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Purpose This study attempts to evaluate the skeletal maturation patterns of the triradiate cartilage (TRC) and the posterior acetabular wall (PA), which can be easily assessed on body computerized tomography (CT). It also examines the effect of gender and age on ossification of both TRC and PA. Materials and Methods This retrospective study included a total of 1324 CT scans for children between the ages of 6 and 16 years. Depending on the extent of ossification in each right or left aspect, determined by the consensus of two observers, the TRC and PA scans were categorized into Grades 4 and 3, respectively. Results The TRC for boys began to ossify at age 10 and closed completely at 14, while the PA for boys started ossification at age 11 and entirely fused at 13. The ages of ossification center appearance and complete fusion in both TRC and PA for girls were exactly two years earlier than boys. The TRC fused within one year after the closure of the PA. Conclusion The appearance and closure of the TRC and PA ossification centers show predictable patterns of development, appearance and merger earlier in females than in males. The suggestion is that development and morphogenesis of both TRC and PA ossification centers can be adequately assessed by using 3-dimensional body CT.

Mitochondrial defects and antimitochondrial cardiolipin (CL) antibodies are frequently detected in autoimmune disease patients. CL from dysregulated mitochondria activates various pattern recognition receptors, such as NLRP3. However, the mechanism by which mitochondrial CL activates APCs as a damage-associated molecular pattern to prime antigen-specific naive T cells, which is crucial for T-cell-dependent anticardiolipin IgG antibody production in autoimmune diseases is unelucidated. Here, we show that CL increases the expression of costimulatory molecules in CD11c+ APCs both in vitro and in vivo. CL activates CD11c+ APCs via TLR2-PI3K-PKN1-AKT/p38MAPK-NF-κB signaling. CD11c+ APCs that have been activated by CL are sufficient to prime H-Y peptide-specific naive CD4+ T cells and OVA-specific naive CD8+ T cells. TLR2 is necessary for anti-CL IgG antibody responses in vivo. Intraperitoneal injection of CL does not activate CD11c+ APCs in CD14 KO mice to the same extent as in wild-type mice. CL binds to CD14 (Kd = 7 × 10-7 M). CD14, but not MD2, plays a role in NF-kB activation by CL, suggesting that CD14+ macrophages contribute to recognizing CL. In summary, CL activates signaling pathways in CD11c+ APCs through a mechanism similar to gram (+) bacteria and plays a crucial role in priming antigen-specific naive T cells.

Sacrococcygeal teratomas are the most common solid tumor in newborn infants. The diagnosis is not difficult in many cases; however, there should be additional information on imaging studies in order to manage those infants properly. Details include histology, morphologic classification, complications such as rupture, bleeding, and mass effects on the adjacent structures. Although imaging features cannot accurately predict the histologic subtypes of the tumors, thorough evaluation of the imaging features can help distinguish malignant tumors from benign tumors. In this article, pathogenesis, histological characteristics, clinical considerations, and morphologic characteristics will be discussed.

Plain radiographs for infants in the neonatal intensive care unit are obtained using the portable X-ray equipment in order to evaluate the neonatal lungs and also to check the position of the tubes and catheters used for monitoring critically-ill neonates. Neonatal respiratory distress is caused by a variety of medical or surgical disease conditions. Clinical information about the gestational week, respiratory symptoms, and any events during delivery is essential for interpretation of the neonatal chest radiographs. Awareness of common chest abnormality in the prematurely born or term babies is also very important for chest evaluation in the newborn. Furthermore, knowledge about complications such as air leaks and bronchopulmonary dysplasia following treatment are required to accurately inform the clinicians. The purpose of this article was to briefly review radiographic findings of chest diseases in newborns that are relatively common in daily practice.

A 37-year-old pregnant woman, para 3, was transferred to our hospital at 30 weeks of gestational age with severe acute abdomen. Ultrasonography of the abdomen confirmed that body fluid around the uterus was retained. Paracentesis was performed and she was diagnosed with hemoperitoneum. Exploratory laparotomy was performed and arterial bleeding from the posterior of left round ligament was found. Spontaneous rupture of uterine vessel in pregnancy is associated with a high rate of mortality. Unfortunately, clinical symptoms of hemoperitoneum are non-specific. For the well-being of the mother and fetus, rapid diagnosis and surgical treatment of hemoperitoneum during pregnancy is needed. Non-invasive and easy-to-implement ultrasonography can be performed periodically to help diagnose quickly.

To establish the risks of developing of hepatic tumours and to investigate their clinical and imaging findings in children with biliary atresia (BA) after Kasai portoenterostomy (Kasai).Among 157 children who had undergone Kasai for BA over an 18 year period, patients who had newly developed hepatic tumours were identified. Patient demographics, clinical features, and imaging findings were retrospectively reviewed.Three male and 10 female patients (mean age 3.9 years) all (8%, of 157) had single hepatic tumours, which were confirmed in 10 explanted and three non-explanted livers. Ten (77%) were benign and three (23%) were malignant. Of the benign hepatic tumours, focal nodular hyperplasia (FNH; n = 6) was the most common, followed by regenerative nodules (n = 3) and adenoma (n = 1). All FNH appeared in young children1 year of age and showed a subcapsular location, bulging contour, and lack of central scar. Malignant tumours included two hepatocellular carcinomas and one cholangiocarcinoma.Hepatic tumours developed in approximately 8% of children with BA after Kasai. Although benign tumours, including FNHs and regenerative nodules, were more common than malignant tumours, screening with alpha-foetoprotein (AFP) levels and regular imaging studies are the mainstay of malignant tumour detection.

