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The bleeding from the upper gastrointestinal tract represent a significant medical but also socio-economic problem.A special group of patients et increased risk consists of critically ill patients in intensive care units. Particularly significant cause of bleeding in intensive care unit patients is bleeding resulting from the stress ulcers caused by damage of themucosa of the stomach and duodenum. The purpose of this review is to present current experience in prevention of upper gastrointestinal tract bleeding using proton pump inhibitors in intensive care units. Combination of endoscopic hemostatic methods and proton pump inhibitors represents golden standard in most cases. Despite some adverse effects treatment with proton pump blockers is essential when upper gastrointestinal tract bleeding appears in critically ill patients in intensive care units. Proton pump inhibitors are more effective in acid suppression, as well as in the prevention of recurrent bleeding after endoscopic hemostasis than histamine 2 receptor blockers. The efficacy of proton pump blockers is higher in the case of a continuous intravenous infusion than in the intermittent mode of administration of the drug. The need for highly elaborate strategy for the prophylaxis of bleeding from the upper parts of gastrointestinal tract in intensive care units is essential, because when it occurs in intensive care units, mortality is high, and therapeutic options become narrow.

Mucocele of the apendix is a rare clinical and pathological entity with a higher incidence in the female population, and non-specific symptomatology that includes more diagnoses including neoplastic and non-neoplastic causes of the apendix dilation and mucin proliferation. With obtained numerous variations in symptomatology, clinical picture, intraoperative presentation, surgical treatment and histopathological findings, the aim of this paper is an inspection into current knowledge of this disease, its histopathological characteristics, differential diagnosis and current treatment modalities. We present a patient with a clinical picture of the acute abdomen and numerous comorbidities. Ultrasound diagnostics indicated the presence of thin-walled dilatation of the small intestine, especially in the lower right quadrant, as well as the presence of free fluid in the peritoneal cavity. Apendectomy and removal of the mucocele and resection of the gangrenous sigmoid colon according to Hartmann were done. A definitive histopathological finding suggests ischemic colitis and retention mucocele of the apendix with normal mucosa. Histopathologically, four types of mucocele apendixes are distinguished, namely the retention mucinous cyst, mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma. Surgical treatment is the method of choice in treating these tumors, either open or laparoscopic. Imperative is the preservation of the integrity of the entire mucocele, due to the possible dissemination of contents of the cyst and the subsequent development of pseudomyxoma peritonei.

Phyllodes tumors of the breast can be benign, malignant, or borderline. Benign and borderline tumors are rare tumor types that have a positive outlook and high survival rate, while the risk of recurrence is typical for malignant breast tumors. Giant phyllodes tumors are larger than 10 cm in diameter and demand a serious diagnostic and treatment approach. In this study we present a case of a female patient treated for an exulcerated breast carcinoma- a giant borderline phyllodes tumor of the breast. The patient presented to the department for the right breast lump with ulcerated skin and nipple abnormalities. The core biopsy was performed and the patient was diagnosed with a benign tumor. Simple mastectomy was performed and final histopathological report revealed a borderline phyllodes tumor. Diagnosis and treatment of a giant phyllodes tumor remain a great challenge for the surgeons. Establishing the preoperative diagnosis based on histopathological findings is imperative to disease management. Surgery is the mainstay of treatment and mastectomy has been the traditional procedure; in cases where suspicious findings in the axilla are revealed, radical mastectomy is performed and the axilla is to be dissected.

Background: Appendiceal mucoceles represent neoplastic and non-neoplastic, dilatated, mucus filled appendix vermiformix. Appendectomy is obligatory due to a possible malignancy. It is crucially important to avoid rupturing of the mucocele because it can result in pseudomyxoma peritonei, with high morbidity and mortality. Case Report: We presented a 52-year-old man with pain and palpable mass in the lower right quadrant of the abdomen. The mucocele was removed without a rupture, and the patient was discharged from the surgical department one day after the surgery without a complication. Discussion: The resection must be done very carefully, because the rupture of a mucocele can cause pseudomyxoma peritonei, a very dangerous and often lethal condition. Due to the concern of rupture, we performed the classical resection through laparotomy. Conclusion: It is very important, especially for young, inexperienced surgeons to be aware of this rare diagnosis and perform a surgical intervention according to the guidelines of good clinical practice.

Background/Aim. Gastric cancer (GC) is fourth most frequent malignant tumor worldwide, frequently diagnosed at advanced stages with poor prognosis. The aim of study was to determine expression of interleukin (IL)-32, proinflammatory and angiogenic mediators in the tumor, peritumor and healthy tissue, in patients with intestinal gastric cancer and the relationship with the disease severity. Methods. The tissue samples of intestinal type of the tumor of 60 patients with GC were analyzed. Expression of IL-32, vascular endothelial growth factor (VEGF), IL-17 and CD31 were measured by immunohistochemistry. Results. IL-32, VEGF and IL-17 expression as well as microvascular density (MVD) were diminished in adjacent tumor tissues compared with the tumor ones. Further, more intense expression of IL-32 and VEGF and enhanced MVD were noticed in patients with severe (TNM stages III and IV) and more progressive GC (lymph vessel invasion). Conclusion. Higher expression of IL-32, VEGF and intense MVD in the tumor tissue of GC patients with detectable lymph vessel invasion may be considered as a sign of the tumor?s malignant progression. This indicates a protumorogenic and proangiogenic role of IL-32 in biology of intestinal type of gastric cancer.

