006—18[F]-Flourodopa PET Scan to Localize Focal Lesions in Congenital Hyperinsulinism

006—18[F]-Flourodopa PET Scan to Localize Focal Lesions in Congenital Hyperinsulinism

216 ABSTRACTS OF THE PEDIATRIC ENDOCRINOLOGY NURSING SOCIETY CONVENTION Methods: The cross-sectional correlational design included 41 mothers of chi...

78KB Sizes 2 Downloads 65 Views

216

ABSTRACTS OF THE PEDIATRIC ENDOCRINOLOGY NURSING SOCIETY CONVENTION

Methods: The cross-sectional correlational design included 41 mothers of children ages 6–10 at three outpatient clinics in the Midwest. The research examined the following variables: (a) maternal coping resources, (b) child behavior, (c) maternal diabetes self-efficacy, (d) maternal diabetes management behavior, and (e) child glycemic control. The corresponding scales were as follows: (a) Coping Resources Inventory; (b) Behavioral Assessment System for Children–Parent Report; (c) Maternal Self-Efficacy for Diabetes Scale; (d) Diabetes Management Scale-Parent, 24-Hour Diabetes Behavior Recall, and downloaded glucose data; and (e) child HgbA1c. Results: Results indicated that only coping resources contributed significantly to mothers’ diabetes self-efficacy, R 2 = .225, adjusted R 2 = .205; F(1, 38) = 11.059, p b .002. No significant relationship was found between the mothers’ environment and diabetes management behavior. This finding, however, is important and suggests that mothers were able to follow through with diabetes management regardless of how their child was behaving. Selfefficacy did not predict maternal diabetes management behaviors. Significant correlations were found between two measures of maternal diabetes management behaviors and child metabolic control. The number of blood glucose tests performed in the past month was negatively correlated to metabolic control, r(27) = .71, p b .01. Specifically, a child with more blood glucose tests had a lower HgbA1c.The maternal 24-hour recall of diabetes behaviors was positively correlated to metabolic control, r(41) = .39, p b .05. Conclusions: The results of this study confirmed that mothers with consistent diabetes management behaviors have children with better metabolic control. Clinical Implications: Nurses need to understand the factors that influence the diabetes management behaviors of a mother to assist mothers in improving the child’s metabolic control.

004—Prevalence of Microalbuminuria in a Population of Children With Type 1 Diabetes. Kathy Montgomery, Steve Willi, Wei He, Kathryn Murphy, Ravi Bamba, Diabetes Center for Children, The Children’s Hospital of Philadelphia, Philadelphia, PA Background: Nephropathy is a significant long-term complication of diabetes. The earliest evidence of nephropathy is the appearance of microalbuminuria (MA). The American Diabetes Association recommends screening for MA annually, beginning after 5 years’ disease duration and/or puberty. Aim: The purposes of this study were to evaluate the prevalence of MA in a population of children with type 1 diabetes and to identify characteristics of children with MA related to age, gender, race, and duration of diabetes. Methods: Our clinical practice guideline recommends that all children with type 1 diabetes diagnosed N1 year be screened annually for MA using a random spot urine specimen for microalbumin:creatinine ratio. Further follow-up studies weed out false-positives. Data were collected on all children with type 1 diabetes who had a documented screening test result for MA during a 13-month period. Laboratory values, age, duration of diabetes, gender, race, and results of subsequent tests were reviewed. Results: One thousand four hundred seventy-five children were eligible to be screened from 10/01/05 to 10/31/06. Of these patients, 835 (57%; females, 390; males, 445) had a documented screening test result. Mean age of screened children was 13.2 years. Mean duration of diabetes was 5.4 years. Race was distributed as follows: Caucasian, 657; African American, 111; Hispanic, 19; Other, 48. Of the 835 children screened, 112 (13.4%; females, 72; males, 40) had an elevated result (N30 Ag/mg). Mean age of these children was 12.9 years, and mean duration of diabetes was 5.2 years. Racial distributions were proportionately similar.

Conclusions: Of the children eligible for screening, only 57% were screened. More female patients had positive random spot MA screening. There were no differences in age, duration of diabetes, or race. Surprisingly, mean duration of diabetes was similar between the positive and negative groups. Clinical Implications: Random spot urine testing identified MA in 16% of our screened population. It is unclear if this includes a group of patients with false-positive results. In view of the early average age of children with elevated MA, consideration should be given to initiating screening for MA at diagnosis of diabetes.

