Analysis of pediatric thrombotic patients in Turkey

This study analyzes the data of thrombotic children who were followed up in different pediatric referral centers of Turkey, to obtain more general data on the diagnosis, risk factors, management, and outcome of thrombosis in Turkish children. A simple two-page questionnaire was distributed among contact people from each center to standardize data collection. Thirteen pediatric referral centers responded to the invitation and the total number of cases was 271. All children were diagnosed with thromboembolic disease between January 1995 and October 2001. Median age at time of first thrombotic event was 7.0 years. Of the children 4% of the cases were neonates, 12% were infants less than 1 year old, and 17% were adolescents. Thromboembolic event was mostly located in the cerebral vascular system (32%), deep venous system of the limbs, femoral and iliac veins (24%), portal veins (10%), and intracardiac region (9%). Acquired risk factors were present in 86% of the children. Infection was the most common underlying risk factor. Inherited risk factors were present in 30% of the children. FVL was the most common inherited risk factor. Acquired and inherited risk factors were present simultaneously in 19% of the patients. Eleven children had a history of familial thrombosis. Due to the local treatment preferences, the treatment of the children varied greatly. Outcome of the 142 patients (52%) was reported: 88 (62%) patients had complete resolution, 47 (33%) had complications, 12 (9%) had recurrent thrombosis, and 34 (24%) died. Three children (2.1%) died as a direct consequence of their thromboembolic disease. The significant morbidity and mortality found in this study supports the need for multicentric prospective clinical trials to obtain more generalizable data on management and outcome of thrombosis in Turkish children.     

Pseudomass of the sternal manubrium in osteogenesis imperfecta

Skeletal abnormalities such as hypertrophic callus formation and "popcorn" calcifications are rare radiological findings of osteogenesis imperfecta, causing tumor-like appearances on imaging. We report on a 7-year-old girl with osteogenesis imperfecta presenting with hepatomegaly and palpable lymphadenopathy in the left inguinal region on physical examination. Computed tomography examination revealed a high-density mass-like lesion of the manubrium sterni. Ultrasonography and a lateral roentgenogram of the chest verified that this was a pseudomass caused by a bowed sternal manubrium.     

Composite tissue allotransplantation in chimeric hosts: part I. Prevention of graft-versus-host disease

BACKGROUND: Mixed allogeneic chimerism (MAC) has been shown to induce tolerance to composite tissue allografts (CTA). However, transplantation of unmanipulated donor-specific limbs results in severe graft-versus-host disease (GVHD). This suggests that nontolerant mature donor-derived cells in the CTA may affect the stability of chimerism, potentially resulting in GVHD. The aim of this study was to develop an approach to study and prevent GVHD in a mixed chimeric-rat hind-limb transplantation model. METHODS: [ACI-->WF] chimeras received a limb from Wistar Furth (WF) (syngeneic), Fisher (third-party), or ACI (irradiated [1,050 cGy] or nonirradiated) rats. In vitro tolerance was assessed using mixed lymphocyte reactivity (MLR) assays at the time the animals were killed. RESULTS:[ACI-->WF] chimeras with greater than 85% chimerism exhibited rejection-free survival of donor-specific hind limbs. However, 100% of these animals developed lethal GVHD 22.4+/-2.8 days after limb transplantation. [ACI-->WF] chimeras that underwent transplantation with irradiated ACI or syngeneic WF limbs showed no signs of rejection or GVHD at 5 months. Nonchimeric and third-party controls rejected limbs within 10 days. CONCLUSIONS: Conditioning of the host WF rats with 950 cGy of irradiation (sublethal, myeloablative) led to high levels of MAC without GVHD. The mature T-cell content of nonirradiated donor (ACI) limbs was sufficient to induce lethal GVHD in 100% of tolerant mixed chimeric [ACI-->WF] hosts. Irradiation of donor limbs before transplantation resulted in long-term donor-specific tolerance and prevented GVHD. These data demonstrate that (1) established chimeras could be susceptible to GVHD caused by immunocompetent donor cells transferred with the hind limb, and (2) inactivating these cells with irradiation prevents GVHD and destabilization of chimerism, and permits rejection-free graft acceptance.     

