Roles of uroplakins in plaque formation, umbrella cell enlargement, and urinary tract diseases.

The apical surface of mouse urothelium is covered by two-dimensional crystals (plaques) of uroplakin (UP) particles. To study uroplakin function, we ablated the mouse UPII gene. A comparison of the phenotypes of UPII- and UPIII-deficient mice yielded new insights into the mechanism of plaque formation and some fundamental features of urothelial differentiation.

Assessment of vasculature using combined MRI and MR angiography.

Objective: The purpose of this study was to compare combined cine gradient-recalled echo MRI and MR angiography with conventional angiography in the evaluation of the pulmonary vascular supply in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

Materials And Methods: Eleven patients who underwent both MRI and conventional angiography were retrospectively reviewed. Contiguous 2D cine gradient-recalled echo images (TR range/TE, 30-80/4.

Suspected appendicitis in children: focused CT technique for evaluation.

Purpose: To determine the accuracy of a focused computed tomographic (CT) technique with oral and intravenous contrast materials for the diagnosis of appendicitis.

Materials And Methods: Ninety-three abdominal-pelvic contrast material-enhanced CT scans obtained during 6 years in 54 girls and 39 boys (age range, 1-18 years) with right lower quadrant pain were retrospectively reviewed. The detected abnormal findings were recorded as being in the region above the upper pole of the right kidney, between the upper pole of the right kidney and the lower pole of the right kidney (RLP), or below the iliac crest.

Epidermolysis bullosa, pyloric atresia, and obstructive uropathy: a report of two case reports with molecular correlation and clinical management.

The epidermolysis bullosa-pyloric atresia-obstructive uropathy (EB-PA-OU) association is a rare, but well-described multisystem disease. While the prognosis at this time is still poor, an increasing number of patients are surviving to adolescence with aggressive care. It is important to understand this syndrome in order to anticipate medical complications and offer preventive strategies where possible.

Obstructive jaundice caused by placement of a nasoenteric feeding tube.

Nasoenteric feeding tubes are a safe and effective means for providing nutritional support to the critically ill patient. Serious complications have been reported, but usually are the result of an improper path of the tube during placement. The authors report a case of ampullary obstruction and jaundice caused by a nasoenteric feeding tube, presumably caused by coiling of the tube in the duodenum.

Interrupted aortic arch: diagnosis with gadolinium-enhanced 3D MRA.

Purpose: Our goal was to describe the use of gadolinium-enhanced 3D MR angiography (MRA) in the diagnosis of interrupted aortic arch (IAA).

Method: A review of our MR data base from a 1 year period yielded three patients (1 day, 8 days, and 16 years old) with IAA. All were referred for evaluation of aortic arch abnormalities, only one of whom had suspected IAA.

MR findings in Shone's complex of left heart obstructive lesions.

Background: Shone's complex is a series of four obstructive or potentially obstructive left-sided cardiac lesions (supravalvular mitral ring, parachute deformity of the mitral value, subaortic stenosis, and coarctation of the aorta). Both the complete form (all four lesions) and incomplete forms (less than four lesions) have been described.

Objective: To determine which abnormalities of Shone's complex could be characterized by MR.

Resonance imaging of a ruptured aneurysm of the sinus of Valsalva.

Ruptured aneurysm of an aortic sinus of Valsalva is a rare cause of left-to-right shunting. We show how resonance imaging can be used to make the diagnosis. This technique can successfully characterize the shunt as well as determine the presence of associated anomalies, such as ventricular septation and aortic regurgitation.

Pediatric hepatic CT: an injection protocol.

Objective: To determine an injection protocol for pediatric hepatic CT and to investigate the use of power injection.

Materials And Methods: Eighty-seven studies were prospectively performed using ioversol (320 mg iodine per cc) at 2 cc/kg. Three techniques were used: helical (1 s/slice); dynamic, non-breath-hold (5.

Traumatic abdominal pseudoaneurysm secondary to child abuse.