Brain ultrasound is widely used for the screening of prematurely born babies. Although the best imaging modality for the central nervous system anomaly is brain MRI, the first imaging study in the post-natal period is brain ultrasonography in most cases. Anomalies could be found incidentally on screening ultrasound, or in those cases already suspected on prenatal ultrasound. In order not to miss congenital structural abnormalities of the brain on screening ultrasound, systematic approaches would be very helpful. The ventricles and sylvian fissures are very important structures to suspect central nervous system anomalies: they are symmetric structures so we should look for any asymmetry or maldevelopment. And then, on sagittal images, the midline structures including the corpus callosum and cerebellar vermis should be observed carefully. Finally, we should look for any abnormality in gyration or cortical development. Skull defect with herniation of intracranial contents, a spectrum of encephalomeningocele, could be alsoidentified on ultrasound. Congenital infections such as cytomegalovirus infectionmay showventriculomegaly and malformation of the cortical development on imaging studies.

This study investigated the self-related and parental factors that influence depression in 204 adolescents from a low social economic status background. The self-related factors were self-trust and emotional regulation, and the parental factors were parental concern and domestic violence. The results showed that girls were more likely to be depressive, but had better emotional regulations and a closer relationship with their parents than boys. There was no significant difference between age groups with respect to parental concern, self-trust and emotion regulation. It was also found by regression analyses that girls` depression was predicted by both emotional regulation and parental concern while boys` depression was predicted only by self-trust. The finding suggested that intervention for boys` depression has to focus more on intrapersonal factors, but should emphasize interpersonal factors for girls.

Leaf aging is a highly regulated developmental process, which is also influenced profoundly by diverse environmental conditions. Accumulating evidence in recent years supports that plant responsiveness to abiotic stress is intimately related with leaf longevity. However, molecular mechanisms underlying the signaling crosstalks and regulatory schemes are yet unknown. In this work, we demonstrate that an abscisic acid (ABA)–responsive NAC transcription factor VND-INTERACTING2 (VNI2) integrates ABA-mediated abiotic stress signals into leaf aging by regulating a subset of COLD-REGULATED (COR) and RESPONSIVE TO DEHYDRATION (RD) genes. The VNI2 gene was induced by high salinity in an ABA-dependent manner. In addition, spatial and temporal expression patterns of the VNI2 gene are correlated with leaf aging and senescence. Accordingly, leaf aging was delayed in transgenic plants overexpressing the VNI2 gene but significantly accelerated in a VNI2-deficient mutant. The VNI2 transcription factor regulates the COR and RD genes by binding directly to their promoters. Notably, transgenic plants overexpressing the COR or RD genes exhibited prolonged leaf longevity. These observations indicate that the VNI2 transcription factor serves as a molecular link that integrates plant responses to environmental stresses into modulation of leaf longevity.

Gibberellic acid (GA) plays a key role in seed germination through coordinate interactions with other growth hormones and external signals. However, the way in which external signals are incorporated into the GA-signaling pathway is largely unknown. Here, we demonstrate that a membrane-bound NAC transcription factor NTL8 mediates the salt regulation of seed germination via the GA pathway, primarily independently of ABA. NTL8 is induced by high salinity. Its expression is also elevated by a GA biosynthetic inhibitor paclabutrazol (PAC), but is repressed by GA. Notably, high salinity greatly represses the GA3 oxidase 1 (GA3ox1) gene, supporting the hypothesis that salt signals inhibit seed germination by repressing GA biosynthesis. Induction of NTL8 and repression of GA3ox1 by high salinity still occur in the ABA-deficient aba3-1 mutant. Accordingly, the germination of a T-DNA insertional ntl8-1 mutant seed is resistant to high salinity and PAC. Interestingly, NTL8 is significantly induced during cold imbibition, but the induction declines quickly in germinating seeds, like RGL2. NTL8 activity is also regulated by controlled proteolytic release of the membrane-bound NTL8 form. Its release from the membranes is activated by PAC and high salinity. Our data support that NTL8 modulates GA-mediated salt signaling in regulating seed germination. This regulatory scheme may provide an adaptative fitness, which delays seed germination under high salinity conditions.

Children are at greater risk of radiation exposure than adults because the rapidly dividing cells of children tend to be more radiosensitive and they have a longer expected life time in which to develop potential radiation injury. Some studies have surveyed computed tomography (CT) radiation doses and several studies have established diagnostic reference levels according to patient age or body size; however, no survey of CT radiation doses with a large number of patients has yet been carried out in South Korea. The aim of the present study was to investigate the radiation dose in pediatric CT examinations performed throughout South Korea. From 512 CT (222 brain CT, 105 chest CT, and 185 abdominopelvic CT) scans that were referred to our tertiary hospital, a dose report sheet was available for retrospective analysis of CT scan protocols and dose, including the volumetric CT dose index (CTDIvol), dose-length product (DLP), effective dose, and size-specific dose estimates (SSDE). At 55.2%, multiphase CT was the most frequently performed protocol for abdominopelvic CT. Tube current modulation was applied most often in abdominopelvic CT and chest CT, accounting for 70.1% and 62.7%, respectively. Regarding the CT dose, the interquartile ranges of the CTDIvol were 11.1 to 22.5 (newborns), 16.6 to 39.1 (≤1 year), 14.6 to 41.7 (2–5 years), 23.5 to 44.1 (6–10 years), and 31.4 to 55.3 (≤15 years) for brain CT; 1.3 to 5.7 (≤1 year), 3.9 to 6.8 (2–5 years), 3.9 to 9.3 (6–10 years), and 7.7 to 13.8 (≤15 years) for chest CT; and 4.0 to 7.5 (≤1 year), 4.2 to 8.9 (2–5 years), 5.7 to 12.4 (6–10 years), and 7.6 to 16.6 (≤15 years) for abdominopelvic CT. The SSDE and CTDIvol were well correlated for patients

