Dimitrios Koliouskas

Methylenetetrahydrofolate reductase C677T polymorphism: Association with risk for childhood acute lymphoblastic leukemia and response during the initial phase of chemotherapy in greek patients

As of late, a number of studies have focused on the association of the gene for methyletetrahydrofolate reductase (MTHFR) with risk for acute lymphoblastic leukemia (ALL) in children and in adults, as well as with response to chemotherapy. The degree of this association may vary according to the ethnic background and geographic localization of the population under study, or the phase of treatment when response to chemotherapy is concerned.

ELECTRICAL POWER LINES AND CHILDHOOD LEUKEMIA: A STUDY FROM GREECE

Residential proximity to electrical power lines of different voltage in relation to childhood leukemia was investigated through a case-control study undertaken in Greece during 1993-1994. The study comprised 117 incident cases of childhood leukemia and 202 age-, gender- and place-of-residence-matched controls. Four measures of exposure to magnetic fields were developed, using data provided by the Public Power Corporation of Greece: Voltage (V) divided by the distance (d), V/d2, V/d3 and an adaptation of the Wertheimer-Leeper code. Conditional-logistic-regression modeling was used to adjust for potential confounding influences of 18 variables. No significant trends of childhood leukemia risk with increasing exposure levels were noted, nor were there statistically significant elevations of disease risk at the higher exposure levels in each measure of exposure. These results do not support a causal link between residential proximity to electrical high-voltage wires and childhood leukemia risk, but in themselves do not refute a weak empirical association.

Intestinal tuberculosis: a diagnostic challenge--case report and review of the literature.

Even though tuberculosis is considered rare in developed countries, its rising incidence, especially in high-risk populations, places intestinal tuberculosis in the differential diagnosis of patients with atypical abdominal symptoms or signs. We, herein, report the case of an immunocompetent woman, from a nonendemic area, who developed intestinal tuberculosis, emphasizing the diagnostic challenges caused due to nonspecific symptoms, inconclusive clinical, laboratory, and imaging findings, which could not rule in or rule out tuberculosis. Antituberculosis treatment was administered based on endoscopic findings and histological features of mucosal biopsies, which were indicative of intestinal tuberculosis, and the patient showed a marked clinical and laboratory improvement. We also review the evidence with regard to the diagnostic accuracy of the different available tests for intestinal tuberculosis.

Trends and geographical distribution of childhood leukemia in Greece in relation to the Chernobyl accident

Parts of Greece have been exposed to fallout radiation from the Chernobyl accident as much as any of the countries boardering with the former Soviet Union, because of the direction of the prevailing winds after the accident. Although fallout radiation did not reach levels expected to be associated with measurable effects, there is widespread concern in Greece that the incidence of childhood leukemia may be rising in the more heavily affected parts of Greece. Patient discharge data from all Greek hospitals treating childhood leukemia were used to calculate the annual incidence of the disease from January 1980 to June 1986 (preaccident period), from July 1986 to June 1988 (immediate postaccident period) and from July 1988 to June 1991 (“relevant” post-accident period, that accommodates the presumed latent period of the disease). Fallout radiation measurements (in Bq/kg Cs-137) were used to create 17 regions of similar (within regions) but highly variable (between regions) levels of fallout deposition. Background radiation (in Bq/kg Ra-226) and annual incidence of childhood leukemia by region were also estimated. There was no evidence of increased incidence of childhood leukemia during the immediate or the “relevant” post-Chemobyl period in any part of the country. Furthermore, regression analyses did not show any significant or suggestive association of childhood leukemia by region with either background or fallout radiation. These results indicate that the Chemobyl accident did not affect noticeably the incidence of childhood leukemia in Greece during the five-year post accident period.

Use of Tigecycline in Pediatric Patients With Infections Predominantly Due to Extensively Drug-Resistant Gram-Negative Bacteria