Background (Pre-)Implantation biopsies provide important data on the quality of donor kidneys. Interstitial fibrosis, as a known predictor for kidney disease progression, is an essential feature of this evaluation. However, the assessment of frozen sections of implantation biopsies is challenging and can result in the disposal of candidate organs. We sought to apply digital image analysis (DIA) to quantify the differences between frozen and paraffin sections when evaluating interstitial fibrosis, identify factors that influence these variations and test the predictive value of the computerised measures. Methods We quantified the differences between frozen and paraffin sections in the same biopsy samples by measuring Sirius red-stained interstitial areas (SRIA) in DIA. We compared them to the original reports, and retrospectively correlated our findings to clinical data, graft function and outcome in 73 patients. Results Frozen sections display a broader interstitial area than paraffin sections, in some cases up to one-third more (mean difference + 7.8%, range − 7 to 29%). No donor-related factors (age or gender, cold ischemia time, or non-heart-beating donor) influenced significantly this difference. Compared to the original assessment of frozen vs paraffin sections in optical microscopy, the DIA of interstitial fibrosis shows a higher consistency (ICC 0.69). Our approach further allows to distinguish SRIA in paraffin sections as an independent predictor for delayed graft function (OR = 1.1; p = 0.028). Conclusions DIA is superior to and more consistent than routine optic microscopy for interstitial fibrosis evaluation. This method could improve implantation biopsy diagnostics and help to reduce disposal of organs. Graphical abstract

Adult intussusception is a rare entity which is distinct from paediatric cases in incidence, aetiology, and management. It represents 5% of all intussusceptions and is the cause of 1% of all intestinal obstructions, 0,08% of all abdominal surgeries and 0,003-0,02% of all hospital admissions. Ileocolic intussusception in adults is a unique variant in which nearly 100% of cases have a malignant lead point. In our report, we described a case of a patient with ileocecal intussusception caused by a rare type of the gastrointestinal tumor. The female patient was admitted to hospital for occasional pain in the lower right quadrant of the abdomen followed by abdominal discomfort and appearance of blood in the stool. The result of CT scan of the abdomen and pelvis showed a tumor mass and intussusception at the ileocecal junction, which was confirmed peroperatively. Open right hemicolectomy was performed adhering to oncological principles. The final pathologic diagnosis indicated the gastrointestinal stromal tumor of the ileocecal valvе. The diagnosis of intussusception in adults is delicate, and timely surgical treatment can be vital. Patients with the palpable abdominal mass, digestive tract obstruction, gastrointestinal bleeding, or lead point computed tomography must undergo a surgical examination. Given a high risk of malignancy, primary surgical resection using oncologic principles presents the best option for the treatment of ileocecal intussusception in adults.

BACKGROUND Cardiovascular disease is the leading nonmalignant cause of late deaths in childhood cancer survivors. Cardiovascular disease and cardiac dysfunction can remain asymptomatic for many years, but eventually lead to progressive disease with high morbidity and mortality. Early detection and intervention are therefore crucial to improve outcomes. OBJECTIVE In our study, we aim to assess the prevalence of preclinical cardiac dysfunction in adult childhood cancer survivors using conventional and speckle tracking echocardiography; determine the association between cardiac dysfunction and treatment-related risk factors (anthracyclines, alkylating agents, steroids, cardiac radiation) and modifiable cardiovascular risk factors (abdominal obesity, hypertension); investigate the development of cardiac dysfunction longitudinally in a defined cohort; study the association between cardiac dysfunction and other health outcomes like pulmonary disease, endocrine disease, renal disease, quality of life, fatigue, strength and endurance, and physical activity; and gain experience conducting a clinical study of childhood cancer survivors that will be extended to a national, multicenter study of cardiac complications. METHODS For this retrospective cohort study, we will invite ≥5-year childhood cancer survivors who were treated at the University Children's Hospital Bern, Switzerland with any chemotherapy or cardiac radiation since 1976 and who are ≥18 years of age at the time of the study for a cardiac assessment at the University Hospital Bern. This includes 544 childhood cancer survivors, of whom about half were treated with anthracyclines and/or cardiac radiation and half with any other chemotherapy. The standardized cardiac assessment includes a medical history focusing on signs of cardiovascular disease and its risk factors, a physical examination, anthropometry, vital parameters, the 1-minute sit-to-stand test, and echocardiography including 2-dimensional speckle tracking. RESULTS We will invite 544 eligible childhood cancer survivors (median age at the time of the study, 32.5 years; median length of time since diagnosis, 25.0 years) for a cardiac assessment. Of these survivors, 300 (55%) are at high risk, and 244 (45%) are at standard risk of cardiac dysfunction. CONCLUSIONS This study will determine the prevalence of preclinical cardiac dysfunction in Swiss childhood cancer survivors, inform whether speckle tracking echocardiography is more sensitive to cardiac dysfunction than conventional echocardiography, and give a detailed picture of risk factors for cardiac dysfunction. The results will help improve primary treatment and follow-up care of children with cancer. CLINICALTRIAL ClinicalTrials.gov NCT03790943; https://clinicaltrials.gov/ct2/show/NCT03790943 INTERNATIONAL REGISTERED REPORT DERR1-10.2196/17724

Introduction. Gastric cancer (GC) represents one of the most common cancers worldwide, frequently diagnosed at advanced stages with poor prognosis, indicating on need for new diagnostic and prognostic markers. The aim of the study was to determine the expression of IL-32, proinflammatory and angiogenic mediators, in patients with diffuse and intestinal gastric cancer and the relationship with clinicopathological aspects. Material and Methods. The tissue samples of diffuse and intestinal types of tumor of 70 patients with gastric cancer were analyzed. Expression of IL-32, VEGF, IL-17, and CD31 was measured by immunohistochemistry. Results. IL-32 expression was significantly lower in tissue samples from patients with diffuse type of gastric cancer that is also a severe and more progressive form (TNM stages III and IV, poor histological differentiation, and higher nuclear grade III). Expression of IL-17 was also decreased in patients with diffuse type of gastric cancer. Microvascular density was diminished in diffuse type of gastric cancer. Conclusions. Downregulated expression of IL-32 in tumor tissue of patients with diffuse type of gastric cancer may implicate on its role in limiting ongoing proinflammatory and proangiogenic processes. This emphasizes on unrecognized role of IL-32 in biology of diffuse type of gastric cancer.

