Pediatric Candidates Research Articles

Pediatric transplantation

Analysis of the OPTN/SRTR database demonstrates that, in 2002, pediatric recipients accounted for 7% of all recipients, while pediatric individuals accounted for 14% of deceased organ donors. For children fortunate enough to receive a transplant, there has been continued improvement in outcomes following all forms of transplantation. Current 1-year graft survival is generally excellent, with survival rates following transplantation in many cases equaling or exceeding those of all other recipients. In renal transplantation, despite excellent early graft survival, there is evidence that long-term graft survival for adolescent recipients is well below that of other recipients. A causative role for noncompliance is possible. While the significant improvements in graft and patient survival are laudable, waiting list mortality remains excessive. Pediatric candidates awaiting liver, intestine, and thoracic transplantation face mortality rates generally greater than those of their adult counterparts. This finding is particularly pronounced in patients aged 5 years and younger. While mortality awaiting transplantation is an important consideration in refining organ allocation strategies, it is important to realize that other issues, in addition to mortality, are critical for children. Consideration of the impact of end-stage organ disease on growth and development is often equally important, both while awaiting and after transplantation.

Organ donation and transplantation trends in the USA, 2003

This summary provides an overview of solid organ transplantation in the USA, produced as part of the 2003 OPTN/SRTR Annual Report. The Annual Report is prepared by the Scientific Registry of Transplant Recipients (SRTR) in collaboration with the Organ Procurement and Transplantation Network (OPTN) under contract with the Health Resources and Services Administration (HRSA). A wide range of activities related to solid organ transplantation in the USA are thoughtfully addressed in this publication with the intention of providing useful information to patients, the transplant community, the public, and the Federal Government.

1152-204 Exercise capacity in pediatric heart transplant candidates

1152-204 Exercise capacity in pediatric heart transplant candidates

MELD/PELD and the allocation of deceased donor livers for status 1 recipients with acute fulminant hepatic failure, primary nonfunction, hepatic artery thrombosis, and acute Wilson's disease.

1. Historical perspective of donor allocation to patients with fulminant hepatic failure (FHF). 2. Predicting prognosis in patients with FHF using the London and Clichy criteria. 3. Model for end-stage liver disease (MELD) is a predictor of mortality in patients with FHF. 4. Outcomes of adults listed as Status 1 in the United States. 5. Outcomes of pediatric candidates listed as Status 1 in the United States. 6. Proposed redefinition for Status 1 in adult and pediatric candidates.

Osteoporosis May Be Overdiagnosed in Children

Bone health in children is important for proper growth and to avert osteoporosis later in life. Dual-energy x-ray absorptiometry (DEXA) is the standard tool for assessing bone mineralization. Investigators reexamined the DEXA scans of 34 pediatric candidates for a NIH trial of osteoporosis therapy. At least …

Outcomes of orthotopic liver transplantation in Chile

Our liver transplant program was started in 1993 in a private clinic and a public hospital. Thereafter, a rapid increase in adults and pediatric candidates for this therapeutic option lead to this analysis of results in 165 orthotopic liver transplants (OLT) in 143 patients between November 1993 and December 2002. Seventy-four OLT were performed in 66 adult patients and 91 in the pediatric group. Liver grafts came from cadaveric donors in 145 cases (74 adults and 71 children). The technique of living-related donor was utilized in 20 pediatric cases. Main indications for OLT in the adult group were HCV cirrhosis, primary biliary cirrhosis; biliary atresia and acute liver failure were the indications in pediatric patients. Retransplantation was needed for 23 patients, including 9 adults and 14 children. The most frequent causes of death were sepsis, graft primary nonfunction, and vascular complications. Actuarial survivals at 1 and 5 years were 80.7% and 72.6% for the adult group and 82% and 74.8% for the pediatric group, respectively. Our results are comparable to those published by large, experienced, international centers, with much better financial support.

