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Biomedical subjects

J L Strife

Publications and source records attributed to J L Strife.

At least 37 records · Page 2Linked to original sources

Radiographic evaluation of the neonate with congenital heart disease.

Remarkable advances in pediatric cardiology have been spurred by the explosion of technologies both in interventional and surgical techniques and the ability to manipulate the genome of experimental animals. After a brief discussion concerning the striking advances in the molecular understanding of congenital heart disease, this article focuses on clues to the diagnosis of congenital heart disease and on chest radiography and common, specific lesions of the neonate such as hypoplastic left heart, transposition of the great vessels, and severe tetralogy of Fallot. The impact of treatment protocols involving interventional cardiology in the neonate also are discussed.

Heart Defects, Congenital↗

Correlation between findings on chest radiography and survival in neonates with congenital diaphragmatic hernia.

OBJECTIVE: Predictors of survival are helpful when deciding on aggressiveness of care of neonates with congenital diaphragmatic hernia and respiratory failure. We evaluated findings on chest radiography as potential predictors of survival in these patients. MATERIALS AND METHODS: Findings on chest radiographs of neonates less than 24 hr old with congenital diaphragmatic hernia were evaluated. Radiographic findings analyzed included percentage of aerated ipsilateral lung, percentage of aerated contralateral lung, mediastinal shift, and hernia contents. Each finding was compared with survival (equated with hospital discharge) using a Mantel-Haenszel chi-square test. Survival was also determined using the total number of poor prognostic findings present in any one patient. RESULTS: In the 73 neonates with congenital diaphragmatic hernia in our study, the overall survival rate was 55%. There were statistically significant relationships between survival rate and percentage of ipsilateral aeration (p = 0.001), percentage of contralateral aeration (p = 0.016), and mediastinal shift (p = 0.026). The survival rate for multiple poor prognostic factors was 0% with four of four factors and 20% with three of four factors (p = 0.001). Survival rate was not influenced by prematurity (p = 0.102), sex (p = 0.104), or side of hernia (p = 0.895). CONCLUSION: Findings on initial chest radiography are helpful in predicting survival in neonates with congenital diaphragmatic hernia.

Female↗

Research in pediatric radiology: preparing for our future.

The changing healthcare environment challenges the traditional role of academic medicine. Pediatric radiologists involved in research, education and teaching are under pressure to forego such endeavors in favor of increased clinical responsibilities. How we perform research must change if our subspecialty is to survive into the future, as we cannot afford to give up our traditional duties as researchers and educators. To do so could summon the beginning of the end for pediatric radiology. Rather, we must begin the process of restructuring research in pediatric radiology to take into account the efficient use of our resources which provide the greatest return on our investment.

Academic Medical Centers↗

Extrinsic airway compression secondary to pulmonary arterial conduits: MR findings.

Abnormal enlargement or malposition of any vascular structure or mass adjacent to the airway can cause extrinsic airway compression. In children with previous surgery for congenital heart disease, mass effect from prosthetic devices or alteration in the anatomic position of normal structures can lead to extrinsic airway compression. Because many children have complex medical problems after cardiac surgery, wheezing may be attributed to cardiac causes and airway compression may not be investigated. Furthermore, the distal airway compression seen in these children often is not visualized on chest radiographs. MR imaging can be useful in evaluating extrinsic airway compression in these patients. We present the MR imaging of two patients with symptomatic extrinsic airway compression secondary to pulmonary arterial conduits.

Airway Obstruction↗

Sleep fluoroscopy for localization of upper airway obstruction in children.

The management of children with upper airway obstruction (UAO) in whom previous airway surgeries or concomitant craniofacial or neuromuscular abnormalities exist is complicated by potential obstruction at multiple sites. Sleep fluoroscopy (SF) provides a dynamic representation of relative degrees of obstruction at multiple levels of the pediatric airway. Fifty-five SF studies were performed on 50 infants and children to localize obstructive sites. Correlation was assessed with findings on direct laryngoscopy and bronchoscopy under spontaneous ventilation. In 24 (44%), endoscopic and SF findings correlated exactly. The SF studies identified a site of UAO in 11 patients with normal findings on endoscopic examination and multiple sites of UAO in 16 others. Two thirds of these occurred at the hypopharynx and tongue base. The SF studies failed to detect 5 airway abnormalities in 4 patients. The sensitivity of SF for endoscopically verified laryngotracheal lesions was lowest for glottic (67%) and subglottic (70%) locations and higher for tracheal (92%) and supraglottic (100%) sites. Sleep fluoroscopy altered the course of treatment in 26 (52%) children. It appears to be a valuable adjunct to endoscopy in the identification and management of pediatric UAO when hypopharyngeal collapse or multiple levels of obstruction are suspected.

