Clinical utility of urinary gluten immunogenic peptides in the follow-up of patients with coeliac disease.

Background: Gluten-free diet (GFD) is the only treatment for patients with coeliac disease (CD) and its compliance should be monitored to avoid cumulative damage.

Aims: To analyse gluten exposures of coeliac patients on GFD for at least 24 months using different monitoring tools and its impact on duodenal histology at 12-month follow-up and evaluate the interval of determination of urinary gluten immunogenic peptides (u-GIP) for the monitoring of GFD adherence.

Methods: Ninety-four patients with CD on a GFD for at least 24 months were prospectively included.

Aortic root translocation and en bloc rotation of the outflow tracts surgery for complex forms of transposition of the great arteries and double outlet right ventricle: A multicenter study.

Objective: There are several choices for the correction of complex transposition of the great arteries and double outlet right ventricle not amenable to the Rastelli-type surgery, but outcome data are limited to small series. This study aims to report results after the aortic root translocation and en bloc rotation of the outflow tract procedures.

Methods: This is a retrospective, multicentric, observational study.

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines.

Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries.

Timing of surgical repair of the stented aortic arch and coarctation in neonates.

We report a neonate with aortic arch hypoplasia and coarctation, in whom patency of the arterial duct could not be re-established and who was too ill to undergo primary surgical correction safely. This patient was treated in two stages: 1) angioplasty and stenting, 2) surgical correction. The safe period for surgical correction may be 2-3 weeks after angioplasty and stenting.

Mechanical ventilation during cardiopulmonary bypass in neonates improves postoperative outcome.

Objectives: Cardiopulmonary bypass generates a systemic inflammatory response. This inflammatory response is reduced if patients are ventilated during bypass, as evidenced by lower levels of postoperative circulating inflammatory mediators. However, this does not appear to make much clinical difference in adults, at least not consistently, but, to our knowledge, has never been assessed in paediatric cardiac surgery, which is the objective of this study.

The Predicament of Surgical Correction of Tetralogy of Fallot.

Surgery for tetralogy of Fallot has a long history, which may be described as both a success story and a failure story. It is a success story because prognosis without surgery is very poor, but surgery makes it possible for affected babies to reach adulthood and lead productive lives. It is a failure story, however, since we still cannot cure this condition; we can only palliate it as illustrated in the sobering long-term outcome of affected patients.

Negative predictive value of the repeated absence of gluten immunogenic peptides in the urine of treated celiac patients in predicting mucosal healing: new proposals for follow-up in celiac disease.

Background: The treatment of celiac disease (CD) is a lifelong gluten-free diet (GFD). The current methods for monitoring GFD conformance, such as a dietary questionnaire or serology tests, may be inaccurate in detecting dietary transgressions, and duodenal biopsies are invasive, expensive, and not a routine monitoring technique.

Objectives: Our aim was to determine the clinical usefulness of urine gluten immunogenic peptides (GIP) as a biomarker monitoring GFD adherence in celiac patients and to evaluate the concordance of the results with the degree of mucosal damage.

The morphologically right and left ventricles cannot be distinguished by their coronary arterial pattern.

Objectives: The morphologically right and left ventricles are distinguished from each other based on their internal anatomical features, because their external (epicardial) surfaces do not appear to have any distinguishing mark for such ventricular identification. Nevertheless, ventricular identification based on epicardial characteristics, if these were possible, would be interesting to surgeons, because this would enable them to identify each ventricle rapidly upon opening the chest. This made us curious as to whether or not the two ventricles may be distinguished based on their epicardial coronary arterial patterns, because this is the most obvious epicardial ventricular feature.

Follow-up of a simple method for aortic valve reconstruction with fixed pericardium in children.

In 2013, we published a simple method for aortic valve reconstruction in children using fixed pericardium in this journal. This was not expected to be a durable solution but a temporizing measure in the absence of other solutions, in order to buy time for growth that would allow subsequent prosthetic valve insertion. As such, this was implemented in 3 patients with excellent immediate results, as reported in our original publication.

Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma.

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that does not metastasize. We report two unique cases of KHE involving the right hemithorax and the upper ipsilateral extremity. Kasabach-Merrit phenomenon and osteolytic lesions in the scapula were observed in both cases.

Ectopic Vas Deferens Inserting Into Distal Retroiliac Ureter in the Currarino Syndrome.