Intrathecal baclofen therapy in children with cerebral palsy: Efficacy and complications

Murphy NA, Nicole Irwin MC, Hoff C. Intrathecal baclofen therapy in children with cerebral palsy: efficacy and complications. Arch Phys Med Rehabil 2002;83:1721-5. Objectives: To describe the efficacy of intrathecal baclofen (ITB) therapy in the management of spasticity in young children with cerebral palsy (CP) and to identify risk factors for complications. Design: Consecutive case series of 25 implanted ITB delivery systems during a 48-month period. Setting: Pediatric specialty hospital and outpatient department. Participants: Twenty-three children (age range, 4.5–17.4y) with CP (spastic diplegia in 22%; spastic quadriplegia in 61%; mixed-type diplegia in 4%; mixed-type quadriplegia in 13%). Intervention: Intrathecal baclofen therapy in children with cerebral palsy. Main Outcome Measures: Ashworth Scale scores before treatment and at 6 and 12 months after ITB therapy; frequency and nature of complications; and relation between patient characteristics and outcomes. Results: Average Ashworth scores ± standard deviation decreased from 3.26±.73 to 2.34±.83 (P≤.01) in the lower extremities and from 2.69±.79 to 2.00±.55 (P≤.05) in the upper extremities 6 months after ITB therapy and remained comparably decreased at 12 months. Explantation was required in 44% (11/25), with wound complications as the leading cause in 73% (8/11). Complications were associated with the diagnosis of mixed-type CP, as compared with pure spastic types (P≤.01). Trends suggest that children of smaller size and younger age, as well as those with gastrostomy tubes and nonambulatory status, were more likely to encounter complications necessitating explantation. Conclusion: ITB therapy effectively reduced spasticity in children with CP. However, complications necessitating explantation can occur. Further research is needed to identify criteria describing the ideal pediatric candidate for ITB. © 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation

Pediatric liver transplantation

Pediatric liver transplantation is a fast-growing and challenging field. Healthcare providers must stay informed of advancements in the management of liver transplant candidates and recipients. The goal of this paper is to provide nurses who care for pediatric liver transplant candidates and recipients with a review of the basic medical management of these patients, from the preoperative evaluation to postoperative care.

Pediatric Liver Transplantation

Pediatric liver transplantation is a fast-growing and challenging field. Healthcare providers must stay informed of advancements in the management of liver transplant candidates and recipients. The goal of this paper is to provide nurses who care for pediatric liver transplant candidates and recipients with a review of the basic medical management of these patients, from the preoperative evaluation to postoperative care.

Selecting Children and Adolescents for Insulin Pump Therapy: Medical and Behavioral Considerations

The use of continuous insulin therapy via a subcutaneously implanted catheter connected to an external pump is being used increasingly in the management of childhood type 1 diabetes. This form of intensive diabetes management places multiple demands on young patients to achieve near-normal blood glucose levels. Therefore, appropriate selection of pediatric candidates for pump therapy is critical to achieving successful outcomes. Although few empirical data are available on readiness evaluations conducted with pump candidates, clinical data suggest several factors important in the consideration of these individuals, including age, medical history, and psychosocial functioning. The focus of this case presentation is to describe key factors for health care professionals to consider in transitioning children and adolescents to the insulin pump, based on our pediatric pump program at Children’s National Medical Center in Washington, D.C. S.P., is a 10-year-old boy with a 3-year history of type 1 diabetes. In May 2000, while on three insulin injections per day, he requested insulin pump therapy in order to increase lifestyle flexibility. His height was at the 90–95th percentile, and his weight was at greater than the 75th percentile. His hemoglobin A1c (A1C) concentration was 6.9%. His anticipated pump start was set to coincide with his tenth birthday. As a requirement of our program, S.P. was asked to learn carbohydrate counting and to obtain psychological consultation per our center’s protocol. After psychological and nutritional evaluations, there were concerns related to untreated anxiety with obsessive-compulsive tendencies as well as the need for additional carbohydrate-counting practice. We therefore delayed his pump start for 5 months and asked S.P. and his family to seek additional psychological counseling and nutritional support to increase the likelihood of pump success. ### Medical and Behavioral Considerations Insulin pump therapy provides a more physiologically precise manner of delivering insulin compared to self-injections with less variable insulin absorption …

Techniques for and outcome of liver transplantation in neonates and infants weighing up to 5 kilograms

