Severe Direct Hyperbilirubinemia as a Consequence of Right Heart Failure in Congenital Heart Disease.

Adult literature documents increased cholestasis in right heart failure yet is poorly documented in the pediatric population. We describe three infants with congenital heart disease who developed significantly elevated direct bilirubin levels of 43, 23, and 12 mg/dL, respectively, in the absence of hepatic dysfunction. The common hemodynamic pathophysiology in these infants is right heart dysfunction with moderate to severe tricuspid regurgitation in the setting of low perfusion state.

Improved Survival in Left Liver-Up Congenital Diaphragmatic Hernia by Early Repair Before Extracorporeal Membrane Oxygenation: Optimization of Patient Selection by Multivariate Risk Modeling.

Background: Delayed repair of congenital diaphragmatic hernia (CDH) for days or longer has become standard, allowing improved stabilization for many, but potentially complicating treatment in severely affected infants who require extracorporeal membrane oxygenation (ECMO) and arrive unrepaired. Survival in left liver-up CDH, the most severe anatomic subset, averages 45% in published studies, with deaths often occurring in patients who failed to improve on ECMO and are repaired late, or not at all. Reliable early prediction of ECMO risk in these patients could identify the best candidates for repair before ECMO.

Cytomegalovirus transmission in pediatric renal transplant recipients during the window period.

We report two CMV naïve children who received deceased donor renal transplants from a CMV IgG-negative single donor. CMV IgG in both recipients and the donor were negative immediately prior to transplant. Both recipients had early recurrences of their original disease in their transplants, requiring multiple sessions of plasmapheresis.

Caring for Pediatric Patients' Families at the Child's End of Life.

Nurses play an important role in supporting families who are faced with the critical illness and death of their child. Grieving families desire compassionate, sensitive care that respects their wishes and meets their needs. Families often wish to continue relationships and maintain lasting connections with hospital staff following their child's death.

Non-accidental Trauma Work-up: Unusual Retinal Finding Leads to a Rare Diagnosis.

Lipoprotein lipase (LPL) deficiency is an autosomal recessive condition due to absent or decreased activity of LPL enzyme. The LPL deficiency is a rare condition that is mainly diagnosed in children, but there is no standard screening method at this time. In our report, we describe a 6-day-old male infant who was found to have hypertriglyceridemia after lipemia retinalis was diagnosed from a fundoscopic examination for nonaccidental trauma work-up.

Delay in Arrival to Care in Perpetrator-Identified Nonaccidental Head Trauma: Observations and Outcomes.

Background: Children who sustained nonaccidental head trauma (NAHT) are at severe risk for mortality within the first 24 hours after presentation.

Objective: Extent of delay in seeking medical attention may be related to patient outcome.

Methods: A 10-year, single-institution, retrospective review of 48 cases treated at a large tertiary Children's Hospital reported to the New York State Central Registrar by the child protection team was conducted.

Anorexia nervosa in a pediatric renal transplant recipient and its reversal with cyclosporine.

We report a 16-yr-old female who developed AN within a month after renal transplantation and its resolution after switching from tacrolimus to cyclosporine. Her initial maintenance immunosuppressive regimen after renal transplantation consisted of tacrolimus, mycophenolate, and steroid. She had 7 kg weight loss within the first month of transplant with subsequent 10, 12, 17, and 19 kg loss after three, five, seven, and nine months of transplant, respectively.

Pediatric Longitudinal Clivus Fracture: Survival With Minimal Morbidity.

Longitudinal clivus fractures are rare in children, with only 5 cases published in the English literature to date. Clivus fractures, particularly longitudinal type, are associated with high mortality and morbidity. We report a case of longitudinal clivus fracture in a teenager with survival and complete neurological recovery.

Nature of the underlying heart disease affects survival in pediatric patients undergoing extracorporeal cardiopulmonary resuscitation.

Objective: To describe the use of extracorporeal membrane oxygenation (ECMO) in acute resuscitation after cardiac arrest in pediatric patients with heart disease, with reference to patient selection and predictors of outcome.

Methods: A retrospective medical record review was performed of all patients aged ≤21 years with heart disease who had undergone ECMO for cardiopulmonary resuscitation (ECPR) at Texas Children's Hospital from January 2005 to December 2012. The most recent Pediatric Overall Performance Category score was determined from the patients' medical records.

Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience.

Background: Diabetes is associated with increased morbidity and mortality in patients with cystic fibrosis (CF). While liver transplantation is well established for CF-related liver disease (CFLD), the role of simultaneous liver-pancreas transplantation is less understood.

Methods: We polled 81 pediatric transplantation centers to identify and characterize subjects who had undergone simultaneous liver-pancreas transplantation and obtain opinions about this procedure in CFLD.

Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific treatment algorithm.

