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Biomedical subjects

H Oren

Publications and source records attributed to H Oren.

At least 19 recordsLinked to original sources

Disseminated intravascular coagulation in pediatric patients: clinical and laboratory features and prognostic factors influencing the survival.

Although disseminated intravascular coagulation (DIC) has been a well-known disorder for many years, there is lack of sufficient number of clinical trials about incidence, frequency of underlying disorders, and prognosis of DIC in children. The aim of this study was to evaluate the frequency, etiologic factors, and clinical and laboratory findings of DIC and to determine the prognostic factors influencing the mortality in hospitalized pediatric patients. Medical records of 5535 children who were hospitalized were investigated. Sixty-two patients who were diagnosed as acute DIC were enrolled. The frequency of DIC was 1.12%. The underlying etiologic factors were infection in 59 patients (95.2%) and major trauma in 3 patients (4.8%). The frequency of bleeding and thrombosis was 48.8 and 4.8%. Respiratory, cardiovascular, hepatic, renal, neurologic, and gastrointestinal dysfunction was present in 71, 67.7, 35.5, 16.1, 16.1 and 11.3% of patients, respectively. Respiratory and cardiovascular dysfunctions were significantly associated with mortality. Multiorgan dysfunction syndrome (MODS) was present in 85.5% of the patients, and 54.8% of the patients had developed acute respiratory distress syndrome (ARDS). Mortality rate was significantly high in patients with MODS and ARDS. In multivariete logistic regression analysis, only ARDS and cardiovascular dysfunction had predictive and prognostic value on mortality. None of the diagnostic laboratory tests had predictive or prognostic value and the degree of abnormality of these tests did not show any correlation with mortality. In conclusion, DIC is not a rare disorder in hospitalized children, especially in patients with sepsis, and MODS, ARDS, and respiratory and cardiovascular system dysfunctions are poor prognostic factors.

Acute Disease↗

Dermal bilirubin kinetics during phototherapy in term neonates.

AIM: To investigate dermal bilirubin kinetics during phototherapy in the presence of neonatal indirect hyperbilirubinaemia. METHODS: 33 neonates with non-haemolytic indirect hyperbilirubinaemia, who required phototherapy, were included in the study. Phototherapy modules containing four normal and four blue fluorescent lamps were used during the study. The transcutaneous bilirubin index (TcBI) was measured in an area of the forehead covered by a 2.5 cm diameter opaque patch and a nearby exposed site. The TcBI obtained from patched and unpatched areas and simultaneous serum bilirubin (SB) concentrations were measured before the start of phototherapy and after 6, 12, 18, 30, 42 and 66 h of phototherapy. RESULTS: SB concentration and the TcBI from the unpatched area decreased significantly during the first 6 h of exposure, while the TcBI obtained from the patched area decreased significantly after 12 h. The TcBI from the unpatched area was consistently lower than that from the patched area during phototherapy. After the onset of phototherapy, there was a weak, non-significant correlation between SB concentrations and the TcBI from patched and unpatched areas. CONCLUSION: Phototherapy was effective for both patched and unpatched areas, but the rate of decline was slower in patched areas, only becoming significant in the second 6 h of treatment. There was no significant correlation between the levels of SB and TcBI after the onset of phototherapy, and therefore the use of TcBI cannot be recommended as a surrogate measure of SB.

Bilirubin↗

Analyses of polymorphism for UGT1*1 exon 1 promoter in neonates with pathologic and prolonged jaundice.

In this study, we investigated whether a TATA box polymorphism in the promoter of the UGT1*1 exon I, the most common detected DNA polymorphism in Gilbert's syndrome, is a contributory factor in unexplained pathologic or prolonged jaundice. 38 neonates who had unexplained pathologic jaundice, 37 neonates who had unexplained prolonged jaundice, and 35 healthy, nonjaundiced neonates were enrolled in the study. Genotypes were assigned as follows: 6/6 (homozygous for a normal allele bearing the sequence [TA](6)TAA), 7/7 (homozygous for an abnormal allele with the sequence [TA](7)TAA), and 6/7 (heterozygous with one of each allele). Of the 110 infants, 10 (9%) had 7/7, 51 (46%) had 6/7, and 49 (45%) had 6/6 genotype; the differences between the three groups were not statistically significant. Also no differences were observed among different genotypes and mean serum total bilirubin concentrations. In conclusion, we showed that TA 7/7 and TA 6/7 genotypes are not rare in our population and that the presence of these polymorphisms alone does not play a significant role in the etiology of unexplained pathologic or prolonged neonatal hyperbilirubinemia.

