Aberrant expression of insulin-like growth factor-2 (IGF-2) in Philadelphia chromosome negative chronic myeloproliferative disorders

Philadelphia chromosome negative chronic myeloproliferative disorders (Ph- CMPD) comprise haematopoietic stem cell disorders with currently unknown underlying molecular defect. Insulin-like growth factor 2 (IGF-2) is an imprinted gene that is known to be involved in the regulation of normal cell growth and that is overexpressed by a variety of tumors. The expression of IGF-2 in bone marrow cells is largely unknown. In order to elucidate gene expression level, protein expression pattern, and a potential role of IGF-2 in the pathogenesis of Ph- CMPD, we quantitatively analyzed the expression of the IGF-2 gene in bone marrow cells of 69 cases with Ph- CMPD and 31 control cases by applying real-time RT-PCR. IGF-2 gene expression in idiopathic myelofibrosis (IMF) was significantly increased by up to 11-fold as compared to the control group (P < 0.0001). IMF also expressed higher IGF-2 gene level as compared to essential thrombocythaemia (ET) and polycythaemia vera (PV) (P < 0.0001, P = 0.005, respectively). Paranuclear IGF-2 protein could be demonstrated in IMF, ET, and PV exclusively in megakaryocytes and myeloid progenitor cells in contrast to undetectable IGF-2 protein in control cases. We conclude that overexpression of the IGF-2 gene is a pathogenic feature in IMF. In addition, an abundant translational and post-translational processing could explain the accumulation of IGF-2 protein detectable in all Ph- CMPD entities in contrast to non-neoplastic haematopoiesis. We conclude that IGF-2 represents a new molecular target for evaluation of underlying fundamental pathomechanisms in Ph- CMPD.     

Prolonged hyperglycemia in the early subacute period after cerebral infarction: effects on short term prognosis

Although the adverse effect of admission hyperglycemia in cerebral infarction on prognosis is well known, studies generally have not questioned the effect of hyperglycemia in the early subacute period on prognosis after a stroke. Forty-six patients with acute ischemic stroke were seperated into 3 groups: Group 1) Known diabetes or admission blood glucose (ABG) > or = 140 mg/dl and HbA1c > or = 8,0%); Group 2) ABG > or = 140 mg/dl and HbA1c < 8,0%; and Group 3) ABG < 140 mg/dl and HbA1c < 8,0%. Blood glucose was followed-up 4 times a day for 10 days after the stroke and the mean of these measurements was calculated as the mean of glycemic regulation (MGR). Neurological evaluation was done at presentation and on day 10 and 30 with the National Institute of Health (NIH) scale. Oedema, lesion size and presence of hemorrhagic transformation were evaluated using CT. The MGR was significantly higher in group 1 compared to the other two groups (p < 0,001 and p < 0,01) and in group 2 compared to group 3 (p < 0,001). Patients with clinical worsening had a significantly higher MGR (p < 0,05). Patients with marked cerebral edema had a significantly higher MGR (p < 0,01) compared to patients with lesser edema. No correlation was found between MGR and lesion size or hemorrhagic transformation. Our results show that hyperglycemia in the early subacute period after cerebral infarction is associated with more pronounced cerebral edema and has an adverse effect on short term prognosis. We suggest that studies investigating the effect of insulin infusion on stroke prognosis should also consider infusions for a longer period than 24 hours.     

Byler's disease and anesthetic consideration

Byler's disease is an autosomal recessive condition characterized by intrahepatic cholestasis, progressive fibrotic changes and finally cirrhosis that leads to death during childhood. This is a report of a six-year-old girl with Byler's disease and retrobulbar hematoma as a result of trauma who underwent enucleation and implantation. This case report describes the anesthetic features of a patient with Byler's disease in which anesthetic agents with no or minimal hepatotoxic effect should be used to avoid deterioration of liver function.     

