Targeting B7-H3-A Novel Strategy for the Design of Anticancer Agents for Extracranial Pediatric Solid Tumors Treatment.

Recent scientific data recognize the B7-H3 checkpoint molecule as a potential target for immunotherapy of pediatric solid tumors (PSTs). B7-H3 is highly expressed in extracranial PSTs such as neuroblastoma, rhabdomyosarcoma, nephroblastoma, osteosarcoma, and Ewing sarcoma, whereas its expression is absent or very low in normal tissues and organs. The influence of B7-H3 on the biological behavior of malignant solid neoplasms of childhood is expressed through different molecular mechanisms, including stimulation of immune evasion and tumor invasion, and cell-cycle disruption. It has been shown that B7-H3 knockdown decreased tumor cell proliferation and migration, suppressed tumor growth, and enhanced anti-tumor immune response in some pediatric solid cancers. Antibody-drug conjugates targeting B7-H3 exhibited profound anti-tumor effects against preclinical models of pediatric solid malignancies. Moreover, B7-H3-targeting chimeric antigen receptor (CAR)-T cells demonstrated significant in vivo activity against different xenograft models of neuroblastoma, Ewing sarcoma, and osteosarcoma. Finally, clinical studies demonstrated the potent anti-tumor activity of B7-H3-targeting antibody-radioimmunoconjugates in metastatic neuroblastoma. This review summarizes the established data from various PST-related studies, including in vitro, in vivo, and clinical research, and explains all the benefits and potential obstacles of targeting B7-H3 by novel immunotherapeutic agents designed to treat malignant extracranial solid tumors of childhood.

Congenital prepubic urachal-cutaneous fistula.

Congenital prepubic fistula is a very rare congenital anomaly that manifests as a punctiform opening in the midline of the pubic region. According to Campbell, congenital prepubic fistulae are duplicates of the dorsal urethra. Recent investigations have shown that this problem may have a complex etiology. We report a case of a congenital prepubic fistula in 2-month old female neonate, which manifested as a simple punctiform opening in the pubic region, with purulent secretion that resulted from applying pressure to the region. The surgical treatment was successful, and the infant had an uneventful postoperative course of healing.

Transverse colon volvulus in neurologicaly imparied patient as an emergency surgical condition: A case report.

Introduction: Transverse colon volvulus is an uncommon cause of bowel obstruction in general. Predisposing factors are mental retardation, dysmotility disorders, chronic constipation and congenital megacolon. Case report: We presented transverse colon volvulus in a 16-year-old boy with cerebral palsy. Chronic constipation in neurologicaly impaired patient was a risk factor predisposing to volvulus. The patient was admitted to the hospital with enormous abdominal distension and acute respiratory insufficiency. A boy was emergently taken to the operating room for exploratory laparotomy. During the surgery, a 360º clockwise volvulus of the transverse colon was found. After reduction of volvulus, an enormous transverse colon was resected and colostomy was formed. In the postoperative period, despite the good functioning of stoma and intraabdominal normotension, numerous and long lasting respiratory problems developed. The patient was discharged from our institution after 8 months. Conclusion: Though very rare in pediatric group, the possibility of a transverse colon volvulus must be considered in the differential diagnosis of acute large bowel obstruction.

Gastric tube esophageal reconstruction in children with esophageal atresia and caustic stricture Study of clinical value based on 25 single-center. Centre experience.

AIM: The aim of this study was estimation of clinical value of gastric tube esophagoplasty in children based on one single center experience. METHODS: Forty-nine patients with diagnosis of EA (n=22) and caustic esophageal injury (n=27) had undergone reverse gastric tube esophagoplasty during 25 years at our institution. Almost all EA patients received initially gastrostomy and cervical esophagostomy. Majority of CS patients had initially repeated dilatation therapy. Demographics, preoperative features (initial condition, initial treatment and nutritional status) and postoperative features (leakage, stenosis, nutritional status and disease-specific symptoms) were retrospectively reviewed and analyzed. Mean follow up time was 8.2 years. RESULTS: Of 49 patients, 2 patients died and gastric tube failed in one patient. The most common complications were anastomotic leak (52%) and anastomotic stricture (47,8%). Disease-specific symptoms (dysphagia and reflux symptoms) with important clinical manifestations were observed in 10.87% of patients. Preoperatively, all patients were eutrophic. One year after operation 96% of patients stayed eutrophic and two EA patients were malnourished. CONCLUSION: The most common complications such as anastomotic leak and anastomotic stricture may be considered benign. Postoperative nutrition, as good indicator of the procedure's adequacy, for the majority of patients is satisfactory. Gastric tube esophagoplasty is an appropriate alternative for esophageal replacement in children. KEY WORDS: Children, Gastric tube, Esophagoplasty.

Analysis of Prognostic Factors and Mortality in Children with Esophageal Atresia.

OBJECTIVE: The survival rate in newborns with congenital esophageal atresia (EA) is about 85-90 %, and it raises over 95 % in the developed countries. The survival rate in developing countries is much lower and the authors describe their experience with 60 newborns. METHODS: Medical records of 60 newborns (40 boys and 20 girls) with congenital EA were reviewed for the prognostic factors and mortality. RESULTS: The birth weight, mean Apgar score (AS) value, gestational age and birth-operative treatment time had significant influence on the mortality of treated patients (p < 0.05). Thirty five percent newborns had aspirational pneumonia at the moment of hospitalization and 86.7 % of them were operated during the first 48 h. The presence of associated anomalies considerably affected the death rate of treated patients (p < 0.05). The incidence of postoperative complications was similar to those in developed countries but the total mortality was higher (28.3 %); sepsis being the main cause of mortality. The postoperative complications and sepsis significantly influenced the mortality of patients (p < 0.05). CONCLUSIONS: Total mortality in newborns with EA was high; sepsis being the most frequent cause of death. The high total mortality was also caused by prematurity, delay in diagnosis, increased incidence of the aspiration pneumonia and shortage of qualified nurses.

Cystic echinococcosis in children - the seventeen-year experience of two large medical centers in Serbia.

Cystic echinococcosis (CE) is a public health problem in countries having such endemic areas. Epidemiological studies of CE, especially pediatric, are rare. The aim of this study was to evaluate epidemiological and clinical characteristics of CE in children in Serbia. Data were obtained retrospectively from the case records of patients under the age of 18 years admitted for surgical treatment of CE at two large pediatric medical institutions in the period 1990-2006. Patients' age, number of cysts and their anatomic location were evaluated in relation to differences by patients' gender and socio-geographic status (urban or rural origin). The study included 149 children with 272 hydatid cysts. The mean age of patients was 10.1+/-3.8 years. There were no significant differences in the number of patients in relation to gender and urban:rural origin. There were no significant differences in patients' age at the time of surgery or the number of cysts per patient when patients' gender or socio-geographic status was evaluated. The anatomic location of cysts was as follows: liver (N=165; 60.7%), lungs (N=82; 30.1%), and other locations (N=25; 9.2%). Multiple cysts, and combined liver/lung involvement were identified in 34.2% (N=51), and 6.0% (N=9) of patients, respectively. Hepatic cysts were significantly more common in girls than in boys. There were no significant differences in anatomic location of cysts between socio-geographic groups. The large number of infected children during a long period of investigation indicates an active transmission of disease and a lack of program for control and prevention of CE in Serbia.