Alleles↗

Treatment outcome of adolescents with acute lymphoblastic leukemia.

Despite intensified chemotherapy, adolescents with acute lymphoblastic leukemia (ALL) still have lower rates of survival than younger children. The purpose of our study was to compare the treatment outcome and presenting clinical and laboratory features of adolescent and younger children with newly diagnosed ALL who were treated at our pediatric hematology department. Between April 1991 and February 2000, 42 children up to 18 years of age who were newly diagnosed with ALL and treated adequately with modified ALL Berlin-Frankfurt-Münster (BFM) 90 or 95 protocols were included in this study. The patients were examined in two groups according to their ages: the first group consisted of children who were <14 years old and the second group consisted of adolescents who were >14 years old. The median age of 42 patients was 6.5 years (range: 1-16.5 years); 26% of the patients were adolescents. The results of this study demonstrated that after a median observation time of 6 years the overall survival (OS) and event-free survival (EFS) of patients who were <14 and >14 years of age were 75% vs 49% and 70% vs 40%, respectively. When adolescent and younger patients were compared to each other according to gender, WBC count at administration, French-American-British (FAB) classification, immunophenotypes, risk groups, early deaths, and relapse rates, there were no statistically significant differences. Comparative data from other studies and data from this study indicate that adolescents with ALL still have shorter OS and EFS than younger children and a steady improvement in treatment outcome for adolescents with ALL over time suggests that more intensive therapy favorably influences prognosis.

Adolescent↗

Autoimmune hemolytic anemia occurring with myelodysplastic syndrome: report of a pediatric case and review of the literature.

In this case report, we present a child who was admitted to hospital with the features of autoimmune hemolytic anemia (AIHA) and was diagnosed with myelodysplastic syndrome (MDS)-related AIHA. A 14-year-old female patient was admitted to our hospital with the chief complaints of palpitation, icterus, and fatigue for 2 months. She was pale and icteric. Diffuse hepatosplenomegaly was palpated. Hematological examination revealed a hemoglobin of 3.4 g/dl, red blood cell count of 2x10(12)/l, white blood cell count of 3x10(9)/l, platelet count of 14x10(9)/l, and reticulocyte count of 1.7%. Blood smear examination revealed significant anisocytosis, poikilocytosis, and tear drop cells. The direct Coomb's test was positive. Bone marrow aspirate showed hypercellularity, micromegakaryocytes, dyserythropoiesis, and dysmyelopoiesis with 2% blasts. The patient was diagnosed with MDS-refractory anemia and AIHA secondary to MDS. Rarely, AIHA can occur secondary to MDS. To our knowledge, this patient is the first pediatric case with MDS and AIHA reported in the literature.

Adolescent↗

Association between serum macrophage colony-stimulating factor levels and monocyte and thrombocyte counts in healthy, hypoxic, and septic term neonates.

