Intestinal Perforation during the Stabilization Period in a Preterm Infant with Congenital Diaphragmatic Hernia.

 Delayed surgery after stabilization of infants with congenital diaphragmatic hernia (CDH) is an accepted strategy. However, the evidence favoring delayed versus immediate surgical repair is limited. We present an extremely rare case of a very low-birth-weight infant with prenatally diagnosed left-sided CDH and unexpected transmural bowel perforations developing within the postnatal stabilization period.

Antenatal assessment of liver position, rather than lung-to-head ratio (LHR) or observed/expected LHR, is predictive of outcome in fetuses with isolated left-sided congenital diaphragmatic hernia.

Objectives: Respiratory morbidity in congenital diaphragmatic hernia (CDH) is associated with high mortality and adverse outcome. Accurate prenatal diagnosis is essential for prognosis and potential treatment in utero. The aim was to evaluate the prenatal ultrasound findings in assessing the respiratory prognosis in fetuses with isolated left-sided CDH.

Fanconi anemia with biallelic FANCD1/BRCA2 mutations - Case report of a family with three affected children.

Fanconi anemia, complementation group D1 with bi-allelic FANCD1 (BRCA2) mutations, is a very rare genetic disorder characterized by early onset of childhood malignancies, including acute leukemia, brain cancer and nephroblastoma. Here, we present a case report of a family with 3 affected children in terms of treatment outcome, toxicity and characterization of the malignancies using comprehensive cytogenetic analysis. The first child was diagnosed with T-cell acute lymphoblastic leukemia when he was 11 months old.

Abnormalities in pulmonary function in infants with high-risk congenital diaphragmatic hernia.

Aims: The aim of the study was to analyze lung growth and abnormality of infant pulmonary function tests (IPFT) in congenital diaphragmatic hernia (CDH) survivors younger than three years of age with respect to unfavorable prognostic factors.

Methods: Thirty high-risk CDH survivors at the age of 1.32±0.

[Nephroblastoma - 30-years period of its treatment in the University Hospital Motol, Prague].

Introduction: Nephroblastoma (Wilms tumor - WT) is the most common solid tumor of kidney in children. We present treatment development of WT at the Department of Pediatric Hematology and Oncology, Charles University in Prague, 2nd Faculty of Medicine and University Hospital Motol (KDHO) in the Czech Republic over 30 years. Patients that were treated prior to access to the International Society of Pediatric Oncology (SIOP) protocols are considered to be the historical group, then we have patients treated according to SIOP 9, SIOP 93-01 and SIOP 2001 protocols as full participants of SIOP studies.

Defect-diaphragmatic ratio: a new parameter for assessment of defect size in neonates with congenital diaphragmatic hernia.

Aim: The aim of our study is to introduce a new objective method of perioperative evaluation of the size of diaphragmatic defect to enable comparison of results among various centres and methods used for diaphragmatic reconstruction.

Materials And Methods: Prospective observational study of neonates with congenital diaphragmatic hernia (CDH) and respiratory distress within 24 h of birth operated on from January 2009 to December 2011. Weight, length, thoracic shape and the diameters of diaphragmatic defect were measured.

Large diaphragmatic defect: are skeletal deformities preventable?

Purpose: The aim of study was to compare growth, nutritional status and incidence of chest wall deformities and scoliosis in survivors of large congenital diaphragmatic hernia (CDH) defect (Gore-Tex patch reconstruction) with survivors with smaller defects and primary reconstruction.

Materials And Methods: An anthropometric study of 53 children who underwent CDH repair in neonatal period was carried out. Weight, height, and skin-fold thickness were measured, scoliosis and chest wall deformity were evaluated.

[Treatment results in patients treated from 1980 to 2004 for Wilms' tumour in a single centre].

Backgrounds: The principle behind the treatment of nephroblastoma has been similar for at least 4 decades, based on vincristine and dactinomycine, radiotherapy in selected stages. The last three decades have been characterised by the aim to reduce the intensity and length of treatment.