Hyperhomocysteinemia is one of the unconventional risk factors of cardiovascular morbidity - it is causally associated in both directions with oxidative stress, endothelial dysfunction, mechanisms of cell signaling and apoptosis. Numerous clinical and experimental studies have explained the mechanisms and clinical expression of atherosclerotic and prothrombotic effects of homocysteine. The main pathological substrate, previous therapy, and surgical procedure-intervention itself are associated with the state of oxidative stress and endothelial dysfunction, which is one of important factors for elevated plasma homocysteine levels. Recent studies point to the possibility of a successful correction of hyperhomocysteinemia, especially secondary one.

New findings on the activity of myeloperoxidase in the course of an inflammatory process associated with infections and vascular lesions indicate its role in the state of oxidative stress and endothelial dysfunction as well as its predictive value for cardiovascular insults. The products of actions by myeloperoxidase represent an integrative mechanism connecting the tissue lesions, infectious, inflammatory processes, oxidative stress, endothelial dysfunction and the development of vascular insult (the most common are the arterial and venous thrombosis and consecutive embolism). The most common complications of an operative surgical treatment are vascular. Seen from a conservative standpoint the most common are arterial and venous thrombosis, but more broadly suture dehiscence could be considered as a consequence of the impaired microcirculation. Operational procedures as well as the underlying pathology lead to the activation of inflammatory cascade with the activation of myeloid lineage cells whereby a release and activation of myeloperoxidase, which in addition to antimicrobial exerts multiple vascular operations. Increased concentrations of myeloperoxidase in the plasma up to three months before the clinical expression of vascular insult indicates its predictive value. Current research portrays the possibility of adjusting the successful result of the action of myeloperoxidase and prevention of vascular complications.

Objectives Reconstruction of the right ventricular outflow tract plays a major role in congenital cardiac surgery. With the advent of the Contegra bovine jugular vein graft and the Shelhigh pulmonic xenograft, hopes were high that the lack of availability of homografts would be overcome. The present study evaluated both grafts and investigated the influence of known risk factors for premature graft failure. Methods From December 1999 to September 2008, 84 consecutive patients (mean age, 12 ± 15 years) with a total of 100 implanted conduits (43 Contegra bovine jugular vein grafts and 57 Shelhigh pulmonic xenografts) were included in this study. Primary end points were reintervention, reoperation, and death. Results The rate of overall conduit replacement was 25% for the Shelhigh pulmonic xenograft versus 26% for the Contegra bovine jugular vein graft. The predominant mode of failure was conduit stenosis for both groups (23% for the Shelhigh pulmonic xenograft vs 19% for the Contegra bovine jugular vein graft), with a mean time to replacement of 18 ± 9 months for the Shelhigh pulmonic xenograft versus 42 ± 4 months for the Contegra bovine jugular vein graft ( P = .25). Histopathological analysis revealed a similar chronic inflammatory reaction in both conduits, but it was significantly stronger in the Shelhigh pulmonic xenograft group. The Contegra bovine jugular vein graft showed frequently the formation of a stenotic membrane at the distal anastomosis site. Age of less than 1 year, body surface area, pulmonary stenosis, and conduit size of less than 14 mm could not be identified as risk factors for premature failure. Conclusions Both conduits fail predominantly because of stenosis and are subject to a chronic inflammatory reaction, although this was stronger in the Shelhigh pulmonic xenograft group. Mean time to replacement was 18 ± 9 months for the Shelhigh pulmonic graft group versus 42 ± 4 months for the Contegra bovine graft group ( P = .25). Because there is a trend toward earlier failure in the Shelhigh pulmonic xenograft group, we currently prefer to implant the Contegra bovine jugular vein graft for right ventricular outflow tract reconstruction.

Remarkable advances in ultrasound imaging technology have made it possible to diagnose fetal cardiovascular lesions as early as 12-14 weeks of gestation and to assess their physiological relevance by echocardiography. Moreover, invasive techniques have been developed and refined to relieve significant congenital heart disease (CHD), such as critical aortic and pulmonary stenoses in the pediatric population including neonates. Recognition of the fact that certain CHDs can evolve in utero, and early intervention may improve the outcome by altering the natural history of such conditions has led to the evolution of a new fetal therapy, i.e. fetal cardiac intervention. Two entities, pulmonary valvar atresia and intact ventricular septum (PA/IVS) and hypoplastic left heart syndrome (HLHS), are associated with significant morbidity and mortality even with postnatal surgical therapy. These cases are believed to occur due to restricted blood flow, leading to impaired growth and function of the right or left ventricle. Therefore, several centers started the approach of antenatal intervention with the primary goal of improving the blood flow through the stenotic/atretic valve orifices to allow growth of cardiac structures. Even though centers with a reasonable number of cases seem to have improved the technique and the immediate outcome of fetal interventions, the field is challenged by ethical issues as the intervention puts both the mother and the fetus at risk. Moreover, the perceived benefits of prenatal treatment have to be weighed against steadily improving postnatal surgical and hybrid procedures, which have been shown to reduce morbidity and mortality for these complex heart defects. This review is an attempt to provide a balanced opinion and an update on fetal cardiac intervention.