OBJECTIVE: Macrophage colony-stimulating factor (M-CSF) is a hematopoietic growth factor that mainly stimulates the growth, differentiation, and proliferation of cells of the monocyte-macrophage lineage. There are only limited numbers of studies about M-CSF levels in neonates, but high levels of serum M-CSF have been reported in septic and some thrombocytopenic adult patients. In this study, we investigated the serum M-CSF levels in healthy, septic, and hypoxic term neonates on the first day of life and examined the relationship of serum M-CSF levels and circulating monocyte and thrombocyte counts in these newborn infants. STUDY DESIGN: Three groups were defined in this prospective study: group 1, healthy neonates with no risk factors (n = 40); group 2, neonates who had severe hypoxia (n = 20); and group 3, neonates who fulfilled the criteria for early-onset sepsis (n = 18). Blood samples were collected for complete blood cell count and serum M-CSF levels by peripheral venipuncture from each infant in the first 24 hours after birth before any medical therapy. RESULTS: The gestational ages and birth weights did not differ significantly between the groups. Serum M-CSF levels of the septic neonates were significantly higher than of both healthy and hypoxic neonates, but did not differ significantly between the healthy and hypoxic neonates. There was no significant correlation between serum M-CSF levels and circulating monocyte counts, but there was a significant inverse correlation between serum M-CSF levels and thrombocyte counts. When this correlation was analyzed according to groups, we determined that this inverse correlation between M-CSF levels and thrombocyte counts was especially significant in the septic neonate group, but not significant in the healthy and hypoxic neonate groups. CONCLUSIONS: Serum M-CSF levels are significantly higher in neonates with sepsis. High serum M-CSF levels may have a possible role in the pathogenesis of thrombocytopenia in neonates with sepsis.

Asphyxia Neonatorum↗

Urinary nitrite excretion after prophylactic intravenous immunoglobulin in premature infants.

AIMS: To investigate the correlation between the prophylactic administration of intravenous immunoglobulin (IVIG) to preterm infants and urinary nitrite levels, which can be utilized as an index of endogenous nitric oxide (NO) formation, and to determine if NO formation plays a role in both therapeutic and adverse effects of IVIG. METHODS: 28 healthy preterm infants were included in this prospective study. They had a mean gestational age of 29.4 +/- 2.2 weeks and weight of 1,387 +/- 371 g. Prophylactic IVIG infusion at a dose of 0.5 g/kg/day was administered when they were 3-10 days old. Urine samples of the neonates were obtained for analysis on days 1, 2 and 3 after IVIG administration as well as 1 day before. Urinary nitrite levels obtained in the subjects were normalized for urinary creatinine concentrations. RESULTS: The mean urinary nitrite levels were: 2.77 +/- 1.66 micromol/mmol creatinine before IVIG administration; 4.33 +/- 3.88 micromol/mmol creatinine on the 1st day of IVIG; 3.77 +/- 2.73 micromol/mmol creatinine on the 2nd day, and 3.64 +/- 3.28 micromol/mmol creatinine on the 3rd day. There was a significant increase in urinary nitrite levels between before and after IVIG administration. There was no statistical difference in urinary nitrate levels between days 1, 2 and 3 after IVIG administration. CONCLUSION: We demonstrated that urinary nitrite excretion is significantly elevated in preterm infants after prophylactic IVIG administration and this result suggests that endogenous NO formation may play an important role in both the therapeutic and adverse effects of IVIG.

Birth Weight↗

Mutational analysis of beta-thalassemia cases from the Aegean region of Turkey using an allele-specific oligonucleotide hybridization technique.

The aim of the present study was to evaluate the point mutations of beta-thalassemia patients from the Aegean region of Turkey by using an allele-specific oligonucleotide hybridization technique. DNA isolated from peripheral blood samples of 75 children with beta-thalassemia major or intermedia was analyzed using a Bio-Rad mD(x)(TM)-Be Tha Gene 1 kit. We determined mutations in 56 (74.6%) patients. The allelic frequency of mutations in 150 chromosomes was as follows: IVS-I-110 (G-A) 44.1%, IVS-I-1 (G-A) 28.2%, IVS-I-6 (T-C) 13.3%, IVS-II-745 (C-G) 9.3%, IVS-II-1 (G-A) 2.7%, Cd 39 (C-T) 2.4%, -87 (C-G) 0% and Cd 6 (-A) 0%. The distribution of the mutation types was consistent with the findings of other research groups.

Alleles↗

Development of acute promyelocytic leukemia with isochromosome 17q after BCR/ABL positive chronic myeloid leukemia.