Design: To retrospectively compare survival rates and treatment success in a cohort of patients aged under 19 years, treated from 1980 to 2004 at a single centre by five consecutive treatment protocols.

Diagnostic efficacy of ERCP in cholestatic infants and neonates--a retrospective study on a large series.

Background And Study Aims: Cholestatic jaundice in infants is a serious condition, requiring timely and accurate diagnostic evaluation. Our aim was to determine the safety and diagnostic efficacy of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of cholestatic liver disease in neonates and infants.

Patients And Methods: ERCP procedures in cholestatic infants performed in our endoscopy unit between December 1998 and March 2008 were reviewed retrospectively (n = 104 children, 48 boys, 56 girls; mean age 7 weeks, range 3 - 25 weeks; mean weight 4.

Cytogenetic and array comparative genomic hybridization analysis of a series of hepatoblastomas.

Hepatoblastoma is the most common primary hepatic tumor in children, and only a limited number of detailed karyotypic analyses have been reported to date. In the present study, cytogenetic abnormalities were identified in nine cases of hepatoblastoma from a single institution. Among characteristic chromosomal changes detected were simple numerical aberrations, structural alterations of chromosomes 1, 2, and 8, and the recurrent unbalanced rearrangements der(4)t(1;4)(q25.

Dendritic cell-based immunotherapy induces transient clinical response in advanced rat fibrosarcoma - comparison with preventive anti-tumour vaccination.

In this study we present the models of preventive and therapeutic vaccination of sarcoma-bearing rats with dendritic cells that present tumour antigens from killed tumour cells. We present the characteristics of dendritic cell-based vaccine and its capacity to induce anti-tumour immune response both in vitro and in vivo. We show that preventive vaccination efficiently prevents tumour growth.

[Current options of prenatal diagnosis in congential diaphragmatic hernia].

Objective: The aim of this article is to review the current options of prenatal diagnosis in congential diaphragmatic hernia (CDH).

Subject: Systematic review.

Setting: Institute for the Care of Mother and Child, 3rd Medical Faculty, Prague.

[Clinical relevance of chromosomal aberrations in bone and soft tissue tumors in children and young adults].

Background: We present the results of a cytogenetic and molecular cytogenetic analysis of a series of patients with bone and soft tissue tumors. PATIENTS ANDMETHODS: We analyzed a cohort of 26 patients with Ewing sarcoma/PNET, 15 patients with rhabdomyosarcoma, 5 with synovial sarcoma and one patient with an undifferentiated sarcoma using the cytogenetic and molecular cytogenetic techniques M-FISH and arrayCGH.

Results: We found nonrandom chromosomal structural and numerical changes with diagnostic and prognostic relevance in most patients.

Total prepyloric transection of stomach and vertebral trauma: case report and review of the literature.

A rare injury of stomach associated with vertebral trauma in a 10-year-old girl, a victim of a traffic accident, is presented. Early X-ray and computerized tomography scan revealed no free abdominal air, only signs of pancreas contusion and fracture of the Th 12 and L 1 vertebral body were evident. Transection of stomach was revealed during endoscopy (an attempt to perform retrograde cholangio-pancreaticography) 20 h after the trauma.

[Approximative intestinal anastomosis in newborns with multifocal necrotizing enterocolitis].

Multifocal necrotizing enterocolitis (NEC) may result in extensive bowel necrosis and short bowel syndrome. Authors present case report of premature newborn (BW 1700 g, gestational age 30 w.) where an extensive multisegmental NEC of small and large intestine was found during first explorative laparotomy.

Surgical management of major pancreatic injury in children.

Introduction: Major or complicated pancreatic trauma in children is uncommon and management strategies remain controversial. The aim of this study was to evaluate our experience with both early and delayed surgery in these pediatric cases.

Methods: We carried out a retrospective analysis of data of pediatric patients with major or complicated pancreatic injury operated on between January 1994 and December 2005 in our pediatric trauma center.

