Clinical, electrophysiological and neuropsychological findings of twenty-two children with mesial temporal sclerosis

Twenty-two patients, followed with the diagnosis of epilepsy between the ages of 8.5 and 19 years who were found to have mesial temporal sclerosis (MTS) on brain magnetic resonance imaging (MRI) were evaluated according to their clinical, electrophysiological and neuropsychological characteristics, and assessed for the significance of neuropsychological tests on lateralization. In both MTS groups, a remarkable number of patients had their intial seizure before one year of age (45.5% in the right MTS group, and 36.4% in the left MTS group). Half of the patients were seizure-free for a period of two months-9.5 years (average 3.9 years). The duration between the febrile seizure and first non-febrile seizure was 6.2 years. Eleven patients had MTS on the left temporal lobe. Interictal EEG was normal in five (22.5%) patients; 10 (45.5%) had temporal spike on the corresponding side with MTS. Six (27.3%) patients had paroxysmal activity and voltage asymmetry on cortical areas other than temporal region. The Wechsler Memory Scale (WMS), Visual Reproduction Subtest and Auditory Verbal Learning Test (AVLT) Delayed Recall and Retrieval Subtest performances were poor in the right MTS group.     

Single-voxel MR spectroscopy and diffusion-weighted MRI in two patients with <Emphasis Type="SmallCaps">l</Emphasis>-2-hydroxyglutaric aciduria

l-2-Hydroxyglutaric aciduria is a rare inherited, neurometabolic disorder. The underlying metabolic defect and the pathophysiology of l-2-hydroxyglutaric aciduria have not yet been defined. We present MR spectroscopy and cranial MR imaging findings, including diffusion-weighted sequences in two male siblings (aged 10 and 12 years). MR spectroscopy revealed a multiplet at 2.10–2.50 ppm and two broad peaks at 0.9–1.6 ppm. The multiplet at 2.10–2.50 ppm might have been created by elevated glutamate and glutamine or l-2-hydroxyglutaric acid itself, which has a similar chemical structure to glutamate. Diffusion-weighted images demonstrated increased diffusion of water molecules in the white-matter lesions.     

Mosaic form (45X/46XX) of Turner's syndrome. A case report

BACKGROUND: Turner's syndrome (TS) is a sex chromosome disorder occurring in 1 in 2,500 female births and in approximately 50 in 100,000 adult females and is characterized by retarded growth, gonadal dysgenesis and infertility. The 45,X/46,XX chromosomal pattern is the most frequent mosaic type of this disease (36%). CASE: The mosaic form of TS was diagnosed in a 25-year-old, nulliparous woman whose major symptoms were menstrual irregularity and menorrhagia. She had normal development of secondary sexual characteristics and spontaneous menarche despite a very low amount (7%) of normal cells. CONCLUSION: Dysfunctional uterine bleeding is very uncommon in TS. Mosaic forms of TS may have very few features of TS despite a very low range of normal cells.     

Polycythemia vera in a 12-year-old girl: a case report

A case of a 12-year-old girl presenting with headache and splenomegaly and fulfilling the diagnostic criteria of polycythemia vera is reported. Her peripheral blood values were as follows: hemoglobin 18 g/dL, red blood cells 7,000,000/mm 3 , white blood cells 22,000/mm 3 , and platelets 1,248,000/mm 3 . Phlebotomy was performed initially but was ineffective. Afterward 100 mg/kg per day aspirin and 30/mg/kg per day hydroxyurea were given. The patient has been asypmtomatic for 1 year and her recent hemoglobin level is 15.5 g/dL, platelet count 922,000/mm 3 , and white blood cell 12,800/mm 3 . Polycythemia vera is an extremely uncommon disease in childhood and for this reason its treatment is not well established.     

Synthesis and antimicrobial activity of flavone-3'-carboxaldehyde oxime ether derivatives

A new series of flavonyl oxime ether derivatives (FO1-FO6) was prepared by reaction of flavone-3'-carboxaldehyde (III) with O-substituted hydroxyl amine derivatives (IV). The synthesized compounds were tested for their in vitro antifungal and antibacterial activities. All the compounds exhibited antimicrobial activity.     

