Radiosynthesis of 1-(2-[<sup>18</sup>F]Fluoroethyl)-L-Tryptophan using a One-pot, Two-step Protocol

The kynurenine pathway (KP) is a primary route for tryptophan metabolism. Evidence strongly suggests that metabolites of the KP play a vital role in tumor ...

Nemours/Alfred I. duPont Hospital for Children

As childhood obesity rates rise, laparoscopic adjustable gastric banding (LAGB) is being investigated as a bariatric surgical option in adolescents.

Pre- and postoperative imaging of adolescents undergoing laparoscopic adjustable gastric banding surgery.

To assess lower extremity bone mineral density (BMD) of children with congenital spinal dysfunction and examine factors that may influence BMD in this population.

Lower extremity bone mineral density in children with congenital spinal dysfunction.

Evaluating the bone health of children with disabilities is challenging and requires consideration of many factors in clinical decision-making. Feeding problems and growth deficits, immobility/inability to bear weight, effect of medications, and the nature of his or her disease can all directly affect a child's overall picture of bone health. Familiarity with the tools available to assess bone health is important for practitioners. The most commonly used method to assess bone density, dual energy x-ray absorptiometry, can be performed effectively when one appreciates the techniques that make scanning patients with disabilities possible. There are specific techniques that are especially useful for measuring bone density in children with disabilities; standard body sites are not always obtainable. Consideration of clinical condition and treatment must be considered when interpreting dual energy x-ray absorptiometry scans. Serial measurements have been shown to be effective in monitoring change in bone content and in providing information on which to base decisions regarding medical treatment. 

Assessment of bone health in children with disabilities.

Achondroplasia is the most common form of skeletal dysplasia. Although the radiographic features are well described, MRI features of the knee in achondroplasia have not been reported.

Magnetic resonance evaluation of the knee in children and adolescents with achondroplasia.

Some children with cerebral palsy (CP) have frequent fractures due to low bone mineral density and receive treatment with pamidronate, an intravenous bisphosphonate. Our review evaluates the outcome of pamidronate treatment in these children.

Pamidronate Treatment to Prevent Reoccurring Fractures in Children With Cerebral Palsy.

Mucopolysaccharidosis IV A (MPS IV A), Morquio A, is caused by deficiency in lysosomal enzyme N-acetylgalactosamine-6-sulfate sulfatase (GALNS), which is responsible for the catabolism of the glycosaminoglycans (GAGs) keratan sulfate (KS) and chondroitin 6-sulfate (C6S). Accumulation of GAGs results in disrupted cartilage formation and skeletal dysplasia. In this prospective cross-sectional study, bone mineral density (BMD) of the whole body (WB), lumbar spine (LS), and lateral distal femur (LDF) was acquired by dual-energy X-ray absorptiometry (DXA) on patients with MPS IV A. Functional abilities, medical history, Tanner score, and laboratory results were reviewed. Age and sex-matched norms were used to calculate Z-scores. Participants included 18 patients (13 females; 16 were unrelated) with a mean age of 21.4years (3.3 to 40.8years). While every patient was able to bear weight, 9 were full-time ambulators. Whole-body DXA could be obtained on only 6 patients (5 full-time ambulators) because of respiratory compromise caused by the position, presence of hardware, or positioning difficulties. Mean WB Z-score was -2.0 (range-0.3 to -4.1). Technical issues invalidating LS DXA in 8 patients included kyphosis at the thoracolumbar junction resulting in overlap of vertebrae in the posterior-anterior view. Mean LS BMD Z-score in full-time ambulators was -3.4 (range-1.6 to -5.0) and in the non-/partial ambulator was -4.0 (-3.7 to -4.2). Lateral distal femur BMD was acquired on every patient, and average Z-scores were -2 or less at all sites; full-time ambulators exhibited higher BMD. In conclusion, the LDF proved to be the most feasible site to measure in patients with MPS IV A. The higher LDF values in ambulators suggest this should be a consideration in promoting bone health for this group. 

Bone mineral density in MPS IV A (Morquio syndrome type A).

The aim of this study was to evaluate the activity of daily living (ADL) and surgical interventions in patients with mucopolysaccharidosis IVA (MPS IVA). The factor(s) that affect ADL are age, clinical phenotypes, surgical interventions, therapeutic effect, and body mass index. The ADL questionnaire comprises three domains: "Movement," "Movement with cognition," and "Cognition." Each domain has four subcategories rated on a 5-point scale based on the level of assistance. The questionnaire was collected from 145 healthy controls and 82 patients with MPS IVA. The patient cohort consisted of 63 severe and 17 attenuated phenotypes (2 were undefined); 4 patients treated with hematopoietic stem cell transplantation (HSCT), 33 patients treated with enzyme replacement therapy (ERT) for more than a year, and 45 untreated patients. MPS IVA patients show a decline in ADL scores after 10years of age. Patients with a severe phenotype have a lower ADL score than healthy control subjects, and lower scores than patients with an attenuated phenotype in domains of "Movement" and "Movement with cognition." Patients, who underwent HSCT and were followed up for over 10years, had higher ADL scores and fewer surgical interventions than untreated patients. ADL scores for ERT patients (2.5years follow-up on average) were similar with the-age-matched controls below 10years of age, but declined in older patients. Surgical frequency was higher for severe phenotypic patients than attenuated ones. Surgical frequency for patients treated with ERT was not decreased compared to untreated patients. In conclusion, we have shown the utility of the proposed ADL questionnaire and frequency of surgical interventions in patients with MPS IVA to evaluate the clinical severity and therapeutic efficacy compared with age-matched controls.