005—Mothers and Youths Coping After Insulin Pump Therapy was Initiated. Audrey E. Nelson, Earline Edwards, Sangeeta Agrawal, University of Nebraska Medical Center, Omaha, NE Background: Type 1 diabetes treatment includes multiple demands such as administration of insulin, monitoring blood glucose levels, adjusting foods eaten, and participating in planned exercises. Each day, mothers and youths with type 1 diabetes must deal with these demands. No studies were found to examine how both mothers and their children responded to treatment demands after the insulin pump therapy was initiated. Aim/Methods: This longitudinal study was designed to describe how mothers and their children coped with insulin pump therapy and related treatment activities. Twenty nine of 49 potential subject pairs (59% mothers and youths, 10–18 years old) agreed to participate and signed approved consent/assent forms prior to completing reliable questionnaires at 1 (T1), 4 (T2), 8 (T3), and 12 (T4) months after insulin pump therapy was initiated. Results: Over time, 77–90% of mothers reported being a little bothered or not bothered by the treatment activities, and 31–59% found the activities somewhat or not hard to perform. Also, 69–89% of youths reported little or no difficulty with the treatment, and 85–95% reported having diabetes or performing diabetes treatment activities was a little or not upsetting. Nine mother–youth pairs participated at each data collection time. Having difficulty with treatment activities was reported by two pairs at T1, by one pair at T2, by one pair at T3, by one pair at both T1 and T4, and by one pair at each time. Being upset by treatment activities was reported by two pairs at T1. Being upset and having difficulty with treatment activities was reported by two pairs at T1 and by one pair at T4. Conclusion: Most of these mothers and youths appear to cope with the treatment demands in a positive perspective. There were some mothers and youths who did report having difficulty with performing treatment activities and/or that treatment activities were upsetting during the first year of insulin pump therapy. A 69% subject attrition rate over time may have reduced the percentage of mothers and youths who had less positive coping. Implications: Health care professionals need to discuss with each youth and mother how treatment activities are affecting them and how to modify treatment protocol to reduce the perceived negative demands.

006—18[F]-Flourodopa PET Scan to Localize Focal Lesions in Congenital Hyperinsulinism. Susan Becker O’Rourke, Olga T. Hardy, N. Scott Adzick, Abass Alavi Charles A. Stanley, The Congenital Hyperinsulinism Center, Division of Endocrinology, The Children’s Hospital of Philadelphia, Philadelphia, PA, Hospital of the University of Pennsylvania, Division of Nuclear Medicine, Department of Radiology, Philadelphia, PA Background: As presented at the Pediatric Endocrinology Nursing Society in 2005, preliminary studies in seven infants showed that

ABSTRACTS OF THE PEDIATRIC ENDOCRINOLOGY NURSING SOCIETY CONVENTION

[18F]-fluorodopa (F-DOPA) with positron emission tomography (PET) could be useful in imaging focal lesions. This is a follow-up report that summarizes our results with F-DOPA PET scans in a large group of children with congenital hyperinsulinism (HI). Aims: The aims of the project were to determine the sensitivity and specificity of PET scanning with F-DOPA to detect a focal HI lesion preoperatively and to locate residual disease in children who remain hypoglycemic after partial pancreatectomy. Methods: Patients underwent an F-DOPA PET scan 24 or more hours preoperatively under general anesthesia at the PET facility at a collaborating hospital. Interpretation of the scan is compared with the postoperative histology to validate accuracy of the images. Results: To date, 50 patients who were referred surgery for medically uncontrolled hypoglycemia have been studied. Of these, 46 had surgery and are included in the data analysis; 48% had focal lesions, and 52% were diffuse on histopathological interpretation. The PET scan was incorrect only in three focal cases (i.e., images were interpreted as diffuse, but on surgery, they were found to have a focal lesion). Unexpectedly, one patient who had two previous pancreatectomies was found to have ectopic lesions of pancreatic tissue in the bowel wall. Conclusions: F-DOPA PET scan is a highly accurate imaging procedure for focal HI with a sensitivity of 86% and a specificity of 100%. It is capable of both distinguishing focal adenomatosis from diffuse HI and localizing focal lesions. It is a less invasive method than the previously available tests. Clinical Implications: F-DOPA PET scan is conducted under a research investigational new drug application for the radiopharmaceutical agent and, therefore, is not available at most institutions. This protocol could not be accomplished without nursing coordination of the large multidisciplinary team. Our nursing team partners with staff RNs to educate members of the multidisciplinary team on the purposes and benefits of PET. Although this innovative technology is valuable, it is not appropriate for all patients, and nurses on the team are in a position to educate referring providers, as well as parents, on this test’s applicability in individual cases.