Background. Emergence of extensively drug-resistant (XDR) bacteria has forced clinicians to use off-label antimicrobial agents such as tigecycline. We present our experience on salvage use of tigecycline for the treatment of infections caused by XDR Gram-negative bacteria in critically ill children and review published cases. Methods. We conducted a retrospective chart review in pediatric departments of a tertiary level hospital from January 2009 to May 2014. Patients were identified using pharmacy database. For the literature review, relevant articles were identified from PubMed. Results. In our case series, 13 children (7 males) with a median age of 8 years (range, 2.5 months-14 years) received tigecycline for ≥2 days as treatment for healthcare-associated infections including 5 bacteremias, 6 lower respiratory tract infections, and 3 other infections. Isolated pathogens were XDR Gram-negative bacteria except 1. A loading dose (range, 1.8-6.5 mg/kg) was given in all except 2 cases. Maintenance dose was given at 1-3.2 mg/kg q12 h. Other antimicrobials including colistin and aminoglycosides (85% and 62%, respectively) were coadministered to all patients. No serious adverse events were detected in these very ill children. Twenty cases of children treated with tigecycline were previously published, mostly for multidrug-resistant/XDR bacteria. An episode of acute pancreatitis and neutrophil engraftment delay in 2 cases were reported during tigecycline treatment. Analyzing reported and all our cases together, mortality in bloodstream infections was 86%, whereas in nonbacteremic cases it was 24% (P = .009). Conclusions. Tigecycline, given at the range of administered doses as salvage therapy and in combination with other antimicrobial agents, seemed to be well tolerated in a series of mainly critically ill pediatric patients and demonstrated relatively good clinical response in nonbacteremic patients.

Lissencephaly and mongolian spots in hurler syndrome

Hurler disease or syndrome is a disorder of mucopolysaccharide metabolism, inherited as an autosomal recessive trait. We describe a case of a 15-month-old female exhibiting with clinical and laboratory characteristics of the syndrome, central nervous system lesions (lissencephaly, excessive ventricular enlargement and Dandy Walker malformation with vermis atrophy, cerebellar cyst) and mongolian spots in the trunk and extremities. The combination of mongolian spots and severe central nervous system lesions in Hurler syndrome is considered a rare clinical occurrence, while the association with lissencephaly has never been reported.

Russell’s diencephalic syndrome

A 21-month-old boy was initially admitted to our hospital because of failure to thrive, which began at the age of 6 months. Physical examination revealed emaciation (weight < third percentile), normal body length, normal head circumference with a characteristic “pseudohydrocephalic” face (figure, A), as well as a mild pyramidal tract dysfunction, …

INCREASED EXPRESSION OF MULTIDRUG RESISTANCE GENE (MDR1) AT RELAPSE IN A CHILD WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Modern treatment protocols lead to complete remission in a high proportion of patients with childhood acute lymphoblastic leukemia (ALL). However, a large number of them show a relapse of the disease. Treatment failure in these patients is mainly attributable to de novo or acquired resistance to a wide variety of cytotoxic drugs, which is called multi drug resistance (MDR). Expression of multi drug resistance 1 gene (MDR1) is implicated in the drug-resistance mechanism. In order to contribute further information we present a rare case of a 15-month old girl with newly diagnosed CALLA positive pre-B acute lymphoblastic leukemia with favourable prognostic factors at diagnosis who experienced a relapse of the disease. Using reverse transcriptase polymerase chain reaction method, m-RNA expression of the MDR1 gene upon relapse, was five-fold compared with that at diagnosis. This is the first report on increased mRNA expression at relapse in a paired sample of a child with ALL in our region.

4,4′-Methylenedianiline-induced hepatitis in an industrial worker: Case report and review of the literature

4,4′-Methylenedianiline (MDA) is a chemical used in manufacturing and insulation processes and is a well-known hepatotoxin. We report the case of a 42-year-old construction-site worker who was accidentally exposed to large amounts of MDA and developed acute liver damage. The clinical course is described, with particular emphasis on the timely identification of the underlying cause and prompt management that led to an uneventful recovery. We review the relevant literature and discuss the safety measures necessary to minimize similar occupational hazards in industrial workers.

A Case of Churg-Strauss Syndrome Revealed by Eosinophilic Gastroenteritis

Churg-Strauss syndrome (CSS) is a rare and usually underrecognized entity that often manifests late in the process of the disease. The syndrome is a small vessel-necrotizing vasculitis characterized by asthma, extravascular necrotizing granulomas, hypereosinophilia, and lung infiltrates [1]. According to the classification of CSS by the American College of Rheumatology, the presence of four or more of the criteria required to establish a diagnosis of CSS yield a sensitivity of 99.7% for CSS [2, 3] (Table 1). The annual incidence of the disease ranges between 0.5 and 6.8 per million inhabitants. The average age of the patient at diagnosis is 50 years; there is no sex predominance, and the annual incidence among asthma patients ranges from 0 to 97 per million [1]. The pathogenesis and etiology of CSS remain unclear, but the disease is most likely autoimmune, at least for those 30–40% anti-neutrophil cytoplasmic antibodies (ANCA)positive patients [4].