Hyun-Hae Cho

Clinical experiences of different dosing schedules of rituximab in pemphigus with various disease severities.

Rituximab, a monoclonal antibody directed against B lymphocytes, has been found to be a therapeutic agent for severe, refractory autoimmune bullous diseases. However, a dosing schedule or treatment indication of rituximab has not yet been established.

Application of IFN‐γ releasing assay for the diagnosis of erythema induratum of Bazin

Background  Erythema induratum of Bazin (EIB) is regarded to be a hypersensitive reaction to the concomitant tuberculosis. Recently, interferon‐γ releasing assay (IGRA) has been focused as a promising tool in the diagnosis of latent tuberculosis. However, there has been no large scale study to investigate the usefulness of IGRA in the diagnosis of EIB.

Ultrasound-guided contrast enema for meconium obstruction in very low birth weight infants: Factors that affect treatment success

INTRODUCTION This study aimed to assess the therapeutic results of ultrasound (US)-guided water-soluble contrast enema in very low birth weight (VLBW) preterm infants (<1,500 g) with meconium obstruction and to study factors that affect therapeutic results. METHODS This study included a total of 33 consecutive VLBW infants with clinically diagnosed meconium obstruction underwent US-guided water-soluble contrast enema, from April 2007 to March 2014. Patients were classified into two groups based on to procedure outcome: the success group (evacuation of the meconium plug resolution followed by improved bowel distention within 2 days of the procedure, without additional interventions), and the failure group (the contrast enema failed to relieve the obstruction, or other procedure-related complications occurred). Patient- and mother-related clinical factors and procedure-related factors were compared between both groups. RESULTS Overall success rate was 54.5%, with 18 successful (M:F=10:8), and 15 failure (M:F=7:8) cases. When compared with the failure group, the success group patients showed statistically significant older gestational age (29(+1) vs. 27 weeks; p=0.028), larger birth weight (1023.1g vs. 790.3g; p=0.048), and higher body weight on the day of the procedure (1036.2g vs. 801.6g, p=0.049). However, no statistically significant differences were seen between other patient and maternal factors. Among the procedure-related factors, retrial of contrast injection during the procedure was associated with significantly higher success than the single trial (p=0.027). The presence of refluxed contrast into the distal ileum was the statistically significant predictor for success of the procedure (p=0.038). There were three cases of bowel perforation (9.1% per person). CONCLUSION US-guided water-soluble contrast enema in VLBW infants with meconium obstruction showed a 54.5% success rate and a 9.1% perforation rate per person. Among the procedure-related factors, retrial of contrast injection during the procedure and the presence of refluxed contrast into the distal ileum were related to the success of the procedure.

Case of mild Schmid-type metaphyseal chondrodysplasia with novel sequence variation involving an unusual mutational site of the COL10A1 gene.

Schmid-type metaphyseal chondrodysplasia (MCDS) is characterized by short stature with short legs, bowing of the long bones, coxa vara, and waddling gait. MCDS is a relatively common form of MCD. Most mutations that cause MCDS occur within the carboxyl-terminal non-collagenous domain (NC1) of the COL10A1 gene. We performed mutational analysis of the COL10A1 genes in 4 unrelated Korean patients with diagnosed MCDS. Mutational analysis of COL10A1 identified c.1904_1915delinsT (p.Gln635LeufsX10) and c.1969dupG (p.Ala657GlyfsX10), 2 novel frameshift mutations, and c.2030T>A (p.Val677Glu) and c.862G>C (p.Gly288Arg) at unusual mutational sites, which could be pathogenic. We present the first report of the molecular characteristics of MCDS in 4 Korean patients. Our findings suggest that a novel sequence variation involving an unusual mutational site of the COL10A1 gene can cause mild MCDS.

Pediatric Chest CT: Wide-Volume and Helical Scan Modes in 320-MDCT

OBJECTIVE The purpose of this study was to compare wide-volume and helical pediatric 320-MDCT of the chest with respect to radiation dose and image quality. MATERIALS AND METHODS From November 2012 to September 2013, 59 wide-volume and 47 helical pediatric chest 320-MDCT images were obtained. The same tube potential and effective tube current-time product were applied in the two groups according to patient weight (group A, < 10 kg, n = 18; group B, 10-19.9 kg, n = 60; group C, 20-39.9 kg, n = 28). To compensate for overranging, adjusted CT dose index (CTDI) was calculated by dividing dose-length product (DLP) by the scan ranges imaged. Adjusted CTDI, DLP, overall image quality, motion artifact, noise, and scan ranges were compared by Mann-Whitney U test or t test. RESULTS The adjusted CTDI was significantly lower in the group who underwent wide-volume CT than in the group who underwent helical CT (weight group A, p < 0.001; group B, p < 0.001; group C, p = 0.003). The DLP was lower in the wide-volume group than in the helical CT group in weight groups A (p < 0.001) and B (p < 0.001) but not in group C (p = 0.162). All CT scans were of diagnostic quality, and there was no significant difference between the wide-volume and helical CT groups (p = 0.318). The motion artifact score was significantly higher in the wide-volume group than in the helical CT group in groups B (p < 0.001) and C (p = 0.010) but not in group A (p = 0.931). The noise was significantly lower in the wide-volume group than in the helical CT group (p < 0.001). CONCLUSION In pediatric chest CT, use of wide-volume CT can decrease radiation exposure while preserving image quality. It is associated with less noise than helical CT but may be subject to more motion artifact.