The objective of this study was to assess the computed tomography (CT) features of Mycoplasma pneumoniae pneumonia. We retrospectively reviewed CT findings of 16 patients (M:F = 9:7, age range 1-74 years, median 9 years) with serologically proven Mycoplasma pneumoniae pneumonia and with chest CT scan available. Two distinctive patterns of CT features of M. pneumoniae pneumonia were noted between the paediatric (age18 years) and the adult (ageor = 18 years) groups. The pediatric group (n=11) showed lobar or segmental consolidation (100%) with frequent pleural effusion (82%) and regional lymphadenopathy (82%) and mild volume decrease of the involved lobe (73%), while four of the five adult patients showed diffuse and/or multifocal, centrilobular or peribronchovascular areas of ground-glass attenuation (80%) with a lobular distribution, and frequent thickening of interlobular septa (60%) and the bronchial walls (40%) were also detected at high-resolution CT. The CT finding of a lobar or segmental consolidation with a parapneumonic effusion seen in our children with M. pneumoniae pneumonia was similar to that of bacterial lobar pneumonia. In contrast, the CT findings noted in our adult patients consisted of a mixture of a bacterial bronchopneumonia pattern and a viral interstitial pneumonia pattern.

Trichothiodystrophy is a rare neuroectodermal disorder of autosomal recessive inheritance that is characterized by brittle hair, nail dysplasia, ichthyosis, mental retardation, and gonadal failure. We describe a female patient whose cranial MRI revealed almost total lack of myelination in the supratentorial white matter, which is similar to the previously described cases. In addition, there was progressive cerebellar and cerebral atrophy, which has not been well documented in association with trichothiodystrophy.

Objective The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors. Materials and Methods Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain. Results This encephalopathy predominantly affected the bilateral thalami (n=14), pons (n=12), and midbrain (n=10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n=12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n=6) to severe (n=6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015). Conclusion Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

Glioblastoma is relatively uncommon in childhood and maybe difficult to differentiate from other brain tumors such as primitive neuroectodermal tumor, ependymoma, or benign astrocytoma. To describe the characteristic MR features in children with glioblastoma and to evaluate the usefulness of diffusion and perfusion MR imaging and MR spectroscopy in pediatric glioblastoma. MR imaging in 11 children (12 tumors) with biopsy-proven glioblastoma was reviewed retrospectively. In one patient, there was a recurrent glioblastoma. We reviewed CT and MRI imaging for tumor location, density/signal intensity, and enhancement pattern. Routine MR imaging was performed with a 1.5-T scanner. In six patients, diffusion-weighted MR images (DWIs) were obtained with a single-shot spin echo EPI technique with two gradient steps, and apparent diffusion coefficients (ADCs) were calculated. Using the gradient EPI technique, perfusion-weighted MR images (PWIs) were obtained in four patients from the data of dynamic MR images. The maximum relative cerebral blood volume (rCBV) ratio was calculated between the tumor and contralateral white matter in two cases. In three patients, proton MR spectroscopy was performed using a single voxel technique with either STEAM or PRESS sequences. The locations of the tumor were the thalamus and basal ganglia (n=8), deep white matter (n=3), and brain stem (n=1). Intratumoral hemorrhage was seen in four tumors. The tumors showed high-signal intensity or DWIs, having a wide range of ADC values of 0.53–1.30 (mean ±SD=1.011±0.29). The maximum rCBV ratios of glioblastoma were 10.2 and 8.5 in two cases. MR spectroscopy showed decreased N-acetylaspartate (NAA) and increased choline in three cases. The MR findings of glioblastoma in children were: a diffusely infiltrative mass with hemorrhage involving the deep cerebral white matter, thalami, and basal ganglia. Diffusion/perfusion MR imaging and MR spectroscopy are very helpful in diagnosing glioblastoma, determining the biopsy site, and evaluating tumor recurrence.

Numerous viruses, including influenza virus, measles virus, Hantavirus, adenovirus, herpesviruses, varicella-zoster virus, cytomegalovirus, and Epstein-Barr virus, can cause lower respiratory tract infection in adults. Viral pneumonia in adults can be classified into two clinical groups: so-called atypical pneumonia in otherwise healthy hosts and viral pneumonia in immunocompromised hosts. Influenza virus types A and B cause most cases of viral pneumonia in immunocompetent adults. Immunocompromised hosts are susceptible to pneumonias caused by cytomegalovirus, herpesviruses, measles virus, and adenovirus. The radiographic findings, which consist mainly of patchy or diffuse ground-glass opacity with or without consolidation and reticular areas of increased opacity, are variable and overlapping. Computed tomographic findings, which are also overlapping, consist of poorly defined centrilobular nodules, ground-glass attenuation with a lobular distribution, segmental consolidation, or diffuse ground-glass attenuation with thickened interlobular septa. The radiologic findings reflect the variable extents of the histopathologic features: diffuse alveolar damage (intraalveolar edema, fibrin, and variable cellular infiltrates with a hyaline membrane), intraalveolar hemorrhage, and interstitial (intrapulmonary or airway) inflammatory cell infiltration. Clinical information such as patient age, immune status, community outbreaks, symptom onset and duration, and presence of a rash remain important aids in diagnosis of viral causes.

