David L. Gilday

Neurologic manifestations of pediatric systemic lupus erythematosus.

Central nervous system involvement is a common but rarely reviewed feature of pediatric systemic lupus erythematosus (SLE). We retrospectively reviewed the charts of 91 patients with pediatric SLE and using a standardized data abstraction form documented 40 patients with central nervous system (CNS-SLE) involvement. The mean age of onset of SLE was 13.3 years. In 19 patients the CNS manifestation was a presenting symptom, in 12 patients CNS involvement was present within the first year of diagnosis, and in 9 patients it took up to 7 years for CNS disease to become evident. Nineteen children (48%) manifested neuropsychiatric SLE, which included depression, concentration or memory problems, and frank psychosis. Seizures were present in 8 patients (20%), 6 had cerebral ischemic events (15%), 1 had chorea (3%), 2 had papilledema (5%), and 2 patients had a peripheral neuropathy (5%). Nine patients (22%) had severe headache consistent with lupus headache. Seven children had more than one CNS manifestation. In the investigation of CNS-SLE, computed tomography and/or magnetic resonance imaging scans were helpful in patients with focal ischemic lesions and venous sinus thrombosis. Electroencephalography was abnormal only in 33% of patients with seizure disorders and rarely helpful in patients with diffuse neuropsychiatric symptoms. Single-photon emission computed tomography scans were abnormal in most patients with neuropsychiatric SLE, especially in those with frank psychosis. The lupus anticoagulant was present in the patient with chorea and was frequently present in patients with cerebral vascular events. Long-term outcome was good: only 1 child died of cerebral hemorrhagic infarction and 3 others had significant persistent CNS deficits. The majority of patients (90%) had excellent recovery from CNS-SLE.

Initial experience with FSE STIR whole-body MR imaging for staging lymphoma in children

Our objective was to compare fast spin-echo (FSE) short inversion time inversion recovery (STIR) whole-body MR imaging with standard procedures in staging children with lymphoma. Eight children (age range, 2–16 years) underwent multi-station FSE STIR whole-body MR at initial staging (n=5) or for restaging following completion of therapy (n=5). Whole-body MR and conventional staging procedures, including CT (n=10), gallium-67 scintigraphy (n=9), bone scintigraphy (n=3) and bone marrow biopsy (n=7) were retrospectively compared for detection of sites involved by lymphoma and for the assigned stage. FSE STIR whole-body MR detected more sites of possible lymphomatous involvement at initial staging (87/88) and at restaging (5/5) than did conventional imaging (74/88, 3/5). MR was more sensitive than conventional imaging in detecting bone marrow involvement at initial staging. Following treatment, however, residual and therapy-induced bone marrow signal abnormalities could not be differentiated from lymphomatous involvement. Detection of nodal and visceral involvement correlated well. Our results suggest that FSE STIR whole-body MR imaging is a sensitive technique for evaluating lymphomatous involvement of bone marrow as well as non-marrow sites. Larger prospective trials are needed to determine if FSE STIR whole-body MR can replace standard radiographic procedures for initial staging and contribute in the follow-up of lymphoma in children.

Noninvasive measurement of blood flow and extraction fraction

We describe the theory of a technique for the noninvasive measurement of organ blood flow which is based on the principle of fractionation of cardiac output and is applicable with any recirculating gamma emitting tracer. The technique effectively determines the count rate that would be recorded over the organ if the tracer behaved like radiolabelled microspheres and was completely trapped in the organs vascular bed on first pass. After correction for organ depth, the estimated first pass activity plateau, expressed as a fraction of the injected dose, is equal to the organs fraction of the cardiac output (CO). By extending the theory, organ extraction fraction of extractable tracers or mean transit time of nonextractable tracers can be measured. Renal blood flow (RBF) to the normal left kidney in 18 subjects without evidence of renal disease was estimated by the technique to be 10.4% CO (S.D. 1.2), for the right kidney 9.0% CO(S.D. 1.1), and for both, 19.4% CO (S.D. 1.5). In a separate series of eight patients, RBF values were highly reproducible when obtained on successive days (standard deviation of change: 0.67% CO for the left kidney, 0.63% CO for the right and 0.78% CO for both). The extraction fraction of DTPA (filtration fraction) was 8.5% (1.7) in the left kidney and 9.7% (2.1) in the right kidney. This parameter was less reproducible than RBF, with standard deviations of the changes equal to 1.17% for the left kidney and 1.31% for the right. Stable, well-functioning, renal allografts in children had a mean blood flow of 20.6% CO (S.D. 3.7). Transplant blood flow in eight patients with rejection was less than 5.2% CO. In patients without splenomegaly, splenic blood flow, splenic extraction fraction of sulphur colloid and splenic red cell mean transit time were 4.3% CO (S.D. 0.9), 41% (S.D. 13.8), and 36.9 s (S.D. 4.6), respectively.