Vas deferens ectopia is a rare congenital anomaly frequently associated with anorectal abnormalities and hypospadias. We present a Currarino syndrome case with an ectopic vas deferens terminating in a distal retroiliac ureter. A left vasectomy, ureteral decussation over the iliac vessels, and a Cohen's-type ureteral reimplantation were performed.

Analysis of the therapeutic evolution in the management of airway infantile hemangioma.

Aim: To analyze the evolution in the management of airway infantile hemangioma (AIH) and to report the results from 3 pediatric tertiary care institutions.

Methods: A retrospective study of patients with diagnosis of AIH and treated in 3 pediatric tertiary care institutions from 1996 to 2014 was performed.

Results: Twenty-three patients with diagnosis of AIH were identified.

Effectiveness of propranolol in the treatment of infantile hemangioma beyond the proliferation phase.

During the last 5 years, many studies have shown the efficacy of propranolol as first-line treatment for infantile hemangiomas (IHs), but not much has been written about the role of propranolol beyond the proliferation phase of IH (>1 year). Our aim was to assess propranolol efficacy and safety in the treatment of patients older than 1 year. A retrospective study of patients older than 1 year diagnosed with IH and treated in our vascular anomalies clinic between 2009 and 2013 was performed.

The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon.

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas.

Lower Extremity Fibro-Adipose Vascular Anomaly (FAVA): A New Case of a Newly Delineated Disorder.

The acronym FAVA (Fibro-Adipose Vascular Anomaly) has been recently given to a distinct vascular entity that is characterized by fibrofatty infiltration of muscle, unusual phlebectasia with pain, and contracture of the affected extremity. We report a new case of FAVA in a 10-year-old girl with pain in her right lower leg and equinus contracture. As in our case, FAVA typically presents in young females with calf involvement and limited ankle dorsiflexion with local pain.

Association Between Lymphatic Malformations of the Mediastinum and Congenital Venous Ectasia: Is It Just Coincidental?

The association between lymphatic malformations and congenital venous ectasia is extremely rare, and the relative rarity of both entities makes it difficult to determine the relationship between the two. Here we present four new cases and review the current literature. We hypothesize that there might be a strong association between mediastinal lymphatic malformations and segmental phlebectasia; furthermore, there is a molecular background that may justify the association between these two entities.

Differential diagnosis and management of liver tumors in infants.

During the first year of life, most of the liver neoplasms are benign in origin, but some of these histologically benign lesions may be challenging in their management. Although most hepatic hemangiomas can be safely observed until involution is documented, some patients will need treatment due to progressive hepatomegaly, hypothyroidism and/or cardiac failure. Large mesenchymal hamartomas may require extensive hepatic resection and an appropriate surgical plan is critical to obtain good results.

Hepatoblastoma cells express truncated neurokinin-1 receptor and can be growth inhibited by aprepitant in vitro and in vivo.

Background & Aims: Multidrug resistance presents a major problem in hepatoblastoma (HB), and new anti-tumor strategies are desperately needed. The substance P (SP)/neurokinin-1 receptor (NK1R) complex has been discovered to be pivotal in the development of a variety of human cancers, and NK1R antagonists, such as the clinical drug aprepitant, are promising future anticancer agents. Yet, the role of the SP/NK1R complex as a potential anticancer target in HB is unknown.

Lumbosacral abortive hemangioma with intradural extension.

Abortive hemangioma (AH) is a true hemangioma of infancy that expresses glucose transporter-1 protein in the endothelial cells, with an arrested growth cycle. We present the rare case of a lumbosacral AH with intramedullary extension.

Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon.

Background: . This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA).

Procedure: .

The relationship between the site of metastases and outcome in children with stage IV Wilms Tumor: data from 3 European Pediatric Cancer Institutions.

The aim of this study was to analyze in detail the site of metastasis of stage 4 Wilms tumor (WT) and its correlation with outcome. The databases from 3 major European pediatric cancer institutions were screened for children with WT between 1994 and 2011. Of 208 children identified, 31 (14.

Primary paratesticular neuroblastoma: an important differential diagnosis.

Primary neuroblastoma as a paratesticular tumor is extremely rare with only 8 described cases worldwide. In this article, we present the case of a 5-month-old boy with this rare tumor location and give an update on the current literature. As in our case, typically these tumors present as Stage 1 disease and simple tumorectomy alone leads to excellent outcome and long-term survival.