A Comparative Analysis of Clinical Screening Test and Language Specific Test in Language Delay Children.

The aim of this study was to investigate the usefulness of a clinical screening test [the Korean Infant and Child Developmental Test (KICDT)] compared to language specific tests: the sequenced language scale for infant (SELSI) and the Preschool Receptive-Expressive language Scale (PRES) in children with delayed language development. A retrospective chart review was conducted on 615 children who visited the Department of Pediatrics at Chonbuk National University Hospital from January 2013 to December 2016. All patients were evaluated with KICDT as a clinical screening test and SELSI or PRES as a language specific test. Language Developmental Quotients (LDQs) from the KICDT were compared with the Receptive Language Quotient (RLQ) and expressive language quotient (ELQ) from the SELSI or PRES. The sensitivity, specificity and predictive values of LDQ of KICDT were calculated by comparing with SELSI/PRES. Language DQs from the KICDT were significantly correlated with the RLQ (r=0.706), ELQ (r=0.768), and total language quotient (TLQ) (r=0.766) from the SELSI/PRES (p<0.05). In cross tabulation, the patients belonging to the retardation groups in both KICDT and SELSI/PRES were 417 (67.8%). Otherwise, patients belonging to the normal group in KICDT but not in SELSI/PRES were 151 (24.6%). Sensitivity and specificity of LDQ of KICDT relative to SELSI/PRES were 72.3% and 92.2% respectively (p<0.05). Our data suggests that clinical screening tests alone, not cumbersome language specific tests, can determine language developmental delays in children.

Acute blindness as a presenting sign of left atrial myxoma in a pediatric patient: A case report and literature review.

RATIONALE: Central retinal artery occlusion (CRAO) due to cardiac myxoma primarily occurs in elderly individuals. Early detection and surgical resection of myxoma are extremely important because CRAO causes complete blindness in most cases. However, due to the extremely low incidence of CRAO caused by cardiac myxoma in the pediatric age group, such condition is rarely reported. PATIENT CONCERNS: A 16-year-old female patient visited our hospital due to sudden onset of vision loss in the left eye, dysarthria, and right-sided hemiplegia. DIAGNOSES: She was diagnosed with CRAO via fundoscopy. Results showed a cherry-red spot, indicating CRAO. Brain magnetic resonance imaging (MRI) revealed multifocal diffusion-restricted foci, particularly in the left frontal lobe. Echocardiography revealed a left atrial mass measuring 4.21 cm × 2.25 cm. The mass was attached to the interseptum and moved along the inflow of the mitral valve. Cardiac computed tomography (CT) revealed an enhanced mass measuring 3 cm × 2.2 cm × 3 cm and with irregular margin on the anterior wall of the left atrium and the border of the fossa ovalis. INTERVENTIONS: The patient underwent surgical excision under general anesthesia. Intraoperative finding showed a huge, jelly-like, and extremely friable mass. Pathological examination confirmed myxoma. OUTCOMES: During a follow-up of 2 years after diagnosis, she did not present with other neurological deficits and no residual mass was observed on echocardiography. However, visual impairment of the left eye persisted. LESSONS: Most patients with CRAO may present with other mild symptoms that are often be neglected before CRAO development. We recommend that patients who present with frequent syncopal attack or symptoms of transient ischemic attack should undergo echocardiography.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea.

BACKGROUND AND OBJECTIVES: We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. METHODS: Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. RESULTS: Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p<0.001). We investigated the response rate to infliximab and the incidence of significant CAA (z-score >5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p<0.001). CONCLUSIONS: This study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Effects of low-dose topiramate on language function in children with migraine.