Purpose The aim of this study was to investigate cortical thickness and gray matter volume abnormalities in benign childhood epilepsy with centrotemporal spikes (BCECTS). We additionally assessed the effects of comorbid attention-deficit/hyperactivity (ADHD) on these abnormalities. Methods Surface and volumetric MR imaging data of children with newly diagnosed BCECTS ( n =20, 14 males) and age-matched healthy controls ( n =20) were analyzed using FreeSurfer (version 5.3.0, https://surfer.nmr.mgh.harvard.edu). An additional comparison was performed between BCECTS children with and without ADHD (each, n =8). A group comparison was carried out using an analysis of covariance with a value of significance set as p p Results Children with BCECTS had significantly thicker right superior frontal, superior temporal, middle temporal, and left pars triangularis cortices. Voxel-based morphometric analysis revealed significantly larger cortical gray matter volumes of the right precuneus, left orbitofrontal, pars orbitalis, precentral gyri, and bilateral putamen and the amygdala of children with BCECTS compared to healthy controls. BCECTS patients with ADHD had significantly thicker left caudal anterior and posterior cingulate gyri and a significantly larger left pars opercularis gyral volume compared to BCECTS patients without ADHD. Conclusion Children with BCECTS have thicker or larger gray matters in the corticostriatal circuitry at the onset of epilepsy. Comorbid ADHD is also associated with structural aberrations. These findings suggest structural disruptions of the brain network are associated with specific developmental electro-clinical syndromes.

Multiple synostosis syndrome (SYNS) is an autosomal dominant skeletal disorder characterized by facial dysmorphism, progressive fusion of multiple joints, and conductive hearing loss. Currently, three genes, NOG, GDF5, and FGF9, have been identified as causative of SYNS. However, due to the phenotypic and genotypic heterogeneity of SYNS, as well as its extreme rarity, it is difficult to diagnose, either by clinical or genetic means. Here, we describe three unrelated Korean families with three different, novel NOG mutations. These mutations are located on the region of the protein critical for appropriate NOG function. The patients shared the general features of SYNS, but the phenotype was expressed differently both within and between the families. In addition, this phenotypic diversity was irrespective of the age of patients, indicating the importance of surveillance for the full spectrum of SYNS in each affected patient. Our report expands understanding of this rare condition from both clinical and genetic perspectives.

Background The aberrant cervical thymus is an uncommon entity to be considered in the differential diagnosis of neck masses in children, so a preoperative diagnosis has rarely been made. Objective The purpose of this study was to assess the ultrasound features of aberrant cervical thymus and determine if recognition of the previously described unique intrathymic echo pattern allows us to make a specific diagnosis. Materials and methods We present four patients with an aberrant cervical thymus and one patient with a cervical thymic cyst. All five patients had ultrasound, which was evaluated with special attention to the detailed echo pattern of the mass. In two patients, the mass was surgically removed. Four patients had MRI, which was evaluated. Medical records were reviewed. Results In all cases, the mass was painless and nontender. All occurred in the expected path of the thymopharyngeal duct. In four patients, ultrasound demonstrated multiple echogenic linear structures and foci previously described as characteristic echo pattern of normal thymic tissue. In one patient, ultrasound showed a large cystic mass and echogenic solid component superiorly with a characteristic echo pattern of normal thymus. Conclusions The ultrasound appearance of thymic tissue is unique, allowing a specific diagnosis of aberrant cervical thymus. Biopsy or other imaging is probably not indicated when it presents with typical clinical features.

Background. Various mediastinal interfaces and lines are well known in adults, but not fully understood in children. Objective. To review the mediastinal interfaces and lines on plain radiographs in the paediatric age group with CT correlation. Materials and methods. Soft copies of 195 sets of CT examinations and concurrent chest radiographs in 180 paediatric patients (age 1 month to 15 years) were retrospectively reviewed. The frequency of visualisation and the anatomical basis of the mediastinal interfaces and lines were assessed. The evaluated mediastinal interfaces and lines were superior vena cava interface, descending aorta interface, left paraspinal interface, azygoesophageal recess interface, right paratracheal stripe, anterior junction line and posterior junction line. Results. Chest radiographs showed the SVC interface in 161, the descending aorta interface in 155, the left paraspinal interface in 98, the azygoesophageal recess in 94, the right paratracheal stripe in 53, the anterior junction line in 15 and the posterior junction line in 10. Non-visualisation of mediastinal interfaces and lines on plain radiographs was explained by normal anatomical difference compared with the adult and underlying intrathoracic abnormalities when they were correlated with CT. The frequency of visualisation of the SVC interface, descending aortic interface, left paraspinal interface and azygoesophageal recess fluctuated with age, while the frequency of the right paratracheal stripe, anterior junction line and posterior junction line increased with age. Conclusions. Awareness of the frequency of visualisation and the anatomical basis of the mediastinal interfaces and lines in paediatric patients may be helpful for interpretation of chest radiographs.

Background. Rhabdoid tumour of the kidney (RTK) is a rare tumour, but it is the most aggressive malignant neoplasm of the kidney in children. Objective. To analyse the radiological findings of RTK in children. Materials and methods. The clinical and radiological findings in seven children (age range 6 months to 4.7 years; median 18 months) with pathologically proven RTK were retrospectively reviewed. We analysed tumour size, tumour location, tumour margin, subcapsular haematoma, tumour necrosis, haemorrhage, calcification and lymphadenopathy. Results. Tumour size varied from 5 to 12 cm. Four tumours were located mainly in the central portion of the kidney, while three tumours were mainly sited peripherally. The margins of the tumour were ill-defined in four (57 %) of seven cases, a lobulated tumour surface was depicted in all seven (100 %), subcapsular haematoma was present in four (57 %), tumour necrosis or haemorrhage in seven (100 %), calcifications in three (43 %) and retroperitoneal lymphadenopathy in four (57 %). Conclusions. Imaging findings of RTK are subcapsular haematoma, a lobulated surface of the tumour, calcification and tumour necrosis or haemorrhage.