Background: Neonates and small infants represent less than 5% of paediatric candidates for liver replacement. Most cases present under urgent conditions and receive grafts from large donors. Surgical techniques must be adapted for adequate graft preparation, vascular reconstruction, and abdominal closure. Methods: Technical aspects and outcome of 15 liver transplantations in infants weighing less than 5 kg performed at our unit were analysed retrospectively. Results: Liver transplantation was performed under urgent or highly urgent condition in 13 cases. Reduced or split liver grafts were used in all cases (median donor to recipient weight ratio, 9), including a monosegmental graft in 2 cases. In 10 cases, vascular reconstruction was done using a vascular conduit (5, 4, and 1 for artery, portal, and hepatic veins, respectively) and a delayed closure of the abdomen was necessary in 7 children. Postoperative complications were as follows: thrombosis of hepatic artery (n = 1) or portal vein (n = 1), gastrointestinal haemorrhage (n = 2), intraperitoneal bleeding (n = 1), biliary stricture (n = 2), septicaemia (n = 1). Two infants died of brain damage with a functioning graft. One child underwent retransplant for chronic rejection. Conclusions: Overall, survival rate is 60% (median follow-up, 34 months), which compares favourably with older patient groups when case mix is comparable. J Pediatr Surg 37:159-164. Copyright © 2002 by W.B. Saunders Company.

CHALLENGES IN THE TREATMENT OF HEPATITIS C IN CHILDREN

Hepatitis C infection in children is associated with a unique set of challenges for clinicians and investigators. Although the prevalence of HCV infection is lower in children than in adults, it is important to identify infected children to monitor progression of liver disease and to make appropriate interventions to minimize factors that may exacerbate progression. Identification requires understanding of risk factors important in children, primarily exposure at or near the time of birth. The natural history of this infection in most children is either more benign or significantly prolonged than that of infection acquired in adulthood. Reasons for this difference in natural history must be explored and possibly even exploited in the care of adult patients with HCV infection. Identification of appropriate pediatric candidates for treatment and definition of optimal therapy for these children require ongoing study. Lastly, as perinatal transmission becomes the primary mode of acquisition for new pediatric infections, factors that increase or decrease the likelihood of this transmission must be identified, and effective preventive interventions must be put into practice. There are important differences in the clinical features, natural history, and response to therapy between pediatric and adult patients with HCV infection. Understanding of these differences will allow optimal care for affected children and perhaps better understanding of the pathophysiology and pure natural history of this disease.

Temporal lobe epilepsy due to hippocampal sclerosis in pediatric candidates for epilepsy surgery.

To characterize the clinical, EEG, MRI, and histopathologic features and explore seizure outcome in pediatric candidates for epilepsy surgery who have temporal lobe epilepsy (TLE) caused by hippocampal sclerosis (HS). The authors studied 17 children (4 to 12 years of age) and 17 adolescents (13 to 20 years of age) who had anteromesial temporal resection between 1990 and 1998. All patients had seizures characterized by decreased awareness and responsiveness. Automatisms were typically mild to moderate in children and moderate to marked in adolescents. Among adolescents, interictal spikes were almost exclusively unilateral anterior temporal, as opposed to children in whom anterior temporal spikes were associated with mid/posterior temporal, bilateral temporal, extratemporal, or generalized spikes in 60% of cases. MRI showed hippocampal sclerosis on the side of EEG seizure onset in all patients. Fifty-four percent of children and 56% of adolescents had significant asymmetry of total hippocampal volumes, whereas the remaining patients had only focal atrophy of the hippocampal head or body. Subtle MRI abnormalities of ipsilateral temporal neocortex were seen in all children and 60% of adolescents studied with FLAIR images. On histopathology, there was an unexpectedly high frequency of dual pathology with mild to moderate cortical dysplasia as well as HS, seen in 79% of children and adolescents. Seventy-eight percent of patients were free of seizures at follow-up (mean, 2.6 years). A tendency for lower seizure-free outcome was observed in patients with bilateral temporal interictal sharp waves or bilateral HS on MRI. The presence of dual pathology did not portend poor postsurgical outcome. TLE caused by HS similar to those in adults were seen in children as young as 4 years of age. Focal hippocampal atrophy seen on MRI often was not reflected in total hippocampal volumetry. Children may have an especially high frequency of dual pathology, with mild to moderate cortical dysplasia as well as HS, and MRI usually, but not always, predicts this finding. Postsurgical seizure outcome is similar to that in adult series.

