Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases

Background

Cavernous malformations (CMs) of the central nervous system (CNS) are angiographically occult vascular lesions that affect approximately 0.5 % of the general population, and one quarter of all CMs occurs in children.

Methods

We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution.

Results

The mean age of the children at first presentation and at operation was 8.7 and 9.6 years, respectively. However, a bimodal age distribution was found with peak under 4 years and above 12 years. Seizure was the most common single presenting symptom (38.9 %), and 61.1 % of patients had at least one seizure before the admission. Focal neurological deficits (410.7 %), intracranial hypertension (27.8 %), and headache (2.8 %) were the other manifestations. Acute/subacute hemorrhage was evident at presentation in 63.9 %. The patients under 6 years of age were found to have significantly more giant cavernomas (69 vs 20 %; p?=?0.011), and more overt hemorrhages (81 vs 47 %; p?=?0.065) at diagnosis than those patients above 12 years. Surgery was performed in 31 patients (32 CMs), with 26 total and 6 incomplete resections. Mean follow-up duration was 6.9?±?4.1 years. Of all patients, 63.8 % had excellent and 30.5 % had good clinical outcomes, and also 90.9 % of the epileptic patients were seizure-free (Engel Class I) at the last follow-up.

Conclusions

Younger children tend to harbor larger CMs and present with hemorrhage more frequently than older ones. Microsurgical resection should be the treatment of choice in symptomatic and accessible CMs.     

The value of serum Bcl-2 levels in advanced lung cancer patients

Overexpression of the Bcl-2 protein was associated with a favorable prognostic factor for survival in lung cancer patients, especially nonsmall cell lung carcinoma. The present study was conducted to investigate the value of serum Bcl-2 levels in advanced lung cancer patients. Fifty patients with advanced lung carcinoma pathologically verified and 18 healthy controls were investigated. Serum samples were obtained on the first admission before the chemotherapeutic treatment were given. Serum Bcl-2 levels were determined by using anti-Bcl-2 monoclonal coating antibody. The baseline serum Bcl-2 levels were significantly higher in patients with lung cancer than in the control group (p<0.001). Serum Bcl-2 levels were elevated in 48 (96%) advanced lung cancer patients. None of the prognostic parameters analyzed, such as age of patient, gender, histology, stage of disease, erythrocyte sedimentation rate, serum albumin, hemoglobin, CEA, NSE, LDH, performance of patient, weight loss, and response to chemotherapy, was significantly correlated with Bcl-2 serum concentrations. The serum Bcl-2 concentrations were not changed with cisplatin-based cytotoxic chemotherapy regardless of response (p=0.76). No prognostic value of serum Bcl-2 was determined. In conclusion, the results of the present study, which is the first study to determine serum Bcl-2 levels in lung cancer, suggest that decreased apoptosis occurred due to the effect of serum Bcl-2 elevation in lung cancer patients. Serum Bcl-2 level was of diagnostic but not prognostic value in lung cancer patients. However, more studies are needed to define the role of Bcl-2 in the diagnosis and prognosis of lung cancer.     

HLA alleles and haplotypes in the Turkish population: relatedness to Kurds, Armenians and other Mediterraneans

Turkish and Kurdish HLA profiles are studied for the first time. The comparative study of their allele frequencies, characteristic haplotypes, genetic distances with other Mediterraneans is complemented by neighbor-joining dendrograms and correspondence analyses. Turks, Kurds, Armenians, Iranians, Jews, Lebanese and other (Eastern and Western) Mediterranean groups seem to share a common ancestry: the older "Mediterranean" substratum. No sign of the postulated Indo-European (Aryan) invasion (1200 B.C.) is detected by our genetic analysis. It is concluded that this invasion, if occurred, had a relatively few invaders in comparison to the already settled populations, i.e. Anatolian Hittite and Hurrian groups (older than 2000 B.C.). These may have given rise to present-day Kurdish, Armenian and Turkish populations.     

The relation between genitourinary prolapse and joint hypermobility in Turkish women

Objective  

In this cross-sectional study, we compared frequency of hypermobility syndrome in pelvic organ prolapse (POP) patients and normal healthy Turkish women.     