We sought to assess the safety and efficacy of percutaneous closure of atrial septal defects (ASDs) under fluoroscopic guidance only, without periprocedural echocardiographic guidance.Percutaneous closure of ASDs is usually performed using simultaneous fluoroscopic and transthoracic, transesophageal (TEE), or intracardiac echocardiographic (ICE) guidance. However, TEE requires deep sedation or general anesthesia, which considerably lengthens the procedure. TEE and ICE increase costs.Between 1997 and 2008, a total of 217 consecutive patients (age, 38 ± 22 years; 155 females and 62 males), of whom 44 were children ≤16 years, underwent percutaneous ASD closure with an Amplatzer ASD occluder (AASDO). TEE guidance and general anesthesia were restricted to the children, while devices were implanted under fluoroscopic guidance only in the adults. For comparison of technical safety and feasibility of the procedure without echocardiographic guidance, the children served as a control group.The implantation procedure was successful in all but 3 patients (1 child and 2 adults; 1.4%). Mean device size was 23 ± 8 mm (range, 4-40 mm). There was 1 postprocedural complication (0.5%; transient perimyocarditis in an adult patient). At last echocardiographic follow-up, 13 ± 23 months after the procedure, 90% of patients had no residual shunt, whereas a minimal, moderate, or large shunt persisted in 7%, 1%, and 2%, respectively. Four adult patients (2%) underwent implantation of a second device for a residual shunt. During a mean follow-up period of 3 ± 2 years, 2 deaths and 1 ischemic stroke occurred.According to these results, percutaneous ASD closure using the AASDO without periprocedural echocardiographic guidance seems safe and feasible.

Differential expression of sarcoplasmic reticulum Ca(2+)-ATPase (SERCA2a) and phospholamban (PLB) has been shown in heart failure and atrial arrhythmias. We investigated the influence of volume overload and age on their expression in pediatric atrial myocardium. Right atrial specimens from 18 children with volume overloaded right atrium (VO) and 12 patients without overload were studied. Each group was further divided into patients less than and older than 12 months of age. Only in the younger patients SERCA2a was significantly reduced in the VO group. In younger patients PLB mRNA level tended to be lower in VO. The PLB:SERCA protein ratio was significantly reduced in the VO group. Age itself did not influence the SERCA2a and PLB expression, if the hemodynamic overload was not taken into account. This study is the first to show a combined influence of volume overload and age on atrial SERCA2a expression.

Zu untersuchen, ob verschiedene Ultrafiltrationsmethoden oder Filtertypen den Zytokinverlauf bei Kinderherzoperationen unterschiedlich beeinflussen. 41 Kinder

ObjectiveThis study was undertaken to assess whether different filter types or ultrafiltration methods influence inflammatory markers in pediatric cardiac surgery.MethodsForty-one children younger than 5 years were prospectively randomized to groups A (polyamid filter with conventional ultrafiltration), B (polyamid filter with modified ultrafiltration), C (polysulfon filter with conventional ultrafiltration), and D (polysulfon filter with modified ultrafiltration). Interleukin 6, interleukin 10, tumor necrosis factor, terminal complement complex, and lactoferrin were measured before the operation (T0), before rewarming (T1), after ultrafiltration (T2), at 6 (T3) and 18 hours (T4) after the operation, and in the ultrafiltrate.ResultsAll markers changed with both ultrafiltration methods, both filter types, and in all groups (except tumor necrosis factor) along the T0 to T4 observation time (P < .0001). Their patterns of changes were different for terminal complement complex, with less decrease after use of the polysulfon filter (P < .05), and among groups A through D for interleukin 6 (P = .01), with more decrease in group C than group A (P < .02). Interleukin 10 decreased with the polyamid filter (P < .001) but not with the polysulfon filter. In the ultrafiltrate, tumor necrosis factor was higher with the polysulfon filter than the polyamid filter (6.8 ± 5 pg/mL vs 4.0 ± 3.7 pg/mL, P < .05). The ultrafiltrate/plasma ratio of interleukin 6 was higher with conventional ultrafiltration than modified ultrafiltration (0.018 ± 0.017 vs 0.004 ± 0.007, P < .005).ConclusionsThe polysulfon filter showed a filtration profile for inflammatory mediators superior to that of the polyamid filter for interleukin 6, tumor necrosis factor, and interleukin 10. Interleukin 6 was most efficiently removed by conventional ultrafiltration with a polysulfon filter, and tumor necrosis factor was best removed by modified ultrafiltration with a polysulfon filter, whereas other inflammatory mediators were not influenced by filter type or ultrafiltration method. Therefore combined conventional and modified ultrafiltration with a polysulfon filter may currently be the most effective strategy for removing inflammatory mediators in pediatric heart surgery.

Splenicne ciste veoma su retke i javljaju se sa incidencom od 0,5–2,0% u opstoj populaciji. Najcesce su parazitarne, a mnogo ređe neparazitarne etiologije. Neparazitarne ciste mogu biti prave i lažne u zavisnosti od toga da li zid supljine ima epitelni omotac ili ne. U ovom radu predstavljamo pacijentkinju od 24 godine sa ogromnom, neparazitarnom epidermalnom cistom slezine.

Dilation of the ascending aorta is rare in childhood. When seen, it is usually associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We describe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, also in infancy. A third child has had repair of an atrioventricular septal defect with exclusively atrial shunting, whereas the fourth patient had a structurally normal heart. The aortic valve had three leaflets, and was functionally normal in all. The dilation of the ascending aorta was progressive in all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.