PURPOSE: This study aimed to verify the safety of low-dose topiramate on language development in pediatric patients with migraine. METHODS: Thirty newly diagnosed pediatric patients with migraine who needed topiramate were enrolled and assessed twice with standard language tests, including the Test of Language Problem Solving Abilities (TOPs), Receptive and Expressive Vocabulary Test, Urimal Test of Articulation and Phonology, and computerized speech laboratory analysis. Data were collected before treatment, and topiramate as monotherapy was sustained for at least 3 months. The mean follow-up period was 4.3±2.7 months. The mean topiramate dosage was 0.9 mg/kg/day. RESULTS: The patient's mean age was 144.1±42.3 months (male-to-female ratio, 9:21). The values of all the language parameters of the TOPs were not changed significantly after the topiramate treatment as follows: Determine cause, from 15.0±4.4 to 15.4±4.8 (P>0.05); making inference, from 17.6±5.6 to 17.5±6.6 (P>0.05); predicting, from 11.5±4.5 to 12.3±4.0 (P>0.05); and total TOPs score, from 44.1± 13.4 to 45.3±13.6 (P>0.05). The total mean length of utterance in words during the test decreased from 44.1±13.4 to 45.3±13.6 (P<0.05). The Receptive and Expressive Vocabulary Test results decreased from 97.7±22.1 to 96.3±19.9 months, and from 81.8±23.4 to 82.3±25.4 months, respectively (P>0.05). In the articulation and phonology validation in both groups, speech pitch and energy were not significant, and all the vowel test results showed no other significant values. CONCLUSION: No significant difference was found in the language-speaking ability between the patients; however, the number of vocabularies used decreased. Therefore, topiramate should be used cautiously for children with migraine.

Comparison of Percutaneous Endoscopic Gastrostomy and Surgical Gastrostomy in Severely Handicapped Children.

PURPOSE: Gastrostomy is commonly used procedures to provide enteral nutrition support for severely handicapped patients. This study aimed to identify and compare outcomes and complications associated with percutaneous endoscopic gastrostomy (PEG) and surgical gastrostomy (SG). METHODS: A retrospective chart review of 51 patients who received gastrostomy in a single tertiary hospital from January 2000 to May 2016 was performed. We analyzed the patients and the complications caused by the procedures. RESULTS: Among the 51 patients, 26 had PEG and 25 had SG. Four cases in the SG group had fundoplication for gastroesophageal reflux disease. PEG and SG groups were followed up for an average of 29 months and 44 months. Major complications occurred in 19.2% of patients in the PEG group and 20.0% in the SG group, but significant differences between the groups were not observed. Minor complications occurred in 15.4% of patients in the PEG group and 52.0% in the SG group. Minor complications were significantly lower in the PEG group than in the SG group (p=0.006). The average use of antibiotics in the PEG and SG groups was 6.2 days and 15.7 days (p=0.002). Thirteen patients died of underlying disease but not related to gastrostomy, and only one patient died due to complications associated with general anesthesia. CONCLUSION: The duration of antibiotics use and incidence of minor complications were significantly lower in the PEG group than those in the SG group. Early PEG could be recommended for nutritional supports.

Medium- or Higher-Dose Acetylsalicylic Acid for Acute Kawasaki Disease and Patient Outcomes.

OBJECTIVE: To investigate the effect of medium- or higher-dose acetylsalicylic acid (ASA) for treating acute-phase Kawasaki disease to prevent coronary artery aneurysm (CAA). STUDY DESIGN: Among the children with acute Kawasaki disease investigated in the eighth nationwide survey in the Republic of Korea, 8456 children with adequate data were included in this study. The subjects were divided into 2 groups according to the use of medium- or higher-dose ASA (≥30 mg/kg/day), or-low dose ASA (3-5 mg/kg/day) during the acute febrile phase. Both z- score-based criteria and Japanese criteria for CAA were used. RESULTS: The prevalence of CAA based on z-score (24.8% vs 18.3%; P = .001) and on the Japanese criteria (19.0% vs 10.4%; P < .001) was higher in the 7947 patients who received medium- or higher-dose ASA compared with the 509 patients who received low-dose ASA. The use of medium- or higher-dose ASA was a significant predictor of CAA based on both sets of criteria by univariate analysis (based on z-score: OR, 1.472, 95% CI, 1.169-1.854, P = .001; based on Japanese criteria: OR, 2.013, 95% CI, 1.507-2.690, P < .001) and multivariate logistic regression analysis (OR, 1.527, 95% CI, 1.166-2.0, P = .003 and OR, 2.198, 95% CI, 1.563-3.092, P < .001, respectively). CONCLUSIONS: The use of medium- or higher-dose ASA in acute Kawasaki disease did not prevent CAA. A future randomized controlled trial is needed to determine the optimum dose of ASA.