Case Presentations 007–Accurate Diagnosis of Glycogen Storage Disease in a Child Presenting With Hepatomegaly and Hypoglycemia. Amanda J. Beattie, Lori P. Halaby, Charles A. Stanley, Division of Endocrinology, The Children’s Hospital of Philadelphia, Philadelphia, PA Patient Demographics: J.L. is a 23-month-old Hispanic male. Clinical Presentation: J.L. presented to our clinic with hepatomegaly, fasting hypoglycemia, and suspected glycogen storage disease (GSD) of an unknown type. Past History: During hospitalization for a respiratory infection at 3 months, J.L. was noted to have hepatomegaly, increased liver function tests, and hypoglycemia after fasting 4–5 hours. He underwent two liver biopsies, which showed significant glycogen storage without evidence of fibrosis. These results were inconclusive for the type of GSD. No further testing was performed. His parents were advised to limit fasting to 3–4 hours. Evaluation: J.L. presented to our clinic at 22 months. His fasting tolerance had improved but remained abbreviated with glucose levels in the 50s after fasting 10–12 hours overnight. He had significant hepatomegaly. Length was from 5th to 10th percentile, demonstrating a marked decline in growth velocity compared to

217

previous records. Inpatient evaluation for clinical testing to define the type of GSD included an oral glucose tolerance test, which revealed a rise in lactate from 1.0 to 6.6 mmol/L, whereas the blood glucose rose from 54 to 103 mg/dl. A fed glucagon stimulation test showed no rise in glucose or lactate. After 6 hours of fasting, J.L.’s glucose level dropped to 58 mg/dl with a rise in ketones to 3.4 mmol/L and no elevation of lactate. These tests excluded Type I GSD and suggested Type III or Type VI/IX, with Type III more likely based on the short fasting tolerance. Blood was sent for genetic mutation analysis for Type III. Interventions: J.L. was started on a regimen of cornstarch (1 g/kg) twice overnight to maintain blood sugars. Parents were instructed to limit his fasting interval to b4 hours during the day and give frequent, low-glycemic index feeds. Discussion/Recommendations: Although GSD commonly presents with hepatomegaly, liver biopsy cannot be exclusively relied upon for accurate diagnosis. Fasting and stimulation testing should be performed initially to determine the probable biochemical pathway, helping to define the most likely type of GSD. If inconclusive, clinical testing results can guide the use of biopsy specimens for definitive diagnosis. Type-specific treatment can then be prescribed to maintain blood sugars and minimize metabolic abnormalities, which will maximize growth potential and decrease the risk for long-term sequelae.

008–Slipped Capital Femoral Epiphysis in a Patient on GnRH-Agonist Therapy. Jan M. Foote, Blank Children’s Hospital, Des Moines, IA Patient Demographics: A 10.7-year-old Caucasian female on GnRH-agonist (GnRHa) therapy developed slipped capital femoral epiphysis (SCFE). Clinical Presentation: The patient reported a 2-month history of progressive left hip pain and limping. She denied any fever, redness, or injury. She had discomfort and limited motion upon internal rotation of her left hip. Complete blood count and C-reactive protein were normal. Past History: She had growth acceleration and signs of gonadarche before 8 years of age. She had Tanner Stage IV breast development and Tanner Stage II pubic hair. Her evaluation was consistent with central precocious puberty. Prior to treatment, her bone age (BA) was 10.5 years, as compared to her chronological age (CA) of 8.6 years. She started treatment with Lupron Depot-Ped, 15 mg, im, every 28 days at a CA of 8.7 years. At that time, height standard deviation score (SDS) was +3.4, growth velocity was 9.3 cm/year, and body mass index (BMI) was 24.3 kg/m2 (N97th percentile). Her ?BA:?CA ratio was 2.8 years/year prior to treatment compared with 0.8 years/year during treatment, and she had no further progression of breast development. Evaluation: Radiographs showed unilateral Grade I SCFE. She underwent percutaneous internal fixation of her left hip. She was seen in the pediatric endocrinology clinic 4 weeks later. At that time, height SDS was +3.6, BA was 12.8 years, and BMI was 28.1 kg/m2. Growth velocity was 7.0 cm/year in the last year of treatment. Laboratory studies showed suppressed luteinizing hormone, folliclestimulating hormone, and estradiol. Thyroid function and insulinlike growth factor-1 were normal. Interventions: Since she attained her target height, GnRHa therapy was discontinued. Because of the risk for bilateral SCFE, we discussed the importance of reporting any symptoms in the other leg. Counseling regarding weight management was continued. Discussion: In the literature, we found reports of six patients who developed SCFE during treatment with other GnRH agonists or shortly after discontinuing treatment. Our patient had risk factors for SCFE, including tall stature, increased growth velocity, and obesity, making Lupron Depot-Ped therapy less likely as a cause of SCFE.