Ultrasonography evaluation of infants with Alagille syndrome: In comparison with biliary atresia and neonatal hepatitis

OBJECTIVE To evaluate the ultrasonography (US) features of Alagille syndrome (ALGS), as compared with biliary atresia (BA) or neonatal hepatitis (NH). METHODS Our study included 23 ALGS, 75 BA and 70 NH patients. The initial US images were retrospectively reviewed for gallbladder (GB) morphology with systemic classification, GB length and luminal area, presence of triangular-cord (TC) sign and hypertrophied hepatic-artery. The presence of anomalies associated with ALGS was evaluated. The diagnostic values of each finding and their combinations were evaluated. RESULTS Both ALGS (57%) and BA (79%) were more frequently associated with abnormal GB shapes than NH (19%, all P<0.001). The short and small GBs were more frequently observed in ALGS and BA than in NH (all P<0.001). None in the ALGS and NH showed TC sign, while 41% in the BA did (all P<0.001). Hypertrophied hepatic-artery was noted less frequently in both ALGS (13%) and NH (14%) than in BA (83%, all P<0.001). The combination of US criteria with associated anomalies increased the positive-predictive-value for ALGS. CONCLUSION Abnormal shaped GB with absence of the TC sign and hypertrophied hepatic-artery and presence of associated anomalies can be a differential point of ALGS.

Free-Breathing Radial 3D Fat-Suppressed T1-Weighted Gradient-Echo Sequence for Contrast-Enhanced Pediatric Spinal Imaging: Comparison With T1-Weighted Turbo Spin-Echo Sequence

OBJECTIVE The purpose of this study was to compare free-breathing radially sampled 3D T1-weighted gradient-echo acquisitions (radial volumetric interpolated breath-hold examination [VIBE]) with a T1-weighted turbo spin-echo (TSE) sequence for contrast-enhanced spinal imaging of children with CNS tumors. MATERIALS AND METHODS Twenty-eight consecutively registered children with CNS tumors underwent evaluation of leptomeningeal seeding with 1.5-T MRI that included both radial VIBE and T1-weighted TSE sequences. For qualitative analysis, overall image quality; presence of motion, CSF flow, and radial artifacts; and lesion conspicuity were retrospectively assessed with scoring systems. The signal-intensity uniformity of each sequence was evaluated for quantitative comparison. The acquisition times for each sequence were compared. RESULTS Images obtained with the radial VIBE sequence had a higher overall image quality score than did T1-weighted TSE images (3.61 ± 0.73 vs 2.80 ± 0.69, p < 0.001) and lower motion artifact (0.82 ± 0.43 vs 1.29 ± 0.56, p = 0.001) and CSF flow artifact (0 vs 1.68 ± 0.67, p < 0.001) scores. Radial artifacts were found only on radial VIBE images (1.36 ± 0.31 vs 0, p < 0.001). In 13 patients with spinal seeding nodules, radial VIBE images showed greater lesion conspicuity than did T1-weighted TSE images (4.23 ± 0.52 vs 2.47 ± 0.57, p = 0.005). Radial VIBE images had diminished signal-intensity variation compared with T1-weighted TSE images in air, spine, and muscle (p < 0.01). The mean acquisition times were not significantly different between the two sequences (p = 0.117). CONCLUSION For pediatric spinal imaging, radial VIBE images had better image quality and lesion conspicuity and fewer CSF and respiratory motion artifacts than did T1-weighted TSE images in a similar acquisition time.