Purpose: The purpose of this work was to compare the use of MRI and [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) in the diagnosis of focal cortical dysplasia (FCD). Method: Nineteen patients with surgically proven FCD were analyzed retrospectively. MRI was performed in all patients, and FDG-PET was performed in 17 patients. We compared the MR and FDG-PET findings of FCD according to the histologic findings that were classified into three grades. Results: Four cases were classified as Grade I, 4 cases as Grade II, and 11 cases as Grade III FCD. The lesions were detected on MRI in 9 (82%) of the 11 patients with Grade III FCD and in only 1 (13%) of the 8 patients with Grade I and II FCD. Cortical hypometabolism of the lesion was revealed on FDG-PET in 6 (86%) of the 7 patients with Grade I and II FCD and in 9 (90%) of the 10 patients with Grade III FCD. The extent of the cortical abnormality was larger on FDG-PET than on MRI in 11 (65%) of the 17 patients. Conclusion: FDG-PET is more useful in delineating the cortical abnormality in patients with mild degrees of FCD. The extent of the lesion was larger or similar on FDG-PET compared with that of the MRI.

To analyze the findings of intramuscular vascular malformations of an extremity on MR imaging and to correlate these findings with histopathologic examination.The findings on MR imaging and the medical records of 14 patients with an intramuscular vascular malformation of the extremity were retrospectively studied. All patients underwent surgical excision. Diagnoses were based on the results of pathologic examination. Findings on MR imaging were noted and correlated with the histopathologic findings.Intramuscular vascular malformations of an extremity showed multi-septate, honeycomb, or mixed appearance on MR imaging. Multi-septate areas correlated with dilated and communicating vascular spaces with flattened endothelium. Honeycomb areas corresponded to vascular spaces with inconspicuous small lumina and thickened vascular walls. Areas of increased signal intensity on T2-weighted images were found in all intramuscular vascular malformations. Infiltrative margins were more commonly seen in intramuscular lymphaticovenous malformations. Adherence to neurovascular structures and orientation of the lesion along the long axis of the affected muscle were more commonly seen in intramuscular venous malformations.Intramuscular vascular malformations showed either a multi-septate, honeycomb, or mixed appearance, reflecting the size of the vascular spaces and the thickness of the smooth muscles of the vessel walls. Prediction of the subtype of an intramuscular vascular malformation of an extremity on MR imaging seems to be difficult, although there are associated findings that may be helpful in the differential diagnosis of each subtype.

Purpose: To assess the feasibility and effectiveness of transperineal ultrasonography (TPUS) for the evaluation of perianal Crohn’s disease (PCD) in pediatric patients. Methods: Between September 2010 and August 2013, 64 TPUS examinations were performed in 43 patients (34 males and 9 females; mean age ± standard deviation, 13.3 ± 2.4 years; age range, 6–17 years) to evaluate the PCD. The pain severity, location and activity of perianal fistula, the presence of an abscess, and anal canal hyperemia was retrospectively evaluated. Spearman’s rank correlation analysis was performed to assess the relationship between the severity of the pain and the fistula activity, the presence of an abscess, and anal canal hyperemia. Results: All examinations were successfully performed Thirty-nine examinations (60.9%) were performed without any pain experienced by the patient, 19 examinations (29.7%) with mild pain, five examinations (7.8%) with moderate pain, and one examination (1.6%) with severe pain. The pain severity was correlated with the fistula activity (P

We present two newborn infants with hypernatremic dehydration with central nervous system (CNS) involvement. Both patients showed similar imaging findings, demonstrating generalized brain parenchymal abnormality and multifocal areas of hemorrhage or hemorrhagic infarction. These findings are compatible with previously described CNS pathologic findings in hypernatremia.

Many benign and malignant bone tumors and tumor-like lesions affect children. Plain radiography remains the most important screening tool, and in many cases benign bony lesions do not require further imaging study. MRI is necessary for evaluating the malignant bone tumors with the detailed information of tumor extent, signal characteristics, adjacent structures with neurovascular bundles, and separate lesions. MRI is also very useful for follow-up examinations in the oncologic patients in evaluating tumor recurrence, metastasis, and changes related to the treatment. Overview of benign and malignant bone tumors which are relatively commonly encountered in children is given with illustrative cases.

Infectious and traumatic diseases in the head and neck regions affect many children. Neck lumps in children are more likely benign, and reactive or inflammatory cervical lymphadenopathy is much more common than neoplastic adenopathy. Ultrasound is the first choice of imaging modality for infectious or inflammatory diseases of the neck, whereas plain radiography plays a major role for screening in the traumatic cases. CT, and sometimes MRI, is valuable for more detailed evaluation of the head and neck diseases. In cases of infection involving a specific location in the child’s neck, the possibility of underlying congenital anomalies such as the branchial arch anomaly should be kept in mind for accurate diagnosis.

The most common site of metastasis in gestational trophoblastic disease (GTD) is the lung. To the best of our knowledge, arteriovenous malformations (AVMs) associated with pulmonary metastatic lesions are extremely rare in patients with GTD. Here, we report a case of a nineteen-year-old woman who presented with pulmonary AVMs which developed in the areas of pulmonary metastases, complicated by recent hemorrhage.

Purpose To investigate structural brain changes and their relationship with cognitive function by determining the alterations of white matter integrity and changes in hippocampal volume that occur during chronic obstructive pulmonary disease (COPD). Materials and Methods Diffusion tensor images and 3D-T1 MR images were acquired in 13 male nonhypoxemic COPD patients and 13 age- and gender-matched healthy controls. Global probabilistic tractography was used to assess a total of 18 major tracts. We examined the association between the hippocampal volume and diffusivity parameters of white matter tracts. Results A significant difference in diffusion parameters between groups was identified for 11 white matter bundles (p < 0.05). No association was demonstrated between the normalized hippocampus volume and diffusion parameters among the participants. Significant associations between Korean version of the Mini-Mental State Examination (K-MMSE) scores and diffusivities were found for six tracts (p < 0.05). Of note, the mean diffusivity (MD) and redial diffusivity (RD) of the left superior longitudinal fasciculus parietal segment showed a significant negative correlation with the K-MMSE score (MD: r = −0.623, p = 0.001; RD: r = −0.408, p = 0.048). Conclusion Altered white matter integrity was demonstrated across regional white matter bundles, including the superior longitudinal fasciculus, in COPD patients, and it was related to cognitive function. Changes in the volume of the hippocampus should be investigated in further studies.