Atrial septal defects (ASDs) are typically asymptomatic in infancy and early childhood, and elective defect closure is usually performed at ages of 4 to 6 years. Severe pulmonary hypertension (PH) complicating an ASD is seen in adulthood and has only occasionally been reported in small children. A retrospective study was undertaken to evaluate the incidence of severe PH complicating an isolated ASD and requiring early surgical correction. During a 10-year period (1996 to 2006), 355 pediatric patients underwent treatment for isolated ASDs either surgically or by catheter intervention at 2 tertiary referral centers. Two hundred ninety-seven patients had secundum ASDs, and 58 had primum ASDs with mild to moderate mitral regurgitation. Eight infants were found with isolated ASDs (6 with secundum ASDs and 2 with primum ASDs) associated with significant PH, accounting for 2.2% of all patients with ASDs at the centers. These 8 infants had invasively measured pulmonary artery pressures of 50% to 100% of systemic pressure. They were operated in the first year of life and had complicated postoperative courses requiring specific treatment for PH for up to 16 weeks postoperatively. The ultimate outcomes in all 8 infants were good, with persistent normalization of pulmonary pressures during midterm follow-up of up to 60 months (median 28). All other patients with ASDs had normal pulmonary pressures, and the mean age at defect closure was significantly older, at 6.2 years for secundum ASDs and 3.2 years for primum ASDs. In conclusion, ASDs were rarely associated with significant PH in infancy but then required early surgery and were associated with excellent midterm outcomes in these patients.

Few data exist on the incidence of spontaneously occurring ventricular tachycardia (VT) in an unselected pediatric population. The aim of this study was to define the incidence and outcomes of VT in a general pediatric population. A retrospective analysis was performed of all documented episodes of VT in children referred to a single center during a 10-year study period ending in December 2005. The study center drains a stable referral area with 252,000 children aged

The aim of this study was to evaluate two putative predictive genetic markers for hypertension in children and adolescents with diabetes mellitus. Ambulatory blood pressure measurements were performed in 199 patients with type I diabetes mellitus (mean age 16.5 years, mean duration of diabetes 7.7 years) and compared to those of 1141 healthy children. The local allele frequencies were established based on a control population consisting of 181 healthy subjects. The allele frequencies of the angiotensinogen gene M235T polymorphism was nearly identical in insulin-dependent diabetes mellitus patients (MM 33%, MT 51%, TT 16%) and controls (MM 35%, MT 49%, TT 16%). In contrast, the genotype distribution of the angiotensin I converting enzyme gene insertion/deletion (I/D) polymorphism was different between patients with type I diabetes mellitus (DD 26%, ID 49%, II 25%) and the control group (DD 37%, ID 44%, II 19%) (P=0.04). Relative nocturnal systolic and diastolic pressures in patients with diabetes were higher than in healthy age- and height-matched controls; no association was found with the angiotensinogen gene M235T polymorphism. Relative nocturnal diastolic blood pressure was higher in patients homozygous for the I allele of the angiotensin I converting enzyme gene.Nocturnal systolic and diastolic blood pressure is higher in patients with type I diabetes than in healthy children. The formerly described, but controversial, association of the M235T polymorphism with arterial hypertension could not be confirmed in this study.

Introduction. Blount disease is developmental disorder of the lower leg, manifested by multiplanar deformity. Surgical treatment includes corrective osteotomy, lateral hemiepiphisyodesis, distraction of the proximal tibial physis, physeal bar resection and elevation of the medial tibial plateau. Case Outline. A case of a 4-year-old girl with bow legs is presented. Condition was recognized as Blount disease, type five. Both lower legs were operated by medial methaphyseal semi-osteotomy of the proximal tibia with the elevation of the medial tibial plateau. Prospectively, bilateral proximal hemiepiphysiodesis was done. Total follow-up period was eighteen years. The patient has no disturbances, clinical and radiographic findings are excellent. Improvement of the femoro-tibial angle is 22° on the right side, and 21° on the left side. Improvement of the varus angle is 7° on the right side, and 27° on the left side. Medial plateau depression is completely bilaterally reduced; improvement on the right side is 46°, and 51° on the left side. Conclusion. It is known today that multiplanar deformity is a part of the disease; varus, antecurvatum and internal rotation of the lower leg. By elevation of the medial plateau varus of the lower and antecurvatum component of deformity can be solved, while internal torsion cannot be solved. This deformity has to be either skillfully neglected, or corrected by an additional osteotomy by the elevation of the medial tibial plateau. Lateral hemiepiphysiodesis serves as extra stabilisator of the achieved result, and it is recommended to be done in combination with surgical elevation of the medial tibial plateau and derotative corrective osteotomy of the tibia. [Projekat Ministarstva nauke Republike Srbije, br. 41004]

Introduction. Femoral neck fractures in children and adolescents are rare. However, their complications are frequent - avascular necrosis, femoral neck pseudoarthrosis, premature physeal closure with consequent growth disturbance and coxa vara deformity. Case Outline. A 9.5­year­old boy was injured in a car accident, and femoral neck fracture was diagnosed. Prior to admission at our hospital he was surgically treated several times. He was admitted at our hospital eight months following the accident. On the X­ray transcervical pseudoarthrosis of the femoral neck was found, as well as coxa vara deformity and metaphyseal avascular necrosis. He was operated at our hospital; all previously placed ostefixation material was removed, valgus osteotomy of 30 degrees was done as well as additional local osteoplasty using the commercial osteoindactive agent (Osteovit®). Postoperatively, we applied skin traction, bed rest and physical therapy. At the final follow­up, the patient was recovered completely. He is now painless, the legs are of equal length, range of movements in the left hip is full, life activity is normal. The X­ray shows that the femoral neck pseudoarthrosis is fully healed. Conclusion. This case is presented in order to encourage other colleagues to challenge the problematic situation such as this one. Also, we would like to remind them what one should think about and what should be taken into consideration in the primary treatment of femoral neck fractures in children. Valgus femoral osteotomy, as a part of the primary treatment of femoral neck fracture in children (identically as in the adults) can prevent the occurrence of femoral neck pseudoarthrosis.

