A study on the effects of pica and iron-deficiency anemia on oxidative stress, antioxidant capacity and trace elements.

Pica is defined as developmentally inappropriate consumption of nonnutritive substances for at least 1 month. There are a few studies on serum trace element levels of patients with pica. The literature contains contracting data on the levels of oxidative stress and antioxidant levels in patients with iron-deficiency anemia (IDA). The effect of pica on oxidative stress and antioxidant capacity has not been investigated yet. The present study evaluated the effects of pica and IDA on oxidative stress and antioxidant capacity as well as on the levels of trace elements including serum zinc and selenium in 47 children with IDA plus pica, 22 children with IDA only and 21 nonanemic children as controls. The results demonstrated significantly lower levels of serum selenium and zinc in pica and IDA groups compared to the control group. Total oxidant levels were highest in the pica group and consistently, the lowest total antioxidant capacity was observed again in the pica group. Comparison of pica and IDA groups yielded significantly lower levels of total antioxidant levels and significantly higher oxidative stress index in the pica group. Consequently, it is thought that the detrimental effects of pica within the organism were mediated by adverse impacts on antioxidant capacity and oxidative stress. These effects should be kept in mind while managing patients with pica.

Efficacy of FEIBA for acute bleeding and surgical haemostasis in haemophilia A patients with inhibitors: a multicentre registry in Turkey.

Long used in established industrialized nations to treat patients with haemophilia and inhibitors, factor eight inhibitor bypassing activity (FEIBA) has, in recent years, been introduced into more geographically diverse settings. Data are needed on how successfully FEIBA therapy has been implemented in new regions. To determine the efficacy and safety of FEIBA for the treatment of acute bleeding and surgical haemostasis in a newly industrialized country. A multicentre registry of haemophilia A patients with inhibitors receiving FEIBA treatment was established in Turkey. With a standardized case report form, data were collected retrospectively on: patient demographics; characteristics of acute bleeding episodes and surgical interventions; FEIBA regimen; and treatment outcomes. Thirty-seven patients received a total of 112 FEIBA treatment courses, 90 for acute bleeding and 22 for surgical haemostasis. The median FEIBA dose per infusion for acute bleeding was 50 IU kg(-1), and for surgery was 100 IU kg(-1). For both acute joint and muscle/soft tissue bleeding and in surgery, haemostasis was attained in a median of two FEIBA infusions. FEIBA was judged effective in 92% of treatment courses for acute bleeding, with a 95% confidence interval (CI) of 85-97%. Rates of haemostatic efficacy did not differ significantly between anatomical sites of acute bleeding. The haemostatic efficacy rate of FEIBA in surgery was 86% (CI, 65-97%). No thromboembolic complications or other adverse events occurred during any treatment course. FEIBA has been successfully integrated into clinical practice in Turkey, with rates of haemostatic efficacy comparable to those reported in countries with a longer history of FEIBA usage.

Central nervous system involvement in a case of familial hemophagocytic lymphohistiocytosis with perforin mutation.

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive lethal condition characterized by fever, cytopenia, hepatosplenomegaly and hemophagocytosis. The presence of central nervous system involvement has a profound impact on the prognosis, treatment, and clinical outcome of the disease. Therefore, the identification of the clinical manifestations of the disease and the characterization of the accompanying neurological symptoms are of prime importance for the rapid diagnosis and subsequent clinical management of the disease. Herein, we report a case of FHL with homozygosity for perforin gene mutation, who presented with central nervous system involvement in the absence of systemic findings.

Ceftriaxone induced thrombocytopenia in a child.

A variety of diseases, hereditary conditions, toxins and drugs may cause thrombocytopenia. Thrombocytopenia induced by ceftriaxone has been rarely reported. In this case, ceftriaxone-induced thrombocytopenia is presented in a 2-year-old girl due to rare presentation (Ref. 10). Full Text in free PDF www.bmj.sk.

Serosurveillance study on transmission of H5N1 virus during a 2006 avian influenza epidemic.