Mental retardation in pediatric candidates for epilepsy surgery: the role of early seizure onset.

We sought to determine whether early age at seizure onset is a risk factor for mental retardation, independent of etiology. Assessment of risk for mental retardation with continued uncontrolled seizures plays a role in considerations of timing for epilepsy surgery. Previous studies have indicated that onset of seizures in the first years of life may be a risk factor for mental retardation, but the etiologies of the epilepsies were not included in the analyses. Intellectual function was assessed at ages 2-20 years during presurgical evaluation in 100 patients with intractable epilepsy due to focal lesions limited to part of one lobe of the brain. Mental retardation (MR) was defined as Full-Scale Intelligence Quotient (FSIQ) < or =70. The age at seizure onset and the seizure frequency were obtained retrospectively. Younger ages at seizure onset were associated with lower FSIQ scores, and mean FSIQ was also significantly lower for patients with onset of epilepsy at < or =24 months of age (74.0 +/- 21.5) versus that in patients with onset of epilepsy later in life (87.8 +/- 18.8; p = 0.005). The frequency of patients with MR was significantly higher for patients with seizure onset at < or =24 months of age (15 of 33, 46%) than for patients with seizure onset later in life (eight of 67, 12%; p < 0.001). This difference persisted within etiologic subgroups. For patients with focal malformation of cortical development, MR was seen in eight (50%) of 16 patients with seizure onset at < or =24 months versus two (10%) of 20 patients with seizure onset at >24 months (p < 0.001); for patients with tumor, MR was seen in four (50%) of eight patients with seizure onset at < or =24 months versus four (13%) of 30 patients with seizure onset at >24 months (p = 0.003); and for patients with hippocampal sclerosis, MR was seen in two (28%) of seven patients with seizure onset at < or =24 months versus none of 30 patients with seizure onset at >24 months (NS). Within the subgroup with daily seizures, MR was present in 13 (65%) of 20 patients with seizure onset at < or =24 months versus five (17%) of 29 patients with seizure onset later in life (p = 0.001). These results indicate that onset of intractable epilepsy within the first 24 months of life is a significant risk factor for MR, especially if seizures occur daily. The risk based on early age at seizure onset appeared independent of etiology and persisted within subgroups of patients with focal malformation of cortical development, tumor, or hippocampal sclerosis. Prospective studies will be important to clarify whether early surgical intervention may reduce the risk for subsequent MR in carefully selected infants.

Mental Retardation in Pediatric Candidates for Epilepsy Surgery: The Role of Early Seizure Onset

Summary Purpose: We sought to determine whether early age at seizure onset is a risk factor for mental retardation, independent of etiology. Assessment of risk for mental retardation with continued uncontrolled seizures plays a role in considerations of timing for epilepsy surgery. Previous studies have indicated that onset of seizures in the first years of life may be a risk factor for mental retardation, but the etiologies of the epilepsies were not included in the analyses. Methods: Intellectual function was assessed at ages 2–20 years during presurgical evaluation in 100 patients with intractable epilepsy due to focal lesions limited to part of one lobe of the brain. Mental retardation (MR) was defined as Full‐Scale Intelligence Quotient (FSIQ) ≤70. The age at seizure onset and the seizure frequency were obtained retrospectively. Results: Younger ages at seizure onset were associated with lower FSIQ scores, and mean FSIQ was also significantly lower for patients with onset of epilepsy at ≤24 months of age (74.0 ± 21.5) versus that in patients with onset of epilepsy later in life (87.8 ± 18.8: p = 0.005). The frequency of patients with MR was significantly higher for patients with seizure onset at ≤24 months of age (15 of 33, 46%) than for patients with seizure onset later in life (eight of 67, 12%; p &lt;0.001). This difference persisted within etiologic subgroups. For patients with focal malformation of cortical development, MR was seen in eight (50%) of 16 patients with seizure onset at ≤24 months versus two (10%) of 20 patients with seizure onset at &gt;24 months (p &gt; 0.001); for patients with tumor, MR was seen in four (50%) of eight patients with seizure onset at &gt;24 months versus four (13%) of 30 patients with seizure onset at &gt;24 months (p = 0.003); and for patients with hippocampal sclerosis, MR was seen in two (28%) of seven patients with seizure onset at ≤24 months versus none of 30 patients with seizure onset at &gt;24 months (NS). Within the subgroup with daily seizures. MR was present in 13 (65%) of 20 patients with seizure onset at &gt;24 months versus five (17%) of 29 patients with seizure onset later in life (p =0.001). Conclusions: These results indicate that onset of intractable epilepsy within the first 24 months of life is a significant risk factor for MR, especially if seizures occur daily. The risk based on early age at seizure onset appeared independent of etiology and persisted within subgroups of patients with focal malformation of cortical development, tumor, or hippocampal sclerosis. Prospective studies will be important to clarify whether early surgical intervention may reduce the risk for subsequent MR in carefully selected infants.