Objectives: Surgical correction of complex tracheo-esophageal obstructions due to vascular rings and slings has long been considered as ultima ratio. It was unclear whether patients would benefit in the long-term or if relief of symptoms was only temporary. Aim of the current study was to provide long-term follow-up beyond infancy. Patients and methods: Data were retrospectively collected from all patients that underwent surgery for complex tracheo-esophageal obstructions due to vascular rings and slings at this institution since 1992. Results: Twenty-seven patients were identified and provided a mean follow-up of 9.4y. Two patients were lost to follow-up. Diagnosis was double aortic arch (10 [40%]), right-sided aortic arch (6 [24%]), A. lusoria (3 [12%]), pulmonary artery sling (2 [8%]) and miscellaneous (4 [16%]). Tracheo- or bronchomalacia was present in 10 [40%] and 3 [12%] patients, respectively. Two-thirds of patients (16 [64%]) suffered from associated cardiac defects. Two-thirds of patients (17 [67%]) had onset of symptoms shortly after birth whereas one-third (8 [33%]) presented later in childhood. Diagnosis was established at an average age of 6y (0–14y). Average time between onset of symptoms and referral for surgery was 4.1y. Operative mortality was zero. Technical success was confirmed by perioperative bronchoscopy. Only 4 patients demonstrated residual symptoms during follow-up leading to re-operation in 2 patients due to persistent dysphagia and recurrent tracheal stenosis, respectively. Conclusion: Patients with complex tracheo-oesophageal lesions due to vascular malformations benefit from surgical intervention in the long-term. The current data suggests that surgery is a viable treatment option in this patient population and should not only be considered as a bail-out.

Management of homozygous familial hypercholesterolaemia is notoriously difficult. For these patients, LDL apheresis is considered the treatment of choice. Treatment initiation is advocated generally from the age of seven years onwards (Thompson et al., Atherosclerosis 198:247-255, 2008). Here, we present the case of a young girl from a large inbred family of Turkish descent with homozygous familial hypercholesterolaemia and fatal outcome at the early age of 4½ years.In conclusion, this case suggests that management of homozygous familial hypercholesterolaemia may require earlier and more aggressive treatment, including LDL apheresis before the age of seven years.

Although U-clip anastomoses were studied for hemodynamics and patency, their potential for unimpeded growth after congenital cardiovascular surgery has not been investigated yet. In 53 children aged 2.1+/-3.3 years operated on between March 1998 and August 2005 growth of U-clip (U) vs. polypropylene running sutured (P) anastomoses in coarctation repair (Coarc; n=26), bi-directional Glenn (BDG; n=13) and arterial switch operation (ASO; n=14) was retrospectively analysed. Coarc showed 2.39+/-4.33 vs. 3.09+/-2.24 mm of growth during the observation period (21+/-16 vs. 30+/-27 months); no growth (0 vs.16%), restenosis (14 vs. 37%) and reinterventions (14 vs. 11%) were similar (all in U vs. P, P=ns). BDG showed 3.68+/-3.43 vs. 2.50+/-2.55 mm (P=ns) of growth during 15+/-5 vs. 29+/-18 months (P=0.046); no growth (17 vs. 0%), stenosis (0 vs. 14%) and reinterventions (0%) were similar in U vs. P, respectively (P=ns). Main pulmonary artery (MPA) anastomosis in ASO showed 0.28+/-1.73 vs. 1.30+/-3.16 mm of growth during 8+/-14 vs. 28+/-28 months; no growth (60 vs. 14%), stenosis (50 vs. 63%) and reinterventions (0%) were similar (all in U vs. P, P=ns). Anastomotic growth, stenosis and reintervention rates show no difference between interrupted U-clip and polypropylene running sutured technique in Coarc repair, BDG and MPA anastomosis in ASO.

BACKGROUND: Newborns with hypoplastic left heart syndrome (HLHS) or right heart syndrome or other malformations with a single ventricle physiology and associated hypoplasia of the great arteries continue to be a challenge in terms of survival. The vast majority of these forms of congenital heart defects relate to abnormal morphogenesis during early intrauterine development and can be diagnosed accurately by fetal echocardiography. Early knowledge of these conditions not only permits a better understanding of the progression of these malformations but encourages some researchers to explore new minimally invasive therapeutic options with a view to early pre- and postnatal cardiac palliation. DATA SOURCES: PubMed database was searched with terms of "congenital heart defects", "fetal echocardiography" and "neonatal cardiac surgery". RESULTS: At present, early prenatal detection has been applied for monitoring pregnancy to avoid intrauterine cardiac decompensation. In principle, the majority of congenital heart defects can be diagnosed by prenatal echocardiography and the detection rate is 85%-95% at tertiary perinatal centers. The majority, particularly of complex congenital lesions, show a steadily progressive course including subsequent secondary phenomena such as arrhythmias or myocardial insufficiency. So prenatal treatment of an abnormal fetus is an area of perinatal medicine that is undergoing a very dynamic development. Early postnatal treatment is established for some time, and prenatal intervention or palliation is at its best experimental stage in individual cases. CONCLUSION: The upcoming expansion of fetal cardiac intervention to ameliorate critically progressive fetal lesions intensifies the need to address issues about the adequacy of technological assessment and patient selection as well as the morbidity of those who undergo these procedures.

We present a 12-year-old girl with clinical manifestation of restenosis after coarctation repair in the neonatal period. However, MR-angiography revealed hypoplasia of the distal arch, complete absence of the descending aorta and severe hypoplasia of the thoraco-abdominal aorta. Treatment consisted of an extra-anatomic ascending-descending PTFE graft via sternotomy with normalization of upper-body hypertension and disappearance of leg claudication. It demonstrates how this very rare pathology of true segmental aortic agenesia may successfully be treated in older children with coexisting aortic arch or isthmus anomalies. Long-term follow-up is needed to assess the potential benefit of this approach.