In 2006 an outbreak of avian influenza A(H5N1) in Turkey caused 12 human infections, including four deaths. We conducted a serological survey to determine the extent of subclinical infection caused by the outbreak. Single serum samples were collected from five individuals with avian influenza whose nasopharyngeal swabs tested positive for H5 RNA by polymerase chain reaction, 28 family contacts of the cases, 95 poultry cullers, 75 individuals known to have had contact with diseased chickens and 81 individuals living in the region with no known contact with infected chickens and/or patients. Paired serum samples were collected from 97 healthcare workers. All sera were tested for the presence of neutralizing antibodies by enzyme-linked immunoassay, haemagglutination inhibition and microneutralization assays. Only one serum sample, from a parent of an avian influenza patient, tested positive for H5N1 by microneutralization assay. This survey shows that there was minimal subclinical H5N1 infection among contacts of human cases and infected poultry in Turkey in 2006. Further, the low rate of subclinical infection following contact with diseased poultry gave further support to the reported low infectivity of the virus.

Inappropriate antimicrobial use in Turkish pediatric hospitals: a multicenter point prevalence survey.

OBJECTIVES: Although well-defined principles of rational antimicrobial use are available, inappropriate prescribing patterns are reported worldwide. Accurate information on the usage of antimicrobials, including factors associated with and influencing their use, is valuable for improving the quality of prescription practices. METHODS: In this cross-sectional point prevalence survey, data on patients hospitalized in 12 different children's hospitals were collected on a single day. Appropriateness of prescription was compared between the types of antimicrobials prescribed, indications, wards, and presence of/consultation with an infectious disease physician (IDP). RESULTS: A total 711 of 1302 (54.6%) patients evaluated were receiving one or more antimicrobial drugs. The antimicrobial prescription rate was highest in pediatric intensive care (75.7%) and lowest in the surgery wards (37.0%). Of the 711 patients receiving antimicrobials, 332 patients (46.7%) were found to be receiving at least one inappropriately prescribed drug. Inappropriate use was most frequent in surgery wards (80.2%), while it was less common in oncology wards (31.8%; p<0.001). Respiratory tract infection was the most common indication for antimicrobial use (29.4%). Inappropriate use was more common in deep-seated infections (54.7%) and respiratory infections (56.5%). Fluoroquinolones were used inappropriately more than any other drugs (81.8%, p=0.021). Consultation with an IDP appears to increase appropriate antimicrobial use (p=0.008). CONCLUSIONS: Inappropriate antimicrobial use remains a common problem in Turkish pediatric hospitals. Consultation with an IDP and prescribing antimicrobial drugs according to microbiological test results could decrease the inappropriate use of antimicrobials.

The frequency of consanguineous marriage in eastern Turkey.

UNLABELLED: The frequency of consanguineous marriage in Eastern Turkey: OBJECTIVE: The rate of consanguineous marriage (CM) varies depended on different factors such as race, characteristics of population, and religion and moral features in different countries. Gene frequency and genetic structure are changed by CMs. The aim of the present study is to assess the prevalence of CM and its effects on miscarriage, stillbirth, congenital malformation and ratio of newborn death. METHODS: This study was performed in Van region, Eastern Turkey, between September 2005 and April 2006. A total of 650 families from 24 districts chosen in accordance with the number of inhabitants were included in this study. First cousin marriages were accepted as a first degree CMs, sesquialter and second cousin marriages as second degree and marriages between distant relatives were accepted as a third degree CM. Monthly income of the families was classified in accordance with minimum wage determined by government. RESULTS: Of all families, 224 (34.4%) had CM, and 168 (75%) had first-degree consanguinity. A lower CM rate was found in mothers who graduated from secondary school or upgrading (p < 0.01). However, no relationship was found between CM and fathers' education level. While a low CM rate was found in families who had two or less children (p < 0.01), high rate was observed in families who had five or more children. In addition, a high rate of miscarriage, stillbirth and mental-motor retardation was found in families with CM (p < 0.05). The rate of child mortality between the aged 0-2 years was found to be higher in families with CM (p < 0.01). The higher CM rate was observed in families who married due to pressure or insistence of their families than married voluntarily (p < 0.05). CONCLUSION: Our study showed that CM rate was very high, 34.4%, in our region Eastern Turkey.