ARM ANTHROPOMETRY IN EVALUATION OF MALNUTRITION IN CHILDREN WITH CANCER

Malnutrition in children with cancer is reported to be relatively uncommon at the time of diagnosis. However, in most studies nutritional status measurement has relied almost exclusively on weight-related indices. This can be misleading, because in children with malignancy, tumor masses can reach more than 10% of total body weight. A controlled study was performed in 62 patients using arm anthropometry to provide a more accurate evaluation of the nutritional status of children with cancer at presentation. Height, weight, midupper arm circumference (MUAC), and triceps skinfold thickness (TSFT) were measured in all patients (40 boys, 22 girls) and controls (18 boys, 13 girls). Weight for height (W FH) of each patient was compared with the national standards. MUAC and TSF T were also interpreted according to the standards developed by A. Roberto Frisancho. The mean ages were 6.5 3.7 years (range 0.08-13) and 5.7 4.7 years (range 0.25-15) in patients and control group, respectively. Results showed that although the W FH values for patients were normal, MUAC and TSF T values were significantly less than control values (P &lt; 0.001). Moreover, 27% of patients showed malnutrition (they had MUAC and TSF T below 5th percentile). Patients with intraabdominal solid tumors had significantly lower MUAC and TSF T values than those with extraabdominal solid tumors (P &lt; 0.05). The data strongly indicate that malnutrition is common at the time of diagnosis in children with cancer, and arm anthropometry should replace the use of weight-related indices to identify malnutrition in children.     

Prognostic factors and treatment outcome in childhood Hodgkin disease

BACKGROUND: The goals of this study included: (1) Identification of factors prognostic for event-free survival (EFS) and overall survival (OS), and (2) Definition of risk groups for risk adapted therapy in children with Hodgkin disease (HD). PROCEDURE: From 1991 to 2003, 69 children with newly diagnosed, untreated biopsy-proven stage I-IV HD were treated with chemotherapy (CT) and low-dose involved field radiotherapy (LD-IFRT). The relationship of pretreatment factors to EFS and OS was analyzed by univariate and multivariate analysis. RESULTS: The 5-year EFS and OS for all patients were 90.77% and 96.22%, respectively with a median follow-up of 73 months (3-137 months). Male to female ratio was 3:1 and 21 children (32.3%) were less than 7 years of age. Mixed cellularity was the predominant histologic subtype (38.5%). Factors associated with inferior EFS by univariate analysis were extranodal disease, hemoglobin level <11 g/dl, number of involved lymph node regions and stage. By multivariate analysis only stage IV disease was significant. CONCLUSION: Our study confirms that excellent results are achievable with combined modality therapy in childhood HD. In order to use risk-adapted therapy in children with HD, clinical prognostic factors should be validated with large, multicentered prospective clinical studies.     

Diagnostic Value of Magnetic Resonance Imaging in Gynaecology

In this study 23 patients with various gynaecological pathologies were evaluated. Ultrasonography and magnetic resonance imaging were performed in all cases, but only 14 were evaluated with computed tomography. On the basis of ultrasonography, 4 patients were labelled as having malignant ovarian tumours, however, this diagnosis was confirmed by magnetic resonance imaging in only 1 of the 4. Magnetic resonance imaging also determined the correct diagnosis in a patient with endometrioma whereas computed tomography showed only a simple cyst, and ultrasonography diagnosed a subserous myoma. Tumour was demonstrated by magnetic resonance imaging in 2 patients with cervical carcinoma although computed tomography and ultrasonography had previously shown no tumour mass in these patients. It was concluded that magnetic resonance imaging is much superior to computed tomography and ultrasonography in gynaecological diagnosis. This advantage results from the correct detection of the contents of ovarian cysts, the number and localization of uterine leiomyomas in T2 scans, invasion of uterine malignancies and differential diagnosis of subserous uterine leiomyomas from ovarian tumours.     

Serum bcl-2 and survivin levels in melanoma

This study was conducted to investigate the serum levels of bcl-2 and survivin in patients with melanoma and the relationship with tumour progression and known prognostic parameters. Forty-four patients with cutaneous melanoma were investigated. Serum samples were obtained on first admission before adjuvant and metastatic treatment were given and at follow-up. Serum bcl-2 and survivin levels were determined using enzyme immunometric assay (EIA) and enzyme-linked immunosorbent assay (ELISA). The baseline serum bcl-2 levels were significantly higher in patients with melanoma than in the control group (P=0.01). For the serum survivin levels, no difference was found (P=0.6). No significant correlations were found between the prognostic parameters analysed and the serum survivin concentrations. The same was true of the serum bcl-2 values, except for the age of the patient (P=0.025) and nodal involvement (P=0.003). No significant relationship was found between the serum levels of bcl-2 and survivin (r=-0.13, P=0.4). In node-positive patients (n=8) both of these anti-apoptotic substances were unchanged after interferon-alpha-2b therapy. However, serum survivin concentrations were significantly increased in 10 patients with metastatic melanoma who underwent dacarbazine (DTIC)-based cytotoxic chemotherapy (P=0.047). A similar finding was not determined for the serum bcl-2 levels. In conclusion, the results of this study suggest that decreased apoptosis is associated partly with an increase in serum bcl-2. However, much research continues in this field, and exciting new knowledge will ultimately emerge.