The effects of granulocyte colony-stimulating factor on the healing of tracheal anastomosis following radiation therapy in rats

Objective: This study evaluates the effects of granulocyte colony-stimulating factor on the healing of tracheal anastomosis following radiation therapy in rats. Methods: Fifty-six male Wistar rats were divided into four groups. Group 1 underwent tracheal anastomosis. Group 2 underwent radiation therapy followed by tracheal anastomosis. Group 3 underwent radiation therapy followed by tracheal anastomosis and received granulocyte colony-stimulating factor. Group 4 underwent sham radiation therapy followed by sham tracheal anastomosis. At 10 days following radiation therapy, the trachea was dissected for histopathological, mechanical and biochemical evaluation. Results: Median scores for inflammation were three points for Group 1, two points for Group 2, two points for Group 3 and one point for Group 4. Median scores for angiogenesis were four points for Group 1, two points for Group 2, three points for Group 3 and one point for Group 4. Median scores for connective tissue regeneration were four points for Group 1, two points for Group 2, three points for Group 3 and one point for Group 4. Median scores for epithelial regeneration were two points for Group 1, one point for Group 2, one point for Group 3 and one point for Group 4. Mean anastomotic bursting pressures were 853 mmHg for Group 1, 293 mmHg for Group 2, 417 mmHg for Group 3 and 966 mmHg for Group 4. Mean hydroxyproline concentrations were 159 μg/mg for Group 1, 177 μg/mg for Group 2, 120 μg/mg for Group 3 and 117 μg/mg for Group 4. Conclusions: This study suggests that granulocyte colony-stimulating factor contributes to the healing of tracheal anastomosis following radiation therapy through improved connective tissue regeneration.     

Antrochoanal polyps in children

OBJECTIVE: To evaluate the characteristics of antrochoanal polyps (ACPs) in children. METHODS: 10 children operated for ACP were investigated retrospectively. Demographic characteristics, surgical and histopathological findings were evaluated. RESULTS: The mean age was 10.2 years. The antral part of ACP was removed through middle meatal antrostomy in four patients, and transcanine sinuscopy was needed in six patients. It was found that the polyp passed through the main ostium in seven patients and accessory ostium in three patients. The antral part of the polyp was found to be cystic in six patients and polypoid in four patients. There was recurrence of polyps during follow-up period in two cases in which antral part of the polyp was seen to be removed through middle meatal antrostomy during primary surgery. In histologic examination, prominent eosinophilia was detected only in one patient and no mucous gland was detected in any patient. CONCLUSION: Endoscopic surgery through the middle meatal antrostomy combined with transcanine sinuscopy ensures the complete removal of the antral part of ACP in children.     

Clinical significance of serum MMP-2 and MMP-7 in patients with ovarian cancer

Matrix metalloproteinases (MMPs) are frequently expressed in malignant tumors and play an important role in tumor invasion and metastasis. The aim of this study was to evaluate role of serum MMP-2 and MMP-7 levels in patients with ovarian cancer. Serum levels of MMP-2 and MMP-7 were measured in 28 patients with ovarian carcinoma, 2 with borderline ovarian tumors, 10 with non-malignant gynecological disease and 30 healthy women by Enzyme-Linked Immunosorbent Assay (ELISA). Serum MMP-7 level was significantly (10.24 ± 1.35 ng/ml) higher in the patients with ovarian malign tumors than healthy controls (3.29 ± 1.64 ng/ml) (P < 0.05). Postoperative levels of MMP-7 (7.68 ± 1.17 ng/ml) were significantly lower in patients with malign ovarian tumors than those of preoperative level (10.24 ± 1.35 ng/ml) (P < 0.05). Serum MMP-2 levels were significantly lower in the patients with ovarian malign tumors (227.51 ± 9.91 ng/ml) than those in the healthy controls (279.12 ± 73 ng/ml) (P < 0.05). There was no significant difference in serum levels of MMP-2 and MMP-7 in patients with benign ovarian disease when compared to healthy controls and patients with malignant disease (P > 0.05). As a conclusion, MMP-7 can be a useful serum marker to show disease activity in malignant ovarian tumors.