Evaluation of lymphocyte subgroups in children with subacute sclerosing panencephalitis.

The aetiology of subacute sclerosing panencephalitis (SSPE) remains to be fully elucidated, although it follows infection with a hypermutant defective M-protein measles virus. This study analysed peripheral blood lymphocyte subgroups to determine their role in the pathophysiology of SSPE. It included 22 children with SSPE aged 2 - 15 years (patient group) and 22 age- and gender-matched healthy children (control group). In children < 6 years old, there were no statistically significant differences between the two groups in the proportions of lymphocytes expressing the surface markers CD3, CD8, CD19 or CD16/56, or in CD4/CD8 ratio. The proportion of CD4(+) lymphocytes in SSPE patients < 6 years of age was significantly lower compared with the control group. In children >or= 6 years old, there were no significant differences in the lymphocyte subgroups. In conclusion, these findings suggest that a low CD4(+) lymphocyte count might be responsible for SSPE in younger children.

Acute respiratory distress syndrome due to overdose desferrioxamine: report of a child.

In this article, we present an 18-month-old girl with acute iron poisoning who died from acute respiratory distress syndrome due to overdose of desferrioxamine. Our purpose is to emphasize the importance of close follow-up children with acute iron poisoning for desferrioxamine toxicity.

Benefit of high-dose methylprednisolone in comparison with conventional-dose prednisolone during remission induction therapy in childhood acute lymphoblastic leukemia for long-term follow-up.

Eight-year event-free survival (EFS) was evaluated in 205 patients with acute lymphoblastic leukemia (ALL), to consider the efficacy of high-dose methylprednisolone (HDMP) given during remission induction chemotherapy between 1 and 29 days. The St Jude Total XI Study protocol was used after some minor modifications in this trial. Patients were randomized into two groups. Group A (n = 108) received conventional dose (60 mg/m(2)/day orally) prednisolone and group B (n = 97) received HDMP (Prednol-L, 900-600 mg/m(2) orally) during remission induction chemotherapy. Complete remission was obtained in 95% of the 205 patients who were followed-up for 11 years; median follow-up was 72 months (range 60-129) and 8-year EFS rate was 60% overall (53% in group A, 66% in group B). The EFS rate of group B was significantly higher than of group A (P = 0.05). The 8-year EFS rate of groups A and B in the high-risk groups was 39% vs 63% (P = 0.002). When we compared 8-year EFS rate in groups A and B in the high-risk subgroup for both ages together /=10 years, it was 44% vs 74%, respectively. Among patients in the high-risk subgroup with a WBC count >/=50 x 10(9)/l, the 8-year EFS was 38% in group A vs58% in group B. During the 11-year follow-up period, a total of 64 relapses occurred in 205 patients. In group A relapses were higher (39%) than in group B (23%) (P = 0.05). These results suggest that HDMP during remission-induction chemotherapy improves the EFS rate significantly for high-risk patients in terms of the chances of cure.

Ophthalmo-acromelic syndrome in a Turkish infant: case report.

Ophthalmo-acromelic syndrome (OAS) is an extremely rare autosomal recessive disorder characterised by eye malformations ranging from true anophthalmia to mild microophthalmia and acromelic malformations. In this article, we report a newborn infant with OAS because of its rare presentation. He was the fourth sibling affected in the family. The parents were healthy but there was a close blood relationship between the parents. Physical examination revealed bilateral true anophthalmia and oligodactyly (bilateral four toes) on the feet. He had no other additional abnormalities. We consider that this rare syndrome could be relatively more common in our country because six Turkish cases of OAS have been reported in the English literature to date.

Platelet functions in patients with protein-energy malnutrition.