Cerebral salt-wasting syndrome in a child with Wernicke encephalopathy treated with fludrocortisone therapy: A case report.

RATIONALE FOR THIS CASE REPORT: Cerebral Salt-Wasting Syndrome (CSWS) is characterized by hyponatremia and sodium wasting in the urine. These conditions are triggered by various neurosurgical disorders such as subarachnoid hemorrhage, brain tumor, head injury, and brain surgery. To our knowledge, CSWS caused by Wernicke encephalopathy (WE) has been rarely reported. PRESENTING CONCERNS OF THE PATIENT: A 2-year-old male patient presented to our hospital due to a seizure attack. He had been neglected and refused to take food for a long time (body weight < 3rd percentile). During admission, the patient showed low serum osmolality, high urine osmolality, dehydration state, increased urine output, and negative water balance, a diagnosis of CSWS was made. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Brain MRI displayed symmetrical lesions of T2WI and FLAIR high signal intensity in the peri-aqueductal and hypothalamic areas, which suggests Wernicke encephalopathy. For the early diagnosis of WE, neuroimaging studies can be an important marker. Thiamine hydrochloride was administered at a dose of 100 mg/day for 3 weeks. Cerebral salt-wasting syndrome was subsequently diagnosed due to persistent hyponatremia, dehydrated state, and high urine sodium with massive urination. MAIN LESSONS LEARNED FROM THIS CASE: Wernicke encephalopathy is a very rare cause of cerebral salt-wasting syndrome in pediatrics patients. The patient had a good outcome after hypertonic solution and fludrocortisone therapy.

Acoustic improvements after surgical correction in congenital heart disease.

BACKGROUND: Hoarse or asthenic voice is frequently associated with various pediatric cardiac disorders. The aim of this study was to investigate the changes in voice physiology after surgical correction in patients with congenital heart diseases. METHODS: We performed voice analysis using induced crying of 40 infants with congenital heart disease (CHD) such as ventricular septal defect (VSD), patent ductus arteriosus (PDA), and atrial septal defect (ASD; 31 girls, 24 boys; mean age, 11 ± 8.9 months). Cries were serially recorded immediately prior to operation, then 1 week, and 1 month after surgical correction, respectively. Acoustic parameters, fundamental frequency (F0 ), duration of cry, noise to harmonic ratio (NHR), jitter, and shimmer, were extracted using Multi-Dimensional Voice Program™ (MDVP) a computerized speech analysis system. Cries were compared with 30 normal healthy infants of corresponding age. RESULTS: Among the 25 infants with VSD, cry duration, jitter, and shimmer improved after the operation (P < 0.05). F0 and NHR, however, were not significantly different. F0 in patients with PDA improved, but was not statistically significant. The duration of cry, jitter, shimmer, and NHR improved in the PDA group (P < 0.05). The jitter and shimmer parameters improved significantly (P < 0.05), but F0 , cry duration, and NHR in patients with ASD did not show any significant changes. CONCLUSIONS: Deviated voice patterns in pediatric patients with CHD can normalize after surgical correction. In addition, non-invasive analysis such as MDVP can be used to identify vocal paralysis, even in the early postoperative period.

A case of unusual visceral heterotaxy syndrome with isolated levocardia.