T-tube ileostomy for intestinal perforation in extremely low birth weight neonates.

To evaluate the results of use of T-tube ileostomy in selected cases of intestinal perforation in extremely low birth weight (ELBW) neonates. The records of 288 ELBW neonates treated at author's institution, from 1998 to 2003 were retrospectively reviewed to identify neonates operated for intestinal perforation with T-tube placement. T-tube was inserted into the bowel through the site of perforation or proximally to the perforated gut via separate stab incision.

[Surgical complications following oesophageal replacement by stomach in childhood].

Aim: Assessment of surgical complications and options for their therapy following replacement of oesophagus by stomach. METHODOLOGY AND MATERIAL: A retrospective study of clinical and pathological data collected from 25 children, who underwent replacement procedures of their oesophagus by stomach during the 1992-2005 period. 18 subjects were indicated for the procedure for oesophageal atresia with large stubs distance and 7 subjects were indicated for extensive oesophageal strictures following acid burns.

Congenital diaphragmatic hernia: onset of respiratory distress and size of the defect: analysis of the outcome in 104 neonates.

The purpose of this study was to evaluate the outcome in neonates with congenital diaphragmatic hernia (CDH) either presenting within the first 24 h of life or diagnosed prenatally. The study was particularly focused on the time of onset of respiratory distress and on the use of the Gore-Tex (GT) patch for diaphragmatic reconstruction. Records of 104 neonates with CDH were retrospectively reviewed.

Acute gastro-intestinal obstruction as a late presentation of congenital diaphragmatic hernia. A report of three cases.

Three cases of acute gastro-intestinal obstruction due to incarceration of congenital diaphragmatic hernia (Bochdalek hernia) in infants are reported. The level of incarceration was stomach, small and large intestine. All of the presented posterolateral diaphragmatic defects were small and without a sac.

Porcelain gallbladder with extrahepatic bile duct obstruction in a child.

An extrahepatic bile duct obstruction was diagnosed in a 13-year-old boy presenting with pruritus, abdominal pain and jaundice. Several weeks after sphincterotomy and biliary stenting via endoscopic retrograde cholangiopancreaticography which relieved the obstruction, the patient was operated on. Severe fibrosis encased the extrahepatic biliary tract, so only cholecystectomy was performed because planned hepaticojejunoanastomosis could jeopardize the vascular supply to the liver.

Congenital diaphragmatic hernia associated with esophageal atresia, tracheoesophageal fistula, and truncus arteriosus in a premature newborn.

The occurrence of coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is extremely rare and is considered highly lethal. The combination of CDH with EA/TEF and truncus arteriosus communis (TAC) has not been reported in the literature to date. The authors describe a premature neonate with this association.

[Are there any new procedures for treating necrotising enterocolitis in neonates with very low birth weight?].

Objective: To evaluate some perinatal and neonatal risk factors of occurrence of necrotising enterocolitis (NEC) and the current possibilities of treatment.

Design: A retrospective data analysis.

Setting: Mother and Child Care Institute, Prague.

[Necrotizing enterocolitis in extremely immature newborns. The surgical treatment potential].

Aim: The aim of this study is to assess a contemporary treatment potential for necrotizing enterocolitis in newborns with birth weights under 1000 g. METHODOLOGY AND SUBJECTS: This is a retrospective study of clinical and pathological data in a group of 19 newborns with birth weights under 1000 g treated for necrotizing colitis (NEC) by the authors' team from 1999 to 2003. Only newborns with the second and third grade NEC according to Bell were included in the trial group.

[Treatment of the Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine, from 1979 to 2004].

Background: Survey of surgical techniques, strategy and results of the treatment of patients with Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine in Prague from 1979 to 2004 is presented.

Methods And Results: Paper summarises a twenty-five years long retrospective study of medical records of 274 patients who were operated on 1979-2004. 173 (67,3 %) pts had classical rectosigmoid (CRA) and/or long colonic aganglionosis (LCA).