Complications related to transhepatic venous access in the catheterization laboratory--a single center 12-year experience of 124 procedures.

Objectives: To identify complications and measures that can be undertaken to prevent complications from transhepatic central venous access.

Background: Utility and safety data from a large experience of patients undergoing transhepatic venous access are lacking.

Methods: The records of patients who underwent transhepatic venous access between June 2000 and October 2012 at The Center for Pediatric and Congenital Heart Disease at The Cleveland Clinic were reviewed.

Transhepatic Broviac catheter placement for long-term central venous access in critically ill children with complex congenital heart disease.

Objective: Critically ill children with cardiac disease often require prolonged central venous access. Thrombosis of systemic veins or the need to preserve vessels for future cardiac procedures limits sites for placement of central venous catheters in these patients. This study evaluates the use of Broviac placement via the transhepatic approach for this patient population.

Nursing interventions in pediatric palliative care.

Caring for chronically ill children whose health is declining and thosechildren who are critically ill is stressful and can be overwhelming to bothhealthcare professionals and parents. Palliative care teams aim to providethe multidisciplinary and holistic support staff and families need. Families,nurses, and primary medical teams value the involvement of a pediatric pal-liative medicine team to improve the quality of living.

Acute pancreatitis after anesthesia with propofol in a child with glycogen storage disease type IA.

Glycogen storage disorder type 1A (GSD 1A) is an inherited disorder of glycogen metabolism characterized by fasting hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. These children have a higher risk of developing pancreatitis because of hypertriglyceridemia. Drug-induced pancreatitis accounts for a small proportion of cases of pancreatitis.

Analysis of prognostic factors of acute lymphoblastic leukemia in infants: report on CCG 1953 from the Children's Oncology Group.

Infant acute lymphoblastic leukemia (ALL) has a poor therapeutic outcome despite attempts to treat it based on prognostic factor-guided therapy. This is the first cooperative group trial characterizing all infants at the molecular level for MLL/11q23 rearrangement. All infants enrolled on Children's Cancer Group (CCG) 1953 were tested for MLL rearrangement by Southern blot and the 11q23 translocation partner was identified (4;11, 9;11, 11;19, or "other") by reverse-transcriptase polymerase chain reaction (PCR).

Pediatric mechanical circulatory support.

Extracorporeal membrane oxygenation (ECMO) is based on technology that has been used clinically for decades. Largely because of its widespread use in pediatric patients with respiratory failure, familiarity with this technology has established ECMO as the most commonly used form of circulatory support for children. However, clinical applications and technical aspects of ECMO support continue to evolve, ensuring the ongoing importance of this modality for pediatric circulatory support.

The pedipump: development status of a new pediatric ventricular assist device.

The PediPump is a new rotary dynamic ventricular assist device designed specifically for pediatric applications. Although it is capable of providing support for adults, the small size of the PediPump makes it suitable for newborn circulatory support while retaining excellent hemodynamics. Current and future development plans include: (1) determination of the basic engineering requirements for hardware and control logic, including design analysis for system sizing, evaluation of control concepts and bench testing of prototypes; (2) performance of preclinical anatomical fitting studies using computed tomography-based three-dimensional modeling; and, (3) evaluation with animal studies to provide characterization and reliability testing of the device.

Management of the failing systemic right ventricle.

Conditions in which the right ventricle serves as the systemic pumping chamber are frequently complicated by the development of right ventricular failure and tricuspid valve regurgitation. The right ventricle is the systemic ventricle in conditions of ventriculoarterial discordance with atrioventricular concordance (transposition of the great arteries) or with atrioventricular discordance (congenitally corrected transposition of the great arteries). Concerns regarding actual or potential systemic right ventricular failure in these cases may lead to surgical evaluation and treatment designed to reestablish the left ventricle as the systemic pump.

Gastroschisis revisited: role of intraoperative measurement of abdominal pressure.

Background: Animal studies have shown that visceral circulation is well preserved when intraabdominal pressure does not exceed 20 mm Hg. Our aim was to analyze the outcomes of a series of infants with gastroschisis whose surgical management was directed by the intraoperative measurement of bladder pressure.

Methods: Forty-two neonates with gastroschisis were surgically managed using intraoperative measurement of bladder pressure at a tertiary care center between July 31, 1992, and March 20, 2004, and their outcome was evaluated.

Apical right ventriculotomy for closure of apical ventricular septal defects.

Background: Apical ventricular septal defects (VSDs) are difficult to visualize through a transatrial approach, while the use of a left ventriculotomy may result in progressive ventricular dysfunction. Transcatheter closure has not been reliable, especially in small infants. Apical right ventriculotomy provides good exposure, preserves left ventricular function, and allows secure closure of apical VSDs.

A brief group cognitive-behavioral intervention for social phobia in childhood.