Traumatic injury to the descending thoracic and abdominal aorta is uncommon in children and is usually secondary to recognized blunt trauma. Child abuse has not been previously reported as a cause. We report a 3-year-old boy who was kicked in the abdomen by his father.

Application of thin-section low-dose chest CT (TSCT) in the management of pediatric AIDS.

The aim of this study was to evaluate the usefulness of thin-section low-dose computed tomography (TSCT) in the management of children with AIDS, as chest radiographs (CXR) often fail to adequately explain the patients' clinical status. We performed 54 noncontrast TSCTs on 32 children. The patients aged from 3 months to 14.

Monitoring of girls undergoing medical therapy for isosexual precocious puberty.

We evaluated the use of sonography in monitoring the efficacy of suppressive therapy with a gonadotropin releasing hormone analogue in girls being treated for isosexual precocious puberty. Ten girls 5 to 9 years of age underwent serial sonography and hormonal stimulation tests on the same day. Sonographic trends of decreasing ovarian volume and uterine length indicated early suppression even when absolute values were above threshold.

Feasibility of high-resolution, low-dose chest CT in evaluating the pediatric chest.

Thin-section, high-resolution (1.0/1.5 mm thick slices), low-dose chest CT scans were performed in 55 infants and children.

Opportunistic infections and tumors in immunocompromised children.

HIV infection is responsible for a major proportion of the immunodeficiency disease seen in the pediatric population. The radiologic findings are varied and generally non-specific. The development of secondary neoplasms may present new diagnostic and therapeutic challenges as therapy for superimposed infections becomes more successful.

Pictorial essay. Current evaluation of the patient with abnormal visceroatrial situs.

Patients suspected of having derangement of solitus asymmetry should be evaluated individually to determine abdominal visceral and vascular arrangement and to investigate associated problems. This evaluation should begin with plain films to assess cardiac and gastric positions and pulmonary vascularity. Assessment of the bronchial branching patterns should be attempted in all cases.

Sonography of the simple and complicated ipsilateral fused kidney.

Anomalies of renal fusion and their sonographic findings can be straightforward or quite complex. Sonograms of eight cases of simple and complicated anomalies of renal fusion in pediatric patients are reviewed to determine characteristic sonographic findings in such cases. The correct diagnosis can be suspected by evaluation of the following parameters: (1) the echoic texture of the renal "mass"; (2) the differential orientation of the renal pelves; (3) the content of the contralateral renal fossa; (4) the size of the ipsilateral kidney; (5) extension of the isthmus medially anterior to the spine; and (6) presence of a deep anteroposterior notch.

Bilateral duplex ectopic ureters terminating in the seminal vesicles: sonographic and CT diagnosis.

A 15-year-old boy presented with a short history of right-sided flank pain was found to have bilateral duplex renal systems with obstructed upper poles. Sonographic examination and contrast-enhanced delayed computed tomography (CT) performed at the conclusion of the excretory urography (EU) demonstrated termination of both obstructed ureters in the seminal vesicles. These noninvasive studies allowed accurate preoperative diagnosis of this abnormality.

Spondylothoracic dysplasia. Clinical and sonographic diagnosis.

Spondylothoracic dysplasia, also known as short-trunk dwarfism or Jarcho-Levin syndrome, is a fatal autosomal recessive disorder characterized by vertebral and spinal defects with a short thorax. Until recently, in utero diagnosis could only be made radiographically. Sonographic criteria for antenatal diagnosis are discussed in conjunction with a review of the literature.

Congenital anomalies of the lungs in adults: MR diagnosis.

Over an 18-month period, 11 adults with congenital pulmonary anomalies within the sequestration spectrum, identified either surgically or radiographically by CT and/or angiography, were evaluated with MR. These included seven patients with bronchogenic cysts, two with intralobar pulmonary sequestrations, one with scimitar syndrome, and one with bronchial atresia. In each case, MR showed at least some findings indicative of the correct diagnosis.