We describe a pediatric case of acute promyelocytic leukemia with an i(17q) after treatment of BCR/ABL positive chronic myeloid leukemia (CML) for 3.5 years. The patient was treated with Busulphan, alpha-2a interferon, hydroxyurea, and cytosine arabinoside at various times in the course of the chronic phase of CML, because he had no HLA-identical donor for bone marrow transplantation. Hematologic remission was achieved for a short time, but cytogenetic remission was never possible. When promyelocytic blast crisis was diagnosed according to the French-American-British classification, cytogenetic studies revealed an i(17q) as a new feature in our patient. The promyelocytic transformation was associated with the appearance of an i(17q) preceding CML are discussed in the light of recent literature.

Bone Marrow↗

Factor VIII inhibitors in patients with hemophilia A.

In 58 hemophilia A patients aged 1-18 years (mean 9.5 +/- 4.7 years), the prevalence of inhibitors was found to be 27% by the Bethesda method in November 1995. Inhibitor activity was not detected in any of 14 patients with mild hemophilia while it was present in 9 of 27 (33%) patients with moderate, and 7 of 17 (41%) with severe disease. During follow-up, the inhibitors were transient in 10 of 16 patients (17%) and the prevalence of inhibitors was 10% at the end of the study. Our study has demonstrated that the patients' age, factor VIII (F VIII) coagulant activity levels, type of F VIII replacement therapy, and frequency of F VIII administration affect inhibitor development, and these factors should be considered in the follow-up of hemophiliacs.

Adolescent↗

Recombinant alpha-2a interferon treatment in a child with T-cell leukemia and chronic hepatitis B.

We present the case of a 12-year-old boy with T-cell acute lymphoblastic leukemia (ALL) who developed a chronic hepatitis-B virus (HBV) infection during the consolidation phase of chemotherapy and was unable to receive further therapy because of hepatotoxicity. Recombinant alpha-2a interferon (alpha-IFN) treatment (5 million units/m2 per dose, three times a week) was started for chronic HBV infection at the end of the sixth month, and vincristine (1.5 mg/m2) was administered once a month as the only well-tolerated chemotherapeutic agent. During follow-up, the dose of alpha-IFN was increased to 10 million units/m2 three times a week, depending on the patient's laboratory data. Three months later, elimination of HBe Ag and HBV DNA and seroconversion from HBe Ag to HBe Ab occurred. The duration of alpha-IFN therapy was prolonged to 18 months, since other chemotherapeutic agents caused hepatotoxicity whenever they were tried and alpha-IFN treatment can be used in children with refractory T-cell leukemia in view of its antitumor effect. Our patient has now been in complete remission for 4 years. Alpha-IFN therapy should be considered as an alternative treatment for patients with T-cell ALL who cannot receive chemotherapy because of HBV infection or for any other reasons.

Antineoplastic Agents↗

Diffuse erythrodermic cutaneous mastocytosis with bone marrow infiltration.

We report a case of diffuse erythrodermic cutaneous mastocytosis with bone marrow infiltration. An 11-month-old female patient was referred to our hospital for intermittent flushing, fever, intense itching, erythematous rash and bullous lesions. Cutaneous biopsy demonstrated diffuse cutaneous mastocytosis. The bone marrow aspirate revealed mast cell infiltration. Ketotifen treatment was very effective.

Biopsy↗

Patient assessment of care in a pain management unit.

Seventy individuals, 40 females and 30 males with a mean age of 58.5, suffering from protracted pain and associated incapacitation (due, mainly, to rheumatoid or osteoarthrotic conditions) were treated as outpatients with the purpose of relieving their pain and improving their function. The quality of care provided was evaluated based on the outcome approach, using patient assessment of the reduction of their pain and improvement of their limitations--complemented by the assessment of the physician who treated them--and their satisfaction with the care as the measurements of their assessments. The results of the assessment indicate that on completion of treatment 23% of patients had no pain, while the pain level of the rest decreased by 64% with only three still suffering from severe pain and none from very severe pain. In the majority of patients considerable improvement of the associated functional limitation was achieved. There was a high degree of satisfaction with care received in 90% of patients.

Adult↗