Assessment of myocardial perfusion and function in childhood systemic lupus erythematosus

OBJECTIVES To determine the prevalence of abnormalities in myocardial perfusion or function in children with systemic lupus erythematosus (SLE), and describe potential factors that may predict their development. STUDY DESIGN Patients (n = 40; 30 female) were enrolled through the Lupus Clinic at The Hospital for Sick Children between 1990 and 1992. Resting and exercise thallium myocardial perfusion scans, radionuclide angiography with multiple gated acquisition (MUGA), and resting M-mode and two-dimensional echocardiography were performed. RESULTS All patients were free of symptoms, and none had a history of ischemic heart disease. Their median age was 15.9 years (range 10.5 to 19.8 years) at enrollment. Abnormalities of coronary perfusion were found in 5 (16%) of 31 patients (95% confidence interval: 3%, 29%) and included a large fixed perfusion defect in 1; 5 of 27 MUGA scans showed marginally low left ventricular ejection fractions at rest, whereas all had normal exercise responses. In the group with abnormal thallium scans, three of five patients had antiphospholipid antibodies detected, and two of four had an abnormal plasma lipid profile. This group tended to have a shorter disease duration and had received a lower cumulative dose of corticosteroids; these differences were not statistically significant compared with the group with normal scans. CONCLUSION Asymptomatic abnormalities of myocardial perfusion occur in children with SLE and are more common than previously suspected. Patients with these abnormalities of myocardial perfusion may be predisposed to the previously recognized early-onset ischemic heart disease seen in adults with SLE.

Decrease in gastric emptying time and episodes of regurgitation in children with spastic quadriplegia fed a whey-based formula

The gastric emptying times associated with three whey-based formulas were significantly shorter than that associated with a casein-based formula in nine gastrostomy-fed patients with spastic quadriplegia (p less than 0.001). Patients fed whey-based formulas had significantly fewer episodes of emesis than when they were fed casein-based formula (p less than 0.001). We conclude that whey-based formulas reduce the frequency of emesis by improving the rate of gastric emptying.

Focal cortical dysplasia in children with localization-related epilepsy: EEG, MRI, and SPECT findings

A retrospective analysis was conducted of 9 children with focal cortical dysplasia and localization-related epilepsy who underwent epilepsy surgery. Focal cortical dysplasia includes malformed lesions with extensive abnormalities of neuronal morphology, architecture, and lamination. The patients were examined by EEG and video EEG telemetry, CT, MRI, and SPECT using 99mTc-HmPAO. EEG disclosed interictal localized epileptiform activity in 8 patients and nonepileptiform activity with slow waves in 1. Ictal EEG telemetry demonstrated a predominantly localized seizure onset in 8 patients and MRI demonstrated an abnormal loss of gray and white matter distinction in 6. Decreased regional cerebral blood flow (rCBF) was detected in 4 patients by interictal SPECT, and increased rCBF in the same epileptogenic focus in 2 by postictal SPECT. Pathologic analysis found focal cortical dysplasia in 8 patients. One had extensive focal polymicrogyria, pachygyria, and extensive white matter heterotopias. It is concluded that MRI can detect focal cortical dysplasia, which corresponds to the epileptogenic focus on EEG, and SPECT may help to detect a functional abnormality in the same region.

Radionuclide skeletal survey for pediatric neoplasms

A prospective study comparing the radionuclide skeletal survey with the radiographic survey was performed for 159 children with known primary neoplasm. Forty-four showed radionuclide evidence of metastases, while only 14 had radiographic evidence. Thus, 68% of metastases were discovered by bone imaging alone. No false negative images for metastases were obtained, though there was one false negative image for primary tumor. Radionuclide skeletal survey is recommended as the primary evaluation for bony metastases in all children with neoplasms. Suspect areas should then receive complete radiographic examination.