Background Despite advances in surgical and interventional techniques, the optimal surgical treatment of severe aortic (re) coarctation and hypoplastic aortic arch is still controversial. Anatomic repair may require extensive dissection, cardiopulmonary bypass, and deep hypothermic circulatory arrest with their inherent risks. The aim of this study was to analyze the outcome of off-pump extraanatomic aortic bypass as a surgical alternative to local repair. Methods From February 2000 to December 2005, ten consecutive patients (median age 20 years; range, 11 to 38 years) with severe aortic (re) coarctation (n = 4) and (or) hypoplastic aortic arch (n = 7) underwent off-pump extraanatomic aortic bypass through median sternotomy. All but three patients had undergone previous surgery for coarctation and angioplasty or stenting. Three patients underwent concomitant replacement of the ascending aorta because of an aneurysm using cardiopulmonary bypass. Results Postoperative hospital course was uneventful in all patients. There was no perioperative mortality or significant morbidity. During a mean follow-up of 48 ± 22 months no patient required additional procedures. All patients were free of symptoms; no patient showed signs of heart failure after follow-up. At last follow-up, no patient presented with claudication, nor any patient experienced orthostatic problems due to a steal phenomenon. During follow-up, hypertension resolved in all patients with residual mild hypertension in two patients. Conclusions Off-pump extraanatomic aortic bypass is an attractive treatment option for complex aortic (re) coarctation and hypoplastic aortic arch. Perioperative risks are minimized, hypertension is influenced favorably, and midterm survival is event-free.

Myocardial depression after cardiac surgery is modulated by cardiopulmonary bypass (CPB) and the underlying heart disease. The sodium pump is a key component for myocardial function. We hypothesized that the change in sodium pump expression during CPB correlates with intraoperative and postoperative laboratory and clinical parameters in neonates and children with various congenital heart defects. Sodium pump isoforms alpha1 (ATP1A1) and alpha3 (ATP1A3) mRNA expression in right atrial myocardium, excised before and after CPB, was quantified. Groups were assigned according to presence (VO group, n = 8) or absence (NO group, n = 8) of right atrial volume overload. CPB and aortic clamp time correlated with postoperative troponin-1 values and ICU stay. ATP1A1 (P = 0.008) and ATP1A3 (P = 0.038) mRNA expression were significantly reduced during CPB. Longer aortic clamp times were associated with lower postoperative ATP1A1 (P = 0.045) and ATP1A3 (P = 0.002) mRNA expression. Low postoperative ATP1A1 (P = 0.043) and ATP1A3 (P = 0.002) expressions were associated with high troponin-I values. These results were restricted to the VO group. No correlation of sodium pump mRNA expression was found with the duration of ICU stay or ventilation. The postoperative troponin-I and clinical parameters correlated with the length of CPB, regardless of volume overload. In contrast, only dilated right atrium seemed to be susceptible to CPB in terms of sodium pump expression, showing a reduction during the operation and a correlation of sodium pump with postoperative troponin-I values.

A differential expression of sarcoplasmic reticulum calcium-ATPase (SERCA2a) and phospholamban (PLB) characterizes the remodeling process in heart failure and atrial arrhythmias in adult patients. Gender is known to modulate the course and Prognosis of different forms of heart disease. We hypothesized that gender plays a role in molecular changes of myocardial calcium regulating components already in childhood. Moreover, we studied the influence of volume overloaded (VO) on SERCA2a and PLB in pediatric patients. Quantitative reverse transcription-polymerase chain reaction was used to measure mRNA expression of SERCA2a and PLB in atrial myocardium from 30 pediatric patients (12 girls, 18 boys). Eighteen patients had VO right atria, and 12 patients had not-overloaded atria (NO). Protein expression was studied by Western blot. In the entire population, SERCA2a and PLB expression was not different between girls and boys. If hemodynamic overload was taken into account, SERCA2a mRNA expression was significantly reduced in the VO group compared with the NO group (P = 0.021). The VO versus NO difference was restricted to toys, which corresponds to a highly significant interaction of gender versus VO status (P = 0.002). The PLB to SERCA2a Protein ratio was significantly lower in girls (P = 0.028). The decrease in SERCA2a mRNA expression in VO atrial myocardium and the PLB to SERCA2a ratio of protein expression was modulated by gender in this pediatric population. To our knowledge, this study is the first to show the impact of gender on the differential expression of calcium-regulating components in Pediatric cardiac patients.

Objective(s) Even though the mechanism is not clearly understood, direct intramyocardial cell transplantation has demonstrated potential to treat patients with severe heart failure. We previously reported on the bioengineering of myoblast-based constructs. We investigate here the functional outcome of infarcted hearts treated by implantation of myoblast-seeded scaffolds. Methods Adult Lewis rats with echocardiography-confirmed postinfarction reduced ejection fraction (48.3% ± 1.1%) were randomized to (1) implantation of myoblast-seeded polyurethane patches at the site of infarction (PU-MyoB, n=11), (2) implantation of nonseeded polyurethane patches (PU, n=11), (3) sham operation (Sham, n=12), and (4) direct intramyocardial myoblast injection (MyoB, n=11). Four weeks later, the functional assessment by echocardiography was repeated, and we additionally performed left ventricular catheterization plus histologic studies. Results The ejection fraction significantly decreased in the PU (39.1% ± 2.3%; P = .02) and Sham (39.9% ± 3.5%; P = .04) groups, whereas it remained stable in the PU-MyoB (48.4% ± 3.1%) and MyoB (47.9% ± 3.0%) groups during the observation time. Similarly, left ventricular contractility was significantly higher in groups PU-MyoB (4960 ± 266 mm Hg/s) and MyoB (4748 ± 304 mm Hg/s) than in groups PU (3909 ± 248 mm Hg/s, P = .01) and Sham (4028 ± 199 mm Hg/s, P = .01). Immunohistology identified a high density of myoblasts within the seeded scaffolds without any migration toward the host cardiac tissue and no evidence of cardiac cell differentiation. Conclusions Myoblast-seeded polyurethane scaffolds prevent post–myocardial infarction progression toward heart failure as efficiently as direct intramyocardial injection. The immunohistologic analysis suggests that an indirect mechanism, potentially a paracrine effect, may be assumed.

