Successful Treatment for Hepatoblastoma in Trisomy 18: A Case Report.

Children with trisomy 18 tend to develop hepatoblastoma. Since the introduction of appropriate management for organ malfunction, individuals with trisomy 18 have come to have a longer life expectancy. However, the predisposition to hepatoblastoma becomes a significant issue for the quality of a case. Here, we present a rare multifocal hepatoblastoma involving predominantly Couinaud segments 5 and 7 in a 10-month-old boy with trisomy 18. Though the first-line cisplatin monotherapy resulted in unsatisfactory tumor shrinkage, the second-line neoadjuvant chemotherapy administrating irinotecan and vincristine gave rise to significant tumor reduction in volume, leading to the completion of partial resection of the liver without the microscopic residual disease. The patient has been free from recurrence for 44 months. Because anatomical right hepatectomy can cause circulatory instability, including acute onset of pulmonary hypertension in trisomy 18 patients, physicians should balance treatment benefits and potential adverse effects. Our successful experience utilizing a combination of efficacious and less cardiotoxic neoadjuvant chemotherapy followed by the partial hepatectomy encourages physicians to treat a patient with trisomy 18 and tackle hepatoblastoma with a genetic background.

Ultrasonographic study of intestinal Doppler blood flow in infantile non-IgE-mediated gastrointestinal food allergy.

BACKGROUND: Although non-IgE-mediated gastrointestinal food allergy has increased rapidly in Japan, a small number of reports has evaluated B-mode and Doppler ultrasonographic findings in the acute phase of infantile gastrointestinal milk allergy. The aim of the present study was to compare the diagnostic utility of ultrasonographic findings and laboratory allergic data in non-IgE-mediated infantile gastrointestinal milk allergy. METHODS: Sixteen cases of active non-IgE-mediated infantile gastrointestinal milk allergy, diagnosed by food elimination tests and oral food challenge tests (OFCTs) (group A), 15 cases of acute viral gastroenteritis (AGE) (group B), and 15 controls (group C) were enrolled. 1) B-mode abdominal ultrasound findings, 2) laboratory allergic data including eosinophil counts (Eos), serum IgE, and the antigen-specific lymphocyte proliferation test (ALPT) against milk protein, and 3) vessel density (VD) indirectly quantified by gastrointestinal Doppler flow at jejunum, ileum, and sigmoid colonic mucosae were evaluated and compared among the groups. RESULTS: In the small intestine, wall thickening, dilation, mesenteric thickening, and poor peristalsis were found in 100%, 62.5%, 93.7%, and 100%, respectively, in group A. Eos, IgE, ALPT, and VD were positive in 25.0%, 0%, 87.5%, and 100%, respectively, in group A. Small intestinal VD was significantly greater in group A than in groups B (jejunum p < .001; ileum p < .001) and C (jejunum p < .001; ileum p < .001), with no significant differences between groups B and C (jejunum: p = .74; ileum: p = .73). CONCLUSIONS: Abdominal Doppler ultrasonography and small intestinal VD at symptomatic state can support the diagnosis and evaluation of non-IgE-mediated infantile gastrointestinal milk allergy with symptoms of vomiting, diarrhea, and failure to thrive.

Color Doppler Ultrasound Evaluation of Ureteral Jet Angle to Detect Vesicoureteral Reflux in Children.

PURPOSE: Vesicoureteral reflux is the most common urinary tract abnormality in children. Although voiding cystourethrography is the gold standard for diagnosing reflux, this approach is invasive. We evaluated color Doppler ultrasound measurement of ureteral jet angle as a noninvasive screening tool for detecting reflux. MATERIALS AND METHODS: We retrospectively evaluated 125 children (250 renal units) who presented with urinary tract infections or hydronephrosis. All patients underwent color Doppler ultrasonography and voiding cystourethrography. Ureteral jet angle was measured as angle between the direction of the ureteral jet and interureteral ridge. RESULTS: Reflux was diagnosed in 80 patients (117 renal units). Mean ± SD ureteral jet angle was significantly greater in refluxing units (67.9 ± 18.7 degrees) than in nonrefluxing units (47.8 ± 17.3 degrees, p <0.0001). Angle value in each reflux grade became significantly greater according to grade (p <0.0001). Overall ability of ureteral jet angle to detect grade III to V and grade IV/V reflux, as given by the area under the receiver operating characteristic curve, was 0.81 and 0.88, respectively. A cutoff angle of 55 degrees or greater detected grade III to V and grade IV/V reflux with a sensitivity of 85.5% and 94.7%, respectively. A cutoff angle of 70 degrees or greater diagnosed grade IV/V reflux with a sensitivity of 81.6% and a specificity of 82.7%. CONCLUSIONS: Color Doppler ultrasound measurement of ureteral jet angle represents a simple and noninvasive method that is applicable as a screening tool to detect high grade vesicoureteral reflux in children.