Situs inversus of the abdominal organs in the presence of normally placed heart on the left side of the thorax is known as situs inversus with isolated levocardia. This rare condition is commonly associated with severe congenital defects of the heart. We report a case of situs inversus with levocardia in a 19-year-old asymptomatic male patient with completely normal heart on the left chest. Spiral computed tomography of the thorax and abdomen and echocardiographic studies revealed situs inversus of abdominal organs, normal heart (levocardia), mirrored left lungs, a midline liver, a left-sided inferior vena cava connecting to the right atrium, multiple splenic masses in the abdominal right upper quadrant, and aneurysmal dilatation of a splenic artery.

The Development Study of a Medical Ethics Education Program by Using Simple ISD Model.

PURPOSE: Simple instructional systems design (ISD) model is based on a fast development, usability test, and continuos feedback, which are necessary for educational program development in medical school. This study aims to figure out the usability of Simple ISD model for a medical ethics education program by describing a developmental details of each phase and its evaluation results. METHODS: Research has been conducted in two steps. First, while researchers participated in the program development by using Simple ISD model, we collected empirical data of each development activities. Second, the developed program was evaluated by students' web-based usability test, a 8-students' focus group interview and 5 faculty members' individual interviews in 4 domains; learning contents, instructional methods and strategies, achievement evaluation, and self-evaluation. RESULTS: Following the circular process of analysis, design, development, and usability test of Simple ISD model, a 10-week medical ethics program covering 9 instructional topics was developed. The average points of response on the developed medical ethics program in 2008 and 2009 are increased from 3.96 to 4.59 and 4.41, respectively. The prospects and limitations of the program are discussed. CONCLUSION: From a development study of the medical ethics program by using Simple ISD model, we could implement a more usable medical ethics program, and found 4 different usability of the Simple ISD model; the rapid development of educational program, program improvement by continuous feedback, faculty members' engagement in instructional design, and professional development of the faculty members.

A survey of pediatricians in private practices who participated in community-based clerkships: an intellectual, inspirational and professional growth experience.

PURPOSE: To examine how pediatricians in private practices are affected by the process of training medical students in their clinics as part of a community-based clerkship program. MATERIALS AND METHODS: In 2007, a questionnaire was sent to 35 pediatricians who had provided private clinical settings for clerkship training for the previous 3 years. The questionnaire covered a number of points, including the pediatricians' motivation to join and/or reasons to quit the program; if there were changes seen in their stress levels while supervising students; changes in their treatment procedures or attitudes because of the students' presence; responses of patients and/or their guardians in regard to have medical students treating them, and whether the doctors were inspired to grow professionally by participating in the program. RESULTS: Of the 35 pediatricians, 31 (88.5%) responded. Eighteen respondents (58%) selected 'responsibility to cooperate with medical school' as a reason to participate. Fifteen physicians (48.3%) answered that the clerkship program had a positive impact on their treatment procedures and their attitude towards patients. CONCLUSION: Based on the pediatricians' responses, the community-based clerkship program may instill intellectual inspiration and promote professional growth among the pediatricians in private practices, resulting in potentially better treatment for patients.

Demonstration of peripheral pulmonary stenosis and supravalvular aortic stenosis by different cardiac imaging modalities in a patient with Williams syndrome--usefulness of noninvasive imaging studies.

With technical improvement, noninvasive cardiac imaging modalities, including multislice computed tomography and magnetic resonance imaging, can offer sufficient imaging quality to differentiate cardiac anatomies. These noninvasive modalities also can identify aortic and pulmonary vascular abnormalities clearly in the same study. We report a case with severe pulmonary hypertension associated with peripheral pulmonary stenosis and supravalvular aortic stenosis in a 29-year-old woman with Williams syndrome, which was identified similarly with both invasive and noninvasive imaging modalities. The need for invasive tests can be reduced with appropriate improvement and validation of these noninvasive tests.

Erdheim-Chester disease in a child with MR imaging showing regression of marrow changes.