Ureteral membranous band causing ureteral obstruction after the ureterolithotomy

A 45-year-old male admitted to our clinic of symptoms with moderate left flank pain and intermittant macroscopic hematuria. During the last 6 months these complaints had worsened. He had history of open retroperitoneal ureterolithotomy 2 years previously for ureteral stone. He was investigated for calculi, infection and other urinary abnormalities. Although direct urinary system graphy was normal, excretory urography revealed left ureteric obstruction. On ureteroscopy membranous band like an ureteral valve causing ureteral obstruction was observed and removed with biopsy pens without resection.     

The impact of serum oestradiol concentration prior to progesterone administration on live birth rate in single vitrified–warmed blastocyst transfer cycles

Research questionCan serum oestradiol concentrations on the day of progesterone initiation predict live birth rates in single, autologous vitrified–warmed blastocyst transfers following artificial endometrial preparation?DesignThis retrospective study included the first transfers of 468 patients with unexplained or tubal factor infertility who underwent freeze-all cycles using single, top-quality blastocysts after artificial endometrial preparation from January 2015 to January 2018. Patients were stratified into four groups based on serum oestradiol concentration percentiles on the day of progesterone initiation: Group 1 (<25th percentile), Group 2 (25–50th percentile), Group 3 (51–75th percentile) and Group 4 (>75th percentile). The primary outcome was live birth rate. The secondary outcomes were implantation, clinical pregnancy and multiple pregnancy rates. Receiver operating characteristic (ROC) curves were generated to evaluate serum oestradiol concentrations in predicting implantation, clinical pregnancy and live birth.ResultsSimilar live birth rates of 51.6%, 55.1%, 54.9% and 56.4% for Groups 1, 2, 3 and 4, respectively, were found. The groups also showed similar implantation and clinical pregnancy rates. ROC analysis revealed that serum oestradiol concentrations on the day of progesterone initiation were not predictive for implantation (area under the curve [AUC] 0.490, 95% CI 0.445–0.554), clinical pregnancy (AUC 0.507, 95% CI 0.453–0.561) or live birth (AUC 0.514, 95% CI 0.461–0.566).ConclusionsSerum oestradiol concentration monitoring just prior to progesterone administration does not appear to be predictive for live birth rates in good prognosis patients undergoing single, autologous vitrified–warmed blastocyst transfer after artificial endometrial preparation. Therefore, the current practice of monitoring serum oestradiol concentration is not supported by this study.     

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.     

Efficacy of immunization against hepatitis B virus infection in children with cancer

BACKGROUND: The purpose of this study was to evaluate the impact of hepatitis B prophylaxis in preventing hepatitis B infection in children with malignancy. PROCEDURE: Between May, 1993, and September, 1998, a total of 151 children (95 boys, 56 girls), 29 (19%) with lymphoma, 58 (39%) with leukemia, and 64 (42%) with solid tumor, were screened for hepatitis B virus (HBV). The mean age was 7. 5 +/- 2.5 years. Children with negative serology received active and/or passive immunization. HBsAg and anti-HBs were positive prior to vaccination in 16 (10%) and 17 (11%) children, respectively. One hundred eighteen children (78%) of one hundred fifty-one with negative serology were included in the vaccination program. The vaccine dose was 40 microg. Children with solid tumor and lymphoma received recombinant hepatitis B vaccine at diagnosis, repeated at months 1, 2, and 12. Hyperimmunglobulin was administered monthly in children with leukemia during the intensive chemotherapy period. They were then vaccinated following the third month of maintenance therapy with the schedule described above. Anti-HBs titers were measured every 3 months, and titers above 10 mlU/ml were accepted as protective. RESULTS: Anti-HBs positivity after the first three doses was 77% in solid tumors, 88% in acute leukemia, and 48% in lymphomas. Anti-HBs positivity with respect to diagnosis in children completing the vaccination schedule was 94% in solid tumor, 90% in leukemia, and 74% in lymphoma (P > 0.05). Thirty-three percent of children have not received the fourth dose as yet. In total 78% of the children developed protective antibody titers with or without the fourth dose, and none was infected with HBV during 3 years of follow-up. Ten (39%) of twenty-six children who remained unresponsive to immunization were infected with HBV. CONCLUSIONS: These data reveal that HBV prophylaxis is necessary and that our vaccination schedule is effective in preventing HBV infection in these children.