We describe a case of cytomegalovirus (CMV) ventriculoencephalitis and retinitis with compartmentalization of antiviral drug-resistant CMV across the blood-brain barrier after T cell-depleted haploidentical peripheral blood stem cell transplantation.

Trilateral retinoblastoma is the association of bilateral retinoblastomas and an intracranial primitive neuroectodermal tumour. Bilateral retinoblastomas with a pineal tumour are typical manifestations of this association. However, there may be variants, including unilateral retinoblastoma with intracranial tumour. The intracranial tumour can occur in the suprasellar or parasellar regions even before presentation of the retinal mass. We report a female infant with a suprasellar retinoblastoma and unilateral intraocular retinoblastoma.

Mesenteric lymphadenopathy has been rarely reported in pediatric patients with Gaucher disease, developing despite the enzyme replacement therapy. The clinical implication of this condition is undetermined, with no consensus on treatment strategies. However, this condition can reflect the progression of Gaucher disease. Moreover, it can be accompanied by the serious complication, protein-losing enteropathy. Our experience underlines the importance of careful monitoring and early intervention for mesenteric lymphadenopathy, especially in pediatric patients with neuronopathic Gaucher disease.

Aim The aim was to review the clinical and imaging features of hypervascular hepatic nodule (HHN) in childhood cancer survivors. Materials and methods We retrospectively reviewed 11 pediatric patients (female:male, 7:4; age range, 4.0–12.3 years) who had HHNs detected by surveillance computed tomography (CT) after treatment of a malignant solid tumor and subsequently followed by serial imaging without evidence of recurrent malignancy. The lesions were analyzed in terms of number, size, location, CT and ultrasonographic (US) features, and changes in background liver. In addition, clinical features were investigated along with follow-up changes of HHNs by imaging monitoring. Results Time between initial diagnosis of malignancy and HHN occurrence ranged from 3.2 to 8.5 years (median, 5.8 years). Ten patients had received high-dose chemotherapy and autologous stem cell transplantation for advanced neuroblastoma. A total of 22 nodules were detected, being multiple in six patients and measuring 0.5–3.0 cm in size. At sequential postcontrast CT, nodules demonstrated moderate to strong enhancement during the earlier phase and were isoattenuated during the later phase. On US, they appeared as hypo- or isoechoic lesions. During follow-up, 11 nodules (50%) regressed, 6 (27%) progressed, and 5 (23%) remained stable. Additional HHNs were noted in four patients during follow-up. Conclusion Childhood cancer survivors are at risk of developing HHNs, which are often multiple and small, years after completing chemotherapy. They are nonaggressive and tend to have a benign course, making conservative management reasonable.

Aim To evaluate the clinical outcome and the survival benefits of transarterial chemoembolization (TACE) for unresectable intrahepatic cholangiocarcinoma (ICC) compared with supportive therapy. Materials and methods From January 1996 to April 2009, a total of 155 patients with unresectable ICC met the entry criteria and underwent TACE (72 patients) or supportive treatment (83 patients). Their survival was the primary end point. Results The baseline patients and tumour characteristics were well-balanced in the two groups. The median number of sessions per patient was 2.5 (range 1–17 sessions) in the TACE group. After TACE, the incidence of significant (≥grade 3) haematological and non-haematological toxicities was 13 and 24%, respectively, and no patients died within 30 days following TACE. The objective tumour regression (≥partial response) was achieved in 23% of the patients in the TACE group. The Kaplan–Meier survival analysis showed that the survival period was significantly longer in the TACE group (median 12.2 months) than in the symptomatic treatment (median 3.3 months) group ( p Conclusions TACE is safe and offers greater survival benefits than supportive treatment for the palliative treatment of unresectable ICC.

To evaluate the safety and efficacy of transcatheter arterial chemo-lipiodol infusion (TACL) in high-risk patients with unresectable hepatocellular carcinoma (HCC).From January 2005 to April 2009, 96 patients underwent TACL. All patients had diffuse, infiltrative or multifocal tumours. Twenty-nine (30%) patients had an increased serum bilirubin level (or=2mg/dl), and 75 patients (78%) had a low serum albumin level (3.5mg/dl). The Child-Pugh (CP) score was 9 or more in 13 (14%) patients. Sixty-five patients (68%) had major portal vein occlusion. Sixteen patients (17%) had biliary dilatation.TACL was technically successful in all patients. After TACL, 18 (19%) of the 96 patients showed tumour response using computed tomography (CT) criteria. The 30 day mortality and morbidity rates were 1 and 2%, respectively. The median survival period was 8.6 months, and the overall 6 month, 1, 2, and 3 year survival rates were 59, 44, 26, and 15%, respectively. Portal vein occlusion (p0.001) was the only significant risk factor associated with the length of the survival period after TACL, whereas the CP score (p=0.498), serum bilirubin level (p=0.153), serum albumin level (p=0.399), and biliary obstruction (p=0.636) had no significant effect.TACL can be performed safely in high risk HCC patients resulting in a median survival rate of 8.6 months in the present series.