Twenty-three preadolescent children (ages 8-11) meeting criteria for social phobia were randomly assigned to either a 3-week cognitive-behavioral group intervention or a wait-list control group. The intervention consisted of psychoeducation, cognitive strategies, and behavioral exposure. Outcome measures included diagnostic interview as well as parent and child report measures of anxiety and depression.

Selective timing for the arterial switch operation.

Background: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status.

Methods: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution.

Results: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age.

Trial of metronidazole vs. azithromycin for treatment of cyclosporine-induced gingival overgrowth.

Gingival overgrowth usually characterized by increased cellular growth of gingival fibroblasts appears to be multifactorial. In patients receiving CyA for more than 3 months, the incidence can approach 70% and can be attributed to pharmaceutical immunosuppression. Case reports have reported regression of overgrowth with both metronidazole and azithromycin.

Creating an international comprehensive web-based curriculum in pediatric radiology.

Objective: This article describes the creation of a standardized comprehensive resident curriculum in pediatric radiology that uses adult learning principles authored by international experts and addresses the six general competencies required by the Accreditation Council for Graduate Medical Education.

Conclusion: Web-based learning with an online curriculum has the potential to become an integral component of residency training. The use of radiology experts as authors allows compilation of an authoritative, comprehensive, and current body of knowledge that enhances the current teaching file approach to Web-based instruction in radiology.

Genetic basis for resistance to parathyroid hormone.

Pseudohypoparathyroidism (PHP) is associated with biochemical hypoparathyroidism (i.e. hypocalcemia and hyperphosphatemia) due to parathyroid hormone (PTH) resistance rather than to PTH deficiency.

Pulmonary arteriovenous malformations after cavopulmonary anastomosis.

Pulmonary arteriovenous malformations (PAVMs) are a cause of progressive cyanosis after cavopulmonary anastomosis in children with single ventricle physiology who are on the pathway leading to a Fontan procedure. Investigations into possible mechanisms for the etiology of PAVMs are ongoing and suggest that the liver might play a key regulatory role in the development of these lesions.

Intracapsular tonsillar reduction (partial tonsillectomy): reviving a historical procedure for obstructive sleep disordered breathing in children.

Objective: We sought to reintroduce a historical procedure-intracapsular tonsillar reduction (partial tonsillectomy or tonsillotomy)-for tonsillar hypertrophy causing obstructive sleep disordered breathing (OSDB) in children, as well as to determine whether partial tonsillectomy, compared with conventional (total) tonsillectomy when performed by more than one surgeon, is equally effective for the relief of OSDB while resulting in less pain and more rapid recovery.

Study Design: We conducted a retrospective case series at a tertiary children's hospital. The charts of children who underwent partial tonsillectomy and total tonsillectomy (1998 through 2002) for postoperative complications were reviewed.

Incidence and severity of vesicoureteral reflux in children related to age, gender, race and diagnosis.

Purpose: We determine if the incidence and grade of vesicoureteral reflux (VUR) differs in children based on age, race and gender, and if the incidence and severity of VUR are related to race in girls younger than 7 years presenting for evaluation after urinary tract infection (UTI).

Materials And Methods: The records of all children who underwent a voiding cystourethrogram or radionuclide cystogram between 1993 and 2001 were retrospectively reviewed. Age, gender, race, clinical indication and highest grade of VUR were recorded for the first voiding cystourethrogram or radionuclide cystogram.

Results of the double switch operation for congenitally corrected transposition of the great arteries.

Objective: To determine the outcomes for a program that utilizes the double switch operation as the primary approach for congenitally corrected transposition.

Methods: The records of 46 consecutive patients from a single institution who had undergone a double switch operation from October 1993 to March 2002 were reviewed. The records of 24 patients who were evaluated during the same period and felt not to be candidates for the double switch operation or who are awaiting double switch after pulmonary artery banding were also reviewed.

Anatomic correction for corrected transposition after pulmonary unifocalization.

A Senning plus Rastelli operation was performed in a patient who had a rare combination of congenitally corrected transposition of the great arteries (S,L,L) with dextrocardia, major aortopulmonary collaterals, and diminutive central pulmonary arteries with arborization defects. The patient required four preparatory operations including bilateral unifocalizations of the aortopulmonary artery collaterals. Pulmonary artery to systemic pressure ratio after the double switch operation was 0.

Fate of the pouch in 151 pediatric patients after ileal pouch anal anastomosis.

Background/purpose: Ileal pouch anal anastomosis (IPAA) offers many pediatric patients a surgical cure for mucosal ulcerative colitis (MUC) with preservation of anal continence. However, some patients incur serious problems after surgery including chronic pouchitis and pouch failure. The goal of this study is to identify clinical and pathologic factors that are associated with an adverse outcome of IPAA.

Which children and adults should receive the chickenpox vaccine?

Chickenpox vaccine is safe and effective and elicits long-standing immunity. Primary care physicians caring for children and adults should be aware of its benefits.