Adolescent↗

Systemic arterial collateral esophageal indentations in pseudotruncus arteriosus.

Barium esophagrams were obtained in two patients with respiratory problems whose underlying congenital heart disease was pseudotruncus arteriosus type I. The esophagrams revealed vascular indentations on the esophagus, one posteriorly, and the other anteriorly. Both types of indentations were secondary to aorto-pulmonary communicating arteries from the descending aorta.

Barium Sulfate↗

Plain-film assessment of the neonate with D-transposition of the great vessels.

The presenting chest radiographs of 27 new-born patients with D-transposition of the great vessels (D-TGV) were evaluated for the degree of pulmonary flow as well as other findings classically described in D-TGV (narrow superior mediastinum, radiographically absent thymus, inapparent main pulmonary artery, non-visualization of the malpositioned aortic arch, asymmetric pulmonary blood flow, and cardiomegaly). Of the 27 patients, 22 (82%) demonstrated normal or decreased flow. The majority of the D-TGV patients also failed to demonstrate any of the other classically described radiographic findings. A normal chest radiograph is the most common presenting scenario in the neonate with D-TGV.

Cardiac Catheterization↗

Multicystic dysplastic kidney in children: US follow-up.

Eighty-one cases of multicystic dysplastic kidney (MCDK) in children were diagnosed over the past 11 years at the authors' institution: 25 children had their kidneys surgically removed, eight with bilateral total involvement died, and 48 underwent serial follow-up ultrasonography (US) of their kidneys. Follow-up included 193 serial ultrasound (US) studies (mean, four per patient) for a total of 1,468 months (mean, 30.5 months). In the 48 patients followed up, 32 (67%) kidneys showed a decrease in size, nine (19%) showed no change, and five (10%) increased in size, and in two (4%), a change in size could not be determined. In seven of the 48 (15%) children, the MCDKs decreased in size, and, at follow-up US, no renal tissue could be found. In those patients in whom MCDKs decreased in size. Serial US characteristics changed from predominantly an enlarged cystic structure to a small dysplastic or absent kidney. Two of the five kidneys that increased in size were surgically removed, and MCDK was pathologically confirmed. A nonsurgical approach to the treatment of patients with MCDK is supported by this study.

Child, Preschool↗

Comparison of velopharyngeal gap size in patients with hypernasality, hypernasality and nasal emission, or nasal turbulence (rustle) as the primary speech characteristic.

Velopharyngeal insufficiency was assessed using multiview videofluoroscopy on eight patients with hypernasality, 10 patients with hypernasality and audible nasal emission, and 10 patients with nasal turbulence (rustle). Patients demonstrating hypernasality, with or without audible nasal emission, were found to have a significantly larger velopharyngeal gap than those with nasal rustle. This finding suggests that the degree of the velopharyngeal insufficiency can be predicted to some extent based on perceptual assessment. If the presence of a nasal rustle suggests a small velopharyngeal gap, despite the severe distortion of speech, then speech therapy should be considered prior to surgery in those cases.

Adolescent↗

Left-lobe hepatic transplants: spectrum of normal imaging findings.

The limited availability of suitable size-matched donor whole organs has been a major cause of morbidity and mortality in infants and children requiring liver transplantation. Techniques for transplanting segments of the liver have successfully addressed this problem. The surgical anatomy of the left hepatic lobe transplant is unique in three primary respects: the cut edge, the presence of an enteric Roux loop for biliary drainage, and the alteration in the position and number of hepatic vessels. The spectrum of normal imaging findings in 17 left-lobe allografts is illustrated.

Anastomosis, Surgical↗

Low-grade vesicoureteral reflux. Variability in grade on sequential radiographic and nuclear cystograms.

Paired voiding cystourethrogram (VCU) and nuclear cystogram (NC) studies in 68 children with vesicoureteral reflux (VUR) were analyzed to determine the variability of VUR on sequential studies. Sequential paired examinations included 82 studies where a VCU was followed by a repeat VCU study and 128 studies where a VCU was followed by a NC study. Grade I VUR on the initial VCU was difficult to duplicate on repeat cystography, regardless of the follow-up modality. When the initial VCU showed no VUR, 20% of follow-up studies demonstrated VUR. Follow-up VCU and NC studies showed similar results in detecting increases and decreases in the grade of VUR. Low-grade VUR may be intermittent, and clinical therapeutic decisions should take this variability into account.

Child↗

Hyperechoic renal medullary pyramids in infants and children.

