ABSTRACT
AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.
Subject(s)
Kidney Neoplasms/therapy , Wilms Tumor/therapy , Adolescent , Child , Child, Preschool , Combined Modality Therapy , Female , Humans , Infant , Infant, Newborn , Kidney Neoplasms/mortality , Male , Wilms Tumor/mortalityABSTRACT
BACKGROUND: The purpose of this study was to evaluate left and right ventricular functions by using the Doppler myocardial performance index (MPI), in children with sickle cell anemia (SCA). METHODS: We examined 32 patients with SCA and 30 age-matched healthy children. Echocardiography and Doppler examinations were completed for each of the subjects. MPI was calculated from the Doppler tracings. RESULTS: Compared with controls, left ventricle (LV) end-diastolic diameter, end-systolic diameter, early-diastolic mitral flow velocity and late-diastolic mitral flow velocity were significantly higher in the patients with SCA. Although, the LV ejection fractions were in the normal ranges in patients and controls, both LV and right ventricle (RV) MPI were significantly higher in patients than those in normal children. CONCLUSION: MPI may be a useful noninvasive and sensitive tool for assessing the sub-clinical cardiac LV and RV dysfunctions in patients with SCA.
Subject(s)
Anemia, Sickle Cell/physiopathology , Echocardiography, Doppler/methods , Ventricular Dysfunction, Left/diagnostic imaging , Ventricular Dysfunction, Right/diagnostic imaging , Adolescent , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/diagnostic imaging , Case-Control Studies , Child , Child, Preschool , Female , Heart Ventricles/diagnostic imaging , Heart Ventricles/physiopathology , Humans , Male , Prospective Studies , Sensitivity and Specificity , Young AdultABSTRACT
Extracorporeal photochemotherapy (ECP) is an alternative treatment approach in the management of chronic graft-versus-host disease (cGVHD). However, few reports have investigated the optimal treatment schedule of ECP. We treated seven pediatric patients with steroid-resistant or -dependent cGVHD with ECP. Using the Therakos UVAR XTS system, ECP was begun three (three patients) or five (four patients) times a week initially. Complete and partial responses were obtained in three patients. Three of seven patients died of GVHD-related complications. In this group initial intensive ECP did not improve the rate of response. However, this schedule appeared well tolerated.
Subject(s)
Graft vs Host Disease/drug therapy , Photochemotherapy/methods , Adolescent , Child , Child, Preschool , Chronic Disease , Female , Humans , Male , Treatment OutcomeABSTRACT
Dorfman-Chanarin syndrome is a rare, autosomal recessive disorder characterized by congenital ichthyosis and presence of intracellular lipid droplets in most tissues. Here, we present a patient from Turkey, who is the fourth Turkish case in the literature with this syndrome, and we review the previous reported cases. He was also the second case reported with hyperlipidemia.