Protein-energy malnutrition (PEM) has been known to cause several organ damages or dysfunctions. In this study, using optic method, platelet aggregation (PA) analyses were performed in patients with PEM. Adenosine diphosphate (ADP)- and collagen-induced PA were significantly decreased in the patients with PEM. The low values reached normal when the patients caught up the optimal growing curve for their age. PA with ristocetin and epinephrine were not significantly altered in the patients. Conversely, mean platelet count was increased at the beginning compared to those of after therapy and to the control. These results indicate that some platelet functions (ADP- and collagen-induced platelet aggregations), as well as other body functions, have been decreased in PEM.

Relapse in hairy cell leukemia due to isolated nodular skin infiltration.

Leukemic skin infiltration is quite uncommon in certain types of leukemia. Here, a child with hairy cell leukemia who developed isolated skin infiltration during remission is reported. The failure to diagnose the leukemic infiltration until the nodule reached a diameter of 2 cm is emphasized.

Immunoglobulin subgroups in children with febrile seizures.

BACKGROUND: The aim of the present study was to determine whether or not there was a role for immunoglobulin (Ig) or IgG subgroups in the pathogenesis of febrile seizures (FS). METHODS: Serum levels of IgA, total IgG, IgM, IgE, IgGI, IgG2, IgG3 and IgG4 were measured in 34 children with FS and in 37 healthy children used as a control group. Both patients and controls were divided into two groups according to age (group I, 6-24 months; group II, 25-72 months). RESULTS: Compared with controls, mean IgG4 levels in patients were found to be decreased in both groups I and II (group I: 95 +/- 14 vs 57 +/- 5, respectively, P = 0.01; group II: 178.5 +/- 38.5 vs 65.1 +/- 24.5, respectively, P < 0.01), while mean IgG2 levels were found to be decreased only in group II patients (170 +/- 16 vs 103 +/- 22; P < 0.05). CONCLUSIONS: The results of the present study suggest that Ig subclass deficiencies may be responsible for the infections connected with FS or that they may be related to the pathogenesis of FS in some children.

Beta-Thalassemia Mutations in the East of Turkey.

This study was planned to determine the frequency of ß-thalassemia trait and mutations in Van Lake region, which is in the eastern part of Turkey, surrounded by Iran in the east and by Iraq in the south. This study consist of 1014 healthy students, between the age of 12 and 18 years who are studying in boarding schools in Van city and cities surrounding Van Lake that includes Van, Agri, Hakkari, Bitlis, Mus and Siirt. The students were randomly selected according to their school numbers. Their origin was not taken into consideration in selection. Complete blood counts were performed on all donors. The accepted lower limit of mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) were 78 fL and 27 pg respectively. HbA2 was measured by DE-52 microcolumn chromatography method. ß-thalassemia trait with high HbA2 was found in 6 children. ß-thalassemia homozygous form of -30 (T-A) mutation was determined in one child and HbD-Los Angeles heterozygous in another. The ß-thalassemia homozygous individual was accepted as two cases in prevalence calculation. We found that the prevalence of ß-thalassemia trait was 0.78% in this area. In DNA analyses, there were ß-thalassemia gene mutations of FSC 8/9 (+G), -30 (T-A), IVS I-110 (G-A), IVS II-1 (G-A) and IVS I-130 (G-A) in 3, 2, 1, 1 and 1 individuals, respectively. We conclude that although five different ß-thalassemia mutation exist in Van Lake region, ß-thalassemia is not a potential risk in the east of Turkey.

T-ALL with monoclonal gammopathy and hairy cell features.

The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL) with hairy cell (HC) features and monoclonal gammopathy. The disease process had an acute onset and followed a rapid, progressive course. The patient had minimal splenomegaly and bicytopenia, but the bone marrow displayed increased numbers of reticulin fibers. The blasts were positive for tartrate-resistant acid phosphatase (TRAP) and CD11c. Molecular analysis revealed rearrangement of immunoglobulin heavy chain genes and a rearranged T-cell receptor (TcRbeta) beta gene. The patient responded to conventional ALL therapy. Acute T-cell ALL with HC features in childhood has not been reported previously, either alone or in association with monoclonal gammopathy. We propose "T-ALL with hairy cell features" to describe this case.