that is partially due to periodic limb movements during sleep and arousals that result in objective diurnal sleepiness.