OBJECTIVE: Erdheim-Chester disease is a disseminated xanthogranulomatous infiltrative disease of unknown origin that generally presents in adulthood. A review of the English-language literature demonstrated that pediatric cases were extremely rare, and to our knowledge, only two cases, a 7- and 14-year-old, have been published. DESIGN AND PATIENT: We report a case of Erdheim-Chester disease in a 10-year-old girl evaluated with MR imaging. Radiographs revealed typical bilateral, symmetric osteosclerosis of the metaphyseal regions of long bones of the upper and lower extremities. RESULTS: A histologic examination demonstrated foamy histiocytes in bone marrow smears. Bilateral symmetric low signal intensities of both proximal tibiae and distal femurs were demonstrated on T1-weighted MR images. After oral steroid therapy for 8 months, follow-up MR imaging showed remarkable restoration of normal high signal intensity in both the tibial and femoral metaphyses. CONCLUSION: To our knowledge, this may be the first case of Erdheim-Chester disease that showed normal restoration of the abnormal signal intensities in the metaphyses of long bones after steroid therapy.

Papaverine blocks hKv1.5 channel current and human atrial ultrarapid delayed rectifier K+ currents.

Papaverine, 1-[(3,4-dimethoxyphenyl)methyl]-6,-7-dimethoxyisoquinoline, has been used as a vasodilator agent and a therapeutic agent for cerebral vasospasm, renal colic, and penile impotence. We examined the effects of papaverine on a rapidly activating delayed rectifier K(+) channel (hKv1.5) cloned from human heart and stably expressed in Ltk(-) cells as well as a corresponding K(+) current (the ultrarapid delayed rectifier, I(Kur)) in human atrial myocytes. Using the whole cell configuration of the patch-clamp technique, we found that papaverine inhibited hKv1.5 current in a time- and voltage-dependent manner with an IC(50) value of 43.4 microM at +60 mV. Papaverine accelerated the kinetics of the channel inactivation, suggesting the blockade of open channels. Papaverine (100 microM) also blocked I(Kur) in human atrial myocytes. These results indicate that papaverine blocks hKv1.5 channels and native hKv1.5 channels in a concentration-, voltage-, state-, and time-dependent manner. This interaction suggests that papaverine could alter cardiac excitability in vivo.

Open channel block by KCB-328 [1-(2-amino-4-methanesulfonamidophenoxy)-2-[N-(3,4-dimethoxyphenethyl)-N-methylamino]ethane hydrochloride] of the heterologously expressed human ether-a-go-go-related gene K+ channels.

KCB-328 [1-(2-amino-4-methanesulfonamidophenoxy)-2-[N-(3,4-dimethoxyphenethyl)-N-methylamino]ethane hydrochloride] is a newly synthesized class III antiarrhythmic drug and is known to be highly effective against various types of arrhythmias induced by coronary artery ligation, reperfusion, and programmed electrical stimulation. To understand the potential ionic mechanisms, we examined the effects of KCB-328, which encodes the rapidly activating delayed rectifier K(+) current in cardiac tissues, on human ether-a-go-go-related gene (HERG) channels expressed in Xenopus oocytes. The amplitudes of steady-state currents and tail currents of HERG were decreased by KCB-328 dose dependently. The decrease became more pronounced at more positive potential, suggesting that the block of HERG by KCB-328 is voltage-dependent. IC(50) values at -30, -20, -10, 0, +10, +20, +30, and +40 mV were 7.6 +/- 0.5, 4.8 +/- 0.4, 3.2 +/- 0.3, 2.1 +/- 0.3, 1.7 +/- 0.2, 1.4 +/- 0.2, 1.3 +/- 0.1, and 1.2 +/- 0.1 microM, respectively. Induction of block depended on depolarization beyond the threshold for channel opening. In addition, time-dependent block developed slowly, with tau = 1.7 +/- 0.3 s (100 microM) at 0 mV, and was delayed by a stronger depolarization to +80 mV, at which HERG channel is inactivated. We can conclude that KCB-328 preferentially blocks open (or activated) HERG channels. The block of HERG current might in part explain the underlying ionic mechanism for the antiarrhythmic and proarrhythmic effect of KCB-328.