Limited Dorsal Myeloschisis and Congenital Dermal Sinus: Comparison of Clinical and MR Imaging Features

These investigators retrospectively reviewed the clinical and MR imaging findings of 12 patients with limited dorsal myeloschisis and 10 patients with congenital dermal sinus. A crater covered with pale epithelium was the most common skin lesion in limited dorsal myeloschisis (83%). Infectious complications were common in congenital dermal sinus (60%), but not found in limited dorsal myeloschisis. They show that limited dorsal myeloschisis has distinct MR imaging features including a visible intrathecal tract with dorsal tenting of the cord at the tract-cord union. BACKGROUND AND PURPOSE: While limited dorsal myeloschisis is a distinctive form of spinal dysraphism, it may be confused with congenital dermal sinus. The aim of this study was to describe clinical and MR imaging findings of limited dorsal myeloschisis that can distinguish it from congenital dermal sinus. MATERIALS AND METHODS: We retrospectively reviewed the clinical and MR imaging findings of 12 patients with limited dorsal myeloschisis and 10 patients with congenital dermal sinus. Skin abnormalities, neurologic deficits, and infectious complication were evaluated on the basis of clinical information. We evaluated the following MR imaging features: visibility of the tract along the intrathecal course, attachment site of the tract, level of the conus medullaris, shape of the spinal cord, and presence of intradural lesions such as dermoid/epidermoid tumors. RESULTS: A crater covered with pale epithelium was the most common skin lesion in limited dorsal myeloschisis (10/12, 83%). Infectious complications were common in congenital dermal sinus (6/10, 60%), whereas none were found in limited dorsal myeloschisis (P = .003). The following MR imaging findings were significantly different between the 2 groups (P < .05): 1) higher visibility of the intrathecal tract in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), 2) the tract attached to the cord in limited dorsal myeloschisis (12/12, 100%) versus various tract attachments in congenital dermal sinus, 3) dorsal tenting of the cord in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), and 4) the presence of dermoid/epidermoid tumors in congenital dermal sinus (6/10, 60%) versus none in limited dorsal myeloschisis. CONCLUSIONS: Limited dorsal myeloschisis has distinct MR imaging features: a visible intrathecal tract with dorsal tenting of the cord at the tract-cord union. Limited dorsal myeloschisis was not associated with infection and dermoid/epidermoid tumors.

Quantitative Assessment of Neovascularization after Indirect Bypass Surgery: Color-Coded Digital Subtraction Angiography in Pediatric Moyamoya Disease

BACKGROUND AND PURPOSE: For the postoperative follow-up in pediatric patients with Moyamoya disease, it is essential to evaluate the degree of neovascularization status. Our aim was to quantitatively assess the neovascularization status after bypass surgery in pediatric Moyamoya disease by using color-coded digital subtraction angiography. MATERIALS AND METHODS: Time-attenuation intensity curves were generated at ROIs corresponding to surgical flap sites from color-coded DSA images of the common carotid artery, internal carotid artery, and external carotid artery angiograms obtained pre- and postoperatively in 32 children with Moyamoya disease. Time-to-peak and area under the curve values were obtained. Postoperative changes in adjusted time-to-peak (ΔTTP) and ratios of adjusted area under the curve changes (ΔAUC ratio) of common carotid artery, ICA, and external carotid artery angiograms were compared across clinical and angiographic outcome groups. To analyze diagnostic performance, we categorized clinical outcomes into favorable and unfavorable groups. RESULTS: The ΔTTP at the common carotid artery increased among clinical and angiographic outcomes, in that order, with significant differences (P = .003 and .005, respectively). The ΔAUC ratio at the common carotid artery and external carotid artery also increased, in that order, among clinical and angiographic outcomes with a significant difference (all, P = .000). The ΔAUC ratio of ICA showed no significant difference among clinical and angiographic outcomes (P = .418 and .424, respectively). The ΔTTP for the common carotid artery of >1.27 seconds and the ΔAUC ratio of >33.5% for the common carotid artery and 504% for the external carotid artery are revealed as optimal cutoff values between favorable and unfavorable groups. CONCLUSIONS: Postoperative changes in quantitative values obtained with color-coded DSA software showed a significant correlation with outcome scores and can be used as objective parameters for predicting the outcome in pediatric Moyamoya disease, with an additional cutoff value calculated through the receiver operating characteristic curve.

Optimal insertion lengths of right and left internal jugular central venous catheters in children

BackgroundKnowledge of the optimal lengths for central venous catheterization prior to the procedure may lessen the need for repositioning and prevent vascular complications.ObjectiveTo establish the optimal lengths for non-tunneled central venous catheter insertion through the right and left internal jugular veins.Materials and methodsWe included 92 children who received US-guided central venous catheterization via right or left internal jugular veins in intensive care units. The calculated distance between the skin and carina was considered the optimal length for right and left internal jugular venous catheterization. Univariate and multivariate linear regression analyses was used to identify predictors.ResultsAge, height and weight showed significant correlations with optimal insertion lengths for right and left internal jugular vein approaches on univariate analysis, while height was the only significant independent predictor of optimal insertion length.ConclusionThe optimal insertion lengths (cm) suggested by our data are, for the right internal jugular vein 0.034 × height (cm) + 3.173, and for the left 0.072 × height (cm) + 2.113.