Borderline pediatric cochlear implant candidates: preoperative and postoperative results.

Borderline pediatric cochlear implant candidates: preoperative and postoperative results.

Catastrophic epilepsy in infants and children: Identification of surgical candidates

ABSTRACT Some pediatric candidates for epilepsy surgery have epilepsy which is considered catastrophic because of a high seizure frequency, often many per day, in association with developmental stagnation or regression. Increasing numbers of infants with catastrophic epilepsy are being considered for surgical treatment, if they have the key elements of surgical candidacy including medically intractable epilepsy, a localized epileptogenic zone, and a low risk of new, unacceptable, postoperative neurologic deficits. Most of these infants have had low grade tumor, cortical development malformations, or other epileptogenic lesions visualized on preoperative MRI. The assessment of potential risks and benefits for surgery in these patients involves complex, age‐related issues including the possible impact of uncontrolled seizures or surgery, on cognitive development. This review will address some of these issues in the context of two clinical cases.

New UNOS rules: historical background and implications for transplantation management

The algorithm for the allocation of donor hearts used by the United Network for Organ Sharing (UNOS) was changed in January 1999. The new scheme alters the medical urgency criteria from a 2-tiered to a 3-tiered system. Blood type O and blood type B candidates are less disadvantaged and pediatric candidates are somewhat advantaged with regard to adolescent donors. The new allocation algorithm allows an individual with life-threatening ventricular arrhythmias to be listed in the highest urgency status. Increased regulation will occur with the establishment of a review for the highest urgency status and the establishment of regional review boards.

028 Feasibility of the Wada test in paediatric candidates for epilepsy surgery

028 Feasibility of the Wada test in paediatric candidates for epilepsy surgery

Hearing results in pediatric patients after canal-wall-up and canal-wall-down mastoid surgery

Hearing results were studied in pediatric patients after canal-wall-up and canal-wall-down mastoid surgery in a private practice setting. A retrospective chart review was performed on 69 patients who underwent surgery from 1991-1995. Twenty-six patients underwent canal-wall-up mastoidectomies, and 43 patients underwent canal-wall-down mastoidectomies. Various types of ossicular reconstruction were performed in patients in both groups. Hearing was evaluated for preoperative and postoperative pure-tone averages, speech-reception thresholds, and air-bone gaps for the various types of ossicular reconstruction. Patients with canal-wall-up mastoidectomies and an intact ossicular chain had an average postoperative air-bone gap of 15 dB, whereas reconstruction of the ossicular chain (regardless of technique) resulted in an average postoperative air-bone gap of 19 dB. Patients with canal-wall-down mastoidectomies and an intact ossicular chain had an average air-bone gap of 15 dB, while all techniques of ossicular reconstruction had an average air-bone gap of 29 dB. Canal-wall-up and canal-wall-down mastoid surgeries in pediatric patients have similar hearing results. Variables other than hearing should be used to make treatment decisions regarding the canal wall in pediatric candidates for mastoid surgery. (Otolaryngol Head Neck Surg 1998;119:439-43.)