Cardiopulmonary bypass (CPB) may induce serious side effects, potentially leading to myocardial failure. The Na(+)-K(+)-ATPase is a key component for myocardial function. Due to its developmental regulation, results from adult studies cannot be adopted to the situation in childhood. Right atrial myocardium from patients with left-to-right shunts at atrial level (VO, n=8) and those without (NO, n=8) was excised during heart surgery before and after CPB. Na(+)-K(+)-ATPase isoforms ATP1A1 (p=0.008) and ATP1A3 (p=0.038) decreased during CPB, which decrease was restricted to the VO group. This study highlights the importance of the underlying heart defect for susceptibility to the effects of CPB, showing a reduced Na(+)-K(+)-ATPase mRNA expression only in patients with left-to-right shunts on the atrial level. This seemed to be an early molecular event, as apart from one, none of the patients showed heart failure before or after surgery.

Background The study aimed at defining the excess morbidity or mortality caused by an additional airway malformation in children with congenital heart disease requiring surgery. Methods All patients requiring surgery for heart disease during an 8-year period ending in 2003 who had an associated upper airway malformation were retrospectively studied. All patients were seen in 2004 for a prospective follow-up examination. Results Eleven patients with upper airway anomalies were identified (tracheobronchial malacia in 6 patients, long-segment tracheal stenosis in 3, and bilateral vocal cord paralysis and tracheal hemangioma in 1 patient each). They accounted for 1.5% of the entire cardiac surgical load of 764 patients. In 5 infants, the airway anomaly was diagnosed before cardiac repair, in 6 patients thereafter. Diagnosis was made by bronchoscopy in all patients, by additional bronchography in 2. Failure of rapid postoperative extubation was the most common finding. Airway management was surgical in 2 and conservative in 8 patients, 1 newborn having been denied therapy because of the severity of airway hypoplasia. Compared with patients with isolated cardiac disease, those with additional airway anomalies had significantly longer duration of postoperative mechanical ventilation (median, 24 days versus 3), perioperative hospitalization (median, 72 days versus 11) and total number of days of hospitalization during the first year of life (median, 104 days versus 14). After a maximum follow-up of 8 years (median, 37 months) only 3 of 10 surviving patients remained symptomatic owing to the airway malformation. Conclusions Upper airway anomalies accompanying heart disease in infancy resulted in a significant prolongation of perioperative intensive care and hospital stay, as well as duration of mechanical ventilation. Failure of early postoperative extubation was the leading symptom.

Background Current techniques for repair of the right ventricular outflow tract (RVOT) may require interposition of a valved conduit between the right ventricle and the pulmonary artery bifurcation. Recently, the Contegra conduit (Medtronic, Inc.) was introduced as an alternative xenograft tissue for RVOT reconstruction. Promising early hemodynamic and clinical results have been reported so far, but still less is known about mid-term adverse outcome. Methods A total of 38 Contegra valved conduits (12 to 22 mm) were implanted from October 1999 to June 2004, in 36 children less than 5 years old and in 2 patients 8 and 21 years old. Diagnosis included the following: tetralogy of Fallot (n = 21); pulmonary atresia (n = 4); double outlet right ventricle + pulmonary stenosis (n = 3); d-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (n = 3); truncus arteriosus (n = 3); and other complex malformations (n = 4). Results There was no mortality following initial surgery and no valved-conduit–related early morbidity. Early postoperative echocardiographic assessment after 3 months demonstrated favorable hemodynamics in all patients. However, during further follow-up, 5 conduits had to be replaced because of severe stenosis at the level of the distal anastomosis (2 of them had moderate to severe dilatation of the conduit proximally to the valve). Excessive intimal peel formation and severe perigraft scarring reaction were observed in all cases. One child died before surgery. Conclusions The Contegra valved conduit is an interesting concept for reconstruction of the RVOT. However, because of unpredictable incidence of supravalvar stenosis during mid-term results, we cannot recommend routine use of this material.

Many different mechanical vascular anastomotic devices have been developed recently, mostly rigid stenting mechanisms applicable only in coronary artery bypass grafting surgery. U-Clips, however, allow the precise construction of any vascular interrupted anastomosis, preserving pulsatility and perhaps growth potential.We report the first use worldwide of U-Clips for congenital cardiac surgery in 10 pediatric patients (mean age, 2.3 +/- 1.7 years). The operations took place between July 2001 and July 2002 for coarctation repair (3 patients), Glenn shunt (5 patients), Blalock-Taussig shunt (1 patient), and arterial switch (1 patient).Device handling, primary hemostasis, and patency were excellent, and no device-related complications occurred. Because of the learning curve, aortic cross-clamp times were significantly longer for the repair of coarctation with U-Clips than with the running-suture technique (21.7 +/- 2.3 minutes versus 14.4 +/- 2 minutes; P = .012). Postoperative recoveries were uneventful in all patients except for a baby with pulmonary atresia with intact ventricular septum, who died after 62 days. After a mean follow-up period of 11.9 +/- 4.4 months, echocardiographic controls of all anastomoses showed nonturbulent flow without any restriction.The U-Clip device may be a useful adjunct in congenital cardiac surgery for facilitating the creation of interrupted vascular anastomoses. Further evaluation is warranted for determining the long-term benefits of these devices with respect to growth preservation and restenosis.