Adam Steinmetz

Differentiated Thyroid Carcinoma in Pediatric Patients: Comparison of Presentation and Course between Pre-pubertal Children and Adolescents

OBJECTIVE To evaluate the clinical characteristics, course, and outcome of differentiated thyroid carcinoma (DTC) in pre-pubertal children compared with adolescents. STUDY DESIGN The records of 10 pre-pubertal and 17 pubertal patients in whom DTC was diagnosed and who were observed in our tertiary pediatric endocrine clinic were reviewed. Extension of tumor at presentation, treatment modality, course, and outcome were analyzed. RESULTS A positive family history of DTC was more prevalent in the pre-pubertal group (P = .037). At diagnosis, they had a greater degree of extrathyroid extension (P = .012), lymph node involvement (P = .009), and lung metastases (P = .009). The extent of surgery was similar in both groups, whereas the weight-adjusted radioiodine (I(131)) ablative dose was higher in the pre-pubertal group (P = .004). During the median follow-up of 5 years, the overall survival rate was 100% for both groups, with no significant difference in evidence of residual tumor after initial therapy or the recurrence rate. CONCLUSION DTC has a more aggressive presentation in pre-pubertal children. Rigorous initial surgical and I(131) treatment, followed by thyrotropin suppression, was found to result in an outcome similar to that achieved in the pubertal group.

Benign Hereditary Chorea: Clinical, Neuroimaging, and Genetic Findings

Benign hereditary chorea is an autosomal dominant disease with an early onset of symptoms. In some families, symptoms tend to decrease in adulthood, suggesting that the disorder results from a developmental disturbance in the brain. Individuals with benign hereditary chorea, a nonprogressive disease, have normal or slightly below normal intelligence. The locus for benign hereditary chorea is on chromosome 14. Benign hereditary chorea is a result of mutations in the thyroid transcription factor 1 gene. Previous neuroimaging and pathological investigations of the brain showed no notable abnormalities in patients with this condition. In this study, 5 patients from 1 family with typical clinical features of benign hereditary chorea are presented. Clinical severity varied considerably in the family. Brain magnetic resonance imaging results were normal. Brain single photon emission computed tomography in 3 children, performed 1 hour after intravenous injection of 0.35 mCi/kg of body weight of technetium 99m ethyl cysteinate dimer, showed markedly decreased uptake in the right striatum and the right thalamus in 1 child. The oldest child had mildly reduced uptake in the right putamen and the left thalamus. Brain single photon emission computed tomographic findings in the youngest child were normal. Contrary to other reports of radionuclide brain imaging, notable brain single photon emission computed tomography changes were detected in 2 of 5 patients. Brain single photon emission computed tomography findings did not seem to correlate with the clinical status of the children.

FDG PET/CT Early Dynamic Blood Flow and Late Standardized Uptake Value Determination in Hepatocellular Carcinoma

PURPOSE To prospectively determine whether fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) early dynamic blood flow estimates could be used to discriminate hepatocellular carcinoma (HCC) from background liver and to characterize HCC in patients with and those without angioinvasion; and to evaluate the association between blood flow measures at FDG PET/CT with metabolism in HCCs. MATERIALS AND METHODS Institutional review board approval and written informed consent were obtained for this prospective study. Twenty-one consecutive patients (mean age, 65 years) with 30 established HCCs (mean size, 5.5 cm; seven lesions in five patients with angioinvasion) underwent a blood flow study with an FDG dynamic scan divided into 18 sequences of 5 seconds each and a standard PET/CT scan. On the dynamic study, three independent operators obtained volumes of interest (VOIs) for which three blood flow estimates were calculated (hepatic perfusion index [HPI], time to peak [TTP], and peak intensity [PI]). On the late study, a VOI was placed on the fused scan for each HCC, and maximum standardized uptake value (SUV(max)) was obtained. By using a mixed-effects model analysis, comparison of blood flow estimates between HCC with and that without angioinvasion and background liver was performed. The association between blood flow estimates and SUV(max) was also assessed. RESULTS HPI and TTP showed better performance than did SUV(max) for discriminating HCC and background liver (areas under receiver operating characteristic curve: 0.96, 0.95, and 0.83, respectively; P < .05). HPI was higher in HCC in patients with angioinvasion (0.91 ± 0.15 [standard deviation]) than in those without angioinvasion (0.80 ± 0.18; P = .03). There was no difference in SUV(max) between HCC in patients with and those without angioinvasion (7.8 ± 2.9 vs 6.3 ± 3.4; P = .85). No clear association was found between HPI, PI, or TTP and SUV(max) (P = .49, .77, and .91, respectively). CONCLUSION Early dynamic blood flow FDG PET/CT may be used to help discriminate and characterize HCC tumors.