Effects of extended-wear soft contact lenses on the ocular surface and central corneal thickness

PURPOSE: New contact lens materials with high oxygen permeability are said to be convenient for the extended-wear (EW) schedule. We evaluated the short-term effect of EW soft contact lens usage on ocular surface and central corneal thickness. PATIENTS AND METHODS: Fifty-two eyes of 26 patients were included in the study. Sixteen of the patients used the high oxygen-permeable lenses for a 30-day extended-wear schedule (group I). Ten patients used the lenses on a daily wear basis (DW, group II). All patients were evaluated for visual acuity and central corneal thickness by corneal and conjunctival biomicroscopy, Schirmer and tear break-up time (BUT) tests. Impression cytology was performed in the superior/temporal, inferior/nasal quadrants of the conjunctiva, 1 mm from the limbus, with cellulose acetate filter paper. The specimens were graded under light microscopy according to Nelson's classification. RESULTS: Three patients in the EW group were excluded from the study as they discontinued lens usage due to slight discomfort and hyperemia. Biomicroscopic findings and tear function tests indicated no increased pathological results. Central corneal thickness decreased from 554 +/- 9.55 to 546 +/- 9.30 microm (mean +/- SEM) in the EW group (p = 0.002), whereas no significant change occurred in the DW group. Cytological evaluation of the EW group showed an 88% increase in metaplasia (p = 0.007), especially close to the limbal areas and in the superior quadrants; this ratio was 30% in the DW group. Snake-like chromatin changes were found to be increased, from 4 to 6 patients, in the EW group while it remained the same in the DW group (p > 0.05). Individual comparisons of the initial and final pathological conditions of the ocular surfaces in the two groups showed that there was an increase in pathology in the EW group while there was no significant difference in the DW group. CONCLUSIONS: On a 30-day extended-wear basis with high oxygen-permeable lenses, the possible development and increase of conjunctival cytologic changes secondary to mechanical trauma is one of the main remaining problems. Conjunctival and related immunologic pathologies should not be overlooked when prescribing an extended-wear lens schedule.     

Neonatal cerebral venous thrombosis coexisting with bilateral adrenal hemorrhage

We report a case of severe perinatal asphyxia with both cerebral venous thrombosis and adrenal hemorrhage who survived with severe sequela including multicystic encephalomalasia, acquired microcephaly and blindness. Hematological investigations showed normal levels of anticardiolipin antibodies, protein C and S levels and activity, antithrombin III levels. Factor V Leiden mutation was negative. The adrenal hemorrhage resolved within three months with glucocorticoid therapy, the cerebral venous thrombosis resolved within two months without treatment. The literature on neonatal cerebral venous thrombosis is also reviewed.     

Reference ranges for sonographic dimensions of the liver and spleen in preterm infants

Background

Preterm infants usually have multiple comorbidities that affect spleen and liver. Ultrasonographic measurement of organ sizes is an important and reliable parameter in evaluation of spleen and liver pathology in preterm newborns.

Objective

The purpose of this study was to determine reference values of ultrasonographic measurements of the liver and spleen in preterm newborns.

Materials and methods

We prospectively performed sonography on 498 preterm newborns in the first week of life. We measured spleen and liver dimensions and statistically analyzed relationships between the dimensions and gender, gestational age (based on mother’s last menstrual period), height and weight. Reference ranges of dimensions were defined.

Results

Longitudinal and anteroposterior dimensions of the liver and spleen were statistically significantly different between the boys and girls (P?<?0.05) and showed high correlation with the gestational age, weight and height. Weight was the parameter best correlated with the dimensions.

Conclusion

Nomograms from these data are useful for sonographic evaluation of the liver and spleen in preterm newborns.     

Cefuroxime-induced lupus

Drug-induced lupus erythematosus (DILE) is a syndrome that shares symptoms and laboratory characteristics with idiopathic systemic lupus erythematosus. Recognition of DILE is important because it usually reverts within a few weeks after stopping the offending drug. Antibiotics are uncommonly associated with DILE, and cefuroxime has never been incriminated as a cause. We present herein the first case of DILE induced by cefuroxime. Although this is the first report of cefuroxime-induced DILE, we should be aware of this occurrence.     

Effects of distractors on upright balance performance in school-aged children with attention deficit hyperactivity disorder,preliminary study

Introduction

Attention deficit hyperactivity disorder is a common impairing neuropsychiatric disorder with onset in early childhood. Almost half of the children with attention deficit hyperactivity disorder also experience a variety of motor-related dysfunctions ranging from fine/gross motor control problems to difficulties in maintaining balance.