Ventricular performance before and after fontan repair for univentricular atrioventricular connection: Angiographic and radionuclide assessment

OBJECTIVES This study was designed to evaluate changes in ventricular volume, mass and cardiac function before and after creation of an atrial to pulmonary connection in patients with a univentricular atrioventricular connection. BACKGROUND Intact systolic and diastolic performance is critical for successful establishment of an atrial dependent circulation, and few studies are available comparing cardiac performance before and after creation. METHODS With the use of radionuclide blood pool imaging and ventricular cineangiography, 54 patients (mean age 6.4 +/- 3.4 years) were studied. Twenty-eight patients were investigated preoperatively and 36 greater than 1 year after repair and compared with a control population. RESULTS Before operation, end-diastolic volume and wall mass were significantly increased compared with those of control subjects; however, the mass/volume ratio was normal (1.08 +/- 0.31 g/ml for the preoperative group; 0.97 +/- 0.19 for control subjects). Although end-diastolic volume returned to normal after the procedure, wall mass remained elevated and contributed to an elevated mass/volume ratio (1.20 +/- 0.38 g/ml). After the procedure, systemic vascular resistance index was significantly elevated compared with that before surgery or with that of control subjects (1,199 +/- 373, 2,120 +/- 645, 1,556 +/- 275 dynes.s.cm-5.m2: pre- and postrepair and control subjects, respectively). Radionuclide studies demonstrated that preoperative ejection fraction (52 +/- 9, 50 +/- 9, 60 +/- 8%), peak ejection (2.58 +/- 0.66, 2.95 +/- 0.81, 3.73 +/- 0.70 EDV/s) and peak filling rates (2.84 +/- 0.75, 2.75 +/- 0.79, 3.84 +/- 0.51 end-diastolic volumes [EDV/s]) were significantly reduced compared with those of control subjects and remained so after surgery. CONCLUSIONS These data suggest that systolic and diastolic function is depressed preoperatively in these patients, remains unchanged after the creation of an atrial-dependent circulation and is associated with an increased systemic vascular resistance. Long-term issues addressing preservation of cardiac function need to be prospectively studied.

Splenic trauma—Nonoperative management and long-term follow-up by scintiscan

1972 TO April, 1977, 28 children with splenic rupture were managed nonoperatively. The diagnosis was confirmed by splenic scintigraphy with 99mTechnetium-sulfide colloid. Those patients whose condition was stable and satisfactory or maintained stable with intravenous fluids or blood transfusions were not operated upon. All patients sustained their ruptured spleens as a result of trauma, except for one spontaneous rupture in a patients with infectious mononucleosis. Emergency laparotomy for deterioration of clinical condition or late complications was never necessary. Splenic scans were repeated in 22 patients to assess resolution. Fourteen patients had minimal residual defects but there were no late complications detected.

Transient myocardial ischemia of the newborn infant demonstrated by thallium myocardial imaging

Five term and two premature newborn infants were referred for respiratory distress and congestive heart failure, and were found to have electrocardiographic Q or ST-T abnormalities suggesting ischemia. Echocardiographic and/or hemodynamic assessment excluded anatomic heart disease in six infants. In three infants, moderate or severe hemodynamic impairment within 36 hours of age was suggested by these studies. Myocardial perfusion images in all patients showed very poor myocardial uptake of thallium 201, compatible with global myocardial ischemia. Infants of similar age with myocarditis, or with congenital heart disease and congestive failure, had normal myocardial uptake. Rapid clinical improvement occurred within three to seven days. Two to five months later, all infants were well. Two had persistent electrocardiographic abnormalities but repeat thallium 201 imaging in six demonstrated almost normal myocardial uptake. These data provide further evidence that perinatal respiratory distress may be associated with myocardial dysfunction and congestive heart failure in some infants without anatomic heart disease, and suggest that myocardial dysfunction in these infants is associated with global myocardial ischemia, most of which is transient. The timing and nature of the insult causing the ischemia are unclear.