Unilateral lower limb rest tremor is not necessarily a presenting symptom of Parkinson's disease†

Lower leg rest tremor is an uncommon symptom of neurological disease. Review of the files of 16 patients who presented with lower leg tremor (average age 58 ± 16 years; average disease duration 6.8 ± 8.5 years) yielded a diagnosis of Parkinsons disease (PD) in 5 and probable multiple system atrophy (MSA) in 3. In 4 patients with an indeterminate diagnosis, cardiac MIBG SPECT was positive in 3, indicating PD, and negative in one, suggesting MSA. Two patients each had psychogenic tremor and drug‐induced parkinsonism. Although lower leg tremor is considered an unusual presentation of PD, it should raise suspicions of MSA and other neurodegenerative disorders.

Pediatric Thyroid Cancer: Postoperative Classifications and Response to Initial Therapy as Prognostic Factors

CONTEXT Prognostic factors for pediatric differentiated thyroid cancer (DTC) are not well established. OBJECTIVE The objective of the study was to retrospectively compare the postoperative risk-stratification systems: American Thyroid Association (ATA) risk categories, Schneider Childrens Medical Center of Israel (SCMCI) score, and the response to initial therapy as predictors for disease outcome. PATIENTS AND METHODS Fifty-four DTC patients, median age at diagnosis 13.9 years (range 1.9-17 y), followed up for a median of 8.8 years (range 2.6-20.5 y) were stratified into prepubertal (n = 9), pubertal (n = 25), and postpubertal (n = 20) groups. All patients underwent total/near-total thyroidectomy; 48 received radioiodine therapy. The extent of DTC was evaluated by applying the ATA risk categories and the novel SCMCI score. Postoperative risk stratifications (low/intermediate/high) were determined using histopathological, laboratory, and imaging findings. Response to initial therapy (complete/acceptable/incomplete) was based on stimulated thyroglobulin and imaging results during the first 2 years of follow-up. RESULTS The risk for recurrent/persistent disease, as assessed by the postoperative ATA risk-stratification system and the SCMCI score and by the response to initial therapy, was higher in the prepubertal group (P < .001, P = .002, and P = .02, respectively). Outcome prediction by the risk-stratification systems was applicable: ATA risk categories, P = .014, R(2) = 0.247, predictive ability 80.4%; SCMCI score, P < .001, R(2) = 0.435, predictive ability 86.3%; and response to initial therapy stratification, P < .001, R(2) = 0.789, predictive ability 96.1%. The proportion of variance explained by the ATA risk categories (0.25), SCMCI score (0.44), and response to initial therapy (0.79) indicated that the latter was the most precise predictor and that the SCMCI score reflected the disease outcome better than ATA risk categories. CONCLUSIONS Our data confirm that the postoperative pediatric ATA stratification system and the novel SCMCI score are suitable for predicting the risk of recurrent/persistent disease in this population. The response to initial therapy classification performed 1-2 years after the initial therapy may be more appropriate for guiding surveillance recommendations.

Comparison of continuous breath test and gastric scintigraphy for the measurement of gastric emptying rate in healthy and dyspeptic individuals.

Objective To determine normative gastric emptying rates for the continuous breath test and to compare its findings with gastric scintigraphy in healthy volunteers and dyspeptic patients. Methods A standard 250 kcal meal double-labeled with 1 mCi 99mTc colloid and 100 &mgr;g nonradioactive 13C-octanoic acid was administered to 20 healthy individuals and 22 dyspeptic patients attending a tertiary medical center in 2009–2010. Gastric emptying rate was measured simultaneously with sequential gastric scintigraphy and the continuous breath test. The results of the healthy controls were used as a normative reference. The findings of the two tests were analyzed by linear regression and &kgr; statistics. In addition, the gastric half-emptying times (T ½) were compared by a &kgr; test for evaluating the agreement of normal/abnormal results in both methods. Results Background features were as follows: healthy individuals – 15 men/5 women, mean age 44.9±14 years and mean BMI 26.8±3.5; dyspeptic patients – 5 men/17 women, mean age 58.0±13 years and mean BMI 25.3±5.5. The upper limit of normal for gastric half-emptying time (T ½) was 140 min by the breath test. The linear correlation of T ½ between the methods was 0.64. The agreement of normal/abnormal T ½ between the methods was 0.65. Conclusion The continuous breath test may be a suitable method for the evaluation of gastric emptying. A larger study is required to determine its feasibility as the standard of care in dyspeptic patients.