Leaf senescence is a highly regulated genetic process that constitutes the last stage of plant development and provides adaptive fitness by relocating metabolites from senescing leaves to reproducing seeds. Characterization of various senescence mutants, mostly in Arabidopsis, and genome-wide analyses of gene expression, have identified a wide array of regulatory components, including transcription factors and enzymes as well as signaling molecules mediating growth hormones and environmental stress responses. In this work we demonstrate that a membrane-associated NAC transcription factor, NTL9, mediates osmotic stress signaling in leaf senescence. The NTL9 gene is induced by osmotic stress. Furthermore, activation of the dormant, membrane-associated NTL9 is elevated under the same conditions. A series of senescence-associated genes (SAGs) were upregulated in transgenic plants overexpressing an activated form of NTL9, and some of them were slightly but reproducibly downregulated in a T-DNA insertional NTL9 knockout mutant. These observations indicate that NTL9 mediates osmotic stress responses that affect leaf senescence, providing a genetic link between intrinsic genetic programs and external signals in the control of leaf senescence.

Multifocal micronodular pneumocyte hyperplasia (MMPH) is a relatively rare pulmonary disorder that can be associated with tuberous sclerosis complex (TSC). It has been rarely reported in children or adolescents. MMPH is a hamartomatous process of the lung with multiple small nodules, composed of type II pneumocytes. Plain radiography and chest CT in MMPH may demonstrate numerous small nodules measuring 1–10 mm in diameters, distributed randomly throughout both lungs. If MMPH is an initial presentation of TSC, and unless we are familiar with this lung manifestation of TSC, radiologic findings can mimic miliary tuberculosis or metastatic disease. We report a teenage girl with TSC and histologically confirmed MMPH which mimicked miliary tuberculosis at the initial presentation.

Pediatric pancreatitis is not uncommon and results in considerable morbidity and mortality in the affected children. Unlike adults, pediatric pancreatitis is more frequently associated with underlying structural abnormalities, trauma, and drugs rather than an idiopathic etiology. Magnetic resonance cholangiopancreatography (MRCP) is a good imaging modality for evaluating pancreatitis and determining etiology without exposure to radiation. This article focuses on MRCP findings associated with various causes of pancreatitis in children, particularly structural abnormalities of the pancreaticobiliary system, as well as describing the feasibility, limitations, and solutions associated with pediatric MRCP.

Controlled proteolytic cleavage of membrane-associated transcription factors (MTFs) is an intriguing activation strategy that ensures rapid transcriptional responses to incoming stimuli. Several MTFs are known to regulate diverse cellular functions in prokaryotes, yeast, and animals. In Arabidopsis, a few NAC MTFs mediate either cytokinin signaling during cell division or endoplasmic reticulum (ER) stress responses. Through genome-wide analysis, it was found that at least 13 members of the NAC family in Arabidopsis contain strong alpha-helical transmembrane motifs (TMs) in their C-terminal regions and are predicted to be membrane-associated. Interestingly, most of the putative NAC MTF genes are up-regulated by stress conditions, suggesting that they may be involved in stress responses. Notably, transgenic studies revealed that membrane release is essential for the function of NAC MTFs. Transgenic plants overexpressing partial-size NAC constructs devoid of the TMs, but not those overexpressing full-size constructs, showed distinct phenotypic changes, including dwarfed growth and delayed flowering. The rice genome also contains more than six NAC MTFs. Furthermore, the presence of numerous MTFs is predicted in the whole transcription factors in plants. We thus propose that proteolytic activation of MTFs is a genome-wide mechanism regulating plant genomes.

Tracheobronchial disruption is an uncommon injury associated with blunt chest trauma. We report CT features and pathologic findings of two pediatric cases in which a bronchial injury was unnoticed initially but was diagnosed later by appearance of delayed bronchostenosis with distal atelectasis after blunt chest trauma in recent motor vehicle accidents. Pathologically, obliteration of the bronchial lumen was caused by dense fibrous overgrowth and granulation tissue.

1055 isseminated cryptococcosis commonly occurs in immunocompromised patients, particularly in HIV-infected patients, and rarely in immunocompetent patients. In patients with disseminated disease, meningitis is the form most frequently observed [1]; disseminated disease affecting predominantly the lung and abdominal organs is rare [2]. To our knowledge, no report has described the CT features of disseminated cryptococcosis concomitantly involving the thorax and abdomen. We describe two cases of disseminated cryptococcosis that primarily affected the lung, lymph nodes, liver, and spleen in patients without HIV infection.

Correction: Pediatr Gastroenterol Hepatol Nutr 2015;18:73-84. http://dx.doi.org/10.5223/pghn.2015.18.2.73 This article was originally published on June 30, 2015. The following errors were discovered by the authors after publication: missing references and missing words. Firstly, the following new references have been added. 7. Kandula L, Lowe ME. Etiology and outcome of acute pancreatitis in infants and toddlers. J Pediatr 2008;152:106-10. 18. Choi IY, Jin SH, Choi KD, Kim KM. A Case of autoimmune chronic pancreatitis in a child. Korean J Pediatr Gastroenterol Nutr 2007;10:215-20. Accordingly, reference numbers "[7-16]" should be corrected as "[8-17]" and "[17-39]" should be corrected as "[19-41]" in main text and reference list, in order to maintain the sequential order of in-text citations. Secondly, the sentence on page 73 "It has been reported that the structural abnormalities of the pancreaticobiliary system, including biliary stones and sludge, are the leading causes of recurrent pediatric pancreatitis [6]." has been replaced with: "It has been reported that biliary diseases including the structural abnormalities of the pancreaticobiliary system are the leading causes of recurrent pediatric pancreatitis even for infants and toddlers [5-7]." The sentence on page 77 "Autoimmune pancreatitis is increasingly encountered in adults, but a few cases have been also reported in children and adolescents [16]." has been replaced with: "Autoimmune pancreatitis is increasingly encountered in adults, but a few cases have been also reported in children and adolescents [17,18]."

