Children's Healthcare Of Atlanta Research Articles

Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation

Clinical experience suggests that craniopharyngiomas may temporarily increase in size after radiation therapy (RT). The study goal is to determine the incidence and natural history of this response in a cohort of patients managed at Children's Healthcare of Atlanta (CHOA) or Emory Healthcare (EHC). Between 08/1998 and 06/2009, 41 children and young adults were diagnosed with craniopharyngioma at CHOA and/or EHC. Of these, 21 received external-beam radiation and were included in our analysis. Serial magnetic resonance imaging (MRI) studies were evaluated volumetrically to assess response to RT. Median age at diagnosis was 8.2 years (range 3.2-23.5 years). Median radiation dose was 54.0 Gy using standard fractionation (1.8-2.0 Gy/day). With median follow-up of 41.3 months (range 7.2-121.8 months), actuarial local control and overall survival rates at 5 years were 78.7 % and 100 %, respectively. Of subjects, 52.4 % of subjects (11 of 21) were noted on serial MRI evaluation to have tumor enlargement (mostly cystic component) after radiation before eventual shrinkage without further intervention. For tumors that expanded, the median volume increase was 33.9 % (range 15.6-224.4 %). Median time to maximal tumor/cyst expansion was 1.5 months (range 1.0-5.0 months). Finally, nearly all patients (20 of 21) showed a measurable objective response to therapy by MRI regardless of ultimate disease control. Median time to maximal response post-radiation, as defined by MRI, was 9.5 months (range 3.5-39.9 months). In summary, RT is effective for managing craniopharyngioma. However, despite good ultimate responses, approximately 50 % of the patients show tumor/cyst expansion on MRI over the first few months post-radiation. Caution should be taken not to subject these patients to "salvage surgery" or cyst aspiration during this early time unless there are other overriding surgical indications. Understanding the natural history of this phenomenon could potentially help guide the management of these craniopharyngioma patients.

SickleREMOTE: A Two-Way Text Messaging System for Pediatric Sickle Cell Disease Patients.

Sickle cell disease, the most common hemoglobinopathy in the world, affects patient lives from early childhood. Effective care of sickle cell disease requires frequent medical monitoring, such as tracking the frequency, severity, and duration of painful events. Conventional monitoring includes paper- or web-based reporting diaries. These systems require that patients carry forms, which are easily lost, or laptop computers, which are impractical to scale to large populations. Both are prone to sporadic use by older adolescents due to lack of reminders. In this paper, we design and prototype a Sickle cell disease REporting and MOnitoring TElemedicine system (SickleREMOTE), aiming to resolve limitations of conventional monitoring diaries. This monitoring system is configured as automated short message service text (SMS-text) messages that arrive at a mobile phone anywhere on a cellular network. The messages may be reminders to encourage treatment adherence or questionnaires to collect self-assessed clinical data relating to treatment adjustments. Patients respond to the messages using pre-determined templates and a cloud database parses and stores messages automatically. Providers use a web-based interface to view, analyze, and download collected data. SickleREMOTE is developed by Georgia Institute of Technology in conjunction with Children's Healthcare of Atlanta (CHOA). System effectiveness will be evaluated using a trial of 30 adolescents with sickle cell disease and measured by response rate, time to response, error rate, and correspondence with data collected by telephone calls.

Role of FDG-PET in the Definition of Involved-Field Radiation Therapy and Management for Pediatric Hodgkin's Lymphoma

To evaluate positron emission tomography-computed tomography (PET-CT) influences in involved-field radiation therapy (IFRT) field design in pediatric Hodgkin's lymphoma (HL). From June 2003 to February 2008, 30 pediatric HL patients were treated at Children's Healthcare of Atlanta (CHOA) and Emory University Department of Radiation Oncology with both chemotherapy and IFRT. Diagnostic contrast-enhanced CT and PET-CT were coregistered using image fusion software. Both were reviewed for all potential sites of involvement and correlated to determine concordance and discordance. They were used in IFRT planning to determine the influence of PET-CT on target volumes and field design. There were 546 regions analyzed by both PET and CT modalities. Image sets were concordant in 468 regions and discordant in 78, yielding 86% concordance overall. Analysis by weighted κ statistic showed "intermediate to good" fit overall and for nodal sites, but "poor" agreement for extranodal sites. If discordant, a site was most likely PET+/CT-. Integration of PET information caused a change in staging in 15 (50%) patients, 7 upstaged and 8 downstaged. The IFRT volumes were adjusted on the basis of initial PET-CT finding in 21 (70%) patients, with 32 sites added and 15 excluded. There were four relapses, only one outside IFRT fields, but all were successfully salvaged. PET-CT represents an important tool in the management of pediatric patients with HL and has a substantial influence on both initial staging and radiation treatment target definition and field design.

Liver transplant in a four‐month‐old child with biliary atresia, unilateral pulmonary agenesis, and diaphragmatic hernia: First case report

Bilateral pulmonary agenesis (PA) is a rare embryological defect incompatible with life. Unilateral PA has a wide range of clinical presentations: its prognosis depends on the presence and severity of other associated anomalies. Fetal biliary atresia has been associated with a number of congenital anomalies, but the etiology is still not understood. An unusual case of a child with right PA, right diaphragmatic hernia, and delayed diagnosed biliary atresia leading to liver failure is presented herein. At the age of 4 months the patient was referred to the Transplant Department at Children Healthcare of Atlanta at Egleston with cholestasis and failure to thrive. With a rapidly progressive liver insufficiency, this child was evaluated for liver transplantation. In the absence of any respiratory symptom, the patient received a deceased donor size-matched left lateral segment liver transplant, which covered the diaphragmatic defect, with no further repair required. Twenty-seven months post-transplant, the patient has good graft function, a normal Z-score and is thriving. In spite of the increased physiological and surgical challenges (absence of right lung tissue, hemi-diaphragm, and ectopic position of the liver in the right chest), liver transplantation was performed with positive outcome in this high-risk child. Whether PA, may have developmentally contributed to expression of biliary atresia will need further investigation.

The challenges of developing a modern cardiac research program.

The development of a cardiac research program in the current health care setting can bring many challenges. The myriad of changing regulations involving research with vulnerable populations combined with the need to protect patient confidentiality is an issue that must be addressed in the initial stages of development. Also, the lack of an experienced workforce can often make the search for research staffing difficult at best. In 2001, Children's Healthcare of Atlanta initiated a cardiac research program. Over the last year, many obstacles have been overcome, and this experience has led to the appreciation of the challenges ahead.