Activity of daily living for Morquio A syndrome.

To date, the only published reports of bone mineral density (BMD) in MPS IV involve patients with MPS IVA; no reports exist describing BMD for MPS IVB. In this prospective study of BMD in three patients with MPS IVB, BMD was acquired by dual-energy X-ray absorptiometry (DXA) at whole body (WB), lumbar spine (LS), and lateral distal femur (LDF). Functional abilities, ambulatory status, medical history, and height z-score were evaluated. Three patients with MPS IVB (two females), aged 17.7, 31.4 and 31.7 years, were evaluated. Every patient was ambulatory and one sustained two fractures caused by trauma. Whole body and hip DXA scans were technically invalid in every patient due to the presence of prosthetic hip hardware. Lumbar spine was valid in only 1 patient due skeletal abnormalities, and was normal (Z-score of - 0.8). The LDF was valid in every patient and was low at all three regions of interest: average LDF z-scores were - 3.1 (range, - 2.9 to - 3.6), - 2.3 (range, - 2.0 to - 2.5), and - 2.1 (range, - 2.0 to - 2.3) for region 1-region 3, respectively. Patients with MPS IVB have low BMD of the lower extremities even with full-time ambulation. Routine body sites to measure by DXA were problematic; hip and WB were invalid due to artifact, and LS had limited utility. The LDF was the only body site consistently available on all patients. Patients did not experience low-energy fractures despite low BMD.

Bone mineral density in mucopolysaccharidosis IVB.

The pelvis is composed of three paired primary ossification centers: the pubis, the ischium and the ilium. During maturation, multiple secondary ossification centers can be seen in the synchondroses between these bones and in the pelvic apophyses. The fragmented appearance of these centers can be confused with pathology.

Maturation of pelvic ossification centers on computed tomography in normal children.

The Small Parts Test Fixture (SPTF) (16 CFR 1501) was developed from cadavers of young children and foreign body (FB) data. Recent FB studies reveal that the SPTF misses outliers. Computerized tomography (CT) provides detailed dimensional data for young children. Our null hypothesis is that the SPTF (31.75 mm) is smaller than relevant portions of the aerodigestive tract.

Computerized Tomography Analysis of Young Children for Prevention of Aspiration Injuries.

Gunshot injuries are a leading cause of morbidity and mortality in the pediatric population. The Pediatric Trauma Society supports the use of tourniquets for exsanguinating hemorrhage in severe extremity trauma. The Combat Application Tourniquet (CAT) used with success in adults has not been prospectively tested in children. Our objective with this study was to determine if the CAT is successful in arresting extremity arterial blood flow in school-aged children.

Adult Tourniquet for Use in School-Age Emergencies.

Patients with Rett syndrome (RS) are at risk for low bone mineral density (BMD) and femoral fractures. In patients with RS, assessment with lateral distal femur (LDF) dual-energy X-ray absorptiometry (DXA) is recommended and clinically relevant. This study is the first to assess LDF BMD in girls with RS, and to compare LDF BMD results with lumbar spine BMD results in RS. Method Eleven girls (mean age 8.4 yr) with molecularly diagnosed RS and clinical DXA scan(s) were identified; medical charts were retrospectively reviewed. Baseline and serial lumbar spine and LDF BMD Z-scores were evaluated based on patients' ambulation status, presence of epilepsy, and mutation type. Results At the time of first scan, 8 of 11 patients had normal lumbar spine BMD and low LDF BMD Z-scores. Two patients had fracture history. Fully ambulatory (3) patients had higher lumbar spine and LDF BMD than partially (5) and nonambulatory (3) patients. Patients with epilepsy had lower average BMD at all sites. No difference was seen in lumbar spine or LDF BMD in patients with high-risk BMD mutations. Seven patients had serial DXA scans with an average observation of 5.1 yr (range 3.1 yr to 6.2 yr). Lumbar spine BMD over time was variable, while LDF bone mass accrual occurred at a lower rate than typically developing girls. Conclusion Females with RS exhibited lower BMD Z-scores at the LDF than at the lumbar spine. LDF and lumbar spine results were discordant. Ambulatory status and the presence of epilepsy were related to BMD. LDF BMD accrual deviated from normal as patients aged.

A Comparison of Lumbar Spine and Lateral Distal Femur Bone Density in Girls With Rett Syndrome.

Mucopolysaccharidosis type IVA (MPS IVA) is characterized by progressive skeletal dysplasia and respiratory issues with difficult airway management during anesthesia.

Heidi H. Kecskemethy

Diagnostic & Research PET/MRI Center,
Department of Radiology

Nemours/Alfred I. duPont Hospital for Children

Radiosynthesis of 1-(2-[¹⁸F]Fluoroethyl)-L-Tryptophan using a One-pot, Two-step Protocol

Heidi H. Kecskemethy

.css-o250i3{font-size:18px;font-family:Open Sans,sans-serif;line-height:21.6px;}Diagnostic & Research PET/MRI Center,Department of Radiology

Nemours/Alfred I. duPont Hospital for Children

Radiosynthesis of 1-(2-[18F]Fluoroethyl)-L-Tryptophan using a One-pot, Two-step Protocol

Diagnostic & Research PET/MRI Center,
Department of Radiology

Radiosynthesis of 1-(2-[¹⁸F]Fluoroethyl)-L-Tryptophan using a One-pot, Two-step Protocol