To compare contrast material-enhanced T1-weighted and fluid-attenuated inversion recovery (FLAIR) magnetic resonance (MR) images with or without gadolinium in depicting the leptomeningeal ivy sign in children with moyamoya disease.Twenty-nine sets of FLAIR and postcontrast T1-weighted MR images were available in 19 consecutive children with primary moyamoya disease confirmed with conventional and MR angiography. Contrast-enhanced FLAIR MR images also were available in 15 sets. Two pediatric radiologists reviewed FLAIR and postcontrast T1-weighted images in separate sessions for the leptomeningeal ivy sign and assigned a rating of "present," "absent," or "equivocal" by consensus. Unenhanced and contrast-enhanced FLAIR MR images were compared side by side to determine which better depicted leptomeningeal high signal intensities.Postcontrast T1-weighted MR images revealed the leptomeningeal ivy sign in 40 hemispheres (frequency of visualization, 71% [40 of 56 hemispheres]), whereas unenhanced FLAIR MR images depicted it in 26 hemispheres (frequency of visualization, 46% [26 of 56 hemispheres]). An equivocal rating was given in 21 hemispheres versus in 11 on FLAIR and postcontrast T1-weighted images, respectively. FLAIR and postcontrast T1-weighted images agreed in 40 hemispheres. There was no case with a positive rating on FLAIR images when postcontrast T1-weighted images were negative. Unenhanced FLAIR MR imaging was superior to contrast-enhanced FLAIR imaging in seven hemispheres, whereas enhanced FLAIR was better in four of 28 hemispheres. In the remaining 17, findings with each sequence were similar.Contrast-enhanced T1-weighted images are better than FLAIR images for depicting the leptomeningeal ivy sign in moyamoya disease.

Background. Proper recognition, classification and assessment of the tracheobronchial anatomy are essential for surgical management planning in left pulmonary artery sling (LPAS). Objective. To demonstrate the various imaging modalities in diagnosing and assessing anatomical relationships with tracheobronchial tree in LPAS. Materials and methods. Seven patients (three infants, three children, one adult) with LPAS were evaluated. The confirmative imaging modalities included electron-beam tomography (EBT, n = 2), MRI (n = 1) and helical CT (n = 4). Other imaging modalities included plain chest radiographs (n = 7), barium oesophagogram (n = 2), echocardiography (n = 3), cardiac and pulmonary angiography (n = 2) and bronchoscopy (n = 5). Results. There were two cases of type IA, one case of type IIA and four cases of type IIB. All five cases of type II had a long segment of tracheobronchial stenosis. A long segment of thoracic tracheal stenosis was unusually associated in one case of type IA. All confirmative modalities were sufficient for diagnosing LPAS and assessing the tracheobronchial tree anomaly. However, helical CT could not obviate angiography due to associated congenital cardiac defects. Conclusions. Sectional imaging modalities of EBT, MRI and helical CT were all sufficient for diagnosing and assessing the anatomical relationships of the tracheobronchial tree in LPAS.

AIM: The objective of this study was to analyse the MR imaging findings of infantile fibromatosis of childhood and to correlate them with histopathological features. MATERIALS AND METHODS: Seven patients with histologically proven infantile fibromatosis were included in this study. The findings on MR images were retrospectively evaluated and then correlated with the pathological features. Findings on MR imaging evaluated included signal intensity, extent of hyperintense area on T2-weighted images, margins of the lesion, the degree and pattern of enhancement and the presence of fatty tissue. Pathological features evaluated included cellularity, collagenization, and myxoid change. A five point scale was used for the evaluation of the extent of hyperintense area on MR imaging, and each of pathological features. RESULTS: On T1-weighted images, the lesions were iso-intense in two patients; iso- and hypointense in three; and iso-, hypo- and hyperintense in two. On T2-weighted images, iso-, hypo- and hyperintense areas were mixed in all patients, the hyperintense area being the largest portion of the lesion. The margins of the lesions were infiltrative in four patients (57%), smooth in two (29%) and mixed in one (14%). Enhancement was marked in five patients (72%) and diffuse in five (71%). Regardless of the hyperintense signal intensity on T2-weighted images, the grades of each pathologic feature were variable. CONCLUSION: Infantile fibromatosis on MR imaging causes an enhancing mass, that is largely hyperintense on T2-weighted images. Areas of high signal intensity on T2-weighted images corresponded to variable grades of cellularity, collagenization, or myxoid change.Ahn, J. M. (2000). Clinical Radiology 55 , 19–24.

Our purpose was to evaluate the usefulness of MR angiography in revealing moyamoya disease before and after encephaloduroarteriosynangiosis.Twenty-six patients (51 hemispheres) with angiographically confirmed moyamoya disease who underwent encephaloduroarteriosynangiosis were included in the study. Findings on preoperative MR angiography were compared with those on conventional angiography. Postoperative neurologic status was categorized as poor, fair, good, or excellent. Postoperative MR angiography was examined for the appearance of the superficial temporal artery, changes in moyamoya vessels, and transdural collateral vessels into the middle cerebral artery territory.Preoperative MR angiography revealed moyamoya disease in all patients (diagnostic accuracy, 100%). MR angiography correctly depicted the degree of internal carotid artery stenosis in 37 arteries (73%), moyamoya vessels in 33 hemispheres (65%), and the degree of stenosis in the middle, anterior, and posterior cerebral arteries in 125 (82%) of 153 arteries. After surgery, 39 hemispheres showed an excellent outcome, eight showed a good outcome, two a fair outcome, and two a poor outcome. On postoperative MR angiography, vascular supply to the middle cerebral artery territory via transdural collateral vessels increased in 28 hemispheres (55%) and decreased in four (8%). The size of the superficial temporal artery increased in 41 (80%) of 51 hemispheres. The extent of moyamoya vessels decreased in 27 hemispheres (53%) after surgery.MR angiography can show the changes in the superficial temporal artery and development of transdural collateral vessels after encephaloduroarteriosynangiosis. Because MR angiography is noninvasive, it is valuable for evaluating postoperative changes.