Fifty-five children (34 boys, 21 girls; age range, 1 day to 18 years) with increased echogenicity of the renal medullary pyramids at ultrasound evaluation were identified. The clinical diagnoses associated with hyperechoic medullary pyramids could be separated based on the presence or absence of hypercalciuria. Patients with drug-induced hypercalciuria included 10 infants treated with furosemide, two treated with long-term steroid therapy, and one treated with excessive amounts of vitamin D. Other clinical conditions associated with hypercalciuria included renal tubular acidosis (n = 10), Bartter syndrome (n = 5), hyperparathyroidism (n = 3), Williams syndrome (n = 2) and medullary sponge kidney (n = 2). Ten children with transient renal insufficiency and three with sickle cell disease had normal urine calcium concentration. Isolated disease entities accounted for the remainder of cases. A specific diagnosis can usually be made in a patient with hyperechoic renal medullary pyramids by using a systematic clinical approach that includes evaluation of patient age, serum and urine calcium concentration, and renal function.

Acidosis, Renal Tubular↗

Acquired structural genitourinary abnormalities contributing to deterioration of renal function in older patients with nephropathic cystinosis.

The natural progression of nephropathic cystinosis to end stage renal disease can be delayed, sometimes by many years, by the reducing agent, cysteamine, which lowers intracellular cystine content to near normal. We report on two patients with nephropathic cystinosis who were treated with cysteamine and developed structural genitourinary abnormalities which may have contributed to an increase in the rate of decline of renal function. One patient, aged 11 years, was found to have massive megacystis and hydroureteronephrosis but no anatomic bladder outlet obstruction. His abnormality was presumed to be related to chronic high urine volumes leading to megacystis and physiologic ureteral obstruction. Vesicostomy stabilized renal function. The second patient, aged 11 1/2 years, was found to have bilateral renal cystic disease which presumably was acquired and may have been related to long-standing hypokalemia. Minor renal abnormalities were found by ultrasound in five additional cystinotic children. We concluded that older children with nephropathic cystinosis may be prone to acquire structural abnormalities of their kidneys or urinary tract.

Creatinine↗

Ileocolic intussusception: extensive reflux of air preceding pneumatic reduction.

The use of an air enema for diagnosis and treatment of intussusception has recently gained popularity. The current end point for reduction is the reflux of air into the terminal ileum. The authors report three cases in which air freely refluxed into the terminal ileum without complete reduction of the intussusceptum. Thus, reflux of air alone cannot be relied on as the sole criterion for reduction. Close examination of the cecum for a persistent filling defect is imperative to exclude unsuccessful reduction.

Enema↗

Evaluation of children for liver transplantation: value of MR imaging and sonography.

Diagnostic imaging plays a major role in the diagnosis and treatment of chronic liver disease in children. Decisions regarding the need for sclerotherapy, palliative shunts, and anatomic suitability for hepatic transplantation are based on the upper abdominal anatomy. Thirty-six children with various forms of chronic liver disease were referred for diagnostic imaging, including MR and sonography. Each study was evaluated independently without knowledge of the other examination. The first six patients were evaluated retrospectively and the last 30 patients prospectively. The size of the portal vein and inferior vena cava varied in this population, with excellent agreement between sonography and MR. A 2-mm portal vein was shown only on MR imaging in two patients, but MR missed a 3-mm portal vein. Portosystemic collateral vessels also were evaluated and noted to be detected more readily with MR imaging (64%) than with sonography (22%). In particular, paraumbilical veins were detected more often with MR (28% vs 6% by sonography). Associated or coexisting anomalies were relatively common in children with chronic liver disease (14%). These included polysplenia (two cases), preduodenal portal vein (one), unilateral dysplastic kidneys (two), ureteropelvic junction obstruction (one), and splenic (one) and renal (one) cysts. MR imaging was more sensitive than sonography for detection of abnormal anatomy in this group of patients and should be considered the pretransplantation imaging technique of choice in children with end-stage liver disease.

Adolescent↗

The position of the trachea in infants and children with right aortic arch.

In infants, the site of the aortic arch is frequently inferred from the position of the intrathoracic trachea. We retrospectively reviewed the plain frontal chest radiographs of 72 patients with cyanotic congenital heart disease and right aortic arch documented by cardiac catheterization. In infants, the position of the trachea was variable: 47% had the trachea on the right, 33% had the trachea midline and in 7% the trachea was on the left. As the child grows, the aortic arch progressively indents the trachea, and the descending aorta is better visualized. Since the position of the trachea in infants with right aortic arch is variable, one must be cautious in inferring the site of the aortic arch by the position of the trachea.

Aorta, Thoracic↗