A novel continuous breath test versus scintigraphy for gastric emptying rate measurement.

Background Gastric scintigraphy (GS) is considered the gold standard for gastric emptying rate (GER) measurement; however, it requires expensive equipment and special licensing for radioactive substances. Aim To compare a new nonradioactive, real-time, continuous breath test (CBT) method with GS for the GER measurement. Methods Simultaneous GER analysis by both GS and CBT was carried out on 8 dyspeptic patients and 6 healthy controls. After a 14 hour fast, participants ate a standard meal of 250 Kcal double labeled with 1 mCi of Tc-99m and 100 &mgr;g of C-13 labeled octanoic acid. The participants underwent simultaneous GS for 120 minutes on single-detector &ggr; camera and CBT carried out by attaching the nasal cannula of the system (Oridion, BreathID, Israel), which automatically and continuously collected and analyzed breath samples with real time display. A linear fit model was used to calculate gastric empting half-time. A half-time of more than 100 minutes for GS and 80 minutes for CBT were considered pathologic. The GS and CBT were compared by &kgr; test of agreement in normal/abnormal results. Results Good correlation was found for GER measurements between GS and the CBT methods with a linear correlation coefficient of R=0.74. The &kgr; test indicated excellent agreement with value of 0.86 for the qualitative determination of pathologic and normal results. Conclusions The novel CBT provides reliable and reasonably accurate data for on-line GER estimate, in a simple manner suitable for medical clinics or bedside setting, without the use of radioactive substances.

Vomiting and Dysphagia Predict Delayed Gastric Emptying in Diabetic and Nondiabetic Subjects

Background. Gastroparesis is a heterogeneous disorder most often idiopathic, diabetic, or postsurgical in nature. The demographic and clinical predictors of gastroparesis in Israeli patients are poorly defined. Methods. During the study period we identified all adult patients who were referred to gastric emptying scintigraphy (GES) for the evaluation of dyspeptic symptoms. Of those, 193 patients who were referred to GES from our institution were retrospectively identified (76 (39%) males, mean age 60.2 ± 15.6 years). Subjects were grouped according to gastric half-emptying times (gastric T 1/2). Demographic and clinical data were extracted from electronic medical records or by a phone interview. Key Results. Gastric emptying half-times were normal (gastric T 1/2 0–99 min) in 101 patients, abnormal (gastric T 1/2 100–299 min) in 67 patients, and grossly abnormal (gastric T 1/2 ≥ 300 min) in 25 patients. Vomiting and dysphagia, but neither early satiety nor bloating, correlated with delayed gastric emptying. Diabetes was associated with grossly abnormal gastric T 1/2. Idiopathic gastroparesis was associated with a younger age at GES. No correlation was observed between gastric T 1/2 values and gender, smoking, H. pylori infection, HBA1C, or microvascular complication of diabetes. Conclusions Inferences. Vomiting and dysphagia are predictive of delayed gastric emptying in both diabetic and nondiabetic subjects. Diabetes is associated with more severe gastroparesis.

Patterns of papillary muscle ischemia in myocardial PET

Purpose: Conventional nuclear medicine equipment lacks sufficient spatial resolution to reliably visualize the papillary muscles (PM). Positron emission tomography (PET), however, can adequately visualize these structures using various positron emitters. Methods and Patients: We present various patterns of PM observed on myocardial PET imaging in 4 patients. These patterns demonstrate different pathologic conditions such as PM ischemia of varying severity, as well as hibernation, using both N-13 NH3 and F-18 FDG as perfusion and metabolic agents, respectively. These patterns of infarction, stress-induced myocardial ischemia, or hibernation can be identified in one or both PM using PET scanning. Normal PM visualization on chest F-18 FDG PET images is also presented. Conclusion: This report illustrates the potential ability of myocardial PET as a noninvasive modality to study the perfusion and metabolic abnormalities of the PM.

Urinary diarrhea detected by Tc-99m DTPA scintigraphy in a 3-year-old girl.

The authors describe a 3-year-old girl who was hospitalized for diarrhea and electrolyte imbalance. Urinary obstruction was considered because of an elevated serum creatinine level and a history of multiple surgical interventions. Renal scintigraphy revealed a urinary leak from the right urinary system to the right colon as a result of an unexpected ureteroenteric fistula. This uncommon scintigraphic finding led to a correct diagnosis and appropriate treatment for this patient.