Objectives: To assess the impact of varying approaches to congenital diaphragmatic hernia (CDH) repair timing on survival and need for ECMO when controlled for anatomic and physiologic disease severity in a large consecutive series of patients with CDH.

Background: Our publication of 60 consecutive patients with CDH in 1999 showed that survival was significantly improved by limiting lung inflation pressures and eliminating hyperventilation.

Methods: We retrospectively reviewed 268 consecutive patients with CDH, combining 208 new patients with the 60 previously reported.

Missed viral surveillance testing visits associate with full blown viral diseases in children receiving kidney transplants.

Surveillance testing for major viral infections such as CMV, EBV, and BKV early in their natural history course may allow for early intervention and prevention of FBVD, but the testing is expensive and optimal interval/frequency are uncertain. At our center we initiated routine monthly viral surveillance for CMV, EBV, and BKV in July 2008 for the first 12 months post-transplant. Here, we retrospectively analyzed for outcome of the patients who missed three or more surveillance tests in the first 12 months post-transplant vs.

Herpes simplex virus hepatitis in infants: clinical outcomes and correlates of disease severity.

Objective: To better characterize the clinical outcomes of infants with herpes simplex virus (HSV) infection and identify useful correlates of disease severity.

Study Design: Infants aged ≤6 months with HSV infection treated between 1999 and 2009 were identified. In patients with concurrent hepatitis, laboratory and clinical variables were examined to identify predictors of specific outcomes, including death or the need for liver transplantation and the need for intensive care.

Improved survival with recent Post-Transplant Lymphoproliferative Disorder (PTLD) in children with kidney transplants.

Post-transplant lymphoproliferative disorder (PTLD) has been associated with high mortality, but recent anecdotal survival appeared better. From 1988 to 2010, the NAPRTCS registry had 235 registered PTLD cases. We sent a special 25-point questionnaire study to the NAPRTCS centers with the most recent 150 cases to obtain additional follow-up data not collected in the master registry, our objective being to determine the recent outcomes after PTLD and determine prognostic factors.

The BK virus in renal transplant recipients-review of pathogenesis, diagnosis, and treatment.

The BK virus, a DNA virus from the Polyomavirus group, represents an opportunistic infection of immunosuppressed transplant recipients. Though the virus was discovered approximately 40 years ago, the emergence of BK virus nephropathy since 1995 onwards, with associated high graft loss rates, has revolutionized renal transplantation medicine. Kidney transplant professionals realized that the consequences of over-immunosuppression were as severe as the consequences of under-immunosuppression and we entered the era of immunosuppressive minimization.

Hepatic expression of cell proliferation markers and growth factors in giant cell hepatitis: implications for the pathogenetic mechanisms involved.

Objectives: The aim of this study is to determine whether amitotic division or nuclear proliferation is involved in the formation of giant cells (GCs) in giant cell hepatitis (GCH).

Patients And Methods: Liver sections from 18 pediatric patients with idiopathic infantile GCH and 12 patients with postinfantile GCH were evaluated for the expression of proliferating cell nuclear antigen (PCNA) and human histone 3 (H3) mRNA, transforming growth factor-alpha (TGF-α), TGF-β1, hepatocyte growth factor (HGF), and epidermal growth factor receptor (EGFR).

Results: Proliferation markers were detected in 1% to 80% in the nuclei of GC and non-GC hepatocytes in 10 of 18 (56%) infantile GCH biopsies and 11 of 12 (92%) postinfantile GCH biopsies, but not in normal liver.

Comparison of generic tacrolimus and Prograf drug levels in a pediatric kidney transplant program: brief communication.

A generic version of tacrolimus was approved for use in the USA in August 2009. These narrow therapeutic index generics are tested for bioequivalence only in adults. No data are available on generic tacrolimus levels in children with allografts.

Post-transplant lymphoproliferative disorder in children: recent outcomes and response to dual rituximab/low-dose chemotherapy combination.

PTLD is a major complication after transplantation. Treatment options for PTLD are not standardized, usually sequential, starting with reduction in immunosuppression. Recently, we have used a dual combination of rituximab and reduced dose chemotherapy (R/C) directly after failed RI.

Retransplantation after BK virus nephropathy in prior kidney transplant: an OPTN database analysis.

BK virus (BKV) has emerged as a major complication of kidney transplantation. Since June 30, 2004, the OPTN in the USA collects BKV as a primary or secondary cause of graft loss and also if treatment for BK virus (TBKV) is administered. In this study, we determined characteristics of those recipients of repeat kidney transplants from the OPTN database, where either (a) a graft loss occurred between June 30, 2004 and December 31, 2008 and database recorded prior TBKV in that allograft or (b) a graft loss between June 30, 2004 and December 31, 2008 was attributed primarily or secondarily due to BKV.